Harriet J.

Paltiel
Edward Y. Lee
Editors

Pediatric Ultrasound

123
Pediatric Ultrasound
Harriet J. Paltiel • Edward Y. Lee
Editors

Pediatric Ultrasound
Editors
Harriet J. Paltiel Edward Y. Lee
Division of Ultrasound Division of Thoracic Imaging
Department of Radiology, Boston Children’s Department of Radiology, Boston Children’s
Hospital and Harvard Medical School Hospital and Harvard Medical School
Boston, MA Boston, MA
USA USA

ISBN 978-3-030-56801-6    ISBN 978-3-030-56802-3 (eBook)

https://doi.org/10.1007/978-3-030-56802-3

© Springer Nature Switzerland AG 2021

This work is subject to copyright. All rights are reserved by the Publisher, whether the whole or part of the material is
concerned, specifically the rights of translation, reprinting, reuse of illustrations, recitation, broadcasting, reproduction
on microfilms or in any other physical way, and transmission or information storage and retrieval, electronic adaptation,
computer software, or by similar or dissimilar methodology now known or hereafter developed.
The use of general descriptive names, registered names, trademarks, service marks, etc. in this publication does not
imply, even in the absence of a specific statement, that such names are exempt from the relevant protective laws and
regulations and therefore free for general use.
The publisher, the authors and the editors are safe to assume that the advice and information in this book are believed
to be true and accurate at the date of publication. Neither the publisher nor the authors or the editors give a warranty,
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This Springer imprint is published by the registered company Springer Nature Switzerland AG
The registered company address is: Gewerbestrasse 11, 6330 Cham, Switzerland
To Eric and Emil, to the memory of Sarah and Daniel, and to all the children
whose images grace these pages.
To my coeditor, Edward Y. Lee, MD, MPH, for sharing his vast knowledge
and experience in pediatric imaging.
– Harriet J. Paltiel, MDCM

To my parents, Kwan-Pyo and Kang-Ja, and my family for their constant

support and encouragement.
To the trainees and radiologists that I have met during my academic career
for sharing their enthusiasm for learning.
To my coeditor, Harriet J. Paltiel, MDCM, for sharing her expertise and
passion for pediatric ultrasound.
– Edward Y. Lee, MD, MPH
Preface

Ultrasound imaging plays an essential role in the diagnosis and management of pediatric dis-
orders. Its ease of use, flexibility, lack of ionizing radiation, and relatively low cost compared
to CT and MRI all enhance its attractiveness as a first-line imaging modality in patient evalu-
ation. Recent technological advances, including contrast-enhanced ultrasound and elastogra-
phy, continue to expand its diagnostic capabilities.
This book is intended to provide a comprehensive review of current knowledge in the field
of pediatric ultrasound and to serve as a reference for practicing physicians, residents and fel-
lows, medical students, sonographers, and all others with an interest in the wide applicability
of this important imaging tool in infants and children. An introductory chapter on the physics
of ultrasound imaging techniques and artifacts is followed by chapters devoted to ultrasound
of the brain, spine, and neck; chest, abdomen, and pelvis; musculoskeletal and vascular sys-
tems; and breast. The recent approval of ultrasound contrast agents for imaging of focal liver
lesions and for voiding cystography in the United States has opened the door to the develop-
ment and implementation of new applications of contrast-enhanced ultrasound of which many
examples are featured throughout the book. Applications of elastography to pediatric liver
diseases are also described. The rapid development and increased use of ultrasound in the
diagnosis and management in children with musculoskeletal and vascular disorders has
prompted the inclusion of chapters entirely devoted to these topics. Both American and inter-
national authors have been selected for their expertise and to reflect the global perspective and
intended audience for this book.
A systematic approach to each organ system is provided, including an overview of ultra-
sound imaging techniques, normal development, and anatomy. This is followed by a discus-
sion of normal variants, congenital anomalies, infectious and inflammatory disorders, traumatic
lesions, and benign and malignant tumors. A brief review of the etiology and pathophysiology
of each disorder precedes a description and illustration of the ultrasound imaging findings,
relevant correlative imaging, and current treatment approach. Every chapter includes pertinent
anatomical drawings, diagrams, and tables. Up-to-date references for each topic are provided.
A selection of cine clips is also included in the online version of the book which is available in
a fully searchable format.
We hope that this work, with its accessible style and outstanding images, will be valuable
to all readers in their daily practice and deepen their appreciation of the wide utility and appli-
cability of ultrasound in pediatric medicine.

Boston, MA, USA Harriet J. Paltiel

 Edward Y. Lee

vii
Acknowledgments

We are deeply grateful to the many colleagues whose efforts have contributed to this work. We
thank all of the authors for their outstanding contributions. Much appreciation goes to Stephanie
Frost, Development Editor, for her meticulous attention to detail in bringing this project to
fruition and to Margaret Moore, Publishing Editor, Springer Nature, for providing the resources
to accomplish this monumental task, and for her unwavering support and encouragement
through the entire gestation of the book.

ix
Contents

1 Ultrasound Imaging Techniques and Artifacts���������������������������������������������������������   1

Don-Soo Kim, Harriet J. Paltiel, Phillip Jason White, and Elisabetta Sassaroli
Introduction�����������������������������������������������������������������������������������������������������������������    1
Acoustics���������������������������������������������������������������������������������������������������������������������    2
Wavelength and Frequency�������������������������������������������������������������������������������������    2
Sound Propagation �������������������������������������������������������������������������������������������������    2
Acoustic Impedance�����������������������������������������������������������������������������������������������    4
Reflection ���������������������������������������������������������������������������������������������������������������    4
Refraction���������������������������������������������������������������������������������������������������������������    7
Attenuation�������������������������������������������������������������������������������������������������������������    7
Distance Measurement�������������������������������������������������������������������������������������������    8
Instrumentation�����������������������������������������������������������������������������������������������������������   11
Transmitter �������������������������������������������������������������������������������������������������������������   11
Transducer���������������������������������������������������������������������������������������������������������������   12
Receiver �����������������������������������������������������������������������������������������������������������������   12
Image Display���������������������������������������������������������������������������������������������������������   14
Image Storage���������������������������������������������������������������������������������������������������������   14
Mechanical Transducer�������������������������������������������������������������������������������������������   14
Array Transducer ���������������������������������������������������������������������������������������������������   19
Transducer Selection�����������������������������������������������������������������������������������������������   22
Harmonic Imaging �����������������������������������������������������������������������������������������������������   23
Spatial Compounding�������������������������������������������������������������������������������������������������   24
Three-Dimensional Ultrasound�����������������������������������������������������������������������������������   25
Doppler Ultrasound�����������������������������������������������������������������������������������������������������   26
Continuous Wave Doppler �������������������������������������������������������������������������������������   29
Pulsed Wave Doppler ���������������������������������������������������������������������������������������������   29
Color Doppler���������������������������������������������������������������������������������������������������������   31
Power Doppler �������������������������������������������������������������������������������������������������������   31
B-Flow �����������������������������������������������������������������������������������������������������������������������   32
Elastography Imaging�������������������������������������������������������������������������������������������������   32
Quasi-Static Strain Elastography ���������������������������������������������������������������������������   32
Dynamic Elastography�������������������������������������������������������������������������������������������   34
Ultrasound Contrast Imaging�������������������������������������������������������������������������������������   35
Contrast Agents�������������������������������������������������������������������������������������������������������   35
Pulse Inversion Imaging�����������������������������������������������������������������������������������������   36
Ultrasound Artifacts ���������������������������������������������������������������������������������������������������   37
Grayscale Artifacts�������������������������������������������������������������������������������������������������   37
Mirror Image: Multipath Reflection�������������������������������������������������������������������   37
Refraction�����������������������������������������������������������������������������������������������������������   37
Reverberation, Comet-Tail, and Ring-Down Artifacts���������������������������������������   38
Side Lobe Artifact�����������������������������������������������������������������������������������������������   39

xi
xii Contents

Enhancement and Shadowing Attenuation Artifacts�������������������������������������������   39

Partial Volume Artifact���������������������������������������������������������������������������������������   40
Doppler Artifacts�����������������������������������������������������������������������������������������������������   41
Technically Related Doppler Artifacts���������������������������������������������������������������   42
Inappropriate Doppler Settings�����������������������������������������������������������������������   42
Aliasing (Wraparound)�����������������������������������������������������������������������������������   43
Color Doppler Noise���������������������������������������������������������������������������������������   43
Flow Directional Abnormalities���������������������������������������������������������������������   44
Anatomically Related Doppler Artifacts�������������������������������������������������������������   44
Spectral Mirror Image Artifact�����������������������������������������������������������������������   44
Tissue Vibration Artifact���������������������������������������������������������������������������������   45
Twinkling Artifact�������������������������������������������������������������������������������������������   45
Blooming Artifact�������������������������������������������������������������������������������������������   46
Three-Dimensional Ultrasound Artifacts���������������������������������������������������������������   47
Ultrasound Contrast Agent Artifacts�����������������������������������������������������������������������   47
Blooming Artifact�����������������������������������������������������������������������������������������������   47
Systolic Peak Velocity Increase �������������������������������������������������������������������������   47
High-Intensity Transient Signals������������������������������������������������������������������������   47
Ultrasound Safety�������������������������������������������������������������������������������������������������������   47
Thermal Bioeffects�������������������������������������������������������������������������������������������������   47
Nonthermal Bioeffects �������������������������������������������������������������������������������������������   48
Regulations and Policies�����������������������������������������������������������������������������������������   48
References�������������������������������������������������������������������������������������������������������������������   49
2 Brain����������������������������������������������������������������������������������������������������������������������������� 51
Helen H. R. Kim, Wendy G. Kim, Edward Y. Lee, and Grace S. Phillips
Introduction�����������������������������������������������������������������������������������������������������������������   51
Technique�������������������������������������������������������������������������������������������������������������������   52
Patient Positioning �������������������������������������������������������������������������������������������������   52
Ultrasound Transducer Selection ���������������������������������������������������������������������������   52
Imaging Approaches�����������������������������������������������������������������������������������������������   52
Normal Development and Anatomy���������������������������������������������������������������������������   57
Normal Development���������������������������������������������������������������������������������������������   57
Normal Anatomy�����������������������������������������������������������������������������������������������������   57
Cerebral Cortex�������������������������������������������������������������������������������������������������������   58
Basal Ganglia and Thalami�������������������������������������������������������������������������������������   58
Ventricles�����������������������������������������������������������������������������������������������������������������   59
Cisterna Magna�������������������������������������������������������������������������������������������������������   60
Extra-Axial Fluid Spaces ���������������������������������������������������������������������������������������   61
Congenital Brain Anomalies���������������������������������������������������������������������������������������   61
Dorsal Induction Disorders�������������������������������������������������������������������������������������   61
Chiari Malformations�����������������������������������������������������������������������������������������   61
Anencephaly�������������������������������������������������������������������������������������������������������   63
Hydranencephaly �����������������������������������������������������������������������������������������������   63
Ventral Induction Disorders �����������������������������������������������������������������������������������   63
Holoprosencephaly���������������������������������������������������������������������������������������������   63
Septo-Optic (Pituitary) Dysplasia�����������������������������������������������������������������������   64
Anomalies of the Corpus Callosum �������������������������������������������������������������������   65
Dandy–Walker Syndrome�����������������������������������������������������������������������������������   67
Neuronal Proliferation Disorders���������������������������������������������������������������������������   68
Hemimegalencephaly�����������������������������������������������������������������������������������������   68
Neuronal Migration Disorders �������������������������������������������������������������������������������   68
Lissencephaly�����������������������������������������������������������������������������������������������������   68
Gray Matter Heterotopia�������������������������������������������������������������������������������������   68
Post-Migration Disorders���������������������������������������������������������������������������������������   68
Contents xiii

Polymicrogyria���������������������������������������������������������������������������������������������������   68
Schizencephaly���������������������������������������������������������������������������������������������������   68
Intracranial Hemorrhage���������������������������������������������������������������������������������������������   70
Preterm Infants�������������������������������������������������������������������������������������������������������   70
Periventricular Hemorrhagic Infarction �������������������������������������������������������������   72
Cerebellar Hemorrhage���������������������������������������������������������������������������������������   72
Term Infants �����������������������������������������������������������������������������������������������������������   72
Epidural Hemorrhage�����������������������������������������������������������������������������������������   72
Subdural Hemorrhage�����������������������������������������������������������������������������������������   74
Subpial Hemorrhage�������������������������������������������������������������������������������������������   75
Parenchymal Hemorrhage�����������������������������������������������������������������������������������   75
Choroid Plexus Hemorrhage�������������������������������������������������������������������������������   75
Hypoxic–Ischemic Injury�������������������������������������������������������������������������������������������   75
Global Hypoxic–Ischemic Injury���������������������������������������������������������������������������   76
Preterm Infants���������������������������������������������������������������������������������������������������   76
Term Infants �������������������������������������������������������������������������������������������������������   77
Focal Hypoxic–Ischemic Injury�����������������������������������������������������������������������������   78
Arterial Ischemic Stroke�������������������������������������������������������������������������������������   78
Venous Sinus Thrombosis�����������������������������������������������������������������������������������   81
Sickle Cell Disease���������������������������������������������������������������������������������������������   81
Infectious Brain Disorders �����������������������������������������������������������������������������������������   84
Viral Infections�������������������������������������������������������������������������������������������������������   84
Bacterial Infections�������������������������������������������������������������������������������������������������   86
Fungal Infections ���������������������������������������������������������������������������������������������������   88
Neoplastic Brain and Ventricular Disorders���������������������������������������������������������������   88
Neoplasms of the Brain�������������������������������������������������������������������������������������������   88
Neoplasms of the Ventricle�������������������������������������������������������������������������������������   88
Vascular Brain Disorders �������������������������������������������������������������������������������������������   89
High-Flow Malformations �������������������������������������������������������������������������������������   90
Low-Flow Malformations���������������������������������������������������������������������������������������   90
Hydrocephalus �����������������������������������������������������������������������������������������������������������   90
Benign External Hydrocephalus�����������������������������������������������������������������������������   92
Scalp Masses���������������������������������������������������������������������������������������������������������������   93
Congenital Scalp Lesions���������������������������������������������������������������������������������������   93
Dermoid/Epidermoid Cyst ���������������������������������������������������������������������������������   93
Cephalocele���������������������������������������������������������������������������������������������������������   94
Lymph Node�����������������������������������������������������������������������������������������������������������   94
Extracranial Birth Trauma���������������������������������������������������������������������������������������   95
Vascular Scalp Lesions�������������������������������������������������������������������������������������������   95
Infantile Hemangioma ���������������������������������������������������������������������������������������   95
Sinus Pericranii���������������������������������������������������������������������������������������������������   96
Suture Evaluation�������������������������������������������������������������������������������������������������������   97
Craniosynostosis�����������������������������������������������������������������������������������������������������   97
Positional Plagiocephaly�����������������������������������������������������������������������������������������   99
References�������������������������������������������������������������������������������������������������������������������   99
3 Spine����������������������������������������������������������������������������������������������������������������������������� 103
Maddy Artunduaga, Domen Plut, Abbey J. Winant, Ricardo Restrepo,
and Edward Y. Lee
Introduction����������������������������������������������������������������������������������������������������������������� 103
Technique������������������������������������������������������������������������������������������������������������������� 103
Patient Positioning ������������������������������������������������������������������������������������������������� 103
Ultrasound Transducer Selection ��������������������������������������������������������������������������� 104
Imaging Approaches����������������������������������������������������������������������������������������������� 104
xiv Contents

Normal Development and Anatomy��������������������������������������������������������������������������� 104

Normal Development��������������������������������������������������������������������������������������������� 104
Normal Anatomy����������������������������������������������������������������������������������������������������� 104
Spinal Cord��������������������������������������������������������������������������������������������������������� 107
Central Canal ����������������������������������������������������������������������������������������������������� 108
Ventriculus Terminalis ��������������������������������������������������������������������������������������� 108
Filar Cyst������������������������������������������������������������������������������������������������������������� 109
Fatty Filum��������������������������������������������������������������������������������������������������������� 109
Positional Nerve Root Clumping (“Pseudomass”)��������������������������������������������� 110
Pseudosinus Tract����������������������������������������������������������������������������������������������� 110
Dysmorphic Coccyx������������������������������������������������������������������������������������������� 110
Congenital Spinal Anomalies������������������������������������������������������������������������������������� 110
Spinal Dysraphism ������������������������������������������������������������������������������������������������� 111
Open Defect ������������������������������������������������������������������������������������������������������� 112
Meningocele��������������������������������������������������������������������������������������������������� 112
Myelocele and Myelomeningocele����������������������������������������������������������������� 112
Closed Defect����������������������������������������������������������������������������������������������������� 113
Simple Closed Defects����������������������������������������������������������������������������������� 113
Complex Closed Defects��������������������������������������������������������������������������������� 114
Lipomatous Tissue and Dural Defect (Lipomyelomeningocele,
Lipomyelocele)����������������������������������������������������������������������������������������������� 116
CSF-Containing Defects��������������������������������������������������������������������������������� 116
Tethered Cord��������������������������������������������������������������������������������������������������������� 116
Caudal Regression Syndrome��������������������������������������������������������������������������������� 118
Spinal Lipoma��������������������������������������������������������������������������������������������������������� 119
Segmental Spinal Dysgenesis��������������������������������������������������������������������������������� 119
Infectious and Inflammatory Spinal Disorders����������������������������������������������������������� 120
Epidural Abscess����������������������������������������������������������������������������������������������������� 120
Pilonidal Sinus and Cyst����������������������������������������������������������������������������������������� 120
Neoplastic Spinal Disorders��������������������������������������������������������������������������������������� 121
Intramedullary Tumors������������������������������������������������������������������������������������������� 122
Extramedullary Tumors ����������������������������������������������������������������������������������������� 122
Sacrococcygeal Teratoma��������������������������������������������������������������������������������������� 122
Traumatic Spinal Disorders ��������������������������������������������������������������������������������������� 124
Lumbar Puncture����������������������������������������������������������������������������������������������������� 124
Spinal Cord Injury��������������������������������������������������������������������������������������������������� 124
Spinal Cord Laceration and Transection������������������������������������������������������������� 124
Epidural/Subdural Hematoma and Hematomyelia��������������������������������������������� 124
Hydromyelia/Syringomyelia/Syrinx��������������������������������������������������������������������������� 125
References������������������������������������������������������������������������������������������������������������������� 125
4 Neck����������������������������������������������������������������������������������������������������������������������������� 127
Patricia T. Acharya, Sharon R. Gordon, Mark C. Liszewski, Ricardo Restrepo,
and Edward Y. Lee
Introduction����������������������������������������������������������������������������������������������������������������� 127
Technique������������������������������������������������������������������������������������������������������������������� 127
Patient Positioning ������������������������������������������������������������������������������������������������� 127
Ultrasound Transducer Selection ��������������������������������������������������������������������������� 127
Imaging Approaches����������������������������������������������������������������������������������������������� 128
Normal Development and Anatomy��������������������������������������������������������������������������� 128
Neck ����������������������������������������������������������������������������������������������������������������������� 128
Anterior Triangle������������������������������������������������������������������������������������������������� 128
Posterior Triangle����������������������������������������������������������������������������������������������� 128
Contents xv

Lymph Nodes ��������������������������������������������������������������������������������������������������������� 129

Thyroid Gland��������������������������������������������������������������������������������������������������������� 130
Parathyroid Glands������������������������������������������������������������������������������������������������� 131
Salivary Glands������������������������������������������������������������������������������������������������������� 131
Parotid Glands����������������������������������������������������������������������������������������������������� 131
Submandibular Glands��������������������������������������������������������������������������������������� 132
Sublingual Glands����������������������������������������������������������������������������������������������� 133
Neck ��������������������������������������������������������������������������������������������������������������������������� 133
Congenital Neck Anomalies����������������������������������������������������������������������������������� 133
Congenital Nonvascular Neck Masses��������������������������������������������������������������� 133
Congenital Benign Cystic Neck Masses��������������������������������������������������������� 133
Congenital Benign Solid Neck Masses����������������������������������������������������������� 138
Congenital Vascular Neck Masses ��������������������������������������������������������������������� 138
Hemangiomas������������������������������������������������������������������������������������������������� 138
Vascular Malformations ��������������������������������������������������������������������������������� 140
Infectious and Inflammatory Neck Disorders��������������������������������������������������������� 144
Cervical Lymphadenitis and Abscess����������������������������������������������������������������� 144
Mycobacterial Infection ������������������������������������������������������������������������������������� 145
Other Infectious and Inflammatory Disorders ��������������������������������������������������� 146
Neoplastic Neck Disorders������������������������������������������������������������������������������������� 146
Benign Neck Neoplasms������������������������������������������������������������������������������������� 146
Myofibromatosis��������������������������������������������������������������������������������������������� 146
Neurofibroma ������������������������������������������������������������������������������������������������� 147
Lipoma and Lipoblastoma ����������������������������������������������������������������������������� 147
Malignant Neck Neoplasms������������������������������������������������������������������������������� 149
Lymphoma ����������������������������������������������������������������������������������������������������� 149
Rhabdomyosarcoma��������������������������������������������������������������������������������������� 150
Metastatic Disease ��������������������������������������������������������������������������������������������� 150
Thyroid Gland������������������������������������������������������������������������������������������������������������� 151
Congenital Thyroid Gland Anomalies ������������������������������������������������������������������� 151
Dysgenesis ��������������������������������������������������������������������������������������������������������� 151
Dyshormonogenesis������������������������������������������������������������������������������������������� 152
Focal Thyroid Gland Lesions��������������������������������������������������������������������������������� 152
Cystic Focal Thyroid Gland Lesions ����������������������������������������������������������������� 152
Colloid Cysts��������������������������������������������������������������������������������������������������� 152
Simple Cysts��������������������������������������������������������������������������������������������������� 152
Complex (Hemorrhagic) Cysts����������������������������������������������������������������������� 152
Solid Focal Thyroid Gland Lesions ������������������������������������������������������������������� 152
Benign Solid Focal Thyroid Gland Lesions��������������������������������������������������� 154
Malignant Solid Focal Thyroid Gland Lesions����������������������������������������������� 155
Diffuse Parenchymal Thyroid Gland Lesions��������������������������������������������������������� 157
Multinodular Goiter (Nodular Hyperplasia)������������������������������������������������������� 157
Infectious Diffuse Parenchymal Thyroid Gland Disorders��������������������������������� 159
Acute Suppurative (Bacterial) Thyroiditis ����������������������������������������������������� 159
Subacute (De Quervain) Thyroiditis��������������������������������������������������������������� 159
Autoimmune-Mediated Diffuse Parenchymal Thyroid Gland Lesions ������������� 159
Graves’ Disease����������������������������������������������������������������������������������������������� 159
Hashimoto Thyroiditis ����������������������������������������������������������������������������������� 160
Parathyroid Glands����������������������������������������������������������������������������������������������������� 161
Parathyroid Cyst����������������������������������������������������������������������������������������������������� 161
Parathyroid Hyperplasia����������������������������������������������������������������������������������������� 162
Parathyroid Adenoma��������������������������������������������������������������������������������������������� 162
Salivary Glands����������������������������������������������������������������������������������������������������������� 163
xvi Contents

Congenital Salivary Gland Anomalies ������������������������������������������������������������������� 163

First Branchial Cleft Cyst����������������������������������������������������������������������������������� 163
Ranula����������������������������������������������������������������������������������������������������������������� 163
Infectious and Inflammatory Salivary Gland Disorders����������������������������������������� 164
Acute Infectious and Inflammatory Disorders��������������������������������������������������� 164
Viral (Nonsuppurative) Inflammation������������������������������������������������������������� 164
Bacterial Parotitis and Abscess����������������������������������������������������������������������� 164
Recurrent and Chronic Infectious and Inflammatory Disorders������������������������� 165
Chronic Sialadenitis ��������������������������������������������������������������������������������������� 165
Neoplastic Salivary Gland Disorders ��������������������������������������������������������������������� 166
Benign Salivary Gland Masses��������������������������������������������������������������������������� 166
Infantile Hemangioma ����������������������������������������������������������������������������������� 166
Lymphatic Malformation ������������������������������������������������������������������������������� 166
Malignant Salivary Gland Neoplasms ��������������������������������������������������������������� 166
Mucoepidermoid Carcinoma ������������������������������������������������������������������������� 166
Acinar Cell Carcinoma����������������������������������������������������������������������������������� 166
Other Malignant Salivary Gland Neoplasms ������������������������������������������������� 167
Sialolithiasis����������������������������������������������������������������������������������������������������������� 167
References������������������������������������������������������������������������������������������������������������������� 168
5 Lung����������������������������������������������������������������������������������������������������������������������������� 173
Eric S. Bih, Monica Epelman, Ricardo Restrepo, and Edward Y. Lee
Introduction����������������������������������������������������������������������������������������������������������������� 173
Technique������������������������������������������������������������������������������������������������������������������� 173
Patient Positioning ������������������������������������������������������������������������������������������������� 173
Ultrasound Transducer Selection ��������������������������������������������������������������������������� 173
Imaging Approaches����������������������������������������������������������������������������������������������� 174
Contrast-Enhanced Ultrasound������������������������������������������������������������������������������� 174
Normal Development and Anatomy��������������������������������������������������������������������������� 174
Normal Development��������������������������������������������������������������������������������������������� 174
Normal Anatomy����������������������������������������������������������������������������������������������������� 175
Ultrasound Signs��������������������������������������������������������������������������������������������������������� 175
The Pleural Line����������������������������������������������������������������������������������������������������� 175
Lung Sliding����������������������������������������������������������������������������������������������������������� 175
A-Lines������������������������������������������������������������������������������������������������������������������� 176
B-Lines������������������������������������������������������������������������������������������������������������������� 176
M-Mode Appearance of the Normal Lung������������������������������������������������������������� 176
Hepatization and Air Bronchograms����������������������������������������������������������������������� 176
Congenital Lung Anomalies��������������������������������������������������������������������������������������� 177
Congenital Lobar Hyperinflation ��������������������������������������������������������������������������� 178
Foregut Duplication Cyst ��������������������������������������������������������������������������������������� 179
Congenital Pulmonary Airway Malformation ������������������������������������������������������� 180
Bronchopulmonary Sequestration��������������������������������������������������������������������������� 180
Intralobar Bronchopulmonary Sequestration����������������������������������������������������� 181
Extralobar Bronchopulmonary Sequestration����������������������������������������������������� 182
Hybrid Congenital Lung Anomalies������������������������������������������������������������������� 183
Atelectasis and Consolidation������������������������������������������������������������������������������������� 183
Pulmonary Necrosis ����������������������������������������������������������������������������������������������� 183
Pulmonary Abscess������������������������������������������������������������������������������������������������� 184
Interstitial Lung Disease��������������������������������������������������������������������������������������������� 185
Pulmonary Lymphangiectasia������������������������������������������������������������������������������������� 188
Pulmonary Masses ����������������������������������������������������������������������������������������������������� 188
Benign Pulmonary Masses������������������������������������������������������������������������������������� 189
Contents xvii

Malignant Pulmonary Neoplasms��������������������������������������������������������������������������� 189

Primary Malignant Pulmonary Neoplasms��������������������������������������������������������� 189
Pleuropulmonary Blastoma����������������������������������������������������������������������������� 189
Rhabdomyosarcoma��������������������������������������������������������������������������������������� 189
Metastases����������������������������������������������������������������������������������������������������������� 190
References������������������������������������������������������������������������������������������������������������������� 191
6 Pleura��������������������������������������������������������������������������������������������������������������������������� 195
Nathan David P. Concepcion, Bernard F. Laya, Ross A. Myers,
and Edward Y. Lee
Introduction����������������������������������������������������������������������������������������������������������������� 195
Technique������������������������������������������������������������������������������������������������������������������� 195
Patient Positioning ������������������������������������������������������������������������������������������������� 196
Ultrasound Transducer Selection ��������������������������������������������������������������������������� 196
Imaging Approaches����������������������������������������������������������������������������������������������� 196
Normal Development and Anatomy��������������������������������������������������������������������������� 196
Normal Development��������������������������������������������������������������������������������������������� 197
Normal Anatomy����������������������������������������������������������������������������������������������������� 197
Anatomic Variants��������������������������������������������������������������������������������������������������� 199
Normal Ultrasound Anatomy��������������������������������������������������������������������������������� 199
A-Lines��������������������������������������������������������������������������������������������������������������� 200
B-Lines��������������������������������������������������������������������������������������������������������������� 200
T-Lines ��������������������������������������������������������������������������������������������������������������� 200
Z-Lines��������������������������������������������������������������������������������������������������������������� 201
Pleural Effusion���������������������������������������������������������������������������������������������������������� 201
Simple Pleural Effusion ����������������������������������������������������������������������������������������� 202
Complex Pleural Effusion��������������������������������������������������������������������������������������� 202
Parapneumonic Effusion����������������������������������������������������������������������������������������� 203
Empyema ����������������������������������������������������������������������������������������������������������� 204
Fibrothorax��������������������������������������������������������������������������������������������������������� 204
Traumatic Effusion������������������������������������������������������������������������������������������������� 205
Hemorrhagic Effusion����������������������������������������������������������������������������������������� 205
Extrapleural Hematoma ������������������������������������������������������������������������������������� 206
Chylous Effusion����������������������������������������������������������������������������������������������������� 206
Pneumothorax������������������������������������������������������������������������������������������������������������� 207
Absence of Lung Sliding ��������������������������������������������������������������������������������������� 208
Stratosphere or Barcode Signs ������������������������������������������������������������������������������� 209
Absence of B-Lines������������������������������������������������������������������������������������������������� 209
Lung Point��������������������������������������������������������������������������������������������������������������� 209
Absence of Lung Pulse������������������������������������������������������������������������������������������� 209
Pleural Masses ����������������������������������������������������������������������������������������������������������� 210
Malignant Pleural Masses��������������������������������������������������������������������������������������� 211
Primary Neoplasms��������������������������������������������������������������������������������������������� 211
Pleuropulmonary Blastoma����������������������������������������������������������������������������� 211
Rhabdomyosarcoma��������������������������������������������������������������������������������������� 212
Metastases����������������������������������������������������������������������������������������������������������� 214
References������������������������������������������������������������������������������������������������������������������� 214
7 Mediastinum��������������������������������������������������������������������������������������������������������������� 219
Sumera Ali, Abbey J. Winant, Ricardo Restrepo, Pedro Daltro,
and Edward Y. Lee
Introduction����������������������������������������������������������������������������������������������������������������� 219
Technique������������������������������������������������������������������������������������������������������������������� 219
Patient Positioning ������������������������������������������������������������������������������������������������� 219
xviii Contents

Ultrasound Transducer Selection ��������������������������������������������������������������������������� 220

Imaging Approaches����������������������������������������������������������������������������������������������� 220
Normal Development and Anatomy��������������������������������������������������������������������������� 220
Normal Development��������������������������������������������������������������������������������������������� 220
Normal Anatomy����������������������������������������������������������������������������������������������������� 221
Compartment Approach ����������������������������������������������������������������������������������������� 221
Thymus������������������������������������������������������������������������������������������������������������������� 223
Trachea������������������������������������������������������������������������������������������������������������������� 224
Esophagus��������������������������������������������������������������������������������������������������������������� 224
Mediastinal Masses����������������������������������������������������������������������������������������������������� 225
Prevascular (Anterior) Mediastinal Masses ����������������������������������������������������������� 225
Teratoma������������������������������������������������������������������������������������������������������������� 225
Lymphoma ��������������������������������������������������������������������������������������������������������� 226
Lymphatic Malformation ����������������������������������������������������������������������������������� 227
Visceral (Middle) Mediastinal Masses������������������������������������������������������������������� 229
Foregut Duplication Cysts ��������������������������������������������������������������������������������� 229
Lymphadenopathy����������������������������������������������������������������������������������������������� 231
Infectious Lymphadenopathy������������������������������������������������������������������������� 231
Neoplastic Lymphadenopathy������������������������������������������������������������������������� 232
Paravertebral (Posterior) Mediastinal Masses��������������������������������������������������������� 233
Neuroblastoma ��������������������������������������������������������������������������������������������������� 233
Ganglioneuroblastoma ��������������������������������������������������������������������������������������� 233
Ganglioneuroma������������������������������������������������������������������������������������������������� 234
Cardiophrenic Angle Masses ������������������������������������������������������������������������������������� 235
Pericardial Cyst������������������������������������������������������������������������������������������������������� 235
Lymphadenopathy��������������������������������������������������������������������������������������������������� 235
References������������������������������������������������������������������������������������������������������������������� 236
8 Chest Wall������������������������������������������������������������������������������������������������������������������� 239
Jessica Kurian
Introduction����������������������������������������������������������������������������������������������������������������� 239
Technique������������������������������������������������������������������������������������������������������������������� 239
Patient Positioning ������������������������������������������������������������������������������������������������� 239
Ultrasound Transducer Selection ��������������������������������������������������������������������������� 239
Imaging Approaches����������������������������������������������������������������������������������������������� 241
Protocols������������������������������������������������������������������������������������������������������������� 241
Annotations��������������������������������������������������������������������������������������������������������� 241
Contrast-Enhanced Ultrasound������������������������������������������������������������������������������� 241
Normal Development and Anatomy��������������������������������������������������������������������������� 241
Normal Development��������������������������������������������������������������������������������������������� 241
Thoracic Skeleton����������������������������������������������������������������������������������������������� 241
Chest Wall Soft Tissues��������������������������������������������������������������������������������������� 242
Normal Anatomy����������������������������������������������������������������������������������������������������� 243
Thoracic Skeleton����������������������������������������������������������������������������������������������� 243
Musculature��������������������������������������������������������������������������������������������������������� 244
Ultrasound Appearance of Normal Chest Wall Anatomy��������������������������������������� 246
Congenital Chest Wall Anomalies ����������������������������������������������������������������������������� 247
Vascular Tumors and Malformations ��������������������������������������������������������������������� 247
Hemangioma������������������������������������������������������������������������������������������������������� 248
Venous Malformation����������������������������������������������������������������������������������������� 250
Lymphatic Malformation ����������������������������������������������������������������������������������� 251
Osseous and Cartilaginous Lesions ����������������������������������������������������������������������� 251
Osteochondroma (Exostosis) ����������������������������������������������������������������������������� 251
Asymmetric Cartilaginous Costochondral Junction������������������������������������������� 252
Enlarged Rib Ends ��������������������������������������������������������������������������������������������� 253
Contents xix

Infectious Disorders of the Chest Wall����������������������������������������������������������������������� 255

Cellulitis����������������������������������������������������������������������������������������������������������������� 255
Abscess������������������������������������������������������������������������������������������������������������������� 256
Neoplastic Disorders of the Chest Wall ��������������������������������������������������������������������� 256
Benign Chest Wall Neoplasms������������������������������������������������������������������������������� 257
Lipoma ��������������������������������������������������������������������������������������������������������������� 257
Mesenchymal Hamartoma ��������������������������������������������������������������������������������� 257
Malignant Chest Wall Neoplasms��������������������������������������������������������������������������� 258
Rhabdomyosarcoma������������������������������������������������������������������������������������������� 258
Ewing Sarcoma��������������������������������������������������������������������������������������������������� 259
Osteosarcoma����������������������������������������������������������������������������������������������������� 260
Lymphoma ��������������������������������������������������������������������������������������������������������� 261
Metastases����������������������������������������������������������������������������������������������������������� 262
Traumatic Disorders of the Chest Wall����������������������������������������������������������������������� 262
Hematoma��������������������������������������������������������������������������������������������������������������� 262
Rib Fracture ����������������������������������������������������������������������������������������������������������� 264
Foreign Bodies ����������������������������������������������������������������������������������������������������������� 265
References������������������������������������������������������������������������������������������������������������������� 266
9 Diaphragm������������������������������������������������������������������������������������������������������������������� 271
Wendy G. Kim, Helen H. R. Kim, Grace S. Phillips, and Edward Y. Lee
Introduction����������������������������������������������������������������������������������������������������������������� 271
Technique������������������������������������������������������������������������������������������������������������������� 271
Patient Positioning ������������������������������������������������������������������������������������������������� 271
Ultrasound Transducer Selection ��������������������������������������������������������������������������� 271
Imaging Approaches����������������������������������������������������������������������������������������������� 272
Normal Development and Anatomy��������������������������������������������������������������������������� 272
Normal Development��������������������������������������������������������������������������������������������� 272
Normal Anatomy����������������������������������������������������������������������������������������������������� 273
Congenital Diaphragmatic Anomalies ����������������������������������������������������������������������� 274
Diaphragmatic Hernia��������������������������������������������������������������������������������������������� 274
Bochdalek Hernia����������������������������������������������������������������������������������������������� 274
Morgagni Hernia������������������������������������������������������������������������������������������������� 275
Hiatal Hernia������������������������������������������������������������������������������������������������������� 275
Diaphragmatic Eventration������������������������������������������������������������������������������������� 277
Acquired Diaphragmatic Disorders ��������������������������������������������������������������������������� 278
Diaphragmatic Dysfunction ����������������������������������������������������������������������������������� 278
Diaphragmatic Inversion����������������������������������������������������������������������������������������� 279
Primary Diaphragmatic Masses ��������������������������������������������������������������������������������� 279
Benign Masses ������������������������������������������������������������������������������������������������������� 279
Malignant Neoplasms��������������������������������������������������������������������������������������������� 280
Traumatic Disorders��������������������������������������������������������������������������������������������������� 280
References������������������������������������������������������������������������������������������������������������������� 281
10 The Gastrointestinal Tract����������������������������������������������������������������������������������������� 283
Marthe M. Munden and Harriet J. Paltiel
Introduction����������������������������������������������������������������������������������������������������������������� 283
Esophagus������������������������������������������������������������������������������������������������������������������� 284
Technique��������������������������������������������������������������������������������������������������������������� 284
Patient Positioning ��������������������������������������������������������������������������������������������� 284
Ultrasound Transducer Selection ����������������������������������������������������������������������� 284
Imaging Approaches������������������������������������������������������������������������������������������� 284
Normal Development and Anatomy����������������������������������������������������������������������� 284
Normal Development����������������������������������������������������������������������������������������� 284
xx Contents

Normal Anatomy������������������������������������������������������������������������������������������������� 285

Gastroesophageal Reflux ��������������������������������������������������������������������������������������� 286
Hiatal Hernia����������������������������������������������������������������������������������������������������������� 286
Stomach����������������������������������������������������������������������������������������������������������������������� 288
Technique��������������������������������������������������������������������������������������������������������������� 288
Patient Positioning ��������������������������������������������������������������������������������������������� 288
Ultrasound Transducer Selection ����������������������������������������������������������������������� 289
Imaging Approaches������������������������������������������������������������������������������������������� 289
Normal Development and Anatomy����������������������������������������������������������������������� 290
Normal Development����������������������������������������������������������������������������������������� 290
Normal Anatomy������������������������������������������������������������������������������������������������� 290
Congenital Anomalies��������������������������������������������������������������������������������������������� 290
Gastric Atresia����������������������������������������������������������������������������������������������������� 290
Microgastria ������������������������������������������������������������������������������������������������������� 290
Gastric Diaphragm (Antral Web) ����������������������������������������������������������������������� 292
Acquired Obstruction��������������������������������������������������������������������������������������������� 292
Hypertrophic Pyloric Stenosis ��������������������������������������������������������������������������� 292
Pylorospasm������������������������������������������������������������������������������������������������������� 293
Prostaglandin-Induced Foveolar Hyperplasia����������������������������������������������������� 294
Gastric Volvulus ������������������������������������������������������������������������������������������������� 294
Gastric Wall Thickening����������������������������������������������������������������������������������������� 297
Gastritis��������������������������������������������������������������������������������������������������������������� 297
Ménétrier Disease����������������������������������������������������������������������������������������������� 297
Eosinophilic Gastroenteritis������������������������������������������������������������������������������� 297
Chronic Granulomatous Disease of the Stomach����������������������������������������������� 298
Benign Masses of the Stomach������������������������������������������������������������������������������� 298
Gastric Duplication Cyst������������������������������������������������������������������������������������� 298
Gastric Teratoma������������������������������������������������������������������������������������������������� 300
Gastric Lipoma��������������������������������������������������������������������������������������������������� 300
Focal Foveolar Hyperplasia of the Stomach������������������������������������������������������� 300
Inflammatory Gastric Myofibroblastic Tumor ��������������������������������������������������� 300
Gastric Bezoar����������������������������������������������������������������������������������������������������� 301
Other Benign Masses ����������������������������������������������������������������������������������������� 301
Malignant Gastric Tumors ������������������������������������������������������������������������������������� 301
Lymphoma ��������������������������������������������������������������������������������������������������������� 301
GI Stromal Tumor����������������������������������������������������������������������������������������������� 303
Other Malignant Masses������������������������������������������������������������������������������������� 303
Small Bowel��������������������������������������������������������������������������������������������������������������� 305
Technique��������������������������������������������������������������������������������������������������������������� 305
Patient Positioning ��������������������������������������������������������������������������������������������� 305
Ultrasound Transducer Selection ����������������������������������������������������������������������� 305
Imaging Approaches������������������������������������������������������������������������������������������� 305
Normal Development and Anatomy����������������������������������������������������������������������� 305
Normal Development����������������������������������������������������������������������������������������� 305
Normal Anatomy������������������������������������������������������������������������������������������������� 305
Congenital Anomalies��������������������������������������������������������������������������������������������� 306
Duodenal Atresia, Stenosis, and Web����������������������������������������������������������������� 306
Intestinal Atresia������������������������������������������������������������������������������������������������� 307
Jejunal and Ileal Stenosis ����������������������������������������������������������������������������������� 308
Midgut Malrotation��������������������������������������������������������������������������������������������� 309
Meconium Ileus ������������������������������������������������������������������������������������������������� 311
Meconium Peritonitis and Pseudocyst ��������������������������������������������������������������� 312
Acquired Obstruction��������������������������������������������������������������������������������������������� 313
Contents xxi

Intussusception��������������������������������������������������������������������������������������������������� 313
Small Bowel Intussusception ������������������������������������������������������������������������� 313
Ileocolic Intussusception��������������������������������������������������������������������������������� 315
Small Bowel Wall Thickening ������������������������������������������������������������������������������� 316
Infectious Enteritis ��������������������������������������������������������������������������������������������� 316
Crohn Disease����������������������������������������������������������������������������������������������������� 317
Hemorrhage��������������������������������������������������������������������������������������������������������� 319
Trauma ����������������������������������������������������������������������������������������������������������� 319
Henoch–Schönlein Purpura ��������������������������������������������������������������������������� 319
Eosinophilic Gastroenteritis������������������������������������������������������������������������������� 320
Lymphangiectasia����������������������������������������������������������������������������������������������� 321
Cystic Fibrosis ��������������������������������������������������������������������������������������������������� 321
Graft-Versus-Host Disease��������������������������������������������������������������������������������� 322
Meckel Diverticulum ��������������������������������������������������������������������������������������������� 323
Benign Masses ������������������������������������������������������������������������������������������������������� 324
Duplication Cyst������������������������������������������������������������������������������������������������� 324
Mesenteric Cyst ������������������������������������������������������������������������������������������������� 326
Intestinal Polyp��������������������������������������������������������������������������������������������������� 327
Vascular Anomalies��������������������������������������������������������������������������������������������� 327
Infantile Hemangioma ����������������������������������������������������������������������������������� 327
Blue Rubber Bleb Nevus Syndrome��������������������������������������������������������������� 327
Cutaneovisceral Angiomatosis with Thrombocytopenia ������������������������������� 328
Malignant Masses��������������������������������������������������������������������������������������������������� 329
Hodgkin and Non-Hodgkin Lymphoma������������������������������������������������������������� 329
Appendix��������������������������������������������������������������������������������������������������������������������� 330
Technique��������������������������������������������������������������������������������������������������������������� 330
Patient Positioning ��������������������������������������������������������������������������������������������� 330
Ultrasound Transducer Selection ����������������������������������������������������������������������� 330
Imaging Approaches������������������������������������������������������������������������������������������� 330
Normal Development and Anatomy��������������������������������������������������������������������������� 330
Normal Development��������������������������������������������������������������������������������������������� 330
Normal Anatomy����������������������������������������������������������������������������������������������������� 330
Acute Appendicitis������������������������������������������������������������������������������������������������� 331
Cystic Fibrosis of the Appendix����������������������������������������������������������������������������� 332
Benign Masses of the Appendix����������������������������������������������������������������������������� 333
Mucocele of the Appendix ��������������������������������������������������������������������������������� 333
Malignant Tumors of the Appendix ����������������������������������������������������������������������� 333
Carcinoid������������������������������������������������������������������������������������������������������������� 333
Lymphoma of the Appendix������������������������������������������������������������������������������� 333
Colon��������������������������������������������������������������������������������������������������������������������������� 334
Technique��������������������������������������������������������������������������������������������������������������� 334
Patient Positioning ��������������������������������������������������������������������������������������������� 334
Ultrasound Transducer Selection ����������������������������������������������������������������������� 334
Imaging Approaches������������������������������������������������������������������������������������������� 334
Normal Development and Anatomy��������������������������������������������������������������������������� 334
Normal Development��������������������������������������������������������������������������������������������� 334
Normal Anatomy����������������������������������������������������������������������������������������������������� 335
Congenital Anomalies��������������������������������������������������������������������������������������������� 335
Anorectal Malformations ����������������������������������������������������������������������������������� 335
Colonic Wall Thickening ��������������������������������������������������������������������������������������� 337
Necrotizing Enterocolitis ����������������������������������������������������������������������������������� 337
Ulcerative Colitis ����������������������������������������������������������������������������������������������� 337
Crohn Disease����������������������������������������������������������������������������������������������������� 339
xxii Contents

Infectious Colitis������������������������������������������������������������������������������������������������� 340

Pseudomembranous Colitis��������������������������������������������������������������������������������� 341
Neutropenic Colitis��������������������������������������������������������������������������������������������� 342
Cystic Fibrosis ��������������������������������������������������������������������������������������������������� 343
Hemolytic–Uremic Syndrome ��������������������������������������������������������������������������� 343
Benign Masses ������������������������������������������������������������������������������������������������������� 344
Juvenile Polyp����������������������������������������������������������������������������������������������������� 344
Duplication Cyst������������������������������������������������������������������������������������������������� 345
Malignant Tumors��������������������������������������������������������������������������������������������������� 345
Lymphoma ��������������������������������������������������������������������������������������������������������� 345
Adenocarcinoma������������������������������������������������������������������������������������������������� 345
References������������������������������������������������������������������������������������������������������������������� 347
11 Liver����������������������������������������������������������������������������������������������������������������������������� 355
Jeannie K. Kwon, Maddy Artunduaga, Javier D. Gonzalez,
Alexandra M. Foust, Elisabeth P. Moredock, Süreyya Burcu Görkem,
and Harriet J. Paltiel
Introduction����������������������������������������������������������������������������������������������������������������� 356
Technique������������������������������������������������������������������������������������������������������������������� 356
Patient Positioning ������������������������������������������������������������������������������������������������� 356
Ultrasound Transducer Selection ��������������������������������������������������������������������������� 356
Imaging Approaches����������������������������������������������������������������������������������������������� 356
Grayscale Imaging ������������������������������������������������������������������������������������������������� 356
Doppler Ultrasound������������������������������������������������������������������������������������������������� 357
Contrast-Enhanced Ultrasound������������������������������������������������������������������������������� 358
Elastography����������������������������������������������������������������������������������������������������������� 359
Normal Development and Anatomy��������������������������������������������������������������������������� 360
Normal Development��������������������������������������������������������������������������������������������� 360
Normal Anatomy����������������������������������������������������������������������������������������������������� 363
Segmental and Lobar Anatomy��������������������������������������������������������������������������� 363
Ligaments����������������������������������������������������������������������������������������������������������� 364
Hepatic Circulation��������������������������������������������������������������������������������������������� 366
Normal Hepatic Parenchyma ����������������������������������������������������������������������������� 367
Anatomic Variants����������������������������������������������������������������������������������������������� 367
Congenital Anomalies������������������������������������������������������������������������������������������������� 369
Liver Cyst��������������������������������������������������������������������������������������������������������������� 369
Polycystic Liver Disease����������������������������������������������������������������������������������������� 369
Congenital Portosystemic Shunts��������������������������������������������������������������������������� 370
Diffuse Parenchymal Disease������������������������������������������������������������������������������������� 371
Nonalcoholic Fatty Liver Disease��������������������������������������������������������������������������� 371
Fibrosis������������������������������������������������������������������������������������������������������������������� 372
Hemochromatosis��������������������������������������������������������������������������������������������������� 374
Cirrhosis����������������������������������������������������������������������������������������������������������������� 374
Infection ��������������������������������������������������������������������������������������������������������������������� 375
Viral Hepatitis��������������������������������������������������������������������������������������������������������� 375
Bacterial Infection��������������������������������������������������������������������������������������������������� 375
Fungal Infection ����������������������������������������������������������������������������������������������������� 378
Parasitic Infection��������������������������������������������������������������������������������������������������� 378
Trauma ����������������������������������������������������������������������������������������������������������������������� 380
Blunt Abdominal Trauma��������������������������������������������������������������������������������������� 380
Umbilical Vein Catheterization������������������������������������������������������������������������������� 380
Portal Hypertension ��������������������������������������������������������������������������������������������������� 382
Budd–Chiari Syndrome ��������������������������������������������������������������������������������������������� 383
Sinusoidal Obstruction Syndrome ����������������������������������������������������������������������������� 384
Contents xxiii

Peliosis Hepatis����������������������������������������������������������������������������������������������������������� 385

Passive Venous Congestion����������������������������������������������������������������������������������������� 386
Portal Venous Gas������������������������������������������������������������������������������������������������������� 386
Tumors ����������������������������������������������������������������������������������������������������������������������� 387
Benign Masses ������������������������������������������������������������������������������������������������������� 387
Congenital Hemangioma ����������������������������������������������������������������������������������� 387
Infantile Hemangioma ��������������������������������������������������������������������������������������� 391
Mesenchymal Hamartoma ��������������������������������������������������������������������������������� 392
Focal Nodular Hyperplasia��������������������������������������������������������������������������������� 393
Hepatic Adenoma����������������������������������������������������������������������������������������������� 394
Malignant Tumors��������������������������������������������������������������������������������������������������� 397
Hepatoblastoma��������������������������������������������������������������������������������������������������� 397
Hepatocellular Carcinoma ��������������������������������������������������������������������������������� 400
Fibrolamellar Hepatocellular Carcinoma����������������������������������������������������������� 401
Rare Primary Tumors����������������������������������������������������������������������������������������� 402
Metastases����������������������������������������������������������������������������������������������������������� 403
Lymphoma ��������������������������������������������������������������������������������������������������������� 404
Posttransplant Lymphoproliferative Disorder����������������������������������������������������� 405
Leukemia������������������������������������������������������������������������������������������������������������� 407
Liver Transplantation ������������������������������������������������������������������������������������������������� 408
Introduction������������������������������������������������������������������������������������������������������������� 408
Surgical Technique������������������������������������������������������������������������������������������������� 409
Whole Liver Transplantation ����������������������������������������������������������������������������� 410
Living-Related Donor and Split Liver Grafts����������������������������������������������������� 410
Preoperative and Postoperative Imaging Considerations��������������������������������������� 411
Normal Liver Transplant Ultrasound ��������������������������������������������������������������������� 412
Rejection����������������������������������������������������������������������������������������������������������������� 412
Biliary Complications��������������������������������������������������������������������������������������������� 413
Bile Leak������������������������������������������������������������������������������������������������������������� 413
Biliary Stricture��������������������������������������������������������������������������������������������������� 414
Vascular Complications ����������������������������������������������������������������������������������������� 415
Hepatic Artery Thrombosis��������������������������������������������������������������������������������� 415
Hepatic Artery Stenosis��������������������������������������������������������������������������������������� 415
Hepatic Artery Pseudoaneurysm������������������������������������������������������������������������� 418
Portal Vein Thrombosis��������������������������������������������������������������������������������������� 418
Portal Vein Stenosis�������������������������������������������������������������������������������������������� 419
Hepatic Vein Outflow Obstruction ��������������������������������������������������������������������� 420
Inferior Vena Caval Stenosis and Thrombosis ��������������������������������������������������� 422
Fluid Collections����������������������������������������������������������������������������������������������������� 423
Extrahepatic Collections������������������������������������������������������������������������������������� 423
Intrahepatic Collections ������������������������������������������������������������������������������������� 425
References������������������������������������������������������������������������������������������������������������������� 426
12 Gallbladder and Biliary Tract����������������������������������������������������������������������������������� 433
Christian L. Carlson, Mitchell W. Boehnke, and Harriet J. Paltiel
Gallbladder����������������������������������������������������������������������������������������������������������������� 433
Technique��������������������������������������������������������������������������������������������������������������� 433
Patient Positioning ��������������������������������������������������������������������������������������������� 433
Ultrasound Transducer Selection ����������������������������������������������������������������������� 434
Imaging Approaches������������������������������������������������������������������������������������������� 434
Normal Development and Anatomy����������������������������������������������������������������������� 434
Normal Development����������������������������������������������������������������������������������������� 434
Normal Anatomy������������������������������������������������������������������������������������������������� 434
Anatomic Variants��������������������������������������������������������������������������������������������������� 436
xxiv Contents

Congenital Anomalies��������������������������������������������������������������������������������������������� 436

Agenesis/Hypoplasia������������������������������������������������������������������������������������������� 436
Ectopia ��������������������������������������������������������������������������������������������������������������� 437
Septate Gallbladder��������������������������������������������������������������������������������������������� 438
Duplication��������������������������������������������������������������������������������������������������������� 439
Cholelithiasis���������������������������������������������������������������������������������������������������������� 440
Sludge��������������������������������������������������������������������������������������������������������������������� 442
Cholecystitis����������������������������������������������������������������������������������������������������������� 444
Acute Calculous Cholecystitis ��������������������������������������������������������������������������� 444
Acute Acalculous Cholecystitis ������������������������������������������������������������������������� 447
Biliary Dyskinesia����������������������������������������������������������������������������������������������� 447
Chronic Cholecystitis����������������������������������������������������������������������������������������� 449
Porcelain Gallbladder����������������������������������������������������������������������������������������� 449
Hydrops������������������������������������������������������������������������������������������������������������������� 449
Torsion ������������������������������������������������������������������������������������������������������������������� 450
Polyps��������������������������������������������������������������������������������������������������������������������� 450
Adenomyomatosis����������������������������������������������������������������������������������������������� 451
Cholesterol Polyps ��������������������������������������������������������������������������������������������� 451
Inflammatory Polyps������������������������������������������������������������������������������������������� 452
Other Polypoid Lesions��������������������������������������������������������������������������������������� 452
Other Disorders������������������������������������������������������������������������������������������������������� 453
Gallbladder Varices��������������������������������������������������������������������������������������������� 453
Trauma ��������������������������������������������������������������������������������������������������������������� 453
Biliary Tract ��������������������������������������������������������������������������������������������������������������� 455
Technique��������������������������������������������������������������������������������������������������������������� 455
Normal Development and Anatomy����������������������������������������������������������������������� 455
Normal Development����������������������������������������������������������������������������������������� 455
Normal Anatomy������������������������������������������������������������������������������������������������� 455
Anatomic Variants��������������������������������������������������������������������������������������������������� 456
Congenital Anomalies��������������������������������������������������������������������������������������������� 457
Choledochal Cysts ��������������������������������������������������������������������������������������������� 457
Caroli Disease����������������������������������������������������������������������������������������������������� 459
Biliary Tract Obstruction ��������������������������������������������������������������������������������������� 462
Biliary Atresia����������������������������������������������������������������������������������������������������� 462
Neonatal Hepatitis Syndrome����������������������������������������������������������������������������� 463
Alagille Syndrome ��������������������������������������������������������������������������������������������� 465
Byler Disease ����������������������������������������������������������������������������������������������������� 469
Choledocholithiasis��������������������������������������������������������������������������������������������� 469
Inspissated Bile Syndrome��������������������������������������������������������������������������������� 470
Sclerosing Cholangitis ��������������������������������������������������������������������������������������� 471
Mirizzi Syndrome����������������������������������������������������������������������������������������������� 471
Bile Duct Stricture ��������������������������������������������������������������������������������������������� 472
AIDS Cholangiopathy����������������������������������������������������������������������������������������� 472
Spontaneous Perforation of the Extrahepatic Bile Ducts��������������������������������������� 472
Biliary Tract Trauma����������������������������������������������������������������������������������������������� 472
Tumors ������������������������������������������������������������������������������������������������������������������� 473
Benign Masses ��������������������������������������������������������������������������������������������������� 473
Granular Cell Tumor��������������������������������������������������������������������������������������� 473
Bile Duct Adenoma����������������������������������������������������������������������������������������� 473
Malignant Bile Duct Tumors ����������������������������������������������������������������������������� 473
Rhabdomyosarcoma of the Bile Duct������������������������������������������������������������� 473
Cholangiocarcinoma��������������������������������������������������������������������������������������� 474
Neuroendocrine Tumor����������������������������������������������������������������������������������� 474
Metastases������������������������������������������������������������������������������������������������������� 474
Contents xxv

References������������������������������������������������������������������������������������������������������������������� 475
13 Spleen and Peritoneal Cavity������������������������������������������������������������������������������������� 481
Patrick Duffy, Ilse Castro-Aragon, Patrick Tivnan, Frank M. Volberg,
Ella Kipervasser, Zoltan Harkanyi, and Harriet J. Paltiel
Spleen������������������������������������������������������������������������������������������������������������������������� 482
Introduction������������������������������������������������������������������������������������������������������������� 482
Technique��������������������������������������������������������������������������������������������������������������� 482
Patient Positioning ��������������������������������������������������������������������������������������������� 482
Ultrasound Transducer Selection ����������������������������������������������������������������������� 482
Imaging Approaches������������������������������������������������������������������������������������������� 482
Normal Development and Anatomy����������������������������������������������������������������������� 482
Normal Development����������������������������������������������������������������������������������������� 482
Normal Anatomy������������������������������������������������������������������������������������������������� 482
Anatomic Variants��������������������������������������������������������������������������������������������������� 487
Lobulations and Clefts ��������������������������������������������������������������������������������������� 487
Accessory Spleen ����������������������������������������������������������������������������������������������� 487
Congenital Anomalies��������������������������������������������������������������������������������������������� 490
Wandering Spleen����������������������������������������������������������������������������������������������� 490
Polysplenia, Hyposplenia, and Asplenia������������������������������������������������������������� 491
Nonparasitic Splenic Cysts��������������������������������������������������������������������������������� 493
Splenogonadal Fusion����������������������������������������������������������������������������������������� 493
Splenopancreatic Fusion������������������������������������������������������������������������������������� 493
Infection ����������������������������������������������������������������������������������������������������������������� 494
Pyogenic Abscess����������������������������������������������������������������������������������������������� 494
Fungal Abscess��������������������������������������������������������������������������������������������������� 494
Tuberculous Infection����������������������������������������������������������������������������������������� 497
Epstein-Barr Viral Infection������������������������������������������������������������������������������� 498
Parasitic Infection����������������������������������������������������������������������������������������������� 498
Acquired Immunodeficiency Syndrome������������������������������������������������������������� 501
Inflammatory Disorders ����������������������������������������������������������������������������������������� 502
Sarcoidosis ��������������������������������������������������������������������������������������������������������� 502
Rheumatic Disorders������������������������������������������������������������������������������������������� 503
Granulomatosis with Polyangiitis����������������������������������������������������������������������� 504
Hemoglobinopathies����������������������������������������������������������������������������������������������� 504
Lysosomal Storage Diseases����������������������������������������������������������������������������������� 506
Portal Hypertension ����������������������������������������������������������������������������������������������� 508
Trauma ������������������������������������������������������������������������������������������������������������������� 508
Splenosis������������������������������������������������������������������������������������������������������������� 511
Vascular Anomalies������������������������������������������������������������������������������������������������� 511
Lymphatic Malformation ����������������������������������������������������������������������������������� 511
Venous Malformation����������������������������������������������������������������������������������������� 513
Kaposiform Lymphangiomatosis ����������������������������������������������������������������������� 513
Peliosis ������������������������������������������������������������������������������������������������������������������� 515
Benign Masses ������������������������������������������������������������������������������������������������������� 516
Hamartoma��������������������������������������������������������������������������������������������������������� 516
Extramedullary Hematopoiesis��������������������������������������������������������������������������� 516
Inflammatory Myofibroblastic Tumor����������������������������������������������������������������� 516
Hemangioma������������������������������������������������������������������������������������������������������� 517
Littoral Cell Angioma����������������������������������������������������������������������������������������� 517
Malignant Tumors��������������������������������������������������������������������������������������������������� 518
Lymphoma ��������������������������������������������������������������������������������������������������������� 518
Leukemia������������������������������������������������������������������������������������������������������������� 519
Angiosarcoma����������������������������������������������������������������������������������������������������� 520
xxvi Contents

Kaposiform Hemangioendothelioma ����������������������������������������������������������������� 521

Metastatic Disease ��������������������������������������������������������������������������������������������� 521
Neuroblastoma ����������������������������������������������������������������������������������������������� 521
Langerhans Cell Histiocytosis ������������������������������������������������������������������������������� 521
Complications of Pancreatitis��������������������������������������������������������������������������������� 522
Gamna-Gandy Bodies��������������������������������������������������������������������������������������������� 523
Peritoneal Cavity��������������������������������������������������������������������������������������������������������� 524
Introduction������������������������������������������������������������������������������������������������������������� 524
Technique��������������������������������������������������������������������������������������������������������������� 524
Patient Positioning ��������������������������������������������������������������������������������������������� 524
Ultrasound Transducer Selection ����������������������������������������������������������������������� 524
Imaging Approaches������������������������������������������������������������������������������������������� 524
Normal Development and Anatomy����������������������������������������������������������������������� 524
Normal Development����������������������������������������������������������������������������������������� 524
Normal Anatomy������������������������������������������������������������������������������������������������� 524
Omentum������������������������������������������������������������������������������������������������������������� 526
Mesentery����������������������������������������������������������������������������������������������������������� 526
Peritoneal Fluid��������������������������������������������������������������������������������������������������� 528
Normal Flow of Peritoneal Fluid ������������������������������������������������������������������� 528
Ascites��������������������������������������������������������������������������������������������������������������������� 528
Hemoperitoneum������������������������������������������������������������������������������������������������� 530
Chylous Ascites��������������������������������������������������������������������������������������������������� 531
Urine Ascites������������������������������������������������������������������������������������������������������� 532
Localized Peritoneal Fluid Collections������������������������������������������������������������������� 532
Cerebrospinal Fluid Pseudocyst������������������������������������������������������������������������� 532
Biloma����������������������������������������������������������������������������������������������������������������� 533
Pancreatic Pseudocyst����������������������������������������������������������������������������������������� 534
Peritoneal Inclusion Cyst ����������������������������������������������������������������������������������� 535
Diaphragmatic Mesothelial Cyst������������������������������������������������������������������������� 535
Peritoneal Inflammation����������������������������������������������������������������������������������������� 535
Infective Peritonitis��������������������������������������������������������������������������������������������� 535
Tuberculous Peritonitis����������������������������������������������������������������������������������� 536
Chemical Peritonitis������������������������������������������������������������������������������������������� 537
Granulomatous Peritonitis ��������������������������������������������������������������������������������� 537
Sclerosing Encapsulating Peritonitis������������������������������������������������������������������� 537
Abscess��������������������������������������������������������������������������������������������������������������� 539
Pneumoperitoneum������������������������������������������������������������������������������������������������� 540
Omental Cyst ��������������������������������������������������������������������������������������������������������� 540
Segmental Omental Infarction ������������������������������������������������������������������������������� 540
Mesenteric Lymphadenitis ������������������������������������������������������������������������������������� 541
Vascular Malformations ����������������������������������������������������������������������������������������� 542
Lymphatic Malformation ����������������������������������������������������������������������������������� 542
Venous Malformation����������������������������������������������������������������������������������������� 542
Benign Masses ������������������������������������������������������������������������������������������������������� 545
Infantile Hemangioma ��������������������������������������������������������������������������������������� 545
Lipoma ��������������������������������������������������������������������������������������������������������������� 546
Lipomatosis��������������������������������������������������������������������������������������������������������� 546
Lipoblastoma/Lipoblastomatosis ����������������������������������������������������������������������� 547
Neurofibroma ����������������������������������������������������������������������������������������������������� 547
Plexiform Neurofibroma��������������������������������������������������������������������������������� 547
Desmoid Tumor ������������������������������������������������������������������������������������������������� 548
Castleman Disease ��������������������������������������������������������������������������������������������� 549
Inflammatory Myofibroblastic Tumor����������������������������������������������������������������� 550
Contents xxvii

Malignant Tumors��������������������������������������������������������������������������������������������������� 551

Primary Tumors ������������������������������������������������������������������������������������������������� 551
Lymphoma ����������������������������������������������������������������������������������������������������� 551
Rhabdomyosarcoma��������������������������������������������������������������������������������������� 551
Desmoplastic Small Round Cell Tumor��������������������������������������������������������� 553
Malignant Mesothelioma ������������������������������������������������������������������������������� 554
Metastatic Disease ��������������������������������������������������������������������������������������������� 555
Neuroblastoma ����������������������������������������������������������������������������������������������� 555
Adenocarcinoma��������������������������������������������������������������������������������������������� 555
Wilms’ Tumor������������������������������������������������������������������������������������������������� 555
Germ Cell Tumor ������������������������������������������������������������������������������������������� 555
Intracranial Neoplasms����������������������������������������������������������������������������������� 555
References������������������������������������������������������������������������������������������������������������������� 555
14 Pancreas, Adrenal Glands, and Retroperitoneum��������������������������������������������������� 563
Anastasia L. Hryhorczuk and Harriet J. Paltiel
Introduction����������������������������������������������������������������������������������������������������������������� 563
Pancreas ��������������������������������������������������������������������������������������������������������������������� 564
Technique��������������������������������������������������������������������������������������������������������������� 564
Patient Positioning ��������������������������������������������������������������������������������������������� 564
Ultrasound Transducer Selection ����������������������������������������������������������������������� 564
Imaging Approaches������������������������������������������������������������������������������������������� 564
Normal Development and Anatomy����������������������������������������������������������������������� 564
Normal Development����������������������������������������������������������������������������������������� 564
Normal Anatomy������������������������������������������������������������������������������������������������� 564
Anatomic Variants��������������������������������������������������������������������������������������������������� 566
Lobulated Parenchymal Contour ����������������������������������������������������������������������� 566
Congenital Anomalies��������������������������������������������������������������������������������������������� 566
Pancreas Divisum����������������������������������������������������������������������������������������������� 566
Annular Pancreas ����������������������������������������������������������������������������������������������� 566
Common Pancreaticobiliary Channel����������������������������������������������������������������� 567
Partial Pancreatic Agenesis��������������������������������������������������������������������������������� 567
Accessory Pancreatic Lobe��������������������������������������������������������������������������������� 567
Ectopic Pancreas������������������������������������������������������������������������������������������������� 569
Congenital Hyperinsulinism������������������������������������������������������������������������������� 569
Congenital Pancreatic Cyst��������������������������������������������������������������������������������� 569
Genetic Disorders with Associated Pancreatic Abnormalities������������������������������� 570
Cystic Fibrosis ��������������������������������������������������������������������������������������������������� 570
Shwachman-Diamond Syndrome����������������������������������������������������������������������� 570
Beckwith-Wiedemann Syndrome����������������������������������������������������������������������� 571
Autosomal Dominant Polycystic Kidney Disease ��������������������������������������������� 571
Von Hippel-Lindau Disease ������������������������������������������������������������������������������� 571
Acute Pancreatitis��������������������������������������������������������������������������������������������������� 572
Acute Peripancreatic Fluid Collections ������������������������������������������������������������� 573
Pseudocysts��������������������������������������������������������������������������������������������������������� 573
Pancreaticopleural Fistula����������������������������������������������������������������������������������� 575
Necrotizing Pancreatitis ������������������������������������������������������������������������������������� 575
Vascular Complications ������������������������������������������������������������������������������������� 576
Acute Recurrent and Chronic Pancreatitis ������������������������������������������������������������� 576
Trauma ������������������������������������������������������������������������������������������������������������������� 577
Pancreatic Venous and Lymphatic Malformations������������������������������������������������� 579
Benign Pancreatic Neoplasms��������������������������������������������������������������������������������� 579
Serous Cystadenoma������������������������������������������������������������������������������������������� 579
xxviii Contents

Mucinous Cystadenoma������������������������������������������������������������������������������������� 579

Infantile Hemangioma ��������������������������������������������������������������������������������������� 579
Cystic Teratoma ������������������������������������������������������������������������������������������������� 579
Lipoma ��������������������������������������������������������������������������������������������������������������� 580
Inflammatory Myofibroblastic Tumor����������������������������������������������������������������� 580
Leiomyoma, Neurofibroma, and Schwannoma ������������������������������������������������� 580
Malignant Pancreatic Tumors��������������������������������������������������������������������������������� 580
Pancreatoblastoma ��������������������������������������������������������������������������������������������� 580
Solid Pseudopapillary Tumor����������������������������������������������������������������������������� 582
Islet Cell Tumor ������������������������������������������������������������������������������������������������� 582
Acinar Cell Carcinoma��������������������������������������������������������������������������������������� 584
Ductal Adenocarcinoma������������������������������������������������������������������������������������� 585
Lymphoma ��������������������������������������������������������������������������������������������������������� 585
Neuroblastoma ��������������������������������������������������������������������������������������������������� 585
Primitive Neuroectodermal Tumor��������������������������������������������������������������������� 586
Kaposiform Hemangioendothelioma ����������������������������������������������������������������� 586
Rhabdomyosarcoma������������������������������������������������������������������������������������������� 587
Fibrosarcoma������������������������������������������������������������������������������������������������������� 587
Metastatic Disease ��������������������������������������������������������������������������������������������� 587
Pancreatic Transplantation ����������������������������������������������������������������������������������������� 588
Adrenal Glands����������������������������������������������������������������������������������������������������������� 588
Technique��������������������������������������������������������������������������������������������������������������� 588
Patient Positioning ��������������������������������������������������������������������������������������������� 588
Ultrasound Transducer Selection ����������������������������������������������������������������������� 588
Imaging Approaches������������������������������������������������������������������������������������������� 588
Normal Development and Anatomy����������������������������������������������������������������������� 589
Normal Development����������������������������������������������������������������������������������������� 589
Normal Anatomy������������������������������������������������������������������������������������������������� 589
Anatomic Variants��������������������������������������������������������������������������������������������������� 590
Discoid Adrenal Gland��������������������������������������������������������������������������������������� 590
Congenital Anomalies��������������������������������������������������������������������������������������������� 591
Congenital Adrenal Agenesis����������������������������������������������������������������������������� 591
Fusion Abnormalities ��������������������������������������������������������������������������������������������� 591
Circumrenal Adrenal Gland ������������������������������������������������������������������������������� 591
Horseshoe Adrenal Gland����������������������������������������������������������������������������������� 592
Adrenal Rests ��������������������������������������������������������������������������������������������������������� 592
Adrenal Heterotopia����������������������������������������������������������������������������������������������� 593
Genetic Disorders��������������������������������������������������������������������������������������������������� 593
Congenital Adrenal Hyperplasia������������������������������������������������������������������������� 593
Congenital Lipoid Adrenal Hyperplasia������������������������������������������������������������� 593
Wolman Disease������������������������������������������������������������������������������������������������� 594
Adrenal Cyst����������������������������������������������������������������������������������������������������������� 594
Idiopathic Adrenal Cyst ������������������������������������������������������������������������������������� 594
Infection ����������������������������������������������������������������������������������������������������������������� 595
Congenital Herpes Simplex Infection����������������������������������������������������������������� 595
Granulomatous Infection ����������������������������������������������������������������������������������� 595
Xanthogranulomatous Adrenalitis ��������������������������������������������������������������������� 595
Abscess��������������������������������������������������������������������������������������������������������������� 595
Adrenal Hemorrhage����������������������������������������������������������������������������������������������� 596
Adrenocortical Tumors������������������������������������������������������������������������������������������� 597
Adrenocortical Adenoma and Carcinoma����������������������������������������������������������� 597
Neural Crest Tumors����������������������������������������������������������������������������������������������� 598
Ganglioneuroma������������������������������������������������������������������������������������������������� 598
Contents xxix

Ganglioneuroblastoma ��������������������������������������������������������������������������������������� 599

Neuroblastoma ��������������������������������������������������������������������������������������������������� 601
Pheochromocytoma������������������������������������������������������������������������������������������������� 606
Myelolipoma����������������������������������������������������������������������������������������������������������� 607
Hemangioma����������������������������������������������������������������������������������������������������������� 608
Teratoma����������������������������������������������������������������������������������������������������������������� 608
Leiomyoma������������������������������������������������������������������������������������������������������������� 609
Lymphoma ������������������������������������������������������������������������������������������������������������� 609
Metastases��������������������������������������������������������������������������������������������������������������� 609
Nonvascular Disorders of the Retroperitoneum��������������������������������������������������������� 609
Technique��������������������������������������������������������������������������������������������������������������� 609
Patient Positioning ��������������������������������������������������������������������������������������������� 609
Ultrasound Transducer Selection ����������������������������������������������������������������������� 609
Imaging Approaches������������������������������������������������������������������������������������������� 609
Normal Development and Anatomy����������������������������������������������������������������������� 609
Normal Development����������������������������������������������������������������������������������������� 609
Normal Anatomy������������������������������������������������������������������������������������������������� 610
Infection and Abscess��������������������������������������������������������������������������������������������� 611
Hemorrhage������������������������������������������������������������������������������������������������������������� 613
Fibrosis������������������������������������������������������������������������������������������������������������������� 613
Extramedullary Hematopoiesis������������������������������������������������������������������������������� 614
Venous and Lymphatic Malformations������������������������������������������������������������������� 614
Lymphadenopathy��������������������������������������������������������������������������������������������������� 615
Infection ������������������������������������������������������������������������������������������������������������� 615
Lymphoma ��������������������������������������������������������������������������������������������������������� 615
Metastatic Disease ��������������������������������������������������������������������������������������������� 616
Benign Masses ������������������������������������������������������������������������������������������������������� 617
Hemangioma������������������������������������������������������������������������������������������������������� 617
Mature Teratoma������������������������������������������������������������������������������������������������� 617
Retroperitoneal Lipoma and Lipoblastoma ����������������������������������������������������������� 617
Neurofibroma and Schwannoma����������������������������������������������������������������������������� 617
Neural Crest Tumors: Ganglioneuroma, Ganglioneuroblastoma,
and Neuroblastoma������������������������������������������������������������������������������������������������� 617
Malignant Tumors��������������������������������������������������������������������������������������������������� 620
Rhabdomyosarcoma������������������������������������������������������������������������������������������� 620
Infantile Fibrosarcoma ��������������������������������������������������������������������������������������� 620
Malignant Germ Cell Tumor/Immature Teratoma ��������������������������������������������� 620
Smooth Muscle Tumors ����������������������������������������������������������������������������������������� 621
Undifferentiated Pleomorphic Sarcoma (Malignant Fibrous Histiocytoma)��������� 621
Ewing Sarcoma������������������������������������������������������������������������������������������������������� 621
Inflammatory Myofibroblastic Tumor��������������������������������������������������������������������� 622
Kaposiform Hemangioendothelioma ��������������������������������������������������������������������� 622
References������������������������������������������������������������������������������������������������������������������� 622
15 Male Genital Tract ����������������������������������������������������������������������������������������������������� 629
Judy H. Squires and Harriet J. Paltiel
Introduction����������������������������������������������������������������������������������������������������������������� 629
Scrotum������������������������������������������������������������������������������������������������������������������� 629
Technique��������������������������������������������������������������������������������������������������������������� 629
Patient Positioning ��������������������������������������������������������������������������������������������� 629
Ultrasound Transducer Selection ����������������������������������������������������������������������� 629
Imaging Approaches������������������������������������������������������������������������������������������� 630
Normal Development and Anatomy����������������������������������������������������������������������� 630
xxx Contents

Normal Development����������������������������������������������������������������������������������������� 630

Normal Anatomy������������������������������������������������������������������������������������������������� 631
Scrotum����������������������������������������������������������������������������������������������������������� 631
Testes��������������������������������������������������������������������������������������������������������������� 631
Epididymis ����������������������������������������������������������������������������������������������������� 632
Spermatic Cord����������������������������������������������������������������������������������������������� 632
Testicular Appendages ����������������������������������������������������������������������������������� 633
Blood Supply ������������������������������������������������������������������������������������������������� 633
Anatomical Variants ����������������������������������������������������������������������������������������������� 635
Testicular Appendages ��������������������������������������������������������������������������������������� 635
Vessels����������������������������������������������������������������������������������������������������������������� 635
Congenital Anomalies��������������������������������������������������������������������������������������������� 636
Testicular Agenesis��������������������������������������������������������������������������������������������� 636
Cryptorchidism��������������������������������������������������������������������������������������������������� 636
Anorchidism������������������������������������������������������������������������������������������������������� 636
Testicular Regression Syndrome������������������������������������������������������������������������� 636
Testicular Hypoplasia����������������������������������������������������������������������������������������� 637
Polyorchidism����������������������������������������������������������������������������������������������������� 637
Testicular Ectopia����������������������������������������������������������������������������������������������� 637
Cystic Dysplasia of the Rete Testis��������������������������������������������������������������������� 638
Splenogonadal Fusion����������������������������������������������������������������������������������������� 638
Bell Clapper Deformity��������������������������������������������������������������������������������������� 639
Acute Scrotal Pain��������������������������������������������������������������������������������������������������� 639
Testicular Torsion����������������������������������������������������������������������������������������������� 639
Segmental Testicular Infarction ������������������������������������������������������������������������� 641
Arterial Segmental Testicular Infarction��������������������������������������������������������� 642
Venous Testicular Infarction��������������������������������������������������������������������������� 642
Torsion of Testicular Appendages����������������������������������������������������������������������� 643
Inflammatory Disorders ������������������������������������������������������������������������������������� 644
Acute Epididymitis and Epididymo-orchitis ������������������������������������������������� 644
Isolated Orchitis ��������������������������������������������������������������������������������������������� 644
Testicular Abscess������������������������������������������������������������������������������������������� 645
Epididymal Abscess ��������������������������������������������������������������������������������������� 645
Scrotal Abscess����������������������������������������������������������������������������������������������� 645
Henoch-Schönlein Purpura����������������������������������������������������������������������������� 646
Idiopathic Scrotal Edema������������������������������������������������������������������������������� 646
Fournier Gangrene ����������������������������������������������������������������������������������������� 649
Chronic Epididymitis ��������������������������������������������������������������������������������������������� 649
Dancing Megasperm����������������������������������������������������������������������������������������������� 649
Scrotal and Spermatic Cord Fluid Collections������������������������������������������������������� 649
Scrotal Hydrocele����������������������������������������������������������������������������������������������� 649
Spermatic Cord Hydrocele��������������������������������������������������������������������������������� 650
Abdominoscrotal Hydrocele������������������������������������������������������������������������������� 651
Scrotal Hematocele��������������������������������������������������������������������������������������������� 651
Scrotal Lymphocele ������������������������������������������������������������������������������������������� 652
Scrotal Calcification������������������������������������������������������������������������������������������� 652
Testicular Microlithiasis��������������������������������������������������������������������������������� 652
Loose Bodies��������������������������������������������������������������������������������������������������� 653
Meconium Peritonitis������������������������������������������������������������������������������������� 653
Trauma ������������������������������������������������������������������������������������������������������������������� 653
Blunt Scrotal Trauma ����������������������������������������������������������������������������������������� 653
Testicular Hematoma ������������������������������������������������������������������������������������� 654
Testicular Fracture ����������������������������������������������������������������������������������������� 655
Contents xxxi

Testicular Rupture������������������������������������������������������������������������������������������� 655

Scrotal Hematocele����������������������������������������������������������������������������������������� 656
Penetrating Scrotal Trauma��������������������������������������������������������������������������������� 656
Foreign Body ������������������������������������������������������������������������������������������������� 656
Scrotal Urinoma ��������������������������������������������������������������������������������������������� 657
Repetitive Scrotal Microtrauma ��������������������������������������������������������������������� 657
Inguinal Hernia������������������������������������������������������������������������������������������������������� 657
Indirect Inguinal Hernia������������������������������������������������������������������������������������� 657
Direct Inguinal Hernia ��������������������������������������������������������������������������������������� 658
Varicocele��������������������������������������������������������������������������������������������������������������� 659
Intratesticular Varicocele������������������������������������������������������������������������������������� 660
Testicular Masses ��������������������������������������������������������������������������������������������������� 660
Non-Neoplastic Lesions������������������������������������������������������������������������������������� 660
Adrenal Rests ������������������������������������������������������������������������������������������������� 660
Leydig Cell Hyperplasia��������������������������������������������������������������������������������� 661
Hamartoma����������������������������������������������������������������������������������������������������� 661
Simple Cyst����������������������������������������������������������������������������������������������������� 661
Sinus Histiocytosis (Rosai-Dorfman-Destombes Disease)����������������������������� 662
Primary Testicular Tumors������������������������������������������������������������������������������������� 662
Germ Cell Tumors����������������������������������������������������������������������������������������������� 662
Yolk Sac Tumor����������������������������������������������������������������������������������������������� 662
Teratoma��������������������������������������������������������������������������������������������������������� 663
Seminoma������������������������������������������������������������������������������������������������������� 663
Gonadoblastoma��������������������������������������������������������������������������������������������� 663
Embryonal Carcinoma ����������������������������������������������������������������������������������� 663
Teratocarcinoma��������������������������������������������������������������������������������������������� 664
Choriocarcinoma��������������������������������������������������������������������������������������������� 664
Stromal Tumors��������������������������������������������������������������������������������������������������� 664
Leydig Cell Tumor ����������������������������������������������������������������������������������������� 665
Sertoli Cell Tumor������������������������������������������������������������������������������������������� 665
Granulosa Cell Tumor������������������������������������������������������������������������������������� 665
Other Testicular Tumors������������������������������������������������������������������������������������� 666
Epidermoid Cyst��������������������������������������������������������������������������������������������� 666
Dermoid Cyst ������������������������������������������������������������������������������������������������� 666
Fibroma����������������������������������������������������������������������������������������������������������� 666
Neurofibroma ������������������������������������������������������������������������������������������������� 666
Lipoma ����������������������������������������������������������������������������������������������������������� 667
Hemangioma��������������������������������������������������������������������������������������������������� 667
Leiomyoma����������������������������������������������������������������������������������������������������� 668
Adenomatoid Tumor��������������������������������������������������������������������������������������� 668
Follicular Lymphoma������������������������������������������������������������������������������������� 668
Secondary Testicular Tumors��������������������������������������������������������������������������������� 668
Leukemia and Lymphoma����������������������������������������������������������������������������������� 668
Neuroblastoma ��������������������������������������������������������������������������������������������������� 668
Wilms’ Tumor����������������������������������������������������������������������������������������������������� 669
Langerhans Cell Histiocytosis ��������������������������������������������������������������������������� 669
Carcinoid Tumor������������������������������������������������������������������������������������������������� 669
Rhabdomyosarcoma������������������������������������������������������������������������������������������� 669
Retinoblastoma��������������������������������������������������������������������������������������������������� 669
Paratesticular Masses ��������������������������������������������������������������������������������������������� 669
Non-Neoplastic Lesions������������������������������������������������������������������������������������� 669
Spermatocele��������������������������������������������������������������������������������������������������� 669
Epididymal Cyst��������������������������������������������������������������������������������������������� 669
xxxii Contents

Fibrous Pseudotumor ������������������������������������������������������������������������������������� 670

Fibrous Hamartoma of Infancy����������������������������������������������������������������������� 670
Juvenile Xanthogranuloma����������������������������������������������������������������������������� 670
Spermatic Granuloma������������������������������������������������������������������������������������� 670
Cystic Dysplasia of Epididymis��������������������������������������������������������������������� 670
Ectopic Adrenal Rest��������������������������������������������������������������������������������������� 670
Vascular Anomalies��������������������������������������������������������������������������������������������� 670
Lymphatic Malformation ������������������������������������������������������������������������������� 670
Venous Malformation������������������������������������������������������������������������������������� 671
Arteriovenous Malformation��������������������������������������������������������������������������� 671
Benign Tumors��������������������������������������������������������������������������������������������������� 671
Lipoma ����������������������������������������������������������������������������������������������������������� 671
Adenomatoid Tumor��������������������������������������������������������������������������������������� 672
Leiomyoma����������������������������������������������������������������������������������������������������� 672
Dermoid ��������������������������������������������������������������������������������������������������������� 672
Neurofibroma ������������������������������������������������������������������������������������������������� 672
Papillary Cystadenoma����������������������������������������������������������������������������������� 672
Malignant Tumors����������������������������������������������������������������������������������������������� 673
Primary Tumors ��������������������������������������������������������������������������������������������� 673
Secondary Tumors ����������������������������������������������������������������������������������������� 673
Prostate and Seminal Vesicles������������������������������������������������������������������������������������� 674
Introduction������������������������������������������������������������������������������������������������������������� 674
Technique��������������������������������������������������������������������������������������������������������������� 674
Patient Positioning ��������������������������������������������������������������������������������������������� 674
Ultrasound Transducer Selection ����������������������������������������������������������������������� 674
Imaging Approaches������������������������������������������������������������������������������������������� 674
Normal Development and Anatomy����������������������������������������������������������������������� 674
Normal Development����������������������������������������������������������������������������������������� 674
Normal Anatomy������������������������������������������������������������������������������������������������� 674
Prostate Gland������������������������������������������������������������������������������������������������� 674
Seminal Vesicles��������������������������������������������������������������������������������������������� 675
Congenital Anomalies��������������������������������������������������������������������������������������������� 675
Enlarged Prostatic Utricle and Prostatic Utricle Cyst����������������������������������������� 675
Müllerian Duct Cyst������������������������������������������������������������������������������������������� 675
Seminal Vesicle Cyst������������������������������������������������������������������������������������������� 675
Seminal Vesicle Agenesis or Hypoplasia ����������������������������������������������������������� 677
Inflammatory Disorders ����������������������������������������������������������������������������������������� 678
Prostatitis������������������������������������������������������������������������������������������������������������ 678
Prostatic Abscess��������������������������������������������������������������������������������������������� 678
Seminal Vesiculitis ����������������������������������������������������������������������������������������� 678
Tumors of the Prostate and Seminal Vesicle������������������������������������������������������� 678
Rhabdomyosarcoma��������������������������������������������������������������������������������������� 678
Leukemia��������������������������������������������������������������������������������������������������������� 678
Seminal Vesicle Tumors��������������������������������������������������������������������������������� 678
References������������������������������������������������������������������������������������������������������������������� 678
16 Female Genital Tract ������������������������������������������������������������������������������������������������� 683
Erica L. Riedesel and Harriet J. Paltiel
Introduction����������������������������������������������������������������������������������������������������������������� 683
Technique: Patient Positioning, Transducer Selection, and Imaging Approaches����� 683
Transabdominal Ultrasound����������������������������������������������������������������������������������� 683
Transvaginal Ultrasound����������������������������������������������������������������������������������������� 684
Three-Dimensional Ultrasound������������������������������������������������������������������������������� 686
Contents xxxiii

Transperineal Ultrasound��������������������������������������������������������������������������������������� 686

Normal Development and Anatomy��������������������������������������������������������������������������� 686
Normal Development��������������������������������������������������������������������������������������������� 686
Gonads and Reproductive Tract����������������������������������������������������������������������������� 686
External Genitalia��������������������������������������������������������������������������������������������������� 688
Normal Anatomy����������������������������������������������������������������������������������������������������� 688
Ovary and Fallopian Tube��������������������������������������������������������������������������������������� 688
Uterus and Cervix��������������������������������������������������������������������������������������������������� 689
Vagina��������������������������������������������������������������������������������������������������������������������� 689
Ovarian and Uterine Changes Associated with the Menstrual Cycle��������������������� 690
Anatomic Variants������������������������������������������������������������������������������������������������������� 692
Arcuate Uterus ������������������������������������������������������������������������������������������������������� 692
Congenital Anomalies������������������������������������������������������������������������������������������������� 692
Müllerian Duct Anomalies������������������������������������������������������������������������������������� 692
Müllerian Agenesis��������������������������������������������������������������������������������������������� 692
Uterine Agenesis��������������������������������������������������������������������������������������������� 693
Mayer-Rokitansky-Küster-Hauser Syndrome (MRKH)��������������������������������� 693
Disorders of Lateral Fusion ������������������������������������������������������������������������������� 694
Septate Uterus������������������������������������������������������������������������������������������������� 694
Bicornuate Uterus������������������������������������������������������������������������������������������� 694
Uterus Didelphys ������������������������������������������������������������������������������������������� 696
Unicornuate Uterus����������������������������������������������������������������������������������������� 696
Disorders of Vertical Fusion������������������������������������������������������������������������������� 696
Imperforate Hymen����������������������������������������������������������������������������������������� 696
Transverse Vaginal Septum����������������������������������������������������������������������������� 698
Atresia of Cervix or Vagina���������������������������������������������������������������������������� 698
OHVIRA Syndrome��������������������������������������������������������������������������������������� 700
Disorders of Sex Development����������������������������������������������������������������������������������� 701
Sex Chromosome Disorders of Sex Development������������������������������������������������� 701
45,X (Turner Syndrome)������������������������������������������������������������������������������������� 701
46,XX Disorders of Sex Development��������������������������������������������������������������� 701
Congenital Adrenal Hyperplasia����������������������������������������������������������������������������� 702
Cloacal Malformation��������������������������������������������������������������������������������������������� 702
Adnexal Masses ��������������������������������������������������������������������������������������������������������� 703
Ovarian Masses������������������������������������������������������������������������������������������������������� 703
Functional Cyst��������������������������������������������������������������������������������������������������� 703
Endometrioma����������������������������������������������������������������������������������������������������� 705
Germ Cell Tumors����������������������������������������������������������������������������������������������� 705
Teratoma��������������������������������������������������������������������������������������������������������� 706
Gonadoblastoma��������������������������������������������������������������������������������������������� 706
Dysgerminoma����������������������������������������������������������������������������������������������� 707
Yolk Sac Tumor����������������������������������������������������������������������������������������������� 708
Choriocarcinoma��������������������������������������������������������������������������������������������� 708
Mixed Germ Cell Tumor��������������������������������������������������������������������������������� 709
Epithelial Tumors����������������������������������������������������������������������������������������������� 709
Cystadenoma��������������������������������������������������������������������������������������������������� 709
Borderline Epithelial Tumor and Cystadenocarcinoma��������������������������������� 710
Stromal Tumors��������������������������������������������������������������������������������������������������� 710
Thecoma-Fibroma������������������������������������������������������������������������������������������� 710
Juvenile Granulosa Cell Tumor���������������������������������������������������������������������� 710
Sertoli-Leydig Cell Tumor ����������������������������������������������������������������������������� 711
Secondary Tumors ��������������������������������������������������������������������������������������������� 711
Paraovarian Cyst����������������������������������������������������������������������������������������������������� 711
xxxiv Contents

Peritoneal Inclusion Cyst ��������������������������������������������������������������������������������������� 712

Adnexal Torsion ����������������������������������������������������������������������������������������������������� 713
Isolated Tubal Torsion����������������������������������������������������������������������������������������� 713
Massive Edema of the Ovary ����������������������������������������������������������������������������� 714
Ectopic Pregnancy������������������������������������������������������������������������������������������������������� 715
Pelvic Inflammatory Disease ������������������������������������������������������������������������������������� 716
Uterine Masses����������������������������������������������������������������������������������������������������������� 717
Benign Masses ������������������������������������������������������������������������������������������������������� 717
Leiomyoma (Fibroid)����������������������������������������������������������������������������������������� 717
Adenomyosis������������������������������������������������������������������������������������������������������� 718
Malignant Tumors��������������������������������������������������������������������������������������������������� 719
Lymphoma ��������������������������������������������������������������������������������������������������������� 719
Cervical Masses ��������������������������������������������������������������������������������������������������������� 719
Benign Tumors������������������������������������������������������������������������������������������������������� 719
Nabothian Cyst��������������������������������������������������������������������������������������������������� 719
Malignant Tumors��������������������������������������������������������������������������������������������������� 720
Rhabdomyosarcoma������������������������������������������������������������������������������������������� 720
Vaginal Masses����������������������������������������������������������������������������������������������������������� 720
Benign Masses ������������������������������������������������������������������������������������������������������� 720
Gartner Duct Cyst����������������������������������������������������������������������������������������������� 720
Bartholin Cyst����������������������������������������������������������������������������������������������������� 721
Inclusion Cyst����������������������������������������������������������������������������������������������������� 721
Paraurethral Duct Cyst��������������������������������������������������������������������������������������� 721
Fibroepithelial Polyp������������������������������������������������������������������������������������������� 722
Müllerian Papilloma������������������������������������������������������������������������������������������� 722
Malignant Tumors��������������������������������������������������������������������������������������������������� 722
Rhabdomyosarcoma������������������������������������������������������������������������������������������� 722
Clear-Cell Adenocarcinoma and Endodermal Sinus Tumor������������������������������� 722
Vaginal Foreign Body������������������������������������������������������������������������������������������������� 723
Pubertal Disorders������������������������������������������������������������������������������������������������������� 723
Precocious Puberty������������������������������������������������������������������������������������������������� 723
Amenorrhea������������������������������������������������������������������������������������������������������������� 724
Polycystic Ovary Syndrome������������������������������������������������������������������������������� 724
Canal of Nuck Disorders��������������������������������������������������������������������������������������������� 725
Hydrocele of the Canal of Nuck����������������������������������������������������������������������������� 725
Hernia of the Canal of Nuck����������������������������������������������������������������������������������� 726
References������������������������������������������������������������������������������������������������������������������� 726
17 Urinary Tract��������������������������������������������������������������������������������������������������������������� 729
Ghadir H. Kassab, Ian Robinson, Roisin Hayes, Harriet J. Paltiel, D. Gregory
Bates, Harris L. Cohen, Richard A. Barth, and Gabrielle Christina Maria
Colleran
Introduction����������������������������������������������������������������������������������������������������������������� 730
Technique������������������������������������������������������������������������������������������������������������������� 730
Patient Positioning ������������������������������������������������������������������������������������������������� 730
Ultrasound Transducer Selection ��������������������������������������������������������������������������� 730
Imaging Approaches����������������������������������������������������������������������������������������������� 730
Grayscale Imaging ������������������������������������������������������������������������������������������������� 730
Doppler Ultrasound������������������������������������������������������������������������������������������������� 730
Contrast-Enhanced Ultrasound������������������������������������������������������������������������������� 730
Elastography����������������������������������������������������������������������������������������������������������� 731
Normal Development and Anatomy��������������������������������������������������������������������������� 731
Normal Development��������������������������������������������������������������������������������������������� 731
Normal Anatomy����������������������������������������������������������������������������������������������������� 734
Contents xxxv

Kidney����������������������������������������������������������������������������������������������������������������� 734
Infant��������������������������������������������������������������������������������������������������������������� 734
Older Child and Adolescent ��������������������������������������������������������������������������� 734
Anatomic Variants������������������������������������������������������������������������������������������� 734
Ureter ����������������������������������������������������������������������������������������������������������������� 738
Normal Anatomy��������������������������������������������������������������������������������������������� 738
Ureteral Jets ��������������������������������������������������������������������������������������������������� 738
Bladder��������������������������������������������������������������������������������������������������������������� 738
Normal Anatomy��������������������������������������������������������������������������������������������� 738
Anatomic Variants������������������������������������������������������������������������������������������� 740
Urethra ��������������������������������������������������������������������������������������������������������������� 740
Normal Anatomy��������������������������������������������������������������������������������������������� 740
Congenital Anomalies������������������������������������������������������������������������������������������������� 741
Anomalies of Renal Number, Position, Fusion, and Growth��������������������������������� 741
Renal Agenesis��������������������������������������������������������������������������������������������������� 741
Renal Duplication����������������������������������������������������������������������������������������������� 742
Supernumerary Kidney��������������������������������������������������������������������������������������� 743
Renal Ectopia ����������������������������������������������������������������������������������������������������� 744
Simple Ectopia ����������������������������������������������������������������������������������������������� 744
Crossed Renal Ectopia ��������������������������������������������������������������������������������������� 744
Horseshoe Kidney����������������������������������������������������������������������������������������������� 745
Pancake Kidney��������������������������������������������������������������������������������������������������� 745
Renal Hypoplasia ����������������������������������������������������������������������������������������������� 746
Anomalies of the Renal Collecting System and Ureter ����������������������������������������� 746
Classification of Prenatal and Postnatal Hydronephrosis����������������������������������� 746
Ureteropelvic Junction Obstruction ������������������������������������������������������������������� 746
Ureteropelvic Junction Obstruction Caused by Crossing Vessel ������������������� 748
Congenital Megacalyces������������������������������������������������������������������������������������� 748
Congenital Infundibulopelvic Stenosis��������������������������������������������������������������� 748
Calyceal Diverticulum ��������������������������������������������������������������������������������������� 750
Congenital Ureterovesical Junction Obstruction ����������������������������������������������� 751
Ectopic Ureter����������������������������������������������������������������������������������������������������� 752
Ectopic Ureterocele��������������������������������������������������������������������������������������������� 752
Retrocaval Ureter ����������������������������������������������������������������������������������������������� 752
Vesicoureteral Reflux ����������������������������������������������������������������������������������������� 752
Contrast-Enhanced Ultrasound Diagnosis of Vesicoureteral Reflux ������������� 755
Imaging of Endoscopically Placed Bulking Agents��������������������������������������� 755
Anomalies of the Bladder��������������������������������������������������������������������������������������� 755
Urachal Anomalies��������������������������������������������������������������������������������������������� 755
Patent Urachus ����������������������������������������������������������������������������������������������� 757
Vesicourachal Diverticulum��������������������������������������������������������������������������� 757
Umbilicourachal Sinus����������������������������������������������������������������������������������� 758
Urachal Cyst��������������������������������������������������������������������������������������������������� 758
Bladder Diverticula��������������������������������������������������������������������������������������������� 758
Bladder Exstrophy ��������������������������������������������������������������������������������������������� 758
Cloacal Exstrophy����������������������������������������������������������������������������������������������� 759
Cloacal Malformation����������������������������������������������������������������������������������������� 760
Bladder Duplication ������������������������������������������������������������������������������������������� 761
Bladder Agenesis ����������������������������������������������������������������������������������������������� 762
Prune-Belly Syndrome��������������������������������������������������������������������������������������� 762
Megacystis-Microcolon-Intestinal Hypoperistalsis Syndrome��������������������������� 763
Anomalies of the Urethra��������������������������������������������������������������������������������������� 764
Posterior Urethral Valves ����������������������������������������������������������������������������������� 764
xxxvi Contents

Anterior Urethral Valves������������������������������������������������������������������������������������� 765

Urethral Duplication������������������������������������������������������������������������������������������� 766
Acquired Ureteral Obstruction����������������������������������������������������������������������������������� 766
Intraluminal Obstruction����������������������������������������������������������������������������������������� 766
Extrinsic Compression ������������������������������������������������������������������������������������������� 767
Neurogenic Bladder ��������������������������������������������������������������������������������������������������� 767
Urinary Tract Infection����������������������������������������������������������������������������������������������� 768
Acute Pyelonephritis����������������������������������������������������������������������������������������������� 768
Renal Abscess��������������������������������������������������������������������������������������������������������� 769
Pyonephrosis����������������������������������������������������������������������������������������������������������� 770
Fungal Infection ����������������������������������������������������������������������������������������������������� 770
Parasitic Infection��������������������������������������������������������������������������������������������������� 772
Opportunistic Infection������������������������������������������������������������������������������������������� 772
Chronic Pyelonephritis������������������������������������������������������������������������������������������� 772
Xanthogranulomatous Pyelonephritis��������������������������������������������������������������������� 773
Cystitis ������������������������������������������������������������������������������������������������������������������� 773
Renal Cystic Disease����������������������������������������������������������������������������������������������� 774
Autosomal Recessive Polycystic Kidney Disease ��������������������������������������������� 775
Autosomal Dominant Polycystic Kidney Disease ��������������������������������������������� 775
Cystic Renal Dysplasia��������������������������������������������������������������������������������������� 776
Nephronophthisis ����������������������������������������������������������������������������������������������� 776
Medullary Cystic Disease����������������������������������������������������������������������������������� 777
Glomerulocystic Kidney Disease����������������������������������������������������������������������� 777
Syndromes with Renal Cysts ��������������������������������������������������������������������������������� 778
Tuberous Sclerosis ��������������������������������������������������������������������������������������������� 778
Von Hippel-Lindau Disease ������������������������������������������������������������������������������� 778
Acquired Cystic Kidney Disease ������������������������������������������������������������������������������� 779
Renal Vascular Disease����������������������������������������������������������������������������������������������� 779
Renal Artery Stenosis��������������������������������������������������������������������������������������������� 779
Renal Artery Thrombosis ��������������������������������������������������������������������������������������� 781
Renal Artery Pseudoaneurysm������������������������������������������������������������������������������� 781
Renal Vein Thrombosis������������������������������������������������������������������������������������������� 781
Arteriovenous Fistula��������������������������������������������������������������������������������������������� 783
Medical Renal Disease����������������������������������������������������������������������������������������������� 784
Acute Kidney Injury����������������������������������������������������������������������������������������������� 784
Chronic Kidney Disease����������������������������������������������������������������������������������������� 786
Renal Transplantation������������������������������������������������������������������������������������������������� 786
Surgical Technique������������������������������������������������������������������������������������������������� 787
Normal Posttransplant Imaging ����������������������������������������������������������������������������� 788
Complications��������������������������������������������������������������������������������������������������������� 788
Vascular Complications ������������������������������������������������������������������������������������� 788
Renal Artery Thrombosis ������������������������������������������������������������������������������� 788
Renal Artery Stenosis������������������������������������������������������������������������������������� 789
Renal Vein Thrombosis����������������������������������������������������������������������������������� 789
Arteriovenous Fistula������������������������������������������������������������������������������������� 789
Pseudoaneurysm��������������������������������������������������������������������������������������������� 789
Parenchymal Complications������������������������������������������������������������������������������� 791
Acute Tubular Necrosis����������������������������������������������������������������������������������� 791
Rejection��������������������������������������������������������������������������������������������������������� 792
Drug Toxicity ������������������������������������������������������������������������������������������������� 792
Urologic Complications ������������������������������������������������������������������������������������� 793
Transplant Urine Leak ����������������������������������������������������������������������������������� 793
Transplant Ureteral Obstruction��������������������������������������������������������������������� 794
Contents xxxvii

Transplant Vesicoureteral Reflux ������������������������������������������������������������������� 794

Transplant Pyelonephritis������������������������������������������������������������������������������� 795
Perinephric Fluid Collections����������������������������������������������������������������������������� 796
Transplant Lymphocele����������������������������������������������������������������������������������� 796
Transplant Urinoma ��������������������������������������������������������������������������������������� 796
Transplant Hematoma and Seroma����������������������������������������������������������������� 796
Transplant Abscess����������������������������������������������������������������������������������������� 796
Posttransplant Tumors����������������������������������������������������������������������������������������� 797
Urinary Tract Calcification����������������������������������������������������������������������������������������� 798
Renal Cortical Calcification����������������������������������������������������������������������������������� 798
Medullary Nephrocalcinosis����������������������������������������������������������������������������������� 798
Renal Vein Thrombosis Calcifications ������������������������������������������������������������������� 799
Dystrophic Calcification����������������������������������������������������������������������������������������� 799
Urinary Stasis��������������������������������������������������������������������������������������������������������� 799
Urolithiasis������������������������������������������������������������������������������������������������������������� 799
Risk Factors ������������������������������������������������������������������������������������������������������� 800
Kidney Stone Risk Factors����������������������������������������������������������������������������� 800
Bladder Stone Risk Factors����������������������������������������������������������������������������� 800
Trauma ����������������������������������������������������������������������������������������������������������������������� 801
Renal Trauma ��������������������������������������������������������������������������������������������������������� 801
Contrast-Enhanced Ultrasound Diagnosis of Trauma����������������������������������������� 801
Bladder Trauma������������������������������������������������������������������������������������������������������� 802
Tumors and Malformations����������������������������������������������������������������������������������������� 803
Renal Tumors ��������������������������������������������������������������������������������������������������������� 803
Benign Renal Tumors����������������������������������������������������������������������������������������� 803
Mesoblastic Nephroma����������������������������������������������������������������������������������� 803
Angiomyolipoma ������������������������������������������������������������������������������������������� 804
Multilocular Cystic Renal Tumor������������������������������������������������������������������� 805
Metanephric Adenoma ����������������������������������������������������������������������������������� 805
Inflammatory Myofibroblastic Tumor������������������������������������������������������������� 806
Ossifying Renal Tumor of Infancy����������������������������������������������������������������� 806
Primary Malignant Renal Tumors����������������������������������������������������������������������� 806
Wilms’ Tumor������������������������������������������������������������������������������������������������� 806
Renal Cell Carcinoma������������������������������������������������������������������������������������� 810
Rhabdoid Tumor��������������������������������������������������������������������������������������������� 811
Clear Cell Sarcoma����������������������������������������������������������������������������������������� 811
Renal Medullary Carcinoma��������������������������������������������������������������������������� 812
Primitive Neuroectodermal Tumor����������������������������������������������������������������� 812
Other Rare Primary Malignant Renal Tumors ����������������������������������������������� 813
Secondary Malignant Renal Tumors������������������������������������������������������������������� 813
Leukemia��������������������������������������������������������������������������������������������������������� 813
Lymphoma ����������������������������������������������������������������������������������������������������� 813
Metastases������������������������������������������������������������������������������������������������������� 814
Primary Ureteral Tumors ��������������������������������������������������������������������������������������� 815
Fibroepithelial Polyp������������������������������������������������������������������������������������������� 815
Urothelial Tumor������������������������������������������������������������������������������������������������� 816
Secondary Ureteral Tumors ����������������������������������������������������������������������������������� 816
Extension of Wilms’ Tumor��������������������������������������������������������������������������� 816
Bladder Malformations and Tumors����������������������������������������������������������������������� 816
Vascular Malformations ������������������������������������������������������������������������������������� 816
Lymphatic Malformation ������������������������������������������������������������������������������� 816
Venous Malformation������������������������������������������������������������������������������������� 816
Benign Bladder Tumors ������������������������������������������������������������������������������������� 818
xxxviii Contents

Urothelial Papilloma��������������������������������������������������������������������������������������� 818

Papillary Urothelial Neoplasm of Low Malignant Potential ������������������������� 818
Fibroepithelial Polyp��������������������������������������������������������������������������������������� 818
Inflammatory Myofibroblastic Tumor������������������������������������������������������������� 818
Leiomyoma����������������������������������������������������������������������������������������������������� 819
Neurofibroma ������������������������������������������������������������������������������������������������� 819
Paraganglioma������������������������������������������������������������������������������������������������� 819
Nephrogenic Adenoma����������������������������������������������������������������������������������� 820
Malignant Bladder Tumors��������������������������������������������������������������������������������� 821
Rhabdomyosarcoma��������������������������������������������������������������������������������������� 821
Transitional Cell Carcinoma��������������������������������������������������������������������������� 822
Leiomyosarcoma��������������������������������������������������������������������������������������������� 823
Angiosarcoma������������������������������������������������������������������������������������������������� 823
Urethral Tumors ����������������������������������������������������������������������������������������������������� 824
Urethral Polyp����������������������������������������������������������������������������������������������������� 824
Urinary Diversion������������������������������������������������������������������������������������������������������� 824
References������������������������������������������������������������������������������������������������������������������� 825
18 Musculoskeletal System��������������������������������������������������������������������������������������������� 835
Delma Y. Jarrett
Introduction����������������������������������������������������������������������������������������������������������������� 835
Technique������������������������������������������������������������������������������������������������������������������� 836
Patient Positioning ������������������������������������������������������������������������������������������������� 836
Ultrasound Transducer Selection ��������������������������������������������������������������������������� 836
Imaging Approaches����������������������������������������������������������������������������������������������� 836
Normal Development������������������������������������������������������������������������������������������������� 837
Soft Tissues����������������������������������������������������������������������������������������������������������������� 837
Normal Anatomy and Imaging Approaches����������������������������������������������������������� 837
Skin and Subcutaneous Tissues ������������������������������������������������������������������������� 837
Muscles��������������������������������������������������������������������������������������������������������������� 837
Tendons��������������������������������������������������������������������������������������������������������������� 838
Infectious/Inflammatory Disorders������������������������������������������������������������������������� 838
Cellulitis������������������������������������������������������������������������������������������������������������� 838
Pyomyositis��������������������������������������������������������������������������������������������������������� 839
Soft Tissue Abscess��������������������������������������������������������������������������������������������� 839
Trauma ������������������������������������������������������������������������������������������������������������������� 840
Fat Necrosis ������������������������������������������������������������������������������������������������������� 840
Foreign Bodies ��������������������������������������������������������������������������������������������������� 841
Muscle Tears and Intramuscular Hematomas����������������������������������������������������� 841
Myositis Ossificans��������������������������������������������������������������������������������������������� 843
Muscle Hernia����������������������������������������������������������������������������������������������������� 843
Tendinopathy and Tendon Tears������������������������������������������������������������������������� 844
Tumors ������������������������������������������������������������������������������������������������������������������� 844
Subcutaneous Granuloma Annulare������������������������������������������������������������������� 845
Pilomatricoma����������������������������������������������������������������������������������������������������� 845
Lipoma ��������������������������������������������������������������������������������������������������������������� 846
Lipoblastoma������������������������������������������������������������������������������������������������������� 846
Bones/Cartilage����������������������������������������������������������������������������������������������������������� 847
Normal Anatomy and Imaging Approaches����������������������������������������������������������� 847
Congenital/Developmental Abnormalities������������������������������������������������������������� 849
Congenital Rib Anomalies ��������������������������������������������������������������������������������� 849
Infectious/Inflammatory Disorders������������������������������������������������������������������������� 850
Osteomyelitis ����������������������������������������������������������������������������������������������������� 850
Contents xxxix

Trauma ������������������������������������������������������������������������������������������������������������������� 851

Epiphyseal Separation����������������������������������������������������������������������������������������� 851
Classic Metaphyseal Lesion������������������������������������������������������������������������������� 852
Tumors ������������������������������������������������������������������������������������������������������������������� 853
Osteochondroma������������������������������������������������������������������������������������������������� 853
Joints��������������������������������������������������������������������������������������������������������������������������� 854
Infectious/Inflammatory Disorders������������������������������������������������������������������������� 854
Septic Arthritis ��������������������������������������������������������������������������������������������������� 854
Juvenile Idiopathic Arthritis������������������������������������������������������������������������������� 854
Hemarthrosis����������������������������������������������������������������������������������������������������������� 855
Shoulder ��������������������������������������������������������������������������������������������������������������������� 856
Normal Anatomy and Imaging Approaches����������������������������������������������������������� 856
Normal Anatomy������������������������������������������������������������������������������������������������� 856
Patient Positioning ��������������������������������������������������������������������������������������������� 856
Imaging Approaches������������������������������������������������������������������������������������������� 856
Congenital/Developmental Abnormalities������������������������������������������������������������� 858
Glenohumeral Dysplasia������������������������������������������������������������������������������������� 858
Elbow ������������������������������������������������������������������������������������������������������������������������� 860
Normal Anatomy and Imaging Approaches����������������������������������������������������������� 860
Normal Anatomy������������������������������������������������������������������������������������������������� 860
Patient Positioning ��������������������������������������������������������������������������������������������� 860
Imaging Approaches������������������������������������������������������������������������������������������� 860
Anterior Approach ����������������������������������������������������������������������������������������� 860
Posterior Approach����������������������������������������������������������������������������������������� 861
Medial Approach��������������������������������������������������������������������������������������������� 861
Lateral Approach��������������������������������������������������������������������������������������������� 861
Annular Ligament��������������������������������������������������������������������������������������������������� 862
Elbow Fat Pads and Joint Effusion������������������������������������������������������������������������� 862
Congenital/Developmental Abnormalities������������������������������������������������������������� 863
Congenital Radial Head Dislocation������������������������������������������������������������������� 863
Trauma ������������������������������������������������������������������������������������������������������������������� 864
Distal Humeral Epiphyseal Separation��������������������������������������������������������������� 864
Apophyseal Avulsion ����������������������������������������������������������������������������������������� 864
Pulled Elbow������������������������������������������������������������������������������������������������������� 866
Wrist and Hand����������������������������������������������������������������������������������������������������������� 866
Normal Anatomy and Imaging Approaches����������������������������������������������������������� 866
Normal Anatomy������������������������������������������������������������������������������������������������� 866
Patient Positioning ��������������������������������������������������������������������������������������������� 867
Imaging Approaches������������������������������������������������������������������������������������������� 867
Ganglia������������������������������������������������������������������������������������������������������������������� 868
Congenital/Developmental Abnormalities������������������������������������������������������������� 869
Carpal Boss��������������������������������������������������������������������������������������������������������� 869
Tumors ������������������������������������������������������������������������������������������������������������������� 869
Giant Cell Tumor of the Tendon Sheath������������������������������������������������������������� 869
Hip������������������������������������������������������������������������������������������������������������������������������� 869
Normal Anatomy and Imaging Approaches����������������������������������������������������������� 869
Normal Anatomy������������������������������������������������������������������������������������������������� 869
Patient Positioning ��������������������������������������������������������������������������������������������� 870
Imaging Approaches������������������������������������������������������������������������������������������� 871
Neonates and Infants for Hip Dysplasia��������������������������������������������������������� 871
Assessment for Synovitis and Effusion ��������������������������������������������������������� 872
Congenital/Developmental Abnormalities������������������������������������������������������������� 872
Developmental Dysplasia of the Hip ����������������������������������������������������������������� 872
xl Contents

Imaging����������������������������������������������������������������������������������������������������������� 873
Treatment ������������������������������������������������������������������������������������������������������� 875
Proximal Focal Femoral Deficiency������������������������������������������������������������������� 876
Infectious/Inflammatory Disorders������������������������������������������������������������������������� 877
Transient Synovitis and Septic Arthritis������������������������������������������������������������� 877
Trauma ������������������������������������������������������������������������������������������������������������������� 877
Slipped Capital Femoral Epiphysis��������������������������������������������������������������������� 877
Knee ��������������������������������������������������������������������������������������������������������������������������� 878
Normal Anatomy and Imaging Approaches����������������������������������������������������������� 878
Normal Anatomy������������������������������������������������������������������������������������������������� 878
Patient Positioning ��������������������������������������������������������������������������������������������� 879
Imaging Approaches������������������������������������������������������������������������������������������� 879
Patellofemoral Joint ��������������������������������������������������������������������������������������� 879
Extensor Mechanism��������������������������������������������������������������������������������������� 879
Tibial Tubercle ����������������������������������������������������������������������������������������������� 880
Menisci����������������������������������������������������������������������������������������������������������� 880
Joint Effusion and Baker Cyst ��������������������������������������������������������������������������� 881
Joint Effusion ������������������������������������������������������������������������������������������������� 881
Baker Cyst������������������������������������������������������������������������������������������������������� 881
Congenital/Developmental Abnormalities������������������������������������������������������������� 882
Tibial Hemimelia ����������������������������������������������������������������������������������������������� 882
Congenital Knee Dislocation ����������������������������������������������������������������������������� 882
Congenital Patellar Dislocation ������������������������������������������������������������������������� 883
Bipartite/Multipartite Patella ����������������������������������������������������������������������������� 884
Discoid Meniscus and Meniscal Tears��������������������������������������������������������������� 885
Trauma ������������������������������������������������������������������������������������������������������������������� 886
Osgood-Schlatter Disease����������������������������������������������������������������������������������� 886
Sinding-Larsen-Johansson Syndrome����������������������������������������������������������������� 886
Ankle and Hindfoot����������������������������������������������������������������������������������������������������� 887
Normal Anatomy and Imaging Approaches����������������������������������������������������������� 887
Normal Anatomy������������������������������������������������������������������������������������������������� 887
Patient Positioning ��������������������������������������������������������������������������������������������� 887
Imaging Approaches������������������������������������������������������������������������������������������� 888
Joint Effusion ������������������������������������������������������������������������������������������������� 888
Calcaneal Apophysis��������������������������������������������������������������������������������������� 889
Trauma ����������������������������������������������������������������������������������������������������������������������� 889
Apophyseal Avulsion Injuries��������������������������������������������������������������������������������� 889
Vascular Anomalies����������������������������������������������������������������������������������������������������� 889
Vascular Tumors����������������������������������������������������������������������������������������������������� 889
Infantile Hemangioma ��������������������������������������������������������������������������������������� 889
Congenital Hemangioma ����������������������������������������������������������������������������������� 890
Kaposiform Hemangioendothelioma ����������������������������������������������������������������� 891
Vascular Malformations ����������������������������������������������������������������������������������������� 891
Venous Malformation����������������������������������������������������������������������������������������� 892
Lymphatic Malformation ����������������������������������������������������������������������������������� 892
Arteriovenous Malformation and Arteriovenous Fistula ����������������������������������� 893
References������������������������������������������������������������������������������������������������������������������� 894
19 Vascular Imaging ������������������������������������������������������������������������������������������������������� 899
Harriet J. Paltiel
Introduction����������������������������������������������������������������������������������������������������������������� 899
Neck Vessels��������������������������������������������������������������������������������������������������������������� 899
Technique��������������������������������������������������������������������������������������������������������������� 899
Contents xli

Patient Positioning ��������������������������������������������������������������������������������������������� 899

Ultrasound Transducer Selection ����������������������������������������������������������������������� 899
Imaging Approaches��������������������������������������������������������������������������������������� 900
Carotid Artery��������������������������������������������������������������������������������������������������������� 901
Normal Development and Anatomy������������������������������������������������������������������� 901
Normal Development������������������������������������������������������������������������������������� 901
Normal Anatomy��������������������������������������������������������������������������������������������� 902
Anatomic Variants����������������������������������������������������������������������������������������������� 902
Thrombosis and Stenosis ����������������������������������������������������������������������������������� 902
Aneurysm����������������������������������������������������������������������������������������������������������� 905
Dissection����������������������������������������������������������������������������������������������������������� 905
Internal Jugular Vein����������������������������������������������������������������������������������������������� 906
Normal Development and Anatomy������������������������������������������������������������������� 906
Normal Development������������������������������������������������������������������������������������� 906
Normal Anatomy��������������������������������������������������������������������������������������������� 906
Anatomic Variants����������������������������������������������������������������������������������������������� 906
Congenital Anomalies����������������������������������������������������������������������������������������� 906
Jugular Vein Phlebectasia������������������������������������������������������������������������������� 906
Thrombosis��������������������������������������������������������������������������������������������������������� 906
Stenosis��������������������������������������������������������������������������������������������������������������� 908
Aneurysm����������������������������������������������������������������������������������������������������������� 908
Extremity Arteries������������������������������������������������������������������������������������������������������� 909
Technique��������������������������������������������������������������������������������������������������������������� 909
Patient Positioning ��������������������������������������������������������������������������������������������� 909
Ultrasound Transducer Selection ����������������������������������������������������������������������� 910
Imaging Approaches������������������������������������������������������������������������������������������� 910
Normal Development and Anatomy����������������������������������������������������������������������� 913
Upper Extremity������������������������������������������������������������������������������������������������� 913
Normal Development������������������������������������������������������������������������������������� 913
Normal Anatomy��������������������������������������������������������������������������������������������� 913
Lower Extremity������������������������������������������������������������������������������������������������� 913
Normal Development������������������������������������������������������������������������������������� 913
Normal Anatomy��������������������������������������������������������������������������������������������� 913
Anatomic Variants��������������������������������������������������������������������������������������������������� 914
Stenosis and Thrombosis ��������������������������������������������������������������������������������������� 914
Aneurysm��������������������������������������������������������������������������������������������������������������� 916
Pseudoaneurysm����������������������������������������������������������������������������������������������������� 916
Arteriovenous Fistula��������������������������������������������������������������������������������������������� 916
Extremity Veins����������������������������������������������������������������������������������������������������������� 918
Technique��������������������������������������������������������������������������������������������������������������� 918
Patient Positioning ��������������������������������������������������������������������������������������������� 918
Ultrasound Transducer Selection ����������������������������������������������������������������������� 918
Imaging Approaches������������������������������������������������������������������������������������������� 919
Normal Development and Anatomy����������������������������������������������������������������������� 922
Upper Extremity������������������������������������������������������������������������������������������������� 922
Normal Development������������������������������������������������������������������������������������� 922
Normal Anatomy��������������������������������������������������������������������������������������������� 922
Lower Extremity������������������������������������������������������������������������������������������������� 922
Normal Development������������������������������������������������������������������������������������� 922
Normal Anatomy��������������������������������������������������������������������������������������������� 922
Anatomic Variants��������������������������������������������������������������������������������������������������� 923
Thrombosis������������������������������������������������������������������������������������������������������������� 923
Acute Deep Vein Thrombosis����������������������������������������������������������������������������� 923
xlii Contents

Chronic (Residual) Deep Vein Thrombosis ������������������������������������������������������� 926

Retroperitoneal Vessels����������������������������������������������������������������������������������������������� 927
Technique��������������������������������������������������������������������������������������������������������������� 927
Patient Positioning ��������������������������������������������������������������������������������������������� 927
Ultrasound Transducer Selection ����������������������������������������������������������������������� 927
Imaging Approaches������������������������������������������������������������������������������������������� 927
Aorta����������������������������������������������������������������������������������������������������������������������� 927
Normal Development and Anatomy������������������������������������������������������������������� 927
Normal Development������������������������������������������������������������������������������������� 927
Normal Anatomy��������������������������������������������������������������������������������������������� 927
Thrombosis��������������������������������������������������������������������������������������������������������� 927
Stenosis��������������������������������������������������������������������������������������������������������������� 928
Aneurysm����������������������������������������������������������������������������������������������������������� 929
Dissection����������������������������������������������������������������������������������������������������������� 931
Inferior Vena Cava ������������������������������������������������������������������������������������������������� 931
Normal Development and Anatomy������������������������������������������������������������������� 931
Normal Development������������������������������������������������������������������������������������� 931
Normal Anatomy��������������������������������������������������������������������������������������������� 931
Congenital Anomalies����������������������������������������������������������������������������������������� 933
Interruption of the IVC with Azygos Continuation ��������������������������������������� 933
Retrocaval Ureter ������������������������������������������������������������������������������������������� 933
Duplicated IVC����������������������������������������������������������������������������������������������� 934
Left-Sided IVC����������������������������������������������������������������������������������������������� 934
Thrombosis��������������������������������������������������������������������������������������������������������� 934
May-Thurner Syndrome��������������������������������������������������������������������������������� 936
References������������������������������������������������������������������������������������������������������������������� 936
20 Breast��������������������������������������������������������������������������������������������������������������������������� 941
Nadia Nagra-Mahmood, Angie L. Miller, Jennifer L. Williams, and Harriet J.
Paltiel
Introduction����������������������������������������������������������������������������������������������������������������� 941
Technique������������������������������������������������������������������������������������������������������������������� 941
Patient Positioning ������������������������������������������������������������������������������������������������� 941
Ultrasound Transducer Selection ��������������������������������������������������������������������������� 941
Imaging Approaches����������������������������������������������������������������������������������������������� 941
Normal Development and Anatomy��������������������������������������������������������������������������� 942
Normal Development��������������������������������������������������������������������������������������������� 942
Normal Anatomy����������������������������������������������������������������������������������������������������� 946
Anatomic Variants������������������������������������������������������������������������������������������������������� 947
Accessory Breast Tissue����������������������������������������������������������������������������������������� 947
Congenital Anomalies������������������������������������������������������������������������������������������������� 947
Poland Syndrome ��������������������������������������������������������������������������������������������������� 947
Polythelia/Polymastia��������������������������������������������������������������������������������������������� 947
Amastia/Athelia/Amazia����������������������������������������������������������������������������������������� 947
Developmental Anomalies ����������������������������������������������������������������������������������������� 948
Premature Thelarche����������������������������������������������������������������������������������������������� 948
Juvenile (Virginal) Hypertrophy����������������������������������������������������������������������������� 948
Gynecomastia��������������������������������������������������������������������������������������������������������� 948
Inflammatory Lesions������������������������������������������������������������������������������������������������� 949
Mastitis and Abscess����������������������������������������������������������������������������������������������� 949
Non-neoplastic Lesions����������������������������������������������������������������������������������������������� 950
Mammary Duct Ectasia������������������������������������������������������������������������������������������� 950
Retroareolar Cysts (Obstructed Glands of Montgomery)��������������������������������������� 950
Contents xliii

Fibrocystic Disease������������������������������������������������������������������������������������������������� 951

Galactocele������������������������������������������������������������������������������������������������������������� 952
Hematoma��������������������������������������������������������������������������������������������������������������� 953
Fat Necrosis ����������������������������������������������������������������������������������������������������������� 953
Intramammary Lymph Node����������������������������������������������������������������������������������� 954
Vascular Malformations ����������������������������������������������������������������������������������������� 954
Venous Malformation����������������������������������������������������������������������������������������� 954
Lymphatic Malformation ����������������������������������������������������������������������������������� 955
Neoplasms������������������������������������������������������������������������������������������������������������������� 955
Benign Tumors������������������������������������������������������������������������������������������������������� 955
Fibroadenoma����������������������������������������������������������������������������������������������������� 955
Hemangioma������������������������������������������������������������������������������������������������������� 955
Intraductal Papilloma ����������������������������������������������������������������������������������������� 956
Juvenile Papillomatosis��������������������������������������������������������������������������������������� 957
Pseudoangiomatous Stromal Hyperplasia����������������������������������������������������������� 957
Lactating Adenoma��������������������������������������������������������������������������������������������� 958
Desmoid Tumor ������������������������������������������������������������������������������������������������� 959
Granular Cell Tumor������������������������������������������������������������������������������������������� 960
Malignant Tumors��������������������������������������������������������������������������������������������������� 960
Cystosarcoma Phyllodes������������������������������������������������������������������������������������� 960
Carcinoma����������������������������������������������������������������������������������������������������������� 961
Angiosarcoma����������������������������������������������������������������������������������������������������� 962
Hematologic Malignancies��������������������������������������������������������������������������������� 962
Metastases����������������������������������������������������������������������������������������������������������� 963
References������������������������������������������������������������������������������������������������������������������� 964
Index������������������������������������������������������������������������������������������������������������������������������������� 969
About the Contributors and Editors

Contributors

Patricia T. Acharya, MD Department of Radiology, Children’s Hospital of Los Angeles,

Keck School of Medicine, University of Southern California, Los Angeles, CA, USA
Sumera Ali, MD Department of Radiology, Arkansas Children’s Hospital, University of
Arkansas for Medical Sciences, Little Rock, AR, USA
Maddy Artunduaga, MD Department of Radiology, Pediatric Radiology Division, University
of Texas Southwestern Medical Center, Children’s Health Medical Center, Dallas, TX, USA
Richard A. Barth, MD Department of Radiology, Lucile Packard Children’s Hospital,
Stanford University School of Medicine, Stanford, CA, USA
D. Gregory Bates, MD Department of Radiology, Nationwide Children’s Hospital, The Ohio
State University College of Medicine, Columbus, OH, USA
Eric S. Bih, MD Edward B. Singleton Department of Radiology, Texas Children’s Hospital,
Baylor College of Medicine, Houston, TX, USA
Mitchell W. Boehnke, MD Department of Radiology, Children’s Hospital Colorado,
University of Colorado School of Medicine, Aurora, CO, USA
Christian L. Carlson, MD, MS Department of Radiology, Brooke Army Medical Center,
Uniformed Services University of the Health Sciences, Fort Sam Houston, TX, USA
Ilse Castro-Aragon, MD Division of Pediatric Radiology, Department of Radiology, Boston
Medical Center, Boston University School of Medicine, Boston, MA, USA
Harris L. Cohen, MD Department of Radiology, Le Bonheur Children’s Hospital, University
of Tennessee Health Science Center, Memphis, TN, USA
Gabrielle Christina Maria Colleran, MB BCh BAO Department of Radiology, National
Maternity Hospital and Children’s Health Ireland at Temple Street, University College Dublin
School of Medicine, Dublin, Ireland
Nathan David P. Concepcion, MD Department of Radiology, St. Luke’s Medical Center
College of Medicine - William H. Quasha Memorial, Quezon City, Philippines
Pedro Daltro, MD, PhD Department of Radiology, Alta Excelência Diagnóstica/DASA and
Clínica de Diagnóstico por Imagem/DASA, Rio de Janeiro, Brazil
Patrick Duffy, MB BCh BAO, MSc Department of Radiology, Boston Children’s Hospital
and Harvard Medical School, Boston, MA, USA
Monica Epelman, MD Department of Radiology, Nemours Children’s Hospital, University
of Central Florida College of Medicine, Orlando, FL, USA

xlv
xlvi About the Contributors and Editors

Alexandra M. Foust, DO Department of Radiology, Boston Children’s Hospital and Harvard

Medical School, Boston, MA, USA
Javier D. Gonzalez, DO, MBA Medical Center Radiology Group, Orlando Health Arnold
Palmer Hospital for Children, Orlando, FL, USA
Sharon R. Gordon, MD Department of Radiology, Montefiore Medical Center and the Albert
Einstein College of Medicine, Bronx, NY, USA
Süreyya Burcu Görkem, MD Division of Pediatric Radiology, Department of Radiology,
Erciyes University School of Medicine, Kayseri, Turkey
Zoltan Harkanyi, MD, PhD Department of Radiology, Heim Pal National Pediatric Institute,
Budapest, Hungary
Roisin Hayes, MB BCh BAO Department of Radiology, Children’s Health Ireland at Crumlin,
Dublin, Ireland
Anastasia L. Hryhorczuk, MD Department of Radiology, C.S. Mott Children’s Hospital,
Michigan Medicine, Ann Arbor, MI, USA
Delma Y. Jarrett, MD Department of Radiology, New York Presbyterian Hospital, Weill
Cornell Medical College, New York, NY, USA
Ghadir H. Kassab, BM, BCh, MSc Department of Radiology, Children’s Health Ireland at
Temple Street, Dublin, Ireland
Don-Soo Kim, PhD Department of Radiology, Boston Children’s Hospital and Harvard
Medical School, Boston, MA, USA
Helen H. R. Kim, MD Department of Radiology, Seattle Children’s Hospital, University of
Washington School of Medicine, Seattle, WA, USA
Wendy G. Kim, MD Department of Radiology, Boston Children’s Hospital and Harvard
Medical School, Boston, MA, USA
Ella Kipervasser, MD Department of Radiology, Mount Auburn Hospital, Cambridge, MA,
and Harvard Medical School, Boston, MA, USA
Jessica Kurian, MD Division of Pediatric Radiology, Departments of Radiology and
Pediatrics, Montefiore Medical Center and the Albert Einstein College of Medicine, Bronx,
NY, USA
Jeannie K. Kwon, MD Department of Radiology, Children’s Medical Center Dallas,
University of Texas Southwestern Medical Center, Dallas, TX, USA
Bernard F. Laya, MD, DO Department of Radiology, St. Luke’s Medical Center College of
Medicine - William H. Quasha Memorial, Quezon City, Philippines
Edward Y. Lee, MD, MPH Division of Thoracic Imaging, Department of Radiology, Boston
Children’s Hospital and Harvard Medical School, Boston, MA, USA
Mark C. Liszewski, MD Division of Pediatric Radiology, Departments of Radiology and
Pediatrics, Montefiore Medical Center and the Albert Einstein College of Medicine, Bronx,
NY, USA
Angie L. Miller, MD Department of Radiology, Children’s Hospital Colorado, University of
Colorado School of Medicine, Aurora, CO, USA
About the Contributors and Editors xlvii

Elisabeth P. Moredock, MD Department of Radiology, Children’s Medical Center Dallas,

University of Texas Southwestern Medical Center, Dallas, TX, USA
Marthe M. Munden, MD Department of Radiology, Shawn Jenkins Children’s Hospital,
Medical University of South Carolina, Charleston, SC, USA
Ross A. Myers, MD Department of Radiology, Lehigh Valley Reilly Children’s Hospital,
Allentown, PA, USA
Nadia Nagra-Mahmood, MD Edward B. Singleton Department of Radiology, Texas Children’s
Hospital, Baylor College of Medicine, Houston, TX, USA
Harriet J. Paltiel, MDCM Division of Ultrasound, Department of Radiology, Boston
Children’s Hospital and Harvard Medical School, Boston, MA, USA
Grace S. Phillips, MD Department of Radiology, Seattle Children’s Hospital, University of
Washington, Seattle, WA, USA
Domen Plut, MD, PhD Unit of Pediatric Radiology, Clinical Institute of Radiology, Ljubljana
University Medical Centre, Ljubljana, Slovenia
Ricardo Restrepo, MD Department of Interventional Radiology and Body Imaging, Nicklaus
Children’s Hospital, Miami, FL, USA
Erica L. Riedesel, MD Departments of Radiology and Imaging Sciences and Pediatrics,
Children’s Healthcare of Atlanta, Emory University School of Medicine, Atlanta, GA, USA
Ian Robinson, MB ChB Department of Radiology, National Maternity Hospital and Children’s
Health Ireland at Temple Street, University College Dublin School of Medicine, Dublin, Ireland
Elisabetta Sassaroli, PhD Department of Physics, Bridgewater State University, Bridgewater,
MA, USA
Judy H. Squires, MD Department of Radiology, UPMC Children’s Hospital of Pittsburgh,
University of Pittsburgh School of Medicine, Pittsburgh, PA, USA
Patrick Tivnan, MD Department of Radiology, Boston Medical Center, Boston University
School of Medicine, Boston, MA, USA
Frank M. Volberg, MD Department of Radiology, UVA University Hospital, University of
Virginia School of Medicine, Charlottesville, VA, USA
Phillip Jason White, PhD Department of Radiology, Brigham and Women’s Hospital,
Harvard Medical School and Department of Physics, Simmons University, Boston, MA, USA
Jennifer L. Williams, MD Department of Radiology, University of Central Florida College
of Medicine, Maitland, FL, USA
Abbey J. Winant, MD Department of Radiology, Boston Children’s Hospital and Harvard
Medical School, Boston, MA, USA
xlviii About the Contributors and Editors

About the Editors

Harriet J. Paltiel, MDCM is a Pediatric Radiologist at

Boston Children’s Hospital where she heads the Ultrasound
Division, and an Associate Professor of Radiology at Harvard
Medical School. She is a Fellow of the American Institute of
Ultrasound in Medicine, and a Fellow and Past President of the
Society of Radiologists in Ultrasound.

Edward Y. Lee, MD, MPH is a Pediatric Radiologist at

Boston Children’s Hospital where he heads the Thoracic
Division, and an Associate Professor of Radiology at Harvard
Medical School. He is the Past President of the New England
Roentgen Ray Society (NERRS) and the Medical Staff
Organization (MSO) at Boston Children’s Hospital. Dr. Lee is
the current President of the International Society of Pediatric
Thoracic Imaging (ISPTI).
I am a part of all that I have met;
Yet all experience is an arch wherethro’
Gleams that untravell’d world whose margin fades
For ever and forever when I move.
― Alfred, Lord Tennyson, Ulysses

xlix
 Ultrasound Imaging Techniques
and Artifacts 1
Don-Soo Kim, Harriet J. Paltiel, Phillip Jason White,
and Elisabetta Sassaroli

Abbreviations IVC Inferior vena cava

M Motion
4D Four-dimensional MI Mechanical index
A Amplitude MR Magnetic resonance
A/D Analog-to-digital NCRP National Council on Radiation Protection and
AIUM American Institute of Ultrasound in Medicine Measurement
ARFI Acoustic radiation force impulse NEMA National Electrical Manufacturers Association
AVF Arteriovenous fistula ODS Output Display Standard
B Brightness PRF Pulse repetition frequency
C Constant-range PRP Pulse repetition period
CGS Centimeter-gram-second PZT Lead zirconate titanate
CNR Contrast-to-noise ratio PW Pulsed wave
CT Computed tomography ROI Region of interest
CW Continuous wave SI International System
D/A Digital-to-analog SSI Supersonic imaging
DICOM Digital Imaging and Communications in Medicine SNR Signal-to-noise ratio
DR Dynamic range SSWE Supersonic shear wave elastography
DVD Digital video disc SWI Shear wave imaging
EFSUMB European Federation of Societies for Ultrasound TGC Time gain compensation
in Medicine and Biology THI Tissue harmonic imaging
EI/B ratio Elastography image size (EI) to B-mode (B) ratio TI Thermal index
Eq. Equation TIB Bone thermal index
FDA Food and Drug Administration TIC Cranial thermal index
FOV Field of view 3D Three-dimensional
FR Frame rate TIPS Transjugular intrahepatic portosystemic shunt
FT Frame time T/R Transmit/receive
2D Two-dimensional
D.-S. Kim (*) UCA Ultrasound contrast agents
Department of Radiology, Boston Children’s Hospital and Harvard US United States
Medical School, Boston, MA, USA WFUMB World Federation for Ultrasound in Medicine
e-mail: don-soo.kim@childrens.harvard.edu and Biology
H. J. Paltiel Z Acoustic impedance
Division of Ultrasound, Department of Radiology,
Boston Children’s Hospital and Harvard Medical School,
Boston, MA, USA
P. J. White Introduction
Department of Radiology, Brigham and Women’s Hospital,
Harvard Medical School and Department of Physics, Ultrasound plays a crucial role in the diagnosis and manage-
Simmons University, Boston, MA, USA
ment of pediatric disorders. The ease of study performance,
E. Sassaroli portability, real-time capability, lack of ionizing radiation,
Department of Physics, Bridgewater State University,
Bridgewater, MA, USA

© Springer Nature Switzerland AG 2021 1

H. J. Paltiel, E. Y. Lee (eds.), Pediatric Ultrasound, https://doi.org/10.1007/978-3-030-56802-3_1
2 D.-S. Kim et al.
and cost-effectiveness relative to other cross-sectional imag-
ing techniques highlight its many advantages.
In this chapter, an overview of the physics and techno­logical
principles of diagnostic ultrasound is provided, including an
exploration of ultrasound-tissue interactions; the pulse-echo
technique; transducers and ultrasound machine instrumentation
for the detection and signal processing of echoes and image for-
mation; Doppler ultrasound; as well as B-mode and Doppler
ultrasound artifacts. Advanced ultrasound modes are also dis-
cussed, including harmonic and compound imaging, elastogra-
phy, 3D imaging, ultrasound contrast, bioeffects, and ultrasound
safety.
Acoustics
Wavelength and Frequency
Since its first clinical use in 1946, ultrasound imaging has
played a major role in pediatrics. The key advantages over
other diagnostic imaging techniques are its lack of ionizing
radiation, real-time multiplanar imaging capability, and porta-
bility. Medical ultrasound operates at frequency ranges from
1 to 20 MHz, which exceed the upper limit of the audible
range in humans, that is, from 20 to 20,000 Hz (Fig. 1.1).
Sound Propagation
Ultrasound is a high-frequency sound wave. Sound can be
defined as a disturbance that propagates through a medium
such as soft tissue carrying mechanical energy from one region
to another. In air, water, and soft tissue, sound propagates as a
longitudinal wave, where the movement of the particles in the
medium is in the same direction as the direction of propagation
of the wave. This is in contrast to transverse waves where the
movement of particles is perpendicular to the direction of wave
Frequency (MHz) 1 2
Therapy ABD/OB/CARD
Infrasound Audible
Frequency (Hz) 101 102 103
0
l in Tissue (m) 10 101 100
Fig. 1.1 Diagram illustrating the range of ultrasound frequencies and
wavelengths (bottom row) and the ranges typically associated with dif-
ferent medical applications of ultrasound (upper row). In medicine, low
frequencies less than 2 MHz are used for therapeutic applications. The
propagation (e.g., circular ripples generated by the drop of a
stone in a pond propagating outward on the water surface). Two
types of motion occur during sound propagation: the oscilla-
tory motion of each particle of the medium and the motion of
the wave carrying energy. As sound propagates through the
medium, it sets each particle of the medium into an oscillatory
back and forth motion. This oscillatory motion is similar to the
sinusoidal oscillations of a block attached to a spring (Fig. 1.2).
An important parameter is the period T, which is the time it
takes for a given particle of tissue to complete a full sinusoidal
oscillation as ultrasound waves travel through it (e.g., from
crest to crest or from trough to trough). This full oscillation is
k
m
x
-x0 0 x +x0
Oscillation
Fig. 1.2 Mechanical model of longitudinal sound wave propagation
through a medium conceptualized as individual particles within the
medium connected by springs. A block of mass m attached on a spring
with spring constant k sits on a very smooth surface. The spring is com-
pressed by pushing the block along a distance x0, which is then released.
After being released (and neglecting friction), the mass oscillates back
and forth about the relaxed position of the spring (the equilibrium posi-
tion), alternatively compressing and stretching the spring. The equation
describing this motion is a sine or cosine function, for example:
æ 2p ö where x is the amplitude (the maximum displace-
x ( t ) = x0 cos ç t ÷, 0
è T ø
ment from the equilibrium position), t is the time, and T is the period (the
time interval it takes to complete one complete oscillation or cycle)
5 10 20
PV/DERM/OPHTH
Ultrasound (Medical US)
104 105 106 107 108
10-1 10-2 10-3 10-4 10-5
frequency range of 2–7 MHz is used in abdominal (ABD), obstetric
(OB), and cardiac (CARD) imaging. Higher frequencies of 7–20 MHz
are used for peripheral vascular (PV), dermatology (DERM), and oph-
thalmological (OPHTH) imaging
1 Ultrasound Imaging Techniques and Artifacts 3

known as the wave cycle. The inverse of the period is the fre-
1
quency: f = (Fig. 1.3).
T T
A simple mechanical model for a longitudinal sound
wave is to consider that the particles of the medium are con-
nected by springs (Fig. 1.4). At any given instant, the com-
Displacement

x0
bined sinusoidal oscillations of all the particles generate
Particle

regions of compression, where the particles are crowded

time
Fig. 1.3 Particle displacement as a function of time. x0 is the maxi-
mum displacement from the equilibrium position and is also known as
the amplitude. Displacements are as small as a few μm. 𝑇 is the period
of time required for a particle to complete a full sinusoidal oscillation.
The inverse of the period (1/𝑇) is the frequency of the wave cycle
together and the springs are compressed, and regions of rar-
efaction, where the particles are spaced far apart and the
springs are stretched. In the regions of compression, the
pressure is increased above the baseline, and in the regions of
rarefaction, the pressure is lower than the baseline. It is these
regions of variation in pressure that propagate through the
medium as a wave with constant speed c.
The oscillations of the ultrasound transducer pressed against
the patient’s skin generate these variations in pressure that then
propagate through tissue as a sound wave, also known as a com-
pressional wave. As a function of distance or depth in tissue, the
pressure oscillates (sinusoidal oscillation), increasing and decreas-
ing above and below the mean pressure in the tissue (Fig. 1.5).

Transverse Particle Velocity

Longitudinal Particle Velocity

Fig. 1.4 A simple mechanical model for transverse and longitudinal waves. Particles oscillate up and down in a transverse wave, and back and
forth in a longitudinal wave

Amplitude (A0) Wavelength (λ ) Distance or Depth (x)

C R C R C R ...

Fig. 1.5 Diagram illustrating the combined sinusoidal oscillations of mum variation in tissue pressure from its mean value and determines
tissue particles resulting in regions of compression (C) in white and the amount of energy carried by the ultrasound wave. x is position, and
rarefaction (R) in black. At a given instant in time, along the direction λ is the wavelength of a longitudinal wave. The wavelength is the dis-
of propagation of the wave, the pressure variations are sinusoidal in tance between two sequential bands of compression or rarefaction
æ 2p ö
space (p = A sin ç x ÷), where the amplitude A represents the maxi-
è l ø
4 D.-S. Kim et al.

The amplitude A is the maximum variation of the pressure from
its mean value in the tissue. It is an important parameter because
the square of the pressure amplitude determines the amount of
energy carried by an ultrasound wave.
The wavelength of a longitudinal wave is the distance
between two bands of compression or rarefaction (crest to crest
or trough to trough).
A simple relationship exists between the velocity of prop-
agation of a sound wave (c), the wavelength (λ), and the fre-
quency (f) given by the equation (Eq. 1.1)
c=lf
of sound in soft tissue, for a frequency of 2 MHz, the wavelength
is λ = 0.77 mm and for a frequency of 10 MHz, λ = 0.15 mm.
Acoustic Impedance
The acoustic impedance (Z) refers to the resistance of a par-
ticular tissue or material to the propagation of sound. Acoustic
impedance is the product of mass density (ρ) and the velocity
(c) of sound in the medium:
(1.1) Z = rc (1.3)

The speed of sound depends on the mass density and the
elastic properties of the medium. It is nearly independent of
wavelength and frequency. Given the mass density (ρ) and
the bulk modulus (B) of a medium, which characterizes how
a tissue volume changes in response to compressional forces
such as those generated by longitudinal (compressional)
mechanical waves, the speed of sound can be expressed as:
B
c=
r
Figure 1.6 illustrates the speed of sound in a number of
tissue types and in a piezoelectric material, the major compo-
nent of ultrasound transducers, in order of increasing speed of
sound propagation.
Sound travels with an average speed of c = 1540 m/sec in soft
tissue and 4080 m/sec in bone. In medical ultrasound, the fre-
quency (f) of the ultrasound beam remains constant as it propa-
gates through different tissue types, while its wavelength (λ)
changes as the propagation speed c of the ultrasound varies some-
what from one soft tissue to another. Assuming the average speed
Acoustic impedance is expressed in units of
g cm−2s−1 × 10−5 in the centimeter-gram-second (cgs) sys-
tem or kg m−2 s−1 in the International System (SI) of units.
The SI unit is the Rayl. Tissues with similar acoustic imped-
ance values will be indistinguishable on an ultrasound image
even if histologically they are very different.
(1.2) Reflection
As ultrasound propagates through tissue, it loses energy, and
becomes exponentially attenuated. Part of this energy is
absorbed by the tissue and converted into heat, while another
portion is removed from the transmitted beam because of ultra-
sound-tissue interactions. These interactions include reflection,
scattering, and refraction. Reflected and scattered sound gener-
ates echoes for image formation, whereas transmitted sound
does not contribute to image formation.
Specular reflection of ultrasound refers to the interaction
of ultrasound with a smooth and relatively large surface. This

4500
4080 4000
4000

3500

3000
Phase speed (m/s)

2500

2000
1540 1565 1560 1555 1600
1450 1480
1500

1000
600
500 330

0
Air Lung Fat Water Soft Kidney Blood Liver Muscle Skull PZT
Tissue
Medium

Fig. 1.6 Diagram illustrating the speed of sound in a variety of different tissues, as well as in air and lead zirconate titanate (PZT), the major
component of ultrasound transducers
1 Ultrasound Imaging Techniques and Artifacts 5

Diffuse
Scattering

Specular Diffuse
Reflection Reflection

Fig. 1.7 Ultrasound-tissue interactions important for image formation include specular reflection, diffuse reflection, and diffuse scattering. Scattering
of sound occurs in every direction although the transducer detects only the backscattered sound

Z1 = ρ1c1

Z2 = ρ2c2

Z1 = Z2 Z1 ≅ Z2 Z1 >> Z2
or
Z1 << Z2

a b c d

Fig. 1.8 Diagram illustrating the relative amount of reflection of the
ultrasound wave between tissues of different acoustic impedance, Z. (a)
Reflection of ultrasound at a large and smooth interface between two
different tissues. (b) Total transmission of the ultrasound energy when
the two tissues have identical acoustic impedance (Z1 = Z2). No energy
is reflected, and so there will be no echo, and the interface will not be
seen in the ultrasound image. (c) Partial reflection of ultrasound occurs
when the two tissues have somewhat similar impedance (Z1 ≅ Z2, e.g., a
liver-tissue-fat interface). A fraction of the beam is reflected, and the rest
is transmitted. The interface will be seen in the image. (d) Almost total
reflection of ultrasound occurs at an interface between two tissues with
a very large acoustic impedance difference (Z1 ≫ Z2 or Z1 ≪ Z2, e.g., a
soft-tissue-air interface). Most of the ultrasound energy is reflected. The
interface will be seen in the image as a strong linear structure

interaction plays a major role in the generation of organ
images in diagnostic ultrasound imaging (Fig. 1.7).
When the ultrasound beam enters the tissue at a right
angle relative to the smooth interface and the interface is
much larger than the width of the beam, the incident
beam will be partially reflected toward the sound source.
The amount of reflection is determined by the difference in
the acoustic impedance of the two tissues (Fig. 1.8). The
greater the acoustic mismatch, the greater the amount of
reflection.
An interface between soft tissue and air reflects almost the
entire (99.9%) ultrasound beam, while an interface between
soft tissue (e.g., muscle) and lung reflects about 52%, and a
soft tissue (e.g., muscle) to bone interface reflects about 43%
6 D.-S. Kim et al.

(Fig. 1.9) [1]. Because of the high reflection of ultrasound at amplitudes creates a sound wave of cumulatively higher
the interface between air and soft tissue, ultrasound beam amplitude, whereas the sum of the echo amplitudes with
transmission is optimized by coupling the transducer to the destructive interference cancels each other out. Speckles pro-
skin with a liquid gel to remove intervening air pockets. duced from randomly distributed scatterers degrade image
Within the parenchyma of most organs (e.g., liver), there quality by decreasing contrast. Furthermore, speckle changes
are many small-scale variations in acoustic properties that with depth. Close to the transducer, the speckle appears quite
are caused, for example, by the presence of capillaries and fine-grained, whereas at greater depths it is coarser in appear-
red blood cells. These very small, very weak reflectors are ance (Fig. 1.12).
comparable in size or smaller than the incident sound beam
wavelength. Their interaction with the ultrasound beam is
referred to as “scattering.”
There are two important consequences of scattering for
ultrasound imaging. First, the scattered wave is much weaker
than the echo arising from an interface and is distributed in
multiple directions, not in a single direction as occurs with
reflection. Second, most of the echoes that are used to form
a typical ultrasound image arise from scattering of ultra-
sound by randomly distributed scatterers within a particular
organ and not from reflection at interfaces. Hence, the char-
acteristics and appearance of the scattered echoes are very
important for diagnostic purposes. These scattered echoes
are also responsible for the random granular bright and dark
echo texture visible in any ultrasound image, which is known
as speckle (Figs. 1.10 and 1.11).
At any given time, the scattered echo at a particular posi-
tion in the image is the sum of the scattered echo amplitudes.
Constructive interference occurs where the sum of the echo Structure
Surrounding
Tissue

Fig. 1.10 Diagram illustrating ultrasound wave transmitted from the

transducer (solid arrow) and scattered echoes (dotted arrows) arising
from the structure located in the surrounding tissue (black dot). The
impedance of the structure differs from that of the surrounding tissue
Muscle

1.7
MRayl

Fat Bone Lung Air

1.3 7.8 0.2 0.0004

MRayl MRayl MRayl MRayl

Fig. 1.9 Diagram illustrating the different degrees of reflection of the
ultrasound wave at the interface between muscle and various tissues
according to the degree of acoustic mismatch. Fat allows the most
transmission and air the least. Bone permits more transmission than lung
Fig. 1.11 Speckle artifact in a 14-year-old female. A transverse gray-
scale ultrasound image of the left hepatic lobe shows a grainy, mildly
heterogeneous appearance of the parenchyma (inside oval) caused by
scattering of sound waves from the surface of small structures such as
blood vessels
1 Ultrasound Imaging Techniques and Artifacts
Fig. 1.12 Speckle artifact in a 16-year-old female. Longitudinal gray-
scale ultrasound image of the right hepatic lobe demonstrates a grainy
appearance of the parenchyma (ovals) that is somewhat more pro-
nounced in the deeper portion of the liver (arrow) than in the superficial
portion (arrowhead)
θi θr
c1
c2
θ t2
sin θ i
=
θ t1 sin θ t
c2 > c1
c2 < c1
Fig. 1.13 Diagram illustrating Snell’s law, a formula used to describe the
relationship between the angles of incidence and refraction for two tissues
with sound wave speeds c1 and c2, respectively. When c2 < c1, the transmit-
ted angle θt1 is smaller than the incident angle θi. When c2 > c1, the trans-
mitted angle θt2 is larger than the incident angle θi. θr is the reflection angle
Reflection occurs at a flat, smooth interface. However,
some interfaces may appear slightly rough on the scale of a
wavelength and reflect ultrasound waves over a range of
angles, particularly as the ultrasound frequency increases, an
effect similar to scattering from small structures. This type of
reflection is known as diffuse reflection.
7
Refraction
Refraction is a change in the direction of propagation of the
incident ultrasound beam that occurs when the beam strikes
a tissue interface at an angle other than 90 degrees. The fre-
quency of ultrasound remains constant, but its speed and
wavelength are slightly different in the second tissue. The
clinical significance of refraction is that it can cause artifacts
where imaged structures appear in an incorrect location.
When the ultrasound beam travels from a tissue with
speed of sound c1 into a second tissue with the speed of c2, a
portion of the incident ultrasound will be reflected back into
the first tissue with an angle θr, that is the same as the inci-
dent angle θi: θr= θr. The reflected beam does not travel back
to the transducer and the interface will not be visible. The
remainder of the sound will be transmitted into the second
tissue with a changed direction of propagation, that is, it will
be refracted with transmitted angle θt.
The angles θi, θr, and θt are located between the direction
of propagation of ultrasound and a line perpendicular to the
tissue interface as shown in Fig. 1.13. The angle of refraction
can be calculated by Snell’s law:
sin q i c1
= (1.4)
sin q t c2
If the speed of sound in the second tissue, c2, is lower than
in the first tissue c1, (c2 < c1), as in the case of ultrasound
entering the interface from muscle to fat, the transmitted
angle θt is smaller than the incident angle θi, as shown in
Fig. 1.13. If c2 > c1, as in the case of ultrasound entering the
interface from fat to muscle, θt is larger than θi. For example,
for θi = 30°, θ t = 33.5°. As θi increases, so does θt. At a par-
ticular incident angle θc, called the critical angle, θt = 90°. At
the critical angle, there is no transmitted sound to the second
tissue and all incident sound runs along the interface between
c1 the two tissues. This phenomenon is known as total
c2 reflection.
The critical angle can be calculated from Snell’s law by
substitution of sin θt = 1 into Eq. 1.4:
c1
sin q c = (1.5)
c2
For example, the critical angle for fat-muscle interface is
θc = 65°.
Attenuation
As ultrasound propagates through tissue, it becomes ­attenuated.
Attenuation is caused by energy absorption by the ultrasound-
tissue interactions discussed in the prior section on refraction.
The absorbed energy is converted into heat. Because of the
attenuation of the incident beam, ultrasound intensity becomes
8 D.-S. Kim et al.
I0
I(x) = I0e-2αx
x
αs
Fig. 1.14 Ultrasound intensity is exponentially attenuated as it propa-
gates through tissue. The attenuation coefficient depends on both scat-
tering and absorption. The scattering of sound occurs in every direction,
exponentially attenuated as a function of depth (Fig. 1.14).
Attenuation limits our ability to image regions deep within the
body. Furthermore, ultrasound is highly attenuated by air and
bone, thereby severely limiting our ability to image organs such
as the lungs and the brain.
Intensity is defined as the energy delivered by the ultra-
sound wave per unit time, per unit area perpendicular to
the direction of propagation of the wave. Because of the
enormous variation in intensity during ultrasound propa-
gation, it is common to use a logarithm scale for intensity
measurement. The attenuation in intensity is given in deci-
bel units as
æI ö
attenuation in dB = 10 ´ log ç 1 ÷ , dB (1.6)
è I2 ø
I1
where I 2 is the ratio between the input and the output ultra-
sound intensity, and log is the base 10 logarithm. Attenuation
increases with depth and with frequency. For a round-trip,
the attenuation at a depth d in dB is
attenuation in dB = a ´ ( 2 ´ d ) ´ f (1.7)
where α is the attenuation coefficient for a given tissue in dB/
cm/MHz, f is the ultrasound frequency in MHz, and d is the
distance travelled by the ultrasound beam in cm. The average
soft tissue attenuation is 0.5 dB/cm/MHz.
The attenuation coefficient depends on both scattering and
absorption. As an example, a transmitted ultrasound beam of
frequency 5 MHz traveling a depth of 10 cm from the skin
surface will be attenuated by 25 dB. The returning echo will
be also attenuated by 25 dB as it returns back to the probe, for
a total of 50 dB attenuation. This means that the echo return-
α = αs + αa
Thermo-viscous losses
Molecular relaxation
αa
but only the backscattered echoes reach the transducer. Absorption is
caused by thermo-viscous losses and molecular relaxation
ing to the transducer from a 10 cm depth will be 50 dB weaker
than the transmitted ultrasound beam. This value substituted
in Eq. 1.6 shows that the intensity of the echo is 100,000
times less than the transmitted beam at the skin surface.
The depth of penetration is defined as the maximum depth
beyond which the echoes are too small to be detected by the
ultrasound transducer. Penetration becomes increasingly
smaller as ultrasound frequency increases. Because image
quality increases with frequency, the highest frequency com-
patible with a given depth of penetration is chosen by the
ultrasound operator.
Distance Measurement
Diagnostic ultrasound imaging is primarily implemented with
the pulse-echo technique, which is made possible by the
relatively slow speed of propagation of ultrasound in soft tis-
sue, where c = 1540 m/s and the use of short-duration ultra-
sound pulses.
With the pulse-echo technique, the ultrasound transducer
sends out an ultrasound beam of short duration. Structures
within the beam path cause some of the ultrasound to be
reflected and scattered. Some of this reflected and scattered
ultrasound (i.e., echoes) travels back along the path of the
transmitted beam to the transducer where it is detected. The
ultrasound machine determines the time between the trans-
mission of the ultrasound pulse and the reception of a given
echo. This time is known as time-of-flight. The time-of-flight
is then used by the machine to calculate the distance between
the transducer and the structure causing the echo:
1 Ultrasound Imaging Techniques and Artifacts
c´t
d= (1.8)
2
with c = speed of sound in soft tissue. In ultrasound machines,
this speed of sound is taken to be the average speed of sound
in soft tissue: c = 1540 m/s. For example, with a depth of
1 cm, the time-of-flight is 13 μs, and for 10 cm, it is 130 μs.
For this time scale, it is relatively simple for the electronic
circuits to distinguish reflections at different depths with
good resolution, and therefore to locate reflectors at different
depths.
To improve localization of a reflector, the pulse must be
short. A short-duration pulse is obtained when the transducer
generates ultrasound for a short-time duration of several
microseconds (μs). The pulse duration is usually expressed in
terms of the number of wave cycles. A typical pulse has a
Pulse sum of different frequencies
∑ is defined as the range of frequencies that comprise the pulse.
≡
Sinusoidal waves of different
frequencies
Fig. 1.15 An ultrasound pulse consists of a sum of continuous sinusoi-
dal waves spanning a range of frequencies. The pulse travels with a
group velocity, and each sinusoidal wave has a phase velocity. The dif-
ference between phase velocity and group velocity is a small ultrasound
wave that it is usually ignored. Each sinusoidal wave is assumed by the
ultrasound machine to travel at speed c = 1540 m/s
Amplitude
1/t
0 1 2 3 4 5
Frequency (MHz)
Fig. 1.16 Diagram of a frequency spectrum and pulse bandwidth 1/τ
9
duration of 3–5 cycles. At a frequency of 5 MHz, the period
is T = 0.2 μs, and therefore 4 cycles last for about 0.8 μs.
Fourier analysis shows that a short pulse consists of a
continuous sum of sinusoidal waves of different frequen-
cies (p = A sin (2πft)) (Fig. 1.15). This sum is within a defined
range of frequencies and is dominated by the central fre-
quency of the pulse, which has the highest amplitude and
therefore carries the most energy.
The ultrasound machine assumes that each frequency that
makes up the beam travels at the same speed c = 1540 m/s. This
is a good approximation, as the variation of speed as a function
of frequency (i.e., dispersion) is very small for ultrasound, and
therefore, the phase velocity and group velocity for ultrasound
waves are very close to each other [2]. More important factors
affecting ultrasound speed are the elastic properties and mass
density of each tissue and the amplitude of the wave.
The range of frequencies that make up a pulse is referred to
as the frequency spectrum, and it can be represented graphi-
cally by displaying the amplitude associated with each fre-
quency within the pulse (Fig. 1.16). The graph is centered on
the center frequency, which has the highest amplitude, and
therefore, the highest energy associated with it. The bandwidth
Bandwidth is related to the pulse duration by
1
Bandwidth = (1.9)
t
For example, a 5 MHz pulse of duration 0.8 μs has band-
width of 1.25 MHz. A short pulse yields a satisfactory time
resolution and therefore good distance resolution (i.e., axial
resolution) and its echoes contain information from a wide
range of frequencies. In contrast, the information contained
in an echo from a long pulse is concentrated near the central
frequency and gives a stronger signal at that frequency.
However, a longer pulse has poor distance resolution.
In order to generate an image, the ultrasound machine
must repeat the process of transmitting a pulse and receiving
the echoes several times, moving the beam so that it passes
through a different volume of tissue each time.
Pulse repetition frequency (PRF) is defined as the number
of pulses transmitted by the ultrasound machine in one sec-
ond. PRF is limited by the maximal depth of penetration,
which depends on the individual transducer settings and the
tissue being evaluated. The ultrasound machine does not trans-
mit a new pulse before it receives all of the echoes from the
previous pulse. The time to receive the echo from a maximum
depth penetration (i.e., range) Dmax is derived from Eq. 1.8:
2 ´ Dmax (1.10)
tmax =
c
This is the shortest time between two consecutive pulses,
6
and it defines the pulse repetition period (PRP), which is the
time between two consecutive pulses (Fig. 1.17). The inverse
10 D.-S. Kim et al.

PRP

Pulse
duration
PRP
(~1-2 µs) Dmax = c
2
PRP (µs) PRF (kHz) Range (cm)
100 10 7.7

150 6.7 11.6

Duty cycle = 0.2-2%
250 4 19.3

500 2 38.5

Fig. 1.17 The pulse repetition period (PRP) is determined by the range Dmax (maximum depth of penetration) and is the time interval between two
consecutive pulses. Some values for Dmax and corresponding PRF are shown

FOV

D FOV
FOV
D FOV

N N

PRP
D = Depth (m)
FOV = Field of View (m)
LD = N / FOV = Line Density (m-1)
N = Number of lines
FT = PRP x N = Frame Time (s)

FR = 1/FT = PRF/N = Frame Rate (fps or s-1)

Fig. 1.18 N scan lines (lines-of-sight) are required to generate an image for a given depth D. The field of view (FOV) is constant for a linear array
transducer. The FOV changes as a function of depth for a sector array transducer

of this number is the maximum number of pulses that can be
transmitted in one second and is given by:
c
PRFmax = (1.11)
2 ´ Dmax
Therefore, for a small depth of penetration, the machine
increases the PRF and for a larger depth, it reduces the
PRF. For example, for a range Dmax = 7.7 cm, the PRF is
10 kHz. If the depth is increased to Dmax = 19.3 cm, the PRF
is reduced to 4 kHz. The duty cycle (i.e., the time occupied
by the cycle of operation of the ultrasound machine as a per-
centage of available time) is the ratio between the pulse dura-
tion and the PRP. The PRP is much longer than the pulse
duration, so that following the transmission of the pulse,
most of the time is spent by the transducer in “listening” to
the returning echoes.
The frame rate (FR) is defined as the number of images
generated in one second. For image creation, an ultrasound
machine transmits a number of sequential pulses. Each pulse
travels along the path defined by the ultrasound beam width.
The beam is often represented as a line known as the scan line
or line-of-sight (Fig. 1.18). When creating an image, the ultra-
sound machine transmits N pulses with the total number of
pulses generated in one second equal to N × FR. This number
is by definition the PRF, so that the FR can be expressed as
PRF
FR = (1.12)
N
1 Ultrasound Imaging Techniques and Artifacts 11

The inverse of the FR is the frame time (FT): returning echoes, signal processing of the acquired echo
signals, and formation of the ultrasound images. Ultrasound
N
FT = = N ´ PRP . By inserting Eq. 1.11 into Eq. 1.12, machines have digital processing with analog and digital
PRF electronics, with a trend toward increasing levels of
we obtain the maximum FR digitization.
The transducer can convert electrical signals (voltage
c
FR max = (1.13) pulses) into ultrasound pulses and ultrasound echoes back into
2 ´ Dmax ´ N electrical signals. These electrical signals are analog signals.
Equation 1.13 is usually written as An analog electrical signal is a voltage pulse that varies
continuously as a function of time. It is converted to a “digital”
c
FR max ´ Dmax ´ N = = 77, 000 cm / s (1.14) signal by an analog-to-digital converter (A/D converter).
2 A digital signal is a series of binary numbers spaced at
If a high FR is desired, the depth of penetration will be regular time intervals. Each number represents the value of
lower and/or a relatively small number of transmit pulses the analog signal at the instant when it was detected (Fig. 1.19).
will be available to create each image. For example, if An A/D converter can also convert a digital signal into an
N = 100 and Dmax = 25 cm, the maximum FRmax is 30 frames analog signal. For example, when an echo is transformed
(images) per second. by the transducer into an analog echo signal, after pre-­
amplification, the A/D converter samples (i.e., measures) it
at regular time intervals (e.g., 0.1 μs). The resulting digitized
Instrumentation signal consists of ten million binary numbers (“samples”) per
second. Provided that the number of samples per second is
The four key elements of diagnostic ultrasound imaging sufficiently high (a sampling rate of 20–40 MHz), no infor-
include generation of the ultrasound beam, detection of the mation is lost in the digitization process.
In state-of-the-­art-ultrasound machines, beamforming is
implemented using digital techniques (covered later in this
chapter). A schematic block of a high-end ultrasound machine
is shown in Fig. 1.20.
Electrical signal

time (sec)
Fig. 1.19 Electrical signal sampling. Electrical signal is sampled at regular
time intervals (indicated by the red dots) and converted into binary numbers
Transmitter
The generation of the ultrasound beam is achieved by a
transmitter (also known as a pulser), the transmit beam-
former, and the transducer. State-of-the-art ultrasound
machines perform digital beamforming, where each trans-
ducer element has its own transmitter, A/D converter, ampli-

Receive Beamformer

A/D Delay + Processing Processing

T/R Switch Central Controller Display

D/A Delay + Processing Console

Transmit Beamformer

Fig. 1.20 Schematic block diagram of an ultrasound machine.

T/R, Transmit/receive; A/D, analog-to-digital; D/A, digital to analog
12 D.-S. Kim et al.

fier, and transmit-receive switch. The transmitter provides

voltage pulses (electrical signals) to the transducer for excit-
ing the piezoelectric transducer elements which generate the
ultrasound pulses that are then transmitted into the body.
The transmitter is programmed by the machine’s central con-
troller (computer) to produce voltage pulses of the correct fre-
quency, pulse duration, amplitude, and appropriate PRF. These
settings are initially set at default values by the machine when the
probe and examination type are selected at the start of the exami-
nation. However, the user has the ability to subsequently change a
number of these settings. The transmitter also controls the output
transmit power by adjusting the applied voltage amplitude. The
maximum voltage is limited by federal regulation for purposes of
patient safety. Onboard electronics
The transmit beamformer manipulates the electrical pulses
to be sent to the transducer elements so that the transmitted
ultrasound beam has the correct position, shape, and size. The
transmit beamformer delays the transmitted electrical signals
sent to each transducer element by an appropriate time interval Transducer backing
so that the beam is focused at the chosen depth. This process
is covered in more detail below. Piezoelectric element
In high-end ultrasound machines, this manipulation is achieved Matching layer
digitally, and the A/D converter of each element converts the digi-
tal signal into a suitable analog signal. When the switch is in the Fig. 1.21 Diagram of a typical linear array transducer
transmit mode, the analog signal excites the transducer element.
Immediately following the ultrasound pulse transmission, the shapes with chemical additives to produce various properties
switch is set to receive mode. that can be used in a variety of imaging applications. Until
recently, all ultrasound transducers were made from ceramic
materials which limited bandwidth.
Transducer Pulses with large bandwidth are required to improve
image quality. Currently, larger bandwidth is achieved with
The transducer that is contained inside the ultrasound probe a new generation of materials that are composites of ceram-
is the active component of the probe. An ultrasound trans- ics and polymers. Ultrasound probes made of these materi-
ducer is an electroacoustic device that produces ultrasound als are referred to as broadband probes. For the sake of
by converting the electrical signal pulse generated by the simplicity, we will only consider PZT transducers in this
transmitter into an ultrasound pulse that is sent into the body. discussion. However, composite transducers are similar to
The same transducer detects the echo pulse and converts it purely ceramic transducers, with the main difference con-
into an electrical pulse. The major components of a trans- sisting in the material used as for the piezoelectric plate.
ducer include a piezoelectric plate, a matching layer, a back- A damping layer is placed behind the ceramic plate. This
ing layer, and onboard electronics. A simplified diagram of a layer is designed to absorb energy and keep the transmit pulse
typical transducer is shown in Fig. 1.21. relatively short; it is generally made of plastic that contains metal
The piezoelectric plate is a thin crystal that can produce particles. A suitable matching layer which is also usually made
and detect mechanical vibrations. The thickness of the of plastic, is placed in front of the ceramic plate to minimize the
crystal is designed to be exactly half the wavelength of the acoustic impedance mismatch between the ceramic material and
generated ultrasound pulse. A higher frequency transducer the soft tissues of the body so that more acoustic energy can be
that is used for high-resolution images of superficial struc- transmitted into the tissue. Finally, a rubber lens along the trans-
tures has a thinner piezoelectric crystal than a lower fre- ducer face makes contact with the patient.
quency transducer used for imaging tissues and structures
at greater depth. While some naturally occurring materials
such as quartz have piezoelectric properties, their efficiency Receiver
is very low.
The development of ceramic materials has made ultra- The detection of echoes is achieved by the transducer and the
sound imaging possible, with lead-zirconate-titanate (PZT) receive beamformer. The transducer detects echoes as a func-
the most commonly used. PZT can be molded into different tion of time (or depth). Once the returning echo interacts with
1 Ultrasound Imaging Techniques and Artifacts
Amplification
TGC
Compression
Rectification
Demodulation
Rejection
Fig. 1.22 Processing of the echo signal includes time gain compensation (TGC), dynamic range compression, rectification, demodulation, and noise
rejection
the piezoelectric plate, the plate is deformed and changes in
thickness. This deformation generates a voltage difference
across the two electrodes.
Since ultrasound is highly attenuated with depth, an echo
signal can be very weak. The echo received by each array
element is boosted by the amplifier to a higher amplitude to
facilitate subsequent processing and for minimizing the
effect of electronic noise. The amount of amplification (or
gain) is preset by the machine, but the user can make
adjustments.
The amplified echo signal is then transformed into a digi-
tal echo signal by the A/D converter. The receive beamformer
delays the received echo signal from each transducer element
by an appropriate interval so that the ultrasound beam is
focused at the depth from which the echo arises, as discussed
below. After the echo signal is augmented by the amplifier
and digitized by the A/D converter, it is ready for processing.
Signal processing can be divided into the following pro-
cedures: time gain compensation (TGC), rectification,
demodulation, filtering (noise rejection), and dynamic range
compression (Fig. 1.22).
The ultrasound beam is attenuated with depth, and differ-
ent tissues have varying rates of attenuation. TGC is used by
the machine to apply a time-varying gain to the signals,
beginning with a relatively low gain for superficial depths and
increasing steadily with time as the echoes are received from
greater depths. Because different tissues have variable attenu-
ation, it is often necessary for the user to fine-­tune the TGC
function to account for these variations. This fine-tuning is
generally provided in the form of a series of slider controls.
Dynamic range (DR) refers to the overall range of echo
intensities used to form the image. It can be described math-
ematically as
13
æI ö (1.15)
DR = 10 ´ log ç max ÷ , dB
è I min ø
where Imax is the intensity of the strongest echo, and Imin
is the intensity of the weakest echo. The dynamic range of
I max
60 dB corresponds to a ratio = 1, 000, 000. The dynamic
I min
range of an ultrasound signal is 60 dB or more. In compari-
son, human vision has a dynamic range of at most 30 dB.
The echo signal is then compressed into 30 dB of gray-
scale dynamic range by the dynamic range compression
function of the machine.
The machine rectifies the echo signal (i.e., inverts the
negative amplitude values to positive values) and removes
the variations associated with the ultrasound frequency, leav-
ing only the echo signal amplitude. This process is referred
to as demodulation and amplitude detection. Finally, the
digital signal is filtered to reduce electronic noise (noise
rejection). Electronic noise has equal intensity at all
frequencies. Noise is eliminated for the frequencies outside
the bandwidth of the echo signal. Once the echo signals are
processed as described above, image processing occurs.
The scan converter writes the echo signals into the image
memory. The image memory is a part of the ultrasound
machine’s digital memory, which is used to store the processed
echo signals. It consists of a two-dimensional (2D) array of
pixels (pixel elements). A typical size is 1024 pixels wide by
768 pixels high, yielding a total of 786,432 pixels. Each pixel
stores a digital number which determines the displayed shade
of gray. This number is typically 8 or 12 bits long, providing
28 = 256 or 212 = 4096 different gray levels.
14 D.-S. Kim et al.

The echo information is acquired one scan line at a time. mid-gray, a strong echo as white, and no detectable echo as
The scan converter assembles the echo signals in the image black. B-mode scanning produces an image of a tissue slice
memory by ordering information from the beamformer on in any anatomical plane.
the time of transmission and direction of the ultrasound The B-mode display is used for 2D grayscale imaging as
beam so that each processed echo signal is at the correct follows: the scan line in B-mode is swept throughout the
position in the image memory. The scan lines may or may plane of interest to generate a 2D tomographic image built by
not pass through every pixel in the image memory. multiple scan lines, as shown in Fig. 1.18. The grayscale
The scan converter uses a mathematical technique known as range should be wide enough to properly display small differ-
“interpolation” to fill in the gaps between the scan lines with ences in the amplitude of the returning echoes arising from
appropriate values, so that the image is not degraded. Some pre- different sites in the body.
processing of the processed echo-signal is implemented prior to A-mode was widely used in the early days of diagnostic
writing to the image memory. This may include the depth dis- ultrasound. In A-mode scanning, the beam is kept at a fixed
played on the image, zoom, persistence (average of recently position and the machine transmits and receives along the
acquired images to reduce speckle), extended field of view (FOV), beam direction. The display shows echo amplitude as a func-
and compound imaging. These changes made during preprocess- tion of depth. Since A-mode scanning displays detected
ing are permanent. Postprocessing changes that may be imple- echoes in unaltered form as long as the transducer is station-
mented when the stored image is read out for display are not ary, it is useful for certain clinical applications such as tissue
permanent and may include measurements, color mapping, post- characterization, localization of foreign bodies in the eye, or
processing zoom, and grayscale postprocessing curves. identification of breast cysts.
M-mode is used to provide information regarding movement
and is mainly used in obstetrical imaging to detect embryonic
Image Display cardiac activity and in echocardiography to provide information
regarding motion of the walls of the heart and cardiac valves. As
Once image processing is complete, the image can be dis- with A-mode, in M-mode the ultrasound beam is fixed in posi-
played or stored. Brightness (B)-mode imaging is the most tion and repeatedly transmits pulses and receives echoes along
commonly used technique in clinical ultrasound imaging. this position. Structures that are stationary relative to the probe
Two other imaging modes used for specific applications (e.g., the chest wall) are displayed at a constant depth as hori-
include amplitude (A)-mode and motion (M)-mode. zontal lines, while structures that move toward and away from
When a reflector is included within the ultrasound beam the probe (e.g., the walls of the heart) move up and down with
as previously discussed, the reflector is placed at the correct their position displayed as a function of time (Fig. 1.23).
depth and location on the mid-line of the beam (Fig. 1.23). Constant-range (C)-mode is a method of display of cross-­
This midline is referred to as either the scan line or the line-­ sectional echo data where the imaged plane is at a constant
of-­sight. In B-mode imaging, the reflector is shown as a range from the transducer and perpendicular to the interro-
brightness dot, which represents the echo intensity. A weak gating beam.
echo will appear as dark gray, a moderate intensity echo as

Image Storage

In addition to appearing on the ultrasound machine display

monitor during a clinical examination, ultrasound images can
be stored in machine memory, on a removable storage device
(e.g., a digital video disc [DVD]), or on a network. Digital
A-Mode (1D) Imaging and Communications in Medicine (DICOM) is the
B-Mode (1D) most commonly used standard for storing and transmitting
medical images, including ultrasound images and related data.
B-Mode (2D)
C-Mode (2D)

Mechanical Transducer
Time

M-Mode (1D+1D)
Fig. 1.23 Diagram illustrating imaging of a reflector in brightness (B),
amplitude (A), constant-range (C), and motion (M) modes
The simplest type of mechanical transducer is a piezoelectric
plate. A sketch of a plane rectangular transducer is shown in
Fig. 1.24. A diagram of a plane circular transducer with
radius a is shown in Fig. 1.25. The scan plane is the x-z plane
1 Ultrasound Imaging Techniques and Artifacts 15

Elevational, Slice Thickness

P
r

z Axial, Longitudinal
φ

x Lateral, Azimuthal

Fig. 1.24 Diagram of a plane rectangular transducer. The Cartesian coordinates (𝑥, z) and the spherical polar coordinates (𝑟, 𝜃, 𝜙) of a point P are
shown. Transducer is located in the x-y plane in the diagram. The scan plane is the x-z plane, and the z-y plane is the elevation plane

y The beam patterns for a disc transducer on the scan plane

(x-z plane) are shown in Figs. 1.26, 1.27, 1.28, and 1.29.
These figures are generated from diffraction theory as
applied to ultrasound transducers. A discussion of diffraction
r
theory is beyond the scope of this chapter but can be further
a
explored by the interested reader [3, 4].
z The main portion of the ultrasound beam is located within
the bounds of the transducer surface up to a depth:

x l
Fig. 1.25 Diagram of a plane circular transducer of radius a. The rect-
angular coordinates (x, y, z) and the polar coordinates (r, θ) are shown
a2
Focal plane =
λ
Near-field Far-field
(Fresnel) (Fraunhofer)
Fig. 1.26 Diagram illustrating pressure amplitude along the trans-
ducer axis and in the scan plane
and the transverse (i.e., lateral or radial) planes are perpen-
dicular to the z-axis and parallel to the x-y plane. The trans-
ducer axis is the z-axis. An ultrasound wave is generated by
the back-and-forth oscillations of the disc and the wave
propagates in a direction perpendicular to the disc.
a2 (1.16)
z0 =
This depth defines the location of the focal plane. Beyond
this plane, the beam diverges (Fig. 1.30). The region from
the transducer to the focal plane is called the near-field or
Fresnel zone, and the region beyond the focal plane is the
far-field or Fraunhofer zone. The depth z0 in Eq. 1.16 is the
near-field depth. The spreading of the beam in the far field is
due to diffraction and is caused by the relatively large ultra-
sound wavelength. This divergence is the major factor limit-
ing spatial resolution in ultrasound imaging.
As shown in Fig. 1.26, in the near-field zone, the pressure
amplitude along the transducer axis has an oscillatory pattern.
At z0, the pressure reaches a final maximum value. In the far-
field zone, the pressure amplitude falls approximately as 1/z
1
and therefore the intensity as 2 . As shown in Figs. 1.26
z
and 1.27, the oscillatory pattern of crests and troughs in the
near-field zone is not restricted to the transducer axis but
occurs in the entire near-field zone.
In the far-field zone, the beam pattern becomes simpler
(Fig. 1.27). The pressure amplitude has a maximum value
on the z axis (beam axis) that decreases as 1/z (Fig. 1.26).
The pressure amplitude falls off with radial distance r from
the beam axis (Fig. 1.27). In this zone, the main beam width
increases linearly with depth of approximately λz/a.
In Fig. 1.27, the contour plot of the pressure amplitude is
also shown. The contour lines are symmetrical relative to trans-
16 D.-S. Kim et al.

Far-field
(Fraunhofer)

Focal plane a2/λ

Near-field
(Fresnel)

Fig. 1.27 Diagram of pressure amplitude and contour plot in the scan plane. Pressure amplitude in transverse planes at different locations is
shown along the transducer axis

a/λ= 2.0 a/λ= 3.2 a/λ= 6.4 a/λ= 10

a
Fig. 1.28 Pressure amplitude in the scan plane in the near-field zone for different values of
l

ducer axis and on each contour line the pressure amplitude has
the same value. This figure also shows the pressure amplitude
as a function of r at different depths. In the near-field zone, the
pressure amplitude in the transverse planes shows several small
oscillations. At the focal plane z0, the pressure amplitude shows
a main lobe with the highest peak and the smallest beam width
(about equal to the transducer radius a). In addition, there are
two weaker side beams, termed side lobes. In the far-field zone
the pressure amplitude in the transverse direction is signifi-
cantly reduced and is spread due to diffraction.
In both the near-field and far-field, the pressure undergoes
rapid oscillations. However, these variations are generally
averaged out and the signal obtained by a receiving trans-
ducer varies little within the near-field zone. As a result, a
1 Ultrasound Imaging Techniques and Artifacts 17

a
= 0 0.2 0.5 0.7
l

0.8 0.9 1.0 2.0

3.0 5.0 10.0 ∞

a
Fig. 1.29 Pressure amplitude in the scan plane in the far-field zone for different values of l

Near-field Far-field

Circular Flat a
Divergence Angle q
Transducer

z0

Focal plane

a2
Fig. 1.30 Illustration of the near-field and far-field of a circular flat transducer with radius a and focal length z0 = . The divergence angle θ is
defined as the angle between the transducer axis and the edge of the main central lobe l

transducer with radius a remains approximately collimated to
the near-field depth and then diverges. The beam diverges as
sin q = 0.61 ( l / a ) (1.17)
where θ is the angle between the transducer axis and the edge
of the main central lobe, as shown in Fig. 1.30.
A circular flat transducer can therefore generate a good lat-
eral (transverse) resolution up to about the focal plane
a2
( z0 = ). It is clinically important to have good lateral reso-
l a = 7.5 mm and operates at a central frequency of 3 MHz:
lution in order to correctly image a given structure since the
ultrasound machine places all the echoes gathered by a par-
ticular beam along the midline of the beam. The narrower the
beam, the more accurate the location of the structure in the
ultrasound image.
When, a ≅ λ, the near-field is short and the beam diverges
rapidly in the far-field, i.e., θ is large. When a is large com-
pared to λ, the near-field is long and there is little divergence
in the far-field, i.e., θ is small, an optimal imaging situation.
These trends are illustrated in Fig. 1.28 where the scan plane
beam patterns in the near-field zone are shown for different
values of a/λ. As shown in the figure, the oscillatory pattern
a
increases as a function of , but are averaged out at
detection. l
This information can provide a way to design ultrasound
transducers to have approximately near-field performance up
to a given depth z0. For example, if the transducer has a radius
a
λ = 0.51 mm (wavelength in soft tissue), = 11, the focal
l
plane depth is z0 = 110 mm, and the divergence angle is small
with θ = 2.4°. For a higher frequency, a smaller radius a is
required in order to maintain a collimated beam up to the focal
plane. For example, if the transducer operates at a central fre-
quency of 10 MHz and has a radius a = 2 mm:
a
λ = 0.51 mm, = 13, the focal plane depth is z0 = 26,and an
l
angle of divergence remains small at 2.6°.
18 D.-S. Kim et al.

Increased frequency allows the source diameter and the

beam width to be scaled down, while maintaining the beam
width divergence small, even if the focal depth is short. Focused
Increased frequency also results in greater ultrasound beam (Concave)
Transducer
attenuation.
The beam patterns in the focal plane for different values of
a/λ are shown in Fig. 1.28. For small values of a/λ, i.e., for
low frequency or for a relatively small transducer, pressure is Focal Zone
nearly independent of direction. As a/λ increases, the ultra-
sound intensity along the beam axis increases. For a flat
­circular transducer of radius a = 7.5 mm and f = 3 MHz, Plane
(Linear)
a Transducer
= 16, the beam pattern includes a main lobe and sides lobes.
l
To overcome the shortcoming of unfocused transducers
like the one shown in Fig. 1.25, focused transducers were Acoustic Lens

introduced. Focused transducers are curved or have an Fig. 1.31 Diagram of a focused transducer with a concave crystal
attached acoustic lens as depicted in Fig. 1.31. The effect of (upper image) and a plane (linear) transducer focused with an acoustic
the transducer curvature or the presence of the lens is that at a lens (lower image)
depth equal to the focal depth z = F as shown in Fig. 1.32, the
beam width is the same as that obtained for the circular plate
transducer in the far-field zone and can be approximated as:

lF Curved
Beam width = 2.44 (1.18) Transducer
D

with D transducer aperture. This is the so-called diffraction F (Focal length)

limit and is the best theoretical value that can be obtained for
the beam width for a given aperture D and wavelength.
Beyond that the beam diverges as it does with unfocused
transducers. Thus, a focused transducer with a larger aper-
ture D compared to λ can generate a small beam at this depth.
The additional advantage of using focused transducers is
that the beam pressure (or intensity) can be much larger in
the focal plane. This result can be obtained from diffraction
theory as well. The focus or focal depth is defined as the point
on the beam axis where the beam intensity is the highest and
the beam width is the smallest, and is given by Eq. 1.18. The
beam can be narrowed not just at the focal plane but over a
range of depths known as the focal zone (Fig. 1.33). Focusing
can be strong or weak.
A strongly focused transducer has a narrow beam at the
focus and a short focal zone with rapid beam divergence in
Fig. 1.32 Curved transducer with a radius of curvature of F. Spherical
wavefronts are also shown where all points of the propagating wave
have the same phase. If the pressure waves are emitted in phase by each
point on the surface of a spherically curved transducer, they will all
intersect at the common point (i.e., the geometrical focus in figure) in
phase. This occurs since they all travel the same distance to produce
maximum constructive interference, and therefore maximum intensity
at the focus. The wavefronts do not collapse to a point, because of the
diffraction limit. Beyond the focus the beam diverges
a Focal zone
Strongly
Focused Curved
Transducer

the far-field. A weakly focused transducer, with F/D > 1 has

a longer focal zone, diverges less rapidly than the strongly
focused transducer, and is more suitable for medical
imaging.
The beam patterns generated by a flat disc transducer and b Focal zone
Weakly
a curved or lens transducer can be obtained in a rigorous way Focused Curved
from diffraction theory but can be also understood intuitively Transducer
by considering the superposition principle and Huygens’
principle. The superposition principle states that when two
Fig. 1.33 Two curved transducers, one strongly focused (a) and one
or more waves reach the same location at the same time, their weakly focused (b). The focal zone is shorter and more superficial with
amplitudes sum algebraically (Fig. 1.34). the strongly focused transducer
1 Ultrasound Imaging Techniques and Artifacts
A
+ +
B
=
=
A+B
a b
Fig. 1.34 Superposition principle for two waves with same amplitude.
(a) If the waves arrive at the same location with the same phase (i.e.,
their peaks and troughs coincide in time), their amplitudes are additive
(constructive interference). (b) If the waves reach the same location
with opposite phase (i.e., the peaks of one coincide with the troughs of
the other), the result is complete cancellation (destructive interference)
Consider, for example, two waves of the same amplitude.
If they arrive at the same location with the same phase (i.e.,
with their peaks and troughs coinciding in time), their ampli-
tudes are additive (constructive interference). If they reach
the location with opposite phase (i.e., the peaks of one coin-
cide with the troughs of the other), the result is complete
cancellation (destructive interference). If they reach the loca-
tion with different phases (not opposite), the wave sum has a
lower amplitude. Huygens’ principle states that points on the
transducer surface act as point sources of spherical ultra-
sound waves, which have the same amplitude and frequency,
and are emitted in phase.
The pressure amplitude at a location P in the ultrasound
beam is determined by the sum of the spherical waves from all
of the points on the transducer face. If r is the distance between
a transducer point and a location P, the phase acquired by the
spherical wave emitted by this point is ϕ = (2πf)r/c, where r/c
is the time-delay and f is the central frequency.
In a circular plate transducer, different points on the trans-
ducer face have different distances to location P, so the waves
acquire different phases. At some location, this results in
overall constructive interference, giving rise to an amplitude
maximum. At other locations, the overall effect is destruc-
tive, and a minimum amplitude is formed. This is the reason
why in the near-field, for example, the pressure amplitude is
not constant but shows many peaks and troughs.
With spherically curved transducers, when waves are
emitted in phase at the transducer surface, they will acquire
the same phase traveling to the focus (ϕ = 2πfF/c), their
amplitudes sum and the intensity at the focus is maximum.
19
For a location different from the focus, the distance between
this location and a point on the transducer surface changes.
Therefore, the waves emitted by the point sources on the
transducer surface will arrive at the same location with dif-
ferent phases, as they travel different distances, and the wave
sum will have a much lower amplitude.
A lens in front of a flat-surface transducer will achieve the
same result as a spherically curved transducer. The lens is
thickest at its center. This will delay the wave traveling
through the center more than the waves traveling at the edges
of the lens. These delays lead to an in-phase sum at the focus.
The earliest and simplest transducers for medical applica-
tions used a fixed focused single element to generate and
receive ultrasound pulses. These transducers were supported
by a mechanical arm and moved along the body surface and
could be tilted. Position data were encoded by the mechani-
cal arm system, which made it possible to display echoes as
intensities on the display monitor.
Mechanical transducers are relatively inexpensive and easy
to operate. However, their low-speed mechanical scanning,
lack of variable ­focusing, and lack of real-time imaging subse-
quently led to the development of multiple-element electronic
transducers. These arrays can electronically reproduce the
effect of focusing a curved transducer or a lens.
Array Transducer
One of the remarkable advancements in ultrasound instrumen-
tation has been the development of multiple-element electronic
transducers, or array transducers. In an array transducer, the
ceramic plate is sliced into a large number of identical trans-
ducer “elements” to facilitate electronic focusing at different
depths and steering (change of direction) of the ultrasound
beam to a region of interest (ROI). Narrowing the beam to
obtain better lateral resolution at a given depth is referred to as
focusing. Array transducers achieve focusing electronically. As
discussed above, better focusing is obtained when the ultra-
sound frequency is high and/or the transducer aperture is large.
In array transducers, the ultrasound machine electronically cre-
ates the same effect of a curved transducer or a lens.
To illustrate the principle of electronic focusing, we can
consider the simplest case of a phased-array transducer. A
phased-array transducer uses all of the elements to form a
transmit pulse. Once the focal depth is selected by the user,
the machine determines the distance between each element
and the focus. This information together with the assumed
speed of sound in soft tissue of 1540 m/s is used by the
transmit beam-former to set a suitable time-delay for each
electrical signal sent to an element, so that each element is
activated at a slightly different time. The outermost elements
transmit first, followed by the elements adjacent to them and
so forth. The central element transmits last.
20
The beam is essentially identical to one that a spherically
curved transducer would produce, since the wavefronts
converge to the focus (Fig. 1.35). Different focal depths can
be chosen (Fig. 1.36). If the phased-array has 128 elements,
then a total of 128 electronic delays are required. The term
Signals
n = 64-128
Fig. 1.35 Focused beam generated by a phased-array transducer.
All the elements are used to generate the beam
Signals
Fig. 1.36 Beams generated by a phased-array transducer that are focused at different depths. All the elements are used to generate the beams
D.-S. Kim et al.
“channel” is used for these circuits. The number of channels
typically ranges from 64 to 128 elements, depending on the
quality and cost of the machine.
In order to generate an image, a phased-array transducer
scans the beam by steering sequentially in a number of direc-
tions (Fig. 1.37). The steering of the beam can be also obtained
by applying suitable time delays. The delays required to steer
the beam are added to the delays required to focus the beam.
A typical phased-array sweeps the beam approximately
90° from left to right, with each scan line (i.e., the midline of
the beam or line-of-sight) rotated by about 1° to the previous
scan line. This process requires about 90 transmit pulses to
form an image.
Focusing in reception is based on the same principle as
focusing in transmit. For each scan line, the echo received from
a given depth arrives at each element of the array at a slightly
different time. The echo arrives at the center element first, so the
receive beam-former applies the longest delay to the electrical
signal for this echo. The echo arrives at the outermost elements
last and therefore the beam-former applies the shortest delay to
them. As a result of these time delays, the signals are in phase
when they are added together electronically for processing.
Unlike transmit focusing, focusing in reception is auto-
matically controlled by the machine, a process termed
dynamic focusing. At each instant, the machine can deter-
mine the depth of the returning echoes. Since the time needed
for a two-way trip increases by 13 μs for every additional
1 cm of depth, the machine automatically advances the receive
focus at the rate of 1 cm every 13 μs, starting immediately
after the transmission of the pulse.
At the same time, as focus in reception is advanced, the
number of elements used to receive the echoes increases
Signals Signals
Focal plane
1 Ultrasound Imaging Techniques and Artifacts
Signals Signals
Fig. 1.37 Steering of the beam in different directions by a phased-array transducer
Signals
n = 256-512
n ~ 20
Fig. 1.38 Group of active elements in a linear array generating a beam
that is perpendicular to the transducer face
(receive aperture). This is known as “dynamic aperture.” The
reason for this is that the beam width at the focus is inversely
proportional to the transducer aperture (Eq. 1.18). It is there-
fore advantageous for the active receiving group to have as
many elements (i.e., as large an aperture) as possible.
However, there is no benefit in using a large group when
receiving echoes from superficial structures since elements far
from the center of the group will not receive echoes from these
21
Signals
structures. Thus, the maximum number of elements included
in the beam increases with time after transmission, in propor-
tion to the depth of the reception focus. As a result, the beam
width in successive focal zones remains fairly constant, keep-
ing lateral resolution as uniform as possible at all depths.
A typical linear array transducer has between 256 and 512
elements. The beam is created using an adjacent group of ele-
ments, ranging from 20 to 128. With a 256 linear array, a first
group of elements (i.e., 1, 2, 3, … to 20) generates an ultra-
sound beam (Fig. 1.38). Once all the echoes from this group are
detected, an adjacent group of elements is activated. This is
achieved by dropping an element from one end of the old group
and adding a new one at the other end (e.g., 2, 3, to 21), and so
on. The last beam is generated using elements 237–256, and
therefore the image is constructed using a total of 237 scan
lines. There is no steering of the beam in B-mode imaging for
linear arrays, so all the scan lines are parallel to the transducer
face (Fig. 1.38), and the image shape is rectangular.
There are modes that will be discussed later in this chap-
ter that require steering (Fig. 1.39), where a number of ele-
ments generate a steered beam. This occurs in pulsed Doppler
mode, where the direction of the B mode beams is unchanged,
but the Doppler beam may be steered to provide the appro-
priate angle between the beam and a blood vessel. In color
Doppler mode, all the elements may generate a steered beam
to achieve an appropriate Doppler angle while keeping the B
mode lines unchanged. In compound imaging, several B
mode images are created, each with a different beam-steer-
ing direction, which are then summed.
22 D.-S. Kim et al.

Array transducers provide focusing on the scan plane but
even today use a lens to achieve focusing along the elevation
plane, which is the plane perpendicular to the scan plane
(Fig. 1.24). This figure shows the (x, y, z) coordinates and the
spherical polar coordinates (r, θ, ϕ) of a point P. The scan
plane is the x-z plane, and the z-y plane is the elevation plane.
Transducer Selection
A medical ultrasound machine is equipped with multiple
probes for different clinical applications. The standard
probes include linear array, curvilinear array, and phased
array (Fig. 1.40).
Signals
n = 256-512
As discussed in the previous section, the image generated
by a linear array provides a rectangular FOV. A curvilinear
array functions as a linear array. However, owing to the probe
curvature, the beams are not parallel but change direction as
they step along the transducer face. Therefore, there is a
radial component to its scan pattern. This type of probe pro-
vides a wider FOV than the linear array.
In a phased array, the point of origin of the beam remains
fixed, but the beam is steered in a number of different direc-
tions. The resulting image provides a good FOV at depth, but
practically no information about superficial tissues.
The selection of a particular probe depends on the organ(s)
of interest and its depth from the probe face. The depth of
penetration is inversely proportional to the frequency, and
each transducer has a range of frequencies from which to
choose. In general, the highest frequency transducer that per-
mits adequate penetration to a tissue or organ of interest
should be selected. Frequencies of 5 MHz or lower are useful
for imaging organs such as the liver or kidney, while trans-
ducers with frequencies as high as 7–18 MHz are used to
acquire high-resolution images of superficial structures such
as muscle, tendon, testis, and thyroid. The neonatal brain can
also be imaged at higher frequencies.
The physical size of the probe, often called its “footprint,”
and the available “acoustic window” into the patient, i.e., the
area in the superficial tissues through which ultrasound can
travel, are also important. Linear arrays tend to have higher
frequency and are used for superficial structure imaging and
biopsy. Their FOV is limited by the probe size. Curvilinear
arrays tend to have a lower frequency and are used to image
structures at depth. The FOV diverges with depth, so it is not
so limited by probe size. When the acoustic window is

Fig. 1.39 Steered beam generated by a group of active elements in a

linear array transducer

Sector Arrays

Linear Array Curvilinear Array Phased Array

Narrow FOV Wide FOV

Superficial imaging Imaging at depth
High frequency Low frequency Small footprint
Biopsy guidance Abdominal imaging Variable frequency
Cardiac imaging

Fig. 1.40 Diagram of standard ultrasound probes, including linear array, curvilinear array, and phased array.
FOV, Field of view
1 Ultrasound Imaging Techniques and Artifacts
extremely limited, as, for example, when imaging the heart,
phased arrays are used.
Harmonic Imaging
It was stated above that the transmitted ultrasound pulse trav-
els at the average speed of sound in soft tissues (Fig. 1.15). It
was also implicitly assumed that although the pulse is attenu-
ated and that its amplitude decreases as it propagates in tis-
sue, its shape remains the same. This description of wave
propagation is from linear acoustics and is a very good
approximation to reality when the wave intensity is small. At
higher wave intensity, however, this assumption breaks down
and nonlinear propagation effects become noticeable. One of
these effects, which is at the foundation of harmonic imag-
ing, is pulse distortion.
In reference to Fig. 1.15, the wave intensity is highest in
the central portion of the pulse (i.e., at the highest crest and
lowest trough). The change of shape of the pulse is due to the
fact that tissue becomes stiffer where the pressure is highest
and more elastic where the pressure is lowest. When tissue
stiffness increases, the propagation speed c increases and
when the tissue becomes more elastic, speed c is reduced. As
a consequence, the crests will travel a bit faster and the
troughs a bit more slowly, causing the pulse to become
increasingly distorted as it travels. This distortion occurs
only in those regions where the pulse intensity is the highest,
i.e., along the beam axis.
Another important point is that the distorted pulse has
a different spectrum than the transmitted pulse. As dis-
cussed above, the transmit pulse is composed of a contin-
Frequency
f0
Fig. 1.41 The left panel shows a simulated image of the transmitted
beam in the scan plane with a central transmitted frequency f0. The
beam shows a main lobe and side lobes. The highest intensity (dark
23
uous sum of sinusoidal waves with a central frequency
f0 and a bandwidth 1/τ. When the pulse becomes distorted,
harmonics of the central frequency (integer multiples of
the central frequency): 2f0, 3f0, and so on, are generated.
If, for example, the central frequency is f0 = 4 MHz, the
second harmonic is 8 MHz, and the third harmonic is
12 MHz. The spectrum of the distorted pulse shows the
frequency f0 and associated bandwidth, a frequency 2f0
and associated bandwidth, and a frequency 3f0 and associ-
ated bandwidth, and so on.
The central frequency carries the most energy and the har-
monics carry increasingly less energy. If the transmitted
pulse is distorted as it propagates in tissue, the echoes caused
by the interactions of this pulse with tissue structures will
also be distorted. As a consequence, the distorted echo will
also have harmonic components. In practice, the second har-
monic is the most important harmonic since it carries the
most energy and is the least attenuated while the other har-
monic components are not considered. However, in keeping
with the prior discussion, echoes will have a significant sec-
ond harmonic component only where the intensity is high
and therefore near the axis of the main beam.
Figure 1.41 shows a simulated ultrasound beam with a
main lobe where the intensity decreases away from the beam
axis and side lobes. The dark red region near the central axis
is where the intensity is the highest. Only the echoes coming
from this region have a significant harmonic component.
This fact is exploited in harmonic imaging, where an image
is formed with echoes at the second harmonic 2f0 of the cen-
tral transmitted frequency f0. If, for example, the machine
transmits a 4 MHz central frequency, the harmonic image
will be created with echoes at 8 MHz.
Frequency
f0 2f0
red) is near the central axis of the beam. The right panel demonstrates
the portion of the beam near the central axis that has the highest sec-
ond harmonic component
24 D.-S. Kim et al.

In harmonic imaging, a broad bandwidth transducer is

used to transmit the central frequency and receives both the
central and the second harmonic frequencies. A filter is then
applied to remove the echo signal at the fundamental fre-
quency and to preserve only the harmonic component for
image formation.
Tissue harmonic imaging (THI) increases image quality
by suppressing weak echoes caused by artifacts and thereby
improving the signal-to-noise ratio (SNR). This improve-
ment occurs for two main reasons. First, the weak echoes
arising away from the axis of the main lobe and from the
side lobes and the slice thickness do not contribute signifi-
cantly to the harmonic image. Therefore, beam-width, side
lobe, and slice thickness artifacts are significantly reduced.
Second, the transmit pulse becomes gradually distorted as
it propagates through tissue.
In superficial tissue (up to 1 or 2 cm depth), the transmit-
ted pulse has not yet developed a significant distortion. This
means that the echoes coming from these superficial tissues
contain little energy at the second harmonic and consequently
reverberation artifacts (discussed in a later section) are
reduced. In addition, an improvement in spatial resolution is
to be expected with harmonic imaging since the image is
formed at twice the central frequency.
Harmonic imaging has the drawback of decreased pene- Fig. 1.42 Diagram of a compound scan. Several images are formed
tration. The second harmonic frequency is higher than the from different scanning angles and combined to form a single com-
pound image
central transmit frequency, hence it undergoes greater atten-
uation, and the depth of penetration is reduced.

Spatial Compounding

Spatial compound imaging mode is widely used with array

transducers. Array transducers have the capability of electronic
steering of the ultrasound beam to image the same tissue multi-
ple times along different directions. The beam steering produces
a set of images with a different lateral angle. The echoes from
these multiple images from different scanning angles are com-
pounded into a single image (Fig. 1.42). The operator can
change the number of images used to form the compound
image.
Spatial compounding significantly improves the contrast-­
to-­noise ratio (CNR) and the definition of tissue boundaries
by reducing the level of speckle, noise, clutter, and refractive Fig. 1.43 Spatial compounding. The combination of several images
shadows (Fig. 1.43). As a result, artifacts such as enhance- from different scanning angles generates a compound image that better
ment from weak reflectors and shadowing from strong reflec- displays the boundaries of a given structure
tors are reduced.
When a target is imaged from different approaches, it Better delineation of tissue boundaries, particularly of
appears at the same point in the final compound image, but curved boundaries such as blood vessel walls, can be under-
noise and speckle are uncorrelated from image to image and stood as follows: due to reflection, only the portion of a ves-
tend to cancel out, thereby improving contrast. Averaging sel wall that is perpendicular to the transmitted beam will
these images will theoretically increase the SNR by a factor generate a strong echo. Echoes from parts of the wall at other
of n with n equal to the number of images. angles relative to the beam will be reflected away and will
1 Ultrasound Imaging Techniques and Artifacts 25

not be seen. By combining images of the vessel taken at dif- sound imaging is useful for depiction of fetal anatomy, cardiac
ferent angles, the boundary of the target will be better dis- imaging, gynecologic scanning, and virtual endoscopy.
played in the compound image. The drawback of this mode In conventional 2D imaging, an image is formed by
is a reduced frame rate. acquiring echoes from a slice of tissue. The operator scans an
Because of the improved CNR, spatial compound imag- ROI, often changing the probe orientation. Some examples
ing is often used for imaging peripheral blood vessels and for of scanning techniques for imaging different parts of the
musculoskeletal applications. body are shown in Fig. 1.44. By sweeping the probe through
an ROI, the operator can form a 3D image of a particular
structure or lesion.
Three-Dimensional Ultrasound With 3D imaging, echoes from a volume of tissue are
acquired automatically. The sequence of 2D images and the
Three-dimensional (3D) ultrasound is used for viewing a vol- information about the position of each image in the scanned
ume of interest by combining multiple scan planes, as well as region are stored, and algorithms are used to form a 3D view of
for accurately estimating the volume of a lesion. 3D ultra- the ROI. This 3D view can be shown in a variety of formats.
The 3D imaging systems available commercially can be
divided into two groups depending on the ultrasound probe
used: conventional probes and 2D array probes. The scan-
ning methods used with conventional probes are free-hand
and mechanical. Electronic scanning is used with 2D array
Musculoskeletal
probes. The 3D image can be rotated through different planes
to be viewed from many angles.
Translational

In free-hand scanning, the imager manually sweeps the

transducer across the volume of interest with or without a
position-sensing device. Free-hand scanning permits ­imaging
Intravascular of a large volume, but measurements of distance, area, and/
Transrectal
or volume are generally not as accurate as other scanning
methods. Mechanical scanning employs a phased array
mounted in an assembly, which mechanically oscillates in a
sector movement or translates linearly over the body surface.
Images acquired by the latter technique provide better reso-
lution and accurate 3D reconstruction since the acquired 2D
images are parallel to one another, and their intervals are
well defined.
Ocular In electronic scanning, a 2D array probe is used to directly
image a 3D volume. A 2D array probe contains several thou-
sand square elements arranged in a two-dimensional array.
Rotational

Without moving the array, the 3D volume is built up by elec-

tronic steering of the beam through the volume of interest to
produce a pyramidal 3D view (Fig. 1.45). With a matrix probe,
it is possible to achieve equivalent resolution in the scan and
Cardiac (transcostal) elevational planes by applying the same degree of electronic
Cerebral (transfontanelle)
focusing and aperture control to both planes. Simultaneous
data acquisition from each image plane enables a real-time 3D
image display.
The process of turning a volume of data into an image is
Fig. 1.44 Scanning techniques for acquiring conventional two-­ referred to as rendering. As with computed tomography (CT)
dimensional (2D) images. For musculoskeletal imaging, the scan plane and magnetic resonance (MR) imaging, three types of ren-
is translated along an axis perpendicular to the plane and passing
through its center, sweeping a cuboid volume. For intravascular and dering are implemented in 3D ultrasound imaging: multipla-
intrarectal applications, the scan plane is translated in the same way, nar reformatting, volume rendering in the form of maximum
sweeping a cylindrical volume. For ocular applications, the scan plane projection, and surface rendering.
is rotated through a vertical axis located at the center of the scan plane, In multiplanar reformatting, a plane within the volume of
sweeping a cylindrical volume. For cardiac and cerebral applications,
the scan plane is rotated through a vertical axis located at one edge of data is chosen by the operator and the machine generates an
the scan plane, also sweeping a cylindrical volume image from the echoes lying in this plane. In maximum pro-
26
2D Array
Transducer
C-Scans
Fig. 1.45 Diagram of a 2D array transducer that produces a pyramidal 3D view. Volume data can be displayed as a series of B-scan planes, as
C-scan planes or rendering planes
Fig. 1.46 The Doppler effect is illustrated with a point source (target,
black dot) and a transducer. (Top) When the target is stationary, the
transmitted and reflected pulses have the same central frequency.
(Middle) When the target is moving toward a stationary transducer, the
jection, the machine software divides the volume of data into
view lines and for each line selects and displays the strongest
echo. In surface rendering, the most significant echo for each
line is chosen and displayed. The machine then highlights
the chosen echoes, permitting visualization of a surface of
interest such as the fetal face.
Repetitions of the 3D acquisition and their display at a
low frame rate can be performed with 2D array transducers
and is referred to as four-dimensional (4D) imaging.
D.-S. Kim et al.
Rendering Planes
B-Scans
central frequency of the reflected pulse is higher than the central fre-
quency of the transmitted pulse. (Bottom) When the source is moving
away from a stationary transducer, the central frequency of the reflected
pulse is lower than the central frequency of the transmitted pulse
Doppler Ultrasound
When a detector and a source of waves move relative to each
other, the observed frequency is not the same as the fre-
quency emitted by the source. When the source (target) of
the ultrasound waves moves toward the transducer, the fre-
quency detected by the transducer is higher. Conversely,
when the target is moved away from the transducer, the
observed frequency is lower (Fig. 1.46). In ultrasound imag-
1 Ultrasound Imaging Techniques and Artifacts 27

ing, when sound is reflected off a moving object such as

flowing blood or moving tissue, it undergoes a shift (i.e.,
change) in frequency.
Doppler ultrasound is widely used to detect blood flow.
More specifically, the Doppler frequency shift is used to
obtain information about the presence, absence, direction,
velocity, and temporal change of flow in blood vessels. The
only components of blood that scatter ultrasound are the red
blood cells, but the echoes that they produce are generally
very weak and not normally detected on B-mode imaging.
However, when the machine operates in Doppler mode, it
can detect this frequency shift and therefore detect blood
flow, particularly in the largest blood v­ essels. Doppler modes θD
include continuous wave (CW) Doppler, pulsed wave (PW)
u
Doppler, color Doppler, and power Doppler.
The change in frequency between the transmitted and Fig. 1.47 Diagram of ultrasound transducer emitting a sound wave
received frequencies is called the Doppler frequency shift that impinges on a blood vessel at angle θD. Blood flows within the ves-
(also known as the Doppler shift or Doppler frequency), FD. sel at velocity υ
The Doppler shift can be calculated by subtracting the fre-
quency of the transmitted beam, FT from the frequency of the
received beam, FR, and can be expressed as:
2 FT
FD = DF = FR - FT = v cos q D (1.19)
c
where v is the speed of the moving object such as blood, and
θD is the Doppler angle defined as the angle that the beam
axis makes with the direction of flowing blood (Fig. 1.47).
Velocity is a vector and therefore has a direction in space
and a magnitude. The speed is the magnitude of the veloc- a Stationary target:
(FR – FT) = 0
ity and is always a positive number. The factor 2 in Eq. 1.19
is due to the fact that sound travels a round trip from the
transducer to the blood vessel and back to the transducer.
The Doppler shift is determined by both the speed of the
red blood cells in the interrogated vessel and the angle the
blood vessel makes with the ultrasound beam axis.
The speed of a moving object is calculated from the mea-
sured Doppler shift and Doppler angle by rearranging
Eq. 1.19:
FD c b Target motion toward transducer:
v= (1.20) (FR – FT) > 0
2 FT cos q D
There is no Doppler effect when there is no flowing blood.
When blood is moving toward the transducer: θD = 0, the
2 v FT
Doppler shift is maximum: FD = (positive velocity)
c
and when it travels away from the transducer, the Doppler
shift is minimum FD = - 2 v FT (negative velocity) (Fig. 1.48).
c
For example, if blood is moving toward the transducer at a
speed of 100 cm/s and the transmitted beam has a central fre- c Target motion away from transducer:
quency of 4 MHz, then the Doppler shift is 5.2 kHz; if blood is (FR – FT) < 0
moving away from the transducer, the Doppler shift is –5.2 kHz.
Fig. 1.48 No Doppler shift is detected when blood (red blood cells) is
This frequency falls within the audible range of the human ear. not flowing. Detected frequency FR is slightly higher than transmitted
More generally, within the range of observed blood speeds, the frequency FT when blood is flowing toward the transducer, and slightly
Doppler shift usually falls in the audible range. Therefore, listen- lower when it is moving away from transducer
28 D.-S. Kim et al.

FT FR
FT FR

u u

Beam axis
Beam axis

∥ ∥

a b

Fig. 1.49 Blood flow generally occurs at an angle relative to the ultra- is the magnitude of the vector v. In (a), this component points away

sound beam axis, the Doppler angle θD. The blood velocity v can be from the transducer. In (b), it points toward the transducer. The fre-
considered as the vector sum of two vectors:  one
 parallel
 to the beam quency of the reflected sound beam is reduced in proportion to the
direction and the other perpendicular to it: v = v + v^. The component cosine of θ.
of velocity along the direction of the ultrasound beam is v cos θD, which FR, Reflected frequency; FT, transmitted frequency; v, velocity

θ = 90° axis (Fig. 1.49). Figure 1.50 shows the Doppler shift for
cos θ = 0.0
∆F = 0.0
several angles: at θD = 0, the blood is moving toward the
θ = 60° transducer and the Doppler shift is maximum; at θD = 60°,
cos θ = 0.5
∆F = 0.5 cos 60° = 0.5 and the Doppler shift is half that detected at
θD = 0; and at θD = 90°, no frequency shift should be
observed since cos 90° = 0.
In situations where blood flow direction can be estimated
from B-mode images, the operator activates the Doppler
mode (e.g., PW Doppler) and places the angle marker in the
θ = 0° direction of the blood flow within the Doppler sample vol-
cos θ = 1.0 ume. The ultrasound machine then calculates the Doppler
∆F = 1.0
angle and the blood flow velocity. There is some uncertainty
associated with this calculated value due to imprecision in
determining the exact direction of flow, subjectivity in place-
ment of the angle marker, and intrinsic spectral broadening,
an artifact discussed later.
Fig. 1.50 Diagram illustrating the effect of the Doppler angle on the fre-
The most important source of error is the estimation of
quency shift. The frequency shift is decreased in proportion to the cosine of blood flow direction. Flow is complex in blood vessels
the Doppler angle. At an angle of 90 degrees, there is no relative movement and not necessarily parallel to the vessel wall. However,
of the target toward or away from the transducer, and no frequency shift is in practice, the operator places the angle marker parallel
detectable. At an angle of 60 degrees, the detected frequency shift is half
that at an angle of 0 degrees. For a transducer with central frequency
to the blood vessel wall. The error in velocity is small
4 MHz, the Doppler shift is 5.2 kHz at θ = 0, 2.6 kHz at θ = 60°, and 0 at
when the angle θD = 0 and increases as a function of θD.
θ = 90°. ∆F = frequency shift
For this reason, 60° is usually considered the largest
acceptable angle for velocity estimation. For angles of
ing to the Doppler shift can provide useful information in addi- more than 60°, the cosine of the Doppler angle changes
tion to analyzing images. significantly. In echocardiography, angles close to zero
The Doppler shift is determined by both the blood are common, but for other applications, angles close to
speed and the angle the blood flow makes with the beam 60° occur more often.
1 Ultrasound Imaging Techniques and Artifacts 29

Continuous Wave Doppler

CW Doppler is the simplest Doppler device and is typically

housed in a handheld unit with an integrated speaker, such as T

those used in obstetrics for fetal heart monitoring. CW Doppler

units operate at low acoustic power levels, are relatively inex-
pensive, and are easy to use with minimal training.
The transducer is kept stationary over the ROI, and a sinu- R
soidal ultrasound wave is transmitted continuously. The
transducer contains two piezoelectric elements: one element
to transmit sound waves (FT) and the other to receive the
returned echoes (FR). This separation is necessary because
ultrasound is transmitted continuously. The two elements are
slightly tilted toward each other to allow overlap between the
transmit and receiving beams (Fig. 1.51).
Only blood and tissue within this sample volume are exposed
Fig. 1.51 Diagram of a continuous wave (CW) ultrasound transducer.
to the transmitted ultrasound and “listened” to by the receiving There are two piezoelectric elements, one to continuously transmit
element, and therefore, the Doppler shifted echoes are only sound waves (T) and the second to continuously receive the reflected
detected for this region. The region where the two beams over- echoes (R). The two elements are slightly tilted toward each other to
permit overlap between the transmitting and receiving beams
lap is termed the sensitive volume or sample volume.
In a typical CW Doppler device, the transmitter sends a
CW signal with frequency FT to both the transmitting ele-
ment and the detector. The detector calculates the difference
between the echo signal with frequency FR (detected by the
receiving element) and the transmitted signal, to output a sig-
nal at the difference frequency (beat frequency): the Doppler
shifted frequency FD.
Range gate
After wall filtering, where part of the signal with a fre-
quency below the filter cutoff is removed, the Doppler signal
is sent to a loudspeaker for monitoring. The primary function
of the wall filtering is to remove echoes from moving tissue.
In echocardiography, the CW Doppler device is built into an
ultrasound machine, and the signal is processed to generate a
Doppler spectral display, which is described below. Spatial
resolution is degraded in CW Doppler because time-local-
ized pulses are not used. In addition, CW sample volume is
generally quite large. Fig. 1.52 Diagram of a pulsed wave (PW) Doppler device. Following
transmission of a short burst of sound waves, the device listens for the
returning echoes before generating another burst. The received signals
can be electronically gated to detect echoes within a short time interval
Pulsed Wave Doppler that corresponds to a specific depth

In contrast to CW Doppler, with PW Doppler it is possible to

more accurately define the sample volume, typically with
dimensions of several mm. Since PW Doppler is used in com-
bination with B-mode, the sample volume can be accurately
positioned on the B-mode image. If the direction of the blood
flow is known, the ultrasound machine can estimate the
Doppler angle and therefore determine blood velocity.
As with B-mode, PW Doppler is based on the pulse-
echo principle and can therefore determine the depth of the
structure causing the echo. The operator places the sample
volume within the vessel. This allows the machine to calcu-
late the beam direction and the depth of the sample volume.
If angle correction is applied, the Doppler angle can be cal-
culated. The machine then generates a short pulse of ultra-
sound to transmit and collect the echo signal within a short
time interval of 1 to 2 μs, the range gate. Echoes that arrive
before and after the range gate are ignored.
The time of initiation of the range gate is calculated by
the machine to ensure that the echoes are coming from the
correct depth. A schematic diagram of a range gate is shown
in Fig. 1.52. The axial length of the sensitive volume can be
as small as 1 mm. For each sample volume, this process of
transmit and receive is repeated at regular intervals. This
30
process is similar to B-mode except that the beam does not
move. The number of transmit-receive cycles in one second
defines the Doppler PRF.
The sequence of echoes collected within a sample vol-
ume is combined in one PW Doppler signal. This signal is
then wall-filtered to remove noise caused by tissue move-
ment and is sent to a loudspeaker for monitoring and pro-
cessed to generate a spectral display.
An example of a spectral display in Fig. 1.53 shows a
distribution of velocities as a function of time. When flow
is toward the transducer, by convention the spectral display
is placed above the reference line of 0 cm/s. When flow is
away from the transducer, the spectral display is placed
below the reference line. If angle correction is not applied,
the machine assumes the Doppler angle to be zero, and the
velocity readings are therefore incorrect.
The left image (a) in Fig. 1.53 demonstrates spectral broad-
ening with a beat frequency of varying amplitude that is caused
by the fluctuation of the detected signal as red blood cells move
in and out of the sample volume. Typically, a sample volume
contains millions of red blood cells that do not move at the same
velocity. The velocity is generally highest at the center of the
blood vessel and falls to near zero close to the vessel wall.
The Doppler signal reflects the range of red cell veloci-
ties within the sample volume. In the presence of disease,
the blood velocity and its distribution can change. These
changes alter the PW Doppler signal in a way that can be
quantified in the spectral display.
Intrinsic spectral broadening can also occur, which is an
artifact. As with many artifacts, the cause lies in the ideal-
ized assumptions made by the machine. The ultrasound
machine assumes the ultrasound beam to be a single line
that originates from a single point on the transducer surface.
a b
Fig. 1.53 Normal spectral Doppler ultrasound signatures vary accord-
ing to the vessel in question. (a) Spectral Doppler waveform from a
normal main renal artery in an 18-year-old male demonstrates spectral
broadening (between arrowheads). (b) Spectral Doppler waveform of
D.-S. Kim et al.
In reality, the beam has a finite extent and originates from a
finite aperture, i.e., from a group of elements. The presence
of this intrinsic spectral broadening causes a systematic
error in every measurement of blood velocity. The greater
the spectral broadening, the more the blood velocity is over-
estimated. In addition, when blood flow becomes turbulent
because of disease, the spectrum has a wider range of
Doppler shifts than found in a normal vessel. Intrinsic spec-
tral broadening may mimic the spectral broadening due to
disease.
The lower and upper limits of the spectral display deter-
mine the maximum Doppler shift that can be correctly
­measured. These are the “Nyquist limits,” and their value
is half the Doppler PRF. The Doppler PRF is also called
the velocity scale because an increase or decrease will
increase or decrease the range of Doppler frequencies or
velocities that can be displayed. Additional operator con-
trols include sample volume length that affects the range
gate, Doppler gain, and the wall frequency cutoff.
The power of the transmitted ultrasound is higher in PW
Doppler than in B mode in order to increase the scattering
signal from blood. This is generally achieved by transmitting
longer pulses than in B mode. Although this process degrades
spatial resolution, it has the advantage of increasing trans-
mitted power without increasing peak pressure, which can be
harmful to the patient.
Another drawback of PW Doppler mode is that it is a time-
consuming process to examine a vessel since only one sample
volume at a time can be processed. The combination of real-time
B-mode imaging with CW or PW Doppler is known as duplex
Doppler sonography. It can be difficult to analyze blood flow in
small organs using PW Doppler if only a standard grayscale
image is used to identify the position of tiny vessels of interest.
the upper abdominal aorta in the same individual reveals a clear “win-
dow” (arrowhead), indicating that all the blood cells in the sample vol-
ume are moving at the same speed, so-­called “plug” flow
1 Ultrasound Imaging Techniques and Artifacts
Color Doppler
Color Doppler imaging, also known as color flow Doppler
and color flow imaging, is a form of duplex Doppler that
combines 2D grayscale imaging with the color mapping of
flow information in real time, as seen in the color image of
Fig. 1.54. Color Doppler is a technique that is complemen-
tary to PW Doppler. It can be used to locate blood vessels, to
guide placement of the sample volume for PW Doppler, and
to assess the vascularity of given organ or tissue.
It is important to remember that at each point of a color
Doppler image, the color represents the Doppler frequency
shift and not blood velocity. In color Doppler mode, there is
no audible sound or spectral display because of the way the
Doppler color image is acquired.
When initiating a color Doppler examination, the opera-
tor places a color box over the ROI. The machine creates a
grayscale image and then acquires the color image by scan-
ning the beam throughout the color box, collecting Doppler
shift information along each scan line. This information is
superimposed on the grayscale image to generate the color
Doppler image. This entire process is repeated many times,
according to the set frame rate.
For each scan line, the machine creates a large number of
sample volumes within the color box from top to bottom. As
with PW Doppler, color Doppler uses a gating technique,
but it is implemented differently since echoes from many
sample volumes need to be acquired, processed, and dis-
played. To speed up the process of collecting echoes, only a
small number of transmit pulses (typically 8) are used for
each scan line, compared to PW Doppler that typically uses
128 pulses.
R L
Fig. 1.54 Transverse color Doppler ultrasound image of a varicocele
in an 18-year-old male. Flow within the enlarged veins of the left-sided
varicocele (arrows) is well depicted. Flow information is superimposed
on grayscale images of the right (R) and left (L) testes
31
For each scan line, many range gates are simultaneously
active, each collecting echoes from different depths along
the scan line. For each sample volume on each scan line, the
echoes coming from the eight transmit pulses are fast-­
processed to yield three Doppler parameters: the mean
(average) Doppler shift, the variance, and the Doppler sig-
nal power.
The mean Doppler shift determines which color is dis-
played at each point in the color box. It is proportional to
the mean blood velocity within the sample volume and
also depends on the Doppler angle at any particular point.
By convention, shades of red are used to represent flow
toward the transducer and shades of blue to represent flow
away from the transducer. The color bar on the image indi-
cates the amount of Doppler shift, ranging from more satu-
rated shades of red and blue for low velocities to less
saturated shades for higher velocities. Areas where flow is
absent or too slow to be detected will appear dark.
The range of Doppler frequencies that can be measured
and displayed is determined by the Nyquist limit and is
half the Doppler PRF. The velocity values displayed at the
top and bottom of the color bar indicate what the blood
velocity at the Nyquist limits would be if the Doppler
angle were 0°. These values are therefore inaccurate unless
the Doppler angle is about 0°.
The variance is a measure of spectral broadening and is
not usually displayed in the color Doppler images except for
echocardiography, where large regions of increased spectral
broadening are assigned a green color in the image.
Compared to PW Doppler, color Doppler measures
Doppler shifts less accurately because it cannot correct for
Doppler angle, is less effective in removing noise caused by
moving tissues, and is less able to detect slow-moving blood
because the Doppler PRF has to be constantly readjusted.
When the ROI is deep in location, the color Doppler FR
can be low. The FR is improved by using a coarser line den-
sity and by reducing the size of the color box. Some high-end
machines have the capability of generating a number of
transmit beams simultaneously.
Power Doppler
Power Doppler, also known as energy Doppler or amplitude
Doppler, is a specialized form of color Doppler. Unlike color
Doppler, power Doppler does not display the mean Doppler
shift or direction of blood flow. Instead, it displays the power
(strength) of the Doppler signal. The power of the Doppler
32
Fig. 1.55 Normal power Doppler ultrasound image of the kidney in an
11-year-­old male. Flow in small blood vessels is well depicted
signal is encoded in color which is then superimposed on a
grayscale image as illustrated in Fig. 1.55.
Power Doppler has several advantages compared to color
Doppler, including greater sensitivity to flow, better edge defi-
nition, and depiction of the course of tortuous and irregular
vessels. The power of the Doppler signal is proportional to the
number of moving red blood cells. A map of perfusion can be
formed by displaying the amplitude of signals coming from
moving red blood cells in an ROI. The power is displayed
using a single color with a brightness scale reflecting ampli-
tude. Some machines can provide information about the blood
flow direction with directional power Doppler images.
Power Doppler is efficient in detecting low velocity flow
in small vessels because it is easier for the ultrasound
machine to detected signal power than the mean Doppler
shift when the echo is weak. Power Doppler is therefore the
preferred Doppler mode when weak signals need to be
detected and displayed. Power Doppler can also detect flow
when the Doppler angle is 90° and there is theoretically no
Doppler shift. This is due to the spectral mirror artifact, dis-
cussed later in this chapter. Power Doppler is not affected by
aliasing, except when the directional power display is used.
The main limitation of power Doppler is its extreme sensitiv-
ity to motion.
B-Flow
B-flow imaging, or B-mode flow imaging, is a non-Doppler
technique that produces real-time imaging of blood flow dur-
ing grayscale B-mode ultrasound imaging. B-flow imaging is
relatively simple to perform and is less prone to various arti-
D.-S. Kim et al.
facts and limitations than is color Doppler ultrasound which
requires manipulation of more parameters. Applications of
B-flow imaging include evaluation of blood flow in superficial
vascular structures such as the carotid arteries [5] and liver [6].
Slow-moving blood is well depicted at B-flow imaging
with high spatial resolution. Slow blood flow is not shown on
Doppler ultrasound studies that use high-pass filters (also
called wall filters) to remove the low-amplitude frequency
shifts coming from slow movement of blood and soft tissue
vibration around blood vessels. B-flow imaging does not
provide information regarding velocity or direction of flow.
Elastography Imaging
Ultrasound elastography imaging generates images of tis-
sue elasticity [7–9]. It is an emerging technique that is cur-
rently under evaluation in clinical practice. Ultrasound
elastography exploits the fact that diseased tissues often
have different elasticity than healthy tissues. For example,
cancerous tissue is usually stiffer than normal tissue as the
density of cells and blood vessels is higher. The two main
elastography methods currently implemented clinically are
(1) quasi-static strain elastography and (2) dynamic elas-
tography that applies a time-varying force to tissues. The
main clinical applications of ultrasound elastography to
date have been for imaging the stiffness of the liver and
breast.
Quasi-Static Strain Elastography
In some quasi-static strain elastography methods, the ultra-
sound probe is used to induce a quasi-static tissue deforma-
tion. Deformation occurs along the three spatial directions
although the largest deformation occurs along the direction
of the applied force, which is the direction of ultrasound
beam propagation.
Soft tissue is difficult to compress but easy to deform. As a
result, a deformation applied to a soft tissue mainly causes a
change in tissue shape, while the volume remains nearly
unchanged. This is the reason why soft tissues are considered
nearly “incompressible,” and the soft tissue bulk compressional
modulus, which characterizes the volume changes of a tissue in
response to compression, is generally several orders of magni-
tude larger than the shear modulus, which describes how a tis-
sue responds to a shear (transverse) force.
Given a lesion of volume V and surface area A, the applica-
tion of a force per unit area of lesion (known as stress) results
1 Ultrasound Imaging Techniques and Artifacts
x
x’
y y’
x=y x’ < y’
Precompression Compression
a b
Fig. 1.56 (a) Quasi-static compression of two identical-­sized lesions of
different stiffness. The quasi-static compression decreases the axial
length of both lesions, with x’ < x and y’ < y. The softer lesion undergoes
less axial strain (y’ > x’), assuming the transducer exerts approximately
Fig. 1.57 B-mode and elastography strain images of a thyroid nodule
in a 15-year-old female. Longitudinal grayscale ultrasound image (left
panel) shows a hypoechoic solid nodule (arrow) with a few internal
in an increase in cross-sectional area and a decrease in axial
length (Fig. 1.56a), while volume remains nearly constant. For
a lesion of axial length x, the strain along the axial direction is
defined by:
x¢ - xDx (1.21)
e= = axial direction is the measurement typically provided in an
x x
where x’ is the axial length after compression and x > x’.
Strain images are obtained by comparing the echo signals
from the lesion and surrounding tissue before and after
33
Shear waves
the same stress in each lesion. (b) The deformation of tissue induced by
the radiation force at the focal depth gives rise to a low-frequency (sonic)
shear wave that propagates primarily perpendicularly to the direction of
propagation of the focused beam
comet-tail artifacts. Ultrasound elastography (right panel) shows a pre-
dominantly elastic (green) pattern (arrow)
applying a small axial displacement to the tissue. Speckle-­
tracking algorithms are used to estimate the small axial dis-
placement [10–12]. The average levels of strain produced in
the tissue are usually on the order of 1%. The strain of a soft
lesion is larger than the strain of a hard lesion. Strain in the
elastography strain image (elastogram) (Fig. 1.57). Tissue
strain is encoded in a color map superimposed on a B-mode
image. The strain image can be quantified, yielding an elas-
ticity score [13].
34
Diagnosis by means of a color pattern is subjective,
although strain images can also provide numerical values
such as the strain ratio and the length ratio. The strain ratio
represents the hardness of the lesion and is defined as the
strain of the reference tissue region divided by the strain of
the lesion. A soft lesion is deformed more than the sur-
rounding tissue, and therefore its strain ratio is less than
one. A hard lesion is deformed less than the surrounding
tissue, and therefore its strain ratio is greater than one. The
length ratio, elastography image size (EI) to B-mode (B)
ratio, or EI/B ratio, is the ratio between the maximal hori-
zontal or vertical length of the lesion measured at elastog-
raphy imaging and the corresponding length measured in
B-mode imaging.
With some clinical ultrasound equipment, the probe is not
used to apply a stress but instead just touches the skin with
deformation caused by involuntary muscle contraction in the
hand of the operator and vibration caused by muscle contrac-
tion and breathing of the patient. However, adequate elastog-
raphy images cannot always be obtained for deeper lesions
with this method. The main limitation of this technique is
that the unknown stress that is applied affects the elasticity
image and consequently the elasticity score, strain ratio, and
length ratio. Another limitation is that selection of the images
for elasticity evaluation is user-dependent.
Dynamic Elastography
Dynamic elastography methods exploit the radiation force gen-
erated by the ultrasound beam. Two main techniques have been
implemented clinically: acoustic radiation force impulse (ARFI)
[14] imaging and shear wave speed imaging (SWI) [15–17].
ARFI, also known as qualitative ARFI, exploits the radia-
tion force induced by a focused ultrasound pulse of high
mechanical index (MI) (1.5–3.0) and longer duration than
B-mode pulses: 0.1–0.5 msec. At the focal spot, the radiation
force produces a displacement peak in the axial direction of
about 10–20 μm, with the tissue returning to its resting position
within about 5 msec. The axial tissue displacement is then
determined using speckle-tracking algorithms as for strain
elastography.
ARFI produces strain images of the focal spot, enabling
the imaging of lesions located at deeper depths. The same
probe is used to generate the pulse to image an ROI, a high
MI pulse to displace the tissue in the focal zone (called a
“pushing pulse”), and a second imaging pulse to record the
position of the displaced tissue. By sequentially interrogat-
ing different scan lines, a 2D strain image is created.
The “push” pulses are of longer duration than standard
diagnostic pulses, and this has the disadvantage of increasing
the acquisition time and the deposited energy in the tissue,
which can lead to heating. For this reason, images are acquired
at relatively low frame rate which reduces temporal resolution.
D.-S. Kim et al.
SWI techniques rely on the detection of shear wave prop-
agation for estimating tissue stiffness. Shear waves are easily
generated by the application of either a transient or oscilla-
tory force to deform tissue. Unlike longitudinal ultrasound
waves, however, high-frequency shear waves are highly
attenuated in tissue and cannot propagate. Low-frequency
shear waves (10 Hz–2000 Hz) are less attenuated and can
propagate in tissue, but at a much lower speed than longitu-
dinal ultrasound waves between 1 and 50 m/s. Shear wave
speed (cs) is related to the tissue shear modulus (µ), the tissue
mass density (ρ), and Young’s modulus (E) by
m E
cs = = (1.22)
r 3r
The Young’s modulus (E) is defined as the ratio between
the applied stress and the resulting strain: E = stress/strain.
Equation 1.22 provides a way for estimating tissue stiffness
based on knowledge of the shear-wave speed.
A number of techniques implemented clinically generate
shear waves inside tissue using the radiation force exerted by
a focused ultrasound beam. The deformation of tissue induced
by the radiation force at the focal depth gives rise to a low-
frequency (sonic) shear wave which propagates primarily
perpendicularly to the direction of propagation of the focused
beam (Fig. 1.56b).
For example, when determining ARFI shear wave speed
(also known as quantitative ARFI), an ROI is chosen by the
user from the B-mode image (Fig. 1.58). The user then acti-
vates a pushing pulse to generate the shear wave on one side
of the ROI, which then travels through the ROI. Time-of-
flight shear speed estimation methods using high-frame rate
conventional B-mode imaging are employed to quantify the
shear wave speed from the displacement data. These methods
estimate the time of the arrival of the wave at multiple lateral
locations and use this information to estimate the speed of the
wave propagation between the locations. The shear wave
Fig. 1.58 Hepatic fibrosis in a 12-year-old female with congenital
heart disease palliated with a Fontan procedure. Transverse 2D shear-
wave elastography ultrasound image of the right lobe of the liver
demonstrates an abnormally elevated shear-wave speed of 2.94 m/s
1 Ultrasound Imaging Techniques and Artifacts
Fig. 1.59 (a) Pushing pulses at different depths. (b) Supersonic images
(SSI) of a cylindrical shear-wave propagating in a phantom at different
times. The wavefront is distorted when passing through a harder inclu-
sion (right image) since it propagates more quickly. The gray level indi-
cates displacement in the phantom. (© 2011 Bercoff J. Published in
Bercoff [18] under CC BY 3.0 license. Available from: https://doi.
org/10.5772/19729)
speed of the ROI is displayed, from which the operator can
estimate the Young’s modulus using Eq. 1.22.
Another technique is supersonic shear wave elastography
(SSWE), which employs very high frame rates, up to 5000 Hz,
to capture 2D images of the propagating shear wave. The word
“supersonic” alludes to the way shear wave is generated. As
with ARFI, a pushing pulse is used to generate shear waves.
However, in SSWE, a number of pushing pulses are succes-
sively focused at different depths in an organ, generating a shear
wave at each focal zone. If the focus is moved faster than the
shear waves, the waves accumulate on the so-called Mach cone,
generating two plane shear waves (Fig. 1.59).
Immediately after generation of the pushing pulse, the
machine sends out an imaging sequence at very high frame
rates (5000 Hz) to catch the shear wave created by the push-
ing pulse. This imaging sequence consists of unfocused
ultrasound beams (ultrasound plane waves) that spread over
the ROI and record the resulting echoes which are used to
form an image of the ROI. From that sequence, the local
shear wave speed and thus the Young’s modulus of the tissue
are estimated.
An example of an SSWE image of a breast mass taken at
a very high frame rate (5000 Hz) is shown in Fig. 1.60,
together with the corresponding B-mode image. The Young’s
modulus (E) in kPa is shown with a color map.
Transient elastography uses a vibrator in contact with the
body to generate low-frequency spherical shear waves which
are detected by an ultrasound transducer placed on the vibra-
tor and operating at a very high frame rate.
35
Fig. 1.60 Supersonic shear-wave elastography of breast lesion. Lower
image: BI-RADS category 3 lesion (probably benign) in B-mode. Upper
image: In supersonic shear-wave elastography (SSWE), there is heteroge-
neous stiffness with a maximum Young’s modulus (E) of 180 kilopascals
(kPa) (7.7 m/s). Biopsy showed invasive ductal carcinoma. From Berg
WA, Cosgrove DO, Dore CJ, Schäfer FK, Svensson WE, Hooley RJ, et al.
Shear-wave elastography improves the specificity of breast US: the BE1
multinational study of 939 masses. Radiology. 2012;262(2):435–49. [19]
Ultrasound Contrast Imaging
Contrast Agents
Ultrasound contrast agents (UCA) are gas microbubbles
encapsulated by a thin shell made of protein, lipid, poly-
mer, or surfactant. Most UCA range in size from 2 to 6 μm
in diameter as illustrated in Fig. 1.61, and contain air or,
more commonly, a low-solubility gas such as perfluoro-
carbon. UCA underwent significant development in the
1990s for diagnostic purposes and more recently have
been investigated for potential therapeutic applications
such as facilitation of ultrasound-mediated drug and gene
delivery.
In diagnostic ultrasound, microbubbles injected intra-
venously increase the echoes from blood vessels and flow-
ing blood. Microbubbles are extremely powerful acoustic
36
s­catterers owing to their impedance mismatch, resonance,
and highly nonlinear behavior.
At low-pressure amplitudes, microbubbles produce linear
backscatter enhancement. As the power is increased, micro-
bubbles scatter nonlinearly. It is the detection of these nonlin-
ear components which forms the basis of contrast-­specific
imaging techniques such as contrast harmonic imaging, pulse
inversion imaging, and contrast Doppler, which are used to
suppress tissue signals and enhance small blood vessel echoes.
In therapy, microbubbles have been investigated for a
number of potential new applications. Ultrasound enhanced
2-6 µm
Stable Gas
Biodegradable shell
Fig. 1.61 A microbubble contrast agent is a gas microbubble encapsu-
lated by a thin biodegradable shell, ranging in size from 2–6 μm and
containing a low-solubility gas such as perfluorocarbon
Incident pulse Linear echo
t t
+ +
t t
= =
Sum, s(t)
Fig. 1.62 Diagram illustrating the principle of pulse inversion imag-
ing. (A) A sound pulse enters the body and returning echoes are received
from the contrast agent and the stationary tissues. (B) An inverted copy
of the first sound pulse is transmitted in the same direction with result-
D.-S. Kim et al.
with microbubbles increases cell membrane permeability
to large molecules (both drugs and genes) [20, 21], facili-
tates the delivery of large molecules to interstitial tissues
[22–26], and promotes blood clot lysis [27].
Ultrasound contrast agents are used to improve imaging
for applications such as echocardiography, liver, urinary
tract, and transcranial color Doppler ultrasound.
Pulse Inversion Imaging
Echoes from microbubbles contain harmonic compo-
nents because they often scatter ultrasound nonlinearly.
Harmonic imaging is therefore quite suitable for imaging
UCA. In fact, harmonic imaging was originally developed
for contrast-agent imaging under the assumption that tis-
sue emits linear echoes and microbubbles emit nonlinear
echoes.
Pulse inversion imaging was developed in order to over-
come the limitations of conventional harmonic imaging. In
conventional harmonic imaging, the image is formed from
echoes centered at twice the transmit frequency and uses
these echoes to form the grayscale harmonic image. This
approach fails when the frequency spectra of the fundamental
and second harmonic overlap with each other. The only way
to prevent overlapping is to use longer transmit pulses that
keep the spectrum bandwidth smaller. However, longer pulses
degrade axial resolution.
Pulse inversion imaging can be used even when the fre-
quency spectra overlap. The technique is illustrated in
Fig. 1.62, where one incident pulse in B has the same wave-
Nonlinear echo
t
A
t
B
t t
C=A+B
ing echoes. (C) The sum of the linear echoes from the stationary tissues
are inverted copies of each other and their sum is zero. The even nonlin-
ear components of the microbubble echoes produce a harmonic signal
when summed
1 Ultrasound Imaging Techniques and Artifacts 37

length and amplitude as the pulse in A, but its phase is exactly
inverted (180° out of phase). Upon reception, the echoes
reflected from stationary tissues are linear and cancel each
other out when added together. However, when microbubble
contrast agents are present, they generate nonlinear echoes
from harmonic scattering of moving targets.
Pulse inversion imaging isolates this nonlinear echo to cre-
ate a nonzero sum signal that depicts a real-time distribution
and concentration of the microbubbles. Since two nonlinear
echo signals are not completely out of phase, combining these
echoes results in a high-amplitude signal. Pulse inversion
imaging can be used with broadband ultrasound transmission
and detection for improved axial resolution.
Ultrasound Artifacts
Reflection earlier in this chapter). Since the machine assumes
that ultrasound travels in a straight line, the structure is dis-
played behind the interface and often at its true location as
well (Figs. 1.63 and 1.64).
Refraction
As previously discussed, refraction is a change in the direc-
tion of propagation of the incident ultrasound beam that
occurs when it strikes a tissue interface at an angle other
than 90°. As schematically shown in Fig. 1.13, refraction in
ultrasound imaging results in apparent misplacement of a
structure in an image from its true position. Refraction arti-
facts are frequent at the interface between soft tissue
(c = 1540 m/sec) and fat (c = 1450 m/sec), or between soft
tissue and fluid (c = 1480 m/sec).

An ultrasound artifact is any alteration of an image that does not

represent the anatomy being scanned. Artifacts are more com-
mon in ultrasound imaging than in other imaging modalities.
They can be related to the machine (faulty equipment, electrical
noise) and to the operator (control settings not properly set).
Certain artifacts are inevitable because of the nature of
ultrasound-tissue interactions and the assumptions made by
the machine about these interactions. Artifacts do not always
contribute negatively to image interpretation and can often
provide useful information. Understanding the cause and the
generation of various artifacts can help an operator in arriv-
ing at a correct diagnosis.
Several assumptions are made when creating ultrasound *
images that can result in image artifacts. These include the
following:

• The ultrasound wave travels at the same speed of 1540 m/

sec in all tissues.
• The transmitted wave travels in a straight path from the
transducer to the reflector and back to the transducer.
• Attenuation of sound in tissue is uniform. Fig. 1.63 Mirror image of the bladder caused by gas-filled bowel in a
• Each reflector produces only a single echo. 7-month-old male. Longitudinal grayscale ultrasound image reveals
bowel gas (arrowheads) that serves as a mirror to produce a reflection
• Beam dimensions are small in both the lateral and eleva-
(asterisk) of the bladder (B)
tional directions.
• All detected echoes originate from the central axis of the
beam. S

Grayscale Artifacts *
 irror Image: Multipath Reflection
M
Mirror image artifact (multipath reflection artifact) is pro-
duced when a structure is near a highly reflective interface
such as the diaphragm (the interface between the chest and
the abdomen), and can be imaged by reflected ultrasound. Fig. 1.64 Mirror image of the scalp caused by the skull in a 3-month-­
old male. Transverse grayscale ultrasound image of soft-tissue swelling
The geometry of reflection is simple, with the angle of inci- of the scalp (S) demonstrates a mirror image artifact (asterisk) deep to
dence equal to the angle of reflection (see discussion of the bone (arrow)
38 D.-S. Kim et al.

Two of the most common refraction artifacts are duplica-

tion refraction (ghost) artifact and refractive shadowing arti-
fact. Duplication artifact usually occurs when the ultrasound
beam is refracted from a superficial structure resulting in
apparent duplication of a deeper structure. Refractive shad-
owing artifact (edge shadowing) is caused by loss of beam
intensity due to defocusing at the edge of a cystic structure.
Refractive shadowing appears as a linear shadow that dif-
fers from the shadowing caused by significantly reduced
transmission. Refractive artifacts can be eliminated when the
structure in question is scanned from different angles or by
rotating the transducer. An example of refraction artifact is
shown in Fig. 1.65.

 everberation, Comet-Tail, and Ring-Down

R
Artifacts
Reverberation artifact is the production of multiple echoes
generated from repeated reflections between two closely
spaced interfaces. Reverberation often occurs when the
acoustic impedance mismatch between two interfaces is
high. The first transmitted pulse is reflected at the interface,
received by the transducer and displayed in the image. Some
of the echo energy returned to the transducer is reflected at
the transducer surface and is redirected back into the patient,
where it undergoes a second reflection at the same interface
and returns to the transducer to be displayed on the image as Fig. 1.66 Reverberation artifact from bowel gas in a 22-month-old male.
another echo at twice the depth. The reflection and redirec- Transverse grayscale ultrasound image of the right upper quadrant of the
abdomen reveals a bright linear echo near the ultrasound transducer
tion of the initial transmitted pulse can occur multiple times caused by intraluminal bowel gas (arrows). Repeated reflection of sound
at the same interface, and the sequence is repeated and dis- waves between the parietal peritoneum of the abdomen and gas in the
played as a series of equidistant bands of decreasing bright- bowel lumen results in multiple evenly spaced linear artifacts at increasing
ness (intensity) (Fig. 1.66). depth (arrowheads)

a b

Fig. 1.65 Rectus muscle refraction artifact in a 17-year-old male. tion (arrowheads) of the superior mesenteric artery (SMA) and aorta
Transverse grayscale ultrasound images of the mid-abdomen. When the (arrows). When the transducer is positioned to the right of the midline
transducer is positioned (a) in the midline there is an apparent duplica- (b), only a single SMA (arrowhead) and aorta (arrow) are visualized.
1 Ultrasound Imaging Techniques and Artifacts 39

The subcutaneous fat-muscle interface often generates  ide Lobe Artifact

reverberation artifacts. Two strong reflectors along a beam
path can produce a series of reverberation artifacts (comet-
tail artifact) with equally spaced bands, which is equivalent
to the space between the reflectors (Figs. 1.67 and 1.68).
Ring-down artifacts arise from from resonant vibrations
within trapped tetrahedrons of air bubbles. They produce a
continuous sound wave that appears as a series of parallel
bands or as a streak deep to a focus of gas such as a pneumo-
peritoneum (Fig. 13.69a).
S
Side lobes are inevitable and present with all transducers.
Piezoelectric elements expand and contract not only in a
thickness direction but also in a radial direction. Side lobes
are emissions of low-amplitude energy that project radi-
ally from the axis of the main ultrasound pulse and are
caused by the radial expansion and contraction of the
piezoelectric elements. Side lobe artifact is caused by
echoes received from the side-lobe energy, which are
incorrectly placed on the image of the main beam because
the transducer interprets all echoes as if they originated
from the main beam.
The intensity of side lobes is so weak (less than 10% of the
main beam intensity) that they do not usually generate visible
artifacts on soft tissue imaging. However, side lobe artifacts
become more visible when a highly reflective object is located
outside the main beam plane, such as a biopsy needle, or gas
in the bowel that appears as a ghost image in the urinary blad-
der in the displayed image. An ex An example of refraction
artifact ample of side lobe artifact is shown in Fig. 1.69.

Enhancement and Shadowing Attenuation

Artifacts
Enhancement and shadowing attenuation are the most promi-
nent and useful artifacts in ultrasound imaging, frequently aid-
ing in the characterization of a variety of lesions. Attenuation of
Fig. 1.67 Comet-tail artifact arising from the gallbladder in a 3-month-­ ultrasound depends on the tissue through which it propagates.
old male with cholesterolosis. Longitudinal grayscale ultrasound image Enhancement artifact, also called increased through-­
reveals numerous foci of reverberation artifact (arrowheads) along the
transmission, appears when a focal lesion has a lower attenua-
inner aspect of the anterior gallbladder wall. These are due to the pres-
ence of cholesterol crystals that are highly reflective of the ultrasound tion than the surrounding tissue. The result is an area of increased
beam

Fig. 1.68 Comet-tail artifact arising from inspissated colloid in a thy-
roid nodule of a 11-year-old female. Transverse grayscale ultrasound
image of the right thyroid lobe reveals a small hypoechoic nodule con-
taining a central echogenic focus with posterior reverberation artifact
(arrow)
Fig. 1.69 Side-lobe artifact in the gallbladder of a 21-month-old male.
Longitudinal grayscale ultrasound image shows intraluminal echoes
(arrow) from an adjacent gas-containing loop of bowel (arrowhead)
40 D.-S. Kim et al.

brightness behind a hypoechoic structure. Cysts and other fluid-

filled structures such as the gallbladder are less attenuating than
the surrounding soft tissues, so that the more distal tissues often
produce brighter signals than those generated by similar adja-
* cent tissues at the same depth. The degree of enhancement
(brightness) can be controlled using TGC. Examples of enhance-
ment artifact are shown in Figs. 1.70 and 1.71.
Shadowing artifact appears as an area of decreased
brightness behind a strongly attenuating structure such as a
calcification, metallic foreign body, bone, and some solid
masses. This artifact is caused by significant attenuation of
the ultrasound by either absorption in the structure or reflec-
tion at an interface, and results in little transmission of
sound, leaving a long dark shadow posterior to the structure.
Examples of shadowing artifact are shown in Figs. 1.72,
1.73, and 1.74.

Partial Volume Artifact

Partial volume artifact is also referred to as slice thickness
artifact or beam width artifact, depending on the angulation

Fig. 1.70 Enhancement artifact associated with a breast cyst in a

16-year-old female. Longitudinal grayscale ultrasound image of the
breast demonstrates a cyst (asterisk). There is a relative increase in the
intensity of the echoes posterior to the cyst (arrows) compared to the
adjacent soft tissues

Fig. 1.71 Enhancement artifact associated with a Wilms’ tumor in an Fig. 1.72 Shadowing artifact from a kidney stone in a 16-year-old
8-month-old male. Longitudinal grayscale ultrasound image of the male. Transverse grayscale ultrasound image of the right kidney shows
right kidney demonstrates a solid upper pole mass (asterisk). Increased clean shadowing (arrow) deep to a large stone (asterisk)
intensity of echoes posterior to the mass (arrows) relative to the adja-
cent soft tissues is identified. The homogeneity of the solid mass results
in a lower attenuation of the sound waves compared to the neighboring
soft tissues
1 Ultrasound Imaging Techniques and Artifacts
of the transducer relative to the scan plane. Partial volume
artifact can appear in either an in-plane direction (lateral)
or at 90° to the scan plane (slice thickness). This artifact is
caused by simultaneous sampling of tissues with different
acoustic properties when scanned with a beam width that is
greater than the dimensions of the structure. It appears in
Fig. 1.73 Shadowing artifact due to bowel gas in a 17-year-old male.
Longitudinal grayscale ultrasound image of the left upper quadrant
demonstrates dirty shadowing (arrows) posterior to a gas-filled bowel
loop (asterisk). The artifact is caused by sound wave reflections at the
gas-­soft-­tissue interface
a b
Fig. 1.75 Partial volume artifact of the bladder in an 8-day-old male.
(a) Transverse grayscale ultrasound image reveals pseudo-­ sludge
(arrows) in the posterior aspect of the bladder. (b) Transverse grayscale
41
the image as pseudo sludge or as echoes in an otherwise
echo-­free area.
A beam width artifact can be reduced by optimal posi-
tioning of the focal zone. A slice thickness artifact is inte-
gral to the transducer and cannot be entirely eliminated. An
example of partial volume artifact is shown in Fig. 1.75.
Doppler Artifacts
Artifacts in both spectral and color flow Doppler imaging are
often classified as technically related and anatomically related
artifacts.
Fig. 1.74 Shadowing artifact associated with renal angiomyolipoma in
a 13-year-old female. Longitudinal grayscale ultrasound image shows a
large echogenic mass (asterisk) in the lower pole of the right kidney.
There is posterior acoustic shadowing (arrows) related to absorption of
sound waves by fat in the tumor
ultrasound image obtained after repositioning of the focal zone closer to
the posterior wall of the bladder resulted in complete elimination of the
artifact
42
a Technically Related Doppler Artifacts
b
c The Doppler PRF (number of pulses emitted per second),
Fig. 1.76 Effect of gain settings on depiction of flow in the carotid
artery of a 17-year-old male. (a) Longitudinal color Doppler ultrasound
image obtained with a low gain setting (arrowhead) results in reduced
detection of blood flow. (b) Color Doppler ultrasound image obtained
with a high gain setting (arrowhead) leads to excessive electronic noise
in the image (arrow). (c) Color Doppler ultrasound image with an
appropriate gain setting (arrowhead) results in satisfactory depiction of
uniform flow throughout the vessel
a b
Fig. 1.77 Effect of wall filter settings on the depiction of renal blood
flow in a 14-year-old female. (a) Longitudinal color Doppler ultrasound
image of the right kidney obtained with a low filter setting (arrowhead)
demonstrates color signal throughout the renal parenchyma. Depiction
of the vasculature is limited by prominent color noise. (b) Image of the
right kidney obtained with a high filter setting (arrowhead) has reduced
D.-S. Kim et al.
Inappropriate Doppler Settings
The most important controls for correctly displaying blood flow,
especially for slow flow, are the gain, Doppler PRF (also termed
velocity scale), filtration setting, and transducer focal depth.
When the gain is too low (Fig. 1.76a), the very weak
echoes from blood are too small to be detected. If the gain
is too high, excessive electronic noise becomes visible in
the Doppler image (Fig. 1.76b). The spectral Doppler gain
setting should be adjusted so that the velocity envelope is
thin, and flow is seen in a uniform direction (Fig. 1.76c).
It is appropriate to adjust the color Doppler gain setting
by increasing it to the point at which noise starts to appear in
the image and then decreasing it until the noise just clears
from the image or is barely noticeable.
also called the velocity scale on some ultrasound machines,
should be set as low as possible until aliasing, noise, or tissue
motion (discussed below) becomes apparent.
The Doppler signal contains not only the low-amplitude,
higher Doppler frequencies from blood, but also the high-­
amplitude, lower Doppler frequencies from slow-moving tis-
sues such as the vessel walls.
The wall filter controls the ability to distinguish moving
blood from moving tissue by removing the low Doppler fre-
quencies while retaining the higher frequencies. There is,
however, a compromise in the selection of the cut-off fre-
quency to be used, as it is important not to suppress the low
frequencies associated with slow velocity flow. A low filter
setting increases the likelihood of low-velocity flow detec-
noise but prevents visualization of the smaller peripheral parenchymal
vessels. (c) Image of the right kidney obtained several months earlier
with a medium filter setting (white arrowhead) reveals peripheral paren-
chymal vessels (black arrowhead), as well as satisfactory depiction of
the larger central vessels. Incidentally noted is a prominent color Doppler
twinkling artifact (arrow) associated with a renal pelvic stone
1 Ultrasound Imaging Techniques and Artifacts
tion, but also contains noise (Fig. 1.77). A high-filter setting
suppresses low-frequency shifts arising from slowly flowing
blood.
The Doppler transmit frequency to be used depends on
the depth of the vessel. A high transmit frequency is more
sensitive for detecting blood flow at the expense of penetra-
tion. If penetration is an issue, a lower transmit frequency
should be chosen.
Aliasing (Wraparound)
For a given Doppler PRF (velocity scale), there is a limit to
the maximum Doppler frequency shift that can be estimated
and visualized correctly. This limit is known as the Nyquist
PRF
limit and is half the Doppler PRF: fD = . Since flow
2 by aliasing, is often used instead of PW Doppler. A major
velocity is proportional to the Doppler frequency shift
(Eq. 1.20), increasing or decreasing the PRF (velocity scale)
will increase or decrease the range of frequencies, and there-
fore the displayed velocities.
The Nyquist limit is related to the ability of the Doppler
processor to process the Doppler signal. If the Doppler fre-
quency shift exceeds the Nyquist limit, the Doppler proces-
sor cannot reconstruct the signal correctly because the signal
sampling rate is too low. Aliasing occurs for both PW
Doppler and color Doppler when the Doppler shift frequen-
PRF
cies exceed the Nyquist limit ( ).
2 the range of frequencies can increase, particularly when the
In PW Doppler, aliasing can be seen as waveform peak
cutoff with the peaks displaced below the baseline (Fig. 1.78).
With color Doppler, aliasing can cause an abrupt change in
color suggesting incorrectly that the direction of flow has
a b
Fig. 1.78 Pulsed wave (PW) Doppler ultrasound depiction of aliasing
in the carotid artery of a 17-year-old male. (a) Longitudinal color and
spectral Doppler ultrasound image of the carotid artery shows a low
pulse repetition frequency (PRF; arrowhead) with aliasing of the arte-
43
changed (Fig. 1.79). A true flow reversal is characterized by adja-
cent areas of dark red and dark blue. Color aliasing is a valuable
artifact because it displays regions of high-­frequency Doppler
shifts, which could potentially be sites of abnormal flow.
Increasing the Doppler PRF can eliminate or reduce alias-
ing but at the expense of depth of penetration. If that is not
possible, using a lower transducer frequency reduces the
Doppler frequency shift, which can help to decrease or elimi-
nate aliasing. Increasing the Doppler angle can also reduce
the Doppler shift. However, this option is often not feasible
because the acoustic window to the vessel is either too lim-
ited to allow angle variation, or because the angle is already
close to 60° and cannot be increased any further.
In echocardiography, CW Doppler, which is not affected
drawback is its relatively large sample volume.
Color Doppler Noise
Noise is often related to inappropriately high Doppler gain. A
spectral gain setting that is too high degrades the velocity
envelope, causing it to appear thickened. This appearance
mimics spectral broadening.
Spectral broadening is an important diagnostic indicator
and has two causes. One is due to the fact that the Doppler
sample volume contains a range of velocities, so the Doppler
signal has a range of frequencies. In the presence of disease,
blood flow becomes turbulent (e.g., post-stenotic turbu-
lence). The other cause is intrinsic spectral broadening.
The ultrasound beam used to acquire the Doppler signal is
generated by a group of ultrasound elements. Therefore, the
rial systolic peaks that are displaced below the baseline. (b) Spectral
Doppler evaluation of the carotid artery after increasing the PRF results
in an appropriate display of the arterial systolic peaks above the base-
line in the same direction as the rest of the arterial waveforms
44
a b
Fig. 1.79 Color Doppler aliasing in a 4-year-old male with mid-aortic
syndrome. (a) Longitudinal grayscale ultrasound image shows marked
focal thickening of the wall of the mid-abdominal aorta (arrows) with
significant luminal narrowing. (b) Longitudinal color Doppler ultra-
Fig. 1.80 Effect of insonation angle on the depiction of color Doppler
flow in a 14-year-old female with heterotaxy and a left-sided inferior
vena cava (IVC; arrows). Transverse color Doppler ultrasound image
demonstrates a small colorless segment (arrowheads) in the vascular
lumen where the interrogating ultrasound beam intersects the vessel at
a 90-degree angle
Doppler signal is acquired by ultrasound traveling along
multiple paths. For each of these paths, the Doppler angle is
different, and so there will be a different Doppler shift. The
overall Doppler signal contains a combination of all these
Doppler shifts resulting in spectral broadening.
When the color gain setting is too high, color signal
appears throughout the image as a random admixture of red
and blue (i.e., color speckles) rather than having a homoge-
neous color, which is typical of flow within a vessel. Any
structure that moves can also cause a random Doppler shift.
D.-S. Kim et al.
sound image reveals aliasing as foci of light blue color assignment
(arrowheads) in the zone of luminal narrowing. Unlike true flow rever-
sal, there is no separation of the red and blue hues by a black region
without flow
Rapid movement of the transducer is another cause of a
Doppler shift and artifactual color signal. Power Doppler is
particularly sensitive to artifactual motion, such as move-
ment of the transducer.
Flow Directional Abnormalities
The detected Doppler shift frequencies are dependent on the
cosine of the angle of insonation (θ). As the Doppler angle
approaches 90°, the Doppler signal can be very small and
therefore may be removed by the wall filter. Flow direction
can also be inappropriately displayed if the interrogating ultra-
sound beam intersects the vessel at a 90° angle.
If a curved or phased array is used, every beam used to
generate the image has a different angle and therefore a differ-
ent Doppler angle. Hence, a range of color will be seen with a
small colorless segment in the lumen of the vessel (Fig. 1.80).
Anatomically Related Doppler Artifacts
Spectral Mirror Image Artifact
This artifact, already discussed for grayscale imaging, also
occurs in Doppler imaging. As with grayscale imaging, Doppler
mirror images arise close to strongly reflecting interfaces which
act as mirrors, causing a tissue or structure located above the
interface to be replicated below the interface. If the region that is
being mirror-imaged contains blood vessels, they will be dis-
played in the mirror image as shown in Figs. 1.81 and 1.82.
Because the artifactual signal is generated by blood flow in
a real vessel but is simply improperly positioned, it usually has
the same size and shape as the signal from the true vessel. Its
intensity, however, is often weaker. This is because the artifac-
tual signal arises from the sound reflected off the mirror which
1 Ultrasound Imaging Techniques and Artifacts 45

A V

Fig. 1.81 Mirror image artifact of the subclavian artery in a 21-year-­

old female with thoracic outlet syndrome. Longitudinal power Doppler
ultrasound image shows the left subclavian artery (A) with a mirror
image (arrowheads) located inferiorly. The interface between the vessel
lumen and the underlying lung tissue acts as a mirror in reflecting the
incident sound

Fig. 1.82 Mirror image artifact of the right subclavian vein in an

is weaker than the Doppler signal from the true vessel that 18-year-old male. Longitudinal color Doppler ultrasound image depicts
arises from the original sound pulse. the normal right subclavian vein (V) with a mirror image (arrowheads)
Scanning from an angle that excludes the source vessel or located inferiorly
decreasing the power output and Doppler gain settings should
eliminate or reduce the mirror image. Occasionally, weaker
acoustic interfaces will act as mirrors for color Doppler imag-
ing. For example, bone can reflect enough sound to produce
color Doppler mirror images.

Tissue Vibration Artifact

Turbulent blood flow causes pressure fluctuations in the A
lumen of the vessel and vibration of the vessel wall, which
generates a detectable Doppler signal. This signal is dis-
played as focal random red and blue color patches in the V
perivascular space at the site of the abnormal vessel. The
artifact is most prominent during systole when the velocities
are greatest, and less prominent during diastole.
Waveforms from vibrating tissues are typically strong,
low-frequency signals that are symmetric above and below
the baseline. Perivascular tissue vibration artifact can be seen
in any situation where there is sufficiently turbulent blood
flow. The commonly encountered lesions include arteriove-
nous fistulas (AVF), stenotic arteries, aneurysms, pseudoan- Fig. 1.83 Perivascular tissue vibration from an arteriovenous fistula in
eurysms, and vascular anastomotic sites (Fig. 1.83). a 52-year-old female with recent cardiac catheterization for congenital
heart disease. Longitudinal color Doppler ultrasound image shows a
fistulous connection (arrows) between the common femoral artery (A)
Twinkling Artifact
and vein (V) with adjacent tissue vibration artifact (arrowheads) mani-
Small calcified and crystalline structures can produce echoes fested as a mixture of blue and red speckles
that mimic movement. With color Doppler, they typically
appear as a random mixture of red and blue colors fluctuating
in time (Fig. 1.84). For this reason, this artifact is known as The twinkling artifact has been exploited most commonly
“twinkling” artifact. The associated waveforms show a high-­ in the detection of urinary tract stones. The twinkling artifact
intensity, non-physiologic signal with aliased components on from urinary stones is likely generated by a random strong
both sides of the baseline. The artifact is typically accentuated reflection and multiple inner reflections of the incident pulse
at low transmit frequencies and high Doppler scale settings. at the rough interface formed by the crystalline aggregate.
46
Fig. 1.84 Color Doppler twinkling artifact associated with a renal
stone in a 23-year-­old male. (a) Longitudinal grayscale ultrasound
image reveals a small echogenic focus (arrow) in the lower pole of the
left kidney without posterior acoustic shadowing. (b) Longitudinal
Fig. 1.85 Blooming artifact in a 2-month-old premature infant with
vena caval thrombi. (a) Longitudinal grayscale ultrasound image reveals
two linear echogenic thrombi (arrows) in the IVC. (b) Longitudinal
Although the twinkling artifact is most often used to aid in
detection of small renal calculi, it is also helpful in the set-
ting of ureteral and bile duct stones, pancreatic calcifications,
and foreign bodies. This artifact may be easier to detect than
acoustic shadowing and is useful in stone identification.
Blooming Artifact
When the ultrasound beam is centered on soft tissue that is
within or immediately adjacent to a blood vessel, the beam
D.-S. Kim et al.
color Doppler ultrasound image demonstrates a twinkling artifact
(arrowheads) with alternating color signals extending posterior to the
site of the echogenic focus in the lower renal pole
color Doppler ultrasound image displays color signal in the IVC that
completely obscures the thrombi
may still detect the Doppler signal from the vessel. This
occurs because the spatial resolution of color Doppler is
lower than that of grayscale. The resulting image will dis-
play color Doppler signal in the pixels corresponding to
the soft tissue, thus masking underlying grayscale infor-
mation (Fig. 1.85). This can obscure abnormalities within
the vessel lumen, such as nonobstructive thrombus or arte-
rial plaques, as well as lesions in the tissues around the
vessel.
1 Ultrasound Imaging Techniques and Artifacts
Three-Dimensional Ultrasound Artifacts
3D imaging cannot fix the 2D ultrasound artifacts covered in
Sections “Gray-Scale Artifacts” and “Doppler Artifacts”. If
some artifacts are present in the 2D images, they will be also
present in the volume of echo data. However, the artifacts
present in the 2D images may be visible in a 3D image with-
out the presence of the causative structure. This is because
their presence may not be available in a specific image but
will be expressed throughout the volume and present in mul-
tiple planes and rendered images. For example, the rendering
of a shadow from an overlying structure such as bone or a
calculus may be seen as a hollow void on a 3D image without
the representation of the bone or calculus itself. Therefore, it
is important to interpret 3D images in combination with 2D
imaging to identify the origin of the artifact.
There are artifacts that are unique to 3D volume acquisi-
tion and display processing. Manual acquisition of a volume
of echo data by sliding the transducer can result in artifacts
related both to the varying speed of transducer movement
and some types of patient motion (respiration, cardiac
motion, vessel pulsation, general patient movement) that
occurs during the relatively long acquisition time. This can
result in data volumes which do not correctly reproduce
some of the object dimensions. Automatic volume acquisi-
tion with newer ultrasound probes has overcome this issue to
a large extent.
The post-processing of the volume of echo-data is quite
complex and can generate several artifacts. The quality of the
rendered images often depends on the choice of the render-
ing parameters such as threshold, opacity, lighting, and so
on. The threshold parameter, which is used to define the level
of gray voxels to be visualized, is a particularly important
parameter. Excessive thresholding can remove part or all of
an important structure. When the threshold parameter is set
appropriately, it can minimize or eliminate noise and improve
image quality.
Ultrasound Contrast Agent Artifacts
Blooming Artifact
In color Doppler mode, the injection of contrast agents can
accentuate the blooming artifact described in Section
“Doppler Artifacts.” It occurs soon after the administration
of contrast agent at the time of peak enhancement. As the
contrast agents enters a vessel, the magnitude of the echo
increases with a correspondingly large increase in Doppler
signal. This increase in signal can expand the width of the
vessel, causing “blooming.”
A slow injection limits blooming artifact because it
decreases peak signal intensity.
47
 ystolic Peak Velocity Increase
S
An artifactual increase in systolic peak velocity of up to 50%
can occur at the time of contrast peak enhancement, reflect-
ing the limited dynamic range of the system. This artifact can
be minimized by reducing the Doppler gain and using a slow
injection. The increased systolic velocity can potentially
result in overestimation of the degree of a stenotic lesion if
contrast images are interpreted without conventional color
Doppler images. The presence of flow disturbances on the
color Doppler images should help in grading stenotic lesions.
High-Intensity Transient Signals
High-intensity transient artifacts can be seen with pulsed
Doppler at the time of peak contrast enhancement or during
late enhancement. They can also be detected with color and
power Doppler ultrasound as higher-intensity color pixels
within the more uniform background of the vessel.
Ultrasound Safety
Ultrasound imaging does not produce carcinogenic ionizing
radiation and has an outstanding safety record. However,
ultrasound strongly interacts with tissues and as a result may
cause harm by increasing their temperature and through the
exertion of mechanical forces. The changes that ultrasound
can cause to a tissue are classified as thermal and nonthermal
bioeffects [27–29].
Thermal Bioeffects
The thermal bioeffects of ultrasound are primarily affected
by the ultrasound frequency, beam intensity, transmit power
(energy radiated by the transducer every second), scanning
mode, and tissue absorption properties. As the ultrasound
propagates through tissue, some energy is absorbed and con-
verted into heat. Absorption increases with frequency and
beam intensity.
The absorption properties of the tissue also affect heating.
Blood absorbs much less energy than bone or lung tissue.
Other soft tissues fall in between with muscle having the high-
est absorption. Blood flow also affects tissue heating because
it carries away heat. Well-perfused tissues heat up significantly
less than poorly perfused tissues. Interfaces between soft tis-
sue and bone are a particular concern as a large part of the
ultrasound energy is reflected at these interfaces. This can
cause substantial localized heating at these interfaces.
Another important factor is whether the beam is scanned
through the tissue, as it is in B-mode and color Doppler
imaging, or whether it is stationary, as with PW Doppler and
CW Doppler. When the beam is scanned, the heat is spread
through a larger volume of tissue, reducing temperature
48
increase. B-mode imaging is generally safe since it uses the
lowest exposure parameters. Doppler imaging requires a
higher transmit power than B-mode imaging to overcome
low scattering from blood and therefore potentially poses
more risk to the patient than B-mode imaging.
PW Doppler has the highest risk of substantial tissue
heating because it requires a higher transmit power, and the
beam is kept stationary during acquisition. Even though the
beam is kept stationary for CW Doppler, the risk is less
because the pressure amplitude and therefore the intensity
(which is proportional to the pressure amplitude squared)
are kept lower than for PW Doppler. CW Doppler is a sim-
pler technique than PW Doppler, and requires less transmit
power than PW Doppler to achieve a comparable echo
signal.
The thermal index (TI) was developed to estimate the
increase in temperature that might occur during an ultrasound
scan. It is essentially a measure of the likely maximum tem-
perature rise of the tissue during the scan. A TI of 0.9 implies
an expected temperature rise of about 0.9 °C. TI is defined as
the ratio of the acoustic power produced by the transducer
(W) to the power required to raise the tissue temperature by
1 °C. Bone absorbs more heat than soft tissue.
Two TIs have been defined for bone: (1) bone TI (TIB)
that takes into account the extra heating that occurs at bone-
soft tissue interfaces; and the cranial TI (TIC) when the
probe is placed in proximity to the patient’s skull.
Nonthermal Bioeffects
Cavitation is the most important nonthermal bioeffect of
ultrasound. Acoustic cavitation describes the interaction of
gas bubbles with a sound field. At low pressure amplitude,
bubbles undergo stable cavitation, i.e., they oscillate, often
nonlinearly, around an equilibrium position. They remain
relatively stable in solution, and may oscillate for many
cycles of the acoustic pressure. As the pressure amplitude is
further increased, cavitation can become inertial. Inertial
cavitation is characterized by the sudden expansion and
rapid collapse of bubbles. The bubbles may fragment or
repeat the growth/collapse cycle a number of times.
Bubble collapse can create extremely high localized tem-
peratures in the gas contained inside a contrast microbubble,
while in the liquid surrounding the microbubble they can
generate shock waves that produce harmful bioeffects.
Inertial cavitation can also generate free ­ radicals within
microbubble gas and at gas-liquid interfaces that can alter
biomolecules such as DNA, proteins, and lipids.
Cavitation is important because gas bubbles occur in areas
such as the lungs and gastrointestinal tract. In addition, the
D.-S. Kim et al.
risk of inertial cavitation increases as the peak negative pres-
sure increases. The peak negative pressure, also known as
rarefaction peak pressure, is the most negative pressure that
occurs during an ultrasound pulse. In water, a sufficiently high
negative pressure can pull apart molecules and create a gas
bubble. If there are preexisting gas bubbles or dust particles in
the water, cavitation also occurs more easily. A similar effect
can also occur in tissue, especially if there are preexisting gas
bubbles.
The likelihood of inertial cavitation increases with
decreasing ultrasound frequency. The MI quantifies the like-
lihood that exposure to diagnostic ultrasound will produce an
adverse bioeffect by inertial cavitation. The MI is defined as
the estimated peak rarefactional pressure in vivo, Pr, divided
by the square root of the center frequency f of the beam:
Pr
MI = (1.23)
f
Regulations and Policies
Ultrasound equipment sold in the United States (US) must
meet the US Food and Drug Administration (FDA) regula-
tions requiring that manufacturers supply information on
acoustic power output and ensure that certain acoustic
parameters do not exceed allowable levels. In 1991, The
FDA set new regulations for the output of diagnostic ultra-
sound that has increased permissible output intensity for
fetal, neonatal, cardiac, and ophthalmological applications.
These new regulations superseded earlier ones established
in 1976, which were more restrictive.
Manufacturers generally provide real-­time on-screen dis-
play of acoustic power as Output Display Standard (ODS),
as defined by the American Institute of Ultrasound in
Medicine (AIUM) and the National Electrical Manufacturers
Association (NEMA). Two main acoustic parameters of the
ODS are the TI and MI as discussed above. Manufacturers
are required to display the TI and MI during the scan when-
ever the probe-machine combination is capable of producing
a value for either index greater than 1.0. In general, the fol-
lowing upper limits should be observed during an ultrasound
scan: MI <1.9 and TI < 1.5.
In addition, a number of professional organizations offer
guidelines and advice regarding safety and bioeffects, includ-
ing the AIUM, the National Council on Radiation Protection
and Measurement (NCRP), the World Federation for
Ultrasound in Medicine and Biology (WFUMB), and the
European Federation of Societies for Ultrasound in Medicine
and Biology (EFSUMB).
1 Ultrasound Imaging Techniques and Artifacts 49

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2015;41(1):187–96.
12. Ophir J, Alam SK, Garra BS, Kallel F, Konofagou EE, Krouskop
27. O'Brien WD Jr. Ultrasound-biophysics mechanisms. Prog Biophys
T, et al. Elastography: imaging the elastic properties of soft tissues
Mol Biol. 2007;93(1–3):212–55.
with ultrasound. J Med Ultrason (2001). 2002;29(4):155.
28. Nyborg WL. Biological effects of ultrasound: development of
13. Itoh A, Ueno E, Tohno E, Kamma H, Takahashi H, Shiina T, et al.
safety guidelines. Part II: general review. Ultrasound Med Biol.
Breast disease: clinical application of US elastography for diagno-
2001;27(3):301–33.
sis. Radiology. 2006;239(2):341–50.
29. Dalecki D. Mechanical bioeffects of ultrasound. Annu Rev Biomed
14. Nightingale KR, Palmeri ML, Nightingale RW, Trahey GE. On
Eng. 2004;6:229–48.
the feasibility of remote palpation using acoustic radiation force. J
Acoust Soc Am. 2001;110(1):625–34.
 Brain
2
Helen H. R. Kim, Wendy G. Kim, Edward Y. Lee,
and Grace S. Phillips

IVH Intraventricular hemorrhage

Abbreviations LM Lymphatic malformation
MCA Middle cerebral artery
3D Three-dimensional MR Magnetic resonance
AHW Anterior horn width NAIS Neonatal arterial ischemic stroke
AIS Acute ischemic stroke PIVH Periventricular/intraventricular hemorrhage
AVF Arteriovenous fistula PVHI Periventricular hemorrhagic infarction
AVM Arteriovenous malformation PVL Periventricular leukomalacia
BEH Benign external hydrocephalus SIDS Sudden infant death syndrome
CM Capillary malformation STOP Stroke Prevention in Sickle Cell Anemia
CMV Cytomegalovirus TAMMX Time-averaged mean of the maximum
CNS Central nervous system velocity
CSF Cerebrospinal fluid TCD Transcranial Doppler
CT Computed tomography TOD Thalamo-occipital distance
DVA Developmental venous anomaly VI Ventricular index
ECMO Extracorporeal membrane oxygenation VM Venous malformation
Hib Haemophilus influenza type b VOGM Vein of Galen Malformation
HIE Hypoxic ischemic encephalopathy
HII Hypoxic ischemic injury
HIV Human immunodeficiency virus
HSV Herpes simplex virus Introduction
IHW Interhemispheric width
ISSVA International Society for the Study of Cranial ultrasound is a valuable screening and diagnostic
Vascular Anomalies examination with distinct advantages compared to alterna-
tive imaging modalities, particularly in the young child.
These advantages include easy accessibility, relatively low
H. H. R. Kim cost, no radiation, and a short examination time.
Department of Radiology, Seattle Children’s Hospital,
University of Washington School of Medicine, Seattle, WA, USA
In this chapter, up-to-date imaging techniques, anatomy,
embryology, and disorders of the brain commonly encoun-
W. G. Kim
Department of Radiology, Boston Children’s Hospital and
tered in clinical practice are discussed. Rare and challenging
Harvard Medical School, Boston, MA, USA but important diagnoses are also presented along with limita-
E. Y. Lee
tions of cranial ultrasound and common pitfalls. A clear
Division of Thoracic Imaging, Department of Radiology, understanding of the imaging techniques and abnormalities
Boston Children’s Hospital and Harvard Medical School, detected by cranial ultrasound is of great utility in the care of
Boston, MA, USA
G. S. Phillips (*)
Department of Radiology, Seattle Children’s Hospital, University
of Washington, Seattle, WA, USA
e-mail: grace.phillips@seattlechildrens.org

© Springer Nature Switzerland AG 2021 51

H. J. Paltiel, E. Y. Lee (eds.), Pediatric Ultrasound, https://doi.org/10.1007/978-3-030-56802-3_2
52
pediatric patients with congenital and acquired cranial
disorders.
Technique
Cranial ultrasound is the imaging modality of choice to screen
for intracranial pathology in the young child. The advantages of
ultrasound include easy accessibility, portability allowing for
bedside imaging in critically ill patients, noninvasiveness, lack of
radiation exposure, lack of sedation, and relatively low cost.
Disadvantages of ultrasound compared to other imaging modali-
ties such as computed tomography (CT) and magnetic resonance
(MR) imaging are operator dependence and limited assessment
of some intracranial structures, especially the extra-axial spaces
along the convexities of the brain and the posterior fossa.
Sound waves penetrate osseous structures poorly; there-
fore, ultrasound imaging of the brain needs to be performed
through acoustic windows such as the anterior and posterior
fontanelles, the temporal window, mastoid fontanelle, and
the foramen magnum.
The most common acoustic window used in clinical prac-
tice is the anterior fontanelle. It is the widest window and can
be easily localized on physical examination as a soft spot
along the anterosuperior aspect of the skull in the newborn.
The anterior fontanelle begins to close at approximately
9 months of age and is completely fused by the end of the
second year of life [1]. It closes later in patients with prema-
turity, trisomy 13, trisomy 18, trisomy 21, and/or underlying
poor bone mineralization from conditions such as achondro-
plasia, osteogenesis imperfecta, rickets, congenital hypothy-
roidism, and increased intracranial pressure [2].
The posterior fontanelle closes much earlier than the anterior
fontanelle, typically by 2–3 months of age [3]. The trans-­
mastoid approach behind the ear is generally accessible until the
age of 1.5 years.
Patient Positioning
Cranial ultrasound is often performed at the bedside in the neo-
natal intensive care unit. When imaging through the anterior and
posterior fontanelles, the infant is examined in a supine position.
When imaging through the mastoid fontanelle, the head is
turned either to the right or to the left and the ultrasound trans-
ducer is placed directly behind the ear. For transcranial Doppler
(TCD) ultrasound studies, the patient is examined in either a
supine position or sitting upright. Images are obtained anterosu-
perior to the ear through the temporal bone.
H. H. R. Kim et al.
Ultrasound Transducer Selection
Optimal intracranial images are obtained with a small foot-
print, high-frequency phased array transducer with fre-
quencies on the order of 5–8 MHz. A sector field of view
allows deeper penetration through a small acoustic window
and the ability to change the focus of the ultrasound beam.
In premature babies, a 7–10 MHz or higher phased array
transducer can be used [4]. In term babies and young infants,
a 3.5–5-MHz phased array transducer may be necessary [5]. A
7.5–12-MHz high-frequency linear array transducer is used to
assess superficial structures in a midsagittal plane, such as the
superior sagittal sinus and the extra-axial spaces, as well as the
subcutaneous layer of the scalp. In addition to static grayscale
images, cine clips are especially helpful for problem-solving,
especially when images are obtained portably in the neonatal
intensive care unit when the physician responsible for image
interpretation may not be physically present.
Color and pulsed Doppler ultrasound imaging is useful in
assessing the intracranial vasculature and in the evaluation of
vascular anomalies. Three-dimensional (3D) ultrasound of
the brain permits single sweep volumetric data acquisition.
This technology offers the possibility of reconstructing
images in different planes to further aid in the diagnosis of
pathology of the neonatal brain [6].
Imaging Approaches
A standardized cranial ultrasound protocol should be followed
in order to optimize the quality of examination and to ensure
consistency of imaging between studies (Fig. 2.1). A series of
coronal and sagittal images are obtained through the anterior
fontanelle. Additional information can be obtained as needed
by acquiring images through the posterior fontanelle, tempo-
ral bone, squamosal suture, mastoid fontanelle, and foramen
magnum at the posterior craniocervical junction.
Centering the probe over the fontanelle is essential for the
acquisition of symmetric images of the cerebral hemispheres.
Multiple coronal images are obtained by angling anteropos-
teriorly with a rocking motion while maintaining contact
with the anterior fontanelle.
A minimum of seven coronal images are obtained from
anterior to posterior as follows (Figs. 2.2a–g): (a) the first
image is acquired of the frontal cortex, anterior to the anterior
horns of the lateral ventricles; (b) the second image is through
the anterior horns of the lateral ventricles and includes the
suprasellar cistern at the skull base; (c) the third image is
through the body of the lateral ventricles at the level of the
2 Brain
BV
A G
B OH
FH
FH C
D 3 F J
E
TH
CB
4
Fig. 2.1 Standard images of the brain are obtained in a series of coronal
(line A-G) and sagittal (line H-K) planes through the anterior fontanelle.
Each line corresponds to the following ultrasound images (Fig. 2.2a–k). FH,
Frontal horn; BV, body of lateral ventricle; OH, occipital horn; TH,
paired foramina of Monro with the brainstem in the far-field;
(d) the fourth image is through the posterior aspect of the third
ventricle; (e) the fifth image is at the level of the quadrigemi-
nal cistern; (f) the sixth image is through the atria of the lateral
ventricles with visualization of the choroid plexus in each lat-
eral ventricle; (g) the seventh and final image is through the
parieto-occipital lobes, posterior to the lateral ventricles.
Sagittal images are acquired by placing the probe parallel to
the sagittal suture across the anterior fontanelle. Multiple sagit-
tal images are obtained by angling laterally from the midline to
each side (Fig. 2.2h–k). The first sagittal image (Fig. 2.2h)
obtained in the midline demonstrates the corpus callosum; the
comma-shaped, fluid-filled cavum septum pellucidum between
the lateral ventricles; and the cingulate gyrus above the corpus
callosum. The normal sulci and gyri do not extend to the ven-
tricles due to the presence of the cingulate gyrus. The tectum of
the midbrain and the anechoic fourth ventricle outlined by echo-
genic cerebellar vermis are also seen.
The second parasagittal image (Fig. 2.2i) is obtained at the
caudothalamic junction, a shallow groove where the caudate
and thalamus meet at the floor of the lateral ventricle. The
caudothalamic junction is clinically important as a site where
subependymal, germinal matrix hemorrhage may occur that
53
CC
K
CS
CS
3
I
H
temporal horn; CB, cerebellum; CC, corpus callosum; CS, cavum
septum pellucidum; 3, third ventricle; 4, fourth ventricle; choroid
plexuses are in yellow
can subsequently lead to intraventricular hemorrhage in pre-
term infants; and it demarcates the anterior-most extent of the
choroid plexus. Echogenic foci anterior to the caudothalamic
junction therefore represent hemorrhage and not an extension
of the choroid plexus (Fig. 2.3). The caudothalamic junction
appears as a thin echogenic line by ultrasound. The caudate
nucleus is slightly more echogenic compared to the thalamus.
The third parasagittal image (Fig. 2.2j) demonstrates the lat-
eral extent of the lateral ventricles, where the choroid plexus is
located. The last parasagittal image (Fig. 2.2k) includes the
periphery of the frontotemporoparietal cortex beyond the lateral
ventricles. The sylvian fissure is partially seen on this image.
The posterior fontanelle is useful for assessing the occipi-
tal horn and posterior periventricular white matter. Layering
blood products along the dependent portion of the occipital
horn can be visualized.
The transmastoid view provides significantly more detailed
images of the posterior fossa in comparison to images obtained
through the anterior or posterior fontanelles (Fig. 2.4). This
view is especially helpful in assessing for cerebellar hemor-
rhage in patients treated with extracorporeal membrane oxy-
genation (ECMO).
54
Fig. 2.2 Normal ultrasound anatomy of the brain. Images correspond
to the anatomical planes shown in Fig. 2.1. (a) Line A. Coronal gray-
scale ultrasound image through the orbitofrontal lobes (F). Orbits (O)
are also seen. (b) Line B. Coronal grayscale ultrasound image through
the frontal horns (asterisks) of the lateral ventricles. Frontal horns are
bounded inferolaterally by the caudate nuclei (C) and superiorly by the
corpus callosum (CC). Parallel echogenic lines of the cavum septum
pellucidum (arrowheads) are seen dividing the right from left lateral
ventricles. Anterior temporal lobes (T) are seen bilaterally beneath the
Sylvian fissures (arrows). (c) Line C. Coronal grayscale ultrasound
image through the lateral ventricles (asterisks) at the level of the paired
foramina of Monro (arrowheads). Corpus callosum (CC) crosses the
H. H. R. Kim et al.
midline and is seen above the lateral ventricles. Echogenic choroid
plexus is seen in the roof of the third ventricle (3), extending through
the foramina of Monro, and along the floor of the lateral ventricles.
Pons (P) and medulla (M) are partially visualized in the far-field.
Temporal lobes (T) are seen beneath the Sylvian fissures (arrows). (d)
Line D. Coronal grayscale ultrasound image through the posterior
aspect of the third ventricle and body of the lateral ventricles (asterisks).
The third ventricle (3) is slit-like. Echogenic choroid plexus (arrow-
head) is noted in the roof of the third ventricle (3) and the floor of the
lateral ventricles. Paired thalami (TH) and cerebral peduncles (CP) are
seen (Fig. 2.2 continues)
2 Brain
Fig. 2.2 (continued) (e) Line E. Coronal grayscale ultrasound image
through the quadrigeminal cistern. The bodies of the lateral ventricles
(asterisks) are seen along with the echogenic choroid plexuses. The echo-
genic quadrigeminal cistern (Q) is bordered superiorly by the paired thal-
ami (TH) and inferiorly by the cerebellum (CB). (f) Line F. Coronal
grayscale ultrasound image through the atria of the lateral ventricles
(asterisks) with prominent paired choroid plexuses (arrowheads).
Cerebellum (CB) is partially visualized in the far-field. (g) Line G. Coronal
grayscale ultrasound image through the parietal-occipital lobes posterior
55
to the lateral ventricles. (h) Line H. Sagittal midline grayscale ultrasound
image. The entire length of the corpus callosum is seen (arrowheads) with
the cingulate gyrus (CG) located above the corpus callosum. Cavum sep-
tum pellucidum (asterisk) and anterior third ventricle (3) are seen. The
massa intermedia (MI) is located posterior to the third ventricle. The
anechoic fourth ventricle (4) is outlined by echogenic cerebellar vermis
(V). Fluid-­filled space of cisterna magna (CM) is seen in the far-field (Fig.
2.2 continues)
56
i j
C
TH
Fig. 2.2 (continued) (i) Line I. Sagittal grayscale ultrasound image
obtained at the caudothalamic junction (arrow). The caudothalamic
junction is a shallow groove where the caudate (C) and thalamus (TH)
meet at the floor of the lateral ventricle. Echogenic choroid plexus
(asterisk) is seen along the body of the lateral ventricle. (j) Line
J. Sagittal grayscale ultrasound image obtained at the lateral aspect of
H. H. R. Kim et al.
C
TH
the lateral ventricles, with choroid plexus (asterisk) still visible.
Caudate (C) and thalamus (TH) are seen. There is increased echo-
genicity of the posterior periventricular white matter (arrowhead), the
peritrigonal blush, a normal finding. (k) Line K. Sagittal grayscale
ultrasound image obtained lateral to the lateral ventricle. Sylvian fis-
sure (arrow) is partially visualized
2 Brain
Fig. 2.3 Blood products in both frontal horns. Coronal grayscale ultra-
sound image anterior to the third ventricle displays heterogeneous
echogenic foci (arrows) in the right and left frontal horns, correspond-
ing to evolving intraventricular blood products. Of note, these foci of hem-
orrhage must not be mistaken for normal choroid plexus which does not
extend into the frontal horns
T
C The brain consists of the forebrain, midbrain, and hindbrain
V tegmentum, and colliculi. The hindbrain structures include
4
C normal anatomy of the brain and its appearance on ultra-
Fig. 2.4 Mastoid view. Transverse grayscale ultrasound image of the pos-
terior fossa shows the cerebellar hemispheres (C), posterior vermis (V),
fourth ventricle (4), temporal horn (T), and cisterna magna (asterisk)
57
Normal Development and Anatomy
Normal Development
In the 3rd week of gestation, embryos form a long linear band
called the primitive streak (Fig. 2.5). At the cranial end of the
primitive streak, the notochord forms which grows and
migrates in a craniocaudal fashion [7]. The notochord induces
the cranial end to develop into the central nervous system
(CNS). The remainder of the notochord influences the devel-
opment of the peripheral nervous system.
In the 4th week of gestation, primary CNS development
takes place, forming the prosencephalon (i.e., the forebrain)
and the rhombomeres (that eventually develop into the mid-
and hindbrain). The prosencephalon gives rise to the telen-
cephalon and diencephalon. The telencephalon develops into
the paired cerebral hemispheres, ventricles, and caudate
nuclei. The diencephalon develops into the third ventricle,
thalami, and hypothalamus.
An appreciation of the structural changes associated
with congenital anomalies of the brain is best approached
through an understanding of cerebral cortical formation.
There are five stages of central nervous system (CNS)
development (Table 2.1) [8]: (1) dorsal induction at 3 to
4 weeks of gestation; (2) ventral induction at 5–8 weeks of
gestation; (3) neuronal proliferation at 8–18 weeks of gesta-
tion; (4) migration at 12–22 weeks of gestation; and (5)
development after migration and organization. The last three
developmental stages have been classified and updated on
the basis of genetics, molecular biology, and imaging [9].
Errors in any one of the last three stages can lead to malfor-
mations of cortical development.
Normal Anatomy
(Fig. 2.6). The forebrain structures include the cerebral
hemispheres, thalamus, hypothalamus, and pituitary gland.
The midbrain structures include cranial nerve nuclei, tectum,
the brainstem (midbrain, pons, and medulla), the cranial
nerves, and the cerebellar hemispheres. In this section, the
sound imaging are discussed.
58 H. H. R. Kim et al.

3 weeks
9 months
Future
forebrain 6 months
3 months
7 weeks
4 weeks
Midbrain Hind
brain
Hind
brain
Future Spinal
spinal cord
cord

Optic Forebrain
vesicle

Fig. 2.5 Embryology of the central nervous system (CNS). In the 3rd
week of gestation, the embryo forms a long linear band known as the
primitive streak. The notochord forms at the cranial end of the primitive
streak, which grows and migrates in a craniocaudal fashion. The noto-
chord induces the cranial end to develop into the CNS. The remainder
of the notochord develops into the spinal cord.
In the 4th week of gestation, primary CNS development takes place,
forming the prosencephalon (forebrain) and rhombomeres (mid- and
hindbrain). The prosencephalon further develops into the telencephalon
and diencephalon. The telencephalon develops into the paired cerebral
hemispheres, ventricles, and caudate nuclei. The diencephalon devel-
ops into the third ventricle, thalami, and hypothalamus.
In the second and third trimesters, the brain structures develop fur-
ther, including microscopic pathways such as neuronal migration, and
macroscopic pathways including gyral and fissure formation. Brain vol-
ume increases steadily until early childhood

Table 2.1 Simplified five stages of central nervous system (CNS) development
Dorsal induction Ventral induction disorders Neuronal proliferation Neuronal migration Post-migration
disorders disorders disorders disorders
Chiari malformation Holoprosencephaly Hemimegalocephaly Lissencephaly Polymicrogyria
Anencephaly Septo-optic (pituitary) dysplasia Focal cortical dysplasia Gray matter heterotopia Schizencephaly
Hydranencephaly Callosal dysgenesis/agenesis
Intracranial lipoma
Dandy–Walker syndrome

Sagittal Midline Brain Cerebral Cortex

Sulci are echogenic curvilinear lines separating the gyri, which

Thalamus
Hypothalamus
Fig. 2.6 Normal sagittal anatomy of the brain in the midline. The brain
consists of the forebrain, midbrain, and hindbrain. Forebrain structures
include the cerebrum, corpus callosum, thalamus, hypothalamus, and
pituitary gland. Midbrain structures include cranial nerve nuclei, tec-
tum, tegmentum, and colliculi. Hindbrain structures include the brain-
stem (midbrain, pons, and medulla), cranial nerves, and the cerebellum.
The ventricles are fluid-filled spaces within the brain which act as con-
duits for cerebrospinal fluid
appear relatively hypoechoic. The sulci appear echogenic due to
the interface of collagen and blood vessels in the pia mater
against the underlying brain parenchyma that consists largely of
water. The subcortical white matter is more echogenic centrally
and becomes less so as it extends peripherally (Fig. 2.7) [10].
The subcortical white matter myelinates in the postna-
tal period [11]. Therefore, in newborns, the subcortical
white matter is more hyperechogenic compared to the cor-
tical ribbon, deep gray nuclei, thalamus (especially ven-
trolateral thalamus), and posterior limb of internal capsule
due to lack to myelination [12]. In normal full-term
infants, the posterior limb of the internal capsule is
myelinated at birth.
Basal Ganglia and Thalami
The basal ganglia are a group of subcortical nuclei that
include the caudate, putamen, and globus pallidus in the
2 Brain 59

T T

Fig. 2.7 Normal gray–white matter differentiation. A sagittal gray-

scale ultrasound image of the lateral aspect of the brain shows the
hypoechoic gyri (arrow), subcortical white matter (asterisk), and echo-
genic sulci (arrowhead)

Fig. 2.8 Echogenic thalami in a 25-week-old premature female infant.

cerebral hemispheres, the substantia nigra in the midbrain, Coronal grayscale ultrasound image of the basal ganglia and thalami
and the subthalamic nucleus in the diencephalon. On ultra- (T) demonstrates homogenous hyperechogenicity relative to the adja-
sound, the caudate, putamen, globus pallidus, and thala- cent white matter. The surface of the brain appears smooth. Both find-
mus can be distinctly visualized. In healthy full-term ings are expected in preterm infants born at less than 32 weeks of
gestation
neonates, the basal ganglia and thalamus are isoechoic to
the cortical gray matter and hypoechoic to the subcortical
white matter (Fig. 2.2i). In preterm infants born prior to
32 weeks of gestation, the basal ganglia and thalamus are
homogenously hyperechoic relative to the adjacent white
matter (Fig. 2.8) [13].

Ventricles

The ventricles are anechoic fluid-filled cavities, which act

as conduits for the cerebrospinal fluid (CSF). The lateral
ventricles are composed of a body, an atrium, and anterior,
temporal, and occipital horns. The lateral ventricle on each
side abuts the caudate nucleus, thalamus, and cerebral
peduncle. The foramen of Monro is a short connecting pas-
sage between each lateral ventricle and the third ventricle
(Fig. 2.9).
The normal width of the body of the lateral ventricle is
less than 10 mm. However, patient positioning can affect Fig. 2.9 Foramen of Monro. Coronal grayscale ultrasound image dem-
the size of the ventricle. Slit-like ventricles are observed in onstrates a short connecting passage (arrowhead) located between the
60% of normal full-term newborns and in 30% of normal lateral (asterisks) and third (3) ventricles. Unless it is dilated, the fora-
men of Monro is often not well seen
preterm infants (Fig. 2.10) [14]. Asymmetry of lateral ven-
tricle size is a common finding; the left lateral ventricle is
often slightly larger than the right (Fig. 2.11) [15, 16]. adjacent to the superolateral walls of the frontal horns or
Connatal cysts, also known as coarctation of the lateral body of the lateral ventricles (Fig. 2.12a). They are con-
ventricles or frontal horn cysts, are small cystic areas sidered a normal anatomical variant. On ultrasound, mul-
60
tiple cysts can be seen, forming a string of pearls
appearance (Fig. 2.12b). Connatal cysts are rare and are
seen in up to 1.05% of premature or low birth weight
infants [17]. They eventually resolve spontaneously with-
out adverse neurological effects.
Fig. 2.10 Slit-like ventricles in a normal full-term infant. Coronal gray-
scale ultrasound image shows small frontal horns (arrowheads) of the lat-
eral ventricles, a normal finding in the absence of intracranial pathology
a b
Fig. 2.12 Connatal cysts in a 1-day-old male. (a) Coronal grayscale
ultrasound image at the level of the third ventricle (3) shows bilateral
connatal cysts (arrowheads) adjacent to the superolateral walls of the
frontal horns of the lateral ventricles (asterisks). (b) Parasagittal gray-
H. H. R. Kim et al.
Cisterna Magna
The cisterna magna is an extra-axial fluid space located infe-
rior to the echogenic cerebellar vermis and superior to the
occipital bone (Fig. 2.2h). The normal cisterna magna mea-
sures less than 8–10 mm in the axial and sagittal planes [18].
Mega cisterna magna is a normal variant where the extra-axial
fluid space measures more than 8–10 mm in the axial and
Fig. 2.11 Asymmetrical ventricular size. Coronal grayscale ultrasound
image shows that the frontal horn of the left lateral ventricle is slightly
larger than the right, a normal and relatively common finding
scale ultrasound image of the brain shows two connatal cysts (arrow-
heads) along the body of the lateral ventricle, forming a string of pearls
appearance
2 Brain
a b
C V
Fig. 2.13 Mega cisterna magna in a 1-month-old male, a normal vari-
ant. (a) Sagittal midline grayscale ultrasound image shows an enlarged
CSF subarachnoid space (asterisk) inferior and posterior to the cerebel-
mid-sagittal planes (Fig. 2.13). Mega cisterna magna can be
differentiated from an abnormality such as an arachnoid cyst,
by its lack of associated mass effect, and from a Dandy–
Walker syndrome by the presence of the cerebellar vermis.
Extra-Axial Fluid Spaces
Extra-axial fluid spaces are CSF spaces located between the
brain and the skull. The extra-axial fluid spaces are bounded
by the leptomeninges and traversed by blood vessels, which
are highly echogenic. The extra-axial fluid spaces are best
seen in the midline using a high-frequency linear array trans-
ducer on the order of 12 MHz. The lateral, anterior, and pos-
terior aspects of the extra-axial fluid spaces are difficult to
visualize with ultrasound. With color Doppler, cortical veins
can normally be seen extending from the brain surface to the
skull within the extra-axial space.
Congenital Brain Anomalies
Dorsal Induction Disorders
Chiari Malformations
Chiari malformations are characterized by caudal descent of
the cerebellar tonsils inferior to the foramen magnum. Chiari
I malformation is the most common and demonstrates descent
61
C
lum (C). (b) Axial FLAIR MR image depicts the mega cisterna magna
(asterisk) posterior to the cerebellum (C) and vermis (V). There is no
associated mass effect on the brain
of the cerebellar tonsils more than 5 mm below the foramen
magnum without displacement of the medulla or fourth ven-
tricle [19]. A common association is a small osseous poste-
rior fossa, which results in crowding and inferior herniation
of the cerebellar tonsils (Fig. 2.14). Other associations
include multisutural craniosynostosis, as with Crouzon syn-
drome and Pfeiffer syndrome. Symptoms depend on the
degree of inferior tonsillar herniation.
Ultrasound imaging of Chiari I malformation is challeng-
ing since the herniated cerebellar tonsils cannot be directly
visualized with conventional transfontanellar ultrasound
imaging. Indirect signs of Chiari I malformation include dif-
ficult visualization of an elongated fourth ventricle, an unusu-
ally low position of the cerebellar vermis, an enlarged massa
intermedia, and obliteration of the cisterna magna.
Hydrocephalus is seen in up to 30% of cases [20], with
dilated lateral and third ventricles that can be an indirect clue to
Chiari I malformation. When there is a suspicion for Chiari 1
malformation, MR imaging of the brain and spine is needed for
further assessment. MR imaging allows accurate assessment of
the degree of inferior tonsillar descent, patency of CSF flow, and
evaluation for associated hydrocephalus and/or syrinx [21].
Treatment depends on the symptoms, degree of ventriculomeg-
aly, and presence of a syrinx.
Chiari II malformation is a complex malformation of the
hindbrain, spine, and mesoderm of the skull base and spinal
column. It is characterized by a small posterior fossa associated
with caudal herniation of the lower part of the cerebellum and
62
Normal brain
Fig. 2.14 Chiari malformations. This group of malformations is characterized by caudal descent of the cerebellar tonsils below the foramen mag-
num, a small bony posterior fossa and inferior herniation of the cerebellar tonsils
a b
C
Fig. 2.15 Chiari II malformation in a 5-day-old female following
myelomeningocele repair. (a) Sagittal grayscale ultrasound image of
the brain shows signs of Chiari II malformation, including a small pos-
terior fossa with a low-lying cerebellum (C) and hydrocephalus (aster-
isk). A low position of the tentorium (arrows) and torcula (arrowhead)
are also identified. (b) Coronal T2-weighted MR image of the brain
fourth ventricle (Fig. 2.15). It is present in all children with open
spinal dysraphism. Other associated findings include hydro-
cephalus, callosal dysgenesis, gray matter heterotopia, and col-
pocephaly [22]. The fourth ventricle is usually small and
narrowed in the anteroposterior dimension, vertically oriented
and inferiorly displaced. The brainstem is abnormal with infe-
rior displacement of the pons and medulla. The pons is hypo-
plastic and the medulla occasionally extends below the foramen
magnum.
Since most patients with Chiari II malformation present with
myelomeningocele, this neural tube defect is often detected by
H. H. R. Kim et al.
Chiari malformation
c
C
confirms a low-­lying cerebellum (C) and a small posterior fossa. The
lateral ventricles (asterisks) are dilated. (c) Sagittal T2-weighted MR
image of the spine shows inferior descent of the cerebellar tonsils
(asterisk) with obliteration of the cisterna magna (arrow). There is a
low-lying spinal cord (arrowhead) at the level of S2-S3. The exposed
neural placode is not included on the image
prenatal imaging. After birth, MR imaging is the modality of
choice for precise evaluation of brain and spine morphology
[22]. Ultrasound imaging is useful for monitoring the brain for
hydrocephalus both prior to and after surgery, as well as to
assess for post-surgical complications, including intracerebral
and extra-axial hemorrhage and other fluid collections.
The availability of fetal ultrasound and MR imaging has
facilitated the prenatal diagnosis of Chiari II malformation and
has been the major impetus for the development of fetal sur-
gery. However, this new treatment option has generated a
number of controversies, particularly with respect to maternal
2 Brain
morbidity and premature birth of the fetus. Postnatal repair of
myelomeningocele remains the standard of care in most insti-
tutions in the United States and around the world.
Anencephaly
Anencephaly is the absence of the forebrain, skull, and scalp
with partial preservation of the posterior fossa. Anencephaly
is the most severe form of neural tube defect in which the
cephalic end of the neural tube fails to close. It is often diag-
nosed during antenatal ultrasound screening. Maternal serum
alpha-fetoprotein is markedly elevated during pregnancy. On
ultrasound imaging, no skull bones and parenchymal tissues
are seen above the level of the orbits (Fig. 2.16). Portions of
the occipital bones may be present, along with the posterior
fossa. Anencephaly is incompatible with life. Therefore, if it
is detected prenatally, termination of pregnancy is typically
considered.
Hydranencephaly
Hydranencephaly denotes destruction of the cerebral hemi-
spheres. The skull is intact, in contrast to anencephaly. The
cerebral hemispheres are liquefied and replaced by CSF
within an intact membranous sac. The basal ganglia and
a b
Fig. 2.16 Fetus with anencephaly. (a) 3D reformatted ultrasound
image shows absence of the brain, skull, and scalp (arrow) consistent
with anencephaly. In addition, micrognathia (asterisk), low set ears
63
brainstem are intact. There are numerous causes of hydran-
encephaly, including bilateral supraclinoid internal carotid
arterial occlusion, extensive leukomalacia, diffuse hypoxic–
ischemic injury leading to necrosis, and infection.
Hydranencephaly may be diagnosed prenatally, although in
the absence of a prenatal diagnosis, affected pediatric
patients may present later in infancy with seizures, flaccidity,
respiratory failure, or vegetative state.
On ultrasound, the falx is usually present. The cortical tis-
sues are largely absent and replaced by CSF (Fig. 2.17).
Some residual tissue can be seen in the occipital poles and
medial temporal lobes. The basal ganglia, thalami, and pos-
terior fossa are preserved. Hydranencephaly is generally not
compatible with life after birth. Therefore, if detected prena-
tally, termination of pregnancy is typically considered.
Ventral Induction Disorders
Holoprosencephaly
Holoprosencephaly is a congenital brain malformation
caused by incomplete cleavage of the forebrain and the face.
There are varying degrees of fusion of the paired cerebral
(arrowhead), and edema of the remaining head are seen. (b) Sagittal
T2-weighted MR image shows absent brain and skull (arrow) and intact
globe (arrowhead). B, Bladder
64
a b
CSF
MB
Fig. 2.17 Hydranencephaly in a 2-day-old male. (a) Coronal grayscale
ultrasound image shows near-complete destruction of the cerebral hemi-
spheres that are replaced by cerebrospinal fluid (CSF). Residual brain paren-
chyma (asterisks) is seen at the periphery of the skull, along with an intact
MV
T ami are fused. There are rudimentary-appearing temporal
Fig. 2.18 Holoprosencephaly (alobar type) in a 1-day-old male. Coronal
grayscale ultrasound image shows a monoventricle (MV), fused bilateral
thalami (T), and fusion (asterisk) of the frontal lobes
hemispheres, lateral ventricles, olfactory tracts, and thalami.
Facial anomalies can include cyclopia, cleft lip/palate,
hypertelorism, and a solitary median maxillary central inci-
sor. Depending on the severity of the fusion anomalies, there
are three subtypes of holoprosencephaly that range from
mild to severe: lobar, semilobar, and alobar types.
Holoprosencephaly is often detected prenatally, and life
expectancy depends on the subtype.
H. H. R. Kim et al.
MB
midbrain (MB). (b) Coronal T2-weighted, fat-suppressed MR image dem-
onstrates near-complete absence of the cerebral hemispheres with minimal
residual brain parenchyma (asterisks). The falx and midbrain (MB) are pres-
ent. In contrast to anencephaly, the cranial vault (arrow) is intact
On ultrasound imaging of alobar holoprosencephaly, the lat-
eral ventricles are fused and form a monoventricle (Fig. 2.18).
There are undivided cerebral hemispheres and fused thalami.
There is lack of division between the frontal, parietal, temporal,
and occipital lobes and the ventricular horns. The posterior
fossa and brainstem remain relatively normal.
Semilobar holoprosencephaly demonstrates partial cleav-
age of the cerebral hemispheres posteriorly. The frontal lobes
remain fused and malformed with a monoventricle. The thal-
and occipital horns.
Lobar holoprosencephaly is the mildest form of holopros-
encephaly where the cerebral hemisphere is divided posteri-
orly. However, there is fusion of the anterior horns of the
lateral ventricles forming a squared and flattened roof beneath
the corpus callosum. The septum pellucidum is absent. The
remaining portions of the lateral ventricles are divided nor-
mally. The thalami appear normal and are not fused.
 epto-Optic (Pituitary) Dysplasia
S
Septo-optic dysplasia, or the currently preferred term “septo-­
optic pituitary dysplasia”, is a midline congenital brain mal-
formation with abnormalities that include a partially or
completely absent septum pellucidum, optic nerve hypopla-
sia, and hypopituitarism. All three abnormalities occur in
approximately 30% of affected individuals. Absence of the
septum pellucidum occurs in approximately 60% of patients
[23]. There is also an association with schizencephaly and
gray matter heterotopia. Clinical presentation can include
seizures, visual abnormalities, developmental delay, and pre-
cocious puberty.
2 Brain
On ultrasound imaging, absence of the septum pellu-
cidum is readily identified (Fig. 2.19a). To assess for optic
nerve hypoplasia and pituitary abnormality, further evalua-
tion with MR imaging is needed (Fig. 2.19b, c).
Treatment requires a multidisciplinary approach to address
the associated neurodevelopmental, ophthalmological, and
endocrinological abnormalities.
 nomalies of the Corpus Callosum
A dysplasia, and Aicardi syndrome [25].
The corpus callosum is a thick white matter tract that con-
nects the cerebral hemispheres and enables communication
between them. Between 8 and 20 weeks of gestation, the cor-
pus callosum is formed in an anterior-to-posterior fashion,
a b
Fig. 2.19 Septo-optic (pituitary) dysplasia in a 4-year-old male. (a)
Coronal grayscale ultrasound image at birth reveals absence of the sep-
tum pellucidum (asterisk). (b) Coronal T2-weighted MR image shows
hypoplasia (arrow) of the optic chiasm and absence (asterisk) of the
65
with the exception of the rostrum, which forms last [24]. The
corpus callosum can be completely absent (agenesis) or only
partially formed (callosal dysgenesis) [25].
There are more than 130 syndromes with associated anoma-
lies of the corpus callosum. The more commonly encountered
associations include cortical malformations (polymicrogyria,
pachygyria, and heterotopia), Chiari II malformation, Dandy–
Walker syndrome, holoprosencephaly, septo-optic pituitary
Children with isolated corpus callosum anomalies often
appear normal or near-normal by 3 years of age, but as the
complexity of school tasks increases, subtle deficient cogni-
tive functions often become noticeable.
septum pellucidum. There was right greater than left optic nerve hypo-
plasia as well (not shown). (c) Sagittal T1-weighted MR image shows
an ectopic pituitary gland with a bright spot (arrow) at the proximal end
of the infundibulum
66 H. H. R. Kim et al.

a b

c d

Fig. 2.20 Agenesis of the corpus callosum in an 8-day-old male. (a)
Coronal grayscale ultrasound image at the level of the third ventricle (3)
demonstrates absence of the corpus callosum with widely separated
anterior horns of the lateral ventricles (arrowheads). (b) Coronal gray-
scale ultrasound image obtained via the posterior fontanelle demon-
strates a parallel configuration of the lateral ventricles (arrowheads).
Asterisks, Choroid plexuses. Sagittal grayscale ultrasound image (c)
and T1-weighted MR image (d) show a high-riding third ventricle (3)
with radially oriented gyri and sulci (arrowheads) converging on the third
ventricle (3) and the expected location of the corpus callosum (asterisks)

Ultrasound imaging of callosal agenesis reveals separa- partly or completely absent, best identified on sagittal
tion of the lateral ventricles which run parallel to each other images. The third ventricle is high-riding and there is associ-
(sometimes referred to as the “Texas longhorn” sign or the ated colocephaly, or abnormal dilation of the occipital horns
“moose head” sign) (Fig. 2.20). The corpus callosum is of the lateral ventricles. There is a radiating sulcal pattern of
2 Brain 67

the gyri that converge on the third ventricle. In some cases,
bundles of Probst are identified. These are re-routed callosal
fibers seen in the medial aspect of the lateral ventricles. The
anterior cerebral artery has an abnormal posterior course.
Intracranial lipoma occurs in about half of patients with
callosal dysgenesis as a result of abnormal differentiation
of the embryologic primordium of the meninges. It can be
found anywhere in the brain, although the pericallosal
region is the most common, followed by the quadrigeminal
cistern, suprasellar cistern, cerebellopontine angle, Sylvian
fissure, and rarely, in the choroid plexus [26]. On ultra-
sound, an intracranial lipoma is seen as a well-defined,
hyperechoic mass that may demonstrate posterior acoustic
shadowing due to punctate foci of calcification (Fig. 2.21).

Dandy–Walker Syndrome
Dandy–Walker syndrome is a spectrum of cystic posterior
fossa malformations characterized by a hypoplastic vermis, a
dilated fourth ventricle which communicates with a promi-
nent CSF space of the posterior fossa, and elevation of the
torcula [27].
In the postnatal period, hydrocephalus develops in 70–90%
of patients, with macrocephaly being the most common pre-
sentation. In approximately 70% of cases, other intracranial
abnormalities are seen, including holoprosencephaly, schizen-
cephaly, callosal dysgenesis, cortical dysplasia, polymicrogy-
ria, gray matter heterotopia, lipoma, and cephalocele [28].
Trisomies 13, 18, and 21, and other syndromes including
Aicardi syndrome, Cornelia de Lange syndrome, and
Walker–Warburg syndrome, can be associated with Dandy–
Walker syndrome [29]. Motor-related symptoms are com-
mon, including spasticity, hypotonia, and delayed motor
function, as well as intellectual disability.
The ultrasound features of Dandy–Walker syndrome include
a hypoplastic or aplastic vermis, a dilated fourth ventricle com-
municating with the posterior fossa CSF space, a markedly
Fig. 2.21 Lipoma of the corpus callosum in a 2-month-old male. prominent cisterna magna (greater than 10 mm diameter), and a
Sagittal midline grayscale ultrasound image demonstrates a well-­
defined hyperechoic mass (arrowheads) in the pericallosal region
CSF gap between the cerebellar hemispheres (Fig. 2.22).

a b

C
4
4
P
C P

Fig. 2.22 Dandy–Walker malformation in a 1-month-old female. (a) lus with dilated lateral (asterisk) and third (3) ventricles. C, Cerebellum.
Sagittal grayscale ultrasound image near the midline demonstrates (b) Axial FLAIR MR image demonstrates a “keyhole” appearance of the
absence of the vermis and a dilated fourth ventricle (4) communicating absent vermis and dilated fourth ventricle (4) communicating (arrow)
(arrow) with the posterior fossa (P) CSF. There is associated hydrocepha- with the posterior fossa (P) CSF
68
Hypoplastic vermis with rotation (previously referred to
as “Dandy–Walker variant”) denotes a posterior fossa mal-
formation where there is hypoplasia of the vermis and a
dilated fourth ventricle that are less pronounced when com-
pared to the classic Dandy–Walker syndrome. There is no
enlargement of the posterior fossa.
Neuronal Proliferation Disorders
Hemimegalencephaly
Hemimegalencephaly is a rare cortical disorder character-
ized by hamartomatous overgrowth of the ipsilateral cerebral
hemisphere. It is caused by abnormal neuronal proliferation
and/or decreased neuronal apoptosis. Pediatric patients with
hemimegalencephaly present with macrocephaly, develop-
mental delay, and seizures. In addition to hamartomatous
overgrowth of the ipsilateral cerebral hemisphere, other
organizational and post-­ migration disorders can be seen,
including lissencephaly, pachygyria, and polymicrogyria.
On ultrasound imaging, there is an enlarged and dysplas-
tic appearance of the ipsilateral cerebral hemisphere. The
affected cortex is thickened with irregular gyri, and pachygy-
ria or polymicrogyria are often seen. Dystrophic calcifica-
tions occur at the gray–white matter junctions. There is
enlargement of the ipsilateral lateral ventricle without hydro-
cephalus and midline shift.
Treatment is aimed at symptomatic control, particularly
seizures. For refractory seizures, hemispherectomy can be
considered if the contralateral cerebral hemisphere is not
affected by the hemimegalencephaly.
Neuronal Migration Disorders
Lissencephaly
Lissencephaly, also known as agyria, is a rare disorder of
cortical malformation resulting in a smooth brain surface.
There is an arrest in the transmantle migration of neurons,
which results in either a severe form of absent gyri (agyria
or complete lissencephaly) or the presence of a few gyri
(pachygyria or incomplete lissencephaly). Agyria and
pachygyria often coexist in different parts of the brain of an
affected patient. In the setting of severe lissencephaly, cal-
losal dysgenesis with a vertically oriented splenium can be
seen. Lissencephaly can be sporadic or associated with dis-
orders such as Miller–Dieker syndrome or Norman–Roberts
syndrome.
On ultrasound imaging, a smooth brain surface is seen
with a thickened cortex and a lack of, or paucity of sulca-
tion. In addition, there is ventriculomegaly, especially col-
pocephaly, without hydrocephalus; the Sylvian fissures are
widened; and the subarachnoid space is prominent. In
H. H. R. Kim et al.
pachygyria, abnormally formed gyri are typically broad
with shallow sulci. Lissencephaly can be reliably diag-
nosed during or after the third trimester of pregnancy.
Follow-up imaging in late pregnancy is therefore recom-
mended if there is an early diagnosis of or suspicion for
lissencephaly [30].
 ray Matter Heterotopia
G
Gray matter heterotopia is a frequently encountered neuronal
migration disorder. Cortical neurons are arrested between 6
and 16 weeks of gestation along the radial pathway from the
germinal matrix of the ventricle to the cortical surface [9].
Gray matter heterotopia is divided into three groups: subep-
endymal, subcortical, and band heterotopia. Unless subepen-
dymal gray matter heterotopia bulges into the ventricles
(Fig. 2.23), other forms of gray matter heterotopia are diffi-
cult to diagnose by ultrasound.
Post-Migration Disorders
Polymicrogyria
Polymicrogyria is a congenital malformation characterized
by excessive folding of the cerebral cortex. Neurons from
the germinal matrix reach the cortex but are abnormally
organized, forming multiple small and undulating gyri.
Polymicrogyria is a feature of genetic disorders such as
Aicardi and Joubert syndromes, metabolic processes such
as Zellweger syndrome and mitochondrial diseases, in utero
injury and infection [31]. If there are co-existing auditory or
visual symptoms, infection such as cytomegalovirus (CMV)
should be suspected.
There is a predilection of polymicrogyria for the perisyl-
vian region, and syndromic cases may be bilateral. Symptoms
include seizures and developmental delay. The severity of
symptoms correlates with the extent of polymicrogyria and
associated anomalies.
On ultrasound imaging, the excessively small and undu-
lating gyri are difficult to detect. High-resolution MR imag-
ing is typically needed for diagnosis.
Schizencephaly
Schizencephaly is a congenital post-migrational and organi-
zational disorder characterized by clefts in the cerebral hemi-
sphere extending from the ventricle to the cortical surface
[32]. The cleft is lined with heterotopic gray matter, often
forming polymicrogyria. There are two types which are
defined by whether or not there is apposition of the gray mat-
ter lining the cleft: closed-lip (gray matter apposition) or
open-lip (gray matter separation).
Schizencephaly is bilateral in up to 50% of cases. Pediatric
patients with closed-lip schizencephaly can be asymptomatic but
often present with seizures and developmental delay. Patients
2 Brain 69

a b

V V

Fig. 2.23 Gray matter heterotopia (subependymal type) in a 13-day-­ demonstrate subependymal gray matter heterotopia (arrowheads) adja-
old female. (a) Sagittal grayscale ultrasound image shows multiple cent to the bodies of the lateral ventricles (V) with the same signal char-
nodules (arrows) projecting into the body of the left lateral ventricle acteristics as gray matter
(V). (b) Sagittal T1-weighted and (c) axial T2-weighted MR images

with open-lip schizencephaly have increased morbidity and pres- cortex (Fig. 2.24). There may be a nipple-like outpouching
ent with macrocephaly, seizures, or developmental delay. of the ventricle at the origin of the cleft. Closed-lip schizen-
On ultrasound imaging, open-lip schizencephaly demon- cephaly is difficult to visualize by ultrasound. MR imaging is
strates a fluid-filled cleft extending from the ventricle to the therefore needed for definitive diagnosis.
70 H. H. R. Kim et al.

a b

CSF

Fig. 2.24 Schizencephaly in a newborn male. (a) Coronal grayscale septum pellucidum is absent. (b) Coronal T2-weighted MR image
ultrasound image demonstrates a large open-lip schizencephaly (arrow) shows the open-lip schizencephaly (arrow) lined with heterotopic gray
of the right cerebral hemisphere, with the lateral ventricle (V) in direct matter (arrowheads)
communication with an enlarged extra-axial CSF space (CSF). The

Table 2.2 Papile grading system for preterm infants with periven-
Intracranial Hemorrhage tricular/intraventricular hemorrhage
Grade Description
In premature infants, particularly those of low birth weight,
Grade 1 Blood products confined to
germinal matrix and intraventricular hemorrhage are com- subependymal region of germinal
monly seen. In term infants, extra-axial hemorrhage, includ- matrix in the caudothalamic groove
ing epidural, subdural, and subarachnoid bleeds, are typically Grade 2 Blood products extend beyond the
related to birth trauma. Intra-parenchymal hemorrhage can germinal matrix into the ventricles
without dilation
occur in both term and pre-term infants as a result of numer- Grade 3 Blood products extend beyond the
ous underlying causes. caudothalamic groove into the enlarged
ventricles; or hemorrhage occupies more
than 50% of the ventricular volume
Grade 4 Parenchymal hemorrhage (due to venous
Preterm Infants
infarction) along with germinal matrix
hemorrhage
In preterm infants, neonatal hemorrhage occurs in the germi-
nal matrix because the subependymal lining is highly vascular.
Neonatal periventricular/intraventricular hemorrhage (PIVH)
occurs in up to 25% of preterm infants who are less than (Fig. 2.25). Grade 2 PIVH extends outside the caudothalamic
33 weeks of gestation or infants weighting less than 1500 groove, and into a non-­dilated ventricle (Fig. 2.26). Grade 3
grams [33]. The Papile classification [34] is the most com- PIVH is hemorrhage within the ventricle with associated
monly used system to grade PIVH (Table 2.2). Grade 1 PIVH ventricular dilation, or hemorrhage occupying more than
is confined to the caudothalamic groove, without extension 50% of the ventricular volume (Fig. 2.27). It is important to
into the ventricular system. note that progression of a Grade 2 to a Grade 3 PIVH requires
On ultrasound, echogenic foci consistent with hemor- follow-up assessment for post-hemorrhagic hydrocephalus
rhage are seen at or anterior to the caudothalamic groove where intervention might be considered.
2 Brain 71

Fig. 2.25 Periventricular/intraventricular hemorrhage (PIVH) grade 1. Sagittal (left panel) and coronal (right panel) grayscale ultrasound
(a) Normal caudothalamic groove. Sagittal grayscale ultrasound image images show an ovoid, echogenic focus of hemorrhage (arrowheads) at
demonstrates the shallow groove (arrow) where the caudate (C) and the caudothalamic groove
thalamus (T) meet at the floor of the lateral ventricle. (b) PIVH grade 1.

Fig. 2.27 Periventricular/intraventricular hemorrhage (PIVH) grade 3.

Coronal grayscale ultrasound image shows blood (arrowheads) within
the dilated lateral ventricles. Dangling choroid plexuses (asterisks) are
also seen
Fig. 2.26 Periventricular/intraventricular hemorrhage (PIVH) grade 2.
Sagittal grayscale ultrasound image shows a loss of the groove at the
caudothalamic junction (black arrow) and hypoechoic material consistent
with blood products. Echogenic material in keeping with blood extends
adjacent and inferior (white arrow) to the choroid plexus without ventricular
dilation
72
Serial cranial ultrasound examinations are an excellent
method to assess the evolution of hemorrhage and to monitor
for the development of hydrocephalus. In addition, posterior
fontanelle images are useful to assess for layering of blood in
the occipital horns that is difficult to visualize from an ante-
rior fontanelle approach.
 eriventricular Hemorrhagic Infarction
P preterm infants, particularly following Grade 3 or PVHI/
Periventricular hemorrhagic infarction (PVHI) is also known
as Grade 4 PIVH in the original Papile classification. The
mechanism of Grade 4 PIVH is different from the other
grades of PIVH. The periventricular white matter is drained
by the deep medullary veins, which are fan-shaped vessels
draining into the terminal veins along the ventricular mar-
gins (Fig. 2.28). PVHI is caused by bleeding in the germinal
matrix, which causes stasis and engorgement of the terminal
veins and subsequently the medullary veins. Eventually,
venous thrombosis and parenchymal venous hemorrhagic
infarction occur (Fig. 2.29).
Medullary
veins
LV
Foramen
of Monro
Terminal
vein
Germinal
matrix
Fig. 2.28 Diagram of the deep medullary veins. These vessels fan out to
drain the white matter of the cerebral hemispheres into the terminal veins of
the germinal matrix along the lateral ventricular margins. Germinal matrix
hemorrhage interferes with this drainage leading to engorgement and
thrombosis. LV, Lateral ventricle
H. H. R. Kim et al.
Approximately 10–15% of preterm neonates with PIVH
will develop PVHI [35]. The chronic neuroimaging manifes-
tation of PVHI is a porencephalic cyst replacing the injured
periventricular white matter and cortex (Fig 2.29e). PVHI
can also occur in isolation as well as in utero, without associ-
ated germinal matrix hemorrhage.
Post-hemorrhagic hydrocephalus is seen in 20–25% of
Grade 4 PIVH. Preterm infants are at higher risk for multi-
compartmental obstructive hydrocephalus because they
have a relative deficiency of fibrinolytic function and they
are therefore prone to fibroproliferative subependymal
gliosis. Grade 3 and PVHI/Grade 4 PIVH typically require
ventricular shunting while this rarely required for grades 1
and 2.
Cerebellar Hemorrhage
Cerebellar hemorrhage is more frequently seen in preterm
infants than in term infants, and occurs in up to 19% of preterm
infants of less than 32 weeks’ gestational age [36]. Cerebellar
hemorrhage is best imaged through the trans-mastoid fonta-
nelle. Hemorrhage is usually localized to the cerebellar hemi-
sphere and less frequently in the vermis. Etiologies include
primary parenchymal hemorrhage, venous infarction, and intra-
ventricular bleed extending to the cerebellar hemisphere. The
patient is often asymptomatic. However, when the intracerebel-
lar hemorrhage is large, it can cause mass effect on the adjacent
brainstem leading to apnea or respiratory symptoms. With the
increasing use of extracorporeal membrane oxygenation
(ECMO) in infants, cerebellar hemorrhage has been increasing
in incidence (Fig. 2.30).
Term Infants
In term infants, birth-related intracranial hemorrhage is rela-
tively common and generally benign. Extra-axial hemor-
rhage can be epidural, subdural, or subpial in location.
Intracerebral hemorrhage can be localized to the parenchy-
mal, subependymal, cerebellar, or choroid plexus regions.
Epidural Hemorrhage
Epidural hemorrhage is an accumulation of blood in the poten-
tial space between the dura mater and the overlying skull.
Epidural hemorrhage is relatively uncommon in newborns but
can be seen in the setting of traumatic or instrument-­assisted
birth injury. In infants, epidural hemorrhage is more often due
to venous tears than to arterial tears because there are abun-
dant dural and diploic veins in the rapidly growing skull [37].
2 Brain 73

b c

d e

Fig. 2.29 Periventricular hemorrhagic infarction (PVHI), or grade 4
periventricular/intraventricular hemorrhage (PIVH) in a 3-day-old male.
(a) Coronal grayscale ultrasound image shows left germinal matrix hem-
orrhage (asterisk) as well as blood filling most of the left lateral ventricle.
(b) Axial T1-weighted MR image shows blood products extending from
the germinal matrix into the left lateral ventricle (asterisk) and the adja-
cent periventricular white matter (arrow). (c) Axial susceptibility-weighted
MR image shows blooming artifact related to blood products in the
germinal matrix and ventricle (asterisk), as well as in the adjacent peri-
ventricular white matter (white arrow). There is engorgement of the peri-
ventricular terminal medullary veins (black arrow). (d) Follow-up
coronal grayscale ultrasound image obtained 2 weeks after the initial
insult demonstrates clot retraction (asterisk) in the lateral ventricle and
adjacent periventricular white matter (arrow). The clot is now heteroge-
neously hypoechoic, an expected change. (e) Follow-up coronal gray-
scale ultrasound image at 3 months demonstrates a porencephalic cyst
(arrow) replacing the injured periventricular white matter and communi-
cating with the left lateral ventricle
74
The epidural blood cannot cross suture lines and therefore
manifests as a lentiform collection adjacent to the skull. It is
difficult to differentiate epidural from subdural hemorrhages
Fig. 2.30 Cerebellar bleed in a 15-day-old premature male weighing
455 grams. Coronal grayscale ultrasound image demonstrates a hyper-
echoic focus (arrow) in the right cerebellar hemisphere consistent with
a bleed
a b
Fig. 2.31 Subdural fluid collections in a 2-month-old male with sus-
pected non-accidental trauma. (a) Coronal grayscale ultrasound image
demonstrates bilateral subdural fluid collections (asterisks) of different
echogenicity suggesting hemorrhage of different ages from repetitive
H. H. R. Kim et al.
by ultrasound and, therefore, additional cross-sectional imag-
ing with CT is needed for diagnosis.
Subdural Hemorrhage
Subdural hemorrhage is an accumulation of blood that
results from tearing of the bridging veins in the potential
space between the dura mater and the arachnoid mater as
they cross the subdural space, or of the dural sinuses.
Subdural hemorrhage is seen in up to 8% of term deliveries,
particularly in traumatic or instrument-­assisted births [38].
Unlike epidural hematoma, subdural hematoma can cross
suture lines and typically forms a hematoma that overlies
and conforms to the shape of the parenchymal convexity
(Fig. 2.31).
Ultrasound evaluation of a subdural hemorrhage requires
use of a high-frequency linear transducer with color Doppler
to assess the position of the cortical veins. A subdural hema-
toma displaces the cortical veins toward the cerebral cortex.
A subdural space filled with blood lacks blood vessels and
none is seen with color Doppler. A small subdural hematoma
without mass effect on the adjacent brain parenchyma has an
excellent prognosis. However, when a subdural hematoma is
located in the infratentorial region or there is mass effect on
adjacent vital structures, especially the brainstem, the poten-
tial consequences are dire and surgical evacuation must be
considered.
injury. (b) Coronal T2-weighted MR image shows the bilateral subdural
fluid collections (asterisks) with associated displacement of a cortical
vein (arrowhead) from the inner table of the skull
2 Brain
Subpial Hemorrhage
Subpial hemorrhage is rare and refers to the accumulation of
blood in the potential space between the pia mater with spar-
ing of and the cerebral cortex. The etiology of subpial hem-
orrhage is controversial but may include birth-related trauma
or cortical vein stasis and thrombosis. Pediatric patients with
subpial hemorrhage present with a variable degree of neuro-
logical symptoms.
On ultrasound, subpial hemorrhage is generally difficult
to distinguish from subarachnoid hemorrhage. It is often
localized to the cortical surface (Fig. 2.32a) without spread
over the convexity as occurs with subarachnoid hemor-
rhage. There may be associated cerebral ischemia with
hemorrhagic infarction occurring deep to the subpial bleed
(Fig. 2.32b).
Parenchymal Hemorrhage
There are many potential causes of parenchymal hemor-
rhage in term infants, including birth trauma, hypoxic–isch-
emic injury (HII), infection, emboli, vein of Galen
malformation (VOGM), and treatment with ECMO. On
ultrasound, an acute hemorrhage appears echogenic. As a
hematoma evolves, it will liquefy and appear hypoechoic to
anechoic. Patients on ECMO are at risk of parenchymal
bleed due to the routine use of anticoagulation. Multi-
compartmental and intra-­parenchymal hemorrhages can be
seen in these patients with a predilection for the cerebellar
hemispheres.
Fig. 2.32 Subpial hemorrhage in a premature infant female with
apnea. (a) Coronal grayscale ultrasound image demonstrates a small,
echogenic subpial hemorrhage (asterisk) localized to the space between
the pia mater and the cortical surface without spread over the convexity.
75
Choroid Plexus Hemorrhage
On ultrasound, the normal choroid plexus is highly echo-
genic with a lobular configuration. Choroid plexus hemor-
rhage is often difficult to diagnose due to the similar
echogenicity of acute hemorrhage and the choroid plexus.
Choroid plexus hemorrhage is suspected when there is ipsi-
lateral occipital horn enlargement, asymmetrical enlarge-
ment of the choroid plexus, or serial ultrasound examinations
demonstrate a sequential decrease in the apparent size of
the choroid plexus.
It is believed that choroid plexus hemorrhage is more com-
mon in full-term infants than in preterm infants. However, cho-
roid plexus hemorrhage is difficult to detect in premature
infants, especially those with grade 2 or higher PIVH. The inci-
dence of choroid plexus bleed in premature infants is, therefore,
probably more common than previously appreciated. Studies
are needed to further evaluate the incidence of choroid plexus
hemorrhage in premature infants with pathologic/ultrasound
correlation [39].
Hypoxic–Ischemic Injury
Hypoxic–ischemic injury (HII) remains a major cause of mor-
tality and morbidity in neonates. HII refers to brain damage
caused by low oxygen supply (hypoxia) and diminished perfu-
sion (ischemia) in the pre- and postnatal periods. HII can lead to
hypoxic–ischemic encephalopathy (HIE).
(b) Coronal grayscale ultrasound image demonstrates a second focus of
subpial hemorrhage (asterisk) with associated cerebral ischemia and
hemorrhagic infarction (H)
76 H. H. R. Kim et al.

Week 37

Week 28

Extreme Preterm Term

Preterm
Week 40
Week 13

Week 0

First Trimester
Second Trimester
Third Trimester

weeks
3 10 13 15 25 28 40

Neural Basic Brain Neuronal Strucutral Synaptic Myelination Begins

Tube Region Migration Formation Pruning Brain Growth Spurt
Formation Formation Begins Begins Begins
Begins

Fig. 2.33 Insults at specific time points during brain maturation lead to predictable types of brain malformation and destruction. “Preterm” is any
live birth before 37 weeks of gestation. “Extreme preterm” is any live birth before 28 weeks of gestation

HIE is a clinical diagnosis of neonatal encephalopathy,
based on the following diagnostic criteria: hypoxia or isch-
emia by Apgar score of < 5 at 10 mins, fetal umbilical artery
acidemia (cord pH < 7.0), encephalopathy, presence of multi-­
organ failure and/or neuroimaging evidence of acute brain
injury. Importantly, imaging represents just one of the multiple
diagnostic criteria for HIE.
HII depends on three factors: gestational age (Fig. 2.33), the
severity of injury, and the underlying mechanism of the insult,
such as stroke or meningitis. Although there is a substantial over-
lap between the neuroimaging characteristics of the different
types of HII, different patterns are seen in preterm and term
infants that depend on the severity and duration of the insult.
Global Hypoxic–Ischemic Injury
Preterm Infants
Preterm infants are prone to ischemic injury due to poor auto-
regulation of cerebral blood flow, an immature cardiorespira-
tory system, and immature myelination of the central nervous
system. The vascular supply of preterm infants less than
32 weeks of gestation is unique and different from that of
older preterm or full-term infants. Instead of cerebral blood
flow from the circle of Willis being directed to the periphery
as occurs in children and adults, the periventricular white
matter is supplied by perforating vessels originating from the
pia mater that are directed centrally. The periventricular
white matter is therefore located further from the origin of
the blood supply, resulting in increased susceptibility to vas-
cular insult.
When mild to moderate hypotension occurs in prema-
ture neonates less than 32 weeks of gestation, periventricu-
lar white matter ischemic injury leads to periventricular
leukomalacia (PVL). Bilateral, fairly symmetrical foci of
white matter necrosis develop around the lateral ventricles,
­particularly in the frontal and occipital lobes. Large necrotic
lesions undergo cavitation in 2–4 weeks and remain cystic
(cystic PVL). Small necrotic lesions do not undergo cavita-
tion or form small cysts that collapse into glial scars (non-
cystic PVL).
The corticospinal tracts and optic radiations are located at
the common sites of periventricular white matter injury, and
therefore the sequelae of PVL can include quadriplegia and/
2 Brain
a b
Fig. 2.34 Periventricular leukomalacia (PVL) in a male born at
29 weeks of gestation. (a) Coronal grayscale ultrasound image of the
brain obtained at 1 day of age reveals increased echogenicity (arrows)
of the right and left periventricular white matter posterior to the bodies
or visual deficits. Injury occurs not only at the time of the orig-
inal insult but also during reperfusion.
Cranial ultrasound is an excellent imaging modality for
the detection and assessment of the severity and evolution
of PVL injury. Immediately after injury, ultrasound find-
ings are generally normal. Within 2 weeks of injury, peri-
ventricular white matter echogenicity increases (Fig. 2.34).
Between 2 and 6 weeks after the insult, cystic cavities may
develop. Cysts can be single or multiple, varying in degree
from a focal cyst adjacent to the ventricle, to diffuse cystic
change throughout the deep white matter (Fig. 2.35).
Typical chronic imaging manifestations of PVL include
a decrease in thickness of the deep white matter with dila-
tion of the lateral ventricles which often display undulating
margins. The changes of PVL need to be distinguished
from a normal peritrogonal echogenic “blush” (Fig. 2.2j)
and Grade 4 PIVH [40].
When severe hypotension occurs in premature neonates
less than 32 weeks of gestation, the most metabolically
active regions of the brain where myelination occurs are
prone to insult. These regions include the thalami, basal gan-
glia, corticospinal tract, hippocampi, and brainstem. The cere-
bral cortex is relatively spared, including the perirolandic
77
of the lateral ventricles. (b) Coronal grayscale ultrasound image of the
brain obtained at 4 weeks of age shows small cystic spaces (arrow-
heads) within the zones of abnormal white matter, more numerous on
the left
cortex, because there are collaterals from the meningeal arte-
rial anastomoses, which involute at term [41]. In addition,
myelination of the perirolandic white matter does not occur
until 35 weeks of gestation.
Term Infants
When mild to moderate hypotension develops in term neo-
nates, blood is shunted to the vital structures of the brain,
including the deep gray nuclei and the brainstem. The vul-
nerable watershed or border zones of the cerebral cortex are
located between the territories supplied by the anterior, pos-
terior, and middle cerebral arteries. The motor cortex is often
affected, especially the innervation to the upper limb, that
can lead to spastic quadriplegia, cognitive deficits, and sei-
zures in later life.
In the setting of severe hypotension, other metabolically
active regions of the brain are affected as well, including
the thalami (especially their ventrolateral aspects), cortico-
spinal tracts, basal ganglia (especially the posterior putam-
ina), perirolandic cortex, hippocampi, and the dorsal
brainstem.
In mild to moderate cerebral edema, there is increased
echogenicity of the subcortical white matter, which accentu-
78
a b
Fig. 2.35 Cystic periventricular leukomalacia (PVL) in an 11-day-old
female born at 28 weeks of gestation. (a) Sagittal color Doppler ultra-
sound image obtained with a linear high-frequency transducer demon-
strates extensive cyst formation (arrow) extending from the periventricular
white matter to the subcortical white matter. (b) Axial FLAIR MR image
ates gray–white matter differentiation (Fig. 2.36a) [42, 43]. In
severe cerebral edema, there is loss of gray–white matter dif-
ferentiation along with blurring of the sulci, interhemispheric
and Sylvian fissures, slit-like ventricles, and decreased size of
the extra-axial spaces (Figs. 2.36b, c and 2.37) [43].
Focal Hypoxic–Ischemic Injury
 rterial Ischemic Stroke
A Patients with perinatal stroke are often male. Approximately
Acute ischemic stroke (AIS) is an acute disturbance of neu-
rological function with symptoms lasting more than 24 hours.
Once thought of as a rare condition in the pediatric popula-
tion, AIS is now recognized as one of the top ten leading
causes of death in children [44]. Clinical presentation differs
depending on age. Infants present with nonspecific symp-
toms such as seizures, hypotonia, lethargy, or, most often, no
clinical symptoms at all. Older children can present in a fash-
ion similar to adults, but due to nonspecific symptoms, the
diagnosis of stroke is often delayed.
H. H. R. Kim et al.
CV
V V
obtained 3 months later demonstrates volume loss (asterisks) of the deep
white matter with ex vacuo dilation of the lateral ventricles (V). CV,
Cavum vergae. Note the smooth brain contours in keeping with marked
prematurity
There are two categories of neonatal acute ischemic
stroke (NAIS): perinatal or presumed perinatal. Perinatal
NAIS is an acute arterial ischemic stroke event occurring
anywhere from 20 weeks of fetal life to the end of the neona-
tal period (28 days). Presumed perinatal NAIS is an acute
arterial ischemic stroke event presenting clinically in an
infant more than 28 days of age with signs of chronic infarc-
tion on neuroimaging. Therefore, presumed perinatal NAIS
is diagnosed retrospectively.
70% of the time, the perinatal stroke occurs via the anterior circu-
lation and 73% of the time it involves the left hemisphere (espe-
cially the MCA territory) [45]. Pediatric patients with presumed
perinatal stroke either have no clinical symptoms or present
with hemiparesis. Etiologies of presumed NAIS include con-
genital heart disease and hypercoagulable states. The etiology
remains unknown in about half of the cases [45].
The ultrasound findings of early NAIS are subtle. Asymmetric
echogenicity of a focal region of the brain such as the thalamus
(Fig. 2.38) or the periventricular white matter can be identified
2 Brain 79

b c

Fig. 2.36 Cerebral edema in two different 1-day-old full term males.
(a) Longitudinal grayscale ultrasound image of the right cerebral hemi-
sphere shows diffusely increased white matter echogenicity as well as
accentuated gray-white matter differentiation. (b) Coronal gray-
scale ultrasound image of the brain in a second patient reveals diffusely
increased parenchymal echogenicity with decreased gray-white matter
differentiation and slit-like frontal horns of the lateral ventricles (arrow-
heads). (c) Axial diffusion-weighted MR image depicts restricted diffu-
sion involving the frontal lobes, the insulae, left occipital lobe and
thalami (arrowheads)
80 H. H. R. Kim et al.

a b

Fig. 2.37 Severe cerebral edema in a 12-day-old full-term male. (a) Axial CT image of the brain demonstrates generalized loss of gray–
Coronal grayscale ultrasound image demonstrates diffusely abnormal white matter differentiation and sulcal effacement consistent with
increased echogenicity of the cortical gyri (asterisks) and effacement of severe hypoxic–ischemic injury. A small amount of layering blood
the CSF spaces consistent with cerebral hypoxic–ischemic injury. (b) (arrowhead) is seen in the left occipital horn

a b

Fig. 2.38 Thalamic stroke in a 2-day-old female. (a) Coronal gray- thalamus. (b) Axial diffusion-weighted MR image demonstrates a small
scale ultrasound image shows subtle increased echogenicity (arrow- area of restricted diffusion (arrow) in the right thalamus consistent with
head) of the right thalamus in comparison to the normal hypoechoic left acute ischemia or infarction
2 Brain 81

(Fig. 2.39). MR imaging is recommended for confirmation and
further characterization of abnormalities.
Venous Sinus Thrombosis
Venous sinus thrombosis is rare in the pediatric popula-
tion, with neonates accounting for up to 40% of venous
sinus thrombosis cases [46]. There is under-diagnosis of
venous sinus thrombosis due to the nonspecific and vari-
able presentation of patients, suboptimal diagnostic imag-
ing techniques, and rapid recanalization of the cerebral
veins. Sagittal sinus thrombosis is the underlying cause in
65% of cases.
Clinical presentation is nonspecific and varies greatly, but
may include seizures, altered mental status, focal neurologi-
cal symptoms, headache, lethargy, and vomiting. In neo-
nates, birth trauma can cause cortical vein disruption and
venous sinus thrombosis. In older children, common head
and neck infections causing fever, dehydration, and anemia
can lead to venous sinus thrombosis. Chronic illnesses such
as a hypercoagulable state, systemic lupus erythematosus,
congenital heart disease, and nephrotic syndrome, also
increase the risk of venous sinus thrombosis.
On ultrasound, venous thrombosis typically appears as a
hyper- to hypoechoic intraluminal focus, depending on the age
of the hemorrhage, with associated absence of flow on color
and spectral Doppler evaluation (Fig. 2.40a–c). When the
thrombus is non-occlusive, it is outlined on color Doppler
imaging. Parenchymal venous infarction is a complication of
cerebral venous thrombosis. On ultrasound, venous infarction
demonstrates increased patchy parenchymal echogenicity
­
extending toward the cortex.
Ultrasound can be an excellent imaging modality to detect
thrombosis when the involved vessels can be adequately iden-
tified and imaged. However, due to the limited acoustic win-
dows of the cranial vault, it can be challenging to detect and
visualize the entire extent of thrombosis involving the poste-
rior aspects of the dural sinuses and the small cortical veins.
When cortical vein thrombosis is identified by ultrasound, MR
imaging should subsequently be performed to fully character-
ize the extent of thrombosis (Fig. 2.40d).
Treatment of dural sinus thrombosis is often conservative
with attention paid to correcting the underlying cause of
thrombosis. When indicated, anticoagulant and thrombolytic
therapy can be administered.
 ickle Cell Disease
S
Sickle cell disease is an inherited red blood cell disorder
that at low oxygen levels distorts the red blood cells into a
crescentic or sickle shape. Sickle-shaped red blood cells
adhere to one other, causing arterial wall damage and
blockage. Pediatric patients with sickle cell disease have a
risk of stroke that is approximately 250 times higher than

a b

Fig. 2.39 Perinatal middle cerebral artery (MCA) stroke in a 2-day-­ frontoparietal periventricular white matter. (b) Axial diffusion-weighted
old male. (a) Coronal grayscale ultrasound image at the level of the MR image demonstrates restricted diffusion involving the left MCA
posterior third ventricle displays an asymmetrical slit-like left lateral territory (asterisk) consistent with acute infarction
ventricle (arrowhead) and increased echogenicity (arrow) of the left
82
a c
b d
Fig. 2.40 Superior sagittal sinus thrombosis in a 6-week-old male.
Coronal (a) and sagittal (b) high-resolution grayscale ultrasound images
demonstrate abnormally increased echogenicity (arrowhead, asterisks)
within the superior sagittal sinus. (c) Sagittal color Doppler ultrasound
that of a healthy child [47]. The risk of stroke increases
after 2 years of age, once the protective effect of fetal
hemoglobin is no longer present. One in ten patients with
homozygous sickle cell disease experiences a stroke
before the age of 20 years with often devastating conse-
quences [48].
H. H. R. Kim et al.
image shows a complete lack of flow (asterisks) in the superior sagittal
sinus consistent with occlusive thrombosis. (d) Sagittal T1-weighted
MR image demonstrates extensive heterogeneously hyperintense signal
(arrows) within the superior sagittal sinus
Stroke Prevention in Sickle Cell Anemia (STOP) was a
multicenter randomized trial initiated in 1995 that success-
fully demonstrated the reliability of transcranial Doppler
(TCD) to stratify patients with sickle cell disease at risk for
developing stroke. Furthermore, it showed the efficacy of
blood transfusion as a treatment to reduce risk.
2 Brain 83

TCD is a simple, inexpensive, fast, and effective way to
monitor the cerebral vessels and guide therapeutic decisions
regarding blood transfusion treatment to reduce the risk of
stroke in patients with sickle-cell disease. TCD ultrasound is
performed through the temporal squamosal bone via a
2–6 MHz transducer in children between 2 and 16 years of
age. With color Doppler, the circle of Willis (Fig. 2.41) can
be identified to sample the velocities of the major cerebral
arteries, particularly the distal internal carotid artery and mid-
dle cerebral artery.
Based on the STOP I [49] and STOP II [50] trials, the time-­
averaged mean of the maximum velocity (TAMMX) of the
terminal internal carotid artery or middle cerebral artery is
measured and interpreted as follows (Table 2.3): normal if all
mean velocities are less than 170 cm/sec, therefore repeat
annually; low conditional if at least one mean velocity is 170–
185 cm/sec, therefore repeat in 3–6 months; high conditional
if at least one mean velocity is 186–199 cm/sec, ­therefore
repeat in 1–3 months; abnormal if at least one mean velocity is
200 cm/sec or higher, therefore repeat within 1–2 weeks; and

Anterior Anterior
cerebral artery communicating
Middle artery
cerebral artery Internal carotid
Part of temporal artery
lobe removed to Posterior
reveal middle communicating
cerebral artery artery
Posterior Posterior
cerebral artery cerebral artery
Basilar artery Superior
cerebellar artery
Anterior
spinal artery
Pontine arteries
Vertebral artery

Part of cerebellum Anterior inferior

removed to reveal cerebellar artery
posterior cerebral Posterior inferior
artery cerebellar artery

Fig. 2.41 Diagram of the arterial supply to the brain. The circle of
Willis forms a circulatory anastomosis between the anterior and the
posterior arterial systems via the anterior and the posterior communi-
cating arteries. The internal carotid arteries supply the anterior circula-
tory system. The vertebral arteries supply the posterior circulatory
system. During transcranial Doppler (TCD) examination, the circle of
Willis is identified with color Doppler, and velocities of the major cere-
bral arteries are sampled, with particular attention paid to the distal
internal carotid and middle cerebral arteries

Table 2.3 Transcranial Doppler (TCD) result and clinical guidance based on Stroke Prevention in Sickle Cell Anemia (STOP) I and II
trials for patients with sickle cell disease
Stroke risk categories Velocity criteria Clinical guidance and recommendation
Normal All TAMMX less than 170 cm/sec Repeat TCD annually
Low conditional At least one TAMMX of 170–185 cm/sec Repeat TCD in 3–6 months
High conditional At least one TAMMX of 186–199 cm/sec Repeat TCD in 1–3 months
Abnormal At least one TAMMX of 200 cm/sec or higher Repeat TCD within 1–2 weeks.
Discuss risk of stroke and possible prophylactic treatment such
as blood transfusion
Inadequate Unable to obtain right or left middle cerebral Use other imaging modality such as CTA or MRA
and/or internal carotid arteries
TAMMX, Time-averaged mean of the maximum velocity; CTA, computed tomography angiography; MRA, magnetic resonance angiography
84
a b
Fig. 2.42 Transcranial Doppler (TCD) ultrasound images of a 6-year-­
old male with sickle cell anemia. (a) Color and spectral Doppler ultra-
sound image obtained via a transtemporal window demonstrates the
circle of Willis. Normal arterial waveforms are depicted in the right
terminal internal carotid artery with the time-averaged mean of the
inadequate if no readings from the right or left middle cerebral
or internal carotid arteries could be obtained. An abnormal
TAMMX greater than 200 cm/s has been associated with a
40% chance of stroke within 3 years [51].
It is thought that an elevated TAMMX (Fig. 2.42) in
patients with sickle cell disease is due to anemia, vessel ste-
nosis caused by a damaged wall, and/or vessel dilation caused
by hypoxia. Transfusion reduces TAMMX by improving
these abnormalities to some degree and may explain the suc-
cessful treatment response of reducing the stroke risk in these
patients [52, 53].
Infectious Brain Disorders
Overall, 2–3% of congenital brain anomalies are due to peri-
natal infection [54]. When the infection is transmitted in the
first or second trimesters of pregnancy, the neuroepithelium
is destroyed, leading to a spectrum of hypoplasia, defective
organogenesis, congenital malformations, and migration dis-
orders. When the infection is transmitted in the third trimes-
ter, destructive processes occur in the brain parenchyma,
such as gliosis, necrosis, and dystrophic parenchymal
calcifications.
H. H. R. Kim et al.
maximum velocity (TAMMX) measuring 110 cm/s. (b) Color and spec-
tral Doppler ultrasound image obtained via a transtemporal window
reveals an abnormally increased TAMMX in the right middle cerebral
artery measuring 202 cm/s
Viral Infections
The most common congenital viral infections are referred to
as TORCH infections: Toxoplasmosis, Other, Rubella,
Cytomegalovirus (CMV), and Herpes simplex virus (HSV).
The “Other” infections include syphilis, varicella-zoster, mumps,
parvovirus B19, and human immunodeficiency virus (HIV).
Most TORCH infections cause mild symptoms in pregnant
mothers but can lead to serious morbidity and mortality in the
fetus. TORCH infections are transmitted via the placenta with
the exception of HSV and HIV which are acquired during pas-
sage through an infected birth canal at the time of delivery.
Most of the imaging findings of TORCH infections are
nonspecific, and diagnosis generally requires specific anti-
body detection and viral cultures. However, there are certain
features that are characteristic of the different congenital
infections that can help guide further patient evaluation. CMV
is the most common TORCH infection, affecting almost 1% of
all newborns in the United States [55]. The second most com-
mon TORCH infection is toxoplasmosis.
The pre- and postnatal ultrasound imaging findings of
CMV infection include periventricular calcification and sub-
ependymal cysts (Fig. 2.43) [56]. Eventually, gliosis occurs
leading to brain atrophy, which then causes prominent CSF
2 Brain
a b
Fig. 2.43 Congenital cytomegalovirus (CMV) in a 13-day-old boy. (a)
Sagittal grayscale ultrasound image reveals an enlarged lateral ventricle
with subependymal cysts (arrowhead) and calcifications (arrow). (b)
Coronal T2-weighted MR image demonstrates punctate dark signal
(arrow) corresponding to calcification in the periventricular region.
spaces, ventricular dilation, and microcephaly. Mineralized
thalamostriate arteries appear as echogenic, linear branching
structures (Fig. 2.44). This appearance of the thalamostriate
vessels is not specific to CMV infection and has been
reported in infants with a variety of other conditions [57], as
well as a normal variant.
Other imaging findings seen in infants with congenital
CMV infection include lissencephaly and localized polymi-
85
Hyper­intense subependymal cysts (black arrowhead) are noted along
with gliosis. There is polymicrogyria (white arrowheads) in both frontal
lobes. (c) Sagittal T1-weighted MR image shows extensive subependy-
mal cysts and gliosis (arrows) in the periventricular region, with associ-
ated ex vacuo dilation of the lateral ventricle (V)
crogyria. Toxoplasmosis causes similar imaging findings but
more characteristically results in cortical and basal ganglia
calcifications, as well as hydrocephalus in some cases.
Inflammation of the ventricles causes hydrocephalus due to
obstruction of CSF flow.
HSV type 2 (HSV-2) is a leading cause of neonatal
meningoencephalitis transmitted via an infected birth canal.
HSV-2 infection is a more common cause of meningoen-
86
cephalitis in neonates, while HSV type 1 (HSV-1) infection
is more common in immunocompromised older children
and adults. Newborns infected with HSV-2 have a late-onset
presentation, often 2 weeks after birth, with symptoms of
lethargy, irritability, seizures, apnea, and fever. The mortal-
Fig. 2.44 Mineralized thalamostriate vessels in a 32-week gestational
age male with congenital cytomegalovirus (CMV) infection. Sagittal
grayscale ultrasound image shows a branching pattern of linear, echo-
genic vessels (arrows) in the thalamus. This is a nonspecific finding that
can be seen in a number of clinical settings, including CMV infection
as well as a normal variant
Fig. 2.45 Herpes simplex virus type 2 (HSV-2) meningoencephalitis
in a 17-day-old male. (a) Coronal grayscale ultrasound image shows
abnormalities of both cerebral hemispheres, more on the left, character-
ized by increased echogenicity of the white matter and cystic changes
(asterisk) extending from the ependymal surface to the subcortical
H. H. R. Kim et al.
ity rate is up to 15% [58]. HSV-2 causes nonspecific zones
of cerebral edema, which can destroy gray and white matter
and lead to cystic changes (Fig. 2.45) [59]. Unlike HSV-1,
the temporal lobes are not involved.
Newly emerging viruses affecting newborns include Zika
virus, enterovirus, and human parechovirus-3. Zika virus is a
member of the mosquito-borne flavivirus family. Infection
during pregnancy causes microcephaly and congenital brain
lesions, including cortical malformations, callosal dysgene-
sis, and ventriculomegaly [60].
Bacterial Infections
Meningitis, an inflammation of the pia-arachnoid and CSF
spaces, is a late manifestation of neonatal sepsis. Since the
introduction of Haemophilus influenzae type b (Hib) vaccina-
tion, H. influenzae is no longer the leading cause of neonatal
meningitis in the United States. Common causative organisms
are Group B streptococci (50%), Escherichia coli (20%), and
Listeria monocytogenes (10%) [61]. Less common bacterial
infections include Enterococci and Staphylococcus aureus.
Cronobacter sakazakii is a class of Enterobacteriaceae which
has been recently linked to powdered infant formula. C. saka-
zakii can cause periventricular hemorrhage, ischemic injury,
and cerebral abscess [62].
white matter. There is sulcal (S) thickening of the left cerebral hemi-
sphere with loss of normal gray–white matter differentiation. (b)
Coronal contrast-enhanced, T1-weighted, fat-suppressed MR image
demonstrates diffuse leptomeningeal enhancement (arrowheads), con-
sistent with meningitis
2 Brain
In sepsis, there is bacterial seeding to the choroid
plexus, resulting in CSF infection and ventriculitis. As an
initial response to the infection in the brain, inflammatory
V V
Fig. 2.46 Sulcal thickening in a 20-day-old female with Group B
streptococcal sepsis and meningitis. A coronal grayscale ultrasound
image shows sulcal thickening (arrowheads) and enlarged ventricles
(V) due to increased CSF production and decreased reabsorption
a b
S S
V V
Fig. 2.47 Meningitis in a 7-month-old male. Coronal (a) and sagittal (b) high-resolution grayscale ultrasound images demonstrate a large, complex
subarachnoid empyema (asterisks) containing debris. A reactive subdural effusion (S) is also identified. V, Frontal horns of lateral ventricles
87
exudate accumulates in the sulci, causing sulcal thicken-
ing (>2 mm) and increased echogenicity [63]. Sulcal
thickening is the earliest cranial ultrasound finding of
meningitis (Fig. 2.46).
In the acute phase of bacterial meningitis, there is
increased CSF production and decreased CSF resorption,
causing enlargement of the ventricles. In the later phase of
meningitis, exudates in the ventricles can form septations
resulting in obstructive hydrocephalus. On ultrasound, ven-
triculitis manifests as thickened, irregular walls of the epen-
dymal lining of the ventricles. Once infection spreads to the
meninges and subarachnoid spaces, other complications
occur, such as empyema formation, cerebritis, cerebral
abscess, venous thrombosis, and infarction.
On ultrasound imaging, these complications (especially
small empyemas) are difficult to differentiate from subdural
effusions. However, use of a high-frequency linear trans-
ducer permits delineation of a large empyema and its internal
complexity (Fig. 2.47), which is helpful in differentiation
from a simple, anechoic subdural effusion. A subdural effu-
sion is generally reactive, sterile, and of no clinical signifi-
cance. A brain abscess is a rare complication of meningitis.
On ultrasound, an abscess appears as a heterogeneous collec-
tion within the brain parenchyma with a thick rim and
hypoechoic contents that may include a fluid-fluid level or
debris (Fig. 2.48).
88
Fig. 2.48 Brain abscess in a 5-week-old male with Group B streptococcus infection. Coronal (a) and sagittal (b) grayscale ultrasound images
show a relatively well-defined, hypoechoic cystic lesion (arrows) with internal septations in the left parietal lobe
Fungal Infections
CNS fungal infections are a less common cause of meningo-
encephalitis compared to bacterial infections in neonates.
The most common is Candida albicans, which affects pre-
mature and immunocompromised infants. Premature infants
with candida infection can present with seizures, poor feed-
ing, irritability, and bradycardia. Microabscesses are difficult
to visualize with ultrasound, but complications of obstructive
hydrocephalus or infarction can be seen. Treatment includes
antifungal and supportive therapy and if there is obstructive
hydrocephalus, shunting is considered.
Neoplastic Brain and Ventricular Disorders
Neoplasms of the Brain
Although brain neoplasms are a leading cause of death in
the pediatric population, brain neoplasms in neonates are
rare, accounting for less than 1% of brain tumors in chil-
dren [64]. With the advent of prenatal ultrasound and MR
imaging, the prenatal diagnosis of brain tumors has been
increasing in frequency. In these patients, postnatal MR
imaging of the brain is acquired for management purposes.
Depending on the location and grade of the brain tumor,
therapy includes a combination of surgery, chemotherapy,
and/or radiation.
Two-thirds of brain neoplasms in neonates occur in the
supratentorial region, whereas in older children there is a
H. H. R. Kim et al.
predilection for the infratentorial region [65]. Common
neonatal brain neoplasms include teratoma, astrocytoma,
embryonal tumors, and choroid plexus papilloma. Affected
children present with a rapidly increasing head circumfer-
ence, bulging fontanelles, vomiting, and focal neurological
symptoms, including seizures.
Cranial ultrasound is helpful for the screening of CNS
neoplasms but has a limited role in pretreatment characteriza-
tion, which is best accomplished by MR imaging. However,
identification of the location of the tumor can help narrow the
differential diagnosis. Teratomas commonly occur in the
suprasellar and pineal regions and contain mixed solid and
cystic foci with calcification and fat. Astrocytomas arise from
the optic pathways, particularly in patients with neurofibro-
matosis type 1, as well as from the thalamus and hypothala-
mus. Less frequent tumors include ependymoma, germinoma,
ganglioglioma, and meningioma.
Neoplasms of the Ventricle
Ventricular tumors arise from the choroid plexus, particularly
from the trigone of the lateral ventricle in children as com-
pared to the fourth ventricle in adults [66]. The majority of
choroid plexus tumors are papillomas rather than carcinomas.
Ultrasound demonstrates an enlarged, lobulated choroid
plexus mass with associated ventriculomegaly due to increased
CSF volume and/or obstruction (Fig. 2.49). Prominent vascu-
larity can be seen within the tumor with color Doppler. Spinal
drop metastases occur with both papilloma and carcinoma,
2 Brain
Fig. 2.49 Choroid plexus papilloma in a 3-month-old female. Sagittal
(a) and coronal (b) grayscale ultrasound images demonstrate a well-
circumscribed, homogenously echogenic mass (asterisks) extending
from the trigone (arrowhead) to the temporal horn of the right lateral
ventricle (LV). The left lateral ventricle is dilated. (c) Sagittal contrast-
enhanced, T1-weighted MR image shows avid enhancement of the
although they are more common with carcinoma [67]. MR
imaging of the brain and spine must be performed prior to
surgical intervention.
Vascular Brain Disorders
The International Society for the Study of Vascular Anomalies
(ISSVA) classification of vascular anomalies is an attempt to
categorize the two main groups of disorders, vascular tumors
89
mass (asterisk) extending from the trigone of the right lateral ventricle
to the temporal horn (arrow). There is surrounding edema (arrowheads)
in the right parietal, temporal, and occipital lobes. (d) Coronal contrast-
enhanced, T1-weighted, fat-suppressed MR image shows the mass
(asterisk) with surrounding edema (arrowheads), leftward midline shift,
and left lateral ventricular (LV) enlargement
and malformations, using a consistent nomenclature. Since
Mulliken and Glowacki first classified these lesions in 1982
[68], their description has been repeatedly updated, most
recently in 2018 [69], by incorporating new knowledge of
genetic disorders into the classification.
The most important vascular neoplasm affecting the cra-
nium is infantile hemangioma, which is discussed later in this
chapter under vascular scalp lesions. Vascular malformations
are further categorized into high-flow and low-flow lesions.
High-flow malformations include arteriovenous malforma-
90
tion (AVM) and arteriovenous fistula (AVF). Low-­flow mal-
formations include venous malformation (VM), lymphatic
malformation (LM), and capillary malformation (CM). These
high- and low-flow malformations can be solitary (simple) or
occur as part of a more complex disorder (combined). Many
syndromes are associated with vascular anomalies, including
Klippel–Trenaunay syndrome and Sturge–Weber syndrome.
High-Flow Malformations
AVM is a congenital high-flow vascular anomaly that lacks
a normal capillary network, and has innumerable small,
abnormal AV connections that bypass a normally controlled,
high-­resistance vascular bed. AVF is a direct connection
between an artery and a vein without an intervening capil-
lary bed. Patients with a high-flow malformation typically
present with high-output cardiac failure, neurological defi-
cits, and seizures. There is an increased risk of hemorrhage
and vascular steal phenomena, resulting in ischemia and
hydrocephalus.
Vein of Galen malformation (VOGM) consists of dilation
of the median prosencephalic vein associated with AVFs
within the wall of the vein or multiple shunts communicating
with the anterior portion of the vein. There are two main types
of VOGM: choroidal (type 1) and mural (type 2) based on
feeding vessels and location of the shunts. Approximately
80% of VOGM are of the choroidal type [70].
Choroidal VOGM has multiple arterial feeders from the
thalamoperforator, choroidal, pericallosal, and anterior cere-
bral arteries, forming AVFs to the median prosencephalic vein.
This is a high-flow lesion that can lead to high-output conges-
tive heart failure and hydrops immediately after birth. In the
rarer mural type of VOGM, there are fewer arterial feeders
connecting to the median prosencephalic vein. Mural VOGM
presents later in life with clinical symptoms of hydrocephalus
or seizure and does not cause high output heart failure.
On fetal and postnatal ultrasound, a markedly dilated,
cystic vascular lesion is seen in the midline, posterior to the
third ventricle (Fig. 2.50). Spectral Doppler evaluation
reveals disordered, arterialized blood flow within the aneu-
rysmally dilated portion of the malformation and arterializa-
tion of the draining veins (Fig. 2.50d). In the choroidal type,
significantly increased flow within the arterial feeders sup-
plying the lesion is seen. On parasagittal or mastoid views,
abnormal dilation of the falcine and straight sinuses and tor-
cular herophili is seen.
On grayscale images, parenchymal calcifications and
encephalomalacia related to chronic ischemic brain injury
are sometimes observed. Subarachnoid and intraparenchy-
mal hemorrhage are occasionally seen, as the vessels of the
malformation are fragile and tend to bleed easily.
Management of VOGM includes treatment of congestive
heart failure and endovascular coiling of the lesion. Ultrasound
H. H. R. Kim et al.
imaging is useful in follow-up to assess the response to endo-
vascular treatment.
Dural AVF is an abnormal direct connection between an
artery and a draining vein, usually involving the meningeal
arteries, with drainage into the transverse or sigmoid sinuses.
Low-Flow Malformations
The most commonly encountered low-flow malformations in
the brain are developmental venous anomaly (DVA), cavern-
ous malformation, and capillary telangiectasia. None of
these lesions is detectable with ultrasound and they are typi-
cally diagnosed by MR imaging.
Hydrocephalus
CSF is mainly produced by the ependymal lining and cho-
roid plexus of the lateral, third, and fourth ventricles, with
the remainder produced by the arachnoid lining of the
brain and spine. CSF flows from the lateral ventricles into
the third ventricle via the foramen of Monro, into the
fourth ventricle via the cerebral aqueduct, and through the
cisterna magna and basal cisterns via the foramina of
Magendie and Luschka.
Hydrocephalus develops when there is an obstruction to
the flow of CSF, or when there is an imbalance between CSF
production and resorption. There are two types of obstructive
hydrocephalus: non-communicating and communicating.
Non-communicating hydrocephalus occurs when there is
a blockage along the ventricular pathway of CSF flow, typi-
cally at the narrowest sites such as the foramina of Monro,
cerebral aqueduct, and the outlet of the fourth ventricle.
Communicating hydrocephalus occurs when there is
inadequate reabsorption of CSF, with obstruction in the non-­
ventricular pathway of CSF flow, typically as a result of scar-
ring or fibrosis in the subarachnoid space that can follow
hemorrhage or infection (e.g., meningitis), or by CSF seed-
ing of tumor. Rarely, it may be due to functional impairment
of the arachnoidal granulations which are located along the
superior sagittal sinus and are the site of CSF reabsorption
into the venous system.
Ventricular size can be monitored through serial cranial
ultrasound examinations. Ultrasound imaging is also helpful
in differentiating between ex vacuo dilation from periven-
tricular white matter loss and progressive hydrocephalus due
to an obstructive lesion.
The Levene index is used to measure ventricular size in
infants up to 40 weeks of gestational age [71]. It is measured
on a coronal image at the level of the foramen of Monro as
the horizontal length from the midline falx to the most lateral
margin of the frontal horn. After 40 weeks of gestational age,
the ventricular index (VI) is used. This is the ratio of the dis-
2 Brain 91

a b

c d

Fig. 2.50 Vein of Galen malformation in a 3-day-old male. (a) Coronal
grayscale ultrasound image shows a dilated cystic lesion (asterisk) pos-
terior to the third ventricle. (b) Coronal color Doppler ultrasound image
demonstrates the vascular nature (asterisk) of the lesion. (c) Sagittal
color Doppler ultrasound image shows multiple arterial feeders from
thalamoperforator, choroidal, pericallosal (arrowhead), and anterior
cerebral arteries supplying the malformation (asterisk). (d) Sagittal
color and spectral Doppler ultrasound image of the brain reveals arteri-
alized flow in the straight sinus. (e) Frontal radiograph of the chest
shows cardiomegaly related to high-output congestive heart failure
92
a b
Fig. 2.51 Measurement of the anterior horn width (AHW) and thalamo-­
occipital distance (TOD). (a) Coronal grayscale ultrasound image dem-
onstrates AHW which is the oblique distance (solid white line) of the
widest width of the anterior horn of the lateral ventricle. (b) Sagittal gray-
tance between the most lateral aspect of the ventricles divided
by the bi-parietal diameter.
Additional popular methods include the anterior horn
width (AHW) and thalamo-occipital distance (TOD) [72].
AHW is measured as the oblique distance of the widest
width of the anterior horn of the lateral ventricle on a cor-
onal image (Fig. 2.51a). Thalamo-occipital distance
(TOD) is measured from the outer margin of the thalamus
abutting the choroid plexus to the outer margin of the
occipital horn on a parasagittal image (Fig. 2.51b). AHW
and TOD are more sensitive in detecting progressive ven-
tricular dilation compared with VI [73]. AHW greater
than 6 mm is considered abnormal. Given that the occipi-
tal horn is often the first to dilate after hemorrhage, the
measurement of TOD has a potentially important clinical
role [74, 75].
Fifteen percent of preterm infants with severe PIVH
develop progressive hydrocephalus [76]. In this group of
patients, CSF drainage can improve cerebral blood flow and
possibly reduce further brain injury. Treatment involves
shunt catheter placement to divert the flow of CSF and
thereby decrease intracranial pressure. A ventriculoperito-
neal catheter is commonly used to divert CSF to the perito-
neal cavity. In patients with cerebral aqueductal stenosis,
endoscopic third ventriculostomy can be performed.
H. H. R. Kim et al.
scale ultrasound image demonstrates the TOD which is the oblique dis-
tance (solid white line) from the outer margin of the thalamus abutting the
choroid plexus to the outer margin of the occipital horn
Benign External Hydrocephalus
Benign external hydrocephalus (BEH), also known as benign
macrocrania of infancy, is a condition defined as prominent
CSF spaces with associated macrocephaly in infancy. The
etiology is unclear but is thought to be due to delayed CSF
reabsorption by immature arachnoid villi. There is often a
family history of macrocephaly. Males are affected more fre-
quently than females. BEH is the most common cause of
macrocephaly, with infants presenting from 2 to 7 months of
age with a head circumference above the 95th percentile.
These patients are typically neurologically normal other than
an occasional transient delay of ­psychomotor development
[77]. The condition usually resolves by the age of 2 years.
Ultrasound is the modality of choice for evaluating sus-
pected BEH. Findings include prominent, bilaterally sym-
metric subarachnoid spaces along the frontoparietal convexities
and a widened interhemispheric space (Fig. 2.52a). The remain-
der of the study is normal, with no evidence of obstructive
hydrocephalus.
Use of a high-­frequency linear array transducer with
Doppler is helpful in demonstrating the cortical veins cross-
ing a prominent CSF space to enter the superior sagittal
sinus and thereby excluding a chronic subdural fluid collec-
tion or hygroma (Fig. 2.52b). In addition, an interhemi-
2 Brain
a b
Fig. 2.52 Benign external hydrocephalus in a 2-month-old male with a
rapidly enlarging head circumference. (a) Coronal grayscale ultrasound
image demonstrates prominent symmetric CSF spaces (asterisks) over
the frontoparietal convexities with widening of the interhemispheric
spheric width (IHW) measurement can be obtained. An
IHW greater than 5.0 mm in neonates or 8.5 mm in 1-year-
olds is considered abnormal [78]. BEH is a self-limited con-
dition that typically does not require intervention. Rarely,
patients with benign external hydrocephalus can present
with a subdural hematoma [79].
Scalp Masses
“Lumps and bumps” of the scalp are a common complaint
and a source of concern for many parents. Scalp lesions tend
to be small, and ultrasound provides superb resolution when
compared to other imaging modalities.
Since scalp lesions are often superficial, a high-frequency
(9–3 MHz, 12–5 MHz, or 17–5 MHz) linear array transducer
is typically used with a copious amount of transducer gel.
Imaging can be optimized by centering the focal zone of the
ultrasound transducer at the depth of the lesion. For soft tis-
sue or vascular scalp masses, color and spectral Doppler
imaging should be performed to assess for vascular flow and
to identify the waveforms as arterial and/or venous.
Characterization of a scalp lesion depends on patient age,
laterality, and imaging appearance. Some lesions may require
biopsy or resection in order to obtain a final diagnosis. Con­
genital scalp lesions usually present under the age of 5 years
93
S
space. (b) Coronal color Doppler ultrasound image demonstrates corti-
cal veins (arrowheads) coursing through the prominent CSF spaces.
There is no displacement of these veins to suggest a chronic subdural
fluid collection or hygroma. S, Superior sagittal sinus
[80, 81]. Most are benign and include birth trauma-­related
hematoma (including ossified cephalohematoma), dermoid/
epidermoid cyst, cephalocele, reactive lymph nodes, heman-
gioma, and sinus pericranii. In patients older than 5 years of
age, aggressive lesions are more frequently seen, such as
Langerhans cell histiocytosis, metastasis, and primary bone
tumor of the cranial vault.
Congenital Scalp Lesions
Dermoid/Epidermoid Cyst
Dermoid and epidermoid cysts are the most commonly
encountered masses in the scalp and skull in the pediatric
population [82]. They are not true neoplasms, consisting of
sequestrations of stratified squamous epithelium. The main
difference between the two lesions is the presence of epithe-
lial appendages in dermoid cysts [83]. Dermoid cysts tend to
occur near the midline and close to sutures [84], while epi-
dermoid cysts occur further from the midline in the lateral
skull/scalp. Affected patients often present for imaging after
a mass is incidentally noted by care providers. Patients are
generally asymptomatic, and the lesion is non-tender when
palpated.
Based on ultrasound imaging alone, the two entities cannot
be differentiated. Both lesions appear as a well-­circumscribed,
94
thin-walled cyst without vascular flow (Fig. 2.53). The internal
contents are anechoic to slightly echogenic. The cyst tends to
abut the skull, which causes smooth bone remodeling without
cortical destruction. Surgical excision is performed for both
diagnosis and treatment [82].
Cephalocele
A cephalocele is an umbrella term used to describe outward
herniation of the intracranial contents through osseous defects
in the cranial vault or skull base. Depending on the herniated
intracranial contents, cephaloceles are further categorized as
D (Fig. 2.54). Surgical resection is the treatment of choice [85].
Fig. 2.53 Dermoid cyst in a 5-month-old male. Transverse grayscale
ultrasound image demonstrates a well-­ circumscribed, thin-walled
cystic structure (D) in the subcutaneous tissues of the scalp without
vascular flow (not shown). The lesion abuts the skull without asso­
ciated cortical destruction. An intact right lambdoid suture (arrow) is
identified
a b
G
Fig. 2.54 Nasal glioma in a 6-day-old male. (a) Transverse grayscale
ultrasound image demonstrates a left paramedian heterogeneous soft
tissue mass (G), which extends into the nasal cavity (N) through a
defect (arrow) in the nasal bone. (b) Sagittal T2-weighted, fat-­
suppressed MR image shows a soft tissue mass (G) at the bottom of the
H. H. R. Kim et al.
meningoencephalocele or encephalocele (brain parenchyma);
meningocele (meninges and CSF); gliocele (CSF); and atretic
(dura, fibrous tissue, and atretic brain tissue). Common loca-
tions are near the midline in the occipital, parietal, and fronto-
nasal regions. When a cephalocele is small, it is difficult to
differentiate from a dermoid or epidermoid cyst on physical
examination, especially when it is located in the midline.
The role of ultrasound imaging is to assess for underlying
osseous defects of the cranial vault and to characterize the
herniated intracranial contents. Unlike a dermoid or epider-
moid cyst, a cephalocele is associated with a skull defect that
allows the cephalocele to communicate with the underlying
intracranial structures.
A nasal glioma is similar to a cephalocele but with an absence
of intracranial connections. Nasal glioma contains dysplastic
glial cells and typically occurs at the bridge of the nose
Lymph Node
A benign lymph node in the scalp is a common lump noticed
by parents and caretakers. As elsewhere in the body, a benign
lymph node demonstrates a central fatty hilum, a short axis
length of less than 10 mm (except for submental and sub-
mandibular nodes that can measure up to 15 mm in short
axis), and a smooth cortical contour. Abnormal size, loss of
the central fatty hilum, and a cystic or necrotic appearance
warrant further investigation.
G
N
nasal bridge with extension into the nasal cavity (N). A thin fibrous
stalk (arrow) arising from the mass and connecting to the dura is identi-
fied without an associated CSF component. The signal characteristics
of the lesion were similar to gray matter on all MR imaging sequences
2 Brain 95

Extracranial Birth Trauma

There are unique extracranial scalp and cranial vault injuries

that occur in the setting of birth trauma. Hematoma can F
develop between the various layers of the scalp and cranial
vault, including the subcutaneous fat of the scalp, the galea
aponeurotica, the subgaleal fascia, and the periosteum of the
cranial vault [86].
A hematoma localized to the subcutaneous tissues of the
scalp is a caput succedaneum. Caput succedaneum occurs in
up to 25% of vaginal births [87]. It is associated with a small
volume of blood loss and resolves after 48–72 hours. On Fig. 2.56 Cephalohematoma in a 5-day-old male. Coronal grayscale
ultrasound imaging, a subcutaneous fluid collection is seen ultrasound image shows a complex, hypoechoic fluid collection (F) in
the scalp overlying the right parietal bone. The fluid collection does not
that crosses suture lines. extend beyond the sagittal suture (arrow)
A subgaleal hematoma develops when bleeding occurs
beneath the galea aponeurotica and is often associated with a
vacuum-assisted vaginal delivery [88]. Subgaleal hematoma
can result in massive blood loss within the subgaleal poten-
tial space that extends from the orbits to the occipital region. F
On ultrasound imaging, a sharply demarcated hematoma is
seen crossing suture lines, and can shift with gravity or man-
ual compression (Fig. 2.55).
A cephalohematoma occurs in the subperiosteal space of
the outer table of the cranial vault, where the blood accumu-
Fig. 2.57 Partially ossifying cephalohematoma in a 20-day-old male
lates between the pericranium and the cranial bone. Analogous
born via vacuum-assisted extraction. Coronal color Doppler ultrasound
to an epidural hematoma, a cephalohematoma is confined by image demonstrates a subperiosteal fluid collection (F) overlying the
the periosteum and therefore does not cross suture lines occipital bone. There is partial ossification (arrowhead) of the collection
(Fig. 2.56). Cephalohematoma occurs in 2–3% of newborns with associated posterior acoustic shadowing
[89] and often resolves spontaneously after 2–3 weeks. When
it persists beyond the perinatal period, a cephalohematoma Vascular Scalp Lesions
can ossify (Fig. 2.57) and be incorporated into the outer table
of the cranial vault; such patients present with a palpable, Infantile Hemangioma
hard lump. Infantile hemangioma is a benign vascular tumor of small
capillary-sized blood vessels. It is the most common head
and neck vascular tumor of childhood. On the scalp, it
appears as a bright red strawberry-like firm nodule, which
is generally absent or small at birth but usually appears
within the first 6 weeks of life.
On ultrasound, hemangioma demonstrates a well-circum-
S scribed hypo- or hyperechoic mass with hyper-vascularity
consisting of both arterial and venous waveforms on spectral
Doppler analysis (Fig. 2.58). Unlike other vascular tumors,
infantile hemangiomas usually involute spontaneously.
Unless there are associated complications such as rapid
growth with bleeding and/or ulceration, most infantile hem-
angiomas do not require treatment. When necessary, the
majority of infantile hemangiomas will respond to oral beta-
Fig. 2.55 Subgaleal hematoma in a 1-month-old male. Coronal gray- blockers (propranolol) [90].
scale color Doppler ultrasound image shows an anechoic fluid collec-
tion (S) in the scalp overlying the periosteum and extending across the Infantile scalp hemangioma can occur as a component of
sagittal suture (arrow) PHACE syndrome, an association of disorders characterized by
96
a b
H
Fig. 2.58 Infantile scalp hemangioma in a 5-month-old male. (a)
Longitudinal grayscale ultrasound image of the scalp demonstrates a well-
circumscribed, hypoechoic mass (H) with mildly lobulated margins. (b)
Longitudinal color Doppler ultrasound image reveals enlarged blood ves-
a large infantile hemangioma on the face, scalp, and/or neck, as
well as defects in the brain, blood vessels, eyes, heart, and chest.
Sinus Pericranii
Sinus pericranii is an abnormal venous lake within the
scalp that drains into the dural sinus through the transdi-
ploic space of the skull [91]. It develops as a result of an
abnormal communication between the intra- and extra-cra-
nial veins, usually via an emissary transosseous vein. It is
commonly located in the midline of the frontal bones of the
skull or at the vertex. Affected patients present with a soft
H. H. R. Kim et al.
sels both within and surrounding the mass. (c) Longitudinal color Doppler
ultrasound image with spectral analysis shows low resistance arterial blood
flow within the lesion
and boggy scalp mass, which dilates when the child cries
and with any other maneuver that increases intra-abdomi-
nal pressure.
Anechoic tubular structures are seen on grayscale imaging,
which demonstrate vascular flow on color Doppler imaging and
venous waveforms on spectral waveform analysis (Fig. 2.59).
Associated skull defects can be seen on plain radiography or
CT.
Management is typically conservative as embolization or
surgical resection can lead to complications such as paren-
chymal venous infarction [92].
2 Brain
a b
S
c d
Fig. 2.59 Sinus pericranii in a 3-year-old female. (a) Coronal gray-
scale ultrasound image shows a well-defined anechoic lesion (S) in the
subcutaneous tissues of the scalp. There is no underlying bone destruc-
tion. A patent sagittal suture (arrow) is seen abutting the lesion. (b)
Coronal color Doppler ultrasound image reveals the vascular nature of
the lesion (S) which is connected to a transcalvarial vessel (arrow)
extending through the left parietal skull. The extra-calvarial portion of
Suture Evaluation
Craniosynostosis
Craniosynostosis is an abnormal early closure of the cra-
nial sutures resulting in skull deformity. Craniosynostosis
can be primary or secondary. Primary craniosynostosis is
often idiopathic and can be further divided into syndromic
and non-syndromic (isolated) forms, while secondary cra-
niosynostosis is due to identifiable causes, such as under-
lying metabolic bone disease, skeletal dysplasia, or
decreased intracranial volume from shunting or extensive
brain injury.
The incidence of craniosynostosis is low: three to ten
cases per 10,000 live births [93]. The syndromic form of
97
S
the lesion overlies the patent sagittal suture (asterisk). (c) Longitudinal
color Doppler ultrasound image with spectral analysis reveals venous
flow within the vessel. (d) Sagittal contrast-enhanced, T1-weighted, fat-
suppressed MR image shows a homogenously enhancing vascular
lesion (arrow) in the scalp near the vertex. Tiny trans­cranial vessels
coursing through the left parietal skull are better visualized by
ultrasound
primary craniosynostosis often involves more than one
suture and presents with other clinical and radiographic
findings, such as midface hypoplasia, hypertelorism, and/or
limb abnormalities. Apert, Crouzon, Pfeiffer, and Saethre–
Chotzen syndromes are commonly associated with cranio-
synostosis [94]. Patients with primary craniosynostosis
typically present in the neonatal period with a misshapen
skull. The secondary form of craniosynostosis can present
later in infancy.
Requests for suture evaluation with ultrasound to exclude
craniosynostosis have increased substantially following the
advent of the “Safe to Sleep” and “Back to Sleep” campaigns
to prevent sudden infant death syndrome (SIDS), which
launched in 1994 with resultant increased rates of positional
posterior plagiocephaly.
98
The cranial sutures are evaluated using a high-frequency
transducer to assess the outer and inner tables of the skull.
With craniosynostosis, there will be bony ridges instead of
an outer and inner skull table due to premature suture closure
(Fig. 2.60). Sagittal craniosynostosis is the most common
a d
Fig. 2.60 Unilateral coronal craniosynostosis in a 6-week-old female.
Transverse grayscale ultrasound images of the left coronal suture (a)
near the midline demonstrate an abnormal bony ridge (arrow) in the
expected location of the suture, and (b) complete bony fusion (arrow)
near the left ear. (c) Transverse grayscale ultrasound image of the nor-
mal right coronal suture (arrow) demonstrates patency. (d) Frontal
H. H. R. Kim et al.
abnormality, accounting for roughly 50% of non-syndromic
primary craniosynostosis [95].
It is important to note that the metopic suture can nor-
mally close at as early as 3 months of age [96]. In infants
more than 3 months of age, extreme caution should be exer-
radiograph of the skull shows elevation of the superolateral corner of
the left orbital wall (arrow), the so-called “harlequin eye” deformity
due to near-complete fusion of the left coronal suture (arrowhead). (e)
3D reformatted CT image confirms near-complete fusion of the left
coronal suture (arrowhead)
2 Brain
cised when diagnosing metopic suture craniosynostosis. A
prominent osseous ridge in the expected location of the
metopic suture should be observed, along with a trigonoce-
phalic configuration of the skull.
Positional Plagiocephaly
Positional plagiocephaly has increased in incidence in
association with supine positioning of infants during
sleep. It is defined as a deformation of the posterior or
dependent portions of the skull without true craniosynos-
tosis. It is important to exclude craniosynostosis in infants
with plagiocephaly and to educate care providers to man-
age this entity conservatively by changing the sleep posi-
tion of the infant [97].
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 Spine
3
Maddy Artunduaga, Domen Plut, Abbey J. Winant,
Ricardo Restrepo, and Edward Y. Lee

Abbreviations and young infants due to the absence of ionizing radiation,

bFFE Balanced fast field echo
CSF Cerebrospinal fluid
CT Computed tomography
M Motion
MR Magnetic resonance
Introduction
Assessment of the pediatric spine and intraspinal contents is
critical since permanent neurologic deficits can develop if
spinal disorders are not diagnosed and treated in a timely
fashion. Understanding the complex anatomy and develop-
ment of the spine and intraspinal structures allows for opti-
mal recognition and diagnosis of normal anatomic variants
and pathologic entities in children.
Ultrasound is the imaging modality of choice for the initial
assessment of the spine and intraspinal structures in newborns
M. Artunduaga (*)
Department of Radiology, Pediatric Radiology Division,
University of Texas Southwestern Medical Center, Children’s
Health Medical Center, Dallas, TX, USA
e-mail: maddy.artunduaga@utsouthwestern.edu
D. Plut
Unit of Pediatric Radiology, Clinical Institute of Radiology,
Ljubljana University Medical Centre, Ljubljana, Slovenia
A. J. Winant
Department of Radiology, Boston Children’s Hospital and Harvard
Medical School, Boston, MA, USA
R. Restrepo
Department of Interventional Radiology and Body Imaging,
Nicklaus Children’s Hospital, Miami, FL, USA
E. Y. Lee
Division of Thoracic Imaging, Department of Radiology,
Boston Children’s Hospital and Harvard Medical School,
Boston, MA, USA
portability, no need for sedation, and cost-effectiveness com-
pared to magnetic resonance (MR) imaging and computed
tomography (CT) [1–4]. The non-ossified posterior spinal
elements provide an excellent acoustic window for visualiz-
ing the spinal cord, identifying the location of the conus, and
assessing the nerve roots [1–3, 5, 6]. Initial spinal ultrasound
can also help determine whether further evaluation with MR
imaging is required [2, 3].
The most common indications for ultrasound evaluation of
the pediatric spine include midline or paramedian soft tissue
masses, dimples and other cutaneous lesions of the back
(hairy tufts, skin tags, skin discoloration, and hemangiomas);
suspected spinal cord abnormalities (e.g., tethering); postop-
erative evaluation for cord re-tethering; caudal regression
syndrome spectrum; lumbar puncture guidance; and f­ ollowing
injury (e.g., birth trauma or traumatic lumbar puncture) [3].
This chapter reviews the technical aspects of pediatric
spine ultrasound and provides an overview of the normal
development and anatomy of the spine. A broad spectrum of
normal variants and pathologic entities that can affect the
pediatric spine is presented, with an emphasis on character-
istic clinical and imaging findings.
Technique
Patient Positioning
Ultrasound examination of the spine is performed with the
patient prone. A small pillow or rolled towel under the lower
abdomen/pelvis can sometimes aid in the acquisition of
images by increasing the distance between the spinous pro-
cesses and thereby increasing the size of the acoustic win-
dow [1, 3, 7–9]. Placing the patient in a lateral decubitus
position or prone on a caregiver’s lap may at times be useful.
Flexion of the neck allows for optimal visualization of the

© Springer Nature Switzerland AG 2021 103

H. J. Paltiel, E. Y. Lee (eds.), Pediatric Ultrasound, https://doi.org/10.1007/978-3-030-56802-3_3
104
craniocervical junction, when assessment of this region is
indicated [5, 8].
Other positions are sometimes used, such as a supported
seating position with the patient bending forward in cases of
failed lumbar puncture, since gravity can distend the lower
portion of the thecal sac with cerebrospinal fluid (CSF) [3].
An upright position may aid lumbar puncture guidance and
better demonstrate a meningocele, and an anterior scanning
approach can be used to assess anterior meningoceles and
presacral masses [3, 7].
Ultrasound Transducer Selection
Ultrasound of the pediatric spine is typically performed
using high-frequency linear transducers on the order of
7–15 MHz [1, 3, 6, 8, 9, 10]. Images of the craniocervical
junction are best acquired with a small footprint vector trans-
ducer or with a curved transducer [3]. A standoff pad or
application of a thick layer of gel on the skin can improve
visualization and assessment of superficial subcutaneous
abnormalities such as a dermal sinus tract [3, 6].
Imaging Approaches
Ultrasound examination of the infant spine should be per-
formed in a warm room so that normal patient body tempera-
ture is maintained [3]. Feeding prior to evaluation and the
use of warm gel are also recommended to decrease infant
motion and fussiness that may potentially limit the study [1].
If feeding is not possible, a pacifier dipped in glucose solu-
tion may comfort the child and decrease motion during
image acquisition [3].
Images are obtained by scanning directly over the midline
of the back. The lumbosacral junction is an important land-
mark when evaluating the lumbosacral spine. The lumbosa-
cral junction is identified in the longitudinal plane as a
change in orientation of the vertebral bodies [1, 3, 5, 11].
Identification of vertebral body level can also be achieved by
counting inferiorly from the last rib (presumably the 12th
rib), or by counting superiorly from the coccygeal tip,
although these methods are less reproducible [1, 3, 5, 10].
Ossification of the first coccygeal segment can be vari-
able. At birth, the coccygeal ossification centers are more
rounded compared to the rectangular configuration of the
sacral vertebral ossification centers [3, 6, 9]. In indetermi-
nate cases, a radiopaque BB marker can be placed on the
skin over the tip of the conus medullaris after localization
with ultrasound, and a radiograph of the lumbosacral spine
M. Artunduaga et al.
can subsequently be obtained to determine the correspond-
ing vertebral level [3, 6, 7].
Images of the intraspinal contents, osseous structures, and
subcutaneous soft tissues are obtained in both longitudinal
and transverse planes using grayscale and color Doppler as
needed [5, 6]. The appearance of the spinal cord, filum termi-
nale, and cauda equina is assessed, and the mobility of the
nerve roots is determined. A search is made for masses and
abnormal fluid collections. Vertebral body morphology and
any subcutaneous soft tissue abnormalities are also noted [1,
3, 5, 6].
Motion (M)-mode ultrasound imaging is helpful in evalu-
ating nerve root movement. In patients who have undergone
spinal cord release, M-mode imaging can be particularly
useful in the detection of cord re-tethering, by demonstrating
absence of normal nerve root pulsations [3, 5, 8]. Panoramic
ultrasound imaging with an extended field of view is a con-
venient method for demonstrating the termination level of
the spinal cord and its relationship to the spine [7].
Assessment of the entire spine with panoramic imaging
from the craniocervical junction to the coccyx may be indi-
cated, as in patients with a Chiari II malformation [3, 6, 9].
Oblique, paramedian, gluteal, or anterior approaches can
occasionally be advantageous [1, 8, 9].
Normal Development and Anatomy
Normal Development
The central nervous system develops during the first 8 weeks
of gestation, beginning in the third week with the process of
neurulation, where the primitive neural plate folds into a
cylindrical neural tube [5, 10]. Once the neural tube has
closed, around the 28th day, canalization begins: the distal
portion of the neural tube fuses with the caudal cell mass,
and the primitive spinal cord begins to form [6, 10].
At 38 days, development of the conus medullaris, ven-
triculus terminalis, and filum terminale is initiated as part
of a process termed retrogressive differentiation, where the
caudal cell mass and the caudal portion of the neural tube
involute by apoptosis [5, 6, 10]. By the end of the seventh
week, formation of the spinal and intraspinal structures is
complete (Figs. 3.1, 3.2, 3.3, and 3.4) [1].
Normal Anatomy
On ultrasound imaging, the pediatric spinal cord appears
hypoechoic with a central hyperechoic line that corresponds
3 Spine 105

a b c d
Neural plate Neural fold Neural groove Neural tube closed

Fig. 3.1 Diagram of normal neural tube development. (a) On day 17 of
life, the margins of the neural plate (ectoderm that becomes neuroecto-
derm after signaling from the notochord – dark purple) begin to thicken
to form the neural folds. (b) During the third week of gestation, the
neural groove develops as the neural folds begin to close in the midline,
forming the neural tube. (c) At the same time, the cutaneous ectoderm
detaches from the neural tube and fuses in the midline through the pro-
cess of disjunction. (d) Neural crest cells that lie at the borders of the
neural folds (light purple) migrate between the ectoderm and the neural
tube to give rise to the peripheral nerves, among other structures.
Closure of the neural tube completes the stage of neurulation around the
28th day of life

Vertebrae

Dura
mater
Spinal Conus
L
cord Medullaris
(L1-L2)
L
Root of
first sacral Pia
nerve Mater Cerebrospinal
L Fluid in
S Subarachnoid
Arachnoid Space
Dorsal
root
C
ganglion Root of first
S sacral nerve
(part of cauda
First trimester equina)

End of thecal
C sac (S2)

S Filum
Second – Third terminale
trimester

Adult

Fig. 3.2 Diagram of the normal appearance of the spinal and intraspi-
nal structures during different stages of development and growth. After
the first trimester of fetal development, the spine grows more rapidly
than the spinal cord. The caudal portion of the spinal cord ascends,
tapers, and forms the conus medullaris, which typically terminates near
the L1–L2 level shortly after birth. The lumbosacral roots elongate and
form the cauda equina. The conus medullaris continues caudally as the
filum terminale, which terminates on the dorsal aspect of the coccyx.
C, Coccygeal vertebrae; L, lumbar vertebrae; S, sacral vertebrae

to the central canal (Fig. 3.5) [2, 5, 9]. In the transverse plane,
the spinal cord is larger in diameter in the cervical, lower
thoracic, and thoracolumbar junction levels due to the pres-
ence of the normal cervical and lumbar plexuses. On trans-
verse imaging, the spinal cord appears oval-shaped in the
cervical region and rounder at lower levels [5, 8, 9, 11].
The nerve roots of the cauda equina are hyperechoic and
course dependently within the spinal canal; they normally
move with the cardiac-related pulsations of the CSF [3, 6, 9].
Nerve root pulsations may not be very noticeable in infants
less than 2 months of age, and M-mode imaging may be
more sensitive, as previously described.
106 M. Artunduaga et al.

Posterior Posterior
intermediate median septum
septum
Lateral funiculus
Posterior gray
horn
Anterior gray
horn Root filaments

Dorsal root
ganglion

Pia mater

Anterior
median
fissure

Arachnoid

Dura mater

Fig. 3.3 Diagram of normal spinal cord anatomy. Three meningeal layers cover the spinal cord (from outer to inner): dura mater, arachnoid mater,
and pia mater. The subarachnoid space contains cerebrospinal fluid (CSF) and is located between the arachnoid mater and the pia mater

Complete
vertebra

Spinal cord

Spinal
nerves

Fig. 3.4 Diagram of normal spinal canal contents and lumbosacral spine structures
3 Spine
* *
L1 L2
b c
Fig. 3.5 Normal ultrasound anatomy of the lumbosacral spine. (a)
Longitudinal extended field of view grayscale ultrasound image shows
the conus medullaris (white asterisk) terminating at the L1–L2 level and
the echogenic central canal (arrow). The cauda equina (black asterisk)
courses along the dependent portion of the spinal canal. White arrow-
The thecal sac normally terminates at S2 [3, 5, 9]. The
unossified vertebrae and intervertebral discs are hypoechoic,
while ossified structures are echogenic with posterior acous-
tic shadowing [5, 7].
Spinal Cord
The spinal cord terminates distally as the conus medullaris,
with a caudal cord-like fibrous extension, the filum termi-
nale, that fuses inferiorly with the dura mater and attaches to
the dorsal aspect of the coccyx (Fig. 3.6) [5, 8, 9, 11]. The
107
head, Filum terminale; black arrowhead, ossification center of first coc-
cygeal segment. Transverse grayscale ultrasound images demonstrate
(b) the rounded lumbar spinal cord (arrow), and (c) the more inferiorly
located conus medullaris (arrow). The dorsal and ventral lumbosacral
nerve roots (arrowheads) comprise the cauda equina
filum terminale is mildly echogenic and should measure no
more than 2 mm in thickness [1–3, 9, 12].
The normal position of the conus medullaris is above
the L2–L3 disc space in most infants [3, 13]. When the
conus terminates between the L2–L3 disc space and the
mid aspect of the L3 vertebral body in an asymptomatic
patient, and there are no other abnormal ultrasound find-
ings, no further evaluation is typically necessary (Fig. 3.7)
[13]. For premature infants with a conus medullaris termi-
nating at the L3 vertebral body level, a short interval fol-
108
low-up study is recommended between 40 weeks and a
corrected age of 6 months, as the position of the conus
often normalizes [3, 9].
Fig. 3.6 Normal spinal cord in a 2-month-old male with a sacral dim-
ple. Longitudinal grayscale ultrasound image demonstrates the
hypoechoic cord with a normal hyperechoic central canal (arrowhead),
the conus medullaris (white arrow) terminating at L1–L2, and a normal
filum terminale (black arrow)
Fig. 3.7 Incidental borderline low-lying conus medullaris in a 3-day-old term female with a sacral dimple. Longitudinal grayscale ultrasound
image demonstrates the conus (arrow) terminating at the level of the superior endplate of L3. Nerve root pulsations were normal. No lesion was
identified
a b
Fig. 3.8 (a) Transient dilation of the central canal in a 2-week-old
male with a sacral dimple. Longitudinal grayscale ultrasound image
demonstrates mild dilation (arrow) of the most caudal aspect of the cen-
tral canal, immediately cranial to the conus medullaris. (b) Normal
M. Artunduaga et al.
Central Canal
The central canal of the spinal cord is the interface between
the myelinated ventral white commissure and the central
portion of the anterior median commissure [5, 8]. On ultra-
sound evaluation the central canal appears as an echogenic
line referred to as the central echo complex [5, 8]. Rarely, the
central canal may appear slightly dilated in newborns and
young infants. Isolated mild central canal dilation with no
additional ultrasound abnormalities is considered a transient
and benign finding and requires no additional imaging fol-
low-up (Fig. 3.8) [5, 8].
Ventriculus Terminalis
Ventriculus terminalis, also known as the “fifth ventricle”,
is an ependymal-lined CSF-filled cavity within the conus
medullaris. It is a common incidental finding on spinal
ultrasound and is of no clinical significance. It results
from a failure of regression of the embryonic terminal
ventricle in the conus medullaris. On ultrasound it appears
as a well-defined, ovoid cystic structure in the distal-most
comparison in a 31-day-old male with a sacral dimple. Longitudinal
grayscale ultrasound image demonstrates a non-dilated central canal
(arrow) also immediately cranial to the conus medullaris
3 Spine 109

aspect of the conus medullaris (Fig. 3.9) [5, 8, 9, 10]. A
ventriculus terminalis can also be seen as a well-circum-
scribed, T2 hyperintense cystic structure in the conus
medullaris on MR imaging [10].
Filar Cyst
A filar cyst is a cystic structure in the filum terminale [9, 10].
Although its etiology is uncertain, some authors have pro-
posed that it represents a developmental variant that regresses
over time as it is not identified in adults [14]. The incidence
of filar cyst ranges from 12% to 25% in patients younger
than 16 months of age, and its frequency is inversely related
to age [4, 6, 14].
Frequently confused with ventriculus terminalis, a filar
cyst occurs in the most cranial portion of the filum termi-
nale, whereas a ventriculus terminalis affects the tip of the
conus. On ultrasound, a filar cyst appears as a well-defined,
­fusiform, anechoic to hypoechoic cystic structure in the
filum terminale, immediately distal to the conus medullaris
(Fig. 3.10) [6, 10, 11].
Filar cysts do not contain septations, calcification, or mural
nodules, and Doppler evaluation demonstrates an absence of
vascularity. On MR imaging, the best sequence for follow-up
of a filar cyst is a heavily T2-weighted sequence [15].
Fatty Filum
Fatty filum refers to a fat-containing filum terminale, a fre-
quently asymptomatic finding [12]. On ultrasound, a fatty
filum appears markedly echogenic and is often thickened. If
a fatty filum is normal in thickness (i.e., <2 mm) and there

Fig. 3.9 Ventriculus terminalis in a 1-day-old female. Longitudinal grayscale ultrasound image demonstrates an ovoid cystic structure (arrow)
near the tip of the conus medullaris

a b

Fig. 3.10 Filar cyst in a 1-month-old female with a sacral dimple. Transverse (a) and longitudinal (b) grayscale ultrasound images demonstrate a
fusiform cystic structure (arrows) in the proximal filum terminale, just distal to the tip of the conus medullaris (M)
110 M. Artunduaga et al.

a b

Fig. 3.11 Fatty filum in a 2-month-old male with a sacral dimple. Longitudinal (a) and transverse (b) grayscale ultrasound images show a thick-
ened (>2 mm), echogenic filum terminale (arrows)

a b

Fig. 3.12 Pseudosinus tract in an 18-day-old male with a sacral dim- coccyx. No mass or fluid is identified along its course. (b) Longitudinal
ple. (a) Longitudinal grayscale ultrasound image shows a tract (arrow) color Doppler ultrasound image does not demonstrate internal vascular-
in the subcutaneous soft tissues that connects the skin to the tip of the ity of the tract (arrow) or surrounding hyperemia

are no additional ultrasound abnormalities, it is considered a
normal variant (Fig. 3.11) [12]. In general, MR imaging is
recommended in patients with a fatty filum to evaluate for
additional anatomic abnormalities.
 ositional Nerve Root Clumping (“Pseudomass”)
P cyx is more prominent than usual and can clinically present as a
A “pseudomass” refers to the ultrasound appearance of a
transient intraspinal mass due to clumping of the cauda
equina nerve roots. Most often, a pseudomass is observed
when a patient is scanned in a supine or lateral decubitus
position and resolves when imaging is performed in a prone
position [10, 11].
and/or there is clinical evidence of CSF leakage, further eval-
uation with MR imaging should be pursued [10].
Dysmorphic Coccyx
Dysmorphic coccyx is a normal variant where the tip of the coc-
palpable abnormality [10, 11]. On ultrasound imaging, a dys-
morphic coccyx appears prominent and is dorsally oriented,
without a sinus tract or other associated abnormality (Fig. 3.13).
Congenital Spinal Anomalies

Pseudosinus Tract Congenital anomalies occur when there is a disruption of nor-

A pseudosinus tract is a linear fibrous cord that extends from
a skin dimple to the coccyx and appears hypoechoic by ultra-
sound (Fig. 3.12) [10, 11]. Its location is an important feature
to recognize since a true dermal sinus tract is usually located
at a more cranial level [10]. Nonetheless, if fluid is visualized
along the tract, an associated soft tissue mass is identified,
mal embryologic development [12]. Entrapment of mesoder-
mal components such as fat can occur when there is premature
ectodermal separation from the neural tube. Spinal dysra-
phism and caudal abnormalities (e.g., caudal regression syn-
drome) can result from failed neurulation and impairment of
caudal cell mass development and involution, respectively [8,
3 Spine 111

a b

Fig. 3.13 Dysmorphic coccyx in a 1-day-old male with a sacral dim- scale ultrasound image shows the unossified coccyx (arrow) slightly off
ple. (a) Longitudinal grayscale ultrasound image depicts acute angula- midline and minimally tilted towards the right. No soft tissue sinus tract
tion of the unossified coccyx in its mid portion (arrowhead) and a more is identified
superficial position of its distal portion (arrow). (b) Transverse gray-

Bone
Cord

Osseous Osseous Osseous

defect defect defect

Normal spine Spina bifida Meningocele Myelomeningocele

occulta

Fig. 3.14 Diagram comparing the normal spine to different forms of spinal dysraphism

Table 3.1 Ultrasound features of open spinal dysraphism

12]. Ultrasound is helpful in the detection and initial evalua-
tion of these congenital spine abnormalities. Lesion Ultrasound features
Meningocele Rare, more commonly part of closed spinal
dysraphism spectrum (see Table 3.2)
Myelocele Neural placode attached to skin
Spinal Dysraphism No expansion of the subarachnoid space
Myelomeningocele Neural placode attached to skin
Spinal dysraphism results from a failure of neurulation, dis- Expansion of the subarachnoid space results in
dorsal protrusion of neural placode
junction, canalization, and/or retrogressive differentiation
that normally occur during embryologic development [7].
Spinal dysraphism can be classified as open or closed, and Spinal dysraphism often results in progressive and per-
the distinction is based on the absence or presence of skin manent neurological deficits, depending on the level of
coverage, respectively (Fig. 3.14, Tables 3.1 and 3.2) [7]. involvement. Clinical presentation ranges from asymptom-
112 M. Artunduaga et al.

Table 3.2 Ultrasound features of closed spinal dysraphism atic to a readily apparent lesion on physical examination
Lesion Ultrasound features [16]. Neonatal spine ultrasound screening allows for the
Simple early recognition of clinically suspected spinal dysraphism,
Filar lipoma Hyperechoic fat-containing mass with a sensitivity comparable to MR imaging, and facili-
Arises from the filum terminale
tates the implementation of timely and appropriate treat-
Dermal sinus Hypoechoic tract, connects skin to spinal
canal ment that can lead to better clinical outcomes in affected
More cranial in location compared to patients [16]. Typically, defects of the posterior elements
pseudosinus tract are best detected with ultrasound images obtained in a
Tight filum syndrome Normal position of conus medullaris transverse plane [3].
Lack of nerve root pulsations on cine
images and/or M-mode imaging
Complex Open Defect
Neurenteric cyst/fistula
Cyst with gut signature located ventral to Open defect spinal dysraphism is characterized by the
the cervicothoracic spine absence of skin coverage (Fig. 3.15) and is associated with
Fistulous connection between mesentery/ progressive and permanent neurological deficits [1].
bowel and/or dorsal skin
Diastematomyelia Hemicords
One dural sac, or two separate dural sacs Meningocele
with osseous/cartilaginous septum The vast majority of meningoceles manifest as a skin-­
Lipomyelocele Neural placode/lipomatous tissue interface covered defect. Rarely, a meningocele can occur as a non-­
within spinal canal skin-­covered lesion. Meningocele is discussed later in this
No expansion of subarachnoid space
Lipomyelomeningocele Neural placode/lipomatous tissue interface
chapter in the “Closed Defect” section.
outside spinal canal
Expansion of subarachnoid space Myelocele and Myelomeningocele
Terminal Caudal herniation of large syrinx into a Myelocele and myelomeningocele are slightly more com-
myelocystocele large dorsal meningocele
mon in females than males [8]. Myelomeningocele is found
Subarachnoid space communicates only
with meningocele (not with syrinx) in approximately 98% of non-skin-covered cases of spinal
Meningocele Herniation of dura mater, arachnoid, and dysraphism [9, 11]. In both entities there is dorsal exposure
subarachnoid space of the neural placode which is abnormally attached to the
Does not contain neural elements skin, with eversion of the mesenchymal structures, such as

Myelomeningocele

Spinal cord

Incomplete
vertebra

Spinal
nerves

Fig. 3.15 Diagram illustrating an open dorsal defect with protrusion of the spinal cord, nerve roots, and meninges
3 Spine
the vertebral pedicles and laminae [8]. In myelomeningo-
cele, there is also expansion of the ventral subarachnoid
space that results in dorsal protrusion of the neural placode
(Fig. 3.16) [8, 9]. In myelocele, the placode is embedded
within the dorsal skin plane, and there is no expansion of the
subarachnoid space [8, 9].
These anomalies are typically associated with Chiari II
malformation. Chiari II malformation results from under-
development of the hindbrain due to chronic CSF leakage
during fetal life [1]. Myelomeningocele and myelocele
are associated with tethered cord (70–90%), hydromyelia
or syringomyelia (40–80%), diastematomyelia (20–40%),
and arachnoid cysts (2%) [8]. Spinal cord tethering (and
re-tethering after surgery) is a common complication that
can be seen after repair of myelomeningocele and myelo-
cele [8].
Although ultrasound of the spine can help to distinguish
between myelocele and myelomeningocele, it is not often
performed prior to initial surgery due to concern for direct
damage and infection of the exposed neural elements [1, 9].
When spinal ultrasound examination is performed, the
entire spine and the craniocervical junction should be eval-
uated because of the association between myelomeningoce
le/myelocele and additional spinal abnormalities (e.g.,
a b
Fig. 3.16 Myelomeningocele in a neonate with an obvious lower back
mass. (a) Longitudinal grayscale ultrasound image demonstrates a
CSF-filled lesion that contains neural elements (arrow). (b) Sagittal
113
hydromyelia, spinal lipoma, arachnoid cyst and diastema-
tomyelia) [1, 9].
Prompt surgical treatment of myelomeningocele/ myelocele
is necessary because exposure of the neural placode can result
in additional injury and secondary inflammation [1, 9]. In utero
repair of open spinal dysraphism can significantly improve
postnatal neurologic function in some patients [1].
Closed Defect
A closed defect encompasses any skin-covered absence or
underdevelopment of the posterior elements of the spine
with associated protrusion of any of the intraspinal elements
(neural placode, meninges, or adipose tissue) (Fig. 3.17)
[12]. The presence of cutaneous stigmata (e.g., hemangioma,
hairy tuft) should raise suspicion for a skin-covered spinal
anomaly [11]. Ultrasound plays an important role in the
detection of closed defects which can present with or without
an associated mass [6, 11, 17].
Simple Closed Defects
Simple closed defects include filar lipoma, dermal sinus, and
tight filum syndrome. These entities are designated as “sim-
ple” since they are not associated with significant intraspinal,
skeletal, or systemic abnormalities.
T2-weighted, fat-suppressed MR image demonstrates extension of the
lesion (arrow) into the soft tissues of the back
114 M. Artunduaga et al.

Tuft of hair

Incomplete
vertebra

Spinal cord

Spinal
nerves

Fig. 3.17 Diagram of a closed defect with protrusion of the nerve roots and meninges with a hairy tuft on the skin of the back

Affected patients may present with a small dimple and

L4 L5
Fig. 3.18 Filar lipoma in a newborn male with an asymmetric gluteal
cleft. Longitudinal grayscale ultrasound image shows a thickened, echo-
genic filum terminale (black arrow). There is a tethered cord as evi-
denced by termination of the conus medullaris at L4–L5 (white arrow)
associated discharge. On ultrasound, the tract appears as a
hypoechoic band that extends from the skin surface to the
intraspinal canal structures (Fig. 3.19) [17]. Treatment of
congenital dermal sinus involves complete resection of
the tract as well as intradural exploration. Prophylactic
surgical removal of the congenital dermal sinus tract per-
mits neurological and bladder function to be maintained
[19, 20].
In tight filum syndrome, the filum terminale is tethered
despite the conus medullaris being in a normal position [12].
Associated findings include a lipoma or a central cyst [1, 8].
On ultrasound, cine imaging will show an absence of normal
nerve root pulsations [1, 12]. Treatment consists of surgical
sectioning of the lower end of the filum.

Complex Closed Defects

Filar lipoma is a fat-containing soft tissue mass that arises
from the filum terminale and is thought to result from abnor-
mal canalization and retrogressive differentiation during
embryonic development [12]. Filar lipoma is often associ-
ated with myelomeningocele and tethered cord [12]. On
ultrasound, a filar lipoma appears as a hyperechoic, avascu-
lar solid mass that replaces the filum terminale (Fig. 3.18).
When patients have tethered cord syndrome and a low-lying
conus, surgical intervention is usually performed, with sec-
tioning of the filum terminale [18]. Asymptomatic patients
do not require intervention.
Dermal sinus is an abnormal communication between the
skin surface and the intraspinal structures [17]. Dermal sinus is
thought to occur due to partial disjunction between the cutane-
ous and neural ectoderm, resulting in an epithelial-­lined tract
that persists during normal ascent of the spinal cord in fetal life.
In about half of affected patients, the dermal sinus tract ends in
an epidermoid or dermoid cyst that lies either in the spinal canal
or in the spinal cord with associated tethering.
Complex closed defects are associated with significant intra-
spinal, skeletal, or systemic abnormalities, including neuren-
teric cyst, neurenteric fistula, and diastematomyelia.
Neurenteric cyst and neurenteric fistula result from
incomplete resorption of the neurenteric canal during embry-
onic development, with abnormal communication between
the neuroectoderm and the endoderm [1]. Neurenteric cysts
commonly occur ventral to the spinal cord at the level of the
cervicothoracic spine. A neurenteric fistula connects the spi-
nal canal with the bowel mesentery and/or the dorsal skin
and is associated with significant infection.
Ultrasound may demonstrate a gut signature of the wall
of a neurenteric cyst [11]. MR imaging is more sensitive for
the detection and delineation of the abnormalities associ-
ated with neurenteric cysts and fistulae [1].
Surgical resection is the first-line treatment for neuren-
teric cysts and fistulae. When possible, total excision is the
ideal treatment given the association between partial resec-
tion and cyst recurrence [21]. However, vertebral anoma-
3 Spine
a b
S1
Fig. 3.19 Dermal sinus tract in a 2-day-old female with a tethered cord
and intraspinal lipoma. (a) Longitudinal grayscale ultrasound image of
the lumbosacral spine reveals a low-lying spinal cord (arrowhead) at the
level of S1 terminating in an intraspinal lipoma (asterisk). A dorsal der-
Fig. 3.20 Diastematomyelia in two different patients. (a) 2-month-old
male with a sacral dimple. Transverse grayscale ultrasound image
shows two hemicords (asterisks). (b) 20-week gestational age fetus
lies or extensive adhesions to neural structures can make
complete resection difficult and hazardous.
Diastematomyelia refers to the presence of two hemi-
cords that may share the same dural sac or have two different
dural sacs separated by an intervening osseous or cartilagi-
nous septum [7–9]. Since up to 85% of cases involve the
thoracic or lumbar spine, examination of the entire spine
should be performed [7, 9].
Affected children commonly present with cutaneous stig-
mata such as a tuft of hair, skin tag, or skin discoloration [8].
115
*
mal sinus tract (arrow) extends from the intraspinal lipoma to the skin
surface. (b) Coronal T1-weighted MR image shows the dermal sinus
tract (arrowhead) extending to the intraspinal lipoma (asterisk)
with complex spinal dysraphism. Coronal grayscale ultrasound image
shows widening of the lower lumbar spine with a centrally located bony
septum (arrow)
Although most hemicords reunite caudally, those with a low
cleft can occasionally co-­exist with two distinct coni medul-
laris [8].
Diastematomyelia should be documented with transverse
ultrasound images and off-center scanning in order to elimi-
nate potential artifacts [3]. The diagnosis of diastematomy-
elia can be completely overlooked on longitudinal imaging
since only a focal bulge or an irregular contour may be seen
(Fig. 3.20) [7]. Associated anomalies include hydromyelia
(50% of the cases), vertebral segmentation/fusion anoma-
116 M. Artunduaga et al.

lies, myelomeningocele, lipoma, dermal sinus, and dermoid the syrinx and the meningocele, the latter communicates
[7–9]. with the subarachnoid space within the thecal sac [8, 9].
Surgery is performed in symptomatic patients and includes When this abnormality occurs elsewhere in the spinal canal,
decompression and removal of the spur. This can be accom- it is referred to as a nonterminal myelocystocele [6, 7].
plished with or without resection and repair of the duplicated Associated abnormalities include isolated cloacal exstrophy
dural sacs [22]. or cloacal exstrophy related to OEIS complex (omphalocele,
cloacal exstrophy, imperforate anus, and spinal anomalies) [9].
Lipomatous Tissue and Dural Defect Spine ultrasound shows dilation of the central canal which
(Lipomyelomeningocele, Lipomyelocele) herniates into a large meningocele through a site of dysra-
Lipomyelomeningocele and lipomyelocele present as fat-­ phism [6, 7].
containing soft tissue masses that may be mainly intradural Meningocele consists of herniation of the dura mater,
in location or can involve the subcutaneous soft tissues [1, arachnoid, and CSF-containing subarachnoid space (Fig. 3.23)
11]. These lipomatous lesions with associated dural defects [7, 9]. Skin-covered meningoceles most frequently occur in
account for almost 90% of cases of closed dysraphism with a the lumbosacral spine followed by the cervical spine [9]. The
back mass and 50% of closed dysraphism with cutaneous spinal cord may be tethered [9].
stigmata [17]. Dorsal meningocele is rare and manifests with a posterior
The difference between lipomyelomeningocele and lipo- back mass (Fig. 3.24) [6, 17]. Anterior meningocele is an
myelocele is that the lipoma-placode interface occurs out- uncommon autosomal dominant disorder that typically pres-
side of the spinal canal and is associated with expansion of ents as a presacral mass and represents one component of the
the CSF-containing subarachnoid space in lipomyelomenin- Currarino triad [9, 11, 17]. A lateral meningocele herniates
gocele, while the interface lies within the spinal canal with- through an enlarged intervertebral foramen, typically in the
out subarachnoid expansion in lipomyelocele [6, 11]. thoracic or lumbar spine, and is associated with neurofibroma-
These anomalies have a better prognosis with greater pres- tosis, Marfan syndrome, and Ehlers-Danlos syndrome [8, 11].
ervation of neurologic function compared to open spinal dysra- Ultrasound shows a fluid-filled cyst that communicates
phism, because skin coverage does not allow intrauterine CSF with the thecal sac and can occasionally contain bands of pia
leakage or neural placode exposure to the amniotic fluid [1]. mater, arachnoid, or dura mater [9]. Surgical treatment is
Ultrasound evaluation demonstrates a variable-sized fatty required for all of these lesions.
mass adjacent to a distorted neural placode and appears
hyperechoic or isoechoic to the regional subcutaneous fat,
with or without expansion of the anterior CSF space Tethered Cord
(Figs. 3.21 and 3.22) [1, 11]. Ultrasound can also demon-
strate cord tethering or hydromyelia [1, 17]. In general, MR Tethered cord (i.e., a low-lying conus medullaris) refers to
imaging is required to provide an evaluation of the full extent the abnormal anchoring and stretching of the filum terminale
of both of these lipomatous lesions [6]. Surgical treatment of and cauda equina [7, 12]. It is characterized by increased
lipomyelomeningocele and lipomyelocele is performed to cord tension which worsens with patient growth [7, 12].
prevent the development of neurological deficits [23]. Affected children can present at any age with weakness,
abnormal reflexes, bladder and/or bowel dysfunction [4, 12].
CSF-Containing Defects Associated findings include a thickened filum terminale,
Terminal myelocystocele refers to the herniation of a large dysraphism, spinal masses, sinus tracts, and the VATER
syrinx, a fluid-filled cavity within the spinal cord, through a association (Vertebral defects, Anal atresia, Tracheo-
large dorsal meningocele in the caudal portion of the spinal Esophageal fistula (TEF) with esophageal atresia, Radial and
canal [6, 9]. Although there is no communication between renal dysplasia) [12].
3 Spine 117

a b

Fig. 3.21 Lipomyelomeningocele in a 1-day-old male with a skin-­ skin that also contains neural elements (arrowheads). There is hyper-
covered soft tissue mass. Longitudinal (a) and transverse (b) grayscale echoic fatty tissue (white arrow) arising in the spinal canal that herni-
ultrasound images show a CFS-filled mass (black arrows) covered by ates through the osseous defect

a b

Fig. 3.22 Lipomyelocele in a 2-day-old female with a skin-covered into the subcutaneous tissues and abuts the neural placode (P). (b)
soft tissue mass. (a) Longitudinal grayscale ultrasound image demon- Sagittal T1-weighted, fat-suppressed MR image shows that the mass
strates an echogenic intradural soft tissue mass (arrows) that extends (arrows) contains fat and is covered by skin
118 M. Artunduaga et al.

Meningocele

Cerebrospinal
fluid
Spinal
cord

Incomplete
vertebra

Fig. 3.23 Diagram of meningocele. There is herniation limited to the thecal sac through a skin-covered posterior osseous defect

a b c

Fig. 3.24 Dorsal meningocele in a 3-month-old female with a subcu- skin at the thoracolumbar junction. (c) Sagittal balanced fast field echo
taneous mass of the mid back. Longitudinal (a) and transverse (b) gray- (bFFE) MR image in the sagittal plane shows the lesion (arrowheads)
scale ultrasound images show a CFS-filled mass (arrows) covered by and the presence of arachnoid strands (arrow) but no neural elements

On ultrasound, a tethered cord is characterized by an abnor-

mally low and eccentric position of the conus medullaris,
below the L2–L3 level, and there is frequently absence of nor-
mal nerve root pulsation on real-time imaging (Fig. 3.25) [3, 8,
12].
Prompt diagnosis and timely surgical release of the
filum terminale can prevent the loss of normal nerve root
­function [7, 12]. As affected pediatric patients continue to
grow, repeat surgical release may be needed as re-tethering
S4
can occur [12].
S3

Caudal Regression Syndrome S1 S2

Caudal regression syndrome is a spectrum of osseous under-

development that can affect the coccyx, sacrum, and lumbar Fig. 3.25 Tethered cord in a newborn female with a cutaneous heman-
gioma of the back. Longitudinal grayscale ultrasound image shows an
spine in a progressive fashion, extending from caudal to cra-
elongated conus medullaris (arrow) that terminates at the mid S2 level
3 Spine 119

Fig. 3.26 Caudal regression syndrome in a 2-day-old female. (a) lipoma (asterisk), focal kyphosis at the lumbosacral junction, and a pos-
Longitudinal grayscale ultrasound image shows underdevelopment of terior meningocele (M). (b) Sagittal T1-weighted MR image confirms
the sacrum with only two sacral vertebrae (arrows), a low-lying conus absence of the distal sacrum and coccyx, and presence of an intradural
medullaris terminating at L3–L4 associated with an intradural spinal lipoma (asterisk) and meningocele (M)

nial is due to disruption of caudal cell mass formation in
embryonic life [12]. Caudal regression syndrome frequently
occurs in its infants of diabetic mothers and can be associ-
ated with anal, genitourinary, vertebral, and limb anomalies
[1, 12, 17].
Ultrasound is useful in demonstrating additional associ-
ated spinal abnormalities, including an abrupt termination of
the conus medullaris or a tethered cord (Fig. 3.26) [17].
Multiple operations may be necessary to treat the many
abnormalities associated with caudal regression syndrome.
Anticholinergic drugs are often given to treat the associated
urological abnormalities.
lipomas can increase in size due to somatic growth,
although this growth is most significant during the first
year of life [12]. Spinal lipoma is associated with tethered
cord, spinal dysraphism, fatty filum, filar lipoma, and ver-
tebral anomalies [12].
On ultrasound, spinal lipoma appears as an echogenic
mass with absent or minimal vascularity, and it follows fat
signal on MR imaging (Fig. 3.27) [12]. Treatment consists
of either surgical resection or debulking, depending on the
extent of the lesion and associated abnormalities [12].
Segmental Spinal Dysgenesis

Spinal Lipoma Segmental spinal dysgenesis comprises a spectrum of ver-

tebral segmentation and fusion anomalies. Ultrasound may
Spinal lipoma is an intradural and/or extradural fat-­ be useful in the initial identification of a spinal cord abnor-
containing soft tissue mass that results from entrapment mality. However, cross-sectional imaging with CT or MR
of the mesenchyme due to premature embryologic separa- imaging will provide a more detailed assessment of verte-
tion of the neural and cutaneous ectoderm [12]. Spinal bral anatomy [1].
120
a b
L1 L2 L3
Fig. 3.27 Spinal lipoma in a 50-day-old female with a tuft of hair on
the lower back. (a) Longitudinal grayscale ultrasound image demon-
strates a well-defined, echogenic, intradural soft tissue mass (asterisk)
that is inseparable from the dorsal aspect of the conus medullaris and
I nfectious and Inflammatory Spinal
Disorders
In general, spine ultrasound is limited for the assessment of
infectious and inflammatory processes that involve the intra-
spinal structures although it is the modality of choice in the
evaluation of superficial abnormalities such as a pilonidal
sinus or cyst.
Epidural Abscess
Epidural abscess typically develops from hematogenous
spread of infection and is located dorsal to the thecal sac
[24]. The most common causative organism in spinal epi-
dural abscess is Staphylococcus aureus. The role of ultra-
sound in the detection and extension of epidural abscess is
limited, and MR imaging remains the diagnostic imaging
modality of choice [17]. Treatment is usually surgical
decompression followed by 4–6 weeks of antibiotic therapy.
M. Artunduaga et al.
L4 L5
filum terminale. Note the low-lying conus medullaris (arrow) at the
level of the superior endplate of L3. (b) Sagittal T1-weighted MR
image shows that the mass (asterisk) is of the same signal intensity as
the subcutaneous fat (F)
Pilonidal Sinus and Cyst
Pilonidal disease is a common suppurative condition of the
subcutaneous tissues of the sacrococcygeal region. Although
the underlying pathophysiology is uncertain, the most widely
accepted explanation is that a pit corresponding to a distended
and enlarged hair follicle enhanced by repeated episodes of
friction in the gluteal area serves as a nidus that elicits an
inflammatory response [25, 26].
Associated factors include obesity, trauma, hirsutism, poor
hygiene, hyperhidrosis, and family history. Pilonidal disease
clinically presents with drainage when infected [26].
Spine ultrasound typically shows an anechoic or hypoechoic
oval lesion located between the dermis and hypodermis that
may connect to the skin surface and may also contain an elon-
gated hyperechoic focus, corresponding to a hair fragment
(Fig. 3.28) [26]. Ultrasound is the imaging modality of choice
for the evaluation of pilonidal disease as it delineates the lesion
better than clinical inspection.
3 Spine 121

a b

Fig. 3.28 Pilonidal cyst in a 14-year-old female. Transverse (a) and lon- that almost reaches the skin surface. There is a hyperechoic structure
gitudinal (b) grayscale ultrasound images show a mildly echogenic fluid (arrows) within the fluid collection representing a hair fragment
collection (calipers) in the subcutaneous soft tissues of the sacral region

a b

Fig. 3.29 Diagram of intraspinal tumors. (a) Extramedullary lesion ullary tumor that expands the spinal cord and effaces the surrounding
located in the left posterolateral aspect of the spinal canal with antero- subarachnoid space
lateral displacement of the spinal cord. (b) Centrally located intramed-

While a small pilonidal sinus or cyst is treated with surgi-
cal excision and primary closure, a large lesion may require
open drainage with healing by secondary intention [26].
Neoplastic Spinal Disorders
The use of spine ultrasound in the detection and diagnosis of
intramedullary and extramedullary spinal tumors is limited
compared with MR imaging, which provides excellent ana-
tomical delineation and full characterization of tumor exten-
sion and imaging features (Fig. 3.29) [27]. Intraoperative
ultrasound assessment of spinal tumors can be used to confirm
the appropriate level of surgical exposure [6, 7]. Intraoperative
spine ultrasound can also guide catheter placement in the set-
ting of a syrinx, confirm adequate Chiari I malformation
decompression, visualize fenestration of an arachnoid cyst, and
guide resection of cavernous malformations.
122
a b
c d
Fig. 3.30 Intramedullary tumor in a 12-year-old male with a ganglio-
glioma. Intraoperative longitudinal grayscale ultrasound images (a), (b)
show expansion of the cervical spinal cord due to the presence of an
intramedullary lesion (arrows, calipers) that contains a centrally
Intramedullary Tumors
Intraoperative ultrasound evaluation of intramedullary tumors
typically shows a hyperechoic lesion with minimally ill-
defined margins that expand the spinal cord (Fig. 3.30) [6].
Intramedullary tumors often appear heterogeneous as they
may contain cystic, hemorrhagic, or calcified components.
Extramedullary Tumors
Intraoperative ultrasound evaluation of extramedullary tumors
can confirm cystic components of a lesion and further charac-
terize its internal contents to help narrow the differential diag-
nosis (e.g., between epidermoid/dermoid cyst and arachnoid
cyst) (Fig. 3.31) [6].
M. Artunduaga et al.
located, linear echogenic focus (arrowheads) in keeping with hemor-
rhage from recent biopsy. (c) Axial T2-weighted, fat-suppressed and
(d) Sagittal T1-weighted MR images depict the lesion (arrows) at the
level of C4–C5
Sacrococcygeal Teratoma
Sacrococcygeal teratoma arises from pluripotent cells
within the caudal cell mass and represents one of the most
common neonatal tumors [1, 9]. Females are more fre-
quently affected than males (3:1) [1]. Up to 70% of sacro-
coccygeal teratomas are diagnosed during the postnatal
period because they can present as palpable and/or visible
pelvic abnormalities [1, 9]. Classification of sacrococcy-
geal teratomas is based on the extension of the lesion
(intrapelvic versus extrapelvic) and is critical for preopera-
tive planning (Fig. 3.32) [9].
Imaging characteristics can help differentiate a benign
from a malignant teratoma, since cystic, fatty, and calcified
components occur more frequently in benign lesions, while
homogeneous solid components are more common in malig-
3 Spine 123

Fig. 3.31 Epidermoid/dermoid cyst in a 3-day-old female with a sacral ciated spinal cord tethering. Color Doppler imaging revealed no internal
dimple. Longitudinal grayscale ultrasound images (a), (b) show a rounded, flow (not shown)
hypoechoic extramedullary lesion (arrows) below the level of L5 with asso-

Type I Type II

Type III Type IV

Fig. 3.32 Diagram illustrating the four major types of sacrococcygeal nent. Type III: predominantly intrapelvic with an extrapelvic compo-
teratoma. Type I: predominantly extrapelvic with a minimal presacral nent. Type IV: completely intrapelvic
component. Type II: extrapelvic with a substantial intrapelvic compo-
124
a b
Fig. 3.33 Sacrococcygeal teratoma in a female neonate with a palpa-
ble left buttock mass. (a) Longitudinal grayscale ultrasound image of
the left buttock shows a mixed cystic and solid mass (calipers) that
Fig. 3.34 Epidural hematoma in a 1-day-old female with a recently
failed lumbar puncture attempt. Longitudinal color Doppler ultrasound
image shows an avascular, hypoechoic, lentiform fluid collection (arrow)
in the epidural space that abuts the thecal sac at the level of the conus and
proximal cauda equina
nant lesions (Fig. 3.33) [1]. Although neonatal ultrasound
serves as an initial imaging examination, MR imaging is nec-
essary for complete delineation of tumor extent and assess-
ment of treatment response [1].
Treatment of sacrococcygeal teratoma is complete surgi-
cal resection that should include the coccyx and may also
include portions of the sacrum.
Traumatic Spinal Disorders
Ultrasound of the spine is often performed after a traumatic
lumbar puncture. While spine ultrasound may serve as a
screening tool in clinically suspected cases of birth trauma,
MR imaging remains the best modality for assessment of
traumatic injuries.
M. Artunduaga et al.
arises from the coccyx. (b) Transverse grayscale ultrasound image
demonstrates a punctate calcification (arrow) within the solid compo-
nent of the mass
Lumbar Puncture
Ultrasound of the spine can aid in the diagnosis of complica-
tions following traumatic lumbar puncture by revealing the
presence of a fluid collection such as an epidural or subdural
hematoma (Fig. 3.34) [6, 9, 28]. Ultrasound is also helpful in
guiding lumbar puncture in clinically difficult cases or after
failed attempts, as it clearly demonstrates spinal anatomy
and allows targeting of the best location with the largest
accumulation of CSF [28–30].
Spinal Cord Injury
Ultrasound may be helpful in the initial assessment of spinal
cord injury related to traumatic delivery at birth, although
MR is the preferred imaging modality. Ultrasound may
reveal increased echogenicity of the spinal cord, reflecting
edema and/or hemorrhage [8, 11].
 pinal Cord Laceration and Transection
S
Spinal cord laceration and transection are devastating compli-
cations that can occur due to birth trauma [8, 11]. Predisposing
factors include the use of forceps and fetal malposition (typi-
cally a breech presentation) [8]. Management is supportive.
 pidural/Subdural Hematoma and Hematomyelia
E
Epidural/subdural hematoma and hematomyelia are other
complications that can result from birth trauma [8].
Predisposing factors are the same as for laceration and tran-
section of the spinal cord. Treatment includes surgical
decompression and high-dose corticosteroids.
3 Spine
Fig. 3.35 Syrinx in a 5-day-old male with a sacral dimple. Longitudinal
grayscale ultrasound image shows dilation of the central canal (arrow)
Hydromyelia/Syringomyelia/Syrinx
Hydromyelia is a dilation of the ependymal-lined central spi-
nal canal, while syringomyelia denotes a paracentral cavity
lined by gliotic parenchyma in which CSF has permeated as
a result of disruption of the ependymal lining [8]. Syrinx is a
general term that encompasses both abnormalities since the
distinction between hydromyelia and syringomyelia can
sometimes be challenging.
Symptom onset is typically in late adolescence or early
adulthood with sensory abnormalities, weakness, spastic-
ity, and scoliosis. Ultrasound of the spine will show a fluid-
filled lesion within the spinal cord (Fig. 3.35) [8]. Surgery
is usually recommended for symptomatic individuals.
There are two general forms of treatment: restoration of
normal CSF flow around the spinal cord and direct drainage
of the syrinx.
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 Neck
4
Patricia T. Acharya, Sharon R. Gordon, Mark C. Liszewski,
Ricardo Restrepo, and Edward Y. Lee

RI Resistive index
Abbreviations SCM Sternocleidomastoid
Tc Technetium
ACR American College of Radiology TDC Thyroglossal duct cyst
AVF Arteriovenous fistula TI-RADS Thyroid Imaging Reporting and Data System
AVM Arteriovenous malformation TSH Thyroid-stimulating hormone
BCCs Branchial cleft cysts
CM-AVM Capillary malformation–arteriovenous
malformation
CMV Cytomegalovirus Introduction
CT Computed tomography
FDG Fluorodeoxyglucose Ultrasound plays an important role in the evaluation of the
HIV Human immunodeficiency virus neck in children. Neck lesions found on physical examina-
I Iodine tion can be confirmed and characterized, including their pre-
MHz Megaherz cise anatomic location and effect on adjacent structures.
MIBG Metaiodobenzylguanidine Abnormalities not detectable by physical examination can
MPNST Malignant peripheral nerve sheath tumor often be demonstrated by ultrasound.
MR Magnetic resonance In this chapter, up-to-date ultrasound imaging techniques,
PHACES Posterior fossa malformations, hemangiomas, normal development and anatomy, and pathological pro-
arterial anomalies, coarctation of aorta and car- cesses of the neck that can occur in infants and children are
diac defects, eye abnormalities, sternal notching discussed.
PPD Purified protein derivative

P. T. Acharya (*) Technique

Department of Radiology, Children’s Hospital of Los Angeles,
Keck School of Medicine, University of Southern California, Patient Positioning
Los Angeles, CA, USA
e-mail: pacharya@chla.usc.edu
Neck ultrasound should be performed with the patient supine
S. R. Gordon
and the neck in hyperextension to optimize anterior exposure
Department of Radiology, Montefiore Medical Center and the
Albert Einstein College of Medicine, Bronx, NY, USA and permit evaluation of the neck from the mandible to the
thoracic inlet.
M. C. Liszewski
Division of Pediatric Radiology, Departments of Radiology and
Pediatrics, Montefiore Medical Center and the Albert Einstein
College of Medicine, Bronx, NY, USA Ultrasound Transducer Selection
R. Restrepo
Department of Interventional Radiology and Body Imaging, A high-frequency (9–3 MHz, 12–5 MHz, or 17–5 MHz) lin-
Nicklaus Children’s Hospital, Miami, FL, USA
ear transducer provides optimal spatial resolution for neck
E. Y. Lee imaging. There is a trade-off between resolution and beam
Division of Thoracic Imaging, Department of Radiology,
penetration with higher-frequency transducers providing
Boston Children’s Hospital and Harvard Medical School,
Boston, MA, USA improved resolution of the superficial tissue layers compared

© Springer Nature Switzerland AG 2021 127

H. J. Paltiel, E. Y. Lee (eds.), Pediatric Ultrasound, https://doi.org/10.1007/978-3-030-56802-3_4
128
to lower-frequency transducers but with limited penetration
of the deeper soft tissues. The ultrasound transducer should
therefore be carefully selected to operate at the highest clini-
cally appropriate frequency. For large neck lesions, a curvi-
linear transducer may be helpful by providing a wider field
of view.
Imaging Approaches
Ultrasound images of the neck are typically acquired in trans-
verse and sagittal planes. Color and spectral Doppler imaging
are important for evaluation to characterize lesion vascularity
and flow hemodynamics. Cine ultrasound ­imaging is useful
in demonstrating lesion compressibility, to assess the extent
of a lesion larger than the transducer field of view, and to
compare the lesion to the surrounding soft tissues. Comparison
views with dual-phase imaging of the opposite side are also
helpful in detecting asymmetry. Additional ultrasound images
obtained while the patient is swallowing can aid in the detec-
tion of lesions in the upper and lower poles of the thyroid
gland.
Normal Development and Anatomy
Neck
The neck is anatomically delineated by the mylohyoid mus-
cle superiorly and the first rib inferiorly. By convention, the
Anterior midline of neck
Axillary inlet
Superior thoracic aperture
Fig. 4.1 Diagram of the anterior and posterior triangles of the neck
P. T. Acharya et al.
sternocleidomastoid (SCM) muscle is the landmark that
divides the anterior and posterior triangles of the neck, with
structures anterior to the SCM muscle in the anterior triangle
and those posterior to the SCM muscle in the posterior tri-
angle (Fig. 4.1).
Anterior Triangle
The right and left anterior triangles are formed by the body
of the mandible superiorly, the midline of the neck medially,
and the SCM muscle posteriorly. The hyoid bone divides
each anterior triangle into suprahyoid and infrahyoid por-
tions. The digastric muscle further subdivides the suprahyoid
portion into submental and submandibular triangles.
The midline submental triangle is unpaired, flanked by the
anterior belly of the digastric muscle and the hyoid bone, and
contains small lymph nodes and branches of the facial artery
and vein. The anterior and posterior bellies of the digastric mus-
cles and the mandibular rami form the submandibular triangle,
which contains lymph nodes and the submandibular gland.
The trachea, esophagus, thyroid and parathyroid glands,
vagus nerve, carotid artery, and internal jugular vein are located
in the infrahyoid portion of the anterior cervical triangle.
Posterior Triangle
The posterior triangle is formed by the sternocleidomastoid
muscle anteriorly, the trapezius muscle posteriorly, the deep
cervical fascia superiorly, and the clavicle inferiorly. Lymph
nodes, the spinal accessory nerve, portions of the subclavian
artery and vein, and of the brachial plexus and phrenic nerve
are located in the posterior triangle.
Sternocleidomastoid muscle
Anterior triangle
Trapezius muscle
Posterior triangle
4 Neck 129

Lymph Nodes
In the neck, lymph nodes are divided into superficial (parotid,
submandibular, facial, and occipital) and deep (sublingual,
retropharyngeal, and lateral cervical) groups (Fig. 4.2). The
lateral cervical lymph nodes can be further subdivided into
superficial (external jugular) and deep (internal jugular, spi-
nal accessory, and transverse cervical) groups. The internal
jugular chain lymph nodes are the largest in the upper neck,
with the jugulodigastric node being the largest node in this
chain. They drain the nasopharynx, oropharynx, tonsils,
hypopharynx, and larynx, and are the most commonly
involved in disease processes.
On ultrasound, unenlarged, benign lymph nodes can be
seen as ovoid, hypoechoic structures with an echogenic lin-
ear hilum that is vascular on color Doppler imaging (Fig. 4.3).

Retropharyngeal node

Infrahyoid node

Prelaryngeal nodes

Paratracheal nodes

Fig. 4.2 Diagram of the normal lymph nodes of the neck

a b

Fig. 4.3 Normal cervical lymph node in a 15-year-old male. (a) Longitudinal grayscale ultrasound image shows a normal left cervical lymph node
(arrow) with a fatty hilum (arrowhead). (b) Longitudinal color Doppler ultrasound image shows normal hilar blood flow (arrow)
130 P. T. Acharya et al.

Thyroid Gland The thyroid gland is divided into two lobes located on
either side of the trachea (Fig. 4.5). The isthmus of the thyroid
The thyroid gland develops in the first trimester of pregnancy gland connects the lobes and crosses anterior to the trachea. A
near the base of the tongue and later descends caudally into pyramidal lobe is sometimes present extending superiorly
the neck to its final position in front of the larynx at the level from the isthmus or from the medial right or left lobe.
of C5–C7, initially preserving its connection to the tongue Anteriorly, the thyroid is bordered by the thin strap muscles
via the thyroglossal duct (Fig. 4.4). Later, in normal develop- (sternohyoid, sternothyroid, and omohyoid), laterally by the
ment, the thyroglossal duct involutes and disappears. sternocleidomastoid muscles, and posteriorly by the longus
colli muscles along the lateral aspects of the anterior verte-
brae. The common carotid arteries and internal jugular veins
Foramen cecum
are located posterolateral to each thyroid lobe. Medially, the
Parathyroid III
Thyroglossal duct trachea can be seen on the right and the esophagus on the left.
The ultrasound appearance of a normal thyroid gland is
Third branchial
pouch
Median thyroid homogeneous and hyperechoic when compared to the adjacent
muscles (Fig. 4.6), with scattered, readily detectable internal
blood flow seen with color or power Doppler, roughly similar
Parathyroid IV

Fourth
branchial
pouch

Fifth branchial
pouch

Ultimobranchial Thymus
body

Trachea

Esophagus

Fig. 4.4 Diagram of the embryological development of the thyroid and
parathyroid glands. The thyroid gland arises from the pharyngeal floor at
the foramen cecum. It later descends into the neck along the thyroglossal
duct. The parathyroid glands develop from the cranial portions of the
third and fourth pharyngeal pouches in association with the thymus
Fig. 4.6 Normal thyroid gland in a 6-year-old male. Transverse gray-
scale ultrasound image demonstrates the right (R) and left (L) thyroid
lobes joined by the isthmus (I). The thyroid gland is draped anteriorly
over the trachea (T)

b Isthmus

Right Left
lobe lobe

Larynx
Thyroid gland (anterior view)

c Parathyroid
glands
Thyroid gland

Trachea

Thyroid gland (posterior view)

Fig. 4.5 Diagram of the normal thyroid gland

4 Neck
to that seen in other superficial solid organs (e.g., the testes).
The lobes are triangular on transverse images and elliptical on
sagittal images. The size of the thyroid gland varies with age.
Parathyroid Glands
The parathyroid glands are paired endocrine glands, the
superior glands arising from the fourth branchial pouch and
the inferior glands from the third branchial pouch (Fig. 4.4).
The superior glands are usually located posterior to the mid
portion of the thyroid, while the inferior glands are slightly
more variable in location (Fig. 4.7). As with the thyroid
gland, the parathyroid glands can be ectopic, although this is
rare. Occasionally, supernumerary glands can occur that are
often associated with the thymus.
Median thyroid
Lateral thyroid lobe
Parathyroid
Thymus
Esophagus
Fig. 4.7 Diagram showing the relationship of the parathyroid glands to
the thyroid gland
a b
T
Fig. 4.8 Normal parathyroid gland in an 18-year-old male with a goi-
ter. Longitudinal (a) and transverse (b) grayscale ultrasound images
demonstrate an oval nodule (arrows) posterior to the thyroid gland (T).
131
Normal parathyroid glands are oval in shape (Fig. 4.8) and are
usually difficult to differentiate from the adjacent thyroid tissue
because of their similar echotexture. They are not usually visual-
ized by ultrasound due to their small size (<5 mm in length).
Salivary Glands
Parotid Glands
The paired parotid glands are the largest salivary glands
(Fig. 4.9). Each gland is bordered anteriorly by the ramus of
the mandible and masseter muscle and posteriorly by the
mastoid process and SCM muscle. The facial nerve, which
travels within the parotid gland, divides it into superficial
(lateral) and deep (medial) lobes. However, as the facial
nerve is typically poorly delineated on ultrasound, the retro-
mandibular vein, which lies deep to the nerve and lateral to
the external carotid artery, is used as the ultrasound landmark
to separate the superficial and deep lobes.
The superficial lobe is the larger portion of the parotid,
accounting for the majority of the gland. In 20% of patients, a
separate accessory parotid gland can be seen, lying superfi-
cial to the masseter muscle and anterior to the main parotid
gland [1]. Acini in the parotid gland drain through the Stensen
duct, found along the anterior border of the parotid gland
below the zygomatic arch and exiting above the upper second
molar tooth.
On ultrasound, the parotid gland is generally homoge-
neous and hyperechoic relative to the adjacent masseter mus-
cle due to intraglandular fat. However, in very young
children, its echogenicity can be similar to that of the muscle.
The parotid gland appears round or ovoid on transverse
views and elliptical on longitudinal images. Small
hypoechoic, ovoid intraglandular lymph nodes may occa-
sionally be seen (Fig. 4.10). A normal caliber Stensen duct is
typically not visible with ultrasound.
T
The contour of the thyroid gland is mildly lobulated and the paren-
chyma is somewhat heterogeneous. Asterisk, Longus colli muscle
132 P. T. Acharya et al.

Parotid duct

Parotid
gland

Sublingual
ducts

Sublingual
gland Parotid
gland

Submandibular
duct
Submandibular
gland

Fig. 4.9 Diagram of the salivary glands

a b

Fig. 4.10 Normal parotid gland in a 17-year-old female. Longitudinal grayscale ultrasound image (a) and axial T2-FLAIR magnetic resonance
(MR) image (b) of the right parotid gland containing intraparenchymal lymph nodes (arrowheads), a normal finding

Submandibular Glands On ultrasound, the submandibular glands should be

The paired submandibular glands lie within the posterior
submandibular space and are bordered by the body of the
mandible laterally and the mylohyoid muscle superiorly and
medially (Fig. 4.9). A small portion of the gland may lie pos-
terior to the mylohyoid muscle within the sublingual space.
The facial artery courses laterally, and the facial vein runs
along the anterosuperior aspect of the gland. The Wharton
duct drains the gland, extending along the mylohyoid muscle
laterally, and the hypoglossus muscle medially, terminating
at an orifice lateral to the frenulum of the tongue.
examined in two perpendicular planes with the transducer in
a submental position. The glands are triangular, homoge-
neous, and moderately echogenic, greater than muscle but
less echogenic than the parotid glands (Fig. 4.11) [2]. Fine
intraparenchymal linear streaks can be seen, representing
intraglandular ductules. The Wharton duct is normally too
small to be seen unless saliva production is stimulated.
Movement of the patient’s tongue may aid in visualization
of the duct [3].
4 Neck
Fig. 4.11 Normal submandibular gland in an 11-year-old male.
Longitudinal grayscale longitudinal ultrasound image of the right sub-
mandibular gland (arrow) demonstrates homogeneous, moderately echo-
genic parenchyma
Sublingual Glands
The paired sublingual glands are the smallest of the major
salivary glands, lying below the floor of the mouth on the
mylohyoid muscle between the mandible and genioglossus
muscle (Fig. 4.9). The excretory submandibular duct and
sublingual vessels lie medial to the gland. The lateral border
of the sublingual gland is adjacent to the mandible.
Ultrasound evaluation of the sublingual glands should
be performed in two perpendicular planes with the trans-
ducer placed on the submental portion of the mandible. On
transverse ultrasound imaging, the gland is oval, and lenti-
form in shape on longitudinal sections obtained parallel to
the mandibular body. The echogenicity of the sublingual
gland is similar to that of the parotid and submandibular
glands [4], and branches of the lingual artery and vein may
be seen within the gland on color Doppler imaging [5].
Neck
Congenital Neck Anomalies
Congenital Nonvascular Neck Masses
Congenital Benign Cystic Neck Masses
Thyroglossal Duct Cyst
Thyroglossal duct cyst (TDC) is the most common ­congenital
pediatric neck mass and results from failure of the normal
developmental involution of the thyroglossal duct. It can
occur anywhere along the course of the duct, from the fora-
men cecum at the base of the tongue to the pyramidal lobe of
the thyroid gland, with the most common location at the level
of the hyoid bone [6, 7].
A child with a TDC at the suprahyoid or hyoid level typi-
cally presents with an anterior midline cervical mass, while
133
Fig. 4.12 Thyroglossal duct cyst in a 5-month-old male with stridor
and a palpable neck mass. Transverse grayscale ultrasound image
shows a midline cystic structure (arrow) anterior to the trachea (aster-
isk) at the level of the thyroid gland (T). The cyst demonstrates poste-
rior acoustic enhancement and contains echogenic, layering debris
an infrahyoid TDC may have a median or paramedian loca-
tion and can occur within the strap muscles of the neck. A
TDC typically presents during childhood in the first decade
of life [7] as a gradually enlarging, painless, fluctuant cervi-
cal mass, or may be asymptomatic until it becomes infected
and manifests as a painful neck mass.
Ultrasound evaluation of TDC typically demonstrates a
well-circumscribed hypo- or anechoic cystic structure with
posterior acoustic enhancement and possible internal com-
plexity, such as septa and debris or internal echoes from pro-
teinaceous material, even in the absence of infection
(Fig. 4.12). Ultrasound findings of an infected TDC include
a thickened and irregular cyst wall with increased peripheral
vascularity (Fig. 4.13). An associated soft tissue mass can
represent ectopic thyroid tissue or, rarely, a carcinoma, with
papillary thyroid carcinoma being the most common at his-
topathologic examination [8].
Elective resection is recommended because of the poten-
tial for malignant transformation as well as superimposed
infection. It is essential to confirm the presence of a normally
located thyroid gland before surgical removal, as ectopic
thyroid tissue within the TDC may be the only functional
thyroid tissue in the body.
Branchial Cleft Cyst
During embryological development, the branchial apparatus
appears by the fourth week of gestation in the region of the
pharyngeal foregut, between the developing brain and heart.
The apparatus consists of six pairs of arches, clefts, and
pouches. Four mesodermal arches are lined on either side by
four ectodermal clefts externally and four endodermal
134 P. T. Acharya et al.

Fig. 4.13 Infected thyroglossal duct cyst in a 7-year-old female with an is edema (arrow) of the adjacent subcutaneous soft tissues. (b) Longitudinal
enlarging neck mass. (a) Longitudinal grayscale ultrasound image shows a color Doppler ultrasound image shows hyperemia at the cyst periphery
thick-walled cystic structure (asterisk) containing echogenic debris. There (arrowheads), as well as hyperemia of the overlying soft tissues

pouches internally. The fifth and sixth arches are too small to
be visualized macroscopically.
The second arch enlarges and surrounds the second through
fourth branchial clefts as it grows caudally toward the fifth
pharyngeal arch, leaving a small sinus opening called the cer-
vical sinus of His [9], which later obliterates. The first bran-
chial cleft persists into adulthood, and the remaining branchial
clefts obliterate during development along with the cervical
sinus [8, 9]. However, if there is a failure of obliteration, bran-
chial cleft anomalies can occur, including branchial cleft cysts
(BCCs), branchial fistulas, and branchial sinus tracts.
BCCs occur when the embryologic remnant of the cervi-
cal sinus or the branchial clefts fail to obliterate [8, 9].
Branchial sinuses are incomplete tracts with a single internal
Fig. 4.14 Branchial cleft cyst in a 3-year-old male with induration
opening. Branchial fistulas communicate both externally and near the parotid gland and cervical lymphadenopathy. Longitudinal
internally with multiple openings due to failure of both the grayscale ultrasound image shows an ovoid cyst containing a small
branchial cleft and branchial pouch to involute. Unlike a amount of internal, layering debris (asterisk) and displaying posterior
branchial cleft fistula or branchial sinus, BCCs are self-­ acoustic enhancement (arrow)
contained, without communication with the skin or internal
structures such as the pharynx. for an associated branchial cleft sinus or branchial fistula should
Second BCCs comprise the majority of branchial anomalies be performed during BCC imaging, as these tracts will need to
[10], and can present as a painless, fluctuant lateral neck mass, be resected to prevent cyst recurrence [12, 13].
usually in childhood or late adolescence. However, if the BCC
becomes superinfected, it may manifest as a painful mass. Cervical Thymic Cyst
A BCC typically presents on ultrasound as a well-­ A cervical thymic cyst is rare, with the majority detected inci-
circumscribed, round or oval, hypo- or anechoic cystic dentally in the first decade of life [14]. It can occur anywhere
structure with posterior acoustic enhancement (Fig. 4.14). It is along the path of the thymopharyngeal duct, from the angle of
commonly located in the anterior triangle of the neck, anterior the mandible to the thoracic inlet, immediately adjacent to the
to the SCM muscle and anterolateral to the great vessels of the carotid sheath. Affected children are usually asymptomatic
neck. It may adhere to the internal jugular vein or may be located with a slowly enlarging, painless lateral neck mass located
between the internal and external carotid arteries [6, 9, 11]. With near the thoracic inlet, either anterior or deep to the SCM mus-
superimposed infection, a complex cyst and peripheral hyper- cle. Newborns may present with dysphagia, stridor, and respi-
vascularity may be seen with ultrasound (Fig. 4.15). Evaluation ratory distress, although this presentation is less common [8].
4 Neck
a b
Fig. 4.15 Infected branchial cleft cyst in a 1-year-old female with an
enlarging fluctuant neck mass. (a) Longitudinal grayscale ultrasound
image shows a complex cystic structure with a thick, irregular wall
Fig. 4.16 Thymic cyst in an 8-year-old female. (a) Transverse grayscale
ultrasound image reveals a multiloculated, cystic mass (arrowheads) in
the right neck, elevating and medially displacing the right thyroid lobe
(T). There is a fluid-fluid level (arrow) in one of the cystic spaces. (b)
Longitudinal color Doppler ultrasound image documents the avascular
On ultrasound, a thymic cyst often appears as a large, cystic
mass extending caudally, parallel to the SCM muscle. CT
characteristically demonstrates a uniloculated or multilocu-
lated, hypoattenuating cystic mass adjacent to the carotid
space that can sometimes extend to the mediastinum. On mag-
135
(arrows) containing echogenic debris (asterisk). (b) Transverse power
Doppler ultrasound image shows two hypoechoic cystic foci (asterisks)
with surrounding hyperemia (arrowheads) indicative of inflammation
nature of the lesion. THY, Right thyroid lobe. Cervical thymic tissue
(arrow) is noted inferior to the lesion. (c) Coronal inversion recovery MR
image shows the hyperintense fluid-containing septated cyst (arrow) in
the right neck that abuts a cervical extension (arrowhead) of the thymus
(asterisk)
netic resonance (MR) imaging, it is typically hypointense on
T1-weighted images and hyperintense on T2-weighted images
(Fig. 4.16).
Treatment involves surgical resection, including any con-
nection to the mediastinal thymic tissue, either by direct
136
extension or via a fibrous cord (a remnant of the thymopha-
ryngeal duct) as well as any fibrous connection between the
thymic cyst and the thyroid gland [8].
Duplication Cyst
A foregut duplication cyst occasionally occurs in the lower
neck. Affected pediatric patients may present with stridor from
compression of the trachea by a bronchogenic cyst [15]. On
ultrasound, it usually appears as a thin-walled, anechoic or
hypoechoic cyst. When it contains hemorrhagic, proteinaceous,
or purulent material, it can demonstrate a thick wall with inter-
nal debris and fluid–fluid levels (Fig. 4.17). Foregut duplication
cysts can usually be successfully managed surgically.
a b
Fig. 4.17 Foregut duplication cyst of the floor of the mouth and sub-
mandibular region in a 4-month-old male. Transverse (a) and longitudi-
nal (b) grayscale ultrasound images of the submandibular region reveal
a bilobed (asterisks) midline cystic collection containing a moderate to
P. T. Acharya et al.
Teratoma and Dermoid Cyst
A teratoma is a germ cell tumor arising from ectopic pluripotent
stem cells of two or three germ cell layers that fail to migrate
from the yolk sac endoderm to the gonadal ridge [16]. Up to
90% of childhood teratomas contain all three germ cell layers
with variable differentiation, and 75–85% of head and neck
tumors contain neuroectodermal tissue [17]. A dermoid cyst is a
benign lesion that develops when there is inclusion of ectoder-
mal tissue during the fusion of the branchial arches and is lined
only by mature ectodermal tissue of the skin and adjacent tis-
sues (sudoriferous glands, hair, and sebaceous glands) [17].
Cervical teratomas are rare, and most are benign [17–19].
They typically present as large, bulky, cystic, and solid masses
large amount of echogenic debris. A suggestion of wall gut signature
(arrow) is noted on the transverse image. (c) Sagittal contrast-­enhanced
computed tomography (CT) image shows the two cystic components
(arrows) of the lesion
4 Neck
at or near the midline, in the anterior suprahyoid portion of
the neck, and adjacent to or within a thyroid lobe [18, 20].
Symptoms from airway impingement commonly occur with
large lesions. Ultrasound typically demonstrates a multilocu-
lar, heterogeneous mass with solid and cystic components
and echogenic foci representing fat or calcification (Fig. 4.18).
On Doppler imaging, cervical teratomas are ­relatively avas-
cular. Mortality is high if large lesions are not resected.
A cervical dermoid cyst usually presents as a painless,
slow-growing para-midline suprahyoid mass. It can occur
a b
Fig. 4.18 Mature teratoma in a 1-day-old male who presented
with a left-sided neck mass. (a) A longitudinal grayscale ultrasound
image shows a heterogeneous lesion containing both echogenic com-
ponents and innumerable tiny anechoic cystic spaces. (b) Sagittal
Fig. 4.19 Dermoid cyst in an 8-year-old female with a palpable supra-
sternal neck mass. (a) Longitudinal grayscale ultrasound image shows
a well-circumscribed cystic lesion with echogenic contents and poste-
rior acoustic enhancement (arrowhead). (b) Transverse color Doppler
137
in the floor of the mouth or less often in the thyroidal or
suprasternal area [21]. Ultrasound typically demonstrates
a well-circumscribed, thin-walled, unilocular mass that is
homogeneous or heterogeneous depending on cyst con-
tent. Internal echoes may be seen, reflecting the presence
of keratinous or sebaceous debris, and mural calcifica-
tions can be present (Fig. 4.19). As these lesions are at
risk for rupture or infection, and rarely (5%) may undergo
malignant degeneration to a squamous cell neoplasm, sur-
gical resection is recommended [22].
contrast-enhanced, T1-weighted, fat­-suppressed MR image shows a
complex mass (arrow) in the left submandibular space with minimal
enhancement of the solid components
ultrasound image reveals the avascular nature of the lesion. (c) Axial
T1-weighted, fat-suppressed MR image reveals no enhancement of the
lesion (asterisk)
138
Congenital Benign Solid Neck Masses
Ectopic Thymus
The embryonic thymus arises from the third and fourth pha-
ryngeal pouches in the neck and later migrates to its final
superior mediastinal location. Failure of descent, or failure of
involution during embryonic life can result in an ectopic thy-
mus located anywhere along the normal path of descent from
the angle of the mandible to the thyroid gland [23–25].
Affected children typically present with an asymptomatic
midline or lateral neck mass. Ectopic thymus can be differen-
tiated from other masses on ultrasound by the appearance of
the parenchyma which is the same as that of the normal medi-
astinal thymus, pliability, and lack of mass effect (Fig. 4.20).
Cervical Extension of Normal Thymus
Superior cervical extension of the normal mediastinal thymus
can be seen in nearly two-thirds of children and young adults
[26]. It is important to be familiar with this normal variant in
Fig. 4.20 Ectopic thymic tissue in a 7-year-old male with a palpable cervical
“mass.” Longitudinal grayscale ultrasound image at the site of the lump shows
normal thymic tissue (arrow) located inferior to the right thyroid lobe (T)
Fig. 4.21 Prominent but normal cervical thymic tissue in a 6-year-old
male. Transverse grayscale ultrasound image of the base of the left neck
demonstrates normal thymic tissue (arrow) with a characteristic “starry
sky” appearance
P. T. Acharya et al.
order to distinguish thymic tissue from a pathologic mass. On
ultrasound, cervical extension of the normal thymus appears
in continuity with the normal mediastinal thymus and has an
identical parenchymal appearance (Fig. 4.21).
Fibromatosis Colli
Fibromatosis colli is fusiform enlargement of the SCM mus-
cle caused by benign fibroblastic proliferation that usually
occurs within the first few weeks of life. Although the etiol-
ogy is unknown, it may represent a scar-­like reaction to
injury of the SCM muscle in the last trimester of pregnancy
or during delivery. Clinical presentation in neonates and
young infants is usually with a hard, growing neck mass and
torticollis. There is often a history of birth trauma, such as
forceps delivery, or breech presentation [6, 27]. More com-
monly found on the right side, fibromatosis colli may con-
tinue to enlarge for weeks after birth [27, 28].
Ultrasound is the imaging modality of choice in the evalu-
ation of fibromatosis colli and demonstrates either focal (most
often in the lower two-thirds of the muscle) or diffuse enlarge-
ment of the SCM muscle. There is fusiform thickening and
shortening of the SCM muscle (Fig. 4.22), which leads to tilt-
ing and rotation of the head toward the side of the lesion. At
ultrasound, the affected portions of the muscle are well defined
and of variable echogenicity, often with a hypoechoic rim. The
mass moves synchronously with the remainder of the SCM
muscle on real-time imaging. Hyperechoic, calcific foci with
distal shadowing can occasionally be seen, and are thought to
represent sequelae of prior hemorrhage.
Fibromatosis colli is typically a self-limiting condition,
resolving within 4–8 months with conservative management
and physical therapy [6].
Congenital Vascular Neck Masses
Hemangiomas
Hemangiomas are benign neoplasms composed of ­vascular
channels lined by endothelial cells, and are the most common
head and neck tumors of infancy [11, 29]. Congenital heman-
giomas are fully grown at birth. Infantile hemangiomas are
characteristically small or absent at birth, and typically prolif-
erate and enlarge rapidly during the first year of life with grad-
ual involution during early childhood [11, 29]. Cutaneous
hemangioma can present as a blanching skin lesion with areas
of fine telangiectasia [30]. A deep or subcutaneous hemangi-
oma may have a more bluish hue. If one or multiple infantile
hemangiomas are present in a trigeminal dermatome distribu-
tion (frequently V1), PHACES syndrome (Posterior fossa mal-
formations, Hemangiomas, Arterial anomalies, Coarctation of
aorta and cardiac defects, Eye abnormalities, and Sternal
notching) should be considered.
Ultrasound evaluation of a hemangioma demonstrates dis-
crete cutaneous or subcutaneous soft tissue masses with
prominent internal vascularity (Fig. 4.23). Low-resistance
4 Neck 139

a b

Fig. 4.22 Fibromatosis colli in a 7-week-old female with torticollis and a palpable neck mass. Transverse (a) and longitudinal (b) grayscale
ultrasound images of the left neck show enlargement and heterogeneity of the sternocleidomastoid muscle (asterisk)

a b

c d

Fig. 4.23 Infantile hemangioma in an 8-month-old female with a left-
sided neck mass and overlying soft tissue discoloration. (a) Transverse
grayscale ultrasound image shows a well-circumscribed, hypoechoic
mass with mildly lobulated margins in the subcutaneous soft tissues. (b)
Transverse color Doppler ultrasound image shows multiple enlarged ves-
sels within the lesion. (c) Transverse color Doppler ultrasound image
with spectral analysis of the lesion shows low-resistance arterial flow
(arrowhead) above the baseline and venous flow (arrow) below the base-
line. (d) Axial T2-weighted MR image shows a homogeneous left-sided
neck mass containing numerous vascular flow voids (arrowheads)
140 P. T. Acharya et al.

arterial blood flow and venous flow can be seen within the Arteriovenous Fistula
mass [29]. MR imaging may be necessary for further charac- An arteriovenous fistula (AVF) is a direct communication
terization, where hemangioma typically appears isointense between an artery and vein without an intervening capil-
relative to muscle on T1-weighted images and hyperintense lary bed. There is no vascular nidus as occurs with an
on T2-weighted images, with diffuse contrast enhancement AVM. AVFs are often acquired following trauma, but like
from high-flow central and peripheral vessels. AVMs, they can also occur sporadically, in the setting of
Most infantile hemangiomas are managed conservatively. hereditary hemorrhagic telangiectasia or capillary malfor-
However, complications such as bleeding, ulceration, or mation–arteriovenous malformation (CM-AVM) syn-
impingement on the airway or orbits may require treatment drome [33].
[30]. Treatment options generally include administration of On ultrasound, the fistula may be seen as an abnormal,
antiangiogenesis medications such as propranolol and corti- high-velocity, direct connection between an artery and vein
costeroids, chemotherapy, α-interferon therapy, ­embolization, (Fig. 4.25). Venous enlargement also occurs as a result of
sclerotherapy, radiotherapy, cryotherapy, laser therapy, or the increased volume of flow. Spectral Doppler ultrasound
surgical excision [30–32]. reveals increased arterial diastolic flow and arterialized
venous flow.
Vascular Malformations
Vascular malformations, in contrast to hemangiomas, are con- Venous Malformation
genital anomalies that are present at birth and enlarge as the A venous malformation consists of dysplastic venous chan-
child grows. They do not involute, increasing in size in propor- nels that may manifest as blue or purple skin discoloration or
tion to surrounding structures, and are present throughout life. as a compressible mass that enlarges with the Valsalva
Vascular malformations are categorized into lymphatic, capil- maneuver [11]. On ultrasound, it can appear as a collection
lary, arteriovenous, venous, or mixed types and are divided into of compressible vascular channels with low-velocity venous
low-flow and high-flow lesions [30]. Low-flow lesions include blood flow (Fig. 4.26). Phleboliths are often present within
venous malformations, lymphatic malformations, or mixed the lesion. Treatment includes compressive garments, sclero-
venolymphatic lesions, while high-­flow lesions consist of arte- therapy, or surgery [30].
riovenous malformations and arteriovenous fistulas [30].
Grayscale ultrasound with color and spectral Doppler Lymphatic Malformation
offers an excellent, noninvasive means of assessing these Lymphatic malformation is the second most common type of
lesions and the adjacent soft tissues. Vessel size and flow char-
vascular malformation after venous malformation and most
acteristics aid in determining the nature of the lesion, which in
often affect the head and neck region, especially the posterior
turn can guide any further investigation and therapy. cervical triangle [34, 35]. It is composed of chyle-filled cysts
lined with endothelium and results from sequestered lym-
Arteriovenous Malformation phatic sacs that fail to communicate with peripheral draining
Arteriovenous malformation (AVM) is a high-flow lesion channels [35].
with direct arteriovenous communication and no intervening Lymphatic malformations can be classified into three
capillary bed, resulting from disturbances in angiogenesis types: macrocystic, microcystic, or combined. Macrocystic
[30]. Typically present at birth and indolent in childhood, an lesions contain cysts 1–2 cm in diameter, microcystic lesions
AVM typically grows at puberty and may be stimulated to contain smaller cysts, and combined lesions contain a mix-
further growth after trauma, hormonal changes, infection or ture of micro- and macrocysts surrounded by a connective
spontaneous hemorrhage [29]. tissue stroma [36–38]. While most lymphatic malformations
At ultrasound, an AVM is characterized by a tangle of tor- are sporadic, they can also appear as a component of several
tuous blood vessels, the nidus, where the feeding arteries are syndromes, including Noonan syndrome, Turner syndrome,
directly connected to a venous drainage network without the and trisomies 13, 18, and 21 [11]. Clinically, lymphatic mal-
interposition of a capillary bed. There is no associated soft-­ formations usually present in the first 2 years of life as
tissue mass (Fig. 4.24). Color Doppler ultrasound reveals smooth, soft, nontender masses with a rubbery or doughy
arteriovenous shunting with high-velocity, low-resistance consistency [11, 37].
arterial waveforms, and arterialized venous flow. Treatment On ultrasound, lymphatic malformations can appear as
generally involves endovascular therapy with transarterial multilocular cystic masses containing internal septa of vary-
embolization [30]. ing thickness, and, unlike venous malformations, they are
4 Neck
a b
c d
e f
Fig. 4.24 Arteriovenous malformation in a 9-year-old girl with purple
discoloration of the left upper neck. (a) Longitudinal grayscale ultra-
sound image reveals the presence of a hypoechoic soft tissue lesion
(arrow) in the subcutaneous tissues of the left neck containing multiple
anechoic, tubular structures (arrowheads). (b) Longitudinal color
Doppler ultrasound image shows that the lesion consists of innumera-
141
ble small vascular channels. Longitudinal color Doppler ultrasound
images with spectral analysis demonstrate arterial (c) and venous (d)
flow within the mass. The venous spectral waveform is mildly pulsatile,
in keeping with arteriovenous shunting. Lateral angiographic images
show (e) multiple intralesional vascular channels with (f) early venous
drainage (arrow)
142
a b
V
c d
e f
Fig. 4.25 Vertebro-epidural spinal arteriovenous fistula in a 3-year-
old male. Transverse (a) and longitudinal (b) grayscale ultrasound
images of the left neck at the level of C6-C7 show a direct connection
(arrowheads) between the left vertebral artery (A) and an enlarged,
tortuous epidural vein (V). (c) Transverse color Doppler ultrasound
image shows prominent flow (arrow) in the dilated epidural vein. (d)
P. T. Acharya et al.
V A
Spectral Doppler sampling of the vertebral artery shows high-velocity
flow. (e) Spectral Doppler sampling of the epidural vein reveals arterial-
ized flow in keeping with arteriovenous shunting. (f) Frontal angio-
graphic image shows the abnormal connection (white arrowhead)
between the ­vertebral artery and a cluster of prominent veins (arrow)
with rapid drainage into dilated epidural veins (black arrowheads)
4 Neck
a b
c d
Fig. 4.26 Venous malformation of the face, floor of mouth, and neck in a
5-month-old male. (a) Clinical photograph shows enlargement and bluish
discoloration of the tongue. There is a blue skin stain along the left man-
dibular region and the left side of the neck is enlarged. (b) Transverse gray-
scale ultrasound image of the left neck demonstrates multiple anechoic
tubular structures (arrowheads) in the soft tissues in keeping with enlarged
143
vessels. (c) Transverse color Doppler ultrasound image reveals promi-
nent flow within the enlarged vessels. (d) Color and spectral Doppler
ultrasound evaluation of the enlarged vessels demonstrates venous wave-
forms. (e) Frontal angiographic image obtained during sclerotherapy
depicts contrast material filling multiple enlarged, communicating vas-
cular spaces in the left face and neck
144 P. T. Acharya et al.

noncompressible (Fig. 4.27). When large, lymphatic malfor-
mations can occupy more than one space in the neck and
insinuate between normal structures, causing compression
and resulting in respiratory difficulty or dysphagia [11].
Additional cross-sectional imaging with computed tomogra-
phy (CT) or MR imaging is useful to evaluate for deeper
extension into the neck or chest.
Complications such as hemorrhage or infection may
occur, causing enlargement of these lesions. In this setting,
surgery is the preferred method of treatment, although com-
plete excision may not be possible. Other treatment options
include sclerotherapy, aspiration, steroid therapy, laser
treatment, radiofrequency ablation, or cautery [38]. Medical
therapy with sirolimus has shown promise in microcystic
lymphatic malformations, demonstrating radiologic and
clinical improvement in some treated patients [36].
Infectious and Inflammatory Neck Disorders
 ervical Lymphadenitis and Abscess
C
Cervical adenitis refers to inflammation of the cervical lymph
nodes, and is a common disorder in children. Cervical adenitis
is most often caused by a viral infection, resulting in bilateral
cervical nodal enlargement. If unilateral, bacterial infection of
oropharyngeal origin is often the underlying cause [6, 11, 39].
Clinical presentation is typically a painful cervical mass in a
child that often resolves with conservative medical treatment,
without the need for further imaging evaluation.
Ultrasound with color and spectral Doppler shows enlarged
lymph nodes with increased vascularity and low-­resistance
arterial flow, particularly in the submandibular, parotid, and
upper internal jugular nodal chain regions (Fig. 4.28) [39, 40].
Reactive lymph nodes maintain a normal internal architecture

a b

Fig. 4.27 Lymphatic malformation in a 2-year-old male with a supra- level (asterisk). (b) Transverse color Doppler ultrasound image shows a
clavicular neck mass. (a) Transverse grayscale ultrasound image shows modest amount of vascular flow (arrowheads) within the septations of the
a cystic lesion containing multiple septations (arrows) and a fluid-debris lesion

a b

Fig. 4.28 Lymphadenitis in a 6-year-old male with a tender neck mass. (a) Longitudinal grayscale ultrasound image shows enlarged cervical
lymph nodes (asterisks). (b) Longitudinal color Doppler ultrasound image shows marked nodal hyperemia
4 Neck
with a distinct hilum. A conglomerate mass of lymph nodes
may be seen in uncomplicated cervical adenitis, but nodal
shape, homogeneous, hypoechoic parenchyma, and distinct
echogenic nodal hila are preserved.
Fig. 4.29 Suppurative adenitis in a 7-week-old male with a neck mass.
Longitudinal grayscale ultrasound image shows an inflammatory lesion
comprised of multiple confluent lymph nodes (arrowheads) with scat-
tered hypoechoic foci compatible with necrosis
a b
Fig. 4.30 Retropharyngeal abscess in a 4-month old male with a pal-
pable neck mass. (a) Longitudinal grayscale ultrasound image shows a
large fluid collection (asterisk) within the deep soft tissues of the neck
145
Complications of cervical adenitis can occur, such as sup-
puration and abscess formation, and are readily evaluated
with ultrasound. Suppurative lymph nodes demonstrate het-
erogeneously decreased echogenicity with progressive cen-
tral necrosis, decreased definition or loss of the echogenic
hila, and decreased vascularity on color Doppler evaluation
(Fig. 4.29). Central nodal complexity with debris, septa, or
foci of air can also be identified. There is often adjacent reac-
tive edema of the surrounding perinodal soft tissues with
effacement of the normal fat planes. Abscess formation
appears on ultrasound as a heterogeneously hypoechoic or
anechoic collection with a thick and irregular, hypervascular
rim (Fig. 4.30).
If lymphadenopathy does not resolve after a trial of anti-
biotic therapy, tissue sampling is often required to evaluate
for malignancy.
Mycobacterial Infection
Cervical adenitis secondary to mycobacterial infection
such as tuberculosis is uncommon in developed countries
but can occur in immunocompromised individuals. In
regions where tuberculosis is endemic, mycobacterial
infection is a much more common cause of lymphadenop-
athy in children.
Enlarged, caseating, matted lymph nodes can be seen by
ultrasound predominantly in the internal jugular chain, pos-
abutting the cervical spine (arrows). (b) Axial contrast-enhanced CT
image shows the fluid collection (arrows) in the retropharyngeal space
with minimal peripheral enhancement
146
Fig. 4.31 Scrofula (tuberculous cervical lymphadenitis) in a 7-year-­
old male with neck swelling, erythema, and a positive purified protein
derivative (PPD) skin test. (a) Longitudinal grayscale ultrasound image
shows a complex cystic structure with internal debris (asterisk) and
thick septations (arrows). (b) Transverse power Doppler ultrasound
Fig. 4.32 Lymphadenopathy secondary to cat-scratch disease in a
4-year-old male with submandibular swelling. Longitudinal grayscale
ultrasound image shows multiple enlarged, rounded, mildly heteroge-
neous lymph nodes (asterisks)
terior triangle, and supraclavicular fossa, with eventual sup-
puration and central necrosis as well as adjacent reactive
cellulitis or myositis (Fig. 4.31) [6, 11, 40, 41].
 ther Infectious and Inflammatory Disorders
O for inoperable cases.
Enlarged, hypoechoic, and sometimes heterogeneous lymph
nodes can be seen in other less common infectious and inflam-
matory disorders such as human immunodeficiency virus
(HIV) infection, cat-scratch disease (Fig. 4.32), Kawasaki dis-
ease, sinus histiocytosis, and sarcoidosis. The ultrasound
appearance of enlarged lymph nodes related to these underly-
ing diseases cannot be distinguished from infectious or neo-
plastic lymphadenopathy by imaging alone. Correlation with
laboratory data and/or biopsy is required for definitive
diagnosis.
P. T. Acharya et al.
image shows blood flow (arrow) at the periphery of the lesion. (c) Axial
contrast-enhanced CT image of the neck shows a peripherally enhanc-
ing collection posterior to the left mandible containing multiple unen-
hancing, necrotic foci (arrowheads)
Neoplastic Neck Disorders
Benign Neck Neoplasms
Myofibromatosis
Myofibromatosis is the most common fibrous tumor of child-
hood [42] and is histologically composed of well-­
circumscribed nodules of plump spindle-shaped cells with
staining characteristics intermediate between that of smooth
muscle cells and fibroblasts. Central necrosis with focal cal-
cification is often present [43]. These lesions can occur in the
skin, muscle, bone, viscera or intracranially [44]. Most
lesions develop in children less than 2 years of age.
There are three forms of the disease: (1) the solitary type,
also called “infantile myofibroma”; (2) the multicentric type
without visceral involvement; and (3) the multicentric type
with visceral involvement [45]. The solitary and the multi-
centric types without visceral involvement have a generally
good prognosis, and recurrence is rare. When feasible, surgi-
cal excision is the treatment of choice. Chemotherapy is used
On ultrasound, lesions appear as a nonspecific soft tissue
mass, often with a lobulated contour, and central mixed
anechoic and hyperechoic components (Fig. 4.33). Thick
septations may be present, and the lesions are typically hypo-
vascular by color Doppler, although vascularity can be vari-
able [44]. Additional cross-sectional imaging with CT and
MR imaging is useful to assess disease extent, determine
bone and soft tissue involvement, and to screen for additional
lesions.
4 Neck
Fig. 4.33 Myofibromatosis in a 7-day-old male with a right neck mass.
(a) Transverse grayscale ultrasound image shows a lobulated, hypo­
echoic soft tissue mass containing a central anechoic, fluid-filled zone
Fig. 4.34 Neurofibroma in a 5-year-old female with neurofibromatosis
type I. Longitudinal grayscale ultrasound image of the neck shows a
lobulated, hypoechoic mass (arrowheads)
Neurofibroma
A neurofibroma is a benign tumor arising from the nerve
sheath, and along with schwannoma, are the most common
neurogenic tumors. In the neck, they are most often found
in the posterior triangle [46]. Although neurofibromas can
be sporadic, they are frequently seen in children with neu-
rofibromatosis type 1 (von Recklinghausen disease), an
autosomal dominant phakomatosis characterized by the
development of widespread cutaneous, subcutaneous, and
deep neurofibromas, including plexiform neurofibromas. A
slow growing uniform, elongated soft tissue mass located
along the path of a nerve is typically seen.
On ultrasound, a neurofibroma usually appears as a well-­
circumscribed, homogeneous, and hypoechoic mass (Fig. 4.34)
147
(asterisk) consistent with a cystic component. (b) Transverse color
Doppler ultrasound image shows minimal flow within the lesion
that may display posterior acoustic enhancement. It can also
have a target appearance with peripherally hypoechoic and
centrally hyperechoic zones [47]. A plexiform neurofibroma
arises from multiple nerves and demonstrates diffuse periph-
eral nerve involvement rather than a focal mass, and may
exhibit infiltrative behavior, extending into adjacent structures,
including skin, muscle, bone, and internal organs [48, 49].
Malignant peripheral nerve sheath tumor (MPNST) is a
high-grade sarcoma that can arise from within a preexisting
plexiform neurofibroma, or de novo at a site of prior irradia-
tion. It is difficult to differentiate between MPNST and neu-
rofibroma by ultrasound, although some MPNSTs may
demonstrate hyperemia [49]. Increased uptake of Gallium-67
citrate by MPNST compared to neurofibroma can be seen on
radionuclide imaging. MR imaging may demonstrate altered
signal intensity in MPNST compared to benign lesions [50].
Lipoma and Lipoblastoma
Almost all fatty tumors in children are lipomas or lipoblasto-
mas. A lipoma is composed of mature adipose tissue and
most often occurs in the subcutaneous tissues of the extremi-
ties [51]. On ultrasound, it appears as a well-defined, homo-
geneous mass of variable echogenicity (iso-, hyper- or
hypoechoic to the adjacent soft tissues). It is typically avas-
cular on Doppler evaluation (Fig. 4.35).
Lipoblastoma is a benign tumor that occurs in infants and
children younger than 5 years of age [51]. It contains multiple
lobules of immature fatty tissue separated by fibrous septa.
Most arise in the superficial soft tissues or subcutaneous tissues
of the extremities, although they can occur in the neck or trunk.
On ultrasound, a lipoblastoma typically cannot be differentiated
from a lipoma (Fig. 4.36), although it can have myxoid compo-
148 P. T. Acharya et al.

Fig. 4.35 Lipoma in a 12-year-old male with a painless, soft lump in ultrasound image reveals no increased vascularity within the lesion. (c)
the left submandibular region. (a) Transverse grayscale ultrasound Coronal contrast-enhanced CT image shows a fatty tumor (arrow) in the
image reveals a well-circumscribed, mildly echogenic, lobulated mass left submandibular region
(asterisk) in the subcutaneous tissues. (b) Longitudinal power Doppler

a b

Fig. 4.36 Lipoblastoma in a 7-month-old female with a painless right-­ the subcutaneous tissues. (b) Coronal T1-weighted MR image shows the
sided neck mass. (a) Longitudinal grayscale ultrasound image reveals a right neck mass (asterisk) medially displacing the adjacent common carotid
well-circumscribed, mildly heterogeneous, hyperechoic mass (asterisk) in artery (arrow). The hyperintensity of the mass is due to its fat content
4 Neck 149

nents that predominate and demonstrate enhancement on CT or

a
MR imaging after intravenous contrast administration.
Lipoblastoma can sometimes be diffuse and infiltrative,
growing along fascial planes and invading muscles, a condi-
tion referred to as diffuse lipoblastomatosis. Since liposar-
coma can have a similar imaging appearance, definitive
differentiation requires tissue sampling. However, liposar-
coma is rare in children.

Malignant Neck Neoplasms

Lymphoma
Lymphoma is the third most common malignant tumor of b
childhood, accounting for about half of all head and neck
cancers in children [6]. A neck mass is a common initial
finding with non-Hodgkin and Hodgkin lymphoma sub-
types typically affecting younger and older children,
respectively. There are additional categories of lymphoma,
which are classified according to the cell line involved and
histological features, with some more aggressive than oth-
ers. It is not possible to differentiate between them by
imaging alone, and a tissue diagnosis is always necessary.
The ultrasound features of lymphoma are not specific,
with overlap in the appearance of benign and malignant
lymph nodes. Common findings include enlarged, round,
hypoechoic nodes with distorted hilar anatomy and increased
central and peripheral vascularity (Fig. 4.37) [40]. Internal
reticulation, a micronodular appearance, and elevated arterial
resistance on spectral Doppler evaluation can also be seen
[39, 40]. There may be a matted, mass-like ­conglomeration of
nodes. Nodal calcification can be seen if the patient has
already received treatment.
Cross-sectional imaging is important for accurate staging,
and is used to evaluate the extent of disease, including medias-
tinal masses and distant sites of lymphadenopathy. Ultrasound Fig. 4.37 Lymphadenopathy due to diffuse large B-cell lymphoma in a
is useful for assessing changes in the superficial nodes during 12-year-old male. (a) Longitudinal grayscale ultrasound image shows a
markedly enlarged, lobulated, and hypoechoic cervical lymph node. (b)
and after treatment. MR imaging is used to determine central
Axial positron emission tomography (PET)-CT image shows increased
nervous system involvement. fluorodeoxyglucose (FDG) activity (asterisk) within the left cervical
The thyroid and salivary glands, most commonly the lymph node chain
parotid gland, can undergo lymphomatous infiltration, and
will appear enlarged and hypoechoic. thetic ganglia. Neuroblastoma in the neck usually represents
Many types of lymphoma are curable with chemotherapy metastatic disease [53]. Primary cervical neuroblastoma accounts
alone or in combination with radiation therapy, and cure for less than 5% of all neuroblastoma cases, and has a more
rates are high compared to many other malignancies [52]. As favorable outcome in comparison to other primary sites [54].
prognosis depends not only on histological subtype and Affected children typically present with a nontender lateral neck
grade but also on stage, imaging plays an important role in mass. Additional symptoms related to compression of adjacent
patient management. structures can also occur, including Horner syndrome, dyspha-
gia, and stridor [6, 54, 55].
Neuroblastoma Ultrasound typically shows a solid, complex, or hypoechoic
Neuroblastoma is the third most common malignant neoplasm in mass arising posterior to the carotid sheath, sometimes with
children, and arises from neural crest cells within the sympa- shadowing calcifications, and displacement or encasement
150
of the carotid artery and internal jugular vein (Fig. 4.38)
[54]. However, ultrasound findings in neuroblastoma are
nonspecific. Therefore, CT, MR imaging, and/or radionu-
clide imaging with I-123 metaiodobenzylguanidine (MIBG)
are usually performed to confirm the diagnosis.
The addition of cross-sectional imaging also aids in assess-
ing involvement and mass effect on adjacent structures. On
CT, calcification, hemorrhage, or necrosis may be seen within
the tumor. MR imaging usually demonstrates low signal on
T1- and mildly high signal on T2-weighted images with areas
of internal restricted diffusion and intense contrast enhance-
ment [11]. MR imaging can also be helpful in the evaluation
of encasement and/or displacement of the major neck vessels
as well as determining intraspinal involvement. I-123 MIBG
scintigraphy is highly sensitive in ­demonstrating significantly
increased uptake at sites of disease (Fig. 4.38c).
Treatment depends on the stage of disease. Surgical exci-
sion is the treatment of choice for localized tumors, consid-
ered to be low risk with good prognosis. High-risk tumors
require a combination of surgery, chemotherapy, and/or bone
marrow transplantation. When tumors are very large, neoad-
juvant chemotherapy may be given in an attempt to down-
stage the tumor [56].
Rhabdomyosarcoma
Rhabdomyosarcoma is the most common soft-tissue sarcoma of
childhood, although the neck is the least frequent primary loca-
tion. When rhabdomyosarcoma arises from the head and neck, it
usually originates from the orbital, facial, or cervical muscula-
Fig. 4.38 Neuroblastoma in a 5-year-old male with right facial swelling
and Bell’s palsy. (a) Longitudinal grayscale ultrasound image shows a
lobulated mass containing calcifications (arrowheads) within the soft tis-
sues of the right neck. (b) Axial contrast-enhanced CT image shows a
P. T. Acharya et al.
ture [57]. Histologically, rhabdomyosarcoma is a low-grade,
small, round blue cell tumor similar to other pediatric malignan-
cies such as neuroblastoma, Ewing sarcoma, and lymphoma
[57]. Rhabdomyosarcoma often behaves aggressively and can
invade adjacent structures such as the salivary glands and metas-
tasize early in its course, leading to lymphadenopathy.
Ultrasound is helpful when evaluating superficial rhabdo-
myosarcomas of the neck and in identifying suspicious adja-
cent lymph nodes [58]. A primary cervical rhabdomyosarcoma
is typically solid on ultrasound with low-to-medium echo-
genicity and variable internal vascularity with Doppler
(Fig. 4.39a). Further evaluation with MR imaging is needed
to assess disease extent for staging purposes. MR imaging
typically depicts isointense to slightly hyperintense lesions
on T1-weighted images and T2 hyperintensity, with marked
contrast enhancement. Intralesional hemorrhage and/or
necrosis can also occur (Fig. 4.39b) [11]. Tissue sampling is
always required for definitive diagnosis [58].
Treatment is based on location of the primary tumor and
extent of disease, with a primary neck tumor being associ-
ated with a better prognosis [6, 57, 59]. First line treatment is
chemotherapy, with radiation therapy and surgery used for
local tumor control [57].
Metastatic Disease
Neuroblastoma, leukemia, rhabdomyosarcoma, non-­Hodgkin
lymphoma (Fig. 4.37), and Hodgkin lymphoma are the most
common causes of metastatic cervical nodal disease in chil-
dren [60]. Ultrasound demonstrates round, hypoechoic lymph
large, heterogeneously enhancing right neck mass (asterisk) extending
into the retropharyngeal space. (c) Whole body iodine (I)-123 metaiodo-
benzylguanidine (MIBG) image shows abnormal uptake within the right
cervical soft tissues (arrow) corresponding to the site of neuroblastoma
4 Neck
Fig. 4.39 Rhabdomyosarcoma in a 2-year-old female with left neck
swelling. (a) Longitudinal grayscale ultrasound image shows a mildly
heterogeneous, hypoechoic mass (arrows). (b) Axial contrast-enhanced,
Fig. 4.40 Ectopic thyroid tissue in a 2-year-old female with an anterior
neck bump. (a) Transverse grayscale ultrasound image shows no thy-
roid tissue in the thyroid bed (arrows). (b) Transverse grayscale ultra-
nodes with an absent or eccentric hilum and irregular margins.
Calcification can be seen in metastatic nodal disease from
papillary thyroid carcinoma. Peripheral vascularity is often
identified on color Doppler evaluation with minimal to absent
central or hilar flow.
Thyroid Gland
Congenital Thyroid Gland Anomalies
Dysgenesis
Thyroid dysgenesis is the most common cause of neonatal
hypothyroidism and manifests as three main forms: aplasia,
hypoplasia, and ectopia [61]. Neonates with thyroid dysgenesis
are typically asymptomatic at birth due to circulating maternal
thyroid hormones. Because untreated hypothyroidism can
151
T1-weighted, fat-suppressed MR image shows an enhancing, partially
necrotic left cervical mass (arrows)
sound image obtained higher in the neck, at the level of the thyroid
cartilage, shows right-sided ectopic thyroid tissue (arrow)
result in growth failure and intellectual disability, routine neo-
natal screening is performed, which shows decreased T3 and
T4 and increased thyroid-stimulating hormone levels. Ultra­
sound is the best imaging modality to evaluate the neck in
patients with congenital hypothyroidism, with classification of
the thyroid gland as large, normal, small, or absent.
Ectopic thyroid tissue, caused by arrest of the normal
descent of the thyroid anlage during embryonic life, is often
lingual in location, but can be found anywhere along the
migration pathway from the base of the tongue to the intra-
thoracic trachea. It is the most common type of thyroid dys-
genesis. In the majority of patients, the ectopic thyroid is the
only functioning thyroid tissue.
Ultrasound of an ectopic thyroid in a neonate typically
shows a well-defined ovoid structure that is hyperechoic rela-
tive to adjacent tissues and highly vascular on color Doppler
imaging (Fig. 4.40) [62]. In older children, the gland may
152
appear hypoechoic with no increase in vascularity. Some chil-
dren with ectopic tissue may be euthyroid and present with a
mass at the base of the tongue. It is important to identify this
tissue as ectopic thyroid since its removal in the absence of
other thyroid tissue will result in hypothyroidism. When ultra-
sound cannot identify ectopic thyroid tissue, nuclear scintigra-
phy can be performed using technetium (Tc)-99m pertechnetate,
iodine (I)-131 or I-123 scintigraphy for the detection of ectopic
thyroid tissue with a high degree of sensitivity.
Dyshormonogenesis
Thyroid dyshormonogenesis is a disorder caused by inborn
errors of thyroid hormone biosynthesis and is a less frequent
cause of neonatal hypothyroidism [63]. When the synthetic
defect results in reduced hormone production, an increase in
thyroid-stimulating hormone (TSH) secretion results, stimu-
lating the thyroid gland. Consequently, patients are either
born with an enlarged thyroid gland (goiter) or develop a
goiter postnatally. On ultrasound, an enlarged, normally
positioned thyroid gland of normal parenchymal echo-
genicity is typically seen. There is often enlargement of the
isthmus, and the pyramidal lobe may also be enlarged [63].
Fig. 4.41 Colloid cysts in a 12-year-old female with a goiter.
Longitudinal grayscale ultrasound image shows two small, hypoechoic,
cystic structures (arrowheads) in the superior aspect of the right thyroid
lobe (T) containing punctate, central hyperechoic foci
a b
T
Fig. 4.42 Simple thyroid cyst in a 14-year-old male. Transverse (a)
and longitudinal (b) grayscale ultrasound images reveal a large cyst
replacing most of the left thyroid (T) lobe. (c) Left anterior oblique
P. T. Acharya et al.
Focal Thyroid Gland Lesions
Cystic Focal Thyroid Gland Lesions
Colloid Cysts
Colloid cysts are non-neoplastic nodules consisting of
enlarged follicles containing abundant colloid, and may
result from hyperplasia and subsequent involution of thyroid
follicles. They are usually hypoechoic and may contain foci
of necrosis or hemorrhage that appear as internal echoes or
septations [64]. Colloid cysts can also contain calcifications
and inspissated colloid, which manifests on ultrasound as a
comet-tail artifact (Fig. 4.41).
Simple Cysts
Simple thyroid cysts are rare, consisting of true epithelial-­
lined cysts [65]. On ultrasound, they appear anechoic with
smooth walls and demonstrate enhanced through transmis-
sion (Fig. 4.42). They are avascular on Doppler imaging.
Complex (Hemorrhagic) Cysts
Bleeding can occur into a colloid cyst or follicular adenoma
leading to a hemorrhagic cyst. Hemorrhage can be spontane-
ous or the result of cervical trauma [66]. Acute hemorrhagic
cysts appear on ultrasound as hyperechoic, complex lesions
with internal septations or debris, and wall irregularity. More
chronic lesions appear hypoechoic with well-defined mar-
gins (Fig. 4.43) and can contain fluid-fluid levels.
 olid Focal Thyroid Gland Lesions
S
Thyroid nodules in children are unusual, with a reported
incidence of approximately 1.5% in prepubertal children
[67]. However, the incidence of differentiated thyroid cancer
has been rising steadily in patients aged 10–19 years through-
out the world. The reasons for this increase are uncertain. A
c
image from an I-123 radionuclide study reveals a large photopenic
defect (arrows) in the lower left thyroid lobe with normal radiotracer
uptake in the remainder of the gland
4 Neck
a b
Fig. 4.43 Hemorrhagic cyst in an 18-year-old female who suddenly
developed left-sided neck swelling and difficulty swallowing. Transverse
(a) and longitudinal (b) grayscale ultrasound images of the thyroid gland
show a complex cyst (white arrows) in the left thyroid lobe that contains
recent review of data from 39 cancer registries in the United
States during 1998–2013 revealed an increase in diagnosis of
these differentiated cancers of 4.43% per year that could not
be explained solely on the basis of increases in medical sur-
veillance during childhood since rates of large and late-stage
tumors have been increasing over time [68]. There may be
dietary, environmental, and genetic factors contributing to
the rising rates of differentiated thyroid cancer.
The American College of Radiology (ACR) Thyroid
Imaging Reporting and Data System (TI-RADS) [69], along
with a Thyroid Ultrasound Reporting Lexicon [70], has been
recently developed to provide a standard system for risk
stratification and management of thyroid nodules detected
by ultrasound. The ACR TI-RADS assigns points in five cat-
egories based on ultrasound features that include composi-
tion, echogenicity, shape, margin, and echogenic foci. The
sum of these points corresponds to a risk level that ranges
from TR1 (benign) to TR5 (highly suspicious). This score is
used along with the maximal dimension of the nodule to
guide management.
ACR TI- RADS has been successfully evaluated and
implemented in adult patients because of its simplicity and
153
lace-like internal septations and dependent debris (arrowhead) in keep-
ing with blood products. Incidental note is made of a small colloid cyst
(black arrow). (c) Transverse power Doppler ultrasound image confirms
the avascular nature of the cyst contents
efficacy. However, the use of ACR TI-RADS criteria for the
management of pediatric thyroid nodules has not been well
established in the literature.
The ACR TI-RADS criteria were developed with the
assumption that not all thyroid cancers in adults are clinically
important, and that the criteria should aid in identification of
nodules with an increased likelihood of being a biologically
significant cancer. While this assumption may be acceptable
for adults with thyroid nodules, it might not apply to children
and adolescents.
A recent study reviewed the scoring of 404 nodules in 314
patients aged 2–18 years, of which 19.1% (77 of 404) were
malignant. Most cancers were papillary carcinoma. Use of
the ACR TI-RADS criteria would have resulted in 17 (22.1%)
of 77 cancers being missed at the initial visit [71]. This man-
agement plan is not acceptable in the pediatric population,
where there is a higher rate of cancer as well as differences in
histopathologic characteristics.
Suggested modifications to the ACR TI-RADS risk
level for pediatric nodules might improve the detection of
pediatric thyroid cancers, including a recommendation
that regardless of ultrasound characteristics, all nodules
154
larger than 4 cm should undergo fine needle aspiration
with consideration for surgical excision, since nodules
larger than 4 cm have a high rate of false-negative cyto-
logical findings [71].
Benign Solid Focal Thyroid Gland Lesions
Follicular Adenoma
A follicular adenoma is the most common benign lesion of the
thyroid and arises from an over-production of follicular cells
[72]. It appears as a solitary, round, well-encapsulated, and
hyperechoic or isoechoic lesion relative to the normal thyroid
gland parenchyma. Its fibrous capsule can be seen on ultra-
sound as a peripheral, thin hypoechoic halo (Fig. 4.44).
a b
T T
Fig. 4.44 Follicular adenoma in a 12-year-old female. Transverse (a)
and longitudinal (b) grayscale ultrasound images show a well-circum-
scribed, hypoechoic nodule (cursors) with a thin, hypoechoic halo in
P. T. Acharya et al.
Hemorrhage into a follicular adenoma can cause sudden
enlargement, which appears on ultrasound as a cystic change.
Inspissated colloid manifests as small echogenic foci with pos-
terior reverberation (comet-tail) artifacts. Peripheral eggshell
calcifications are sometimes seen. Peripheral and occasionally
central vascularity may be visualized with Doppler imaging.
Hyperplastic or Adenomatoid Nodules
Hyperplastic nodules contain follicles, colloid, and fibrous
tissue, and unlike follicular adenomas, are usually unencap-
sulated. They are often multiple and occur in patients with
multinodular goiter, usually peripubertal girls. In children,
genetic susceptibility is a common cause, although autoim-
mune factors may also be involved [73].
the right thyroid lobe (T). (c) Transverse color Doppler ultrasound
image shows hyperemia (arrow) at the periphery of the nodule as well
as internal blood flow (arrowhead)
4 Neck
a b
T T
Fig. 4.45 Adenomatous nodule in a 14-year-old male with a PTEN
mutation. Transverse (a) and longitudinal (b) grayscale ultrasound
images reveal a well-circumscribed, hypoechoic mass (cursors) with a
On ultrasound, the nodules are well defined, of variable
echogenicity, with a halo (Fig. 4.45). Cystic changes may
occur from necrosis or hemorrhage. Internal septations, wall
thickening, and solid mural nodules can be seen. Varying
degrees of peripheral and internal vascularity are detected
with Doppler imaging.
Malignant Solid Focal Thyroid Gland Lesions
Thyroid Cancer
Thyroid cancer is rare in children, with papillary thyroid can-
cer being the most common subtype of pediatric thyroid
malignancy, representing approximately 80% of all cases
155
thin hypoechoic halo (arrowheads) in the left thyroid lobe (T). (c)
Longitudinal color Doppler ultrasound image depicts flow at the periph-
ery of the nodule (arrow) as well as centrally (arrowhead)
[74, 75]. There is an increased risk of thyroid cancer in chil-
dren with a history of prior neck irradiation. It is slightly
more common in females than in males [6, 76, 77]. In con-
trast to adult thyroid cancers, pediatric thyroid cancers are
often at an advanced stage at diagnosis. Nonetheless, they
are usually well differentiated on histopathologic examina-
tion, and have a better prognosis than those occurring in
adults. Pediatric patients often present with a palpable thy-
roid nodule, cervical adenopathy, and distant metastases
(most often to the lungs and bones) [6, 74, 75, 78].
Ultrasound evaluation of malignant thyroid nodules typi-
cally demonstrates predominantly solid and hypoechoic
lesions with irregular or microlobulated borders. There may
156 P. T. Acharya et al.

a b

c d

Fig. 4.46 Papillary thyroid cancer with lymph node metastases in a
17-year-old male. Transverse (a) and longitudinal (b) grayscale ultra-
sound images demonstrate replacement of the left thyroid lobe by a
heterogeneous mass with ill-defined, infiltrative margins and containing
multiple microcalcifications. (c) Longitudinal color Doppler ultrasound
image of the left thyroid lobe shows prominent vascularity throughout
the mass. (d) Longitudinal grayscale ultrasound image of the left neck
adjacent to the internal jugular vein (V) shows echogenic metastatic
tumor deposits (arrowheads) within a lymph node. Additional nodes in
both sides of the neck had a similar appearance (not shown)

be prominent perinodular and/or intranodular vascular flow. Table 4.1 Characteristic ultrasound features of benign and malig-
Microcalcifications within the lesion as well as in involved nant thyroid nodules
lymph node chains may be seen (Fig. 4.46). In addition, Category Ultrasound features
abnormal cervical lymph nodes can be enlarged and hetero- Benign Predominantly cystic
geneous in echotexture with loss of the normal hilar stripe, Smooth peripheral halo
demonstration of a peripheral vascular pattern, and possible Coarse or eggshell calcifications
central necrosis (Table 4.1) [79–81]. A diffusely enlarged
thyroid gland with multiple microcalcifications should raise Malignant Predominantly solid
Taller than wide
similar suspicion, and fine needle aspiration performed for
Hypoechoic
further evaluation [74, 78]. As mentioned previously, there is Microcalcifications
an association with thyroglossal duct cysts [8]. Irregular margins
Treatment of thyroid cancer involves both surgical resec- Absent peripheral halo
tion and administration of radioactive iodine for lymph node Hypervascular parenchyma
metastases. Abnormal lymph nodes
4 Neck
Fig. 4.47 Lymphomatous involvement of the thyroid gland in a
17-year-old female with Hodgkin lymphoma. Transverse (a) and longi-
tudinal (b) grayscale ultrasound images show a hypoechoic nodule
(arrows) in the lower pole of the right thyroid gland containing a thick
internal septation (arrowheads). (c) Longitudinal color Doppler ultra-
Lymphoma
Primary thyroid lymphoma is an uncommon tumor, and usu-
ally occurs in females in the 6th and 7th decades of life.
Hashimoto thyroiditis is present in the majority of patients.
Secondary lymphomatous involvement of the thyroid gland
is also rare in children [82]. Ultrasound demonstrates a soli-
tary or multiple anechoic to hypoechoic masses replacing the
thyroid (Fig. 4.47).
Diffuse Parenchymal Thyroid Gland Lesions
 ultinodular Goiter (Nodular Hyperplasia)
M clinical manifestations of thyroid gland dysfunction can also
Congenital goiter is a rare cause of neck swelling in infants,
characterized by diffuse or nodular enlargement of the thy-
roid gland [83–85]. This condition may be hereditary, as
with inborn errors in fetal hormone production. More
157
sound image of the right thyroid lobe documents flow (arrowhead)
within the internal septation. (d) Coronal contrast-enhanced CT image
of the chest shows a large mediastinal mass (asterisk) extending into the
neck. The small right thyroid nodule (arrowhead) is visible
c­ ommonly, however, it is due to nonhereditary causes such
as thyroid dysgenesis, transplacental passage of maternal
antibodies in the setting of Graves’ disease, maternal inges-
tion of antithyroid medication, or rare mutations of thyrotro-
pin (TSH) receptors (McCune-Albright syndrome) [83–85].
Affected infants are often asymptomatic. However, they
may present with a diffusely enlarged thyroid gland with stri-
dor and respiratory distress due to mass effect. There may be a
prenatal history of polyhydramnios related to dysphagia [83–
85], or the birth history might include a difficult vaginal deliv-
ery due to cervical dystocia. On physical examination, affected
infants may present with cervical hyperextension. Delayed
occur, and patients may only be detected because of abnormal
results on screening thyroid function studies [84, 86].
Early identification of fetal hypothyroidism is impor-
tant for treatment to ensure normal neurologic and motor
158
development [85]. Fetal blood sampling is the only reli-
able means of assessing fetal thyroid function, as amniotic
thyroid ­hormone assays do not correlate with fetal thyroid
status [84].
Fig. 4.48 Congenital goiter in a 2-week-old female with hypothyroid-
ism. Transverse grayscale ultrasound image reveals marked homoge-
neous enlargement of the thyroid gland
a b
Fig. 4.49 Multinodular goiter in a 15-year-old male. (a) Transverse
grayscale ultrasound image shows a predominantly solid nodule in the
thyroid isthmus (asterisk) that contains multiple small cystic spaces. (b)
Transverse grayscale ultrasound image of the right thyroid lobe reveals
P. T. Acharya et al.
A fetal goiter can be seen by ultrasound as a diffusely and
homogeneously enlarged thyroid gland with a circumference
or diameter that is greater than the 95th percentile for gesta-
tional age on the basis of normative values [84, 87]. Postnatal
ultrasound evaluation typically demonstrates diffuse, homo-
geneous enlargement of the thyroid gland with possible mass
effect and narrowing of the adjacent airway (Fig. 4.48).
Tc-99m-pertechnetate imaging can also help in evaluating
for the presence of dyshormonogenesis [88, 89].
Multinodular (adenomatous) goiter is uncommon in chil-
dren. It usually affects adolescent females, and is also associ-
ated with familial and autoimmune factors. Multinodular
goiter has been described in association with Hashimoto thy-
roiditis, McCune-Albright syndrome, renal cystic disease,
polydactyly, and history of radiotherapy. Patients are gener-
ally euthyroid at presentation and present with a palpable
nodule or nodules. On ultrasound evaluation, the nodules are
variable in appearance, and some may appear cystic due to
hemorrhage or necrosis (Fig. 4.49). Long-term follow-up of
a cystic nodule with internal debris. (c) Coronal contrast-­enhanced CT
image shows the enlarged thyroid gland containing nodules (arrow-
heads) that are predominantly cystic in the right lobe and isthmus, as
well as a largely solid nodule (arrow) in the left lobe
4 Neck
a b
Fig. 4.50 Suppurative thyroiditis with an abscess in a 2-year-old
female with fever and a 1-week history of an anterolateral neck mass.
(a) Transverse grayscale ultrasound image shows replacement of the
patients with familial forms of multinodular goiter is recom-
mended because of an increased risk of thyroid cancer.
I nfectious Diffuse Parenchymal Thyroid Gland
Disorders
Acute Suppurative (Bacterial) Thyroiditis
Acute suppurative thyroiditis is rare due to the high iodine
content in the thyroid gland, making it highly resistant to
infection. Staphylococci, streptococci, or anaerobic bacteria
are the common causes of infection. Rarely, a congenital piri-
form sinus (a type III or IV branchial apparatus remnant) with
a fistulous connection to the ipsilateral lobe of the thyroid
gland or the perithyroidal space can become infected [90].
These congenital fistulas are more common on the left side.
Children with suppurative thyroiditis are usually euthyroid
and typically present with fever, sore throat, and swelling in the
lower neck. On ultrasound, heterogeneity of the gland can be
seen with increased vascularity on Doppler imaging (Fig. 4.50).
Abscesses may develop which appear as hypoechoic or com-
plex masses with surrounding hyperemia [90, 91].
Subacute (De Quervain) Thyroiditis
Subacute granulomatous thyroiditis is an uncommon transient
inflammatory disease that usually follows an upper respiratory
viral infection [92]. Affected patients may present with fever
and mild thyrotoxicosis with elevated serum T3 and T4 levels
and decreased TSH levels.
159
left thyroid lobe by a complex cystic mass (asterisk). (b) Axial contrast-
enhanced CT image of the neck shows a peripherally enhancing collec-
tion (arrow) with septations in the left thyroid lobe
On ultrasound, the thyroid gland appears enlarged and
heterogeneous, with poorly defined foci of decreased
echogenicity (Fig. 4.51) [93].When the condition resolves
and the patient is euthyroid, the echogenicity of the
thyroid usually normalizes. Treatment consists of high
doses of nonsteroidal anti-inflammatory medication or
corticosteroids.
 utoimmune-Mediated Diffuse Parenchymal
A
Thyroid Gland Lesions
Graves’ Disease
Graves’ disease is the most common cause of hyperthyroidism
in children. It is an autoimmune disorder caused by thyroid-­
stimulated immunoglobulin binding to the TSH receptor and
resulting in increased production of TSH. It is more common
in girls than in boys, with a peak incidence in adolescence
[91]. Graves’ disease is also more prevalent in patients with
McCune-Albright syndrome. Infants born to mothers with
Graves’ disease are also at risk for congenital hyperthyroidism
from transfer of thyroid-stimulating immunoglobulins.
Affected children typically present with an enlarged thy-
roid gland, exophthalmos, and hyperthyroidism, which can
be diagnosed by elevated serum T4 and T3 levels and
decreased serum TSH. There is diffuse gland enlargement
with a lobulated contour and a heterogeneous appearance of
the thyroid parenchyma on ultrasound. Hypervascularity is
marked, with an appearance on color Doppler imaging
160 P. T. Acharya et al.

Fig. 4.51 Subacute thyroiditis in a 15-year-old male. Transverse gray- heterogeneity with multiple small cystic changes (arrowheads). (c) Four-
scale ultrasound image of the thyroid gland (a) and longitudinal grayscale hour anterior planar image of the neck from an I-123 radionuclide study
ultrasound image of the left thyroid lobe (b) reveal subtle parenchymal shows minimal uptake by the thyroid gland

a b

Fig. 4.52 Graves’ disease in a 14-year-old female. (a) Transverse gray- Doppler ultrasound image demonstrates marked, diffuse hypervascular-
scale ultrasound image shows a diffusely enlarged thyroid gland with a ity of the thyroid gland
heterogeneous appearance of the parenchyma. (b) Transverse color

referred to as “thyroid inferno” (Fig. 4.52). Pulsed Doppler Hashimoto Thyroiditis

imaging demonstrates elevated peak arterial systolic veloci- Hashimoto thyroiditis is an autoimmune disorder caused by
ties with a decreased resistive index (RI) [94]. circulating antibodies that attack thyroglobulin and TSH
Treatment includes antithyroid medication or thyroid receptors, resulting in a diffuse lymphocytic and plasma cell
ablation with radioactive I-131. infiltration. It is the most common cause of thyroid disease in
4 Neck 161

a b

Fig. 4.53 Hashimoto thyroiditis in a 16-year-old female. (a) Transverse Transverse color Doppler ultrasound image shows mildly increased
grayscale ultrasound image shows diffuse enlargement of the thyroid blood flow within the thyroid gland
gland with hypoechoic and slightly heterogeneous parenchyma. (b)

children and adolescents and more frequently affects girls than a

boys [95]. Affected children present with painless enlarge-
ment of the thyroid gland. In the acute phase, patients may be
euthyroid; however, most eventually become hypothyroid. T
There is an association with several syndromes, including
Turner, Noonan, Down, and Klinefelter syndromes. Hashimoto
thyroiditis can also be associated with autoimmune diseases
such as rheumatoid arthritis and lupus erythematosus, as well
as endocrine disorders, including Addison disease, hypopara-
thyroidism, and diabetes mellitus [91].
On ultrasound, the thyroid is often diffusely enlarged, with
hypoechoic parenchyma that may have a coarsened appear-
ance (Fig. 4.53). Blood flow can be normal, increased, or
decreased. Multiple hypoechoic parenchymal micronodules
are often present. Adjacent cervical adenopathy is frequently
noted. With longstanding disease, the thyroid gland is often
atrophic and fibrotic, and may appear small and hyperechoic. b
Symptoms in most pediatric patients resolve spontane-
ously. Suspicious nodules identified on ultrasound should be
followed due to the increased risk of malignancy.

Parathyroid Glands

Parathyroid Cyst

A parathyroid cyst is a rare lesion that arises either from rem-

nants of the third and fourth pharyngeal pouches or from cystic
degeneration of a parathyroid adenoma. It is usually located in
the neck in the inferior parathyroid glands but can also occa-
sionally occur in the mediastinum. Parathyroid cysts are
divided into two groups: functioning and nonfunctioning [96].
Functioning parathyroid cysts can cause primary hyperpara-
thyroidism. Children with nonfunctioning parathyroid cysts are
usually asymptomatic with normal blood calcium levels.
When large, a nonfunctioning parathyroid cyst can cause
symptoms from mass effect, including dysphagia, pain, tra-
cheal compression, and recurrent laryngeal nerve palsy.
Fig. 4.54 Parathyroid cyst in a 17-year-old female with a painless
anterior neck mass. (a) Longitudinal grayscale ultrasound image dem-
onstrates a thin-walled, anechoic cyst abutting the inferior aspect of the
right thyroid lobe (T). (b) Transverse color Doppler ultrasound image
reveals the avascular nature of the lesion
Ultrasound demonstrates a unilocular, smooth-walled,
anechoic lesion with increased through-transmission (Fig.
4.54). The diagnosis of a parathyroid cyst can be challenging
due to its close proximity to the thyroid gland and is frequently
162
a b
Fig. 4.55 Parathyroid adenoma in a 12-year-old female with primary
hyperparathyroidism. Transverse (a) and longitudinal (b) grayscale ultra-
sound images reveal a hypoechoic oval nodule (arrows) inferior to the
misdiagnosed as a thyroid lesion. Percutaneous aspiration aids
in diagnosis, showing elevated parathyroid hormone levels in
the cyst fluid compared to serum levels [96].
Parathyroid Hyperplasia
Parathyroid hyperplasia is the diffuse enlargement of all four
parathyroid glands and is a cause of primary hyperparathy-
roidism [97]. On ultrasound, the enlarged glands may appear
less echogenic than the adjacent thyroid tissue and are sepa-
rated from the thyroid by a fibrous capsule which can be seen
as a highly echogenic line.
Parathyroid Adenoma
A parathyroid adenoma is a benign parathyroid gland tumor
and the most common cause of primary hyperparathyroidism
P. T. Acharya et al.
lower pole of the right thyroid lobe. (c) Anterior planar image from a
nuclear medicine parathyroid scan shows uptake of technetium (Tc)-
99m sestamibi below the right thyroid lobe (arrow)
[98]. Clinical presentation may be nonspecific with fatigue,
lethargy, abdominal pain, nausea, vomiting, and headache [99].
Parathyroid ultrasound can aid in presurgical planning for
targeted resection of a parathyroid adenoma. Normally, the
parathyroid glands cannot be reliably visualized with ultra-
sound. However, with an adenoma, an oval, hypoechoic solid
mass can be identified. With enlargement, an adenoma dis-
sects between the longitudinally oriented tissue planes and
can assume a more elongated shape [100]. Prominent periph-
eral hypervascularity with a feeding artery can be seen on
color Doppler imaging [101].
Typically, parathyroid adenomas are separated from the
thyroid gland by a hyperechoic capsule [76]. However, when
a parathyroid adenoma lacks a capsule, it can be indistin-
guishable by ultrasound from a thyroid nodule (Fig. 4.55). In
the setting of multinodular thyroid disease, a posterior thy-
roid nodule can be mistaken for a parathyroid adenoma [97].
However, the peripheral vascularity that is more typical of a
parathyroid adenoma than a thyroid nodule can help to dif-
4 Neck 163

ferentiate between the two entities. A centrally located cervi-
cal lymph node can also mimic a parathyroid adenoma.
However, the presence of a fatty hilum and a central vascular
pedicle in a lymph node can aid in making the appropriate
diagnosis.
Tc-99m sestamibi scintigraphy is used for preoperative
localization of a parathyroid adenoma, along with intraop-
erative ultrasound (Fig. 4.55c) [102]. Definitive treatment
for any type of parathyroid dysfunction in children is surgi-
cal resection, including primary, secondary, and tertiary
causes of hyperparathyroidism [98].
angle of the mandible, which is unresponsive to antibiotic
therapy and drainage [10]. Drainage to the external auditory
canal can result in otorrhea. Occasionally, a sinus tract
extending to the hyoid bone may be seen [8–10]. Facial
nerve palsy can also accompany the patient’s symptoms.
On ultrasound, a well-defined, anechoic lesion is seen with
increased through-transmission and no internal vascularity [10].
Superimposed infection or hemorrhage can result in increased
complexity of the cyst with echogenic contents (Fig. 4.14).
Complete surgical excision is the only curative therapy
for these lesions.

Ranula
Salivary Glands A ranula is a mucous retention cyst resulting from obstruc-
tion of the sublingual gland or duct by a post-inflammatory
Congenital Salivary Gland Anomalies stricture, trauma, or calculus. Affected children often present
with a painless, para-midline bluish swelling along the floor
 irst Branchial Cleft Cyst
F of the mouth. Large sublingual cysts can extend below the
A first branchial cleft cyst is an uncommon type of branchial level of the mylohyoid muscle, known as “plunging ranulas”
cleft anomaly that results from incomplete fusion of the first [103].
and second branchial arches [8, 9]. It can occur anywhere Ultrasound features are typical of a cyst with well-defined
along the embryologic tract of the first branchial cleft or arch borders and anechoic contents (Fig. 4.56) [103]. Infected
from the external auditory canal to the parotid gland and sub- cysts demonstrate increased complexity with internal debris,
mandibular triangle. Affected children usually present with mural thickening, and surrounding inflammation.
painless swelling. However, the cyst can also manifest as a These lesions are managed with intraoral marsupializa-
recurrent abscess or sinus tract either around the ear or at the tion or surgical resection.

a b

Fig. 4.56 Plunging ranula in a 2-year-old male. (a) Transverse grayscale ultrasound image demonstrates a cystic mass (asterisk) in the left sub-
mandibular region. (b) Axial T2-weighted, fat-suppressed MR image shows the cystic mass (asterisk) centered in the left floor of the mouth
164 P. T. Acharya et al.

I nfectious and Inflammatory Salivary Gland Bacterial Parotitis and Abscess

Disorders
Acute Infectious and Inflammatory Disorders
Viral (Nonsuppurative) Inflammation
Acute inflammation of the salivary glands in children is usually
caused by a viral infection [104]. The parotid gland is the most
frequently involved salivary gland in the pediatric age group.
The most common viral infections include mumps, mononu-
cleosis, and cytomegalovirus (CMV), which can cause painful
unilateral or bilateral swelling of the salivary glands.
On ultrasound, viral salivary gland infection typically
appears as a diffusely enlarged, heterogeneous gland with
increased vascularity on Doppler imaging (Fig. 4.57). There
is frequently bilateral involvement. Enlarged lymph nodes
can also be seen with increased central hilar flow.
Acute parotitis usually results from a viral infection such
as mumps or, more rarely, a bacterial infection (most
commonly Staphylococcus aureus). Acute suppurative
parotitis occurs in children younger than 1 year of age
and in newborns or children with systemic disease [2].
Bacterial parotitis is usually unilateral, and can result
from the spread of infection from adjacent structures such
as the teeth or ears, parotid calculi, or immunosuppression
[100, 105]. Patients with recurrent acute parotitis often
present with periodic parotid gland swelling, fever, and
pain. They are usually between the ages of 2 and 5 years,
with resolution generally occurring around the time of
puberty [2].
There is enlargement and heterogeneity of the parotid gland
in both viral and bacterial parotitis with hypervascularity on
color Doppler imaging (Fig. 4.58). In bacterial parotitis, intra-

a b

Fig. 4.57 Nonsuppurative sialadenitis in a 20-month-old female with bilat- Longitudinal color Doppler ultrasound image shows increased blood flow
eral facial swelling. (a) Transverse grayscale ultrasound image shows an within the gland
enlarged submandibular gland with mildly heterogeneous parenchyma. (b)

a b

Fig. 4.58 Parotitis in an 8-year-old male with bilateral facial swelling. (a) Longitudinal grayscale ultrasound image shows an enlarged and het-
erogeneous left parotid gland. (b) Longitudinal color Doppler ultrasound image of the parotid shows marked hyperemia
4 Neck
parotid lymph nodes can be seen as hypoechoic nodules.
Suppuration manifesting as avascular hypoechoic areas or dis-
crete abscesses can occur in severe cases (Fig. 4.59).
In recurrent acute parotitis, unilateral or bilateral parotid
enlargement can be seen with multiple small hypoechoic
foci that may represent lymphocytic infiltration or sialecta-
sis [2]. Similar findings can also be seen in HIV-associated
parotitis as well as the presence of lymphoepithelial cysts,
which appear as large anechoic areas nearly replacing the
gland [2, 106].
Treatment depends on the underlying etiology, with anti-
biotics administered for the early stages of bacterial paroti-
tis. Children with an abscess related to complicated bacterial
parotitis require surgical management with drainage.
a b
Fig. 4.59 Bacterial parotitis with abscess in a 3-month-old female
with facial swelling and erythema. (a) Longitudinal grayscale ultra-
sound image shows a multiloculated abscess (asterisks) in the parotid
a b
Fig. 4.60 Chronic sialadenitis in an 8-year-old male. (a) Longitudinal
grayscale ultrasound images of the right parotid gland reveal mul-
tiple small, hypoechoic foci scattered throughout the parenchyma.
165
 ecurrent and Chronic Infectious
R
and Inflammatory Disorders
Chronic Sialadenitis
Chronic sialadenitis is caused by inflammation of the glan-
dular acini that prevents adequate gland drainage [107]. It
can be infectious or noninfectious in etiology, and includes
drug sensitivity, food intolerance, or cystic fibrosis as
underlying disorders. It is clinically characterized by inter-
mittent unilateral or bilateral gland swelling and pain, par-
ticularly after meals.
On ultrasound, chronic sialadenitis can be seen as a
normal-­sized to small, heterogeneous gland with punctate,
echogenic foci or with multiple hypoechoic areas (Fig. 4.60)
gland (P). (b) Longitudinal color Doppler ultrasound image shows
hyperemia of the parotid gland and at the periphery of the abscess
(b) Longitudinal color Doppler ultrasound image of the right parotid
gland shows minimally increased vascularity. The left parotid gland had
a similar appearance (not shown)
166 P. T. Acharya et al.

[108]. The punctate areas are believed to represent either
calcification, mucus in dilated ducts, or the walls of ectatic
ducts. The hypoechoic areas represent edema from lym-
phocytic infiltration and/or sialectasis. Increased vascular-
ity can be observed with color Doppler imaging. There may
also be adjacent cervical lymph node involvement.
On ultrasound, a malignant salivary gland neoplasm can
have irregular margins and appear homogeneously or hetero-
geneously hypoechoic (Fig. 4.61). Other associated findings
include regional lymphadenopathy and encasement or inva-
sion of surrounding structures. Increased internal vascularity
with increased RI and high peak systolic flow velocities can
also be seen. Biopsy is required for definitive diagnosis.

Neoplastic Salivary Gland Disorders Acinar Cell Carcinoma

Acinar cell carcinoma is another common malignant salivary
Benign Salivary Gland Masses gland tumor of childhood with the best prognosis [113]. It
most often involves the parotid gland [112]. Ultrasound fea-
Infantile Hemangioma tures include an irregular, heterogeneous, hypoechoic, and
Hemangioma is a common childhood salivary gland vascular poorly vascularized lesion with a mixed cystic and solid
neoplasm, with the majority arising in the parotid gland [109].
Ultrasound shows an infiltrative or well-­circumscribed, typi-
cally hypoechoic lesion relative to the remainder of the gland, a
similar to the previous description of hemangioma of the sub-
cutaneous tissues (Fig. 4.23). An increased number of arteries
and veins can be seen with color Doppler. Arterial spectral
Doppler demonstrates high velocity and low RIs with spectral
broadening.
Most infantile hemangiomas regress spontaneously.
How­ever, medical management with propranolol, laser ther-
apy, or rarely, surgical excision may be needed for large or
problematic lesions.
b
Lymphatic Malformation
Lymphatic malformation is characterized by dilated lym-
phatic channels that can involve the parotid gland or the sub-
mandibular space. Affected pediatric patients typically
present with a soft, asymptomatic mass.
On ultrasound, a lymphatic malformation within a sali-
vary gland shows thin-walled, multilocular cystic spaces of
varying size with echogenic septations, similar to the previ-
ous description of lymphatic malformation of the supracla-
vicular region (Fig. 4.27) [110]. If there is superimposed
hemorrhage or infection, internal layering echogenic debris
may be seen. Color Doppler imaging can demonstrate mod-
est flow in the septations but not within the cystic spaces.

Malignant Salivary Gland Neoplasms

Mucoepidermoid Carcinoma
Mucoepidermoid carcinoma is one of the most common
malignant salivary gland neoplasms of childhood. Affected
children usually present with an enlarging lesion that may be
rapidly growing and painful. The lesion can cause facial
nerve paralysis or adhere to surrounding structures.
Mucoepidermoid carcinoma has a high incidence of regional Fig. 4.61 Mucoepidermoid carcinoma in a 12-year-old female with an
nodal spread. However, most of these tumors are of low asymptomatic submandibular mass. (a) Transverse grayscale ultra-
sound image shows a mixed cystic and solid lesion (arrows). (b)
grade in children with distant metastases being uncommon Transverse power Doppler ultrasound image shows mild vascularity
[111, 112]. (arrowhead) of the mass
4 Neck
appearance and posterior acoustic enhancement (Fig. 4.62).
As with other malignant salivary gland lesions, differentiation
from a benign mass can be difficult on the basis of imaging
alone, and tissue sampling is needed for definitive diagnosis.
Treatment usually involves surgical resection with radio-
therapy reserved for extra-glandular extension, lymph node
metastases, or local recurrence [112].
Other Malignant Salivary Gland Neoplasms
Other malignant salivary gland neoplasms are less common,
including rhabdomyosarcoma, primary lymphoma, adenoid
cystic carcinoma, and sialoblastoma.
a
P ral invasion, which can cause pain and numbness. Ultrasound
b gland. It is focally aggressive with local recurrence. Distant
Fig. 4.62 Acinar cell carcinoma in a 15-year-old female with a local-
ized parotid mass and a history of Hashimoto thyroiditis. (a) Longitudinal
grayscale ultrasound image shows a mixed cystic and solid mass (arrow)
within the parotid gland (P). (b) Longitudinal color Doppler ultrasound
image shows vascularity (arrowhead) within the solid portion of the
mass
a b
P P
Fig. 4.63 Adenoid cystic carcinoma of the parotid gland in an 11-year-
old female with right parotid swelling and intermittent pain. (a)
Longitudinal grayscale ultrasound image shows a mixed cystic and solid
nodule (arrow) within the right parotid gland (P) immediately anterior
167
Rhabdomyosarcoma can arise from any muscle in the head
and neck, and salivary gland involvement frequently occurs by
direct extension. Although typically predominantly solid on
ultrasound, they may have a mixed appearance.
Primary lymphoma of the salivary glands originates from
mucosa-associated lymphoid tissue and thus is termed
MALToma [114]. Patients with Sjögren syndrome and other
autoimmune diseases, including HIV, are at risk for primary
lymphoma. On ultrasound, focal involvement of the gland or
diffuse infiltration may be seen with multiple small, hypoechoic
nodules, or an irregularly shaped heterogeneous mass without
calcification or cystic degeneration. There is often increased
intralesional vascularity.
Adenoid cystic carcinoma is a rare malignant salivary
gland tumor in children that often affects the parotid gland
[112]. It is usually slow-growing and associated with perineu-
features are nonspecific, overlapping in a­ ppearance with other
malignant lesions (Fig. 4.63). Tissue sampling is therefore
required for definitive diagnosis.
Sialoblastoma is an extremely rare neonatal neoplasm of
the salivary gland that most frequently involves the parotid
metastases are uncommon [115]. As with other malignant
salivary neoplasms, its appearance on ultrasound alone can-
not distinguish it from other masses. Ultrasound may show a
lobulated, mixed echogenicity mass with intralesional hem-
orrhage and necrosis.
Sialolithiasis
Sialolithiasis is uncommon in children, with the majority
occurring in the submandibular gland which is prone to cal-
culi because of the alkalinity and high viscosity of its secre-
tions [109]. Stones are also more likely to form in the Wharton
duct because of its long, ascending course. Sialolithiasis has
been associated with cystic fibrosis but can also be an isolated
to the ­temporomandibular joint. The parenchyma of the parotid gland
appears heterogeneous. (b) Longitudinal color Doppler ultrasound
image shows vascularity (arrowhead) within the nodule. P, Parotid gland
168
a b
Fig. 4.64 Sialolithiasis in a 10-year-old female with pain in a left lower
molar tooth and swelling in the sublingual region. (a) Longitudinal gray-
scale ultrasound image demonstrates enlargement and heterogeneity of
the left submandibular gland (S) with a centrally dilated duct (asterisk).
finding. Affected children typically present with recurrent
painful enlargement of the salivary gland while eating, and
superimposed infection can occur from obstruction of the
duct by a stone.
As with stones elsewhere, salivary gland stones appear
as echogenic foci on ultrasound with posterior acoustic
shadowing. Ductal dilation can be seen with intraluminal
stones (Fig. 4.64). Associated inflammatory changes can
result in a heterogeneous appearance of the salivary gland
parenchyma.
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 Lung
5
Eric S. Bih, Monica Epelman, Ricardo Restrepo,
and Edward Y. Lee

Abbreviations Introduction

B Brightness Ultrasound is a practical and useful non-invasive imaging tool in

CEUS Contrast-enhanced ultrasound the evaluation of congenital and acquired lung disorders, espe-
CCAM Congenital cystic adenomatoid malformation cially in the pediatric population. The important benefits of
chILD childhood interstitial lung disease chest ultrasound include real-time assessment, portability
CLH Congenital lobar hyperinflation (including bedside evaluation for critically ill patients), shorter
CPAM Congenital pulmonary airway malformation examination times (compared to magnetic resonance [MR]
CT Computed tomography imaging), lack of ionizing radiation (compared to conventional
DLD Diffuse lung disease radiography and computed tomography [CT]), and the ability to
ILD Interstitial lung disease perform the examination without sedation. Ultrasound is also
M Motion increasingly used to guide interventional procedures.
MR Magnetic resonance This chapter reviews up-to-date ultrasound imaging tech-
MRSA Methicillin-resistant Staphylococcus aureus niques, normal development and anatomy, and ultrasound
PL Pulmonary lymphangiectasia imaging findings of commonly encountered lung abnormali-
PPB Pleuropulmonary blastoma ties in infants and children.
PRMS Pulmonary rhabdomyosarcoma
RDS Respiratory distress syndrome
RMS Rhabdomyosarcoma Technique
SDD Surfactant deficiency disease
TTN Transient tachypnea of the newborn Patient Positioning

Patient positioning depends on the location of the region of

interest and the clinical condition of the patient. Typically,
the posterior aspect of the chest is scanned with the patient
E. S. Bih (*) sitting up and bending slightly forward. Crossing the arms
Edward B. Singleton Department of Radiology, Texas Children’s
across the chest displaces the scapulae laterally and improves
Hospital, Baylor College of Medicine, Houston, TX, USA
e-mail: Esbih@texaschildrens.org visualization of the posterior upper thorax. The anterior and
lateral aspects of the chest are evaluated with the patient in
M. Epelman
Department of Radiology, Nemours Children’s Hospital, either a supine or lateral decubitus position. Raising an arm
University of Central Florida College of Medicine, above the head increases the intercostal space distance and
Orlando, FL, USA enlarges the acoustic window [1–3].
R. Restrepo
Department of Interventional Radiology and Body Imaging,
Nicklaus Children’s Hospital, Miami, FL, USA
Ultrasound Transducer Selection
E. Y. Lee
Division of Thoracic Imaging, Department of Radiology,
Optimal ultrasound transducer and frequencies depend on
Boston Children’s Hospital and Harvard Medical School,
Boston, MA, USA patient body habitus and the location of the region of

© Springer Nature Switzerland AG 2021 173

H. J. Paltiel, E. Y. Lee (eds.), Pediatric Ultrasound, https://doi.org/10.1007/978-3-030-56802-3_5
174
i­nterest. In general, lower-frequency curvilinear transduc-
ers are used in young children and adolescents, while
higher-frequency linear transducers are typically used in
neonates and small infants [3, 4]. In certain instances
where scanning through a small acoustic window is neces-
sary, a phased array transducer with a smaller footprint can
be useful [2].
Imaging Approaches
It is important to review the patient’s prior imaging studies
(often chest radiographs) in order to identify the region of
interest before proceeding with ultrasound evaluation. Imaging
along the intercostal spaces permits the most comprehensive
assessment of the lungs and pleural spaces in infants and chil-
dren, particularly in younger patients, due to the acoustic win-
dow provided by the unossified sternum and relatively large
thymus. Both grayscale and color Doppler ultrasound imaging
are useful in identifying and characterizing abnormalities.
In cooperative pediatric patients or patients on ventilatory
support, abnormalities can be examined in detail with breath-
holding. In general, conventional brightness (B)-mode ultra-
sound is used for evaluation of the lung parenchyma, while
motion (M)-mode imaging is a useful adjunct for the assess-
ment of pneumothorax. When using color Doppler, appropri-
ate wall filter, pulse repetition frequency, and Doppler gain
settings are used to permit adequate assessment of lesion
vascularity while avoiding interference from vessel wall
motion, respiratory and cardiac motion [2, 3, 5].
Contrast-Enhanced Ultrasound
Contrast-enhanced ultrasound (CEUS) is a relatively new
technique within the realm of diagnostic ultrasound that
allows improved characterization of tissue perfusion com-
pared to conventional color Doppler ultrasound imaging.
This technique involves intravenous administration of an
ultrasound contrast agent, which consists of gas-filled
Embryonic Pseudoglandular
phase phase
Weeks 3 to 7 Weeks 7 to 17
Fig. 5.1 Five stages of normal lung development
E. S. Bih et al.
­ icrobubbles surrounded by a stabilizing outer shell [6]. The
m
large acoustic mismatch between gas and surrounding tis-
sues enhances the vascularity of the imaged structures.
Appropriate software that is currently standard on most up-
to-date ­ultrasound units is used when performing CEUS in
order to minimize bubble destruction [6].
Recent publications have evaluated the efficacy of CEUS
in the characterization of peripheral lung lesions in adults [7,
8]. It has also been used in children to improve the evaluation
and management of complicated pneumonia [9]. CEUS may
prove to be a useful tool that potentially provides informa-
tion that cannot be obtained with conventional grayscale and
color Doppler imaging.
Normal Development and Anatomy
Normal Development
Normal antenatal lung development can be divided into five
stages (Fig. 5.1). Fetal lung development begins between 3
and 4 weeks of life as a respiratory diverticulum arising from
the ventral surface of the developing foregut. During this
embryonic stage, the trachea branches into the right and left
main bronchi and then eventually into the lobar airways by
7 weeks [10, 11]. As development progresses into the pseudo-
glandular stage (between 7 and 17 weeks), further branching
of the airways occurs.
In the canalicular stage (between 17 and 27 weeks), the
gas-exchanging portion of the lungs and airways begins to
form, as well as the differentiation of type I and type II pneu-
mocytes and the production of surfactant [11, 12]. As fetal
lung development extends into the saccular stage (between
27 and 38 weeks), airway branching approaches completion
and alveolarization begins [11].
During the saccular stage, there is lengthening and widen-
ing of the terminal (acinar) airways, which result in thinning
of the connective tissues between the airspaces and an increas-
ing surface area for gas exchange. The alveolar stage (between
38 weeks and late infancy) is the final stage and is character-
Canalicular Saccular Alveolar
phase phase phase
Weeks 17 to 27 Weeks 27 to 38 Weeks 38 to 3 years
AGE
5 Lung 175

ized by a dramatic increase in the number of alveoli, as well

a
as maturation of the alveolar architecture. This stage can per-
sist until early childhood (3 years of age) [10, 11]. R
Development of the pulmonary vasculature begins at R
R R
approximately the same gestational age, with the left sixth
aortic arch contributing to the formation of the main and left
pulmonary arteries and ductus arteriosus and the right sixth
aortic arch contributing to the formation of the right main
pulmonary artery [13].

Normal Anatomy

Due to large number of air-filled spaces and high acoustic

impedance difference between soft tissues and air, direct
ultrasound imaging of the lung is difficult as most of the
sound beam is reflected. Direct visualization of the lung
parenchyma is usually indicative of the presence of pathol-
ogy [3]. In the following section, ultrasound signs are dis-
cussed, some of which occur in normal lung and some of
which are associated with underlying pathology.
Fig. 5.2 Normal lung in a 2-week-old female. Longitudinal grayscale
ultrasound image shows pleural line (arrowheads) and A-lines (dashed
arrows). R, Ribs. From Cox M, Soudack M, Podberesky DJ, Epelman M.
Pediatric chest ultrasound: a practical approach. Pediatr Radiol. 2017;
47(9):1058–68. [14]

Ultrasound Signs

Four characteristic signs that should be recognized when

performing chest ultrasound include the pleural line, lung
sliding, A-lines and B-lines.

The Pleural Line

Under normal conditions, the parietal and visceral pleurae can

be visualized between the ribs as a single hyperechoic line
(Fig. 5.2). This thin echogenic line represents the acoustic inter-
face between the air-filled lung and the soft tissues of the chest
wall [14]. The thickness of this line is rarely measured in clinical
practice, and there is no consensus in the literature regarding
normal measurements [15]. Because of its typical appearance,
visualization of the pleural line is usually used as a normal land- * *
mark, and its absence indicates an abnormality in the pleural
space, for example, in the setting of a pleural effusion [4, 16].
* *
Lung Sliding

One important benefit of ultrasound is the capability of real-­

time dynamic examination. Lung sliding is a normal real-­
time ultrasound finding that results from the sliding motion of
the visceral pleura over the parietal pleura during ­respiration
[4, 16]. Using M-mode imaging (Fig. 5.3), ­normal lung slid-
ing results in a characteristic pattern termed the “seashore”
sign, which is manifested by the “granular/sandy” pattern in
the lung resulting from lung motion [14, 17].
Left LUNG BASE
Fig. 5.3 Normal M-mode ultrasound appearance of the lung. M-mode
tracing through an intercostal space in a 2-week-old female. The
echogenic line (arrowheads) is the pleural line. Because of lung
motion, the lung beneath the pleural line demonstrates a granular/
sandy pattern, also known as the “seashore” sign (asterisks). From
Cox M, Soudack M, Podberesky DJ, Epelman M. Pediatric chest
ultrasound: a practical approach. Pediatr Radiol. 2017;47(9):1058–
68. [14]
176
A-Lines
Due to the high acoustic impedance mismatch between the
pleural surface and the underlying normally aerated lung,
reverberation artifacts are produced distal to the pleural line.
These artifacts are collectively known as A-lines (Fig. 5.2).
A-lines are hyperechoic horizontal lines that are regularly
spaced below the pleural line [4, 16, 18, 19].
B-Lines
B-lines are defined as vertically oriented hyperechoic lines
that arise from the pleural line and extend to the bottom of
the image (Fig. 5.4). B-lines represent dynamic comet-tail or
reverberation artifacts that move with lung sliding. B-lines
are thought to occur as a result of irregularities at the lung-­
pleura interface. Three or fewer B-lines may normally be
seen between two ribs, particularly posteriorly [15, 20].
However, the presence of more than three B-lines per inter-
costal space occurs in the setting of numerous interstitial
Fig. 5.4 Pulmonary edema in a 19-day-old female. Transverse grayscale
ultrasound image shows multiple vertical echogenic B-lines (arrows)
E. S. Bih et al.
lung processes, such as pulmonary interstitial edema and
surfactant deficiency disease, and can be used as a marker for
an interstitial abnormality.
M-Mode Appearance of the Normal Lung
In normal lung, the soft tissues manifest as multiple parallel
echogenic lines due to their lack of motion. They are separated
from the lung tissue by the echogenic pleural line. Beneath the
pleural line, the lung parenchyma demonstrates a granular or
sandy appearance, which is the result of lung motion (Fig. 5.3).
Hepatization and Air Bronchograms
In the setting of pulmonary consolidation, affected alveoli
are filled with fluid and debris instead of air. There is loss of
reverberation artifact, and the consolidated lung parenchyma
manifests as an iso- to hypoechoic mass in the thoracic cav-
ity with heterogeneous echotexture that resembles the liver
parenchyma, hence the term “hepatization” (Fig. 5.5). If the
airways are patent, multiple foci of hyperechogenicity in a
linear branching pattern can be observed, i.e., ultrasound air
bronchograms (Fig. 5.6, Table 5.1) [17, 21].
* L
Fig. 5.5 Pneumonia in a 7-year-old female with sickle cell anemia.
Longitudinal grayscale ultrasound image of the right chest and upper
abdomen shows consolidated lung (asterisk) with heterogeneous
echotexture that resembles the liver (L) parenchyma, hence the term
“hepatization”
5 Lung 177

a b

Fig. 5.6 Pneumonia and pleural effusion in a 5-year-old male. (a) the right chest shows consolidated lung with air bronchograms (arrows)
Frontal chest radiograph shows a large right thoracic opacity with air and a pleural effusion (asterisk). (c) Transverse color Doppler ultra-
bronchograms (arrow). (b) Transverse grayscale ultrasound image of sound image shows hyperemia of the consolidated lung

Table 5.1 Normal and pathological signs in lung ultrasound

Signs Imaging findings
Normal
A-lines Hyperechoic horizontal lines below the pleural line caused by reverberation artifact
Lung sliding Dynamic sliding of the parietal and visceral pleurae against each other during respiration
Pathological
Absent lung sliding Accumulation of air and/or fluid in the pleural space that disrupts the parietal-visceral interface with
loss of lung sliding
B-lines (more than 3 per intercostal Vertically oriented hyperechoic lines that arise from the pleural line and extend to the bottom of the
space) image
Can be seen with interstitial processes
Hepatization with ultrasound air Loss of reverberation artifact in setting of consolidation. Lung has a heterogeneous echotexture
bronchograms resembling liver parenchyma
Patent airways demonstrate multiple hyperechoic foci in a linear branching pattern

Congenital Lung Anomalies
Congenital lung anomalies consist of a broad spectrum of
developmental disorders with varying clinical presentation,
prognosis, and management. Some anomalies are asymp-
tomatic and only discovered during chest imaging performed
for other indications. Some are large space-occupying lesions
that hinder normal pulmonary function and require prompt
intervention. The estimated annual incidence of congenital
lung anomalies is between 30 and 42 cases per 100,000 pop-
ulation [10, 22–26]. With the increasing availability of pre-
natal screening with ultrasound and MR imaging, many
178
congenital lung abnormalities can be detected in utero, per-
mitting comprehensive perinatal management [23]. Other
abnormalities typically present in infancy or early/late child-
hood with respiratory symptoms.
Congenital Lobar Hyperinflation
Congenital lobar hyperinflation (CLH), formerly known as
congenital lobar emphysema, is a disorder characterized by
progressive overinflation of one or more pulmonary lobes, as
a result of intrinsic or extrinsic airway narrowing [24]. The
a b
c d
Fig. 5.7 Congenital lobar hyperinflation, (a) Transverse grayscale
ultrasound image of a 28-week gestation fetus shows an echogenic lung
mass (arrows) in the left hemithorax. (b) Sagittal T2-weighted magnetic
resonance (MR) imaging of the lungs in a 29-week gestation fetus
shows a hyperintense mass (arrow) in the left upper lobe. (c) Coronal
E. S. Bih et al.
collapsed and narrowed airway acts as a one-way valve,
resulting in air trapping.
CLH has an incidence of 1 in 20,000 to 1 in 30,000 births
[27], with a male to female ratio of 3:2 [28]. It most frequently
affects the left upper lobe (43%), followed by the right mid-
dle lobe (32%) and the right upper lobe (20%) [28–30].
Depending on the degree of lobar overinflation, the clinical
presentation can range from asymptomatic to respiratory dis-
tress and other symptoms related to compression of the adja-
cent lung and mediastinal structures.
On prenatal ultrasound, CLH presents as a homogeneous,
hyperechoic mass-like lesion without macrocysts (Fig. 5.7) [24].
contrast-enhanced lung window CT image of a 1-month-old female
shows a hyperlucent left upper lobe (arrow). (d) Follow-up frontal chest
radiograph of the same patient as in (c) at 10 years of age shows persis-
tent hyperlucency of the left upper thorax
5 Lung
It may sometimes be hypervascular. Mass effect on adjacent
structures, such as deviation of the heart, can also be seen. On
fetal MR imaging, CLH presents as a mass with homogeneous
hyperintensity on T2-weighted images. On postnatal chest radio-
graphs and CT, the affected lobe is typically opaque due to
retained fluid. As the fluid is reabsorbed over time by the capillary
and lymphatic systems and replaced by air, the affected lobe
hyperinflates and appears hyperlucent on radiographs and CT
[24, 29, 30].
Foregut Duplication Cyst
Foregut duplication cysts arise from defective budding of the
ventral foregut that occurs during the fourth and fifth weeks of
gestation. Foregut duplication cysts include a spectrum of
developmental anomalies that include bronchogenic cysts,
esophageal/enteric duplication cysts, and neurenteric cysts
[31, 32]. Differentiating between these entities is difficult by
imaging alone and often requires histologic analysis [33–35].
Most bronchogenic cysts are located in the middle medias-
tinum, usually in a subcarinal location [30, 33, 34, 36]. Other
a b
Fig. 5.8 Foregut duplication cyst in a 1-month-old female. (a) Transverse
grayscale ultrasound image shows a cystic structure (white arrow) in the
mediastinum posterior to the heart (black arrow). (b) Transverse power
179
less common sites include paratracheal, hilar, and intraparen-
chymal locations. With increasing availability of prenatal
imaging evaluation, many of these duplication cysts are being
discovered during the fetal period.
Clinical presentation depends on the size and location of
the duplication cyst. If the lesion is small, the patient may be
asymptomatic and the lesion is discovered on imaging stud-
ies obtained for an unrelated indication. If the lesion is large,
causes mass effect, or has superimposed infection, the patient
may present with respiratory symptoms, chest pain, or dys-
phagia if there is compression of the esophagus [37].
A duplication cyst typically presents as a unilocular, well-
defined, avascular lesion (Figs. 5.8 and 5.9). Depending on its
contents, echogenicity may range from anechoic to hyper-
echoic. Internal septations, hemorrhage, and mural calcifica-
tion are occasionally seen. If infected, the cyst wall may be
thickened and demonstrate increased vascularity on color
Doppler evaluation. The presence of air within the lesion is
usually a sign of infection.
Definitive treatment is surgical resection in symptomatic
patients. Depending on the severity of the symptoms and
clinical status, percutaneous or transbronchial needle aspira-
Doppler ultrasound image shows the avascular cystic structure (arrow).
(c) Axial contrast-enhanced CT image depicts an oval hypodense lesion
(arrow) posterior to the heart with fluid attenuation
180
Fig. 5.9 Foregut duplication cyst in a 9-year-old female with dyspha-
gia. Coronal STIR MR image shows a cystic structure (arrow) near the
carina
tion may serve as a temporizing measure before definitive
surgical resection [33, 34, 37].
Congenital Pulmonary Airway Malformation
Congenital pulmonary airway malformation (CPAM), for-
merly known as congenital cystic adenomatoid malforma-
tion (CCAM), is a congenital hamartomatous lung lesion
that results from a failure of normal bronchoalveolar devel-
opment. There is proliferation of terminal respiratory units
in a gland-like pattern without normal alveolar formation.
CPAM is the most common congenital lung malformation,
accounting for 30–40% of all congenital lung anomalies, with
a reported incidence of 1:10,000 to 1:35,000 newborns [23,
24, 26]. The lesion may communicate with the proximal tra-
cheobronchial tree via a mal-­developed airway. The majority
of CPAMs receive their blood supply from the pulmonary
artery and drain into the pulmonary vein.
Pediatric patients with CPAM typically present early in
infancy with respiratory symptoms, or are detected prenatally
[10, 22–26]. CPAM can also present with recurrent infections
affecting the same lung zone from adolescence to early adult-
hood. Occasionally, CPAM is asymptomatic and incidentally
discovered when imaging is performed for other indications.
Classification of CPAM is based on cyst size and histology.
Five subtypes have been described based on the Stocker classifi-
cation (Table 5.2) [24]. Type 0, comprising approximately 1–3%
of CPAM, is characterized by a proximal bronchial anomaly and
acinar dysplasia involving all pulmonary lobes. Due to the sever-
ity and extent of this anomaly, type 0 CPAM is incompatible with
life. Type 1 has a frequency of 60–65% and is characterized by a
single or multiple cysts that are > 2 cm in diameter. Type 2 has a
frequency of 10–15% and is characterized by single or multiple
cysts that are ≥ 0.5 cm but ≤ 2 cm in diameter. Type 3 has a fre-
E. S. Bih et al.
Table 5.2 Classification of congenital pulmonary airway malfor-
mations
Type Features
0 Proximal bronchial anomaly and acinar dysplasia involving
all pulmonary lobes
Incompatible with life
1 A single or multiple cysts that are > 2 cm in diameter
2 A single or multiple cysts that are ≥ 0.5 cm in diameter
and ≤ 2 cm
3 Predominantly solid tissue with small cysts that are
< 0.5 cm in diameter
4 Large air-filled cysts that can be difficult to distinguish from
type 1 lesions
quency of 8% and is characterized by predominantly solid tissue
with small cysts that are < 0.5 cm in diameter. Type 4 has a fre-
quency of 10–15% and also demonstrates large air-filled cysts
that can be difficult to distinguish from type 1 [10, 22–26, 30].
On pre- or postnatal ultrasound, all CPAMs present as a
solid, echogenic, lung mass with or without variable-sized,
anechoic, avascular, cystic structures (Fig. 5.10). A type 3
CPAM can appear as a homogeneous, echogenic lung mass
as the cysts are microscopic and only identified by histology
[24]. On fetal MR imaging, CPAMs are generally more
hyperintense than normal lung on T2-weighted imaging
because they are filled with fluid. On postnatal MR imaging,
the cysts may contain fluid or air. Air-containing cysts are
typically hypointense on all MR imaging sequences [24].
On conventional chest radiography, a CPAM usually
appears as a soft tissue density lesion containing cystic struc-
tures with or without air-fluid levels. The CT appearance of
CPAMs correlates closely with their radiographic appearance,
with consolidation or multicystic masses containing fluid- or
air-filled cysts (Fig. 5.11), or cysts with air-fluid levels [30].
The outcome of CPAMs depends on the type and size of
the lesion, as well as other associated congenital abnormali-
ties. The definitive treatment for symptomatic CPAM is sur-
gical resection. As with infected foregut duplication cysts, an
infected CPAM can be temporarily managed with percutane-
ous needle aspiration until definitive surgical management is
carried out. In asymptomatic patients, management is con-
troversial. In a recent review, approximately one of every
four originally asymptomatic CPAM patients went on to
develop a variety of disorders [38], including recurrent infec-
tion, hemoptysis, and pneumothorax. The option of elective
surgical resection has been suggested [10, 22, 24, 26, 39].
Bronchopulmonary Sequestration
A bronchopulmonary sequestration is a congenital malfor-
mation of the lower respiratory tract characterized by non-
functioning, dysplastic lung parenchyma that does not
communicate with the tracheobronchial tree [23, 24]. It
accounts for up to 6.4% of all congenital lung malforma-
tions [26].
5 Lung
a b
Fig. 5.10 Congenital pulmonary airway malformation. (a) Longitudinal
grayscale ultrasound image of a 22-week gestation fetus shows a large
echogenic left lung mass (calipers) with a cystic component (arrow-
Fig. 5.11 Congenital pulmonary airway malformation (CPAM) in a
2-month-old male. Coronal lung window CT image shows a cystic mass
(arrow) pathologically confirmed as a CPAM following resection
In contrast to CPAM, a bronchopulmonary sequestration
receives its blood supply from a systemic artery such as the
aorta. Occasionally it may be supplied by branches of the celiac,
splenic, subclavian, intercostal, or even coronary arteries [40].
Bronchopulmonary sequestrations can be classified as intralo-
bar or extralobar based on the relationship of the abnormal lung
tissue to the pleura and its venous drainage [23, 24, 30, 41].
Intralobar and extralobar bronchopulmonary sequestra-
tions have very similar imaging features, and differentiating
between them often relies on the assessment of their venous
drainage and location. On ultrasound, both types of broncho-
181
head) at the apex. (b) Coronal T2-weighted MR image shows a large
hyperintense mass (arrow) of the left lung with an apical cystic compo-
nent (arrowhead)
pulmonary sequestration usually appear as a homogeneous,
echogenic mass that is oval or triangular in shape, most com-
monly located in the left hemithorax (Fig. 5.12). Identification
of a systemic feeding vessel on color Doppler imaging is
critical for diagnosis.
On chest radiography, a sequestration typically manifests
as a focal lung mass of soft tissue density. Contrast-enhanced
CT is highly helpful in evaluation, where the lesion typically
presents as a solid mass with heterogeneous enhancement.
The aberrant systemic arterial feeding vessel can usually be
localized, particularly with 3D reconstruction. Approximately
50% of the time, the venous drainage pathway can also be
identified [24, 25, 30, 35]. On MR imaging, a sequestration
appears as a homogeneous, hyperintense mass on T2-weighted
sequences [24].
It is important to recognize that the imaging presentation
of a pulmonary sequestration can be highly variable depend-
ing on the presence of associated complications such as
superimposed infection, and the coexistence of other con-
genital lung lesions, i.e., “hybrid” lesions, which are dis-
cussed later in this chapter.
I ntralobar Bronchopulmonary Sequestration
Approximately 75% of bronchopulmonary sequestrations
are intralobar [23, 41]. An intralobar bronchopulmonary
sequestration is typically embedded within and intimately
connected to normal lung parenchyma, and does not have its
own pleura. It is most commonly located in the left lower
lobe and drained via the ipsilateral pulmonary vein [10, 22,
24, 25]. Recurrent infection is the most common associated
complication [24].
182
Fig. 5.12 Bronchopulmonary sequestration. (a) Transverse gray-
scale ultrasound image of a 21-week gestation fetus shows a large
left-sided echogenic lung mass (arrow) with rightward displacement
of the heart (arrowhead). (b) Sagittal T2-weighted MR image shows
the left-sided lung mass (arrow). H, Heart. (c) Axial lung window CT
Spontaneous regression of an intralobar bronchopulmo-
nary sequestration has been observed in some cases and is
thought to occur as a result of progressive fibrosis of the dys-
plastic lung tissue [42]. The definitive treatment for a symp-
tomatic intralobar bronchopulmonary sequestration is
surgical resection. Due to the presence of a dominant feeding
artery, endovascular embolization has shown promising
results as a minimally invasive approach to management
[42–44]. In asymptomatic patients, both expectant manage-
ment and elective surgical resection have been recom-
E. S. Bih et al.
image of the same patient obtained at 4 months of age shows a solid
mass (arrow) in the left lower lobe. The mass has relatively decreased
in size compared to the prenatal images. (d) Coronal maximum
intensity projection reformatted CT image shows a systemic arterial
feeding vessel (arrow) arising from the celiac artery
mended. However, due to the high risk of recurrent infection,
surgery is often performed [10, 22, 24, 25].
 xtralobar Bronchopulmonary Sequestration
E
Approximately 25% of bronchopulmonary sequestrations
are extralobar, with a 4:1 male/female ratio [41, 45]. In con-
trast to an intralobar bronchopulmonary sequestration, an
extralobar bronchopulmonary sequestration is surrounded by
its own pleura and is usually drained via a systemic vein. It
occurs most often in the left lower lobe. Approximately
5 Lung
10–15% of extralobar bronchopulmonary sequestrations are
infra-diaphragmatic in location, usually in the suprarenal
region [45].
Extralobar bronchopulmonary sequestrations occasionally
present early in infancy with respiratory compromise and/or
infection. In rare cases, cardiac failure has been reported due
to extensive arteriovenous shunting. Approximately 50% of
extralobar bronchopulmonary sequestration are associated
with other congenital anomalies, including congenital dia-
phragmatic hernia, CPAM, congenital heart disease, foregut
duplication, and vertebral anomalies [45].
As with intralobar bronchopulmonary sequestration, the
definitive treatment for symptomatic extralobar broncho­
pulmonary sequestration is surgical resection, with endovas-
cular embolization as an alternative, less invasive approach.
Hybrid Congenital Lung Anomalies
A hybrid congenital lung anomaly is a malformation that dem-
onstrates features common to two or more congenital lung
anomalies, most often a bronchopulmonary sequestration with
an associated CPAM-like parenchymal malformation. A
hybrid lesion can be detected on prenatal imaging or present
postnatally with a variety of respiratory symptoms [25].
The imaging features of a hybrid lesion depend on its com-
position. A bronchopulmonary sequestration with CPAM typi-
cally presents as a multicystic lung mass with a systemic feeding
artery. These lesions are generally more readily detected by CT
than ultrasound. Overall, the diagnosis of a hybrid lesion can be
difficult on the basis of imaging alone, and frequently patho-
logical analysis is required [23, 25, 35, 41].
Atelectasis and Consolidation
Pulmonary atelectasis refers to incomplete expansion or
collapse of lung parenchyma. This phenomenon can be cat-
egorized as subsegmental, segmental, lobar, or complete.
Atelectasis can result from bronchial obstruction, adjacent
compression, or, particularly in neonates, surfactant defi-
ciency/dysfunction. Prolonged hypoventilation is also a
cause of pulmonary atelectasis, and this may occur in
patients with a history of recent surgery, chest wall defor-
mity, or neuromuscular disease [46–48].
The clinical presentation of atelectasis depends on the under-
lying cause and the degree of volume loss. A minor degree of
atelectasis may be asymptomatic, while significant atelectasis
can be associated with chest pain, shortness of breath, and
superimposed infection. A large zone of atelectasis may also
lead to hypoxemia due to abnormal gas exchange [49].
On ultrasound, pulmonary atelectasis appears as intratho-
racic solid, non-aerated, hypoechoic soft tissue. Its appear-
ance and echogenicity are similar to that of the liver, and it is
183
therefore also known as “lung hepatization” [4]. Echogenic
foci can be seen in a linear branching pattern in the collapsed
lung, which represent air in the bronchi, an ultrasound air
bronchogram [16, 50]. These findings can also be seen with
consolidation. Pulmonary consolidation refers to a process
where alveolar air is replaced by fluid. Pulmonary consolida-
tion can be due to a wide variety of causes, including infec-
tion, edema, and hemorrhage.
Differentiation between atelectasis and consolidation on
ultrasound is usually challenging, but the presence of moving
echogenic foci, the so-called “dynamic air bronchogram”, favors
the diagnosis of consolidation (Fig. 5.6) [4, 16, 18]. In contrast,
atelectasis is present when the air bronchogram is static [14, 21].
In neonates with atelectasis, collapsed lung causes vol-
ume loss, and the pleura is pulled inward. With consolida-
tion, there is no associated volume loss and therefore no
distortion of the pleural line [50]. In the setting of pneumo-
nia, air within the bronchi is replaced over time with fluid,
resulting in a hypoechoic branching pattern with relatively
echogenic walls.
Management of atelectasis varies according to the under-
lying cause. Atelectasis secondary to hypoventilation can be
treated with chest physiotherapy. Post-­obstructive atelectasis
is approached by alleviating bronchial obstruction. Neonates
with atelectasis associated with surfactant deficiency are
given surfactant. Pediatric patients with small foci of asymp-
tomatic atelectasis can be managed expectantly [48, 51].
Management of the many possible causes of consolida-
tion will also vary according to etiology.
Pulmonary Necrosis
Pulmonary necrosis can be caused by infection, autoim-
mune and vascular disorders, trauma, and malignancy [52,
53]. In some cases, air- or fluid-filled cavitary lesions can be
identified in the lung. Pulmonary necrosis and abscess for-
mation related to infection are discussed in an upcoming
section of this chapter. Vasculitis associated with autoim-
mune disorders such as granulomatosis with polyangiitis
can cause pulmonary necrosis. Pulmonary infarction related
to pulmonary embolism and chemotherapy for treatment of
lung cancer can eventually lead to lung necrosis and cavity
formation.
On ultrasound, pulmonary necrosis is identified as a
focus of decreased echogenicity within a zone of consoli-
dated lung. There is absent blood flow on Doppler evalua-
tion that can be further confirmed with CEUS. CEUS will
show a central unenhancing region surrounded by normally
enhanced lung parenchyma [9]. Pulmonary necrosis can
also be readily detected on contrast-enhanced CT and typi-
cally presents as a focal area of unenhancing tissue within
the consolidated lung [2, 54].
184
Pulmonary Abscess
Pulmonary abscess is defined as necrosis of the pulmonary
parenchyma due to microbial infection. Lung abscesses can
be categorized into acute or chronic types based on duration:
less than 6 weeks is considered acute and more than 6 weeks
is considered chronic [55]. An abscess can be primary or sec-
ondary. A primary lung abscess is due to aspiration of oro-
pharyngeal secretions. A secondary lung abscess occurs in
association with coexisting conditions, such as bronchial
obstruction, parenchymal disease, an immunocompromised
state, or malignancy [55].
a b
Fig. 5.13 Pulmonary abscess in a 12-year-old male with methicillin-­
resistant Staphylococcus aureus (MRSA) pneumonia. (a) Transverse
grayscale ultrasound image shows a fluid collection (white arrow) within
the consolidated lung (black arrow). (b) Transverse color Doppler ultra-
E. S. Bih et al.
The clinical features of a lung abscess include fever, pro-
ductive cough, and other respiratory symptoms. Systemic
manifestations such as night sweats and weight loss have
also been reported [55].
In general, ultrasound is more sensitive and useful in eval-
uating peripheral lung abscesses than those that are deeper
in location, since superficial aerated lung usually obscures
deeper structures. A lung abscess is seen as a hypoechoic
lesion with irregular outer margins surrounded by consoli-
dated lung. There may be complex internal contents such as
septations or echogenic foci due to gas [2, 10, 22, 54]. The
rim of the abscess may be hyperechoic (Fig. 5.13).
sound image shows the fluid collection with a mildly hyperechoic rim
and perilesional vascularity. (c) Axial contrast-enhanced CT image shows
a fluid collection in the lung with an air-fluid level (black arrow) consis-
tent with an abscess
5 Lung
Color Doppler imaging will show perilesional vascu-
larity, which is critical in differentiating a peripheral lung
abscess from an empyema. CEUS can be used to demon-
strate the lack of internal vascularity as well as associated sur-
rounding wall enhancement (Fig. 5.14). On conventional
radiography and CT, a lung abscess appears as a cavitary
lesion with thick, irregular walls. An air-fluid level may be
present [10, 54].
Empiric antibiotic therapy is usually the first line of treat-
ment for lung abscesses until a sputum culture and sensitiv-
ity are available for targeted therapy. Surgery is generally not
required unless the patient fails to respond to medical man-
agement or there are complications. Depending on the clini-
cal status of the patient and the location of the lesion, a lung
abscess may be amenable to percutaneous or endoscopic
drainage [55].
a b
d e
Fig. 5.14 Pulmonary abscess in a 3-year-old male with fever and tachy-
pnea. (a) Frontal chest radiograph shows a right lower lung opacity (black
arrow) with a focal internal lucency (white arrow). (b) Transverse gray-
scale ultrasound image of the right chest shows a heterogeneous hypoechoic
fluid collection (white arrow) with internal echogenic foci (arrowhead)
consistent with gas. There is consolidation of the surrounding lung paren-
chyma (black arrow). (c) Transverse color Doppler ultrasound image
185
Interstitial Lung Disease
Interstitial lung disease (ILD) in children comprises a large,
diverse group of disorders that involve the pulmonary paren-
chyma and interfere with gas exchange. Because the lung inter-
stitium is not always involved, the term ILD is gradually being
replaced by the term “diffuse lung disease,” or DLD. However,
the term childhood ILD (chILD) still appears in the current lit-
erature [56, 57]. Due to the broad spectrum of this diverse group
of disorders with overlapping clinical, radiological, and histo-
pathological manifestations, classification is challenging and
remains controversial. One of the commonly accepted schemes,
previously described by the ChILD Research Co-operative,
broadly categorizes the disorders into those that are more preva-
lent in infancy and those that are not specific to infancy [10, 57].
This classification is shown in Table 5.3.
c
demonstrates increased p­ arenchymal vascularity. (d) Grayscale (left) and
contrast-­enhanced (right) ­ultrasound images show enhancement of the
surrounding lung parenchyma (arrow) but not of the complex fluid collec-
tion (asterisk). (e) Axial contrast-enhanced CT image shows a gas-con-
taining fluid collection (arrow) in the right lower lobe consistent with a
pulmonary abscess
186
Table 5.3 Classification of interstitial lung disease in children
Category Specific diseases
Disorders more prevalent in infancy
Diffuse developmental Acinar dysplasia
disorders Congenital alveolar dysplasia
Alveolar capillary dysplasia with
misalignment of pulmonary veins
Growth abnormalities with Pulmonary hypoplasia
deficient alveolarization Chronic neonatal lung disease
Chromosomal disorders
Congenital heart disease
Surfactant dysfunction Surfactant protein B mutations
disorders Surfactant protein C mutations
ABCA3 mutations
Pulmonary alveolar proteinosis
Conditions of undefined Neuroendocrine cell hyperplasia
etiology of infancy
Pulmonary interstitial glycogenosis
Disorders not specific to infancy
Disorders of the Infection
immunocompetent host Environmental agents
Aspiration
Acute interstitial pneumonia
Nonspecific interstitial pneumonia
Eosinophilic pneumonia
Disorders of the Opportunistic infection
immunocompromised Related to therapeutic intervention
host (chemotherapy, radiation, drug
reaction)
Related to transplantation
Disorders associated with Immune-mediated
systemic disease processes Storage disease
Sarcoidosis
Langerhans cell histiocytosis
Malignant infiltrates
Disorders masquerading Arterial hypertensive vasculopathy
as interstitial lung disease Congestive vasculopathy and
veno-occlusive disease
Lymphatic disorders
Thromboembolic disease
Other than routine history and physical examination, diag-
nostic imaging, particularly high-resolution CT, plays a criti-
cal role in successfully diagnosing this diverse group of
disorders. The role of ultrasound is somewhat limited in evalu-
E. S. Bih et al.
ating DLD, as there is no specific sign for any disease entity.
Several publications have described the use of the B-line arti-
fact to evaluate interstitial lung disease [18, 58, 59].
As described earlier in this chapter, the B-line artifact is a
reverberation artifact that can be seen when the interstitium
is thickened or when fluid accumulates in the alveolar space.
It has low specificity for distinguishing between interstitial
and alveolar pathology. However, a recent study has sug-
gested that the number of B-lines detected can be used to
predict the presence of significant ILD in patients with sys-
temic sclerosis [60].
Respiratory distress is a frequent neonatal symptom.
Among the causes of perinatal dyspnea, surfactant defi-
ciency disease (SDD), formerly known as respiratory dis-
tress syndrome (RDS) or hyaline membrane disease, and
transient tachypnea of the newborn (TTN) are two of the
most common [61, 62]. SDD is primarily caused by defi-
ciency of surfactant in the immature lungs of preterm
infants. TTN is a lung disorder resulting from delayed
resorption and clearance of fetal alveolar fluid. Although
the pathophysiology of these two entities is multifacto-
rial, they do share similar clinical consequences, includ-
ing pulmonary edema and decreased pulmonary compliance.
As the fetal lung has high fluid content, B-lines can be
seen in neonates without respiratory symptoms [62]. Three
or fewer B-lines per intercostal space can be a normal find-
ing. In neonates with SDD, the B-lines appear more promi-
nent and compact. Coalesced B-lines result in an echogenic
“white-lung” appearance (Fig. 5.15). Combined with the
presence of a thickened and irregular pleural line and sub-
pleural lung consolidation, these ultrasound findings are sen-
sitive and specific for SDD [62–64].
The ultrasound appearance of TTN can be similar to SDD,
with compact B-lines and an echogenic “white-lung” appear-
ance (Fig. 5.16). However, in contrast to SDD, the pleural line
appears more regular, and the coalesced B-lines are more notice-
able in the inferior portions of the lung parenchyma, while in the
upper regions the B-lines are not compact [61–64].
5 Lung 187

a b

c d

Fig. 5.15 Surfactant deficiency disease and fluid overload in a 26-day-­ show no pleural effusion. However, the lungs demonstrate coalescent
old female. (a) Frontal chest radiograph shows a large opacity at the right B-lines (arrowheads) consistent with an interstitial process. L, Liver;
lung base, believed to represent a right pleural effusion. Longitudinal S, spleen. (d) Frontal chest radiograph obtained 16 hours later shows
grayscale ultrasound images of the right (b) and left (c) hemithoraces resolution of the previously noted right lung base opacity

a b

Fig. 5.16 Transient tachypnea of the newborn in a 1-day-old male. (a) edema. (b) Longitudinal grayscale ultrasound image of the left hemi-
Frontal chest radiograph shows cardiomegaly, indistinct pulmonary thorax shows coalescent B-lines (arrows), hallmarks of an interstitial
vascularity, and diffuse haziness consistent with interstitial pulmonary process
188
Pulmonary Lymphangiectasia
Congenital pulmonary lymphangiectasia (PL) is a develop-
mental disorder characterized by abnormal pulmonary lym-
phatic dilation. Congenital PL can be classified into two
types: primary and secondary [23, 65]. The cause of primary
PL is unknown, but a genetic and syndromic association with
a primary developmental defect has been postulated [65].
Secondary PL is usually due to underlying cardiovascular or
lymphatic obstruction related to entities such as hypoplastic
left heart syndrome, pulmonary vein atresia, congenital
mitral stenosis, and thoracic duct agenesis.
Historically, congenital PL has carried a grim prognosis,
with a mortality rate of 50–90% [65]. There are a few
reported cases of unilateral PL, which has a better prognosis.
With recent advancements in neonatal critical care, there are
improved respiratory symptoms and survival [65]. Severe
respiratory distress, cyanosis, and pleural effusions shortly
after birth are some of the common presenting features of
congenital PL. Chylothorax, chylopericardium, and chylous
ascites can also be seen.
Congenital PL is most commonly associated with pleural
effusion, which can be readily detected on prenatal ultra-
sound. Chylopericardium, chylous ascites, and generalized
edema can also be seen. On ultrasound, the findings may be
nonspecific, with a slightly heterogeneous appearance of the
pulmonary parenchyma (Fig. 5.17) [65]. On fetal MR imag-
a b
Fig. 5.17 Pulmonary lymphangiectasia in a 26-week gestation fetus.
(a) Transverse grayscale ultrasound image shows bilateral heteroge-
neously echogenic lungs (arrows). (b) Coronal T2-weighted MR image
E. S. Bih et al.
ing, the lungs have a heterogeneous, hyperintense appear-
ance on T2-weighted imaging, with linear, branching tubular
structures extending from the hila to the lung periphery, a
so-called “nutmeg” appearance [23, 66].
Postnatally, the presence of lung surface irregularity and
subpleural cystic-appearing structures has been suggested as
sensitive and specific ultrasound findings of secondary pul-
monary lymphangiectasia. It is hypothesized that the lung
surface irregularity is due to air-filled lung interposed between
dilated lymphatics while the subpleural cystic structures rep-
resent cystic dilation of subpleural lymphatic channels [67].
Pulmonary Masses
Pediatric pulmonary neoplasms are rare. Pulmonary masses
in children are approximately ten times more likely to rep-
resent a developmental abnormality or reactive lesion, such
as an infectious or inflammatory process, than a true neo-
plasm [68]. Of those lesions that are true neoplasms, the
ratio of primary tumor to metastatic tumor is approximately
1:5 [10, 68]. In children, primary malignant tumors are
more common than benign neoplasms, with a ratio of
approximately 3:1 [68].
Given the rarity and nonspecific presenting clinical and
radiological features, timely diagnosis of a primary lung
neoplasm in children is often challenging. In some cases, the
shows bilateral heterogeneously hyperintense lungs (arrows) with lin-
ear branching structures radiating from the hila to the periphery corre-
sponding to dilated lymphatic vessels
5 Lung
neoplasm is detected incidentally on imaging performed for
other reasons. In some cases, diagnosis is made only as a
result of persistent symptoms or a failure to respond to con-
ventional treatment.
Benign Pulmonary Masses
The most common primary benign pulmonary mass is a
hamartoma. The incidence of hamartoma is 0.025 to 0.032%
in adults and is thought to be even rarer in children [69].
Pulmonary hamartoma is a slow-growing, noninvasive
lesion that contains a varying amount of adipose tissue,
bone, cartilage, and smooth muscle bundles. It is often
asymptomatic and detected incidentally on imaging.
Most pulmonary hamartomas are located in the lung
periphery and can occasionally be detected with ultrasound
[69]. There are no specific ultrasound features. However,
since calcification may occur within the tumor, the pres-
ence of shadowing echogenic structures in the lung can
raise the suspicion for this diagnosis. On conventional chest
radiography and CT, a pulmonary hamartoma usually pres-
ents as a solid, round- to oval-shaped mass with lobulated
contours. Larger hamartomas are more likely to contain
calcification.
Approximately 60% of hamartomas have significant fat
deposits that are visible by CT [70]. On MR imaging, a pul-
monary hamartoma usually demonstrates intermediate sig-
nal intensity on T1-weighted imaging and high signal
intensity on T2-weighted imaging. The presence of fat and
calcification can be helpful in making the diagnosis. In chal-
lenging cases with ambiguous radiological features, trans-
thoracic needle biopsy can be performed for definitive
diagnosis [69].
Malignant Pulmonary Neoplasms
In children, metastatic lung neoplasms are more common
than primary malignancies. Several pulmonary neoplasms
have syndromic or familial associations.
Primary Malignant Pulmonary Neoplasms
Pleuropulmonary Blastoma
Among the primary pulmonary malignant neoplasms, pleu-
ropulmonary blastoma (PPB) is the most common in chil-
dren. It is relatively rare, with an estimated prevalence of 1 in
250,000 live births [71]. PPB is associated with germline
mutation in the DICER1 gene. It is estimated that more than
65% of children with PPB have a DICER1 mutation [71, 72].
PPB is primarily diagnosed prenatally, in infancy and in chil-
189
dren less than 6 years of age. It is rare in children older than
12 years of age [73].
PPB has been classified into three subtypes based on its com-
position: purely cystic (type I), mixed cystic and solid (type II),
and purely solid (type III) [72]. Most cases occur in the right
hemithorax. Ultrasound typically demonstrates a mixed solid-
cystic mass (Fig. 5.18) resembling a CPAM [72, 74–76]. In a type
III PPB or in tumors containing microcysts, the cysts may not be
visible and the tumor appears solid. In larger lesions, mass effect
and mediastinal shift may be evident.
The cystic-solid composition of PPB exists along a spec-
trum, with an increased solid component corresponding to
greater aggressivity of the tumor and a poorer prognosis. In
some cases, type I PPB can progress to type II or type III [72,
73, 75]. Depending on the type and size of the neoplasm,
management of PPB includes surgery, chemotherapy, or a
combination of the two. Radiation therapy has also been
used in some cases of types II and III PPB [73, 74].
Rhabdomyosarcoma
Although rhabdomyosarcoma (RMS) is the most common
soft tissue sarcoma in children, primary pulmonary rhabdo-
myosarcoma (PRMS) is a rare neoplasm and accounts for
only approximately 0.5% of all RMS [68].
Two theories have been postulated to explain the develop-
ment of primary PRMS. The first suggests an origin from hetero-
topic striated muscle within abnormal lung tissue such as a
bronchogenic cyst, CPAM, or bronchopulmonary sequestration.
This supports the claim that pulmonary malformations can serve
as a substrate for primary PRMS. It has also been suggested that
pleuropulmonary blastoma has the potential to evolve into a
high-grade sarcoma [77–80]. The second theory proposes that
primary PRMS develops from malignant transformation of
primitive mesenchymal cells.
Of all the subtypes of RMS, the embryonal and alveolar
subtypes are mainly seen in children. Common symptoms
associated with primary PRMS include cough, low-grade
fever, and dyspnea. Obstructive symptoms may be present
with an endobronchial lesion [77–81].
There are no specific ultrasound features for primary
PRMS. Secondary findings such as necrosis and cystic com-
ponents may be evident by ultrasound. Associated atelecta-
sis can also be identified if the lesion is endobronchial. CT
is the main imaging modality used for evaluation of primary
PRMS, which typically manifests as a soft tissue density
mass with heterogeneous enhancement on post-contrast
imaging. The alveolar subtype of RMS is usually large with
foci of necrosis [79, 82]. On MR imaging, RMS generally
demonstrates intermediate to low signal intensity on
T1-weighted images compared to muscle, moderate hyper-
intensity on T2-weighted images, and heterogeneous con-
trast enhancement [82].
190
a b
Fig. 5.18 Pleuropulmonary blastoma in a 4-year-old male with
DICER1 mutation. (a) Transverse grayscale ultrasound image shows a
mixed solid (asterisk) and cystic (arrow) mass in the left chest. (b)
Coronal contrast-enhanced CT image shows a large mixed solid-cystic
Management of primary PRMS depends on the histologic
subtype, tumor size, and location. Surgical excision is the
treatment of choice. Local control is achieved with radiation
therapy and systemic control with chemotherapy [79]. In gen-
eral, embryonal RMS has a better prognosis than alveolar
RMS, with 5-year survival rates of 70% and less than 10%,
respectively [78].
Metastases
Metastatic pulmonary neoplasms are five times more com-
mon than primary pulmonary malignancies in children [10,
68]. Wilms’ tumor and osteosarcoma are the two most com-
mon primary malignancies that result in pulmonary metasta-
ses in children. Approximately 10–15% of children with
osteosarcoma have pulmonary metastases at presentation
E. S. Bih et al.
mass (asterisk) with an air-filled cyst (arrow). (c) Axial proton density-­
weighted MR image shows a solid mass (black arrow) in the left lung
containing an air-filled cyst (white arrow)
[68]. Other less common primary malignancies that metasta-
size to the lung include neuroblastoma, hepatoblastoma, thy-
roid carcinoma, and soft tissue sarcomas. Pulmonary
metastases are typically asymptomatic and detected inciden-
tally or on staging evaluation.
Hematogenous metastases preferentially deposit in the
lung periphery and lower lobes [10, 22]. Lymphangitic metas-
tases such as those arising from thyroid carcinoma can cause a
miliary pattern in the lungs. Cavitation can sometimes occur in
lung metastases from Wilms’ tumor and osteosarcoma [68].
On ultrasound, pulmonary metastases usually present as
single or multiple well-circumscribed nodules or masses that
can be visualized if they are located in the periphery of the lungs
(Fig. 5.19). However, chest radiography and CT are the main
imaging modalities for evaluation of pulmonary metastases.
5 Lung
a b
Fig. 5.19 Metastatic Wilms’ tumor in an 8-year-old male. (a) Transverse grayscale ultrasound image shows a large heterogeneous and echogenic
mass (arrow) in the left chest. (b) Coronal contrast-enhanced CT image confirms the presence of the heterogeneous left chest mass (arrow)
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 Pleura
6
Nathan David P. Concepcion, Bernard F. Laya,
Ross A. Myers, and Edward Y. Lee

Abbreviations There are many advantages of ultrasound over the other

imaging modalities. It is safe, noninvasive, does not require
B Brightness ionizing radiation, is relatively inexpensive and readily avail-
CEUS Contrast-enhanced ultrasound able even at bedside due to its portability. Ultrasound allows
CPAM Congenital pulmonary airway malformation real-time visualization of imaged structures which is highly
CT Computed tomography useful for guidance in interventional procedures. Sedation is
FDG Fluorodeoxyglucose not generally required [1–7].
M Motion Ultrasound does have some disadvantages when evaluating
MR Magnetic resonance the pleura in the pediatric population. The bony thorax and air
PET Positron emission tomography within the lungs act as barriers, interfering with the transmis-
PBB Pleuropulmonary blastoma sion of ultrasound waves and limiting evaluation of deeper
RMS Rhabdomyosarcoma structures. Nonetheless, the artifacts produced by the aerated
VATS Video-assisted thoracoscopic surgery lung can provide important indirect clues about underlying
disease processes including the presence of pneumothorax [8,
9]. Another disadvantage is a small field of view.
Introduction Ultrasound plays a vital role in the evaluation of the
pleura. It can distinguish pleural effusion from parenchy-
A variety of disorders in children may affect the chest wall, mal pathology in a radiographically opaque hemithorax. It
including the pleura and the pleural cavity. These often can be used to estimate the volume of pleural fluid as well
require evaluation with several imaging techniques [1]. Plain as characterize the site, depth, presence or absence of sep-
radiographs remain the initial modality of choice for imag- tations and floating debris, nodules, and pleural thicken-
ing of the pediatric chest. However, because of concerns ing, thereby differentiating simple from complex effusions
about radiation exposure with increased awareness of the [10–12]. Other uses of ultrasound are in the differentia-
“Image Gently” campaign, and the low sensitivity and speci- tion between cystic and solid pleural masses. Color
ficity of radiography, as well as advances in ultrasound trans- Doppler imaging is useful in characterizing mass vascu-
ducer technology, the use of chest ultrasound in children has larity without intravenous contrast.
substantially increased [2]. In this chapter, up-to-date practical ultrasound imaging
techniques as well as fundamental embryology and normal
N. D. P. Concepcion (*) · B. F. Laya anatomy of the pleura are reviewed. In addition, the use of
Department of Radiology, St. Luke’s Medical Center ultrasound for common and rare but clinically important pleu-
College of Medicine - William H. Quasha Memorial, ral disorders in the pediatric population is discussed with
Quezon City, Philippines
emphasis on characteristic ultrasound imaging findings.
e-mail: npconcepcion@stlukes.com.ph
R. A. Myers
Department of Radiology, Lehigh Valley Reilly Children’s
Hospital, Allentown, PA, USA Technique
E. Y. Lee
Division of Thoracic Imaging, Department of Radiology, Infants and young children have relatively less subcutaneous fat
Boston Children’s Hospital and Harvard Medical School, when compared to adults, making ultrasound a first-line modal-
Boston, MA, USA ity for investigating the pleura and pleural cavity [13–15]. The

© Springer Nature Switzerland AG 2021 195

H. J. Paltiel, E. Y. Lee (eds.), Pediatric Ultrasound, https://doi.org/10.1007/978-3-030-56802-3_6
196
ribs and sternum of neonates and infants are only partially ossi-
fied, allowing trans-osseous scanning [16]. This acoustic win-
dow is not available in older children or adults where access is
limited mainly to the intercostal spaces.
Proper selection of technical parameters, patient position-
ing, and imaging approach customized to the clinical indica-
tion are essential for obtaining optimal diagnostic accuracy of
chest ultrasound in the pediatric population [17].
Patient Positioning
Depending on the clinical scenario, pediatric patients are
generally examined either supine, upright, or in a sitting
position. Ultrasound performed for the evaluation of pleural
disorders is usually accomplished with the patient in a supine
position [1]. The patient may be turned obliquely to the side
opposite the abnormality with the arm placed across the
chest to facilitate access and visualization of the posterior
aspect of the hemithorax [18]. In a recumbent patient, the
head of the bed can be elevated to at least 45° to optimally
evaluate the posterior lung recesses, in cases of posterior
pleural lesions and layering effusions.
Ultrasound Transducer Selection
Choosing the appropriate ultrasound transducer greatly affects
the accuracy of the examination [19]. The age of the patient
and location of the pleural lesion determine the type and fre-
quency of the transducer [2]. For pediatric patients, curved or
linear array transducers should be used [1].
High-frequency (7.5–18 MHz), linear transducers are
preferred in neonates and young infants [19–21]. These
transducers also allow superb spatial resolution of superficial
structures, particularly the chest wall and pleura, and for
detection of various abnormalities such as penumothorax.
Convex, micro-convex, or phased-­ array, low-frequency
transducers (1–6 MHz) are better suited for larger children
and for visualization of deep structures. These provide good
penetration for optimal visualization of effusions or to guide
interventional procedures [22].
Imaging Approaches
Each hemithorax can be divided into anterior, lateral, and poste-
rior zones by the anterior and posterior axillary lines. These
regions can be further subdivided into upper and lower portions
N. D. P. Concepcion et al.
Fig. 6.1 Ultrasound imaging approach to the chest. Each hemithorax can
be divided into anterior, lateral, and posterior zones by the anterior and
posterior axillary lines (solid lines). These zones are further subdivided
into upper and lower regions at the level of the nipples (dashed line)
at the level of the nipples (Fig. 6.1) [23, 24]. Several acoustic
windows are available for evaluation of the pleura and pleural
cavity. These include supraclavicular, suprasternal, parasternal,
intercostal, subcostal/subxiphoid, transdiaphragmatic, and
transabdominal approaches.
The unossified or partially ossified thoracic cage permits
parasternal or transcostal access for use in neonates and infants
[2, 6, 15, 16]. In older children, an intercostal or transabdomi-
nal approach is performed using a curved, convex transducer
with the liver and spleen serving as acoustic windows to the
lung bases. The anterior and lateral chest wall approaches are
useful in detecting pneumothoraces in recumbent patients
[25].
Real-time, grayscale or conventional brightness (B)-mode
ultrasound is the standard imaging tool for evaluation of the
pleura [1, 20], with clear depiction of the ribs, intercostal
spaces, and subpleural fat layer of the chest wall [26, 27].
Additional useful techniques includes motion (M)-mode and
color flow Doppler imaging. M-mode imaging can be used to
assess the sliding of the pleura for evaluation of pneumotho-
rax. Color Doppler imaging can differentiate vascular from
nonvascular lesions without the need for intravenous contrast.
Normal Development and Anatomy
Each lung is surrounded by the pleura. The pleura consists of
a visceral layer that envelops the lung and a parietal pleura
that lines the inner chest wall. The potential space between
these two layers is the pleural cavity (Fig. 6.2) [28, 29].
6 Pleura 197

a Laryngotracheal
Visceral Parietal tube
pleura pleura
Lung

Intercostal Pleural
muscles sac

Coelomic cavity

Diaphragm
Pleural Intercostal b
cavity muscles

Lung bud
Chest wall (rib cage,
sternum, thoracic vertebrae,
connective tissue, intercostal
muscles)
Coelomic cavity
Fig. 6.2 Diagram of the chest wall and pleura. There are two pleural
layers separated by the pleural cavity. The visceral layer covers the lung
and the parietal layer lines the inner chest wall
c
Parietal pleura

Normal Development

The pleura forms ahead of the lung during the third week of Visceral pleura
gestation [30, 31]. The lungs start to develop a week later
[32]. The lateral mesoderm forms the somatic and splanch-
nic mesoderm which eventually become the parietal and vis-
ceral pleura, respectively. The pleura is separated from the d Parietal pleura
pericardium and peritoneum at 9 weeks of gestation, and the
pleural cavity is complete within the third month of gestation
(Fig. 6.3) [33, 34].
Visceral pleura
Pleural cavity

Normal Anatomy

The pleura is a serous membrane consisting of five layers,

e
including an outer fibroelastic layer, a subpleural highly vas-
cularized loose connective tissue layer, a superficial elastic
tissue layer, a submesothelial loose connective tissue layer, Lung

and an inner mesothelial layer. The pleura is about 0.2– Thoracic

Root of lung
0.4 mm in thickness [1, 11]. The mesothelial layer is involved wall Visceral pleura
in both cellular and humoral immunity and plays an active Pleural cavity
role in pleural fluid production and reabsorption [35]. Parietal pleura
Diaphragm
As the pleura folds onto itself, it forms a cavity that contains
a small amount of fluid of up to about 18 ml. The fluid is evenly Costodiaphragmatic
recess
distributed throughout the pleural space, which decreases fric-
tion as the visceral pleura moves relative to the parietal pleura
and chest wall during respiration [36]. The parietal pleura has Fig. 6.3 Formation of the layers of the pleura and the pleural cavity. (a)
The coelomic cavity forms as a single cavity in the third week of gestation.
four parts, including the costal, cervical, mediastinal, and dia- During the embryonic phase of lung development in the fourth week of
phragmatic regions. A line of pleural reflection occurs where the gestation, (b) the lung bud divides into left and right bronchial branches and
parietal pleura changes direction as it passes from one wall of (c) further divides into the lobes and segments. (d) In the ninth week, the
the pleural cavity to another (Fig. 6.4). pleura separates from the pericardium and peritoneum. (e) Development of
the pleural cavity is complete in the third month of gestation
The blood supply of the visceral pleura is derived from
both the pulmonary and bronchial arteries but mainly from
the latter, with drainage via the pulmonary veins. The pari-
etal pleura is supplied by systemic arteries, including the
198 N. D. P. Concepcion et al.

Visceral
pleura
Pleural
Parietal reflection
pleura
Visceral
Pleural pleura
reflection
Inferior
Visceral pleura Parietal pulmonary
lining fissure pleura ligament
Costophrenic Pericardium
sinus
(diaphragmatic Costomediastinal
recess) sinus

a b c

Fig. 6.4 Diagram of pleural anatomy. The cavity between the visceral and
parietal pleura contains a small amount of evenly distributed fluid. (a, b)
The visceral pleura extends along the accessory lobes and fissures. The
pleural sinuses are potential spaces that are only completely filled during
inspiration and are partially collapsed during expiration. A line of pleural
reflection occurs where the parietal pleura changes direction as it passes
from one wall of the pleural cavity to another. (c) The inferior pulmonary
ligament a double fold of pleura that extends from the mediastinum to the
dome of the hemidiaphragm. It allows the hilar vessels sandwiched
between two layers of visceral pleura to expand during inspiration

Parietal
pleura-intercostal
artery
Pleural fluid
Visceral filtration
pleura-pulmonary
artery

Fig. 6.5 Diagram of pleural blood supply. Blood supply to the vis-
ceral pleura arises predominantly from the bronchial and pulmonary Mediastinal
arteries, while the parietal pleura is supplied by intercostal and lymphatics
phrenic arteries Costal
lymphatics

phrenic artery and intercostal arteries, and drained by the

phrenic and intercostal veins (Fig. 6.5).
The lymphatics of the parietal pleura communicate with
the pleural space via 8–10 μm holes [1] which exude fluid Diaphragmatic
lymphatics
into the diaphragmatic, mediastinal, and inferior costal spaces
(Fig. 6.6). This fluid drains into the mediastinal lymph nodes,
then into the thoracic duct, and ultimately into the systemic
venous system [1]. The visceral pleura plays a minor role in
the clearance of pleural fluid and does not appear to have
direct communication to the lymphatics. Fig. 6.6 Diagram illustrating pleural lymphatic drainage. The lym-
phatics of the parietal pleura exude fluid into the diaphragmatic, medi-
The nervous innervation of the pleura, particularly the astinal, and inferior costal spaces via micrometer-­sized stomata. This
parietal layer, includes somatic, sympathetic, and parasym- fluid first drains into the mediastinal lymph nodes, then into the thoracic
pathetic fibers, which are supplied via the intercostal and duct, and ultimately into the systemic venous system
phrenic nerves.
6 Pleura
Anatomic Variants
Interlobar fissures are visceral pleural depressions that
extend from the outer surface of the lung into its inner
and hilar regions and separate the lungs into lobes. The
obliquely oriented right major fissure separates the right
upper and middle lobes from the right lower lobe, while
the horizontally oriented minor fissure separates the
right upper and middle lobes. The left lung has an
obliquely oriented major fissure that separates the left
upper and lower lobes [37].
Variations of the interlobar fissural anatomy in the form of
incomplete or interrupted fissures do occur, even in healthy
individuals. Similarly, congenital fissures are occasionally
seen which form accessory lobes and segments. An interseg-
mental fissure in the medial basal segment, an azygos fissure
(Fig. 6.7), and a left minor fissure are all examples of acces-
sory fissures. In these cases, only the visceral pleura continues
along the accessory fissure (Fig. 6.4) [37–39].
There is generally no anatomical connection between the
right and left pleural cavities in humans. Congenital com-
munication between the pleural cavities is common in other
mammals such as swine and cows, but is more commonly
acquired in humans. However, one case of a congenital com-
mon pleural cavity in a 3-year-old male observed during sur-
gery for pectus excavatum has been reported [40].
Normal Ultrasound Anatomy
The skin, subcutaneous fat, pectoral and intercostal muscles
conduct sound waves, but the pleural surface as well as the air
a b
Fig. 6.7 Congenital fissures are occasionally seen which delineate
accessory lobes. (a) Frontal upright chest radiograph shows an azygos
fissure (arrows) and lobe. The nodular density in the right suprahilar
199
within the lung reflects these waves, which are seen as artifacts.
The ribs, when ossified, block the transmission of sound waves
causing a hyperechoic appearance of the superficial cortex with
shadowing posterior to the ribs.
The interpretation of thoracic ultrasound is based on a set
of imaging signs and associated ultrasound-related artifacts.
Thoracic ultrasound is highly sensitive and specific for sev-
eral normal and pathologic conditions such as pleural effu-
sion and pneumothorax. The diagnostic accuracy is between
90% and 100%.
The pleural line (Fig. 6.8) is an acoustic interface between
the soft tissues of the chest wall and the air-filled lung. The
space between the pleural line and the bottom edge of the
image is called “Merlin’s space”. Under difficult scanning
conditions (e.g., patient motion, thick subcutaneous fat), the
“bat” sign is a helpful feature for the recognition of the pleural
line: the reflections from the ribs mimic the wings of a bat,
while the back of its body is outlined by the parietal pleura.
The visceral pleura moves to and fro along the parietal
pleura with respiration when these layers are in apposition.
This motion is described as “lung sliding” [41]. A shimmer-
ing or twinkling effect has also been described as “ants
marching on a string”. Sliding is best observed at the lung
base and is imperceptible at the lung apex.
The pleural line also appears echogenic on M-mode ultra-
sound. The non-moving soft tissues of the chest wall are seen as
parallel, horizontal, echogenic lines between the transducer and
the pleural line. Placing the cursor over the pleura, the normal
lung sliding posterior to the pleural line results in a coarse or
grainy pattern resembling sand on a beach (the “seashore” sign)
(Fig. 6.9). A still image of this sign is considered equivalent to a
video clip of lung sliding on conventional B-mode imaging.
region represents the azygos vein (arrowhead). (b) Coronal lung win-
dow CT image demonstrates the same findings of an azygos fissure
(arrows) and an azygos vein (arrowhead) as in (a)
200
Fig. 6.8 Depiction of the pleural line and A-lines in a newborn male.
Longitudinal grayscale ultrasound image shows normal A lines (arrows)
which are echogenic, evenly spaced, horizontally oriented reverbera-
tion artifacts produced by the normal air-filled lung posterior and paral-
lel to the pleural line (solid arrowheads). The ovoid, hypoechoic structure
(open arrowhead) represents an unossified rib
Fig. 6.9 Example of the “seashore” sign. M-mode ultrasound image of
the right anterior chest in a female infant shows a grainy or sandy pat-
tern (asterisk) below the pleural line (arrowheads) resembling sand on a
beach. This appearance is equivalent to the lung sliding motion seen on
conventional B-mode imaging
N. D. P. Concepcion et al.
A-Lines
A-lines are echogenic, evenly spaced, horizontally oriented
reverberation artifacts produced by the normal air-filled lung
posterior and parallel to the pleural line (see Fig. 6.8). These
lines represent a substantial change in acoustic impedance at
the pleura-lung interface, seen on both dynamic and static
ultrasound images [42].
B-Lines
B-lines are vertically oriented artifacts originating from
interlobular septa. They move with lung sliding, arise from
the pleura and extend indefinitely in a vertical direction
while obscuring the normal A lines. They are believed to
represent subpleural thickened interlobular septa [42–44].
Sporadic (one or two) B-lines can be seen in healthy indi-
viduals, or in the normal neonatal lung due to its high fluid
content. However, three or more B-lines (Fig. 6.10) or a
confluence of B-lines is considered a nonspecific marker of
lung abnormality. These include interstitial changes such as
pulmonary edema, as well as infection, contusion, and atel-
ectasis [45].
Although nonspecific, the number of B-lines on ultra-
sound correlates with the extent of parenchymal changes
seen on computed tomography in children.
T-Lines
T-lines are also referred to as the “lung pulse” sign. The lung
pulse is described as the rhythmic movement of the pleura in
synchrony with cardiac activity. In M-mode, this occurs in
normal lung as a result of the transmission of cardiac pulsa-
tions to the pleural from the lung (Fig. 6.11).
Fig. 6.10 Demonstration of B-lines in a 13-month-old male with con-
gestive heart failure and a pleural effusion (asterisk). Longitudinal
grayscale ultrasound image shows multiple vertically oriented echo-
genic bands (arrows) in the right lung base radiating from the right
pleural line and caused by interstitial edema. L, Liver
6 Pleura 201

Fig. 6.11 T-lines in a 5-year-old female. M-mode ultrasound image

shows vertical lines (arrows) extending from the pleural line (arrow-
heads) to the bottom of the image that are synchronous with the cardiac
pulsations and caused by transmission of the pulsations through the
lung to the pleura

Z-Lines
Z-lines are a normal finding, consisting of echogenic
comet-tail artifacts that arise from and are perpendicular
to the pleural line. Unlike B-lines, Z-lines extend for only
a short distance (2–4 cm) and do not obscure the A-lines
(Fig. 6.12).

Pleural Effusion

Ultrasound is the primary imaging tool for pleural evalua-

tion, and is the worldwide standard of care [46–48]. One of
the most useful applications of ultrasound is when a child
presents with a radiographically opaque chest. Ultrasound
can distinguish pleural effusion from consolidation or
masses.
A pleural effusion is an abnormal accumulation of fluid
within the pleural cavity that results from an imbalance between
the rates of fluid production and absorption. Chest radiographs
have low sensitivity in the detection of small volumes of pleural
fluid, but ultrasound can show even less than 10 ml of pleural
fluid, especially when performed with the patient in an upright
position [49, 50].
Ultrasound can assess the nature of the fluid, including its
echogenicity and the presence of debris and/or septations. These
ultrasound capabilities help characterize the type of pleural effu-
sion, which can guide clinicians in choosing the best treatment
option for their patients. Ultrasound can also safely guide inter-
ventional pleural procedures since images are obtained in real
time, lowering not only complication rates but also healthcare
costs [51].
The most common cause of pleural effusion in children is
infection, followed by congestive heart failure and malig-
nancy. Other causes include renal disease and trauma. Viral
Fig. 6.12 Z-line in an 8-year-old male. An echogenic, comet-tail arti-
fact (arrow) is identified perpendicular to the pleural line (black arrow-
head). Compared to a B-line, it is short (2–4 cm) and does not obscure
the A-lines (white arrowheads)
infection is more common than bacterial infection. The inci-
dence of pleural effusion in children worldwide is unknown
due to limited data and variations related to study population
and location [52].
The clinical presentation of pleural effusion depends on
the underlying disease and the size and site of the fluid col-
lection. The most common symptom is shortness of breath
due to restricted diaphragmatic excursion. Pleuritic pain may
also occur which usually lessens as the effusion increases in
size.
Quantification of fluid volume is difficult to determine
for a variety of reasons: complex geometry of the thorax,
presence of secondary findings (e.g., atelectasis), and the
variable size of pediatric patients. Therefore, a qualitative
estimate of fluid volume (i.e., minimal, small, moderate, or
large) is adequate for most clinical decision-making situa-
tions [53–56].
202
Simple Pleural Effusion
A simple pleural effusion is a fluid collection within the
pleural cavity characterized as clear and free-flowing, usu-
ally serous or transudative in nature. It gravitates to the most
dependent portions of the pleural cavity, commonly in the
posterolateral costophrenic sulci in upright or semi-recum-
bent pediatric patients.
The typical ultrasound finding is homogeneously anechoic
fluid (Fig. 6.13). Minimal swirling internal echoes may occa-
sionally be observed. Color Doppler typically demonstrates
movement in a simple, free-flowing effusion. On M-mode,
motion of the lung within the effusion can be observed, with
the distance between the pleura and lung decreasing during
inspiration and increasing on expiration (“sinusoid” sign).
Small pleural effusions may be asymptomatic and can be
treated conservatively. However, for moderate to large pleural
effusions and/or in pediatric patients with respiratory compro-
mise, mediastinal shift, and underlying lung disease, ultra-
sound-guided thoracentesis can be both diagnostic and
therapeutic. Movement of fluid with change in position or with
respiration reflects amenability of the fluid to aspiration. A
minimum depth of 1.5 cm between the visceral and parietal
pleura has been recommended when performing this proce-
dure. Percutaneous thoracostomy tube placement can also be
performed with ultrasound guidance.
L
Fig. 6.13 Simple pleural effusion in a 3-year-old male with dengue
fever. Longi­tudinal grayscale ultrasound image shows a large, anechoic
pleural fluid collection (asterisk) in the right hemithorax. L, Liver
N. D. P. Concepcion et al.
Complex Pleural Effusion
A complex pleural effusion is usually an exudate contain-
ing internal echoes with or without septations. Swirling and
floating echoes (“plankton” sign) within the effusion may
represent agglomerations of protein, blood, and/or fibrin.
These can be seen in the setting of empyema, hemothorax,
or malignancy. Septations are adhesions or strands of fibrin
indicating loculation.
Although effusions can be anechoic, echoes or debris are usu-
ally visualized within the fluid on B-mode ultrasound (Fig. 6.14).
The “fluid color” sign is used to distinguish pleural fluid from
pleural thickening. With an effusion, color signal is seen in the
fluid during cardiac and respiratory movement but not with pleu-
ral thickening [57]. It has a reported sensitivity of 89% and a
specificity of 100%. Fibrinous stranding, septations (Fig. 6.15),
and loculations are more readily identified and characterized on
ultrasound than computed tomography (CT). Multiple locula-
tions demonstrate a “honeycomb” appearance (Fig. 6.16).
In pediatric patients with a complex effusion, therapeutic
drainage often requires temporary insertion of a catheter. Large-
bore chest tubes may be placed to drain viscid pus, although
recent evidence suggests that small-bore catheters with instilla-
tion of thrombolytics may be as effective and performed with
less discomfort [58]. The presence of echogenic or loculated
fluid on ultrasound is an indication for intrapleural fibrinolytics
LIVER
Fig. 6.14 Complex pleural effusion in a 14-year-old male with pneu-
monia. Longitudinal grayscale ultrasound image of the right hemitho-
rax shows echogenic debris within the pleural fluid (asterisk)
6 Pleura
S common etiologic agents are Streptococcus pneumoniae and
Fig. 6.15 Complex pleural effusion in a 2-year-old female with strepto-
coccal pneumonia. Longitudinal grayscale ultrasound image of the left
hemithorax reveals a pleural effusion (asterisk) with multiple septations
(arrowheads) and atelectatic lung (arrow). S, Spleen
Fig. 6.16 Empyema in a 7-year-old male with necrotizing pneumonia.
Initial chest radiograph revealed an opacified right hemithorax (not
shown). Longitudinal grayscale ultrasound image of the right chest
shows a multi-loculated fluid collection (asterisk) with echogenic
debris. Video-assisted thoracoscopic surgery revealed empyema. L, Liver
203
[44] such as tissue plasminogen activator and hydrolyzing
enzymes such as deoxyribonuclease. Video-assisted thoraco-
scopic surgery (VATS) is the preferred intervention to lyse sep-
tations and evacuate infected materials when chest tube drainage
has failed.
Parapneumonic Effusion
A parapneumonic effusion is a pleural fluid accumulation
that develops secondary to an underlying pulmonary infec-
tion. The fluid may be free-flowing or can develop into a
complex pleural collection known as an empyema. The most
Staphylococcus aureus. Parapneumonic effusions develop in
50–70% of patients with complicated pneumonia [59], and
are more common in infants and young children than in older
children.
The three stages of a parapneumonic effusion include
the exudative, fibrinopurulent (early and late phases), and
organizing stages [60, 61]. An exudative effusion is a clear,
viscous, sterile, free-flowing fluid. In the early fibrinopuru-
lent phase, fibrin accumulates and leads to septations and
loculations (Fig. 6.17). In the late fibrinopurulent phase,
pus accumulates in the pleural cavity. The organizing or
consolidation stage demonstrates a thick fibrous rind of
Fig. 6.17 Empyema in a 15-year-old male with Staphylococcus aureus-
positive pneumonia. Transverse grayscale ultrasound image of the right
pleural space demonstrates a complex, multi-­loculated collection (white
asterisk) with multiple thick internal septations and internal debris. The
right lung (black asterisk) is collapsed
204
pleura (“pleural peel”) around the complex pleural fluid
causing impaired respiratory function due to limited lung
re-expansion [60–62]. This also increases the risk for recur-
rent infection.
Empyema
An empyema is a pleural collection of pus and is often locu-
lated or septated. Pediatric patients with empyema usually
present with clinical manifestations of bacterial pneumonia,
including fever, pleuritic chest pain, cough, dyspnea, and
occasionally cyanosis. Abdominal pain and vomiting can
also occur [63].
Ultrasound findings of empyema typically include swirling,
echogenic fluid that often contains septations. Septations appear
as echogenic strands within the fluid collection (Figs. 6.16
and 6.17). Early septations are thin fibrin strands that float in the
pleural fluid. Mature septations are thick and largely composed
of collagen.
On CT, an empyema is a loculated, pleural-based collection
with enhancing, thickened, parietal and visceral walls (“split
pleura” sign) (Fig. 6.18). A bronchopleural fistula is suggested
when air is identified within a parapneumonic effusion or
empyema (Fig. 6.19) in the absence of any recent history of
intervention.
The presence of septations predicts the need for intrapleural
fibrinolytic therapy, longer duration of drainage, and possible
surgery. Surgical options include VATS or open thoracotomy.
Fig. 6.18 Empyema in a 10-month-old male with necrotizing pneumo-
nia. Axial contrast-enhanced CT image shows thickening and enhance-
ment of both pleural layers (arrowheads), the “split pleura” sign. Fluid
aspiration confirmed empyema
N. D. P. Concepcion et al.
Fig. 6.19 Bronchopleural fistula in a 9-month-old male with necrotiz-
ing pneumonia. Axial contrast-enhanced CT image shows air locules
(arrows) within the pleural fluid (asterisk) highly suggestive of bron-
chopleural fistula. Non-enhancing regions (arrowheads) in the lung rep-
resent necrosis
Bronchopleural fistula can be managed initially with a percuta-
neous thoracostomy tube. However, VATS or open thoracotomy
can be performed to surgically repair the fistula if chest tube
drainage fails.
Fibrothorax
Fibrothorax is fibrosis within the pleural space, with or with-
out calcification, and is an inflammatory response to a vari-
ety of pathological conditions, commonly from long-standing
empyema, tuberculosis, or hemothorax [64]. Fibrothorax is
characterized by relatively smooth pleural thickening, i.e., a
“pleural peel.” The visceral pleura is usually involved and is
responsible for restricted ventilation. However, the mediasti-
nal pleura is spared. Diffuse pleural thickening of >10 mm in
thickness may be associated with substantial lung functional
impairment and dyspnea [65].
On ultrasound, pleural fibrosis is initially hypoechoic and
hypovascular, becoming heterogeneous (Fig. 6.20) and vas-
cularized over time. Contrast-enhanced ultrasound (CEUS)
may be helpful to differentiate complex fluid from fibrosis.
Pleural calcifications may occur and are seen as hyperechoic
deposits with acoustic shadowing.
Lung decortication is performed if the pleural fluid is not
adequately drained despite thoracentesis, thoracostomy tube
drainage, and VATS, or if there is severe pleural fibrosis.
Decortication involves removal of the thickened layer from
the pleural surface [64–66].
6 Pleura 205

a b

Fig. 6.20 Pleural fibrosis in an 11-year-old female with primary pulmo- image reveals markedly thickened pleura (arrows) with multi-loculated
nary tuberculosis. (a) Longitudinal grayscale ultrasound image of the fluid in keeping with progressive tuberculosis, pleural dissemination and
right chest shows an echogenic pleural fluid collection (asterisk) with developing fibrothorax
pleural thickening (arrows). L, Liver. (b) Coronal contrast-enhanced CT

a b

L L

Fig. 6.21 Hemothorax in a 6-month-old male with heterotaxy and recent clot. (b) Transverse color Doppler ultrasound image of the left chest dem-
cardiac surgery. (a) Longitudinal grayscale ultrasound image of the left onstrates avascularity of the pleural collection. L, Liver
chest shows clumping of echogenic material (asterisk) representing blood

Traumatic Effusion nary infarction, and post-pericardiotomy syndrome can

Hemorrhagic Effusion
Hemothorax is the presence of blood within the pleural
cavity, which is usually related to blunt or penetrating
trauma. However, inflammation, malignancy, rupture of
bronchopulmonary sequestration or arteriovenous malfor-
mation, spontaneous intrathoracic vessel rupture, pulmo-
also present with hemothorax. Pleural fluid analysis shows
a hematocrit of more than 50% compared to that of periph-
eral blood [52, 56].
Ultrasound is a reliable tool for identifying hemothorax. An
acute blood collection is usually echo-free, whereas old blood
is echogenic, with clumping representing clots (Fig. 6.21).
Hemothorax may show increasing echogenicity toward the
206
dependent part of the effusion (the “hematocrit” sign) due to a
layering effect of the cells. Multiple thick septations can be
seen with long-­standing hemothorax, similar to empyema.
The affected child typically has a history of decreasing hemo-
globin and hematocrit levels [2, 11, 49].
Hemothorax is usually managed by ultrasound-guided
placement of a simple chest tube.
Extrapleural Hematoma
Extrapleural hematoma results from the accumulation of blood
in the extrapleural space between the parietal pleura and the
endothoracic fascia [67–71]. It is uncommon and usually occurs
in association with a rib fracture from injury to the intercostal
vessels or during traumatic insertion of a subclavian venous
catheter. There is no specific clinical symptom that identifies an
extrapleural hematoma, but affected pediatric patients may
present with chest pain and dyspnea. A large extrapleural hema-
toma can mimic a hemothorax [70–73].
Ultrasound is rarely used for the diagnosis of extrapleu-
ral hematoma. A pleural reflection may be identified at the
lower margin of the hematoma, but the costophrenic angle is
not obliterated. CT can show a displaced extrapleural fat
layer and parietal pleura (the “pleural lining” sign) which is
pathognomonic for extrapleural hematoma.
a b
Fig. 6.22 Chylothorax in a 13-month-old female with a congenital tho-
racic lymphatic malformation. (a) Longitudinal grayscale ultrasound
image of the left chest shows a large anechoic fluid collection (asterisk)
with layering debris (arrows). (b) Coronal T2-weighted, fat-suppressed
N. D. P. Concepcion et al.
Treatment of extrapleural hematoma depends on the clini-
cal status of the patient. A stable patient with a small hema-
toma can be managed conservatively. However, needle
aspiration or thoracotomy may be needed to evacuate blood
clots in a large extrapleural hematoma because of respiratory
and circulatory compromise.
Chylous Effusion
Chylous effusion or chylothorax is a rare cause of pleural
effusion in the pediatric population, although congenital
chylothorax is the most common cause of pleural effusion in
the first week of life [74–76]. It arises from the leakage of
chyle or lymphatic fluid into the pleural space as a result of
damage to the thoracic duct or its tributaries by rupture, lac-
eration, or compression [77–79].
Chylothorax may be congenital or acquired. Con­genital
chylothorax may be due to lymphatic anomalies (Fig. 6.22),
thoracic cavity defects, or other congenital malformations. It
is a rare occurrence, estimated to affect 1 in 10,000 births.
Acquired cases are commonly due to birth trauma or malig-
nant, post-surgical, or infectious causes such as tuberculosis or
filariasis. Lymphoma is the most common malignant, nontrau-
MR image demonstrates a multi-loculated, fluid-filled structure (arrow-
heads) that extends from the upper retroperitoneal space to the posterior
aspect of the left mediastinum adjacent to the thoracic aorta (arrow) in
keeping with a lymphatic malformation
6 Pleura
a b
Fig. 6.23 Chylothorax in a 7-year-old male with left subclavian vein
thrombosis. (a) Longitudinal grayscale ultrasound image of the left
chest shows a large pleural effusion (asterisk) with echogenic debris.
Extensive atelectasis of the lung (L) is seen inferiorly. (b) Aspiration
matic etiology, especially non-Hodgkin lymphoma. Iatrogenic
injury to the thoracic duct during cardiothoracic surgery is also
a common cause of chylothorax in the pediatric population. A
small chylothorax is usually asymptomatic, but dyspnea and
tachypnea may occur with larger chylous effusions.
Ultrasound and CT can demonstrate similar findings of a
simple pleural effusion of near water density, without or with
debris (Fig. 6.23) in pediatric patients with chylous effusion.
However, a chylous effusion may be echogenic in the setting
of a thoracic malignancy.
Chylothorax typically responds to conservative treatment
[80–82] since the thoracic duct leak closes spontaneously in
nearly 50% of patients. Initial fluid aspiration is performed
for diagnostic purposes. Continuous thoracostomy drainage
can be performed for recurrent fluid accumulation to main-
tain lung expansion. To reduce lymphatic fluid production,
treatment with total parenteral nutrition, a fat-restricted diet
of medium-chain triglycerides, and administration of soma-
tostatin or its analogue octreotide may be instituted [83, 84].
Surgical interventions including thoracic duct ligation, pleu-
ral abrasion, pleurodesis with doxycycline, thoracoscopic pari-
etal pleural clipping [85], or pleural-to-peritoneal shunts can be
performed if conservative treatment fails.
207
revealed milky fluid (asterisk) consistent with a chylothorax (Images
courtesy of Dr. Ricardo Restrepo, Nicklaus Children’s Hospital, Miami,
FL, USA)
Pneumothorax
Pneumothorax is an abnormal accumulation of air within the
pleural space. It can be primary and spontaneous or second-
ary to underlying pulmonary pathology, connective tissue
disease, or infection (Fig. 6.24) [86].
Pneumothorax is considered primary if there is no underly-
ing predisposing condition. The pathophysiology is believed
to be due to rupture of blebs or bullae. The highest incidence of
primary spontaneous pneumothorax in the pediatric population
is in tall, thin males 13–16 years of age [86–88].
In the neonatal or perinatal period, pneumothorax is com-
monly seen in patients with surfactant deficiency or meconium
aspiration syndrome. Both disorders result in alveolar rupture or
air leak. The overall incidence of pneumothorax according to
gestational maturity is 4%, 2.6%, and 6.7% in early preterm,
moderate-late preterm, and term neonates, respectively [89, 90].
Pneumothorax can be visualized on ultrasound along the
anterior chest wall of supine pediatric patients since air col-
lects in the non-dependent portions of the pleural cavity
[91–93]. Generally, the most common site is along the ante-
rior 2nd–4th intercostal spaces in the midclavicular line.
The pleural space should be explored from the sternum to
208 N. D. P. Concepcion et al.

Pneumothorax

Closed Open Tension

pneumothorax pneumothorax pneumothorax

a b c

Air in pleural space Air in pleural space Air in pleural space

increasing and unable to escape

Fig. 6.24 Pneumothorax can be classified according to the relationship pleural cavity and atmospheric pressures. (c) A tension pneumothorax
between pleural and atmospheric pressure. (a) A closed pneumothorax occurs when pleural cavity pressure is greater than atmospheric pressure
has an intact chest wall with pleural cavity pressure less than atmospheric resulting in contralateral cardiomediastinal shift
pressure. (b) An open pneumothorax has chest wall disruption with equal

Table 6.1 Chest ultrasound signs and imaging findings without and with pneumothorax
Without With
Ultrasound signs Imaging findings pneumothorax pneumothorax
Lung sliding Visceral pleura slides against parietal pleura during respiration Present Absent
Seashore sign Coarse or grainy pattern resembling sand on a beach on M-mode Present Absent
Stratosphere/ Uniformly parallel horizontal lines above and below the pleural line on Absent Present
Barcode sign M-mode
B-lines Vertically oriented echogenic lines that arise from the pleural line and extend May be present Absent
to the bottom of the image
Lung point Transition from normal lung sliding to absence of lung sliding Absent Present
Lung pulse Rhythmic movement of the pleura in synchrony with cardiac activity Present Absent

midclavicular line and from the clavicle to the anterior dia-
phragm. Comparison with the contralateral side may also be
helpful.
Ultrasound can diagnose a pneumothorax with a high degree
of accuracy [6]. It is more sensitive than chest radiography but
is similarly specific [94, 95]. However, quantification of pneu-
mothorax on ultrasound is difficult, and therefore ultrasound is
unlikely to completely replace the need for a chest radiograph.
The high accuracy of ultrasound is due to several reliable arti-
facts or signs which are discussed in the following sections and
summarized in Table 6.1.
Absence of Lung Sliding
Absence of lung sliding is diagnostic of a pneumothorax [96–
99], with a reported sensitivity of 95.3%, specificity of 91.1%,
and negative predictive value of 100% (p < 0.001) [7].
6 Pleura
Caution should be exercised, however, in making a diagnosis
of pneumothorax, since patients with bullae, hyperinflation, air
trapping, or previous pleurodesis can also demonstrate a similar
lack of lung sliding. Absence of lung sliding also occurs in sur-
factant deficiency, obese patients, in individuals with an acute
abdomen and reduced respiratory excursion, as well as lung
contusion and subcutaneous emphysema.
Stratosphere or Barcode Signs
In the presence of pneumothorax, a linear pattern on M-mode
ultrasound is noted both superficial and deep to the pleural
line (the “stratosphere” or “barcode” sign) with loss of the
normal granular “seashore” pattern below the pleural line
(Fig. 6.25). This is due to a sharp reflection from the strong
interface between the pleura and the pneumothorax resulting
in deeper reverberation artifact [98, 99].
Absence of B-Lines
B-lines exclude the presence of a pneumothorax. Conversely,
the absence of this artifact may be seen in the setting of a
pneumothorax [99].
Fig. 6.25 Pneumothorax. M-mode ultrasound image shows parallel
horizontal lines (white box) above and below the pleural line (arrows)
with loss of the normal granular pattern (the “barcode” sign). (Image
courtesy of Dr. Jovan Lovrenski, Institute for Children and Adolescents
Health Care of Vojvodina, University of Novi Sad, Serbia)
209
Lung Point
The “lung point” sign delineates the transition between normal
lung sliding and the presence of a pneumothorax (Fig. 6.26)
[99–101]. It is seen with smaller pneumothoraces as it relies on
a portion of the lung being in contact with the chest wall and is
therefore not observed with a large pneumothorax. This sign has
a low sensitivity but 100% specificity [102], and is therefore
pathognomonic for pneumothorax [49].
Absence of Lung Pulse
As previously described, a lung pulse is the vertical motion of
the pleura in synchrony with cardiac pulsations. Air in the
pleural cavity will prevent transmission of cardiac movement
through the lung to the parietal pleural interface. Therefore, if
a lung pulse is present, a pneumothorax can be excluded.
Absence of a lung pulse therefore contributes to the diagnosis
of a pneumothorax [103, 104].
There is substantial practice variation in the management of
pediatric pneumothorax [86]. Small- to medium-sized pneu-
mothoraces can be managed with a non-surgical approach that
includes observation, oxygen support, and simple aspiration.
Fig. 6.26 Pneumothorax. Longitudinal grayscale ultrasound image
shows a “lung point” sign (arrow) identifying the transition between
the normal lung (asterisk) and a pneumothorax (P). (Image courtesy
of Dr. Jovan Lovrenski, Institute for Children and Adolescents Health
Care of Vojvodina, University of Novi Sad, Serbia)
210 N. D. P. Concepcion et al.

A large pneumothorax requires insertion of a chest drain for
initial management [105, 106]. Surgical management usu-
ally involves resection of blebs/bullae and pleurodesis or
­pleurectomy. This is typically performed via VATS which is a
minimally invasive approach proven to be safe, effective, and
associated with low recurrence rates, decreased hospital stay,
decreased postoperative pain and morbidity compared to tradi-
tional open thoracotomy.
Pleural Masses
Pleural neoplasms in children are uncommon, and most are
malignant [107–109]. Chest wall lesions caused by tumors such
as rhabdomyosarcoma and metastatic neuroblastoma result in
palpable abnormalities that are usually associated with pain or
tenderness.
Ultrasound is the first-line screening modality in the
assessment of palpable, superficial thoracic chest wall masses.
High-resolution ultrasound imaging with color Doppler
allows differentiation between solid and cystic lesions and
assessment of tumor vascularity [110]. Cross-sectional imag-
ing, especially magnetic resonance (MR) imaging, can further
characterize the tumor and its extent and relationship to adja-
cent vital structures, which permits local staging [111].
Radiography has a limited role in the characterization of pleu-
ral masses.
Tumors originating from the lung but abutting the pleura
usually have acute angles with the chest wall (Fig. 6.27a) and
engulf the pulmonary vasculature. A pleural neoplasm shows
obtuse angles with the chest wall (Fig. 6.27b), tapered margins,
displacement of the pulmonary vasculature, lack of rib erosion,
and outward displacement of the extrapleural fat. Extrapleural
neoplasms displace the extrapleural fat inward [112].

a b

Fig. 6.27 Differentiation of juxtapleural lung tumors from tumors of pleural origin. (a) Lung tumor abutting the pleura forms acute angles with
the chest wall. (b) Pleural neoplasm forms obtuse angles with the chest wall
6 Pleura
Malignant Pleural Masses
Primary Neoplasms
Pleuropulmonary Blastoma
Primary malignant tumors of the pleura are less common than
metastatic tumors. Pleuropulmonary blastoma (PPB) is rare but
is the most common primary malignancy of the lung and pleura
in the pediatric population [113–117]. PPB represents about
0.25–0.50% of all primary lung malignancies [115, 118, 119].
PPB is the most common tumor associated with the DICER1
syndrome [119–125]. PPB is also discussed in Chap. 5.
DICER1 syndrome is a familial cancer predisposition
syndrome that arises from a mutation in the DICER1 gene
located on chromosome 14q32. Inheritance is autosomal
dominant with moderate penetrance. It is associated with an
increased risk of a variety of benign and malignant neo-
plasms largely occurring in early life, not only in the lungs
but also in the kidneys, ovaries, thyroid gland, and brain.
When PPB is diagnosed, monitoring of the patient for other
associated tumors is recommended [126].
There are three pathologic types of PPB: I, II, and III [122,
127]. Type I has a purely cystic appearance, while type III is
solid, although foci of necrosis may be present and mistaken
for cysts. Type II contains both cystic and solid components
[128]. An additional category, the I-regressed (Ir) type has also
been described. It is cystic with a microarchitecture similar to
type I lesions, with delicate septa and no malignant cells
[129]. A type I tumor can progress to a type II or III lesion
by approximately 36–75 months of age in up to 10% of
patients [115, 119, 129].
PPB occurs almost exclusively before 6 years of age.
Median age at diagnosis is 10, 34, and 44 months for type I,
II, and III tumors, respectively. PPB has a fairly equal male
to female distribution. It occurs slightly more often on the
right side (54%), but can also be bilateral (9%) [119, 127].
A type I tumor usually presents with mild to severe respi-
ratory distress or can be detected incidentally on a chest
radiograph. A type II or type III tumor can manifest with
nonspecific symptoms of dyspnea, fever, cough, chest or
abdominal pain, malaise, and/or anorexia which can mimic
pneumonia [130].
Distinguishing congenital lung masses such as cystic con-
genital pulmonary airway malformation (CPAM) from PPB can
be challenging [115, 122]. Type 1 CPAM and type 1 PPB are
indistinguishable at imaging [131]. Prenatal detection and the
presence of a systemic feeding vessel favor the more common
211
congenital masses. Cystic PPB can be identified prenatally as
early as the 31st week of gestation. Hence, lesions that were not
visible on the mid-second trimester ultrasound should be consid-
ered as tumor. Bilateral or multi-segmental involvement, cyst
complexity, spontaneous pneumothorax, progressive enlarge-
ment, family history, or observation of other DICER1 pheno-
types favors PPB.
PPB is a peripherally located mass in the lung, usually
large (greater than 10 cm) at presentation. Associated pleural
effusion and pneumothorax are frequently observed.
The imaging appearance of PPB varies according to
tumor type. PPB may initially appear on chest radiographs as
an air-filled cyst or cysts. Complete opacification of the
hemithorax with associated cardiomediastinal shift can occur
with a type III lesion [132]. Ultrasound of type I PPB may
demonstrate a cystic mass, while type II and III lesions may
show a heterogeneous mass with cystic and necrotic areas
(Fig. 6.28).
CT is the imaging modality of choice. Type I or Ir PPBs
show a thin-walled single or multi-locular, macrocystic mass
which may lead to lobar expansion masses. Type II masses
show air- or fluid-filled cavities with solid internal nodules.
Intralesional hemorrhage and air-fluid levels may be seen in
types I and II masses. Type III tumors are solid, low-­attenuating
neoplasms with heterogeneous enhancement after intravenous
contrast administration.
On MR imaging, these heterogeneously enhancing lesions
may present with liquefactive or hemorrhagic necrosis and
restricted diffusion. The solid components also show increased
fluorodeoxyglucose (FDG) uptake on positron emission tomog-
raphy (PET). The chest wall, including the sternum, may be
involved, although the presence of chest wall involvement
favors other more common metastatic malignancies such as
neuroblastoma over PPB.
Type I and Ir lesions require only surgery as the treatment
of choice. Five-year survival rate is about 91%. Types II and
III have a poorer prognosis with 5-year survival rates of 71%
and 53%, respectively, despite surgery and chemotherapy
[113, 115–117, 122]. Early diagnosis of type I PPB is neces-
sary to potentially avoid progression and fatal outcome
[133]. Metastasis has not been reported in type I PPB. For
type II and III tumors, metastases occur to the skeletal and
central nervous systems in approximately 30% of cases [115,
119, 128].
Adjuvant chemotherapy is recommended for incompletely
resected or ruptured tumors, because of the risk of recurrence
212 N. D. P. Concepcion et al.

a b

M M

HRT

c d

HRT

M M

St
Sp

Fig. 6.28 Pleuropulmonary blastoma type II in a 3-year-old male with
DICER1 germline mutation. (a) Transverse grayscale ultrasound image
of the left pleural space demonstrates a mixed cystic and solid mass (M)
containing cystic components (arrows) with low-level internal echoes
likely due to hemorrhage or necrosis. The partially visualized heart
(HRT) is displaced to the right. (b) Transverse color Doppler ultrasound
image of the left pleural space obtained from a plane slightly superior to
(a) shows vascularity within the mass (M) and an adjacent pleural effu-
sion (asterisk). (c) Axial contrast-enhanced CT image depicts the mixed
cystic and solid components of the mass (M) with small crescentic lucen-
cies (arrowheads) concerning for cavitation. The heart (HRT) is displaced
to the right. (d) Coronal contrast-enhanced CT image reveals compres-
sion of the left hemidiaphragm (arrowheads) by the mass (M), with infe-
rior displacement of the stomach (St) and spleen (Sp)

as a higher-grade lesion. Radiation therapy is rarely used [109,
116, 128].
Rhabdomyosarcoma
Rhabdomyosarcoma (RMS) is a high-grade malignant neo-
plasm that arises from the primitive embryonic, pluripotent
mesenchyme of virtually any organ except cortical bone. It
is the most common soft tissue sarcoma of childhood,
accounting for more than 60% of soft tissue sarcomas in
older children [134–136] and about 5% of all childhood
cancers [108–110, 137]. It is also the second most common
malignancy of the chest wall after Ewing sarcoma.
There are three types of rhabdomyosarcoma: embryonal,
alveolar, and pleomorphic. Most of the chest wall lesions are
of the alveolar subtype, typically occurring in adolescents,
with the worst prognosis. RMS can be seen in neonates and
is usually of the embryonal type. The single most important
prognostic factor is the presence of metastatic disease at the
time of diagnosis, regardless of subtype. RMS typically pres-
ents as a rapidly growing, firm, soft tissue mass that is usu-
ally painful due to nerve compression.
RMS can be well-defined or infiltrative, but typically does
not invade the adjacent bone except in advanced stages. The
lack of osseous involvement can differentiate RMS from
6 Pleura
a b
c d
Fig. 6.29 Rhabdomyosarcoma in a 4-year-old male with progressive
dyspnea. (a) Frontal upright chest radiograph demonstrates complete
opacification of the left hemithorax causing rightward cardiomediastinal
shift (arrowheads). (b) Longitudinal grayscale ultrasound image of the
left hemithorax shows a heterogeneous and lobulated soft tissue mass
(M) and a pleural effusion (asterisk). (c) Axial contrast-enhanced CT
other chest wall malignancies. On imaging, ultrasound can
show a heterogeneous, predominantly solid, hypoechoic
mass (Fig. 6.29) with varying degrees of internal vascularity
on color Doppler evaluation. Cystic or necrotic components
may be seen.
Cross-­sectional imaging is recommended for optimal
tumor evaluation and staging. CT can depict extension intra-
thoracic of the lesion as well as metastases to the lungs and
bones. MR imaging demonstrates intermediate T1 signal,
isointense to hyperintense T2 signal, and marked contrast
enhancement of the solid tumor components [138]. PET is
213
image confirms the presence of a lobulated, heterogeneously enhancing
soft tissue mass (asterisk) in the left posterior hemithorax with invasion of
the chest wall (arrowhead). (d) Sagittal contrast-enhanced CT image
shows p­ osterior extension of the mass (asterisk) with splaying of the adja-
cent ribs (arrows)
useful for staging and to assess initial response to chemother-
apy [139].
Treatment for RMS involves a multimodality approach.
Systemic chemotherapy includes vincristine, actinomycin D,
and cyclophosphamide. Surgery and radiotherapy are also
used to maximize local tumor control. Primary surgical
resection may be performed if it does not result in substantial
functional compromise, organ dysfunction, or disfigurement.
Radiotherapy is recommended for resected tumors with pos-
itive margins at surgery and for clinically or radiologically
suspicious lymph nodes.
214
a b
M
Fig. 6.30 Wilms’ tumor and extensive pleural metastases in an 8-year-­
old male. (a) Transverse grayscale ultrasound image demonstrates near-
complete filling of the left thoracic cavity and pleural space by a large
heterogeneous mass (M). There is a malignant pleural effusion (aster-
isk) and atelectasis (arrows) of the lung. (b) Coronal contrast-enhanced
Metastases
Metastatic pleural tumors are more common than primary
tumors. Metastatic spread to the pleura can be observed with
a number of primary tumors, commonly Wilms’ tumor, neu-
roblastoma, and osteogenic sarcoma. The possibility of met-
astatic disease should be considered in patients with a known
primary cancer who develop a pleural effusion [108, 140] or
pleural thickening [5, 60].
Ultrasound can show multiple, hypoechoic, ovoid- or
irregular-shaped nodules (Fig. 6.30) of various dimensions,
accompanied by large, echogenic effusions. Nodularity is
the most notable feature of a malignant pleural effusion
[141]. Metastatic nodules show obtuse angles with the tho-
racic wall, absence of the “lung sliding” sign, and disrup-
tion of the pleural line. MR imaging has superior soft tissue
contrast and therefore is most useful for both pleural effu-
sion and for pleural-based metastatic nodules [108, 140].
Management of pleural metastases depends on the underly-
ing primary neoplasm. For malignant pleural effusion, obser-
vation and monitoring of fluid volume are typically performed
for asymptomatic patients. In symptomatic patients, an
indwelling pleural catheter or chemical pleurodesis is recom-
mended [142, 143].
N. D. P. Concepcion et al.
L
CT image shows the mass (black asterisk) centered in the lower pleural
cavity and effacing the collapsed left lung (L). Additional heteroge-
neously enhancing masses (arrows) are seen along the parietal and vis-
ceral pleura with associated pleural effusion (white asterisk) and
rightward cardiomediastinal displacement
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 Mediastinum
7
Sumera Ali, Abbey J. Winant, Ricardo Restrepo,
Pedro Daltro, and Edward Y. Lee

Abbreviations Introduction

CT Computed tomography Ultrasound is an important, first-line imaging tool for evalua-

CEUS Contrast-enhanced ultrasound tion of the mediastinum in infants and young children, whose
EBV Epstein-Barr virus small size and non-ossified thoracic cage provide an optimal
FDCs Foregut duplication cysts acoustic window. The most common clinical indication for
GCTs Germ cell tumors pediatric mediastinal ultrasound is confirmation of a normal
GI Gastrointestinal but prominent or ectopic thymus in infants and young chil-
HL Hodgkin’s lymphoma dren. However, ultrasound can also visualize normal medias-
HRS Hodgkin/Reed-Sternberg tinal structures such as the trachea, esophagus, and great
IDRFs Image-defined risk factors vessels. It can detect and characterize mediastinal masses and
INRG International Neuroblastoma Risk Group their effect on adjacent vital structures. Ultrasound of medias-
ISSVA International Society for the Study of Vascular tinal masses can help narrow the differential diagnosis and
Anomalies guide further imaging.
ITMIG International Thymic Malignancy Interest Group In this chapter, up-to-date pediatric ultrasound imaging
LM Lymphatic malformation techniques and fundamental pediatric mediastinal embryol-
MIBG Meta-iodobenzylguanidine ogy and anatomy are reviewed. In addition, the use of ultra-
MR Magnetic resonance sound for both common and rare but clinically important
NHL Non-Hodgkin’s lymphoma pediatric mediastinal disorders is discussed with an empha-
SVC Superior vena cava sis on characteristic imaging findings. As a discussion of
TB Tuberculosis echocardiography and ultrasound imaging of the thoracic
great vessels is beyond the scope of this book, this chapter
focuses on nonvascular mediastinal disorders encountered in
S. Ali (*) infants and children.
Department of Radiology, Arkansas Children’s Hospital,
University of Arkansas for Medical Sciences,
Little Rock, AR, USA
e-mail: sali@uams.edu Technique
A. J. Winant
Department of Radiology, Boston Children’s Hospital Patient Positioning
and Harvard Medical School, Boston, MA, USA
R. Restrepo It is important to place a child comfortably in a supine posi-
Department of Interventional Radiology and Body Imaging, tion before initiating ultrasound evaluation of the mediasti-
Nicklaus Children’s Hospital, Miami, FL, USA num. A small pillow or roll placed underneath the neck can
P. Daltro help to improve transducer placement and visualization of the
Department of Radiology, Alta Excelência Diagnóstica/DASA and upper mediastinal structures. A visual distraction such as a
Clínica de Diagnóstico por Imagem/DASA, Rio de Janeiro, Brazil
television or video screen placed directly above the child’s
E. Y. Lee head may also help to extend the neck as well as to improve
Division of Thoracic Imaging, Department of Radiology,
Boston Children’s Hospital and Harvard Medical School, patient cooperation. In older children, a semi-upright position
Boston, MA, USA may be helpful. A wide area on the chest should be exposed

© Springer Nature Switzerland AG 2021 219

H. J. Paltiel, E. Y. Lee (eds.), Pediatric Ultrasound, https://doi.org/10.1007/978-3-030-56802-3_7
220 S. Ali et al.

since ultrasound evaluation of the mediastinum often requires visceral pleura. The mediastinum contains vital cardiovascular,
imaging from a variety of ultrasound windows. respiratory, lymphatic, and gastrointestinal structures.
During the fourth week of gestation, the primordial body
cavity, the intraembryonic coelom, separates into three cavi-
Ultrasound Transducer Selection ties: the pericardial cavity, peritoneal cavity, and paired peri-
cardioperitoneal canals (Fig. 7.1a). These cavities are lined
Depending on the clinical question and patient size, use of a by mesodermal mesothelium, which eventually forms the
variety of ultrasound transducers may be needed for diagnos- visceral pleural boundaries of the mediastinum. At 5 weeks
tic evaluation of the mediastinum. In neonates and small of gestation, a paired partition of pleuropericardial folds
infants, it is usual to initiate the examination with a high-­ develops that partially separates the pericardioperitoneal
resolution linear transducer on the order of 9–15 MHz. The canal and the pericardial cavity (Fig. 7.1b).
use of a high-frequency transducer has the advantage of opti- During the seventh week of gestation, the two pleuroperi-
mal resolution, while the limited depth of penetration is less cardial folds fuse in the midline and ventral to the esophagus
of a concern in smaller patients. In older children, the to create a primitive mediastinum. The pleuropericardial
increased depth of penetration of a sector transducer in the folds divide the thoracic cavity into a single pericardial cav-
2–7.5 MHz range or a lower frequency linear transducer on ity and two pleural cavities (Fig. 7.1c). The embryonic medi-
the order of 2–4 MHz may be helpful for visualizing the astinum contains a mass of mesenchyme that extends from
mediastinal structures. A sector transducer has a smaller the sternum anteriorly to the primitive vertebral column pos-
footprint and can be used for small acoustic windows, such teriorly. The embryonic mediastinum contains vital vascular,
as the suprasternal notch or an intercostal space. respiratory, and gastrointestinal structures, including the
aorta, esophagus, and bronchial buds.
Although the mediastinal structures each develop at
Imaging Approaches somewhat different stages of gestation, their growth is inter-
twined. For example, the trachea and esophagus develop
Standard approaches for ultrasound imaging of the pediatric from single embryonic structure, the anterior foregut tube.
mediastinum include (1) the suprasternal notch, (2) the right During the fourth to sixth weeks of gestation, a respiratory
and left parasternal windows, (3) the subxiphoid window, diverticulum (lung bud) arises from the ventral anterior fore-
and (4) the subcostal window. The suprasternal notch is a gut. The ventral respiratory diverticulum is gradually sepa-
useful window for visualization of the anterior mediastinal rated from the dorsal anterior foregut by the esophagotracheal
structures, including the thymus [1, 2]. The right and left septum.
parasternal views can visualize the anterior mediastinal and This process results in two separate mediastinal struc-
subcarinal structures [3]. The subxiphoid and subcostal win- tures, the trachea and the esophagus. The esophagus rapidly
dows are useful for evaluation of the pericardium and the elongates and completely obliterates its lumen by epithelial
detection of cardiophrenic angle masses. A lower-frequency proliferation around the fifth week of life and subsequently
curved transducer with a frequency range of 1–6 MHz allows recanalizes around the ninth week. The trachea bifurcates
for increased depth of penetration which is necessary for into two primary bronchi during the fourth week of life and
imaging of the paravertebral and posterior mediastinal struc- continues to branch until the sixth month of gestation.
tures and of any focal abnormalities. The thymus is a major lymphatic organ that plays an
important role in the development of the immune system,
specifically cellular immunity, by generating circulating T
Normal Development and Anatomy lymphocytes. The thymic primordium arises from the third
and fourth pharyngeal pouches during the sixth week of ges-
An understanding of the embryologic origins and develop- tation. It then elongates to become cylindrical in shape and
ment of normal pediatric mediastinal structures is important migrates caudally to the anterior mediastinum during the
when assessing the imaging appearance of pediatric ana- seventh to eighth weeks of gestation. Ectopic thymic tissue
tomic variants and pathologic processes. can occur anywhere along the path of descent, as discussed
later in this chapter.
Typically, a normal thymus is quite large in infants and
Normal Development young children. It reaches a maximum weight relative to body
mass before birth and continues to grow, attaining the greatest
The mediastinum extends craniocaudally from the thoracic inlet absolute weight at puberty. At puberty, the thymus begins to
to the diaphragm and is bound laterally by the left and right involute, with epithelial atrophy and fatty replacement.
7 Mediastinum
P P P P
F L
H PC
S H
a b
Fig. 7.1 Schematic illustration of the embryo at various stages of
mediastinal development. (a) Cross-section of the embryo at 24 days of
gestation shows the relationship between the paired pericardioperito-
neal canals (P) and the neural tube (N) dorsally, foregut (F) in the mid-
dle, and septum transversum (S) ventrally, which later forms the central
tendon of the diaphragm. (b) Cross-section of the embryo at 5 weeks of
gestation shows the connection between the pericardioperitoneal canals
Normal Anatomy
Compartment Approach
In the past, the mediastinum was divided into compartments
based on landmarks present on lateral chest radiographs. With
increasing clinical use of computed tomography (CT)and mag-
netic resonance (MR) imaging, cross-sectional imaging land-
marks have been found to be more accurate and useful than
radiographic landmarks.
In 2014, the International Thymic Malignancy Interest Group
(ITMIG) introduced a new, three-compartment classification of
the mediastinum based on cross-sectional imaging which has
been adopted as a new standard [4]. This mediastinal classifica-
tion helps to precisely localize lesions, allowing for more accurate
differential diagnoses, better preoperative planning, and improved
communication with multidisciplinary providers.
The three compartment model divides the mediastinum
into three spaces: prevascular (anterior), visceral (middle),
and paravertebral (posterior) compartments (Fig. 7.2). These
three compartments are bounded superiorly by the thoracic
inlet (defined as a ring of osseous structures that includes the
first thoracic vertebra posteriorly, the first set of thoracic ribs
laterally, and the superior border of the manubrium to anteri-
orly), and bounded inferiorly by the diaphragm.
The prevascular (anterior) compartment is demarcated by
the sternum anteriorly, the parietal pleura bilaterally, and the
anterior aspect of the pericardium posteriorly. The prevascular
compartment contains the thymus, mediastinal fat, lymph nodes
(thoracic lymph node station 3A), and the left brachiocephalic
vein.
221
A
E
V
BB
L PL
PF
PC
c
(P) and the pericardial cavity (PC), which is partially separated by the
pleuropericardial folds (PF). The bronchial buds (BB) and the primor-
dial heart (H) are also seen at this stage. (c) Cross-section of the embryo
at 6 to 7 weeks of gestation demonstrates the two expanded pleural
cavities (PL) and the pericardial cavity (PC), which is formed by central
fusion of the two pleuropericardial folds. The embryonic mediastinum
contains the aorta (A), esophagus (E), and vena cava (V)
The visceral (middle) compartment is delimited anteri-
orly by the posterior boundary of the prevascular compart-
ment and posteriorly by a vertical line along the thoracic
spine 1 cm posterior to the anterior spinal line. The contents
of the visceral compartment include the heart, most of the
great vessels (i.e., superior vena cava (SVC), ascending tho-
racic aorta, aortic arch, descending thoracic aorta, intraperi-
cardial pulmonary arteries), the thoracic duct, the trachea
and carina, the esophagus, and lymph nodes (thoracic lymph
node stations 2, 3P, and 4–8).
The paravertebral (posterior) compartment is bounded
anteriorly by the posterior margin of the visceral compart-
ment and posterolaterally by a vertical line extending along
the posterior aspect of the chest wall along the lateral mar-
gins of the transverse processes. The paravertebral compart-
ment includes the thoracic spine and paravertebral soft
tissues.
Accurate anatomic localization of a mediastinal mass
can occasionally be challenging, particularly when the
mass is large or occupies more than one compartment.
Localization of large and/or trans-compartmental masses
can be particularly difficult with ultrasound, which is lim-
ited in its ability to visualize the deeper aspects of large
masses in their entirety. In such cases, two tools described
by ITMIG may be helpful [4]: (1) the “center method,”
where the center of a lesion is localized to a particular
compartment, and (2) the “structure displacement” tool,
where a large mass is characterized by the structures it
displaces in the adjacent compartments. The structure dis-
placement method can be useful when evaluating a promi-
nent thymus, for example, as a normal thymus should
222 S. Ali et al.

Prevascular

Visceral

Paravertebral

Prevascular

Transverse
Vena cava aorta
Bifurcation Visceral
of trachea
Esophagus
Thoracic duct
Azygos vein

T4 Paravertebral

Prevascular

Visceral

Heart

Esophagus

Thoracic duct Aorta

Azygos vein
Paravertebral

T8
c

Fig. 7.2 Schematic illustration of the mediastinal compartments as devel-
oped by the International Thymic Malignancy Interest Group (ITMIG). (a)
Sagittal diagram depicts the three compartments in the sagittal plane which
include the prevascular (anterior), visceral (middle), and paravertebral
(posterior) compartments as defined by the dotted red lines. (b) Axial dia-
gram at the level of the aortic arch demonstrates the boundaries of the pre-
vascular (pink solid line), visceral (green solid line), and paravertebral
(orange solid line) compartments. (c) Axial diagram demonstrates the
mediastinal compartments and their constituents at the level of the heart
7 Mediastinum 223

never displace adjacent structures from the visceral (mid-

dle) compartment.

Thymus

The thymus is a soft, pliable, bilobed lymphatic organ located

in the prevascular (anterior) mediastinum. Each thymic lobe
is composed of numerous parenchymal lobules measuring
0.5–2 mm in diameter, with each lobule further divided into *
a cortex and medulla.
The thymus is typically easily evaluated with ultrasound
in infants and young children and is the imaging modality of
choice for further evaluation of a prominent thymic silhou-
ette in this age group. The thymus is located anterior to the
great vessels and extends inferiorly to the superior border of
the heart. On longitudinal views, the right thymic lobe has an
inverted teardrop shape, while the left lobe has a more trian-
gular or sickle shape.
Fig. 7.3 Cervical extension of the thymus in a 3-year-old male who
A prominent but otherwise normal thymus is a common presented with a neck mass. A. Longitudinal grayscale ultrasound
anatomic variant that should not be mistaken for pathology. image depicts the normal hypoechoic appearance of the thymus (arrow)
Occasionally a prominent thymus can extend superiorly into above the level of the clavicle (asterisk). Characteristic thymic paren-
the neck (Fig. 7.3) and inferiorly to the level of the diaphragm. chyma is seen with multiple branching linear echogenic strands and
foci
Ectopic but otherwise normal thymic tissue can also be found
in the middle and posterior mediastinum and can encircle the
left innominate vein [5]. Ectopic thymus can occur anywhere
along the normal path of decent from the angle of the mandi-
ble to the mediastinum due to arrest of descent or failure of
involution [6]. Prominent but normal thymus and ectopic thy-
mus have an appearance identical to normal thymic tissue on
all imaging modalities and do not exert mass effect on adja-
cent structures.
By ultrasound, a normal thymus has homogenous paren-
chyma. Sometimes described as having a “dot-dash” echotex- H
ture, normal thymic parenchyma is characterized by multiple
linear echogenic branching structures and foci representing the
connective tissue septa and septal blood vessels (Fig. 7.4) [7].
A normal thymus is soft and pliable. In particular, during
real-time ultrasound scanning, it changes in shape in response
to cardiac and respiratory motion, in contrast to a mediasti-
nal mass, which is generally more rigid. In rare cases, intra-
thyroidal ectopic thymus can present as a thyroid nodule and
is correctly identified by recognition of the typical dot-dash
parenchymal pattern (Fig. 7.5) [8]. Fig. 7.4 Normal thymus in a 6-month-old female. Longitudinal gray-
scale ultrasound image depicts the normal hypoechoic appearance of
the thymus. Multiple branching linear echogenic strands and foci
(arrows) are observed that give the thymus its characteristic appearance.
These echogenic foci represent the connective tissue septa and blood
vessels. H, Heart
224 S. Ali et al.

a b

Fig. 7.5 Ectopic intrathyroidal thymus in a 5-year-old male. (a) Longitudinal and (b) transverse grayscale ultrasound images show a left-sided thyroid
nodule (arrows) with multiple non-shadowing linear echogenic foci characteristic of the thymic parenchyma

a b

Fig. 7.6 Normal trachea in a 5-year-old male. (a) Transverse grayscale caused by the soft tissue-air interface. (b) Longitudinal grayscale ultra-
ultrasound image shows the inverted “U” appearance of the hypoechoic sound image reveals a “string of beads” appearance of the hypoechoic
tracheal cartilage (arrow). Posterior reverberation artifact (asterisk) is tracheal cartilages (arrow)

Trachea Ultrasound is useful in point-of-care evaluation of endo-

Although ultrasound is not typically used for evaluation of
the large airways, the trachea is often imaged during ultra-
sound evaluation of the pediatric mediastinum. Therefore, an
awareness of the normal ultrasound appearance of the tra-
chea is important.
The cervical and the upper thoracic portions of the tra-
chea are typically easily visualized via the suprasternal
notch. On ultrasound, the tracheal lumen is largely obscured
although the anterior tracheal wall has a characteristic
appearance of an inverted “U” on transverse imaging, and
the hypoechoic, cartilaginous rings resemble a string of
beads on sagittal views (Fig. 7.6). More posteriorly, rever-
beration artifact is caused by the soft tissue-air interface.
tracheal intubation to confirm appropriate positioning within
the trachea [9]. Accidental esophageal intubation is identi-
fied by the “double trachea” sign, where the intraesophageal
endotracheal tube creates a second air-mucosal interface,
simulating the appearance of a second trachea.
Esophagus
The esophagus is a tubular structure located posterolateral to
the trachea, usually on the left side. Best visualized via the
suprasternal notch, the esophagus is located behind the thy-
roid superiorly and extends inferiorly through the medias-
tinum (Fig. 7.7). The esophagus can be identified during
7 Mediastinum 225

real-time ultrasound evaluation by asking the patient to swal-
low and documenting normal esophageal peristalsis. One
important potential pitfall is to mistake the esophagus for an
abnormal parathyroid gland or thyroid nodule because of its
normal proximity to the thyroid gland.
Mediastinal Masses
Ultrasound is useful in the evaluation of mediastinal
masses for confirming the mediastinal origin of a mass
(and not an extension of a primary pulmonary or pleural
lesion); localizing a mass to an anatomic mediastinal com-
partment which aids in arriving at a correct diagnosis; and
characterization of lesions based on their ultrasound
appearance, including identification of solid and/or cystic
components and detection of echogenic fat and/or shadow-
ing calcification. Proximity to the normal mediastinal ana-
tomic structures can also help narrow the differential
diagnosis (Table 7.1).
Fig. 7.7 Normal esophagus in a 6-year-old female. Transverse gray-
scale ultrasound image shows the esophagus (arrow) at the level of the
upper tracheal rings. Peristaltic movement of the esophagus can be
observed by asking the patient to swallow
Prevascular (Anterior) Mediastinal Masses
The prevascular (anterior) mediastinum is the most common
location for a mediastinal mass in children. The majority of
pediatric anterior mediastinal masses arise from the thymus
or the prevascular lymph nodes. Ultrasound is useful for the
detection and diagnosis of the three most common anterior
mediastinal masses: teratoma, lymphoma, and lymphatic
malformation [10].
Teratoma
Germ cell tumors (GCTs) are believed to arise from pri-
mordial germ cells that fail to complete normal migration.
The mediastinum is the third most common location for
extragonadal GCT in the pediatric population after sacro-
coccygeal and intracranial GCTs [11]. Mediastinal germ
cell tumors include mature teratoma, immature teratoma,
germinoma, and non-germinoma.
Mature teratomas are commonly benign in the mediasti-
num, in contrast to mature teratomas of the testes, which
have malignant potential. Mature teratomas arise from at
least two of the three germ layers (ectoderm, mesoderm,
and endoderm) and contain well-differentiated cells. They
are slow-growing tumors, often incidentally noted or with
an indolent onset of symptoms.
Mature teratomas are heterogenous masses with varying
combinations of soft tissue, fluid, fat, and calcium. On
ultrasound, teratomas appear as well-circumscribed masses.
Soft tissue components are the most common feature
(100%), followed by fluid (88%), fat (76%), and calcium
(53%) [12]. Echogenic fat and/or shadowing calcification
may obscure the deeper aspects of the teratoma, limiting
visualization. It is difficult to determine whether a GCT is
benign or has malignant components based solely on its
ultrasound appearance. Malignant potential is typically
determined on histological evaluation and by the presence
of metastases (Fig. 7.8).

Table 7.1 Differential diagnosis of pediatric mediastinal masses based on location and components
Predominantly solid Predominantly cystic
Prevascular (anterior) mediastinal Ectopic thymus Thymic cyst
masses Thymoma or thymic malignancy Pericardial cyst
Lymphoma Lymphatic malformation (with macrocystic
Germ cell tumor component)
NUT carcinoma
Visceral (middle) mediastinal masses Infectious lymphadenopathy Foregut duplication cysts
Neoplastic lymphadenopathy  Bronchogenic cyst
Sarcoidosis  Esophageal duplication cyst
Castleman disease Lymphatic malformation
Paravertebral (posterior) mediastinal Sympathetic chain ganglion origin tumors Foregut duplication cyst
masses  Neuroblastoma Neurenteric cyst
 Ganglioneuroblastoma Lymphatic malformation
 Ganglioneuroma
Nerve root origin tumors
Extramedullary hematopoiesis
226
a b
*
Fig. 7.8 Malignant teratoma with pleural metastasis in a 2-year-old
female who presented with respiratory distress. (a) Transverse gray-
scale ultrasound image through the mediastinum demonstrates a large
heterogeneous, predominantly hyperechoic mass (white asterisk). It is
associated with a large, complex right pleural effusion (black asterisk)
Mature teratoma is treated with surgical resection and is
almost always curative [13]. Complete resection may not
be feasible when vital structures are involved, and in this
situation, subtotal resection to relieve compression is per-
formed. Primary malignant germ cell tumors of the medias-
tinum are generally first treated with neoadjuvant
chemotherapy to decrease tumor bulk followed by delayed
radical resection [14].
Lymphoma
Lymphoma is the third most common malignancy of child-
hood, comprising 12–15% of all childhood tumors [15]. The
two main histologic subtypes of lymphoma, Hodgkin lym-
phoma (HL) and non-Hodgkin lymphoma (NHL), vary sub-
stantially in their epidemiological and clinical presentation.
HL is most common childhood cancer in the 15–19-year
age group and is rare in infants and young children [15]. On
pathology, HL is characterized by the presence of clonal
Hodgkin/Reed-Sternberg (HRS) cells, which are large, mul-
tinucleated lymphocytes. HL is often associated with
Epstein-Barr virus (EBV) infection, and when present, EBV
can be detected in HRS cells.
Up to 65% of pediatric patients with HL will have a visi-
ble mediastinal mass on chest radiography at the time of pre-
sentation [16]. These bulky mediastinal masses may result in
symptoms due to mass effect, such as dysphagia, dyspnea, or
SVC syndrome. The majority of affected patients present
with painless lymphadenopathy, frequently cervical or supra-
clavicular in location.
In contrast, NHL is more common in a younger age group.
The median age of pediatric NHL onset is approximately
S. Ali et al.
*
*
and atelectatic lung (arrow). (b) Axial contrast-­enhanced computed
tomography (CT) image identifies a solid mass with calcification and
necrosis (arrow) in the prevascular space with extension into the adja-
cent anterior pleura and chest wall (white asterisk). Black aster-
isk, Pleural effusion
10 years of age, with a slight male predilection. NHL is the
fourth most common pediatric malignancy in children under
15 years of age [15]. The usual subtypes of NHL in the devel-
oped world include Burkitt lymphoma, diffuse large B-cell
lymphoma, lymphoblastic T-cell or B-cell lymphoma, and ana-
plastic large cell lymphoma. Children with underlying immu-
nodeficiency are at an increased risk for developing NHL.
Clinical presentation of pediatric NHL varies substantially
depending on the subtype and the anatomic region involved.
Masses caused by NHL can evolve rapidly (e.g., as quickly as
1–3 weeks), and affected children may present with acute
symptoms. If NHL involves the mediastinum, it may present
in a fashion similar to HL, with symptoms due to mass effect
on mediastinal structures, including airway obstruction, SVC
syndrome, and pericardial tamponade. Most often, NHL pres-
ents with painless lymphadenopathy. Burkitt lymphoma, a
unique NHL subtype, usually involves the abdomen.
On ultrasound, both HL and NHL lymphoma may present
as a bulky mediastinal soft tissue mass, or as a conglomeration
of enlarged mediastinal lymph nodes, often with mass effect
on adjacent structures, including narrowing of vessels.
Lymphomas are most frequently hypoechoic compared to the
adjacent mediastinal fat, although they can appear iso- or even
hyperechoic (Fig. 7.9).
Lymphoma can also involve the thymus; in these cases, the
thymus typically demonstrates a more nodular appearance,
often with internal cystic change. After treatment, the echo-
genicity of treated lymphomatous tissue may increase until it
is difficult to delineate from the surrounding soft tissues.
Lymphoma can also calcify after treatment and demonstrate
prominent posterior acoustic shadowing.
7 Mediastinum
a b
*
Fig. 7.9 Hodgkin lymphoma in a 9-year-old female. (a) Longitudinal
grayscale ultrasound image shows a hypoechoic mass (arrow) in the pre-
vascular space anterior to the pulmonary trunk (P) and aorta (A). (b)
Sagittal contrast-enhanced CT image (rotated counterclockwise by 90
The treatment of HL is based on risk stratification, as
determined by disease stage and the presence or absence of
bulky disease [17]. In children, bulky disease is defined as a
lymph node mass ≥6 cm which differs from the definition of
bulky disease in adults. Children with low-risk disease are
usually treated with chemotherapy followed by low-dose
field radiation. Intermediate-to-high-risk patients with HL
receive more intensive chemotherapy and field ­radiation.
Combination chemotherapy is the primary mode of treat-
ment for NHL. The treatment regimen and outcomes differ
based on the histologic subtype and disease stage. Radiation is
not commonly used in children as studies have shown that it
does not improve outcomes in early stage disease or when
given as prophylaxis for central nervous system disease [18].
Lymphatic Malformation
Lymphatic malformation (LM) is a slow flow vascular anom-
aly thought to arise from abnormal budding of the lymphatic
system or failure to connect with the venous system during
embryogenesis. Based on the International Society for the
Study of Vascular Anomalies (ISSVA) classification for vas-
cular anomalies, LMs can be classified into three types: mac-
227
*
degrees) depicts the lymphomatous mass (asterisk) in the same anatomic
plane as (a). (Images courtesy of Dr. Markus Renno, Arkansas Children’s
Hospital, University of Arkansas for Medical Sciences, AR, USA)
rocystic, microcystic, and mixed. LMs typically follow the
lymphatic system and may occur anywhere in the body,
although they are most frequent in the head and neck, axilla,
mediastinum, groin, and retroperitoneum.
Primary mediastinal LM is rare. However, mediastinal
extension of head and neck LMs may occur, most often
into the prevascular space (Fig. 7.10). Approximately half
of all mediastinal LMs (57%) may require airway man-
agement if they involve the major airways. If affected
patients have airway compromise at the time of diagnosis
of mediastinal LM, they are also at higher risk for respira-
tory complications following sclerotherapy, frequently
requiring ventilatory support [19]. Treatment of mediasti-
nal LMs is complex and requires a multidisciplinary
approach, best managed at a dedicated vascular anomalies
center.
Regardless of their anatomic location, LMs vary greatly
in size and appearance at ultrasound, ranging from small,
well-circumscribed lesions to large, infiltrative masses with
poorly defined margins. Lesions may or may not contain vis-
ible cysts, depending on the size of the abnormal lymphatic
channels. Discrete cysts measuring more than 1–2 cm are
228
a b
*
*
Fig. 7.10 Mixed macro- and microcystic lymphatic malformation in a
1-week-old male with a prenatal diagnosis of a neck mass. (a) Transverse
grayscale ultrasound image of the neck demonstrates a heterogenous,
multiloculated mass containing both cystic spaces (asterisks) and solid-­
appearing soft tissue. (b) Transverse grayscale ultrasound image through
the upper mediastinum reveals the inferior extent of the lymphatic mal-
considered to be macrocysts and show multiple thin-walled
rims and septa with minimal enhancement [20].
When purely macrocystic, an LM is usually anechoic
and compressible and has thin septations that often contain
small arteries and veins. If an LM becomes infected or
develops internal hemorrhage, the cystic spaces may
become non-compressible with internal debris (pus or
blood products) and fluid-fluid levels, reflecting sedimen-
tation of hemorrhagic or purulent debris. Thickening,
mural irregularity, and septations can also be seen in the
S. Ali et al.
*
*
T *
*
HRT
formation (asterisks) in the prevascular space surrounding the thymus
(T). HRT, Heart. (c) Axial T2-weighted, fat-suppressed MR image of the
mediastinum shows the malformation (arrowheads) extending from the
subcutaneous tissues through the mediastinum and into the left paraver-
tebral compartment
setting of superimposed bleeding or infection. Peripheral
hyperemia on Doppler evaluation suggests infection.
While ultrasound is useful for LM diagnosis, treatment
planning requires further evaluation with CT or MR imag-
ing to determine the full extent of the lesion and its rela-
tionship to critical structures.
Depending on the particular lesion, surgical therapy may
be an option. However, complete surgical excision is often
not possible due to the extensive and infiltrative nature of
many LMs. Sclerotherapy is useful in treating the macrocys-
7 Mediastinum
tic components of a lesion. Medical therapy with sirolimus
has recently shown promise in treating microcystic LMs,
demonstrating both clinical and radiologic improvement in
some patients [20].
Visceral (Middle) Mediastinal Masses
Foregut duplication cysts (FDCs) and lymphadenopathy are
the two most common causes of a visceral (middle) medias-
tinal mass in children [10]. These lesions are sometimes
amenable to ultrasound evaluation in infants and young
children.
 oregut Duplication Cysts
F differentiate from bronchogenic cysts. An esophageal
FDCs are developmental malformations that are classified
pathologically into bronchogenic, enteric, and neurenteric
cysts. Histologically, most foregut duplication cysts are
lined by respiratory epithelium or bronchial glands and less
commonly contain gastrointestinal mucosa or a combina-
tion thereof. Most children with FDCs are asymptomatic,
although patients can present with cough, dyspnea, or dys-
phagia due to mass effect [21].
Bronchogenic cysts are usually located in the visceral
(middle) compartment, and are typically subcarinal, para-
tracheal, or hilar in location. Occasionally, they may be
located within the lungs, most often in the medial lower
lobes. A mediastinal bronchogenic cyst is not typically
a b
Fig. 7.11 Bronchogenic cyst in an 11-year-old male who presented with
chest pain and fever. (a) Transverse grayscale ultrasound image demon-
strates a round, cystic lesion (calipers) in the right visceral and paraver-
tebral spaces containing a moderate amount of layering debris (arrow).
229
connected to the adjacent airway, and the presence of
internal air should raise concern for superimposed infec-
tion. The majority of cysts are asymptomatic; however,
they may cause symptoms if they compress adjacent
structures.
On ultrasound, a bronchogenic cyst appears as a solitary,
round or ovoid mass with smooth borders (Fig. 7.11). The
wall of the cyst may demonstrate echogenic foci with shad-
owing indicating rim calcification. Bronchogenic cysts dem-
onstrate variable echogenicity on ultrasound depending on
the presence of proteinaceous contents. Bronchogenic cysts
demonstrate no internal vascularity on color Doppler
evaluation.
Esophageal duplication cysts are rare and difficult to
duplication cyst occurs in close proximity to the esopha-
gus and may have an intramural origin. It is most com-
monly located along the inferior third of the esophagus,
usually on the right side. Esophageal duplication cysts
may contain gastric mucosa and, consequently, can bleed
and ulcerate. On ultrasound, they usually appear as a
spherical, unilocular cyst with a thin wall, close to the
esophagus (Fig. 7.12). The contents of an esophageal
duplication cyst are variable in echogenicity depending
on their composition.
Neurenteric cysts, also known as spinal enteric cysts,
result from a failure of separation of the primitive neural
crest from the ventral endoderm during embryonic devel-
H, Heart. (b) Axial contrast-enhanced CT image shows a well-cir-
cumscribed, homogenous, fluid-attenuation mass (asterisk) in the right
hilar region and immediately posterior to the left atrium. There is a
small right pleural effusion (arrow)
230 S. Ali et al.

a b

T *

Fig. 7.12 Esophageal duplication cyst in a 1-month-old infant. (a)
Transverse grayscale ultrasound image through the upper mediastinum
demonstrates an oval, anechoic cystic lesion in the left paraesophageal
space with a small amount of layering debris (arrowhead). Gut signa-
ture of the wall is noted, with a hypoechoic muscular layer (arrow)
between the echogenic mucosa and the serosa. T, Trachea; E, esopha-
gus. (b) Axial contrast-enhanced CT image shows a well-­circumscribed,
homogenous, fluid-attenuation esophageal cyst (asterisk) that displaces
the trachea (arrow) to the right

a b

T2

Fig. 7.13 Neurenteric cyst in a 2-week-old female with respiratory contrast-enhanced CT image depicts the well-­circumscribed, homoge-
distress. (a) A large, hypoechoic midline cystic lesion is noted anterior nous, fluid-attenuation mass (asterisk)
to the T2 vertebral body containing internal debris (arrow). (b) Axial

opment. Mediastinal neurenteric cysts occur most often in the thoracic spine. On ultrasound, a neurenteric cyst appears
the paravertebral compartment and may communicate with anechoic to hypoechoic compared to the surrounding soft
the spinal canal. They are sometimes associated with verte- tissues (Fig. 7.13). Associated osseous anomalies may be
bral abnormalities, most often anterior fusion anomalies of identified on prenatal ultrasound studies.
7 Mediastinum
Ultrasound is limited in its ability to detect intraspinal
extension of the lesion, and cross-sectional imaging, par-
ticularly MR imaging, is typically required for a complete
assessment of the lesion.
Current management for any foregut duplication cyst is
complete surgical resection. Expectant management is not
advised as symptomatic patients have a higher rate of
­intraoperative complications [22]. Superior mediastinal cysts
may share a wall with the trachea with a higher risk of air-
way injury [23].
Lymphadenopathy
Mediastinal lymphadenopathy can result from infectious,
inflammatory, or neoplastic processes. There are no strict cri-
teria for normal lymph node size in children, in part because
nodal size varies with age. One study has suggested that a
normal lymph node on CT can measure up to 7 mm in infants
and young children (≤10 years of age,) and up to 10 mm in
older children (>10 years of age), although these criteria are
not universally applied [24].
Ultrasound allows visualization and measurement of lymph
node size as well as evaluation of internal architecture and per-
fusion [25]. Abnormal lymph nodes are typically hypoechoic
when compared to the thymus and mediastinal fat and rela-
tively hyperechoic compared to adjacent vessels. Depending
on the underlying pathology, they can also demonstrate loss of
normal internal architecture with non-visualization of a nor-
mal echogenic hilum.
Infectious Lymphadenopathy
Bacterial Lymphadenopathy
Bacterial pulmonary infections are relatively common in chil-
dren, and bacterial pneumonia often leads to enlargement of the
regional mediastinal lymph nodes. Typical bacterial respiratory
pathogens in children younger than 5 years of age include
Streptococcus pneumoniae, Staphylococcus aureus, and S. pyo-
genes. In school-age children older than 5 years of age, the most
common bacterial respiratory pathogens are S. pneumoniae,
Mycoplasma pneumoniae, and Chlamydia pneumoniae.
Although imaging is not routinely performed, it may be
indicated in children with severe or recurrent pneumonia and
to assess for complications. Mediastinal lymph nodes often
enlarge as part of the immune response to bacterial pneumo-
nia, although the organism has not necessarily infected the
node itself.
On ultrasound, a mediastinal lymph node reactive to bac-
terial infection is generally increased in size, often with an
elliptical or ovoid shape. The long axis remains larger than
the short axis, typically with a ratio of >2. The nodal cortex
may thicken and become more hypoechoic, especially when
compared to adjacent muscle. However, the normal echo-
genic fatty hilum is typically preserved, and color Doppler
231
evaluation demonstrates central hilar vascularity with
branching vessels extending into the adjacent nodal paren-
chyma [25].
Mycobacterium Tuberculosis Lymphadenopathy
According to the World Health Organization, tuberculosis
(TB) ranks among the top ten causes of death worldwide
[26]. There were an estimated 10.0 million new cases of TB
in 2018, 11% of which affected children under 15 years of
age. Clinical diagnosis of TB in children is challenging, as
symptoms can be nonspecific and tuberculin skin testing also
has limitations.
Mediastinal lymphadenopathy is common in pediatric
patients with tuberculous infection and is usually multifocal.
It may be obscured on chest radiography due to a large thy-
mus and variability in the size of affected lymph nodes.
However, it can often be detected with ultrasound.
Ultrasound has been proposed as an adjunct to chest
radiographs for evaluation of mediastinal lymphadenopathy
due to TB in children, especially in resource-poor settings,
which are common endemic areas for TB [2]. One study
found that ultrasound was able to detect mediastinal lymph-
adenopathy in 67% of children with pulmonary TB who had
normal chest radiographs [27]. Ultrasound has also been
effective in documenting the decrease in size and number of
mediastinal lymph nodes during treatment [28].
When using ultrasound for evaluation of tuberculous
mediastinal lymphadenopathy, a four-view approach has
been described for ease of communication and standardiza-
tion [29]. Two orthogonal views are obtained through the
suprasternal notch and two views through the left parasternal
intercostal space using the thymus as an acoustic window.
When obtaining transverse images from a suprasternal
approach, anatomic landmarks include the right and left bra-
chiocephalic veins, SVC, aorta, pulmonary trunk, and thy-
mus. In the oblique suprasternal view, anatomic landmarks
include the left brachiocephalic vein, aortic arch and its
branches, and the thymus.
On ultrasound, enlarged tuberculous lymph nodes are
hypoechoic and round or oval in shape (Fig. 7.14). Enlarged
nodes may be solitary or resemble a mass with multiple matted
or conglomerate nodes. They may develop central necrosis
with posterior acoustic enhancement. On color Doppler imag-
ing, the vascular pattern is variable but commonly manifests as
preserved but displaced hilar vascularity. The central hilar ves-
sels may be absent in nodes with central necrotizing, granulo-
matous inflammation.
Tuberculous nodes often demonstrate capsular hyperemia
or inflammatory change in the surrounding soft tissues (i.e.,
periadenitis). Nodal calcification is not seen in acute primary
TB infection (the most common TB infection in children).
However, echogenic shadowing calcifications may develop
during treatment or with recurrent infection.
232
E self-limited. However, any severe infection or disseminated
*
Fig. 7.14 Mediastinal lymphadenopathy in a 2-year-old male with
tuberculosis. Longitudinal grayscale ultrasound image shows a soft tis-
sue mass with central areas of low echogenicity due to necrosis (aster-
isk) and focal calcification (arrow). E, Pleural effusion
Tuberculosis in children is treated with a short multidrug
regimen. Treatment should be initiated promptly as TB has
the potential to disseminate quickly and cause complica-
tions. The duration of the therapy depends on whether the
patient has been previously treated and if the lungs are
involved. New cases of pulmonary TB are treated with an
aggressive four-drug combination for 2 months followed by
a two-drug combination for 4 months. Imaging is not recom-
mended during treatment or follow-up, as there will be no
significant decrease in nodal size [30].
Fungal Lymphadenopathy
Fungal respiratory infections are most common in children
with primary or acquired immunodeficiency, as with immuno-
suppression after transplantation, chemotherapy, steroids, or
chronic illness. Fungal infection is often suspected when severe
infectious symptoms persist despite antibiotic treatment.
Common fungal respiratory pathogens affecting children
include aspergillus, candida, pneumocystis, coccidioidomyco-
sis, cryptococcus, and histoplasmosis. Several fungal patho-
gens are endemic to certain regions of the United States,
including histoplasmosis in the Ohio/Mississippi river valley
and coccidioidomycosis in the southwest.
The ultrasound appearance of pulmonary fungal infection
in children depends on the underlying pathogen.
Coccidioidomycosis frequently results in hilar lymphade-
nopathy, whereas histoplasmosis may cause hilar and medi-
astinal lymphadenopathy. On ultrasound, fungal lymph
nodes are indistinguishable from tuberculous lymph nodes,
as both can develop necrosis and calcification after treat-
ment. Enlarged, matted thoracic lymph nodes can coalesce to
form a mediastinal granuloma, which may compress adja-
cent mediastinal structures.
Treatment of fungal diseases in children depends on their
severity and the underlying immune status of the child.
S. Ali et al.
Immunocompetent children with mild histoplasmosis or coc-
cidioidomycosis may not require treatment as the disease is
histoplasmosis requires antifungal medication and possibly a
short course of corticosteroid therapy to control symptoms
and limit complications [31].
Fibrosing Mediastinitis
Fibrosing mediastinitis is a benign but locally aggressive
inflammatory disease characterized by extensive fibrosis in
the mediastinum, often resulting in luminal narrowing of
vital mediastinal structures including the airways, vessels,
and esophagus. The underlying etiology of fibrosing medias-
tinitis is currently unknown, however genetic factors are
thought to play a role [32].
Although no organism has been isolated from biopsy
specimens of fibrosing mediastinitis, it occurs most often
following histoplasmosis infection and is believed to result
from leakage of fungal antigens from infected mediastinal
lymph nodes. Exposure to the fungal antigens is thought to
incite a profound immune inflammatory response with sub-
sequent development of fibrosis. Morbidity from fibrosing
mediastinitis depends on the extent of fibrosis and its effect
on adjacent mediastinal structures, including the airways,
esophagus, and vessels.
On ultrasound, identification of ill-defined, nonvascular
mediastinal soft tissue, often with calcification and narrow-
ing of vascular structures, should raise concern for fibrosing
mediastinitis in a child with known histoplasmosis infec-
tion. The diffuse type of fibrosing mediastinitis can involve
multiple compartments, and appreciation of its entire extent
may be limited with ultrasound. Therefore, cross-sectional
imaging with CT or MR imaging is typically required for
complete assessment.
Extensive fibrosis associated with granulomatous inflam-
mation of the mediastinum generally does not respond to
antifungal therapies and may require endoscopic surgical
intervention in severe cases. However, mediastinal adenitis
that causes obstruction of the airway, esophagus or vascular
structures can be treated with a combination of antifungal
medication and corticosteroids [31].
Neoplastic Lymphadenopathy
Neoplastic mediastinal lymphadenopathy in children can
occur either as a primary malignancy, such as lymphoma, or
as metastatic lymph nodes. Lymphoma and lymphomatous
involvement of the mediastinal nodes are discussed in an ear-
lier section of this chapter.
On ultrasound, metastatic lymph nodes typically appear
enlarged and with a rounded contour. There is frequently
effacement of the central fatty hilum. The borders of meta-
static lymph nodes may be lobulated or irregular but remain
well-defined. Matting of lymph nodes can occur, where
7 Mediastinum
lymph nodes coalesce into a single mass-like lesion. On
color Doppler imaging, internal hilar vascularity of the meta-
static lymph nodes is lost. Instead, there is peripheral vascu-
larity since tumor vessels arise from the nodal periphery.
Calcification of metastatic lymph nodes can occur with some
malignancies, including osteosarcoma, neuroblastoma, and
extragonadal germ cell tumor.
Treatment of metastatic mediastinal lymph nodes depends
on the nature of the underlying tumor.
Paravertebral (Posterior) Mediastinal Masses
Almost 90% of paravertebral (posterior) mediastinal masses
in children are of neurogenic origin [33]. Neurogenic tumors
are the most common extracranial solid tumors in the pediat-
ric population. Neurogenic tumors can arise from any neuro-
nal structure, including the nerve sheath, sympathetic
ganglion, or paraganglion, although the majority arise from
the autonomic ganglia, including benign ganglioneuroma,
ganglioneuroblastoma of variable malignant potential, and
malignant neuroblastoma.
Neuroblastoma
Neuroblastoma is the third most common pediatric malig-
nancy after leukemia and central nervous system tumors [34].
The mediastinum is the second most common location for
neuroblastoma after the abdomen and generally carries a bet-
ter prognosis relative to other sites [35]. Children with tho-
racic neuroblastoma commonly present with pain, shortness
of breath, weight loss, and peripheral nerve deficits from neu-
ral foraminal invasion. Less often, neuroblastoma can present
with Horner syndrome and opsoclonus-myoclonus.
Neuroblastoma is traditionally staged using the International
Neuroblastoma Staging System, which is based on the surgi-
cal and pathological findings. A newer staging system,
proposed by the International Neuroblastoma Risk Group
(INRG) in 2009, emphasizes the use of image-defined risk
factors (IDRFs) [36]. IDRFs describe the surgical risk factors
that would make tumor resection difficult at the time of diag-
nosis, such as whether the tumor encases the aorta or its major
branches.
Currently, multiple imaging modalities are used for initial
staging and follow-up of neuroblastoma, including ultrasound,
MR imaging, CT, and nuclear medicine I-123 meta-iodoben-
zylguanidine (MIBG) scans. On ultrasound, neuro­blastoma
typically appears as a heterogenous paravertebral mass, which
may be solid or mixed solid and cystic in appearance (Fig. 7.15).
It can extend through the neural foramina, which is best appre-
ciated in the neonatal period.
Almost half of all neuroblastomas demonstrate calcification
which can be coarse, stippled, or curvilinear and is usually
233
detected with radiographs or CT, although occasionally it can
be visualized with ultrasound. On color Doppler evaluation,
neuroblastoma demonstrates internal vascularity but is not
highly vascular and does not typically demonstrate a domi-
nant feeding vessel which helps to differentiate it from a con-
genital lung malformation such as a bronchopulmonary
sequestration.
Neuroblastoma is a diverse disease, and its prognosis
depends on several factors, some of which are patient-­related,
although most are tumor-related. Current therapy of neuro-
blastoma is based on the risk categories as developed by the
Children’s Oncology Group. Patients are classified into low-,
intermediate-, and high-risk categories based on the stage of
the disease, age at presentation, histology of the tumor, MYCN
oncogene status, and DNA content of the tumor.
The general treatment for the low-risk tumor category is
surgical resection, and outcomes are excellent. In a subset of
low-risk infants under 1 year of age with a localized primary
tumor or with tumor dissemination limited to the skin, liver,
and/or bone marrow, observation is an option since there is a
high rate of spontaneous regression. For intermediate-­risk dis-
ease, a combination of surgery and chemotherapy is the current
standard of care. For high-risk disease, an aggressive approach
is pursued that includes chemotherapy, surgical resection, stem
cell rescue, radiation, and immunologic therapy [37].
Ganglioneuroblastoma
Ganglioneuroblastoma is an intermediate tumor on the spec-
trum of tumors arising from the autonomic (sympathetic)
ganglia. Ganglioneuroblastoma shares characteristics of
both malignant neuroblastoma and benign ganglioneuroma.
Neuroblastoma and ganglioneuroblastoma are often grouped
together for cancer reporting, staging, and survival statistics,
as both have malignant potential.
The imaging characteristics of ganglioneuroblastoma are
variable and can range from a well-encapsulated lesion to a
poorly marginated lesion associated with metastases.
Histologically, a ganglioneuroblastoma contains more than
50% differentiated cells, while neuroblastoma contains less
than 50%.
On ultrasound, ganglioneuroblastoma can appear as a solid
homogenous lesion or as a more cystic and poorly marginated
paravertebral mass. Imaging, therefore, cannot reliably differ-
entiate ganglioneuroblastoma from neuroblastoma.
Ganglioneuroblastoma together with ganglioneuroma
accounts for a quarter of localized neurogenic tumors.
Chemotherapy is not effective for these lesions, and non-­
radical or subtotal tumor resection is an acceptable form of
treatment as long as the margins of the residual tumor are
less than 2 cm in any dimension [38]. Small amounts of
residual tumor do not appear to affect survival, although reg-
ular long-term follow-up is warranted [39]. In cases where
234
a b
Fig. 7.15 Thoracic neuroblastoma in a 5-month-old female who pre-
sented with respiratory distress. (a) Transverse grayscale ultrasound
image of the upper mediastinum demonstrates a large, round, hetero-
geneous mass with calcification (arrowheads). (b) Transverse color
surgery might be associated with significant morbidity, a
watch-and-wait approach has been suggested [40].
Ganglioneuroma
Ganglioneuroma is a benign tumor composed entirely of
ganglion cells, with no malignant potential. School-age
­children are affected, with a median age of diagnosis ranging
from 5 and 10 years. Ganglioneuromas may arise de novo or
can result from maturation of a neuroblastoma or ganglio-
neuroblastoma. The most common locations for ganglioneu-
roma include the posterior mediastinum, retroperitoneum,
S. Ali et al.
Doppler ultrasound image reveals vascular flow to the lesion. (c) Axial
contrast-enhanced CT image shows a large, calcified paraspinal mass
that displaces the right lung anteriorly and shifts the mediastinum
(arrow) to the left
adrenal gland, and neck [41]. Ganglioneuromas are usually
asymptomatic but may rarely present with symptoms of cat-
echolamine excess, such as flushing, if the tumor produces
significant levels of catecholamines.
On ultrasound, ganglioneuroma typically appears as an
elongated, vertically oriented, well-circumscribed mass in
the paravertebral region, along the course of the sympathetic
chain. It is usually homogenously hypoechoic and vascular
by color Doppler. Calcifications are seen in 40–60% of cases
and may be fine, speckled, or coarse [41]. Calcification is
generally better identified with CT.
7 Mediastinum
Although ganglioneuromas are often less aggressive in
appearance, because of the substantial overlap in imaging
features of ganglioneuroma, ganglioneuroblastoma, and
neuroblastoma, it is difficult to reliably distinguish between
these tumors based on imaging alone, and pathological eval-
uation is usually required for definitive diagnosis.
Cardiophrenic Angle Masses
Pericardial Cyst
A pericardial cyst most often develops as a congenital abnor-
mality of mesothelial origin resulting from failure of fusion of
one of the mesenchymal lacunae that form the pericardial sac
[42]. It is characterized by a thin wall and usually contains
simple fluid. A pericardial cyst is commonly connected to the
pericardium and typically occurs in the anterior cardiophrenic
angle, frequently on the right side. Some pericardial cysts have
been described as developing after infection, inflammation, or
trauma. They are usually asymptomatic but can present with
symptoms related to compression of adjacent structures.
On ultrasound, a pericardial cyst typically appears as a
well-circumscribed unilocular cystic structure, predomi-
nantly anechoic with a thin wall (Fig. 7.16). On Doppler
evaluation, it is avascular. Complexity of the fluid and/or
septations may be present if there is superimposed hemor-
rhage or infection.
a b
Fig. 7.16 Pericardial cyst in a 9-year-old male with a history of pleu-
ropulmonary synovial sarcoma. (a) Axial contrast-enhanced CT image
shows a small, well-circumscribed lesion (arrowhead) adjacent to the
right heart border. Although the density of the lesion is compatible with
235
The clinical course of a pericardial cyst is usually benign.
When asymptomatic, it can be followed, and spontaneous
resolution can occur. When symptomatic or causing com-
pression of cardiac structures, intervention should be consid-
ered. Treatment options include cyst aspiration, chemical
sclerosis, and surgical resection [42].
Lymphadenopathy
Pericardial lymph nodes are located in or around the pericar-
dial fat in the prevascular space. Pericardial lymph nodes are
typically not visible on imaging studies in normal pediatric
patients. Consequently, visible pericardial nodes are likely to
be pathologic [43].
On ultrasound, the appearance of a pericardial lymph
node can vary depending on the underlying pathology. A
reactive node is typically enlarged with an elliptical or ovoid
shape. The cortex can appear thickened and hypoechoic as
compared to the adjacent pericardial fat. A normal echogenic
fatty hilum is preserved. In contrast, a malignant cardio-
phrenic lymph node is much larger and typically round in
shape (Fig. 7.17). The normal echogenic fatty hilum is lost,
and prominent central and peripheral vascularity is observed.
Treatment of enlarged pericardial lymph nodes depends on
the underlying pathology which may include ­antibiotics for
infection or antineoplastic regimens for metastatic disease.
H H
a cyst, the patient’s history of tumor raised concern for a metastasis. (b)
Sagittal contrast-enhanced ultrasound image (CEUS) reveals the avas-
cular (arrow), benign nature of the lesion. H, Heart
236
a b
Fig. 7.17 Pericardial lymph node in a 19-year-old male with relapsed
hepatocellular carcinoma. (a) Axial non-contrast CT image shows a lobu-
lated soft tissue mass (arrow) adjacent to the right heart border in keeping
with a metastatic pericardial lymph node. (b) Transverse CEUS image of
the lymph node after an initial cryoablation procedure reveals a central
non-enhancing zone consistent with ablated tumor. There is an irregular
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 Chest Wall
8
Jessica Kurian

Abbreviations and cartilage (sternum, rib cage, spine), vessels, and nerves.
Importantly, in children the chest wall is less rigid than in
ABC Aneurysmal bone cyst adults, due to its greater cartilaginous component.
CEUS Contrast-enhanced ultrasound Ultrasound is particularly well-suited for evaluation of
CH Congenital hemangioma the pediatric chest wall, as acoustic windows are excellent
CT Computed tomography when there is thin adipose tissue and incomplete ossification
DLBCL Diffuse large B-cell lymphoma of the bones, and most of the structures of interest are super-
ES Ewing sarcoma ficial. Ultrasound also allows for efficiently focused imaging
IH Infantile hemangioma of palpable chest wall abnormalities (Table 8.1).
LM Lymphatic malformation This chapter reviews up-to-date techniques for ultrasound
MH Mesenchymal hamartoma imaging of the pediatric chest wall, as well as chest wall
MHE Multiple hereditary exostoses embryology and normal anatomy. The clinical and imaging
MR Magnetic resonance features of various congenital and acquired disorders of the
MRSA Methicillin-resistant Staphylococcus aureus chest wall in infants and children are also discussed.
NICH Non-involuting congenital hemangioma
NHL Non-Hodgkin lymphoma
OS Osteosarcoma Technique
PICH Partially involuting congenital hemangioma
PNET Primitive neuroectodermal tumor Patient Positioning
RICH Rapidly involuting congenital hemangioma
RMS Rhabdomyosarcoma Optimal positioning is important for maintaining patient com-
VATS Video-assisted thoracoscopic surgery fort, reducing motion, and thereby maximizing image quality.
VM Venous malformation To examine the anterior and lateral chest wall, children can be
scanned in supine and lateral decubitus positions, with the
arms raised when necessary. To examine the posterior chest
Introduction wall, the patient can be sitting while using a bedside table to
rest the arms and support the chest during scanning. For infants
The chest wall consists of the bone and soft tissue structures and young children, a bolster (such as a foam wedge or rolled
that surround the lungs and the vascular structures of the towel) should be used for support, and it is important to have a
chest. It contributes to the physiologic mechanism of respira- parent, caregiver, or child life specialist at the bedside to assist
tion, protects the lungs and mediastinal structures, and pro- with positioning and motion reduction.
vides mechanical stabilization to the thorax, shoulder girdle,
and arms. The chest wall is made up of multiple components,
including the skin and subcutaneous tissue, muscles, bones Ultrasound Transducer Selection

J. Kurian (*) High-frequency (7–15 MHz) linear array transducers (also known
Division of Pediatric Radiology, Departments of Radiology and as “small parts” transducers) provide the best near-­field resolution
Pediatrics, Montefiore Medical Center and the Albert Einstein and are therefore optimal for examination of the superficial struc-
College of Medicine, Bronx, NY, USA
tures that comprise the chest wall. In general, one should use the
e-mail: jkurian@montefiore.org

© Springer Nature Switzerland AG 2021 239

H. J. Paltiel, E. Y. Lee (eds.), Pediatric Ultrasound, https://doi.org/10.1007/978-3-030-56802-3_8
240 J. Kurian

Table 8.1 Palpable pediatric chest wall lesions

Lesion Description Location US Features
Congenital
Asymmetric Angular deformity of cartilage Junction of cartilage and ossified Hypoechoic cartilage is angulated or enlarged –
costochondral (benign anatomic variant) rib, parasternal anterior chest must compare to the other side to see
junction wall asymmetry
Congenital vascular Vascular tumors and Subcutaneous tissue > muscle See Table 8.2
lesions malformations
Infectious
Cellulitis Bacterial infection of the skin Subcutaneous tissue Thickening, hyperechogenicity, and “disarray” of
and subcutaneous tissue subcutaneous fat; “cobblestoning”; hyperemia
Abscess Localized collection of pus with Usually subcutaneous tissue Fluid collection with variable echogenicity, thick
surrounding inflammation. hyperemic capsule, swirling echoes
Develops from cellulitis or
wound
Neoplastic
Benign
Osteochondroma Benign osteocartilaginous Rib >> scapula, clavicle, spine, Protuberance continuous with the rib, posterior
excrescence of the bone, sternum acoustic shadowing, hypoechoic cartilage cap
often affects chest wall in
MHE
Lipoma Benign tumor of mature fat cells Subcutaneous tissue >> muscle, Well circumscribed, usually hypoechoic, thin
commonly upper back hyperechoic linear bands, no flow or minimal
flow
Malignant
Sarcoma Group of malignancies arising Rhabdomyosarcoma: Chest wall Heterogeneous solid mass, irregular contour,
from the soft tissue, bone, musculature internal vascularity, necrosis
cartilage, or bone marrow Ewing sarcoma: rib >> other sites Osseous origin with sunburst lines and large
Osteosarcoma: rib >> other sites extraosseous portion in Ewing sarcoma
Posterior acoustic shadowing from osteoid matrix
in osteosarcoma
Lymphoma Group of malignancies arising May involve lymph nodes Enlarged lymph nodes, nonspecific solid mass,
from lymphocytes (axillary, pectoral, muscle infiltration (rare)
cardiophrenic) or chest wall
bone and muscle
Traumatic
Hematoma Localized extravascular Subcutaneous tissue or muscle Appearance varies by age: initially hyperechoic
collection of blood products. becoming hypoechoic with liquifaction;
Caused by trauma or bleeding fluid-debris levels, septations.
disorder Avascular unless chronically organized
Rib fracture Break in the rib, usually caused Osseous portion of the rib or Acute: Discontinuity or step-off of anterior
by trauma costal cartilage cortical echogenic line, “chimney
phenomenon” acoustic shadow, surrounding
fluid collection
Healing: Calcific prominence (callus) with
acoustic shadowing
Foreign body Any external object that becomes Usually subcutaneous tissue Hyperechoic structure with clean or dirty acoustic
retained in the patient, via shadow; “comet-tail” artifact from metal or
penetrating wound or glass; surrounding inflammation or hypoechoic
iatrogenic placement fluid collection if retained >24 hours
MHE, Multiple hereditary exostoses

highest frequency possible that still allows adequate depth visual-
ization of the lesion in question. For very small or very superficial
lesions (e.g., skin lesions), a high-frequency L-shaped transducer
(also known as a “hockey stick” transducer) is a useful adjunctive
tool. This transducer has a small acoustic window with a small
footprint on the order of 25 mm, compared to 38 mm or larger for
commonly used linear transducers [1].
Low-­frequency transducers may be required on some occa-
sions when greater depth penetration is required, such as for
larger patients, or when examining lesions with a deeper
extent, usually greater than 6 cm from the skin surface [2, 3].
If there is a disease process that extends deep to the chest wall
or requires a wider viewing area, a phased array transducer is
appropriate. In these cases, probes with small footprints are
helpful for scanning through the intercostal spaces [3].
8 Chest Wall
Imaging Approaches
Protocols
When performing ultrasound of the chest wall, rather than
trying to develop a standardized protocol, it is more efficient
to tailor each study to the location and size of the suspected
lesion. For the majority of ultrasound examinations of the
chest wall, the scan is targeted directly to a region of con-
cern, usually a palpable mass. Technical parameters such as
frequency, focal depth, gain, etc. should be adjusted appro-
priately for the position, depth, and size of the targeted
lesion. Color Doppler ultrasound should be used to deter-
mine the vascular properties of any chest wall mass, particu-
larly soft tissue abscesses and vascular malformations. For
children who are old enough to follow instructions, short
periods of breath holding can be helpful, especially during
Doppler assessment.
Because the majority of chest wall examinations deal
with superficial structures, it is important to use an adequate
amount of coupling gel, and the imager’s hand should fully
support the weight of the transducer. This minimizes trans-
ducer pressure which can cause morphological distortion of
superficial lesions and reduce detectability of Doppler flow
[4, 5]. When evaluating deeper structures or large pediatric
patients, these techniques are not always applicable, since
some degree of manual pressure may be necessary to
improve image quality [6].
Ultrasound images should be obtained in sagittal and
transverse planes that extend through the abnormality. Cine
clips are helpful for characterization of irregularly shaped
lesions. When evaluating anterior chest wall deformities,
comparison images of the contralateral side at the same level
must be obtained to assess symmetry. It is often helpful to
initially study the asymptomatic side to familiarize oneself
with the patient’s normal anatomy. Extended field of view or
panoramic images can be used as necessary to depict large
lesions.
Annotations
It is important to document images using standard landmarks
based on the surface anatomy of the chest. Transducer posi-
tion should be documented by referencing these landmarks,
including the mid-sternal or anterior median line (midline
from sternal notch to xiphoid), mid-clavicular line (drawn
vertically through the middle of the clavicle), axillary fossa
(bordered by the scapula, clavicle, first rib, pectoralis and
latissimus dorsi muscles), anterior and posterior axillary lines
(through the anterior and posterior axillary folds), mid-axil-
lary line (through the apex of the axillary fossa), mid-scapular
241
line (drawn vertically through the inferior angle of the scap-
ula), and mid-­spinal line (midline posterior chest) [7].
Contrast-Enhanced Ultrasound
In recent years, contrast-enhanced ultrasound (CEUS) has
gained a preliminary foothold in the evaluation of lesion vas-
cularity patterns, particularly of liver and kidney masses [8,
9]. Second-generation contrast agents are now in use, which
consist of microbubbles of gas suspended in a phospholipid
shell and administered through a peripheral intravenous line.
These microbubbles resonate when exposed to the ultra-
sound beam, resulting in vascular “enhancement” that
appears as hyperechogenicity on dynamic grayscale imag-
ing. This technique allows visualization of vascular anatomy
and temporal perfusion patterns.
There is relatively little experience with the use of CEUS in
the evaluation of extremity soft tissue tumors in children or
of the chest wall. However, early studies of CEUS imaging
of extremity masses in adults show that it increases accuracy
in distinguishing between benign and malignant lesions [10–
13]. It is feasible that the same principles can be translated
into evaluation of chest wall masses. This would reduce the
need for computed tomography (CT) or magnetic resonance
(MR) imaging and thereby avoid the risks associated with
ionizing radiation, sedation and anesthesia, and gadolinium
administration. Further study is needed in this area.
Normal Development and Anatomy
Normal Development
The musculoskeletal structures of the chest wall form between
the fourth and eighth week of gestation; however, some compo-
nents continue to develop even after birth [14]. The chest wall
and trunk originate from paraxial mesoderm and somatic (lateral
plate) mesoderm [15]. The paraxial mesoderm organizes itself
segmentally along the neural tube into 40 somites. The somites
then segregate into sclerotomes and dermomyotomes [16].
Thoracic Skeleton
The sclerotomes become the bony structures of the chest
wall [16]. They are initially located ventromedially and
migrate toward the neural tube to become the vertebrae.
Each vertebra is formed by two successive somite levels.
Sclerotomic cells surround the notochord to form the inter-
vertebral discs. In the thorax, foci of mesenchyme develop
242 J. Kurian

Costovertebral
Vertebral
joint
Vertebral Costal arch
arch process
Rib
Rib

Vertebral
body

Growing Vertebral Vertebral

rib body body

35 days 38 days 40 days

Fig. 8.1 Appearance of the ribs and vertebrae at progressive stages of horizontal direction. The transverse processes of the vertebrae grow
embryonic development. The costal processes develop from the mesen- outward to meet the tubercle of the rib. A joint also forms between the
chyme at the lateral aspect of the thoracic vertebral arches and extend vertebral body and the head of the rib (“costovertebral joint”)
outward to become the growing ribs. The ribs continue to lengthen in a

Clavicle Manubrium

Clavicle

Rib

Ossification
centers
Xiphoid
Sternal bars process
Mesenchymal Ribs (begin to chondrify and fuse
condensations as ribs become connected)

43 days 45 days Birth

Fig. 8.2 Appearance of the sternum at progressive stages of fetal devel- craniocaudad manner. Segmental ossification centers form in the manu-
opment. Mesenchymal condensations in the midline thorax form the brium and sternum, also in a craniocaudad order. Two or more ossifica-
two longitudinal sternal bars. The sternal bars and anterior aspects of tion centers can form within each segment (but will later fuse). The
the ribs are cartilaginous (indicated in blue). The sternal bars fuse in a xiphoid process does not ossify until the second or third decade of life

lateral to the vertebral arches to become the costal processes,
which then lengthen to form the ribs (Fig. 8.1). Later in
development, primary ossification centers form near the rib
angles, and the cartilaginous ribs undergo endochondral
ossification. Complete ossification of the ribs only occurs
during adolescence, at which time secondary ossification
centers form at the heads and tubercles of the ribs.
The sternum is independently derived later in gestation
(10 weeks) from somatic (lateral plate) mesoderm [16]. In
the midline, mesenchymal tissue forms two longitudinal car-
tilaginous sternal bars, which eventually fuse in a craniocau-
dal direction (Fig. 8.2). As with the ribs, the cartilaginous
sternum later develops segmental ossification centers that
will fuse to each other (Fig. 8.3). The xiphoid completes its
ossification only in the second or third decade of life, some-
times even as late as 40 years of age [16, 17].
Chest Wall Soft Tissues
The dermomyotomes become the soft tissues of the chest
wall [16]. They are positioned dorsolaterally and subse-
quently separate into dermatomes and myotomes. The myo-
tomes give rise to the skeletal musculature, still maintaining
their segmental arrangement. The myotomes further subdi-
vide into two components: epimeres which form the deep
muscles of the back and hypomeres which form the muscles
of the ventrolateral body wall, including the intercostal mus-
8 Chest Wall 243

cles (Fig. 8.4). The dermatomes give rise to the dermis and
connective tissue of the dorsal chest wall. In contrast, the
ventral dermis is formed from somatic mesoderm. Each der-
1 matome and myotome is innervated by a segmental nerve.

Normal Anatomy
2
Thoracic Skeleton
The thoracic skeleton consists of the 12 thoracic vertebrae
(T1-T12), 12 paired ribs and costal cartilages, and the ster-
3
num (Fig. 8.5). A typical rib consists of a head, neck, tuber-
Fuse to Form cle, angle, and shaft. The head of each rib articulates with the
Sternal Body superior articular facet of the vertebra it is numbered after
4
and also with the inferior articular facet of the vertebra
above; this forms the costovertebral joint. The tubercle of the
rib articulates with the transverse process of the vertebra,
5 forming the costotransverse joint. These facet joints are
synovial joints. The shaft of the rib has a costal groove that
houses the intercostal neurovascular bundle as it travels for-
6 ward from the spine and aorta.
The first through seventh ribs are termed “true” ribs, as
they connect directly to the sternum and manubrium. The 8th
Fig. 8.3 Anterior and lateral views of the sternum. The sternum con- through 12th ribs are termed “false” ribs, as they do not artic-
sists of six ossification centers: the manubrium (1), the four segments of ulate with the sternum. The costal cartilages are bands of
the sternal body (2–5), and the xiphoid process (6). The sternal body hyaline cartilage that attach the medial ends of the ribs to the
segments 2–5 begin fusing in adolescence and complete fusion by age
25 years. The manubrium does not fuse to the sternum (except occa- sternum. The costal cartilages of ribs 1–7 curve superiorly to
sionally in old age) and remains as the manubriosternal symphysis, also articulate with the sternum, and the costal cartilages of ribs
known as the sternal angle or angle of Louis 8–10 articulate with the cartilage of the rib above. The 11th

Epimere
Extensor muscle
Intermuscular of spine
septum
Dorsal primary
Spinal
ramus
cord Spinal
ganglion

Hypomere Outer,
intermediate,
Dorsal and inner
aorta muscular
layer of
Ventral primary hypomere
ramus as found
in wall
of thorax
and
Coelomic cavity
abdomen

Rectus column

a b

Fig. 8.4 Transverse sections of the embryonic thorax at (a) 5 weeks ventrolateral body wall muscles by separating into three layers (outer,
and (b) 7 weeks. The skeletal muscle is formed from myotomes, which intermediate, and inner) intercostal muscles. A segmental nerve associ-
divide into epimeres and hypomeres. The epimeres form the deep ates with each myotome to provide motor innervation via the dorsal and
extensor muscles of the back (erector spinae). The hypomeres form the ventral rami
244
Sternum
Sternal
Clavicle notch Manubrium
2
3
True ribs
4
(1-7)
5 Tubercle
6 Costal groove
7 Superior
8 Tubercle
11
False ribs 9
12
(8-12)
10
Costal cartilages
Floating ribs
(11-12)
Fig. 8.5 Anterior view of the thoracic skeleton. The true, false, and
floating ribs are denoted. The anatomy of the ribs is shown, consisting
of the head, neck, tubercle, angle, and shaft. The costal groove houses
the intercostal vein, artery, and nerve. The head of the rib has two artic-
and 12th ribs are termed “floating” ribs because their carti-
lage terminates in the muscles of the abdominal wall. The
sternocostal joints are synovial joints, except for the first rib
which is fused to the manubrium as a synchondrosis.
The sternum consists of three flat bones: the manubrium,
sternal body, and xiphoid process. The jugular or suprasternal
notch is the palpable landmark at the superior aspect of the
manubrium, bounded by the clavicles on each side. The ster-
nal angle or angle of Louis is a bony ridge at the manubrioster-
nal joint, which is continuous with the second rib and is the
landmark for the level of the tracheal bifurcation. The xiphoid
is the smallest and thinnest bone, located at the inferior aspect
of the sternum. The paired internal thoracic arteries (also
known as the internal mammary arteries) are branches of the
subclavian arteries that supply the sternum (Fig. 8.6). The ven-
tral chest wall is also supplied by collateral vessels between
the subclavian artery and the deep epigastric artery.
Musculature
The muscles of the anterior chest wall include the pectoralis
major, a large fan-shaped muscle originating from the clavi-
cle and sternum and inserting on the bicipital groove of the
humerus (Fig. 8.6). The pectoralis minor lies deep to the pec-
toralis major, originating from ribs 3–5 and inserting on the
J. Kurian
Xiphoid
Angle Body process
Neck Head
Angle
Articular
facets
Middle Rib:
Posterior View
1st Left Rib: Head
view Neck
(no angle)
Subclavian vein
& artery grooves Head
2nd Left Rib: Neck
Superior View
Tubercle
Angle
ulations, one with the costal facet of the vertebra at the same level and
one with the cephalad level (costovertebral joints). The tubercle of the
rib articulates with the transverse process of the vertebra (costotrans-
verse joint)
coracoid process of the scapula. The other superficial or
extrinsic muscles of the back include the serratus anterior
and latissimus dorsi. The serratus anterior consists of multi-
ple slips of muscle coursing from the medial scapula along
the anterolateral chest wall between ribs 1 and 8. The latis-
simus dorsi is the largest muscle in the body and has exten-
sive attachments to the spine (T6 or T7 through L5), ribs
(posterior ribs 8 or 9 through 12), inferior border of the scap-
ula, humerus, and posterior iliac and sacral bones.
The smaller extrinsic muscles of the chest wall include the
trapezoids, rhomboids, subclavius, and serratus posterior.
Additionally, some of the abdominal wall muscles have attach-
ments to the ribs and xiphoid process, including the rectus
abdominis, external and internal oblique muscles, and transver-
sus abdominis. The intrinsic or deep back muscles include sev-
eral groups of muscles that fuse to the vertebral bodies,
functioning to maintain posture and control vertebral movement.
These include the erector spinae and transversospinalis groups.
The deepest muscles of the chest wall are the intercostal
muscles (Figs. 8.6 and 8.7). There are three layers of muscle
spanning each intercostal space: the external and internal inter-
costal muscles, which course obliquely in opposite directions
to each other, and the innermost intercostal muscle, which is a
thin layer that is separated from the former two muscles by the
8 Chest Wall 245

Sternohyoid muscle
Sternocleidomastoid
Internal
ernal jugular vein muscle
Middle
e scalene muscle Anterior scalene
Anteriorr scalene muscle muscle
Posteriorr scalene muscle Trapezius muscle

Clavicle Omohyoid muscle

Common Pectoralis major

carotid artery muscle

Internal thoracic Deltoid muscle

artery and vein External intercostal
Intercostal vein, muscles
artery and nerve Intercostobrachial
nerve membranes
Transversus thoracic
muscle Internal intercostal
muscles
Musculophrenic
artery and vein External intercostal
membrane
External intercostal
muscles Digitations of serratus
anterior muscle
Superior epigastric
artery and vein Latissimus dorsi
muscle
Rectus abdominis
muscle

Fig. 8.6 Anterior view of the thorax. The muscles and neurovascular struc- intercostal muscle layers and the transversus thoracic muscle. The anterior
tures of the chest wall are denoted, with their relationships to the thoracic aspect of the external intercostal muscle thins into an aponeurosis called the
skeleton. The extrinsic muscles that are shown here include the trapezius, external intercostal membrane, which inserts on the sternum. The pectoralis
serratus anterior, and latissimus dorsi. The intrinsic muscles refer to the muscle connects the anterolateral thoracic wall to the upper extremity

Rami
communicantes

Anterior ramus
(intercostal nerve)
Internal intercostal Posterior intercostal
membrane artery
External intercostal Lateral pectoral
muscle cutaneous branch artery
Lateral pectoral
cutaneous branch nerve Site of anastomosis/potential
Innermost intercostal collateral pathway between
muscle Parietal pleura Sympathetic Aorta posterior and anterior
Internal intercostal (cut edge) tunk intercostal arteries
muscle
Common membrane Transversus Internal
of innermost thoracis thoracic artery
intercostal and
transversus thoracis
muscles
External intercostal
membrane
Anterior pectoral
cutaneous branch
nerve
Anterior Anterior
perforating intercostal artery
branch artery

Fig. 8.7 Transverse section through the mid-thorax at T4-T5. This sec- branches. The posterior intercostal arteries arise from the thoracic aorta,
tion illustrates the intrinsic muscle layers of the chest wall, as well as and the anterior intercostal arteries arise from the internal thoracic
the segmental arterial blood supply and spinal innervation. The anterior (internal mammary) artery. Perforating arteries supply the muscle, fas-
rami give rise to the intercostal nerves, which supply pectoral cutaneous cia, and skin
246 J. Kurian

Skin: epidermis
a Skin
and dermis Subcutaneous
Subcutaneous
fat Fascia
Fascia
Muscle
Skeletal muscles Rib Intercostal Rib
Internal intercostal
Pleura
muscle
External intercostal
muscle
Rib
Lung
Vein, artery, and
nerve

Parietal pleura
Skin
Intrapleural space b
Subcutaneous
Visceral pleura

Lung parenchyma Fascia

Muscle

Rib
Intercostal
Fig. 8.8 Sectional view of the layers of the chest wall. The fascia rep-
resents a layer of connective tissue that lies below the subcutaneous fat Pleura
and encases the skeletal muscle. The extrinsic layers of skeletal muscle
vary according to the region of the chest wall (e.g., pectoralis, serratus
anterior, etc.)
Lung
neurovascular bundle. The transversus thoracis muscle can be
considered part of the innermost layer and attaches to the ster-
num, xiphoid, and costal cartilage of ribs 2–6. Blood supply to
Fig. 8.9 Normal chest wall anatomy on ultrasound in (a) a 15-month-­
these muscles is from the posterior intercostal branches of the
old female and (b) a 12-year-old male. The layers of the chest wall,
thoracic aorta and the anterior intercostal branches of the inter- from outer to inner, are the skin (dermis and epidermis), subcutaneous
nal thoracic artery. The intercostal nerves arise from the ante- fat, fascia, skeletal muscle, rib, and intercostal muscles. Deep to this lie
rior rami of the 1st through 11th thoracic nerves. the pleura and lung parenchyma. The subcutaneous fat demonstrates a
lobular appearance and contains thin septa. Linear echogenic striations
can be seen in the skeletal muscle. The cartilaginous portion of the ribs
in the younger patient (a) is hypoechoic. The ossified rib in the older
 ltrasound Appearance of Normal Chest Wall
U patient (b) casts a posterior acoustic shadow
Anatomy

The layers of the chest wall, from outer to inner, are the
skin, subcutaneous fat, fascia, skeletal muscle, and bone,
underneath which lie the pleura and lung (Fig. 8.8). These
layers can be readily demonstrated using high-frequency
ultrasound and are important to recognize in localizing the
origin of a mass (Fig. 8.9).
The epidermis and dermis appear together as a single thin
hyperechoic layer. Deep to this, the subcutaneous fat can be of
variable thickness and demonstrates hypoechoic lobules of fat
with randomly arranged echogenic septa. The fascia separates
the fat and superficial muscle layer, appearing as a thin hyper-
echoic band. Skeletal muscles are encountered next, recog-
nized by hypoechogenicity with characteristic echogenic
striations. This layer includes the pectoralis muscles, serratus
Fig. 8.10 Normal chest wall anatomy on ultrasound in an 11-year-old
anterior, latissimus dorsi, rhomboids, and trapezius muscles. female. The developing breast tissue (asterisk) is hypoechoic, with sur-
In pubertal females, breast tissue can be seen overlying rounding echogenic connective tissue and fat. The breast tissue overlies
the pectoral muscles between the second and sixth ribs the pectoralis major muscle (arrows), which contains echogenic stria-
(Fig. 8.10) [4]. In larger females, a retromammary fat layer tions that are typical of skeletal muscle
8 Chest Wall
S1 S2 S3
M
Fig. 8.11 Normal sternum in a 5-day-old male. Sagittal grayscale
ultrasound image at the mid-sternal line demonstrates the ossification
centers of the manubrium (M) and the first three sternal body segments
(S1, S2, S3). The fourth segment and the xiphoid are beyond the field of
view. Hypoechoic cartilage (white arrowheads) is seen between the
sternal ossification centers. The cartilage at the manubriosternal articu-
lation (black arrowhead) has an outward prominence which corre-
sponds to the palpable sternal angle. Thymic tissue (asterisks) is noted
in the retrosternal space
Fig. 8.12 Xiphoid process in a 17-year-old male. Sagittal grayscale
ultrasound image at the mid-sternal line demonstrates the hypoechoic
cartilage (black arrows) of the xiphoid and the ossified portion (white
arrow) of the inferior sternal body. The xiphoid may not ossify until the
third decade of life
can sometimes be seen between the breast tissue and the pec-
toral muscle.
Deep to the muscles are the bony structures of the thorax
(ribs, sternum, scapulae, vertebrae). The cortical surface of
ossified bone appears as a sharp hyperechoic line with
intense posterior shadowing. The non-ossified hyaline carti-
lage of the ribs is hypoechoic or nearly anechoic (due to
high water content), sometimes with a thin hyperechoic bor-
der and internal scattered punctate bright foci (Fig. 8.9a).
The echogenicity of cartilage increases with skeletal matu-
ration [18]. Cartilage can also be seen in the sternum of
young patients prior to fusion of the ossification centers, as
well as at the xiphoid (Figs. 8.11 and 8.12).
Deep to the rib cage is a strong hyperechoic line repre-
senting the interface between the pleura and aerated lung
247
R
R
Fig. 8.13 Intercostal vessels in a 17-year-old male. Sagittal color
Doppler ultrasound image (with inverted color scale) of the anterior
chest at the level of the cartilaginous ribs (R) demonstrates the intercos-
tal vein (white arrow) and intercostal artery (black arrow) along the
inferior margin of the rib
(Fig. 8.9). The pleural line can be visualized sliding against
the chest wall during respiration.
The vessels related to the chest wall are not routinely
evaluated; however with careful inspection, the intercostal
artery and vein can be observed (Fig. 8.13) [19]. The inter-
costal nerves are generally not visible by ultrasound unless
involved by a pathologic process.
Congenital Chest Wall Anomalies
Vascular Tumors and Malformations
Vascular lesions are divided into two major categories,
according to the classification scheme of the International
Society for the Study of Vascular Anomalies (ISSVA):
tumors and malformations [20]. Vascular tumors are catego-
rized into three groups: benign, locally aggressive, or border-
line. Vascular malformations, which are nonneoplastic, are
categorized into four groups: simple, combined, those of
major named vessels, and those associated with other anom-
alies. A third broad category is used for provisionally unclas-
sified anomalies, such as lesions with features that overlap
between tumor and malformation.
Ultrasound is highly important in the assessment of vas-
cular anomalies and is usually the first imaging test. It is used
248
Table 8.2 Clinical and ultrasound features of selected vascular lesions of the pediatric chest wall
Lesion Clinical presentation
Infantile Blue-red lesion
hemangioma Appears shortly after birth, proliferates for weeks/
months, involutes beginning at 6–12 months for
several years
May remain as focal fibrofatty tissue after
involution
Congenital Violaceous mass, may ulcerate
hemangioma Fully formed at birth
RICH: 90% involution by 3 months, total
involution by 14 months
NICH: May grow commensurate with patient
PICH: Begins as RICH but does not fully involute
and then persists like a NICH
Venous Single or multiple lobular bluish masses,
malformation compressible
Lymphatic Wide range of size and extent
malformation May be identified at birth or in the first 2 years
Spontaneous regression possible
Sudden enlargement from hemorrhage, infection
RICH, Rapidly involuting congenital hemangioma; NICH, non-involuting congenital hemangioma; PICH, partially involuting congenital
hemangioma
to identify the type of lesion; assess its size, extent, and
degree of vascularity; and monitor response to treatment.
The grayscale and Doppler features, combined with the clin-
ical appearance, allow for categorization of the lesion and
guidance of therapy. Ultrasound features that must be deter-
mined include lesion echogenicity; presence of solid tissue,
cysts, calcification, or thrombi; compressibility; density of
vascularity on color Doppler; and presence of arterial and
venous waveforms, with measurement of velocities and
resistive indices on spectral Doppler.
This section of the chapter does not cover all the diagnoses
in the ISSVA classification scheme but does address those
entities that are most commonly encountered in the pediatric
chest wall (Table 8.2). It is helpful to note that the ultrasound
properties of these lesions can be applied to any body part in
which they occur. It should also be noted that vascular malfor-
mations may occur in combination with each other resulting in
a “mixed” lesion with overlapping ultrasound features.
Hemangioma
Hemangioma is the most common pediatric vascular neoplasm,
of which there are two forms, infantile and congenital [21].
Infantile hemangioma (IH) is the most common vascular lesion
in children, with a prevalence of 5–10%, and is far more likely
to be seen in the chest wall than congenital hemangioma (CH)
[22, 23]. IH develops shortly after birth as a blue-red lesion and
then undergoes a proliferative phase over the next few months to
1 year and then an involution phase over the next few years,
sometimes leaving behind fibrofatty tissue [23, 24].
Certain large or deep segmental IHs are associated with
syndromes and occur in characteristic locations [22]. Examples
J. Kurian
Grayscale ultrasound Doppler ultrasound
Well-defined, ovoid or Diffusely hypervascular (“lights up”)
lobular shape Low-resistance arterial waveforms
Solid with variable
echogenicity,
heterogeneous
Large, heterogeneous Abundant enlarged peripheral vessels,
Calcification, hemorrhage, large feeding arteries and draining
large vessels veins
Hypoechoic, spongiform Slow velocity venous flow or
May appear as a collection undetectable flow
of vessels
Thickened subcutaneous fat
Calcified phleboliths
Thin-walled cystic spaces No vascularity apart from some flow in
Debris, fluid-fluid levels septa
Echogenic and mass-like if
microcystic
include PHACE syndrome (posterior fossa brain malforma-
tion; large IH of the face, neck, or scalp; arterial anomalies;
cardiac defects and aortic coarctation; and eye anomalies), and
LUMBAR syndrome (Lower body IH; Urogenital anomalies;
ulceration; Myelopathy; Bony deformities; Anorectal malfor-
mation; arterial anomalies; and Renal anomalies) [25].
CHs are present at birth as fully grown masses. There are
three types of CH which are characterized by their clinical
time course: rapidly involuting congenital hemangioma
(RICH), non-involuting congenital hemangioma (NICH), and
partially involuting congenital hemangioma (PICH) [26].
Diagnosis of small hemangiomas, which are usually the
IH form, can be made on clinical grounds and does not
require imaging. For larger lesions with uncertain anatomic
extent, ultrasound is performed as the initial assessment.
When it develops in the chest wall, IH is usually confined to
the subcutaneous tissue [22]. During the proliferative phase,
it has a nonspecific grayscale ultrasound appearance, pre-
senting as a well-defined mass, which may be hyperechoic,
hypoechoic, or heterogeneous (Figs. 8.14 and 8.15) [27].
Color Doppler demonstrates rich vascularity with high
vessel density (greater than five vessels or color pixels per
square cm) [28]. Spectral Doppler shows the presence of
both arteries and veins, usually with high-velocity, low-resis-
tance arterial waveforms. Although ultrasound is not typi-
cally used during the involution phase, the shrinking lesion
demonstrates decreased vessel density and hyperechogenic
fatty tissue.
CHs are usually solitary and tend to be distinct in appearance
from IH, in that they are heterogeneous and contain large intral-
esional vessels which are visible on grayscale, representing arter-
8 Chest Wall
a ies, dysplastic veins, and venous lakes [21, 29]. Arteriovenous
b the liver), and high-output cardiac failure [30, 31].
Fig. 8.14 Hemangioma in a 4-week-old female. (a) Longitudinal gray-
scale ultrasound image of the right lateral chest wall shows a well-­defined
lesion (calipers) in the subcutaneous fat that is hyperechoic to the skeletal
muscle. (b) Transverse color Doppler ultrasound image demonstrates
vascularity throughout the lesion, typical of hemangioma
a b
Fig. 8.15 Hemangioma in a 19-month-old female. (a) Longitudinal
grayscale ultrasound image of the right anterior chest wall shows a
mildly heterogeneous lesion (calipers) in the subcutaneous fat that is
hyperechoic to the skeletal muscle. (b) Longitudinal power Doppler
249
shunting, venous thrombi, and calcifications have also been
described [21, 22, 28]. These features are not seen in IH.
If IH is multiple with five or more skin lesions identified
on physical examination, affected pediatric patients must be
screened for the presence of liver hemangiomas using ultra-
sound [23]. IH does not generally require treatment because it
is expected to involute, although treatment may be needed in
certain situations, including complications due to growth in
high-risk locations (e.g., airway, orbit), local complications
(ulceration, hemorrhage), multiple IHs (usually visceral, e.g.,
Oral propranolol is the usual treatment choice. Second-
line therapies include corticosteroids, interferon alpha, vin-
cristine, and pulsed dye laser [30, 31]. Ultrasound is used to
assess treatment response, and special attention must be paid
to any portions of the lesion that are not visible to the clini-
cian [22].
Treatment of CH depends on observation of changes dur-
ing the first few days and weeks of life. RICH begins involut-
ing shortly after birth, whereas NICH grows in proportion to
the child, although the prognosis is favorable. PICH demon-
strates intermediate behavior, initially acting as RICH but
evolving to a persistent NICH [26]. Infrequently, therapy for
CH may be needed due to symptoms from size, growth, ulcer-
ation, bleeding, thrombocytopenia, or heart failure. Treatments
include excision, embolization, or pulsed dye laser.
ultrasound image at the same level shows diffuse vascularity with high
density of vessels. (c) Spectral Doppler sample of the lesion reveals
low-resistance arterial waveforms
250
Venous Malformation
Venous malformation (VM) is a low-flow lesion, consist-
ing of dysplastic veins (blood-filled channels) with defi-
ciency of smooth muscle, which form a single or multifocal
mass [23, 32]. VM is the most common vascular malfor-
mation, usually seen in the skin or subcutaneous tissue;
however, it can occur anywhere including deeper struc-
tures such as the muscle, bone, and internal organs [21,
33]. Most cases are sporadic, although there are some
familial forms that affect both the skin and mucosa, such
as the blue rubber bleb nevus syndrome where multiple
VMs occur in the gastrointestinal tract [20].
VMs are present at birth and grow with the child; how-
ever, they sometimes are not apparent until the child is older
a b
R S
Fig. 8.16 Venous malformation in a 13-year-old male who presented
with a bluish skin lesion on the right anterior chest wall. (a) Longitudinal
grayscale ultrasound image shows a serpiginous hypoechoic lesion
(arrows) in the subcutaneous tissue overlying the muscle (M) and rib
(R). (b) Power Doppler ultrasound image at the same level demonstrates
a b
X
+ +
Fig. 8.17 Venous malformation in a 17-year-old male who presented
with a left anterior chest wall mass. (a) Transverse grayscale ultrasound
image demonstrates a well-defined hypoechoic mass (calipers) with a
spongiform echotexture and internal linear septa. There is a shadowing
calcification (arrow) in the mass consistent with a phlebolith, which is
J. Kurian
and tend to enlarge during puberty [20, 34]. On clinical
examination, VM usually presents as a soft mass with over-
lying bluish skin discoloration but sometimes may present as
a group of dilated superficial veins [20]. There are multifocal
and diffuse forms which can involve large areas such as an
entire limb. VM may enlarge with a Valsalva maneuver or
gravity dependence (change in positioning) and cause pain
and swelling after vigorous activity, or thrombophlebitis [20,
21, 34, 35].
On ultrasound, VM appears as a well-defined hypoechoic
mass with spongiform echotexture, due to enlarged blood-­
filled spaces with intervening septa (Figs. 8.16 and 8.17) [21].
In some lesions, subcutaneous fat can be seen interdigitating
between dysplastic veins [22]. Doppler examination demon-
a few areas of color signal, with the majority of the lesion appearing
avascular due to slow flow. (c) Axial T2-weighted, fat-­suppressed mag-
netic resonance (MR) image of the anterior chest wall demonstrates the
corresponding superficial soft tissue lesion (arrow), with hyperintense
signal typical of a venous malformation. S, Sternum
pathognomonic of a venous malformation. (b) Axial contrast-enhanced,
T1-weighted, fat-suppressed MR image shows the corresponding mass
within the left pectoralis musculature. The mass demonstrates diffuse
gadolinium uptake and signal voids (arrow) due to phleboliths
8 Chest Wall
strates slow venous flow, sometimes so slow as to be undetect-
able, although intralesional thrombosis can also be seen
[20–22]. Augmentation with compression or Valsalva maneu-
ver can help detect flow [36]. The lesions are usually compress-
ible, in keeping with their venous nature. Although uncommon,
the presence of calcified phleboliths is pathognomonic, as they
are rarely seen in other vascular anomalies [22, 27].
In cases of uncertain diagnosis or large extent, MR imag-
ing evaluation is required. The deep veins of the involved
area must be imaged prior to directed therapy [34].
Treatment is not required for small or asymptomatic VMs
but can be indicated in VM complicated by pain, disfigure-
ment, or functional impairment [37, 38]. Supportive therapies
include compression garments, analgesics, and anticoagulants
in cases with localized intravascular coagulopathy (intrale-
sional thrombus). Directed therapies include sclerotherapy
(first-line), surgery (for small, well-defined lesions), and tar-
geted agents such as sirolimus [39]. Ultrasound and/or MR
imaging is performed pre- and post-­sclerotherapy to demon-
strate efficacy of treatment.
Lymphatic Malformation
Lymphatic malformation (LM) is another low-flow vascular
malformation consisting of abnormal lymphatic channels,
often manifested as dilated endothelial-lined cysts [23]. The
individual locules are variable in size, termed macrocystic
when >1–2 cm, microcystic when <1 cm (may be solid-
appearing), or combined macro- and microcystic. Imaging
determination of cyst size is important for guiding therapy.
LM occurs in a wide range of size and extent, sometimes
confined to subcutaneous tissue, but characteristically able to
violate fascial planes and extend trans-spatially into deeper
structures. LMs are congenital and therefore often visible at
birth or identified in the first 2 years of life, presenting as a
a b
+
Fig. 8.18 Lymphatic malformation with internal hemorrhage in a
20-month-old female who developed a chest wall mass and pain after a
fall. (a) Longitudinal grayscale ultrasound image demonstrates a thin-­
walled multiloculated cystic lesion (calipers) in the subcutaneous tis-
sue. Anechoic lymphatic fluid (asterisk) is seen in one of the locules,
251
soft palpable mass [34]. LMs grow in proportion to the child;
however, they can spontaneously regress, or they can sud-
denly enlarge and cause pain due to triggers such as viral
illness, intralesional hemorrhage, or infection [22, 40].
On ultrasound, macrocystic LMs appear as multiple adja-
cent thin-walled cystic spaces with posterior acoustic enhance-
ment (Figs. 8.18 and 8.19). There is no vascularity except for
flow that is sometimes present in the intervening septa. Internal
echoes or fluid levels can be seen in locules that contain blood
products or protein. Microcystic LM can appear as echogenic
mass-like tissue. However, this is due to the high density of
small cysts; a true LM has no solid component. As with VM,
MR imaging is used as a complementary modality for imaging
of LM when dealing with extensive lesions.
Similar to VM, treatment of LM is indicated in pediatric
patients with pain, disfigurement, or functional impairment.
Surgical resection may offer definitive therapy, although it
can be challenging when lesions have poorly defined borders
or involve critical structures. Image-guided sclerotherapy is
the first-line treatment for macrocystic LM [22]. Medical
therapy with oral and topical agents such as sirolimus can be
given adjunctively and is also used as primary therapy to
control microcystic LM and certain other LMs involving
large areas [41, 42]. As noted in the previous section, siroli-
mus also plays a role in the treatment of VM, as well as some
vascular tumors [39, 43].
Osseous and Cartilaginous Lesions
Osteochondroma (Exostosis)
Osteochondroma or exostosis is the most common benign
bone tumor; however, it is rare in the chest wall because it does
not usually arise from membranous bone. Osteochondroma is
+ +
+
and fluid-debris levels (arrows) are present in other locules due to layer-
ing blood products. (b) Transverse color Doppler ultrasound image
shows flow within a cyst wall. Posterior acoustic enhancement is also
demonstrated, confirming that the lesion is fluid-filled
252
a b
+
+
R + 2.9 cm
Fig. 8.19 Lymphatic malformation with internal hemorrhage in a
12-month-old male who presented with an enlarging chest wall mass.
(a) Longitudinal grayscale ultrasound image demonstrates a thin-
walled multilocular lesion (calipers) in the subcutaneous tissue which
contains cystic spaces of different size. There is also varying echo-
an osteocartilaginous lesion consisting of a bony excrescence
that is continuous with the cortex and medulla of the parent
bone, overlaid by a cap of hyaline cartilage. Chest wall osteo-
chondromas are present in 50% of patients with multiple
hereditary exostoses (MHE) beyond the age of 2 years and
can be the presenting abnormality [44, 45]. Osteochondroma
formation can also be induced by radiation therapy to the
chest as in patients treated for lymphoma [46].
Osteo­chondromas can arise from any bone, including the
ribs, scapula, clavicle, posterior elements of the spine, and
rarely the sternum [47]. Rib lesions usually develop at the
costochondral junction, a metaphyseal equivalent [48, 49].
Chest wall osteochondromas can present with cosmetic
deformity or pain from impingement of other structures,
and bursa formation has been reported with scapular
lesions [50]. Rarely, costal lesions can result in complica-
tions such as pleural effusion, hemothorax, pneumothorax,
or pericardial effusion [44, 51]. Thickness of the cartilage
cap greater than 2 cm raises suspicion for malignant degen-
eration to chondrosarcoma [52, 53].
Diagnosis of osteochondroma is usually made by plain
radiography. However, when it arises from the external sur-
face of a rib, it may be encountered during ultrasound exami-
nation of a palpable chest wall lump. Ultrasound demonstrates
a calcific protuberance that causes posterior acoustic shad-
owing, and the echogenic line representing the cortical sur-
face is seen to be continuous with the underlying bone
(Fig. 8.20) [54]. The cartilage cap is well visualized by ultra-
sound because it is hypoechoic and easily distinguishable
from the underlying bone and overlying fat, allowing for
accurate measurement of cap thickness [54, 55].
Ultrasound is limited in visualizing lesions that arise from
the internal surfaces of the thoracic skeleton because they are
inaccessible to the transducer. In such cases, MR imaging
can be used to identify the lesion, define the cartilage cap,
and assess any effects on adjacent structures.
J. Kurian
+
+
R
+ 1.7 cm
genicity of the cysts, and a fluid-debris level is present (arrow), consis-
tent with recent hemorrhage. (b) Longitudinal grayscale ultrasound
image of the same region 2 months later after symptoms resolved shows
interval contraction of the malformation. R, Rib
M
M
R
Fig. 8.20 Osteochondroma of the rib in a 4-year-old male who pre-
sented with an enlarging palpable mass of the left chest wall.
Longitudinal grayscale ultrasound image demonstrates a shadowing
bony excrescence (white arrow), which arises from a rib (R) and bulges
the contour of the overlying skeletal muscle (M). The cortex (arrow-
heads) of the rib is in continuity with the lesion. An overlying thin
hypoechoic cartilage cap (black arrow) is visible
In most cases, osteochondroma does not require treatment
and stops growing when the child reaches skeletal maturity.
In patients with cosmetic deformity or symptoms from
mechanical compression, local resection can be performed,
and video-assisted thoracoscopic surgery (VATS) is often a
suitable surgical approach for chest wall lesions [44].
Although treated lesions in the long bones are monitored for
potential regrowth, in the chest wall, osteochondromas gen-
erally do not recur after excision [44, 54].
 symmetric Cartilaginous Costochondral
A
Junction
Anatomic variations of the chest wall are the most common
cause of palpable, firm, non-tender chest wall masses in chil-
dren and are present in up to one-third of patients [56, 57].
The most frequent finding is unilateral angular deformity of
the costochondral junction [58]. This usually occurs at a sin-
8 Chest Wall
C C
Fig. 8.21 Paracostal nodule in a 6-month-old male who presented with
a palpable chest wall mass. Sagittal grayscale ultrasound image demon-
strates a solitary rounded hypoechoic nodule (arrow) overlying the cos-
tal cartilages (C). The lesion demonstrates identical echogenicity to that
of the adjacent costal cartilages, supporting the diagnosis of a cartilagi-
nous nodule
gle level but can be multilevel and is sometimes seen along
with hypertrophy of the costal cartilage. Although most
causes of anterior chest wall masses in children are benign,
either due to anatomic variations such as cartilaginous asym-
metry or benign tumors such as osteochondroma, these
patients are often sent for imaging to exclude malignancy.
Other developmental variants of the chest wall that may
present as a firm mass include anterior convexity of the ribs,
tilted sternum, parasternal chondral nodules, and mild pectus
deformities (Fig. 8.21) [56, 57]. No treatment is required.
Ultrasound is an excellent diagnostic tool for targeted eval-
uation of costochondral cartilage deformities and is especially
useful when a child presents with a firm anterior chest wall
mass and negative radiography [58]. Ultrasound is superior to
radiography because of its high-­contrast resolution for carti-
lage, and it precludes the need for CT or MR imaging.
The costochondral junction is located in the parasternal
region, with the cartilage appearing hypoechoic and continu-
ous with the ossified portion of the rib that demonstrates pos-
terior acoustic shadowing (Fig. 8.22). When scanning, it is
imperative to obtain images of the contralateral chest wall at
the same level to assess asymmetry of an angular deformity
(Fig. 8.23) [58]. It is also helpful to compare the costochon-
dral junction to the levels above and below the area of
concern.
 nlarged Rib Ends
E junction of one of the upper ribs, which is distinguished from
An enlarged rib end is a nonspecific finding that can be seen
in a variety of conditions. Diffuse enlargement of the rib
ends at the costal cartilages is one of the classic radiographic
253
C
R
Fig. 8.22 Normal costochondral junction in a 12-year-old male.
Transverse grayscale ultrasound image of the right parasternal region
shows the normal appearance of the costochondral junction (arrow).
The ossified portion of the rib (R) has a posterior acoustic shadow,
while the cartilaginous portion of the rib, i.e., the costal cartilage (C), is
hypoechoic with thin echogenic borders. Since the cartilage is non-­
shadowing, an echogenic border is visible along both the anterior and
posterior margins of the cartilage, unlike the ossified rib where only the
anterior surface can be seen
findings of rickets. In vitamin D-deficient rickets, there is
failed mineralization of osteoid due to low calcium and
phosphate levels, resulting in loss of the zone of provisional
calcification. This leads to overgrowth of mature cartilage at
the costochondral junction and a flared appearance at the
ends of the ribs. On physical examination, the enlarged rib
ends appear as rows of nodules on the anterior chest wall,
reminiscent of a string of beads and termed a “rachitic
rosary.”
The differential diagnosis for diffusely enlarged rib ends is
a variety of metabolic and genetic disorders, including scurvy,
in which there is a sharp angular step-off at the costochondral
junction termed a “scorbutic rosary,” chondrodystrophies (i.e.,
certain skeletal dysplasias such as achondroplasia), acromeg-
aly, mucopolysaccharidoses, and others [45, 59].
If a single enlarged rib end occurs as a solitary finding,
anatomic variation is the likely cause (similar to that
described in the previous section). However, other consider-
ations include benign tumors (e.g., osteochondroma), malig-
nant tumors, traumatic sequelae, and inflammatory processes.
Tietze syndrome is a rare inflammatory condition causing
painful firm enlargement of the cartilage at the costochondral
costochondritis by the presence of swelling and other signs
of inflammation [59–61].
254
Fig. 8.23 Asymmetry of the costal cartilage in a 2-year-old female
who presented with a palpable right anterior chest wall mass. Transverse
(a) and sagittal (b) grayscale ultrasound images of the right chest wall
in the area of concern. Comparison transverse (c) and sagittal (d) views
a c
Fig. 8.24 Solitary enlarged rib end in a 5-year-old male who presented
with a 1-year history of a painless, palpable left chest wall mass. (a)
Sagittal grayscale ultrasound image shows that the area of concern cor-
responds to a rib (white arrows), which is larger in size compared to the
rib (arrowhead) at the level below. (b) Sagittal grayscale ultrasound
J. Kurian
of the left chest wall demonstrate an asymmetrically thicker costal car-
tilage (white arrows) on the right, with a prominent outward convexity
that is not seen on the left (black arrow). S, Sternum
image obtained more medially through the anterior cartilaginous por-
tion demonstrates that the rib divides distally into two costal cartilages
(black arrows). (c) Frontal chest radiograph shows the corresponding
expansion (arrow) of the end of the left fourth rib. These findings repre-
sent an anatomic variant of no clinical significance
8 Chest Wall
In cases of diffuse rib end enlargement such as rickets and
genetic syndromes, a diagnosis is usually made on the basis
of other clinical and radiologic findings. However, in condi-
tions that affect a solitary rib, affected patients may present
for ultrasound assessment. Since many of these disorders are
related to the morphology of the costochondral junction,
ultrasound is very useful in localizing the abnormality to the
cartilage and determining whether it is due to simple ana-
tomic variation or a pathologic process that requires further
imaging. Anatomic variants that may cause enlarged rib ends
and can be identified by ultrasound include bifid or forked
ribs, congenital or acquired fusion anomalies, and supernu-
merary articulations between ribs (Fig. 8.24) [62].
Infectious Disorders of the Chest Wall
Cellulitis
Cellulitis is an acute bacterial infection of the soft tissues,
which can involve the skin, subcutaneous fat, and superficial
fascia but spares the underlying muscle and deep fascia [63].
It usually results from a disruption of the skin barrier, as with
penetrating wounds and insect bites [64]. The most common
causative agents are Staphylococcus aureus or Streptococcus
pyogenes. The incidence of cellulitis and other soft tissue
infections has risen dramatically in the twenty-­first century
due to the emergence of methicillin-resistant S. aureus
(MRSA). Cellulitis is a clinical diagnosis, based on patient
history and physical findings of swelling, erythema, pain,
warmth, fever, and chills. However, ultrasound may be
requested to distinguish between uncomplicated cellulitis
a SC
M M
Fig. 8.25 Cellulitis in a 5-year-old female who presented with pain,
swelling, and warmth of the right anterior chest wall. (a) Transverse
grayscale ultrasound image demonstrates increased echogenicity of the
subcutaneous (SC) layer, which is edematous, with fluid tracking
255
and purulent cellulitis or abscess, especially when the clini-
cal examination is indeterminate or standard treatment has
failed [64, 65]. Timely diagnosis of purulent infections is
important to prevent extension of ­disease to muscle (pyo-
myositis), bone (osteomyelitis), or systemic spread (sepsis).
Cellulitis of the chest wall follows the same imaging pat-
terns as it does for other areas of the body. In its early stages,
cellulitis demonstrates soft tissue thickening, which later
(>3 days) progresses to tissue “disarray” [66, 67]. The most
common ultrasound finding in cellulitis is “cobblestoning”
or branching interstitial fluid, representing hypoechoic fluid
that interdigitates between subcutaneous fat lobules in a pat-
tern reminiscent of a cobblestone street (Fig. 8.25) [67, 68].
The subcutaneous tissue also becomes thickened and
hyperechogenic, and there is loss of normal architecture with
blurring of the planes between the epidermis, dermis, and
hypodermis [68]. Color Doppler can demonstrate hyper-
emia, which is a sign of inflammation.
As with all ultrasound studies of the chest wall, compari-
son to the unaffected side will help in recognition of these
abnormalities, especially if they are subtle. The ultrasound
findings must be interpreted in consideration of the clinical
findings, as some of these features are nonspecific; for exam-
ple, interstitial fluid is also seen in soft tissue edema from
noninfectious causes (e.g., deep venous thrombosis, lymph-
edema) [69].
In most cases of community-acquired uncomplicated celluli-
tis, affected children can be treated with oral antibiotics. In more
severe infections, including those associated with lethargy,
hypotension, and other signs of systemic toxicity, hospitaliza-
tion and parenteral antibiotics are indicated [64, 70]. Necrotizing
fasciitis or gangrenous infections require surgical debridement.
b SC
between the fat lobules, giving rise to a “cobblestone” appearance. (b)
Transverse grayscale ultrasound image demonstrates fluid tracking in
the plane of the superficial fascia (arrowheads) at the deepest extent of
the infectious process. M, Muscle
256
Abscess
Abscess is an uncommon manifestation of chest wall infec-
tions in children. A chest wall abscess may develop from
progression of purulent cellulitis or as a result of open
wounds or thoracic surgery [71]. Children with cellulitis can
form abscesses in 48 hours or less [65]. An abscess is a local-
ized collection of pus (white blood cells and bacteria), which
is organized into a necrotic central core surrounded by neu-
trophils, dilated blood vessels, and proliferating fibroblasts.
The clinical presentation is similar to cellulitis in terms of
localized inflammation. However, in abscesses, physical
examination can demonstrate a palpable indurated or nodu-
lar area, with fluctuance. Edema and/or cellulitis of the sur-
rounding soft tissues is present.
The ultrasound appearance of an abscess, which is not spe-
cific to the chest wall, is of a focal fluid collection of variable
echogenicity (Fig. 8.26). In a classic abscess, the collection is
spherical and surrounded by a thick capsule demonstrating
hyperemia. Swirling of fluid during transducer compression
indicates the presence of pus and should be captured with a
cine clip (Fig. 8.27) [69]. It is important to recognize that not
all purulent infections result in classic abscess formation [67].
Some purulent collections, particularly in MRSA infections,
are small and irregularly shaped, with ill-defined borders [72].
Additionally, purulence with drainage may be clinically appar-
ent even in the absence of a visible fluid collection on ultra-
sound [67].
Ultrasound plays an important role in assessment of chest
wall soft tissue infections, since identification of an abscess
indicates the need for incision and drainage. Antibiotics may
be considered as adjunctive therapy [70]. Repeat ultrasound
can be performed to assess response to therapy, if needed.
a b
+
+
Fig. 8.26 Abscess in a 17-year-old female who presented with a pain-
ful lump on the upper back. (a) Longitudinal grayscale ultrasound
image demonstrates a rounded fluid collection (calipers) in the subcuta-
neous tissue with a thick, irregular echogenic wall. Posterior acoustic
enhancement is present indicating the cystic nature of the lesion. There
J. Kurian
Neoplastic Disorders of the Chest Wall
In general, the role of imaging in the workup of chest wall
tumors is primarily for localization and extent of disease, as
many of the imaging features are nonspecific and tissue sam-
pling is required for definitive diagnosis. MR imaging is
often the imaging modality of choice for its high tissue con-
trast, ability to depict the entire extent of the mass, and iden-
tify osseous involvement. However, ultrasound is often
performed as the first step in the workup of a clinically suspi-
cious mass, and it is a useful tool for “triage” of cases into
benign or possibly malignant categories [73].
Concerning clinical features include new onset or pro-
gressive enlargement, pain, size greater than 5 cm, and deep
location. Ultrasound features of malignant lesions include
Fig. 8.27 Abscess in a 16-year-old female who presented with a
nodular area of fluctuance of the anterior chest wall. Transverse color
Doppler ultrasound image shows an ovoid fluid collection with poste-
rior acoustic enhancement and peripheral vascularity. The fluid contains
numerous low-level echoes, which on real-time scanning demonstrated
swirling when light pressure was placed on the transducer. This finding
indicates the presence of pus
is inflammation of the surrounding fat with thickening and hyperecho-
genicity. (b) Transverse color Doppler ultrasound image shows some
flow at the edges of the collection and hyperemia of the surrounding
subcutaneous tissue
8 Chest Wall
heterogeneous echotexture, distortion of surrounding anat-
omy, cortical bone destruction, and “chaotic” or disorga-
nized Doppler flow (neoangiogenesis) [73, 74]. Ultrasound
findings can suggest a preoperative diagnosis when they are
taken into consideration together with clinical, radiographic,
and MR imaging findings [75].
As mentioned earlier in this chapter, CEUS is a promising
tool for evaluation of soft tissue masses. Either the presence
of homogeneous enhancement or the absence of enhance-
ment correlate with benignity. Heterogeneous enhancement
is associated with malignancy [10, 11]. Recent work with
ultrasound contrast agents has shown that targeting of
enhancing regions improves the diagnostic yield of biopsy
[76]. Also promising are early investigations in the pediatric
population that indicate that CEUS can be used to assess
treatment response of solid malignancies [77].
Benign Chest Wall Neoplasms
Lipoma
Lipoma is a benign tumor composed of mature fat cells and
is the most common soft tissue neoplasm [78]. The chest
wall and trunk are relatively common sites for benign lipo-
mas, especially the upper back. Most lipomas are superficial,
i.e., subcutaneous, and are smaller than 5 cm [78, 79]. Deep
lipomas can arise below the superficial fascia and be located
within or between muscles. However, these are less frequent
and are especially rare in the chest wall [78–80]. The term
lipomatosis refers to an unencapsulated version which is dif-
fusely proliferative and infiltrates the musculature.
Clinical examination is usually sufficient for diagnosis of
a superficial lipoma, which is palpable as an ovoid, mobile,
rubbery, or “doughy” mass [5, 78]. On ultrasound, superfi-
a b
+
+
LD
Fig. 8.28 Lipoma in a 12-year-old female who presented with a soft
mobile mass of the left lateral chest wall. (a) Longitudinal grayscale
ultrasound image shows a well-defined oblong subcutaneous mass (cal-
ipers) overlying the latissimus dorsi (LD) muscle, with its long axis
257
cial lipomas are well circumscribed with a thin capsule,
oblong in shape, and relatively homogeneous in echotexture,
aside from occasional delicate hyperechoic bands oriented
parallel to the skin surface (Fig. 8.28) [78, 81].
The echogenicity of adipocytic lesions is variable, as it
depends on the amount of fat content relative to stroma or
other admixed tissue types. However, lipomas represent pure
fat except for the fibrous septa (parallel linear bands), and the
superficial form is usually isoechoic to the surrounding sub-
cutaneous fat [79]. Some lipomas are hyperechoic or lack the
parallel linear bands (Fig. 8.29). There is no posterior acous-
tic shadowing or enhancement. Although lipomas are sup-
plied by a rich vascular network, color Doppler flow is
usually undetectable, likely due to compression of vessels by
large adipocytes [78, 79, 81]
Management of lipoma depends on location and size. In
the appropriate clinical setting, ultrasound can be diagnostic
for lipomas under 5 cm. However, any lesions that are larger
and deep, have atypical features, or change clinically warrant
repeat ultrasound and/or MR imaging [5]. The majority of
superficial lipomas are asymptomatic and do not require
treatment; however, when indicated, surgical excision is
curative [79].
Deep lipomas are also treated with excision. Incomplete
resection and local recurrence are known complications,
due to the more expansile nature of these lesions [78]. Deep
lipomas are larger and more variable in appearance by
ultrasound than classic lipomas. They should be further
assessed with MR imaging for confirmation of diagnosis
and surgical planning [78, 81].
Mesenchymal Hamartoma
Mesenchymal hamartoma (MH) is an exceedingly rare lesion
arising in the chest wall of newborns and young infants, and
oriented parallel to the skin surface. The echotexture is reminiscent of
subcutaneous fat, and multiple thin echogenic bands are seen through-
out the lesion. (b) Color Doppler ultrasound image at the same level
shows no vascularity in the mass (minor flash artifact is noted)
258
Fig. 8.29 Lipoma with alternative appearance in a 19-year-old male
who presented with a long-standing posterior chest wall mass. (a)
Longitudinal grayscale ultrasound image demonstrates an oblong sub-
cutaneous mass (calipers), which is fairly homogeneous but mildly
occasionally identified in the prenatal period. MH is a benign
proliferation of skeletal tissue arising from a rib, with a
prominent cartilaginous component [82]. Characteristic fea-
tures include intrathoracic projection with a large extrapleu-
ral mass, internal calcification, and secondary aneurysmal
bone cyst (ABC) formation with multiple hemorrhage-filled
cavities. There is wide variability in clinical presentation of
MH, with some lesions detected incidentally in asymptom-
atic pediatric patients and others causing extreme respiratory
distress [83, 84]. Symptoms depend on the size of the lesion
and degree of mass effect on the lungs and heart. Physical
findings are also varied, ranging from a minor chest wall
deformity to a visible mass.
Ultrasound features of MH are nonspecific and have pri-
marily been reported for prenatal cases [85]. On prenatal
ultrasound, MH appears as a rounded, heterogeneous intra-
thoracic mass with a hyperechoic capsule, located adjacent
to the posterior ribs [85, 86]. The mass grows as the preg-
nancy ­progresses, and may become hypoechoic centrally,
presumably due to hemorrhage [85]. Large lesions with mass
effect can cause mediastinal shift and pulmonary hypoplasia
[86]. MH can be a source of pleural bleeding and a pleural
effusion may be identified on ultrasound [84, 85].
MH may be confused with a congenital lung lesion due to
its intrathoracic extension or with a malignant lesion due to its
size and distortion of the chest [84]. However, fetal MR imag-
ing can identify chest wall involvement and the presence of
secondary ABC and/or hemorrhage with fluid-fluid levels,
features which are highly suggestive of MH [82, 86]. Postnatal
ultrasound features have not been well-described. Radiographs
and CT are more useful in the postnatal period to demonstrate
the costal origin of the mass, erosion or molding of adjacent
bones, and osteoid or chondroid matrix [82, 84, 86].
Although MH appears aggressive on imaging and his-
topathology, it is a benign lesion and conservative man-
J. Kurian
hyperechoic relative to the adjacent muscle and fat. A few thin internal
echogenic bands are present. (b) Transverse color Doppler ultrasound
image shows lack of flow in the majority of the mass, except for one
focus of vascularity in its right lateral aspect
agement is favored. Lesions have usually reached peak
size at birth and shrink within the first 2 years of life [84,
85, 87]. However, clinically unstable patients require
tumor resection via thoracotomy and may also need
reconstruction of the chest wall [84]. Surgery should be
considered in children with substantial physical disfigure-
ment [82]. Complete resection is curative. Radiofrequency
thermoablation can also be performed [24, 82].
Malignant Chest Wall Neoplasms
The ultrasound features of chest wall malignancies described
below have been extrapolated from established features of
extremity tumors, because of their rare incidence in the chest
wall and paucity of specific literature, particularly in the
pediatric population.
Rhabdomyosarcoma
Rhabdomyosarcoma (RMS) is the most common soft tissue
sarcoma in children, accounting for one-half of all pediatric
sarcomas [88]. It arises from primitive mesenchyme that dif-
ferentiates into cells simulating striated muscle, and can
occur in any body part excluding bone [48, 89, 90]. In the
chest wall, it originates from the musculature, and the
absence of a costal origin helps distinguish it from other
types of sarcoma. Most pediatric chest wall RMS are of the
alveolar or embryonal subtype [89, 90].
RMS can occur at any age but is most common in young
children aged 2–5 years [89]. RMS of the chest wall has also
been identified by prenatal ultrasound [91, 92]. RMS pres-
ents as a mass that is usually non-tender on palpation
although it can be a source of chest pain [89, 93]. Signs and
symptoms of metastatic disease include lymphadenopathy,
fatigue, weight loss, and low blood counts.
8 Chest Wall
a b
X
+
Fig. 8.30 Rhabdomyosarcoma in a 21-month-old female who presented
with swelling of the chest wall. (a) Transverse grayscale ultrasound image
shows a solid chest wall mass (calipers) with heterogeneous echotexture
and poor definition of its right lateral margin. (b) Axial contrast-enhanced,
On ultrasound, RMS typically appears as a heteroge-
neous solid mass, which may have an irregular contour
(Fig. 8.30). Internal vascularity is present on color Doppler.
Tumor necrosis can occur, resulting in anechoic, avascular
cystic spaces. There is no shadowing calcification within
the tumor; however, 20% of affected patients have bone
invasion [94]. The embryonal subtype can invade the
mediastinum.
Prognosis of RMS depends on age, histologic type (with
alveolar type having the poorest prognosis), degree of dif-
ferentiation, DNA analysis, tumor size, and extent of dis-
ease, including resection margins [89, 90]. RMS of the chest
wall has an unfavorable prognosis, with a tendency for local
recurrence and metastasis [88, 90]. Treatment of RMS
requires a coordinated approach that includes chemotherapy,
surgery, and radiation [90]. When used as part of serial imag-
ing during therapy, ultrasound can assess changes in tumor
size, although there are no specific changes in tumor echotex-
ture that occur [95].
Ewing Sarcoma
The Ewing sarcoma (ES) family of tumors is the most com-
mon group of chest wall malignancies in the pediatric popu-
lation. It includes classic Ewing sarcoma of the bone,
extraosseous Ewing sarcoma, and primitive neuroectodermal
tumor (PNET) [96]. The extraosseous form is rare; however,
the chest wall is one of the most common locations, where it
is termed an Askin tumor [89, 97]. ES tumors are aggressive
small round blue cell tumors, thought to arise from embryo-
nal neural crest cells. PNET is considered to have more neu-
roectodermal differentiation than classic ES; however, 90%
259
T1-weighted, fat-suppressed MR image demonstrates the mass (arrows) in
the upper right chest wall and axilla, which has heterogeneous signal and
surrounding infiltrative enhancement. Biopsy showed rhabdomyosarcoma
with involvement of subpectoral lymph nodes
of tumors in the Ewing family share a common karyotype
abnormality with a balanced 11;22 chromosomal transloca-
tion [33, 89, 97].
The most common location for ES of the bone to arise in
the chest wall is from a rib. It can also develop from the ster-
num, scapula, and clavicle [96]. The peak age of incidence
is 10–15 years, and the presentation includes nonspecific
findings of pleuritic chest pain and swelling or a palpable
mass [97]. Fever and an elevated erythrocyte sedimentation
rate can also be present. These findings as well as the radio-
graphic appearance can mimic an infectious process.
Ultrasound can be used to identify the soft tissue compo-
nent of the tumor, which is typically larger than the intraos-
seous component (Fig. 8.31) [45]. With careful inspection,
the osseous origin of the tumor can be identified by demon-
strating that the mass surrounds the bone, causes cortical
irregularity or erosion, and exhibits a “sunburst” appearance
with an array of echogenic lines arising perpendicular to the
bone [98, 99]. The sunburst appearance corresponds to
aggressive periosteal reaction, which is also seen on radio-
graphs. Calcification in the soft tissue component is uncom-
mon [97]. Doppler vascularity is generally present, with high
arterial velocities in the periphery of the lesion [98–100].
Tumors with a relatively small extraosseous portion are
homogeneous and hypoechoic [101]. Large tumors undergo
necrosis and hemorrhage, becoming heterogeneous in
echotexture. In the case of necrotic tumors, ultrasound is
helpful in identifying high-­yield areas of non-necrotic solid
tissue for biopsy [97, 100].
In extraosseous ES, the soft tissue tumor is usually
hypoechoic and may contain anechoic regions of hemor-
260 J. Kurian

a b

Fig. 8.31 Ewing sarcoma of the rib in a 6-year-old boy with abdomi- with prominent posterior acoustic shadowing (arrowhead). (b) Contrast-
nal pain, weight loss and fever. (a) Transverse grayscale ultrasound enhanced axial computed tomography (CT) image shows the mass aris-
image shows a heterogeneous soft tissue mass encircling a rib (arrow) ing from the left 12th rib that displays lytic destruction (arrow)

rhage or necrosis [97, 102]. Intratumoral calcification can

occur in up to 25% of cases. Doppler vascularity is present.
Treatment of ES includes initial chemotherapy and surgi- +
cal resection, which may be followed by radiation therapy +
[90]. Wide excision usually includes parts of adjacent ribs,
musculature, and pleura, with large defects necessitating
chest wall reconstruction.
While ultrasound is not used for staging purposes, it can
be used to monitor the extraosseous portion of the tumor dur-
ing therapy. On MR imaging a decrease in tumor vascularity
and enhancement indicate response to chemotherapy [97].
Similarly, a decrease in intratumoral blood flow can be seen
on Doppler ultrasound, as well as increased arterial resistive
indices on spectral analysis [97, 100, 103]. However, Doppler
findings are not consistent across studies, and in general,
ultrasound is not widely used for the purpose of follow-up.
Fig. 8.32 Osteosarcoma of the chest wall in a 10-year-old female who
presented with known multifocal tumor. Transverse grayscale ultra-
Osteosarcoma sound image demonstrates an irregularly shaped mass (calipers) with
Osteosarcoma (OS) is a high-grade malignant, bone-forming coarse echogenic regions and strong posterior acoustic shadowing due
mesenchymal tumor predominantly affecting adolescents to osteoid matrix
and young adults. Primary thoracic OS is rare, but it can arise
from the ribs, sternum, scapula, or clavicle [4, 33, 93, 96]. can demonstrate a poorly demarcated, heterogeneous, mixed
OS usually presents as a painful mass. When it affects the echogenicity mass [100, 106]. Necrotic regions appear
chest wall, it can also cause respiratory distress [94, 105]. anechoic [101]. Central irregular hyperechoic areas with
The rare extraosseous form of OS can also present in the posterior acoustic shadowing correspond to osteoid matrix
chest wall but is more often seen in older adults after radia- that is seen on radiography (Fig. 8.32). In some cases, ultra-
tion or other toxic exposures [93, 104, 105]. sound can also demonstrate cortical destruction by identify-
Since OS is primarily intramedullary in location, the role ing defects in the echogenic surface of the bone, as well as
of ultrasound is limited. When ultrasound is performed, it the sunburst or “hair-on-end” pattern of periosteal reaction,
8 Chest Wall
Fig. 8.33 Lymphoma in an 18-year-old male who presented with a
palpable anterior chest wall mass due to relapsed Hodgkin’s disease. (a)
Transverse grayscale ultrasound of the palpable region demonstrates a
Fig. 8.34 Chest wall mass in a 2-year-old male with stage III
Burkitt lymphoma. Sagittal grayscale ultrasound image shows several
hypoechoic masses (arrows), some ovoid shaped and some ill-defined.
There is thickening and increased echogenicity of the surrounding soft
tissues
with echogenic spicules perpendicular to the bone similar to
those seen in ES [107].
On Doppler imaging, OS is hypervascular, with predomi-
nantly peripheral flow that demonstrates high velocity and
low resistance [100, 101]. However, tumors with >90%
necrosis show less neovascularity [108].
Treatment of OS usually consists of neoadjuvant chemo-
therapy and resection, sometimes followed by postoperative
chemotherapy [93]. OS is less sensitive to radiation compared
to other tumors, although radiation may be given after incom-
plete resection [90, 93]. OS of the chest wall has a poor prog-
nosis, with greater rates of recurrence and metastatic disease
compared to OS of the extremities [94, 104, 109].
261
solid lobular mass (calipers) with heterogeneous echotexture. (b) Axial
contrast-enhanced CT image through the lower chest reveals that the
lesion is a protruding left cardiophrenic nodal mass (arrow). L, Liver
Lymphoma
Lymphoma of the chest wall is unusual, accounting for only
2% of chest wall tumors. It usually occurs in adults, and inci-
dence rates in children are unknown [90, 94]. Non-­Hodgkin
lymphoma (NHL), specifically diffuse large B-cell lymphoma
(DLBCL), is the most common diagnosis, although Hodgkin
lymphoma as well as a wide variety of other lymphoid neo-
plasms can present with a chest wall mass [110–113].
Primary extranodal chest wall lymphoma can arise from
the bone, muscle, or breast. Secondary chest wall involve-
ment is more common and results from direct invasion of
lymphoma from the mediastinum, pleura, or lung [110, 114].
Widespread disseminated lymphoma can affect the lymph
node groups of the chest wall or spread to the ribs, sternum,
spine, muscle, or skin.
Presenting symptoms of chest wall lymphoma include
localized pain, palpable mass, or pathologic fracture. Of
note, leukemia, particularly acute lymphoblastic leukemia,
can have a similar presentation and is a diagnostic consider-
ation in children with chest wall pain or multiple destructive
bone lesions [115].
Chest wall lymphoma can be seen on ultrasound imaging
of the bone, muscle, or lymph nodes (Figs. 8.33 and 8.34). In
general, the ultrasound findings are nonspecific and cannot
be distinguished from other malignancies or even an inflam-
matory process [110]. Ultrasound can identify nodal enlarge-
ment, with the most common chest wall sites being the
pectoral and subpectoral groups [114].
Lymphoma of the bone tends to have an imaging appear-
ance similar to other small round blue cell neoplasms such as
ES, with permeative osseous destruction [110]. As described
for chest wall sarcomas, these lesions can potentially be
identified by ultrasound if a substantial extraosseous compo-
262
nent is present. Primary lymphoma of the muscle is a rare
entity that affects older adults and on ultrasound may appear
as a discrete intramuscular mass or diffuse infiltration and
enlargement of the muscle [110, 116]. This is a scenario in
which ultrasound of the contralateral muscle for comparison
is helpful in detecting the abnormality as an asymmetry.
Isolated chest wall lymphoma requires biopsy for diagno-
sis. Biopsy may not always be required in patients with
known lymphoma, but identification of chest wall involve-
ment is important for disease staging and treatment.
Chemotherapy is the mainstay of treatment, with or without
radiotherapy [90].
Metastases
Metastatic disease is responsible for a minority of chest wall
lesions in children [24]. Metastases to bones or soft tissues of
the chest wall usually occur in patients with known widely
disseminated disease [96]. The primary diagnostic consider-
ation is neuroblastoma, which can involve the chest wall
through direct extension from the posterior mediastinum or
through hematogenous metastases to the ribs or vertebrae
[24]. Other tumors that can metastasize to the chest wall
include rhabdomyosarcoma, lymphoma, and leukemia.
Soft tissue lesions generally demonstrate ultrasound fea-
tures similar to the primary tumor from which they origi-
nate, although most appear as a nonspecific solid mass
(Fig. 8.35). The majority of chest wall metastases consist of
Fig. 8.35 Metastatic disease of the chest wall in a 16-year-old female
with chronic liver disease and hepatocellular carcinoma. (a) Transverse
grayscale ultrasound image of the posterolateral left chest shows a het-
erogeneous soft tissue mass (calipers), which encompasses an area of
shadowing calcification (white arrow). The mass also contains an
J. Kurian
lytic bone lesions, for which cross-sectional imaging evalu-
ation is required.
Traumatic Disorders of the Chest Wall
Hematoma
A hematoma is defined as a localized extravascular collection
of blood. In the chest wall, a hematoma can occur in the sub-
cutaneous tissue or muscle. Etiologies include trauma, sur-
gery, bleeding disorders, and anticoagulation [6]. Substantial
trauma is usually required to cause a chest wall hematoma;
however, hemorrhage can also occur within chest wall neo-
plasms and vascular malformations, either spontaneously or
with minor trauma.
Intramuscular hematoma is a rare finding and can be seen
in the setting of a bleeding diathesis such as hemophilia or
after athletic injuries such as trauma to the pectoralis mus-
cles in weight lifters. Children with chest wall hematoma are
likely to experience localized pain and swelling with bruis-
ing over the site.
The ultrasound appearance of hematoma depends on its
location and age, although hematomas in different tissues
tend to show common changes on serial ultrasound examina-
tions [117]. In the subcutaneous tissues, acute hematomas
can have ill-defined borders and variable internal echo-
anechoic area of necrosis (black arrow). (b) Sagittal contrast-­enhanced
CT image of the thorax through the same region shows a destructive
soft tissue mass (arrows) centered at the left tenth rib. The remaining
spicules (arrowhead) of the rib inside the mass correspond to the calci-
fication seen on ultrasound
8 Chest Wall 263

a b

+
+

Fig. 8.36 Acute hematoma in a 20-year-old male who presented with overlying the left seventh rib (R) that is isoechoic to the muscle. (b)
chest wall swelling after trauma. (a) Longitudinal grayscale ultrasound Transverse power Doppler ultrasound image shows no flow within the
image shows an ovoid mass-like intramuscular structure (calipers) lesion, consistent with an early phase of hemorrhage

genicity (hypoechoic, intermediate, or hyperechoic) and are a

avascular on color Doppler (Fig. 8.36). As the hematoma M
X
subsequently liquefies, it tends to become hypoechoic or +
anechoic [6]. Fluid-debris levels may appear due to separa- M
tion of serum and cellular debris. Over the next 3–4 weeks,
the collection becomes more heterogeneous and can demon- +
strate septations due to fibrin strands and solid-appearing X
areas due to retracting clot (Fig. 8.37) [118, 119]. As the
hematoma resorbs, it becomes smaller with increased echo-
genicity at the periphery.
The time course for resolution is variable, and in some b
cases, hematomas become organized with a fibrous capsule
that prevents blood resorption, resulting in a palpable hard
mass. Ultrasound of organized hematoma can show a
whorled appearance, with variable amounts of liquid and
solid tissue, and even internal Doppler vascularity, making
the diagnosis more challenging.
Intramuscular hematomas of the chest wall can result
from muscle tears or tendon injuries and can appear as a
hyperechoic mass with shaggy margins inside the body of
the muscle or as diffuse enlargement of the muscle with loss
of normal filamentous echotexture [41, 120, 121]. With heal-
ing, there is a variety of outcomes, including hyperechoic
scar tissue, myositis ossificans with acoustic shadowing, and
intramuscular cysts [121].
Usually, hematomas spontaneously resolve with conser-
Fig. 8.37 Subacute hematoma in a 19-year-old male who presented
vative management. Ultrasound-guided aspiration can be with chest wall swelling after trauma. (a) Extended field of view longi-
considered when more rapid resolution is needed [122]. tudinal grayscale ultrasound image demonstrates a large heterogeneous
Rarely, hematomas may instead slowly expand, a phenome- collection (calipers) in the pectoralis musculature (M). (b) Transverse
non that is termed chronic expanding hematoma [123]. This grayscale ultrasound image reveals low-level particulate echoes through-
out the collection, as well as irregular echogenic, solid-appearing mate-
lesion is thought to be caused by bleeding from proliferating rial (asterisks) due to retracting clot
264 J. Kurian

vessels within the granulation tissue along the fibrous cap-

sule of the hematoma [124]. Ultrasound demonstrates a non-
specific complex mass with peripheral solid nodules and
vascularity, and biopsy may be needed to distinguish this
lesion from a sarcoma [125]. There is little literature on this
in children. It has been reported to occur in the chest wall of
adults after trauma and surgery [126–128].

Rib Fracture

Rib fractures are the most common traumatic injury of the

thorax and are typically caused by blunt force [129]. Other
etiologies include stress/overuse injury, pathologic fractures
due to demineralization or neoplasm, and child abuse. The
most common presenting symptom is pain.
Although plain radiographs are usually obtained to
assess acute rib fractures, 50% of fractures may not be
visible at presentation [61, 129]. Additionally, fractures of
the costal cartilage cannot be demonstrated on radiogra-
phy. Ultrasound is more sensitive than radiography and
can be a useful tool in these situations [130, 131].
The primary ultrasound finding of acute rib fracture is
a discontinuity of the linear echogenic line of the anterior
cortex, which may also have a step-off depending on the
degree of displacement [61]. This is best demonstrated
with the probe held parallel to the long axis of the rib
[132]. Costal cartilage fractures have similar findings
except that the cartilage margins are thinner and less
echogenic than the bone [133].
Some rib fractures with prominent discontinuity may
demonstrate a focal acoustic shadow at the level of the frac-
ture, a sign that has been referred to as the “chimney phe- Fig. 8.38 Healing rib fracture in a 5-year-old male who presented with
nomenon” [61]. Hypoechoic fluid collections representing a painful mass and history of injury 2 weeks previously. Radiographs at
acute hematoma may be seen adjacent to the fracture. It is the time of injury were negative. (a) Transverse grayscale ultrasound
image of the palpable area demonstrates coarse calcification with strong
helpful to also screen for adjacent fractures by scanning the posterior acoustic shadowing typical of bone. (b) Longitudinal gray-
rib levels above and below and to perform a brief ultrasound scale ultrasound image at the same level shows that the mass (arrows)
survey of the ipsilateral thoracic cavity to identify any asso- overlies an angular deformity of a rib (R) and represents the callus of a
ciated pleural effusion or hemothorax. Fractures of the lower healing occult fracture
ribs (ribs 9–12) can also have associated injuries of the liver
or spleen, which may be recognized incidentally during While ultrasound is more sensitive than radiography for
scanning [129]. rib fracture, in current practice, it is not routinely used for all
At times, the only indication of rib fracture is the presence cases of thoracic trauma. Its most efficient use is probably in
of signs of healing on follow-up imaging. In the healing stage the setting of minor trauma, to identify rib fractures in hemo-
of rib fracture, ultrasound can demonstrate callus formation, dynamically stable patients in whom there is high clinical
which appears as a calcific prominence of the rib with pro- suspicion but negative radiography. Tenderness during scan-
nounced acoustic shadowing (Fig. 8.38). In the absence of ning, obesity, and breast tissue can sometimes be limiting fac-
reported trauma, healing fractures on ultrasound can mimic tors [132]. In pediatric patients with higher-level trauma, CT
other rib lesions. For example, occult costal cartilage frac- should be used instead as the initial modality as it is sensitive
tures can present weeks or months after initial injury as a for fractures as well as associated complications.
parasternal mass and be mistaken for malignancy on imaging Uncomplicated rib fractures are managed conservatively
and histology [133, 134]. Therefore, the ultrasound findings with pain control. The presence of multiple fractures is con-
should be interpreted in the context of a detailed clinical sidered an indicator of more severe trauma, and additional
history. interventions may be required for concomitant injuries.
8 Chest Wall
Examples include thoracostomy for pneumothorax or hemo-
thorax, intubation of patients with respiratory distress due to
chest wall instability, and embolization of active bleeding
from the intercostal arteries.
Foreign Bodies
Foreign bodies of the chest wall are rare but may encom-
pass a broad range of items. In children, potential sources
of retained foreign bodies include penetrating wounds
(e.g., knives, glass), traffic accidents, gunshot injuries,
acupuncture, and others [135, 136]. Foreign bodies can
also result from self-embedding behavior, a rare form of
self-injury seen in adolescents with psychiatric illness
[135, 137]. Pediatric patients with retained foreign bodies
usually present with pain at the time of the inciting injury.
In some cases, foreign bodies are unrecognized and may
not become symptomatic until long after the initial wound
has healed [135].
Iatrogenic foreign bodies that may be seen in the pediatric
chest wall include therapeutic devices such as subcutaneous
chemotherapy ports, tunneled central lines, ventriculoperito-
neal shunt catheters, vagal nerve stimulators, cardiac pace-
makers, implantable defibrillators, and orthopedic hardware.
a b
Fig. 8.39 Retained foreign body in a 16-year-old male who presented
with chest wall pain and a remote history of a gunshot wound. (a) Sagittal
grayscale ultrasound image of the left anterior chest wall demonstrates
punctate and linear echogenic foci (white arrow) with posterior acoustic
265
Certain foreign bodies such as wood and plastic are non-­
radiopaque. Ultrasound offers an advantage over plain radi-
ography for detection of these objects. It can also be helpful
in providing more accurate localization of radiopaque foreign
bodies. In addition, ultrasound is an important tool for assess-
ing complications such as infection (abscess) and granuloma
formation. Foreign bodies appear as hyperechoic structures
with clean or dirty posterior acoustic shadowing, commonly
linear in shape (Figs. 8.39 and 8.40) [122, 135, 138, 139].
Orientation of the probe parallel to the foreign body pro-
vides the best ultrasound demonstration of its length; how-
ever, perpendicular scanning is optimal for identifying the
acoustic shadow [138]. Metal and glass objects can produce a
reverberation artifact or “comet-tail” artifact [135].
Foreign bodies that have been retained for 24 hours or
more can elicit a surrounding inflammatory reaction [138].
On ultrasound, a hypoechoic halo can develop around the
object, representing edema, granulation tissue, or fluid col-
lection (Fig. 8.39). Ultrasound can also detect fluid collec-
tions and signs of infection associated with therapeutic
devices of the chest wall (Fig. 8.40).
The decision to remove a chest wall foreign body depends
on multiple factors. Indications include pain, infection, and
migration, as well as sharp objects positioned in critical loca-
tions, e.g., near pleura, nerves, or blood vessels. Organic for-
shadowing (black arrows), within the pectoralis major muscle. There is an
irregular fluid collection (arrowheads) surrounding the foreign body.
R, Rib. (b) Coned-down view of a frontal chest radiograph shows metallic
shrapnel in the chest which corresponds to the ultrasound findings
266
a b
Fig. 8.40 Retained foreign body in a 15-year-old female who presented
with chest wall pain and drainage after pacemaker removal. (a) Sagittal
grayscale ultrasound image through the left anterior chest wall shows two
echogenic structures (arrow and arrowhead) with posterior acoustic shad-
owing, representing abandoned pacemaker leads. Reverberation artifact
from one of the foreign bodies (arrow) is visible, which can be caused by
eign bodies (e.g., wood) and contaminated wounds are
particularly prone to infection [139]. In an appropriate clini-
cal setting, ultrasound-guided percutaneous removal can be
attempted. Surgery is an alternative option; however, ultra-
sound is still useful to provide guidance for marking the inci-
sion site.
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 Diaphragm
9
Wendy G. Kim, Helen H. R. Kim, Grace S. Phillips,
and Edward Y. Lee

Abbreviations it allows for dynamic imaging of anatomic and functional

abnormalities. Ease of access and lack of ionizing radiation
B Brightness make ultrasound an appropriate first-line diagnostic modal-
BH Bochdalek hernia ity, particularly in critically ill pediatric patients.
CDE Congenital diaphragmatic eventration This chapter provides an overview of the ultrasound evalu-
CDH Congenital diaphragmatic hernia ation of the diaphragm in the pediatric population, beginning
DP Diaphragmatic paralysis with up-to-date ultrasound imaging techniques, diaphrag-
ECMO Extracorporeal membrane oxygen matic embryology, and normal anatomy. The remainder of
HH Hiatal hernia the chapter focuses on the ultrasound findings and relevant
M Motion clinical features of congenital and acquired diaphragmatic
MH Morgagni hernia disorders in the pediatric population.
TDR Traumatic diaphragmatic rupture

Introduction
The diaphragm is a skeletal muscle that separates the tho-
racic and abdominal cavities and is essential for the normal
development of the thoracic and abdominal viscera. The dia-
phragm also performs critical functions in respiration and
digestion. Congenital or acquired disorders of the diaphragm
cause substantial morbidity and mortality in the pediatric
population, and prompt diagnosis is essential. Ultrasound is
best suited for evaluation of the pediatric diaphragm because
W. G. Kim
Department of Radiology, Boston Children’s Hospital and Harvard
Medical School, Boston, MA, USA
H. H. R. Kim
Department of Radiology, Seattle Children’s Hospital, University
of Washington School of Medicine, Seattle, WA, USA
G. S. Phillips
Department of Radiology, Seattle Children’s Hospital, University
of Washington, Seattle, WA, USA
E. Y. Lee (*)
Division of Thoracic Imaging, Department of Radiology,
Boston Children’s Hospital and Harvard Medical School,
Boston, MA, USA
e-mail: edward.lee@childrens.harvard.edu
Technique
Patient Positioning
Ultrasound evaluation of the diaphragm is best performed
with the patient in a supine position, which allows for greater
diaphragmatic excursion and greater reproducibility. This is
thought to be due to decreased gravitational impedance of
abdominal visceral movement and decreased compensatory
active breathing compared to the upright or sitting positions
[1]. Sitting or lateral decubitus positions can sometimes be
helpful to optimize the ultrasound window in pediatric
patients with external support devices or dressings along the
anterior chest wall.
Ultrasound Transducer Selection
The optimal ultrasound transducer frequency should be tai-
lored to both patient size and the specific clinical question.
High-frequency (9–17 MHz) linear array transducers can
provide optimal spatial resolution and are particularly useful
in infants and small children. Low-frequency (2–6 MHz)
convex or sector transducers allow greater depth of penetra-
tion and field of view and are useful for examination of older

© Springer Nature Switzerland AG 2021 271

H. J. Paltiel, E. Y. Lee (eds.), Pediatric Ultrasound, https://doi.org/10.1007/978-3-030-56802-3_9
272
children and adolescents and also for the evaluation of dia-
phragmatic excursion [1, 2].
Imaging Approaches
An oblique transverse subxiphoid approach allows for dynamic
evaluation of symmetric diaphragmatic excursion. Sector trans-
ducers are typically better suited for this approach, permitting
visualization of both medial hemidiaphragms in the ultrasound
window. Evaluation of critically ill, ventilated pediatric patients
should be coordinated with the respiratory therapist to assess
diaphragmatic excursion both on and temporarily off ventilator
support. Bilateral longitudinal transdiaphragmatic approaches
are helpful for anatomic evaluation of each hemidiaphragm
using either the liver, spleen, or stomach as an acoustic
window.
Conventional brightness (B)-mode ultrasound is typically
used to qualitatively evaluate the diaphragm and peridia-
phragmatic structures. Motion (M)-mode ultrasound can also
Septum
transversum
DAY 27
Septum
transversum
T1
Pleuroperitoneal
membrane
L1
DAY 43
Fig. 9.1 Development of the diaphragm. Diagram illustrating fetal
development of the diaphragm occurring between the 4th and 12th
weeks of gestation. The transverse septum originates anteriorly and
W. G. Kim et al.
be used for quantitative measurements of both the amplitude
and direction of motion of diaphragmatic excursion. Normal
excursion should be greater than 4 mm, and usually there is
less than a 50% difference in excursion between the hemidia-
phragms [3, 4]. Color Doppler imaging is useful for charac-
terizing juxta-diaphragmatic masses or vascular structures.
Normal Development and Anatomy
Normal Development
The diaphragm forms between the 4th and 12th weeks of
life from four main embryologic precursors. These include
the transverse septum centrally, the bilateral pleuroperito-
neal folds posterolaterally, the body wall musculature
peripherally, and the esophageal mesoderm posteromedi-
ally (Fig. 9.1). A failure of either development or appro-
priate fusion of these structures can result in a congenital
diaphragmatic hernia [5].
Septum
transversum
Liver
DAY 34
Septum
transversum
T1
L1
Pleuroperitoneal
membrane
DAY 54
forms the central portion of the diaphragm. The pleuroperitoneal mem-
branes form posterolaterally on either side, while the mesoderm devel-
ops into the body wall musculature and esophageal mesoderm
9 Diaphragm 273

Sternum

Vena cava
Central tendon passing through
of diaphragm caval opening

Esophagus
passing through
esophageal hiatus

Aorta passing
through aortic
Diaphragm hiatus

12th (floating) ribs

Left psoas major

Vertebrae

Left quadratus
lumborum

Fig. 9.2 Anatomy of the diaphragm. Illustration of the diaphragm as vertebral level, the esophagus traverses the esophageal hiatus at the T10
viewed from below shows the attachments of the diaphragm to the chest vertebral level, and the aorta passes through the aortic hiatus at the T12
wall and spine. The caval opening is located at approximately the T8 vertebral level

Normal Anatomy

The diaphragm is a thin, domed musculotendinous struc-

ture separating the abdominal and thoracic cavities. The
fibrous central tendon constitutes the apex of the domed
diaphragm, while the radially oriented muscular fibers
extend laterally to the ribs at their costal attachments. The
crural diaphragm is thicker and located posteromedially,
surrounding the esophagus and aorta and attaching to the
upper lumbar vertebrae. The right and left crura are joined
by the median arcuate ligament. The medial and lateral
arcuate ligaments attach the posterior diaphragm to the
spine and 12th ribs, while the anterior and lateral attach- Fig. 9.3 Normal appearance of the diaphragm in a 14-day-­old female.
ments include the inferior sternum, xiphoid, lower six Transverse grayscale ultrasound image at a subxiphoid midline position
demonstrates a symmetric appearance of the posterior hemidiaphragms
ribs, and costal cartilages (Fig. 9.2) [6]. (arrows) which are depicted as echogenic curved lines. The hypoechoic
The right and left hemidiaphragms are innervated by the diaphragmatic crura (arrowheads) can also be seen at midline draping
right and left phrenic motor nerves (C3–C5) and vascularized over the aorta (asterisk)
by the phrenic, internal thoracic, and intercostal arteries.
On ultrasound, the diaphragm appears as a thin hypoechoic between the lung and abdominal viscera due to ultrasound
muscular sheath between echogenic layers of peritoneum reverberation artifact between the lung and diaphragm [7].
and pleura (Fig. 9.3). With low-frequency transducers, the During inspiration, the diaphragm moves inferiorly, and the
diaphragm frequently appears as a single echogenic layer muscle fibers appear to thicken [1].
274 W. G. Kim et al.

Congenital Diaphragmatic Anomalies Bochdalek Hernia

Diaphragmatic Hernia
Congenital diaphragmatic hernia (CDH) is a focal defect in
the diaphragm that can result in displacement of the abdomi-
nal contents into the thoracic cavity. CDH occurs in approxi-
mately 0.02–0.06% of births and continues to have substantial
associated morbidity and mortality despite recent medical
advances [8, 9].
Clinical prognosis is usually determined by the degree of
pulmonary hypoplasia and functional derangement of the
lungs and associated structures. Additional anomalies are
present in up to 50% of patients with CDH, including neural
tube defects, cleft palate, congenital heart disease, esopha-
geal atresia, and genetic syndromes, and often portend a
worse prognosis [10].
CDH is classified by the location of the diaphragmatic
defect, with a Bochdalek hernia in the posterolateral dia-
phragm, a Morgagni hernia in the anteromedial parasternal
diaphragm, and a hiatal hernia occurring centrally through
the esophageal hiatus (Fig. 9.4, Table 9.1).
Bochdalek hernia (BH) is the most common type of CDH
(approximately 90% of cases) and usually affects the left
hemidiaphragm [9, 10]. It is thought to occur secondary to a
developmental malformation of the pleuroperitoneal mem-
brane or failure of attachment to the thoracic wall [11].
Diagnosis is usually made during routine prenatal imaging
or in the neonatal period due to pulmonary hypoplasia and
symptoms of respiratory distress.
BH can usually be detected by routine prenatal ultra-
sound as early as 18 weeks of gestation [12]. On ultrasound,
a left-sided CDH typically manifests as displacement of the
stomach, bowel, and/or left hepatic lobe into the left chest
with contralateral mediastinal shift (Fig. 9.5). Right-sided
CDH presents with displacement of the hepatic dome, gall-
bladder, omentum, and/or bowel into the right chest with
leftward mediastinal shift.
Color Doppler evaluation may demonstrate an abnormal
course of the umbilical and hepatic veins [10]. Fetal lung vol-
ume can also be measured using three-dimensional ultrasound
to determine prognosis. Measured lung volumes less than
15–25% of expected volume, along with herniation of the liver

Morgagni
hernia

Other anterior
hernias
Hiatal hernia

Bochdalek hernia Morgagni hernia and Hiatal

other anterior hernias (sliding and paraesophageal)
hernia
a b c

Fig. 9.4 Diagram demonstrating different types of diaphragmatic hernia. (a) Bochdalek hernia is typically large and occurs posteriorly. (b) Morgagni
hernia occurs anteriorly. (c) Hiatal hernia occurs in the region of the esophageal hiatus

Table 9.1 Types of diaphragmatic hernia and distinguishing factors

Bochdalek hernia Morgagni hernia Hiatal hernia
Location Posterior Anterior Esophageal hiatus
Side Left more common than right Right more common than left Midline
Developmental Pleuroperitoneal membrane Failed fusion of the septum Enlargement of the hiatus secondary to
etiology malformation or failure to attach to transversum and the anterior thoracic delayed descent of the stomach
the thoracic wall wall
9 Diaphragm
a b
S
Fig. 9.5 Bochdalek hernia in a 19-week gestational age male fetus. (a)
Transverse grayscale ultrasound image demonstrates a large left dia-
phragmatic hernia with intrathoracic location of the stomach (S), spleen
(asterisk), and multiple loops of bowel (arrow) with rightward displace-
into the chest, associated cardiac malformations, and maternal
polyhydramnios, can signify a worse prognosis [10, 11].
Postnatal ultrasound is usually performed in conjunction
with radiography and is particularly useful in identifying
herniation of solid organs (Fig. 9.6). Recent studies have
demonstrated the utility of postnatal ultrasound evaluation to
guide surgical management by quantifying the size of the
defect, determining the presence or absence of a diaphrag-
matic rim, and differentiating the defect from diaphragmatic
eventration [7, 13, 14].
Management of BH involves a multidisciplinary approach.
Primary medical treatment includes low-pressure, high-­
frequency mechanical ventilation to prevent lung injury and
pharmacologic treatment of pulmonary hypertension. Early ini-
tiation of extracorporeal membrane oxygen (ECMO) should be
considered in infants who fail to improve with conventional
management [15].
Surgical repair is usually delayed until the patient is physio-
logically stabilized [16]. A subcostal approach laparotomy is
typically used to place the herniated abdominal viscera back
into the abdominal cavity. Small defects can be repaired with
primary apposition, while larger defects are repaired by patch or
graft closure. Thoracoscopic and laparoscopic surgical
approaches have recently been gaining favor, with good clinical
outcomes [17].
Morgagni Hernia
Morgagni hernia (MH) occurs most commonly on the right
side and is thought to result from a failure of fusion of the
275
S
ment of the heart (arrowhead). (b) Sagittal oblique grayscale ultrasound
image shows the liver (L) which remains intra-abdominal in location,
with the stomach (S), bowel (arrow), and spleen (asterisk) displaced into
the thorax
septum transversum and the anterolateral thoracic wall [6].
Affected patients can be asymptomatic or present later in life
with mild respiratory distress, recurrent pneumonia, or gas-
trointestinal symptoms [8].
Prenatal and postnatal ultrasound findings of MH are similar
to those of BH (Fig. 9.7). The herniated portion of the liver may
demonstrate abnormal echogenicity due to vascular engorge-
ment [10]. On ultrasound, demonstrating d­iscontinuity and
folding of the free edge of the diaphragm with a narrow angle
waist has been shown to be a specific finding for right-sided
hernias [7].
Surgical repair is performed due to the risk of bowel
incarceration and strangulation [10]. Laparoscopic and open
repair have demonstrated similar favorable outcomes, while
the use of patch repair has more recently been shown to
reduce the rate of recurrence [18].
Hiatal Hernia
Hiatal hernia (HH) is usually acquired due to weakening or
atrophy of the diaphragmatic crura. However, congenital
HH is thought to occur as a result of delayed descent of the
stomach during development, which causes enlargement of
the esophageal hiatus [10]. HHs are further subdivided into
sliding hernias, where both the gastroesophageal junction
and portions of the stomach move into the chest; parae-
sophageal hernias, where only portions of the stomach are
displaced into the chest; and congenital short esophagus,
where the stomach is fixed inside the chest [10]. Affected
children typically present with gastroesophageal reflux,
276 W. G. Kim et al.

a b

c d

Fig. 9.6 Bochdalek hernia in a 1-month-old male with trisomy 18, sound image demonstrates herniation (arrow) of the spleen (S) into the
congestive heart failure and multiple congenital anomalies. (a) Frontal left chest. Sagittal grayscale ultrasound images demonstrate (c) a nor-
chest radiograph demonstrates cardiomegaly, pulmonary edema, and a mal thin echogenic diaphragm (arrowhead) on the right and (d) a dis-
rounded opacity (arrow) at the left lung base. (b) Sagittal grayscale ultra- continuous diaphragm (arrow) on the left

a b

Fig. 9.7 Morgagni hernia in a 4-month-old male with trisomy 21. lobe into the anterior chest. The liver directly abuts the heart (H). Note
Sagittal (a) and transverse (b) grayscale ultrasound images of the liver the intact portions (arrowheads) of the diaphragm posteriorly and
demonstrate focal herniation (arrows) of a portion of the right hepatic laterally
9 Diaphragm
recurrent respiratory tract infections, emesis, and failure to
thrive [19].
Prenatal diagnosis of HH can be made with ultrasound,
which demonstrates an anechoic or hypoechoic mass or tubular
structure in the posterior mediastinum that is continuous with
the stomach [20]. An intact diaphragm should also be con-
firmed. Peristalsis of the herniated stomach can be identified on
real-time ultrasound imaging [21]. Postnatal imaging diagnosis
usually involves radiography or a fluoroscopic contrast study of
the upper gastrointestinal tract.
Ultrasound can be useful to exclude other causes of gas-
troesophageal reflux in young children. Determination of
esophageal length, diameter, wall thickness, and the angle
at the gastroesophageal junction can also provide diagnos-
tic indicators of reflux or the presence of an HH (Fig. 9.8)
[22, 23].
Symptomatic HH is treated surgically, which involves
reducing the herniated contents, excising the hernia sac,
closing the crural defect, and performing an antireflux proce-
dure such as a Thal or Nissen fundoplication. Laparoscopic
technique has been shown to have favorable outcomes and
reduced recurrence rates [19, 24].
Fig. 9.8 Hiatal hernia in a 6-month-old female with vomiting. Sagittal
grayscale ultrasound image of the diaphragmatic hiatus demonstrates a
portion of the gas-filled stomach (asterisk) extending superiorly into the
thorax through a widened hiatus (between arrows). The stomach was
seen to slide dynamically through the hiatus on real-time imaging
277
Diaphragmatic Eventration
Congenital diaphragmatic eventration (CDE) refers to the
abnormal elevation or contour of an otherwise intact diaphragm,
which is thought to occur in the setting of congenitally thinned
diaphragmatic musculature [6]. Partial eventration occurs more
commonly anteromedially on the right side and usually encom-
passes a portion of the liver [25]. Complete eventration occurs
more commonly on the left side, with a slight male predomi-
nance [26]. CDE has been associated with other anomalies,
including ipsilateral lung hypoplasia, congenital heart disease,
and pectus excavatum [27].
Focal weakness of the diaphragm can also be acquired in the
setting of trauma, infection, ischemia, or neuromuscular dys-
function and can be distinguished pathologically from CDE by
the presence of a normal number of muscle fibers [25, 28].
Although the majority of patients with CDE are asymptomatic,
clinical manifestations can vary widely, with some pediatric
patients developing severe life-­threatening respiratory distress.
Affected children most commonly present with tachypnea,
vomiting, recurrent respiratory tract infections, and cough [27].
Ultrasound evaluation of focal CDE demonstrates an intact,
echogenic diaphragm covering a portion of the elevated
abdominal viscera (Fig. 9.9). The appearance of a fixed, broad-
angled waist during respiratory excursion at the site of even-
tration has been shown to be specific for the diagnosis of CDE
[7]. The imaging appearance of complete CDE is difficult to
distinguish from diaphragmatic paralysis, as both usually
demonstrate asymmetric diaphragmatic excursion.
Fig. 9.9 Diaphragmatic eventration in a 6-month-old female. Sagittal
grayscale ultrasound image shows an abnormal contour (arrow) of the
left medial hemidiaphragm containing a portion of the left hepatic lobe
(L). In contrast to a true hernia, the diaphragm is intact and demon-
strated normal excursion on real-time imaging
278
Symptomatic CDE is typically treated with diaphrag-
matic plication, with more recent literature demonstrating
the efficacy and improved outcomes of minimally invasive
thoracoscopic approaches [29, 30].
Acquired Diaphragmatic Disorders
Diaphragmatic Dysfunction
Diaphragmatic paralysis (DP) or weakness can be a rare cause
of respiratory distress in the pediatric population, with rela-
tively more clinically significant sequelae when compared to
adults. Infants and children have increased compliance of the
chest wall and weaker accessory respiratory muscles, which
decreases their compensatory efforts, even for unilateral dys-
function. This can lead to lung atelectasis, pneumonia, or dif-
ficulty weaning off mechanical ventilation [11].
Phrenic nerve injury due to birth trauma and cardiotho-
racic surgery is the most common cause of DP in infants
under 1 year of age [31]. Phrenic nerve paralysis can also
result from infection, inflammation, metabolic neuropathies,
central nervous system pathology, or extrinsic compression
from vascular abnormalities or masses [28]. Myopathies and
neuromuscular disorders may also cause diaphragmatic
weakness.
a b
Fig. 9.10 Diaphragmatic dysfunction in a 15-month-old male with congenital cardiac disease and difficulty weaning off ventilation after surgery. Sagittal
M-mode ultrasound images of the right (a) and left (b) hemidiaphragms demonstrate asymmetrically diminished excursion (arrowheads) on the right
W. G. Kim et al.
Dysfunction is usually unilateral, although bilateral dys-
function can also occur and results in more clinically evident
respiratory failure [5]. Symptoms of DP include respiratory
distress, paradoxical breathing, and increased work of breath-
ing. Affected infants may also present with feeding difficulty
and vomiting [31].
Imaging diagnosis of DP involves real-time evaluation
with both B-mode and M-mode ultrasound. A subxiphoid
approach typically demonstrates asymmetric excursion of
the hemidiaphragms on B-mode ultrasound, while midaxil-
lary views of each hemidiaphragm can allow quantification
of excursion on M-mode ultrasound (Fig. 9.10).
A diaphragmatic excursion less than 4 mm and a greater
than 50% difference in excursion between the hemidiaphragms
are indicative of dysfunction [3, 4]. The use of ultrasound for
serial monitoring of diaphragmatic function in mechanically
ventilated pediatric patients is being increasingly used to guide
management of critically ill children [32, 33].
Management of DP depends on the severity of the clinical
symptoms and whether or not phrenic nerve injury is thought
to be reversible. Ventilatory support is required in pediatric
patients presenting with hypoxia or failure to thrive. It can
involve endotracheal ventilation or less invasive continuous or
bi-­level positive airway pressure ventilation [31]. Symptomatic
DP due to irreversible phrenic nerve injury is typically treated
surgically with diaphragmatic plication, either via thoracot-
9 Diaphragm
omy or minimally invasive thoracoscopic or laparoscopic
techniques. Children with intact phrenic nerve function may
benefit from an implanted phrenic nerve stimulator device [5].
Diaphragmatic Inversion
Inversion of the diaphragm can occur in the setting of a large
pleural or pericardial effusion, tension pneumothorax, or
thoracic mass. It can occur in any age group but should be
recognized as a cause of respiratory distress in the pediatric
population. The right hemidiaphragm is less commonly affected
than the left due to the presence of the liver, although bilateral
inversion has also been reported [34].
Inversion is difficult to identify on radiographs alone, as
the diaphragm is usually obscured by the primary cause of
inversion. Ultrasound evaluation can diagnose both inversion
of the diaphragm and the underlying cause (Fig. 9.11).
Diaphragmatic excursion can be diminished, exaggerated, or
L anechoic on ultrasound, although a bronchogenic cyst may
K
Fig. 9.11 Diaphragmatic inversion in a 1-month-old female with
history of meconium aspiration receiving extracorporeal membrane
oxygenation (ECMO). Longitudinal grayscale ultrasound image
shows a large right hemothorax (asterisk) causing diaphragmatic
inversion (arrow). The right kidney (K) and liver (L) are seen below
the inverted diaphragm
a b
Fig. 9.12 Mesothelial cyst in a 6-year-old male with an incidentally
identified cystic lesion of the left hemidiaphragm. Transverse (a) and sag-
ittal (b) grayscale ultrasound images of the left hemidiaphragm demon-
279
paradoxical [25, 34, 35]. Inversion may affect the entire
hemidiaphragm or only a small portion [25].
Management of diaphragmatic inversion typically involves
treatment of the underlying cause. Pleural or pericardial effu-
sions are most often treated with thoracentesis.
Primary Diaphragmatic Masses
Benign Masses
There are few documented reports of benign diaphragmatic
masses that occur in the pediatric population. Reported
lesions include cyst, lymphatic malformation, hemangioma,
and lipoma (Table 9.2). Benign lesions can be asymptomatic
and are often found incidentally.
While benign cystic masses such as mesothelial cyst and
bronchogenic cyst are the most common primary diaphrag-
matic neoplasms in adults, they are rare in children [36, 37].
A mesothelial cyst is typically located along the right postero-
lateral hemidiaphragm near the costophrenic angle, while a
bronchogenic cyst is typically located at the diaphragmatic
crus (Fig. 9.12). Both types of cyst are thin walled and
sometimes appear echogenic due to proteinaceous or hemor-
rhagic contents [38, 39].
A lymphatic malformation may appear as a multicystic
mass that encases but does not compress the adjacent vascu-
lature and may be associated with a chylothorax [40, 41]. A
hemangioma frequently displays enlarged vascular channels
Table 9.2 Diaphragmatic masses
Benign lesions Malignant lesions
Mesothelial cyst Rhabdomyosarcoma
Bronchogenic cyst Undifferentiated sarcoma
Lymphatic malformation Yolk sac tumor
Hemangioma Extra-osseous Ewing sarcoma
Lipoma
strate a well-defined hypoechoic and avascular cystic structure (arrows)
adjacent to the spleen that moved with the diaphragm on real-­ time
imaging
280
on color Doppler ultrasound and can also be associated with
a pleural effusion [42, 43]. Lipoma has a slight predilection
for the left hemidiaphragm and can be sessile or hourglass in
morphology [44]. Ultrasound delineation of an intact dia-
phragm is helpful in differentiating a lipoma from a fat-con-
taining diaphragmatic hernia.
Management of benign lesions depends on symptomatol-
ogy and malignant potential. Most masses are typically surgi-
cally resected, both to establish a diagnosis and to alleviate
symptoms. Mesothelial cysts can sometimes spontaneously
regress, and some researchers suggest serial imaging surveil-
lance [38]. Mesothelial cysts and larger unresectable lym-
phatic malformations have also been successfully treated with
sclerotherapy [40, 45].
Malignant Neoplasms
Primary diaphragmatic neoplasms in the pediatric popula-
tion are exceedingly rare, and the majority are malignant
(Table 9.2) [36]. There is no particular gender predilection,
and lesions occur with equal frequency in the right and left
hemidiaphragms [36]. The most common primary tumor
of the diaphragm in children is rhabdomyosarcoma, an
aggressive neoplasm of mesenchymal origin. Other docu-
mented malignant neoplasms include undifferentiated sar-
coma, yolk sac tumor, and extra-osseous Ewing sarcoma
(Fig. 9.13). Sarcomas tend to occur in slightly older chil-
dren, with an average age of 12 years, while yolk sac
tumors have been reported exclusively in children under
the age of 2 years [46, 47].
Clinical presentation is dependent on age at diagnosis,
along with size and histology of the mass. Affected pediatric
Fig. 9.13 Yolk sac tumor in a 4-year-old male with respiratory distress.
Transverse grayscale ultrasound image shows a heterogeneous solid
mass (arrows) adjacent to the esophagus (asterisk) and inseparable from
the left diaphragm
W. G. Kim et al.
patients most commonly present with symptoms of chest
pain, shortness of breath, and cough [36]. Left-sided dia-
phragmatic lesions may cause gastrointestinal symptoms due
to gastric compression [48].
Diaphragmatic masses present a diagnostic challenge
due both to their rarity and difficulty in delineating their
site of origin. Because of their aggressive nature, sarcomas
and yolk sac tumors may be large at the time of diagnosis,
appearing as heterogeneous, vascular lesions on ultrasound.
Larger tumors are often misdiagnosed as originating from
adjacent organs such as the liver, lung, spleen, stomach,
and pericardium [36]. Their diaphragmatic origin is some-
times better demonstrated after tumor shrinkage with
chemotherapy.
Helpful imaging clues in determining diaphragmatic ori-
gin include the pattern of organ displacement, presence of an
obtuse angle between the mass and the diaphragm, and a
“claw” sign demonstrating portions of the diaphragm extend-
ing around the mass [49]. Differential considerations include
common juxta-diaphragmatic masses such as hepatic, perito-
neal, or pleural-based neoplasms.
It is important to note that the diaphragm may be involved in
direct extension of adjacent tumors, particularly those arising
in the retroperitoneum [50]. Metastatic involvement of the dia-
phragm has been documented in cases of thymoma and ovarian
cancer which are both exceedingly rare in children [37].
Treatment of malignant diaphragmatic tumors is deter-
mined by histopathology and usually involves some combi-
nation of chemotherapy, radiation, and surgical resection.
Larger tumors may require partial resection of the dia-
phragm, with muscular flap or graft reconstruction [36].
Traumatic Disorders
Traumatic diaphragmatic rupture (TDR) is uncommon in
children and is typically associated with high-impact, multi-
organ trauma. TDR occurs in approximately 3% of children
with blunt abdominal trauma [51]. Males are more commonly
affected than females, with an average age of 7 years [51].
TDR more commonly occurs on the left side, although trau-
matic defects tend to be larger on the right side. While adults
presenting with TDR usually present with abdominal symp-
toms, the majority of pediatric patients present with
­respiratory distress and decreased breath sounds. TDR is fre-
quently associated with injury to the liver, lung, pelvis, and/or
kidney, although isolated TDR has also been reported [51].
Ultrasound can demonstrate unilateral thickening with
focal discontinuity of the diaphragm. Fluid may be seen above
and below the affected diaphragm with an appearance of a
floating free edge of the ruptured diaphragm [52–54]. In cases
of associated visceral herniation, real-time imaging may show
intrathoracic location of the liver and/or peristalsing bowel
9 Diaphragm
loops [55]. Indirect signs of TDR include non-­visualization of
the left diaphragm or spleen due to herniation of the stomach
and associated loss of a usual ultrasound window [56].
Surgical management is determined by the location of
injury and most often requires laparotomy. Thoracotomy or
transdiaphragmatic surgical approaches are also reported.
Traumatic diaphragmatic defects are usually repaired with
primary apposition and suturing [57].
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 The Gastrointestinal Tract
10
Marthe M. Munden and Harriet J. Paltiel

Abbreviations Ig Immunoglobulin
ISSVA International Society for the Study of Vascular
AFP Alpha-fetoprotein Anomalies
ALARA As Low As Reasonably Achievable LES Lower esophageal sphincter
BRBNS Blue rubber bleb nevus syndrome MII Multiple intraluminal impedance
CD Crohn disease MR Magnetic resonance
CeCS Contrast-enhanced colosonography NASPGHAN North American Society for Pediatric Gastro­
CEUS Contrast-enhanced ultrasound enterology, Hepatology, and Nutrition
CF Cystic fibrosis NEC Necrotizing enterocolitis
CFTR Cystic fibrosis transmembrane regulator NHL Non-Hodgkin lymphoma
CMV Cytomegalovirus PEG Polyethylene glycol
CT Computed tomography PET Positron emission tomography
DIOS Distal intestinal obstruction syndrome SBI Small bowel intussusception
DSRCT Desmoplastic small round cell tumor SMA Superior mesenteric artery
ECMO Extracorporeal membrane oxygenation SMV Superior mesenteric vein
ESPGHAN European Society for Pediatric Gastroen­ UC Ulcerative colitis
terology, Hepatology, and Nutrition UGI Upper gastrointestinal
FDG Fluorodeoxyglucose
GER Gastroesophageal reflux
GI Gastrointestinal Introduction
GIST Gastrointestinal stromal tumor
GVHD Graft-versus-host disease Ultrasound is an important imaging modality for evaluation of
HCG Human chorionic gonadotropin the gastrointestinal tract in children. Ultrasound is often the
HLA Human leukocyte antigen first-line imaging modality in children presenting to the
HPS Hypertrophic pyloric stenosis Emergency Department with vomiting, abdominal pain, and
HSP Henoch–Schönlein purpura lethargy not explained by initial plain abdominal radiographs.
HUS Hemolytic–uremic syndrome There is no need for preparation in the emergent setting for
IBD Inflammatory bowel disease ultrasound imaging and the lack of imaging with radiation is
in accordance with the As Low As Reasonably Achievable
(ALARA) principle. Ultrasound techniques for imaging of the
pediatric GI tract are reviewed, followed by normal develop-
M. M. Munden (*)
Department of Radiology, Shawn Jenkins Children’s Hospital, ment and anatomy. The definition, incidence, and typical clini-
Medical University of South Carolina, Charleston, SC, USA cal presentation of entities involving the GI tract from the
e-mail: mundenm@musc.edu esophagus to the anorectal region are discussed, including infec-
H. J. Paltiel tious, inflammatory, congenital, neoplastic, as well as anatomic
Division of Ultrasound, Department of Radiology, Boston Children’s variants that can predispose to volvulus and obstruction. A
Hospital and Harvard Medical School, Boston, MA, USA brief overview of management is also provided.

© Springer Nature Switzerland AG 2021 283

H. J. Paltiel, E. Y. Lee (eds.), Pediatric Ultrasound, https://doi.org/10.1007/978-3-030-56802-3_10
284 M. M. Munden and H. J. Paltiel

Esophagus vical esophagus can be obtained centering just to the left of

Technique
Patient Positioning
Cooperation varies with age, but in most pediatric patients, the
cervical esophagus is easily evaluated with ultrasound by plac-
ing the patient supine, using a small pillow or towel roll to
extend the neck for optimal visualization. The upper thoracic
esophagus can be difficult to assess due to acoustic reflectors
from the lungs. However, the heart can be used as an acoustic
window to visualize the lower thoracic esophagus [1]. The gas-
troesophageal junction can easily be studied in the supine infant.
midline with the esophagus located posterior to the left
lobe of the thyroid gland. Sagittal imaging of the cervical
esophagus is relatively easily performed using a left lateral
approach to study the majority of the cervical esophagus. A
right lateral approach can be used to image the lower cervi-
cal esophagus [1].
Filling the stomach with clear liquid and then placing the
transducer in a sagittal plane in the midline along the aortic
hiatus allows for assessment of the lower thoracic esophagus
and gastroesophageal junction. This approach can be used to
assess for gastroesophageal reflux, hiatal hernia, as well as
intrathoracic positioning of the stomach.

Ultrasound Transducer Selection

The highest resolution linear array transducers, typically in
the range of 15–18 MHz, are used for imaging with settings
optimized for the bowel wall. A small footprint transducer is
ideal in the study of the cervical esophagus in infants and
younger children.
Imaging Approaches
No special preparation is needed although fasting for at
least 4 hours is recommended in the non-emergent setting
for optimal imaging to reduce artifact from food content in
the stomach and small bowel. Transverse images of the cer-
Normal Development and Anatomy
Normal Development
The gastrointestinal tract arises from the endoderm of the tri-
laminar embryo in the third week of life and extends from the
buccopharyngeal membrane to the cloacal membrane. The GI
tract and its associated organs will subsequently receive con-
tributions from all three germ cell layers. In the fourth week of
life three distinct regions, the foregut, midgut, and hindgut,
extend the length of the embryo and will develop into the dif-
ferent components of the GI tract (Fig. 10.1).

Pharynx

Aorta

Stomodeum
Esophageal
Heart region

Septum Gastric and

transversum duodenal regions

Celiac artery
Yolk stalk and
vitelline artery Primordium
of liver
Allantois
Superior
mesenteric
Proctodeum artery to midgut

Cloacal Inferior mesenteric

membrane artery

Cloaca Hindgut

Fig. 10.1 Diagram of a 4-week-old embryo showing early development of the gastrointestinal tract and its arterial blood supply
10 The Gastrointestinal Tract 285

Normal Anatomy proximal foregut separates into a ventral respiratory and dorsal
The esophagus is a fibromuscular tube which consists of cervi- gastrointestinal tube. The ventral tracheobronchial diverticulum
cal, thoracic, and abdominal components (Fig. 10.2). The esoph- eventually separates from the dorsal foregut to become the
agus arises from the embryonic endoderm with the early respiratory tract. A disturbance in this process of separation
digestive tract, dividing into a foregut, midgut, and hindgut. The underlies the development of tracheoesophageal malforma-
tions. Bronchogenic cysts and esophageal duplication cysts also
arise from aberrant budding of the tracheobronchial tree.
During the fourth month of gestation, the embryonic cili-
Pharynx ated epithelium is replaced by stratified squamous epithelium.
Cricoid cartilage The circular muscle and ganglion cells form during week 6,
Left flexure of while blood vessels enter the submucosa during week 7.
Trachea
esophagus The striated muscle of the upper esophagus and upper
esophageal sphincter is derived from branchial arches 4, 5, and
6 and is innervated by the vagus nerve. The smooth muscle of
the lower esophagus and lower sphincter are derived from
mesenchyme of somites surrounding the foregut. The esopha-
Arch of aorta
gus also receives parasympathetic and sympathetic innerva-
Left bronchus
tion which regulates glandular secretion, striated and smooth
Descending muscle activity, and blood supply [2].
Right flexure aorta
The cephalad portion of the cervical esophagus begins
of esophagus
inferior to the cricoid cartilage at the lower border of the
Intra-abdominal
esophagus pharynx at the sixth cervical vertebra and ends at the upper
mediastinum, located between the trachea anteriorly and the
vertebra posteriorly (Fig. 10.3). The thoracic esophagus crosses
Fundus of the diaphragm at the esophageal hiatus around the tenth tho-
stomach racic vertebral level and the short portion of the abdominal
esophagus joins the gastric cardia (Fig. 10.4). The thoracic
Cardia esophagus courses to the right of the cephalad aorta and poste-
Esophageal rior to the left atrium more caudally.
hiatus
There is an upper and lower esophageal functional sphinc-
Greater
Pylorus
curvature
ter. The cricopharyngeus forms the primary muscle of the
upper sphincter, triggered by the swallowing reflex. The lower
Descending Lesser
curvature
esophageal sphincter (LES), like the lower esophagus, is com-
duodenum
posed of smooth muscle which thickens at the LES, anchored
by the right diaphragmatic crus. The lower sphincter provides
Fig. 10.2 Diagram of the normal esophagus and stomach an antireflux mechanism at the diaphragmatic hiatus [3].

a b

Fig. 10.3 Normal ultrasound appearance of cervical esophagus in a a cervical extension (arrowhead) of the thymus gland. (b) Transverse
5-year-old child. (a) Sagittal grayscale ultrasound image shows normal grayscale ultrasound image shows the normal esophagus (arrow) poste-
alternating layers (arrows) of the esophageal wall. Incidentally noted is rior to the left thyroid lobe (T)
286
Fig. 10.4 Normal gastroesophageal junction in a 3-month-old infant.
Sagittal grayscale ultrasound image of the gastroesophageal junction
with normal length of abdominal esophagus. Swallowed echogenic air
bubbles (arrow) are identified in the lower thoracic esophagus
The esophagus narrows at the cricoid cartilage; at the
level of the aortic arch where it is compressed by the left
main bronchus; and at the esophageal hiatus; all common
sites at which food or foreign bodies may become lodged
within the esophagus. The abdominal esophagus is a short
portion extending from the esophageal hiatus of the diaphragm
to the cardia of the stomach [4].
The esophageal wall is comprised of an inner echogenic
mucosal layer, surrounded sequentially by a thin hypoechoic
muscular layer, an echogenic submucosal layer, and a
hypoechoic layer of circular and longitudinal muscle. The
outer, adventitial layer consists of echogenic fat.
Gastroesophageal Reflux
Gastroesophageal reflux (GER) is defined as the retrograde
passage of gastric contents into the esophagus. Gatrointestinal
reflux disease (GERD) is defined as reflux with associated
complications such as aversion to feeding, esophageal stric-
tures, or failure to thrive. GER is considered a normal physi-
ologic entity that occurs several times a day in normal
infants, associated with transient relaxation of the lower
esophageal sphincter. Up to 50% of normal infants spit up
daily during the first 6 months of life [5].
GERD is associated with symptoms that include poor
weight gain, refusal to eat, vomiting with irritability, choking,
wheezing, and failure to thrive. The incidence of GERD is
lower in breast-fed infants, peaks around 4 months of age, and
M. M. Munden and H. J. Paltiel
declines by 12 months of age. When present in older children,
symptoms include heartburn, chest pain, and dysphagia [5, 6].
Reflux of gastric contents can be easily seen during an ultra-
sound examination. Infants with GER have a shorter intra-
abdominal esophagus compared to those without reflux [1].
Contrast-enhanced color Doppler ultrasound has been used
to diagnose GER in children [6]. However, the retrograde
motion of gastric content is readily detected on grayscale ultra-
sound (Fig. 10.5) simply by filling of the stomach with milk or
water [7–9]. A recent update by the North American Society
for Pediatric Gastroenterology, Hepatology, and Nutrition
(NASPGHAN) and the European Society for Pediatric Gas­
troenterology, Hepatology, and Nutrition (ESPGHAN) [10]
reported a 95% sensitivity of color Doppler ultrasound in the
detection of GER but only an 11% specificity when comparing
ultrasound assessment with 24-hour pH testing.
Although there is no evidence to support the use of ultra-
sound as a primary means of diagnosing GERD, it may be use-
ful to assess for other causes of GER, such as hiatal hernia and
pyloric stenosis [11]. Imagers should be aware of the “mass-
like” appearance of the gastroesophageal junction after surgical
fundoplication wrap for treatment of GERD (Fig. 10.6), closely
resembling the appearance associated with intussusception.
The upper gastrointestinal tract (UGI) barium study is not
the study of choice to assess for pathologic reflux but is useful
in the evaluation of possible anatomic obstruction, and for the
diagnosis of a web, stenosis, or malrotation that could predis-
pose to GER.
Continuous intraluminal esophageal pH monitoring is being
used less often as a primary modality for diagnosis, while mul-
tiple intraluminal impedance (MII) is emerging as a better
method for detection of GERD. MII and pH electrodes are
placed using a single catheter [11]. Upper endoscopy can be
helpful in those who do not respond to routine management by
directly visualizing the esophageal mucosa and permitting
evaluation of the severity of injury and inflammation.
GERD symptoms often respond to thickening of formula,
cessation of overfeeding, and avoidance of supine position-
ing and tobacco smoke [12]. Various pharmacologic agents
are available, including acid suppressants, histamine 2 recep-
tor inhibitors, protein pump inhibitors, and prokinetic agents.
Fundoplication surgery is performed when GER cannot be
controlled medically [13].
Hiatal Hernia
A hiatal hernia is defined as herniation of a portion of the
stomach through the esophageal hiatus into the thorax, and is
associated with a variety of diaphragmatic abnormalities,
including enlargement of the esophageal hiatus, congenital
defects, and post-traumatic damage. Most hiatal hernias in
children are congenital in origin.
10 The Gastrointestinal Tract 287

a b

Fig. 10.5 Gastroesophageal reflux in a 6-month-old infant. (a) Sagittal (b) Upper gastrointestinal (UGI) series image reveals reflux of barium
grayscale ultrasound image of the gastroesophageal junction shows (arrow) into the thoracic esophagus
reflux (arrow) of liquid gastric content into the lower esophagus.

Fig. 10.6 Fundoplication wrap in a 2-year-old male. (a) Sagittal gray- diaphragm (arrowhead). (b) Transverse grayscale ultrasound image
scale ultrasound image of the gastroesophageal junction shows a mass- depicts the rounded fundoplication wrap (arrow) that mimics the layers
like appearance (arrow) of the fundoplication wrap just below the of an intussusception
288
The four types of hiatal hernia include: type I–sliding hia-
tal hernia, type II–paraesophageal hernia (often seen with
prior fundoplication), type III–components of sliding hiatal
hernia and paraesophageal hernia, and type IV–herniation of
all or part of the stomach into the thorax (usually with organo-
axial malrotation of the stomach) [14–16]. The clinical mani-
festations of a hiatal hernia can be vague, including frequent
burping, spitting, epigastric pain, hosrseness, or wheezing.
In the presence of a hiatal hernia, the intra-abdominal esoph-
agus is shortened, the angle between the abdominal esophagus
and the posterior gastric wall is increased, and sliding of the
gastric fundus toward the diaphragm may be observed [1]. It is
not uncommon to see a gas-filled hiatal hernia on chest radio-
graphs, often in patients with a history of prior fundoplication
that may have loosened, or in those with neurological disease
and increased intra-abdominal pressure predisposing to hiatal
hernia. Gas-filled hiatal hernias can be identified on ultrasound,
barium studies, plain chest radiographs, and CT examinations
(Fig. 10.7). Please refer to Chap. 9: Diaphragm for additional
information about hiatal hernia.
a b
S
Fig. 10.7 Hiatal hernia in 3 different patients. (a) Sagittal grayscale
ultrasound image of the lower left chest demonstrates the gastric fundus
(calipers). S, Spleen. (b) UGI series image shows a barium-filled gastric
M. M. Munden and H. J. Paltiel
Pharmacologic agents (including those used for treatment
of GERD) and diet adjustment can be used to treat symptoms
related to a sliding hiatal hernia. Surgery is performed when
there is no relief after medical management. For hiatal hernias
of type II, III, and IV, surgery should be performed to prevent
complications.
Stomach
Technique
Patient Positioning
The stomach is easily accessible for ultrasound evaluation
with the patient in a supine position. The gastroesophageal
junction is best seen in a sagittal plane anterior to the aorta.
The gastric fundus and greater curvature are well-assessed
with the patient in a left lateral decubitus position. The gastric
antrum and pylorus are best studied in a right lateral decubitus
position.
fundus (arrow) in the lower chest. (c) Chest radiograph reveals a gas-
filled hiatal hernia (arrow)
10 The Gastrointestinal Tract
Ultrasound Transducer Selection
The highest resolution linear transducer that penetrates ade-
quately (usually 12–18 MHz) provides the best detail, with set-
tings adjusted for bowel imaging. Gentle compression using the
liver as an acoustic window permits evaluation of the gastric
body and antrum. Cine loops can be used to document gastric
motility. Color Doppler is used to detect hyperemia.
Imaging Approaches
A 4-hour fast is recommended to reduce gastric food con-
tent with an overnight fast of greater than 8 hours improv-
ing visibility even further in older children. Filling the
stomach with clear fluid and using the liver as an acoustic
window permits evaluation of the gastric wall, peristalsis,
gastric outlet, and abnormal wall thickening due to masses,
a b
Fig. 10.8 Normal gastric wall in a 6-week-old infant presenting with
frequent non-bilious emesis. (a) Transverse grayscale ultrasound image
of the posterior gastric wall (arrow) shows normal definition of the wall
layers that appear thickened due to luminal under-distention. (b)
289
inflammation, or retained, undigested material forming a
gastric bezoar. Oral contrast agents such as iso-osmolar
polyethylene glycol (PEG) can improve bowel distension
but water often suffices.
Wall measurements should be obtained with the stomach
fully distended with fluid when possible, and the scan should
be performed in the mid-longitudinal plane proximal to the
pyloric canal. Echogenicity of the 5 gastric wall layers repre-
sents a combination of the interface echoes and the echo-
genic properties of the histologic layers of the stomach wall.
The normal gastric mucosal thickness is 2–3 mm, the normal
thickness of the wall of the gastric antrum is about 2 mm, and
the normal pyloric muscle thickness is 2 mm or less.
The variability of measurements between distended and
nondistended wall measurements (Fig. 10.8) has been
Transverse grayscale ultrasound image of the anterior wall of the stom-
ach (arrows) after the lumen is partially distended with water. (c)
Transverse grayscale ultrasound image shows a normal thin gastric wall
(arrows) after complete luminal distention with water
290
reported as between 4 mm and 5 mm when measuring from
the inner echogenic mucosa to the outer border of the gastric
wall [17–19]. On cross-sectional imaging, if the image obtained
is too close to the contracted pyloric canal, thickening of the
pyloric muscle can be erroneously suggested. Similarly, if tan-
gential images are obtained in a longitudinal plane, the mus-
cle may appear falsely thickened.
Normal Development and Anatomy
Normal Development
At the end of the fourth week of life, the stomach is a
straight, hollow tube (Fig. 10.9). From the fourth to sixth
weeks, it initially dilates in a fusiform manner followed by
preferential growth of its dorsal wall. This results in a
bulge that will eventually develop into the greater curva-
ture. In the sixth to eighth weeks of life, the stomach
rotates simultaneously along the longitudinal axis and the
anteroposterior axis.
Normal Anatomy
The four main regions of the stomach are the cardia, the fun-
dus, the body, and the pylorus (Fig. 10.2). The cardia is the
zone surrounding the lower esophagus and receives esopha-
geal contents. The dome of the fundus is located to the left of
the esophageal junction and cephalad to the cardia. The gas-
tric body, the largest portion of the stomach, extends from
the fundus to the antrum with the concave border of the lesser
curvature and the convex greater curvature along the inferior
body of the stomach. The gastric antrum ends at the pylorus
where gastric contents exit to the duodenal bulb.
In addition to the circular and longitudinal muscle layers
present in the bowel wall, the stomach has an inner oblique
smooth muscle layer which allows for the mechanical break-
down of food products. The superficial mucosal lining con-
tains mucous glands. Deep to the epithelial layer is the lamina
propria containing lymphoid cells and small blood vessels. A
thin muscular layer underlies the lamina propria, with over-
lying submucosa, muscularis propria, and serosa forming the
outer layer [20]. The greater omentum hangs down from the
greater curvature of the stomach.
Congenital Anomalies
The fetal stomach can be visualized as early as 9 weeks of
gestation with an identifiable fundus, lesser and greater curva-
tures, and a pylorus identified around 14 weeks [21]. Various
congenital anomalies may affect gastric size. In esophageal
atresia with no distal tracheoesophageal fistula, the stomach
M. M. Munden and H. J. Paltiel
may be absent or small on prenatal imaging. In cases of duo-
denal atresia, the stomach may be large. Congenital obstruc-
tions of the stomach are felt to represent the sequelae of
vascular accidents in fetal life.
Gastric Atresia
Gastric atresia is a congenital defect with complete occlu-
sion of the pylorus. It represents less than 1% of all con-
genital intestinal obstructions and may have an autosomal
recessive inheritance. There is also an association with tri-
somy 21 and epidermolysis bullosa [22]. The incidence of
gastric or pyloric atresia is 1 in 100,000 births, seen prena-
tally as polyhydramnios with a distended single bubble and
obstruction of the gastric outlet [23]. Infants present with
non-bilious emesis with the first feeding.
Ultrasound will show a distended stomach with obstruc-
tion at the outlet. A distended stomach is seen on radiographs
with absence of gas distal to the obstruction. The lack of gas
in the duodenum helps to distinguish gastric atresia from the
more common “double bubble” sign of duodenal atresia
described later in this chapter.
Gastric atresia is treated surgically soon after birth with
resection of the obstructed segment and creation of a
gastroduodenostomy.
Microgastria
Microgastria is a very rare anomaly in which the stomach is
a small tubular structure without a recognizable fundus, body,
or antrum. The greater and lesser curvatures do not develop.
Microgastria can be associated with intestinal malrotation,
megaesophagus, asplenia, polysplenia, and congenital heart
disease [24, 25] as well as upper limb anomalies, and is sel-
dom an isolated abnormality [26]. The etiology is unknown
with a suspected defect in mesodermal development during
the fourth or fifth week of gestation.
Prenatally, there is polyhydramnios with non-visualiza-
tion of the stomach. A dilated esophagus with deficient peri-
stalsis is frequently present on prenatal imaging. The
gastroesophageal junction is incompetent and patients with
microgastria present with vomiting and failure to thrive dur-
ing infancy, some with aspiration pneumonia due to associ-
ated gastroesophageal reflux [27].
A small tubular stomach at ultrasound should raise suspi-
cion for microgastria. Plain films at birth can show gas
throughout the gastrointestinal tract. On barium studies, a
small, tubular midline stomach is seen with microgastria as
well as esophageal dilation [25].
Treatment options for microgastria include medical man-
agement with frequent small feedings or jejunal tube feed-
ings [26]. Surgical options include gastric augmentation with
10 The Gastrointestinal Tract 291

Liver
Dorsal aorta

Stomach Spleen
Small Spleen
Gallbladder Dorsal intestine
pancreatic bud
Midgut loop

Umblical Cranial limb

cord

Omphaloenteric
duct Caudal limb Inferior
Superior
mesenteric artery mesenteric artery

a a1 b b1

Dorsal Dorsal aorta

mesogastrium
Liver
Omental
Liver Spleen bursa
Ventral Stomach Cecum Spleen
mesentery
Transverse
Gallbladder Duodenum
colon
Umblical cord Hindgut

Cecal swelling

c c1 d d1

Lesser omentum

Desending colon

Small intestine
Ascending
colon Sigmoid colon

Rectum

Cecum and
e appendix

Fig. 10.9 Diagrams of normal embryological development of the gas-
trointestinal (GI) tract. (a) By week four of development, the division of
the GI tract into the foregut, midgut, and hindgut has occurred. The
growth of the GI tract exceeds the volume of the abdominal cavity so
that the developing intestine herniates into the umbilicus. The GI tract
then undergoes a 90-degree counterclockwise rotation around the supe-
rior mesenteric artery. (b) The superior limb of the intestinal loop will
form the ileum and the inferior limb will form the colon. The bowel will
continue to grow and rotate for the next 5 weeks. (c) The proximal por-
tion of the midgut loop has migrated from a superior position to the right
side of the body and the distal portion of the loop has migrated from an
inferior position to the left. (d) In week ten, the bowel retracts back into
the abdominal cavity where it undergoes a 180-degree counterclockwise
rotation. The cecum is then located in the right upper quadrant of the
abdomen. (e) Enlargement of the large intestine pushes the cecum down
to its final position in the right lower quadrant of the abdomen
292
a jejunal loop and gastric excision with a Roux-en-Y esoph-
agojejunostomy [28, 29].
 astric Diaphragm (Antral Web)
G
A gastric diaphragm (or antral web) is a thin membrane
about 2–4 mm in thickness extending across the gastric
antrum at a distance from 1 to 7 cm proximal to the pylorus
which can lead to gastric outlet obstruction. The web is a
mucosal structure and can vary from a crescent of mucosa to
a circumferential web with a central opening. The etiology of
this unusual finding in children is uncertain, with an inci-
dence of 1 in 100,000 births [30]. Neonates may present with
difficulty feeding and non-bilious emesis. Some patients
present later in life with vague symptoms of early satiety,
epigastric pain, postprandial vomiting, and transient epi-
sodes of vomiting.
Ultrasound of a fluid-stomach may reveal an echogenic
fold or diaphragm-like structure crossing the distal stomach
from the lesser to the greater curvature with associated gas-
tric dilation and delayed gastric emptying. The pyloric chan-
nel is normal [30, 31]. On barium studies, an antral web is
seen as a persistent band-like linear defect at the gastric
antrum causing a “double bulb” sign with the antral chamber
between the pylorus and the web appearing as one bulb and
the proximal duodenum appearing as the second bulb.
Antral webs can be treated with surgical myotomy and
endoscopic dilation. Intraoperative endoscopic gastroduode-
noscopy combined with operative antral web resection has
been beneficial in localizing the webs at surgery [32].
Acquired Obstruction
 ypertrophic Pyloric Stenosis
H studies for the diagnosis of HPS, with an accuracy approach-
Hypertrophic pyloric stenosis (HPS) is defined as an abnor-
mal thickening of the muscular layer and abnormal elonga-
tion of the pyloric canal with failure of relaxation leading to
gastric outlet obstruction and projectile, forceful non-bil-
ious emesis (Fig. 10.10). Symptoms typically occur from 2
to 12 weeks of age, but very rarely after 12 weeks. Though
unusual, HPS also can present before 2 weeks of age. Those
infants with a very early presentation are more likely to
have a positive family history of HPS.
HPS occurs in about 2–3.5 per 1000 live births and is
more common in males than in females with a 1.5-fold
increased risk in first-born children and a fivefold male pre-
dominance. It is less common in infants of older mothers.
Prematurity of less than 37 weeks may be a risk factor [33].
Premature infants develop HPS at a later chronological age
(but earlier post-menstrual age) than term infants, with some
studies showing a higher female preponderance [34, 35].
Maternal smoking increases the risk of HPS by 1.5- to two-
fold. Some studies have found an increased incidence with
bottle feeding (not expressed breast milk) [36, 37].
M. M. Munden and H. J. Paltiel
Esophagus
Duodenum
Pyloric stenosis Stomach
Fig. 10.10 Diagram of pyloric stenosis
The etiology of HPS remains unclear although there is a
genetic predisposition, with a study from Denmark showing
an almost 200-fold higher rate among monozygotic twins,
and a 20-fold increased rate among dizygotic twins and sib-
lings [38]. Studies have postulated abnormal innervation of
the muscle layer, with findings of a reduced number of nerve
terminals, abnormal nitric oxide synthase activity, and defi-
cient interstitial cells of Cajal [39].
An overly distended stomach may be noted on plain radio-
graphs in patients with HPS. Ultrasound has replaced barium
ing 100% [40]. The gastric outlet is easily studied by placing
the infant in a right lateral decubitus position, using the liver as
an acoustic window. Although the stomach in HPS is often
already distended, a small amount of clear fluid can help delin-
eate pyloric anatomy. However, over-distention of the stomach
displaces the pyloric channel posteriorly and may require
repositioning of the patient into either a left lateral decubitus
position, or switching to a lower frequency transducer for
deeper penetration and optimal imaging.
Pyloric measurements should be obtained in the mid-
longitudinal plane of the stomach or in cross section at or
just proximal to the pyloric channel, measuring the muscle
thickness of a single wall, excluding the mucosa and sub-
mucosa. If obtained in a tangential plane, the muscle may
appear falsely thickened. In infants with HPS, the pyloric
ring is no longer clearly defined and the thickened pyloric
channel measures from 1.4 to 2 cm in length (Fig. 10.11).
The muscle thickness of the elongated pyloric channel
changes rather abruptly from the 1-mm thickness of the
normal antrum to 3 mm or greater in the hypertrophied
10 The Gastrointestinal Tract
canal [40]. During the ultrasound examination, the pyloric
channel should at no time appear normal with gastric peri-
stalsis. In premature infants with HPS, ultrasound measure-
ments are not affected by weight or corrected gestational
age [35].
Treatment for HPS is surgical. Laparoscopic pyloromy-
otomy is widely used now as an alternative to open pyloro-
myotomy with good success. Laparoscopic procedures allow
a shorter time to full feeding with slightly shorter hospital
a b
Fig. 10.11 Hypertrophic pyloric stenosis in a 3-week-old male with pro-
jectile vomiting and a normal gastric antrum shown for comparison. (a)
Transverse grayscale ultrasound image demonstrates an elongated pyloric
channel and marked muscle wall thickening (arrow). (b) Transverse gray-
293
stays [41]. Persistent vomiting in the post-surgical infant can
be evaluated by ultrasound, but should be interpreted with
caution, as the muscle can remain thickened up to 8 months
after surgery [42, 43].
Pylorospasm
Pylorospasm has a similar presentation to HPS but repre-
sents a spasmodic contraction of the pylorus, not a fixed
obstruction. Pylorospasm is characterized by a transient spasm
scale ultrasound image shows a normal pyloric channel (arrow) for com-
parison with no thickening of the antropyloric muscle, allowing passage
of gastric contents. (c) Abdominal radiograph reveals a distended stom-
ach (arrows) due to underlying pyloric stenosis
294
of the pyloric muscle without gastric outlet obstruction.
Clinical presentation is similar to hypertrophic pyloric steno-
sis with persistent non-bilious vomiting in the young infant.
A transient pyloric canal elongation and muscle thicken-
ing may be present during ultrasound evaluation of the pyloric
channel and may mimic HPS or raise suspicion for pyloric
stenosis in evolution. However, the thickened pyloric muscle
relaxes with observation to allow gastric emptying [39, 42].
The muscle wall thickness of pylorospasm can overlap with
that of HPS, although it is not a constant finding (Fig. 10.12,
Table 10.1). On UGI studies, intermittent flow of contrast into
the duodenum differentiates pylorospasm from HPS.
In those infants with a pyloric muscle thickness of 2–3 mm
and an elongated pyloric channel that does not relax through-
out the ultrasound examination, continued clinical monitor-
ing and short-term interval ultrasound follow-up may be
warranted to assess for pyloric stenosis in evolution.
 rostaglandin-Induced Foveolar Hyperplasia
P With organoaxial gastric volvulus, the stomach rotates
Foveolar cells cover the gastric epithelium and focal polyp-
oid hyperplasia can occur in the gastric antral mucosa, and
to a lesser extent in the intestine. In the neonate or infant
with cyanotic congenital heart disease treated with pro-
longed prostaglandin therapy to maintain patency of the
ductus arteriosus, gastric outlet obstruction can develop as
a result of foveolar hyperplasia. This can lead to elongation
and polypoid thickening of the antropyloric channel [44,
45]. Foveolar hyperplasia can clinically mimic HPS in
patients who present in the age range typical of hypertro-
phic pyloric stenosis.
Fig. 10.12 Pylorospasm in a 5-week-old infant presenting with force-
ful emesis. (a) Transverse grayscale ultrasound image shows an elon-
gated pyloric channel with thickening of the pyloric muscle (arrow),
M. M. Munden and H. J. Paltiel
Ultrasound of the stomach in patients with prostaglandin-
induced foveolar hyperplasia will show a polypoid, echogenic
thickening of the antral mucosa without extension into the lam-
ina propria (Fig. 10.13). This contrasts with HPS that involves
the muscular layer, not the mucosa. Color Doppler imaging of
the mucosa in patients with foveolar hyperplasia may show
increased vascularity in the deep gastric mucosa [46].
Foveolar hyperplasia resulting from prostaglandin therapy
is treated medically, with spontaneous resolution after cessa-
tion of treatment. There have been a few reported cases of the
coexistence of both foveolar hyperplasia and HPS, a possibil-
ity that should be considered in young infants if symptoms do
not resolve after discontinuation of prostaglandins [47].
Gastric Volvulus
Gastric volvulus is a rare abnormal rotation of the stomach
leading to gastric outlet obstruction, and is classified into
organoaxial and mesenteroaxial types.
around its long axis and becomes obstructed with an inver-
sion of the greater and lesser curvatures if rotated greater
than 180 degrees. Organoaxial volvulus accounts for about
two-thirds of gastric volvulus in children. The most com-
monly associated condition is congenital diaphragmatic her-
nia, although acquired diaphragmatic hernia, eventration, and
paraesophageal hernia can be complicated by gastric volvulus
(Fig. 10.14) [48, 49].
With mesenteroaxial gastric volvulus, the stomach rotates
around its short axis with a displacement of the antrum and
pylorus above the gastroesophageal junction, presenting
which mimics pyloric stenosis. (b) Transverse grayscale ultrasound
image after several minutes of observation shows a patent, normal-
appearing channel (arrow). The stomach is not distended
10 The Gastrointestinal Tract 295

Table 10.1 Ultrasound features of hypertrophic pyloric stenosis acutely with severe obstruction. Although organoaxial vol-
versus pylorospasm vulus is more common overall than mesenteroaxial volvulus,
Pyloric stenosis Pylorospasm most cases of mesenteroaxial volvulus occur in children.
Muscle wall thickness >3 mm May be >3 mm Early and accurate diagnosis of acute gastric volvulus is
Change with time No Yes
essential to prevent ischemia and perforation. Acute gastric
Associated gastric distention Yes No
volvulus usually occurs in a child less than 5 years of age

a b

Fig. 10.13 Prostaglandin-induced foveolar hyperplasia in a 1-month- show polypoid thickening (arrows) of the gastric wall. (c) Chest radio-
old infant with vomiting. Patient receiving extracorporeal membrane graph reveals diffuse gastric wall thickening (arrow) as well as support
oxygenation (ECMO) for complex congenital heart disease. Transverse apparatus for ECMO
(a) and sagittal (b) grayscale ultrasound images of the gastric body
296
a b
Fig. 10.14 Diaphragmatic hernia with organoaxial gastric volvulus in
a neonate. (a) Chest radiograph shows the intrathoracic stomach
(arrows) due to a diaphragmatic hernia. (b) Oblique power Doppler
ultrasound image obtained through the lower chest shows the gastric
who presents with non-bilious emesis, epigastric distention,
and pain. Chronic volvulus in infants may manifest with
emesis, feeding difficulties, and failure to thrive.
A diagnosis of gastric volvulus is usually made on the
basis of plain radiographs and UGI series. Radiographic
findings include spherical gastric distension, herniation of
the stomach above the diaphragm, diaphragmatic eleva-
tion, and a paucity of distal bowel gas. With organoaxial
volvulus, the greater curvature of the stomach may be
seen above the lesser curvature. Ultrasound does not gen-
erally play a role in diagnosis, although in experienced
hands it can be diagnostic in children with mesenteroaxial
M. M. Munden and H. J. Paltiel
antrum (arrow) lying posterior to the gastric fundus (asterisk). (c)
Image from an UGI series reveals organo-axial volvulus of the intratho-
racic stomach (arrow). F, Fundus of stomach
gastric volvulus, revealing a displacement of the antrum
above the gastroesophageal junction. An UGI series can
evaluate the rotation of the stomach and identify gastric
outlet obstruction. CT will show the rotated stomach and
a transition point [50].
Treatment of gastric volvulus is surgical, with a reduction
in the volvulus, gastropexy, and repair of any potential dia-
phragmatic hernia or defect. Acute gastric volvulus can
induce shock, and immediate gastric suction with removal of
gastric contents is done to temporarily improve any associ-
ated compartment syndrome [51].
10 The Gastrointestinal Tract
Gastric Wall Thickening
Gastritis
Gastritis, gastropathy, and peptic ulcer disease are often
considered together in the spectrum of acid peptic disease.
If untreated, gastritis can progress to peptic ulcer disease.
Helicobacter pylori is the most common gastric microbial
pathogen and is a major risk factor for gastritis, gastric and
duodenal ulcers. The gastric antrum is the most common
site of inflammation and the submucosal layer is often col-
onized. About half of the world’s population is estimated
to be infected with H. pylori, usually acquired within the
first 5 years of life. The prevalence of infection is higher in
developing countries and is the most important cause of
primary duodenal ulcers in children [52].
Less common infectious etiologies of gastritis include
cytomegalovirus, fungi such as histoplasmosis, and parasites.
High-dose steroids, non-steroidal anti-inflammatory drugs,
stress/trauma, inflammatory bowel disease, systemic masto-
cytosis, and chronic renal disease can also lead to gastritis
[53]. Symptoms are nonspecific and include epigastric pain,
nausea, vomiting, anorexia, and occasionally hematemesis.
Ultrasound of gastritis shows nonspecific gastric antral
wall and mucosal thickening (Fig. 10.15). Significantly
greater thickening of the muscularis mucosa and muscular
layers of the antrum occur in the setting of infection than in
patients with other forms of gastritis [54]. UGI barium stud-
ies show nonspecific gastric fold thickening primarily in the
antrum in patients with H. pylori gastritis [54, 55].
Fig. 10.15 Gastritis in a 4-month-old male. Transverse grayscale
ultrasound image of the distal stomach reveals thickening (arrow) of all
layers of the antropyloric region of the stomach
297
The gold standard for diagnosis of H. pylori gastritis is
endoscopy. Noninvasive urea breath testing and stool antigen
testing are used in follow-up. Treatment for gastritis is medi-
cal. Triple therapy using proton pump inhibitors in combina-
tion with antibiotics has been effective in patients with H.
pylori gastritis.
Ménétrier Disease
Pediatric Ménétrier disease is a rare protein-losing gas-
tropathy in children with marked hypertrophy of the folds
of the gastric body and fundus, and sparing of the antrum
[56]. It may be clinically confused with eosinophilic gastri-
tis as peripheral eosinophilia occurs in more than half of the
patients with Ménétrier disease. The etiology is unknown with
speculation regarding its link to chemical irritants, toxins, and
possibly autoimmunity. Cytomegalovirus (CMV) infection of
the stomach is found in about one-third of patients [57, 58].
Ménétrier disease can present with nausea, vomiting, and
abdominal pain, with symptoms often developing after a
respiratory infection. Peripheral edema is reported in the
majority of children due to the severe protein loss which can
lead to initial suspicion of a renal disorder.
The diagnosis can be suspected upon detection of thick-
ened mucosal folds during imaging with ultrasound, barium
studies, or CT. Although a specific diagnosis may not be
made by ultrasound, it can still be used to follow therapy.
Clinical and pathologic correlations are needed to make the
diagnosis, as endoscopic biopsy alone is insufficient. A full
thickness gastric biopsy is often required.
Ménétrier disease in children is generally benign and self-
limited, lasting about 5 weeks. Treatment of CMV, when
detected, usually leads to remission. In the absence of CMV
infection, treatment is usually supportive and includes hydra-
tion, histamine 2 receptor antagonists, and proton pump inhib-
itors with albumin replacement [59, 60].
Eosinophilic Gastroenteritis
Eosinophilic gastroenteritis is an uncommon benign inflam-
matory infiltration by eosinophils that is either primary or
secondary (due to allergies or parasites) in etiology. Any seg-
ment of the GI tract can be involved, but the stomach and
small intestine are the most commonly affected. Prevalence
in the United States ranges from 8 to 28 per 100,000, and is
more common in children than in adults. Excess weight,
higher socioeconomic status, and Caucasian race may be
risk factors with a possible hereditary component. About one
half of patients have concomitant allergic disorders, includ-
ing asthma and eczema. Peripheral eosinophilia is present in
about 70% of patients [61].
Eosinophilic gastroenteritis usually presents with chronic,
nonspecific symptoms including abdominal pain, dysphagia,
diarrhea, nausea, and weight loss. Protein-losing enteropa-
thy, ascites, and GI bleeding can also occur.
298 M. M. Munden and H. J. Paltiel

Radiologic findings are nonspecific, variable, and can even

be absent. Ultrasound may show gastric fold thickening and
pseudopolyposis of the gastric body and antrum along with
small bowel wall thickening and ascites. Barium studies show
a nonspecific mucosal fold thickening and nodularity that is
most prominent in the gastric antrum. The antrum can be nar-
rowed and nodular in chronic disease and may result in
obstruction. CT shows diffuse thickening of mucosal folds,
submucosal edema, ascites, and occasionally obstruction [62].
Diagnosis requires the presence of GI symptoms, histo-
logic evidence for eosinophilic infiltration, and exclusion of
other causes of tissue eosinophilia. Biopsy plays an impor-
tant role in diagnosis, although multiple biopsies may be
required due to the patchy distribution of the eosinophilic
infiltrates [63]. Spontaneous remission occurs in about a
third of cases and food allergy appears to play an important
role. Therapeutic options include dietary modification as the
first option, followed by steroids, leukotriene inhibitors, and
mast cell stabilizers. Relapses are frequent [63, 64].

 hronic Granulomatous Disease of the Stomach

C
Chronic granulomatous disease is a rare inherited primary
immunodeficiency commonly transmitted as an X-linked or
autosomal recessive disorder affecting about 4–5 per million
live births [65]. Chronic granulomatous disease affects males
more than females and about two-thirds have the X-linked
recessive form. There is a defect in the genes encoding for the
nicotinamide dinucleotide phosphate (NADPH) oxidase
complex which leads to a defective oxidative burst of the neu-
trophils and macrophages resulting in an inability to destroy
catalase-positive bacteria and fungi [66].
Patients are susceptible to bacterial and fungal infections
which lead to inflammation and granuloma formation in the
affected tissues (lung, cervical nodes, GI tract, liver, and spleen).
Patients receive life-long antibiotic and antifungal treatment,
which has led to improved overall survival. Children are usually
diagnosed within the first 5 years of life due to severe, recurrent
bacterial and fungal infections. When the stomach or intestinal
wall is involved, the inflammation can cause gastric pain, diar-
rhea, vomiting, and gastric outlet obstruction.
On ultrasound examination, there will be circumferential
wall thickening of the gastric antrum with narrowing, a dis-
tinctive finding seen in approximately 16% of affected chil-
dren (Fig. 10.16) [67]. On UGI series, the antrum will show
concentric narrowing with an elongated pyloric channel and
delay in gastric emptying. Patients with X-linked disease are
more prone to gastric outlet obstruction. Computed tomogra-
phy (CT) may show enlarged, low attenuation mesenteric
nodes that can later calcify. Magnetic resonance (MR) imag-
ing can distinguish acute from chronic thickening with acute
disease being hyperintense on T2-weighted images and show-
ing post-contrast enhancement, while chronic disease is not
T2 hyperintense nor does it enhance.
Fig. 10.16 Chronic granulomatous disease in a 10-year-old male with
bilious vomiting. Transverse grayscale ultrasound image shows marked
thickening (arrow) of the gastric wall in the antropyloric region
Patient survival has improved with long-term antibiotic
and antifungal therapy. The only definitive treatment at pres-
ent is hematopoietic stem cell transplantation with an overall
survival rate of greater than 90% for children undergoing
transplantation when less than 14 years of age. Recent pub-
lications describe gene therapy for X-linked recessive chronic
granulomatous disease as an alternative for those without a
human leukocyte antigen (HLA) matched donor [68, 69].
Benign Masses of the Stomach
 astric Duplication Cyst
G
Duplication cysts and mesenteric cysts are the most common
cysts of the GI tract with an incidence of 1 in 4500 births.
Enteric duplication cysts can occur anywhere from the base of
the tongue to the anus but are most commonly seen in the dis-
tal ileum along the mesenteric border (33%). Only 7% involve
the stomach [70]. They are often detected prenatally or within
the first few years of life. They are believed to arise between
the fourth and eighth weeks of gestation with several proposed
theories as to etiology, including failed luminal recanalization
in the esophagus, small bowel, and colon. A reported increased
incidence of associated spinal defects, cardiac, or urinary mal-
formations occurs in 16–26% [70].
Heterotopic tissue is present in about 35% of gastric dupli-
cation cysts and up to 10% contain ectopic pancreatic tissue in
the submucosal layer. The rare intrapancreatic duplication cyst
10 The Gastrointestinal Tract
can be confused with a pancreatic pseudocyst. Intrapancreatic
duplication cysts fill with secretions and can erode into the
pancreatic duct leading to pancreatitis. Duplication cysts
involving the pancreas are most commonly found in infants
and children [71, 72]. Duplication cysts may be an incidental
finding on routine ultrasound examination but can lead to
gastric outlet obstruction, pancreatitis, bleeding from ulcer-
ation, or vague epigastric fullness.
The characteristic well-defined inner echogenic mucosal
layer and outer hypoechoic muscular layer of the duplication
cyst – the “gut signature” – can help establish the diagnosis by
Fig. 10.17 Duplication cyst in the region of the gastric outlet in a
2-week-old infant with persistent vomiting. Transverse (a) and sagittal
(b) grayscale ultrasound images of the gastric outlet reveal an anechoic,
299
ultrasound (Figs. 10.17 and 10.18). Eighty percent of duplica-
tion cysts are spherical and have no communication with the
bowel lumen, while 20% are tubular and do communicate with
the bowel lumen [73]. Foci of ectopic tissue within a duplica-
tion cyst can lead to inflammation, ulceration, and hemorrhage,
resulting in a more complex appearance by ultrasound. Some
cysts may be pedunculated. Most gastric duplication cysts are
located along the greater gastric curvature [74].
Imaging pitfalls exist, as there are other cysts within the
abdomen that can have a double layer “pseudo gut signa-
ture” appearance that may be misinterpreted as a duplication
avascular cyst (calipers; arrow). Gut signature is not well-demonstrated.
(c) Axial T2-weighted, fat-suppressed MR image demonstrates fluid
signal (arrow) within the lesion
300
Fig. 10.18 Gastric duplication cyst in a 17-month-old female. Transverse
grayscale ultrasound image obtained in the right mid-abdomen demon-
strates a well-circumscribed cyst containing internal echogenic debris.
The cyst wall has a characteristic “gut signature,” with an echogenic inner
mucosal layer and a hypoechoic outer muscular layer
cyst [75]. Barium studies show an intramural filling defect
impressing the gastric contour. CT with contrast demonstrates
a thick-walled, low-attenuation lesion with enhancement of
the lining, occasionally with calcification in the wall. MR
imaging will show increased signal within the cyst on fluid-
weighted sequences.
Rare reports of malignant transformation exist, so surgery is
the recommended treatment in all cases [76]. Surgical removal
requires complete cyst excision along with the shared wall with
the stomach. Marsupialization of cysts that communicate with
the stomach risks exposure of the cyst mucosa to gastric con-
tents. Surgical excision is also the mainstay of therapy for the
rare pancreatic duplication cyst.
Gastric Teratoma
Extragonadal teratomas are very rare in children, with the
majority occurring in the sacrococcygeal region or within the
mediastinum. Gastric teratoma has been reported but is quite
unusual, representing 1% of all teratomas, occurring before
1 year of age and with a male predominance. The majority of
these tumors arise from the greater curvature and posterior
wall of the stomach. The lesions can present as a palpable
mass or with emesis, although those with intramural exten-
sion may present with gastrointestinal bleeding and gastric
perforation [77].
As with teratomas elsewhere, calcification, fat, and cystic
areas are identified at imaging. While these tumors may be
found at initial ultrasound examination and calcifications
may be seen on plain radiographs, the large size of these
tumors usually requires cross-sectional imaging with CT or
MR for diagnosis.
M. M. Munden and H. J. Paltiel
Patients with a mature teratoma or a low-grade immature
teratoma and serum alpha-fetoprotein (AFP) and beta-human
chorionic gonadotropin (HCG) hormone values in a normal
range are treated by surgical excision with close clinical fol-
low-up. These tumors carry a good prognosis and plasma
AFP levels can be used for monitoring purposes [78–80].
Gastric Lipoma
Gastric lipoma represents less than 1% of all gastric tumors
and is usually detected incidentally at autopsy. It is benign
and composed of mature adipose tissue and usually occurs in
the submucosa of the gastric antrum. In adults, gastric lipo-
mas can be larger with ulcerations. A few cases have been
reported in children presenting with hematemesis due to muco-
sal ulceration, intermittent vomiting, and melena [81].
Most of the imaging findings have been reported in adults
and diagnosed by CT after initially noted on an UGI series as
an ulcerated lesion with smooth margins. A low attenuation
mass can be seen on barium studies due to the presence of
fatty tissue [82].
Surgical removal is the treatment of choice in large,
symptomatic gastric lipomas. Smaller lesions can be treated
by endoscopic removal [83].
 ocal Foveolar Hyperplasia of the Stomach
F
Focal foveolar hyperplasia is rare in children. It is caused by
an accumulation of inflammatory cells in the gastric mucosa
resulting in large, hypertrophic folds that form broad-based
polyps. Foveolar hyperplasia occurs in response to injury,
involving both the gastric antrum and body in the presence of
H. pylori gastritis, but is confined to the antrum in non-H.
pylori gastritis [84]. Primary bile reflux has also been
reported to lead to foveolar hyperplasia and vascular conges-
tion in children [85]. The few reported cases located in the
gastric antrum presented with non-bilious vomiting, weight
loss, gastric outlet obstruction, and gastrointestinal bleeding
[86].
Ultrasound shows superficial lobulated thickening of the
gastric mucosa leading to partial gastric outlet obstruction.
Color Doppler reveals intense hyperemia of the gastric antral
wall deep to the mucosa [46]. On an UGI series, there may
be elongation and narrowing of the pylorus which can mimic
pyloric stenosis in the appropriate age range.
Diagnosis is difficult and may require endoscopy with
biopsy. Treatment is symptomatic and supportive. If H.
pylori is documented, appropriate therapy aimed at eradi-
cating the infection is undertaken.
I nflammatory Gastric Myofibroblastic Tumor
Inflammatory myofibroblastic tumor is another very rare
benign mesenchymal neoplasm of unknown etiology that
can involve the stomach, although more often these tumors
involve the lung. They occur in fewer than 1 in one million
individuals and are composed of myofibroblastic spindle
10 The Gastrointestinal Tract
cells with an inflammatory infiltrate of plasma cells, lympho-
cytes, and eosinophils.
There is a wide range of age at presentation with females
affected more than males, and a mean age of 9 years at diag-
nosis [87, 88]. A solitary mass is the most frequent, although
visceral and cutaneous involvement can occur with the mul-
ticentric form of tumor. Clinically, patients may present with
weight loss, fever, anemia, thrombocytosis, or with a slow
growing mass leading to obstruction.
Ultrasound reveals a hypovascular, solid mass with central
hypoechoic necrosis and calcification. Tumor size can range
from several cm to more than 10 cm. They may be lobulated in
contour and will demonstrate prominent enhancement on
CT. On MR imaging they are typically isointense to muscle on
T1-weighted sequences with variable T2 intensity depending
on the fibrous content [89].
Biopsy is required for diagnosis and tumors should be
surgically removed. While solitary and multicentric tumors
often have a benign course, those with positive margins after
surgical resection are associated with recurrence and
increased mortality [89, 90]. Adjuvant chemotherapy is used
if there is a local invasion or in rare cases of metastasis.
Gastric Bezoar
A bezoar consists of ingested material that mainly accumu-
lates in the stomach although it can develop in any portion
of the gastrointestinal tract and result in obstruction.
Lactobezoars occur mainly in infants who are fed improp-
erly constituted or high caloric formula. Many are preterm
infants fed a high casein–whey ratio, highly concentrated for-
mula. Lactobezoars can occur in full-term infants as well, per-
haps related to poor gastric emptying and decreased gastric
secretions. Trichobezoars are more common and occur in
older children from chewing and swallowing hair that accu-
mulates in the stomach, sometimes extending into small bowel
[91]. Phytobezoars develop as a result of poorly digested fruit
and vegetable fibers. Bezoars in adults often occur following
gastric surgery and will not be further discussed.
A bezoar can be suspected by plain radiography on the
basis of a large amount of mass-like undigested gastric
material in patients presenting with early satiety, abdomi-
nal pain, poor weight gain, and bowel obstruction. It may
appear as mottled solid matter filling the stomach or small
bowel, although a large, recently ingested meal can have a
similar appearance.
Ultrasound can be used for diagnosis, although a cor-
rect diagnosis of a gastric bezoar was made by ultrasound
in only 25% of patients in one series [92]. Filling the stom-
ach with clear fluid and positioning the patient upright and
in both decubitus positions to separate the bezoar from the
301
gastric wall is useful to exclude a gastric mass (Fig. 10.19).
Trichobezoars form an echogenic arc with marked acoustic
shadowing caused by air trapped within the conglomerate of
hair fibers. This appearance helps to differentiate a gastric
bezoar from non-pathologically ingested food content.
Barium should be administered with caution as it can
interfere with therapeutic endoscopy. CT will show a low-
density mass mixed with air bubbles, better seen with win-
dow manipulation for optimal visualization [92].
Lactobezoars are usually medically treated with bowel
rest and a change to predigested formula. Some success has
been described with the use of intra-gastric N-acetylcysteine
for lactobezoars as well as with the ingestion of Coca-Cola®
for phytobezoars [93, 94]. Large-channel endoscopes have
been used to remove gastric phytobezoars with suction.
Trichobezoars are often surgically removed although some
have been treated with endoscopic removal [95].
 ther Benign Masses
O
Other benign gastric masses are rare in children. Heterotopic
pancreas, plasma cell granuloma, and leiomyoma can
occur, among others. These lesions may manifest as focal
thickening of the gastric wall or as a polypoid mass [96,
97]. The ultrasound features of these lesions are nonspe-
cific and diagnosis rests with clinical correlation and/or
biopsy.
Malignant Gastric Tumors
Lymphoma
Non-Hodgkin lymphoma (NHL) is not common in the
pediatric population, affecting about 700–800 children per
year in the United States. Burkitt and Burkitt-like lympho-
mas are the most frequent subtypes of non-Hodgkin lym-
phoma in childhood, accounting for 35–40% of NHL [98].
The sporadic form seen in North America is associated with
Epstein–Barr virus in 15% of cases. Most pediatric cases of
NHL are high grade with aggressive behavior. Burkitt lym-
phoma involves the GI tract in a reported 23% of cases,
affecting the distal small bowel, cecum, and appendix most
commonly, and often presenting with obstructive symp-
toms or pain.
Burkitt lymphoma is the most rapidly growing tumor in
children [99]. The stomach is rarely involved. The peak age
for Burkitt lymphoma is 5–15 years although those present-
ing younger than age 5 years have a slightly better outcome
[100]. Rare reported cases of gastric involvement have pre-
sented with vague, episodic abdominal pain, vomiting,
weight loss, and constipation.
302
a b
c d
Fig. 10.19 Gastric trichobezoars in two adolescent females pre-
senting with early satiety and weight loss. (a) Transverse grayscale
ultrasound image of the stomach in the first patient shows the arc-
like contour of a trichobezoar (asterisk) in the upright position,
outlined by water administered during the study. (b) UGI series
image of the second patient reveals a trichobezoar (arrow) outlined
Ultrasound findings of gastric lymphoma include thicken-
ing of the hypoechoic layers of the gastric wall with or without
loss of layer stratification (Fig. 10.20). Masses can protrude
into the lumen and result in luminal narrowing [100, 101].
Plain radiographs may show gastric wall thickening and con-
trast studies will show a gastric mass. Contrast-enhanced CT
and fluorodeoxyglucose (FDG) positron emission tomogra-
M. M. Munden and H. J. Paltiel
by barium. (c) Coronal contrast-enhanced computed tomography
(CT) image shows a conglomerate of hair (arrows) filling the
stomach and containing entrapped air. (d) Gastric trichobezoar fol-
lowing surgical removal. (Image courtesy of Dr. Harry Kozakewich,
Boston Children’s Hospital and Harvard Medical School, Boston,
MA, USA)
phy (PET) provide anatomic and metabolic imaging, now the
standard of care. PET/MR imaging may provide an alternative
to PET/CT yielding high-quality images with a reduction in
radiation dose [98].
Treatment is surgical for localized disease, although most
children with NHL are treated with chemotherapy. Treatment
is based on lymphoma type and stage.
10 The Gastrointestinal Tract
a b
Fig. 10.20 An 11-year-old female with Burkitt lymphoma involving
the stomach. (a) Transverse grayscale ultrasound image reveals a large,
hypoechoic mass (asterisk) encasing the gas-filled stomach (S). (b)
GI Stromal Tumor
GI stromal tumor (GIST) is the most common neoplasm of the
stomach in children and the most common mesenchymal neo-
plasm of the GI tract. It originates from the interstitial cells of
Cajal, the cells that mediate communication between the auto-
nomic nervous system and the smooth muscle. GI stromal
tumors are distinguished from other mesenchymal neoplasms
pathologically by their expression of KIT (CD117), a tyrosine
kinase growth factor receptor.
GIST has a reported incidence of 0.11 cases per million
in the pediatric population and 0.4% of all GIST patients
are less than 20 years of age [102]. GIST in children is
distinct with characteristics that vary from the adult form,
including a median age at diagnosis in the second decade
compared to 63 years in adults, a female predilection
(70% of cases) compared to an equal gender distribution in
adults, and lack of mutation in KIT or platelet-derived
growth factor receptor A (PDGFRA) in 85% of cases
[102]. Tumors lacking these mutations are referred to as
“wild type.”
A pediatric GIST is most likely to present as a GI bleed due
to its predilection for the stomach, but can also manifest with
pain, vomiting, and early satiety. GIST can occur in isolation or
in association with Carney’s triad (GIST, pulmonary chon-
droma, and paraganglioma), Carney–Stratakis syndrome (GIST
and paraganglioma), and neurofibromatosis type I [103].
No distinguishing ultrasound or other imaging feature can
identify a GIST from other mesenchymal neoplasms such as
leimyoma or leiomyosarcoma. GIST is generally exophytic
and rarely calcifies (Fig. 10.21). It contains cystic regions and
303
Coronal contrast-enhanced CT image shows the low-attenuation mass
(asterisk) surrounding the stomach. There is a large ulceration (arrow)
along the lesser gastric curvature
hemorrhage and may invade adjacent organs and metastasize
to the liver and omentum. Plain radiographs may show a mass
indenting the gastric wall, rarely with calcification. Barium
studies will show a smooth, well-circumscribed mass along
the gastric wall. CT, MR imaging, and PET may be used in
staging and follow-up of an intramural mass with extragastric
extension. The majority of these lesions enhance on CT and
MR imaging. MR imaging features depend on the degree of
necrosis and hemorrhage [103].
Treatment is surgical excision. In patients with localized
disease, complete surgical removal with negative margins is
achieved in 93%. However, the event-free survival rate at
10 years is only 16% [104].
 ther Malignant Masses
O
Other malignant gastric tumors are extremely rare in chil-
dren. Leiomyosarcoma represents 2–4% of soft tissue sarco-
mas in the pediatric population although they rarely involve
the stomach. It is crucial that leiomyosarcoma be differenti-
ated from GIST for treatment purposes. Presentation with
severe anemia with a large gastric filling defect on UGI
series has been reported [105].
Primary gastric adenocarcinoma is exceedingly rare in chil-
dren, constituting 0.05% of pediatric GI malignancies. While
H. pylori is commonly implicated as an etiology in adults, the
etiology of this rare tumor is unknown in children. Symptoms
include vomiting and abdominal pain with metastatic disease
often found at presentation due to the nonspecific clinical pre-
sentation and rarity of the disease [105].
304 M. M. Munden and H. J. Paltiel

a b

Fig. 10.21 GI stromal tumor (GIST) in a 15-year-old female with
unexplained anemia. (a) Transverse grayscale ultrasound image
shows multiple echogenic nodules (arrowheads) arising from the wall
of the stomach, with a large exophytic component (asterisk) along the
lesser curvature. (b) Sagittal grayscale ultrasound image of the distal
stomach (S) reveals multiple lobulated, exophytic masses (arrows)
extending to the liver (L). (c) Coronal contrast-enhanced CT image
depicts the heterogeneous, low-attenuation masses (arrows) arising
from the stomach (S)
10 The Gastrointestinal Tract
Small Bowel
Technique
Patient Positioning
For ultrasound of the small bowel, the patient is mainly
scanned in a supine position, with additional decubitus posi-
tioning used depending on the clinical question. To assess
the duodenum for hematoma or normal retroperitoneal posi-
tion, a right lateral decubitus position is helpful in identify-
ing the proximal duodenum which can then be followed with
the patient supine as it extends transversely between the
aorta and the superior mesenteric artery. Placing the patient
in a left lateral decubitus position often helps displace super-
ficially located bowel loops and improves access to the
deeper regions of the abdomen. With necrotizing enterocoli-
tis in infants, supine and decubitus positioning assists with
distinguishing air within the bowel lumen from air in the
bowel wall, i.e., pneumatosis.
Ultrasound Transducer Selection
High-resolution transducers (15–18 MHz) with settings opti-
mized for bowel detail are used for both grayscale and color
Doppler imaging. In the older child, a convex lower fre-
quency transducer (5–9 MHz) may be necessary for adequate
penetration.
Imaging Approaches
Ultrasound imaging in the emergent setting requires no prep-
aration, while patients undergoing evaluation for inflamma-
tory bowel disease (IBD) or other elective small bowel
studies should not eat solids for 4 hours prior to examination.
Ingestion of clear liquids (12–16 ounces) is encouraged for
elective studies to distend the small bowel and fill the blad-
der, which elevates the small bowel loops out of the pelvis.
Filling the stomach with water provides an acoustic window
for evaluation of the duodenum and proximal jejunum.
Carbonated drinks are discouraged in order to avoid gas arti-
fact. Ultrasound contrast is useful for evaluating flares and
response to therapy in patients with IBD [106].
The normal duodenum can typically be examined in the
fasting patient from the gastric antrum with the patient in a
right lateral decubitus position, then along its retroperitoneal
position between the superior mesenteric artery (SMA) and
aorta with the patient supine. If water or milk is given, the
gastric content can be followed through the duodenum. The
orientation of the SMA and superior mesenteric vein (SMV)
should be noted. When an inflammatory process is encoun-
tered in the right lower quadrant, the small bowel proximally
within the left lower quadrant and jejunal loops in the left
305
upper quadrant are studied in sagittal and transverse planes
to assess for bowel wall thickening, dilated loops, mesenteric
adenopathy, and interloop abscess. The remaining small bowel
can be studied with gentle, graded compression in a supine
position.
To evaluate the distal ileum for infectious enteritis or
Crohn disease, gentle anterior compression with simulta-
neous posterior manual compression is useful in separat-
ing bowel loops. Cine clips are obtained to document
small bowel motility.
Normal Development and Anatomy
Normal Development
In contrast to the stomach which develops a dorsal bulge in
early embryonic life, the duodenum develops a ventral bulge
(Fig. 10.9). The proximal duodenum, from the pylorus to just
past the papilla, is derived from the foregut, and maintains its
blood supply from the celiac axis, the major artery to the
foregut. The rest of the duodenum is derived from the mid-
gut, and is supplied by the superior mesenteric artery, the
major artery to the midgut, through the dorsal mesentery.
When the stomach rotates during embryonic life, the duo-
denum also alters in position, with its formerly concave dor-
sal border subsequently opening to the left, the so-called
“C-loop.” There is subsequent resorption of the dorsal mes-
entery that leaves a covering of visceral peritoneum along its
anterior surface. The loss of mesenteric attachment results in
the eventual retroperitoneal location of the duodenum
beyond the level of the bulb [107].
Normal Anatomy
On ultrasound evaluation, normal small bowel wall thickness
is less than 2.5 mm with the inner hyperechoic mucosa and
outer hypoechoic muscular layers visible. Small bowel loops
are decompressed if no fluid has been administered. Normal
bowel is not hyperemic with only minimal flow detected.
With normal bowel rotation, the four segments of the
duodenum can be identified from the pylorus to the duode-
nojejunal junction, with the third portion coursing posterior
to the pancreas, between the aorta and the superior mesen-
teric artery. The third portion of the duodenum can be docu-
mented in the aorto-mesenteric space in the sagittal plane
(Fig. 10.22). Normal folds of jejunum transition to the
smoother appearance of ileum in the mid-abdomen and
right lower quadrant. Jejunal loops show more peristalsis
than normal ileum [106]. The terminal ileum joins the
cecum in the right lower quadrant, entering just cephalad to
the appendix.
306
a b
Fig. 10.22 Normal ultrasound appearance of the duodenum and proxi-
mal small bowel in an infant. (a) Transverse grayscale ultrasound image
demonstrates the third portion of the duodenum (arrow) coursing between
Congenital Anomalies
 uodenal Atresia, Stenosis, and Web
D ing with delayed passage of ingested liquids across the repair site.
Duodenal atresia is a congenital obstruction that usually
occurs distal to the ampulla of Vater due to a failure of
recanalization of the duodenum during the eighth to tenth
weeks of gestation. Of all potential causes for congenital
duodenal obstruction, the majority are due to duodenal atre-
sia, although annular pancreas, duodenal stenosis, and duo-
denal web may also cause partial obstruction. The diagnosis
of duodenal atresia is often made on prenatal imaging and is
one of the most common causes of fetal bowel obstruction.
Duodenal atresia occurs in 1 in 2500–5000 births with a
3% prevalence in patients with trisomy 21. About 30% of
patients with duodenal atresia have trisomy 21, and there is
also an association with VACTERL (vertebral, anorectal,
cardiac, tracheoesophageal, renal, and limb anomalies)
[108]. Infants with duodenal atresia present with bilious
emesis soon after birth or after the first feeding.
On prenatal ultrasound, a “double bubble” sign and polyhy-
dramnios can be seen, representing the fluid-filled, distended
stomach and proximal duodenum. Initial radiographs show the
classic “double bubble” sign of the air-filled, distended stomach
and proximal duodenum. Often there is no air distal to the dou-
ble bubble. Air injected through an enteric tube provides excel-
lent contrast for evaluation by abdominal radiographs which are
often all that is needed to make the diagnosis.
Ultrasound can demonstrate the fluid-filled stomach and
level of obstruction of the dilated duodenum (Fig. 10.23).
Ultrasound can also assess for possible associated malrota-
tion and extrinsic compression from an annular pancreas that
can partly or completely surround the second portion of the
duodenum [109]. When duodenal atresia is located between
two bile duct orifices, a rare anomaly, gas may be seen distal
to the dilated proximal duodenum [108].
Duodenal atresia is treated surgically by duodeno-duodenos-
tomy or duodeno-jejunostomy with operative evaluation for
M. M. Munden and H. J. Paltiel
the superior mesenteric artery (arrowhead) and the aorta (A). (b) Sagittal
grayscale ultrasound image of the left flank shows normal, collapsed
proximal small bowel loops (arrow)
additional areas of intestinal obstruction. Postoperative compli-
cations include a leak at the anastomosis and persistent narrow-
Duodenal stenosis is relatively rare and may not be diag-
nosed until later in childhood depending on the degree of
narrowing. Duodenal web is a result of incomplete recanali-
zation between the eighth and tenth weeks of gestation result-
ing in either a complete obstruction or a perforated ­diaphragm.
The second portion of the duodenum is the most common
location, distal to the ampulla.
Additional abnormalities described in patients with duode-
nal atresia and stenosis include malrotation and a preduodenal
portal vein [110]. Associated anomalies in patients with a duo-
denal web include malrotation and trisomy 21 [111]. Infants
with a complete web will present with duodenal obstruction and
bilious emesis soon after birth, while those with a fenestrated
web may present later in life with recurrent episodes of bilious
emesis, abdominal distention, and failure to thrive.
Ultrasound diagnosis of duodenal stenosis and duodenal
web can be made if the stomach is filled with fluid. Luminal
narrowing (stenosis) or an obstructing intraluminal membrane
(web) with a dilated proximal duodenum proximal to the nar-
rowed area will be demonstrated (Fig. 10.24). Imaging of a
stenosis can mimic the “double bubble” sign on plain abdomi-
nal radiographs although distal gas is often present, depending
on the degree of stenosis.
Upper GI studies of duodenal stenosis will show an abrupt
duodenal narrowing distal to a dilated bulb with small
amounts of contrast identified beyond the stenosis. A web
appears as a faint radiolucent line outlined by barium. Over
time, the web elongates as a result of continual peristalsis,
resulting in a wind sock configuration of an intraluminal
duodenal diverticulum [112]. The role of CT in the evalua-
tion of the duodenum in children is limited.
Both duodenal stenosis and web are treated surgically. There
are also published reports of successful endoscopic balloon
dilation in children with these abnormalities [113, 114].
10 The Gastrointestinal Tract
a b
Fig. 10.23 Duodenal atresia in a newborn infant. (a) Transverse gray-
scale ultrasound image of the epigastric region shows dilation (arrow)
of the duodenum proximal to the atresia. (b) Abdominal radiograph
a b
Fig. 10.24 Duodenal web in a 3-year-old male with failure to thrive.
(a) Transverse grayscale ultrasound image shows a web (arrows) nar-
rowing the lumen of the proximal duodenum. (b) UGI series image
Intestinal Atresia
The prevalence of jejunoileal atresia is 1–3 per 10,000 live
births and is weakly associated with cystic fibrosis, malrota-
tion, and gastroschisis. It is thought to occur as a consequence
of intra-uterine mesenteric vascular accidents, possibly due to
volvulus or bowel incarceration in utero. The different types
of intestinal atresia include septal “web” atresia, blind-ending
atresia, mesenteric gap atresia, and apple peel atresia, as well
as multiple atresias. The most common type is blind-ending
atresia. The incidence of multiple bowel atresias is 15%.
After the duodenum, the ileum is the most common site of
involvement. Bowel atresia associated with gastroschisis
(5–15% of cases) is a poor prognostic indicator [115]. The
307
demonstrates a typical “double bubble” appearance with a dilated duo-
denum (arrow) proximal to the site of atresia
with patient in a right lateral position reveals circumferential narrowing
(arrow) caused by the web with dilation (asterisk) of the proximal
duodenum
rare “apple peel” form of atresia occurs when the distal small
bowel wraps around its vascular supply, resembling an apple
peel, and affects the duodenum and proximal jejunum. This
form of atresia may have a genetic component [116].
Small bowel atresia is often detected prenatally with
dilated bowel and polyhydramnios. Patients present soon
after birth with bilious emesis, abdominal distention, and
failure to pass meconium.
Prenatal ultrasound may show dilated, fluid-filled proxi-
mal bowel loops and evidence of in utero perforation as well
as polyhydramnios. Postnatally, a diagnosis of intestinal atre-
sia is usually made on plain radiographs. Ultrasound findings
include dilated proximal, blind-ending bowel with no gas in
308
the decompressed distal bowel. A small bowel “whirlpool”
sign located in the mid-abdomen and right lower quadrant
unrelated to midgut volvulus has been described with apple
peel atresia. This sign has been shown to better depict the
cause of obstruction when compared to barium studies [117].
Plain films will show dilated bowel loops proximal to the
atretic segment and an absence of distal gas. A “triple bubble”
appearance is seen with jejunal atresia that is equivalent to the
“double bubble” sign of duodenal atresia, with the third bub-
ble representing proximal jejunal dilation. Peritoneal calcifi-
cations related to in utero perforation may be seen (Fig. 10.25).
a b
c d
Fig. 10.25 Newborn infant with jejunal atresia. (a) Transverse grayscale
ultrasound image demonstrates dilated proximal small bowel (arrow)
with collapsed distal bowel loops. (b) Sagittal right upper quadrant gray-
scale ultrasound image reveals peritoneal calcifications (arrows) overly-
M. M. Munden and H. J. Paltiel
Contrast enema is performed to exclude additional sites of
atresia and associated malrotation. An unused microcolon will
be present with both jejunal and ileal atresia [22].
Treatment for bowel atresia is surgical. Apple peel atresia,
previously associated with a poor outcome, more recently
has a reported excellent long-term outcome although postop-
erative complications occur in the majority of patients [118].
J ejunal and Ileal Stenosis
Jejunal stenosis is a narrowing of the jejunum without disrup-
tion in continuity or defect in the adjacent mesentery. Stenoses
ing the surface of the liver (L). (c) Abdominal radiograph identifies
dilated proximal small bowel (black arrow) and peritoneal calcifications
(white arrow). (d) Contrast enema shows a microcolon and dilated, gas-
filled bowel loops (arrow) proximal to the atretic jejunum
10 The Gastrointestinal Tract
of the bowel are a relatively rare cause of neonatal obstruction,
accounting for approximately 5–11% of all jejunoileal obstruc-
tions [119]. At the stenotic site, there is often a short, narrow
segment with a minute lumen where the muscularis is irregu-
lar and the submucosa is thickened. Occasionally, there is an
obstructing membrane [120]. The resultant intestinal obstruc-
tion is incomplete. Clinically, there may be a history of mater-
nal polyhydramnios, and postnatally infants may present with
bilious emesis and abdominal distention. Often there is a fail-
ure to pass meconium. With incomplete obstruction, diagnosis
may be delayed.
A diagnosis of jejunal or ilieal stenosis is generally made
on plain radiographs or on small bowel follow-through exam-
inations that reveal proximally dilated loops with delayed
passage of contrast and normal caliber loops beyond the ste-
nosis. Ultrasound will show dilated loops proximal to the site
of stenosis.
Treatment is surgical with either limited intestinal resection
or longitudinal incision with resection of the stenotic segment.
Stomach
Duodenum
Ladd
bands
Cecum
Small
intestine
Intestinal
malrotation
a b
Fig. 10.26 Diagrams of intestinal malrotation and volvulus. (a) In intesti-
nal malrotation, there is a short mesenteric attachment. Ladd bands attach
the malrotated cecum in the right upper quadrant to the retroperitoneum.
309
Midgut Malrotation
Midgut malrotation refers to any deviation from the normal
270-degree counterclockwise rotation of the midgut during
development and presents clinically in approximately 1 in
2500 live born infants less than 1 year of age. Anatomically it
is much more common, occurring in 0.2%–1% of the normal
population [121]. Malrotation involves malpositioning of the
bowel with abnormal fixation. The normally broad mesenteric
attachment is foreshortened, predisposing to midgut
volvulus.
Ladd bands are fibrous peritoneal bands that attach the
malrotated cecum in the right upper quadrant to the retro-
peritoneum and extend over the second portion of the duode-
num where they may result in extrinsic compression or
obstruction (Fig. 10.26). The most severe complication of
malrotation is midgut volvulus where the mesenteric base
twists around the superior mesenteric artery (SMA), obstruct-
ing the bowel and compromising gut perfusion, a surgical
emergency.
Midgut
volvulus
The mesenteric base is predisposed to twist around the superior mesenteric
artery. (b) When midgut volvulus occurs and the mesenteric base twists
around the superior mesenteric artery, gut perfusion is compromised
310
Intestinal nonrotation is a subtype of malrotation in which
the small bowel is mainly located in the right hemiabdomen
and the cecum in the left hemiabdomen. The risk of volvu-
lus is much lower in complete nonrotation because patients
have the effective anatomy of someone who has undergone a
Ladd procedure. Reversed rotation occurs when a 90-degree
­clockwise rotation of the bowel occurs during development
rather than the normal 270-degree counterclockwise rota-
tion. The duodenum passes in front of the superior mesen-
teric artery, and the colon lies in front of the mesentery [121].
Patients born with omphalocele, gastroschisis, and con-
genital diaphragmatic hernias will have bowel malrotation. It
also frequently occurs in the setting of heterotaxy (30–90%
of patients), and is associated with bowel atresia, annual pan-
creas, and Hirschsprung disease [122, 123]. More than half
of affected individuals will present in the first month of life
with midgut volvulus and bilious emesis. Some patients have
more indolent symptoms resulting in chronic volvulus with a
delayed diagnosis [124].
Malrotation results in an abnormal position of the SMV
and the SMA, with the SMV located anterior to, or to the left
of the SMA rather than in normal position to the right of the
artery (Fig. 10.27). With midgut volvulus, a dilated proximal
a b
Fig. 10.27 Relationship of the superior mesenteric artery (SMA) to
the superior mesenteric vein (SMV). (a) Transverse grayscale ultra-
sound image depicts the SMV (arrow) normally positioned anterior and
M. M. Munden and H. J. Paltiel
duodenum is seen tapering at the site where the SMV twists
around the SMA, the so-called “whirlpool” sign (Fig. 10.28).
This sign has a reported 87% sensitivity for the detection of
midgut volvulus [125].
Recently, the “whirlpool” sign has been described in neo-
nates with congenital intestinal obstruction unrelated to mid-
gut malrotation, related instead to segmental volvulus of the
small intestine or coiled distal small intestine associated with
apple peel atresia [117].
Although these ultrasound findings are valuable, their
absence does not completely exclude malrotation or volvu-
lus, and an UGI series remains the reference standard for
diagnosis. Ultrasound documentation of a normal position
of the third portion of the duodenum posterior to the SMA
and anterior to the aorta has been shown to exclude bowel
malrotation [126]. CT can also document the retro-mesen-
teric, normal position of the third portion of the duode-
num, although it is not the imaging modality of choice in
young children. The obstructed bowel proximal to a vol-
vulus and the “whirlpool” sign can also be seen on CT.
Once diagnosed, malrotation with midgut volvulus is
treated with emergent surgery (Fig. 10.29). Malrotation
without associated volvulus is treated surgically on a non-
to the right of the SMA (arrowhead). (b) Transverse grayscale ultra-
sound image depicts the SMV (arrow) abnormally positioned anterior
and to the left of the SMA (arrowhead)
10 The Gastrointestinal Tract
Fig. 10.28 Midgut volvulus in a 2-week-old neonate presenting with
bilious emesis. (a) Transverse grayscale ultrasound image demonstrates
a dilated, fluid-filled stomach (S) and dilated third portion of the duode-
num (arrow) ending in a narrowed beak. (b) Transverse color Doppler
emergent basis with the Ladd procedure, where the abnor-
mal peritoneal bands are released, and the appendix is
removed.
Meconium Ileus
Meconium ileus occurs when the distal ileum is obstructed
by tenacious meconium. Meconium ileus presents soon after
birth with abdominal distention, bilious emesis, and delayed
passage of meconium. It can be suspected on prenatal ultra-
sound when intestinal obstruction is seen in association with
echogenic bowel. Approximately 20% of infants with cystic
fibrosis (CF) present with meconium ileus, many of whom
have the dF508 mutation on amniocentesis. The thick, inspis-
311
ultrasound image of the duodenum shows a “whirlpool” sign with the
superior mesenteric vein (arrow) and mesentery wrapped around the
superior mesenteric artery (arrowhead). (c) UGI series image reveals a
beak (arrow) at the site of obstruction caused by the volvulus
sated secretions from the exocrine glands in CF result in
abnormal meconium [127].
Meconium ileus typically presents in the neonate as a dis-
tal small bowel obstruction that responds to contrast enema
with resolution of the obstruction in 39% [128]. Meconium
ileus can be associated with bowel atresia, necrosis, and per-
foration. Perforation can occur in utero, leading to extrusion
of meconium and digestive enzymes into the peritoneal cav-
ity resulting in an intense chemical peritonitis followed by
peritoneal calcification.
In utero imaging will show polyhydramnios, ascites, and
matted echogenic bowel. There may be a focal mass and intra-
peritoneal calcification if perforation has occurred. Postnatal
312 M. M. Munden and H. J. Paltiel

ultrasound imaging shows the dilated small bowel loops, asci-

tes, echogenic meconium and extraluminal calcifications.
Plain films in the neonate may show proximally dilated loops,
absent air-fluid levels on decubitus or upright imaging, and a
“bubbly” pattern of distended intestinal loops (Fig. 10.30).
Calcifications and mass effect from a meconium pseudo-
cyst may be identified on plain films although ultrasound can
detect more subtle extraluminal calcification. A meconium
pseudocyst frequently manifests as a calcified soft tissue
mass on plain films, and as a mass with peripheral calcifica-
tion with color Doppler twinkling artifact and heterogeneous
content on ultrasound [129].
Ultrasound of meconium ileus shows bowel filled with
echogenic, thick meconium proximal to a microcolon, while
ileal atresia will demonstrate dilated loops of bowel filled
with air and fluid proximal to a microcolon. Both disorders
are associated with a microcolon on contrast enema. A diag-
nostic contrast enema is typically carried out to the level of
obstruction in the setting of atresia. With meconium ileus,
contrast enema with water-soluble material is both diagnos-
tic and therapeutic.
Non-operative management for simple meconium ileus
includes repeated attempts to dissolve the thick meconium
by water-soluble enema. Surgery is required for persistent
bowel obstruction, volvulus, and/or perforation [129].

 econium Peritonitis and Pseudocyst

M
Meconium peritonitis follows in utero bowel perforation,
leading to leakage of meconium into the peritoneal cavity
and a sterile, chemical inflammatory response. A meconium
cyst develops when the intraperitoneal meconium is con-
Fig. 10.29 Diagram of surgical release of Ladd bands in the setting of
malrotation with volvulus
tained by fibrous adhesions. Bowel loops can become

Fig. 10.30 Newborn male with meconium ileus. (a) Sagittal grayscale loops (arrowhead). (b) Supine abdominal radiograph demonstrates
ultrasound image of the left lower quadrant shows echogenic content dilated proximal bowel loops due to meconium ileus
within a collapsed microcolon (arrow) with dilated proximal bowel
10 The Gastrointestinal Tract
entrapped within the cyst, and mural calcification often
occurs. Bowel perforation can result from in utero volvulus,
bowel atresia, vascular accidents, and meconium ileus. In
one study, only 8% of patients with meconium peritonitis
had underlying cystic fibrosis [129].
A morbidity rate of 34% and a mortality rate of 2% have
been reported for infants with meconium peritonitis [130].
Prenatal ultrasound imaging commonly shows fetal asci-
tes and dilated bowel. Infants may present with intestinal
obstruction and pneumoperitoneum. A meconium pseudocyst
can present as a distended abdomen. Plain films may show
diffuse peritoneal calcifications. Postnatal ultrasound imag-
ing can show punctate echogenic peritoneal calcifications,
subtle free air, dilated small bowel loops, and complicated
ascites as well as the calcified mass of a meconium pseudo-
a b
c d
Fig. 10.31 Meconium pseudocyst in a newborn infant with calcifications
seen prenatally suspicious for in utero perforation. (a) Transverse gray-
scale ultrasound image shows a soft tissue mass (arrows) with internal
calcifications in the mid-abdomen. (b) Transverse color Doppler ultra-
sound image of the meconium pseudocyst demonstrates peripheral flow
313
cyst with heterogeneous contents (Fig. 10.31). Dilated loops
from an obstruction may be present as well. Subtle calcifica-
tions not seen on plain film can be detected by ultrasound.
For complex meconium ileus associated with bowel atre-
sia, necrosis and perforation, surgical intervention is manda-
tory [129].
Acquired Obstruction
Intussusception
Small Bowel Intussusception
Small bowel intussusception occurs when a segment of bowel
(the intussusceptum) telescopes into a distal segment (the intus-
(arrow) with no internal perfusion. (c) Sagittal grayscale ultrasound image
of the right upper quadrant reveals subtle calcification (arrow) along the
liver surface related to in utero bowel perforation. (d) Supine abdominal
radiograph does not reveal the hepatic calcification or the meconium
pseudocyst
314
suscipiens), and is a common etiology of acute abdominal pain.
Small bowel intussusception differs from the more common
ileocolic intussusception as it is frequently an incidental, tran-
sient finding on ultrasound. A small bowel intussusception can
persist when it occurs in association with a lead point such as a
Meckel diverticulum, polyp, or duplication cyst [131].
Symptomatic small bowel intussusception occurs in the
setting of Henoch–Schönlein purpura, celiac disease, Peutz–
Jeghers syndrome with small bowel polyps, and indwelling
gastrojejunal feeding tubes. Small bowel intussusception is
also a known complication after abdominal surgery, respon-
sible for 5–15% of all postoperative bowel obstruction [132].
Non-transient, symptomatic small bowel intussusception can
present with intermittent abdominal pain, vomiting, GI tract
bleeding, and occasionally as a palpable mass.
a b
Fig. 10.32 Transient small bowel intussusception found incidentally in
two different patients. (a) Transverse grayscale ultrasound image shows
a small diameter (arrow) small bowel intussusception that resolved dur-
a b
Fig. 10.33 Non-transient small bowel intussusception due to a polyp
in a 4-year-old male with abdominal pain. (a) Longitudinal grayscale
ultrasound image shows a small bowel intussusception (arrows).
M. M. Munden and H. J. Paltiel
Ultrasound is the imaging modality of choice for all types
of intussusception, with high sensitivity and specificity as well
as high negative predictive values, showing a “doughnut” or
“pseudo-kidney” sign of alternating bowel layers with internal
echogenic mesenteric fat and nodes. The sensitivity for diag-
nosing intussusception by ultrasound is reported as 98% with
a specificity of 98% [133].
A transient intussusception is usually asymptomatic, and
has been shown in several studies to be smaller than an ileoco-
lic intussusception, with a maximum diameter of less than
3.5 cm, with normal bowel wall thickness and no associated
dilated bowel loops (Fig. 10.32). Small bowel intussusceptions
with pathologic lead points are typically larger (Fig. 10.33).
Mean fat core diameter, the ratio of inner fat core diame-
ter to outer wall thickness, and the presence of lymph nodes
ing the study. (b) Axial contrast-enhanced CT image demonstrates a
transient small bowel intussusception (arrow) in a child with no GI
symptoms
(b) Coronal contrast-enhanced CT image identifies the extensive small
bowel intussusception (arrows) caused by a small bowel polyp identi-
fied at surgery
10 The Gastrointestinal Tract 315

have also been suggested as ways of differentiating small

a
bowel from large bowel intussusception [131, 134]. In post-
operative patients, plain films may show a small bowel
Colon
obstruction. In the past, ultrasound was not thought to be
reliable for the diagnosis of postoperative small bowel intus-
susception. However, a recent series found ultrasound to be
highly accurate [135]. CT also will show the site of a non-
transient intussusception and the level of bowel obstruction
in those cases where the diagnosis cannot be made by ultra- Intussuscipiens

sound or plain film. Barium studies are not typically useful.

Patients with non-transient small bowel intussusception Intussusceptum

and an identifiable lead point are treated surgically.

Management of non-transient small bowel intussusception
includes short-term observation to ensure resolution, or care-
Ileum
ful follow-up to confirm spontaneous reduction [136].

Ileocolic Intussusception
Ileocolic intussusception is an invagination of proximal bowel
(the intussusceptum) into more distal bowel (the intussuscipi- Cecum

ens) which occurs mainly due to hyperplasia of Peyer patches

in the terminal ileum that act as a lead point (Fig. 10.34).
Ileocolic intussusception is the most common abdominal
emergency in infancy and early childhood with approximately
two-thirds presenting in the first year of life. Infants less than
b
3 months of age are relatively spared [137]. More than 50% of
cases occur in children under 1 year of age, and 80–90% under
Colon
2 years of age [138, 139]. An underlying lead point is less
common, found in about 25% of patients, such as a duplica-
tion cyst, Meckel diverticulum, or lymphoma.
Intussusceptum
Cases of ileocolic intussusception associated with a lead
point tend to occur in older children. Following reduction,
the recurrence rate is from 8 to 15%, which should always
raise the question of a pathologic lead point. Intussusception
of the appendix can occur in isolation or as part of an ileoco-
Ileocecal
lic intussusception. The appendix is the lead point in about valve
Constricted
0.2% of all cases of ileocolic intussusception [140]. Isolated blood supply
appendiceal intussusception does occur in children, and the Cecum

majority of them will be inflamed [141]. Ileum

The typical clinical presentation for idiopathic intussus-

ception is a child with irritability, intermittent abdominal
pain, emesis, and bloody stools. “Currant jelly” stool, a Appendix

result of sloughed mucosa, blood, and mucus, occurs in a

minority of patients.
Ultrasound is the imaging modality of choice for the diag-
nosis or exclusion of intussusception, with a sensitivity
approaching 100% and a specificity of 88–100%. Ultrasound
features include a mass with concentric alternating hypo- and
hyperechoic rings of bowel resulting in a “doughnut” or tar-
get-like appearance on axial images and a “pseudokidney” on
longitudinal images. Internal echogenic mesenteric fat and
nodes are usually identified near the base of an intussuscep-
tion. The absence of blood flow on ultrasound suggests bowel
Fig. 10.34 Diagrams of ileocolic intussusception. (a) There is invagi-
nation of the terminal ileum (the intussusceptum) into the cecum and
ascending colon (the intussuscipiens) (b) The blood supply to the
invaginated ileum is constricted and may become compromised if the
obstruction is not relieved in a timely fashion
ischemia and the presence of interloop fluid correlates signifi-
cantly with ischemia and irreducibility (Fig. 10.35) [142].
A false-positive diagnosis of intussusception can occa-
sionally be made in other conditions that cause bowel wall
316
a b
Fig. 10.35 Ileocolic intussusception in two separate patients. (a) Trans­
verse grayscale ultrasound image of the right lower quadrant in an
18-month-old female with abdominal pain shows bulky mesenteric
nodes (arrowhead) adjacent to an ileocolic intussusception (arrow). (b)
thickening, such as Henoch–Schönlein purpura. Plain films
are often nondiagnostic, although occasionally a mass is
identified in the right mid- or upper abdomen with decreased
bowel gas in the right lower quadrant. Dilated small bowel
due to early obstruction can also be seen.
In the absence of peritonitis, bowel perforation, or shock,
pressure-monitored reduction is the first line of treatment for
ileocolic intussusception which can be performed fluoro-
scopically or with ultrasound, using liquid or air. The success
rate of air enema is approximately 83% compared to 70% for
liquid enema reduction [143]. Delayed repeat air enema in a
clinically stable patient is an option for an initially irreduc-
ible intussusception located at the ileocecal valve, with a
90% reported success rate [142]. Those intussusceptions that
cannot be reduced are surgically treated [144].
Small Bowel Wall Thickening
Infectious Enteritis
Gastroenteritis is defined as three or more loose or watery
stools in 24 hours, with or without vomiting, fever, or abdom-
inal pain. Bacterial enterocolitis in children can be caused by
a variety of organisms, including Shigella, Salmonella,
Escherichia coli, Yersinia, and Campylobacter. An estimated
211–375 million episodes of diarrheal illness occur each year
in the United States, many related to food-borne illness. In
2011, the incidence of Salmonella infections in the United
States was 1645 per 100,000 people [145].
Patients with sickle cell disease are at risk for Salmonella
infection due to splenic hypofunction, with associated high
morbidity and mortality rates. Campylobacter is one of the
leading causes of bacterial enterocolitis in the world, with
an estimated 1.3 million patients in the United States
M. M. Munden and H. J. Paltiel
Longitudinal color Doppler ultrasound image of the right lower quad-
rant in a 2-year-old male with bloody diarrhea and lethargy shows an
ileocolic intussusception with interloop fluid (arrow). Bowel perfusion
is preserved
affected each year. Campylobacter is present in the gastroin-
testinal tracts of poultry, cattle, swine, and sheep as well as
in dogs and cats. Disease transmission is usually food-borne
through undercooked meat or contaminated dairy products,
although contaminated water and ice can also spread the
disease. The incubation period is typically 2–5 days, although
it can be longer.
Viral gastroenteritis is even more common than bacterial
gastroenteritis, and according to the Centers for Disease
Control accounts for more than 200,000 deaths of children
worldwide per year [146]. Viral gastroenteritis commonly
causes outbreaks of enteritis on cruise ships, in daycare cen-
ters, and schools that are usually self-limited and resolve in
1–3 days. Transmission is often via the fecal–oral route.
Enteric viruses such as rotavirus and norovirus are leading
causes of gastroenteritis worldwide. Vaccines against rotavi-
rus were implemented in 95 countries in 2018 [147].
On ultrasound, viral gastroenteritis may demonstrate
prominent lymph nodes and ascites. The bowel wall is not
usually thickened. Rarely, pneumatosis can be seen in
infants and children infected with rotavirus [148].
Thickening of the terminal ileum and cecum occurs with
bacterial enteritis. Lymphoid hyperplasia with thickening
of the terminal ileum and mesenteric lymph node enlarge-
ment occur with Yersinia and Salmonella infection. Ascariasis
infestation is clearly shown when mobile worms are found
within the lumen of bowel, and the echogenic, well-devel-
oped digestive tract of the worm can also be imaged [149].
The sites of involvement in patients with enteritis is a key
differentiating feature on both ultrasound and CT. Parasitic
infections such as Giardia and Strongyloides involve the
proximal small bowel while bacterial infections such as
Salmonella, Shigella, and Yersinia affect the distal small
bowel (Table 10.2) [150].
10 The Gastrointestinal Tract
Table 10.2 Typical bowel involvement of common infectious organisms
Bowel segment Organism
Proximal small bowel Giardia, Strongyloides
Distal small bowel Salmonella, Yersinia, Shigella
Distal ileum and cecum TB
Ascending colon Yersinia, Salmonella
Cecum and ascending colon Campylobacter
Descending colon Shigella
Pancolitis Clostridium difficile, CMV,
Escherichia coli
TB, Tuberculosis; CMV, cytomegalovirus
Most cases of bacterial gastroenteritis are self-limited and
do not require stool cultures or antimicrobial therapy. Treatment
is supportive, with replacement of fluid and electrolytes. Stool
cultures are only indicated for patients with severe or prolonged
diarrhea, bloody diarrhea, presence of stool leukocytes, lacto-
ferrin, or occult blood. Treatment for viral gastroenteritis is also
symptomatic, with the goal of maintaining hydration.
Crohn Disease
Inflammatory bowel disease (IBD) represents a chronic inflam-
matory disease of the gastrointestinal tract that typically mani-
fests as episodes of relapsing inflammation. There is a peak
onset between 15 and 30 years of age. Inflammatory bowel
disease includes Crohn disease (CD), ulcerative colitis (UC),
atypical UC, and IBD-unclassified [151]. Approximately
25% of cases of IBD present before the age of 18 years, and
the incidence in children is increasing globally in both devel-
oped and developing nations [152].
Pediatric-onset IBD often has atypical features, making
classification more difficult. The upper GI tract is more
affected by IBD in children than in adults, and can occur
with both CD and UC. Crohn’s disease is characterized by
transmural inflammation of bowel, skip lesions, and symp-
toms that wax and wane. It is usually progressive, with half
of those affected experiencing complications such as fistulas
and strictures. Patients with a childhood onset of CD usually
have more progressive and extensive disease than those that
with onset of the disease in adulthood. Colonic involvement
is frequent in pediatric CD, especially the left colon. With
early-onset CD in children less than 5 years of age, there
may be isolated colonic disease [153].
Diagnosis of IBD can be challenging with patients often
experiencing atypical symptoms of diarrhea, rectal bleeding,
and abdominal pain as well as extra-intestinal symptoms
such as unexplained fever, arthritis, and chronic anemia.
Detection of disease activity is important for early institu-
tion of biologic therapy. The reference standard for assessing
response to therapy is endoscopy, an invasive procedure.
Transabdominal ultrasound is highly effective for detecting
inflammation due to Crohn’s disease and can be used both as
317
the initial study and for follow-up, which is ideal for children
due to its lower cost and lack of radiation when compared to
MR and CT enterography.
The ultrasound examination should begin in the ileocecal
region, given the predilection for involvement of the terminal
ileum. High-frequency transducers are used to evaluate focal
regions of interest, while lower frequency transducers are
used in the assessment of deep fluid collections, optimizing
settings for high contrast and low flow on Doppler settings. In
experienced hands, perianal ultrasound can be used to assess
abscesses and fistulas [154].
Detecting active disease can be clinically challenging, as
many patients are asymptomatic despite recurrent disease.
Bowel wall thickness is important for assessing disease activity
as is color Doppler. Bowel wall thickening (greater than
2.5 mm for small bowel) and loss of normal bowel wall strati-
fication are identified. With active disease, segments of bowel
are hyperemic, non-compressible, greater than 4–5 mm in thick-
ness, and demonstrate decreased peristalsis (Fig. 10.36). Thick­
ened loops with more than 2 vessels per square cm on color
Doppler are strongly associated with active disease [155].
Superior mesenteric arterial flow velocities are elevated in
patients with active disease. The mesentery may be thickened
and echogenic, corresponding to the creeping fat seen by
CT. Mesenteric nodes are commonly enlarged and hyperemic.
Contrast-enhanced ultrasound (CEUS) is a relatively new
technique that in adult patients has been shown to provide
both subjective and objective information about bowel wall
and mesenteric blood flow that is useful in determining dis-
ease activity in patients with IBD [156]. Shear wave elastog-
raphy has also been used to measure bowel stiffness, and
together with CEUS can distinguish between inflammatory
and fibrostenotic bowel [157]. To date, there have been only
a few descriptions of the use of either CEUS or shear wave
elastography in the evaluation of IBD in children [158, 159].
On ultrasound evaluation, strictures appear as thickened
bowel with luminal narrowing and more proximal dilated
bowel. The sensitivity of ultrasound in detecting strictures
varies between 60 and 85% [160]. Barium studies are less
sensitive than CT and MR enterography for the detection of
IBD. MR enterography is the noninvasive technique with the
highest diagnostic accuracy for evaluation of disease activity
in children and adults [161, 162]. However, its use is more
limited in younger children who require sedation.
The goal of treatment is to achieve mucosal healing which
is associated with improved clinical outcome. Biologic treat-
ment with anti-tumor necrosis factor agents such as inflix-
imab and adalimumab, the most potent drug class to induce
and lead to maintained mucosal healing, has benefited a large
portion of patient’s with CD of all ages [163]. When given
within 3 months of diagnosis, early biological treatment is
associated with significantly improved outcomes [164].
318
Fig. 10.36 Active Crohn disease in a 16-year-old female with fistula
formation and secondary abscess. (a) Transverse color Doppler ultra-
sound image of the right lower quadrant demonstrates wall thickening
and hyperemia (arrow) of the terminal ileum with adjacent fatty prolif-
eration (arrowhead). (b) Transverse extended field of view grayscale
ultrasound image of the right lower quadrant shows the substantial
thickening (arrows) of the distal ileum. (c) Transverse grayscale ultra-
sound image of the right lower quadrant depicts a fistula (white arrow)
between the abscess (arrowhead) and the diseased distal ileum (black
M. M. Munden and H. J. Paltiel
arrow). (d) Longitudinal color Doppler ultrasound image reveals hyper-
emia surrounding the abscess (calipers) and the adjacent fistula (arrow).
(e) Coronal contrast-enhanced CT image demonstrates a gas bubble
(arrowhead) within the abscess, and loss of the fat planes (arrow)
between the diseased ileal loops at the site of fistula formation. (f)
Coronal T2-weighted, fat-suppressed magnetic resonance (MR) image
depicts the diseased distal ileum (arrow) with adjacent fatty prolifera-
tion and an abscess (arrowhead) in the right lower quadrant
10 The Gastrointestinal Tract
Hemorrhage
Trauma
Duodenal hematoma in children is not uncommon, occur-
ring in the setting of both accidental and non-accidental
trauma, as well as a rare complication of endoscopic
biopsy. Blunt hollow visceral injury in children in the United
States ranges from 1% to 5% of cases of blunt pediatric
abdominal trauma, arising from handlebar and seat belt
injury as well as direct blows in the setting of non-acciden-
tal trauma [165].
Physical examination is usually suggestive of intestinal
injury in the majority of patients who present with general-
ized tenderness and abdominal bruising. Bowel injury in
non-accidental trauma is more frequent than in accidental
trauma [166]. There are rare reports of jejunal hematomas
resulting from blunt trauma. Intramural duodenal hematoma
following upper GI endoscopy is uncommon, usually in
patients with a bleeding diathesis [167].
In the setting of trauma and non-accidental trauma with
suspected bowel injury, CT is the imaging examination of
choice. A partial thickness tear of the bowel wall results in
a hematoma, most often occurring in the duodenum. CT
typically shows an eccentric mass extending into the bowel
lumen which can cause partial proximal obstruction [168].
Acutely, the hematoma will have slightly increased attenu-
ation becoming low attenuation with evolution. Ultrasound
can be useful in the follow-up of a known hematoma to reso-
lution, demonstrating circumferential or eccentric bowel wall
thickening, sometimes with adjacent free fluid (Fig. 10.37).
a b
Fig. 10.37 Duodenal hematoma in a 5-year-old female after duodenal
biopsy at endoscopy. (a) Transverse grayscale ultrasound image of the
mid-abdomen reveals marked distension of the duodenum (arrow) with
319
Duodenal hematoma is managed non-operatively with naso-
gastric tube suction, bowel rest, and parenteral nutrition [169].
Henoch–Schönlein Purpura
Henoch–Schönlein purpura (HSP) is a multisystemic hyper-
sensitivity-mediated small vessel vasculitis that affects chil-
dren between 3 and 10 years of age and frequently involves
the GI tract. It is the most common vasculitis in children.
Patients present with colicky abdominal pain, vomiting, and
melena. The purpuric rash is the earliest manifestation in
most patients. About 75% of affected children will develop a
transient arthritis of the large joints, and about 50% have GI
tract involvement. Hematuria can also occur. There is intra-
mural bowel hemorrhage that is confined to the mucosa and
submucosa. About 5% of patients with HSP will develop
complications related to bowel hemorrhage, including small
bowel and ileo-colic intussusceptions, massive GI hemor-
rhage, ileal perforation, and stricture [170, 171].
Ultrasound manifestations of HSP include bowel wall
thickening usually affecting the ileum, although the jejunum
and sometimes the duodenum are involved (Fig. 10.38).
Mesenteric adenopathy and ascites are often present. The
wall thickening of HSP is a known ultrasound mimic of intus-
susception. However, secondary intussusception does occur
in approximately 5% of patients with HSP and can be diag-
nosed by ultrasound [172].
Plain films may show focal bowel wall thickening. CT
can depict multifocal, circumferential bowel wall thicken-
ing with a target pattern of enhancing mucosal and serosal
layers, vascular engorgement of the mesentery and mesen-
teric adenopathy. Rare complications of massive hemor-
heterogeneous, echogenic material in keeping with blood clot. (b)
Coronal contrast-enhanced CT image shows the blood-filled, dilated
duodenum (arrow)
320
Fig. 10.38 Henoch–Schönlein purpura in a 6-year-old male. Sagittal
grayscale ultrasound images of the left lower quadrant (a) and midline
(b) show circumferential wall thickening (arrows) of small bowel with
rhage, ileal perforation, and stricture can also be readily
diagnosed [171].
The intramural hemorrhages of HSP will resolve spon-
taneously with conservative management in most patients.
Steroids are used in uncomplicated cases. Ultrasound
can be useful in documenting resolution of bowel wall
involvement.
Eosinophilic Gastroenteritis
Eosinophilic gastroenteritis is an uncommon disorder char-
acterized by the triad of eosinophilic infiltration of portions
of the GI tract, disordered GI function, and peripheral eosin-
ophilia. The etiology is unknown. Some cases may be
M. M. Munden and H. J. Paltiel
moderate ascites (arrowhead). B, Bladder (c) Sagittal color Doppler
ultrasound image reveals bowel wall hyperemia (arrow). The appear-
ance of the bowel loops mimics an intussusception
caused by a hypersensitivity to certain foods or other
unknown allergens. A family history of allergic disorders is
often present. The prevalence of eosinophilic gastroenteritis
in the United States is approximately 25 per 100,000 and is
most common in the northeastern United States and in urban
areas [173].
Any portion of the GI tract can be involved, although the
gastric antrum and the proximal small bowel are almost
always affected. Three subtypes have been described: muco-
sal, muscular, and serosal. In the mucosal form of disease,
there is focal or diffuse mucosal fold thickening, as well as
polyps, ulcerations, and luminal narrowing. Mucosal involve-
ment can lead to protein-losing enteropathy. The muscular
10 The Gastrointestinal Tract
form manifests as bowel wall stenosis or obstruction, rigid-
ity, and dysmotility. The presence of ascites is regarded as
the main feature of the serosal pattern.
Given that multiple layers of the bowel wall can be
involved in eosinophilic gastroenteritis, multiple abnor-
malities often coexist. Symptoms vary with the site and
type of involvement. The stomach and small bowel are
most commonly affected, causing abdominal pain, bloody
diarrhea, and rectal bleeding. The disease often has a
chronic and relapsing course. Allergic disorders, includ-
ing asthma and eczema, and food intolerances are present
in 45–63% of reported cases [174]. Peritoneal fluid shows
a predominance of eosinophilic white blood cells. Endoscopic
findings include friable mucosa, ulcers, and polyps.
Ultrasound features include thickening of the mucosal
folds and intestinal wall, luminal narrowing, and ascites. The
gastric findings of eosinophilic gastroenteritis are discussed
earlier in this chapter. On CT, a “halo” sign and the “araneid-
limb-like” sign have been described due to submucosal
edema causing layering enhancement of the bowel wall and
a spider-leg appearance. Pneumatosis is sometimes identi-
fied [175–177].
Treatment with corticosteroids is the mainstay of therapy.
In patients with a chronic and relapsing course, additional
long-term therapies may be required, including dietary
restriction, and treatment with leukotriene inhibitors and
azathioprine [174]. Ultrasound can be used to follow the
response to therapy.
Lymphangiectasia
Lymphangiectasia is a rarely encountered, chronic, debilitat-
ing condition that can occur anywhere in the body but most
often affects the pulmonary and intestinal lymphatics.
Intestinal lymphangiectasia (Waldmann disease) involves
focal or diffuse dilation of the mucosal, submucosal, and
subserosal lymphatics [178]. The diffuse, widespread form
has a reported association with Noonan and Turner syn-
dromes and trisomy 21 [179].
Lymphangiectasia is usually diagnosed before the third
year of life. Common symptoms of primary intestinal lym-
phangiectasia include persistent diarrhea, peripheral edema,
hypoproteinemia, and steatorrhea.
Ultrasound demonstrates segmental or diffuse bowel wall
thickening and mesenteric edema (Fig. 10.39). MR lym-
phangiography will show dilated central lymphatics.
Treatment is symptomatic with a low-fat diet of medium-
chain triglycerides, steroids, and albumin infusions. The
prognosis is good for infants presenting after the neonatal
period. Octreotide, an analogue of somatostatin, has been
successful in treating more severe cases. A prospective study
of sirolimus is currently underway [180].
321
Fig. 10.39 Lymphangiectasia in a 22-year-old female. Sagittal gray-
scale ultrasound image of the right lower quadrant of the abdomen
reveals marked, polypoid mural thickening of bowel loops which are
separated by echogenic, edematous mesenteric fat
Cystic Fibrosis
Cystic fibrosis (CF), an autosomal recessive disease, results
from a defect in the cystic fibrosis transmembrane regulator
(CFTR) which results in viscous secretions in multiple organ
systems, most notably the lung and gastrointestinal tract. CF
affects about 70,000 people worldwide. About 60% of individu-
als with CF are born with pancreatic exocrine insufficiency
which progresses to affect about 90% of patients over time.
GI manifestations of CF include bowel dysmotility which
can manifest as meconium ileus in the neonate, constipation,
small bowel bacterial overgrowth, and distal intestinal obstruc-
tion syndrome (DIOS). DIOS is defined as complete intestinal
obstruction with bilious emesis and/or fluid levels in the small
intestine on abdominal radiographs with a fecal mass at the
ileocecal region and abdominal pain and/or distention [181].
DIOS affects between 10% and 20% of older children and
adolescents with CF, with an increasing incidence with age.
Once an individual has had an episode of DIOS, the recur-
rence risk is as high as 77% [182]. Chronic excess pancreatic
enzyme replacement can result in a fibrosing colopathy with
inflammation of the ileocecal region. The DIOS that occurs in
older children results from thick mucus and undigested fecal-
like material that causes obstruction at the ileocecal region.
Small intestinal bacterial overgrowth can result from the poor
motility in patients with CF and presents with abdominal pain,
distention, diarrhea, steatorrhea, anemia, and weight loss.
On plain radiographs of the abdomen, DIOS appears as a
bubbly, granular mass of stool in the right lower quadrant.
While this appearance is useful in differentiating between con-
322
stipation and distal intestinal obstruction in CF patients, it is not
recommended as a standard diagnostic stool [181]. Ultrasound
and CT can be used to confirm the diagnosis by demonstrating
dilated loops of intestine with a fecal mass in the distal small
bowel (Fig. 10.40). The wall of the cecum will appear thick-
ened and there is usually pericecal fat stranding [183].
Chronic low-grade bowel obstruction in patients with CF
is treated with a variety of approaches that include adequate
hydration, osmotic laxatives such as polyethylene glycol,
and strict adherence to pancreatic enzymatic replacement.
Small intestinal bacterial overgrowth is treated with antibiot-
ics and probiotics. Newer therapies using lubiprostone, a
synthetic eicosanoid which activates the CLC2 chloride chan-
nel and enhancing fluid secretion into the gut, have become
available as an adjunct to therapy in selected cases [184].
Installation of water-soluble contrast into the colon and
ileum under fluoroscopic control has been found to reduce
the need for surgical intervention.
Graft-Versus-Host Disease
Graft-versus-host disease (GVHD) is an immunologic com-
plication resulting from functionally competent donor T lym-
phocytes responding to foreign human leukocyte antigens
(HLAs) expressed by recipient cells. GVHD is a common
complication of hematopoietic stem cell transplantation with
an incidence of up to 60% and affects the skin, GI tract, and
hepatobiliary systems. GVHD can be acute, developing within
a b
Fig. 10.40 Cystic fibrosis and distal intestinal obstruction syndrome
(DIOS) in a 15-year-old male. (a) Supine abdominal radiograph shows
several dilated upper abdominal bowel loops with a paucity of distal
M. M. Munden and H. J. Paltiel
the first 100 days after transplantation, or chronic. Acute
GVHD can be life threatening, and accounts for almost half of
posttransplantation deaths not related to recurrent tumors. The
number of chronic GVHD cases is increasing with an inci-
dence of about 40% in matched sibling donors and 70% with
unrelated donors [185].
In the gastrointestinal tract, there is epithelial cell apopto-
sis that can lead to frank necrosis and sloughing of mucosa.
The terminal ileum and colon are the predominant sites of
involvement. Patients present with nonspecific symptoms of
watery diarrhea, abdominal pain, GI bleeding, ileus, and
protein-losing enteropathy. Early detection is important for
optimizing outcome. The differential diagnosis includes
infection and drug-related toxicity [186].
Ultrasound findings in the GI tract include fluid-filled
and dilated bowel with an accentuated distinction between
bowel wall layers, marked bowel wall thickening, and asci-
tes (Fig. 10.41). Some bowel loops demonstrate hyperemia
with color Doppler while others have decreased, high-resis-
tance flow in the SMA which correlates with a poor
outcome.
Experimental studies have shown a damaged gut muco-
sal barrier in the walls of patients with GVHD [187]. CT
imaging shows bowel wall thickening with a “target” sign
due to low attenuation edema of the submucosal layer
located between the markedly enhancing mucosal and sero-
sal layers [188].
bowel gas concerning for obstruction. (b) Longitudinal grayscale ultra-
sound image of the right lower quadrant of the abdomen shows multiple
dilated, fecalized loops of small bowel
10 The Gastrointestinal Tract
a b
Fig. 10.41 2-year-old male with graft-vs-host disease after bone marrow
transplantation for treatment of leukemia presenting with abdominal pain.
(a) Transverse grayscale ultrasound image of the right lower quadrant dem-
onstrates two thick-walled loops of ileum surrounded by a small amount of
Treatment for acute and chronic GVHD is complex,
including improved conditioning regimens prior to trans-
plantation. The aim is to achieve immune reconstitution in
order to prevent disease relapse. Corticosteroids are used for
initial treatment followed by targeted therapies.
Meckel Diverticulum
Meckel diverticulum is the most common congenital anom-
aly of the GI tract. It is a remnant of the omphalomesenteric
duct and is present in about 2% of the population. The duct
typically obliterates in the first trimester of pregnancy.
Failure of obliteration can lead to a diverticulum, fistula, or
323
free fluid. (b) Longitudinal color Doppler ultrasound image of the right
lower quadrant shows significant mural hyperemia of a distal ileal bowel
loop. (c) Longitudinal power Doppler ultrasound image of the left lower
quadrant reveals a thickened, hyperemic, and edematous mesentery (arrow)
fibrous band. A Meckel diverticulum can vary in location,
with approximately 75% found within 100 cm of the ileoce-
cal valve, arising from the antimesenteric border of the distal
small bowel [189]. About 60% contain ectopic gastric mucosa
with hemorrhage the most common complication in chil-
dren, with a reported incidence of up to 55%.
Additional possible complications include bowel obstruc-
tion from a Meckel band, small bowel intussusception with
the diverticulum serving as the lead point, and umbilical dis-
charge [190]. Painless bright red blood per rectum is the
most common complication, and bleeding can be massive.
Acute Meckel diverticulitis is less common and presents in a
fashion similar to acute appendicitis. Rarely, a carcinoid
tumor can arise in a Meckel diverticulum.
324
For suspected hemorrhage related to a Meckel diverticu-
lum, technetium-99m pertechnetate scintigraphy is the imag-
ing modality of choice. Simultaneous activity will appear in
the stomach and in the ectopic gastric mucosa located within
the diverticulum.
Ultrasound plays a role in identifying complications related
to a Meckel diverticulum, such as small bowel intussusception
or obstruction caused by an omphalomesenteric band. Findings
associated with Meckel diverticulitis may include a blind-end-
ing tubular structure with mural thickening, and surrounding
inflammation (Fig. 10.42) [106]. Meckel diverticulum is not
often identified on ultrasound, barium studies, or CT [191].
The treatment for symptomatic Meckel diverticulum is surgi-
cal excision.
Fig. 10.42 Meckel diverticulum in a 19-month-old female with a his-
tory of anemia and rectal bleeding. Transverse (a) and longitudinal (b)
grayscale ultrasound images of the right lower quadrant of the abdomen
show a short, thick-walled, blind-ending tubular structure (arrows) sur-
M. M. Munden and H. J. Paltiel
Benign Masses
Duplication Cyst
Duplication cysts are congenital malformations of the
bowel with an epithelial lining containing bowel mucosa,
sharing a common wall with the bowel along it mesenteric
border. Duplication cysts occur in 1 of 4500 births, and can
be found anywhere along the gastrointestinal tract. They
are cystic or tubular in shape. The majority do not commu-
nicate with the adjacent bowel lumen. One third of all
duplication cysts are located in the ileum, the most com-
mon site. Ectopic gastric mucosa occurs in 20–30% which
can lead to complications such as hemorrhage, and ectopic
pancreatic tissue in 3%.
rounded by collapsed, matted loops of small bowel. (c) Coronal image
from a Meckel scan demonstrates intense uptake in the right lower
quadrant (arrow) that persisted at 30 minutes. The kinetics of this focus
paralleled that of the gastric mucosa
10 The Gastrointestinal Tract
Associated congenital anomalies, including spinal
defects, cardiac, or urinary tract malformations, occur in
16–26% [70]. When located in the distal small bowel, a
duplication cyst may serve as a lead point for intussuscep-
tion, obstruct the distal small bowel, and bleed or perforate if
it contains ectopic gastric mucosa. Duplication cysts are
often asymptomatic and found incidentally on both prenatal
and postnatal imaging.
High-resolution ultrasound imaging can readily depict
the 5 bowel wall layers of a duplication cyst (i.e., hyper-
echoic mucosa, hypoechoic muscularis mucosa, hyperechoic
submucosa, hypoechoic muscularis propia, and outer hyper-
echoic serosa). However, a multilayered appearance can also
be seen with a mesenteric cyst, abscess, and choledochal
cyst [192]. The “Y” sign has been described which repre-
a b
Fig. 10.43 Duplication cyst of the distal ileum in a 2-month-old asymp-
tomatic female with an abdominal lesion found prenatally. Transverse
grayscale (a) and color Doppler (b) ultrasound images of the right lower
325
sents the splitting of the shared hypoechoic muscularis pro-
pria between the cyst and adjacent loop of bowel, a sign not
described for other abdominal cysts. A subtle change in the
shape and contour of the cyst can be noted, even in the
absence of peristalsis of adjacent bowel, attributed to con-
traction of the muscular layer of the cyst.
Echogenic debris and internal fluid levels can be seen
when a duplication cyst is complicated by bleeding from
ectopic gastric mucosa or infection (Fig. 10.43). While CT
is not typically performed for diagnosis, a duplication cyst
may be found incidentally. CT is also used in older patients
to assess for associated complications. CT will show a c­ ystic
mass with a thin, slightly enhancing outer wall. Protein­
aceous material may be seen within the lumen after bleed-
ing. On MR imaging, cysts will be low in signal intensity on
quadrant show the more medial portion of a large, bilobed distal ileal
cyst (calipers, arrow) containing layering debris. (c) Transverse color
Doppler ultrasound image reveals marked mural hyperemia
326
T1-weighted images and high in intensity on T2-weighted
images [70].
Surgical treatment of duplication cysts is necessary because
of potential complications such as obstruction, intussusception,
hemorrhage, and infection.
Mesenteric Cyst
Mesenteric cysts arise from the small or large bowel mesentery
as a proliferation of lymphatic tissue that does not communicate
with the lymphatic system. They have a reported incidence of
fewer than 1 per 100,000 hospital admissions for abdominal
pain. A mesenteric cyst can occur anywhere along the mesen-
tery and can also extend into the retroperitoneum, but are most
commonly located in the ileal mesentery. They may present as
Fig. 10.44 Mesenteric cyst causing bowel obstruction in a 3-year-
old male with abdominal pain and vomiting. (a) Transverse grayscale
ultrasound image shows bowel (arrow) trapped within a large abdomi-
nal cyst (arrowheads) containing echogenic debris. (b) Axial contrast-
M. M. Munden and H. J. Paltiel
an abdominal mass or as an incidental finding on imaging.
Bowel obstruction has been reported in infants with rare reports
of intra-cystic bleeding and rupture [193]. Complications
include torsion, volvulus, hemorrhage, and infection.
On ultrasound, a mesenteric cyst can be unilocular or
multiseptated, thin or thick-walled, and can contain internal
echogenic debris (Fig. 10.44). The appearance is relatively
nonspecific, as many other lesions, including lymphatic mal-
formation, ovarian cyst, abscess, and meconium pseudocyst
can have similar imaging features. CT typically shows low
attenuation of the cystic lesion.
While most mesenteric cysts are incidental findings, min-
imally invasive surgery is the treatment of choice when they
are symptomatic.
enhanced CT image of the abdomen depicts the collapsed bowel loop
(arrow) trapped within the large cystic lesion. (c) Surgical specimen of
the resected mesenteric cyst
10 The Gastrointestinal Tract
Intestinal Polyp
Intestinal polyps are epithelial or submucosal growths that
protrude into the bowel lumen. The juvenile polyp is the
most common polyp in children, and occurs within the colon.
Numerous polyposis syndromes are associated with small
bowel polyps. Peutz–Jeghers syndrome is the most common
polyposis syndrome. The polyps are hamartomatous, and are
not considered to be premalignant lesions. Juvenile polypo-
sis syndrome is associated with small and large bowel ham-
artomatous polyps as well as gastric polyps, with a high risk
of developing malignancy [194]. Small bowel polyps can
serve as lead points for intussusception. Children with pol-
yps often present with bleeding or pain.
Small polyps can be identified on fluoroscopic contrast
studies of the GI tract. On ultrasound, a polyp can occasion-
ally be detected as the lead point of a small bowel intussus-
ception (Fig. 10.45). Surveillance of patients predisposed to
small bowel polyps is generally performed with CT enterog-
raphy, MR enterography, and capsule endoscopy [195].
Small bowel polyps are removed either by endoscopy or
by surgery.
Vascular Anomalies
Vascular anomalies are a biologically and morphologically
diverse group of vascular channel abnormalities. Some vas-
Fig. 10.45 Polyp acting as lead point for small bowel intussusception
in a 14-year-old female with Peutz-Jeghers syndrome. Transverse gray-
scale ultrasound image shows a large intraluminal hypoechoic polyp
(arrow)
327
cular anomalies are comprised of a single vessel type such
as a vein, artery, capillary, or lymphatic vessel, while more
complex lesions can involve a combination of vessel types.
Lesions may be solitary, multiple, or part of a multisystemic
condition. The classification of vascular anomalies is based
on a combination of endothelial characteristics, biological
properties, and clinical behavior.
According to the classification scheme of the International
Society for the Study of Vascular Anomalies (ISSVA), vas-
cular anomalies are broadly classified into two categories,
tumors and malformations. These categories are further sub-
divided according to the vascular channels involved and other
biological characteristics. Named entities with other associ-
ated anomalies are also included. Some vascular anomalies
with features of both tumor and malformation remain provi-
sionally unclassified [196].
Vascular anomalies involving the GI tract manifest clini-
cally with four distinct patterns of signs and symptoms: (1)
GI bleeding, overt or occult; (2) abdominal pain due to mass
effect or intestinal obstruction; (3) abdominal distension,
hepatomegaly, high-output cardiac failure due to arteriove-
nous shunting, for example, hepatic hemangioma, or arte-
riovenous fistula; and (4) diarrhea, edema, ascites, and
hypoalbuminemia due to intestinal lymphangiectasia [196].
Infantile Hemangioma
Infantile hemangioma is the most common benign tumor of
infancy, occurring in 4–5% of term neonates, and as high as
23% in premature infants [197]. Infantile hemangioma
affecting the bowel and mesentery is rare [198]. On ultra-
sound, hypoechoic tumor masses are identified in the wall of
the small bowel and adjacent mesentery (Fig. 10.46). The
ascending colon is occasionally involved as well. The SMA,
SMV, and portal vein will appear dilated. Similar findings
are shown by CT and MR imaging [198]. Patients are usually
successfully treated with propranolol or corticosteroids.
Blue Rubber Bleb Nevus Syndrome
Blue rubber bleb nevus syndrome (BRBNS), a multifocal
venous malformation, is a congenital vascular anomaly that
often presents with multiple skin and soft tissue lesions accom-
panied by recurrent or chronic GI bleeding. Intestinal lesions
range in number from tens to hundreds. The lesions typically
have a sessile, polypoid appearance. Individual lesions can
involve any or all layers of the bowel wall. Histologically, the
lesions are composed of thin-walled venous channels that
include large cystic zones. Patients often present with GI bleed-
ing in early childhood that results in chronic anemia and a
dependency on repeated red blood cell transfusions or intrave-
nous iron administration.
328
a b
c d
Fig. 10.46 Mesenteric hemangioma in a 7-week-old female who pre-
sented with anemia and rectal bleeding. (a) Transverse grayscale ultra-
sound image of the abdomen shows a well-circumscribed, echogenic
mass (arrows) in the left upper quadrant that contains several enlarged
vessels (arrowheads), and surrounds and encases bowel loops. (b)
Transverse contrast-enhanced ultrasound (CEUS) image obtained in the
Multiple imaging modalities are often needed to show
all lesions. There is generally a limited role for ultrasound
in the evaluation of the GI tract lesions of patients with
BRBNS. Labeled red blood cell scintigraphy is the best
noninvasive method for revealing small bowel i­ nvolvement.
Endoscopic ultrasound also plays a valuable role. Imaging
is used to evaluate complications, particularly those stem-
ming from gastrointestinal lesions, and to plan appropriate
therapy [199].
In the past, control of bleeding associated with BRBNS
has relied on endoscopic or surgical ablation or resection.
M. M. Munden and H. J. Paltiel
arterial phase shows diffuse enhancement of the mass. (c) Transverse
CEUS image obtained in the venous phase shows rapid washout of con-
trast from the mass and s­ everal large draining mesenteric veins (arrow-
heads). (d) Coronal contrast-enhanced, T1-weighted, fat-suppressed
MR image shows relatively hypo-enhancing loops of bowel (arrowheads)
surrounded by markedly enhancing tumor (arrow)
More recently, Sirolimus has been used successfully to con-
trol BRBNS-related bleeding [196, 200].
Cutaneovisceral Angiomatosis with Thrombocytopenia
Cutaneovisceral angiomatosis with thrombocytopenia (CAT),
also called multifocal lymphangioendotheliomatosis with
thrombocytopenia (MLT), is a multifocal vascular anomaly
that shares features of both tumor and malformation and,
therefore, remains provisionally unclassified [196]. Imaging
evaluation has not played a significant role in the diagnosis of
these patients to date [201].
10 The Gastrointestinal Tract
Malignant Masses
 odgkin and Non-Hodgkin Lymphoma
H reported 23% of cases, affecting the distal small bowel,
Hodgkin and non-Hodgkin lymphoma (NHL) are common
malignancies in children. Hodgkin lymphoma is more com-
mon in adolescents and involves the nodal system. Non-
Hodgkin lymphoma is more common in children and more
frequently involves the GI tract. Non-Hodgkin lymphoma is
diagnosed in approximately 700–800 children per year in the
United States. Burkitt and Burkitt-like lymphomas represent
the most frequent subtype of non-Hodgkin lymphoma in
childhood, accounting for 35–40% of NHL [98]. The spo-
radic form seen in North America is associated with Epstein–
Barr virus in 15% of cases.
a b
Fig. 10.47 Burkitt lymphoma acting as a lead point for small bowel
intussusception in a 6-year-old male with abdominal pain. (a) Longitudinal
grayscale ultrasound image of the abdomen reveals multiple heteroge-
neous soft tissue masses (arrows) within the mesentery. (b) Longitudinal
grayscale ultrasound image reveals a mass in the left lower quadrant
with a bowel-within-bowel appearance. The bowel walls are markedly
329
Most pediatric cases of NHL are high grade with aggres-
sive behavior. Burkitt lymphoma involves the GI tract in a
cecum, and appendix most frequently, with abdominal or pel-
vic masses in 45%. Patients present with a palpable mass,
obstructive symptoms, and/or pain. Ileocolic intussusception
is a rare mode of presentation. Burkitt lymphoma is the most
rapidly growing tumor in children [99].
Abdominal radiographs may show bowel wall thickening
with mass effect and calcification. Ultrasound will demonstrate
diffuse bowel wall thickening with adjacent nodal masses in the
mesentery and retroperitoneum [99]. Intussusception secondary
to Burkitt’s lymphoma can also be identified (Fig. 10.47).
Imaging with intravenous contrast-enhanced CT and positron
thickened. There is a thin crescent of fluid (arrow) between the intus-
suscepted loops of bowel, as well as a small amount of adjacent free
fluid (asterisk). (c) Axial contrast-enhanced CT image of the abdomen
shows the intussusception (arrow) in the left lower quadrant with proxi-
mal thick-walled bowel loops (arrowheads)
330 M. M. Munden and H. J. Paltiel

emission tomography (PET)/CT provide anatomic and meta-
bolic information that are the current standard of care. PET/MR
imaging may prove to be an acceptable alternative in providing
high-quality images with a reduction in radiation dose com-
pared with PET/CT [98].
Chemotherapy is the main treatment for childhood Burkitt
lymphoma.
of the appendix and for surrounding inflammatory changes or
abscess. Dependent regions are evaluated for complex fluid
collections, especially in children under the age of 4 years who
lack a well-developed omentum to contain a perforation.
Assessment should include all four abdominal quadrants and
the pelvis to search for abscess collections.

Normal Development and Anatomy

Appendix
Normal Development
Technique
The cecal diverticulum arises from the midgut in week 6 of
Patient Positioning gestation and is the precursor of the cecum and the appendix.
The patient is scanned in a supine position and asked to point At 8 weeks the appendix is histologically identifiable. As the
to the site of maximal tenderness. As the majority of appen- colon elongates, the cecum and appendix undergo medial
dixes are retrocecal in location, it is important to image the rotation along with the midgut and descend into the lower
paracolic gutter to search for a retrocecal appendix. If the right side of the abdomen (Fig. 10.9). In weeks 14 and 15 the
appendix is not identified, placing the patient in a left lateral appendiceal mucosa develops lymphoid tissue.
decubitus position while applying gentle anterior compres-
sion with the ultrasound transducer and posterior compres-
sion with the non-scanning hand will separate bowel loops Normal Anatomy
and often improves visualization of the appendix. Another
approach is to place the patient prone for several minutes, The normal appendix has a diameter less than 6 mm with
and then re-image in a supine position. compression and a trilaminar appearance with a thin, echo-
genic central stripe (Fig. 10.48). The length can be up to
Ultrasound Transducer Selection 10–12 cm. Single wall thickness is less than 2 mm. Normal
The highest resolution linear ultrasound transducer opti- diameter is usually on the order of 3–4 mm. The lamina pro-
mized for bowel wall detail is used that will permit adequate pria normally contains lymphoid follicles which can hyper-
penetration, usually on the order of 15–18 MHz in children. trophy in response to gastroenteritis.
If needed, a lower resolution transducer of about 9 MHz can
be used to search for the appendix deep in the right lower
quadrant or pelvis. Less often, an even lower frequency cur-
vilinear transducer may be needed to assess the abdomen and
pelvis for adequate imaging of the findings associated with
appendiceal perforation.

Imaging Approaches
A methodical approach is undertaken to identify the appendix
which is usually located 2 cm distal to the terminal ileum.
Initial assessment is made using gentle, yet firm graded com-
pression in the right lower quadrant at the site of maximal ten-
derness. If the appendix is not readily seen, imaging of the
lateral border of the ascending colon is performed followed
caudally to the level of the cecum. The appendix is retrocecal
in 65% of patients [202–204]. Abdominal guarding can be
used as a guide to find the appendix which is sometimes
located deep in the pelvis or posterior and deep to the iliac ves-
sels. The tip of the appendix must be found to differentiate the
appendix, a blind-ending viscus, from the terminal ileum.
Fig. 10.48 Normal appendix in a 4-year-old male complaining of right
Appendiceal diameter is measured with compression, from lower quadrant pain. Transverse grayscale ultrasound image shows a
serosa to serosa. Color Doppler is used to assess for hyperemia normal, slender appendix (arrows) with a trilaminar appearance of the
wall draped (arrows) across the psoas muscle and common iliac vessels
10 The Gastrointestinal Tract
Acute Appendicitis
The overall incidence of appendicitis is 7% and is the most
common cause for emergency surgery in children. In accor-
dance with the ALARA principle, both the American College
of Radiology and the American Academy of Pediatrics recom-
mend ultrasound as the initial imaging modality for evaluation
of suspected appendicitis. The more slender the body habitus,
the better the imaging detail. However, even some children
with a large body habitus can be successfully imaged by ultra-
sound for appendicitis. A body mass index of less than 30 is
used by some centers as a cut-off for ultrasound evaluation.
On ultrasound, an acutely inflamed appendix appears as a
blind-ending tubular structure that is non-compressible and
measures 6 mm or more in diameter. The size of the appen-
dix can vary significantly for both normal and abnormal
appendixes, and the 6-mm criterion is more useful for exclud-
ing appendicitis than for confirming it (Fig. 10.49).
Ultrasound findings of acute appendicitis include a diam-
eter of 6 mm or more with compression, an outer wall thick-
ness greater than 3 mm, hyperemia, surrounding echogenic
mesenteric fat, and adjacent echogenic fluid (Table 10.3).
Thinning of the appendiceal wall to less than 2 mm in thick-
ness and right lower quadrant loculated fluid collections have
been shown to be highly specific (>90%) for perforation.
The presence or absence of an intraluminal fecalith should
be noted. A small amount of simple fluid may be seen adjacent
a b
Fig. 10.49 Acute retrocecal appendicitis in a 9-year-old female with
2 days of right lower quadrant pain. (a) Sagittal grayscale ultrasound
image of the right paracolic gutter demonstrates a thickened outer
appendiceal wall and distended lumen measuring more than 6 mm in
Table 10.3 Ultrasound features of the normal and abnormal appendix
Normal appendix Appendicitis
Diameter with compression < 6 mm > 6 mm
Outer wall thickness < 3 mm Increased outer wall thickness > 3 mm
Hyperemia No Yes
Surrounding inflammation No Yes
331
to the appendix, even in the absence of perforation. Mesenteric
lymphadenopathy frequently accompanies appendicitis, but
by itself is a nonspecific finding also seen with other types of
abdominal inflammation. Thickening and increased echogenicity
of the periappendiceal mesenteric fat is a valuable secondary
finding of appendicitis. The presence of more than one ultra-
sound sign of inflammation should be considered strong evi-
dence of appendicitis, even if other definitive signs are absent
[204]. Secondary abscess formation and an extruded fecalith
are sometimes identified (Figs. 10.50 and 10.51).
Imaging pitfalls include lymphoid hyperplasia, where the
muscular wall will be thickened but smooth from hyper­
trophied lymphoid follicles and the lumen will contain no
­exudate. With lymphoid hyperplasia, the appendix may be
non-compressible, with a 6–8-mm diameter. In the absence
of periappendiceal inflammatory fluid, hyperechoic periap-
pendiceal fat, and mural hyperemia, there is a low likelihood
of appendicitis (Fig. 10.52) [205]. Fecal impaction can also
distend the appendix, but the normal bowel wall layers will
be maintained and there will be no associated hyperemia or
surrounding inflammatory changes (Table 10.3) [206].
Treatment of acute appendicitis has historically been surgi-
cal appendectomy. However, there is now evidence that uncom-
plicated cases of acute appendicitis can be treated with antibiotics
alone [207]. Therefore, features of complicated appendicitis,
including the presence or absence of a fecalith, signs of perfora-
tion, and fluid collections are important to document.
diameter (­calipers). There is minimal adjacent free fluid. (b) Sagittal
color Doppler ultrasound image reveals marked hyperemia of the
inflamed appendiceal wall (arrow)
Lymphoid hyperplasia Inspissated stool
Can be > 6 mm Can be > 6 mm
Thickened lamina propria Normal
Sometimes No
No No
332
Fig. 10.50 Perforated appendicitis in a 12-year-old male presenting
with 4 days of right lower quadrant pain. Transverse color Doppler
ultrasound image of the right lower quadrant demonstrates a focal fluid
collection (arrow) adjacent to a distended appendix (arrowhead)
Fig. 10.51 Large fecalith with perforated appendicitis in a 14-year-old
female. Transverse color Doppler ultrasound image shows a large,
shadowing fecalith (arrow) within a distended appendix. There is
marked thickening of the surrounding soft tissues
Cystic Fibrosis of the Appendix
The diameter of the appendix in asymptomatic patients with
CF is often greater than 6 mm due to mucoid and stool
impaction with a reported mean diameter of 8.3 mm
M. M. Munden and H. J. Paltiel
Fig. 10.52 Lymphoid hyperplasia of the appendix in a 10-year-old
male with right lower quadrant pain. Transverse color Doppler ultra-
sound image of the right lower quadrant shows an enlarged, thick-
walled and hyperemic appendix (arrow)
Fig. 10.53 Inspissated fecal material in the appendix of a 15-year-old
female with cystic fibrosis. Sagittal grayscale ultrasound image of the
appendix (cursors) reveals echogenic intraluminal material (arrow) but
no thickening of the wall
(Fig. 10.53) [208]. Acute appendicitis in patients with cystic
fibrosis is rare, with an incidence of 1–2% compared with an
overall incidence of 7% in the general population [209].
Chronic abdominal pain is common in patients with CF, who
also frequently receive antibiotics for treatment of pulmo-
nary infections. Both of these factors can delay the diagnosis
of acute appendicitis. As a consequence, appendiceal perfo-
ration and other complications of acute appendicitis are
higher in patients with CF than in the general population.
An ultrasound diagnosis of acute appendicitis in a CF patient
should therefore be based on signs of inflammation, including focal
tenderness with compression, loss of definition of wall layers, sur-
rounding omental or mesenteric fat infiltration, and abnormal intra-
peritoneal fluid, rather than appendiceal diameter.
10 The Gastrointestinal Tract
Benign Masses of the Appendix
Mucocele of the Appendix
Mucocele of the appendix refers to a cystically dilated appen-
dix filled with mucin caused by a non-acute obstruction of the
appendiceal neck. It is very rare in children, typically occur-
ring in middle-aged adults [210].
Malignant Tumors of the Appendix
Carcinoid
Malignant neoplasms of the appendix are rare, occurring in
approximately 1% of appendectomy specimens, with carcinoid
tumor the most common appendiceal malignancy [211].
Carcinoid tumor is a neuroendocrine neoplasm and is often found
incidentally on pathological examination of appendectomy spec-
imens and tends not to be aggressive. About 75% of tumors are
localized to the appendiceal tip. A rare, functioning tumor can
produce vasoactive substances and manifest as carcinoid syn-
drome with diarrhea and wheezing. However, most carcinoid
tumors of the appendix are much more likely to present clinically
as acute appendicitis rather than with carcinoid syndrome.
Ultrasound findings are those of typical appendicitis with
carcinoid tumor usually discovered unexpectedly on patho-
a b
Fig. 10.54 Lymphoma of the appendix in a 14-year-old male with
large B-cell lymphoma. Sagittal (a) and transverse (b) grayscale ultra-
sound images of the right lower quadrant of the abdomen reveal a mark-
edly thickened, hypoechoic appendix, with loss of definition of the
333
logic examination. Calcification in an appendiceal carcinoid
has been reported to mimic an appendicolith [212].
Treatment is surgical, sometimes requiring removal of the
adjacent cecum and right colon for larger masses. As the
majority of carcinoid tumors are localized to the tip of the
appendix, surgery is curative if the mass is less than 2 cm in
size [213, 214].
Lymphoma of the Appendix
The GI tract is the most common site for extra-nodal
NHL. The distal small bowel, terminal ileum, cecum, and
appendix are common sites of involvement. Most reported
pediatric cases of appendiceal lymphoma are caused by the
Burkitt lymphoma subtype. The incidence of Burkitt lym-
phoma has been increasing over the last several decades.
The median age of children with Burkitt lymphoma is 8 years
and the tumor doubling time is approximately 24 hours.
Most patients present with abdominal pain, a palpable mass,
and obstruction, as well as symptoms that can mimic acute
appendicitis. Actual appendiceal involvement is rare,
reported to be 1–3% [99].
Ultrasound findings include enlargement of the appendix
with diffuse mural thickening (Fig. 10.54). CT cases of the
rare appendiceal lymphoma have shown soft tissue masses in
the right lower quadrant with no discernible normal appen-
normal wall layers. (c) Transverse grayscale ultasound image shows
that the appendix (arrowhead) abuts a large right lower abdominal and
pelvic soft tissue mass (arrows)
334
dix [215]. PET/CT is performed to evaluate for additional
sites of disease.
Treatment depends on extent of disease, and in children
involves aggressive chemotherapy. Five-year survival is between
80% and 90% [216].
Colon
Technique
Patient Positioning
For ultrasound study of the large bowel, the patient is usually
examined in a supine position but can be placed in a left lat-
eral decubitus position with posterior manual compression
while scanning from an anterior approach, gently compress-
ing anteriorly with the ultrasound transducer to separate
bowel loops.
Ultrasound Transducer Selection
A high-resolution linear transducer on the order of 12–18 MHz
and optimized for bowel technique with a low wall filter and
low pulse repetition frequency is used to initially evaluate
the entire colon. The bowel wall is assessed with grayscale
and color Doppler imaging for thickness, definition of wall
layers and hyperemia (Fig. 10.55). Cine loops are used to
evaluate colonic motility. If an abnormality is encountered, a
lower frequency transducer is used to identify abnormal fluid
collections or abscesses in all dependent regions of the abdo-
men and pelvis.
Fig. 10.55 Normal ascending colon in a 16-year-old male. Sagittal
grayscale ultrasound image reveals a thin wall (between arrows) with
normal definition of wall layers
M. M. Munden and H. J. Paltiel
Imaging Approaches
In the emergent setting, no bowel preparation is required.
If a study is scheduled to assess for IBD, a patient ideally
should be fasting for 4 hours prior to ultrasound evalua-
tion. Hydration with clear liquids distends the bladder
which can be used as an acoustic window, and also dis-
places small bowel loops from the pelvis. The bladder can
be emptied after the initial assessment to perform deeper
compression.
The colon should be studied in transverse and sagittal
planes, beginning with the right lower quadrant. The posi-
tion of the colon and the presence of haustra aid in distin-
guishing the colon from the small bowel. The rectum is
imaged by using the fluid-filled bladder as an acoustic
window. Pathological colonic wall thickening is easily
detected, as well as hyperemia. Normal colon typically
shows little vascularity. A retrograde saline enema can be
used to help identify a polyp or mass. The five layers of
the colonic wall should be readily depicted in most
patients [105].
Normal Development and Anatomy
Normal Development
By the fourth week of gestation, the gastrointestinal tract has
divided into foregut, midgut, and hindgut. The small and
large intestines undergo rapid growth in the fourth and fifth
weeks of life and quickly exceed the available space in the
abdominal cavity. The midgut herniates into the umbilical
cord, forming a loop. The superior limb of the intestinal loop
forms the ileum while the inferior limb forms the colon. The
herniated intestine rotates ninety degrees counterclockwise
around the mesentery so that the proximal portion of the loop
migrates from a superior position to the right side and the
distal portion of the loop migrates from an inferior position
to the left. The superior limb of the loop forms the ileum
while the inferior limb forms the colon.
The herniated intestine rotates ninety degrees counter-
clockwise around the mesentery so that the inferior limb
migrates to the left. In the tenth week, the bowel retracts
back into the abdominal cavity and rotates 180 degrees coun-
10 The Gastrointestinal Tract
terclockwise. The cecum is then positioned in the right upper
quadrant of the abdominal cavity. Colonic growth displaces
the cecum into its final position in the right lower quadrant
(Fig. 10.9).
The hindgut initially consists of the cloaca, which is later
separated by a septum into a dorsal GI compartment and a
ventral urogenital compartment (Fig. 17.5). The blood sup-
ply of the hindgut comes mainly comes from inferior mesen-
teric artery. The hindgut subsequently differentiates into the
distal third of transverse colon, the descending colon, sig-
moid colon, rectum and upper anus. The transverse colon
and sigmoid colon have a mobile mesentery, while the mes-
entery of the descending colon becomes fixed to the dorsal
wall. The upper third of the rectum is ultimately intraperito-
neal in location, the middle third retroperitoneal and the
lower third infraperitoneal along with the superior anus.
Normal Anatomy
The colon is the largest portion of the intestine where water
is absorbed from the digestive content and waste material is
stored as feces. Bacterial fermentation of undigested mate-
rial takes place in the colon. The first portion of the colon is
the cecum, joined to the terminal ileum at the ileocecal
valve. The cecum continues to the ascending colon, trans-
verse colon, descending colon, sigmoid colon, and rectum.
As described in the Development section above, the ascend-
ing colon, descending colon, and rectum are normally fixed
in the retroperitoneum while the remainder of the colon is
intraperitoneal. The transverse colon is encased in perito-
neum and connected to the posterior abdominal wall by the
transverse mesocolon. Unlike the small bowel, the colon has
distinct haustral markings caused by contraction of the tae-
niae coli, three bands of smooth muscle that run the length of
the colon.
The arterial supply to the colon is via the superior and infe-
rior mesenteric arteries and a connecting marginal artery that
runs the length of the colon. Normal colonic wall thickness is
2 mm [105].
335
Congenital Anomalies
Anorectal Malformations
Anorectal malformations are among the most common
congenital anomalies found in the newborn period, occur-
ring in approximately 1 in 5000 births, and ranging from
minor to very complex lesions. Thirty-six percent are
reportedly isolated lesions while about 60% are associated
with other abnormalities. Anorectal malformations occur
above the levator muscles in about 45%, and are low in
position, passing through the levator ani muscles in about
55% [217].
Anorectal malformations frequently occur as a compo-
nent of the VACTERL association. The more proximal the
colonic malformation, the more frequent the associated
anomalies. The incidence of associated urinary tract anoma-
lies with imperforate anus ranges from 26% to 50%.
Associated chromosomal defects occur in 8% of patients
[218]. Patients present at birth with bowel obstruction, fail-
ure to pass meconium, and an absent or abnormal anus. High
imperforate anus is often associated with a rectovesical or
rectourethral fistula.
When urine mixes with meconium, a characteristic enter­
olithiasis can be seen by both plain radiography and ultra-
sound. Ultrasound can be used to confirm its intraluminal
location and differentiate it from extraluminal meconium
peritonitis [106]. Transperineal high-resolution ultrasound
imaging can demonstrate the site of the fistula and the dis-
tance of the blind-ending rectum to the perineal skin surface
(Fig. 10.56). The study should not be performed in a crying
infant as the increased abdominal pressure moves the rectal
pouch closer to the perineal surface. A pouch-to-perineum
distance of 15 mm has a sensitivity of 100% and a specificity
of 86% in separating low from high and intermediate forms
of imperforate anus [219].
Treatment of anorectal malformations is surgical. If the
rectum is low in position, a single procedure can be per-
formed to pull the rectum through the anal sphincter. If the
rectum is in a high position, a three-staged correction is
typical with the first stage consisting of colostomy to allow
336 M. M. Munden and H. J. Paltiel

a b

c d

Fig. 10.56 Newborn female with low imperforate anus and meco-
nium passing from the vagina. (a) Sagittal transperineal grayscale
ultrasound image shows the anus (arrow) just deep to the skin surface
with small echogenic bubbles (arrowhead) in the rectovaginal fistula.
(b) Transverse grayscale ultrasound image demonstrates a stool-filled
rectum (arrow) posterior to the decompressed bladder (B). (c) Prone
abdominal radiograph with metallic marker (arrow) placed over distal
rectum during the ultrasound study underestimates the distal extent of
the rectum. (d) Transverse grayscale ultrasound image of the right
lower quadrant reveals a large amount of stool (arrow) within the sig-
moid colon

stool passage and creation of a mucous fistula to permit
mucous drainage. A colostogram is then performed to assess
the distance of the blind-ending rectum from the skin sur-
face and to assess for the presence of a fistula. The second
operation is performed to close the fistula. The third proce-
dure is closure of the colostomy.
Voiding cystourethrography combined with distal colo-
nography via a mucous fistula has traditionally been used to
demonstrate the fistulous connections in patients with ano-
rectal malformation. Contrast-enhanced colosonography
(ceCS) is a new technique that involves the instillation of
ultrasound contrast material into the mucous fistula fol-
lowed by scanning from an anterior sagittal, transperineal,
and posterior sagittal approach. This technique has been
used to outline the distal colon and identify the presence
and location of fistulas without the use of ionizing radiation
[220]. Similarly, voiding urosonography performed after
installation of ultrasound contrast material into the bladder
can also outline rectourethral fistulous tracts as the patient
voids (Fig. 10.57).
10 The Gastrointestinal Tract
U pneumatosis than plain radiography [222].
R
B
Fig. 10.57 Imperforate anus with rectourethral fistula in a 2-month-old
male. Transperineal longitudinal image from a contrast-enhanced void-
ing urosonogram (ceVUS) shows the bladder (B) and urethra (U). A
fistulous tract (arrow) is seen extending posteriorly from the prostatic
portion of the urethra to the rectum (R)
Colonic Wall Thickening
Necrotizing Enterocolitis
Necrotizing enterocolitis (NEC) is a form of intestinal inflam-
mation that is estimated to occur in 5–14% of infants weigh-
ing less than 1500 grams at birth, with a mortality rate ranging
from 25% to 40% [221]. The pathogenesis of NEC is multi-
factorial, with immaturity and a compromised intestinal epi-
thelial barrier leading to bacterial overgrowth, intestinal
inflammation, and systemic infection.
NEC can present with increasing gastric residuals, bowel
distention, and bloody diarrhea and progress to intestinal perfo-
ration, shock, and death. The distal ileum and proximal large
bowel are most commonly involved, although extensive involve-
ment of the entire colon can occur. Diagnosis is based on clini-
cal findings, laboratory values, and imaging features. NEC can
progress from a medically managed disorder to surgical NEC
with the need for abdominal drainage or bowel resection.
Imaging findings on plain radiography include bowel
distention, pneumatosis, portal venous gas, and pneumo-
peritoneum when there is perforation of necrotic bowel.
Periodic supine and lateral decubitus films are performed
337
for patients on a “NEC watch.” Bowel ultrasound has
become a useful adjunct to plain radiography for the diag-
nosis of NEC and its complications.
Ultrasound can be used to evaluate bowel wall thickness
and perfusion and detect abdominal fluid collections. It can
also detect pneumatosis, portal venous gas, and free air
(Fig. 10.58). Pneumatosis appears as echogenic foci within
the bowel wall that do not change with compression, while
intraluminal gas is mobile and changes with bowel com-
pression. Ultrasound is more sensitive for the detection of
Bowel wall thickness, echogenicity, and perfusion are
initially increased due to inflammation [223]. As the dis-
ease progresses, the bowel wall becomes thinned with
decreased-to-absent perfusion and loss of peristalsis. Bowel
wall thinning is defined as a thickness of less than 1 mm
[224]. Contrast-enhanced ultrasound of the bowel has been
shown to be more sensitive than color Doppler imaging in
the detection of blood flow to the bowel [225].
Treatment of NEC involves bowel rest, replacement of
fluids, and intravenous antibiotics. Surgical intervention is
required in the event of bowel perforation or necrotic bowel.
Secretory immunoglobulin (Ig)A acquired via the ingestion
of human milk helps to provide protection against NEC.
Ulcerative Colitis
Ulcerative colitis (UC) is a chronic, relapsing inflammatory
condition of the colon that can present with bloody diarrhea,
tenesmus, crampy abdominal pain, and weight loss. The
incidence of pediatric-onset UC is from 1 to 4 per 100,000 in
the United States and Europe [226]. The course and extent of
pediatric-onset disease tend to be more severe, with a
30–40% colectomy rate at 10 years. Affected children also
suffer from delayed puberty and skeletal maturation. Studies
have shown that 60–80% of pediatric patients present with
pancolitis, compared with only 20–30% in adults [227].
While UC has historically been perceived as a superficial
inflammatory process that uniformly involves the rectum and
progressed contiguously toward the proximal colon, more
recent data have shown rectal sparing in 5% of children at
diagnosis [227]. Gastritis is commonly seen in association
with UC and erosions may be present [228].
No special bowel preparation is required for ultrasound
assessment. Bowel loops are evaluated for diminished peri-
stalsis, hyperemia, wall thickening, strictures, and adjacent
mesenteric inflammatory changes (Fig. 10.59). Conventional
transabdominal ultrasound provides limited assessment of
the sigmoid colon and rectum. Barium studies can be used to
assess for stricture formation.
338 M. M. Munden and H. J. Paltiel

a b

Fig. 10.58 Necrotizing enterocolitis in a 1-week-old premature infant. day later shows an echogenic ring of pneumatosis (arrow) and extensive
(a) Transverse midline abdominal power Doppler ultrasound image bowel wall hyperemia. (c) Sagittal grayscale ultrasound image of the
reveals thinning (arrow) of the bowel wall with surrounding hyperemia. right upper quadrant demonstrates a small echogenic focus of air (arrow)
(b) Sagittal power Doppler ultrasound image of the subhepatic region one along the upper liver edge consistent with early bowel perforation

a b

Fig. 10.59 Ulcerative colitis in a 22-year-old male complaining of the wall of the descending colon with loss of normal haustral folds. (b)
left-sided abdominal pain. (a) Longitudinal grayscale ultrasound image Longitudinal color Doppler ultrasound image reveals mural hyperemia
of the left lower abdominal quadrant shows thickening (arrowheads) of (arrowheads)

Treatment goals in children with UC depend on disease disease but not for long-term administration. In refractory
severity. Aminosalicylates are used to induce remission in cases, infliximab and oral tacrolimus can be used. Maintenance
patients with mild-to-moderate disease. Oral steroids are rec- therapy is continued indefinitely. Colectomy is considered
ommended for inducing remission in patients with moderate when patients are unresponsive to medical therapy [226].
10 The Gastrointestinal Tract
Crohn Disease
Approximately 60% of patients with Crohn disease have
colonic involvement. Approximately a half of these patients
have synchronous involvement of the small intestine; the rest
have disease limited to the colon. Colonic Crohn disease can
be segmental or involve the entire colon. As with Crohn
­disease elsewhere in the GI tract, it is often associated with
normal skip areas. Fistulas involving the colon are usually
secondary to small intestinal disease although primary
colonic fistulas also occur. Focal colonic perforation is com-
mon and can lead to an abscess or fistula [229]. Abdominal
or pelvic abscess can present with a wide range of clinical
findings, from low-grade fever and mild abdominal pain to
sepsis and peritonitis.
As previously described for ulcerative colitis, ultrasound
evaluation of the colon in patients with Crohn disease can
detect altered peristalsis, hyperemia, wall thickening, stric-
tures, adjacent mesenteric inflammatory changes, as well as
a b
c d
Fig. 10.60 Crohn disease in an 18-year-old male. (a) Transverse grayscale
ultrasound image of the right lower quadrant of the abdomen reveals thick-
ening of the wall of the terminal ileum and cecum (arrow) with luminal
narrowing. There is loss of definition of the normal bowel wall layers. (b)
Transverse power Doppler ultrasound image shows mural hyperemia. (c)
339
ascites and abscesses. Transperineal imaging provides infor-
mation regarding perirectal disease [230].
CT enterography is favored over MR enterography in an
acute setting using neutral oral contrast to visualize mural
enhancement, and is preferred for its ability to detect pneuma-
tosis and free air. MR enterography is the modality of choice
for assessment of perianal and perirectal complications of
Crohn disease and its ability to detect active disease [231].
Contrast-enhanced ultrasound can be performed to assess
colonic wall vascularity at the microcirculatory level. Prominent
mural enhancement is associated with active disease, an
important distinction from fibrosis, since active disease is
managed medically and fibrotic strictures typically require
surgical treatment. Time-intensity curves can be used to
compare disease activity over time and inform patient man-
agement decisions (Fig. 10.60) [232].
Shear wave elastography can help to distinguish between
fibrotic and non-fibrotic zones of colonic narrowing [233].
Transverse CEUS image demonstrates marked transmural enhancement of
the terminal ileum and cecum. (d) Time–intensity curves generated from
regions of interest placed over the wall of the abnormal bowel segments.
These curves can be used to more objectively assess treatment response
when compared over time
340 M. M. Munden and H. J. Paltiel

Treatment depends on disease location and severity. Medical
therapies include immunosuppressant and anti-tumor necro-
sis factor drugs. Monoclonal antibodies have been recently
been approved for clinical use [234].
Infectious Colitis
Infectious colitis is a common cause of acute abdominal pain
in children and is characterized by the passage of three or more
loose stools per day. In developing countries, diarrheal disease
can be devastating in children under 5 years of age. Most cases
of infectious gastroenteritis are viral in etiology with rotavirus,
norovirus, adenovirus, and astroviruses the common pathogens
(Fig. 10.61) [146]. Rotavirus is the leading cause of infantile diar-
rheal illness worldwide [235]. Gram-negative bacteria includ-
ing Shigella, Salmonella, Campylobacter, Escherichia coli, and
Yersinia also frequently cause enteritis.
The primary mode of transmission of bacterial enteritis is
via the fecal–oral route, through ingestion of contaminated
food and water as well as direct human-to-human contact.
Bacterial colitis can be noninflammatory or inflammatory.
Noninflammatory bacterial pathogens such as E. coli and
Staphylococcus lead to watery diarrhea without an associated
febrile illness. Enterohemorrhagic E. coli can cause hemor-
rhagic colitis after ingestion of contaminated foods. Yersinia,
Campylobacter, Shigella, Chlamydia, and Salmonella infec-
tions may have bloody and mucopurulent diarrhea associated
with fever and severe abdominal pain [147].
Yersinia and Salmonella usually infect the ascending colon
(Fig. 10.62) while Shigella infects the descending colon
(Fig. 10.63). Salmonella infection frequently has associated
adenopathy and splenomegaly. Cytomegalovirus, pseudomem-
branous colitis, and E. coli usually result in pancolitis [150].
Ultrasound is useful in identifying bowel wall thickening,
hyperemia, and free fluid. When the cecum and ascending
colon demonstrate thickened, echogenic walls, Campylobacter
infection should be considered. Wall thickening with promi-
nent ileocolic nodes caused by Yersinia infection can mimic
Crohn disease [236].

a b

Fig. 10.61 Adenoviral enteritis in a 6-year-old male. Sagittal grayscale ultrasound images of (a) the ascending colon (arrow) and (b) the rectum
(arrow) show diffuse wall thickening

a b

Fig. 10.62 Salmonella enteritis in a 3-year-old female. (a) Sagittal color Doppler ultrasound image of the affected ascending colon reveals
grayscale ultrasound image of the ascending colon shows diffuse wall hyperemia (arrow) of the bowel wall
thickening (calipers) with loss of haustral folds (arrow). (b) Sagittal
10 The Gastrointestinal Tract
Viral gastroenteritis is usually self-limited, and treatment
is symptomatic and supportive. Patients with severe bacterial
enteritis are treated with antibiotics.
S
Fig. 10.63 Shigella enteritis in a 2-year-old male with bloody diar-
rhea. Transverse grayscale ultrasound image of the splenic flexure
reveals circumferential bowel wall thickening (arrow). S, Spleen
Fig. 10.64 Clostridium difficile colitis in a 12-year-old male who
completed a recent course of antibiotics. Sagittal grayscale ultrasound
images show diffuse wall thickening of (a) the ascending colon and (b)
341
Pseudomembranous Colitis
Pseudomembranous colitis is usually precipitated by a
course of antibiotics that interferes with the normal colonic
bacterial flora and results in colonization by Clostridium dif-
ficile. Yellow-white nodules or plaques form pseudomem-
branes on the mucosal surface of the colon. Clindamycin,
penicillin, fluoroquinolones, and cephalosporins are typi-
cally associated with pseudomembranous colitis, although
any antibiotic can potentially be implicated. The incidence of
C. difficile infections has been increasing steadily over the
past two decades [237].
Infection is mediated by the production of two exotoxins,
toxin A and toxin B. Patients commonly present with abdom-
inal pain, profuse, watery and foul-smelling diarrhea, fever,
and leukocytosis about 3–9 days after initiation of antibiotic
treatment. Approximately 3–8% will develop fulminant
infection with severe ileus, toxic megacolon, colonic perfo-
ration, and/or septic shock [238].
Ultrasound imaging shows diffuse and marked wall thick-
ening of the entire colon and ascites (Fig. 10.64). Plain films
of the abdomen may show a colonic ileus, thumb-printing,
ascites, and a toxic megacolon (greater than 5.6 cm diame-
the descending colon (arrows), respectively. (c) Axial contrast-enhanced
CT image reveals a “double halo” sign (arrow) of submucosal edema
342
ter) in severe cases. CT, if needed, often shows diffuse
colonic wall thickening and nodularity, bowel wall stranding
with edema, ascites, the “accordion” sign when oral contrast
is trapped between thickened haustral folds or the “double
halo” sign of submucosal edema with 2 or 3 concentric rings
in the large bowel. With the exception of Crohn’s disease, the
wall thickening that occurs with pseudomembranous colitis
is greater than with any other infectious or inflammatory pro-
cesses [239, 240].
Treatment consists of administration of metronidazole or
vancomycin with most patients responding within 3–4 days.
Neutropenic Colitis
Neutropenic colitis, or typhlitis, is a life-threatening trans-
mural inflammation of the cecum, terminal ileum, and ascend-
ing colon that affects approximately 5% of children who
Fig. 10.65 12-year-old male with acute lymphocytic leukemia and
neutropenic colitis. Sagittal (a) and transverse (b) ultrasound images
with grayscale (left panels) and color Doppler (right p­ anels) show
M. M. Munden and H. J. Paltiel
receive chemotherapy, with a mortality rate of about 5%. It
occurs primarily in neutropenic patients, most often children
with leukemia. Patients with Burkitt lymphoma may also
have an increased risk of neutropenic colitis [241].
Cytotoxic chemotherapy causes a breakdown of the gut
mucosa leading to mucosal ulceration and necrosis, and
bowel wall edema [242]. More extensive involvement of the
colon, including the rectum, has been reported [243]. Patients
usually present with abdominal pain, fever, and neutropenia.
Diarrhea and vomiting are also common.
Imaging plays an important role in the diagnosis of neu-
tropenic colitis. Ultrasound is often sufficient for diagnosis,
showing a bowel wall thickness of 3 mm or greater with cir-
cumferential thickening of the cecum that extends into the
terminal ileum and ascending colon (Fig. 10.65). Pneumatosis
can affect the entire colon, and there are surrounding inflam-
diffuse wall thickening (arrows) and hyperemia (arrowheads) of the
ascending colon
10 The Gastrointestinal Tract
matory changes and ascites. Infectious etiologies that need to
be distinguished from neutropenic colitis include pseudo-
membranous colitis, GVHD, and cytomegaloviral colitis. C.
difficile colitis typically affects the entire colon, while GVHD
involves both small and large bowel with prominent mucosal
enhancement on CT.
While ultrasound is usually sufficient for diagnosis of
neutropenic colitis, CT is useful for detection of complica-
tions such as perforation and abscess, showing circumferen-
tial wall thickening with pericecal fat stranding and
pneumatosis [239, 242, 244].
Medical management with antibiotics, granulocyte col-
ony-stimulating factor, and total parenteral nutrition has led
to improved outcomes and decreased mortality rates.
Cystic Fibrosis
Cystic fibrosis transmembrane regulator (CFTR) dysfunc-
tion in the GI tract is present in all CF patients, regardless
of genotype. The CFTR protein controls the secretion of
chloride, bicarbonate, and fluid into the colon. The most
serious intestinal complication of CF is obstruction.
Patients may develop rectal prolapse and ileocolic intus-
susception due to enlarged lymphoid follicles or thick-
ened stool. There is an increased incidence of Crohn
disease and fibrosing colonopathy in patients with
CF. Fibrosing colonopathy is an iatrogenic complication
that tends to develop in patients under the age of 10 years
who are receiving high-dose pancreatic enzyme replace-
ment [183].
Ultrasound will show colonic dilation, wall thickening,
and hyperemia. Ileocolonic intussusception and ascending
colon strictures can also be identified [245]. Radiologic fea-
tures of fibrosing colonopathy include nodular thickening,
loss of haustral folds, and stricture formation predominantly
involving the ascending colon. Pericolonic and mesenteric
fat proliferation are shown on CT, as well as signs of perfora-
tion or bowel ischemia.
343
Water-soluble enemas are used to relieve severely impacted
stool. Lubiprostone, a calcium chloride channel activator, is
used for treatment of chronic constipation. Ivacaftor, a trans-
membrane conductance regulator–potentiator, has been
shown to improve the gut microbial flora and to reduce intes-
tinal inflammation [246].
Hemolytic–Uremic Syndrome
Hemolytic–uremic syndrome (HUS) is a disorder character-
ized by low red blood cells, acute kidney failure, and low
platelets. Children typically develop diarrhea, stomach
cramping, and fever 3–4 days after eating food contaminated
with bacteria that produce the Shiga toxin that then incites an
autoimmune response. E. coli-type O157:H7 is the respon-
sible organism in about 80% of cases. Other organisms
including Streptococcus pneumoniae, Shigella, and Salmonella
and certain medications can also cause HUS.
Patients initially present with bloody diarrhea, fever,
vomiting, and weakness. Renal dysfunction and low platelets
develop as the diarrhea is improving. Atypical HUS is often
due to a genetic mutation and presents differently. Both
forms of HUS can lead to widespread inflammation and
thrombotic microangiopathy.
HUS typically occurs in children under the age of 5 years
and is a leading cause of acute kidney failure in young chil-
dren, affecting about 1.5 per 100,000 people per year [247,
248]. Hemorrhagic colitis is the most common GI manifesta-
tion of HUS. Ischemia leading to bowel perforation can
occasionally occur.
Ultrasound will show concentric wall thickening that most
often involves the cecum and ascending colon, as well as asci-
tes (Fig. 10.66). Renal involvement manifests as enlarged,
echogenic kidneys. CT will show wall thickening, luminal
narrowing, and pericolonic fat stranding [239].
Treatment is supportive, and may include corticosteroids,
dialysis, and plasmapheresis. About 70–85% of patients will
have a full recovery.
344
Fig. 10.66 6-year-old female with hemolytic–uremic syndrome. (a)
Transverse grayscale ultrasound image shows a thick-walled colonic
loop in the right lower quadrant of the abdomen. (b) Transverse
color Doppler ultrasound image reveals hyperemia of the colonic
Benign Masses
Juvenile Polyp
Juvenile polyps are hamartomatous lesions and are the most
common benign colonic tumors in childhood, with an inci-
dence of approximately 1%. They occur most often in the
first 10 years of life, with a peak incidence between 2 and
5 years of age. There is a male-to-female ratio of 1.4:1
Solitary juvenile polyps carry no malignant potential.
However, adolescents and adults with multiple juvenile pol-
yps do carry a significant risk of malignancy [249]. Patients
with 2–5 polyps fulfill the criteria for multiple juvenile pol-
yps while those with more than 5 polyps meet the definition
M. M. Munden and H. J. Paltiel
wall. (c) Sagittal grayscale ultrasound image of the right kidney (K)
demonstrates increased cortical echogenicity compared to the adja-
cent liver (L)
of juvenile polyposis syndrome (JPS). The number of juve-
nile polyps is important since more than five polyps increase
the risk for colorectal cancer, which is discussed below.
A challenge occurs when managing a patient with three or
four juvenile polyps because it is unclear whether the patient
will go on to develop juvenile polyposis syndrome and there-
fore be at significant risk for intestinal cancer. Juvenile pol-
yposis syndrome is an autosomal dominant condition in which
colonic polyps continue to increase in number during adoles-
cence and adulthood. Extra-colonic polyps can also be found
throughout the GI tract.
The cumulative lifetime risk for colorectal cancer in
patients with juvenile polyposis syndrome is 39%. Solitary
10 The Gastrointestinal Tract
Fig. 10.67 Juvenile polyp in a 3-year-old male with painless rectal bleed-
ing. Sagittal grayscale ultrasound image of the left lower quadrant shows
a round, heterogeneous, solid lesion (calipers) in the descending colon
juvenile polyps are typically left-sided, measuring from 1
to 3 cm in size and are usually pedunculated. These polyps
usually present with painless rectal bleeding although some
may manifest as a prolapsing mass or with mucopurulent
stools [250].
Colonoscopy is the modality of choice for the detection of
juvenile polyps [251, 252]. Ultrasound has been shown to
have a low sensitivity of 47% but a high specificity of 100%
in the detection of colonic polyps in children who do not
undergo bowel preparation prior to examination [251].
Polyps appear as intraluminal, vascular, hypoechoic lesions
containing internal cystic areas (Fig.10.67). Colonic filling
defects are seen on contrast enema.
Solitary polyps are removed by polypectomy. Suggested
surveillance of patients with juvenile polyposis syndrome
includes colonoscopy every 3 years from the time of symptom
occurrence or in the early teenage years if symptoms have not
occurred in the setting of a family history; and upper endos-
copy every 2 years beginning at 15 years of age [249].
Duplication Cyst
Approximately 6.8% of all duplication cysts occur in the
colon with about 30% containing ectopic gastric mucosa
[253]. Colonic duplication cysts may be asymptomatic or
can present with obstruction, abdominal pain, and bleeding.
As previously described for duplication cysts occurring
elsewhere in the GI tract, ultrasound can demonstrate the
characteristic “gut signature” of these lesions with the hyper-
echoic inner mucosal layer surrounded by a hypoechoic
layer of smooth muscle. Internal contents can be simple and
anechoic or complex with internal contents from bleeding
345
and/or inflammation [70, 73]. Treatment of a colonic dupli-
cation cyst consists of surgical removal.
Malignant Tumors
Lymphoma
A recent review of NHL of the GI tract noted that approxi-
mately 29% of tumors involved the colon. Burkitt lymphoma
was the most common subtype, followed by diffuse large
B-cell lymphoma. Colonic tumors had the best survival rate
[254]. Clinical presentation varies, including abdominal pain,
weight loss, diarrhea, and intussusception [255]. The non-
specific presentation may lead to delays in diagnosis.
Bowel wall thickening, bulky adenopathy, and hepato-
splenomegaly can be seen with ultrasound and CT (Fig. 10.68)
[256]. The ileocecal region is commonly affected by B-cell
lymphoma. Well-defined tumor margins with preserved fat
planes are typically seen with CT.
Colonic lymphoma is treated with chemotherapy. Surgery
is performed for palliation of pain and treatment of obstruc-
tion, perforation, and bleeding [256].
Adenocarcinoma
Colorectal adenocarcinoma in children is extremely rare,
representing about 1% of all pediatric neoplasms. The annual
incidence in children is about one in ten million in patients
less than 20 years of age, with a peak incidence at 15 years.
Despite its rarity, it is still the most common primary solid
malignancy of the GI tract in children [257, 258]. Predisposing
conditions are identified in 10–30% of patients with colorec-
tal adenocarcinoma, including familial adenomatous polypo-
sis, UC, Crohn disease, and Peutz–Jeghers syndrome. In a
large case series of colorectal carcinoma in children, 22%
had colonic polyps and 8 of 77 had multiple polyps [259].
A high microsatellite instability is one of the most impor-
tant genetic mutations associated with childhood colorectal
carcinoma. Tumors are evenly distributed between the right
and left sides of the colon with most diagnosed pathologi-
cally as mucinous adenocarcinoma. Presenting symptoms
are nonspecific, and include abdominal pain and vomiting,
weight loss, altered bowel habits, hematochezia, and anemia.
Diagnosis is therefore often delayed in children due to a low
initial clinical suspicion for colorectal carcinoma. Duration
of symptoms prior to diagnosis can range from 2 to 6 months,
so that children are more likely to have advanced disease at
diagnosis, which may contribute to the overall poor out-
comes associated with this tumor [260].
Ultrasound imaging can show concentric, hypoechoic
colonic wall thickening with a target or “pseudokidney”
appearance consisting of central echogenic mucosa sur-
rounded by thickened bowel wall. Imaging findings are
346 M. M. Munden and H. J. Paltiel

a b

c d

Fig. 10.68 Burkitt lymphoma in a 5-year-old male complaining of quadrant shows similar mass-like thickening of the colon (arrow). (c)
abdominal pain for a month. (a) Sagittal grayscale ultrasound image of Transverse color Doppler ultrasound image of the right lower quadrant
the right upper quadrant shows mass-like thickening (arrows) of the mass reveals internal perfusion. (d) Coronal contrast-enhanced CT
colon. (b) Transverse grayscale ultrasound image of the right lower image depicts the large right colonic mass (arrow)
10 The Gastrointestinal Tract
Fig. 10.69 Adenocarcinoma of the colon in a 15-year-old male. Supine
radiograph of the abdomen reveals psammomatous calcifications (arrow)
in the distal colon associated with mucinous adenocarcinoma
similar to those in adults with case reports of plain film
and contrast studies documenting lower GI obstruction.
Plain films may reveal a paralytic ileus with large soft tis-
sue masses containing calcifications in advanced cases
(Fig. 10.69). Barium studies often reveal an “apple core”
lesion. CT will show a colonic mass with segmental, cir-
cumferential wall thickening. Liver and peritoneal masses
are readily depicted. PET/CT is often used to depict the
overall extent of disease.
Treatment involves surgical removal of the cancer. There
is some controversy as to the role and type of chemotherapy
to use in children with colonic adenocarcinoma. Monoclonal
antibody target therapy has shown promising results for met-
astatic disease. As colon cancer is so rare in children, sur-
vival rates are uncertain. The stage at diagnosis is one of the
most important factors in determining mortality rate [249].
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 Liver
11
Jeannie K. Kwon, Maddy Artunduaga, Javier D. Gonzalez,
Alexandra M. Foust, Elisabeth P. Moredock,
Süreyya Burcu Görkem, and Harriet J. Paltiel

Abbreviations FDG Fluorodeoxyglucose

FLHCC Fibrolamellar variant of HCC
2D SWE Two-dimensional shear-wave elastography FNH Focal nodular hyperplasia
ADPKD Autosomal dominant polycystic kidney disease GLUT-1 Glucose transporter protein-1
AFP Alpha-fetoprotein HBV Hepatitis B virus
AML Acute myeloid leukemia HCC Hepatocellular carcinoma
ARFI Acoustic radiation force impulse HCV Hepatitis C virus
BCS Budd–Chiari syndrome HIDA Hepatobiliary iminodiacetic acid
CEUS Contrast-enhanced ultrasound HIV Human immunodeficiency virus
CH Congenital hemangioma HMH Hepatic mesenchymal hamartoma
CMV Cytomegalovirus HSCT Hematopoietic stem cell transplantation
CT Computed tomography HSV Herpes simplex virus
CVP Central venous pressure HU Hounsfield unit
DWI Diffusion-weighted imaging IHH Infantile hepatic hemangioma
EBV Epstein–Barr virus IVC Inferior vena cava
e-FAST Extended focused assessment with sonography IQR Interquartile range
in trauma MRCP MR cholangiopancreatography
EHE Epithelioid hemangioendothelioma MRE MR elastography
ERCP Endoscopic retrograde MR Magnetic resonance
cholangiopancreatography NAFLD Nonalcoholic fatty liver disease
FALD Fontan-associated liver disease NASH Nonalcoholic steatohepatitis
NHL Non-Hodgkin lymphoma
J. K. Kwon (*) · E. P. Moredock NICH Noninvoluting congenital hemangioma
Department of Radiology, Children’s Medical Center Dallas, PET Positron emission tomography
University of Texas Southwestern Medical Center, Dallas, TX, USA PHL Primary hepatic lymphoma
e-mail: jeannie.kwon@utsouthwestern.edu
PICH Partially involuting congenital hemangioma
M. Artunduaga PLD Polycystic liver disease
Department of Radiology, Pediatric Radiology Division,
PRETEXT Pretreatment extent of tumor
University of Texas Southwestern Medical Center, Children’s
Health Medical Center, Dallas, TX, USA pSWE Point shear-wave elastography
PTLD Posttransplant lymphoproliferative disorder
J. D. Gonzalez
Medical Center Radiology Group, Orlando Health Arnold Palmer RI Resistive index
Hospital for Children, Orlando, FL, USA RICH Rapidly involuting congenital hemangioma
A. M. Foust ROI Region of interest
Department of Radiology, Boston Children’s Hospital and Harvard SHL Secondary hepatic lymphoma
Medical School, Boston, MA, USA SOS Sinusoidal obstruction syndrome
S. B. Görkem TACE Transarterial chemoembolization
Division of Pediatric Radiology, Department of Radiology, TE Transient elastography
Erciyes University School of Medicine, Kayseri, Turkey
TE Echo time
H. J. Paltiel TIPS Transjugular intrahepatic portosystemic shunt
Division of Ultrasound, Department of Radiology, Boston Children’s
(abbreviations continue)
Hospital and Harvard Medical School, Boston, MA, USA

© Springer Nature Switzerland AG 2021 355

H. J. Paltiel, E. Y. Lee (eds.), Pediatric Ultrasound, https://doi.org/10.1007/978-3-030-56802-3_11
356 J. K. Kwon et al.

(abbreviations continued) the liver should be obtained in the transverse, sagittal, and
TPN Total parenteral nutrition coronal planes. Asking the patient to perform deep inspira-
UES Undifferentiated embryonal sarcoma tion with breath-hold causes the liver to move inferiorly
UVC Umbilical vein catheter and may avoid the acoustic shadowing from the lower right
VOD Veno-occlusive disease ribs and improve visualization of the hepatic dome. Liberal
use of color and pulsed Doppler imaging as well as con-
trast-enhanced ultrasound (CEUS) imaging can help distin-
guish blood vessels from bile ducts and characterize
Introduction vascular lesions.

A wide spectrum of disorders can affect the pediatric liver.

This chapter will focus on the role of ultrasound in the diag- Grayscale Imaging
nosis and management of pediatric hepatic disorders, includ-
ing a review of transducer selection and imaging techniques, Grayscale imaging is used to obtain a global assessment
liver development and anatomy, and an overview of the most of the hepatic parenchyma (Fig. 11.1, Table 11.1). For
clinically relevant anatomical variants, congenital anoma-
lies, as well as benign and malignant hepatic disorders
encountered in daily practice. Ultrasound imaging of liver
transplantation is also discussed.
L
Technique

Patient Positioning

Ultrasound examination of the liver is performed with

patients in a supine position. Left posterior oblique position- K
ing aids visualization of the right hepatic lobe, by allowing
easier placement of the transducer along the right lateral or
right posterior body wall. Having the patient raise the right
arm above their head results in opening of the lower intercos-
tal spaces, allowing for a wider sonographic window. Fig. 11.1 Normal liver in a 5-month-old male. Sagittal grayscale ultra-
sound image shows normal parenchymal echotexture of the liver (L)
and right kidney (K)

Ultrasound Transducer Selection

In infants and small children, the liver is ideally imaged Table 11.1 Causes of diffuse change in hepatic echotexture
with a linear or curved-array transducer with a frequency of Decreased Increased
5–9 MHz. In larger patients, a 2.5–5 MHz transducer may Infectious hepatitis (hepatitis Steatosis
be required to enable greater penetration and visualization B, hepatitis C)
of the deeper portions of the larger right hepatic lobe. High-­ Inflammatory hepatitis Drug-induced (steroids,
(non-alcoholic chemotherapeutic agents)
frequency linear transducers up to 12 MHz or more are use- steatohepatitis, drug-­
ful in smaller infants and for the evaluation of superficial induced, autoimmune)
structures, including the liver surface contour. Transducers Neoplastic infiltration Cirrhosis
with a smaller footprint permit intercostal scanning. (leukemia, lymphoma)
Miscellaneous (fasting state, Nutritional (total parental nutrition,
passive venous congestion, extreme malnutrition)
septic shock)
Imaging Approaches Metabolic (glycogen storage
disorder, α1-antitrypsin
Fasting for 3–4 hours prior to the ultrasound examination deficiency, Wilson disease,
hemochromatosis, cystic fibrosis,
for infants, and 4–6 hours for older children, minimizes tyrosinemia)
bowel gas and aids visualization of the liver [1]. Images of Chronic hepatitis
11 Liver
example, a generalized increase in echogenicity is most
commonly seen in the setting of hepatic steatosis. In con-
trast, a diffusely hypoechoic appearance of the liver with
relatively increased echogenicity of the portal triads, the
so-­called “starry sky” appearance, is a reflection of paren-
chymal edema (Fig. 11.2) [2]. Grayscale imaging also depicts
blood vessels, bile ducts, and cysts, which appear as tubu-
lar or round, anechoic structures coursing through the
liver.
Fig. 11.2 “Starry sky” appearance of the liver in a 7-year-old female in
septic shock. Transverse grayscale ultrasound image shows diffusely
hypoechoic hepatic parenchyma due to edema with the portal triads
appearing relatively more echogenic than normal
a b
Fig. 11.4 Normal spectral Doppler waveforms in a 1-month-old
female. (a) Oblique color Doppler ultrasound image of the liver
reveals a normal mildly pulsatile waveform in the main portal vein.
357
Doppler Ultrasound
Doppler ultrasound is most useful for the evaluation of blood
vessels or for blood flow detection within a cystic or solid struc-
ture (Fig. 11.3). Analysis of the spectral Doppler waveforms
provides information regarding flow velocity and direction
(Fig. 11.4). Flow indices such as the arterial resistive index (RI)
can be calculated based on peak systolic velocity, end diastolic
velocity, and mean velocity of blood flow. Color and pulsed
Doppler imaging are commonly performed to assess posttrans-
plant vascular structures or vascular masses; evaluate flow in the
portal vein; and differentiate blood vessels from bile ducts or
other fluid-filled structures (Fig. 11.5) [1]. Power Doppler can
be useful in detecting low-flow, especially in the setting of sus-
pected portal vein and hepatic vein thrombosis.
Fig. 11.3 Normal color Doppler appearance of the main portal vein in
a 19-month-old female. Transverse color Doppler ultrasound image of
the liver shows normal flow toward the liver (hepatopetal flow) encoded
in red (arrow). Flow away from the liver (hepatofugal) is encoded in blue
(b) Oblique color Doppler ultrasound image of the liver shows a nor-
mal low-resistance arterial waveform in the main hepatic artery with
a resistive index (RI) measurement of 0.60
358 J. K. Kwon et al.

microbubbles remain confined to the vascular space as blood

pool agents [3, 4]. CEUS is useful to evaluate focal liver
lesions, with the ability for real-time characterization of
enhancement patterns and a greater ability to detect blood flow
compared to Doppler imaging [3, 5–8]. In the arterial phase,
the extent and pattern of the vascular supply of a lesion can be
determined. Portal venous and delayed phase imaging is used
to assess the patterns of washout of contrast material, which
help to differentiate between benign and malignant lesions [9].
Patterns of enhancement can also be used to differentiate solid
lesions from complex cystic collections. One advantage of
CEUS over computed tomography (CT) is that it provides excel-
lent temporal resolution without the use of ionizing radiation.
Compared with magnetic resonance (MR) imaging, CEUS
examinations tend to be shorter in duration and can accommo-
date more patient motion, decreasing the need for sedation, an
important concern in children [7, 10, 11]. In addition, ultrasound
Fig. 11.5 Parenteral-nutrition-associated liver disease in a 19-month-­
old male. Transverse power Doppler ultrasound image of the liver uses contrast agents have a high safety profile, without nephrotoxicity
a color map to depict the amplitude of the Doppler signal in the right or hepatotoxicity [3, 12–15]. Repeat bolus administration of the
portal vein and its branches. Flow velocity and direction are not indi- contrast agent can also be performed as needed.
cated, but noise is diminished, thereby permitting higher gain settings Imaging is best performed with contrast-specific software,
and increased sensitivity for the detection of flow. Note the abnormally
increased echogenicity of the hepatic parenchyma which uses a low mechanical index to minimize microbubble
destruction within the imaging field of view. Typically, a dual-
Contrast-Enhanced Ultrasound display mode is used, with side-by-side grayscale imaging for
anatomic localization, and contrast-­mode with background
Contrast-enhanced ultrasound (CEUS) uses microbubbles that tissue suppression to display areas of enhancement (Fig. 11.6)
are smaller than the size of a red blood cell to assess the vas- [16]. Quantitative analysis with time–intensity curves can also
cularity of a tissue or organ. When injected intravenously, the be performed [17].

Fig. 11.6 Normal liver depicted by contrast-enhanced ultrasound head). (b) Transverse CEUS in the delayed phase (right panel) with
(CEUS) in a 12-day-old male. (a) Sagittal CEUS image of the liver in reference grayscale image (left panel) reveals that intravenous contrast
the arterial phase (right panel) with reference grayscale image (left has homogeneously distributed throughout the liver parenchyma
panel) shows intravenous contrast filling the hepatic arteries (arrow-
11 Liver 359

Elastography reported as median values in units of m/sec and/or kilopascals

(kPa) (Fig. 11.8), with quality assessment values reported as
Elastography is a relatively new ultrasound technique that interquartile range (IQR)/median (%) [18]. As in adults, good
noninvasively assesses the stiffness of tissue in response to correlation between ARFI and histological staging of liver
an applied stress, which is estimated by tracking and measur- fibrosis has been observed in pediatric patients [19–21]. In the
ing the speed of shear waves propagating through tissues. liver, elastography is useful for the assessment of fibrosis, with
Three main methods for liver assessment exist, which differ clinical applications continuing to expand, including the eval-
in the source of the applied stress, the size of the sampling uation of biliary atresia and Fontan-associated liver disease
area, and the output parameters reported (Table 11.2) [18]. [22–24].
One-dimensional transient elastography (TE) is a method
developed and used only for liver fibrosis assessment [19]. It
employs a mechanical impulse created by a piston-like probe
(FibroScan®, Echosens, Paris, France) that is applied at the
skin surface over a small region of interest (ROI). TE mea-
surements are obtained without imaging guidance.
Both point shear-wave elastography (pSWE) and two-
dimensional shear-wave elastography (2D SWE) are incorpo-
rated into many clinical ultrasound systems and use an acoustic
radiation force impulse (ARFI), rather than a mechanical
impulse, to generate shear waves. With these ARFI methods,
grayscale ultrasound imaging can be used to guide placement
of the ROI and avoid nonparenchymal structures such as ves-
sels and the gallbladder. Added benefits of 2D SWE are a
larger ROI and the generation of color elastogram maps that
Fig. 11.7 Hepatic cirrhosis in a 19-year-old female with cystic fibrosis.
correspond to degrees of tissue stiffness (Fig. 11.7). Transverse grayscale elastography ultrasound image shows diffusely
Up to 10 measurements are obtained in the same location echogenic liver parenchyma with elevated mean velocity measurement
of the liver (typically, segments VII or VIII), and results are consistent with cirrhosis

Table 11.2 Comparison of elastography methods

Transient elastography (TE) Shear-wave elastography (SWE) MR elastography (MRE)
Measurement region One-dimensional Point (pSWE) 4 axial selections
2-dimensional (2D SWE)
Region of interest size 4 cm3 1.0 cm3 (pSWE) or 250 cm3
Small region of interest 20 cm3 (2D SWE) Evaluates entire liver
Larger regions of interest
Relative cost $ $$ $$$
Impulse sources Mechanical impulse Acoustic radiation force impulse (ARFI) Mechanical impulse
Image guidance and No imaging assessment of Grayscale evaluation of hepatic Concurrent conventional MR imaging can
correlations liver parenchyma further assess for masses and cirrhosis
Patient-related Limited by ascites and body Limited by body habitus Possible need for sedation
limitations habitus More studies needed for validation Contraindications to MR imaging
MR, Magnetic resonance
360
Fig. 11.8 Normal liver stiffness values in a 17-year-old female. Oblique
transverse 2D shear-wave elastography ultrasound image of the right lobe
of the liver demonstrates side-by-side display of the measurement confi-
dence map (left) and the corresponding color stiffness map (right). Two
Normal Development and Anatomy
Normal Development
In the fourth week of life, the hepatic diverticulum emerges
as a ventral outgrowth of the caudal end of the foregut and
will eventually form the liver (Fig. 11.9). The hepatic diver-
ticulum extends into the septum transversum, which forms
part of the diaphragm and the ventral mesentery. The ventral
mesentery gives rise to the lesser omentum and the falciform
J. K. Kwon et al.
small, circular regions of interest are placed within the maps in the green
areas (left) indicating high confidence, with corresponding shades in the
cool end of the color spectrum (blue) showing normal stiffness levels
(right). Note the diffusely echogenic parenchyma consistent with fatty liver
ligament, which carries the umbilical vein. As cells of the
hepatic diverticulum proliferate, interlacing cords of hepatic
cells join to form hepatic sinusoids.
There are at least 20 discrete cell populations in the fully
developed liver, including hepatocytes, endothelial cells,
cholangiocytes, hepatic stellate cells, and Kupffer cells [25].
At 7 weeks, the ductus venosus emerges as a large shunt that
connects the umbilical vein to the inferior vena cava, allow-
ing blood to bypass the liver (Fig. 11.10).
11 Liver 361

Aorta
Hepatic
diverticulum
Duodenum

Heart Peritoneal cavity

Hepatic diverticulum
growing into the septum
Septum Level of transversum
transversum section B

Superior
mesenteric artery

Septum
a b transversum

Diaphragm Dorsal mesentery

Heart Lesser omentum Duodenum

Developing liver
Liver
Level of
Falciform section D
ligament
Dorsal aorta Visceral peritoneum
Free border
of ventral
mesentery
Parietal peritoneum

Inferior mesenteric artery Peritoneal cavity

Falciform ligament
c d

Fig. 11.9 Diagram of embryologic development of the liver. (a) section of a 5-week embryo. (d) Transverse section through the plane of
Sagittal section of a 4-week embryo. (b) Transverse section through the the dashed line in (c). The liver is joined to the ventral abdominal wall
plane of the dashed line in (a). The hepatic diverticulum extends into by the falciform ligament and to the stomach and duodenum by the
the septum transversum as the peritoneal cavity expands. (c) Sagittal lesser omentum

Hepatic portion of
inferior vena cava

Hepatic vein
(right vitelline) Hepatic vein
(left vitelline)
Ductus
venosus

Portal vein Left umbilical vein

Splenic vein

Superior
mesenteric vein

Fig. 11.10 Ventral view of the liver in a 7-week embryo. Hepatic sinusoids form the ductus venosus which carries blood from the umbilical vein
to the inferior vena cava
362
In fetal life, oxygen-rich blood is supplied by the placenta
through the umbilical vein. Blood in the umbilical vein drains
into the left portal vein and from there flows through the ductus
venosus to the hepatic vein confluence and into the inferior vena
a b
LP
LP
Fig. 11.11 Umbilical vein closure in a 1-day-old female. (a) Transverse
grayscale ultrasound image of the liver shows the left portal vein (LP)
with the umbilical vein (white arrow) anteriorly seen in cross-section as
a round echogenic structure. The ductus venosus (black arrow) is clos-
ing, and appears as a band-like echogenic focus extending posteriorly
from the left LP to the IVC (arrowhead). (b) Transverse color Doppler
J. K. Kwon et al.
cava (IVC). After birth, the umbilical vein and ductus venosus
close, and their remnants persist as the round ligament of the
liver and the ligamentum venosum, respectively (Fig. 11.11).
LP
ultrasound image of the liver shows flow within the LP and absence of
flow in the umbilical vein (white arrow). This appearance should not be
confused with thrombosis of the left portal vein. (c) Longitudinal color
Doppler ultrasound image of the liver shows the course of the umbilical
vein (white arrows) within the liver as it approaches the LP. Note that
there is flow within the LP, but not in the umbilical vein
11 Liver 363

Normal Anatomy

 egmental and Lobar Anatomy

S
The liver is divided into three lobes: left, right, and caudate.
Three vertically oriented planes containing the right, mid-
dle, and left hepatic veins, respectively, radiate from the IVC
to divide the liver into four sections.
The right and left lobes are separated by the main lobar PH
H
fissure, which contains the middle hepatic vein and runs
PV
through the gall bladder fossa to the IVC. The right interseg-
mental fissure contains the right hepatic vein and divides the CL
IVC
right lobe into anterior and posterior sections. The left inter-
segmental fissure contains the left hepatic vein and divides
the left lobe into medial and lateral sections.
The caudate lobe is located at the posterior aspect of
the liver, separated from the left hepatic lobe by the fis-
sure for the ligamentum venosum anteriorly, and bounded
by the IVC posteriorly (Figs. 11.12 and 11.13). The pap- Fig. 11.12 Normal ultrasound anatomy of the caudate lobe in a
illary process is the anteromedial extension of the cau- 12-year-old male. Longitudinal grayscale ultrasound image of the left
date lobe. hepatic lobe shows the caudate lobe (CL) located in the posterior aspect
of the liver and separated from the left hepatic lobe by the fissure for the
ligamentum venosum (black arrow) anteriorly, and bounded by the
inferior vena cava (IVC) posteriorly. The portal vein (PV), hepatic
artery (H), and pancreatic head (PH) are also seen

Anterior
Quadrate lobe
Gallbladder

Round ligament
Cystic duct of liver

Porta hepatis
Right lobe Hepatic artery proper
Common hepatic duct
Hepatic portal vein
Coronary
ligament

Inferior
vena cava
Left lobe

Bare area
Caudate lobe Ligamentum
venosum

Posterior

Fig. 11.13 Diagram of segmental and lobar anatomy of the liver, inferior view. The hepatic artery proper, common hepatic duct, and hepatic portal
vein course through the porta hepatis. The quadrate lobe lies anterior to the porta hepatis; the caudate lobe lies posterior
364
The Couinaud classification system of liver anatomy further
divides the right and left hepatic lobes into superior and inferior
segments by an imaginary horizontal plane containing the right
and left main portal branches [26]. Its usefulness lies in the fact
that each segment is a functional unit that contains its own dual
inflow blood supply (arterial and portal venous), lymphatic
drainage, and biliary drainage, and can be individually resected.
Segment I is the caudate lobe. Segments II to VIII are
numbered in a clockwise fashion beginning in the left lat-
eral superior liver. Segments II and III comprise the left
lateral section, superior and inferior to the portal plane,
respectively. Segment IV is the quadrate lobe and com-
prises the left medial section. It is divided into segments
IVa and IVb, located superior and inferior to the portal
plane, respectively. Segments V and VI comprise the right
hepatic lobe inferior to the portal plane, in the anterior and
posterior sections, respectively. Segments VII and VIII
comprise the right hepatic lobe superior to the portal plane,
in the anterior and posterior sections, respectively
(Figs. 11.14 and 11.15).
Right posterior Right anterior
section section
Right hepatic vein
VII
VIII
VI
V
Gall bladder
Cystic
duct
Fig. 11.14 Couinaud classification of liver anatomy. The liver is divided
into eight functionally independent segments based on a transverse plane
that passes through the bifurcation of the main portal vein, each with its
own vascular inflow, outflow, and biliary drainage. In the center of each
segment, there is a branch of the hepatic artery, portal vein, and bile duct.
J. K. Kwon et al.
Ligaments
The liver is covered by a thin layer of fibrous connective tis-
sue known as Glisson’s capsule. The majority of the liver is
covered by the visceral peritoneum and is associated with
several peritoneal ligaments that anchor the liver to the
abdominal wall (Fig. 11.16).
The ligaments can be seen by ultrasound as linear, echo-
genic structures that are more conspicuous when they con-
tain fibrofatty tissue or are surrounded by ascites. The
coronary ligament attaches the superior surface of the liver
to the inferior surface of the diaphragm via its anterior and
posterior folds. The bare area of the liver is the portion of the
liver surface located between the anterior and posterior folds
of the coronary ligament that is not covered by peritoneum.
Adjacent to the bare area, the right and left triangular liga-
ments are formed by the union of the anterior and posterior
folds of the coronary ligament and attach the liver to the dia-
phragm (Fig. 11.17).
Anteriorly, the right and left triangular ligaments con-
verge to form the falciform ligament, which attaches the
Left medial Left lateral
section section
Middle hepatic vein
Left hepatic vein
II
I
IV
III
Inferior
vena cava
Hepatic duct
Hepatic artery
Portal vein
Bile
duct
Along the edges of each segment, there is venous outflow through the
hepatic veins so that each hepatic vein drains two adjacent segments.
The division of the liver into independent units means that individual
segments can be resected without damaging the remaining segments
11 Liver 365

a b

III
IVa/b II/III IVb

M
VIII
V/VIII I
R I R
* *
VII VI/VII

Fig. 11.15 Ultrasound hepatic segmental anatomy in a 14-year-old male.
(a) Transverse grayscale ultrasound image of the liver at the level of the left
portal vein (arrow). The left hepatic lobe is divided by the left portal vein into
the superior segments (IVa and II) and the inferior segments (IVb and III).
The superior segments of the right hepatic lobe (VII and VIII) are divided by
the right hepatic (R) and middle hepatic (M) veins. The caudate lobe (I) is
located anterior to the IVC (asterisk) (b) Transverse grayscale ultrasound
image of the liver at the level of the right portal vein (arrow). The right
hepatic lobe is divided by the right portal vein into the superior segments
(VII and VIII) and the inferior segments (V and VI). The medial left hepatic
lobe segments (IVa and IVb) and the lateral left hepatic lobe segments are
divided by the left hepatic vein (not pictured). I, Caudate lobe; asterisk, IVC

Bare area
(diaphragmatic
surface of liver)
Coronary ligament

Left triangular ligament

Right triangular ligament

Fibrous
appendix
of liver

Left lobe,
diaphragmatic
surface
Right lobe, Falciform ligament
diaphragmatic
surface
Round ligament of liver
(obliterated
umbilical vein)

Inferior border
Gallbladder,
fundus

Fig. 11.16 Diagram of an anterior view of the liver with its ligamentous peritoneal attachments

liver to the anterior abdominal wall and carries the umbili-
cal vein to the liver during fetal development (Fig. 11.18).
The umbilical vein remnant forms the round ligament of
the liver and runs along the free edge of the falciform liga-
ment to the origin of the left portal vein. The ductus veno-
sus, which carries fetal blood from the umbilical vein to the
IVC, closes after birth to form the ligamentum venosum.
The hepatogastric and hepatoduodenal ligaments are peri-
toneal folds that attach the porta hepatis of the liver to the
stomach and proximal duodenum and form the lesser omen-
tum. The hepatoduodenal ligament contains the main portal
vein, the proper hepatic artery, and the common bile duct.
The hepatogastric ligament extends from the ligamentum
venosum and contains the left gastric artery and vein.
366
FF
L related to cardiac and respiratory activity. In the fasting state,
K giving off the gastroduodenal artery, the common hepatic
Fig. 11.17 Ultrasound demonstration of the right triangular ligament
in a 15-year-old female. Transverse grayscale ultrasound image of the
right hepatic lobe (L) shows the right triangular ligament (white arrow)
which is well depicted because of the adjacent perihepatic free fluid
(FF). K, Right kidney
Fig. 11.18 Ultrasound demonstration of the falciform ligament in an
18-year-old male. Transverse grayscale ultrasound image shows a lin-
ear echogenic band (arrowhead) extending to the liver capsule
Hepatic Circulation
The liver has a dual blood supply from the portal vein and the
hepatic artery. About 70% of the afferent blood flow into the
liver is from the portal venous system, unique vessels that
begin and end in a capillary system [27].
The portal vein supplies the liver with approximately half
of its oxygen requirements by bringing partially deoxygen-
J. K. Kwon et al.
ated, nutrient-rich blood from the spleen and intestines. The
main portal vein divides into right and left branches to supply
the right and left hepatic lobes, respectively. The right portal
vein subsequently divides into anterior and posterior branches.
The walls of the portal veins are echogenic by ultrasound,
making them easily distinguishable. On spectral Doppler
imaging, the normal portal vein demonstrates mild phasicity,
the normal peak flow velocity in the main portal vein should
vary between approximately 20–40 cm/s [28]. Slower veloc-
ities occur in the setting of portal hypertension, and faster
velocities are noted postprandially.
Classic hepatic arterial anatomy, in which the celiac axis
branches into the left gastric, splenic, and common hepatic
arteries, is seen in approximately 50% of the population. After
artery becomes the proper hepatic artery, which then divides
into the right and left hepatic arteries [27]. The proper hepatic
artery supplies approximately 50% of the liver’s oxygen
requirements. The hepatic artery branches accompany the por-
tal vein branches, and, along with bile duct branches, form the
portal triads, which are enclosed in a connective tissue sheath.
The normal hepatic artery shows pulsatile, low-resistance
flow, with resistive indices ranging between 0.55 and 0.70 [28].
Elevated resistance may be seen in the postprandial state; in
chronic hepatocellular disease, including cirrhosis; in hepatic
venous congestion; and in transplant rejection. Decreased resis-
tance may be seen in proximal arterial narrowing (such as trans-
plant anastomotic stenosis) or distal vascular shunts (such as
posttraumatic or iatrogenic fistulas) [29].
Blood flow leaves the liver through the right, middle, and
left hepatic veins (Fig. 11.19). The hepatic veins in turn drain
IVa
M L II
VIII
R
IVC
VII
Fig. 11.19 Ultrasound demonstration of normal hepatic veins in a 12-year-
old male. Transverse color Doppler ultrasound image of the liver demon-
strates the right (R), middle (M), and left (L) hepatic veins converging at
the inferior vena cava (IVC). Couinaud segments VII, VIII, IVa, and II are
separated by the hepatic veins in the superior portion of the liver
11 Liver
into the IVC. The right hepatic vein drains segments V–VIII. It
typically courses through the right intersegmental fissure and
separates the superior anterior and posterior segments of the
right hepatic lobe (Couinaud segments VIII and VII, respec-
tively). The middle hepatic vein drains segments IV, V, and
VIII. It courses through the main lobar fissure and separates
segments IVa and VIII, while the left hepatic vein, which drains
segments II and III, courses through the left intersegmental fis-
sure and separates segments II and IVa. The caudate lobe drains
separately into the IVC via multiple perforating veins.
The typical hepatic venous spectral Doppler waveform has
four phases with inflection points reflecting right atrial systole
(A wave), right ventricular systole (S wave), right ventricular
diastole (V wave), and right atrial diastole (D wave). The larger
S and D waves demonstrate flow toward the heart.
 ormal Hepatic Parenchyma
N the lateral segment of the left hepatic lobe extends far leftward
The basic structural units of the liver are the acini, which are
oriented in a radial configuration to form a lobule (Fig. 11.20).
The dual blood supply enters an acinus at the periphery of the
lobule via the hepatic arteriole and portal venule which con-
verge to allow blood to mix in the sinusoids. Blood then exits
the acinus at the central vein, which is at the center of the
lobule. Kupffer cells located along the sinusoids serve a filter-
ing function for blood. The space of Disse, which is located
adjacent to the sinusoidal endothelium and separates it from
the hepatocytes, contains stellate and other stromal cells.
Running in parallel alongside the sinusoids are the bile
canaliculi, which carry bile that flows in the opposite direc-
tion to the blood, toward the periphery of the lobule. The
hepatic artery proper, common hepatic duct, and hepatic por-
tal vein course through the porta hepatis (Fig. 11.21).
On ultrasound, the normal liver demonstrates homoge-
neous echogenicity with low-level echoes. Typically, the
Kupffer cell
Bile ductule
Portal venule
Hepatic arteriole Portal field
Fig. 11.20 Diagram of a liver acinus, the smallest functional unit of the liver. Blood flows from the portal venule through a hepatic sinusoid to
the central vein. Bile flows in the opposite direction within the bile ductule
367
walls of the portal veins are echogenic, which can help to
differentiate them from the hepatic veins (Fig. 11.22). In
children less than 6 months of age, the liver may be mildly
hypoechoic or isoechoic relative to the normal right renal
cortex. By 6 months of age, the liver is typically mini-
mally hyperechoic relative to the right kidney and mildly
hypoechoic relative to the spleen.
Anatomic Variants
Familiarity with anatomic variations in liver anatomy will
help to avoid confusion or misdiagnosis, and their recogni-
tion can be critical in treatment planning of diseased livers.
Reidel’s lobe is an inferior tongue-like projection of hepatic
parenchyma that can extend quite inferiorly along the right
paracolic gutter and be mistaken for hepatomegaly (Fig. 11.23).
“Sliver of liver” or “Beaver tail liver” refers to a variant where
and appears to wrap around the spleen. The papillary process of
the caudate lobe is an anteromedial extension of hepatic paren-
chyma that may mimic a mass near the head of the pancreas.
Typically, the right and left hepatic arteries arise from the
common hepatic artery at the porta hepatis. Some of the more
common variations in hepatic arterial anatomy include: (1) a
replaced or accessory left hepatic artery arising from the left
gastric artery; (2) a replaced right hepatic artery arising from
the superior mesenteric artery; and (3) a replaced common
hepatic artery arising from the superior mesenteric artery.
Standard portal venous anatomy consists of a main portal
vein branching into right and left portal veins, with subse-
quent division of the right portal vein into anterior and pos-
terior branches. Variations include trifurcation of the main
portal vein into right anterior, right posterior, and left portal
veins. Occasionally, the right posterior portal vein arises as
the first branch of the main portal vein.
Central
vein
Sinusoid
Stellate cell
Space of Disse
Endothelial cell
Hepatocyte
368
Portal vein atresia can occur when involution of the fetal
umbilical vein is excessive. The fetal umbilical vein nor-
mally drains into the left portal vein and spontaneously
involutes at birth. When involution is excessive, portal vein
stenosis or atresia can develop. When there is atresia of a
major branch of the portal vein, there can be associated
absence of the corresponding hepatic lobe [27]. The preva-
Fig. 11.21 Ultrasound demonstration of a normal porta hepatis in an
8-year-old female. Longitudinal oblique grayscale (left) and color
Doppler (right) ultrasound images of the porta hepatis through which
Fig. 11.22 Normal hepatic parenchyma in a 17-year-old male.
Transverse grayscale ultrasound image of the liver depicts a normal
liver contour and normal hepatic parenchymal echotexture. The right,
middle, and left hepatic veins are seen converging and draining into the
IVC (asterisk). Portal veins (white arrowhead) are identified by their
thin, echogenic walls which differentiates them from hepatic veins
(white arrow) which typically have imperceptible walls
J. K. Kwon et al.
lence of portal vein hypoplasia in the setting of biliary atre-
sia has been reported to be up to 26% [27]. Other congenital
anomalies are described in Section “Congenital Anomalies.”
Anatomic variations of the hepatic veins are common. For
example, there may be an accessory right inferior hepatic vein
that drains directly into the IVC, and an accessory right supe-
rior anterior vein that drains into the middle hepatic vein. The
course the hepatic artery proper (black arrows), common hepatic duct
(black arrowheads), and main portal vein (calipers, white arrows). The
hepatic duct is the most anteriorly located of the three structures
Fig. 11.23 Riedel lobe in an 18-year-old female. Sagittal grayscale
ultrasound image shows an elongated, tongue-like projection of the
right hepatic lobe (arrowheads) extending inferior to the lower pole of
the right kidney, a normal variant
11 Liver
middle and left hepatic veins often form a common trunk prior
to draining into the IVC. The caudate lobe veins are also vari-
able, with one or more veins draining separately into the IVC.
Congenital Anomalies
Liver Cyst
Congenital liver cysts are thought to represent hyperplastic
bile duct rests that progressively dilate due to failure of devel-
opment of normal connections with the biliary tree. They
consist of an outer layer of fibrous tissue and are lined with
biliary-type epithelium. As there is no connection to the bili-
ary tree, the contained fluid is clear and typically does not
contain bile. These cysts are most often identified as an inci-
dental finding on prenatal or postnatal imaging and are not
associated with any genetic syndrome or cysts in other organs.
The overall prevalence of congenital liver cysts has been
reported to be 2.5% [30], and they occur more often in girls
than boys. The increasing use of cross-sectional imaging has
shown that they are more common than originally believed.
Congenital liver cysts are usually asymptomatic, ranging in
size from a few mm to several cm.
Rarely, cysts may become large enough to produce symp-
toms, such as dull right-upper-­ quadrant pain, abdominal
bloating, or nausea. Complications are more common in
larger cysts and can include cholestasis due to bile duct com-
pression, spontaneous hemorrhage, infection, or torsion in
the case of exophytic cysts.
On ultrasound, these lesions appear as an anechoic, sim-
ple unilocular or multilocular cyst, with a thin or impercep-
tible wall and posterior acoustic enhancement (Fig. 11.24).
They may be multiple in number and vary in size, with larger
cysts measuring more than 4 cm in diameter. CT and MR
imaging demonstrate a smooth-walled, fluid attenuation or
intensity lesion without enhancement after intravenous con-
a b
Fig. 11.24 Congenital hepatic cyst in a 69-day-old male. (a) Transverse
grayscale ultrasound image shows a simple-appearing cystic lesion
(arrowhead) within the hepatic parenchyma adjacent to the gallbladder
369
trast administration. Rarely, these cysts can appear complex
due to hemorrhage or superinfection.
Most congenital hepatic cysts are incidentally detected and
will remain stable in size or undergo resolution without the
need for intervention. Larger, symptomatic cysts may be
excised or fenestrated via open or laparoscopic surgery [31].
Simple aspiration is not favored due to the risk of recurrence.
Polycystic Liver Disease
Polycystic liver disease (PLD) comprises a heterogeneous group
of autosomal dominant conditions that lead to defective biliary
cell growth. Defective proteins in primary cilia lead to abnormal
development of the ductal plate [32]. This results in variable, but
progressive development of cystic hamartomas (von Meyenburg
complexes) throughout the liver which are isolated and not part
of the biliary tree. Hepatic cysts increase in size and number with
increasing age and are rare in young children.
PLD is the most common extrarenal manifestation of
autosomal dominant polycystic kidney disease (ADPKD)
(Fig. 11.25) [33]. Rarely, milder forms of PLD occur in the
absence of ADPKD, although the pathologic appearance is
identical. Female patients have a greater severity of disease,
thought to be related to estrogen exposure [33, 34]. Most
patients are asymptomatic and maintain normal liver func-
tion, although large cysts and massive hepatomegaly may
cause mass effect leading to n­ ausea, dyspnea, severe abdom-
inal pain, abdominal distention, and early satiety [33].
Imaging findings include predominantly simple cysts
which may vary in size and number, depending on patient
age. Often, there are more than 20 liver cysts, with only a few
kidney cysts [32]. Over time, more than 50% of the liver
parenchyma will be replaced by cysts ranging from less than
10 mm to 80 mm in size [35].
Dominant cysts are typical. Peribiliary cysts may appear
as sub-centimeter cysts or tubular structures oriented along
(asterisk). (b) Color Doppler ultrasound image reveals the completely
avascular nature of the lesion
370 J. K. Kwon et al.

a b

Fig. 11.25 Autosomal dominant polycystic liver disease in a 21-year-­ consistent with a cyst. (b) Transverse grayscale ultrasound image of the
old male. (a) Transverse grayscale ultrasound image of the liver shows a right kidney shows multiple cortically based cysts with enhanced through
well-circumscribed, anechoic lesion (arrow) in the right lobe of the liver transmission (arrowheads)
that demonstrates enhanced through transmission of sound (arrowheads)

the portal vessels. Hemorrhagic or infected cysts can be het-
erogeneous in echotexture on ultrasound, with increased
attenuation on unenhanced CT, and increased T1-weighted
signal on MR imaging. Other findings, such as fluid-fluid lev-
els, cyst wall thickening, and calcification suggest infection.
Somatostatin analogues have been shown to reduce liver
volume in patients with PLD [36]. Dominant liver cysts can
be treated with aspiration and sclerotherapy, fenestration, or
segmental hepatic resection. Liver transplantation is indi-
cated in only a minority of patients [37].
Congenital Portosystemic Shunts
Congenital portosystemic shunts are rare vascular abnormal-
ities that occur as a result of abnormal development or invo-
lution of the fetal vasculature. Blood from the splanchnic
venous circulation is shunted to the systemic circulation,
bypassing the hepatic sinusoids in a variety of anatomic con-
figurations. The prevalence is about 1:30,000 children [27].
With intrahepatic shunts (including abnormal persistence of
a patent ductus venosus), abnormal connections occur between
branches of the portal vein and the hepatic veins or IVC
(Fig. 11.26). Extrahepatic shunts (also known as Abernethy
malformation) may demonstrate splanchnic venous return to
any systemic vein, with or without preservation of portal venous
perfusion to the liver [38].
Extrahepatic shunts associated with congenital absence of
the portal vein are more common in females and are often
associated with other congenital anomalies, including skele-
tal defects, congenital heart d­ isease, polysplenia, intestinal
malrotation, and genitourinary anomalies.
Clinical manifestations of congenital portosystemic shunts
may be related to the bypassing of splanchnic blood from the
liver and include hypergalactosemia, hyperammonemia, hepatic
encephalopathy, hypoxia due to hepatopulmonary syn-
drome, and pulmonary hypertension. Deprivation of portal
flow to the liver may result in elevated liver enzymes, liver
atrophy, and the development of hepatic lesions, including
regenerative nodules, focal nodular hyperplasia, adenoma,
hepatoblastoma and hepatocellular carcinoma [27].
Doppler ultrasound is the first-line imaging modality
for identifying congenital portosystemic shunts. Imaging
findings are variable, depending upon the location of the
portosystemic shunt, and can include anomalous porto-
systemic connections, absence of the main portal vein or
its branches, and compensatory enlargement of the hepatic
artery. The portal vein may have a biphasic or triphasic
waveform that reflects variations in right heart pressure
[1].
CT or MR imaging can be used to define shunt anatomy if it
is not completely delineated by ultrasound. MR imaging can
also evaluate associated focal liver lesions. Unlike acquired por-
tosystemic shunts, congenital shunts are not associated with
imaging features of portal hypertension (such as varices, ascites,
or splenomegaly), which can help to differentiate between the
two.
Many intrahepatic shunts will spontaneously close by 1
year of age without requiring definitive treatment. In older
patients, surgical shunt occlusion or transcatheter coil emboli-
zation may be effective in treating symptoms and preventing
complications. However, in patients with total absence of por-
tal venous supply, liver transplantation is the appropriate ther-
apeutic option.
11 Liver 371

a b

V
V

c d

Fig. 11.26 Congenital intrahepatic portosystemic shunt in a 6-year-­ trast-enhanced computed tomography (CT) images of the abdomen
old male. Sagittal grayscale (a) and color Doppler (b) ultrasound show the abnormal side-­to-­side connection (arrowheads) between the
images demonstrate an abnormal connection (arrows) between the main main portal vein/proximal left portal vein and the intrahepatic IVC
portal vein (asterisks) and the IVC (V). Axial (c) and sagittal (d) con-

Extreme malnutrition, exogenous steroids, and drugs such as

Diffuse Parenchymal Disease
Nonalcoholic Fatty Liver Disease
Nonalcoholic fatty liver disease (NAFLD) encompasses the
entire spectrum of fatty liver disease, defined by fat content
exceeding 5% of the liver weight in the absence of secondary
causes of hepatic fat accumulation. Nonalcoholic steatohepatitis
(NASH) refers to the form of NAFLD where inflammation and
hepatocyte injury are present. NASH can lead to fibrosis and
cirrhosis, which are also included in the spectrum of NAFLD.
NAFLD is the most common form of pediatric chronic liver
disease in the industrialized world, and is associated with vis-
ceral adiposity, male sex, higher insulin levels, and Hispanic
ethnicity [39]. It affects up to 10% of the general pediatric
population and 80% of obese or overweight children [40].
amiodarone and methotrexate are additional causes of fatty
liver. Most children are asymptomatic or have nonspecific
complaints such as fatigue, malaise, and vague abdominal pain.
Aspartate aminotransferase, alanine aminotransferase, and
ultrasound are used to screen for NAFLD in obese children.
Ultrasound findings of diffuse fatty liver (steatosis) include
increased echogenicity of the liver relative to the normal right kid-
ney, loss of echogenicity of the portal vein walls, attenuation of the
ultrasound beam, and poor visualization of the diaphragm through
the liver (Fig. 11.27). Diagnostic confidence increases with
increasing severity of the fatty liver. Hepatomegaly is common.
Areas of focal fatty sparing appear as hypoechoic foci in
comparison to the surrounding echogenic, fatty liver, and at
times may appear mass-like. Fatty sparing is often located
along the porta hepatis, falciform ligament, or gall bladder
fossa. On unenhanced CT, the fatty liver demonstrates hypoat-
372 J. K. Kwon et al.

a b

Fig. 11.27 Nonalcoholic fatty liver disease in a 17-year-old female. (a) Transverse and (b) sagittal grayscale ultrasound images show an enlarged,
diffusely echogenic liver

tenuation, and measures less than 40 Hounsfield units (HU),
or at least 10 HU less than the spleen.
Several methods for assessing hepatic steatosis by MR imag-
ing are also available. In-phase and opposed-phase MR imaging
(also referred to as dual echo, or chemical shift imaging) can
qualitatively and quantitatively estimate the fat fraction in the
liver, with higher fat content areas demonstrating greater signal
dropout on opposed-phase images. Multi-echo Dixon sequences
to calculate proton density fat fraction and MR spectroscopy are
two additional quantitative methods with a high degree of accu-
racy for evaluating fat content in the liver [41].
The mainstay of treatment of nonalcoholic fatty liver in
children is lifestyle modification, including diet, exercise,
and weight loss.
Liver biopsy, the traditional standard for assessing liver
fibrosis, has limitations due to its invasiveness, complica-
tions, subjectivity, and sampling variability, hence the
efforts to develop reproducible imaging-based techniques
[43].
Mild liver fibrosis often has a normal imaging appearance.
As fibrosis advances, some features will overlap with cirrho-
sis, the end stage of liver fibrosis. On ultrasound, the liver may
demonstrate a coarse echotexture with variable hypertrophy or
atrophy of hepatic segments (Figs. 11.28 and 11.29) [42].
Lace-like areas of hypoattenuation on CT and hyperintensity
on MR imaging may be present. Focal confluent fibrosis
may appear mass-like, and delayed hyperenhancement

Fibrosis

Hepatic fibrosis refers to the excess deposition of proteins,

including collagen fibers, in the extracellular matrix of the
liver, in response to all causes of chronic liver inflammation
and hepatocyte injury [42, 43]. This results in compression
of the portal venules and leads to portal hypertension. Early K
diagnosis and treatment of fibrosis may be reversible,
whereas progression of fibrosis leads to cirrhosis [44].
Underlying causes of fibrosis that present in the pediatric
and young adult population include biliary atresia, Fontan-­
associated liver disease (FALD), total parenteral nutrition
(TPN)-associated liver fibrosis, Alagille syndrome, ciliopa-
thies (including autosomal recessive polycystic kidney disease),
cystic fibrosis, and NAFLD. Patients are often asymptomatic,
Fig. 11.28 Hepatic fibrosis in a 17-year-old male with steatohepatitis.
with liver disease detected during the workup or monitoring of Sagittal grayscale ultrasound image of the liver shows a coarsened, het-
associated clinical entities [43]. erogeneous appearance of the parenchyma. K, Right kidney
11 Liver 373

on MR imaging helps to differentiate fibrosis from hepatocel-
lular carcinoma [42].
Fibrosis can be assessed by quantifying liver tissue stiff-
ness with ultrasound or MR elastography techniques [42].
Both smaller (pSWE) (Fig. 11.30) and larger region of
­interest samples (2D SWE) of liver tissue can be evaluated in
real-time. Imaging ultrasound elastography techniques have
the added benefit of concurrent anatomic and morphologic
assessment of the liver, with color maps demonstrating stiff-
ness values on 2D SWE (Fig. 11.31).
MR elastography uses an external device placed on the
patient’s right upper abdomen to generate shear waves in the
liver and allows for the entire liver to be assessed by the genera-
tion of colorized wave images and quantitative elastograms.
It should be noted that increased liver stiffness can be due to
factors other than fibrosis, such as a postprandial state and right
heart failure. In addition, patient breath-hold and operator scan-
ning techniques may produce increased stiffness levels. Direct
comparison of quantitative results of different methods has also
been problematic due to lack of standardization between equip-
ment manufacturers and techniques. A recent study that assessed

Fig. 11.29 Congenital hepatic fibrosis in a 20-month-old male with auto-
somal recessive polycystic kidney disease. Transverse grayscale ultra-
sound image shows an extremely heterogeneous and coarse appearance of
the hepatic parenchyma consistent with fibrosis. Hepatic biliary ductal
dilation (arrowheads) is also present, in keeping with Caroli disease
Fig. 11.30 Hepatic fibrosis in a 21-year-old female with Fontan-­
associated liver disease. Transverse ultrasound elastography image of
the liver shows heterogeneous parenchyma. Point shear-wave elastogra-
phy measurements are consistent with mild to moderate fibrosis

Fig. 11.31 Hepatic fibrosis in a 17-year-old female with Fontan-­ ness map (right). Two circular regions of interest placed within the
associated liver disease. Oblique transverse 2D shear-wave elastogra- maps in the green zones indicate high confidence (left), with elevated
phy ultrasound images of the right hepatic lobe demonstrates side-by-side stiffness levels (right). Note the coarse hepatic parenchyma
display of the confidence map (left) and the corresponding color stiff-
374
the repeatability and agreement of shear-wave speeds (SWSs)
across six state-of-the-art ultrasound 2D SWE systems showed
good to excellent intersystem agreement of measured SWS in
elastic phantoms and in vivo livers [43].
Given the wide spectrum of insults that cause hepatic
fibrosis, treatment is tailored to the underlying etiology.
Hemochromatosis
Hemochromatosis refers to iron overload with an ­abnormal
accumulation of iron in the body. Primary hemochromatosis
(also referred to as genetic or hereditary hemochro­matosis) is an
autosomal recessive disorder that leads to increased absorption
of intestinal iron with symptoms typically developing adult-
hood. Secondary hemochromatosis refers to all other causes of
iron overload, such as multiple red blood cell transfusions,
excessive dietary intake, or normal dietary intake with increased
absorption as can be seen with ineffective erythropoiesis accom-
panying certain liver diseases and inherited anemias.
Excess iron may accumulate in the liver, pancreas, heart,
skin, and synovium, leading to inflammation and fibrosis
caused by reactive oxygen species. Patients may be asymp-
tomatic or present with complaints of fatigue, skin hyperpig-
mentation, and arthralgias.
Fig. 11.32 Hemochromatosis in an 18-year-old female with Ewing
sarcoma. Transverse (a) and sagittal (b) grayscale ultrasound images of
the liver show mild, diffusely increased hepatic parenchymal echo-
J. K. Kwon et al.
Ultrasound findings are not specific to hemochromatosis.
Liver fibrosis and cirrhosis may be present, seen as increased
or coarse echogenicity of liver parenchyma (Fig. 11.32).
Unenhanced CT can demonstrate marked diffuse hepatic
hyperattenuation. Dual-energy CT and gradient-echo MR
imaging sequences with progressively increasing echo times
(TEs) can be used to quantify iron deposition, which is quali-
tatively noted as low signal intensity on T2-weighted images.
Venesection (phlebotomy) remains the mainstay of therapy.
Cirrhosis
Cirrhosis represents the end stage of liver fibrosis due to pro-
gressive liver disease. Patients with cirrhosis are at risk of
developing complications such as portal vein thrombosis,
varices, hepatocellular carcinoma, and liver failure. The most
common causes of cirrhosis in the first years of life are bili-
ary atresia and genetic/metabolic diseases. In older children,
cirrhosis is usually caused by chronic viral hepatitis and
autoimmune disorders [45]. Patients may be asymptomatic,
or present with abdominal distention, jaundice, gastrointesti-
nal bleeding, hepatomegaly, and/or splenomegaly.
Morphologic changes that can be detected with ultra-
sound, CT, and MR imaging include a nodular contour of
genicity. (c) Axial T2-weighted, fat-suppressed MR image shows gen-
eralized decreased signal throughout the liver and spleen consistent
with secondary hemochromatosis
11 Liver
Fig. 11.33 Cardiac cirrhosis in a 7-year-old male. Transverse gray-
scale ultrasound image shows a small liver (arrows) with an irregular
contour. There is a large volume of ascites (asterisk)
the liver, right posterior lobe hepatic notch, and an enlarged
gallbladder fossa (Fig. 11.33) [46–48]. Caudate lobe hyper-
trophy and segmental atrophy are attributed to alterations of
intrahepatic blood flow [49]. The hepatic echotexture may
be heterogeneous or coarse.
Ultrasound findings associated with portal hypertension
include slow hepatopetal or hepatofugal portal venous flow,
splenomegaly, ascites, and varices associated with portosys-
temic shunts [46]. Regenerative, siderotic, or dysplastic liver
nodules may develop; these are often poorly visualized by
ultrasound and are better assessed with MR imaging where
they are characterized by a lack of washout on delayed phase
imaging.
The treatment of cirrhosis is aimed at the underlying dis-
order and associated complications. Ultimately, liver trans-
plantation may be needed.
Infection
Viral Hepatitis
A variety of viruses can infect the liver and cause hepatitis. In
the newborn, herpes simplex virus (HSV) usually causes a
severe multisystemic disorder with encephalitis and liver fail-
ure [50]. Cytomegalovirus (CMV) is the most common cause
of congenital infection, affecting 1–2% of newborns, and may
present with a petechial rash, hepatosplenomegaly, and jaun-
dice [50]. Other viruses that can cause hepatitis include human
immunodeficiency virus (HIV) and coxsackievirus.
Infection of the liver with a hepatotropic virus can lead to
a spectrum of conditions, from asymptomatic to fulminant
hepatic failure, and persistent subclinical infection to rapidly
progressive chronic liver disease and cirrhosis [51]. Acute
375
Fig. 11.34 Viral hepatitis in a 7-year-old male. Transverse grayscale
ultrasound image shows a diffusely hypoechoic liver with increased
echogenicity of the portal triads (arrowheads) consistent with a “starry
sky” appearance
hepatitis due to hepatitis B virus (HBV) and hepatitis C virus
(HCV) may progress to chronic hepatitis, cirrhosis, and
hepatocellular carcinoma [51]. Chronic infection occurs
among 80%–90% of infected infants with 25% of them ulti-
mately developing cirrhosis [52, 53].
The clinical presentation of viral hepatitis in children is vari-
able, as patients may be asymptomatic or develop signs and
symptoms such as fever, fatigue, abdominal pain, and jaundice.
Findings of viral hepatitis on ultrasound are nonspecific
and include hepatomegaly, decreased echogenicity of the liver
with a relative increase in echogenicity of the portal vein walls
(the so-called “starry sky” appearance) (Fig. 11.34), enlarged
porta hepatis nodes, and gallbladder wall thickening [2, 54].
Similarly, periportal edema or hepatocyte edema may appear
as hypodense areas on CT, or increased T2-weighted signal
intensity on MR imaging with heterogeneous enhancement
[55]. Imaging can also be normal in patients with acute hepa-
titis, or periportal lymphadenopathy may be the only imaging
abnormality [54]. In chronic hepatitis, liver echogenicity may
increase and become coarse or heterogeneous, with an appear-
ance indistinguishable from fatty liver [55].
Treatment for acute HBV infection includes antiviral
agents and supportive care. Universal vaccination of new-
borns is recommended in most countries and has resulted in
a significant decrease in mother-to-child transmission [56].
Bacterial Infection
Bacterial infection of the liver is relatively rare compared with
viral infection and most commonly manifests as liver abscess,
acute bacterial hepatitis, or granulomatous liver disease (e.g.,
tuberculous liver disease and bartonellosis) [57]. Etiologies are
typically polymicrobial and broad-spectrum, due to enteric
376
pathogens entering the liver via the biliary tract, portal vein,
hepatic artery, or direct extension from trauma or invasive
procedures.
Bacterial abscesses account for 75% of all liver
abscesses in the developed world [58]. They are commonly
caused by infections arising from the biliary tract (such as
ascending cholangitis or cholecystitis), hematogenous spread
(as through the hepatic artery in sepsis), or from portal vein
pyemia in complicated abdominal infections (such as
perforated appendicitis) [59]. Bacterial infections frequently
occur in patients with acute-on-chronic liver disease and are
associated with high mortality [60]. Symptoms are often
nonspecific and include fever, abdominal pain, fatigue, and
nausea.
Abscesses may be unilocular, multilocular, and/or multifo-
cal. At ultrasound, smaller hepatic abscesses (less than 2 cm
in diameter) may appear as hypoechoic nodules or vague areas
of altered echogenicity. Larger abscesses can range from
hypoechoic to hyperechoic, depending on the internal contents
(debris and/or thickened septa) and may even appear solid
[59]. Posterior acoustic enhancement and the absence of inter-
nal blood flow by color Doppler may be useful to help differ-
entiate an abscess from a solid neoplastic lesion (Fig. 11.35).
CEUS can increase diagnostic confidence over gray-
scale ultrasound, with demonstration of an avascular, non-
enhancing center, and peripheral enhancement without or
with internal septal enhancement (Fig. 11.36). Notably,
enhancing areas may demonstrate washout, a feature also
seen in hepatic malignancy (Fig. 11.37) [61]. Confidence
a b
Fig. 11.35 Hepatic abscess in a 16-year-old male. (a) Traverse grayscale ultrasound image shows a complex bilobed, cystic intrahepatic fluid
collection (arrows) in the right hepatic lobe that (b) demonstrates no internal flow on color Doppler evaluation
J. K. Kwon et al.
in the diagnosis is increased by the presence of gas within
the abscess [59].
At contrast-enhanced CT, hepatic abscesses appear as well-
defined, low-attenuation lesions with an enhancing rim [59]. A
peripheral low-attenuation outer ring in the portal venous
phase, representing perilesional edema (“double target” sign),
may show enhancement on delayed phase imaging [59].
At MR imaging, abscesses typically demonstrate central
T1-weighted hypointensity and T2-weighted hyperintensity,
although their protein content may cause variation of this
pattern [59]. The rim of the abscess demonstrates early and
persistent enhancement, while perilesional edema appears as
T2-weighted hyperintensity with enhancement in the delayed
phase. Diffusion-weighted imaging (DWI) reveals restricted
diffusion [62]. Organizing abscesses may mimic solid
masses, with central granulation tissue that may occasionally
demonstrate enhancement [63].
Acute bacterial hepatitis may appear normal or demon-
strate nonspecific imaging findings. Granulomatous liver dis-
ease can have a variable appearance, including the absence of
imaging abnormalities. Findings are nonspecific and include
lesions of varying size which are commonly hypoechoic at
ultrasound, hypoattenuating at contrast-­ enhanced CT, and
hyperintense on T2-weighted MR imaging [59].
Therapy includes antibiotic treatment for the underlying
disease. For liver abscesses, image-guided transhepatic per-
cutaneous needle aspiration and/or catheter drainage are
commonly employed, particularly in larger abscesses with a
diameter greater than 5 cm [64].
11 Liver 377

Fig. 11.36 Hepatic abscess in a 9-month-old female with septic shock. Transverse CEUS image shows a hypoechoic lesion in the right lobe of
the liver with increased through transmission on the reference grayscale image (left panel) that demonstrates no internal flow (right panel)

Fig. 11.37 Hepatic abscess in a 16-year-old male with fungal infec- (b) Longitudinal CEUS image obtained in the portal venous phase of
tion. (a) Longitudinal CEUS image of the liver obtained in the late enhancement 3 months later shows internal enhancement (arrow), con-
phase of enhancement shows a well-circumscribed, unenhancing focus sistent with healing
(arrow) with a thick hypoenhancing rim in keeping with an abscess.
378 J. K. Kwon et al.

Fungal Infection more lesions than imaging performed during other phases, with
an increase in the number of identifiable hyperenhancing foci
Hepatic fungal infection occurs in patients with prolonged [67]. Hypoattenuating lesions are usually seen on portal phase
neutropenia and immunosuppression or immunodeficiency imaging, with enhancement patterns varying depending on the
due to medical disorders or medication. Very low birth-­ phase of contrast injection and stage of disease [68]. MR imag-
weight (less than 1500 g) and extremely premature neonates ing features include a T2-weighted hyperintense lesion with a
are also at increased risk [65]. Candida species, Aspergillus nonenhancing center representing necrosis that gradually
species, and Cryptococcus neoformans account for 80% of resolves with treatment [67]. Fungal abscesses may demon-
all fungal infections [66]. Hepatic infection often occurs in strate restricted diffusion on diffusion-weighted images [62].
conjunction with splenic involvement. Due to challenges in accurate and prompt diagnosis of
Clinical manifestations such as fever, abdominal pain, fungal infections, the use of empiric antifungal agents in
jaundice, and hepatomegaly are nonspecific and overlap with febrile neutropenic patients has been advocated [69]. Hepatic
other invasive infections. Blood cultures have a high false- candidiasis can be refractory to conventional therapy or
negative rate and reluctance to perform invasive diagnostic require an extensive course of antifungal therapy [67].
procedures in affected patients makes the diagnosis of invasive
fungal disease challenging [67]. Therefore, imaging plays a
critical role in prompt detection and initiation of therapy. Parasitic Infection
The imaging features of fungal diseases share similar charac-
teristics and are not useful for identifying a specific pathogen. A variety of parasites may infect the liver, and generally
Microabscesses are the characteristic pattern of disease by both demonstrate nonspecific imaging findings, with the excep-
imaging and histology (Fig. 11.38). tion of amebic abscess and echinococcal cyst. Although par-
At ultrasound, four patterns of hepatosplenic candidiasis asitic hepatic infection is most problematic in endemic areas
have been described: hypoechoic nodule (most common, but in the developing world, its incidence is increasing due to
least specific); echogenic focus (2–5 mm, seen late in disease tourism and human migration [70].
and representing areas of calcification); bull’s-eye (hyperechoic The most common extraintestinal complication of
inner inflammatory nidus with a peripheral hypoechoic ring of Entamoeba histolytica infection is amebic liver abscess [68].
fibrosis, generally seen with active infection); and “wheel within Ingested pathogens colonize the colon and then travel to the
a wheel” (the bull’s-eye appearance with an additional central liver via the portal venous system. Symptoms include mal-
hypoechoic nidus representing necrosis, seen early in the course aise and right upper quadrant pain. Diagnosis is aided by
of disease) [68]. stool sample or blood serum analysis [71].
CT and MR imaging are superior to ultrasound for the iden- The imaging appearance of amebic abscess is classically
tification of fungal hepatic lesions [67]. Specifically, arterial unilocular and solitary in the right hepatic dome, but other-
phase imaging at CT and MR imaging depicts significantly wise indistinguishable from a pyogenic abscess [68, 72].

a b

Fig. 11.38 Hepatosplenic candidiasis in a 6-year-old female. Transverse grayscale ultrasound images of the liver (a) and spleen (b) show multiple
tiny hypoechoic lesions (arrowheads)
11 Liver 379

Larger abscesses are at risk for spontaneous rupture and
can lead to the development of pericardial, pleural, or peri-
hepatic fluid collections [73].
Metronidazole therapy is the mainstay of medical treat-
ment for amebic abscess [68]. In uncertain cases, aspiration
or percutaneous drainage aids in identification of the caus-
ative pathogen and treatment. Amebic abscesses may take up
to 2 years to resolve on imaging [68, 72].
Hydatid disease develops after the ingestion of Echi­
nococcus granulosus found in contaminated food, water, and
soil [68]. Embryos invade the bowel mucosa and travel to the
liver via the portal system. As the cyst matures in the liver, the
inner germinal layer (endocyst) invaginates to develop daugh-
ter cysts within the parent cyst, pathognomonic for hydatid
disease.
At ultrasound, the appearance of a hydatid cyst ranges
from anechoic to diffusely echogenic (Figs. 11.39 and 11.40)
[68]. Smaller daughter cysts of varying size can be identified
and may rupture and become detached, appearing as floating
membranes (“water lily” sign) [68]. Intrabiliary rupture is a
common complication and may lead to biliary distortion and
dilation. Shadowing, echogenic foci representing varying

a b

Fig. 11.39 Hepatic hydatid disease in an 18-year-old female. Transverse grayscale ultrasound image of the liver (a) shows a well-circumscribed,
echogenic mass with (b) no internal vascularity by color Doppler

a b

Fig. 11.40 Hepatic hydatid disease in a 16-year-old male. (a) Transverse grayscale ultrasound image shows a well-circumscribed hypoechoic mass
in the liver which demonstrates increased through transmission. (b) Transverse color Doppler ultrasound image shows no internal vascularity
380
degrees of calcification may be present in the periphery of
the cyst, usually indicative of nonviability [68].
CT demonstrates a well-defined, hypoattenuating lesion,
often with internal daughter cysts that may be of lower density
than the parent cyst [68]. MR imaging demonstrates hyperin-
tense T2-weighted and hypointense T1-weighted parent and
daughter cysts, although some variation may be seen due to
internal debris. The peripheral layer of the cyst is hypointense
on T1- and T2-weighted images, due to fibrous and calcified
components.
Treatment of hydatid cysts includes the administration of anti-
parasitic drugs, percutaneous drainage and instillation of a scle-
rosing agent within the cystic cavity, and surgical excision [68].
Trauma
Blunt Abdominal Trauma
The liver is the most frequently injured organ in blunt abdomi-
nal trauma and is often seen in conjunction with other organ
injuries [74]. Hepatic lacerations involve the capsule and are
associated with hemoperitoneum. Hematomas may be intrapa-
renchymal or subcapsular. Bile duct and major hepatic vascular
injuries may also occur. Common causes include motor vehicle
collisions, pedestrian accidents, falls, and sports injuries [75].
Point-of-care emergency extended focused assessment
with sonography in trauma (e-FAST) ultrasound imaging
may be used in unstable patients to depict free fluid indicat-
ing hemoperitoneum that can develop secondary to hepatic
laceration [74]. Standard grayscale ultrasound is of limited
Fig. 11.41 Liver laceration in a 13-year-old male who sustained blunt abdominal trauma. Reference transverse grayscale ultrasound image (left
panel) shows a faint hypoattenuating focus (arrow) in the liver that is more conspicuous (arrowhead) by CEUS (right panel)
J. K. Kwon et al.
diagnostic accuracy for hepatic injury and may depict vague
areas of near-isoechogenicity or hyperechogenicity and free
fluid [3, 76]. Conversely, CEUS has demonstrated promise in
improving the diagnostic accuracy of ultrasound, compara-
ble to that of the gold-standard CT [3, 74, 75, 77].
By both CT and CEUS, traumatic hepatic lesions appear as
areas of hypo-enhancement or nonenhancement of varying size,
shape, and location within the liver, depending on the nature and
force of the injury (Figs. 11.41 and 11.42) [3, 76]. On CEUS,
active bleeding may present as mobile foci of increased echo-
genicity within an anechoic or hypoechoic lesion [74].
Nonoperative management is the mainstay of treatment in
most hemodynamically stable children [78]. Interventional
procedures such as angioembolization are performed for
hepatic arterial hemorrhage. Laparotomy may be neces-
sary in patients with peritonitis or hemodynamic instability.
Delayed complications include hemorrhage, abscess, pseu-
doaneurysm, and bile leakage, many of which can be treated
by interventional radiologic techniques [79].
Umbilical Vein Catheterization
The umbilical vein is commonly used for central venous
access in neonates. An umbilical vein catheter (UVC) nor-
mally courses from the umbilical vein to the left portal vein,
through the ductus venosus and hepatic venous confluence
toward the right atrium, with the tip ideally located at the
inferior cavoatrial junction [27, 80, 81]. A malpositioned
UVC can perforate the vessel wall, leading to liver parenchy-
mal injury or hematoma and extravasation of infused fluids
11 Liver 381

Fig. 11.42 Liver laceration in a 9-year-old male. Reference sagittal grayscale ultrasound image (left panel) shows a faint linear hypoattenuating
focus (arrow) in the liver which becomes more conspicuous (arrowhead) by CEUS (right panel)

a b

Fig. 11.43 Malpositioned umbilical vein catheter (UVC) in an 8-day-­ shows two well-circumscribed, avascular, heterogeneously hyperechoic
old male with extravasation of infused total parenteral nutrition (TPN) foci (arrowheads) in the right lobe of the liver consistent with complex
fluids. (a) Frontal chest radiograph shows an abnormal lateral position fluid collections. A large amount of ascites (asterisk) is present
of the UVC tip (arrow). (b) Sagittal color Doppler ultrasound image

[27, 82]. Symptoms include unexplained acute clinical dete-
rioration, hypotension, drop in hematocrit, and abdominal
distension due to hepatomegaly or ascites [80].
Ultrasound may demonstrate an intrahepatic or subcap-
sular fluid collection that can be anechoic or complex,
depending on the contents of the extravasated fluid and the
duration of injury. Lesion size, shape, and echogenicity can
be variable [81]. Some lesions may be echogenic and lobu-
lated or branching in configuration [80]. Internal flow will
be absent on Doppler evaluation (Fig. 11.43).
Due to the location of the umbilical vein and ductus veno-
sus, abnormalities are more commonly seen in the left hepatic
382
lobe and can be associated with left portal vein thrombosis
[80]. Foci of air and calcification may be detected within the
fluid collection by ultrasound or radiography in the subacute
and late stages of UVC perforation [27]. Additional radio-
graphic findings include an enlarging hepatic silhouette or
mass effect secondary to the fluid collection.
Management is conservative. After removal of the mis-
placed catheter, the fluid collection often resolves with time
[27]. In cases of severe abdominal distension or mass effect,
paracentesis or drainage of fluid from the intrahepatic or
perihepatic collection may speed recovery.
Portal Hypertension
Portal hypertension refers to the pathologic increase in portal
venous pressure due to obstruction of flow that may occur at
the presinusoidal level (e.g., portal vein thrombosis, umbili-
cal vein catheterization, prothrombotic disorders, extrinsic
compression of the portal vein); sinusoidal level (e.g., cir-
rhosis); or postsinusoidal level (e.g., right heart failure, IVC
obstruction) (Table 11.3) [83, 84].
Portal vein thrombosis is the most common cause of extrahe-
patic portal vein obstruction and is a major cause of portal
Table 11.3 Causes of portal hypertension
Prehepatic Hepatic
Presinusoidal
Portal vein thrombosis Granulomatous disease
Umbilical vein catheterization Lymphomas
Splenic or superior mesenteric Polycystic liver disease
vein thrombosis Chronic hepatitis
Prothrombotic disorders Schistosomiasis
Extrinsic compression of portal Primary biliary cirrhosis
vein Primary sclerosing
Partial or complete agenesis of cholangitis
portal vein Cystic fibrosis
Liver disease
NAFLD, Nonalcoholic fatty liver disease
Fig. 11.44 Portal hypertension in an 18-year-old male. (a) Oblique
transverse color Doppler ultrasound image of the main portal vein with
spectral analysis shows reversed (hepatofugal) flow. (b) Sagittal gray-
J. K. Kwon et al.
hypertension in children [85]. In approximately half of cases,
the etiology is idiopathic [83, 84, 86]. In the absence of underly-
ing hepatic parenchymal disease, patients with portal hyperten-
sion frequently present with upper gastrointestinal bleeding due
to ruptured gastric and esophageal varices [86]. Splenomegaly
with or without hypersplenism is also a common clinical mani-
festation. Younger patients may experience growth retardation.
Portosystemic varices may manifest clinically as dilated abdom-
inal wall veins (“caput medusae”) and hemorrhoids.
Ultrasound with Doppler evaluation of portal hypertension
may reveal enlargement of portal venous diameter and
decreased velocity or reversal of flow (hepatofugal flow)
within the portal veins (Fig. 11.44) [87]. Acute portal vein
thrombosis may appear as an anechoic filling defect within the
portal vein, best detected with color Doppler imaging, while
chronic thrombus appears more echogenic. Over time, tortu-
ous portal vein collaterals (also known as cavernous transfor-
mation of the portal vein) may develop and appear as an
irregular tangle of vessels at the porta hepatis, well ­visualized
with color Doppler imaging [84, 86].
A recanalized umbilical vein or enlarged paraumbilical
vein may be seen in the area of the falciform ligament [87].
Other associated findings include splenomegaly, ascites, and
liver cirrhosis. Ultrasound elastography is emerging as a
Post-hepatic
Sinusoidal Post-sinusoidal
Cirrhosis Sinusoidal obstruction Budd–Chiari syndrome
Congenital hepatic syndrome (hepatic vein
fibrosis Chemotherapy obstruction)
Biliary atresia Hematopoietic stem cell Inferior vena cava
NAFLD transplant obstruction
Viral hepatitis Right heart failure
Hemochromatosis Tricuspid regurgitation
Wilson disease Constrictive pericarditis
Peliosis hepatitis
a b
scale ultrasound image reveals marked splenomegaly and hilar varices
(arrow), secondary signs of portal hypertension
11 Liver
noninvasive screening tool for portal hypertension in
patients with chronic liver disease [83, 88, 89].
CT and MRI performed with intravenous contrast agents
also provide visualization of the presence and extent of por-
tal vein thrombosis, portosystemic collateral vessels and
varices [87].
Management requires treatment of the underlying cause
of portal hypertension and its complications. Strategies include
pharmacologic, endoscopic, interventional radiologic,
and surgical approaches [85, 86]. Gastrointestinal variceal
bleeding is treated by endoscopic band ligation. Extrahepatic
portal venous obstruction in patients with refractory variceal
bleeding can be treated by the creation of a surgical Rex shunt
(mesoportal bypass) to restore hepatopetal portal perfusion
[85].
Interventional radiology procedure options include
thrombectomy, angioplasty, and transjugular intrahepatic
portosystemic shunt (TIPS) creation [83, 90]. Patients with
end-stage liver disease and portal hypertension may require
liver transplantation [83].
Budd–Chiari Syndrome
Budd–Chiari syndrome (BCS) refers to the spectrum of
clinical manifestations caused by hepatic venous outflow
obstruction occurring at any level from the small hepatic
veins to the inferior cavoatrial junction [91, 92]. Primary
BCS is due to intrinsic causes of venous obstruction, such as
thrombus or membranes, whereas secondary BCS is due to
extrinsic causes, such as compression by tumor, abscess, or
hyperplastic nodules [93]. Hypercoagulable states account
for the majority of cases [93, 94].
BCS is an example of postsinusoidal portal hypertension,
where hepatic vein occlusion leads to increased sinusoidal and
a b
Fig. 11.45 Budd–Chiari syndrome in an 8-year-old male. (a) Transverse
grayscale ultrasound image shows a markedly thickened gallbladder wall
(arrowheads) and ascites (asterisk). (b) Transverse color Doppler ultra-
383
portal pressure [92]. The severity of clinical presentation is
dependent on the duration and extent of obstruction. Patients
with only a single t­hrombosed hepatic vein may be asymptom-
atic. With extensive venous outlfow obstruction, hepatocellular
hypoxia may result in necrosis and fulminant liver failure [92].
Patients may present acutely with fever, right upper quad-
rant pain, hepatomegaly, coagulopathy, ascites, and renal fail-
ure [95]. Insidious progression of hepatic vein obstruction
may lead to fibrosis and eventual cirrhosis. Chronic BCS may
present with additional manifestations of portal hypertension,
such as progressive abdominal distention due to ascites and
splenomegaly (Fig. 11.45) [92, 93].
On grayscale ultrasound imaging, acute thrombosis of the
hepatic veins or IVC may appear anechoic or hypoechoic, with
documentation of absent flow greatly aided by color and spectral
Doppler evaluation. In the setting of thrombus, flow may be
absent, slow, retrograde, or bi-directional. An obstructing mem-
brane may appear as a linear echogenic structure within the ves-
sel lumen. This membrane, or other focal narrowing, may cause
abrupt cutoff of flow, turbulent flow, or focally increased flow
velocities. Proximal to the site of obstruction, loss of phasicity of
the venous spectral waveform may be demonstrated.
In other instances, the hepatic veins may not be visualized
due to extrinsic compression, or they may appear echogenic
and cord-like. Over time, intrahepatic and extrahepatic veno-
venous collateral vessels may develop, appearing as enlarged,
tortuous, abnormally located vascular channels [93].
Other ultrasound findings include compensatory enlarge-
ment of the caudate lobe, with an enlarged caudate vein
draining directly into an unobstructed IVC. The parenchyma
of the liver segments affected by hepatic vein thrombosis
may acutely appear hypoechoic due to congestion and edema,
or heterogeneous due to infarction or fibrosis with or without
regenerative nodules. Splenomegaly and ascites are also
commonly seen in BCS [92].
sound image of the liver with spectral analysis shows a recanalized
umbilical vein (arrow) with hepatofugal flow. No flow was detected in the
hepatic veins (not shown)
384
CT and MR imaging will demonstrate the same morpho-
logic features depicted by ultrasound. Following intravenous
contrast administration, there will be a lack of enhancement of
thrombosed vessels and corresponding hepatic segments [93,
96]. Liver parenchymal findings include patchy, decreased
peripheral enhancement due to stasis in the hepatic sinusoids
and portal veins, with relatively increased enhancement in the
central portions of the liver and the caudate lobe [92, 93, 97]. On
T2-weighted MR imaging, heterogeneously increased signal
intensity is seen in the liver periphery.
Diagnostic catheter venography is performed prior to
interventional procedures and may demonstrate occlusion or
stenosis of the hepatic veins or IVC, large intrahepatic col-
lateral vessels, or a spiderweb-like appearance of smaller
collaterals [92, 98, 99].
Initial treatment options include angioplasty, stent place-
ment, and catheter-directed thrombolysis [100]. Transjugular
intrahepatic portosystemic shunt (TIPS) creation and liver trans-
plantation have improved mortality in recent years [92, 101].
Long-term anticoagulation is often required in most patients
with BCS due to an underlying hypercoagulable state [101].
a b
Fig. 11.46 Sinusoidal obstruction syndrome in an 18-month-old
female. (a) Transverse grayscale ultrasound image shows gallbladder
wall thickening (arrowhead) and a small amount of ascites (arrows).
J. K. Kwon et al.
Sinusoidal Obstruction Syndrome
Sinusoidal obstruction syndrome (SOS), previously known as
veno-occlusive disease (VOD), is a disorder that causes venous
outflow obstruction in the liver due to epithelial injury and sinu-
soidal obstruction. First recognized in patients who ingested
toxic plant alkaloids, SOS has been reported in up to 77% of
patients who have undergone hematopoietic stem cell transplan-
tation (HSCT) [102]. Patients receiving chemotherapy and
immunosuppression without HSCT have also been affected.
Classically, patients present with painful hepatomegaly,
weight gain, and ascites within the first 21 days following
HSCT, but novel therapies have led to an increase in late-
onset disease [103]. Advanced disease may lead to fibrosis or
the rapid onset of multi-organ failure [104].
Ultrasound, MR imaging, and CT may all show hepato-
megaly, ascites, and gallbladder wall thickening, which are
nonspecific findings (Fig. 11.46) [104, 105]. Elevated hepatic
artery resistive indices can also be demonstrated. While
reversal of portal venous flow with Doppler ultrasound is
diagnostic, it is usually only present in the late stages of the
Oblique transverse color Doppler ultrasound images with spectral analy-
sis show (b) diminished diastolic flow in the hepatic artery with an ele-
vated resistive index (RI), and (c) pulsatile flow in the main portal vein
11 Liver
disease and its absence does not exclude SOS. Therefore,
diagnosis is largely based on clinical criteria [105].
Aggressive fluid management is essential in treating
SOS. Mild cases can be managed with supportive measures
only. Defibrotide is the only approved agent for the treatment
of SOS in children and adults. Its mechanism of action is not
completely understood, but it is believed to play a role in endo-
thelial protection. High-dose steroids are also of benefit [105].
Peliosis Hepatis
Peliosis hepatis is a rare, benign vascular condition of the
liver characterized by dilated sinusoidal spaces, with or
without an endothelial lining, resulting in blood-filled cystic
­cavities of varying size, ranging from 1 mm to several cm
[106]. Other organs, such as the spleen and bone marrow,
can also be involved.
While the pathogenesis of peliosis hepatis remains unclear,
hepatocellular necrosis and sinusoidal damage likely play a role.
It is associated with a variety of conditions, including drug-­related
(steroids, chemotherapeutic agents, oral contraceptives, azathio-
prine), immunosuppression (posttransplantation or hematologic
disorders), and infection (tuberculosis, HIV, and Bartonella
henselae). Rare pediatric associations include X-linked myotubu-
lar myopathy, Escherichia coli sepsis, and cystic fibrosis. In
20–50% of patients, no risk factor is identified [106].
Peliosis hepatis is often discovered incidentally on imaging or
autopsy, as many patients are asymptomatic. Abdominal pain,
cholestasis, hepatomegaly, portal hypertension, or liver failure
may be present. Larger lesions can rupture, leading to intrahepatic
hemorrhage or hemoperitoneum and hypovolemic shock [106].
Fig. 11.47 Peliosis hepatitis in a 20-year-old female with a history of
liver transplantation. (a) Transverse grayscale ultrasound image shows
multiple small, round, echogenic foci (arrowheads) throughout the right
lobe of the liver. (b) Axial T2-weighted, fat-suppressed MR image
shows multiple tiny hyperintense foci in the right lobe of the liver which
385
Imaging findings are nonspecific and variable, based on
the size and number of the lesion cavities, the physical state
of the blood components, and the presence of hepatic steato-
sis. Innumerable small lesions or several large cystic cavities
may be present. On ultrasound, lesions tend to be hyper-
echoic, although they may appear hypoechoic in the setting
of hepatic steatosis, or heterogeneously hypoechoic due to
internal hemorrhage (Fig. 11.47) [106]. Doppler imaging
may reveal peri- or intra-lesional vascularity. At CEUS, peli-
otic lesions may demonstrate an early arterial central
enhancement compatible with a target-sign pattern, followed
by centrifugal filling and homogeneous enhancement com-
parable to that seen at contrast-enhanced CT and MR imag-
ing [107].
Unenhanced CT typically demonstrates a hypoattenuating
lesion, although hemorrhage and calcification may increase
attenuation. On MR imaging, lesions usually appear hyperin-
tense on T2-weighted images, and hypointense on T1-weighted
images, although hyperintense T1- and T2-weighted foci may
represent foci of hemorrhage [106]. Thrombosed cavities may
only enhance peripherally, mimicking the appearance of an
abscess. In some instances, centripetal enhancement is remi-
niscent of the appearance of a hemangioma. Some lesions may
mimic liver tumors, although the lack of mass effect with peli-
osis hepatis helps to differentiate between them. Lesions
smaller than 1 cm may go undetected.
When associated with an offending agent, peliosis hepatis
may resolve with discontinuation of the medication or treatment
of infection [106]. Due to the potential for serious complica-
tions, periodic surveillance or surgical resection should be con-
sidered. Successful transcatheter embolization in the emergent
setting of massive hemorrhage has been described [108].
are not in continuity with the biliary tree. These foci did not enhance on
the arterial or early portal venous phases of the study. Enhancement was
identified on delayed imaging (not shown). Subsequent biopsy con-
firmed peliosis hepatitis
386
Passive Venous Congestion
Passive venous congestion of the liver, also known as congestive
hepatopathy, refers to the spectrum of liver abnormalities that
are due to any cause of right heart failure and elevated central
venous pressure (CVP) [108]. The elevated CVP is transmitted
to the hepatic vessels, leading to sinusoidal dilation and conges-
tion, perisinusoidal hemorrhage, thrombosis, and fibrosis [109].
The mottled gross pathological appearance of the liver with pas-
sive venous congestion has been described as a “nutmeg liver,”
caused by nodular zones of red cell accumulation. Chronicity
may lead to the development of focal nodular hyperplasia, cir-
rhosis, and hepatocellular carcinoma [109, 110].
Children and adolescents with congenital heart disease
and single-ventricle anatomy who undergo palliative Fontan
reconstruction, whereby systemic venous return bypasses
the right ventricle and passively flows to the pulmonary
arteries, are becoming an increasingly significant group of
patients who require screening for liver disease [110].
Symptoms relating to the underlying cardiac disease typi-
cally dominate; dull right upper quadrant pain and mild ele-
vation of liver enzymes may be present.
Ultrasound findings include a dilated IVC and dilated
hepatic veins (Fig. 11.48) [109]. Spectral Doppler evaluation
may reveal diminished respiratory variation along with
dampening or loss of the normal tetraphasic hepatic venous
waveform [109]. Acute congestion may result in hepatomeg-
aly while chronic congestion can lead to cirrhosis with a
nodular liver contour, splenomegaly, and ascites.
In the setting of high right atrial pressures, CT with con-
trast administered via an upper extremity vein may demon-
strate early enhancement of the IVC and hepatic veins due
to reflux of contrast material [109]. Patchy enhancement of
Fig. 11.48 Passive venous congestion in a 20-month-old female with
right-sided heart failure. Transverse grayscale ultrasound image of the
liver shows engorged hepatic veins (arrowheads) and ascites (asterisk)
J. K. Kwon et al.
the liver parenchyma with reticular areas of hypoenhance-
ment is noted particularly in the liver periphery during the
portal venous phase, with corresponding T2 hyperintensity
on MR imaging. Delayed MR imaging using a hepatocyte-
specific contrast agent can show nonenhancing linear or
reticular bands representing fibrous septa next to the hepatic
veins with sparing of the regions next to the portal triads.
Periportal edema manifests as periportal tracks of hypoin-
tensity on CT, hyperintensity on T2-weighted images, and
delayed phase enhancement. Hypervascular nodules may
be present.
Ultrasound and MR elastography are useful in detecting
increased liver stiffness due to venous congestion, particu-
larly in the liver periphery, but cannot otherwise differentiate
between congestion, fibrosis, and other causes of increased
liver stiffness.
There is no specific treatment for passive venous congestion.
Therapy is aimed at treating the underlying cardiac disease,
including cardiac transplantation. Diuretics and pulmonary vas-
cular vasodilator therapy may be beneficial [111].
Portal Venous Gas
Portal venous gas can be seen in a variety of conditions,
ranging from benign to life-threatening [112]. Gas may enter
small or large intestinal veins via a compromised mucosa, or
as a result of increased intraluminal pressure, and subse-
quently travel to the portomesenteric venous system [113].
In children, portal venous gas is commonly associated with
pneumatosis intestinalis resulting from necrotizing enteroco-
litis in premature infants, or occurs secondary to umbilical
vein catheterization [1, 114].
Portal venous gas can be seen in other conditions with or
without vascular compromise, bowel ischemia, or bowel
mucosal injury, such as intraabdominal infection, inflamma-
tory bowel disease, bowel dilation, following an interven-
tional procedure or after steroid administration. Uncommon
causes include occult child abuse [115], liver transplantation
[116], and hypertrophic pyloric stenosis [117].
Ultrasound has a high sensitivity for the detection of por-
tal venous gas [114]. On grayscale images, it appears as
punctate or short linear, echogenic foci oriented in a branch-
ing configuration (Figs. 11.49 and 11.50). The echogenic
foci may be mobile, traveling toward the liver periphery and
conforming to the course and direction of blood flow within
portal veins [114, 118]. The typically peripheral location
helps to differentiate portal venous gas from air in the biliary
tree (pneumobilia), which is more centrally located within
the liver.
Duplex Doppler ultrasound shows mobile echogenic foci
within the lumen of the portal vein, producing sharp bidirec-
tional spikes superimposed on the normal monophasic portal
11 Liver
Fig. 11.49 Portal venous gas in a premature 4-day-old male with com-
plex congenital heart disease. Transverse grayscale ultrasound image
shows multiple punctate echogenic foci (arrowheads) in a linear distri-
bution in the left lobe of the liver
a b
Fig. 11.51 Congenital liver hemangioma in a 1-day-old female. (a)
Transverse grayscale ultrasound image of the liver shows a large, mul-
tilobulated, soft tissue mass in the right hepatic lobe that contains
venous wave pattern [114]. Radiographically, portal venous
gas appears as peripheral branching lucencies in the liver;
similarly, on CT, branching air-density foci are demonstrated
in the portal vein and its branches, often predominantly in
the left hepatic lobe.
Treatment for portal venous gas is aimed at the underly-
ing cause and may include antibiotics, bowel decompression,
and urgent laparotomy.
387
Fig. 11.50 Portal venous gas in a premature 12-day-old female with
necrotizing enterocolitis and abdominal distention. Transverse gray-
scale ultrasound image shows echogenic foci (arrow) in the main portal
vein and diffusely throughout the liver within distal intrahepatic portal
venous branches
numerous internal anechoic tubular structures. (b) Color Doppler ultra-
sound image reveals multiple enlarged vessels surrounding and extend-
ing into the mass
Tumors
Benign Masses
Congenital Hemangioma
Congenital hemangioma (CH) is a rare, benign vascular tumor
that is typically focal and fully grown at birth (Fig. 11.51,
Tables 11.4 and 11.5). CHs occur in the liver but are more
388 J. K. Kwon et al.

Table 11.4 Characteristics of liver masses in children

Typical
Age of ultrasound Contrast-enhanced Calcification/
Entity presentation appearance ultrasound features Hemorrhage AFP levels Associations
Benign
Hemangioma, First year of Hyperechoic with Arterial peripheral + (CH, if large) Elevated initially Low birth weight,
congenital life prominent nodular enhancement (CH) cutaneous
(CH), and vascular with gradual central hemangioma,
infantile channels, IHH filling PHACES syndrome,
(IHH) may be hypothyroidism
multifocal (IHH)
Mesenchymal Less than Predominantly Minimal enhancement – (uncommon) Normal (can be Typically isolated,
hamartoma 2 years cystic with mildly DICER1 gene
septations, elevated) mutation may
internal predispose to other
low-level echoes malignancies
Focal nodular Varies Isoechoic or Early arterial – (uncommon) Normal Prior chemotherapy,
hyperplasia hypoechoic, enhancement of a hereditary
may have a feeding vessel, spoke hemorrhagic
central scar wheel pattern telangiectasia,
portosystemic shunt
Hepatic Adolescence Hyperechoic with Rapid arterial ++ Normal Oral contraceptives,
adenoma internal hyperenhancement anabolic steroids,
heterogeneity with centripetal glycogen storage
filling, isoechoic or disease type I, FAP,
rare washout on Hurler syndrome
delayed phases
Malignant
Hepatoblastoma Less than Heterogeneous, Early arterial ++ Elevated Prematurity, very low
5 years cystic and solid, hyperenhancement birth weight,
portal or hepatic with washout on Beckwith–
vein invasion portal venous phase Wiedemann, FAP,
glycogen storage
disease type IA
Hepatocellular Over 5 years Heterogeneously Early arterial + Elevated Perinatal HBV
carcinoma hypoechoic, but hyperenhancement infection, biliary
(HCC) may be mixed with washout on atresia,
or hyperechoic portal venous phase hemochromatosis,
glycogen storage
disease type I,
Wilson disease,
hereditary
tyrosinemia
Fibrolamellar Adolescence Heterogeneous, Heterogeneous arterial + Usually normal None identified
HCC central enhancement with
echogenic washout on portal
strands, may be venous phase
multifocal
Undifferentiated 6–10 years Solid with areas of Nonspecific/limited data – (uncommon) Normal Rarely may arise within
embryonal cystic change or existing
sarcoma necrosis mesenchymal
hamartoma
AFP, Alpha-fetoprotein; CH, congenital hemangioma; FAP, familial adenomatous polyposis; HVB, hepatitis B virus; IHH, infantile hepatic
hemangioma

Table 11.5 Characteristics of liver hemangiomas

Type Congenital Infantile
Subtype Rapidly involuting (RICH) Non-involuting (NICH) Partially involuting (PICH) Infantile hemangioma (IHH)
Growth Fully grown at birth Grows proportionately with child Fully grown at birth Proliferates in the first year of life
Involution Typically involutes by 14 None Initial regression followed Slow regression up to 10 years
months by stability of size
GLUT-1 Negative Negative Negative Positive
Focality Unifocal Unifocal Unifocal Typically multifocal/diffuse
GLUT-1, Glucose transporter protein-1
11 Liver
a b
c d
Fig. 11.52 Rapidly involuting congenital liver hemangioma (RICH) in a
2-month-old male. (a) Transverse grayscale ultrasound image shows a
large, heterogeneous right hepatic lobe mass containing anechoic tubular
structures and focal calcification. The mass is smaller when compared to
prior studies (not shown). (b) Transverse color Doppler ultrasound image
often identified in the skin or subcutaneous tissues of the head
and neck region and lower extremities. Three subtypes have
been recognized: rapidly involuting congenital hemangioma
(RICH), which typically involutes completely by 14 months
of age (Fig. 11.52); noninvoluting congenital hemangioma
(NICH), which does not regress, but grows proportionately
with the child’s overall growth; and partially involuting con-
genital hemangioma (PICH), which undergoes initial growth
regression followed by stabilization (Fig. 11.53) [119–122].
Immunostaining for glucose transporter protein-1 (GLUT-
1) is an important differentiator between CH, which is GLUT-1
negative, and infantile hepatic hemangioma (IHH), which is a
389
shows marked hypervascularity of the lesion (c) Transverse color
Doppler ultrasound image with spectral analysis reveals low resistance
arterial flow within the mass. (d) Sagittal grayscale ultrasound image
shows marked tapering of the abdominal aorta (A) below the level of the
liver (arrow) due to arteriovenous shunting within the mass. L, Liver
separate entity with distinctive clinical features and GLUT-1
positivity [123, 124]. Serum alpha-fetoprotein (AFP) levels
may be elevated initially but should subsequently decrease.
Hepatic CH may be detected prenatally or incidentally, and
most are asymptomatic. Larger CH may present as an abdomi-
nal mass in an otherwise healthy infant. Transient thrombocy-
topenia and anemia occur some infants. High-output heart
failure can develop in infants with large CH, due to arteriove-
nous shunting through the tumor and cardiac overload [123].
Most CHs are unifocal, whereas IHH is more commonly
multifocal or diffuse (see further discussion of IHH in
Section “Infantile Hemangioma”).
390
a b
c d
g
f
Fig. 11.53 Partially involuting congenital liver hemangioma (PICH) in
a 2-month-old male. Serial follow-up showed partial involution that then
stabilized. (a) Transverse grayscale ultrasound image of the right hepatic
lobe demonstrates a large, heterogeneous mass with internal tubular and
round anechoic structures consistent with prominent vascular channels
or areas of intralesional hemorrhage. (b) Longitudinal power Doppler
ultrasound image of the right hepatic lobe demonstrates blood flow at the
periphery and within the mass (c) Longitudinal CEUS image obtained
during the early arterial phase demonstrates a nodular, hyperenhancing
periphery (white arrowheads). (d) Longitudinal CEUS image obtained
J. K. Kwon et al.
e
during the early arterial phase one second after the image in (c) demon-
strates further centripetal enhancement of the mass (white arrowheads).
(e) Longitudinal CEUS image obtained during the portal venous phase
demonstrates near-complete enhancement of the mass. Small, persistent,
central unenhancing foci represent intralesional bleeding, thrombus, and/
or fibrosis. (f) Axial contrast-enhanced, T1-weighted, fat-suppressed MR
image shows thick, peripheral and heterogeneous central enhancement of
the hemangioma. (g) Axial T2-weighted, fat-suppressed MR image shows
diffuse hyperintensity of the lesion with central hypointense flow voids
11 Liver
On ultrasound imaging, features common to both types of
hemangioma include well-circumscribed solid tissue that
may appear hyperechoic, hypoechoic, or of mixed echo-
genicity with prominent vascular channels. CH often dem-
onstrates focal calcifications and macroscopic arteriovenous
shunting. Doppler examination will reveal low-­resistance,
high-velocity arterial flow.
On CEUS, CT, or MR imaging, there is arterial peripheral
nodular enhancement with gradual central filling and diffuse
enhancement with lack of washout on delayed imaging. In
smaller lesions, the fill-in is more rapid and may appear instanta-
neous, probably due to the abundant arteriovenous shunts [125].
For CH, exceptions to the typical pattern of diffuse enhance-
ment may occur in the setting of intralesional bleeding, throm-
bosis, fibrosis, or coarse calcification, resulting in a
heterogeneous, solid and cystic appearance [126]. On MR
imaging, lesions demonstrate T2-weighted hyperintensity and
T1-weighted hypointensity, and flow voids may be identified.
Arteriovenous shunting may result in enlargement and
increased flow velocity of hepatic arteries and veins associated
with the hemangioma, and tapering of the aorta distal to the
celiac trunk [1, 122, 123, 127]. RICH will eventually completely
involute, often leaving only a clump of coarse calcifications.
RICH typically involutes by the age of 14 months, and
treatment is not usually necessary. For symptomatic patients,
propranolol is the mainstay of medical therapy. Continued
monitoring for at least 1 year, with documentation of stable
size and vascularity, is recommended [128].
Infantile Hemangioma
Infantile hepatic hemangioma (IHH) is the most common
benign tumor of infancy, occurring in 5% of infants, with a
a b
Fig. 11.54 Infantile liver hemangioma in a 10-month-old female. (a)
Reference transverse grayscale ultrasound image of the liver shows a
well-circumscribed, echogenic focus (arrow) that (b) demonstrates prompt,
391
higher incidence in girls, preterm infants, and in Caucasians
(Tables 11.4 and 11.5) [127, 129]. IHH is frequently associ-
ated with cutaneous infantile hemangiomas. Immunostaining
for GLUT-1 is an important differentiator between IHHs,
which are GLUT-1 positive, and CHs, which are GLUT-1
negative. Unlike CH, IHH is generally not present at birth but
usually becomes apparent within 4–6 weeks. It proliferates in
the first year of life, followed by a slow involution that can last
up to 10 years [130].
IHHs usually present as multi-focal or diffuse lesions
that cause hepatomegaly. When diffuse, almost the entire
liver parenchyma can be replaced, putting patients at risk
for severe complications, including high-­output cardiac fail-
ure due to high volume arteriovenous shunting, hypothy-
roidism secondary to overproduction of type III
iodothyronine deiodinase, bleeding, and abdominal com-
partment syndrome [123, 131–133].
As noted above for CH, the tumor nodules may appear as
hyperechoic, hypoechoic, or mixed echogenicity well-­
circumscribed masses with prominent vascular channels.
Doppler examination reveals low-resistance, high-velocity arte-
rial flow. In contrast to CH, these tumors do not generally exhibit
internal hemorrhage, necrosis, or calcification. On CEUS, CT, or
MR imaging, there is typically arterial peripheral nodular
enhancement with gradual central filling and diffuse enhance-
ment with lack of washout on delayed imaging (Fig. 11.54).
The majority of IHHs are discovered incidentally during
routine imaging and do not require specific treatment.
Ultrasound screening for liver IHH is recommended when
five or more cutaneous IHHs are noted [134]. Propranolol is
the mainstay of medical therapy.
rapid uptake (arrowheads) during the arterial phase of CEUS, greater
than that of the adjacent hepatic parenchyma
392
Mesenchymal Hamartoma
Hepatic mesenchymal hamartoma (HMH) is the second most
common benign liver mass in children, after hemangioma
(Table 11.4) [135]. HMH is primarily seen in children under
the age of 2 years and typically manifests as a large multicystic
liver mass composed of a disordered arrangement of primitive
mesenchyme, cysts lined with biliary-type epithelium, and
hepatic parenchyma. Although the etiology is not well under-
stood, HMH is associated with microRNA dysregulation and
the DICER1 gene mutation [136, 137].
Children with HMH may present with an enlarging, pain-
less abdominal mass that may lead to respiratory distress
a b
Fig. 11.55 Mesenchymal hamartoma of the liver in a 2-year-old female.
(a) Transverse grayscale ultrasound image shows a large, cystic mass with
thick septations. The cysts are of varying size and contain low-­level echoes.
J. K. Kwon et al.
from mass effect or lower limb edema due to compression of
the IVC [135]. If the mass is very large, complications can
occur, including ascites, jaundice, and congestive heart fail-
ure due to intratumoral arteriovenous shunting. Paradoxically,
while there are reports of spontaneous regression, HMH can
rarely progress to an aggressive malignant undifferentiated
embryonal sarcoma (UES) [135, 138]. AFP levels may be
elevated.
The classic imaging appearance of HMH is a complex
cystic mass with thin internal septations (Fig. 11.55) [132,
139]. However, the appearance may range from predomi-
nantly cystic with thin or thick septa to predominantly solid-­
(b) Transverse color Doppler ultrasound image demonstrates no evidence
of septal hypervascularity. (c) Coronal T2-weighted, fat-suppressed MR
image reveals that the mass replaces most of the right hepatic lobe
11 Liver
appearing due to the presence of internal thick, proteinaceous
material or hemorrhage, with a few small cysts. On ultra-
sound, anechoic spaces of varying size and number are seen
and may contain low-level echoes, and thin or thick echo-
genic linear bands. The solid portions are echogenic. There
is little color Doppler activity, which is limited to the septa-
tions and solid portions of the mass.
On CT, the cystic portions of the mass are of fluid density
and nonenhancing, while septations and solid portions dem-
onstrate hypoenhancement compared to the background nor-
mal liver [132]. On MR imaging, the cystic portions of HMH
demonstrate T2 hyperintensity, with variable T1 hypointen-
sity depending on the protein content of the cyst fluid.
Septations and solid portions demonstrate hypointensity on
T1- and T2-weighted images, and mild enhancement follow-
ing contrast material administration [138].
Surgical resection has traditionally been the treatment of
choice. Less invasive techniques, such as ultrasound-guided
cyst aspiration, either prenatally or postnatally [140], or
­laparoscopic fenestration, have been used [141]. Some inves-
tigators have opted for “watchful waiting” in asymptomatic
patients, while others advocate resection of all HMHs due to
their malignant potential [135, 139].
 ocal Nodular Hyperplasia
F images; and has a hyperintense central scar on T2-weighted
Focal nodular hyperplasia (FNH) is a benign epithelial liver
tumor marked by the nodular proliferation of hyperplastic
hepatocytes and biliary epithelium (Table 11.4) [3]. It is
thought to occur as a result of a vascular abnormality origi-
nating within the lesion’s fibrous central stellate scar. An
increased prevalence of FNH has been observed in patients
with hereditary hemorrhagic telangiectasia and in children
with a history of chemotherapy, which suggests that chemo-
therapy-induced vascular injury can lead to the development
of FNH [142, 143]. FNH is a benign mass with nonaggres-
sive features. It is often detected incidentally, most com-
monly in adult women, young children and adolescents.
Patients may complain of right upper quadrant pain. Rare
complications include hemorrhage and tumor rupture.
As FNH is mostly composed of hepatocytes, its appear-
ance may be subtle on imaging, with detection aided by the
393
visualization of a central vascular scar or mass effect on
adjacent structures.
On ultrasound, FNH appears as a homogeneous mass
that is isoechoic, hypoechoic, or hyperechoic to normal
liver. Doppler evaluation reveals arterial flow within the
hyperechoic central scar, with blood flowing to the
periphery in a spoke-wheel configuration and displace-
ment of vessels along the periphery of the lesion
(Fig. 11.56) [3, 144].
CEUS is a favorable alternative to CT and MR imaging
due to the lack of associated ionizing radiation, shorter scan
times, and without the need for sedation [3]. CEUS findings
include early arterial enhancement of a central feeding ves-
sel leading to branching vessels in a spoke-wheel configura-
tion [3]. Subsequently, there is centrifugal hyperenhancement
of the lesion in the portal venous phase, and absence of
washout on delayed phase imaging. A nonenhancing central
scar may be seen (Fig. 11.57).
Multi-phase CT and MR imaging may demonstrate char-
acteristic features of FNH. On unenhanced CT, FNHs are
hypodense or isodense to background liver, although they
may be hyperdense if the liver is fatty. On MR imaging, FNH
is typically isointense or hypointense on T1-weighted
images; slightly hyperintense or isointense on T2-weighted
images. The postcontrast appearance of an FNH is similar on
CT and MR imaging. In the arterial phase, there is homoge-
neous hyperenhancement, except for the central scar. In the
portal venous and delayed phases, the lesion may become
more isoenhancing, and the central scar may show some
enhancement.
If a hepatocyte-specific MR imaging contrast agent such as
gadoxetate disodium (Eovist®, Bayer HealthCare Pharma­
ceuticals) is used, FNHs will demonstrate late hepatocyte-
phase imaging (20 minutes after injection of contrast) due to
the presence of functioning hepatocytes, a feature which helps
to differentiate FNH from other liver lesions [145].
FNHs are typically asymptomatic lesions requiring no
treatment. In symptomatic patients, transarterial emboliza-
tion has been used to successfully control symptoms and
reduce lesion size [146].
394
a b
Fig. 11.56 Focal nodular hyperplasia of the liver in a 16-year-old
female. (a) Transverse grayscale ultrasound image shows a well-defined
mass in the right hepatic lobe which is isoechoic to the liver paren-
chyma and contains a mildly echogenic, central stellate scar (arrow-
Hepatic Adenoma
Hepatic adenoma is a benign lesion formed by the prolif-
eration of pleomorphic hepatocytes that contain lipid and
glycogen and are arranged in sheets or cords rather than a
normal acinar pattern (Table 11.4) [127]. They are typically
solitary, occur more often in females than males, and are
associated with the use of estrogen-containing oral contra-
ceptives and anabolic androgenic steroids [132]. In chil-
J. K. Kwon et al.
heads). (b) Transverse color Doppler ultrasound image demonstrates
peripheral vessels within the mass. (c) Axial T2-weighted, fat-sup-
pressed MR image depicts the hyperintense central scar (arrow)
dren, hepatic adenomas are associated with glycogen
storage disease where multiple lesions are often present
[127].
Patients are usually asymptomatic but may also present
with hepatomegaly and abdominal pain due to spontaneous
rupture leading to hemorrhage, including life-threatening
hemoperitoneum [127].
11 Liver
Fig. 11.57 Focal nodular hyperplasia of the liver in a 17-year-old
male. (a) Longitudinal CEUS image obtained in the early arterial phase
shows a spoke-wheel pattern of enhancement. (b) Longitudinal CEUS
On grayscale ultrasound imaging, the appearance of
hepatic adenoma is nonspecific (Fig. 11.58). It is well-cir-
cumscribed and may be hyperechoic due to the high lipid
content of hepatocytes and heterogeneous due to the pres-
ence of hemorrhage and calcification [127]. In the setting of
fatty infiltration of the liver as with glycogen storage disease,
395
image obtained in the late phase and (c) axial contrast-enhanced CT
image show diffuse hyperenhancement of the lesion, with a central
unenhanced focus (arrows) consistent with a scar
an adenoma may be hypoechoic relative to the adjacent liver
parenchyma (Fig. 11.59) [127]. There is no central scar.
CEUS of hepatic adenoma typically shows rapid arterial
hyperenhancement with centripetal filling of the lesion. In
the portal venous and late phases, hepatic adenoma is typically
isoechoic to the surrounding liver parenchyma. Occasionally,
396
a b
Fig. 11.58 Hepatic adenoma in a 15-year-old male. (a) Sagittal gray-
scale ultrasound image shows a heterogeneous mass (arrowheads) in
hepatic segment VII. (b) Sagittal color Doppler ultrasound image demon-
strates flow at the periphery of the lesion. (c) Axial gadoxetate disodium
in the late phase, gradual washout may occur, making the
differentiation from malignancy more challenging and
necessitating biopsy for diagnosis [3].
Postcontrast CT and MR imaging show enhancement
patterns that are similar to those of CEUS [127]. On non-
contrast CT, an adenoma may be isodense, hyperdense, or
hypodense, depending on the presence of surrounding fatty
infiltration and any intralesional fat, hemorrhage, or calcifi-
cation. On MR imaging, intralesional blood products may
create significant heterogeneity; out-of-­phase imaging typi-
J. K. Kwon et al.
(Eovist®) contrast-enhanced, T1-weighted, fat-suppressed MR image in
the hepatobiliary phase demonstrates a hypointense mass (arrowheads) in
the right hepatic lobe
cally demonstrates signal dropout due to the presence of
microscopic fat [127].
Cessation of any hormonal therapy may lead to spontane-
ous regression [127]. Due to the risk of spontaneous rupture
and risk of malignant transformation, surgical resection is
considered the standard of care for larger adenomas [147].
Transarterial embolization and microwave ablation are
emerging minimally invasive interventional radiology alter-
natives to surgical resection [148].
11 Liver
Fig. 11.59 Hepatic adenoma in a 17-year-old female with glycogen
storage disease type I. (a) Longitudinal grayscale ultrasound image of
the liver demonstrates multiple hepatic masses. The largest lesion
(arrow) is of mixed echogenicity while the smaller lesions (arrowheads)
are hyperechoic relative to the surrounding hepatic parenchyma. (b)
Longitudinal CEUS image of the largest lesion in (a) shows early arte-
rial enhancement. (c) Longitudinal CEUS image obtained less than one
second after the image in (b) shows rapid centripetal enhancement. (d)
Malignant Tumors
Hepatoblastoma
Hepatoblastoma is the most common primary hepatic malig-
nancy of childhood (Table 11.4) [149]. It is composed of
embryonic cells with a spectrum of differentiation, from a
poorly differentiated epithelial pattern, to well-differentiated
cell types. Hepatoblastomas are divided into two broad cat-
egories: epithelial type and mixed epithelial and mesenchy-
mal type. When present, mesenchymal cells, also with a
range of differentiation, confer a heterogeneous appearance
on imaging and pathologic analysis [149–152].
Hepatoblastoma has been associated with Beckwith–
Wiedemann syndrome, Gardner syndrome (familial colorectal
polyposis), trisomy 18, and type 1A glycogen storage disease
397
Longitudinal CEUS image obtained in the portal venous phase reveals
persistent hyperenhancement of the lesion compared to the adjacent
normal hepatic parenchyma. (e) Longitudinal CEUS image obtained
during the delayed phase reveals iso- to minimal hyperenhancement of
the lesion compared to the adjacent normal hepatic parenchyma. No
washout of contrast from the lesion relative to the normal parenchyma
was demonstrated
[149]. An increased prevalence in very low birth weight infants
has been observed [151, 153]. Two-thirds of cases present in the
first 2 years of life and 90% are seen in patients before the age
of 5 years [149, 154]. There is a slight male predominance.
Most children present with abdominal distension or nonspe-
cific symptoms such as anorexia, weight loss, and vomiting.
Jaundice is infrequently present. Common sites of metastatic
disease include the lungs, bone, brain, and lymph nodes. Serum
AFP levels are abnormally elevated in 90% of patients [149].
On imaging, hepatoblastoma appears as a well-circumscribed,
lobulated, or septated mass, often large enough to cause sig-
nificant hepatomegaly. Depending on its cellular make-up,
the appearance may be homogeneous or heterogeneous, and
coarse calcifications are often present [149]. Portal or hepatic
vein invasion is commonly seen.
398
The PRE-Treatment EXTent of tumor (PRETEXT) stag-
ing system for children with a primary hepatic malignancy
is based on determining the number of contiguous tumor-
free liver sections. It is used to guide prognosis and man-
agement and takes into consideration the extent of tumor
within the liver, as well as additional annotation factors
such as vascular involvement, extrahepatic extent, multifo-
cality, and tumor rupture [155]. PRETEXT is widely
accepted as a means of describing tumor extent in a stan-
dardized manner and has been proven to correlate with
patient prognosis. PRETEXT staging is optimally deter-
mined by MR imaging [152, 155].
a c
Fig. 11.60 Hepatoblastoma in a 10-month-old male. (a) Transverse
grayscale ultrasound image shows a large heterogeneous mass (arrows)
replacing most of the right hepatic lobe. Internal echogenic foci (arrow-
heads) demonstrate posterior acoustic shadowing consistent with calci-
fication. (b) Coronal contrast-enhanced, T1-weighted, fat-suppressed
J. K. Kwon et al.
On ultrasound, hepatoblastoma is usually hyperechoic with
hypoechoic septa, and variable degrees of heterogeneity, marked
by echogenic shadowing calcifications or anechoic foci repre-
senting hemorrhage or necrosis (Fig. 11.60) [152, 156, 157].
CEUS shows early peripheral enhancement during the arterial
phase and brisk contrast agent washout during the late venous
phase (Fig. 11.61) [3]. CT demonstrates a well-circumscribed,
hypodense mass on unenhanced and contrast-enhanced images,
with varying degrees of heterogeneity [152, 156, 158, 159].
Speckled or amorphous calcification is often seen [149].
On MR imaging, hepatoblastoma may appear homoge-
neously hypointense on T1-weighted images and hyperin-
MR image depicts a solid mass in the right hepatic lobe with central
­nonenhancing foci consistent with necrosis. (c) Transverse grayscale
ultrasound image obtained after chemotherapy reveals decreased size of
the tumor and increased coarse calcifications (arrow) within the mass
11 Liver
Fig. 11.61 Hepatoblastoma in a 7-year-old male. (a) Transverse grayscale
ultrasound image of the liver demonstrates a heterogeneous mass (arrow) in
the left hepatic lobe compressing the gallbladder (asterisk). (b) CEUS arte-
rial phase image shows mildly increased contrast uptake by the mass com-
pared to the surrounding hepatic parenchyma. Several anechoic foci
(arrowheads) within the lesion represent tumor necrosis and/or hemor-
399
rhage. (c) CEUS portal venous phase image reveals early contrast washout
(arrowheads) from several sites within the mass. (d) Axial gadoxetate diso-
dium (Eovist®) contrast-enhanced, T1-weighted, fat-suppressed MR
image in the hepatobiliary phase demonstrates a heterogeneous, predomi-
nantly hypointense mass (arrow) in the left hepatic lobe. There is a subcap-
sular liver hematoma (asterisk) with a fluid-fluid level (arrowhead)
400
tense on T2-weighted images; mixed tumors demonstrate
heterogeneous signal intensity and enhancement [149, 152,
156, 158, 160]. Fibrotic septa are hypointense on both T1-
and T2-weighted images and enhance after contrast adminis-
tration. Hemorrhagic areas may appear hyperintense on
T1-weighted images. MR angiography is useful for the pre-
operative evaluation of the hepatic vasculature.
Surgical resection is the definitive treatment for hepato-
blastoma. Neoadjuvant chemotherapy is used to decrease
tumor bulk and treat pulmonary metastases prior to resec-
tion or transplant [149, 152, 159, 162]. Interventional
radiology procedure options, such as transarterial che-
moembolization (TACE), radiofrequency ablation, and
microwave ablation, are promising emerging techniques
[152, 163, 164].
Hepatocellular Carcinoma
Hepatocellular carcinoma (HCC) is a malignant tumor of
cells of hepatocyte differentiation and the second most com-
mon primary hepatic malignancy in children after hepato-
blastoma. Children who develop HCC are typically older,
between 6 and 10 years of age, and may or may not have
antecedent liver disease (Table 11.4) [165].
In parts of the world where HBV is endemic, perinatally
acquired HBV is a major risk factor, with a high prevalence of
HCC and a documented decline in incidence follow­ing mass
HBV immunization [55]. Elsewhere in the world, non­infectious
causes predominate, such as hereditary tyrosinemia, progres-
sive familial intrahepatic cholestasis, glycogen storage disorder
Fig. 11.62 Hepatocellular carcinoma in a 15-year-old female. (a)
Transverse grayscale ultrasound image shows multiple heterogeneously
hypoechoic masses in the liver (arrowheads). (b) Axial image from CT
J. K. Kwon et al.
type I, alpha-1-antitrypsin deficiency, Wilson disease, congeni-
tal portosystemic shunts, autoimmune hepatitis, and Alagille’s
syndrome, and are indications for routine imaging and AFP
surveillance [166].
Children usually present with an abdominal mass and
pain. Advanced disease may present with weight loss, fever,
or jaundice. Serum AFP levels are markedly elevated in most
patients [149].
HCC may present as a solitary, multinodular, or diffuse
infiltrative mass with a variable or mosaic appearance. On
ultrasound, HCC typically appears as a large, lobulated, het-
erogeneously hypoechoic mass [167]. Echogenic areas may
represent fat or acute hemorrhage, while hypoechoic or
anechoic areas may represent old hemorrhage or necrosis
(Fig. 11.62). Doppler evaluation can depict arterial flow
within the mass, and assess for tumor invasion into the portal
vein, hepatic veins, or IVC.
CEUS, CT, and MR imaging demonstrate arterial phase
hyperenhancement with washout in the portal venous and
delayed phases [168]. Sites of metastasis include lymph
nodes, lungs, brain, and bone. See Section “Hepatoblastoma”
for a brief discussion of the PRETEXT staging system for
children with a primary hepatic malignancy.
The long-term survival rate remains poor. Management
is targeted to complete surgical removal either by resec-
tion or liver transplantation [169]. Neoadjuvant chemo-
therapy and interventional radiology techniques are
employed to downstage the tumor and improve survival
[163, 164].
angiogram of the abdomen demonstrates prominent arterial enhance-
ment of the lesions (arrowheads)
11 Liver 401

Fig. 11.63 Fibrolamellar hepatocellular carcinoma in a 16-year-old liver. (b) Longitudinal color Doppler ultrasound image of the mass shows
male. (a) Transverse grayscale ultrasound image shows a large, exo- internal vascularity
phytic, heterogeneously hypoechoic mass (arrowheads) arising from the

 ibrolamellar Hepatocellular Carcinoma

F
The fibrolamellar variant of HCC (FLHCC) is a distinct, rare
variant of HCC with features that distinguish it from conven-
tional HCC. In the pediatric population, it is more commonly
seen in patients without underlying chronic liver disease
(Table 11.4) [150]. Compared with pediatric HCC, FLHCC
presents in older children (more than 12 years of age), is less
often ­multifocal, and metastatic disease is often present, par-
ticularly to the lungs [167, 170]. Nodal metastases may be
seen in the porta hepatis, retroperitoneum, pelvis, and medias-
tinum. Unlike conventional HCC, AFP levels are usually
normal.
Histologically, abundant collagenous fibrous sheets con-
taining cords of tumor cells are arranged in a parallel configu-
ration, hence the term “fibrolamellar” [171]. Pathologically, a
central stellate scar is seen, another feature not typical for
conventional HCC. Patients frequently present with abdomi-
nal pain, hepatomegaly, or abdominal mass. Malaise and
weight loss may occur. Fig. 11.64 Fibrolamellar hepatocellular carcinoma in a 6-year-old
male. Transverse grayscale ultrasound image of the right hepatic lobe
On ultrasound, FLHCC is typically a solitary well-­ demonstrates a well-defined, heterogeneous mass (arrow). An echo-
defined, lobulated mass that is heterogeneously isoechoic or genic central scar aids in diagnosis when present, but may not be seen,
hyperechoic (Figs. 11.63 and 11.64). Echogenic strands may as in this case
be identified, representing the central scar with or without
shadowing calcifications [167]. On MR imaging, FLHCC typically demonstrates T1-
On multi-phase CT, FLHCC may present as a well-­ weighted hypointensity and T2-weighted hyperintensity. The
defined hypodense mass on unenhanced imaging, with het- central scar usually appears hypointense on T1- and
erogeneous arterial hyperenhancement. Portal venous and T2-weighted images, which helps to distinguish FLHCC
delayed phases show variable degrees of enhancement and from FNH, which usually has a T2-hyperintense scar [171].
washout. A central stellate scar is often present, though not Surgical resection is the mainstay of treatment. Tumor
pathognomonic for FLHCC, as both FNH and FLHCC may recurrence is common. The results of chemotherapy and radia-
contain a central scar with a variable degree and timing of tion have been poor [167]. TACE and radiofrequency ablation
enhancement [149, 171]. However, when present, calcifica- have been used with positive outcomes in limited reports.
tion within a thick, greater than 2 cm-wide central scar is a Radioembolization with Yttrium-90 may be used to down-
helpful distinguishing feature of FLHCC [172]. stage initially unresectable masses [173].
402
Rare Primary Tumors
Undifferentiated embryonal sarcoma (UES) is a rare
malignant mesenchymal tumor, most commonly found in
patients between 6 and 10 years of age (Table 11.4) [167,
174]. UES consists of undifferentiated cells associated
with a myxoid stroma. Patients present with an abdominal
mass and pain. Tumor rupture has been reported at pre-
sentation, with associated hemoperitoneum and peritoneal
seeding [175].
The classic imaging description is of a large, solitary
mass with a predominantly solid, hyperechoic appearance
on ultrasound and water density/intensity on CT/MR imag-
ing, due to the high water content of the myxoid stroma
Fig. 11.65 Undifferentiated embryonal sarcoma of the liver in a 6-year-old
female. (a) Reference sagittal grayscale ultrasound image (left panel) and
CEUS image (right panel) show a well-circumscribed, heterogeneously
hypoechoic mass (arrows) with enhancing solid components. The mass
J. K. Kwon et al.
[137]. However, predominantly anechoic masses have also
been described [176]. Enhancing soft tissue components
may be present at the periphery or in the form of septa
(Fig. 11.65). Treatment consists of neoadjuvant chemother-
apy with resection or liver transplantation.
Epithelioid hemangioendothelioma (EHE) and angiosar-
coma are malignant vascular tumors that may occur in the liver
and can present in childhood. EHE is a low-to-­intermediate-­
grade vascular sarcoma, while angiosarcoma is a rare, aggres-
sive malignant tumor [138]. For both tumors, multifocal
involvement is typical, and the imaging appearance can be quite
variable. The ultrasound appearance includes multiple nodules,
a large mass, and a diffuse heterogeneous echotexture.
demonstrates internal, anechoic spaces (arrowheads) consistent with necro-
sis and/or hemorrhage. (b) Coronal contrast-enhanced, T1-weighted, fat-
suppressed MR image shows a large, multiloculated, hypointense and
heterogeneously enhancing mass (arrows) centered in the right hepatic lobe
11 Liver
Masses are typically hypodense on CT. Variable patterns
of arterial enhancement have been described, including rim-
like and heterogeneous [138]. Contrast-enhanced CT dem-
onstrates progressive enhancement on portal and delayed
phases [175]. When subcapsular in location, capsular retrac-
tion is a distinctive feature of EHE [138].
There is no standardized treatment for EHE. Recently, the
mammalian target of rapamycin inhibitor, sirolimus, has
been suggested as a possible therapeutic option in children
[177]. With respect to hepatic angiosarcoma, the prognosis is
very poor regardless of the treatment. The mean survival
noted in the literature is 10 months to 2 years [178].
Rhabdoid tumors are uncommon and highly aggressive
tumors of early childhood, most commonly occurring in the
kidney and central nervous system [179, 180]. Primary hepatic
rhabdoid tumors are extremely rare. On histology, cells resem-
ble rhabdomyoblasts. Patients are often asymptomatic, and
tumors are large upon presentation. The imaging appearance is
nonspecific. Masses are predominantly solid and heteroge-
neous, and cystic components and calcifications may be present
[180, 181].
Because of the rarity of this disease, there is no standard ther-
apeutic algorithm. In a recent retrospective study of six pediatric
patients treated at a single center, treatment for the three survi-
vors consisted of intensive multiagent chemotherapy, early surgi-
cal resection of the tumor and radiotherapy in one child [182].
Embryonal rhabdomyosarcoma of the biliary tree is a
highly malignant tumor which presents exclusively in chil-
dren mainly under the age of 5 years [149]. It commonly
arises in the extrahepatic biliary tree but may grow into the
intrahepatic biliary tree and invade the liver [138, 149].
Patients may present with signs and symptoms that mimic
viral hepatitis, including jaundice, abdominal distension, and
fever. Ultrasound findings include biliary ductal dilation
with an intraluminal mass. The portal vein is often displaced,
Fig. 11.66 Metastatic rhabdomyosarcoma in an 11-year-old female. (a) Transverse grayscale ultrasound image shows a hyperechoic intrahepatic
mass (arrowheads) with a hypoechoic rim. (b) Transverse color Doppler ultrasound image reveals a small amount of blood flow within the lesion
403
without thrombosis [138]. Polypoid or grape-like projections
are common. The tumor has a variable imaging appearance,
ranging from solid to cystic components [149].
Metastases
Metastatic disease is the most frequent malignancy of the
liver [183]. The liver is a common site of metastatic disease,
owing to its dual blood supply and cellular and molecular
microenvironment that permits cancer cell growth [184].
The most common site of origin of liver metastases in
children with solid tumors is neuroblastoma followed by
Wilms’ tumor. Other solid malignant tumors that can metas-
tasize to the liver include germ cell tumor, gastrointestinal
stromal tumor, osteosarcoma, desmoplastic small round cell
tumor, and neuroendocrine tumor [185].
Metastatic disease is often associated with a poor progno-
sis. A unique exception is neuroblastoma stage 4S (defined
as localized primary tumor with dissemination limited to
skin, liver, and/or bone marrow involvement less than 10% in
infants younger than 12 months of age), which frequently
regresses spontaneously without requiring therapy.
Liver metastases are usually asymptomatic and detected
during staging workup of patients with malignancy.
Metastases are typically multifocal and involve both hepatic
lobes. The imaging appearance of metastatic disease is quite
variable and is dependent upon the nature of the primary
tumor and the presence and degree of underlying hepatic ste-
atosis, particularly for ultrasound and CT imaging [183].
Lesions may be solid or cystic, hypoechoic and well
defined, hyperechoic, or targetoid due to central hemorrhage
(Fig. 11.66) [183].
CEUS increases the accuracy of detection of metastatic
disease compared to ultrasound performed without contrast
administration, and has a similar sensitivity to CT [14, 186].
In particular, washout of contrast material in the portal
404
venous phase is highly associated with malignancy, similar
to CT and MR imaging (Fig. 11.67) [9, 183].
Treatment of metastatic liver disease will vary according
to the underlying primary tumor, but may involve surgery,
radiation, and/or chemotherapy.
Lymphoma
Primary hepatic lymphoma (PHL) is defined as lymphoma
confined to the liver and perihepatic nodal sites. It is rare,
Fig. 11.67 Metastatic renal cell carcinoma in a 21-year-old female. (a)
Oblique longitudinal grayscale ultrasound image shows two echogenic
lesions (arrowheads) in the right hepatic lobe. (b) Oblique longitudinal
CEUS image (right panel) reveals prominent arterial enhancement of
J. K. Kwon et al.
accounting for less than 1% of all non-Hodgkin lym-
phoma (NHL) [187]. PHL is associated with HBV, HCV,
Epstein–Barr virus (EBV), and human immunodeficiency
virus (HIV) [188]. In contrast, the vast majority of lym-
phomatous involvement of the liver is due to secondary
involvement by non-Hodgkin lymphoma [187, 188].
Patients may present with right upper quadrant pain or
jaundice, as well as systemic symptoms of fever and
weight loss.
the two masses (arrows). Reference grayscale image in the left panel
(c) Oblique longitudinal CEUS image in portal venous phase (right
panel) demonstrates rapid contrast washout (arrow) from the larger
lesion. Reference grayscale image in the left panel
11 Liver
PHL usually presents as a solitary, large liver mass that
enhances heterogeneously (Fig. 11.68) [188]. Secondary
hepatic lymphoma (SHL) most commonly presents as homo-
geneous, multifocal lesions or diffuse infiltration without a
dominant mass, often with splenic lesions. A miliary pattern
of numerous small hypoenhancing nodules throughout the
liver is a less common presentation of SHL [188, 189].
On ultrasound, lymphomatous nodules are usually hypoechoic,
without posterior acoustic enhancement (Fig. 11.69) [188]. Some
nodules may demonstrate a target-like appearance, with a hyper-
echoic center and a peripheral hypoechoic rim [188, 189].
CEUS reveals portal and late phase washout, similar to other
hepatic malignancies [190].
On CT and MR imaging, lesions demonstrate hypoen-
hancement compared with background liver parenchyma
[188]. On MR imaging, the lymphomatous nodules tend to
be hypointense or isointense on T1-weighted images and
moderately hyperintense on T2-weighted images with
restricted diffusion on diffusion-weighted imaging [188].
Some lesions may demonstrate a target appearance with cen-
tral T2-weighted hyperintensity and a peripheral hypoin-
Fig. 11.68 Epstein–Barr virus (EBV)-associated lymphoproliferative
disease in a 12-year-­old female. (a) Transverse grayscale ultrasound
image of the left hepatic lobe shows a round, well-circumscribed, het-
a b
Fig. 11.69 Hepatic involvement in a 2-year-old female with immu-
nodeficiency and diffuse large B-cell lymphoma. (a) Sagittal gray-
scale ultrasound image demonstrates a hypoechoic, geographic region
405
tense rim [188]. Fluorodeoxyglucose (FDG)-positron
emission tomography (PET)/CT typically demonstrates avid
hypermetabolic activity and is routinely used for staging and
evaluation of treatment response [188].
Hepatic lymphoma is typically treated with a chemother-
apy regimen based on the histologic subtype.
 osttransplant Lymphoproliferative Disorder
P
Posttransplant lymphoproliferative disorder (PTLD) repre-
sents a continuum of disease complications ranging from
benign lymphoid hyperplasia to high-grade malignant lym-
phoma [191]. It occurs in the setting of solid organ or hema-
topoietic stem cell transplantation and immunosuppression.
Most cases develop within the first year following transplan-
tation when immunosuppression rates are highest [192, 193].
A second peak in incidence occurs 4–5 years following
transplantation [192, 193].
95% of PTLD cases are associated with EBV infection,
which causes the proliferation of B-cells without the normal
T-cell response due to immunosuppression [194].
erogeneous, solid mass with a thin hypoechoic rim. (b) Transverse
color Doppler ultrasound image shows peripheral hyperemia with sev-
eral enlarged vessels extending into the mass
(arrowhead) along the periphery of the right hepatic lobe. (b) Sagittal
color Doppler ultrasound image shows increased vascularity within
the hypoechoic focus
406
Children are at higher risk of developing PTLD than
adults, likely related to lower rates of EBV seropositivity in
children prior to transplant, with seroconversion occurring
following exposure to EBV-seropositive donor organs [195].
PTLD often manifests within or near the site of the allograft,
in nodal or extra-nodal locations. The liver is the most com-
monly involved abdominal organ [188]. Clinical features are
based on location and pattern of organ involvement, and, when
the liver is involved, may include fever, right upper quadrant
pain, jaundice, and abnormal liver enzymes [191].
a b
Fig. 11.70 Posttransplant lymphoproliferative disorder in a 10-year-old
female with a liver homograft. Transverse (a) and sagittal (b) grayscale
ultrasound images show a well-delineated, heterogeneously hypoechoic
J. K. Kwon et al.
Several patterns of solid organ involvement by PTLD
may occur: The obstructive pattern of involvement of the
liver appears as a mass in the region of the porta hepatis
causing biliary ductal dilatation with or without vascular
encasement and often without vessel occlusion [188]. The
parenchymal (scattered) pattern appears as multiple scat-
tered lesions throughout the liver parenchyma. PTLD in the
liver can also manifest as one or more discrete masses
(Fig. 11.70). Lastly, the infiltrative pattern of involvement
appears as a mass extending into regional structures such as
mass with a central echogenic focus in the left hepatic lobe. (c) Sagittal
color Doppler ultrasound image shows no internal vascularity
11 Liver 407

the abdominal wall or adjacent organs, causing soft tissue
edema and obscuration of fat planes [191].
On ultrasound, PTLD lesions are hypoechoic. On CT,
PTLD lesions appear hypodense. MR imaging shows low or
intermediate signal intensity abnormality on T1- and
T2-weighted imaging, although lesions can occasionally
appear hyperintense on T2-weighted imaging. Following
contrast administration, lesions demonstrate hypoenhance-
ment. They appear FDG-avid on PET [191].
Treatment approaches include reduction of immunosup-
pressive therapy, immunotherapy with the CD20 monoclonal
antibody (rituximab), antiviral therapy, and/or chemother-
apy. EBV-specific cytotoxic T lymphocytes are of limited
availability but have been used with success [188, 196].
Leukemia
Acute leukemia accounts for approximately 30% of all child-
hood malignancies and is the most common cancer in chil-
dren [197]. Leukemic infiltrates may involve the liver,
particularly along the portal tracts [198]. Myeloid sarcoma
(synonymous with chloroma or granulocytic sarcoma) is a
rare extramedullary mass of myeloid precursor cells that usu-
ally manifests during remission or disease relapse of acute
myeloid leukemia (AML) [199]. Over half of children with
leukemia have a palpable liver, palpable spleen, pallor, fever,
or bruising on diagnosis [197].
The ultrasound appearance of leukemic infiltration of the
liver includes a coarsened echotexture and hepatomegaly. The
imaging features of hepatic myeloid sarcoma are nonspecific
and are similar to those of hepatic lymphoma. The diagnosis of
myeloid sarcoma can be suggested when there is a history of
AML [188].
Similar to hepatic lymphoma, myeloid sarcoma can appear
as hypoechoic lesions on ultrasound, with hypoenhancement
or washout following intravenous contrast administration by
CEUS, CT, or MR imaging (Fig. 11.71). However, compared
with hepatic lymphoma, discrete masses of myeloid sarcoma
are typically more heterogeneously enhancing and less well-
circumscribed [185].
Chemotherapy is used for the treatment of leukemia, with
the possible addition of localized radiation therapy for
myeloid sarcoma.

a b

Fig. 11.71 Hepatic leukemia in a 10-month-old male. (a) Sagittal grayscale ultrasound image shows a subcapsular, heterogeneously hypoechoic
mass (arrowhead). (b) Sagittal color Doppler ultrasound image reveals minimal internal vascularity
408 J. K. Kwon et al.

Liver Transplantation common [203]. Over the years, improved survival and qual-
ity of life of hepatic graft recipients has been possible thanks
Introduction to advancements in surgical techniques and immunosuppres-
sive therapies, optimization of preoperative, operative, and
Advancements in surgical technique and immunosuppres- postoperative care, and timely consideration of the need for
sive therapy have allowed liver transplantation to become a re-transplantation [202, 203].
suitable treatment option for pediatric patients with end-­
stage liver disease. Ultrasound with Doppler assessment
serves as the main imaging modality in the evaluation of Table 11.7 Vascular complications of liver transplantation
liver transplants. A thorough understanding of the surgical Types Grayscale and Doppler findings
approach and the specific anastomotic anatomy is vital in Hepatic artery
the interpretation of postoperative imaging studies. In this Thrombosis Absent flow or non-occlusive filling defect with
section, a review is provided of the various transplantation slow flow (< 50 cm/s)
Low-resistance distal arterial waveforms
options for children, pre- and postoperative imaging consid-
Stenosis High-resistance proximal arterial waveforms
erations of the donor and recipient, postoperative monitor- Elevated peak velocity (3–4x) at anastomosis
ing of the graft, as well as the most important vascular and Distal low-resistance waveforms with dampening
nonvascular postoperative complications (Tables 11.6, 11.7, Pseudoaneurysm Rounded structure with turbulent bidirectional
and 11.8). flow, “yin-yang” sign, to-and-fro flow
Portal vein
Liver transplantation is an effective treatment option for
Thrombosis Absent flow with or without a filling defect
children with end-stage liver disease [200, 201]. According Stenosis Caliber < 3–3.5 mm
to the Organ Procurement and Transplantation Network > 50% stenotic ratio
database, 17,560 pediatric liver transplants have been per- > 3–4:1 velocity ratio
formed in the United States since 1988, and a total of 551 Hepatic veins
children received hepatic grafts in 2019 [202]. Biliary atresia Stenosis Monophasic waveforms (although nonspecific)
Inferior vena cava
is the most common etiology of pediatric end-stage liver dis-
Thrombosis Intraluminal filling defect with absent flow
ease, followed by metabolic disorders and acute hepatic Stenosis Narrowed caliber
necrosis. Decreased waveform phasicity
During organ procurement, patient weight and size are the
major determinants of the type of graft that will be trans-
Table 11.8 Nonvascular complications of liver transplantation
planted. In children, partial liver transplantation (living-­
Types Grayscale and Doppler findings
related donor or split cadaveric liver graft) is the most
Parenchymal
Rejection Nonspecific heterogeneity
Biliary
Table 11.6 Expected transient postoperative liver transplant Leak Free simple fluid, can develop into an
ultrasound findings anechoic fluid collection (biloma)
Stricture Focal narrowing with upstream dilation
Location and type Findings
Fluid collections
Grayscale findings Extrahepatic Different shapes, no internal Doppler
Intrahepatic Parenchymal edema at hepatectomy flow
margin Contents may vary from anechoic to
“Starry sky” parenchymal hyperechoic
appearance Typically simple fluid for biloma
Periportal edema Slightly heterogeneous contents for
Extrahepatic Ascites, small seroma/hematoma
Fluid collections, small Markedly heterogeneous with thick
Right pleural effusion, small margins and/or air foci in abscess
Regional lymph nodes Intrahepatic Similar to above, except for focal
Color and spectral Doppler findings infarction/necrosis which appears as
All graft vasculature Slight narrowing at surgical round/oval area with no internal flow,
anastomoses can contain air
Hepatic artery Elevated hepatic artery RI up to 0.95 Other
(<24 hours) Posttransplant Lymphadenopathy (porta hepatis mass)
Tardus-parvus waveform (<72 hours) lymphoproliferative Bowel wall mass or hepatic lesions
Main portal vein Turbulence disorder (extranodal involvement, classically
Hepatic veins Biphasic and dampened waveforms involving the GI tract)
RI, Resistive index GI, Gastrointestinal
11 Liver 409

Surgical Technique
Three surgical options are currently used for pediatric liver
transplantation: whole liver graft (cadaveric), split liver
graft (cadaveric), and living-related donor graft (Fig. 11.72).
While small children weighing less than 20 kg are suit-
able recipients for a left lateral segment graft, larger children
can receive a full left lobe graft [204]. Occasionally, a left
lateral segment hepatic graft from an adult donor may need
to be reduced to a single segment or subsegment in order to
maintain an adequate graft-to-recipient weight ratio (<5%)
and thereby lessen the risk of complications. For adult-sized
pediatric patients, a whole liver, right lobe or full left lobe
graft are suitable options.
Liver transplant procedures in the pediatric population
are challenging due to technical and immunologic consider-

a Whole liver b Split liver c Living donor- right lobe liver

Conventional technique transplantation

II + III
II + III IV
VIII VII VIII
VII IV
V
V VI
VI

Piggyback technique Living donor- left lobe liver

transplantation

VIII IV IV II + III
VII II + III VII VIII
V V
VI
VI

Adult Pediatric

Living donor- left lateral segment

transplantation

VIII II + III
VII IV

V
VI

Fig. 11.72 Diagram of pediatric liver transplant surgical techniques.
(a) Whole liver transplant from a cadaveric donor. Usual end-to-end
anastomoses for all structures in a conventional technique, and with
end-to-end anastomoses for all structures except for the IVC in the
“piggyback” technique. Note that with a “piggyback” technique, the
donor IVC is anastomosed to the recipient IVC or hepatic veins in a
“piggyback” configuration that may include a blind-ending stump of
distal donor IVC. (b) Split liver transplant from a cadaveric donor. The
donor liver is cleaved along the left-sided margin of the medial segment
of the left hepatic lobe (Couinaud segment IV), resulting in an extended
right hepatic lobe graft (Couinaud segments I – not shown, V–VIII and
IV) suitable for an adult patient or an adult-sized pediatric recipient;
and a left lateral segment graft (Couinaud segments II and III) appropri-
ate for a small pediatric recipient. The extended right hepatic lobe trans-
plant is completed with end-­to-­end anastomoses for all structures. The
left lateral segment graft can be performed with end-to-end anastomo-
ses for the left-sided inflow and outflow structures and a hepaticojeju-
nostomy for the left biliary duct. (c) Living donor liver transplant.
Depending on the chosen hepatectomy plane, grafts can be right lobe
(Couinaud segments V, VI, VII, and VIII), complete left lobe (Couinaud
segments II, III, and IV) or left lateral segment (Couinaud segments II
and III). Anastomotic anatomy is similar to the techniques described in
(a) and (b)
410
ations that depend on the condition of the recipient [200].
The risk of technical complications is higher for pediatric
grafts related to small recipient size, especially the risk of
hepatic artery thrombosis [200, 205].
It is important to be aware of the anatomical details of
each patient’s graft prior to image acquisition, particularly
the sites of vascular anastomosis [206]. Occasionally, arterial
or venous interposition (jump) grafts or conduits are placed
during the transplantation procedure and two different anas-
tomoses will be present [203].
Whole Liver Transplantation
Whole liver grafts are transplanted into adult-sized pediatric
patients (Fig. 11.73). Surgical anastomoses between the donor
and the recipient are typically end-to-end for the portal vein
and biliary duct, and end-to-end or end-to-side for the hepatic
artery [203, 207]. A choledochojejunostomy is performed in
cases where the biliary duct is small and not suitable for end-
to-end anastomosis [207]. For grafts that contain the suprahe-
patic, intrahepatic, and infrahepatic portions of the IVC, the
recipient’s IVC is resected above the level of the renal veins
and replaced by the donor IVC. This requires anastomoses at
the infrahepatic and suprahepatic portions of the IVC [203].
 iving-Related Donor and Split Liver Grafts
L while the recipient IVC is left intact. The graft may contain
Living-related donor and split cadaveric liver transplants
are most commonly performed in children as the availabil-
a b
e f g h
Fig. 11.73 Normal whole liver transplant in a 16-year-old female.
Transverse (a, b) and sagittal (c, d) grayscale ultrasound images dem-
onstrate normal hepatic echotexture. Transverse color and spectral
Doppler ultrasound images show normal flow in the main portal vein
J. K. Kwon et al.
ity of pediatric whole liver grafts is limited [200, 203].
These reduced-size liver transplants have significantly
decreased the mortality of children with end-stage liver dis-
ease [206].
The donor liver is divided into right and left segments
using Couinaud segment IV as the cleavage plane [208]. This
results in a donor graft comprising Couinaud segments II and
III (i.e., a left lateral segment graft) (Fig. 11.74), or Couinaud
segments II, III, and IV (i.e., a complete left hepatic lobe
graft). These reduced-size livers can be used in children who
are smaller than the donor.
With split cadaveric transplants, grafts consisting of
Couinaud segment I and segments V–VIII (i.e., a right lobe
graft) or Couinaud segments I, IV, and V–VIII (i.e., an
extended right lobe graft) can also be used (Fig. 11.72).
Since left hepatic grafts contain left-sided inflow and out-
flow structures, the preferred surgical techniques include a
hepaticojejunostomy for the left bile duct, end-to-end anas-
tomosis for the portal vein, end-to-side anastomosis for the
hepatic artery, and a side-to-side “piggyback” anastomosis
for the IVC-hepatic vein anastomosis.
The piggyback technique involves fashioning of the recip-
ient hepatic venous confluence into a cuff that is then anasto-
mosed to the donor hepatic veins or suprahepatic vena cava
the donor intrahepatic IVC which will terminate distally as a
blind-ended stump [203, 207].
c d
i
proximal (e), at (f), and distal (g) to the surgical anastomosis. Transverse
color and spectral Doppler ultrasound images show normal flow in the
main hepatic artery (h) and left hepatic vein (i)
11 Liver
a b
d e
Fig. 11.74 Normal left lateral segment graft (Couinaud segments II
and III) in a 25-month-old male. Transverse grayscale ultrasound
images (a–c) demonstrate a normal appearance of the hepatic paren-
 reoperative and Postoperative Imaging
P adults [203, 209]. Serial Doppler ultrasound examinations
Considerations
Pretransplant evaluation of potential donors and recipients is
obtained with Doppler ultrasound. Liver and biliary tree
anatomy are assessed as well as vascular patency. The hepatic
parenchyma is examined for focal lesions, fibrosis, and ste-
atosis. The presence of portosystemic collateral vessels is
also documented [207].
Ultrasound is the main imaging modality used to diagnose
postoperative complications (Tables 11.7 and 11.8) [207].
The goal is to make an early and accurate diagnosis in order
to initiate timely management that can prevent irreversible
changes and thereby improve patient outcome.
Transverse and longitudinal grayscale images of the entire
liver transplant permit evaluation of the parenchyma, the
intrahepatic bile ducts, and identification of fluid collections.
Color and spectral Doppler are used to evaluate flow in the
hepatic arteries, portal veins, hepatic veins, IVC, and patency
of the vascular anastomoses.
In general, vascular and biliary complications are more
common in pediatric liver transplant recipients compared to
411
c
f
chyma. Color and spectral Doppler ultrasound images show a normal
hepatic vein (d), hepatic artery (e), and portal vein anastomosis (f)
obtained shortly after transplantation have allowed for the
early diagnosis and treatment of complications and have
been associated with improved overall outcomes.
CEUS is a relatively new ultrasound technique that can
provide additional information regarding parenchymal per-
fusion and vasculature patency of the graft, although to date
its use in the setting of pediatric liver transplantation has
been limited [210–212].
Ultrasound two-dimensional shear-­wave elastography is
a useful noninvasive technique for the assessment of postop-
erative liver transplant damage [213]. Liver stiffness and
shear-wave dispersion slope values are higher in patients
with biopsy-proven graft damage compared to recipients
without damage. It has been suggested that shear-wave dis-
persion slope values may perform better than liver stiffness
in the detection of hepatic graft damage.
Pre- and postoperative CT/CT angiography and MR/MR
angiography/MR cholangiopancreatography (MRCP) are
reserved for instances where ultrasound examination is of
limited value or has revealed abnormalities that require fur-
ther clarification or confirmation [207, 214].
412
Normal Liver Transplant Ultrasound
Immediate ultrasound examination is performed to confirm
patency of the anastomosed vessels, either in the surgical suite
or at the bedside shortly after surgery, and then daily for approx-
imately 3 days [203]. A grayscale evaluation of the hepatic
parenchyma and biliary tree is performed along with a survey of
the perihepatic spaces and the pelvis for fluid collections. Color
and pulsed Doppler evaluation of the hepatic vasculature is per-
formed with an assessment of the patency of the anastomoses of
the hepatic artery, portal vein, hepatic veins, and IVC.
Periportal edema, small-volume ascites, small fluid
collections, and a small right pleural effusion are transient
abnormalities that are frequently observed following liver
transplantation [207, 214]. Other common findings
include a slight narrowing at the anastomotic sites, a
“starry sky” parenchymal appearance, and the presence of
reactive lymph nodes at the porta hepatis and portocaval
region that usually resolve after few weeks [207].
Postoperative Doppler findings may include an elevated
hepatic artery resistive index in the first 24 hours, and a tar-
a b
c d
Fig. 11.75 Late acute rejection in an 8-year-old male with a split liver
transplant. Transverse (a) and sagittal (b) grayscale ultrasound images
reveal diffusely echogenic and heterogeneous hepatic parenchyma. (c)
Transverse color and spectral Doppler ultrasound image shows abnor-
J. K. Kwon et al.
dus-parvus waveform in the hepatic artery(ies) in the first
72 hours [203, 214]. Turbulence in the main portal vein in
addition to biphasic and dampened hepatic venous wave-
forms are also acceptable when encountered during the early
postoperative period (Table 11.6) [203, 207]. Persistence of
these findings on follow-up Doppler ultrasound evaluation is
generally indicative of an underlying structural abnormality
[215].
Rejection
Rejection usually develops in the first year after liver trans-
plantation and is the primary cause of graft failure [207]. On
ultrasound, acute rejection can manifest with nonspecific
heterogeneity of the hepatic parenchyma (Figs. 11.75, 11.76,
and 11.77) [216]. The main role of imaging in the setting of
graft rejection is to detect other abnormalities that may have
a similar clinical presentation [216, 217]. Optimization of
immunosuppression can reduce the rates of acute rejection
[200, 201].
mally pulsatile flow in the hepatic artery with reversal of end-diastolic
flow. (d) Sagittal color and spectral Doppler ultrasound image of the
portal vein demonstrates reversed flow direction. The Doppler findings
are nonspecific and reflect increased intrahepatic vascular resistance
11 Liver 413

a b c

Fig. 11.76 Chronic rejection in a 21-year-old female with a whole liver strates dampened flow. (c) Transverse color and spectral Doppler ultra-
transplant. (a) Transverse grayscale ultrasound image demonstrates dif- sound image of the left hepatic vein reveals loss of the usual tetraphasic
fusely coarse and heterogeneous hepatic parenchyma. (b) Sagittal color waveform
and spectral Doppler ultrasound image of the main portal vein demon-

a b

Fig. 11.77 Chronic rejection in a 5-year-old male. (a) Longitudinal dimensional shear-wave elastography image of the right hepatic lobe
grayscale ultrasound image reveals a diffusely coarse and heteroge- demonstrates abnormally elevated shear-wave values which are a sur-
neous echotexture of the hepatic parenchyma. (b) Transverse two-­ rogate marker for hepatic fibrosis

Biliary Complications Bile Leak

Biliary complications are the second most common cause of
hepatic graft dysfunction after rejection, occurring in approx-
imately 10–30% of pediatric liver transplants and usually in
recipients of reduced-sized grafts. The most frequent com-
plications include bile leak and biliary stricture which are
associated with substantial morbidity and mortality
(Table 11.8) [209, 218]. Other complications include biliary
sludge, stones, sphincter of Oddi dysfunction, and cystic
duct remnant mucocele [214].
Bile leaks usually develop at the site of anastomosis. Their
incidence is influenced by graft type, donor characteristics,
and biliary reconstruction technique [209, 218]. Ultrasound
will show an anechoic fluid collection either within the region
of the falciform ligament, in a subhepatic location, or as free
fluid within the peritoneal cavity (Fig. 11.78). Extra-­intestinal
radiotracer accumulation on a hepatobiliary iminodiacetic
acid (HIDA) scan can confirm the diagnosis [214]. Treatment
consists of biliary diversion, either by endoscopic biliary stent-
ing or by placement of a percutaneous drain. Surgical revision
of the anastomosis may sometimes be necessary [209].
414 J. K. Kwon et al.

a b c

Fig. 11.78 Bile leak in a 10-month-old male with a split left lateral anechoic subcapsular fluid collection (white arrows) tracking along the
segment hepatic homograft. Transverse (a, b) and sagittal (c) grayscale anterior surface of the liver. At surgery there were two small bile leaks
ultrasound images reveal a mildly heterogeneous, predominantly along the cut edge of the liver

a b

c d e f

Fig. 11.79 Stenosis at the biliary-enteric anastomosis in an 11-year-­ Cholangiographic images depict intrahepatic biliary ductal dilation
old female with a left hepatic lobe transplant (Couinaud segments II, with severe narrowing at the biliary-enteric anastomosis (white
III, IV). Transverse grayscale (a) and color Doppler (b) ultrasound arrows). Ductoplasty was subsequently performed (e) with modest
images reveal intrahepatic biliary ductal dilation (cursors). (c, d) improvement in the degree of ductal dilation (f)

Biliary Stricture for the early detection of stricture(s), MRCP or percutaneous

The presence of biliary stricture(s) or bile duct dilation raises transhepatic cholangiography should be obtained in patients in
concern for hepatic artery thrombosis or stenosis [203, 209]. whom biliary obstruction is suspected [203]. Strictures can be
Anastomotic strictures can also occur as a result of focal isch- treated with percutaneous or endoscopic balloon dilation and
emia with scarring. As ultrasound is not a sensitive examination stent placement or ductoplasty (Figs. 11.79 and 11.80) [219].
11 Liver 415

a b

Fig. 11.80 Biliary stricture in a 2-year-old female with a liver transplant. (a) Transverse color Doppler ultrasound image shows marked dilation
(arrows) of the central bile ducts concerning for a distal stricture. (b) Cholangiogram confirms a high-grade stricture (arrow) at the anastomotic site

Vascular Complications patients with a history of prior hepatic malignancy and in

Vascular complications can affect any of the hepatic vessels
(Table 11.7) [205]. The inherently small caliber vasculature
of pediatric liver transplant recipients is associated with a
higher risk of thrombosis and stenosis when compared to
adult liver transplant recipients, and can lead to hepatic graft
dysfunction and loss.
Hepatic Artery Thrombosis
In normal individuals, the role of the hepatic artery in sup-
plying blood flow to the liver is secondary to that of the
­portal vein. In the setting of liver transplantation, this role is
reversed, and the liver parenchyma and bile ducts are highly
dependent on the hepatic artery in order to maintain viability,
particularly in the immediate postoperative period.
Hepatic artery thrombosis is the most common vascular
complication of liver transplantation, and the most frequent
cause of graft loss in children. The incidence in the early
postoperative period ranges from approximately 3% to 9%
[205]. Hepatic artery thrombosis usually occurs at the donor-
recipient anastomosis. The risk of thrombosis is higher in
those with a high donor/recipient weight ratio [214].
Doppler ultrasound evaluation allows for early detection
and early treatment that can lead to improved hepatic graft
survival and overall outcomes [203]. Characteristic ultra-
sound findings include the absence of arterial Doppler flow
or the presence of a partially occlusive thrombus with
abnormal downstream arterial tardus-parvus waveforms
[203, 220]. CT angiography or CEUS can serve as problem-
solving imaging modalities to confirm this serious compli-
cation (Fig. 11.81) [207, 210–212, 214]. Treatment consists
of immediate surgical thrombectomy or thrombolysis to
preserve the graft.
 epatic Artery Stenosis
H
The diagnosis of hepatic artery stenosis can be challenging
because normal donor-to-recipient hepatic arterial size dis-
crepancy can potentially mimic stenosis [203]. Stenosis usu-
ally occurs at the surgical anastomosis. Disordered flow with
postanastomotic dilation can sometimes be seen in cases of
normal size discrepancy, the peak velocity at the surgical
anastomosis should be minimally increased, and the down-
416 J. K. Kwon et al.

a b

Fig. 11.81 Hepatic artery thrombosis in a 4-month-old male with a
deceased-donor liver transplant and a jump graft extending from the
infra-renal aorta to the donor hepatic artery. Transverse color and spec-
tral Doppler ultrasound images (a, b) reveal no appreciable flow within
the hepatic artery. Transverse color and spectral Doppler ultrasound
image (c) demonstrates arterial flow (arrow) in the proximal portion of
the jump graft. Axial arterial phase contrast-enhanced CT images (d, e)
show delayed hepatic parenchymal enhancement. There is an abrupt
cutoff (arrow) in the proximal jump graft (d). (e) Multifocal splenic
parenchymal infarcts (arrowheads) are also seen
11 Liver 417

stream arterial waveforms are typically normal. In contrast, the stenotic portion of the artery approximately 3–4 times
Doppler findings of hepatic artery stenosis include proximal that in the prestenotic portion, and downstream tardus-par-
high-resistance arterial waveforms, elevated peak velocity in vus waveforms (Figs. 11.82 and 11.83) [203, 220].

a b

Fig. 11.82 Hepatic artery stenosis in a 5-year-old female with a liver color Doppler ultrasound image with spectral analysis of the left hepatic
transplant. (a) Transverse color Doppler ultrasound image with spectral artery reveals a tardus-parvus waveform. (c) Angiographic image dem-
analysis at the surgical anastomosis demonstrates elevated peak veloc- onstrates marked focal hepatic artery narrowing (arrow) consistent with
ity in the proper hepatic artery of more than 300 cm/s. (b) Transverse stenosis

a b c

Fig. 11.83 Hepatic artery anastomotic stenosis in a 7-year-old male velocity and color aliasing are observed at the hepatic artery anastomo-
with a whole liver transplant. (a) Transverse color and spectral Doppler sis. (c) The post-anastomotic hepatic artery segment demonstrates a
ultrasound image show a normal peak systolic velocity in the preanas- tardus-parvus waveform with a low peak systolic velocity
tomotic segment of the hepatic artery. (b) An elevated peak systolic
418
 epatic Artery Pseudoaneurysm
H Portal Vein Thrombosis
Hepatic artery pseudoaneurysm is an infrequent complica-
tion of pediatric liver transplantation. It is usually related to
prior angioplasty and generally occurs at the surgical anas-
tomosis. It can also develop within the liver as a complica-
tion of percutaneous biopsy and biliary procedures [207,
221]. Patients are generally asymptomatic. However, if the
pseudoaneurysm ruptures into the peritoneal cavity, the
clinical presentation may be that of acute shock. If rupture
occurs into the gastrointestinal tract or biliary tree, there can
be gastrointestinal bleeding or jaundice [207].
Pseudoaneurysms usually appear as rounded sacs in close
proximity to, and often connected by a neck to the feeding
vessel. The characteristic ultrasound findings are of a rounded,
fluid-filled structure containing disordered, bidirectional flow
with a “yin-yang” sign on color Doppler imaging and “to-and-
fro” flow on pulsed Doppler. However, the internal appearance
of the sac is variable, depending on the degree of thrombosis
(Fig. 11.84). Treatment consists of urgent surgical repair or
stent graft placement [222].
Fig. 11.84 Hepatic artery pseudoaneurysm in a 13-year-old female
with a left lateral segment liver transplant and recent biliary interven-
tion. (a) Transverse grayscale ultrasound image shows an irregular,
ovoid, hypoechoic focus with a surrounding thick, echogenic rim. (b)
J. K. Kwon et al.
Portal vein thrombosis tends to occur in living donor liver
transplants and technical variant grafts due to the smaller size
of the vessels and the shorter vascular pedicles. The incidence
of portal vein thrombosis ranges between 1% and 5.5%, and
there is a high associated mortality, especially in the early
postoperative period [210, 214, 220]. It usually develops at the
surgical anastomosis during the first postoperative month.
On ultrasound, there is a thrombus in the main portal vein
that may be partly or completely occlusive (Fig. 11.85). An
acute thrombus may be completely anechoic so that evalua-
tion with color and pulsed Doppler is imperative when throm-
bosis is suspected. If the thrombus is completely occlusive,
there will be no flow detected by color or pulsed Doppler.
With a partially occluding thrombus, Doppler evaluation will
show decreased flow that may be in a normal direction (i.e.,
hepatopetal), or to-and-fro [220].
Treatment is variable, depending on the timing of throm-
bosis relative to time of transplantation, the severity of symp-
toms and of graft injury. Emergency revascularization can be
Color Doppler ultrasound image reveals flow within the lesion arising
from a branch of the left hepatic artery (arrow). (c) Angiographic image
demonstrates contrast extending from a branch of the artery (arrow)
into the pseudoaneurysm (arrowhead)
11 Liver
Fig. 11.85 Portal vein thrombosis in a 9-month-old male with hetero-
taxy and a left lateral segment liver transplant. Transverse color Doppler
ultrasound image of the liver transplant reveals an echogenic thrombus
(arrow) within the portal vein with no evident intraluminal flow. Flow is
noted in the adjacent hepatic artery (arrowhead)
a b
c d
Fig. 11.86 Portal vein stenosis in a 13-year-old male with a segmental
liver transplant. Longitudinal grayscale (a) and (b) color Doppler with
spectral analysis ultrasound images reveal anastomotic narrowing of
419
performed either by surgical re-anastomosis or by endovas-
cular procedures. In the setting of irreversible damage to the
graft, re-transplantation is the only option [205].
Portal Vein Stenosis
Portal vein stenosis is a late complication of pediatric liver
transplantation, with an incidence of approximately 5.6%
[220]. It typically occurs at the site of anastomosis in reduced-
sized or split liver grafts. Patients may present with portal
hypertension, ascites, and thrombocytopenia. Splenomegaly
may be present [214].
Grayscale and Doppler ultrasound findings suggestive of this
diagnosis include a diminutive portal vein diameter (less than
3–3.5 mm), a greater than 50% stenotic ratio (prestenotic diam-
eter–stenotic diameter/prestenotic diameter) and a greater than
3–4:1 velocity ratio (peak velocity at stenosis/peak velocity at
prestenotic site) (Figs. 11.86 and 11.87) [203, 214]. There may
be poststenotic portal vein dilation [203].
the portal vein (arrow) with an elevated flow velocity. (c) Pre- and (d)
post-balloon angioplasty images show an increase in caliber at the anas-
tomosis (arrows) following the procedure
420
Fig. 11.87 Portal vein stenosis in a 2-year-old male with a left lateral
segment liver transplant. Transverse color and spectral Doppler ultrasound
images proximal to (a), at (b), and distal to (c) the portal vein anastomosis
reveal markedly elevated flow velocities at the anastomosis (b) with disor-
Balloon dilation is an effective treatment for portal vein
stenosis, although recurrence has been reported in 28–50% of
patients. Recurrence can be treated with stent placement and
repeat balloon dilation. A combination of anticoagulant ther-
apy using low molecular weight heparin, warfarin, and aspi-
rin can also significantly lower the risk of recurrence [205].
 epatic Vein Outflow Obstruction
H increase in the ratio of the velocity at the anastomosis to the
Hepatic vein outflow obstruction is an uncommon complica-
tion with a reported incidence of between 1% and 6% of
pediatric recipients of living donor transplantation [220].
Obstruction occurs at the surgical anastomotic site and may
be caused by partial or complete thrombosis, or graft rotation
J. K. Kwon et al.
dered flow. Dilation (arrow) is observed in the post-anastomotic portion of
the portal vein (c). Angiographic images of the portal vein demonstrate (d)
the short stenotic segment (arrow) at the portal vein anastomosis which
was treated by angioplasty (e) with resolution of the stenosis (f)
that results in twisting or compression of the venous outflow
tract. Patients may present with abdominal pain, ascites,
abnormal liver function tests, and coagulopathy [223].
Grayscale ultrasound findings include intraluminal throm-
bus and focal luminal narrowing (Fig. 11.88). Although not
specific for hepatic venous obstruction, a monophasic wave-
form can be seen with pulsed Doppler evaluation. A fourfold
velocity in the hepatic vein 1–2 cm proximal to the anasto-
mosis has been shown to be 83% sensitive and 76% specific
for hepatic vein outflow obstruction [224].
Patients have been successfully treated with percutaneous
balloon dilation and/or stent placement [205].
11 Liver 421

a b

d e

Fig. 11.88 Hepatic vein stenosis in a 2-year-old male with a split liver
transplant. (a) Transverse grayscale ultrasound image reveals a short
segment of severe narrowing (arrow) at the hepatic vein anastomosis
with the IVC. (b) Transverse color Doppler ultrasound image shows
aliasing (arrow) at the site of narrowing in keeping with elevated blood
flow velocity. (c) Coronal contrast-enhanced CT image of the abdomen
confirms severe narrowing (arrow) at the hepatic vein anastomosis with
the IVC. Angiographic images show (d) the short stenotic segment
(arrowhead) at the hepatic vein anastomosis that (e) improved after
angioplasty
422
I nferior Vena Caval Stenosis and Thrombosis
Stenosis and thrombosis of the IVC usually occurs in con-
junction with stenosis and thrombosis of the hepatic veins
[205]. The stenotic segment is typically located at the supe-
a b
d e
g h
Fig. 11.89 Chronic IVC stenosis in a 13-year-old male with a whole liver
transplant. (a) Transverse grayscale ultrasound image shows tapering of the
hepatic veins (arrows) at the anastomosis with the IVC and an echogenic
focus (arrowhead) in the expected location of the intrahepatic IVC. (b)
Sagittal grayscale ultrasound image and (c) coronal contrast-enhanced CT
image show a dilated infrahepatic IVC (asterisks) that tapers dramatically
J. K. Kwon et al.
rior caval surgical anastomosis [216]. Associated factors
include vascular size discrepancy at the anastomosis, kink-
ing secondary to graft rotation, neointimal hyperplasia, and
fibrosis (Fig. 11.89) [205, 216].
c
f
(arrows) as it approaches the liver Venographic images show near-complete
occlusion of the intrahepatic IVC (arrowheads) with dilation of the (d)
suprahepatic and (e) infrahepatic IVC (arrows) with (f) extensive collateral
venous flow (arrows). (g) Angioplasty balloon (arrow) inflated in the intra-
hepatic IVC. (h) Post-angioplasty documentation of a successfully recana-
lized hepatocaval anastomosis (arrow)
11 Liver
Fluid Collections
Fluid collections are common in the postoperative period and
include hematoma, seroma, and biloma (Table 11.8). They
occur adjacent to the hepatic graft, particularly next to the vas-
cular and biliary anastomotic sites, and usually resolve within
several weeks [216]. In general, the nature and sterility of the
fluid collections cannot be determined solely on the basis of
their imaging features, although an increase in size and/or a
Fig. 11.90 Extrahepatic hematoma in a 13-month-old male with a left split
liver transplant. (a, b) Transverse grayscale ultrasound images obtained on
postoperative day 1 show complex hypoechoic fluid collections (arrows) with
423
change in the fluid characteristics are indicative of an active
process. MR imaging performed with a hepatocyte-specific
contrast agent can be useful in the diagnosis of a biloma [207].
Extrahepatic Collections
A hematoma is typically initially hyperechoic on grayscale
evaluation and becomes hypoechoic and smaller over time
(Fig. 11.90) [214]. A biloma is often simple in appearance
while an abscess may have septations and demonstrate
internal septations adjacent to the transplant. (c) Transverse grayscale image
of the right perihepatic collection (arrow) on postoperative day two reveals a
decrease in size indicative of partial resolution L, Liver
424 J. K. Kwon et al.

internal debris (Figs. 11.91). A biloma should be considered ment and should not be confused with an intrahepatic fluid
in cases where the fluid collection either does not resolve or collection. Of note, a fluid collection that lies within the
enlarges over time (Fig. 11.92) [203]. Occasionally, free or gallbladder fossa is always abnormal since all graft recipi-
loculated fluid can be identified within the falciform liga- ents undergo cholecystectomy prior to transplantation.

a b c

Fig. 11.91 Extrahepatic abscess in a 3-year-old male with a split hypoechoic, layering debris abutting the liver. (c) Color Doppler ultra-
liver transplant. Sagittal (a) and transverse (b) grayscale ultrasound sound image reveals no associated hyperemia. Fluid cultures grew
images demonstrate a complex fluid collection (arrows) containing Enterobacter cloacae

Fig. 11.92 Extrahepatic infected biloma in a 17-year-old male who containing echogenic, layering material. (b) Color Doppler ultrasound
received a liver transplant 12 years earlier. (a) Longitudinal grayscale image reveals no internal vascularity. Subsequent drainage procedure and
ultrasound image shows a fluid collection (arrow) along the hepatic dome fluid culture revealed a biloma infected with Escherichia (E.) coli
11 Liver
Intrahepatic Collections
Hematoma, biloma, and abscess can also develop within
the hepatic parenchyma (Figs. 11.93 and 11.94). An intra-
parenchymal abscess can have a variable appearance. It
will often demonstrate thick rim enhancement at CEUS
and may contain foci of gas. Focal infarction can also
a b
Fig. 11.93 Intrahepatic biloma in an 18-month-old male 1 day fol-
lowing whole liver transplantation. Transverse (a) and sagittal (b)
grayscale and color Doppler (c) ultrasound images demonstrate a well-­
circumscribed, anechoic fluid collection (arrows) in the left hepatic lobe
a b
Fig. 11.94 Intrahepatic abscess in a 3-year-old female with a left lateral
segment liver transplant and Escherichia coli septicemia. Transverse gray-
scale (a) and color Doppler (b) ultrasound images show a heterogeneous
hypoechoic fluid/phlegmonous collection with mild surrounding hyper-
425
occur as a consequence of diminished flow in the hepatic
artery or, less often, the portal vein. An infarct will appear
as a wedge-shaped or round, hypoechoic focus without
enhancement at CEUS. Clinical presentation and labora-
tory findings are useful in differentiating abscess from
infarction [207].
c
without associated vascularity. This collection was found to represent
a small biloma. The portal triads demonstrate increased echogenicity
secondary to postoperative edema
emia. (c) Axial contrast-enhanced CT image shows a peripherally enhanc-
ing, centrally hypoattenuating collection (arrow) in the anterior right aspect
of the split liver transplant
426 J. K. Kwon et al.

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 Gallbladder and Biliary Tract
12
Christian L. Carlson, Mitchell W. Boehnke,
and Harriet J. Paltiel

PBMJ Pancreaticobiliary maljunction

Abbreviations PET Positron emission tomography
PFIC Progressive familial intrahepatic cholestasis
AIDS Acquired immune deficiency syndrome PJS Peutz–Jeghers syndrome
BASM Biliary atresia splenic malformation RAD Right anterior duct
BMI Body mass index RPD Right posterior duct
CBD Common bile duct Tc Technetium
CCK Cholecystokinin TPN Total parenteral nutrition
CT Computed tomography UDCA Ursodeoxycholic acid
ERCP Endoscopic retrograde cholangiopancreatography WES Wall-echo-shadow
FDG Fluorodeoxyglucose
FGID Functional gastrointestinal disorder
GGT Gamma-glutamyl transferase
HIDA Hepatobiliary iminodiacetic acid Ultrasound is the ideal imaging technique for the evaluation of
HIV Human immunodeficiency virus the gallbladder and biliary tree given its excellent spatial reso-
IPMN Intraductal papillary mucinous neoplasm lution and inherently high contrast between the fluid-­filled
MIP Maximum intensity projection gallbladder and the bile ducts compared to the adjacent soft
MRCP Magnetic resonance cholangiopancreatography tissues. Ultrasound evaluation is performed for the investiga-
MR Magnetic resonance tion of congenital anomalies, cholelithiasis, biliary tract obstruc-
NHS Neonatal hepatitis syndrome tion, and tumors. An overview of normal development and
anatomy of the gallbladder and biliary tree will be provided,
and the ultrasound imaging findings of a broad spectrum of
The view(s) expressed herein are those of the author(s) and do not
reflect the official policy or position of Brooke Army Medical Center, abnormalities, along with pertinent clinical features of each
the U.S. Army Medical Department, the U.S. Army Office of the entity and treatment approach will be discussed.
Surgeon General, the Department of the Army, the Department of the
Air Force, and Department of Defense or the U.S. Government.
Electronic Supplementary Material The online version of this chap- Gallbladder
ter (https://doi.org/10.1007/978-3-030-56802-3_12) contains supple-
mentary material, which is available to authorized users.
Technique
C. L. Carlson (*) Depending on patient age, adequate preparation requires
Department of Radiology, Brooke Army Medical Center,
Uniformed Services University of the Health Sciences, Fort Sam 2–6 hours of fasting prior to the examination to allow suffi-
Houston, TX, USA cient gallbladder distention and minimize interference from
e-mail: christian.l.carlson.mil@mail.mil gas in the adjacent bowel loops. Scanning is performed with-
M. W. Boehnke out sedation. Grayscale and color Doppler imaging are rou-
Department of Radiology, Children’s Hospital Colorado, tinely used to assess gallbladder anatomy and blood flow.
University of Colorado School of Medicine, Aurora, CO, USA
H. J. Paltiel Patient Positioning
Division of Ultrasound, Department of Radiology, Boston Children’s Scanning is performed with the patient in the supine, left lateral
Hospital and Harvard Medical School, Boston, MA, USA
decubitus, and/or left posterior oblique positions. An upright or

© Springer Nature Switzerland AG 2021 433

H. J. Paltiel, E. Y. Lee (eds.), Pediatric Ultrasound, https://doi.org/10.1007/978-3-030-56802-3_12
434
prone position can sometimes aid in confirming the presence or
absence of mobile intraluminal echogenic contents such as stones.
Ultrasound Transducer Selection
The highest frequency transducer that will provide adequate
visualization of the gallbladder should be used. In most chil-
dren, a curvilinear 5–7 MHz transducer is adequate. In a par-
ticularly large or obese patient, a lower frequency transducer,
on the order of 3.5 MHz, may be necessary. Linear array
transducers can be used to provide high-resolution images of
the gallbladder in small or thin patients.
Imaging Approaches
The gallbladder neck, body, and fundus are scanned in trans-
verse and longitudinal planes. An oblique imaging plane is
required to image confluence of the right and left hepatic ducts
and the common bile duct (CBD) in the porta hepatis, a subcos-
tal oblique view with the left edge of the transducer more ceph-
alad than the right edge. The distal CBD is often obscured by
overlying duodenal or transverse colonic gas. The cystic duct is
not usually visualized in children unless it is dilated, and is best
imaged in long axis near its confluence with the common
hepatic duct in the supine or left posterior oblique positions.
A distended gallbladder provides a good acoustic window
for visualization of the distal CBD and pancreatic head.
Examination of the patient in an upright position or asking
the patient to protrude the abdomen often results in displace-
ment of shadowing bowel gas with improved visibility of the
CBD and pancreatic head.
Use of harmonic imaging and spatial compound imaging
improves the contrast between the bile ducts and the adjacent
soft tissues with improved depiction of stones as well as
other intra- and periductal structures.
In order to distinguish gallbladder stones from other enti-
ties, it is important to assess any intraluminal echogenic foci
for mobility. When feasible, this is accomplished by moving
the patient rapidly from a supine into a left lateral decubitus
position or vice versa, while maintaining the ultrasound
transducer in a fixed position on the patient’s skin.
Flow in the proximal body and neck of the gallbladder can
usually be visualized with color Doppler, and is more readily seen
when the gallbladder is contracted [1, 2]. Food consumption, fatty
food in particular, will stimulate gallbladder contraction which
can result in an artifactually thickened wall and prevent visualiza-
tion of intraluminal abnormalities. The gallbladder is ideally
evaluated during suspended respiration. However, this may not be
possible in young or uncooperative patients.
Normal Development and Anatomy
Normal Development
The gallbladder develops from an outpouching of the ventral
duodenum near the end of the fourth week of life to form the
C. L. Carlson et al.
primordial gallbladder which vacuolates and expands, initially
becoming hollow. It then fills with proliferative ­epithelium
which is followed by complete recanalization by the twelfth
week. The stalk eventually becomes the cystic duct [3–5].
Normal Anatomy
Bile flows both forward and backward through the cystic duct
which joins with the common hepatic duct to form the com-
mon bile duct (Fig. 12.1). The pear-shaped gallbladder is
located within the interlobar fissure/gallbladder fossa at the
inferior margin of the liver between the right and left hepatic
lobes in the same plane as the middle hepatic vein. The gall-
bladder consists of a fundus, body, infundibulum, and neck.
The gallbladder fundus is usually located anterolateral and
caudal to the body and neck, and often projects below the
inferior margin of the liver (Fig. 12.2).
The infundibulum is the tapered portion of the gallbladder
located between the body and the neck. The body and the neck
are directed to the left of the fundus in a posteromedial orienta-
tion toward the porta hepatis. The neck of the gallbladder tapers
near its confluence with the cystic duct. The spiral valves of
Heister are undulating folds or valves in the mucosa of the
proximal cystic duct. The exact role of these valves is uncer-
tain, although it is speculated that they may aide gallbladder
emptying in response to neural and hormonal stimulation.
Gallbladder length varies with age, ranging from 1.3 to
3.4 cm (mean 2.5 cm) in infants less than 1 year of age; and
between 3.8 and 8.0 cm (mean 6.1 cm) in children aged
Cystic duct
Gallbladder:
Neck
Common
hepatic duct
Body
Bile flow
Bile Common
bile duct
Fundus
Fig. 12.1 Diagram of normal anatomy of the gallbladder, cystic duct,
common hepatic, and common bile ducts. The normal flow of bile from
the liver into and out of the gallbladder is indicated, as well as through
the common hepatic duct and common bile duct
12 Gallbladder and Biliary Tract 435

Liver Right and left

hepatic ducts

Cystic duct

Common
Gallbadder hepatic duct

Bile duct
Accessory
pancreatic
duct Pancreatic duct

Minor duodenal Tail of pancreas

papilla

Major duodenal Body of pancreas

papilla

Duodenum Head of pancreas

Fig. 12.2 Diagram of the gallbladder and biliary tract showing their relationship to the liver, pancreas, and duodenum. The ampulla of Vater is
located at the major duodenal papilla

a b

Fig. 12.3 Normal gallbladder in a 14-year-old female. Longitudinal (a) and transverse (b) grayscale ultrasound images demonstrate the fundus
(asterisk), body (B), and neck (arrow). Gallbladder wall thickness (arrowheads) is normal

12–16 years [6]. Gallbladder length, width, and volume cor-
relate significantly with age, height, weight, and body surface
area. In particular, gallbladder length is highly significantly
correlated with height [7]. A gallbladder can be considered
abnormally small if its length is less than 2.8 cm [7].
The gallbladder is round or oval on transverse images
and oval or pear-shaped on longitudinal images (Fig. 12.3).
The wall is thin, echogenic, and smooth. In a fasting patient,
the gallbladder wall should not measure more than 3 mm in
thickness [6]. In a non-fasting patient, the gallbladder con-
tracts. However, the gallbladder wall usually remains less
than 3 mm in thickness, even when contracted (Fig. 12.4).
The interlobar fissure is echogenic and extends between the
gallbladder inferiorly and the right portal vein superiorly.
436 C. L. Carlson et al.

a b

Fig. 12.4 Contracted gallbladder in a 17-day-old male. Longitudinal (a) and transverse (b) grayscale ultrasound images show a collapsed but
otherwise normal gallbladder (arrowheads) with the wall measuring less than 3 mm in thickness

Anatomic Variants

Knowledge and recognition of normal anatomic variants is

important in preventing an incorrect diagnosis of gallbladder
pathology. Gallbladder folds are common anatomic variants
that are readily identified by ultrasound (Fig. 12.5). A Phrygian
cap is a fold located where the gallbladder fundus joins the
body, and is the most common anatomic variant of the gallblad-
der (Figs. 12.6 and 12.7). A Hartmann pouch is a focal dilation
of the wall of the gallbladder in the region between the neck and
the cystic duct, a common site for stone impaction (Figs. 12.6
and 12.8) [8, 9]. Scanning the patient in multiple planes and
positions is useful in confirming the presence of a fold.

Congenital Anomalies
Agenesis/Hypoplasia
Gallbladder agenesis is rare [10–12], with an incidence of
approximately 1 in 6500 live births [10], caused by a failure
of development of the cystic bud/caudal division of the prim-
itive hepatic diverticulum [13–15], and is more common in
females than in males [15, 16]. Absence of the gallbladder
may be associated with biliary atresia, and anomalies of the
genitourinary, gastrointestinal, cardiovascular, and musculo-
skeletal systems as well as with a variety of syndromes [17–
21]. This diagnosis should be considered when no gallbladder
Fig. 12.5 Normal gallbladder folds in a 13-year-old male. Longitudinal
grayscale ultrasound image demonstrates folds between the gallbladder
fundus and the body (white arrowhead) and between the body and the
neck (black arrowhead)
is seen in the gallbladder fossa/interlobar fissure after ade-
quate fasting (Fig. 12.9).
Gallbladder hypoplasia occurs when there is failure or
incomplete recanalization of the solid primordium [10, 21,
22] and is associated with cystic fibrosis, cholangitis, neona-
tal hepatitis, and biliary atresia. Ultrasound evaluation will
show an abnormally small gallbladder (Fig. 12.10).
12 Gallbladder and Biliary Tract 437

Phrygian cap

Hartmann
pouch

a b

Fig. 12.6 Diagram of normal gallbladder anatomic variants. (a) Phrygian cap. (b) Hartmann pouch

Fig. 12.7 Phrygian cap in a 6-month-old male. Longitudinal grayscale

ultrasound image shows a Phrygian cap (arrow) in the fundus of the
gallbladder

Fig. 12.9 Agenesis of the gallbladder in a 1-month-old fasting female

with biliary atresia. Longitudinal grayscale ultrasound image reveals no
evidence of a gallbladder in the expected location along the inferior
margin of the liver (L)

Ectopia
Fig. 12.8 Hartmann pouch in a 16-year-old female. Longitudinal
grayscale ultrasound image shows a focal dilation (asterisk) of the wall
of the gallbladder in the region between the neck (arrow) and the cystic
duct (arrowhead)
An ectopic gallbladder can be found in a wide variety of loca-
tions, including a right or left retrohepatic, suprahepatic, or
intrahepatic position; or less often beneath the left hepatic
lobe, in the falciform ligament, lesser peritoneal sac, lower
abdomen, retroperitoneum, or anterior abdominal wall [23–
27]. If the gallbladder is not identified in the gallbladder fossa
by ultrasound, care should be taken to look in these other
locations for an ectopic gallbladder (Figs. 12.11 and 12.12).
A floating gallbladder that hangs from a long mesentery in
the abdomen or pelvis may undergo torsion. A gallbladder
located beneath the left hepatic lobe is susceptible to herniation
438
Fig. 12.10 Microgallbladder in an 18-year-old female with cystic
fibrosis. Longitudinal power Doppler ultrasound image shows a tiny
gallbladder (asterisk) filled with echogenic sludge
a b
Fig. 12.12 Ectopic gallbladder in a 10-year-old male with a history
of congenital diaphragmatic hernia. Transverse grayscale ultrasound
image (a) and corresponding axial T2-weighted, fat-suppressed
through the epiploic foramen. For these reasons, some authors
advocate surgical resection of an ectopic gallbladder [28].
Septate Gallbladder
Congenital gallbladder septations are thought to be second-
ary to incomplete vacuolization of the developing gallblad-
der bud [29]. Septations may be confused with gallbladder
folds. However, septations do not change shape or size with
C. L. Carlson et al.
Fig. 12.11 Ectopic gallbladder in an 8-year-old male with a history of
gastroschisis and liver cirrhosis. Transverse grayscale ultrasound image
shows an ectopic gallbladder (asterisk) adjacent to the stomach (S) and
remote from the liver (L). Note the coarse appearance of the liver
­parenchyma in keeping with cirrhosis
magnetic resonance (MR) image (b) demonstrate an intrahepatic
location (arrowheads) of the gallbladder
changes in patient position, whereas folds will vary in
appearance. Although often asymptomatic, a septate gall-
bladder can be associated with colicky right upper quadrant
pain, cholelithiasis, cholecystitis, choledochal cyst, and
duodenal bands [29–35].
Ultrasound will show echogenic septa of variable thick-
ness that divide the gallbladder into two or more compart-
ments (Fig. 12.13, Cineclip 12.1). Multiple septa oriented in
12 Gallbladder and Biliary Tract 439

a b

Fig. 12.13 Septate gallbladder in a 5-year-old male. Longitudinal (a) and transverse (b) grayscale ultrasound images depict several thin septations
dividing the gallbladder into multiple compartments

Duplication
Fig. 12.14 Septate gallbladder in a 34-day-old male. Longitudinal
grayscale ultrasound image shows multiple thin septations resulting in
a honeycomb appearance. There is a moderate amount of mildly echo-
genic intraluminal sludge (arrowheads)
different planes can result in a honeycomb appearance
(Fig. 12.14) [31, 32, 36]. Cholecystectomy provides relief in
symptomatic patients.
Gallbladder duplication is rare, with an incidence of approx-
imately 1 in 4000 births [37, 38].
Duplication occurs during the fifth and sixth weeks of
gestation as a result of a failure of regression of outpouch-
ings that normally arise from the extrahepatic biliary system.
Their persistence leads to the development of an accessory
gallbladder [39].
Gallbladder duplication can be classified into two main
types: (1) a longitudinal septum or invaginating cleft separates
the gallbladder lumen into two chambers, with both gallblad-
ders sharing a common cystic duct; and (2) two separate gall-
bladders are present, each with its own cystic duct [38, 40–42].
Ultrasound features will vary according to the type of dupli-
cation. A completely duplicated gallbladder demonstrates two
ovoid, fluid-filled structures (Fig. 12.15). Triplicate and quadru-
plicate gallbladders have also been described (Fig. 12.16). The
same spectrum of disease that affects single gallbladders can
affect one or both gallbladders [43–45].
Treatment of a symptomatic gallbladder duplication
requires special attention to biliary ductal and arterial anat-
440 C. L. Carlson et al.

a b

Fig. 12.15 Gallbladder duplication in an 11-year-old male. Sagittal grayscale ultrasound images reveal (a) two adjacent, non-communicating, fluid-filled
structures (asterisks) in the gallbladder fossa. (b) There is a prominent fold (arrow) in the fundus of the more superiorly located gallbladder

a omy. Laparoscopic cholecystectomy with intraoperative chol-

angiography is generally recommended. Removal of an
asymptomatic duplicated gallbladder is controversial [46].

Cholelithiasis

The prevalence of gallstones in children is not well studied

b therapy, infection, furosemide, congenital biliary tract anom-
Fig. 12.16 Gallbladder triplication in a 13-month-old male. Longitudinal
(a) and transverse (b) grayscale ultrasound images show three fluid-filled
structures (asterisks) that appear to communicate inferiorly
and is known to vary between countries. In Japan, it is about
0.13%, while the highest prevalence has been reported from
the Netherlands where, based on ultrasound screening, it is
approximately 1.9% [47, 48]. Most children with gallstones
have an underlying condition or disease that predisposes to
stone formation while only a small proportion are idiopathic.
In neonates, gallstones are associated with hemolytic ane-
mia, polycythemia, prematurity, necrotizing enterocolitis,
bowel resection and short gut syndrome, dehydration, photo-
alies, and bile stasis from total parenteral nutrition (TPN)
and prolonged fasting [49, 50].
Gallstones in older children and adolescents are associ-
ated with sickle cell disease, hemolytic anemia, cystic fibro-
sis, malabsorption, TPN, liver disease, Crohn disease, bowel
resection, obesity, the antibiotic ceftriaxone, oral contracep-
tives, and metachromatic leukodystrophy [51–53].
Four types of gallstones have been described in chil-
dren: cholesterol, black pigment, brown pigment, and cal-
cium carbonate stones. Cholesterol stones are more common
in adolescents and in obese patients. Pigment stones occur
in prepubertal patients, while calcium carbonate stones
are associated with prematurity [51, 54]. Younger children
12 Gallbladder and Biliary Tract 441

p­ resent with nonspecific symptoms of vomiting, irritabil-

ity, jaundice, and failure to thrive [53]. Right upper quad-
rant pain, nausea, and vomiting occur in older children
[55].
On grayscale ultrasound imaging, gallstones appear as
echogenic intraluminal foci that produce “clean” distal
acoustic shadowing (Fig. 12.17). The presence of distal
acoustic shadowing of great importance with regard to the
specificity of diagnosis, since non-shadowing foci may
represent sludge, polyps, mural folds, or septa, or other
­
lesions [56]. Stones will also exhibit a color Doppler twin-
kling artifact (Fig. 12.18).
Gallstones are usually mobile and will move with changes
in patient position (Fig. 12.19) unless they are lodged in the gall-
bladder neck or in a Hartmann pouch. Stones typically layer
dependently within the gallbladder lumen due to a higher spe-
cific gravity than that of bile. If their specific gravity is less than
bile, they may float within the bile rather than layer dependently.
Small ­cholesterol stones can adhere to the gallbladder wall and
may not move or produce a distal acoustic shadow.
Even with optimal scanning technique, tiny stones less than
3 mm in diameter may not produce an acoustic shadow. In this
situation, imaging the child in either an upright or decubitus
position may allow small stones to aggregate dependently and Fig. 12.17 Cholelithiasis in an 11-year-old female with sickle cell dis-
to layer on top of each other so as to produce an aggregate ease and right upper quadrant pain. Sagittal grayscale ultrasound image
reveals echogenic stones (arrow) with posterior acoustic shadowing
acoustic shadow similar to that of a larger stone. (arrowheads)
The “wall echo shadow” (WES) sign or “double-arc-
shadow” sign occurs in a gallbladder filled with stones. Two
parallel, curved, echogenic lines with an intervening thin
hypoechoic zone and posterior acoustic shadowing (“WES”
sign) confirms the diagnosis of gallbladder calculi (Fig. 12.20).
The anatomic basis of the “WES” sign is the well-
defined echogenic gallbladder wall (W), echoes (E) from
gallstones located immediately beneath the gallbladder
wall, and prominent posterior acoustic shadowing (S) that
results from sound attenuation caused by the calculi. The
hypoechoic region between the echogenic gallbladder wall
and subjacent calculi represents a thin layer of interposed
bile [57]. Visualization of the lumen is sometimes possible
by repositioning the patient and allowing the gallstones to
shift into a more dependent region of the gallbladder.
Cholecystitis and pancreatitis are the most common com-
plications, occurring in approximately 10% of children with
Fig. 12.18 Cholelithiasis in a 16-year-old male with cystic fibrosis.
gallstones. Ascending cholangitis and choledocholithiasis
Longitudinal color Doppler ultrasound image shows multiple layering,
can rarely occur [51, 55]. Asymptomatic gallstones may echogenic stones (arrowheads) with posterior acoustic shadowing (aster-
resolve spontaneously. If symptomatic, cholecystectomy is isk), and twinkling artifact (arrows)
the treatment of choice.
442
a b
Fig. 12.19 Cholelithiasis in a 13-year-old female with thalassemia major. Longitudinal (a) and transverse (b) grayscale ultrasound images show
dependent echogenic stones (arrowheads) with clean posterior acoustic shadowing (arrows) that moved when patient changed position
Fig. 12.20 “Wall echo shadow” (WES) sign in a 17-year-old female
with cholelithiasis. Longitudinal grayscale ultrasound image shows the
echogenic gallbladder wall (arrow), echogenic rim of the intraluminal
gallstones (arrowhead), and posterior acoustic shadowing (asterisk). A
thin crescent of anechoic fluid is seen between the gallbladder wall and
the echogenic rim of the intraluminal stones
Sludge
Gallbladder sludge refers to particulate matter in bile. It is com-
posed of calcium bilirubinate, cholesterol crystals, and mucin,
and occurs when solutes of these molecules precipitate in bile.
It is often associated with bile stasis following prolonged fast-
ing, TPN, and extrahepatic bile duct obstruction. Sickle cell dis-
C. L. Carlson et al.
ease and other hemolytic disorders, as well as treatment with
ceftriaxone also predispose to sludge formation. Sludge may
resolve spontaneously or lead to cholelithiasis. Persistent sludge
can result in obstruction (Table 12.1) or infection.
Sludge is typically asymptomatic. Rarely, it is associated
with biliary colic and/or nonspecific signs and symptoms
such as nausea, vomiting, and acholic stools.
On ultrasound, sludge appears as low to medium level
echoes similar in echogenicity to the liver. It can be associated
with posterior acoustic enhancement. Sludge layers depend-
ently within the gallbladder, appearing as a bile-sludge level.
The viscous composition of sludge leads to slow movement
of the sludge-fluid level with changes in patient position
(Fig. 12.21). When the gallbladder is completely filled with
sludge, it may be difficult to differentiate from the liver and is
referred to as “hepatization” of the gallbladder (Fig. 12.22).
Avascularity of the intraluminal contents on color Doppler
imaging can help to differentiate a sludge-filled gallbladder
from the liver [58].
Sometimes sludge transiently coalesces to form a non-
shadowing, echogenic, mobile mass referred to as tumefac-
tive sludge or a sludge ball (Fig. 12.23). Its lack of posterior
acoustic shadowing and spontaneous resolution differenti-
ates it from stones, whereas its mobility and disappearance
on subsequent ultrasound studies distinguishes it from pol-
yps and other masses.
Low-level echoes in the gallbladder lumen are sometimes
artifactual, caused by side-lobe artifacts from adjacent strong
reflectors outside the main ultrasound beam, i.e., the bowel and
liver. The artifacts produced within the gallbladder are known as
12 Gallbladder and Biliary Tract 443

Table 12.1 Ultrasound features of bile duct obstruction

a
Causes of bile duct
obstruction Ultrasound findings
Biliary atresia Normal or increased liver size and
echogenicity
“Triangular cord” sign
Absent or small and abnormally shaped
gallbladder
Indistinct gallbladder wall with an irregular
or lobulated contour
Lack of gallbladder contraction
Absent common bile duct
Enlarged hepatic artery
Hepatic subcapsular flow
Neonatal hepatitis Normal or increased liver size and echogenicity
syndrome Small, normal, or large gallbladder
Gallbladder size change after feeding favors
neonatal hepatitis syndrome over biliary
atresia
Alagille syndrome Absent or small, abnormally shaped b
gallbladder
Bile duct paucity
± Cholestasis
± Portal hypertension
± Cirrhosis
Byler disease Multiple saccular cystic lesions
“Central dot” sign
Cysts do not communicate with bile ducts
Choledocholithiasis Echogenic shadowing stone(s) in common
bile duct
Associated intrahepatic biliary ductal
dilation
Inspissated bile Dilated intra- and extrahepatic bile ducts
syndrome with intraluminal echogenic bile and
fluid-debris levels with no acoustic
shadowing
± Sludge in distended gallbladder
Sclerosing cholangitis Thick-walled, dilated intrahepatic ducts with
intervening focal narrowing (“string of
beads”)
± Intraductal stones
± Cholelithiasis
± Gallbladder wall thickening
± Portal hypertension
± Cirrhosis
Mirizzi syndrome Shadowing stone(s) in the cystic duct or Fig. 12.21 Gallbladder sludge in a 12-year-old male with pancreatitis.
gallbladder neck Longitudinal (a) and transverse (b) grayscale ultrasound images show
Dilation of the common hepatic and dependent, echogenic sludge (arrowheads) within the gallbladder with
intrahepatic ducts a bile-sludge level
Normal/decompressed common duct below
obstructing stone pseudo-sludge (Fig. 12.24). True sludge and pseudo-sludge can
Bile duct stricture Long or short segment extrahepatic bile duct be differentiated by imaging the patient in different positions.
narrowing
True sludge will shift and layer with positional changes, whereas
AIDS cholangiopathy Dilation, irregularity, and segmental
narrowing of intra- and extrahepatic pseudo-sludge will not layer and may vanish.
ducts In asymptomatic patients, sludge can be managed expec-
Tapering and stenosis of distal CBD tantly without treatment. In symptomatic patients and those
Echogenic nodule in distal CBD (edema of
with complications of cholecystitis, cholangitis, or pancre-
papilla of Vater)
Thick-walled, dilated gallbladder atitis, the treatment of choice is cholecystectomy. Endoscopic
Choledochal cyst Fusiform or spherical dilation of extrahepatic sphincterotomy is a less invasive procedure and ursodeoxy-
bile duct or cystic mass in porta hepatis cholic acid (UDCA) is a medical therapy that can prevent
separate from gallbladder but in sludge formation and recurrent pancreatitis [59].
continuity with extrahepatic duct
± Intracystic sludge, stones UDCA is only recommended for patients who are not surgi-
AIDS, Acquired immune deficiency syndrome; CBD, common bile duct
cal candidates due to a high incidence of gallstone recurrence.
444
Fig. 12.22 “Hepatization” of the gallbladder due to intraluminal sludge in
a 16-year-old female with graft-vs-host disease following treatment for leu-
kemia. (a) Transverse grayscale ultrasound image shows sludge almost
completely filling the gallbladder (asterisk). The echogenicity of the sludge
is comparable to that of the adjacent liver (L). A crescent of anechoic bile is
seen layering along the superior aspect of the gallbladder (arrow). (b)
Longitudinal power Doppler ultrasound image shows blood flow within the
liver (L). The sludge within the gallbladder is avascular
Fig. 12.23 Tumefactive biliary sludge in a neonate. A longitudinal
power Doppler ultrasound image shows an avascular, mass-like collec-
tion of sludge (arrowheads) in the lumen of the gallbladder
Elective cholecystectomy has been recommended in children
with sickle cell anemia since most sickle cell patients with
sludge will eventually develop gallstones [60].
C. L. Carlson et al.
Fig. 12.24 Pseudo-sludge in a 16-year-old female with abdominal pain.
(a) Longitudinal grayscale ultrasound image shows apparent echogenic,
layering sludge (arrowhead) in the body of the gallbladder. (b) Longitudinal
grayscale ultrasound image obtained after repositioning of the patient
reveals a rounded, echogenic focus in the gallbladder fundus (asterisk). The
“sludge” in (a) is no longer visualized. The echogenic foci in both images
are presumably due to side-lobe artifacts related to an adjacent air-filled
bowel loop in (a) and the liver in (b). L, Liver
Cholecystitis
 cute Calculous Cholecystitis
A
Acute calculous cholecystitis is much less common in chil-
dren than adults. Gallstone obstruction of the cystic duct leads
to gallbladder distention, mucosal damage, and inflammation
[48, 61]. Presenting symptoms in older children and adoles-
cents are similar to those typically seen in adults, and include
right upper quadrant or epigastric pain and tenderness, nausea,
and vomiting. Younger children are less likely to present with
classic symptoms, while jaundice and fever are more common
in infants. Leukocytosis is often present [47, 48, 62].
Characteristic ultrasound findings of acute calculous chole-
cystitis include discrete intraluminal echogenic shadowing
stone(s), sludge, gallbladder enlargement, a wall thickness
12 Gallbladder and Biliary Tract
greater than 3 mm, focal tenderness over the gallbladder (the
sonographic Murphy sign), and pericholecystic fluid (Fig. 12.25,
Cineclip 12.2). Ultrasound findings in pediatric cholecystitis
have lower sensitivity and positive predictive value than reported
in adults. These differences may be explained by the higher
prevalence of chronic cholecystitis in the pediatric population.
Children may have milder episodes of self-limited gallbladder
inflammation compared with adults, and this may lead to a
delay in treatment. Cholelithiasis and gallbladder tenderness
have been reported as having the greatest positive predictive
value in the diagnosis of acute cholecystitis in children [63].
a b
Fig. 12.25 Acute calculous cholecystitis in a 6-year-old male with
right upper quadrant abdominal pain, vomiting, and a sonographic
Murphy sign. (a) Transverse grayscale ultrasound image shows an
intraluminal stone (arrowhead) with distal acoustic shadowing, a thick-
a b
Fig. 12.26 Acute calculous cholecystitis in a 12-year-old female. (a)
Longitudinal grayscale ultrasound image of the gallbladder shows mul-
tiple dependent stones (arrowhead) with distal acoustic shadowing,
445
Although it is a relatively insensitive sign, color Doppler
demonstration of prominent blood flow within the gallbladder
wall in the distal body and fundus may be seen in acute cholecys-
titis (Fig. 12.26) whereas in a normal gallbladder, flow is usually
identified only in the proximal body and gallbladder neck [1].
Isolated acute calculous cholecystitis in children generally
has an excellent prognosis. The greatest indicator for a poor
outcome is the presence of an underlying condition such as
obesity, cystic fibrosis, hepatic disease, sickle cell disease, dia-
betes mellitus, and immunocompromise. Potential complica-
tions include gallbladder perforation, abscess, empyema, and
ened gallbladder wall (asterisk), and a small amount of pericholecystic
fluid (arrow). (b) Longitudinal grayscale ultrasound image shows the
stone (arrowhead) in the gallbladder neck
pericholecystic fluid (arrow), and mild wall thickening (asterisk). (b)
Longitudinal color Doppler ultrasound image of the gallbladder shows
wall hyperemia (arrow)
446
gangrene. Increased intraluminal pressure can lead to mural
ischemia and necrosis, resulting in gangrenous cholecystitis.
Ultrasound features of gangrenous cholecystitis include
focal gallbladder wall thickening, intraluminal membranes due
to sloughed mucosa, mural ulcerations, and pericholecystic
a b
Fig. 12.27 Acute cholecystitis with perforation in a 17-year-old female
with acute abdominal pain, distention, guarding, fever, and elevated white
blood cell count. (a) Transverse grayscale ultrasound image shows an echo-
genic stone (arrow) in the neck of the gallbladder. The gallbladder wall
(arrowhead) is mildly thickened. (b) Transverse grayscale ultrasound
C. L. Carlson et al.
fluid. When fever persists, or there is increasing pain or tender-
ness, gallbladder perforation is more likely. Perforation usually
occurs at the gallbladder fundus (Fig. 12.27). A local perfora-
tion can give rise to an abscess, extend into the peritoneum and
lead to peritonitis, or result in a cholecystoenteric fistula [64].
image reveals focal discontinuity (arrowhead) of the gallbladder wall with
an adjacent fluid collection (asterisk). Arrow, Stone. (c) Sagittal contrast-
enhanced computed tomography (CT) image demonstrates the fundal per-
foration (arrow) of the gallbladder and a heterogeneous, enhancing abscess
(arrowheads) surrounding the anteroinferior aspect of the right hepatic lobe
12 Gallbladder and Biliary Tract
Table 12.2 Ultrasound features of cholecystitis in children
Cholecystitis type Ultrasound findings
Acute calculous Gallstones
Focal tenderness (sonographic Murphy sign)
Sludge
Gallbladder dilation and enlargement
Wall thickening >3 mm
Pericholecystic fluid
Distal gallbladder body/fundus mural blood
flow on color Doppler
± Mural striations
Emphysematous Non-dependent mural or intraluminal
echogenic foci of gas with dirty posterior
acoustic shadowing and ring-down
artifact
± Pneumobilia
Gangrenous Intraluminal echogenic membranes
paralleling gallbladder wall
Asymmetric or irregular wall thickening
Mass-like intraluminal protrusions
Loss of gallbladder wall–mucosa interface
Focal perfusion defects
Mural striations
Pericholecystic fluid
± Fundal perforation
Pericholecystic hypervascular inflammatory
fat
Acute acalculous Presence of ≥ 2 of the following in the
absence of gallstones:
Sludge
Luminal distention
Mural thickening (> 3 mm)
Pericholecystic fluid
± Pericholecystic inflammatory changes
Biliary dyskinesia Normal-appearing gallbladder
Abnormal gallbladder ejection fraction on 3D
and 4D ultrasound (fasting followed by
CCK or fatty meal-stimulated gallbladder
contraction)
Chronic Gallstones
Contracted gallbladder
± Focal or diffuse mural thickening
± Sludge
Xanthogranulomatous Gallstones
Hypoechoic mural nodules or bands
Asymmetrically thickened and inflamed
gallbladder wall
CCK, Cholecystokinin
Laparoscopic cholecystectomy is the treatment of choice
in calculous cholecystitis. Cholecystostomy with stone
removal can be performed in patients for whom a function-
ing gallbladder is important, as in Crohn disease. Patients
with complicated cholecystitis will require antibiotic treat-
ment as well as surgical management. Spontaneous ­resolution
of cholelithiasis may occur in neonates, even when compli-
447
cated by acute cholecystitis, and has been successfully man-
aged non-operatively with intravenous fluids and antibiotics
with complete resolution [65]. Cholecystectomy is necessary
when gangrene is suspected (Table 12.2) [64].
 cute Acalculous Cholecystitis
A
Acalculous cholecystitis is inflammatory disease of the gall-
bladder in the absence of gallstones. The overall incidence of
cholecystitis is rising in the pediatric population and acalcu-
lous cholecystitis is more common than calculous cholecys-
titis in children [66]. The cause is unknown but is associated
with immediate postoperative states, trauma, and burns.
Sepsis from a variety of organisms has been associated with
this condition, including Leptospira, group A beta-hemolytic
streptococci, Shigella, Salmonella, and Escherichia coli,
although no organism has emerged as the definitive cause.
A variety of other conditions are also associated with
acalculous cholecystitis, including diabetes, systemic vascu-
litis, malignant disease, and congestive heart failure. Because
no obstructing stone is present, gallbladder stasis likely plays
an important role in its pathogenesis. Stasis may be due to
fever, dehydration, prolonged fasting, ileus, or TPN [64].
Nonspecific clinical manifestations include cholestasis
and liver dysfunction, biochemical abnormalities, right upper
quadrant pain, leukocytosis, and may also include nausea
and vomiting, fever, and jaundice [67, 68].
Ultrasound features of acute acalculous cholecystitiss are
similar to those observed in calculous cholecystitis except
that intraluminal calculi are absent. Typical findings include
intraluminal sludge, luminal distension, mural thickening
(greater than 3 mm), and pericholecystic fluid (Figs. 12.28,
12.29). The presence of at least two of these abnormalities in
the absence of gallstones supports the diagnosis of acalcu-
lous cholecystitis.
Most children with acalculous cholecystitis are managed
conservatively with antibiotics and supportive care, although
hospitalization is advised for monitoring of potential compli-
cations. When surgery is necessary, either cholecystectomy
or cholecystostomy can be performed [64].
Biliary Dyskinesia
Biliary dyskinesia is a diagnosis that is being made increas-
ingly in children. Biliary dyskinesia is defined as abdominal
pain that is biliary in nature based on its location and charac-
ter, with a completely normal-appearing gallbladder on
imaging evaluation (usually ultrasound), and decreased gall-
bladder contraction in response to a pharmacological stimu-
lus [69].
448
a a
c Fig. 12.29 Acute acalculous cholecystitis in a 3-year-old male with
Fig. 12.28 Acute acalculous cholecystitis in a 10-year-old female with
relapsed leukemia, septic shock, liver dysfunction and cholestasis.
Longitudinal (a) and transverse (b) grayscale ultrasound images show a
thickened, edematous gallbladder wall. There is a tiny amount of free
fluid (arrow) adjacent to the gallbladder. (c) Longitudinal color Doppler
ultrasound image reveals hyperemia of the gallbladder wall. There is no
intraluminal stone
C. L. Carlson et al.
severe pneumonia and sepsis. (a) Longitudinal grayscale ultrasound
image shows a gallbladder with a mildly thickened wall (arrow) and intra-
luminal sludge (arrowhead). (b) Transverse color Doppler ultrasound
image shows hyperemia of the wall and a small amount of pericholecystic
fluid (arrow). Arrowhead, Sludge. There is no intraluminal stone
Biliary dyskinesia is thought to be related to increased pres-
sure within the gallbladder lumen caused by uncoordinated
gallbladder contractions in the setting of either structural or
functional outflow obstruction, or partial cystic duct obstruction
from inflammatory stenosis or spasm occurring during gallblad-
der contraction. There is no standard, validated method for
assessing gallbladder motility in children. Techniques using
either ultrasound or scintigraphy have been described [69–71].
Both dynamic 3D and 4D ultrasound techniques, fasting
followed by cholecystokinin or fatty meal-stimulated gall-
bladder contraction, correlate well with scintigraphy and
may be advantageous for pediatric patients as a screening
method or in place of scintigraphy given its safety, efficiency,
and availability [71, 72].
12 Gallbladder and Biliary Tract
a
b The extent of gallbladder wall involvement varies from a
Fig. 12.30 Chronic cholecystitis in a 17-month-old male born prema-
turely at 24 weeks of gestation. Longitudinal (a) and transverse (b)
grayscale ultrasound images reveal a contracted gallbladder (arrow-
heads) filled with echogenic stones and associated with distal acoustic
shadowing (arrows)
The outcomes of operative and conservative treatment are
equivalent, and less aggressive medical, dietary, and psycho-
logical approaches are currently advocated [69, 70, 73].
Chronic Cholecystitis
Chronic cholecystitis in children is significantly more common
than acute cholecystitis, and results from chronic irritation sec-
ondary to gallstones, recurrent bouts of acute cholecystitis, or
cystic fibrosis-related biliary disease, with chronic inflamma-
tion leading to mural fibrosis. Patients may be asymptomatic
and have mild disease.
Ultrasound findings of chronic cholecystitis include gall-
stones, a contracted gallbladder, focal or diffuse wall thick-
ening, and/or sludge. A sonographic Murphy sign and mural
hyperemia are invariably absent (Fig. 12.30) [74–76].
Xanthogranulomatous cholecystitis is a rare form of
chronic cholecystitis with gallstones and hypoechoic nodules
or bands within an asymmetrically thickened, inflamed wall
representing lipid-laden xanthogranulomatous nodules [77].
The standard treatment for chronic cholecystitis in children
is cholecystectomy. The fact that gallstones and chronic chole-
cystitis are independent risk factors for the development of
449
gallbladder malignancy in adults has provided justification for
intervening earlier in symptomatic children. Chronic inflam-
mation can make operative intervention more difficult, increas-
ing the risk of complications. Some authors have therefore
recommended that early cholecystectomy be considered when
cholelithiasis and symptoms are present [74].
Porcelain Gallbladder
The term “porcelain gallbladder” refers to calcification of the gall-
bladder wall. It is extremely rare in childhood. Although the etiol-
ogy of this calcification is unknown, the mechanism is thought to
be similar to that of gallstone development. Porcelain gallbladder
occurs in association with gallstone disease, and it may represent a
form of chronic cholecystitis. It is often found incidentally on plain
abdominal radiographs or other imaging studies.
single calcified plaque adherent to the mucosal layer to com-
plete full-thickness replacement of the entire gallbladder wall
with calcium. Abdominal pain and epigastric tenderness have
been described along with hepatobiliary biochemical abnor-
malities. However, many patients are asymptomatic [78–81].
Ultrasound findings of porcelain gallbladder include a
continuous or discontinuous hyperechoic gallbladder wall
with posterior acoustic shadowing (Fig. 12.31). Whether or
not porcelain gallbladder is associated with gallbladder car-
cinoma is controversial, as is prophylactic cholecystectomy.
Given the possible risk of malignancy and long life expec-
tancy, cholecystectomy is usually performed in children with
a porcelain gallbladder [79–81].
Hydrops
Hydrops of the gallbladder represents an acute, massive
enlargement without associated congenital malformation,
stone, or inflammation. Hydrops is thought to be caused
either by a transient and self-limited obstruction of the
cystic duct or to increased mucus secretion by the gall-
bladder with ineffective emptying. It is associated with
Kawasaki disease, TPN, familial Mediterranean fever,
scarlet fever, typhoid fever, leptospirosis, ascariasis, and
sepsis.
Hydrops can occur from the neonatal period into adoles-
cence, with an average age of 5 years. There is a 2:1 ratio of
affected males to females [82, 83]. Children with gallbladder
hydrops may be asymptomatic, or can present with right
upper quadrant pain or a mass. Patients are afebrile and the
bile is sterile, features that help to differentiate hydrops from
acute cholecystitis.
Ultrasound findings include striking gallbladder enlarge-
ment without wall thickening (Fig. 12.32). Occasionally,
there may be intraluminal sludge. The intra- and extrahepatic
bile ducts are not usually dilated.
Rehydration and resolution of the underlying disease usu-
ally lead to spontaneous resolution of gallbladder hydrops.
450 C. L. Carlson et al.

a a

b
b

Fig. 12.31 Porcelain gallbladder in a 21-month-old male. Transverse

(a) and longitudinal (b) grayscale ultrasound images show a sludge-­
filled gallbladder (asterisks) with an extensively calcified wall (arrows)
and associated posterior acoustic shadowing (arrowheads)

Torsion
Gallbladder torsion is a rare entity that only infrequently
affects children. Although its precise etiology is uncertain,
certain anatomic variants are thought to predispose to tor-
sion. In some cases, the gallbladder has its own mesentery; in
others, the cystic duct and artery have a mesentery, and the
gallbladder lies free within the peritoneal cavity.
The peak incidence of gallbladder torsion in children is
between 6 and 13 years with a 2.5–4:1 male-to-female prepon-
derance, with the youngest reported patient being 5 days old
[84]. Clinical signs and symptoms are typically nonspecific.
Ultrasound imaging features include fluid between the
gallbladder and the liver bed, a horizontal orientation of the
long axis of the gallbladder at a distance from the gallbladder
fossa, mural thickening, and signs of inflammation, isch-
emia, or necrotic change [85]. A spiral appearance of the cys-
tic artery has also been described [84, 86].
Fig. 12.32 Gallbladder hydrops in a 9-year-old male. Longitudinal (a)
and transverse (b) grayscale ultrasound images show a distended gall-
bladder that measures more than 10 cm in length
Prompt cholecystectomy results in an excellent progno-
sis. However, a correct preoperative diagnosis of torsion can
be challenging, and there is a reported mortality rate of
approximately 5–6% [84, 86].
Polyps
The majority of gallbladder polyps are asymptomatic, inci-
dentally discovered lesions. Adenomyomatosis, cholesterol
polyps, and inflammatory polyps are the most common, aris-
ing from the gallbladder wall and projecting into the lumen
(Table 12.3).
12 Gallbladder and Biliary Tract 451

Table 12.3 Ultrasound features of gallbladder polyps in children

Polyp type Ultrasound findings
Adenomyomatosis Gallbladder wall thickening with
echogenic mural foci
Large (≥ 10 mm) intraluminal nodules
containing small cystic spaces
(“adenomyomas”)
Cholesterol Small (2–10 mm; rarely up to 20 mm),
smooth, ovoid, mural lesions
“Ball on the wall” sign
± Fine pattern of echogenic foci in larger
lesions
Inflammatory Nonspecific appearance
Benign Pedunculated
± Irregular shape
Iso- to hyperechoic to gallbladder wall
Increased heterogeneity with larger size
± Blood flow on color Doppler
Malignant Solitary
> 10 mm
Wide polyp base
Contour irregularity
Focal wall thickening >3 mm
Rapid increase in size
Gallstones Fig. 12.33 Adenomyomatosis in a 3-month-old male. Longitudinal gray-
Metastasis Nonspecific, similar to primary lesion scale ultrasound image shows multiple echogenic foci (arrowheads) along
Gastric and pancreatic Nonspecific appearance the gallbladder wall with associated comet-tail artifacts (arrows)
heterotopia
Hamartoma Nonspecific appearance
gallbladder fundus. When the body is involved, it can cause
narrowing of its mid-portion, resulting in an “hourglass”
gallbladder.
Adenomyomatosis When echogenic foci, cystic spaces, or twinkling artifact
Adenomyomatosis of the gallbladder is a benign disorder are absent, further evaluation with magnetic resonance (MR)
characterized by hypertrophy of the mucosal epithelium that imaging is recommended to rule out a mass. T2-weighted
invaginates into the interstices of the thickened smooth mus- MR imaging sequences are useful in depicting the Rokitansky–
cle, forming so-called Rokitansky–Aschoff sinuses. Adeno­ Aschoff sinuses. A “pearl necklace” sign consisting of mul-
myomatosis can be focal or diffuse, and most patients are tiple round hyperintense spaces has been reported with a
asymptomatic. It is rare in children, with reported patients 92% specificity for adenomyomatosis [91].
ranging in age from 4 months to 11 years, with an equal sex Although evidence regarding optimal management in chil-
distribution [87–90]. The prevalence in childhood is unknown dren is limited, cholecystectomy has been advocated in both
[88, 89]. Although adenomyomatosis is associated with gall- symptomatic and asymptomatic patients because of the
stones in more than 90% of adult cases, this association does uncertain relationship between adenomyomatosis and
not occur in children [89]. ­gallbladder cancer. Preoperative mapping of the extrahepatic
When symptoms occur in childhood, they are usually biliary tree with magnetic resonance cholangiopancreatogra-
nonspecific, and similar to those of chronic gallbladder dis- phy (MRCP) is recommended to prevent iatrogenic injury
ease, including diffuse or right upper quadrant abdominal since major anatomic variants and anomalies have been found
pain, sometimes associated with nausea and vomiting. in up to 18% of patients [89].
Laboratory values are usually normal.
The most common appearance of adenomyomatosis on Cholesterol Polyps
ultrasound is of tiny echogenic foci in the wall of the gall- Cholesterol polyps are the focal form of gallbladder
bladder with associated comet-tail artifacts (Fig. 12.33). cholesterolosis, a common non-neoplastic disorder of
­
Adenomyomas are larger, mass-like lesions that contain unknown etiology. Cholesterolosis results in the accumulation
cystic spaces. Focal adenomyomatosis usually affects the of cholesterol esters and triglycerides within the epithelial
452 C. L. Carlson et al.

macrophages of the gallbladder mucosa and submucosa. The

a
diffuse form of gallbladder cholesterolosis is not visible on
imaging, and is commonly known as “strawberry gallbladder”
due to its gross pathological appearance [64].
The risk factors for cholesterolosis are similar to those of
gallstone disease, although the two disorders rarely co-exist
[92]. Cholesterolosis is typically asymptomatic, but may be
associated with vague, dull right upper quadrant or epigastric
biliary-type pain.
On ultrasound imaging, cholesterol polyps are small,
round, smooth intraluminal lesions that appear to be stuck to
the wall, described as the “ball on the wall” sign. They are
usually echogenic with no associated acoustic shadowing
(Fig. 12.34, Cineclip 12.3). Unlike gallstones, they are not
mobile. When cholesterol polyps are greater than 1 cm in
diameter or when there are multiple confluent polyps, they
cannot be confidently differentiated from other benign or
malignant lesions [91].
No treatment is necessary for small, asymptomatic cho- b
lesterol polyps. Cholecystectomy is indicated in symptom-
atic patients, or for lesions greater than 1 cm in diameter.

Inflammatory Polyps
Inflammatory polyps tend to occur in a setting of gallstone
disease and chronic cholecystitis. Histologically they are
composed of granulation and fibrous tissue with lympho-
cytes and plasma cells that infiltrate the lamina propria. They
represent up to 10% of all gallbladder polyps, may be single
or multiple, and range in size from 5 to 10 mm in diameter.
No evidence of an associated increased risk of adenocar-
cinoma from inflammatory polyps has been established [91,
92]. Inflammatory gallbladder polyps are either asymptom-
atic or can be associated with symptoms similar to those of c
gallstone disease and chronic cholecystitis.
Inflammatory polyps have a nonspecific appearance on
ultrasound imaging, and therefore a definitive diagnosis can-
not be made [91]. Surgery is not required and when discov-
ered at cholecystectomy, inflammatory polyps are nearly
always incidental findings.

 ther Polypoid Lesions

O
Other polypoid masses of the gallbladder are extremely unusual
in children. There are a few reports of adenoma, fibroepithe-
lial polyp, mucous retention cyst, heterotopic gastric and
pancreatic tissue, and hamartoma, among others. These
lesions have a variable appearance on ultrasound imaging,
and a definitive diagnosis can only be made on pathological
evaluation [93, 94].
Fig. 12.34 Cholesterol polyps in a 17-year-old female. Longitudinal
Primary malignant tumors of the gallbladder are extremely
(a, b) grayscale and transverse (c) color Doppler ultrasound images
rare in children with only a handful of cases reported in the reveal small, non-mobile, echogenic lesions attached to both the depen-
dent (a) and non-dependent (b, c) portions of the gallbladder wall.
There is no associated distal acoustic shadowing
12 Gallbladder and Biliary Tract 453

literature [95]. In most cases, gallbladder carcinoma is associ-

a
ated with gallstones. Metastases and lymphoma can uncom-
monly manifest as polypoid gallbladder lesions [91].

Other Disorders

Gallbladder Varices
Gallbladder varices are seen in the setting of portal hyper-
tension or portal vein thrombosis and represent porto-portal
collateral vessels [96–98]. Gallbladder varices do not pro-
duce specific symptoms and patients usually present with
symptoms attributable to their underlying disease. Their
clinical significance lies in their propensity to bleed during
biliary surgery [98]. They can rarely cause spontaneous b
hemobilia, intraabdominal bleeding, or gallbladder rupture.
Grayscale ultrasound imaging shows a thickened
­gallbladder wall containing dilated tubular structures that
demonstrate venous flow on color and spectral Doppler
evaluation (Fig. 12.35).
Treatment of the underlying condition such as portal vein
thrombosis or portal hypertension may alleviate varices.
Although endoscopic sclerotherapy has been successfully
used to treat varices, surgery remains the treatment of choice
for control of bleeding in symptomatic patients [98–100].

Trauma
Gallbladder trauma in children is extremely unusual, but can
result in significant morbidity when it occurs [101]. Most
c
cases are due to blunt abdominal trauma with injuries includ-
ing contusion, laceration, perforation, and avulsion. Protection
of the gallbladder by the liver, intestines, omentum, and rib
cage accounts for its exceptionally rare incidence. Distention
of the gallbladder predisposes to injury. Clinical presentation
is often delayed, with vague symptoms. Signs and symptoms
of traumatic gallbladder injury can be nonspecific, including
fever, abdominal pain and distention, localized tenderness,
vomiting, diarrhea, and/or ileus. Hepatic enzyme blood levels
are frequently increased [101–103].
Imaging findings on ultrasound include pericholecystic fluid,
intraluminal blood of varying echogenicity (depending on the
timing of the study relative to the injury), and a thickened, occa-
sionally discontinuous gallbladder wall (Figs. 12.36 and 12.37).
Hepatobiliary scintigraphy can be used to confirm perforation
and biloma [102–104].
A simple contusion can be managed conservatively, Fig. 12.35 Gallbladder varices in a 10-year-old female with portal
while cholecystectomy is necessary for the treatment of hypertension and cavernous transformation of the portal vein. (a)
total avulsion or perforation. In clinically stable patients, Longitudinal grayscale ultrasound image shows a thickened gallbladder
wall that contains multiple tubular structures (arrowheads). (b)
serial observation and imaging are sufficient. Diagnostic Longitudinal power Doppler ultrasound image demonstrates flow
laparoscopy or laparotomy is advocated in patients with (arrowheads) within the mural tubules in keeping with varices. (c) Axial
more than physiological amounts of free intraperitoneal contrast-enhanced CT image confirms the presence of multiple enhanc-
fluid [101, 103]. ing vessels (arrowheads) in the gallbladder wall
454
a b
Fig. 12.36 Gallbladder hematoma in a 12-year-old male who sustained
blunt abdominal trauma. Longitudinal (a) and transverse (b) grayscale
images show heterogeneous material in the gallbladder in keeping with
hematoma. (c) Longitudinal color Doppler ultrasound image of the common
a c
Fig. 12.37 Gallbladder injury in a 3-month-old male with non-­accidental
trauma. Longitudinal (a) and transverse (b) grayscale ultrasound images
reveal diffusely abnormal, echogenic gallbladder mucosa and dependent,
layering echogenic material (arrowheads) in keeping with blood. The gall-
bladder wall (white arrows) is thickened and edematous, and there is adja-
C. L. Carlson et al.
c
bile duct reveals dilation down to the level of the pancreatic head (P).
There is echogenic material (arrowheads) in the distal duct in keeping
with blood
cent free fluid (black arrows) containing echogenic strands consistent with
blood products. (c) Sagittal contrast-enhanced CT image shows layering,
high attenuation material (arrow) in the gallbladder compatible with blood.
There is ascites (asterisk) and periportal edema (arrowheads)
12 Gallbladder and Biliary Tract 455

Biliary Tract
Technique
Patient positioning, ultrasound probe selection, and imaging
approaches to the biliary tract are described above in the sec-
tion on Gallbladder.
Normal Development and Anatomy
Normal Development
The biliary tree develops from an outpouching of the ventral
duodenum, the hepatic diverticulum, early in the fourth week
of life. Duct elongation is followed by plugging of the lumen
with epithelial cells. Recanalization is complete by the sev-
enth week [3].
Normal Anatomy
The biliary tree contains both intra- and extrahepatic bile
ducts. The intrahepatic bile ducts along with adjacent portal
veins and hepatic arteries form the portal triads. The intrahe-
patic ducts course from the periphery of the liver to the
hepatic hilum where they converge to form the main right
and left hepatic ducts (Fig. 12.38). The normal intrahepatic
bile ducts are not routinely visualized by ultrasound due to
their small size.
The common hepatic duct is the initial segment of the
extrahepatic bile duct formed by the confluence of the main
right and left hepatic ducts and proximal to the cystic duct. It
typically lies to the right and anterior to the main portal vein
and hepatic artery. In a minority of patients, the common
hepatic duct lies posterior to the hepatic artery.
The CBD is the segment of the extrahepatic bile duct that
extends from the confluence of the cystic and common
hepatic ducts to the head of the pancreas along with the
hepatic artery and portal vein within the hepatoduodenal
ligament. In most patients, the CBD and pancreatic duct
unite and drain through a common aperture at the ampulla of
Vater (Fig. 12.2). In a minority of patients, the ducts enter the
duodenum separately.
On transverse ultrasound views obtained at the porta
hepatis, the CBD and hepatic artery usually appear as two
small circles anterior to the larger circle formed by portal
vein, giving an appearance of a face with two ears, some-
times called the “Mickey Mouse” sign, with the CBD as the
right ear, the hepatic artery as the left ear, and the portal vein
as the face (Fig. 12.39) [105]. Color and spectral Doppler
ultrasound are also useful in the identification of the CBD,
portal vein, and hepatic artery.
CBD diameter is age-dependent [106, 107]. A recent
study found that mean CBD size was 1.24 ± 0.54 mm in
children less than 1 year of age; 1.97 ± 0.71 mm at 1–10
years, and 2.98 ± 1.17 mm over the age of 10 years [107].

Right
hepatic
duct

Posterior
branch

Left
hepatic
duct
Anterior
branch

Common
hepatic duct

Cystic duct

Common
bile duct

Fig. 12.38 Diagram of the gallbladder and biliary tree

456
a c
P
C
Fig. 12.39 Normal anatomy of the common bile duct (CBD) in an
8-year-old female. (a) Longitudinal grayscale ultrasound image of the
porta hepatis shows a normal CBD (arrow) extending into the head of
the pancreas (asterisk). (b) Longitudinal color Doppler ultrasound
image demonstrates the avascular CBD (arrow) adjacent to the main
Duct size can increase by 1 mm or more with the Valsalva
maneuver or deep inspiration [108]. CBD size also increases
following cholecystectomy [109].
Anatomic Variants
An awareness of common anatomical variations in bile duct
anatomy is necessary in order to prevent complications dur-
ing the performance of biliary interventions and surgical
procedures. A knowledge of the Couinaud functional anat-
omy of the liver is critical when describing intrahepatic bile
duct anatomy (see Chap. 11).
The most common biliary tract branching pattern occurs in
approximately 56–58% of individuals, consisting of a right
hepatic duct formed from the right anterior and right posterior
branches that drain the anterior (segments V and VIII) and
posterior (segments VI and VII) segments of the right hepatic
lobe. On the left side, branches from segments II and III
branches join to form the left hepatic duct. This duct becomes
C. L. Carlson et al.
C
portal vein (P), inferior vena cava (C), and hepatic artery (arrowhead).
(c) Transverse oblique grayscale ultrasound image at the porta hepatis
depicts the main portal vein (P) posterior to the CBD (arrowhead) on
the right and the hepatic artery (arrow) on the left, the so-called “Mickey
Mouse” sign. C, Inferior vena cava
extrahepatic in location as it extends to the right of the falci-
form ligament, where it is joined by ducts of Couinaud seg-
ments IV and I (Figs. 12.38 and 11.14) [110–112].
Most common variants of bile duct branching are due to
differences in the site of insertion of the right posterior duct
(RPD) in segments VI and VII. The RPD frequently extends
toward the porta hepatis in a cranial direction, superior and
posterior to the right anterior duct (RAD). It then extends
caudally to join the RAD and form the right hepatic duct.
Three other frequent sites of RPD insertion account for
most biliary anatomic variations. If the RPD extends more
to the left than usual, it can join the left hepatic duct (in
about 13%), or the junction of the right and left hepatic
ducts (the “trifurcation pattern” in about 8%). If the RPD
extends in a medial and caudal direction, it can directly join
the common hepatic duct or CBD (in about 5%). Direct
drainage of the individual segmental hepatic ducts into the
common hepatic duct or CBD is less common.
The cystic duct usually joins the common hepatic duct below
the confluence of the right and left hepatic ducts. Three com-
12 Gallbladder and Biliary Tract
a b
d e
Fig. 12.40 Diagram of cystic duct variations. (a) Usual confluence of
cystic duct with the common hepatic duct to form the common bile duct
(CBD). (b) Medial insertion of cystic duct onto the common hepatic
duct. (c) Absent cystic duct with fusion of the gallbladder neck with the
mon variants include low insertion, medial insertion, and a par-
allel course (≥2 cm segment) with the common hepatic duct
(Fig. 12.40). Several less common anatomic variants can also
occur, usually consisting of an aberrant and/or accessory duct.
An aberrant duct has an anomalous confluence pattern and is the
only duct draining a particular segment. An accessory duct is a
supernumerary duct that drains a particular segment of the liver
in addition to the normal duct [112].
Congenital Anomalies
Choledochal Cysts
Choledochal cysts are a diverse group of congenital abnor-
malities that present as focal or diffuse dilation of the biliary
tree. Up to two-thirds of reported cases occur in Asia where
they are considerably more common, with an incidence as
high as 1 in 1000 in Japan [113]. They are more common in
457
c
f
common hepatic duct. (d) Low insertion of cystic duct onto common
hepatic duct. (e) Parallel course of cystic duct and common hepatic duct
(≥2 cm segment). (f) Aberrant or accessory right posterior hepatic duct
drains into the cystic duct
females with a female-to-male ratio of 3:1 to 4:1. Patients
usually present with abdominal pain and jaundice [114].
Although most choledochal cysts are detected early in life,
about 20% of patients are diagnosed in adulthood when
undergoing evaluation for biliary tract symptoms [115].
The most commonly used Todani classification scheme
separates choledochal cysts into five types: type I (50%–
80%), a fusiform dilation of the CBD, the most common
form; type II (2%), a diverticulum of the CBD; type III
(1.4%–4.5%), a choledochocele, consisting of a diverticu-
lum of the intraduodenal portion of the CBD; type IV (15%-
35%), multiple communicating intra- and extrahepatic bile
duct cysts that can be divided into 2 subtypes: IVa, multiple
intrahepatic and extrahepatic bile duct cysts; and IVb, cysts
confined to the extrahepatic biliary tree; and type V (20%),
Caroli disease (Fig. 12.41) [116].
Since Caroli disease has a different embryological origin,
it is considered by many to not represent a true choledochal
458
Liver
Gallbladder
Cyst
Type I Type II
Type IVa Type IVb
Fig. 12.41 Diagram of Todani choledochal cyst classification. Type I –
fusiform or cystic dilation of the common hepatic and common bile ducts.
Type II – diverticulum of the common bile duct. Type III – choledochocele
in the duodenal wall at the pancreaticobiliary junction. Type IVa –
cyst, and will be discussed separately. Type I and type IVa
choledochal cysts are associated with an abnormally long
common channel (>20 mm) between the distal CBD and the
pancreatic duct. The long common channel may permit the
reflux of pancreatic secretions into the CBD, causing mural
weakness and dilation. This theory remains controversial.
On ultrasound evaluation, a type I cyst appears as a well-­
defined, fluid-filled structure in the porta hepatis that is in con-
tinuity with the extrahepatic bile duct and separate from the
gallbladder. There is associated central intrahepatic bile duct
dilation in approximately 50% of patients. A type II cyst
appears as an eccentric outpouching of the CBD. A type III
cyst (choledochocele) manifests as a fluid-filled mass protrud-
ing into the wall of the duodenum. Type IV cysts appear as
numerous intra- and extrahepatic cysts (Figs. 12.42, 12.43,
C. L. Carlson et al.
Common
hepatic duct
Duodenum
Type III
Type V
multiple intrahepatic and extrahepatic bile duct cysts; Type IVb – cysts
confined to the extrahepatic biliary tree. Type V (Caroli disease) – one or
more intrahepatic cysts without extrahepatic cysts
and 12.44, Cineclip 12.4). When there is associated biliary
atresia, intrahepatic ductal dilation is usually absent.
Although ultrasound is a good screening tool for evaluat-
ing biliary ductal dilation, MRCP is the current reference
standard for initial evaluation and diagnosis of choledochal
cysts. MRCP techniques can accurately assess intra- and
extrahepatic biliary ductal anatomy, evaluate the pancreati-
cobiliary junction, and assess for complications [113].
Choledochal cysts can be associated with complica-
tions related to biliary stasis, including biliary stones,
cholangitis, and pancreatitis. They may coexist with bili-
ary atresia. There is also an increased risk of biliary
malignancy, with cholangiocarcinoma the most common
associated tumor. The risk of malignancy increases with
patient age [113].
12 Gallbladder and Biliary Tract
a b
Fig. 12.42 Type I choledochal cyst in a 17-year-old female with
abdominal pain. (a) Longitudinal grayscale ultrasound image shows
fusiform dilation (calipers) of the CBD. (b) Magnetic resonance chol-
Type I choledochal cysts are surgically managed with
complete cyst excision and Roux-en-Y hepaticojejunos-
tomy or hepaticoduodenostomy reconstruction. Surgical
management of type II cysts includes complete cyst exci-
sion with Roux-en-Y hepaticojejunostomy or primary clo-
sure over a T-tube. Treatment of type III cysts may include
endoscopic retrograde cholangiopancreatography (ERCP)
with sphincterotomy in cysts smaller than 2 cm, or cyst
excision with sphincteroplasty.
Management of type IV cysts includes complete extrahe-
patic cyst excision with biliary-enteric anastomosis because
of the potential for the development of malignancy. Complete
resection is not always possible. If only the left hepatic lobe
is involved, a left hepatic lobectomy is performed. If there is
diffuse intrahepatic disease, intrahepatic cyst drainage with
bilateral stenting is recommended for alleviation of biliary
stasis and associated complications [113].
Caroli Disease
Caroli disease is characterized by multifocal segmental dila-
tion of the intrahepatic bile ducts. Unlike the other types of
459
angiopancreatography (MRCP) 3D coronal maximum intensity projec-
tion (MIP) image demonstrates dilation of the CBD (arrowhead) that
tapers near the pancreatic head
choledochal cyst, it is a known congenital malformation of the
ductal plate, with a presumed autosomal recessive inheritance.
Patients often present in adolescence or early adulthood with
recurrent cholangitis, abdominal pain, or jaundice. Caroli dis-
ease affects the large intrahepatic bile ducts. Abnormal devel-
opment of small interlobular bile ducts results in congenital
hepatic fibrosis.
If all levels of the biliary tree are affected, features of both
Caroli disease and congenital hepatic fibrosis are present, a
condition sometimes referred to as Caroli syndrome.
Occasionally the disorder manifests later with signs of portal
hypertension and cirrhosis.
Many complications are due to bile stasis, including bili-
ary tract stones, recurrent cholangitis, hepatic abscesses, and
intrahepatic biliary strictures. Patients also have an increased
risk of developing cholangiocarcinoma. Caroli disease is
associated with renal abnormalities, including autosomal
recessive polycystic kidney disease, medullary sponge kid-
ney, and medullary cystic disease.
Ultrasound imaging of Caroli disease will depict saccular
or fusiform dilation of the intrahepatic bile ducts (Fig. 12.45).
460
a b
Fig. 12.43 Type II choledochal cyst in a 3-month-old female. (a)
Longitudinal grayscale ultrasound image shows a large cystic mass
(arrowhead) extending inferiorly from the hilum of the liver (L). (b)
Axial T2-weighted, fat-suppressed MR image shows a lobulated, cystic
structure (arrowhead) posteromedial to the liver. (c) Image from a hepa-
ERCP and hepatobiliary phase contrast-enhanced MR imag-
ing will confirm communication with the biliary tree. On
MRCP, Caroli disease appears as multiple T2 hyperintense
cystic dilations of the intrahepatic bile ducts.
A feature sometimes seen on cross-sectional imaging
and occasionally by ultrasound, is the “central dot” sign,
where a dilated portion of the bile duct surrounds the
adjacent hepatic artery and portal vein, producing an
C. L. Carlson et al.
tobiliary iminodiacetic acid (HIDA) scan shows accumulation of radio-
tracer (arrowhead) within the lesion in keeping with a connection to the
biliary tree. (d) Intraoperative cholangiogram shows the cyst arising as
a diverticulum (arrowhead) from the biliary tree
echogenic focus or dot in the center of the dilated duct
(Fig. 12.46).
With focal hepatic involvement, a lobectomy can be per-
formed. Management of Caroli disease is difficult when
there is diffuse intrahepatic involvement. Initially, a conser-
vative approach is favored with cyst drainage, stone removal,
and antibiotics. Liver transplantation is the definitive treat-
ment for diffuse hepatic involvement [113].
12 Gallbladder and Biliary Tract
Fig. 12.44 Type IVa choledochal cyst in a 5-year-old female. (a) Transverse
grayscale ultrasound image of the liver shows marked dilation (arrowheads)
of the intrahepatic bile ducts. (b) Longitudinal grayscale ultrasound image
of the porta hepatis shows marked dilation (arrowheads) of the extrahepatic
bile ducts with tapering (arrow) at the level of the pancreatic head.
P, Pancreas. (c) Intraoperative endoscopic retrograde cholangiopancreatog-
461
raphy (ERCP) image confirms marked intrahepatic (arrowheads) and extra-
hepatic (arrow) bile duct dilation. (d) Axial T2-weighted, fat-suppressed
MR image shows marked dilation of the intrahepatic bile ducts with associ-
ated intraluminal hypointense defects (arrowheads) compatible with stones.
(e) Coronal MRCP image shows marked dilation of the CBD (arrow) with
intraluminal hypointense defects (arrowheads) reflecting stones
462 C. L. Carlson et al.

a
L

Fig. 12.46 Caroli syndrome in a 5-year-old female. Transverse gray-

scale ultrasound image shows multiple hepatic cysts containing a “cen-
tral dot” sign (arrowheads) representing intrahepatic portal venous
branches surrounded by dilated bile ducts. The coarse appearance of the
liver parenchyma (L) reflects the presence of fibrosis

Biliary Tract Obstruction

Biliary Atresia
c tree leads to bile duct obstruction. If untreated, progressive
Fig. 12.45 Caroli disease in a 6-month-old male with autosomal reces-
sive polycystic disease. (a) Transverse grayscale ultrasound image of the
liver (L) shows cystic dilation of the intrahepatic biliary ducts containing
the “central dot” sign (arrowheads) representing intrahepatic portal
venous branches surrounded by dilated bile ducts. (b) Transverse color
Doppler ultrasound image shows flow (arrowhead) within a portal
venous branch surrounded by a dilated bile duct (arrow). (c) Longitudinal
grayscale ultrasound image shows an enlarged, echogenic kidney (cur-
sors) containing numerous microcysts. There is loss of normal cortico-
medullary differentiation
Biliary atresia is a rare destructive, inflammatory condition
of unknown etiology where progressive fibrosis of the biliary
liver cirrhosis results in death by 2 years of age. Recent prev-
alence estimates for biliary atresia in Western countries are
in the range of 0.5–0.8 per 10,000 live births.
Biliary atresia is often classified into three types, depend-
ing on the level of biliary obstruction (Fig. 12.47). In type I
there is obliteration of the CBD with a patent cystic duct and
common hepatic duct, accounting for about 5% of cases.
Type II is subdivided into types IIa and IIb. Type IIa is char-
acterized by obliteration of the common hepatic duct with a
patent cystic duct and CBD, sometimes with a cyst at the
hilum of the liver (hence the term “cystic biliary atresia”).
Type IIb is characterized by obliteration of the common
hepatic duct, cystic duct, and CBD. Together types IIa and
IIb account for approximately 2% of cases.
In biliary atresia types I and II, there is some preservation
of the intrahepatic ducts although they typically do not dilate
despite the presence of distal obstruction because of their
intrinsic abnormality. Biliary atresia type III involves oblit-
eration of the left and right main hepatic ducts at the level of
12 Gallbladder and Biliary Tract 463

Liver Liver
I IIA
CHD CHD
GB GB
CBD CBD
Bowel Bowel
Liver Liver
IIB III
CHD CHD
GB GB
CBD
CBD
Bowel Bowel
Fig. 12.47 Diagram of the Kasai classification of biliary atresia. The
hatched markings indicate obliteration. CBD, Common bile duct; CHD,
common hepatic duct; GB, gallbladder.
Type I: obliteration of CBD with patent cystic duct and CHD.Type II:
IIa – obliteration of CHD with patent cystic duct and CBD, sometimes
with a cyst at hilum (i.e., “cystic biliary atresia”). IIb – obliteration of
CHD, cystic duct, and CBD. Type III: obliteration of left and right main
hepatic ducts at level of porta hepatis, and of cystic duct and CBD (most
common, > 90%)
the porta hepatis, the cystic duct, and CBD, and accounts for
more than 90% of cases.
On abdominal exploration, a dense inflammatory proxi-
mal remnant is noted at the porta hepatis. The gallbladder is
usually small or absent, but may have a small lumen [117].
Approximately 10–20% of patients with biliary atresia
have associated congenital abnormalities, including chole-
dochal cyst, congenital heart defects, heterotaxy with poly-
splenia, azygous continuation of the inferior vena cava, and
preduodenal portal vein [118].
An infant with suspected biliary atresia typically presents
with persistent jaundice lasting longer than 2 weeks after birth,
acholic stools, and dark urine. Laboratory findings include
conjugated hyperbilirubinemia and elevated transaminases,
alkaline phosphatase, and c-glutamyl transpeptidase.
Ultrasound usually reveals a shrunken, atrophic gallblad-
der, although approximately 20% of affected children may
have what seems to be a normal gallbladder that at operation
is noted to be a gallbladder mucocele. The “triangular cord”
sign represents the fibrous ductal remnant of the extrahepatic
bile duct in biliary atresia. This sign has been described as a
thickness of 4 mm or more of echogenic soft tissue along the
anterior wall of the right portal vein on an oblique longitudi-
nal ultrasound image (Fig. 12.48, Cineclip 12.5).
The “triangular cord” sign and gallbladder abnormalities
are the most accurate ultrasound signs used to diagnose or
exclude biliary atresia with a reported combined sensitivity of
95% and specificity of 89% [119]. The main hepatic artery is
often enlarged, and there is frequently increased hepatic sub-
capsular flow [120, 121]. Other features, such as ­polysplenia
or situs inversus, may be present (Fig. 12.49).
Additional imaging tests include hepatobiliary techne-
tium (Tc)-99m-iminodiacetic acid (HIDA) scanning that will
reveal rapid uptake of the radiotracer by the liver with an
absence of gut excretion. If there is hepatic dysfunction
related to biliary atresia or hepatic parenchymal disease from
another cause, there may be delayed uptake of the radioiso-
tope and no gut excretion. Excretion into the gut, however,
effectively rules out biliary atresia.
Percutaneous liver biopsy has a diagnostic accuracy of
90% to 95%. Histology reveals preservation of the hepatic
architecture with bile duct proliferation, bile plugs, and peri-
portal fibrosis [117].
At surgery, a cholangiogram is performed to confirm
the diagnosis of biliary atresia, followed by a Kasai porto-
enterostomy. If the Kasai procedure fails, liver transplan-
tation is required [117].
 eonatal Hepatitis Syndrome
N
Neonatal hepatitis syndrome is a term used for hepatic inflam-
mation presenting within the first 1 or 2 months of life after
structural disorders resulting in prolonged neonatal cholesta-
sis have been excluded. The term emphasizes the uniform
clinical phenotype of the many infectious (e.g., TORCH),
genetic, toxic, and metabolic causes (e.g., α1-antitrypsin defi-
ciency, galactosemia, glycogen storage disease, and tyrosin-
osis) that can cause impaired bile secretion and excretory
function.
A diagnosis of idiopathic neonatal hepatitis is made once
specific causes of prolonged neonatal cholestasis have been
excluded. With advances in biochemical and genetic diagno-
ses of specific inherited and metabolic conditions, the num-
ber of patients with idiopathic neonatal hepatitis has
significantly decreased [122, 123]. The prevalence of idio-
pathic neonatal hepatitis is approximately 1 in 2500 live
births and represents about 25% of patients with prolonged
neonatal cholestasis, similar to the prevalence of biliary atre-
sia. Other causes include infection, TPN, metabolic defects,
and perinatal hypoxia/ischemia [122]. Clinical features of
cholestasis and jaundice usually manifest at 3–4 weeks of
life.
464
Fig. 12.48 Biliary atresia in a 7-week-old female with jaundice and
conjugated hyperbilirubinemia. (a) Sagittal grayscale ultrasound image
reveals no evidence of a gallbladder in the expected location. There is
prominent echogenic soft tissue in the porta hepatis (between arrows). (b)
Oblique longitudinal grayscale ultrasound image of the right portal vein
reveals adjacent soft tissue thickening (arrowhead) of greater than 4 mm
C. L. Carlson et al.
in keeping with a “triangular cord” sign. Asterisk, Right portal vein. (c)
Serial images from a hepatobiliary iminodiacetic acid (HIDA) scan
acquired over an hour show prompt hepatic uptake of radiotracer without
excretion into the biliary tree or gastrointestinal tract. There is radiotracer
excretion via the kidneys with visualization of the bladder (arrowhead).
(Fig. 12.48 continues)
12 Gallbladder and Biliary Tract
Fig. 12.48 (continued) (d) Four-hour anterior and posterior views show
no evidence of delayed biliary excretion of radiotracer. (e) Intraoperative
As with biliary atresia, liver size and echogenicity may be
normal or increased in neonatal hepatitis. Ultrasound and
other imaging modality findings are nonspecific and often
normal (Fig. 12.50) [123]. The gallbladder may be small,
normal, or large. A change in gallbladder size after feeding
favors neonatal hepatitis over biliary atresia.
Definitive treatment for the underlying cause of neonatal
hepatitis syndrome should be started as soon as a specific
etiology is determined.
465
transhepatic cholangiogram reveals no evidence of a gallbladder with an
interstitial injection (arrow) of contrast material
Alagille Syndrome
Alagille syndrome (arteriohepatic dysplasia or syndromic
intrahepatic bile duct paucity) is an autosomal dominant
multisystemic disorder with variable penetrance caused by
heterozygous mutations in the JAG1 and NOTCH2 genes of
the Notch signaling pathway. Prevalence varies from
1:30,000 to 1:100,000 live births [124, 125]. It is the most
common familial intrahepatic cholestatic syndrome,
although cases can also be sporadic. Pathologically there is
466 C. L. Carlson et al.

a b

L S

Fig. 12.49 Biliary atresia in a 20-year-old female with heterotaxy and
liver cirrhosis. A Kasai procedure was performed in early infancy. (a)
Transverse grayscale ultrasound image of the mid-abdomen shows a
dysmorphic, malpositioned liver (L) with heterogeneous parenchyma.
The main portal vein (arrowhead) is tiny. (b) Transverse color Doppler
ultrasound image of the right flank reveals a dymorphic spleen (S).
There are numerous enlarged vessels posterior to the spleen (arrows) in
keeping with varices. (c) Longitudinal color Doppler ultrasound image
of the liver (L) shows no intrahepatic inferior vena cava. An enlarged
azygous vein (arrowhead) is identified posterior to the liver
12 Gallbladder and Biliary Tract 467

a b

c d

Fig. 12.50 Idiopathic neonatal hepatitis in a 7-week-old male with
cholestasis. (a) Longitudinal grayscale ultrasound image of the liver
shows a collapsed but otherwise normal appearance of the gallbladder
(arrow). (b) Oblique longitudinal grayscale image of the porta hepatis
shows a normal CBD (cursors). (c) Serial images from a HIDA scan
acquired over an hour show delayed hepatic uptake of radiotracer with
persistence of tracer in the blood pool and increased activity in the
heart, urinary bladder, and soft tissues indicative of hepatocellular
­dysfunction. (d) 22-hour anterior view demonstrates mild activity in the
right upper abdomen inferior to the liver (arrow) that represents colonic
excretion of radiotracer
468
usually a paucity of intrahepatic bile ducts. Hepatic involve-
ment is not always present, with cholestasis reported in 89%
and bile duct paucity in 75% [124].
Diagnosis requires three of seven major clinical features or
congenital anomalies which include the following: 1 – cardiac
defects (peripheral pulmonic stenosis, tetralogy of Fallot), 2 –
hepatic manifestations (cholestasis, hyperbilirubinemia, pruri-
tus, xanthomas, cirrhosis), 3 – renal abnormalities (dysplasia,
renal tubular acidosis), 4 – skeletal abnormalities (butterfly/
hemivertebrae, pathologic fractures), 5 – ophthalmologic mani-
festations (posterior embryotoxon, optic disc drusen), 6 – dys-
morphic facies (frontal bossing, deep-set eyes, hypertelorism,
prominent ears, triangular face with pointed chin, and broad
a b
c d
Fig. 12.51 Alagille syndrome in a 3-year-old female with severe chronic
cholestasis. (a) Oblique longitudinal grayscale ultrasound image shows a
tiny gallbladder (arrow). (b) Oblique longitudinal grayscale ultrasound
image reveals a normal CBD (cursors). (c) Serial images from a HIDA
scan acquired over an hour show delayed hepatic uptake of radiotracer
C. L. Carlson et al.
nasal bridge with bulbous tip nose), and 7 – vascular abnormali-
ties (intracranial hemorrhage, aneurysm, moyamoya, systemic
anomalies of the aorta, renal, celiac, mesenteric, and subclavian
arteries) [126].
Other associated features include short stature, failure to
thrive, immunodeficiency/recurrent infection, developmen-
tal delay, delayed puberty, and s­upernumerary flexion
creases. Most patients present with jaundice or cardiac-
related symptoms in the newborn period.
Ultrasound features of Alagille syndrome can include an
absent or small, abnormally shaped gallbladder and bile duct
paucity (Fig. 12.51). Absence of both the “triangular cord”
sign and an enlarged hepatic artery can help to differentiate
with persistence of tracer in the blood pool and increased activity in the
heart, urinary bladder, and soft tissues indicative of hepatocellular dys-
function. Bowel activity is noted although gallbladder activity is not defin-
itively identified. (d) Image from an ERCP shows a small gallbladder
(arrowhead) with filling of several diminutive left-sided bile ducts (arrow)
12 Gallbladder and Biliary Tract 469

Alagille syndrome from biliary atresia. Less commonly,

a
findings of cholestasis, portal hypertension, and cirrhosis
may be seen [124–128].
The prognosis and treatment of Alagille syndrome depends
on organ system involvement and disease severity. Treatment of
liver disease is mainly supportive, with use of agents that will
improve the flow of bile. The Kasai procedure used for patients
with biliary atresia does not benefit children with Alagille syn-
drome and may worsen outcome. Liver transplantation for end-
stage liver disease has an 80% five-year survival rate, with
improving liver function and catch-up growth [129].

Byler Disease
Byler disease (progressive familial intrahepatic cholestasis,
or PFIC) is an autosomal recessive disorder initially
described in an Amish kindred [130]. The disease is now
classified into three subgroups. All three types of PFIC are b
caused by defects in bile secretion from the hepatocyte to the
canaliculi. In PFIC-1 and PFIC-2, bile acid secretion is
abnormal, while in PFIC-3, bile phospholipid secretion is
impaired. Histologically, there is cholestasis, bile duct
proliferation, and fibrosis [130, 131]. Symptoms usually
­
develop by 1 year of age and include jaundice and hepato-
megaly. The disorder may progress to cirrhosis.
On ultrasound, there are multiple saccular cystic lesions,
some of which demonstrate central echogenic foci (a “cen-
tral dot” sign). The dot represents a portal venous branch
surrounded by fluid. The cysts in Byler disease do not com-
municate with the bile ducts, a feature that distinguishes it
from Caroli disease [132].
Medical therapy aims to enhance bile flow, inhibit the
accumulation of metabolites, avoid malabsorption of fat-­
soluble vitamins, prevent malnutrition and growth restric-
Fig. 12.52 Choledocholithiasis in a 7-month-old male with short
tion, relieve pruritus, and slow disease progression. However, bowel syndrome born at 24 weeks of gestation. A previous ultrasound
medical treatment commonly fails, and surgical options study showed stones and sludge in the gallbladder. (a) Oblique longitu-
include biliary diversion, ileal bypass, and liver transplanta- dinal grayscale ultrasound image reveals an echogenic stone (arrow)
with posterior acoustic shadowing (arrowhead) in the dilated CBD
tion [130, 131].
(asterisk). (b) Oblique longitudinal color Doppler ultrasound image
demonstrates a twinkling artifact (arrowhead) associated with the stone
Choledocholithiasis
Choledocholithiasis, or CBD stones, usually occurs when Leukocytosis and cholestatic enzymes (alkaline phosphatase
gallbladder stones migrate distally into the CBD. Less often, and gamma-­glutamyl transferase [GGT]) may be elevated.
stones form in the CBD secondary to infection. Once consid- Ultrasound demonstration of an echogenic shadowing
ered rare in children, choledocholithiasis has become more stone or stones in the CBD confirms the diagnosis of choledo-
common in recent years [64]. This may be due, at least in cholithiasis (Fig. 12.52). There is often associated intrahepatic
part, to increasing obesity and body mass index (BMI) in the biliary ductal dilation. Overlying bowel gas and an echogenic
pediatric population [133]. Most common duct stones cause pancreatic head can make detection of a distally impacted
obstruction in the pancreatic head, but obstruction can occur stone more difficult. Scanning performed with the patient in
anywhere within the CBD. an erect or oblique position may aid in detection [64]. MRCP
Children present with jaundice, with or without pain, and is useful if suspected stones are not detected by ultrasound or
can develop symptoms similar to cholecystitis, with right if the extent of stones is presumed to be greater than what is
upper quadrant pain and fever. Less common presentations depicted on ultrasound. Endoscopic ultrasound is a more inva-
include pancreatitis (“gallstone pancreatitis”) and cholangitis. sive modality that can also be used in diagnosis.
470
ERCP is being used more often in children and infants for
both diagnosis of choledocholithiasis and treatment with stone
extraction [64]. Stent placement, lithotripsy, and surgery are
other interventions that can be performed if the obstruction is
not relieved by ERCP.
I nspissated Bile Syndrome
Inspissated bile syndrome, or bile plug syndrome, is a rare
cause of jaundice in neonates due to sludge within the intra-
or extrahepatic bile ducts associated with partial or complete
biliary ductal obstruction. Inspissated bile syndrome may be
a b
Fig. 12.53 Inspissated bile syndrome in a 10-day-old boy. (a)
Longitudinal grayscale ultrasound image demonstrates a sludge-filled
gallbladder (arrow) and sludge-filled, dilated CBD (arrowheads). (b)
Image from a diagnostic cholangiogram reveals multiple filling defects
C. L. Carlson et al.
associated with massive hemolysis due to Rh incompatibility,
hemorrhage (intraabdominal, retroperitoneal, or intracranial),
and increased enterohepatic circulation associated with a
number of intestinal diseases (Hirschsprung disease, intesti-
nal atresia, and stenosis). Patients commonly present with
jaundice, acholic stools, worsening liver function tests, dark
urine, non-bilious vomiting, and failure to thrive [134].
Ultrasound imaging shows dilated intra- and extrahepatic
bile ducts with echogenic intraluminal bile and fluid-debris
levels without acoustic shadowing. Sludge can also be seen
within the gallbladder (Fig. 12.53) [135].
(arrowheads) within the gallbladder and dilated CBD consistent with
sludge. (c) After flushing with saline, there is clearance of the sludge
from the gallbladder and CBD, with normal drainage of contrast mate-
rial into the duodenum (arrow)
12 Gallbladder and Biliary Tract 471

Inspissated bile syndrome usually resolves after treatment

a
of the underlying disorder, hydration, and other conservative
measures. If obstruction persists, more invasive treatment may
be required, such as open or laparoscopic cholecystectomy,
ERCP with sphincterotomy, percutaneous transhepatic chol-
angiographic saline flushing, or infusion of N-acetylcysteine
into the extrahepatic bile ducts to prevent recurrent cholangitis
and pancreatitis [134, 135].

Sclerosing Cholangitis
Sclerosing cholangitis is a chronic inflammatory disorder
characterized by progressive obliterative fibrosis of the intra-
and extrahepatic bile ducts that can progress to portal hyper-
tension and biliary cirrhosis.
Four main clinical types of sclerosing cholangitis have
been described: neonatal sclerosing cholangitis, sclerosing
cholangitis with autoimmune features, sclerosing cholangitis b
without autoimmune features and of unknown etiology (pri-
mary sclerosing cholangitis), and sclerosing cholangitis asso-
ciated with a variety of diseases, including Langerhans cell
histiocytosis and immunodeficiencies [136]. Most children
with sclerosing cholangitis either have autoimmune hepatitis,
inflammatory bowel disease, or both.
Histological features include thickening of the bile duct
walls and diverticula or ectasia between segmental strictures.
Patients present with jaundice, right upper quadrant pain,
and laboratory findings of cholestasis.
Ultrasound abnormalities include thick-walled, dilated
intrahepatic ducts with intervening focal narrowing due to
stricture formation (Fig. 12.54). Intraductal stones, choleli-
thiasis, and gallbladder wall thickening may also be seen
along with findings of portal hypertension and cirrhosis in
patients with longstanding disease.
The prognosis of sclerosing cholangitis in children is
Fig. 12.54 Sclerosing cholangitis in a 17-year-old female with ulcer-
poor. Liver disease invariably progresses to biliary cirrhosis, ative colitis. (a) Longitudinal grayscale ultrasound image shows a
and the overall median survival without liver transplantation mildly dilated CBD (asterisk) with an abnormally thickened and
is about 10–13 years from the apparent onset of disease. slightly irregular wall (arrowhead). (b) Coronal T2-weighted, fat-sup-
Due to its beneficial effect in adults, ursodeoxycholic pressed MIP MR image demonstrates beading of the intrahepatic bile
ducts (arrowheads) and cystic duct (white arrow). The CBD (black arrow)
acid (UDCA), a hydrophilic bile acid that enriches the bile is slightly dilated
acid pool and decreases the biliary saturation of c­ holesterol,
is also administered in childhood, although its efficacy in
the pediatric population has not yet been established. Liver chronic inflammation. A fistula between the gallbladder and
transplantation is required for the 20–30% of children with the common hepatic duct or CBD can also occur. Clinical
sclerosing cholangitis who develop end-stage liver disease presentation is usually nonspecific, with findings of obstruc-
[137]. tive jaundice. Occasionally a patient may present with gall-
stone ileus.
Mirizzi Syndrome Ultrasound findings of Mirizzi syndrome include shad-
Mirizzi syndrome is a rare complication of gallstone disease owing stone(s) in the cystic duct with dilated ducts down to
where a gallstone becomes impacted in the neck or cystic the level of the common hepatic duct, and acute or chronic
duct of the gallbladder leading to compression of the com- cholecystitis. ERCP is the reference standard diagnostic
mon hepatic duct and resulting in obstruction and jaundice. technique as it provides superior visualization of the extrahe-
The obstructive jaundice can be caused either by direct patic bile ducts, clearly demonstrates extrinsic compression
extrinsic compression by the stone or from fibrosis caused by of the common hepatic duct and CBD by impacted gall-
472
stones, as well as the presence and location of biliary obstruc-
tion and fistulas. Therapeutic intervention such as stent
placement or nasal bile drainage can also be performed dur-
ing ERCP prior to definitive treatment with cholecystectomy
[138, 139].
 ile Duct Stricture
B
Bile duct strictures are rare in children. The most common
etiology of a localized benign biliary stricture is an iatro-
genic injury after cholecystectomy or a bile duct procedure.
Less frequent causes include choledocholithiasis, chronic
pancreatitis, and trauma. As discussed earlier in this chapter,
biliary tract strictures in primary sclerosing cholangitis are
usually multiple, although there have been reports of chil-
dren presenting with isolated lesions at the confluence of the
hepatic ducts [140].
Patients may present with jaundice, pruritus, and dark
urine from renal excretion of bilirubin. Generalized symp-
toms such as weight loss, fever, nausea, vomiting, and mal-
aise can also occur; and depend on the underlying cause.
Severe biliary obstruction can be complicated by ascending
cholangitis, hepatic abscesses, and gram-­negative septicemia
[141].
Ultrasound findings of bile duct stricture include thicken-
ing and narrowing of the strictured duct with proximal dila-
tion. In general, computed tomography (CT), MRCP, and
ERCP are better at imaging the morphology of strictures
than ultrasound [142]. Treatment options include stricture
excision, biliary reconstruction, or both.
AIDS Cholangiopathy
Acquired immune deficiency syndrome (AIDS) cholangiop-
athy is an inflammatory process that affects the biliary tree in
the advanced stages of human immunodeficiency virus
(HIV) infection. It is usually caused by an opportunistic
infection, with Cryptosporidium parvum and cytomegalovi-
rus the most common pathogens. Patients present with severe
epigastric or right upper quadrant pain, cholestasis, and sig-
nificantly elevated serum alkaline phosphatase with a normal
bilirubin level [143, 144]. The incidence of AIDS cholangi-
opathy has significantly decreased since the initiation of
potent antiretroviral therapy in the mid-1990s.
Ultrasound findings of AIDS cholangiopathy in children
include diffuse thickening of the gallbladder wall and of the
intrahepatic and extrahepatic bile ducts with focal strictures
and dilation indistinguishable from those of primary scleros-
ing cholangitis. There is CBD dilation caused by an inflamed
and stenotic papilla of Vater. The inflamed papilla itself can
be seen as an echogenic n­ odule protruding into the distal
duct [144–146]. Following screening ultrasound examina-
C. L. Carlson et al.
tion, MRCP can provide more detailed visualization of the
intra- and extrahepatic biliary tract abnormalities [147].
Treatment of AIDS cholangiopathy includes antiretro-
viral therapy and endoscopic decompression sphincterot-
omy [143].
 pontaneous Perforation of the Extrahepatic
S
Bile Ducts
Spontaneous perforation of the extrahepatic bile ducts in
children is rare, but most often occurs in early childhood,
with a peak incidence during the first year of life. The most
common location for perforation is at the junction of the cys-
tic duct and common hepatic duct [148, 149]. Most cases are
idiopathic, although some are associated with pancreatico-
biliary malunion or distal CBD obstruction by stones or atre-
sia. The pathogenesis is uncertain [148–150].
Older children usually present acutely with peritonitis or
sepsis whereas younger children tend to present less acutely
with abdominal distension, failure to thrive, and jaundice
with acholic stools. A history of biliary tract disease is usu-
ally absent, helping to distinguish bile duct perforation from
other causes of peritonitis [148, 149].
Ultrasound imaging shows free fluid or loculated ascites.
Biliary ductal dilation is usually absent due to decompres-
sion after perforation. MR imaging with hepatocyte-specific
contrast may show leakage of contrast from the biliary tree
with accumulation in the porta hepatis, or a biloma.
Hepatobiliary scintigraphy can demonstrate bile leakage into
the peritoneum, but lacks anatomic specificity and exposes
the child to radiation [148–150].
Treatment of a bile leak with percutaneous or surgical
drainage is effective in 85% of patients. A persistent biliary
fistula should prompt evaluation for distal duct obstruction.
Biliary-enteric anastomosis is reserved for the rare bile duct
stricture or non-healing fistula [148].
Biliary Tract Trauma
Intra- or extrahepatic biliary tract injury from blunt or pene-
trating trauma is very rare in children. Intrahepatic ductal
injury may accompany hepatic injury. Due to a more conser-
vative approach to pediatric hepatic and other solid organ
abdominal injury in recent years, diagnosis of biliary tract
injuries is often delayed [101, 151].
Injuries encompass biliary tract contusion, puncture, par-
tial or complete transection, or avulsion. Rising bilirubin lev-
els or a biloma should prompt diagnostic evaluation for
biliary tract injury following abdominal trauma. Fever,
12 Gallbladder and Biliary Tract
abdominal pain and distension, prolonged ileus, generalized
abdominal tenderness, and guarding are the most common
symptoms associated with biliary tract injury [101, 151].
Ultrasound findings include free or contained peri- or
intrahepatic fluid collections and ascites. Large fluid collec-
tions outside of the gallbladder fossa should raise concern
for bile duct injury. Hepatobiliary scintigraphy can help
detect active bile leaks by demonstrating accumulation of
radiotracer in the abdomen. MRCP with hepatobiliary con-
trast agents provides a noninvasive means of evaluating the
biliary tract without radiation exposure and can often reveal
very detailed anatomical information to aid in treatment
planning [101, 151–153].
The majority of minor ductal injuries can be effectively
treated with laparoscopic and percutaneous drainage alone.
Major ductal injuries require ERCP, sphincterotomy, and
biliary stenting with or without percutaneous drainage.
Complete bile duct disruption requires hepaticojejunostomy
for definitive repair [101, 151].
Tumors
Benign Masses
Granular Cell Tumor
Granular cell tumor, the most common nonepithelial tumor
of the extrahepatic bile ducts, is a benign solitary or multi-
centric mesenchymal tumor which is extremely rare in chil-
dren, with young African-American females most often
affected [154–156]. The clinical presentation depends on
tumor location, ranging from biliary colic from cystic duct
obstruction to jaundice, abdominal pain, weight loss, and
other nonspecific symptoms from hepatic duct or CBD
obstruction. There is often an associated biliary ductal
stricture.
Ultrasound reveals an intraductal, immobile, echogenic,
non-shadowing mass with dilation of the biliary tree proxi-
mal to the obstructing lesion [155, 157]. Unless the lesion is
large, color Doppler imaging does not reveal significant vas-
cularity. Preoperative MR imaging, endoscopy, and percuta-
neous cholangiography often show a smooth circumferential
narrowing of the involved bile ducts.
Surgical resection of the lesion is curative and may require
choledochojejunostomy, choledochoduodenostomy, or hepat­
icojejunostomy. Pancreaticoduodenectomy is required for
lesions in the pancreatic segment of the common bile duct [155].
Bile Duct Adenoma
Bile duct adenoma is an indolent, benign epithelial tumor
that is extremely rare in children. Lesions may be single or
multiple, intra- or extrahepatic (including the cystic duct and
473
ampulla of Vater). Children with familial adenomatous
­polyposis, Lynch syndrome, and Peutz–Jeghers syndrome
have an increased risk for adenoma at the ampulla.
Bile duct adenoma is composed of an aggregate of non-
cystic bile ductules with small or inapparent lumens lined by
cuboidal epithelium with bland, regular nuclei. Mitoses,
cytologic atypia, and nuclear pleomorphism are absent.
Papillary adenoma is the most common type of bile duct
adenoma. A recent molecular classification divides papillary
adenomas into cuboidal and columnar cell types, similar to
intraductal papillary mucinous neoplasms of the pancreas.
Although bile duct adenomas are benign with low mitotic
activity, they do have the potential for malignant transforma-
tion. When in an extrahepatic location they often cause
obstructive jaundice as well as abdominal pain, vomiting,
pruritus, and weight loss [158–161].
Lesions vary in size from 1 to 20 mm. Their appearance
on ultrasound is variable, ranging from hypoechoic to mixed
echogenicity to a hyperechoic intrahepatic or extrahepatic
mass with or without a hypoechoic halo. Depending on their
location, ultrasound may show dilation of the intra- and
extrahepatic biliary tree and pancreatic duct. Contrast-­
enhanced ultrasound shows arterial enhancement with slow
washout during the late venous phase. CT and MR imaging
reveal similar hyperenhancement on arterial and portal venous
phases that persists on the late venous and delayed phases
[161–163].
Surgical resection is required due to the malignant poten-
tial of bile duct adenoma. The prognosis is good following
resection [160].
Malignant Bile Duct Tumors
Rhabdomyosarcoma of the Bile Duct
Biliary tract rhabdomyosarcoma is rare, but is the most com-
mon tumor of the biliary tree in children, and the most fre-
quent cause of malignant obstructive jaundice in childhood
[164, 165]. Mean age at diagnosis is approximately 3 years,
with the majority of patients presenting before the age of
6 years. There is a second peak of incidence in adolescence
[166, 167].
Histologically, biliary tract rhabdomyosarcoma is typi-
cally botryoid or embryonal and usually involves the central
biliary tree and porta hepatis [167]. Tumor spread is by direct
extension to contiguous structures or by lymphatic and
hematogenous dissemination to the lungs, bones, bone mar-
row, liver, and lymph nodes. Obstructive jaundice and
abdominal pain are the presenting symptoms and are often
associated with abdominal distention, vomiting, and fever
[167].
If a soft tissue mass is detected within a dilated central bile
duct in a child, biliary rhabdomyosarcoma should be strongly
474
Fig. 12.55 Rhabdomyosarcoma of the common bile duct in a 3-year-­
old female with painless jaundice. (a) Longitudinal grayscale ultra-
sound image shows a hypoechoic mass (cursors) within a dilated CBD.
considered. On ultrasound, tumors typically appear as hypo- or
isoechoic intraductal masses in the porta hepatis, filling the extra-
hepatic ducts with a grape-like or branching pattern (Fig. 12.55)
[168]. Cystic change due to necrosis may be seen in larger
masses [165]. On MR imaging, tumors are typically hypointense
on T1-weighted images and hyperintense on T2-weighted
images, and usually demonstrate hyperenhancement.
Treatment and prognosis depend on tumor extent within
the liver, as well as regional and distant metastases. Localized
disease has a favorable prognosis. Metastases occur in up to
30% of cases and extrahepatic disease has a poor prognosis
[168]. Surgery is performed primarily for diagnostic and
staging purposes. Neoadjuvant chemotherapy usually relieves
the biliary obstruction and is generally followed by delayed
resection and radiation therapy [164, 167, 168].
Cholangiocarcinoma
Cholangiocarcinoma is primarily a tumor of adulthood,
although it can rarely occur in adolescence [169]. As many
as 90% of children with cholangiocarcinoma have associated
comorbidities such as primary sclerosing cholangitis, con-
genital biliary tract malformation, immune deficiency,
inflammatory bowel disease, post-radiation, choledochal
cyst, progressive familial intrahepatic cholestasis, and Caroli
disease [168, 169]. Abdominal pain, jaundice, and pruritus
are the most common presenting symptoms.
On ultrasound, tumors appear as hypo- to isoechoic soft
tissue masses. CT shows an ill-defined lesion in the porta
C. L. Carlson et al.
(b) Coronal contrast-enhanced CT image shows a heterogeneous mass
(arrow) within the CBD. There is associated dilation (arrowheads) of
proximal intrahepatic bile ducts
hepatis which can be mistaken for periportal fibrosis on
unenhanced images. MR imaging reveals a T1 hypointense,
T2 hyperintense lesion with restricted diffusion and minimal
enhancement on delayed images [168].
Early detection and prompt surgical intervention are
essential for survival. Unfortunately, overall three-year sur-
vival rates are poor, ranging from 35% to 50% [169].
Neuroendocrine Tumor
Neuroendocrine tumor of the extrahepatic bile ducts is an
extremely rare neoplasm of endocrine cells of varying malig-
nant potential. It is composed of multipotential cells with the
capability of secreting many hormonal substances and vaso-
active peptides. However, extrahepatic bile duct neuroendo-
crine tumors rarely manifest symptoms associated with these
substances or peptides. They occur most often in young
females and usually present with painless jaundice [170].
Due to their rarity, a definitive diagnosis is usually made fol-
lowing histopathological examination (Fig. 12.56, Cineclip
12.6). These tumors grow slowly, and surgical resection is
the only curative treatment.
Metastases
Metastatic disease to the biliary tree from other primary malig-
nancies is extremely rare. In children, the most common
metastases to the bile ducts are from primary tumors of the
liver [171]. Neuroblastoma, Wilms’ tumor, ­rhabdomyosarcoma,
germ cell tumors, neuroendocrine pancreatic tumors, pancre-
12 Gallbladder and Biliary Tract 475

a b

Fig. 12.56 Neuroendocrine tumor of the CBD in a 16-year-old female dilation (arrow). (b) Corresponding coronal T2-weighted, fat-sup-
who presented with abdominal and back pain, nausea, and abnormal pressed MR image shows an intermediate signal intensity mass (arrow-
liver function tests. (a) Longitudinal grayscale ultrasound image dem- head) in the distal CBD with proximal dilation (arrow)
onstrates a solid, round mass (arrowhead) in the CBD with proximal

atoblastoma, gastrointestinal stromal tumor, desmoplastic

small round cell tumor, and lymphoma can all metastasize to
the liver and secondarily involve the biliary tree (Fig. 12.57)
[172].

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 Spleen and Peritoneal Cavity
13
Patrick Duffy, Ilse Castro-Aragon, Patrick Tivnan,
Frank M. Volberg, Ella Kipervasser, Zoltan Harkanyi,
and Harriet J. Paltiel

ERCP Endoscopic retrograde

Abbreviations cholangiopancreatography
FAST Focused assessment with sonography in trauma
α Alpha
FDG Fluorodeoxyglucose
AAST American Association for the Surgery of Trauma
GLUT1 Glucose transporter 1 protein
AIDS Acquired immune deficiency syndrome
HbS Hemoglobin S
ALK Anaplastic lymphoma kinase
HbSC Hemoglobin SC disease
β Beta
HbSS Hemoglobin SS disease
CCLA Central conducting lymphatic anomaly
HHV Human herpesvirus
CEUS Contrast-enhanced ultrasound
HIV Human immunodeficiency virus
CMV Cytomegalovirus
IMT Inflammatory myofibroblastic tumor
CSF Cerebrospinal fluid
IR Inversion recovery
CT Computed tomography
IV Intravenous
DSRCT Desmoplastic small round cell tumor
IVC Inferior vena cava
EBV Epstein-Barr virus
KHE Kaposiform hemangioendothelioma
KLA Kaposiform lymphangiomatosis
LCH Langerhans cell histiocytosis
P. Duffy (*) LM Lymphatic malformation
Department of Radiology, Boston Children’s Hospital and Harvard MAI Mycobacterium avium-intracellulare
Medical School, Boston, MA, USA MHz Megahertz
e-mail: patrick.duffy@childrens.harvard.edu
MR Magnetic resonance
I. Castro-Aragon NFI Neurofibromatosis type I
Division of Pediatric Radiology, Department of Radiology, Boston
Medical Center, Boston University School of Medicine, NHL Non-Hodgkin lymphoma
Boston, MA, USA NK Natural killer
P. Tivnan NSAIDs Nonsteroidal anti-inflammatory drugs
Department of Radiology, Boston Medical Center, PCR Polymerase chain reaction
Boston University School of Medicine, PET Positron emission tomography
Boston, MA, USA PNET Primitive neuroectodermal tumor
F. M. Volberg SAAG Serum-ascites-albumin gradient
Department of Radiology, UVA University Hospital, University of SD Standard deviation
Virginia School of Medicine, Charlottesville, VA, USA
TB Tuberculosis
E. Kipervasser TIPS Transjugular intrahepatic portosystemic shunt
Department of Radiology, Mount Auburn Hospital, Cambridge,
MA, and Harvard Medical School, Boston, MA, USA TPN Total parenteral nutrition
VM Venous malformation
Z. Harkanyi
Department of Radiology, Heim Pal National Pediatric Institute, VP Ventriculoperitoneal
Budapest, Hungary
H. J. Paltiel
Division of Ultrasound, Department of Radiology, Boston Children’s
Hospital and Harvard Medical School, Boston, MA, USA

© Springer Nature Switzerland AG 2021 481

H. J. Paltiel, E. Y. Lee (eds.), Pediatric Ultrasound, https://doi.org/10.1007/978-3-030-56802-3_13
482
Spleen
Introduction
The spleen is often affected by systemic disorders that
exaggerate normal splenic function. It is also a common
site of infectious, inflammatory, vascular, and neoplastic
disorders. When children undergo investigation for splenic
abnormalities, ultrasound evaluation is frequently the ini-
tial imaging study to be performed. Ultrasound is useful in
confirming generalized splenomegaly as well as in charac-
terizing focal splenic lesions. In this chapter, an overview
of normal splenic development and anatomy is presented,
as well as a discussion of imaging technique, anatomic
variants, congenital anomalies, and the characteristic ultra-
sound imaging features of a broad spectrum of disorders
that affect the spleen.
Technique
Patient Positioning
Ultrasound imaging of the spleen is performed with the
patient supine and the transducer placed in a lower left inter-
costal space in a coronal plane, along the posterior axillary
line. Moderate inspiration will move the left hemidiaphragm
and the spleen inferiorly, thereby improving visualization.
Since it is located high in the left upper quadrant of the abdo-
men, shadowing from ribs, overlying lung and bowel gas can
result in a suboptimal examination. Turning the patient into a
right lateral or oblique position permits better access to the
spleen via a posterior approach. When the liver or spleen is
enlarged, imaging of the spleen via an anterior approach is
also feasible. The spleen can be used as a window to visual-
ize the tail of the pancreas.
Ultrasound Transducer Selection
In neonates and small children, a high-frequency curved
array transducer on the order of 5–8 MHz is ideal, in order to
provide adequate penetration and image resolution. In older
children and adolescents, curved array transducers are used
(C 9–2 or C5–1). High-frequency linear array transducers
(L12–5 or L 12–3) are useful for depiction of greater paren-
chymal detail. On high-resolution images, granular or reticu-
lonodular patterns of echogenicity can be seen in children.
The small nodules in the splenic parenchyma likely represent
white pulp lymphoid follicles [1].
P. Duffy et al.
Imaging Approaches
Every splenic examination should include a coronal view of
the spleen and the left upper renal pole (Fig. 13.1). Examination
in the coronal plane will generally provide an accurate assess-
ment of splenic size and the presence of any lesion within or
adjacent to the spleen. Splenic length is measured in a coronal
or coronal oblique view that includes the hilum. An estimate
of splenic volume can be calculated from length measure-
ments obtained on longitudinal and transverse images by
using the formula for a prolate ellipsoid: length × width ×
height × 0.52.
Harmonic imaging and compound imaging are techniques
used to enhance image quality. Evaluation of the splenic vascu-
lature is performed with color and spectral Doppler. Contrast-
enhanced ultrasound (CEUS) is useful in the assessment of
blunt abdominal trauma and can also be helpful in the depiction
of focal lesions [2, 3].
Normal Development and Anatomy
Normal Development
The spleen forms during the fifth week of life from outpouchings
of mesenchymal cells within the dorsal mesentery (Fig. 13.2).
During gastric rotation at approximately the seventh week of
gestation, the spleen moves from the midline to the left side of
the abdominal cavity. The primordial spleen is in close proximity
to the developing gonads and epididymis in males [4]. The
spleen grows and remains connected to the dorsal mesogastrium
by the lienorenal ligament, which connects to the left kidney and
posterior abdominal wall. The spleen is also fixed by two liga-
ments that are created by splitting of the dorsal mesogastrium:
the gastrosplenic ligament joins the spleen to the greater curva-
ture of the stomach and the phrenicosplenic ligament which joins
the spleen with the parietal peritoneum of the diaphragm.
Normal Anatomy
The spleen is comma shaped and located in the left sub-
phrenic space with an oblique long axis parallel to the tenth
rib. The superior pole is posterior and the inferior pole is
anterior [6]. The spleen has a convex and superior diaphrag-
matic surface, a superior and medial gastric impression, a
lower and medial renal impression, and a colonic impression
at the inferior margin. The pancreatic tail indents the spleen
at the hilum. The left lateral pulmonary diaphragmatic recess
can extend to the level of the inferior border of the spleen [5].
13 Spleen and Peritoneal Cavity 483

a b

S
S
K

A
V

Fig. 13.1 Normal spleen in a 3-year-old male. Coronal (a) and transverse splenic artery (A) and vein (V). Spectral Doppler analysis shows a normal
(b) grayscale ultrasound images of the spleen (S) reveal homogeneous low-­resistance arterial waveform above the baseline (d) and a slightly pul-
parenchyma that is more echogenic than the adjacent left kidney (K). P, satile venous waveform below the baseline (e)
Pancreas. (c) Transverse color Doppler ultrasound image depicts the
484 P. Duffy et al.

Left kidney

Dorsal
mesentery

Aorta

Spleen

Stomach

Liver

Gastrohepatic
ligament Left kidney
Ventral
mesentery
Falciform Splenic artery
ligament
Area of fusion
Dorsal
mesentery
Lienorenal
ligament
Gastrosplenic
ligament

Fig. 13.2 Diagram of normal development of the spleen. (a) The rela- During gastric rotation at approximately the seventh week of life, the
tionship of the developing spleen to the dorsal mesentery and adjacent spleen moves from the midline to the left side of the abdominal cavity
structures is shown at approximately the fifth to sixth weeks of life. (b)

The gastrosplenic ligament, spleen, and lienorenal ligament
form the lateral margin of the lesser peritoneal sac. The spleno-
pancreatic ligament connects the splenic hilum with the pancre-
atic tail and encases the splenic vessels. The plica splenoomentalis
extends between the inferior margin of the spleen and greater
omentum at the left colic flexure [5, 6]. Diaphragmatic eventra-
tion or herniation can lead to an intrathoracic location of the
spleen, and a deep lateral peritoneal recess can result in posi-
tioning of the spleen posterior to the left kidney [7].
The splenic artery and segmental branches arise from the
celiac artery. The splenic vein joins the superior mesenteric
vein to form the main portal vein. The vessels course along
the posterior and inferior margin of the pancreas (Fig. 13.3).
Blood enters the spleen through the splenic artery, which
divides into trabecular arteries that branch throughout the
parenchyma into central arterioles surrounded by lymphoid
cells. The splenic arterioles either drain into the venous
sinuses or terminate in the parenchyma, discharging blood
into the red pulp (Fig. 13.4) [8].
The spleen is a highly vascular organ comprised of two
compartments, the red pulp and the white pulp. The red
pulp is a blood filter that removes damaged red blood cells
and foreign material from the blood and is a storage site
for iron, red blood cells, and platelets. The white pulp
consists of lymphatic tissue that initiates immune response
to blood-borne antigens. The white pulp surrounds the
13 Spleen and Peritoneal Cavity 485

Short gastric
arteries

Spleen

Splenic artery Splenic vein

Pancreas Left gastroepiploic

artery and vein

Inferior
mesenteric vein

Fig. 13.3 Diagram illustrating the splenic blood supply and its relationship to the pancreas

Capsule

Trabecula

Vascular
sinusoid

White pulp
Artery

Vein Red pulp

Fig. 13.4 Diagram illustrating the internal anatomy of the spleen

486 P. Duffy et al.

Trabecular Lymphoid Penicillar

artery follicle arteriole

Red pulp
Periarteriolar lymphoid
sheath (T cells)
Trabecular
vein
Central Splenic
arteriole sinus
Closed
circulation
Trabecula
Peripheral white
pulp (B cells) Open
circulation

Marginal zone
sinuses

Fig. 13.5 Diagram highlighting the anatomy of the two splenic compartments: the red pulp and the white pulp

central arterioles and contains three subcompartments:
the periarteriolar lymphoid sheath, the follicles, and the
marginal zone [9]. A capsule composed of dense fibrous
tissue, elastic fibers, and smooth muscle surrounds the
spleen (Fig. 13.5).
The echotexture of the spleen is homogeneous, slightly
more echogenic than the renal cortex and liver. The left lobe
of the liver can extend to the left lateral abdominal wall and
wrap around the spleen or displace the spleen inferiorly,
occasionally simulating a perisplenic mass or being con-
fused for the spleen (Fig. 13.6). Careful evaluation of the
portal triads and liver vasculature and confirmation of the
splenic hilum are important to avoid this pitfall.
Ultrasound values for normal splenic length in children have
been published and describe a roughly logarithmic correlation
between length and age. Upper limits of normal splenic length
of 13 cm for boys and 12 cm for girls have been determined
[10]. The average splenic length in the newborn is 4.5 cm, and
in older adolescents, it is 10.5 cm [11]. Ultrasound findings that
suggest splenic enlargement are increased length and volume,
medial and anterior displacement of the stomach, inferomedial
displacement of the bowel, lower pole extending beyond the
inferior pole of the left kidney, extension medial to the aorta,
and loss of the inferomedial concavity [12]. Splenomegaly is a
frequent manifestation of both primary splenic disorders and
systemic disease (Table 13.1).
13 Spleen and Peritoneal Cavity 487

Table 13.1 Causes of splenomegaly

a
Infection Viral
Bacterial
Fungal
Parasitic
Hematologic malignancy Leukemia
Hodgkin and non-Hodgkin
lymphoma
Other hematologic disorders Langerhans cell histiocytosis
Hemolytic anemia Congenital
Acquired
Liver disease (cirrhosis) Portal hypertension
Portal vein thrombosis
Disorders of immune Rheumatic disorders
regulation
Inflammatory disorders Sarcoidosis
Storage diseases Gaucher
Niemann-Pick
Mucopolysaccharidoses
Other masses Cyst
Lymphatic and venous
malformations
Inflammatory myofibroblastic tumor
Hamartoma
Cardiovascular disease Congestive heart failure

Anatomic Variants

Lobulations and Clefts

In early fetal life, the spleen is lobulated. At birth, the lobules
b are usually no longer present [6]. However, lobulations can
persist into adult life as normal variants and should not be
mistaken for a mass (Fig. 13.7). Splenic notches or clefts are
common and represent remnants of the grooves that separate
the fetal splenic lobules. They usually have sharp borders
and can be as deep as 2–3 cm (Fig. 13.8). They are most
commonly located on the medial surface or the superior bor-
der of the diaphragmatic surface of the spleen [13]. They
have no clinical significance and should not be misinter-
preted as a splenic laceration. The presence of normal splenic
enhancement and absence of free abdominal or perisplenic
fluid are principal imaging features that aid in differentiating
this normal variant from a laceration.

Accessory Spleen
A supernumerary or accessory spleen (splenule) represents
Fig. 13.6 Left hepatic lobe mistaken for spleen in a 14-year-old male ectopic splenic tissue and is common, with identification in
with sickle-cell anemia. (a) Coronal grayscale ultrasound image of the 10–30% of all autopsies [6]. It develops as a result of failure
left upper abdominal quadrant reveals a parenchymal structure (arrow- of cell fusion during embryological development. It can be
heads) initially thought to be the spleen. (b) Coronal T2-weighted, fat-­
suppressed magnetic resonance (MR) image reveals absence of the
single (85% of cases) or multiple, and it is usually small in
spleen. The left hepatic lobe (asterisk) occupies the left upper quadrant
488 P. Duffy et al.

Fig. 13.7 Normal splenic lobule simulating a mass in a 15-month-old the upper pole of the left kidney (K). (c) Axial T2-weighted, fat-­
male. Transverse (a) and coronal (b) grayscale ultrasound images of the suppressed and (d) coronal inversion recovery (IR) MR images confirm
left upper abdominal quadrant show a lobule of splenic tissue (asterisks) that the “mass” (arrows) represents normal splenic tissue. K, Left kidney
extending medially from the inferior portion of the spleen and indenting

a b

Fig. 13.8 Splenic cleft in a 1-month-old female. (a) Transverse gray- anterior aspect. (b) Coronal grayscale ultrasound image shows the cleft
scale ultrasound image shows an obliquely oriented, linear, hypoechoic (arrowheads) as a thin, echogenic line extending superiorly from the
zone (arrowheads) extending through the splenic parenchyma from its medial aspect of the spleen
13 Spleen and Peritoneal Cavity
size, generally 1–2.5 cm in diameter and rarely over 4 cm. In
75% of cases, it is located in the hilum of the spleen [14]. It
can also occur in the gastrosplenic ligament, splenorenal
ligament, pancreatic tail, left ovary, left testicle, retroperito-
neum, or peritoneal cavity (Fig. 13.9).
An accessory spleen is usually asymptomatic. It may
rarely undergo torsion, hemorrhage, spontaneous rupture, or
Fig. 13.9 Diagram illustrating possible locations of an accessory spleen
Fig. 13.10 Accessory spleen in a 13-year-old male. (a) Coronal gray-
scale ultrasound image demonstrates a round mass (arrow) inferior to
the spleen (S) with the same echogenicity and echotexture, consistent
489
cyst formation and present with an acute abdomen and acute
or chronic abdominal pain with or without abdominal mass.
The ultrasound characteristics of an accessory spleen are
similar to those of a normal spleen. It is smooth in contour,
round, ovoid, or minimally lobulated (Fig. 13.10). It can be
confused with a lymph node or neoplasm of the adrenal gland,
pancreas, stomach, intestine, retroperitoneum, or pelvis. An
Pancreas tail
Hilum of the
main spleen
Gastrosplenic ligament
Splenorenal ligament
Greater omentum
Pelvis
with an accessory spleen. K, Left kidney. (b) Coronal color Doppler
ultrasound image shows separate but similar blood supplies to the
spleen and to the accessory spleen
490
Fig. 13.11 Torsion of an accessory spleen in a 15-year-old male. (a)
Transverse grayscale ultrasound image reveals a round, hypoechoic
structure (arrow) adjacent to the anteromedial aspect of the spleen (S).
(b) Longitudinal color Doppler ultrasound image reveals an absence of
accessory spleen can demonstrate compensatory hypertrophy
after splenectomy. In patients with hematologic disorders, it
can be a site of disease recurrence after splenectomy [15].
Ultrasound findings of an accessory spleen that has under-
gone torsion include a round, solid structure surrounded by
echogenic fat and demonstrating an absence of blood flow
(Fig. 13.11). The absence of perfusion can be confirmed with
CEUS [16].
An accessory spleen does not require any treatment when
asymptomatic. Surgical removal may be necessary if it is
associated with an acute abdomen.
Congenital Anomalies
Wandering Spleen
A wandering or migrating spleen occurs when the spleen is
able to move into the lower abdomen or pelvis due to hyper-
mobility of the ligaments that normally fix the spleen to the
diaphragm, kidney, stomach, or left colic flexure. One third
of cases occur in children under the age of 10 years. The
diagnosis is ten times more frequent in females of reproduc-
P. Duffy et al.
flow (arrow) within the lesion. Normal flow (arrowheads) is identified
within the adjacent splenic parenchyma. There is fluid (asterisk) within
a splenic cleft. (c) Axial contrast-enhanced CT image confirms the lack
of perfusion (arrow) within the accessory spleen
tive age, likely related to hormonal influence on the laxity of
the splenic ligaments [17]. Common associations include
congenital diaphragmatic hernia, prune-belly syndrome,
renal agenesis, and gastric volvulus.
Wandering spleen can present as an asymptomatic, pal-
pable abdominal mass or with acute, chronic, or intermittent
pain due to torsion with associated ischemia and infarction.
It is estimated that half of all cases of wandering spleen
remain undiagnosed.
Ultrasound can confirm absence of the spleen from the left
upper quadrant and will demonstrate its location more inferiorly
in the abdomen or pelvis. In the setting of torsion, the splenic
hilum is often located anteriorly and a twisted vascular pedicle
can be seen. The spleen may appear enlarged with heterogeneous
echotexture and surrounding ascites. On Doppler ultrasound,
there will be decreased or absent flow in the splenic parenchyma,
and spectral analysis of the splenic artery may show low diastolic
flow with an elevated resistive index (Fig. 13.12).
Approximately two thirds of patients with wandering
spleen complicated by torsion will require splenectomy. If
the diagnosis is made early, however, splenopexy may permit
preserved function.
13 Spleen and Peritoneal Cavity
a b
Fig. 13.12 Wandering spleen in an 8-year-old female with acute
abdominal pain. At surgery, the spleen was twisted on its vascular ped-
icle and infarcted. (a) Frontal supine abdominal radiograph shows a
soft-tissue mass (arrows) inferior to the liver in the right lower quadrant
of the abdomen with extension across the midline. (b) Longitudinal
 olysplenia, Hyposplenia, and Asplenia
P Hyposplenia can rarely be congenital but is more com-
Polysplenia develops when there is absence of fusion of the
primordial cells of the spleen. This results in multiple (2–16)
small, similar-sized spleens ranging from 1 to 6 cm in diam-
eter located along the greater gastric curvature or in the right
upper abdominal quadrant in the setting of organ heterotaxy
(situs ambiguous).
With polysplenia, ultrasound readily demonstrates the
multiple spleens located on the same side of the body as the
stomach (Fig. 13.13). When there is associated heterotaxy,
40–70% of patients will also have associated abnormalities
that can include a left-to-right intracardiac shunt, interrup-
tion of the infrahepatic portion of the inferior vena cava
(IVC) with azygos continuation, a preduodenal portal vein,
intestinal malrotation, pancreatic anomalies, and absent gall-
bladder [14].
491
grayscale ultrasound image of the right lower quadrant reveals an
enlarged spleen (S) with anechoic fluid (asterisk) adjacent to its lower
pole. (c) Longitudinal color Doppler ultrasound image shows a com-
plete absence of splenic parenchymal blood flow. S, Spleen
monly acquired, as in sickle cell disease, celiac disease,
malaria, or cirrhosis. Hyposplenia can be identified by the
presence of Howell-Jolly bodies (nuclear remnants in circu-
lating mature erythrocytes) or pitted cells (vesicles underly-
ing the membrane that occur in aged red cells) in the
peripheral blood smear.
Ultrasound features include a small, echogenic, and hetero-
geneous spleen. The diagnosis of hyposplenia can be con-
firmed with radionuclide scintigraphy using 99 m-technetium
(Tc)-labeled sulfur colloid which assesses the phagocytic
function of the spleen, or with 99 m-Tc-labeled heated autolo-
gous red blood cells which are trapped within the spleen [9].
Asplenia (Ivemark syndrome) is a rare disorder affect-
ing multiple organ systems of the body. It is characterized
by splenic hypoplasia or aplasia, cardiac malformations,
492
Fig. 13.13 Heterotaxy syndrome with polysplenia in a 7-month-old
female. Transverse (a) and longitudinal (b) grayscale ultrasound images
of the right upper quadrant of the abdomen reveal multiple spleens
(asterisks). K, Right kidney. (c) Transverse grayscale ultrasound image
and heterotaxy. Affected patients are more susceptible to
sepsis, most commonly encapsulated bacteria such as pneu-
mococcus. Asplenia is associated with complex cyanotic
heart disease, midgut malrotation, microgastria, and gall-
bladder duplication. The spleen is most commonly absent,
but rarely a rudimentary spleen can be detected by ultra-
sound [15].
P. Duffy et al.
of the left upper quadrant of the abdomen demonstrates a left-sided liver
(L). (d) Longitudinal color Doppler ultrasound image of the right flank
shows an enlarged azygos vein (V). A, Aorta
Ultrasound can document absence of the spleen as well as
many of the associated anatomical abnormalities of the
abdominal organs and vasculature.
Since heterotaxy syndromes are associated with a high
prevalence of cardiac and extracardiac anomalies, treatment
and outcomes will depend on the severity of the associated
anomalies.
13 Spleen and Peritoneal Cavity
 onparasitic Splenic Cysts
N On ultrasound imaging, congenital cysts are usually uni-
The most common focal lesion in the pediatric spleen is a
cyst [12]. Nonparasitic splenic cysts can be classified into
primary and secondary cysts. Primary cysts can be congeni-
tal or neoplastic (lymphatic malformation, a congenital vas-
cular anomaly, and neoplastic cysts are discussed later in this
chapter). Congenital cysts comprise 10% of all splenic cysts
and are more common in girls [12, 15]. They are sometimes
referred to as epidermoid cysts since they are lined with epi-
thelium [18]. Most congenital cysts are asymptomatic.
Occasionally, they may be associated with pain or present as
a palpable mass in the left upper quadrant. Secondary splenic
cysts account for 80% of all splenic cysts and develop as a
result of prior trauma, infection, or infarction [15].
a one of the embryonic excretory organs. Splenogonadal
b as an acute scrotum.
Fig. 13.14 Congenital splenic cyst in a 13-year-old female. (a) Longi­
tudinal grayscale ultrasound image shows a large unilocular anechoic
cyst replacing most of the spleen with a small amount of parenchyma
(asterisks) identified in the upper and lower splenic poles. (b) Coronal
contrast-enhanced CT image confirms the presence of a large unilocular
splenic cyst (arrow) with compressed upper and lower pole parenchyma
(asterisks)
493
locular with a thin wall and contain anechoic fluid (Fig. 13.14).
Secondary cysts are small, multilocular lesions. The cyst wall
is often calcified, and the cyst fluid may be echogenic as a
result of proteinaceous debris or hemorrhage.
Treatment is not generally required when the cyst is less
than 5 cm in diameter and asymptomatic. Larger, symptomatic
cysts can be treated with percutaneous drainage and partial or
total splenectomy [19]. Some tissue-preserving surgical
approaches are associated with high rates of recurrence.
Percutaneous drainage followed by sclerotherapy has a high
success rate and allows for preservation of splenic tissue.
Splenogonadal Fusion
Splenogonadal fusion is a rare congenital anomaly where the
spleen fuses with a gonad or a remnant of the mesonephros,
fusion can be continuous or discontinuous. In the continuous
type (58% of cases), there is a direct connection between the
spleen and the gonad. In the discontinuous type, ectopic
splenic tissue is attached to the gonad, but there is no connec-
tion to the orthotopic spleen [6]. This malformation is more
common in males, with a male-to-female ratio of 15:1. It can
affect the descent of the testicle, and one third of cases are
associated with cryptorchidism.
Splenogonadal fusion can be discovered incidentally dur-
ing repair of an inguinal hernia or as an asymptomatic mass
in the scrotum or inguinal canal. Occasionally, it can present
Ultrasound can demonstrate accessory splenules along the
path of testicular descent as well as splenic tissue adjacent to
the testicle or, rarely, adjacent to the ovary (see Chap. 15, Fig.
15.19). The presence of splenic tissue can be confirmed prior
to surgical removal with a 99 m-Tc-sulfur colloid scan [20].
The aberrant splenic tissue can usually be excised without
injury to the testicle. However, if the splenogonadal fusion is
misdiagnosed as malignancy, patients are at risk for an
unnecessary radical orchiectomy. Cases of testicular cancer
arising in splenogonadal fusion are very rare.
Splenopancreatic Fusion
Splenopancreatic fusion refers to fusion of the spleen with
the tail of the pancreas. This anomaly is thought to occur as
a result of the close proximity of the splenic primordium in
the dorsal mesogastrium to the dorsal pancreatic bud in the
dorsal mesoduodenum. Splenopancreatic fusion is com-
monly associated with other severe congenital malforma-
tions, including trisomy 13, trisomy 21, and Schinzel-Giedion
syndrome.
Ultrasound can demonstrate fusion of the pancreatic tail
with the lower pole of the spleen, splenic hilum, and/or
accessory spleen [21].
494
Identification of this condition is important if the patient is
scheduled to undergo distal pancreatectomy or splenectomy,
in order to avoid bleeding or pancreatic duct leakage [21].
Infection
Pyogenic Abscess
Splenic abscesses are rare since the spleen has phagocytic
immune function. In children, bacterial abscesses are typi-
cally solitary and tend to occur in immunocompromised
patients. Other risk factors include sickle cell disease, endo-
carditis and other systemic bacterial infections, splenic
injury, and adjacent sites of infection. Symptoms include left
upper quadrant or diffuse abdominal pain, left-sided chest
pain, fever, splenomegaly, diarrhea, dyspnea, and pleural
effusion [22]. Gram-negative bacteria are most frequently
cultured, including Salmonella (nontyphoidal and typhoi-
dal), Acinetobacter, and Escherichia coli [23–25].
On ultrasound, the typical appearance of a splenic abscess
is of a single, ill-defined anechoic or hypoechoic lesion that
can contain debris, fluid levels, and internal septations of
varying thickness (Fig. 13.15). The rim of the abscess can be
thick and irregular. Echogenic foci with “dirty shadowing”
occur with intralesional gas, an infrequent but pathogno-
monic sign [7, 12]. CEUS imaging of abdominal abscesses
demonstrates either a total absence of enhancement, rim,
and/or septal enhancement.
Fig. 13.15 Pyogenic splenic abscess in a 17-year-old male with a his-
tory of splenic laceration and splenic artery embolization. Cultures of
aspirated fluid subsequently grew Cutibacterium acnes. Coronal gray-
scale ­ultrasound image of the spleen shows a large centrally located
fluid collection (asterisk) with irregular margins containing internal
debris (arrowhead)
P. Duffy et al.
Treatment approaches include broad-spectrum antibiot-
ics, percutaneous aspiration in children with large abscesses
(>3 cm) and poor response to antibiotic therapy, and splenec-
tomy [24].
Cat-scratch disease is caused by Bartonella henselae, an
intracellular gram-negative bacillus. Infection occurs as a result
of a cat scratch or bite, although some patients have no history
of feline contact [25]. In 75–90% of cases, regional lymphade-
nitis occurs following skin injury. In the remaining10–25% of
patients, symptoms are variable and may include fever of
unknown origin, abdominal pain, arthralgias, visceral lymph
node enlargement, hepatic, or splenic lesions [26]. Diagnosis is
made on the basis of serological testing, polymerase chain reac-
tion (PCR) blood testing, histology, and imaging.
Ultrasound findings include splenomegaly with or with-
out small focal hypoechoic lesions, hepatomegaly, and intra-­
abdominal lymphadenopathy that often involves the porta
hepatis.
Hepatosplenic disease is usually treated with antibiotics,
including trimethoprim-sulfamethoxazole, rifampin, ciproflox-
acin, and/or gentamicin. In immunocompromised patients, a
prolonged course of antibiotic treatment may not be suffi-
cient, with frequent relapses and bacteremia that in some
cases requires lifelong antibiotic administration [27].
Hepatosplenic abscesses are seen in 1.2% of patients with
Brucellosis, a zoonotic infection caused by gram-negative
bacilli consumed in unpasteurized milk or undercooked meat
from infected animals, or close contact with their secretions.
There are only four cases of hepatosplenic involvement
reported in children less than 14 years of age, and the spleen
was affected in all four. The most common pattern of disease
consisted of multiple splenic abscesses with hepatic
abscesses in two of the four affected children. Unlike adults,
no central lesion calcification was identified [28].
Ultrasound can confirm splenomegaly and multiple
hypoechoic lesions in the spleen and liver. Acute infec-
tions are treated with antibiotics (usually a combination
of doxycycline with an a­minoglycoside), and response
can be monitored with serial ultrasound examinations.
Fungal Abscess
Fungal infections typically present as multiple abscesses. They
occur most commonly in immunocompromised individuals
following chemotherapy, bone marrow or organ transplanta-
tion, and in patients with diseases associated with neutropenia
such as chronic granulomatous disease of childhood. The most
common causative organisms include Candida albicans,
Aspergillus fumigatus, and Cryptococcus neoformans [15].
Hepatosplenic candidiasis is characterized by a chronic debili-
13 Spleen and Peritoneal Cavity
tating course that can be refractory to conventional therapy or
require an extensive course of antifungal therapy.
Imaging is important for diagnosis and monitoring of
treatment response. However, lesions are not usually detect-
able with imaging techniques prior to neutrophil count
recovery.
On ultrasound, fungal abscesses are most commonly seen
as multifocal lesions that diffusely involve the liver and/or
spleen. The lesions vary in size from a few millimeters to
less than 4 cm in diameter. The lesions may be hypoechoic,
hyperechoic, or layered in appearance. They can be difficult
to distinguish from lymphoma, leukemic infiltrates, or
metastases. A characteristic appearance has been described
a b
Fig. 13.16 Multiple fungal splenic abscesses in a 7-year-old male with
acute leukemia and disseminated Trichosporon infection. Coronal (a)
and transverse (b) grayscale ultrasound images of the spleen reveal
multiple punctate echogenic foci with a surrounding hypoechoic rim
495
as a “target” or “bull’s eye” pattern consisting of a central
hyperechoic lesion surrounded by a rim of peripheral
hypoechoic tissue (Fig. 13.16). The hyperechoic center can
sometimes undergo necrosis and is then hypoechoic, result-
ing in a “wheels within wheels” appearance consisting of a
hypoechoic center surrounded by hyperechoic inflammatory
cells and a peripheral hypoechoic zone due to fibrosis [12].
Although CT and magnetic resonance (MR) imaging are gen-
erally superior to ultrasound for the identification of lesions asso-
ciated with hepatosplenic candidiasis, they may involve greater
risk to these often fragile patients with respect to the potential
need for transportation, sedation, and/or ionizing radiation.
CEUS may improve the accuracy of ultrasound in the detection
(arrowheads), the so-­called “target” or “bull’s eye” pattern. (c) Axial
contrast-enhanced, T1-weighted, fat-suppressed MR image shows numer-
ous tiny, faintly-enhancing parenchymal lesions (arrowheads)
496
a b
Fig. 13.17 Fungal abscesses of the spleen in a 20-year-old female with
leukemia and disseminated Cunninghamella infection. Coronal (a) and
transverse (b) grayscale ultrasound images of the spleen reveal a solitary
hypoechoic splenic lesion (arrowheads). (c) Coronal oblique contrast-­
of splenic fungal lesions (Fig. 13.17) [29]. Histoplasmosis of the
spleen is a common cause of diffuse punctate splenic calcifica-
tions (Fig. 13.18).
Therapy of splenic fungal abscesses depends on the
underlying organism, clinical condition of the patient, and
extent of infection. They usually respond to several weeks of
P. Duffy et al.
enhanced ultrasound (CEUS) image shows two rounded, nonenhancing
foci (arrowheads) within the spleen in keeping with abscesses. (d) Axial
contrast-enhanced CT image confirms the presence of the two splenic
lesions (arrows)
antimicrobial therapy alone. Noncandidal invasive fungal
infections have a high risk of mortality and require systemic
antifungal agents. Percutaneous or laparoscopic abscess drain-
age or splenectomy may be required for large abscesses and
those that do not resolve with antimicrobial therapy, espe-
cially multiple candidal abscesses [30].
13 Spleen and Peritoneal Cavity
Fig. 13.18 Histoplasmosis of the spleen in a 20-year-old female. Lon­
gitudinal grayscale ultrasound image reveals multiple small echogenic
foci (arrowheads) in the spleen with associated distal shadowing in keep-
ing with calcified granulomas
Tuberculous Infection
The diagnosis of tuberculosis (TB) in children is difficult and
many cases remain undiagnosed, despite recent advancements
in molecular diagnostic techniques and culture methods.
Immunosuppressed, malnourished, and young children are at
particular risk for rapid progression and dissemination of dis-
ease. Splenic enlargement has been described in 16% of chil-
dren with abdominal tuberculosis, with active pulmonary
tuberculosis in 34% of those with abdominal tuberculosis [31].
In a recent study, focal splenic lesions were seen by ultra-
sound in 5% of children with suspected pulmonary TB and
were strongly associated with confirmed infection. Abdominal
findings were more common in children co-­infected with
human immunodeficiency virus (HIV) and TB, with splenic
microabscesses reported in 23% of these patients [31]. Other
common imaging findings in children with abdominal TB
include ascites, lymphadenopathy, omental thickening, and
thickening of intestinal loops [32].
Patterns of splenic tuberculous infection can be micro- or
macronodular. On ultrasound, the most commonly identi-
497
Fig. 13.19 Splenic abscesses in a 3-year-old female with dissemi-
nated tuberculosis. (a, b) Coronal grayscale ultrasound images of the
spleen demonstrate multiple small hypoechoic parenchymal lesions
(arrowheads)
fied form is the micronodular or miliary pattern, with sple-
nomegaly, multiple tiny hypoechoic lesions, or less often,
hyperechoic lesions. Splenic foci are best seen using a high-
frequency linear ultrasound transducer (Fig. 13.19) [33].
The macronodular form presents as solitary or multiple
hypoechoic lesions with a diameter of 1–3 cm. Hyperechoic
nodules are less common.
Most splenic abnormalities will resolve within 3 months
of initiation of appropriate therapy, and ultrasound can be
used to monitor treatment response. Persistence of lesions
beyond this time suggests drug resistance, severe immune
reconstitution inflammatory syndrome, nonadherence to
treatment, or an alternative diagnosis [33].
Standard multidrug regimens are used to treat drug-­
susceptible tuberculosis in children, and outcomes are generally
favorable. Prompt initiation of treatment is critical since TB can
spread rapidly in young patients. Longer duration of therapy
may be warranted for immunocompromised hosts. Treatment of
all forms of drug-resistant TB is tailored to the specific form of
resistance with appropriate and effective drug regimens.
498 P. Duffy et al.

Epstein-Barr Viral Infection to contact sports 3 weeks after the onset of the disease if they
Epstein-Barr viral infection, or infectious mononucleosis, is a are afebrile and have no other associated abnormalities.
common cause of splenomegaly in immunocompetent adoles-
cents. The importance of the disease is related to the severity Parasitic Infection
and duration of the acute illness and its long-term association Although parasitic infection of the spleen is unusual in
with the development of certain cancers such as Burkitt lym- developed countries, parasitic infections are endemic in
phoma and autoimmune disorders such as multiple sclerosis. many parts of the world. Given the high prevalence of global
Clinical presentation includes fever, enlarged cervical lymph
nodes, and splenomegaly which can be massive and painful
although it occurs in fewer than 20% of cases. a
Splenic rupture is the most severe complication, occur-
ring in less than 1% of cases, usually within 3 weeks after
diagnosis of infectious mononucleosis and may occur spon-
taneously [34]. There is currently no reliable means of pre-
dicting the risk of splenic rupture. Anecdotally, the risk
appears to be highest in patients who are involved in contact S
sports and activities that generate high intra-abdominal pres-
sure such as weight lifting [34]. Other complications include
thrombocytopenia, hemolytic anemia, neutropenia, and
hemophagocytic syndrome.
Ultrasound can be used to confirm splenomegaly, with
peak enlargement usually occurring within 2–3.5 weeks
from the onset of symptoms (Fig. 13.20). In the majority of
cases, splenomegaly resolves within 4–6 weeks of symptom
onset [35]. In the rare instance of splenic rupture, ultrasound
b
can depict intra- and subcapsular hematomas as well as free
fluid (Fig. 13.21).
Treatment is supportive for patients with fever and malaise,
and includes hydration and rest. Athletes are allowed to return

Fig. 13.21 Splenic rupture in a 17-year-old male diagnosed with Epstein-

Fig. 13.20 Splenomegaly in a 17-year-old female with Epstein-Barr
viral infection. Coronal grayscale ultrasound image shows an enlarged
spleen (S) with homogeneous parenchyma extending below the inferior
margin of the left kidney (K)
Barr viral infection 2 weeks earlier. (a) Coronal grayscale ultrasound image
of the spleen (S) demonstrates a heterogeneous subcapsular fluid collec-
tion (asterisk). (b) Longitudinal grayscale ultrasound image of the pelvis
reveals echogenic free fluid (asterisk) in keeping with acute hemorrhage.
B, Bladder
13 Spleen and Peritoneal Cavity
travel in the recent period, patients with parasitic infection
can be found anywhere in the world and eliciting a travel his-
tory is essential in order to prevent delays in diagnosis and
treatment of these disorders.
Malaria is caused by parasites of the genus Plasmodium
and is a leading cause of mortality around the world, with
children being the main victims. Malaria is transmitted to
humans through the bite of an infected female mosquito
of the species Anopheles. The Plasmodium life cycle is
very complex, and it includes sexual and asexual phases,
vector mosquitoes, and vertebrate hosts. The sexual phase
of the life cycle occurs in the vector mosquitoes. The
asexual phase of the life cycle occurs in humans, the inter-
mediate host.
Human malaria is transmitted only by female mosquitoes
of the genus Anopheles. Following a bite by an infected
female mosquito, the parasite enters the human blood stream
and is taken up by the cells of the liver where it multiplies and
is subsequently released back into the blood stream where it
enters the red blood cells and undergoes further replication.
The diagnosis of malaria is usually accomplished through
microscopic examination of thick and thin blood smears.
Splenomegaly occurs in nearly all patients with malaria [36].
The consequences of Plasmodium infection vary in sever-
ity depending on both host factors and the infecting species.
Typical initial symptoms are low-grade fever, shaking chills,
muscle aches, and, in children, digestive symptoms. Symptoms
can be of sudden onset (paroxysms) and progress to high
fever, drenching sweats, and exhaustion. These symptoms
coincide with hemolysis of the infected red blood cells.
Complications are due to parasitic microvascular obstruc-
tion. Severe malaria is often fatal, manifesting as profound
anemia and evidence of multiorgan damage, including cere-
bral involvement.
Despite the high prevalence of disease, very little has
been published regarding its imaging manifestations, includ-
ing those on ultrasound imaging. Reduced contrast enhance-
ment and focal infarcts of the spleen have been described on
CT [37, 38].
Treatment depends on the infecting Plasmodium spe-
cies; the clinical status of the patient; expected drug sus-
ceptibility of the infecting parasite as determined by the
geographic area where the infection was acquired; and
prior use of a­ ntimalarials, including those taken for malaria
chemoprophylaxis. The most common antimalarial drugs
include artemisinin-based combination therapies and chlo-
roquine phosphate.
Echinococcus granulosus infection (hydatid disease) is
caused by the tapeworm Echinococcus that resides in the
small intestine of the definitive host, dogs, and other carni-
vores. Eggs of the adult tapeworm are excreted in the stool
and ingested by an intermediate host, usually sheep, and
other ruminants. Humans are usually infected by ingesting
499
food or drink contaminated with dog feces containing the
eggs of the worm.
The eggs hatch in the digestive system of the intermediate
host, producing larvae that penetrate the intestinal wall and
are carried by the bloodstream to the liver, lungs, brain, and
other organs, including the spleen. The embryos then grow
into fluid-containing cysts with a wall consisting of three
layers: pericyst, ectocyst, and endocyst. In humans, the cysts
persist and grow for years. The inner layer of the cyst forms
freely floating embryos that are identifiable by ultrasound as
“hydatid sand.” Daughter cysts can form within a mother
cyst, and their presence is characteristic of the disease. A
dead cyst will decrease in size and eventually calcify.
Hydatid cysts are usually asymptomatic until their
increasing size produces symptoms, or they are accidentally
discovered. Cyst disruption, either spontaneous or iatrogenic
(e.g., from liver biopsy), can be life threatening due to ana-
phylactic shock. Diagnosis is based on a combination of his-
tory, serological testing, and imaging. Demonstration of
scolices (i.e., the anterior end of the tapeworms bearing
suckers and hooks for attachment) following fine-needle
aspiration is diagnostic.
Splenic involvement has been reported in 0.9–8% of
patients with hydatid disease and develops as a result of sys-
temic dissemination or intraperitoneal spread of a ruptured
liver cyst [39]. Hydatid disease is the most frequent cause of
a splenic cyst in regions where infection is endemic [7].
Isolated splenic involvement is rare. The most frequent clini-
cal signs and symptoms are abdominal pain, splenomegaly,
and fever.
On ultrasound, splenic hydatid cysts are usually solitary,
and their imaging characteristics are similar to those of
hepatic hydatid cysts. A simple hydatid cyst can be anechoic
or contain hydatid sand, the free-floating scolices of the tape-
worm. The wall of the cyst often appears as a double echo-
genic line. When the patient moves, the hydatid sand is
dispersed in the cyst and appears as falling snowflakes, the
“snowstorm” sign [40]. Cyst wall calcification may be
present.
Treatment varies according to cyst type, location, size,
and complications. For some patients, chemotherapy with
benzimidazoles is the preferred treatment. Percutaneous
drainage or surgery may be necessary to evacuate large or
complicated cysts, and ultrasound can be useful in guiding
drainage. Every effort is made to avoid peritoneal spillage
which can cause secondary seeding and anaphylaxis.
Schistosomiasis, also known as snail fever and bilharzia,
is a disease caused by schistosomes, parasitic flatworms. The
disease is spread by contact with water contaminated with
parasites that are released from infected freshwater snails.
The organisms can penetrate unbroken human skin and
migrate via the lymph or blood to different parts of the body.
They colonize in the veins and reproduce sexually to yield-
500 P. Duffy et al.

ing multiple eggs that are either trapped in the target organs
or excreted. Clinical manifestations depend on specific organ
involvement.
Schistosomiasis is especially common among children in
developing countries. A diagnosis is made by isolating the
eggs of the parasite in the urine or stool, or through detection
of serum antibodies to schistosomes. Many individuals do
not experience any initial symptoms. If symptoms do occur,
they generally appear 4–6 weeks following infection and
include malaise and a skin rash at the site of organism entry.
Other symptoms can include fever, aches, cough, diarrhea,
chills, or gland enlargement.
Clinical manifestations of schistosomal infection will vary
over time as the worms and their eggs migrate through the
body. The eggs deposited in the target organs provoke a
granulomatous reaction and fibrotic changes, resulting in
significant pathological changes in the affected organs. The
severity of the clinical symptoms depends on worm species
and load.
Worms located in the mesenteric venules release hun-
dreds to thousands of eggs per day that are carried through
the portal system into the liver where they lodge in the presi-
nusoidal portal venous branches and lead to periportal fibro-
sis and portal hypertension. Since inflammation does not
involve the hepatic lobule, there is no hepatocyte destruction,
lobular architecture is preserved, and liver enzymes are nor-
mal. The fibrotic areas will eventually undergo dystrophic
calcification.
Ultrasound, computed tomography (CT) and MR imaging
are used for diagnosis and for evaluation of disease severity
and complications [41, 42]. Ultrasound will demonstrate the
splenic manifestations of portal hypertension, including
splenomegaly, siderotic nodules (Gamna-Gandy bodies),
and portosystemic venous shunts (Fig. 13.22). It is also used
to assess the degree of periportal fibrosis [43].
Discrimination between cirrhosis and chronic hepato-
splenic schistosomiasis is important because their treatment
and prognosis differ. Striking surface nodularity of the liver
and regenerative nodules are absent in schistosomiasis,
whereas periportal fibrosis, caudate lobe hypertrophy, sple-
nomegaly, and splenic siderotic nodules are more severe in
schistosomiasis compared to cirrhosis [41]. Fig. 13.22 Schistosomiasis of the spleen in a 7-year-old male. (a)
Treatment of schistosomiasis depends on the stage of Longitudinal grayscale ultrasound image obtained immediately prior to
diagnosis shows an enlarged spleen that measured 10.2 cm in length.
infection and clinical presentation. Praziquantel is the main- (b) Longitudinal grayscale ultrasound image obtained 2 months after
stay of chronic schistosomiasis treatment, with cure rates initiation of antibiotic treatment reveals a decrease in size of the spleen
ranging between 60% and 90%. Even patients who are not that measured 8.5 cm in length
completely cured have substantial reductions in the number
of excreted schistosome eggs which greatly reduces the like- shunt surgery, and decompressive surgery are considered in
lihood of long-term sequelae [43]. Endoscopic therapy is patients with failure of endoscopic treatment.
usually effective in controlling acute variceal bleeding in Babesiosis is a malaria-like disease caused by infection
patients with portal hypertension. Interventional therapy with the parasite Babesia that is transmitted by tick vectors
with a transjugular intrahepatic portosystemic shunt (TIPS), from small mammal reservoir hosts. In the United States,
13 Spleen and Peritoneal Cavity
Babesia microti is endemic in the Northeast and upper
Midwest. The incubation period after a tick bite is typically
1–4 weeks. The disease can also be transmitted through
blood transfusions and through the placenta from mother to
child [44]. The severity of infection depends on the immune
status of the host. Asymptomatic infection is common, espe-
cially in children. Symptomatic patients present with fever,
fatigue, and malaise.
Splenomegaly with or without hepatomegaly may be
noted on ultrasound. Splenic infarcts or splenic rupture has
been described in severe disease. The disease can be fatal,
most often if the child is immunocompromised or if there is
asplenia. Treatment includes antimicrobial drug combina-
tions and in severe cases, partial or exchange transfusion
[45].
Leishmaniasis is a parasitic disease that occurs in south-
ern Europe, as well as in the tropics and subtropics. It is
caused by infection with Leishmania parasites that are spread
by the bite of female phlebotomine sand flies. Leishmaniasis
can be cutaneous, mucocutaneous, or visceral. Two species
of Leishmania are known to lead to the visceral form of the
disease. The species found in Europe, North Africa, and
Latin America is L. infantum, also known as L. chagasi,
while the species commonly found in East Africa and the
Indian subcontinent is L. donovani. The visceral form of the
disease usually affects the spleen, liver, and bone marrow.
Visceral leishmaniasis, also known as kala-azar, is char-
acterized by irregular bouts of fever, substantial weight loss,
hepatosplenomegaly, and anemia. If left untreated, the fatal-
ity rate in developing countries can be as high as 100%
within 2 years. The incubation period is usually 2–6 months.
Symptoms are insidious with slow progression of mal-
aise, fever, weight loss, and splenomegaly (with or without
hepatomegaly). Since parasites replicate in the reticuloendo-
thelial system, high parasite loads accumulate in the spleen,
liver, and bone marrow, and severe anemia can occur. Definitive
diagnosis requires demonstration of the parasite by histopa-
thology or culture from the bone marrow or spleen.
Immunosuppression increases the risk for secondary bacte-
rial infections.
Ultrasound can confirm splenomegaly, and nodular
lesions have been described [46]. Serial studies can be used
to assess treatment response by confirming decrease in the
size of the spleen. Serologic tests are not reliable for assess-
ing treatment response since they remain positive for months
or years after therapy.
Treatment is dictated by where the disease is acquired, the
species of Leishmania, and the type of infection. For visceral
leishmaniasis in India, South America, and the Mediterranean,
liposomal amphotericin B is the recommended treatment and
is often used as a single dose. Rates of cure with a single dose
of amphotericin have been reported as greater than 90% [47].
501
In India, almost all infections are now resistant to pen-
tavalent antimonials. In Africa, a combination of pentavalent
antimonials and paromomycin is recommended although
they can have significant side effects. Miltefosine, an oral
medication, is effective against both cutaneous and visceral
leishmaniasis. Side effects are usually mild, although it can
cause birth defects if taken within 3 months of conception.
 cquired Immunodeficiency Syndrome
A
As of 2018, of the estimated nearly 38 million people world-
wide living with human immunodeficiency virus (HIV),
approximately 1.7 million are children under 15 years of age.
More than 90% of HIV infections in children result from
mother-to-child transmission, either during pregnancy, child-
birth, or breastfeeding. The risk of transmission increases in
direct relation to the severity of the mother’s HIV infection [48].
HIV can be definitively diagnosed by virologic testing in
most nonbreastfed infants with perinatal HIV exposure by 1–2
months of age and in virtually all infants with HIV by 4–6 months
of age. Antibody tests, including the newer antigen-antibody
combination immunoassays, do not establish the presence of
HIV in infants because of transplacental transfer of maternal HIV
antibodies, and therefore, a virologic test must be used to confirm
the diagnosis [49, 50].
The HIV-induced chronic inflammatory response results
in lymphoid tissue damage and fibrosis, including frequent
involvement of the spleen which is a major site of secondary
lymphoid tissue, accounting for 25% of total body lympho-
cytes [51].
Children with acquired immune deficiency syndrome
(AIDS) are at increased risk for opportunistic infections,
including Pneumocystis carinii, cytomegalovirus, Candida
albicans, Mycobacterium tuberculosis, and M. avium-intra-
cellulare. The incidence of TB is five times higher in HIV-
infected children with severe immunosuppression compared
to HIV-infected children without significant immunosup-
pression [52]. Children and adolescents living with HIV
account for approximately 17% of all childhood deaths
from TB.
The ultrasound features of splenic microabscesses due to
the many opportunistic infections that can develop in chil-
dren with AIDS are nonspecific, and can have a variety of
appearances, ranging from diffuse splenomegaly to multiple
small hypo- or hyperechoic foci [53, 54]. Splenic abscesses
can calcify after treatment (Fig. 13.23). In children with TB,
ultrasound has been shown to detect splenic microabscesses
in 38% of children with concurrent HIV infection [52].
Bacillary angiomatosis-bacillary peliosis is an infection
caused by Bartonella henselae or B. quintana and occurs pri-
marily in severely immunocompromised patients, including
those with HIV. The infection has no age predilection, having
been reported in patients ranging from infancy to old age.
502 P. Duffy et al.

Cutaneous and subcutaneous lesions are the most com-

mon manifestation of bartonella infection in the immuno-
compromised host and have been described in 55% of
patients. In addition, 24% of patients present with fever and
abdominal symptoms [55]. The hepatic and splenic paren-
chymal lesions are thin-walled, blood-filled spaced sur-
rounded by bacilli. Ultrasound evaluation of the spleen
demonstrates multiple small hypoechoic lesions [56].
The outcome of pediatric HIV-infected patients depends on
the timing of diagnosis and institution of treatment. Early
intervention with administration of highly effective combina-
tion antiretroviral therapy has resulted in a significant decline
in mortality, a decreased incidence of opportunistic infections,
and an improved growth. In the absence of diagnosis and treat-
ment, one third of infected infants will die before 1 year of
age, and almost half before their second birthday [48].

Inflammatory Disorders

Sarcoidosis
Fig. 13.23 Chronic Mycobacterium avium-intracellulare infection of
the spleen in a 14-year-old male with acquired immune deficiency syn-
drome (AIDS). Coronal (a) and transverse (b) grayscale ultrasound
images of the spleen reveal multiple punctate, echogenic parenchymal
foci compatible with calcification and consistent with healed granulo-
mas. Arrow, Accessory spleen
Sarcoidosis is a granulomatous disease characterized by
multiple noncaseating granulomas that can affect multiple
organs. The disease is less common in children than in adults,
with an incidence that increases with age and peaks at
13–15 years of age. The true incidence in children is unknown
(estimated 0.29/100,000 children per year), because there is
no definitive diagnostic test, even in symptomatic children,
and sarcoidosis is a diagnosis of exclusion [57].
Two distinct forms of childhood sarcoidosis appear to
exist. Children less than 5 years of age with early-onset sar-
coidosis have a sporadic mutation of the NOD2 gene and
present with a clinical triad of uveitis, arthritis, and skin rash.
Blau syndrome is a rare, autosomal dominant inflammatory
disease characterized by a clinical triad of granulomatous
dermatitis, recurrent granulomatous uveitis, and systemic
polyarthritis, also with a mutation of the NOD2 gene.
Older children and young adults present most frequently
with multisystemic disease that includes lymphadenopathy,
pulmonary, ocular and cutaneous involvement (erythema
nodosum), and subsequently develop joint and hepatosplenic
manifestations. The disease patterns and clinical outcomes
are similar to those in adults.
Approximately 25% of affected individuals with sarcoid-
osis develop chronic or progressive disease. Manifestations
of sarcoidosis associated with poor prognosis and a relatively
high risk of death include treatment-resistant pulmonary,
cardiac, neurological, and multiorgan sarcoidosis [57, 58].
On ultrasound, the spleen is enlarged with hypoechoic
nodules that measure from 1 mm to 3 cm (Fig. 13.24) [15].
There may be enlarged lymph nodes in the splenic hilum and
retroperitoneum.
13 Spleen and Peritoneal Cavity
a b
Fig. 13.24 Sarcoidosis of the spleen in a 15-year-old male with hyper-
calcemia and interstitial lung disease. Coronal (a) and transverse (b)
grayscale ultrasound images show splenic enlargement and inhomoge-
neity related to the presence of innumerable hypoechoic parenchymal
Corticosteroids are the mainstay of therapy in sarcoidosis.
Low-dose methotrexate is an effective, steroid sparing, and
safe adjunct for treating patients with multiorgan involve-
ment. The overall prognosis of pediatric sarcoidosis is good
when compared with the prognosis for adults, with most
children experiencing considerable improvement in clinical
manifestations, chest radiographic findings, and pulmonary
function test results. The prognosis is worse in younger chil-
dren and when there is multiorgan involvement [58].
Rheumatic Disorders
Juvenile idiopathic arthritis is a heterogeneous group of disor-
ders characterized by arthritis of unknown origin with onset
before the age of 16 years. Systemic juvenile idiopathic arthri-
tis (Still disease) is characterized by extra-articular features
such as fever, rash, hepatosplenomegaly, lymphadenopathy,
serositis, and laboratory abnormalities, including elevated
inflammatory markers and leukocytosis [59]. Splenomegaly
occurs in about 50% of patients (Fig. 13.25) [60].
503
nodules. The left-sided renal pyramids (arrowheads) are diffusely echo-
genic in keeping with nephrocalcinosis. K, Left kidney. (c) Axial lung
window CT image of the chest reveals innumerable bilateral interstitial
micronodules
Childhood rheumatic disorders can rarely present with or
develop macrophage activation syndrome. The criteria for
diagnosis of this syndrome include fever, splenomegaly, pan-
cytopenia, hypertriglyceridemia, hypofibrinogenemia, hemo-
phagocytosis in the spleen, marrow or lymph nodes, low or
absent natural killer (NK) cell activity, and hyperferritinemia.
This syndrome can be life threatening and is associated with
central nervous system dysfunction and hemorrhage [61].
Although it occurs most often in the setting of systemic juve-
nile idiopathic arthritis, it can also occur in association with
systemic lupus erythematosus, sarcoidosis, dermatomyositis,
and Kawasaki disease [62, 63]. Episodes are most commonly
triggered by viral infections or during periods of high disease
activity such as disease onset.
The clinical presentation of macrophage activation syn-
drome at the onset of juvenile idiopathic arthritis has been
described in 22% of patients [61]. A mild subclinical course
can be seen in up to one third of patients with active systemic
disease [59, 63]. Some studies support the concept that sys-
504
Fig. 13.25 Systemic juvenile idiopathic arthritis (Still disease) in a 17-year-
old female with a history of arthralgias and fever of unknown origin.
Longitudinal grayscale ultrasound image demonstrates a mildly enlarged
spleen that measured 14 cm in length. The splenic parenchyma is homo-
geneous in echotexture
temic juvenile idiopathic arthritis and macrophage activation
syndrome are different points along a spectrum of disease [59].
Hepatosplenomegaly and lymphadenopathy occur in less
than one third of patients with systemic juvenile idiopathic
arthritis [59]. However, splenomegaly is present in 58% of
patients with juvenile idiopathic arthritis who present with
macrophage activation syndrome [61].
Although the imaging findings are nonspecific, ultrasound
can readily demonstrate the hepatosplenomegaly associated
with these varied rheumatic disorders.
Treatment of pediatric rheumatic disorders depends on the
severity of the presentation and whether there are signs of
macrophage activation syndrome. The initial choice of therapy
is nonsteroidal anti-inflammatory drugs. If there is a poor ini-
tial response or when other serious systemic manifestations
are present, biologic agents that inhibit interleukin or gluco-
corticoids and methotrexate are used. Hematopoietic stem-cell
transplantation is a potential option in patients with severe or
refractory disease.
Granulomatosis with Polyangiitis
Granulomatosis with polyangiitis, formerly known as Wegener
granulomatosis, is a rare multisystemic autoimmune disease
of unknown etiology. Its main features include necrotizing
granulomatous inflammation and pauci-immune vasculitis
P. Duffy et al.
affecting small- and medium-sized blood vessels. This is a rare
disorder that usually affects adults and is extraordinarily rare
in children [64].
Typically, the upper and lower respiratory tracts are
affected, and the kidneys are often involved as well. Other
organs, including the skin, joints, heart, eyes, and central
nervous can also be affected, although less frequently. In
contrast, splenic involvement has only rarely been reported.
A range of abnormalities have been described, including
splenomegaly, capsular adhesions, and infarction. A limited
number of autopsy studies have shown a high percentage of
splenic lesions, such as vasculitis, granuloma formation,
necrosis, and capsulitis, suggesting that asymptomatic spleen
involvement in this disorder may be fairly common.
Ultrasound imaging of infarction can have a variable appear-
ance [65]. Typical findings include a peripheral, wedge-shaped,
hypoechoic lesion. However, a splenic infarct can also manifest
as a multinodular or mass-­like focus with irregular margins.
Hemoglobinopathies
Hemoglobinopathies are genetic disorders affecting the struc-
ture or production of the hemoglobin molecule. They fall into
two main groups: sickle cell disease, a group of structural
hemoglobin variants, and thalassemia syndromes. The main
structural hemoglobin variants are hemoglobin (Hb)S, HbE,
and HbC. The main types of thalassemia are alpha (α)- and
beta (β)-thalassemia. There are many subtypes and combined
types in each group of disorders.
The clinical manifestations of the hemoglobinopathies
are highly variable, ranging from mild hypochromic anemia
to severe, lifelong, transfusion-dependent anemia with mul-
tiorgan dysfunction [66, 67].
Sickle cell disease virtually always affects the spleen. In
affected individuals, the spleen is normal in size and function
at birth. Splenic dysfunction (hyposplenism) is inversely
related to the amount of circulating fetal hemoglobin and
reflects the severity of hemolysis and intravascular sickling.
Functional asplenia in young patients is a high-risk factor for
overwhelming infection with encapsulated bacteria [68, 69].
Repeated vaso-occlusive episodes lead to ischemia, fibro-
sis, and progressive atrophy of the spleen, also known as
autosplenectomy. In most patients, vaso-occlusive events in
the spleen are clinically silent and result in progressive atro-
phy of the organ. In a small number of children, acute splenic
sequestration may precipitate hyposplenism [9]. As a result
of improved supportive care and the use of acute and chronic
transfusion therapy, autosplenectomy is currently rare in
children less than 5 years of age.
The spleen is considered small if its length on ultrasound
evaluation is between 2 and 3 standard deviations (SDs) below
the mean for age (Fig. 13.26). If splenic length is less than 3 SD
below the expected mean for age or the spleen is not visible, the
patient is considered to have undergone autosplenectomy.
13 Spleen and Peritoneal Cavity
a
S
b associated with sickle cell disease and thalassemia. In older
S children, contact sports should be restricted in order to avoid
K splenic enlargement due to trapping of a significant proportion
Fig. 13.26 Atrophic spleen with regenerative nodule in an 11-year-old
male with sickle cell disease. Longitudinal (a) and transverse (b) gray-
scale ultrasound images show a shrunken spleen (S) with hyperechoic
parenchyma that is significantly more echogenic than the adjacent left
kidney (K). There is a centrally located, hypoechoic regenerative
splenic nodule (arrows)
Patients who have been treated with blood transfusions
have echogenic spleens and a higher frequency of regenera-
tive nodules than patients who have not been transfused [70].
Regenerative nodules are usually hypoechoic compared to
the surrounding echogenic parenchyma, although in young
children they can appear echogenic and demonstrate vascu-
larity when evaluated with color Doppler ultrasound. Focal
splenic infarcts also occur. Foci of chronic infarction may
demonstrate curvilinear or punctate calcifications [15].
Splenomegaly results from trapping of sickled red cells in
the red pulp. Persistently high levels of fetal hemoglobin
promote splenomegaly. Splenomegaly can coexist with func-
tional hyposplenism and is most commonly seen in infancy.
Splenic volume increases in most infants with sickle-cell
anemia, although size does not correlate with function [71].
505
In patients with thalassemia major, splenomegaly and severe
anemia can develop in patients by 6–24 months of age if a
transfusion program has not been initiated. Other clinical
features include growth retardation, poor muscle develop-
ment, high output cardiac failure, jaundice, hepatomegaly,
genu valgum, leg ulcers, expansion of the bone marrow, and
extramedullary hematopoiesis [67].
When the spleen is ­palpable on physical examination or
when hypersplenism is clinically suspected, ultrasound is use-
ful in confirming the presence of splenomegaly with a splenic
length greater than 2 SD above the mean for patient age. A
spleen is considered to be massively enlarged if its lower pole
is located within the pelvis or if it crosses the midline.
There is no specific management for the splenomegaly
traumatic splenic rupture.
Acute splenic sequestration is a major cause of morbidity
and mortality in children with sickle cell disease and other
hereditary hemolytic anemias. It is characterized by sudden
of the blood volume, a rapid drop in hematocrit with hypovo-
lemia, and thrombocytopenia. Acute sequestration usually
occurs in infants and young children [9]. The acute event may
be self-limited and transient or lead to irreversible infarction.
Death can occur from anemia and hypovolemic shock [71].
Ultrasound imaging in patients with acute sequestration
demonstrates an enlarged spleen with hypoechoic zones
related to infarction (Fig. 13.27). Hyperechoic foci can some-
times be seen as well that reflect recent bleeding. Doppler US
will reveal patency of the splenic vasculature [7].
Immediate treatment includes blood transfusion. Sple­
nectomy is recommended for children older than 2–3 years of
age. Repeated blood transfusions may decrease the risk of
recurrence in children less than 2 years of age [66].
The only currently available treatment that can offer a poten-
tial cure for sickle cell disease is stem-cell ­transplantation.
Clinical trials of gene therapies for sickle cell disease are ongo-
ing and may offer a cure in the future. Drugs used to treat the
symptoms of sickle cell disease include analgesics, antibiotics,
ACE inhibitors, and hydroxyurea.
Although blood transfusion is an effective treatment for
the management of both acute and chronic complications
of sickle cell disease, it is expensive as is the required iron
chelation therapy used to prevent liver and other organ
damage as a result of iron overload. Transfusion is also
associated with the risk of alloimmunization. More than
90% of patients with sickle ­cell disease will survive into
adulthood. Optimally treated patients have a projected life
span of 50–60 years.
Stem-cell transplantation is the preferred treatment for
the severe forms of thalassemia. Supportive care consists of
506
lifelong periodic blood transfusions combined with iron che-
lation therapy.
Sickle-cell trait is a benign carrier state, and affected indi-
viduals usually have none of the symptoms of sickle-cell
anemia. Rare complications such as splenic infarction can
occur at high altitude associated with activities such as
mountain climbing, skiing, or flying in unpressurized air-
craft. Treatment is generally conservative.
c 0.2% [73].
Fig. 13.27 Acute splenic sequestration in a 12-year-old male with
sickle cell disease. Transverse (a) and longitudinal (b) grayscale ultra-
sound images demonstrate an enlarged, echogenic spleen with multiple
hypoechoic subcapsular foci (arrowheads) compatible with infarction.
(c) Longitudinal power Doppler image of the spleen reveals absence of
flow in the abnormal subcapsular regions
P. Duffy et al.
Lysosomal Storage Diseases
Lysosomal storage diseases are genetic disorders character-
ized by metabolic pathway deficiencies that lead to abnormal
accumulation of materials within the lysosome, such as
mucopolysaccharides, glycoproteins, amino acids, and lip-
ids. Many of these diseases manifest in infancy or early
Fig. 13.28 Gaucher disease in an 8-year-old male. Longitudinal gray-
scale ultrasound image shows an enlarged spleen (S) with homoge-
neous parenchyma. K, Left kidney
childhood and may have devastating consequences if
untreated, including early death. Early recognition is there-
fore imperative given the availability of new treatment
options to prevent irreversible damage [72].
Gaucher disease is an autosomal recessive disorder and is
the most common of the lysosomal storage diseases. Among
Ashkenazi Jews, there is an estimated birth incidence of
Gaucher disease is caused by deficiency of the enzyme
glucocerebrosidase, resulting in accumulation of glucosylce-
ramide in the reticuloendothelial system. There are three
main clinical variants: type I—onset in childhood or adult-
hood without neurodegeneration, type II—infantile onset,
and type III—onset in childhood or early adulthood. Types II
and III result in neurodegeneration [74].
Ultrasound findings include splenomegaly in all patients
(Fig. 13.28). Focal hypoechoic hepatic and splenic lesions
can also be seen in a smaller percentage of children
[73, 75]. These lesions are well defined, of varying size,
round or geographic in shape, and may be hypoechoic,
hyperechoic, or of mixed echogenicity (Fig. 13.29). These
nodules are thought to represent Gaucheromas, or clusters
13 Spleen and Peritoneal Cavity
a
b can confirm splenomegaly.
c used as disease designations) [72].
Fig. 13.29 Gaucher disease with splenic Gaucheromas in a 17-year-
old female. Coronal (a) and transverse (b) grayscale ultrasound images
of the spleen reveal multiple small echogenic parenchymal nodules
(arrowheads). (c) Coronal color Doppler ultrasound image shows
that the nodules are relatively avascular compared to the adjacent
splenic parenchyma
507
of Gaucher cells. After treatment, these lesions may resolve
completely without sequelae or can calcify.
In asymptomatic and untreated patients, incremental
changes in hepatic and splenic volume are proportional to
growth. Treated patients will also show a decrease in the
degree of splenomegaly. Early enzyme replacement ther-
apy with Ceredase or Cerezyme is effective in reducing
organomegaly, improving hematological parameters and
bone pain in patients with Gaucher disease [73].
Niemann-Pick disease includes a number of fatal autoso-
mal recessive disorders involving dysfunctional metabolism
of sphingolipids. The main clinical features include hepato-
splenomegaly, thrombocytopenia, and widespread neurolog-
ical dysfunction, including dystonia, abnormal postures,
supranuclear gaze palsy, dementia, and seizures [74]. There
are two main groups of patients, types A and B. Type B
patients present with hepatosplenomegaly in childhood with
slow deterioration and usual survival into adulthood. Ultrasound
No specific treatment is currently available. Enzyme
replacement has proven difficult. A source of targeted sphin-
gomyelinase, such as implantation of genetically engineered
autologous skin fibroblasts, has been proposed. Liver trans-
plantation and bone marrow transplantation have been tried
without significant success. Gene replacement may eventu-
ally become feasible [74].
The mucopolysaccharidoses are a group of inherited
disorders that manifest as a deficiency in enzymes that
degrade glycosaminoglycans. There are seven distinct
types: I—Hurler syndrome, II—Hunter syndrome, III—
Sanfilippo syndrome, IV—Morquio syndrome, VI—
Maroteaux-Lamy syndrome, VII—Sly syndrome, and
IX—Natowicz syndrome (types V and VIII are no longer
These conditions are differentiated by their clinical fea-
tures and age of presentation. Most types are inherited in
an autosomal recessive manner, although one specific form
(type II—Hunter syndrome) follows an X-linked pattern of
inheritance. The accumulated glycosaminoglycans cause
enlargement of multiple organs, with involvement of the
liver and spleen in Hurler and Sly syndromes.
Supportive management can improve the quality of life
for these patients and their families but does not prevent
the inevitable decline in function. However, specific ther-
apies such as enzyme replacement or hematopoietic cell
transplantation may alter the natural history of these
disorders.
The choice of therapy depends upon the particular
mucopolysaccharidosis and the disease severity in the
individual patient. Hematopoietic cell transplantation is
routinely offered to patients with Hurler syndrome who
are less than 2 years of age.
508
Portal Hypertension
Portal hypertension is an increase in the pressure within the
portal venous system. In children, one of the earliest signs of
portal hypertension is enlargement of the spleen. Splenomegaly
in portal hypertension is believed to result from blood pooling
within the red pulp of the spleen as a result of increased splenic
venous pressure [76]. However, studies in both adults and chil-
dren after liver transplantation have shown that in a proportion
of patients, the splenomegaly does not completely resolve
with reduction of the portal pressures [77–79].
Animal studies have suggested that in addition to vascular
congestion, splenomegaly in portal hypertension could be
due to lymphoid hyperplasia, angiogenesis, and fibrosis, pos-
sibly mediated through the mTOR pathway [80, 81].
Blockage of this pathway lead to reduction in splenic size in
rat models for both prehepatic and intrahepatic forms of por-
tal hypertension [80].
The progressive fibrosis that the spleen undergoes in the
setting of portal hypertension is increasingly being recog-
nized as a diagnostic marker for the severity of portal
hypertension. Splenic stiffness measured by elastography
is a good predictor for the presence of esophageal varices,
both in children and in adults, and is possibly more reliable
than measurements of liver stiffness [82–86]. It is possible
that these assessments will become a standard component
of ultrasound examination of the abdomen in the future
when evaluating patients with suspected or confirmed por-
tal hypertension.
Hypersplenism can be seen with portal hypertension with
decreased levels of red blood cells, white blood cells, and
platelets resulting from sequestration within the spleen. The
degree of hypersplenism does not always correlate well with
splenic size, however, since other factors such as bone mar-
row suppression can play a role in some underlying causes of
portal hypertension such as cirrhosis [87, 88].
In cases where the underlying etiology of portal hyperten-
sion and splenomegaly cannot be adequately treated and the
degree of hypersplenism is severe, total splenectomy can be
performed to improve the anemia and thrombocytopenia
[89]. Due to the desire to preserve the spleen for its immuno-
logic role in children, a distal splenorenal shunt (Warren
shunt) can be performed [90].
By ultrasound, an enlarged spleen in the setting of
­portal hypertension will have a homogenous echotexture.
The splenic vein will often appear enlarged on grayscale
imaging. Spectral Doppler ultrasound may demonstrate
increased pulsatility with to-and-fro flow, and reversal of
P. Duffy et al.
flow in severe cases (Fig. 13.30). Flow velocity can also
be diminished. In children, the main portal vein normally
has a diameter ranging from 5 to 9 mm [91, 92]. A splenic
vein diameter approaching these values should raise con-
cern for portal hypertension.
Additional signs of portal hypertension include peri-
splenic, splenorenal collateral veins, and perisplenic ascites.
Gamna-Gandy bodies (siderotic nodules), further described
below, can also be seen.
Children with portal hypertension, thrombocytopenia, and
splenomegaly are at risk for hemorrhage from esophageal vari-
ces. These patients are usually evaluated with ­endoscopy where
endoscopic variceal ligation can be performed. Treatment of
the underlying liver disease can often control the degree of
hypertension. Acute bleeding from varices is managed with vol-
ume restitution to maintain hemodynamic stability.
Trauma
The spleen is one of the most commonly injured organs in
children following blunt abdominal trauma and is also
damaged after penetrating injuries [93]. In children, the
spleen is only partially protected by the rib cage. Blunt
traumatic injures most often occur in motor vehicle acci-
dents that result in left-sided rib fractures and splenic lac-
erations [93, 94]. Contact sports, bicycle accidents, and
falls can also lead to blunt splenic injury, particularly in
children at increased risk, such as those with a recent his-
tory of mononucleosis.
The FAST (focused assessment with sonography for
trauma) scan is frequently performed in the emergency
department to assess unstable patients with high-energy
blunt abdominal trauma to visualize free fluid in the hepa-
torenal recess, subxiphoid pericardium, pouch of Douglas,
and the perisplenic region. This study is performed to
quickly evaluate patients for hemoperitoneum that may be
the cause of the hemodynamic instability and will require
immediate surgery. Hemothorax and pneumothorax can
also be recognized. However, conventional ultrasound is
not sufficiently sensitive to reliably identify parenchymal
injury.
A FAST scan that shows no free fluid in the abdomen
can be falsely reassuring with respect to solid organ
injury, and it is no longer recommended as an appropriate
imaging modality in pediatric abdominal trauma [95]. The
American Association for the Surgery of Trauma (AAST)
classification scheme is an injury scoring scale commonly
13 Spleen and Peritoneal Cavity
a b
Fig. 13.30 Portal hypertension in a 6-year-old male with biliary atresia
and liver cirrhosis. (a) Transverse grayscale ultrasound image reveals a
massively enlarged spleen (S) and prominent hilar vessels (arrows). (b)
Color Doppler ultrasound image with spectral analysis shows mildly
pulsatile flow in the splenic vein. (c) Transverse color Doppler ultra-
used to categorize splenic injuries in both children and
adults (Fig. 13.31). This system has five grades of injury
that include the extent of any subcapsular hematoma, intra-
parenchymal hematoma, and/or parenchymal laceration as
determined by CT imaging [96].
509
sound image demonstrates a splenorenal shunt with the splenic vein
(SPL) draining directly (arrowhead) into the left renal vein (LRV). S,
Spleen; K, left kidney. (d) Transverse grayscale ultrasound image of the
epigastrium shows multiple enlarged round and tubular anechoic struc-
tures (asterisks) representing mesenteric collateral veins
While ultrasound can detect hemoperitoneum as well as
hematomas and lacerations (Fig. 13.32), it has generally had
a poor performance in the detection of solid visceral injury
when compared to CT. The diagnostic accuracy of ultra-
510 P. Duffy et al.

Grade I Grade II

Laceration Laceration
< 1 cm 1–3 cm

Subcapsular Subcapsular
hematoma hematoma
< 10% of 10-50% of
surface area surface area

Grade Ill

Laceration
> 3 cm Ruptured
subcapsular or
Subcapsular parenchymal
hematoma hematoma
>50% of
surface area

Grade IV Grade V

Shattered spleen

Hilar injury

Segmental or
hilar vascular
injury Devascularization
> 25% of spleen

Fig. 13.31 Diagram illustrating the 5 grades of splenic injury according to the American Association for the Surgery of Trauma (AAST) classifi-
cation scheme
13 Spleen and Peritoneal Cavity
a
b Splenosis
Fig. 13.32 Grade III splenic laceration in an 8-year-old male after a
fall. (a) Longitudinal grayscale ultrasound image of the spleen reveals
a heterogeneous appearance of the lower pole parenchyma (arrows)
with a small amount of perisplenic fluid (asterisk). (b) Coronal con-
trast-enhanced CT image confirms the intraparenchymal hematoma
(arrow) and surrounding subcapsular hematoma (asterisks)
sound is significantly improved by performing CEUS and is
similar to that of CT. Active bleeding can also be identified.
The spleen should be evaluated during the portal venous
and delayed phases of contrast enhancement. The portal
venous phase starts at about 30–45 seconds following
injection and continues for 2–3 minutes, while the delayed
phase can last up to about 5–7 minutes postinjection.
Splenic injuries can appear as linear, branched, or complex
511
perfusion defects compared to the adjacent uniformly enhanc-
ing splenic parenchyma (Fig. 13.33). Perisplenic and subcap-
sular fluid also become more visible.
Congenital clefts are sometimes be mistaken for trau-
matic injuries but have regular and homogeneous margins
compared to lacerations. CEUS can also be useful in fol-
low-up of known traumatic injuries and avoid the need for
ionizing radiation associated with CT [3, 97].
Management is dictated by the clinical course of the
patient. Hemodynamically stable patients can be treated
nonoperatively, which is possible in more than 90% of the
time [93]. Very few patients will require a blood transfusion.
Splenorrhaphy, splenic artery embolization, partial splenec-
tomy, or splenectomy can be considered if there is hemody-
namic instability.
Splenosis is a rare, acquired condition where there is autotrans-
plantation of one or more focal deposits of splenic tissue in dif-
ferent locations within the body. It most commonly occurs as a
result of traumatic splenic rupture or abdominal surgery. While
the location of these implants is generally intraperitoneal, there
have been reports of unusual sites of implantation that include
the chest, pancreas, and wall of the colon [98–101]. While these
lesions are benign in nature, they can sometimes be mistaken
for other abnormalities such as tumor.
By ultrasound, splenosis appear as small, rounded 1–3 cm
masses that are virtually identical to accessory spleens
(Fig. 13.34). If a normal spleen is present, these nodules will
appear similar in echogenicity and vascularity to the spleen.
Unlike accessory spleens which are usually located near the
splenic hilum, the masses of splenosis occur throughout the
peritoneum and the retroperitoneum.
Differentiation of splenosis from malignancy can be
achieved either with a radionuclide 99 m-Tc-sulfur colloid
scan or with a heat damaged 99 m-Tc-red blood cell scan
demonstrating uptake similar to the spleen. These are inci-
dental lesions that do not require intervention.
Vascular Anomalies
Lymphatic Malformation
Lymphatic malformation (LM) of the spleen is rare and
occurs most often as a component of a complex lesion where
there are numerous sites of lymphatic malformation through-
out the body. In the spleen, the capsule and trabeculae con-
tain the largest number of lymphatics, and therefore, LMs
512 P. Duffy et al.

a c

Fig. 13.33 Grades II–III splenic laceration in a 16-year-old male sus- zone of absent parenchymal enhancement (arrows) in keeping with a
tained in a motor vehicle accident. (a) Longitudinal grayscale ultra- laceration. (c) Longitudinal contrast-enhanced CT image depicts the
sound image of the spleen reveals no abnormality. (b) Longitudinal splenic injury (arrowhead)
CEUS image obtained several minutes later shows an irregular linear

tend to occur in the periphery or hilum of the spleen and can and soft tissues; pleural effusions are also frequently present.
be solitary or multiple [102–106]. Gorham-Stout disease, also known as “disappearing bone
Generalized lymphatic anomaly is a disorder that includes disease,” manifests with progressive regional osteolysis due
multifocal well-­circumscribed lesions of the spleen, bones, to an infiltrative LM and osteoclastic activity. Visceral involve-
13 Spleen and Peritoneal Cavity 513

amount of flow with color and spectral Doppler, LMs are

a
avascular.
Image-guided intralesional sclerotherapy alone or in
combination with surgical resection is the standard treatment
for LMs. However, these procedures are rarely curative if the
lesions are extensive or infiltrating. More recently, medical
therapy with sirolimus has been used successfully for treat-
ment of microcystic LMs [108].

Venous Malformation
Venous malformation (VM) of the pediatric spleen is rare
[109, 110]. It is characterized by dysplastic venous channels
frequently deficient in elastic lamina and smooth muscle
[111]. These lesions can be solitary or multiple, with multi-
ple sites occurring in the setting of complex venous malfor-
mations such as blue rubber bleb syndrome [112].
The true incidence of splenic VM is difficult to accurately
determine due to the lack of consensus in the literature
regarding nomenclature, with these lesions often described
as “hemangiomas” and “tumors.” Histologically, there are
dilated veins lined by a thin layer of endothelial cells. Smooth
muscle may be present in the vessel walls and intraluminal
b calcified thrombi can also be identified.
VMs of the spleen are slow-flow lesions that often appear
by ultrasound as cystic spaces of varying size (Fig. 13.37).
Focal calcification related to phleboliths can often be seen.
Venous flow can sometimes be detected with color and spec-
tral Doppler, although frequently no signal is identified [15].
Treatment of VM involves a multidisciplinary approach
with observation, sclerotherapy, with or without surgical
resection depending on the extent of involvement [97].

Kaposiform Lymphangiomatosis
Fig. 13.34 Splenosis in a 14-year-old female who underwent splenec-
tomy 7 years earlier. (a) Transverse grayscale ultrasound image of the
epigastrium shows a rounded, homogeneous, hypoechoic mass (arrow)
abutting the anterior peritoneal surface of the abdomen. (b) Axial con-
trast-enhanced CT image depicts the mass of ectopic intra-­abdominal
splenic tissue (arrow)
ment and macrocystic LMs are less frequent than with gen-
eralized lymphatic anomaly.
By ultrasound, an LM will usually appear as an anechoic
or hypoechoic solitary cyst, as a cluster of multiple cysts,
or as a dominant cyst with satellite lesions (Figs. 13.35 and
13.36) [107]. The cysts may contain fluid-fluid levels, espe-
cially if they have undergone hemorrhage. Apart from some
small septal arteries and veins that may show a small
Kaposiform lymphangiomatosis (KLA) is a rare, complex
lymphatic anomaly that exhibits features of both malformation
and neoplasia. The histopathology combines malformed lym-
phatic channels and a proliferative spindle cell component. The
spindle cell component in kaposiform lymphangiomatosis is
usually sparse, with dispersed and poorly marginated clusters
of parallel-oriented spindle cells [113]. The dilated lymphatic
channels can leak lymphatic fluid, resulting in chylothorax and
chylous ascites. Two thirds of patients have mild-to-moderate
thrombocytopenia, and about half of them have associated
splenomegaly. Affected spleens may demonstrate clusters of
round, anechoic, or hypoechoic lesions.
The natural history of KLA and the optimal treatment
regimen are currently uncertain. The majority of patients
with KLA require a combination of medical and interven-
tional treatment. Splenectomy has been performed for treat-
ment of thrombocytopenia. Medical therapies, including
vincristine, corticosteroids, interferon, and sirolimus, have
all been recommended although the response to therapy is
514 P. Duffy et al.

a b

Fig. 13.35 Lymphatic malformation (LM) of the spleen in a 6-year-old male who presented with abdominal pain. (a) Longitudinal grayscale ultrasound image
shows an irregular, anechoic cyst (asterisk) in the lower pole of the spleen (S). (b) Transverse color Doppler ultrasound image shows no flow in the lesion

a b
e

c d

Fig. 13.36 LM of the spleen in an 8-year-old female with central conduct-
ing lymphatic anomaly (CCLA). (a) Coronal grayscale ultrasound image
of the spleen demonstrates multiple anechoic parenchymal cystic structures
(arrowheads). (b) Coronal grayscale ultrasound image of the lower pole of
the spleen depicts a complex cyst (arrow) that is avascular when evaluated
with color Doppler (c). (d) Transverse grayscale ultrasound image of the
pelvis shows a multiloculated cystic mass (arrowheads) medially d­ isplacing
the bladder (B). e) Coronal maximum intensity projection (MIP) image
from an MR lymphangiogram shows multiple abnormalities, including
dilated left-sided lumbar lymphatic channels (black arrow), a cystic right
lumbar channel (black arrowhead), and bulbous dilation (white arrowhead)
of the terminal segment of the thoracic duct
13 Spleen and Peritoneal Cavity
a b
Fig. 13.37 Venous malformation (VM) of the spleen in a 7-year-old
female with blue rubber bleb nevus syndrome. (a) Longitudinal grayscale
ultrasound image of the spleen demonstrates two rounded, anechoic
parenchymal lesions (arrows). (b) Transverse color Doppler ultrasound
image of the spleen reveals no detectable flow within the lesions (arrows).
(c) Coronal contrast-enhanced, soft tissue window CT image of the chest
unpredictable. The mortality rate of KLA is high despite
aggressive multimodal therapy [114].
Peliosis
Peliosis is a rare disorder of the reticuloendothelial system that
can involve the liver, spleen, and occasionally the bone marrow.
Isolated splenic peliosis is very uncommon. Epithelial break-
515
shows a large mediastinal mass (arrows) containing punctate foci of
increased density representing phleboliths, consistent with a VM. Two
round, nonenhancing lesions (arrowheads) in the right thyroid lobe are
also compatible with VMs. The patient had a right pneumonectomy in
early childhood
down leads to multiple blood-filled cystic spaces within the
affected organs. Peliosis is usually discovered incidentally and
is associated with oral contraceptives, steroids, hematologic
malignancy, and HIV/AIDS. Rarely, the cysts can rupture spon-
taneously and cause life-­threatening hemoperitoneum [115].
Ultrasound imaging reveals hypoechoic lesions through-
out the spleen. CT shows unenhancing or mildly enhancing
hypodense lesions. MR imaging depicts multiple foci with
516 P. Duffy et al.

variable signal on both T1- and T2-weighted images that

a
reflect the age of the blood within the cysts [115].
Once a diagnosis of peliosis has been made, some authors
advocate that an effort be made to determine its presence in
other organs and to establish a possible cause since spontane-
ous rupture of an affected organ is an ever-present threat [116,
117]. Definitive treatment is splenectomy, and ­prophylactic
surgery should be considered to avoid the possibility of
splenic rupture.

Benign Masses

Hamartoma
A hamartoma is a benign growth consisting of a disorganized
mixture of cells and tissues that are normally found in the body b
part where the growth occurs. In the spleen, this proliferation
consists of vascular endothelial lining cells [118]. A hamartoma
is often solitary although multiple hamartomas can occur in
tuberous sclerosis and Wiskott-Aldrich syndrome [119].
By ultrasound, a hamartoma appears hypoechoic or hetero-
geneous without a visible capsule and demonstrates increased
vascularity with color Doppler (Fig. 13.38). Splenectomy is
curative.

Extramedullary Hematopoiesis
Extramedullary hematopoiesis is the production of blood
cells outside of the bone marrow which occurs when there
is inadequate production of blood cells. The most common
causes include myelofibrosis, diffuse osseous metastatic
disease replacing the bone marrow, leukemia, sickle cell
disease, and thalassemia. During fetal development, hema-
topoiesis occurs primary in the fetal liver with additional
sites in the yolk sac and spleen. After birth, hematopoiesis
typically transitions entirely to the bone marrow although
quiescent hematopoietic stem cells are still present in the
spleen [120].
When blood cell production by the bone marrow is defi-
cient, these stem cells can be activated to assist in sustaining
hematopoiesis. The liver and the spleen are the most com-
mon sites for extramedullary hematopoiesis. Patients can
present with hepatosplenomegaly and abdominal discom-
fort, as well as shortness of breath in the case of massive
enlargement. Patients with splenic infarction can present
with pain.
On ultrasound evaluation, the spleen is typically uniformly
enlarged. Less often, it may demonstrate numerous small
hypo-, iso-, or hyperechoic nodules (Fig. 13.39) [121–123].
Symptomatic treatments include blood transfusion and sple-
nectomy. Hematopoietic stem-cell transplant is the only cura-
tive treatment for myeloproliferative neoplasm-­ associated
myelofibrosis [124].
Fig. 13.38 Hamartoma of the spleen in a 13-year-old male undergo-
ing evaluation for splenomegaly. (a) Longitudinal grayscale ultra-
sound image demonstrates a solid, well-circumscribed and slightly
hypoechoic mass (calipers) in the mid-portion of the spleen. (b)
Longitudinal color Doppler ultrasound image of the lesion shows
moderate internal vascularity
Inflammatory Myofibroblastic Tumor
Inflammatory myofibroblastic tumor is a rare mesenchymal
neoplasm characterized by proliferation of myofibroblasts
with an inflammatory infiltrate that can develop in numerous
organs of the body, including the spleen [125–127]. The eti-
ology of this tumor is not well understood, although there
does appear to be an association with Epstein-Barr viral
infection [126, 128], as well as disruption of a specific RNA
decay pathway [129]. Symptoms include left upper quadrant
pain, fever, and malaise [126, 130].
By ultrasound, this lesion typically presents as a well-­
defined, lobulated, hypoechoic mass without a capsule or
13 Spleen and Peritoneal Cavity
a b
c
Fig. 13.39 Extramedullary splenic hematopoiesis in an 8-year-old
female with sickle beta zero thalassemia. (a, b) Longitudinal grayscale
ultrasound images of the spleen show multiple rounded, echogenic
increased vascularity. Partial or total splenectomy is both
diagnostic and curative, and the prognosis is generally
favorable.
Hemangioma
Infantile hemangioma is the most common vascular tumor in
children. It can be focal or diffuse and expresses the glucose
transporter 1 protein (GLUT1). Congenital hemangiomas are
less common vascular tumors which are present at birth. They
are GLUT1 negative and can be rapidly involuting, partially
involuting, or noninvoluting [131].
As previously noted in the discussion of venous malfor-
mation of the spleen, the true incidence of splenic hemangio-
mas, infantile and congenital, is difficult to ascertain since
the nomenclature and basis for classification of vascular
lesions of the pediatric spleen are not well standardized. A
517
d
parenchymal nodules (arrows). Longitudinal color (c) and power (d)
Doppler ultrasound images reveal moderate hypervascularity of the
nodules
handful of case reports in the literature have described what
may have been bona fide congenital [132] and infantile [133]
hemangiomas based on their clinical presentation, high-flow
vasculature, and clinical course.
 ittoral Cell Angioma
L
Littoral cell angioma is a rare primary vascular tumor that
arises in the spleen from the red sinus shore cells of the retic-
uloendothelial system that line the red pulp [134]. Most of
the reported cases describe a benign tumor that can present
as a solitary lesion as well as multiple recurrent lesions,
although it can occasionally metastasize [135, 136]. The lit-
toral cells express both endothelial and histiocytic antigens,
a characteristic feature of double differentiation, in contrast
to the splenic red pulp cells that only express endothelial
markers. Diagnosis relies on morphology and immunohisto-
518
chemical studies. Fine-needle aspiration cytology can aid in
narrowing the differential diagnosis in some cases [134].
On ultrasound, the spleen is often enlarged. Solitary or
multiple ill-defined, hypo- or hyperechoic foci of variable
vascularity can be observed [137–139].
Splenectomy is usually performed since the imaging fea-
tures of littoral cell angioma are not sufficiently characteris-
tic to permit differentiation from other lesions, including
malignant tumors.
Malignant Tumors
Lymphoma
Primary lymphoma of the spleen is rare, accounting for less
than 1% of all cases of lymphoma [140]. More often, the
spleen is one of several sites of involvement, occurring in
approximately 30% of cases of Hodgkin lymphoma and
a c
Fig. 13.40 B-cell lymphoma in a 12-year-old female with splenomegaly.
Coronal (a) and transverse (b) grayscale ultrasound images reveal a mas-
sively enlarged spleen (S) without a well-defined focal nodule. There is a
tiny amount of free fluid (arrowhead) at the lower splenic pole. Several
P. Duffy et al.
30–40% of cases of non-Hodgkin lymphoma [141]. Involvement
of the spleen in lymphoma can affect staging, influence treat-
ment, and change overall prognosis.
The appearance of the spleen with lymphoma is variable
[141]. Massive enlargement is usually indicative of splenic
involvement in a patient with known lymphoma, although
mild-to-moderate reactive splenomegaly without lymphoma-
tous infiltration occurs in 30% of patients with Hodgkin lym-
phoma and 70% of patients with non-Hodgkin lymphoma.
Conversely, splenic involvement with lymphoma is seen in
one third of patients without splenic enlargement, and it is
the abdominal site most commonly affected by occult dis-
ease [142].
Four imaging patterns have been described for lymphoma of
the spleen with imaging correlation: homogeneous splenomeg-
aly without a focal lesion, diffuse infiltration with miliary
lesions less than 5 mm, multiple focal nodular lesions (1–10 cm),
and a large solitary mass (Figs. 13.40, 13.41, and 13.42) [143].
enlarged, hypoechoic hilar lymph nodes (arrows) are identified. K, Right
kidney. (c) Coronal contrast-enhanced CT image shows extensive intra-
peritoneal lymphadenopathy (arrowheads) in addition to homogeneous
splenomegaly. S, Spleen
13 Spleen and Peritoneal Cavity
a c
Fig. 13.41 Hodgkin lymphoma in a 14-year-old male with miliary splenic
involvement. (a) Transverse grayscale ultrasound image shows innumera-
ble small hypoechoic nodules throughout the spleen. (b) Axial fused posi-
tron emission tomography (PET)/CT and (c) coronal maximum intensity
On ultrasound, focal lesions tend to be hypoechoic without
acoustic enhancement [7]. Dystrophic calcification secondary
to necrosis is seen in patients with treated high-grade lym-
phoma. Lymphomatous involvement of the spleen increases
susceptibility to hypersplenism, infarction, trauma, and splenic
vein thrombosis.
Most treatment protocols use combination chemother-
apy regimens with or without radiation therapy. The role
of surgery is limited to biopsy to establish a diagnosis.
Patients with Hodgkin lymphoma and refractory disease
or relapse may be candidates for autologous hematopoi-
etic stem-cell transplant. Most children and adolescents
with lymphoma have a good prognosis. Survivors are at
increased risk for long-term sequelae of therapy and sec-
ondary malignancies.
Leukemia
The spleen is a common site of involvement in both acute
and chronic forms of leukemia, with splenomegaly a more
reliable marker of disease than in lymphoma. The spleen can
519
projection image from PET/CT data demonstrate multiple foci of intense
fluorodeoxyglucose (FDG) uptake (arrows) in the spleen. There is also
extensive FDG-­avid lymphadenopathy in the neck, right mediastinum,
upper abdomen, and retroperitoneum
be massively enlarged in several types of pediatric leukemia,
occasionally leading to rupture [144].
By ultrasound, most leukemias will show homogenous
enlargement of the spleen without a focal lesion (Fig. 13.43).
Focal leukemic lesions of the spleen occur primarily in the
setting of hairy cell leukemia which occurs almost exclu-
sively in adults [143].
Children are usually treated with a multiphase, multidrug
regimen (i.e., induction, consolidation, and maintenance).
Treatment protocols vary depending on the tumor immuno-
phenotype and risk category and can take 2–3 years to com-
plete. Supportive management includes transfusion and
antibiotics, correction of metabolic imbalances, and treat-
ment of adverse effects related to drug toxicity and tumor
lysis.
Treatment-related deaths remain a challenge and are of
particular concern in children less than 1 year of age, between
10 and 19 years of age, and in those with organ failure.
Allogeneic hematopoietic cell transplantation is an option
for some high-risk patients.
520
a a
Fig. 13.42 Hodgkin lymphoma in a 14-year-old male with a focal nodu-
lar splenic lesion. (a) Coronal color Doppler ultrasound image shows a
hypoechoic nodule (arrow) in the superolateral aspect of the spleen with
minimal internal vascularity. (b) Coronal image from an intravenous
CEUS study in the portal venous phase demonstrates early contrast wash-
out from the nodule (arrows), a sign strongly suggestive of malignancy
Angiosarcoma
Angiosarcoma of the spleen is an aggressive neoplasm aris-
ing from endothelial cells. The disease is seen mainly in
adults and only rarely in children. There is a high fatality rate
with a 5-year survival of approximately 40% despite treat-
ment [145]. Patients present with splenomegaly and left
upper quadrant abdominal pain. The major complication is
splenic rupture which occurs in approximately 13% of
patients and is considered a grave prognostic factor given the
high probability of widely disseminated disease [146].
P. Duffy et al.
K K
Fig. 13.43 Acute lymphoblastic leukemia with splenic involvement in
a 10-year-old male. (a) Transverse grayscale ultrasound image of the
spleen reveals moderate enlargement without a focal lesion. (b) Coronal
T2-weighted, fat-suppressed MR image shows homogeneous spleno-
megaly (S). Note the peripheral striated, hypointense foci in both kid-
neys (K) compatible with leukemic infiltrates. There is extensive body
wall edema (white arrowheads) and subcutaneous fluid collections
(black arrowheads)
Ultrasound findings are nonspecific in most cases. The
most common findings are splenomegaly, splenic heteroge-
neity, and poorly defined solid and cystic lesions represent-
ing sites of hemorrhage and necrosis. The more solid,
echogenic portions of the tumor will appear hypervascular
on color Doppler evaluation.
Tumors appear heterogeneous on CT with zones of low
density and necrosis. Noncontrast CT images may show areas
of high density caused by recent hemorrhage or calcification
from the hemosiderin deposition. Intravenous (IV) contrast
administration shows marked heterogeneous enhancement.
High-density ascites is highly suggestive of the spontaneous
hemorrhage from splenic rupture. Hypervascular metastatic
13 Spleen and Peritoneal Cavity 521

deposits have also been described primarily in the liver, but
also in the lungs, bones, and lymphatic organs.
MR imaging findings depend on the extent of hemor-
rhage and necrosis in the tumor. Areas of increased and
decreased signal intensity may be seen on images obtained
with both T1- and T2-weighted imaging.
In children, survival has been reported from less than
1 month to 16 years. Biopsy is contraindicated because of
the high risk of splenic rupture, and therefore, definitive
diagnosis is only made after splenectomy. Angiosarcoma in
children may be responsive to chemotherapy in some cases.
However, surgical extirpation of the primary neoplasm and
all metastases is necessary to achieve durable remission.
Kaposiform Hemangioendothelioma
Kaposiform hemangioendothelioma (KHE) is a rare, locally
aggressive, vascular tumor most commonly encountered during
infancy and childhood. The typical presentation is of an enlarg-
ing, tender mass typically located in the extremities, trunk, or
retroperitoneum that has the ability to cross tissue planes [147].
Splenic KHE is very rare with only a few published cases [148,
149]. This tumor is associated with the Kasabach-Merritt phe-
nomenon, a consumptive coagulopathy resulting from seques-
tration of platelets in the tumor. On ultrasound, KHE has been
described as a solid, hypoechoic, and hypervascular mass.
Due to concerns for the development of spontaneous
splenic rupture, thrombocytopenia, coagulopathy, and anemia,
patients are managed with partial or total splenectomy.
Alternative approaches to unresectable lesions include angi-
ography and embolization, radiation therapy, corticosteroids,
alpha interferon, antifibrinolytic agents, and vincristine.
Administration of blood products and fresh-frozen plasma
should be limited since this can increase the sequestration and
the size of the lesion [150].
Langerhans Cell Histiocytosis
Langerhans cell histiocytosis (LCH), the most common histio-
cytic disorder, includes conditions characterized by aberrant
function and differentiation or proliferation of cells of the
mononuclear phagocyte system. Granulomatous lesions com-
prising langerin-positive (CD207+) histiocytes and an inflam-
matory infiltrate can arise in virtually any organ system.
Splenic involvement by LCH usually presents as a part of a
multifocal systemic disease, whereas isolated splenic LCH is
very rare. Children with liver, spleen, or bone marrow involve-
ment are at highest risk for death from LCH and are therefore
classified as having high-risk LCH [151]. In children, the
spleen is involved in approximately 15% of cases [152].
Splenic involvement in LCH is usually manifested as dif-
fuse enlargement (Fig. 13.44). On ultrasound, there may be
hypoechoic nodules of varying size without increased vascu-
larity (Fig. 13.45) [153–155].
Although clinical outcomes have steadily improved over the
years, standard-of-care chemotherapy (vinblastine, prednisone,
and mercaptopurine) fails to cure more than 50% of children
with high-risk disease. Advances in treatment have been ham-
pered by a persisting uncertainty as to whether LCH is primar-
a
S

Metastatic Disease
K
Neuroblastoma
Spread of solid malignant tumors to the spleen occurs typically
with widespread disease. In children, neuroblastoma is the
most common tumor to metastasize to the spleen [7]. Despite b
its frequency at autopsy, splenic metastases from neuroblas-
S
toma are only rarely identified on clinical imaging studies.
Neuroblastoma is an embryonal malignancy of the sympathetic
nervous system that develops from pluripotent sympathetic
cells, and it is the most common extracranial tumor of child-
hood [150]. These tumors most commonly arise in the adrenal
medulla or paraspinal ganglia but can develop in any location
that contains sympathetic nervous tissue. The spleen does not
contain significant autonomic nervous tissue, and there are no
reports of primary splenic neuroblastoma. Fig. 13.44 Langerhans cell histiocytosis of the spleen in a 3-year-old
On ultrasound evaluation, splenic metastases from neuro- male. Coronal (a) and transverse (b) grayscale ultrasound images show
blastoma tend to be hypoechoic with varying degrees of vas- diffuse enlargement of the spleen (S) without a focal lesion. There is a
small amount of free fluid (arrow) adjacent to the lower splenic pole. K,
cularity with color Doppler [12]. Left kidney
522
a c
Fig. 13.45 Langerhans cell histiocytosis with extensive nodular splenic
involvement in a 2-year-old female. (a) Longitudinal grayscale ultrasound
image demonstrates multiple hypoechoic nodules (arrowheads) throughout
ily a neoplastic disorder, an immunodysregulatory disorder, or
a disorder with characteristics of both [151]. Recurrent rates
are high (30–50%) in patients with multisystem disease [156].
Hematopoietic stem-cell transplantation is an effective treat-
ment option for patients with severe refractory disease.
Complications of Pancreatitis
Due to the proximity of the splenic vessels and the pancreatic
tail to the spleen, pancreatitis can involve the spleen. The
most common splenic complication is thrombosis of the splenic
vein due to mass effect either from a pancreatic pseudocyst or
from inflammation of the splenic vein as it courses along the
body and tail of the pancreas.
The splenic artery can be damaged by the release of pan-
creatic enzymes where the resulting inflammation leads to
pseudoaneurysm formation [103, 157]. A splenic pseudoan-
eurysm can bleed into a pseudocyst, pancreatic duct, a bowel
loop, the retroperitoneum, or abdominal cavity.
P. Duffy et al.
the spleen. (b) Longitudinal color Doppler ultrasound image reveals no evi-
dence of nodular hypervascularity. (c) Axial T2-weighted, fat-suppressed
MR image depicts numerous hypointense splenic nodules. S, Spleen
Additional potential complications include splenic hema-
toma, pseudocyst extension into the splenic parenchyma,
abscess formation from septic pancreatitis, splenic infarc-
tion, and rupture [12].
Color and spectral Doppler will show no flow in a throm-
bosed splenic vein (Fig. 13.46). An arterial pseudoaneurysm
can be seen with color Doppler imaging, with aneurysmal
dilation defined as a focal dilation greater than 50% com-
pared to the normal vessel diameter. The normal diameter of
the splenic artery is approximately 4–5 mm.
Patients with splenic vein thrombosis, particularly with
varices seen on CT, should undergo regular upper gastroin-
testinal endoscopy with splenectomy reserved for those who
experience bleeding or have an increased risk of hemorrhage
[158]. Splenic artery pseudoaneurysms are generally observed
with intervention recommended only if they are 2 cm or
greater in diameter due to an increased risk of rupture.
Pseudoaneurysms can be treated using transcatheter emboli-
zation and/or surgical intervention with the choice of treat-
ment dependent on clinician and patient preference.
13 Spleen and Peritoneal Cavity
a b
Fig. 13.46 Splenic vein thrombosis in an 11-year-old female with nec-
rotizing pancreatitis. (a) Longitudinal grayscale ultrasound image of
the left upper quadrant shows a mildly enlarged spleen (S) with homo-
geneous parenchyma. The distal pancreas (arrowheads) appears dif-
fusely hypoechoic and swollen. (b) Transverse color Doppler ultrasound
image of the spleen shows the splenic artery (arrow) but no splenic vein.
(c) Spectral Doppler analysis of a small splenic hilar vessel shows a
Gamna-Gandy Bodies
Gamna-Gandy bodies, also known as siderotic nodules,
are discrete foci of hemosiderin and calcium that are depos-
ited in the splenic parenchyma after episodes of microhe-
morrhage. They occur most often in the setting portal
hypertension and sickle cell disease, although they can be
523
low-amplitude venous waveform compatible with flow in a collateral
vessel. (d). Axial contrast-enhanced CT image demonstrates a diffusely
swollen, nonenhancing pancreas (P). The medial splenic vein (arrow-
head) is dilated and contains low-attenuation intraluminal thrombus.
The lateral portions of the splenic vein are not visualized. Arrow, Superior
mesenteric vein
seen in other hemolytic anemias, following trauma and in
patients with acquired hemochromatosis [12]. On ultra-
sound, the nodules appear as numerous small echogenic
foci throughout the spleen either with or without distal
acoustic shadowing depending on the amount of depos-
ited calcium (Fig. 13.47) [12]. These lesions require no
treatment.
524
a
S
K The peritoneal cavity is evaluated with the highest frequency
b used in infants and small children, as well as when evaluat-
S Imaging Approaches
Fig. 13.47 Gamna-Gandy bodies in the spleen of a 17-year-old male
with cystic fibrosis and portal hypertension. Longitudinal (a) and trans-
verse (b) grayscale ultrasound images reveal marked enlargement of the
spleen (S). The splenic parenchyma contains numerous punctate echo-
genic foci without associated distal shadowing. There is a small amount
of fluid (asterisk) adjacent to the upper splenic pole. K, Right kidney
Peritoneal Cavity
Introduction
The peritoneal cavity is a potential space between the parietal
peritoneum and the visceral peritoneum, the membranes that
line the walls of the abdomen and pelvis and surround the
abdominal and pelvic organs. It usually contains a small
amount of fluid that acts as a lubricant. The peritoneal cavity
is not often the site of origin of pediatric disorders, although it
is frequently involved in a wide variety of disease processes
that originate at other sites in the abdomen and pelvis.
Ultrasound techniques for evaluating the peritoneal cavity are
reviewed along with a discussion of normal development and
anatomy. An overview of the ultrasound features of ascites,
localized peritoneal fluid collections, peritoneal inflammation,
P. Duffy et al.
and pneumoperitoneum is provided. Additional benign and
malignant disease processes affecting the peritoneal cavity are
also discussed.
Technique
Patient Positioning
Ultrasound of the peritoneal cavity is usually performed with
the patient in a supine position. Placing the patient in a lat-
eral decubitus position can help in distinguishing a fluid col-
lection from a cyst or other fluid-filled structure.
Ultrasound Transducer Selection
curvilinear transducer that can provide adequate depth pen-
etration [159]. A higher frequency linear probe can often be
ing superficial structures and the bowel.
The peritoneum and peritoneal cavity are initially surveyed
with a 3.5-MHz or 5-MHz transducer using a field of view that
includes the full depth of the peritoneal cavity. Power and gain
settings are modified to accurately assess the echogenicity of
free fluid and any particulate debris, and to optimize visualiza-
tion of masses or nodules. Higher frequency transducers can
be used to more accurately characterize superficial structures
and to assess the bowel. Color and spectral Doppler as well as
CEUS are used to assess the vascularity of any lesions.
Normal Development and Anatomy
Normal Development
The primitive foregut separates the upper portion of the
embryonic coelomic cavity into left and right cavities via the
dorsal and ventral mesenteries. The foregut then rotates 90
degrees to the right which situates the ventral mesentery to
the right of the stomach and the dorsal mesentery to the left.
The midgut is suspended by the dorsal mesentery and
early in development outgrows the body cavity, herniating
through the umbilicus before returning to the body at the
eighth to tenth weeks of life [161]. A counterclockwise
rotation of 270 degrees occurs which situates the duodeno-
jejunal flexure in the left upper quadrant and the cecum in
the right lower quadrant of the abdomen (Fig. 13.48). The
greater omentum develops from the dorsal mesentery, and
the lesser omentum develops from the ventral mesentery.
Normal Anatomy
The peritoneum is a continuous serous membrane consisting
of a single layer of mesothelium supported by a thin layer of
connective tissue. The peritoneum lines the abdominal and
13 Spleen and Peritoneal Cavity 525

Ventral Ventral

Liver
L Liver

Right S
Stomach Stomach

Left
Spleen Spleen

Right kidney Right kidney

L
Left kidney Left kidney
Dorsal Dorsal

a 5-week-old fetus b 10-week-old fetus

Ventral
Stomach Falciform ligament

Gastrohepatic ligament
Liver
Gastrosplenic ligament
Pancreas

Gastropancreatic ligament

Hepatoduodenal Spleen
ligament
Splenorenal ligament
Right kidney
Left kidney
Dorsal

c Adult

Fig. 13.48 Diagrams of the peritoneal cavity in the axial plane of (a) a 5-week-old fetus, (b) a 10-week-old fetus, and (c) an adult

pelvic cavities (the parietal peritoneum) and surrounds the
abdominal organs, including the gastrointestinal tract (the
visceral peritoneum).
The peritoneum supports the viscera as well as providing
pathways for their blood vessels and lymphatics [160]. It
contains numerous folds, including the mesenteries, omenta,
and ligaments, which serve to suspend the viscera within the
peritoneal cavity, to provide protection and prevent friction
with movement.
The peritoneum stores fat, secretes fluid in response to
injury or infection, and walls off infection. The parietal peri-
toneum is supplied by nerves to the adjacent body wall and
is generally very sensitive to noxious stimuli, whereas the
visceral peritoneum is insensitive. The parietal peritoneum
receives its blood supply from somatic vessels of the abdom-
inal and pelvic walls. Parietal peritoneal lymphatics join
those of the body wall and drain to parietal lymph nodes. The
visceral peritoneum obtains its blood supply from the vis-
cera, and its lymphatics join those of the visceral vessels and
drain to the regional lymph nodes.
The peritoneal cavity is divided into two main compart-
ments: the greater sac and the lesser sac. The greater sac is
the main cavity of the abdomen and extends from the dia-
phragm to the pelvis. The lesser sac, also known as the
omental bursa, is a potential space that lies posterior to
greater and lesser omenta, between the posterior wall of the
stomach and the anterior peritoneal surface of the left kidney.
It communicates with the greater sac through the epiploic
foramen (Fig. 13.49).
The peritoneal cavity is subdivided into a number of com-
municating compartments by the transverse mesocolon and
small bowel mesentery [162].
The supramesocolic space lies above the root of the trans-
verse mesocolon, while the inframesocolic space lies below
the root of the transverse mesocolon.
526
Arrow in
epiploic foramen
Stomach
Transverse colon
Greater omentum
Small intestine
Fig. 13.49 Diagram of the peritoneal cavity in the sagittal plane showing the relationship of the greater sac (dark red) to the lesser sac (light blue).
The greater and lesser sacs communicate through the epiploic foramen (arrow)
The supramesocolic compartment is further divided into
right and left peritoneal spaces. The right supramesocolic
space has three subspaces: the right subphrenic space, the
right subhepatic space (further divided into anterior and pos-
terior right subhepatic spaces), and the lesser sac. The poste-
rior right subhepatic space is commonly known as Morison’s
pouch.
The left supramesocolic space has two subspaces: the left
perihepatic space (further divided into anterior and posterior
left perihepatic spaces) and the left subphrenic space (further
divided into anterior and posterior left subphrenic [peri-
splenic] spaces).
The inframesocolic space is divided by the small bowel
mesentery into right and left infracolic spaces (Fig. 13.50).
These communicating spaces are not usually visualized on
imaging studies unless they are distended with fluid.
Omentum
The greater omentum is formed from four layers of perito-
neum: two from the stomach and two from the transverse
colon. It extends inferiorly from the greater curvature of the
P. Duffy et al.
Superior layer of
coronary ligament
Bare area of liver
Inferior layer of
the coronary ligament
Pancreas
Duodenum
Aorta
Mesentery
stomach and the proximal duodenum before folding back
on itself and ascending to the transverse colon. The lesser
­omentum joins the lesser curvature of the stomach, proxi-
mal duodenum, and liver. The greater and lesser omenta are
relatively smaller in children compared with adults as their
fat content increases with age.
The normal omenta can be difficult to visualize by ultra-
sound. In the presence of pathology such as large volume
ascites or omental metastatic disease, their relatively super-
ficial position may allow for visualization with a high-fre-
quency linear transducer (Fig. 13.51).
Mesentery
Mesenteries are specially adapted peritoneal folds that sup-
port the abdominal contents and facilitate bowel peristalsis.
Mesenteries contain blood vessels and lymphatics. In the
presence of ascites, the mesenteries can be visualized as freely
floating leaves within the fluid (Figs. 13.52 and 13.53). The
small bowel mesentery extends obliquely from the second
lumbar vertebra to the right iliac fossa, connecting the jeju-
num and ileum to the posterior abdominal wall.
13 Spleen and Peritoneal Cavity 527

Faciform ligament Left diaphragm

Left triangular ligament

Right diaphragm

Hepatoduodenal
Gastrohepatic ligament
ligament

Left subphrenic space

Right suphrenic space Gastrophrenic ligament

Right triangular ligament Stomach

Right subhepatic space Spleen

Gastrosplenic ligament
Transverse mesocolon
Phrenicosplenic ligament
Right paracolic gutter
Kidney

Right infracolic space Splenorenal ligament

Small bowel mesentery

Phrenicocolic ligament
Ileocecal valve

Ligament of Treitz Gastrocolic ligament

Left infracolic space

Sigmoid mesocolon Inframesocolic space

D Left paracolic gutter

Supramesocolic space

Fig. 13.50 Diagram of the peritoneal cavity in the coronal plane demonstrating the compartments formed by the transverse mesocolon and small
bowel mesentery

Fig. 13.52 Normal mesentery in a 1-day-old male with ascites related

to congenital heart disease. Transverse grayscale ultrasound image of
Fig. 13.51 Tumor infiltration of the greater omentum (omental “cake”)
the abdomen shows normal leaves (arrowheads) of the small bowel
in an 18-year-old female with hepatoblastoma widely metastatic to the
mesentery. B, Small bowel loop; Asterisks, ascites
peritoneal cavity. Transverse grayscale ultrasound image of the mid-­
abdomen reveals thickening and heterogeneity of the greater omentum
(arrows) that abuts the anterior abdominal wall (arrowheads). Additional
metastatic intraperitoneal masses (asterisks) are depicted
528 P. Duffy et al.

Peritoneal Fluid
a
The visceral and parietal layers of the peritoneum are nor-
mally separated by a small volume of physiologic fluid, on
the order of 50–100 ml. Physiologic free fluid is anechoic on
ultrasound, and is commonly identified in the dependent por-
tions of the peritoneal cavity, including the rectouterine
space (pouch of Douglas) in girls or rectovesical space in
boys, as well as in the posterior right subhepatic space
(Morison’s pouch) and between bowel loops [162].

Normal Flow of Peritoneal Fluid

Fluid within the peritoneal cavity circulates under the influ-
ence of negative pressure generated by the diaphragm, bowel
peristalsis, and gravity. It flows toward the subdiaphragmatic
spaces where it is drained by the subphrenic submesothelial
lymphatics. Fluid travels along pathways determined by the
position of the mesentery and peritoneal reflections. This
natural flow pattern also determines the route of spread of
intraperitoneal disease processes.
Peritoneal fluid initially accumulates in gravity-depen-
dent spaces, including the deep pelvic recesses and lateral
paravesical spaces. It then ascends through the paracolic gut-
b
ters to the subdiaphragmatic spaces.
Most of the fluid flows along the right paracolic gutter
since the left paracolic gutter is shallow and discontinuous
with the left subdiaphragmatic space at the level of the
phren­icocolic ligament. The circulatory pathway is completed
by redirection of fluid into the pelvis via the inframesocolic com-
partment (Fig. 13.54).
Abscesses and metastases will preferentially develop in
zones of fluid stasis, including the pelvic peritoneal recesses
(pouch of Douglas and rectovesical space), the right lower
abdominal quadrant near the distal end of the small bowel
mesentery at the ileocecal junction, the right paracolic gut-
ter, and superior aspect of the sigmoid mesocolon.

Ascites

Ascites is an abnormal accumulation of fluid within the peri-

Fig. 13.53 Thickened mesentery in an 18-year-old female with intes- toneal cavity and can result from increased production or
tinal lymphangiectasia. (a) Transverse grayscale ultrasound image of
impaired drainage of peritoneal fluid. Ascites may contain a
the mid-abdomen shows thickening (arrowheads) of the leaves of the
mesentery and a mildly thickened small bowel loop (arrow). Asterisks, transudate (fluid with a low-protein content and low specific
Ascites. (b) Axial contrast-enhanced CT image of the abdomen demon- gravity) or an exudate (fluid with a high-protein content and
strates a thickened, edematous mesentery (white arrowheads); multiple high specific gravity).
mildly thick-walled small bowel loops (black arrowheads); and ascites
(asterisks)
13 Spleen and Peritoneal Cavity
There are many causes of ascites in children that vary accord-
ing to age and geographical location. Neonatal ascites is usually
biliary, urinary, or chylous in origin. Beyond the neonatal period,
infections such as tuberculosis are more common in developing
regions, while hepatobiliary disease, cardiac abnormalities, and
neoplasia are the usual causes in more developed locales.
Lesser
sac
Bare area of liver
Fig. 13.54 Diagram showing the normal circulation pathways of
intraperitoneal fluid, indicated by arrows
Fig. 13.55 Simple ascites in a 16-year-old female with cystic fibrosis and hepatic cirrhosis. Longitudinal (a) and transverse (b) grayscale ultra-
sound images of the pelvis demonstrate a large amount of anechoic free fluid surrounding the uterus (U). B, Bladder; Asterisks, ovaries
529
Ascites can also be categorized according to its serum-asci-
tes-albumin gradient (SAAG) (Table 13.2) [163]. Ascites
related to liver failure, heart failure, and associated pathologies
will tend to have a high (>1.1 g/dl) SAAG, while ascites due to
malignant infiltration, pancreatitis, nephropathies, and protein-­
losing enteropathies will tend to have a low (<1.1 g/dl) SAAG.
On ultrasound evaluation, simple ascites will appear anechoic
(Fig. 13.55). Hemorrhagic, inflammatory, and neoplastic causes
of ascites may contain debris or septations. Small amounts of
ascites are best appreciated in the dependent portions of the
peritoneal cavity. As the volume of ascites increases, it will be
evident throughout the abdomen (Fig. 13.56).
Changes in patient position can help determine whether
ascites is free flowing or loculated. Free fluid generally con-
forms to the shape of the adjacent structures and often dem-
onstrates acute angles where it contacts the v­iscera. In
contrast, loculated fluid has rounded contours and exerts
mass effect on adjacent structures (Fig. 13.57). Diagnostic
fluid sampling is usually performed under ultrasound guid-
Table 13.2 Classification of ascites according to the serum-ascites-­
albumin gradient (SAAG)
SAAG > 1.1 mg/dl SAAG < 1.1 mg/dl
Cardiac failure Nephrotic syndrome
Cirrhosis Tuberculous peritonitis
Portal vein thrombosis Pancreatitis
Budd-Chiari syndrome Bowel obstruction/infarction/perforation
Veno-occlusive disease Peritoneal carcinomatosis
Pseudomyxoma peritonei
530 P. Duffy et al.

K
L

Fig. 13.56 Simple ascites in a 1-day-old male with congenital heart

disease. Longitudinal grayscale ultrasound image shows a moderate
amount of anechoic free fluid surrounding the right lobe of the liver (L). b
K, Right kidney

ance [164]. Occasionally air-filled bowel can float above the

ascitic fluid and obscure the imaging window.
Treatment with drainage catheters can be used to provide
relief from symptomatic ascites. A simple pigtail catheter is
frequently effective, although some patients will suffer from
recurrent fluid accumulation (as in patients with peritoneal
carcinomatosis) and tunneled drainage catheters will provide
better long-term results [165]. Specific causes of ascites and
management approaches are further discussed below.

Hemoperitoneum
The most common cause of hemoperitoneum is trauma,
including both accidental and nonaccidental forms. Other
etiologies include bleeding disorders, hemorrhagic pancre-
atitis, ectopic pregnancy, and neoplasm rupture. The appear-
ance of hemoperitoneum will vary on ultrasound, depending
on the volume and the acuity of the hemorrhage. It may
appear anechoic or more complex. Complex fluid may
appear particulate, layered, or contain septations [166].
As with other forms of ascites, blood will collect preferen-
tially in the dependent portions of the peritoneum and layering
Fig. 13.57 Loculated ascites in a 2-year-old male with veno-occlusive
disease after stem-cell transplantation for treatment of neuroblastoma.
Transverse (a) and longitudinal (b) grayscale images demonstrate an
anechoic fluid collection (asterisks) in the left subhepatic space
(arrows). Note the rounded inferior contour of the collection (arrows)
that did not alter with changes in patient position
of the components may occur. Blood clots may also be visual-
ized (Fig. 13.58). Treatment of hemoperitoneum depends on
the underlying disorder.
13 Spleen and Peritoneal Cavity 531

Chylous Ascites
a Leakage of lymphatic fluid into the peritoneal cavity results in
chylous ascites. Any disruption to the normal drainage of lym-
phatic fluid can lead to chylous ascites although congenital
S abnormalities of the lymphatic system and trauma are the most
common underlying disorders in children [167]. The diagnosis is
K usually confirmed by paracentesis, which shows markedly ele-
vated triglyceride levels with a predominance of lymphocytes.
The appearance of chylous ascites on ultrasound evalua-
tion is variable: it can be purely anechoic, or more complex
with fat/fluid levels (Fig. 13.59) [168, 169]. Further imaging

L
c

Fig. 13.58 Hemoperitoneum in a 14-day-old male receiving antico-

agulation while on extracorporeal membrane oxygenation (ECMO).
Longitudinal (a) and transverse (b) grayscale ultrasound images of the
left flank demonstrate a large amount of complex fluid containing echo-
genic strands (arrowheads) and clumps of debris (arrows) in keeping
with blood products. K, Left kidney; S, splenic tip. (c) Transverse gray- Fig. 13.59 Chylous ascites in a 5-year-old male after surgical resection
scale ultrasound image of the bladder reveals a large intraluminal blood of neuroblastoma. (a) Transverse grayscale ultrasound image of the right
clot (asterisk) surrounding a Foley catheter that displays posterior upper quadrant of the abdomen reveals particulate free fluid (asterisks)
acoustic shadowing (arrowhead). The catheter balloon (arrow) is filled overlying the liver (L). (b) Longitudinal grayscale ultrasound image of
with echogenic clot the right pelvis demonstrates free fluid with a fat-fluid level (arrows)
532
of the lymphatic system may be warranted to assess the site
of leakage, including lymphoscintigraphy, MR lymphangi-
ography, and/or conventional lymphangiography.
Treatment of chylous ascites with bowel rest and diet
modification is effective in some patients. Published case
reports have shown that somatostatin and octreotide either
alone or in combination with total parenteral nutrition (TPN)
can be effective in the management of chylous ascites due to
a variety of causes [167]. If there is no clinical improvement
after 4–8 weeks of conservative management, surgical inter-
vention may be necessary.
Urine Ascites
Urine ascites can develop as a result of urinary tract obstruc-
tion or trauma. The fluid is usually retroperitoneal in loca-
tion, although it can accumulate within the peritoneal cavity
if the usual boundaries separating the retroperitoneum from
the peritoneal cavity have been disrupted. Posterior urethral
valves account for approximately 70% of cases of urine asci-
tes in neonates (Fig. 13.60) [170]. Bladder rupture can result
in intra- or extraperitoneal urine leak. Extraperitoneal leak is
a b
K
Fig. 13.60 Urine ascites in a 1-day-old male with posterior urethral
valves. (a) Longitudinal grayscale ultrasound image of the left kidney
(K) demonstrates a crescentic subcapsular fluid collection (asterisk)
related to forniceal rupture and adjacent ascites (arrowhead). The left
kidney has abnormally increased parenchymal echogenicity, and there
is moderate hydronephrosis. S, Splenic tip. (b) Transverse grayscale
ultrasound image of the pelvis reveals a thick-walled bladder (B) con-
P. Duffy et al.
much more common, with intraperitoneal leakage usually
occurring in the setting of a full bladder.
On ultrasound, a urinoma will often have the appearance of
simple ascites. In the setting of trauma, a urinoma and hemo-
peritoneum may coexist.
Management of urine ascites depends on the underly-
ing cause of urinoma. Percutaneous nephrostomy and/or
nephroureteral stenting may be required as well as surgical
intervention.
Localized Peritoneal Fluid Collections
 erebrospinal Fluid Pseudocyst
C
Free intra-abdominal cerebrospinal fluid (CSF) is an expected
finding in patients with a ventriculoperitoneal shunt, with an
ultrasound appearance identical to that of simple ascites.
Loculated collections of CSF can develop when adhesions
form around the shunt which may impair drainage and lead
to increased intracranial pressure [171]. These collections
can become very large and have significant mass effect.
c
B
U
taining a catheter (arrowhead). There is a small-to-moderate amount of
pelvic free fluid (arrow) with a fluid-fluid level. (c) Lateral oblique fluo-
roscopic image of the pelvis obtained during voiding cystourethrogra-
phy shows posterior urethral valves (arrow) with marked focal
narrowing of the posterior urethra and significant proximal urethral
dilation (U). Bilateral vesicoureteral reflux (arrowheads) is also
identified
13 Spleen and Peritoneal Cavity
a b
Fig. 13.61 Cerebrospinal fluid pseudocyst in a 17-year-old female with
fever and abdominal pain. (a) Longitudinal grayscale ultrasound image
demonstrates a large multilocular abdominal and pelvic cyst surrounding
the ventriculoperitoneal shunt catheter (arrow). B, Bladder. (b) Axial
On ultrasound, a CSF pseudocyst appears as a well-­
circumscribed fluid collection that may be unilocular or mul-
tilocular. The shunt tubing is usually identified within the
fluid collection or adjacent to it and appears as a linear echo-
genic structure (Fig. 13.61).
Management of a CSF pseudocyst usually requires shunt
revision and occasionally percutaneous drainage of the pseu-
docyst [172].
Biloma
Most bilomas are iatrogenic in etiology and can develop
after liver transplantation, biliary tract surgery, and partial
hepatic resection for liver tumor. Other associations include
various types of liver lesion ablation and trauma. Bile collec-
533
contrast-enhanced CT image shows the fluid-­attenuation pseudocyst (aster-
isk) with a faintly enhancing rim and contained shunt catheter (arrow-
heads). Note that the loculations seen with ultrasound are not evident by
CT
tions tend to become walled-off relatively quickly due to the
resulting inflammatory response and are commonly seen in
the right upper quadrant of the abdomen. They may become
infected and lead to peritonitis. A biliopleural fistula is a rare
complication where there is communication between the
biliary system and the pleural cavity that can result in a bile
empyema.
Ultrasound imaging of biloma usually reveals a round or
oval anechoic fluid collection (Fig. 13.62) [173].
If a biloma is small and uncomplicated, it may resolve
spontaneously. In more complex cases, treatment can range
from endoscopic retrograde cholangiopancreatography (ERCP)
with sphincterotomy or stent placement to surgical hepatico-
jejunostomy [174].
534
Fig. 13.62 Biloma in a 2-year-old male with recent left lateral segment
liver transplant. Transverse (a) grayscale and (b) color Doppler ultrasound
images demonstrate a small anechoic, avascular fluid collection (arrows)
along the posterior aspect of the liver (L). (c) Axial contrast-enhanced CT
Pancreatic Pseudocyst
A pancreatic pseudocyst is a complication of acute and
chronic pancreatitis with the wall consisting of fibrous and
granulation tissue. While the vast majority of pancreatic
pseudocysts are retroperitoneal in location, they may very
rarely rupture into the peritoneal cavity [175]. Rupture of a
P. Duffy et al.
image shows a gas-containing fluid collection (arrow) posterior to the liver
(L). (d) Hepatobiliary scintigraphy reveals activity (arrow) along the pos-
terosuperior aspect of the liver corresponding to the biloma
pancreatic pseudocyst into the peritoneal cavity results in
features of peritonitis and occasionally hemorrhagic shock.
Imaging features include a decrease in size of the pseudocyst
and rapid development of ascites [176, 177]. Emergent surgi-
cal exploration is usually required with abdominal lavage
and external drainage.
13 Spleen and Peritoneal Cavity
 eritoneal Inclusion Cyst
P of the diaphragm [161, 180]. It is usually located in the right
A peritoneal inclusion cyst is cystic mass that results from a
nonneoplastic, reactive mesothelial proliferation that occurs
almost exclusively in premenopausal females with active
ovaries and pelvic adhesions secondary to pelvic surgery,
pelvic trauma, endometriosis, or pelvic inflammatory disease
that results in impaired absorption of secreted ovarian fluid
[178, 179]. Histologically, the cysts are lined by flattened or
cuboidal mesothelial cells. Patients usually present with
intermittent or chronic lower abdominal or pelvic pain.
On ultrasound, a peritoneal inclusion cyst can have a vari-
ety of appearances that range from a unilocular cyst that can
simulate a para-ovarian cyst to a complex, multilocular cys-
tic lesion that can be mistaken for a malignant ovarian neo-
plasm (Fig. 13.63). The lesion can sometimes completely
surround the ovary so that it appears entrapped within the
cyst. The cyst fluid may contain debris or hemorrhage.
Nodular excrescences or septations may be present that sim-
ulate an ovarian neoplasm. Vascular flow can be detected
within the septations with Doppler imaging.
When the cyst is large, cross-sectional evaluation with CT or
MR imaging is useful for determining the anatomic relationship
of the mass to adjacent pelvic and abdominal structures [179].
Treatment options include the use of oral contraceptives
to decrease the amount of fluid produced by the ovary to sur-
gical adhesiolysis, laparoscopic and percutaneous drainage,
and open resection [178].
 iaphragmatic Mesothelial Cyst
D patients who are immunocompromised or receiving continu-
Diaphragmatic mesothelial cyst is a congenital cystic lesion
that arises from coelomic remnants on the peritoneal surface
a b
U B
Fig. 13.63 Peritoneal inclusion cyst in a 17-year-old female with ulcer-
ative colitis and a history of many abdominal operations. Transverse
color Doppler (a) and longitudinal grayscale (b) ultrasound images of
the pelvis show a cystic, avascular mass (arrows) in the left adnexa with
535
posterolateral costophrenic angle between the thoracic wall
and the liver and is most often an incidental finding.
On ultrasound evaluation, it manifests as a simple, thin-­
walled cyst of variable size (Fig. 13.64). It may be bilobed
and contain one or more thin septations.
Symptomatic lesions can be treated with percutaneous
aspiration or sclerotherapy.
Peritoneal Inflammation
Peritonitis is an inflammation of the parietal and visceral
peritoneum that can be infectious or noninfectious in
­etiology. Infectious causes include bacteria, viruses, fungi,
and parasites. Noninfectious causes are less frequent and
include chemical peritonitis, granulomatous peritonitis, and
sclerosing peritonitis.
Infective Peritonitis
Most patients develop infective peritonitis as a complication
of disease processes affecting the abdominal organs.
Frequent causes include perforated appendicitis, bowel
necrosis secondary to ischemia, inflammatory bowel disease,
and postoperative leaks. Bacterial cultures of the exudate
usually show mixed flora with a preponderance of anaerobes
and gram-negative bacilli. Peritonitis caused by viruses,
fungi, and parasites is unusual and is seen most often in
ous ambulatory peritoneal dialysis. Spontaneous or primary
bacterial peritonitis in children is much rarer, usually occur-
c
a slightly irregular contour that was separate from the left ovary (not
shown). U, Uterus. (c) Sagittal contrast-enhanced CT image reveals the
cyst (asterisk) posterior to and indenting the bladder (B)
536
a b
c d
Fig. 13.64 Diaphragmatic mesothelial cyst complicated by bleeding
in a 14-year-old male with idiopathic thrombocytopenic purpura.
Longitudinal (a) and posterior transverse (b) grayscale ultrasound
images show a well-circumscribed, heterogeneous mass with mildly
lobulated contours located between the diaphragm (arrow) and the liver
ring in the s­ etting of nephrotic syndrome, hepatic cirrhosis,
or immunodeficiency [181].
The ultrasound features of peritonitis are variable, includ-
ing ascites with echogenic debris, septations, or gas [182,
183]. There can be heterogeneous exudate between loops of
bowel, and thickening of the parietal and visceral perito-
neum, mesentery, and omentum (Fig. 13.65).
Treatment of infective peritonitis includes general sup-
portive measures such as IV rehydration and correction of
electrolyte disturbances. Antibiotics are also routinely
administered. Surgical exploration may be required with
peritoneal lavage to limit the anatomical damage caused by
the peritonitis.
Tuberculous Peritonitis
Tuberculosis (TB) is caused by the bacterium Mycobacterium
tuberculosis, and it is a highly prevalent condition worldwide,
affecting close to ten million patients per year and resulting in
approximately 1.5 million deaths [184]. It is most commonly
P. Duffy et al.
(L). (c) Longitudinal color Doppler ultrasound image of the lesion
reveals no internal flow. (d) Axial contrast-enhanced CT image reveals
a fluid-filled cyst (asterisk). Note that the heterogeneity of the cyst con-
tents seen with ultrasound is not identified by CT
seen in the developing world, as well as in immigrant popula-
tions and in patients with AIDS in developed countries.
Peritoneal TB usually develops via hematogenous spread
after reactivation of latent TB involving primary lung foci,
although it can also occur during active pulmonary TB infec-
tion. It can also develop following rupture of mesenteric
lymph nodes, gastrointestinal dissemination, or gynecologic
involvement. Symptoms of tuberculous peritonitis include
fever, night sweats, anorexia, weight loss, abdominal pain,
and abdominal distention from ascites.
Ultrasound features include lymphadenopathy, high-­density
ascites with or without septations, peritoneal abscesses, omen-
tal and/or peritoneal thickening, and bowel wall thickening.
Imaging can also depict ancillary findings such as hepatomeg-
aly and parenchymal abscesses.
The diagnosis of tuberculous peritonitis can be confirmed
with ascitic fluid analysis or laparoscopy with peritoneal
biopsy. Treatment consists of 6–9 months of antibiotics with
follow-up to confirm resolution of disease.
13 Spleen and Peritoneal Cavity
a b
Fig. 13.65 Spontaneous bacterial peritonitis in a 20-year-old female
with a history of heart transplantation and immunosuppression. Lon­
gitudinal (a) and (b) transverse grayscale ultrasound images of the pel-
vis reveal complex ascites with multiple septations. Incidental note is
made of a unilocular right ovarian cyst (asterisk). B, Bladder; O, ovary.
Chemical Peritonitis
Chemical peritonitis is a noninfectious peritonitis incited by
the contact of various substances with the peritoneum, includ-
ing bile, pancreatic, or gastric secretions. Barium can also cause a
severe chemical peritonitis and should be avoided in patients
in whom a bowel perforation is suspected.
Meconium peritonitis is an aseptic chemical inflamma-
tion caused by intrauterine bowel perforation [185, 186]. The
meconium is sterile and incites an inflammatory reaction that
can subsequently calcify. Underlying causes of meconium
peritonitis include intestinal atresia, volvulus, intussuscep-
tion, and meconium ileus.
In most patients, the etiology of the perforation cannot be
identified. A deficient vascular ­supply and bowel atresia have
been postulated as potential causes. Certain viruses (cyto-
megalovirus, rubella, parvovirus B19) have also been linked
to meconium peritonitis. The incidence of cystic fibrosis in
infants with meconium peritonitis has been reported to vary
between 15% and 40% [186].
Ultrasound features of meconium peritonitis include dif-
fuse peritoneal calcification, meconium pseudocyst, signs of
bowel obstruction, ascites, pneumoperitoneum, and volvulus
(Fig. 13.66).
Treatment of chemical peritonitis depends on the under-
lying disorder. Leakage of sterile fluids such as bile or pan-
creatic sections can rapidly become infected, so that
antibiotics are usually administered. The need for surgery
in patients with meconium peritonitis is based on clinical
symptoms and the child’s overall condition. Asymptomatic
infants, even those with evidence of peritoneal calcification
and fluid collections, can often be managed nonoperatively.
A recent study showed that the need for surgery could be
predicted from a combination of radiographic imaging
537
c
(c) Longitudinal grayscale ultrasound image of the left upper quadrant
demonstrates ascites (asterisks). There is irregular hypoechoic material
(arrowheads) along the serosal surfaces of several bowel loops in keep-
ing with inflammatory exudate
findings, including bowel obstruction, ascites, pneumoperi-
toneum, and volvulus [186].
Granulomatous Peritonitis
Nontuberculous granulomatous peritonitis can be caused by
a variety of infectious, malignant, and idiopathic inflamma-
tory conditions, including disseminated infection with histo-
plasmosis, Crohn disease, granulomatosis with polyangiitis,
and lymphoproliferative disorders. Talc and cornstarch from
surgical glove powder have also been implicated [187, 188].
The ultrasound imaging features of granulomatous peritoni-
tis are relatively nonspecific, and may include ascites, debris,
and septations. Inflammation due to histoplasmosis may dem-
onstrate nodular thickening of the peritoneal surfaces.
Treatment will depend on the underlying disorders.
Patients with noninfectious granulomatous peritonitis may
be given corticosteroids and nonsteroidal anti-inflammatory
medication such as indomethacin in an attempt to limit the
formation of adhesions.
 clerosing Encapsulating Peritonitis
S
Sclerosing encapsulating peritonitis (also known as “abdomi-
nal cocoon” and “encapsulating peritoneal sclerosis”) is char-
acterized by the formation of a connective tissue membrane
that overlies the peritoneum with partial or complete encase-
ment of the small bowel that leads to recurrent episodes of
small bowel obstruction. It can be idiopathic or occur in asso-
ciation with continuous ambulatory peritoneal dialysis, TB,
ventriculoperitoneal, or peritoneovenous shunts. Patients can
present at any age with abdominal pain, vomiting, and/or
bowel obstruction.
Ultrasound features include increased peristalsis in
multiple bowel loops and ascites that can be simple or loc-
538
a b
d
L S
Fig. 13.66 Meconium peritonitis in a premature 2-day-old male with
terminal ileal perforation. (a) Supine frontal radiograph of the chest and
abdomen demonstrates a large ovoid lucency (black arrowheads) in the
upper mid-abdomen that compresses the lesser curvature of the stom-
ach and adjacent bowel loops. Several punctate calcifications (white
arrowhead) overlie the lateral aspect of the upper right abdomen.
Coronal (b) and transverse (c) grayscale ultrasound images show a
rounded, echogenic collection (arrows) in the mid-abdomen consisting
of matted bowel loops and surrounding echogenic material correspond-
ulated. Over time, the fluid tends to become more complex
with prominent stranding (Fig. 13.67). The involved bowel
loops appear matted together and are fixed to the posterior
abdominal wall by a characteristic enveloping membrane.
The membrane is depicted as a uniformly echogenic layer
measuring several millimeter in thickness.
Contrast-enhanced CT is considered the reference stan-
dard imaging technique, which will usually show a combina-
P. Duffy et al.
e
T M
ing to the lucent lesion seen on the radiograph. L, Liver; A, aorta;
arrowhead, catheter in the inferior vena cava. (d) Transverse grayscale
ultrasound image of the upper mid-abdomen demonstrates complex
ascites (white arrowheads) containing echogenic debris and septations.
Calcifications (black arrowheads) are present on the surface of the liver
(L). S, Spleen. (e) Longitudinal grayscale ultrasound image of the left
inguinal canal shows a large echogenic collection of meconium (M)
adjacent to the lower pole of the left testis (T)
tion of peritoneal enhancement, thickening, and calcification;
adhesion of bowel loops; signs of bowel obstruction; and/or
fluid loculations/septations [189, 190].
Although the imaging findings are often suggestive of
sclerosing encapsulating peritonitis, exploratory laparotomy
is frequently performed for diagnosis. Management includes
treatment of the underlying condition and/or elimination of
possible inciting agents (e.g., peritoneal dialysis, medica-
13 Spleen and Peritoneal Cavity
tions, and infections) and nutritional support, frequently with
total parenteral nutrition.
Specific treatment depends on the stage of disease. In the
early inflammatory stage, corticosteroids are the treatment of
choice, while in the late fibrotic stage, tamoxifen may be
beneficial. In practice, distinguishing between stages may be
difficult and both treatments may be used.
Surgical intervention consisting of peritonectomy and
enterolysis is reserved for situations in which conservative
medical therapy fails. Mortality in patients with sclerosing
encapsulating peritonitis and a history of peritoneal dialysis
approaches 50% 1 year after diagnosis [191].
Abscess
A peritoneal abscess can develop if treatment of peritonitis is
delayed, or at the site of a localized perforation, frequently
related to appendicitis or Crohn disease in children. Common
sites of abscess formation include the dependent portions of the
abdomen, including the subhepatic and subphrenic spaces, and
the pelvis.
a b
Fig. 13.67 Sclerosing encapsulating peritonitis in a 21-year-old male
with a history of long-standing peritoneal dialysis. (a) Longitudinal
grayscale ultrasound image of the right lower quadrant of the abdomen
shows dilated small bowel loops matted together and encased in a
hypoechoic membrane (arrowheads) along their serosal surfaces. There
is complex ascites (arrow) containing echogenic debris. (b) Transverse
grayscale ultrasound image of the right lower quadrant of the abdomen
Fig. 13.68 Peritoneal abscess in a 6-year-old male with perforated
appendicitis and generalized peritonitis. (a) Longitudinal grayscale
ultrasound image of the right lower quadrant of the abdomen shows a
distended, fluid-filled appendix containing an echogenic appendicolith
(arrowhead) with posterior acoustic shadowing. The appendix is sur-
rounded by thickened, echogenic omental fat (arrows). (b) Longitudinal
539
Findings on ultrasound imaging include a well-defined,
round, or oval fluid collection with an irregular wall that often
contains internal septations and debris (Fig. 13.68). The col-
lection may demonstrate a hypervascular rim with color
Doppler and the adjacent fat may appear more echogenic than
usual due to edema. Gas within the collection will appear as
echogenic foci with posterior reverberation artifact.
Care must be taken to differentiate nonperistaltic bowel
from an abscess which can sometimes be challenging.
Examples of percutaneous drains inadvertently placed within
bowel have been described [192]. Detection of postoperative
intra-abdominal abscesses can be difficult since patients are
relatively immobile and frequently have paralytic ileus with
prominent bowel gas that limits ultrasound assessment. Open
wounds and skin dressings also restrict access of the ultra-
sound transducer. If an adequate ultrasound examination
cannot be obtained, further evaluation with cross-sectional
imaging is recommended, generally with CT although some
authors advocate the use of rapid MR imaging with diffusion-­
weighted sequences [193].
c
shows a thickened, edematous mesentery (M) that is surrounded by a
hypoechoic membrane (arrowheads). (c) Coronal contrast-enhanced
CT image of the abdomen shows loculated ascites (asterisk) with pari-
etal peritoneal enhancement (arrow). The small bowel loops are dilated
and kinked, with serosal thickening and enhancement. There is focal
bowel wall and mesenteric calcification (arrowheads)
grayscale ultrasound image of the right lower quadrant of the abdomen
obtained 4 days after image (a) and laparoscopic appendectomy shows
a complex, bilobed fluid collection. (c) Longitudinal color Doppler
ultrasound image of the collection reveals no internal vascularity. A
small amount of blood flow (arrowheads) is identified at the periphery
of the lesion
540
Treatment of an abscess requires surgery or percutaneous
drainage with adjunctive antibiotic treatment.
Pneumoperitoneum
Pneumoperitoneum is most often the result of bowel perfora-
tion, although other nonsurgical conditions can also be associ-
ated with pneumoperitoneum. Although CT is considered the
reference standard for the detection, localization, and quantifi-
cation of pneumoperitoneum, ultrasound examination is fre-
quently the initial imaging study performed in patients with
abdominal symptoms, so that its recognition is important [194].
Ultrasound imaging for pneumoperitoneum is best per-
formed using a linear array transducer with the patient supine
or in a left posterior oblique position where the air will fre-
quently move into the right upper quadrant (Fig. 13.69).
Pneumoperitoneum is most readily identified immediately
deep to the parietal peritoneum between the liver and anterior
abdominal wall as a highly echogenic line with ring-­down
artifact referred to as the “peritoneal stripe” sign [195, 196].
Pneumoperitoneum may or may not require treatment,
depending on the underlying cause.
a b
Fig. 13.69 Pneumoperitoneum in a 16-year-old female 1 day after chole-
cystectomy. (a) Transverse grayscale ultrasound image of the right upper
abdomen anterior to the liver (L) obtained with the patient supine reveals
a small zone of relatively increased echogenicity of the parietal perito-
neal stripe (arrow) with ring-down artifact from free air. (b) Upright
P. Duffy et al.
Omental Cyst
The term “omental cyst” generally refers to a congenital
lesion of lymphatic origin, now more commonly referred to
as a lymphatic malformation (see discussion of lymphatic
malformation later in this chapter) [197]. Some omental
cysts may be of mesothelial origin [161].
Segmental Omental Infarction
Omental infarction is an uncommon cause of abdominal pain
in children that can present in a fashion similar to acute
appendicitis. Omental infarction does not usually occur before
the age of 4 years, likely related to the relative paucity of intra-
abdominal fat and omental mass in early childhood [198].
Obesity appears to be an important risk factor for the
development of omental infarction, which can occur pri-
marily, as a result of torsion or thrombosis of the vascular
supply of the greater omentum, or secondary to other
abnormalities such as a cyst, hernia, or scar. Patients usu-
ally experience the rapid onset of localized, usually right-
frontal radiograph of the abdomen confirms the presence of free air
(arrow) beneath the right hemidiaphragm. There is a lesser amount of
free air (white arrowhead) beneath the left hemidiaphragm. Right upper
quadrant surgical clips (black arrowhead) are from the cholecystectomy
13 Spleen and Peritoneal Cavity 541

a b

Fig. 13.70 Segmental omental infarction in a 12-year-old female with
a history of prior colectomy for treatment of Crohn disease. (a)
Transverse grayscale ultrasound image of the left upper quadrant of the
abdomen shows an ovoid mass (arrows) immediately deep to the ante-
rior abdominal wall that is hyperechoic relative to the adjacent perito-
neal fat. (b) Axial contrast-enhanced CT image reveals a ring-­like zone
(arrow) of increased attenuation in the anterolateral aspect of the left
abdomen consistent with omental infarction. There is inflammatory
stranding (arrowhead) of the adjacent peritoneal fat

sided abdominal pain that often develops after a heavy

meal or sudden movement.
On ultrasound, segmental infarction usually appears as a
“triangular” or “cake-like” echogenic mass in the anterior
aspect of the peritoneal cavity located immediately deep to the
anterior abdominal wall and anterolateral to the colon that is
hyperechoic to the adjacent peritoneal fat and solid viscera
[199, 200]. The echotexture may be homogeneous or hetero-
geneous with hypoechoic zones corresponding to zones of
hemorrhagic infarction (Fig. 13.70).
When infarction is a result of omental torsion, a hypoechoic,
avascular tubular structure representing the infarcted omentum
can be seen within the hyperechoic mass [199]. Since the main
differential diagnosis is inflamed omental fat resulting from
another intra-abdominal inflammatory processes such as acute
appendicitis, it is important to identify a normal appendix in
patients with ultrasound abnormalities consistent with omental
Fig. 13.71 Mesenteric lymphadenitis in a 4-year-old male with a
infarction. 1-week history of abdominal pain. Longitudinal grayscale ultrasound
The diagnosis of segmental omental infarction is often image of the right lower quadrant of the abdomen demonstrates multi-
only made at the time of surgery. However, when diagnosed ple mesenteric lymph nodes (arrows), several of which are enlarged.
There is a small amount of simple free fluid (asterisk). The appendix
preoperatively, omental infarction is a self-limited condition
was normal (not shown)
that can be managed safely without surgical intervention.
or lymph node inflammation. This diagnosis of exclusion is
frequently made in children undergoing evaluation for acute
Mesenteric Lymphadenitis appendicitis when a normal appendix is visualized and promi-
nent lymph nodes are noted on imaging evaluation (Fig. 13.71).
Enlarged mesenteric lymph nodes are often present in children The diagnosis is also invoked in patients with chronic or recur-
with abdominal pain. In the absence of other disorders, the rent abdominal pain with no evidence of any other abnormal-
enlargement is ascribed to primary mesenteric ­lymphadenitis ity after appropriate clinical and imaging evaluation. A viral
542
etiology for the lymph node enlargement is generally
presumed.
There is some controversy associated with this diagnosis,
particularly since there is no general consensus as to what
cutoff measurements to use for the diagnosis of nodal
enlargement [201, 202]. Furthermore, very few studies have
addressed the clinical characteristics of mesenteric lymph-
adenitis and their comparison with acute appendicitis.
One recent report showed that mesenteric lymphadenitis
and acute appendicitis could be differentiated by multiple
clinical and laboratory parameters, including the incidence
and duration of symptoms prior to presentation, white blood
cell count and differential, and C-reactive protein levels
[203]. Importantly, no significant difference was found
between patients presenting with small (<10 mm in short
axis) or large (>10 mm in short axis) lymph nodes. The
results of this study support the notion that mesenteric lymph
node enlargement is a nonspecific finding and should not be
considered a clinical diagnosis.
Vascular Malformations
Lymphatic Malformation
An LM is a slow-flow congenital vascular anomaly consist-
ing of abnormal lymphatic channels that do not communi-
cate with the normal lymphatic drainage pathways. It can
consist of macrocysts of 1–2 cm in diameter or larger, micro-
cysts, or a combination of both. An LM is present from birth
and grows commensurately with the child. It may cause mass
effect on adjacent structures and can suddenly enlarge after
internal hemorrhage or if it becomes infected.
LMs often arise from the mesentery and omentum and may
remain asymptomatic for long periods. Occasionally, they do not
become apparent until adulthood. Intra-abdominal LM is more
frequently macrocystic than microcystic. Children tend to present
acutely with abdominal pain, distension, nausea, diarrhea, or con-
stipation [204].
On ultrasound evaluation, a macrocystic LM appears as a
fluid collection with septations of varying thickness
(Fig. 13.72). Flow in small septal arteries and veins can be
identified with color and spectral Doppler. Internal cystic
echoes can be seen representing blood, pus, or chyle and
may result in fluid-fluid levels [106]. A microcystic LM
appears solid and echogenic.
CT or MR imaging can be used to delineate the complete
extent of a large lesion or a lesion that involves multiple
intra-abdominal organs and tissues. Compared to MR imag-
ing, CT images are less affected by peristalsis, pulsation, and
motion artifact. Oral and IV contrast administrations help in
P. Duffy et al.
evaluation of intra-abdominal LMs on CT by improving con-
trast resolution and lesion conspicuity (Fig. 13.73). However,
CT entails the use of ionizing radiation, a disadvantage.
Large and especially diffuse infiltrative and microcystic
LMs are generally best delineated with gadolinium-­enhanced
MR imaging because of its superior tissue contrast resolution.
Macrocystic LM is generally hypointense on T1-weighted
images and hyperintense on fluid-­sensitive images, although
the signal intensity can be heterogeneous depending on the
cyst contents. After gadolinium administration, there is fre-
quently faint peripheral and septal enhancement. Microcystic
LM appears hypointense on T1-weighted images and hyper-
intense on fluid-sensitive sequences with minimal or absent
enhancement after IV contrast administration.
Treatment of large or symptomatic intra-abdominal LMs
is indicated. Surgical resection is best avoided as it may
involve significant risk of bowel injury and result in persis-
tent chylous ascites and enlargement of any residual lesion
[204]. Macrocystic lesions can usually be reduced in size
with percutaneous image-guided intralesional sclerotherapy.
Lymphatic cysts are aspirated under ultrasound guidance and
the sclerosant is injected into the cyst, under fluoroscopic
guidance to ensure proper distribution of the agent.
Doxycycline is the most commonly used sclerosing agent.
Microcystic lesions are more usually asymptomatic.
Surgical resection is occasionally necessary for large micro-
cystic lesions causing mass effect, such as urinary tract
obstruction.
Venous Malformation
A VM is a slow-flow congenital vascular anomaly consisting of
abnormal venous channels [106]. As with an LM, a VM is pres-
ent from birth and grows along with the child. Most abdominal
VMs involve the bowel and present with gastrointestinal bleed-
ing. Multifocal lesions can occur, usually in the setting of blue
rubber bleb nevus syndrome. Although this disorder most com-
monly affects the skin and gastrointestinal tract, any part of the
body can be involved, including the peritoneal cavity [112,
204]. Diffuse VM of the abdomen can encompass large seg-
ments of the bowel in addition to the mesentery, retroperito-
neum, and pelvis.
On ultrasound, a VM can manifest as a discrete, rounded
cystic mass; as a network of abnormal tubular channels; or as
a combination of the two. Lesions may contain thrombi or
phleboliths (Fig. 13.74). Evaluation with color Doppler
sometimes demonstrates flow within the vascular channels,
although in many lesions flow will not be detectable because
of the small size and low flow within the vessels. Stagnant
blood within the cystic cavities or channels will frequently
appear as multiple internal fluid-fluid levels.
13 Spleen and Peritoneal Cavity
a b
c d
Fig. 13.72 Intraperitoneal lymphatic malformation in a 2-year-old female.
Longitudinal grayscale ultrasound images of the right upper quadrant of
the abdomen (a) and right flank (b) demonstrate an extensive, predomi-
nantly cystic fluid collection containing multiple septations. L, Liver. (c)
Longitudinal color Doppler ultrasound image of the right lower quadrant
MR imaging will generally be required for determining the
overall extent of peritoneal involvement and will show fluid sig-
nal intensity within the masses and/or tubular channels. Stagnant
blood will frequently appear as multiple fluid-fluid levels within
the lesion. Following gadolinium administration, there will be
complete or near complete enhancement except for regions of
543
of the abdomen shows minimal flow in a more solid, microcystic portion
of the lesion (arrowhead). (d) Longitudinal contrast-enhanced CT image
shows the multiloculated lesion (arrow) extending along the anterior
abdominal wall from the liver (L) to the bladder (B)
thrombosis. Thrombi will be dark on T2-weighted images and
may be bright on T1-weighted images, although phleboliths are
dark on all image sequences due to calcification.
Treatment of widespread VM that involves the perito-
neum generally focuses on eradication of bleeding rather
than on removal of all of the abnormal lesions [204].
544 P. Duffy et al.

a b

c d

V
A

Fig. 13.73 Infected intraperitoneal lymphatic malformation in a septic
9-month-old male. Transverse (a) and longitudinal (b) grayscale ultra-
sound images of the right abdomen reveal a heterogeneous bilobed mass
(arrows) inferior to the liver (L) and right kidney (K) with enhanced
through-transmission. (c) Longitudinal color Doppler ultrasound image
reveals minimal flow (arrowhead) at the periphery of the lesion. V, Inferior
vena cava; A, aorta. (d) Contrast-enhanced ultrasound image shows no
internal flow within the mass which has a thick, enhancing rim (arrow-
heads). (e) Coronal contrast-enhanced CT image shows the rim-enhanc-
ing, bilobed cystic lesion (arrow). Following resection, an infected
lymphatic malformation was diagnosed at pathology
13 Spleen and Peritoneal Cavity
a b
Fig. 13.74 Blue rubber bleb nevus syndrome with mesenteric involve-
ment in a 44-year-­old female. (a) Longitudinal grayscale ultrasound
image of the left flank depicts numerous round and oval submucosal
nodular masses (arrowheads) within bowel loops in keeping with venous
malformations (VMs). (b) Transverse grayscale ultrasound image of the
Benign Masses
A wide variety of benign masses can arise within the perito-
neal cavity as discussed below (Table 13.3).
Infantile Hemangioma
Infantile hemangioma is a benign vascular neoplasm with a
characteristic clinical course marked by initial proliferation
followed by spontaneous involution. It is the most common
tumor of infancy and usually affects the skin, although extra-
cutaneous involvement can also occur. Gastrointestinal
involvement is relatively rare [205, 206]. Tumors arise exclu-
sively in the midgut (jejunum, ileum, and ascending colon)
in the distribution of the superior mesenteric artery, and
involve the bowel wall and mesentery.
545
mid-­abdomen shows several lesions (arrowheads) in the mesentery
between bowel loops (B). (c) Axial IR MR image demonstrates multiple
hyperintense nodular VMs in the body wall (white arrowheads), paraspi-
nal muscles (arrows), and mesentery (black arrowhead)
Patients present with melena and hematochezia in the first
4 months of life, and in some infants, there can be profound
anemia. A significant proportion of patients have skin
involvement.
Ultrasound features include thickened, hypervascular bowel
loops with enlargement of the mesenteric vessels. Contrast-
enhanced CT and MR imaging show diffuse wall thickening
and enhancement of the involved bowel loops [207]. Similar
findings are seen with CEUS (see Chap. 10, Fig. 10.46).
As with other infantile hemangiomas that require sys-
temic treatment, first-line therapy for gastrointestinal hem-
angiomas is propranolol and/or corticosteroid. Treatment
with both agents may be tried in initially unresponsive
patients. Unlike cutaneous lesions, bowel hemangiomas can-
not be directly visualized to assess the efficacy of drug treat-
546 P. Duffy et al.

ment. Once bleeding stops, the duration of therapy is based The echogenicity of adipose tumors is thought to depend on
on clinical judgment without a clear endpoint [205]. their histological heterogeneity. Mesenteric lipomas have a
histological structure slightly less heterogeneous than that of
Lipoma the surrounding mesentery, and they are thought to provide
Lipomas are benign, well-defined, noninvasive, and encap- fewer interfaces which may lead to the hypoechoic appear-
sulated tumors of mature adipose tissue that can arise in any ance on ultrasound. CT and MR imaging can be useful in
part of the body. Although mesenteric, omental and perito- differentiating a lipoma from adjacent structures.
neal lipomas are rare. Most lipomas occur in adults and are Due to the rarity of this lesion, a definitive management
relatively unusual in children. Small lipomas are usually strategy has not been established. Children with mesenteric
asymptomatic. A large lipoma can present as a mass or with and omental lipomas have been successfully managed with
abdominal pain related to compression of adjacent structures both open and laparoscopic surgery [208–210].
or volvulus where the lipoma is the lead point [208–210].
On ultrasound, a mesenteric lipoma appears as a well-­ L  ipomatosis
circumscribed, homogeneous, and hypoechoic oval mass Lipomatosis is a distinct clinicopathologic entity character-
containing multiple fine linear echoes (Fig. 13.75) [208]. ized by the development of unencapsulated lipomas in the
subcutaneous tissues. In this disorder, there are masses that
Table 13.3 Peritoneal cavity neoplasms in children resemble simple lipomas except for their extensive infiltra-
Benign Infantile hemangioma tive distribution. It very rarely occurs in children. Involvement
Lipoma of the face, neck, extremities, trunk, abdomen, and the pelvis
Lipomatosis has been reported [211, 212].
Lipoblastoma Lipomatosis is usually idiopathic, but can occur as a com-
Neurofibroma plication of long-term steroid therapy [213]. Intraperitoneal
Mesenteric adenitis
fat deposition manifests clinically with abdominal disten-
Desmoid tumor
Castleman disease
sion, early satiety, bloating, and constipation simulating irri-
Inflammatory myofibroblastic tumor table bowel syndrome.
Malignant Lymphoma A diffuse accumulation of fat or a fatty mass can be seen
Rhabdomyosarcoma with ultrasound (Fig. 13.76) [211]. However, in most patients,
Small round cell desmoplastic tumor CT or MR imaging is used to delineate the extent of fatty
Malignant mesothelioma infiltration.
Neuroblastoma
Treatment of obstructive symptoms may require surgical
Adenocarcinoma
Wilms’ tumor
resection. In the case of steroid-induced lipomatosis, treat-
Germ cell tumor ment is mainly conservative, including tapering of exoge-
Peritoneal dissemination of intracranial nous steroids and weight loss. With endogenous hormonal
malignancy via ventriculoperitoneal shunt excess, specific therapy is indicated.

a b

Fig. 13.75 Mesenteric lipoma in a 7-year-old female. (a) Longitudinal rior abdominal wall. Numerous fine linear echoes are seen within the
grayscale ultrasound image of the mid-lower abdomen reveals a well- lesion. (b) Coronal contrast-enhanced CT image demonstrates a mid-
circumscribed, hypoechoic mass (arrows) immediately below the ante- line fat density lesion (arrow) superior to the B, Bladder
13 Spleen and Peritoneal Cavity
a b
Fig. 13.76 Peritoneal lipomatosis in a 4-year-old male with CLOVES
syndrome. (a) Transverse grayscale ultrasound image of the left lower
quadrant of the abdomen demonstrates an extensive fibrofatty tissue
mass (arrows) within the peritoneal cavity. (b) Transverse grayscale
Lipoblastoma/Lipoblastomatosis
Lipoblastoma and lipoblastomatosis are benign tumors of
infancy and early childhood that arise from the postnatal pro-
liferation of embryonic white fat [214]. On histopathological
examination, these are cellular neoplasms composed of imma-
ture adipocytes with relatively well-defined septa, frequent
lipoblasts, a fine vascular network, and often have a myxoid
appearance resembling myxoid liposarcoma [215].
Lipoblastoma is a well-circumscribed, encapsulated tumor,
whereas lipoblastomatosis is a diffuse, infiltrative lesion. Intra-
abdominal lipoblastoma is infrequent and usually retroperitoneal
in location. Intraperitoneal lipoblastoma is exceptionally rare.
The clinical and imaging features of lipoblastoma are simi-
lar to those of lipoma so that a preoperative differential diag-
nosis is not usually possible. Abdominal tumors tend to present
as very large masses associated with nonspecific gastrointesti-
nal symptoms related to compression of adjacent structures,
although occasionally they can manifest with acute symptoms
related to volvulus or intestinal obstruction.
On ultrasound, both lipoblastoma and lipoblastomatosis
appear as echogenic fatty masses. A definitive diagnosis can
only be made on histological examination.
The treatment of choice for these tumors is complete surgi-
cal excision to avoid growth of residual tumor and prevent
recurrence, although this can be challenging for lipoblastoma-
tosis [216]. At least 2 years of follow-up is recommended.
Neurofibroma
Neurofibromas of the abdomen and pelvis usually occur in the
setting of neurofibromatosis type I. Solitary gastrointestinal
neurofibromas are unusual sporadic lesions of the stomach and
jejunum, while mesenteric lesions are exceedingly rare [217].
547
c
ultrasound image of the left upper quadrant shows the fibrofatty tissue
(arrows) surrounding the left kidney (K). (c) Coronal T2-weighted, fat-
suppressed MR image reveals extensive fat (asterisks) throughout the
abdomen and pelvis with displacement of the bladder (B) to the left
Plexiform Neurofibroma
Neurofibromatosis type I (NFI), or von Recklinghausen dis-
ease, affects approximately 1 in 3000 people. It is inherited as
an autosomal dominant disorder although 50% of cases occur
as spontaneous mutations [218]. The majority of affected indi-
viduals present in childhood with cafe au lait spots, Lisch nod-
ules, axillary or inguinal freckling, and neurofibromas.
Plexiform neurofibromas are pathognomonic for NFI and
can occur in a variety of locations, including the abdomen and
pelvis. In children with NFI, the nerves of the mesenteric,
subserosal, and myenteric plexuses can give rise to neurofi-
bromas and plexiform neurofibromas of the mesentery and
gastrointestinal tract. Although neurofibromas may appear as
well-defined masses on gross inspection, they frequently
infiltrate into adjacent adipose, muscle, or viscera. Tumors
can encase the mesenteric vessels and involve the bowel wall.
There is also a risk of transformation to a malignant periph-
eral nerve sheath tumor [161]. Symptoms may occur related
to compression and distortion of adjacent structures.
On ultrasound, a mesenteric plexiform neurofibroma appears
as a mass composed of multiple well-defined nodules or as an
infiltrating lesion that extends from the root of the mesentery to
the wall of the intestine. It can be homogeneously or heteroge-
neously hypoechoic in echotexture (Fig. 13.77).
At CT, it is usually homogeneous and low in attenuation.
MR imaging reveals numerous small, round lesions displaying
a “target” sign on T2-weighted images consisting of peripheral
signal hyperintensity and central signal hypointensity [219].
Evidence of malignant transformation includes a rapidly
enlarging lesion, increasing lesion heterogeneity, or increased
fludeoxyglucose (FDG) uptake at positron emission tomog-
raphy (PET).
548 P. Duffy et al.

a c d

B
M

Fig. 13.77 Plexiform neurofibroma of the pelvis in a 20-year-old male
with neurofibromatosis type I. (a) Transverse grayscale ultrasound image
of the pelvis shows a large, hypoechoic mass composed of innumerable
nodules (arrowheads) with hypoechoic rims. (b) Longitudinal grayscale
ultrasound image depicts the pelvic mass (M) displacing the bladder
(B) superiorly into the lower abdomen. (c) Transverse color and spectral
Doppler ultrasound image reveals a small amount of high-resistance
intra-tumoral arterial blood flow. (d) Sagittal T2-weighted, fat-suppressed
MR image demonstrates an extensive mass with nodules displaying the
“target” sign of a hyperintense rim and hypointense center. The mass
displaces the bladder (arrowhead) superiorly, as well as surrounding and
compressing the rectum (arrow)

A symptomatic mesenteric plexiform neurofibroma

should be surgically resected if feasible, provided it does not
encase vital structures or reveal evidence of malignant
transformation.

Desmoid Tumor
Desmoid tumor, also known as aggressive fibromatosis or
mesenteric fibromatosis, is a rare tumor that can arise in a
variety of locations in the body, including the small bowel
and sigmoid mesenteries, the omentum, and peritoneal lig-
aments [220].
Desmoid tumor can be sporadic or associated with familial
adenomatous polyposis (Gardner syndrome), and it is more
common in girls than in boys [161]. Its behavior can vary from
entirely benign to locally aggressive, with morbidity caused Fig. 13.78 Intraperitoneal desmoid tumor in a 9-year-old male with
by encroachment on vital intra-abdominal structures. familial adenomatous polyposis (Gardner syndrome). Transverse gray-
scale ultrasound image of the mid-abdomen demonstrates a well-cir-
Histologically, desmoid tumors are composed of fibro-
cumscribed, heterogeneous mass (arrowheads)
blasts within a dense collagenous stroma. The degree of cel-
lularity and the amount of collagen are variable, and the
stroma may contain areas of myxoid change. erogeneous echotexture. Posterior acoustic enhancement is
On ultrasound, tumors appear as oval, well-circumscribed seen in most lesions, and there is no central necrosis or calci-
solid soft-tissue masses of variable echogenicity that depends fication. Many desmoid tumors demonstrate substantial flow
on the relative proportions of cells, collagen, and myxoid with high arterial resistive indices on color Doppler imaging
matrix (Fig. 13.78). Tumors with dense collagenous stroma and spectral analysis [221].
appear homogeneous, while those with significant myxoid At CT, tumors usually display an attenuation similar to
change display cystic components. Mixed tumors have a het- that of skeletal muscle. Extensive myxoid change is associ-
13 Spleen and Peritoneal Cavity
ated with hypoattenuation, while masses with a dense col-
lagenous stroma appear homogeneous. Tumors of mixed
fibrous and myxoid composition appear heterogeneous.
Tumor margins are usually well defined but may be infiltra-
tive. At MR imaging, desmoid tumors can be hypointense on
both T1-weighted and T2-weighted images related to the
presence of fibrous tissue, although substantial heterogeneity
and T2-weighted signal hyperintensity are observed in some
lesions related to myxoid change.
Treatment depends on the size of the lesion, its rate of
growth, and involvement of structures such as the mesenteric
vessels, and includes observation, surgical resection, chemo-
therapy, radiotherapy, anti-estrogen therapy, and nonsteroi-
dal anti-inflammatory drugs (NSAIDs) [161, 220].
Castleman Disease
Castleman disease comprises a heterogeneous group of disor-
ders defined by shared lymph node histopathological features,
including atrophic or hyperplastic germinal centers, prominent
follicular dendritic cells, hypervascularization, polyclonal lym-
phoproliferation, and/or polytypic plasmacytosis [222].
Each subtype of Castleman disease has varying clinical
features, causes, treatments, and outcomes. Unicentric
Castleman disease involves a single enlarged lymph node or
region of lymph nodes, whereas multicentric Castleman dis-
ease involves multiple regions of enlarged lymph nodes.
Multicentric disease is further divided based on Kaposi sar-
coma-associated herpesvirus/human herpesvirus-­8 (HHV-8)
infection status.
Needle biopsy with histological assessment of an
enlarged lymph node is required for diagnosis, while a
combination of imaging examinations and laboratory tests
is needed to determine the correct subtype and assess the
extent of disease.
a b
M M
Fig. 13.79 Castleman disease with intraperitoneal adenopathy in a
10-year-old male. (a, b) Longitudinal grayscale ultrasound images of the
mid-abdomen show a rounded, hypoechoic soft-tissue mass (M) with
absence of normal internal nodal architecture. Several smaller lymph
549
The unicentric form of Castleman disease is more com-
mon in children, is associated with abdominal pain or mass,
and follows a benign clinical course [220]. Pathologically,
the involved lymph node is enlarged with a mean diameter of
5 cm and preservation of nodal architecture. Central fibrosis,
hemorrhage, or cystic necrosis may be observed in large
tumors. Two main histologic subtypes are recognized: hya-
line vascular and plasma cell. The hyaline vascular type
accounts for up to 90% of cases of unicentric disease. The
plasma cell subtype is more often associated with multicen-
tric disease [220].
Ultrasound imaging of patients with unicentric abdom-
inal and pelvic Castleman disease demonstrates a hetero-
geneously hypoechoic lesion containing thin septa,
usually with posterior acoustic enhancement and marked
vascularity on Doppler evaluation (Fig. 13.79) [223].
On CT, enhancement is homogeneous in smaller masses
but may be heterogeneous in masses larger than 5 cm.
Nonenhancing central areas correlate with fibrosis or cystic
necrosis at pathology. The degree of enhancement is vari-
able, particularly in the plasma cell type, but it is usually
intense in the hyaline vascular type, with prominent periph-
eral vessels. Focal calcifications are seen in up to about
30% of cases.
MR imaging demonstrates hypointense signal compared
with muscle on T1-weighted images and heterogeneously
hyperintense signal on T2-weighted images. Peripheral flow
voids representing feeding vessels may be seen, especially
with the hyaline vascular type. Enhancement after IV gado-
linium administration is usually early and intense, with
delayed washout. In large hyaline vascular-type tumors,
there may be central hypointense zones on T2-weighted
images with radiating bands extending to the periphery that
do not enhance on early-phase dynamic T1-weighted images.
c
nodes (arrowheads) are identified inferior to the mass. (c) Transverse
color Doppler ultrasound image reveals mild hyperemia of the mass as
well as of the adjacent mesentery (arrow)
550
These zones correspond pathologically with lamellated
fibrosis.
FDG PET imaging will show lower standardized uptake
values compared to active lymphoma with resolution of
increased metabolic activity after treatment [220].
The unicentric form of Castleman disease follows a
benign clinical course and is usually cured by excision. The
multicentric form is associated with chronic progression so
that systemic chemotherapy is required. There is also an
increased risk of the subsequent development of B-cell lym-
phoma, Kaposi sarcoma, or follicular dendritic cell sarcoma
so that close follow-up is warranted.
Inflammatory Myofibroblastic Tumor
Inflammatory myofibroblastic tumor (IMT) is a mesenchy-
mal neoplasm of intermediate biological behavior that pri-
marily affects the lung, abdomen, and pelvis. IMT belongs
to the neoplastic subset of the family of inflammatory pseu-
dotumors, an umbrella term for spindle cell proliferations of
uncertain histogenesis with a variable inflammatory compo-
nent. At histology, IMTs have characteristic fasciitis-like,
compact spindle cell and hypocellular fibrous patterns as
well as distinctive molecular features. Patients usually pres-
ent with fever and an abdominal mass.
a c
Fig. 13.80 Intraperitoneal inflammatory myofibroblastic tumor in a
4-day-old male. (a) Transverse grayscale ultrasound image shows a
large, well-­circumscribed mass (arrows) with an echogenic rim and
hypoechoic center that surrounds the gastric antrum and proximal duo-
denum (arrowheads). S, Stomach. (b) Transverse power Doppler ultra-
P. Duffy et al.
The imaging findings of IMT reflect the pathologic fea-
tures and vary from a well-circumscribed, soft-tissue mass to
an ill-defined, infiltrating lesion due to variability in inflam-
matory, stromal, and myofibroblastic components [224].
At ultrasound, it can appear as a well-defined or poorly
circumscribed heterogeneous, solid, or hypo- or hyperechoic
mass (Fig. 13.80) [224, 225]. Shadowing echogenic calcifi-
cation or central hypoechoic regions corresponding to necro-
sis are sometimes seen. Doppler evaluation shows tumor
vascularity with low-resistance arterial waveforms.
CT demonstrates a well-circumscribed, homogeneous or
heterogeneous mass with dense central calcifications in a
small number of cases. Contrast enhancement is variable and
includes early peripheral enhancement of vascular tissue and
delayed central enhancement of fibrotic components. Large
tumors may demonstrate hypoattenuating, nonenhancing
zones of central necrosis. IMT will rarely show aggressive
features such as circumferential growth around vessels and
bowel extension with mural ulceration.
On MR imaging, it is typically hypointense relative to
skeletal muscle on T1-weighted images and hyperintense
on T2-weighted images. Tumors with prominent fibrotic
stroma may be dark on both T1- and T2-weighted images.
Heterogeneous enhancement is seen after gadolinium admin-
d
sound image reveals marked hyperemia of the central, hypoechoic
portion of the mass. (c) Spectral Doppler analysis of the lesion demon-
strates low-­resistance arterial waveforms. (d) Coronal T2-weighted,
fat-suppressed MR image of the mass (arrow) depicts a relatively
hyperintense central portion with a hypointense rim
13 Spleen and Peritoneal Cavity
istration, and there may be delayed enhancement of fibrotic
components. FDG PET/CT reveals nonspecific increased
uptake of radiotracer similar to that seen in other malignan-
cies and is useful in detection of the primary tumor, local
recurrence, and distant metastases [220].
IMT has a propensity for local and regional recurrence
and a small risk of distant metastases. The treatment of choice
is complete surgical resection. When there is extensive local
invasion, positive margins, or incomplete resection, adjuvant
chemotherapy and radiotherapy have been used with variable
results.
There appears to be some correlation between anaplastic
lymphoma kinase (ALK) expression and biologic behavior
of IMT, and early experience with therapy targeted to the
ALK oncogene has been promising. Close clinical follow-up
is necessary since 15–37% of patients with mesenteric
tumors will develop recurrence [220].
Malignant Tumors
Both primary and secondary malignant tumors can develop
within the peritoneal cavity (Table 13.3).
Primary Tumors
Malignant peritoneal tumors are far less frequent in children
as compared to adults, likely related to the relatively low
frequency of gastrointestinal, ovarian, and uterine tumors
which often manifest with peritoneal carcinomatosis. When
peritoneal tumors do develop in children, they usually rep-
resent metastatic disease from spillage at the time of surgery
or from extension of tumor through an organ capsule.
Primary peritoneal neoplasms are exceedingly rare, with
lymphoma and rhabdomyosarcoma the most common enti-
ties [226].
Since malignant cells are distributed along the usual flow
pathways of the peritoneal fluid, the most common locations
of tumor seeding are the rectovesical and rectouterine pouches
as well as the small bowel mesentery, sigmoid mesocolon,
and right paracolic gutter.
Hallmarks of peritoneal tumor include focal hypo- or hyper-
echoic masses on the visceral or parietal surfaces of the perito-
neum; diffuse peritoneal thickening; and/or ascites that can be
complex, with septations and/or debris [159]. Extensive omen-
tal involvement by tumor can result in the appearance of an
“omental cake,” diffuse thickening that can be caused by a vari-
ety of benign and malignant disease processes [161, 220].
Lymphoma
Non-Hodgkin lymphoma (NHL) is the third most frequent
malignancy in children less than 15 years of age and the most
common malignancy to involve the mesentery and omentum.
551
NHL has a male predominance, a predilection for children
less than 10 years of age, and occurs most often in Caucasians.
Sporadic Burkitt lymphoma is the most common NHL sub-
type in children, with widespread disease usually noted at
presentation.
On ultrasound evaluation of Burkitt lymphoma, extra-­
nodal sites of involvement usually appear as large, hypoechoic
masses. Peritoneal, mesenteric, and omental involvement can
manifest as a solitary mass or multiple masses (Fig. 13.81).
Large tumors may have central necrosis. Encasement of
bowel loops and mesenteric vessels is a common feature.
Bowel wall thickening is often identified, and intussuscep-
tion is a frequent complication. Peritoneal seeding appears as
diffuse peritoneal thickening or nodularity along the liver
capsule and peritoneal reflections. Malignant ascites occurs
in 25% of cases [220, 227].
At CT, mesenteric and peritoneal tumors are of intermedi-
ate attenuation and appear similar to nonopacified bowel.
Vascular encasement due to local extension through the base
of the mesentery usually appears as a mildly enhancing, con-
fluent mass surrounding the mesenteric vessels.
MR imaging reveals masses of homogeneous low-signal
intensity on T1-weighted images and heterogeneous interme-
diate-to-high signal intensity on T2-weighted mages. There is
mild enhancement after gadolinium administration.
FDG PET/CT is the imaging technique of choice for ini-
tial staging of pediatric Burkitt lymphoma, as the tumor is
highly FDG avid. It is also useful in follow-up as it permits
distinction of residual fibrotic masses from metabolically
active residual or recurrent tumor.
With intensive combination chemotherapy, sporadic Burkitt
lymphoma has 5-year event-free survival rates of 80–90%, even
in patients who present with advanced disease [228]. Relapse
may occur within the first year after treatment so that close fol-
low-up is required.
Rhabdomyosarcoma
Rhabdomyosarcoma is the most common pediatric soft-­
tissue tumor and can arise in or metastasize to almost any site
in the body, including the peritoneal cavity. It develops from
primitive mesenchymal cells that retain the capacity for skel-
etal muscle differentiation.
The embryonal and alveolar subtypes of rhabdomyosar-
coma occur in children. Approximately 10% of patients have
intraperitoneal involvement, which may represent primary or
metastatic disease, with metastases being more common
[230]. About 70% of patients are less than 10 years of age at
diagnosis, and tumors are more frequent in boys and
Caucasians [226]. Although most affected children have no
identifiable risk factors, rhabdomyosarcoma is associated
with Li-Fraumeni syndrome, NF1, Beckwith-Wiedemann
syndrome, and the DICER1 mutation.
552
a b
Fig. 13.81 Burkitt lymphoma with extensive intraperitoneal involve-
ment in a 3-year-old male. (a) Transverse grayscale ultrasound image
reveals large, lobulated, hypoechoic masses (asterisks) filling the pelvis
and displacing the bladder (B) anteriorly. (b) Transverse grayscale ultra-
sound image of the mid-abdomen shows additional hypoechoic masses
throughout the abdomen. The right and left renal pelves (arrowheads) are
On ultrasound imaging, ascites is identified in most cases
with peritoneal spread. Tumors are predominantly solid and
of mixed echogenicity (Fig. 13.82). Doppler evaluation may
show markedly increased blood flow. Omental caking has
been described in primary intraperitoneal tumors [229].
On CT, intraperitoneal and mesenteric nodules and
masses show hypoattenuation with variable contrast enhance-
ment. Calcification is rare. A pseudomyxoma peritonei-like
appearance is sometimes seen.
P. Duffy et al.
c
mildly dilated because of ureteral compression. (c) Transverse grayscale
ultrasound image of the left mid-­abdomen depicts hypoechoic tumor
(asterisk) infiltrating the wall of a bowel loop (arrowheads) medial to the
left kidney (K). (d) Coronal contrast-enhanced CT image demonstrates
tumor masses filling the abdomen and pelvis
MR imaging of omental caking and mesenteric adenopa-
thy has shown high T2 signal intensity of the involved struc-
tures [230]. Rhabdomyosarcoma is F ­ DG-­avid with FDG
PET imaging both highly sensitive and specific for the detec-
tion of nodal metastases.
Most patients are treated with neoadjuvant chemotherapy
and surgical resection. Prognosis is better for the embryonal
subtype than for the alveolar subtype. Because of advances in
multimodality treatment, the overall 5-year event-free survival
13 Spleen and Peritoneal Cavity
Fig. 13.82 Peritoneal rhabdomyosarcoma in a 4-year-old male. (a)
Transverse grayscale ultrasound image of the mid-abdomen shows a
large, well-circumscribed, echogenic mass with a central anechoic zone
(asterisk) in keeping with necrosis. There is adjacent thickening of the
greater omentum, or “omental cake” (arrows). (b) Transverse color
Doppler ultrasound image shows a small amount of flow at the periph-
rate for children with rhabdomyosarcoma is now approximately
70–75% [220],
Desmoplastic Small Round Cell Tumor
Desmoplastic small round cell tumor (DSRCT) is a rare
aggressive lesion that is a member of the small round blue
cell group of tumors that includes Wilms’ tumor, neuroblas-
toma, Ewing sarcoma, primitive neuroectodermal tumor
(PNET), and rhabdomyosarcoma. It usually develops as a
primary peritoneal tumor of the abdomen or pelvis, although
it can arise from other sites as well, including the salivary
glands, pleura, testes, or bone.
553
ery of the mass. (c) Transverse grayscale ultrasound image of the right
lower quadrant of the abdomen demonstrates ascites (asterisk). B,
Bladder. (d) Axial T2-weighted, fat-suppressed MR image of the mid-­
abdomen shows the large mass filling the peritoneal cavity. There is
central necrosis (asterisk) and a small amount of adjacent ascites
(arrowheads)
DSRCT occurs mainly in adolescents and young adults,
with a 4:1 male-to-female predominance [226]. Presenting
symptoms may include abdominal distension, abdominal
mass, and pain. Peritoneal dissemination is the most com-
mon pattern of metastatic spread [231].
Disseminated disease is often present at the time of initial
imaging, with multiple peritoneal, omental, and serosal
tumors. Masses are usually round or ovoid, with an average
size of about 11 cm. A single dominant mass is usually iden-
tified. Pelvic tumors often cause ureteral obstruction.
At ultrasound, tumor masses can be hyper- or hypoechoic,
depending on the degree of associated hemorrhage or necrosis
554
a b
c d
Fig. 13.83 Intraperitoneal desmoplastic small round cell tumor in a 15-year-​
old female. (a) Transverse grayscale ultrasound image of the mid-abdo-
men shows a rounded, heterogeneous mass with a large, bilobed central
fluid zone (asterisk). (b) Longitudinal grayscale ultrasound image demon-
strates a multilobulated, heterogeneous mass (arrows) in the hilum of the
(Fig. 13.83). Echogenic shadowing calcifications may be identi-
fied. Tumor vascularity is variable on color Doppler evaluation.
CT imaging of small masses will show low attenuation with
uniform enhancement after IV contrast administration. Larger
masses (>10 cm) may demonstrate a central low-attenuation
zone with heterogeneous contrast enhancement resulting from
necrosis, old hemorrhage, or fibrosis. Small calcifications can
also be seen. Liver metastases and abdominal and retroperito-
neal lymph nodes show decreased attenuation.
At MR imaging, tumors are usually hypo- to isointense to
skeletal muscle on T1-weighted images, and heterogeneously
iso- to hyperintense on T2-weighted images. Intratumoral hem-
orrhage may be seen on T1-weighted images as foci of high-
signal intensity. Occasional hypointense signal on T2-weighted
images is probably related to dense cellular components or des-
moplastic stroma. Modest, heterogeneous enhancement is seen
after IV gadolinium administration. Nonenhancing central foci
may be seen that could reflect hemorrhage, necrosis, or a fibro-
myxoid component of the tumor [220].
P. Duffy et al.
spleen (S). (c) Longitudinal grayscale ultrasound image of the pelvis
reveals a moderate amount of ascites (asterisks) surrounding the uterus
(U). (d) Axial contrast-enhanced CT image shows the mid-abdominal
intraperitoneal mass with a large central zone of low attenuation (aster-
isk) that may be due to hemorrhage or infarction
Despite multimodality treatment of DSRCT with surgery,
chemotherapy, and radiotherapy, the prognosis is very poor,
with a 5-year overall survival rate of only 25% [232].
Malignant Mesothelioma
Mesothelioma is a primary malignant tumor of the pleural,
peritoneal, and pericardial surfaces and of the tunica vagina-
lis that is extremely rare in childhood. Unlike adults, the
tumor is not clearly related to prior asbestos exposure, radia-
tion, or isoniazid. Clinical presentation usually includes pain
and abdominal distention.
Imaging findings may include a peritoneal mass, but more
often, there are peritoneal and mesenteric nodules and dif-
fuse or plaque-like peritoneal thickening or mesenteric thick-
ening without a dominant mass. Ascites is common [161,
220, 226]. A definitive diagnosis is usually made at the time
of tissue biopsy.
Due to the rarity of this disease in children, treatment pro-
tocols are not well established. Most patients have received
13 Spleen and Peritoneal Cavity
a b
L
M
Fig. 13.84 Peritoneal metastases in a 13-year-old female with Ewing
sarcoma. (a) Longitudinal grayscale ultrasound image shows a large,
rounded peritoneal mass (M) adjacent to the undersurface of the left
chemotherapy, sometimes combined with surgery. In the
majority of children, the disease is fatal [233].
Metastatic Disease
Metastatic disease to the peritoneal cavity in children can
occur from a variety of primary tumors, most often neuro-
blastoma, adenocarcinoma, Wilms’ tumor, germ cell tumor,
as well as intracranial neoplasms that spread along the path
of a ventriculoperitoneal (VP) shunt catheter [226]. Peritoneal
thickening, discrete masses, lymphadenopathy, bowel dila-
tion related to obstruction, and ascites are common manifes-
tations of metastatic peritoneal involvement regardless of the
primary tumor type (Fig. 13.84). Treatment and prognosis
will depend on the underlying tumor.
Neuroblastoma
Neuroblastoma is a common neoplasm of young children
that frequently spreads to the mesentery through the subperi-
toneal space and can also extend to the peritoneum [234].
When neuroblastoma arises from the organ of Zuckerkandl
in the pelvis, it can also metastasize to the peritoneum.
Although the primary tumor is typically calcified, the perito-
neal metastases of neuroblastoma do not calcify [220].
Adenocarcinoma
Duodenal adenocarcinoma is rare in children. When it
occurs, the second portion of the duodenum is usually
affected. Common patterns of metastatic spread are pancre-
atic invasion and lymphadenopathy. Peritoneal metastatic
disease is uncommon but can sometimes occur. The imag-
ing findings of peritoneal carcinomatosis include fibronod-
ular stranding, nodules, plaques, and masses. Mesenteric
555
M
S
M
hepatic lobe (L). There is a small amount of ascites (asterisk). (b) Longi­
tudinal color Doppler ultrasound image demonstrates tumor masses (M)
on either side of the spleen (S)
thickening can produce a “pleated” or stellate pattern on
CT [226].
Wilms’ Tumor
Peritoneal invasion by Wilms’ tumor can occur if it breaks
through the capsule prior to surgery or during surgical
manipulation. Peritoneal involvement manifests as plaque-­
like thickening or nodular implants in the mesentery and/or
omentum. There can also be include drop metastases in the
pelvis [226, 235].
Germ Cell Tumor
Peritoneal involvement by germ cell tumor is usually due to
metastatic spread of a primary ovarian mass. Intraperitoneal
spread may cause ascites, peritoneal nodules and masses,
and omental caking [226, 236].
Intracranial Neoplasms
Peritoneal seeding via a ventriculoperitoneal shunt can occur
in children with high-grade primary brain neoplasms, most
commonly medulloblastoma and pineal tumors [237].
Patients present with increasing abdominal distension and
ascites.
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 Pancreas, Adrenal Glands,
and Retroperitoneum 14
Anastasia L. Hryhorczuk and Harriet J. Paltiel

Abbreviations IVC Inferior vena cava

KHE Kaposiform hemangioendothelioma
ADPKD Autosomal dominant polycystic MEN1 Multiple endocrine neoplasia type 1
kidney disease MHz Megahertz
AIDS Acquired immunodeficiency MIBG Metaiodobenzylguanidine
syndrome MR Magnetic resonance
ANCs Acute necrotic collections MRCP Magnetic resonance
APFCs Acute peripancreatic fluid cholangiopancreatography
collections PET Positron emission tomography
CAH Congenital adrenal hyperplasia PNET Primitive neuroectodermal tumor
CEUS Contrast-enhanced ultrasound SEER National Cancer Institute
CFTR Cystic fibrosis transmembrane Surveillance, Epidemiology, and
conductance regulator End Result
COG Children’s Oncology Group SPECT Single-photon emission computerized
CT Computed tomography tomography
DOPA Dihydroxyphenylalanine VHL Von Hippel-Lindau
DOTA Dodecanetetraacetic acid WON Walled-off necrosis
EBV-SMTs Epstein-Barr virus-associated XGP Xanthogranulomatous
smooth muscle tumors pyelonephritis
ECMO Extracorporeal membrane
oxygenation
ERCP Endoscopic retrograde Introduction
cholangiopancreatography
F Fluoro The retroperitoneum is the anatomical space in the abdomen
HIV Human immunodeficiency virus located behind the peritoneum. Structures that lie between
HSV Herpes simplex virus the parietal peritoneum and the abdominal wall that are not
IEP Interstitial edematous pancreatitis suspended by a mesentery are classified as retroperitoneal
INRG International Neuroblastoma Risk in location and include the pancreas, adrenal glands, kid-
Group neys, duodenum, portions of the colon, great vessels, lymph
INRGSS International Neuroblastoma Risk nodes, and lymphatics. Disorders of the kidney, bowel, and
Group Staging System great vessels are reviewed in other chapters. In this chapter,
normal development and anatomy of the pancreas, adrenal
A. L. Hryhorczuk (*)
glands, and retroperitoneum are discussed, as well as ana-
Department of Radiology, C.S. Mott Children’s Hospital, Michigan
Medicine, Ann Arbor, MI, USA tomic variants, congenital abnormalities, inflammatory, trau-
e-mail: ahryhorc@med.umich.edu matic and neoplastic disorders.
H. J. Paltiel
Division of Ultrasound, Department of Radiology, Boston Children’s
Hospital and Harvard Medical School, Boston, MA, USA

© Springer Nature Switzerland AG 2021 563

H. J. Paltiel, E. Y. Lee (eds.), Pediatric Ultrasound, https://doi.org/10.1007/978-3-030-56802-3_14
564
Pancreas
Visualization of the pancreas in children is enhanced by a rela-
tive paucity of subcutaneous and intraabdominal fat, as well as
a relative prominence of the left lobe of the liver and spleen
which can be used as acoustic windows into the retroperito-
neum [1]. The pancreas is also proportionately larger in chil-
dren than in adults, which makes it easier to visualize [2].
Technique
Patient Positioning
Examination of the pancreas is performed with the patient
supine. Improved visualization of the distal body and tail of
the pancreas can be achieved by placing the patient in a right
lateral decubitus position or by having the patient drink
water and using the gastric fluid as an acoustic window. A
left lateral decubitus position can be useful in imaging the
portions of the gland nearest to the duodenum.
Ultrasound Transducer Selection
Images are obtained using the highest-frequency transducer
that provides adequate penetration, generally on the order of
5.0 to 7.5 Megahertz (MHz). High-frequency linear array
transducers can provide detailed images of the pancreatic
parenchyma (Fig. 14.1). A lower-frequency transducer may
be required for imaging in larger individuals.
L bile duct at the ampulla of Vater and drains into the major
V
A
Fig. 14.1 Normal pancreas in a 3-year-old male. Transverse midline
grayscale ultrasound image demonstrates the pancreas (arrows), with a
mildly lobulated contour and homogeneous, hypoechoic parenchyma.
The left lobe of the liver (L) serves as an acoustic window. The normally
oriented superior mesenteric artery (A), surrounded by echogenic fat, and
superior mesenteric vein (V) are identified posterior to the pancreas
A. L. Hryhorczuk and H. J. Paltiel
Imaging Approaches
Patients should be fasting to minimize gaseous distension of
bowel, which can prevent or diminish visualization of the pan-
creas. The pancreas is imaged in transverse and sagittal planes
with grayscale and color Doppler. The pancreatic head and
uncinate process are best viewed from a right subcostal or ante-
rior subxiphoid approach. The neck and body are usually opti-
mally imaged from an anterior subxiphoid approach, using the
left hepatic lobe as an acoustic window. The tail is ideally
depicted on coronal scans through the spleen or left kidney.
Portions of the liver should be included when imaging the
pancreas so that their echogenicity can be compared. In order
to ensure complete coverage of the gland, imaging should be
performed superiorly at least to the level of the celiac axis
and inferiorly to the level of the portal vein.
Normal Development and Anatomy
Normal Development
The pancreas appears at approximately the fifth week of gesta-
tion as separate ventral and dorsal buds that arise from the endo-
dermal lining of the duodenum. Each bud is joined to the foregut
through a duct. The ventral portion of the pancreas rotates
toward the dorsal portion, which ultimately join together. The
ventral bud develops into the pancreatic head and uncinate pro-
cess, with the caudal portion of the head wrapping behind the
superior mesenteric artery and vein. The dorsal bud develops
into the neck, body, and tail of the pancreas.
The ducts of both buds fuse to create the major pancreatic
duct of Wirsung that extends medially to join the common
papilla of the duodenum. The remaining dorsal duct devel-
ops into the accessory duct of Santorini that drains separately
into the minor duodenal papilla (Fig. 14.2) [3].
Normal Anatomy
On ultrasound, the normal pancreas has well-defined mar-
gins and homogeneous echotexture. It abuts the splenic
hilum and the duodenum (Fig. 14.3). The echogenicity of the
pancreas typically is equal to or greater than that of the liver.
Premature and newborn infants tend to have relatively hyper-
echoic pancreatic parenchyma. Pancreatic echogenicity in
children is related to the amount of parenchymal tissue. The
relatively increased pancreatic echogenicity in older indi-
viduals is related to the presence of fat and fibrous tissue
within the gland. With high-frequency linear transducers, a
normal pancreatic duct may be identified. The normal mean
diameter of the duct is 1.65 mm (± 0.45 mm) and can mea-
sure up to 2.2 mm in adolescents [4].
14 Pancreas, Adrenal Glands, and Retroperitoneum 565

a b

c d

Fig. 14.2 Diagram of normal pancreatic development. (a) In the fifth
week of gestation, the ventral (arrow) and dorsal (arrowhead) buds of the
pancreas emerge from the endodermal lining of the duodenum. (b) Both
the ventral (arrow) and dorsal (arrowhead) buds are joined to the duode-
num by a pancreatic duct. (c) The ventral bud rotates posteriorly (arrow)
and fuses with the dorsal bud. (d) Ducts within the dorsal and ventral
pancreas fuse, creating the major pancreatic duct of Wirsung which
inserts inferiorly at the major papilla, and the accessory pancreatic duct
of Santorini which drains superiorly into the minor papilla

Common Common Pancreatic

Right and left hepatic duct bile duct duct Spleen
hepatic ducts
of liver
Cystic duct

Gallbladder
Tail
Duodenum
Body
Minor duodenal
papilla

Accessory Head
pancreatic duct
Major duodenal Pancreas
papilla
Jejunum

Fig. 14.3 Diagram of normal pancreatic anatomy. The normal pan- major and minor duodenal papillae serve as the insertion sites for the
creas is a retroperitoneal organ, with the head associated with the major and minor accessory ducts, respectively
C-loop of the duodenum and the tail located at the splenic hilum. The
566
Anatomic Variants
 obulated Parenchymal Contour
L point to pancreas divisum as an independent risk factor for
The pancreatic contour may appear lobulated, simulating a
mass. These pseudomasses or bulges typically occur to the right
of the gastroduodenal and pancreaticoduodenal arteries or along
the anterior margin of the pancreas. A pseudomass will have an
echogenicity and echotexture identical to that of the normal
pancreas, which permits differentiation from a true pancreatic
lesion [5].
Congenital Anomalies
Disordered development, rotation, and/or fusion of the pancre-
atic buds are manifested in several well-described congenital
abnormalities of the pancreatic ducts and parenchyma.
Pancreas Divisum
Pancreas divisum is the most common developmental abnor-
mality of the pancreas, with a reported incidence ranging from
4.5% to 7% in the general population [6, 7]. In pancreas divi-
sum, the dorsal and ventral pancreatic ducts fail to fuse. This
causes the dorsal pancreatic duct to drain to the minor papilla,
while the ventral pancreatic duct drains to the major papilla
(Fig. 14.4). In this situation, drainage of the pancreas is pre-
dominantly through the dorsal duct which opens into the
minor papilla instead of through the ventral duct that opens
into the major papilla. The relatively small diameter of the
minor papilla increases the pressure in the dorsal pancreatic
duct that can lead to obstruction of pancreatic exocrine secre-
tions, ductal distention, and recurrent pancreatitis.
D Pancreas
Fig. 14.4 Diagram of pancreas divisum. The dorsal pancreatic duct
(arrowhead) fails to fuse with the ventral duct (arrow), leading the dor-
sal duct to drain to the minor papilla, while the smaller ventral duct
drains to the major papilla. D, Duodenum
A. L. Hryhorczuk and H. J. Paltiel
Although the role of pancreas divisum in the pathogenesis
of pancreatitis has long been debated, recent investigations
pediatric pancreatitis [7].
On ultrasound, pancreas divisum is difficult to identify,
especially in the absence of an irregular or dilated pancre-
atic duct. If the main pancreatic duct is visualized and is
seen to drain superiorly to the minor papilla, a diagnosis of
pancreas divisum can be made. Prior to further interven-
tion, magnetic resonance cholangiopancreatography
(MRCP) is typically performed for definitive diagnosis of
pancreas divisum [8].
Although patients with pancreas divisum are typically
asymptomatic, between 5% and 10% of individuals with
variant pancreatic drainage will develop pancreatitis [9].
Endoscopic retrograde cholangiopancreatography (ERCP)
with sphincterotomy and ductal stenting is typically the ini-
tial treatment for pancreas divisum and is designed to
improve drainage from the minor papilla. If this fails, surgi-
cal treatment, including open sphincteroplasty or pancreatic
head resection, can be considered [9].
Annular Pancreas
Annular pancreas is an embryologic abnormality where pan-
creatic parenchyma surrounds the second portion of the duo-
denum. It occurs in approximately 1 in 1000 individuals.
Although the specific developmental event that leads to
annular pancreas has not been elucidated, many hypothesize
that it occurs when the ventral bud of the pancreas adheres to
the duodenal wall, leading the pancreatic parenchyma to
encircle the duodenum during further development
(Fig. 14.5) [10].
Duodenum
Fig. 14.5 Diagram of annular pancreas. Pancreatic parenchyma encir-
cles the duodenum, with associated compression of the encased bowel
14 Pancreas, Adrenal Glands, and Retroperitoneum
P hepaticojejunostomy [16].
Fig. 14.6 Annular pancreas in a 1-day-old male with trisomy 21 and
proximal bowel obstruction. Transverse grayscale ultrasound image
shows the duodenum (arrow) encircled by the pancreatic head (P). The
duodenum appears diminutive as it passes through the pancreatic head.
Duodenal atresia was identified at surgery
Annular pancreas is associated with chromosomal abnor-
malities, including trisomy 21, and other gastrointestinal abnor-
malities such as malrotation [11]. Patients generally present as
neonates, usually with duodenal stenosis or atresia. However, a
smaller number of patients present later in life.
Annular pancreas can be difficult to diagnose with ultra-
sound; the duodenum may not be clearly surrounded by pan-
creatic parenchyma, or it may be atretic in the region of the
pancreatic head, making it difficult to delineate (Fig. 14.6).
Decompression of a gas-filled stomach with a nasogastric
tube and introduction of fluid into the stomach and proximal
duodenum can improve ultrasound visualization [12].
Gastrojejunostomy or duodenoduodenostomy are the pre-
ferred treatments for patients with annular pancreas. Because
of the complex ductal anatomy of the pancreas, resection of
the annular portion of the pancreas is not recommended [13].
 ommon Pancreaticobiliary Channel
C and treatment, since it can be difficult to distinguish between a
A common pancreaticobiliary channel develops when the
pancreatic duct and the common bile duct join proximal to
the duodenum, resulting in a common channel measuring
more than 1.5 cm in length. Contraction of the sphincter of
Oddi (the smooth muscle in the duodenum that usually sur-
rounds the distal ends of the common bile duct and main
pancreatic duct, and controls the flow of bile and pancreatic
567
secretions into the small bowel) is ineffective in this setting.
Pancreaticobiliary reflux can occur, leading to pancreatitis
and cholangitis [14]. There is also an association between a
common pancreaticobiliary channel with choledochal cyst
and biliary tract carcinoma.
Ultrasound identification of a common pancreaticobiliary
channel may be difficult. The diagnosis can be suggested if a
dilated ductal structure is seen with an abnormal junction with
the pancreatic duct. Signs of pancreatitis or cholangitis can
also raise the possibility of this diagnosis (Fig. 14.7) [15].
Patients with an abnormal pancreaticobiliary junction will
require resection of the extrahepatic bile duct and hepatico-
enterostomy. In most cases, this will consist of a Roux-­en-­Y
 artial Pancreatic Agenesis
P
Congenital partial agenesis of the pancreas is rare. Patients
usually lack the dorsal portion of the pancreas, with preserved
ventral pancreatic tissue. This abnormality can be visualized
by ultrasound, computed tomography (CT), or magnetic reso-
nance (MR) imaging (Fig. 14.8). Complete absence of both
the ventral and dorsal pancreatic tissue is typically fatal, with
severe associated intrauterine growth restriction [17].
In typical cases of partial pancreatic agenesis, the pancre-
atic neck, body, and tail will be absent, with loops of bowel
identified in the expected location of the pancreas (Fig. 14.9).
Some patients may present with neonatal diabetes, while
other cases are identified incidentally if the residual pancre-
atic tissue provides adequate endocrine function [17].
Treatment requires medical replacement of pancreatic endo-
crine and exocrine function in symptomatic patients. Pancreatic
enzymes can be used if there are signs of pancreatic exocrine
deficiency, and insulin is required for treatment of diabetes.
 ccessory Pancreatic Lobe
A
Accessory pancreatic lobes are rarely encountered. When an
accessory lobe is present, there is an aberrant duct that con-
nects to the main pancreatic duct [18]. Patients are predisposed
to abdominal pain and recurrent pancreatitis, which can occur
from obstruction within the accessory pancreatic duct.
Several case reports have noted an association between
accessory pancreatic lobes and gastric duplication cysts, hypoth-
esizing that an abnormal connection between the duct draining
the accessory pancreatic lobe and the duplication cyst may
incite episodes of recurrent pancreatitis [19]. The presence
of an associated duplication cyst may confound management
pancreatic pseudocyst and a duplication cyst on CT or MR
imaging [20].
By ultrasound, a duplication cyst or a dilated pancreatic
duct can be identified, but the accessory pancreatic lobe may
not be appreciated [21]. MRCP permits visualization of the
accessory pancreatic duct and may be useful in surgical plan-
ning if the diagnosis is suspected.
568
Fig. 14.7 Common pancreaticobiliary channel in 15-year-old female
with pancreatitis. (a) Transverse grayscale ultrasound image of the mid-­
abdomen demonstrates a dilated common bile duct (arrow), which
could be traced back to its junction with the pancreatic duct. P, Pancreas.
(b) Transverse grayscale ultrasound image of the mid-abdomen obtained
slightly inferior to image in (a) shows a mildly dilated common pancre-
a Complete absence of
dorsal pancreas
Fig. 14.8 Diagram of partial pancreatic agenesis. This disorder typically involves either complete (a) or partial (b) absence of the dorsal pancreas.
The ventral pancreas is usually preserved
A. L. Hryhorczuk and H. J. Paltiel
aticobiliary channel (arrow) terminating in the gas-filled duodenum
(asterisk). (c) Coronal magnetic resonance cholangiopancreatography
(MRCP) image demonstrates marked dilation of the intra- and extrahe-
patic bile ducts compatible with a type IV choledochal cyst, as well as
dilation of the common pancreaticobiliary ­channel (arrow)
b Partial absence of
dorsal pancreas
14 Pancreas, Adrenal Glands, and Retroperitoneum
Fig. 14.9 Partial pancreatic agenesis in an 11-year-old female with a his-
tory of neonatal diabetes. (a) Transverse color Doppler ultrasound image
demonstrates the splenic vein (arrows) without visualization of any pan-
creatic tissue between the liver (L) and splenic vein. (b) Transverse
Surgical resection of the accessory lobe is required for
definitive treatment and symptomatic relief.
Ectopic Pancreas
Ectopic pancreatic rests are present in 1.7% of individuals at
autopsy, with the majority located in the gastric antrum and
duodenum and the rest throughout the small bowel [22].
Pancreatic rests are not commonly encountered on transab-
dominal ultrasound imaging, although they may be identified
by endoscopic ultrasound, where they appear as submucosal
masses of variable echogenicity [22].
Typically, ectopic pancreatic rests contain multiple small
ducts that allow for adequate drainage and prevent obstruc-
tion or inflammation [13]. Often, they will be encountered
incidentally during endoscopic or surgical intervention.
However, they may cause bowel obstruction or bleeding and
require surgical removal.
Congenital Hyperinsulinism
At birth, patients with congenital hyperinsulinism will pres-
ent with refractory hypoglycemia, which can be profound
and difficult to control. Underlying genetic abnormalities,
which affect the control of insulin production from pancre-
atic β cells, may lead to diffuse or focal congenital hyperin-
sulinism [23]. Patients with diffuse hyperinsulinism will
569
grayscale ultrasound image reveals a small portion of pancreatic tissue
(asterisk) in the expected location of the pancreatic head, reflecting the
preserved ventral portion of the gland. L, Liver
have involvement of the entire pancreatic gland, without a
discrete lesion that can be surgically resected. Conversely,
those with focal hyperinsulinism will have a defined intra-
pancreatic lesion that can be identified on imaging that
directs curative surgical resection.
Currently, preoperative imaging for patients with ­congenital
hyperinsulinismrequires18-fluoro(F)-dihydroxyphenylalanine
(DOPA) positron emission tomography (PET)/CT, which is
essential for the detection of a focal pancreatic lesion [24].
Ultra­sound can play an important adjunctive role in defining the
­anatomic borders of the lesion, both preoperatively and intraop-
eratively. These masses have been described as predominantly
hypoechoic, with poorly defined margins that can be difficult to
identify without imaging [25].
Resection of any mass is imperative for patients with
focal hyperinsulinism. In conjunction with frozen sections,
intraoperative ultrasound can provide the guidance required
for a tissue-sparing pancreatic resection in this challenging
surgical population [26].
 ongenital Pancreatic Cyst
C
Congenital pancreatic cysts are extremely rare and are usu-
ally definitively diagnosed only on histological examination.
Patients are often asymptomatic, although they can present
with an enlarging abdominal mass. These lesions are thought
570 A. L. Hryhorczuk and H. J. Paltiel

to originate from an abnormality in ductal development, where
an embryonic pancreatic duct fails to regress and becomes
obstructed [27].
On ultrasound, a congenital pancreatic cyst appears as a
fluid-filled structure in the vicinity of the pancreas. Since
many other cystic lesions can occur in this location, congeni-
tal pancreatic cysts in the body or tail of the pancreas are
typically mistaken for pancreatic pseudocysts or gastrointes-
tinal duplication cysts, while cysts in the head or neck of the
pancreas are often assumed to be choledochal cysts [28].
Although the origin and etiology of these cysts may be
difficult to diagnose prior to pathologic examination, analy-
sis of the cystic fluid after drainage may show high concen-
trations of amylase, which suggests the correct diagnosis of
a congenital pancreatic cyst.
pancreatic parenchyma by macrocysts larger than 1 cm in
diameter in patients with cystic fibrosis and can be well-­
delineated by ultrasound (Fig. 14.11). Patients are treated
with replacement of endocrine and exocrine hormones, as
needed.
Shwachman-Diamond Syndrome
Shwachman-Diamond syndrome is a rare autosomal recessive
disorder that is associated with bone marrow failure, exocrine
pancreatic deficiency, and significant growth delay. On imag-
ing, the appearance of the pancreas is similar to patients with
cystic fibrosis with near-complete fatty replacement of the

 enetic Disorders with Associated Pancreatic

G
Abnormalities

Many systemic genetic disorders have pancreatic abnormali-

ties as a component of the disorder.

Cystic Fibrosis
In patients with cystic fibrosis, the abnormal viscous mucoid
secretions produced by the pancreas lead to pancreatic ductal
obstruction, pancreatic autodigestion, and eventual exocrine
insufficiency. This pathologic process culminates in fatty
replacement, cyst formation, calcification, and atrophy. On
ultrasound, the pancreas is usually small and echogenic and Fig. 14.10 Cystic fibrosis in a 10-year-old male. Transverse grayscale
ultrasound image demonstrates fatty replacement (asterisks) of most of
may contain focal calcifications (Fig. 14.10) [25]. The term the pancreas with only a small portion of preserved pancreatic paren-
“pancreatic cystosis” refers to complete replacement of the chyma (arrow)

a b

Fig. 14.11 Pancreatic cystosis in a 16-year-old female with cystic contrast-enhanced computed tomography (CT) image shows multiple
fibrosis. (a) Transverse grayscale ultrasound image shows complete fluid-filled cysts (arrowheads) in the expected location of the pancreas
replacement of the pancreas with cysts of varying size. (b) Coronal
14 Pancreas, Adrenal Glands, and Retroperitoneum
pancreatic parenchyma (Fig. 14.12). Histologically, islet cells
are preserved within the fatty pancreas, but patients may
require exocrine pancreatic supplementation [29].
Beckwith-Wiedemann Syndrome
Beckwith-Wiedemann syndrome comprises a variety of genetic
and molecular abnormalities. While some patients have classic
features (macroglossia, unilateral overgrowth, omphalocele,
hyperinsulinemia, Wilms’ tumor/nephroblastomatosis), others
patients may manifest with isolated hemihypertrophy or only a
few clinical features [30]. At birth, infants may be identified
with macrosomia, macroglossia, abdominal wall defects such as
omphalocele, and neonatal hypoglycemia. Neonatal hypoglyce-
mia is typically due to diffuse pancreatic hypertrophy, without a
discrete focal pancreatic abnormality.
S PCKD is suggestive of a PKD2 mutation, rather than a PKD1
Fig. 14.12 Shwachman-­Diamond syndrome in an 18-year-old male.
Transverse grayscale ultrasound image demonstrates fat (asterisks) in
the expected location of the pancreas anterior to the splenic vein (arrow).
S, Stomach
a b
Fig. 14.13 Pancreatic cysts in a 21-year-old female with autosomal
dominant polycystic kidney disease (ADPKD). (a) Transverse gray-
scale ultrasound image shows several cysts (arrowheads) in the distal
571
The pancreas may appear diffusely enlarged by ultrasound,
without a focal lesion. Beckwith-Wiedemann patients are typ-
ically followed closely with serial abdominal ultrasound
examinations designed to screen for solid tumors, including
Wilms’ tumor, hepatoblastoma, neuroblastoma, adrenal corti-
cal carcinoma, and rhabdomyosarcoma. Screening protocols
typically include serial abdominal ultrasound studies every
3 months until 4 years of age, followed by renal ultrasound
studies every 3 months until 7 years of age [30]. Vigilant mon-
itoring and supportive management for neonatal hypoglyce-
mia is required.
 utosomal Dominant Polycystic Kidney Disease
A
For patients with autosomal dominant polycystic kidney disease
(ADPKD), pancreatic cysts are a minor component of their over-
all clinical presentation. However, pancreatic cysts are identified
in these patients at a greater frequency than in the general popu-
lation [31]. On ultrasound evaluation, these cysts have typical
features, including anechoic contents, an imperceptible wall
and increased through transmission (Fig. 14.13). Although pan-
creatic cysts may not alter the management of the renal and
vascular findings that typically dominate the clinical course of
these patients, the presence of pancreatic cysts in patients with
mutation which has prognostic implications [31].
 on Hippel-Lindau Disease
V
Von Hippel-Lindau (VHL) disease is caused by a germline
mutation in the VHL gene, a tumor suppressor gene on chro-
mosome 3. This disease is inherited in families in an autoso-
mal dominant fashion and is typically associated with renal
cell carcinoma, hemangioblastoma, and pheochromocytoma
[32]. Patients with VHL are also at risk for pancreatic neo-
plasms. Pancreatic disorders associated with VHL include
neuroendocrine tumor, serous cystadenoma, and pancreatic
cyst. Pancreatic disease is rare in children with VHL and
L
pancreatic body. A distal pancreatectomy had been performed several
years earlier. (b) Longitudinal grayscale ultrasound image of the right
kidney reveals multiple cysts in keeping with ADPKD. L, Liver
572 A. L. Hryhorczuk and H. J. Paltiel

typically presents at a later age, usually in the fourth decade time, APFCs or ANCs may either resolve or persist, developing
of life [33]. On ultrasound, a neuroendocrine tumor usually a mature wall to become a pseudocyst or a WON, respectively.
appears as a solid pancreatic mass. Any collection subtype can become infected. However, this
Pancreatic cysts and small serous cystadenomas are typically most often occurs with necrotic collections [36].
managed expectantly, while larger serous cystadenomas require Ultrasound is used as the initial imaging modality in the
resection [32]. Pancreatic neuroendocrine tumors carry a poten- diagnosis of acute pancreatitis in children. CT is generally per-
tial for metastatic disease and are resected if they demonstrate formed to detect complications in patients who do not respond
rapid growth or are greater than 3 cm in size at diagnosis [34]. to initial treatment. MR imaging is used to determine underly-
ing anatomical abnormalities that predispose pediatric patients
to pancreatitis. Early imaging in patients with pancreatitis may
Acute Pancreatitis be unremarkable. In patients with confirmed acute pancreatitis
imaged within 1 week of presentation, only about 52% of
Acute pancreatitis is the most common disorder of the pancreas patients have positive findings on ultrasound examination [39].
in children. Its incidence ranges from 3 to 13 cases per 100,000 The most common ultrasound findings in the acute period
children/year, and has been slowly increasing over the past include pancreatic and peripancreatic edema, with peripancre-
20 years [35]. Common etiologies of pediatric pancreatitis atic fluid collections and pancreatic ductal dilation occurring in
include trauma, biliary tract disease, drug-related causes, and about 20% of patients (Figs. 14.14 and 14.15) [39].
systemic disease [36]. Between 10% and 20% of cases are
regarded as idiopathic.
Acute pancreatitis is defined by the International Study
Group of Pediatric Pancreatitis in Search of a Cure
(INSPPIRE) as a reversible inflammation of the pancreatitic
parenchyma where two of the three following criteria are met: P
(1) abdominal pain typical for acute pancreatitis (e.g., acute
in onset, located in the epigastric region); (2) serum amylase
and/or lipase greater than three times the upper limit of nor-
mal; and (3) imaging findings on any modality compatible
with acute pancreatitis [37]. Once a diagnosis has been
established, patients can be classified as having mild, moder-
ate, or severe pancreatitis.
Mild acute pancreatitis is diagnosed when there is no evi-
dence of organ failure, an absence of local or systemic compli- Fig. 14.14 Acute pancreatitis in a 2-year-old girl. Transverse gray-
cations, and resolution of symptoms within 1 week. Moderate scale ultrasound image demonstrates a diffusely enlarged pancreas (P)
acute pancreatitis includes patients who experience transient with minimal peripancreatic fluid (arrows)
organ dysfunction (<48 hours), local complications, and/or
worsening of a preexisting comorbidity. Severe pancreatitis is
characterized by organ dysfunction that lasts longer than 48 hours
[35].
The 2012 revised Atlanta classification is a standardized
clinical and radiologic nomenclature for acute pancreatitis and
its complications in adults that divides acute pancreatitis into
two subtypes:interstitial edematous pancreatitis (IEP) and nec-
P
rotizing pancreatitis, based on whether or not necrosis is pres-
ent. There is currently no similar classification scheme for
pediatric pancreatitis. However, since the complications in chil-
dren are similar to those in adults, it provides a useful frame-
work [38].
Four distinct collection subtypes are identified on the basis of
the presence of pancreatic necrosis and time elapsed since the
onset of pancreatitis. Acute peripancreatic fluid collections
(APFCs) and pseudocysts occur in IEP and only contain fluid.
Fig. 14.15 Acute pancreatitis in 7-year-old male with acute lympho-
Acute necrotic collections (ANCs) and walled-off necrosis blastic leukemia. Transverse grayscale ultrasound image of the pan-
(WON) occur only in patients with necrotizing pancreatitis and creas (P) reveals hypoechoic parenchyma and increased echogenicity of
contain variable amounts of fluid and necrotic debris. APFCs the peripancreatic fat (arrows) compatible with inflammatory change.
and ANCs develop within 4 weeks of disease onset. After this No peripancreatic fluid collection is identified
14 Pancreas, Adrenal Glands, and Retroperitoneum
In many cases, pediatric patients with acute pancreatitis
will be treated conservatively, with pain control and nasogas-
tric tube decompression when there is significant vomiting
[36]. Children will often recover within a week.
Approximately 20% of pediatric patients will experience a
local or systemic complication. Local complications include
peripancreatic fluid collections.
 cute Peripancreatic Fluid Collections
A atitis. Pseudocysts are surrounded by a defined wall and
Acute peripancreatic fluid collections are defined as col-
lections seen in the first 4 weeks following a diagnosis of
pancreatitis. When evaluated by ultrasound, these collec-
tions contain simple fluid, lack a defined wall, and are
P
Fig. 14.16 Acute peripancreatic fluid collection in a 5-year-old female
with propionic acidemia and a 1-week history of pancreatitis. Transverse
grayscale ultrasound image of the pancreas (P) demonstrates a small,
ovoid fluid collection (asterisk) adjacent to the pancreatic body and tail
a b
Fig. 14.18 Pancreatic pseudocyst in 14-year-old male. (a) Transverse
grayscale ultrasound image of the left upper quadrant demonstrates a
complex fluid collection (white asterisk) with internal debris. The col-
lection appears bilobed, with a deeper, less-well visualized heteroge-
neous component (arrow). Adjacent retroperitoneal fat (black asterisk)
573
c­onfined to the fascial planes adjacent to the pancreas
(Fig. 14.16) [40]. In most cases, acute peripancreatic fluid
collections resolve spontaneously as the patient’s clinical
condition improves.
Pseudocysts
Pancreatic pseudocysts represent fluid collections that per-
sist for more than 4 weeks after an episode of acute pancre-
may contain a combination of fluid and debris (Fig.14.17)
[36]. On ultrasound, these collections demonstrate a dis-
crete wall and may produce significant mass effect with
displacement of adjacent structures (Fig. 14.18).
P
Fig. 14.17 Pancreatic pseudocyst in an 8-year-old-male with a history
of pancreatitis. Transverse grayscale ultrasound image of the pancreas
(P) shows an anechoic cystic structure (asterisk) in the pancreatic tail
is echogenic, in keeping with local inflammatory change. (b) Axial
T2-weighted MR image depicts a complex, heterogeneous collection
in the left upper quadrant (arrows) extending to the pancreatic tail.
The collection contains both superficial and deep components. An
additional cystic lesion (asterisk) is identified in the pancreatic head
574
Although some pseudocysts can be managed expectantly,
if there is persistent pain, enteric, or biliary obstruction, or
continuous enlargement over time, drainage is indicated.
In adults, endoscopic drainage of pancreatic pseudocysts
is the standard of care [41]. Techniques include endoscopic
ultrasound with cystogastrostomy and stent placement
(Fig. 14.19). Although the few published pediatric series on
a b
Fig. 14.19 Pancreatic pseudocyst in a 6-year-old female. (a) Transverse
grayscale ultrasound image of the left upper quadrant reveals a complex
fluid collection (asterisk) with internal debris. (b) Intraoperative fluoro-
scopic image shows a cystogastrostomy tube (arrows) externalized through
A. L. Hryhorczuk and H. J. Paltiel
the use of endoscopic ultrasound contain small sample sizes,
endoscopic techniques are generally regarded as promising
for the management of pancreatic pseudocysts in symptom-
atic children [42]. However, these treatments can be limited
in children due to their small size which restricts the types of
stents and accessories that can be advanced through the
endoscope during the procedure [43].
a gastric stoma. A gastrojejunostomy tube (arrowhead) is also identi-
fied. (c) Follow-up grayscale ultrasound image obtained 2 weeks later
demonstrates a portion of the cystogastrostomy tube (arrow) within the
stomach, and interval resolution of the pseudocyst. L, Liver
14 Pancreas, Adrenal Glands, and Retroperitoneum
Pancreaticopleural Fistula
A pancreaticopleural fistula is an extremely rare complica-
tion of acute pancreatitis, where an intraabdominal pseudo-
cyst extends through a diaphragmatic defect to cause a
pleural effusion. Discrete diaphragmatic defects may be dif-
ficult to visualize by ultrasound, especially on the left, where
the close association of the stomach and the diaphragm may
obscure a small area of communication between the intra-
peritoneal and pleural spaces [44]. If pleural fluid is sampled,
high levels of pancreatic enzymes will confirm the diagnosis.
Treatment of a pancreaticopleural fistula is conservative,
with most fistulas closing following repeated thoracentesis
and resolution of the underlying pancreatitis [44].
a b
L
Fig. 14.20 Necrotizing pancreatitis in a 12-year-old female. (a) Transverse
grayscale ultrasound image of the pancreas (arrows) demonstrates edema-
tous, hypoechoic parenchyma. L, Liver. (b) Axial contrast-enhanced CT
image reveals poor enhancement of the pancreatic parenchyma (P), with
575
Necrotizing Pancreatitis
Necrotizing pancreatitis is typically defined by CT criteria, with
non-enhancement of at least 30% of the pancreas or a 3 cm
focus of necrosis on CT serving as the main diagnostic features
[45]. However, once a diagnosis of necrotizing pancreatitis has
been made, ultrasound can aid in following complications,
including acute fluid collections and pseudocysts (Fig. 14.20).
Treatment of necrotizing pancreatitis centers around supportive
care and management of complications. Typically, children with
necrotizing pancreatitis require longer hospital stays compared
to those with uncomplicated acute pancreatitis, with a high rate
of late complications, including pancreatic endocrine and exo-
crine insufficiency [45].
P
extensive surrounding fat stranding and retroperitoneal edema (arrow).
Ascites (asterisks) is also present. (c) Transverse grayscale ultrasound
image obtained 2 months later shows a pseudocyst (asterisk) of the pancre-
atic tail. P, Pancreas
576 A. L. Hryhorczuk and H. J. Paltiel

a b

Fig. 14.21 Splenic vein thrombosis in a 2-year-old female with necro- ultrasound image shows reconstitution of the splenic vein (arrow)
tizing pancreatitis. (a) Transverse color Doppler ultrasound image of more centrally, with patency of the superior mesenteric vein (arrow-
the spleen (S) demonstrates a patent splenic artery (arrows), with non-­ head). A large pseudocyst (asterisk) is seen anteriorly
visualization of the splenic vein. S, Spleen (b) Transverse color Doppler

Vascular Complications
Vascular complications can also affect children with acute
pancreatitis. Pseudoaneurysms are rare but can be fatal.
Endovascular management with coiling or stenting may
provide a less invasive way of treating pseudoaneurysms,
replacing open surgery that may require extensive pancre-
atic debridement [46]. Splanchnic venous thrombosis can P
also occur, most often involving the splenic vein (Fig. 14.21)
[47]. Most patients with splenic vein thrombosis will be
asymptomatic, although extensive thrombosis with involve-
ment of the superior mesenteric vein may result in portal
hypertension, variceal bleeding, and persistent ascites [48].

Acute Recurrent and Chronic Pancreatitis

Although most children with acute pancreatitis will recover
without further episodes, acute recurrent or chronic pancre-
atitis occurs in a smaller group of patients. Studies have
estimated that between 9% and 35% of children with acute
pancreatitis ultimately develop acute recurrent or chronic
pancreatitis [49]. In many cases, these children are eventu-
ally identified as having a predisposing underlying genetic
abnormality, including cystic fibrosis transmembrane con-
ductance regulator (CFTR) mutations [50]. Others have
anatomic abnormalities or biliary tract disease that ­provides
the substrate for recurrent/chronic pancreatitis. Genetic
mutations and a family history of pancreatitis are more
common in patients who present at or less than 6 years of
age [49].
Fig. 14.22 Chronic pancreatitis in a 10-year-old female. Transverse gray-
scale ultrasound image depicts an atrophic pancreas (P) with an irregular,
prominent pancreatic duct (arrow). A linear focus of increased echogenicity
(arrowhead) within the pancreatic head represents calcification
Recurrent/chronic pancreatitis can manifest multiple abnor­
malities on ultrasound evaluation, including ductal and
parenchymal abnormalities. Ductal findings include dilation,
intraductal calcification, and wall irregularity. Parenchymal
changes include atrophy, lobulation, and soft tissue calcifica-
tion (Figs. 14.22 and 14.23) [51].
For children with acute recurrent/chronic pancreatitis,
genotyping may be of benefit to identify a predisposing
mutation. As research in this area advances, knowledge of a
specific genotypic abnormality may help direct personalized
prognostic data as well as identify targeted treatments [52].
14 Pancreas, Adrenal Glands, and Retroperitoneum
Currently, however, treatment of chronic pancreatitis in chil-
dren focuses on identifying exocrine and endocrine dysfunc-
tion, with enzyme and insulin replacement as needed.
Fig. 14.23 Chronic pancreatitis in an 8-year-old female. Transverse
grayscale ultrasound image demonstrates significant atrophy of the pan-
creatic parenchyma (arrowheads). The pancreatic duct (arrow) is dilated
and filled with echogenic debris
a
Fig. 14.24 Pancreatic transection in a 13-year-old male after a bicycle
handlebar injury. (a) A transverse contrast-enhanced ultrasound image
(right panel) shows transection of the pancreas (asterisk) with a small
fluid collection between the two pancreatic moieties (arrowheads).
577
Surgical treatments typically used in adults to address
chronic pain, including resection or pancreaticojejunostomy,
are less effective in children [52]. Total pancreatectomy with
islet cell autotransplantation, which is described later in this
chapter, may allow for pain relief in children while decreas-
ing the risk of post-surgical diabetes. This surgical treatment
is typically reserved for select patients after extensive coun-
seling regarding procedural risks [53].
Trauma
Pancreatic injury is uncommon in pediatric blunt abdominal
trauma. An analysis of the National Trauma Data Bank from
2007 to 2011 reveals that only 0.3% of registered pediatric cases
included blunt pancreatic injury [54]. Injuries to the pancreas
typically result from motor vehicle collisions, bicycle/handlebar
injuries, and direct blows to the abdomen. Patients may present
acutely or following a delay from the inciting event.
Ultrasound findings of isolated pancreatic injury may be
subtle, although recent investigations have shown potential for
contrast-enhanced ultrasound (CEUS) as a tool for identifying
pancreatic injuries (Fig. 14.24). In experienced hands, CEUS
L
There is also a liver (L) laceration (arrow). A reference grayscale image
is shown in the left panel. (b) Axial contrast-enhanced CT image con-
firms the pancreatic (arrowhead) and liver (arrow) lacerations
578
of the pancreas has been shown to have a sensitivity of 95.5%
for diagnosis of blunt pancreatic trauma when compared to
CT as the gold standard [55]. Nonetheless, at most medical
centers, CT is still the primary imaging modality used for
diagnosis (Fig.14.25).
On CT, a discrete cleft may be identified within the pan-
creatic parenchyma, with surrounding fluid or inflammatory
Fig. 14.25 Pancreatic laceration in a 6-year-old male following a bicycle
accident. (a) Transverse grayscale ultrasound image of the pancreas dem-
onstrates a subtle linear focus of decreased echogenicity (arrowheads) in
Fig. 14.26 Non-accidental pancreatic trauma in a 2-year old male. (a)
Axial contrast-enhanced CT image depicts a cleft of low attenuation
(arrowheads) at the pancreatic head-neck junction in keeping with a
laceration. The patient was treated conservatively with a pancreatic
ductal stent. (b) Transverse grayscale ultrasound image of the pancreas
A. L. Hryhorczuk and H. J. Paltiel
change. However, visualization of the pancreatic duct may
be difficult by CT, requiring MRCP for further assessment
[56]. Following diagnosis of pancreatic injury, ultrasound
can be used to assess for pseudocyst formation or ductal
stricture (Fig. 14.26).
Treatment of pancreatic injury is variable, with most cen-
ters using a variety of operative and non-operative techniques
the body of the gland. (b) Axial contrast-­enhanced CT image reveals the
pancreatic laceration (arrow)
obtained 1 month later reveals a prominent distal duct (asterisk) mea-
suring between 2 and 3 mm in greatest diameter, which terminates
abruptly at the pancreatic head-neck junction. Dilation likely related to
a stricture at the site of injury
14 Pancreas, Adrenal Glands, and Retroperitoneum 579

[57]. ERCP can assist in diagnosing ductal injury and treat- can also occur [64]. The appearance of these lesions on ultra-
ing late complications, including ductal strictures [58]. sound, CT, and MR imaging mirrors their histology.
Further research into the management of pediatric pancreatic Although asymptomatic pancreatic serous cystadenoma can
injury will be essential for optimizing future care. be monitored without intervention in adults, the few reports of
serous cystadenoma in children describe symptomatic lesions
that are usually rapidly treated with surgical resection [65].
Pancreatic Venous and Lymphatic
Malformations Mucinous Cystadenoma
Unlike benign serous tumors, pancreatic mucinous cystade-
Venous and lymphatic malformations of the pancreas are noma has malignant potential and will require resection. In
extremely rare, accounting for less than 0.1% of all pediatric most cases, this tumor occurs in middle-aged women, although
vascular malformations [59, 60]. These lesions are more com- a few reports of this tumor in children have been published
mon in females than males and typically present during adult [66, 67].
life, although case reports have described pancreatic lymphatic In mucinous pancreatic cystadenoma, cysts are larger
malformations in children as young as 2 years of age [61]. than in the serous lesions and may have a thick peripheral
On ultrasound, venous and lymphatic malformations of pseudocapsule and possible calcifications [63]. As these
the pancreas may appear as partially cystic masses. Venous lesions are considered pre-malignant, surgical resection is
flow within vascular channels may be difficult to demon- standard management.
strate, and lesions may be indistinguishable from other cystic
pancreatic lesions [61]. Further assessment with CT or MR Infantile Hemangioma
imaging is typically required prior to biopsy or pre-­surgical Infantile hemangioma is a vascular soft tissue tumor char-
planning. acterized by an abnormal proliferation of endothelial cells
As pancreatic venous and lymphatic malformations may and aberrant blood vessel architecture. These lesions typi-
be difficult to diagnose prospectively, most patients are usu- cally demonstrate a growth phase during early infancy,
ally scheduled for surgical resection for a suspected cystic followed by a period of spontaneous involution that usu-
pancreatic neoplasm. If the lesion is biopsied and preopera- ally starts by 1 year of age [68]. Infantile hemangioma is
tively determined to be a venous or lymphatic malformation, most often encountered as a cutaneous or subcutaneous
sclerotherapy can be performed for further treatment. vascular mass, usually readily identified on physical
examination. Solid organ infantile hemangiomas also
occur, most commonly within the liver. Pancreatic infan-
Benign Pancreatic Neoplasms tile hemangiomas are rarely reported and have usually
been diagnosed when they cause biliary obstruction and
Serous Cystadenoma jaundice [69, 70].
Serous cystadenoma of the pancreas is a benign tumor not Ultrasound imaging of infantile hemangioma will demon-
typically seen in the pediatric population. It is usually identi- strate a discrete vascular mass; Doppler evaluation will dem-
fied incidentally in the pancreatic body or tail of a middle-­ onstrate arterial and venous flow. Since children identified
aged to older woman. However, this tumor is one of the with pancreatic infantile hemangiomas are usually symp-
many pancreatic lesions that can occur in patients with von tomatic from biliary obstruction, intra- and extrahepatic bili-
Hippel-Lindau (VHL) disease where it may be identified at a ary ductal dilation will be seen by ultrasound. In many cases,
younger age. Serous cystadenoma should be considered in these patients may undergo MRCP or CT prior to further
the differential diagnosis when a cystic pancreatic mass is treatment.
identified in a patient without a h­ istory of pancreatitis or in a In the few reported cases of pancreatic infantile heman-
child with a known diagnosis of VHL. gioma, the presenting symptom of biliary obstruction was
Although case reports of pancreatic serous cystadenoma in relieved by cholecystostomy tube placement and biliary
the pediatric population have been published, there is no cur- bypass surgery. The hemangiomas involuted over time, with
rent consensus regarding optimal imaging or management eventual complete resolution [69, 70].
strategies [62]. Histologically, these tumors are usually com-
prised of microcysts with a central stellate scar, with larger Cystic Teratoma
cysts seen at the periphery of the lesions [63, 64]. Oligocystic As with cystic teratomas elsewhere in the body, pancre-
lesions are comprised of fewer, larger cysts, and solid lesions atic cystic teratoma is a germ cell neoplasm that arises
580
a b
Fig. 14.27 Pancreatic teratoma in a 15-month-old male. (a) Transverse
grayscale ultrasound image of the mid-abdomen demonstrates a cystic
and solid mass (arrows). The organ of origin is not well-depicted. (b)
from one or more of the three germ cell layers. Pancreatic
­teratomas can be mature or immature. Mature teratomas
can present with vomiting, abdominal pain, or a palpable
mass [71].
On ultrasound, the mass is frequently cystic with internal
septations. It may contain foci of calcification or fat that can
help in confirming the diagnosis although the organ of origin
may be difficult to determine (Fig. 14.27). CT or MR imag-
ing can be useful for further characterization of tissue within
the mass and for pre-surgical planning.
Typically, these neoplasms are managed by surgical resec-
tion that preserves as much normal pancreatic ­parenchyma as
possible. Post-surgical follow-up imaging is necessary, as these
lesions can recur following treatment [71].
Lipoma
Pancreatic lipoma is usually incidentally identified in adults
on cross-sectional imaging. This tumor is rare in children,
although a few pediatric cases have been reported [72].
If identified on ultrasound, pancreatic lipoma will appear
more echogenic than the surrounding pancreatic parenchyma,
without evidence of invasion into adjacent structures [72]. MR
imaging can confirm that the lesion consists of fat, with a thin
peripheral capsule.
Most cases of pancreatic lipoma are treated conservatively.
Inflammatory Myofibroblastic Tumor
Inflammatory myofibroblastic tumor is a benign, inflammatory
neoplasm composed of fibroblasts, myofibroblasts, and inflam-
matory material [73]. Approximately one third of these tumors
arise in the lungs, with the orbit, mediastinum, and abdomen
representing other common sites of disease [73]. Patients may
present with systemic symptoms ­secondary to the underlying
inflammatory process, including fever or failure to thrive.
A. L. Hryhorczuk and H. J. Paltiel
Axial contrast-enhanced CT image of the abdomen demonstrates a cys-
tic and solid mass (arrows) replacing the pancreatic body and tail, with
foci of fluid (F), fat (arrowhead), and soft tissue
Pancreatic inflammatory myofibroblastic tumor is extremely
uncommon, with only scattered case reports in the pediatric
population [74].
On ultrasound examination, these lesions can appear as
solid or partially cystic pancreatic masses, without specific
features. In adults, nearly all inflammatory myofibroblastic
tumors of the pancreas occur in the pancreatic head, while
pediatric reports have documented these tumors in the pan-
creatic body and tail as well [74].
Biopsy is necessary for diagnosis, and surgical resection
is considered definitive treatment. Follow-up imaging is
essential in these patients, as inflammatory myofibroblastic
tumor can recur after resection and require repeated treat-
ment [75].
 eiomyoma, Neurofibroma, and Schwannoma
L
Leiomyoma, neurofibroma, and schwannoma are benign
mesenchymal tumors that rarely involve the pancreas in
either children or adults [76, 77]. On ultrasound, these
lesions may appear similar to other solid pancreatic masses,
and surgical resection is required for diagnosis.
Malignant Pancreatic Tumors
Pancreatoblastoma
Although rare, pancreatoblastoma is the malignant pancre-
atic tumor most often diagnosed in young children. The aver-
age age of presentation is 5 years, and boys are more often
affected than girls [78]. While the majority of cases are diag-
nosed in patients without an underlying condition, pancre-
atoblastoma diagnosed at 3 months of age or younger is
more closely associated with Beckwith-Wiedemann syn-
drome and hemihypertrophy [79]. Patients with pancreato-
14 Pancreas, Adrenal Glands, and Retroperitoneum
blastoma typically have elevated alpha-fetoprotein levels,
which may lead to difficulty in initially differentiating pan-
creatoblastoma from hepatoblastoma, especially if the
abdominal mass is large and closely associated with both the
pancreas and liver [80].
Pancreatoblastoma is a heterogeneous tumor, with both cys-
tic and solid components identified on ultrasound (Fig. 14.28).
Similar to neuroblastoma, pancreatoblastoma may contain
prominent calcifications that can be identified as shadowing
foci by ultrasound or CT. Pancreatoblastoma tends to present as
a large intraabdominal mass and can extend from the pancreas to
a b
Fig. 14.28 Pancreatoblastoma in a 2-year-old female with abdomi-
nal pain. Transverse (a) and longitudinal (b) grayscale ultrasound
images of the left upper quadrant demonstrate a large (10 cm) mass
(calipers) with both cystic (asterisks) and solid (arrows) components.
581
invade the lesser sac, with tumor encasing the retroperitoneal
vascular structures [81]. All of these features may contribute to
difficulty in identifying the organ of origin for pancreatoblas-
toma, and imaging features may overlap considerably with
neuroblastoma.
Surgical resection is an essential component of clinical
management. While many tumors will not be amenable to
resection at presentation, neoadjuvant chemotherapy may
allow for eventual surgical resection [80]. Although com-
plete resection may permit long-term survival, tumor recur-
rence is common, and patients require close surveillance
(c) Longitudinal color Doppler ultrasound image reveals blood flow
(arrow) within the solid component of the mass. (Images courtesy of Dr.
Arzu Kovanlikaya, New York Presbyterian Hospital, Weill Cornell Medical
College, NY, USA)
582
after initial treatment [82]. Radiation therapy is typically
used only if a tumor is unresectable, and the value of this
treatment is uncertain [80].
Solid Pseudopapillary Tumor
Solid pseudopapillary tumor of the pancreas is a rare neo-
plasm of low malignant potential. It is the most frequently
diagnosed primary pancreatic malignancy in teenagers and
young adults. Although many patients will present with a pal-
pable mass or abdominal pain, approximately 40% of these
tumors will be identified incidentally in an asymptomatic
patient [83].
On imaging, a solid pseudopapillary tumor can be identi-
fied in any portion of the pancreas. Although typically
described as cystic, or cystic with solid components, it can
also be almost entirely solid. Solid tumors are often seen in
male patients [84]. Ultrasound will demonstrate a pancreatic
mass, which can be confirmed and further characterized by
a b
c d
Fig. 14.29 Solid pseudopapillary tumor of the pancreas in a 16-year-­
old female. (a) Transverse grayscale ultrasound image of the pancreatic
tail (P) demonstrates a focal, heterogeneous mass (arrows). (b) Color
Doppler ultrasound image of the mass reveals subtle peripheral vascu-
larity (arrow), with an internal fluid-fluid level (arrowhead) and mildly
A. L. Hryhorczuk and H. J. Paltiel
other imaging modalities (Fig. 14.29). However, patient
demographics are helpful in suggesting the final diagnosis.
Since pancreatic solid pseudopapillary tumor has malig-
nant potential, surgical resection is essential for appropriate
management. 95% of patients are considered cured after
resection, and mortality from metastatic disease is only 2%
[83]. However, follow-up after resection is essential, as
patients may develop late recurrence or metastatic disease
that will require further treatment (Fig. 14.30). Currently,
follow-up to at least 5 years is recommended when monitor-
ing for tumor recurrence [83].
Islet Cell Tumor
Pancreatic islet cell tumor/neuroendocrine tumor is rare in chil-
dren and represents only 15% of primary pediatric pancreatic
tumors [85]. Most pediatric islet cell tumors are functional
masses, and children will present with symptoms secondary to
the underlying endocrine abnormality, such as hypoglycemia
increased through transmission suggestive of a partially cystic compo-
nent. (c) Axial contrast-enhanced, T1-weighted, fat-suppressed MR
image depicts subtle enhancement in the lateral aspect of the mass
(arrow). (d) Axial diffusion-weighted MR image shows restricted diffu-
sion (arrows) at the periphery of the lesion
14 Pancreas, Adrenal Glands, and Retroperitoneum
a c
Fig. 14.30 Metastatic solid pseudopapillary tumor of the pancreas in a
12-year-old female. (a) Transverse T2-weighted, fat-suppressed MR
image obtained at the time of diagnosis at 9 years of age reveals a het-
erogeneous mass (arrows) in the pancreatic tail (P). (b) Longitudinal
grayscale ultrasound image of the left hepatic lobe obtained 3 years
with an insulinoma or multiple ulcers and diarrhea with a gastri-
noma. A non-functioning islet cell tumor or multiple islet cell
tumors in the same child suggest an underlying diagnosis of
multiple endocrine neoplasia type 1 (MEN1) and should prompt
genetic evaluation [85].
On ultrasound, an islet cell tumor will appear as a solid
pancreatic mass (Fig. 14.31). On CT and MR imaging, it typi-
583
after distal pancreatectomy demonstrates a focal, echogenic nodule
(arrow). L, Liver. (c) Transverse grayscale ultrasound image of the right
hepatic lobe shows additional hypoechoic lesions (arrows). Biopsy
revealed metastatic tumor. L, Liver
cally demonstrates arterial enhancement. The diagnosis can
be confirmed with PET imaging using Gallium-68 dodecane-
tetraacetic acid (DOTA) ­octreotate, which binds to neuroen-
docrine tumors that express somatostatin receptors. A
meta-analysis of the utility of PET imaging with Gallium-68
DOTA-octreotate demonstrated a pooled sensitivity of 93%
and specificity of 95%, rendering this modality essential in
584
a b
G S
c d
Fig. 14.31 Metastatic undifferentiated neuroendocrine carcinoma of
the pancreas with liver metastasis in an 18-year-old male. (a) Transverse
grayscale ultrasound image shows a mass (arrowheads) in the pancreatic
head with an echogenicity very similar to that of the adjacent normal
pancreatic parenchyma (P). G, Gallblader; S, stomach. (b) Transverse
grayscale ultrasound image of the liver reveals a metastatic echogenic
lesion (arrow) with central necrosis in segment VIII. (c) Coronal con-
the evaluation of islet cell tumors and distant metastatic dis-
ease [86].
Since most children with islet cell tumors will have function-
ing masses, it is essential to treat the presenting clinical symp-
toms, including hypoglycemia in the setting of an insulinoma
that may be life-threatening. Surgical resection can provide a
cure in the setting of a solitary functional islet cell tumor with-
out metastatic spread. If the specific presentation suggests
MEN1, further genetic testing of the patient and family may be
of benefit to evaluate for this autosomal dominant disorder.
 cinar Cell Carcinoma
A this tumor [87, 88]. Cystic degeneration may occur, leading
Acinar cell carcinoma of the pancreas occurs in both chil-
dren and adults. In children, it represents 7% of all reported
A. L. Hryhorczuk and H. J. Paltiel
trast-enhanced, T1-weighted, fat-suppressed MR image shows the pan-
creatic head mass (white arrow) and the liver metastasis (arrowhead).
The common bile duct (black arrow) is dilated as a result of obstruction
by the pancreatic tumor. (d) Fused octreotide single-photon emission
computerized tomography (SPECT)/CT image shows abnormal uptake
by the pancreatic (white arrow) and hepatic (black arrow) tumors. A
stent (arrowhead) is present in the common bile duct
pancreatic malignancies [87]. It appears to be slightly more
common in boys than girls, with children usually presenting
at about 10 years of age [87]. Acinar cell carcinoma can be
associated with a lipase hypersecretion syndrome, where
patients present with diffuse fat necrosis and joint pain [88].
However, this unusual syndrome is rarely seen in clinical
practice. The more typical presentation is with an abdominal
mass or pain.
On imaging evaluation these tumors are usually large and
solid. Ultrasound may show a solid mass centered in the
head or tail of the pancreas, the most common locations of
to a more complex imaging appearance. In most cases, CT or
MR imaging will be obtained for further characterization.
14 Pancreas, Adrenal Glands, and Retroperitoneum
Approximately 20% of patients with pancreatic acinar
cell carcinoma will have distant metastatic disease [87].
For the majority of patients with local disease or only
regional metastases, treatment consists of surgery with or
without adjuvant chemotherapy and radiotherapy. Prognosis
in children is better than in adults, with 45% of children
reported alive and disease-free after chemotherapy [87].
Ductal Adenocarcinoma
Although ductal adenocarcinoma dominates discussions of
pancreatic malignancy in the adult population, pancreatic
adenocarcinoma is uncommon in children. A review of the
National Cancer Institute Surveillance, Epidemiology, and
End Result (SEER) database over a 30-year period identified
only seven children with pancreatic adenocarcinoma, repre-
senting 6% of all reported pancreatic malignancies [89]. As
in the adult population, pediatric pancreatic adenocarcinoma
can present with jaundice and abdominal pain caused by
obstruction of ductal structures.
Imaging findings of ductal adenocarcinoma have not
been well defined in children, but they appear to be simi-
lar to those in adults, where solid, intrapancreatic masses
hypoechoic to the adjacent parenchyma may obstruct the
pancreaticobiliary ductal system (Fig. 14.32). Although
ultrasound can demonstrate biliary and/or pancreatic duc-
tal dilation, CT or MR imaging is essential for identifying
the relationship of the tumor to major arterial and venous
structures, which will determine resectability [90].
If vascular structures are free of tumor, or adjacent to but
not involved by tumor, patients may be candidates for resec-
tion. However, assessment of vascular involvement by tumor
a b
Fig. 14.32 Pancreatic ductal adenocarcinoma in a 16-year-old male.
(a) Transverse grayscale ultrasound image shows subtle enlargement of
the pancreatic head (arrowheads) with a mildly lobulated contour. The pan-
creatic duct (arrow) is dilated. Asterisk, fluid in duodenum. (b) Coronal
585
can be difficult, requiring careful correlation with ductal ade-
nocarcinoma staging systems and clear communication with
the referring surgical team [91]. Neoadjuvant chemotherapy
can also be considered to reduce the size of a primary tumor
and potentially allow for a later pancreatic resection [90].
Lymphoma
Pancreatic involvement by lymphoma is typically seen in
children with large cell lymphoma or Burkitt lymphoma
[77]. In most cases, the pancreas is secondarily involved by
an adjacent lymph node mass or is one site of involvement in
a more diffuse neoplastic process. Children may present with
abdominal pain or pancreatitis, although they often have rap-
idly evolving systemic symptoms that include fever, fatigue,
and tumor lysis syndrome [92].
At imaging, pancreatic lymphoma can manifest as a soli-
tary mass, multiple masses, or diffuse infiltration of the
pancreas. Ultrasound will usually demonstrate one or more
hypoechoic masses within the pancreatic parenchyma
(Fig. 14.33). An extrinsic lymph node mass is sometimes
identified extending into the pancreas. When lymphoma-
tous involvement leads to pancreatitis, peripancreatic inflam-
matory changes and pseudocysts may develop.
If additional lymph node masses are identified that are
easily accessible to biopsy, direct biopsy of the pancreatic
mass is not necessary for initiation of treatment. Systemic
treatment is tailored to the underlying lymphoma.
Neuroblastoma
As a neoplasm of neural crest origin, neuroblastoma usually
arises from the adrenal medulla and sympathetic ganglion
contrast-enhanced CT image shows the low-­density mass (arrow) in the
pancreatic head. The common bile duct (arrowhead) is dilated. (Images
courtesy of Dr. M. Beth McCarville, St. Jude Children’s Research Hospital,
Memphis, TN, USA)
586
a b
Fig. 14.33 Lymphomatous involvement of the pancreas in a 10-year-­
old female with Burkitt lymphoma. (a) Transverse grayscale ultrasound
image shows a hypoechoic mass (arrows) in the pancreatic head with
dilation (arrowhead) of the distal pancreatic duct. (b) Axial T2-weighted,
cells. However, neural crest cells are involved in the forma-
tion of pancreatic ganglia and, in rare cases, can serve as the
progenitor cells for primary pancreatic neuroblastoma [93].
A primary pancreatic mass is an exceptionally rare presenta-
tion of this common pediatric tumor, with only nine reported
cases in the literature [93]. Patients may present with a pal-
pable abdominal mass or a paraneoplastic syndrome such as
opsoclonus-myoclonus [94]. As in typical cases of adrenal or
retroperitoneal neuroblastoma, urine catecholamines may be
elevated. However, with intrapancreatic neuroblastoma,
mass effect on the common bile duct can result in jaundice
and hyperbilirubinemia, which are not typically seen with
neuroblastoma.
Ultrasound imaging of the tumor may demonstrate an
intrapancreatic tumor with encasement of the common
bile duct and adjacent vessels, including the portal vein,
celiac axis, and superior mesenteric artery. Imaging fea-
tures of pancreatic neuroblastoma are similar to its appear-
ance of neuroblastoma in other primary sites [94]. CT and
MR imaging are typically used to assess the entire extent
of the primary tumor as well as adjacent lymph nodes and
retroperitoneal disease. Nuclear medicine imaging with
metaiodobenzylguanidine (MIBG) is essential to assess
for distant disease.
Treatment of pancreatic neuroblastoma is directed by
clinical protocols, such as those established by the Children’s
Oncology Group (COG). This treatment is partially dic-
tated by the stage of the patient’s primary tumor. Staging is
based on the International Neuroblastoma Risk Group
Staging System (INRGSS). This system is used to stratify a
patient’s pre-surgical risk by assessing the location and
extent of the primary tumor, as well as involvement of adja-
A. L. Hryhorczuk and H. J. Paltiel
fat-suppressed MR image of the abdomen demonstrates expansion of
the pancreatic head (white asterisk) compatible with tumor infiltration.
The pancreatic duct (arrowhead) is dilated. Ascites (black asterisks) and
splenomegaly (S) are also present
cent vital structures (image-defined risk factors). Data from
anatomic staging is combined with other factors, including
patient age, histology, and tumor genetics/biology, which
categorizes a patient as low, intermediate, or high risk [95].
Neuroblastoma is discussed in greater detail later in this
chapter.
Primitive Neuroectodermal Tumor
Primitive neuroectodermal tumor (PNET) is one of many
tumors in the Ewing sarcoma family of tumors, which includes
Ewing sarcoma, extraosseous Ewing sarcoma, and Askin
tumor [96]. Pancreatic PNET is a rare tumor, with fewer
than 30 cases reported in the literature. Patients have ranged
in age from 2 to 37 years, with an average age of 18 years
[96]. It most often arises in the pancreatic head and can pres-
ent with abdominal pain or jaundice caused by bilious
obstruction.
Ultrasound will demonstrate a solid pancreatic mass,
sometimes with associated biliary obstruction. Further eval-
uation with cross-sectional imaging is usually performed.
Given the rare nature of these tumors, a clear management
pathway has yet to be established. PNET is typically an aggres-
sive neoplasm, with only 50% patient survival at 5 years
[97]. Patients may be treated with a combination of surgical
resection and chemotherapy, but generalizable treatment rec-
ommendations are not available.
Kaposiform Hemangioendothelioma
Kaposiform hemangioendothelioma (KHE) is a rare, locally
aggressive vascular tumor, typically seen in neonates and
young infants. It most commonly occurs in the head and neck,
extremities, and trunk, although pancreatic lesions have also
14 Pancreas, Adrenal Glands, and Retroperitoneum
been reported [60, 98]. KHE can be life-­threatening due to the
commonly associated Kasabach-Merritt phenomenon, a con-
sumptive coagulopathy that leads to profound thrombocytope-
nia and the potential for catastrophic hemorrhage [98].
On ultrasound imaging, KHE may present as a discrete mass
or as a diffuse and infiltrative lesion containing vessels with
prominent arterial and venous flow [99]. Infiltrative lesions are
more commonly associated with Kasabach-­Merritt phenome-
non. On MR imaging, KHE may be iso- or h­ yperintense to mus-
cle on T1-weighted sequences, with large enhancing vessels or
vascular flow voids on T2-weighted sequences [99].
Most patients with KHE are treated with a combination of
medical therapies. Sirolimus is the medication of choice for pro-
moting regression of KHE; it can be used alone or in conjunc-
tion with steroids, vincristine, or cyclophosphamide [98, 100].
Rhabdomyosarcoma
A rhabdomyosarcoma arising from the biliary tree near the
ampulla of Vater or within the pancreatic portion of the com-
mon bile duct can present as a pancreatic mass [101]. Since
these lesions are typically associated with the biliary tree,
patients may present with obstructive jaundice, with the
underlying mass only identified by imaging.
Ultrasound may demonstrate intrahepatic biliary dilatation,
terminating in an obstructive mass within the pancreatic head.
The mass may contain mixed cystic and solid components and
a b
Fig. 14.34 Pancreatic metastasis from fibrolamellar hepatocellular car-
cinoma in a 12-year-old female. (a) Transverse grayscale ultrasound
image identifies a solid mass (asterisk) in the pancreatic head. Pancreatic
parenchyma (arrowheads) surrounds the mass with a claw-like configu-
ration. There is mild dilation (arrow) of the pancreatic duct. (b) Coronal
587
can mimic a choledochal cyst [102]. Treatment consists of sur-
gical resection of the primary tumor and chemotherapy [101].
Fibrosarcoma
Fibrosarcoma of the pancreas is exceedingly rare [77, 103].
Its appearance on imaging is variable, and a definitive diag-
nosis is made on pathological examination.
Metastatic Disease
Hematogenous metastases to the pancreas are very rare but
have been reported, most frequently from non-Hodgkin lym-
phoma and mesenchymal sarcoma [104]. Typically, metasta-
ses will be identified either by symptom progression or on
surveillance imaging (Fig. 14.34). More often, the pancreas
is secondarily involved by direct extension of an adjacent
tumor, usually neuroblastoma [105]. Pancreatic metastases
from neuroblastoma can manifest as direct extension of a ret-
roperitoneal mass into the pancreatic parenchyma, one or
more focal pancreatic masses, or diffuse enlargement and
heterogeneity of the pancreas, compatible with infiltrative
disease [105, 106]. Sarcomas can also metastasize to the
pancreas (Fig. 14.35).
These patients have a poor prognosis, as pancreatic
involvement generally occurs in the setting of widespread
disease [107]. Treatment for pancreatic metastases depends
on the underlying tumor.
T2-weighted, fat-suppressed MR image demonstrates a pancreatic mass
(white asterisk) with dilation (black asterisk) of the common bile duct. The
pancreatic duct (arrowhead) appears prominent. There is diffuse signal
abnormality throughout the left hepatic lobe (L), related to the primary
tumor
588
Fig. 14.35 Pancreatic metastasis in a 26-year-old female, initially diag-
nosed with osteosarcoma at 15 years of age. Transverse grayscale ultra-
sound image of the pancreatic tail (P) reveals a solid pancreatic mass
(arrows). Biopsy demonstrated metastatic osteosarcoma
Pancreatic Transplantation
Whole pancreas transplantation is extremely rare in chil-
dren, if not performed in the context of multivisceral trans-
plantation [108]. Typically, pancreatic transplantation is
reserved for patients with end-stage complications of type
1 diabetes, including hypoglycemic unawareness, severe
neuropathy, and chronic renal failure. As these late-stage
complications are uncommon in children, few receive pan-
creatic transplants.
Autologous islet cell transplantation is used to treat
children with chronic pancreatitis who require pancreatic
explantation for severe abdominal pain. In these cases, the
pancreas is removed and chemically digested by an enzy-
matic solution that allows for islet cell isolation [108].
Islet cells can then be introduced into the portal venous
system, where they engraft in the hepatic sinusoids [53].
Immediately following islet cell autotransplantation,
ultrasound is necessary to assess the portal vein, which has
been reported to thrombose in 3% of patients [109]. Once the
islet cells are fully engrafted, the liver may appear nodular
and echogenic (Fig. 14.36). This appearance is thought to
reflect a combination of hepatic steatosis caused by exposure
to high concentrations of insulin as well as to embolization
of the terminal portal venous branches during islet cell
implantation [110].
A. L. Hryhorczuk and H. J. Paltiel
Fig. 14.36 Appearance of the liver following pancreatectomy and islet
cell transplantation in a 19-year-old male with a history of chronic pan-
creatitis. The liver is diffusely echogenic compared to the adjacent right
kidney (K). Subtle hypoechoic nodules (arrows) are also noted within
the hepatic parenchyma
Adrenal Glands
Technique
Patient Positioning
The adrenal glands are best imaged with the patient in a
supine position or an oblique lateral position. In infants,
prone positioning is sometimes useful for visualization of
suprarenal structures.
Ultrasound Transducer Selection
In infants, use of a 9.0 to 15 MHz linear array transducer can
provide detailed images of the adrenal glands. In older chil-
dren, the highest-frequency curved array transducer that pro-
vides adequate penetration should be used, usually on the
order of 3.0 to 9.0 MHz [111].
Imaging Approaches
The liver can serve as an acoustic window for ultrasound
imaging of the right adrenal gland, while the spleen provides
an acoustic window for the left adrenal gland. The adrenal
glands have a “Y” or inverted “V” shape. The right adrenal
gland is located anterosuperior to the right kidney, posterior
to the superior vena cava, lateral to the right diaphragmatic
crus, and medial to the right hepatic lobe (Fig. 14.37), while
the left adrenal gland is located anteromedial to the left kid-
14 Pancreas, Adrenal Glands, and Retroperitoneum 589

a b

L L

Fig. 14.37 Normal right adrenal gland in a female infant. (a) medulla (asterisk) is echogenic. L, Liver; K, right kidney. (b) Transverse
Longitudinal grayscale ultrasound image of the right adrenal gland. The grayscale ultrasound image of the right adrenal gland. The gland (arrow-
adrenal cortex (arrowhead) is prominent and hypoechoic. The adrenal heads) is lateral to the inferior vena cava (IVC; asterisk). L, Liver

The adrenal medulla has a separate embryologic origin

S invade the medial aspect of the cortical tissue along its cen-
K tral vein to attain a central location (Fig. 14.40) [114].
Fig. 14.38 Normal left adrenal gland in a male infant. Longitudinal
grayscale ultrasound image demonstrates the left adrenal gland (arrows)
between the spleen (S) and left kidney (K)
ney, lateral to the aorta, lateral to the left diaphragmatic crus,
and medial to the spleen (Fig. 14.38) [112, 113].
Normal Development and Anatomy
Normal Development
The adrenal gland is comprised of a peripheral cortex and
central medulla. In the embryo, the adrenal cortex arises
from mesodermic tissue within the urogenital ridge,
medial to the developing gonads (Fig. 14.39). This close
relationship between the adrenal cortex and the gonads
explains why adrenal rests can often occur in the testes
and ovaries [13].
and is derived from neural crest tissue in a sympathetic
ganglion near the celiac plexus. At 7 weeks of embryologic
development, these cells migrate to the adrenal cortex and
Later in gestation, the adrenal medullary cell mass becomes
fully incorporated within the adrenal cortical tissue, resulting
in an arrangement where the medullary tissue is surrounded by
a peripheral cortex. The adrenal cortex subsequently differen-
tiates into three distinct zones which are fully developed by
4 years of age: the zona glomerulosa which produces aldoste-
rone, the zona fasciculata which produces corticosteroids, and
the zona reticularis which produces androgens (Fig. 14.41).
Normal Anatomy
The embryologic development of the adrenal cortex and
medulla is reflected in the distinctive layered appearance of
the normal adrenal gland in infants on ultrasound. At birth,
the hypoechoic adrenal cortex is prominent and contrasts
with the echogenic adrenal medulla. An adrenal limb should
measure no greater than 4 mm in thickness during infancy,
although the gland can maintain convex borders until approx-
imately 1 year of age [113]. Within the first several months
after birth, the adrenal cortex diminishes in size, while the
medulla enlarges. By 5–6 months of age, the adrenal gland is
homogenously echogenic. At 1 year of age, the adrenal gland
appears homogeneously hypoechoic, similar in appearance
to an adult adrenal gland [112]. Although normal adrenal
glands are readily identified by ultrasound in infants and
young children, they are generally difficult to identify in
older patients unless they demonstrate pathologic changes.
590 A. L. Hryhorczuk and H. J. Paltiel

7 week fetus

Mesonephros
Urogenital ridge
Gonadal ridge

Root of bowel
mesentery
Spine

Developing adrenal
cortex

Urogenital
ridge

Sympathetic
ganglion
Adrenal
medulla

Adrenal
cortex

Developing
gonads

Fig. 14.39 Diagram of early adrenal gland development. Adrenal corti- ral crest. By 7 weeks of life, the medullary tissue migrates to the adre-
cal tissue arises in the urogenital ridge, in close association with the nal cortex and begins to invade the cortical cells. The medullary cell
developing gonads. Medullary tissue has a separate origin from the neu- mass is not fully enveloped by the cortex until later in gestation

Primordium
of cortex
Medulla

Fetal
zone Medulla

Zona
Fetal reticularis
medulla
Zona
fasciculata

Zona
glomerulosa

7 weeks Newborn 4 years

Fig. 14.40 Diagram of later adrenal gland development. At 7 weeks of However, cortical differentiation into a zona glomerulosa, zona fascicu-
life, the primitive adrenal medullary tissue begins to invade the cortex. lata, and zona reticularis is not complete until later in childhood
At birth, the medullary cell mass is fully surrounded by the cortex.

Anatomic Variants On ultrasound, a discoid adrenal gland will appear elon-

gated and flattened, with absence of the ipsilateral kidney
 iscoid Adrenal Gland
D (Fig. 14.42). Care should be taken to differentiate the adjacent
In the absence of an ipsilateral kidney, the adrenal gland can diaphragmatic crus from the adrenal gland, as it can simulate
assume a discoid configuration where it appears flat or elon- a normal adrenal gland limb [115]. A normally shaped adrenal
gated. It has been theorized that the adjacent kidney is essen- gland in the setting of an absent kidney suggests renal dyspla-
tial for normal adrenal morphology, exerting upward pressure sia and involution, since the adrenal gland was presumably
on the gland during development [114]. molded by a kidney that is no longer present.
14 Pancreas, Adrenal Glands, and Retroperitoneum 591

A discoid adrenal gland requires no specific treatment.

However, as there will be either a solitary or ectopic kidney,
Capsule
further assessment of renal anatomy and function may be
indicated.
Zona
glomerulosa

Congenital Anomalies

Zona  ongenital Adrenal Agenesis

C
fasciculata Congenital adrenal agenesis is an extremely rare disorder
that usually occurs in the setting of multiple complex con-
genital anomalies [116, 117]. Patients will have a defi-
Zona ciency of cortisol and aldosterone (normally synthesized in
reticularis the adrenal cortex), as well as catecholamines (normally
Medulla
synthesized in the adrenal medulla). Rapid postnatal diag-
nosis of this abnormality can be lifesaving. Ultrasound will
reveal absence of the adrenal glands. Definitive diagnosis
depends on documentation of low levels of serum cortisol,
critically low blood glucose, and low aldosterone [118].
Affected infants may demonstrate hyperpigmentation.
Rapid hormonal and salt replacement is essential to sur-
vival [119].
Capsule

Adrenal
cortex Fusion Abnormalities
Adrenal
medulla
 ircumrenal Adrenal Gland
C
A circumrenal adrenal gland is a rare congenital abnormality
where the limbs of the adrenal gland encircle the kidney with
Fig. 14.41 Diagram of normal adrenal gland anatomy a ring of adrenal tissue. In the few reported cases of this

a b

S
S

Fig. 14.42 Discoid left adrenal gland in a male infant with no left kid- (arrows). Loops of gas-filled bowel (asterisks) are identified in the
ney. Longitudinal (a) and transverse (b) grayscale ultrasound images of expected location of the left kidney. S, Spleen
the left renal fossa demonstrate a flattened, elongated adrenal gland
592
entity, no additional congenital abnormalities have been
associated with this adrenal configuration, and no further
management is required [120].
 orseshoe Adrenal Gland
H adrenocorticotropic hormone prevents the usual involution of
A horseshoe adrenal gland extends across the midline,
anterior to the aorta (Fig. 14.43). It is associated with a
variety of congenital anomalies, included asplenia, neural
tube defects, and renal abnormalities, including horseshoe
kidney and renal agenesis [113, 121]. These associations
are likely ­secondary to an abnormality in the development
of the midline structures, resulting in abnormal fusion of
the adrenal glands as well as the other reported congenital
abnormalities [121].
When a horseshoe adrenal gland is identified, further
ultrasound evaluation is necessary to identify any coexisting
renal abnormalities and to determine the presence and loca-
tion of the spleen. Assessment for possible cardiac abnor-
malities and neural tube defects should also be performed.
Although a horseshoe adrenal gland does not require fur-
ther management, the associated abnormalities may require
further investigation and will ultimately determine the
patient’s clinical course [121].
Adrenal Rests
Adrenal rests represent small fragments of adrenal cortical tis-
sue that are trapped in an ectopic location during development.
L L
Fig. 14.43 Horseshoe adrenal gland in an infant girl with heterotaxy and
asplenia. Transverse grayscale ultrasound image demonstrates a large,
midline liver (L). The left and right adrenal glands (arrows) are fused,
extending across the midline and draped anteriorly over the aorta (A)
A. L. Hryhorczuk and H. J. Paltiel
This tissue may be present in up to 50% of neonates, but typi-
cally atrophies rapidly [114]. Adrenal rests are clinically sig-
nificant when encountered in the testes of boys with congenital
adrenal hyperplasia. In these patients, continued exposure to
the rests. Instead, they grow over time, developing into dis-
crete testicular masses that can be palpated on physical exami-
nation and are visible by ultrasound (Fig. 14.44).
These masses are not usually identified in patients less than
5 years of age and grow over time. They can either grow rapidly,
resolve over time, or remain stable [122]. Enlargement or
decrease in size of these masses is not always clearly related to
control of the patient’s underlying disease; in some reported
cases, patients with multiple tumors had growth of one tumor
with regression of a second [122]. In general, however, these
tumors are seen in patients with poorly controlled disease and
higher steroid requirements [123].
Although intratesticular adrenal rests are benign lesions, they
can compress the testicular parenchyma, leading to impaired
spermatogenesis as well as diminished testosterone production.
In adults, this can lead to infertility. To prevent this outcome,
boys with congenital adrenal hyperplasia should be screened for
adrenal rests beginning at 4–6 years of age [123]. Treatment
includes increased corticosteroid treatment to control the under-
lying disease. In order to preserve future fertility, teenaged
patients should be offered semen cryopreservation [124]. This
entity is discussed further in Chap. 15.
Fig. 14.44 Testicular adrenal rests in an 18-year-old male with con-
genital adrenal hyperplasia. Longitudinal grayscale ultrasound image
reveals multiple intratesticular masses (arrowheads)
14 Pancreas, Adrenal Glands, and Retroperitoneum
Adrenal Heterotopia
Heterotopic adrenal tissue has been reported in multiple
locations, including the kidney, where it may mimic a renal
mass, the fallopian tube, and the groin [125–127]. Most
cases of adrenal heterotopia are identified in adults, when a
small amount of adrenal tissue is incidentally found on
pathology examination of a surgical specimen. Heterotopic
adrenal tissue is not generally identified preoperatively. No
specific treatment is required [126].
Genetic Disorders
 ongenital Adrenal Hyperplasia
C cally seen in congenital adrenal hyperplasia, while patients
Congenital adrenal hyperplasia (CAH) is a group of inherited
autosomal recessive disorders, most often caused by
21-hydroxylase deficiency, that affect both males and females
and may be mild or severe [128]. The severe form usually
presents in the neonatal period or early childhood, while the
mild form can become symptomatic anywhere from infancy to
adulthood. In the severe form of the disease, affected girls will
present with ambiguous genitalia, while boys present with
precocious puberty. Most patients may develop salt wasting in
the first weeks of life, resulting in hypokalemia, hyponatre-
mia, and hypotension that can be life-threatening.
a b
Fig. 14.45 Congenital adrenal hyperplasia in a 46,XX female infant
with ambiguous genitalia. (a) Transverse grayscale ultrasound image
shows an enlarged right adrenal gland (between arrowheads) with a
593
On ultrasound, adrenal hyperplasia should be suspected if
an adrenal limb is greater than 4 mm in thickness and the
gland is more than 2 cm in length (Fig. 14.45) [129].
Following treatment with steroid replacement, the appear-
ance of the adrenal glands should normalize [130]. Please
see Chap. 16 for a fuller discussion of this disorder in
females. Adrenal rests in the setting of CAH is discussed ear-
lier in this chapter.
 ongenital Lipoid Adrenal Hyperplasia
C
Congenital lipoid adrenal hyperplasia is typically caused by
mutations in the steroidogenic acute regulatory protein gene,
which leads to failed production of both adrenal and gonadal
steroids. All patients will present with the salt wasting typi-
with a 46,XY karyotype will appear phenotypically female
secondary to failure of testicular steroid synthesis [131].
Congenital lipoid adrenal hyperplasia is most commonly
seen in Korean and Japanese populations [131].
Fat deposition occurs within the adrenal cortex, with spar-
ing of the medulla. Ultrasound demonstrates significantly
enlarged adrenal glands that do not improve in appearance
after steroid replacement therapy. This is because the adrenal
cortical lipid deposition does not resolve after treatment
[132, 133]. Patients will require long-term hormone replace-
ment therapy.
cerebriform appearance. The left adrenal gland had a similar appear-
ance (not shown). (b) Longitudinal grayscale ultrasound image of the
pelvis reveals a normal uterus (arrows). B, Bladder
594
Wolman Disease
Wolman disease is a congenital lysosomal acid lipase deficiency
that presents in the neonatal period with hepatosplenomegaly
and failure to thrive. As fatty acids accumulate within the adre-
nal glands, they become enlarged and calcified as a result of
saponification of the deposited adrenal fat [134, 135].
Ultrasound may demonstrate enlarged adrenal glands
with prominent echogenic foci associated with posterior
acoustic shadowing. There will also be hepatosplenomeg-
aly. These findings have also been reported on prenatal
ultrasound studies.
Wolman disease has a poor prognosis, with few patients
surviving beyond the first year of life. Care is typically sup-
portive and involves treatment of adrenal insufficiency and
liver failure [134].
a b
Fig. 14.46 Adrenal cyst in a 5-year-old female with an intraabdominal
lymphatic malformation. (a) Longitudinal grayscale ultrasound image
of the right upper quadrant of the abdomen reveals an anechoic supra-
renal mass (asterisk) with increased through transmission, compatible
with a simple cyst. L, Liver; K, right kidney. (b) Coronal T2- weighted,
A. L. Hryhorczuk and H. J. Paltiel
Adrenal Cyst
I diopathic Adrenal Cyst
Adrenal cysts are usually classified into four types: pseudo-
cyst, epithelial, endothelial, or parasitic (the latter usually in
the setting of echinococcal infection) [113, 136]. An idiopathic
adrenal cyst is an incidental finding, with symptoms only
occurring in rare cases where enlargement causes mass effect.
By ultrasound, an adrenal cyst appears as an anechoic,
avascular mass with increased through transmission and a
thin wall (Fig. 14.46). It will demonstrate fluid attenuation
on CT and follow fluid signal on MR imaging. An idiopathic
simple adrenal cyst does not require intervention. However,
if there are complex internal features, the possibility of a cys-
tic neoplasm should be considered [136].
fat-suppressed MR image of the abdomen and pelvis demonstrates fluid
signal within the lesion (asterisk). A portion of the extensive intraab-
dominal lymphatic malformation (arrows) is seen within the lower
abdomen and pelvis
14 Pancreas, Adrenal Glands, and Retroperitoneum
Infection
 ongenital Herpes Simplex Infection
C tiocytes and foamy lipid-laden macrophages and is transformed
Infection with herpes simplex virus (HSV) is typically
acquired during passage through the birth canal from viral
shedding or exposure to active maternal herpetic lesions,
although infection can develop in utero as well. Adrenal calci-
fication is a rare manifestation of both congenital and acquired
HSV infection [137, 138]. Ultrasound imaging will demon-
strate echogenic, shadowing foci within the adrenal glands.
Patients are treated with high-dose parenteral acyclovir, an
antiviral medication, and supportive care.
Granulomatous Infection
Granulomatous diseases, including tuberculosis and histoplas-
mosis, and atypical mycobacteria such as mycobacterium
avium-intracellulare in immunocompromised patients, can
involve the adrenal glands. In the early stages of disease, the
adrenal glands may be diffusely enlarged, with central necrosis
(Fig. 14.47). After long-standing infection, the glands can cal-
cify, which can portend adrenal gland insufficiency and second-
ary Addison’s disease [139]. Dense calcifications can be seen
with ultrasound, and may also be evident on plain radiographs.
Treatment consists of antibiotics for the underlying infection. If
there is adrenal insufficiency, long-term replacement corticoste-
roids and mineralocorticoids will be required as well.
Xanthogranulomatous Adrenalitis
Retroperitoneal xanthogranulomatous disease typically affects
the kidneys as xanthogranulomatous pyelonephritis. However,
Fig. 14.47 Disseminated mycobacterium avium-intracellulare in a
24-year-old male. (a) Longitudinal prone grayscale ultrasound image
shows an enlarged, lobulated right adrenal gland (asterisk). L, Liver.
595
xanthogranulomatous disease can involve other organs, includ-
ing the adrenal glands. The adrenal gland is infiltrated with his-
into an inflammatory mass. An underlying infection may trigger
this process, although the specific inciting pathogen may be dif-
ficult to identify. This disorder is much less common than xan-
thogranulomatous pyelonephritis, with only a few reported
cases in the literature [140, 141].
Ultrasound will show a complex adrenal mass with both
solid and avascular components. These masses may appear
aggressive and infiltrative in appearance, with poor defini-
tion of margins. In children, it is difficult to differentiate xan-
thogranulomatous adrenalitis from a malignancy, including
neuroblastoma, and tissue sampling is required for diagno-
sis. Surgical resection is essential for management, as this
disorder will not resolve with medical treatment.
Abscess
Adrenal abscess in children is rare. It usually occurs in neo-
nates after bacterial seeding of a preexisting adrenal hem-
orrhage [142]. It can be difficult to distinguish between an
infected adrenal hemorrhage and a cystic neuroblastoma,
although symptoms of active infection and the absence of
elevated serum catecholamine levels may point to the cor-
rect diagnosis [143].
Ultrasound will show a focal adrenal mass with a thick
wall and internal heterogeneity. The mass may demonstrate a
thick wall, with increased peripheral vascularity. As noted
above, the appearance of an adrenal abscess can overlap with
that of a cystic neuroblastoma.
The left adrenal gland had a similar appearance (not shown). (b) Axial
contrast-enhanced CT image shows markedly enlarged, lobulated adre-
nal glands (arrows)
596
An adrenal abscess requires drainage. Successful percuta-
neous drainage under ultrasound guidance and open surgical
debridement are two acceptable management approaches.
Antibiotics are also required for successful treatment.
Adrenal Hemorrhage
Neonatal adrenal hemorrhage occurs in approximately 0.3%
of births [144]. Hemorrhage is more common following vag-
inal delivery, in macrosomic infants, and in infants with fetal
or neonatal acidemia [144]. Infants may present with anemia
a b
Fig. 14.48 Adrenal hemorrhage in a female neonate with sepsis. (a)
Transverse grayscale ultrasound image shows a complex cystic struc-
ture (asterisk) with internal debris in the expected location of the right
adrenal gland. (b) Transverse color Doppler ultrasound image of the
A. L. Hryhorczuk and H. J. Paltiel
or indirect hyperbilirubinemia as a result of blood loss and
degradation of blood products. Many adrenal hemorrhages
are discovered incidentally, raising concern for possible neu-
roblastoma. A rare presentation in male infants consists of
scrotal swelling and discoloration from blood extending
through a patent processus vaginalis into the scrotum [145].
On ultrasound, an adrenal hemorrhage will be identified
as a focal adrenal mass that may be mildly echogenic or
isoechoic when compared to the normal adrenal gland. No
internal vascularity should be identified within the mass
(Fig. 14.48). The hematoma may involve only one limb of
the adrenal gland, with an adjacent normal adrenal limb
mass reveals no internal vascularity. (c) Transverse grayscale ultra-
sound image of the right adrenal gland obtained 6 weeks later docu-
ments a significant decrease in size of the lesion (asterisk). A normal
portion of the adjacent adrenal gland (arrow) is identified. L, Liver
14 Pancreas, Adrenal Glands, and Retroperitoneum
identified [113]. Since the primary clinical concern is dif-
ferentiation of an adrenal hemorrhage from a congenital neu-
roblastoma, follow-up imaging is essential and should
demonstrate increasing liquefaction of the hematoma, with
an increasingly hypoechoic appearance and progressive
decrease in size.
Recent investigations have shown that adrenal hemorrhage
typically resolves within 3 months, with no residual mass.
Adrenal calcification may develop in approximately 30% of
patients [146]. In contrast, a congenital neuroblastoma will
either remain stable in size or enlarge and demonstrate internal
vascularity on Doppler imaging. Neonatal adrenal hemor-
rhage is typically managed expectantly. Ultrasound is used to
follow the hemorrhage to resolution.
In older children, adrenal hemorrhage can occasionally
occur in the setting of blunt abdominal trauma and is usually
diagnosed by CT. Hemorrhage typically involves the right
adrenal gland and may be associated with injuries of the liver
or right kidney. Ultrasound is useful in follow-up [147].
Adrenocortical Tumors
 drenocortical Adenoma and Carcinoma
A in patients with larger tumors, positive tumor margins at
Adrenocortical adenoma and carcinoma are rare in children.
Approximately 25 new pediatric adrenal cortical neoplasms
are diagnosed each year in the United States, with the major-
ity being carcinomas. There is a bimodal age distribution,
with almost half presenting in children under the age of
3 years and approximately 40% presenting after the age of
a b
RK
Fig. 14.49 Adrenocortical adenoma in a 4-month-old male with
Beckwith-Wiedemann syndrome. (a) Transverse prone grayscale ultra-
sound image shows a small, hypoechoic mass (arrow) in the right adrenal
gland. RK, Right kidney. (b) Transverse contrast-enhanced, T1-weighted,
597
10 years [148, 149]. Patients may have a personal or family
history of Li-Fraumeni syndrome or Beckwith-Wiedemann
syndrome.
In the absence of clear metastatic disease, both clinical and
imaging findings may fail to distinguish an adrenocortical
adenoma from an adrenocortical carcinoma. 80% of children
with an adrenal cortical tumor have signs of adrenocortical
hormone excess, including precocious puberty with viriliza-
tion or Cushing syndrome [148]. These hormonal abnormali-
ties are common in patients with both benign and malignant
lesions.
Imaging features of adrenocortical adenoma and carci-
noma significantly overlap. In all cases, ultrasound will
depict a focal adrenal tumor (Fig. 14.49). An adrenocortical
carcinoma is typically larger, more heterogeneous, and more
likely to contain calcification than an adenoma (Figs. 14.50
and 14.51) [150]. Patients will typically require CT or MR
imaging to assess for metastatic disease.
Biopsy with tissue diagnosis of the tumor is imperative
for further treatment. Hormonally active adrenal adenomas
are resected which resolves the symptoms associated with
excess adrenocortical hormone. Treatment for adrenocorti-
cal carcinoma depends on tumor stage. Mortality is higher
surgery, and invasion of adjacent structures [149]. Patients
with metastatic disease and those diagnosed after 4 years of
age also have poorer outcomes. Overall 5-year survival for
patients 0–4 years of age is about 91%, while 5-year sur-
vival for patients 5–19 years of age drops to approximately
30% [151].
fat-suppressed MR image demonstrates a non-enhancing lesion (arrow) in
the center of the right adrenal gland. An adrenocortical adenoma was diag-
nosed following adrenalectomy
598 A. L. Hryhorczuk and H. J. Paltiel

Fig. 14.50 Adrenocortical carcinoma in a 3-year-old female with viriliza- Doppler ultrasound image demonstrates a small amount of internal vascular-
tion. (a) Longitudinal grayscale ultrasound image shows a large adrenal ity. (d) Axial contrast-enhanced CT image reveals punctate peripheral calcifi-
mass (asterisk). K, Right kidney. (b) Transverse grayscale ultrasound image cations (arrowheads). The mass compresses the adjacent IVC (asterisk)
reveals heterogeneity of the lesion (between arrows). (c) Longitudinal color

Neural Crest Tumors

L Ganglioneuroma, ganglioneuroblastoma, and neuroblastoma all

develop from neural crest cells. In the adrenal gland, they arise
from the medulla. These tumors exist on a spectrum, with gan-
glioneuroma comprised of mature ganglion cells, ganglioneuro-
K blastoma containing both differentiated and undifferentiated
cells, and neuroblastoma consisting of neuroblasts. The propor-
tion of immature elements within a tumor directs treatment. The
more mature tumors tend to occur in older children, while neu-
roblastoma, the least mature tumor type, is diagnosed at a
median age of 17 months [152].

Ganglioneuroma
Ganglioneuroma commonly occurs in the posterior medias-
Fig. 14.51 Adrenocortical cortical carcinoma in a 1-year-old male
with precocious puberty. Longitudinal grayscale ultrasound image dem-
tinum and retroperitoneum, with approximately 20% arising
onstrates a large adrenal mass (asterisk). L, Liver; K, right kidney in the adrenal gland. Since this tumor contains mature cells
14 Pancreas, Adrenal Glands, and Retroperitoneum
and is slow growing, it is typically identified incidentally and
can pose a management dilemma in an otherwise asymptom-
atic child [113].
On ultrasound, an adrenal ganglioneuroma is typically well-
circumscribed and hypoechoic with focal calcification, although
larger lesions can appear more heterogeneous (Fig. 14.52)
[113]. As the appearance of this mass overlaps significantly
with other adrenal lesions, further laboratory evaluation is
essential to assess for abnormal catecholamine or adrenocortical
hormone production. Radiotracer uptake can be variable at
MIBG imaging, and the study can be interpreted as falsely posi-
tive for neuroblastoma [153].
a b
Fig. 14.52 Adrenal ganglioneuroma discovered incidentally in a
10-year-old male with pneumonia receiving extracorporeal membrane
oxygenation (ECMO). (a) Lateral radiograph of the chest and upper
abdomen demonstrates anterior displacement (arrows) of the venove-
nous ECMO cannula which normally should extend directly through
the right atrium to the IVC. (b) Longitudinal grayscale ultrasound
599
Ganglioneuromas are typically resected, as there is a the-
oretical concern that they might dedifferentiate into an
immature tumor with features of neuroblastoma. However,
recent investigations have demonstrated that surgical resec-
tion of ganglioneuromas carries significant morbidity. In
instances where the mass is intimately related to vital struc-
tures, a watchful waiting approach may be reasonable [154].
Ganglioneuroblastoma
Ganglioneuroblastoma is a tumor where both differentiated
and immature neural tissues are present. At pathology, this
tumor can be very heterogeneous, and it is important to sam-
image shows a homogenous, hypoechoic adrenal mass (asterisk). There
is a small, anechoic pleural effusion (arrow). Short linear, echogenic air
bronchograms (arrowheads) are identified within the consolidated right
lung. L, Liver. (c) Axial T2-weighted, fat-suppressed MR image reveals
a hyperintense adrenal mass (asterisk) with anterior displacement and
compression (arrowheads) of the IVC
600
ple multiple sites in order to identify the genetic and histo-
pathologic features that will predict tumor behavior.
On ultrasound evaluation, an adrenal ganglioneuroblastoma
is often echogenic with zones of hemorrhage or necrosis [155].
Calcifications are frequently identified and are larger and coarser
a b
Fig. 14.53 Adrenal ganglioneuroblastoma in a 13-year-old female. (a)
Axial contrast-enhanced CT image depicts a right adrenal mass (asterisk)
containing punctate calcifications (arrowhead). (b) Longitudinal gray-
scale ultrasound image demonstrates an elongated, echogenic mass
(arrows) with a mildly lobulated contour arising from the adrenal medulla.
A. L. Hryhorczuk and H. J. Paltiel
than the calcifications typically seen in ganglioneuroma
(Fig. 14.53). Tumor assessment with CT or MR imaging is nec-
essary to determine image-defined risk factors, as described
below. Treatment strategies usually mirror those used for neuro-
blastoma, also discussed in the next section of this chapter [156].
The mass contains multiple central, punctate, echogenic foci. K, Right
kidney; L, liver. (c) Transverse color Doppler ultrasound image shows a
prominent twinkling artifact (arrowhead) within the central portion of the
adrenal mass compatible with c­ alcification, L, Liver
14 Pancreas, Adrenal Glands, and Retroperitoneum 601

Neuroblastoma Table 14.2 International Neuroblastoma Risk Group Staging

Neuroblastoma is the most common pediatric extracranial System (INRGSS)
solid tumor, with approximately 650 new cases per year in Stage Description
North America [157]. The myriad presentations and varying L1 Localized tumor; in one body compartment; no image-defined
risk factors
outcomes in patients with neuroblastoma have long troubled
L2 Local/regional tumor with image-defined risk factors
clinicians: infants can present with solitary masses or wide- M Any tumor with distant metastatic disease
spread disease that spontaneously resolve, while older chil- MS Infant less than 18 months of age with metastases only to the
dren may have unresectable primary masses and extensive skin, liver, bone marrow (under 10% malignant cells)
metastatic disease that responds poorly to maximal therapy From Monclair T, Brodeur GM, Ambros PF, Brisse HJ, Cecchetto G,
[156]. Recent investigations into the tumor biology and Holmes K, et al. The International Neuroblastoma Risk Group (INRG)
genetic features of neuroblastoma have provided some staging system: an INRG task force report. J Clin Oncol. 2009;
27(2):298–303. [159]
insight into the risk factors that may predict aggressive clini-
cal behavior in a particular patient. Although neuroblastoma
can arise at any site in the sympathetic nervous system,
approximately 50% occur in the adrenal gland [158]. Table 14.3 Image-defined risk factors from the International
Neuroblastoma Risk Group
Imaging is central to neuroblastoma staging. In the past
this was done using the International Neuroblastoma Staging Anatomic region Description
System, which was designed for post-surgical staging Multiple body Ipsilateral tumor passing between 2 body
compartments compartments:
(Table 14.1) [158]. However, the need for a pre-surgical stag- Neck-chest
ing system led to the introduction of the International OR
Neuroblastoma Risk Group Staging System (INRGSS) in Chest-abdomen
2009 (Table 14.2), where staging is based on the presence or OR
Abdomen-pelvis
absence of 20 image-defined risk factors that span multiple Neck Encasement of 1 or more of the following
organ systems and are determined prior to surgery or other structures:
therapy to predict surgical outcomes and provide risk strati- Carotid artery, vertebral artery, internal
fication (Table 14.3) [159, 160]. jugular vein
Tumor extending to skull base
Although neuroblastoma is often initially evaluated with Tumor compressing trachea
ultrasound, CT or MR imaging is used to improve assessment Cervicothoracic Encasement of 1 or more of the following
of the image-defined risk factors of the INRGSS. junction structures:
Fetal neuroblastoma can be detected on prenatal ultra- Brachial plexus roots, subclavian vessels,
carotid artery, vertebral artery
sound imaging in the third trimester, where the differential
Tumor compressing trachea
diagnosis will include adrenal hemorrhage and an infradia- Thorax Encasement of aorta or branches
phragmatic extralobar sequestration. Fetal MR imaging and Tumor compressing trachea or central
bronchi
Tumor in lower mediastinum infiltrating
Table 14.1 International Neuroblastoma Staging System costovertebral junction from T9-T12
Thoracoabdominal Encasement of aorta or IVC
Stage Description junction
Stage 1 Localized tumor; complete gross excision; +/− Abdomen and pelvis Encasement of one or more of the following
microscopic residual disease; negative local lymph nodes vascular structures:
Stage 2A Localized tumor; incomplete gross excision; negative Celiac axis origin, superior mesenteric artery
local lymph nodes origin, superior mesenteric arterial
Stage 2B Localized tumor; either complete or incomplete gross branches in the mesenteric root, aorta,
excision; positive local lymph nodes; negative IVC, iliac vessels
contralateral lymph nodes Tumor infiltration of porta hepatis or
Stage 3 Unresectable unilateral tumor crossing midline; hepatoduodenal ligament
+/− regional lymph nodes Tumor invasion of renal pedicle
OR Pelvis tumor extending through sciatic notch
Unilateral tumor with positive contralateral regional Intraspinal tumor Intraspinal tumor extension with one of the
lymph nodes extension following:
OR 1/3 of canal in axial plane is filled
Midline tumor with bilateral infiltration or bilateral lymph Obliteration of perimedullary
node involvement leptomeningeal spaces
Stage 4 Any primary tumor with distant metastatic disease, including Abnormal cord signal intensity
bone marrow, bone, lymph nodes, liver, skin, or other sites Infiltration of adjacent Mass directly invades the pericardium,
Stage 4s Infant less than 1 year of age with: organs and structures diaphragm, kidney, liver,
Localized primary tumor (stage 1, 2A, 2B) duodenopancreatic block, or mesentery
Metastasis limited to the skin, liver, and bone marrow IVC, Inferior vena cava. From Brisse HJ, McCarville MB, Granata C, Krug
(under 10% malignant cells) KB, Wootton-Gorges SL, Kanegawa K, et al. Guidelines for imaging and
From Mueller S, Matthay KK. Neuroblastoma: biology and staging. Curr staging of neuroblastic tumors: consensus report from the International
Oncol Rep. 2009;11(6):431–8. [158] Neuroblastoma Risk Group Project. Radiology. 2011;261(1):243–57. [160]
602
postnatal imaging are helpful in differentiating between
these different disorders [161].
Ultrasound is the initial imaging examination in most patients
with suspected neuroblastoma. In the neonate, a variety of pre-
sentations is possible, including a solitary solid or cystic adrenal
mass (Figs. 14.54 and 14.55); bilateral adrenal masses; or diffuse
a b
Fig. 14.54 Adrenal neuroblastoma in a female neonate. (a) Longitudinal
grayscale ultrasound image shows a large adrenal mass (asterisk) com-
pressing and displacing the right kidney (K) inferiorly. (b) Longitudinal
color Doppler ultrasound image demonstrates blood flow (arrowhead)
A. L. Hryhorczuk and H. J. Paltiel
liver involvement indicative of metastatic disease (Fig. 14.56).
Ultrasound can be used to demonstrate certain image-defined
risk factors, including vascular encasement and involvement of
the renal hilum. I-123 MIGB nuclear medicine evaluation should
be obtained to assess for metastatic disease (Fig. 14.57).
within the mass. (c) Longitudinal grayscale ultrasound image obtained at
1 year of age documents complete resolution of the tumor. K, Right kidney;
L, liver
14 Pancreas, Adrenal Glands, and Retroperitoneum 603

a b

Fig. 14.55 Adrenal neuroblastoma in a male neonate. (a) Transverse
grayscale ultrasound image of the right adrenal gland reveals an echo-
genic soft tissue mass (asterisk) containing rounded hypoechoic foci.
L, Liver. (b) Posterior 123I- metaiodobenzylguanidine (MIBG) planar
image of the body to the level of the upper thighs shows radiotracer
uptake (arrowhead) in the right adrenal gland region compatible with
neuroblastoma. (c) Longitudinal grayscale ultrasound image obtained
13 months later reveals persistence of the mass (asterisk). Surgical exci-
sion was eventually performed
604
a b
c d
Fig. 14.56 Stage MS/4S neuroblastoma in a 6-week-old female with
non-­bilious projectile vomiting. (a) Transverse grayscale ultrasound
image demonstrates elongation and thickening of the pyloric muscle
(asterisk) compatible with pyloric stenosis. Incidentally noted innumer-
able hypoechoic lesions (arrowheads) throughout the liver concerning
for metastatic disease. (b) Longitudinal grayscale ultrasound image of
the left upper quadrant shows a large adrenal mass (arrows) with inter-
A. L. Hryhorczuk and H. J. Paltiel
nal heterogeneity and punctate echogenic foci (arrowhead) compatible
with calcification. S, Spleen. (c) Transverse grayscale ultrasound image
of the liver obtained 2 weeks later reveals an increased number of
parenchymal lesions (arrowheads). (d) Coronal T2-weighted, fat-sup-
pressed MR image documents innumerable hyperintense liver masses
(arrowheads) and an adrenal mass (asterisk)
14 Pancreas, Adrenal Glands, and Retroperitoneum 605

a b
L

Fig. 14.57 Stage 4/M neuroblastoma in a 2-year-old female. (a) Longi­
tudinal grayscale ultrasound image demonstrates a large, heterogeneous
adrenal mass (asterisk) that extends into the right kidney (K). (b)
Transverse grayscale ultrasound image of the mass (asterisk) demon-
strates anterior displacement and marked compression (arrowhead) of
the IVC with near-complete obliteration of the lumen. The mass extends
close to the aorta (A), and focally invades the liver (arrow). L, Liver. (c)
Posterior 123I-MIBG planar image of the chest, abdomen, pelvis, and
hips reveals radiotracer uptake within the right flank (arrow) as well as
diffuse uptake (arrowheads) throughout the spine, pelvis, and proximal
femurs compatible with metastatic disease
606
Treatment for neuroblastoma is varied and depends on the
child’s risk of disease progression. Risk stratification is per-
formed by assessing patient age, INRG stage, tumor grade and
histology, as well as tumor genetic factors including DNA
ploidy, NMYC amplification, and q11 chromosome abnormali-
ties [162]. Children are then placed into high-risk and non-high-
risk groups. Children who are not at high risk may undergo
observation or lower dose, shorter course chemotherapy. High-
risk patients may receive a combination of high-dose induction
chemotherapy followed by surgical resection, myeloablative
chemotherapy with stem cell rescue, and MIBG therapy [162].
Even with maximal therapy, neuroblastoma contributes
significantly to pediatric cancer mortality, representing only
8% of pediatric cancers but resulting in 15% of pediatric can-
cer deaths [162]. Innovative therapeutic strategies are being
introduced, including T-cell therapies and personalized genomic
medicine, and may eventually contribute to improved long-
term outcomes for children with neuroblastoma.
Pheochromocytoma
Pheochromocytoma is a catecholamine-secreting tumor that
originates in the adrenal medullary tissues. It is closely related
to paragangliomas that originate from extra-adrenal portions
a b
L
Fig. 14.58 Right adrenal pheochromocytoma in a 9-year-old male with
hypertension, headache, and palpitations. (a) Longitudinal grayscale
ultrasound image shows an adrenal mass (asterisk) with an adjacent
A. L. Hryhorczuk and H. J. Paltiel
of the sympathetic and parasympathetic nervous systems
[163]. Pediatric pheochromocytoma is associated with several
hereditary syndromes, including multiple endocrine neoplasia
type 2, neurofibromatosis type 1, von Hippel-­Lindau dis-
ease, and hereditary paraganglioma-pheochromocytoma
syndrome. The number of pheochromocytomas that arise in the
setting of these genetic syndromes is uncertain, although it is
thought that up to 80% of children with pheochromocytoma may
have an underlying genetic predisposition, far outnumbering the
children with sporadic tumors [164].
Clinically, most children present with hypertension and head-
aches; about half report flushing, sweating, or pallor [163]. In
some cases, pheochromocytoma will be identified in an asymp-
tomatic child with a known syndrome undergoing screening
evaluation.
On ultrasound, a pheochromocytoma appears as a solid
adrenal mass with an echotexture similar to or hypoechoic to
the liver (Fig. 14.58) [113]. Echotexture of the mass can be
variable, especially in larger tumors containing foci of inter-
nal necrosis and hemorrhage. Doppler i­maging may show
internal vascularity [136]. Pheochromocytoma can be bilat-
eral in up to 40% of cases (Fig. 14.59). MR imaging provides
further information regarding the extent of the mass and is
also useful in assessing the contralateral adrenal gland.
Typical MR imaging findings of pheochromocytoma include
L
normal adrenal limb (arrowhead). L, Liver. (b) Transverse grayscale
ultrasound image reveals compression of the adjacent liver (L) and IVC
(C) by the mass (asterisk)
14 Pancreas, Adrenal Glands, and Retroperitoneum
a b
LK
Fig. 14.59 Bilateral pheochromocytomas in a 15-year-old male with von
Hippel-Lindau disease and hypertension. (a) Transverse grayscale ultra-
sound image of the right upper quadrant demonstrates an adrenal mass
(asterisk). L, Liver. (b) Transverse color Doppler ultrasound image shows
pronounced T2 hyperintensity and prominent postcontrast
enhancement. I-123 MIBG scintigraphy is performed prior
to resection to assess for multifocal disease or metastatic
lesions [113].
Surgical resection is critical for management of pediatric
pheochromocytoma. However, patients require careful periop-
erative management, as surgical manipulation of the tumor can
lead to massive catecholamine release followed by a hyperten-
sive crisis, cardiac arrhythmias, or stroke [163, 165]. Patients
also require genetic evaluation, as pheochromocytoma can be
the initial presentation of a familial syndrome that requires spe-
cific screening and assessment of other family members.
607
prominent vascularity (arrowheads) within the mass. (c) Longitudinal gray-
scale ultrasound image of the left kidney (LK) reveals a suprarenal mass
(asterisk)
Myelolipoma
Adrenal myelolipoma is not usually encountered in the
pediatric population, with only rare case reports in children
[166]. However, approximately 10% of these myelolipo-
mas develop in adults with a history of congenital adrenal
hyperplasia. They contain macroscopic fat and hematopoi-
etic tissue, and although they are benign, they can be asso-
ciated with hypersecretion of cortisol or aldosterone.
Endocrine abnormalities affect approximately 13–18% of
patients [166, 167].
608
By ultrasound, an adrenal myelolipoma appears echogenic,
related to its fat content. MR imaging is essential to document
the presence of larger amounts of macroscopic fat which dis-
tinguishes myelolipomas from other adrenal masses that may
contain only trace amounts of macroscopic fat.
When an incidental adrenal myelolipoma is identified, no
further management is indicated. Symptomatic patients may
require surgical resection. With larger myelolipomas, resec-
tion can be considered to avoid a rare but life-threatening
retroperitoneal hemorrhage. Hemorrhage is usually associ-
ated with masses greater than 10 cm in diameter [168].
Hemangioma
Retroperitoneal and adrenal infantile hemangiomas are uncom-
mon. At a large vascular anomalies center, a database identified
only 11 retroperitoneal infantile hemangiomas in an 18-year
period, and only one affected the adrenal gland [169]. Patients
were typically identified with cutaneous hemangiomas, and 3 of
the 11 patients were asymptomatic.
Like other infantile hemangiomas, adrenal and retroperi-
toneal hemangiomas will appear on ultrasound evaluation as
solid, vascular masses. Care must be taken to differentiate
infantile hemangioma of the adrenal gland and retroperito-
neum from the more aggressive kaposiform hemangioendo-
thelioma, which can also be seen in the retroperitoneum and
is discussed later in this chapter.
a
L K
Fig. 14.60 Adrenal teratoma in a 12-year-old male. (a) Longitudinal
grayscale ultrasound image demonstrates a right adrenal mass (arrows)
with a solid, echogenic portion (black asterisk), a cystic portion (white
asterisk), and punctate echogenic foci (arrowhead) associated with pos-
A. L. Hryhorczuk and H. J. Paltiel
Patients with adrenal infantile hemangiomas must be
assessed for involvement of other visceral organs. As at other
sites, infantile hemangioma of the adrenal gland and retroperito-
neum is expected to regress over time; many patients are treated
with propranolol or corticosteroids to hasten the involution of
the mass [169].
Teratoma
Adrenal teratoma is an extremely rare tumor; at a single cen-
ter where 7706 adrenalectomies were performed over a
64-year period, only 3 adrenal teratomas were identified, all
in patients between 24 and 29 years of age [170]. A literature
review identified only three reports of children diagnosed
with adrenal teratoma [170].
As with teratomas elsewhere in the body, these tumors con-
tain remnants of multiple germ cell layers and can be mature
or immature. They are most often left-sided and may contain
multiple cysts and calcifications at ultrasound (Fig. 14.60). If
a large fatty component is present, it may be difficult to dif-
ferentiate an adrenal teratoma from a myelolipoma.
Adrenal teratomas are surgically resected as it is difficult
to differentiate them from a malignancy. Children will typi-
cally be monitored for signs of recurrence. There is little data
to offer guidance for postoperative management [170].
b
terior acoustic shadowing compatible with calcification. K, Right kid-
ney; L, liver. (b) Coronal contrast-enhanced CT image of the mass
(arrow) reveals fat (white asterisk), soft tissue (black asterisk), and cal-
cifications (arrowhead)
14 Pancreas, Adrenal Glands, and Retroperitoneum
Leiomyoma
With only five case reports of this entity in children, adrenal
leiomyomas are not a common adrenal tumor in the pediatric
population [171]. They are hypothesized to arise from
smooth muscle cells within the adrenal vein and may present
as an incidental adrenal mass [171]. Although the specific
factors that cause adrenal leiomyomas are unknown, several
cases have been identified in immunocompromised patients,
often with human immunodeficiency virus (HIV)/acquired
immunodeficiency syndrome (AIDS) [172].
Ultrasound of an adrenal leiomyoma will demonstrate a
solid mass, without findings to suggest the specific diagno-
sis. Indeed, in most reported cases, a correct diagnosis was
not considered prospectively. As most adrenal leiomyomas
have been reported in adults, these masses are commonly
suspected to be non-functioning adrenal adenomas prior to
resection [172].
Following tumor resection, no further treatment is required.
However, it is important to consider the possibility of an
underlying immunodeficiency in these patients, and HIV test-
ing should be performed.
Lymphoma
Primary adrenal lymphoma is not typically a disease of chil-
dren. It is usually a manifestation of a diffuse B-cell lym-
phoma that affects an elderly adult population, with few
patients under the age of 40 years [173]. These patients usu-
ally have aggressive disease and a poor prognosis. Although
a few reports of adrenal lymphoma in children have been
published, they are sporadic [174, 175].
Ultrasound imaging of adrenal lymphoma can show
bilateral adrenal masses or diffuse adrenal enlargement.
Additional signs of lymphoma include enlarged lymph
nodes or and splenomegaly.
Treatment for adrenal lymphoma is directed by the pathol-
ogy of the underlying tumor and includes chemotherapy.
Metastases
Primary malignant tumors in children do not commonly metas-
tasize to the adrenal glands. Pulmonary, gastric, and esophageal
carcinomas are the most common tumors a­ ssociated with adre-
nal metastases [176]. However, if a child with a known malig-
nancy presents with unilateral or bilateral adrenal masses,
metastatic disease should be considered.
By ultrasound, adrenal metastases usually appear as solid
masses. In adults, they are usually small, with a mean size of
2 cm [176].
Suspected adrenal metastatic disease can be confidently
diagnosed with tissue sampling. Treatment will be dictated
by the underlying disease process.
609
Nonvascular Disorders
of the Retroperitoneum
Ultrasound is not generally used as the primary imaging
modality in defining nonvascular disorders of the retroperito-
neum. When they are identified by ultrasound, further char-
acterization of their nature and extent with cross-sectional
imaging is usually required.
Technique
Patient Positioning
Patients are typically positioned supine, with prone position-
ing sometimes used in younger children and infants.
Transducer pressure can displace bowel loops and permit
evaluation of the retroperitoneal structures. Rotating the
patient into right and left lateral decubitus positions can also
displace bowel loops and improve visualization.
Ultrasound Transducer Selection
The highest-frequency transducers that can achieve adequate
penetration and delineation of the retroperitoneal structures
are recommended. Linear and high-frequency transducers on
the order of 9.0–15 MHz can be used in infants and young
children, while older and larger patients generally require
lower-frequency curved transducers (3.0–9.0 MHz).
Imaging Approaches
Techniques for pancreatic and adrenal imaging are covered
earlier in this chapter. Assessment of other retroperitoneal
structures and abnormalities is often difficult due to the pres-
ence of overlying bowel gas. Gentle compression of the
abdomen with the transducer, as well as changes in patient
position, may result in improved imaging.
Normal Development and Anatomy
Normal Development
In embryonic life, the mesenchyme in the posterior portion of
the embryo develops into the vertebral bodies and muscular
components of the body wall, including the paraspinal, psoas,
and transverse abdominal muscles. It is covered by a layer of
connective tissue that separates it from the abdominal cavity,
the transversalis fascia. Fatty tissue located posteriorly between
the transversalis fascia and the parietal peritoneum of the
abdominal cavity develops into the posterior pararenal space.
This space will eventually contain the kidneys. Development
of the kidneys is discussed in Chap. 17. The kidneys ascend
into the fat-­containing retroperitoneal space with their sur-
rounding fascial layers that comprise the perirenal space. The
fascial layers ultimately fuse superiorly to the diaphragm, thereby
delimiting the anterior pararenal space anteriorly and the poste-
rior pararenal space posteriorly [177].
610 A. L. Hryhorczuk and H. J. Paltiel

Normal Anatomy
The retroperitoneum is divided into three separate compart-
ments: the anterior pararenal space, lying between the poste-
rior surface of the parietal peritoneum and the anterior
reflection of the perirenal fascia; the perirenal space, sur-
rounded by the perirenal fascia and in continuity across the
midline with the opposite perirenal space; and the posterior
pararenal space, located between the posterior renal fascia
anteriorly and the transversalis fascia posteriorly [178].
The anterior pararenal space contains the pancreas, duode-
num, ascending colon, and descending colon; the perirenal
space contains the kidneys and adrenal glands, and the poste-
rior pararenal space contains fat (Figs. 14.61 and 14.62) [178].
A fourth compartment that is not well-delimited by fascial

Right adrenal
gland

Right kidney
Pancreas
Duodenum

Ascending
Descending
colon
colon
Inferior
vena cava
Aorta

Ureter

Bladder

Fig. 14.61 Diagram of retroperitoneal structures. The adrenal glands, retroperitoneal location during development. The bladder is inferior to
kidneys, ureters, aorta, and IVC are primary retroperitoneal structures. the retroperitoneal space
The pancreas, duodenum, ascending and descending colon assume a

Anterior
Parietal Anterior renal pararenal Descending
peritoneum fascia Duodenum Pancreas space colon

Lateroconal fascia Perirenal space

Ascending colon
Retrorenal fascia

Transversalis fascia
Posterior
pararenal space

Fig. 14.62 Diagram of the normal retroperitoneal compartment and potential spaces. The retroperitoneum consists of three compartments: the
anterior pararenal space, the perirenal space, and the posterior pararenal space
14 Pancreas, Adrenal Glands, and Retroperitoneum
planes contains the abdominal aorta, inferior vena cava (IVC),
lymph nodes, and fat.
In addition, there are potential spaces between these com-
partments. The retromesenteric space between the anterior
pararenal space and the perirenal space permits the spread of
fluid throughout the retroperitoneum in the setting of pancre-
atitis. The retrorenal space between the perirenal space and
the posterior pararenal space also allows passage of fluid,
hemorrhage, or infection throughout the retroperitoneum.
Inferiorly, the fascial structures that create the retromesen-
teric space and retrorenal space merge to form the interfas-
cial plane. This plane extends to the pelvis and represents
another potential pathway for the spread of disease from the
retroperitoneum [178].
In addition to the kidneys, adrenal glands, pancreas, and
portions of the bowel, the retroperitoneum also contains
lymph nodes, lymphatics, nerves, fat, and connective tissue.
When any of these structures is enlarged or otherwise altered
by pathological processes, ultrasound often serves as the ini-
tial imaging modality and helps to guide further investiga-
tion and treatment.
K
Fig. 14.63 Perinephric abscess in a 4-year-old female with a fever. (a)
Longitudinal grayscale ultrasound image reveals a retroperitoneal fluid col-
lection (arrows) adjacent to the right kidney (K). (b) Sagittal T2-weighted,
611
Infection and Abscess
Retroperitoneal abscesses in children typically develop from
bacterial infection of retroperitoneal structures [179]. A review
of retroperitoneal abscesses found that in 41 children with ret-
roperitoneal abscesses, 21 involved the anterior retroperito-
neum and originated from the pancreas, esophagus, duodenum,
and lower GI tract. Six abscesses were perinephric, seven were
pelvic, and seven involved retrofascial muscular structures,
including the psoas and iliacus muscles (Figs. 14.63 and
14.64) [180]. In comparison with intra-abdominal abscesses,
children with retroperitoneal abscesses may have vague symp-
toms that are poorly localized. This can lead to a delay in diag-
nosis and increased morbidity [179].
By ultrasound, an abscess involving the anterior parare-
nal structures will be similar to abscesses in other anatomic
locations: a discrete, thick-walled fluid collection contain-
ing echogenic debris without internal vascularity and
peripheral hyperemia depicted with color Doppler. Gas
identified within an abscess suggests a connection to the
intestinal tract. Since infections within the anterior parare-
K
fat-suppressed MR image shows an abscess (asterisks) along the posterior
aspect of the right kidney (K)
612
Fig. 14.64 Psoas abscess in a 13-year-old male with Crohn disease.
Oblique grayscale ultrasound image obtained along the plane of the
psoas muscle (P) demonstrates a small bowel loop with significant wall
Fig. 14.65 Perforated appendix with retroperitoneal abscess in a 2-year-
old female. (a) Transverse grayscale ultrasound image of the right lower
quadrant shows a heterogeneous inflammatory mass (arrows) containing
fluid (white asterisk) and echogenic debris. The surrounding retroperito-
neal soft tissues (black asterisks) are of increased echogenicity, in keeping
nal space can occur as a sequela of ruptured appendicitis or
in the setting of inflammatory bowel disease with perfora-
tion, care should be taken to identify the appendix and to
evaluate adjacent bowel loops for wall thickening and
hypervascularity. If an echogenic structure is seen within
an abscess, possible appendiceal rupture with an extruded
appendicolith should be considered (Fig. 14.65).
A. L. Hryhorczuk and H. J. Paltiel
thickening (asterisk) and surrounding echogenic fat (arrowheads) com-
patible with inflammatory change. A discrete abscess (arrows) is pres-
ent within the psoas muscle
with inflammation. An appendicolith (arrowhead) with distal shadowing is
identified within the inflammatory mass compatible with perforated appen-
dicitis. P, Psoas muscle. (b) Transverse grayscale ultrasound image reveals
hyperechoic soft tissues (asterisks) surrounding the right kidney (K), com-
patible with inflammation
If a retroperitoneal abscess occurs in the deep pelvis, it
may originate from a sacroiliac, pelvic, or vertebral infec-
tion. Iliopsoas abscesses occur most commonly in the con-
text of direct trauma with hematogenous seeding. MR
imaging should be considered if there is any concern for an
underlying osseous infection.
14 Pancreas, Adrenal Glands, and Retroperitoneum
In children, retroperitoneal abscesses are treated with
drainage, which can be guided by ultrasound or CT. Antibiotic
treatment is targeted to the underlying source of infection.
When an abscess is identified in the setting of appendicitis,
drainage and scheduling of surgery will be dictated by local
appendicitis treatment guidelines.
Hemorrhage
The retroperitoneum represents a large potential space
where significant blood loss can occur, with the possibility
for ­catastrophic hemorrhage. Bleeding can occur following
trauma, iatrogenic injury (e.g., after vascular catheteriza-
tion), and in the presence of a friable retroperitoneal mass
that can bleed spontaneously. Children with coagulation
disorders can also develop hemorrhage after only minimal
trauma. All of these potential causes of retroperitoneal
hemorrhage should be considered in a child with an acute
drop in hemoglobin and flank pain.
Ultrasound findings of retroperitoneal hemorrhage are
varied and reflect the acuity of presentation as well as the
underlying cause (Fig. 14.66). After significant blunt
trauma, retroperitoneal hemorrhage can result from exten-
sive pelvic fractures that are usually documented by CT
[181]. In this setting, ultrasound is useful in follow-up of
the associated hematoma.
When a retroperitoneal hematoma develops following
femoral artery catheterization, ultrasound is essential for
identification of a pseudoaneurysm that may be contributing
a b
Fig. 14.66 Perinephric hematoma in a 15-year-old male with Takayasu
arteritis. (a) Transverse grayscale ultrasound image of the right kidney
(K) demonstrates a large perinephric hematoma (arrows). The right renal
613
to a persistent bleed. Hemorrhage into a tumor will appear as
an avascular, often complex collection within a soft tissue
mass that may contain fluid-fluid levels [182, 183]. If there is
an underlying coagulation disorder, ultrasound can provide
rapid identification of an acute retroperitoneal fluid collec-
tion or a hemophiliac pseudotumor (Fig. 14.67) [184].
In an acute setting with active bleeding and an unstable
patient, children with retroperitoneal hemorrhage may undergo
catheter angiography for both diagnostic purposes and possible
embolization. If the patient is stable, management will shift
toward addressing the underlying cause of the hemorrhage and
ensuring that coagulation parameters are normalized.
Fibrosis
Retroperitoneal fibrosis is a usually a disease of older adults
[185]. Children who develop retroperitoneal fibrosis usually
have an underlying immunologic disease, such as systemic
lupus erythematosus, juvenile idiopathic arthritis, lym-
phoma, or pulmonary hyalinizing granuloma. Affected
patients may present with ureteral compression causing
hydronephrosis, renal artery compression producing hyper-
tension, and venous compression resulting in lower extrem-
ity edema [186–188].
Since most patients with retroperitoneal fibrosis present
with hydronephrosis, ultrasound can provide a rapid, acces-
sible technique for monitoring renal collecting system dila-
tion. However, it can be difficult to visualize and monitor the
soft tissue encasing the retroperitoneal structures with ultra-
collecting system (asterisk) is dilated. (b) Coronal contrast-enhanced CT
image shows the perinephric hematoma (asterisk) as well as an aneurysm
(arrowhead) of the distal aorta near its bifurcation
614
a b
Fig. 14.67 Retroperitoneal hematoma in a 14-year-old male with hemo-
philia and left flank pain after a snowboarding injury. (a) Longitudinal
grayscale ultrasound image of the left kidney (K) depicts an echogenic
sound. MR imaging is considered the first-line technique for
monitoring retroperitoneal fibrosis, and 15-minute delayed
imaging is especially useful in demonstrating contrast reten-
tion in fibrotic tissue [187].
Corticosteroid treatment is commonly used as first-line
therapy for pediatric retroperitoneal fibrosis [187]. Additional
immunosuppressive therapies are also used, including aza-
thioprine [189]. Surgical therapy to relieve ureteral or vascu-
lar obstruction is rarely pursued. However, for patients with
severe hydronephrosis, a percutaneous nephrostomy tube
can relieve acute obstruction while immunosuppressive ther-
apy is initiated.
Extramedullary Hematopoiesis
In extramedullary hematopoiesis, blood cells are produced
outside the bone marrow. Although this normally happens
in fetal life, where the liver and spleen participate in hema-
topoiesis, the bone marrow exclusively produces hemato-
poietic cells at the time of birth. If there is a demand for
blood cell production that outstrips the capacity of the bone
marrow, extramedullary hematopoiesis will occur. In some
cases, extramedullary hematopoiesis produces focal masses
adjacent to the axial skeleton [190]. Typically, patients
have a history of anemia or myelofibrosis.
Mass-like lesions most often develop in a paraspinal or
presacral location. Because of their deep location, they may
be difficult to identify with ultrasound. When visible, retro-
peritoneal extramedullary hematopoiesis often appears echo-
A. L. Hryhorczuk and H. J. Paltiel
perinephric fluid collection (asterisk). (b) Longitudinal color Doppler
ultrasound image shows no evidence of internal vascularity within the
collection (asterisk)
genic due to a prominent fatty content that occurs in about
70% of patients [190].
In many cases, extramedullary hematopoiesis requires no
specific treatment, although patients may receive t­ransfusions
to diminish the physiologic demand for hematopoiesis [191].
In some patients, large retroperitoneal masses will compress
the peripheral nerves or extend into the spinal canal and cause
neurologic symptoms. Low-dose radiotherapy can be consid-
ered in these cases to preserve neurologic function [192].
Venous and Lymphatic Malformations
Retroperitoneal venous and lymphatic malformations are
rare. Occasionally these malformations are identified prena-
tally. There may be associated fetal hydrops or fetal demise,
while other patients are asymptomatic at birth [193].
Venous lesions have low flow and may be difficult to
identify and characterize by ultrasound. Lymphatic malfor-
mations may be microcystic or macrocystic. Macrocystic
lymphatic malformations will demonstrate cysts of varying
size that may contain fluid levels related to proteinaceous
debris or internal hemorrhage (Fig. 14.68). Color Doppler
imaging may reveal small arteries and veins in the septa of
the lesion. Prior to definitive treatment, MR imaging should
be obtained to provide a more detailed assessment of the
lesion and its relationship to adjacent anatomic structures.
Treatment of venous and lymphatic malformations often
requires a combination of surgical resection and sclerotherapy
[194]. Laparoscopic surgery is sometimes performed [193].
14 Pancreas, Adrenal Glands, and Retroperitoneum
a b
Fig. 14.68 Retroperitoneal lymphatic malformation in a 6-year-old
male with abdominal and left flank pain. (a) Transverse grayscale ultra-
sound image of the left lower quadrant demonstrates a fluid-filled mass
(asterisks) with internal septations (arrowheads) overlying the psoas
muscle (P). (b) Axial contrast-enhanced CT image of the abdomen
Lymphadenopathy
Infection
Enlarged retroperitoneal lymph nodes can occur in associa-
tion with any retroperitoneal infectious process. Disorders
that primarily affect the abdominal lymph nodes, such as
tuberculosis, present with lymphadenopathy in both the mes-
entery and retroperitoneum [195].
Reactive retroperitoneal lymph nodes appear enlarged
and hyperemic, with preservation of their normal ovoid
shape and internal architecture, with a well-defined hilum.
The enlarged retroperitoneal lymph nodes in patients with
abdominal tuberculosis are situated in the upper abdomen,
adjacent to the aorta [195]. They will frequently demonstrate
a central hypoechoic zone and may contain small calcifica-
tions. In long-standing tuberculosis, lymph nodes may be
frankly necrotic and contain coarse calcifications [196].
Treatment is determined by the underlying infection. In
patients with tuberculous lymphadenopathy, the approach is
identical to that for pulmonary tuberculosis, consisting of a
9-month course of isoniazid and rifampin or a 6-month
course of these two agents as well as pyrazinamide.
615
shows the left lower quadrant fluid collection (asterisk). (c) Transverse
grayscale ultrasound image after sclerotherapy shows resolution of the
mass, with bowel loops (asterisks) filling the left lower quadrant. A cal-
cification with distal shadowing (arrowhead) reflects post-sclerotherapy
changes. P, Psoas muscle
Lymphoma
Peritoneal and retroperitoneal lymph node involvement in
children typically occurs with diffuse large B-cell lymphoma
and anaplastic large cell lymphoma. Patients with both
Hodgkin and non-Hodgkin lymphoma can have enlarged ret-
roperitoneal lymph nodes, although primary infradiaphrag-
matic disease is rare with Hodgkin lymphoma, occurring in
fewer than 4% of patients. Enlarged retroperitoneal lymph
nodes in these children are usually due to extensive disease
with involvement of lymph node groups both above and
below the diaphragm [197].
Patterns of lymph node involvement are variable in non-
Hodgkin lymphoma, and both enlarged retroperitoneal and
extranodal disease can occur. Retroperitoneal lymph node
involvement is less common in Burkitt lymphoma [197–199].
On ultrasound evaluation, lymphomatous lymph nodes
appear hypoechoic with a micronodular/reticular appearance
[200, 201]. These nodes are often rounder in shape, and a dis-
tinct hilum cannot be identified [201]. They may become con-
fluent and form a mass-like nodal conglomeration (Fig. 14.69).
Further cross-sectional imaging is essential; MR imaging or CT
will provide a more comprehensive assessment of regional
616
a b
Fig. 14.69 Recurrent diffuse B-cell lymphoma in a 16-year-old female.
(a) Transverse grayscale ultrasound image depicts a retroperitoneal soft
tissue mass (asterisk) adjacent to the left kidney (K) and spleen (S). (b)
a b
Fig. 14.70 Metastatic testicular seminoma in a 22-year-old male with
flank pain. (a) Transverse grayscale ultrasound image demonstrates a
large retroperitoneal mass (asterisk) medial to the right kidney (K).
involvement, while FDG-PET imaging is required to determine
the extent of disease and to monitor response to therapy.
In cases of suspected lymphoma, biopsy and tissue diag-
nosis provide the first step in initiating appropriate therapy.
Retroperitoneal lymph nodes should not be biopsied if more
readily accessible targets can be identified. Once a patho-
logic diagnosis is made, imaging is used to follow response
to therapy.
Metastatic Disease
Retroperitoneal lymph node metastases can occur with primary
renal and gonadal tumors that drain through the gonadal veins
into the retroperitoneum. Identification of local lymph node
metastases from Wilms’ tumor is assessed during surgical
removal of the primary tumor. The presence of nodal disease
increases tumor stage and supports an intensified therapeutic
pathway [202]. Patients with testicular or paratesticular tumors
do not routinely undergo surgical staging of the retroperitoneum
at the time of primary tumor resection. Imaging determination
of retroperitoneal nodal disease is therefore critical.
A. L. Hryhorczuk and H. J. Paltiel
A
S
Axial contrast-enhanced CT image of the abdomen shows the mass
(asterisk) in the expected location of the left para-aortic lymph nodes.
A, Aorta; S, spleen
(b) Longitudinal grayscale ultrasound image of the right testis shows
the small, hypoechoic primary parenchymal tumor (asterisk). Testicular
microlithiasis is also identified (arrowheads)
In the setting of a known primary tumor, evaluation of
nodal disease with CT or MR imaging is superior to ultra-
sound and preferred. However, patients with testicular neo-
plasms sometimes present with symptoms caused by
retroperitoneal venous compression or flank pain (Fig. 14.70).
If an abdominal ultrasound study is obtained prior to clinical
assessment of the scrotum, enlarged retroperitoneal lymph
nodes may be identified in the typical locations for lymphatic
spread of testicular neoplasms: inter-aortocaval nodes with a
right testicular tumor and left para-aortic nodes with a left-
sided tumor [203]. Similar patterns have been described for
paratesticular rhabdomyosarcoma that can spread to the ret-
roperitoneum via lymphatic channels.
If a child with a testicular neoplasm has retroperitoneal
lymph node metastases, the disease remains curable. Treatment
typically involves radical orchiectomy with cisplatin-­based che-
motherapy, retroperitoneal lymph node dissection, and radiation
therapy [203]. For children with paratesticular rhabdomyosar-
coma, retroperitoneal lymph node dissection is recommended
only for patients 10 years of age and older [204].
14 Pancreas, Adrenal Glands, and Retroperitoneum
Benign Masses
Hemangioma
Retroperitoneal infantile hemangioma is uncommon. When
it occurs, it can be visceral in location or identified adjacent
to the retroperitoneal vessels [169]. Almost all patients
with a retroperitoneal hemangioma will have associated
cutaneous involvement that brings them to clinical
attention.
Non-visceral retroperitoneal infantile hemangioma usu-
ally occurs next to the aorta, although it can also occur adja-
cent to the IVC or iliac vessels. Demonstration of these
lesions by ultrasound can be technically difficult, as they are
located deep within the body. MR imaging is typically used
to identify and characterize these lesions as well as other ret-
roperitoneal visceral hemangiomas.
Infantile hemangiomas will eventually regress but can be
treated with propranolol or corticosteroids to hasten involu-
tion. It is important to differentiate a retroperitoneal infantile
hemangioma from KHE, a benign but aggressive vascular
tumor that requires different treatment and is described ear-
lier in this chapter [169].
Mature Teratoma
Mature retroperitoneal teratoma is rare and usually identified
in female infants and young women [205]. They may contain
fat, fluid, soft tissue, and calcification with cystic and solid
components.
When imaged by ultrasound, the mass may contain shad-
owing calcification, internal fluid-fluid levels in cystic compo-
nents, and soft tissue. Fat may be difficult to reliably distinguish
from other soft tissue components by ultrasound [206].
In rare cases, a mature retroperitoneal teratoma can undergo
malignant transformation. However, most tumors are success-
fully treated with resection.
Retroperitoneal Lipoma and Lipoblastoma
Lipoma and lipoblastoma are benign fatty tumors that develop
at multiple sites throughout the body. Lipoma contains mature
adipose tissue and most often occurs in adults. Lipoblastoma
contains immature lipoblasts and mesenchymal cells and only
occurs in children, with more than 80% of tumors identified in
patients less than 3 years of age [207]. Lipoma and lipoblas-
toma rarely occur in the retroperitoneum during childhood,
with most patients presenting in the fifth or sixth decade of life
[205]. Lipoblastoma usually appears on the trunk and extremi-
ties. In a case series of 33 children with lipoblastoma, only 1
patient had a retroperitoneal tumor [208].
617
On ultrasound evaluation, lipoma and lipoblastoma
appear as well-circumscribed tumors contain echogenic fat.
While lipoma is typically homogenous, lipoblastoma may
demonstrate internal septations and myxoid material.
Lipoblastoma reveals increased vascularity with color
Doppler, while lipoma is relatively avascular [207].
A lipoma is usually removed when it is large or symptom-
atic. Lipoblastoma is typically large at presentation and aggres-
sive, requiring complete excision. Although it is a benign lesion,
patients require long-term follow-up of 10 years or more, as
there is a risk of late recurrence that may require further resec-
tion [209].
Neurofibroma and Schwannoma
Neurofibroma and schwannoma are peripheral nerve sheath
tumors that can occur at multiple sites in the body, including
the retroperitoneum. Retroperitoneal neurofibroma is usually
solitary, although 30% of solitary tumors occur in the setting
of type 1 neurofibromatosis (NF-1) [210]. The presence of a
retroperitoneal plexiform neurofibroma or multiple neurofi-
bromas is diagnostic of NF-1. Schwannomas are slightly
more common than neurofibromas, affect females more than
males, and typically occur in patients between the ages of 20
and 50 years [210].
There are scant descriptions of the ultrasound features of
retroperitoneal peripheral nerve sheath tumors. A heteroge-
neous echotexture with variable through transmission has
been described [211]. Further assessment with cross-­sectional
imaging is essential (Fig. 14.71). MR imaging is usually pre-
ferred, as it can demonstrate extension of tumor through the
neural foramina with possible spinal cord impingement.
Retroperitoneal nerve sheath tumors can be followed when
they are asymptomatic. If neurologic symptoms develop, sur-
gical resection should be performed. A rapidly growing mass
in a patient with NF-1 suggests malignant transformation that
necessitates more aggressive treatment, including resection,
chemotherapy, and radiation [212].
 eural Crest Tumors: Ganglioneuroma,
N
Ganglioneuroblastoma, and Neuroblastoma
As described earlier in this chapter, the three neural crest
tumors, ganglioneuroma, ganglioneuroblastoma, and neu-
roblastoma, can arise anywhere in the sympathetic nervous
system, including the adrenal medulla as well as in sympa-
thetic ganglia extending from the neck to the posterior
mediastinum, retroperitoneum, and pelvis. In approxi-
mately 30–37% of cases, these neural crest tumors will
618
a b
B
U
R
Fig. 14.71 Plexiform neurofibroma in a 16-year-old female with neu-
rofibromatosis type 1. (a) Sagittal grayscale ultrasound image of the
pelvis reveals an extensive neurofibroma (arrows) that extends superi-
orly into the retroperitoneum. U, Uterus; B, bladder. (b) Longitudinal
grayscale ultrasound image of the left kidney shows hydronephrosis
develop in the extra-adrenal retroperitoneal tissues. When
the tumors are large, it may be difficult to definitively iden-
tify their site of origin [155].
Imaging features of primary retroperitoneal masses are
identical to those of the adrenal gland: they may be calcified
and infiltrative, revealing vascular encasement and involve-
ment of adjacent vital structures (Figs. 14.72, 14.73, and
14.74). Assessment of image-defined risk factors is identical
A. L. Hryhorczuk and H. J. Paltiel
(asterisk) due to ureteral obstruction by the neurofibroma. (c) Coronal
T2-weighted, fat-­suppressed MR image demonstrates a large plexiform
neurofibroma (asterisk) of the pelvis, with a portion extending into the
left retroperitoneum (arrowheads). There is significant displacement of
the uterus (U) and rectum (R)
to those of primary adrenal masses, although special atten-
tion should be paid to neural foraminal invasion which can
occur with tumors originating next to the vertebrae. Spine
MR imaging is performed for identification of this specific
image-­defined risk factor.
Treatment of neural crest tumors of the retroperitoneum is
identical to that of adrenal medullary tumors discussed ear-
lier in this chapter.
14 Pancreas, Adrenal Glands, and Retroperitoneum 619

a b

Fig. 14.72 Retroperitoneal ganglioneuroma in a 5-year-old female. (a) retroperitoneal mass compressing the anterior margin of the right kidney
Transverse grayscale ultrasound image of the right upper quadrant demon- (K). A mature ganglioneuroma was diagnosed at pathology following
strates a homogenous, hypoechoic mass (asterisk) arising anteromedial to resection
the right kidney (K). (b) Axial contrast-enhanced CT image shows a cystic

a b

Fig. 14.73 Retroperitoneal ganglioneuroblastoma in a 6-year-old female (K). Focal calcifications (arrowheads) are identified. (b) Axial T2-weighted,
with a palpable back mass. (a) Transverse posterior approach grayscale fat-suppressed MR image demonstrates a retroperitoneal mass (asterisk)
ultrasound image of the right flank shows a soft tissue mass (asterisks) infiltrating into the posterior spinal musculature (arrow). There is neural
extending superficially from the retroperitoneum and medial to the kidney foraminal invasion (arrowhead). K, Right kidney
620
a b
Fig. 14.74 Retroperitoneal neuroblastoma in 9-month-old female who
presented with a urinary tract infection. (a) Longitudinal grayscale
ultrasound image of the left kidney (K) shows a retroperitoneal mass
(arrow) adjacent to the renal hilum. There is mild dilation of the renal
pelvis (asterisk), suggesting obstruction by the mass. (b) Longitudinal
color Doppler ultrasound image of the left kidney demonstrates renal
Malignant Tumors
Rhabdomyosarcoma
Primary retroperitoneal rhabdomyosarcoma is rare and
may grow to a large size prior to presentation so that the
organ of origin can be difficult to identify [156].
Ultrasound may show a heterogeneous mass with areas of
internal necrosis, and further assessment with CT or MR
imaging is typically performed (Fig. 14.75). Patients are
assessed for metastatic disease, which most commonly
involves the lungs and bones. Distant lymph node metas-
tases can also occur, although they are more common in
patients with primary tumors arising from the extremities
[213].
Although complete surgical resection is typically consid-
ered to be an essential early step in the management of rhab-
domyosarcoma, primary retroperitoneal masses are difficult
to remove in their entirely due to their frequently large size
at presentation and characteristic invasion of multiple adja-
cent structures. Patients may be given chemotherapy to assess
tumor response, followed by delayed primary excision and
radiation therapy [214].
Infantile Fibrosarcoma
Infantile fibrosarcoma most commonly occurs as a deep soft
tissue mass of the extremities or trunk, usually in infants less
than 2 years of age [156, 215]. Although infantile fibrosar-
A. L. Hryhorczuk and H. J. Paltiel
c
hilar vessels (arrowhead) abutting the left renal mass (asterisk), con-
cerning for tumor invasion of the vascular pedicle, an image-defined
risk factor. (c) Transverse T2-weighted, fat-suppressed MR image of
the abdomen depicts the mass (arrows) encasing the left renal artery
(arrowhead)
coma can present with a retroperitoneal mass, it is not a com-
mon site, occurring in only 7% of patients [216]. Affected
patients will present with abdominal distension related to the
mass. These masses can be very large at presentation, and on
imaging their site of origin can be difficult to appreciate.
Imaging features are nonspecific [217].
Surgical resection is the primary treatment in approxi-
mately 45% of patients. An equivalent number of patients also
receive chemotherapy. Radiation therapy is used infrequently.
Long-term outcomes for patients with infantile fibrosarcoma
are excellent, with an 89% overall 5-year survival rate [215].
 alignant Germ Cell Tumor/Immature
M
Teratoma
Malignant germ cell tumor/teratoma in the retroperitoneum is
usually a metastatic lesion that originated from an ovarian or
testicular germ cell tumor [205]. These tumors may grow to a
large size, and patients can present with a palpable mass or signs
of local compression, including constipation, abdominal pain,
and lower extremity edema from venous compromise.
On ultrasound, visualization of small internal calcifications
can suggest their germ cell origin. CT and MR imaging will
depict any fatty components and determine their extent. Imaging
evaluation requires careful gonadal assessment, where the pri-
mary tumor may reside. In some cases, this finding can be very
subtle, with only scant testicular calcifications remaining from a
“burned-out” testicular tumor [218].
14 Pancreas, Adrenal Glands, and Retroperitoneum
a b
A A
Fig. 14.75 Rhabdomyosarcoma in a 10-year-old girl with abdominal
pain. (a) Transverse grayscale ultrasound image demonstrates a large
retroperitoneal mass (asterisk) and ascites (A). The gallbladder (G) con-
tains echogenic, layering sludge. (b) Transverse color Doppler ultra-
Treatment of a malignant germ cell tumor is initiated with
chemotherapy, using a cisplatin-based regimen, followed by
resection of residual tumor or radiation therapy.
Smooth Muscle Tumors
Smooth muscle tumors, including leiomyosarcoma, are
extremely rare in the pediatric population, with one main
exception: children with immune deficiencies or immuno-
suppression. These children typically have AIDS with a
significantly depressed CD4 count, or immunosuppression
after organ transplantation, and are predisposed to develop
Epstein-Barr virus-associated smooth muscle tumors
(EBV-SMTs) [156]. In comparison with leiomyosarcoma,
EBV-SMTs are less aggressive, without cellular atypia or
tumor necrosis. In some cases, these tumors may be retro-
peritoneal, although they can occur throughout the body
[219, 220].
The ultrasound appearance of EBV-SMTs has not been
well studied: they generally appear as solid masses in the
retroperitoneum or within a solid organ.
EBV-SMTs are not typically treated with aggressive therapy.
In some patients, immunosuppression is reduced; in others, sur-
gical resection of an individual tumor is performed. Overall
prognosis is related to intracranial involvement, with intracra-
nial lesions associated with an overall poor prognosis [220].
 ndifferentiated Pleomorphic Sarcoma
U Because retroperitoneal Ewing sarcoma tends to be large
(Malignant Fibrous Histiocytoma)
Undifferentiated pleomorphic sarcoma (previously known as
malignant fibrous histiocytoma) is a rare pediatric sarcoma, usu-
621
c
sound image shows blood flow (arrows) within the mass. (c) Axial
T2-weighted, fat-suppressed MR image depicts the retroperitoneal mass
(asterisk), a peritoneal implant (arrowhead), and ascites (A)
ally developing in patients who have received prior radiation for
acute lymphoblastic leukemia. In children they usually occur in
the extremities and rarely develop in the retroperitoneum [221,
222]. In adults, retroperitoneal undifferentiated pleomorphic
sarcoma primarily involves the psoas muscle [223].
Ultrasound features of these tumors are not well described.
Like many retroperitoneal masses, they may grow to a large
size prior to discovery.
Management of these rare tumors typically requires surgical
resection. When resection involves the psoas muscle, special
care must be taken to identify and preserve the femoral nerve if
it is not directly involved with tumor [223]. Chemotherapy and
radiation are also used for disease control.
Ewing Sarcoma
Ewing sarcoma is a small round blue cell tumor that usually
affects patients under the age of 20 years. Disease can be osse-
ous or extraosseous, although 80% of cases occur within bone
[224]. Although extraosseous primary tumors, including retro-
peritoneal masses, account for approximately 50% of Ewing
sarcoma in adults [224], they are far less frequent in children,
who may present with back pain or difficulty walking.
As with many of the rare primary retroperitoneal tumors
previously described, extraosseous Ewing sarcoma of the
retroperitoneum can grow for a long period without detec-
tion. Imaging may identify a large retroperitoneal mass with
heterogeneous areas of internal necrosis.
at diagnosis and involve multiple adjacent vital structures,
surgical resection can be very challenging. If surgical resec-
tion is not possible, chemotherapy and local radiation ther-
apy can be administered [156].
622
Fig. 14.76 Kaposiform hemangioendothelioma in a 6-week-old male.
(a) Transverse grayscale ultrasound image of the mid-abdomen shows
a large, heterogeneous, soft tissue mass (arrows) that displaces the nor-
mal intraabdominal structures. S, Spine. (b) Longitudinal color Doppler
ultrasound image shows the IVC (arrowheads) displaced and com-
Inflammatory Myofibroblastic Tumor
As discussed earlier in this chapter, inflammatory myofibroblas-
tic tumors are usually benign but locally aggressive lesions.
They can occur in the retroperitoneum, either within the soft
tissues or solid organs, including the pancreas and kidney [225].
Ultrasound features are nonspecific, revealing a large
retroperitoneal mass that is difficult to distinguish from
other retroperitoneal tumors. On MR imaging, internal sig-
nal may be hyperintense on T2-weighted sequences, with
heterogeneous enhancement.
Surgical resection is essential to management, although
steroids and anti-inflammatory medications can also help to
shrink these masses [73]. Local recurrence can occur, and
patients should be closely monitored. In rare cases, metasta-
ses may develop that do not appear to respond well to adju-
vant chemotherapy or radiation therapy [226].
Kaposiform Hemangioendothelioma
As previously described, kaposiform hemangioendothelioma
(KHE) is a vascular neoplasm that commonly presents in
infancy. It usually manifests as a cutaneous lesion but also
occurs in the chest and retroperitoneum. Thoracoabdominal
and retroperitoneal KHEs are problematic, as in nearly all
cases they are associated with Kasabach-­Merritt phenomenon
[227]. This life-threatening thrombocytopenia is caused by
platelet trapping in the tumor and can be the presenting feature
in patients with a retroperitoneal KHE.
KHE can manifest as a discrete mass or as a diffuse and
infiltrative lesion containing vessels with prominent arterial
A. L. Hryhorczuk and H. J. Paltiel
pressed by the infiltrating mass (asterisks) that crosses tissue planes.
The tumor contains a small number of blood vessels. (c) Axial T2-weighted,
fat-­suppressed MR image shows bilateral hydronephrosis (asterisks). The
tumor (arrows) extends through the retroperitoneum and mesentery.
A, Ascites
and venous flow (Fig. 14.76) [228]. MR imaging may show
heterogeneous enhancement, with better visualization of the
vascular mass infiltrating through tissue planes than is pos-
sible by ultrasound.
For infants with KHE, surgical resection represents a
first-line therapy [60]. The size and infiltrative nature of ret-
roperitoneal masses, however, can make surgical resection
impossible. In these patients, a variety of medical therapies
is used to promote tumor regression, previously discussed in
the section on pancreatic KHE.
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 Male Genital Tract
15
Judy H. Squires and Harriet J. Paltiel

Abbreviations of pain or palpable abnormality. Contrast-enhanced ultra-

sound and elastography may supply additional information
AFP Alpha-fetoprotein in certain conditions affecting the testes and paratesticular
ALK Anaplastic lymphoma kinase tissues. The prostate gland and seminal vesicles can also be
AVM Arteriovenous malformation evaluated. In this chapter, imaging techniques, normal devel-
CEUS Contrast-enhanced ultrasound opment and anatomy, congenital anomalies, inflammatory
CT Computed tomography disorders and tumors of the male genital tract are discussed.
ESUR European Society of Urogenital Radiology
HSP Henoch-Schönlein Purpura
Ig Immunoglobulin Scrotum
IMT Inflammatory myofibroblastic tumor
IV Intravenous Exquisite imaging detail of the scrotum can be obtained
LCH Langerhans cell histiocytosis with the use of high-frequency transducers because of the
MAG3 Mercaptoacetyltriglycine superficial location of the intrascrotal structures and rela-
MR Magnetic resonance tively thin scrotal wall, which provides an excellent acous-
NF1 Neurofibromatosis type 1 tic window [1–3]. Color and spectral Doppler are routinely
SWE Shear wave elastography used to assess the vascularity of the testis, epididymis, and
TNM Tumor, Node, Metastasis other scrotal structures, as well as in the evaluation of scrotal
masses. Contrast-enhanced ultrasound (CEUS) may have a
role to play in the evaluation of parenchymal perfusion in the
setting of trauma [4], and in combination with elastography
Introduction may provide information regarding the nature of testicular
and extratesticular tumors [5].
Ultrasound is the preferred imaging modality for evaluating
the male genital tract. Normal anatomy can be readily visual-
ized using grayscale imaging, and Doppler provides valuable Technique
additional information regarding blood flow in the setting
Patient Positioning
Scrotal ultrasound is best performed with the patient supine
Electronic Supplementary Material The online version of this chapter and the scrotum elevated by a towel placed between the
(https://doi.org/10.1007/978-3-030-56802-3_15) contains supplemen-
tary material, which is available to authorized users. thighs. The penis is placed on the abdomen and covered with
another towel to improve visualization of the scrotum and
maximize comfort during imaging.
J. H. Squires (*)
Department of Radiology, UPMC Children’s Hospital of
Pittsburgh, University of Pittsburgh School of Medicine, Ultrasound Transducer Selection
Pittsburgh, PA, USA The highest frequency linear array transducer that will allow
e-mail: judy.squires@chp.edu
adequate depth penetration of the entire scrotal contents is
H. J. Paltiel optimal, usually in the range of 7.5–18 MHz. A lower-fre-
Division of Ultrasound, Department of Radiology, Boston Children’s
Hospital and Harvard Medical School, Boston, MA, USA

© Springer Nature Switzerland AG 2021 629

H. J. Paltiel, E. Y. Lee (eds.), Pediatric Ultrasound, https://doi.org/10.1007/978-3-030-56802-3_15
630
quency transducer should be used if greater depth penetra-
tion is needed, as in the setting of scrotal swelling.
Imaging Approaches
Testicular images are obtained in transverse and sagittal
planes. Color and pulsed Doppler settings should be opti-
mized for the detection of low flow in the testes and intra-
scrotal structures [1]. Transverse grayscale and color Doppler
images that include both testes are acquired to assess for sym-
metry. Both static images and cine clips should be obtained.
When evaluating the testis for patency of the testicular artery,
the Doppler sample volume should be placed in the center of
the testis since the periphery contains both capsular branches
of the testicular artery as well as branches of the cremasteric
and deferential arteries, as described in greater detail below.
Testicular volume measurement and the size and appear-
ance of the epididymis should be compared on each side. The
scrotum should also be evaluated for extratesticular masses,
fluid collections, and other abnormalities. Additional tech-
niques such as the Valsalva maneuver or upright positioning
are useful for the diagnosis of inguinal hernia or varicocele.
In the setting of a new right-sided varicocele, evaluation of
the right retroperitoneum should be considered. This will be
described in greater detail later in this chapter.
Normal Development and Anatomy
Normal Development
The development of the male genital tract is closely associ-
ated with development of the urinary tract and, prior to the
sixth week of gestation, is similar in males and females. In
response to the sex-determining region of the Y chromosome
(SRY), male development occurs and anti-müllerian hor-
mone is created, which causes regression of the müllerian
ducts and male differentiation [6].
Remnants of the müllerian ducts include the appendix tes-
tis and prostatic utricle, detailed later. The testes form from
the paired mesonephric (wolffian) ducts in the genital ridges,
with a complex interplay between genetic and hormonal fac-
tors influencing development, including the SRY protein and
testosterone secreted by Leydig cells [6, 7]. The mesoneph-
ric ducts eventually regress, leaving the appendix epididymis
as a persistent remnant.
The testes begin to form in the retroperitoneum and
descend into the scrotum prenatally with their accompany-
ing vascular supply. This occurs under the influence of a lig-
amentous cord, the gubernaculum, which extends from the
embryonic testis superiorly to the genital swelling (future
scrotum) inferiorly [6]. Testicular descent occurs in two
phases: transabdominal and inguinoscrotal, with the former
dependent on insulin-like hormone 3 and the latter depen-
dent on androgens, among other factors [7, 8]. In the seventh
J. H. Squires and H. J. Paltiel
Abdominal
Processus musculature
vaginalis Testicle Peritoneum and fascia
Gubernaculum
Future
scrotum
Fig. 15.1 Diagram of testicular descent during development. The tes-
ticle is pulled by the gubernaculum through the inguinal canal into the
future scrotum, typically by the 35th week of gestation
to 12th week of gestation, the gubernaculum shortens, pull-
ing the testis along with the ductus deferens and vessels infe-
riorly into the scrotum through the inguinal canal (Fig. 15.1)
[7–9]. An extracorporeal position of the testes is necessary
for normal spermatogenesis.
Although the testes are typically located in the scrotum
by the 35th week of gestation [9], a hormonal surge occurs
between 2 and 4 months after birth, which may stimulate
spontaneous descent. After 6 months of age, spontaneous
descent is unlikely. Failure of testicular descent either pre-
natally or in early postnatal life is thought to be related to
insufficient androgen stimulation.
At the same time as testicular descent is occurring, the
processus vaginalis, an outpouching of the peritoneum, begins
to form the inguinal canal anterior to the lower aspect of the
gubernaculum. The processus vaginalis elongates, which
allows the testis to descend into the scrotum [3, 6, 10]. During
its inferior descent, the processus vaginalis pushes out fascial
layers of the three abdominal wall muscles to form the inter-
nal spermatic fascia of the spermatic cord (from the transver-
salis fascia), the cremasteric fascia (from the internal oblique
muscle fascia), and the external spermatic fascia (from the
external oblique muscle fascia).
The upper portion of the processus vaginalis that extends
from the internal inguinal ring to the upper pole of the testis
normally fibroses and obliterates within the first year after
birth [6, 10]. The processus vaginalis first closes superiorly,
at the level of the internal ring, followed by closure inferiorly
just above the testis, and finally closes in its midportion over-
lying the spermatic cord (i.e., the funicular process) [11].
The lowermost portion of the processus vaginalis per-
sists and forms the tunica vaginalis, which is a closed pouch
within each hemiscrotum, partially folded around the testis
15 Male Genital Tract 631

anteriorly. Only the posterior aspect of the testis is not in
continuity with the tunica vaginalis, at the site of attachment
of the testis and epididymis.
Normal Anatomy
Scrotum
The scrotum is a fibromuscular sac divided by a midline raphe
into a right and left hemiscrotum. Each hemiscrotum contains a
testis, epididymis, spermatic cord, blood vessels, and lymphatics.
The layers of the scrotal wall include the epidermis, dartos mus-
cle, dartos fascia, external spermatic fascia, cremasteric muscle,
and fascia, and internal spermatic fascia [6]. On ultrasound, the
scrotal wall should be between 3 and 6 mm in thickness [1].
Testes
Each testis is suspended within a mesothelial-lined sac, the
tunica vaginalis. The left testis usually lies slightly lower than
the right. A small amount of fluid is usually present within the
tunica vaginalis [3]. The outer, parietal layer of the tunica vagi-
nalis lines the wall of the hemiscrotum and is attached to the
testicular fascia. The inner, visceral layer of the tunica vagina-
lis covers the testis, epididymis, and lower spermatic cord. The
tunica vaginalis separates the testis from the scrotal wall except
for a small zone located posteriorly, as noted above [6, 10].
The protective fibrous capsule of the testis, the tunica
albuginea, lies directly beneath the visceral layer of the
tunica vaginalis. Along the posteromedial aspect of the testis,
the tunica albuginea projects into the testis to form a fibrous
septum, the mediastinum. The mediastinum testis provides
support for the testicular arteries, veins, and ducts.
Numerous fibrous septations radiate into the testicular
parenchyma from the mediastinum, dividing the testis into
several hundred lobules [1]. The lobules contain seminifer-
ous tubules that drain to the rete testis located in the medias-
tinum. The seminiferous tubules converge centrally to form
the larger straight tubules or tubuli recti, which in turn form
a network of channels near the mediastinum testis known as
the rete testis. The rete testis drains into efferent ductules,
which extend into the head of the epididymis (Fig. 15.2).
On ultrasound, the testes are ovoid structures of uniform
echogenicity that increases with age from infancy to puberty.
Normal testes are ovoid in shape, and are homogeneous and
symmetric in echogenicity and vascularity. These changes
in echogenicity reflect the maturation of both the germ cells
and seminiferous tubules. The fibrous septa may be seen as
linear hypoechoic bands. In older children and adolescents,
the mediastinum testis appears as a linear echogenic struc-
ture extending craniocaudally through the testis (Fig. 15.3).
The rete testis is sometimes identified as a hypoechoic
zone near the mediastinum testis. The tunica albuginea is
depicted as a thin, continuous, echogenic line surrounding
the testis [12]. The tunica vaginalis and the potential space
between its layers are not normally identified. A trans-testic-

Head of
epididymis

Efferent
ductules Cavity of tunica
vaginalis
Body of
epididymis Seminiferous
tubules
Epididymis
Tunica
Testicular artery vaginalis

Testicular vein Rete testis

Parietal layer of Straight
tunica vaginalis seminiferous
testis tubules
Ductus deferens
Septa testis
Tail of epididymis
Visceral layer
of tunica
Tunica albuginea vaginalis testis
of testis

Fig. 15.2 Diagram of the normal anatomy of the testis and surrounding structures
632 J. H. Squires and H. J. Paltiel

ular or trans-mediastinal vessel can sometimes be seen as a Table 15.1 Reference values of mean testicular volume for age
hypoechoic linear structure extending through the testis. Age Volume (mL) ± SD
At strain elastography, the postpubertal testis has been 0 months 0.27 ± 0.02
described as having a “three-ring” appearance, with the cen- 5 months 0.44 ± 0.03
tral testis the least stiff and the peripheral portion the stiffest 9 months 0.31 ± 0.02
12 months 0.48 ± 0.1
(Fig. 15.4). Correspondingly, at 2D shear wave elastography
2 years 0.46 ± 0.09
(SWE), the central testis will have the lowest shear-wave speeds, 3 years 0.51 ± 0.15
while the periphery will have higher shear-wave velocities. Exact 4 years 0.51 ± 0.16
values will vary depending on what equipment is used [13]. 5 years 0.58 ± 0.15
Testicular size generally increases with age and is sym- 6 years 0.63 ± 0.26
metric [14–16]. Reference volumes according to age are 7 years 0.65 ± 0.17
provided in Table 15.1. Testicular volumes in boys born pre- 8 years 0.66 ± 0.22
9 years 0.79 ± 0.46
maturely are similar to those born at term [15]. At the time of
10 years 0.97 ± 0.51
birth, testicular volume averages 0.27 mL (± 0.02) [14]. The 11 years 1.33 ± 1.03
volume increases over the first 5 months of life to a mean vol- 12 years 2.33 ± 1.77
ume of 0.44 mL (± 0.03), then decreases to 0.31 mL (± 0.02) at 13 years 4.42 ± 2.66
9 months of life. This volume peak correlates with the physi- 14 years 7.31 ± 4.11
ologic so-called mini-puberty, which occurs due to a peak in 15 years 8.69 ± 2.91
gonadotropic hormones [14]. 16 years 11.51 ± 3.03
17 years 12.12 ± 2.8
From 9 months to 6 years of age, testicular volumes do
18 years 13.73 ± 3.51
not substantially change. After the first decade of life, and
SD, Standard deviation
Created from Kuijper EA, van Kooten J, Verbeke JI, van Rooijen M,
Lambalk CB. Ultrasonographically measured testicular volumes in 0- to
6-year-old boys. Hum Reprod. 2008;23(4):792–6; Goede J, Hack WW,
Sijstermans K, van der Voort-Doedens LM, Van der Ploeg T, Meij-de Vries
A, et al. Normative values for testicular volume measured by ultrasonog-
raphy in a normal population from infancy to adolescence. Horm Res
Paediatr. 2011;76(1):56–64; Sotos JF, Tokar NJ. Appraisal of testicular
volumes: volumes matching ultrasound values referenced to stages of
genital development. Int J Pediatr Endocrinol. 2017;2017:7. [14–16]

particularly around puberty, the testicular volume increases

substantially, with larger differences in testicular volume
between patients in this age group.

Fig. 15.3 Normal testis in a 9-year-old male. Sagittal grayscale ultra-
sound image shows the normal ovoid shape of the testis with homoge-
neous echogenicity and linear echogenic mediastinum testis (arrow)
Epididymis
The epididymis is a tubular structure that overlies the testis,
with its head (globus major) located at the superior aspect
of the testis, and the body and tail (globus minor) along the
posterolateral margin of the testis, extending to the inferior
aspect of the testis. The epididymis is covered by the vis-
ceral layer of the tunica vaginalis, except for a small poste-
rior region, as previously described [3]. On ultrasound, the
epididymis should be homogeneous in echogenicity, similar
to or slightly hypoechoic compared to the testis [1].

Fig. 15.4 Normal transverse strain elastogram of the right testis in an
8-year-old male. The testis is softest in its central portion and stiffest at
the periphery. Color key: S, Soft (red); H, hard (blue)
Spermatic Cord
The vas (or ductus) deferens is a continuation of the epididy-
mis and is located posterior to the testis and epididymis. The
vas deferens and deferential (or ductal) artery, lymphatics,
pampiniform (or venous) plexus, and testicular artery along
with the genitofemoral nerve together comprise the sper-
matic cord, which is surrounded by the cremaster muscle
and fascia [3]. At ultrasound, the spermatic cord appears as a
linear echogenic band in the inguinal canal (Fig. 15.5) [12].
15 Male Genital Tract
Testicular Appendages
Both the epididymis and testis may have appendages that can
be sessile or pedunculated [17], and are typically best visual-
ized in the presence of a hydrocele. The appendix testis is
thought to be a remnant of the abdominal portion of the invo-
luted müllerian duct and is always found at the superior por-
tion of the testis or between the testis and epididymal head.
It is seen in up to 92% of males at autopsy [3]. The testicular
appendage diameter varies from 1 to 7 mm [18]. The appen-
dix epididymis is thought to be a remnant of the involuted
cranial portion of the wolffian duct and is always found at
the head of the epididymis. It occurs in less than 30% of
males [17]. Epididymal appendages may be slightly larger
than testicular appendages, usually measuring 2–8 mm in
diameter [18].
At ultrasound, a normal appendage can be challenging to
visualize without the presence of a hydrocele, discussed later
in this chapter. The normal appendix testis is usually ses-
sile and is situated between the epididymis and testis, while
the epididymal appendage is usually pedunculated and arises
from the epididymal head. Both appendages are isoechoic to
the head of the epididymis and measure less than 6 mm in
diameter (Fig. 15.6) [12, 19].
Fig. 15.5 Normal spermatic cord in a 2-month-old male. Sagittal gray-
scale ultrasound image demonstrates the normal linear echogenic band-
like appearance of the spermatic cord (arrow) in the inguinal canal
Fig. 15.6 Normal testicular appendage in a 1-year-old male. Split-
screen grayscale ultrasound images obtained in the sagittal (left) and
transverse (right) planes show the appendage (arrows) surrounded by a
hydrocele (asterisks)
633
Blood Supply
The testes are primarily supplied by the testicular artery
(Fig. 15.7), which arises from the infrarenal abdominal aorta and
joins the spermatic cord at the deep inguinal ring, entering the
testis at its posterior surface. In about half of all males, a small
branch of the testicular artery runs centrally along the mediasti-
num testis, often accompanied by a vein. In the other half, there
is no transmediastinal artery, and the testicular artery branches
into capsular arteries, which course along the periphery of the
testis deep to the tunica albuginea, and then extend into the tes-
ticular parenchyma toward the mediastinum (Fig. 15.8) [3].
A small amount of arterial blood flow is contributed to
the testis by the cremasteric artery that arises from the infe-
rior epigastric artery, and from the deferential artery, which
arises from the superior vesical artery. These two arteries
also supply the epididymis and vas deferens [3]. The scrotal
Aorta
Testicular
artery
Internal
Inferior iliac artery
epigastric
artery
Deferential
artery
Cremasteric
artery
Testicular
artery
Vesicular
artery
Fig. 15.7 Diagram of the normal arterial supply to the testis. The
major contribution is from the testicular artery which arises from the
aorta, with additional flow supplied by branches of the internal iliac
artery and inferior epigastric artery
634 J. H. Squires and H. J. Paltiel

Testicular
artery
Vas deferens
artery
Cremasteric artery

Posterior
Epididymal epididymal artery
artery

Anterior
epididymal
artery
Artery to vas
deferens

Mediastinal
artery Recurrent rami
Fig. 15.10 Normal spectral Doppler arterial waveforms from the testis
Capsular
of a 2-year-old male. Sagittal color Doppler ultrasound image with
artery
spectral analysis demonstrates a high-resistance arterial waveform in
Centripetal the mediastinal artery with absent diastolic flow
artery

Fig. 15.8 Diagram of the arterial supply to the testis and intratesticular
branches. The testicular artery divides into capsular arteries that extend
into the testicular parenchyma. In about 50% of all males, a mediastinal
artery is seen centrally

Fig. 15.9 Normal testes in a 9-year-old male. Transverse color Doppler

ultrasound image demonstrates normal bilateral mediastinal arteries
(arrowheads)

wall is primarily supplied by the internal pudendal artery that

arises from the internal iliac artery.
Testicular and mediastinal arteries may be relatively incon-
spicuous at color Doppler imaging, particularly in prepuber-
tal boys whose testes have relatively low flow (Fig. 15.9).
The normal intratesticular arterial waveform varies with
the developmental stage of the patient. In the prepubertal
child, the testes often have little to no detectable diastolic
flow, with a relatively high-resistance arterial waveform
(Fig. 15.10). At puberty and thereafter, normal testes have
arterial diastolic flow throughout the cardiac cycle with a
lower resistance waveform (Fig. 15.11) [20, 21].
Venous drainage is via the pampiniform plexus that forms
at the superior aspect of the epididymis, extends into the sper-
matic cord, and continues superiorly as the testicular vein
Fig. 15.11 Normal spectral Doppler arterial waveforms from the testis of
a 9-year-old male. Sagittal color Doppler ultrasound image with spectral
analysis demonstrates flow throughout the cardiac cycle, with lower resis-
tance waveforms compared to those of a younger male (see Fig. 15.10)
(Fig. 15.12). The right testicular vein drains directly into the
inferior vena cava, while the left testicular vein drains into
the left renal vein. Normal testicular veins are generally less
than 3 mm in diameter [22].
As with the testicular arteries, optimization of low flow
color and spectral Doppler parameters may be necessary to
visualize the testicular veins in prepubertal boys at ultra-
sound, because there is relatively little testicular blood flow
(Fig. 15.13).
15 Male Genital Tract 635

Anatomical Variants

Testicular Appendages
An accessory appendage may be encountered in the same
location as both the testicular and epididymal appendages,
near the upper pole of the testis or near the head of the epidid-
ymis, and is believed to be a remnant of a cystic mesonephric
tubule. Multiple testicular and epididymal appendages can
occur; this is seen most often with epididymal appendages
(Fig. 15.14) [17].
Inferior
Left renal vein
vena cava
Vessels
Testicular
vein Variations in the origin and course of the testicular arteries
and veins can be seen [23, 24]. An awareness of these vari-
Internal ants is of importance to surgeons in order to avoid inadver-
iliac vein
tent intraoperative trauma.

Vesicular vein

Pampiniform
plexus

Paradidymis

Fig. 15.12 Diagram of the normal venous drainage of the testis via the
pampiniform plexus and the testicular vein. The right testicular vein
drains directly into the inferior vena cava while the left testicular vein
drains into the left renal vein Vas deferens

Epididymis

Epididymal
appendage

Testis

Testicular
appendage

Fig. 15.13 Normal spectral Doppler venous waveforms from the testis Fig. 15.14 Diagram of possible locations of intrascrotal appendages
of a 16-year-old male. Sagittal color Doppler ultrasound image with
spectral analysis demonstrates low-velocity flow in a parenchymal vein
636
Congenital Anomalies
Testicular Agenesis
Failure of testicular development may be associated with
other abnormalities of the genitourinary tract [25]. True
testicular agenesis is uncommon. Absence of a testis within
the scrotum is more commonly related to failure of descent
or the so-called vanishing testis [26] (also called testicular
regression syndrome), both of which are detailed later in this
chapter.
Cryptorchidism
Cryptorchidism refers to a testis that is not located in the scro-
tum. An undescended testis may be located anywhere along the
course of the gubernaculum testis from the retroperitoneum to
the inguinal ring (Fig. 15.15). In 75–80% of cases, the testis is
located in the inguinal canal, with the remainder located in the
abdomen [27]. There is debate regarding the utility of ultrasound
in the evaluation of cryptorchidism, [8, 9, 28], particularly when
searching for an intra-abdominal location of an undescended
testis. Cryptorchidism may lead to diminished vascularity of
the undescended testis, there may be testicular hypotrophy, and
there is an increased risk of malignancy [8, 9, 29].
Cryptorchidism
True Ectopic
Abdominal Prepenile
Inguinal Superficial
ectopic
Suprascrotal
Femoral
Transverse
scrotal
Perineal
Fig. 15.15 Diagram of potential sites of cryptorchid testes. In true
cryptorchidism, testicular descent is arrested along the course of the
gubernaculum. In testicular ectopia (false cryptorchidism), the testis
does not descend along the course of the gubernaculum
J. H. Squires and H. J. Paltiel
B
Fig. 15.16 Cryptorchidism in a newborn male. Sagittal grayscale
ultrasound image of the left lower pelvis just above the inguinal canal
demonstrates an undescended testis identified by the echogenic medias-
tinum testis (arrowhead) and located adjacent to loops of bowel (B)
At ultrasound, a cryptorchid testis is most commonly
located in the inguinal canal. The undescended testis is usu-
ally smaller and more hypoechoic than a normal intrascrotal
testis. Visualization of the mediastinum testis may be helpful
to confirm that an inguinal soft tissue structure is, in fact, a
cryptorchid testis (Fig. 15.16) [30].
Treatment for cryptorchidism can be hormonal and/or
surgical [8]. If surgical orchiopexy is performed as treat-
ment, it should be done by 2 years of age to prevent testicular
hypotrophy.
Anorchidism
Anorchidism is defined as the absence of one testis (mon-
orchidism) or both testes (testicular regression syndrome),
and is rare. It may result from partial or complete failure of
formation of the gonadal ridge, lack of hormonal stimula-
tion, or a vascular insult resulting in cessation of develop-
ment [31, 32] and may be associated with other anomalies
[25]. Anorchidism can be associated with disorders of sex
development where there is a 46,XY karyotype but testes
are absent, including androgen insensitivity syndrome [33].
Monorchidism is unilateral absence of a testis, and may
be congenital or acquired. When congenital, it is thought
to be due to an in utero insult (such as testicular torsion)
more often than a lack of formation. Non-congenital etiolo-
gies include regression following a postnatal insult (detailed
below), or surgical resection. The remaining testis typically
undergoes compensatory hypertrophy.
 esticular Regression Syndrome
T
Testicular regression syndrome comprises several disorders
that have in common the absence, significantly reduced size,
15 Male Genital Tract
or involution of both testes. These include rudimentary tes-
tes, congenital bilateral anorchidism, vanishing testis syn-
drome, and Sertoli cell-only syndrome [26, 34, 35].
At ultrasound, one or both testes will be small or absent.
Treatment is controversial. If there is a testicular remnant,
some urologists recommend surgical exploration, while oth-
ers recommend non-operative management. Testicular pros-
thesis placement is a consideration and hormone replacement
therapy may be necessary [33].
Testicular Hypoplasia
Testicular hypoplasia is a term used to describe a small tes-
tis, and there are many potential etiologies. Endocrine dis-
orders include Klinefelter syndrome, Kallman syndrome,
Prader-Willi syndrome, and hormone-secreting pituitary
tumors [36]. Testicular disorders include undescended testis,
varicocele, compressive hydrocele, prior infection or infarc-
tion (including torsion), and prior partial surgical excision
(Fig. 15.17) [36]. Treatment depends on etiology.
Polyorchidism
Polyorchidism refers to the presence of supernumerary testes
(more than two) and is rare. Most often, supernumerary tes-
tes are left-sided (65%) although they may be bilateral (4.3%),
with the remainder of cases occurring on the right [37]. The
etiology may be related to an abnormal division of the genital
ridge [38]. The most common time of presentation is in the late
teenage years, and is usually an incidental finding.
The supernumerary testis may be located superior to the
testis (including in the inguinal canal) or inferior to the tes-
tis, while some occur at a similar level to the normal testis.
Most supernumerary testes are located within the scrotum
[38]. There are reports of absent epididymides in cases of
polyorchidism, although spermatogenesis is usually normal
in these patients. Neoplasm may occur in the supernumerary
testis [37]. They are also more mobile than normal testes and
therefore have an increased rate of torsion.
At ultrasound, the appearance of a supernumerary testis is
identical to that of a normal testis, with similar echogenicity,
echotexture, and vascularity (Fig. 15.18) [39]. Importantly,
there is normal-appearing testicular tissue in both the ipsi-
lateral and contralateral hemi-scrotum or inguinal canal;
otherwise, consideration should be given to a diagnosis of
transverse testicular ectopia (detailed later).
Although no guidelines currently exist, when a supernu-
merary testis is diagnosed at ultrasound, treatment involves
orchiectomy of the supernumerary testis, typically the smaller
testis and/or extra-scrotal testis, because of the associated risks
of torsion and malignancy. Close clinical follow-up of polyor-
chidism is recommended, including evaluating relevant tumor
serum markers and performance of follow-up ultrasound stud-
ies. Histologic confirmation of a supernumerary testis may be
necessary to exclude other paratesticular masses, including
tumor, particularly when the diagnosis is unclear.
637
Fig. 15.17 Atrophic testis in a 12-year-old male with a history of right
inguinal herniorrhaphy in infancy. Sagittal grayscale ultrasound image
of the right inguinal canal demonstrates the remnant right testis (arrow)
Fig. 15.18 Polyorchidism in a 10-year-old male. Sagittal grayscale
ultrasound image demonstrates the presence of two testes (arrows) in
the right hemiscrotum, with the inferior testis smaller than the superior
testis
Testicular Ectopia
An ectopic testis is another rare developmental anomaly,
where testicular descent does not follow the typical course
of the gubernaculum testis from the retroperitoneum into
the scrotum. The most common ectopic site is located
between Scarpa’s fascia and the external oblique fascia
superior to the external inguinal ring (Fig. 15.15) [40].
Other sites include femoral, pubic, penopubic, penile, and
perineal locations.
A very rare form of ectopic testis is transverse testicular
ectopia (also called “crossed testicular ectopia”) where the
testis descends into the contralateral inguinal ring and both
testes are located on the same side, with an empty contralat-
eral hemiscrotum. This condition usually presents by the age
of 4 years, and there is an association with inguinal hernia,
malignancy, and persistent müllerian duct syndrome, the lat-
ter characterized by the presence of a uterus or fallopian tube
in a 46,XY otherwise phenotypic male [41].
At ultrasound, normal-appearing testicular tissue will
be located in an abnormal position and close evaluation for
associated inguinal hernia or mass should be performed.
Testicular ectopia is generally treated surgically, since
fibrous attachments prevent spontaneous descent into a nor-
mal scrotal position and the ectopic testis is at increased
risk of malignancy. Inguinal exploration with orchiopexy or
trans-septal contralateral orchiopexy is performed.
638 J. H. Squires and H. J. Paltiel

 ystic Dysplasia of the Rete Testis

C a
Cystic dysplasia of the rete testis is a rare benign condition
[42]. This entity is different from, and should not be confused
with, ectasia of the rete testis, which occurs with high fre-
quency in middle-aged and elderly adult men [43]. The etiol-
ogy of cystic dysplasia of the rete testis is unknown but it is
postulated that there is disruption between the normal inter-
action of the wolffian duct and the rete tubules [30, 42]. This
failed connection causes development of small cysts with
non-obstructed ductal dilation in the mediastinum testis.
There are often associated ipsilateral urogenital tract
anomalies, such as cystic dysplasia of the kidney or renal
agenesis that are thought to be due to the same pathologic b
mechanism [42, 44]. This is one potential etiology of pain-
less testicular enlargement [30].
At ultrasound, multiple cysts of varying size and shape
will be encountered along the mediastinum testis. There are
no solid components to the cysts, which is an important dis-
tinction from cystic testicular neoplasm, and serum tumor
markers will be normal. If cysts are tiny and only the cyst
walls are seen, they may mimic echogenic foci, although
with the use of linear high-frequency transducers and with
the improved image quality of newer generation ultra- Fig. 15.19 Discontinuous splenogonadal fusion in a 6-year-old male.
sound equipment, this distinction may be easier to make. (a) Sagittal grayscale ultrasound image demonstrates splenic tissue
Depending on the size of the cysts, the testicular tissue sur- (arrowhead) superior to the left testis (arrow). (b) Sagittal color Doppler
rounding the mediastinum testis may be quite reduced. ultrasound image demonstrates splenic tissue (arrowhead) with a cen-
tral feeding vessel (asterisk) superior to the left testis (arrow)
Evaluation of the ipsilateral genitourinary tract should be
performed to evaluate for associated renal cystic dysplasia and
agenesis. Cysts may resolve spontaneously, although mass Most often, splenic tissue is present in the scrotum, and this
effect on adjacent testicular tissue may necessitate surgical condition presents as a scrotal mass [1].
intervention. At ultrasound, the fibrous cord may be seen connecting
the spleen in the left upper abdomen to the scrotum, with
Splenogonadal Fusion the testis visible separately. Doppler evaluation may demon-
Splenogonadal fusion is rare, and occurs more often in strate a central vessel within the fibrous cord arising from the
males than females although it may be underdiagnosed in splenic vasculature (Fig. 15.19).
females due to the intrapelvic location of the ovaries [1, 45]. Surgical exploration is generally required to exclude
This congenital anomaly is caused by abnormal fusion of malignancy, which allows for definitive diagnosis as well as
splenic tissue to the gonadal-mesonephric structures [46], surgical removal of the ectopic splenic tissue. Orchiectomy
thought to be related to an in utero insult that results in infe- is not necessary.
rior descent of a portion of splenic tissue with descent of The discontinuous type of splenogonadal fusion is a vari-
the testis. ant of a splenule, with accessory splenic tissue located in
There is an association between splenogonadal fusion, the scrotum and attached to the testis within the tunica albu-
undescended testis, and inguinal hernia, since the presence of ginea. There is no connection of the accessory tissue to the
the abnormal splenic tissue may interfere with appropriate tes- orthotopic spleen. This type of splenogonadal fusion is rarely
ticular descent and/or closure of the processus vaginalis. associated with other anomalies. It may be difficult to distin-
Two types of splenogonadal fusion have been described: guish discontinuous splenogonadal fusion from malignancy.
continuous and discontinuous [46]. In the continuous type, Evaluating serum tumor markers may be clinically helpful.
the testis is linked to the spleen by a fibrous cord that extends Evaluation and treatment involve exclusion of malignancy
from the superior aspect of the spleen to the testis. There and resection of the accessory splenic tissue. Typically, the
is an association between continuous splenogonadal fusion testis can be spared and left in place if the diagnosis is pre-
and other lesions, including limb deficiency anomalies, dia- operatively suspected, although a diagnosis of splenogonadal
phragmatic hernia, micrognathia, and other abnormalities. fusion is usually made at pathology after resection.
15 Male Genital Tract 639

 ell Clapper Deformity

B
Bell clapper deformity is a congenital anomaly where the
testis and epididymis are freely suspended in the scrotum
within the tunica vaginalis [47]. It occurs in up to 12% of
males [3], and is thought to be related to an abnormally high
attachment of the tunica vaginalis to the spermatic cord [48].
The tunica vaginalis completely encircles the epididymis,
rather than attaching normally to the posterolateral aspect of
the testis and epididymis. This results in a shortened pedicle
of attachment to the testis, increasing the likelihood of twist-
ing. There may be an abnormal attachment of both the epi-
didymis and testis, and its presence is the primary risk factor
for intravaginal testicular torsion (detailed below).
Identification of a bell-clapper deformity is occasionally
made fortuitously in the setting of a hydrocele that is noted to
completely encircle the testis and epididymis (Fig. 15.20 and
Video 15.1). This rare situation permits planning of elective
surgical orchiopexy to prevent torsion.

Acute Scrotal Pain

The most common causes of acute scrotal pain include testic-

ular torsion, torsion of the testicular and epididymal append-
ages, epididymitis, and epididymo-orchitis [1]. Table 15.2
highlights important differences in these diagnoses. Fig. 15.20 Bell clapper deformity in a 13-year-old male. Transverse
grayscale ultrasound image of the left scrotum shows a hydrocele
Testicular Torsion (asterisk) with fluid completely encircling the left testis except at its
Testicular torsion is an emergency, requiring surgical or manual pedicle of attachment (arrowhead)
detorsion as soon as possible. Because the clinical diagnosis
may be challenging, ultrasound is crucial for differentiating tes-
ticular torsion from other etiologies of scrotal pain.

Table 15.2 Causes of acute scrotal pain in children, typical age and clinical presentation, ultrasound appearance, and complications
Etiology Clinical presentation Ultrasound appearance Complications
Intrascrotal appendage 7–14 years most common Ovoid avascular focus adjacent to testis or Development of scrotal
torsion “Blue dot” sign epididymis pearl
Preserved cremasteric reflex Surrounding hyperemia
Testicular torsion Enlarged, heterogeneous testis
Neonatal period or adolescence Complete or segmental
Sudden onset of nausea/ Increased visibility of testicular lobar anatomy testicular infarction
vomiting Epididymal head enlargement and cystic areas
Transverse and/or high without hyperemia
testicular lie Reactive hydrocele with bell clapper deformity
Absent cremasteric reflex Twisted or “whirlpool” spermatic cord
Decreased or absent Doppler flow
Hyperemia in setting of reperfusion in torsion/
detorsion
Absent or reversed diastolic flow
Pudendal hyperemia (“halo” of surrounding
hypervascularity)
Epididymitis or Common in males >18 years of Epididymal enlargement, hypervascularity, Epididymal and/or
epididymo-orchitis age particularly of the tail testicular abscess
Associated with voiding Associated testicular hyperemia and low resistance Pyocele
dysfunction in boys arterial waveforms if concomitant orchitis
< 5 years Spermatic cord may be inflamed
640
In the pediatric population, torsion typically occurs in a
bimodal distribution: in the neonatal period and in adoles-
cence (Fig. 15.21) [48]. In the neonatal period, the twisting
typically occurs at the level of the external inguinal ring, so-
called extra-vaginal (or supravaginal) testicular torsion. The
scrotal contents are only loosely attached to the scrotal wall
and inguinal canal in utero and in the newborn period [47].
In adolescents and young adults, a bell-clapper deformity
permits twisting of the spermatic cord structures, so-called
intravaginal testicular torsion. Preceding trauma is thought
to be an underlying etiology of torsion in up to 8% of cases
[49]. Torsion can also be associated with a long mesorchium,
an anatomical variant commonly occurring in association
with cryptorchidism. Testicular torsion is further classified
according to time of presentation, with acute torsion present-
ing less than 24 hours after the onset of symptoms, subacute
(or missed torsion) presenting at 1 to 7–10 days, and chronic
torsion at more than 10 days.
There are varying degrees of twisting, ranging from 90
degrees to 1080 degrees if multiple twists are present. Clinical
symptoms and signs include acute onset of pain, nausea, trans-
verse testicular lie, high-riding testis, and absence of the crem-
asteric reflex.
Intravaginal
torsion
Twisted cord
a b
Tunica
vaginalis
Fig. 15.21 Diagram of different types of testicular torsion. (a) Intravaginal torsion. (b) Extravaginal torsion. (c) Torsion related to a long
mesorchium
J. H. Squires and H. J. Paltiel
Grayscale ultrasound imaging findings of acute tes-
ticular torsion include an enlarged, heterogeneous, and
hypoechoic testis without hyperemia, especially if symp-
toms have been present for at least 4 hours. There may be
increased conspicuity of testicular lobar anatomy due to
septal edema; scrotal wall thickening/edema; enlargement
and edema of the epididymis without hyperemia; and reac-
tive hydrocele.
Visualization of a redundant or twisted spermatic cord or
the “whirlpool” sign has high specificity for testicular tor-
sion but may not be as sensitive as other abnormalities [47].
An anteromedially directed mediastinum testis and epididy-
mis can indicate abnormal testicular fixation. Horizontal or
oblique lie of the testis and changes in testicular position
may be noted during the imaging examination in cases of
intermittent torsion.
The appearance of the epididymis may be helpful when
evaluating for testicular torsion [50]. The epididymis can be
enlarged, have a more mass-like or globular shape (rather
than a normal elongated shape), with enlargement, hetero-
geneity, hyperechogenicity, cystic spaces, and decreased or
absent vascularity. However, the epididymis may be hyper-
vascular, particularly in the acute setting. A large number of
Extravaginal
torsion
Torsion due
to long
mesorchium
c
15 Male Genital Tract
epididymal cystic structures may portend a lower likelihood
of testicular salvage [51].
Color and power Doppler imaging may show asymmetri-
cally decreased or absent perfusion of the affected testis, in
which case the diagnosis is less of a challenge. However, there is
variability in testicular blood flow in the setting of acute torsion.
Spectral Doppler findings depend on the degree of twist-
ing. Venous flow may be absent since venous compression
typically precedes arterial occlusion. There may be decreased
testicular artery velocity, absence of a dicrotic notch with a
monophasic waveform, absent or reversed diastolic flow,
or complete absence of vascular flow. Comparison with the
unaffected testis can be very helpful to evaluate for subtle
perfusion differences.
In subacute testicular torsion, there may be pudendal
hyperemia, resulting in a halo of hypervascularity sur-
rounding the testis. In intermittent torsion, there may be
hyperemia during periods of untwisting due to reperfu-
sion and associated low resistance arterial waveforms
(Fig. 15.22 and Video 15.2). In chronic testicular torsion,
the affected testis becomes smaller and stiffer than the
a b
c d
Fig. 15.22 Intermittent torsion (torsion/detorsion) in a 16-year-old
male. Sagittal color (a) and spectral Doppler (b) ultrasound images
demonstrate normal flow and arterial waveforms. (c) Transverse mid-
line color Doppler ultrasound image of the scrotum obtained several
641
unaffected testis, with hyperechogenicity and absent vas-
cular flow (Fig. 15.23).
There is early evidence that the addition of shear wave
elastography to grayscale and color Doppler ultrasound
may increase diagnostic confidence, with cases of testicu-
lar torsion demonstrating increased stiffness values [52].
CEUS may also aid in the diagnosis of testicular torsion,
with improved ability to detect decreased testicular blood
flow, particularly in neonates [53]. There may also be
improved visualization of segmental testicular infarction
related to intermittent testicular torsion with use of CEUS.
In both intra- and extravaginal torsion, rapid diagnosis is
imperative so that untwisting can be performed as quickly
as possible to ensure testicular salvage. If untwisting occurs
within 6 hours of clinical presentation, salvage rates are as
high as 100%. However, if untwisting occurs after 12 hours,
salvage rates are only up to 20% [1, 47].
Segmental Testicular Infarction
Segmental testicular infarction is uncommon, but can be
idiopathic or caused by testicular torsion, vasculitis, sickle
minutes later demonstrates absent flow to the left testis (asterisk) with
normal flow to the right testis (R). (d) Transverse grayscale ultrasound
image of the left scrotum obtained subsequently demonstrates a “cord
knot” (arrow) adjacent to the twisted left testis
642
a
Fig. 15.23 Chronic testicular torsion in a 1-day-old male. (a) Sagittal
grayscale ultrasound image demonstrates a heterogeneous appearance
of the left testis (arrow) with internal hypoechoic foci. Sagittal color (b)
cell disease, epididymo-orchitis, or trauma [54, 55]. It may
be arterial or venous in etiology, and is thought to be related
to vascular compression against the rigid tunica albuginea.
Most often, segmental infarction presents with acute testicu-
lar pain, which may be clinically challenging to distinguish
from other etiologies of testicular pain.
Arterial Segmental Testicular Infarction
On grayscale imaging, arterial segmental testicular infarction
will most often appear as a focal, hypoechoic, wedge-shaped
zone confined to a lobule of seminiferous tubules. On color
or power Doppler imaging, there will be no internal vascu-
lar flow. This absence of flow helps to distinguish infarction
from tumor, which generally demonstrates internal vascular-
ity [54, 56]. CEUS may be helpful to confirm the absence of
flow to the infarcted parenchyma [53, 55, 57].
If evaluated in the subacute phase, there may be periph-
eral hyperemia surrounding the infarct, which can make the
J. H. Squires and H. J. Paltiel
c
and spectral Doppler (c) ultrasound images demonstrate no flow to the
left testis (arrow) with absence of vascular waveforms
distinction from an abscess challenging. Absence of pos-
terior through transmission on grayscale imaging can help
to distinguish segmental infarction from abscess as well as
the absence of any clinical signs or symptoms of infection.
Treatment is non-operative, with supportive conservative
management. There will be gradual resolution of symptoms
and the lesion will either resolve or remain unchanged on
follow-up ultrasound studies.
Venous Testicular Infarction
Venous outflow obstruction can be caused by epididymi-
tis or epididymoorchitis (Fig. 15.24). Trauma can lead to
testicular compression by a hematocele or hematoma with
subsequent venous thrombosis and infarction [47], includ-
ing segmental testicular infarction [54]. Venous infarction
can also occur in hypercoagulable states. On ultrasound,
a venous testicular infarct may have a more rounded con-
figuration than an arterial infarct. CEUS may be helpful
15 Male Genital Tract
a b
c d
Fig. 15.24 Testicular venous outflow obstruction in a 17-year-old
male with urethral stricture causing bacterial epididymitis and pyocele.
(a) Transverse grayscale ultrasound image of both testes demonstrates
marked thickening of the left scrotal wall (asterisk) and heterogeneous
fluid (arrow) surrounding the left testis compatible with pyocele. (b)
Transverse color Doppler ultrasound image of both testes reveals
to confirm abnormal vascular flow by non-enhancement of
the infarcted testis [55, 57]. Treatment of global testicular
infarction is surgical in order to prevent complications.
Torsion of Testicular Appendages
The most common cause of acute scrotal pain in children is
torsion of the intrascrotal appendages, more often a testicu-
lar appendage than an epididymal appendage [18, 48, 53].
It is most frequently encountered between the ages of 7 and
14 years. On physical examination there may be a “blue dot”
sign due to the appearance of the appendage through the scro-
tal wall. The twisted appendage is often palpable, and the
cremasteric reflex is preserved [2, 18, 53].
As previously discussed, the testicular and epididy-
mal appendages are remnants of the paramesonephric and
643
hyperemia of the left scrotal wall. There is no flow to the left testis. (c)
Sagittal grayscale ultrasound image of the left testis shows heterogene-
ity of the testicular parenchyma with a contour abnormality (arrow-
head) of the tunica albuginea. (d) Sagittal color Doppler ultrasound
image with spectral analysis of the left testis demonstrates reversal of
arterial diastolic flow
mesonephric ducts, respectively. A normal appendage can
occasionally be identified by ultrasound, particularly when
outlined by scrotal fluid. The testicular appendage is usually
located on the medial side of the upper pole of the testis. A
twisted appendage has a variable ultrasound appearance over
time. Acutely, it will appear enlarged and hypoechoic with
echogenic internal foci (Fig. 15.25). After 24 hours, it typi-
cally becomes hyperechoic. Color Doppler will demonstrate
the absence of internal flow.
There may be an associated reactive hydrocele or scro-
tal wall thickening, occasionally with reactive epididymitis
and orchitis. This condition is self-limited and treatment is
symptomatic, including anti-inflammatory medication. The
appendage can subsequently calcify, resulting in a scrotal
pearl, a mobile intrascrotal body [58].
644
Fig. 15.25 Torsion of testicular appendage in a 3-year-old male. (a)
Sagittal grayscale ultrasound image demonstrates a heterogeneous
ovoid focus (arrow) above the left testis (asterisk). (b) Sagittal color
Inflammatory Disorders
 cute Epididymitis and Epididymo-orchitis
A Zones of epididymal inflammation are hypervascular, while
Epididymitis is inflammation of the epididymis and its
incidence is increasing [59]. While epididymitis is the
most common cause of painful scrotal swelling in patients
over 18 years of age [27], it is important to note that epi-
didymitis is uncommon in preadolescent boys and is less
common than testicular torsion in this age group. Care
should therefore be taken to ensure appropriate diagno-
sis [48]. In children less than 5 years of age, potential
infectious etiologies include viruses, Escherichia coli,
Pseudomonas, and Aerobacter that have spread from the
urinary tract [2, 48].
Reflux of sterile urine related to voiding dysfunction is
another recognized cause of epididymitis and can also be
associated with urinary tract malformations such as ectopic
ureter and hypospadias [59, 60]. In this younger population,
imaging evaluation with renal ultrasound and voiding cysto-
urethrography is recommended to evaluate for genitourinary
malformations and reflux into the ejaculatory ducts. In ado-
lescents, infectious etiologies, including sexually transmit-
ted infections such as Neisseria gonorrhoeae and Chlamydia
are most likely [53]. They lead to epididymitis either via ret-
rograde infection through the urethra or ductus deferens, or
via a hematogenous route [27].
Trauma, including iatrogenic injury, is a potential etiology at
any age [2]. Vasculitis and sarcoidosis are uncommon etiologies
[61]. Clinical symptoms include dysuria and epididymal tender-
ness to palpation. In up to 25% of cases, inflammation extends
to the adjacent testis, resulting in epididymo-orchitis [48].
J. H. Squires and H. J. Paltiel
Doppler ultrasound image depicts hyperemia of the left epididymal
head and testis, with no flow (arrow) to the testicular appendage
Ultrasound findings include enlargement of the epididymis,
altered epididymal and peritesticular hyperemia (Fig. 15.26).
hypovascular regions with surrounding hyperemia can be seen
with abscess formation. The spermatic cord may be enlarged.
The epididymal head can appear normal. Involvement of
the epididymal tail increases specificity for the diagnosis of
epididymitis.
If there is associated orchitis, spectral Doppler evaluation
of the intratesticular arteries will demonstrate increased peak
systolic velocity with a decreased resistive index of both the
testicular and epididymal arteries. These findings can occa-
sionally help to distinguish epididymitis from testicular tor-
sion, where there is generally high resistance flow.
Treatment is antibacterial therapy tailored to the expected
pathogen, which is age-dependent. Organ-sparing surgery
may be necessary if there are complications, including pyo-
cele, epididymal or testicular abscess, or if clinical improve-
ment does not occur within 48–72 hours following institution
of antibiotic therapy [62].
Isolated Orchitis
Isolated orchitis is rare [61], particularly since the introduc-
tion of the mumps vaccine in 1968 (along with measles-
mumps-rubella or MMR vaccine) [63]. However, there may
be an increased incidence in unvaccinated patients, particu-
larly pubertal and postpubertal males. A history of anteced-
ent parotitis is helpful in diagnosis as is a history of recent
mumps exposure, along with a normal urinalysis. Additional
rarer causes include syphilis, Coxsackie B virus, Brucella,
tuberculosis, and sarcoidosis [27, 64].
15 Male Genital Tract
a b
c d
Fig. 15.26 Epididymo-orchitis in a 13-year-old male. (a) Transverse
grayscale ultrasound image of both testes demonstrates a swollen right
testis (R), thickening of the right scrotal wall and reactive hydrocele
(asterisk). (b) Transverse color Doppler ultrasound image of both testes
reveals asymmetric hypervascularity of the right testis. (c) Sagittal
At ultrasound, testicular echogenicity will be heteroge-
neously decreased, with hyperemia observed on color Doppler,
with increased venous flow in particular [3]. In the majority of
patients with mumps orchitis, testicular involvement is unilat-
eral. A reactive hydrocele may be present. It should be noted
that when mumps is the etiology, associated epididymitis is
present in up to 85% of patients.
Shear wave elastography may be helpful to exclude
malignancy when the clinical and imaging findings are
unclear, with decreased shear wave velocity seen in the set-
ting of orchitis whereas increased shear wave velocity occurs
in the setting of neoplasm. Mumps orchitis usually resolves
spontaneously in a few days to weeks. Potential complica-
tions include testicular abscess or infarct, and infertility.
Treatment is supportive since the etiology is viral.
Testicular Abscess
Any form of testicular infection can be complicated by tes-
ticular abscess formation, although it is unusual (about 3–5%
of cases of epididymo-orchitis) and usually develops only
if the initial treatment is inadequate [57, 61, 65]. At ultra-
645
grayscale ultrasound image of the epididymis (arrowheads) shows an
enlarged epididymal tail which is similar in size to the epididymal head
(HD). (d) Sagittal color Doppler ultrasound image shows hyperemia of
the entire epididymis and adjacent scrotal wall
sound, a testicular abscess will appear as a fluid collection of
variable echogenicity, without internal vascularity and with
rim hyperemia [61]. CEUS may be beneficial in the diagno-
sis of testicular abscess, where absence of central enhance-
ment and peripheral hyperenhancement correspond to zones
of abscess [61, 66]. Treatment requires antibiotic therapy,
which may be prolonged. Surgical debridement is sometimes
necessary.
Epididymal Abscess
Epididymal abscess is an uncommon complication of epi-
didymitis, resulting from uncontrolled infection or non-
treatment [65, 67]. At ultrasound, an epididymal abscess will
appear as a heterogeneous fluid collection with absence of
internal vascularity and peripheral hyperemia. Aspiration
of abscess fluid has been reported as a successful treatment
since antibiotic therapy may not be effective [67].
Scrotal Abscess
Scrotal abscess, or pyocele, is an infected extra-testicular
scrotal fluid collection, most commonly developing from
646
Fig. 15.27 Scrotal abscess in a 7-year-old male. (a) Sagittal grayscale
ultrasound image of the right hemiscrotal soft tissues demonstrates a
fluid collection (arrowheads) with irregular margins. (b) Sagittal color
Doppler ultrasound image reveals avascularity of the collection with
surrounding hyperemia
secondary spread of peritonitis via a patent processus vagi-
nalis or as a complication of trauma, surgery, or epididymo-
orchitis. It rarely occurs in children [68, 69].
Hematogenous spread of infection to a pre-existing hydro-
cele can also occur, with reports of Haemophilus influenza,
Citrobacter freundii, Staphylococcus aureus, S. epidermidis,
enterococci, and Escherichia coli as pathogens. Clinically,
patients present with scrotal edema and erythema. Treatment
involves appropriate antimicrobial therapy and fluid removal
either via percutaneous aspiration or surgical exploration.
Ultrasound will demonstrate a fluid collection of varying
echogenicity within the scrotum. As with abscesses in other
parts of the body, mobile fluid can be detected with compres-
sion imaging. A fluid-fluid level may be seen, with depen-
dent echogenic debris. When gas is present within the fluid,
it appears as echogenic foci with dirty shadowing. There is
thickening and hyperemia of the scrotal wall and surround-
ing soft tissues (Fig. 15.27). Antibiotic therapy is usually
adequate, although surgical debridement may be necessary.
Henoch-Schönlein Purpura
Henoch-Schönlein purpura (HSP) is a systemic, necrotizing,
non-thrombocytopenic immunoglobulin (Ig) IgA-mediated
vasculitis, and is the most common vasculitis in children.
Patients are usually 2–7 years of age, and it is more com-
mon in boys [30, 70]. HSP affects small vessels of the mus-
J. H. Squires and H. J. Paltiel
culoskeletal system (presenting as a characteristic palpable
purpuric rash with arthralgias), gastrointestinal tract, and
genitourinary tract [70, 71]. Genitourinary involvement can
include HSP nephritis, ureteritis, and/or urethritis, which
may cause urinary tract obstruction when severe, as well
as hemorrhagic cystitis, epididymo-orchitis, spermatic cord
inflammation, and scrotal involvement.
HSP accounts for up to 3% of cases of acute scrotal
pain and occurs in almost 40% of cases of HSP [70, 71].
The most frequently involved scrotal structure is the epi-
didymis, with evidence of necrotic vasculitis and hemor-
rhage in the absence of infection. Clinical presentation
can include a scrotal rash, erythema, swelling, and pain,
which may be unilateral or bilateral. Scrotal involvement
can present later than the purpuric rash and arthralgias in
affected individuals.
Ultrasound typically shows scrotal wall thickening and
hyperemia. Epididymal enlargement may be seen as well as
a hydrocele. Importantly, the testes will appear normal with
normal blood flow (Fig. 15.28) [1, 70]. The normal appear-
ance of the testes is useful in distinguishing HSP from tes-
ticular torsion.
Treatment is conservative and supportive, with nonsteroi-
dal anti-inflammatory medication given for pain relief and
steroids for more severe cases.
I diopathic Scrotal Edema
Idiopathic scrotal edema is uncommon, but should be con-
sidered in boys less than 10 years of age who present with
scrotal swelling and erythema, which is often striking in
degree [30, 53]. Pain is typically relatively mild or absent
compared to the extent of swelling that may involve the
entire scrotum with extension into the inguinal regions,
perineum, and anterior lower abdomen. There may be asso-
ciated fever.
Other etiologies of scrotal swelling should be excluded,
including swelling related to third space fluid accumulation,
liver or heart failure, and nephrotic syndrome [30]. Serum
laboratory values will demonstrate eosinophilia without
leukocytosis. The presence of peripheral eosinophilia in
the majority of cases has led to the hypothesis that a hyper-
sensitivity/allergic reaction is a possible etiology, similar to
angioneurotic edema [72].
At ultrasound, acute idiopathic scrotal edema, like HSP,
will have scrotal wall thickening, with a heterogeneously
striated appearance related to fluid tracking between the
thickened skin and dartos fascia [1, 53, 72]. Color Doppler
imaging demonstrates scrotal wall hyperemia, which may be
marked. When edema is bilateral, some use the term “foun-
tain” sign to describe the marked hyperemia, given the radiat-
ing appearance of the scrotal wall hypervascularity between
the normal testes (Fig. 15.29). Testicular blood flow is pre-
served. Reactive inguinal lymph nodes may be present.
15 Male Genital Tract 647

L L

R R

Fig. 15.28 Henoch-Schönlein purpura (HSP) in a 9-year-old male. (a) with sagittal views on the left and transverse views on the right show a
Transverse color Doppler ultrasound image of both testes demonstrates normal homogeneous appearance of the left (L) and right (R) testes with
enlargement and hyperemia of the epididymides (arrows) adjacent to the marked enlargement and heterogeneous echogenicity of the epididymi-
right (R) and left (L) testes. (b, c) Split-screen grayscale ultrasound images des (arrows); (Fig. 15.28 continues)
648 J. H. Squires and H. J. Paltiel

Fig. 15.28 (continued) (d) Split-screen grayscale ultrasound images tomography (CT) image of the abdomen and pelvis shows marked small
of the body of the left epididymis with sagittal view on the left and bowel wall thickening (white arrow) and prominent reactive mesenteric
transverse view on the right show mass-like enlargement and heteroge- lymph nodes (black arrow) compatible with gastrointestinal HSP
neous echogenicity (arrows). (e) Coronal contrast-enhanced computed
15 Male Genital Tract
Idiopathic scrotal edema is usually self-limited, with
symptoms lasting 2–5 days followed by spontaneous regres-
sion, although recurrence can be seen in about 20% of cases.
Fournier Gangrene
Fournier gangrene is a rapidly progressive superficial infec-
tion of the penis or scrotum, usually occurring after minor
trauma in a patient with immunodeficiency, and is usu-
ally caused by E. coli or polymicrobial infection [61, 65].
Infection spreads between the fascial planes of the scrotal
tissues, causing necrosis and gas formation, and there may
be rapid progression of infection. Clinically, soft tissue ery-
thema and induration are present, with gangrene and subcu-
taneous emphysema in severe cases or in those with a late
presentation.
Ultrasound can be invaluable in the diagnosis of Fournier
gangrene, since patients may be too ill to undergo cross-sec-
tional imaging, although the deep extent of infection may
be difficult to visualize. Ultrasound recognition of echogenic
a At ultrasound, findings include hydrocele, calcification,
T
T
b
Fig. 15.29 Acute idiopathic scrotal edema in a 5-year-old male. (a)
Transverse grayscale ultrasound image of the scrotum through both tes-
tes (T) demonstrates marked thickening of the scrotal wall soft tissues
(asterisk) with intervening hypoechoic fluid. (b) Transverse color
Doppler ultrasound image of the scrotum through both testes demon-
strates striking hypervascularity of the markedly thickened scrotal wall
soft tissues with a so-called “fountain” sign (arrow)
649
soft tissue foci as gas is important for timely diagnosis. Gas
within the scrotal wall is diagnostic of Fournier gangrene
(Fig.15.30). Typically, there is no associated orchitis due to
the separate blood supply to the testis. Mortality has been
reported in up to 50% of cases. Treatment is rapid surgical
debridement, ideally within 24 hours of presentation.
Chronic Epididymitis
Chronic epididymitis is less common than acute epididy-
mitis and is best defined as persistent swelling and/or pain
localized to the epididymis for more than 3 months [73].
Genitourinary tract anomalies may predispose to develop-
ment of chronic epididymitis in boys [59] and it is more
frequently associated with chronic granulomatous diseases,
such as tuberculosis and brucellosis; or syphilis, parasitic and
fungal infections in older males [38]. Chronic inflammation
of the epididymis leads to fibrosis which causes firmness to
palpation, and patients with chronic epididymitis may pres-
ent with concern for an extratesticular mass.
and scrotal wall thickening [38]. Ultrasound diagnosis is
challenging and epididymectomy may be necessary for
definitive diagnosis.
Dancing Megasperm
Epididymal obstruction in a postpubertal male can lead to
ultrasound visualization of echogenic particles with a random
to-and-fro appearance corresponding to clumps of aggluti-
nated sperm [74, 75]. This may be seen following vasectomy
in up to 12% of older men, although rarely it is identified in
adolescents with scarring of the vas deferens, such as follow-
ing epididymitis [74, 76] or surgery for inguinal hernia [77].
A similar ultrasound appearance may be seen with lymphatic
obstruction caused by filarial disease in endemic tropical and
subtropical areas [74].
When megasperm are encountered in adults there is usu-
ally no clinical concern. When present in a young patient, epi-
didymal obstruction should be a consideration with potential
implications for future fertility.
Scrotal and Spermatic Cord Fluid Collections
Scrotal Hydrocele
The most common cause of scrotal swelling in boys is a scrotal
hydrocele, an accumulation of fluid between the parietal and
visceral layers of the tunica vaginalis [10, 30]. It should be
noted that 1–2 mL of fluid is considered normal and more than
this amount should be present for hydrocele diagnosis [69].
650
a c
Fig. 15.30 Fournier gangrene in a 15-year-old male with leukemia. (a)
Transverse grayscale ultrasound image of the left hemiscrotum reveals
edema of the scrotal wall as well as ill-defined dependent echogenic
foci (asterisks) medial and lateral to the left testis (T). (b) Transverse
grayscale ultrasound image of the left hemiscrotum highlights the
A congenital hydrocele may be entrapped peritoneal fluid
(non-communicating) [2] or communicating fluid with a pat-
ent processus vaginalis (Fig. 15.31). An acquired hydrocele
can occur in older males secondary to other scrotal abnor-
malities, including infection, torsion, trauma, and/or tumor,
excessive fluid production, or reduced absorption by the
mesothelial lining [30, 69].
Simple serous fluid is usually present and is anechoic at
ultrasound, although there may be septations if it is infected.
It may contain mobile echogenic foci related to protein aggre-
gation or cholesterol crystals (Fig. 15.32). When a hydrocele
is large, pressure exerted on the testis may lead to diminished
vascularity, with decreased diastolic flow detected with spec-
tral Doppler evaluation. Color Doppler may show increased
visibility of the capsular arteries and there can be enlarge-
ment of the ipsilateral testis. Removal of fluid improves
testicular perfusion. Communicating hydroceles resolve by
2 years of age in up to 80% of cases [2, 69].
J. H. Squires and H. J. Paltiel
marked soft tissue swelling and prominent dependent echogenic foci
due to air in the scrotal wall (arrows). (c) Radiograph of the pelvis
shows gas (arrowheads) in the left scrotal wall extending into the left
extraperitoneal space
Spermatic Cord Hydrocele
Hydrocele of the spermatic cord appears as a loculated fluid
collection along the spermatic cord, superior to the testis
and epididymis, and is a result of abnormal closure of the
processus vaginalis. Clinically, it presents with swelling in
the groin or upper scrotum [11]. There are three types of
spermatic cord hydrocele: communicating, funicular, and
encysted [69]. With communicating spermatic cord hydro-
cele, there is complete patency of the processus vaginalis.
Fluid may be seen extending from the peritoneum through
the deep inguinal ring down to the scrotum.
A funicular spermatic cord hydrocele develops when the
processus vaginalis closes just above the testis at the deep
inguinal ring, with a focal fluid collection that communicates
with the peritoneum but not with the scrotum (Fig. 15.33).
Because there is communication with the peritoneum, fluid
may increase with crying and/or the Valsalva maneuver. It
appears as a fluid-containing peritoneal diverticulum, and
15 Male Genital Tract 651

Abdominal
cavity

Testicle

Scrotum

Normal Non-communicating Communicating Hydrocele

hydrocele hydrocele of the cord

Fig. 15.31 Diagram of the different types of hydrocele

may contain septations as a result of partial closure and

a inflammation. It may occasionally have a beaded appearance
termed “pachyvaginalitis”.
T An encysted spermatic cord hydrocele consists of a focus
of fluid located along the spermatic cord with closure of the
T
processus vaginalis superiorly and inferiorly, without com-
munication to either the peritoneum or scrotum.
Because communicating and funicular spermatic cord
hydroceles may be considered forms of indirect inguinal her-
nia, herniotomy is usually performed. An encysted spermatic
cord hydrocele is non-communicating and typically resolves
spontaneously. Surgical intervention is reserved for those
b that persist into childhood.

Abdominoscrotal Hydrocele
An abdominoscrotal hydrocele is a very rare hydrocele vari-
ant, where a large inguinoscrotal hydrocele protrudes through
the inguinal ring into the abdominal cavity, with focal fluid
seen within both the scrotum and the lower abdomen [78].
There are fewer than 100 case reports of this entity [69], and
it may be mistaken clinically for an inguinal hernia.

Fig. 15.32 Bilateral hydroceles in a 2-month-old male. (a) Transverse
grayscale ultrasound image of the scrotum demonstrates anechoic fluid
(asterisks) within the tunica vaginalis of both testes (T). (b) Transverse
color Doppler ultrasound image of the scrotum demonstrates normal
flow to both testes. Asterisks, Hydrocele
Scrotal Hematocele
A scrotal hematocele is defined as hemorrhage within the
layers of the tunica vaginalis and may be seen following tes-
ticular trauma, more frequently developing on the right side
[69]. Hematocele can also occur in the setting of hemato-
logical disorders and vasculitis. Intraperitoneal hemorrhage
652 J. H. Squires and H. J. Paltiel

Fig. 15.33 Funicular spermatic cord hydrocele in a 2-year-old male.

Sagittal grayscale ultrasound image of the right inguinal canal demon-
strates anechoic fluid (asterisk) superior to the right testis, but without
extension into the scrotum

can lead to a hematocele if the processus vaginalis is patent

(Fig. 15.34).
At ultrasound, a hematocele in the hyperacute phase
will appear anechoic. In the acute phase after initial blood
clotting, a hematocele will appear echogenic. After hemo-
lysis, it will decrease in size and appear hypoechoic to
anechoic, usually with associated septations. Calcification
can occur over time, giving the appearance of an encap-
sulated lesion. There will be no internal flow on color
Doppler evaluation.
Treatment may be needed to address the underlying cause
of hemorrhage. When a hematocele is large or complex, there
can be compression of the testicular parenchyma leading to
atrophy, so surgical intervention is sometimes necessary.

Scrotal Lymphocele
A scrotal lymphocele is uncommon and is a result of lym-
phatic fluid accumulation [69]. It may occur after renal
transplantation and is thought to be caused either by
scrotal lymphatic obstruction or peritransplant lymphatic
dissection. Lymphatic filariasis, commonly known as
elephantiasis, is an underlying etiology in endemic areas.
The appearance of lymphocele at ultrasound is similar to
that of a septated hydrocele. It may become more echo-
genic if there is superimposed hemorrhage or infection.
When symptomatic, treatment typically consists of drain-
age, either surgically with wall fenestration or percutane-
ously, with sclerotherapy [79].

Scrotal Calcification
Testicular Microlithiasis
Testicular microlithiasis is defined as the presence of five or
more non-shadowing echogenic foci within the seminiferous
tubules of a testis on one ultrasound image, and is present in
up to almost 4% of boys [1, 80]. There may be an associa­
tion with Klinefelter syndrome and trisomy 21 [81]. At ultra-
Fig. 15.34 Hematocele in a 2-year-old male with bleeding Meckel
diverticulum. (a) Transverse grayscale ultrasound image demonstrates
echogenic fluid (asterisk) surrounding the right testis (T). (b) Sagittal
color Doppler ultrasound image reveals echogenic fluid (asterisks) sur-
rounding the right testis (T) and epididymis (arrowhead), both of which
have normal parenchymal blood flow. (c) Coronal contrast-enhanced
CT image of the abdomen and pelvis shows a Meckel diverticulum
(arrowhead) with a small amount of surrounding hemorrhagic fluid that
extends into the right inguinal canal (arrow)
15 Male Genital Tract
sound, microlithiasis can be diffuse or segmental [29] and may
be unilateral or bilateral (Fig. 15.35). The microcalcifications
are thought to represent calcified degenerated intratubular
cells and debris, although this is speculative.
Recent recommendations from the European Society
of Urogenital Radiology (ESUR) for adults do not suggest
monitoring for possible tumor development in the setting of
testicular microlithiasis [82]. However, these recommenda-
tions may not apply to the pediatric population. In children,
there appears to be a strong association between testicular
microlithiasis and testicular tumors, as recently described in
a large retrospective review, with tumors identified in nearly
5% of patients with testicular microlithiasis [80].
Fig. 15.35 Testicular microlithiasis in a 10-year-old male. Transverse
grayscale ultrasound image of the right testis demonstrates multiple
echogenic foci scattered diffusely throughout the parenchyma
Fig. 15.36 Scrotal pearl in a 7-year-old male. Sagittal grayscale ultra-
sound image shows a normal testis with an adjacent echogenic shadow-
ing scrotal calculus (arrowhead)
653
No specific treatment is required for isolated testicular
microlithiasis. Pediatric patients are generally encouraged
to perform self-examination and are provided reassurance.
Imaging and urological referral are usually reserved for chil-
dren with associated risk factors such as cryptorchidism, and
a, family or personal history of testicular malignancy. For
children presenting with testicular microlithiasis and a tes-
ticular mass, immediate referral to a pediatric urologist or
oncologist is mandatory.
Loose Bodies
Scrotal calculi, or scrotal pearls, are mobile calcified lesions
located between the parietal and visceral layers of the
tunica vaginalis and may be due to a variety of causes,
including prior hematoma or infection, or prior torsion of
an epididymal or testicular appendage [58]. An additional
proposed source of scrotal calculi is chronic inflammation
and/or trauma to the tunica vaginalis with decreased reab-
sorption of cholesterol, calcium, and fibrin, resulting in cal-
culus formation.
At ultrasound, scrotal calculi appear as echogenic foci
with posterior acoustic shadowing or comet-tail artifact
(Fig. 15.36). No specific treatment is required.
Meconium Peritonitis
Meconium peritonitis is caused by in utero bowel perfora-
tion (usually small bowel), and occurs with congenital bowel
obstruction related to intestinal atresia or meconium ileus
in the setting of cystic fibrosis [1, 83]. After perforation,
extruded meconium and digestive enzymes cause a chemi-
cal peritonitis and inflammation that leads to foreign-body
granulomas and calcification. In addition to intra-abdominal
meconium and calcification, meconium peritonitis can extend
from the peritoneal cavity into the scrotum through a patent
processus vaginalis. Meconium-containing fluid and calcifi-
cation are seen in the scrotum between the layers of the tunica
vaginalis (meconium periorchitis) (Fig. 15.37) [1, 84].
At ultrasound, calcifications within the tunica vaginalis
in a newborn male should raise the possibility of meconium
peritonitis, and careful evaluation of the abdomen for evi-
dence of bowel obstruction, pneumoperitoneum, ascites, and
volvulus should be undertaken. Treatment involves manage-
ment of the primary bowel pathology, often requiring surgi-
cal management.
Trauma
Blunt Scrotal Trauma
Blunt trauma is the most frequently occurring mechanism of
scrotal injury (80–85% of cases) [29]. Peak age of incidence
is from 10 to 30 years and is usually sports-related. Other eti-
654
ologies include straddle injuries or motor vehicle collisions,
and more often occur on the right side than the left. This is
due to the typically higher position of the right testis, permit-
ting compression against the pubic bone or inner thigh [65,
85]. Non-accidental trauma should be considered depending
on the clinical scenario.
a can occur in association with other scrotal injuries as detailed
T T
b
Fig. 15.37 Meconium peritonitis in a 3-week-old male. (a) Transverse
grayscale ultrasound image of the scrotum reveals multiple punctate
echogenic foci with distal shadowing in both hemiscrota lateral to the
testes (T). (b) Sagittal grayscale ultrasound image of the left hemiscro-
tum shows extensive deposits of calcified material
a b
Fig. 15.38 10-year-old male with testicular and epididymal hemato-
mas and hematocele after blunt scrotal trauma. (a) Sagittal grayscale
ultrasound image shows swelling of the scrotal skin. The testicular
parenchyma is heterogeneous with a focus of increased echogenicity
(white arrow) in keeping with hematoma. The epididymis is enlarged
and heterogeneous (arrowheads), also likely reflecting internal hemor-
J. H. Squires and H. J. Paltiel
Injury to the epididymis typically occurs in the setting of
testicular injury, with imaging findings similar to those seen
with infectious epididymitis [29, 86]. Treatment is typically
conservative. Table 15.3 reviews the more common ultra-
sound findings associated with traumatic scrotal injuries.
Testicular Hematoma
In the setting of scrotal trauma, intratesticular hematoma is
common [49]. Hematoma is usually an isolated injury but
below [85]. At ultrasound, focal areas of heterogeneous tes-
ticular parenchymal echogenicity are identified corresponding
to contusion, hematoma, and/or infarction (Fig. 15.38) [49,
65]. The appearance of an intratesticular hematoma will vary
depending on its age, with an acute hematoma predominantly
hyperechoic to the testicular parenchyma while a chronic
hematoma may be hypoechoic or anechoic. Color Doppler
is helpful in demonstrating absence of flow within the hema-
Table 15.3 Scrotal abnormalities associated with blunt trauma
Abnormality Ultrasound appearance
Testicular Isoechoic or heterogeneous focus within the
hematoma testicular parenchyma with no internal vascular
flow
Testicular Linear hypoechoic focus extending through
fracture testicular parenchyma, intact tunica albuginea
(normal testicular shape)
Testicular Disrupted tunica albuginea causing loss of normal
rupture testicular shape, scrotal parenchymal protrusion
into the tunica vaginalis
Hematocele Hemorrhagic echogenic fluid between tunica
vaginalis layers
rhage. The superior aspect of the testis is indented (black arrow)
although the tunica albuginea appears intact. Echogenic material within
the scrotal sac (asterisk) represents a hematocele. (b) Transverse gray-
scale ultrasound image shows the enlarged, heterogeneous epididymis
(arrowheads), fluid level (black arrow), and septations (white arrow)
within the hematocele
15 Male Genital Tract 655

toma. CEUS may also be helpful in documenting the absence
of enhancement at sites of hematoma [53, 57].
Treatment is typically conservative. Follow-up ultrasound
is recommended to ensure that infection and necrosis do
not develop, which require surgical intervention. Surgery
involves removal of the avascular portions of the testis, with
evacuation of any residual intratesticular hematoma.
At ultrasound, visualization of frank discontinuity of the
tunica albuginea is an important sign in diagnosing testicular
rupture. Irregular testicular contour is an additional helpful
feature, which occurs due to testicular parenchymal protru-
sion through the disrupted tunica albuginea (Fig. 15.40 and
Video 15.3). Large associated hematoceles may make visu-

Testicular Fracture
Testicular fracture is defined as a linear disruption of the nor-
mal testicular parenchyma [85]. At ultrasound, visualization
of a discrete fracture line is rare, only seen in up to 17% of
cases [49]. When identified, it appears as a discrete linear
hypoechoic focus extending across the testis [49, 65]. There
is often an associated testicular hematoma. Testicular frac-
ture can be associated with disruption of the tunica albuginea
(Fig. 15.39).
Color Doppler may be helpful to demonstrate absent
vascularity corresponding to the parenchymal fracture line
[49]. Vascular flow to the testicular parenchyma surrounding
the track is important to exclude adjacent ischemia, which
requires emergent surgery [86]. Otherwise, treatment is
conservative.

Testicular Rupture
Testicular rupture is defined as disruption of the tunica
albuginea with protrusion of seminiferous tubules. Rupture
is almost always accompanied by testicular parenchymal
injury and disruption of the tunica vasculosa, the inner vas-
cular layer of the tunica albuginea that may result in segmen-
tal ischemia [49, 65].
Fig. 15.39 13-year-old male with testicular fracture. Transverse gray-
scale ultrasound image of the right hemiscrotum reveals a hypoechoic
linear fracture (arrow) extending through the mid-right testis. The frac-
ture is associated with a focal tear of the tunica albuginea with an ele-
vated flap of tissue (arrowhead). There is a large, mildly echogenic
hematocele (asterisk)

Fig. 15.40 Testicular rupture in a 16-year-old male who sustained
direct trauma to the left hemiscrotum. (a) Sagittal grayscale ultrasound
image of the left testis (T) demonstrates preserved architecture of its
superior portion with abnormal echogenicity and contour of its mid and
lower portions (arrows) compatible with rupture of the tunica albuginea,
parenchymal protrusion, and hematoma formation. H, Hematoma. (b)
Sagittal color Doppler ultrasound image of the left testis demonstrates
preserved vascularity (arrow) of the superior portion of the testis. Flow
is absent (arrowheads) to the mid and lower testis. H, Hematoma
656
alization of the tunica albuginea challenging. A diagnosis
of testicular rupture is important to make rapidly, because
a greater than 80% testicular salvage rate can be achieved if
surgical repair is performed within 72 hours of injury.
Scrotal Hematocele
A scrotal hematocele is the most common finding after blunt
scrotal trauma. It is discussed earlier in this chapter in the
section on scrotal fluid collections.
Penetrating Scrotal Trauma
Compared to blunt scrotal trauma, penetrating scrotal trauma
is more likely to require surgical intervention, but is encoun-
tered less commonly (15–20% of cases) [29]. Potential etiolo-
gies include gunshot, stab, or bite wounds [49]. Injury extent
is variable depending on the type and location of the trauma.
Minor injuries can result in a hematocele, while major injuries
may lead to testicular rupture and associated damage to the
adjacent soft tissues of the thigh, perineum, and penis.
a c
Fig. 15.41 17-year-old male with a scrotal bullet. (a) Transverse gray-
scale ultrasound image of the right hemiscrotum above the right testis
shows a rounded hypoechoic structure with echogenic walls (arrow-
heads) associated with marked posterior shadowing (asterisk). (b)
J. H. Squires and H. J. Paltiel
Ultrasound can demonstrate the presence of soft tissue
gas extending along a hypoechoic linear track, and lack of
vascularity. Treatment will vary based on extent of injury,
with surgical debridement of necrotic areas. In cases of ani-
mal bites, the high risk of infection necessitates antimicro-
bial therapy.
Foreign Body
When penetrating injury is caused by a pellet or gunshot
wound, a foreign body may be retained within the scrotum
(Fig. 15.41) [49]. Other foreign bodies encountered after
penetrating injury include wood, glass, metal, plastic, or
gravel [87]. Erosion of magnets into the scrotum has also
been reported [88]. Clinically, a history of penetrating injury
is typically present. The foreign body insertion site is usually
visually apparent, although it can be imperceptible if there
is secondary infection or inflammation. At ultrasound, most
foreign bodies are echogenic and typically can be readily
seen. Treatment involves surgical removal of the object.
Sagittal grayscale ultrasound image of the right hemiscrotum shows the
relationship of the bullet (arrow) to the normal right testis (T). (c) A
radiograph of the pelvis shows the radiopaque bullet fragment in the
right hemiscrotum
15 Male Genital Tract
Scrotal Urinoma
Extravasation of urine from the bladder can extend into the
scrotum through the inguinal canal via a patent processus
vaginalis. This has been reported following penetrating scro-
tal injury, renal transplantation complicated by urine leak
[89] and urinary bladder leak related to urolithiasis [90]. At
ultrasound, a urinoma appears similar to a hydrocele, with
fluid between the parietal and visceral layers of tunica vagi-
nalis. The peritoneal origin of the urine becomes apparent
when imaging the inguinal canal and pelvis [91]. History is
helpful in determining the source of the leak and treatment
requires repair of the underlying cause.
Repetitive Scrotal Microtrauma
Mountain Biking
Mountain bicycles, also called off-road bicycles, may cause
repetitive microtrauma to the scrotum due to vibrations and
impact from the bicycle seat. Almost half of all mountain
bikers experience scrotal tenderness on physical examina-
tion, despite lacking a history of a well-documented incident
of scrotal trauma. Scrotal abnormalities are seen by ultra-
sound in up to 94% of extreme mountain bikers, including
epididymal cysts, scrotal calculi, hydroceles, and varicoceles
[92]. Use of full-suspension bicycles and padded seats may
help to minimize repetitive microtrauma.
Equestrian-Related Injuries
Acute straddle injuries can occur as a result of equestrian-
related events, accounting for up to 7% of cases of scrotal
trauma [93]. While acute severe scrotal trauma may occur,
Normal
Epigastric
vessels
Abdominal Inguinal canal
musculature
Fig. 15.42 Diagram of the normal inguinal canal
657
horseback riding has also been linked to repetitive scrotal
microtrauma due to contact of the perineum with the saddle.
The risk may be higher in jumping riders. As with mountain
bikers, half of all equestrians experience scrotal tenderness
on physical examination, and more than 75% of all equestri-
ans will have an abnormal scrotal ultrasound study.
Lesions include varicocele, hydrocele, epididymal or
testicular cyst, epididymal or testicular calcification, and
scrotal calcification, similar to the findings in mountain bik-
ers described above. The presence of a varicocele can have
implications for future fertility.
Inguinal Hernia
Indirect Inguinal Hernia
Inguinal hernias are common abnormalities, more fre-
quent in males than females, usually unilateral and right-
sided [10]. The inguinal canal extends superiorly from the
superficial (external) inguinal ring to the deep (internal)
inguinal ring, bordered anteriorly by the external oblique
muscle aponeurosis, laterally by the internal oblique mus-
cle, posteriorly by the fascia transversalis, and medially
by the lacunar ligament (Fig. 15.42). An inguinal hernia
occurs when intra-abdominal structures protrude into the
inguinal canal [94]. Clinically, patients will present with
an inguinal bulge that enlarges with crying or the Valsalva
maneuver.
Most inguinal hernias are indirect in children, where
abdominal contents extend through the deep inguinal ring.
They originate at the deep inguinal ring, lateral to the
Peritoneum
658
Indirect Inguinal Hernia
Lateral to
epigastric vessels
Fig. 15.43 Diagram of an indirect inguinal hernia that originates lateral to the inferior epigastric vessels and courses through the deep inguinal ring
inferior epigastric vessels (Fig. 15.43) [10]. Up to 90%
of cases of indirect inguinal hernia are associated with a
patent processus vaginalis and are considered congenital
abnormalities.
Direct Inguinal Hernia
In contrast to the lateral origin of an indirect inguinal her-
nia, a direct inguinal hernia arises medial to the inferior epi-
gastric vessels and protrudes directly into the inguinal canal
(Fig. 15.44), rather than extending through the deep inguinal
ring. A direct inguinal hernia is less common than an indirect
hernia [10]. It is not typically congenital in nature, result-
ing instead from age-related thinning of the abdominal wall
musculature and fascia. It usually occurs in older males, but
can be seen in younger patients with collagen vascular dis-
eases such as Ehlers-Danlos syndrome [94]. A true congeni-
tal direct inguinal hernia is rare.
At ultrasound, both direct and indirect inguinal hernias
may contain fluid, small bowel, appendix (an Amyand her-
nia), colon, omental fat, ureter, and/or urinary bladder. In
young children, waiting for the patient to cry may be neces-
sary to visualize herniation. Upright positioning can often
improve ultrasound visualization of a hernia and its con-
tents. Ultrasound can distinguish between indirect and direct
inguinal hernias by showing the location of the hernia neck
in relation to the inferior epigastric vessels. Bowel peristalsis
can be well demonstrated by acquiring cine clips with a sta-
tionary transducer (Fig. 15.45 and Video 15.4).
Inguinal hernias can be further classified as reducible, incar-
cerated, or strangulated. With a reducible hernia, the contents
of the hernia can be pushed back into the abdominal cavity.
With an incarcerated hernia, the contents of the hernia can-
not be returned to the abdominal cavity, either spontaneously
J. H. Squires and H. J. Paltiel
Projects through
inguinal ring
Direct Inguinal Hernia
Medial to Projects through
epigastric abdominal wall
vessels
Fig. 15.44 Diagram of a direct inguinal hernia that originates medial
to the inferior epigastric vessels and does not extend through the deep
inguinal ring
or by manual or transducer compression. Strangulated hernia
contents have compromised vascularity, a surgical emergency.
Absence of vascular flow at color Doppler imaging is
helpful in diagnosing strangulation, although with early
strangulation there may be hyperemia. Additional findings
associated with strangulation include free fluid in the her-
nia sac and absent bowel peristalsis. Gas in the hernia sac
or pneumatosis of the herniated bowel raises concern for
bowel perforation. When a bowel-containing inguinal hernia
is identified, consideration should be given to assessing the
abdomen for dilated bowel loops, which may indicate bowel
obstruction caused by the hernia.
Inguinal hernias are treated surgically with herniorrhaphy
along with high ligation of the patent processus vaginalis.
A mesh is often placed across the hernia opening in older
patients and in those with a direct hernia. The mesh may be
visible by ultrasound as an echogenic structure with poste-
rior acoustic shadowing.
15 Male Genital Tract
Fig. 15.45 Direct inguinal hernia in a 1-month-old male. (a) Sagittal
grayscale ultrasound image of the left hemiscrotum demonstrates a nor-
mal left testis (T) and epididymal head (E) surrounded by a hydrocele
(asterisk). The herniated bowel (arrow) contains gas that is depicted as
echogenic foci with posterior dirty shadowing. (b) Sagittal grayscale
ultrasound image of the left inguinal canal shows the deep inguinal ring
(arrowheads) and inferiorly herniated bowel loops (B)
A spermatic cord hematoma can develop postoperatively
due to the close approximation of the spermatic cord and
the inguinal ligament, and is usually the result of injury to
the spermatic cord vessels [49]. Postoperative fluid collec-
tions may occur. Recurrent hernia is seen in up to 15% of
patients following herniorrhaphy. Testicular infarction can
develop after surgical repair of inguinal hernia, with atrophy
and associated infertility.
Varicocele
A varicocele is an abnormal dilation of the veins in the pampi-
niform plexus and may present clinically as a palpable, soft
scrotal mass [95]. Most varicoceles are idiopathic in etiology.
They are usually detected in adolescence or young adulthood.
A varicocele is one of the most commonly encountered abnor-
malities when evaluating patients for infertility. The vessels
of the left spermatic cord are affected in up to 85% of cases.
A right-sided varicocele usually occurs in association
with a left-sided varicocele and is rarely encountered in iso-
lation [29]. This is because the right gonadal vein drains to
the inferior vena cava, a larger vessel with greater flow com-
pared to the left renal vein, a smaller vessel with less flow
[30]. When a new, isolated right-sided varicocele is encoun-
659
tered, consideration should be given to evaluating for a right
retroperitoneal mass causing obstruction.
Although the diagnostic criteria are somewhat contro-
versial, a varicocele is often said to be present when at
least two veins in the pampiniform plexus have a diameter
of 2 mm or more (Fig. 15.46). Venous diameter and color
Doppler flow will increase during the Valsalva maneuver
or upright patient positioning.
Dilated veins will appear as anechoic, tubular structures
extending along the spermatic cord [96]. When large, a vari-
cocele can be associated with testicular hypotrophy due to the
increased ambient temperature related to the dilated veins.
Therefore, evaluation of testicular volume is important when-
ever a varicocele is diagnosed. Shear wave elastography has
been used in patients with varicoceles, with increasing stiff-
ness values seen with increasing grades of the varicocele and
is inversely correlated with testicular volume [95].
Treatment of varicocele in pediatric patients is contro-
versial. Closure of the gonadal vein can be performed to
redirect blood flood flow, either surgically via emboliza-
tion, or with percutaneous sclerotherapy [97]. Treatment
is considered in adolescents with testicular hypotrophy,
a large varicocele with decreased sperm count at semen
analysis, or when the varicocele causes symptoms of pain,
swelling, and/or heaviness.
a
T
b
Fig. 15.46 Varicocele in a 13-year-old male. (a) Transverse grayscale
ultrasound image of the left scrotum shows a normal testis (T) with
dilated veins (arrowhead) along its lateral aspect. (b) Sagittal color
Doppler split-screen ultrasound image of the left inguinal canal with the
patient at rest (left panel) and during a Valsalva maneuver (right panel).
The dilated veins of the pampiniform plexus (arrowheads) increase in
size and flow velocity with the Valsalva maneuver
660
Intratesticular Varicocele
While extratesticular varicoceles are common, intratesticular
varicoceles are rare. They may or may not be associated with
extratesticular varicocele, are more frequent on the left side,
can be bilateral, and may cause pain [69, 98, 99]. Importantly,
an intratesticular varicocele is more common in patients who
have undergone prior orchiopexy for cryptorchidism and may
be seen in the setting of epididymo-orchitis. The pathogenesis
of intratesticular varicocele is thought to be similar to that of an
extratesticular varicocele.
At ultrasound, an intratesticular varicocele will be seen
as serpiginous vessels measuring 2 mm or more in diameter
within the mediastinum testis or subcapsular region. These
vessels contain venous flow that increases with crying or
the Valsalva maneuver (Fig. 15.47). Sensitive Doppler tech-
niques may be necessary to demonstrate slow flow in these
vessels to help distinguish them from cystic dysplasia of the
rete testis.
Percutaneous sclerotherapy for intratesticular varico-
celes has been reported, as has surgical varicocelectomy.
a b
Fig. 15.47 Intratesticular varicocele in a 16-year-old male. (a) Sagittal
grayscale ultrasound image of the left testis shows multiple peripheral,
hypoechoic, tubular and ovoid vessels. More than five punctate echo-
genic parenchymal foci are identified in keeping with testicular micro-
J. H. Squires and H. J. Paltiel
Spontaneous resolution has also been noted when the intra-
testicular varicocele is associated with epididymo-orchitis.
Testicular Masses
Non-Neoplastic Lesions
Adrenal Rests
Testicular adrenal rests are relatively common in boys with
congenital adrenal hyperplasia, which is usually caused by a
deficiency of 21-α- hydroxylase, and rarely may be seen in
males with Cushing syndrome [81, 100]. During fetal devel-
opment, adrenal rests may become entrapped within the
developing gonad and can form large masses when exposed
to elevated adrenocorticotropic hormone.
At ultrasound, adrenal rests are located near the medias-
tinum testis. They are usually multiple, bilateral, and well-
circumscribed with round or lobulated margins, generally
hypoechoic and may demonstrate posterior acoustic shadow-
lithiasis. Sagittal color Doppler ultrasound images of the left testis
obtained (b) at rest showing a small amount of flow within the periph-
eral parenchymal vessels that increases (c) during a Valsalva maneuver
15 Male Genital Tract 661

content. They range from 1 to 6 mm in size [106, 108]. There

is typically no hypervacularity with color Doppler evaluation
and no posterior acoustic shadowing.
Testicular hamartomas have no effect on spermatogenesis
or testicular function and there is no specific therapy.

Fig. 15.48 Adrenal rests in a 14-year-old male with congenital adrenal
hyperplasia. Sagittal grayscale ultrasound image of the right testis
shows three well-circumscribed hypoechoic foci (arrowheads) near the
mediastinum testis
ing (Fig. 15.48) [81, 101]. They are hypervascular by color
Doppler imaging. Treatment of the underlying endocrinopa-
thy typically causes the masses to stabilize in size or involute.
Simple Cyst
A simple testicular cyst is usually discovered incidentally and
is not generally palpable. Cysts range in size from 2 mm to
2 cm and are more common in older men [109]. They are usu-
ally located near the mediastinum testis and are associated
with extratesticular spermatoceles [69] or a dilated rete testis.
Cysts can also occur within the tunica albuginea at the periph-
ery of the testis [81]. Some cysts are associated with cystic
dysplasia of the rete testis or von Hippel-Lindau disease [109].
At ultrasound, a simple testicular cyst is anechoic with
increased posterior through-transmission and no perceptible
wall [69]. It may be unilocular or multi-loculated (Fig. 15.49).
CEUS may be helpful to confirm the diagnosis of a cyst by
revealing no enhancement when there is confusion caused

Leydig Cell Hyperplasia

Leydig cells are the interstitial cells of the testicular stroma a
that secrete testosterone and estrogen [81]. Boys with pri-
mary Leydig cell hyperplasia typically present with preco-
cious puberty [102]. Secondary Leydig cell hyperplasia can L
result from stimulation of the Leydig cells of the testis with
exogenous hormone administration. In older males with sec-
ondary Leydig cell hyperplasia, it is often discovered inciden-
tally during evaluation for infertility, gynecomastia, or scrotal
pain and swelling [102, 103]. An association with Klinefelter
syndrome and congenital adrenal hyperplasia has also been
reported, although Leydig cell hyperplasia is rare [102, 104,
105]. Clinically, Leydig cell hyperplasia is not palpable.
At ultrasound, it can appear similar to congenital testicu-
lar adrenal rests, with multiple small hypoechoic lesions that
are hypervascular on color Doppler evaluation. Hyperechoic
foci with intervening hypoechoic fibrotic testicular paren-
chyma have also been reported.
Treatment of Leydig cell hyperplasia first entails confir- b
mation of the diagnosis by history and laboratory evaluation,
with possible open-wedge biopsy to exclude Leydig cell
tumor. There is currently no consensus regarding manage- E
ment, but partial orchidectomy, enucleation, and medical
treatment have been suggested.

Hamartoma
Similar to hamartomas elsewhere in the body, testicular ham-
artomas arise from germ cell layers and may be associated
with hamartomatous syndromes such as Cowden disease
Fig. 15.49 Testicular cyst in a 3-month-old male. (a) Transverse grayscale
(multiple hamartoma syndrome) [106, 107]. ultrasound image of the scrotum shows a large cyst replacing the right tes-
At ultrasound, testicular hamartomas manifest as multiple tis. L, Left testis. (b) Sagittal grayscale ultrasounds image of the right
bilateral, well-defined hyperechoic foci that reflects their fat hemiscrotum shows a septation (arrow) within the cyst. E, Epididymal head
662
by the presence of internal echogenic content related to pro-
teinaceous material or hemorrhage [57]. If there is any solid
component or nodularity of the cyst, a cystic teratoma should
be considered in the differential diagnosis. A simple testicu-
lar cyst does not require treatment.
Sinus Histiocytosis (Rosai-Dorfman-Destombes
Disease)
Sinus histiocytosis is a rare, non-Langerhans cell histiocyto-
sis where affected structures have abnormal accumulations of
activated histiocytes. This disorder usually occurs in males and
those of African descent [110]. The disease can occur in asso-
ciation with autoimmune, hereditary, and malignant diseases, or
in isolation. While classically presenting with bilateral, massive,
painless cervical lymphadenopathy, extranodal involvement
can also occur. Scrotal involvement is rare and may present as
a painful testicular or epididymal mass that can mimic tumor or
epididymitis, or as a diffusely large and stiff testis.
At ultrasound, hypervascular masses may be noted in the
testes and epididymides [111]. A hypervascular scrotal mass
accompanying painless cervical lymphadenopathy may be
helpful in suspecting the diagnosis. Sinus histiocytosis is
usually self-limited, and patients do not require treatment
unless they are symptomatic. Steroids are generally used as
a first-line therapy.
Primary Testicular Tumors
Testicular tumors are uncommon, accounting for only 1–2%
of all pediatric solid tumors [112, 113]. There is a bimodal
age distribution, with the first peak occurring at 2–4 years of
age, and the second peak at 15–18 years. Most pediatric tes-
ticular tumors present as a painless palpable mass or with a
firm, enlarged scrotum. They can be categorized as germ cell
or non-germ cell tumors based on the cell of origin [81]. Up
to 75% of testicular tumors in prepubertal boys are benign,
whereas after puberty, most are malignant [112, 114].
Currently, there is no widely accepted means of distin-
guishing between benign and malignant testicular tumors by
ultrasound. Elastography may be helpful to distinguish tumor
from non-neoplastic benign lesions such as focal orchitis or
segmental testicular infarction, because tumors are typically
stiff [13, 115]. However, both benign and malignant tumors
may have increased stiffness values, and studies with large
numbers of pediatric testicular tumors have not yet been
performed to validate these early findings reported in adults.
CEUS can demonstrate the absence of vascularity, to help
confirm the benignity of a lesion [57].
Table 15.4 summarizes the ultrasound appearance and
typical clinical features of the most frequent testicular
masses.
J. H. Squires and H. J. Paltiel
Table 15.4 Ultrasound and clinical features of pediatric testicular
masses
Mass Ultrasound and Clinical Features
Adrenal rest Well-circumscribed hypoechoic foci along
mediastinum testis with hypervascularity in a
boy with congenital adrenal hyperplasia
Leydig cell Multiple small hypoechoic foci with
hyperplasia hypervascularity
Hamartoma Multiple well-defined hyperechoic foci
Simple cyst Anechoic round focus with no perceptible wall
and posterior acoustic enhancement
Epidermoid cyst Well-circumscribed echogenic mass with
“onion-ring” lamellated appearance
Sinus Hypervascular masses in the testes and
histiocytosis epididymides in a boy with cervical
lymphadenopathy
Mixed germ cell Heterogeneous mass with possible cyst
tumor formation
Yolk sac tumor Solid focal hypervascular mass
Teratoma Well-circumscribed complex mass with possible
internal echogenic fluid or calcification
Seminoma Well-defined, homogeneously hypoechoic
hypervascular mass, more commonly seen in
fourth decade of life
Gonadoblastoma Heterogeneous mass with cystic and solid areas
in a phenotypic female with a Y chromosome
Sertoli cell tumor Well-circumscribed round or lobular mass
Large calcifications can occur in patient with
Peutz-Jeghers syndrome
Leydig cell tumor Heterogeneous testicular mass with internal
hemorrhage/necrosis in a male with
precocious puberty
Granulosa cell Multicystic mass with complex appearance in a
tumor male with abnormal Y chromosome or
disorder of sex differentiation
Germ Cell Tumors
Germ cell tumors comprise approximately 60% to 75% of
all testicular tumors in childhood [112]. Yolk sac tumor and
teratoma are the most common, with other germ cell tumor
types including seminoma, gonadoblastoma, embryonal car-
cinoma, teratocarcinoma, and choriocarcinoma.
On ultrasound, malignant germ cell tumors are heteroge-
neous in appearance with hyperechoic foci related to calci-
fication and cystic zones reflecting necrosis or hemorrhage.
They may demonstrate prominent vascularity with color
Doppler (Fig. 15.50).
Rarely, a germ cell tumor can present with bulky ret-
roperitoneal nodal masses, but with a normal-appearing
testis, a small testicular lesion, or a small calcific focus, a
so-called “burned-out” or “Azzopardi” tumor [116, 117].
Yolk Sac Tumor
Yolk sac tumor is the most common prepubertal testicular
malignant tumor, accounting for up to 80% of cases [114],
and usually developing in boys under 2 years of age [112].
15 Male Genital Tract
a
b pubertal male [81]. An adolescent with a malignant teratoma
Fig. 15.50 Mixed germ cell tumor in an 18-year-old male. (a) Sagittal
grayscale ultrasound image shows a heterogeneous mass (arrowheads)
in the inferior aspect of the testis with a small amount of adjacent fluid
(asterisk). (b) Sagittal color Doppler ultrasound image shows blood
flow within and surrounding the tumor
In prepubertal patients, most yolk sac tumors present at a
low stage [113], with only 4–6% having retroperitoneal or
pulmonary metastases [112]. Tumors manifest as a painless
mass with elevated serum alpha-fetoprotein (AFP) levels.
At ultrasound, a yolk sac tumor appears as a solid, focal
mass and may be hyperechoic, iso-echoic, or hypoechoic,
often with heterogeneity that may be related to intratumoral
hemorrhage and necrosis (Fig. 15.51). Color Doppler imag-
ing will demonstrate hypervascularity within and surround-
ing the tumor.
Treatment involves resection with radical orchiectomy
and may include chemotherapy. Screening for pulmonary
metastases should be obtained. Lymph node dissection is
performed in children with retroperitoneal lymphadenopa-
thy and elevated or increasing AFP levels [81, 114].
Teratoma
Mature teratoma is the second most common germ cell
tumor in boys, and contains all three germ cell layers, includ-
ing endoderm, mesoderm, and ectoderm [81]. Thirty to forty
percent of testicular tumors in the prepubertal age group are
mature teratomas [112]. There is a peak incidence at just
over 1 year of age up to about 4 years of age. Teratomas in
prepubertal males are benign, but may be malignant in ado-
lescents [114].
663
At ultrasound, a teratoma appears as a well-circumscribed,
complex mass (Fig. 15.52). Cystic components are common
and can contain echogenic serous, mucoid, or keratinous
fluid. Echogenic foci representing cartilage, calcification,
and fibrosis may also be present, with or without posterior
acoustic shadowing.
Treatment depends on patient age. In a prepubertal male
with normal AFP levels, testis-sparing surgical resection is
performed rather than radical orchiectomy. Postoperative
imaging follow-up is not recommended [112, 113]. This is
in contrast to the treatment of a malignant teratoma in a post-
is treated in a fashion similar to adults, with radical orchiec-
tomy as well as staging and surveillance imaging. Staging is
performed according to the Tumor, Node, Metastasis (TNM)
classification.
Seminoma
Although a seminoma is the most common testicular germ
cell tumor in males, it occurs extremely rarely in the pedi-
atric population, typically in an adolescent male. The mean
age at diagnosis of seminoma is 35–39 years. It usually pres-
ents as a solid, palpable painless mass [81, 116]. Beta-human
chorionic gonadotropin and lactate dehydrogenase may be
elevated, while AFP levels are typically normal. At ultra-
sound, a seminoma appears as a variable sized, well-defined,
homogeneously hypoechoic mass [13, 116]. Contours are
lobulated and color Doppler will demonstrate hypervacular-
ity. At strain elastography, seminomas will be uniformly stiff
[13]. At CEUS, there will be rapid enhancement compared
to adjacent testicular parenchyma, and early washout often
occurs [57].
Gonadoblastoma
Gonadoblastomas occur almost exclusively in patients with
a Y chromosome and gonadal dysgenesis, or streak gonads
in the setting of Turner syndrome. Gonadoblastomas contain
both sex cord-stromal elements and germ cells [81] and may
present with virilization. At ultrasound, these tumors have
been described as heterogeneous, with cystic and solid areas.
They are the most common tumors associated with disorders
of sex differentiation, with a risk of development as high as
25%, and age of presentation as young as 5 years [112, 118].
For this reason, streak gonads are surgically removed to pre-
vent the development of gonadoblastoma. Prognosis for these
patients is favorable because these tumors are radiosensitive.
Embryonal Carcinoma
Embryonal carcinoma usually presents at 25–35 years of
age and is the most common component of mixed germ cell
tumors. A pure embryonal carcinoma is rare. Its behavior
is aggressive, with invasion of local structures including
the tunica albuginea. At ultrasound, embryonal carcinomas
664
a a
Fig. 15.51 Yolk sac tumor in an 18-year-old male. (a) Sagittal gray-
scale ultrasound image demonstrates complete replacement of the right
testis by a heterogeneous mass. (b) Axial contrast-enhanced CT image
of the abdomen shows a metastatic retroperitoneal lymph node (arrow)
are heterogeneous and have ill-defined margins. Staging is
according to the TNM classification and treatment is radical
orchiectomy with chemotherapy [81].
Teratocarcinoma
Teratocarcinoma is very rare and is a mixture of differenti-
ated tumor cells that form a variety of tissue structures and
undifferentiated stem cells, similar to embryonal carcinoma
cells [119, 120]. No specific ultrasound appearance has been
reported. Teratocarcinomas are resistant to chemotherapy
and treatment is surgical removal.
Choriocarcinoma
Choriocarcinoma has the worst prognosis of all of the germ
cell tumors, and is rare in its pure form. It more frequently
occurs as a component of a mixed germ cell tumor where the
prognosis is slightly better [81]. A mixed germ cell tumor
J. H. Squires and H. J. Paltiel
Fig. 15.52 Teratoma in a 14-year-old male. (a) Transverse grayscale
ultrasound image of the left testis shows a well-circumscribed, hetero-
geneous lesion (arrowheads) below the mediastinum testis that contains
echogenic foci. (b) Sagittal color Doppler ultrasound image reveals no
internal flow
with choriocarcinoma components and human chorionic
gonadotropic levels >50,000 portends a poor prognosis.
Unlike other germ cell tumors which spread via the lym-
phatics, a pure choriocarcinoma typically demonstrates early
hematogenous spread. No specific ultrasound appearance
has been reported. Choriocarcinoma usually presents in
the second and third decades of life, often with widespread
metastatic disease to the lung, liver, gastrointestinal tract,
and brain. Survival beyond 1 year after diagnosis is unusual.
Staging is according to the TNM classification.
Stromal Tumors
Stromal tumors are rare in children, although the incidence
is relatively higher than in adults. These tumors are usually
benign, and include Leydig cell, Sertoli cell, and granulosa
cell tumors [81, 112]. In adults, there is some evidence that
15 Male Genital Tract
b a
Fig. 15.53 Leydig cell tumor in a 7-year-old male. (a) Sagittal gray-
scale ultrasound image shows a small, hypoechoic lesion (arrow) in the
lower portion of the testis with a surrounding echogenic rim. Scattered
microcalcifications are present in the testicular parenchyma. (b) Sagittal
color Doppler ultrasound image shows vascular flow within the nodule
in keeping with a solid mass
stromal tumors will demonstrate early arterial enhancement
at CEUS that persists beyond that in the adjacent testicular
parenchyma [57].
Leydig Cell Tumor
Leydig cell tumor is the most common testicular stromal
tumor, with a peak age of incidence at 4–5 years. This tumor
also occurs in males 10–30 years of age as well as in men
over the age of 50 years [81, 112]. Because Leydig cells pro-
duce testosterone, about 30% of young male patients will
present with precocious puberty. Estrogen production also
occurs, associated with gynecomastia or decreased libido in
older patients. At ultrasound, Leydig cell tumors are small
and solid, with cystic areas related to hemorrhage or necrosis
(Fig. 15.53). Treatment is surgical, consisting of either orchi-
ectomy or testis-sparing surgery, with tumor enucleation.
Sertoli Cell Tumor
Sertoli cell tumors are uncommon, comprising about 1% of
all pediatric testicular tumors. However, they are the second
most frequent pediatric testicular stromal tumor [81, 112],
occurring between 4 months and 10 years of age. They can
rarely present with gynecomastia or precocious puberty. At
ultrasound, Sertoli cell tumors are usually unilateral, round
or lobulated, and well-circumscribed.
665
b
Fig. 15.54 Large-cell calcifying Sertoli cell tumor discovered inciden-
tally in an 18-year-old male. (a) Sagittal and (b) transverse grayscale
ultrasound images reveal a tiny hypoechoic nodule with a central punc-
tate calcification
In young patients, a large-cell calcifying Sertoli cell tumor
may develop, where there can be multiple bilateral calcified
masses (Fig. 15.54). Large-cell calcifying Sertoli cell tumors
are associated with Peutz-Jeghers syndrome and Carney
syndrome. As with Leydig cell tumors, treatment is surgical,
either orchiectomy or testis-sparing surgery (particularly in
prepubertal males) with tumor enucleation. Metastases are
rare.
Granulosa Cell Tumor
Testicular granulosa cell tumors are divided into juvenile and
adult types. Although exceedingly rare, juvenile granulosa
cell tumor is the most common testicular tumor occurring
in infants less than 6 months of age [112]. There is a sec-
ond peak in incidence in young adulthood. There may be
structural abnormalities of the Y chromosome and disorders
of sex development are common. Granulosa cell tumors are
usually hormonally inactive and serum markers are nega-
tive [121]. At ultrasound, they appear as a multicystic mass
with a complex appearance (Fig. 15.55). Treatment consists
of orchiectomy or testis-sparing tumor enucleation surgery.
666 J. H. Squires and H. J. Paltiel

Chromosomal analysis is recommended in patients diag-

nosed with this tumor.

Other Testicular Tumors

Epidermoid Cyst
Epidermoid cysts account for about 15% of pediatric tes-
ticular tumors [81, 112]. The cysts contain a laminated or
layering cheesy material, which explains their characteris-
tic “onion-skin” appearance on ultrasound [81, 113]. They
are well circumscribed, round or ovoid, with a hyperechoic
and occasionally calcified wall (Fig. 15.56). At strain elas-
tography, they display increased stiffness. At CEUS, there
is no internal enhancement [57]. Treatment is typically tes-
ticular-sparing enucleation, rather than orchiectomy [122].
The presence of irregular borders should raise concerns for
possible malignancy, including teratoma, rather than an epi-
dermoid cyst.

Dermoid Cyst a
Testicular dermoid cysts are rare, benign lesions distinct from
epidermoid cysts. They also differ from mature teratomas
as well [123]. Patients present with a painless palpable
mass, usually from the age of 17 to more than 40 years.
Pathologically, they contain keratinizing epithelium, sebum,
and hair. On ultrasound, a dermoid cyst appears as a well-
circumscribed, hypoechoic mass with homogeneous internal
low-level echoes or fine linear echogenic foci. There is no
internal vascularity, similar to the more common epidermoid
cyst [124]. Treatment is enucleation or orchiectomy.

Fibroma
Fibroma of the testis is a very rare tumor, with fewer than 30
cases reported, and is usually paratesticular in location [125]. b
These tumors are considered to have a gonadal stromal origin,
with or without minor sex cord elements, and are analogous to
similarly named tumors of the ovary [126]. Patients are usually
older in age (16–67 years), although the tumor has been diag-
nosed in males as young as 2 years of age. Testicular fibromas
may present as either a painful or painless slow-growing mass.
They are not associated with abnormal hormonal activity. At
ultrasound, a fibroma appears homogeneous and well circum-
scribed. Treatment consists of orchiectomy.

Neurofibroma
Testicular neurofibroma is a benign nerve sheath tumor usu-
ally seen in association with neurofibromatosis type 1, and
very rarely occurring in isolation. At ultrasound, a neurofi-
broma appears hypoechoic and well circumscribed [127]. c
Treatment is surgical resection.
Fig. 15.55 Juvenile granulosa cell tumor in a 7-day-old male.
Transverse (a) and sagittal (b) grayscale ultrasound images of the left
hemiscrotum demonstrate a multicystic mass replacing most of the left
testis. (c) Transverse color Doppler ultrasound image shows enlarged
vessels at the periphery of the mass
15 Male Genital Tract 667

Lipoma
a
Intratesticular lipomas are rare, and are more commonly
encountered arising from the spermatic cord [128]. They
can occur in patients with Cowden syndrome [107]. At ultra-
sound, these tumors appear homogeneously hyperechoic.

Hemangioma
Capillary hemangioma of the testis is very rare, with fewer
than 100 reported cases [129] and are of unknown etiol-
ogy [130]. The term “hemangioma” is most likely outdated
b based on the 2018 classification of the International Society
for the Study of Vascular Anomalies because these lesions
are not true vascular neoplasms, and are more aptly termed
“capillary malformation” [131]. Because they are vascular
malformations, serum tumor and hormonal markers will be
negative.
At ultrasound, they are described as heterogeneously
hypoechoic with irregular margins and marked hypervascu-
larity with color Doppler (Fig. 15.57). They can be difficult
to distinguish from true neoplasms. At CEUS, they have
been reported to display hyperenhancement and centripetal
Fig. 15.56 Epidermoid cyst in a 17-year-old male. Sagittal (a) and
transverse (b) grayscale ultrasound images of the left testis demon- filling [130]. Given the difficulty in prospectively excluding
strate a lobulated mass (arrowheads) with a multi-layered, “onion neoplasia, these lesions are usually surgically resected.
skin” appearance

a c

Fig. 15.57 Benign capillary malformation in an 11-year-old male. (a) images reveal (b) marked hypervascularity of the lesion with (c) arterial
Sagittal grayscale ultrasound image reveals a well-circumscribed, blood flow identified on spectral analysis
hypoechoic nodule in the left testis. Sagittal color Doppler ultrasound
668 J. H. Squires and H. J. Paltiel

Leiomyoma
Testicular leiomyoma is extremely rare, with only a few
reported cases. Leiomyomas are benign, arise from smooth
muscle, and can be found anywhere that smooth muscle
occurs. In the testis, they probably originate from the con-
tractile cells of the seminiferous tubules [132, 133]. They
may develop in the epididymis, spermatic cord, and tunica
albuginea and are typically slow-growing. At ultrasound,
they are hypoechoic with central iso-echogenicity. Although
leiomyomas are benign tumors, they are usually surgi-
cally resected for confirmation of the diagnosis as well as
treatment.

Adenomatoid Tumor
Adenomatoid tumors are benign and usually arise from
the epididymis. They are considered to be of mesothe-
lial origin and consist of cords and tubules of cuboidal
to columnar cells with vacuolated cytoplasm and fibrous
stroma. Testicular adenomatoid tumors arising from the tunica
albuginea are very rare [134]. At ultrasound, testicular
adenomatoid tumors are round and located at the testicular
periphery. They tend to be echogenic and demonstrate vari-
able flow on color Doppler evaluation. They can enhance
at CEUS and have increased stiffness at shear wave elas-
tography, similar to other testicular neoplasms. As with
many other benign testicular lesions, adenomatoid tumors
are usually resected since a preoperative diagnosis is not
generally possible.

Follicular Lymphoma
Follicular lymphoma is an extremely rare primary testicu-
lar tumor that usually occurs in late middle-aged males, but
has been occasionally diagnosed in the pediatric popula-
tion [135]. Prognosis is favorable, with current management
including orchiectomy followed by chemotherapy.
Fig. 15.58 Leukemic testicular involvement in a 15-year-old male. (a)
Sagittal grayscale ultrasound image of the left testis shows a large, geo-
graphic zone (arrowheads) of decreased echogenicity. (b) Transverse
color Doppler image of the scrotum reveals diffuse enlargement of the
left testis (arrow) with marked hyperemia of the hypoechoic zone noted
in image (a)

Secondary Testicular Tumors
Leukemia and Lymphoma
Leukemia and lymphoma are the most common malignan-
cies that spread to the testes in children, but account for
about only about 10% of all pediatric testicular masses [136].
Burkitt lymphoma involves the testis up to 4% of the time
and may be site of the initial presentation. Pediatric patients
with acute lymphoblastic leukemia and bulky disease at
diagnosis have up to a 20% testicular relapse rate [112].
At ultrasound, the involved testis may be diffusely
enlarged and hypoechoic, or multifocal hypoechoic nodules
may be seen [137]. Lesions will be hypervascular at color
Doppler imaging (Fig. 15.58) [96]. Elastography shows
increased stiffness of lymphomatous infiltrates [13].
In the past, testicular involvement with primary leukemia
and lymphoma was treated with radiation, which is no longer
standard therapy. Newer chemotherapy regimens are more
effective in treating extramedullary disease sites. Radiation
is only indicated for patients with persistent testicular dis-
ease after repeat induction chemotherapy [138].
Neuroblastoma
Neuroblastoma is the most common extracranial tumor in
childhood and arises from immature neural crest cells. Scrotal
involvement is rare, with only 30 cases of testicular and para-
testicular involvement reported [139]. In patients with scrotal
involvement, a paratesticular location is more common and
there is usually extensive abdominal and retroperitoneal dis-
ease. The ultrasound appearance has been described as solid,
15 Male Genital Tract 669

hypoechoic masses with well-defined margins and prominent

vascularity on color Doppler imaging [139, 140]. Treatment
includes chemotherapy and surgical resection.

Wilms’ Tumor
Wilms’ tumor usually arises from the kidney, and rarely from T
an extrarenal site. Testicular metastases from primary renal
Wilms’ tumor are very rare, with potential spread from the
ipsilateral gonadal vein [141, 142]. Metastatic Wilms’ tumor
has been described at ultrasound as heterogeneous in appear-
ance. Treatment includes chemotherapy, surgical resection, a
and possible radiation, with an overall good prognosis.

 angerhans Cell Histiocytosis

L
Langerhans cell histiocytosis (LCH) is a rare disease with
many potential sites of involvement, most commonly bone
[143]. Testicular involvement is rare. Although recurrence
T
rates are high, mortality from LCH is relatively low unless
there is multisystemic involvement and the patient is less
than 2 years of age. No specific ultrasound appearance has
been described for testicular involvement by LCH. Diagnosis
requires biopsy. Treatment consists of chemotherapy.

Carcinoid Tumor
Carcinoid tumor is a potentially malignant neoplasm, which
may secrete 5-hydroxyindoleacetic acid [144]. Carcinoid
tumor of the testis can arise as a metastasis from an extrates-
ticular primary, as a component of a teratoma, or as a primary
tumor, with primary tumor being most common [145, 146].
There is no age predilection. The ultrasound appearance is
heterogeneously solid, or mixed cystic and solid with hyper-
vascularity. Treatment is surgical excision with orchiectomy,
adjuvant chemotherapy, and possible radiation.
Rhabdomyosarcoma
Primary testicular rhabdomyosarcoma is rare, with less than
20 reported cases. More commonly, rhabdomyosarcoma
involves the paratesticular region of the scrotum. Case reports
describe the ultrasound appearance as homogeneous and
solid [147]. Primary testicular rhabdomyosarcoma is clini-
cally aggressive and recurs frequently. Treatment includes
surgical resection, chemotherapy, and possibly radiation.
Retinoblastoma
Retinoblastoma is very curable in the developed world
because it is usually diagnosed when disease is confined to
the retina. Extraocular involvement is uncommon but can
occur if there is a large amount of choroidal invasion [148].
Testicular metastasis from retinoblastoma is rare but has
been described [2]. The ultrasound appearance of testicular
retinoblastoma has not been specifically reported. Treatment
includes high-dose chemotherapy and autologous stem cell
rescue.
b
Fig. 15.59 Spermatocele in a 19-year-old male. Sagittal (a) and trans-
verse (b) grayscale ultrasound images of the scrotum show a large,
anechoic cyst in the head of the right epididymis. T, Right testis
Paratesticular Masses
Non-Neoplastic Lesions
Spermatocele
A common extratesticular cyst is a spermatocele, which con-
sists of dilated efferent ducts in the head of the epididymis
[3]. It develops after puberty, and usually occurs as a result
of prior injury or inflammation [149]. At imaging, spermato-
celes are almost always anechoic, with internal echogenic,
dependent foci related to proteinaceous material and sper-
matozoa (Fig. 15.59). They are usually unilocular, occasion-
ally multilocular, and measure up to 2 cm in diameter. In
adults, there is an increased incidence following vasectomy.
No treatment is typically necessary unless there is associated
discomfort from mass effect.
Epididymal Cyst
Similar to a spermatocele, an epididymal cyst consists of
dilated efferent ducts in the head of the epididymis, but does
not contain sperm [149]. Its appearance is identical to that of
a spermatocele, and clinically the distinction is likely irrel-
evant. There is an increased incidence in boys with in utero
exposure to diethylstilbestrol [3]. At ultrasound, the cysts are
670
a [153, 154]. Intratesticular and paratesticular masses have
b spontaneous regression, although surgical resection may some-
Fig. 15.60 Epididymal cyst in a 13-year-old male. (a) Sagittal (left
panel) and transverse (right panel) split-screen grayscale ultrasound
images show an anechoic focus in the right epididymal head (arrows)
with posterior acoustic enhancement. (b) Sagittal color Doppler ultra-
sound image shows absence of flow within the cyst
almost always located in the epididymal head and appear
anechoic (Fig. 15.60). No treatment is typically necessary
unless discomfort develops from mass effect.
Fibrous Pseudotumor
Fibrous pseudotumor, also called nodular fibrous periorchitis,
fibroma, or inflammatory pseudotumor, is a benign reactive,
non-neoplastic lesion of the spermatic cord, epididymis, and
tunica vaginalis, thought to be caused by an inflammatory
reaction. Age at presentation is quite variable with a history
of a slow-growing, painless mass, although prior infection and
trauma have also been described [38]. At ultrasound, distinc-
tion from a paratesticular rhabdomyosarcoma is not possible
[3, 150]. The fibrous proliferation may be quite extensive
and complex in appearance [151]. Treatment is surgical and
involves local excision with no need for orchiectomy [149].
Fibrous Hamartoma of Infancy
Fibrous hamartoma of infancy is a benign tumor usually
located in the dermis and subcutaneous tissues of children less
than 2 years of age. It may be congenital, and occurs more fre-
quently in males. Although usually found on the trunk, almost
15% of cases occur in the scrotum and are usually solitary.
At ultrasound, the mass will be superficial, heterogeneously
hyperechoic, with a “serpentine pattern” and ill-defined or
lobulated margins. With color Doppler imaging, there is no
associated hyperemia. Treatment is surgical excision [152].
Juvenile Xanthogranuloma
Juvenile xangthogranuloma is an uncommon, benign, non-
Langerhans cell histiocystic proliferative disorder that can
involve the scrotum and usually presents by the age of 2 years
J. H. Squires and H. J. Paltiel
been reported, with epididymal involvement by an infiltra-
tive-appearing mass. At ultrasound, the appearance has been
described as circumscribed, hypoechoic, and hypervascular.
Lesions are typically treated conservatively, with eventual
times be required.
Spermatic Granuloma
A spermatic granuloma can occur after trauma or recurrent
epididymitis, although it is usually diagnosed in post-vasec-
tomy patients [38, 149]. Pathologically, it is a foreign body
giant cell reaction to extravasated sperm. At ultrasound, it
usually appears as a well-circumscribed, solid, hypoechoic
mass of variable size, although most are smaller than 1 cm
in diameter. While most spermatic granulomas spontane-
ously resolve or remain stable, excisional biopsy is often
performed for diagnostic confirmation.
Cystic Dysplasia of Epididymis
Cystic dysplasia of the epididymis is a congenital anomaly
that may be seen in the setting of other cystic dysplasias,
including renal cystic dysplasia, cystic dysplasia of the rete
testis, ureteral obstruction, vesicoureteral reflux, posterior
urethral valves, and congenital absence of the vas defer-
ens [149]. The etiology may be related to a disorder of
mesonephric differentiation [155]. At ultrasound, there is
tubular dilation of the small ducts within the epididymis,
with an appearance similar to that of tubular ectasia of the
rete testis. Prognosis typically depends on the associated
anomalies.
Ectopic Adrenal Rest
Ectopic adrenal tissue in the setting of congenital adrenal
hyperplasia usually occurs in the testis, as discussed ear-
lier in this chapter, but can also occur in the extratesticular
soft tissues, usually the spermatic cord [149]. Spermatic
cord rests are usually found in conjunction with intrates-
ticular rests and the ultrasound appearance is similar, with
variable echogenicity and hypervascularity compared to
the adjacent testicular parenchyma. Treatment requires
attention to the underlying endocrinopathy, not surgical
resection.
Vascular Anomalies
Lymphatic Malformation
Lymphatic malformations are soft masses composed of
lymphatic vessels that may involve the scrotum, are slow-
growing, and can be complicated by infection or hemorrhage
[149]. They can be macrocystic, microcystic, or combined
with microcystic lymphatic channels less than 1-2 cm in
diameter. At ultrasound, they often appear as multiloculated
15 Male Genital Tract
a b
c
Fig. 15.61 Lymphatic malformation of the scrotum in a 13-year-old
male. Transverse (a) and sagittal (b) grayscale ultrasound images of the
scrotum demonstrate a right-sided extratesticular multicystic mass con-
forming to the shape of the hemiscrotum. (c) Sagittal color Doppler
cystic lesions with a modest degree of vascular flow detected
in the septations (Fig. 15.61). These lesions are generally
treated with percutaneous sclerotherapy when symptomatic,
although surgical excision may be required.
Venous Malformation
Venous malformations are slow-flow vascular malforma-
tions that may involve the scrotum and clinically pres-
ent with swelling and bluish skin discoloration related to
venous stasis and thrombosis [149]. At ultrasound, they
appear as dilated, hypoechoic, serpentine channels with
slow or minimal venous flow and scattered phleboliths.
Similar to lymphatic malformations, venous malforma-
tions are usually treated with sclerotherapy and/or surgical
resection.
Arteriovenous Malformation
Arteriovenous malformation (AVM) of the scrotum is a rare
high-flow congenital lesion [149]. Similar to AVMs else-
where, it consists of a mass of dilated vessels with abnormal
671
d
ultrasound image reveals a small amount of blood flow in the septa of
the lesion. (d) Axial T1-weighted, fat-suppressed magnetic resonance
(MR) image shows mild septal enhancement (arrow) of the lesion
communications between arteries and veins, and may pres-
ent clinically with a palpable thrill on physical examination.
Spectral Doppler evaluation of vessels within the malfor-
mation will identify low resistance arterial flow and venous
waveforms will be arterialized. AVMs are treated with embo-
lization at angiography.
Benign Tumors
Lipoma
Lipoma is the most common benign tumor of the parates-
ticular soft tissues, comprising nearly 50% [151], and may
be found incidentally during surgery [150]. Paratesticular
lipomas are painless. At ultrasound, they are well-cir-
cumscribed masses that are variable in size and usually
appear homogeneously echogenic (Fig. 15.62). They may
mimic non-fatty lesions when they appear hypoechoic
compared to the adjacent soft tissues [156]. Treatment is
only required for symptomatic lesions which are surgi-
cally excised [157].
672
T
a is recommended to avoid unnecessary orchiectomy and
b Treatment consists of local excision. There have been no
c diagnosis and to prevent recurrence.
Fig. 15.62 Lipoma of the spermatic cord in a 15-year-old male. (a)
Sagittal grayscale ultrasound image of the left hemiscrotum shows
echogenic soft tissue (arrows) adjacent to the upper pole of the left tes-
tis (T). (b) Sagittal grayscale and color Doppler (c) ultrasound images
of the left groin show the fatty mass (arrows) completely surrounding
the vessels (arrowheads) of the spermatic cord which are patent
Adenomatoid Tumor
Adenomatoid tumor is the most common neoplasm of the
epididymis, usually involving the epididymal head or, less
frequently, the lower pole. It occurs most often in males
older than 20 years of age [151]. Occasionally, it may arise
from the testicular tunica, spermatic cord, or the testis, as
described earlier in this chapter [151].
The ultrasound appearance of adenomatoid tumor is
variable, but is usually well circumscribed and hyper-
J. H. Squires and H. J. Paltiel
echoic or isoechoic relative to the adjacent epididymis.
Adenomatoid tumors are usually small in size (< 2 cm
in diameter) and hypovascular compared to the adjacent
epididymis [149]. At CEUS, they have been described as
demonstrating enhancement followed by early contrast
washout [57].
Because the distinction of adenomatoid tumor from para-
testicular rhabdomyosarcoma (described later in this chap-
ter) may be challenging, intraoperative tumor frozen section
thereby restrict surgery to local excision [149].
Leiomyoma
Leiomyoma is comprised of smooth muscle, and is the sec-
ond most common epididymal tumor, usually diagnosed in
older men and much more rarely in the pediatric population.
It presents as a painless, slowly growing mass. At ultrasound,
these tumors usually involve the epididymal head, are well-
circumscribed, solid or cystic, and can contain calcifications
[151]. Internal flow may be seen with color Doppler imag-
ing [156]. Half of all epididymal leiomyomas are associ-
ated with a hydrocele. Although they are benign lesions, a
definitive diagnosis is challenging to make preoperatively.
reports of recurrence to date.
Dermoid
Dermoid of the paratesticular soft tissues is very rare, especially
in children, with reports describing involvement of the sper-
matic cord and intertesticular region [158, 159]. Dermoid cysts
contain keratin, hair follicles, sweat, and sebum, and are well
circumscribed and mobile on physical examination. Ultrasound
is useful in localizing the tumor, although the imaging appear-
ance is non-specific. Surgical resection is recommended for
Neurofibroma
As previously discussed, solitary neurofibroma of the scro-
tum is a very rare benign, peripheral unencapsulated nerve
sheath tumor composed of Schwann cells and fibroblasts
[127]. There is low malignant potential when not associ-
ated with neurofibromatosis type 1 (NF1), and up to 4%
malignant degeneration potential when associated with
NF1. In the scrotum, a few case reports exist, with most
arising from the spermatic cord and a few intratesticular
in location. At ultrasound, neurofibroma appear well cir-
cumscribed, homogeneously hypoechoic, with minimal
internal vascularity seen with color Doppler. Surgical exci-
sion is recommended for diagnosis and treatment.
Papillary Cystadenoma
Epididymal papillary cystadenoma is rare, but has been reported
sporadically in children. It occurs in a majority of male patients
15 Male Genital Tract
with von Hippel-Lindau disease [3, 151]. These lesions arise
from the ejaculatory duct and range in size from about 1 to
4 cm. The ultrasound appearance is variable, ranging from
almost completely cystic to solid. Tumors usually grow
slowly and may cause dilation of the ejaculatory ducts. Local
excision of epididymal papillary cystadenoma with testis-
sparing surgery is usually possible, although some patients
do undergo orchiectomy.
Malignant Tumors
Primary Tumors
Rhabdomyosarcoma
Up to 7% of rhabdomyosarcomas involve the paratesticular
soft tissues and frequently occur in infants and children [38,
151, 156]. There is a bimodal age distribution, with peaks
at 5 and 16 years of age. The embryonal subtype is most
common and patients usually present with painless swelling
[150].
At ultrasound, the appearance of rhabdomyosarcoma is
variable, but is usually predominantly solid with internal foci
of cystic degeneration and hemorrhage (Fig. 15.63). Color
and spectral Doppler will demonstrate internal vascularity
with low resistance arterial waveforms.
The Rhabdomyosarcoma is locally aggressive and may
invade the adjacent scrotal tissues, the testis and tunica albu-
ginea. At presentation, it is often very large, and metastases
are present in 40% of patients, to lymph nodes, lungs, and
bone. Treatment requires aggressive surgical resection [149].
Inflammatory Myofibroblastic Tumor
Inflammatory myofibroblastic tumor (IMT) is a tumor of
spindle cell proliferation and chronic inflammatory infil-
trate and is similar to a sarcoma [160, 161]. It can arise
from the soft tissues, viscera, and occasionally from the
genitourinary system, usually the urinary bladder. Very
Fig. 15.63 Paratesticular rhabdomyosarcoma in a 15-year-old male.
Sagittal grayscale ultrasound image demonstrates a heterogeneous
mass (arrowhead) superior to the left testis
673
rarely, it can involve the testis or spermatic cord with a
diagnosis made by histopathology. Ultrasound is useful
in localizing the tumor, although its imaging appearance is
not well described.
While benign, IMT is locally aggressive and treatment
involves complete resection, with testis-sparing surgery if
possible. There are new anaplastic lymphoma kinase (ALK)-
inhibitor chemotherapy agents such as Crizotinib that can be
used for ALK-positive cases.
Liposarcoma
Liposarcoma is uncommon in children, but does occur rela-
tively more often in patients more than 16 years of age [38,
156]. At ultrasound, liposarcoma appears similar to a lipoma,
but is more complex and heterogeneous. It can also manifest
as a solid, hypoechoic mass, and may contain calcifications.
Liposarcoma is usually vascular on color Doppler imaging.
The diagnosis may be challenging to make by ultrasound
[38]. Complete surgical resection is usually necessary for
both diagnosis as well as treatment.
Secondary Tumors
Wilms’ Tumor
As noted previously, paratesticular metastases from Wilms’
tumor are rare, and may involve the testis, epididymis, or
spermatic cord. Proposed mechanisms of spread include hema-
togenous, retrograde venous, lymphatic, and transperitoneal
via a patent processus vaginalis. At ultrasound, a parates-
ticular mass is identified, sometimes with an accompanying
hydrocele [2, 162]. Treatment includes surgical resection,
chemotherapy, and occasionally radiation [142].
Neuroblastoma
As previously discussed, paratesticular metastases from neu-
roblastoma are rare [2], and usually indicate advanced dis-
ease [163]. Treatment includes resection and chemotherapy
with stem-cell transplantation a potential option.
Leukemia
Leukemia can involve the paratesticular tissues, sometimes
in association with testicular involvement [156]. At ultra-
sound, tumors appear homogeneously hypoechoic and infil-
trative, although vascular distortion may be minimal, unlike
other paratesticular tumors. Treatment with chemotherapy is
similar to that given for other sites of involvement.
Lymphoma
Extratesticular lymphoma is less common than testicular
lymphoma, but can occur in the epididymis and spermatic
cord [38]. Although involvement is usually microscopic, it
can manifest at ultrasound as multiple nodules or diffuse
infiltration, and usually occurs in the setting of disease recur-
674
rence rather than at initial presentation. Treatment is with
chemotherapy.
Leiomyosarcoma
Leiomyosarcoma is a rare malignant tumor of smooth mus-
cle origin, usually arising from the spermatic cord in older
males in the sixth decade of life [156].
Fibrosarcoma
Fibrosarcoma is a rare malignant tumor involving the
spermatic cord and usually presents as a unilateral, firm,
slowly enlarging mass [164]. It occurs most often in older
males in the seventh decade of life [156].
Prostate and Seminal Vesicles
Introduction
The prostate gland and seminal vesicles are infrequently the
primary organs of interest when imaging children. However,
when investigation is necessary, ultrasound is the initial imaging
modality of choice. More often, the prostate gland and seminal
vesicles are evaluated during the workup of other abnormalities
of the lower genitourinary tract [165].
Technique
Patient Positioning
The pediatric prostate gland and seminal vesicles can usually
be well evaluated from a transabdominal approach with the
patient in a supine position, ideally with a distended urinary
bladder [166]. When a transperineal approach is necessary,
the patient is positioned supine with the transducer placed
immediately below the scrotum to include the ventral aspect
of the base of the penis in young boys [167]. In adolescents,
a modified lithotomy position is optimal, using a bed with
stirrups if available.
Ultrasound Transducer Selection
A 5–7 MHz transducer is used for transabdominal imaging.
If necessary, a higher-frequency transducer, on the order of
7–12 MHz, can be used for transperineal imaging.
Imaging Approaches
Both the prostate gland and seminal vesicles are imaged
in the transverse and sagittal planes. Color and pulsed
Doppler imaging parameters should be optimized for the
detection of low blood flow. Both static and cine clips are
obtained, and prostate gland volume measurements are
performed.
J. H. Squires and H. J. Paltiel
Normal Development and Anatomy
Normal Development
Prostate gland development begins at about 10 weeks of ges-
tation from endodermal protrusions that arise from the pos-
terior urogenital sinus. The prostatic utricle develops from a
remnant of the paramesonephric (müllerian) duct [165, 168].
The verumontanum is a round eminence on the posterior
wall of the prostatic portion of the urethra, which forms from
components of the urogenital sinus and müllerian duct. The
seminal vesicles also begin to form around the tenth week
of gestation from the mesonephric (wolffian) duct [6, 169].
Because seminal vesicle development is closely related to
development of the vas deferens, kidneys, and ureters, abnor-
malities frequently occur together [170].
Normal Anatomy
Prostate Gland
The prostate gland is a walnut-shaped structure located in the
deep pelvis surrounding the proximal urethra (the prostatic
portion) and ejaculatory ducts [168]. The gland is conical in
shape, and is situated superior to the urogenital diaphragm
and neck of the urinary bladder. It contains glandular and
stromal elements within a pseudocapsule and produces semi-
nal fluid. The base of the prostate is located superiorly and
the apex inferiorly. It is subdivided into a central zone, tran-
sition zone, peripheral zone (the largest component of the
gland), and anterior fibromuscular stroma.
Prior to puberty, the prostate gland does not have a well-
defined lobar anatomy. In young males, prostate volume
ranges from 0.4 to 5.2 mL and increases to 12–20 mL during
adolescence [165]. At ultrasound, the prostate is a hypoechoic
structure seen at the base of the urinary bladder (Fig. 15.64).
B
Fig. 15.64 Normal prostate gland in an 11-year-old male. Transverse gray-
scale ultrasound image shows the prostate gland (arrowheads). B, Bladder
15 Male Genital Tract
B bladder neck, grade 2 extending over the bladder neck, and
Fig. 15.65 Normal seminal vesicles in a 17-year-old male. Transverse
grayscale ultrasound image depicts the normal oval, hypoechoic appear-
ance of the seminal vesicles (arrowheads) located posterior to the blad-
der (B) and superior to the prostate gland (not shown)
Seminal Vesicles
The seminal vesicles are paired, tubular, extraperitoneal
structures in the pelvis at the base of the prostate gland,
located posterior to the urinary bladder and anterior to the
rectum [170]. The seminal vesicles produce and secrete sem-
inal fluid and are located posterior to the trigone of the blad-
der and ureters [171]. They enlarge over time with increasing
patient age, and a slight size asymmetry is common. The
seminal vesicles join the distal portions of the vasa defer-
entia to become the ejaculatory ducts, which drain into the
prostatic urethra through the verumontanum.
In adolescents and young men, seminal vesicle volume
is normally 13.7 +/− 3.7 mL [171]. Normal pediatric semi-
nal vesicle volumes have not been reported. At ultrasound,
the seminal vesicles are elongated, tubular, hypoechoic, or
multiseptated cystic structures located just above the prostate
gland (Fig. 15.65).
Congenital Anomalies
 nlarged Prostatic Utricle and Prostatic Utricle Cyst
E autosomal dominant polycystic kidney disease [169]. At
The prostatic utricle can be seen when it is enlarged, as in
the setting of trisomy 21, hypospadias, posterior urethral
valves, or imperforate anus [165]. When larger than 1 cm,
an enlarged prostatic utricle is sometimes called a prostatic
675
utricle cyst. This cyst results from incomplete degeneration
of the müllerian duct or decreased androgen stimulation of
the urogenital sinus [169]. The cyst communicates with the
urethra. It is an uncommon anomaly and is usually discov-
ered in the first or second decade of life [172].
Grading of a prostatic utricle cyst is based on its appear-
ance at voiding cystourethrography, with grade 0 confined
to the verumontanum, grade 1 located below the urinary
grade 3 opening distal to the external sphincter [172]. At
ultrasound, a prostatic utricle cyst always arises from the
verumontanum, and is a midline fluid-filled structure. The
fluid may appear echogenic if complicated by hemorrhage or
infection (Fig. 15.66).
Unlike müllerian duct cysts (described below), prostatic
utricle cysts do not extend above the base of the prostate [173].
They are usually asymptomatic. However, postvoid leakage of
urine can occur because they communicate with the urethra
[169, 174]. In postpubertal males, they may contain sperma-
tozoa. Urine stasis can lead to infection or stone formation.
A large utricle cyst can compress the bladder neck and may
obstruct the urinary bladder. Treatment options include antibi-
otic therapy, aspiration of fluid, and/or excision.
 üllerian Duct Cyst
M
Müllerian duct remnants can develop into a cyst which
does not communicate with the prostatic urethra, unlike
a prostatic utricle cyst [171]. At ultrasound, it will appear
as an anechoic fluid-filled structure behind the urinary
­bladder [165].
Müllerian duct cysts can be challenging to distinguish
from a prostatic utricle cyst. It can arise slightly above the
verumontanum, may be lateral to the midline, and can extend
superior to the prostate, unlike a utricle cyst [174]. Müllerian
duct cysts are usually asymptomatic, but may present with
urinary retention and infection, and there are case reports of
complication by carcinoma. Treatment includes resection or
percutaneous aspiration [173].
Seminal Vesicle Cyst
Seminal vesicle cysts are usually identified incidentally,
although they can present with hematuria, urinary tract
infection, or infertility [171]. In up to two-thirds of cases,
there is associated ipsilateral renal agenesis or dysgenesis
(Fig. 15.67), along with other genitourinary anomalies,
although it may be an isolated finding or associated with
ultrasound, aneochoic cystic lesions may be visible in the
seminal vesicle [173]. Most seminal vesicle cysts are
asymptomatic [170]. When large, they can cause voiding
difficulty and drainage or excision may be necessary.
676 J. H. Squires and H. J. Paltiel

a d

Fig. 15.66 Prostatic utricle cyst in a 9-year-old male. (a) Transverse
grayscale ultrasound image of the pelvis shows a midline cystic struc-
ture (arrow) behind the urinary bladder (B). (b) Sagittal grayscale ultra-
sound image reveals persistence of the cyst (arrow) after voiding.
B, Bladder. (c) Oblique fluoroscopic image obtained during voiding
cystourethrography shows contrast filling both cyst (arrow) and bladder
(B). (d) Prone whole body image obtained during the late perfusion
phase of a Tc-99m-mercaptoacetyltriglycine (MAG3) nuclear medicine
scan shows a solitary left kidney (arrow)
15 Male Genital Tract 677

a b

c
d

Fig. 15.67 Seminal vesicle cyst in a 13-year-old male. Transverse (a) renal fossa reveals absence of a kidney. (d) Axial T2-weighted, fat-
and sagittal (b) grayscale ultrasound images of the pelvis reveal a cystic suppressed MR image confirms the presence of a seminal vesicle cyst
structure behind the left side of the bladder that contains a fluid/fluid with a fluid/fluid level (arrowhead)
level (arrowhead). (c) Sagittal grayscale ultrasound image of the left

 eminal Vesicle Agenesis or Hypoplasia

S cystic fibrosis as affected males are otherwise healthy, with-
Unilateral seminal vesicle agenesis occurs if there is an in out pulmonary manifestations of cystic fibrosis. Because the
utero insult prior to the seventh week of gestation when the seminal vesicles can be challenging to see by ultrasound in
ureteric bud arises from the mesonephric duct. In almost younger males, MR imaging may be necessary to confidently
80% of cases, it is associated with ipsilateral renal agenesis make this diagnosis [170].
[171]. Bilateral seminal vesicle agenesis is associated with Seminal vesicle hypoplasia is defined as a maximum
cystic fibrosis gene mutations and the kidneys are usually diameter less than 50% of normal or less than 5 mm in
normal. This abnormality is thought to be an isolated form of adults [170], with no diagnostic criteria defined in boys. At
678
ultrasound, one or both seminal vesicles will be small. As
with seminal vesicle agenesis, hypoplasia should prompt
evaluation of the kidneys given the known association with
renal abnormalities.
Inflammatory Disorders
Prostatitis
Prostatitis can occur at any age, but there is increasing inci-
dence with advancing age [174]. Infection is usually bacte-
rial and caused by E. coli, and is thought to be related to
ascending infection from the urethra via reflux or hematog-
enous spread. Patients present with infectious symptoms,
including fever, chills, lower abdominal and perineal pain.
At ultrasound, a hypoechoic rim surrounding the prostate
gland may be seen, there can be hyperemia of the gland by
color Doppler imaging, and hyperenhancement at CEUS.
Prostatitis is treated with antibiotics.
Prostatic Abscess
Prostatic abscess is rare in the pediatric population. It is
much more frequently encountered in middle-aged males
as a result of acute bacterial prostatitis caused by E. coli.
In young males, there may be an associated immunodefi-
ciency such as chronic granulomatous disease, and abscess
occurs as a result of hematogenous spread of organisms
such as S. aureus [175].
At ultrasound, a prostatic abscess is depicted as hypoechoic
foci within the gland and may only be seen with transperineal
imaging. Treatment includes antibiotic therapy and abscess
drainage [173].
Seminal Vesiculitis
Seminal vesiculitis is usually related to bacterial prostati-
tis [171] and also occurs with epididymitis [170]. At ultra-
sound, the seminal vesicle will be diffusely enlarged with
thickening of septa, and may result in abscess formation.
Concomitant prostate enlargement may be noted. Treatment
consists of antibiotic therapy. Drainage may be necessary if
there is abscess formation [170].
Tumors of the Prostate and Seminal Vesicle
Rhabdomyosarcoma
Fifteen percent of cases of rhabdomyosarcoma are genito-
urinary, with up to 10% consisting of bladder and prostate
masses [177, 178]. These tumors are high-grade, fast-grow-
ing malignant mesenchymal neoplasms, and may present
with urinary retention, hematuria, constipation, or with a
palpable mass. The botryoid subtype of rhabdomyosar-
coma arises from the submucosa and may grow as an exo-
phytic, polypoid mass resembling a bunch of grapes. Tumors
J. H. Squires and H. J. Paltiel
originating from the urinary bladder or prostate are of the
embryonal subtype in 90% of cases, with the remainder of
the alveolar subtype.
At ultrasound, rhabdomyosacoma appears heterogeneously
hypoechoic and is hypervascular with color Doppler imaging.
Once a bladder or prostatic rhabdomyosarcoma is suspected,
further staging with MR imaging is performed. Tumors are
aggressive with frequent recurrences. Treatment includes surgi-
cal resection, chemotherapy and sometimes radiation.
Leukemia
Leukemic infiltration of the prostate gland is uncommon, but
has been reported, with a hypoechoic mass that is occasion-
ally identified by ultrasound [179]. Treatment is with sys-
temic chemotherapy.
Seminal Vesicle Tumors
While primary seminal vesicle tumors are very rare, second-
ary involvement is more likely, related either to direct spread
from the adjacent prostate, urinary bladder, rectum or vas
deferens [171].
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 Female Genital Tract
16
Erica L. Riedesel and Harriet J. Paltiel

PID Pelvic inflammatory disease

Abbreviations RMS Rhabdomyosarcoma
SCST Sex chord stromal tumors
2D Two-dimensional SRU Society of Radiologists in Ultrasound
3D Three-dimensional SRY Short arm of the Y-chromosome
AFP Alpha fetoprotein TDF Testis-determining factor
β-hCG Beta human chorionic gonadotropin TOA Tubo-ovarian abscess
CA Cancer antigen UFE Uterine fibroid embolization
CAH Congenital adrenal hyperplasia
CEUS Contrast-enhanced ultrasound
CT Computed tomography
DES Diethylstilbestrol Introduction
DHT Dihydrotestosterone
DSD Disorders of sex development Ultrasound is the primary imaging modality used in the
FEPV Fibroepithelial polyp of the vagina investigation of disorders of the female genital tract, includ-
FSH Follicle-stimulating hormone ing evaluation of ambiguous genitalia, prepubertal bleeding,
GCTs Germ cell tumors primary amenorrhea, pelvic mass, and pelvic pain. Computed
GU Genitourinary tomography (CT) and magnetic resonance (MR) imaging are
HIFU High-intensity focused ultrasound generally reserved for further characterization of congenital
IUD Intrauterine device malformations or tumors.
IUP Intrauterine pregnancy In this chapter, ultrasound techniques used in examination
JGCT Juvenile granulosa cell tumor of the pediatric female genital tract are reviewed, as well as
LDH Lactate dehydrogenase the normal appearance of the uterus and ovaries at differ-
LH Luteinizing hormone ent ages and stages of maturation. Congenital abnormalities
MCT Mature cystic teratoma are discussed, including müllerian anomalies and disorders
MIS Müllerian-inhibiting substance of sex development. An overview of adnexal, uterine, cervi-
MR Magnetic resonance cal, and vaginal pathology is provided with an emphasis on
MRKH Mayer-Rokitansky-Küster-Hauser syndrome ultrasound features.
NHL Non-Hodgkin lymphoma
OHVIRA Obstructed hemivagina and ipsilateral renal
anomaly  echnique: Patient Positioning, Transducer
T
PCOS Polycystic ovary syndrome Selection, and Imaging Approaches

Transabdominal Ultrasound
E. L. Riedesel (*)
Departments of Radiology and Imaging Sciences and Pediatrics,
Children’s Healthcare of Atlanta, Emory University School of Ultrasound is the technique of choice for imaging the pedi-
Medicine, Atlanta, GA, USA atric female pelvis and is performed via an anterior abdomi-
e-mail: erica.riedesel@choa.org
nal approach [1, 2]. Curved and linear array transducers are
H. J. Paltiel generally adequate for transabdominal pelvic ultrasound
Division of Ultrasound, Department of Radiology, Boston Children’s
studies. Higher frequency transducers (3–9 MHz) should
Hospital and Harvard Medical School, Boston, MA, USA

© Springer Nature Switzerland AG 2021 683

H. J. Paltiel, E. Y. Lee (eds.), Pediatric Ultrasound, https://doi.org/10.1007/978-3-030-56802-3_16
684
a b
Fig. 16.1 Normal uterus in a 15-year-old female. Transabdominal sagittal (a) and transverse (b) grayscale ultrasound images readily depict the
uterus using the full bladder as an acoustic window
be used in infants and young children for greater soft tissue
definition. Lower frequency transducers (1–6 MHz) may
be required for deeper penetration in older children and
adolescents.
A well-distended urinary bladder is vital to obtaining
optimal transabdominal images of the pelvic organs. The
fluid-filled bladder acts as an acoustic window and displaces
gas-filled bowel loops. Adequate bladder filling can usually
be achieved with oral fluid intake. Bladder catheterization
with instillation of sterile fluid may be required if more rapid
assessment is needed [1].
Images of the uterus should be obtained in the sagittal
and transverse planes (Fig. 16.1). Angling the transducer
obliquely to the right or left or rolling the patient into a decu-
bitus position may also improve visualization of the ovaries.
Transvaginal Ultrasound
Transvaginal ultrasound is performed with a specialized
transducer placed internally into the upper vagina, allowing
E. L. Riedesel and H. J. Paltiel
for improved visualization of the cervix, uterus, and ovaries.
In the pediatric population, transvaginal imaging is usually
reserved for adolescents who require detailed evaluation of
the pelvic organs at the request of a referring pediatric gyne-
cologist or emergency room physician, or patients who have
significantly limited imaging windows on transabdominal
ultrasound.
Transvaginal ultrasound should not be performed in vir-
ginal patients or in patients who do not willingly consent to
the examination. A chaperone must always be present during
the examination in addition to the sonographer or sonologist
performing the study.
An empty bladder is optimal for transvaginal imaging.
Images of the uterus are obtained in the sagittal and coronal
planes by gently rotating and angling the endovaginal probe
from side to side (Fig. 16.2) [2].
An adequate explanation of the procedure to the patient
is essential. It is important to stress that it is a simple, pain-
less procedure and only a portion of the transducer will be
inserted into the vagina. Patients should be offered the oppor-
tunity to insert the transducer themselves if they so choose.
16 Female Genital Tract 685

a b

Fig. 16.2 Normal uterus in a 17-year-old female. Transvaginal sagittal (a) and transverse (b) grayscale ultrasound images depict uterine anatomy
in exquisite detail

a b

Fig. 16.3 Malpositioned intrauterine device (IUD) in a 17-year-old cervical canal (c) Coronal reconstructed image from three-dimensional
female. Transvaginal sagittal (a) and transverse (b) grayscale ultra- (3D) ultrasound shows the low position (arrowheads) of the IUD
sound images of the uterus demonstrate an IUD (arrows) in the endo-
686
Three-Dimensional Ultrasound
Three-dimensional (3D) ultrasound is a fast-evolving imag-
ing technique that holds great potential for use in adolescent
gynecology [2, 3]. 3D ultrasound can be performed via a trans-
abdominal or a transvaginal approach. Automated 3D transduc-
ers gather two-dimensional (2D) ultrasound information, and
advanced post-processing algorithms generate 3D volume ren-
derings that can be viewed in multiple orthogonal planes. This
can be extremely useful in evaluating suspected congenital uter-
ine anomalies or when evaluating the position of an intrauterine
device (IUD) (Fig. 16.3).
Transperineal Ultrasound
A transperineal (or translabial) ultrasound approach is a
valuable adjunct imaging technique in evaluation of abnor-
malities involving distal gynecological structures, as well as
the urethra, rectum, and anus [1, 4].
Transperineal ultrasound is best performed using a
high-­frequency linear transducer (8 MHz or greater) with
a thick coating of gel over the perineum to eliminate air
artifact. Initially, the transducer is placed in the midline of
the perineum in a longitudinal orientation (Fig. 16.4). The
midsagittal plane is identified by the presence of the pubic
symphysis and urethra on the same image. Superficial
lesions may be better evaluated with a stand-off gel pad.
a b
Fig. 16.4 Transperineal transducer placement for depiction of normal
female anatomy. (a) Sagittal diagram depicts placement of a linear
array transducer on the perineum. (b) Corresponding transperineal sag-
E. L. Riedesel and H. J. Paltiel
Normal Development and Anatomy
Normal Development
The development of the genital system depends on the com-
plex interaction of the genital ridge; two pairs of internal sex
ducts, the wolffian (mesonephric) duct and the müllerian
(paramesonephric) duct; and the genital tubercle.
The genital system begins to develop in the fifth week
of gestation. In the seventh week, the gonadal ridge differ-
entiates into either a testis or ovary as determined by the
sex chromosomes. The embryologic gonads in turn drive
the development of the genital tract and external genitalia.
Gonads and Reproductive Tract
The ovaries descend from the upper abdomen into the pelvis
during fetal development and are typically located at the supe-
rior margin of the broad ligament in the upper pelvis (Fig. 16.5).
The presence or absence of a Y-chromosome determines
the development of the gonads and reproductive tract.
The sex-determining region on the short arm of the
Y-chromosome (the SRY gene) encodes for testis-­determining
factor (TDF), a DNA-binding protein. The presence of TDF
triggers active development of the testis from the genital
ridge.
U V R
ittal grayscale ultrasound image. U, Urethra; V, vagina; R, rectum.
From Paltiel HJ, Phelps A. US of the pediatric female pelvis. Radiology.
2014;270(3):644–57. [1]
16 Female Genital Tract 687

a
b

Fig. 16.5 Normal anatomy of the female pelvis and reproductive system. (a) Sagittal view of female pelvic anatomy. (b) Coronal view of the
uterus, vagina, ovaries and fallopian tubes

a b
Suspensory
ligament Appendix
Paramesonephric of ovary of ovary
duct
Paroophoron Hydatid of
Morgagni
Round ligament
Epoophoron
of ovary
Fallopian tube
Ovary

Round ligament
Uterus of uterus
Mesonephric
Gartner
tubules duct
Gartner
cyst

Vagina
Mesonephric
duct

Müllerian tubercle
(uterovaginal plate)
Urogenital sinus

Fig. 16.6 Normal embryologic development of the female pelvic organs. and cranial two thirds of the vagina. Note the vestigial embryologic
(a) In the seventh week of gestation, the developing ovary stimulates devel- remnants of the mesonephric/wolffian structures that persists as the
opment of the paramesonephric/müllerian structures. (b) Mature configu- epoophoron
ration of müllerian structures comprising the uterus, fallopian tubes,

Within the testis, Sertoli cells secrete müllerian-inhibiting
substance (MIS) which results in complete involution of the
embryologic müllerian duct. Leydig cells produce testoster-
one which is converted to dihydrotestosterone (DHT) in the
target tissues of the wolffian duct. DHT is required for the
wolffian duct system to subsequently develop into the epi-
didymis, vas deferens, ejaculatory duct, and seminal vesicles.
In the absence of a Y-chromosome no TDF is generated,
and the gonad passively differentiates into an ovary between
11 and 17 weeks of gestation. The absence of MIS leads to
persistence of the müllerian (paramesonephric) structures
which ultimately develop into the fallopian tubes, uterus,
cervix, and upper vagina while the wolffian (mesonephric)
structures involute (Fig. 16.6).
688 E. L. Riedesel and H. J. Paltiel

The müllerian ducts begin to fuse around the tenth week of
gestation. They fuse sequentially from caudal to cranial, from
the cervix to the uterine fundus. A median septum is formed
by fusion of the medial walls of the ducts that eventually invo-
lutes by 20 weeks of gestation, resulting in a single cervical
canal and single uterine cavity. The cranial ends of the paired
ducts remain unfused and become the fallopian tubes.
The cranial two thirds of the vagina develops from the mül-
lerian ducts, while the caudal third develops from the sinovag-
inal bulbs, a pair of endodermal outgrowths of the urogenital
sinus, which later fuse to form the lower part of the vagina.
The upper and lower portions of the vagina join together dur-
ing the process of recanalization of the fused müllerian ducts.
External Genitalia
Androgen production from the gonads also directs the devel-
opment of the external genitalia. In the presence of testos-
terone, the genital tubercle evolves into a phallus, and the
urogenital folds fuse to form the scrotum. In the absence of
testosterone, the genital tubercle evolves into a clitoris, and
the urogenital folds become the labia minora and majora.
Normal Anatomy
Ovary and Fallopian Tube
Each ovary is ovoid in shape with its long axis paralleling
the broad ligament. Ovarian size is determined by measuring
the ovary in three dimensions (longitudinal, transverse, and
anteroposterior diameters) on views obtained in two orthog-
onal planes [2]. Ovarian volume is calculated by the formula
for a simple ellipsoid (0.5 x length x width x height).
Mean ovarian volume changes with age. Ovarian size
remains relatively unchanged at 1–2 cm3 until the onset of
puberty. In post-menarchal girls, expected ovarian volumes
are more widely variable, typically between 7 and 9 cm3 in
volume (Table 16.1) [1, 5].
Pre-menarchal ovaries contain numerous small cystic
structures that represent primordial follicles. In neonates,
follicles may be large – greater than 9 mm in diameter –
owing to maternal hormone stimulation. In young children,
follicles are generally smaller, measuring between 6 and
7 mm, but may enlarge up to 9 mm due to pulsatile secretion
of low levels of follicle-stimulating hormone (FSH) before

Table 16.1 Mean pelvic ultrasound measurements per age year

Age (years) Uterine volume Left ovarian Right ovarian Endometrial stripe Longitudinal Anteroposterior Transverse uterine
(cm )
3
volume (cm )
3
volume (cm ) thickness (mm)
3
uterine axis (cm) uterine axis (cm) axis (cm)

n Mean SD n Mean SD n Mean SD n Mean SD n Mean SD n Mean SD n Mean SD

0 44 1.6 1.0 31 1.2 2.1 31 1.4 1.7 26 1.6 1.3 44 2.9 0.7 44 0.8 0.2 44 1.2 0.4
1 29 1.1 0.4 30 0.6 0.4 30 0.6 0.5 12 1.3 0.7 29 2.7 0.4 29 0.8 0.2 29 1.1 0.4
2 25 1.2 0.6 21 0.6 0.4 21 08 0.5 8 1.1 0.6 25 2.7 0.6 25 0.9 0.3 25 1.0 0.4
3 31 1.3 0.9 32 0.8 0.5 32 0.9 1.5 16 0.9 0.5 31 2.8 0.6 31 0.8 0.3 31 1.1 0.4
4 34 1.4 1.2 32 0.9 1.2 32 0.9 0.6 14 1.3 0.9 34 2.9 0.7 34 0.8 0.4 34 1.2 0.5
5 32 1.1 0.6 35 0.9 0.8 35 0.8 0.4 10 1.2 0.4 32 2.7 0.7 32 0.8 0.3 32 1.1 0.5
6 36 1.3 0.8 36 1.0 0.7 36 1.1 0.7 14 1.2 0.6 36 3.0 0.7 36 0.7 0.2 36 1.2 0.4
7 46 2.0 2.4 46 1.4 1.3 46 1.7 2.1 22 1.3 1.0 46 3.2 0.8 46 0.7 0.3 46 1.3 0.5
8 48 2.5 3.9 45 1.7 1.4 45 1.7 1.2 18 1.6 1.6 48 3.5 0.8 48 0.8 0.8 48 1.4 0.7
9 34 2.7 3.4 35 2.0 1.1 35 2.1 1.0 10 1.3 0.9 34 3.6 0.6 34 0.8 0.3 34 1.5 0.5
10 36 6.4 8.0 35 2.6 2.1 35 2.7 2.1 21 2.7 2.7 36 4.1 1.2 36 1.1 0.6 36 2.0 0.8
11 41 20.1 17.3 37 4.7 4.2 37 4.7 3.3 31 3.8 2.3 41 5.4 1.7 41 1.9 1.1 41 2.9 0.9
12 43 31.6 17.4 36 4.9 2.6 36 6.2 3.5 39 5.6 3.6 43 6.5 1.1 43 2.5 0.6 43 3.5 0.8
13 44 40.8 21.9 37 5.6 2.8 37 6.9 3.6 40 6.2 3.4 44 7.2 1.2 44 2.8 0.6 44 3.7 0.8
14 54 48.1 18.6 45 7.1 5.0 45 9.5 7.3 49 6.6 4.0 54 7.4 1.3 54 3.0 0.6 54 4.0 0.8
15 50 45.8 17.4 40 7.1 4.4 40 7.4 4.6 45 6.5 3.8 50 7.0 1.0 50 3.0 0.7 50 4.2 0.8
16 52 54.3 24.4 47 7.2 4.0 47 8.2 5.5 47 5.9 3.8 52 7.5 0.9 52 3.1 0.6 52 4.3 0.9
17 55 48.5 17.5 45 7.0 4.2 45 8.0 4.5 48 5.4 3.5 55 7.3 1.0 55 3.0 0.5 55 4.1 0.7
18 44 53.5 23.9 40 6.7 4.3 40 7.5 4.1 41 6.1 4.0 44 7.3 0.9 44 3.2 0.8 44 4.3 0.7
19 49 53.0 18.8 36 6.7 5.4 36 7.5 4.5 43 5.4 3.0 49 7.5 1.0 49 3.0 0.5 49 4.3 0.6
20 41 48.2 19.9 37 7.2 3.8 37 7.9 4.3 37 5.7 3.6 41 6.7 0.9 41 3.0 0.6 41 4.4 0.9
Total 868 25.5 27.0 778 4.0 4.1 778 4.5 4.7 591 4.5 3.7 868 5.2 2.2 868 2.8 1.5 868 25.5 27.0
n, Number; SD, standard deviation
From Gilligan LA, Trout AT, Schuster JG, Schwartz BI, Breech LL, Zhang B, et al. Normative values for pelvic organs throughout childhood and
adolescence. Pediatr Radiol. 2019;49(8):1042–50. [5]
16 Female Genital Tract
Fig. 16.7 Normal ovaries. (a) Longitudinal grayscale ultrasound image
of the left ovary in a 4-year-old female demonstrates multiple small,
anechoic follicles less than 9 mm in diameter. (b) Longitudinal grayscale
the onset of puberty (Fig. 16.7a). Following puberty, the ova-
ries increase in size and descend deeper into the pelvis.
Post-menarchal ovaries contain multiple stimulated and
unstimulated follicles (Fig. 16.7b). The appearance of the
normal ovary will vary slightly with changes in hormonal
stimulation throughout the menstrual cycle, discussed later
in this chapter.
The fallopian tubes run along the superior margin of the
broad ligaments [6]. They are not typically visualized on
routine transabdominal or transvaginal ultrasound. However,
they can be identified when thickened or fluid-filled as a
result of pelvic inflammatory disease or adnexal torsion.
The ovaries receive a dual blood supply from the ovar-
ian artery and uterine artery. The ovarian artery arises
directly from the abdominal aorta below the renal artery.
The uterine artery arises from the internal iliac artery and
supplies an adnexal branch that anastomoses with the uter-
ine artery [7].
Uterus and Cervix
The uterus develops from the müllerian structures in the
fetus under appropriate stimulation by the gonads. The nor-
mal size and shape of the uterus vary greatly throughout the
course of childhood (Table 16.1) [5, 8].
In late gestation the fetal uterus increases in size as a
result of maternal hormone stimulation that persists into
the newborn period. The normal neonatal uterus is broadly
tubular in shape on sagittal ultrasound images, with the uter-
ine fundus and cervix having an equal thickness in antero-
posterior diameter and a length of 2–4 cm. A thin echogenic
689
ultrasound image of the left ovary in a 17-year-old female demonstrates
multiple stimulated (arrowheads) and unstimulated follicles
endometrial stripe is visualized in almost all female neo-
nates (Fig. 16.8a).
After the newborn period, the uterus begins to decrease
in size but maintains a narrowed tubular shape with the fun-
dus and cervix of relatively equal thickness. From infancy
to late childhood, the uterus changes very little in size or
shape. The myometrium is homogeneous and hypoechoic
relative to surrounding soft tissues, and no echogenic endo-
metrial stripe is seen (Fig. 16.8b, c).
At the onset of puberty, the uterus descends deeper into the
pelvis with the other adnexal structures. The uterine fundus
thickens and elongates, resulting in a typical “pear-shaped”
appearance (Fig. 16.8d, e). The myometrium contains three dis-
tinct zones: a hypoechoic inner junctional zone, a moderately
echogenic intermediate zone, and a hyperechoic outer zone [8].
The cervix also elongates, developing a central echogenic
line that can be identified with high-frequency transducers.
The ultrasound appearance of the endometrium changes
with the phases of the menstrual cycle, discussed later in
this chapter.
Vagina
On ultrasound, the vagina is best demonstrated in a midline
sagittal plane via a transabdominal approach. However, visu-
alization may be limited on transabdominal ultrasound due
to deep positioning of the vagina within the pelvis. A trans-
perineal approach often better delineates the distal, superfi-
cial portions of the vagina (Fig. 16.4b).
The normal vagina appears as a midline tubular structure
immediately posterior to the urinary bladder and urethra. The
690 E. L. Riedesel and H. J. Paltiel

a b

c d

Fig. 16.8 Normal ultrasound appearance of the uterus by age. Sagittal grayscale ultrasound images of (a) 3-week-old female. (b) 11-month-old
female. (c) 4-year-old female. (d) 13-year-­old female. (e) 16-year-old female

prepubertal vagina is isoechoic relative to adjacent tissues the one follicle destined for ovulation continues to enlarge,
and may be difficult to discern. The postpubertal vagina is while the other follicles undergo atrophy. This dominant
characterized by a central echogenic lining and outer hyper- or graafian follicle produces a surge in estrogen that is
echoic wall. The vagina is typically collapsed; however, it followed by a surge in LH and prostaglandin production.
can be fluid-filled in the setting of vaginal reflux of urine. Ovulation occurs within 12 to 24 hours of the LH peak.
At the time of ovulation, the graafian follicle can mea-
sure up to 20 mm in diameter (Fig. 16.9). With ovulation –
Ovarian and Uterine Changes Associated typically at day 14 of a 28-day cycle – the graafian follicle
with the Menstrual Cycle ruptures and releases the ovum into the fallopian tube. As
the follicle collapses, its walls contract and it accumulates
Under the influence of the pituitary gonadotropins, the ovary blood, resulting in a “corpus hemorrhagicum” that can be
passes through three phases in each menstrual cycle: the fol- identified in the first 1–2 days following ovulation.
licular phase, ovulation, and the luteal phase. The luteal phase begins after ovulation. The granulosa
The follicular phase begins on day 1 of the menstrual cells surrounding the corpus hemorrhagicum enlarge, accu-
cycle and continues until ovulation. In response to follicle-­ mulating lipid and lutein, and the corpus hemorrhagicum
stimulating hormone (FSH) and luteinizing hormone (LH), becomes increasingly vascularized. These changes result
primordial follicles develop into primary ovarian follicles in the formation of the corpus luteum that secretes proges-
that produce estrogen. After day 9 of the menstrual cycle, terone and smaller amounts of estrogen. The corpus luteum
16 Female Genital Tract 691

persists for 14 days, after which it degenerates quickly in the
absence of a fertilized ovum. As the corpus luteum atrophies,
it is replaced by scar tissue, resulting in a corpus albicans.
The endometrium of the uterus also undergoes significant
changes during the menstrual cycle. During the menstrual
phase (days 1–4) and the proliferative phase (days 5–13),
the endometrium is mildly echogenic and undergoes gradual
thickening (Table 16.2). At the time of ovulation (day 14),
the endometrium has developed a striated appearance.
In the secretory phase (days 16–28), the striated endo-
metrial appearance is replaced by progressively more
homogeneous echogenicity, and the endometrium reaches
a maximal thickness due to distension of the endometrial
glands by mucin and glycogen that persists until menses
(Fig. 16.10). The appearance and thickness of the endome-
trium should be considered abnormal if they do not align
with the expected changes associated with each phase of
the menstrual cycle [8].

a b

Fig. 16.9 Graafian (“dominant”) follicle in a 21-year-old female. Transverse (a) and longitudinal (b) grayscale ultrasound images of the left ovary
on day 10 of the menstrual cycle demonstrate an anechoic follicle (cursors) measuring up to 1.7 cm

Table 16.2 Mean uterine ultrasound measurements in females > 12 years by week of menstrual cycle
Week of menstrual cycle Uterine volume (cm3) Endometrial stripe thickness (mm)
Mean SD Mean SD
1 45.3 16.2 4.5 2.8
2 45.7 19.0 6.0 3.6
3 49.8 24.0 6.6 3.8
4 52.2 20.9 7.6 3.8
5+ 51.5 23.3 5.7 2.8
SD, Standard deviation
Modified from Gilligan LA, Trout AT, Schuster JG, Schwartz BI, Breech LL, Zhang B, et al. Normative values for pelvic organs throughout childhood
and adolescence. Pediatr Radiol. 2019;49(8):​1042–50. [5]

a b c d

Fig. 16.10 Ultrasound appearance of normal endometrial changes (cursors) during the menstrual cycle. Sagittal grayscale ultrasound images obtained
on (a) day 5, early proliferative phase; (b) day 12, late proliferative phase; (c) day 17, early secretory phase; (d) day 26, late secretory phase
692 E. L. Riedesel and H. J. Paltiel

Anatomic Variants Müllerian Duct Anomalies

Arcuate Uterus
An arcuate uterus is considered a variant of normal anatomy.
The arcuate configuration results from incomplete resorption
of the longitudinal septum between the two müllerian uterine
horns, leaving a small soft tissue indentation at the top of the
uterine fundus that extends less than 1 cm into the endome-
trial canal. An arcuate uterus has a normal, smooth, external
fundal contour and a single endometrial cavity (Fig. 16.11).
Individuals with an arcuate uterus have normal menses and
normal reproductive potential.
Anomalies occur when there is a failure of fusion of the paired
müllerian ducts or incomplete resorption of the vertical mid-
line septum. The incidence of müllerian duct anomalies is esti-
mated at approximately 0.1–10% of the general population [9,
10]. The American Society of Reproductive Medicine has clas-
sified seven types of müllerian anomalies based on embryology
(Fig. 16.11) [11]. Most of these anomalies are identified during
evaluation for primary amenorrhea.
The association of uterine and renal anomalies is well
established. When a gynecologic anomaly is identified, the
kidneys and urinary tract should be carefully assessed for
additional abnormalities and vice versa.

Congenital Anomalies Müllerian Agenesis

Given the complex and integrated sequence of events asso-
ciated with development of the reproductive tract, there are
many opportunities for abnormal development resulting in
structural anomalies.
Arrested development of the müllerian ducts results in com-
plete agenesis or hypoplasia of the vagina, uterus, or both
[12]. The stage of embryologic development at which arrest
occurs determine which structures are affected. Of note,
patients with müllerian agenesis will have normal ovaries

I Hypoplasia/agenesis II Unicornuate III Didelphus

(a) Vaginal (b) Cervical (a) (b) Non

Communicating Communicating
IV Bicornuate

(c) Fundal (d) Tubal (e) Combined

(c) No cavity (d) No horn

(a) Complete (b) Partial

V Septate VI Arcuate VII DES drug related

(a) Complete (b) Partial

Fig. 16.11 Classification of müllerian anomalies according to the American Society for Reproductive Medicine System. Buttram VC Jr,
Gibbons WE. Müllerian anomalies: a proposed classification. Fertil Steril. 1979;32(2):40–6. [11]
16 Female Genital Tract
with normal external female genitalia development and
undergo the normal changes of puberty.
Uterine Agenesis
Uterine agenesis is characterized by a complete absence
of the uterus. The fallopian tubes will also be absent.
Importantly, patients with isolated uterine agenesis will have
a normal distal vagina, and the ovaries are typically normal
in structure and function, though they may be in an atypical
location. Patients will present with primary amenorrhea. On
ultrasound, a thin strip of fatty tissue will be seen between
the bladder and rectum (Fig. 16.12). Uterine transplantation
is a recently introduced therapeutic option that is still in its
infancy [12].
Mayer-Rokitansky-Küster-Hauser Syndrome (MRKH)
Mayer-Rokitansky-Küster-Hauser syndrome (MRKH), also
known as vaginal atresia, is defined as congenital absence
of the upper two thirds of the vagina in association with
variable müllerian duct anomalies [1]. The absence of the
vagina is the characteristic that distinguishes MRKH from
the other müllerian anomalies and has significant anatomic,
a b
b
Fig. 16.12 Uterine agenesis. (a) Longitudinal grayscale ultrasound
image of the pelvis in a 7-year-old female. No uterus is identified.
Sagittal (b) and axial (c) proton-density-weighted, fat-suppressed mag-
netic resonance (MR) images confirm the absence of the uterus with a
693
physiologic, and psychologic implications for the patient.
Most patients will also have associated cervical agenesis.
MRKH has a reported incidence of 1 per 4000–5000
females and most often presents as primary amenorrhea [13].
The diagnosis may be suspected clinically after an abnor-
mal physical examination. Affected individuals may have
associated non-­gynecologic anomalies, most commonly uni-
lateral renal agenesis, abnormalities of the middle ear, and
abnormal development of the vertebral bodies (Klippel-Feil
syndrome).
Transabdominal ultrasound is the initial imaging study of
choice for the evaluation of the uterus and adnexal structures,
although MR imaging may be necessary for definitive diag-
nosis. Uterine anomalies vary from complete agenesis to a
single midline uterine remnant with or without an endome-
trial cavity (Fig. 16.13). As with uterine agenesis, the ovaries
are typically normal, and patients may not be diagnosed for
some time due to normal estrogen-driven development of the
breasts and external genitalia.
Treatment includes psychological counseling and sup-
port, as well as creation of a functional vagina. This can be
achieved through the use of dilators or surgery [14].
c
r
normal vagina (v) identified between the bladder (b) and rectum (r).
Note the presence of normal ovaries (arrowheads) in the right and left
superolateral aspects of the pelvis on the axial image
694
a b
d e
Fig. 16.13 Mayer-Rokitansky-Küster-Hauser syndrome (MRKH) in a
16-year-old female. Longitudinal grayscale ultrasound images of the pel-
vis demonstrate (a) a small, tubular soft tissue structure (arrow) p­ osterior
to the bladder without an endometrial stripe representing a uterine rem-
nant. Normal right (b) and left ovaries (c) are identified. Longitudinal
 isorders of Lateral Fusion
D Bicornuate Uterus
Incomplete lateral fusion of the paired müllerian ducts dur-
ing embryologic development can result in a spectrum of
abnormalities.
Septate Uterus
Septate uterus is the most common müllerian anomaly seen
in routine clinical practice. It results from failure of the
final step of embryologic development and resorption of the
median septum between the paired müllerian ducts.
There are two types of septate uterus: complete septate
uterus with a septum extending to the level of the internal
cervical os; and partial septate uterus with a septum partially
extending into the endometrial cavity without reaching the
cervix (Fig. 16.11). The remnant midline septum consists of
fibrous material that appears hypoechoic on ultrasound. The
external uterine fundal contour may be flat or mildly (less
than 1 cm) indented (Fig. 16.14).
E. L. Riedesel and H. J. Paltiel
c
o o
f g
grayscale ultrasound images reveal (d) a solitary right kidney and (e)
absence of the left kidney. Sagittal (f) and (g) axial T2-weighted, fat-
suppressed MR images confirm vaginal agenesis. Small, paired soft tis-
sue structures adjacent to the normal ovaries (o) and connected by a
fibrous band (arrowheads) are suggestive of rudimentary uterine buds
Partial fusion of the two müllerian ducts leads to the for-
mation of a bicornuate uterus with two uterine horns joined
together caudally by a central muscular septum.
Two types of bicornuate uterus have been described, defined
by involvement of the cervical canal (Fig. 16.11). In complete
bicornuate uterus (bicornuate bicollis), the central myometrium
extends to the level of the external cervical os resulting in two
separate cervical canals (Fig. 16.15). In partial bicornuate uterus
(bicornuate unicollis), the central myometrium extends only to
the internal cervical os resulting in a single cervical canal that
communicates with the two uterine horns.
The distinction between a septate and bicornuate uterus
is made by careful evaluation of the external uterine con-
tour. In a bicornuate uterus, the two horns are more widely
separated, typically by an intercornual distance greater than
4 cm, with a deep indentation (more than 1 cm) in the fundal
contour (Fig. 16.16).
16 Female Genital Tract 695

a b c

Fig. 16.14 Septate uterus in a 13-year-old female. (a) Transverse gray- ultrasound image of the cervix demonstrates extension of the midline
scale ultrasound image of the uterus demonstrates a hypoechoic muscu- septum (arrowhead) through the endocervical canal. (c) Oblique axial
lar septum (arrow) dividing the endometrial cavity. The external fundal T2-weighted, fat-suppressed MR image depicts the muscular septum
contour is also slightly indented (arrowheads). (b) Transverse grayscale ­(arrowheads) extending along the entire length of the uterus and cervix

a b

Fig. 16.15 Bicornuate bicollis uterus in a 15-year-old female. Transverse grayscale ultrasound images of the uterus (a) and cervix (b) show two
distinct uterine horns and cervices separated by myometrium

a b c
3
1 2 3
1 2 1 2
3

Fig. 16.16 Illustration of the anatomical differences between a bicor- (arrow) above the intercornual line. (c) In a septate uterus the apex of
nuate and a septate uterus. In a bicornuate uterus (a), the concavity of the external uterine contour (3) lies more than 5 mm (arrow) above the
the external uterine contour (3) lies below (arrow) a straight line con- intercornual line
necting the cornua (1 and 2); or (b) the concavity (3) lies less than 5 mm
696
If there is any uncertainty on ultrasound evaluation, MR
imaging should be considered, as differentiation between
these two entities is critical for patient counseling and future
family planning. Septate uterus can be effectively treated
surgically, leading to improved obstetric outcomes, while a
bicornuate uterus is not amenable to surgical intervention.
Uterus Didelphys
Complete failure of müllerian duct fusion results in uterus
didelphys, defined as two completely separate uterine horns,
each with its own endometrial cavity and cervix (Fig. 16.11).
Two cervices must be documented either by ultrasound or
clinical examination to confirm the diagnosis of uterus
didelphys.
The two widely splayed uterine cavities and separate
cervices can be identified by transabdominal ultrasound
(Fig. 16.17). 3D ultrasound is useful in identifying the fundal
cleft that serves to distinguish this condition from a complete
septate uterus. A vaginal septum is seen in up to 75% of cases
and may be complete or partial. If a vaginal septum results
in obstruction of one or both uterine horns, it can be identi-
fied with ultrasound. MR imaging or physical examination
may be needed for complete evaluation. Surgical treatment
of uterus didelphys with a Strassman metroplasty is usually
reserved for women with recurrent pregnancy loss [15].
Unicornuate Uterus
Failure of one müllerian duct to elongate while the other
develops normally results in a unicornuate uterus. Different
types of unicornuate uterus have been described by the
American Fertility Society (Fig. 16.11) [11]. An isolated
unicornuate uterus is the most common, with a reported fre-
quency of 35%. However, a rudimentary uterine horn may
develop from the abnormal müllerian duct.
Fig. 16.17 Uterus didelphys in a 14-year-old female. Transverse grayscale
ultrasound image shows two widely separated uterine cavities (arrows)
E. L. Riedesel and H. J. Paltiel
With the exception of MRKH, urinary tract anomalies are
seen more often with unicornuate uterus than with any other
müllerian anomaly. Renal agenesis ipsilateral to the abnormally
developed müllerian duct is the most common abnormality [9].
Ultrasound imaging of a unicornuate uterus may be chal-
lenging, especially via a transabdominal approach. A uni-
cornuate uterus is typically smaller than a normal uterus
and may be laterally displaced in the pelvis (Fig. 16.18).
Identification of a rudimentary horn may be particularly dif-
ficult. If possible, these patients should be imaged in the
latter half of the menstrual cycle in order to better visualize
the thickened endometrium [16].
Use of 3D ultrasound can increase diagnostic accuracy
[3]. However, many of these patients need further MR
imaging for complete evaluation of the pelvic structures. A
unicornuate uterus is characterized on MR imaging by its
typically banana-shaped external contour, reduced uterine
volume, and asymmetric configuration [15].
Surgical treatment is restricted to patients with a rudimen-
tary uterine horn that can be resected when there is associated
severe dysmenorrhea, chronic pain related to endometriosis,
or dyspareunia. Surgery is also recommended for patients
with a functional rudimentary horn that has an endometrial
cavity in order to prevent a potentially life-­threatening preg-
nancy within the horn [15].
 isorders of Vertical Fusion
D
The upper and lower portions of the vagina are fused vertically
during the process of recanalization of the müllerian duct. The
hymen, a squamous mucosal tissue structure of the urogenital
sinus, invaginates cranially from the perineum to meet the lon-
gitudinal vaginal canal of the müllerian duct. As the müllerian
duct recanalizes, both the transverse vaginal plate and hymenal
tissue resorb to create a tubular vaginal canal. Failure of the
recanalization process results in vaginal obstruction.
Imperforate Hymen
During normal development of the vaginal canal, only a
small remnant of circumferential redundant hymenal tis-
sue remains at the vaginal introitus. An imperforate hymen
occurs when the hymenal tissue fails to completely resorb.
An imperforate hymen may present in the neonatal period
as hydrocolpos or mucocolpos. However, patients are typi-
cally asymptomatic until the onset of menses when vagi-
nal obstruction results in hematometrocolpos. On physical
examination, a patient with an imperforate hymen usually
presents with a vaginal bulge of thin hymenal tissue with a
dark or bluish hue caused by the accumulation of menstrual
blood in the proximal vagina [17].
On transabdominal ultrasound the obstructed vagina
appears as a round or tubular mass in the pelvic midline
16 Female Genital Tract 697

a b

c d

Fig. 16.18 Left-sided unicornuate uterus and obstructed right hemi-­
uterus in a 13-year-old female. (a) Transverse grayscale ultrasound
image of the pelvis reveals right and left-sided uteri with hypoechoic
material (arrow) within the right endometrial cavity. (b) Longitudinal
grayscale ultrasound image of the left pelvis reveals a well-developed
uterus with a normal-appearing, echogenic endometrial lining (arrow-
head). (c) Longitudinal grayscale ultrasound image of the right pelvis
reveals an abnormal, truncated shape of the rudimentary right uterine
horn. (d) Coronal T2-weighted, fat-­suppressed MR image of the pelvis
depicts the banana-shaped left uterus and the small, obstructed right
hemi-uterus containing hypointense endometrial material (arrow).
Arrowheads, Right and left ovaries

between the posterior wall of the bladder and anterior filled and dilated. Clinical history, physical examination,
wall of the rectum (Fig. 16.19). Internal echoes are seen and ultrasound demonstration of hematometrocolpos with
from accumulated cervical mucus or hemorrhagic mate- otherwise normal pelvic organs are often sufficient for
rial from menses. The uterine cavity may also be fluid- diagnosis.
698 E. L. Riedesel and H. J. Paltiel

a b

V V

Fig. 16.19 Imperforate hymen with hematocolpos in an 11-year-old female. (a, b) Longitudinal grayscale ultrasound images of the pelvis dem-
onstrate the markedly dilated, fluid-filled vagina (V) compressing the bladder (asterisk). U, Uterus

Surgical intervention is necessary only in symptom-
atic prepubertal patients. Once the diagnosis of imperfo-
rate hymen is confirmed, surgical intervention is usually
deferred because the hymen may open spontaneously at
puberty once estrogenization has occurred. Treatment
includes incision (hymenotomy) or excision. Excision is
preferred if the hymen is thickened or if the patient is an
adolescent or older [17].
Transverse Vaginal Septum
Failure of canalization of the transverse vaginal plate results
in a transverse vaginal septum. A transverse septum is a
membrane of connective tissue with vascular and muscular
components that divides the vagina into two segments. The
septum can vary in location, occurring in the upper (46%),
middle (40%), or lower (14%) portions of the vagina [1]. The
presenting symptoms of a transverse vaginal septum are sim-
ilar to those of imperforate hymen with amenorrhea, cyclical
pain, and a pelvic mass. However, on examination the hymen
is normal, and there is a short, blind-ending vagina.
Ultrasound findings in patients with imperforate hymen
and transverse vaginal septum are similar. Differentiation of
imperforate hymen and imperforate transverse vaginal sep-
tum relies on identification of the level of vaginal obstruction
and evaluation of the thickness and composition of the band
of tissue obstructing the vagina (Fig. 16.20). Transperineal
ultrasound may be helpful in diagnosis (Fig. 16.21).
Preoperatively it is essential to determine the location
and thickness of the septum and to identify the presence or
absence of a cervix. This is usually accomplished by com-
bining the physical examination with findings at ultrasound
and MR imaging. Most septa are 1 to several mm in thick-
ness, but occasionally a partial vaginal agenesis coexists,
leading to a very thick obstruction, which changes the surgi-
cal approach. There are rare cases of cervical agenesis that
resemble a high vaginal septum but require different surgical
treatment [18].
Atresia of Cervix or Vagina
Cervical atresia is a rare müllerian anomaly consisting of
absence or aplasia of the cervix. It is characterized either
by the absence of any cervical tissue or by the presence of
markedly abnormal cervical tissue. It typically occurs in
association with absence of the upper vagina and is usually
diagnosed at menarche, when patients present with amenor-
rhea and cyclical lower abdominal pain. Delays in diagnosis
and treatment can lead to endometriosis.
Ultrasound is useful in documenting the absence of the cer-
vix and can identify blood products in the uterine cavity (i.e.,
hematometra). Pelvic endometriomas may be seen as uni- or
multilocular fluid collections containing low level echoes, inter-
nal septations, and mural nodules with no internal vascularity
on color Doppler evaluation. Laparoscopy is the reference stan-
dard for the diagnosis of endometriosis although MR imaging is
increasingly being used, especially to assess deep foci of disease.
There is no consensus regarding optimal treatment of
cervical atresia. Total hysterectomy is one option, while
uterovaginal anastomosis offers an alternative for patients
to maintain menstruation [19].
Isolated vaginal atresia occurs when the caudal portion
of the vagina normally contributed by the urogenital sinus
fails to form and is replaced with fibrous tissue. In contrast
to the vaginal atresia associated with MRKH, this anomaly
is extremely rare [20]. Patients present with primary amen-
16 Female Genital Tract
a b
U
Fig. 16.20 Transverse vaginal septum in a 16-year-old female. (a)
Longitudinal grayscale ultrasound image of the pelvis demonstrates a
dilated distal vagina with internal echoes suggestive of hemorrhagic
material. Hypoechoic tissue at the inferior aspect of the dilated upper
Fig. 16.21 Transperineal imaging in the setting of hematocolpos may
help differentiate between imperforate hymen and a transverse vaginal
septum. (a) Transperineal longitudinal grayscale ultrasound image of an
imperforate hymen in a 13-year-old female. Hematocolpos extends close
to the vaginal introitus as measured by the calipers. (b) Transperineal
longitudinal grayscale ultrasound image of a collapsed distal vagina in a
12-year-old female with a transverse vaginal septum. The proximal
699
V
vagina (arrow) represents the vaginal septum. (b) Sagittal T2-weighted,
fat-suppressed MR image confirms the presence of a transverse septum
(arrow), with low-­intensity material filling the endometrial cavity of the
uterus (U) and vagina (V)
vagina (V) is distended with blood products, while the distal vagina (cali-
pers) appears collapsed. (c) Transabdominal longitudinal grayscale ultra-
sound image identifies the transverse vaginal septum (arrow) distal to the
fluid-filled vagina (V). Images b and c from Paltiel HJ, Phelps A. US of
the pediatric female pelvis. Radiology. 2014;270(3):644–57. B, Bladder;
R, rectum; U, urethra
700
orrhea and severe cyclical pelvic pain. Ultrasound imaging
reveals hematometrocolpos. Although there is no consensus
regarding optimal surgical treatment, pull-through vagino-
plasty has been reported as an effective option [21].
OHVIRA Syndrome
Obstructed hemivagina and ipsilateral renal anomaly
(OHVIRA), also known as Herlyn-Werner-Wunderlich
syndrome, is a müllerian anomaly characterized by uterus
didelphys, unilateral obstructed hemivagina, and an ipsilateral
renal anomaly. Patients with this syndrome usually present after
a b
c d
Fig. 16.22 Obstructed hemivagina and ipsilateral renal anomaly
(OHVIRA) syndrome in an 18-year-old female. (a) Transverse grayscale
ultrasound image of the pelvis demonstrates uterus didelphys. (b)
Longitudinal grayscale ultrasound image of the low pelvis reveals hema-
E. L. Riedesel and H. J. Paltiel
menarche with recurrent severe dysmenorrhea, pelvic pain,
or occasionally with a vaginal mass due to an obstructed
hemivagina that can be confused with a transverse vaginal
septum.
Transabdominal ultrasound is the initial imaging study
of choice and can usually identify the uterus didelphys and
hematocolpos [22]. When OHVIRA is suspected, further
evaluation with MR imaging is usually performed for preop-
erative planning (Fig. 16.22).
The goal of surgical treatment in OHVIRA is to relieve
symptoms and guarantee successful reproductive outcomes.
tocolpos of the left upper vagina (V). Coronal T2-weighted, fat-sup-
pressed MR images confirm (c) the presence of fluid (arrowhead) in the
obstructed proximal left hemivagina and (d) ipsilateral left renal
agenesis
16 Female Genital Tract
Treatment of choice is resection of the vaginal septum in
order to achieve continuity of the vagina [23].
Disorders of Sex Development
Disorders of sex development (DSD) are defined as congeni-
tal conditions in which the development of the chromosomal,
gonadal, or anatomic sex is atypical. DSD may be caused by
chromosomal abnormality, gonadal dysgenesis, abnormali-
ties of hormone production, or abnormal end-organ hormone
response. DSD may be first suspected when a baby is born with
an ambiguous appearance of the external genitalia; however,
they can also occur in patients with normal external genitalia.
Nomenclature and classification of these complex con-
ditions has been proposed by the International Consensus
Conference on Intersex and the European Society for
Paediatric Endocrinology [24]. Previous terms (intersex, her-
maphrodite, pseudohermaphrodite) should be avoided.
Imaging plays an important role in the diagnosis of patients
with suspected DSD. Ultrasound is used to establish the pres-
ence or absence of gonads and müllerian derivatives. Evaluation
should include imaging of the pelvis to assess for the presence or
absence of the uterus and ovaries, inguinal and perineal regions
to document the presence or absence of testes, and renal and
adrenal regions to document any abnormalities of these organs
[25]. MR imaging can serve as an additional problem-solving
tool to search for the gonads and clarify the internal anatomy.
 ex Chromosome Disorders of Sex
S ment with growth hormone. Hormone replacement therapy
Development
Sex chromosome DSD occurs when there is an abnormal
number of sex chromosomes or localized genetic alterations
of the sex chromosomes.
a b
Fig. 16.23 Streak ovaries in a 17-year-old female with Turner syndrome (45,X). Longitudinal grayscale ultrasound images of the right (a) and
(b) left pelvis reveal tiny, dysplastic ovaries (cursors) that are significantly smaller than expected for age
701
45,X (Turner Syndrome)
Turner syndrome is the most common sex chromosome
DSD, caused by the absence of all or part of one of the X
chromosomes. About 40–50% of females with Turner syn-
drome have the 45,X karyotype, 15–25% have mosaicism
with 45,X/46,XX, 20% have an isochromosome, and ring
X chromosomes are present in a few patients [25]. Affected
individuals are born with a normal uterus, fallopian tubes, and
vagina. However, the absence of a second X-chromosome
leads to abnormal development of the ovaries.
The ovaries consist of streaks or ridges of connective tis-
sue in the mesosalpinx parallel to the fallopian tubes. There
is delayed development of the external genitalia and a delay
in the onset of puberty. Patients present with primary amen-
orrhea. Individuals with monosomy X are typically diag-
nosed in infancy or early childhood due to a classic physical
appearance (short stature, low set ears, short webbed neck)
and other congenital anomalies including Hashimoto thy-
roiditis, congenital heart disease, renal anomalies, diabetes,
and skeletal abnormalities [1].
Streak ovaries are difficult to image with ultrasound
due to their location and size and are typically less than
1 ml in volume (Fig. 16.23). If ultrasound fails to identify
ovarian tissue, MR imaging can be considered for further
evaluation. The uterus and vagina will have a prepubertal
appearance.
Treatment of patients with Turner syndrome depends on
age and involves hormone replacement therapy as well as
management of associated abnormalities. In childhood and
adolescence, management is focused on growth and treat-
is used to induce puberty, to maintain feminization in adult
life, and to reduce morbidity and mortality [25].
46,XX Disorders of Sex Development
Females with 46,XX DSD have a female genotype and two
ovaries. However, their external genitalia may show a vari-
able degree of virilization due to excess androgen hormone.
702
Congenital Adrenal Hyperplasia
Congenital adrenal hyperplasia (CAH) is a family of auto-
somal recessive disorders that is the most common cause of
ambiguous genitalia seen in clinical practice. More than 90%
of cases are caused by 21-hydroxylase deficiency, an enzyme
that plays a vital role in steroid biosynthesis. There are mild
and severe forms of the disorder, with the severe form ­usually
detected in infancy or early childhood and the milder form
presenting anywhere from infancy to adulthood. Equal num-
bers of females and males are affected.
In the fetus, the absence of 21-hydroxylase leads to
chronic stimulation of the adrenal glands by the pituitary
gland leading to adrenal cortical hyperplasia, excess testos-
terone production, and abnormal development of the external
genitalia and lower genitourinary tract [26].
Affected female infants will have masculinized exter-
nal genitalia and lower genitourinary tract. The clitoris is
enlarged, and there is partial or complete labial fusion. The
urethra and lower vagina often unite to form a common
channel, a urogenital sinus, and in this setting, there will be
no separate vaginal opening on the perineum [1]. The inter-
nal female pelvic organs will develop normally.
Ultrasound plays an important role in the diagnosis of
CAH. The adrenal glands are diffusely enlarged, often with a
“cerebriform” appearance (Fig. 16.24). The uterus and ovaries
appear normal. Reflux of urine into the vagina and uterus in
patients with a urogenital sinus can lead to hydrometrocolpos.
Recently, contrast-enhanced ultrasound (CEUS) has been pro-
posed as an alternative method to conventional genitography
and voiding cystourethrography for delineation of the anatom-
ical abnormalities associated with urogenital sinus [27].
Early and accurate identification of CAH is imperative
as patients are at risk for developing primary adrenal insuf-
ficiency. If untreated, this disorder can lead to hypoglyce-
mia, hyponatremia, hyperkalemia, cardiac arrhythmias, and
shock. Management requires a multidisciplinary approach
that includes input from specialists in genetics, endocrinol-
ogy, surgery, psychology, or psychiatry in order to provide a
a b
Fig. 16.24 Congenital adrenal hyperplasia (CAH) in a 7-day-old
female. Sagittal grayscale ultrasound images of the right (a) and left (b)
adrenal glands demonstrate enlargement with a typical “cerebriform”
E. L. Riedesel and H. J. Paltiel
detailed diagnosis as well as appropriate hormonal, surgical,
and mental health treatment, as necessary [28].
Cloacal Malformation
Cloacal malformations are a group of non-hereditary ano-
rectal malformations that result from interruption of the
typical development of the urinary, genital, and gastrointes-
tinal tracts. Failure of formation of the urorectal septum or
urovaginal septum results in incomplete separation of these
tracts. The result is a “cloaca,” a single channel that con-
nects the urinary bladder, uterus/vagina, and rectum to the
perineum.
The etiology of cloacal malformation is not completely
understood but is thought to result from a combination of
genetic abnormality and hormonal influences. There is fre-
quently an associated failure of fusion of the müllerian ducts
which leads to duplication of the uterus and proximal vagina.
The association of cloacal malformation and virilized exter-
nal genitalia in 46,XX females without evidence of congeni-
tal adrenal hyperplasia is well recognized [29].
These patients are typically identified in infancy.
Ultrasound in the early neonatal period plays an impor-
tant role in diagnosis with evaluation of the pelvic organs,
identification of the gonads and exclusion of adrenal hyper-
plasia. When a diagnosis is made, additional imaging with
­genitography and/or voiding cystourethrography as well as
contrast material-enhanced examination of the distal limb of
the colostomy is needed to detail anatomy.
Recently, the use of CEUS in the elucidation of the con-
nections between the urinary, genital, and gastrointestinal
tracts has been proposed as an alternative to conventional
fluoroscopic studies (see Chap. 17) [27]. Pelvic MR imaging
is performed at a later stage when a pull-through procedure
and closure of the colostomy are anticipated [30]. Treatment
of the genitourinary abnormalities as well as of the associ-
ated congenital anomalies is complex and often requires
many reconstructive operations.
c
appearance. (c) Sagittal grayscale ultrasound image of the pelvis shows
a normal neonatal uterus (arrow). B, Bladder
16 Female Genital Tract
Adnexal Masses
The most common clinical symptoms of an adnexal mass
are pelvic or abdominal pain. Occasionally a patient may
present with a palpable abnormality on physical examina-
tion. Pelvic ultrasound is the initial imaging study of choice
and is used to define the site of origin, document size, and
determine the cystic, solid, or complex internal architecture
of the mass.
Ovarian Masses
Functional Cyst
Functional ovarian cysts are the most commonly seen
benign ovarian mass in all females, both pediatric and adult.
Functional cysts occur when a dominant follicle fails to ovu-
late or when a corpus luteum does not regress after ovula-
tion. While functional cysts are most frequent in postpubertal
girls, they can also occur in neonates with follicular develop-
ment in response to maternal hormone stimulation [31].
On ultrasound, simple functional cysts have anechoic
internal contents, a thin wall, and posterior acoustic enhance-
ment (Figs. 16.25 and 16.26). Differentiation between a
functional cyst and a physiologic follicle is based on size.
Functional cysts usually range between 3 and 5 cm in diam-
eter, while physiologic follicles are less than 3 cm.
The “daughter cyst” sign, a peripherally based simple
cyst within a larger simple cyst, has also been described
as a specific indicator of a stimulated ovarian follicle lead-
ing to a functional ovarian cyst, particularly in infants and
young children (Fig. 16.27). Simple cysts typically resolve
Fig. 16.25 Simple ovarian cyst in a 16-year-old female. Sagittal (a) and transverse (b) grayscale ultrasound images demonstrate a simple anechoic
cyst (calipers) of the right ovary. (c) Longitudinal color Doppler ultrasound image reveals that the cyst is avascvular
703
spontaneously in 2–3 months or over the course of 2–3
menstrual cycles.
Consensus guidelines from the Society of Radiologists in
Ultrasound (SRU) suggest follow-up ultrasound in patients
with simple ovarian cysts measuring more than 5 cm in diam-
eter. Repeat ultrasound is recommended at 2–6 months and
again at 6–12 months to evaluate change in size and appear-
ance over time [32]. If a simple cyst resolves or decreases in
size at follow-up ultrasound and the patient remains asymp-
tomatic, further imaging is not indicated.
Surgery for functional simple cysts in the adolescent is
indicated when there are significant symptoms or the cyst
does not resolve on follow-up ultrasound. Debate about the
risk of adnexal torsion in larger cysts applies to the adoles-
cent patient as well as to adults, with some surgeons recom-
mending intervention for larger cysts [31]. Once a decision
is made to operate, surgical options include cyst aspiration,
fenestration, or cystectomy.
Ovarian cysts in the newborn may present with symptoms
of abdominal pain or urinary tract obstruction if they are
extremely large. Other possible presentations include acute
torsion or spontaneous intracyst hemorrhage, which can be
significant enough to lead to shock. All newborns with symp-
tomatic ovarian cysts require surgical intervention to treat
the underlying cyst [33].
Functional cysts may develop internal hemorrhage when
theca interna vessels rupture into the cyst cavity. Most
patients with hemorrhagic ovarian cysts present with acute
onset of intermittent lower abdominal or pelvic pain, thought
to result from sudden distension of the ovarian cyst by blood.
On ultrasound, hemorrhagic ovarian cysts display a spec-
trum of findings reflecting the age of the blood products.
704
a b
Fig. 16.26 Corpus luteum cyst in a 15-year-old female. Longitudinal
(a) and transverse (b) grayscale ultrasound images of the right ovary on
day 21 of the menstrual cycle show a cyst (calipers) with internal echoes
Fig. 16.27 “Daughter cyst” sign in a 22-month-old female. Sagittal
grayscale ultrasound image of the pelvis demonstrates an anechoic cyst
arising from the left ovary that contains a peripheral internal cyst
(arrowhead)
Acute blood products are usually hyperechoic, becoming
more heterogeneous and eventually anechoic as the blood
clots and then resorbs. Internal “lacy” or “cobweb” avas-
cular septations are a classic imaging finding (Fig. 16.28).
Rupture of a hemorrhagic ovarian cyst should be suspected
if echogenic fluid is seen in the pelvis or peritoneal cavity.
E. L. Riedesel and H. J. Paltiel
and thin internal septations. Note appropriate thickening of the endo-
metrium (arrowhead) in the adjacent uterus (u)
Fig. 16.28 Hemorrhagic ovarian cyst in a 16-year-old female. Transverse
grayscale ultrasound image demonstrates a cyst in the left ovary (LT OV)
with internal lacy septations. UT, Uterus
Similar to simple cysts, hemorrhagic ovarian cysts
should decrease in size and involute completely with time.
Observation with repeat ultrasound examination at 6-week
intervals is recommended [32]. Cysts that persist for several
menstrual cycles may need to be investigated further with
MR imaging to exclude cystic neoplasm.
16 Female Genital Tract 705

Endometrioma sound appearance. Serial ultrasound imaging may be consid-

Endometriomas, also known as “chocolate cysts,” are a
localized form of endometriosis that occurs within the ovary.
While the mean age of diagnosis is in the third decade,
endometriosis is not uncommon among adolescents. Typical
symptoms include pelvic pain and dysmenorrhea.
On ultrasound, endometriomas are characteristically
unilocular cysts with diffuse internal “ground glass”
echoes. However, atypical features are seen in up to 50%
of cases. These features include multilocular cyst, hyper-
echoic mural foci, and a mixed cystic-solid appearance
[34]. Endometriomas may be unilateral or bilateral. If
bilateral, the echogenicity of the internal contents may be
slightly different.
Endometriomas, hemorrhagic cysts, and cystic neoplasms
(discussed elsewhere in this chapter) can overlap in ultra-
ered; however, MR imaging has been shown to have greater
specificity for the diagnosis of endometriomas than other
noninvasive imaging techniques and is recommended for
further evaluation if the diagnosis is in question.
Although endometriomas are usually benign, there is a
reported malignant transformation rate of approximately 1%
in adulthood. If the lesions are not surgically excised, yearly
ultrasound follow-up is suggested to evaluate for changes in
imaging characteristics or size.
Germ Cell Tumors
Germ cell tumors (GCTs) are by far the most commonly seen
ovarian neoplasm in the pediatric population. Table 16.3
summarizes the most important clinical, pathologic, and
imaging features of ovarian tumors in children.

Table 16.3 Clinical, pathologic, and imaging features of ovarian tumors in children
Tumor type
Germ Cell Tumors
Mature cystic teratoma
Immature teratoma
Dysgerminoma
Yolk sac tumor
Non-gestational
choriocarcinoma
Epithelial tumors
Cystadenoma
Borderline (low malignant
potential)
Cystadenocarcinoma
Sex Chord Stromal Tumors
Fibroma – Thecoma
Juvenile granulosa cell tumor < 30 years Most common sex chord stromal
Sertoli-Leydig cell tumor
Other
Gonadoblastoma
Abbreviations: LDH, Lactate dehydrogenase; AFP, alpha fetoprotein; β-hCG, beta human chorionic gonadotropin
Age range Clinical features Imaging features
10 – Most common ovarian neoplasm in Unilocular cystic mass with echogenic mural nodule and
20 years pediatrics “tip of the iceberg” sign
10% bilateral
Torsion may occur in 1/3
10 – Often large Predominantly solid mass with internal fat and calcific foci
20 years May metastasize to liver, peritoneum Vascular solid components suspicious for malignancy
and lung
15 – Most common malignant ovarian Large, solid mass with internal fibrovascular septa
19 years neoplasm in pediatrics
Elevated serum LDH
10 – Elevated serum AFP Mixed cystic and solid mass
30 years Rapidly growing
Local and distant metastases common at diagnosis
Mean age Very rare Highly vascular solid tumor with internal cystic,
14 years Elevated serum β-hCG hemorrhagic, and necrotic regions
< 20 years Rare before puberty Unilocular or multilocular cystic masses
May be large, causing abdominal Serous – simple fluid
distension Mucinous – low-level internal echoes
< 20 years Rare before puberty Unilocular or multilocular cystic masses
Thick, irregular septations
Papillary projections
< 20 years Rare in children Mixed cystic and papillary/solid masses
Up to 20% bilateral
> 30 years Thecoma – estrogen production Homogeneous or heterogeneous solid mass
leads to precious puberty Fibroma – Meigs syndrome with associated ascites and
pleural effusion
Mixed cystic and solid mass
tumor in pediatrics Internal vascularity
Precocious puberty (75%) Uterine enlargement and thickening of endometrial stripe
from tumor estrogen production
< 30 years Virilization Predominantly solid mass with peripheral or intratumoral
DICER-1 mutation cysts
< 20 years XY gonadal dysgenesis Large, lobulated, predominantly solid mass
Internal fibrovascular septa
706
Ovarian GCTs develop from ova-producing cells and
are composed of tissue derived from more than one of the
three primitive embryonic layers: ectoderm, mesoderm, and
endoderm. Because they arise from primitive cells, they have
variable degrees of cellular differentiation, cellular maturity,
and variable neoplastic potential.
Up to 30% of ovarian GCTs in children and adoles-
cents are malignant. As defined by the World Health
Organization, the histologic subtypes of ovarian GCTs
include teratoma, dysgerminoma, yolk sac tumor, embry-
onal carcinoma, non-­ gestational choriocarcinoma, and
mixed GCT [35].
Germ cell tumor should be suspected when ultrasound
demonstrates a solid or mixed cystic and solid mass associ-
ated with the ovary. In general, ultrasound imaging cannot
reliably distinguish between the various GCT types although
it can usually identify which masses are benign or malignant
based on the proportion of soft tissue to cystic components.
Benign tumors are usually cystic, whereas malignant tumors
usually consist of greater than 50% soft tissue components
[36]. Cystic areas reflect hemorrhage or necrosis and their
walls may be thick and irregular.
Due to the heterogeneous nature of ovarian germ cell
tumors, treatment is determined by the histology of a partic-
ular tumor and may include one of the following approaches:
surgical resection followed by careful monitoring for disease
recurrence; initial surgical resection followed by platinum-­
based chemotherapy; or diagnostic tumor biopsy and preop-
erative platinum-based chemotherapy followed by definitive
tumor resection.
Teratoma
Ovarian teratomas can be divided into two main subtypes,
mature and immature, according to the degree of cellular dif-
ferentiation of their components.
a b
Fig. 16.29 Mature cystic teratoma in a 16-year-old female. (a) Lon­
gitudinal and (b) transverse grayscale ultrasound images of the pelvis
demonstrate a cystic adnexal mass inseparable from the left ovary that
contains a densely echogenic peripheral nodule (arrow) with posterior
E. L. Riedesel and H. J. Paltiel
Mature cystic teratoma (MCT), often called ovarian der-
moid cyst, is the most common and accounts for up to 50%
of all pediatric ovarian neoplasms [35]. MCTs are composed
of mature or well-differentiated tissues derived from more
than one germ cell layer.
The characteristic imaging appearance of MCT on ultra-
sound is a cystic adnexal mass inseparable from the ovary
with a densely echogenic peripheral nodule (Rokitansky
nodule or dermoid plug) that demonstrates posterior acous-
tic shadowing (Fig. 16.29). The solid echogenic components
may represent fat, sebaceous material, hair, and/or calcifica-
tion from teeth or bone (Fig. 16.30).
Shadowing may obscure deeper parts of the mass, a find-
ing called the “tip of the iceberg” sign. Other ultrasound
features of MCT include fat-fluid levels, floating echogenic
debris, or multiple thin echogenic bands attributable to hair
within the lesion (Fig. 16.31). No internal vascularity should
be present on Doppler evaluation. Careful attention should
be paid to the contralateral ovary as MCTs are bilateral in up
to 26% of cases [35].
Immature teratomas are composed of primitive embryo-
nal tissues admixed with mature tissues derived from the
three germ cell layers. These tumors tend to be larger and
more aggressive than MCT [35–37]. In contrast to MCT,
immature teratomas are predominantly solid on ultrasound
and often appear heterogeneous due to internal foci of fat.
They may contain numerous small cystic areas and punctate
calcifications may be present.
Optimal treatment of GCT in the pediatric population is
surgical excision due to a small risk of malignant transfor-
mation and the risk of associated ovarian torsion.
Gonadoblastoma
Gonadoblastoma is a rare ovarian tumor which typically
occurs in the gonads of patients with XY gonadal dysgen-
c
acoustic shadowing. (c) Axial T2-weighted, fat-suppressed MR image
shows the mass (arrowhead) with loss of signal in the peripheral nodule
in keeping with fat
16 Female Genital Tract
a b
C
Fig. 16.30 Mature cystic teratoma in a 12-year-old female. (a) Lon­
gitudinal grayscale ultrasound image of the pelvis reveals a predomi-
nantly cystic pelvic mass (C) with complex peripheral nodule (arrow).
B For patients with dysgenetic gonads, bilateral oopho-
Fig. 16.31 Mature cystic teratoma in a 17-year-old female. Longitudinal
grayscale ultrasound image of the left adnexa reveals a predominantly cys-
tic mass containing an echogenic, fat-containing mural nodule (arrow) and
a clump of hair (arrowheads) manifested as thin, radiating bands of tissue.
B, Bladder. (Image courtesy of Dr. Casey Sams, Warren Alpert Medical
School, Brown University, RI, USA)
esis (mixed gonadal dysgenesis or mosaic Turner syndrome)
[38]. Occurrence in phenotypically and chromosomally nor-
mal females is extremely rare.
Gonadoblastomas contain a mixture of germ cells and
sex cord-stromal derivatives. On ultrasound, these lesions
707
C
(b) Coronal contrast-enhanced computed tomography (CT) image
shows a mixed cystic and solid mass (C) containing focal calcification/
ossification (arrowhead)
are typically large, lobulated, and predominantly solid with
internal fibrovascular septa. Associated simple and complex
free fluid in the pelvis has also been reported [35, 38, 39].
Although gonadoblastoma is benign, it frequently coexists
with a malignant germ cell tumor, most often dysgerminoma.
rectomy and hysterectomy are usually recommended. In
patients with a 46,XX karyotype, the necessity of bilateral
gonadectomy is less clear. If the contralateral gonad is left
in place in an attempt to preserve fertility, surveillance ultra-
sound should be performed.
Dysgerminoma
Dysgerminoma originates from undifferentiated germ
cells in the ovary and is the histologic counterpart to tes-
ticular seminoma in boys. As with gonadoblastoma, these
tumors may arise in dysgenetic gonads. However, they
are just as frequently seen in genetically normal gonads.
While most cases of dysgerminoma occur in the second
and third decades of life, approximately 10% develop in
adolescence [35]. Serum lactate dehydrogenase may be
elevated in some patients with dysgerminoma.
On ultrasound, a dysgerminoma appears as a predomi-
nantly solid, multilobulated mass that may contain foci
of hemorrhage and necrosis and punctate calcification.
Doppler evaluation can demonstrate internal fibrovascular
septations [36, 37]. Dysgerminoma occurs bilaterally in up
to 15% of cases and occasionally spreads to the regional
lymph nodes (Fig. 16.32) [35]. For that reason, the contra-
lateral ovary and adjacent pelvic and retroperitoneal nodal
708
a b
Fig. 16.32 Bilateral dysgerminomas in a 12-year-old 46,XY pheno-
typic female. (a) Sagittal grayscale ultrasound image of the mid-pelvis
shows a juvenile uterus (arrow). Sagittal grayscale ultrasound images of
the left (b) and right (c) pelvis reveal a dysplastic appearance of both
a b
Fig. 16.33 Yolk sac tumor in an 18-year-old female. (a) Sagittal color
Doppler ultrasound image of the pelvis depicts a predominantly solid
mass containing small cystic spaces and a minimal amount of blood
chains should be closely evaluated at diagnosis and on fol-
low-up imaging.
Yolk Sac Tumor
Yolk sac tumor, also called endodermal sinus tumor, is a rare,
aggressive, malignant GCT that often grows rapidly. Local
and distant nodal metastases as well as peritoneal metastases
are commonly seen at the time of diagnosis. This tumor pro-
duces alpha fetoprotein (AFP), which can be used to support
the diagnosis and as a tumor marker for follow-up [36].
On ultrasound, yolk sac tumor is typically large at the
time of diagnosis and predominantly solid but may contain
internal cystic spaces due to necrosis and internal hemor-
rhage (Fig. 16.33). Ascites may be present.
E. L. Riedesel and H. J. Paltiel
c
ovaries (arrowheads) that contain central echogenic foci and no recog-
nizable follicles. Both ovaries were subsequently removed and each
contained tissue consistent with dysgerminoma
flow. There is a small amount of free pelvic fluid (asterisk). (b) Sagittal
contrast-enhanced CT image reveals low attenuation of the mass
(arrow) that may reflect necrosis. Asterisk, Free pelvic fluid
Close evaluation for local and distant metastases should
be performed. Most patients are treated with surgical resec-
tion and chemotherapy.
Choriocarcinoma
Choriocarcinoma most often occurs as a malignant transfor-
mation of a molar pregnancy (gestational choriocarcinoma).
Very rarely, non-gestational primary ovarian choriocar-
cinoma can arise as a germ cell tumor. A non-gestational
ovarian choriocarcinoma may be mixed with other germ cell
tumors or arises as a primary pure choriocarcinoma of the
ovary. Serum beta human chorionic gonadotropin (β-hCG)
levels can be used to assist in diagnosis and follow-­up after
treatment.
16 Female Genital Tract
On imaging, choriocarcinoma appears as a highly vas-
cular solid tumor with internal cystic, hemorrhagic, and
necrotic regions [36]. Of note, the presence of an adnexal
mass in association with elevated β-hCG levels in patients
of reproductive age may cause significant clinical confusion,
and careful evaluation should be performed to exclude an
ectopic pregnancy, a far more common condition than ovar-
ian choriocarcinoma [40].
Mixed Germ Cell Tumor
Ovarian mixed germ cell tumor is composed of more than
one germ cell element. The most commonly occurring are
dysgerminoma, teratoma, and yolk sac tumor, although other
elements such as choriocarcinoma and embryonal carcinoma
may be present [39]. A large number of histologically malig-
nant elements are associated with more aggressive clinical
behavior. The ultrasound appearance is variable and reflects
the number and types of different tumor components present
in the mass. Intralesional fat or calcification may be seen if
an immature teratoma element is present (Fig. 16.34) [36].
Definitive diagnosis is made at pathology.
a b
Fig. 16.34 Mixed germ cell tumor in an 8-year-old female (a). Sagittal
grayscale and (b) transverse color Doppler ultrasound images demon-
strate a large, mixed cystic and solid mass arising from the right adnexa.
The mass contains a small amount of internal blood flow. (c) Axial
Fig. 16.35 Mucinous cystadenoma in a 15-year-old female. (a)
Longitudinal grayscale ultrasound image of the pelvis reveals a large cys-
tic mass with peripheral papillary projections (arrowheads) and containing
internal echoes suggestive of mucin. (b) Sagittal T2-weighted, fat-sup-
709
Epithelial Tumors
Epithelial ovarian tumors develop from the cells that cover
the outer surface of the ovary and account for 15–20% of all
pediatric ovarian tumors [35]. These tumors are potentiated
by hormone production and their incidence increases with
patient age, with most developing after the onset of men-
arche. Epithelial ovarian tumors can be classified as benign,
borderline (of low malignant potential), or malignant depend-
ing on their histologic characteristics and clinical behavior.
Cystadenoma
Benign cystadenoma is the most common ovarian epithe-
lial tumor seen in children. There are two subtypes: (1)
serous, which contains clear watery fluid, and (2) mucinous,
which contains thick viscous mucin [37]. On ultrasound, a
­cystadenoma appears as a thin-walled unilocular or multilocu-
lar cystic mass. These tumors can be very large at the time of
diagnosis, measuring up to 20–30 cm and extending superiorly
into the abdomen. Low-level internal echoes within cystic fluid
should suggest mucinous content (Fig. 16.35). Conservative
surgery with cystectomy is the appropriate treatment.
c
contrast-enhanced CT image shows inhomogeneous tumor enhance-
ment. Pathology revealed a mixed germ cell tumor (60% teratoma, 40%
yolk sac tumor)
pressed MR image depicts the mass anterior to the uterus (arrowhead) and
extending superiorly into the abdomen. The mass contains multiple thin
septations. (c) Axial contrast-enhanced, T1-weighted, fat-suppressed MR
image shows no significant enhancement of the internal septations
710
Borderline Epithelial Tumor and Cystadenocarcinoma
Borderline epithelial tumor (also called a tumor of low
malignant potential) and malignant cystadenocarcinoma are
exceedingly rare in the pediatric population, accounting for
less than 1% of all ovarian tumors in children and adoles-
cents [41]. Both borderline and malignant epithelial ovar-
ian tumors are usually of low grade and stage in pediatric
patients and therefore have a better prognosis compared with
adults. An elevated serum cancer antigen (CA)-125 level
may suggest a borderline or malignant tumor type.
Findings on ultrasound that should raise concern for a bor-
derline tumor include a cyst greater than 10 cm in diameter
with thick and irregular walls, vascular septations, papillary
or solid components with flow on color Doppler imaging,
and associated ascites [41]. Malignant cystadenocarcinoma
is more likely to contain complex solid components which
can demonstrate internal hemorrhage and necrosis.
Up to 20% of more advanced tumors (borderline masses
and carcinomas) can be bilateral [36]. Additional MR imag-
ing is suggested in these patients to better evaluate for pelvic
organ invasion, implants (peritoneal, omental, mesenteric),
or evidence of nodal metastases.
Borderline ovarian tumors in children and adolescents are
treated conservatively, with fertility-preserving techniques
and surveillance associated with good outcomes. The role of
adjuvant therapy is not currently known [41].
Stromal Tumors
Sex chord stromal tumors (SCST) are a rare class of tumor
that develop from the stroma of the ovary and are composed
of a variable combination of granulosa cells, theca cells,
Leydig and Sertoli cells, and fibrous connective tissue cells.
SCSTs account for 10–20% of all pediatric ovarian tumors,
particularly in girls less than 15 years of age. While fibromas
are the most commonly seen tumor type in adult, juvenile
a b
Fig. 16.36 Juvenile granulosa cell tumor in a 15-year-old female. (a)
Transverse grayscale ultrasound image shows a solid right adnexal mass
containing numerous cystic spaces of varying size. (b) Transverse color
Doppler ultrasound image with spectral analysis shows vessels within the
E. L. Riedesel and H. J. Paltiel
granulosa cell tumor (JGCT) and Sertoli-Leydig cell tumors
are the most commonly seen in the pediatric age group [36].
As with other ovarian tumors, SCSTs present with the
typical symptoms of an adnexal mass, including abdominal
pain, distention, and rarely ovarian/adnexal torsion. SCSTs
also frequently manifest signs of hormonal production by
their cellular components [35].
Thecoma-Fibroma
These tumors are rare in children and adolescents and account
for less than 2% of all pediatric ovarian tumors. They usually
occur in patients more than 30 years of age [35].
Thecomas are composed of lipid-containing theca cells
and a variable number of fibroblasts, while fibromas are
characterized by fibrous components. On ultrasound these
tumors are typically solid masses which may be homoge-
neous or heterogeneous. Internal calcifications can be seen.
Associated ascites and pleural effusion (Meigs syndrome)
suggests ovarian fibroma, while estrogen effect with uterine
enlargement and thickening of the endometrial stripe should
suggest thecoma.
Juvenile Granulosa Cell Tumor
Juvenile granulosa cell tumor (JGCT) is the most common
SCST seen in the pediatric population. Up to 75% of these
tumors produce estrogen resulting in isosexual precocious
puberty.
These tumors have variable imaging characteristics. By
ultrasound, the most typical appearance is a large, unilat-
eral, multilocular cystic mass with an internal solid portion
and occasionally with irregular septa (Fig. 16.36). However,
JGCT can also appear as a predominantly solid mass with
variable cystic areas. Internal vascularity – peripheral, central,
or both – is typically seen on Doppler evaluation. Secreted
estrogen from these tumors may cause uterine enlargement
c
mass containing arterial and venous flow. (c) Coronal T2-weighted, fat-
suppressed MR image of the pelvis confirms the numerous cystic spaces
within the mass
16 Female Genital Tract
and endometrial thickening. Metastases are uncommon, but
when seen are typically to the peritoneal surfaces and liver.
JGCT has a favorable prognosis in patients with stage
I disease after surgical resection alone. Adjuvant chemo-
therapy may be indicated for patients with higher-stage
tumors [42].
Sertoli-Leydig Cell Tumor
Sertoli-Leydig cell tumor is a rare stromal lesion that
accounts for less than 0.5% of all malignant ovarian neo-
plasms in children [35]. Patients may present with viriliza-
tion and hirsutism due to excess androgen secretion by the
tumor [43].
These tumors are usually detected early due to their clini-
cal symptoms and are often confined to the ovary on initial
diagnostic imaging. Small tumors may be difficult to iden-
tify on transabdominal ultrasound, and additional MR imag-
ing should be considered if clinical suspicion is high. On
ultrasound the tumor ranges from cystic to solid. It typically
appears as a predominantly solid mass with peripheral or
intratumoral cysts.
DICER1 syndrome is associated with Sertoli-Leydig cell
tumors. Patients with this diagnosis should undergo surveil-
lance screening for other DICER1-related tumors [44]. This
is often accomplished with whole body MR imaging.
Secondary Tumors
Although rare, secondary ovarian tumors should be con-
sidered in the differential diagnosis of ovarian and adnexal
masses [35, 37, 45].
Similar to the testes, the ovaries may serve as a “sanctuary
site” for surviving leukemic cells in children after chemo-
therapy. Leukemic infiltrates of the ovary are often found at
a b
Fig. 16.37 Burkitt lymphoma of the ovary in a 7-year-old female. (a)
Transverse grayscale ultrasound image of the pelvis reveals a solid,
hypoechoic mass in the right adnexal region posterior to the uterus (U).
A normal right ovary was not identified. (b) Coronal contrast-enhanced
711
autopsy; however, they are rarely clinically detected during
the course of disease.
Ovarian infiltration at presentation of pediatric non-­
Hodgkin lymphoma (NHL) accounts for less than 2% of
all pediatric ovarian tumors [45]. It occurs most often with
Burkitt lymphoma and is considered a local manifestation of
systemic disease rather than metastatic disease (Fig. 16.37).
Metastatic spread to the ovaries occurs through four
pathways: hematogenous spread, lymphatic spread, surface
implantation from free intraperitoneal tumor cells, or direct
spread from adjacent neoplasm. In one study, the most com-
monly seen hematogenous metastases to the ovary were
from mucinous adenocarcinoma of the colon, rhabdomyo-
sarcoma, Wilms’ tumor, neuroblastoma, and retinoblastoma.
Surface implantation was also reported in colon cancer [45].
Findings suggestive of ovarian metastasis rather than
a primary ovarian tumor are concurrent presence of meta-
static foci in other organs and bilateral ovarian involvement.
Treatment of ovarian metastases depends on the primary
tumor type.
Paraovarian Cyst
A paraovarian cyst can be of mesothelial, mesonephric, or
paramesonephric origin and is found along the broad liga-
ment adjacent to the ovary. The classic ultrasound appear-
ance is of a round or oval, anechoic, thin-walled cyst in the
adnexa separate from the ovary (Fig. 16.38). Cyst fluid may
be anechoic or echogenic. Unlike a functional ovarian cyst,
a paraovarian cyst is not hormonally sensitive and there-
fore does not show cyclical changes and does not regress
on follow-­up ultrasound examination. A paraovarian cyst
CT image demonstrates bulky nodal disease in the pelvis (arrowhead)
and enlargement of the right ovary (arrow) due to lymphomatous
involvement
712
can be identified during the ultrasound examination by
using gentle compression to separate the ovary from the
paraovarian cyst.
Paraovarian cysts can sometimes be complicated by hem-
orrhage, torsion, or rupture. Large or symptomatic cysts are
B Peritoneal inclusion cysts may manifest as unilocular or
C pelvis (Fig. 16.39). Adhesions may form irregular and thick
Fig. 16.38 Paraovarian cyst in a 16-year-old female. Longitudinal
grayscale ultrasound image of the left adnexa demonstrates a simple
cyst (C) separate from the left ovary (arrow). B, Bladder
a b
U
Fig. 16.39 Peritoneal inclusion cyst in a 12-year-old female with a
history of many prior abdominal operations. (a) Longitudinal grayscale
ultrasound image of the pelvis demonstrates a fluid collection (asterisk)
E. L. Riedesel and H. J. Paltiel
often resected, and a laparoscopic approach can be used [46].
Smaller asymptomatic cysts are treated conservatively.
Peritoneal Inclusion Cyst
A peritoneal inclusion cyst develops when peritoneal fluid
absorption is significantly impaired in the setting of perito-
neal inflammation and extensive peritoneal adhesions. Non-­
absorbed peritoneal fluid becomes trapped, creating a cyst-like
fluid pocket [47]. Patients with a peritoneal inclusion cyst typ-
ically present with acute or chronic pelvic pain in the setting of
prior surgery, trauma, or pelvic inflammatory disease.
multilocular fluid collections in the adnexa. Unlike ovarian
or paraovarian cysts, the fluid conforms to the contours of the
septations within the fluid collection. The ipsilateral ovary
may become “entrapped” within adhesive bands and fluid
and is sometimes described as having the appearance of a
spider sitting inside a spider web.
Conservative treatment is typically advocated for perito-
neal inclusion cysts. Oral contraceptive use may suppress
ovulation to decrease ovarian fluid production, the predom-
inant source of pelvic free fluid. Surgical intervention is
reserved for select cases as it may exacerbate the underly-
ing problem.
B
superior to the bladder (B). U, Uterus. (b) Coronal T1-weighted MR
image shows the fluid collection (asterisk) indenting the dome of the
bladder (B)
16 Female Genital Tract
Adnexal Torsion
Adnexal torsion is the fifth most common gynecologic emer-
gency in the United States, with approximately 30% of cases
occurring in females less than 20 years of age [48].
Adnexal torsion in young children and adolescent females
most often occurs spontaneously as a result of increased
mobility of the adnexal structures from ligamentous laxity
and a relatively increased potential space within the pelvis
that permits twisting [49]. When torsion is associated with
an adnexal mass, the most common are benign functional
ovarian cyst and benign ovarian teratoma.
Torsion of the adnexal structures occurs around the vascu-
lar pedicle of the broad ligament. Twisting results in obstruc-
tion of the venous and lymphatic drainage of the affected
ovary as well as arterial inflow, resulting in ovarian edema
followed by infarction and necrosis.
Transabdominal ultrasound is the imaging study of choice
in pediatric patients with suspected adnexal torsion. The most
reliable finding is unilateral ovarian enlargement. In this
population, comparison of ovarian volume with established
normal values at various ages and stages of development is
extremely important (Table 16.1) [50].
In pre-menarchal girls, ovarian enlargement to 6–8 cm3
should raise concern for torsion. In post-menarchal girls,
ovarian volume greater than 45–60 cm3 should increase sus-
picion for torsion [50, 51]. Size comparison with the contra-
lateral ovary is also helpful. In the setting of adnexal torsion,
affected ovaries are typically 3 to 4 times the volume of the
normal, contralateral ovary. An ovarian volume <20 ml in a
post-menarchal pediatric patient has been reported as reliably
excluding adnexal torsion [51].
Increased echogenicity of the ovarian stroma, peripherally
displaced ovarian follicles, and free fluid in the pelvis may
also be seen (Fig. 16.40). Identification of a coiled vascular
b c
Fig. 16.40 Right adnexal torsion in a 12-year-old female. (a) Transverse
grayscale ultrasound image of the right ovary demonstrates enlargement
with a volume of 96 cm3 (about 9× the volume of the left ovary). There is
markedly increased echogenicity of the ovarian stroma with a central
anechoic zone. The follicles are peripherally displaced. (b) Transverse
713
pedicle (referred to as the “whirlpool” sign) is highly specific
for adnexal torsion but not commonly visualized [50].
Arterial flow may be preserved in the torsed ovary as a
result of additional arterial supply from the uterine arter-
ies or because of intermittent torsion. As a result, Doppler
­evaluation is not reliable for confirming or excluding adnexal
torsion. In fact, one study reported normal Doppler arterial
blood flow in as many as 60% of surgically confirmed cases
of adnexal torsion [52].
MR imaging can be considered for further evaluation;
however, the diagnostic reference standard remains direct
visualization of a torsed adnexa on diagnostic laparoscopy
[53, 54]. To reduce the number of negative laparoscopies
performed, a scoring system has been proposed to better
identify torsion before surgery. Multiple indices of clinical
and radiologic predictors of adnexal torsion are combined
into a composite score that more accurately predicts torsion
and need for surgical intervention [55].
In the past, oophorectomy of the affected ovary was per-
formed if an associated adnexal cyst or mass was identified
at surgery for fear of leaving behind a potential malignancy.
However, given the importance of ovarian preservation for
young patients and the extremely low incidence of malig-
nant lesions associated with torsion in this population, many
surgeons have more recently adopted a more conservative
approach that includes intraoperative ovarian detorsion with
follow-up postoperative imaging at 6–12 weeks. Ovarian
oophoropexy is typically reserved for patients with recurrent
torsion [48].
I solated Tubal Torsion
Isolated tubal torsion is a rare cause of acute lower quad-
rant pain mimicking adnexal torsion that is most often seen
in post-menarchal adolescent girls. Isolated tubal torsion
is often associated with a pathologic “lead point” such as
color Doppler ultrasound image of the right ovary shows no vascular flow.
(c) Transverse color Doppler ultrasound image with spectral analysis
reveals normal flow in the left ovary, with low-resistance arterial
waveforms
714
an ovarian or paraovarian mass or with a history of pelvic
inflammatory disease [6, 56]. Preoperative diagnosis is often
difficult due to a lack of specific signs [57].
Ultrasound typically demonstrates a unilateral dilated
fallopian tube with thickened, echogenic walls and inter-
nal debris (Fig. 16.41). A “beak” sign may be identified at
the site of torsion, similar to that seen in closed-loop bowel
obstruction or volvulus. Importantly, the ipsilateral ovary
should appear normal in size and morphology with normal
Doppler evaluation.
Tubal detorsion should be attempted in all cases, and a
laparoscopic approach has been advocated as it reduces the
risk of abdominal adhesions compared to open surgery, an
a b
c d
Fig. 16.41 Isolated tubal torsion in a 10-year-old female. (a)
Transverse and (b) sagittal grayscale ultrasound images of the right
adnexa reveal a dilated fallopian tube with thickened, echogenic walls,
and layering internal debris. The right ovary (arrow) appears normal.
E. L. Riedesel and H. J. Paltiel
important consideration in girls of reproductive age [57].
Resection can be considered if ischemic damage appears to
be irreversible.
 assive Edema of the Ovary
M
Massive edema of the ovary can occur in the setting of
recurrent partial or intermittent adnexal torsion where there
is preservation of arterial inflow but obstruction of venous
and lymphatic drainage. Most cases are unilateral, and the
affected ovary remains viable without infarction, unlike
ovarian torsion where the arterial supply is eventually com-
promised [58]. Patients typically present with chronic inter-
mittent low abdominal and pelvic pain.
(c) Sagittal color Doppler ultrasound image reveals no significant flow
within the wall of the dilated tube. (d) Intraoperative photograph docu-
ments isolated tubal torsion (arrow) around an adhesive band related to
prior appendectomy
16 Female Genital Tract
Ultrasound findings correspond to the degree of associ-
ated adnexal torsion and ovarian edema and significantly
overlap with the features of “complete” adnexal torsion.
The affected ovary is typically enlarged with increased
echogenicity of the ovarian stroma due to edema. Often,
the ovary appears solid with few physiologic follicles seen.
Arterial blood flow is identified within the affected ovary
although it is often decreased.
Clinical differentiation between massive edema and
torsion is difficult and often requires laparoscopy. Most
reported cases of massive ovarian edema have been treated
with salpingo-oophorectomy because of preoperative and
intraoperative concern for a malignant ovarian tumor. Wedge
biopsy with frozen section during surgery has been urged to
avoid unnecessary removal of the ovary [58].
Ectopic Pregnancy
Ectopic pregnancy, an embryo implanted outside a normal
intrauterine location, is reported in up to 2% of all pregnan-
cies in the United States, and is the leading cause of first
trimester bleeding [59, 60]. Ninety-five percent of ectopic
pregnancies occur in a fallopian tube. Other ectopic loca-
tions include the ovary, uterine interstitium, cervix, surgical
scar, and the abdominal or pelvic cavities.
Early and accurate diagnosis of an ectopic pregnancy is
essential for appropriate treatment, which may include local
Fig. 16.42 Ectopic pregnancy in a 17-year-old female with sudden
severe hypotension. (a) Sagittal grayscale ultrasound image shows a
normal uterus without evidence of an intrauterine pregnancy. There is a
large amount of hypoechoic fluid posterior to the uterus (U) consistent
715
or systemic medications or surgery. Most patients with ecto-
pic pregnancy present with a 5- to 9-week history of amenor-
rhea, pelvic pain, and vaginal spotting; however, up to 50%
of patients may be asymptomatic [60].
Initial evaluation of patients with suspected ectopic preg-
nancy includes a quantitative measurement of serum β-hCG.
β-hCG levels increase progressively early in pregnancy until
they reach a plateau at approximately 9–11 weeks [61].
Quantitative β-hCG level and knowledge of the expected
gestational age of pregnancy can be useful in guiding ultra-
sound evaluation.
Transabdominal ultrasound can be used to identify
an intrauterine pregnancy (IUP) of 8 weeks’ gestation or
more, or when the serum β-hCG is greater than 6500 IU/l.
Transvaginal ultrasound is the preferred method for imag-
ing evaluation as it can be used much earlier in pregnancy,
reliably visualizing an IUP by 5 weeks of gestation and with
serum β-hCG levels as low as 2000 IU/l.
The absence of an intrauterine gestational sac in a
patient with appropriate β-hCG levels should trigger a
detailed search for an ectopic pregnancy. An adnexal mass
separate from the ovary is the most common finding of a
tubal pregnancy and is seen on ultrasound images in up to
89–100% of patients [61]. The mass may be surrounded
by a hyperechoic, intensely vascular ring, the so-called
“ring of fire” sign. Findings are more specific for an ecto-
pic pregnancy if a yolk sac or a living embryo is identified
(Fig. 16.42).
with hemorrhage. The more echogenic material abutting the uterine
edge (arrow) represents blood clot. (b) Transverse color Doppler ultra-
sound image of the right lower quadrant reveals a rounded mass with a
peripheral “ring of fire,” a classic sign of an ectopic pregnancy
716
a b
Fig. 16.43 Endometritis and cervicitis in an 18-year-old female with
culture-positive Chlamydia trachomatis. Transabdominal longitudinal
grayscale (a) and color Doppler (b) ultrasound images of the uterus
Intrauterine findings of an ectopic pregnancy include a
“normal endometrium,” a pseudo-gestational sac, a trilaminar
endometrium, and a thin-walled decidual cyst. Extrauterine
findings include pelvic free fluid, hematosalpinx, and hemo-
peritoneum. The presence of echogenic fluid in Morison’s
pouch and the pelvic cul-de-sac should raise concern for a
ruptured ectopic pregnancy. If neither an intrauterine nor an
extrauterine pregnancy is identified on transvaginal imaging,
the patient should be classified as having a “pregnancy of
unknown location” with clinical and imaging follow-up until
the final pregnancy outcome is known [60, 61].
Pelvic Inflammatory Disease
Pelvic inflammatory disease (PID) usually occurs as a result
of ascending infection from the vagina and cervix through
the endometrial cavity. From the endometrial cavity, infec-
tion can spread to the fallopian tubes and into the pelvis. The
most common organisms associated with PID are Chlamydia
trachomatis and Neisseria gonorrhoeae. Typically, patients
present with fever, pelvic pain, and purulent vaginal dis-
charge; however, PID may have an indolent course and be
clinically unrecognized [62].
E. L. Riedesel and H. J. Paltiel
demonstrate thickening and increased vascularity of the myometrium
and cervix
PID can present with a spectrum of ultrasound imag-
ing findings [62]. Early on, there may be endometritis with
enlargement of the uterus, increased color Doppler flow in
the uterine serosa and myometrium, and associated inflam-
matory changes in the pelvis (Fig. 16.43). More advanced
cases may demonstrate infection of the fallopian tubes (pyo-
salpingitis) with enlarged, fluid-filled fallopian tubes. The
tube walls may be thickened and hyperemic with increased
flow on color Doppler. Fluid within the inflamed fallopian
tube is typically echogenic and may have internal septations.
The most advanced cases of PID will present with a tubo-­
ovarian abscess (TOA). On ultrasound, a TOA appears as a
heterogeneous, complex cystic structure in the adnexa that
completely replaces the normal ovarian tissue (Fig. 16.44).
Given the complexity and varied appearance of TOA, in the
absence of classic clinical symptoms it may be confused
with other adnexal masses. Further evaluation with CT or
MR imaging can be used to confirm the diagnosis and evalu-
ate the extent of inflammation in the pelvis.
Treatment for PID depends on the extent of infection.
Early on, oral or intramuscular antibiotic therapy is often
adequate. However, more advanced cases may require intra-
venous antibiotic therapy, percutaneous abscess drainage,
and surgery [63].
16 Female Genital Tract 717

a b

Fig. 16.44 Tubo-ovarian abscess in a 19-year-old female. (a) Longitudinal grayscale ultrasound image of the right adnexa shows a thick-­walled
cystic structure containing hypoechoic debris. (b) Longitudinal color Doppler ultrasound image reveals hyperemia of the cyst wall

Uterine Masses toms related to compression by the tumor, such as urinary

Uterine masses are seldom seen in young children or ado-
lescents. With the onset of puberty and menarche, benign
lesions such as leiomyoma and adenomyosis may develop.
Endometrial hyperplasia and endometrial polyps noted in
older women are not common in the pediatric population.
Primary uterine malignancy is exceedingly rare.
Benign Masses
Leiomyoma (Fibroid)
Uterine leiomyomas (uterine fibroids) are benign tumors of
smooth muscle and connective tissue originating from the
uterine myometrium. These benign tumors are hormonally
dependent, responding to both estrogen and progesterone,
and can be seen in adolescents and young women follow-
ing the onset of puberty. Early age at menarche and obesity
are risk factors for the development of fibroids, likely due to
the increased exposure to estrogen. The majority of young
patients with fibroids are asymptomatic; however, 20–50%
will have complaints of menorrhagia, pelvic pain, and symp-
frequency or urgency [64].
Uterine fibroids are classified according to their location as
submucosal, intramural, or subserosal [64, 65]. Submucosal
fibroids are the least common but are the most likely to present
with abnormal bleeding because they project into and expand
the endometrial cavity. Subserosal fibroids may extend exophyti-
cally into the pelvic and abdominal cavities and be confused with
ovarian masses. Pedunculated subserosal fibroids can undergo
torsion, resulting in the acute onset of severe pain. Large fibroids
can also outgrow their blood supply, leading to infarction.
Ideally, both transabdominal and transvaginal ultrasound
examinations should be performed to evaluate uterine fibroids.
Transabdominal imaging provides a superior evaluation of the
overall uterine contour, while transvaginal imaging is more
sensitive for the diagnosis of small fibroids [64]. 3D ultra-
sound with coronal reconstructions can also be helpful [2].
On ultrasound, fibroids typically appear as well-
defined, solid masses with a whorled appearance. They
are usually isoechoic to the myometrium but can be
hypoechoic. Lesions may demonstrate edge shadowing.
Color Doppler evaluation may demonstrate peripheral ves-
sels or large feeding vessels arising from the serosal sur-
718
a b
Fig. 16.45 Uterine leiomyoma (fibroid) in a 14-year-old female. (a)
Sagittal (left panel) and transverse (right panel) grayscale ultrasound
images of the uterus reveal an isoechoic oval mass (calipers) within the
myometrium at the uterine fundus that demonstrates marked edge shad-
face of the uterus (Fig. 16.45). Distortion of the uterine
contour suggests a subserosal or large intramural lesion.
A bulky appearance of the uterus suggests intramural or
submucosal lesions.
Further evaluation with MR imaging should be consid-
ered, as it is widely considered the most accurate imaging
technique for identification and classification of uterine
lesions and can be used to determine treatment options in
patients with significant symptoms.
Laparoscopic myomectomy is the standard treatment for
symptomatic uterine fibroids in females of reproductive age.
Nonsurgical treatments include uterine fibroid embolization
and MRI-guided high-intensity focused ultrasound (HIFU)
[66]. Oral contraceptives, hormone-releasing intrauterine
devices, and nonsteroidal anti-inflammatory drugs can be
used to control menorrhagia and pain.
E. L. Riedesel and H. J. Paltiel
owing consistent with an intramural leiomyoma. (b) Transverse power
Doppler ultrasound image demonstrates peripheral feeding serosal ves-
sels. (c) Sagittal contrast-enhanced CT image shows the intramural
leiomyoma (arrow)
Adenomyosis
Adenomyosis is defined as the presence of ectopic endo-
metrial glands and stroma within the myometrium. Most
often, adenomyosis is a diffuse disease involving the entire
myometrium but can also present in a focal area of the
uterus as an adenomyoma. Most patients with adenomy-
osis are asymptomatic. When symptoms do occur, they
include dysmenorrhea, menometrorrhagia, and chronic
pelvic pain.
On ultrasound, globular enlargement of the uterus and
innumerable tiny echogenic foci representing cystic spaces
in the myometrium are the most specific findings for adeno-
myosis [67]. Sub-endometrial echogenic striations may be
seen, representing invasion of endometrial glands into the
myometrium. Color Doppler evaluation can demonstrate
randomly scattered vessels throughout the region of myo-
16 Female Genital Tract
metrial irregularity, a finding that helps differentiate adeno-
myosis from leiomyoma [67, 68]. MR imaging should be
considered to confirm the diagnosis and to determine the
extent of uterine involvement (Fig. 16.46) [69].
Malignant Tumors
Lymphoma
The uterus is an uncommon extranodal site of primary NHL.
However, uterine foci of lymphoma cells are frequently seen
at autopsy. When diffuse enlargement of the uterus is seen
in a patient with a known diagnosis of lymphoma, the pos-
sibility of uterine involvement should be raised. Findings on
ultrasound are nonspecific, and further MR imaging is rec-
ommended [70].
a b
Fig. 16.46 Uterine adenomyosis in a 15-year-old female. (a) Longitudinal
grayscale ultrasound image of the uterus demonstrates globular enlarge-
ment with punctate echogenic myometrial foci. (b) Sagittal T2-weighted,
fat-­suppressed MR image confirms increased thickness of the junctional
a b
Fig. 16.47 Nabothian cyst of the cervix in an 18-year-old female. Longitudinal grayscale (a) and transverse color Doppler (b) ultrasound images
show a simple, avascular cyst (arrowheads) within the cervix
719
Cervical Masses
Cervical masses are very rare, with the most common being
a nabothian cyst.
Benign Tumors
Nabothian Cyst
A nabothian cyst results from cystic accumulation of cervi-
cal mucus within dilated glands lining the cervical canal [71,
72]. On ultrasound, a nabothian cyst is typically a solitary,
well-circumscribed, unilocular cyst within the cervix that may
demonstrate posterior acoustic enhancement (Fig. 16.47) [71].
Diagnosis is based on location and absence of any clinical
symptoms. No treatment is usually required.
c
zone (arrowheads). (c) Axial T2-weighted, fat-suppressed MR image
shows band-like regions of T2 hypodensity (arrows) perpendicular to the
junctional zone consistent with focal adenomyosis
720
Malignant Tumors
Rhabdomyosarcoma
Rhabdomyosarcoma (RMS) is a soft tissue tumor arising
from primitive mesenchymal cells. RMS is a relatively com-
mon tumor in children, usually occurring in the head and
neck region. However, up to 3.5% of pediatric RMS devel-
ops in the female genitourinary (GU) tract [73, 74]. RMS has
a bimodal age distribution with the first peak between 2 and
6 years of age, and the second peak between 14 and 18 years
of age [1]. Most cases of RMS are sporadic but some are
associated with genetic syndromes, most often Li-Fraumeni
syndrome. Embryonal and botryoid variants are typically
found in the GU tract. The embryonal type has the best prog-
nosis, with a 5-year survival rate of 69%.
Most cases of RMS are sporadic but some are associated
with genetic syndromes, most often Li-Fraumeni syndrome.
Clinical manifestations of RMS can be highly variable and
depend on the location of the primary tumor and on the pres-
ence or absence of metastases. RMS may be locally aggres-
sive, invading the uterus and adjacent pelvic structures.
Approximately 10–20% of patients present with distant
metastases, most often to the lungs, bone, bone marrow, or
lymph nodes [74].
Any solid, vascular mass of the cervix or vagina seen on
ultrasound should raise significant suspicion for rhabdomyo-
sarcoma (Fig. 16.48). Imaging findings may vary based on
tumor subtype. The most common subtype seen in the GU
tract is the botryoid form that presents as a “cluster of grapes”
with both cystic and solid components. Embryonal, alveolar,
or pleomorphic subtypes may appear more solid [1, 43].
Once the diagnosis of RMS is confirmed histologically,
nearly all children will undergo chemotherapy prior to defin-
itive treatment with surgery or radiotherapy [74].
a b
Fig. 16.48 Cervical rhabdomyosarcoma in a 4-year-old female. (a)
Sagittal grayscale ultrasound image reveals a large, heterogeneous solid
mass arising from the cervix and distending the vagina. Calipers overlie
the uterus. (b) Transverse color Doppler ultrasound image of the cervix
E. L. Riedesel and H. J. Paltiel
Vaginal Masses
Evaluation of the vagina may be limited on transabdomi-
nal ultrasound due to its deep location within the pelvis.
In older, sexually active adolescents, the vagina can be
visualized as an endovaginal transducer is advanced toward
the cervix, although the anatomy may be distorted. A
transperineal/translabial imaging approach can be consid-
ered in younger patients and for evaluation of superficial
lesions. Sonovaginography, a technique where the vagina
is distended with sterile ultrasound gel or saline, has been
described in adults but has not yet been adopted in pediatric
practice [75].
Benign Masses
Benign cystic lesions of the vagina are frequently encoun-
tered during routine pelvic imaging. Solid masses in the
vagina are more concerning for malignancy, although benign
solid masses also occur and should be included in the dif-
ferential diagnosis.
 artner Duct Cyst
G
A Gartner duct cyst arises from vestigial remnants of the
mesonephric ducts along the anterolateral vaginal wall. If
portions of the ducts fail to regress, secretory activity gives
rise to cysts within the wall of the vagina [72]. Typically,
a Gartner duct cyst is small and anechoic, with an average
diameter of 2 cm (Fig. 16.49). However, cysts may enlarge to
the point where they are mistaken for other structures, such
as a cystocele or urethral diverticulum [76].
Of note, a Gartner duct cyst can also be associated with
abnormalities of the urinary tract. Cases of ectopic ureter,
c
and upper vagina reveals prominent vascularity within the mass. (c)
Sagittal contrast-­
enhanced, T1-weighted, fat-suppressed MR image
shows heterogeneous enhancement. The bladder (asterisk) is com-
pressed along its posterior aspect and displaced superiorly
16 Female Genital Tract
a b
Fig. 16.49 Gartner duct cyst in a 12-year-old female. (a) Longitudinal and (b) transverse grayscale ultrasound images demonstrate a simple cyst
(arrowheads) in the mid vagina. A Foley catheter (F) is present in the bladder
unilateral renal agenesis, and renal hypoplasia have been
reported in association with Gartner duct cyst [76].
Treatment depends on the symptoms and desire of the
patient. When asymptomatic, many patients choose clinical
observation. When symptomatic, cyst drainage, aspiration,
or intra-cystic tetracycline can be performed. In large and
symptomatic or recurrent cysts, excision or marsupialization
is indicated [77].
Bartholin Cyst
The paired Bartholin’s glands develop from the urogenital
sinus and are located symmetrically at the 4 and 8 o’clock
positions at the vaginal vestibule. These glands produce
mucus for vaginal lubrication. When the ducts of the gland
become obstructed, accumulation of mucus results in a
Bartholin cyst. On ultrasound they are typically unilateral,
unilocular simple cysts located in the distal vagina and mea-
suring between 1 and 4 cm in diameter. Clinically, they may
become infected, leading to abscess.
Rarely, these cysts can be congenital and should be consid-
ered in the differential diagnosis of a cystic mass located in the
introitus of a neonate. A large, neonatal cyst can cause urinary
obstruction, and discovery of a Bartholin cyst should prompt
further evaluation of the kidneys and urinary bladder [76, 78].
Current treatment approaches to symptomatic Bartholin
cyst or abscess include marsupialization with incision, drain-
age, and insertion of a Word catheter [79].
721
Inclusion Cyst
Epidermal inclusion cyst (epidermoid cyst) is the most
common non-embryological type of vaginal cyst [76]. It
develops from buried epithelial fragments following a sur-
gical procedure or other trauma. Inclusion cysts vary in
size from a few mm to several cm in diameter. Epidermal
inclusion cysts of the vagina have a similar appearance
on ultrasound as elsewhere in the body: they are typically
well-defined, ovoid masses with heterogeneous internal
contents. They may show posterior acoustic enhancement.
Since most of these cysts are asymptomatic, they do not
require intervention. An enlarging or symptomatic cyst can
be excised.
 araurethral Duct Cyst
P
The paraurethral Skene glands are located in the floor of the
urethra (Fig. 16.50). Obstruction of the ducts results in cyst
formation [76]. A paraurethral cyst is typically small and
of no clinical significance, although cysts larger than 2 cm
may cause patients to seek treatment for dysuria or obstruc-
tive voiding symptoms. The diagnosis can usually be made
on physical examination. Renal ultrasound and additional
urological investigation are necessary in cases of urinary
outflow obstruction. Ultrasound is useful in differentiating
a paraurethral duct cyst from other interlabial masses such
as prolapsed ectopic ureterocele, prolapsed urethra, and
rhabdomyosarcoma of the vagina [77].
722 E. L. Riedesel and H. J. Paltiel

a b

U U

Fig. 16.50 Paraurethral cyst in a 20-month-old female. Transperineal longitudinal grayscale (a) and color Doppler (b) ultrasound images demon-
strate a simple ovoid cyst (arrowheads) in the anterior midline, adjacent to the urethra (U) and anterior to the introitus

Excision is recommended for larger symptomatic cysts.

It is important to determine whether a vaginal cyst is of ure-
thral origin, as excision without the aid of a urethral catheter
and lack of postoperative urethral drainage may result in a
urethrovaginal fistula.
B
Fibroepithelial Polyp U
Fibroepithelial polyp of the vagina (FEPV) is a benign
mucosal polypoid lesion that can arise in the lower female
genital tract. Histologically it consists of a connective tis-
sue core covered by squamous epithelium. It is thought
to be rare, as few cases are reported in literature. These
lesions appear to be hormonally mediated and are rarely
seen before menarche, although there are a handful of case
reports of FEPV in neonates where they can present as
an interlabial mass [80]. Treatment is by simple excision,
and recurrence after complete resection is uncommon.
These lesions do not usually require imaging. However, Fig. 16.51 Vaginal rhabdomyosarcoma in a 3-year-old female.
Longitudinal grayscale ultrasound image of the pelvis demonstrates a
abdominal ultrasound examination can be useful in ruling mixed solid and cystic mass (arrow) within the distal vagina. U, Uterus;
out any association of the polyp with the urinary tract. B, bladder

Müllerian Papilloma
Benign müllerian papilloma is a rare childhood tumor of the
female reproductive tract that typically presents with vaginal
bleeding in a prepubertal female. Its ultrasound appearance
is similar to malignant rhabdomyosarcoma. Pathological
examination is critical to differentiate the two entities, as
benign müllerian papilloma can be treated with simple surgi-
cal excision [81].
Malignant Tumors
Rhabdomyosarcoma
Rhabdomyosarcoma of the vagina typically arises from
the anterior wall. On ultrasound, any solid vascular mass
of the cervix or vagina should raise significant suspicion
for rhabdomyosarcoma. Imaging findings may vary based
on tumor subtype. The botryoid subtype most commonly
occurs in the GU tract, presenting as a “cluster of grapes”
with both cystic and solid components (Fig. 16.51).
Embryonal, alveolar, or pleomorphic subtypes may appear
more solid [1, 43].

Vaginal tumors are more likely to be malignant in children
than in adults. The most common is rhabdomyosarcoma,
although clear-cell adenocarcinoma and endodermal sinus
tumor can also arise from the vagina [43].
 lear-Cell Adenocarcinoma and Endodermal
C
Sinus Tumor
Both clear-cell adenocarcinoma and endodermal sinus tumor
of the vagina are very rare tumors [82, 83]. Prenatal exposure
16 Female Genital Tract
to diethylstilbestrol (DES) is associated with an increased
risk for clear-cell adenocarcinoma of the vagina and cervix
early in life. Both clear-cell adenocarcinoma and endoder-
mal sinus tumor of the vagina often become very large and
fill the vagina at the time of diagnosis. On imaging, these
tumors are indistinguishable from rhabdomyosarcoma, and
definitive diagnosis is made with tissue sampling.
Vaginal Foreign Body
Vaginal foreign body may be suspected in patients who pres-
ent with vaginal bleeding or vaginal discharge [1]. They are
most often found in girls of 2 to 12 years of age. Common
foreign bodies include toilet paper, beads, small parts of toys,
and crayons. On ultrasound, foreign bodies are more echo-
genic than the surrounding tissues. One study found that for
foreign bodies larger than 5 mm, the rate of ultrasound accu-
racy in diagnosis reached 100% [84]. Foreign bodies often
introduce air into the vagina resulting in “dirty shadowing”
(Fig. 16.52). Their presence is confirmed at vaginoscopy at
which time they are extracted [84, 85].
Fig. 16.52 Vaginal foreign body (tampon) in a 15-year-old female.
Longitudinal grayscale ultrasound image of the vagina demonstrates a
prominent, linear echogenic focus with distal dirty shadowing
723
Pubertal Disorders
Puberty is broadly defined as the time at which a child
develops secondary sexual characteristics and reproductive
function. Gonadarche results from sex hormone produc-
tion triggered by LH and FSH secretion from the pituitary
gland. Adrenarche represents the increase in adrenal andro-
gen production that leads to the development of pubic hair
(pubarche), axillary hair, and apocrine glands. Thelarche is
the onset of breast development with the development of
breast buds. Menarche is the onset of menstrual cycles in
females [86]. Sexual maturity can be clinically described
using the Tanner staging system [87].
In females, the onset of puberty is usually between 8
and 13 years of age, with mean age of breast development
being10 years. Menarche typically follows 2.5 years after the
onset of breast development, at an average age of 12.5 years
[87]. Precocious puberty is defined as pubertal onset before
8 years of age. Delayed puberty is defined as the absence of
breast development in females by 13 years of age.
Precocious Puberty
There are four types of precocious puberty: central or “true”
isosexual precocity, peripheral or “pseudo” isosexual pre-
cocity, premature adrenarche, and premature thelarche.
The majority of cases of true isosexual precocious puberty
are a result of idiopathic activation of the hypothalamic-­
pituitary-­gonadal axis. The remaining causes are the result
of pituitary gland or hypothalamic lesions [88]. Peripheral or
pseudosexual precocity may result from ovarian neoplasms
(granulosa theca cell tumors), adrenal adenoma and carci-
noma, or ingestion of estrogen compounds.
Initial evaluation of abnormal pubertal development includes
assessment of hormone levels, thyroid function testing, and
bone age radiographs. In cases of diagnostic uncertainty, pelvic
ultrasound can be used to evaluate the size and appearance of
the uterus and ovaries. Careful evaluation for an ovarian mass
should be performed in patients with pseudosexual precocity.
A prepubertal appearance of the uterus and ovaries in a patient
with precocious breast development or pubic hair should sug-
gest isolated premature thelarche or adrenarche.
Ultrasound can also be used to monitor the effects of
treatment of precocious puberty. With appropriate therapy,
ovarian and uterine size should revert to expected prepuber-
tal volumes and configuration (Table 16.1).
724
Amenorrhea
Amenorrhea, the absence of menses, can be primary (no
menarche by age 15 years) or secondary (absence of men-
ses for more than 6 months in females who were previously
menstruating) [86]. Primary amenorrhea can result from
hypothalamic and pituitary lesions, adrenal tumor, primary
abnormalities of the ovary, and structural abnormalities of
the uterus or vagina.
 olycystic Ovary Syndrome
P have been described but do not contribute to the formal ultra-
Polycystic ovary syndrome (PCOS) is one of the most com-
mon endocrine disorders affecting up to 6–10% of females
of reproductive age [89]. PCOS is diagnosed by the pres-
ence of two of the three following criteria: androgen excess,
ovulatory dysfunction, and polycystic ovarian morphology
[90]. Clinical presentation is variable, and in adolescents,
clinical features of PCOS may be difficult to distinguish
from ­ pubertal changes. However, early diagnosis may
allow for screening of known metabolic complications and
timely intervention to reduce circulating androgen levels
which may significantly improve quality of life and long-
term health [91].
Fig. 16.53 Polycystic ovary syndrome (PCOS) in a 19-year-old
female. Longitudinal (left panel) and transverse (right panel) grayscale
ultrasound images of the (a) right and (b) left ovaries demonstrate bilat-
eral enlargement (volumes >10 mL) with increased echogenicity of
E. L. Riedesel and H. J. Paltiel
Diagnostic criteria for PCOS on ultrasound were first
established in 2003 [91]. Using the original 2003 Rotterdam
criteria, a polycystic ovary contains 12 or more follicles that
measure between 2 and 9 mm or an ovary with a volume
greater than 10 mL. These criteria have been the subject of
debate and revision in recent years. The number of ovarian
follicles has been adjusted to reflect improved ultrasound
technology with new diagnostic criteria requiring >20 fol-
licles per ovary.
Other morphological features of the polycystic ovary that
sound diagnostic criteria include peripheral location of folli-
cles (“string of pearls” sign) and hyperechoic central ovarian
stroma (Fig. 16.53).
Most importantly, international experts now endorse that
the performance of ultrasound criteria for diagnosis of PCOS
is poor in adolescents due to overlap with normal findings of
multi-follicular ovaries at this stage of life. In summary, while
ultrasound findings may provide supportive evidence of PCOS,
it is no longer required for definitive diagnosis of PCOS in the
adolescent [89, 91]. When ultrasound is performed, strict use
of the adjusted Rotterdam criteria is reserved for patients who
are more than 8 years beyond menarche.
a c
the central ovarian stroma and multiple peripheral follicles. (c) Sagittal
T2-weighted, fat-suppressed MR image shows >20 follicles in the right
ovary. A similar number were seen in the left ovary, thereby meeting
Rotterdam criteria for diagnosis of PCOS
16 Female Genital Tract
Canal of Nuck Disorders
The processus vaginalis is a normal embryologic outpouch-
ing of the parietal peritoneum that passes through the ante-
rior abdominal wall at the internal inguinal ring and extends
caudally toward the scrotum in males and the labia majora in
females. In females, the patent processus vaginalis is called
the canal of Nuck. A patent processus vaginalis can result in
a communicating hydrocele or organ herniation, most often
involving the bowel and pelvic organs [92].
Because obliteration of the processus vaginalis begins dur-
ing gestation, disorders of the canal of Nuck are associated
with prematurity [92]. The differential diagnosis for a palpable
lump in the groin of a female patient includes a canal of Nuck
Fig. 16.54 Ultrasound identification of the inguinal canal/canal of
Nuck. Transverse color Doppler image of the right anterior abdominal
wall demonstrates a normal appearance of the inferior epigastric vessels
(arrow) deep to the rectus abdominis muscle. The deep inguinal ring
(asterisk) is located just lateral to the inferior epigastric vessels
Fig. 16.55 Hydrocele of the canal of Nuck in a 17-year-old female. (a)
Transverse grayscale ultrasound image of the right pubic region demon-
strates a simple, anechoic fluid structure. (b) Longitudinal grayscale
725
hydrocele or hernia, a subcutaneous cyst such as a dermoid/
epidermoid cyst, and a lymph node or nodal abscess.
Ultrasound evaluation of the canal of Nuck in females
should be approached in a fashion similar to evaluation of
the inguinal canal in males. Use of a high-frequency linear
transducer is recommended.
The inferior epigastric vessels are a critical landmark (see
Fig. 15.43). These vessels originate from the external iliac
artery and vein immediately above the inguinal ligament.
There are usually three vessels that run together – two veins
and an artery – which are best identified in the transverse
plane over the rectus abdominis, just below the umbilicus.
These vessels can be traced inferiorly along the anterior
abdominal wall to a point at which they pass from superficial
to deep to the rectus abdominis muscles (Fig. 16.54). This
point is the medial boundary of the deep inguinal ring and
marks the superior aspect of the canal of Nuck.
Once the deep inguinal ring is identified, ultrasound
images should be obtained in a transverse plane along the
course of the canal of Nuck from the deep inguinal ring to
the labia majora. Longitudinal images are obtained by rotat-
ing the ultrasound probe along the long axis of the inguinal
ligament, angled obliquely toward the labia majora.
Hydrocele of the Canal of Nuck
An entirely patent processus vaginalis can result in a commu-
nicating hydrocele. On ultrasound, a communicating canal
of Nuck hydrocele appears as a tubular, anechoic inguinal
structure extending from the deep inguinal ring (Fig. 16.55).
ultrasound image shows extension of the fluid from the deep inguinal
ring along the expected course of the canal of Nuck
726
Visualization may be limited when ultrasound is performed
in the supine position in a calm child [93]. Evaluation is
improved when intra-abdominal pressure is increased with
crying or a Valsalva maneuver. Alternatively, older children
can be asked to stand for ultrasound evaluation.
If only the inferior part of the processus vaginalis remains
patent, an encysted hydrocele can form analogous to a hydro-
cele of the spermatic cord in males. Cysts of the canal of Nuck
are typically found in the superior aspect of the labia majora
[92]. On ultrasound, encysted hydrocele of the canal of Nuck
should be suspected in any well-circumscribed, anechoic cys-
tic structure in the subcutaneous tissues of the groin.
Hernia of the Canal of Nuck
Hernias of the canal of Nuck are uncommon, occurring most
often in premature infants. As the deep inguinal ring is at the
superior end of the canal of Nuck and lateral to the inferior
epigastric vessels, the canal of Nuck hernia is classified as
an indirect hernia. Herniation of the bowel or ovary is seen
most commonly (Fig. 16.56). Herniation of the uterus with
the ovary has also been reported (Fig. 16.57).
Although hernias of the canal of Nuck are rare, they can
result in serious complications that require prompt diagnosis
and clinical management. Incarceration, where the herniated
structure is trapped in the canal of Nuck and cannot be read-
ily reduced to its original location, has been reported in up
to 43% of inguinal hernias involving an ovary. One possible
explanation is that the herniated, edematous ovary is less
compressible than herniated bowel [92]. Doppler evaluation
should be performed to assess the blood flow in herniated
pelvic organs. Strangulation, where blood flow to the herni-
ated structure is compromised, may lead to necrosis. This
finding requires urgent surgical intervention.
Fig. 16.56 Hernia of the canal of Nuck in a newborn female. Transverse
grayscale ultrasound image shows the left ovary in the proximal ingui-
nal canal. (Image courtesy of Dr. Casey Sams, Warren Alpert Medical
School, Brown University, RI, USA)
E. L. Riedesel and H. J. Paltiel
Fig. 16.57 Hernia of the canal of Nuck in a 12-day-old female with
mixed gonadal dysgenesis. Longitudinal grayscale ultrasound image
shows a hemi-uterus (arrow) in the inguinal canal
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 Urinary Tract
17
Ghadir H. Kassab, Ian Robinson, Roisin Hayes,
Harriet J. Paltiel, D. Gregory Bates, Harris L. Cohen,
Richard A. Barth, and Gabrielle Christina Maria Colleran

MCDK Multicystic dysplastic kidney

Abbreviations MIBG Metaiodobenzylguanidine
MR Magnetic resonance
AAST American Association for the Surgery of Trauma
MRU MR urography
ACKD Acquired cystic kidney disease
PET Positron emission tomography
AIDS Acquired immunodeficiency syndrome
PNET Primitive neuroectodermal tumor
AKI Acute kidney injury
PSV Peak systolic velocity
AVF Arteriovenous fistula
PUNLMP Papillary urothelial neoplasm of low malignant
ATN Acute tubular necrosis
potential
ATRT Atypical teratoid rhabdoid tumor
PUV Posterior urethral valves
CEUS Contrast-enhanced ultrasound
RAS Renal artery stenosis
CeVUS Contrast-enhanced voiding urosonography
RCC Renal cell carcinoma
CKD Chronic kidney disease
RI Resistive index
COG Children’s Oncology Group
RVT Renal vein thrombosis
CPDN Cystic partially differentiated nephroblastoma
SIOP International Society of Paediatric Oncology
CT Computed tomography
STING Subureteric transurethral injection
DMSA Dimercaptosuccinic acid
SWE Shear wave elastography
eGFR estimated glomerular filtration rate
TCC Transitional cell carcinoma
ESRD End-stage renal disease
Tc Technetium
FDG Fluorodeoxyglucose
UPJO Ureteropelvic junction obstruction
IVC Inferior vena cava
(abbreviations continue)
MAG3 Mercaptoacetyltriglycine

Electronic Supplementary Material The online version of this chap-

ter (https://doi.org/10.1007/978-3-030-56802-3_17) contains supple-
mentary material, which is available to authorized users.

G. H. Kassab D. G. Bates
Department of Radiology, Children’s Health Ireland at Temple Department of Radiology, Nationwide Children’s Hospital, The
Street, Dublin, Ireland Ohio State University College of Medicine, Columbus, OH, USA
I. Robinson ∙ G. C. M. Colleran (*) H. L. Cohen
Department of Radiology, National Maternity Hospital Department of Radiology, Le Bonheur Children’s Hospital,
and Children’s Health Ireland at Temple Street, University University of Tennessee Health Science Center,
College Dublin School of Medicine, Memphis, TN, USA
Dublin, Ireland
R. A. Barth
e-mail: gcolleran@nmh.ie
Department of Radiology, Lucile Packard Children’s Hospital,
R. Hayes Stanford University School of Medicine, Stanford, CA, USA
Department of Radiology, Children’s Health Ireland at
Crumlin, Dublin, Ireland
H. J. Paltiel
Division of Ultrasound, Department of Radiology,
Boston Children’s Hospital and Harvard Medical School,
Boston, MA, USA

© Springer Nature Switzerland AG 2021 729

H. J. Paltiel, E. Y. Lee (eds.), Pediatric Ultrasound, https://doi.org/10.1007/978-3-030-56802-3_17
730
(abbreviations continued)
UTD Urinary tract dilation
UTI Urinary tract infection
UVJ Ureterovesical junction
UVJO Ureterovesical junction obstruction
VCUG Voiding cystourethrography
VHL Von Hippel–Lindau
VUR Vesicoureteral reflux
Introduction
Ultrasound is the imaging modality of choice for the assess-
ment of the urinary tract in children, particularly in the
workup of hydronephrosis, urinary tract infection, kidney
masses, and bladder abnormalities. Other important applica-
tions include the evaluation of stone disease, kidney failure,
renovascular disease, and kidney transplants. This chapter
reviews the development and anatomy of the urinary tract
followed by an overview of ultrasound imaging features of
urinary tract disorders in children. Brief discussions of clini-
cal presentation and treatment approach are also included.
Technique
Patient Positioning
Examination of the kidneys is usually performed with the
patient supine or in a lateral decubitus position, using the
liver or spleen as an acoustic window. A posterior approach
with the patient prone can be used if bowel gas obscures
visualization of the kidneys and is also useful for less coop-
erative patients. The supine and lateral decubitus positions
are best for visualization of the upper renal poles, while
prone positioning is optimal for imaging of the lower renal
poles. The kidneys of uncooperative younger children can
also be scanned from the back, while seated on a caregiver’s
lap. Bladder images are acquired with the patient supine.
Sedation is rarely required during the examination. Infants
can be fed or given a pacifier. Children older than a year of
age can usually be distracted by playing with toys, reading a
book, or watching a movie.
Ultrasound Transducer Selection
Studies should be performed using the highest frequency
transducer that will penetrate the region being examined to
optimize image resolution. A curved linear or sector trans-
G. H. Kassab et al.
ducer should be used for evaluating the kidneys. A curved
linear transducer is used to obtain bladder images.
Imaging Approaches
No special preparation is required for most patients. A mod-
erately full urinary bladder is desirable when a study is
­performed for urinary tract stones or hematuria. Children
undergoing renal Doppler evaluation should have nothing to
eat or drink prior to the study for a time period that varies
according to patient age.
Image acquisition in infants should begin with the urinary
bladder as they may void suddenly. Post-void images of the
bladder are obtained in patients who are toilet-trained.
Images of the kidneys are acquired in transverse and lon-
gitudinal planes [1, 2]. In order to assess renal parenchymal
echogenicity, longitudinal images must include a portion of
the liver and the spleen. The ureters are assessed at their ori-
gin from the renal pelvis and where they enter the bladder.
Grayscale Imaging
Grayscale imaging is the cornerstone of the renal ultrasound
examination and is used to assess renal size and parenchymal
echogenicity, document urinary tract dilation, and diagnose
stones and masses. Harmonic imaging is often helpful to mini-
mize artifacts.
Doppler Ultrasound
Doppler ultrasound is used in the evaluation of the renal vas-
culature when arterial or venous disease is suspected, as well
as in the routine evaluation of renal transplants.
Contrast-Enhanced Ultrasound
Contrast-enhanced ultrasound (CEUS) involves the intravenous
or intracavitary administration of ultrasound microbubbles and
can be used for real-time evaluation of the renal microvascula-
ture. This can be particularly beneficial in suspected vascular
disorders (infarction, cortical necrosis); differentiating between
solid and cystic renal lesions and between tumors and pseu-
dotumors; characterization and follow-up of complex cystic
masses; and identification of renal abscesses. It can be a valu-
able alternative to computed tomography (CT) or magnetic
resonance (MR) imaging where CT or MR imaging contrast is
contraindicated [3]. Contrast-enhanced voiding urosonography
17 Urinary Tract 731

is a nonionizing radiation alternative to conventional voiding Somites

cystourethrography.

Nephrotome
Elastography region

Mesonephric
Chronic kidney disease eventually results in intra-renal Gut region
fibrosis, with fibrosis degree correlating with disease sever-
ity. Shear wave elastography (SWE) is an imaging technique
that permits the noninvasive assessment of renal stiffness. Metanephric
SWE uses focused acoustic energy pulses to produce micro- Allantois region
scopic tissue displacement, which induces perpendicular
shear waves that are tracked by ultrasound as they progress Cloaca Ureteric
through tissue. Based on the physical principal that stiffer bud
tissues produce higher shear waves velocities, higher SWE
values correlate with higher degree of renal fibrosis. This Fig. 17.1 Diagram of the embryo depicting the nephrotome, meso-
may permit earlier detection of chronic renal disease, allow- nephric, and metanephric regions from which the pronephros, meso-
ing for prompt institution of appropriate treatment [4–6]. nephros, and metanephros will respectively arise. The pronephros and
mesonephros will regress, while the metanephros develops into the per-
However, there is currently very little published data on the manent kidney
use of elastography in pediatric chronic kidney disease.

Posterior
Normal Development and Anatomy Aorta Glomerulus cardinal vein

Normal Development

Development of the urinary tract starts in the fourth gesta-

tional week. A longitudinal ridge of mesodermal tissue, the
urogenital ridge, arises from the posterior wall of the abdom-
inal cavity along both sides of the abdominal aorta. The Mesonephric
urogenital ridge develops into the nephrogenic cords, the tubule

origins of the urinary system; and the gonadal ridge, which

ultimately forms the genital system.
Three pairs of excretory organs develop sequentially in the
human embryo, from cranial to caudal: the pronephros, the
mesonephros, and the metanephros (Fig. 17.1). The glomer-
uli and mesonephric tubules arise from the mesonephros and
drain into the mesonephric (wolffian) duct (Fig. 17.2). The
mesonephric duct in turn opens into the cloaca, the common
embryonic excretory cavity (Fig. 17.3). At the end of the first
trimester, the mesonephros regresses.
The ureteric bud, a diverticulum of the mesonephric duct,
along with the metanephric blastema, which arise from the
nephrogenic cord, gives rise to the metanephros in the fifth
week of gestation (Fig. 17.4). The stalk of the ureteric bud
becomes the ureter, and the branching of the ureteric bud
forms the renal pelvis, calyces, and collecting tubules. The
metanephric blastema leads to the formation of the glomeru-
lus, proximal tubule, loop of Henle, and distal tubule.
The cloaca is separated by the urorectal septum into the
urogenital sinus anteriorly and the rectum posteriorly in
the fourth to sixth weeks of gestation. The urinary bladder
Mesonephric
duct
Glomerular Subcardinal
Gut capsule vein
Fig. 17.2 Diagram of the functional unit of the mesonephros
develops from the superior portion of the urogenital sinus,
and its development is complete by the fourth gestational
month. The inferior portion of the urogenital sinus leads to
the development of the membranous urethra in both males
and females. In males, the caudal portion of the urogenital
sinus also gives rise to the spongy portion of the urethra that
meets the penile urethra. The penile urethra is derived from
a cord of ectoderm that grows from the tip of the glans penis
(Fig. 17.5).
732 G. H. Kassab et al.

Degenerating
Primary nephrotomes Gonad
Nephrotomes
tomes
nephric
duct

Mesonephric
tubules
Mesonephric
duct

Uninduced Metanephrogenic
intermediate blastema
mesoderm
Ureteric bud

Cloaca

Fig. 17.3 Diagram showing, from left to right, sequential development of the mesonephros, drainage of its duct into the cloaca, and early appear-
ance of the metanephros. Gonadal development proceeds in parallel with renal development and is depicted in the far-right panel

a b

Gut Mesonephric Urachus Mesonephric

Allantois duct duct

Metanephric Bladder Renal pelvis

Cloaca diverticulum

Cloaca Ureter

c d e
Buds of
arched
Cephalic major collecting
calyx tubules

Renal pelvis
Renal Primary straight
pelvis collecting tubules
Caudal major
Ureter calyx Metanephrogenic
tissue
Ureter
Developing
minor calyces

Fig. 17.4 Diagram demonstrating sequential development of the kidney from the ureteric bud and metanephros
17 Urinary Tract 733

a b

Mesonephros

Urogenital Mesonephric Mesonephros

sinus duct

Cloacal Metanephric
diverticulum Metanephric
membrane diverticulum

Urorectal Mesonephric
septum duct

c d

Allantois

Vesical part Mesonephros

Genital
Pelvic part Urogenital Mesonephric
tubercle
sinus duct
Phallic part
Metanephros
Rectum (primordium of
permanent kidney)

Ureter
Mesonephric duct
Mesonephros
e f Gonad

Metanephros Mesonephros

Urinary Metanephros
bladder
Ureter
Ureter
Urorectal
septum Rectum Mesonephric
duct

Pelvic part of
urogenital sinus

g h
Uterine tube

Kidney
Urachus Kidney Urachus

Urinary Testis
Ovary bladder
Uterus
Ureter
Penis
Clitoris
Ductus
Vagina Spongy deferens
urethra

Female Male

Fig. 17.5 Diagram depicting the sequential division of the cloaca into neys and gonads, as well as their relationships to the cloaca, urogenital
the urogenital sinus and the rectum, as well as development of the uri- sinus, and bladder are shown. Panels a, c, e, g, and h are lateral views.
nary bladder and urethra. The association between the developing kid- Panels b, d, and f are dorsal views
734 G. H. Kassab et al.

Normal Anatomy the liver and spleen. The renal pyramids also become less
prominent and more echogenic (Fig. 17.7). Renal sinus fat
Kidney increases with age resulting in increased echogenicity. At
The normal kidney is a bean-shaped organ that has a smooth any age, the maximum anteroposterior diameter of the renal
convex contour along its anterior, posterior, and lateral bor- pelvis should not exceed 1 cm [1].
ders. The concave medial surface of the kidney is the renal
hilum, which is in continuity with the renal sinus, a centrally Anatomic Variants
located cavity. The renal sinus contains the collecting system
of the kidney including the calyces and pelvis, as well as the Fetal Lobulation
main branches of the renal artery and renal vein. The remain- These are remnants of interlobar grooves that develop during
der of the renal sinus contains fat that increases with age. progressive fusion of the embryonic renal parenchymal lob-
ules termed “renunculi” (Fig. 17.8). They may be mistaken
Infant for renal scars or tumors. However, the indentations are
The anatomy and ultrasound appearance of the kidney vary regularly spaced and spare the pyramids, whereas the paren-
with age. In the premature infant and neonate, the echo- chyma overlying the pyramids is thinned with scarring [9].
genicity of the renal cortex is equal to or greater than that of
the liver. This is probably due to a greater volume of glom-
eruli in the renal cortex compared to adults.
L
The renal pyramids are more prominent and hypoechoic
in the neonatal kidney than in the older child and adolescent
(Fig. 17.6). This likely reflects the relatively larger medul-
lary volume in the neonatal kidney relative to the older child
or adult. In newborns, a transient increase in echogenicity
of the tips of the renal pyramids is commonly seen. This is
physiologic and usually resolves after a few days as the glo-
merular filtration rate increases [7]. The pyramids attain an
adult appearance by around 12 months of age.
The renal sinus is less echogenic in the neonatal kidney
relative to the adult. This is believed to be due to a paucity of
renal sinus fat in the neonatal kidney [8]. Fig. 17.7 Normal appearance of the kidney in a 4-year-old female.
Longitudinal grayscale ultrasound image of the right kidney demon-
Older Child and Adolescent strates renal cortical echogenicity less than that of the adjacent liver (L).
The renal pyramids (arrowheads) are much less perceptible compared
Renal cortical echogenicity decreases in the first year of to those of the neonate, and the echogenic central renal sinus (asterisk)
life and becomes isoechoic or slightly less echogenic than is more prominent

S
L

Fig. 17.6 Normal appearance of the kidney in a neonate. Longitudinal
grayscale ultrasound image of the right kidney in 1-week-old boy
reveals renal cortical echogenicity equal to that of the adjacent liver (L).
The renal pyramids (arrows) appear markedly hypoechoic, and there is
a paucity of renal sinus fat (asterisk)
Fig. 17.8 Fetal lobulations in a 6-month-old male. Longitudinal gray-
scale ultrasound image of the left kidney depicts an undulating contour
related to parenchymal indentations (arrowheads) that are located between
the renal pyramids. S, Spleen
17 Urinary Tract
Junctional Parenchymal Defect
Partial fusion of renunculi results in the so-called junctional
parenchymal defect, which appears as an echogenic trian-
gle in the renal cortex. This should be differentiated from a
parenchymal scar by its characteristic location, at the border
of the upper pole and interpolar region of the kidney, and its
continuity with the renal sinus (Fig. 17.9). A parenchymal
scar is characterized by loss of renal cortex, whereas a junc-
tional parenchymal defect is not [10].
Dromedary Hump
A dromedary hump is a focal protrusion in the lateral border
of the midportion of the left kidney caused by indentation
of the renal cortex by the adjacent spleen. It is one of the
Fig. 17.9 Junctional parenchymal defect in a 3-year-old female.
Longitudinal grayscale ultrasound image demonstrates a peripheral tri-
angular echogenic focus (arrowhead) in the upper pole of the right kid-
ney. L, Liver
a b
Fig. 17.10 Dromedary hump in a 2-year-old female. (a) Longitudinal
grayscale ultrasound image reveals a focal protrusion of the lateral
aspect of the left kidney (between arrows) that has a mass-like appear-
ance. A normal medullary pyramid (arrowhead) is seen in the central
735
classical “renal pseudotumors”; however, the characteristic
location, similar echogenicity to renal cortex, and further
extension of the adjacent calyx into the dromedary hump
relative to other calyces are helpful distinguishing features
(Fig. 17.10) [11].
Hypertrophied Column of Bertin
A hypertrophied column of Bertin is another anatomical
variant that can be mistaken for a renal tumor. It appears as
an oval or round mass of similar echogenicity to the renal
cortex and extends to the renal sinus between two medullary
pyramids. A smooth overlying renal contour is a unique fea-
ture that helps differentiate a hypertrophied column of Bertin
from a renal mass (Fig. 17.11) [12].
Fig. 17.11 Hypertrophied column of Bertin in a 12-year-old male.
Longitudinal grayscale ultrasound image of the right kidney (cursors)
demonstrates an oval mass (arrows) of similar echogenicity to the renal
cortex located between 2 pyramids (asterisks) and extending to the renal
sinus. The contour of the kidney overlying the “mass” (arrowheads) has a
smooth contour
portion of the “mass,” and the surrounding renal parenchyma is normal
in thickness and echogenicity. (b) Longitudinal color Doppler ultra-
sound image depicts normal vessels (arrowheads) within the mass-like
zone
736
a b
Fig. 17.12 Compensatory hypertrophy of the left kidney in a 3-year-­old
male with a non-functioning right multicystic dysplastic kidney (MCDK).
Longitudinal grayscale ultrasound images of the right (a) and left (b)
Fig. 17.13 Compound calyx in a 15-year-old male. Longitudinal grayscale ultrasound image demonstrates a branching, hypoechoic focus in the
lower renal pole (arrow). The overlying renal contour is normal without parenchymal thinning
Compensatory Hypertrophy
Compensatory hypertrophy of the kidney is a common cause
of unilateral renal enlargement. It can occur in association
with a contralateral renal congenital anomaly such as a mul-
ticystic dysplastic kidney (MCDK) where there is reduced or
absent renal function, or in association with acquired abnor-
malities such as unilateral scarring or following nephrectomy
(Fig. 17.12). It occurs in almost all children with a single
kidney. Compensatory renal hypertrophy can be detected in
utero and eventually develops regardless of whether or not
the nonfunctioning kidney is removed [13, 14].
Compound Calyx
Compound calyces are associated with compound pyramids
and they usually appear as branching, hypoechoic foci in the
upper and lower renal poles. They are associated with a nor-
mal renal contour without cortical thinning or calyceal dis-
G. H. Kassab et al.
flanks show an MCDK (arrowheads) on the right surrounded by abnor-
mal echogenic parenchyma. The normal left kidney (cursors) is larger
than expected for age, measuring 8.9 cm in length
tortion (Fig. 17.13). These features help to distinguish them
from abnormalities such as obstruction of the upper pole of a
duplex system, focal caliectasis, simple cyst, hydrocalyx due
to infundibular stenosis, or a hypoechoic mass [15].
Accessory Renal Artery
Accessory renal arteries are a very common, clinically rel-
evant variant of renal vasculature, especially in the assess-
ment of renal transplant donors, renal artery embolization, or
diagnostic search for renal artery stenosis. They occur in up
to one third of the population (30% unilateral; 10% bilateral;
very rarely more than two).
Accessory renal arteries can be hilar or polar arteries; the
former typically demonstrates a similar caliber to the main
renal artery, while the latter is usually smaller (Fig. 17.14).
The most common origin of a single renal artery is at the
level of L1-L2, whereas accessory renal arteries can arise
17 Urinary Tract
a b
A
Fig. 17.14 Accessory polar renal artery in a one-month-old male.
Longitudinal color Doppler ultrasound images of the left kidney with
spectral analysis show (a) the main renal artery (arrow) extending from
a b
A
C
Fig. 17.15 Retroaortic left renal vein in a 9-year-old male. (a) Transverse
grayscale ultrasound image shows a markedly compressed retroaor-
tic left renal vein (arrowheads) as it passes behind the aorta (A).
from the aorta or iliac arteries anywhere from the level of
T11 to the level of L4. They can also very rarely arise from
the lumbar or mesenteric arteries [16, 17].
Retroaortic Left Renal Vein
Retroaortic left renal vein is a rare (2–3%) vascular variant
of renal anatomy where the left renal vein courses dorsal to
737
A
the aorta (A) to the renal hilum. (b) An accessory polar renal artery
(arrowhead) arises more distally (arrowhead) from the aorta (A) and
extends to the lower renal pole
C
L
C, Inferior vena cava (IVC); L, proximal left renal vein. (b) Axial con-
trast-enhanced CT image confirms the presence of a retro-aortic left
renal vein (arrow). C, IVC; L, proximal left renal vein
the aorta to join the inferior vena cava (IVC) (Fig. 17.15),
or less commonly the iliac veins. Preoperative recognition
of this variant is very important in donor nephrectomy,
especially for a laparoscopic approach. Retroaortic left
renal vein can present clinically as hematuria secondary
to venous congestion from compression of the renal vein
against the spine [18].
738
a b
RK
C RK
Fig. 17.16 Circumaortic left renal vein in a 10-day-old female. (a) Coronal
color Doppler ultrasound image with spectral analysis shows normal
venous flow in the left renal vein (arrowheads) proximal to its bifurcation.
A, Aorta; C, IVC; RK, right kidney. (b) Coronal color Doppler ultrasound
Circumaortic Left Renal Vein
Circumaortic left renal vein is the most common anatomical
variant of the left renal venous system, occurring in about
5–7% of the population. The left renal vein bifurcates into
anterior and posterior branches that encircle the abdominal
aorta (Fig. 17.16). Recognition of a circumaortic left renal
vein is critical during retroperitoneal surgery or nephrectomy
to avoid potentially fatal hemorrhage [19].
Ureter
Normal Anatomy
The ureter is a tubular structure that extends through the ret-
roperitoneum from the kidney to the bladder. The proximal
ureter tapers smoothly from its origin at the ureteropelvic
junction (UPJ) and terminates at the ureterovesical junction
(UVJ). The distal portion of the ureter extends obliquely
through the bladder wall and opens into the bladder lumen
at the ureteral orifice. The ureteral wall consists of three
muscular layers with an outer adventitial layer that contains
blood vessels and lymphatics. The ureter lies adjacent to the
psoas muscle and passes anterior to the common and exter-
nal iliac vessels as it enters the pelvis.
Ureteral Jets
When urine in the ureter approaches the vesicoureteral
junction, it is ejected into the urinary bladder by smooth
muscle contraction at the vesicoureteral sphincter. Prior
research has shown that color Doppler imaging is more
sensitive for demonstrating ureteral jets compared to gray-
scale ultrasound [20]. On grayscale ultrasound, ureteral jets
are seen as a burst of low intensity echoes emitted from
the ureteral orifice into the urinary bladder (Fig. 17.17). It
G. H. Kassab et al.
image of the aorta and IVC shows that the left renal vein bifurcates into an
anterosuperior branch (arrows) and a posteroinferior branch (arrowheads)
that encircle the aorta (A) before each drains separately into the IVC (C).
RK, Right kidney. The left kidney is outside the field of view
is believed that the presence of a ureteral jet is a sign of a
non-obstructed UVJ. The complete absence of a ureteral jet
on either side is suggestive of ipsilateral UVJ obstruction
[21, 22].
Bladder
Normal Anatomy
The bladder is located in the pelvis, posterior to the pubic
bones and anteroinferior to the peritoneal cavity. The perito-
neum is reflected off the anterosuperior aspect of the bladder.
The bladder trigone is a smooth triangular region within the
inner posterior portion of the bladder that is demarcated by
the ureteric and internal urethral orifices. The bladder neck
is the zone at the base of the trigone that surrounds the inter-
nal urethral orifice and opens into the urethra. Specialized
­detrusor smooth muscle bundles encircle the bladder neck
and form the internal urethral sphincter. The bladder neck
and trigone are constant in shape and position, whereas the
remainder of the bladder undergoes changes in shape and
position depending on the volume of contained urine.
The normal urinary bladder has a thin wall. Bladder vol-
ume and wall thickness are affected by the degree of bladder
filling (Fig. 17.18). The distal ureters may be visible at the
bladder base, especially if the child is well hydrated, likely
related to the normal transient passage of urine associated
with peristalsis. Bladder wall thickness can increase with
inflammation or muscular hypertrophy.
In infants, the urinary bladder is partially intra-abdomi-
nal in location and only assumes a more pelvic position with
growth. It does not truly become pelvic until about the sixth
year of life, and the ureters are completely intra-abdominal
in location until then as well.
17 Urinary Tract 739

a b

Fig. 17.17 Ureteral jets in a 15-year-old male. Transverse color Doppler ultrasound images of the bladder show jets of urine from the right (a)
and left (b) ureteral orifices

a b

c d

Fig. 17.18 Normal bladder in a 4-year-old male. Transverse (a) and verse (c) and longitudinal (d) images obtained several minutes later
longitudinal (b) grayscale ultrasound images obtained with a moderate with the bladder markedly filled
degree of filling show a thicker bladder wall (arrowheads) than trans-
740
a b
Fig. 17.19 Bladder ears in a 2-week-old male. Transverse grayscale
(a) and contrast-enhanced voiding urosonography (ceVUS) (b) images
of the pelvis demonstrate outpouchings (asterisks) from the right and
The urachal remnant connects the bladder to the umbili-
cus. The dome of an unfilled bladder lies midway between
the pubis and the umbilicus. With filling, the bladder dome
may reach the umbilicus [23].
Anatomic Variants
Bladder Ears
Bladder ears are a normal physiologic finding in young
infants, where the lateral portions of the urinary bladder
protrude into the inguinal canals. The higher relative posi-
tion of the urinary bladder in infants compared to its position
in adults places it in close proximity to the internal inguinal
rings. Bladder ears can be demonstrated on voiding cystoure-
thrography, intravenous pyelography, ultrasound, and CT. On
ultrasound, bladder ears are seen as fluid-filled diverticula
extending from the lateral aspect of the bladder (Fig. 17.19).
Unlike true diverticula, they are smooth walled, with a wide
neck, and more transient in nature [24].
G. H. Kassab et al.
left anterolateral aspects of the bladder in keeping with bladder ears. (c)
Image from a voiding cystourethrogram (VCUG) depicts the bladder
ears (arrows) protruding into the right and left inguinal canals
Bladder ears are usually asymptomatic and resolve spontane-
ously. Nevertheless, failure of recognition during inguinal hernia
repair can potentially result in injury due to partial or complete
excision of bladder ears contained in the hernia sac [25].
Urethra
Normal Anatomy
The urethra is a tubular structure that connects the urinary
bladder neck to the urethral meatus for excretion of urine
from the body. In males and females, the internal and the
external sphincters control urethral function. The involuntary
internal sphincter is formed by the smooth muscles lining
the bladder neck and urethra and is innervated by the sym-
pathetic nervous system. The somatic external sphincter is
innervated by the pudendal nerve.
The proximal urethra can be imaged via a transabdominal
approach, while a transperineal approach is useful for imag-
ing the more distal portions of the urethra in both genders. In
17 Urinary Tract
a b
B B
Fig. 17.20 Normal urethra in a 38-day-old male. (a) Longitudinal
transperineal grayscale ultrasound image depicts the urethra (arrow-
heads) extending from the base of the bladder (B) along the length of
the penis with a small amount of anechoic urine (arrow) within the
U V
R spongiosum along the length of the penis (Fig. 17.20). The
Fig. 17.21 Normal female urethra. Transperineal longitudinal gray-
scale ultrasound image depicts the urethra (U), vagina (V), and rec-
tum (R)
older, sexually active teenage girls, a transvaginal approach can
also be considered [26, 27]. Urine is anechoic on ultrasound
where it is seen centrally in the tubular urethra. The urethra is
located anterior to the vagina in females, anterior to the rectum
in males, and deep to the pubic symphysis in both genders.
Male Urethra
The male urethra originates at the bladder neck and opens
at the external urethral meatus. The male urethra is approxi-
mately 5 cm in length at birth, 8 cm at 3 years of age, and
17 cm in adulthood. The prostatic and membranous segments
comprise the posterior urethra in males, while the bulbar and
penile segments comprise the anterior urethra. The prostatic
utricle, ejaculatory ducts, and prostatic ducts drain into the
verumontanum, a ridge in the floor of the posterior urethra
at the junction of the prostatic and membranous segments
741
c
proximal urethra. (b) Longitudinal transperineal ceVUS image shows a
normal urethra (arrowheads). B, Bladder. (c) Image from a VCUG con-
firms a normal bladder and urethra
of the urethra. The narrowest portion of the urethra is the
membranous portion where it passes through the external
urethral sphincter. The external sphincter in males is located
just inferior to the prostate gland and surrounds the membra-
nous urethra. The penile urethra extends through the corpus
urethral glands (glands of Littré) drain into the penile urethra.
Female Urethra
The female urethra extends from the bladder neck and opens
directly onto the perineum between the clitoris and the vagina
(Figs. 17.21 and 17.22). It is approximately 2 cm in length at
birth, 2.5 cm at 5 years of age, and 4 cm in adulthood. The
external urethral sphincter is located within the distal two
thirds of the urethra and is composed of striated muscle.
The urethra plane extends obliquely in an anteroinferior
direction. The proximal two-thirds of the female urethra are
lined by transitional epithelium, whereas the distal one third
is lined by stratified squamous epithelium.
Congenital Anomalies
 nomalies of Renal Number, Position, Fusion,
A
and Growth
Congenital renal anomalies are not uncommon, occurring in
3–5 per 1000 live births. They are considered the leading cause
of renal failure in children and may predispose to stone forma-
tion, infection, hypertension, and renal failure [28].
Renal Agenesis
Renal agenesis refers to complete absence of one or two kid-
neys that results from failure of induction of the metanephric
blastema by the ureteric bud. Renal agenesis can be diagnosed
on prenatal ultrasound imaging. Bilateral renal agenesis is
742 G. H. Kassab et al.

a b

Fig. 17.22 CeVUS of the urethra in a 17-month-old female. (a) Longitudinal transperineal image shows a normal urethra (arrowheads).
B, Bladder. (b) Image from a VCUG confirms a normal bladder and urethra

is more common than renal agenesis). Knowledge of the

embryological pathway of the normal ascent of the kidney is
important to avoid missing a kidney in an abnormal position
somewhere along this path.

L Renal Duplication
Fig. 17.23 “Lying down” adrenal sign in a 6-year-old female with
right renal agenesis. Longitudinal grayscale ultrasound image of the
right renal fossa demonstrates absence of the kidney and a flattened,
elongated appearance of the adrenal gland (arrows). L, Liver
usually incompatible with life; it results in oligohydramnios
that leads to severe pulmonary hypoplasia [28]. Unilateral
renal agenesis is much more common, occurring in 1/1300
pregnancies. Most children with unilateral renal agenesis are
asymptomatic and have a normal life expectancy [29].
Ultrasound features include absence of the kidney and
ipsilateral renal artery with a flattened appearance of the
ipsilateral adrenal gland, the so-called “lying down” adre-
nal sign (Fig. 17.23). Careful examination of the abdomen
should be carried out to search for an ectopic kidney (which
Renal duplication is the most common congenital renal tract
anomaly, with an incidence of 0.7% in the general popula-
tion and 2–4% in patients investigated for urinary tract infec-
tion (UTI) [30]. It is much more common in girls than boys.
Renal duplication can be unilateral or bilateral, partial or
complete. The spectrum of partial duplication ranges from a
bifid pelvis with a single ureter to bifid ureters, which unite at
some point before emptying into the urinary bladder. Partial
duplication accounts for 95% of cases of all renal duplica-
tions, is usually asymptomatic, and is clinically insignificant
with no increased risk of urinary tract disorders compared to
the general population.
In a complete duplication, there are two ureters that drain
separately into the urinary bladder. Patients with compli-
cated renal duplication may present with UTI, failure to
thrive, abdominal mass, hematuria, or symptoms of bladder
outlet obstruction related to a ureterocele.
According to the Weigert-Meyer rule, the future lower
pole ureter separates early from the wolffian duct and
migrates superiorly and laterally during bladder growth,
inserting orthotopically into the bladder trigone, whereas
the upper pole ureter remains with the wolffian duct longer
and inserts ectopically in the bladder or into a wolffian
duct remnant [31].
In a complete duplication, the lower pole ureter is prone
to vesicoureteral reflux (VUR), whereas the upper pole
ureter is more likely to insert ectopically with or without
a uretrocele and is often obstructed.
17 Urinary Tract 743

Fig. 17.24 Renal duplication in a 10-year-old female. Longitudinal

grayscale ultrasound image of the right kidney shows an oblique band
of normal renal parenchyma (asterisk) that divides the renal sinus
(arrows) into two parts

On ultrasound, a duplex kidney is sometimes larger than a c

normal single-system kidney. The echogenic renal sinus may
appear divided by an intervening column of renal parenchyma
(Fig. 17.24). If there is associated vesicoureteral reflux to the
lower moiety or obstruction of the upper moiety, hydronephro-
sis may be present (Figs. 17.25 and 17.26). Hydronephrosis of
differing severity in the upper and lower moieties is a useful
indicator of the presence of a completely duplicated system.
It is not uncommon for the upper pole moiety to be dysplas-
tic and replaced by cysts.
The vast majority of duplex kidneys do not require inter-
vention. Depending on the function of the upper moiety, an
ectopic ureter may be treated surgically with ureteroureter-
ostomy (where the ectopic upper pole ureter is anastomosed
to the normally located lower pole ureter) or with reimplan-
tation to the urinary bladder. A nonfunctioning or dysplastic
renal moiety may require heminephrectomy and resection of
the proximal ureter [32].

Supernumerary Kidney
A supernumerary kidney is an accessory kidney, a very rare
congenital anomaly thought to result from an abnormal
division in embryonic life of the nephrogenic cord into two
metanephric blastemas with or without division of the ure-
teric bud. The supernumerary kidney may have partially or
completely duplicated ureters [33]. It is usually located on
the left side of the abdomen caudal to the normotopic kidney
and very rarely may be bilateral.
The accessory kidney is usually small in size with reduced
function. Patients may present with fever, abdominal pain, or
abdominal mass, which are related to hydronephrosis, pyelo-
nephritis, stone disease, or tumor.
A supernumerary kidney can be evaluated by ultra-
sound (Fig. 17.27), CT, MR imaging, or nuclear scintigraphy.
Fig. 17.25 Bilateral duplex kidneys in a 3-month-old female with vesico-
ureteral reflux and unilateral obstruction. (a) Longitudinal grayscale ultra-
sound image reveals a duplex right kidney (cursors) with marked dilation of
the lower pole collecting system (asterisk) and diffuse thinning of the over-
lying renal parenchyma. The upper pole moiety appears normal. (b)
Longitudinal grayscale ultrasound image of the left kidney demonstrates
significant dilation of the upper pole collecting system (asterisk) with dif-
fuse thinning of the overlying renal parenchyma. Mild dilation of the lower
pole collecting system (arrowhead) is also present. (c) Image from a fluoro-
scopic VCUG shows reflux (arrowheads) into the right upper and lower
pole renal collecting systems with partial ureteral duplication, and into the
left lower pole moiety. The absence of reflux to the dilated left upper pole
moiety indicates obstruction
744
a b
Fig. 17.26 Duplex kidney with ectopic ureterocele in a 12-month-old
female. (a) Longitudinal grayscale ultrasound image reveals a duplex
left kidney with severe collecting system dilation (asterisk) and a tortu-
ous, dilated ureter (arrows). There is also mild dilation of the lower pole
Fig. 17.27 Supernumerary kidney in a 6-year-old male. (a) Longitudinal
grayscale ultrasound image of the right renal fossa demonstrates two
fused kidneys (cursors). K1, Kidney 1; K2, kidney 2. (b) Longitudinal
grayscale ultrasound image of the left renal fossa reveals a single normal-
appearing kidney (cursors)
Ultrasound is useful for morphological characterization, while
nuclear scintigraphy and MR urography (MRU) are useful for
functional assessment.
Owing to the frequent presence of associated pathology,
two-thirds of these kidneys are symptomatic and will ulti-
mately require nephrectomy.
G. H. Kassab et al.
collecting system (arrowhead). (b) Longitudinal grayscale ultrasound
image of the pelvis demonstrates the dilated distal left ureter (U) and
the ectopic ureterocele (asterisk) at the base of the bladder (B)
Renal Ectopia
Simple Ectopia
During embryological development, the kidneys normally
ascend from the pelvis to the retroperitoneum. Renal ectopia
occurs as a result of arrested migration of the kidney. Ectopia
can be either simple or crossed. In simple ectopia, the kidney
and the ureter are on the expected side of the spine and are
usually located in the pelvis. On ultrasound imaging, the kid-
ney is customarily smaller, malrotated, and has a dysmorphic
configuration (Fig. 17.28).
Due to the superficial position of the pelvocalyceal sys-
tem compared to the normal kidney, the normal sinus echo
complex is absent in an ectopic kidney [34]. The ureter is
usually short. The ectopic kidney retains the embryonic
aorto-iliac branches that usually degenerate during normal
ascent. Very rarely, an ectopic kidney can be located in the
posterior thorax, usually on the left side.
 rossed Renal Ectopia
C
In crossed renal ectopia, the ectopic kidney is positioned on
the side opposite to its expected location in the retroperito-
neum. The left kidney is more often involved than the right.
The upper pole of the ectopic kidney usually fuses with the
lower pole of the orthotopic kidney. The ureter draining the
ectopic kidney crosses the midline to drain into the bladder
in a normotopic location on the trigone.
Ultrasound will show a “mass” consisting of the fused
kidneys with two renal sinuses and absence of a kidney in the
contralateral renal fossa (Figs. 17.29 and 17.30).
Crossed renal ectopia can be an incidental finding,
although there is an increased risk of hydronephrosis, renal
stones, and infection in these kidneys [35].
17 Urinary Tract
a b
U B
Fig. 17.28 Ectopic left kidney in a 3-month-old male. (a) Longitudinal
grayscale ultrasound image of the left pelvis shows a small, dysplastic
kidney (arrow) with a dilated ureter (U) adjacent to the bladder (B).
Fig. 17.29 Crossed renal ectopia in a 2-year-old male. Oblique longi-
tudinal grayscale ultrasound image of the right upper quadrant demon-
strates fusion of the lower pole of a normally located right kidney (R) to
the upper pole of the ectopic left kidney (L)
Horseshoe Kidney
Horseshoe kidney is the most common congenital renal
fusion anomaly, occurring in approximately one in 400 live
births with a male predominance of 2:1 [35]. It is reported in
about 20% of individuals with trisomy 18 and in at least one
third of females with Turner syndrome [36].
In this anomaly, the lower poles of both kidneys fuse to
produce an isthmus, either fibrous or parenchymatous, that
crosses the midline anterior to the aorta. The isthmus hin-
ders the cranial ascent and normal rotation of the kidney as
it encounters the inferior mesenteric artery. As a result, the
lower poles of the kidneys are rotated medially and the kidney
is low-lying. A horseshoe kidney usually receives anomalous
blood supply either from the lower aorta or iliac arteries.
There is an increased incidence of stone formation, infec-
tion, and risk of renal obstruction due to abnormal orientation
745
(b) Longitudinal grayscale ultrasound image of the left flank reveals an
empty renal fossa. S, Spleen
RK
Fig. 17.30 Crossed renal ectopia in a 6-month-old female with a multi-
cystic dysplastic kidney (MCDK). Oblique longitudinal grayscale ultra-
sound image of the right upper quadrant shows fusion of the lower pole of
the right kidney (RK) to the upper pole of an ectopic left MCDK (arrows)
of the ureters. A horseshoe kidney is also more susceptible to
traumatic injury. A horseshoe kidney is associated with addi-
tional congenital anomalies in about one third of patients,
that can include vertebral, anorectal, tracheal, and esopha-
geal malformations [37].
Ultrasound features include abnormal rotation and infe-
rior location of the kidney resulting in poor visualization of
the inferior pole and frequent underestimation of the length
of the right and left moieties. The renal pelves are located
anteriorly, and an isthmus of tissue is seen crossing anterior
to the spine and retroperitoneal vessels (Fig. 17.31).
Pancake Kidney
Pancake kidney, sometimes referred to as a cake, discoid, or
lump kidney, is a very rare anomaly representing less than 2%
of renal fusion anomalies [38]. It results from fusion of the
746 G. H. Kassab et al.

To address the perceived need for a unified classification

system with a widely accepted terminology for the diagno-
sis and management of antenatal and postnatal urinary tract
dilation, a classification system was devised in 2014 during a
consensus meeting of eight medical societies involved in the
prenatal and postnatal diagnosis and management of urinary
tract dilation [40, 41].
The urinary tract dilation or UTD grading system is cor-
related with the risk of postnatal uropathy and is based on six
ultrasound features: (1) anterior-posterior renal pelvic diame-
ter, (2) calyceal dilation with differentiation between postnatal
central and peripheral calyceal dilation, (3) renal parenchy-
mal thickness, (4) renal parenchymal appearance, (5) bladder
abnormalities, and (6) ureteral abnormalities (Fig. 17.32).
For antenatal studies, an additional ultrasound feature
Fig. 17.31 Horseshoe kidney in a 5-year-old male. Transverse gray- that is reported is the quantity of amniotic fluid. Normal
scale ultrasound image through the mid-abdomen shows fusion of the
imaging parameters are also defined. A postnatal follow-up
lower poles of the right (K) and left kidneys (L) by an isthmus of renal
parenchyma (cursors) anterior to the spine (S) scheme has also been proposed based on the UTD classifica-
tion (Fig. 17.33) [40]. The hope is that future comparisons
of the grading system with clinical outcomes such as renal
superior and inferior poles of the kidneys with no intervening function and the need for surgical intervention will lead to
septum. It is usually located in the pelvis anterior to the aor- refinements in the classification scheme and thereby increase
tic bifurcation. With very few exceptions, a pancake kidney is its value in guiding the clinical management of infants and
drained by two separate, anteriorly located ureters that enter children with hydronephrosis.
the urinary bladder in a normal location. Pancake kidney is at
risk of similar complications as horseshoe kidney.  reteropelvic Junction Obstruction
U
Ureteropelvic junction obstruction (UPJO) is the most com-
Renal Hypoplasia mon cause of neonatal hydronephrosis and congenital uri-
Renal hypoplasia, which is a congenitally small kidney with nary tract obstruction. Congenital UPJO is usually caused by
fewer calyces and papillae than normal (less than 6), results intrinsic stenosis of the proximal ureter at the UPJ and less
from incomplete renal development. The function of a hypo- commonly by extrinsic compression from a crossing renal
plastic kidney can be normal or slightly reduced. vessel. Although the underlying mechanism is uncertain, it
Renal hypoplasia is usually unilateral and can be global or is thought that there is abnormal development of the circu-
segmental. Global hypoplasia can be differentiated from renal lar musculature and/or collagen fibers of the proximal ure-
atrophy resulting from chronic pyelonephritis by the smooth ter. The majority of cases are identified antenatally, although
outline and normal calyceal system in the former compared to UPJO can present at any age.
the irregular outline and focal dilation of renal calyces result- In the neonatal period, UPJO is usually asymptomatic and
ing from scarring in the latter. Differentiation of global hypo- presents with a palpable abdominal mass while in older chil-
plasia from a small kidney with a smooth outline resulting dren it can present with abdominal pain, hematuria, recurrent
from chronic vascular disease is challenging. Segmental renal urinary tract infection, stone formation, and occasionally,
hypoplasia, also known as Ask-Upmark kidney, is a curable intermittent pain after drinking large volumes of fluid or flu-
cause of secondary hypertension in young adults [28, 39]. ids with a diuretic effect.
Renal dysplasia may develop as a result of congenital
UPJO, with the degree of dysplasia dependent on the severity
Anomalies of the Renal Collecting System of the obstruction and its time of onset during gestation [42].
and Ureter If obstruction is incomplete and develops after the 36th week
of gestation, varying degrees of hydronephrosis are present
Classification of Prenatal and Postnatal without histologic evidence of renal dysplasia. Incomplete
Hydronephrosis obstruction that develops between the 10th and 36th week of
Urinary tract dilation is identified in 1–2% of fetuses on prena- gestation may result in dysplastic parenchymal changes with
tal ultrasound imaging and is associated with a wide range of or without cyst formation along with hydronephrosis. At the
possible postnatal outcomes. The relative absence of evidence-­ most severe end of the spectrum, multicystic dysplastic kid-
based information correlating the severity of prenatal dilation ney results from complete obstruction before 8–10 weeks of
to postnatal urologic abnormalities has led to significant varia- gestation [43].
tion in the clinical management of infants and children with Rupture of the renal collecting system from severe obstruc-
antenatally diagnosed urinary tract dilation. tion can also occur in utero resulting in urinary ascites and
17 Urinary Tract
Fig. 17.32 Urinary Tract Dilation (UTD) Risk Stratification—Postnatal
Presentation for UTD P1 (Low Risk), UTD P2 (Intermediate Risk), and
UTD P3 (High Risk). Stratification is based on the most concerning ultra-
sound finding. APRPD, Anterior-posterior renal pelvic diameter; abnl,
Fig. 17.33 Management schema based on Urinary Tract Dilation (UTD)
classification system risk stratification of UTD P1, UTD P2, and UTD
P3. (From Nguyen HT, Benson CB, Bromley B, Campbell JB, Chow J,
subcapsular urinoma. Congenital UPJO may be associated
with other genitourinary abnormalities, including vesico-
ureteral reflux, ureterovesical junction obstruction (UVJO),
duplex kidney, horseshoe kidney, and crossed fused ectopia.
Ultrasound imaging will often show dilated calyces com-
municating with a dilated renal pelvis (Fig. 17.34). The
proximal ureter appears collapsed and the urinary blad-
der is normal. Occasionally, the ipsilateral ureter may be
747
abnormal. (From Nguyen HT, Benson CB, Bromley B, Campbell JB,
Chow J, Coleman B, et al. Multidisciplinary consensus on the classifica-
tion of prenatal and postnatal urinary tract dilation (UTD classification
system. J Pediatr Urol. 2014;10(6):982–98. [40])
Coleman B, et al. Multidisciplinary consensus on the classification of
prenatal and postnatal urinary tract dilation (UTD classification system.
J Pediatr Urol. 2014;10(6):982–98. [40])
dilated due to associated vesicoureteral reflux or UVJO.
Renal parenchymal thickness and echogenicity are variable
depending on the chronicity of the condition and the degree
of associated parenchymal dysplasia, if present. Renal dys-
plasia manifests as increased cortical echogenicity with or
without cysts. Doppler ultrasound is useful in differentiating
a dilated renal pelvis from prominent renal vessels and can
also identify aberrant renal vessels [43].
748
a b
Fig. 17.34 Ureteropelvic junction obstruction in a 6-year-old female.
Longitudinal (a) and transverse (b) grayscale ultrasound images of the
right kidney demonstrate moderate-to-severe hydronephrosis with mild
diffuse thinning of the renal parenchyma (arrowheads). Anteroposterior
Indications for surgical intervention include pain, infec-
tion, and renal stones; massive hydronephrosis with a renal
pelvic diameter greater than 5 cm; increasing hydronephro-
sis and decreased renal function (generally less than 40% of
split renal function or serial loss of more than 10% function);
and patient and/or parental request for definitive treatment
[44]. Surgery usually consists of a dismembered pyeloplasty,
where the obstructed segment is excised and the renal pelvis
is re-anastomosed to the ureter.
Ureteropelvic Junction Obstruction Caused by
Crossing Vessel
About 10% of cases of ureteropelvic junction obstruc-
tion (UPJO) are caused by an aberrant or accessory renal
artery that crosses the lower pole of the kidney result-
ing in compression of the UPJ and blockage of urine flow.
Approximately 60% of patients will present with recurrent
renal colic (pain, nausea, vomiting) compared to only10%
with intrinsic UPJO.
Since vascular obstruction is usually intermittent, most
patients have normal renal function. Ultrasound imaging
during a symptomatic episode is usually necessary to make
the diagnosis of UPJO. Color Doppler ultrasound can some-
times directly visualize the crossing vessel.
If UPJO is thought to be caused by a crossing vessel, a dis-
membered pyeloplasty is performed. An open surgical approach
is recommended rather than an endoscopic or laparoscopic
pyelotomy in order to avoid potential vascular complications
[45]. Re-anastomosis is performed anterior to the crossing
vessel.
Congenital Megacalyces
Congenital megacalyces is an anomaly characterized by
nonobstructive, nonprogressive calyceal dilation that can
mimic hydronephrosis. The medullary pyramids are hypo-
G. H. Kassab et al.
c
pelvic diameter measurement is obtained at the renal hilum (cursors). (c)
Image from technetium (Tc)-99m-mercaptoacetyltriglycine (MAG3)
diuretic reno­graphy obtained 1 hour after injection shows retention of
radioisotope in the right renal collecting system (arrow). R, Right side
plastic and have a semilunar configuration rather than a
normal triangular shape. Calyceal fornices and papillary
impressions are absent. The abnormal calyces are also
increased in number and have a faceted, polygonal appear-
ance. The renal pelvis is usually normal in size.
Patients are generally asymptomatic. However, the dilated
calyces can predispose to stasis, infection, and stone forma-
tion. Rarely, congenital megacalyces can coexist with primary
­megaureter, and recognition of their coexistence is important
in order to avoid unnecessary surgery on the ureteropelvic
junction [46, 47].
Ultrasound findings include a large kidney with normal
cortical thickness [48]. The calyces will appear dilated
while the renal pelvis will usually be normal in size, a
potential clue to the diagnosis. If there is coexisting pri-
mary megaureter, vesicoureteral reflux, or another cause
of distal obstruction, the renal pelvis may appear dilated
(Fig. 17.35). MRU or contrast-­enhanced CT will demon-
strate the polygonal appearance of the dilated calyces. No
treatment is required unless the condition is complicated
by infection or stone formation, which then may require
specific management.
 ongenital Infundibulopelvic Stenosis
C
Infundibulopelvic stenosis is an exceedingly rare form of
congenital calyceal dilation resulting from stenosis of the
infundibula, which drain to a variably hypoplastic or ste-
nosed renal pelvis. Some authors consider this disorder an
obstructive dysplastic renal disease. It can be unilateral or
bilateral and is often associated with other congenital anom-
alies in the ipsilateral or contralateral kidney (e.g., renal
malrotation, megaureter, multicystic dysplastic kidney, renal
agenesis, and vesicoureteral reflux). Associated malforma-
tions can also involve the skeletal, cardiovascular, gastroin-
testinal, and nervous systems [49].
17 Urinary Tract
a b
c d
Fig. 17.35 Congenital megacalyces in an 11-year-old female with a
neurogenic bladder. Longitudinal grayscale ultrasound images of the
right (a) and left (b) kidneys reveal an increased number of calyces with
an abnormal faceted appearance. The renal pelves (asterisks) are mod-
Ultrasound findings include mild-to-severe calyceal dila-
tion without associated pelvic dilation (Fig. 17.36). MRU or
contrast-enhanced CT will provide more detailed anatomical
information.
There are limited data on the natural history of infundibu-
lopelvic stenosis. However, many cases of unilateral involve-
ment have been observed without evidence of deterioration
in renal function. Surgical correction appears unnecessary
749
e
erately dilated, and the renal parenchyma is diffusely thinned. VCUG
images demonstrate moderate-to-severe reflux into the right-sided (c)
and left-sided (d) renal collecting systems. (e) The neurogenic bladder
has an abnormally thickened, trabeculated wall
in nonprogressive cases, but long-term follow-up and serial
renal function monitoring are required. Intervention should
be considered in cases of progressive calyceal dilation or
renal insufficiency [49]. The goal of surgical intervention is
to offer an opportunity to arrest or reduce progressive renal
insufficiency. Preoperative three-dimensional modeling with
MRU has been used to guide placement of multiple calicocal-
icostomies that drain to a lower pole ureterocalicostomy [49].
750
a c
b d
Fig. 17.36 Congenital infundibulopelvic stenosis in a 16-year-old
female. Longitudinal (a) and transverse (b) grayscale ultrasound images
of the right kidney demonstrate dilated upper pole calyces (arrow-
heads). A small stone (white arrows) with distal shadowing is present in
Calyceal Diverticulum
A calyceal diverticulum is a urine-containing outpouching
within the renal parenchyma that communicates with the
pelvocalyceal system by a narrow neck. Calyceal diverticula
are usually congenital lesions, although some are acquired
following infection or rupture of a simple cyst into the col-
lecting system. There are two recognized types. Type 1 is
the more common form, which is related to a minor calyx
and usually located in the upper pole. Type 2 is less com-
mon, larger, and communicates with the pelvis or a major
calyx in the central portion of the kidney [50].
Calyceal diverticula are usually asymptomatic and found
incidentally on imaging, although they may be complicated
by stone formation and infection. They can sometimes be
G. H. Kassab et al.
the lower dilated calyx. The renal hilum (black arrow) appears normal,
without pelvic dilation. Coronal contrast-enhanced CT images (c, d)
depict the dilated upper pole calyces (arrowheads) and stone (white
arrow). The renal hilum (black arrow) is normal
mistaken for more serious disorders such as a tumor or
abscess [51].
On ultrasound examination, a calyceal diverticulum may
appear similar to a simple cyst. Demonstration of an infun-
dibulum between the cystic lesion and the collecting system
is diagnostic but not always visible (Fig. 17.37). The pres-
ence of mobile echogenic material within a calyceal diver-
ticulum is consistent with milk of calcium and also suggests
the correct diagnosis [52].
No treatment is required for the majority of calyceal diver-
ticula. However, they may rarely cause renal colic, urinary
tract infection, and hematuria, and appropriate treatment will
be required. Prior to surgical intervention, confirmation of
the diagnosis by MRU or contrast-enhanced CT is required.
17 Urinary Tract
a b
Fig. 17.37 Calyceal diverticulum in a 7-year-old male. (a) Longitudinal
grayscale ultrasound image reveals an anechoic cystic structure (cali-
pers) in the interpolar region of the left kidney. (b) Longitudinal color
Doppler ultrasound image demonstrates the avascular nature of the
 ongenital Ureterovesical Junction Obstruction
C 1–3 years. Patients with persistent upper tract dilation gener-
Congenital ureterovesical junction obstruction (UVJO)
results from narrowing and aperistalsis of the distal ureter or
from an ectopic insertion. In “primary megaureter,” the jux-
tavesical ureter may demonstrate muscular hypoplasia and/
or mural fibrosis, whereas the submucosal tunnel and ure-
teral orifice are normal.
Ultrasound findings include variable dilation of the ureter
proximal to the site of narrowing and of the intrarenal col-
lecting system (Fig. 17.38). The lower ureter is often dispro-
portionately dilated compared with the upper ureter and renal
collecting system. Hyperperistalsis of the dilated lower ure-
ter is frequently present, with to-and-fro movement of urine
readily detectable with real-time imaging [53]. Technetium
(Tc)-99m-mercaptoacetyltriglycine (MAG3) diuretic renog-
raphy is used to assess the degree of obstruction.
Most children with congenital UVJO are managed con-
servatively with a high rate of spontaneous resolution after
a b
C
P
C
Fig. 17.38 Ureterovesical junction obstruction in a 6-month-­old female.
(a) Longitudinal grayscale ultrasound image of the left kidney (calipers)
demonstrates moderate dilation of the pelvocalyceal system. P, Renal
pelvis; C, Calyces. (b) Longitudinal grayscale ultrasound image of the
751
c
lesion. (c) Right posterior oblique projection from an intravenous pyelo-
gram obtained 30 minutes after contrast administration demonstrates
contrast filling of the lesion (arrow), thereby proving its connection with
the pelvocalyceal system of the left kidney
ally require long-term follow-up since symptoms can occur
later in childhood as well as in adulthood.
There are no universally accepted guidelines regarding
the appropriate timing of surgical intervention for congeni-
tal UVJ obstruction. A consensus statement published by the
British Association of Paediatric Radiologists in 2014 rec-
ommends surgery when the initial differential renal function
is less than 40%, especially when associated with massive
hydroureteronephrosis; and when there is failure of conser-
vative management (i.e., breakthrough febrile urinary tract
infections pain, worsening dilation, or deteriorating differen-
tial renal function on serial renographic scans).
In patients more than 1 year of age with significant obstru­
ction, ureteral tapering and reimplantation are performed.
This procedure may be challenging in infancy with proposed
alternatives including insertion of a temporary JJ stent or
performance of a refluxing reimplantation [54].
c
B
L
U
pelvis reveals a dilated distal left ureter (U) posterior to the bladder (B).
Voiding cystourethrogram was normal (not shown). (c) MAG3 diuretic
renography post-­void image obtained at 1 hour reveals delayed washout
of radiotracer from the collecting system and ureter. L, Left side
752
B ectopic ureter to the normally located lower moiety ureter
Fig. 17.39 Ectopic ureter in an 8-year-old female. Longitudinal gray-
scale ultrasound image of the pelvis reveals a dilated distal ureter (cali-
pers) that extends below (arrows) the expected location of the ureterovesical
junction. B, Bladder
Ectopic Ureter
Ectopic ureter is a ureter that does not insert on the trigone
of the urinary bladder. Ectopic ureteral insertion results from
caudal, instead of normal cephalic, migration of the ureteral
bud due to failure of separation from the wolffian duct with
the ureteral opening situated inferior to its usual location.
In females, an ectopic ureter may insert anywhere from
the bladder neck to the perineum, including into the vagina,
uterus, or rectum (Fig. 17.39). When an ectopic ureter ter-
minates distal to the bladder neck and external urethral
sphincter, it results in continuous incontinence of urine [31].
In males, an ectopic ureter always inserts above the exter-
nal sphincter or pelvic floor into the lower bladder, semi-
nal vesicle, vas deferens, or ejaculatory duct so that urinary
continence is not affected (Fig. 17.40). However, ectopic
ureters in males are frequently associated with obstructive
symptoms.
Ectopic ureter is associated with a duplex renal collect-
ing system in 70% of cases and is much more common in
girls than boys. According to the Weigert-Meyer rule, in a
duplex system, the ectopic ureter will drain the upper pole
moiety, which inserts inferior and medial to the orifice of the
ureter draining the lower renal moiety. The most commonly
associated anomaly with an ectopic ureter is dysplasia of the
associated renal moiety, with strong correlation between the
degree of ectopia and the degree of renal dysplasia.
Ultrasound imaging is used to determine whether or not a
duplex kidney is present, to document the presence of hydro-
ureter and site of ureteral insertion, and to identify an ectopic
ureterocele [55]. Non-dilated ectopic ureters are difficult to
demonstrate by ultrasound. In these cases, MRU or contrast-­
enhanced CT can often provide this information [56].
The surgical options for treatment of an ectopic ure-
ter include upper pole hemi-nephrectomy in the setting of
G. H. Kassab et al.
severe upper pole hydronephrosis or poor function. If there
is mild hydronephrosis and reasonable function of the upper
pole moiety, ureteroureterostomy with anastomosis of the
with ureteral reimplantation is performed.
Ectopic Ureterocele
An ectopic ureterocele is a cystic dilation of the distal sub-
mucosal portion of an ectopic ureter that protrudes into the
bladder lumen. It is almost invariably associated with a
duplex renal collecting system and represents the termina-
tion of the ureter from the upper renal moiety. An ectopic
ureterocele can be unilateral or bilateral. It is far more com-
mon in girls compared to boys [57]. In cecoureterocele, an
uncommon type of ectopic ureterocele, the intravesical por-
tion dissects submucosally below the trigone with a tongue-­
like projection of the lumen extending beyond the orifice.
This projection may herniate into the urethra and present as
a perineal mass [58].
On ultrasound, an ectopic ureterocele appears as a
round, thin-walled, anechoic intravesical cystic structure
(Fig. 17.26). In the majority of cases, the ureterocele can be
seen to connect with a dilated upper pole moiety of a duplex
renal system draining into a dilated tortuous ureter [59].
Ectopic ureterocele is usually treated by endoscopic inci-
sion and unroofing, after which the ureterocele is collapsed
and appears as an echogenic lesion in the urinary bladder
base. The ectopic ureter associated with the ureterocele is
treated as previously described.
Retrocaval Ureter
Retrocaval ureter, also known as circumcaval ureter,
describes the abnormal course of the proximal ureter pos-
terior to the IVC, after which it emerges to the right of the
aorta, eventually lying anterior to the right iliac vessels. It
results from anomalous development of the IVC, in particu-
lar, failure of development of the right s­ upracardinal system,
and persistence of the right posterior cardinal vein. This
anomaly almost invariably occurs on the right side. Although
retrocaval ureter is usually asymptomatic, it can predispose
to partial right ureteral obstruction or recurrent urinary tract
infections. Ultrasound will show pelvocalyceal and ureteral
dilation down to the level where the ureter crosses behind the
IVC. If symptomatic, this anomaly can be treated by surgical
relocation of the ureter anterior to the IVC [18].
Vesicoureteral Reflux
Vesicoureteral reflux (VUR) is a common abnormality in
children where there is abnormal flow of urine from the blad-
der into the upper urinary tract. The prevalence of VUR is
about 1% and is identified in 30–50% of all children, boys
and girls, presenting with a first urinary tract infection [60]. It
results from an abnormal anti-reflux valve mechanism, usu-
17 Urinary Tract
a d
Fig. 17.40 Ectopic ureter in a 10-year-old male who presented with a
palpable right-sided abdominal mass. (a) Longitudinal grayscale ultra-
sound image reveals an empty right renal fossa. A normal right kidney
was not identified. L, Liver. Transverse (b) and longitudinal extended
field of view (c) grayscale ultrasound images demonstrate a multicystic
structure with an elongated inferior component extending along the right
753
e
U
flank into the pelvis. (d) Coronal contrast-enhanced, T1-weighted, fat-
suppressed, magnetic resonance (MR) image shows a dysplastic, multi-
cystic kidney (arrowhead) and markedly distended ureter (asterisk)
extending into the pelvis where it ends blindly. (e) Intraoperative photo-
graph shows the dilated ureter (U) terminating in an atretic segment that
inserted into the vas deferens
754
ally due to primary immaturity of the vesicoureteral junction
or a short distal ureteric submucosal tunnel in the bladder. It
can occur in isolation or in association with other congenital
urinary tract abnormalities. The majority of VUR cases are
primary, although it can develop secondary to bladder outlet
obstruction, neurogenic bladder, or voiding dysfunction.
Children with VUR are at increased risk of renal infec-
tion (pyelonephritis) and postinfectious renal scarring due
to intra-renal reflux of infected urine; this risk is especially
high in neonates and infants. Renal scars are of prognostic
importance, as they are associated with the long-term devel-
opment of hypertension and deterioration of renal function.
Therefore, early detection of VUR is crucial to allow prompt
prophylactic antibiotic treatment to decrease the risk of renal
scarring [61].
a b
c d
Fig. 17.41 Bilateral vesicoureteral reflux in a 2-month-old male with
urosepsis. Longitudinal grayscale ultrasound images of the right (a) and
left (b) kidneys demonstrate moderate-to-severe bilateral hydronephro-
sis and proximal hydroureter. Echogenic material representing purulent
debris (arrowheads) is identified in both renal pelves. (c) Transverse
G. H. Kassab et al.
Grayscale ultrasound is useful in imaging of patients with
VUR for evaluation of renal size, thickness, and appearance
of the renal parenchyma, presence of collecting system and
ureteral dilation, bladder wall thickness, and pre-void and
post-void bladder volume in children who are toilet-trained
(Fig. 17.41). Congenital malformations of the kidneys and
bladder can also be identified [62].
In some patients with VUR, intermittent dilation of the
collecting system and ureter is seen. Patients born with
severe VUR, such as infant males with the so-called mega-
cystis-megaureter syndrome, will have a large, thin-walled
bladder, massive bilateral hydroureteronephrosis, and small
dysplastic-­appearing kidneys [63]. In the majority of patients
with VUR, however, the kidneys will appear normal. It is
important to recognize that a normal ultrasound study does
grayscale ultrasound image of the pelvis demonstrates dilated distal
ureters (arrowheads). Echogenic debris in the distal ureters and bladder
(B) is identified, as well as multiple short, linear echogenic foci thought
to represent air from recent bladder catheterization. (d) VCUG image
demonstrates bilateral severe vesicoureteral reflux
17 Urinary Tract
not exclude the presence of VUR. Further evaluation of the
urinary tract with a voiding cystourethrogram (VCUG) may
be indicated in selected patients, regardless of the ultrasound
findings.
Contrast-Enhanced Ultrasound Diagnosis
of Vesicoureteral Reflux
Contrast-enhanced voiding urosonography (CeVUS) is now
widely accepted as an effective imaging technique for the inves-
tigation and management of vesicoureteral reflux (VUR). In
addition to its enhanced safety profile and the complete lack of
ionizing radiation, CeVUS is more sensitive and detects higher
grades of reflux compared with VCUG [64].
The bladder is catheterized, and a solution of ultrasound
microbubbles in normal saline is infused under gravity while
sequential imaging of the bladder and kidneys is performed
during bladder filling and voiding (Fig. 17.42). Transperineal
images of the urethra are obtained during voiding [65]. Static
images, as well as cineclips, are used to document the exami-
nation. The diagnosis of VUR is based on detecting retro-
grade passage of the microbubbles to the ureter and/or renal
pelvis from the urinary bladder. Intra-renal reflux appears as
multiple punctate hyperechoic foci more commonly found
in the polar regions of the kidney or as diffuse enhancement
of the renal parenchyma [64, 66]. The severity of VUR can
be graded using an I-V category scale comparable to the
International Grading System used for conventional fluoro-
scopic VCUG [65].
In most children, VUR will ultimately resolve, and treat-
ment is conservative, with prophylactic antibiotics and serial
follow-up ultrasound studies and cystography. Patients with
bladder and bowel dysfunction are identified and treated
appropriately. Surgery is generally reserved for patients with
Grade IV or V reflux that persists beyond 2 or 3 years of
age, those who fail medical therapy with breakthrough infec-
a b
B B
Fig. 17.42 Vesicoureteral reflux in a 4-year-old male depicted by
ceVUS. Transverse (a) and longitudinal (b) images from a ceVUS study
show reflux (white arrowheads) into the distal right ureter. Shadowing
from an intravesical catheter (black arrowhead) is noted. B, Bladder. (c)
755
tions, children with significant side effects from continuous
prophylactic antibiotics, patient noncompliance with either
antibiotic prophylaxis or failed follow-up after a febrile ill-
ness, and family preference.
Imaging of Endoscopically Placed Bulking Agents
Endoscopic submucosal, subureteric injection of bulking agents
is an accepted treatment method for VUR, first popularized in
the 1980s as the STING (subureteric transurethral injection) pro-
cedure. The most commonly used injectable material is Deflux,
a dextranomer-hyaluronic acid copolymer which is biodegrad-
able, with volume stability and a relatively large particle size
that prevents distant migration. Other previously used materials
such as Teflon and silicone are no longer recommended due to
the risk of distant migration and granuloma formation.
The bulking agent is injected submucosally under cystoscopic
guidance into the intravesical portion of the ureter in order to
narrow the ureteric orifice, thereby preventing reflux. Familiarity
with the characteristic imaging appearance of bulking agents
will prevent misdiagnosis and unnecessary interventions [67].
On ultrasound, the bulking agent appears as a focal,
round, or oval isoechoic or hyperechoic mound in the tri-
gonal region of the posterior bladder wall that may protrude
into the bladder lumen (Fig. 17.43). The mound may calcify
and display posterior shadowing, occasionally simulating a
distal ureteral stone [68].
Anomalies of the Bladder
Urachal Anomalies
The urachus is the fibrous vestigial remnant of the allantois, a
connection between the urinary bladder and the umbilical cord
during fetal life. The urachus is located in the retropubic space
and anterior to the peritoneum in the space of Retzius. Before
c d
Longitudinal ceVUS image of the right flank shows grade II–III reflux
of contrast material into the renal collecting system and proximal ureter.
(d) Image from VCUG performed 1 year earlier demonstrates a similar
degree of right-sided reflux
756 G. H. Kassab et al.

a b

Fig. 17.43 Bilateral subureteric Deflux mounds used to treat reflux in isks) in the trigonal region of the posterior bladder wall. (b) Longitudinal
a 3-year-old female. (a) Transverse grayscale ultrasound image of the grayscale ultrasound image shows the left-sided mound protruding into
bladder shows bilateral ovoid, echogenic subureteric mounds (aster- the bladder lumen

a b

Fig. 17.44 Urachal remnant in a 5-year-old male. (a) Longitudinal and (b) transverse grayscale images demonstrate an ovoid, hypoechoic struc-
ture (arrows) in the anterosuperior wall of the urinary bladder

birth, the lumen of the urachus usually obliterates and forms
the median umbilical ligament, a midline parietal peritoneal
fold, extending from the apex of the bladder to the umbilicus.
A normal urachal remnant is frequently seen as a small
hypoechoic structure superior to the bladder on routine uri-
nary tract ultrasound in children (Fig. 17.44) [69].
If the urachal lumen is not completely obliterated, a
spectrum of urachal remnants may persist, including patent
urachus, urachal sinus, vesicourachal diverticulum, umbili-
courachal sinus, and urachal cyst (Fig. 17.45) [70].
Congenital urachal remnant abnormalities frequently
coexist with congenital lower urinary tract obstruction
such as posterior urethral valves or prune-belly syndrome,
and ventral abdominal wall defects such as omphalocele
[55].
Surgical excision has historically been the standard
approach to treating urachal anomalies owing to concern for
infection and the rare chance of developing a urachal malig-
nancy later in life. However, there is no evidence demon-
strating that urachal anomalies in pediatric patients increase
the likelihood of future urachal malignancy. In recent years,
nonoperative management of symptomatic patients has
gained increasing recognition as a reasonable alternative to
surgical treatment [71, 72].
17 Urinary Tract 757

Patent urachus Urachal

sinus

Anterior
Bladder
abdominal
wall

Urachal
cyst

Urachal
diverticulum

Fig. 17.45 Diagram of urachal anomalies

a b c

U B
B
U

Fig. 17.46 Patent urachus in a 2-day-old female with urine leakage Transverse grayscale ultrasound image shows a large amount of fluid in
from the umbilicus. (a) Longitudinal grayscale ultrasound image of the the center of the umbilicus (asterisk). (c) Lateral image of the bladder
bladder (B) reveals a tubular structure (arrowheads) containing anechoic (B) at VCUG shows contrast filling a patent urachus (arrowheads) and
urine extending from the bladder dome toward the umbilicus (U). (b) draining anteriorly through the umbilicus (U)

Patent Urachus Vesicourachal Diverticulum

A patent urachus is characterized by a persistent connection
between the bladder and the umbilicus, presenting with urine
leakage from the umbilicus.
On ultrasound imaging, a patent urachus appears as a
tubular structure extending between the anterosuperior
aspect of the bladder and the umbilicus (Fig. 17.46) [73, 74].
A vesicourachal diverticulum develops when the distal end
of the urachus fails to close, resulting in an outpouching
from the anterosuperior aspect of the bladder (Fig. 17.47).
This anomaly is usually asymptomatic and identified inci-
dentally as a urine-filled diverticulum extending from the
bladder dome [73, 74].
758
U
Fig. 17.47 Vesicourachal diverticulum in a 12-year-old male. Sagittal
grayscale ultrasound image of the bladder (B) demonstrates a complex
cystic structure (asterisk) at the bladder dome that extends superiorly
and is in continuity (arrow) with the bladder lumen. U, Umbilicus
Umbilicourachal Sinus
An umbilicourachal sinus results when the umbilical end of
the urachus persists while the bladder end obliterates, result-
ing in a blind-ending umbilical tract. These lesions may be
asymptomatic or can present with infection manifesting as
abdominal pain and drainage of fluid. On ultrasound imag-
ing, a thickened and fusiform dilation of the urachus at the
umbilical end is identified, which does not communicate
with the bladder [73, 74].
Urachal Cyst
A urachal cyst occurs when there is obliteration of both
the proximal and distal ends of the urachal lumen with
persistence of a fluid-filled intervening portion. Urachal
cysts are asymptomatic unless complicated by infection
or bleeding.
On ultrasound, a urachal cyst appears as a simple cys-
tic structure in the midline of the anterior abdominal wall
between the umbilicus and the bladder dome. An infected
urachal cyst (Fig. 17.48) will demonstrate echogenic con-
tents and a thickened wall [73, 74].
Bladder Diverticula
Bladder diverticula may be congenital (primary) or acquired
(secondary). Congenital bladder diverticula are noted inci-
dentally in 2% of children, usually males [55]. They can
be unilateral or bilateral and consist of herniated bladder
mucosa through fibers of the detrusor muscle.
They have also been described in children with connec-
tive tissue disorders such as Ehlers-Danlos syndrome [75].
Secondary bladder diverticula are usually related to bladder
G. H. Kassab et al.
B
Fig. 17.48 Infected urachal cyst in a 14-year-old female. Longitudinal
grayscale ultrasound image demonstrates a heterogeneous fluid collec-
tion (arrows) superior to the dome of the bladder (B) with posterior
acoustic enhancement (arrowheads). The bladder contains a moderate
amount of echogenic debris (asterisk)
outlet obstruction, as with neurogenic bladder or posterior
urethral valves.
Diverticula are commonly identified at the ureterovesical
junction where they are often associated with VUR because they
alter the normal oblique insertion of the ureter into the bladder
[76]. It is important to differentiate between bladder diverticula
and bladder ears in male infants. As previously discussed, blad-
der ears are lateral outpouchings of the urinary bladder into the
inguinal canals that resolve with age. Lateral imaging at VCUG
usually helps to distinguish between these two conditions.
On ultrasound, a bladder diverticulum appears as a round
or oval anechoic fluid collection, often arising from the blad-
der base or adjacent to the ureteral orifice (Fig. 17.49). VCUG
is the reference standard technique for identification of blad-
der diverticula. Surgical excision of a bladder diverticulum is
only indicated if it is associated with infection, obstruction, or
VUR. Recurrence after surgery is common [75].
Bladder Exstrophy
Bladder exstrophy is a rare congenital malformation charac-
terized by an infraumbilical abdominal wall defect, incom-
plete bladder closure with mucosal continuity with the
anterior abdominal wall, epispadias, and associated abnor-
malities of the pelvic bones and musculature. This malfor-
mation is one part of the exstrophy-epispadias complex,
with epispadias at the mild end of the spectrum and cloacal
exstrophy at the severe end. Bladder exstrophy is the most
common of these abnormalities and usually occurs in boys.
Clinical findings of bladder exstrophy include bladder ever-
sion, epispadias, wide diastasis of the pubic bones, and anterior
displacement of the anus secondary to muscular deficiency.
In males, the penis is short with wide separation of the cor-
poral attachments and a dorsal curvature. In girls, there is a
bifid clitoris. The ureters insert low in the bladder with a typical
17 Urinary Tract
a b
Fig. 17.49 Bladder diverticula in a 1-year-old female. (a) Transverse grayscale ultrasound image reveals two rounded fluid collections (asterisks)
arising from the bladder (B). (b) The presence of diverticula (arrowheads) is confirmed on VCUG. B, Bladder
J-shaped course due to an enlarged recto-uterine pouch with
inferolateral displacement of the ureters. All patients with blad-
der exstrophy will develop VUR after exstrophy closure, so
ureteral reimplantation is performed at the time of repair [77].
Modern staged repair of bladder exstrophy is a three-step
process in males and a two-step process in females. In stage
I, the bladder and abdominal wall defects are closed in both
sexes 2–3 days after birth, with epispadias repair only in
females. Iliac osteotomies may be performed at this time if
the pubic diastasis is greater than 4 cm or if the malleability
of the pelvis is poor. In stage II, the male urethra is closed,
usually between 6 and 12 months of age. In stage III, blad-
der neck reconstruction and bilateral ureteral reimplantation
are performed in males and females when the child can take
part in a voiding program, generally at 4–5 years of age [78].
After repair, ultrasound imaging will show a small blad-
der with irregular contours and a thickened wall (Fig. 17.50).
The kidneys usually appear normal, although hydronephro-
sis may occur as a result of vesicoureteral reflux, bladder
outlet obstruction, or urethral stricture. Renal scarring can
develop from obstructive uropathy after bladder neck recon-
struction or recurrent pyelonephritis.
Bladder augmentation using a segment of bowel is often
performed in children who cannot undergo bladder neck
reconstruction because of a low bladder capacity, abnormal
bladder compliance, or incontinence. Ileocystoplasty is the
usual choice, although other bowel segments can be used.
The augmented bladder segment with an undulating con-
tour is identified by ultrasound above the native bladder. The
characteristic “gut signature” of the augmented segment is
usually evident (Fig. 17.51) [79]. Echogenic intraluminal
mucus or debris is frequently noted.
759
Cloacal Exstrophy
Cloacal exstrophy is the most severe manifestation of the
exstrophy-epispadias complex where there is abnormal embry-
ological development of the cloacal membrane. In contrast to
bladder exstrophy where normal separation of the bladder and
hindgut occurs, in cloacal exstrophy the urorectal septum fails
to form. It is a multisystem anomaly that is often referred to as
the OEIS complex (omphalocele-­exstrophy-imperforate anus-
spinal defects) and includes other malformations of the gas-
trointestinal, urogenital, and ­skeletal systems, and neurospinal
axis. Males and females are equally affected.
The classic presentation of cloacal exstrophy includes
two exstrophied hemi-bladders separated by a segment of
exstrophied cecum, and usually accompanied by a segment
of prolapsed ileum. The hindgut is shortened and often
blind-ending, with an imperforate anus. A split phallus is
present with one half on either side of a widened pubic
diastasis [80].
Immediately after birth and stabilization of the newborn, the
exposed organs and mucosal surfaces are protected by moisten-
ing surfaces with saline and covering with sterile plastic wrapping
or by enclosing the lower torso in a bowel bag. These measures
help to prevent evaporative losses, trauma, and infection.
Initial imaging of cloacal exstrophy includes ultrasound
evaluation of the kidneys, since abnormalities of the upper uri-
nary tract have been reported in 41–66% of patients, includ-
ing hydronephrosis, pelvic kidney, horseshoe kidney, fusion
anomalies, and unilateral renal agenesis [81].
The surgical management of cloacal exstrophy includes
pelvic osteotomies and immobilization, bladder and abdomi-
nal wall closure, ureteral reimplantation, and usually aug-
mentation cystoplasty with continent urinary diversion.
760
a b
Fig. 17.50 Repaired bladder exstrophy in a 12-week-old female. (a)
Transverse grayscale ultrasound image of the bladder obtained soon
after complete primary repair shows mural thickening and irregularity
B to establish the connections between the urethra, vagina(s), rec-
Fig. 17.51 Ileocystoplasty in a 20-year-old male with bladder exstro-
phy. Longitudinal grayscale ultrasound image shows the bladder (B) with
the augmented segment (asterisk) located superiorly. The augmented seg-
ment has an undulating contour with an inner hyperechoic mucosal layer
(arrowhead) and an outer hypoechoic muscular layer (arrow)
Cloacal Malformation
Cloacal malformations are characterized by a single perineal
orifice and confluence of the distal portions of the urinary,
genital, and gastrointestinal tracts. They represent the most
G. H. Kassab et al.
(arrowheads). (b) Image from a postoperative VCUG shows a small
capacity bladder with a mildly lobulated contour. There is significant
symphyseal diastasis (asterisks)
complex end of the spectrum of female anorectal malforma-
tions and are rare, occurring in only 1 of 50,000 live births.
On physical examination, there is a single perineal orifice and
no anal opening [82]. There is frequently failure of mullerian
duct fusion with duplication of the uterus and proximal vagina,
a persistent urogenital sinus and abnormal lower vagina and
hymen. At birth, patients undergo immediate colostomy and cre-
ation of a mucous fistula to decompress the gastrointestinal tract.
The role of imaging in patients with cloacal malformation is
tum, and common channel and to determine their relationship to
the spine [83]. This information will help to determine the surgi-
cal approach to repair. When the cloaca is mainly located below
the tip of the spine, a posterior sagittal approach can be used
rather than a combined anterior and posterior sagittal approach.
Ultrasound evaluation of the pelvis and kidneys is performed
in the early management of patients with a cloacal malforma-
tion in order to determine whether significant hydrocolpos is
present and if it is associated with hydronephrosis, which will
determine the need for vaginal drainage (Fig. 17.52). Other
imaging techniques, including fluoroscopy, three-dimensional
(3D) CT, and MR imaging are currently used to further charac-
terize the various anatomical abnormalities.
Recently, contrast-enhanced genitosonography (ceGS)
has been described as a potentially useful, nonionizing radi-
ation technique similar to conventional genitography that
provides superb anatomical detail of the opacified organs
(Fig. 17.53) [84].
17 Urinary Tract 761

a b

R
V V

V B V

Fig. 17.52 Cloacal malformation in a 4-day-old female. (a) Transverse in the bladder (B). Refluxed contrast material is present in each vagina
grayscale ultrasound image of the pelvis reveals duplicated vaginas (V) (V) and in the rectum (R) that joins the cloaca between the 2 vaginas
massively dilated with fluid. (b) VCUG image shows a catheter (arrow) (arrowheads)

The long-term functional outcome of females with the

cloacal malformation depends on the position of the anus
and the length of the cloaca, with a shorter cloaca (<3 cm)
and more normally positioned anus being associated with
better surgical outcomes [82].

Bladder Duplication
Bladder duplication is a rare anomaly. Duplication can
be incomplete, where the bladder is either completely or C B
incompletely separated in the coronal or sagittal planes
with a single urethra, or complete, with duplication of both
the bladder and urethra. Sagittal duplication is more com-
mon, with the two bladders lying side by side with each
bladder receiving the ureter of the ipsilateral kidney. With
coronal duplication, the two bladders are located one in V V
front of the other and separated by a fibromuscular septum.
On ultrasound, there are two separate bladders side by
side (sagittal duplication) or in front of each other (coronal
duplication), each with its own muscular wall (Fig. 17.54).
Further delineation of the anatomical abnormalities can be
demonstrated by VCUG after instillation of contrast material Fig. 17.53 Cloacal malformation in a 9-month-old female evaluated
by contrast-enhanced genitosonography (ceGS). Longitudinal transab-
into the urethral opening(s). dominal image demonstrates the colon (C) inserting into the septum
In patients with a sagittal septum, duplication of the exter- (arrowheads) between the two vaginas (V). B, Bladder. The distal spine
nal genitalia occurs in 30%; caudal gastrointestinal tract anom- (arrows) is identified posteriorly
762
a b
Fig. 17.54 Bladder duplication in a 1-day-old female. (a) Transverse
grayscale ultrasound image of the bladder demonstrates a thick septum
(arrow) arising from the posterior wall and extending anteriorly. (b) VCUG
alies including stenosis, atresia, and ectopic rectum occur in
40%; duplication of the spine and meningocele in 10%; and
fistulas between the bladder, rectum, vagina, and urethra can
also occur [85]. In patients with a coronal septum, anomalies
outside the urogenital system are less c­ ommon and urogeni-
tal anomalies more frequent, including ectopic kidney and
VUR [86].
Bladder Agenesis
Agenesis of the bladder is an extremely rare congenital anom-
aly with fewer than 70 cases reported in the literature. The vast
majority of affected individuals are female. The cause of bladder
agenesis is unknown. Lack of assimilation of the mesonephric
ducts and ureters into the trigone, which prevents urine filling
and distension of the bladder, may lead to loss of the anterior
division of cloaca (the urogenital sinus) and possibly result in
agenesis of the bladder. Posterior division of the cloaca (ano-
rectum) is usually unaffected, and the hindgut is therefore nor-
mal in these patients [87].
Ultrasound can demonstrate absence of the bladder and
depict the severe bilateral renal dysplasia that is usually asso-
ciated with this condition (Fig. 17.55).
Bladder agenesis is frequently associated with other severe
genitourinary, neurological, and musculoskeletal anomalies.
This anomaly is only compatible with life if the ureters drain
ectopically into normally developed müllerian structures, such
G. H. Kassab et al.
confirms the presence of incomplete sagittal bladder duplication. The blad-
ders communicate inferiorly (asterisk) and drain into a single urethra. Arrow,
Urethral catheter
as the uterus, vagina, or vestibule in females, or the rectum or
a patent urachus in males. Bladder agenesis carries a very poor
prognosis, and most infants with this anomaly are stillborn [88].
Prune-Belly Syndrome
Prune-belly syndrome is a rare, multisystemic condition
typically characterized by deficient or absent abdominal wall
musculature, bilateral intra-abdominal cryptorchid testes and
urinary tract anomalies, including renal dysplasia, hydroure-
teronephrosis, megacystis, and megalourethra. The degree of
renal dysplasia exists along a broad continuum, and many
patients also have many associated non-genitourinary abnor-
malities, including cardiopulmonary, gastrointestinal, and
musculoskeletal anomalies.
While the etiology of prune-­belly syndrome is unknown,
there appears to be a genetic component. There are two theo-
ries regarding pathogenesis. The first theory postulates a
mesenchymal defect, while the second hypothesizes that a
hypoplastic or dysplastic prostate or abnormal urethra pre-
vents the outflow of urine, leading to proximal bladder, ure-
teral, and renal dilation with secondary poor development
of the abdominal wall and urinary tract musculature [89].
Prune-belly syndrome occurs almost exclusively in males,
with only a few reported cases of affected females. At birth,
the appearance of the abdominal wall usually suggests the
diagnosis of prune-belly syndrome.
17 Urinary Tract
a b
Fig. 17.55 Bladder agenesis in a 2-year-old female. (a) Transverse
grayscale ultrasound image of the pelvis reveals absence of the bladder.
Longitudinal grayscale ultrasound images of the right (b) and left (c)
Fig. 17.56 Prune-belly syndrome in a 4-month-old male. Longitudinal
grayscale ultrasound images of the right (a) and left (b) kidneys show
dilated renal pelves (P) and proximal ureters (U). (c) Transverse gray-
Ultrasound is used to assess the kidneys for dysplas-
tic changes and to document hydroureteronephrosis. A
patent urachus and dilated prostatic urethra are common
findings. Further evaluation of the bladder and urethra, as
well as documentation of VUR, is performed with VCUG
[90]. MRU is useful in providing both functional and ana-
tomical information and allows for detailed characteriza-
tion of upper tract abnormalities. There is usually some
degree of bilateral hydroureteronephrosis (Fig. 17.56).
Other frequently seen abnormalities include renal dyspla-
sia, scarring, and/or calyceal diverticula. The abdominal
wall musculature and location of the testes can also be
assessed [91].
The postnatal course of patients with prune-belly syn-
drome is determined by the severity of any co-­morbidities
such as pulmonary hypoplasia. Perinatal mortality rates are
high, ranging between 10% and 25%, with early demise
largely attributable to the degree of prematurity and pulmo-
nary hypoplasia.
There are no guidelines or consensus regarding the man-
agement of children with prune-­ belly syndrome, largely
related to the rarity of the condition, wide spectrum of
abnormalities and manifestations of the syndrome. Repair of
cryptorchidism, abdominal wall flaccidity, and amenable uri-
nary tract anomalies is performed in most affected children.
763
c
kidneys demonstrate bilateral dysplasia with echogenic parenchyma,
loss of corticomedullary differentiation, and left-sided cysts
scale ultrasound image of the pelvis demonstrates the dilated distal left
ureter (U) behind the smooth-walled bladder (B)
Approximately 15% of patients ultimately require renal
transplantation [89, 91].
Megacystis-Microcolon-Intestinal
Hypoperistalsis Syndrome
Megacystitis-microcolon-intestinal hypoperistalsis syndrome
is a disorder of smooth muscle function affecting the genito-
urinary and gastrointestinal tracts. It is inherited in an autoso-
mal recessive fashion and is much more common in females,
with fewer than 30% of reported cases occurring in males
[92]. It is one of several conditions caused by mutations in
the ACTG2 gene that arises either as a result of a de novo
mutation or is inherited in an autosomal dominant fashion.
Patients usually present in the neonatal period with an inabil-
ity to void or abdominal distension. Symptoms may mimic
other more common neonatal disorders such as bladder outlet
obstruction or proximal bowel obstruction, which can result
in a delay in diagnosis.
The most common imaging findings include hydroure-
teronephrosis, megacystitis, microcolon, and small bowel
dilation related to hypoperistalsis. The urinary tract abnor-
malities are well-depicted by ultrasound [93, 94]. Multiorgan
transplantation is currently the only intervention that has
been somewhat effective in prolonging the lives of these
patients [95].
764
Anomalies of the Urethra
Posterior Urethral Valves
Posterior urethral valves (PUV) are the most frequent cause
of bladder outlet obstruction in children, with an incidence
of approximately 1:7000–8000 live births [96]. Typically,
there is a membrane extending from the verumontanum that
attaches distally in the posterior urethra in males, resulting in
a valve-like obstruction to the outflow of urine that is associ-
ated with deleterious effects on the urinary bladder and the
upper urinary tracts. PUV usually occurs as an isolated dis-
order, although it can be associated with many other con-
genital urological anomalies, including UPJO, crossed renal
ectopia, hypospadias, and prune-belly syndrome [97].
PUV usually manifests as hydronephrosis during routine
prenatal ultrasound, with a definitive diagnosis made after
birth [98]. Patients with PUV that are not detected prenatally
Fig. 17.57 Posterior urethral valves (PUV) in a 3-day-old male. Lon­
gitudinal grayscale ultrasound images of the right (a) and left (b) kidneys
demonstrate dilation of the pelvocalyceal systems and right proximal ureter
(U). P, Renal pelvis; C, calyx. (c) Transverse grayscale ultrasound image
G. H. Kassab et al.
may present with a poor urinary stream, urinary tract infec-
tion, or failure to thrive. Older children present with voiding
dysfunction, infection, or hematuria.
Ultrasound is used to assess renal parenchymal thick-
ness, echogenicity, and dysplastic changes such as cysts;
the degree of hydronephrosis and hydroureter; and bladder
wall thickening (Fig. 17.57). Good corticomedullary dif-
ferentiation is a reassuring sign in terms of renal function,
while increased renal parenchymal echogenicity and sub-
cortical cysts are poor prognostic signs for renal function
(Fig. 17.58).
A thick-walled bladder may indicate poor compliance that
can eventually lead to upper tract damage. A thick-walled blad-
der also tends to physically obstruct the distal ureters as they
fill [99]. Occasionally, the high pressures generated during
attempted voiding results in forniceal rupture and the develop-
ment of a subcapsular or perirenal urinoma, or urinary ascites.
of the bladder (B) depicts severe wall thickening with dilation of the distal
ureters (arrows). (d) VCUG image demonstrates marked dilation of the
posterior urethra (PU). Arrow indicates the site of the valves
17 Urinary Tract
a b
C
P
Fig. 17.58 Dysplastic renal parenchymal changes in a 1-month-old
male with PUV. Longitudinal grayscale ultrasound images of the right
(a) and left (b) kidneys show bilaterally echogenic renal parenchyma.
VCUG is the traditional imaging modality of choice for
the diagnosis of PUV. Findings include dilation and elon-
gation of the posterior urethra, a thick-walled, trabeculated
bladder, and a dilated posterior urethra. Unilateral VUR
may occur in up to 35% of boys with PUV and is associ-
ated with protected renal function [100].
The dilated posterior urethra can sometimes be identified
on conventional grayscale ultrasound imaging via a trans-
abdominal or transperineal approach, particularly when the
patient is voiding, which results in proximal urethral dila-
tion. The abnormal valve tissue is occasionally identified
as a linear echogenic focus in the inferior portion of the
dilated posterior urethra.
As discussed earlier, ceVUS is a useful, radiation-free
alternative to VCUG for evaluation of the pediatric urinary
tract [101]. PUV are well-depicted on ceVUS, where a cali-
ber change is identified in the urethra with proximal ure-
thral dilation (Fig. 17.59), comparable to the findings seen
on VCUG [102].
Management of PUV consists of relieving the obstruc-
tion and pressure on the urinary tract while maintaining nor-
mal bladder and renal function for as long as possible. This
usually consists of catheter drainage of the bladder at birth,
with monitoring of serum electrolytes and renal function,
and antibiotics to prevent UTI. Patients who improve with
catheter drainage undergo endoscopic ablation of the valves
unless they weigh less than 2000 g, in which case a vesi-
costomy is performed to alleviate the obstruction until the
child is large enough for definitive treatment. There may also
be an advantage to vesicostomy in cases of severe reflux, as
bladder pressure is maximally reduced after this procedure.
If, however, hydronephrosis and renal function worsen, an
upper tract diversion may be warranted [97].
765
C
P
The renal pelvocalyceal systems are dilated, and peripheral cysts
(arrows) are noted on each side. C, Calyx; P, pelvis
Anterior Urethral Valves
Anterior urethral valves are a rare congenital anomaly caus-
ing lower urinary tract obstruction in children, which can
occur as an isolated entity or in association with a proximal
diverticulum. An anterior urethral valve is a fold in the ure-
thral mucosa that is typically located along the ventral sur-
face. Obstruction results as the valve or fold rises and flattens
against the dorsal surface of the urethra during voiding. Most
valves have a semilunar appearance on endoscopy (70%),
while the remaining 30% resemble an iris [103].
The location of the anterior urethral valve is variable,
with 40% in the bulbar urethra, 30% at the penoscrotal junc-
tion, and 30% in the penile urethra. Dribbling of urine, poor
urinary stream, and UTI or urosepsis are the more com-
mon features at presentation. Occasionally, there may be a
penile mass that represents the dilated proximal urethra or
diverticulum.
Typical features of obstructive uropathy can be demon-
strated on ultrasound imaging of the kidneys and bladder,
although they are nonspecific. Longitudinal transpenile
ultrasound has been used to identify the anterior valve tissue
[104]. VCUG is the most widely accepted imaging technique
for the diagnosis of anterior urethral valves. Typically, the
urethra appears dilated proximal to the valve and narrowed
distally with a linear filling defect along the ventral wall rep-
resenting the valve. VCUG can also identify VUR or other
associated anomalies.
The methods of treatment for anterior urethral valves
vary, depending on the presence of a diverticulum and other
anomalies. The treatment of choice for isolated anterior ure-
thral valves is endoscopic valve ablation. In the presence of a
small diverticulum with adequate penile spongiosum, trans-
urethral resection is the favored treatment method. If there
766
a b
Fig. 17.59 PUV demonstrated by ceVUS in a 6-year-old male. (a)
Sagittal transperineal ultrasound image obtained during voiding shows
dilation of the posterior urethra (U) with only a tiny amount of contrast
in the more distal urethra (arrows). B, Bladder. (b) Image obtained dur-
is a large diverticulum with no adjacent supportive tissues,
open diverticulectomy with primary repair is favored [103].
Urethral Duplication
Urethral duplication is a rare anomaly of the lower uri-
nary tract with many anatomical variants described in the
literature. Duplications are either sagittal or coronal in ori-
entation. Sagittal urethral duplications can be separated into
pre-pubic congenital sinus, epispadiac urethral duplication,
and hypospadiac urethral duplication. Although the embryo-
logical basis for urethral duplication is unknown, similarities
among patients in each group suggest that different distur-
bances at distinct moments of urinary tract embryogenesis
likely explain the varying abnormalities.
Most cases of duplicated urethra are sagittal in orienta-
tion. The ventral urethra is usually the more functional of
the two urethras and contains the sphincteric mechanism.
Coronal duplications usually occur in association with
bladder duplication [105]. Urethral duplication can be par-
tial or complete, ending in one meatus or two, and can be
G. H. Kassab et al.
ing voiding phase of VCUG shows marked focal narrowing of the ure-
thra at the site of the valves (arrow) with dilation of the posterior urethra
(arrowhead)
associated with a perineal or rectal fistula (Y-type duplica-
tion) [106].
Clinical examination, VCUG, and urethrocystoscopy
are required for the diagnosis of urethral duplication, while
the role of ultrasound is limited. Surgical repair of urethral
duplication must be individualized according to the anatomi-
cal findings in every patient [106].
Acquired Ureteral Obstruction
Acquired ureteral obstruction can be due to intraluminal
obstruction or extrinsic compression.
Intraluminal Obstruction
Stones, blood clots, sloughed papillae, or benign polyps are
the main causes of intrinsic ureteral obstruction. On ultra-
sound, these appear as an echogenic filling defect in a dilated
17 Urinary Tract
ureter (Fig. 17.60). Stones will be associated with posterior
acoustic shadowing, while blood clot and sloughed papilla
will not; a polyp may show internal vascularity. Assessing
the ureteral jet with color Doppler can also be helpful in
the detection and qualitative assessment of obstruction. An
absent ureteral jet or continuous low-level jets are seen in
high-grade obstruction on the symptomatic side, whereas in
low-grade obstruction, the ureteral jet is normal or mildly
diminished [107].
Extrinsic Compression
Extrinsic compression of the ureter can result from an adja-
cent inflammatory process such as an abscess or phlegmon
complicating acute appendicitis or Crohn disease, or from
mass effect caused by a retroperitoneal tumor or enlarged
lymph node [108].
P in patients who perform clean intermittent bladder cath-
U capacity or high-pressure bladders. A bowel segment is
Fig. 17.60 Ureteral stone in a 9-year-old male. Oblique grayscale
ultrasound image of the right flank shows moderate dilation of the renal
pelvis (P) and proximal ureter (U) with a mid-ureteral echogenic focus
(arrow) associated with posterior shadowing
a b
B
Fig. 17.61 Neurogenic bladder in a 17-month-old female. Transverse
(a) and left parasagittal (b) grayscale ultrasound images depict an elon-
gated, irregular, and thick-walled bladder (B) with a left-sided diverticu-
767
Neurogenic Bladder
Neurogenic bladder is the term applied to a dysfunctional
urinary bladder where the detrusor muscle and the internal
and external sphincters are not working in co-ordination to
allow urine storage and elimination. In children, the majority
of cases are congenital due to a meningocele or myelome-
ningocele. Less commonly, it results from an injury to the
central or peripheral nerves in the setting of trauma, vascu-
lar insult, encephalitis, or meningitis. Symptoms can include
overflow incontinence, frequency, urgency, urge incontinence,
and urinary retention. Complications include recurrent infec-
tion, VUR, and autonomic dysreflexia.
The ultrasound appearance of the bladder depends
on the level of the lesion. If lower motor neurons are
damaged, the urinary bladder is smooth and thin walled
with an increased capacity. With an upper motor neuron
lesion, the bladder is trabeculated and thick walled, with
a small capacity (Fig. 17.61). Ultrasound can also dem-
onstrate distal ureteral dilation, incomplete bladder emp-
tying, mucosal changes related to infection, and bladder
stones. Any child with a neurogenic bladder is predis-
posed to stone formation, although they occur most often
eterization [109].
Bladder augmentation is a surgical procedure that is
performed to prevent renal damage in children with small-­
detubularized and anastomosed to the urinary bladder
to increase its capacity. Ultrasound imaging will often
show the gut signature of the bowel wall. Intraluminal
debris related to mucus production is frequently identi-
fied [79, 110].
c
B
lum (asterisks). Both distal ureters (arrowheads) are moderately dilated.
(c) Left anterior oblique image from a VCUG shows the vertically ori-
ented bladder with trabeculated wall and left-sided diverticulum (arrow)
768 G. H. Kassab et al.

Urinary Tract Infection
Urinary tract infection (UTI) is common in children and a fre-
quent indication for renal ultrasound examination. Despite its
prevalence, the proper management approach to a child with
UTI is still controversial. In recent years, a number of medi-
cal organizations concerned with the diagnosis and treatment
of children with UTI have issued management guidelines that
differ with respect to their recommendations, particularly in
the realm of imaging evaluation (Table 17.1) [111].
Imaging of children with UTI is generally initiated after
a first urine culture has documented infection in a child less
than 3 years of age. The purpose of imaging is to identify
congenital anomalies, obstruction, and other abnormalities
that may predispose the patient to infection. Ultrasound of the
urinary tract, including the kidneys and bladder, is used for
initial screening. VCUG is indicated after a first UTI only
if hydronephrosis, scarring, or other abnormalities sugges-
tive of high-grade vesicoureteral reflux (VUR) or obstructive
uropathy are seen with ultrasound, or in patients with com-
plex clinical conditions. VCUG is also recommended if there
is a recurrence of a febrile UTI [112].
Most centers in the United States currently perform con-
ventional fluoroscopic VCUG for evaluation of the bladder and
urethra and to diagnose VUR. Nuclear cystography can also be
used for diagnosis of VUR and is associated with a lower radia-
tion dose to the male gonads. Since girls rarely have urethral
abnormalities, nuclear cystography is used in some centers as
the initial study for girls with a normal renal and bladder ultra-
sound examination, as well as to follow reflux in all children.
CeVUS is being increasingly used worldwide as both an
initial imaging study in selected patients and in the follow-up
of patients with prior VCUG documentation of VUR. It is an
appealing alternative to conventional fluoroscopic VCUG due
to its increased sensitivity for the diagnosis of reflux and lack
of ionizing radiation [113].
Acute Pyelonephritis
Acute pyelonephritis, an infection of the renal parenchyma
and urothelium of the renal collecting system, is one of the
most serious bacterial illnesses of childhood. The most com-
mon pathogen is Escherichia coli, with other pathogens
including Proteus species, Staphylococcus saprophyticus, and
Pseudomonas aeruginosa. The majority of cases result from
ascending infection from the lower urinary tract due to vesico-
ureteral reflux. Less commonly, it can occur due to hematog-
enous seeding. The clinical presentation differs with patient age.
Symptoms in neonates and infants are nonspecific, and include
fever, malaise, vomiting, irritability, and failure to thrive. Older
children usually present with flank pain and fever [114, 115].

Table 17.1 Comparison of urinary tract infection imaging recommendations

Guideline Patient age Ultrasound (US) VCUG DMSA
NICE < 6 months All children Atypical/recurrent UTI Atypical/recurrent UTI
6 months-3 years Atypical/recurrent UTI Atypical/recurrent UTI AND Atypical/recurrent UTI
specific featuresa
> 3 years Atypical/recurrent UTI Not indicated Recurrent UTI
AAP ≤ 24 months All children Abnormal US or other specific –
circumstances
ISPN ≤ 36 months All children Abnormal US or risk factorsb Abnormal US or VUR
CPS < 2 years All children Abnormal US; recurrent UTI Only when diagnosis of
> 2 years Not specified Abnormal US UTI is in doubt
PSPN < 2 years All children Atypical/recurrent febrile Recurrent pyelonephritis,
> 2 years Pyelonephritis; atypical/recurrent UTI or UTI; abnormal US; family history VUR III-IV
risk factors for recurrent UTIc of VUR
EAU/ All children – VCUG or DMSA in bottom-up or VCUG or DMSA in
ESPU top-down approach bottom-up or top-down
approach
NICE, National Institute for Health and Care Excellence; AAP, American Academy of Pediatrics; ISPN, Italian Society of Pediatric Nephrology;
CPS, Canadian Paediatric Society; PSPN, Polish Society of Pediatric Nephrology; EAU, European Association of Urology; ESPU, European
Society for Pediatric Urology
US, Ultrasound; UTI, urinary tract infection; VCUG, voiding cystourethrography; DMSA, dimercaptosuccinic acid scintigraphy; VUR, vesicoure-
teral reflux
a
Dilation on ultrasound, poor urine flow, non-Escherichia coli infection, family history of VUR
b
First-degree relative with VUR, septicemia, chronic kidney disease, age < 6 months in a male infant, likely noncompliance of the family, abnormal
bladder emptying, no clinical response to correct antibiotic treatment within 72 h, bacteria other than E. coli
c
Abnormal US in past, positive family history of UTI or congenital anomalies of kidney and urinary tract, bladder catheterization, abnormal void-
ing, abnormal defecation, or sexual activity in girls
Modified from Okarska-Napierała M, Wasilewska A, Kuchar E. Urinary tract infection in children: diagnosis, treatment, imaging – comparison
of current guidelines. J Pediatr Urol. 2017;13(6):567–73. [111]
17 Urinary Tract
L presentation and laboratory evaluation, not on imaging
Fig. 17.62 Acute pyelonephritis in a 1-month-old male. Longitudinal gray-
scale ultrasound image of the right kidney shows globular enlargement with
increased parenchymal echogenicity compared to the adjacent liver (L)
Fig. 17.63 Acute focal pyelonephritis in a 13-year-old female. Trans­
verse grayscale ultrasound image shows a focal zone of swelling and
increased parenchymal echogenicity (arrows) in the interpolar region of
the kidney
Fig. 17.64 Focal renal scarring in a 10-year-old female with a recent
episode of acute pyelonephritis. (a) Longitudinal grayscale ultrasound
image of the right kidney demonstrates thinning and increased echo-
genicity of the upper pole parenchyma (arrowheads) with focal calyceal
769
Diagnosis of acute pyelonephritis is based on clinical
findings. If ultrasound is performed early on, it is usually
normal in the majority of uncomplicated cases. In more
severe cases, ultrasound may demonstrate renal enlarge-
ment, diffusely or focally increased cortical echogenicity,
poor corticomedullary differentiation, and/or urothelial
thickening (Figs. 17.62 and 17.63). Color Doppler eval-
uation may show renal hypoperfusion due to vascular
compression from renal swelling. Ultrasound can also
document any anatomical abnormalities of the kidneys,
ureters, or bladder [116].
Antibiotic therapy is the mainstay of treatment and should
be started promptly. Despite adequate therapy, renal scarring
sometimes cannot be prevented (Fig. 17.64) [117].
Renal Abscess
Renal abscess is a collection of purulent material in the kid-
ney that results from liquifactive necrosis. When antibiotic
therapy of acute pyelonephritis fails to lead to an adequate
clinical response, renal abscess formation should be sus-
pected. Renal abscess presents clinically with fever, flank
pain, and pyuria. Notably, up to 15–20% of cases have a
negative urine culture [118].
The typical appearance of a renal abscess on ultrasound
imaging is a hypoechoic mass with thick, irregular walls,
posterior acoustic enhancement, and absence of internal
flow on color Doppler imaging (Fig. 17.65). Internal sep-
tations and mobile debris may also be seen. Gas-forming
organisms will produce echogenic foci with dirty posterior
shadowing. Extra-parenchymal extension of a renal abscess
may occur to the ipsilateral psoas muscle and other retro-
peritoneal structures.
a b
dilation (arrow). (b) Tc-99m-dimercaptosuccinic acid (DMSA) renal
scintigraphy shows a corresponding photopenic zone in the upper right
kidney (arrowhead). L, Left side
770
The management of a renal abscess depends on its size.
Small abscesses are treated with antibiotics, whereas large
abscesses are drained percutaneously under ultrasound or
CT guidance. Ultrasound is useful to monitor resolution of
the abscess and to detect renal scarring.
Pyonephrosis
Pyonephrosis refers to an accumulation of pus in a dilated
renal collecting system. In children, the most common
underlying causes are obstructive uropathy, stones, or stric-
tures. Prompt diagnosis is crucial because if left untreated,
pyonephrosis can lead to gram-negative septicemia and sep-
tic shock with rapid, often permanent, deterioration of renal
function [118].
Fig. 17.65 Renal abscess in a 15-year-old male. Longitudinal gray-
scale ultrasound image shows focal renal parenchymal swelling in the
interpolar region (arrows) with central liquefaction (arrowhead)
a b
LP
Fig. 17.66 Pyonephrosis and pyoureter in a 6-month-old female with
renal duplication and ectopic ureterocele of the upper pole moiety. (a)
Longitudinal grayscale ultrasound image demonstrates a duplex renal
collecting system. The renal pelvis of the upper moiety (arrows) is
severely dilated and filled with heterogeneous, echogenic material. There
G. H. Kassab et al.
Ultrasound imaging features include dilation of the pelvo-
calyceal system that contains mobile, echogenic debris with
fluid-debris levels, and occasionally dirty shadowing of col-
lecting system gas (Fig. 17.66). Echogenic debris is the most
reliable sign of pyonephrosis with a sensitivity of 90% and
specificity of 97% [118, 119].
Treatment of pyonephrosis requires immediate percu-
taneous or surgical drainage and appropriate antimicrobial
therapy.
Fungal Infection
Candidiasis is the most common fungal urinary tract infec-
tion, with Candida albicans accounting for the majority of
cases. In children, predisposing conditions include prolonged
antibiotic use, chronic indwelling catheters, and immuno-
compromise (e.g., transplant recipients, immune deficiency
disorders, and malignancy). Patients typically present with
fever and chills, and occasionally with flank pain.
The majority of urinary fungal infections are confined
to the bladder and urethra; however, the ureters and kidney
can be involved due to hematogenous seeding or ascending
infection from the lower urinary tract [120].
On ultrasound imaging, renal fungal infection most com-
monly manifests as multiple tiny, hypoechoic parenchymal
foci in keeping with microabscesses (Fig. 17.67). Larger
abscesses may develop, which are typically heterogeneous
by ultrasound. Additional nonspecific findings may include
renal enlargement, increased cortical echogenicity, and loss
of corticomedullary differentiation. Fungal balls are con-
B
is no remaining upper pole parenchyma. The lower pole renal moiety
(LP) is normal. (b) Longitudinal grayscale ultrasound image of the
bladder (B) reveals a dilated ureter (calipers) filled with echogenic
material. There is a pus/fluid level (arrowhead). The distal ureter termi-
nates in a ureterocele (arrow) that protrudes into the bladder lumen
17 Urinary Tract 771

a b

Fig. 17.67 Renal fungal infection in a 20-year-old female with leukemia. focus. (b) Longitudinal contrast-enhanced ultrasound (CEUS) image of
(a) Longitudinal grayscale ultrasound image of the right kidney reveals a the right kidney shows a central avascular zone surrounded by a mildly
small, hypoechoic lesion (arrow) in the upper pole with a punctate central hyperenhancing rim (arrowheads) in keeping with a microabscess

a b

C
C

Fig. 17.68 Renal candidiasis in an immunocompromised 18-month-­ a focal echogenic, non-shadowing mass (calipers) in a calyx in keeping
old male. Transverse (a) and longitudinal (b) grayscale ultrasound with a fungal ball
images of the mid-left kidney show mild calyceal (C) dilation. There is

sidered a specific feature of fungal infection, appearing as Systemic antifungal therapy is the mainstay of treatment.
non-­shadowing, echogenic masses in the renal collecting Urinary diversion is sometimes required in complex cases
system (Fig. 17.68). They are often mobile and can cause with obstructive hydronephrosis [121].
obstruction.
772
Parasitic Infection
The most common parasitic infection of the urinary tract
is schistosomiasis caused by Schistosoma haematobium.
Although schistosomiasis occurs most frequently in Africa
and the Middle East, it is found throughout the world, most
often infecting infants and young children. Schistosomiasis
preferentially affects the bladder base and trigone. Initial
distal ureteral dysfunction occurs, which leads to dilation.
Subsequent fibrosis results in stricture and calcification,
which can progress toward the proximal ureter. Although the
kidneys are not the primary target of infection, they are even-
tually affected due to ureteral obstruction and reflux. Patients
usually present with hematuria. Schistosomiasis is a known
risk factor for the development of squamous cell cancer later
in life.
When imaged in the acute phase of disease, the uri-
nary bladder will appear nodular and thickened on ultra-
sound (Fig. 17.69). In the chronic phase of disease, the
bladder appears contracted and fibrotic with a thick wall.
Curvilinear mural calcifications, a common finding in
schistosomiasis, are best demonstrated on plain radiogra-
phy or CT [122]. The kidneys appear normal until late in
the disease process.
The drug of choice for treatment of schistosomiasis is
praziquantel, a quinolone derivative.
Renal involvement occurs in 2% of patients with hyda-
tid disease, a multisystemic parasitic infection caused by
Echinococcus granulosus. Patients typically present with
flank pain or an abdominal mass. Ultrasound features sug-
gestive of hydatid disease include a homogeneous, multi-
cystic, hypoechoic mass with septations and daughter cysts
typically arranged peripherally in a rosette pattern. Renal
hydatid cysts can be treated with surgical excision or with
a b
Fig. 17.69 Schistosomiasis of the bladder in 2 different 10-year-old males,
both of whom presented with hematuria. (a) Transverse grayscale ultra-
sound image shows marked nodular thickening of the urothelium at the
bladder base (arrow). (b) Transverse grayscale ultrasound image of the
­bladder obtained one month after institution of therapy reveals a significant
G. H. Kassab et al.
image-guided aspiration. Extreme caution must be paid dur-
ing treatment to avoid intra-abdominal spillage of cyst con-
tent that may trigger an anaphylactic reaction [122].
Opportunistic Infection
Opportunistic infections occur in severely immunocompro-
mised patients and can affect the urinary tract, including
fungal and mycobacterial infection. Transplant recipients
on immunosuppressive medications, patients with acquired
immunodeficiency syndrome (AIDS), bone marrow failure,
or malignancy undergoing chemotherapy are at particular
risk. On ultrasound, Pneumocystis jirovecii, a fungus that
commonly causes infection in immune-compromised indi-
viduals, can manifest as nephrocalcinosis and cortical cal-
cification [123]. The urinary tract findings associated with
fungal infection are described earlier in this chapter.
Chronic Pyelonephritis
While some authors believe that chronic pyelonephritis is an
active chronic infection, others believe that it represents sta-
ble changes from a remote single infection. The ultrasound
imaging findings include renal scarring, atrophy and cortical
thinning, hypertrophy of residual normal tissue (which may
occasionally mimic a mass), calyceal clubbing secondary to
retraction of the renal papillae from overlying scar tissue,
and urothelial thickening. Hypertension is often a long-term
complication [116].
Although ultrasound can detect significant renal paren-
chymal scarring, it is relatively insensitive for milder lesions.
Power Doppler evaluation increases the sensitivity of scar
c
decrease in urothelial thickening (arrow). (c) Longitudinal grayscale ultra-
sound image of the second patient depicts nodular thickening of the antero-
superior bladder urothelium (arrow). (Images courtesy of Dr. Tracy Kilborn,
Red Cross War Memorial Children’s Hospital, University of Cape Town,
South Africa)
17 Urinary Tract
a b
Fig. 17.70 Chronic pyelonephritis in a 12-year-old female with long-
standing reflux. (a) Longitudinal grayscale ultrasound image shows a
small kidney with thin, irregular parenchyma. (b) Longitudinal CEUS
detection [124]. Renal nuclear scintigraphy with Tc-99m-
dimercaptosuccinic acid (DMSA) is currently the reference
standard imaging technique for demonstrating focal areas of
inflammation and parenchymal scarring. Because mild scars
do not alter the course of therapy, the radiation dose of a
renal cortical scan is not required in the management of most
patients with UTI [125].
CEUS is another potentially promising technique to
assess focal renal scarring (Fig. 17.70) [126]. Diuretic renog-
raphy with MAG3 can be performed to determine split renal
function and to distinguish between obstructive and nonob-
structive causes of renal or ureteral dilation [127].
Xanthogranulomatous Pyelonephritis
Xanthogranulomatous pyelonephritis is a chronic, destruc-
tive granulomatous inflammatory process caused by recur-
rent bacterial infection. The renal parenchyma is ultimately
replaced by lipid-laden macrophages resulting in a non-
functioning kidney. Although it occurs mainly in adults, it
can rarely occur in children [128].
Xanthogranulomatous pyelonephritis can be diffuse or focal.
The majority of cases are associated with hydronephrosis and
a renal stone, typically a staghorn calculus. The focal form is
less common overall but is more frequently seen in children,
usually associated with a stone in an adjacent calyx or a stag-
horn calculus in one moiety of a duplicated renal collecting sys-
tem (Fig. 17.71). The most common causative organisms are
Escherichia coli and Proteus mirabilis. Symptoms are often
773
image accentuates the appearance of focal zones of scarring (arrow-
heads) in the upper pole and mid-portion of the kidney
nonspecific, including low-grade fever, malaise, flank pain, and
hematuria [114, 129].
The ultrasound features of xanthogranulomatous pyelo-
nephritis include an enlarged kidney, distorted renal archi-
tecture, and a central echogenic focus with distal ­shadowing
corresponding to a renal pelvic staghorn calculus. The
inflammatory process can extend to the ipsilateral psoas
muscle, other retroperitoneal structures, or the abdominal
wall. Renal atrophy can occur in some cases, although it is
not characteristic.
Focal xanthogranulomatous pyelonephritis may respond
to antibiotics, although partial nephrectomy is frequently
required. Treatment of diffuse xanthogranulomatous pyelo-
nephritis usually requires nephrectomy [130].
Cystitis
Infectious cystitis is usually bacterial in origin. Other less
common causes include fungal infection, schistosomiasis, and
tuberculosis. Adenovirus is a rare cause of urinary tract infec-
tion and may cause hemorrhagic cystitis, with pronounced
bladder wall thickening [131]. Bladder inflammation can also
occur as a result of drug treatment, as occurs with the chemo-
therapeutic agent cyclophosphamide [132]. Patients complain
of dysuria, frequency, urgency, malodorous urine, inconti-
nence, hematuria, and suprapubic pain. However, in newborns
and infants, fever may be the only sign.
Ultrasound demonstrates bladder wall thickening and
irregularity, mobile echogenic debris, or a fluid-debris level
774 G. H. Kassab et al.

a b
L

Fig. 17.71 Xanthogranulomatous pyelonephritis in a 12-year-old female lower pole calyx. The renal parenchyma is diffusely thinned. L, Liver.
with myelodysplasia. (a) Transverse grayscale ultrasound image of the (b) Longitudinal T2-weighted MR image of the hydronephrotic right kid-
right kidney demonstrates markedly dilated calyces containing echo- ney shows multiple calyceal fluid-debris levels (arrowhead)
genic debris (arrowhead). An echogenic stone (arrow) is identified in a

a b

Fig. 17.72 Hemorrhagic cystitis in a 16-year-old female. (a) dependent echogenic mass (arrowhead). (b) Transverse power Doppler
Transverse grayscale ultrasound image of the bladder reveals diffuse ultrasound image shows mural hypervascularity. The focal lesion is
wall thickening with intraluminal echogenic debris and a more focal avascular in keeping with blood clot

in the urinary bladder. Hypervascularity is seen with color
Doppler imaging (Fig. 17.72). Occasionally, an inflamma-
tory pseudotumor may develop that will resolve after treat-
ment. Cystitis is treated with antibiotics.
Renal Cystic Disease
Renal cystic disease can be classified on the basis of ana-
tomical distribution of the cysts within the nephron or on the
genetic or nongenetic origin of the disorder [133].
17 Urinary Tract
A detailed investigation of any relevant family history
is essential whenever renal cystic disease is suspected, and
screening ultrasound for the parents is sometimes indicated
to assess for unknown familial disease [134].
Renal ultrasound is the cornerstone of imaging in renal
cystic disease. There are two ultrasound features that lead to
suspicion of renal cystic disease: cysts and increased renal
parenchymal echogenicity. Although cysts can be present in
fetal life, they typically develop after birth. Ultrasound can
easily identify the location, size and number of renal cysts,
and whether they are unilateral or bilateral. The renal cortex
is normally hypoechoic to the liver and spleen in children
although relatively increased renal cortical echogenicity is
an expected finding in the postnatal period as discussed ear-
lier in this chapter [135, 136]. Increased renal parenchymal
echogenicity can be cortical, medullary, or diffusely involve
the entire kidney.
 utosomal Recessive Polycystic Kidney Disease
A
Autosomal recessive polycystic kidney disease (ARPKD) is
a hereditary renal cystic disease caused by mutations of the
polycystic kidney and hepatic disease 1 gene (PKHD1) on
chromosome 6p. This mutation results in fusiform dilation
(1–2 mm in diameter) of the collecting ducts largely in the
medulla with extension to the cortex. The glomeruli are nor-
mal [137].
The degree of renal and liver involvement (congenital
hepatic fibrosis, Caroli syndrome, or both) is variable, and
will determine the overall clinical course [138]. Patients with
ARPKD may have pulmonary hypoplasia and musculoskel-
etal abnormalities related to in utero oligohydramnios asso-
ciated with impaired renal function.
On ultrasound, renal size is variable, ranging from nor-
mal to massively enlarged. Increased renal parenchymal
echogenicity can be limited to the medulla or diffusely
involve the entire kidney. There may be macrocysts of
variable size, number, and location [139]. Renal parenchy-
mal echogenicity is frequently heterogeneous, sometimes
described as a “salt-­ and-­
pepper” appearance due to the
presence of multiple tiny cysts not clearly distinguishable
by ultrasound that nevertheless disrupt the normal echo pat-
tern (Fig. 17.73).
Severe cases of ARPKD may be identified on prenatal
ultrasound as hyperechoic kidneys with oligohydramnios,
while milder cases are identified later in life [140]. Ultrasound
is helpful in assessing the severity of liver involvement.
Annual abdominal ultrasound surveillance is recommended
to monitor patients for signs of portal hypertension [139].
Treatment options depend on disease severity. Children
with significant renal functional impairment may require
dialysis or kidney transplantation. Combined kidney and
liver transplantation may be needed for severe congenital
hepatic fibrosis and portal hypertension.
775
Fig. 17.73 A 1-day-old male with autosomal recessive polycystic dis-
ease. Longitudinal grayscale ultrasound image reveals an enlarged kid-
ney with increased parenchymal echogenicity, loss of corticomedullary
differentiation, and multiple cysts of varying size throughout the cortex
and medulla
 utosomal Dominant Polycystic Kidney Disease
A
Autosomal dominant polycystic kidney disease (ADPKD)
is the most common hereditary renal cystic disease, occur-
ring in approximately one in 1000 individuals [141]. It
results from a mutation in either the polycystin 1 gene
(PKD1) (chromosomal locus 16p) or the polycystin 2 gene
(PKD2) (chromosomal locus 4q), although spontaneous
mutations can occur, explaining the absence of family his-
tory in 5–15% of cases [142]. These mutations result in the
formation of multiple variable-sized cysts in the renal cor-
tex and medulla. The PKD1 gene mutation is more common
and associated with an earlier onset and more severe renal
dysfunction [143].
ADPKD is usually asymptomatic until adulthood; how-
ever, it can be detected in childhood during screening of fam-
ily members of individuals with known ADPKD. Clinical
manifestations of renal disease include hematuria, proteinuria,
and hypertension. Extrarenal manifestations in childhood are
rare compared to adults. These include cysts in the liver, pan-
creas, or spleen, and subarachnoid hemorrhage caused by rup-
ture of an intracranial aneurysm [144]. End-stage renal disease
(ESRD) and renal insufficiency occur later in life.
On prenatal ultrasound, the kidneys may appear normal or
can be large and hyperechoic. During infancy, the kidneys usu-
ally appear normal or may contain a few isolated cysts. Very
rarely they may be completely replaced by cysts. Cysts are
located in both the cortex and medulla with intervening normal
renal tissue. Although the cysts in ADPKD are macroscopic,
they are relatively small in childhood and tend to increase in
size with age (Fig. 17.74), ultimately resulting in near-com-
plete replacement of the renal parenchyma [145]. Hemorrhagic
cysts can occur. In a child with a positive family history, the
presence of at least one renal cyst, renal enlargement, or both
should be considered highly suggestive of ADPKD [139].
776
P
C
Fig. 17.74 Autosomal dominant polycystic disease in a 4-year-old
female. Longitudinal grayscale ultrasound image shows multiple periph-
eral cortical cysts (arrowheads) of variable size. P, Renal pelvis; C, calyx
Although ADPKD is a progressive disease, it generally
has a benign course in childhood. Treatment is largely con-
servative, aimed at blood pressure control and minimization
of nephrotoxin exposure. Specific interventions are only
needed if the patient is symptomatic, including image-guided
aspiration of symptomatic cysts and ethanol sclerotherapy
with excellent long-term outcomes. Renal transplantation is
usually needed by the fifth to seventh decades of life [145].
 ystic Renal Dysplasia
C ultrasound evaluation every 1–2 years is recommended to
A multicystic dysplastic kidney (MCDK) is a nonhereditary
condition and is considered the most severe form of the spec-
trum of cystic renal dysplasia. It is believed to result from in
utero urinary tract obstruction. The affected kidney is replaced
by numerous noncommunicating cysts and is nonfunctional.
Histological examination reveals multiple small cysts with
intervening dysplastic tissue containing primitive cartilage
and ductules suggestive of abnormal renal differentiation.
MCDK is more common in males and usually incidentally
detected on antenatal ultrasound examination. Associated
abnormalities can occur in up to 30% of affected individuals,
the most common being ureteropelvic junction obstruction,
ureterovesical junction obstruction, and vesicoureteral reflux
[134, 146].
The more frequent pelvoinfundibular form of MCDK man-
ifests as numerous, randomly distributed noncommunicating
cysts of varying size. There is no renal pelvis or renal sinus,
and parenchymal tissue is absent or dysplastic (Fig. 17.75).
The less common hydronephrotic form of MCDK consists of
multiple small, peripheral cysts and a larger central cyst, with
some connections between the central and peripheral cysts.
The whole kidney is usually involved and may be small, nor-
mal in size, or enlarged. There is no or minimal functional
renal tissue. The contralateral kidney usually demonstrates
compensatory hypertrophy [147].
G. H. Kassab et al.
Fig. 17.75 Multicystic dysplastic kidney (MCDK) in a 2-week-old
male. Longitudinal grayscale ultrasound image demonstrates multiple
randomly distributed, noncommunicating cysts of variable size scat-
tered within an echogenic stroma. No normal renal parenchyma or renal
pelvis is identified
Although usually affecting an entire kidney, multicystic
dysplasia can be confined to one segment, especially the
upper pole moiety of a duplex system [148]. Usually, the
affected kidney tends to involute while the contralateral
kidney undergoes compensatory hypertrophy. Follow-up
document involution.
Due to the natural history of spontaneous involution,
MCDK is not treated surgically unless it is complicated by
respiratory compromise from thoracic compression, infec-
tion, or pain from compression on adjacent structures.
Although there are a few case reports of increased risk of
either hypertension or malignancy in patients with MCKD,
this association is not universally accepted. Patients may
require longer follow-up if there are associated abnormalities
in the contralateral kidney [149].
Less severe forms of cystic renal dysplasia result in
abnormal kidneys that usually have a combination of cysts
and echogenic parenchyma (Fig. 17.76). Dysplasia may be
focal or diffuse and by ultrasound appears as echogenic tis-
sue without identifiable corticomedullary differentiation.
Nephronophthisis
Nephronophthisis is an autosomal recessive disease charac-
terized by chronic tubulointerstitial nephritis due to genetic
mutations that affect urine concentrating ability, with 11
mutations identified to date [150]. Nephronophthisis is a
slowly progressive form of renal failure and can be classified
according to the age of onset of ESRD as infantile, juvenile,
adolescent, or late onset [151, 152]. Children present with
polydipsia, polyuria, salt wasting, anemia, and growth retarda-
17 Urinary Tract
tion. Nephronophthisis is associated with several syndromes,
including Jeune syndrome (asphyxiating thoracic dystrophy),
Meckel-Gruber syndrome, and Joubert syndrome [150].
On ultrasound evaluation, the kidneys are usually small
in size with increased parenchymal echogenicity and loss
of corticomedullary differentiation. In the infantile form,
the kidneys may be large and contain multiple cysts remi-
niscent of ADPKD [152]. Renal cysts may not be evident
early in life but will increase in size and number with
increasing patient age. Cysts are usually located in the
medulla and at the corticomedullary junction with rela-
tive cortical sparing and will vary in size, measuring up to
1.5 cm (Fig. 17.77).
L Bowman’s space that can be seen with multiple clinical dis-
Fig. 17.76 Cystic renal dysplasia in a 4-day-old male. Longitudinal
grayscale ultrasound image of the right kidney demonstrates echogenic
parenchyma with cysts (arrows) and absent corticomedullary differen-
tiation. There is moderate dilation of the renal pelvis (asterisk). L, Liver
a b
Fig. 17.77 Nephronophthisis in a 4-year-old female. Longitudinal grayscale ultrasound images of the right (a) and left (b) kidneys demonstrate
countless tiny cysts in the medulla and at the corticomedullary junction of each kidney
777
Currently, nephronophthisis is incurable. The majority of
patients will progress to ESRD, eventually requiring renal
replacement therapy or transplantation.
 edullary Cystic Disease
M
Medullary cystic kidney disease shares some clinical and his-
tological features with nephronophthisis, including tubular
basement membrane disintegration, tubular cyst formation,
and tubulointerstitial inflammation and fibrosis. However,
medullary cystic disease has an autosomal dominant inheri-
tance, presents later in life (after the second decade), and has
a later onset of renal failure (after the fourth decade of life).
It has the same ultrasound features and cannot be differenti-
ated from nephronophthisis on the basis of its imaging char-
acteristics [145]. Similarly, there is no specific therapy, and
patients are managed supportively.
 lomerulocystic Kidney Disease
G
Glomerulocystic kidney disease is not a single disorder
but a histologic description that involves cystic dilation of
orders. It can occur in sporadic or familial forms, in isolation,
or in association with other malformations (e.g., tuberous
sclerosis complex; medullary cystic kidney disease; Jeune
syndrome; nephronophthisis; Meckel-Gruber syndrome; tri-
somies 9, 13, and 18; the short rib-polydactyly syndromes;
and Zellweger syndrome) [153].
On ultrasound, the kidneys are echogenic and may be
small, normal in size, or enlarged (Fig. 17.78). Small renal
volumes have typically been reported in cases of hereditary
glomerulocystic kidney disease. Kidney cysts are not always
evident by ultrasound [154]. Treatment will depend on the
underlying disorder.
778 G. H. Kassab et al.

(Fig. 17.79). Renal cysts in these patients do not require regu-

lar surveillance imaging.

Fig. 17.78 Glomerulocystic disease in a 5-day-old male. Longitudinal
grayscale ultrasound image shows an enlarged, echogenic kidney with
loss of normal corticomedullary differentiation, and innumerable tiny
cysts throughout the cortex and medulla
Syndromes with Renal Cysts
 on Hippel-Lindau Disease
V
Von Hippel-Lindau (VHL) disease is an inherited autosomal
dominant cancer-predisposing disorder caused by a muta-
tion of the VHL tumor suppressor gene on chromosome 3.
Sporadic mutations account for 20% of cases [157].
Renal manifestations of VHL disease include cysts and
renal cell carcinoma. By ultrasound, cysts can appear as sim-
ple or complex lesions (Fig. 17.80) that increase in number
with age. Even simple-appearing cysts in VHL syndrome are
considered premalignant lesions [158]. Renal cell carcinoma
associated with VHL disease usually appears as a complex
cystic lesion and is often multifocal and bilateral [159].
Patients who are suspected of having renal cell carcinoma
are usually followed until the lesions are greater than 3 cm in
diameter and then treated with partial nephrectomy or radio-
frequency ablation [160].

Tuberous Sclerosis
Tuberous sclerosis is one of the hereditary neurocuta- L
neous multisystemic disorders. It is caused by muta-
tion of tuberous sclerosis 1 (TSC1) or tuberous sclerosis
2 (TSC2) genes on chromosome 9 or 16, respectively.
Sporadic mutation occurs in approximately two-thirds of
cases, although it can also be inherited in an autosomal
dominant fashion [155]. The classical clinical diagnostic
triad consists of seizures, mental retardation, and adenoma
sebaceum. The renal manifestations of tuberous sclerosis
include angiomyolipomas, renal cysts, and renal cell car-
cinoma. Angiomyolipomas are present in 80% of patients
Fig. 17.79 Tuberous sclerosis in a 17-year-old female. Longitudinal
with tuberous sclerosis. grayscale ultrasound image of the right kidney shows several small cor-
Renal cysts develop in 18–53% of patients with tuber- tical cysts (arrowheads). The rounded echogenic lesions in the lower
ous sclerosis. Their prevalence increases with age and may pole (arrows) represent angiomyolipomas. L, Liver
be more common in males than females. They are usually
asymptomatic unless they occur in patients with a contigu-
ous mutation in the TSC2 and PKD1 genes on chromosome
16 (in 2–3% of patients) in which case the cysts have an
early onset of development. They tend to increase in size
and number over time with an appearance similar to that
seen with ADPKD, and cause hypertension or renal failure
in early adulthood [155, 156].
The incidence of renal cell carcinoma in individuals
with tuberous sclerosis is similar to that in the general
population (2–3%). However, the age at presentation is
much younger, with an average age of 28 years compared
to 53 years of age in the general population [156].
In most patients with tuberous sclerosis, the number Fig. 17.80 Von Hippel-Lindau disease in a 12-year-old male. Longitudinal
of renal cysts is limited, their size is small, and they cause grayscale ultrasound image demonstrates multiple variable-sized renal
no symptoms. They are readily identified by ultrasound cortical cysts (arrows)
17 Urinary Tract
Acquired Cystic Kidney Disease
Acquired cystic kidney disease (ACKD) is a condition that
occurs in patients with ESRD, who are on hemodialysis and
do not have a history of other cystic renal disease. It occurs
less frequently in those who are on peritoneal dialysis.
ACKD is an isolated renal condition with no involvement
of other organs. Its incidence increases with the duration
of renal failure, which has been estimated at about 20%,
40%, and 90% after 3, 5, and 10 years of hemodialysis,
respectively.
ACKD is usually asymptomatic, although cyst hemor-
rhage may cause flank pain and hematuria. Complications
include hemorrhage into cysts and development of renal
cell carcinoma. The risk of renal cell carcinoma in ACKD
is about 5%, and papillary renal cell carcinoma is the
most common histological subtype, accounting for 50%
of the cases compared to 10% in the general population
[161, 162].
The typical ultrasound appearance of ACKD is of small
kidneys with multiple bilateral cysts measuring 0.5–2 cm,
mainly localized to the cortex although they can also occur in
the medulla (Fig. 17.81). Both hemorrhagic cysts and renal
cell carcinoma appear as echogenic masses. While hemor-
rhagic cysts are avascular, renal cell carcinoma can show
internal vascularity on color Doppler, which helps to differ-
entiate between the two entities [162].
Although ACKD may regress after kidney transplanta-
tion, the increased risk of renal cell carcinoma persists.
Recommendations for screening of patients with ESRD
for ACKD and renal cell cancer are controversial [162,
163].
Fig. 17.81 Acquired renal cystic disease in a 13-year-old male on
chronic hemodialysis. Longitudinal grayscale ultrasound image shows
a small kidney with diffuse cortical thinning, loss of corticomedullary
differentiation, and multiple parenchymal cysts (arrows)
779
Renal Vascular Disease
Renal Artery Stenosis
Renal artery stenosis (RAS) is a relatively uncommon but
potentially curable cause of hypertension in children. Causes
of RAS in children include fibromuscular dysplasia, neurofi-
bromatosis, tuberous sclerosis, Williams syndrome, Takayasu
arteritis, and middle aortic syndrome [164].
Fibromuscular dysplasia is the most common cause
of RAS in children. It is characterized by idiopathic, non-
inflammatory, and non-atherosclerotic angiopathy of small
and medium-sized arteries that eventually results in arterial
stenosis (Fig. 17.82).
The most common histological subtype of fibromuscu-
lar dysplasia is medial dysplasia. Fibromuscular dysplasia
is usually asymptomatic but can present with hypertension,
failure to thrive, seizures, and stroke. The renal, internal
carotid, and vertebral arteries are usually affected [165]. The
celiac, mesenteric, splenic, hepatic, and iliac vessels can also
be involved, although less often.
Fibromuscular dysplasia is often multifocal and bilateral
(up to 60% when involving the renal arteries). It affects the
mid-to-distal segments of the vessel and spares the origin,
with alternating zones of stenosis and dilation resulting in a
characteristic beaded appearance. Affected vessels are more
prone to dissection and aneurysm formation due to wall
weakness [166].
Grayscale ultrasound findings in RAS can include a decrease
in renal size on the affected side, and the renal artery may appear
small and beaded [166]. Flow parameters and Doppler crite-
ria for the diagnosis of RAS in children have not been widely
evaluated or validated. It is uncertain whether the commonly
accepted adult criteria, listed below, are applicable to children:
(1) a peak systolic velocity (PSV) > 200 cm/sec at the stenotic
site with evidence of post-stenotic turbulence causing spectral
broadening; (2) a renal artery to aorta PSV ratio > 3.5; and (3)
in severe RAS, a tardus-parvus waveform in the parenchymal
arteries (acceleration index < 300 cm/sec2), with a prolonged
acceleration time (> 0.07 seconds) [167].
Given the uncertainty regarding Doppler velocity criteria,
it has been recommended that pediatric patients with hyper-
tension and no clinical, laboratory or ultrasound evidence of
vascular disease whose blood pressure is controlled on one or
two drugs be monitored closely without further imaging. In
children where the probability of renal vascular disease is very
high, digital subtraction angiography should be performed
regardless of the renal Doppler ultrasound findings [168].
780 G. H. Kassab et al.

a b

c d

e f

Fig. 17.82 Renal artery stenosis secondary to fibromuscular dysplasia
in a 14-year-old female with hypertension. Longitudinal grayscale ultra-
sound images of the right (a) and left (b) kidneys reveal a size discrep-
ancy with the left diffusely smaller than the right. Longitudinal color
Doppler ultrasound images with spectral analysis of (c) the left proximal
main renal artery demonstrates a markedly elevated peak systolic veloc-
ity of 339 cm/sec. Peak systolic velocity in (d) an upper pole branch of
the left main renal artery measures 138 cm/sec, a normal value. However,
the waveform is abnormal, with a delayed systolic upstroke (pulsus
­tardus). (e) There is a pulsus tardus and a low arterial resistive index (RI)
in a left upper pole artery indicative of significant upstream stenosis.
(f) Peak systolic velocity in the abdominal aorta is 131.9 cm/sec.
(g) Angiographic image shows a “string of beads” appearance (arrow) of
the left main renal artery in its mid-to-distal portion extending to the
level of the trifurcation
17 Urinary Tract 781

The arterial resistive index (RI) is elevated in RAS ommended for monitoring of kidney function, early detec-
although it should be interpreted with caution based on the tion and management of hypertension and chronic kidney
child’s age. The RI can be as high as 0.9 in preterm infants, disease [173].
0.6–0.8 in neonates and infants, and only reaches adult val-
ues of 0.5–0.7 after the first year of life. An RI >0.85 in
neonates and >0.7 in children are considered abnormal Renal Artery Pseudoaneurysm
[168].
Asymptomatic cases are clinically observed. Management Renal artery pseudoaneurysm is an uncommon vascular find-
of symptomatic cases with renovascular hypertension includes ing, with the majority occurring after an intervention such
antihypertensive therapy, percutaneous angioplasty of severe as percutaneous renal biopsy or percutaneous nephrostomy.
stenosis, and reconstructive surgery. Pseudoaneurysms can also complicate renal transplantation,
blunt abdominal trauma, as well as renal inflammatory and
neoplastic processes [174]. A pseudoaneurysm develops
Renal Artery Thrombosis when the wall of the renal artery is injured with leakage of
blood into the adjacent soft tissues. This collection is eventu-
Renal artery thrombosis is a rare condition in the pediatric pop- ally enclosed in a layer of fibrous tissue. Although usually
ulation. In infants, it is nearly always associated with a history asymptomatic, these lesions can rupture, leading to life-
of umbilical artery catheterization. It can also occur in infants threatening hemorrhage [175].
of diabetic mothers, infants with dehydration or sepsis, or from A pseudoaneurysm appears as an anechoic cystic lesion
an embolism through a patent ductus arteriosus [169]. In older on grayscale ultrasound. Doppler imaging plays a key role in
children, renal artery thrombosis is usually related to trauma, diagnosis, showing a swirling pattern of blood flow within the
vasculitis, and valvular heart disease. It presents clinically with pseudoaneurysm with a “yin-yang” pattern of to-and-fro flow
abdominal pain and hematuria with leukocytosis and elevated (Fig. 17.84). Pulsed Doppler evaluation of flow at the neck of
serum lactate dehydrogenase levels [170]. Segmental renal the pseudoaneurysm shows disordered bidirectional flow.
artery infarction can result in hypertension with salt depletion Endovascular selective coil embolization is the mainstay
(hyponatremia-hypertension syndrome) [171]. of treatment for pseudoaneurysms [176]. Main renal artery
Grayscale ultrasound shows global or segmental wedge-­ pseudoaneurysms may require stent placement or surgical
shaped, hypoechoic zones with an absence of blood flow ligation.
on color and spectral Doppler (Fig. 17.83). This can
be confirmed by CEUS as hypoperfusion or absence of
enhancement in the infarcted regions compared to normal Renal Vein Thrombosis
parenchymal enhancement [172]. Over time, hyperechoic
scars will develop at the sites of infarction. Renal vein thrombosis (RVT) is the most common vascular
Management of neonatal renal artery thrombosis requires abnormality of the newborn kidney, accounting for approxi-
a multidisciplinary team that includes neonatologists, radiol- mately 10% of all venous thromboemboli in newborns.
ogists, pediatric hematologists, and nephrologists. In addition Almost 80% of all cases of RVT present within the first
to removal of the umbilical catheter and supportive therapy, month of life, most often in the first week. RVT is bilateral
prophylactic heparin is administered in most cases to pre- in about 25% of cases [177]. The most common predispos-
vent thrombus extension. Thrombolytic therapy is reserved ing factors in newborns include dehydration, sepsis, birth
for bilateral thrombosis compromising kidney function. asphyxia, maternal diabetes, polycythemia, and an indwell-
In older children, treatment depends on the acuity of ing umbilical venous catheter. Prenatal cases have also been
thrombosis and the underlying predisposing disorders. described, particularly in fetuses of diabetic mothers [178].
Thrombolytic therapy, surgical thrombectomy, and arte- In older children, RVT can be associated with trauma,
rial bypass are therapeutic options. Long-term sequelae, neoplastic processes, or nephrotic syndrome. There are two
such as kidney atrophy, systemic hypertension, and mechanisms of renal vein thrombosis. In the first, more com-
chronic kidney disease are common, and follow-up is rec- mon form in neonates, thrombosis is initiated at the level of
782
a b
c d
Fig. 17.83 Renal artery thrombosis in a 14-year-old male with acute myo-
carditis and severe biventricular dysfunction. (a) Longitudinal grayscale
ultrasound image shows a swollen left kidney with a blotchy appearance of
the parenchyma and loss of normal corticomedullary differentiation. (b)
Longitudinal color Doppler ultrasound image of the left renal hilum does not
show a normal renal artery. Small hilar vessels (arrowheads) are noted.
the arcuate and interlobular veins, with proximal extension
into the larger renal veins and ultimately into the IVC. In the
second form, the thrombus arises initially in the IVC and
extends in a retrograde fashion into the renal vein. This latter
mechanism occurs in older children [179].
Clinically, the presence of a flank mass, gross hematuria,
or thrombocytopenia raises the suspicion of RVT, especially
in a neonate with risk factors. Adrenal hemorrhage is a rec-
ognized association with RVT, especially on the left side due
to extension of thrombosis to the adrenal vein.
G. H. Kassab et al.
e
V, Main renal vein. Longitudinal color Doppler ultrasound images with
spectral analysis reveal (c) low amplitude, low resistance arterial flow in the
small hilar vessels, and (d) low amplitude, slightly dampened venous flow in
the main renal vein. (e) Coronal contrast-enhanced CT image shows a linear
thrombus (arrow) in the left main renal artery. The left renal parenchyma
demonstrates a complete absence of enhancement (arrowhead)
The ultrasound appearance of RVT varies depending on
when the examination is performed relative to the onset and
extent of thrombosis. Early grayscale ultrasound features
include renal swelling, increased parenchymal echogenicity,
and loss of corticomedullary differentiation. Small paren-
chymal venous thrombi initially appear as highly echogenic
streaks that persist for several days. Renal vein and vena
caval thrombi may appear as intraluminal echogenic foci.
Since thrombi are initiated in small venules and subse-
quently propagate toward the hilum, early parenchymal
17 Urinary Tract
Fig. 17.84 Pseudoaneurysm in a 17-year-old male with a history of a
left lower pole kidney laceration. (a) Longitudinal grayscale ultrasound
image of the left kidney shows an anechoic lower pole cystic structure
(arrow). (b) Longitudinal color Doppler ultrasound image reveals a
“yin-yang” pattern of blood flow (arrow) due to swirling of blood
abnormalities are frequently present without visualization
of a renal vein thrombus (Fig. 17.85). Kidney swelling
progresses over the first week, the cortex becomes more
echogenic, and the renal pyramids appear relatively more
hypoechoic. Subsequently, the kidney becomes more het-
erogeneous in appearance due to focal areas of edema and
hemorrhage [180].
Over the next 1–2 weeks, a ring of reduced echogenicity
develops around the affected pyramids, and an echogenic band
may develop at the extreme apex of the pyramid. These find-
ings represent focal apical changes and fibrosis related to renal
tubular damage [181].
The long-term sequelae of RVT depend on the extent of
thrombosis and the formation of collateral veins. If there are
sufficient collateral vessels to maintain renal perfusion, the
affected kidney may recover completely. Otherwise, hemor-
rhagic infarction will result from venous congestion, which
later heals by fibrosis, resulting in the formation of renal
scars or diffuse renal atrophy [182].
783
within the pseudoaneurysm. There is prominent blood flow (arrow-
heads) within the adjacent lower pole vessels feeding and draining the
pseudoaneurysm. (c) Longitudinal color Doppler ultrasound image
with spectral analysis reveals disordered aneurysmal blood flow that
appears both above and below the baseline
Doppler ultrasound findings in RVT include absent or
decreased renal venous flow, and high-resistance arterial
flow with decreased, or reversed diastolic arterial flow. Even
when low venous flow is demonstrated, as with non-occlu-
sive thrombus or in the presence of renal vein collaterals, the
venous flow appears relatively monotonous, with absence of
the usual transmitted pulsations from the right atrium [182].
Since the left adrenal vein drains into the left renal vein, a
renal vein thrombus can propagate directly into the left adre-
nal vein, which commonly manifests as adrenal hemorrhage.
Arteriovenous Fistula
An arteriovenous fistula (AVF) is an abnormal direct
communication between an artery and a vein without an
intervening capillary network. The majority of cases are
acquired lesions occurring after renal biopsy, nephrostomy,
blunt or penetrating trauma, inflammation, malignancy, or
784
a b
d e
Fig. 17.85 Renal vein thrombosis and adrenal hemorrhage in a
14-day-­old female. Longitudinal grayscale ultrasound images of the
left flank reveal (a) a swollen, echogenic kidney with loss of normal
corticomedullary differentiation and multiple linear, echogenic paren-
chymal streaks (arrowheads) representing venous thrombi. (b) There is
a fluid collection in the left adrenal region (asterisk) displacing the left
kidney inferiorly and containing echogenic, dependent debris (arrow)
renal surgery. Small renal AVFs are asymptomatic. A large
AVF can result in vascular steal and may present with flank
pain, hematuria, hypertension, high output cardiac failure,
renal insufficiency, massive hemorrhage, or thromboembolism.
Clini­cally, a continuous abdominal bruit or palpable thrill may
be present [183].
Grayscale ultrasound often reveals no abnormality or an
AVF can appear as a cystic lesion. With color Doppler, an
AVF may demonstrate soft tissue color artifact caused by tis-
sue vibration (Fig. 17.86). Pulsed Doppler findings include
high ­systolic velocity and high diastolic flow leading to a
low-resistance waveform in the afferent artery; turbulent
flow at the site of fistula; and arterialized flow in the efferent
vein [183, 184].
Small, asymptomatic AVFs do not require treatment
and may regress spontaneously. Symptomatic AVFs can be
treated with angioembolization or surgical ligation [184].
G. H. Kassab et al.
c
AV
consistent with blood products. (c) Longitudinal color Doppler ultra-
sound image of the left kidney shows flow in the main renal artery (A)
and main renal vein (V). Longitudinal color Doppler ultrasound images
with spectral analysis show (d) a normal waveform in the main renal
vein. The arterial waveform in the main renal artery (e) is highly abnor-
mal, with increased velocity and reversal of end-diastolic flow. This
pattern is an indirect but characteristic sign of renal vein thrombosis
Medical Renal Disease
Medical renal disease may result in acute kidney injury or
chronic kidney disease.
Acute Kidney Injury
Acute kidney injury (AKI), previously referred to as acute
renal failure, is a rapid deterioration of renal function, result-
ing in an inability to maintain fluid, electrolyte, and acid-­
base balance. It is clinically defined as a 50% or greater rise
in serum creatinine or a 25% or greater fall in estimated glo-
merular filtration rate (eGFR) known or presumed to have
occurred within the preceding 7 days.
Causes of AKI can be classified into pre-renal, renal, and
post-renal categories. Pre-renal causes are most common in
17 Urinary Tract 785

a b

c d

Fig. 17.86 Post-biopsy arteriovenous fistula (AVF) in a 15-year-old
male with end-stage renal disease. (a) Longitudinal grayscale ultra-
sound image shows a small kidney with echogenic parenchyma and loss
of normal corticomedullary differentiation. There is an ovoid anechoic
focus (arrowhead) in the lower renal pole. A hypoechoic perirenal
hematoma (asterisks) is present. (b) Longitudinal color Doppler ultra-
sound image of the left kidney reveals markedly increased blood flow at
the lesion site (white arrowhead), as well as adjacent soft tissue color
artifact (black arrowheads) associated with tissue vibration. There is a
subcapsular hematoma (white arrows) in addition to the perirenal
hematoma (asterisk). (c) Longitudinal color Doppler ultrasound image
obtained with a significantly increased velocity setting depicts the feed-
ing and draining vessels (arrows) associated with the AVF (arrowhead).
(d) Longitudinal color Doppler ultrasound image with spectral analysis
of the AVF demonstrates high-amplitude, low-­resistance arterial flow
above the baseline and arterialized venous flow below the baseline

children, especially in the neonatal period (about 70–80% of
cases), including renal hypoperfusion due to severe dehydra-
tion, heart failure, and septic shock. Intrinsic renal causes
such as acute glomerulonephritis, acute tubular necrosis,
hemolytic-uremic syndrome, Henoch-Schönlein purpura,
nephrotic syndrome, and drug nephrotoxicity account for
10% of AKI cases and usually affect older children. Post-
renal causes account for about 10% of cases and include uri-
nary obstruction, especially from posterior urethral valves in
early childhood [185].
786
a b
Fig. 17.87 Acute kidney injury secondary to cardiogenic shock in an
18-day-old female. (a) Longitudinal grayscale ultrasound image of the
right kidney shows increased parenchymal echogenicity compared to the
Renal ultrasound is the imaging modality most often used in
the evaluation of patients with AKI. The main role of ultrasound
is to determine renal size and to exclude anatomical abnor-
malities as the cause of AKI [186]. Doppler techniques yield
information regarding renal perfusion and vascular abnormali-
ties (Fig. 17.87). Ultrasound is also used to guide percutane-
ous renal biopsy. Functional information can be obtained from
nuclear medicine studies that can help differentiate between
pre-renal, renal, and post-renal causes of AKI.
Treatment of AKI depends on the underlying cause.
Chronic Kidney Disease
Chronic kidney disease (CKD) is a clinical syndrome char-
acterized by a gradual loss of kidney function over time. The
Kidney Disease: Improving Global Outcomes guidelines have
defined CKD as abnormalities of kidney structure or function
present for more than 3 months [187]. CKD can have a devas-
tating impact on children, potentially leading to malnutrition,
growth impairment, developmental delay, bone growth disor-
ders, anemia, and hypertension.
The most common causes of CKD in children are congeni-
tal abnormalities of the kidney and urinary tract, (e.g., renal
hypoplasia, renal dysplasia, reflux nephropathy, and obstruc-
tive uropathy), steroid-resistant nephrotic syndrome, chronic
glomerulonephritis, and renal ciliopathies. Other less com-
mon causes include polycystic kidney disease (both autosomal
recessive and autosomal dominant), thrombotic microangi-
opathies (especially atypical hemolytic-­ uremic syndrome),
nephrocalcinosis/nephrolithiasis, interstitial kidney disease,
and infection.
Structural causes of CKD (e.g., renal hypoplasia or pos-
terior urethral valves) predominate in younger patients,
G. H. Kassab et al.
liver (L). Asterisk, Ascites. (b) Transverse color Doppler ultrasound
image with spectral analysis reveals increased arterial pulsatility and an
elevated RI of 0.84
whereas the incidence of glomerulonephritis increases in
children more than 12 years of age [188].
Imaging plays a crucial role in diagnosis, assessment of
renal function, and monitoring of the results of treatment
in children with CKD. As with AKI, ultrasound is the main
imaging modality used to investigate CKD and to guide
percutaneous biopsy. Assessment of renal and bladder mor-
phology permits the diagnosis of congenital anomalies and
determination of renal size. Monitoring of renal size is essen-
tial for long-term follow-up. Hypoplastic/dysplastic and
scarred kidneys are usually small, whereas enlarged kidneys
occur with obstruction, glomerulonephritis, and some cystic
disorders. As CKD progresses, there is a gradual reduction
in kidney size and loss of corticomedullary differentiation.
Most patients will also have echogenic renal parenchyma.
Treatment of CKD is supportive. Renal transplantation
is the treatment of choice for children with end-stage renal
disease, with improved patient survival and quality of life
compared to dialysis.
Renal Transplantation
Renal transplantation is the treatment of choice for patients
with end-stage renal disease who are maintained on perito-
neal dialysis or hemodialysis. There are three types of allografts:
mismatched cadaveric renal grafts, nonidentical living-related
grafts, and human lymphocyte antigen–identical grafts with
reported 1-year survival rates of 80%, 90%, and 95%, respec-
tively [189].
Ultrasound and radionuclide imaging are the imaging modal-
ities employed in renal transplantation assessment. Ultrasound is
used in the postoperative period and for long-­term follow-up. It
is also helpful in guiding diagnostic or therapeutic interventions,
17 Urinary Tract 787

including biopsy drainage of fluid collections. Radionuclide
imaging is performed to assess graft function.
Surgical Technique
An understanding of the surgical techniques commonly
used for renal transplantation is essential prior to imaging
in order to anticipate and recognize complications, and
to provide guidance regarding further imaging and inter-
vention. Surgical techniques vary with patient age and by
institution.
In younger children, the renal transplant is placed intra-
peritoneally. An end-to-side anastomosis is created between
the donor renal artery and vein and the recipient distal
aorta and IVC, respectively. In older children, the renal
transplant is placed retroperitoneally in the iliac fossa with
the external iliac artery and vein most commonly chosen
for anastomosis (Fig. 17.88). An antirefluxing ureteral
anastomosis to the urinary bladder is created. In complex
cases, the native ureter of the recipient can be used as a
conduit.

Diseased
kidneys

Incision Transplanted
kidney

Bladder

Donor kidney

Donor renal artery

and vein attached
to external iliac
vessels

External iliac
artery and vein

Donor ureter
attached to bladder

Fig. 17.88 Diagram of retroperitoneal surgical technique used for renal transplantation in older children
788 G. H. Kassab et al.

a b c

Fig. 17.89 Normal appearance of a renal allograft in a 12-year-old ultrasound image demonstrates normal intraparenchymal blood flow.
female. (a) Longitudinal grayscale ultrasound image of a renal allograft (c) Color Doppler ultrasound image with spectral analysis of an interlo-
(cursors) in the right iliac fossa shows normal corticomedullary differ- bar renal artery shows a sharp systolic upstroke with antegrade flow
entiation and echogenic renal sinus fat. (b) Longitudinal color Doppler throughout diastole

Normal Posttransplant Imaging

On ultrasound, the normal renal transplant has a similar

appearance to the normal native kidney (Fig. 17.89). The
proximity of the transplant kidney to the anterior abdomi-
nal wall accentuates the contrast between the renal cortex,
medulla, and renal sinus.

Complications

Vascular Complications
Children are at increased risk of early vascular thrombosis
following transplantation compared with adults because of
their small size and frequent discrepancies between the size
of the donor and recipient vessels.
Fig. 17.90 Lower pole arterial thrombosis 1 day after renal transplan-
tation in a 16-year-old female. Longitudinal color Doppler ultrasound
image reveals a complete absence of blood flow (arrow) to the lower
pole of the allograft

Renal Artery Thrombosis

Renal artery thrombosis with graft infarction is a rare complica-
tion of renal transplantation, occurring in only 3–4% of all pedi-
atric renal transplants [190, 191]. It usually develops within the
first 2 days after transplantation and is associated with en bloc
transplants from young cadaveric donors that contain paired
renal allografts, as well as the ureters, main renal arteries, and
veins, and segments of the juxtarenal aorta and IVC. Most cases
are related to arterial kinking or intimal dissection.
Segmental infarction sometimes occurs in the early post-
operative period in allografts with multiple renal arteries
(Fig. 17.90), although these lesions more often develop as a
late complication in association with acute or chronic rejec-
tion (Fig. 17.91). Clinical symptoms of infarction include ten-
derness and swelling over the graft and anuria. Optimization Fig. 17.91 Segmental infarction of renal allograft in a 12-year-old male
4 years after transplantation. Longitudinal grayscale ultrasound image
of Doppler imaging parameters for the detection of slow flow
reveals increased cortical echogenicity with poor corticomedullary dif-
is mandatory to prevent misdiagnosis of arterial thrombosis ferentiation. Focal wedge-shaped hypoechoic zone of cortical thinning
when acute rejection is present, since severe rejection has (arrow) in the upper pole of the allograft represents an old infarct
17 Urinary Tract
been reported to cause markedly diminished blood flow that
mimics arterial thrombosis.
Graft failure is the usual outcome of arterial thrombosis,
although urgent thrombolytic therapy or thrombectomy can
be attempted.
Renal Artery Stenosis
Renal artery stenosis is a late complication of renal trans-
plantation, with a reported incidence of 4–18% [192, 193].
Clinical presentation includes new or progressive hyperten-
sion, marked hypertension refractory to medical therapy,
hypertension associated with graft dysfunction in the absence
of rejection, and hypertension associated with a systolic bruit
audible over the graft.
The incidence of transplant renal artery stenosis in chil-
dren is difficult to determine as there is no consensus regard-
ing what degree of arterial narrowing is clinically significant.
The most widely accepted criterion is elevation of peak sys-
tolic velocity at the site of narrowing, although the literature
conflicts regarding optimal cutoff values. In adults, peak sys-
tolic values greater than 250 or 300 cm/sec are highly sensi-
tive and specific for the diagnosis of transplant renal artery
stenosis [194, 195]. However, data regarding the usefulness
of these criteria in children are minimal [196].
Color and spectral Doppler ultrasound evaluation
reveals elevated velocities in the narrowed segment with
spectral broadening of the arterial waveform. Occasionally,
there is an associated downstream tardus-parvus wave-
form in the intrarenal arteries with associated low resistive
indices. There may be color aliasing within the stenotic
portion of the artery and perivascular soft tissue vibration
artifacts caused by turbulent flow in the stenotic segment.
The velocity measurements in the main renal artery, iliac
artery, and the segmental renal arteries should be inter-
preted together.
MR or CT angiography can be performed prior to conven-
tional angiography, the current reference standard imaging
test. These additional studies may detect a significant steno-
sis of the transplant main renal artery.
There is frequently stable graft function in patients with a
significant stenosis identified by imaging, and many patients
will respond well to conservative treatment. When there are
functional consequences of renal artery stenosis, percutane-
ous transluminal angioplasty with or without stent placement
can be performed.
789
Renal Vein Thrombosis
Renal vein thrombosis (RVT) is a rare complication of renal
transplantation that usually occurs in the first week of the
postoperative period. Predisposing factors include shock
or dehydration, venous compression from a peritransplant
fluid collection, surgical technique, and decreased flow
secondary to rejection. Left lower quadrant allografts are
at higher risk of RVT due to compression of the left com-
mon iliac vein between the sacrum and the left common
iliac artery (May-Thurner or “silent iliac compression”
syndrome). Clinical suspicion for RVT is raised when a
patient has abrupt cessation of urinary function, swelling,
and tenderness over the allograft [197].
On grayscale ultrasound, the allograft may appear swol-
len and hypoechoic with echogenic material in the renal
vein representing thrombus material. There will be absent or
reduced flow in the renal vein with increased resistance in
the renal artery resulting in reversed diastolic flow on pulsed
Doppler imaging (Fig. 17.92) [198].
Prompt diagnosis of RVT is crucial because the renal trans-
plant can sometimes be salvaged by urgent thrombectomy.
Nevertheless, graft infarction may be inevitable even with
early diagnosis, and transplant nephrectomy may be required.
Arteriovenous Fistula
An arteriovenous fistula (AVF) is the most common compli-
cation of graft biopsy and is almost never of clinical signifi-
cance. Most AVFs are asymptomatic and more than 75% will
spontaneously resolve. The ultrasound features of AVF in a
renal homograft are the same as those described in the native
kidney earlier in this chapter: possibly inapparent on gray-
scale imaging, but obvious with color and spectral Doppler
evaluation (Fig. 17.93) [199]. Symptomatic fistulas leading
to ischemia are treated with embolization.
Pseudoaneurysm
Pseudoaneurysm is another complication of renal allograft
biopsy. It also tends to be asymptomatic and to resolve
spontaneously. As previously described for the native
kidney, a pseudoaneurysm is depicted as a cystic struc-
ture on grayscale ultrasound with a “yin-yang” pattern
of to-and-fro flow on color and spectral Doppler imag-
ing (Fig. 17.94). Progressive enlargement or a diameter
greater than 2 cm are indications for embolic treatment
[199].
790
Fig. 17.92 Renal vein thrombosis 1 day after kidney transplantation in a
20-month-old male. (a) Longitudinal grayscale ultrasound image of the right
lower quadrant renal allograft demonstrates diffuse swelling with echogenic
parenchyma and decreased corticomedullary differentiation. Longitudinal
color Doppler ultrasound images with spectral analysis reveal (b) diminished
G. H. Kassab et al.
flow in the main renal vein with a dampened waveform and (c) markedly
pulsatile flow in the main renal artery with reversal of end-­diastolic flow. (d)
Axial contrast-enhanced CT image shows an extensive thrombus (arrow) in
the main renal vein of the allograft. The allograft (asterisk) is nonperfused
except for a thin rim of tissue at the periphery (arrowheads)
17 Urinary Tract
a b
Fig. 17.93 Post-biopsy AVFs of renal allograft in a 10-year-old female.
(a) Longitudinal grayscale ultrasound image shows two hypoechoic
lesions (arrowheads) in the lower pole of the allograft. A tubular anechoic
a b
Fig. 17.94 Post-biopsy pseudoaneurysm of renal allograft in a 15-year-old
male. (a) Longitudinal grayscale ultrasound image reveals an anechoic cys-
tic structure (calipers) in the mid-kidney. (b) Longitudinal color Doppler
Parenchymal Complications
Parenchymal complications of renal transplantation pres-
ent as decreased renal function, and ultrasound evaluation
is generally most helpful in ruling out causes of graft dys-
function such as a large vessel abnormality, urinary col-
lecting system obstruction, or perinephric fluid collection.
US-guided biopsy is usually needed to identify the specific
cause of graft failure. Parenchymal complications include
acute tubular necrosis, rejection, and drug toxicity and are
discussed further below.
Acute Tubular Necrosis
Acute tubular necrosis (ATN) occurs to some degree in most
renal transplants immediately postoperatively. ATN is clini-
791
structure (arrow) extending from the more proximal lesion represents a
draining vein. (b) Longitudinal color Doppler ultrasound image demon-
strates disordered blood flow within each lesion
c
ultrasound image demonstrates a “yin-yang” pattern of swirling blood flow
in the pseudoaneurysm. (c) Spectral analysis of the pseudoaneurysm
depicts disordered blood flow
cally characterized by poor renal function and oliguria and
is thought to result from prolonged ischemia and reperfusion
injury. ATN affects cadaveric grafts more than grafts from liv-
ing related donors (35% vs 10%) and usually resolves sponta-
neously over the first 2 weeks after transplantation [200].
Ultrasound findings of ATN include diffuse allograft
enlargement, increased or decreased cortical echogenicity,
loss of corticomedullary differentiation, prominent pyra-
mids, urothelial thickening, and effacement of the central
renal sinus echo complex. In severe cases, pulsed Doppler
evaluation may show increased arterial resistive indices
(>0.8) and reversed diastolic flow (Fig. 17.95) [189]. These
findings are nonspecific and can also be seen with acute graft
rejection and drug toxicity.
792
a b
Fig. 17.95 Acute tubular necrosis 3 days after renal transplantation in
a 12-year-old male. (a) Longitudinal grayscale ultrasound image dem-
onstrates diffuse swelling of the renal allograft with increased cortical
Rejection
Rejection is the most common cause of allograft failure
and can be classified into hyperacute, acute, and chronic
types.
Hyperacute rejection is characterized by ischemia and
necrosis of the graft that occurs from the time of transplan-
tation up to 48 hours after transplantation. It is believed to
be caused by cytotoxic antibodies present in the recipient
that respond to tissue antigens on the donor organ. Patients
with hyperacute rejection are not usually imaged. Antibody-­
mediated rejection is frequently associated with graft loss.
However, early and less severely affected kidneys can occa-
sionally be salvaged by increasing the dosages of the immu-
nosuppressant medication and administering antilymphocyte
antibodies. In highly presensitized cases with high levels of
circulating antibodies, plasmapheresis can be attempted if
the patient is hemodynamically stable.
Acute rejection is common, with up to 50% of patients
experiencing at least one episode in the first year after trans-
plantation. Clinically, the patient may present with oliguria,
malaise, fever, weight gain, or tenderness over the allograft;
or they may be asymptomatic, particularly if they are receiv-
ing cyclosporine [201]. There are two histological types of
acute rejection: interstitial (or cellular), which is more com-
mon, and vascular (or humoral). In interstitial rejection, there
is cellular infiltration of the renal interstitium with sparing of
the arterioles and glomeruli so that diastolic flow and vascular
impedance are not affected. Vascular rejection results from
endovasculitis with subsequent vascular damage and throm-
bus formation resulting in increased vascular impedance.
The clinical and ultrasound features of acute rejection are
similar to those of ATN, with the two entities best differen-
tiated by their differing time course. Acute rejection rarely
occurs in the first few days after transplantation, usually
G. H. Kassab et al.
echogenicity and decreased corticomedullary differentiation. (b) Color
Doppler ultrasound image with spectral analysis of an interlobar artery
depicts abnormally pulsatile flow with an RI = 0.8
presenting as a progressive decrease in renal function within
the first 3 months after transplantation. Pulsed Doppler find-
ings depend on the subtype of acute rejection. In interstitial
rejection, vascular impedance is not affected, and the arte-
rial resistive index (RI) is normal (Fig. 17.96). In vascular
rejection, there is increased vascular impedance with a high
arterial RI (Fig. 17.97) [202].
Chronic rejection is the most common cause of late graft
failure, occurring 3 months to years after transplantation.
Patients will usually present with azotemia and hyperten-
sion. Renal function progressively declines and eventually
fails. Renal biopsy is essential for the diagnosis and dem-
onstrates proliferation of graft arteries and arterioles, inter-
stitial cellular infiltration and fibrosis, tubular atrophy, and
glomerular changes [201]. Ultrasound findings of chronic
rejection include a small kidney; a thin, echogenic cor-
tex; decreased corticomedullary differentiation; and mild
hydronephrosis.
Drug Toxicity
Drug nephrotoxicity is another cause of decreased renal
function and allograft failure. The calcineurin inhibitors
cyclosporine and tacrolimus are the foundation of immu-
nosuppressive therapy in pediatric renal transplantation.
However, their nephrotoxic potential can result in graft
injury. Clinical features are similar to those associated with
ATN and rejection, including abdominal pain, fever, rising
serum creatinine, and decreased urine output.
Ultrasound features of drug nephrotoxicity are nonspe-
cific, with graft enlargement, loss of corticomedullary dif-
ferentiation, and decreased diastolic arterial blood flow with
elevated resistive indices [203].
Treatment consists of a reduction in the dose of the immu-
nosuppressive drugs [204].
17 Urinary Tract
Fig. 17.96 Biopsy-proven acute interstitial rejection in a 7-year-old
male with a renal allograft. (a) Longitudinal grayscale ultrasound
image demonstrates a swollen kidney with preservation of normal
Fig. 17.97 Biopsy-proven acute vascular rejection in a 9-year old
female with a renal allograft. (a) Longitudinal color Doppler ultra-
sound image of the right iliac fossa demonstrates poor corticomedul-
lary differentiation of the allograft. There is only a small amount of
Urologic Complications
Ureteroneocystostomy is the reconstructive technique cur-
rently used for restoration of urinary tract continuity in patients
who undergo renal transplantation. This has led to a lower inci-
dence of urologic complications (4–8%) compared to older
procedures such as ureteroureterostomy or ­pyeloureterostomy
(10–25%). About 60–70% of early urologic complications,
793
parenchymal echogenicity and corticomedullary differentiation. (b)
Color Doppler ultrasound image with spectral analysis of an interlobar
artery reveals normal arterial waveforms with an RI of 0.68
central blood flow with absent peripheral flow. (b) Color Doppler ultra-
sound image with spectral analysis of the main renal artery shows abnor-
mal pulsatility with reversed end-diastolic flow
including urine leak and obstruction, occur in the first few
weeks after transplantation [205, 206].
Transplant Urine Leak
Urine leak is a rare complication of transplantation and is
usually apparent in the first 2 weeks after surgery. Urine
extravasation can occur anywhere from the renal calyces to
794
a b
Fig. 17.98 Urinoma in a 16-year-old male related to necrosis of the
renal pelvis and ureter 2 weeks after kidney transplantation. (a, b)
Longitudinal grayscale ultrasound images of the left iliac fossa show a
multiloculated fluid collection (arrows) along the inferolateral aspect of
the ureteroneocystostomy site. A urine leak can result in a
localized perinephric urinoma or in urine ascites, depending
on whether the graft is intra- or extraperitoneal in location.
Urine leak can result from ureteral necrosis secondary to
ischemia or increased pressure related to obstruction. Urine
leak is clinically suspected when there is decreased urine
output, swelling and tenderness around the graft, ipsilateral
leg swelling, and scrotal or labial edema.
On grayscale ultrasound, a urinoma can appear as an amor-
phous, anechoic fluid collection surrounding the graft or may
have a more complex appearance. A urinoma can compress
the ureter, leading to hydronephrosis (Fig. 17.98). Follow-up
ultrasound studies are useful in documenting changes in col-
lection size. Ultrasound-guided percutaneous aspiration can be
performed when further characterization of the fluid is required.
Technetium (Tc)-99m-­ mercaptoacetyltriglycine (MAG3)
radio­nuclide studies are often used to distinguish a urinoma
from a lymphocele or seroma since they are typically indistin-
guishable on imaging. Radiotracer excreted by the kidneys accu-
mulates in a urinoma, but a lymphocele demonstrates persistent
photopenia. Excretory MR urography is an alternative imaging
technique that provides both functional and anatomic informa-
tion without radiation exposure [203].
Close patient monitoring or stent placement can be used
for small, non-obstructing collections. However, approxi-
mately half the patients with a urine leak will eventually
require surgical intervention [207].
Transplant Ureteral Obstruction
Urinary obstruction complicates 2% of renal allografts,
almost always within the first 6 months after transplantation.
The most common site of obstruction is at the ureteroneo-
cystostomy and is related to ischemia or rejection, technical
anastomotic failure, or ureteral kinking. Other less common
causes include intrinsic lesions causing intraluminal obstruc-
tion (e.g., calculi, papillary necrosis, fungus ball, and clot)
or external compression from adjacent peritransplant fluid
collections [208].
G. H. Kassab et al.
c
the renal allograft. There is moderate dilation of the renal collecting
system (arrowheads). (c) Nephrostogram image shows contrast leakage
(arrow) arising from the ureteropelvic junction and extending along the
inferolateral aspect of the allograft (arrowheads)
Patients usually present with deteriorating renal function.
Diagnosis of renal obstruction is sometimes delayed by the
absence of renal colic typically associated with obstruction
because of denervation of the allograft during transplantation.
The presence or absence of mild-to-moderate dilation of
the urinary tract correlates poorly with the presence or absence
of urinary tract obstruction in the posttransplant patient [209].
However, depiction of moderate-to-severe hydronephrosis and
hydroureter that increases progressively over time warrants
further evaluation to rule out obstruction. Tc-99m-MAG3
radionuclide studies or MRU can be performed, with the more
invasive percutaneous antegrade urography used to localize
the site of obstruction. A nephrostomy tube can be placed for
purposes of urinary tract decompression and to permit stenting
or balloon ureteroplasty [203].
Transplant Vesicoureteral Reflux
Vesicoureteral reflux (VUR) in the setting of pediatric renal
transplantation occurs in approximately 12% of patients
[210]. There is increased incidence of posttransplant VUR
in children with a noncompliant bladder, detrusor overac-
tivity, posterior urethral valves, or urethral stenosis [211].
However, the presence of VUR does not appear to be asso-
ciated with either an increased frequency of posttransplant
UTI or decreased long-term graft function [212, 213].
As previously discussed earlier in this chapter, the ultra-
sound features associated with VUR are usually nonspecific
but can be associated with hydronephrosis and/or renal scar-
ring (Fig. 17.99). Fluoroscopic and radionuclide voiding cys-
tography are currently the standard imaging techniques for
detection of VUR, although there will probably be an increas-
ing role for ceVUS in the future.
Low-grade VUR is managed with surveillance and long-­
term antibiotic prophylaxis, whereas high-grade reflux may
require surgical reimplantation of the ureter. Revision of the
ureterovesical anastomosis can be challenging due to scar
tissue and vascular insufficiency of the transplant ureter that
increase the risk of necrosis and anastomotic failure [210].
17 Urinary Tract
a b
Fig. 17.99 Vesicoureteral reflux into the renal allograft of a 10-year-­old
female. (a) Longitudinal grayscale ultrasound image of the left-sided kidney
transplant shows mild-to-moderate dilation of the collecting system
a b
Fig. 17.100 Pyelonephritis of a renal allograft in a 17-year-old female.
(a) Longitudinal grayscale ultrasound image of the renal allograft demon-
strates diffuse parenchymal swelling with urothelial thickening (arrow-
heads). (b) Longitudinal grayscale ultrasound image of the allograft ureter
Transplant Pyelonephritis
Infection is very common in renal transplant recipients,
especially in the first year after surgery, and can manifest as
pyelonephritis, parenchymal or perinephric abscess, pyo-
nephrosis, or fungus ball. Pyelonephritis is associated with
vesicoureteral reflux in pediatric renal transplant recipients.
Renal infection can be an early or late complication. In the
first few weeks after transplantation, the causative organ-
isms are similar to those that typically develop in non-
immunocompromised patients after surgery. Opportunistic
infections occur 1–6 months after transplantation, while
infections common in the general population are seen after
6 months.
795
(arrows). (b) Coronal radionuclide cystography image shows a moderate
degree of reflux into the transplant ureter and renal collecting system. L, Left
c
also shows diffuse urothelial thickening (arrowheads). Asterisk, Renal
allograft. (c) Transverse grayscale ultrasound image of the bladder shows
echogenic intraluminal debris (arrow) related to urinary tract infection
On ultrasound evaluation, the transplant kidney usually
appears normal in the setting of pyelonephritis, but may
occasionally demonstrate swelling and altered parenchymal
echogenicity (Fig. 17.100). A parenchymal abscess is an
unusual complication of graft pyelonephritis. Abscesses usu-
ally appear within the first postoperative month. Although
the ultrasound appearance of an abscess is relatively non-
specific, prominent septations, internal debris, and mural
hyperemia all suggest an infectious etiology. In the setting
of pyelonephritis, the presence of mobile, hyperechoic foci
within a dilated collecting system should raise concern for
pyonephrosis [214]. Fungus balls appear as discrete intralu-
minal masses [215].
796
Prompt diagnosis and treatment of infection are essential to
prevent graft loss and improve outcome. Parenchymal abscesses
can be treated with either ultrasound- or CT-guided percutane-
ous drainage and systemic antibiotics [215].
Perinephric Fluid Collections
Transplant Lymphocele
Lymphocele is the most frequently encountered perinephric
collection that occurs in about 15% of patients 1–2 months
after surgery. These collections develop due to surgical disrup-
tion of the lymphatic channels along the iliac vessels or of the
hilar lymphatics of the transplanted kidney. Most lymphoceles
are asymptomatic, incidentally discovered, and do not require
therapy. However, they can potentially exert mass effect on
the kidney causing obstruction or renal functional impair-
ment. Lymphoceles can occasionally present with edema of
the abdominal wall, scrotum, labia, or lower extremity [216].
On ultrasound imaging, a lymphocele appears as a uniloc-
ular or multilocular collection with internal septations located
between the kidney and urinary bladder (Fig. 17.101). It can
cause hydronephrosis if it exerts mass effect on the ureter.
Percutaneous aspiration with drain placement is reserved for
symptomatic patients. Recurrent collections can be treated with
surgical marsupialization or transcatheter sclerotherapy [203].
Transplant Urinoma
See earlier section on Transplant Urine Leak.
Transplant Hematoma and Seroma
A hematoma or seroma can develop immediately after trans-
plantation and are expected sequelae of surgery [200]. The
clinical significance of a hematoma depends on it size and
location. A small hematoma is usually clinically insignifi-
cant, whereas a large hematoma can cause urinary obstruc-
tion from mass effect or a drop in hematocrit. A seroma is
Fig. 17.101 Lymphocele in an 8-year-old female with a renal allograft.
Longitudinal grayscale ultrasound image shows two fluid collections
(asterisks) adjacent to the renal allograft. The lower collection is sepa-
rate from the bladder (not shown) and contains a small amount of echo-
genic, dependent debris (arrow)
G. H. Kassab et al.
distinct from a hematoma as it contains almost no red blood
cells. It is thought to develop as plasma from local bleeding
accumulates at the site of recent tissue disruption from sur-
gery or trauma.
The ultrasound appearance of a hematoma depends on its
age. In the immediate postoperative period, a hematoma will
appear as an anechoic or hypoechoic collection. Once clot-
ting has occurred, an acute hematoma will appear moderately
echogenic, with homogeneous echotexture. As it matures,
a hematoma demonstrates an increasingly heterogeneous
internal structure, with peripheral organizing clot and thick
internal septa superimposed on an anechoic serous compo-
nent (Fig. 17.102). A hematoma can completely resolve over
time or develop into a chronic seroma with imaging char-
acteristics of simple fluid [202]. A seroma manifests as a
simple fluid collection. It can develop nodular margins as it
matures.
Aspiration is not recommended for an asymptomatic col-
lection because of its typically transient nature and the risk of
introducing infection. Percutaneous image-guided aspiration
of a perinephric hematoma or seroma can be performed if
there is concern for superimposed infection. When a subcap-
sular hematoma causes impaired graft function, percutane-
ous drainage or surgical capsulotomy are treatment options.
In the setting of active extravasation with hypotension, angi-
ography with transcatheter embolization is the treatment of
choice [203].
Transplant Abscess
Perinephric abscesses usually develop within the first few
weeks after transplantation. They usually result from bacte-
rial seeding of a lymphocele, hematoma, or urinoma or, less
commonly, are associated with pyelonephritis.
Fig. 17.102 Hematoma adjacent to the renal allograft of a 10-year-old
male. Transverse grayscale ultrasound image reveals a heterogeneous
collection (arrows) medial to the allograft (asterisk). Percutaneous aspi-
ration of the collection revealed blood
17 Urinary Tract
a b
V
A
Fig. 17.103 Perinephric abscess of renal allograft in a 16-year-old male.
(a) Longitudinal grayscale ultrasound image of the right iliac region
shows an ill-defined collection (arrowheads) with both cystic and solid
components inferior to the allograft (asterisk). V, External iliac vein. (b)
Transverse color Doppler ultrasound image shows the ill-­defined collec-
tion (arrowheads) abutting the medial aspect of the allograft (asterisk) in
a b
Fig. 17.104 Posttransplant lymphoproliferative disorder (PTLD) in a
20-year-old female with a renal allograft. (a) Longitudinal grayscale
ultrasound image of the right iliac fossa shows the lower pole of a renal
allograft (asterisk) with an echogenic, solid mass (arrows) arising from
Immunosuppression may obscure the symptoms of infec-
tion in these patients, although they may present with fever,
pain or symptoms related to the pressure of the abscess on
the transplanted kidney. Fever should be treated with a high
index of suspicion in transplant patients and any perirenal
fluid collection should be treated as infected in febrile trans-
plant recipients until proven otherwise.
On ultrasound, abscesses appear as hypoechoic complex
fluid collections with debris and/or septations (Fig. 17.103).
Highly reflective echoes with acoustic shadowing are caused
by the presence of gas.
797
c
the region of the renal sinus and encircling the iliac vessels. A, External
iliac artery; V, external iliac vein. (c) Coronal contrast-enhanced CT
image shows the minimally enhancing, irregular collection (arrowheads)
abutting the renal sinus (asterisk). Purulent material was subsequently
drained via a percutaneous approach
the wall of an adjacent bowel loop (B). (b) Coronal contrast-enhanced
CT image shows the mass (arrows) arising from a loop of bowel (arrow-
heads) next to the renal allograft (asterisk)
Abscesses are treated with either ultrasound- or CT-guided
percutaneous drainage and systemic antibiotics.
Posttransplant Tumors
Long-term immunosuppression predisposes renal transplant
recipients to the development of tumors, both within the trans-
plant kidney and in other organs. Posttransplant lymphopro-
liferative disorder (PTLD) and inflammatory myofibroblastic
tumor have both been described.
The imaging appearance of PTLD depends on the site of
involvement (Fig. 17.104). When the transplant is affected,
798
it usually appears as multiple ill-defined, hypoechoic paren-
chymal masses [206]. Inflammatory myofibroblastic tumor
is a rare and usually benign neoplasm that is described later
in this chapter in the Renal Tumors section.
Urinary Tract Calcification
Renal Cortical Calcification
Renal cortical calcification, also known as cortical nephro-
calcinosis, results from pathological deposition of calcium
in the renal cortex and is much less common than medullary
nephrocalcinosis. The usual causes of cortical calcification
are chronic glomerulonephritis and acute cortical necrosis.
Less common causes include Alport syndrome, chronic hyper-
calcemic states, ethylene glycol poisoning, oxalosis, and
sickle cell disease. Cortical calcification can also be seen in
renal transplants in the setting of chronic rejection.
On ultrasound, cortical calcification appears as a thin,
echogenic rim outlining the cortex with a “tramline” appear-
ance (Fig. 17.105). Less commonly, calcium deposition in
necrotic glomeruli results in a spotty appearance [217].
There is no specific treatment for renal cortical necrosis.
Medullary Nephrocalcinosis
Medullary nephrocalcinosis is the most common form of
nephrocalcinosis and refers to the deposition of calcium salts
in the medulla of the kidney. It is almost always bilateral, dif-
fuse, and symmetric in distribution. The most common causes
of medullary nephrocalcinosis are hyperparathyroidism and
Fig. 17.105 Renal cortical calcification in a 1-year-old male with a
history of cortical necrosis. Longitudinal grayscale ultrasound image of
the right kidney demonstrates a diffusely thinned, markedly echogenic
cortex (arrowheads) in keeping with calcification
G. H. Kassab et al.
distal renal tubular acidosis (type 1). Asymmetric medullary
nephrocalcinosis can be seen in medullary sponge kidney as
a result of calcium deposition within dilated collecting ducts.
Medullary nephrocalcinosis tends to occur early in life and is
frequently associated with inborn errors of metabolism, pre-
maturity, and the use of certain medications, especially furo-
semide. Nephrocalcinosis is clinically asymptomatic and is
usually identified incidentally during imaging performed for
other reasons.
On ultrasound, early nephrocalcinosis may be seen as
a peripheral rim of increased medullary echogenicity with
relative sparing of the central pyramid. A sequence has
been described where this peripheral pattern progressively
fills the pyramid leading to a diffuse increase in medul-
lary echogenicity without distal shadowing (Fig. 17.106).
High-­resolution, ­focused ultrasound reveals that the most
peripheral portion of the pyramid is spared, appearing as
a narrow hypoechoic rim [7]. Even with diffuse involve-
ment of the medullary pyramids, renal function is often
normal.
A pattern of medullary nephrocalcinosis characterized by
focal increased echogenicity at the tip of the renal pyramid is
most commonly seen in preterm infants where small calculi
develop in the calyces adjacent to the affected papillae. This
finding has previously been attributed to furosemide therapy,
although it is also seen in preterm infants who have never
received furosemide, and therefore other factors are presum-
ably involved [7].
Treatment is aimed at correcting the underlying cause of
nephrocalcinosis. Prognosis depends on the underlying eti-
ology, with the majority of the cases without an associated
genetic defect resolving spontaneously in the first few years
of life. Progression to end-stage renal failure is uncommon.
Follow-up ultrasound imaging is recommended to document
resolution or progression.
Fig. 17.106 Medullary nephrocalcinosis in a 3-year-old female with
renal tubular acidosis type 1. Longitudinal grayscale ultrasound image
of the right kidney reveals diffusely hyperechoic renal pyramids (arrows)
due to calcium deposition
17 Urinary Tract
a b
Fig. 17.107 Dystrophic calcification in a 7-year-old male with prior
upper pole nephrectomy for treatment of an ectopic ureter. Longitudinal
(a) and transverse (b) grayscale ultrasound images of the right kidney
Renal Vein Thrombosis Calcifications
Finely branching calcifications often develop within the renal
cortex and medulla following renal vein thrombosis that repre-
sent microthrombi within the intrarenal veins (Fig. 17.85a) [182].
Dystrophic Calcification
Dystrophic calcification of abnormal tissue can occur any-
where in the urinary tract, including the wall of a cyst, at
sites of inflammation, or within a tumor (Fig. 17.107) [218].
Urinary Stasis
Any form of urinary stasis predisposes to calcium deposi-
tion. The tubular ectasia that occurs in autosomal polycystic
kidney disease and in medullary sponge kidney is frequently
associated with deposits of calcium in the pyramids, at sites
of dilated tubules. Similarly, milk of calcium, a viscous col-
loidal suspension of various calcium salts, may deposit in
calyceal diverticula or in the renal pelvis in the setting of
ureteropelvic junction obstruction. Staghorn calculi are
occasionally seen in children with renal obstruction and
infection. Stones can also develop in the setting of bladder
diverticula, bladder outlet obstruction, and neurogenic blad-
der (Fig. 17.108) [218].
799
reveal a dense focus of calcification (arrows) in the upper pole nephrec-
tomy bed with associated distal shadowing
Urolithiasis
Urolithiasis refers to macroscopic calcifications that occur
in the kidney, ureter, or bladder. Renal stones in children are
uncommon, but their frequency has been increasing in recent
years. Underlying causes include urinary tract infection,
structural abnormalities of the renal tract, and metabolic dis-
orders. Dietary, genetic, and climatic factors also influence
the risk of developing renal stones.
Approximately 40–60% of stones are predominantly
composed of calcium oxalate, 10–20% are mainly calcium
phosphate, 10–25% are mixed stones containing both cal-
cium oxalate and calcium phosphate, 17–30% are magne-
sium ammonium phosphate (struvite or infection related),
6–10% are cystine, and 2–10% are uric acid [219]. Flank
pain and hematuria are typical presenting symptoms [220].
Adequate hydration prior to imaging is important to max-
imally distend the portions of the urinary tract proximal to
the obstruction and to optimize visualization of stones in the
distal ureters and bladder [221].
Ultrasound is the initial imaging modality of choice, with
noncontrast CT performed only when ultrasound is not diag-
nostic. By ultrasound, renal stones are identified as echogenic
foci with or without posterior acoustic shadowing (Fig. 17.109).
The color Doppler twinkling (or twinkle) artifact can be help-
ful in the diagnosis of small stones that are not associated with
shadowing, hydronephrosis, or hydroureter (Fig. 17.110). This
artifact appears as a discrete focus of alternating color Doppler
800
Fig. 17.108 Staghorn calculus in an 18-year-old male with a neuro-
genic bladder. (a) Longitudinal grayscale ultrasound image of the left
kidney demonstrates extensive echogenic foci within the renal collecting
Fig. 17.109 Renal stone in a 13-year-old female. Longitudinal gray-
scale ultrasound image demonstrates an echogenic focus (calipers) in
the renal pelvis with posterior acoustic shadowing. There is associated
mild renal collecting system dilation
signal immediately deep to the object that causes it, with or
without an associated color comet-tail artifact [222, 223]. This
sign has a high false-­positive rate when compared with 5-mm
thick, unenhanced CT images [223]. The different types of uri-
nary tract stone cannot be differentiated by ultrasound.
A common pitfall in the ultrasound diagnosis of urinary
tract calculi is mistaking air for a stone. Air in the collect-
ing system usually results from recent catheterization or uri-
nary diversion. Air can be differentiated from a stone by its
“dirty” or poorly defined shadowing compared to the “clean”
or sharply defined shadowing associated with stones. Color
G. H. Kassab et al.
system with prominent distal shadowing (arrowheads). (b) Radiograph
of the upper abdomen shows a large branching stone (arrow) in the left
renal calyces and pelvis
Doppler can help in the diagnosis of obstructive stones at the
UVJ by documenting the absence or decreased velocity of
the ureteral jet (Fig. 17.111) [107].
Risk Factors
Approximately 70% of children with urolithiasis have an
underlying predisposing condition such as chronic infection,
urinary stasis, or hypercalciuria [219].
Kidney Stone Risk Factors
In children, idiopathic hypercalciuria is the most common
underlying metabolic abnormality and is associated with the
formation of calcium-containing stones. Primary oxlauria
and secondary oxaluria increase the risk of developing cal-
cium oxalate stones, as in patients with fat malabsorption
related to cystic fibrosis and inflammatory bowel disease.
Infection with urease-producing bacteria such as Proteus
species predisposes to the formation of struvite stones, espe-
cially in boys less than 5 years of age with structural abnor-
malities of the urinary tract such as primary megaureter,
bladder exstrophy, and neurogenic bladder. Uric acid stones
commonly develop after treatment of myeloproliferative dis-
orders or in the Lesch-Nyhan syndrome, a rare metabolic
disorder affecting the uric acid pathway [217, 218].
Bladder Stone Risk Factors
Urinary bladder stones usually occur in the setting of stasis.
Risk factors include infection with urease splitting organisms
(usually Proteus), an intravesical foreign body, neurogenic
bladder, bladder augmentation or reconstruction, bladder
diverticulum, and congenital bladder anomalies [217, 218].
17 Urinary Tract
a b
Fig. 17.110 Color Doppler twinking artifact with color comet-tail arti-
fact in an 18-year-old female with a tiny renal stone. (a) Longitudinal
grayscale ultrasound image of the left kidney shows a tiny echogenic focus
(arrowhead) in the lower pole without associated distal shadowing or caly-
ceal dilation. (b) Longitudinal color Doppler ultrasound image reveals a
a b
Fig. 17.111 Absence of ureteral jet in a 16-year-old female with a ure-
terovesical junction (UVJ) stone. Longitudinal (a) and transverse (b)
grayscale ultrasound images of the bladder reveal a small stone (arrow-
heads) in a mildly dilated distal right ureter (arrows). (c) Transverse
Trauma
Renal Trauma
Children are at increased risk for renal trauma due to several
anatomic characteristics that expose the kidneys and make them
more vulnerable to injury, such as larger kidney size relative
to body size, positioning lower in the abdomen and therefore
less protected by the rib cage, a smaller amount of perirenal fat,
weaker abdominal wall musculature, and a weaker, less calci-
fied thoracic rib cage. The renal capsule and Gerota’s fascia are
less developed than in adults, creating a greater potential for
parenchymal laceration and extravasation of urine [224].
In contrast to adults, hematuria is a very unreliable sign
in determining the need to screen for renal injures in chil-
dren [225]. Contrast-enhanced CT is the current modality
of choice for initial imaging in clinically stable patients
with suspected renal trauma. However, contrast-enhanced
ultrasound has recently become an appealing alternative to
contrast-enhanced CT in the evaluation of pediatric patients
with blunt abdominal trauma, particularly with respect to the
potential reduction of population-level exposure to ionizing
radiation, as discussed below [226, 227].
801
c
zone of alternating colors (arrowhead) at the site of the echogenic focus
seen in (a) that is in continuity with a linear band of aliased color extend-
ing away from the ultrasound transducer. (c) Axial nonenhanced CT
image confirms the presence of a small left lower pole renal stone (arrow)
c
color Doppler ultrasound image of the bladder demonstrates a normal
left-sided ureteral jet (arrow). There is no right-sided ureteral jet. The
right-sided UVJ stone (arrowhead) is associated with a prominent color
Doppler twinkling artifact and color comet-tail artifact
 ontrast-Enhanced Ultrasound Diagnosis of
C
Trauma
Contrast-enhanced ultrasound (CEUS) diagnosis of visceral
injury in the setting of abdominal trauma is of particular
interest in children who are more vulnerable to the hazards
of ionizing radiation than adults. CEUS is useful in stable
pediatric patients with isolated blunt, low-to-­moderate energy
abdominal trauma to rule out solid organ injuries; to further
evaluate uncertain CT findings; and in the follow-up of conser-
vatively managed traumatic injuries. CEUS can detect abnor-
malities that may not be apparent by conventional ultrasound,
including infarcts, pseudoaneurysms, and active bleeding.
Following the intravenous bolus administration of ultra-
sound contrast material, the renal cortex usually enhances
immediately and very intensively, while the pyramids enhance
from the periphery to the center in about 30 seconds. The
optimal time frame for detection of renal injuries is up to
about 2.5 minutes after contrast injection. Each kidney is
assessed with a separate bolus [228].
The typical appearance of a renal laceration on CEUS is
an absence of parenchymal perfusion compared to the adja-
cent normally perfused kidney (Fig. 17.112, Cineclip 17.1).
Active hemorrhage can be detected as focal extravasation of
802
a b
Fig. 17.112 Renal laceration and perirenal hematoma in a 15-year-old
male. (a) Longitudinal grayscale ultrasound image reveals an ill-
defined zone of hypoechoic parenchyma (arrowheads) in the left mid-
kidney with a surrounding anechoic fluid collection (asterisk). (b)
Longitudinal CEUS image demonstrates a linear parenchymal defect
a b
Fig. 17.113 Bladder rupture in a 4-day-old female. (a) Transverse
color Doppler ultrasound image of the abdomen reveals a large amount
of ascites (asterisk) in the peritoneal cavity. L, Liver. (b) Longitudinal
contrast material. However, since ultrasound contrast agents
remain intravascular and are not filtered or excreted by the
kidney, CEUS is unable to directly image traumatic lesions
of the collecting system or ureter which can only be inferred
by the presence of a fluid collection.
The American Association for the Surgery of Trauma
(AAST) divides traumatic renal injuries into five categories.
Grade I–III injuries are managed conservatively, while grade
V injuries generally require surgical exploration and repair.
Management of patients with grade IV injury remains contro-
versial although there has been a recent shift toward conser-
vative management and the use of interventional radiological
procedures as long as the patient remains clinically stable [229].
Bladder Trauma
Bladder injury occurs as a result of blunt, penetrating, or
iatrogenic injury. The risk of bladder injury in children is
greater than in adults due to the tendency of the full bladder
G. H. Kassab et al.
c
(arrow) in the mid-­kidney that extends to the hilum (see Cineclip 17.1)
in keeping with a grade IV American Association for the Surgery of
Trauma (AAST) injury. Asterisk, Anechoic fluid collection. (c) Axial
contrast-enhanced CT image confirms the presence of a deep renal lac-
eration (arrowhead) and perirenal hematoma (asterisk)
CEUS image of the bladder (B) demonstrates a posterosuperior defect
(calipers) of the bladder wall with extensive extravasation of contrast
(arrowhead) into the peritoneal cavity. FC, Foley catheter
to extend into the abdomen where it is not afforded the same
protection as when surrounded by the bony pelvis. Blunt
trauma is the most common cause of urinary bladder injury
in children [230]. Bladder injury can lead to extraperitoneal
or intraperitoneal rupture, or a combination of the two [231].
In the setting of trauma, bladder injury should be suspected
when there is anuria, inability to insert a urinary catheter, and
free fluid in the abdomen.
Conventional grayscale ultrasound is generally not
helpful in the diagnosis of bladder injury, with fluoro-
scopic VCUG or CT cystography the traditional imaging
modalities of choice. With the advent of contrast-enhanced
voiding urosonography, there may be an expanded role
in the future for ultrasound diagnosis of these injuries
(Fig. 17.113).
Extraperitoneal rupture occurs approximately 60%
of the time as a result of pelvic fracture or penetrating
trauma with extra-peritoneal extravasation of contrast
material noted on imaging. Intraperitoneal rupture is iden-
tified by the presence of contrast material around bowel
17 Urinary Tract
loops, between mesenteric folds and in the paracolic gut-
ters [231].
Treatment of extra-peritoneal bladder rupture is usually
conservative. Treatment of intraperitoneal bladder rupture
usually requires surgical repair.
Tumors and Malformations
Renal Tumors
Most abdominal masses in children arise from the kidney,
with hydronephrosis and multicystic dysplastic kidney
(MCDK) the most frequent diagnoses. Solid tumors are
much less common. Initial imaging usually includes ultra-
sound and an abdominal radiograph. Ultrasound can usually
determine the site of origin of the mass, whether it is cystic
or solid, and assess its vascularity.
If the mass arises from the kidney and is cystic, the dif-
ferential diagnosis is between a hydronephrotic kidney and
MCDK. The appearance of the cysts by ultrasound and, if
necessary, the presence of function on nuclear renography
can usually distinguish between these two diagnoses. If the
mass is solid and related to the kidney, a Wilms’ tumor is
most likely.
Tumor invasion of the renal vein or inferior vena cava and
the presence of metastases are determined during the ultra-
sound examination. Tumor staging is usually done with CT
or MR imaging. If the mass is related to another organ and
Fig. 17.114 Mesoblastic nephroma in an 8-month-old male. (a) Lon­
gitudinal grayscale ultrasound image demonstrates a large, homoge-
neous, solid mass arising from the hilum of the left kidney (K).
803
the kidney is normal, imaging evaluation will be tailored to
the relevant organ.
Benign Renal Tumors
Mesoblastic Nephroma
Mesoblastic nephroma is rare, accounting for only 3–6% of
all pediatric renal tumors, but is the most common solid renal
tumor in neonates and infants, generally presenting within
6 months of age [232]. It manifests clinically as a palpable
flank mass or, occasionally, with hypertension. Although meso-
blastic nephroma is usually considered a benign tumor, it can
spread through local invasion.
There are two main histologic subtypes of mesoblastic
nephroma: classic and cellular, with 10–20% of tumors com-
posed of a mixture of both patterns [233]. The cellular subtype
is more common (about two-thirds of cases), more aggressive,
tends to be larger, and occurs in older patients (>3 months of
age). Chromosomal abnormalities are only identified in the
cellular type [234].
On ultrasound, classic mesoblastic nephroma appears as a
homogeneous, solid mass with concentric hyperechoic and
hypoechoic rims surrounding the tumor, the so-called “ring”
sign [235]. This ring is vascular on color or spectral Doppler.
Although involvement of the renal hilum is frequent (Fig. 17.114),
there is no invasion of the renal vessels. Calcifications are uncom-
mon. The cellular subtype appears heterogeneous and often con-
tains fluid-filled spaces representing hemorrhage, necrosis, and
cyst formation [236, 237].
(b) Longitudinal color Doppler ultrasound image shows minimal inter-
nal tumor vascularity with prominent flow in the adjacent compressed
renal hilar vessels (arrowheads)
804 G. H. Kassab et al.

a b

Fig. 17.115 Bilateral renal angiomyolipomas in a 12-year-old male with tuberous sclerosis. Longitudinal grayscale ultrasound images of the right
(a) and left (b) kidneys reveal innumerable hyperechoic parenchymal nodules of varying size

Rarely, mesoblastic nephroma may appear predominantly
cystic. These cystic tumors are larger than the classic subtype
and frequently cross the midline due to a relatively aggres-
sive growth pattern. In addition, they have the propensity to
encase vessels and invade adjacent organs.
Surgery is usually curative. Approximately 10% of meso-
blastic nephromas will relapse, and the majority that relapse
are cellular in type. Recurrences typically occur within
1 year of initial diagnosis [237].
Angiomyolipoma
Angiomyolipoma is a benign solid tumor composed of vary-
ing proportions of mature adipose tissue, thick-walled blood
vessels, and smooth muscle. Approximately 80% of angio-
myolipomas are sporadic, with the remaining 20% occurring
in association with tuberous sclerosis as discussed earlier in
this chapter. Angiomyolipomas that occur in the setting of
tuberous sclerosis are usually bilateral, multifocal, and larger
in size than the sporadically occurring lesions [232, 238].
They are generally asymptomatic and discovered inciden-
tally, although large tumors can present with flank pain, full-
ness, and hematuria.
The vascular component of angiomyolipomas is
elastin-­poor and can lead to the formation of aneurysms,
which may bleed. The risk of retroperitoneal bleed-
ing depends on the size of the tumor. Masses exceeding
3.5 cm in diameter have a significantly higher risk of
bleeding [239].
Angiomyolipomas can be classified as “fat-rich,” “fat-
poor,” or “fat-invisible” based on their imaging appearance
and fat content. On ultrasound, a “fat-rich” lesion appears as
a highly echogenic (more echogenic than the renal sinus fat),
non-shadowing focus (Fig. 17.115). “Fat-poor” and “fat-
invisible” tumors can be missed on ultrasound since they
may be isoechoic to the renal parenchyma. They can some-
times be indistinguishable from a Wilms’ tumor or renal cell
carcinoma.
If CT and MR imaging fail to reveal the correct diag-
nosis, image-guided percutaneous renal biopsy may be
necessary despite the potential risk of bleeding [240]. Color
and spectral Doppler can depict pseudoaneurysms with their
characteristic “to-and-fro” flow. In patients with tuberous
sclerosis, routine ultrasound surveillance is recommended
for early detection of angiomyolipomas.
Symptomatic masses and masses greater than 4 cm are
treated. Treatment with mTOR inhibitors such as sirolimus
or everolimus are recommended for tumors larger than 3 cm
[241, 242]. Coil embolization is the treatment of choice for
hemorrhagic tumors, and partial nephrectomy is an option
when embolization fails.
17 Urinary Tract 805

Multilocular Cystic Renal Tumor

Multilocular cystic renal tumor is an umbrella term that
encompasses two histologically distinct but radiologi-
cally similar benign tumors arising from the metanephric
­blastema: cystic nephroma and cystic partially differenti-
ated nephroblastoma (CPDN) [243]. These tumors have
a bimodal age and sex distribution, being more common
in young males (3 months to 4 years of age) and adult
females (40–60 years of age) [244]. Tumors usually pres-
ent as a painless abdominal mass, or occasionally with
hematuria.
The DICER1 gene encodes an enzyme that is important
to RNA synthesis. Individuals with germline DICER1 muta-
tions are at increased risk for developing multiple benign
and malignant tumors. The typical renal manifestation of
a DICER1 mutation is a pediatric cystic nephroma distinct
from other lesions that can have a similar appearance, includ-
ing adult cystic nephroma, cystic Wilms’ tumor, and CPDN
[145]. Pediatric cystic nephromas, including those related to Fig. 17.116 Multilocular cystic nephroma in a 2-year-old male. Trans­
DICER1 mutations, occur equally in males and females, and verse grayscale ultrasound image shows a complex mass (cursors) con-
taining cysts of different sizes separated by thin septations
almost all are diagnosed in the first 4 years of life. Increasing
evidence indicates that most childhood cystic nephromas are
probably related to DICER1 mutations.
On ultrasound imaging, a pediatric renal cystic nephroma rare [246]. Clinical examination and ultrasound are per-
appears as a complex lesion that usually contains multiple formed for follow-­up evaluation.
thin septations and may abut or protrude into the renal
hilum (Fig. 17.116). A small amount of blood flow can usu- Metanephric Adenoma
ally be demonstrated within the septa. Herniation of the Metanephric adenoma is a purely epithelial tumor derived
tumor into the renal collecting system is a typical feature. from the metanephric blastema. It typically occurs in middle-­
Portions of the lesion may appear solid as many of the tiny aged females, although it also develops in children older than
cysts are below the resolution limits of ultrasound [234]. 14 months of age. It is frequently found incidentally, but can
Calcifications may sometimes occur. These lesions may be also present with a palpable mass, hematuria, and hyperten-
smaller and less complex in younger children. On cross-­ sion. In 12% of cases, metanephric adenoma is associated
sectional imaging, the septa typically enhance after con- with polycythemia [247]. Although these tumors are consid-
trast administration. ered benign, there have been a few cases where metastases
Although a pediatric cystic nephroma is a benign lesion, it have occurred [248].
cannot be readily distinguished from other cystic renal neo- On ultrasound, metanephric adenoma is typically well-
plasms, including a cystic Wilms’ tumor. Surgical resection defined and echogenic (Fig. 17.117) but can occasionally
is the treatment of choice for multilocular cystic renal tumor, contain cysts and hypoechoic foci from prior hemorrhage or
with nephron conserving surgery the preferred option when necrosis [249]. Calcifications are commonly seen. The tumor
the diagnosis is suspected preoperatively [245]. Although is hypovascular on Doppler evaluation [250].
surgical resection is generally curative, CPDN histology Although considered a benign tumor, there is potential
requires close imaging follow-up as it contains microscopic for malignant transformation. Treatment consists of nephron
blastemal elements, and local recurrence is possible albeit sparing surgery or partial nephrectomy [251].
806
Fig. 17.117 Metanephric adenoma in a 14-year-old male. (a) Longi­
tudinal grayscale ultrasound image shows a well-circumscribed, echo-
genic and homogeneous mass (arrow) arising from the upper renal pole.
Inflammatory Myofibroblastic Tumor
Inflammatory myofibroblastic tumor, also known as inflam-
matory pseudotumor, is a rare lesion composed of myofi-
broblasts and inflammatory infiltrates. Although originally
described in the lung, it has subsequently been described
in multiple extrapulmonary sites. It can develop following
solid organ and bone marrow transplantation. When it arises
in the urogenital tract, it usually originates in the urinary
bladder but can rarely occur in the kidney [252]. Although
it may present at any age, it is unusual in children.
Ultrasound findings of inflammatory myofibroblastic
tumor are nonspecific and can mimic malignant tumors such
as Wilms’ tumor and renal cell carcinoma (Fig. 17.118).
Surgical excision is curative with no risk of local recur-
rence [253].
Ossifying Renal Tumor of Infancy
Ossifying renal tumor of infancy is an extremely rare benign
tumor that arises from the papillary region of the renal
pyramid. Histology reveals an osteoid core, osteoblast-like
cells, and spindle cells. The tumor has a male predilection
and typically occurs in infancy [254]. It can present with an
abdominal mass and gross hematuria from extension into the
renal pelvis.
Ultrasound imaging demonstrates a heterogeneously
echogenic intrarenal mass with posterior acoustic shadow-
ing due to the osteoid component. Its appearance can closely
mimic that of a staghorn calculus [232, 255]. Intra-tumoral
blood flow can be seen with color Doppler.
G. H. Kassab et al.
(b) Longitudinal color Doppler ultrasound image depicts minimal vas-
cularity within the mass
Although it is considered benign, all reported cases of
ossifying renal tumor of infancy have been treated with par-
tial or total nephrectomy. Given its rarity and unknown natu-
ral course, long-term follow-up is recommended [256].
Primary Malignant Renal Tumors
Wilms’ Tumor
Wilms’ tumor is the most common renal tumor in the pedi-
atric age group, accounting for at least 90% of renal tumors
in this population [257]. It arises from pluripotential meso-
dermal precursors of the renal parenchyma and occurs most
often in children younger than 5 years of age, with a peak
incidence at age 2–3 years [258]. Wilms’ tumor is usually sol-
itary but can be multifocal and bilateral, and synchronous or
metachronus. Although most cases occur sporadically, there
is a known association between Wilms’ tumor and several
disorders, including hemihypertrophy, Beckwith-­Wiedemann
syndrome, and WAGR syndrome [259].
Children with Wilms’ tumor are usually asymptomatic and a
palpable abdominal mass is identified by a parent or caregiver.
Malaise, painless hematuria, and hypertension are other modes
of presentation. The lung is the most common site of tumor
metastasis followed by the liver. Bone metastases are rare [244].
At ultrasound, Wilms’ tumor appears as a heterogeneous
mass of renal origin with distortion and compression of
the surrounding renal parenchyma. Sharp margins formed
by the normal renal parenchyma stretched around the mass
give rise to the “claw” sign (Fig. 17.119). The mass may
17 Urinary Tract 807

a b

M M

c d

Fig. 17.118 Inflammatory myofibroblastic tumor in an 11-year-old female
on chronic immunosuppression after renal transplantation. Longitudinal
grayscale (a) and color Doppler (b) ultrasound images of the right-sided
renal allograft demonstrate a lobulated, iso-to-­hypoechoic solid mass (M) in
the renal sinus that displays minimal internal vascularity and peripherally
displaces the sinus vessels (arrowheads). (c) Longitudinal color Doppler
ultrasound image of the right iliac fossa reveals extension of the mass into
the ureter (U) with significant ureteral expansion and posterior displacement
(arrow) of the adjacent iliac vessels. (d) Coronal contrast-enhanced CT
image depicts extensive tumor involvement of the renal collecting system
and ureter (arrows)

contain hypoechoic and anechoic areas representing hem-
orrhage, necrosis, and/or cysts (Cineclip 17.2). Shadowing
echogenic calcifications can also occur. Color Doppler
imaging is used to assess for tumor invasion of the ipsilat-
eral renal vein and inferior vena cava, findings that affect
management (Fig. 17.120). Screening of the contralateral
kidney is also imperative because of the possibility of syn-
chronous or metachronous lesions (Fig. 17.121) [260].
Wilms’ tumor must be differentiated from neuroblastoma,
a common tumor that occurs in a similar location and age
808 G. H. Kassab et al.

a b

c d

Fig. 17.119 Wilms’ tumor in a 9-year-old male. (a) Longitudinal
grayscale ultrasound image reveals a large, heterogeneous mass (M)
replacing most of the left kidney with a rim of normal parenchyma
(asterisk) along its inferior margin, a “claw” sign. (b) Longitudinal
color Doppler ultrasound image reveals displacement (arrowheads) of
the normal renal hilar vessels around the mass. The mass appears rela-
tively avascular. (c) Longitudinal CEUS image reveals multiple
enhancing tumor nodules (asterisks) with a large avascular zone
(arrows). (d) Longitudinal contrast-enhanced CT image depicts near-
complete replacement of the left kidney by tumor with residual normal
parenchyma (arrow) along its inferoposterior margin

group. Features that suggest Wilms’ tumor rather than neu-
roblastoma include the “claw” sign described above, and
venous tumor invasion compared to vascular encasement
and displacement in neuroblastoma. Tumor that crosses the
midline and extends through neural foramina into the spi-
nal canal, and skeletal metastases are all features commonly
seen with neuroblastoma but not Wilms’ tumor [232, 234].
Calcification occurs in 15% of Wilms’ tumors compared to
80–90% of neuroblastomas [234].
17 Urinary Tract 809

a b

M
LK

c d

V
M
V C

RK

Fig. 17.120 Wilms’ tumor with invasion of the renal vein and IVC in
a 3-year-old female. (a) Longitudinal grayscale ultrasound image dem-
onstrates a large, echogenic mass (arrow) arising from the upper and
mid-portions of the left kidney (LK). (b) Transverse grayscale ultra-
sound image shows extension of the mass (M) into the left renal vein
(V). (c) Transverse color Doppler ultrasound image reveals compres-
sion and distortion of the IVC (arrow) by tumor in the left renal vein
(V). RK, Right kidney. (d) Axial contrast-enhanced CT image shows
infiltration of tumor (arrowhead) into the anterior aspect of the IVC (C).
M, Mass; V, left renal vein

a b c
L

Fig. 17.121 Bilateral Wilms’ tumors in a 3-year-old female. (a) Transverse sound image reveals a similar solid mass (arrow) in the mid and lower
grayscale ultrasound image shows a large, mildly heterogeneous mass portions of the left kidney (L). (c) Coronal contrast-enhanced CT image
(arrow) arising from the right kidney (R). (b) Longitudinal grayscale ultra- depicts bilateral heterogeneous, hypo-­enhancing renal masses (asterisks)
810
The role of renal biopsy in Wilms’ tumor is controversial
due to the risk of tumor seeding and unrepresentative sam-
pling. In most centers, if the age of the child and the appear-
ance of the mass are strongly suggestive of unilateral Wilms’
tumor, a biopsy is not performed.
In North America, management usually follows the
Children’s Oncology Group (COG) recommendations and
includes initial total nephrectomy followed by chemother-
apy, and in some cases radiotherapy. In Europe, children are
treated according to the International Society of Paediatric
Oncology (SIOP) protocol, with chemotherapy given prior
to surgical nephrectomy in order to reduce the size of the
tumor and the risk of intra-operative rupture. Bilateral
tumors are treated with neoadjuvant chemotherapy followed
by nephron-sparing surgery.
Nephrogenic Rests and Nephroblastomatosis
Nephrogenic rests are foci of persistent metanephric blastema
in the kidney after 36 weeks of gestation. When they are mul-
tiple or diffuse, they are referred to as nephroblastomatosis.
They are considered precursors for Wilms’ tumor and account
for 30–40% of unilateral and almost all bilateral Wilms’
tumors [251, 259]. In addition, they are associated with mul-
tiple genetic syndromes such as sporadic aniridia with or
without WAGR syndrome, Beckwith-Wiedemann syndrome,
hemihypertrophy, and Denys-Drash syndrome [244].
Nephrogenic rests may be perilobar or intralobar in distri-
bution. Perilobar rests are seen at the periphery of the renal
lobule, either in the columns of Bertin or in a subcapsular
location. Intralobar rests are found within the renal lobule
and may be located in the renal sinus, pelvocalyceal wall, or
deep in the cortex. Both types of rest may be focal, multifo-
cal, or diffuse. Patients with diffuse rests are usually less than
2 years of age and present with unilateral or bilateral flank
masses. Focal and multifocal rests are usually asymptomatic
and are incidentally discovered during a screening examina-
tion or at the time of imaging evaluation of Wilms’ tumor.
a b
Fig. 17.122 Diffuse nephroblastomatosis in a 22-month-old female. (a)
Transverse grayscale ultrasound image of the right kidney reveals multi-
ple nodular, hypoechoic, subcapsular masses (arrows). (b) Longitudinal
grayscale ultrasound image of the left kidney reveals a similar appearance
G. H. Kassab et al.
Nephrogenic rests can appear as rounded, ovoid, or
poorly defined foci of variable echogenicity compared to the
adjacent renal parenchyma (Fig. 17.122). Diffuse nephro-
blastomatosis manifests as renal enlargement, diffuse hyper-
echogenicity, and loss of corticomedullary differentiation.
The typical appearance of a peripheral rind of abnormal
tissue that displaces the normal renal parenchyma centrally
is usually better evaluated by CT or MR imaging than with
ultrasound [234].
Management of nephroblastomatosis in the absence of
Wilms’ tumor is controversial. While some institutions prefer
to give chemotherapy, others question its effectiveness and
instead recommend close follow-up with serial ultrasound
studies every 3 months up to the age of 8 years [244, 259]. A
child with nephroblastomatosis should also be screened for
associated syndromes.
Renal Cell Carcinoma
Renal cell carcinoma (RCC) is the second most common
pediatric renal malignancy after Wilms’ tumor, with an
incidence of four cases per million children. However, it is
more common than Wilms’ tumor in the second decade of
life with a median age of 9 years at diagnosis [261]. The
incidence of RCC increases with age with an equal gender
distribution [262].
Pediatric RCC differs from adult RCC and is considered
a separate entity. It is associated with von Hippel-Lindau
disease, tuberous sclerosis, and Beckwith-Wiedemann syn-
drome. The most frequent histologic subtypes of RCC in the
pediatric age group are translocation carcinoma, papillary
carcinoma, and medullary carcinoma, with chromophobe
and clear cell carcinoma being quite rare, whereas in adults,
papillary, chromophobe and clear cell carcinoma are the
most common subtypes [263]. In addition, spread of RCC
to the regional lymph nodes is more common in children and
is associated with a more favorable prognosis compared to
adults [261].
c
and distribution of subcapsular masses (arrows). (c) Axial contrast-
enhanced CT image shows multiple bilateral hypo-enhancing renal
masses surrounding and compressing the normally enhancing renal
parenchyma (arrowheads)
17 Urinary Tract
Clinical presentation includes macroscopic hematuria,
flank pain, palpable abdominal mass, anemia, and fever.
At ultrasound, RCC has a variable appearance. It may
appear as a hypo-, iso-, or hyperechoic intrarenal mass with
homogeneous or heterogeneous echotexture, depending on
the presence of calcification, necrosis, and hemorrhage. It
can be well-defined and surrounded by a pseudocapsule or
unencapsulated and infiltrating (Fig. 17.123). Some tumor
subtypes have a characteristic appearance: translocation
RCC has “egg-shell” calcifications mimicking benign renal
tumors [264]. RCC can invade retroperitoneal structures, the
renal vein, and the inferior vena cava. It can metastasize to
local or distant lymph nodes, lung, liver, and bones.
It is not possible to differentiate between Wilms’ tumor and
RCC by ultrasound, although the older age of the patient at
presentation is a helpful guide to the correct diagnosis [232].
Radical nephrectomy is the treatment of choice for RCC
in the pediatric population, with a possibility for nephron-
sparing surgery in small tumors [265]. This tumor is resis-
tant to chemotherapy and radiotherapy. Therefore, adequate
surgical resection and sampling of lymph nodes are critical.
Prognosis depends on tumor staging with the survival rate
ranging from >90% for stage I disease to <15% for stage IV
disease [262].
Rhabdoid Tumor
Rhabdoid tumor of the kidney is a rare aggressive neoplasm
that arises from the renal medulla and occurs exclusively
in children. It is mainly diagnosed in the first year of life,
accounting for fewer than 2% of all pediatric renal tumors,
and carries a poor prognosis [266].
Rhabdoid tumor has a unique association with synchro-
nous primary and secondary intracranial neoplasms in 15%
of cases, mainly primitive neuroectodermal tumor (PNET)
and rhabdoid tumor of the brain (known as atypical teratoid
rhabdoid tumor) [267].
a b
Fig. 17.123 Renal cell carcinoma in a 9-year-old female with painless
gross hematuria. (a) Longitudinal grayscale ultrasound image shows a
solid, mildly heterogeneous mass (arrows) arising from the mid-right
kidney. (b) Longitudinal grayscale ultrasound image shows the mass
811
Children with rhabdoid tumor commonly present with
hematuria from invasion of the renal collecting system,
hypercalcemia, or other symptoms related to metastasis. The
majority of patients present with advanced disease at the
time of diagnosis. The lungs, brain, and bones are the most
common sites of metastatic disease [268].
Rhabdoid tumor usually appears similar to Wilms’ tumor
on ultrasound, manifesting as a large, ill-defined, hetero-
geneous mass that usually extends into the renal hilum
(Fig. 17.124). A perinephric or subcapsular fluid collection
due to either hemorrhage or necrosis occurs in 50–70% of
rhabdoid tumors; however, it is nonspecific and can occasion-
ally be seen with other renal tumors as well [269]. Additional
features that indicate rhabdoid tumor include renal capsular
thickening, a multilobulated appearance, and curvilinear cal-
cification outlining the tumor lobules [270]. As with Wilms’
tumor, rhabdoid tumor can invade the renal vein and inferior
vena cava.
Rhabdoid tumor is very aggressive and has the worst prog-
nosis of any pediatric renal tumor, with overall survival rates
of only 20–25%. A worse outcome is expected with patients
under 1 year of age, those with central nervous system tumors
and metastatic disease. Treatment includes radical nephrec-
tomy and lymph node resection followed by chemotherapy.
The overall survival rate remains low even with aggressive
treatment [234].
Clear Cell Sarcoma
Clear cell sarcoma, an aggressive renal tumor arising from
the renal medulla, accounts for 4–5% of pediatric primary
renal neoplasms [267]. The peak incidence is at the age of
36 months with a male predominance (male to female ratio
2:1) [271]. The most common presentation is a palpable
abdominal mass; hematuria and bone pain are less frequent.
Clear cell sarcoma has a higher predilection for bone metas-
tasis compared to Wilms’ tumor and other pediatric renal
c
compressing but not invading the IVC (asterisk). The mass has a faintly
visualized, partially calcified rim (arrowhead). (c) Axial unenhanced
CT image clearly demonstrates the calcified rim (arrowheads) of the
tumor
812
Fig. 17.124 Rhabdoid tumor of the kidney in a 6-year-old male with a
history of atypical teratoid rhabdoid tumor (ATRT) of the brain. (a)
Longitudinal grayscale ultrasound image shows a hypoechoic mass
(arrows) arising from the lower pole of the kidney. (b) Coronal contrast-
Fig. 17.125 Clear cell sarcoma in a 4-year-old male. Sagittal grayscale
ultrasound image demonstrates a large heterogeneous mass arising
from the kidney that was a pathologically proven clear cell sarcoma
tumors. Additional common sites for metastasis include the
lymph nodes, brain, liver, and lungs [263].
Ultrasound features of clear cell sarcoma are nonspe-
cific, appearing as a predominantly solid, heterogeneous
renal mass involving the renal medulla and containing areas
of cystic change (Fig. 17.125). Occasionally, large cystic
spaces with echogenic septa similar to multilocular cystic
renal tumor are the predominant feature [272].
Clear cell sarcoma is difficult to treat and has a worse
prognosis than Wilms’ tumor, especially in infants. Radical
G. H. Kassab et al.
enhanced, fat-suppressed, T1-weighted MR image of the brain obtained
when the patient was 4 months of age reveals an enhancing midline mass
(arrow) that was a pathologically proven ATRT. There is associated
hydrocephalus (asterisks)
nephrectomy with lymph node dissection followed by che-
motherapy and sometimes radiotherapy are the cornerstones
of therapy. Even with aggressive treatment, relapse occurs
in 20–40% of cases. However, with recent intense chemo-
therapy regimens, 5-year overall survival has improved from
25% to 86% [273].
Renal Medullary Carcinoma
Renal medullary carcinoma is a rare aggressive renal tumor
that has a strong association with sickle cell trait [274]. It
occurs in older children with a mean age of 15 years and
affects males three times more often than females in the pedi-
atric age group. It presents clinically with gross hematuria and
flank pain, and less often with an abdominal mass and fever.
Ultrasound demonstrates a large, heterogeneous, centrally
located mass with ill-defined margins infiltrating the cortex
and renal sinus. The tumor characteristically expands the
kidney while maintaining its reniform shape and causes focal
caliectasis without pelviectasis. Extensive necrosis is a com-
mon feature in medullary carcinoma, whereas calcifications
are rare. The tumor is hypovascular on Doppler ultrasound
[275]. There is a high propensity for invasion of vessels
and perinephric fat, and involvement of the regional lymph
nodes. Metastases are frequently found at the time of diagno-
sis, particularly in the liver and lung.
Medullary carcinoma is resistant to chemotherapy and
radiotherapy with a mean survival of 4 months after surgery.
Primitive Neuroectodermal Tumor
Primitive neuroectodermal tumor (PNET), an aggressive
small round cell tumor, accounts for fewer than 1% of pedi-
17 Urinary Tract
a b
Fig. 17.126 Acute leukemic infiltration in a 13-year-old male. Longitudinal grayscale ultrasound images of the right (a) and left (b) kidneys
reveal diffuse enlargement with increased cortical thickness and echogenicity
atric renal tumors. It has a characteristic chromosomal trans-
location, t(11;22)(q24;q12) [276]. Based on its histological
appearance, it is classified with the Ewing family of tumors.
Affected children present with flank pain, a mass and hema-
turia, or less frequently, with systemic symptoms. Metastasis
to liver, lung, and/or bones is commonly present at the time
of diagnosis.
At ultrasound, a PNET appears as an ill-defined hypo- or
isoechoic mass nearly replacing the kidney and frequently
containing areas of hemorrhage, necrosis, and occasionally
calcification. Extension into the inferior vena cava can also
occur [262].
As PNET typically presents at an advanced stage, the
prognosis is very poor. Treatment involves nephrectomy fol-
lowed by chemotherapy and radiotherapy [277].
Other Rare Primary Malignant Renal Tumors
Synovial cell sarcoma is a very rare malignant renal tumor
affecting older children and young adults. Although synovial
cell sarcoma predominantly affects the extremities, primary
renal synovial cell sarcoma has been described as a separate
entity.
The ultrasound appearance is of a well-circumscribed
solid mass with prominent areas of necrosis, hemorrhage,
and cysts. Additional features include subcapsular hemor-
rhage and renal vein invasion. Lymphadenopathy is usually
absent.
Treatment consists of radical nephrectomy and adjuvant
chemotherapy. Recurrence is common [263].
Secondary Malignant Renal Tumors
Leukemia
Renal leukemic infiltration can occur in children with acute
lymphoblastic leukemia, the most common malignancy in
813
children. The presence of renal leukemic involvement does
not usually cause significant renal dysfunction and is gener-
ally asymptomatic.
Renal involvement by leukemia can manifest on ultra-
sound in a variety of ways: as diffuse infiltration with bilateral
nephromegaly, loss of normal corticomedullary differentia-
tion, or as wedge-shaped or geographic zones of abnormal
echogenicity (Fig. 17.126). Unilateral findings and the pres-
ence of a focal renal mass are rare [232, 278].
Treatment includes supportive measures for renal fail-
ure while specific therapy is targeted to the underlying
disease.
Lymphoma
Primary renal lymphoma is very rare due to the absence of
an intrinsic renal lymphatic system. However, the kidneys
are the most frequently affected abdominal organs in lym-
phoma, especially in non-Hodgkin lymphoma, with sec-
ondary renal involvement occurring in 8% of cases. This
involvement is due to hematogenous spread or, less com-
monly, by direct renal infiltration from diseased retroperito-
neal lymph nodes.
Clinically, renal involvement is usually masked by the
­extensive symptoms of the primary disease. Nevertheless,
detection of renal involvement is important because wide-
spread lymphomatous infiltrates can replace the renal paren-
chyma and compress the collecting system, resulting in acute
renal failure [279].
On ultrasound, there are different patterns of renal
involvement in lymphoma. These include solitary or mul-
tiple renal masses (Fig. 17.127), generalized renal enlarge-
ment from diffuse infiltration (Fig. 17.128), or occasionally
isolated perinephric disease. There can be direct renal inva-
sion from adjacent retroperitoneal structures, with a high
propensity for vascular and ureteral encasement. Ultrasound
814
a b
Fig. 17.127 Renal involvement in 4-year-old male with Burkitt lym-
phoma. (a) Longitudinal grayscale ultrasound image demonstrates mul-
tiple mildly echogenic masses (arrowheads) in the right kidney. The left
Fig. 17.128 Diffuse renal infiltration by lymphoma in a 2-year-old
female. Longitudinal grayscale ultrasound image reveals an enlarged
kidney with extensive cortical involvement by echogenic soft tissue
that spares the renal pyramids. A minimal amount of residual normal,
relatively hypoechoic renal parenchyma (arrowheads) is present in
the upper and lower renal poles
evaluation is therefore necessary before initiating chemo-
therapy to exclude renal involvement [280].
Management of secondary lymphoma requires treat-
ment of the primary disease, generally with chemotherapy
and radiotherapy. Impaired renal function typically returns
G. H. Kassab et al.
kidney had a similar appearance (not shown). (b) Coronal contrast-
enhanced CT image shows numerous bilateral, nonenhancing solid renal
masses
to normal after the initiation of treatment. All children with
lymphomatous involvement of the kidneys require careful mon-
itoring of renal function [281].
Metastases
Renal metastases are unusual in children, although they can
occur in the setting of osteosarcoma and neuroblastoma.
Osteosarcoma
Renal metastasis in osteosarcoma is very rare and develops
long after initial treatment. Metastases are frequently calci-
fied and can affect one or both kidneys. Ultrasound demon-
strates masses in the kidneys, with or without calcification
(Fig. 17.129). Bone scintigraphy can detect renal metastases
in 90% of cases. Renal metastasis in osteosarcoma carries a
very poor prognosis, although early detection and possibly
resection can increase survival [282].
Neuroblastoma
Renal invasion by neuroblastoma is relatively common and
occurs in about 20% of cases of abdominal neuroblastoma
[279]. When adrenal neuroblastoma invades the kidney, it can
appear as an intrarenal mass and may be confused with Wilms’
tumor (Fig. 17.130). Features that differentiate neuroblastoma
from Wilms’ tumor are discussed in the Wilms’ tumor section.
17 Urinary Tract
a b
Fig. 17.129 Renal metastasis from osteosarcoma in a 9-year-old male.
(a) Longitudinal grayscale ultrasound image reveals a large, heteroge-
neous mass (arrow) in the lower pole of the kidney. The mass is not
a b
L
Fig. 17.130 Renal metastasis from neuroblastoma in a 5-month-old
male. (a) Longitudinal grayscale ultrasound image of the right upper
quadrant of the abdomen shows the primary right adrenal gland neuro-
blastoma (arrow). There is a hypoechoic metastatic liver lesion (arrow-
head). L, Liver; K, right kidney. (b) Longitudinal grayscale ultrasound
image of the left kidney reveals a small, anechoic structure (arrowhead)
Primary Ureteral Tumors
Primary ureteral tumors can be benign or malignant, and
both are very rare in the pediatric population.
Fibroepithelial Polyp
Fibroepithelial polyp of the ureter is a benign ureteral neoplasm.
Histologically, it is composed of a fibrous stroma covered by
transitional urothelium. The most common presenting symptom
815
ossified. (b) Coronal contrast-enhanced, T1-weighted, fat-suppressed
MR image obtained 2 years earlier shows the enhancing primary tumor
(arrow) of the distal femur
c
in its mid-portion. (c) Coronal contrast-enhanced CT image shows the
nonenhancing mid-left renal lesion (arrow). This lesion regressed after
initiation of chemotherapy, consistent with a metastasis. The adrenal
tumor (asterisk), mesenteric nodal and hepatic metastases (arrowheads)
are demonstrated
is hematuria. Intermittent or recurrent pain may also occur as a
result of torsion, intussusception, or intermittent obstruction.
Preoperative imaging diagnosis of fibroepithelial polyp is
difficult. Ultrasound has a limited role, and CT urography or
MR urography is often required. A fibroepithelial polyp usu-
ally appears as a smooth mass attached to the ureteral wall.
When a definite preoperative diagnosis is not achieved, intra-
operative biopsy is performed with frozen section interpreta-
tion in order to avoid unnecessary ureteronephrectomy [283].
816
Urothelial Tumor
Urothelial tumor is the most common primary malignant
neoplasm of the ureter. It rarely occurs in isolation, and usu-
ally presents as an extension of a renal pelvic or bladder uro-
thelial tumor.
Secondary Ureteral Tumors
Extension of Wilms’ Tumor
While invasion of the renal collecting system in Wilms’
tumor is common, caudal extension into the ureter is unusual,
occurring in only 2% of cases [284]. Ureteral involvement
presents with gross hematuria, hydronephrosis, and/or a non-
functioning kidney.
Ureteral extension of Wilms’ tumor may be difficult to detect
with ultrasound. The finding of hydronephrosis or a nonfunc-
tioning kidney should suggest this possibility (Fig. 17.131).
A diagnosis of ureteral involvement can usually be made pre-
operatively with CT although cystoscopy with a retrograde ure-
terogram may be needed for definitive diagnosis. Preoperative
recognition of ureteral extension is important to allow complete
excision of the involved ureter at the time of nephrectomy and
avoid the need for repeat surgery or radiotherapy.
Tumors with ureteral extension are more resistant to treat-
ment and may require postoperative cystoscopic surveillance
to detect recurrence [285].
Fig. 17.131 Wilms’ tumor extending into the renal pelvis and upper
ureter in a 4-year-old male with gross hematuria. (a) Longitudinal gray-
scale ultrasound image of the right kidney demonstrates a solid, hetero-
geneous mass (arrow) in the upper pole. Echogenic material is present
G. H. Kassab et al.
Bladder Malformations and Tumors
Vascular Malformations
Lymphatic Malformation
Lymphatic malformation of the urinary bladder is exceedingly
rare, with very few cases reported in the literature. It presents
with painless macroscopic hematuria, dysuria, or a palpable pel-
vic mass. The lesion is often multilobulated in contour and can
grow into the bladder lumen, infiltrate the inner wall, protrude
through the full thickness of the bladder wall or grow from the
outer wall of the bladder and extend into the peritoneal cavity.
It usually appears as a multiseptated, cystic mass by ultrasound
that is avascular or shows only a small amount of blood flow
in the internal septations (Fig. 17.132). Treatment may require
partial cystectomy or transurethral resection [286, 287].
Venous Malformation
Venous malformation of the urinary bladder is extremely rare.
It usually presents with painless hematuria. It can be associ-
ated with Klippel-Trenaunay syndrome which is characterized
by port-wine stain skin lesions, varicose veins, hypertrophy
of bone and soft tissues, and complex low flow vascular
malformations.
Ultrasound shows a mass of multiple channels with slow
flow on color Doppler evaluation (Fig. 17.133). Phleboliths are
detected as echogenic foci with posterior shadowing [287].
in the renal pelvis (asterisk) and distended upper ureter (arrowheads).
Axial contrast-enhanced CT images show (b) the heterogeneous renal
mass (arrow) with (c) extension into the renal pelvis (asterisk) and
upper ureter (arrowhead)
17 Urinary Tract 817

a b

Fig. 17.132 Lymphatic malformation impinging on the bladder in a rior to the bladder (B). (b) Longitudinal STIR MR image of the pelvis
10-year-old male. (a) Transverse grayscale ultrasound image of the pel- shows the lesion (arrow) surrounding and deforming the bladder (B)
vis shows a cystic lesion (arrows) with multiple thin septations poste-

a b c

B
B

d e f

B B

Fig. 17.133 Venolymphatic malformation involving the bladder dome
in a 16-year-old male with Klippel-Trenaunay syndrome. (a) Transverse
grayscale ultrasound image of the bladder (B) shows a multicystic lesion
(arrow) with thin septations in the anterior wall. (b) Longitudinal gray-
scale ultrasound image of the bladder (B) reveals focal lobulated thick-
ening (arrows) of the posterosuperior wall. (c) Longitudinal color
Doppler ultrasound image shows a blood vessel (arrowhead) extending
to the zone of bladder wall thickening. (d) Spectral Doppler analysis of
the vessel in (c) shows venous waveforms. Longitudinal T2-weighted,
fat-suppressed (e) and contrast-enhanced, T1-weighted, fat-suppressed
(f) MR images show extensive pelvic involvement by a complex malfor-
mation with a multicystic, nonenhancing component anterosuperior to the
bladder (arrows) consistent with lymphatic malformation; and multiple
enhancing tubular structures (arrowheads) in keeping with venous malfor-
mation that extend along the posterosuperior aspect of the bladder (B)
818
Treatment depends on the size of the lesion and associ-
ated clinical manifestations, and may include conservative
management, cystoscopic fulguration using laser or electro-
cautery, sclerotherapy, and/or excision [287].
Benign Bladder Tumors
Urothelial Papilloma
Urothelial papillomas are benign polypoid neoplasms that
usually develop in middle-aged men, although they can rarely
occur in children. Histologically, they consist of a fibrovas-
cular core covered by normal urothelium with no cytological
atypia.
Urothelial papillomas appear on ultrasound as frond-like
lesions, which tend to occur near the ureteric orifices or along
the posterior bladder wall. However, this appearance is not
pathognomonic [288].
Treatment consists of transurethral excision. Ultrasound
follow-up is necessary due to their tendency to recur.
However, owing to the rarity of this tumor in children, there
is currently no standardized time interval for follow-up [289].
Papillary Urothelial Neoplasm of Low Malignant
Potential
A papillary urothelial neoplasm of low malignant poten-
tial (PUNLMP), previously known as grade I urothe-
lial carcinoma, is a very rare bladder lesion in children.
PUNLMPs are similar to exophytic urothelial papillomas
but have increased cellular proliferation; they are nonin-
vasive, and do not metastasize. They frequently arise from
the posterolateral wall and ureteral orifice of the bladder
[290, 291].
On ultrasound, a PUNLMP appears as a dendriform lesion
(resembling a tree), measuring 1–2 cm, and may have inter-
nal vascularity. It has been described as having the appear-
ance of “seaweed in the ocean.” Differentiating a PUNLMP
from a noninvasive urothelial carcinoma is difficult, and 11%
of cases may increase in size if left untreated.
There are no standardized guidelines for PUNLMP man-
agement and follow-up. The majority of cases are treated by
transurethral resection. Intravesical chemotherapy has shown
promising results, especially in pediatric patients with high-
risk relapsed PUNLMP, increasing the chance of complete
disease remission and bladder sparing [292].
G. H. Kassab et al.
There is a high risk of recurrence after resection (35%);
therefore, follow-up imaging is necessary, preferably with
ultrasound [291, 293].
Fibroepithelial Polyp
Fibroepithelial polyp is a benign urothelial lesion that tends
to occur at or near the bladder neck in children. The mean
age of presentation is 9 years with a strong male predilection
[294]. The majority of these lesions are solitary and smaller
than 5 cm; however, they may occasionally grow in size and
can undergo torsion [295]. The clinical symptoms include
gross hematuria and flank pain. They can produce severe uri-
nary tract obstruction, and a definitive diagnosis can only be
made by pathologic examination.
On histology, fibroepithelial polyps appear as papillary
fronds lined by normal-appearing urothelium without cel-
lular atypia. There may be focal areas of ulceration [296].
Ultrasound shows a pedunculated mass with flow on color
Doppler evaluation (Fig. 17.134). However, this appearance
is nonspecific and can be seen with other pathologic blad-
der lesions, including urothelial papilloma and botryoid-type
rhabdomyosarcoma [296].
These lesions are usually treated with transurethral endo-
scopic resection.
Inflammatory Myofibroblastic Tumor
Inflammatory myofibroblastic tumor, as previously described,
is a rare, benign lesion that can occur in every organ in the
body, including the bladder, where it manifests clinically
with hematuria and abdominal pain. These tumors are usu-
ally located at the bladder dome and tend to spare the trigone.
They are usually sizeable at diagnosis, with a mean diameter
of 5.5 cm [297].
At ultrasound, an inflammatory myofibroblastic tumor
of the bladder appears as a solitary exophytic or pol-
ypoidal mass that may contain cystic foci and surface
ulceration. Color Doppler will show internal vascularity
(Fig. 17.135). These tumors can be locally invasive and
have a substantial extravesical component, making dif-
ferentiation from a malignant lesion impossible without
tissue biopsy [298].
The biological behavior of inflammatory myofibroblas-
tic tumors of the bladder in children is unknown, although
they are known to recur after resection in adults [297, 298].
17 Urinary Tract 819

Fig. 17.134 Fibroepithelial polyp in the bladder of a 14-year-old ultrasound image shows a small amount of blood flow (arrowhead) at
male. Longitudinal (a) and transverse (b) grayscale ultrasound images the base of the lesion. At surgery, a polyp was noted to be arising from
show a small pedunculated mass (arrows) in the region of the bladder the prostatic urethra with prolapse through the bladder neck into the
neck that protrudes into the bladder lumen. (c) Transverse color Doppler bladder lumen

women aged 30–60 years [297]. It can present clinically

with frequent urination, dysuria, hematuria, and/or urinary
obstruction. Bladder leiomyomas can be intravesical (most
commonly), intramural, or extravesical in location.
On ultrasound, a bladder leiomyoma usually appears as
a solid, homogeneous, hypoechoic mass, similar to a uterine
fibroid. It can have a heterogeneous appearance due to the pres-
ence of cystic areas related to prior hemorrhage and necrosis.
Tissue sampling is needed to confirm the diagnosis and to
exclude an underlying leiomyosarcoma. Excision is consid-
ered curative, with no risk of local recurrence.

Neurofibroma
Neurofibroma is a benign nerve sheath tumor that can occur
in the urinary bladder, although it is rare. Approximately
75% of cases are identified in patients younger than 18 years
of age [299]. Neurofibromas of the bladder are usually plexi-
form in type. They are sometimes identified incidentally and
may be the initial manifestation of neurofibromatosis type I.
A neurofibroma appears as a focal solid mass or dif-
fuse bladder wall thickening on ultrasound (Fig. 17.136).
Distribution of the lesion along the neurovascular bundle is a
typical feature [300].
Fig. 17.135 Inflammatory myofibroblastic tumor of the bladder in a Surgical debulking of the lesion can be performed to
12-year-old female. Longitudinal color Doppler ultrasound image dem- alleviate bladder pressure symptoms. Due to the infiltrative
onstrates a plaque-­like mass arising from the bladder dome with inter-
nal vascularity nature of plexiform neurofibromas, total resection may not
be possible.
Treatment approaches include expectant management, high-­
dose steroids, radiotherapy, and surgery. Paraganglioma
Paraganglioma is a tumor of extra-adrenal origin that arises
Leiomyoma from the embryonic rests of chromaffin cells in the sympa-
Bladder leiomyoma is a rare mesenchymal tumor with very thetic plexus of the bladder detrusor muscle. Genitourinary
few cases reported in children, more typically occurring in paraganglioma is very rare, with the urinary bladder the
820
Fig. 17.136 Plexiform neurofibroma of the bladder in a 6-year-old
male with neurofibromatosis type I. Transverse (a) and longitudinal (b)
grayscale ultrasound images of the bladder demonstrate marked thick-
ening of the posterior wall (arrows). A catheter (arrowheads) is present
within the bladder. (c) Longitudinal color Doppler ultrasound image
most commonly affected organ. This tumor is usually
diagnosed in middle-aged adults but can occur in children
as young as 10 years of age. It is usually benign and hor-
monally active. A history of micturition-related syncope,
hypertension, or headache should raise suspicion for para-
ganglioma [301, 302].
Ultrasound reveals an intramural soft tissue lesion that is
usually located in the lateral or posterior bladder wall and
is hypervascular on color Doppler imaging (Fig. 17.137).
The presence of increased tracer activity on iodine-131-­
metaiodobenzylguanidine (MIBG) or fluorodeoxyglucose
(FDG) positron emission tomography (PET) scanning will
confirm the diagnosis.
The treatment of choice is surgical excision, which is usu-
ally curative. Special precautions are required to avoid an
intraoperative hypertensive crisis [303].
G. H. Kassab et al.
reveals hyperemia of the thickened portion of the bladder wall. (d)
Longitudinal T2-weighted, fat-suppressed MR image shows marked
thickening of the bladder wall (arrow). A catheter (arrowhead) is pres-
ent within the bladder
Nephrogenic Adenoma
Nephrogenic adenoma of the urinary bladder is not a true neo-
plastic process, but rather represents a metaplastic change in
the urinary bladder with papillary and tubular growth histologi-
cally similar to renal tubules. It is not a premalignant lesion and
typically affects the lamina propria with sparing of the muscle
layer. These lesions are exceedingly rare, with approximately
10% of cases occurring in the pediatric population [304].
Metaplasia can result from chronic irritation by calculi,
infection, injury, or previous surgery, especially renal trans-
plantation. Frequent urination and hematuria are the most
common symptoms.
On imaging, a nephrogenic adenoma appears as an intra-
vesical sessile or polypoid mass (Fig. 17.138). Its appearance
is nonspecific, and tissue biopsy is required to differentiate it
from other urothelial neoplasms.
17 Urinary Tract 821

a b

c d

Fig. 17.137 Bladder paraganglioma in a 10-year-old male with a suc- Longitudinal color Doppler ultrasound image reveals marked hypervas-
cinate dehydrogenase B subunit germline mutation. Transverse (a) and cularity of the mass. (d) Color Doppler ultrasound image of the lesion
longitudinal (b) grayscale ultrasound images show a small, echogenic with spectral analysis shows both arterial flow (above baseline) and
mass (arrows) arising from the right posterolateral bladder wall. (c) venous flow (below baseline)

Optimal treatment includes endoscopic resection along with
treatment of the underlying lesions (e.g., calculi). Nephrogenic
adenomas carry a high potential for recurrence (60%), so close
clinical follow-up with urinary cytology and cystoscopy is rec-
ommended [305].
Malignant Bladder Tumors
Rhabdomyosarcoma
Rhabdomyosarcoma is the most common soft tissue sarcoma
of childhood with the bladder and prostate the most frequently
822 G. H. Kassab et al.

Fig. 17.138 Nephrogenic adenoma in a 9-year-old female. Transverse

color Doppler ultrasound image shows a sessile isoechoic mass (arrow) Fig. 17.139 Rhabdomyosarcoma of the bladder in a 12-year-old male.
along the posterior bladder wall that demonstrates minimal vascularity Longitudinal grayscale ultrasound image shows a multilobulated intra-
vesical mass (arrows). There is an intraluminal catheter (arrowhead)
with associated distal shadowing
affected sites in the urinary tract [306]. Bladder tumors show a
male predominance (3:1), and there is a bimodal age distribu-
tion (within the first 2 years of life and during adolescence)
[307]. Although most cases occur sporadically, rhabdomyo-
sarcoma is associated with genetic syndromes, including Li-­
Fraumeni cancer syndrome, neurofibromatosis type 1, and
multiple endocrine neoplasia type 2A.
The histopathologic subtypes of rhabdomyosarcoma
include embryonal rhabdomyosarcoma (the most common
form, 90% of cases), botryoid, alveolar, and undifferentiated
types (the rarest and most aggressive). Histologically, all
forms of rhabdomyosarcoma contain malignant spindle cells
intermixed with loose myxoid and edematous stroma with a
highly cellular zone (the cambium layer) located beneath the
urothelium. Fig. 17.140 Rhabdomyosarcoma of the bladder in an 18-month-old
male. Longitudinal color Doppler ultrasound image reveals a multilob-
Metastatic disease at the time of diagnosis is common
ulated vascular mass (asterisks) in the region of the bladder neck. The
(10–20% of cases), mainly to the lungs, bones, and lymph bladder contains a small amount of layering, echogenic debris
nodes [297]. Affected children usually present with hematu- (arrowhead)
ria, dysuria, urinary tract infection, and/or urinary retention.
On ultrasound evaluation, rhabdomyosarcoma can man-
ifest either as a diffusely infiltrative lesion with focal and and increased the survival rate to 70%. Favorable prognostic
irregular wall thickening, or as an echogenic, pedunculated, factors include low stage, botryoid pattern, and embryonal
“grape-like” mass projecting into the urinary bladder lumen histology with absence of metastasis at the time of diagnosis.
(sarcoma botryoides). The tumor often arises from the uri- After completing therapy, bladder surveillance for at least
nary bladder neck or at the trigone (Figs. 17.139 and 17.140), 5 years is required to monitor for recurrence, which develops
and can result in obstruction and hydronephrosis [308]. It is in 30% of cases [309].
often locally invasive, distorting the bladder wall, and may
have a significant extravesical component. Enlarged regional Transitional Cell Carcinoma
metastatic lymph nodes may be identified. Transitional cell carcinoma (TCC), also known as urothelial
Bladder rhabdomyosarcoma is initially treated with che- carcinoma, is rare in children and has a very different clini-
motherapy, which has decreased the need for radical surgery cal course compared to adults. It usually occurs in adoles-
17 Urinary Tract
cents with the majority being low grade, solitary, and rarely
involving the upper urinary tracts [310].
In patients with a history of bladder augmentation, there
is an increased risk of developing TCC, especially at the site
of intestine-bladder anastomosis. In this setting, the tumors
tend to be high grade and have an aggressive clinical course
[311]. Young adult survivors of retinoblastoma and chil-
dren with childhood cancer–predisposing syndromes, such as
Costello syndrome and hereditary nonpolyposis colorectal
cancer syndrome, are also more prone to develop urothelial
carcinoma [312].
On ultrasound, TCC appears similar to other urothelial
bladder lesions with preferential involvement of the trigone
and ureteric orifice (Fig. 17.141).
Low-grade TCC is treated with transurethral resec-
tion. Partial cystectomy is reserved for more advanced
and c­ omplicated cases [310]. Although the risk of recur-
rence is low in children, the presence of multiple tumors
at the time of diagnosis increases this risk. Regular
follow-up ultrasound examinations are recommended
although their frequency and length of follow-up are not
standardized [313].
a b
Fig. 17.141 Transitional cell carcinoma of the bladder in a 14-year-
old female. (a) Transverse grayscale ultrasound image demonstrates an
echogenic polypoid mass with multiple frond-like extensions arising
823
Leiomyosarcoma
Leiomyosarcoma is a malignant bladder tumor rarely seen
in the pediatric population, although it has been reported
in children with a history of systemic chemotherapy with
cyclophosphamide and/or radiotherapy for another neoplasm
[314]. The majority of cases are high grade at presentation
and locally aggressive with an increased risk of local recur-
rence and distant metastasis.
It can be difficult to distinguish leiomyoma from leio-
myosarcoma at imaging, including ultrasound evaluation.
However, leiomyosarcoma tends to be larger in size with ill-­
defined margins, locally invasive, and more internally het-
erogeneous due to necrosis.
Radical cystectomy is the treatment of choice, although
some patients are managed with partial cystectomy. Systemic
chemotherapy and radiotherapy are used for unresectable
tumors [298].
Angiosarcoma
Angiosarcoma is an exceedingly rare vascular soft tissue sar-
coma in the pediatric population, with different biological
behavior in children compared to adults. In adults, angio-
from the trigonal region of the posterior bladder wall. (b) Transverse
color Doppler ultrasound image reveals mild central hypervascularity
824
sarcoma commonly occurs in the head and neck, whereas in
children it tends to arise in the skin, deep tissues, and internal
organs, including the bladder [315]. The cause of angiosar-
coma is unknown, although certain genetic mutations have
been identified in adult patients. The tumor is very aggres-
sive and can spread to the lungs, liver, and bones.
There are no documented descriptions of the ultrasound
features of angiosarcoma to date, including bladder involve-
ment, although angiosarcoma can clinically mimic heman-
gioma [315].
Treatment options include surgical resection, chemother-
apy, radiotherapy, and more recently, biological treatments
such as angiogenesis inhibitors for high-grade angiosarcoma.
Urethral Tumors
Urethral Polyp
Fibroepithelial urethral polyp is a benign entity and a rare
cause of urethral obstruction in males. It usually originates in
the prostate and projects into the urethra. It manifests in the
neonatal period with hematuria or obstructive urinary symp-
toms. The polyp is pedunculated and attached to the veru-
montanum via a stalk, which allows it to prolapse through
the prostatic urethra or to extend through the bladder neck
into the bladder (Fig. 17.134). Ultrasound has a very limited
role in diagnosing urethral lesions, and VCUG is generally
required. The polyp typically appears as a smooth lesion
extending into the midpoint of the bulbar urethra during
voiding [316].
Urinary Diversion
Urinary diversion is a surgical procedure to redirect the stream
of urine. The majority of children requiring this procedure
have myelodysplasia with a neurogenic bladder. Other indi-
cations include severe vesicoureteral reflux in neonates with
febrile urinary tract infection, posterior urethral valves, and
bilateral ectopic ureters. Patients with bladder exstrophy and
cloacal malformation require more complex urinary recon-
struction, which has been discussed earlier in this chapter.
Urinary diversion can be temporary or permanent.
Urinary diversions are subdivided into two categories: con-
tinent and incontinent. A continent urinary diversion maintains
the continence mechanism by using a continent catheterizable
channel. This channel provides an alternative route for bladder
drainage with or without bladder augmentation or reconstruc-
tion of the bladder neck [317].
With an Indiana pouch diversion, the cecum and terminal
ileum are used to augment the bladder. A short segment of
the terminal ileum is used as an efferent limb to facilitate easy
catheterization. The continence mechanism is provided by the
G. H. Kassab et al.
ileocecal valve. With a Mitrofanoff appendicovesicostomy, the
urinary bladder is augmented with a segment of small bowel,
and the appendix is used as a conduit to the abdominal wall.
In circumstances where the appendix is not available or appro-
priate for use in urinary diversion, another option includes the
Monti procedure where a segment of ileum measuring 2–2.5 cm
is used to construct a tube that will accommodate passage of a
16–18F catheter. The tube is implanted into the bladder in an
antirefluxing fashion, and the reservoir is fixed to the anterior
abdominal wall with the stoma often positioned at the umbilicus.
With incontinent urinary diversions, urine drains continu-
ously through a cutaneous ostomy. Incontinent diversions
are usually created as a temporizing measure to protect renal
function until a definitive continent urinary diversion can
be performed. A cutaneous vesicostomy is sometimes per-
formed in infants with recurrent febrile UTI, progressively
worsening hydronephrosis with decreasing renal function,
and outlet obstruction (e.g., in patients with posterior ure-
thral valves who are not candidates for cystoscopy) [317].
Ileal conduits are generally reserved for children too young
to undergo continent urinary diversion, patients without fam-
ily support, and those with poor manual dexterity. These con-
duits are associated with a high complication rate and renal
deterioration in children, and are ­therefore typically reserved
for patients with no other options for urinary diversion.
Augmentation cystoplasty is often performed in chil-
dren undergoing reconstruction of a catheterizable channel.
It improves bladder compliance and capacity and provides
an option for reconstruction using the native bladder and a
continent catheterizable channel to achieve continence. A
variety of intestinal segments can be used, including gastric,
jejunal, ileal, and colonic. The most commonly used tech-
nique is ileocystoplasty because the ileum has been found to
be the most compliant and least contractile.
On ultrasound imaging, an Indiana pouch appears as an
irregular, fluid-filled structure in the right lower quadrant
with gut signature (a hypoechoic outer wall with echo-
genic inner mucosa); haustra are sometimes evident as well.
Echogenic debris from intestinal secretions may also be seen
(Fig. 17.142). With a Mitrofanoff appendicovesicostomy,
a fluid-filled tubular structure, the appendix, can be traced
from the dome of the urinary bladder to the anterior abdomi-
nal wall (Fig. 17.143).
When assessing an augmented bladder, the thick-walled
augmented segment is often undulating in contour even when
the bladder is full. It is located superior to the native bladder,
which is often greatly trabeculated and irregular in appear-
ance, reflecting the underlying bladder dysfunction [79].
Complications of the Indiana procedure include inconti-
nence, stricture, stones, and vesicoureteral reflux. Complications
of the Mitrofanoff and Monti procedures are similar, including
stomal stenosis, leakage, conduit stricture, angulation of the
conduit, difficulty with catheterization, and prolapse. There are
17 Urinary Tract
a b
Fig. 17.142 Indiana pouch in a 17-year-old female born without a
bladder. (a) Longitudinal grayscale ultrasound image of the right lower
abdominal quadrant shows an irregular, fluid-filled structure. The wall
demonstrates a gut signature with inner echogenic mucosa (arrowhead)
B plantation. Clin J Am Soc Nephrol. 2014;9(2):416–29.
Fig. 17.143 Mitrofanoff appendicovesicostomy. Longitudinal gray-
scale ultrasound image of the bladder in a 15-year-old male depicts a
fluid-filled tubular structure (asterisks) extending from the bladder (B)
dome toward the anterior abdominal wall
higher rates of early postoperative complications with the Monti
procedure associated with bowel resection, including ileus and
small bowel obstruction.
Complications of bladder augmentation include intestinal,
infectious, and metabolic abnormalities. Bowel obstruction
can occur, especially with gastrocystoplasty. Pyelonephritis
and bladder rupture may develop. Mucus production, which
is greatest with colonic segments, can impair bladder empty-
ing and predispose patients to urinary tract infection. Mucus
and infection also predispose to bladder and renal stone for-
mation in this group of patients. Many of these complica-
tions are readily imaged by ultrasound.
825
and outer hypoechoic muscle (arrow). Several haustral folds (asterisks)
are identified. (b) Lateral fluoroscopic image of the pouch shows a
haustral fold (arrow)
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 Musculoskeletal System
18
Delma Y. Jarrett

Abbreviations NSAIDS Nonsteroidal anti-inflammatory drugs

OSD Osgood-Schlatter disease
AVF Arteriovenous fistula PFFD Proximal focal femoral deficiency
AVM Arteriovenous malformation PICH Partially involuting congenital hemangioma
AVN Avascular necrosis RICH Rapid involuting congenital hemangioma
BPBP Brachial plexus birth palsy SCFE Slipped capital femoral epiphysis
CKD Congenital knee dislocation SGA Subcutaneous granuloma annulare
CML Classic metaphyseal lesion SGH Scapula-glenoid-humeral head
CPD Congenital patellar dislocation SLJ Sinding-Larsen-Johansson
CT Computed tomography SUFE Slipped upper femoral epiphysis
DDH Developmental dysplasia of the hip VM Venous malformation
DESS Double-echo steady-state ZPC Zone of provisional calcification
DMARDS Disease-modifying anti-rheumatic drugs
GCTTS Giant cell tumor of the tendon sheath
GHD Glenohumeral dysplasia Introduction
GLUT1 Glucose transporter 1
I&D Incision and drainage Ultrasound can be of great utility in pediatric musculoskeletal
IH Infantile hemangioma imaging. The incomplete ossification of the pediatric skeleton
ISSVA International Society for the Study of Vascular offers advantages for ultrasound imaging when compared to
Anomalies skeletally mature patients. The radiolucent, cartilaginous por-
JIA Juvenile idiopathic arthritis tions of the bones are well visualized and do not obscure deeper
KHE Kaposiform hemangioendothelioma structures, while the cortex of ossified bone produces acoustic
KMP Kasabach-Merritt phenomenon shadowing, preventing assessment deep to the outer contour of
LM Lymphatic malformation the bone.
MCP Metacarpophalangeal In general, pediatric patients have less soft tissue between
MEDIC Multi-echo data image combination the skin surface and the joint than adults, which permits
MHE Multiple hereditary exostoses the use of higher-frequency linear transducers that provide
MO Myositis ossificans greater image resolution. Compared to magnetic resonance
MR Magnetic resonance (MR) imaging, ultrasound imaging is lower in cost, has higher
MRSA Methicillin-resistant Staphylococcus aureus spatial resolution, and can be accomplished without seda-
NICH Non-involuting congenital hemangioma tion, even in the youngest patients.
The real-time imaging capability of ultrasound is ide-
ally suited for dynamic imaging, where evaluation of joint
Electronic Supplementary Material The online version of this chap- movement and muscle contraction can help to elucidate a
ter (https://doi.org/10.1007/978-3-030-56802-3_18) contains supple-
variety of diagnoses. Ultrasound also allows for easy com-
mentary material, which is available to authorized users.
parison to the contralateral side in the case of extremity
D. Y. Jarrett (*) pathology.
Department of Radiology, New York Presbyterian Hospital, Weill In this chapter, techniques for imaging the pediatric mus-
Cornell Medical College, New York, NY, USA
culoskeltal system are reviewed, along with relevant infor-
e-mail: dej9009@med.cornell.edu

© Springer Nature Switzerland AG 2021 835

H. J. Paltiel, E. Y. Lee (eds.), Pediatric Ultrasound, https://doi.org/10.1007/978-3-030-56802-3_18
836 D. Y. Jarrett

mation about normal development and anatomy. Anatomic frequency transducers may be needed to adequately image
variants, congenital anomalies, inflammatory disorders and deep lesions.
masses are also discussed.

Imaging Approaches
Technique
For skin lesions and other superficial structures, a standoff
Patient Positioning pad can improve the quality of imaging, by increasing the
distance between the transducer and the area of concern, and
Optimizing patient positioning at the beginning of the exam- placing it within the focal zone. However, standoff pads are
ination will make the imaging process faster and easier. sometimes challenging to use, preventing direct visualization
Factors to consider include patient comfort, accessibility of of a small lesion during scanning, and may be difficult to keep
the area of interest, joint range of motion if dynamic imaging in place if a young patient is moving. In this setting, or when
is required, and the ability of the patient to perform provoca- a standoff pad is not available, a large amount of gel can be
tive maneuvers in the case of muscle hernia, such as muscle placed on the skin surface and the area of interest scanned
contraction and relaxation. with light transducer pressure, so as not to displace the gel.
For imaging of the fingers and toes, a water bath tech-
nique is very useful. The hand or foot is submerged in a basin
Ultrasound Transducer Selection of water, allowing imaging with the transducer either lightly
touching or maintaining a short distance of several mm from
Imaging should be performed with a high-frequency linear the skin surface (Fig. 18.1).
transducer, using the highest frequency that allows adequate The ability to image without direct transducer contact with
depth of penetration. Typically, imaging is performed using the skin provides many advantages for imaging. It allows for
frequencies of 12–15 MHz, although superficial structures improved dynamic imaging since visualization of the area of
are often best imaged with even higher frequencies. Lower-­ interest is maintained even when it is not touching the trans-

a b

c d

Fig. 18.1 Imaging superficial structures with standoff pad versus water
bath. (a) A standoff pad is placed over the area of interest to increase the
distance between the transducer and the skin surface. (b) Longitudinal
grayscale ultrasound image of the third finger demonstrating the
anechoic standoff pad (arrowhead) between the transducer and skin sur-
face. (c) Water bath technique with partial submersion of the fingers in
a basin. The transducer is not entirely in contact with the skin surface.
(d) Longitudinal grayscale ultrasound image of the third finger with
water (arrow) between the transducer and skin surface allowing imag-
ing of the finger in mild flexion
18 Musculoskeletal System
ducer. There is also improved visualization in areas where
bone and soft tissue contours do not allow contact of the
entire transducer footprint with the skin, such as the web
spaces of the hands and feet. The water bath technique can
prevent unintentional transducer compression of superficial
structures and may increase patient cooperation when scan-
ning inflammatory conditions, particularly when skin contact
is painful [1].
Normal Development
Musculoskeletal development begins in the fourth week of
gestation from the paraxial mesoderm, from the parietal layer
of the lateral plate mesoderm, and from cells of the neural
crest. The paraxial mesoderm differentiates into somites that
in turn give rise to sclerotomes and dermomyotomes. The
vertebrae and ribs develop from the sclerotomes, while the
musculature develops from the dermomyotomes [2].
Bone formation occurs by both intramembranous and
endochondral ossification following the condensation of
mesenchymal cells that comprise the embryonic connec-
tive tissue. Intramembranous ossification leads to forma-
tion of the cranial vault, many of the facial bones, and the
clavicle. Endochondral ossification leads to formation of
the skull base, some of the facial bones, as well as the ver-
tebrae, sternum, ribs, and the bones of the limbs and limb
girdles.
Limb development depends on induction by the apical ecto-
dermal ridge, formation of circular constrictions that separate
portions of the limbs, and opposite rotations of the upper and
Fig. 18.2 Normal appearance of the soft tissues of the anterior thigh.
Sagittal grayscale ultrasound image of the anterior thigh through the
rectus femoris muscle (arrow). The echogenic lines and foci within the
837
lower limbs. Development of the skeletal muscle involves the
differentiation of myotome cells into myoblasts.
Soft Tissues
Normal Anatomy and Imaging Approaches
Skin and Subcutaneous Tissues
The outermost layers of skin are the epidermis and dermis.
The subcutaneous tissue or hypodermis is a fatty layer, also
known as the superficial fascia.
On ultrasound, the epidermis and dermis cannot be distin-
guished and are seen as a single hypoechoic band. The hypo-
dermis is of variable thickness and echogenicity depending
on the amount of fat that is present (Fig. 18.2).
Muscles
The outer margin of a muscle is surrounded by a fascial layer,
the epimysium. Within the muscle, there are fascicles, which
are bundles of individual muscle fibers separated from each
other by deep fibrofatty fascia, the perimysium. On longitudi-
nal ultrasound images, the epimysium is an echogenic line; the
hypoechoic muscle fibers and the echogenic perimysium appear
as parallel bundles and lines which converge at the myotendinous
junction. On transverse images, the perimysium appears as echo-
genic dots or dashes between the muscle fascicles (Fig. 18.2).
These fibrous structures exhibit anisotropy on imaging if
they are not oriented perpendicular to the ultrasound beam
and can appear artifactually hypoechoic, simulating injury
[3]. Ultrasound allows for dynamic imaging of the muscles,
Epidermis/Dermis
Hypodermis (Superficial Fascia)
Deep Fascia and Epimysium
Muscle
muscle represent the perimysium, the sheath of connective tissue sur-
rounding the muscle fiber bundles
838 D. Y. Jarrett

a b

Fig. 18.3 Muscle contraction. (a) Transverse grayscale ultrasound head) during muscle contraction. The muscle is decreased in width and
image through the relaxed rectus femoris muscle (arrow). (b) Transverse increased in thickness
grayscale ultrasound image of the same rectus femoris muscle (arrow-

with visualization of morphologic and signal changes dur- a

ing cycles of contraction and relaxation. Muscles become
shorter and thicker with contraction (Fig. 18.3).

Tendons
Tendons are composed of parallel collagen fiber bundles
which appear as numerous parallel echogenic lines on
b
­longitudinal ultrasound images and echogenic dots on trans-
verse images. Tendons display anisotropy; when the ultra-
sound beam is perpendicular to the long axis of the tendon
fibers, there is maximal reflection of sound waves back to the
­transducer, and the tendon will appear echogenic. However,
the tendon will appear increasingly hypoechoic as the insonat-
ing angle decreases and fewer sound waves return to the
transducer (Fig. 18.4, Cineclip 18.1) [4].
c

Infectious/Inflammatory Disorders

Ultrasound can be useful in the evaluation of soft tissue swell-

ing, to distinguish infection from other causative entities such
as deep venous thrombosis or mass. It can identify fluid col-
lections and guide drainage procedures. Infections can be
superficial, involving the skin and hypodermis (cellulitis);
deep, with infection extending below the superficial fascia; or
can involve both the superficial and deep soft tissues.
Cellulitis
Cellulitis is an infection limited to the skin and subcutaneous
tissues. The most common etiology is minor trauma such as
an abrasion or insect bite. Streptococcus pyogenes is the most
Fig. 18.4 Normal tendon anatomy with anisotropy. Flexor tendons at
the wrist. (a) Longitudinal grayscale ultrasound image demonstrating
fibrillar structure of the tendon (asterisk). Transverse grayscale ultra-
sound images with ultrasound beam (b) perpendicular and (c) angled
relative to the tendons (arrows). In (b) the tendons appear echogenic. In
(c) there is apparent hypoechogenicity of the tendons. On both images
the median nerve (arrowheads) demonstrates decreased anisotropy
compared to the tendons and remains hypoechoic
18 Musculoskeletal System
frequently implicated organism, followed by Staphylococcus
aureus. Patients present with local erythema, swelling, and warmth
and may have systemic symptoms of infection, including fever [5].
Diagnosis is typically made clinically, although imaging is useful
if there is concern for extension into the deep tissues and a pos-
sible fluid collection [6].
On ultrasound, cellulitis can appear as swelling of the soft
tissues, with increased thickness and echogenicity. There may
be hypoechoic stranding between echogenic fat lobules with
a “cobblestone” appearance. This appearance is not specific
for cellulitis, as non-infectious soft tissue edema has the
same grayscale features, but demonstration of hyperemia
on color Doppler imaging helps to confirm the presence of
inflammation (Fig. 18.5) [7]. If there is clinical progression,
serial imaging can be performed to assess for development of
a phlegmon or abscess.
Cellulitis is empirically treated with antibiotics directed against
the most common pathogens, S. pyogenes and S. aureus, includ-
ing methicillin-­resistant S. aureus (MRSA), particularly when
infection is secondary to penetrating trauma or if there is MRSA
infection elsewhere in the body [8].
Pyomyositis
Pyomyositis is a bacterial infection of the muscle that results
from bacteremia and hematogenous spread or direct extension
from an adjacent infection. It may be precipitated by muscle
trauma and is most often seen in the larger muscles of the legs.
The most common infectious agent is S. aureus. Typically,
children with pyomyositis are otherwise healthy, unlike adults
who often have pre-existing conditions making them more
susceptible to infection. Patients present with fever and mus-
cle pain. Historically, pyomyositis was generally confined to
tropical locales. However, it is being diagnosed with increas-
ing frequency in temperate zones as well [7, 9].
In stage 1 of pyomyositis, the invasive stage, the muscle
is edematous and demonstrates increased echogenicity on
a b
Fig. 18.5 Cellulitis and skin abscess in a 14-month-old female. (a)
Transverse grayscale ultrasound image of the medial thigh shows an
irregular hypoechoic collection (arrow) in the hypodermis. The surround-
839
ultrasound. The ultrasound appearance of muscle edema is not
specific for pyomyositis. Stage 2, the suppurative stage, occurs
at 10–21 days, with intramuscular abscess formation which
is indicative of bacterial infection in pyomyositis (Fig. 18.6).
The ultrasound appearance of a soft tissue abscess is described
below. In stage 3, the late stage of septicemia, there is multior-
gan failure with a high associated mortality [6, 10].
Treatment for pyomyositis includes antibiotics and/or
abscess drainage, with imaging used to document abscess
resolution [9].
Soft Tissue Abscess
An abscess is a walled off collection of necrotic tissue, bac-
teria, and inflammatory cells. Patients may present with an
erythematous, painful, fluctuant lump in the skin. However,
the edema and induration of the tissues can make it difficult
on physical examination to distinguish an abscess from cel-
lulitis [11].
On ultrasound, an abscess will appear as a well-defined
collection of variable echogenicity, ranging from hypo-,
iso-, to hyperechoic, depending on the nature of its contents.
There may also be posterior acoustic enhancement. On color
Doppler imaging, the surrounding soft tissues, abscess wall,
and any internal septations will be hyperemic. There should
be no internal vascularity (Fig. 18.7, Cineclip 18.2).
Using the ultrasound transducer to apply pressure to the
collection can help to demonstrate motion of the internal
contents and to confirm the fluid nature of the collection. An
abscess may contain gas if it communicates with the skin
or if it contains a gas-forming organism. Ultrasound will
show foci of increased echogenicity with posterior “dirty”
shadowing. Ultrasound can also be used to guide aspiration
or catheter placement for a drainable abscess [7, 10].
Soft tissue abscesses are treated with incision and drain-
age (I&D). Additional antibiotic coverage may be necessary
for a variety of reasons, including possible MRSA infection,
ing tissues are increased in thickness and echogenicity with a cobblestone
appearance. (b) Transverse color Doppler ultrasound image demonstrates
hyperemia of the tissues with no flow seen in the collection (arrow)
840 D. Y. Jarrett

a b

Fig. 18.6 Pyomyositis with intramuscular abscess in a 17-year-old collection (arrow) in the popliteus muscle. (b) Longitudinal color Doppler
female. (a) Longitudinal grayscale ultrasound image of the posterior leg ultrasound image shows peripheral hyperemia with no flow (arrowhead)
at the level of the proximal tibia demonstrates an ovoid, hypoechoic fluid in the anechoic fluid component of the abscess

a b

Fig. 18.7 Subcutaneous abscess in an 11-year-old male. Longitudinal through transmission. There is peripheral hyperemia with no internal
grayscale (a) and color Doppler (b) ultrasound images of the axilla vascularity. An axillary lymph node (arrowheads) is noted deep to the
demonstrate an irregularly shaped collection (arrows) in the subcutane- collection
ous tissues with heterogeneous, echogenic contents and increased

multiple abscesses, systemic infection, immunocompromise,
and lack of response to I&D [8].
Trauma
Fat Necrosis
Fat necrosis presents as a palpable lump and usually occurs
as a sequela of traumatic compression of the soft tissues,
with shear injury of the subcutaneous fat. It can also result
from other processes that impair blood supply or cause
saponification of fat, including vasculitis and subcutane-
ous injections. Since there is often a long delay between
injury and presentation, the child may not recall the ante-
cedent trauma. The focus of fat necrosis will spontaneously
resolve in weeks to months, although there may be residual
fatty atrophy with development of a focal depression in the
soft tissues [12, 13].
18 Musculoskeletal System
a b
Fig. 18.8 Fat necrosis in two different patients. (a) 18-year-old female
with fat necrosis of the thigh. Longitudinal grayscale ultrasound image
shows a poorly defined area of increased echogenicity (white arrow) in
the fat of the hypodermis, with a central hypoechoic focus. (b) 15-day-old
Fat necrosis has a highly variable appearance on ultra-
sound, depending on its stage of evolution. It can appear
as a well-defined mass, with the central zone isoechoic to
fat, and a peripheral, hypoechoic fibrous capsule. It can
also manifest as a poorly defined region of increased echo-
genicity within fat, reflecting inflammation and necrosis, or
as a centrally or diffusely hypoechoic lesion (Fig. 18.8a).
A separate form of subcutaneous fat necrosis occurs in
the newborn, which is a localized or diffuse fat necrosis that
develops following forceps delivery or as a result of systemic
factors that include hypoxia or infection. This form of fat
necrosis can be complicated by hypercalcemia, nephrocal-
cinosis, or renal insufficiency. Fat necrosis of the newborn
is homogeneously echogenic to subcutaneous fat, without
calcification (Fig. 18.8b).
Both forms of fat necrosis are self-limited processes which
are treated conservatively [12].
Foreign Bodies
Retained foreign bodies can lead to infection, including
abscess formation and osteomyelitis. Unlike glass, metal,
and stone which are radiopaque, wood is radiolucent, and
wooden splinters in the soft tissues may not be visualized on
plain radiographs. When properly performed, ultrasound is
reliable for the detection of wooden foreign bodies and can
identify wood fragments as small as 2.5 mm in length [14].
Wooden foreign bodies are often long, narrow splinters.
The location of the skin puncture site provides valuable
information about the likely location and trajectory of the
foreign body. The ultrasound transducer should be oriented
along the expected long axis of the foreign body to maximize
its conspicuity and associated posterior shadowing.
The foreign body may be located several cm from the skin
entry site if a portion was removed during a prior extraction
841
male with fat necrosis of the lower thoracic spine after therapeutic hypo-
thermia. Longitudinal grayscale ultrasound image reveals increased
echogenicity of the subcutaneous fat (black arrow)
attempt. Beginning 24 hours after injury, granulation tissue,
edema, and eventually an abscess develop around the for-
eign body, forming a hypoechoic rim of tissue that aids in
its detection by ultrasound (Fig. 18.9) [14]. The presence of
soft tissue gas, either from laceration at the time of injury
or from attempts at foreign body removal, may obscure its
visualization. If urgent extraction is not required, ultrasound
examination can be re-attempted after waiting several days
for the soft tissue gas to resolve.
A foreign body is treated by percutaneous removal, either
at the bedside or in the operating room, depending on its
depth and location. Ultrasound can be used to aid in localiza-
tion during the procedure.
 uscle Tears and Intramuscular Hematomas
M
Muscle tears and intramuscular hematomas are rare in young
children. In athletes, they occur most commonly from indirect
injury with forceful, eccentric muscle contraction causing a
tear, typically at the myotendinous junction. These injuries pres-
ent with immediate pain in the affected muscle compartment.
Muscles can also be injured by direct blunt trauma, as from
sports-related collisions, with contusion of the deep muscle belly.
These injuries tend to be less symptomatic than muscle tears [3].
On ultrasound, muscle contusion without tearing will
appear as a region of increased echogenicity which can cross
fascial boundaries [15]. Muscle tears are graded using a
three-point scale:
Grade 1: Tearing of less than 5% of the muscle. The muscle
may be normal in appearance or have an ill-defined, hypoechoic
zone with minimal if any disruption of muscle fibers.
Grade 2: Incomplete muscle tear. Disruption of fibers is
seen with a gap between the margins of the torn muscle con-
taining intramuscular hematoma (Fig. 18.10). The hematoma
is variable in appearance and evolves over time. Acutely,
842
a b
Fig. 18.9 Wood splinter in the foot of a 7-year-old male. (a) Transverse
grayscale ultrasound image of the plantar aspect of the foot shows an echo-
genic wood splinter (white arrow) with posterior shadowing (arrowhead).
There is a hypoechoic rim (black arrow) of edema or granulation tissue
a b
Fig. 18.10 Grade 2 muscle tear of the rectus femoris muscle in a
10-year-old female. (a) Transverse grayscale and (b) longitudinal color
Doppler ultrasound images reveal a heterogeneously echogenic fluid
it can appear echogenic and ill-defined. It will eventually
evolve into a well-defined, hypoechoic collection with echo-
genic margins that extend from the periphery to the center of
the lesion [3, 16].
Color Doppler imaging may show hypervascularity sur-
rounding the disrupted muscle fibers. Imaging with contrac-
tion of the muscle can be useful to increase the conspicuity
of the tear. Given the wide range in severity of grade 2 mus-
cle tears, it may be useful to give an estimate of the extent of
cross-sectional muscular involvement.
Grade 3: Complete muscle tear. There is a gap between
the retracted proximal and distal margins with an intervening
hematoma.
D. Y. Jarrett
surrounding the splinter, increasing its conspicuity. (b) Longitudinal gray-
scale ultrasound image of the plantar aspect of the foot shows the 3.9-cm-
long splinter (arrow) in its entirety
collection (arrows) in the muscle consistent with a hematoma located at
a distance from the central myotendinous junction (arrowhead)
Ultrasound can be used to document the healing of
muscle tears, ideally with restoration of normal architec-
ture. However, with severe tears, there may be architec-
tural distortion and fibrous scar formation, identified as an
echogenic linear or stellate focus. MR imaging should be
performed if there is a discrepancy between the patient’s
clinical course and the ultrasound findings, if there is a
hematoma without a history of trauma, or if there is no
healing of the injury by ultrasound at 5–7 weeks, as these
features raise concern for an additional underlying disor-
der [17].
Grade 3 tears may require surgical repair, while lower-­
grade tears are treated conservatively [15].
18 Musculoskeletal System
Myositis Ossificans
Myositis ossificans (MO) is a form of soft tissue heterotopic
ossification. As the name suggests, it occurs in muscle, but
can also involve the subcutaneous fat, where it is some-
times referred to as panniculitis ossificans. Most cases of
MO are post-traumatic in etiology, although a traumatic
episode may not be recalled by the patient. MO is rela-
tively uncommon in children less than 10 years of age, has
a slight male predominance, and often occurs in the anterior
aspects of the arms and thighs. The lesion may be painful
and erythematous and accompanied by systemic signs of
inflammation, including fever and an elevated erythrocyte
sedimentation rate [18].
The histologic findings of MO can mimic those of a soft
tissue sarcoma [19]. It is therefore important to clinically dif-
ferentiate MO from tumor, to avoid undue anxiety for the
patient and unnecessary biopsy, particularly in its early pro-
liferative stage.
The diagnosis of MO is often not suspected, and a child
may present to ultrasound for evaluation of a mass. In its
early stages, the diagnosis of MO can be difficult. On ultra-
sound, early MO lesions have a non-specific appearance.
They are heterogeneously hypoechoic masses with a cen-
tral hyperechoic zone. They may display signs of inflam-
mation on color Doppler imaging, including increased
vascularity in and around the lesion and increased echo-
genicity of the adjacent soft tissues. Differential diagnos-
tic considerations for this appearance include tumor and
infection.
The imaging diagnosis becomes clear in the mature
ossification stage, weeks to months after onset, when MO
develops characteristic peripheral calcifications, which
can be detected on ultrasound before they are radiographi-
cally apparent (Fig. 18.11). Over time, the calcifications
mature and surround the periphery of the lesion, causing
a b
Fig. 18.11 Myositis ossificans in a 15-year-old male. (a) Longitudinal
grayscale ultrasound image shows a mass (arrowheads) in the left rec-
tus femoris muscle with echogenic peripheral calcifications. (b) Lateral
843
posterior acoustic shadowing. The lesion can be followed
on radiographs, with progressive peripheral calcification
followed by central calcification and eventual decrease in
size.
Treatment for post-traumatic MO is conservative, with
lesions regressing spontaneously. Surgical resection is reserved
for masses causing neurovascular impingement, mechanical
issues, or cosmetic deformity [18, 19].
Muscle Hernia
A muscle hernia is a protrusion of muscle through a fascial
defect or a bulge in the muscle from a focal thinning of the
overlying fascia. In adults, it is associated with overuse and
trauma (e.g., penetrating wound, fracture, or direct muscle
injury) causing fascial injury. Muscle hernias can also be
seen in young children, suggesting constitutional weakness
of the fascia as an etiology. It can occur at sites of perforating
nerves and vessels, which may be a contributing factor to its
development [20, 21].
A muscle hernia is most commonly seen in the tibialis
anterior muscle. Patients present with a lump that increases
in conspicuity with contraction of the muscle. It is typi-
cally asymptomatic, with imaging performed to exclude a
soft tissue mass or vascular malformation. Ultrasound is
the ideal modality for imaging a muscle hernia, because
it allows dynamic assessment and easy comparison with
the contralateral side. False negative results can be seen
with MR imaging and computed tomography (CT), as it
is difficult to maintain adequate muscle contraction during
imaging.
Ultrasound evaluation should begin with determining the
patient position or activity that most often elicits the lump.
Depending on the muscle, this may include having the patient
stand and plantar or dorsiflex the ankle. Ultrasound will demon-
strate a defect or focal bulge in the echogenic line of the muscle
radiograph of the left femur obtained 4 months later demonstrates
peripheral calcification (arrow) of the lesion which was not radiograph-
ically apparent at the time of the ultrasound study
844
fascia, with protrusion of the muscle (Fig. 18.12, Cineclip 18.3).
Cine clips obtained as the patient holds and releases the pro-
vocative position will show the dynamic nature of the hernia.
It is important to maintain light pressure with the ultrasound
transducer, so as to not inadvertently reduce the hernia.
As most patients are asymptomatic, once the diagnosis of
a muscle hernia is confirmed, management is conservative.
For those who have associated pain, treatment may include
fasciotomy or fascial patch graft. Primary repair of the fas-
cial defect is not performed because this may lead to the
development of compartment syndrome [20, 21].
a Tendon tears can be degenerative, on the continuum of
b treatment includes rest, physical therapy, and rehabilita-
Fig. 18.12 Hernia of the tibialis anterior muscle in a 3-month-old male.
(a) Longitudinal grayscale ultrasound image shows focal disruption of the
superficial muscular fascia and herniation of hypoechoic muscle (arrow)
through the defect. (b) Longitudinal color Doppler ultrasound image dem-
onstrates a crossing vessel (arrowhead) at the site of the muscle hernia, a
potential contributor to fascial weakness in this location
a b
Fig. 18.13 Patellar tendinopathy and Osgood-Schlatter disease in an
11-year-old male. (a) Longitudinal grayscale ultrasound image of the
normal left patellar tendon (black arrow). (b) Longitudinal grayscale
ultrasound image of the right patellar tendon (white arrow) shows thick-
ening and decreased echogenicity consistent with tendinopathy. There
D. Y. Jarrett
Tendinopathy and Tendon Tears
Tendinopathy results from degeneration of the tendon, with
attempted repair. The tendon will appear thickened and decreased
in echogenicity on ultrasound, with loss of the typical organized
fibrillar structure (Fig. 18.13). As previously discussed, aniso-
tropic effects can simulate decreased tendon echogenicity if
the ultrasound transducer is not oriented 90° to the tendon.
Symptomatic tendinopathy may demonstrate increased vas-
cularity on color Doppler imaging, reflecting angiogenesis,
not inflammation, as tendinopathy is not an inflammatory
process. In the case of chronic tendinopathy, calcification
may be present, with echogenic, shadowing foci seen on
ultrasound [22].
tendinopathy, or may be acute and traumatic. With partial
tendon tears, hypoechoic defects of the tendon fibers are
seen. In full-thickness tears, there is complete discontinu-
ity with fluid separating the distracted ends (Fig. 18.14).
Tendon tears may be treated conservatively or by surgical
repair. There is a wide range of treatment options for tendi-
nopathy. Factors affecting management include the sever-
ity of injury, patient age and activity level. Conservative
tion. Nonsteroidal anti-inflammatory drugs (NSAIDs) and
corticosteroids provide pain relief. Dry needling of the ten-
don and injection of platelet-rich plasma or stem cells have
been investigated; however, further studies are required to
determine their effectiveness. Surgical debridement may be
performed if other methods fail [23].
Tumors
Ultrasound is often the first imaging study performed for
palpable soft tissue masses in young children. It can dis-
tinguish solid from cystic lesions and characterize vascular
c
is an ossific fragment (arrowhead) anterior to the tibial tubercle (T),
with intervening hypoechoic tissue. (c) Lateral right knee radiograph
shows fragmentation of the tibial tubercle (arrowhead) with overlying
soft tissue thickening
18 Musculoskeletal System 845

anomalies, which are discussed later in the chapter. If the
ultrasound findings are characteristic of a specific entity,
the cost and potential sedation necessary for MR imaging
can be avoided. However, the ultrasound appearance of soft
tissue masses is frequently non-specific, necessitating MR
imaging for further evaluation. This section will describe
common soft tissue masses in children that are often diag-
nosed by ultrasound.
is located in the subcutaneous tissues, with no extension into
the underlying muscle or bone (Fig. 18.15). On color Doppler
imaging, vascularity is typically absent. When flow is present,
it is limited to the periphery of the lesion. Differential diag-
nostic considerations for SGA include hematoma, fat necrosis,
and nodular fasciitis.
SGA is treated conservatively, with spontaneous regres-
sion of the lesion over months to years [24].

 ubcutaneous Granuloma Annulare

S Pilomatricoma
Subcutaneous granuloma annulare (SGA) is a benign inflam-
matory mass of uncertain etiology. It is seen almost exclu-
sively in children, most often between 2 and 5 years of age,
with a slight predilection for girls. SGA frequently occurs
in the pretibial region, although it is also seen in the scalp,
especially over the occiput. Patients present with a rapidly
growing, painless, non-mobile lump in the subcutaneous tis-
sues [13, 24].
On ultrasound, SGA is a solid, ill-defined, hypoechoic mass,
sometimes with a peripheral rim of increased echogenicity. It
Pilomatricoma (also known as pilomatrixoma) is a benign
tumor of the skin and subcutaneous tissues, arising from
the cortex of a hair follicle. It is most often seen in children
and adolescents, and is the second most commonly resected
skin mass in children after epidermoid cyst. Pilomatricoma
can arise in any hair-bearing skin, although it is most fre-
quently found in the head and neck [25]. Patients present
with a small, painless, slow-growing, mobile mass, usually
less than 2 cm in size. There may be a bluish discoloration of
the overlying skin [13].

a b

Fig. 18.14 Achilles tendon tear in a 19-year-old female. (a) Longitudinal Longitudinal grayscale ultrasound image of the normal left Achilles ten-
grayscale ultrasound image of the right Achilles tendon (arrow) shows don (arrowhead) shown for comparison
thickening, decreased echogenicity, and disruption of the fibers. (b)

a b

Fig. 18.15 Subcutaneous granuloma annulare in a 6-year-old female. demonstrate a heterogeneously hypoechoic mass (white arrows) with
(a) Transverse and (b) longitudinal grayscale ultrasound images of the ill-defined margins. There is no breach of the underlying tibial cortex
subcutaneous tissues along the anterior aspect of the proximal tibia (arrowhead) or muscular fascia (black arrow)
846 D. Y. Jarrett

a b

Fig. 18.16 Pilomatricoma of the neck in a 6-year-old female. (a) Trans­ with calcification. (b) Longitudinal color Doppler ultrasound image
verse grayscale ultrasound image shows a heterogeneous mass (arrow) reveals peripheral vascularity (arrow)
with a hypoechoic rim and punctate internal echogenic foci compatible

On ultrasound, a pilomatricoma is a well-defined, hyper- a

echoic or isoechoic nodule with a characteristic hypoechoic
rim and variable calcification. Vascularity may be present
on color Doppler imaging, usually peripheral in location.
There can also be increased echogenicity of the soft tissues
surrounding the lesion if there is associated inflammation
(Fig. 18.16).
Pilomatricoma is treated with complete surgical excision
and has an excellent prognosis, with malignant transforma- b
tion exceedingly rare [26].

Lipoma
Lipomas are tumors composed of mature fat and are the
most common adipocytic tumor in children and adolescents,
accounting for approximately two-thirds of fatty tumors
in the first two decades of life. Lipomas are slow-growing
lesions that can arise anywhere in the body where there is fat,
although they are most common in the trunk and proximal
extremities. They are usually located in the subcutaneous tis-
sues, although they can also occur deep to the muscle fascia
or within muscle. Lipomatosis denotes an infiltrative lipoma
Fig. 18.17 Lipoma in two different patients, illustrating variability in
that involves multiple tissues [12].
echogenicity. (a) 15-year-old male with a popliteal lipoma. Longitudinal
As with fat, lipomas have a variable appearance on ultra- grayscale ultrasound image shows an ovoid mass (arrow) containing
sound, ranging from hypo- to hyperechoic, depending on their multiple internal hyperechoic linear bands. The mass appears mildly
location and the adjacent structures. They may contain linear more echogenic than the adjacent subcutaneous fat (arrowheads). (b)
12-year-old male with left groin lipoma. Transverse grayscale ultrasound
echogenic bands and lack internal vascularity, shadowing
image demonstrates an ovoid mass (arrow) of similar echogenicity to the
or posterior acoustic enhancement (Fig. 18.17). Superficial adjacent subcutaneous fat (asterisks)
lipomas have a soft consistency and are compressible by the
ultrasound transducer. The diagnosis on ultrasound is often
aided by high clinical suspicion. If there is any doubt as to
their nature, MR imaging can be used to confirm the diagno- Lipoblastoma
sis, as the appearance of fat is highly specific. Lipoblastoma is a benign tumor that accounts for 30% of adi-
Lipomas are benign and treatment is usually not neces- pocytic tumors in children. Unlike lipoma, lipoblastoma most
sary. Surgical resection can be performed for cosmetic rea- commonly occurs in children less than 3 years of age. It is a
sons and is curative. well-circumscribed mass that is typically located in the sub-
18 Musculoskeletal System
a b
c d
Fig. 18.18 Lipoblastoma in two different patients. (a) 9-month-old
male with lipoblastoma of the left deltoid muscle. Transverse grayscale
ultrasound image shows a lobulated, echogenic mass (arrows) splaying
apart the hypoechoic muscle fibers (arrowheads). (b) Axial T1-weighted
magnetic resonance (MR) image depicts the intramuscular hyperin-
cutaneous tissues and contains both mature and immature adi-
pocytes. Lipoblastoma is a more complex lesion than lipoma,
containing fibrous septa and often a plexiform myxoid matrix.
When lipoblastoma is diffuse and infiltrative, sometimes with
muscular extension, it is referred to as lipoblastomatosis [12].
On ultrasound, lipoblastoma has a variable appearance. It is
often homogenous and echogenic compared to muscle, but can
also be heterogeneous with internal cystic spaces (Fig. 18.18).
As the ultrasound appearance is non-specific, these lesions
often go on to MR imaging. Biopsy may be required to dis-
tinguish lipoblastoma from liposarcoma, although the latter is
very rare in children younger than 10 years of age.
While benign, lipoblastoma can cause complications
related to mass effect and compression of adjacent struc-
tures. It is treated by complete resection. Local recurrence is
common, in up to 47% of cases, and usually with infiltrative
lipoblastomatosis [27].
Bones/Cartilage
Normal Anatomy and Imaging Approaches
A long bone has a tubular shaft, the diaphysis, which extends
most of its length. Each end of the bone terminates in an
847
tense fatty mass (arrows). (c) 6-year-old male with lipoblastoma of the
right knee. Longitudinal grayscale ultrasound image demonstrates a
subcutaneous ovoid mass (arrow) containing linear echogenic bands.
(d) Coronal T1-weighted MR image of the right knee depicts the lateral
subcutaneous fatty mass (arrow)
epiphysis, and a metaphysis is located between the epiphysis
and diaphysis. In children, the epiphysis may be cartilaginous,
with variable presence and size of one or more ossification
centers, depending on the age of the child. There are vascular
canals within the epiphyseal cartilage, containing blood ves-
sels, nerves, and connective tissue which are ­essential for car-
tilage growth.
On ultrasound, the cartilaginous epiphysis appears hypo­
echoic, and the vascular canals are seen as echogenic lines or
dots, depending on whether they are imaged in long or short
axis [28]. If an ossification center is present within the epiphy-
sis, it will appear as an echogenic shadowing structure embed-
ded in the epiphyseal cartilage (Fig. 18.19). At the joint surface,
the overlying articular cartilage will appear hypoechoic, similar
to the epiphyseal cartilage. Although the articular cartilage does
not contain vascular canals, its boundary with the epiphyseal
cartilage cannot be discerned with ultrasound (Fig. 18.20).
Until skeletal maturity has been reached, the epiphysis is
separated from the metaphysis by a cartilaginous physis or
growth plate. An understanding of the anatomy of this region
is critical for an appreciation of the normal imaging appear-
ance as well as the appearance of pathological conditions
that occur at the physeal-metaphyseal junction.
The physis is composed of multiple zones that include (1)
the resting zone that stores nutrients and supplies stem cells
848
a b
Fig. 18.19 Normal proximal humerus in a 4-month-old female. (a)
Frontal radiograph shows a small right humeral head ossification center
(arrowhead). (b) Longitudinal grayscale ultrasound image shows the
hypoechoic cartilage of the unossified humeral head (white arrow) with
echogenic dots corresponding to vascular canals. The cortex of the
C R
Fig. 18.20 Normal elbow in a 34-month-old male. Longitudinal gray-
scale ultrasound image of the anterior radiocapitellar joint. The hypo­
echoic cartilaginous physes (arrows) cannot be differentiated from the
unossified epiphyseal cartilage and articular cartilage (arrowheads) of
the capitellum (C) and radial head (R)
for developing chondrocytes; (2) the proliferation zone that
is the center for longitudinal growth, with rapid chondrocyte
cell division; (3) the hypertrophic zone where chondrocytes
mature, enlarge, and undergo apoptosis; and (4) the zone of
provisional calcification (ZPC) that is the transition zone
between cartilage and bone. Calcification occurs in the inter-
cellular matrix between apoptotic chondrocytes, serving as a
scaffold for new bone formation [29].
The metaphyseal layers include (1) The primary spon-
giosa, abutting the ZPC. It is the site of most recent bone
formation and is comprised of trabeculae formed from lon-
gitudinally oriented struts of cartilage that are covered by a
thin layer of bone; and (2) the secondary spongiosa where
the number of trabeculae decreases, with an increase in size
of the marrow space [30].
D. Y. Jarrett
c
metaphysis and diaphysis is echogenic (black arrow) with posterior
shadowing. (c) Transverse grayscale ultrasound image of the humeral
head reveals a small echogenic focus of ossification (arrowhead) with
posterior shadowing
Fig. 18.21 Normal physeal and periphyseal anatomy. Coronal gray-
scale ultrasound image of the distal left femur in an 83-day-old male
depicts the metaphyseal bone collar (arrow) and the ultrasound zone of
provisional calcification (arrowhead). The distal femoral epiphysis is
mostly cartilaginous, with a small ossification center (asterisk)
On ultrasound there is an echogenic line at the border
of the metaphysis and the physis which represents the pri-
mary spongiosa, also referred to as the sonographic ZPC
(Fig. 18.21) [31, 32].
Surrounding the margin of physis and overlapping with
the adjacent metaphysis is a fibro-chondro-osseous compo-
nent of the perichondrium, the metaphyseal bone collar (also
referred to as the ring of LaCroix, subperiosteal bone collar,
and bone bark). On ultrasound, the metaphyseal bone collar
appears as a longitudinally oriented, echogenic linear focus
extending from the physis to the periphyseal metaphysis
(primary spongiosa) [30–34]. It is most easily identified at
the physis of the distal femur (Fig. 18.21).
Apophyses are centers of growth at ligamentous and
tendinous attachment sites that are cartilaginous at birth. In
children they are separated from the adjacent metaphysis or
metaphyseal equivalent by the cartilaginous physis. Similar
to an epiphysis, an apophysis will undergo ossification and
eventual physeal fusion.
18 Musculoskeletal System 849

Congenital/Developmental Abnormalities
 ongenital Rib Anomalies
C palpable area of concern with the imaging findings and com-
There is a wide variety of congenital rib anomalies, includ-
ing cervical ribs, rib fusion defects and duplications. Pseud­
arthroses may be congenital or result from chronic fracture
non-union [35, 36]. Congenital rib anomalies are most
often isolated, incidental findings, although bifid ribs can be
associated with genetic disorders, such as Gorlin-­Goltz syn-
drome [37]. In children presenting with an anterior chest wall
mass, the most common cause is a rib deformity [38]. Most
congenital rib anomalies are asymptomatic, and patients are
referred to ultrasound for evaluation of a palpable mass.
Imaging may be indicated to exclude a possible tumor, and
in addition to rib deformities, ­ultrasound can detect abnor-
malities involving the costal cartilage not readily apparent on
radiography [38]. Ultrasound also allows correlation of the
parison with the contralateral side to assess for symmetry. The
course of the rib should be followed in the longitudinal and
transverse planes to assess the outer osseous contour and the
costal cartilage for pseudarthrosis, fusion anomaly, angular
deformity, or intrinsic bone lesion (Figs. 18.22 and 18.23).
Most rib anomalies do not require further treatment once
the diagnosis is confirmed. Cervical ribs may be symptom-
atic, causing thoracic outlet syndrome, with compression of
the neurovascular bundle as it passes from the upper chest to
the axilla. This can be treated surgically, with resection of the
rib and possibly the anterior scalene muscle if it contributes
to the compression [39].

a b

Fig. 18.22 Osteochondroma of the rib in a 7-year-old female with left shoulder confirms the presence of an osteochondroma of the rib
multiple hereditary exostoses. (a) Longitudinal grayscale ultrasound (arrow) and additional osteochondromas (arrowheads) of the proximal
image demonstrates a bony excrescence with irregular margins and an left humerus
overlying hypoechoic cartilaginous cap (arrow). (b) Radiograph of the

a b

Fig. 18.23 First rib pseudarthrosis in a 12-year-old female. (a) Longi­ (arrowheads). (b) Frontal radiograph confirms presence of a right first
tudinal grayscale ultrasound image of the right first rib demonstrates rib pseudarthrosis (black arrow)
hypoechoic cartilage (arrow) between the irregular osseous margins
850 D. Y. Jarrett

Infectious/Inflammatory Disorders spread of osteomyelitis has recently been shown to be com-

Osteomyelitis
Acute hematogenous osteomyelitis is more common in
growing children than adults, with half of pediatric cases
developing in children less than 5 years of age. It has an
incidence of 8–10/100,000 in developed countries and 80
per 100,000 in developing countries. Males are affected
twice as often as females, ascribed to the role of trauma
in the development of osteomyelitis. The most commonly
implicated organisms are S. aureus and Kingella kingae [40].
Metaphyses (and metaphyseal equivalents) are the most
frequent primary site of hematogenous infections, as they
are highly vascularized, with leaky endothelium and termi-
nal vessels ending or looping at the physis, allowing bacteria
to lodge at the junction of the physis and metaphysis. For
the first 18 months of life, there are transphyseal vessels that
connect the blood supplies of the epiphysis and metaphysis.
In this age group, epiphyseal osteomyelitis is seen, related to
direct spread of infection from the metaphysis [40–42].
After 18 months of age, the transphyseal vessels involute,
separating the blood supplies of the epiphysis and metaphy-
sis (Fig. 18.24). It was long believed that the avascular phy-
sis acted as a barrier to epiphyseal spread of osteomyelitis
until it closed at skeletal maturity. However, the transphyseal
mon, even in the 2–16-year-old age group [41].
If the cortex is intact, ultrasound cannot detect the intramed-
ullary changes of osteomyelitis in the metaphysis and diaphysis.
However, it can demonstrate abnormalities in the cartilaginous
epiphysis, including an abnormal increase in echogenicity of
the cartilage and focal areas of decreased internal vascularity on
color Doppler imaging. These changes mirror the MR imaging
findings of focally decreased epiphyseal cartilage enhancement
in infectious chondritis, in contrast to the global decrease in per-
fusion which occurs with septic arthritis (Fig. 18.25) [42].
If infection reaches the subperiosteal space from the
metaphysis or by direct seeding, a subperiosteal abscess can
form. In the growing skeleton, the fibrous outer layer of the
periosteum can be easily separated from the cortex of the
underlying diaphysis and metaphysis. However, it is firmly
attached to the cortex at the level of the physis, at the junc-
tion with the perichondrium.
Ultrasound is useful in assessing for subperiosteal abscess,
including size measurement and guiding possible drainage [43,
44]. An abscess appears as a fluid collection which may be
hypoechoic or contain internal echoes, depending on its con-
tents. It is bounded anteriorly and posteriorly by an echogenic
line of cortex and an echogenic line of periosteum. At the level of
the physis, the collection assumes a characteristic “V”-shaped

a b

Epiphysis

Physis

Metaphysis

Fig. 18.24 Diagram illustrating the normal temporal changes to the the physis. (b) After 18 months of age, the transphyseal vessels invo-
epiphyseal and metaphyseal blood supply. (a) In the first 18 months of lute, thereby separating the blood supplies of the metaphysis and
life, blood vessels extend from the metaphysis to the epiphysis through epiphysis
18 Musculoskeletal System
a b
Fig. 18.25 Infectious chondritis and septic arthritis of the shoulder in
a 36-day-old male. (a) Transverse grayscale ultrasound image of the
right humeral head demonstrates a region of abnormally increased echo-
genicity (arrowheads) within the epiphyseal cartilage. A large amount
Fig. 18.26 Subperiosteal abscess in a 68-day-old male. Longitudinal
grayscale ultrasound image of the proximal tibia demonstrates a
hypoechoic subperiosteal fluid collection (arrow), terminating at the
level of the physis in a V-shaped configuration (arrowhead)
configuration where the periosteum and cortex become tightly
attached at the perichondrial junction. It cannot extend beyond
the level of the physis, which distinguishes a subperiosteal col-
lection from a soft tissue collection (Fig. 18.26) [40].
851
of complex fluid (arrow) is present within the joint space. (b) Transverse
grayscale ultrasound image of the normal left humeral head shown for
comparison
Ultrasound can evaluate for the presence of a joint effu-
sion, which may be reactive in the presence of osteomyeli-
tis or secondary to septic arthritis, and can be used to guide
aspiration.
Osteomyelitis is treated with antibiotics, with empiric
therapy targeting the most common organisms, particularly
MRSA. There is some controversy as to whether gram-­negative
therapy for K. kingae should be initiated immediately or only
if there is no rapid response to gram-­positive targeted therapy.
Surgery may be indicated for drainage of the source of infec-
tion, with removal of necrotic tissue and purulent material from
the bone, and drainage of subperiosteal abscess. Surgery also
can be used to confirm the pathogen prior to treatment [45].
Trauma
This section will focus on the use of ultrasound for two
pediatric-­specific injuries, epiphyseal separation and classic
metaphyseal lesions.
Epiphyseal Separation
Epiphyseal separation occurs in the long bones of young
children, when a fracture through the relatively weak physeal
cartilage separates the epiphysis from the metaphysis. It has
been described in the neonatal humerus and femur after birth
trauma, although it can also occur after accidental trauma or
abuse [46, 47].
852
a b
C C
R R
Fig. 18.27 Epiphyseal separation/Salter 2 fracture in a 13-day-old female.
(a) Longitudinal grayscale ultrasound image of the posterior right
elbow demonstrates a Salter 2 fracture of the distal humerus with an
echogenic metaphyseal fragment (arrowhead) attached to the cartilagi-
Epiphyseal separation is sometimes misdiagnosed by
radiography as a joint dislocation if the epiphysis is not
ossified, and the presumptive alignment of the joint is indi-
rectly inferred from metaphyseal position. However, dislo-
cation is very rare in children less than 3 years of age, and
there should be a high suspicion for epiphyseal separation
in this age group. Ultrasound allows direct visualization of
the epiphyseal cartilage, joint alignment, and epiphyseal-
metaphyseal relationship.
Epiphyseal separation can occur with both Salter 1 frac-
tures, entirely through the physis, and Salter 2 fractures, which
involve the physis and metaphysis. In the case of a Salter 1
epiphyseal separation, on ultrasound there will be disruption of
the physis, with an increased distance between the hypoechoic
cartilaginous epiphysis and the echogenic line of the metaphy-
seal primary spongiosa. Intervening intermediate or echogenic
tissue reflects blood products from the fracture. With a Salter
2 epiphyseal separation, a portion of metaphysis is attached
to the epiphyseal fracture fragment, and the echogenic line of
the primary spongiosa will be seen along the margin of the
displaced cartilaginous epiphysis (Fig. 18.27).
Epiphyseal separation can be treated by closed or open
reduction and K-wire fixation [48]. More detailed informa-
D. Y. Jarrett
nous capitellum (C). Capitellar alignment with the radial head (R) is
maintained. (b) Longitudinal grayscale ultrasound image of the normal
posterior left elbow for comparison
tion about distal humeral epiphyseal separation will be pro-
vided in the elbow section.
 lassic Metaphyseal Lesion
C
The classic metaphyseal lesion (CML) is a type of metaphy-
seal fracture which has been shown to be highly specific for
inflicted trauma, most commonly seen in the distal femur
and proximal and distal tibia [49, 50]. The fracture plane of
the CML courses through the subphyseal metaphysis, at the
transition zone between the primary and secondary spon-
giosa. Rather than a completely transverse fracture plane
at the periphery of the metaphysis, the fracture line courses
away from the physis, creating a fracture fragment which is
thicker at the periphery and contains a fragment of metaphy-
sis, including the metaphyseal bone collar [30].
Radiographs are the primary method of evaluating for the
presence of CML (Fig. 18.28). While ultrasound has lower
sensitivity, it can be useful to evaluate the extent of frac-
ture displacement. Ultrasound findings in CML have been
described, including thickening, deformity, or displacement
of the metaphyseal bone collar (Fig. 18.29) [31, 33].
CMLs are typically non-displaced and managed conser­
vatively.
18 Musculoskeletal System
a b
Fig. 18.28 Classic metaphyseal lesions (CMLs) in two different patients.
(a) Frontal radiograph of the distal tibia in a 66-day-old male reveals a
CML (arrowhead) that is triangular in configuration. (b) Frontal radio-
a b
Fig. 18.29 Classic metaphyseal lesion in a 3-month-old female. Lon­
gitudinal grayscale ultrasound images of the knee from anterior (a) and
posterior (b) approaches demonstrate abnormally increased separation
of the metaphyseal bone collar (arrows) from the underlying bone.
Tumors
Osteochondroma
Osteochondroma, also known as exostosis, is the most com-
mon benign bone tumor, comprising 20–50% of benign
tumors, 10–15% of all bone tumors, and occurring in 1–2%
of individuals. It is also the most common benign, radiation-
induced tumor, seen in 6–24% of irradiated patients. It is most
often a solitary lesion, although multiple lesions can be pres-
ent, as seen in multiple hereditary exostoses (MHE) [51, 52].
An osteochondroma is a subperiosteal bony projection
that develops from progenitor cells in the perichondrium. It
is continuous with the adjacent cortex and medullary space
and has an overlying cartilage cap. In MHE, mutations in
perichondrial cells allow the formation of abnormal ectopic
endochondral bone. In addition, perichondrial injury sec-
ondary to trauma and radiation can cause the formation of
a solitary exostosis [32]. In children, the cartilaginous cap
is the site of growth for the osteochondroma. After skeletal
maturity, it should not increase in size.
Diagnosis of osteochondroma is readily made on radio-
graphs, which will demonstrate the osseous protuberance at
853
graph of the distal tibia in a 29-day-old male shows a CML (arrows) with
a bucket handle configuration. (Images courtesy of Dr. Andy Tsai, Boston
Children’s Hospital and Harvard Medical School, Boston, MA, USA)
c
There is hypoechoic fluid (arrowheads) along the periosteum and an
echogenic hematoma (asterisks) indicative of fracture. (c) Lateral
radiograph of the knee confirms the presence of a healing CML
(arrowheads)
the metaphysis or metadiaphysis, in continuity with the cor-
tex and medullary space of the involved bone. Cross-­sectional
imaging, typically MR imaging, may be obtained when there
is concern for complication such as compression of neuro-
vascular structures, bursa formation, fracture, or malignant
transformation into chondrosarcoma or osteosarcoma.
In a skeletally mature patient, a cap thickness of 1–2 cm
raises concern for malignant transformation, which occurs in at
most 1% of solitary lesions, although in a greater percentage of
lesions in patients with MHE. In skeletally immature patients,
particularly those with a solitary osteochondroma, malignant
transformation is rare, and the cartilage cap can be thicker than
in adults, with a typical thickness of 1–3 cm [51, 53].
Osteochondroma can be diagnosed on ultrasound, which
typically occurs during evaluation of a palpable lump. The
imaging findings are the same as on radiography: a bony pro-
jection in cortical continuity with the remainder of the involved
bone. In addition, ultrasound can depict the hypoechoic carti-
lage cap along the distal margin of the ­exostosis (Fig. 18.22)
and provide accurate measurements of its thickness.
Osteochondroma can cause symptoms related to pressure
effects, overlying adventitial bursitis, compression, or dis-
854 D. Y. Jarrett

placement of neurovascular structures. Depending on size Ultrasound findings of septic arthritis include joint effu-
and location, it may also cause cosmetic deformity. MHE is sion, with the fluid appearing hypo- or hyperechoic, depend-
often associated with limb deformities, including shortening ing on its contents. This is a non-specific finding which must
of the ulna, radial head dislocation, and valgus alignment at be interpreted in the appropriate clinical context. Power and
the hip and knee [54]. color Doppler assessment may show increased blood flow
Treatment for symptomatic osteochondroma is surgical in the joint, indicative of hyperemia. However, the absence
excision. Excision is also performed if there is concern for of hyperemia does not exclude infection (Fig. 18.30) [55].
malignant transformation, with further treatment depending Aspiration of the joint and analysis of the synovial fluid
on histology of the lesion. may be required for accurate diagnosis. Ultrasound guid-
ance can be used to guide joint fluid aspiration. It is impor-
tant to note that the absence of a joint effusion does not
Joints exclude septic arthritis, particularly in joints with a non-­
distensible capsule, such as the sternoclavicular joint [7].
Effusion, infection, and inflammation can affect the numer- Septic arthritis is treated by drainage and irrigation, par-
ous synovial joints in the body. The following sections will ticularly when it involves a large joint, with needle aspiration,
review the ultrasound technique for evaluation of the intra-­ arthroscopy, or arthrotomy depending on the joint involved
articular space of specific joints. and the severity of infection. Treatment also includes anti-
biotics with empiric therapy targeting the most common
organisms, including MRSA [45].
Infectious/Inflammatory Disorders
J uvenile Idiopathic Arthritis
Septic Arthritis Juvenile idiopathic arthritis (JIA) is the most common rheumatic
Septic arthritis is a joint infection that most commonly affects the disease in children, with a reported prevalence between 16 and
shoulder, elbow, hip, knee, and ankle. The most common organ- 150 per 100,000 [56]. The term refers to a heterogeneous group
isms are S. aureus, followed by S. pyogenes and Streptococcus of disorders with chronic arthritis developing in children prior
pneumoniae. Additional causative organisms seen in neonates are to 16 years of age and with no identifiable ­etiology. Patients are
group B streptococci and coliform bacteria [7]. divided into different categories, based on factors such as the

a b

Fig. 18.30 Septic arthritis of the shoulder in a 9-year-old male. (a) color Doppler ultrasound image reveals hyperemia (arrows) within the
Longitudinal grayscale ultrasound image shows a large, complex joint joint space and in the adjacent soft tissues (arrowheads)
effusion (arrow) distending the shoulder joint capsule. (b) Transverse
18 Musculoskeletal System
number of joints involved, rheumatoid factor and HLA-B27
positivity, and certain extra-­articular associations [57].
Patients present with joint stiffness that is most pronounced
in the morning and following inactivity, joint pain, swelling,
and limited range of motion. JIA can lead to increased growth
of the affected side, as well as premature physeal closure, with
shortening of the affected limb. Chronic inflammation leads
to loss of joint cartilage with joint space narrowing, bone ero-
sions, and deformity. The hip is the most commonly involved
joint, representing 20–50% of cases [58].
Ultrasound is a useful imaging technique in patients
with JIA as it allows assessment of multiple joints for effu-
sion, synovitis, tenosynovitis, and bursitis at one sitting
(Figs. 18.31 and 18.32). Studies have shown that ultrasound
is better at detecting joint inflammation than clinical exami-
nation [56]. Ultrasound can also visualize the articular carti-
lage and determine cartilage thinning [59, 60].
Ultrasound has been of limited utility in the assessment
of treatment efficacy because of operator dependency and
P recurrent hemarthrosis can lead to synovitis, with synovial
Fig. 18.31 Synovitis of the knee in a 17-month-old female with juve-
nile idiopathic arthritis (JIA). Anterior longitudinal color Doppler ultra-
sound image of the suprapatellar recess demonstrates abnormally
thickened, hypoechoic synovium (arrowheads) and associated hyper-
emia. There is a small amount of joint fluid (arrow) between the thick-
ened synovial layers. P, Patella; F, distal femoral epiphysis
a b
Fig. 18.33 Hemarthrosis of the knee in an 11-year-old female. (a)
Transverse and (b) longitudinal grayscale ultrasound images of the
855
lack of a standardized scoring system. Several issues of con-
cern have been the lack of rigorous ultrasound definitions
of effusion and synovitis, and the fact that synovitis can be
present in zones of synovial hypertrophy even in the absence
of hypervascularity on color Doppler imaging. Another con-
founding factor is that physiologic Doppler signal is nor-
mally present in the joint and synovial recesses [61].
Disease-modifying anti-rheumatic drugs (DMARDS)
are first-line therapy for JIA, including conventional medi-
cations such as methotrexate, and biologic agents such as
tumor necrosis factor inhibitors. NSAIDS can be used along
with DMARDS, and glucocorticoids may also be helpful in
reducing joint inflammation [57, 62].
Hemarthrosis
Hemarthrosis, or intra-articular hemorrhage, can be second-
ary to a wide range of processes such as intra-articular frac-
ture, hemophilia, pigmented villonodular synovitis, and rarely
intra-articular vascular malformation. Treatment depends on
the underlying etiology.
On ultrasound, hemarthrosis will appear as a complex
joint effusion with mobile internal echoes. Longstanding or
thickening visible on ultrasound (Fig. 18.33).
Fig. 18.32 Tenosynovitis in a 12-month-old male with JIA. Transverse
grayscale ultrasound image of the extensor tendons on the dorsal aspect of the
right wrist demonstrates hypoechoic fluid (arrow) within the tendon sheath
suprapatellar joint recess show a large amount of complex fluid (arrows)
containing echogenic debris
856 D. Y. Jarrett

Shoulder humeral head to the metaphysis with no angulation in the

contour or disruption of the bone. This relationship should be
Normal Anatomy and Imaging Approaches evaluated circumferentially around the humerus (Figs. 18.19
and 18.35) [66].
Normal Anatomy
The humeral head is largely cartilaginous at birth. There are
two separate ossification centers in the epiphysis. The more
medial ossification center of the humeral head may be present
at birth in full-term infants and is typically radiographically
evident by 2–3 months of age. The more lateral ossification
center of the greater tuberosity appears by 7 months of age,
with complete fusion of the ossification centers by 5–7 years
of age (Fig. 18.34) [63, 64].
E
Patient Positioning
To evaluate the proximal humeral epiphyseal-metaphyseal
relationship, the patient can be examined supine to assess the
anterior and lateral margins of the humerus, and prone for
posterior assessment.
M
Effusions are most reliably detected at the posterior
recess of the glenohumeral joint, with the patient prone and
the upper arm externally rotated [65].
To assess for subluxation in the setting of glenohumeral dys-
plasia, the patient is imaged sitting upright, either held or in a
booster chair, with the arm adducted and the elbow flexed 90°.
Fig. 18.35 Salter 2 fracture of the proximal humerus in a 14-day-old
Imaging Approaches female. Longitudinal grayscale ultrasound image shows separation
The epiphyseal-metaphyseal relationship can be evaluated (arrowhead) of the cartilaginous epiphysis (E) from the metaphysis (M)
by placing the transducer longitudinally along the proxi- consistent with a physeal fracture. The fracture (arrow) extends through
the lateral margin of the metaphysis. (Compare to the normal appear-
mal humerus. There should be a smooth transition from the
ance of the proximal humerus in Fig. 18.19)

a b

Fig. 18.34 Normal humeral head ossification centers in two different Posterior approach transverse grayscale ultrasound image of the right
patients. (a) Frontal radiograph of a 26-month-old male demonstrates glenohumeral joint in a 9-month-old female shows the medial humeral
the medial ossification center (arrow) of the humeral head and the lat- head ossification center (arrow) and the lateral ossification center
eral ossification center (arrowhead) of the greater tuberosity. (b) (arrowhead) of the greater tuberosity
18 Musculoskeletal System
Glenohumeral joint alignment is best evaluated in the
transverse plane at the level of the mid humeral head. In the
normal shoulder, this produces a transverse image of the
round humeral head centered along the articular surface of
the glenoid. In neonates, this view can be obtained from an
anterior, lateral, or posterior approach. However, as the age
and size of the patient increase and the ossification of the
humeral head increases, a posterior approach will provide
a b
Fig. 18.36 Scanning technique for the glenohumeral joint. (a) With
the patient sitting upright, the ultrasound transducer is placed trans-
versely over the posterior aspect of the right glenohumeral joint. (b)
a b
Fig. 18.37 Shoulder hemarthrosis in a 4-year-old male with hemo-
philia. (a) Posterior approach transverse grayscale ultrasound image
demonstrates the hypoechoic, unossified cartilage (arrowhead) of the
left humeral head overlying the ossified portion (asterisk) of the humeral
head. There is a rim of hypoechoic fluid (arrow) in the joint space.
857
the most reliable view for assessment of the glenohumeral
articulation, although the anterior margin of the glenoid may
not be visible (Fig. 18.36).
Images at the level of the humeral head permit assess-
ment for a shoulder joint effusion. When the humeral head is
incompletely ossified, appropriate gain settings must be used
to allow the hypoechoic cartilage of the humeral head to be
distinguished from surrounding fluid (Fig. 18.37).
Posterior approach transverse grayscale ultrasound image of the shoul-
der in a 5-month-old female demonstrates the humeral head (arrow)
articulating medially with the glenoid fossa (arrowhead)
(b) Posterior approach transverse grayscale ultrasound image of the
right shoulder shown for comparison shows the hypoechoic unossified
cartilage (arrowhead) of the humeral head overlying the ossified
humeral head (asterisk). There is no fluid in the joint space
858
Congenital/Developmental Abnormalities
Glenohumeral Dysplasia
Glenohumeral dysplasia (GHD) is a deformity of the shoul-
der, acquired as a complication of brachial plexus birth palsy
(BPBP). BPBP results from traction on the brachial plexus
during delivery and occurs in up to 3 per 1000 live births
[67]. Some infants have a full recovery from this injury, with
reports ranging from 13% to 80%. Others, however, have pro-
gressive muscle weakness of the shoulder girdle which may
result in abnormal development of the glenohumeral joint and
growth disturbance with a hypoplastic humeral head [68]. It
is not clear exactly how injury of the brachial plexus leads to
GHD. It is likely that the resulting muscle imbalance, with
strong internal rotators and weak external rotators, results in
abnormal growth of the developing glenoid.
Of particular concern in the management of GHD is the
development of posterior subluxation or dislocation of the
humeral head. Complete dislocation has been reported in 8%
of patients with BPBP [69] with a lesser degree of sublux-
ation seen in up to one-third [70]. Physical examination find-
ings that suggest posterior dislocation include limited passive
external rotation at the shoulder, palpable abnormality at the
posterior shoulder, and apparent shortening of the humerus.
However, in one study, 16% of posterior dislocations were
missed by physical examination. Importantly, ultrasound has
been shown to be an accurate screening method for this diag-
nosis [68].
a b
Fig. 18.38 Brachial plexus birth palsy with glenohumeral dysplasia in
a 3-month-old female. (a) Posterior approach transverse grayscale
ultrasound image of the left shoulder in internal rotation reveals poste-
rior subluxation of the humeral head (arrowhead) relative to the dys-
D. Y. Jarrett
From a posterior approach, the transducer is placed in a
transverse oblique plane along the scapular spine, and the
position of the humerus relative to the glenoid is assessed. Images
are often obtained with the shoulder in internal rotation, as this
is the preferred position for infants with GHD. However if
the humeral head is abnormally displaced, dynamic images
of the glenohumeral joint can be obtained as the shoulder is
moved from internal to external rotation, to assess for reduc-
tion of the humeral head in external rotation (Fig. 18.38,
Cineclips 18.4 and 18.5) [71].
In addition to position, the size of the cartilaginous and
ossified portions of the humeral heads should be compared to
assess for potential hypoplasia on the affected side. The pos-
terior transcapular view also demonstrates the infraspinatus
and deltoid muscles, which may be atrophic in the setting of
BPBP and GHD.
Ultrasound is most effective for screening patients with
BPBP for subluxation, and to monitor for change on serial
examinations. MR imaging remains the gold standard for eval-
uating glenoid morphology and glenohumeral joint alignment.
As with hip ultrasound, angle measurements and percent
coverage can be measured on shoulder ultrasound to assess
the severity of deformity. The alpha or scapula–glenoid–
humeral head (SGH) angle is the angle between a line along
the posterior margin of the scapula and a line tangent to the
humeral head which passes through the posterior osseous
margin of the glenoid. It is a measure of the position of the
humeral head relative to the axis of the scapula and in normal
plastic, convex glenoid fossa (arrow). (b) Posterior approach transverse
grayscale ultrasound image of the left shoulder in external rotation
demonstrates reduction of the humeral head (arrowhead) into the gle-
noid fossa (arrow)
18 Musculoskeletal System 859

shoulders should be less than or equal to 30°. Greater than
50% of the humeral head should be anterior to the scapular
line, with lower percentages seen in patients with posterior
subluxation (Fig. 18.39) [72].
When compared to MR imaging, ultrasound has been shown
to underestimate the alpha angle and posterior humeral head
displacement and can only poorly visualize the osseous con-
tours of the glenoid, particularly as humeral head ossifica-
tion increases, limiting its utility in the assessment of glenoid
­version [73].
Proposed treatments for infantile GHD include closed
reduction with shoulder spica cast placement and extra-­
articular tendon transfers. These procedures are most effec-
tive if performed before 2 years of age [70].

a b

H H

c d

H H

Fig. 18.39 Glenohumeral dysplasia in a 4-month-old male. (a) Posterior
approach transverse grayscale ultrasound image of the normal left
shoulder. The scapula-glenoid-humeral head (SGH) angle measures
27°, and more than 50% of the humeral head (H) is anterior to the scap-
ular line. (b) Posterior approach transverse grayscale ultrasound image
of the dysplastic right shoulder in the same patient. The SGH angle
measures 65°, and less than 50% of the humeral head is anterior to the
scapular line. Axial proton density MR image at 13 months of age shows
the normal left shoulder (c) with appropriately positioned humeral head
(H) relative to the glenoid fossa (arrow) and (d) progressive dysplasia
of the right shoulder with posteriorly subluxated humeral head (H) rela-
tive to the glenoid fossa (arrow)
860
Elbow
Normal Anatomy and Imaging Approaches
Normal Anatomy
At birth all of the epiphyses and apophyses of the elbow are
cartilaginous. Ossification typically begins around 6 months
to 1 year of age for the capitellum, 5–6 years for the radial
head, 3–7 years for the highly variable medial epicondyle,
7–8 years for the trochlea, 7–10 years for the olecranon, and
11–13 years for the lateral epicondyle (Fig. 18.40) [74, 75].
Patient Positioning
Imaging the anterior, medial, and lateral structures of the
elbow can be performed with the patient lying supine, with
supination of the elbow and forearm. Posterior imaging of
the elbow is accomplished with the patient prone, arms at the
a b
M L
T C
R
R
Fig. 18.40 Elbow ossification centers in a 9-year-old female. (a)
Coronal double-echo steady-state (DESS) MR image of the left
elbow. (b) Sagittal DESS image of the left lateral elbow. (c) Sagittal
DESS MR image of the left medial elbow. The lateral epicondyle
a b
Fig. 18.41 Anterior view of the normal elbow joint. (a) Longitudinal
transducer placement over the anterior antecubital fossa with elbow
extended. (b) Longitudinal grayscale ultrasound image of the radio-
capitellar joint in a 7-year-old female. The capitellum (white arrow) and
radial head (black arrow) are incompletely ossified, with overlying
D. Y. Jarrett
side, or sitting upright, facing away from the sonographer,
with arms extended at the sides.
Imaging Approaches
Anterior Approach
The radiocapitellar joint can be assessed from an anterior
approach (Fig. 18.41a). Placing the transducer longitudinally
at the lateral antecubital fossa produces a sagittal view of the
radiocapitellar joint. Until skeletal maturity, the hypoechoic
physes are seen separating the capitellum and radial head
from the adjacent metaphyses. Congruent alignment of the
convex capitellar articular surface is seen with the concave
radial head (Fig. 18.41b).
Moving the transducer to the medial aspect of the ante-
cubital fossa demonstrates the olecranon-trochlear joint
(Fig. 18.41c). It is particularly well seen in younger children,
c
O
C
T
(L) is entirely cartilaginous, while the other epiphyses and apophy-
ses are partially ossified. R, Radial head; C, capitellum; M, medial epi-
condyle; O, olecranon; T, trochlea
c
hypoechoic epiphyseal cartilage and a hypoechoic physis (arrowheads)
separating each epiphysis from each metaphysis. (c) Longitudinal gray-
scale ultrasound image of the cartilaginous olecranon process (arrow)
articulating with the trochlea (arrowhead) in a 12-month-old male
18 Musculoskeletal System
before progressive ossification of both the olecranon and the
trochlea obscures joint visualization.
Posterior Approach
The posterior approach is particularly useful in assessment
of the olecranon-trochlear joint in young children, before
ossification of the olecranon process prevents adequate visu-
alization. It also allows for evaluation of the triceps tendon,
which inserts on the olecranon (Fig. 18.42).
Medial Approach
Placing the transducer longitudinally at the medial aspect of
the elbow allows assessment of the olecranon-trochlear joint
in the coronal plane. The medial epicondyle is seen along the
superomedial aspect of the trochlea. The ulnar collateral liga-
a b
Fig. 18.42 Posterior view of the normal elbow joint. (a) Transducer is
oriented longitudinally over the posterior elbow with the patient lying
prone and elbow extended. (b) Longitudinal grayscale ultrasound
a b
Fig. 18.43 Medial view of the normal elbow joint. (a) Transducer
placement over the medial aspect of the extended elbow. (b)
Longitudinal grayscale ultrasound image of the medial elbow in a
3-year-old female. The medial epicondyle (white arrow) is mostly car-
861
ment and common flexor tendon insert on the medial epicon-
dyle (Fig. 18.43). The medial epicondyle can also be seen on
transverse imaging, and this plane is useful to demonstrate
appropriate alignment if there is concern for avulsion injury.
Lateral Approach
Placing the transducer longitudinally at the lateral aspect of
the elbow allows evaluation of the radiocapitellar joint in
the coronal plane (Fig. 18.44). Dynamic imaging, with flex-
ion and extension of the elbow, can be performed to evalu-
ate for change in radiocapitellar alignment with flexion.
The lateral epicondyle is seen along the superolateral
aspect of the capitellum. The radial collateral ligament, lat-
eral ulnar collateral ligament, and common extensor tendon
insert on the lateral epicondyle. The lateral epicondyle can
image of the olecranon-trochlea joint in a 6-month-old male. The olec-
ranon process (O) and trochlea (T) are entirely cartilaginous. The tri-
ceps tendon (arrow) inserts on the olecranon process
tilaginous but shows early ossification. Distal to the epicondyle is the
cartilaginous trochlea (black arrow) articulating with the proximal ulna
(arrowhead)
862
also be seen on transverse imaging, which may be useful to
demonstrate appropriate alignment if there is concern for
avulsion injury.
Annular Ligament
While a detailed description of the ligaments and tendons
of the elbow is beyond the scope of this chapter, the annular
ligament deserves special attention for its role in a com-
mon pediatric elbow injury, the pulled elbow. The annular
ligament is the primary stabilizer of the proximal radioul-
nar joint and allows for rotation of the forearm [76]. As
Fig. 18.44 Lateral view of the normal elbow joint in a 6-year-old
male. Longitudinal grayscale ultrasound image obtained from a lateral
approach shows the cartilaginous lateral epicondyle (white arrow).
Distal to the epicondyle is the cartilaginous, partially ossified capitel-
lum (black arrow) articulating with the cartilaginous radial head
(arrowhead)
a b
Fig. 18.45 Normal annular ligament and supinator muscle. (a) Anterior
approach longitudinal grayscale ultrasound image of the radiocapitellar
joint. The annular ligament (arrow) is a thin, echogenic structure at the
anterior aspect of the radial head and neck. The supinator muscle
D. Y. Jarrett
the name suggests, it tightly encircles the radial head with
bony attachments at the anterior and posterior aspects of
the lesser sigmoid notch of the ulna and a loose attachment
to the radial neck. At the level of the radial head, the liga-
ment is thicker and easier to visualize, and is in continuity
with the joint capsule [76, 77]. It is also fused with the deep
fibers of the supinator muscle [77, 78].
The annular ligament is most easily seen on ante-
rior longitudinal ultrasound images of the radiocapitel-
lar joint as a thin echogenic structure along the anterior
aspect of the radial head. The overlying supinator muscle
can be assessed on both sagittal and transverse images
(Fig. 18.45).
Elbow Fat Pads and Joint Effusion
There are three intracapsular, extra-synovial fat pads in the
elbow, two located anteriorly in the radial and coronoid fos-
sae of the distal humerus, and one located posteriorly in the
olecranon fossa [79]. In the presence of a joint effusion,
radiographs can detect displacement of these fat pads from
their fossae, allowing diagnosis of the effusion.
Ultrasound allows visualization of the fat pads, whether
they are normally positioned or displaced by an effusion, and
can directly demonstrate joint fluid, if present. Ultrasound
can also be used to characterize the fluid as simple or com-
plex, and to assess for synovitis or hyperemia with color
Doppler imaging (Fig. 18.46). The synovial membrane of
the elbow extends from the distal humerus to the radial neck.
In the setting of a large effusion, fluid can be seen at the level
of the radial neck.
(arrowhead) is located anterior to the ligament. (b) Transverse grayscale
ultrasound image of the supinator muscle (arrowhead) at the level of the
radial neck
18 Musculoskeletal System
a b
c d
Fig. 18.46 Elbow joint fat pads and joint effusion. (a) Anterior approach
longitudinal grayscale ultrasound image of the elbow shows the nor-
mally positioned anterior fat pad (arrow). (b) Anterior approach longi-
tudinal grayscale ultrasound image of the elbow demonstrates a
hypoechoic joint effusion (asterisk) displacing the anterior fat pad
(arrow) anteriorly. (c) Posterior approach longitudinal grayscale ultra-
Congenital/Developmental Abnormalities
 ongenital Radial Head Dislocation
C epiphyseal separation of the elbow. Ultrasound can visual-
Congenital dislocation of the radial head is the most com-
mon congenital abnormality of the elbow. It can occur in
isolation, although it is frequently associated with other
congenital anomalies or syndromes, and may be unilat-
eral or bilateral. One etiologic theory is abnormal capitel-
lar development in utero, with hypoplasia and flattening of
the capitellum preventing normal formation of the radio-
capitellar joint. The radial head develops a dome-shaped
configuration and subluxates or dislocates, most frequently
posteriorly. Children may present with decreased range of
motion or visible deformity of the elbow [80, 81].
863
sound image of the olecranon-trochlear joint depicts the posterior fat
pad (arrowhead) normally positioned in the olecranon fossa. (d)
Posterior approach longitudinal grayscale ultrasound image of the
olecranon-trochlear joint shows the posterior fat pad (arrowhead) dis-
placed from the olecranon fossa by joint fluid (asterisk)
In the absence of epiphyseal ossification at the elbow
joint, the radiologic appearance may mimic a traumatic
ize the cartilaginous epiphyses and distinguish between
these two entities. With dislocation, the anterior longitudinal
image of the elbow will show posterior or anterior displace-
ment of the radial head relative to the capitellum, absence of
a physeal fracture of the distal humerus or proximal radius,
and preserved epiphyseal-metaphyseal alignment. In con-
genital dislocation, there may also be morphologic changes
of the epiphyses with a hypoplastic, flattened capitellum and
domed-shaped radial head articular surface (Fig. 18.47).
Congenital radial head dislocation is typically treated
conservatively until the onset of symptoms which often
864
a b
Fig. 18.47 Congenital radial head dislocation in a 4-month-old female.
(a) Longitudinal grayscale ultrasound image of the anterior radiocapitel-
lar joint demonstrates anterior dislocation of the radial head (arrowhead)
develop in adolescence. The most common treatment is
excision of the radial head once the patient reaches skeletal
maturity. Some advocate attempting to surgically reduce
the joint [81].
Trauma
While radiographs remain the preferred modality for evalua-
tion of most elbow fractures, ultrasound is of particular utility
in the assessment of radiographically occult fractures involv-
ing unossified epiphyses and apophyses. Ultrasound allows
visualization of the cartilaginous structures of the pediatric
elbow, without the time, expense, and potential patient seda-
tion required for MR imaging.
 istal Humeral Epiphyseal Separation
D elbow joint effusion or hemarthrosis (Figs. 18.48 and 18.49).
Physeal fracture of the distal humerus is a rare injury, typi-
cally seen in children less than 2 years of age, and can be
caused by birth trauma, a fall on an outstretched hand, or
non-accidental trauma [82]. The distal humeral epiphysis
is separated from the metaphysis and displaced posteriorly
and medially, as seen with supracondylar fractures [83]. The
elbow joint remains intact, with the displaced capitellum and
trochlea maintaining appropriate alignment with the radius
and olecranon.
In neonates, an epiphyseal separation cannot be definitively
differentiated from a posterior elbow dislocation on radio-
D. Y. Jarrett
relative to the capitellum (arrow). (b) Elbow radiograph in the same
patient. The capitellum and radial head are not yet ossified, but the align-
ment of the distal humerus with the radial shaft (R) is abnormal
graphs because the epiphyses are not yet ossified. However,
traumatic elbow dislocations are very rare in neonates, and the
presumed diagnosis should be an epiphyseal separation [83].
One potential clue to the diagnosis on radiographs is the
direction of displacement, with posteromedial displacement
of the radius and ulna most commonly seen with epiphyseal
separation, whereas posterolateral displacement occurs with
dislocation [82, 84].
The diagnosis of distal humeral epiphyseal separation can
be confirmed by ultrasound. Anterior and posterior approach
sagittal views of the elbow will demonstrate disruption of the
distal humeral physis and posterior displacement of the carti-
laginous capitellum and trochlea relative to the metaphysis.
Radiocapitellar and olecranon-trochlear alignment are main-
tained. Echogenic material at the fracture site may be identified,
consistent with hemorrhage and edema. There may also be an
Treatment for distal humeral epiphyseal separation ranges
from immobilization to reduction and pinning [85].
Apophyseal Avulsion
Ultrasound is useful in the evaluation of other radiographically
occult elbow fractures, such as medial epicondyle apophy-
seal avulsions in children less than 5 years of age, before the
apophysis is ossified [86]. Ultrasound allows visualization of
the displaced apophyseal cartilage and hemorrhage/edema at
the physeal fracture site. It can also assess for associated tear
of the ulnar collateral ligament (Fig. 18.50).
18 Musculoskeletal System
a b
Fig. 18.48 Distal humeral epiphyseal separation in a 9-month-old
female with suspected child abuse. (a) Lateral radiograph of the elbow
shows mild posterior malalignment of the capitellar ossification center
(arrow) relative to the humerus. (b) Posterior approach longitudinal
a b
Fig. 18.49 Distal humeral epiphyseal separation in a 6-day-old female.
(a) Frontal right elbow radiograph shows malalignment of the ossified
portion of the distal humerus (H) with the radius and ulna. (b) Posterior
approach longitudinal grayscale ultrasound image of the elbow. There
is preserved alignment of the capitellum (white arrowhead) with the
radius (black arrowhead). The distal humeral metaphysis does not artic-
a b
Fig. 18.50 Medial epicondylar avulsion fracture in a 5-year-old male.
(a) Frontal radiograph of the right elbow. The medial epicondyle is not
ossified. There is medial soft tissue swelling (arrow). (b) Medial
approach longitudinal grayscale ultrasound image of the right elbow.
865
grayscale ultrasound image of the elbow confirms epiphyseal separa-
tion with a Salter 2 fracture of the distal humerus and posterior dis-
placement of the capitellum (arrowhead). There is complex joint fluid
(arrow) consistent with hemarthrosis
c
ulate with the capitellum. A heterogeneous appearance of the adjacent
soft tissues (asterisk) reflects hemorrhage and edema. (c) Anterior lon-
gitudinal grayscale ultrasound image of the right elbow joint depicts the
distal humeral shaft (arrow) without the expected visualization of the
cartilaginous capitellum
There is an avulsion fracture with separation of the cartilaginous medial
epicondyle (E) and underlying bone from the reminder of the metaphy-
sis (M), with intervening hypoechoic fluid (arrowhead)
866
Pulled Elbow
Pulled elbow, also known as nursemaid elbow, is typically seen
in children less than 6 years of age, and results from traction
on an extended, pronated elbow. This injury can occur when a
caretaker pulls on the arm, as when attempting to lift a child or
prevent them from falling [87, 88]. Clinically, there is an acute
onset of pain and limitation of upper extremity movement, and
the arm is held in pronation. While the precise pathophysiology
of this disorder is unclear, one theory is that traction on the radius
causes the radial head to subluxate and become entrapped dis-
tal to the annular ligament. This is possible in younger children
because of the relatively weak attachments of the annular liga-
ment to the radius and increased pliability of the incompletely
ossified radial head [87, 89].
Pulled elbow is often diagnosed clinically, with acute
onset of pain with movement [88]. However, imaging is
sometimes requested when the diagnosis is uncertain, after
attempted elbow manipulation, to assess for appropriate
reduction of the annular ligament and joint, or to evaluate the
annular ligament in recurrent cases.
Ultrasound can demonstrate both direct and indirect find-
ings. In pulled elbow, both the annular ligament and the
superior margin of the supinator muscle (which has a broad
attachment onto the annular ligament) are displaced proxi-
mally into the radiocapitellar joint. This is best visualized
on anterior longitudinal imaging of the radiocapitellar joint.
The thin echogenic band of the annular ligament is no lon-
ger visualized anterior to the radial head and the proximally
displaced supinator muscle curves over the anterior margin
of the radial head, in what has been described as the “J” sign
(Fig. 18.51) [90].
Fig. 18.51 Pulled elbow in a 20-month-old male. Longitudinal gray-
scale ultrasound image of the anterior radiocapitellar joint demonstrates
thickening, increased echogenicity, and proximal displacement of the
supinator muscle (arrow). The annular ligament is not identified ante-
rior to the radial head (R)
D. Y. Jarrett
On transverse images, in the absence of a joint effusion,
the displaced annular ligament may be challenging to iden-
tify at the anterior joint space, although the proximal supina-
tor muscle can be seen as thickened and echogenic, related
to partial tear.
Pulled elbow is treated by manipulation, with preferential
use of hyperpronation compared to a supination maneuver [91].
Wrist and Hand
Normal Anatomy and Imaging Approaches
Normal Anatomy
There are many articulations of the wrist and hand which are
amenable to ultrasound assessment. The three carpal joints
are the radiocarpal, ulnocarpal, and radioulnar joints. The
midcarpal joint separates the proximal and distal carpal rows.
The metacarpophalangeal (MCP) and interphalangeal joints
are located more distally in the hand.
In the wrist, ossification of the capitate occurs at 3–6 months
of age, followed by the hamate, triquetrum, lunate, scaphoid,
trapezium, trapezoid, and lastly the pisiform at 6–8 years of
age. Ossification of the distal radial epiphysis occurs between
8 and 18 months of age, and of the distal ulnar epiphysis at
5–7 years of age. Distal radial and ulnar physeal fusion occurs
at 16–19 years of age (Fig. 18.52) [92].
Fig. 18.52 Normal carpal ossification in an 11-year-old female. Coronal
DESS MR image of the wrist shows partially ossified carpal bones, and
open physes at the distal radius (R) and ulna (U). S, Scaphoid; L, lunate; T,
triquetrum; Tr, trapezium; Tz, trapezoid; C, capitate; H, hamate
18 Musculoskeletal System
Patient Positioning
Imaging of the hand and wrist can be performed with the
patient in any position that allows adequate range of motion
for assessment of the region of interest, often sitting upright
or lying supine. The water bath technique is typically carried
out with the patient sitting upright and the basin of water
placed nearby on a flat surface.
Imaging Approaches
Intercarpal articulations are more easily assessed along
the dorsal aspect of the wrist and hand. Use of the water
bath technique, with shallow submersion of the hand in a
basin of water during scanning, is very useful when imag-
ing multiple adjacent small joints, allowing the probe to
be moved from joint to joint without the need for re-appli-
cation of ultrasound gel or repositioning of a standoff pad
(Fig. 18.1).
The extensor tendons of the hand and wrist are divided
into six compartments, with separate tenosynovial sheaths
[93]. On ultrasound imaging, Lister’s tubercle, a bony
prominence located dorsally at the distal end of the radius,
is a useful landmark that separates compartments II and III
(Fig. 18.53). With the transducer transversely oriented on the
dorsal aspect of the wrist, the tendon of interest can be traced
to the level of the distal radius, to determine the location of
its sheath relative to Lister’s tubercle.
a b
LT
III IV V
II
I IV
c d
Fig. 18.53 Normal flexor and extensor tendon anatomy of the wrist.
(a) Axial DESS image of the wrist at the level of Lister’s tubercle (LT)
demonstrates the extensor tendon compartments. (b) Transverse gray-
scale ultrasound image of the wrist at the level of Lister’s tubercle with
anatomy corresponding to (a). (c) Axial DESS MR image of the wrist
867
Flexor tendons are assessed along the volar aspect of the wrist
and hand (Fig. 18.53). On transverse images, they can be seen
passing through the carpal tunnel, bounded on their volar aspect
by the flexor retinaculum, medially by the hook of the hamate,
and laterally by the tubercle of the trapezium. The flexor carpi
ulnaris, the flexor carpi radialis, and the inconsistently present
palmaris longus tendon remain outside of the carpal tunnel. The
median nerve passes through the carpal tunnel, while the ulnar
nerve is located separately in Guyon’s canal.
The median nerve courses close to the flexor carpi radia-
lis and palmaris longus tendons, and the differential effects
of anisotropy on tendons and nerves can help to distinguish
these structures. Tendons demonstrate uniform anisotropy, and
the entire tendon will appear decreased in echogenicity as
the ultrasound transducer is angled away from the perpen-
dicular plane. However, in peripheral nerves, anisotropy
only affects the echogenic connective tissue surrounding the
nerve fascicles (Fig. 18.4, Cineclip 18.1) [94].
To fully evaluate a tendon, it should be traced from the
myotendinous junction to the bony insertion site. Ultrasound
is very useful in the hand and wrist to identify which tendons
are present in the setting of congenital abnormalities, and to
assess for tendon pathology such as tenosynovitis, tendino­
pathy, and tendon tears. Dynamic imaging, with flexion and
extension of the fingers, can improve visualization of tendon
tears by increasing retraction between the torn margins [94].
LT
II V
III
at the level of the pisiform bone (P) depicts the flexor tendons of the
wrist, median nerve (white arrowhead), and ulnar nerve (black arrow-
head). (d) Transverse grayscale ultrasound image of the volar aspect of
the wrist with anatomy corresponding to (c)
868 D. Y. Jarrett

Ganglia When imaging is warranted, ultrasound has been shown

to be very accurate in the diagnosis of a ganglion cyst.
A ganglion is a cystic lesion surrounded by connective tissue Imaging findings are variable, depending on cyst content. A
and filled with gelatinous fluid. It is commonly juxta-­articular simple ganglion cyst may be hypoechoic with a thin wall and
and most frequently seen at the wrist, although it can also be posterior acoustic enhancement. A ganglion cyst can also be
intra-articular in location [95]. In contrast, a synovial cyst complex with a thick wall and septations. A complex gan-
is a synovial-lined collection of joint fluid which is juxta-­ glion cyst may contain echogenic debris, and rarely, poste-
articular and may or may not communicate with the joint. rior acoustic enhancement may be absent (Fig. 18.54) [98].
Ganglion cyst is the most common wrist mass in children, A ganglion cyst may be mistaken for a venous malformation
often presenting as a painless bump [96]. While older chil- on grayscale imaging. However, color Doppler imaging of
dren and adults are more likely to have a ganglion on the dor- a ganglion cyst should demonstrate no internal vascularity,
sal aspect of the wrist, children less than 10 years of age may with any detectable flow limited to the wall [99].
have an increased incidence of volar wrist and volar retinacular Ganglion cysts in children can be treated conservatively,
ganglion cysts. The volar retinacular ganglion cyst occurs at the with surgery considered only for those with persistent cysts.
level of the MCP joint, arises from the flexor tendon sheath, and Variable rates of cyst persistence and post-surgical recur-
involves the first annular ligament (the A1 pulley) [97]. rence are noted in the literature [100].

b c

Fig. 18.54 Ganglia of the hand and wrist. (a) Volar retinacular cyst in a
14-month-old male. Longitudinal grayscale ultrasound image of the
third ray shows an anechoic cyst (arrow) along the volar aspect of the
extensor tendon at the level of the metacarpophalangeal (MCP) joint
(arrowhead). (b) Dorsal wrist cyst in a 14-year-old male. Transverse
grayscale ultrasound image of the dorsal wrist shows a loculated fluid
collection (arrow) with internal septations and increased through trans-
mission. (c) Longitudinal color Doppler ultrasound image of the same
patient as in (b) shows vascularity (arrowhead) limited to the septations
18 Musculoskeletal System 869

a b c

Fig. 18.55 Carpal boss in a 15-year-old male. (a) Transverse and (b) (arrows) articulating with the metacarpal base. (c) Lateral radiograph of
longitudinal grayscale ultrasound images of the dorsal base of the left the left wrist confirms the presence of a carpal boss (arrow)
third metacarpal bone demonstrate an irregular osseous protuberance

Congenital/Developmental Abnormalities a

Carpal Boss
Carpal boss is a bony prominence at the dorsal wrist which can
also present as a dorsal wrist mass, similar to a ganglion. In
children, it is typically related to an os styloideum, an accessory
bone located between the trapezoid and capitate proximally,
and the second and third metacarpal bases distally. Carpal b
boss is most often asymptomatic, but can cause pain because
of altered joint mechanics leading to degenerative changes, or
from direct contact with the adjacent soft tissues [101].
Ultrasound can readily distinguish a ganglion from a car-
pal boss by placing the probe over the palpable prominence
and demonstrating the echogenic, shadowing contour of the
accessory ossicle, rather than a cyst (Fig. 18.55).
Treatment for carpal boss is usually conservative with
Fig. 18.56 Giant cell tumor of the tendon sheath in an 11-year-old
surgical excision of the bone performed if conservative man- male. (a) Transverse grayscale ultrasound image at the level of the proxi-
agement fails. mal fourth metacarpal bone demonstrates an echogenic mass (arrow)
closely associated with the flexor tendon. (b) Longitudinal grayscale
ultrasound image of the mass (arrow) just distal to the MCP joint (white
arrowhead) reveals anisotropy of the flexor tendon, with decreased echo-
Tumors genicity at the distal aspect of the tendon (black arrowhead)

 iant Cell Tumor of the Tendon Sheath

G
Giant cell tumor of the tendon sheath (GCTTS) is a local-
ized, nodular tenosynovitis which is histologically the same
as pigmented villonodular synovitis [102].
GCTTS presents with gradually increasing soft tissue
swelling and a mass. In adults, it is the second most common
hand mass after ganglion. In children, it is much more rare
and has been shown to have an equal predilection for the
upper and lower extremities [103].
Although variable ultrasound appearances have been
described, GCTTS is most frequently hypoechoic and homo-
geneous in echotexture, with internal vascularity on color
Doppler imaging. It most often involves the volar surface
of the fingers. As it arises from the tendon sheath, GCTTS
will be in close contact with the tendon, possibly encasing
it. However, the mass does not move with the tendon dur-
ing dynamic imaging, and the tendon is usually normal in
appearance (Fig. 18.56, Cineclip 18.6) [93, 102].
GCTTS is treated by surgical excision.
Hip
Normal Anatomy and Imaging Approaches
Normal Anatomy
The hip is a ball and socket joint and is the articulation
between the femoral head and the acetabulum. The articular
surface of the acetabulum has contributions from the ilium,
870 D. Y. Jarrett

ischium, and pubis, with these bones converging at the trira-

diate cartilage (Fig. 18.57).
Femoral head ossification is variable, typically occurring
between 2 and 8 months of age [104]. Ossification centers for
the pelvic bones are present at birth. During puberty, three sec-
ondary ossification centers appear from the pubis, ilium, and
ischium and contribute to the anterior, superior, and posterior
walls of the acetabulum, respectively [105, 106]. The triradiate
cartilage fuses between 12 and 14 years of age [107].
The acetabulum has a fibrocartilaginous structure along
I its periphery, called the labrum, which deepens the acetabu-
lar cup. The hip joint capsule originates from the bony ace-
tabulum near the external aspect of the labrum and attaches
to the femoral neck (Fig. 18.58).

* Patient Positioning
Differing imaging approaches are used for the hip depending
IS P on the age of the patient and the indication for the examina-
tion (Fig. 18.59). To evaluate for developmental dysplasia of
the hip, the patient is imaged somewhere between a supine
and lateral decubitus position to allow the examiner easier
access to the hip. For coronal images, the infant’s hips may
either be flexed or in a physiologic neutral position at 15°–
20° of hip flexion. For transverse images, the infant’s hips
should be flexed to 90°.
In patients of any age, hip ultrasound can be used to
assess for the presence of joint effusion or synovitis. This is
Fig. 18.57 Normal hip joint bony anatomy. Lateral view of three- performed from an anterior approach, with the patient lying
dimensional computed tomography (CT) model of the acetabulum in a supine and the hips and knees extended.
4-year-old male. The ilium (I), ischium (IS), and pubis (P) converge at
the triradiate cartilage (asterisk)

a b

S S
IL RF

IL
RF

Fig. 18.58 Normal hip joint capsular anatomy. (a) Oblique axial pro- femoral neck. Arrows, Joint capsule; IL, iliopsoas muscle; RF, rectus
ton density MR arthrogram image and (b) longitudinal grayscale ultra- femoris muscle; S, sartorius muscle
sound image of the left hip, both oriented along the long axis of the
18 Musculoskeletal System
Imaging Approaches
Neonates and Infants for Hip Dysplasia
Hip ultrasound to assess acetabular morphology and joint
alignment can be reliably performed in the first few months
of life. In infants older than 4–5 months of age, accurate
ultrasound assessment for dysplasia becomes more difficult,
because of obscuration by the growing femoral head ossi-
fication center and the increased thickness of the overlying
soft tissues. After 5–6 months of age, patients are typically
evaluated with radiographs.
A high-frequency linear transducer should be used, which
allows adequate penetration of the soft tissues, typically
around 9 MHz. The morphology of the hip joint is initially
assessed in the coronal plane. The transducer is placed lon-
gitudinally or parallel to the long axis of the body, along the
lateral aspect of the hip (Fig. 18.59).
a b
Fig. 18.59 Patient and transducer positioning for hip joint evaluation.
When assessing for hip dysplasia, coronal images (a) are obtained with
the hip flexed or in neutral position. The transducer is placed longitudi-
nally along the lateral aspect of the hip. Transverse images (b) are
a Lateral
Superior
Inferior
Anterior
IL
IS
Fig. 18.60 Normal hip ultrasound examination in an 11-week-old
female. (a) Coronal and (b) transverse grayscale ultrasound images
show the cartilaginous femoral head (F), iliac bone (IL) which forms
871
The iliac bone will be seen as an echogenic shadowing line,
deep to gluteal muscles. The bony contour of the acetabulum is
located at the inferior margin of the iliac bone. The hypoechoic
cartilage and the acetabular labrum can be discerned along the
articular margin of the acetabulum. The rounded contour of
the femoral head is seen as a hypoechoic cartilaginous struc-
ture, with an echogenic focus of ossification variably present
depending on patient age. The cartilaginous greater trochanter
may also be viewed, particularly if the hip is not flexed to 90°.
Medial to the femoral head is the central acetabular fossa
which is non-articular and contains the pulvinar, a fibrofatty
fat pad which is extra-synovial in location [108]. Deep to
pulvinar are the hypoechoic triradiate cartilage and the echo-
genic, osseous ischium (Fig. 18.60).
The ultrasound transducer should be rotated and adjusted
until all of the imaging criteria are met for the standard coro-
nal plane:
c
obtained with the hip flexed to 90°. The transducer is placed over the
lateral hip joint, aligned parallel to the long axis of the femur. When
evaluating for joint effusion (c), the hip is extended. The transducer is
aligned parallel to the long axis of the femoral neck
b Lateral
F
Posterior
FM IS
the superior acetabulum, pulvinar (white arrowhead), triradiate carti-
lage (black arrowhead), ischium (IS), and proximal femoral metaphysis
(FM)
872
Fig. 18.61 Hip joint effusion in an 8-year-old male with left-sided pain
and limited range of motion. (a) Longitudinal grayscale ultrasound image
along the left femoral neck shows anechoic fluid (arrow) in the anterior
1. The plane is through the deepest part of the acetabulum
which provides the greatest femoral head coverage.
2. The triradiate cartilage and ischium are visible.
3. The iliac bone appears as a straight line, parallel to the
transducer [109].
The hip should next be assessed in the transverse plane.
The hip is flexed to 90°, and the transducer is rotated 90° to
its position for the standard coronal plane, placing it nearly
parallel to the femur. The proximal femoral metaphysis is in
continuity with the cartilaginous femoral head, which articu-
lates with the ischium (Fig. 18.60).
Assessment for Synovitis and Effusion
Ultrasound assessment of the hip joint for synovitis and
effusion can be performed in patients of any age. A linear
transducer should be used if possible, with the highest fre-
quency that will allow adequate depth of penetration to the
level of the femoral neck. The transducer is placed parallel
to the long axis of the femoral neck. As the normal neck-
shaft angle is in the range of 145° in children, the transducer
will not be parallel to the femoral shaft, but must be rotated
so that the superior margin is more medial than the inferior
margin (Fig. 18.59c).
From superficial to deep, the ultrasound image will demon-
strate the subcutaneous tissues and the anterior musculature,
including the sartorius, rectus femoris, and iliopsoas muscles.
Deep to the iliopsoas muscle and superficial to the femoral neck
is the joint capsule. The capsule is composed of an anterior
and posterior layer, separated by the anterior recess of the joint
space. In the normal hip, the two layers are apposed or separated
by minimal fluid, with an average thickness of the combined
capsular layers measuring approximately 5 mm (Fig. 18.58).
D. Y. Jarrett
joint recess. The distance (asterisks) from the anterior joint capsule to the
femoral neck measured 11.5 mm. (b) Transverse grayscale ultrasound
image of the left hip shows the fluid (arrow) in the anterior joint recess
The hip joint capsule thickness has also been shown
to increase with age, from 3.7 mm in the first year of life
to 6.7 mm at 16 years of age and older [110, 111]. In the
absence of a pathologic effusion, there should not be more
than 2 mm of asymmetry in the anterior capsular thickness
between the hips [110]. If a joint effusion is ­present, it will
be seen as anechoic or hypoechoic fluid between the anterior
and posterior layers of the capsule. Rotating the transducer
90°, the anterior hip joint can also be evaluated in the trans-
verse plane (Fig. 18.61).
Congenital/Developmental Abnormalities
 evelopmental Dysplasia of the Hip
D
Developmental dysplasia of the hip (DDH) is an abnormal-
ity in the development of the femoro-acetabular joint which
leads to instability of the hip and/or varying degrees or sub-
luxation or dislocation of the femoral head.
The incidence of DDH varies in different populations,
from 1.5 to 20 per 1000 newborns [112]. The two biggest
risk factors are breech presentation at delivery and female
sex [113]. Additional risk factors for hip dysplasia include
positive family history, being the first-born child, torticol-
lis, and several neuromuscular disorders. Postnatally, pro-
longed swaddling with the hips extended and adducted may
also increase the risk of developing hip dysplasia [104, 114,
115]. Infants less than 3 months of age are routinely screened
by physical examination using the Barlow maneuver which
assesses for subluxability of the hip, and the Ortolani maneu-
ver which assesses for reducibility of a dislocated hip.
There is controversy as to whether imaging evaluation for
hip dysplasia should be limited to patients with risk factors
18 Musculoskeletal System
and/or abnormal examination findings, or if universal ultra-
sound screening should be performed in neonates. Ultrasound
detects more hip dysplasia than physical examination. There
is concern that without universal ultrasound screening, infants
with DDH may face the long-term consequences of a late or
missed diagnosis [116]. Conversely, there is concern that
ultrasound screening leads to false positive results, resulting
in overtreatment of infants who do not have clinically signifi-
cant dysplasia, with the potential iatrogenic consequences of
abduction splinting, including avascular necrosis (AVN) of
the femoral head and femoral nerve palsies [115].
Universal screening has not been shown to decrease the
rate of late diagnosis of DDH. In the United States, the
American Academy of Pediatrics, the American Academy
of Orthopaedic Surgeons, and the American College of
Radiology all recommend selective imaging of infants with
risk factors or physical examination findings that are positive
or equivocal for DDH [112].
Recognition and early treatment of DDH are important.
Late diagnosis after walking age is associated with a need for
surgery and worse outcomes. If left untreated or inadequately
treated, dysplastic hips can cause pain and early osteoarthri-
tis in adults, leading to up to one-third of hip replacements in
adults less than 60 years of age [112].
Imaging
The ultrasound technique to evaluate for neonatal hip dyspla-
sia combines the methods proposed by multiple authors, with
modifications made over the years. Graf first described the
a b
β
α
Fig. 18.62 Comparison of normal and abnormal hip α- and β-angles.
(a) Coronal grayscale ultrasound image of a normal hip in a 44-day-old
male shows an α-angle of 64° and a β-angle of 41°. The labrum (arrow-
head) is an echogenic triangle attached to the hypoechoic acetabular
873
use of ultrasound in DDH, with a focus on hip morphology.
The Graf method uses the standard coronal view of the hip to
measure the alpha angle, the angle between a line along the
lateral cortex of the ilium and a line tangential to the acetabu-
lar roof. Graf also defined a beta angle, the angle between a
line along the lateral cortex of the ilium and a line along the
cartilaginous acetabular labrum. The hip can then be catego-
rized into Graf types 1–4, depending on the alpha and beta
angles, morphology of the bony rim (which is the most lateral
part of the concavity of the acetabular socket), and patient age
(Fig. 18.62, Table 18.1) [104, 112, 117].
The method of Harke advocates for the use of dynamic stress
imaging of the hip to determine the stability of the joint. Morin
and Terjesen include measurements of femoral head coverage
by the iliac bone [104]. While there is still some variation, a
common imaging approach for hip dysplasia is as follows:
A standard coronal view of the hip is obtained, meeting
all of the imaging criteria described above. The alpha angle
of Graf is measured, with 60° or greater considered normal.
On the same standard coronal view, femoral head coverage is
also measured, by drawing a line along the lateral cortex of
the iliac bone which intersects the femoral head. The percent
of the total diameter of the femoral head below the line is
calculated (d/D ratio). Femoral head coverage greater than
50% is considered normal (Figs. 18.62 and 18.63).
Next, a transverse view of the hip is obtained to deter-
mine if the femoral head is appropriately aligned with the
ischium and whether the joint is stable or subluxatable.
With the hip flexed, the sonographer applies gentle pos-
β
α
cartilage. (b) Coronal grayscale ultrasound image of an abnormal hip in
a 62-day-old female shows an α-angle of 50° and a β-angle of 63°.
There is rounding of the bony acetabular rim and a prominent pulvinar
(arrow) in the medial joint space. Arrowhead, Labrum
874 D. Y. Jarrett

Table 18.1 Graf hip types terior pressure on the femur to assess whether the femo-
Cartilaginous ral head moves posterolaterally relative to the acetabulum
Alpha Superior roof and beta (Fig. 18.64). By moving the transducer posterolaterally rel-
Type Age angle bony rim angle
ative to its position on the standard transverse view, the hip
Type Ia Any age ≥ 60° Angular/ < 55°
slightly
can be adducted in a fashion similar to the Barlow maneu-
rounded ver and assessed for change in hip alignment. These stress
Type 1b Any age ≥ 60° Angular/ > 55° maneuvers are not performed in infants being treated for
slightly dysplasia or when the treating orthopedic surgeon other-
rounded
wise requests their omission [109, 118].
Type IIa + 0–12 weeks 50–59 Rounded Covers the
femoral head In the setting of prolonged lateral hip subluxation, the
Type IIa- > 6–12 weeks 50–59 Rounded Covers the pulvinar may hypertrophy, which can be seen on both coro-
femoral head nal and transverse images of the hip as increased echogenic
Type IIb > 12 weeks 50–59 Rounded Covers the tissue in the medial acetabular fossa. When enlarged, the
femoral head
pulvinar may block hip reduction and is sometimes resected
Type IIc Any age 43–49 Rounded to Covers the
flattened femoral head, during open reduction of the hip to improve joint congru-
β < 77 ency. However, the pulvinar has been shown to regress over
Type IId Any age 43–49 Rounded to Displaced; time with spica cast placement [119].
flattened β < 77 In assessing hip dysplasia, false positive results can
Type IIIa Any age < 43 Flattened Displaced occur if the newborn is less than 4 weeks of age, with cap-
upward with
normal sular laxity mimicking instability, and with mild imma-
structure turity of the acetabulum appearing similar to acetabular
Type IIIb Any age < 43 Flattened Displaced dysplasia [112]. To avoid false positive results, screening
upward with ultrasound should occur after 6 weeks of age, unless there
abnormal
structure
is an abnormal finding on physical examination. In addi-
Type IV Any age < 43 Flattened Displaced tion, premature infants have lower alpha and beta angles
downward compared to full-­term infants and should be referred to
From Graf R, Scott S. Hip sonography: diagnosis and management of Orthopedics for assessment and follow-up imaging if nec-
infant hip dysplasia. 2nd ed. Berlin. Springer; 2006 [117] essary to distinguish immaturity of the hips from dysplasia.

a b

Fig. 18.63 Comparison of normal and abnormal acetabular coverage 44-day-old male shows femoral coverage of 56%. (b) Coronal gray-
of the femoral head. Percent coverage is length of dotted line/length of scale ultrasound image of an abnormal hip in a 62-day-old female
solid line. (a) Coronal grayscale ultrasound image of a normal hip in a shows femoral head coverage of 40%
18 Musculoskeletal System
a L
F F
IS
Fig. 18.64 Unstable subluxating hip in a 13-day-old female born via
breech presentation. (a) Transverse grayscale ultrasound image of the
right hip without stress demonstrates the femoral head (F) appropriately
aligned with the ischium (IS). (b) Transverse grayscale ultrasound image
a b
IS
Fig. 18.65 Anterior approach hip ultrasound. (a) Anterior approach gray-
scale ultrasound image in a 58-day-old female wearing a Pavlik harness.
The transducer is oriented longitudinally to the femoral neck. The femoral
head (F) articulates with the pubis (P) anteriorly and the ischium (IS) pos-
Treatment
Initial treatment of DDH in neonates and infants is often
with a Pavlik harness that keeps the hips abducted and the
knees flexed to allow hip reduction while maintaining hip
motion. Ultrasound surveillance is performed while patients
are being treated, and the harness may be weaned following
6 weeks of adequate hip location.
Complications of treatment include AVN and femoral
nerve palsy. Given that hip instability often resolves spon-
taneously in the first few weeks of life, Pavlik harness treat-
ment may not be necessary before 6 weeks of age, and
treatment beyond 4 months has an increased risk of failure.
Other methods used to maintain hips in abduction include
875
b L
P P
IS
of the right hip with posteriorly directed force demonstrates posterolat-
eral subluxation of the femoral head (F) relative to the ischium (IS). L,
Lateral; P, posterior
teriorly. (b) Multi-echo data image combination (MEDIC) MR image of
the hip in a 17-month-old female in a spica cast is reformatted along the
long axis of the femoral neck and shown for anatomic correlation
the Frejka pillow [120]. If abduction splinting is ineffective,
infants may proceed to closed or open reduction of the hip
with spica cast placement.
Attempts to obtain the standard lateral approach views in
a patient wearing a Pavlik harness lead to neutral adduction
of the hip, and the presence of the cast precludes the use of
lateral approach sonography. Use of anterior approach sonog-
raphy has been advocated to assess the position of the femoral
head in patients undergoing treatment with a Pavlik harness
or spica cast. The hips remain abducted and flexed, the trans-
ducer is centered over the femoral head in the transverse
plane, and the relationship of the femoral head to the ischial
limb is assessed for appropriate alignment (Fig. 18.65) [121].
876
Older children and adults may require supra- or periace-
tabular osteotomies and/or femoral osteotomies for treat-
ment of hip dysplasia.
 roximal Focal Femoral Deficiency
P for ultrasound assessment of hip dysplasia. Ultrasound can-
Proximal focal femoral deficiency (PFFD) is an abnormal-
ity of the proximal femur affecting 1:52,000 live births and
is bilateral in 15% of cases. It is defined by proximal defi-
ciency of the femur with an abnormal hip joint, short femur
with leg length discrepancy, and subtrochanteric varus defor-
mity. PFFD can range in severity from mild shortening of the
femur to severe deficiency of the hip and femoral shaft [122].
Clinically, the affected thigh is short, with flexion, a­ bduction,
and external rotation at the hip. 45% of cases have associated
ipsilateral fibular hemimelia [123].
Imaging is important for the diagnosis and classification
of PFFD, with radiographs typically the most useful modal-
ity. There are multiple classification schemes for PFFD. The
most widely used is the Aitken system, with criteria includ-
ing the presence or absence of the femoral head and the
severity of acetabular dysplasia [123].
Ultrasound provides complementary information about
structures which are not radiographically apparent. Prior to
femoral head ossification, hip ultrasound in patients with
a b
Fig. 18.66 Proximal focal femoral deficiency in a 15-day-old female.
(a) Frontal radiograph of pelvis and thighs demonstrates a short, proxi-
mally deficient right femur which is laterally displaced. (b) Coronal
D. Y. Jarrett
PFFD allows confirmation that the femoral head is present,
and assessment for joint laxity, subluxation, or dislocation
(Fig. 18.66).
Rarely, an infant with undiagnosed PFFD may be referred
not reliably diagnose PFFD, and the imaging findings may
overlap with those of hip dysplasia. However, if key anatomic
landmarks are not identified on ultrasound, including the
lower edge of the ilium, the acetabular labrum, and the echo-
genic interface between the proximal femoral metaphysis
and the cartilaginous femoral head, an anatomic abnormality
beyond hip dysplasia should be suspected, and radiographs of
the hip should be obtained for further assessment [124].
Surgical management of PFFD depends on the stability of
the hip joint compared to the contralateral side and the pre-
dicted length of the affected limb at maturity. If the affected
limb will be less than 50% the length of the contralateral
limb, the patient should undergo knee fusion with a Syme
amputation or rotationplasty and will be fitted for a prosthe-
sis. Fusion of the proximal femur to the pelvis is performed
as indicated, for hip stabilization. If the predicted limb length
at maturity of the affected limb will be greater than 50% of
the length of the contralateral limb, the patient may undergo
limb-lengthening procedures [123].
grayscale ultrasound image of the right hip shows a small, cartilaginous
femoral head (H) and a prominent greater trochanter (arrow)
18 Musculoskeletal System
Infectious/Inflammatory Disorders
 ransient Synovitis and Septic Arthritis
T  lipped Capital Femoral Epiphysis
Transient synovitis is a self-limiting disorder of unknown
pathophysiology. Multiple etiologies have been proposed,
including preceding viral infection or trauma; however, none
has been substantiated. It is most commonly seen in children
3–8 years of age. Patients present with acute hip pain, limp,
non-weight bearing, decreased range of motion, and low-
grade fever [125–127].
US findings are of a hip joint effusion, typically anechoic,
with anterior bulging of the joint capsule [110].
Treatment is conservative with rest and administration
of NSAIDS. Symptoms typically resolve over 3–10 days.
Patients may have an increased likelihood of developing
recurrent transient synovitis, and some studies suggest an
increased incidence of Legg-Calve-Perthes disease, pos-
sibly related to pressure on the cervical arteries of the hip
by the effusion, thereby predisposing to ischemia [126,
127].
It is important to differentiate transient synovitis of the
hip from septic arthritis. Both present with acute hip pain
and joint effusion, but the treatments are very different,
and treatment delay of septic arthritis is associated with
poor outcomes [128]. The infected joint fluid is toxic to
cartilage and can lead to joint damage or destruction if
not promptly treated, ideally within 4 days of symptom
onset [125]. Joint aspiration and culture may be neces-
sary to distinguish the two entities. The Kocher criteria
define four predictors of septic arthritis to aid in clini-
cal assessment: history of fever, non-weight bearing,
erythrocyte sedimentation rate ≥ 40 mm/hr, and serum
white blood-cell count >12,000 cells/mm3. Patients with a
greater number of predictors have an increased likelihood
of septic arthritis [128].
a b
M M
Fig. 18.67 Slipped capital femoral epiphysis in a 9-year-old female.
(a) Longitudinal grayscale ultrasound image of the left hip shows pos-
terior displacement of the proximal femoral epiphysis (arrow) relative
to the metaphysis (M). There is a hip joint effusion (arrowhead). (b)
877
Trauma
S
Slipped capital (upper) femoral epiphysis (SCFE or SUFE)
is a disorder of the hip, with shear stress on the unfused prox-
imal femoral physis causing misalignment of the epiphysis
and metaphysis. The femoral head appears posteriorly and
medially displaced relative to the normal position on the
femoral neck, although, in fact, the femoral head remains
normally aligned with the acetabulum, and it is the femoral
neck and shaft that are displaced.
The incidence of SCFE ranges from 1 to 7 per 100,000, typ-
ically seen in boys 12–15 years of age and girls 11–13 years
of age. There is an association with obesity, endocrinopa-
thies (most commonly hypothyroidism), and an increased
incidence in Black males compared to other groups [123].
The primary imaging modality for SCFE is radiography,
with frontal and frog-lateral views of the pelvis allowing com-
parison of proximal femoral epiphyseal-metaphyseal align-
ment between the symptomatic and asymptomatic sides, and
permitting assessment for potential bilateral involvement. A
child with SCFE may present with hip pain and be referred for
ultrasound assessment for infectious or inflammatory condi-
tions, without radiography or with inadequate radiographs. It
is therefore important to recognize the ultrasound findings of
SCFE.
On anterior approach hip ultrasound, with the trans-
ducer oriented parallel to the long axis of the femoral
neck, the femoral head will appear posteriorly displaced
relative the proximal femoral metaphysis, with a step-off
at the physeal margin. A hip joint effusion may be present
(Fig. 18.67) [129].
Treatment of SCFE depends on the severity and stabil-
ity of the slip, stability being defined as the ability to bear
weight on the affected leg. Stable slips typically undergo in
c
Longi­tudinal grayscale ultrasound image of the normal right hip for
comparison shows appropriate alignment of the epiphysis (arrow) rela-
tive to the metaphysis (M). (c) Frog-lateral radiograph of the pelvis
confirms a left-sided slip (arrowhead)
878
situ screw fixation of the femoral head and neck. Unstable
slips, particularly if severe, may be reduced before fixa-
tion. Proximal femoral osteotomies may be necessary to
improve femoral head alignment and proximal femoral
morphology [130].
Knee
Normal Anatomy and Imaging Approaches
Normal Anatomy
The knee joint has a femorotibial and a patellofemoral artic-
ulation (Fig. 18.68).
a b
Fig. 18.68 Normal knee anatomy in a 7-year-old male. (a) Frontal radio-
graph of the left knee, (b) coronal MEDIC MR image and (c) sagittal
a b
F T
Fig. 18.69 Normal knee anatomy in an infant. (a) Longitudinal medial
approach grayscale ultrasound image of the knee in a 28-day-­ old
female. The cartilaginous epiphyses of the distal femur (F) and proxi-
mal tibia (T) have small ossification centers (arrows). (b) Longitudinal
anterior approach grayscale ultrasound image of the knee in the same
D. Y. Jarrett
The femorotibial joint is a hinged synovial joint of the
distal femur and proximal tibia. The distal femoral and
proximal tibial epiphyseal ossification centers are typically
present in full-term infants, with the distal femoral epiphy-
sis ossification center appearing at 36–40 weeks’ gestational
age and the proximal tibial ossification center appearing
between 36 weeks prenatally and 2 months of age (Fig.
18.69) [122].
The patellofemoral joint is a synovial joint, with an artic-
ulation between the patella and the articular surface at the
anterior aspect of the distal femur, the trochlea. The patella
is cartilaginous at birth and does not begin to ossify until
3–5 years of age (Fig. 18.69) [131].
c
MEDIC MR image show the incompletely ossified epiphyses of the femur
(F), tibia (T), and fibula (Fi), as well as the incompletely ossified patella (P)
F
T
patient at 8 months of age. There has been progressive ossification of
the distal femoral and proximal tibial epiphyses (arrows). The patella
(asterisk) is still cartilaginous and does not obscure visualization of the
knee from an anterior approach
18 Musculoskeletal System
Patient Positioning
Generally, evaluation of the anterior structures of the knee
can be performed with the patient lying supine and knees
extended, and posterior structures evaluated with the patient
lying prone and knees extended.
The extensor mechanism of the knee and the anterior joint
recess are best evaluated with the patient lying supine and
with the knee in mild flexion. This can be accomplished by
placing a rolled towel beneath the knee (Fig. 18.70).
Prior to patellar ossification, the patellofemoral joint can
be assessed with the patient supine and knee extended. It can
also be evaluated with the patient sitting upright and the lower
legs hanging over the edge of the examination table, permit-
ting varying degrees of flexion to show patellar position rela-
tive to the trochlea in a range of positions.
Imaging Approaches
Assessment of the femorotibial joint begins with longitudi-
nally oriented ultrasound images of the distal femur and prox-
imal tibia, demonstrating the epiphysis of each bone separated
from the metaphysis by the linear hypoechoic physis and the
echogenic ossification center surrounded by unossified car-
tilage in skeletally immature patients. The alignment of the
epiphysis to the metaphysis can be ­evaluated circumferen-
tially for smooth continuity and lack of physeal disruption.
Alignment of the femur and tibia is most easily seen on
coronal views at the medial and lateral aspects of the knee
joint with the knee extended, demonstrating the femoral con-
dyle articulating with the tibial plateau. Depending on the
size of the patient, these articulations can also be demon-
strated from an anterior or posterior approach, although with
a b
c d
Fig. 18.70 Extensor mechanism of the knee. (a) Transducer place-
ment along the long axis of the quadriceps tendon, with the knee in
flexion. (b) Longitudinal grayscale ultrasound image of the quadriceps
tendon (arrow) inserting on the patella (asterisk). (c) Transducer place-
879
increasing age, patellar ossification may obscure visualiza-
tion anteriorly (Fig. 18.69).
Patellofemoral Joint
Before patellar ossification, the normal patellar-trochlear joint
can be easily visualized in neonates and infants by placing
the transducer at the anterior aspect of the knee, transversely
or longitudinally, producing axial and sagittal views of the
joint, respectively (Figs. 18.69 and 18.71). In older pediat-
ric patients, as in adults, when posterior acoustic shadowing
from the ossified patella obscures anterior visualization of the
joint, the trochlear cartilage can be assessed by positioning the
knee in maximal flexion, which displaces the patella inferiorly
relative to the trochlea, and then placing the transducer trans-
versely along the superior margin of the patella.
Extensor Mechanism
The superior component of the extensor mechanism is the
quadriceps tendon, made up of contributions from the rec-
tus femoris, vastus intermedius, vastus lateralis, and vastus
medialis muscles, that inserts on the superior aspect of the
patella. Inferiorly, the patellar tendon extends from the infe-
rior aspect of the patella to the tibial tubercle. Full assessment
of the tendons includes following their course from origin to
insertion on sagittal and transverse images (Fig. 18.70). The
quadriceps tendon can be traced superiorly to the myoten-
dinous junctions of the quadriceps muscles. Both tendons
should be evaluated for normal echogenicity and thickness to
exclude tendinopathy or tear. As with all tendons, the exten-
sor mechanism demonstrates anisotropy which can mimic
tendinopathy.
ment along the long axis of the patellar tendon, with the knee in flexion.
(d) Longitudinal grayscale ultrasound image of the patellar tendon
(arrow) extending from the inferior pole of the patella (asterisk) to the
partially ossified tibial tubercle (arrowhead)
880 D. Y. Jarrett

Fig. 18.71 Normal patellofemoral joint in an 8-month-old male. Anterior

approach transverse grayscale ultrasound image. The patella is cartilagi-
nous (white arrow). There is a central ossification center (black arrow) c
within the femoral epiphysis

Tibial Tubercle
The tibial tubercle deserves special attention in pediatric
musculoskeletal imaging, because it is a common source
of pain and injury in the growing child. Ehrenborg and
Lagergren have divided the development of the tibial tubercle
into four stages: (1) the cartilaginous stage (aged 0–11 years)
Fig. 18.72 Normal development of the tibial tubercle. (a) Cartilaginous
when the tibial tubercle is completely cartilaginous; (2) the stage. Longitudinal grayscale ultrasound image of the tibial tubercle in an
apophyseal stage (11–14 years) when tibial tubercle ossifi- 8-year-old female. The tubercle (arrow) is entirely cartilaginous and appears
cation begins; (3) the epiphyseal stage (14–18 years) when homogeneously hypoechoic. (b) Apophyseal stage. Longitudinal grayscale
the tibial tubercle apophysis fuses with the proximal tibial ultrasound image of the tibial tubercle in an 11-year-old male. An echo-
genic ossification center (arrowhead) is present within the tubercle. (c)
epiphysis; and (4) the bone stage (over 18 years) when the Bone stage. Longitudinal grayscale ultrasound image of the tibial tubercle
physis is completely fused [132]. in a 15-year-old male. The tubercle (asterisk) is completely ossified and
The stages of tibial tubercle development are impor- fused to the tibia
tant since they determine the pattern of injury when stress
is applied by the patellar tendon. In the pre-ossification
and ossification stages, the cartilage cells of the tubercle
undergo cellular changes making them weaker and more
likely to fail when subjected to tensile stress than the phy-
sis [131].
During the cartilaginous stage, the tibial tubercle has the
same hypoechoic appearance as the remainder of the unossified
cartilage. Progressive ossification is seen with an echogenic
shadowing focus of bone developing within the cartilaginous
tubercle, culminating in complete ossification and physeal Fig. 18.73 Medial meniscus. Medial approach longitudinal grayscale
fusion with loss of the hypoechoic physeal margin between the ultrasound image of the femorotibial joint demonstrates the echogenic
tubercle and the remainder of the tibial epiphysis (Fig. 18.72). medial meniscus (arrow). The radial margin of the meniscus cannot be
seen

Menisci
The medial and lateral menisci are C-shaped fibrocartilagi-
nous structures located between the femorotibial articulations.
Ultrasound evaluation of the menisci is limited as the entire
meniscus cannot be assessed, particularly the inner mar-
gins. However, visualization in children may be better than
in adults, especially when there is incomplete ossification of
the femur and tibia. The menisci are best evaluated on coronal
images at the medial and lateral aspects of the knee where they
are normally demonstrated as hyperechoic, triangular-­shaped
structures with their bases oriented peripherally (Fig. 18.73).
18 Musculoskeletal System
Joint Effusion and Baker Cyst
Joint Effusion
Joint fluid pools in the suprapatellar recess which is bounded
anteriorly by the suprapatellar fat pad and quadriceps ten-
don and posteriorly by the pre-femoral fat pad. This region
is assessed in long and short axes by placing the ultrasound
transducer superior to the patella (Fig. 18.74). Knee joint
effusions can also be identified in the medial and lateral
recesses which are best evaluated in the transverse plane,
with the knee extended, medial and lateral to the patella, and
P asymptomatic [135, 136].
FC
Fig. 18.74 Knee joint effusion. Anterior approach longitudinal gray-
scale ultrasound image of the knee demonstrates a joint effusion, with
hypoechoic fluid (arrow) in the suprapatellar recess. Arrowhead,
Quardiceps tendon; P, patella; FC, femoral condyle
a b
Fig. 18.75 Evaluation for Baker cyst. (a) Transducer is oriented trans-
versely over the posterior aspect of the knee. (b) 4-year-old male with a
Baker cyst. Posterior approach transverse grayscale ultrasound image of
881
overlying the retinacula. The distribution of knee joint fluid
is affected by patient positioning, with optimal evaluation
of the anterior recesses and joint space with 30° of flexion
[133]. Applying pressure on the recess with the transducer
can cause displacement of the joint fluid and lead to under-
reporting of effusion size [134].
Baker Cyst
A Baker cyst, or popliteal cyst, is a fluid-filled bursa in con-
tinuity with the knee joint that extends into the posterome-
dial soft tissues of the knee between the medial head of the
gastrocnemius muscle and the semimembranosus tendon. It
is fairly common in children and, unlike adults, is not associ-
ated with arthritis or other intra-articular pathology. Patients
typically present with a nontender mass and are most often
Evaluation for Baker cyst is performed from a posterior
approach, with the patient lying prone. In the transverse plane,
the medial head of the gastrocnemius muscle should be identi-
fied and traced proximally until the tendon of the semimem-
branosus muscle is noted along its medial aspect. A Baker cyst
will appear as a collection of fluid in the posterior knee, with a
neck extending between these two structures (Fig. 18.75). The
fluid may be anechoic or complex, depending on the contents
of the cyst [134]. Color Doppler imaging should be performed
to confirm the cystic nature of the lesion.
Pediatric popliteal cysts typically decrease in size with
eventual complete resolution without treatment [137].
the left knee demonstrates an anechoic fluid collection (cursors), with
neck extending between the medial head of the gastrocnemius muscle (G)
and the tendon (T) of the semimembranosus muscle
882
Congenital/Developmental Abnormalities
Tibial Hemimelia
Tibial hemimelia is rare, occurring in 1/1,000,000 live births
[138]. It is characterized by dysplasia of the tibia, ranging
from hypoplasia to complete absence. It can also be associ-
ated with other anomalies of the lower extremity, including
hip dysplasia, short femur, and anomalous rays of the foot
[139]. Approximately 70% of cases are unilateral, and it may
be inherited or sporadic [138].
There are multiple classification systems for tibial hemime-
lia which are used to guide management. Older classification
schemes are based on the radiographic appearance of the lower
leg at birth. Weber and Paley have developed newer systems
based on the addition of ultrasound and MR assessment [138].
If no tibia is radiographically visible, ultrasound can be
used to assess for the presence and size of a cartilaginous
tibial anlage, an unossified patella, and the quadriceps tendon
(Fig. 18.76). The results determine surgical management, as
the absence of these structures increases surgical complexity,
with poorer outcomes, and the child may be treated with knee
disarticulation rather than reconstruction.
While fibular hemimelia is more common than tibial
hemimelia, with reports of 7.4–20/1,000,000 live births,
ultrasound has not been shown to have a meaningful role
in the new classification systems which determine manage-
ment [122, 140–142].
a b
Fig. 18.76 Tibial hemimelia in a 21-day-old female. (a) Lateral radio-
graph of the right leg demonstrates absence of the tibia and a posteriorly
deviated foot. (b) Longitudinal grayscale ultrasound image of the distal
D. Y. Jarrett
 ongenital Knee Dislocation
C
Congenital knee dislocation (CKD), also known as “genu
recurvatum,” is a rare anomaly, occurring in 1–2/100,000
live births and characterized by hyperextension of the knee.
The cause is unknown, with possible etiologies including
postural deformity from in utero crowding, genetic abnor-
mality, or intrinsic abnormality of the developing tissues
[143, 144]. It may be isolated, associated with arthrogrypo-
sis multiplex congenita, Larsen syndrome, congenital hip
dislocation, or abnormalities in foot alignment [145]. CKD
is associated with shortening and fibrosis of the quadriceps
tendon, a small suprapatellar bursa, and tightening of the
anterior knee joint capsule [143, 145].
There is a commonly used classification system for CKD:
grade 1, hyperextension (genu recurvatum); grade 2, anterior
tibial subluxation which is reducible; and grade 3, anterior
tibial dislocation (Fig. 18.77).
Given the small size of the distal femoral and proximal
tibial epiphyseal ossification centers in neonates, align-
ment of the knee is best assessed by ultrasound, which
allows for direct visualization of the cartilaginous femoral
condyles and tibial plateau. Anterior approach ultrasound,
with the transducer oriented longitudinally, will allow for
assessment of anterior tibial subluxation (Fig. 18.78). In
cases of tibial dislocation, a posterior approach may be
more useful.
right femur confirms the presence of a quadriceps tendon (white arrow)
with no identifiable patella
18 Musculoskeletal System
Fig. 18.77 Diagram of congenital knee dislocation classification system
Fig. 18.78 Congenital knee dislocation in a neonate. (a) Lateral radio-
graph of the left knee obtained on day 1 of life demonstrates anterior
dislocation of the tibia with a recurvatum deformity of the knee. (b)
In addition to diagnosis and categorization, ultrasound is
useful to monitor alignment of the knee during treatment.
Treatment is typically conservative, including flexion exer-
cises to stretch the quadriceps tendon and anterior capsule,
splinting, or serial casting. If these measures fail to achieve
knee reduction, surgical reduction is required with lengthen-
ing of the quadriceps tendon [143, 145].
883
Anterior approach longitudinal grayscale ultrasound image at 42 days
of age shows persistent anterior subluxation of the tibia (T) relative to
the femur (f). Asterisk, Patella
 ongenital Patellar Dislocation
C
Congenital patellar dislocation (CPD) is a rare disorder, with
permanent and irreducible lateral dislocation of the patella,
unlike patellar subluxation or instability. It is present at birth;
patients have knee flexion contracture, genu valgum, and
external tibial torsion [146, 147].
884
The diagnosis of CPD may be missed by radiography,
as the patella does not ossify until approximately 3 years
of age. Ultrasound, however, will demonstrate the laterally
dislocated cartilaginous patella [148]. Imaging of the flexed
knee joint from an anterior approach will show the trochlea
with absence of the patella. Moving the transducer laterally,
the patella will be seen as a hypoechoic triangular structure
adjacent to the lateral femoral condyle (Fig. 18.79). The dis-
located patella may be palpable, which aids in its detection
by ultrasound.
While serial casting and bracing may reduce the flexion
contracture, surgery is required for definitive treatment, pref-
erably performed before 1 year of age. There are several sur-
gical approaches. As with other lateral patellar dislocations,
treatment may include lateral release and medial plication.
There can be surgical correction of the extensor mechanism,
including medialization of the patellar tendon, and medial-
ization and lengthening of the short and laterally displaced
quadriceps tendon [146, 149].
a b
Fig. 18.79 Congenital patellar dislocation in a 75-day-old female. (a)
Anterior approach longitudinal grayscale ultrasound image of the knee
reveals absence of the patella (arrowhead) from its expected location
anterior to the trochlea. (b) Transverse grayscale ultrasound image at
D. Y. Jarrett
Bipartite/Multipartite Patella
Patellar ossification begins at 3–5 years of age, with cen-
trally located, multifocal ossification centers which usu-
ally fuse rapidly, forming one central ossification center.
Sometimes there are distinct superior and inferior ossifica-
tion centers. A separate accessory ossification center can
develop, typically at the superolateral aspect of the patella,
referred to as a bipartite patella [131]. Although it is most
often an incidental finding, a bipartite patella is occasion-
ally associated with anterior knee pain in children [150].
Radiographically, it can be mistaken for a fracture in the
setting of acute injury.
Ultrasound can be used to differentiate the accessory ossi-
fication center from a fracture fragment by demonstrating an
intact layer of articular cartilage overlying each ossification
center as well as the fibrous tissue or fibrocartilage located
between each ossification center (Fig. 18.80). Ultrasound
has been shown to be as accurate as radiographs in the diag-
nosis of bipartite patella [150].
the lateral aspect of the knee demonstrates a small, laterally dislocated
patella (arrow). (c) Axial proton density, fat-suppressed MR image of
the knee obtained at 17 months of age depicts the small, laterally dislo-
cated patella (arrow)
18 Musculoskeletal System
Bipartite patella can be symptomatic, with patients
presenting with pain following trauma or strenuous activ-
ity. Treatment is initially conservative, including activity
modification, physical therapy, and symptomatic relief
with NSAIDS and steroids. Those who fail conservative
management may require surgery, with excision of an
accessory fragment, release of the lateral retinaculum or
vastus lateralis from the accessory fragment, or internal
fixation of the accessory fragment to the remainder of the
patella [151].
a b
Fig. 18.80 Multipartite patellar ossification centers in a 5-year-old male
with knee pain and concern for patellar fracture. (a) Longitudinal gray-
scale ultrasound image of the right patella demonstrates intact hypoechoic
a b
LFC T
Fig. 18.81 Discoid meniscus in a 7-year-old female. (a) Lateral approach
longitudinal grayscale ultrasound image of the left knee shows an
increased width of the lateral joint space with a thickened lateral menis-
885
 iscoid Meniscus and Meniscal Tears
D
Discoid meniscus refers to a spectrum of abnormal meniscal
morphology, occurring most often in the lateral meniscus.
The normal lateral meniscus is typically 4–5 mm thick and
10–12 mm wide, and covers 70% of the lateral tibial plateau.
The Watanabe classification system defines three types of
discoid menisci. Type 1 is block shaped, increased in thick-
ness, and covers the entire tibial plateau (Fig. 18.81). Type
2 is a partial discoid meniscus, covering 80% or less of the
patellar cartilage (arrow) overlying separate echogenic ossification centers
(arrowheads). (b) Sagittal CT image of the right knee shown for anatomic
correlation demonstrates two separate ossification centers (arrowheads)
cus (arrow) between the lateral femoral condyle (LFC) and tibia (T). (b)
Coronal proton density, fat-suppressed MR image of the knee confirms
a discoid meniscus (arrow)
886
a b
Fig. 18.82 Horizontal tear of the medial meniscus and parameniscal
cyst in a 16-year-old female. (a) Medial approach longitudinal gray-
scale ultrasound image of the left knee shows an irregular, hypoechoic
zone extending through the medial meniscus (white arrow) with an
tibial plateau. Type 3 or Wrisberg variant has thickening of
the posterior horn and is hypermobile, lacking the normal
posterior meniscal attachments [152].
Discoid meniscus may present with knee pain, locking,
popping, or snapping if unstable and is more likely to tear
than a morphologically normal meniscus, accounting for
most traumatic meniscal tears in children less than 10 years
of age [153]. In pediatric patients, acute traumatic meniscal
tears in otherwise normal menisci are typically peripheral
longitudinal tears. However, in type 1 or 2 discoid menisci,
degenerative horizontal cleavage tears are seen.
Meniscal tears are identified by ultrasound as a
hypoechoic line extending to the surface of the hyperechoic
meniscal tissue or irregularity in the margins of the meniscal
tissue. A secondary sign of a meniscal tear is the presence of
adjacent focal hypoechoic fluid indicative of a paramenis-
cal cyst, which has a high association with meniscal tears
(Fig. 18.82).
In the adult literature, there is wide variation in the
reported sensitivity and specificity of ultrasound for detec-
tion of meniscal tears [154, 155]. However, ultrasound has
been shown to accurately detect parameniscal cysts associ-
ated with meniscal tears [134, 156].
Symptomatic discoid menisci are treated with saucerization.
Torn menisci are treated with partial meniscectomy or repair.
Trauma
In the growing skeleton, the proximal and distal attachment
sites of the patellar tendon are unossified cartilage, at the
patella and tibial tubercle, respectively. Activity can lead
to chronic stress with traction injury at the patellar tendon
insertion.
D. Y. Jarrett
adjacent parameniscal cyst (black arrow). (b) Coronal proton density,
fat-suppressed MR image of the left knee in the same patient demon-
strates the meniscal tear (white arrow) and parameniscal cyst (black
arrow)
Osgood-Schlatter Disease
Osgood-Schlatter disease (OSD) is the name given to the
response to chronic traction on the tibial tubercle. It occurs
during the apophyseal and epiphyseal stages of tibial tubercle
development, with tensile stress of the patellar tendon caus-
ing avulsion of the developing ossification center and over-
lying unossified cartilage of the tibial tubercle. This results
in fragmentation of the developing epiphysis [157]. OSD is
typically seen at 12–15 years of age in boys and 8–12 years
of age in girls, with patients presenting with slow onset, pro-
gressive pain and swelling at the tibial tuberosity [132].
Ultrasound can demonstrate the bony changes of
OSD. The ossification center of the tibial tubercle will be
irregular in contour with echogenic, shadowing osseous frag-
ments adjacent to the tubercle. In the acute phase, ultrasound
will also demonstrate the soft tissue changes of OSD. The
distal patellar tendon may be thickened and echogenic, as
seen with tendinopathy. There will be overlying soft tissue
swelling, and fluid may be identified in the deep infrapatellar
bursa (Fig. 18.13) [158].
OSD is typically self-limiting and is managed conserva-
tively, with symptoms potentially waxing and waning for
1–2 years. There may be a residual mass at the tibial tubercle,
related to unfused ossicle(s). For the 5–10% of patients who
have persistent symptoms, surgery may be performed after
skeletal maturity, including excision of ununited ossicles at
the tibial tubercle [132].
Sinding-Larsen-Johansson Syndrome
Sinding-Larsen-Johansson (SLJ) syndrome is the name given
to a traction response at the ossifying inferior margin of the
patella, caused by chronic stress from the patellar tendon inser-
tion. The pathogenesis of SLJ is similar to OSD, since the patel-
lar tendon insertion site is fibrocartilaginous until the patella is
18 Musculoskeletal System 887

F MM

TS
Fig. 18.83 Jumper’s knee in an 18-year-old male with knee pain. Lon­
gitudinal color Doppler ultrasound image of the left proximal patellar
tendon (arrow) demonstrates thickening, decreased echogenicity, and
hyperemia along its deep aspect and in the adjacent soft tissues (asterisk).
There is fragmentation of the inferior pole of the patella (arrowhead)

fully ossified. SLJ is seen most often in males 10–14 years of

age and presents with pain and swelling at the lower pole of the
patella [159, 160]. It can be thought of as the pediatric manifes-
tation of patellar tendinopathy or “jumper’s knee.”
On ultrasound, there is fragmentation at the inferior
pole of the patella and adjacent cartilaginous edema [159, b
161]. The inserting patellar tendon may be thickened
and echogenic, and can eventually develop calcification
(Fig. 18.83).
Treatment is typically conservative, with full recovery T
often occurring in 12–24 months [160].

TS
Ankle and Hindfoot
C
Normal Anatomy and Imaging Approaches

Normal Anatomy
The ankle joint, or talocrural joint, is a hinge joint composed of
articulations between the tibia, fibula, and talus. The distal tib-
ial epiphysis is cartilaginous at birth and begins ossifying in the
first year of life, centrally to peripherally. The medial malleolus
may have a separate ossification center in its central aspect,
with complete ossification of the malleolus by 8–10 years of
age. The distal fibular epiphysis is also cartilaginous at birth,
with ossification beginning by 3 years of age [162]. A talar
ossification center is present at birth (Fig. 18.84a) [163].
The hindfoot consists of the ankle joint and the calcaneus.
The calcaneal ossification center is present at birth. There is
a secondary ossification center in the posterior aspect of the
calcaneus, an apophysis which is cartilaginous at birth and
is the insertion site of the Achilles tendon (Fig. 18.84b). The
early secondary ossification center appears by 7 years of age,
within the plantar third of the apophysis. Ossification then
progresses proximally and distally, with the posterosuperior
and most plantar portions of the apophysis ossifying last,
Fig. 18.84 Normal ankle anatomy in a 3-year-old male. (a) Coronal
proton density MR image of the right ankle shows the ossification cen-
ters of the distal tibial (T) and fibular (F) epiphyses, and of the talus
(TS). The medial malleolus (MM) is entirely cartilaginous. (b) Sagittal
proton density MR image of the ankle shows the cartilaginous apophy-
sis (arrow) of the calcaneus and the insertion site (arrowhead) of the
Achilles tendon
sometimes from separate ossification centers. By 15 years of
age, the apophysis is completely fused in most children [164].
Patient Positioning
Assessment of the Achilles tendon and calcaneal apophy-
sis is performed from a posterior approach, with the patient
lying prone with the foot and ankle dangling over the end of
888 D. Y. Jarrett

a b

Fig. 18.85 Imaging of the Achilles tendon and calcaneal apophysis in the Achilles tendon (arrow) inserting on the incompletely ossified calca-
an 11-year-old male. (a) The patient is prone with feet off the edge of the neal apophysis. Hypoechoic cartilage (arrowhead) is identified between
table. The transducer is oriented along the long axis of the Achilles ten- two calcaneal ossification centers
don. (b) Posterior approach longitudinal grayscale ultrasound image of

a b

T Ta

Fig. 18.86 Tibiotalar joint assessment. (a) The ankle is plantar flexed the tibiotalar joint. (b) Longitudinal grayscale ultrasound image of the
and the transducer is longitudinally oriented over the anterior aspect of normal anterior tibiotalar joint. T, Distal tibial epiphysis; Ta, talus

the examination table, allowing for plantar and dorsiflexion

of the ankle as needed (Fig. 18.85).
The foot and ankle can otherwise be assessed with the
patient lying supine. Plantar flexion of the ankle is required
to adequately view the tibiotalar articulation (Fig. 18.86).

Imaging Approaches
Joint Effusion
Ultrasound assessment of the pediatric ankle can be per-
formed to assess the tibiotalar joint for effusion or syno-
vitis. This is best performed from an anterior approach,
with a midline longitudinal orientation of the ultrasound
transducer. The ankle should be plantar flexed enough to
allow contact of the probe with the skin; however, extreme
plantar flexion should be avoided, as this will displace
fluid from the anterior joint recess. The bony landmarks
are the distal epiphysis and the curved contour of the talar
dome to neck (Fig. 18.86).
There is an echogenic, intracapsular triangle of fat at
the anterior ankle, with the base of the triangle attached
T
Ta
Fig. 18.87 Ankle joint effusion in a 4-year-old male with left ankle
swelling and pain. Longitudinal grayscale ultrasound image of the ante-
rior tibiotalar joint shows anechoic fluid (asterisks) in the joint space
anterior to the talus (Ta) and anteriorly displacing the intracapsular fat
(arrowhead). T, Distal tibial epiphysis
to the joint capsule and the apex extending into the ante-
rior tibiotalar joint. If an effusion is present, hypoechoic
fluid will be seen elevating or displacing the fat pad from
the joint (Fig. 18.87) [165]. With the ankle plantar flexed,
the inferior margin of the joint recess extends to the talar
18 Musculoskeletal System
neck. The tibiotalar joint can also be visualized from a pos-
terior midline approach with the foot in dorsiflexion and
the probe longitudinally oriented. This technique is more
challenging than the anterior approach and may be more
useful in younger patients with decreased penetration depth
required.
Calcaneal Apophysis
The calcaneal apophysis is assessed by placing the trans-
ducer longitudinally over the posterior calcaneus. The distal
Achilles tendon can be seen inserting on the apophysis. In
younger patients, it will be hypoechoic cartilage, and with
development, one or more progressively larger, echogenic
ossification centers will be seen (Fig. 18.85).
Trauma
Apophyseal Avulsion Injuries
Prior to physeal fusion, apophyses are susceptible to avulsion
fractures, when the open physis is the weakest link in the mus-
cle-tendon-bone unit. In the feet, the apophyses are located
at the posterior calcaneus and the base of the fifth metatarsal.
Calcaneal apophyseal avulsion is a rare injury, which
is the result of traction on the proximal portion of unfused
apophysis by the Achilles tendon, a mechanism which could
cause a tendon tear in an adult. Ultrasound will demonstrate
the Achilles tendon inserting on a proximally displaced frag-
ment of the calcaneal apophysis (Fig. 18.88).
a b
Fig. 18.88 Avulsion of the calcaneal apophysis in an 11-year-old
female. (a) Longitudinal grayscale ultrasound image of the distal right
Achilles tendon shows thickened, retracted tendon fibers (asterisk)
inserting on a proximally displaced fragment of the superior calcaneal
889
Vascular Anomalies
Vascular anomalies are a diverse group of entities with
varying presentation and treatment. Accurate character-
ization and categorization are very important in order to
determine prognosis and guide treatment, if necessary.
Adherence to a standard classification system avoids
vague or improper terminology. The International Society
for the Study of Vascular Anomalies (ISSVA) has adopted
revised versions of the classification system first proposed
by Mulliken and Glowacki in 1982 and most recently
updated in 2018. This system divides vascular anomalies
into two groups, vascular tumors and vascular malforma-
tions (Table 18.2) [166].
Vascular Tumors
Vascular tumors consist of proliferating cells with increased
mitotic activity. The most common examples are hemangi-
oma and kaposiform hemangioendothelioma (KHE). These
masses have different clinical presentations and appearance
on physical exam which, when combined with the imaging
findings, are important for diagnosis.
Infantile Hemangioma
The most common type of hemangioma is the infantile heman-
gioma (IH). IH is also the most common tumor of infancy. There
is a female predominance, and risk factors include prematurity
and fair skin. IHs are frequently found in the head/neck region,
apophysis (arrow). (b) Longitudinal grayscale ultrasound image shows
the normal distal left Achilles tendon inserting on the calcaneal apophysis.
The apophysis is incompletely fused, with hypoechoic cartilage (arrow-
head) identified between two separate ossification centers
890
followed by the trunk, and less frequently the extremities [167].
They may be single or multiple, focal or diffuse. Unlike congen-
ital hemangioma and KHE, IHs express the glucose transporter
1 (GLUT 1) protein, allowing these tumors to be distinguished
pathologically [168].
At birth, skin findings may be absent or subtle, typi-
cally appearing 2 weeks to 2 months after birth. IHs then
undergo a characteristic pattern of growth and involution,
peaking in size at 3–8 months of age, plateauing in size at
10–12 months of age, followed by a 1–10-year period of invo-
lution. Superficial IH appears as a red, raised skin lesion,
while deep IH will have less conspicuous findings, such
as a bluish hue to the overlying skin, or no skin findings
[167–169].
When IH has a typical appearance on clinical examina-
tion, imaging may not be necessary. However, ultrasound
is usually the initial imaging study that is performed if
there are any questions or concerns about the diagnosis.
The ultrasound appearance of IH changes over time. In the
early phases, it is a well-circumscribed, echogenic mass
with increased vascularity on color Doppler imaging. Both
arterial and venous waveforms may be present on spectral
imaging, and sometimes the high-flow vessels in IH can be
Table 18.2 Classification of vascular anomalies
Vascular tumors Vascular malformations
Benign Simplea
Infantile hemangioma Capillary malformation
Congenital hemangioma Lymphatic malformation
Tufted angioma Venous malformation
Pyogenic granuloma Arteriovenous malformation
Locally aggressive Arteriovenous fistula
Kaposiform hemangioendothelioma
Malignant
Angiosarcoma
Epithelioid hemangioendothelioma
Combinations of malformations can occur
a As they spontaneously involute, RICH is typically treated
From ISSVA Classification for vascular anomalies© 2018 International
Society for the Study of Vascular Anomalies
a b
Fig. 18.89 Infantile hemangioma in a 58-day-old male with a mass of
the proximal right forearm. (a) Longitudinal grayscale ultrasound image
reveals an echogenic mass (arrow) in the subcutaneous tissues of the
D. Y. Jarrett
seen as anechoic channels. An involuting IH will be lower
in echogenicity, similar to the surrounding soft tissues, and
no flow will be seen on color Doppler imaging (Fig. 18.89).
The natural history of IH is spontaneous involution, so
treatment is rarely required, although it may be indicated if
the location of the growing hemangioma poses a threat, such
as potential airway compromise, or if there is skin ulceration
or thrombocytopenia. Initial treatment is oral propranolol
or steroids. Rarely, intralesional injection, embolization, or
resection is performed. Ulceration can be treated with laser
therapy [167, 168].
Congenital Hemangioma
Congenital hemangiomas are present at birth, usually solitary,
and found in the head and neck region. Most rapidly decrease
in size after birth, with resolution by 14 months of age, and fall
into the category of rapidly involuting congenital hemangioma
(RICH). Less often, a congenital hemangioma does not spon-
taneously resolve and continues to grow proportionally with
the child, maintaining the same relative size, and is referred to
as a non-involuting congenital hemangioma (NICH).
NICH usually appears as a blue or purple skin lesion with
telangiectasia and a peripheral rim of pale skin. Partially
involuting congenital hemangioma (PICH) is a congenital
hemangioma with a distinctive clinical behavior, evolving
from RICH to a persistent NICH-like lesion.
Determination of the timing of lesional appearance and the
presence or absence of GLUT-1 positivity may occasionally be
needed to clinically distinguish congenital and infantile hem-
angioma. The ultrasound appearance of RICH and NICH is
virtually i­dentical. Both are echogenic soft tissue masses with
high vascularity on color Doppler imaging. Compared to IH,
they may be more heterogeneous in echotexture with increased
conspicuity of intralesional vessels. Calcifications may be
present which are not seen with IH (Fig. 18.90) [167].
conservatively while NICH may go on to surgical resection.
right forearm. (b) Transverse color Doppler ultrasound image shows
hypervascularity of the mass
18 Musculoskeletal System 891

a b

Fig. 18.90 Rapidly involuting congenital hemangioma (RICH) in a cutaneous tissues of the left flank with prominent intralesional vessels
3-day-old male. A mass on the left back was present at birth, with spon- (arrows). (b) Transverse color Doppler ultrasound image shows marked
taneous resolution in the first months of life. (a) Longitudinal grayscale hypervascularity
ultrasound image demonstrates a well-circumscribed mass in the sub-

a b

T T

Fig. 18.91 Kaposiform hemangioendothelioma in a 4-day-old male a large, heterogeneous, infiltrative mass (arrows) of the subcutaneous
with an exophytic mass of the left leg, overlying purplish skin discolor- tissues. (b) Transverse color Doppler ultrasound image shows marked
ation and profound thrombocytopenia. (a) Transverse grayscale ultra- hypervascularity of the mass. T, Tibia
sound image obtained over the anteromedial mid-tibia (T) demonstrates

It is important to distinguish hemangiomas from malig-
nant neoplasms. Characteristics not typical of hemangiomas
include pain, rapid growth after 12 months of age, muscu-
lar extension, adenopathy, osseous destruction, calcification,
hemorrhage, or necrosis in a lesion not present at birth. These
findings should raise concern for an aggressive lesion such as
myofibromatosis, sarcoma, and metastases, warranting MR
imaging evaluation and typically biopsy [170].
Kaposiform Hemangioendothelioma
Kaposiform hemangioendothelioma (KHE) is a rare vascular
mass that may be present at birth or present later. Unlike hem-
angioma, it rarely involves the head and neck, preferentially
affecting the trunk, extremities, and retroperitoneum. KHE
is locally aggressive and grows rapidly. The involved skin
appears red to purple and is warm to the touch. An impor-
tant diagnostic clue for KHE is the presence of a consump-
tive coagulopathy, the Kasabach-Merritt phenomenon (KMP),
with 90% of cases of KMP being associated with KHE [167].
On ultrasound, KHE is infiltrative with poorly defined
borders, has more conspicuous vascular channels in the
mass, and also may contain calcifications, which are not
present in IH (Fig. 18.91) [167, 169].
While spontaneous resolution has been reported, KHE
may require medical therapy, including vincristine or corti-
costeroids. Surgical resection is possible in localized cases.
The mortality ranges from 10% to 30%, related to the asso-
ciation with KMP and the effects of local invasion [167].
Vascular Malformations
Vascular malformations are the result of structural abnor-
malities of veins, lymphatics, capillaries, and arteries. They
892
are present at birth and grow in proportion to the child. The
ISSVA classification system divides vascular malformations into
slow-flow and fast-flow lesions. Slow-flow vascular malforma-
tions include venous, lymphatic, and capillary malformations.
Capillary malformations involve superficial layers of skin,
are easily assessed on clinical examination rather than imag-
ing, and will not be discussed further. Fast-­ flow lesions
include arteriovenous malformation (AVM) and arteriove-
nous fistula (AVF).
Venous Malformation
Venous malformations (VMs) are the most common vas-
cular malformation. They consist of dysplastic, dilated
venous channels without normal mural smooth muscle
[171]. They are present at birth and grow proportionally
with the patient, with potential periods of further enlarge-
ment during puberty and pregnancy related to hormonal
influence. The overlying skin may be normal in appear-
ance or have a bluish hue. VMs are often soft and com-
pressible, increasing in size with the Valsalva maneuver,
and are most often seen in the head, neck, and extremities.
Ultrasound can demonstrate the varying morphology of
VMs including anechoic, enlarged vascular channels; cavern-
ous spaces; or heterogeneous, spongiform lesions. As flow is
slow or stagnant, flow with Doppler ultrasound may be diffi-
cult to detect or may be absent if there is associated thrombo-
sis. Phleboliths may be present, seen as echogenic, shadowing
a b
Fig. 18.92 Intramuscular venous malformation in a 17-year-old male.
(a) Transverse grayscale ultrasound image of the left triceps muscle
shows an intramuscular, predominantly echogenic lesion containing a
central dilated, tubular channel (arrow). (b) Transverse color Doppler
D. Y. Jarrett
foci. VMs may also have fluid-fluid levels in cystic spaces
and can be infiltrative with involvement of multiple soft tissue
compartments, extending through subcutaneous fat, muscles,
fascia, or tendons (Figs. 18.92 and 18.93) [169, 171].
When VMs are referred for treatment, potentially because
of pain, loss of function, or cosmetic concerns, they are typi-
cally treated with sclerotherapy.
Lymphatic Malformation
Lymphatic malformations (LMs) are the second most com-
mon vascular malformation after VM [172]. They result
from a congenital abnormality in development, leading to
dilated lymphatic channels that do not communicate with the
lymphatic system. 70–80% of LMs are located in the head
and neck. They are usually asymptomatic and may present
as a rapidly increasing mass secondary to hemorrhage or
infection. LMs contain cystic spaces and are characterized
as microcystic or macrocystic depending on the size of the
cysts. Varying definitions of macrocysts have been proposed,
generally on the order of 1–2 cm in diameter.
On ultrasound, a macrocystic LM appears as thin-walled
cystic spaces, with vascularity limited to the cyst walls. It
may contain echogenic material representing proteinaceous
debris or hemorrhage. In a microcystic LM, the cyst lumen
may be too small to discern with imaging, and the lesion
may appear as echogenic, solid soft tissue due to the wall
interfaces (Figs. 18.94 and 18.95).
ultrasound image with spectral analysis shows internal vascularity with
low-­velocity venous waveforms. A small echogenic, shadowing focus
(arrowhead) within a venous channel represents a phlebolith
18 Musculoskeletal System
a b
Fig. 18.93 Venous malformation in a 7-year-old male with a mass on
the palmar aspect of the right hand. (a) Transverse grayscale ultrasound
image obtained over the second and third metacarpal bones shows a
heterogeneous lesion (arrow) with fluid-filled spaces containing depen-
a b
Fig. 18.94 Microcystic lymphatic malformation in a 17-year-old
male. (a) Transverse grayscale ultrasound image of the right lower leg
shows focal thickening and heterogeneous echogenicity of the subcuta-
Fig. 18.95 Lymphatic malformation in a 5-year-old male with right
upper arm swelling. Longitudinal color Doppler ultrasound image of
the right arm shows a multiloculated cystic lesion in the subcutaneous
tissues containing both macrocysts and microcysts with no internal
vascularity
893
dent, mobile, echogenic material compatible with blood products. A
central echogenic structure represents a thrombus (asterisk). (b) Axial
T2-weighted, fat-suppressed MR image shows the hyperintense malfor-
mation with a central hypointense thrombus (arrowhead)
neous tissues (arrow) with no evident cysts. (b) Transverse grayscale
ultrasound image of the normal left lower leg shown for comparison
 rteriovenous Malformation and Arteriovenous
A
Fistula
AVMs contain a nidus of dysplastic arteries that drain into
veins without an intervening capillary bed. They may be
single or multiple. On physical examination, there will be
a warm, red, pulsatile mass with a palpable thrill. An AVM
may increase in size as a result of hormonal influence during
puberty and pregnancy, trauma, or the recruitment of normal
vessels by shunting across the low-resistance arterial and
venous connections [167].
AVFs may be present at birth or acquired as a result of
trauma. Instead of a nidus, an AVF usually has a single
abnormal arterial to venous connection.
Both AVM and AVF can cause pain and soft tissue ulcer-
ation from a vascular steal phenomenon and resulting ischemia.
On ultrasound imaging, these high-flow malformations
will appear as enlarged arteries with high-flow, low-­resistance
894
a b
Fig. 18.96 Arteriovenous malformation in a 13-year-old male. (a)
Transverse grayscale ultrasound image of the left suprascapular region
shows multiple enlarged vessels without an associated soft tissue mass.
(b) Color Doppler ultrasound image with spectral analysis demonstrates
waveforms and large draining veins with arterialized, abnor-
mally pulsatile waveforms (Fig. 18.96).
AVM can be treated with surgery and embolization. An
AVF is most often treated by coiling of the abnormal arterio-
venous connection.
Acknowledgments The author would like to thank Emil M. Grinberg
and Joseph Anthony Myers for their help in creating the figures for this
chapter.
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 Vascular Imaging
19
Harriet J. Paltiel

SVC Superior vena cava

Abbreviations
US Ultrasound
AVF Arteriovenous fistula
BCV Brachiocephalic vein
CCA Common carotid artery Introduction
CEUS Contrast-enhanced US
CFA Common femoral artery Abnormalities of the neck vessels and peripheral and retroperi-
CFV Common femoral vein toneal vasculature in children are relatively rare. Nonetheless,
CT Computed tomography increasing numbers of hospitalized children and those with
DVT Deep vein thrombosis chronic conditions routinely require ultrasound evaluation
ECA External carotid artery of these vessels prior to diagnostic procedures and follow-
EDV End-diastolic velocity ing therapeutic intervention. In this chapter, ultrasound tech-
FV Femoral vein niques used in evaluation of the pediatric vasculature, pertinent
ICA Internal carotid artery embryology, and normal anatomy are reviewed, and the ultra-
IJV Internal jugular vein sound findings, associated clinical features, and management
IMT Intima-media thickness approach to vascular disorders in children are discussed.
IV Intravenous
IVC Inferior vena cava
MIP Maximum intensity projection Neck Vessels
MR Magnetic resonance
MRA Magnetic resonance angiography Indications for ultrasound evaluation of the carotid artery in
NF Neurofibromatosis type 1 children include assessment for stenosis or thrombosis. The
PICC Peripherally inserted central catheter internal jugular vein (IJV) is usually imaged to determine
PRF Pulse repetition frequency patency prior to central line insertion, to diagnose thrombo-
PSV Peak systolic velocity sis, and to evaluate venous ectasia.
RI Resistive index
SCA Subclavian artery
SCV Subclavian vein Technique
SFA Superficial femoral artery
SMA Superior mesenteric artery Patient Positioning
Ultrasound studies of the carotid artery and IJV are performed
with the patient supine, the neck hyperextended, and the head
Electronic Supplementary Material The online version of this chapter rotated to the opposite side. Exposure of the anterior neck can
(https://doi.org/10.1007/978-3-030-56802-3_19) contains supplemen- be facilitated by placing a pillow under the upper back.
tary material, which is available to authorized users.

H. J. Paltiel (*) Ultrasound Transducer Selection

Division of Ultrasound, Department of Radiology,
Boston Children’s Hospital and Harvard Medical School,
Boston, MA, USA
e-mail: harriet.paltiel@childrens.harvard.edu
High-frequency linear transducers are used to optimize image
resolution. Occasionally, a curvilinear transducer or use of an
extended field of view will be helpful in delineating a large

© Springer Nature Switzerland AG 2021 899

H. J. Paltiel, E. Y. Lee (eds.), Pediatric Ultrasound, https://doi.org/10.1007/978-3-030-56802-3_19
900
abnormality. A small footprint vector transducer may be use-
ful when imaging the medial subclavian vein (SCV), lower
IJV, and the brachiocephalic vein, particularly in young chil-
dren with limited ability to cooperate with the examination or
in patients with decreased mobility of the neck.
Imaging Approaches
Transverse, longitudinal, and oblique ultrasound images of the
neck are obtained with grayscale, color, and spectral Doppler
imaging. Color and power Doppler imaging outline the course
of the patent vessel lumen and facilitate placement of a
Doppler cursor when performing velocity measurements.
Initial grayscale evaluation is performed in the transverse
plane. Images are obtained from the supraclavicular notch to
the angle of the mandible. Inferior angulation of the trans-
ducer in the supraclavicular notch is used to image the infe-
rior portions of the common carotid artery (CCA) and
brachiocephalic vein. Color Doppler studies should be per-
formed with sensitivity and gain settings that anticipate ves-
sel flow velocities and optimize the color display. Pulse
a b
C
I
Fig. 19.1 Common carotid artery (CCA) bifurcation in a 2-year-old
female. (a) Sagittal grayscale ultrasound image of the right CCA (C)
shows mild widening at the bulb (arrows) just proximal to its bifurca-
a b
Fig. 19.2 Normal pulsatility of the internal jugular vein (IJV) in a
12-year-old female. (a) Sagittal color Doppler ultrasound image shows
the IJV at its widest during a normal cardiac cycle. (b) Sagittal color
H. J. Paltiel
repetition frequency (PRF) and frame rate must be set to per-
mit accurate characterization of vascular hemodynamics.
Use of a large color image area will slow down the frame
rate, and the deeper a vessel is located from the transducer
face the slower the required PRF. The whole vessel lumen
should be filled with color flow without leakage of color sig-
nal into the adjacent stationary soft tissues.
When imaging the CCA, the carotid bulb appears as a
widening of the artery close to its bifurcation. Transverse
images of the carotid bifurcation will depict the orientation
of the internal and external carotid arteries and help deter-
mine the optimal longitudinal or oblique planes in which to
perform color and spectral Doppler imaging (Fig. 19.1).
The IJV is readily imaged in both transverse and longitu-
dinal planes. Venous pulsations are seen with grayscale and
Doppler imaging that are related to right heart contraction
(Fig. 19.2). Changes in diameter of the IJV reflect changes in
intrathoracic pressure. Inspiration causes negative intratho-
racic pressure that results in flow to the heart and a decrease
in jugular venous diameter. Expiration and the Valsalva
tion into the external (E) and internal (I) carotid arteries. (b) Transverse
color Doppler ultrasound image depicts the bifurcation into the external
carotid artery (ECA) and internal carotid artery (ICA)
Doppler ultrasound image shows the IJV in a relatively collapsed state
during the same cardiac cycle
19 Vascular Imaging
maneuver will cause increased intrathoracic pressure with
decreased blood flow to the heart and venous enlargement.
Coronal scanning through the supraclavicular fossa is per-
formed to evaluate the confluence of the medial SCV and lower
IJV, as well as the superior portions of the brachiocephalic vein
(Fig. 19.3). Images are obtained with light transducer pressure
to minimize compression of the IJV (Fig. 19.4). Comparison
views of the opposite side are helpful to highlight asymmetry.
Cine clips can be used to demonstrate venous compressibility
and to determine the extent of an abnormality that exceeds the
ultrasound field of view.
a b
Fig. 19.3 Confluence of IJV, subclavian vein (SCV), and brachioce-
phalic vein (BCV) in a 12-year-old female. (a) Coronal color Doppler
ultrasound image of the confluence of the left SCV (asterisk) and bra-
a b
V
C
Fig. 19.4 Normal compressibility of the IJV in a 12-year-old female.
(a) Transverse grayscale ultrasound image of the left neck obtained
with light transducer pressure shows a normal IJV (V) and CCA (C).
901
Carotid Artery
Normal Development and Anatomy
Normal Development
In embryonic life, the paired primitive right and left dor-
sal aortas connect ventrally with paired pharyngeal arch
arteries. The third pharyngeal arch artery gives rise to the
CCA and the proximal part of the internal carotid artery
(ICA). The external carotid artery arises as a bud from this
arch [1].
chiocephalic vein (B). (b). Transverse color Doppler ultrasound image
shows the confluence of the left IJV (arrow) with the medial left SCV
(asterisk)
C
(b) Transverse grayscale ultrasound image of the left IJV obtained with
firm transducer pressure shows normal collapsibility of the vein.
C, CCA
902
Normal Anatomy
In about 75% of the population, the first branch of the aortic
arch is the brachiocephalic artery which divides into the right
common carotid and subclavian arteries. The second branch
of the aortic arch is the left CCA which has a separate origin
from the left subclavian artery (SCA) (Fig. 19.5).
Both common carotid arteries ascend into the neck deep
to the IJV and the sternocleidomastoid muscle and postero-
lateral to the thyroid gland. The CCA usually gives off no
branches in the neck. At its bifurcation it divides into the ICA
and external carotid artery (ECA). The ICA usually gives off
no cervical branches. The ECA has multiple cervical branches
that supply the muscles of the face (Fig. 19.6).
Longitudinal ultrasound images of the CCA depict the
layers of the wall as two parallel, echogenic lines separated
by a hypoechoic zone. The distance between these layers
represents the thickness of the intima and media or intima-
media thickness (IMT) (Fig. 19.7). IMT and carotid distensi-
bility do not appear to increase significantly with age or
differ between males and females [2].
Doppler imaging of the CCA reveals a narrow frequency
spectrum in systole and a somewhat wider spectrum in dias-
tole with a clear spectral window (Fig. 19.8) [3]. Since approx-
imately 80% of CCA flow extends into the ICA, its frequency
spectrum resembles that of ICA, and forward flow occurs
throughout the cardiac cycle. CCA velocity measurements
have not been described in children. In adults, peak systolic
velocities (PSVs) of 78–118 cm/sec, end-diastolic velocities
(EDVs) of 20–32 cm/sec, and resistive indices (RIs) of 0.72–
0.84 have been reported [4].
The ICA supplies the low-resistance cerebral vessels and dem-
onstrates forward flow throughout systole and diastole (Fig. 19.9).
In neonates, PSVs that range between 60 and 68 cm/sec, EDVs of
Internal carotid
artery
External jugular
vein
Internal jugular
vein
Superficial cervical
artery
Dorsal scapular
artery
Suprascapular
artery
Subclavian
artery
Subclavian vein
R L
Brachiocephalic Brachiocephalic
artery vein
Fig. 19.5 Diagram of normal vessels to the neck and upper extremities
H. J. Paltiel
14–17 cm/sec, and arterial RIs of 0.72–0.78 have been reported
[5]. Normal velocity measurements for these vessels in older chil-
dren have not been established. In adults, these measurements are
increased, with PSV of 62–90 cm/sec, EDV of 23–37 cm/sec, and
RIs of 0.54–0.66 [4].
Since the ECA supplies the high-resistance vascular bed
of the facial muscles, flow in the ECA resembles that of other
peripheral arteries, with a sharp systolic peak and a rapid
decline during diastole that may approach zero or briefly
reverse direction (Fig. 19.10) [3]. Normal velocity measure-
ments have not been reported in children. In adults, normal
values include PSV of 57–87 cm/sec, EDV of 11–21 cm/sec,
and RIs of 0.72–0.84 [4].
Anatomic Variants
In approximately 15% of individuals, there is a common ori-
gin of the brachiocephalic and left common carotid arteries,
the so-called “bovine arch” configuration. In the remaining
10%, the left CCA arises from the brachiocephalic artery
proper, rather than from a common trunk.
Thrombosis and Stenosis
Carotid artery thrombosis and stenosis are unusual in chil-
dren. ICA thrombosis can occur as a rare complication of an
impalement injury of the oral cavity, or secondary to vascu-
litis, as in the setting of Takayasu arteritis.
Penetrating palatal wounds are caused by objects in the
mouth such as pencils, toothbrushes, pieces of wood, or plas-
tic [6]. ICA thrombosis in this setting occurs in fewer than
1% of patients. It is thought to result from compression of
the ICA against a cervical vertebra leading to an intimal tear
that serves as the nidus for thrombosis [7]. Signs of ICA
injury include a hematoma in the lateral neck and Horner’s
External carotid
artery
Inferior thyroid
vein
Anterior jugular
vein
Common cartoid
artery
Brachiocephalic
vein
19 Vascular Imaging 903

Basilar

Right internal
carotid

Right external Left internal

carotid carotid
Left external
Vertebral carotid

Right common Left common

carotid carotid
Right subclavian
Left internal
mammary

Right internal
mammary Left subclavian
Brachiocephalic
(innominate) Aortic arch

Fig. 19.6 Diagram of normal arterial supply to the neck, face, and brain

Fig. 19.7 Normal intima-media thickness (IMT) of the right CCA in a

17-year-old female. Sagittal ultrasound image shows the IMT between
arrowheads

Fig. 19.8 Normal CCA in a 15-year-old female. Longitudinal color

syndrome (ipsilateral ptosis, meiosis, and anhidrosis) [8].
These signs can develop up to 3 days after injury [7]. Doppler
ultrasound evaluation of the neck can be performed to diag-
nose a cervical hematoma and to evaluate flow in the ICA in
selected patients.
Takayasu arteritis is an idiopathic, granulomatous vasculi-
tis of the aorta and its major branches and is one of the most
common vasculitides in children. Inflammation and intimal
Doppler ultrasound image with spectral analysis shows normal flow
with relatively low-resistance spectral Doppler waveforms
proliferation result in wall thickening, stenosis, and thrombo-
sis. Aneurysms and dissection result from destruction of the
muscular and elastic layers of the vessel wall. Children typi-
cally present with hypertension, vascular bruits, ­asymmetric
blood pressure between limbs, or asymmetric arterial pulses in
904
Fig. 19.9 Normal internal carotid artery (ICA) in a 15-year-old female.
Longitudinal color Doppler ultrasound image with spectral analysis
shows normal flow with low-resistance spectral Doppler waveforms
the extremities, sometimes accompanied by fever, malaise, or
musculoskeletal symptoms. The absence of specific symptoms
and laboratory biomarkers and difficulties in determining dis-
ease activity and progression often lead to delayed diagnosis
and underestimation of disease activity [9].
The diagnosis is confirmed by imaging documentation of
large vessel wall abnormalities, including stenosis, occlusion,
aneurysms, and collateral circulation on computed tomogra-
phy angiography (CTA), magnetic resonance angiography
(MRA), or conventional catheter angiography. Ultrasound
with color Doppler can detect vascular stenosis, thrombosis,
and aneurysms (Fig. 19.11). Diffuse thickening of the IMT has
been described, the so-­called “Macaroni” sign.
Non-invasive means of monitoring disease activity include
documentation of an abnormally hypoechoic appearance of
the vessel wall that precedes the development of stenosis
or occlusion [10], as well as contrast-­enhanced ultrasound
(CEUS) assessment of wall vascularity [11].
Carotid artery thrombosis is diagnosed when no intralu-
minal flow is detectable. Slow-flow color Doppler sensitivity
settings should be used or power Doppler, with its increased
sensitivity to slow-velocity and low-amplitude signals. The
pulsed Doppler cursor should be located centrally within the
vessel lumen to avoid transmitted pulsations from an adja-
cent vessel.
Most penetrating palatal injuries heal without sequelae.
Optimal treatment of ICA thrombosis after penetrating palatal
trauma has not been determined. Some authors advocate anti-
coagulation, while others recommend thrombolytic agents.
The benefits of surgical intervention are currently unproven
H. J. Paltiel
Fig. 19.10 Normal external carotid artery (ECA) in a 15-year-old
female. Longitudinal color Doppler ultrasound image with spectral
analysis shows normal flow with relatively high-resistance spectral
Doppler waveforms
[7]. The goal in treatment of Takayasu arteritis is to induce and
maintain disease remission. Medical treatment with glucocor-
ticoids combined with antihypertensive and antiplatelet drugs
works well in most patients. Additional immunosuppressant
drugs and biological agents are necessary in some children.
Endovascular or surgical revascularization therapy is an option
in patients with cerebrovascular disease [12].
Extracranial ICA stenosis can also occur in patients with
sickle cell anemia which is associated with an increased risk of
stroke. There are currently no velocity criteria for children
comparable to those used in adults with atherosclerosis to
determine a clinically significant degree of arterial stenosis.
Some authors advocate use of a time-­averaged mean of maxi-
mal velocity technique adapted from the transcranial Doppler
ultrasound method commonly used for screening children
with sickle cell anemia. A velocity ≥ 160 cm/sec is predictive
of extracranial ICA stenosis with 80% sensitivity and 100%
specificity [13].
Treatment of cervical carotid artery disease in patients
with sickle cell anemia consists of acute anticoagulation
and exchange transfusion, with maintenance on a long-
term transfusion program [14].
19 Vascular Imaging
a b
c d
Fig. 19.11 Carotid artery occlusion in a 15-year-old female with
Takayasu arteritis. (a) Sagittal power Doppler ultrasound image reveals
complete occlusion of the left CCA (C). Flow is identified in the IJV
(V). (b) Sagittal color Doppler ultrasound image shows a thick-walled,
narrow left ICA (arrows) with antegrade flow. (c) Sagittal color Doppler
ultrasound image of the left ICA. Spectral Doppler analysis shows a
delayed and blunted systolic upstroke related to more proximal stenosis
Aneurysm
An aneurysm can be defined as a localized increase in the
diameter of an artery of at least 50% compared to the normal
diameter of that artery [15, 16].
Extracranial carotid artery aneurysms in children are
extremely rare but are associated with a high mortality and
morbidity. They can be congenital or inflammatory in etiol-
ogy or may develop as a complication of pharyngeal infec-
tion or trauma. Extracranial carotid aneurysms occur most
often in the ICA, followed by the CCA, and ECA. There is a
nearly 2:1 male predominance in children. Clinical presenta-
tion ranges from an asymptomatic mass to rapidly fatal hem-
orrhage [17, 18].
Although angiography is considered the reference stan-
dard imaging technique, Doppler ultrasound evaluation
is usually initially performed and can provide useful infor-
905
e
and occlusion. (d) Sagittal color Doppler ultrasound image of the left
ECA. The vessel is narrowed and thick-walled. Spectral Doppler analy-
sis demonstrates reversed flow direction with a delayed and blunted
systolic peak. (e) Coronal oblique magnetic resonance angiography
(MRA) image confirms complete occlusion (arrowhead) of the left
CCA just beyond its origin. The right brachiocephalic artery (arrow)
and its branches appear normal
mation regarding aneurysm size, extent, flow characteristics,
presence of mural thrombus, and dissection.
Because the natural history of asymptomatic aneurysms
is unknown and the risk of central nervous system complica-
tions is high, early operative intervention is advised whether
or not the child is symptomatic [17, 19].
Dissection
Craniocervical arterial dissection in childhood occurs in 2.5
children per 100,000 per year and usually presents with symp-
toms of acute ischemic stroke or as a transient ischemic attack
[20]. Risk factors for dissection in children include head and
neck injury, connective tissue disorders, and male gender.
Extracranial dissections account for 5–25% of childhood-­
onset acute ischemic stroke and are often preceded by trauma
[21]. Post-traumatic dissection is associated with cervical
906
spine injuries, deformities, and/or instability and the presence
of basilar skull fractures, facial and/or chest injuries, and focal
neurological deficits. Children are at particular risk for dissec-
tion because of a lower craniocervical stability related to
immature neck musculature, higher dependency on ligamen-
tous rather than bony structures, large head to neck proportion,
and less well-developed protective reflexes [22].
Symptoms of post-traumatic cerebrovascular injuries are
nonspecific and often lead to misdiagnosis. When unrecog-
nized and untreated, hemorrhage and/or acute ischemic stroke
can lead to neurological sequelae, frequently with a devastat-
ing outcome. The extracranial ICA is usually affected more
than 2 cm above its origin in contrast to atherosclerotic dis-
ease, where the ICA is more often involved at its origin or in
the region of the siphon. Patients typically present with head-
ache associated with anterior neck pain or ischemic symp-
toms. Symptoms may develop suddenly or progress slowly
over more than a week [23].
Magnetic resonance (MR) imaging is the reference imag-
ing technique for diagnosis of ICA dissection. Doppler ultra-
sound findings include decreased or absent flow velocities in
the injured vessel and in some cases can directly visualize
the intimal flap. Ultrasound can be useful in follow-up by
documenting a return to normal flow velocities and direc-
tionality [23, 24].
There is no current consensus on the best treatment for
extracranial carotid artery dissection. Recently published
childhood stroke guidelines recommend anticoagulation.
Endovascular or surgical procedures are recommended for
those children with ongoing symptoms despite aggressive
medical therapy [21].
Internal Jugular Vein
Normal Development and Anatomy
Normal Development
The earliest vessels of the cranium form a primordial hind-
brain channel which drains into the precardinal vein. The
cranial portion of the precardinal vein eventually forms the
internal jugular vein (IJV).
Normal Anatomy
The IJV is formed by the union of inferior petrosal and sig-
moid dural venous sinuses in or just distal to the jugular fora-
men. It descends in the carotid sheath along with the ICA
with the vagus nerve located between them.
The IJV receives tributaries from the face and neck and
descends into the thorax behind the two heads of the sterno-
cleidomastoid muscle. It unites with the SCV to form the
brachiocephalic vein (Fig. 19.12).
H. J. Paltiel
The IJV transports venous blood from the brain to the
heart. A normal IJV will show complete collapse with mod-
erate transducer pressure. Patient sniffing will reduce intra-
thoracic pressure with brief collapse of the vein and increased
venous flow toward the heart that can be documented with
spectral Doppler (Fig. 19.4) [25, 26].
Anatomic Variants
An asymmetry in size of the right and left IJVs is very com-
mon, with the left usually larger than the right (Fig. 19.13) [27,
28]. IJV caliber can vary markedly as a reflection of hydration
status, heart rate and function, rate and amplitude of respira-
tion, postural changes, and compression from adjacent struc-
tures [28]. Other variants, such as duplication and fenestration,
are rare and usually discovered incidentally [29].
Congenital Anomalies
Jugular Vein Phlebectasia
Jugular vein phlebectasia is a rare abnormality of the IJV in
children that consists of fusiform dilation of a segment of the
IJV. Clinically it presents as a transient soft, painless mass in
the lateral aspect of the neck when intrathoracic pressure is
increased, as when coughing, sneezing, crying, bending over,
or performing a Valsalva maneuver [30]. It is believed to occur
as a result of focal mural weakness in the wall (Fig. 19.14,
Cineclip 19.1 and 19.2). It affects males more than females
and is usually unilateral and right-sided, although both IJVs
can be affected.
Dynamic ultrasound of the neck with and without a
Valsalva maneuver is useful in confirming the diagnosis of
phlebectasia when the affected vein dilates to a diameter
approximately twice its resting diameter [31]. Color Doppler
ultrasound will depict blood flow and can exclude the pres-
ence of thrombosis.
Treatment of jugular vein phlebectasia is generally con-
servative. However, surgery can be performed if the patient
develops progressive enlargement or thrombosis of the ectatic
vein. Surgical options described in the literature include liga-
tion, venoplasty, and resection [32].
Thrombosis
The most frequent indication for venous Doppler ultrasound
in children is to diagnose deep vein thrombosis (DVT).
Undetected and untreated DVT can lead to fatal pulmonary
embolism. Although the incidence of venous thromboembo-
lism in childhood is significantly lower than that in adults, it
is increasingly recognized in the pediatric population as a
complication of present-day health care. Central venous
catheters are mandatory for the management of critically ill
children, and the most common cause of jugular vein throm-
bosis is the presence of a central venous catheter [33, 34].
19 Vascular Imaging 907

Temporal
Superior
cerebral veins
Deep cerebral
veins
Inferior sagittal
sinus
Cavernous
Superior sinus
sagittal sinus
Maxillary
Straight sinus
Great cerebral
vein
Right transverse
sinus
Superior petrosal
sinus
Occipital sinus Facial
Inferior petrosal
sinus
Internal
Sigmoid sinus jugular
Vertebral
Right
External jugular brachiocephalic
Subclavian
Left
Clavicle brachiocephalic

Axillary Superior vena

cava
First rib Internal thoracic
Second rib

Fig. 19.12 Diagram of normal anatomy of the veins of the head and neck

a b

C C

Fig. 19.13 Asymmetric IJVs in a 7-month-old female. Transverse grayscale ultrasound images of the right (a) and left (b) IJVs. The right (R) IJV
is significantly smaller than the left (L) IJV. C, CCA
908
a b
Fig. 19.14 Jugular vein phlebectasia in a 2-year-old male. (a) Sagittal and (b) transverse grayscale ultrasound images of the left IJV show a mark-
edly ectatic vessel. (c) Spectral Doppler analysis depicts normal venous flow
Additional risk factors include the hypercoagulability asso-
ciated with malignancy, polycythemia, deep neck infections,
immobilization, trauma, head and neck surgery, and intrave-
nous (IV) drug abuse.
Central venous catheter-related IJV thromboembolism is
usually asymptomatic or manifests with chronic symptoms,
including repeated loss of catheter patency, catheter-related
sepsis, and prominent collateral circulation in the skin over
the chest, back, neck, and face [35–37]. Symptomatic throm-
boembolism manifests acutely with swelling of the face, pul-
monary embolism, chylothorax, and/or superior vena cava
(SVC) syndrome (characterized by a sensation of fullness in
the head, dilated neck veins, dyspnea, and mediastinal wid-
ening on chest radiography).
Lemierre syndrome is a rare and potentially lethal oro-
pharyngeal infection that develops in immunocompetent
adolescents and young adults and is mainly caused by
Fusobacterium necrophorum. It usually manifests as a septic
thrombophlebitis of the IJV although other veins of the head
and neck may be affected and can lead to septic pulmonary
emboli. Patients present with fever, neck pain, and tonsillo-
pharyngitis [38].
Ultrasound is the preferred initial imaging modality for the
diagnosis of venous thromboembolism. Abnormalities
include a dilated, non-compressible vein, intraluminal echoes,
lack of color flow, and abnormal spectral Doppler venous
waveforms (Fig. 19.15) [39]. Serial ultrasound examinations
are useful for monitoring the response to therapy. A normally
compressible vein will be seen after complete resolution of
thrombosis. Features of chronic thrombosis include wall
thickening, intraluminal webs, and phleboliths (Fig. 19.16).
Collateral vessels can also be readily imaged.
H. J. Paltiel
c
Ultrasound is also a useful screening modality for Lemierre
syndrome, but it is limited in showing the full extent of throm-
bosis and may not allow adequate assessment of the associated
inflammatory findings which are better depicted at CT [40].
Stenosis
Stenosis in the upper third of the IJV is common and occurs
as a result of compression of the vessel between the trans-
verse process of C1 or C2 and the occipital bone [41].
Aneurysm
A venous aneurysm is defined as an isolated region of dila-
tion that communicates with a normal sized venous segment
and is not associated with prior trauma. Most venous aneu-
rysms arise from the superficial veins in the head, neck, or
extremities. Venous aneurysms differ from phlebectasia in
that they are usually acquired, not congenital lesions, and are
typically diagnosed in adults.
A venous aneurysm differs from a varicose vein in that it is
an isolated lesion, and there is no associated venous elongation
as occurs with a varicosity. Aneurysms of the IJV are rare. Two
types have been described, fusiform (more common) and sac-
cular [42]. Patients present with neck swelling that is com-
pressible and accentuated with the Valsalva maneuver.
Doppler ultrasound imaging depicts a focal enlargement
of the internal jugular vein that increases with straining.
Images of both sides of the neck should be obtained because
the lesions can be bilateral.
Congenital jugular vein aneurysms should be excised
only if they are symptomatic, enlarging, or disfiguring [43].
A unilateral aneurysm can be safely treated by ligation, with
a low complication rate.
19 Vascular Imaging
a b
Fig. 19.15 Right IJV thrombosis in a 19-year-old female with sickle
cell disease and a central venous port catheter. (a) Chest radiograph
shows the central venous port catheter with tip (arrow) at the junction of
a b
Fig. 19.16 Chronic right IJV thrombosis in a 7-month-old female with
congenital heart disease and a right upper extremity peripherally inserted
central catheter (PICC). (a) Sagittal grayscale ultrasound image reveals
marked mural thickening (arrow) of the lower right IJV with significant
luminal narrowing. (b) Sagittal color Doppler ultrasound image with
Extremity Arteries
Arterial diseases that affect the extremities in childhood are
uncommon and are usually due to acute or remote iatrogenic
injury related to arterial cannulation, penetrating trauma, or
vasculitis.
Technique
Patient Positioning
When imaging the upper extremity arteries, the patient’s
head is turned away from the transducer to help visualize the
909
the superior vena cava (SVC) and right atrium. (b) Sagittal grayscale
ultrasound image of the right neck demonstrates a thrombus-filled, dis-
tended right IJV (arrow). C, CCA
c
spectral analysis depicts an abnormal, dampened venous waveform in the
residual lumen. (c) Sagittal color Doppler ultrasound image demonstrates
multiple collateral vessels (arrowheads) adjacent to the narrowed portion
of the lower right IJV
more proximal parts of the subclavian and ­brachiocephalic
arteries. The axillary arteries are examined with the trans-
ducer in the axilla and the patient’s arm elevated. The rest of
the arterial system is examined with the arm slightly abducted
and rotated externally. The position of the transducer for
examination of the SCA varies depending on whether the
distal portion or medial portion of the artery is studied. A
supraclavicular approach is used to evaluate the medial seg-
ment of the SCA, and an ­infraclavicular approach to study
the lateral portion of the SCA.
Imaging of the lower extremity arteries is performed with
the patient supine or in a semi-erect position with the head
elevated about 30°. The leg should be mildly abducted and
910
externally rotated, and the knee slightly flexed. Each arterial
segment is scanned in the longitudinal plane with grayscale
and Doppler imaging. Transverse images may also be useful.
Representative longitudinal color Doppler and/or grayscale
images as well as angle-corrected spectral Doppler wave-
forms with velocity measurements should be documented
for each arterial segment. Cine clips can be used to document
any flow abnormality [44]. When evaluating the popliteal
artery, a decubitus position may be useful.
Ultrasound Transducer Selection
The highest-frequency transducer that permits adequate pen-
etration and visualization should be used, generally a
5–12 MHz linear array transducer, with a higher-frequency
transducer used in areas where the arteries are more superfi-
cial. Occasionally, a 3–5 MHz sector or curved linear array
probe may be necessary in large patients or in individuals
with substantial edema.
Imaging Approaches
Grayscale imaging is initially performed to determine the
presence of echogenic thrombus or an extravascular mass.
Color Doppler imaging permits a preliminary overview of
the area of interest, and spectral Doppler ultrasound is then
used to determine the blood flow patterns. The spectral
Doppler gate is adjusted within the arterial lumen to opti-
mize flow signal. A medium or high wall filter is generally
used in evaluating the arteries. A low wall filter can be used
to improve detection of slow flow. Power Doppler may be
more sensitive than color Doppler in the detection of slow
flow. CEUS can also be used to evaluate patients with periph-
eral arterial occlusive disease [45].
For upper extremity examinations, the subclavian, axillary,
and brachial arteries are evaluated down to the elbow, with other
a b
Fig. 19.17 Normal right upper extremity arteries in a 17-year-old female.
(a) Sagittal grayscale ultrasound image shows the subclavian artery (SCA)
with a smooth, mildly echogenic wall. (b) Sagittal color Doppler ultra-
H. J. Paltiel
vessels studied as clinically indicated, including the innominate,
radial, and ulnar arteries and the palmar arches. Angle-corrected
spectral Doppler measurements of arterial velocity should be
obtained proximal to, at, and beyond any suspected stenosis.
For lower extremity examinations, evaluation begins in the
groin and includes the common femoral artery (CFA); proximal
deep femoral artery; proximal, mid, and distal superficial femo-
ral artery (SFA); and popliteal artery (above and below the
knee). When clinically indicated, imaging of the common and
external iliac arteries, tibioperoneal trunk, and anterior tibial,
posterior tibial, peroneal, and dorsalis pedis arteries should be
performed [44].
The posterior tibial artery is identified on longitudinal imag-
ing of the mid-calf using a posteromedial approach. The artery
can then be traced both proximally and distally. Another method
is to identify the artery at the medial malleolus of the ankle and
then follow it cranially. On anterior imaging of the leg, the ante-
rior tibial artery is identified along the interosseous membrane
near the fibula. The peroneal artery can also be seen from an
anterior approach where it appears posterior to the interosseous
membrane. A posterolateral approach is used to identify the
peroneal artery.
On grayscale imaging, normal extremity arteries have a
smooth wall and an anechoic lumen. Their walls are thicker
than those of the adjacent veins and do not collapse when
light pressure is applied with the transducer. In normal upper
and lower extremity arteries, laminar flow is present without
turbulence or aliasing on color Doppler. At rest, a high-resis-
tance triphasic waveform is typically noted, with a sharp sys-
tolic upstroke, transient flow reversal in early diastole and a
short phase of low-velocity, antegrade flow in late diastole
(Figs. 19.17, 19.18, and 19.19). Following exercise, there is
a relative increase in antegrade diastolic flow that results in a
lower-resistance waveform.
c
sound image with spectral analysis reveals normal color flow and a tripha-
sic waveform. Similar triphasic waveforms are depicted within the (c)
axillary artery (Fig. 19.17 continues)
19 Vascular Imaging 911

d e

f g

Fig. 19.17 (continued) (d) brachial artery, (e) radial artery, and (f) ulnar artery. (g) Transverse color Doppler ultrasound image shows the normal
relationship of the right radial and ulnar arteries

a b c

Fig. 19.18 Normal right lower extremity arteries in a 15-year-old female. Sagittal color Doppler ultrasound images with spectral analysis reveal normal
color flow and triphasic waveforms in the (a) external iliac, (b) common femoral, (c) superficial femoral (Fig. 19.18 continues)
912 H. J. Paltiel

d e

Fig. 19.18 (continued) (d) popliteal, and (e) dorsalis pedis arteries

a b c

Fig. 19.19 Normal right calf arteries in a 14-year-­old female. (a) Spectral Doppler analysis of the posterior tibial artery (b) and peroneal
Sagittal color Doppler ultrasound image shows the posterior tibial artery (c) reveals low-velocity triphasic waveforms
artery (arrow) and the peroneal artery (arrowhead) located more deeply.
19 Vascular Imaging 913

Normal Development and Anatomy arteries grow into the early upper limb buds and form the
subclavian-axillary-brachial trunk.
Upper Extremity The distal portion of the trunk becomes the interosseous
The upper extremity receives its arterial supply from the artery that initially supplies the plexus of arteries in the prim-
SCA. itive hand. A branch of the trunk artery, the median artery,
temporarily replaces the interosseous artery in feeding the
Normal Development hand.
In the embryo, the right fourth pharyngeal arch artery Subsequently, the ulnar and radial arteries develop and
becomes the proximal part of the right SCA. The distal part supply the forearm as well as the superficial and deep palmar
of the right SCA arises from the right dorsal aorta and the arches of hand. The deep branch of the brachial artery as well
right seventh intersegmental artery. The left SCA is not as the arteries around the shoulder and elbow arise later as
derived from a pharyngeal arch artery; it forms instead from branches of the primary axial vessel.
the left seventh intersegmental artery [46]. The subclavian
Normal Anatomy
Right subclavian The vertebral artery, internal mammary artery, thyrocervical
and costocervical trunks, and dorsal scapular artery arise from
the SCA which extends laterally across the upper chest. The
Axillary
SCA lies anterior to the SCV when evaluated from the supra-
clavicular fossa. It continues as the axillary artery at the level
Humeral circumflex
of the first rib. Below the margin of the teres major muscle, it
continues as the brachial artery to the antecubital fossa where
it divides into the radial and ulnar arteries.
Deep brachial The radial and ulnar arteries extend to the wrist where they
anastomose with each other. The radial artery gives rise to the
deep palmar arch which supplies the metacarpals of the hand,
and the ulnar artery gives rise to the superficial palmar arch,
Brachial which supplies the digits of the hand (Fig. 19.20).

Lower Extremity
Ulnar collateral The lower extremities receive their arterial blood supply from
the CFA (Fig. 19.21).

Radial Normal Development

In the embryo, the umbilical artery gives off a small branch,
the ischiadic artery, that temporarily supplies the growing
limb. The external iliac artery arises from the umbilical
Anterior crural artery slightly proximal to the ischiadic artery and soon pro-
interosseous Ulnar
vides most of the blood supply to the limb. The external iliac
artery gives rise to the femoral, popliteal, and posterior tibial
arteries. The popliteal artery anastomoses with the ischiadic
Deep palmar arch
artery, and the lower ischiadic artery eventually develops
Superficial palmar arch into the peroneal and inferior geniculate arteries. The ischi-
adic artery also supplies the early vascular plexus of the foot.
Digital
Normal Anatomy
The CFA arises from the external iliac artery at the level of
the inguinal ligament. It extends caudally for several centi-
Fig. 19.20 Diagram of the upper extremity arteries meters where it divides into the superficial femoral artery
914 H. J. Paltiel

a b
Common
iliac artery
Aorta
External
iliac artery Internal iliac
artery
Circumflex Inferior
iliac artery epigastric artery Adductor canal
Above-knee
Inguinal popliteal artery
ligament
Common Popliteal artery Superior lateral
femoral artery genicular artery
Superficial femoral
artery Sural artery Inferior lateral
genicular artery
Inferior medial Proximal anterior
Popliteal artery genicular artery tibial artery

Tibioperoneal trunk Tibioperoneal Peroneal artery

trunk
Posterior tibial
Posterior tibial artery artery
Anterior tibial
artery Peroneal artery

Fig. 19.21 Diagrams of lower extremity arteries. (a) Anterior view of the right lower extremity arteries. (b) Posterior view of the right lower
extremity arteries

(SFA) and the deep femoral (profunda femoris) artery. The
SFA extends along the medial aspect of the thigh in parallel
with the femoral vein (FV) to the adductor canal. Below the
adductor canal, the popliteal artery runs posterior to the knee,
supplying branches to the calf. The deep femoral artery gives
off perforating branches, as well as the medial and lateral
circumflex arteries that supply the femoral head and the deep
muscles of the thigh.
The popliteal artery divides into the tibioperoneal trunk
and the anterior tibial artery. The tibioperoneal trunk
branches into the posterior tibial and peroneal arteries. At the
level of the ankle, the posterior tibial artery divides into the
medial and lateral plantar arteries.
The anterior tibial artery courses laterally, extending
through the interosseous membrane between the tibia and
fibula, and into the anterior compartment of the lower leg.
The anterior tibial artery continues as the dorsalis pedis
artery. The dorsalis pedis artery joins the lateral plantar
artery to form the plantar arch. The peroneal artery passes
through the interosseous membrane above the ankle to sup-
ply small arterial branches to the lateral ankle and foot.
Anatomic Variants
Upper limb arterial variants are relatively rare. The most
common is a high origin of the radial artery. Other variations
include a superficial position of the brachial, ulnar, and radial
arteries, and accessory brachial arteries [47].
The foot usually receives most of its arterial supply from
branches of the anterior and posterior tibial arteries. A fre-
quent variation of this pattern consists of a dominant pero-
neal artery with a hypoplastic or aplastic posterior tibial artery
or a hypoplastic anterior tibial artery. Peronea arteria magna
is a variant where both the anterior and posterior tibial arter-
ies are hypoplastic and a large, dominant peroneal artery
supplies the entire leg and foot [48].
Stenosis and Thrombosis
Symptoms of acute ischemia include limb cyanosis and pulse-
lessness, while patients with chronic disorders may present
with exercise-induced discomfort, claudication, excessive
19 Vascular Imaging
exertional fatigue, and weakness. In long-standing cases, there
may be a limb-length discrepancy, loss of extremity muscle
mass, and a small foot [49, 50].
Evaluation for stenosis is performed with color and spec-
tral Doppler imaging to document peak systolic and end-­
diastolic flow velocity and Doppler waveform morphology.
Any zones of focal narrowing identified on grayscale imaging
require confirmation with color Doppler. The presence of tur-
bulent color Doppler signal should be further characterized
with spectral Doppler.
When a change in spectral waveform morphology is noted
in an arterial segment, additional proximal and distal evalua-
tion with color and spectral Doppler should be performed to
identify the transition points.
Arterial stenosis is diagnosed by analyzing waveform mor-
phology, PSV, and EDV. Both absolute PSV values and the peak
velocity ratio (defined as peak velocity at or in the downstream
jet divided by peak velocity of the artery 2 cm upstream) are
assessed. A focal increase in the PSV ratio at the stenosis rela-
tive to the adjacent non-stenotic artery exceeding 2.0 along with
spectral broadening and loss of transient flow reversal in the
artery is consistent with at least 50% diameter stenosis [51].
The spectral Doppler waveform distal to a stenosis of
greater than 50% will be abnormal with a tardus-parvus pat-
tern [49]. For the femoral and popliteal arteries, a combina-
tion of a peak systolic velocity over 200 cm/sec and a peak
velocity ratio greater than 2:1 has identified focal stenoses
greater than 70% with a sensitivity of 79% and a specificity
of 99% (Fig. 19.22) [52].
a b
Fig. 19.22 Stenosis with focal occlusion of the right common femoral
artery (CFA) in a 10-year-old male with remote history of right groin
catheterization. (a) Sagittal color Doppler ultrasound image of the CFA
with spectral analysis shows irregular mural thickening (arrowheads)
with luminal narrowing. Peak systolic velocity measures 370 cm/sec.
915
Peripheral arterial thrombosis is rare in children. Most
cases are due to complications related to arterial catheteriza-
tion. Non-catheter-related cases are unusual. Early diagnosis
and rapid institution of appropriate treatment are vital in
order to avoid limb loss [53].
On grayscale imaging, the thrombosed arterial lumen is
usually filled with echogenic thrombus. External pressure on
the vessel by the transducer can be used to show non-­
compressibility. On color and spectral Doppler of an occluded
artery, no flow signal should be detectable. The presence of
collateral vessels suggests chronic occlusion (Table 19.1).
Acute thrombosis superimposed on chronic thrombosis
can also occur. Use of color and spectral Doppler is highly
sensitive and specific for demonstrating the absence of flow
and can distinguish stenosis from occlusion in the lower extrem-
ity with close to 100% accuracy [54]. The sensitivity for
detecting occlusion of the SFA and popliteal artery is also
high [55].
Detection of occlusion in the lower leg is better for the
anterior and posterior tibial arteries than for the peroneal
artery. A recent study in a group of adult patients revealed a
sensitivity of 93% for patency of the tibial artery. However,
there were many false positives as documented at angiogra-
phy [56]. Similar data is not currently available for children.
Infants and children with acute limb ischemia are usually
treated with anticoagulation. Patients who do not respond to
anticoagulation may require systemic thrombolysis or surgical
thrombectomy and arterial repair [50, 57, 58]. Management
strategies for pediatric patients with chronic ischemia are not
c
More distally the artery is occluded (arrow). (b) Sagittal color Doppler
ultrasound image of the CFA distal to the stenosis shows adjacent col-
lateral vessels (arrows). (c) Spectral analysis reveals an abnormal par-
vus-tardus waveform distally in the SFA with peak systolic velocity less
than 100 cm/sec
916
Table 19.1 Ultrasound imaging features of arterial thrombosis
Technique Ultrasound findings
Grayscale imaging Arterial lumen contains echogenic thrombus
Doppler imaging No flow at site of thrombosis
Abnormal flow distal to thrombus
Flow in collateral vessels with chronic occlusion
well-established. Extremity revascularization procedures have
been successfully performed in symptomatic children [49].
Aneurysm
Aneurysms of the peripheral arteries are extremely rare in
children. An underlying condition is often present, such as
infection, trauma, connective tissue disorder, noninfectious
arteritis, and congenital vascular malformation [59]. Infected
(mycotic) aneurysms can develop by direct inoculation of bac-
teria into the arterial wall at the time of vascular injury. They
are frequently seen in IV drug abusers who inadvertently inoc-
ulate themselves with contaminated needles that enter an arte-
rial wall when they are aiming for an adjacent vein.
Peripheral arterial aneurysms can thrombose and lead to
distal emboli and associated soft tissue ischemia or necrosis.
Children with upper extremity aneurysms may present with a
mass at the site of the involved artery or with paresthesias [59].
As previously described, the appearance of an aneurysm
on grayscale and color Doppler imaging will vary according
to the size of the aneurysmal neck, the presence of intralumi-
nal clot, and mural calcification.
Larger and symptomatic aneurysms should be repaired.
Treatment of upper extremity aneurysms usually includes
resection with arterial reconstruction using reversed saphenous
vein interposition grafts [59]. Lower extremity aneurysms are
treated on a case-by-case basis and may require excision, endo-
vascular embolization, or bypass grafting [59, 60]. Doppler US
can be used to monitor the vessel after treatment.
Infected aneurysms have significant morbidity and mortal-
ity. Treatment consists of a combination of antibiotic therapy,
aggressive surgical debridement of infected tissue, and vascu-
lar reconstruction, as needed. Endovascular therapies can be
used for the treatment of ruptured infected aneurysm and in
patients at great risk for open surgery [61, 62].
Pseudoaneurysm
A pseudoaneurysm is a focal disruption of the wall of an artery
that develops as a result of direct trauma or erosion by inflam-
mation or tumor. Blood flows beyond the confines of the ves-
sel wall, and a hematoma develops next to the artery at the site
H. J. Paltiel
of injury that is contained by the surrounding tissues. Once
clot lysis has occurred, a pseudoaneurysm remains. A pseu-
doaneurysm differs from an aneurysm in that the arterial wall
is disrupted. Blood within the pseudoaneurysm flows back
into the feeding artery through a narrow opening. The outer
wall of the pseudoaneurysm consists of compressed thrombus
and the surrounding soft tissues.
Pseudoaneurysms typically present as a painful, pulsatile
mass. They may exert pressure on the overlying skin that
results in ischemia, necrosis, and hemorrhage. Clot that forms
within the pseudoaneurysm can lead to distal embolization.
Grayscale ultrasound will show a round or oval bulge
along the course of the artery that may contain thrombus.
Color Doppler evaluation is performed to detect flow within
the lumen of the pseudoaneurysm. When flow is identified, it
often has a swirling, “yin-yang” pattern. Flow through the
neck of the pseudoaneurysm is typically biphasic with a “to-
and-fro” pattern on spectral Doppler (Figs. 19.23, 19.24;
Cineclip 19.3) [63].
Surgical repair is indicated in patients with pseudoaneu-
rysm due to prior vascular surgical intervention with suture
line disruption, infection, or non-iatrogenic trauma. Other
indications include skin or soft tissue ischemia, associated
arteriovenous fistula (AVF), and injury to the vessel above
the inguinal ligament. Urgent repair is recommended for any
patient with rupture, an expanding pulsatile mass, or com-
pression of surrounding structures causing claudication, neu-
ropathy, or critical limb ischemia.
Femoral artery pseudoaneurysm can be managed with
ultrasound-guided compression, ultrasound-guided throm-
bin injection, or surgery. Endovascular treatments, including
coils, fibrin glue, and stent graft exclusion, have met with
varying degrees of success [64].
Arteriovenous Fistula
An AVF is an abnormal vascular communication between a
high-pressure artery and a low-pressure vein that bypasses
the capillary bed. It can be congenital or acquired as a result
of a traumatic injury. Pain commonly occurs when the AVF
is located on a weight-bearing surface or when a large lesion
ulcerates. Increased blood flow through an extremity fistula
can increase oxygen delivery to the tissues that leads to a
growth discrepancy between the normal and affected limbs.
Cardiac output can also be increased and congestive heart
failure may occur; this happens more often with acquired fis-
tulae than with congenital lesions [65].
Femoral AVFs can develop when a femoral venous punc-
ture is performed below the CFA, where several superficial
branches of the femoral artery overlie the femoral vein or
19 Vascular Imaging
a c
Fig. 19.23 Pseudoaneurysm of the right axillary artery in a 3-year-old
male that developed as a complication of catheterization. (a) Sagittal
grayscale ultrasound image of the right axillary artery shows a rounded
heterogeneous collection (arrow) adjacent to the axillary artery (A). (b)
Fig. 19.24 Pseudoaneurysm of the left axillary artery in a 10-month-­old
male with a history of prior catheterization shows a typical “yin-­yang”
color Doppler pattern within the lumen of the pseudoaneurysm
917
Sagittal color Doppler ultrasound image shows a neck (arrowhead) extend-
ing from the axillary artery into the collection. (c) Spectral Doppler analysis
of the aneurysmal neck shows to-and-fro flow
when arterial and venous punctures are performed on the
same side. A diagnosis of an AVF is made on physical exami-
nation with a finding of a continuous “to-and-­fro” bruit that
can be confirmed by ultrasound.
AVFs are rarely evident on grayscale imaging and are best
depicted on color Doppler imaging (Fig. 19.25). Color Doppler
demonstrates a direct communication between an artery and
vein, turbulent flow in the region of the fistula, and perivascular
soft tissue vibrations. Spectral Doppler analysis of the artery
at the site of the fistulous connection shows a low-resistance
pattern with high diastolic flow that contrasts with the typi-
cally high-resistance pattern of normal peripheral arteries. The
draining vein can show an arterialized waveform [66].
Many of the iatrogenic AVFs are small and close sponta-
neously within 1 year. Ultrasound-guided compression or
surgical repair can occasionally be necessary. Because car-
diac volume overload and limb damage do not often develop
with persistent AVFs, conservative management for at least
1 year is usual. Arterial embolization is the most effective
treatment for high-flow lesions, with subsequent surgical
resection occasionally being necessary [67]. The distinguish-
ing clinical and ultrasound features of arterial aneurysm,
pseudoaneurysm, and AVF are described in Table 19.2.
918 H. J. Paltiel

a b c

A V

Fig. 19.25 Post-traumatic arteriovenous fistula (AVF) in a 3-year-old direct connection (arrowhead) is visible between the two vessels. (b) Color
male with a history of inadvertent placement of a PICC in the left popli- Doppler ultrasound image shows flow through the AFV with prominent
teal artery. (a) Transverse grayscale ultrasound image of the left popli- soft tissue vibration artifact (arrows). (c) Disordered flow is depicted at the
teal vessels shows marked dilation of both the artery (A) and vein (V). A site of the fistula with spectral Doppler

Table 19.2 Clinical and ultrasound features of arterial aneurysm, pseudoaneurysm, and arteriovenous fistula
Aneurysm
Clinical Underlying condition often present (e.g.,
features infection, trauma, connective tissue
disorder, IV drug abuse)
Popliteal artery common site
Pathology Congenital or acquired weakness or
destruction of medial layer of vessel
wall
Ultrasound Focal or diffuse arterial dilation best seen
findings on transverse images
Variable color/spectral Doppler signal
depends on amount of thrombus, size of
aneurysmal neck, and presence of
calcification
Pseudoaneurysm
Develops after trauma or erosion by
inflammation or tumor
Focal disruption of arterial wall with
hematoma at injury site contained
by surrounding tissues
Round or oval anechoic structure
with or without central thrombus
Color/spectral Doppler shows
disorganized internal flow with
“yin-yang” pattern
“To-and-fro” flow in neck
communicating with adjacent artery
Arteriovenous (AV) fistula
Congenital or acquired, usually from trauma
Iatrogenic lesions may resolve
spontaneously
Communication between normal artery and
vein in post-traumatic lesions
Intrinsically abnormal vessels in setting of
arteriovenous malformations
Traumatic fistulas have few grayscale
findings. AV malformations may show
dilated vessels
Color/spectral Doppler demonstrates
low-resistance arterial flow, arterialized
venous flow, and soft tissue vibration
artifact

Extremity Veins Ultrasound Transducer Selection

Technique
Patient Positioning
Patient positioning for examination of the upper and lower
extremity veins is generally the same as that previously
described for the upper and lower extremity arteries. The
popliteal vein is examined with the patient prone and a pil-
low placed under the ankle to slightly flex the knee or with
the patient in a decubitus position (with the side to be evalu-
ated positioned uppermost). The posterior tibial and peroneal
veins are imaged using a medial or posteromedial approach.
The anterior tibial veins are imaged from an anterolateral
approach.
The highest-frequency transducer that permits adequate pene-
tration and visualization should be used, as described above for
the upper and lower extremity arteries, typically a 5–10 MHz
linear array transducer.
When imaging the upper extremities, a small footprint,
high-frequency transducer can be placed in the suprasternal
notch to visualize the junction of the brachiocephalic veins
(BCVs) with the SVC (Fig. 19.26), although in practice imag-
ing of this confluence is often difficult to achieve because of
overlying lung tissue.
In the lower extremities, the superficial great saphenous
veins are easily visualized by ultrasound in most patients. The
deep veins in the proximal and mid-thigh are more difficult to
image because of the thickness of the overlying muscles. Use
19 Vascular Imaging
L ing images for each deep venous segment as recommended by
R
V practice parameter for the performance of peripheral venous
Fig. 19.26 Normal confluence of the brachiocephalic veins (BCVs) with
the SVC in a 16-year-old female. Coronal color Doppler ultrasound image.
R, Right brachiocephalic vein; L, left brachiocephalic vein; V, SVC
of a lower-frequency curved array or sector transducer may
improve visualization of the deep venous system.
Imaging Approaches
Assessment of the upper extremity veins includes grayscale
compression and color and spectral Doppler imaging of the
visualized portions of the IJV; subclavian, axillary, and BCVs
and any focal symptomatic areas (Fig. 19.27).
All veins are evaluated with compression at 1–2 cm inter-
vals in the transverse plane. Venous c­ ompression should be
applied to accessible veins in the transverse plane with suf-
ficient pressure to completely collapse the vessel lumen. An
attempt should be made to visualize the brachial, cephalic,
and basilic veins in the upper arm to the elbow with color
Doppler, and any focal symptomatic areas should be evalu-
ated further with compression [44].
Grayscale images with and without compression or cine
clips obtained with compression should be acquired from the
superior aspect of the IJV in the neck to the thoracic inlet.
The SCV is evaluated from its medial to lateral aspect with
longitudinal color and spectral Doppler images and reviewed
for evidence of luminal patency, transmitted cardiac pulsa-
919
tions, and respiratory variation. An inferiorly angled, supra-
clavicular approach is used to document the superior BCV
and the medial portion of the SCV. An infraclavicular, supe-
riorly angled approach can be used to demonstrate the lateral
aspect of the SCV. In many cases the SCV can be com-
pressed. The midportion of the SCV is often incompletely
imaged as it is located deep to the clavicle.
Spectral Doppler images are obtained from longitudinal
images of the vessel with an insonation angle of 60°or less.
A normal Doppler waveform will always return to the base-
line. The absence of pulsatility raises concern for more cen-
tral stenosis or obstruction [68].
Evaluation of the lower extremity veins includes the follow-
the 2019 American College of Radiology (ACR)-American
Institute of Ultrasound in Medicine (AIUM)-Society for Pediatric
Radiology (SPR)-Society of Radiologists in Ultrasound (SRU)
ultrasound examinations [44]: imaging is performed from the
inguinal ligament to the ankle whenever possible. Venous com-
pression is applied every 2 cm or less in the transverse plane
with sufficient pressure on the skin to completely obliterate the
normal venous lumen [69].
The common femoral, femoral (formerly known as the
superficial femoral) [70], popliteal, posterior tibial, and pero-
neal veins are evaluated. The deep (profunda) femoral vein (FV)
should also be examined at its confluence with the FV. The great
saphenous vein is imaged at the saphenofemoral junction. Focal
symptoms may require additional imaging (Fig. 19.28).
All studies, whether unilateral or bilateral, should include
right and left common femoral or right and left external iliac
venous spectral Doppler waveforms. All spectral Doppler
waveforms should be obtained from the long axis of the ves-
sel. Tracings should be compared for asymmetry and/or loss
of respiratory phasicity [71]. Both sides should be assessed
with similar patient positioning and similar phase of respira-
tion so that symmetry can be assessed.
Popliteal venous spectral Doppler waveforms of the
symptomatic leg should also be obtained. Routine spectral
Doppler distal augmentation is not necessary for the diagno-
sis of DVT [72]. However, in challenging situations, color
Doppler imaging with distal augmentation can aid in identi-
fication of vessels and in differentiating complete from
incomplete occlusion [73].
When studies are normal, static images or cine loops are
recorded at selected sites as a representative subset of the images
acquired during a comprehensive study as described below:
1. Grayscale images (or cine loops) should be recorded
without and with compression at each of the following
levels whenever possible: common femoral vein (CFV);
con­­fluence of the CFV with the great saphenous vein;
deep (profunda) FV at the confluence with the FV; FV in
the upper, mid, and distal portions of the thigh; and pop-
liteal vein.
920
a b
d e f g
h i
Fig. 19.27 Normal left upper extremity veins in an 11-year-old
female. Transverse grayscale ultrasound images of the (a) IJV, (b)
basilic vein (arrow) and paired brachial veins (v) and (c) cephalic vein
imaged with light transducer pressure (left panels) and with firm trans-
ducer pressure (right panels). Sagittal color Doppler ultrasound images
with normal spectral waveforms are shown for the BCV, SCV, and axil-
2. Representative images or cine loops without and with com-
pression of the posterior tibial and peroneal veins.
3. Color and spectral Doppler waveforms should be obta­
ined from the long axis of each of the following vessels:
right common femoral or external iliac vein; left common
femoral or external iliac vein; and popliteal vein on the
symptomatic side or on both sides if the examination is
bilateral.
H. J. Paltiel
c
j
lary veins (d–f). (g) Sagittal color Doppler ultrasound image of normal
paired brachial veins (arrowheads) on either side of the brachial artery
(asterisk). (h) Sagittal color Doppler ultrasound image of a brachial
vein with a normal spectral waveform. Sagittal color Doppler ultra-
sound images with spectral analysis of the cephalic (i) and basilic (j)
veins depict normal waveforms
Abnormal symptoms or findings generally require addi-
tional images for complete documentation. Protocol adjust-
ments may be necessary depending on patient presentation,
clinical indication, or anticipated treatment options (e.g., a
bilateral study or more detailed assessment of the superficial
venous system) [74–76].
When a thrombus is identified in the CFV, its most proxi-
mal extent should be demonstrated by including the external
19 Vascular Imaging
a b
A
A V
A
d e
h i
Fig. 19.28 Normal right lower right extremity veins in an 11-year-old
female. Transverse grayscale ultrasound images obtained with light trans-
ducer pressure (left panels) and firm transducer pressure (right panels)
depict (a) the confluence of the common femoral vein (CFV) and great
saphenous vein (arrow); V, CFV; A, CFA; (b) FV (arrowhead); A,
Superficial femoral artery; and (c) calf veins (arrow). Sagittal color Doppler
921
c
A
f g
ultrasound images with spectral analysis of the CFV (d), deep FV (e), FV (f),
and popliteal vein (g). There is increased flow with augmentation maneuvers
(arrowheads) on images (d), (f), and (g). (h) Sagittal color Doppler ultra-
sound image of the posterior tibial (arrow) and peroneal (arrowhead) calf
vessels. (i) Sagittal color Doppler ultrasound image shows the paired pero-
neal veins on either side of the peroneal artery (arrow)
922 H. J. Paltiel

Table 19.3 Ultrasound imaging features of deep vein thrombosis

(DVT) Internal
Subclavian vein jugular vein
Category Ultrasound findings
Acute DVT Enlarged vein
Lack of compressibility Cephalic vein Brachiocephalic
vein
Thrombus in vessel lumen
No Doppler flow at site of thrombus Clavicle
Loss of flow pulsatility distal to thrombus
Axillary vein
No flow augmentation Brachial veins
No response to Valsalva maneuver
Chronic (residual) DVT Thick or irregular vessel wall Basilic vein
Narrow or irregular lumen Median cubital vein
Thrombus broadly attached to vessel wall Basilic vein
Web-like filaments in vessel lumen Cephalic vein

Ulnar vein
iliac veins and inferior vena cava (IVC) in the evaluation.
Any additional musculoskeletal findings in the imaging field Radial vein
Median antebrachial
vein
should be documented, such as a hematoma, knee joint effu- Deep palmar arch
sion, or popliteal cyst. The ultrasound imaging features of
acute and chronic DVT are summarized in Table 19.3. Superficial palmar
arch

Digital veins

Normal Development and Anatomy

Upper Extremity Fig. 19.29 Diagram of normal upper extremity venous anatomy

Normal Development Lower Extremity

In the embryo, the upper limb buds are drained by marginal
veins. The pre-axial (i.e., radial) and post-axial (i.e., ulnar) por-
tions of the marginal vein develop into the superficial cephalic
and basilic veins, respectively. The deep veins develop along-
side the corresponding upper limb arteries.
Normal Anatomy
The venous drainage of the upper extremity is comprised of
a deep and superficial system, both of which ultimately drain
into the axillary vein (Fig. 19.29) [77].
The deep veins of the hand drain to the paired ulnar and radial
veins of the forearm that join distal to the elbow to form the
paired brachial veins. The brachial veins extend superiorly along
the medial aspect of the arm to join the basilic vein and form the
axillary vein, usually at the level of the teres major muscle.
The axillary vein courses through the axilla to the level of
the first rib. As it extends beneath the first rib, it becomes the
SCV. The medial portion of the SCV joins with the internal
and external jugular veins to form the brachiocephalic (innom-
inate) vein.
The superficial veins arise on the dorsum of the hand. They
drain into the ulnar, radial, and median forearm veins at variable
levels. These veins unite to form the medially located basilic
vein and the laterally located cephalic vein. The basilic vein usu-
ally drains into the brachial veins in the upper arm. The cephalic
vein extends along the superficial soft tissues of the shoulder
and drains into the axillary vein in the lateral aspect of the upper
chest.
The venous drainage of the lower extremity is comprised of
deep and superficial systems, both of which drain into the
CFV (Fig. 19.30) [78, 79].
Normal Development
In the embryo, the lower extremity venous system appears
between weeks 5 and 6 of life. The superficial veins develop
first and include a primitive marginal vein and the small
saphenous vein. Between weeks 7 and 8, three venous plex-
uses mature, including the posterior tibial veins, the great
saphenous vein, and the FV.
Early venous outflow from the primitive lower limb occurs
through a lateral/posterior fibular (peroneal) vein. The primi-
tive fibular vein develops two branches: the anterior tibial vein
and a connecting vessel. The anterior tibial vein becomes the
main deep draining vein of the calf. The anterior tibial vein
and primitive fibular veins together constitute the sciatic vein.
A portion of the primitive fibular vein distal to the anterior
tibial vein branch develops into the short or lesser saphenous
vein. The connecting vessel grows medially from the middle
of the sciatic vein and connects to a proximal medial vessel
that will become the FV and the deep venous system, while
the sciatic vein regresses. The FV extends down the leg as
the posterior tibial vein [80].
Normal Anatomy
The deep veins of the foot drain into the anterior and pos-
terior tibial veins. The posterior tibial vein joins the pero-
19 Vascular Imaging
External iliac
vein
Common femoral
vein
Deep femoral vein
Femoral vein
Popliteal segment
of femoral vein
Anterior tibial vein
Peroneal vein
Posterior tibial
vein
Plantar metatarsal
vein
Fig. 19.30 Diagram of normal lower extremity venous anatomy
neal vein(s) (which are usually paired) before anastomosing
with the anterior tibial vein to form the popliteal vein. The
popliteal vein continues superiorly as the FV after it leaves
the adductor canal in the distal thigh. The FV ascends to
join the deep (profunda) FV just below the inguinal liga-
ment, where it becomes the CFV. The CFV becomes the
external iliac vein above the inguinal ligament.
The superficial venous system includes the small (short)
and great saphenous veins (Fig. 19.31). The small saphe-
nous vein drains the soft tissues overlying the medial mal-
leolus and then ascends posteriorly to anastomose with the
popliteal vein. The great saphenous vein drains the medial
calf and then ascends to join the CFV in the proximal thigh
below the inguinal ligament. The deep and superficial veins
are connected at different levels in the calf by perforating
veins that drain from the skin to the deep veins.
923
Anatomic Variants
FV duplication is identified in approximately 30% of patients
and can be complete or segmental [81]. In about 40% of
patients where multiple vessels are identified in the popliteal
fossa, the cause is a high confluence of the posterior tibial
and peroneal veins, rather than a true duplication of the pop-
liteal vein [82, 83]. It is important to be aware of the possibil-
ity of venous duplication when evaluating patients for deep
vein thrombosis in order to avoid a false negative interpreta-
tion (Fig. 19.32) [84].
Thrombosis
DVTs in children usually occurs as a complication of an
underlying disease, such as sepsis, cancer, and congenital
heart disease, or as a complication of medical, surgical, or
therapeutic intervention. Other common causes include
immobilization, hypercoagulability, and oral contraceptives
[85–87]. Patients with peripheral DVT will present with
pain, swelling, and erythema of the affected limb. DVT of
the lower extremities is much more common in children than
upper extremity DVT. Apart from central venous catheter-
associated venous thrombosis, upper extremity DVT is rare
in children.
Clinical evaluation of the peripheral venous system is
often difficult and frequently inaccurate. Clinical decision
rules to improve pretest probability have been recommended
by the American College of Physicians and the American
Academy of Family Physicians [88–90]. The Wells criteria
generate a score for certain physical examination findings
and pertinent clinical history [91].
Clinical factors associated with increased probability of
DVT include immobilization, active cancer, a swollen extrem-
ity, localized tenderness along the course of the deep venous
system, pitting edema localized to the symptomatic extrem-
ity, collateral superficial veins, and previously documented
DVT. The modified Wells score separates patients into two
groups: DVT unlikely and DVT likely [92].
Current guidelines recommend a D-dimer test for low-
risk patients [93, 94]. The D-dimer test measures a degrada-
tion product of fibrin and has a high negative predictive value
for DVT that is sensitive but not specific. A positive test may
be caused by low levels of fibrin associated with inflamma-
tion, infection, vasculitis, or trauma [95]. When the D-dimer
test result is positive, the patient should undergo a venous
Doppler examination of the symptomatic limb. A negative
D-dimer test effectively rules out the possibility of DVT.
Acute Deep Vein Thrombosis
The ultrasound features of acute DVT include an enlarged
vein with lack of compressibility, visualization of thrombus
924 H. J. Paltiel

Fig. 19.31 Diagram of lower extremity superficial venous drainage. (© Catherine Delphia 2020)

Fig. 19.32 Acute deep vein thrombosis (DVT) in a 14-year-old male duplication with the medial vein (asterisks) distended by thrombus and
carrier for factor V Leiden deficiency with FV duplication. (a) Transverse patency (arrowheads) of the lateral vein. A, femoral artery. (c) Spectral
and (b) sagittal color Doppler ultrasound images of the right FV reveal Doppler analysis shows normal flow in the patent lateral FV
19 Vascular Imaging 925

within the vessel lumen, absence of flow on Doppler imaging
at the site of thrombosis, loss of the normal cardiac- and
respiratory-related pulsations distal to the site of thrombosis,
and lack of flow augmentation or response to the Valsalva
maneuver [78, 79].
On grayscale imaging, an intraluminal thrombus can
sometimes appear anechoic. Flow will be present in veins
with nonocclusive thrombi, and compression and augmen-
tation maneuvers may be normal. Despite these potential
pitfalls, grayscale imaging combined with compression
maneuvers and color Doppler evaluation should permit a
diagnosis of thrombosis in most cases (Fig. 19.33).
In the upper extremities, the sensitivity and specificity
of color flow Doppler imaging for diagnosing DVT range
from 78% to 100% and 82% to 100%, respectively [96].
The accuracy of compression ultrasound for lower extrem-
ity DVT has been shown to reach 95% with 98% specific-
ity, while studies of color Doppler imaging report a
sensitivity of 95%, specificity of 99%, and accuracy of
98% [79]. Detection rates of lower extremity acute venous

a b

c d

Fig. 19.33 Acute near-occlusive right upper extremity DVT in a 14-year- depicted within the upper BCV. (c) Coronal color Doppler ultrasound image
old male. (a) Coronal color Doppler ultrasound image shows an echogenic shows a small amount of flow (arrowhead) in the SCV lateral to the throm-
thrombus (asterisk) in the medial SCV with flow identified in the BCV bus (asterisk). (d) Spectral Doppler ultrasound evaluation reveals sluggish,
(arrowhead). (b) A normal spectral Doppler ultrasound waveform is dampened flow in the SCV lateral to the thrombus
926
thrombosis are greater for thrombosis above the knee than
for thrombosis in the calf since the calf veins are signifi-
cantly smaller and it is difficult to be certain that all of the
calf vessels (some of which may be duplicated) are free of
thrombus [79].
Treatment goals for venous thromboembolism include
resolution of the existing thrombus, prevention of local
thrombus extension, embolization, recurrence, and minimi-
zation of long-term complications such as post-thrombotic
syndrome. The most common treatments for venous throm-
boembolism in children include unfractionated heparin, low
molecular weight heparin, and warfarin. Other options
include fondaparinux, a factor Xa inhibitor, and the direct
thrombin inhibitors [97].
a c
Fig. 19.34 Chronic left upper extremity DVT in a 16-year-old female with
a PICC line. (a) Sagittal grayscale ultrasound image of the left SCV-axillary
venous confluence shows a thickened, echogenic PICC (asterisk) sur-
rounded by hypoechoic thrombus (arrowheads). (b) Transverse grayscale
ultrasound images of the axillary vein without (left panel) and with (right
H. J. Paltiel
For infants and children with major vessel occlusion caus-
ing compromise of organs or limbs, systemic or catheter-
directed thrombolytic therapy may be warranted. Data on the
efficacy, dose, and safety of thrombolytic agents in this set-
ting are limited, and indications for thrombolytic therapy are
highly individualized [98].
 hronic (Residual) Deep Vein Thrombosis
C
Distinguishing acute from residual or chronic DVT by both
clinical and imaging criteria may be challenging. Both acute
and chronic DVT may show non-compressibility of the
involved vessel. Chronic venous thrombosis is characterized
by thickened or irregular walls, a narrowed or irregular lumen,
and collateral vessel formation (Fig. 19.34). The clot is usually
d
panel) compression reveal no collapsibility. The centrally located PICC
(arrows) is surrounded by hypoechoic thrombus (arrowheads). (c) Sagittal
color Doppler ultrasound image of the lateral subclavian vein demonstrates
multiple collateral vessels (arrowheads). Asterisk, Thrombus. (d) Spectral
Doppler evaluation of a collateral vessel depicts venous-type flow
19 Vascular Imaging
hypoechoic. Residual thrombus will become broadly adherent
to the vessel wall. There may be intraluminal echogenic, web-
like filaments within the vein [79]. Differentiation between
hypoechoic thrombus and normal-­flowing blood often requires
further evaluation with color and spectral Doppler imaging.
There are limited data regarding the management of
chronic DVT. However, there are reports of aggressive treat-
ment in some patients to encourage symptom resolution
[99]. Recanalization of occluded veins and venous stenting
have been able to re-establish deep vein flow and decrease
venous hypertension.
Retroperitoneal Vessels
Technique
Patient Positioning
Ultrasound imaging of the retroperitoneal vessels is per-
formed with the patient supine as well as in the right and left
decubitus positions to optimize visualization of the inferior
portions of the abdominal aorta and IVC.
Ultrasound Transducer Selection
The highest-frequency transducer that permits adequate pen-
etration of the abdomen should be used.
Imaging Approaches
Ultrasound imaging is optimally performed after patient
fasting so that air in the bowel is minimized. Grayscale
images of the upper portions of the abdominal aorta and IVC
are best obtained via an anterior midline approach. The infe-
rior portions of these vessels as well as their bifurcation are
often better depicted via a coronal approach through the
flanks. Images should be obtained in both transverse and
­longitudinal planes. Color and spectral Doppler images are
ideally acquired from an oblique or coronal scan plane.
Angle-corrected velocity measurements should be obtained
from the center of a longitudinal image of the aorta or IVC.
Aorta
Normal Development and Anatomy
Normal Development
The development of the aorta begins in the third week of life
when two strands of cells migrate dorsally from the endocar-
dial mesenchyme and extend caudally along the neural
groove to form the dorsal aortas. After about 1 week, the two
aortas fuse into a single aortic trunk that descends caudally.
The right dorsal aorta will become the right subclavian
artery. The left aorta develops into the descending thoracic
927
aorta. Multiple segmental arteries arise from the dorsal aor-
tas that join together to form successive segments. As the
embryo develops, most of these segmental arteries will
regress, except for the precursors to the three major mesen-
teric vessels. The 10th segmental artery will become the
celiac artery that supplies the foregut, the 13th segmental
artery will develop into the superior mesenteric artery (SMA)
that supplies the midgut, and the 22nd segmental artery will
form the inferior mesenteric artery (IMA) that supplies the
hindgut [100].
Normal Anatomy
The mature abdominal aorta extends inferiorly from the dia-
phragmatic hiatus, anterior and slightly to the left of the lumbar
vertebral bodies (Figs. 19.35 and 19.36). It divides into the
right and left common iliac arteries at the level of the fourth
lumbar vertebra. On grayscale imaging, the aorta has echo-
genic walls and an anechoic lumen and is pulsatile on real-time
imaging. Spectral Doppler analysis shows a high-­resistance,
triphasic waveform with a rapid systolic upstroke, a prompt
decline in velocity, and brief flow reversal in early diastole fol-
lowed by low-velocity antegrade flow in late systole (Fig. 19.37)
[8, 101]. The arterial branches of the aorta supplying the solid
abdominal viscera have a biphasic, l­ow-­resistance arterial
waveform with a rapid systolic upstroke followed by continu-
ous antegrade flow through systole and diastole.
Thrombosis
Aortic thrombosis occurs most often in the neonatal period
as a complication of umbilical artery catheterization [102].
Other predisposing causes include cardiac and pulmonary
abnormalities, sepsis, peripartum asphyxia, fetal meconium
aspiration, extreme prematurity, hypertension, and the pres-
ence of a prothrombotic condition such as factor V Leiden
mutation. Aortic thrombosis is frequently asymptomatic and
detected incidentally by US. Patients may present with signs
of lower extremity ischemia, hematuria, or hypertension if
thrombosis involves the renal arteries. Long-term complica-
tions include impaired lower extremity growth and renovas-
cular hypertension.
Ultrasound imaging of infants with suspected aortic
thrombosis should assess the entire abdominal aorta and
both kidneys with grayscale, color, and spectral Doppler.
Ultrasound can reliably localize the position of an intra-­
abdominal umbilical arterial catheter. When an umbilical
catheter is present, it is depicted as an echogenic linear struc-
ture in the lumen of the aorta (Fig. 19.38). The optimal posi-
tion of the catheter tip is either in the abdominal aorta below
the origin of the renal arteries or in the thoracic aorta below
the vessels of the aortic arch.
An acute thrombus will manifest as an echogenic mass
within the aortic lumen. Color Doppler is useful in determin-
ing whether the thrombus is partially or completely occlusive
928 H. J. Paltiel

Internal thoracic
Aortic arch
Parietal (somatic) Visceral branches
branches of of the thoracic aorta
thoracic aorta Bronchial
Intercostal Esophageal
Superior phrenic Mediastinal
Pericardial
Inferior phrenic Thoracic aorta
Diaphragm Aortic hiatus
Celiac trunk
Adrenal
Left gastric
Renal Splenic
Common Superior
hepatic mesenteric
Gonadal Abdominal aorta
Lumbar Inferior
Median sacral mesenteric
External iliac
Common iliac
Internal iliac

Fig. 19.35 Diagram of the aorta and its major intrathoracic and abdominal branches

resolving thrombus will decrease in size and ultimately appear

as a thin, linear intraluminal structure.
Treatment for neonatal aortic thrombosis consists of
removal of the umbilical artery catheter (if present) and ther-
apeutic anticoagulation. In patients with clinical evidence of
ischemia but without imminent tissue loss or visceral injury,
therapeutic anticoagulation with or without systemic throm-
bolysis is recommended, based on anatomic imaging find-
ings and clinical concern for clot propagation. Constant
reassessment is mandatory in order to avoid delays in inter-
vention for those with clinical progression. Patients with
clinical evidence of ischemia with imminent tissue loss or
end-organ dysfunction require immediate therapeutic antico-
agulation and surgical thrombectomy [103]. Doppler sonog-
raphy can be used to document resolution of the thrombus.

Stenosis
Fig. 19.36 Normal abdominal aorta in a 9-day-old male. Sagittal color
Doppler ultrasound image of the abdominal aorta from the level of the
diaphragm to the bifurcation into the common iliac arteries (arrowheads)
and aids in the detection of collateral vessels. In contrast to
the sharp systolic upstroke and triphasic high-resistance flow
of the normal aorta, spectral Doppler evaluation of the ves-
sels distal to the thrombus as well as collateral vessels often
reveals low-velocity waveforms with increased diastolic flow
due to downstream arterial dilation (Fig. 19.38). Over time, a
Aortic stenosis in children usually occurs in the setting of
mid-aortic syndrome, a term used for obstructive lesions of
the mid-aorta, regardless of etiology [104]. Approximately
64% of cases are idiopathic. The remainder have been
described in association with a variety of genetic and
acquired diseases, including Takayasu arteritis, neurofibro-
matosis, and Williams syndrome [105]. Mid-aortic syndrome
usually involves the renal (> 80%) and splanchnic (50–70%)
branches of the aorta. In most patients there is diffuse or seg-
mental narrowing of the abdominal and/or distal descending
19 Vascular Imaging
a b
Fig. 19.37 Normal abdominal aorta in a 15-year-old female. (a)
Sagittal grayscale ultrasound image depicts the echogenic walls of the
aorta and the anechoic lumen. (b) Sagittal color Doppler ultrasound
a b
Fig. 19.38 Aortic thrombosis in an 8-day-old female with a right fem-
oral arterial catheter. (a) Sagittal grayscale ultrasound image of the
abdominal aorta reveals an intraluminal catheter (arrow). (b) Sagittal
grayscale ultrasound image shows echogenic clot (arrowheads) on the
thoracic aorta, with varying involvement of the renal and vis-
ceral branches. Mid-aortic syndrome is an important cause
of renovascular hypertension in children and adolescents.
Most patients present with symptoms of severe hypertension,
absent femoral pulses, an abdominal bruit, and lower leg
claudication. Children with long-standing refractory hyper-
tension may develop encephalopathy and retinopathy.
Grayscale, color, and spectral Doppler ultrasound reveal
narrowing of the abdominal aorta as well as variable narrow-
ing of the major aortic branch vessels, especially the renal
arteries (Fig. 19.39) [106]. Collateral vessels can also be
identified. In the setting of Takayasu arteritis, arterial wall
thickening can be present. Doppler spectral analysis shows
elevated velocities at sites of stenosis with distal diminished
flow velocities.
Management of mid-aortic syndrome is aimed at control-
ling arterial blood pressure, preventing long-term complica-
929
c
image with spectral analysis reveals a characteristic high-resistance tri-
phasic waveform. (c) Spectral Doppler analysis of the celiac artery
shows a normal biphasic, low-resistance waveform
c d
distal catheter tip. (c) Sagittal color Doppler ultrasound image shows
nonocclusive thrombus (arrowheads) along the posterior aortic wall. (d)
Spectral Doppler ultrasound evaluation of the aorta distal to the throm-
bus shows low-velocity, low-resistance waveforms
tions related to hypertension, and preserving end-organ
function (including the heart and kidneys). Treatment can be
pharmacological, endovascular, or surgical [104].
Aneurysm
Aortic aneurysms in infants are most often a complication
umbilical arterial catheterization. The majority are mycotic
in etiology and associated with bacteremia, especially
Staphylococcus aureus and S. albus infection [107].
However, they can also be congenital and detected on prena-
tal ultrasound studies [108]. In older children, aortoiliac
aneurysms are usually a manifestation of a connective tissue
disorder such as Ehlers-Danlos syndrome or Marfan syn-
drome; inflammatory disorders such as Kawasaki disease
and Takayasu arteritis; infection; or trauma [109]. In Marfan
syndrome, there often is associated dilation of the aortic root.
In Kawasaki disease, there may be multiple sites of aneu-
930
Fig. 19.39 Aortic stenosis in a 5-year-old male with mid-aortic syndrome.
(a) Sagittal grayscale ultrasound image of the abdominal aorta reveals
marked narrowing (arrows) in its midportion. (b) Sagittal grayscale ultra-
sound image of the normal right kidney. (c) Sagittal grayscale ultrasound
image of the small left kidney demonstrates loss of normal corticomedul-
lary differentiation. (d) Sagittal color Doppler ultrasound image with spec-
tral analysis shows a proximal aortic velocity of 145.6 cm/sec. (e) Sagittal
color Doppler ultrasound image with spectral analysis of the narrowed por-
a b
LK
Fig. 19.40 Mycotic abdominal aortic aneurysm in a 4-month-old for-
mer 29-week gestational age infant with clinical course complicated by
methicillin-sensitive Staphylococcus aureus bacteremia. (a) Sagittal
grayscale ultrasound image reveals a markedly dilated aorta (asterisk)
with an irregular contour. (b) Transverse grayscale ultrasound image
rysm formation, including the coronary arteries; abdominal
aorta; and iliac, femoral, and splanchnic arteries.
When an aneurysm is suspected, ultrasound images and
measurements of the abdominal aorta are obtained in trans-
verse and longitudinal planes (Fig. 19.40). Grayscale ultra-
sound will show focal or diffuse aortic dilation. Mural
thrombus may also be present. The Doppler ultrasound find-
H. J. Paltiel
tion of the aorta shows an abnormally elevated velocity of 356.7 cm/sec. (f)
Transverse color Doppler ultrasound image with spectral analysis of the left
renal artery at its origin reveals an abnormal, high-resistance waveform due
to stenosis. (g) Transverse color Doppler ultrasound image of the left main
renal artery in the renal hilum shows an abnormal parvus-tardus waveform
downstream from the stenotic arterial origin. (h) Sagittal contrast-enhanced
maximum intensity projection (MIP) CT image shows the mid-abdominal
aortic narrowing (arrowhead)
c d
demonstrates the widest portion of the aneurysm (asterisk) at the level of
the left kidney (LK). (c) Sagittal color Doppler ultrasound image shows
a swirling flow pattern within the aneurysm. (d) Sagittal MIP image
from a contrast-enhanced CT shows superior displacement (arrow) of
abdominal aortic branches by the aneurysm (asterisk)
ings will depend on the size of the aneurysmal neck, the
amount of intraluminal thrombus, and the presence of calci-
fication. The maximal aortic diameter is obtained perpen-
dicular to the axis of the lumen of the aorta and measured
from outer wall to outer wall. Longitudinal images are preferred
for acquiring the most accurate measurements. Transverse
imaging is important for identification of eccentrically located
19 Vascular Imaging 931

a b c

Fig. 19.41 Dissection of the abdominal aorta in a 9-year-old male aortic wall consistent with an intimal flap. (c) Coronal 3D reconstructed
after motor vehicle collision. Sagittal (a) and transverse (b) grayscale image from a contrast-­enhanced CT study confirms a dissection (arrow-
ultrasound images show a subtle irregularity (arrowheads) of the inner head) of the infrarenal abdominal aorta

aneurysms. Normal age-related ultrasound measurements for

the abdominal aorta in children can be consulted for refer-
ence purposes [110].
Treatment of aortic aneurysms in children varies according
to the underlying cause [109]. Mycotic aneurysms are treated
with antibiotics and surgical excision. With systemic inflam-
matory disorders, initial therapy is conservative and aimed at
aneurysm prevention. Patients with Kawasaki disease receive
anti-inflammatory medication in the acute phase, usually ace-
tylsalicylic acid. If aneurysms subsequently develop, anti-
thrombotic treatment is instituted to decrease the complication
rate. Spontaneous aneurysmal rupture may occur that then
requires surgical ligation or resection. Management of patients
with Ehlers-Danlos syndrome is aimed at prevention of injury.
Those with Marfan syndrome receive beta-blockers.

Dissection
Abdominal aortic dissection is very unusual in children. It
occurs most frequently in the setting of Marfan syndrome,
where it is nearly always associated with thoracic aortic dis-
section. Ultrasound does not generally play a role in the
diagnosis which is usually made by CT. Occasionally an inti-
mal flap may be identified by ultrasound (Fig. 19.41, Cineclip
19.4), usually during a study performed for follow-up evalu-
ation of an aneurysm.

Inferior Vena Cava

 ormal Development and Anatomy

N Fig. 19.42 Diagram of embryologic segments leading to formation of
The IVC is the main channel through which venous blood
from the lower extremities and abdominal viscera returns to
the right atrium.
Normal Development
The IVC and the azygos-hemiazygos venous systems arise dur-
ing the 4th to 8th weeks of gestation through a complex pattern
of development, anastomosis, and regression that involves the
vitelline vein and the paired posterior cardinal, supracardinal,
and subcardinal veins.
the inferior vena cava. (© Catherine Delphia 2020)
Normal Anatomy
The mature IVC has four segments: hepatic, suprarenal,
renal, and infrarenal (Fig. 19.42) [111]. The vitelline vein
contributes to the hepatic segment of the IVC. The supra-
renal IVC is formed by a persistent portion of the right
subcardinal vein. The renal segment of the IVC consists
of the anastomosis between the right subcardinal and right
932 H. J. Paltiel

Fig. 19.43 Diagram of embryological vessels leading to the development of the azygos and hemiazygos venous systems. The supracardinal veins
(left panel) ultimately develop into the azygos and hemiazygos veins (right panel). (© Catherine Delphia 2020)

Vertebral
Internal
jugular
External
Superior jugular
vena cava Subclavian
Brachiocephalic
Mediastinal
Axillary
Esophageal
Cephalic
Internal
thoracic
Hemiazygos
Azygos
Intercostal
Hepatic
Inferior vena
cava
Renal
Phrenic

Gonadal Adrenal

Lumbar

Common
iliac
Internal
iliac
External
iliac

Fig. 19.44 Diagram of the major veins of the neck, chest, and abdomen

supracardinal veins. The infrarenal segment is derived
from a p­ ersistent segment of the right supracardinal vein.
The right posterior cardinal vein forms the distal-most
IVC and its bifurcation into common iliac veins.
The embryonic veins also lead to the development of the
azygos, hemiazygos, and common iliac veins. The azygos
venous system arises from the supracardinal veins. The right
supracardinal vein develops into the azygos vein, while the left
supracardinal vein becomes the hemiazygos vein (Fig. 19.43).
The IVC arises at the confluence of the common iliac
veins at the level of the fifth lumbar vertebra (Fig. 19.44). It
runs to the right of the aorta and anterior to the vertebral bod-
19 Vascular Imaging
ies. It extends through the diaphragm at the level of the
eighth lumbar vertebra and after a short intrathoracic course
drains into the right atrium.
On ultrasound evaluation, the IVC has an anechoic lumen.
Its wall is thinner and less echogenic wall than the aorta, and
its size and contour vary with changes in respiration and intra-
abdominal pressure. With deep inspiration, there is a decrease
in the diameter of the IVC, whereas the caliber of the IVC
increases in expiration. The spectral Doppler waveform of the
IVC varies according to location within the vessel. In the prox-
imal IVC, a triphasic waveform is seen that reflects right atrial
pulsatility and is similar to that within the hepatic veins. More
distally, the waveform is less pulsatile and can be monophasic
(Fig. 19.45) [3, 101].
In the setting of severe dehydration or hypovolemic shock,
the IVC will appear collapsed with a relatively monophasic
flow pattern. When right-sided heart pressures are elevated,
the IVC will have an increased diameter, as in the setting of
tricuspid insufficiency, right heart failure, or pericardial tam-
ponade, and the spectral Doppler waveforms will appear
exaggerated.
Congenital Anomalies
Congenital anomalies of the IVC are the result of abnormal
development of the embryological vitelline, posterior cardi-
nal, subcardinal, and supracardinal veins. They occur in
about 4% of the population and are most often asymptomatic
[111, 112].
a b
Fig. 19.45 Normal vena caval flow patterns. (a) Sagittal color Doppler ultrasound image with spectral analysis of the upper inferior vena cava
(IVC) reveals a normal triphasic waveform. (b) Sagittal color Doppler ultrasound image of the mid-IVC demonstrates a less pulsatile waveform
933
Interruption of the IVC with Azygos Continuation
Interruption of the IVC below the liver with azygos or hemi-
azygos continuation is due to a failure of anastomosis of the
embryonic hepatic and prerenal portions of the IVC. As a
consequence, the suprarenal IVC drains either into the azy-
gos vein and returns to the heart through the SVC or into the
hemiazygos vein. The hepatic veins drain directly into the
right atrium. This anomaly can occur as an isolated finding.
However, it is frequently seen in patients with heterotaxy and
complex congenital heart disease (i.e., the cardiosplenic syn-
dromes) [113]. Although interruption of the IVC is much
more common in the setting of polysplenia, it can also occur
with asplenia [112].
Ultrasound features of interrupted IVC include absence
of the intrahepatic portion of the IVC, drainage of the conflu-
ence of hepatic veins directly into the right atrium, and an
enlarged right-sided azygos or left-sided hemiazygos vein.
Unlike a normal IVC, an enlarged azygos vein will be located
posterolateral to the aorta and dorsal to the right renal artery
(Fig. 19.46).
There is no specific treatment for this anomaly. However,
it is important to not mistake an enlarged azygos or hemiazy-
gos vein identified on ultrasound examination for retrocrural
adenopathy [111].
Retrocaval Ureter
Retrocaval ureter is a rare entity with a 3:1 male predomi-
nance. In the majority of cases, this anomaly is asymptom-
934
a b
H
K L
Fig. 19.46 Azygos continuation of the IVC in a 2-year-old female
with heterotaxy. (a) Transverse grayscale ultrasound image of the upper
abdomen reveals a left-sided liver (L), a right-sided spleen (S) and
stomach (asterisk). K, Right kidney. (b) Sagittal grayscale ultrasound
atic. Symptomatic patients usually present with pain in the
second to fourth decades of life. The infrarenal IVC usually
develops from the embryological supracardinal vein. If
instead it develops from the subcardinal vein, the ureter will
pass posterior rather than anterior to the IVC. The aberrant
course of the ureter is believed to result in obstruction. The
ureter will then extend medially and anteriorly between the
aorta and IVC to cross the right iliac vessels and enter the
pelvis and bladder in the usual manner [114].
Two types of retrocaval ureter have been described. In the
more common Type 1, the dilated ureter assumes a reversed-
J or “fish hook” appearance. Ureteral obstruction develops at
the edge of the iliopsoas at the site where the ureter turns
cephalad before passing behind the IVC. In Type II, hydro-
nephrosis is less severe, and the ureter passes horizontally
behind the vena cava without an upward kink. Ureteral
obstruction occurs at the lateral wall of the vena cava where
the ureter is compressed behind the paravertebral muscles.
Retrocaval ureter is best depicted by cross-sectional imaging
such as magnetic resonance urography (MRU) or CT. MRU or
diuretic renography can assess the degree of functional
obstruction. Ultrasound imaging will show dilation of the
right collecting system and proximal ureter. Occasionally, the
compressed retrocaval ureter may be identified. Ultrasound is
useful for follow-up of children with known retrocaval ureter
to assess for evidence of complications.
Treatment is needed only if there is significant obstruc-
tion, infection, urolithiasis, or increasing hydronephrosis.
Surgery includes division of the retrocaval segment of ureter
with excision of any stenotic segment and ureteropelvic or
uretero-ureteric anastomosis anterior to the IVC.
Duplicated IVC
IVC duplication occurs when there is persistence of both
the right and left supracardinal veins that form duplicated
H. J. Paltiel
c
V
image shows the hepatic veins (V) draining directly into the heart (H).
(c) Sagittal color Doppler ultrasound image shows a prominent azygos
vein (arrowhead) posterior to the aorta (arrow). There is no intrahepatic
IVC
infrarenal IVC segments. The left infrarenal IVC will
join the left renal vein and drains into a normal suprarenal
IVC. Identification of a duplicated IVC has implications for
patient treatment in the setting of deep vein thrombosis and
recurrent emboli if placement of an infrarenal IVC filter is
contemplated. If this anomaly is not recognized, recurrent
pulmonary embolism can subsequently develop with poten-
tially fatal consequences [111].
On ultrasound imaging, a left-sided IVC will drain the left
renal vein and then cross the midline to join the right-sided
IVC. A potential imaging pitfall is to mistake a left-sided
IVC for an enlarged lymph node if the vessel is not followed
along its course.
Left-Sided IVC
A left-sided IVC occurs when the right supracardinal vein
regresses and there is abnormal persistence of the left supra-
cardinal vein. As with a duplicated IVC, a left-sided IVC
runs along the left side of the abdominal aorta, joins with the
left renal vein, and empties into a normal right-sided supra-
renal IVC (Fig. 19.47, Cineclip 19.5). Although a left-sided
IVC is asymptomatic, if unrecognized it can lead to prob-
lems with central venous access during interventional proce-
dures. A left-sided IVC may be confused with the abdominal
aorta, limit access options for IVC filter placement, or com-
plicate pulmonary thrombolysis procedures [111].
Thrombosis
IVC thrombosis occurs in the setting of both non-neoplastic
and neoplastic disorders. Non-neoplastic or “bland” throm-
bus is the main cause of IVC obstruction, with its attendant
risk of pulmonary embolism. Risk factors for IVC thrombosis
include venous stasis, focal compression, hypercoagulability,
malignancy, and IVC filters. Bland thrombus in the IVC may
occur in isolation, but usually develops as an extension of
19 Vascular Imaging
a b
A
A
C A
Fig. 19.47 Left-sided IVC in an 8-year-old female. (a) Transverse
grayscale ultrasound image shows the infrarenal IVC (C) located on the
left side of the abdomen. A, Aorta. (b) Transverse grayscale ultrasound
image at the level of the renal veins shows the crossing of the IVC
a b
Fig. 19.48 Left lower extremity DVT extending from the popliteal
vein to the low IVC in a 12-year-old female. (a) Sagittal power Doppler
ultrasound image depicts extensive thrombosis (arrow) of the lower
thrombosed pelvic or lower extremity deep vein thrombosis
(Fig. 19.48). Unlike tumor thrombus, bland thrombus does
not expand the caval lumen and demonstrates no enhance-
ment after IV contrast administration [115].
Neoplastic invasion of the IVC in children most often
occurs in the setting of Wilms’ tumor and is identified in
approximately 4–8% of cases [116, 117]. Recognition of IVC
tumor involvement is important as it may advance tumor stag-
ing, for example, from stage I to stage II, and necessitate an
alteration in treatment approach. IVC extension of tumor is
also associated with increased morbidity during nephrectomy.
Other primary malignancies commonly associated with IVC
invasion include renal cell carcinoma and hepatocellular carci-
noma. Metastatic disease in the liver, kidneys, and adrenal
glands can also involve the IVC via intravascular spread [111].
935
c
C
(arrowheads) from left to right, anterior to the aorta (A). (c) Transverse
grayscale ultrasound image above the renal veins depicts the IVC (C) to
the right of the midline. A, Aorta
c
IVC. (b) Sagittal color Doppler ultrasound image reveals that the upper
IVC (arrow) is patent. L, Liver. (c) Sagittal power Doppler ultrasound
image shows complete thrombosis (arrow) of the left external iliac vein
Both bland and malignant thrombi are echogenic and can
partially or completely fill the caval lumen (Fig. 19.49).
Bland thrombi are typically avascular on color and spectral
Doppler imaging, while tumor thrombi may show internal
vascularity as a result of neovascularization. When luminal
occlusion is complete, flow will be absent in the IVC distal
to the thrombus. With partial obstruction, there will be damp-
ening of the spectral Doppler waveforms. Flow in collateral
vessels can also be identified. IVC thrombi may completely
resolve, leave a linear flap, or calcify. A calcified thrombus
will be depicted as an echogenic, elongated intraluminal
mass that may demonstrate posterior acoustic shadowing.
Anticoagulation is the mainstay of therapy for bland IVC
thrombosis. Vena caval filters can be placed if anticoagula-
tion therapy is contraindicated [115, 118]. The presence of
936
a b
LK
Fig. 19.49 IVC invasion in a 20-month-old male with Wilms’ tumor.
(a) Longitudinal grayscale ultrasound image reveals a large mass
(arrow) replacing most of the left kidney (LK). (b) Coronal color
Doppler ultrasound image of the right kidney (RK) shows a patent right
tumor thrombosis significantly worsens prognosis and will
have a significant impact on the approach to treatment which
will vary according to tumor type [119].
May-Thurner Syndrome
Patients with May-Thurner syndrome, or iliac vein compres-
sion syndrome, are predisposed to iliofemoral thrombosis as
a result of an anatomic variant where the right common iliac
artery overlies and compresses the left common iliac vein
against the lumbar spine [120]. The chronic compression
leads to impaired venous return, and endothelial injury can
lead to thrombosis with the potential for extensive DVT of
the ipsilateral extremity. Patients who develop a left-sided
DVT in the context of May-Thurner syndrome are typically
young adults who will develop sudden swelling of the left
lower extremity after surgery, during immobilization, or dur-
ing pregnancy and the post-partum period. Adolescents can
also be affected.
Although ultrasound is used to confirm the presence of
acute DVT, it is not generally able to image the underlying
venous stenosis and compression of the iliac vessels due to
their deep location within the pelvis. Contrast venography is
the reference standard imaging diagnostic procedure but is
rarely performed. More often, the diagnosis is made by CT
or MR imaging [120].
Patients generally respond poorly to anticoagulation
therapy alone. Catheter-delivered thrombolytics and per-
cutaneous mechanical thrombectomy, either with or with-
out angioplasty and stent placement, are the standard of
care for symptomatic patients with May-Thurner syn-
drome. Postoperative treatment includes anticoagulation
for at least 3 months to prevent re-thrombosis. A retriev-
H. J. Paltiel
c
RK
renal vein (arrowhead) with expansile, echogenic tumor (asterisk) in the
IVC. (c) Coronal contrast-enhanced CT image shows the large left renal
mass (asterisk) and left renal vein tumor extending into (arrowhead)
and distorting the IVC
able IVC filter can also be considered for patients with a
history of pulmonary embolism [121, 122].
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 Breast
Nadia Nagra-Mahmood, Angie L. Miller,
Jennifer L. Williams, and Harriet J. Paltiel
Abbreviations
CT Computed tomography
GCT Granular cell tumor
IMLN Intramammary lymph node
MR Magnetic resonance
PASH Pseudoangiomatous stromal hyperplasia
SMR Sexual Maturity Rating
Introduction
Ultrasound is the imaging modality of choice in evaluation
of the pediatric breast. Mammography is rarely performed,
as the developing breast is highly sensitive to the effects of
ionizing radiation, and the large amount of fibroglandular tis-
sue leads to low diagnostic sensitivity [1]. Since most pediat-
ric breast lesions are benign, a conservative approach to
management and treatment is warranted. Biopsy is generally
avoided as the developing breast is uniquely vulnerable to
iatrogenic injury which can lead to permanent disfigurement
[2]. Most malignant breast masses in children are metastases
from non-breast neoplasms. In this chapter, the ultrasound
features of normal breast development, developmental
N. Nagra-Mahmood (*)
Edward B. Singleton Department of Radiology, Texas Children’s
Hospital, Baylor College of Medicine, Houston, TX, USA
e-mail: nfmahmoo@texaschildrens.org
A. L. Miller
Department of Radiology, Children’s Hospital Colorado,
University of Colorado School of Medicine, Aurora, CO, USA
J. L. Williams
Department of Radiology, University of Central Florida College
of Medicine, Maitland, FL, USA
H. J. Paltiel
Division of Ultrasound, Department of Radiology,
Boston Children’s Hospital and Harvard Medical School,
Boston, MA, USA
© Springer Nature Switzerland AG 2021
H. J. Paltiel, E. Y. Lee (eds.), Pediatric Ultrasound, https://doi.org/10.1007/978-3-030-56802-3_20
20
anomalies, as well as benign and malignant lesions that
affect the breast in children are discussed.
Technique
Patient Positioning
When performing breast ultrasound in young patients, it is
important to ensure their comfort and to provide assurance
while maintaining privacy with appropriate draping and posi-
tioning. Supine positioning is best for evaluating the medial
half of the breast, while a contralateral posterior oblique posi-
tion is better for evaluating the lateral half of the breast. It is
critical to correlate the site of scanning with any palpable
abnormality.
Doppler ultrasound assessment of the breast should be
performed using as little compression pressure as possible
since intralesional flow can be decreased or completely oblit-
erated if compression is overly zealous.
Ultrasound Transducer Selection
High-frequency, high-resolution linear array transducers are
most effective for breast tissue penetration and detection of
lesions as small as 2 mm [3]. For superficial lesions, a stand-­
off pad may be used along with appropriate lesion placement
in the focal zone to optimize image quality. Lower-frequency
curved array transducers may be useful for imaging of deeper
lesions. Power, gain, and focal zone settings must be opti-
mized to permit a distinction between solid masses and cysts.
Imaging Approaches
The imaging field of view should include all breast tissues
from the skin surface to the chest wall. The pectoralis mus-
cle can be used as a landmark to identify the posterior breast
941
942 N. Nagra-Mahmood et al.

border. Imaging must evaluate the nipple, subcutaneous fat,
fibrofatty and fibroglandular tissue, pectoralis muscle, and
ribs.
Artifactual shadowing may occur due to Cooper ligaments,
a prominent costochondral junction, or a rib posterior to the
pectoralis muscle [4]. The nipple or the cartilaginous portion
of a rib may be mistaken for a breast lesion. Appropriate com-
pression of the breast tissue along with transducer angulation
may reduce posterior acoustic s­ hadowing, further reducing the
possibility of mistaking normal tissue for a mass.
Doppler evaluation of breast lesions improves diagnostic
accuracy when used in conjunction with grayscale imaging,
particularly when attempting to differentiate a solid mass from
a cyst. Color Doppler is also useful in aiding ­ultrasound-guided
biopsies in order to avoid puncturing blood vessels that may
result in bleeding or hematoma [5].
Elastography is a useful tool for measuring the stiffness of
a lesion by applying mechanical stress [6]. Using a shear
wave technique, the stiffness of a lesion in the breast is mea-
sured using kilopascals [7]. The stiffer the lesion, the more
likely it is to be malignant. Although elastography is used to
evaluate breast masses in adults, there is very little data on its
utility in the pediatric population.
Normal Development and Anatomy
Normal Development
The breast is composed of lobules that connect to the nipple
via a series of ducts (Fig. 20.1). Normal breast development
occurs in two stages. The first stage begins at approximately
5 weeks of gestation with ectodermal thickening along the
ventral surface of the body from the axilla to the groin to cre-
ate the mammary crest or ridge (Fig. 20.2). This is followed
by normal involution of the mammary ridge, except at the
fourth intercostal space where the primary breast bud is
located. Failure of involution of the mammary ridge results
in the development of accessory nipples (polythelia) or
accessory breast tissue (Fig. 20.3) [8].
Prior to the onset of puberty, the primitive breast tissue pro-
liferates into epithelial lined ducts that end in terminal duct
lobular units at the site of the primary breast bud [3]. These
ducts are often prominent after birth as they respond to mater-
nal hormones and can result in palpable breast nodules in the
neonate. The second stage of breast development, known as
thelarche, usually commences at approximately 9 years of age
in Caucasians and between 7 and 8 years of age in African

Skin(cut) First rib

Pectoralis
major muscle

Suspensory
ligament

Adipose
tissue

Areola Lobe
Lobe
Nipple

Opening of
lactiferous duct

Lactiferous
sinus
Lactiferous
duct

Lobule
containing alveoli

Fig. 20.1 Diagram of normal breast anatomy

20 Breast 943

Level of section C

Mammary Remnant of mammary

ridge ridge which produces
primary mammary bud

a b

Primary mammary
Site of
bud (Primordium of
Mammary pit depressed nipple
mammary gland)
Areola
of breast

Epidermis
Lactiferous
duct

c d e f
Mesenchyme Dermis Secondary Mammary gland
mammary buds

Fig. 20.2 Diagram of normal mammary gland development. (a) Ventral ridges. (c) Transverse section of a mammary ridge at the site of the develop-
view of an embryo at 28 days of gestation showing mammary ridges. (b) ing mammary gland. (d–f) Similar sections showing successive stages of
Similar view at 6 weeks of gestation showing the remnants of the mammary breast development between the 12th week of gestation and birth

Americans [9]. During thelarche, estrogen secretion causes
ductal and fat proliferation, while progesterone secretion leads
to lobular growth and differentiation (Fig. 20.4).
Tanner staging, also known as Sexual Maturity Rating
(SMR), is an objective classification system that is used to
document and track the development and sequence of second-
ary sex characteristics of children during puberty. The scale
defines physical measurements of development based on
external primary and secondary sex characteristics, including
the size of the breasts, genitals, and development of pubic hair.
There are five Tanner stages of breast development under hor-
monal stimulation (Fig. 20.5, Table 20.1) [10].
944 N. Nagra-Mahmood et al.

Table 20.1 Correlation of Tanner stages of breast development

with clinical and ultrasound features
Tanner
stage Clinical presentation Ultrasound features
I Papilla is elevated Small amount of echogenic
tissue in the subareolar
region
II Breast mound forms with Nodular hypoechoic breast
elevation of the papilla bud with a few developing
Areola enlarges in ducts and surrounding
diameter fibrofatty tissue
III Breast and areola complex Increased size of hypoechoic
continue to develop and breast bud and prominence
form a palpable of the ducts
subareolar nodule
IV Areola and papilla now Elongation of hypoechoic
project to form a glandular tissue with greater
secondary mound above extension of ducts into the
the breast surrounding fibrofatty soft
tissue
Subcutaneous fat may be
Milk line identified
V Projection of the papilla Mature breast tissue composed
as areola regresses of nodules of hypoechoic
glandular tissue with
surrounding stromal tissue
Increased subcutaneous fat

Fig. 20.3 Diagram of the milk line or mammary ridge

Fig. 20.4 Normal fibroglandular breast tissue in a 16-year-old female. (arrowheads). (b) Longitudinal grayscale ultrasound images of the right
(a) Transverse grayscale ultrasound image of the breast reveals echogenic (RT) and left (LT) breasts show echogenic fibroglandular tissue (FG) and
fibrous and fatty glandular tissue (asterisks) with hypoechoic nodules the pectoralis muscle (P) forming the posterior border of the breast
20 Breast 945

Tanner staging of breast development

Stage I: No palpable glandular breast tissue

Stage II: Breast bud palpable under areola

Stage III: Breast tissue palpable outside areola.

No areolar development

Stage IV: Areola elevated above contour

of the breast

Stage V: Areolar mound recedes back into

single breast contour with areolar
hyperpigmentation and nipple
protrusion

Fig. 20.5 Tanner staging of breast development

946
Normal Anatomy
In Tanner Stage I, the breast has a prepubertal appearance.
Ultrasound demonstrates echogenic retroareolar tissue
(Fig. 20.6a).
In Tanner Stage II, there is a small breast bud. Ultrasound
demonstrates a hypoechoic retroareolar nodule with centrally
located, linear hypoechoic ducts (Fig. 20.6b).
In Tanner Stage III, the breast bud enlarges beyond the
areola, the areola becomes pigmented, and small glands
form on the areola, the Montgomery glands (Fig. 20.6c) [2,
11]. There is further breast enlargement, but the contours
a b
Fig. 20.6 Ultrasound features of normal female breast development. (a)
Tanner Stage I in a 6-month-old female. Transverse grayscale ultrasound
image shows a small focus of echogenic retroareolar tissue (arrows). (b)
Tanner Stage II in an 8-year-old female. Longitudinal grayscale ultra-
sound image shows a small, hypoechoic retroareolar breast bud (asterisk)
and a few central hypoechoic ducts (arrowheads) surrounded by echo-
genic fibrofatty tissue (arrows). (c) Tanner Stage III in a 10-year-old
female. Longitudinal grayscale ultrasound image shows image shows
increased size of the hypoechoic breast bud and prominence of the ducts
N. Nagra-Mahmood et al.
of the areola are not separate from the breast contours.
Breast ultrasound demonstrates an increased size of the
hypoechoic subareolar nodule and greater prominence of
the ductal projections into the surrounding fibrofatty soft
tissues.
In Tanner Stage IV, the areola and nipple project above the
contour of the breast to form a secondary mound. The areola
enlarges and becomes more pigmented as does the nipple.
Breast ultrasound reveals elongation of the hypoechoic nodu-
lar glandular tissue with greater extension of the ducts into the
surrounding soft tissues (Fig. 20.6d). Subcutaneous adipose
tissue is identified in some children [12].
(arrowheads) extending into the surrounding soft tissues. (d) Tanner
Stage IV in a 14-year-old female. Longitudinal grayscale ultrasound
image shows elongation of the breast bud and greater extension of the
ducts (arrowheads) into the surrounding fibrofatty tissue (arrows). (e, f)
Tanner Stage V in a 20-year-old female. Longitudinal grayscale ultra-
sound images show (e) a well-defined nipple (asterisk) and a mature
breast contour with prominent subcutaneous fat (arrowheads). There is
loss of the hypoechoic central nodule. (f) There are innumerable
hypoechoic, ovoid fibroglandular lobules (arrows)
20 Breast
In Tanner Stage V, breast development is complete.
Ultrasound demonstrates a mixture of hypoechoic and echo-
genic tissues reflecting nodules of glandular tissue dispersed
within stromal tissue. The subareolar hypoechoic nodule
noted in the previous stages is no longer visible (Fig. 20.6e
and 20.6f) [12].
Anatomic Variants
Accessory Breast Tissue
Accessory breast tissue results from a failure of regression of the
primitive mammary tissue. It usually presents as an extension
of tissue from the upper outer quadrant of the breast into the
axilla, but can occur anywhere along the course of the embryo-
logic mammary ridge, from the axilla to the groin and medial
thigh. On ultrasound evaluation, accessory breast tissue is identi-
cal to normal breast tissue and should not be misdiagnosed as an
abnormality (Figs. 20.7 and 20.8) [13, 14].
Axillary tail
Fig. 20.7 Diagram of accessory breast tissue. The normal extension of
breast tissue into the axilla is known as the axillary tail or tail of Spence
a b
Fig. 20.8 Accessory breast tissue in a 17-year-old female. (a) Longitudinal and (b) transverse grayscale ultrasound images of the left axilla show
accessory breast tissue (asterisks)
947
Surgical removal is indicated if the accessory breast tis-
sue causes physical discomfort or for cosmetic reasons.
Congenital Anomalies
Poland Syndrome
Poland syndrome is a congenital partial or total absence of
the pectoralis muscles, particularly the sternocostal head of
the pectoralis major, resulting in absence of the anterior axil-
lary fold. Webbing of the axilla with an anomalous fibrous
band extending from the thorax to the humerus may some-
times occur. In addition, there is usually deformation or absence
of ribs and hypoplasia or absence of the breast, areola, and
subcutaneous tissue [15].
Poland syndrome usually presents at birth as an asymme-
try of the chest with frequent ipsilateral abnormalities. There
is no major role for ultrasound in either diagnosis or manage-
ment. Treatment consists of reconstructive procedures tai-
lored to the deformities present in individual patients.
Polythelia/Polymastia
Failure of regression of the mammary ridge results in poly-
thelia (accessory nipples) or polymastia (accessory mammary
glands) anywhere along the embryologic mammary ridge [4,
12, 16].
Amastia/Athelia/Amazia
Amastia/hypomastia is the congenital absence or underde-
velopment of fibroglandular breast tissue [2, 16]. This entity
is often associated with chronic systemic diseases such as
Crohn disease or malnutrition.
Athelia refers to absent development of one or both nip-
ples. Amazia is the presence of a nipple and areola in the
absence of one or both mammary glands. The most common
cause is iatrogenic, related to biopsy of the immature breast
or excision of the breast bud. Radiation therapy prior to
puberty has also been reported as a cause of amazia [15].
948
Developmental Anomalies
Premature Thelarche
Premature thelarche is defined as breast development in
females younger than 8 years of age. It may occur in isolation
or in association with precocious puberty. If there are signs of
pubic or axillary hair development, further endocrinologic
workup and pelvic ultrasound are indicated. If isolated thelar-
che is noted between the ages of 1 and 3 years, it is usually
self-limited, and no further workup or treatment is required
(Fig. 20.9) [4].
Juvenile (Virginal) Hypertrophy
Juvenile hypertrophy, also known as mammary gigantism,
macromastia, or virginal hypertrophy, is a rare disorder. It is
characterized by a rapid and disproportionate growth of one or
both breasts in girls. Breast enlargement is usually symmetric
and bilateral, but can be unilateral or asymmetric. This condi-
tion usually develops rapidly, within weeks to months, at a
time of a hormonal surge such as puberty or pregnancy.
It is thought to occur as a result of end-organ hypersensi-
tivity to normal levels of sex hormones [17]. It has also been
reported in the setting of autoimmune disease and in associa-
tion with a variety of pharmacological agents where the
pathogenic mechanisms are uncertain [18]. Dense fibroglan-
dular tissue in both breasts without a focal mass has been
documented by ultrasound and magnetic resonance (MR)
imaging [19].
In girls with developing breasts, surgery should be
avoided, and anti-estrogen agents such as tamoxifen should
be the first line of treatment. Reduction mammoplasty results
in an improved appearance of the breast, but it is important to
advise the patient of the possibility of recurrence. Tamoxifen
therapy following surgery may decrease the recurrence rate.
Fig. 20.9 Maternal hormone stimulation in a 1-year-old female with a
left breast mass. Transverse grayscale ultrasound images demonstrate
N. Nagra-Mahmood et al.
If reduction treatment does not stop breast growth, a sub-
cutaneous mastectomy with complete removal of the breast
tissue is the procedure that is least likely to lead to recurrence
but is more deforming. For patients requiring a mastectomy,
psychosocial issues may contribute to stress, depression,
anxiety, and difficulties with body image and acceptance by
peers [20, 21].
Gynecomastia
Gynecomastia is the presence of excessive breast tissue in
males and is thought to be caused by an imbalance between
the stimulatory effect of estrogen and the inhibitory effect of
androgen on the breast tissue [22]. More recent data also
appears to implicate leptin, an enzyme in adipose and breast
tissue that increases estrogen, in the development of gyneco-
mastia [23]. Physiological gynecomastia can develop in the
neonatal period, during puberty, and in old age. It may also
occur in association with various pathological conditions.
Approximately 60% to 90% of all newborns will have
gynecomastia due to transplacental passage of estrogen [23].
Gynecomastia in newborns usually regresses in 2 to 3 weeks
and resolves within the first year of life (Fig. 20.10). Pubertal
gynecomastia has a peak prevalence of 40% to 65% between
13 and 14 years of age and resolves over a period of months
to 2 years in 85 to 90% of cases [23]. Clinically, patients pres-
ent with a palpable retroareolar mass that is unilateral or bilat-
eral. There can be associated tenderness.
Prepubertal or pronounced gynecomastia generally requires
further evaluation to rule out an estrogen-secreting mass such as
a Sertoli-Leydig cell tumor or an adrenal cortical tumor.
Choriocarcinoma, hepatoblastoma, or other gonadotropin-­
secreting tumors must also be considered. Additional medical
conditions, including liver disease, Klinefelter syndrome, neu-
rofibromatosis type I, and androgen insensitivity, can be associ-
ated with male breast enlargement. Anabolic steroids,
corticosteroids, digitalis, marijuana, herbal remedies, and tricy-
asymmetric enlargement of (a) the left (L) breast tissue (arrow) com-
pared to (b) the normal right (R) breast tissue (arrow)
20 Breast
Fig. 20.10 Gynecomastia in a 13-year-old male with right breast develop-
ment. Transverse grayscale ultrasound images demonstrate subareolar
clic antidepressants have all been reported to cause gynecomas-
tia [24].
Ultrasound evaluation is performed to exclude an underlying
mass. Gynecomastia can have a variable appearance, including
that of a heterogeneously dense breast (diffuse pattern), a fan-
shaped density radiating from the nipple (nodular pattern), or a
flame-shaped subareolar density radiating from the nipple with
finger-like projections interdigitating into the deeper adipose
tissue (dendritic pattern) [25]. Pseudogynecomastia occurs in
the setting of general obesity where there is an accumulation of
subareolar adipose tissue rather than breast tissue. In these
patients, ultrasound will show only adipose tissue without glan-
dular breast tissue [3, 13, 17].
Since the majority of cases of gynecomastia are physio-
logical, they do not require treatment other than reassurance.
For pathological gynecomastia, the underlying cause should
be addressed. If gynecomastia persists, tamoxifen is the
treatment of choice. Reduction mammoplasty can be consid-
ered for resistant cases. Counseling is often important and
should be considered in boys with issues of body image and
self-esteem.
Inflammatory Lesions
Mastitis and Abscess
Mastitis occurs most commonly in lactating females, but can
also develop in children with a bimodal distribution. Neonatal
mastitis refers to inflammation of the breast in the first
2 months of life, with or without an abscess [26]. It is uncom-
mon and thought to be due to maternal estrogen stimulation
949
tissue (arrows) asymmetrically enlarged in (a) the right (R) breast com-
pared to (b) the left (L) breast. P, Pectoralis muscle
of the breast that results in breakdown of the skin overlying
the nipple or mucous membranes and permits the entry of
pathogens. It occurs more often in girls and is rarely bilat-
eral. Less than 50% of all patients in this age group will pres-
ent with fever, and fewer than 75% will have leukocytosis
[26]. If the infection does not stay confined to the breast,
other complications such as cellulitis, sepsis, or brain abscess
can occur.
Mastitis in older children is most often due to direct injury
to the breast, infection, mammary duct obstruction, or lacta-
tion. Most cases develop in girls, with the usual pathogen
being Staphylococcus aureus [3, 27, 28]. Most children pres-
ent with a tender inflamed breast, often associated with fever
and leukocytosis.
An abscess is a localized collection of necrotic or inflamma-
tory exudate and usually develops in lactating females as a com-
plication of breast-feeding and mastitis. Skin breaks in the nipple
are believed to lead to the development of a breast abscess. S.
aureus is again the most common underlying pathogen [29].
Ultrasound is a key diagnostic tool in detecting neonatal
mastitis and in identifying abscesses. Ultrasound features of
mastitis include thickened, indurated, echogenic breast tis-
sue caused by inflammation. Color Doppler images demon-
strate increased vascularity.
If an abscess is present, it appears as a hypoechoic, round,
ovoid, or irregular mass containing a variable amount of inter-
nal echogenic debris. The walls of the abscess are often thick-
ened, and color Doppler demonstrates peripheral increased
vascularity with no central flow (Figs. 20.11 and 20.12).
Antibiotic therapy is the treatment of choice. If interven-
tion is required, ultrasound is useful in guiding aspiration and
drainage.
950
a b
A A
Fig. 20.11 Mastitis and abscess in a 14-year-old female. Transverse (a)
and longitudinal (b) grayscale ultrasound images of the left breast demon-
a b
Fig. 20.12 Breast abscess in an 18-year-old female. (a) Longitudinal gray-
scale ultrasound image of the right breast demonstrates a well-defined mass
with thick walls and internal echogenic debris. (b) Longitudinal color
Non-neoplastic Lesions
Mammary Duct Ectasia
Mammary duct ectasia or duct ectasia is a rare entity of unknown
etiology that most often develops in infants and young children.
Histologically there are dilated ducts ­surrounded by fibrosis and
inflammation. Patients can present with nipple discharge, breast
tenderness, or a palpable mass. Affected children are susceptible
to infection, usually by S. aureus and Bacteroides species as a
consequence of stagnant secretions. Ultrasound findings include
dilated, tubular, hypoechoic structures which may contain inter-
nal echogenic debris secondary to hemorrhage (Fig. 20.13).
Management depends on the clinical presentation. Most patients
N. Nagra-Mahmood et al.
strate an ill-defined hypoechoic abscess (A) with internal echoes. The sur-
rounding soft tissues are diffusely echogenic, in keeping with mastitis
Doppler ultrasound image demonstrates increased vascularity at the periph-
ery of the abscess
respond to antibiotic treatment and ­termination of breast-feed-
ing. Surgery may be indicated for those children who do not
respond to conservative measures [30, 31].
 etroareolar Cysts (Obstructed Glands of
R
Montgomery)
Retroareolar cysts or obstructed glands of Montgomery usu-
ally develop in adolescent females [32]. These cysts are
located at the edge of the areola and are sebaceous glands
intimately associated with the terminal portions of lactifer-
ous ducts that arise from the mammary lobules [32]. They
are believed to develop as a result of obstruction of the chan-
20 Breast
Fig. 20.13 Ductal ectasia in a 14-year-old female. (a) Transverse
grayscale ultrasound image demonstrates tubular, thin-walled anechoic
structures (white arrow) with increased posterior through-­transmission
Fig. 20.14 Retroareolar (Montgomery) cysts in a 9-year-old female.
(a) Transverse grayscale ultrasound image demonstrates multiple,
nels that drain Montgomery’s areolar tubercles. Once the
channel is obstructed, metaplasia of the squamous lining
occurs, resulting in faulty resorption of duct secretions. The
distended, obstructed channel and the acini in the accessory
lobes lead to retroareolar cyst development. Retroareolar cysts
may be asymptomatic or symptomatic.
Ultrasound is useful in cyst characterization [32]. Cysts
may be single or multiple and are usually anechoic, round, or
tubular in shape with or without internal debris or a fluid-fluid
level. They are often bilateral and generally measure less than
2 cm in diameter. When there is superimposed cyst infection,
color Doppler will demonstrate mural hyperemia (Fig. 20.14).
Asymptomatic cysts tend to be avascular by ultrasound.
Patients with a painful areolar or tender breast mass may have
cysts with mural vascularity and internal debris.
Most retroareolar cysts resolve spontaneously. Conservative
management is the treatment of choice for asymptomatic cysts
with monitoring by serial ultrasound studies until resolution.
951
(arrowhead). Note the thick-walled duct containing echogenic debris
(black arrow). (b) Transverse color Doppler ultrasound image shows
hyperemia of the thick-walled duct
thin-walled, anechoic cysts in the retroareolar region. (b) Transverse
color Doppler ultrasound image shows no flow within the cysts
Patients with symptomatic cysts are treated with antibiotics and
require more frequent follow-up. Incision and drainage are
reserved for those rare cysts that develop into an abscess [33].
Fibrocystic Disease
Fibrocystic disease is the most commonly encountered
benign disease of the breast [34]. It most often occurs in adult
women but can occasionally develop in adolescent girls.
Fibrocystic breast disease is characterized by marked cell
proliferation in response to fluctuations in estrogen or proges-
terone. Cyst formation is thought to result from a disequi-
librium between fluid production and resorption, resulting in
dilation of the lobular acini of the breast. Cysts can also form
if a duct leading to a terminal lobule becomes obstructed.
In adult females, benign fibrocystic disease has a low rela-
tive risk of malignancy. Only proliferating lesions with atypia
952
b cc
Fig. 20.15 Fibrocystic disease in a 17-year-old female. (a) Transverse
grayscale ultrasound image demonstrates a spherical anechoic structure
(arrowheads) with posterior acoustic enhancement. Transverse g­ rayscale
are associated with a true risk of increased breast cancer [35],
which is extremely rare in the pediatric population. Fibrocystic
breast disease may present as single or multiple cysts through-
out the breast parenchyma and occasionally as a palpable,
non-tender mass. However, if the cysts become inflamed or
infected, they can present as a tender palpable mass.
Ultrasound demonstrates single or multiple dilated,
hypoechoic cysts. The cysts often have thin, imperceptible walls
and demonstrate posterior acoustic enhancement. Thick-walled
cysts with internal echoes can be seen when there is superinfec-
tion (Fig. 20.15).
Treatment of cysts associated with fibrocystic disease is
conservative. If the cyst is symptomatic, aspiration is indicated.
If the cyst appears complex and has a thick wall or superin-
fection is suspected, antibiotics are indicated. The differential
diagnosis includes localized ductal ectasia, abscess, and galac-
tocele.
Galactocele
A galactocele is a thin-walled cyst that contains milk and is
most often encountered in lactating women. Galactoceles are
a rare cause of breast enlargement in children and can occur
N. Nagra-Mahmood et al.
(b) and color Doppler (c) ultrasound images demonstrate a smooth-
walled, anechoic cyst with peripheral rim calcification (arrowhead), pos-
terior acoustic enhancement (arrow), and no internal flow
in males [36]. Patients may present with painless, unilateral,
or bilateral breast enlargement [37]. A galactocele is not usu-
ally associated with other endocrine disorders. Although the
precise etiology is unknown, the cause is thought to be multi-­
factorial, and recently an association with hyperprolactinemia
has been found [38].
The most common associations are (1) the presence of
secretory epithelium that may be stimulated by prolactin as a
result of prior trauma to the breast tissue [24, 39], and (2)
ductal obstruction without fluid resorption, leading to fluid
accumulation and galactocele formation.
Ultrasound findings vary depending on the degree of milk
or protein within the cyst. A galactocele may be anechoic or
hyperechoic and often contains fat-fluid levels. When pre-
dominantly fluid, it will appear relatively hypoechoic. As the
degree of protein or fat increases within the cystic mass, it
will appear more echogenic (Fig. 20.16). MR imaging dem-
onstrates a multiloculated mass with enhancing septa. On
mammography, a fat-fluid level may be appreciated within a
benign-appearing mass.
Conservative management is the treatment of choice in
prepubertal females in order to avoid damage to the develop-
ing breast tissue [37]. Fine needle aspiration of the lesion is
sometimes performed. If an enlarging lesion occurs in a male,
20 Breast
a b
Fig. 20.16 Galactoceles in two different patients. (a) 17-year-old
female with prior breast trauma. Transverse g­ rayscale ultrasound image
depicts a well-circumscribed echogenic mass (arrowhead). (b) 20-year-
a b
Fig. 20.17 Hematoma of the breast in a 13-year-old female. (a) Transverse
grayscale ultrasound image shows a well-circumscribed mass (arrows)
surgical excision is recommended [40]. A galactocele can
persist for months to years and should be considered in the
differential diagnosis of an infant with an enlarging breast
mass.
Hematoma
A hematoma is a localized hemorrhage. In the breast, a hema-
toma develops most often after blunt trauma, frequently dur-
ing athletic activity. It can also be iatrogenic. An adequate
history and physical examination to detect bruising over the
breast are key elements in diagnosis [4, 16]. Patients with
coagulopathic conditions may also be more prone to devel-
oping a breast hematoma.
Ultrasound appearance varies, depending on the age of
the hematoma. In the acute phase, a hematoma appears hyper-
953
old female with recent cessation of breast-feeding. Transverse gray-
scale ultrasound image shows numerous dilated, communicating ducts
(arrows) containing homogeneous echogenic debris
containing internal debris. (b) Transverse color Doppler ultrasound image
demonstrate no flow within the hematoma
echoic with ill-defined borders. As the blood ages and lique-
fies, it becomes more hypoechoic and septated with increased
definition of its walls. Fat-fluid levels can also be present
(Fig. 20.17).
A breast hematoma is followed with serial ultrasound
monitoring until resolution. If it persists or causes pain,
aspiration may be both diagnostic and therapeutic. It is
important to distinguish a hematoma from an abscess or
fat necrosis.
Fat Necrosis
Fat necrosis typically develops after trauma as a result of fat
saponification, the action of tissue lipases on released fat [2].
It can also occur after surgery, biopsy, infection, and duct
ectasia, among other etiologies [41–43]. It may manifest as a
954
fixed or mobile mass frequently located near the nipple or
skin, locations that are particularly vulnerable to injury.
The appearance of fat necrosis by ultrasound is variable
and depends on the extent of the inflammatory response to the
trauma. When there is a prominent inflammatory and fibrotic
reaction, fat necrosis can appear as a solid, spiculated lesion
or a complex cyst with mural nodules. When there is little tis-
sue response, an anechoic oil cyst may develop. As the fat
liquefies, the appearance of the lesion can change, and inter-
nal debris and fat-fluid levels may be identified. Occasionally
the mass may become more solid in appearance. Typically
these lesions become smaller over time. If serial examina-
tions depict an increase in size, it should be biopsied.
Intramammary Lymph Node
An intramammary lymph node (IMLN) may be benign or
malignant and occurs with an incidence of approximately
0.7–48% on evaluation of post-surgical specimens [44, 45].
It can be detected on mammography, computed tomography
(CT), MR imaging, or ultrasound. IMLN is most prevalent in
the upper outer quadrant of the breast and can be differenti-
ated from an axillary lymph node as it is surrounded by
breast tissue.
Ultrasound is a good tool for distinguishing benign from
malignant IMLNs. Features of a benign IMLN include a well-
defined, round or ovoid nodule usually measuring about 1 cm
in diameter and containing an echogenic hilum (Fig. 20.18)
[46]. Features of a malignant or suspicious IMLN include an
irregular shape, indistinct borders, a thickened cortex, and
loss of a distinct central fatty hilum. Color Doppler evaluation
demonstrates increased vascularity compared to a normal or
benign intramammary lymph node.
Not all suspicious IMLNs are malignant. Some may have
concerning features as a result of prior trauma, biopsy, radia-
tion, or surgery. Mastitis or other infectious or inflammatory
a b
Fig. 20.18 Intramammary lymph node in a 13-year-old female. (a)
Transverse grayscale ultrasound image show a normal lymph node in
the breast with a well-defined, echogenic hilum (arrow). (b) Transverse
N. Nagra-Mahmood et al.
lesions of the breast can lead to the development of IMLNs.
Systemic disease such as lymphoma can also be a source of
enlarged IMLNs.
Management depends on the underlying cause of enlarge-
ment. Differentiation between benign and malignant lesions
should be made by fine needle aspiration.
Vascular Malformations
Vascular malformations of the breast are due to developmen-
tal anomalies consisting of endothelial lined, dilated vascular
channels [47]. These lesions are present from birth but may
not become clinically evident until later in life. Vascular mal-
formations are further divided according to channel type and
may be lymphatic, venous, capillary, arterial, arteriovenous
or combined. Of all the vascular malformations, venous mal-
formations are the most common [48, 49]. They are more
likely to be sporadic in nature rather than inherited.
Ultrasound with color and spectral Doppler is an excel-
lent modality for ­evaluation of vascular malformations as it
is a noninvasive means of assessing lesion morphology and
flow characteristics.
Venous Malformation
Venous malformations can occur throughout the body,
including the breast [50]. They often manifest as a bluish,
painless, compressible mass that is present at birth and enlarges
as the child grows. They can be localized or diffuse in extent.
Enlargement of the lesions frequently occurs during puberty
or pregnancy.
Ultrasound features of venous malformations are variable
and include both well-circumscribed and ill-defined masses
that may be hypoechoic and contain numerous vascular
channels of varying size. Depending on channel size, color
Doppler imaging with spectral analysis may or may not be
able to detect internal flow.
color Doppler ultrasound image demonstrates normal lymph node
vascularity
20 Breast
Treatment varies according to lesion size and extent.
Sclerotherapy is often performed [51]. Surgery can be cura-
tive for localized lesions. Combination therapy is reserved
for patients with extensive disease.
Lymphatic Malformation
Lymphatic malformation of the breast is a rare, benign lesion
of the lymphatic vessels. It consists of endothelial-lined dilated
channels. As fluid collects within the channels over time,
they enlarge and may communicate with the skin. Present
from birth, they can be divided into three categories, depend-
ing on the size of their cystic spaces: microcystic, macrocys-
tic, or combined [52].
Most lymphatic malformations of the breast are asymp-
tomatic. They are most frequently located in the upper,
outer quadrant of the breast, in the tail of Spence, or
beneath the areola, likely related to the drainage pattern of
breast lymphatics in the direction of the axilla and tail of
Spence.
Grayscale ultrasound findings of lymphatic malformation
vary from a simple cyst to a multiseptated mass with varying
cystic and apparently solid components. Microcystic lesions
frequently appear solid due to the small size of the compo-
nent lymphatic channels. Color Doppler imaging generally
demonstrates no significant internal vascularity although
small septal vessels are occasionally detected [47]. MR
imaging is the most accurate technique for diagnosing these
lesions and determining their overall extent. Differential
diagnoses include simple cyst, fibrocystic change, lympho-
cele, and hematoma [53].
Treatment depends on the size and location of the lesion.
Treatment options include surgery, sclerotherapy, or pharma-
cologic treatment with sirolimus [52].
Neoplasms
A wide variety of neoplasms can occur within the breast.
Table 20.2 lists benign and malignant breast tumors encoun-
tered in the pediatric population.
Table 20.2 Benign and malignant breast mass classification
Benign Malignant
Fibroadenoma Cystosarcoma phyllodes
Hemangioma Carcinoma
Intraductal papilloma Angiosarcoma
Juvenile papillomatosis Hematologic malignancies
Pseudoangiomatous stromal hyperplasia Metastases
(PASH)
Lactating adenoma
Desmoid tumor
Granular cell tumor
955
Benign Tumors
Fibroadenoma
Fibroadenoma is the most frequently encountered mass in
the pediatric breast and comprises 91% of all pathologically
proven solid breast masses [54]. The average age at diagno-
sis is 16 years [27], and is more frequent in the African
American population.
Histologically, fibroadenoma is a fibroepithelial mass that
results from overgrowth of the connective tissue stroma sur-
rounding the breast lobules. Fibroadenoma only occurs in
females since breast lobules do not occur in males. Fibroade­
noma. These benign tumors are sensitive to the effects of estro-
gen and grow rapidly during puberty, pregnancy, and lactation.
Fibroadenomas are categorized according to their size
and histology. Classic fibroadenomas have a mean diameter
of 2 to 3 cm. A giant fibroadenoma has a mean diameter
greater than 5 cm. Giant fibroadenomas occur in adolescent
girls. Juvenile fibroadenomas are rapidly growing, lesions
that differ from classic fibroadenoma due to their abundance
of stromal tissue.
Fibroadenomas are usually solitary but can be multiple,
especially juvenile fibroadenoma [54]. Patients present with
a mobile, rubbery, non-tender mass. Most fibroadenomas
grow slowly and may remain stable for months to years. Some
lesions can undergo spontaneous regression.
Ultrasound typically demonstrates an anechoic to hypoechoic
oval mass with macrolobulations occurring in up to 57% [54].
Fibroadenomas are oriented parallel to the skin surface, and
most display posterior acoustic enhancement. Features such as
spiculated, angulated margins or posterior acoustic shadowing
are concerning for other pathology. Internal microcalcification,
hemorrhage, and debris may occur, resulting in a more complex
appearance. Color Doppler imaging can show prominent vascu-
larity (Fig. 20.19).
Treatment of fibroadenoma varies according to lesion size.
Short-term follow-up is recommended for a small mass with
benign features and without rapid growth. For a mass greater
than 5 cm in diameter or a mass that is painful, surgical exci-
sion is indicated regardless of its ultrasound characteristics.
Hemangioma
The most common vascular lesion found soon after birth in the
pediatric breast is an infantile hemangioma [2]. These lesions
are benign tumors that generally appear within the first month
of life and undergo a phase of accelerated growth for about
1 year, followed by slow involution over several years.
Ultrasound findings include an oval or lobulated mass of
variable echogenicity with well-defined margins. Within the
mass, dilated vascular channels are present that demonstrate
prominent low resistance arterial blood flow with color and
spectral Doppler. Pulsatile venous flow can also be detected
956
Fig. 20.19 Fibroadenomas in two different patients. (a) Classic fibroade-
noma of the breast in a 17-year-old female. Transverse grayscale ultrasound
image demonstrates a wider-than-tall, hypoechoic elliptical mass with
posterior acoustic enhancement (arrow). (b) Longitudinal color Doppler
due to the presence of microvascular arteriovenous shunting
(Fig. 20.20) [47, 52].
Treatment of infantile hemangioma may not be necessary
unless there are complications such as ulceration, bleeding, or
cosmetic issues. Propranolol, a β-blocker, is the drug of choice
for these lesions when therapy is indicated. Steroids can be
used if an immediate response is required or if the lesion is
unresponsive to propranolol. Topical timolol, also a β-blocker,
is often prescribed for isolated skin lesions. Laser therapy is
useful for ulcerated lesions. Surgery is not usually required.
Intraductal Papilloma
Intraductal papilloma is a benign condition that develops
as a result of ductal epithelial cell growth and prolifera-
tion. It is located in the subareolar central ducts. Although
N. Nagra-Mahmood et al.
ultrasound image reveals a small amount of internal vascularity. (c) Giant
fibroadenoma of the breast in a 15-year-old female. Longitudinal grayscale
ultrasound image demonstrates a large homogeneous hypoechoic mass
(arrow)
it can occur at any age, intraductal papilloma is relatively
rare in children, usually affecting middle-aged women.
Patients present with a non-tender or tender mass with or
without nipple discharge [55].
Ultrasound demonstrates a dilated duct containing a solid,
elongated mass, often located near the nipple, that may or
may not demonstrate hypervascularity with color Doppler
(Fig. 20.21). The dilated duct may also contain anechoic
fluid, depending on the degree of ductal obstruction. The
mass can be densely calcified.
Lesions are usually treated with surgical excision. Histology
is necessary for a definitive diagnosis [56] as the imaging fea-
tures cannot distinguish benign papilloma from a malignant
lesion. Intraductal papillomas rarely recur and are associated
with a good prognosis.
20 Breast 957

a b

Fig. 20.20 Infantile hemangioma of the breast in a 15-month-old (arrowhead). (b) Longitudinal color Doppler ultrasound image shows
female. (a) Longitudinal grayscale ultrasound image demonstrates a multiple enlarged vessels coursing through the lesion
mildly lobulated, hypoechoic mass immediately deep to the nipple

a b

Fig. 20.21 Intraductal papilloma of the breast in a 19-year-old female. acoustic enhancement (arrowhead) is noted (b) Longitudinal color
(a) Longitudinal grayscale ultrasound image demonstrates a well-­ Doppler ultrasound image reveals mild peripheral hyperemia but no inter-
circumscribed intraductal cystic mass (arrow) containing debris. Posterior nal vascularity

Juvenile Papillomatosis excision with wide margins. Patients at an increased risk of

Juvenile papillomatosis of the breast is a rare, non-­malignant,
proliferative process that occurs in older adolescents and young
women less than 30 years of age [2, 57]. It usually presents as
a well-defined, mobile, firm mass located in the periphery of
the breast. Although it is a benign disorder, approximately
33–58% of affected individuals have a strong family history of
breast cancer, and co-existing breast cancer is present in
approximately 10% [58, 59].
The gross pathologic appearance of juvenile papillomato-
sis is that of a mass containing multiple small cysts of vary-
ing size, hence the name “Swiss cheese disease” [27, 58].
Histologically, juvenile papillomatosis is characterized by
multiple intraductal papillomas, ductal hyperplasia, ductal
ectasia, perifocal sclerosing adenosis, and calcification [59].
Findings on ultrasound include hypoechoic masses clearly
delineated from the normal breast parenchyma and filled with
cysts of varying size. Microcalcifications can occur. Color
Doppler shows no abnormally increased vascularity (Fig. 20.22)
[16, 60]. These imaging features are typical but not specific.
MR imaging can be useful in depicting the cysts on T2-weighted
images as well as marked enhancement after intravenous con-
trast administration [16, 61].
Given the association of juvenile papillomatosis with
breast cancer, adequate treatment requires complete surgical
recurrence are those with a family history of breast carci-
noma, multiple sites of involvement, atypical ductal hyper-
plasia, and incomplete excision [60]. Annual monitoring for
breast cancer is recommended, and female family members
should undergo breast screening.
 seudoangiomatous Stromal Hyperplasia
P
Pseudoangiomatous stromal hyperplasia (PASH) is a myofibro-
blastic growth disorder that is thought to be hormonally medi-
ated via progesterone. Myofibroblastic cell nuclei have been
shown to express progesterone receptors [62, 63]. PASH can
present anywhere from adolescence to old age, although it is
most often seen in adolescent females [16, 24]. Both young and
adult males with gynecomastia can also be affected. Clinically
PASH presents as a painless mass that is mobile, rubbery, and
firm and may display rapid growth in adolescents [62].
The ultrasound findings of PASH are nonspecific [16, 64].
Most often there is a well-defined, homogenous, and hypoechoic
mass. Cysts are virtually never identified. Lesions tend to be
oriented parallel to the long axis of the skin surface, similar to
what is seen with fibroadenoma. This similarity in appearance
may cause difficulty in distinguishing between the two entities
(Fig. 20.23). Biopsy or complete excision is generally necessary
to confirm the diagnosis of PASH.
958
a b
Fig. 20.22 Juvenile papillomatosis of the breast in a 17-year-old
female. (a) Transverse grayscale ultrasound image demonstrates a mass
consisting of multiple anechoic cysts (arrowheads) within an echogenic
a b
Fig. 20.23 Pseudoangiomatous stromal hyperplasia (PASH) of the
breast in an 11-year-old female. (a) Longitudinal grayscale ultrasound
image demonstrates a large, solid mass with well-defined borders and
Since PASH is not a pre-malignant lesion, treatment is usu-
ally conservative with follow-up ultrasound studies to confirm
lesion stability or, rarely, spontaneous regression. Surgery is
indicated if there is rapid growth of the mass or if there is a
strong family history of breast cancer [65, 66]. PASH will recur
in up to 18% of patients if incompletely excised and can reap-
pear in either the ipsilateral or contralateral breast [66–68].
N. Nagra-Mahmood et al.
stroma resulting in a characteristic “Swiss cheese” appearance. (b)
Transverse color Doppler ultrasound image reveals the avascular nature
of the lesion
homogeneous echotexture, similar in appearance to a giant fibroade-
noma. (b) Longitudinal color Doppler ultrasound image reveals mini-
mal peripheral vascularity
Lactating Adenoma
Lactating adenoma is a benign tumor that usually presents
toward the end of pregnancy or during lactation as a result of
hormonal fluctuations. Histologically it is characterized by
ductal proliferation with a sparse stromal component [69,
70]. Lactating adenoma presents clinically as a palpable,
painless mass in the third trimester of pregnancy.
20 Breast
Ultrasound reveals a well-defined, wider-than-tall mass
with lobulated borders that is hypoechoic and homogenous
with posterior acoustic enhancement. Large tumors may be
heterogeneous with spiculated, angulated margins and poste-
rior acoustic shadowing suggestive of malignancy. Large
cystic spaces can be present due to necrosis. Tiny hyper-
echoic foci within the masses may represent fat droplets and
are often a key to the correct diagnosis [71].
Most lactating adenomas will involute spontaneously.
However, lesions with ultrasound features concerning for
malignancy or growing lesions should be surgically excised.
Prognosis is excellent with recurrence being very rare.
Desmoid Tumor
Desmoid tumor, also known as aggressive fibromatosis or des-
moid-type fibromatosis, is a rare focally invasive lesion that
does not metastasize. Histological features include interlacing
bundles and fascicles of spindle cells with varying degrees of
a b
Fig. 20.24 Desmoid tumor of the breast in an 18-year-old female.
(a) Longitudinal grayscale ultrasound image shows a well-defined,
heterogeneous solid mass. (b) Sagittal T2-weighted magnetic reso-
nance (MR) image reveals that the mass is hypointense to fluid but
959
surrounding collagen [72]. It most commonly occurs in the
abdominal wall or extremities and accounts for only 0.2% of all
breast tumors [73] and 3% of all solid tumors [74]. It is bilateral
in 4% of patients [75]. Desmoid tumor of the breast is most
often identified in women between the ages of 25 and 45. It can
also develop in younger females and in males.
Clinical associations include a history of silicone breast
implants, chest irradiation and surgical trauma, as well as
Gardner syndrome and familial adenomatous polyposis.
Patients present with a firm, mobile, palpable mass with
overlying skin thickening. If the mass is situated close to the
nipple, nipple retraction may be seen.
Ultrasound features of desmoid tumor mimic those of
breast carcinoma, including a hypoechoic mass with lobu-
lated, ill-defined borders and a thick, hyperechoic rim. Some
masses may appear spiculated with posterior acoustic shadow-
ing. MR imaging can be used to identify pectoralis muscle or
chest wall invasion (Fig. 20.24).
hyperintense to the adjacent normal-appearing breast tissue, features
suggestive of a cellular process. (c) Axial contrast-enhanced,
T1-weighted, fat-suppressed MR image demonstrates avid enhance-
ment of the mass
960
a b
Fig. 20.25 Granular cell tumor in a 19-year-old female. (a) Transverse
grayscale ultrasound image demonstrates a hypoechoic mass (arrow-
Desmoid tumors are locally invasive and have a high
recurrence rate. Treatment is wide local excision with clear
margins [75]. Aggressive and recurrent tumors may require
additional treatment with chemotherapy and radiation.
Granular Cell Tumor
Granular cell tumor is a rare, benign lesion that usually
occurs in the head and neck region, and infrequently in
the breast, in approximately 5–6% of cases [76]. This
tumor is most often seen in young, premenopausal African
American females but has also been reported in women
between the ages of 30 and 50 [77]. Granular cell tumor
is of Schwann cell (perineural) origin. It presents as a
firm, palpable mass close to the skin surface with asso-
ciated skin retraction and can clinically mimic breast
cancer [78].
The ultrasound appearance of granular cell tumor is non-
specific and varied, depending on the amount of reactive fibro-
sis, and ranges from hypo- to hyperechoic and well-­defined to
ill-defined with spiculated margins (Fig. 20.25) [78].
Treatment is wide local excision with negative margins
[79]. Incomplete excision may result in recurrence.
Malignant Tumors
Malignant tumors of the breast are rare in children. Metastases
to the breast are more common than primary breast neo-
plasms, and the most common neoplasms to metastasize to
the breast are lymphoma, leukemia, and rhabdomyosarcoma
[27]. Primary breast neoplasms account for fewer than 1% of
all cancers in childhood and are more common in females
[80, 81].
N. Nagra-Mahmood et al.
head) with spiculated borders. (b) Transverse color Doppler ultrasound
image reveals no significant internal flow
Cystosarcoma Phyllodes
Cystosarcoma phyllodes or phyllodes tumor is a rare fibro-
epithelial tumor [16, 27, 28, 82] and accounts for 0.3–1% of
all breast masses [83]. Peak prevalence is in the fourth decade
of life, although about 5% of phyllodes tumors occur in
females less than 20 years of age. It is the most common
primary breast malignancy in children and adolescents [16].
Histologically phyllodes tumor is characterized by a leaf-
like architecture resulting from enhanced intracanalicular
growth, cleft-like spaces lined by epithelium, and hypercel-
lular stroma [84]. It is categorized as benign, intermediate, or
malignant, based on histological features. Although about
85% of cases in children are benign, some tumors can dem-
onstrate invasion and recurrence. Rare fatalities have also
been documented [2].
Clinically, patients present with a rapidly growing, mobile
breast lump similar in presentation to a juvenile or giant
fibroadenoma. A differentiating feature is that a phyllodes
tumor tends to grow more rapidly. Patients with multiple
masses or a strong family history of breast cancer should be
evaluated for an underlying genetic predisposition [84].
Ultrasound features of phyllodes tumor are similar to those
of a juvenile or giant fibroadenoma. There are well-defined,
lobulated margins with heterogeneous low-level internal
echoes. Small cysts or intralesional clefts may be identified
(Fig. 20.26) [85]. Posterior acoustic shadowing can occur in
up to 15% of cases with posterior acoustic enhancement in
only 5%. Microcalcifications are rare.
Pathological evaluation is required to distinguish between
the benign and malignant forms of phyllodes tumor. Treatment
is complete excision with wide margins to decrease the chance
of recurrence which has been reported as being up to 40%
[86]. Breast conservation is the goal in children, and mastec-
20 Breast
a b
Fig. 20.26 Cystosarcoma phyllodes in two different patients. (a)
Longitudinal grayscale ultrasound image in an 18-year-old female
demonstrates a hypoechoic mass (arrow) with a well-defined capsule.
(b) Longitudinal color Doppler ultrasound image shows markedly
tomy is only indicated for large tumors or in patients with
small breasts [87]. Local recurrence usually occurs within the
first few years after surgery, especially if the surgical margins
were positive. Metastases develop in about 20% of tumors
with malignant histology, particularly to the lungs [2].
Carcinoma
Primary breast carcinoma is extremely unusual in children,
with an incidence of 0.03 cases per 100,000 in those less than
20 years of age [16, 81]. Secretory carcinoma is the most
common primary breast tumor in children and is less aggres-
sive than infiltrating ductal carcinoma [28, 80, 81, 88, 89].
Primary breast carcinoma usually presents with an enlarging,
painless, and fixed mass.
Ultrasound features of a primary breast carcinoma are
inconsistent and nonspecific [90–92]. Masses may appear
hypoechoic with ill-defined or microlobulated margins. Lesions
that are taller than wide with posterior acoustic shadowing
961
increased flow throughout the mass. (c) Longitudinal grayscale
extended field of view ultrasound image in a 20-year-old female dem-
onstrates a well-circumscribed mass (calipers) with heterogeneous
echotexture and a centrally located linear anechoic cleft (arrowhead)
and internal echoes are very concerning and should be biopsied
(Fig. 20.27).
Due to the rarity of primary breast carcinoma in children,
there is no standard management approach. Most cases will
be treated with surgical excision. Sentinel lymph node biopsy
may be indicated for appropriate staging since axillary nodal
metastases are identified in 20–30% of cases [2].
Secondary breast carcinomas are much more likely to
occur in girls with a history of mantle cell radiation for
Hodgkin lymphoma. The risk of a secondary breast cancer
developing in this population is 75 times greater than in the
general pediatric female population, and those irradiated
between the ages of 10 and 16 years are at highest risk [93].
By 40 years of age, the incidence of breast cancer is
12–20% in patients with prior chest irradiation which is
comparable to the incidence in patients with the BRACA 1
or 2 gene or in patients with hereditary cancer syndromes
[16]. Therefore, thorough screening of Hodgkin lymphoma
962
Fig. 20.27 Invasive ductal breast carcinoma in a 17-year-old female.
(a) Transverse and (b) longitudinal grayscale ultrasound images depict
a large, heterogeneous breast mass with irregular borders. (c) Transverse
color Doppler ultrasound image of the mass shows internal vascularity.
survivors with both mammography and MR imaging should
begin at either 25 years of age or 8 to 10 years following
completion of radiation treatment [24, 94].
Angiosarcoma
Angiosarcoma (also known as malignant hemangioendothe-
lioma) is an aggressive spindle cell tumor that accounts for
0.04% of all breast malignancies with an average age at diag-
nosis of 40 years [95–100]. Primary angiosarcoma of the
breast rarely occurs in children, although a low-grade form
has been observed in the second decade of life and is most
common in patients with a prior history of chest irradiation
[97, 98].
Ultrasound findings include a large, ovoid, well-­circumscribed
mass that may be hyper- or hypoechoic with internal heterogene-
ity or hemorrhage. Posterior acoustic enhancement is rarely
seen, and posterior acoustic shadowing is absent. Color Doppler
demonstrates increased vascularity with associated skin or sub-
N. Nagra-Mahmood et al.
(d) Transverse color Doppler ultrasound image of the ipsilateral axilla
reveals abnormally enlarged lymph nodes with prominent vascularity
consistent with metastases
cutaneous edema. Interestingly, up to 33% of angiosarcomas
may not be detected on mammography [99, 101].
Angiosarcoma is treated by surgical excision. There is a
low 5-year survival rate with the lungs the most frequent site
of metastasis.
Hematologic Malignancies
Primary lymphoma of the breast is extremely rare. However,
lymphoma and leukemia are the most common hemato-
logic malignancies to metastasize to the breast [12, 46].
Burkitt lymphoma, a form of non-Hodgkin lymphoma, is
the usual etiology [27]. Patients with metastases due to
lymphoma or leukemia usually present with painful, enlarg-
ing, and bilateral breast masses that may be single or mul-
tiple. Associated axillary lymphadenopathy or chest wall
invasion is common.
Ultrasound demonstrates hypoechoic, lobulated masses with
either well-defined or ill-defined margins. They may contain
20 Breast
a b
R
c d
Fig. 20.28 Juvenile myelomonocytic leukemia of the breast in a 9-year-old
female. Longitudinal grayscale ultrasound images of the (a) right (R) and
(b) left (L) breasts depict irregular, lobulated, heterogeneous masses
internal echoes with p­ osterior acoustic shadowing. Color Doppler
may demonstrate internal vascularity (Figs. 20.28 and 20.29).
Diagnosis is made by either fine needle aspiration or core
biopsy. The prognosis associated with hematological malig-
nancies metastatic to the breast is dismal.
Metastases
Metastatic tumors are the most common neoplasm occurring
in the pediatric breast [81]. Breast metastases occur much
more often in females than in males, most frequently from
rhabdomyosarcoma, neuroblastoma, and hematologic malig-
nancies [16]. The most common malignancy to metastasize to
the breast is the alveolar sub-­type of rhabdomyosarcoma with
breast metastases seen in up to 6% of children [102]. Neuro­
blastoma also frequently metastasizes to the breast and pres-
963
L
with ill-defined borders. (c) Axial and (d) sagittal contrast-enhanced,
T1-weighted, fat-suppressed MR images of both breasts (c) and the left
breast (d) demonstrate diffusely enhancing masses
ents as multiple hypoechoic masses. Hematologic malignancies
are described in the prior section of this chapter.
Less common tumors that can metastasize to the breast
include sarcomas, especially Ewing sarcoma, primitive neu-
roendocrine tumor, and melanoma. Metastases may be sin-
gle or multiple, although a single, enlarging mass is the most
common presentation.
By ultrasound, breast metastases are usually hypoechoic and
heterogeneous with irregular borders and internal hyperechoic
foci (Fig. 20.30) [2, 103–105]. Posterior acoustic shadowing
and associated axillary lymphadenopathy are common. Chest
wall invasion may occur with lymphoma.
Any enlarging breast mass in a patient with a known
extra-mammary primary malignancy must be biopsied to
exclude metastatic disease.
964
a b
Fig. 20.29 Malignant B-cell lymphoma metastatic to the breast in a
19-year-old female. (a) Transverse grayscale ultrasound image shows a
a b
Fig. 20.30 Metastatic liposarcoma to the right breast in a 13-year-old
female with a primary tumor of the right shoulder. (a, b) Transverse
grayscale ultrasound images of the right breast demonstrate two sepa-
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 Index

Page numbers followed by f indicate figures; t, tables

A pulse repetition period, 9, 10f

AAST. See American Association for the Surgery of Trauma rarefaction, 3
Abscess(es) reflection, 4–7, 5f, 6f
abdominal, 339, 494 specular reflection, 4–6
adrenal, bacterial, 595–596 refraction, 7, 7f
amebic, 378 scattering, 4, 6, 5f–6f
bacterial, 375–376, 494 sound propagation, 2–4, 2f–3f
brain, 86–87, 88f speckle artifact, 6, 6f–7f
breast, 949, 950f wavelength, 2, 2f
epidural, 120 Acquired cystic kidney disease (ACKD), 779, 779f
epididymal, 645 Acquired immune deficiency syndrome (AIDS), 472, 501, 502f, 515,
fungal, 378, 494–497, 495f, 496f 536, 609, 621, 772
of kidneys ACR. See American College of Radiology
acute pyelonephritis and, 769–770, 770f Acute ischemic stroke (AIS), 78, 80f, 81f, 905–906
after kidney transplantation, 795–797, 797f Acute kidney injury (AKI), 784–786, 786f
liver Acute lymphoblastic leukemia, 261, 520f, 572f, 620, 668, 813
fungal, 378, 378f Acute pyelonephritis, 768–769, 769f
complication of bile duct stricture, 472 Acute scrotal pain, 639, 649. See also Epididymitis; Epididymo-
complication of Caroli disease, 459 orchitis; Testicular torsion
after liver transplantation, 423–425, 424f–425f Acute suppurative parotitis, 164
parasitic, 378–380, 379f Acute suppurative thyroiditis, 159, 159f
pyogenic, 375–376, 376f–377f Acute tubular necrosis (ATN), 784, 791–792, 792f
pelvic, 339 Adenoid cystic carcinoma, 167, 167f
peritoneal, 539f Adenomatoid tumor(s), 668, 672
prostatic, 678 Adenomyomatosis, 450–451, 451f
pulmonary, 184–185, 184f–186f Adenomyosis, 718–719, 719f
retropharyngeal, 145f, 730 Adnexa. See Female genital tract
scrotal, 645–646, 646f Adnexal masses. See Female genital tract
soft tissue, 839–840, 840f Adnexal torsion. See Female genital tract
splenic, 494, 494f, 497f, 501 ADPKD. See Autosomal dominant polycystic kidney disease
subperiosteal, 851, 851f, 852 Adrenal gland(s)
testicular, 644–645 adrenal cyst
Abdominal abscess(es), 339, 494 idiopathic adrenal cyst, 594, 594f
Accessory breast tissue, 947, 947f adrenal hemorrhage, 596–597, 596f
Accessory pancreatic lobe, 567, 569 adrenal heterotopia, 593
Accessory renal artery, 736–737, 737f adrenocortical tumors
Accessory spleen, 487–490, 489f adrenocortical adenoma and carcinoma, 597, 597f–598f
Acinar cell carcinoma, 166–167, 167f, 584–585 anatomic variants
ACKD. See Acquired cystic kidney disease discoid adrenal gland, 590–591, 591f
Acoustic impedance, 4–5, 5f, 12, 38, 175–176, 200 congenital anomalies
Acoustic radiation force impulse (ARFI) imaging, 34f, 34–35 adrenal rests, testicular, 592, 592f
and liver, 359, 359f, 359t congenital adrenal agenesis, 591
Acoustic(s) fusion abnormalities
acoustic impedance, 4, 5f, 12, 38, 175–176, 200 circumrenal adrenal gland, 591–592
attenuation, 7–8, 8f horseshoe adrenal gland, 592, 592f
compression, 3 genetic disorders
distance measurement, 8–11 congenital adrenal hyperplasia, 593, 593f
frame rate, 10–11 congenital lipoid adrenal hyperplasia, 593
frame time, 11 Wolman disease, 594
frequency, 2, 2f hemangioma, infantile
frequency spectrum, 9, 9f of adrenal, 608
pulse repetition frequency, 9, 10f, 43f, 334, 900 infection

© Springer Nature Switzerland AG 2021 969

H. J. Paltiel, E. Y. Lee (eds.), Pediatric Ultrasound, https://doi.org/10.1007/978-3-030-56802-3
970 Index

Adrenal gland(s) (cont.) intracranial, 468, 775

abscess, 595–596 and Kawasaki disease, 929–931
congenital Herpes simplex viral infection, 595 mycotic, 916, 931
granulomatous infection, 595, 595f of peripheral arteries, 916
xanthogranulomatous adrenalitis, 595 and renal angiomyolipoma, 804
leiomyoma, 609 of renal artery, 779
lymphoma, 609 and Takayasu arteritis, 613f, 903–904
metastases, 609 and tissue vibration artifact, 45
myelolipoma, 607–608 and vein of Galen malformation, 90, 91f
neural crest tumors venous, 908
ganglioneuroblastoma, 599–600, 600f Angiosarcoma
ganglioneuroma, 598–599, 599f of bladder, 823–824
neuroblastoma, 601–602, 601t, 602f–605f, 606 of breast, 962
fetal neuroblastoma, 601–602 of liver, 402–403
normal anatomy, 589, 591f of spleen, 520–521
normal development, 589, 590f Anisotropy
pheochromocytoma, 606–607, 606f–607f of muscles, 837
and hereditary syndromes, 606 of tendons, 838, 838f, 867, 869f, 879
technique of peripheral nerves, 838f, 867
imaging approaches, 588–589, 589f Ankle and hindfoot
patient positioning, 588 apophyseal avulsion injuries, 889, 889f
ultrasound transducer selection, 588 calcaneal apophysis, 888f, 889
teratoma, 608, 608f imaging approaches
Adrenal rests, intratesticular, 592, 592f, 593, 660, 661f calcaneal apophysis, 888f, 889
AFP. See Alpha-fetoprotein joint effusion, 888–889, 888f
Aggressive fibromatosis, 548–549, 959–960, 959f joint effusion, 888–889, 889f
Agyria. See Lissencephaly normal anatomy, 887, 887f
AIDS. See Acquired immune deficiency syndrome patient positioning, 888–888, 888f
AIDS cholangiopathy, 472 Anorchidism, 636–637
AIS. See Acute ischemic stroke Anorectal malformations, 248, 335–336, 336f–337f, 702, 760
AKI. See Acute kidney injury Anterior horn width, 92, 92f
Alagille syndrome, 372, 465, 468, 468f, 469 Antral web, 292. See also Gastric diaphragm
ALARA. See As low as reasonably achievable Aorta
Aliasing aneurysm, 929–931, 930f
in abdominal vascular imaging, 417f, 421f, 789 dissection, 931, 931f
in color Doppler ultrasound, 42–43, 43f–44f normal anatomy, 927, 928f–929f
Alobar holoprosencephaly, 64. See also Holoprosencephaly normal development, 927
Alpha-fetoprotein (AFP) stenosis, 928–929, 930f
anencephaly and, 63 thrombosis, 927–928, 929f
congenital hemangioma and, 389 Appendicolith, 539f, 612, 612f. See also Acute appendicitis; Fecalith
gastric teratoma and, 300 Appendix
liver masses and, 388t acute appendicitis, 323, 331–333, 331f–332f, 331t, 541, 767
pancreatoblastoma and, 581 appendicolith, 539f, 612, 612f
yolk sac tumor and, 663 fecalith, 331, 332f
Amebic abscess(es), 378 benign masses
Amenorrhea, 683, 692–693, 698, 700–701, 715, 724 mucocele, 333
American Association for the Surgery of Trauma (AAST) cystic fibrosis, 332, 332f
and renal trauma, 802 malignant tumors
and splenic trauma, 508–509, 510f carcinoid, 333
American College of Radiology (ACR) lymphoma, 333–334, 333f
and ALARA principle, 331 normal anatomy, 330, 330f
and hip ultrasound screening, 873 normal development, 291f, 330
and lower extremity vein evaluation, 919 technique
and TI-RADS, 153 imaging approaches, 330
Amplitude (A)-mode imaging, 14, 14f. See also Image display patient positioning, 330
Anatomically related Doppler artifacts ultrasound transducer selection, 330
blooming artifact, 46–47, 46f Arcuate uterus, 691–692, 691t
spectral mirror image artifact, 44–45, 45f ARFI. See Acoustic radiation force impulse imaging
tissue vibration artifact, 45, 45f ARPKD. See Autosomal recessive polycystic kidney disease
twinkle artifact, 45–46, 46f Array transducer, 10f, 12f, 19–22, 20f–22f, 24, 26, 26f, 52, 61, 92–93,
Anencephaly, 63, 63f, 64f 174, 196, 239–240, 271, 274f, 284, 356, 434, 482, 539, 564,
Aneurysm(s) 588, 629, 683, 686f, 910, 918, 941
of abdominal aorta, 613f, 929–931,930f Arteriovenous fistula (AVF)
of carotid artery, 905 of dura, 90
clinical and ultrasound features of, 918t of extremity vessels, 916–918
of iliac arteries, 929 of GI tract, 327
Index 971

of kidney, 783–784, 785f neutropenic colitis and, 342–343

transplant biopsy and, 789, 791f
of musculoskeletal system, 892, 894, 894f
of neck, 140, 142f
post-traumatic, 916–918, 918f, 918t
and tissue vibration artifact, 45, 45f
Arteriovenous malformation(s) (AVM)
of musculoskeletal system, 892, 894, 894f
of neck, 140–141
of scrotum, 671
vein of Galen malformation, 90, 91f
Artery(ies). See specific arteries
Arthritis. See also Rheumatic disorders
septic, 850f, 851, 851f, 854, 854f, 877
Artifacts
contrast agent
blooming, 47
high-intensity transient signals, 47
systolic peak velocity increase, 47
Doppler, anatomically related
spectral mirror image, 44–45, 45f
blooming, 46, 46f
tissue vibration, 45, 45f
twinkling, 45–46, 46f
Doppler, technically related
absent Doppler signal, 42–43, 42f
aliasing (wraparound), 43, 43f, 44f
color Doppler noise, 43–44
flow directional abnormalities, 44, 44f
inappropriate Doppler settings, 42–43, 42f
grayscale
comet-tail, 38, 39f
enhancement, 39–40, 40f
mirror image, 37, 37f
partial volume, 40–41, 41f
refraction, 37, 38f
reverberation, 38, 38f, 39, 39f
ring-down, 39
shadowing attenuation, 40–41, 40f–41f
side lobe, 39, 39f
three dimensional ultrasound, 47
Ascites, 528–530, 529f–530f
Budd-Chiari syndrome, 383–384, 383f
eosinophilic gastroenteritis and, 297–298, 320–321
biliary tract trauma and, 472–473
chemical peritonitis and, 537, 538f
chylous, 531–532, 531f
kaposiform lymphangiomatosis and, 513
lymphatic malformation, intra-abdominal, and, 542
pulmonary lymphangiectasia and, 188
cirrhosis and, 374–375, 375f
Crohn disease and, 339
drainage of, 530
graft-versus-host disease
of GI tract, 322–323, 323f
hemolytic-uremic syndrome and, 343
hemoperitoneum, 530, 531f
Henoch–Schönlein purpura and, 319–320, 320f
after liver transplantation, 408t, 412
loculated, 529, 530f
malpositioned umbilical venous catheter and, 380–382, 381f
meconium ileus and, 311–312
meconium peritonitis and, 312–313, 653
mesenteric visualization and, 526, 527f–528f
ovarian tumors and, 708, 710
Meigs syndrome, 705t, 710
pancreatitis, necrotizing, and, 575f
passive venous congestion of liver and, 386, 386f
peritoneal tumor and, 551–555, 553f–555f
portal hypertension, 375, 382, 508
pseudomembranous colitis and, 341–342
sclerosing encapsulating peritonitis and, 537–539, 538f
serum-ascites-albumin gradient, 529, 529t
sinusoidal obstruction syndrome, 384–385, 384f
splenic rupture and, 520
tuberculous peritonitis and, 497
urine, 532, 532f, 746, 764, 794, 802f
vascular anomalies of GI tract and, 327
viral gastroenteritis and, 316
Atelectasis, 183, 190, 200–201, 207f, 214f, 278
Atlanta classification, revised, of acute pancreatitis, 572
ATN. See Acute tubular necrosis
Attenuation, 7–8, 8f, 13, 18, 24
ultrasound artifacts and, 37, 39–40, 40f–41f
Augmentation cystoplasty, 824, 825f
Autosomal dominant polycystic kidney disease (ADPKD), 369, 571,
571f, 775–778
Autosomal recessive polycystic kidney disease (ARPKD),
775, 775f
Avascular necrosis (AVN), 873, 875
AVF. See Arteriovenous fistula
AVM. See Arteriovenous malformation(s)
AVN. See Avascular necrosis
B
Bacterial abscess(es), 375–376, 494
Bacterial infection, 86, 88, 144, 164, 201, 230, 254, 316, 375, 494,
501, 611, 773, 839
Bacterial parotitis, 164–165, 165f
Baker (popliteal) cyst, 881, 881f
Bartholin cyst, 721
Basal ganglia, 58–59, 63, 77, 79f, 85
Beam width artifact, 40–41
Beckwith-Wiedemann syndrome
adrenocortical tumors and, 597, 597f
hepatoblastoma and, 397
nephrogenic rests and, 810
pancreas and, 571
pancreatoblastoma and 631
pediatric renal cell carcinoma and, 810
rhabdomyosarcoma and, 551
Wilms’ tumor and, 806
Bell clapper deformity, 639, 639f, 640
Benign external hydrocephalus, 92–93, 92f
Benign macrocrania of infancy. See Benign external hydrocephalus
β-hCG. See Beta-human chorionic gonadotropin
Beta-human chorionic gonadotropin
gastric teratoma and, 300
ovarian tumors and, 705t, 708
testicular seminoma and, 663
Bezoars. See Gastric bezoar
B-flow, 32
Bile plug syndrome, 463, 470–471, 470f
Biliary atresia, 359, 368, 372, 374, 408, 433, 436, 437f, 458, 462–463,
463f, 464f, 465, 466f, 469, 475f, 509f
Biliary dyskinesia, 447–448
Biliary tract
anatomic variants, 456–457, 457f
Caroli disease, 459–460, 462f
congenital anomalies
972 Index

Biliary tract (cont.) of spleen, 515f

choledochal cysts, 457–459, 458f–461f Blunt abdominal trauma, 281, 379, 380f, 453, 454f, 482, 508, 577,
Todani classification, 457, 458f 597, 781, 801
normal anatomy, 455–456, 455f–456f B-mode flow imaging. See B-flow
normal development, 455 B-mode imaging, 14, 21, 27–30, 32–35, 47–48, 174, 196, 199, 200f,
obstruction 272, 278
AIDS cholangiopathy, 472 Bochdalek hernia, 274, 274f, 275f, 276f
Alagille syndrome, 465, 468, 468f, 469 Bone(s)/Cartilage
bile duct stricture, 472 congenital rib anomalies, 849, 849f
biliary atresia, 462–463, 462f–464f imaging approaches, 847–848, 848f
Kasai classification of, 463f normal anatomy, 847–848, 848f
Kasai procedure, treatment for, 463 osteomyelitis, 850–851, 850f–851f
Byler disease, 469 trauma
choledocholithiasis, 469–470, 469f classic metaphyseal lesion, 852, 853f
inspissated bile syndrome, 470–471, 470f epiphyseal separation, 851–852, 852f
Mirizzi syndrome, 471–472 tumors
neonatal hepatitis syndrome, 443t, 463, 465, 467f osteochondroma, 849f, 853–854
sclerosing cholangitis, 471, 471f BPBP. See Brachial plexus birth palsy
spontaneous perforation, of extrahepatic bile ducts, 472 Brachial plexus birth palsy (BPBP), 858–859, 858f–859f
trauma, 472–473 Brain
tumors benign external hydrocephalus, 92–93, 93f
benign masses congenital anomalies
bile duct adenoma, 473 dorsal induction disorders
granular cell tumor, 473 anencephaly, 63, 63f
malignant bile duct tumors Chiari malformation(s), 61–63, 62f, 65, 113, 121
cholangiocarcinoma, 474 Chiari I, 61, 62f
metastases, 474–475, 475f Chiari II, 61–63, 62f
neuroendocrine tumor, 474, 475f and myelomeningocele, 62, 62f
rhabdomyosarcoma, 473–474, 474f hydranencephaly, 63, 64f
Biloma migration disorders
after liver transplantation, 408t, 423–425, 424f–425f gray matter heterotopia, 68, 69f
after spontaneous perforation of extrahepatic bile ducts, 472 lissencephaly (agyria), 68
after trauma, 453, 533, 534f post-migration disorders
Bladder polymicrogyria, 67–68, 85
agenesis, 762, 763f schizencephaly, 58t, 64, 67–69, 70f
angiosarcoma, 823–824 ventral induction disorders
bladder diverticula, 758, 759f corpus callosum
bladder ears, 740, 740f agenesis, 65–67, 66f
bladder exstrophy, 759–760, 760f callosal dysgenesis, 65, 67f
cloacal exstrophy, 759–760 lipoma of, 67, 67f
cloacal malformation, 760–761, 761f Dandy-Walker syndrome, 58t, 65, 67–68, 67f
duplication, 761–762, 762f holoprosencephaly, 63–64, 64f
fibroepithelial polyp, 818, 819f alobar, 64, 64f
inflammatory myofibroblastic tumor, 818–819, 819f lobar, 64
leiomyoma, 819 semilobar, 64
leiomyosarcoma, 823 septo-optic (pituitary) dysplasia, 64–65, 65f
lymphatic malformation, 816, 817f extracranial birth trauma, 95, 95f
megacystitis-microcolon-intestinal hypoperistalsis syndrome, 763 cephalohematoma, 93, 95, 95f
nephrogenic adenoma, 820–821, 822f subgaleal hematoma, 95, 95f
neurofibroma, 819, 820f hydrocephalus, 90, 92, 92f
neurogenic bladder, 749f, 754, 767, 767f, 800, 824 hypoxic-ischemic injury, 75–84
normal anatomy, 738–740 infectious disorders
papillary urothelial neoplasm of low malignant potential, 818 bacterial infections, 86–88, 87f–88f
paraganglioma, 819–820, 821f fungal infections, 88
prune-belly syndrome, 762–763, 763f viral infections, 84–86, 85f–86f
rhabdomyosarcoma, 821–822, 822f intracranial hemorrhage, 70–75
transitional cell carcinoma, 822–823, 823f neoplasms, of neonatal/infant brain and ventricle, 88–89, 89f
trauma, 802, 802f, 803 neuronal proliferation disorders, 68
urinary diversion, 824–825, 825f hemimegalencephaly, 68
urothelial papillomas, 818 normal anatomy
venous malformation, 816, 817f, 818 basal ganglia, 58–59, 59f
Blooming artifact, 46–47, 46f, 73f cerebral cortex, 58, 59f
Blue rubber bleb nevus syndrome (BRBNS), 250, 327, 515f, 542, 545f cisterna magna, 60–61, 61f
of gastrointestinal tract, 250, 327 connatal cysts, 59–60, 60f
of mesentery, 545f extra-axial fluid spaces, 61
of peritoneal cavity, 542 forebrain structures, 57–58
Index 973

hindbrain structures, 57–58 vascular malformation(s), 954

mega cisterna magna, 60–61, 61f venous malformation, 954–955
midbrain structures, 57–58 normal anatomy, 942f, 946–947, 946f
thalami/thalamus, 59, 59f normal development, 942–943, 943f–945f, 944t
ventricles, 59–60, 59f–60f Tanner staging, 943, 944t, 945f–946f, 946–947
normal development, 57, 58f, 58t technique
cerebral cortical formation, 57 imaging approaches, 941–942
primitive streak, 57 patient positioning, 941
scalp masses (see Scalp masses) ultrasound transducer selection, 941
suture evaluation Bronchogenic cyst(s), 136, 179, 190, 229, 229f, 279, 285
craniosynostosis, 61, 97–99, 98f Bronchopulmonary sequestration
plagiocephaly, positional, 97, 99 extralobar bronchopulmonary sequestration, 182
technique hybrid congenital lung anomalies, 183
imaging approaches, 52–53, 53f–57f intralobar bronchopulmonary sequestration, 181–182, 182f
patient positioning, 52 Budd-Chiari syndrome, 383, 383f
ultrasound transducer selection, 52 Burkitt lymphoma
vascular disorders breast, 962
high-flow malformations, 89–90, 91f chest wall, 261f
vein of Galen malformation, 90, 91f Epstein-Barr viral infection and, 498
low-flow malformations, 89–90 GI tract, 301, 303f, 329f, 329–330, 333, 345, 346f
Branchial cleft cyst(s), 133–134, 134f, 135f, 163 kidney, 814f
BRBNS. See Blue rubber bleb nevus syndrome mediastinum, 226
Breast neutropenic colitis and, 342
accessory breast tissue, 947, 947f ovary, 711, 711f
benign tumors, 955t pancreas, 585, 586f
desmoid, 959–960, 959f peritoneal cavity, 551, 552f
fibroadenoma, 955, 956f testicle, 668
granular cell, 960, 960f Byler disease, 469
hemangioma, 955–956, 957f
intraductal papilloma, 956, 957f
juvenile papillomatosis, 957, 958f C
lactating adenoma, 958–959 CAH. See Congenital adrenal hyperplasia
pseudoangiomatous stromal hyperplasia, 957–958, 958f Callosal agenesis, 66
congenital anomalies Campylobacter, 316, 340
amastia, 947 Canal of Nuck disorders
amazia, 947 differential diagnosis, 725
athelia, 947 hernias, 726, 726f
hypomastia, 947 hydrocele, 725–726, 725f
Poland syndrome, 947 Capillary malformation–arteriovenous malformation (CM-AVM)
polymastia, 947 syndrome, 140
polythelia, 947 Carcinoid tumor, 323, 333, 669
developmental anomalies Carcinoma
gynecomastia, 948–949, 949f acinar cell, 166–167, 167f, 584–585
juvenile hypertrophy, 948 adenoid cystic, 167, 167f
premature thelarche, 948, 948f adrenocortical adenoma and, 597, 597f–598f
imaging field of view, 941–942 clear cell adenocarcinoma, 722–723
inflammatory lesions embryonal, 663–664, 706
abscess, 949–950, 950f fibrolamellar hepatocellular, 401, 401f
mastitis, 949, 950f hepatocellular, 400, 400f
malignant tumors, 955, 955t mucoepidermoid, 166, 166f
angiosarcoma, 962 primary gastric adenocarcinoma, 303
carcinoma, 961–962, 962f renal cell, 778–779, 804, 810–811
and prior mantle radiation for Hodgkin lymphoma, 961 renal medullary, 812
cystosarcoma phyllodes, 960–961, 961f transitional cell, 822–823, 823f
hematologic malignancies, 962–963, 963f–964f urothelial, 818, 822–823, 823f
metastases, 963, 964f Cardiophrenic angle masses
non-neoplastic lesions lymphadenopathy, 235–236, 236f
fat necrosis, 953–954 pericardial cyst, 235, 235f
fibrocystic disease, 951–952, 952f Caroli disease, 373f, 457, 458f, 459–460, 462f, 469, 474
galactocele, 952–953, 953f Cartilage. See Bones
hematoma, 953, 953f Castleman disease, 549–550, 549f
intramammary lymph node, 954, 954f CAT. See Cutaneovisceral angiomatosis
lymphatic malformation, 955 with thrombocytopenia
mammary duct ectasia, 950, 951f Cat-scratch disease
retroareolar cysts (obstructed glands of Montgomery), of cervical lymph nodes, 146, 146f
950–951, 951f of spleen, 494
974 Index

Cauda equina enlarged rib end, 253, 254f, 255

imaging, 104–105, 105f, 107f osteochondroma/exostosis, 251–252, 252f
positional nerve root clumping (“pseudomass”), 110 technique
and tethered cord, 116 contrast-enhanced ultrasound, 241
Caudal regression syndrome, 103, 110, 118–119, 119f imaging approaches
Cavitation. See Nonthermal bioeffects annotations, 241
Cellulitis, 146, 255, 255f–256f, 838–839, 839f, 949 protocols, 241
Cephalocele, 67, 93–94 patient positioning, 239
Cephalohematoma, 93, 95, 95f ultrasound transducer selection, 239–240
Cerebellar hemorrhage, 53, 72, 74f traumatic disorders
and ECMO, 53, 72 hematoma, 262–264, 263f
Cerebral cortex, 58, 68, 74–75, 77 rib fractures, 264–265, 264f
Cerebrospinal fluid (CSF) Chiari malformation(s), 61–63, 62f, 65, 113, 121
cerebrospinal fluid-containing defects, 116 Chiari I, 61, 62f
cerebrospinal fluid pseudocyst, 532–533, 533f Chiari II, 61–63, 62f
Cervical adenitis, 144–145, 144f. See also Lymphadenopathy and myelomeningocele, 62, 62f
Cervical extension of normal mediastinal thymus, 138 Child abuse. See also Non-accidental trauma; Trauma
Cervical teratoma, 136–137 distal humeral epiphyseal separation and, 865f
Cervical thymic cyst, 134 portal venous gas and, 386
Cervix rib fracture and, 264
atresia, 698 Childhood ILD, 185
benign tumors Children’s Oncology Group (COG)
nabothian cyst, 719, 719f and neuroblastoma management, 233, 586
ectopic pregnancy and, 715 and Wilms’ tumor management, 810
malignant tumors Chimney phenomenon, 240t, 264
clear cell adenocarcinoma, 723 Chocolate cysts, 705
rhabdomyosarcoma, 720, 720f, 722 Cholangiocarcinoma, 458–459, 474
normal anatomy, 689 Cholecystitis
normal development, 689 acute acalculous, 447, 448f
PID and, 716, 716f acute calculous, 444–447, 445f–446f
CEUS. See Contrast-enhanced ultrasound gangrenous, 446, 446f
Chest wall chronic, 449, 449f
benign neoplasms porcelain gallbladder, 449, 450f
lipoma, 257–258, 257f–258f Choledochal cyst(s), 457–459, 570
mesenchymal hamartoma, 257–258 Choledocholithiasis, 441, 469, 469f, 470, 472
congenital anomalies Cholelithiasis, 433, 438, 440–442, 441f–442f, 445, 447, 449, 471
vascular tumors and malformations Cholesterol polyp(s), 450–452, 451t, 452f
clinical and ultrasound features, 248, 248t Choriocarcinoma, 662, 664, 706, 708–709, 948
hemangioma, 248–249, 249f Choroid plexus tumors, 88–89, 89f
LUMBAR syndrome, 248 Chronic granulomatous disease, 298, 298f, 494, 649, 678
PHACE syndrome, 248 Chronic kidney disease (CKD), 731, 768t, 781, 785–786, 882
ISSVA classification, 247–248, 248t Chylous effusion/chylothorax, 206–207
lymphatic malformation, 250, 250f–251f Circumcaval ureter, 752. See also Retrocaval ureter
venous malformation, 250–251, 250f Cirrhosis
foreign bodies, 265–266, 265f–266f biliary, 438f, 443t, 459, 462, 466f, 468–469, 471
infectious disorders of liver, 356t, 359f, 359t, 366, 371–372, 374–375, 375f, 382, 382t,
abscess, 256, 256f 487t
cellulitis, 255, 255f and ascites, 529f, 529t
lymphoma, 261–262 and Budd-Chiari syndrome, 383, 383t
malignant neoplasms compared to chronic hepatosplenic schistosomiasis, 500
Ewing sarcoma, 259–260, 260f hyposplenia and, 492
lymphoma, 261f, 261–262f and passive venous congestion, 386
metastases, 262, 262f and portal hypertension, 508, 509f
osteosarcoma, 260–261, 260f and primary bacterial peritonitis, 536
rhabdomyosarcoma, 258–259, 259f Cisterna magna, 55f, 57f, 60–61, 61f–62f, 67, 90
neoplastic disorders, 256–262 CKD. See Chronic kidney disease
normal anatomy Classic metaphyseal lesion (CML), 852, 853f
musculature, 244–246, 245f–246f Clear cell sarcoma, 811–812, 812f
thoracic skeleton, 243–244, 244f Cloacal malformation(s), 702, 761
ultrasound appearance, 246–247, 246f–247f Cloacal exstrophy, 759
normal development exstrophy-epispadias complex and, 758
soft tissues, 242–243, 243f terminal myelocystocele and, 116
thoracic skeleton, 241–242, 242f–243f Closed defects
osseous and cartilaginous lesions complex, of spine, 114–116
costochondral junction, asymmetric cartilaginous, 252–253, simple, of spine, 113–114
253f–254f CML. See Classic metaphyseal lesion
Index 975

Coccyx Comet-tail artifact, 38

dysmorphic, 110, 111f Common carotid artery (CCA), 148f, 900, 900f
Colloid cysts, 152, 152f Compression. See also Elastography; Elastography imaging
Colon of abscess, 256, 256f
benign masses of appendix, 330, 331t
duplication cyst, 345 of arteriovenous fistula, 917
juvenile polyp, 344–345, 345f of breast, imaging approaches, 942
and juvenile polyposis syndrome, 345 of colon, 334
congenital anomalies of extremity veins, 919–920, 925, 926f
anorectal malformations, 335–336, 336f–337f extrinsic, of ureter, 767
and association with VACTERL, 335 of soundwave, 3, 3f, 4
malignant tumors Congenital adrenal hyperplasia (CAH)
adenocarcinoma, 345, 347, 347f adrenal rests in, 592, 592f, 593, 593f, 660–661, 661f, 662t, 670
lymphoma, 345, 346f and disorders of sex development, 702, 702f
normal anatomy, 335 and myelolipoma, 607
normal development, 291f, 334–335, 733f Congenital anomalies, of breast
technique amastia, 947
imaging approaches, 334 amazia, 947
patient positioning, 334 athelia, 947
ultrasound transducer selection, 334, 334f hypomastia, 947
wall thickening Poland syndrome, 947
Crohn disease, 339–340, 339f polymastia, 947
cystic fibrosis, 343 polythelia, 947
hemolytic–uremic syndrome, 343, 344f Congenital benign cystic neck masses
infectious colitis, 340–341, 340f–341f branchial cleft cyst, 133–134, 134f, 135f
necrotizing enterocolitis, 337, 338f cervical thymic cyst, 134–136, 135f
neutropenic colitis, 342–343, 342f dermoid cyst, 136–137, 137f
pseudomembranous colitis, 341–342, 341f foregut duplication cyst, 136, 136f
ulcerative colitis, 337–338, 338f teratoma, 136–137, 137f
Color Doppler ultrasound, 27, 31, 31f. See also Doppler ultrasound; thyroglossal duct cyst, 133, 133f–134f
Ultrasound artifacts; Ultrasound safety Congenital benign solid neck masses
and brain neoplasms, 88 cervical extension of normal mediastinal thymus, 138, 138f
and bronchopulmonary sequestration, 181 ectopic thymus, 138, 138f
differentiation from neuroblastoma, 233 fibromatosis colli, 138, 139f
and congenital portosystemic shunts, of liver, 371f Congenital diaphragmatic anomalies, 274–278
and cortical veins of brain, 61, 93f Congenital diaphragmatic eventration, 277
and Crohn disease, 317, 318f Congenital diaphragmatic hernia (CDH), 274, 274f, 274t, 275f, 294,
differentiation of empyema from lung abscess, 185f 310, 438f, 490, 638
and epididymo-orchitis, 644, 645f Congenital goiter, 158f
and infantile hemangioma of breast, 955–956, 957f Congenital hemangioma
and pleural effusion, 202 non-involuting congenital hemangioma
in pneumonia, 177f of liver, 388t, 389
and portal vein thrombosis, 382, 418, 419f of skin/subcutaneous tissues, 248–249, 248t, 890, 890t
compared to power Doppler, 32 partially involuting congenital hemangioma
and “ring of fire” sign, in ectopic pregnancy, 715, 715f of liver, 388t, 389, 390f
and soft tissue vascular anomalies, 890, 891f–892f, 893, 894f of skin, subcutaneous tissues 248–249, 248t, 890, 891f
TCD, 82–84, 83f–84f, 83t peritoneal cavity, 517
in testicular torsion, 641, 641f rapidly involuting congenital hemangioma
and “thyroid inferno”, 159–160, 160f of liver, 388t, 389, 389f
and tissue vibration artifact, 45, 784, 785f of skin/subcutaneous tissues, 248–249, 248t, 890, 891f
and twinkling artifact, for diagnosis of urinary tract stones, Congenital knee dislocation, 882–883, 883f
799–800, 801f Congenital lobar hyperinflation, 178, 178f
and ureteral jets, 738, 739f Congenital megacalyces, 748, 749f
and varicocele, 659–660, 659f–660f Congenital non-vascular neck masses. See Congenital benign cystic
and vascular chest wall lesions, 247–251, 248t, 249f–251f neck masses; Congenital benign solid neck masses
and vascular neck masses, 138, 140, 141f–144f, 166 Congenital patellar dislocation, 883, 884f
and vascular scalp lesions, 95–96, 97f Congenital pulmonary airway malformation (CPAM),
in vein of Galen malformation, 91f 180, 180t, 181f
and venous malformation of bladder, 816, 817f Congenital ureterovesical junction obstruction (UVJO), 751, 751f
in venous sinus thrombosis, 81, 82f Congenital vascular neck masses
and “whirlpool” sign of midgut volvulus, 310, 311f congenital hemangioma, 138
and “yin-yang” pattern of pseudoaneurysm, 918t vascular malformations, 140
of hepatic artery, in liver transplantation, 408, 418, 418f arteriovenous fistula, 140, 142f
of peripheral arteries, 916, 917f arteriovenous malformation, 140, 141f
of renal artery, 781, 783f lymphatic malformation, 140, 144, 144f
after renal transplant biopsy, 789, 791f venous malformation, 140, 143f
976 Index

Connatal cysts, 59–60, 60f Cystic fibrosis transmembrane regulator (CFTR), 321, 343
Constant-range (C) mode, 14. See also Image display Cystic partially differentiated nephroblastoma, 805
Continuous wave (CW) Doppler, 27, 29, 29f Cystosarcoma phyllodes, 960, 961f
Contrast agents. See Ultrasound contrast agents Cyst(s). See also Dermoid cyst(s); Duplication cyst(s); Epidermoid
Contrast-enhanced ultrasound (CEUS). See also Contrast-enhanced cyst(s); Lymphatic malformation(s); Pancreas, cystic
voiding urosonography; Ultrasound contrast agents neoplasms of; Pseudocyst(s); Polycystic liver disease; Renal
in Crohn disease, 339, 339f cystic disease; Venous malformation(s)
in fungal abscesses adrenal, 594, 594f
of kidney, 771f arachnoid
of spleen, 495–496, 496f epidermoid/dermoid cyst differentiated from, 122
in IBD, 317 intraoperative spine ultrasound and, 121
of liver, 358, 358f, 388t mega cisterna magna differentiated from, 61
of lung, 174 myelomeningocele and myelocele, associated with, 113
of lymphatic malformation, intraperitoneal, 544f Baker, 881, 881f
of mesenteric hemangioma, 328f Bartholin, 721
in necrotizing enterocolitis, 337 branchial cleft, 133–134, 134f–135f
of pericardial cyst, 235f breast
of pleura, 204 cystosarcoma phyllodes and, 960
of scrotum, 629 fat necrosis and, 953–954
in trauma fibrocystic disease and, 951–952, 952f
of bladder, 802, 802f galactocele, 952–953, 953f
of kidney, 730, 801, 802f juvenile papillomatosis and, 957, 958f
of pancreas, 577–578, 577f retroareolar, 950–951, 951f
of urogenital sinus, 702 Byler disease, 443t, 469
Contrast-enhanced voiding urosonography (CeVUS), 730–731, 740f, of the canal of Nuck, 726
755, 802 Caroli disease, 459–460, 462f
Contrast-to-noise ratio, spatial compounding and, 24–25, 24f cervical thymic, 134–136, 135f
Corpus callosum, 53, 53f–55f, 58f, 64–66, 66f–67f choledochal, 443t, 457–459, 458f–461f, 568f
anomalies of, 65–67, 66f–67f congenital pulmonary airway malformation and, 180, 180t, 181f
agenesis, 65–67, 66f connatal, 59–60, 60f
callosal dysgenesis, 65, 67f daughter
lipoma of, 67, 67f in Echinococcosis, 379–380, 379f, 499, 772
Couinaud classification system in functional ovarian cyst, 703, 704f
and anatomic variants of bile duct anatomy, 456 of diaphragm, 269f, 279–280, 279f, 279t, 535, 535f
of liver anatomy, 364, 364f, 366f, 367 filar, 109, 109f
and liver transplantation techniques, 409f, 410, 411f, 414f foregut duplication
CPAM. See Congenital pulmonary airway malformation of GI tract, 285, 298–300, 324–326, 325f, 345
Cranial ultrasound. See Brain of mediastinum, 179–180, 179f–180f, 225t, 229–231,
Craniocervical junction, 52, 104, 113 229f–230f
Craniosynostosis, 61, 97–99, 98f of neck, 136, 136f
Crohn disease ganglion, 868, 868f
colon, 339–340, 339f Gartner duct, 720–721, 721f
small bowel, 317, 318f inclusion
Cryptorchidism, 493, 636, 636f, 640, 653, 660, 763 epidermal
CSF. See Cerebrospinal fluid of peritoneum, 535, 535f, 712, 712f
Curvilinear arrays, 22 of vagina, 721
Cutaneovisceral angiomatosis with thrombocytopenia intramuscular, sequela of hematoma, 263
(CAT), 328 of kidneys, 774–779
CW Doppler. See Continuous wave Doppler echinococcal (hydatid), 772
Cystic dysplasia of rete testis, 638, 660–661 in setting of posterior urethral valves, 764, 765f
Cystic fibrosis (CF) liver, 369, 369f
of appendix, 332, 332f autosomal dominant polycystic kidney disease and, 369–370, 370f
cholecystitis, chronic, and, 449 echinococcal (hydatid), 378–380, 379f
cholelithiasis and, 440, 441f, 445 mesenchymal hamartoma, 392–393, 392f
of colon, 343 in mesoblastic nephroma, 803–804
gallbladder hypoplasia and, 436, 438f müllerian duct, 675
Gamna-Gandy bodies and, 524f in multilocular cystic renal tumor, 805f
gastrointestinal tract and, 321–322, 322f, 343 nabothian, 719, 719f
kidney stones and, 800 neurenteric, 112t, 114–115
of liver, 356t, 359f, 372, 382t, 385, 529f omental, 540
meconium ileus and, 311 ovarian
meconium peritonitis and, 537, 653 chocolate, 705
pancreas and, 570, 570f, 576 dermoid, 706, 706f–707f
seminal vesicle agenesis and, 677 functional, 703–704, 703f–704f
sialadenitis, chronic, and, 165 hemorrhagic, 703–704, 704f
sialolithiasis and, 167 torsion and, 713
Index 977

pancreatic, 569–570 Diaphragm

autosomal dominant polycystic kidney disease and, 571, 571f acquired diaphragmatic disorders
“cystosis” in cystic fibrosis, 570, 570f diaphragmatic dysfunction, 278–279, 278f
VHL disease and, 571–572 diaphragmatic inversion, 279, 279f
parameniscal, 886, 886f anatomy, 276, 276f
paraovarian, 711–712, 712f congenital diaphragmatic anomalies
parathyroid, 161–162, 162f Bochdalek hernia, 274–275, 275f–276f
paraurethral, 721–722, 722f diaphragmatic hernia, 274, 274f, 274t
peliosis hepatis, 385, 385f hiatal hernia, 275, 277, 277f
peliosis, splenic, 515–516 Morgagni hernia, 275, 276f
pericardial, 235, 235f eventration, 277–278, 277f
peritoneal normal anatomy, 273, 273f
inclusion, 535, 535f, 712, 712f normal development, 272, 272f
mesenteric, 326, 326f primary diaphragmatic masses
pilonidal, 120–121, 121f benign, 279–280, 279f, 280t
pleuropulmonary blastoma and, 189, 190f, 211–212, 212f malignant, 280, 280f, 280t
in polycystic ovary syndrome, 724, 724f technique
porencephalic, 72, 73f imaging approaches, 272
and PVL, 76–77, 77f–78f patient positioning, 271
scrotal, extratesticular ultrasound transducer selection, 271–272
dermoid, 672 traumatic diaphragmatic rupture, 280–281
epididymal, 657, 669–670, 670f Diaphragmatic inversion, 279, 279f
spermatocele, 669, 669f Diaphragmatic paralysis, 277–278
seminal vesicle, 675, 677f Diastematomyelia, 113–115, 115f
splenic, 487t,493, 493f DICER1 gene, 189, 211, 392, 805
echinococcal (hydatid),499 DICER1 mutation, 189, 190f, 551, 805
subependymal, 84, 85f DICER1 syndrome, 211, 711
sublingual, 163, 163f Diffuse parenchymal disease, of liver
testicular, 657, 661–662, 661f, 662t cirrhosis, 374–375, 375f
cystic dysplasia of rete testis and, 638 fibrosis, 372–374, 372f–373f
dermoid, 666 hemochromatosis, 374, 374f
epidermoid, 662t, 666, 667f nonalcoholic fatty liver disease, 371–372, 372f
thyroglossal duct, 133, 133f–134f Diffuse parenchymal lesions, of thyroid, 157–159
thyroid DIOS. See Distal intestinal obstruction syndrome
colloid, 152, 152f Discoid meniscus, 885, 885f, 886
hemorrhagic, 153f Disease-modifying anti-rheumatic drugs (DMARDS), 855
simple, 152, 152f Disorders of sex development (DSD)
and tight filum syndrome, 114 cloacal malformation, 702
urachal, 756, 758, 757f–758f congenital adrenal hyperplasia, 702, 702f
utricle, 675, 676f nomenclature and classification, 701
sex chromosome
45,X, 701, 701f
D 46,XX, 701
Dandy-Walker syndrome, 58t, 61, 65, 67–68, 67f Turner syndrome, 701, 701f
DDH. See Developmental dysplasia of the hip Distal intestinal obstruction syndrome (DIOS), and cystic
Deep vein thrombosis (DVT) fibrosis, 321, 322f
acute, 923, 924f, 925–926, 925f Distance measurement, 8–11
causes, 923 Doppler angle, 21, 27–28, 28f, 29–32, 43–44
chronic (residual), 926–927, 926f Doppler artifacts. See Ultrasound artifacts
clinical evaluation, 923 Doppler effect, 26f, 27
Depth of penetration, 8–10, 10f, 11, 22, 24, 43, 220, 271, 836, 872 Doppler frequency shift, 27, 31, 43
Dermal sinus, 104, 110, 113–114, 115f, 116 Doppler modes, 27
Dermoid cyst(s) Doppler shift, 27, 27f, 28, 28f, 29–32, 43–44
of neck, 136–137, 137f Doppler ultrasound. See also Color Doppler imaging; Ultrasound
of ovary, 706, 706f–707f artifacts; Ultrasound safety
of paratesticular soft tissues, 672 blood flow detection, 27, 27f, 28, 28f, 357
of scalp, 93, 94f continuous wave Doppler, 27, 29, 29f
of spine, 114, 123f power Doppler, 31–32, 32f
of testicle, 666 pulsed wave Doppler, 27, 29–30, 29f–30f
Desmoid tumor, 548–549, 548f, 959, 959f, 960 source of error, 28
Desmoid-type fibromatosis, 959 Dorsal induction disorders
Desmoplastic small round cell tumor, 403, 475, 553, 554f anencephaly, 63, 63f
Developmental dysplasia of the hip (DDH) Chiari malformation(s), 61–63, 62f, 65, 113, 121
overview, 872–873 hydranencephaly, 63, 64f
imaging of, 873–874, 873f–875f, 874t “Double bubble” sign, of duodenal atresia, 290, 306, 307f, 308
treatment of, 875–876, 875f Down syndrome. See Trisomy 21
978 Index

Duodenal atresia, 290, 306, 307f, 308, 567f Embryonal rhabdomyosarcoma

Duodenal web, 306, 307f of biliary tree, 403
Duplication cyst(s) of bladder, 822
of colon, 345 Empyema
of esophagus, 225t, 230f, 285 of brain, 87, 87f
of mediastinum, 179–180, 179f–180f, 225t, 229, 229f, 231, 285 of pleural cavity, 202–204, 203f–204f
of neck, 136, 136f Endometrioma 698, 705. See also Chocolate cysts
of small bowel, 314–315, 324–326, 325f End-stage renal disease (ESRD), 775, 785f, 786
of stomach, 298, 299f–300f, 300, 567 Energy/amplitude Doppler, 31
Dynamic elastography, 34 Enhancement artifact, 39–40, 40f
Dynamic range, 13, 13f, 47 Enlarged rib end, 253, 254f, 255
Dysgenesis Entamoeba histolytica infection, 378
callosal, 62, 65, 67–68, 86 Eosinophilic gastroenteritis, 297, 320–321
gonadal, 663, 701, 705t, 726f Epidermoid cyst(s), 93, 94, 94f, 114, 122–123, 136–137, 137f
and gonadoblastoma, 707 of canal of Nuck, 725
of kidney with seminal vesicle cyst, 675 of paratesticular soft tissues, 672
segmental spinal, 119 of scalp, 93–94
of thyroid gland, 151–152, 157 of skin, 845
Dysgerminoma, 706–707, 708f, 709 of spine, 114, 123f
Dyshormonogenesis, of thyroid gland, 152, 158 of spleen, 493, 493f
of testicle, 662t, 666, 667f
of vagina, 721
E Epididymitis, 642, 643, 643f, 662, 670, 678
ECA. See External carotid artery acute, 644
Ecchinococcal infection, 378–379, 499, 594, 772. See also Hydatid chronic, 649
disease Epididymo-orchitis, 639, 639t, 644, 645f
ECMO. See Extracorporeal membrane oxygenation Henoch-Schönlein purpura and, 646, 647f
Ectopia intratesticular varicocele and, 660
crossed renal, 744, 745f, 747, 764 segmental testicular infarction and, 641–642
gallbladder, 437–438 testicular abscess and, 645
renal, 744, 745f Epidural abscess, 120
testicular, 636f, 637 Epidural hemorrhage, 72–73
thyroid, 151 Epiploic foramen, 438, 525, 526f
of ureter, 752 Epithelioid hemangioendothelioma (EHE), 402
Ectopic pregnancy, 530, 709, 715–716, 716f Epstein-Barr virus
Ectopic thymus, 138, 219, 223 Hodgkin lymphoma and, 226
Ectopic thyroid, 133, 151–152, 151f inflammatory myofibroblastic tumor and, 516
Ectopic ureter, 644, 720–721, 743, 744f, 752, 752f–753f, 770f, 799f, primary hepatic lymphoma and, 404
824 PTLD and, 405–407, 405f
EHE. See Epithelioid hemangioendothelioma smooth muscle tumors and, 621
Elastography, 2, 32–33, 33f, 34–35, 34f–35f, 317, 339, 359, 359t, 360f, and splenomegaly, 498, 498f
373, 373f, 382, 386, 411, 413f, 508, 629, 632, 641, 645, Esophageal duplication cyst, 229, 285
659, 662–663, 666, 668, 731, 942 Esophagus
Elastography imaging gastroesophageal reflux, 286, 287f
dynamic elastography, 32, 34–35, 34f–35f hiatal hernia, 286, 288, 288f
acoustic radiation force impulse imaging, 34, 34f imaging approaches, 284
and liver fibrosis, 359, 359f normal anatomy, 285–286, 285f–286f
shear wave speed imaging, 34 normal development, 284, 284f
supersonic shear wave elastography, 35, 35f technique
quasi-static strain elastography, 32–34, 33f patient positioning, 284
Elbow ultrasound transducer selection, 284
annular ligament, 862, 862f imaging approaches, 284
congenital radial head dislocation, 863–864, 864f Estimated glomerular filtration rate (eGFR), 784
fat pads, 862, 863f Ewing sarcoma
imaging approaches, 860–862, 860f–862f and breast metastases, 963
joint effusion, 862, 863f of chest wall, 240t, 259–260, 260f
normal anatomy, 860, 860f of diaphragm, 279t, 280
patient positioning, 860 and peritoneal metastases, 555f
trauma of retroperitoneum, 621
apophyseal avulsion, 864, 865f Exostosis, 251, 853
assessment, 864 External carotid artery (ECA), 131, 900f, 901–902, 904f
distal humeral epiphyseal separation, 864, 865f Extra-axial fluid spaces, 61
pulled elbow, 866, 866f Extracranial birth trauma, to cranial vault and scalp, 95, 95f
Embryonal carcinoma Extralobar bronchopulmonary sequestration, 181–183
of ovary, 706, 709 Extramedullary hematopoiesis
of testicle, 662, 664 mediastinum, 225t
Index 979

retroperitoneum, 614 normal development

spleen, 516, 517f external genitalia, 688
thalassemia major and, 505 gonads and reproductive tract, 686–688
Extramedullary tumors, of spine, 122–123 ovarian masses, 705t
Extracorporeal membrane oxygenation (ECMO) endometrioma, 698, 705
and imaging of neonatal and infant brain, 53, 54, 72, 75 epithelial tumor(s)
Extrapleural hematoma, 206 borderline epithelial tumor and cystadenocarcinoma, 710
cystadenoma, 709, 709f
functional cyst, 703–704, 703f–704f
F germ cell tumors, 705–706
Fallopian tube, 593, 637, 687f, 687–690, 693, 701, 714, 714f, 715 choriocarcinoma, 708–709
Far-field, of ultrasound beam, 15–18, 15f–17f. See also Fraunhofer zone dysgerminoma, 707–708, 708f
in brain imaging, 53, 54f–55f gonadoblastoma, 706–707
FAST, See Focused assessment with sonography for trauma mixed germ cell, 709, 709f
Fat necrosis, 840–841, 841f, 953–954 teratoma, 706, 706f–707f
Fatty filum, 109, 110, 110f, 119 yolk sac, 708, 708f
Fecalith, in acute appendicitis, 331, 332f secondary tumors, 711
Female genital tract stromal tumors
adnexal masses, 703 juvenile granulosa cell, 710–711, 710f
adnexal torsion, 688t, 713, 713f Sertoli-Leydig cell, 711
isolated tubal torsion, 713–714, 714f thecoma-fibroma, 710
massive edema, of ovary, 714–715 paraovarian cysts, 711–712, 712f
amenorrhea pelvic inflammatory disease, 716, 716f–717f
polycystic ovary syndrome, 724, 724f peritoneal inclusion cysts, 712, 712f
anatomic variants pubertal disorders
arcuate uterus, 692, 692f amenorrhea, 724, 724f
canal of Nuck disorders polycystic ovary syndrome, 724, 724f
hernia, 726, 726f precocious puberty, 723
hydrocele, 725–726, 725f technique
inferior epigastric vessels and, 725 three-dimensional ultrasound, 685f, 686
cervical masses transabdominal ultrasound, 683–684, 684f
benign tumors transperineal ultrasound, 686, 686f
nabothian cyst, 719, 719f transvaginal ultrasound, 684, 685f
malignant tumors uterine masses
rhabdomyosarcoma, 720, 720f benign masses
congenital anomalies adenomyosis, 718–719, 719f
Müllerian duct anomalies, 692, 692t leiomyoma (fibroid), 717–718, 718f
Mayer-Rokitansky-Küster-Hauser syndrome, 693, 694f malignant tumors
Müllerian agenesis, 692–693 lymphoma, 719
uterine agenesis, 693, 693f vaginal foreign body, 723, 723f
disorders of lateral fusion vaginal masses
bicornuate uterus, 692f, 694, 695f, 696 benign masses
septate uterus, 692f, 694, 695f Bartholin cyst, 721
unicornuate uterus, 692f fibroepithelial polyp, 722
uterus didelphys, 692f, 696, 696f Gartner duct cyst, 720, 721f
disorders of vertical fusion inclusion cyst, 721
atresia of cervix or vagina, 698, 700 Müllerian papilloma, 722
imperforate hymen, 696–698, 698f paraurethral duct cyst, 721–722, 722f
OHVIRA syndrome (obstructed hemivagina and malignant tumors, 722f, 722–723
ipsilateral renal anomaly), 700–701, 700f clear cell adenocarcinoma and endodermal
transverse vaginal septum, 698, 699f sinus tumor, 722–723
disorders of sex development rhabdomyosarcoma, 722, 722f
cloacal malformation, 702 Femoral vein, 914, 916, 919, 921f
congenital adrenal hyperplasia, 702 Fetal goiter, 158
definition, 701 Fetal hypothyroidism, 157–158
diagnosis, 701 Fetal neuroblastoma, 601–602
nomenclature and classification, 701 Fibroadenoma, of breast, 955, 956f, 957, 958f, 960
sex chromosome disorders, 701 Fibrocystic disease, of breast, 951–952, 952f
Turner syndrome, 701, 701f Fibroepithelial polyp
ectopic pregnancy, 715–716, 716f of bladder, 818, 819f
normal anatomy of gallbladder, 452
ovarian and uterine changes associated with menstrual cycle, of ureter, 815
690–691, 691f, 691t of vagina, 722
ovary and fallopian tube, 688–689, 688t, 689f Fibrolamellar hepatocellular carcinoma,
uterus and cervix, 689t, 690f 401, 401f, 587f
vagina, 689–690, 690f Fibromatosis colli, 138, 139f
980 Index

Fibromuscular dysplasia pulse repetition frequency, 9, 43f, 334, 900

renal artery stenosis and, 779, 780f Fresnel zone, of ultrasound beam, 15–16, 15f–16f. See also Near-field
Fibrosing mediastinitis, 232 FT. See Frame time
Fibrosis Fungal infection
bile ducts, 471 CNS, 88
fibrothorax, 204, 205f hepatic, 377f, 378, 770, 771f
hepatic, 34f, 359, 372–374, 413f, 459, 775 pulmonary, 232
of mediastinum, 232 urinary tract, 770, 772
periportal, 500 Fungal lymphadenopathy, 232
pleural, 205f
quadriceps tendon, 882
renal, 731 G
retroperitoneal, 613, 614 Galactocele, 952–953, 953f
Fibrothorax, 204, 205f Gallbladder
Fibrous pseudotumor, paratesticular, 670 anatomic variants, 436, 436f, 437f
Field of view (FOV) biliary dyskinesia, 447–449
extended, for bowel imaging, 318f cholecystitis
for breast imaging, 941, 961f acute acalculous, 447, 448f
for chest wall imaging, 241, 263f acute calculous, 444–447, 445f–446f
for spine imaging, 104, 107f gangrenous, 446, 446f
for vascular imaging, 899 chronic, 449, 449f
in image generation, 10f porcelain gallbladder, 449, 450f
of standard ultrasound probes, 22f cholelithiasis, 440–441, 441f–442f
in transducer selection, 52 “wall echo shadow” (WES) sign, of cholelithiasis, 441, 442f
Fifth ventricle. See Ventriculus terminalis congenital anomalies
Filar cyst, 109, 109f agenesis 436, 437f
Filar lipoma, 113–114, 114f, 119 duplication, 439–440, 440f
First branchial cleft cyst, 163 ectopia, 437–438, 438f
FNH. See Focal nodular hyperplasia hypoplasia, 436, 438f
Focal foveolar hyperplasia, of stomach, 300 septate gallbladder, 438–439, 439f
Focal hypoxic-ischemic injury triplication, 439, 440f
arterial ischemic stroke, 78, 80f, 81 hydrops, 449, 450f
sickle-cell disease, 81–84, 83f, 83t normal anatomy, 434, 435f–436f
venous sinus thrombosis, 81, 81f, 82 normal development, 434
Focal nodular hyperplasia (FNH), 370, 386, 393, 394f–395f polyps, 451t
Focused assessment with sonography for trauma (FAST), 508 adenomyomatosis, 451, 451f
extended (e)-FAST, 380 cholesterol, 451–452, 452f
Follicular adenoma, of thyroid, 152, 154, 154f inflammatory, 452
Fontan procedure, and associated liver disease, 386 other polypoid lesions, 452–453
Fontanelle(s) sludge, 442–444, 443f–444f, 443t
bulging, in setting of brain neoplasms, 88 and hepatization of gallbladder, 442, 444f
mastoid, 52, 57f, 72 technique
posterior, 52–53, 66f, 72 patient positioning, 433–434
scanning techniques, 25f imaging approaches, 434
Fontanelle, anterior ultrasound transducer selection, 434
imaging through, 52–53, 53f torsion, 450
Foregut duplication cyst(s), 136, 179–180, 229 trauma, 453–454, 454f
Foreign body(ies) varices, 453, 453f
in bladder, as risk factor for bladder stones, 800 Ganglion cyst, of wrist, 868, 868f
chest wall, 240t, 265–266, 265f–266f Ganglioneuroblastoma
scrotum, 656, 656f of adrenal, 599–600, 600f
shadowing attenuation artifact and, 40 of mediastinum, 225t, 233–234
soft tissues, 841 of retroperitoneum, 617–618, 619f
ultrasound-guided localization and removal, 266, 841 Ganglioneuroma
vagina, 723, 723f of adrenal, 598–599, 599f
Fournier gangrene, 649, 650f of mediastinum, 225t, 233–235
FOV. See Field of view of retroperitoneum, 617–618, 619f
FR. See Frame rate Gangrenous cholecystitis, 446, 446f
Frame rate (FR), 10–11, 25–26, 31, 34–35, 900 Gartner duct cyst, 720–721, 721f
Frame time (FT), 11 Gastric atresia, 290
Fraunhofer zone, of ultrasound beam, 15–16, 15f–16f. See also Gastric bezoar, 289, 301, 302f
Far-field Gastric diaphragm (antral web), 292
Frequency Gastric duplication cyst, 298–299, 567
acoustics 2, 2f Gastric lipoma, 300
Doppler shift, 27, 31, 43 Gastric teratoma, 300
linear array transducer, 52, 61, 92, 482, 564 Gastric volvulus, 294–296, 296f, 490
Index 981

Gastric wall thickening Gray matter heterotopia, 62, 64, 67–68, 69f. See also Migration
chronic granulomatous disease, 298, 298f disorders
eosinophilic gastroenteritis, 297–298 Grayscale artifacts
gastritis, 297, 297f enhancement, 39–40
Ménétrier disease, 297 mirror image, 37
Gastritis, 297, 297f, 300, 337 partial volume, 40–41
Gastroenteritis, 297, 316–317, 320–321, 330, 340–341 refraction, 37–38
Gastroesophageal reflux (GER), 275, 277, 284, 286, reverberation, 38–39, 38f–39f
287f, 290 shadowing attenuation, 40–41, 40f–41f
Gastrointestinal stromal tumor (GIST), 303, 304f side lobes, 39, 40f
of stomach, 303, 304f GVHD. See Graft-versus-host disease
metastases Gynecomastia, 661, 665, 948, 949, 949f, 957
to biliary tree, 475
to liver, 403
Gastrointestinal (GI) tract. H
appendix Harmonic imaging, 23f, 23–24, 36, 434, 482, 730
acute appendicitis, 331–332, 331f tissue harmonic imaging, 24
benign masses, 333 Hashimoto thyroiditis, 157–158, 160–161, 161f, 167f, 701
carcinoid, 333 HCC. See Hepatocellular carcinoma
cystic fibrosis, 332, 332f Hemangioma. See also Congenital hemangioma; Infantile
imaging, 330 hemangioma; Non-involuting congenital hemangioma;
lymphoma, 333–334 Partially involuting congenital hemangioma
normal anatomy, 330, 330f of neck, 138
patient positioning, 330 of testis, 667
ultrasound transducer selection, 330 Hemarthrosis, 855, 855f, 857f, 864, 865f
colon, 334–347 Hematocele, 642, 651–652, 652f, 654f, 655, 655f, 656
esophagus, 284–288 Hematoma
small bowel, 305–330 birth trauma-related, 93
stomach, 288–304. See also specific organs breast, 953
Gaucher disease, spleen and, 506–507, 506f–507f duodenal, 319, 319f
Genu recurvatum. See Congenital knee dislocation epidural, 95, 124, 124f
GER. See Gastroesophageal reflux extrahepatic, 423f
Germ cell tumors extrapleural, 206
primary gallbladder, 454f
of mediastinum, 225–226, 225t intramuscular, 263, 841–842
of ovary, 705–709, 705t, 706f–709f perinephric, 613f, 796
of testis, 662–664, 662t, 663f–664f perirenal, 802f
metastatic retroperitoneal, 613, 614f
to peritoneal cavity, 546t, 555 scalp, 95
to retroperitoneum, 620–621 spermatic cord, 659
GI tract. See Gastrointestinal tract splenic, 522
Giant cell tumor of the tendon sheath, of hand, 869, 869f subcapsular, 498, 509, 785
GIST. See Gastrointestinal stromal tumor subdural, 74, 93, 124
Glenohumeral dysplasia, 856, 858f–859f subgaleal, 95, 95f
Global hypoxic-ischemic injury testicular, 654–655
preterm infants, 76–77, 78f Hematomyelia, 124
term infants, 77–78, 79f–80f Hematopoiesis, in fetus, 516
Glomerulocystic kidney disease, 777 Hemimegalencephaly, 68. See also Neuronal proliferation
Goiter disorders
fetal, 158 Hemochromatosis, 374, 374f, 523
multinodular, 157 Hemolytic–uremic syndrome (HUS), 343, 344f, 785–786
Gonadoblastoma Hemorrhagic effusion, 205
of ovary, 705t, 706–707 Hemoperitoneum, 530, 531f
of testicle, 662–663, 662t Hemothorax, 202, 204–206, 252, 264–265, 279f, 508
Graft-versus-host disease (GVHD), of GI tract, Henoch–Schönlein purpura (HSP)
322–323, 323f and AKI, 785
Granular cell tumor and bowel wall thickening from hemorrhage,
of bile duct, 473 316, 319–320, 320f
of breast, 955, 955t and scrotum, 646, 647f–648f
of head and neck, 960, 960f and small bowel intussusception, 314
Granulomatosis with polyangiitis, 504, 537 Hepatic adenoma, 394–396, 396f–397f
Granulosa cell tumor Hepatic artery pseudoaneurysm, 418, 418f
of ovary, 690 Hepatic artery stenosis, 415, 417, 417f
of testicle, 665–666, 666f Hepatic artery thrombosis, 410, 415, 416f, 417
Graves’ disease, 157, 159–160, 160f Hepatic circulation, 366
fetal goiter and, 157 Hepatic mesenchymal hamartoma, 392
982 Index

Hepatic vein(s) of kidney, 772

Budd-Chiari syndrome, 383f, 383–384 of liver, 379, 379f
normal, 363, 366, 366f of spleen, 499
outflow obstruction, in liver transplantation, 420, 421f Hydranencephaly, 63, 64f
Hepatization Hydrocephalus. See also Non-communicating hydrocephalus
of gallbladder, 442, 444f in bacterial meningitis, 87
of lung, 176, 176f, 176t, 183 benign external hydrocephalus, 92–93, 93f
Hepatoblastoma, 190, 370, 397–398, 398f–399f, 400, 527f, in Chiari I malformation, 61
571, 581, 948 in Chiari II malformation, 62
Hepatocellular carcinoma (HCC), 236f, 262f, 370, 373–375, 386, 400, communicating, 90
400f, 401, 401f, 587f, 935 in Dandy-Walker syndrome, 67, 67f
Hereditary hemorrhagic telangiectasia, 140, 394 in fungal infection, 88
Herlyn-Werner-Wunderlich syndrome. See Obstructed hemivagina and in high-flow malformations, 90
ipsilateral renal anomaly non-communicating, 90
Herpes simplex virus, congenital, 595 obstructive, 72, 812f
Hiatal hernia, 274, 274f, 275, 277f, 284, 286, 288, 288f post-hemorrhagic, 70, 70t, 71f, 72
High frequency linear array transducer, 52, 61, 92, 482, 564 in toxoplasmosis, 85
High-intensity transient artifacts, 47 Hydromyelia, 113, 115–116, 125
Hindfoot. See Ankle Hydronephrosis
Hip bladder exstrophy and, 759
developmental dysplasia as cause of abdominal mass, 803
overview, 872–873 chronic rejection after renal transplantation and, 792
imaging of, 873–874, 873f–875f, 874t classification of, 746, 747f
treatment of, 875–876, 875f cloacal malformation and, 760
imaging approaches cloacal exstrophy and, 759
for hip dysplasia, 871–872, 871f crossed renal ectopia and, 744
for synovitis and effusion, 872, 872f ectopic ureter and, 752
normal anatomy, 869–870, 870f extrinsic ureteral obstruction and, 618f, 622f, 794, 794f, 796, 822
patient positioning, 870, 871f fungal infection and, 771
proximal focal femoral deficiency, 876, 876f as indication for urinary diversion, 824
septic arthritis, 877 posterior urethral valves and, 532f, 764–765, 764f–765f
slipped capital femoral epiphysis, 877–878, 877f retrocaval ureter and, 934
synovitis and effusion, 871–872 retroperitoneal fibrosis and, 613–614
transient synovitis, 877 UPJ obstruction and, 746–748, 748f
Histiocytosis ureteral extension of Wilms’ tumor and, 816
Langerhans cell UTI and, 768
of cervical lymph nodes, 146 xanthogranulomatous pyelonephritis and, 773, 774f
of lung, 186t Hydrops
of scalp, 93 of fetus, 614
sclerosing cholangitis and, 471 of gallbladder, 449, 450f
of spleen, 487t, 521–522, 521f–522f vein of Galen malformation and, 90
of testicle, 669 VUR and, 743, 754f, 794
sinus histiocytosis (Rosai-Dorfman-Destombes disease) Hyperparathyroidism
of neck, 146 parathyroid adenoma and, 162
of scrotum, 662, 662t parathyroid cyst and, 161
HIV. See Human immunodeficiency virus parathyroid hyperplasia and, 162, 162f
Hodgkin lymphoma. See also Burkitt lymphoma; Lymphoma; treatment of, 163
Non-Hodgkin lymphoma; specific organs Hyperplastic/adenomatoid nodules, of thyroid, 154
chest wall, 261 Hyperthyroidism
mediastinum, 226–227, 227f Graves’ disease and, 159–160, 160f
neck, 149–150 Hypertrophic pyloric stenosis (HPS), 292, 293f, 294–295, 386
retroperitoneum, 615 Hypertrophy, compensatory
secondary breast carcinoma and history of, 961–962 of accessory spleen, 490
spleen, 487t, 518–519, 519f–520f of kidney, 736, 736f, 776
thyroid, 157f of testis, 636
Holoprosencephaly, 63–64, 64f, 65, 67 Hypothyroidism
alobar, 64, 64f congenital, 151, 152
lobar, 64 delayed anterior fontanelle closure and, 52
semilobar, 64 fetal, 157, 158f
HPS. See Hypertrophic pyloric stenosis infantile hepatic hemangioma (IHH) and, 388t, 391
HSP. See Henoch–Schönlein purpura slipped capital femoral epiphysis and, 877
Human immunodeficiency virus (HIV), 84, 146, 375, 404, 472, 497, Hypoxic-ischemic encephalopathy (HIE), 75
501, 609 Hypoxic-ischemic injury, of brain, 75–84, 76f
HUS. See Hemolytic-uremic syndrome focal hypoxic-ischemic injury
Hyaline membrane disease, 186 arterial ischemic stroke, 78, 78f, 80f, 81
Hybrid congenital lung anomalies, 183 sickle cell disease, 81, 83–84
Hydatid disease venous sinus thrombosis, 81–82
Index 983

global hypoxic-ischemic injury Inflammatory polyps, 452

preterm infants, 76–78 Inflammatory pseudotumor, 550, 806
term infants, 77, 79–80 Infundibulopelvic stenosis, 748–749, 750f
Inspissated bile syndrome, 470–471, 470f
INSS. See International Neuroblastoma Staging System
I Instrumentation
IBD. See Inflammatory bowel disease array transducer, 19–22, 20f, 21f
ICA. See Internal carotid artery harmonic imaging, 23–24
Idiopathic scrotal edema, 646, 649, 649f image display, 14, 14f
IJV. See Internal jugular vein image storage, 14
ILD. See Interstitial lung disease mechanical transducer, 14–19, 15f–19f
Ileal atresia, 307–308, 312. See also Intestinal atresia receiver, 12–14
Ileal stenosis, 308–309 transducer, 11–12, 12f
Ileocolic intussusception, 314–316, 315f–316f, 329, 343 lead-zirconate-titanate, 4f, 12
Image display, 14, 46f transducer selection, 22–23, 22f
A-mode imaging, 14, 14f transmitter, 11–12
B-mode imaging, 14, 14f Intensity, of ultrasound, 7–8, 8f, 13–14, 18, 23, 23f, 39, 44, 48, 189,
C-mode imaging, 14 325–326, 400, 542, 551, 738
M-mode imaging, 14, 14f Internal carotid artery (ICA), 63, 83, 83f, 84, 900f, 902, 904f
Image storage, 14 Internal jugular chain lymph nodes, 129
IMT. See Intima-media thickness Internal jugular vein (IJV)
Inclusion cyst. See also Epidermoid cyst anatomic variants, 906
of peritoneum, 535, 535f, 712, 712f development, 906
of vagina, 721 jugular vein phlebectasia, 906, 908f
Indiana pouch, 824, 825f normal anatomy, 906, 907f
Inertial cavitation, 48 stenosis, 908
Infantile hemangioma thrombosis, 906, 908, 909f
of adrenal gland, 608 venous aneurysm, 908
of breast, 955–956, 955t, 957f International Neuroblastoma Risk Group Staging System (INRGSS),
of gastrointestinal tract, 327, 328f 586, 601t
of liver, 388t, 389, 391 International Neuroblastoma Staging System (INSS), 233, 601, 601t
of neck, 138, 139f, 140 International Society for the Study of Vascular Anomalies (ISSVA)
of pancreas, 579 classification
of peritoneal cavity, 542, 545, 546t chest wall vascular tumors, malformations and, 247–248, 248t
of retroperitoneum, 608, 617 gastrointestinal tract vascular anomalies and, 327
of salivary gland, 166 lymphatic malformations of mediastinum and, 227
of scalp, 95–96, 96f musculoskeletal system vascular anomalies and, 889, 890t, 892
of skin/subcutaneous tissues, 248–249, 889–890, 890f, 890t vascular disorders of brain and, 89
of spleen, 517 International Thymic Malignancy Interest Group (ITMIG), 221, 222f
Infantile hepatic hemangioma, 389, 391 Interstitial lung disease (ILD), 185–186, 186t, 187f, 503f
Infectious colitis, 340–341 Intestinal atresia, 307–308, 308f, 653
Infectious enteritis, 316–317 Intestinal polyp(s), 327, 327f
Infectious lymphadenopathy Intima-media thickness (IMT), 902, 903f
mediastinal, 231–232, 232f Intra-articular hemorrhage, 855, 855f
Inferior vena cava (IVC) Intracranial hemorrhage
anatomy, 931–933, 932f preterm infants
development, 931 cerebellar hemorrhage, 72, 74f
duplication, 934 neonatal periventricular/intraventricular hemorrhage, 70, 70t,
interruption, 932, 934f 71f, 72
left-sided, 934 Papile grading system, 70, 70t, 71f
May-Thurner syndrome, 936 periventricular hemorrhagic infarction, 72, 72f–73f
normal anatomy, 931–933, 931f–933f term infants
normal development, 931 epidural hemorrhage, 72, 74
retrocaval ureter, 933–934 parenchymal hemorrhage, 75
thrombosis, 934–936 subdural hemorrhage, 74–75, 74f
Inflammatory bowel disease (IBD), 297, 305, 317, 471, subpial hemorrhage, 75, 75f
475, 535, 612, 800. See also Crohn disease Intracranial lipoma, 67
Inflammatory myofibroblastic tumor Intraductal papilloma, 956, 957f
bladder, 818–819, 819f Intralobar bronchopulmonary sequestration, 181–182
kidney, 806, 807f Intramammary lymph node, 954, 954f
pancreas, 580 Intramedullary tumors, of spine, 122, 122f
peritoneal cavity, 546t, 550, 550f Intratesticular varicocele, 660, 660f
renal transplantation and, 797–798 Intusussusception
retroperitoneum, 622 ileocolic, 315–316, 315f–316f, 329, 343
scrotum, 673 small bowel, 313–315, 314f, 323–324, 327, 327f, 329f
spleen, 487t, 516–517 ISSVA. See International Society for the Study of Vascular Anomalies
stomach, 300–301 ITMIG. See International Thymic Malignancy Interest Group
984 Index

IVC. See Inferior vena cava Kasabach-Merritt phenomenon (KMP), 521, 587, 622, 891
Ivemark syndrome, 491–492 Kawasaki disease, 146, 503, 929, 931
KHE. See Kaposiform hemangioendothelioma
Kidney. See also Urinary tract
J abdominal masses, 803
Jaundice anomalies of renal collecting system
Alagille syndrome and, 468 calyceal diverticulum, 750, 751f
bile duct stricture and, 472 arteriovenous fistula, 783–784, 785f
bile duct tumors and, 473–474, 474f cystic disease
biliary atresia and, 463, 464f acquired cystic kidney disease, 779, 779f
Byler disease and, 469 autosomal dominant polycystic kidney disease,
Caroli disease and, 459 775, 775f
cholecystitis and, 444, 447 autosomal recessive polycystic kidney disease,
choledochal cyst and, 457 775–776, 776f
choledocholithiasis and, 469 classification, 774–775
cholelithiasis and, 440–441 family history, 775
cirrhosis and, 374 glomerulocystic kidney disease, 777, 778f
embryonal rhabdomyosarcoma and, 403 imaging, 774, 774f
fungal liver infection and, 378 multicystic dysplastic kidney, 736, 736f, 745f, 776–777,
hemoglobinopathies and, 505 776f–777f
hepatic artery pseudoaneurysm rupture and, 418 medullary cystic disease, 777
hepatoblastoma and, 397 nephronophthisis, 776–777, 777f
hepatocellular carcinoma and, 400 tuberous sclerosis, 778, 778f
inspissated bile syndrome and, 470 Von Hippel-Lindau disease, 778, 778f
lymphoma and, 404 medical renal disease
mesenchymal hamartoma and, 392 acute kidney injury, 784–786, 786f
Mirizzi syndrome and, 471 chronic kidney disease, 786
neonatal hepatitis syndrome and, 463 metastases
pancreatic tumors and, 579, 585–587 neuroblastoma, 814–815
PTLD of liver and, 406 osteosarcoma, 814, 815f
sclerosing cholangitis and, 471 pseudoaneurysm, 781, 783f
spontaneous perforation of extrahepatic bile ducts and, 472 renal artery stenosis, 779, 780f, 781
viral hepatitis and, 375 renal artery thrombosis, 781, 782f
Jejunal atresia, 308, 308f. See also Intestinal atresia renal vein thrombosis, 781–783, 784f
Jejunal stenosis, 308–309 trauma, 801–802
Jejunoileal atresia. See Intestinal atresia contrast-enhanced ultrasound diagnosis, 801–802, 802f
JIA. See Juvenile idiopathic arthritis tumors
Joint effusion. See specific joints angiomyolipoma, 804, 804f
Joints clear cell sarcoma, 811–812, 812f
hemarthrosis, 855, 855f imaging considerations, 803
juvenile idiopathic arthritis, 854–855, 855f inflammatory myofibroblastic tumor, 806, 807f
septic arthritis, 854, 854f lymphoma, 813–814, 814f
Jugular vein. See Internal jugular vein leukemia, 813, 813f
Jumper’s knee. See Sinding-Larsen-Johansson syndrome mesoblastic nephroma, 803–804, 803f
Junctional parenchymal defect, 735, 735f metanephric adenoma, 805, 806f
Juvenile fibroadenoma, of breast, 955 metastases
Juvenile granulosa cell tumor neuroblastoma, 814, 815f
of ovary, 710–711, 710f osteosarcoma, 814, 815f
of testicle, 665–666, 666f multilocular cystic renal tumor
Juvenile idiopathic arthritis (JIA) cystic nephroma, 805, 805f
joints and, 854–855, 855f cystic partially differentiated nephroblastoma, 805
spleen and, 503–504, 504f nephroblastomatosis, 810
Juvenile papillomatosis, of breast, 957, 958f nephrogenic rests, 810, 810f
Juvenile polyposis syndrome, 327, 344–345 ossifying renal tumor of infancy, 807
Juvenile polyp(s), 327, 344–345, 345f primitive neuroectodermal tumor, 812–813
Juvenile (virginal) hypertrophy, of breast, 948 renal cell carcinoma, 810–811, 811f
Juvenile xanthogranuloma, of scrotum, 670 renal medullary carcinoma, 812
sickle cell trait and, 812
rhabdoid tumor, 811, 812f
K atypical teratoid rhabdoid tumor of brain and, 811
Kaposiform hemangioendothelioma (KHE) primitive neuroectodermal tumor of brain and, 811
of musculoskeletal system, 891, 891f secondary tumors of, 711, 711f
of pancreas, 586–587 staging, 803
of retroperitoneum, 608, 622, 622f synovial cell sarcoma, 813
of spleen, 521 tumor invasion, 807
Kaposiform lymphangiomatosis (KLA), of spleen, 513, 515 Wilms’ tumor, 806–808, 810, 808f–809f
Index 985

nephrogenic rests, nephroblastomatosis and, 810, 810f peritoneal cavity, 546, 546t, 547f
renal vein and vena caval invasion by, 807, 809f soft tissue, 846
KLA. See Kaposiform lymphangiomatosis Lipomyelocele, 116, 117f
Klippel-Trenaunay syndrome, 90, 816, 817f Lipomyelomeningocele, 116, 117f
Knee Lissencephaly (agyria), 68. See also Migration disorders
alignment of, 878 Littoral cell angioma, 517–518
articulations, 878 Liver
Baker (popliteal) cyst, 881, 881f anatomic variants, 367–369, 368f, 368
Bipartite/multipartite patella, 884–885, 885f bacterial infection, 375–376, 377f
congenital knee dislocation (genu recurvatum), 882–883, 883f benign masses
congenital patellar dislocation, 883–884, 884f congenital hemangioma, 387, 387f, 388t, 389, 389f–390f, 391
discoid meniscus, 885–886, 885f–886f focal nodular hyperplasia, 388t, 393, 394f–395f
extensor mechanism, 879, 879f hepatic adenoma, 388t, 394–396, 396f–397f
imaging approaches, 878f, 879 infantile hemangioma, 388t, 391, 391f
joint effusion, 881, 881f mesenchymal hamartoma, 388t, 392–393, 393f
meniscal tears, 885–886, 885f–886f biliary atresia, 408
menisci, 880, 880f biliary complications of liver transplantation
normal anatomy, 878, 878f bile leak, 413, 414f
Osgood-Schlatter disease, 844f, 886 biliary stricture, 414, 415f
patellofemoral joint, 878f, 879, 880f blunt abdominal trauma, 380, 380f, 381f
patient positioning, 879, 879f Budd–Chiari syndrome, 383–384, 383f
Sinding-Larsen-Johansson syndrome, 886–887, 887f congenital anomalies
tibial hemimelia, 882, 882f congenital portosystemic shunts, 370, 371f
tibial tubercle, 880, 880f cysts, 369, 369f
Kocher criteria, for differentiating septic arthritis and transient polycystic liver disease, 369–370, 370f
synovitis of the hip, 877 contrast-enhanced ultrasound, 358, 358f
diffuse parenchymal disease
cirrhosis, 374–375, 375f
L fibrosis, 372–374, 372f–373f
Ladd bands, 309, 309f, 312f hemochromatosis, 374, 374f
Langerhans-cell histiocytosis (LCH), 521–522, 521f–522f nonalcoholic fatty liver disease, 371–372, 372f
LCH. See Langerhans-cell histiocytosis Doppler ultrasound, 357, 357f–358f
Lead-zirconate-titanate (PZT), 4f, 12 elastography, 359, 359f, 359t, 360f
Leiomyosarcoma fluid collections
bladder, 823 extrahepatic, 423–424, 423f–424f
gastric, 303 intrahepatic, 425, 425f
paratesticular, 674 fungal infection, 378, 378f
retroperitoneal, 621 grayscale imaging, 356–357, 356f, 356t
Lemierre syndrome, 908 imaging approaches, 356
Lesser peritoneal sac, 525, 526f infection
Levene index, for ventricular size measurement, 90 viral hepatitis, 375, 375f
Leydig cell hyperplasia, 661 bacterial infection, 375–376, 376f–377f
Leydig cell tumor, 661, 665, 665f fungal infection, 378, 378f
Li-Fraumeni syndrome parasitic infection, 378–380, 379f
adrenocortical tumors and, 597 malignant tumors
rhabdomyosarcoma and, 551, 720, 822 embryonal rhabdomyosarcoma, 403
Lipoblastoma epithelioid hemangioendothelioma, 402–403
neck, 147–149, 148f fibrolamellar variant of HCC, 388t, 401, 401f
peritoneal cavity, 546t, 547 hepatoblastoma, 388t, 397–400, 398f–399f
retroperitoneum, 617 hepatocellular carcinoma, 388t, 400, 400f
soft tissue, 846–847, 847f leukemia, 407, 407f
Lipoma(s) lymphoma, 404–405, 405f
brain, 58t, 67, 67f metastases, 403–404
chest wall, 240t, 257, 257f–258f posttransplant lymphoproliferative disorder, 405–407, 406f
diaphragm, 279–280, 279t PRETEXT staging system, 398
filum terminale, 112t, 113–114, 114f rare primary tumors, 402–403, 404f
gastric, 300 undifferentiated embryonal sarcoma, 388t, 402
intraspinal, 319f, 116, 119, 120f normal anatomy, 363–369, 363f–368f
neck, 147–149, 148f circulation, 366–367, 366f
pancreas, 580 Couinaud classification system, 364, 364f, 366f, 367
paratesticular, 671, 672f ligaments, 364–365, 365f–366f
peritoneal cavity, 546, 546f, 546t parenchyma, 367, 367f
retroperitoneum, 617 normal development, 360–362, 361f–362f
soft tissue, 846, 846f passive venous congestion, 386, 386f
Lipomatosis peliosis hepatis, 385, 385f
chest wall, 257 portal hypertension, 382–383, 382f, 382t
986 Index

Liver (cont.) consolidation, 177f, 183

Budd-Chiari syndrome, 383–384, 383f hepatization, 176, 176f, 177t
portal venous gas, 386–387, 387f interstitial lung disease, 185–186, 186t, 187f
sinusoidal obstruction syndrome(SOS), 384–385, 384f M-mode appearance, 175f, 176
technique normal anatomy, 175
imaging approaches normal development, 174–175, 174f
contrast-enhanced ultrasound, 358, 358f necrosis, 183
Doppler ultrasound, 357, 357f pulmonary lymphangiectasia, 188, 188f
elastography, 359–360, 359f–360f, 359t pulmonary masses, 188–191
grayscale imaging, 356–357, 356f, 356t benign pulmonary masses
patient positioning, 356 hamartoma, 189
ultrasound transducer selection, 356 malignant pulmonary neoplasms
trauma metastases, 190, 191f
blunt abdominal, 380, 380f–381f primary malignant pulmonary neoplasms
umbilical vein catheterization, 380–382, 381f pleuropulmonary blastoma, 189, 190f
Liver transplantation, 408–425, 408t rhabdomyosarcoma, 189–190
biliary complications, 408t technique
bile leak, 413, 414f contrast-enhanced ultrasound, 174
biliary stricture, 414, 415f imaging approaches, 174
fluid collections, 408t, 423–425 patient positioning, 173
extrahepatic collections ultrasound transducer selection, 173
abscess, 423–424, 424f ultrasound signs
biloma, 423–424, 424f A-lines, 175f, 176, 177t
hematoma, 423, 423f B-lines, 176, 176f, 177t
intrahepatic collections lung sliding, 175, 175f, 177t
abscess, 425, 425f pleural line, 175, 175f
biloma, 425, 425f “Lying down” adrenal sign, in renal agenesis, 742, 742f
hematoma, 425 Lymph node(s)
non-vascular postoperative complications, 408t Castleman disease and, 549–550, 549f
normal liver transplant ultrasound, 412 differentiation from parathyroid adenoma, 162–163
preoperative and postoperative imaging considerations, 408t, 411 Epstein-Barr viral infection and, 498
rejection, 412, 412f–413f infectious, 144–146, 144f–146f, 316, 494, 615
surgical technique intramammary, 954, 954f
living-related donor and split liver grafts, 409–410, 409f, 411f intussusception and, 314
whole liver transplantation, 409–410, 409f–410f mediastinal, 198, 198f, 221, 225, 231–233
vascular complications, 408t metastatic, 150, 167, 213, 236f, 259f, 397, 400, 473, 554, 616, 616f,
hepatic artery pseudoaneurysm, 418, 418f 620, 663, 664f, 673, 707, 720, 810–813, 822, 961, 962f
hepatic artery stenosis, 415, 417, 417f neck, 128–129, 129f
hepatic artery thrombosis, 415, 416f neuroblastoma staging and, 601t
hepatic vein outflow obstruction, 420, 421f parotid gland, 131, 132f
inferior vena caval stenosis and thrombosis, 422, 422f pericardial, 235
portal vein stenosis, 419–420, 419f–420f peritoneal, 525
portal vein thrombosis, 418–419, 419f reactive, 408t, 412, 646, 648
Lobar holoprosencephaly, 64 retroperitoneal, 611
Longitudinal sound wave, 2f, 3 sarcoidosis and, 502
Lower extremities scalp, 93, 94
arteries Lymphadenopathy
anatomy, 913–914 bacterial parotitis and, 164–165
development, 913 cervical, 146, 146f, 149, 149f, 150
veins intramammary, 954
anatomy, 922–923, 923f mediastinal, 231–233, 232f
deep and superficial systems, 922, 922f mesenteric, 541–542, 541f, 648f
development, 922 pericardial, 235, 236f
Lung peritoneal, 536, 615
abscess, 184–185, 184f–185f retroperitoneal, 615–616, 664f
air bronchograms, 176, 177f, 177t sialadenitis, chronic, and, 166
atelectasis, 183 thyroid cancer and, 156, 156f, 156t
bronchopulmonary sequestration, 180–183, 182f viral infection of salivary glands and, 164
extralobar, 182–183 Lymphangiectasia, 188, 188f, 321, 321f
hybrid congenital lung anomalies, 183 Lymphatic malformation(s)
intralobar, 181–182 bladder, 816, 817f
congenital lung anomalies, 177–183 breast, 955
congenital lobar hyperinflation, 178–179, 178f diaphragm, 279–280, 279t
congenital pulmonary airway malformation, 180, 180t, 181f mediastinum, 225, 225t, 227–229, 228f
foregut duplication cyst, 179–180, 179f–180f neck, 140, 144, 144f
hybrid congenital lung anomalies, 183 pancreas, 579
Index 987

peritoneal, 542, 543f–544f, 594f neuroblastoma, 673

retroperitoneal, 614, 615f Wilms’ tumor, 673
salivary gland, 166 non-neoplastic lesions
scrotum, 670–671, 671f cystic dysplasia of epididymis, 670
soft tissue, 892, 893f ectopic adrenal rest, 670
spleen, 511–513, 514f epididymal cyst, 669–670, 670f
thoracic, 206, 206f, 248t, 251, 251f–252f fibrous hamartoma of infancy, 670
Lymphocele fibrous pseudotumor, 670
after renal transplantation, 794, 796, 796f juvenile xanthogranuloma, 670
scrotal, 652 spermatic granuloma, 670
Lymphoma. See also Burkitt lymphoma; Hodgkin lymphoma; spermatocele, 669, 669f
Non-Hodgkin lymphoma; specific organs vascular anomalies
adrenal, 609 arteriovenous malformation, 671
biliary tree, 475 lymphatic malformation, 670–671, 671f
breast, 960, 964f venous malformation, 671
Castleman disease and risk of, 550 prostate and seminal vesicles
chest wall, 261–262, 261f, 963 congenital anomalies
chylous effusion and, 206–207 enlarged prostatic utricle, 675
Epstein-Barr viral infection and, 498 Müllerian duct cyst, 675
gallbladder, 453 prostatic utricle cyst, 675, 676f
GI tract, 301–302, 303f, 315, 329–330, 329f, 333, 333f, 342, 345, seminal vesical agenesis/hypoplasia, 677–678
346f seminal vesicle cyst, 675, 677f
kidney, 813–814, 814f inflammatory disorders
liver, 356t, 382t, 404–405, 405f prostatic abscess, 678
mediastinum, 226–227, 227f prostatitis, 678
neck, 149–150, 149f normal anatomy
ovary, 711 of prostate, 674, 674f
pancreas, 585, 586f, 587 of seminal vesicles, 675, 675f
paratesticular, 673–674 normal development, 674
peritoneal cavity, 546t, 551, 552f technique
retroperitoneum, 615–616, 616f imaging approaches, 674
retroperitoneal fibrosis and, 613 patient positioning, 674
secondary breast carcinoma and history of, 961–962 ultrasound transducer selection, 674
spleen, 487t, 518–519, 518f–520f tumors of prostate and seminal vesicle, 678
testicle, 668 leukemia, 678
thyroid, 157, 157f rhabdomyosarcoma, 678
uterus, 719 scrotum
Lymphoproliferative disorder. See Posttransplant lymphoproliferative acute scrotal pain, 639t
disorder anatomical variants
Lysosomal storage diseases, 506–507, 506f–507f testicular appendages, 635, 635f
vessels, 635
calcification
M loose bodies, 653, 653f
Macrophage activation syndrome, 503–504 meconium peritonitis, 653, 654f
Male genital tract testicular microlithiasis, 652–653, 653f
inguinal hernia testicular tumors and, 653
direct, 658–659, 658f congenital anomalies
indirect, 657–658, 657f–658f anorchidism, 636
Valsalva maneuver and, 657 bell clapper deformity, 639, 639f
paratesticular masses cryptorchidism, 636, 636f
benign tumors cystic dysplasia of rete testis, 638
adenomatoid tumor, 672 polyorchidism, 637, 637f
dermoid, 672 splenogonadal fusion, 638, 638f
leiomyoma, 672 testicular agenesis, 636
lipoma, 671, 672f testicular ectopia, 637–638
neurofibroma, 672 testicular hypoplasia, 637, 637f
papillary cystadenoma, 672–673 testicular regression syndrome, 636–637
malignant tumors, primary fluid collections
inflammatory myofibroblastic tumor, 673 abdominoscrotal hydrocele, 651
liposarcoma, 673 hematocele, 651–652, 652f, 656
rhabdomyosarcoma, 673, 673f hydrocele, 649–650, 651f
malignant tumors, secondary of spermatic cord, 650–651, 652f
fibrosarcoma, 674 lymphocele, 652
leiomyosarcoma, 674 inflammatory disorders
leukemia, 673 acute epididymitis and epididymo-orchitis, 639t, 644, 645f
lymphoma, 673–674 chronic epididymitis, 649
988 Index

Male genital tract (cont.) Wilms’ tumor, 669

dancing megasperm, 649 trauma
epididymal abscess, 645 blunt scrotal trauma, 653–654, 654t
Fournier gangrene, 649, 650f foreign body, 656, 656f
Henoch-Schönlein purpura, 646, 647f–648f penetrating scrotal trauma, 656
idiopathic scrotal edema, 646, 649f repetitive scrotal microtrauma, 657
orchitis, isolated, 644–645 scrotal urinoma, 657
scrotal abscess, 645–646, 646f testicular fracture, 654t, 655, 655f
testicular abscess, 645 testicular hematoma, 654–655, 654f, 654t
normal anatomy testicular rupture, 654t, 655–656, 655f
blood supply, 633–643, 633f–635f varicocele, 659, 659f
epididymis, 632 intratesticular varicocele, 660, 660f
spermatic cord, 632, 633f Malignant gastric tumor(s)
testes, 631–632, 632f, 632t gastrointestinal stromal tumor, 303, 304f
testicular appendages, 633, 633f leiomyosarcoma, 303
normal development, 630–631, 630f lymphoma, 301–302, 303f
segmental testicular infarction, 639t, 641–642, 662 primary gastric adenocarcinoma, 303
arterial segmental testicular infarction, 642 Malignant hemangioendothelioma of breast, 962. See also
venous testicular infarction, 642–643 Angiosarcoma
technique Malignant peripheral nerve sheath tumor (MPNST), 147
imaging approaches, 630 MALToma, 167
patient positioning, 629 Mammary gigantism, 948
ultrasound transducer selection, 629–630 Mature cystic teratoma, of ovary, 706, 706f
testicular masses Mayer-Rokitansky-Küster-Hauser Syndrome (MRKH), 693, 694f
ultrasound appearance and clinical features, 662t May-Thurner syndrome
testicular masses, non-neoplastic, 660–662 iliofemoral thrombosis and, 936
adrenal rests, 660–661, 661f transplant renal vein thrombosis and, 789
hamartoma, 661 MCDK. See Multicystic dysplastic kidney
Leydig cell hyperplasia, 661 Mechanical index (MI), 34
simple cyst, 661–662, 661f Mechanical transducer, 14–19, 15f–19f
sinus histiocytosis (Rosai-Dorfman-Destombes disease), 662 Meckel diverticulum, 323–324, 324f
testicular torsion, 639–641, 639t, 640f–642f Meconium ileus, 311–312, 312f
spermatic cord and, 639t, 640 Meconium peritonitis, 312–313, 537, 538f, 653, 654f
torsion of testicular appendages, 643, 644f Meconium pseudocyst, 313, 313f
testicular tumors, primary, 662t Mediastinal neurenteric cysts, 229
adenomatoid, 668 Mediastinum
“burned out” (Azzopardi), 662 cardiophrenic angle masses
choriocarcinoma, 664 lymphadenopathy, 235, 236f
dermoid cyst, 666 pericardial cyst, 235, 235f
elastography, 663 normal anatomy, 221–225
embryonal carcinoma, 663–664 compartment approach, 221, 222f, 223
epidermoid cyst, 666, 667f esophagus, 224–225, 225f
fibroma, 666 thymus, 220, 223, 223f
follicular lymphoma, 668 trachea, 224, 224f
germ cell, 662, 663f normal development, 220–221, 221f
gonadoblastoma, 663 paravertebral (posterior) mediastinal masses
hemangioma, 667, 667f ganglioneuroblastoma, 233–234
leiomyoma, 668 ganglioneuroma, 234–235
lipoma, 667 neuroblastoma, 233, 234f
neurofibroma, 666 prevascular (anterior) mediastinal masses
seminoma, 663 lymphatic malformation, 227–229
stromal tumors, 664–666 lymphoma, 226–227, 227f
granulosa cell, 665–666, 666f teratoma, 225–226, 226f
Leydig cell, 665, 665f technique
Sertoli cell, 665, 665f imaging approaches, 220
teratocarcinoma, 664 patient positioning, 219–220
teratoma, 663, 664f ultrasound transducer selection, 220
other testicular tumors visceral (middle) mediastinal masses
yolk sac, 662–663, 664f foregut duplication cysts, 229–231, 229f–230f
testicular tumors, secondary lymphadenopathy, 231–233, 232f
carcinoid, 669 Medullary cystic disease, of kidney, 777
Langerhans cell histiocytosis, 669 Medullary nephrocalcinosis, 798, 798f
leukemia, 668, 668f Megacystitis-microcolon-intestinal hypoperistalsis syndrome
neuroblastoma, 668–669 (MMIHS), 763
retinoblastoma, 669 Meigs syndrome, 705t, 710
rhabdomyosarcoma, 669 Ménétrier disease, 297
Index 989

Meningitis, neonatal/infant, 86–88, 88f Near-field, of ultrasound beam, 15–17, 15f–17f, 19. See also
Meningocele, 94, 111t, 112, 112t, 116, 118f–119f, Fresnel zone
762, 767 NEC. See Necrotizing enterocolitis
Mesenchymal hamartoma Neck
of chest wall, 257–258 benign neoplasms
of liver, 388t, 392–393, 392f lipoblastoma, 147, 148f, 149
Mesenteric cyst, 326, 326f lipoma, 147, 148f, 149
Methicillin-resistant Staphylococcus aureus (MRSA), myofibromatosis, 146, 147f
184f, 839, 851, 854 neurofibroma, 147, 147f
MI. See Mechanical index differentiation from malignant peripheral nerve sheath
Microbubbles, 35–37, 36f tumor, 147
Microgastria, 290, 292 congenital neck anomalies, 133–144
Mid-aortic syndrome, abdominal aortic stenosis and, congenital non-vascular neck masses, 133–138, 134f–139f
44f, 928–929, 930f congenital vascular neck masses
Midgut malrotation, 309–311, 309f–311f congenital hemangioma, 138
Mirizzi syndrome, 471–472 vascular malformations, 140, 144, 141f–144f
Mirror image artifact, 37, 37f imaging approaches, 128
Mitrofanoff appendicovesicostomy, 824, 825f infantile hemangioma, 138, 139f, 140
MMIHS. See Megacystitis-microcolon-intestinal hypoperistalsis PHACES syndrome, 138
syndrome infectious and inflammatory disorders
M-mode imaging, 14, 14f, 104–105, 175, 175f, 196, 199–200, cat-scratch disease, 146, 146f
200f–201f, 202, 209, 209f, 278, 278f histiocytosis, 146
Monosomy X. See Turner syndrome human immunodeficiency virus infection, 146
Morgagni hernia, 274, 274f, 274t, 275, 276f Kawasaki disease, 146
MPNST. See Malignant peripheral nerve sheath tumor lymphadenitis and abscess, 144–145, 144f
MRKH. See Mayer-Rokitansky-Küster-Hauser Syndrome mycobacterial infection, 145–146, 146f
Mucoepidermoid carcinoma, of salivary glands, 166, 166f sarcoidosis, 146
Müllerian duct anomalies. See also Female genital tract, congenital malignant neck neoplasms
anomalies lymphoma, 149, 149f
müllerian duct cyst, 675 neuroblastoma, 149–150, 150f
Multicystic dysplastic kidney (MCDK), 736, 736f, 745f, 776–777, rhabdomyosarcoma, 150, 151f
776f–777f metastatic cervical nodal disease, 149f, 150–151
Multifocal lymphangioendotheliomatosis with neoplastic disorders, 146–151
thrombocytopenia, 328 normal development and anatomy, 128–133
Multinodular goiter (nodular hyperplasia), 157–158, 158f anterior triangle, 128
Multipath reflection artifact. See Mirror image artifact lymph nodes, 129, 129f
Multiple hereditary exostoses (MHE), 252, 849f, 853 parathyroid glands, 130f, 131, 131f
Musculoskeletal system. See also Ankle and hind foot; Bones; parotid gland, 131, 132f
Elbow; Hip; Joints; Knee; Shoulder; Soft tissues; posterior triangle, 128
Wrist and hand salivary glands, 131–133
normal development, 837 sternocleidomastoid muscle, 128, 128f
technique sublingual glands, 132f, 133
imaging approaches, 836–837, 836f submandibular glands, 132, 133f
patient positioning, 836 Wharton duct, 132, 167
ultrasound transducer selection, 836 thyroid gland, 130–131, 130f
trauma, tendon tears, 844 technique
vascular anomalies, 890t imaging approaches, 128
arteriovenous fistula, 893–894 patient positioning, 127
arteriovenous malformation, 893–894, 894f ultrasound transducer selection, 127–128
congenital hemangioma, 890–891, 891f Necrotizing enterocolitis (NEC), 337, 338f
infantile hemangioma, 889–890, 890f patient positioning and, 305
kaposiform hemangioendothelioma, 891, 891f portal venous gas and, 386, 387f
and Kasabach-Merritt phenomenon, 891 Neonatal acute ischemic stroke, 78
lymphatic malformation, 892, 893f Neonatal hepatitis syndrome, 443t, 463, 465, 467f
venous malformation, 892, 892f–893f Neonatal meningitis, 86
Mycobacterial infection, 145–146, 772 Nephroblastomatosis, 810, 810f
Mycobacterium tuberculosis lymphadenopathy, 231–232 Beckwith-Wiedemann syndrome and, 571
Myelocele, 112–113 Nephrogenic rests, 810, 801f
Myelomeningocele, 62, 62f, 112–113, 113f, 114, 117f, 767 Nephronophthisis, 776–777, 777f
Myofibromatosis, 146, 147f Neurenteric cyst(s)
Myositis ossificans, 263, 843, 843f of mediastinum, 229–230, 230f
of spine, 114
Neurenteric fistula, 114
N Neuroblastoma
Nabothian cyst, 719, 719f adrenal, 598, 601–602, 601t, 602f–605f, 606
NAFLD. See Nonalcoholic fatty liver disease Beckwith-Wiedemann syndrome and, 571
990 Index

Neuroblastoma (cont.) chylous effusion, 206–207

biliary tree, 474–475 GI tract, 301, 329
breast, 963 kidney, 813
chest wall, 210, 262 liver, 404
differentiation from adrenal hemorrhage, 596–597 mediastinum, 226–227
differentiation from Wilms’ tumor of kidney, 807–808 neck, 149f, 150
kidney, 814, 815f ovary, 711
liver, 403 pancreas, 587
lung, 190 peritoneal cavity, 551
mediastinum, 225t, 233, 234f retroperitoneum, 615
neck, 149–150, 150f spleen, 487t, 518, 518f
ovary, 711 uterus, 719
pancreas Non-involuting congenital hemangioma (NICH)
primary, 585–586 of liver, 388t, 389
metastatic, 587 of skin/subcutaneous tissues, 248–249, 248t, 890, 890t
peritoneal cavity, 546t, 555 Nonsteroidal anti-inflammatory drugs (NSAIDs), 504, 549, 844, 855,
pleura, 214 877, 885
retroperitoneum, 617–618, 620f Nonthermal bioeffects, 48
scrotum, 668–669, 673 NSAIDs. See Nonsteroidal anti-inflammatory drugs
spleen, 521 Nursemaid elbow. See Pulled elbow
Neuroendocrine tumor Nyquist limit(s), 30, 31, 43
bile ducts, 474–475, 475f
carcinoid of appendix, 333
metastases to liver, 403 O
pancreas, 571–572, 582–584, 584f Obstructed glands of Montgomery, 950. See also Retroareolar cysts
Neurofibroma Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA)
bladder, 819, 820f syndrome, 700, 700f
neck, 147, 147f Obstructive hydrocephalus, 72, 87–88, 90, 92
pancreas, 580 OEIS complex. See Cloacal exstrophy
paratesticular, 672 OHVIRA syndrome. See Obstructed hemivagina and ipsilateral renal
peritoneal, 546t, 547–548, 548f anomaly
retroperitoneum, 617, 618f Omental cyst, 540
testicle, 666 Orchitis, isolated, 644–645. See also Epididymo-orchitis
Neurofibromatosis Ortolani maneuver, for hip stability, 872
adrenal pheochromocytoma and, 606 Oscillatory motion, 2
GIST and, 303 Osgood-Schlatter disease, 844f, 886
lateral meningocele and, 116 Ossification centers
neurofibroma of bladder and, 819, 820f, 822 ankle, 887–888, 887f–888f
neurofibroma of neck and, 147 calcaneal apophysis, 889
neurofibroma of paratesticular tissues and, 672 elbow, 860f
neurofibroma of retroperitoneum and, 617, 618f hindfoot, 887, 887f
neurofibroma of testicle and, 666 humeral head, 856, 856f
optic pathway astrocytoma and, 88 knee, 878, 878f
plexiform neurofibroma of mesentery and, 547, 548f long bones, 847
renal artery stenosis and, 779 patella, 884, 885f
schwannoma of neck and, 147 pelvic bones, 870
Neurogenic bladder, 749f, 754, 767, 767f, 800, 824 ribs, 242, 242f
bladder augmentation for, 767 spine, 104
Neuronal migration disorders sternum, 242, 243f, 247, 247f
gray matter heterotopia, 68, 69f Ossifying renal tumor of infancy, 806
lissencephaly, 68 Osteochondroma/exostosis, 251–252, 252f, 253, 849f, 853–854
Neuronal proliferation disorders, 58f, 68 Osteomyelitis, 255, 850–851, 850f–851f
Neutropenic colitis, 342–343, 342f Osteosarcoma, 190, 233, 260–262, 588f, 814, 815f
NICH. See Non-involuting congenital hemangioma Ovary(ies). See also Female genital tract
Niemann-Pick disease, 507 anatomy, 688–689
Non-accidental trauma. See also Child abuse; Trauma development, 686–688
distal humeral epiphyseal separation and, 864, 865f masses, 703–712
duodenal hematoma and, 319 menstrual cycle and, 690–691
gallbladder and, 454f torsion, 713
scrotum, 653–654
subdural fluid collection and, 74f
Non-alcoholic fatty liver disease (NAFLD), 371–372, 372f P
Non-communicating hydrocephalus, 90 Pancreas
Nonfunctioning parathyroid cyst, 161 acute pancreatitis
Non-Hodgkin lymphoma (NHL). See also Burkitt lymphoma; acute peripancreatic fluid collections, 573, 573f
Hodgkin lymphoma; Lymphoma Atlanta classification, revised, 572
chest wall, 261 definition, 572
Index 991

diagnosis, 572 fibrothorax, 204, 205f

etiologies, 572 Parasitic infection. See also Echinococcal disease; Hydatid disease;
necrotizing pancreatitis, 575, 575f Schistosomiasis
pancreaticopleural fistula, 575 of liver, 378–380, 379f
pseudocysts, 573–574, 573f–574f of spleen, 498–501, 500f
ultrasound findings, 572, 572f of small bowel, 316
vascular complications, 576, 576f of urinary tract, 772, 772f
acute recurrent pancreatitis, 576–577 Parathyroid adenoma, 162–163, 162f
anatomic variants Parathyroid cyst, 161–162, 161f
lobulated parenchymal contour, 566 Parathyroid glands, 128, 130f, 131, 131f. See also
benign neoplasms Hyperparathyroidism
cystic teratoma, 579–580, 589t Parathyroid hyperplasia, 162
infantile hemangioma, 579 Paraurethral duct cyst, 721–722, 722f
inflammatory myofibroblastic tumor, 580 Paravertebral (posterior) compartment, of mediastinum, 221, 228f, 230
leiomyoma, 580 Paravertebral (posterior) mediastinal mass(es)
lipoma, 580 ganglioneuroblastoma, 233–234
mucinous cystadenoma, 579 ganglioneuroma, 234–235
neurofibroma, 580 neuroblastoma, 233, 234f
schwannoma, 580 Parenchymal hemorrhage, of brain, 72, 75, 90
serous cystadenoma, 579 Parotid gland, 131–132, 132f, 164, 165f, 166–167, 167f
chronic pancreatitis, 576–577, 576f–577f Partial volume artifact, 40–41, 41f
congenital abnormalities Partially involuting congenital hemangioma (PICH)
accessory pancreatic lobe, 567, 569 of skin/subcutaneous tissues 248–249, 248t
annular pancreas, 566–567, 566f–567f of liver, 388t, 389, 390f
common pancreaticobiliary channel, 567, 568f PASH. See Pseudoangiomatous stromal hyperplasia
congenital hyperinsulinism, 569 Passive venous congestion, of liver, 386, 386f
partial pancreatic agenesis, 567, 568f PCOS. See Polycystic ovary syndrome
ectopic pancreas, 569 Peliosis hepatis, 385, 385f
pancreas divisum, 566, 566f Peliosis, splenic, 515–516
congenital pancreatic cyst, 569–570 Pelvic inflammatory disease (PID), 716, 716f–717f
cystic neoplasms of Pericardial cyst, 235, 235f
mucinous cystadenoma, 579 Peritoneal cavity. See also Peritonitis
serous cystadenoma, 579 ascites
teratoma, 579–580, 580f chylous, 531–532, 531f
genetic disorders exudate, 528
autosomal dominant polycystic kidney disease, 571, 571f hemoperitoneum, 530, 531f
Beckwith-Wiedemann syndrome, 571 serum-ascites-albumin gradient, 529, 529t
cystic fibrosis, 570, 570f transudate, 528
Shwachman-Diamond syndrome, 570–571, 571f treatment, 530
Von Hippel-Lindau disease, 571–572 ultrasound evaluation, 529, 529f–530f
malignant tumors urine, 532, 532f
acinar cell carcinoma, 584–585 benign masses, 545–551, 546t
ductal adenocarcinoma, 585, 585f Castleman disease, 549–550, 549f
fibrosarcoma, 587 desmoid tumor, 548–549, 548f
islet cell, 582–584 inflammatory myofibroblastic tumor, 550–551, 550f
kaposiform hemangioendothelioma, 586–587 infantile hemangioma, 545–546
lymphoma, 585, 586f lipoblastoma, 547
metastatic disease, 587, 587f–588f lipoblastomatosis, 547
neuroblastoma, 585–586 lipoma, 546, 546f
pancreatoblastoma, 580–582, 581f lipomatosis, 546, 547f
primitive neuroectodermal, 586 neurofibroma, 547–548
rhabdomyosarcoma, 587 plexiform neurofibroma, 547–548, 548f
solid pseudopapillary, 582, 582f inflammation
normal anatomy, 564, 565f abscess, 539–540, 539f
normal development, 564, 565f peritonitis, 535–539
pancreatic transplantation, 588, 588f localized fluid collections
technique biloma, 533, 534f
imaging approaches, 564 cerebrospinal fluid pseudocyst, 532–533, 533f
patient positioning, 564 diaphragmatic mesothelial cyst, 535, 536f
ultrasound transducer selection, 564 pancreatic pseudocyst, 534
trauma, 577–579, 577f–578f peritoneal inclusion cyst, 535, 535f
venous and lymphatic malformations, 579 malignant tumors, 546t, 551–555
Paraovarian cyst, 711–712, 712f metastatic disease
Papillary urothelial neoplasm of low malignant potential (PUNLMP) adenocarcinoma, 555
of bladder, 818 germ cell tumor, 555
Parapneumonic effusion, 203–204, 203f intracranial neoplasms, 555
empyema, 204, 204f neuroblastoma, 555
992 Index

Peritoneal cavity (cont.) T-lines (“lung pulse” sign), 200, 201f

Wilms’ tumor, 555 ultrasound transducer selection, 196
primary Z-lines, 201, 201f
desmoplastic small round cell tumor, 553–554, 554f Pleural effusion
lymphoma, 551, 552f causes of, 201
Burkitt lymphoma, 551, 552f chylous effusion (chylothorax), 206–207, 206f–207f
non-Hodgkin lymphoma, 551 clinical presentation, 201
malignant mesothelioma, 554–555 complex pleural effusion, 202–203, 202f–203f
rhabdomyosarcoma, 551–553, 553f parapneumonic effusion, 203–204, 203f
mesenteric lymphadenitis, 541–542, 541f quantification of fluid volume, 201
normal anatomy, 524–528, 526f simple pleural effusion, 202, 202f
mesentery, 526, 527f–528f traumatic effusion
omentum, 526, 527f extrapleural hematoma, 206
peritoneal fluid, 528, 529f hemorrhagic effusion, 205–206, 205f
peritoneum, 524–525 hemothorax, 205–206, 205f
normal development, 524, 525f Pleuritic pain, 201
omental cyst, 540 Pleuropulmonary blastoma (PPB), 189, 190f, 211, 212f
pneumoperitoneum, 540, 540f and DICER1 mutation/syndrome, 189, 190f, 211, 212f
segmental omental infarction, 540–541, 541f Plexiform neurofibroma, 147, 547–548, 548f, 820f
technique Plunging ranula, 163, 163f
imaging approaches, 524 PNET. See Primitive neuroectodermal tumor
patient positioning, 524 Pneumoperitoneum, 540, 540f
ultrasound transducer selection, 524 necrotizing enterocolitis and, 337, 338f
vascular anomalies Pneumothorax, 207–210, 208f, 208t
lymphatic malformation, 542, 543f–544f Poland syndrome, 947
venous malformation, 542–543, 545f Polycystic kidney and hepatic disease 1 gene (PKHD1), 775
Peritoneal inclusion cyst, 712, 712f Polycystic liver disease, 369–370, 370f
Peritonitis Polycystic ovary syndrome (PCOS), 724, 724f
chemical, 537, 538f Polycystin 1 gene (PKD1), 775
meconium, 312–313, 313f, 537, 538f, 653, 654f Polymicrogyria, 67–68, 85. See also Post-migration disorders, of brain
definition of, 535 Polyorchidism, 637, 637f
granulomatous, 537 Popliteal cyst, 881, 881f
infective, 535–536, 537f Porcelain gallbladder, 449, 450f
tuberculous, 536 Portal hypertension, 382–383, 382t, 382f. See also Budd-Chiari
sclerosing encapsulating, 537–539, 539f syndrome; Portal vein stenosis; Portal vein thrombosis
Periventricular hemorrhagic infarction, 72, 73f Alagille syndrome and, 469
Periventricular leukomalacia (PVL), 76–78, 77f–78f ARPKD and, 775
PFFD. See Proximal focal femoral deficiency Caroli disease and, 459
PHACE(S) syndrome, 95–96, 138, 248, 388t flow velocity and, 366
PICH. See Partially involuting congenital hemangioma gallbladder varices and, 453, 453f
PID. See Pelvic inflammatory disease Gamna-Gandy bodies and, 523, 524f
Pilomatricoma, 845–846, 846f hepatic fibrosis and, 372
Pilonidal cyst, 120–121, 121f peliosis hepatis and, 385
Pilonidal sinus, 120–121 portal vein stenosis and, 419
PKD1. See Polycystin 1 gene schistosomiasis and, 500
Plagiocephaly, positional, 97, 99 sclerosing cholangitis and, 471
and SIDS, 97 splenic vein thrombosis and, 576
Pleura splenomegaly and, 487t, 508, 509f
anatomic variants, 199, 199f ultrasound features, 375
normal anatomy, 196–199, 197f–199f Portal vein
normal development, 197, 197f normal, 357f, 366
pleural masses, 210–214, 210f stenosis, 368, 419f, 419–420, 420f
malignant pleural masses thrombosis
metastases, 214, 214f liver cirrhosis and, 374
pleuropulmonary blastoma, 211–212, 212f liver transplantation, complication of, 408t, 419, 419f
rhabdomyosarcoma, 212–213, 213f pancreatitis, complication of, 529t
pneumothorax, 207–210, 208f, 208t portal hypertension, cause of, 382–383, 382t
absent B-lines, 209 and splenomegaly, cause of, 487
absent lung pulse, 209 umbilical vein catheterization and, 382
absent lung sliding, 208–209 and varices of gallbladder, 453
lung point sign, 209, 209f Portal venous gas, 337, 386–387, 387f
stratosphere/barcode signs, 209, 209f Posterior urethral valves (PUV), 532, 532f, 576, 675, 764–765,
technique 764f–765f, 785, 794, 824
A-lines, 200, 200f Post-migration disorders, of brain
B-lines, 200, 200f polymicrogyria, 68
imaging approaches, 196, 196f schizencephaly, 58t, 64, 67–69, 70f
patient positioning, 196 Post-transplant lymphoproliferative disorder (PTLD), 405–407
Index 993

after liver transplantation, 406f, 408t, 475f amenorrhea, 724

after renal transplantation, 797, 797f polycystic ovary syndrome, 724, 724f
Power Doppler, 31–32, 32f precocious puberty, 723
in chronic pyelonephritis, for increased sensitivity of scar Puberty, definition, 723
detection, 772–773 Pulled elbow, 866, 866f
for low-flow detection, 910 Pulmonary abscess, 184–185, 184f
in suspected carotid artery thrombosis, 904, 905f Pulmonary lymphangiectasia, 188, 188f
in suspected hepatic and portal vein thrombosis, 357 Pulmonary masses, 188–191
and motion sensitivity, 44 benign
Premature thelarche, 723, 948, 948f hamartoma, 189
PRETEXT staging system, for primary hepatic malignancies of malignant
childhood, 398 metastases, 190, 191f
Prevascular (anterior) compartment, of mediastinum, 221 primary malignant pulmonary neoplasms
PRF. See Pulse repetition frequency pleuropulmonary blastoma, 189, 190f
Primary diaphragmatic neoplasms rhabdomyosarcoma, 189–190
benign, 279–280, 279f Pulsed wave (PW) Doppler, 27, 29–30, 29f, 43f
malignant, 280, 280f, 280t Pulse-echo technique, 2, 8
Primitive neuroectodermal tumor (PNET) Pulse inversion imaging, 36–37, 36f
of brain, associated with rhabdoid tumor of kidney, 811 Pulse repetition frequency (PRF), 9, 10f, 43f, 334, 900
chest wall, 259 Pulse repetition period (PRP), 9, 10f
kidney, 812–813 Pulser, 11
pancreas, 586 PUNLMP. See Papillary urothelial neoplasm of low malignant potential
Processus vaginalis PUV See Posterior urethral valves
canal of Nuck disorders and, 725–726 PVL. See Periventricular leukomalacia
congenital hydrocele and, 650, 651f PW Doppler. See Pulsed wave Doppler
indirect inguinal hernia and, 658, 658f Pyelonephritis. See also Urinary tract infection
scrotal abscess from peritonitis and, 646 acute, 768–769, 769f
scrotal development and, 630, 630f chronic, 772–773, 773f
scrotal extension of meconium peritonitis and, 653 Pyloric stenosis. See Hypertrophic pyloric stenosis
scrotal hematocele from intraperitoneal hemorrhage and, 652 Pylorospasm, 293–294, 294f
scrotal swelling from adrenal hemorrhage and, 596 Pylorus, 288, 290, 293–294, 300, 305
scrotal urinoma from bladder rupture and, 657 Pyomyositis, 255, 839, 840f
spermatic cord hydrocele and, 650–651, 652f Pyonephrosis, 770, 770f
splenogonadal fusion and, 638 PZT. See Lead-zirconate-titanate
Prostaglandin-induced foveolar hyperplasia, 294, 295f
Prostate gland. See Male genital tract, prostate and seminal vesicles
Prostatitis, 678 Q
Proximal focal femoral deficiency (PFFD), 876, 876f Quadrigeminal cistern, 53, 55f, 67
PRP. See Pulse repetition period Quasi-static strain elastography methods, 32
Pseudoaneurysm(s)
femoral, 916
hepatic artery, after liver transplantation, 418, 418f R
renal artery, 781, 783f Ranula, 163, 163f
after renal transplantation, 789, 791f Rapidly involuting congenital hemangioma (RICH)
splenic, 522 of liver, 388t, 389, 389f
Pseudoangiomatous stromal hyperplasia (PASH), 957–958, 958f of skin/subcutaneous tissues, 248–249, 248t, 890, 891f
Pseudocyst(s) Rarefaction, 3–4, 3f, 48
adrenal, 594 Rarefaction peak pressure, 48
CSF, complication of ventriculoperitoneal shunt, 532–533, 533f RCC. See Renal cell carcinoma
gastric duplication cyst, differentiated from, 567 RDS. See Respiratory distress syndrome
meconium, 312–313, 313f, 537 Receiver, 12–14, 13f
mesenteric cyst, differentiated from, 326 Reflection
necrotizing pancreatitis, complication of, 575, 575f–576f in acoustics, 4–7, 5f–7f, 9, 24
pancreatic, complication of trauma, 578 multipath reflection artifact, 37–38, 37f–38f
pancreatitis and, 522, 534, 585 shadowing artifact caused by, 40, 40f–41f
acute pancreatitis, complication of, 534, 572–574, 573f twinkling artifact and, 45–46, 46f
chronic pancreatitis, complication of, 534 Refraction, 7, 7f
pancreatic duplication cyst, differentiated from, and grayscale artifacts, 37–38, 38f
298–299, 570 Renal agenesis, 592, 638, 675, 677, 693, 696, 700f, 741–742, 742f,
Pseudogynecomastia, 949 748, 759
Pseudomass Renal artery pseudoaneurysm, 781, 783f
of cauda equina nerve roots, 110 Renal artery stenosis, 736, 779, 780f, 781
of pancreas, 566 renal transplantation and, 789
Pseudomembranous colitis, 340–343 Renal artery thrombosis, 781–782, 782f
Pseudosinus tract, 110, 110f renal transplantation and, 788–789, 788f
PTLD. See Post-transplant lymphoproliferative disorder Renal cell carcinoma (RCC), 571, 778–779, 804, 806, 807f, 810–811,
Pubertal disorders, female genital tract 811f, 935
994 Index

Renal cortical calcification, 798, 798f metastatic disease, 616, 616f

Renal cystic disease lymphatic malformations, 614, 615f
acquired cystic kidney disease, 779, 779f malignant tumors
autosomal dominant polycystic kidney disease, 775–776, 776f infantile fibrosarcoma, 620
autosomal recessive polycystic kidney disease, 775, 775f malignant germ cell tumor/immature teratoma, 620–621
classification, 774 rhabdomyosarcoma, 620, 621f
cystic renal dysplasia, 776, 776f neural crest tumors, 617–618, 619f–620f
glomerulocystic kidney disease, 777, 778f normal anatomy, 610, 610f, 611
imaging, 775 normal development, 609
medullary cystic disease, 777 schwannoma, 617, 618f
multicystic dysplastic kidney, 736, 736f, 745f, 776–777, 776f–777f smooth muscle tumors, 621
nephronophthisis, 776–777, 777f technique
tuberous sclerosis, 778, 778f imaging approaches, 609
von Hippel-Lindau disease, 778, 778f patient positioning, 609
Renal hypoplasia, 721, 746, 786 ultrasound transducer selection, 609
Renal medullary carcinoma, 812 undifferentiated pleomorphic sarcoma (malignant fibrous
Renal transplantation histiocytoma), 621
acute tubular necrosis, 791, 792f venous malformations, 614
abscess, 796–797, 797f Reverberation artifact(s), 24, 38–39, 38f–39f
allograft types, 786 A-lines, 175f, 176, 177t, 200, 200f
arteriovenous fistula, 789, 791f B-lines, 176, 176f, 186
drug nephrotoxicity, 792 foreign bodies and, 265–266, 266f
graft failure, 791 gas within abscess and, 539
hematoma, 796, 796f inspissated thyroid colloid and, 154
lymphocele, 796, 796f normal trachea and, 224, 224f
normal renal transplant, 788, 788f pneumothorax and stratosphere/barcode
posttransplant tumors, 797–798, 797f sign, 209, 209f
pseudoaneurysm, 789, 791f pulmonary consolidation and loss of, 176, 176f, 177t
pyelonephritis, 795–796, 795f Rhabdoid tumor(s)
rejection, 792, 793f of brain, 811, 812f
renal artery stenosis, 789 of kidney, 811, 812f
renal artery thrombosis, 788–789, 788f of liver, 403
renal vein thrombosis, 789, 790f Rhabdomyosarcoma
seroma, 796 Beckwith-Wiedemann syndrome and, 571
surgical technique, 787, 787f biliary tree
ureteral obstruction, 794 metastatic, 474–475
urine leak, 793–794, 794f primary, 403, 473–474, 474f
urinoma, 796 bladder, 678, 818, 821–822, 822f
vesicoureteral reflux, 794, 795f breast, metastatic, 960, 963
Renal vein thrombosis (RVT), 781–783, 784f, 789, 799 cervix, 720, 720f
renal transplantation and, 789, 790f chest wall
Repetitive scrotal microtrauma, 657 metastatic, 262
Respiratory distress syndrome (RDS), 186 primary, 240t, 258–259
Retroareolar cysts, 950–951. See also Obstructed glands of diaphragm, 279t, 280
Montgomery lung, 189–190
Retrocaval ureter, 752, 933–934 neck
Retroperitoneal vessels. See Vascular imaging, retroperitoneal vessels metastatic, 149f, 150–151
Retroperitoneum, non-vascular disorders. See also Adrenal gland(s); primary, 150, 151f
Pancreas; Vascular imaging; specific vessels ovary, metastatic, 711
benign masses pancreas, 587
hemangioma, 608, 617 paratesticular, 669, 673, 673f
lipoblastoma, 617 adenomatoid tumor differentiated from, 672
lipoma, 617 fibrous pseudotumor differentiated from, 670
mature teratoma, 617 peritoneal cavity, 546t, 551–553, 553f
neurofibroma and schwannoma, 617, 618f pleura, 210, 212–213, 213f
Ewing sarcoma, 621 prostate, 678
extramedullary hematopoiesis, 614 retroperitoneal
fibrosis, 613–614 metastatic, 616
hemorrhage, 613, 613f primary, 620, 621f
infection and abscess, 611–613, 611f–612f salivary gland, 167
inflammatory myofibroblastic tumor, 622 seminal vesicle, 678
kaposiform hemangioendothelioma, 608, 622, 622f testicle, 669
lymphadenopathy vagina, 720, 722–723, 722f
infection, 615 müllerian papilloma differentiated from, 722
lymphoma, 615–616, 616f paraurethral duct cyst differentiated from, 721
Index 995

Rheumatic disorders, 503–504, 854 SCM. See sternocleidomastoid muscle

macrophage activation syndrome and, 503–504 Scrofula (tuberculous cervical lymphadenitis), 146f
RICH. See Rapidly involuting congenital hemangioma Scrotal and spermatic cord fluid collections, 649–652, 651f–652f
Rib(s) Scrotum. See Male genital tract, scrotum
fractures, 206, 240t, 264–265, 508 Segmental spinal dysgenesis, 119
osseous and cartilaginous lesions, 251–255 Segmental testicular infarction, 639t, 641–642, 662
Ring-down artifact, 38, 540, 540f Semilobar holoprosencephaly, 64
Rosai-Dorfman-Destombes disease, 662 Seminal vesicles. See Male genital tract, prostate and seminal vesicles
Rotavirus, 316, 340 Septic arthritis, 850f, 851, 851f, 854, 854f, 877
Septo-optic (pituitary) dysplasia, 64–65
Sertoli cell tumors, 662t, 665, 665f
S Sertoli-Leydig cell tumor, 705t, 710–711, 948
SAAG. See Serum-ascites albumin gradient Serum-ascites albumin gradient (SAAG), 529, 529t
Sacrococcygeal teratoma, 122, 123f, 124, 124f Sex chord stromal tumors, 705t, 710
Salivary glands. See also Parotid glands; Sublingual glands; Sexual maturity rating, 943
Submandibular glands Shadowing attenuation artifact, 40, 40f–41f
benign masses Shear wave speed imaging, 34
infantile hemangioma, 139f, 166 Shoulder
lymphatic malformation, 144f, 166 glenohumeral dysplasia, 858–589, 858f–859f
congenital anomalies imaging approaches, 848f, 856–857, 856f–857f
first branchial cleft cyst, 134f, 163 normal anatomy, 856, 856f
ranula, 163, 163f patient positioning, 856
infectious and inflammatory disorders Shwachman-Diamond syndrome, 570–571, 571f
acute Sialadenitis, 164f–165f, 165
bacterial parotitis and abscess, 164–165, 164f chronic 165, 165f
viral (nonsuppurative), 164 Sialoblastoma, 167
recurrent and chronic Sialolithiasis, 167–168, 168f
chronic sialadenitis, 165, 165f Sickle-cell disease, 504–506, 505f
malignant neoplasms brain, 81–84, 83t, 84f
acinar cell carcinoma, 166–167, 167f stroke and, 81–82
adenoid cystic carcinoma, 167, 167f TCD ultrasound for, 82–84, 83t, 84f
lymphoma, 149–150 cholecystitis and, 445
primary lymphoma (MALToma), 167 extracranial stenosis of internal carotid artery and, 904
mucoepidermoid carcinoma, 166, 166f extramedullary hematopoiesis and, 516
rhabdomyosarcoma, 167 gallbladder sludge and, 442
sialoblastoma, 167, 167f gallstones and, 440, 441f, 444
parotid gland, 131, 132f Gamna-Gandy bodies and, 523
sialolithiasis, 167–168, 168f pneumonia, 176f
sublingual glands, 132f, 133 renal cortical calcification and, 798
submandibular glands, 132, 132f–133f Salmonella infection and, 316
Sarcoidosis spleen in, 487f, 491, 494, 505f, 506f
cervical lymph nodes, 146 Sickle-cell trait, 506, 812
epididymitis, 644 renal medullary carcinoma and, 812
interstitial lung disease, 186t SIDS. See Sudden infant death syndrome
mediastinum, 225t Side lobe artifact, 39, 39f, 442, 444f
spleen, 487t, 502–503, 503f Simple closed defect(s), of spine, 113–114, 114f–115f
Scalp masses Simple cyst(s), of thyroid, 152, 152f
characterization of, 93 Simple pleural effusion, 202, 207
congenital scalp lesions Sinding-Larsen-Johansson syndrome (Jumper’s knee), 886–887, 887f
cephalocele, 94, 94f Sinus histiocytosis, 662, 662t
dermoid and epidermoid cysts, 93–94, 94f Sinusoidal obstruction syndrome (SOS), 382t, 384–385, 384f
extracranial birth trauma, 95, 95f Sinus pericranii, 93, 96, 97f
high frequency linear array transducer, 92, 93f SIOP. See Society of Paediatric Oncology
lymph node, 94 Slice thickness artifact, 24, 40–41, 41f
vascular scalp lesions Slipped capital femoral epiphysis (SCFE), 877, 877f
infantile hemangioma, 95–96, 96f Small bowel
sinus pericranii, 96, 97f acquired obstruction
SCFE. See Slipped capital femoral epiphysis intussusception
Schistosomiasis ileocolic, 315–316, 315f–316f
of bladder, 772, 772f small bowel, 313–315, 314f
of liver, 382t benign masses
of spleen, 499–500, 500f duplication cyst, 324–326, 325f
Schizencephaly, 58t, 64, 67–69, 70f. See also Post-migration intestinal polyp, 327, 327f
disorders, of brain mesenteric cyst, 326, 326f
Sclerosing cholangitis, 443t, 471, 471f, 472, 474 vascular anomalies
996
Small bowel (cont.)
blue rubber bleb nevus syndrome, 327–328
cutaneous angiomatosis with thrombocytopenia, 328
infantile hemangioma, 327, 328f
congenital anomalies
duodenal atresia, 306, 307f
“double bubble” sign, 290, 306, 307f, 308
duodenal stenosis, 306
duodenal web, 306, 307f
duplication cyst, 324–326, 325f
ileal stenosis, 308–309
intestinal atresia, 307–308, 308f
“triple bubble” sign, of jejunal atresia, 308
jejunal stenosis, 308–309
Meckel diverticulum, 323–324, 324f
meconium ileus, 311–312, 312f
meconium peritonitis, 312–313
meconium pseudocyst, 312–313, 313f
midgut malrotation, 309–311, 309f–310f, 312f
Ladd bands, 309, 309f, 312f
malignant masses
Hodgkin lymphoma, 329–330, 329f
non-Hodgkin lymphoma, 329–330, 329f
midgut volvulus, 309–310, 309f, 311f
normal anatomy, 305, 306f
normal development, 291f, 305
technique
imaging approaches, 305
patient positioning, 305
ultrasound transducer selection, 305
wall thickening
Crohn disease, 317, 318f
cystic fibrosis, 321–322, 322f
eosinophilic gastroenteritis, 320–321
graft-versus-host disease, 322–323, 323f
hemorrhage
Henoch–Schönlein purpura, 319–320, 320f
trauma, 319, 319f
infectious enteritis, 316–317, 317t
lymphangiectasia, 321, 321f
Small bowel intussusception, 313–315, 314f, 323–324, 327,
327f, 329f
Society of Paediatric Oncology (SIOP), 810
and Wilms’ tumor management, 810
Society of Radiologists in Ultrasound (SRU)
consensus guidelines for follow-up of ovarian cysts, 703
Soft tissues
imaging approaches, 837–838, 837f–838f
infectious/inflammatory disorders
cellulitis, 838–839, 839f
pyomyositis, 839, 840f
soft tissue abscess, 839–840, 840f
normal anatomy, 837, 837f
muscles, 837–838, 837f–838f
skin, 837, 837f
subcutaneous tissues, 837, 837f
tendons, 838, 838f
trauma
fat necrosis, 840–841, 841f
foreign body(ies), 841, 842f
intramuscular hematomas, 841–842
muscle hernia, 843–844, 844f
muscle tears, 841–842, 842f
myositis ossificans, 843, 843f
tendinopathy, 844, 844f
tendon tears, 844, 845f
tumors
Index
lipoblastoma, 846–847, 847f
lipoma, 846, 846f
pilomatricoma, 845–846, 846f
subcutaneous granuloma annulare, 845, 845f
SOS. See Sinusoidal obstruction syndrome
Sound propagation, 2–4
Spatial compounding, 24, 24f
Speckle-tracking algorithms, 33–34
Spectral broadening, of Doppler waveform, 28, 30f, 30–31, 43–44
in infantile hemangioma, 166
in peripheral arterial stenosis, 915
in renal artery stenosis, 779, 789
and variance, 31
Spinal cord injury
epidural hematoma, 124
hematomyelia, 124
laceration and transection, 124
subdural hematoma, 124
Spinal dysraphism
clinical presentation, 111
closed defect, 111, 112f, 112t, 113–116, 117f–118f
cerebrospinal fluid-containing defects, 116, 118f
complex, 114–116, 115f
diastematomyelia, 115
neurenteric cyst, 114
neurenteric fistula, 114
lipomyelocele, 116, 117f
lipomyelomeningocele, 116, 117f
simple, 113–114, 114f–115f
dermal sinus, 113–114, 115f, 116
filar lipoma, 113–114, 114f, 119
tight filum syndrome, 112t, 114
neonatal spine ultrasound screening, 112
open defect, 111, 111f, 111t
meningocele, 112, 112t, 118f, 119f
myelocele, 112–113, 113f
myelomeningocele, 112–113, 112f–113f
Spinal lipoma, 119, 120f
Spine
congenital anomalies
caudal regression syndrome, 118–119, 119f
dysmorphic coccyx, 110, 111f
segmental spinal dysgenesis, 119
spinal dysraphism, 111–112, 111f, 111t–112t
spinal lipoma, 119, 120f
tethered cord, 116, 118, 118f
extramedullary tumors, 122, 123f
hydromyelia, 125
infectious and inflammatory disorders
epidural abscess, 120
pilonidal sinus and cyst. 120, 121f
neoplastic spinal disorders
extramedullary tumors, 122, 123f
intramedullary tumors, 122, 122f
sacrococcygeal teratoma, 122, 123f–124f, 124
normal anatomy
cauda equina, 105, 105f, 107f, 110
central canal, 108, 108f
fatty filum, 109–110, 110f
filar cyst, 109, 109f
positional nerve root clumping (“pseudomass”), 110
pseudosinus tract, 110, 110f
spinal cord, 107–108, 108f
ventriculus terminalis, 108–109, 109f
normal development, 104–105, 105f–106f
pilonidal sinus and cyst, 120–121, 121f
sacrococcygeal teratoma, 122–124, 123f–124f
Index
syringomyelia, 125
syrinx, 125, 125f
technique
imaging approaches, 104
M-mode ultrasound, and nerve root pulsations, 104
patient positioning, 103–104
ultrasound transducer selection, 104
traumatic spinal disorders
epidural/subdural hematoma, 124
hematomyelia, 124
lumbar puncture, 124, 124f
spinal cord injury, 124
spinal cord laceration and transection, 124
Spleen
acquired immunodeficiency syndrome, 501–502, 502f
American Association for the Surgery of Trauma (AAST)
classification of splenic injury, 508, 510f
anatomic variants
accessory spleen, 487, 489–490, 489f–490f
lobulations and clefts, 487, 488f
benign masses
extramedullary hematopoiesis, 516, 517f
hamartoma, 516, 516f
hemangioma, 517
inflammatory myofibroblastic tumor, 516–517
Littoral cell angioma, 517–518
congenital anomalies
asplenia, 491–492
hyposplenia, 491–492
nonparasitic splenic cysts, 493, 493f
polysplenia, 491–492, 492f
splenogonadal fusion, 493, 638f
splenopancreatic fusion, 493–494
wandering spleen, 490, 491f
contrast-enhanced ultrasound
of peritoneal cavity, 524
of spleen, 482, 490, 494–495, 496f, 511, 512f, 520f
Gamna-Gandy bodies, 500, 508, 523, 524f
granulomatosis with polyangiitis, 504, 537
hemoglobinopathies, 504–506, 505f–506f
infection
Epstein-Barr viral infection, 498, 498f
fungal abscess, 494–496, 495f–497f
parasitic infection, 498–501
Babesiosis, 500–501
Echinococcus granulosus infection, 499
daughter cysts, 499
hydatid cysts, 499
Leishmaniasis, 501
Malaria, 499
Schistosomiasis, 499–500, 500f
pyogenic abscess, 494, 494f
tuberculous infection, 497, 497f
inflammatory disorders
rheumatic disorders, 503–504, 504f
sarcoidosis, 502–503, 503f
Langerhans-cell histiocytosis, 521–522, 521f–522f
lysosomal storage diseases, 506–507, 506f–507f
Gaucher disease, spleen and, 506–507, 506f–507f
malignant tumors
angiosarcoma, 520–521
kaposiform hemangioendothelioma, 521
leukemia, 519, 520f
lymphoma, 518–519, 518f–520f
metastatic disease
neuroblastoma, 521
normal anatomy, 482, 484, 485f–486f, 486
997
normal development, 482, 484f
pancreatitis, complications of, 522, 523f
peliosis, 515–516
portal hypertension, 508, 509f
sarcoidosis, 502–503, 503f
splenomegaly, causes of, 486, 487t
splenosis, 511, 513f
technique
imaging approaches, 482, 483f
patient positioning, 482
ultrasound transducer selection, 482
trauma, 508–511, 510f–512f
CEUS, 509, 511, 512f
FAST scan, 508
vascular anomalies
kaposiform lymphangiomatosis, 513, 515
lymphatic malformation, 511–513, 514f
venous malformation, 513, 515f
Splenogonadal fusion, 493, 638, 638f
Splenomegaly, 482, 486, 487t, 497
Splenosis, 511, 513f
SRU. See Society of Radiologists in Ultrasound
SSWE. See Supersonic shear wave elastography
Sternocleidomastoid muscle (SCM), 128, 130–131, 134–135, 138,
139f, 902, 903f, 906, 907f
Stomach. See also Gastrointestinal tract
acquired obstruction
gastric volvulus, 294–296, 296f
hypertrophic pyloric stenosis, 292–293, 292f–293f
prostaglandin-induced foveolar hyperplasia, 294, 295f
pylorospasm, 293–294, 294f, 295t
benign masses
focal foveolar hyperplasia, 300
gastric bezoar, 301, 302f
gastric duplication cyst, 298–300, 299f–300f
gastric lipoma, 300
gastric teratoma, 300
inflammatory myofibroblastic tumor, 300–301
other benign masses, 301
congenital anomalies
gastric atresia, 290
gastric diaphragm (antral web), 292
microgastria, 290, 292
gastric wall thickening
chronic granulomatous disease, 298, 298f
eosinophilic gastroenteritis, 297–298
gastritis, 297, 297f
Ménétrier disease, 297
imaging approaches, 289–290, 289f
malignant tumors
gastrointestinal stromal tumor, 303, 304f
leiomyosarcoma, 303
lymphoma, 301–302, 303f
primary gastric adenocarcinoma, 303
normal anatomy, 285f, 290
normal development, 290, 291f
technique
imaging approaches, 289, 290
patient positioning, 288
ultrasound transducer selection, 289
STOP. See Stroke Prevention in Sickle Cell Anemia
Stroke Prevention in Sickle Cell Anemia (STOP), 82, 83t
Subacute (De Quervain) thyroiditis, 159, 160f
Subclavian artery, 45f, 128, 244, 902, 902f,
910f, 927
Subcutaneous granuloma annulare, 845, 845f
Subdural hemorrhage, 74–75
998 Index

Subgaleal hematoma, 95, 95f ultrasound appearance, 662, 662t

Sublingual glands, 132f, 133 yolk sac tumors, 662–663, 664f
Submandibular glands, 128, 132f, 133f, 164, 167, 168f secondary testicular tumors
Subpial hemorrhage, 75, 75f carcinoid, 669
Sudden infant death syndrome (SIDS) Langerhans-cell histiocytosis, 669
and positional plagiocephaly, 97 leukemia, 668
Superficial femoral artery, 910, 913–914, 921f lymphoma, 668
Superior sagittal sinus thrombosis, 82f neuroblastoma, 668–669
Supersonic shear wave elastography (SSWE), 35, 35f retinoblastoma, 669
Surfactant deficiency disease, 176, 186, 187f rhabdomyosarcoma, 669
Syringomyelia, 113, 125 Wilms’ tumor, 669
Syrinx, 61, 112t, 116, 121, 125 Testicular microlithiasis, 652–653, 653f
testicular tumors and, 653
Testicular regression syndrome, 636–637
T Testicular torsion, 639–641, 639t, 640f–642f. See also Acute scrotal
Talocrural joint. See Ankle pain
Tanner staging, of breast development, 943, 944t, 945f–946f, 946–947 Testis-determining factor, 686, 687
Tardus-parvus waveform(s) Tethered cord, 113–114, 114f, 115f, 116–118, 118f
in hepatic artery after liver transplantation, 408t, 415, 417, 417f Thalamo-occipital distance, 92, 92f
stenosis, 408t, 417, 417f Thalamus, 53, 56, 56f, 57–58, 58f, 59, 78, 80f, 86f, 88, 92, 92f
thrombosis, 408t, 415t Thecoma-fibroma, of ovary, 710
in peripheral artery stenosis, 915, 915 Thermal bioeffect(s), 47–48
in renal artery stenosis, 779 Thermal index (TI), 48
in renal artery stenosis after renal transplantation, 789 THI. See Tissue harmonic imaging
TCC. See Transitional cell carcinoma Thoracic skeleton, 241, 243
TCD. See Transcranial Doppler normal anatomy, 241, 243
Teratoma(s) normal development, 241–244
adnexal torsion and, 713 Three-dimensional (3D) ultrasound, 25–26, 25f, 52, 274, 685f, 686
adrenal, 608, 608f Three-dimensional ultrasound artifacts, 47
of brain, 88 Thrombosis. See also Deep vein thrombosis
cervical, 136–137, 137f aorta, 927–928, 929f
gastric, 300 carotid artery, 902–905, 905f
mediastinal, 225–226, 226f hepatic artery, after liver transplantation, 408t, 410, 414, 415, 416f
ovarian, 705t, 706, 706f–707f IJV, central line placement causing, 906, 908, 909f
pancreatic, 579–580, 580f IVC, 934–936, 935f–936f
retroperitoneal, 617, 620 after liver transplantation, 408t, 422, 422f
sacrococcygeal, 122, 124, 123f–124f portal vein, 374, 382–383, 382t, 453, 453f, 487t, 529t
testicular, 662–663, 662t, 664f after liver transplantation, 408t, 418–419, 419f
Terminal myelocystocele, 112t, 116 after umbilical vein catheterization, 382
Testicular ectopia, 636f, 637–638 renal artery, 781, 782f
Testicular hypoplasia, 637, 637f after renal transplantation, 788–789, 788f
Testicular mass(es) renal vein, 781–783, 784f
leiomyoma, 668 calcifications, 784f, 799
non-neoplastic lesions after renal transplantation, 789, 790f
adrenal rests, 660–661, 661f splenic vein, pancreatitis complicated by, 522, 523f, 576, 576f
hamartoma, 661 venous sinus, 81, 82f
Leydig cell hyperplasia, 661 Thymus
simple cyst, 661–662, 661f anterior mediastinal masses and, 225
sinus histiocytosis, 662 cervical extension, 135f, 138, 138f
primary testicular tumors, 662t ectopic, 138, 138f, 224f, 225t
adenomatoid , 668 glands and, 131, 131f
capillary hemangioma, 667, 667f lymphoma, 226
choriocarcinoma, 664 normal anatomy, 220–221, 223, 223f
embryonal carcinoma, 663–664 normal development, 130f
epidermoid cyst, 666, 667f ultrasound imaging of, 220
germ cell, 662, 663f Thyroglossal duct cyst, 133, 133f, 134f
gonadoblastoma, 663 Thyroid cancer, 152–153, 155, 156f, 156t
leiomyoma, 668 Thyroid gland
lipoma, 667 congenital anomalies
seminoma, 663 dysgenesis, 151–152, 151f
stromal tumors, 665, 665f dyshormonogenesis, 152
granulosa cell, 665, 666f diffuse parenchymal lesions
Leydig cell, 665, 665f autoimmune, 159–161
Sertoli cell, 665, 665f Graves’ disease, 159–160, 160f
teratocarcinoma, 664 congenital goiter, 158f
teratoma, 663, 664f fetal goiter, 158
Index 999

fetal hypothyroidism, 157–158 fibromatosis colli and, 138, 139f

Hashimoto thyroiditis, 160–161, 161f parenchymal hemorrhage, of brain, 75
infectious thyroiditis spine, 124
acute suppurative (bacterial), 159 venous sinus thrombosis, 81
subacute (De Quervain), 159, 160f bladder, 802–803
multinodular goiter (nodular hyperplasia), 157–159, 158f CEUS and, 802f
focal lesions chest wall, 240t, 262–265, 263f–264f
cystic lesions foreign body(ies), 265–266, 265f–266f
colloid cysts, 152, 152f classic metaphyseal lesion, 852, 853f
complex (hemorrhagic) cysts, 152, 153f diaphragm
simple cysts, 152, 152f eventration, acquired, 277
solid lesions, 152–157, 154f–157f, 156t phrenic nerve injury, 278
American College of Radiology (ACR) TI-RADS rupture, 280
criteria, 153 distal humeral epiphyseal separation, 864, 865f
benign duodenum, 319, 319f
follicular adenoma, 152, 154, 154f epididymitis/epididymo-orchitis, 644
hyperplastic (adenomatous) nodules, 154–155, 155f epiphyseal separation, 851–852, 852f
malignant lesions fat necrosis, 840–841, 841f
lymphoma, 157, 157f Fournier gangrene, as complication of, 649, 650f
thyroid cancer, 155–156, 156f, 156t gallbladder, 453, 454f
Thyroid Imaging Reporting and Data System (TI-RADS), 153 hemoperitoneum, 530
Thyroid inferno, 160 kidney, 801–802
Tibial hemimelia, 882, 882f CEUS and, 802, 802f
Tietze syndrome, 253 liver
Tight filum syndrome, 112t, 113–114 blunt abdominal, 380, 380f–381f
TI-RADS. See Thyroid Imaging Reporting and Data System e-FAST and, 380
Tissue harmonic imaging, 24. See also Harmonic imaging umbilical vein catheterization and, 380–382, 381f
Tissue vibration artifact, 45 meniscal tears, 885–886, 886f
and arteriovenous fistula, 45f, 784, 785f, 917, 918f, 918t pancreas, 577–579, 577f–578f
and renal artery stenosis, 789 pancreatitis and, 572–573
TORCH infections patellar tendon
and neonatal hepatitis, 463 Osgood-Schlatter disease, 844f, 886
and neonatal/infant brain, 84, 85f Sinding-Larsen-Johansson syndrome, 886–887, 887f
Torsion, testicular. See Testicular torsion peritoneal inclusion cyst, 535, 535f
Transcranial Doppler (TCD) ultrasound, for sickle-cell disease, 82–84, pleural effusion and, 205–207, 205f
83t, 84f renal artery, 781, 783f
Trachea renal vein, 781–783
normal anatomy, 221, 222f, 224, 224f retroperitoneum, 613
normal development, 174, 220 scrotum, 653–657, 654f–656f
Transducer, 12, 12f. See also specific organs hematocele, 651–652
array, 19–22, 20f–22f slipped capital femoral epiphysis, 877–878, 877f
broad bandwidth, in harmonic imaging, 24 soft tissues
mechanical, 14–19, 15f–19f cellulitis, 838–839, 839f
selection of, 22–23, 22f foreign body(ies), 841, 842f
Transient tachypnea of the newborn (TTN), 186, 187f muscle tears/intramuscular hematomas, 841–842, 842f
Transitional cell carcinoma, of bladder, 822–823, 823f myositis ossificans, 843, 843f
Transmit beamformer, 11, 11f, 12, 19 pyomyositis, 839, 840f
Transmitter, 11–12, 29 tendon tears, 844, 845f
Trauma. See also Child abuse; Foreign body(ies); Non-accidental spine
trauma; Pseudoaneurysm(s) lumbar puncture, 103, 124, 124f
adrenal hemorrhage and, 597 spinal cord, 124
apophyseal avulsion, 864, 865f, 889, 889f spleen, 508–511, 509f–512f
arteriovenous fistula CEUS and, 482
of kidney, 783–784, 785f cyst and, 493
of liver, 366 FAST and, 508
of neck 140 rupture, 505
ascites splenosis and, 511, 513f
chylous, 531, 531f testicle
urine, 532 torsion, as etiology for, 640
biliary tract, 472–473 vagina
biloma, 533, 534f epidermal inclusion (epidermoid) cyst, 721
birth “Triple bubble” sign, of jejunal atresia, 308
chylous effusion, 206–207 Trisomy 21
cranial vault and scalp, 93, 95, 95f annular pancreas, associated with, 567, 567f
diaphragmatic dysfunction and, 278 delayed closure of anterior fontanelle, associated with, 52
intracranial, extra-axial hemorrhage, 70, 72, 74–75, 74f–75f duodenal atresia, associated with, 306
1000 Index

Trisomy (cont.) Ureter

duodenal web, associated with, 306 acquired ureteral obstruction
enlarged prostatic utricle, associated with, 675 extrinsic compression, 767
gastric atresia, associated with, 290 intraluminal obstruction, 766–767, 767f
lymphangiectasia, diffuse, associated with, 321 bulking agents, as treatment for vesicoureteral reflux, 755, 756f
splenopancreatic fusion, associated with, 493 contrast-enhanced voiding urosonography, 337, 740f–742f, 755,
TTN. See Transient tachypnea of the newborn 755f, 765, 766f, 768, 794
Tuberous sclerosis, 779, 810 congenital ureterovesical obstruction, 751
angiomyolipoma and, 804, 804f ectopic ureter, 752, 752f–753f
cysts of kidney and, 778, 778f ectopic ureterocele, 752
glomerulocystic disease of kidney and, 777 normal anatomy, 738, 738f
hamartoma of spleen and, 516 primary ureteral tumors, 815–816
renal artery stenosis and, 779 fibroepithelial polyp, 815
renal cell carcinoma and, 810 retrocaval ureter, 752, 933–934
Turner syndrome, 140, 663, 701, 701f, 707, 745 secondary ureteral tumors, 816, 816f
Twinkling artifact, 45–46, 46f, 199, 312, 441, 451, 469f, 600f, 799, Wilms’ tumor extension, 816, 816f
801f ureteral jets, 738, 739f
calcified adrenal ganglioneuroblastoma and, 600f ureteropelvic junction obstruction, 746–748, 748f
calcified meconium pseudocyst and, 312 urinary tract dilation classification, 746, 747f
choledocholithiasis and, 469f urothelial tumor, 816
gallstones and, 441, 441f vesicoureteral reflux, 752–755, 754f
renal stones and, 42f, 45–46, 46f Ureteropelvic junction, 738
ureteral stone and, 801f Ureteropelvic junction obstruction, 746–748, 748f
Two-dimensional array transducers, 25–26, 26f Ureterovesical junction, 738, 801f
Ureterovesical junction obstruction. See Congenital ureterovesical
obstruction
U Urethra
UCA. See Ultrasound contrast agents anterior urethral valves, 765–766
UDCA. See Ursodeoxycholic acid female urethra, 741, 741f
Ulcerative colitis, 317, 337, 338f, 339, 471f, 535f male urethra, 741, 741f–742f
Ultrasound air bronchogram, 176, 177f, 177t, 183, 188, 599 normal anatomy, 740–741
Ultrasound contrast agents (UCA), 35–36, 36f, 257, 358, 802 posterior urethral valves (PUV), 764–765, 764f–766f
microbubbles, 35–37, 36f urethral duplication, 766
and cavitation, 48 urethral polyp, 824
Ultrasound safety Urinary diversion, 759, 771, 800, 824–825, 825f
nonthermal bioeffects, 48 Urinary tract
regulations and policies, 48 anatomic variants
thermal bioeffects, 47–48 bladder
Umbilical artery bladder ears, 740, 740f
aortic thrombosis and catheterization of, 927, 928 kidney
lower extremity development and, 913 accessory renal artery, 736–737, 737f
renal artery thrombosis and catheterization of, 17 circumaortic left renal vein, 738, 738f
Umbilical vein compensatory hypertrophy, 736, 736f
liver development and, 360, 361f, 362, 362f. 365f compound calyx, 736, 736f
perforation from malpositioned catheter in, 380–382, 381f dromedary hump, 735, 735f
portal hypertension as complication of catheterization, 382 fetal lobulations, 734, 734f
portal vein atresia and, 368 hypertrophied column of Bertin, 735, 735f
portal venous gas secondary to catheterization, 386 junctional parenchyma defect, 735, 735f
recanalization in portal hypertension, 382, 383f retroaortic left renal vein, 737, 737f
Umbilical vein catheter (UVC), 380–382, 381f calcification
Undifferentiated embryonal sarcoma, 389t, 392, 402, 402f dystrophic calcification, 799, 799f
UPJ. See Ureteropelvic junction medullary nephrocalcinosis, 798, 798f
UPJO. See Ureteropelvic junction obstruction renal cortical calcification, 798, 798f
Upper extremity renal vein thrombosis calcifications, 784f, 799
arteries urinary stasis, 799, 800f
anatomy, 913, 913f congenital anomalies
development, 913 bladder
subclavian artery, 913, 913f agenesis, 762, 763f
veins cloacal exstrophy, 759
anatomy, 922, 922f cloacal malformation, 760–761, 761f
development, 922 diverticula, 758, 759f
Urachal anomalies duplication, 761–762, 762f
patent urachus, 757, 757f exstrophy, 758, 760f
umbilico-urachal sinus, 758 megacystis-microcolon-intestinal peristalsis syndrome, 763
urachal cysts, 758, 758f prune-belly syndrome, 762–763, 763f
vesicourachal diverticulum, 757, 758f renal collecting system and ureter
Index 1001

calyceal diverticulum, 750, 751f parasitic infection, 772, 772f

congenital infundibulopelvic stenosis, 748–749, 750f prevalence, 768
congenital megacalyces, 748, 748f pyonephrosis, 770, 770f
congenital ureterovesical junction obstruction, 751, 751f renal abscess, 769–770, 770f
ectopic ureter, 752, 752f–753f xanthogranulomatous pyelonephritis, 773, 774f
ectopic ureterocele, 744f, 752 Urolithiasis
retrocaval ureter, 752, 933–934 bladder stones, 767, 800, 801f
ureteropelvic junction obstruction, 746–748, 748f causes, 799
ureteropelvic junction obstruction and crossing vessel, 748 color Doppler twinkling artifact and, 799
vesicoureteral reflux, 752, 754–755, 754f renal stones, 799, 800f, 801f
contrast-enhanced ultrasound diagnosis of, 755, 755f risk factors, 800
imaging of endoscopically placed bulking agents, ureteral jet and UVJ stone, 800, 801f
755, 756f Urothelial carcinoma, 822–823
anomalies of renal number, position, fusion and growth Ursodeoxycholic acid (UDCA), and treatment for
crossed renal ectopia, 744, 745f gallstones, 443
horseshoe kidney, 745, 746f UTD classification. See Urinary tract dilation
pancake kidney, 745–746 UTI. See Urinary tract infection
renal agenesis, 741–742, 742f UVC. See Umbilical venous catheter
renal duplication, 742–743, 743f–744f UVJ. See Ureterovesical junction
renal hypoplasia, 746 UVJO. See Ureterovesical junction obstruction
simple ectopia, 744, 745f
supernumerary kidney, 743–744, 744f
anomalies of urethra V
anterior urethral valves, 765–766 VACTERL
posterior urethral valves, 764–765, 764f–765f and anorectal malformations, 335
contrast-enhanced ultrasound diagnosis of, 765 and duodenal atresia, 306
urachal anomalies, 755–758, 756f–757f Vagina
patent urachus, 757, 757f benign tumors
umbilico-urachal sinus, 758 Bartholin cyst, 721
urachal cyst, 758, 758f fibroepithelial polyp, 722
vesicourachal diverticulum, 757, 758f Gartner duct cyst, 720–721, 721f
urethral duplication, 766 inclusion cyst, 721
metanephric blastema, 731 müllerian papilloma, 722
normal anatomy paraurethral duct cyst, 721–722, 722f
bladder, 738–739, 739f foreign body, 723, 723f
kidney malignant tumors
infant, 734, 734f clear cell adenocarcinoma, 722–723
older child and adolescent, 734, 734f endodermal sinus tumor, 722–723
ureter, 738 rhabdomyosarcoma, 722, 722f
ureteral jets, 738, 739f müllerian agenesis and, 692, 692f
urethra, 740–741, 741f–742f normal anatomy, 686f–687f, 687–690
female, 741, 741f–742f Valsalva maneuver
male, 741, 741f common bile duct size and, 456
normal development, 731, 731f–733f deep vein thrombosis and, 922t, 925
technique inguinal hernia and, 630, 657
imaging approaches, 730 internal jugular vein and, 900, 906, 908
contrast-enhanced ultrasound, 730–731 spermatic cord hydrocele and, 650
elastography, 731 varicocele and, 630, 659–660, 659f–660f
patient positioning, 730 venous malformations and, 140, 250, 892
ultrasound transducer selection, 730 Varicocele, 659, 659f
trauma intratesticular varicocele, 660, 660f
bladder, 802–803, 802f Vascular anomaly(ies). See also specific organs
contrast-enhanced ultrasound diagnosis, ISSVA classification, 889, 890t
801–802, 801f overview, 889
renal, 801 vascular malformations
urinary stasis, 799, 800f arteriovenous fistula, 893–894
urogenital ridge, 731 arteriovenous malformation, 893–894, 894f
urorectal septum, 731 lymphatic malformation, 892, 893f
Urinary tract dilation (UTD) classification, 746, 747f venous malformation, 892, 892f–893f
Urinary tract infection (UTI) vascular tumors
acute pyelonephritis, 768–769, 769f congenital hemangioma, 890–891, 891f
chronic pyelonephritis, 772–773, 773f infantile hemangioma, 889–890, 890f
cystitis, 773–774, 774f kaposiform hemangioendothelioma, 891, 891f
fungal infection, 770–771, 771f Vascular disorders, of brain
imaging evaluation, 768, 768t high flow malformations, 90
opportunistic infection, 772 low flow malformations, 90
1002 Index

Vascular imaging aneurysm, 929–931, 930f

extremities dissection, 931, 931f
arteries normal anatomy, 927, 928f–929f
anatomic variants, 914 normal development, 927
aneurysm, 916, 918t stenosis, 928–929, 930f
arteriovenous fistula, 916–917, 918f, 918t thrombosis, 927–928, 929f
grayscale imaging, 910, 912 inferior vena cava
lower extremity duplication, 934
normal anatomy,913–914, 914f interruption, 933, 934f
normal development, 913 left-sided, 934, 935f
pseudoaneurysm, 916, 917f, 918t normal anatomy, 931–933, 931f–933f
stenosis and thrombosis, 914–916, 915f, 916t normal development, 931
technique retrocaval ureter, 752, 933–934
imaging approaches, 910, 910f–912f thrombosis, 934–936, 935f–936f
patient positioning, 909–910 May-Thurner syndrome, 936
ultrasound transducer selection, 910 technique
upper extremity imaging approaches, 927
normal anatomy, 913, 913f patient positioning, 927
normal development, 913 ultrasound transducer selection, 927
veins VACTERL association, 306, 335
anatomic variants, 923, 924f VATER association, 116
deep vein thrombosis Vein of Galen malformation (VOGM), 90, 91f
acute, 923, 925–926, 925f Veno-occlusive disease. See Sinusoidal obstruction syndrome
causes, 923 Velocity, 27
chronic, 926–927, 926f Venous malformation(s) (VMs). See also Blue rubber bleb nevus
lower extremity syndrome
normal anatomy, 922–923, 923f–924f bladder, 816, 817f
normal development, 922 breast, 954
technique chest wall, 248t, 250, 250f
imaging approaches, 919–920, 920f–921f, 922, 922t GI tract, 327
patient positioning, 918 musculoskeletal system, 890t, 892, 892f
ultrasound transducer selection, 918–919, 919f neck, 140, 143f
upper extremity peritoneal cavity, 542, 545f
normal anatomy, 922, 922f scalp, 89
normal development, 922 scrotum, 671
neck vessels spleen, 487t, 513, 515f
carotid artery Venous sinus thrombosis, 81, 82f
anatomic variants, 902 Ventral induction disorders
aneurysm, 905 corpus callosum, 65–67, 66f–67f
color and power Doppler imaging, 900–901, 900f Dandy-Walker syndrome, 67–68, 67f
common carotid artery, 130, 148f, 900–901, 900f, 904f holoprosencephaly, 63–64, 64f
dissection, 905–906 septo-optic dysplasia, 64–65, 65f
external carotid artery, 902, 904f Ventricles, 59–60, 59f–60f
frequency spectrum, 902, 903f Ventriculus terminalis (fifth ventricle), 108–109, 109f
intima-media thickness, 902, 903f Vesicoureteral reflux (VUR), 742–743, 743f, 752–755, 754f–755f,
internal carotid artery, 63, 83, 83f, 900f, 901 759, 794, 795f
normal anatomy, 902, 902f–904f VHL. See von Hippel-Lindau disease
normal development, 901 Viral hepatitis, 375, 375f
subclavian artery, 45f, 128, 244, 468, 902, 910f, 913, 927 Viral (nonsuppurative) inflammation, of salivary glands, 164, 164f
thrombosis and stenosis, 902–904, 905f Virginal hypertrophy of breast, 948
internal jugular vein Visceral (middle) compartment, of mediastinum, 221
anatomic variants, 906, 907f VOGM. See Vein of Galen malformation
aneurysm, 908 von Hippel-Lindau disease (VHL), 571–572
development, 906 pheochromocytoma and, 606–607, 607f
jugular vein phlebectasia, 900f, 906, 908f renal cell carcinoma and, 778, 810
normal anatomy, 901f, 906, 907f renal cysts in, 778, 778f
normal development, 906 serous cystadenoma of pancreas and, 579
stenosis, 908 testicular cyst and, 661
thrombosis, 906, 908, 909f
venous aneurysm, 908
technique W
imaging approaches, 900–901, 900f–901f WAGR syndrome, 806, 810
patient positioning, 899 Walker-Warburg syndrome, 67
ultrasound transducer selection, 899–900 “Wall echo shadow” (WES) sign, of cholelithiasis, 441, 442f
retroperitoneal vessels Wall filter, 29, 30, 32, 42, 42f, 44, 174, 334, 910
aorta Wandering spleen, 490, 491f
Index 1003

Waveform(s). See also Tardus-parvus waveform(s) ovary, 711

aliasing and, 43 peritoneal cavity, 546t, 555
in adnexal torsion, 713, 713f pleura, 214, 214f
of aorta, normal, 927, 929f retroperitoneal lymph nodes, 616
in aortic stenosis, 928–929, 930f testicle and scrotal structures, 669, 673
in aortic thrombosis, 927, 929f ureteral extension of, 816, 816f
arterial, normal, 30f, 84f, 357, 357f, 483f, 634, 634f, 902, nephroblastomatosis, and 810, 810f
903f–904f, 910, 910f–912f nephrogenic rests and, 810, 810f
in arterial stenosis of extremity, 915, 915f renal biopsy and, 810
in arteriovenous fistulas, 140, 142f, 784, 785f, 893 screening of contralateral kidney, 807, 809f
in arteriovenous malformations, 140, 141f, 671, 893, 894f tumor invasion of renal vein and inferior vena
in deep vein thrombosis of extremity, 923, 924f–925f, 925–926 cava, 807, 809f, 936f
hepatic vein Wolman disease, 594
in Budd-Chiari syndrome, 383, 383f World Federation for Ultrasound in Medicine and Biology
in passive venous congestion, 386, 386f (WFUMB), 48
obstruction after liver transplantation, 420 Wrist and hand
in epididymitis/epididymo-orchitis, 639t normal anatomy, 866, 866f
of IVC, normal, 933, 933f carpal boss, 869, 869f
in IVC thrombosis, 935 ganglia, 868, 868f
of liver vasculature after transplantation, 408t, 412 giant cell tumor of the tendon sheath, 869, 869f
in internal jugular vein thrombosis, 908, 909f imaging approaches, 867, 867f
in pseudoaneurysm, 916–917, 917f patient positioning, 867
in renal vein thrombosis after renal transplantation, 789, 790f
in splenic vein thrombosis, 522, 523f
in testicular torsion, 641, 642f X
in vascular masses, 93, 95–96, 96f–97f, 248, 248t, 249f, 817f, 890, Xanthogranulomatous adrenalitis, 595
892, 892f Xanthogranulomatous cholecystitis, 449
venous, normal, 357, 357f, 367, 483f, 634, 635f, 919–920, Xanthogranulomatous pyelonephritis, 773, 774f
920f–921f
in venous malformations, 140, 143f
Wavelength, and medical applications of ultrasound, 2, 2f Y
Weigert-Meyer rule, 742, 752 Yersinia, 316, 340
Wegener granulomatosis. See Granulomatosis with polyangiitis “Yin-yang” pattern of pseudoaneurysms, 918t
WFUMB. See World Federation for Ultrasound in Medicine and of hepatic artery, in liver transplantation, 408, 418, 418f
Biology of peripheral arteries, 916, 917f
Wharton duct, 132, 167 of renal artery, 781, 783f
Whirlpool sign and renal transplant biopsy, 789, 791f
of adnexal torsion, 713 Yolk sac tumor
of apple peel intestinal atresia, 308, 310 diaphragm, 279t, 280, 280f
of midgut volvulus, 310, 311f ovary, 705t, 706, 708, 708f
of segmental midgut volvulus, 310 testicle, 662t, 662–663, 664f
of testicular torsion, 640
Wilms’ tumor, 40f, 803, 806–808, 808f–809f, 810
Beckwith-Wiedemann syndrome and, 571 Z
differentiation from neuroblastoma, 807–808 Zellweger syndrome, 68, 777
metastases Zika virus, 86
biliary tree, 474 Zona fasciculata, 589, 590f, 591f
liver, 403 Zona glomerulosa, 589, 590f, 591f
lung, 190, 191f Zona reticularis, 589, 590f, 591f