Subhasis Roy Choudhury

Pediatric Surgery

A Quick Guide to
Decision-making

123
Pediatric Surgery
Subhasis Roy Choudhury

Pediatric Surgery
A Quick Guide to Decision-making
Subhasis Roy Choudhury
Kalawati Saran Children’s Hospital
Lady Hardinge Medical College
New Delhi
India

ISBN 978-981-10-6303-9    ISBN 978-981-10-6304-6 (eBook)

https://doi.org/10.1007/978-981-10-6304-6

Library of Congress Control Number: 2018930090

© Springer Nature Singapore Pte Ltd. 2018

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Dedicated to my parents, family, patients, and students.
Foreword

Professor Roy Choudhury has assembled this book of “Pediatric Surgery: A

Quick Guide to Decision Making”, that is beautifully illustrated and provides
a concise guide to the recognition and treatment alternatives. This book will
allow even those who do not practice surgery involving children to recognize
most of the conditions found in pediatric patients that require surgical correc-
tion. The scope of this book provides those who are active in the surgical care
of children an idea to apply the most recent treatment techniques giving their
patients the most modern experience practiced in a variety of centers around
the world. This book truly belongs in the library of diagnosticians and sur-
geons whose practice includes pediatrics.

Children’s Mercy Hospital Keith W. Ashcraft, M.D.,

Kansas City, MO, USA

vii
Preface

Pediatric surgery is currently an established specialty covering a vast spec-

trum of surgical conditions affecting the fetus to adolescents. In this book, the
common surgical diseases affecting children are presented in a concise for-
mat with up-to-date information. The text is presented in an easy reading
language supported with clinical photographs and illustrations for rapid ref-
erence and assimilation. An attempt has been made to provide the informa-
tion in a systematic pattern like embryology, incidence, approach to diagnosis,
and management with the help of flowcharts and algorithm wherever appro-
priate. The aim is to make the reader familiar with pediatric surgical condi-
tions and plan a simple approach to the management. This book covers the
subject of pediatric surgery and its allied subspecialties of pediatric urology,
thoracic surgery, gastrointestinal surgery, oncology, and trauma in a concise
and reader-friendly manner with the latest and up-to-date information.
The recent advances in pediatric surgery including antenatal diagnosis,
fetal interventions, and minimally invasive surgery have been covered.
I acknowledge the help of my esteemed colleagues Prof. Rajiv Chadha,
Dr. Niyaz Ahmed Khan, Dr. Pinaki Ranjan Debnath, Dr. Vikram Khanna,
Dr. Pratap Singh Yadav, Dr. Nitin Pant, and Dr. Amit Gupta for providing
me with clinical information and photographs. I especially acknowledge
Dr. Vishesh Jain for revising the manuscript and Dr. Vikram Khanna for
editing diagrams. I extend my gratitude to my teachers especially Prof.
Soumen Kumar Mitra, Prof. K. L. N. Rao, Dr. K. L. Narasimhan, Dr. Keith
W. Ashcraft, and Dr. Sean Corkery for their inspiration in the pursuit of
excellence. The support and suggestions of my wife Dr. Ruma and daughter
Dr. Shayeri were invaluable. In compilation of the material in this book,
several textbooks and original published articles of several authors and
websites have been consulted which are gratefully acknowledged. I thank
the staff of Springer India for their constant encouragement and support,
without which it would not have been possible to publish this book. My
heart goes out to all the patients who have taught me and many others
through their suffering.
I hope that the medical students, pediatricians, resident doctors in pediat-
rics and surgery, and general practitioners treating children will find the infor-
mation in this book very useful.

New Delhi, India Subhasis Roy Choudhury

ix
Contents

Part 1 General

1 Fluid and Electrolyte Balance in Children����������������������������������    3

Accessing Fluid and Electrolyte Status��������������������������������������������    4
History������������������������������������������������������������������������������������������    4
Clinical Evaluation����������������������������������������������������������������������    4
Cardiovascular Signs��������������������������������������������������������������������    4
Laboratory Evaluation�����������������������������������������������������������������    4
Fluid and Electrolyte Balance������������������������������������������������������    4
Maintenance Fluid Requirement��������������������������������������������������    4
Replacement Fluid ����������������������������������������������������������������������    5
Deficit Therapy����������������������������������������������������������������������������    5
Blood Volume������������������������������������������������������������������������������    5
Electrolyte Disturbances��������������������������������������������������������������    6
Hyponatremia������������������������������������������������������������������������������    6
Management��������������������������������������������������������������������������������    6
Hypernatremia������������������������������������������������������������������������������    6
Potassium ������������������������������������������������������������������������������������    7
Hyperkalemia������������������������������������������������������������������������������    7
Hypokalemia��������������������������������������������������������������������������������    7
Hypocalcemia������������������������������������������������������������������������������    8
Management��������������������������������������������������������������������������������    8
Hypoglycemia������������������������������������������������������������������������������    8
Hypothermia��������������������������������������������������������������������������������    8
Neonatal Hyperbilirubinemia������������������������������������������������������    9
Physiologic Jaundice��������������������������������������������������������������������    9
Pathological Jaundice������������������������������������������������������������������    9
Causes of Neonatal Hyperbilirubinemia��������������������������������������    9
Indications for Phototherapy and Exchange Transfusion������������    9
Suggested Reading��������������������������������������������������������������������������   10
2 Vascular Access in Children����������������������������������������������������������   11
Types of Catheters����������������������������������������������������������������������������   11
Peripheral Catheters ��������������������������������������������������������������������   11
Venous Cutdown��������������������������������������������������������������������������   11
Intraosseous Catheters ����������������������������������������������������������������   11
Peripherally Inserted Central Catheters (PICC)��������������������������   12

xi
xii Contents

Central Venous Catheters ������������������������������������������������������������   13

Implantable Vascular-Access Devices (Ports)������������������������������   13
Other Vascular-Access Sites������������������������������������������������������������   15
Umbilical Vascular Access����������������������������������������������������������   15
Arterial Vascular Access��������������������������������������������������������������   15
Principles of General Care for the Vascular Access��������������������   15
Complications of Vascular Access ����������������������������������������������   15
Long-Term Complications ����������������������������������������������������������   15
Catheter Dislodgement and Migration����������������������������������������   16
Infection ��������������������������������������������������������������������������������������   16
Thrombosis����������������������������������������������������������������������������������   16
Suggested Reading��������������������������������������������������������������������������   16
3 Parenteral Nutrition����������������������������������������������������������������������   17
Normal Pediatric Growth ����������������������������������������������������������������   17
Indications for Giving Parenteral Nutrition ������������������������������������   17
Administration of Total Parenteral Nutrition (TPN)������������������������   17
Composition of Parenteral Formulas ����������������������������������������������   18
Amino Acids��������������������������������������������������������������������������������   18
Dextrose ��������������������������������������������������������������������������������������   18
Lipid Emulsions ��������������������������������������������������������������������������   18
Multivitamins ������������������������������������������������������������������������������   18
Trace Elements����������������������������������������������������������������������������   18
Electrolytes����������������������������������������������������������������������������������   18
Heparin����������������������������������������������������������������������������������������   18
Complications����������������������������������������������������������������������������������   18
Monitoring Patients on TPN������������������������������������������������������������   19
Suggested Reading��������������������������������������������������������������������������   19
4 Antenatal Diagnosis and Fetal Surgery ��������������������������������������   21
Fetus as a Patient������������������������������������������������������������������������������   21
Screening Tests����������������������������������������������������������������������������   23
Antenatal Diagnosis and Treatment ������������������������������������������������   26
Fetal Surgery��������������������������������������������������������������������������������   27
MIS (Minimally Invasive) Fetal Surgery ������������������������������������   27
Indications for In Utero Fetal Intervention��������������������������������������   27
Antenatal Diagnosis and Management of Lower Urinary Tract
Obstruction (LUTO)����������������������������������������������������������������   27
Fetal Therapy for Hydrocephalus������������������������������������������������   27
Fetal Therapy for Meningomyelocele������������������������������������������   28
Fetal Therapy for Congenital Cystic
Adenomatoid Malformation����������������������������������������������������   28
Fetal Management of Congenital Diaphragmatic Hernia������������   28
Antenatal Diagnosis and Management of Abdominal
Wall Defects����������������������������������������������������������������������������   28
Suggested Reading��������������������������������������������������������������������������   28
5 Vascular Anomalies������������������������������������������������������������������������   29
Classification������������������������������������������������������������������������������������   29
Contents xiii

Infantile Hemangioma (Strawberry Angioma)��������������������������������   29

Investigations ������������������������������������������������������������������������������   31
Treatment ������������������������������������������������������������������������������������   32
Vascular Malformation��������������������������������������������������������������������   32
Port-Wine (Capillary Malformation) ������������������������������������������   32
Treatment ������������������������������������������������������������������������������������   33
Venous Malformation����������������������������������������������������������������������   33
Clinical Features��������������������������������������������������������������������������   33
Diagnosis��������������������������������������������������������������������������������������   33
Treatment ������������������������������������������������������������������������������������   33
Lymphatic Malformation (Cystic Hygroma, Lymphangioma)��������   34
Treatment ������������������������������������������������������������������������������������   34
Arteriovenous Malformation (AVM)/Arteriovenous Fistula ����������   34
Diagnosis��������������������������������������������������������������������������������������   34
Treatment ������������������������������������������������������������������������������������   34
Combined Vascular Malformation ����������������������������������������������   34
Summary of Treatment��������������������������������������������������������������������   35
Suggested Reading��������������������������������������������������������������������������   36
6 Lymphangiomas ����������������������������������������������������������������������������   37
Lymphatic Malformation ����������������������������������������������������������������   37
Embryology����������������������������������������������������������������������������������   37
Pathology ������������������������������������������������������������������������������������   37
Sites��������������������������������������������������������������������������������������������������   37
Clinical Features��������������������������������������������������������������������������   37
Investigations ����������������������������������������������������������������������������������   38
Complications������������������������������������������������������������������������������   38
Treatment ������������������������������������������������������������������������������������   38
Suggested Reading��������������������������������������������������������������������������   40

Part 2 Trauma

7 Pediatric Head Injury��������������������������������������������������������������������   43

Assessment of Head Injury by Glasgow Coma Scale (GCS)����������   43
Eyes����������������������������������������������������������������������������������������������   43
Motor��������������������������������������������������������������������������������������������   43
Verbal ������������������������������������������������������������������������������������������   44
Grading of Head Injury��������������������������������������������������������������������   44
Indications for Immediate Computed Tomography (CT) Scan�������   44
Management of Children with Mild Head Injury����������������������������   44
Management of Moderate and Severe Head Injury ��������������������   44
The Warning Signs to Watch by the Parents After Discharge������   46
Suggested Reading��������������������������������������������������������������������������   46
8 Abdominal Trauma������������������������������������������������������������������������   47
Liver Trauma������������������������������������������������������������������������������������   47
Ultrasonography��������������������������������������������������������������������������   47
Computed Tomography Scan������������������������������������������������������   47
Angiogram and Angio-Embolization������������������������������������������   48
xiv Contents

Diagnostic Peritoneal Lavage (DPL) ������������������������������������������   48

Management��������������������������������������������������������������������������������   49
Operative Management����������������������������������������������������������������   49
Role of Interventional Radiology in Liver Injury������������������������   49
Splenic Trauma��������������������������������������������������������������������������������   49
Management��������������������������������������������������������������������������������   49
Indications for Surgical Intervention ������������������������������������������   50
Renal Trauma ����������������������������������������������������������������������������������   50
Investigations ������������������������������������������������������������������������������   50
Management��������������������������������������������������������������������������������   51
Complications of Renal Trauma��������������������������������������������������   51
Pancreatic Trauma����������������������������������������������������������������������������   51
Intestinal Injuries ����������������������������������������������������������������������������   52
Suggested Reading��������������������������������������������������������������������������   52

Part 3 Head, Neck and Spine

9 Hydrocephalus��������������������������������������������������������������������������������   55
Etiology��������������������������������������������������������������������������������������������   55
Types������������������������������������������������������������������������������������������������   55
Congenital������������������������������������������������������������������������������������   55
Acquired��������������������������������������������������������������������������������������   55
Noncommunicating (Obstructive)������������������������������������������������   56
Communicating (Nonobstructive)������������������������������������������������   56
Clinical Features��������������������������������������������������������������������������   56
Investigations ������������������������������������������������������������������������������   57
Medical Treatment ����������������������������������������������������������������������   57
Surgical Treatment ����������������������������������������������������������������������   57
Shunt Operations��������������������������������������������������������������������������   57
Shunt Malfunctions����������������������������������������������������������������������   59
Other Causes of Large Head��������������������������������������������������������   59
Other Types of Hydrocephalus����������������������������������������������������   59
Suggested Reading��������������������������������������������������������������������������   59
10 Neural Tube Defects����������������������������������������������������������������������   61
Types������������������������������������������������������������������������������������������������   61
Embryology��������������������������������������������������������������������������������������   61
Diagnosis������������������������������������������������������������������������������������������   61
Antenatal��������������������������������������������������������������������������������������   61
Postnatal��������������������������������������������������������������������������������������   62
Treatment ����������������������������������������������������������������������������������������   63
Prevention������������������������������������������������������������������������������������   63
Antenatal Diagnosis and Management����������������������������������������   63
Postoperative Complications��������������������������������������������������������   65
Tethered Cord����������������������������������������������������������������������������������   65
Treatment ����������������������������������������������������������������������������������������   65
Suggested Reading��������������������������������������������������������������������������   65
Contents xv

11 Cleft Lip and Cleft Palate��������������������������������������������������������������   67

Incidence������������������������������������������������������������������������������������������   67
Relative Incidence������������������������������������������������������������������������   67
Etiology��������������������������������������������������������������������������������������������   67
Embryology��������������������������������������������������������������������������������������   68
Classification������������������������������������������������������������������������������������   68
Antenatal Diagnosis ������������������������������������������������������������������������   69
Management������������������������������������������������������������������������������������   69
Feeding����������������������������������������������������������������������������������������   69
Management of Respiratory Problems����������������������������������������   69
Principles of Surgery������������������������������������������������������������������������   70
Anatomy������������������������������������������������������������������������������������������   70
Timing of Surgery����������������������������������������������������������������������������   70
Commonly Practiced Procedures����������������������������������������������������   70
Principles of Surgery������������������������������������������������������������������������   70
Secondary Management������������������������������������������������������������������   70
Suggested Reading��������������������������������������������������������������������������   71
12 Neck Cysts, Sinuses, Lymphadenopathy,
and Torticollis ��������������������������������������������������������������������������������   73
Branchial Anomalies������������������������������������������������������������������������   73
Embryology����������������������������������������������������������������������������������   73
Branchial Cyst������������������������������������������������������������������������������   73
Pathology ������������������������������������������������������������������������������������   73
Clinical Features��������������������������������������������������������������������������   73
Branchial Fistula������������������������������������������������������������������������������   73
Etiology����������������������������������������������������������������������������������������   74
Clinical Features��������������������������������������������������������������������������   74
Treatment ������������������������������������������������������������������������������������   74
Thyroglossal Cyst and Fistula����������������������������������������������������������   75
Embryology����������������������������������������������������������������������������������   75
Clinical Features��������������������������������������������������������������������������   75
Differential Diagnosis������������������������������������������������������������������   76
Treatment ������������������������������������������������������������������������������������   76
Cystic Hygroma (Lymphatic Malformation) ����������������������������������   76
Cervical Lymphadenopathy ��������������������������������������������������������   76
Congenital Torticollis������������������������������������������������������������������   77
Suggested Reading��������������������������������������������������������������������������   79

Part 4 Thorax

13 Chest Wall Deformity��������������������������������������������������������������������   83

Pectus Excavatum����������������������������������������������������������������������������   83
Pectus Carinatum ����������������������������������������������������������������������������   84
Poland Syndrome ����������������������������������������������������������������������������   85
Sternal Defects ��������������������������������������������������������������������������������   85
Diffuse Skeletal Disorders with Thoracic Involvement ������������������   85
Suggested Reading��������������������������������������������������������������������������   85
xvi Contents

14 Mediastinal Masses in Children ��������������������������������������������������   87

Cysts and Tumors of the Mediastinum in Children ������������������������   87
Clinical Presentation������������������������������������������������������������������������   87
Evaluation/Imaging��������������������������������������������������������������������������   88
Management������������������������������������������������������������������������������������   91
Suggested Reading��������������������������������������������������������������������������   91
15 Empyema Thoracis������������������������������������������������������������������������   93
Pathology ����������������������������������������������������������������������������������������   93
Microbiology������������������������������������������������������������������������������������   93
Clinical Features������������������������������������������������������������������������������   94
Investigations ����������������������������������������������������������������������������������   94
Radiological Investigations��������������������������������������������������������������   94
Management������������������������������������������������������������������������������������   95
Medical Therapy������������������������������������������������������������������������������   95
Drainage������������������������������������������������������������������������������������������   96
Guidelines for Managing the Intercostal Tube��������������������������������   96
Surgical Management����������������������������������������������������������������������   96
Indications������������������������������������������������������������������������������������   96
Debridement��������������������������������������������������������������������������������   97
VATS for Empyema ��������������������������������������������������������������������   97
Complications of Empyema��������������������������������������������������������   97
Suggested Reading��������������������������������������������������������������������������   98
16 Congenital Diaphragmatic Hernia and Eventration������������������   99
Site of Defect ����������������������������������������������������������������������������������   99
Associated Anomalies����������������������������������������������������������������������   99
Embryology of Congenital Diaphragmatic Hernia (CDH)��������������   99
Types of Diaphragmatic Hernia ������������������������������������������������������ 100
Pathology ���������������������������������������������������������������������������������������� 100
Pathophysiology������������������������������������������������������������������������������ 100
Pathogenesis of Respiratory Distress in CDH ���������������������������� 100
Diagnosis������������������������������������������������������������������������������������������ 100
Antenatal Management�������������������������������������������������������������������� 101
Postnatal Management �������������������������������������������������������������������� 101
X-Ray Chest�������������������������������������������������������������������������������� 101
Eventration of the Diaphragm���������������������������������������������������������� 101
Management of CDH���������������������������������������������������������������������� 102
Operation������������������������������������������������������������������������������������������ 103
Prognostic Factors (Fetal and Postnatal)������������������������������������������ 103
Suggested Reading�������������������������������������������������������������������������� 103
17 Congenital Lung Lesions�������������������������������������������������������������� 105
Types������������������������������������������������������������������������������������������������ 105
Embryology�������������������������������������������������������������������������������������� 105
Antenatal Diagnosis and Treatment ������������������������������������������������ 105
Congenital Cystic Adenomatoid Malformation �������������������������� 105
Congenital Lobar Emphysema���������������������������������������������������� 108
Contents xvii

Pulmonary Sequestration ���������������������������������������������������������������� 109

Definition and Types�������������������������������������������������������������������� 109
Bronchopulmonary Foregut Malformations (Bronchogenic
Cysts, Esophageal Duplication Cysts, Neuroenteric Cysts)�������� 110
Suggested Reading�������������������������������������������������������������������������� 111
18 Esophageal Atresia������������������������������������������������������������������������ 113
Embryology�������������������������������������������������������������������������������������� 113
Prenatal Ultrasonographic Diagnosis (Sensitivity 42%)�������������� 113
Types of Esophageal Atresia������������������������������������������������������������ 113
Clinical Presentation�������������������������������������������������������������������� 113
Diagnosis�������������������������������������������������������������������������������������� 113
Management������������������������������������������������������������������������������������ 115
Preoperative Treatment���������������������������������������������������������������� 115
Operation������������������������������������������������������������������������������������������ 116
Steps of Surgery �������������������������������������������������������������������������� 116
Prognostic Factors for Esophageal Atresia�������������������������������������� 116
Postoperative Complications�������������������������������������������������������� 116
TEF Without Atresia (H-Type TEF)������������������������������������������������ 116
Suggested Reading�������������������������������������������������������������������������� 118
19 Esophageal Replacement�������������������������������������������������������������� 119
Indications for Esophageal Substitution in Children ���������������������� 119
Routes of Substitution���������������������������������������������������������������������� 120
Different Replacement Procedures�������������������������������������������������� 120
Gastric Transposition ������������������������������������������������������������������ 120
Gastric Tube Interposition������������������������������������������������������������ 122
Complications of Gastric Tube���������������������������������������������������� 122
Colon Interposition���������������������������������������������������������������������� 122
Suggested Reading�������������������������������������������������������������������������� 124

Part 5 Abdomen

20 Acute Abdomen in Children �������������������������������������������������������� 127

Relevant Clinical History in the Evaluation of a Child with Acute
Abdomen������������������������������������������������������������������������������������������ 127
Clinical Examination ���������������������������������������������������������������������� 127
Characteristics of Pain �������������������������������������������������������������������� 127
Red Flag Signs for Acute Abdominal Pain in Children ������������������ 128
Etiology: Points to Remember �������������������������������������������������������� 128
Investigations for Evaluation of Acute Abdomen in Children �������� 128
Summary of Common Causes of Acute Abdomen
Seen in Practice�������������������������������������������������������������������������������� 129
Indications for Surgical Consultation in Cases of Acute Abdomen 130
Management������������������������������������������������������������������������������������ 130
Suggested Reading�������������������������������������������������������������������������� 131
xviii Contents

21 Umbilical Anomalies���������������������������������������������������������������������� 133

Embryology of Formation of the Umbilicus������������������������������������ 133
List of Anomalies���������������������������������������������������������������������������� 133
Common Presentations�������������������������������������������������������������������� 133
Umbilical Hernia�������������������������������������������������������������������������� 133
Patent Vitellointestinal Duct (PVID) ������������������������������������������ 134
Umbilical Mass���������������������������������������������������������������������������� 135
Management�������������������������������������������������������������������������������� 135
Infected Umbilical Stump������������������������������������������������������������ 135
Patent Urachus or Urachal Cyst Remnants���������������������������������� 136
Suggested Reading�������������������������������������������������������������������������� 136
22 Abdominal Wall Defects���������������������������������������������������������������� 137
Types������������������������������������������������������������������������������������������������ 137
Definition ���������������������������������������������������������������������������������������� 137
Incidence������������������������������������������������������������������������������������������ 137
Embryology�������������������������������������������������������������������������������������� 137
Embryogenesis of the Defects ���������������������������������������������������� 137
Antenatal Management of Abdominal Wall Defects ������������������ 138
Associated Abnormalities���������������������������������������������������������������� 138
Post-delivery Management of Omphalocele and Gastroschisis������ 139
Initial Management�������������������������������������������������������������������������� 139
Surgical Repair�������������������������������������������������������������������������������� 139
General Principles of Abdomen Closure������������������������������������������ 141
Omphalocele Care During Surgical Closure������������������������������������ 142
Complications������������������������������������������������������������������������������ 142
Management of Intestinal Atresias with Gastroschisis
(Incidence 10%) �������������������������������������������������������������������������� 142
Management of Postoperative Dysmotility of Bowel������������������ 142
Beckwith-Wiedemann Syndrome���������������������������������������������������� 143
Pentalogy of Cantrell ���������������������������������������������������������������������� 143
Amniotic Band Syndrome���������������������������������������������������������������� 143
Bladder and Cloacal Exstrophy ������������������������������������������������������ 143
Prognosis and Outcome ������������������������������������������������������������������ 144
Suggested Reading�������������������������������������������������������������������������� 145
23 Abdominal Tuberculosis in Children ������������������������������������������ 147
Types of Abdominal Tuberculosis in Children�������������������������������� 147
Clinical Features������������������������������������������������������������������������������ 147
Diagnosis������������������������������������������������������������������������������������������ 147
Investigations ���������������������������������������������������������������������������������� 148
Radiological Investigations�������������������������������������������������������������� 149
Histopathological Diagnosis�������������������������������������������������������� 149
Complications���������������������������������������������������������������������������������� 149
Treatment ���������������������������������������������������������������������������������������� 150
Indications for Surgery�������������������������������������������������������������������� 150
Surgical Procedures������������������������������������������������������������������������� 150
Suggested Reading�������������������������������������������������������������������������� 150
Contents xix

24 Splenic and Liver abscess�������������������������������������������������������������� 151

Etiology of Splenic Abscess������������������������������������������������������������ 151
Imaging Studies ������������������������������������������������������������������������������ 151
Management of Splenic Abscess ���������������������������������������������������� 151
Liver Abscess ���������������������������������������������������������������������������������� 152
Incidence�������������������������������������������������������������������������������������� 152
Etiopathogenesis�������������������������������������������������������������������������� 152
Causative Organisms������������������������������������������������������������������������ 153
Clinical Presentation������������������������������������������������������������������������ 153
Investigations ���������������������������������������������������������������������������������� 153
Treatment of Liver Abscess������������������������������������������������������������� 154
Medical Treatment ���������������������������������������������������������������������� 154
Surgical Treatment ���������������������������������������������������������������������� 155
Open Surgical Drainage ������������������������������������������������������������������ 155
Complications and Prognosis���������������������������������������������������������� 156
Suggested Reading�������������������������������������������������������������������������� 156
25 Lesions of the Pancreas and Spleen���������������������������������������������� 157
Diseases of the Pancreas in Children ���������������������������������������������� 157
Acute Pancreatitis���������������������������������������������������������������������������� 157
Causes of Acute Pancreatitis in Children���������������������������������������� 157
Diagnosis������������������������������������������������������������������������������������������ 157
Treatment ���������������������������������������������������������������������������������������� 158
Chronic Pancreatitis ������������������������������������������������������������������������ 158
Diagnosis������������������������������������������������������������������������������������������ 158
Pancreas Divisum���������������������������������������������������������������������������� 159
Pseudocyst of the Pancreas�������������������������������������������������������������� 159
Persistent Hyperinsulinemic Hypoglycemia of Infancy (PHHI)������ 159
Diagnosis of PHHI�������������������������������������������������������������������������� 160
Pathology ������������������������������������������������������������������������������������ 160
Investigations ������������������������������������������������������������������������������ 160
Splenic Disorders in Children���������������������������������������������������������� 160
Splenic Cyst �������������������������������������������������������������������������������� 160
Splenectomy������������������������������������������������������������������������������������ 161
Indications for Splenectomy�������������������������������������������������������� 161
Hematological Changes Following Splenectomy������������������������ 161
Suggested Reading�������������������������������������������������������������������������� 161
26 Infantile Obstructive Cholangiopathy ���������������������������������������� 163
Extra Hepatic Biliary Atresia ���������������������������������������������������������� 163
Etiology�������������������������������������������������������������������������������������������� 163
Classification������������������������������������������������������������������������������������ 165
Diagnosis of Biliary Atresia������������������������������������������������������������ 165
Clinical Features�������������������������������������������������������������������������� 165
Investigations ���������������������������������������������������������������������������������� 165
Management������������������������������������������������������������������������������������ 167
Results and Prognosis Following Kasai Portoenterostomy ������������ 167
Liver Transplantation in Biliary Atresia������������������������������������������ 169
Other Rare Causes of Surgical Jaundice in Infants�������������������������� 169
Suggested Reading�������������������������������������������������������������������������� 169
xx Contents

27 Portal Hypertension in Children�������������������������������������������������� 171

Etiology and Types�������������������������������������������������������������������������� 171
Presentation�������������������������������������������������������������������������������������� 171
Diagnosis������������������������������������������������������������������������������������������ 172
Management������������������������������������������������������������������������������������ 172
Management of Variceal Bleeding ���������������������������������������������� 172
Primary Prophylactic Treatment (Treatment Before Bleed)�������� 173
Surgical Management���������������������������������������������������������������������� 173
Indications of Surgical Intervention�������������������������������������������� 173
Complications of Portal Hypertension�������������������������������������������� 175
Suggested Reading�������������������������������������������������������������������������� 175
28 Choledochal Cyst �������������������������������������������������������������������������� 177
Etiology�������������������������������������������������������������������������������������������� 177
Classification of Choledochal Cyst�������������������������������������������������� 177
Pathology ���������������������������������������������������������������������������������������� 177
Presentation�������������������������������������������������������������������������������������� 177
Diagnosis������������������������������������������������������������������������������������������ 178
Management������������������������������������������������������������������������������������ 179
Suggested Reading�������������������������������������������������������������������������� 180
29 Lesions of the Stomach������������������������������������������������������������������ 181
Peptic Ulcer�������������������������������������������������������������������������������������� 181
Pathology ������������������������������������������������������������������������������������ 181
Clinical Presentation�������������������������������������������������������������������� 181
Diagnosis�������������������������������������������������������������������������������������� 182
Treatment ������������������������������������������������������������������������������������ 182
Foreign Bodies and Bezoars������������������������������������������������������������ 182
Gastric Volvulus ������������������������������������������������������������������������������ 183
Pyloric Atresia���������������������������������������������������������������������������������� 184
Gastric Perforations and Necrosis���������������������������������������������������� 184
Clinical Presentation�������������������������������������������������������������������� 184
Gastric Duplication and Gastric Teratoma���������������������������������� 184
Suggested Reading�������������������������������������������������������������������������� 184
30 Gastroesophageal Reflux, Achalasia Cardia, Congenital
Esophageal Stenosis ���������������������������������������������������������������������� 185
Factors Preventing GER������������������������������������������������������������������ 185
Anatomic Factors ������������������������������������������������������������������������ 185
Physiological Factors ������������������������������������������������������������������ 185
Factors Predisposing to GER ���������������������������������������������������������� 185
Symptoms of GER �������������������������������������������������������������������������� 186
Complications of GER�������������������������������������������������������������������� 186
Differential Diagnosis���������������������������������������������������������������������� 186
Investigations ���������������������������������������������������������������������������������� 186
Treatment ���������������������������������������������������������������������������������������� 187
Medical���������������������������������������������������������������������������������������� 187
Surgical Treatment �������������������������������������������������������������������������� 187
Fundoplication �������������������������������������������������������������������������������� 188
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Achalasia Cardia������������������������������������������������������������������������������ 189

Congenital Esophageal Stenosis (CES) ������������������������������������������ 190
Suggested Reading�������������������������������������������������������������������������� 190
31 Pyloric Stenosis������������������������������������������������������������������������������ 191
Pathology ���������������������������������������������������������������������������������������� 191
Clinical Features������������������������������������������������������������������������������ 191
Differential Diagnosis������������������������������������������������������������������ 191
Investigations ������������������������������������������������������������������������������ 191
Biochemical Alterations�������������������������������������������������������������� 192
Management�������������������������������������������������������������������������������� 192
Surgery ���������������������������������������������������������������������������������������� 192
Suggested Reading�������������������������������������������������������������������������� 193
32 Neonatal Intestinal Obstruction �������������������������������������������������� 195
Intestinal Atresia and Stenosis �������������������������������������������������������� 195
Pyloric Atresia������������������������������������������������������������������������������ 195
Duodenal Atresia and Stenosis���������������������������������������������������� 195
Diagnosis�������������������������������������������������������������������������������������� 196
Treatment ������������������������������������������������������������������������������������ 197
Surgery ���������������������������������������������������������������������������������������� 197
Jejunoileal Atresia and Stenosis�������������������������������������������������� 197
Clinical Presentation�������������������������������������������������������������������� 197
Treatment ������������������������������������������������������������������������������������ 198
Surgery ���������������������������������������������������������������������������������������� 198
Malrotation with Volvulus������������������������������������������������������������ 199
Diagnosis and Management�������������������������������������������������������� 200
Meconium Ileus �������������������������������������������������������������������������� 200
Meconium Plug Syndrome���������������������������������������������������������� 201
Meconium Peritonitis������������������������������������������������������������������ 203
Suggested Reading�������������������������������������������������������������������������� 203
33 Necrotizing Enterocolitis �������������������������������������������������������������� 205
Etiology�������������������������������������������������������������������������������������������� 205
Presentation�������������������������������������������������������������������������������������� 205
Classification������������������������������������������������������������������������������������ 206
Treatment ���������������������������������������������������������������������������������������� 207
Indications for Surgery�������������������������������������������������������������������� 207
Solitary Intestinal Perforation (SIP)������������������������������������������������ 207
Complications���������������������������������������������������������������������������������� 208
Prevention of NEC �������������������������������������������������������������������������� 208
Suggested Reading�������������������������������������������������������������������������� 208
34 Meckel Diverticulum and Duplications of the Intestine ������������ 209
Embryology�������������������������������������������������������������������������������������� 209
Pathology ���������������������������������������������������������������������������������������� 209
Presentation�������������������������������������������������������������������������������������� 209
Diagnosis������������������������������������������������������������������������������������������ 210
Treatment ���������������������������������������������������������������������������������������� 210
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Duplications of Intestine������������������������������������������������������������������ 211

Etiology���������������������������������������������������������������������������������������� 211
Investigations ������������������������������������������������������������������������������ 211
Treatment ������������������������������������������������������������������������������������ 212
Mesenteric Cyst ������������������������������������������������������������������������������ 212
Classification�������������������������������������������������������������������������������� 212
Suggested Reading�������������������������������������������������������������������������� 212
35 Intussusception������������������������������������������������������������������������������ 213
Etiology�������������������������������������������������������������������������������������������� 213
Pathology ���������������������������������������������������������������������������������������� 213
Types with Frequency of Occurence������������������������������������������������ 214
Clinical Features������������������������������������������������������������������������������ 214
Diagnosis������������������������������������������������������������������������������������������ 214
Differential Diagnosis���������������������������������������������������������������������� 214
Treatment ���������������������������������������������������������������������������������������� 214
Suggested Reading�������������������������������������������������������������������������� 216
36 Anorectal Malformations�������������������������������������������������������������� 217
Embryology�������������������������������������������������������������������������������������� 217
Incidence������������������������������������������������������������������������������������������ 217
Associated Abnormalities���������������������������������������������������������������� 217
Types������������������������������������������������������������������������������������������������ 217
Usual Characteristics of High/Intermediate Anorectal
Malformations (HARM)������������������������������������������������������������������ 217
Usual Characteristics of Low Anorectal Malformations (LARM)�� 217
Classification (Krikenbech) ������������������������������������������������������������ 218
Clinical Presentation������������������������������������������������������������������������ 218
Investigations ���������������������������������������������������������������������������������� 222
Management������������������������������������������������������������������������������������ 223
Assessment of Fecal Continence ���������������������������������������������������� 227
Suggested Reading�������������������������������������������������������������������������� 227
37 Acquired Anorectal Disorders������������������������������������������������������ 229
Perianal Abscess and Fistula-in-Ano ���������������������������������������������� 229
Anal Fissure ������������������������������������������������������������������������������������ 230
Anal Tag and Hemorrhoids�������������������������������������������������������������� 230
Rectal Prolapse�������������������������������������������������������������������������������� 231
Management of Rectal Prolapse������������������������������������������������������ 232
Operative Treatment for Rectal Prolapse ���������������������������������������� 232
Suggested Reading�������������������������������������������������������������������������� 232
38 Hirschsprung Disease�������������������������������������������������������������������� 233
Incidence������������������������������������������������������������������������������������������ 233
Pathology ���������������������������������������������������������������������������������������� 233
Hallmark of Histological Diagnosis�������������������������������������������� 233
Cause�������������������������������������������������������������������������������������������� 233
Gross Pathology �������������������������������������������������������������������������� 233
Pathophysiology�������������������������������������������������������������������������� 235
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Clinical Features������������������������������������������������������������������������������ 235

Diagnosis�������������������������������������������������������������������������������������� 235
Differential Diagnosis������������������������������������������������������������������ 236
Management�������������������������������������������������������������������������������� 237
Surgical Treatment ���������������������������������������������������������������������� 237
Suggested Reading�������������������������������������������������������������������������� 239
39 Bleeding Per Rectum �������������������������������������������������������������������� 241
Approach to a Child with Rectal Bleeding�������������������������������������� 241
Polyps of the Colon and Rectum ���������������������������������������������������� 242
Suggested Reading�������������������������������������������������������������������������� 242
40 Constipation and Fecal Incontinence ������������������������������������������ 243
Constipation ������������������������������������������������������������������������������������ 243
Causes of Constipation�������������������������������������������������������������������� 243
Management of Constipation���������������������������������������������������������� 244
Causes of Fecal Incontinence in Children �������������������������������������� 244
True Fecal Incontinence Versus Fecal Pseudoincontinence
(Encopresis) ������������������������������������������������������������������������������������ 244
Enema therapy �������������������������������������������������������������������������������� 245
Suggested Reading�������������������������������������������������������������������������� 245
41 Stoma Care ������������������������������������������������������������������������������������ 247
Stomas in Pediatric Patients������������������������������������������������������������ 247
Stoma Related Complications���������������������������������������������������������� 247
Management of Stomas�������������������������������������������������������������������� 247
Gastrostomy Management ���������������������������������������������������������� 252
Suggested Reading�������������������������������������������������������������������������� 252

Part 6 Pediatric Oncology

42 Wilms’ Tumor �������������������������������������������������������������������������������� 255

Wilms’ Tumor (WT)������������������������������������������������������������������������ 255
Epidemiology���������������������������������������������������������������������������������� 255
Etiopathogenesis������������������������������������������������������������������������������ 255
Clinical Presentation������������������������������������������������������������������������ 256
Pathology ������������������������������������������������������������������������������������ 256
Investigations ������������������������������������������������������������������������������ 256
Staging �������������������������������������������������������������������������������������������� 257
Management�������������������������������������������������������������������������������� 258
Surgical Treatment ���������������������������������������������������������������������� 258
The Essential Steps of the Operation ������������������������������������������ 258
Tumor with Vena Caval/Atrial Thrombus������������������������������������ 258
Bilateral Wilms’ Tumor �������������������������������������������������������������� 258
Chemotherapy���������������������������������������������������������������������������������� 259
Pre-op chemotherapy (CT)���������������������������������������������������������� 259
COG Chemotherapy Protocol������������������������������������������������������ 259
Prognosis and Outcome �������������������������������������������������������������� 260
Congenital Mesoblastic Nephroma���������������������������������������������� 260
Suggested Reading�������������������������������������������������������������������������� 261
xxiv Contents

43 Neuroblastoma ������������������������������������������������������������������������������ 263

Pathology ���������������������������������������������������������������������������������������� 263
Prognostic Factors���������������������������������������������������������������������������� 263
Clinical Presentation������������������������������������������������������������������������ 264
Screening�������������������������������������������������������������������������������������� 265
Diagnosis�������������������������������������������������������������������������������������� 266
Staging �������������������������������������������������������������������������������������������� 266
Principles of Treatment�������������������������������������������������������������������� 266
Low-Risk Tumors������������������������������������������������������������������������ 267
Intermediate-Risk Tumors ���������������������������������������������������������� 267
High-Risk Tumors������������������������������������������������������������������������ 267
Suggested Reading�������������������������������������������������������������������������� 267
44 Hepatoblastoma and Other Liver Masses in Children�������������� 269
Hepatoblastoma�������������������������������������������������������������������������������� 269
Pathology ���������������������������������������������������������������������������������������� 269
Types������������������������������������������������������������������������������������������������ 269
Presentation�������������������������������������������������������������������������������������� 269
Diagnosis������������������������������������������������������������������������������������������ 270
Biochemical Marker�������������������������������������������������������������������� 270
Imaging Studies �������������������������������������������������������������������������� 270
Histopathology/Cytology������������������������������������������������������������ 270
Staging ���������������������������������������������������������������������������������������� 271
Management�������������������������������������������������������������������������������� 271
Infantile Hepatic Hemangioma�������������������������������������������������������� 271
Management�������������������������������������������������������������������������������� 272
Mesenchymal Hamartoma of Liver �������������������������������������������� 273
Focal Nodular Hyperplasia���������������������������������������������������������� 274
Congenital Giant Liver Cyst�������������������������������������������������������� 274
Suggested Reading�������������������������������������������������������������������������� 274
45 Germ Cell Tumors�������������������������������������������������������������������������� 275
Pathology ���������������������������������������������������������������������������������������� 275
Clinical Presentation������������������������������������������������������������������������ 276
Investigations ���������������������������������������������������������������������������������� 277
Staging �������������������������������������������������������������������������������������������� 277
Treatment ���������������������������������������������������������������������������������������� 278
Surgery �������������������������������������������������������������������������������������������� 278
Chemotherapy���������������������������������������������������������������������������������� 278
Fetus in Fetu������������������������������������������������������������������������������������ 279
Suggested Reading�������������������������������������������������������������������������� 279
46 Soft Tissue Sarcomas �������������������������������������������������������������������� 281
Rhabdomyosarcoma������������������������������������������������������������������������ 281
Genetics���������������������������������������������������������������������������������������� 281
Histological Variants of Childhood RMS������������������������������������ 281
Sites of Origin������������������������������������������������������������������������������ 282
Diagnosis�������������������������������������������������������������������������������������� 282
Contents xxv

Staging ���������������������������������������������������������������������������������������� 283

Treatment of RMS ���������������������������������������������������������������������� 283
Suggested Reading�������������������������������������������������������������������������� 285

Part 7 Pediatric Urology

47 Hydronephrosis������������������������������������������������������������������������������ 289
Causes of hydronephrosis���������������������������������������������������������������� 289
Pathology of Hydronephrosis���������������������������������������������������������� 289
Pelviureteric Junction Obstruction���������������������������������������������� 290
Pathology (see above)������������������������������������������������������������������ 290
Imaging�������������������������������������������������������������������������������������������� 291
Obstructive Uropathy in Children���������������������������������������������������� 293
Antenatally Diagnosed Hydronephrosis�������������������������������������� 293
Pyeloplasty Operation (Open or Laparoscopic or Robotic)�������� 293
Suggested Reading�������������������������������������������������������������������������� 295
48 Inguinal Hernia, Hydrocele, Acute Scrotum and Varicocele������ 297
Inguinal Hernia�������������������������������������������������������������������������������� 297
Clinical Features������������������������������������������������������������������������������ 297
Differential Diagnosis���������������������������������������������������������������������� 297
Treatment ���������������������������������������������������������������������������������������� 299
Surgical Procedure �������������������������������������������������������������������������� 299
Complications���������������������������������������������������������������������������������� 300
Hydrocele���������������������������������������������������������������������������������������� 300
Types of Hydrocele���������������������������������������������������������������������� 300
Diagnosis�������������������������������������������������������������������������������������� 300
Treatment of Hydrocele �������������������������������������������������������������� 300
Postoperative Complications�������������������������������������������������������� 300
Causes of Acute Scrotum in Children������������������������������������������ 300
Torsion of the Appendix Testis (Hydatid of Morgagni)�������������� 300
Torsion of the Testis �������������������������������������������������������������������� 301
Presenting Features���������������������������������������������������������������������� 301
Management�������������������������������������������������������������������������������� 302
Varicocele���������������������������������������������������������������������������������������� 302
Clinical Features�������������������������������������������������������������������������� 303
Suggested Reading�������������������������������������������������������������������������� 303
49 Undescended Testis������������������������������������������������������������������������ 305
Embryology�������������������������������������������������������������������������������������� 305
Factors Influencing Testicular Descent�������������������������������������������� 305
Incidence������������������������������������������������������������������������������������������ 305
Classification������������������������������������������������������������������������������������ 306
Terminologies���������������������������������������������������������������������������������� 306
Clinical Features������������������������������������������������������������������������������ 306
Differentiation of Low UDT Vs. Retractile Testis �������������������������� 307
Differentiation of Anorchia (Bilateral Absence of Testis)
Vs. Intra-­abdominal Testis (Cryptorchidism)���������������������������������� 307
Radiological Localization���������������������������������������������������������������� 308
xxvi Contents

Complications���������������������������������������������������������������������������������� 308
Infertility�������������������������������������������������������������������������������������� 308
Malignancy���������������������������������������������������������������������������������� 308
Others������������������������������������������������������������������������������������������ 308
Treatment ���������������������������������������������������������������������������������������� 308
Surgical Treatment �������������������������������������������������������������������������� 309
Suggested Reading�������������������������������������������������������������������������� 310
50 Hypospadias and Epispadias�������������������������������������������������������� 311
Hypospadias������������������������������������������������������������������������������������ 311
Development of the Penile Urethra���������������������������������������������� 311
Incidence�������������������������������������������������������������������������������������� 313
Etiology���������������������������������������������������������������������������������������� 313
Associated Anomalies������������������������������������������������������������������ 313
Ideal Time of Repair�������������������������������������������������������������������� 313
Anatomical Defect ���������������������������������������������������������������������� 313
Objectives of Repair�������������������������������������������������������������������� 313
Principles of Repair�������������������������������������������������������������������������� 313
Commonly Practiced Operations ���������������������������������������������������� 314
Single-Stage Urethroplasty���������������������������������������������������������� 314
Two-Stage Operations������������������������������������������������������������������ 314
Complications������������������������������������������������������������������������������ 315
Epispadias������������������������������������������������������������������������������������ 315
Types of Epispadias ������������������������������������������������������������������������ 315
Isolated���������������������������������������������������������������������������������������� 315
Treatment ������������������������������������������������������������������������������������ 316
Suggested Reading�������������������������������������������������������������������������� 316
51 Prepuce and Circumcision������������������������������������������������������������ 317
Physiology of Foreskin�������������������������������������������������������������������� 317
Management of Nonretractable Foreskin���������������������������������������� 317
Indications for Intervention�������������������������������������������������������������� 318
Indications for Circumcision������������������������������������������������������������ 318
Balanitis xerotica obliterans�������������������������������������������������������� 319
Points to Remember in the Management of Narrow Prepuce���������� 319
Smegma Collection�������������������������������������������������������������������������� 319
Paraphimosis������������������������������������������������������������������������������������ 319
Suggested Reading�������������������������������������������������������������������������� 320
52 Exstrophy of Bladder�������������������������������������������������������������������� 321
Embryology�������������������������������������������������������������������������������������� 321
Antenatal Diagnosis ������������������������������������������������������������������������ 321
Defect���������������������������������������������������������������������������������������������� 321
Exstrophy Variants �������������������������������������������������������������������������� 322
Cloacal Exstrophy���������������������������������������������������������������������������� 322
Management������������������������������������������������������������������������������������ 322
Staged Repair Is Widely Practiced���������������������������������������������� 322
Cloacal Exstrophy���������������������������������������������������������������������������� 323
Complications and Follow-Up for Exstrophy Bladder�������������������� 323
Suggested Reading�������������������������������������������������������������������������� 323
Contents xxvii

53 Urinary Tract Infection ���������������������������������������������������������������� 325

Clinical Features in Suspected UTI ������������������������������������������������ 325
Urine Sampling�������������������������������������������������������������������������������� 325
Symptoms of Lower Urinary Tract Infection���������������������������������� 326
Symptoms of Upper Urinary Tract Infection ���������������������������������� 326
Treatment ���������������������������������������������������������������������������������������� 326
Suggested Reading�������������������������������������������������������������������������� 326
54 Vesicoureteric Reflux (VUR)�������������������������������������������������������� 327
Incidence������������������������������������������������������������������������������������������ 327
Etiology�������������������������������������������������������������������������������������������� 327
Classification������������������������������������������������������������������������������������ 327
Primary and Secondary VUR������������������������������������������������������ 327
Reflux Nephropathy ������������������������������������������������������������������������ 328
Presentation of VUR������������������������������������������������������������������������ 328
Antenatal�������������������������������������������������������������������������������������� 328
Postnatal�������������������������������������������������������������������������������������� 328
Investigations ���������������������������������������������������������������������������������� 328
Grades of VUR�������������������������������������������������������������������������������� 329
Management of VUR ���������������������������������������������������������������������� 329
Conservative (Medical Management)������������������������������������������ 329
Follow-Up Management������������������������������������������������������������������ 330
Indications for Surgical Intervention ���������������������������������������������� 330
Functional������������������������������������������������������������������������������������ 330
Anatomical���������������������������������������������������������������������������������� 330
Surgical Management���������������������������������������������������������������������� 330
Suggested Reading�������������������������������������������������������������������������� 331
55 Neurogenic Bladder ���������������������������������������������������������������������� 333
Etiology of Childhood Neuropathic Bladder ���������������������������������� 333
Physiology of Bladder Function������������������������������������������������������ 333
Types of Neural Lesion with Neurogenic Bladder�������������������������� 333
Secondary Upper Renal Tract Complications������������������������������ 333
Assessment�������������������������������������������������������������������������������������� 334
Clinical Assessment in a Newborn�������������������������������������������������� 334
History������������������������������������������������������������������������������������������ 334
Examination �������������������������������������������������������������������������������� 335
Investigations ���������������������������������������������������������������������������������� 335
Management������������������������������������������������������������������������������������ 336
General Principles of Management�������������������������������������������������� 336
High Postvoid Residue���������������������������������������������������������������� 336
Detrusor Over Activity�������������������������������������������������������������������� 336
Poor Bladder Outlet Resistance ������������������������������������������������������ 337
Dyssynergia�������������������������������������������������������������������������������������� 337
Functional Voiding Disorders���������������������������������������������������������� 337
Clean Intermittent Catheterization (CIC) in Children �������������������� 337
Indications������������������������������������������������������������������������������������ 337
Technique������������������������������������������������������������������������������������ 337
Augmentation Cystoplasty�������������������������������������������������������������� 337
Suggested Reading�������������������������������������������������������������������������� 338
xxviii Contents

56 Posterior Urethral Valves�������������������������������������������������������������� 339

Embryology and Types�������������������������������������������������������������������� 339
Pathophysiology������������������������������������������������������������������������������ 339
Presentation�������������������������������������������������������������������������������������� 339
Fetal Diagnosis���������������������������������������������������������������������������� 339
Neonates�������������������������������������������������������������������������������������� 340
Infants and Older Children���������������������������������������������������������� 340
Investigations ���������������������������������������������������������������������������������� 341
Management������������������������������������������������������������������������������������ 341
Fetal Management������������������������������������������������������������������������ 341
Neonatal Treatment���������������������������������������������������������������������� 342
Assessment of Renal Function�������������������������������������������������������� 343
Assessment of Bladder Function ���������������������������������������������������� 344
Predictors of Poor Outcome������������������������������������������������������������ 344
Prenatal���������������������������������������������������������������������������������������� 344
Postnatal�������������������������������������������������������������������������������������� 344
Indicators of Good Renal Function�������������������������������������������������� 344
Follow-Up Management������������������������������������������������������������������ 345
Bladder Management in PUV Patients�������������������������������������������� 345
Prognosis and Outcome ������������������������������������������������������������������ 345
Suggested Reading�������������������������������������������������������������������������� 346
57 Urinary Incontinence in Children, Ectopic
Ureter and Ureterocele������������������������������������������������������������������ 347
Causes of Urinary Incontinence In Children ���������������������������������� 347
Diagnosis������������������������������������������������������������������������������������������ 347
Investigations ���������������������������������������������������������������������������������� 348
Ectopic Ureters and Ureteroceles���������������������������������������������������� 349
Ureterocele���������������������������������������������������������������������������������� 350
Embryology���������������������������������������������������������������������������������� 350
Investigations ������������������������������������������������������������������������������ 351
Ultrasonography�������������������������������������������������������������������������� 351
Micturating Cystourethrogram���������������������������������������������������� 351
Intravenous Urography���������������������������������������������������������������� 352
Renal Scintigraphy���������������������������������������������������������������������� 352
CT Urography and MRI Urography�������������������������������������������� 352
Treatment ���������������������������������������������������������������������������������������� 352
Surgical���������������������������������������������������������������������������������������� 352
Suggested Reading�������������������������������������������������������������������������� 354
58 Disorders of Sexual Differentiation���������������������������������������������� 355
Development of Gonads and Internal Genitalia������������������������������ 355
Common Types of DSDs ���������������������������������������������������������������� 355
46,XX DSD (Female Pseudohermaphroditism)������������������������������ 355
46,XY DSD (Male Pseudohermaphroditism)���������������������������������� 358
Androgen Insensitivity Syndrome (AIS), Male DSD
(Testicular Feminization Syndrome)�������������������������������������������� 358
Contents xxix

Disorders of Gonadal Dysgenesis���������������������������������������������������� 359

Mixed Gonadal Dysgenesis �������������������������������������������������������� 359
Ovotesticular DSD (True Hermaphrodite)�������������������������������������� 360
Persistent Mullerian Duct Syndrome ������������������������������������������ 360
Summary������������������������������������������������������������������������������������������ 360
Diagnosis�������������������������������������������������������������������������������������� 360
Common Investigations Ordered in the Diagnosis of DSD ������������ 361
Summary of Treatment of DSD ������������������������������������������������������ 361
Components of Female Genitoplasty
(Either or all of the Following)���������������������������������������������������� 361
Components of Male Genitoplasty
(Either or all of the Following) �������������������������������������������������� 361
Suggested Reading�������������������������������������������������������������������������� 361
59 Gynecological and Breast Disorders in Children������������������������ 363
Ovarian Cysts���������������������������������������������������������������������������������� 363
Management of Ovarian Cysts�������������������������������������������������������� 363
Simple Cyst���������������������������������������������������������������������������������� 363
Ovarian Cyst in Prepubertal Child ���������������������������������������������� 363
Ovarian Cysts in Adolescence����������������������������������������������������� 364
Ovarian Torsion���������������������������������������������������������������������������� 364
Labial Adhesions in Infants ������������������������������������������������������������ 364
Vaginal Conditions�������������������������������������������������������������������������� 365
Mayer-Rokitansky-Küster-Hauser Syndrome
(MRKH Syndrome) �������������������������������������������������������������������� 365
Breast Disorders������������������������������������������������������������������������������ 368
Mastitis���������������������������������������������������������������������������������������� 368
Breast Fibroadenoma ������������������������������������������������������������������ 369
Supernumerary (Accessory) Nipple�������������������������������������������� 370
Gynecomastia������������������������������������������������������������������������������ 370
Suggested Reading�������������������������������������������������������������������������� 370

Part 8 Miscellaneous

60 Recent Advances in Pediatric Surgery ���������������������������������������� 373

Progress in Neonatal Surgery���������������������������������������������������������� 373
Esophageal Atresia���������������������������������������������������������������������� 373
Hirschsprung Disease������������������������������������������������������������������ 373
Imperforate Anus ������������������������������������������������������������������������ 373
Congenital Diaphragmatic Hernia ���������������������������������������������� 374
Intestinal Atresias/Abdominal Wall Defects�������������������������������� 374
Recent Advances on Organ Transplantation������������������������������������ 374
Recent Advances in Pediatric Tumors �������������������������������������������� 374
Short Bowel Syndrome���������������������������������������������������������������� 374
Application of Newer Understandings�������������������������������������������� 375
Harvesting Stem Cells���������������������������������������������������������������������� 375
Embryonic Stem Cells ���������������������������������������������������������������� 375
xxx Contents

Tissue Engineering���������������������������������������������������������������������� 375

Molecular Genetics and Gene Therapy���������������������������������������� 375
Advances in Imaging ���������������������������������������������������������������������� 375
Ultrasonography�������������������������������������������������������������������������� 375
Multidetector Computed Tomography (MDCT)�������������������������� 375
Electron Beam Computed Tomography (EBCT)������������������������ 376
Magnetic Resonance Imaging������������������������������������������������������ 376
PET (Positron Emission Tomography) Scan������������������������������� 376
Minimal Access Surgery (MAS) in Pediatric Surgery���������������� 376
Energy Sources in Application for Surgery �������������������������������� 376
Robot in Pediatric Surgery: Da Vinci System���������������������������������� 376
Advantage of Robotic Surgery���������������������������������������������������� 376
Limitation������������������������������������������������������������������������������������ 376
Suggested Reading�������������������������������������������������������������������������� 377
61 Minimally Invasive Surgery in Children ������������������������������������ 379
Advantages of MISs������������������������������������������������������������������������ 379
Disadvantages of Pediatric MIS������������������������������������������������������ 379
Physiological Changes in MIS�������������������������������������������������������� 379
Cardiovascular Changes�������������������������������������������������������������� 379
Renal Effects�������������������������������������������������������������������������������� 380
Neurological Effects�������������������������������������������������������������������� 380
Other Effects�������������������������������������������������������������������������������� 380
Precautions in Pediatric MIS ���������������������������������������������������������� 380
Basic Concepts: VATS (Video-­Assisted Thoracoscopic Surgery) �� 380
Basic Instruments for MIS �������������������������������������������������������������� 380
Energy Source Devices�������������������������������������������������������������������� 380
Electrocoagulation ���������������������������������������������������������������������� 380
Harmonic Scalpel (Ultrasonic Cutting and Coagulation):
Advantages���������������������������������������������������������������������������������� 382
Ligasure (Pressure and Energy for Vessel Sealing) �������������������� 382
Indications for MIS in Pediatric Surgery ���������������������������������������� 382
Suggested Reading�������������������������������������������������������������������������� 382
62 Short Cases in Pediatric Surgery�������������������������������������������������� 383
Tongue-Tie or Ankyloglossia ���������������������������������������������������������� 383
Ranula���������������������������������������������������������������������������������������������� 384
BCG Lymph Adenitis���������������������������������������������������������������������� 384
Tubercular Lymphadenitis �������������������������������������������������������������� 384
Caput Succedaneum������������������������������������������������������������������������ 385
Presacral Cleft���������������������������������������������������������������������������������� 385
Syndactyly���������������������������������������������������������������������������������������� 386
Ludwig’s Angina������������������������������������������������������������������������������ 386
Amniotic Band �������������������������������������������������������������������������������� 386
Mucous Cyst������������������������������������������������������������������������������������ 386
Ganglion Cyst���������������������������������������������������������������������������������� 388
Pyogenic Granuloma������������������������������������������������������������������������ 389
Contents xxxi

Ingrown Toe Nail ���������������������������������������������������������������������������� 389

Dermoid Cyst ���������������������������������������������������������������������������������� 390
Wound Granuloma �������������������������������������������������������������������������� 390
Keloid���������������������������������������������������������������������������������������������� 390
Suggested Reading�������������������������������������������������������������������������� 391
63 Picture Plates (Miscellaneous)������������������������������������������������������ 393
About the Author

Dr. Subhasis Roy Choudhury, M.S., D.N.B., M.Ch., M.D. is currently

Director Professor and Head of the Department of Pediatric Surgery at the
Lady Hardinge Medical College and Affiliated Kalawati Saran Children’s
Hospital, New Delhi, India. After graduating from the NRS Medical College,
Kolkata, in 1984, he completed his postgraduate studies in surgery and pedi-
atric surgery (M.Ch.) at the PGIMER, Chandigarh, India. Subsequently, he
received further training in pediatric surgery at Birmingham Children’s
Hospital, United Kingdom, and at Children’s Mercy Hospital, Kansas City,
United States. He has over 90 publications to his credit and has presented
papers and CME lectures at numerous national and international conferences.
He is an active reviewer of several national and international journals. In
addition to patient care, he has a keen interest in teaching and research and is
a recognized postgraduate teacher for courses on pediatric surgery affiliated
with the University of Delhi, India.

xxxiii
 Part 1
General
 Fluid and Electrolyte Balance
in Children
The physiology of water and electrolyte balance
in new born and pediatric patients is distinctly
different from adults. Clinicians involved in the
management of children should be familiar with
the pathophysiology of water and electrolyte
alterations in order to successfully restore the
balance.
Water constitutes 70–80% of the body weight
in the newborn as compared to 60% in adults.
The total body water (TBW) is distributed into
one third extracellular (ECF) (further divided
into intravascular and interstitial fluid) and two
thirds intracellular (ICF) fluid compartments in
adults. In comparison, a neonate has excess of
extracellular fluid (45% of body mass) in contrast
to intracellular fluid (35% of body mass). Because
ECF is more readily lost from the body and
infants have a larger body surface area, they are
at higher risk of dehydration than adults.
Neonates are born with excess TBW (mainly
ECF); they lose 5–10% of their weight in the first
week of life. Preterm neonate may lose up to
15–20% of TBW in the first week of life. The
neonate is oliguric on the first day of life. The
neonate loses weight due to diuresis during the
next few days which is physiological. With
increasing age there is a gradual decrease in body
water and the extracellular fluid compartment
with a concomitant increase of the intracellular
© Springer Nature Singapore Pte Ltd. 2018
S. Roy Choudhury, Pediatric Surgery, https://doi.org/10.1007/978-981-10-6304-6_1
1
compartment. The body surface area of a new-
born is relatively much greater than the adult;
therefore, insensible fluid loss from the body is a
major factor especially in preterm neonates.
Transparent plastic films are used to cover the
body to prevent the same.
Insensible water loss (IWL) is through the
skin (two thirds) and respiratory tract (one
third). The amount of IWL depends on the ges-
tational age; the more the prematurity, the higher
the IWL.
The kidneys of newborns have low glomeru-
lar filtration rate and poor concentrating ability,
which makes them less tolerant to dehydration
and fluid overload. The kidney in the newborn
can only concentrate to about 400 mOsm/L ini-
tially (500–600 mOsm/L in the full term com-
pared to 1200 mOsm/L for an adult) and
therefore requires 2–4 cc/kg/h urine production
to clear the renal solute load. The older child
needs about 1–2 cc/kg/h and the adult 0.5–1 cc/
kg/h of urine output. Oliguria is defined as urine
output <1 mL/kg/h.
All measurable sources of fluid and electro-
lyte loss should be accounted when considering
the fluid therapy for surgical neonates. Sources
include stool (short gut syndrome, ostomy),
nasogastric losses, drain (chest, abdominal), burn
surface losses, and CSF drains.
3
4 1
 ccessing Fluid and Electrolyte
A plasma osmolality reflect internal homeostasis.
Status
History
Maternal administration of hypotonic fluids,
drugs like oxytocin, ACE inhibitors, indometha-
cin, and furosemide can affect the neonate’s renal
function adversely.
Clinical Evaluation
Careful weight monitoring is critical in all chil-
dren. Monitoring body weight, urine output, and
electrolytes helps to identify overhydration or
underhydration and electrolyte disturbances. The
rate or composition of the intravenous solution
can thus be adjusted accordingly.
An infant’s weight rises significantly, while the
intravascular volume may not have increased in a
number of conditions like long-term use of para-
lytic agents, sepsis, and peritonitis. It is mainly due
to increase in interstitial fluid compartment.
Skin and mucosa changes reflected by sunken
anterior fontanelle and eyes and dry mucous
membranes with altered skin turgor are clinical
signs of dehydration but are not very sensitive
indicators for the management of electrolyte
imbalance.
Cardiovascular Signs
Tachycardia, delayed capillary refill, and hepato-
megaly (sign of congestive cardiac failure) are
features of cardiovascular instability.
Laboratory Evaluation
Serum electrolytes, blood urea nitrogen, creati-
nine, urine electrolytes, specific gravity and
Fluid and Electrolyte Balance in Children
Blood gas analysis metabolic acidosis may be a
sign of inadequate tissue perfusion.
Fluid and Electrolyte Balance
For the first 12–24 h, sodium, potassium, and
chloride are not required. The ideal fluid for the
first 24–48 h is 10% dextrose 60–80 mL/kg/day;
it maintains the glucose infusion rate (GIR) at
6–7 mg/kg/min. In neonates after the first few
days, N/5 in D10 is administered, whereas in
older children, N/2 or N/3 in D5 is preferred
(N = normal saline, D = dextrose).
Electrolytes requirements of the full-term
neonate are:
Sodium 2–3 mEq/kg/day (small preterm has
increased requirements up to 6–8 mEq/kg/day)
Potassium 1–2 mEq/kg/day
Chloride 3–5 mEq/kg/day
Maintenance Fluid Requirement
Premature babies have impaired ability to excrete
sodium load. They are susceptible to both sodium
loss and sodium and volume overloading. High-­
volume intravenous therapy can lead to opening
up PDA, bronchopulmonary dysplasia, enterocoli-
tis, and intraventricular hemorrhage. Estimations
of daily fluid requirements should take into con-
sideration (1) urinary water losses, (2) gastrointes-
tinal losses, (3) insensible water losses, and (4)
surgical losses.
Initial fluid therapy for infants and neonates
(mL/kg/day)
Birth weight <24 h 24–48 h >48 h
<1 kg 100–150 120–150 140–190
1–1.5 80–100 100–120 120–160
>1.5 60–80 80–120 120–160
Accessing Fluid and Electrolyte Status
The maintenance fluid requirements for older
children (Holliday-Segar method) are
100 mL/kg for the first 10 kg, 1000 mL + 50 mL/
kg for 11–20 kg, and 1500 mL + 20 mL/kg for
each kg >20 kg.
The maximum total daily fluid is normally
2400 mL.
Calculation in hours will be 4 mL/kg/h for
0–10 kg, 40 mL/h + 2 mL/kg/h for 10–20 kg, and
60 mL/h + 1 mL/kg/h for each kg >20 kg ( rule of
4–2–1).
Replacement Fluid
Average composition of gastric fluid – sodium (Na),
60 mEq/L; potassium (K), 10 mEq/L; and chloride
(CL), 90 mEq/L. Nasogastric losses should be
replaced volume per volume with N/2 saline in
D5W with 10 mEq/L KCL every 1–6 h. As com-
pared to this, biliary losses have more sodium con-
tent (Na, 130 mEq/L; K, 5–15 mEq/L; and CL,
80–120 mEq/L), so they must be replaced volume
per volume with normal saline (NS) with 10 mEq/L
potassium chloride (KCL) or Ringer’s lactate (RL).
Deficit Therapy
Babies with dehydration require acute intervention
to urgently improve tissue perfusion. Boluses with
isotonic fluids like normal saline/Ringer’s lactate
(NS/RL) at 20 mL/kg are required to rapidly increase
the intravascular volume in resuscitation. The initial
resuscitation and rehydration are complete when the
baby has achieved adequate intravascular volume. In
patients with metabolic alkalosis (hypertrophic
pyloric stenosis), lactated Ringer’s should not be
used, as it would worsen the alkalosis.
Calculate the 24 h fluid requirement (mainte-
nance + deficit), subtract the amount of fluid given
in boluses, and give the rest over the next 24 h.
5
The first step in management of dehydration is
assesment of the degree of dehydration (mild,
moderate or severe) based on this defecit therapy
is planned.
Fliud deficit can be calculated exactly if
­preillness weight is known, however it can also
be assessed based on clinical observations.
Calculated assesment:
Its the most precise method of calculating fluid
deficit
fluid defecit (L) = preillness weight (kg) -
­current weight (kg)
Clinical assesment:
Based on clinical assesment, dehydration is
classified as mild, moderate or severe, accord-
ingly deficit fluid replacement is then
performed.
Infants:
mild dehydration (5%) 50 ml/kg
moderate dehydration (10%) 100 ml/kg
severe dehydration (15%) 150 ml/kg
Children:
mild dehydration (3%) 30 ml/kg
moderate dehydration (6%) 60 ml/kg
severe dehydration (9%) 90 ml/kg
Blood Volume
Premature: 85–100 cc/kg
Term newborn: 85 cc/kg
Infant: 70–80 cc/kg
More than 10% blood loss requires to be
replaced by blood.
6 1
Electrolyte Disturbances
Sodium is the principal cation of ECF and is
responsible for the determination of extracellular
osmolality.
Hyponatremia
Hyponatremia is defined by serum sodium level
<135 mEq/L.
Causes of hyponatremia
Pseudohyponatremia (hyperosmolality)
Hyperlipidemia, mannitol, sucrose
Extra renal losses
Short gut syndrome, stoma losses, vomiting and
nasogastric losses, diarrhea
Renal losses
Thiazide/loop diuretics, post-obstructive diuresis,
renal tubular acidosis, cerebral salt wasting, autosomal
recessive pol, autosomal recessive polycystic kidney
disease
Euvolemic hyponatremia
SIADH, hypothyroidism, water intoxication
(iatrogenic, faulty feeding, psychogenic polydipsia)
Hypervolemic hyponatremia
CHF, cirrhosis, nephrotic syndrome, renal failure,
sepsis, protein-losing enteropathy
Hyponatremia causes the movement of water
from ECF to ICF to maintain the osmotic equilib-
rium. This increase in intracellular water causes
the cells to swell, which is well tolerated at most
places except the brain where cerebral space lim-
itation leads to increased intracranial pressure.
Neurologic symptoms of hypernatremia are
anorexia, vomiting, lethargy, confusion, agita-
tion, headache, seizures, and coma.
Management
It is based on the pathophysiology of specific eti-
ology. Overly rapid correction of hyponatremia is
to be avoided due to the risk of central pontine
myelinolysis (CPM), and even in patients with
severe symptoms (seizures, coma), boluses of
Fluid and Electrolyte Balance in Children
hypertonic saline are given to produce a small
and rapid increase in serum sodium, and no
attempt should be made to correct the value to
normal levels. Each mL of 3% NaCl increases the
serum value by 1 meq. It is advisable to avoid
correcting the serum sodium concentration by
>12 mEq/L/24 h or >18 mEq/L/48 h. Sodium
deficit is calculated by the following formula:
Sodium deficit = (desired serum Na concen-
tration usually 140 − current Na concentra-
tion) × 0.6 × weight in kg
The amount of 3% NaCl required is twice the
amount of sodium deficit.
Hypernatremia
Hypernatremia is defined as sodium concentra-
tion >145 mEq/L.
Causes of hyponatremia
Excessive sodium
Sodium bicarbonate excess, hypertonic saline,
hyperaldosteronism
Water deficit
Nephrogenic diabetes insipidus, central diabetes
insipidus, phototherapy, radiant warmers, premature
infants
Water and sodium deficits
Gastrointestinal losses, burns, osmotic diuresis,
chronic kidney disease, post-obstructive diuresis
Most babies with hypernatremia are dehy-
drated, but they tend to maintain the intravascu-
lar volume due to shifting of fluid from ICF to
ECF.
Hypernatremic babies are irritable, restless,
weak, and lethargic with high-pitched cry. They
have dry mucous membranes, loss of skin turgid-
ity, fits, and coma. Brain hemorrhage due to tear-
ing of intracerebral veins and bridging vessels is
the most devastating complication.
Hypernatremia also should not be corrected
rapidly; the goal is to lower serum sodium by
<12 mEq/L every 24 h, at the rate of 0.5 mEq/L/h.
Water deficit is calculated by water defi-
cit = body weight × 0.6 × (current serum
Na/140 − 1).
Accessing Fluid and Electrolyte Status
Choice of intravenous fluid (IVF): In a hypo-
tensive patient, the choice of fluid is normal
saline. In hypernatremic dehydration, N/2 or N/4
saline should be given. Hypernatremia due to
sodium overload is managed by D5 water
(sodium-free fluid).
Potassium
Potassium is the chief intracellular cation
150 mEq/L; majority of potassium is contained
in muscles.
Hyperkalemia
Hyperkalemia is defined as serum potassium
more than 5.5 mEq/L.
Causes of hyperkalemia
Spurious
Hemolysis, tissue ischemia, thrombocytosis,
leukocytosis
Increased intake
Intravenous KCL/oral supplementation, blood
transfusions
Transcellular shifts
Acidosis, rhabdomyolysis, tissue necrosis, digitalis
intoxication, tumor lysis syndrome, succinylcholine
Decreased excretion
Renal insufficiency, CAH, obstructive uropathy,
Bartter syndrome, ACE inhibitors, K-sparing diuretics
Adverse effects of hyperkalemia are due to its
role in membrane polarization, and most of the
changes are seen in cardiac conduction system
(peaking T waves, ST segment depression,
increased PR interval, flattening of P wave, and
widening of QRS complex). Patients have paresthe-
sias, fasciculations, weakness, and even ascending
paralysis, but cardiac toxicity (ventricular fibrilla-
tion/asystole) usually precedes these symptoms.
Management: Stop all sources of additional
potassium. Management of hyperkalemia has two
goals: stabilize the heart and prevent arrhythmias
and remove excess potassium from the body.
Calcium stabilizes the cell membranes of the heart
7
preventing arrhythmias. The action is immediate.
Bicarbonate, a combination of insulin and glucose,
and beta receptor sympathomimetics cause the
potassium to move intracellularly and decrease
serum potassium. Nebulized beta receptor agonists
also lead to the shift of potassium to ICF and have
the advantage of not requiring intravenous access.
Sodium polystyrene sulfonate is given orally, and it
exchanges Na with K in the gut. Severe hyperkale-
mia will require dialysis to decrease K levels espe-
cially if renal insufficiency is also present.
Hypokalemia
Hypokalemia is defined as serum potassium
<3.5 mEq/L.
Causes of hypokalemia
Spurious
High WBC count
Transcellular shifts
Alkalosis, alpha adrenergic agonists, theophylline,
refeeding syndrome
Decreased intake
Anorexia nervosa
Extrarenal losses
Diarrhea, laxative abuse, excessive sweating
Renal losses
Renal tubular acidosis, post-obstructive diuresis,
emesis/nasogastric losses, Bartter syndrome
Heart and skeletal muscles are especially vul-
nerable to the effects of hypokalemia. ECG
changes include a flattened T wave, a depressed
ST segment, and the appearance of U waves.
Ventricular fibrillation and supraventricular
tachycardias can occur. Skeletal muscle cramps
and weakness are the manifestations of hypoka-
lemia. Hypokalemia also slows the gastrointesti-
nal motility and causes ileus.
Management: Assessment of renal function
and urine output is important before supplement-
ing potassium. Oral potassium is safer but in
urgent situations intravenous potassium may be
required. Oral dose is 2–4 mEq/kg/day with a
maximum of 120–240 mEq/day in divided doses.
8 1
The dose for intravenous replacement is
0.5–1 mEq/kg usually given over 1 h with the
maximum dose of 40 mEq. Addition of potas-
sium to IVF and administered at a concentration
of 20 mEq/L and not exceeding 40 mEq/L. In
patients with hypokalemia and metabolic alkalo-
sis with volume depletion (hypertrophic pyloric
stenosis), restoration of intravascular volume
with sodium chloride reduces urinary potassium
loss.
Hypocalcemia
Hypocalcemia is defined as total serum calcium
<7 mg/dL and ionized calcium <4 mg/dL.
Causes
Prematurity, infants of diabetic mothers, hypo-
parathyroidism, vitamin D deficiency, pancreati-
tis, malabsorption state, anticonvulsants, tumor
lysis syndrome, and hypomagnesemia
Hypocalcemia increases the cellular excitabil-
ity, and patients present with neuromuscular irri-
tability, weakness, paresthesias, fatigability,
laryngospasm, apnea, cardiac arrhythmias, and
seizures.
Trousseau’s sign and Chvostek’s sign are
present.
Management
Rapid intravenous infusion of calcium solutions
can cause bradycardia and cardiac arrest; calcium
infusion should be given slowly under cardiovas-
cular monitoring. For symptomatic hypocalce-
mia, infusion of 10% calcium gluconate
(100–200 mg/kg) is given over 10 min under car-
diovascular monitoring; infusion is stopped if
heart rate is <100 bpm. If there is no clinical
response, then the same dose can be repeated
over 10 min, and maintenance calcium is added
to intravenous fluids.
(10% IV sol = 1 g Ca gluconate/10 mL = 90 mg
(4.5 mEq) elemental calcium; doses expressed as
Ca gluconate salt, except where otherwise
specified)
Fluid and Electrolyte Balance in Children
Hypoglycemia
Neonatal hypoglycemia is defined as blood glu-
cose level of <30 mg/dL in the first 24 h of life,
and <45 mg/dL afterward is a common metabolic
disorder. The exact value that should be consid-
ered abnormal is debatable, but an operational
value of blood glucose <40 mg/dL is used to
guide treatment. Symptoms may include hypoto-
nia, lethargy, apathy, poor feeding, jitteriness,
seizures, cyanosis, apnea, and hypothermia.
Asymptomatic hypoglycemia in a neonate with
blood glucose >25 mg/dL can be given a trial of
oral feeds. Symptomatic babies are treated with
an intravenous bolus of dextrose 10%, at 2.0 mL/
kg. After the bolus is administered, an IV infu-
sion with glucose infusion rate of about 5–8 mg/
kg/min in an infant and about 3–5 mg/kg/min in
an older child should be continued to maintain
normal blood glucose. The need of glucose infu-
sion rate of >12 mg/kg/min for more than 24 h or
persistence of hypoglycemia for more than 5–7
days needs further investigations.
Persistent hyperinsulinemic hypoglycemia
of infancy (PHHI) is relatively rare but one of
the most important causes of severe neonatal
hypoglycemia. Recognition of this entity
becomes important because severe and fre-
quent hypoglycemia may lead to severe perma-
nent neurological damage or even a
life-threatening event if not recognized and
treated effectively in time. Medical treatment
consists of a high level of glucose infusion
(>10 mg/kg/min to maintain blood glucose
level more than 54 mg%) along with continu-
ous glucose feeding and diazoxide, octreotide,
and/or glucagon infusion. Surgical treatment
with near total pancreatectomy (95% or more)
is required in cases with failed medical treat-
ment except in cases of focal lesions. See
Chap. 25 on lesions of the pancreas and spleen.
Hypothermia
The body loses heat to the environment by evapo-
ration, conduction, convection, and radiation.
Surgical newborns are especially prone to intra-
Accessing Fluid and Electrolyte Status
operative hypothermia. They generate heat by
increasing metabolic activity and using brown fat
stored mainly in the neck, axillae, and interscap-
ular region. Severe hypothermia (temperature
below 35 °C) may manifest as lassitude, apnea,
bradycardia, hypoglycemia, and hyperkalemia
leading to metabolic acidosis and shock with
elevated BUN and oliguria (neonatal cold injury
syndrome). These problems are of considerable
importance in premature babies, following pro-
longed surgery and in cases of eviscerated bowel
(like gastroschisis). Practical considerations to
maintain intraoperative temperature control are
the use of humidified and warmed inhalant gases
during anesthesia, warm saline solution for peri-
toneal washes, intraoperative use of servo-­
controlled heating mattresses, and monitoring of
core body temperature. It is very important to
prewarm the operation theater and recovery areas
so as to prevent hypothermia. The ideal environ-
mental temperature should be maintained around
25 °C. Babies should be transported in transport
incubators with warm humidified air. During all
procedures and prolonged examination of new-
borns, the use of a radiant heater with servo-­
controlled mechanism is useful.
Neonatal Hyperbilirubinemia
Hyperbilirubinemia occurs in 85% of term infants
and virtually in all premature infants.
It occurs due to the imbalance between biliru-
bin production and elimination.
Newborn jaundice appears at bilirubin level
>7 mg/dL.
Physiologic Jaundice
Clinical jaundice appears at 2–3 days.
Total bilirubin rises by <5 mg/dL per day.
Peak bilirubin occurs at 3–5 days of age.
• Peak bilirubin concentration in full-term
infant <12 mg/dL
• Peak bilirubin concentration in premature
infant <15 mg/dL
9
Clinical jaundice is resolved by 2 weeks in the
term infant and by 3–4 weeks in the preterm
infant.
Pathological Jaundice
Clinical jaundice appears before 24 h of age.
Bilirubin rises by >0.2 mg/dL/h.
Peak concentration of total bilirubin is more
than 12 mg/dL in the term infant and 15 mg/dL in
the preterm infant.
• Clinical jaundice is not resolved in 8 days in
the term infant and in 14 days in the preterm
infant.
• Clinical jaundice appears again after it has
been resolved.
• Direct bilirubin concentration more than
1.5 mg/dL.
 auses of Neonatal
C
Hyperbilirubinemia
Overproduction: Excessive hemolysis like blood
group incompatibility
Undersecretion: Metabolic and endocrine dis-
orders and obstructive causes
Mixed: Sepsis and infections
Uncertain mechanism: Breast milk jaundice
Kernicterus is due to the deposition of biliru-
bin in the brain especially in the regions of the
hippocampus, thalamus, striatum, auditory, and
oculomotor nuclei.
The two main treatment modalities for neona-
tal hyperbilirubinemia are phototherapy and
exchange transfusion.
I ndications for Phototherapy
and Exchange Transfusion
For infants more than 35 weeks of gestation:
Depending upon the bilirubin level, day of life, ges-
tational age, and presence of risk factors, charts are
present which can help in deciding whether photo-
therapy or exchange transfusion is needed or not.
10
For infants <35 weeks of gestation: Phototherapy
is recommended if the total serum bilirubin is more
than 0.5% or 0.75% of body weight in grams in
healthy and sick neonates, respectively. Blood
exchange transfusion is indicated if bilirubin is
more than 1% of body weight in grams. An approx-
imate estimate will be as follows:
1. Infant weight <1000 g. Phototherapy within
24 h and exchange transfusion at bilirubin
level of 10–12 mg/dL.
2. Infant weight 1000–1500 g. Phototherapy at
bilirubin levels of 7–9 mg/dL and exchange
transfusion at level of 12–15 mg/dL.
3. Infant weight 1500–2000 g. Phototherapy at
bilirubin level of 10–12 mg/dL and exchange
transfusion at level of 15–18 mg/dL.
4. Infant weight 2000–2500 g. Phototherapy at
bilirubin level of 13–15 mg/dL and exchange
transfusion at level of 18–20 mg/dL.
1 Fluid and Electrolyte Balance in Children
Phototherapy works by three reactions that
can occur when bilirubin is exposed to light:
1. Photooxidation
2. Configurational isomerization
3. Structural isomerization
Conjugated hyperbilirubinemia: See Chap. 25
on obstructive cholangiopathy.
Suggested Reading
Kliegman R, Stanton B, Geme JS, Nelson SN. Textbook
of pediatrics. 20th ed. Philadelphia, PA: Saunders,
Elsevier; 2015.
Arcara K, Tschudy M. The harriet lane handbook. 20th
ed. Philadelphia, PA: Mosby Elsevier; 2014.
 Vascular Access in Children
Vascular access is an important and sometimes
difficult procedure in children. Health-care per-
sonnel dealing with hospital pediatric clinical
practice should be familiar with the different
techniques of vascular access and its mainte-
nance and management of any complications
should they arise.
Broadly they can be divided into peripheral and
central venous catheters. Peripheral vascular cath-
eters are used for a variety of indications, such as
the administration of IV fluids and various medica-
tions. For chemotherapy or total parenteral nutri-
tion (TPN), central venous catheters are desirable.
Types of Catheters
Peripheral Catheters
Peripheral venous catheters are the most com-
monly used. They are relatively safe and easy to
insert. Dislodgement and occlusion remain the
main concerns. Veins on the dorsum of the hand
and feet, scalp veins, and saphenous vein are the
preferred sites for insertion (Fig. 2.1a, b). Larger
veins such as the cephalic vein, basilic vein, and
even femoral vein can also be used; however,
they are kept reserved for putting in the peripher-
ally inserted central catheter. Scalp veins are
good for cannulation in neonates. External jugu-
lar vein can be used though they are difficult to
maintain due to short neck in children.
© Springer Nature Singapore Pte Ltd. 2018
S. Roy Choudhury, Pediatric Surgery, https://doi.org/10.1007/978-981-10-6304-6_2
2
Venous Cutdown
This method is seldom used nowadays due to bet-
ter options and techniques enabling easy and reli-
able vascular access. However, venous cutdown
was often used in the past to gain access in diffi-
cult cases and in emergency situations. The
saphenous vein because of its consistent anatomy
and ease of access remains the first choice. A lin-
ear incision just anterior and above the medial
malleolus with blunt dissection with an artery
forceps enables visualization of the saphenous
vein. Venotomy is performed and catheter
inserted and secured. Antecubital and femoral
vein can also be used for cutdown.
Intraosseous Catheters
The intraosseous route is used in life-threatening
situations in critically ill children in whom intra-
venous access cannot be established within 90 s.
A large bore cannula/needle (16–18G) is inserted
in the proximal tibia, 1–2 cm below and medial to
the tibial tuberosity, to gain rapid intraosseous
access in small children. It uses the noncollaps-
ible veins in the medullary sinuses of the bone
marrow draining into the central circulation
through the emissary vein to rapidly deliver
resuscitation drugs, fluid boluses, and even blood
products. Intraosseous catheter should not be
kept more than 12–24 h. Leakage, osteomyelitis,
11
12 2 Vascular Access in Children

a b

Fig. 2.1 (a, b) Peripheral venous catheter

fracture, and occlusion are the common PICC lines are used for delivery of total paren-
complications. teral nutrition, chemotherapy, and all medica-
tions including inotropes and can also be used for
blood sampling. Two types of PICC lines are
Peripherally Inserted Central available commercially – open PICC and
Catheters (PICC) Groshong PICC. Open PICC requires regular
flushing with heparinized saline, and Groshong
Peripherally inserted central catheters (PICC PICC has a two-way pressure-sensitive valve,
lines) have gained widespread acceptance and are which allows infusion of fluids and withdrawing
used in patients requiring intermediate to long-­ of blood sample but does not allow backflow of
term venous access. They can be inserted and
removed bedside without general anesthesia and
do not have the complications associated with
central venous catheters, which makes them very
popular especially in a surgical newborn patient.
The preferred sites for insertion of PICC lines
are the veins of the cubital fossa (basalic,
cephalic, antecubital vein, Fig. 2.2) and the great
saphenous vein. A good size vein appropriate for
the size of the PICC line is carefully selected
(ultrasonographic guidance can also be used),
and the PICC line is placed through an introducer
needle and tip advanced into a larger central vein.
Superior vena cava-atrial junction and the infe-
rior vena cava at the level of the diaphragm
should be the ideal location for the tip of PICC
line, which can be confirmed by a chest or an Fig. 2.2 Percutaneously inserted long line (PICC)
abdominal radiograph. through the antecubital vein
Types of Catheters
Fig. 2.3 Femoral central venous catheter
blood. It does not require any locks/clamps and
neither regular flushing with heparinized saline.
Central Venous Catheters
Central venous catheters are inserted at femoral
(Fig. 2.3), subclavian, and internal jugular (Fig. 2.4)
vein sites. They are used particularly in chronically
ill patients and can be maintained long term and
used to deliver large-volume infusions, blood prod-
ucts, TPN, and chemotherapy and also used for
blood sampling and critical care monitoring.
CVCs are available in many kinds of materials
such as polyethylene catheters, silicone, etc.
Percutaneous polyethylene catheters are placed
using local anesthetic under mild sedation and
strict asepsis using the Seldinger technique. The
right internal jugular is preferred due to its straight
course into the right atrium going in between the
two heads of the sternocleidomastoid muscle
toward the ipsilateral nipple and avoiding the
carotid artery, which lies medial to the vein
(Fig. 2.5b). The subclavian vein is accessed later-
ally and inferior to the midclavicular bend and
aiming toward the suprasternal notch and going
posteriorly at 30° to the chest wall (Fig. 2.5a).
High incidence of pneumothorax has been
reported with attempts at subclavian vein access.
13
Fig. 2.4 Internal jugular central venous catheter
Silicone CVCs (e.g., Hickman, Broviac cath-
eters) are more pliable and less irritant to the vein
and can be kept for long term. They are inserted
using an open surgical technique and have a cuff
at the proximal end which provides fixity and
hence less chances of dislodgement. The other
end has to be tunneled under the skin and brought
out from a different site away from the venous
end. Internal jugular, femoral, and subclavian
veins can be used.
Implantable Vascular-Access Devices
(Ports)
Implantable port system consists of a reservoir in
a subcutaneous pocket attached to a tunneled
central line draining into a central vein. Special
needles are used to puncture and deliver the drug
to the reservoir multiple times (up to 1000 times).
These require the least maintenance as the skin
acts as a natural barrier and the patient can
indulge in regular activities without special han-
dling or dressing. Port-A-Cath and Vascuport are
commercially available port devices. Excellent
durability and lowered infection rate are the
major advantages. Higher cost, surgical insertion
and removal, and special needle for multiple
punctures impede their widespread use.
14 2 Vascular Access in Children

Sternal notch
Clavicle

Subclavian
vein

Subclavian venous access

SCM

IJV

Carotid artery

Internal jugular venous access

Fig. 2.5 (a) Percutaneous subclavian vascular access. The needle is guided toward the sternal notch from just below
the midclavicular point. (b) Percutaneous internal jugular venous access between the heads of the sternomastoid
Other Vascular-Access Sites 15

 rinciples of General Care

P
for the Vascular Access

• Proper positioning
• Adequate assistance
• Radiologic/fluoroscopic guidance
• Negative pressure during needle aspiration
• Asepsis
• Proper fixation/immobilization
• Proper positioning of tip
• Good nursing care

Fig. 2.6 Umbilical venous catheter Complications of Vascular Access

Acute Complications
Other Vascular-Access Sites • Pneumothorax
• Air embolism
Umbilical Vascular Access • Arrhythmias
• Arterial puncture
The umbilical vein can easily be catheterized for • Brachial plexus injury
venous access in newborns during the first few days. • Cardiac tamponade
There are two umbilical arteries and a ­single umbili- • Failure/aberrant catheter placement
cal vein. The vein is the largest of them, thin walled, • Guide wire knotting/fracturing
and present at 12’o clock. The umbilical stump is • Hematoma at insertion site
freshened and the vein is looked for. The catheter is • Hemorrhage
passed into its lumen and extended till the desired • Hemothorax
length. The tip should lie in the inferior vena cava, • Thoracic duct injury
and blood can be aspirated freely. The position is • Vascular damage (e.g., perforation/dissection)
confirmed using an abdominal radiograph. Umbilical
vein catheterization is avoided in newborns with Pneumothorax is the most serious acute com-
abdominal defects, necrotizing enterocolitis and plication with central venous (subclavian or jug-
peritonitis. It can be used for fluid and drug adminis- ular) access in children. An upright chest
tration as well as blood sampling (Fig. 2.6). radiograph (or lateral decubitus image) should
be obtained after central venous access is
attempted.
Arterial Vascular Access

Arterial access is required for repeated blood gas Long-Term Complications

analysis and invasive blood pressure monitoring
in critically ill patients. Radial, posterior tibial,
and dorsalis pedis arteries are commonly used for
arterial cannulation. Though femoral artery can
also be used, it is best avoided in small children.
The Allen test is done to assess the collateral
blood flow. The radial artery is cannulated using
two fingers feeling the pulse wave and visualiz-
ing the course mentally.
• Catheter-associated bloodstream infections
and sepsis
• Thrombotic complications (e.g., deep venous
thrombosis, pulmonary embolus)
• Catheter migration/displacement
• Device occlusion
• Line fracture/embolization
• Phlebitis of the cannulated vessel
16
Catheter Dislodgement
and Migration
The most common long-term complications
include infection, thrombosis, catheter occlusion,
and, in some cases, catheter migration. Migration
may cause the catheter to malfunction. In some
cases, it can lead to perforation of the vessel wall.
Infection
Infection is the most common complication of
long-term vascular access. Incidence increases
with duration of catheter.
Fever, positive blood, and catheter tip cultures
should raise a suspicion of catheter-related infec-
tion. Broad-spectrum antibiotics should be admin-
istered through the catheter. If the child is in sepsis
and not improving, old catheters should be
removed, and a new one should be placed away
from the previous site.
2 Vascular Access in Children
Prevention of catheter-related infection should
be of highest priority for health-care providers.
Meticulous care during insertion, proper hand
washing, and regular change of dressing and
aseptic techniques of handling should be
followed.
Thrombosis
Central vascular-access devices should be flushed
regularly with heparinized sodium chloride to
reduce the incidence of thrombotic occlusion. In
case of partial thrombosis, different agents like
streptokinase, tissue-type plasminogen activator,
or heparin have been used with success.
Suggested Reading
Goldsmith JP, Karotkin E, Suresh G, Keszler M. Assisted
ventilation of the neonate. 5th ed. Philadelphia, PA:
Saunders, Elsevier; 2011.
 Parenteral Nutrition
Parenteral nutrition is administration of balanced
nutrition intravenously to meet daily energy require-
ments, help anabolism and support weight gain.
Normal Pediatric Growth
A term newborn gains weight at a rate of 25–30 g/
day leading to doubling of birth weight by 5
months of age. The birth weight triples by 12
months in an average infant. Similarly the body
weight increases by 50% at the end of 1 year. An
acute nutritional deprivation affects the body
weight and thus alters the weight-to-height ratio,
whereas length is a good indicator for long-term
body growth. Chronic malnutrition results in lag
in both weight and height.
I ndications for Giving Parenteral
Nutrition
Infants who remain in a fasting state for more than
5 days should be considered for nutritional supple-
mentation. Enteral route of feeding should always
be tried first, and even if full feedings are not toler-
ated, a small volume of “trophic” feedings are use-
ful to maintain intestinal function. Those patients
who can not be maintained on enteral nutrition are
candidates for parenteral nutrition.
© Springer Nature Singapore Pte Ltd. 2018
S. Roy Choudhury, Pediatric Surgery, https://doi.org/10.1007/978-981-10-6304-6_3
3
Some clinical conditions requiring parenteral
nutrition:
(a) Congenital anomalies: Gastroschisis, bowel
atresias, volvulus, and meconium ileus.
(b) Gastrointestinal disorders: Short bowel syn-
drome, malabsorption, intractable diarrhea,
inflammatory bowel disease, and enterocuta-
neous fistula.
(c) Others: Radiation therapy to the GI tract,
chemotherapy resulting in intolerance to oral
feeds, and severe respiratory distress syn-
drome in preterm babies.
 dministration of Total Parenteral
A
Nutrition (TPN)
TPN for <15 days of duration can be given by
peripheral access; for longer duration of paren-
teral nutrition, central access is ideal.
Percutaneously inserted central venous catheters
(PICC) are now most commonly used venous
access for TPN. The ideal position of the tip of
the central venous catheter is at the junction of
the superior vena cava and the right atrium.
Peripheral TPN solution should have osmolality
<600–900 mOsm/L and maximum dextrose con-
centration upto 12.5% to prevent venous
thrombophlebitis.
17
18
Composition of Parenteral Formulas
Parenteral nutrition can be partial or total (PPN
or TPN). Parenreral nutrition provides both
macro- and micronutrients.
Amino Acids
Amino acids are provided in crystalline form and
provide both essential and nonessential amino
acids. They provide 4 kcal/g, and approximately
10–15% of total calories are provided by amino
acids. Amino acids are started at the rate of 1 g/
kg/day and advanced to the goal (2.5–3 g/kg/day)
over next 2–3 days. Patients with liver failure and
encephalopathy require lower amounts of amino
acids.
Dextrose
Dextrose provides the major chunk of calories
(50–60%) and acts as a protein-sparing substrate.
Hydrous dextrose provides 3.4 kcal/g. Dextrose
is started at glucose infusion rate (GIR) of
4–6 mg/kg/min and advanced at 1–2 mg/kg/min
daily to a maximum GIR of 10–14 mg/kg/min.
Lipid Emulsions
Lipids are condensed source of energy and essential
fatty acids providing 9 kcal/g of energy. Lipids are
available in 10, 20, and 30% concentrations. Most
of the current available lipids are derived from soya
bean; another source is fish oil which is rich in
omega 3 fatty acids. Lipids provide 20–30% of total
calories. They are initiated at the rate of 0.5 to 1
g/kg/day and advanced by 1 g/kg/day to a maxi-
mum of 3 g/kg/day. Patients who develop parenteral
nutrition-associated cholestasis benefit from a
reduction of lipid content and switching over to fish
oil-based lipids.
3 Parenteral Nutrition
Multivitamins
Parenteral nutrition solutions should contain both
water- and fat-soluble vitamins.
Trace Elements
TPN solutions should provide zinc, copper, man-
ganese, chromium, and selenium.
Electrolytes
Sodium, potassium, chloride, acetate, calcium,
and phosphate are added in the TPN solutions
depending on the patient’s daily requirement.
Heparin
0.5–1 IU/mL heparin is added to TPN solution to
maintain patency of venous catheter.
Complications
(a) Metabolic complications: Hyperglycemia,
hypoglycemia, hypertriglyceridemia, meta-
bolic acidosis, and electrolyte disturbances.
(b) Hepatobiliary complications: Cholestasis,
steatosis, and cholelithasis.
(c) Infectious complications: Sepsis is the
most frequent and serious complication of
central TPN administration. Catheter-
related infections are the most frequent
cause of sepsis.
(d) Complications related to venous catheters:
Pneumothorax, cardiac arrhythmias, vessel
thrombosis, and catheter dislodgement.
(e) Complications from overfeeding: Osmotic
diuresis and immunologic suppression.
Suggested Reading
Monitoring Patients on TPN
Monitoring patients on TPN is essential. Complete
blood count, blood glucose, blood urea nitrogen,
creatinine, serum electrolytes, and liver function
tests need to be monitored. Patients on long-term
TPN require special monitoring of trace elements
to prevent toxicity and deficiencies.
19
Suggested Reading
Kliegman R, Stanton B, Geme JS, Schor N. Nelson
textbook of pediatrics. 20th ed. Philadelphia, PA:
Saunders/Elsevier; 2015.
Arcara K, Tschudy M. The harriet lane handbook. 20th
ed. Philadelphia, PA: Mosby/Elsevier; 2014.
 Antenatal Diagnosis and Fetal
Surgery 4

All pregnant mothers are now offered antenatal Table 4.1 First- and second-trimester markers for
screening tests for early detection of fetal anoma- aneuploidy
lies. Counseling of the family regarding the pos- Soft markers Major markers
sible outcome of antenatally detected anomalies • Nuchal fold thickness • Cardiac defect
becomes essential. This is currently best done by (>5 mm)
a team of experts from obstetrics, pediatric sur- • Mild ventriculomegaly • Duodenal atresia
gery, neonatology, and radiology. • Echogenic bowel • Cystic hygroma
• Humerus and femur length • Hydrops
• Echogenic intracardiac focus • MMC
• Pyelectasis • Omphalocele
Fetus as a Patient • Nasal bone • CDH/EA
• Choroid plexus cyst • Limb anomalies
The role of antenatal counseling is to provide • Clinodactyly • Orofacial
anomalies
information to perspective parents regarding fetal
• Sandal gap toe • Renal anomalies
outcome, possible interventions, appropriate set-
tings, time and route of delivery, and expected
postnatal outcomes. The benefit not only includes Ultrasonography is the ideal screening test. A
care of the newborn in an appropriate center but detailed structural sonography done at 18 weeks
is also associated with high level of family satis- detects major structural abnormalities (Table 4.1).
faction. The care of such patients should begin
early, and counseling should include follow-up Fetal Malformations Which Can Be Detected
and future pregnancy planning. by Antenatal Ultrasonography
Procedures currently available for fetal diag- • Spina bifida and hydrocephalus (Fig. 4.1)
nosis are: • Craniofacial/limb anomalies (Fig. 4.2)
• Twins/conjoined twins
• Ultrasonography and echocardiography • Abdominal wall defects (gastroschisis, exom-
• Amniocentesis phalos) (Fig. 4.3a, b)
• Chorionic villous biopsy (CVB) • Intestinal atresias (esophageal, duodenal,
• Enzymatic assays and genetic studies and bowel) (Figs. 4.4, 4.5, 4.6, and 4.7)
immunohistochemistry • Congenital lung lesions (Fig. 4.8)
• Fetal MRI • Renal tract disease

© Springer Nature Singapore Pte Ltd. 2018 21

S. Roy Choudhury, Pediatric Surgery, https://doi.org/10.1007/978-981-10-6304-6_4
22 4 Antenatal Diagnosis and Fetal Surgery

Fig. 4.2 Antenatal scan showing spina bifida with

meningomyelocele

Fig. 4.1 Scan showing Holoprosencephaly

Fig. 4.3 (a) Antenatal scan showing omphalocele, (b) large omphalocele with sac
Fetus as a Patient
• Congenital diaphragmatic hernia (Fig. 4.9)
• Bladder/cloacal exstrophy
• Cardiac defects
• Tumors: teratomas and lymphangiomas
Fig. 4.4 Antenatal scan showing duodenal atresia
Fig. 4.5 Scan showing intraperitoneal calcification (meconium peritonitis)
23
Screening Tests
A second-trimester (15–20 weeks) screening for
aneuploidy (trisomy 18 and 21 and open neural
tube defects) is done with a quad test which
includes alpha-fetoprotein (AFP), human chori-
onic gonadotropin (hCG), unconjugated estriol
(uE3), and inhibin A. First-trimester screening
(9–13 weeks) is done with pregnancy-associated
plasma protein A (PAPA-A), human chorionic
gonadotropin (hCG), and nuchal translucency
(NT) measurement. A combination of screening
tests and several sequential tests have been advo-
cated. A positive screening test (including nuchal
translucency >5 mm) is highly suggestive of
aneuploidy, and the family should be offered the
invasive tests of chorionic villous sampling
(CVS) between 10 and 12 weeks or amniocente-
sis between 15 and 20 weeks.
24 4 Antenatal Diagnosis and Fetal Surgery

Fig. 4.6 Scan showing fetal ascites

Fig. 4.7 Fetal MRI showing large cystic lesion in the abdomen, meconium pseudocyst
Fetus as a Patient 25

Fig. 4.8 Antenatal scan showing a large cystic lesion in the thorax

Currently cell-free fetal DNA testing in the
maternal blood offers an effective screening
test for trisomy 18 and 21. However, because
of its limitation of detecting fetal abnormali-
ties and questionable cost-effectiveness,
c­onventional screening method is still the
choice. Hence, a combination of screening
tests with a comprehensive ultrasonographic
examination is currently recommended for all
pregnancies.
26
Fig. 4.9 Antenatal scan at 16 weeks showing stomach in the chest suggestive of congenital diaphragmatic hernia
Antenatal Diagnosis and Treatment
Antenatal diagnosis helps in planning further
management.
For antenatally detected anomalies, the fol-
lowing modes of management may be adopted:
• Managed by termination: For malformations
which are considered incompatible with life.
Anencephaly, hydranencephaly, holoprosen-
cephaly, severe chromosomal anomalies like
trisomy 13, bilateral renal agenesis, infantile
polycystic renal disease, metabolic disease like
Tay-Sachs disease, and lethal bone dysplasias
• Correction after term delivery: Most of the
malformations are managed by this mode.
Pediatric surgical team should be available to
manage the new born. Esophageal and gastro-
intestinal atresias; duplications; meconium
ileus; small omphalocele or gastroschisis; uni-
lateral hydronephrosis; craniofacial, limb, or
4 Antenatal Diagnosis and Fetal Surgery
chest wall deformities; small cystic hygromas;
teratomas; mesoblastic nephromas; neuro-
blastomas; cysts like choledochal, ovarian,
and mesenteric; cystic lung malformations
and without hydrops; and diaphragmatic her-
nia without much lung hypoplasia
• Cesarean delivery: For large lesions which
may interfere with normal delivery. Conjoined
twins, large hydrocephalus or meningomyelo-
cele, giant omphalocele, and large sacrococ-
cygeal or cervical teratoma
• Induced preterm delivery: Where continuing
with pregnancy considered harmful. Progressive
hydrops fetalis, intrauterine growth retardation,
progressively enlarging hydrocephalus,
­hydrothorax, abdominal wall defects with dam-
aged bowel, intestinal ischemia, and necrosis
secondary to volvulus or meconium ileus
• EXIT (ex utero intrapartum procedure): The
fetus is maintained on utero placental circula-
tion till the airway is secured. This is done for
Indications for In Utero Fetal Intervention
fetuses with compromised airway e.g. con-
genital high airway obstruction syndrome
(CHAOS), large cervical teratoma, mass (like
cystic hygroma) obstructing airway, congeni-
tal chest masses preventing lung expansion,
and lung immaturity requiring ECMO
• Fetal intervention
Fetal Surgery
Defects Sometimes Treated by Open Fetal
Surgery
• Congenital cystic adenomatoid malformation
and pulmonary sequestration
• Congenital heart disease
• Myelomeningocele
• Sacrococcygeal teratoma
 IS (Minimally Invasive) Fetal
M coamniotic shunting for fetal lower urinary tract
Surgery
The current indications for minimally invasive
fetoscopic surgery (Fetendo) are:
• Twin-twin transfusion syndrome – laser abla-
tion of vessels supplying the parasitic twin
• Fetal bladder obstructions – vesicoamniotic
shunt and fetoscopic valve ablation
• Aortic or pulmonary valvuloplasty and atrial
septostomy
• Congenital diaphragmatic hernia – balloon
tracheal occlusion (PLUG)
• Spina bifida – fetoscopic closure of the
malformation
I ndications for In Utero Fetal
Intervention
 ntenatal Diagnosis and
A Fetal procedures are
Management of Lower Urinary Tract
Obstruction (LUTO)
Fetuses with normal USG findings before 24
weeks fare better than those with abnormal find-
ings before 24 weeks. Ultrasonographic features
27
of bilateral hydroureteronephrosis, enlarged blad-
der, dilated posterior urethra, and ­oligohydramnios
in a male baby with normal karyotype and no
other life-threatening malformations are consid-
ered for intervention. Favorable urinary electro-
lytes are sodium <100 mEq/L, chloride
<90 mEq/L, osmolality <210 mEq/L, and β2-­
microglobulin levels <6 mg/L. Elevated fetal uri-
nary electrolytes and β2-microglobulin levels are
indication of irreversible renal damage.
Available Fetal Interventions
(a) Vesicoamniotic shunt. Double pigtail stent
inserted with one end in the bladder and the
other in the amniotic cavity.
(b) Fetal cystoscopy. A 1–1.3 mm cystoscope is
inserted in the fetal bladder and guided in the
posterior urethra for valve ablation.
The definite advantage of percutaneous vesi-
obstruction (PLUTO trial) is still unclear.
Fetal Therapy for Hydrocephalus
Hydrocephalus is defined as fetal lateral ventric-
ular measurement (at atria) > 10 mm.
Detailed workup for associated abnormalities
includes USG, fetal MRI, karyotype, viral cul-
tures of amniotic fluid, and family history.
Management
With associated severe abnormalities, termina-
tion should be considered.
For isolated hydrocephalus with stable mild
ventricular dilatation can be observe till term.
With progressive ventricular dilatation, if ges-
tational age > 32 weeks, perform delivery and
shunting may be considered.
If gestational age <32 weeks, consider for
fetal therapy but in context of clinical trial.
(a) Serial cephalocentesis
(b) Ventriculo-amniotic shunt
Fetal treatment has no obvious documented
benefit at present.
28
Fetal Therapy for Meningomyelocele
Rationale for Fetal Treatment
• Defective primary neurulation defect with
normal neural tissue
• Neural tissue damage secondary to exposure
to amniotic fluid or delivery
• Fetal repair/covering of defect may improve
neurological outcome
Current fetal surgical treatment is open repair
of meningomyelocele in a fetus with open defect
and presence of hindbrain herniation.
A prospective randomized control trial of prena-
tal versus post natal repair of meningomyelocele
has been completed in the USA (MOMS trial).
Fetal repair of spina bifida was found to be supe-
rior to postnatal repair in terms of the following:
• Incidence of Chiari malformation was found
less in the fetal repair group.
• Need for ventricular shunt was found less in
the fetal repair group.
• Motor skills were better in the fetal repair
group.
 etal Therapy for Congenital Cystic
F
Adenomatoid Malformation
 iagnosis by Imaging
D
Macrocystic lesions are cysts > 5 mm in
diameter.
Microcystic lesions are solid echogenic masses.
Sequestrations are solid echogenic lesions with
an anomalous systemic blood supply.
Monitoring
The parameters are
CCAM volume ratio (CVR): (height × width
× depth × 0.523) ÷ head circumference
CVR > 1.6 is predictive of increased risk of
hydrops; value plateaus at 28 weeks
Indications for in utero intervention (thoraco-­
amniotic shunt or fetal lobectomy):
(a) Evidence of early hydrops
(b) High risk of pulmonary hypoplasia (large
lesion with mediastinal shift and <24 weeks)
4 Antenatal Diagnosis and Fetal Surgery
Outcome
In fetuses with hydrops without intervention, the
mortality rate can be as high as 100%. Post-­
intervention survival rates as high as 74% have
been reported.
 etal Management of Congenital
F
Diaphragmatic Hernia
Plug the lung until it grows (PLUG) is cur-
rently introduced to reverse pulmonary hypopla-
sia in CDH. Fetal tracheal occlusion is induced to
promote lung growth by preventing egress of
fluid from the lung.
Indication for fetal interventions in CDH is
high-risk fetuses with (lung-to-head ratio)
LHR < 1 or equivalent.
Fetal endoscopic tracheal occlusion
(FETO). A 1.3 mm scope can be used to place a
balloon in the trachea. Balloon is placed at 26–29
weeks and deflated by USG-guided puncture/tra-
cheoscopy at 34–36 weeks or by EXIT (ex utero
intrapartum treatment).
Antenatal Diagnosis
and Management of Abdominal Wall
Defects
Accurate prenatal diagnosis of the type of defect is
important (gastroschisis, omphalocele, exstrophy/
cloacal exstrophy) for planning the management.
An isolated defect versus chromosomal and
genetic syndromes affects patient management
and prognosis. Those with severe chromosomal
or genetic defects should be offered termination
(Fig. 4.3).
The clinical outcome of antenatally diagnosed
abdominal wall defects appears to be favorable
with postnatal treatment.
Suggested Reading
Holcomb GW III, Murphy PJ, Ostile DJ, editors.
Ashcraft’s pediatric surgery. 6th ed. Philadelphia, PA:
Saunders/Elsevier; 2014.
Bianchi MD, Crombleholme TM, Malone FD. Fetology:
diagnosis and management of the fetal patient. 2nd ed.
New York: McGraw-Hill Education; 2010.
 Vascular Anomalies
5

There are several confusing nomenclatures for I nfantile Hemangioma (Strawberry

vascular malformations like nevus, angiomas, Angioma) (Figs. 5.2a, b, 5.3, and 5.4)
hemangiomas, etc. However, the classification
needs to be based on growth of blood vessels Infantile hemangiomas (IH) are the most com-
(angiogenesis) and biologic and clinical behav- mon tumors of infancy. They most often occur as
iors which have implication for diagnosis and single lesions with a predilection for the head and
treatment. neck. They appear in the neonatal period gener-
ally around 2 weeks of age. A macule may be
present at birth as a premonitory cutaneous mark.
Classification IH have a characteristic cycle of rapid growth in
the first year of life (proliferative phase) followed
Currently the classification suggested by by spontaneous slow regression between 1 and 6
International Society for the Study of Vascular years of life (involution phase). Spontaneous
Anomalies is being followed (Fig. 5.1). regression is associated with the best cosmetic
Hemangiomas are distinctly different from result as they do not recur.
vascular malformations as shown below. Large hemangiomas (Figs. 5.5a, b and 5.6) may
cause platelet trapping leading to thrombocytope-
Hemangiomas Vascular malformations nia (Kasabach-Merritt phenomenon), and vascular
• Infantile hemangiomas: • They occur due to shunting may cause high-output cardiac failure.
true neoplasm, arise dysmorphogenesis.
from endothelial Normal endothelial cell Infantile hepatic hemangiomas behave similar to
hyperplasia turnover cutaneous hemangiomas with potential for sponta-
• 30% are visible at • Always present at neous regression and thus require close observa-
birth birth tion only. Diffuse hepatic hemangiomas may
• Common in females • M/F equal ratio
• Proliferative and • Grow and expand cause cardiac failure or respiratory failure due to
involuting phase throughout life; do not rapid enlargement and need intervention. Large
• Responds to involute hemangiomas of the face especially with involve-
pharmacologic therapy • Do not respond to ment of the “beard” region are associated with
• GLUT-1 positive pharmacologic therapy
• GLUT-1 negative subglottic hemangioma which may cause airway
compromise. Presence of more than five cutane-

© Springer Nature Singapore Pte Ltd. 2018 29

S. Roy Choudhury, Pediatric Surgery, https://doi.org/10.1007/978-981-10-6304-6_5
30 5 Vascular Anomalies

Vascular Anomalies

Vascular Vascular
Tumors Malformations

Hemangioma Others
· Kaposiform
hemangioendothelioma
Infantile
· Tufted angioma
hemangiomas
· Kaposi sarcoma
· Pyogenic granuloma

Congenital
Hemangioma Slow Flow Fast flow
· Rapidly involuting · Arteriovenous
congenital fistula
hemangioma (RICH) · Arteriovenous
· Non-involuting malformation
congenital
hemangiomas (NICH)
Capillary Combined
malformations · Klippel-Trenaunay
syndrome (Capillary-
lymphatic-veous
Lymphatic
malformation)
malformations
· Parkes Weber
syndrome
Venous (Capillary-Arterivenous
malformation malformation)

Fig. 5.1 Classification of vascular anomalies

a b

Fig. 5.2 (a, b) Infantile hemangioma face

Infantile Hemangioma (Strawberry Angioma) 31

ous hemangiomas warrants abdominal ultrasonog-

raphy to rule out visceral hemangioma.

Investigations

• Ultrasound is only needed when diagnosis is

uncertain or to rule out visceral lesions.
• USG with Doppler – Can demonstrate high-flow
lesions especially during the proliferative phase.
• MRI.

If the number is more than 5, then exclude vis-

ceral lesions.
Fig. 5.3 Infantile hemangioma lip

Fig. 5.6 Combined malformation, limb gigantism, and

Fig. 5.4 Deep hemangioma consumptive coagulopathy

a b

Fig. 5.5 Large infantile hemangioma in the (a) neck and chest (b) parotid region
32
Treatment
1. Observation with reassurance and regular pho-
tography to monitor the progress of the lesion.
Observation over a period gives best cosmetic
result with spontaneous involution.
2. Pharmacotherapy: Intervention is needed for
large ulcerated hemangiomas, hemangiomas
interfering with limb function, or encroachment
to vital structures like the eye, nose, airway, etc.
(a) Steroids (high dose, systemic/intralesional),
prednisolone – 2 mg/kg body weight orally
for 2 weeks and then are gradually tapered;
response rate is 80–90%. Gastritis is a fre-
quent complication, so antacids are also
prescribed. All live vaccines are withheld
till the child is on steroids; steroids act by
inhibiting angiogenesis.
(b) Vincristine.
(c) Interferon 2 alpha a and b (out of favor).
(d) Beta-blockers: Propranolol has recently
been discovered to be useful in manage-
ment of IH and has now become the first
line in pharmacotherapy for IH due to
good efficacy and minimal side effects.
Propranolol is gradually increased to a
a b
Fig. 5.7 (a, b) Port-wine capillary malformation
5 Vascular Anomalies
dose of 2 mg/kg body weight orally for
3–6 weeks or even longer till 9–12 months
of age so as to suppress the proliferative
phase. They act initially by causing vaso-
constriction, later by inhibiting angiogen-
esis and in the long term promote
apoptosis in the lesion.
3. Laser treatment is not ideal.
4. Surgery is indicated for ulceration, hemor-
rhage, interference with vital structures and
functions, and where pharmacotherapy has
failed or for primary treatment of small pedun-
culated lesions.
Vascular Malformation
Port-Wine (Capillary Malformation)
They are flat, pink, purple lesions with geograph-
ical distribution (Fig. 5.7a, b).
Associations are Sturge-Weber syndrome (tri-
geminal distribution), spina bifida, and
encephalocele.
If untreated, they persist in the adulthood, the
color darkens, and the skin thickens.
Venous Malformation 33

Treatment

Indication for treatment is mainly cosmetic.

Flash lamp pulse-dye laser can be applied.
Result of therapy: lightening of color occurs
in 70% cases and a complete disappearance in
15–20% cases.
Disadvantages: need for anesthesia, multiple
sittings, and high cost of treatment.
Complications: thinning, hypo/hyperpigmen-
tation, scarring, and pain.

Venous Malformation

Clinical Features

These lesions are dark colored, diffuse, spongy,

compressible and involve deeper tissue
(Fig. 5.8).

Diagnosis

Imaging with USG Doppler, MRI and MR venog-

raphy aids in the diagnosis.

Treatment

• Graduated compression stocking may be
helpful.
• Intralesional sclerotherapy with agents like
alcohol, sodium tetradecyl sulfate (setrol),
ethanolamine oleate, doxycycline, or polico-
sanol is used with variable success rate.
Multiple sittings may be required. Prior emp-
tying blood from the lesion and injections in
multiple sites with postinjection compression
improves the success rate.
Fig. 5.8 Venous malformation on the chest wall and
upper limb
• Surgical resection for well-localized lesion
can be done.
• Argon/Nd:Yag laser is indicated for only
superficial lesions with high recurrence rate.
34 5 Vascular Anomalies

Lymphatic Malformation Treatment

(Cystic Hygroma, Lymphangioma)
(See Chap. 6) • Angio-embolization
• Surgical excision
Treatment • Embolization followed by surgical excision

• Treatment of infection if present Combined Vascular Malformation

• Sclerotherapy for macrocystic lesion (bleo-
mycin, doxycycline, picibanil)
• Surgery for microcystic lesions, residual
lesion after sclerotherapy
• Radiofrequency ablation (for low-flow lesions)
(a) Klippel-Trenaunay syndrome, slow-flow,
capillary-lymphatic-venous malformation
(b) Parkes Weber syndrome, fast-flow, capillary
arteriovenous malformation

Diagnosis: MRI
 rteriovenous Malformation (AVM)/
A Treatment: combination of different therapy
Arteriovenous Fistula Vascular malformations involving tongue
(Fig. 5.11), breast (Fig. 5.12), parotid region
Diagnosis (Fig. 5.13) and involuting hemangioma
(Fig. 5.14).
High-flow lesion that could be pulsatile. Limb
and tissue overgrowth often occurs (Figs. 5.9a, b
and 5.10).
MR angiography is diagnostic.

a b

Fig. 5.9 (a) Overgrowth of limb due to arteriovenous malformation, (b) MRA angiography showing AVM
Summary of Treatment 35

Fig. 5.10 Arteriovenous malformation foot, pulsatile

lesion, note dilated vessels around the lesion Fig. 5.13 Vascular malformation parotid region

Fig. 5.11 Vascular malformation of tongue

Fig. 5.14 Involuting hemangioma

Summary of Treatment

Type of lesion Treatment

Infantile hemangioma Observation/
pharmacotherapy/surgery
Capillary malformation Laser therapy
Venous malformation Sclerotherapy, compression
therapy, surgery
Lymphatic malformation Sclerotherapy, surgery
Arterial malformation Embolization, surgery
Combined Combination therapy
Fig. 5.12 Vascular malformation involving breast malformation
36 5 Vascular Anomalies

Suggested Reading Gupta DK, Sharma S, Azizkhan RG, editors. Pediatric

surgery—diagnosis and management. 1st ed. New
Delhi: Jaypee Brothers; 2009.
Coran AG, Caldamone A, Scott Adzich N, Krummel TM,
Laberge JM, editors. Pediatric surgery. 7th ed. Oxford:
Elsevier Limited; 2012.
 Lymphangiomas
Lymphangiomas or lymphatic malformations are
a variety of congenital hamartomatous malfor-
mation of the lymphatic system. The lesions can
be superficial or deep and may have mixed
venous elements. The commonest variety in chil-
dren is deep cavernous lymphangioma. Although
they are often known as cystic hygroma, the term
was originally used by obstetricians for fetal
­posterior cervical translucent lesions associated
with high incidence of other anomalies.
Lymphatic Malformation
Embryology
In the sixth week of intrauterine life, the primi-
tive lymph sacs (principal pair) develop between
jugular and subclavian veins. Sequestration of a
portion of jugular lymph sac from the lymphatic
system accounts for this malformation.
Pathology
It consists of multiple cysts giving the appear-
ance of a mass of soap bubbles – larger cyst on
the surface and smaller ones infiltrating into mus-
cle and tissue planes. This makes surgical exci-
sion difficult and challenging. There are
macrocystic or microcystic varieties depending
on the predominant size of the cysts. It contains
clear lymphatic fluid and is lined with endothe-
© Springer Nature Singapore Pte Ltd. 2018
S. Roy Choudhury, Pediatric Surgery, https://doi.org/10.1007/978-981-10-6304-6_6
6
lium. The fluid is lymphocyte-rich which can aid
in pathological diagnosis. The term “cystic
hygroma” was originally used by obstetricians
for posterior nuchal translucent cystic lesions in
the fetus often detected by ultrasonography. Such
lesions have a high association with aneuploidy
and other malformations. These should not be
confused with lymphatic malformations which
are generally larger lesions, situated more anteri-
orly in the head and neck and not associated with
other malformations, hence having better
prognosis.
Sites
They can occur anywhere. Lower third of the
posterior triangle of neck is a common site, other
sites being the cheek, axilla, groin, mediastinum,
intrathoracic and intra-abdominal cavity, and
tongue (Figs. 6.1, 6.2, and 6.3).
Clinical Features
The lesion may present at birth or appear early
in infancy. Large swelling may obstruct labor.
The characteristics of the swelling are soft, cys-
tic, and partially compressible due to intercom-
munication of cysts. It may increase in size with
cough or cry and is usually brilliantly transillu-
minant. Clinical behavior is uncertain, as it may
increase in size due to infection (Fig. 6.4) or
37
38
Fig. 6.1 Extensive lymphangioma of the neck
Fig. 6.2 Lymphangioma of the chest wall
6 Lymphangiomas
Fig. 6.3 Extensive lymphangioma involving the groin
and extremities
hemorrhage and may cause airway obstruction
(Fig. 6.5). It may even decrease in size and dis-
appear. Neck lesions may have intrathoracic
extension (Fig. 6.6).
Investigations
Ultrasonography is useful for diagnosis as it
shows the cystic nature of the swelling. However,
the detailed anatomical extent can be better
defined with magnetic resonance imaging (MRI).
Complications
Infection, hemorrhage, and airway obstruction
(Figs. 12.4 and 12.5) are common complications.
Treatment
Lesions involving small babies and asymptom-
atic small lesions may be observed. For large and
symptomatic lesions, sclerosant injection therapy
is effective.
Sclerosant injection after aspiration of the cyst
fluid is usually successful in the macrocystic
Investigations
variety. However, results are unsatisfactory when
it is used for the microcystic (solid) type. The dif-
ferent agents used are hypertonic saline, bleomy-
cin (0.2 IU/kg body weight, not exceeding
0.5 mg/kg body weight repeated at intervals of
2–3 weeks with maximum cumulative dose not
exceeding 5 mg/kg body weight), sodium tetra-
decyl sulfate, doxytetracycline, and OK-432
therapy (lyophilized mixture of group A
Streptococcus pyogenes and benzylpenicillin).
Application of gentle compression after scle-
Fig. 6.4 Lymphangioma of the neck with infection
a b
Fig. 6.6 (a–c) Chest X-ray (a) and CT scan showing lymphangioma involving the neck with mediastinal extension;
note insinuation between the vessels
39
rosant injection improves the effectiveness and
avoids hematoma formation. Sclerotherapy
results in obliteration of lymphatic channels with
minimal fibrosis. The mechanism of action is
probably cytokine-mediated damage to endothe-
lial cells.
Presence of infection requires treatment with
antibiotics with or without aspiration of the
contents.
Surgical excision is usually done for the residual
lesion after sclerotherapy or for easily removable
lesions. Primary surgical excision for extensive
lesions infiltrating into nerves, vessels, or vital
structures is challenging and better avoided.
Fig. 6.5 Airway obstruction due to neck lymphangioma
c
40 6 Lymphangiomas

Airway obstruction may require tracheostomy Gupta DK, Sharma S, Azizkhan RG, editors. Pediatric
or urgent cyst aspiration. surgery—diagnosis and management. 1st ed. New
Delhi: Jaypee Brothers; 2009.
Holcomb GW III, Murphy PJ, Ostile DJ, editors.
Ashcraft’s pediatric surgery. 6th ed. Philadelphia, PA:
Suggested Reading Saunders, Elsevier; 2014.

Coran AG, Caldamone A, Scott Adzich N, Krummel TM,

Laberge JM, editors. Pediatric surgery. 7th ed. Oxford:
Elsevier; 2012.
 Part 2
Trauma
 Pediatric Head Injury
7

Head injury is a major cause of death and disabil- Once hypoxemia and hypotension have been
ity in children. Young children sustain head corrected and the patient is stabilized, neurologi-
injury as pedestrians or from falls. Older children cal assessment can be undertaken.
sustain head injury as a passenger or driver.
Assault also constitutes a significant cause of
head injury. Airway, breathing, and circulatory  ssessment of Head Injury by
A
(ABC) management takes priority over neuro- Glasgow Coma Scale (GCS)
logical assessment. Head injury may initially
appear the most obvious (Fig. 7.1); it is not ini- Eyes
tially the most important. Oxygenation, ventila-
tion, and maintenance of adequate cerebral <1 year >1 year
perfusion pressure are vital. 4 Opens eyes Opens eyes
spontaneously spontaneously
3 Opens to shout Opens eyes to verbal
command
2 Opens to pain Opens eyes to pain
1 No eye opening No eye opening

Motor
<1 year >1 year
6 Normal movements Obeys verbal
commands
5 Localizes to noxious Localizes to noxious
stimuli stimuli
4 Flexion withdrawal Flexion withdrawal
3 Flexion/decorticate Flexion/decorticate
posturing posturing
2 Extension/decerebrate Extension/
posturing decerebrate posturing
1 No response to noxious No response to
stimuli noxious stimuli
Fig. 7.1 Black eye following head trauma

© Springer Nature Singapore Pte Ltd. 2018 43

S. Roy Choudhury, Pediatric Surgery, https://doi.org/10.1007/978-981-10-6304-6_7
44 7 Pediatric Head Injury

Verbal • Severe mechanism of injury

• Occipital/parietal or temporal hematoma in
0–23 months 2–5 years >5 years <2-year-old child
5 Smiles/coos/ Appropriate Orientated • History of vomiting or severe headache in
cries words/
appropriately phrases
>2-year-old child
4 Cries/ Inappropriate Confused • Focal neurological deficit
consolable words • Loss of consciousness >5 s in <2 years old or
crying/screams any duration in >2 years old
3 Irritable/ Cries/ Inappropriate
inconsolable screams Additional consideration in children:
2 Grunts/agitated Grunts Incomprehensible
1 None None None
• Seizures
• Bruising, swelling, and laceration >5 cm if
<1 year old
Grading of Head Injury • Non-accidental injury
• High-speed traffic accident >40 mph
Mild head injury GCS 14–15 • Fall from height >3 m
Moderate head injury GCS 9–13 • High-speed injury from a projectile or object
Severe head injury GCS 3–8

 anagement of Children with Mild

M
I ndications for Immediate Head Injury (Table 7.1)
Computed Tomography (CT) Scan
 anagement of Moderate and Severe
M
• GCS <13 when assessed Head Injury
• Other signs of altered mental status
• Any deterioration in condition • Arrange pediatric intensive care unit (PICU)
• Suspected open or depressed skull fracture admission.
(Fig. 7.2) • Secure airway if GCS < 8 or worsening neuro-
• Any sign of basal skull fracture logical status.

a b

Fig. 7.2 Traumatic fracture of skull bone with soft tissue hematoma, (a) clinical and (b) CT scan
Management of Children with Mild Head Injury 45

Table 7.1 Management algorithm of children with mild brain injury (i.e., GCS = 14, 15)

Age < 2years Age > 2years

GCS =14 GCS =14

CT
Signs of altered mental status YES recommended YES Signs of altered mental status

Palpable skull fracture Signs of basilar skull fracture

NO
NO

Occipital/temporal/ parietal hematoma Vomiting

History of LOC>5 sec CT NOT History of LOC

NO recommended NO
Severe mechanism of injury Severe mechanism of injury

Child not acting normally as per parent Severe headache

YES

YES
OBSERVATION versus CT on basis of clinical factors

Physician experience

Multiple vs isolated findings

Worsening signs or symptoms

Parental preference

Age < 3months

• The head of the bed should be elevated (with-
out hip flexion). The child’s head should be
kept in the midline, neutral position (to avoid
jugular venous compression and spinal cord
injury).
• Ventilate to low normal PaCO2 or hyperventi-
late while other treatments take effect in
impending cerebral herniation.
• Aggressively treat hypotension with IV fluid
boluses and vasopressors.
• Provide adequate analgesia (morphine) and
sedation (midazolam).
• Paralyze with muscle relaxants.
• Mannitol 0.5–1 g/kg (2.5–5 mL/kg of 20%
mannitol) by intravenous infusion over
20 min.
• Consider hypertonic saline (3–5 mL/kg of 3%
saline) intravenous bolus (if given rapidly may
result in drop of blood pressure).
• Monitor intracranial pressure (ICP) if GCS <8
by ventriculostomy and maintain ICP
<20 mmHg by CSF drainage if necessary.
• Hyperthermia should be avoided (>37.5 °C).
All pediatric head-injured patients that
require intravenous fluid for maintenance or
46 7 Pediatric Head Injury

resuscitation must receive 0.9% NaCl ± • Vomiting

10 mmol KCl/500 mL.
Further management is dependent on CT scan
findings and consultation with a pediatric
neurosurgeon.
 he Warning Signs to Watch by
T
the Parents After Discharge
• Confusion or unusual behavior
• Any change in the way your child walks or
uses their arms/legs
• Blurred vision or slurred speech
• Such signs should be immediately reported to
the hospital.

• Unusual sleepiness Suggested Reading

• Jerking movements of arms, legs, or face—“a
fit”
• Severe headache that paracetamol does not
relieve
Wesson DE, Cooper A, Stylianos S, editors. Pediatric
trauma pathophysiology diagnosis and treatment. 1st
ed. New York, NY: Taylor & Francis; 2006.
 Abdominal Trauma
Abdominal trauma constitutes 3–5% of all pedi-
atric admissions. Blunt injury is more common
than penetrating injuries. A child’s abdomen is
more prone to injury by virtue of its anatomical
characteristics:
• Small size of the abdomen predisposes the
child to multiple injuries as energy from the
force is dissipated over a small area.
• Ribs are flexible and more compliant, hence
less effective in protecting the upper abdomi-
nal structures (e.g., spleen, liver).
• The diaphragm being more horizontal pushes
the liver and spleen below the rib cage.
• Solid organs in child are relatively large; more
surface area is exposed.
• Muscle, fat, and fascia are thin.
• The bladder is intra-abdominal due to shallow
pelvis and hence more prone to injury.
The principle of management follows in the
line of general trauma care. All patients of abdom-
inal trauma should undergo standard trauma care
approach with primary survey; care of airway,
breathing, and circulation (ABC); and X-ray of
cervical spine, chest, and pelvis. Ultrasound
examination of the abdomen offers quick identifi-
cation of any solid organ injury (FAST, see
below). CT scan has become the standard of care
in pediatric trauma patient and is highly accurate
in demonstrating solid organ injuries.
© Springer Nature Singapore Pte Ltd. 2018
S. Roy Choudhury, Pediatric Surgery, https://doi.org/10.1007/978-981-10-6304-6_8
8
Liver Trauma
Grading of liver trauma is done as per American
Association for the Surgery of Trauma (AAST)
liver injury scale (Table 8.1).
Imaging studies are the main diagnostic
modality for evaluation of the presence or
absence of liver injury.
Ultrasonography
Focused assessment with sonography in trauma
(FAST) is advocated as initial evaluation in
trauma. A FAST exam consists of sonographic
evaluation of the pericardium; right upper quad-
rant, including Morrison’s pouch; left upper
quadrant; and the pelvis. This evaluation is to
identify the presence of blood.
Computed Tomography Scan
CT scan is the standard imaging study in trauma
for hemodynamically stable patients (Fig. 8.1a,
b). Grading for severity of injuries is also based
on CT scan examination (Table 8.1). Extravasation
of contrast demonstrated on CT scan suggests
active bleeding from injury site, and further inter-
vention may be warranted.
47
48
Table 8.1 Grading of liver trauma
Grade Type Injury description
I Hematoma Subcapsular, nonexpanding,
<10 cm surface area
Laceration Capsular tear, nonbleeding,
<1 cm parenchymal depth
II Hematoma Subcapsular, nonexpanding,
10–50% surface area;
intraparenchymal nonexpanding
<10 cm diameter
Laceration Capsular tear, active bleeding,
1–3 cm parenchymal
depth <10 cm in length
III Hematoma Subcapsular, >50% surface area
or expanding; ruptured
subcapsular hematoma with
active bleeding; intraparenchymal
hematoma >10 cm or expanding
Laceration >3 cm parenchymal depth
IV Hematoma Ruptured intraparenchymal
hematoma with active bleeding
Laceration Parenchymal disruption involving
25–75% of hepatic lobe or one to
three Couinaud’s segments within
a single lobe
V Laceration Parenchymal disruption involving
>75% of hepatic lobe or >3
Couinaud’s segments within a
single lobe
Vascular Juxtahepatic venous injuries (i.e.,
retrohepatic vena cava/central
major hepatic veins)
VI Vascular Hepatic avulsion
a b
Fig. 8.1 (a) CT scan showing right lobe liver laceration. (b) CT scan showing extensive liver hematoma following
blunt trauma
8 Abdominal Trauma
Angiogram and Angio-Embolization
Angiography has played a vital role in non-­operative
management of liver injuries. Active bleeding dem-
onstrated by contrast leak within or around the
injured organ can be managed with angiography
and angio-embolization in hemodynamically stable
patients. Postoperative angio-­embolization in case
of rebleeding is also reported in damage control sur-
gery prior to removal of packs.
Diagnostic Peritoneal Lavage (DPL)
DPL was widely used to detect hemoperitoneum
in abdominal trauma, but with the availability of
focused assessment with sonography in trauma
(FAST) and CT scans, it is rarely done nowadays.
It may still be useful in suspected hollow viscus
injury with normal CT scan as an alternative to
laparoscopy.
Criteria for positive peritoneal lavage:
• 10 mL gross blood return with lavage catheter
insertion
• >100,000 red blood cells/mm3
• >500 white blood cells/mm3
• Bile, bacteria, or vegetable matter on micro-
scopic examination
• Amylase >175 IU/dL
Splenic Trauma 49

Management also be used to manage symptomatic biloma,

Physiological response of the child to trauma
remains the most important determinant for man-
agement. There is no need for emergent surgical
intervention in case of a hemodynamically stable
child. Primary survey with initial resuscitation
and careful management of fluid volume often
requiring blood transfusions are followed. The
child is carefully monitored under surgical super-
vision with strict bed rest to avoid risk of sudden
exsanguination. Serial hemoglobin monitoring
and repeat serial abdominal examinations are
done. The success rate of non-operative manage-
ment of pediatric liver injuries ranges from 85%
to 90%.
Operative Management
liver, and intra-abdominal abscesses.
Splenic Trauma
Grading of splenic trauma is done as per AAST
splenic injury scale (Table 8.2).
Management
A CT scan of the abdomen helps in grading the
trauma (Fig. 8.2). All grades of splenic injury
(Table 8.2) in children are now managed conser-
vatively which includes bed rest, monitoring of
vital signs, complete blood count every 4–6
hourly, restricted activity for 4 weeks, and

A hemodynamically unstable patient despite Table 8.2 Grading of splenic trauma

aggressive fluid resuscitations and blood trans- Grade Type Injury description
fusions or an associated hollow viscus injury I Hematoma Subcapsular, nonexpanding,
requires emergent exploratory laparotomy. <10% surface area
Large packs are place posteriorly, inferiorly, Laceration Capsular tear, nonbleeding,
<1 cm in parenchymal depth
and superiorly to control the bleeding and then
II Hematoma Subcapsular, nonexpanding,
removed slowly one by one to assess the damage.
10–50% surface area;
Pringle maneuver (occlusion of the porta between
intraparenchymal, nonexpanding,
the thumb and forefinger or using a vascular <5 cm in diameter
clamp) is done in case of uncontrolled hemor- Laceration Capsular tear, active bleeding,
rhage and limited visualization due to blood. The 1–3 cm depth which does not
following interventions could be done, depend- involve a trabecular vessel
ing on the degree of injury to the liver: III Hematoma Subcapsular, >50% surface area
or expanding; ruptured
subcapsular hematoma with
• Simple repair active bleeding;
• Exploration of expanding hematomas intraparenchymal hematoma
• Debridement of devitalized tissue >5 cm or expanding
• Rarely, lobectomy Laceration >3 cm depth or involving
trabecular vessels
IV Hematoma Ruptured intraparenchymal
 ole of Interventional Radiology
R hematoma with active bleeding
in Liver Injury Laceration Laceration involving segmental
or hilar vessels producing major
devascularization (>25% of
Angio-embolization has become the mainstay in spleen)
the management of liver injuries for hemody- V Laceration Completely shattered spleen
namically stable patients with active bleeding. Vascular Hilar vascular injury which
CT-guided percutaneous drainage procedures can devascularizes the spleen
50
Fig. 8.2 CT scan showing splenic laceration (blunt
abdominal trauma)
a­ voidance of contact sports for 3 months. Spleen
preservation remains the goal of the non-opera-
tive care to reduce postslenectomy infections.
Indications for Surgical Intervention
Patients who become hypotensive or tachycar-
diac and remain hemodynamically unstable even
after adequate resuscitation are considered to
have failed non-operative management. They can
be explored for splenectomy or splenic salvage.
Success of conservative treatment for splenic
injury in children exceeds 90%.
Renal Trauma
Renal trauma is usually associated with other
abdominal organ injury. Flank bruise, abdomi-
nal mass and tenderness, a palpable rib fracture,
and nature of injury (rapid deceleration injury)
point toward renal injury. Gross hematuria is
present in 75–90% cases of kidney injury; how-
ever, the degree of hematuria does not always
predict the severity of injury. The grading of
renal injury is done as per AAST kidney injury
scale (Table 8.3).
Investigations
Urine analysis is done to look for microscopic
hematuria, and >50 RBCs/hpf is considered
8 Abdominal Trauma
Table 8.3 Grading of renal injury
Grade Description
I Contusion Microscopic or gross
hematuria: urologic studies
normal
Hematoma Subcapsular, nonexpanding
without parenchymal laceration
II Hematoma Nonexpanding perirenal
hematoma confined to renal
retroperitoneum
Laceration <1 cm parenchymal depth of
renal cortex without urinary
extravasation
III Laceration >1 cm parenchymal depth of
renal cortex without urinary
extravasation or collecting
system rupture
IV Laceration Parenchymal laceration
extending through renal cortex,
medulla, and collecting system
Vascular Main renal artery or vein injury
with contained hemorrhage
V Laceration Completely shattered kidney
Vascular Avulsion of renal hilum, which
devascularizes kidney
s­ ignificant. Nowadays, rapid dipsticks are avail-
able to detect hematuria. Renal imaging is per-
formed in all patients with penetrating trauma
with entry or exit wounds in flank and a high
index of suspicion of renal injury.
Ultrasound is routinely performed but it has a
low sensitivity for the identification of retroperi-
toneal free fluid. Computed tomography (CT)
with intravenous contrast (IV) is the investigation
of choice (Fig. 8.3). The arterial and venous
phase can identify active extravasation, whereas
delayed phase images assess the renal collecting
system and ureteric continuity (Table 8.3).
Angiography can also be performed in cases of
delayed or ongoing hemorrhage, AV fistula or
pseudoaneurysm, and embolization/stenting in
the same sitting.
If the patient undergoes laparotomy without
having an initial CT scan and a renal injury is
noted, an intravenous pyelogram can be per-
formed at the time of surgery (using 2 mL/kg
intravenous contrast). Degree of renal injury as
well as function of the contralateral kidney can
be assessed.
Pancreatic Trauma
Fig. 8.3 CT scan, renal hematoma with splenic
laceration
Management
Non-operative management in a stable child
with renal injury has become the standard of
care. Bed rest, hydration, catheterization, antibi-
otics avoiding aminoglycosides, and serial vital,
hemoglobin and urine sample monitoring are
done routinely. Grade IV unstable vascular
injury may necessitate intervention. Large uri-
nomas require USG-guided drainage. Traditional
treatment in grade V injuries involves interven-
tion and possibly nephrectomy. However, even
successful conservative management of less
severe form of grade V injuries has also been
reported.
Indications for operative management in renal
trauma are life-threatening hemorrhage, renal
pedicle avulsion, major urinary leak, or pulsatile/
expanding retroperitoneal hematoma. Control of
vascular pedicle is the most essential step in renal
exploration.
51
Complications of Renal Trauma
• Urinary leak
• Delayed hemorrhage
• Devitalized renal segment
• Hypertension
• Renal insufficiency
• Infection
Pancreatic Trauma
Pancreatic injury, though rare, remains the most
common cause of acute pancreatitis and pancre-
atic pseudocyst in children. Blunt injury, typi-
cally a bicycle handle bar injury, is the most
common mechanism. Unlike adults, isolated pan-
creatic injury tends to be more common in chil-
dren. The injury may vary in severity from
contusions and minor lacerations to complete
transection of pancreatic duct. CECT abdomen
remains the most sensitive imaging modality to
pick up injuries to pancreas (Fig. 8.4). Raised
serum amylase and lipase also aid in diagnosis.
Management remains mostly conservative with
nil orally, bed rest, pain relief, TPN, IV fluids,
and IV antibiotics. Octreotide may help in reduc-
ing the pancreatic secretions post-injury, though
definite evidence about its role in trauma is lack-
ing. Feeds, if tolerated, can be initiated after
48 hours, once hollow viscus injury is ruled out.
Pancreatic secretions collected in retroperito-
C
Fig. 8.4 Traumatic transaction of the pancreas between
the pancreatic head and body (arrow) with large peripan-
creatic collection (C)
52
neum are allowed to form a pseudocyst, which
can be drained via gastro-cystostomy few weeks
later. Even complete transection of pancreatic
duct is conservatively managed though some pre-
fer stenting. Distal pancreatectomy, an extensive
procedure like Whipple’s operation, is not rou-
tinely performed in children with pancreatic
injury.
Intestinal Injuries
Intestinal injury should be suspected in all chil-
dren with blunt trauma to the abdomen present-
ing with signs of peritonitis such as abdominal
pain, rebound tenderness, guarding, rigidity, etc.
or hemodynamic instability (due to mesenteric
bleeding). Motor vehicle collision with a seatbelt
8 Abdominal Trauma
sign, i.e., contusion marked by seatbelt across the
abdomen, hints toward a hollow viscus injury. CT
abdomen may show pneumoperitoneum or
extravasation of intraluminal contrast.
Exploratory laparotomy is performed, thorough
wash is given, and resection of the unhealthy,
ischemic bowel with diverting stoma/proximal
stoma or an end-to-end anastomosis if edges are
healthy can be performed.
Suggested Reading
Wesson DE, Cooper A, Stylianos S, editors. Pediatric
trauma pathophysiology diagnosis and treatment. 1st
ed. New York, NY: Taylor & Francis; 2006.
Coran AG, Caldamone A, Scott Adzich N, Krummel TM,
Laberge JM, editors. Pediatric surgery. 7th ed. Oxford:
Elsevier; 2012.
 Part 3
Head, Neck and Spine
 Hydrocephalus
Hydrocephalus is defined as disproportionate
increase in the amount of cerebrospinal fluid
(CSF) in the cranium resulting from imbalance
between the CSF production, absorption, and
obstruction to its flow.
Physiology of CSF flow: Total amount of
CSF is 150 mL, daily production is 500 mL, and
circulation of CSF is 3–4 times/day. About 80%
of CSF produced from the choroid plexus and
rest from ventricular ependyma and brain
parenchyma.
Production: This is an active transport pro-
cess regulated by homeostatic environment and
alteration in CSF pressure (hydrostatic gradient
between CSF and venous sinuses).
CSF drainage: After being produced in the lat-
eral ventricle, CSF flows in a caudal direction, first
into the third ventricle through the foramen of
Monro (two on each side) then to the fourth ven-
tricle through aqueduct of Sylvius, and then exits
from the ventricles into the cortical and spinal sub-
arachnoid space via the foramen of Luschka (lat-
eral, two) and Magendie (medial) (Fig. 9.1a, b).
CSF then travels through the tentorial incisura,
passes over the hemispheric convexity, and is
absorbed in the venous system at the level of the
arachnoid villi. CSF absorption is pressure depen-
dent: hydrostatic gradient existing between the
CSF in subarachnoid space and the arachnoid villi
into the venous system. Small amount reabsorbed
via the sleeves of nerve roots. Head circumference
of a newborn baby is 35 cm, that of a 1-year-old is
45 cm, and an adult is about 55 cm. The posterior
© Springer Nature Singapore Pte Ltd. 2018
S. Roy Choudhury, Pediatric Surgery, https://doi.org/10.1007/978-981-10-6304-6_9
9
fontanelle closes within 2–3 months after birth,
whereas the anterior fontanelle closes between 9
and 24 (18) months after birth.
Incidence: 2–4 per 1000 population
Etiology
Imbalance in the CSF circulation due to:
• Overproduction: choroid plexus papilloma
• Decreased absorption: (communicating) at the
level of arachnoid villi
• Obstruction in the CSF flow pathway (obstruc-
tive/noncommunicating)
Types
Congenital
• Arnold-Chiari malformation with spina bifida
(Fig. 9.2)
• Dandy-Walker cyst: large fourth ventricle due to
obstruction of foramen of Luschka or Magendie
Acquired
• Brain tumors
• Premature babies: intracerebral and intraven-
tricular hemorrhage, infections
• Post-infective: pyogenic meningitis, tuberculosis
55
56 9 Hydrocephalus

a b
Interventricular foramen (Monroe)
Lateral ventricle
Lateral Ventricle
3rd ventricle

Cerebral aqueduct

Third Ventricle
Lateral exit foramen (Luschka)

4th ventricle
Fourth Ventricle
Medial exit foramen (Magendie)

Central canal

Fig. 9.1 Diagram of ventricles of the brain. (a) Coronal. (b) Lateral view

Lateral Choroid Subarachnoid Communicating (Nonobstructive)

ventricle plexus space
Arachnoid villi • Meningitis
Saggital sinus
• Subarachnoid hemorrhage

Types of Arnold-Chiari Malformation

Type Presentation
Third 1 Herniation of cerebellar tonsils
ventricle 2 Associated with meningomyelocele due to
Normal
Aqueduct of cerebellum herniation of vermis of cerebellum
sylvius 3 Associated with occipital encephalocele
White outline
Fourth
shows ACM1- 4 Lack of cerebellar development
ventricle
cerebellar tonsils
extending through
foramen magnum
Clinical Features
Spinal
nerves
• Increase head circumference, tense fontanelle,
Fig. 9.2 Arnold-Chiari malformation dilated scalp veins, sunset eye sign, Crack-pot
on percussion (Fig. 9.3)
Noncommunicating (Obstructive) • Children and adults: headache, nausea, vomit-
ing, somnolence, drowsiness, sixth nerve
• Aqueductal stenosis (the most common cause palsy, papilledema on fundoscopy, urinary
of congenital hydrocephalus) incontinence, gait disturbance, secondary
• Colloid cyst of the third ventricle endocrine features like diabetes insipidus,
• Ependymal cyst of the fourth ventricle visual impairment, developmental delay
• Craniopharyngioma
• Pineal, cerebellar tumor
Types 57

Fig. 9.3 Large head due to hydrocephalus

Fig. 9.4 CT scan showing hydrocephalus
Investigations
In case of acute rise in intracranial pressure
• Transcranial USG: Through open fontanelle, (headache, vomiting, drowsiness), urgent ­drainage
measure for ventricular dilatation; ventriculo-­ (tap) followed by shunt operation should be done;
parenchymal ratio more than 33% indicates otherwise there could be loss of vision, loss of brain
hydrocephalus. parenchyma, and loss of intellectual function.
• (One lateral ventricular dilatation indicates
obstruction of the foramen of Monro.)
• X-Ray: “Copper beating” of skull, erosion of Surgical Treatment
pituitary fossa.
• CT scan showing ventricular dilatation 1. Removal of obstructing lesion, e.g., tumor
(Fig. 9.4). 2. Decrease CSF production: ablation of the cho-
• Contrast MRI for detailed anatomy. roid plexus
3. Bypassing a block to normal CSF flow by
cannulation of aqueduct of Sylvius, third
Medical Treatment ventriculostomy, and ventriculo-cysternostomy
4. External drainage in case of infection
Acetazolamide (carbonic anhydrase inhibitor) 5. Internal drainage: shunt, most commonly per-
decreases CSF production by 10–15%. Dose is formed operation for hydrocephalus
10–20 mg/kg/day in three to four divided doses.
Frusemide and glycerol may also be used for
raised intracranial tension. Shunt Operations (Figs. 9.5 and 9.6)
Ventricular tap: done through the lateral
angle of anterior fontanelle in case of raised • Ventriculoperitoneal (VP) shunt (most com-
intracranial tension (ICT). monly done)
58 9 Hydrocephalus

• Ventriculoatrial (VA) shunt: from cerebral

Strata adjustable Ventricular ventricle to atrium through common facial or
shunt system catheter
internal jugular vein
Ventricles

Serious complications of VA shunt include

pulmonary embolism, cor pulmonale, septice-
mia, shunt nephritis (immune complex), cardiac
mural thrombi, and cardiac tamponade:

• Lumboperitoneal shunt: only in communicat-

ing type of hydrocephalus.
• Ventriculopleural shunt: for 6–7 years child
especially when VP or VA shunt cannot be
performed.
• Endoscopic third ventriculostomy (ETV) is
Distal catheter
getting popularity because it is done by mini-
mally invasive endoscopic technique and no
foreign material is inserted (Fig. 9.7). The
Peritoneal cavity floor of the third ventricle is opened to basal
cisterns for CSF drainage bypassing the
obstruction.

Fig. 9.5 VP shunt

Fig. 9.6 VP and

VA shunt
Suggested Reading 59

Ventriculoscope Other Causes of Large Head

Brain Bone • Achondroplasia

Right lateral • Metabolic disease
ventricle • Vitamin A toxicity
Foramen of
monroe
III* ventricle
Other Types of Hydrocephalus
Interpendicular
cistern • Normal pressure hydrocephalus due to demen-
Opening tia/Alzheimer’s disease
• Arrested hydrocephalus
• Hydrocephalus ex vacuo: due to cerebral
agenesis or decrease brain substance
Fig. 9.7 Neuroendoscopic third ventriculostomy • Porencephalic cyst

Shunt Malfunctions Fetal diagnosis and management of hydro-

cephalus are discussed in Chap. 4.
• Upper end block: abnormal position, blockage
by the choroid plexus
• Lower end block: peritonitis, pseudocyst Suggested Reading
formation
Kumar P, Burton BK. Congenital malformations evidence
• Slit valve malfunction based evaluation and management. 1st ed. New York,
• Migration of tip NY: McGraw Hill Medical; 2008.
• Overdrainage: low pressure symptoms, slit Coran AG, Caldamone A, Scott Adzich N, Krummel TM,
ventricle on CT scan Laberge JM, editors. Pediatric surgery. 7th ed. Oxford:
Elsevier; 2012.
• Infection: Staphylococcus epidermidis,
Staphylococcus aureus, Escherichia coli
• Shunt fracture especially at connectors
 Neural Tube Defects
10

Neural tube defects occur due to failure of the patch, pigmentation, fatty lump, and dermal
normal neural tube development. It encom- sinus. There may be associated intradural lesions
passes a wide spectrum of anomalies with a var- like lipoma, dermoid, epidermoid tumor, tether-
ied clinical outcome. Advances in fetal diagnosis ing of the cord with thickening of filum termi-
and possible therapy are currently under nale, and diastematomyelia (bony spur).
evaluation. Bony spina bifida is found in 10% of normal
population.
Incidence is 1–8/1000 population, 90% cases
Types are sporadic, and some are familial. Etiology is
multifactorial. Association of low folic acid
Open—the neural tube and its membranous cov- intake of mother with higher incidences of neural
erings are abnormal, and the overlying skin is tube defects in children has been established.
lacking. Examples are anencephaly (without
brain) and myeloschisis/rachischisis (most severe
form with exposed spinal cord and CSF leak). Embryology
Closed—defect as above but overlying skin is
intact. Examples are spina bifida occulta and Spina bifida results from failure of closure of
lipomeningomyelocele. neural tube. Spina bifida aperta usually results
Spina bifida aperta (open neural tube defect, from defects during primary neurulation, while
Fig. 10.1)—no skin cover, defect in the posterior spina bifida occulta results from defects during
vertebral arch, CSF leak with chance of meningi- secondary neurulation.
tis. Examples are syringocele (open spinal canal)
and rachischisis (open bony defect).
Spina bifida cystica (Fig. 10.2)—variant of Diagnosis
spina bifida aperta with a cystic swelling. It is
usually of two types: meningocele-CSF-­ Antenatal
contained sac without neural tissue and meningo-
myelocele (MMC when there is neural tissue • Neural tube defects can be diagnosed by pre-
within the sac). natal screening with ultrasonography and
Spina bifida occulta (Fig. 10.3a, b): This is maternal serum alpha-fetoprotein (AFP)
due to defect of the posterior vertebral arch, but level.
there is no herniation of neural tube. On the skin • Serum AFP has 75% accuracy in detecting
cover, various changes may be seen—like hairy open neural tube defects if gestational age is

© Springer Nature Singapore Pte Ltd. 2018 61

S. Roy Choudhury, Pediatric Surgery, https://doi.org/10.1007/978-981-10-6304-6_10
62 10 Neural Tube Defects

more than 15 weeks. Amniotic fluid AFP is

accurate in 98% cases of open defects.
• Fetal MRI.

Postnatal

• Most of the lesions are located in the lumbosa-

cral area followed by the thoracic spine
(Fig. 10.4). Other areas involved are frontal,
occipital, and nasoethmoidal (Figs. 10.5, 10.6,
Fig. 10.1 Open neural tube defect over lumbosacral area and 10.7) encephalocele.
• Among the spinal lesions, the higher the
lesion, the more serious the defect.
• Previous children with spina bifida have a
2.8% risk of recurrence in the sibling.
• Neurological deficits (both motor and sen-
sory) in association with lumbosacral. MMC
are bladder/bowel dysfunction, paraplegia,
talipes deformity, and trophic ulcers
(Fig. 10.8). These may not be apparent early
in life.
• Open lesions are associated with increased
risk of meningitis.
• Hydrocephalus is associated in 80% of cases.
This is due to herniation of vermis of the cer-
ebellum causing obstruction to CSF drainage
Fig. 10.2 Lumbosacral meningomyelocele (Arnold-Chiari malformation II).

a b

Fig. 10.3 (a, b) Spina bifida occulta with hairy patch

Treatment
Fig. 10.4 Dorsal meningomyelocele
Fig. 10.5 Frontal encephalocele
• Clinical examination should also include head
circumference measurement and detailed neu-
rological examination.
• Imaging is done with X-ray spine and MRI
spine (Figs. 10.9 and 10.10a, b).
Treatment
Prevention
Folic acid (0.4 mg/day) to expectant mothers has
been found to reduce the incidence of neural tube
63
Fig. 10.6 Occipital encephalocele
Fig. 10.7 Naso-ethmoidal encephalocele
defects. The dose needs to be increased to 4 mg per
day in women with history of neural tube defects.
Antenatal Diagnosis
and Management
Antenatal diagnosis allows for counseling of
family regarding the outcome and helps in appro-
priate decision-making.
Prospective randomized control trial for pre-
natal vs. postnatal surgical repair conducted in
the United States (MOMS trial) for open lesions
64 10 Neural Tube Defects

Fig. 10.8 Trophic ulcer in the buttock in a case of

meningomyelocele
Fig. 10.9 MRI spine, lumbosacral meningomyelocele

a b

Fig. 10.10 (a) Lipomeningocele. (b) MRI showing a lipomeningocele

with hindbrain herniation showed fetal repair of
spina bifida to be superior to postnatal repair in
terms of:
• Incidence of Chiari malformation was found
less in the fetal repair group.
• Need for ventricular shunt was less in the fetal
repair group.
• Motor skills were better in the fetal repair group.
Postnatal management: Early surgical clo-
sure is required to prevent further neurologi-
cal damage.
Surgical procedure is excision and repair of
the defect (Fig. 10.11). Essential steps of the
operation are elliptical skin incision at the junc-
tion of the sac and the normal skin and excision
of the membranous sac with preservation of the
neural plaque and other nerve roots. Mobilization
Suggested Reading
a b
Fig. 10.11 (a) Lumber MMC. (b) After surgical excision and repair
is followed by closure of the dura mater, and
approximation of the fascia is followed by skin
closure. Tethered cord requires untethering of the
cord with laminectomy, removal of lipomatous
mass, diastematomyelia (bony spur), dermal
sinus and neurenteric cyst, release of conus, and
sectioning of filum terminale. Patulous but water-
tight closure of the dura is performed followed by
approximation of muscles, fascia, and the skin.
Spina bifida occulta with tethered cord should
undergo untethering of the cord to prevent further
neurological damage.
Associated hydrocephalus may require ventric-
uloperitoneal shunt or other drainage procedures.
Postoperative Complications
• Wound disruption
• CSF leak which is treated with prone head-­
down position and oral acetazolamide (Diamox)
and may need insertion of a VP shunt
• Tethered cord
Tethered Cord
The lower tip of the spinal cord normally lies
opposite the disk between the 1st and 2nd lum-
ber vertebra. In spina bifida patients (meningo-
myelocele, lipomyelomeningocele), the cord is
65
adhered to the overlying skin and lies lower
than normal (low-lying cord). Those with pre-
viously repaired defects, with the growth of the
child, the tethered or fixed cord can get stretched
causing further damage and interfering with
vascular supply. The other causes of tethered
cord are:
• Dermal sinus tract
• Diastematomyelia
• Lipoma
• Tumor
• Thickened/tight filum terminale (a delicate
filament near the tailbone)
• Spine trauma/spinal surgery
Treatment
Surgical treatment of un-tethering of the cord by
freeing it from all the surrounding adhesions is
required for symptomatic patients. A laminec-
tomy may be necessary to accomplish the proce-
dure. A thickened filum terminale may require
division.
Suggested Reading
Coran AG, Caldamone A, Scott Adzich N, Krummel TM,
Laberge JM, editors. Pediatric surgery. 7th ed. Oxford:
Elsevier; 2012.
 Cleft Lip and Cleft Palate
11

Cleft lip and palate are the most common con-
genital anomaly of the orofacial structures. They
are commonly associated with other congenital
anomalies like congenital heart disease. Thorough
assessment is necessary to exclude other congen-
ital anomalies.
Incidence
affected (1 in 25 live births). Maternal epilepsy
and drugs like steroids, diazepam, and phenytoin
are associated with tenfold increase in
incidence.
Isolated cleft palate is more commonly associ-
ated with syndromes (in up to 50% cases) like
Stickler (ophthalmic and musculoskeletal abnor-
malities), Down, Apert, and Treacher Collins
syndrome (Fig. 11.1).

Cleft lip with cleft palate occurs in 1 in 600 live

births. Isolated cleft palate occurs in 1 in 1000
live births.

Relative Incidence

Cleft lip alone: 15%.

Cleft lip with palate: 45%.
Isolated cleft palate: 40%.
Cleft lip and with cleft palate is common in
males.
Isolated cleft palate is common in females.
Among unilateral cleft lip, 60% are left sided.

Etiology

Both genetic and environmental factors play role Fig. 11.1 Treacher Collin syndrome (micrognathia,
in the etiopathogenesis. Positive family history is underdeveloped zygoma, malformed ears, downward
a high-risk factor for subsequent children to be slanting eyes)

© Springer Nature Singapore Pte Ltd. 2018 67

S. Roy Choudhury, Pediatric Surgery, https://doi.org/10.1007/978-981-10-6304-6_11
68 11 Cleft Lip and Cleft Palate

a b

Fig. 11.2 (a) Unilateral incomplete cleft lip with nasal cleft and (b) bilateral cleft lip

Classification

Cleft Lip (Fig. 11.2a, b):

• Unilateral.
• Bilateral.
• Incomplete—skin web is present across the
floor of nostril (Simonart’s band).
• Complete—cleft extends into the nasal cavity
and usually involves the alveolus.

Cleft palate (Fig. 11.3):

Fig. 11.3 Cleft of secondary palate

• Cleft of primary palate (anterior to incisive
foramen)
Embryology • Cleft of secondary palate (from incisive fora-
men to uvula)
Cleft lip is due to failure of median nasal process –– Complete
either to contact or maintain contact with lateral –– Incomplete
nasal and maxillary process. –– Submucous cleft palate
Cleft palate is due to failure of fusion of the
two palatine processes. Bilateral cleft lip and palate (Fig. 11.4)
Management
Fig. 11.4 Bilateral cleft lip with cleft palate
Antenatal Diagnosis
All defects but isolated palate can be antena-
tally diagnosed after 16 weeks of pregnancy.
Parents will need counseling and support.
Isolated and non-syndromic defects have excel-
lent outcome.
Management
Feeding
Most of the babies feed well and thrive. Some
babies need feeding assistance (modified teats,
bottles, obturator, etc.). Feeding with spoon in
upright position is recommended. Pierre Robin
syndrome consists of isolated cleft palate, ret-
rognathia, and glossoptosis (posteriorly dis-
placed tongue) (Fig. 11.5). They have early
respiratory and feeding difficulties. Airway
problem in Pierre Robin syndrome may be life
threatening.
69
Fig. 11.5 Pierre Robin syndrome
Management of Respiratory Problems
• The following steps may be necessary depend-
ing on the severity of the condition:
• Nursing the baby prone
• Nasogastric feeding
• Nasopharyngeal airway tube
• Intubation of the airway
• Tongue Lip Adhesion (Labioglossoplasty):
Surgical fixation of tongue to lower lip in
order to prevent tongue from falling back
Presurgical prosthetics like nasoalveolar mold
may be needed for severe lip defects to align the
alveolus and premaxilla into a more anatomic
position.
70
Principles of Surgery
The goal is to achieve normal appearance of the lip,
nose, and face with normal speech and dentition:
1. To restore normal anatomy of facial muscles
and skeleton—tissues that are displaced,
deformed, and underdeveloped (rather than
true hypoplasia).
2. Emphasis is placed on muscular reconstruc-
tion of the lip, nose, face, and muscles of soft
palate—normal (or near normal) anatomy
promotes normal function thereby encourag-
ing normal growth and development of the lip,
nose, palate, and facial skeleton.
An in-depth understanding of the anatomy is
invaluable.
Anatomy
Cleft lip
• Cleft lip is associated with disruption of the
muscles of the upper lip and nasolabial region
groups (rings of Delaire).
• Unilateral cleft lip is associated with nasal
deformity, lip retraction, changes in vermil-
lion, and lip mucosa.
• Bilateral cleft lip is associated with flaring of
the nose, protrusion of the premaxilla, and
area of skin prolabium (devoid of muscles).
• Incomplete cleft lip is associated with pres-
ence of nasal sill (Simonart’s band).
• Complete cleft lip has absence of nasal sill
(Simonart’s band).
Cleft palate
• Primary palate is the area anterior to the inci-
sive foramen. Cleft of the primary palate is
associated with cleft lip and alveolus.
• Secondary palate is the area posterior to the
incisive foramen, and its cleft can be isolated
without associated cleft lip.
• Submucous cleft palate is present if an appar-
ent intact palate is associated with muscular
11 Cleft Lip and Cleft Palate
diastasis of velum, bifid uvula, and posterior
notching of the hard palate.
Timing of Surgery
Cleft lip is operated between 3 and 6 months of
age. A rule of 10 as applied includes at least 10
weeks of age, 10 pounds of body weight and
10 gm% of hemoglobin.
Cleft palate surgery is done between 6 and 18
months of age.
Commonly Practiced Procedures
For cleft lip repair: the commonly practiced pro-
cedure is the Millard rotation advancement oper-
ation. An alternative procedure for wide open
defect is Tennison-Randall technique.
For cleft palate repair: Bardach two-flap pala-
toplasty (45%) and Furlow palatoplasty (42%)
are the most commonly used procedure followed
by Veau-­Wardill-­Kilner push back V-Y
palatoplasty.
Principles of Surgery
1. Cleft lip surgery attaches and reconstructs the
muscles around the nasal aperture and oral
sphincter (normal appearance of the lip-­
vermillion border, Cupid’s bow, nose).
Rhinoplasty i.e. reconstruction of the nasal
deformity forms an integral part. It may be
done at the same stage of lip repair or it can be
planned later.
2. Cleft palate surgery brings together muscles and
lengthens the soft palate including uvuloplasty
with minimum scarring. A significant proportion
of these patients need pharyngoplasty later for
residual velopharyngeal incompetence.
Secondary Management
Cleft lip-revision surgery may be required for
residual notching and rhinoplasty.
Suggested Reading
Cleft Palate-Hearing, Speech and Maxillofacial
growth
Due to high incidence of sensorineural and
conductive hearing loss, they require regular
hearing tests for proper speech development.
Management for speech and dental and
facial growth requires multidisciplinary team
approach. Palatal fistula after primary repair
needs closure later on.
Dental and orthodontic management with
alveolar bone graft at 5–10 years of age (elimi-
nates oronasal fistula, promotes normal eruption
of canine and other teeth) followed by secondary
71
revision surgery usually at teenage may be
required.
Suggested Reading
Gupta DK, Sharma S, Azizkhan RG, editors. Pediatric
surgery—diagnosis and management. 1st ed. Jaypee
Brothers: New Delhi; 2009.
Kumar P, Burton BK. Congenital malformations evidence
based evaluation and management. 1st ed. New York:
McGraw Hill Medical; 2008.
Williams NS, Bulstrode CJK, O’Connell PR, editors.
Bailey & Love’s short practice of pediatric surgery.
26th ed. Taylor & Francis Group; 2013
 Neck Cysts, Sinuses,
Lymphadenopathy, and Torticollis
Neck lesions in children can be congenital or
acquired in origin. Accurate diagnosis aids in the
management of these lesions. Thyroglossal cyst
and sinuses are the most common congenital
neck lesion followed by branchial anomalies.
Branchial Anomalies
Embryology
Neck cysts and sinuses in children are usually
congenital in origin. There are six branchial
arches in the human embryo. Each branchial arch
is covered externally with ectoderm, lined inter-
nally with endoderm, and filled with mesoderm,
containing an artery, a nerve, a cartilage rod, and
a muscle. The depressions between the arches are
called clefts externally and pouches internally.
The clefts are lined with ectoderm and the
pouches with endoderm. The cysts and sinuses of
the neck are related to the embryonic process of
fusion, closure, and development of neck struc-
tures (Table 12.1).
Branchial clefts and arches are formed in the
7th week of intrauterine life. The first cleft
becomes external auditory meatus. The 2nd–4th
are normally covered as the 2nd arch overlaps
the others to fuse with the 5th arch thus closing
an ectodermal area superficial to the sternomas-
toid muscle called the cervical sinus of His.
© Springer Nature Singapore Pte Ltd. 2018
S. Roy Choudhury, Pediatric Surgery, https://doi.org/10.1007/978-981-10-6304-6_12
12
Branchial Cyst
It develops from the vestigial remnant of the 2nd
branchial cleft. It contains fluid and toothpaste-­
like material. Branchial cysts usually present in
adult age group.
Pathology
The lining of the cyst wall is by squamous epithe-
lium; the cyst wall is surrounded by lymphoid tis-
sue which may cause recurrent inflammation.
Aspirate fluid from the cyst contains cholesterol
crystals.
Clinical Features
 ite
S
The most common location of the branchial cyst
is under the upper one third of sternocleidomas-
toid muscle (Fig. 12.1), and it may show positive
transillumination.
Treatment is by surgical excision (see below).
Branchial Fistula
This presentation is common in pediatric age
group.
73
74 12 Neck Cysts, Sinuses, Lymphadenopathy, and Torticollis

Table 12.1 Derivatives of branchial arches, clefts, and

pouches
First arch
Cartilage (Meckel’s cartilage): Incus body, malleus,
sphenomandibular ligament, ingula of mandible
muscles (muscle of mastication, tensor tympani, tensor
veli palatini, mylohyoid, and anterior betty of
digastric)
External maxillary artery
Nerve Vth cranial
Cleft: external auditory canal
Pouch: Eustachian tube, middle ear cavity, mastoid air
cells
Second arch
Cartilage: stapes, styloid process, stylohyoid ligament, Fig. 12.1 Branchial cyst
hyoid (lesser horn and part of the body)
Muscles: muscles of facial expression, stapedius,
stylohyoid, and posterior belly of digastric Secondary: Following incising of an infected
Stapedial artery branchial cyst.
Nerve VII, pouch: palatine tonsil, supratonsillar
fossa
Third arch Clinical Features
Cartilage: hyoid (greater horn and part of the body)
Muscles: stylopharyngeus The site of external opening of the branchial fis-
Internal carotid artery tula is at the anterior border of lower one third of
Nerve IX sternomastoid muscle (Fig. 12.2a, b).
Pouch: thymus, inferior parathyroid The site of internal opening is in the pharyn-
Fourth arch geal wall near the posterior tonsillar fossa. The
Cartilage: thyroid cartilage, cuneiform cartilage, part tract may end blindly and thus actually becomes
of epiglottis a sinus rather than a fistula. Although the 3rd and
Muscles: constrictors of pharynx, cricothyroid, levator
4th arch abnormalities are rare, but when present,
veli palatini
the internal opening is at the level of pyriform
Arch of aorta (L), part of subclavian artery (R)
Nerve X
fossa. The opening discharges mucous or muco-
Pouch: superior parathyroid
pus that can be expressed from the tract.
Fifth arch Histology of the lining is by ciliated columnar
Pouch: ultimobranchial body (lateral anlage of thyroid epithelium and the wall contains muscles.
gland)
Sixth arch
Cartilage: cricoid, arytenoid, corniculate Treatment
Muscles: intrinsic muscles of larynx
Pulmonary artery and ductus arteriosus (L) Surgical Excision
Nerve X (recurrent laryngeal) Initial incision is taken around the fistula open-
ing. Dissection of the fistula track is continued
upward which may require one or two additional
Etiology incisions in a step-ladder pattern (Fig. 12.3a, b).
The course of the track may be probed through
Congenital: Due to persistence of the 2nd bran- the opening or delineated by injecting methylene
chial cleft, the occluding membrane of which has blue through the opening. The tract passes
broken down (persistence of cervical sinus of through the fork of the common carotid artery,
His). superficial to the hypoglossal and
Thyroglossal Cyst and Fistula 75

a b

Fig. 12.2 (a, b) Branchial fistula; note the site at lower one third of anterior border of sternomastoid muscle

a b

Fig. 12.3 Surgical excision of branchial fistula. (a) Lower end. (b) Step-ladder incision for gaining access to the upper end

g­ lossopharyngeal nerves but deep to the posterior from the foramen cecum of the tongue.
belly of digastric muscle. Persistence of this median thyroid anlage results
in thyroglossal cyst.

Thyroglossal Cyst and Fistula

Embryology
Thyroid gland develops from the median bud of
the pharynx (thyroglossal duct) which descends
Clinical Features
Thyroglossal cyst is congenital in origin; how-
ever, thyroglossal fistula is always acquired due
to infection or incision of cyst. The lining is
76 12
columnar epithelium, discharges mucous, and
causes recurrent attacks of inflammation. A thy-
roglossal cyst (Fig. 12.4) moves with swallowing
and protrusion of the tongue, and the cyst wall
contains lymphoid tissue.
In 1–2% of cases, the median ectopic thyroid
tissue may present as thyroglossal cyst.
Therefore, patients need to be screened with
thyroid-­stimulating hormone (TSH) level and/
or ultrasonography of the neck. Presence of a
solid swelling with lack of visualization of nor-
mal thyroid gland and elevated TSH warrants
further evaluation with thyroid scan. If the
swelling is indeed confirmed to be the only
functioning thyroid tissue, then only thyroid
hormone supplementation will cause regression
of the swelling.
Differential Diagnosis
• Dermoid cyst
• Lymph node swelling
• Hemangioma/vascular malformation
• Median ectopic thyroid
• Sublingual bursae
Fig. 12.4 Thyroglossal cyst
Neck Cysts, Sinuses, Lymphadenopathy, and Torticollis
Treatment
Surgical excision (Sistrunk’s operation) includes
removal of the body of the hyoid bone (as the
track has a close relation with the hyoid bone)
and a central core of muscles of the tongue (duct
above the hyoid bone spreads into many ductuli
as it approaches the foramen cecum) to avoid any
recurrence. A finger in the patient’s mouth by an
assistant pushing the tongue down aids in the
dissection.
 ystic Hygroma (Lymphatic
C
Malformation) (See Chap. 6)
Cervical Lymphadenopathy
Cervical lymphadenopathy is a matter of concern
for the parents and the physician. The causes may
be due to various inflammatory or neoplastic
conditions.
Acute lymphadenitis presents with fever, mal-
aise, and tender neck mass of short duration.
Primary focus of infection can be tonsillitis or
dental abscess. Initial management is with antibi-
otics and anti-inflammatory antipyretic drugs. If
suppuration occurs, then incision and drainage
become necessary.
Asymptomatic enlargement of superficial pos-
terior cervical lymph nodes is the most common
presentation in outdoor patients (Fig. 12.5).
Although no cause is often found, it is generally
attributed to scalp infection, and on fine-needle
aspiration cytology, nonspecific adenitis is often
reported. Management consists of treatment of
any local infection if present, reassurance to the
parents, and head shampoo to treat any scalp
lesion.
Chronic painless lymphadenopathy may be
due to tuberculosis (Fig. 12.6a, b) or neoplasm
like lymphoma in children (Fig. 12.7a, b). The
deep cervical groups of nodes are generally
involved. In tubercular infection the lymph nodes
may be adhered or matted together. Suppuration
of such lymph node mass gives rise to cold
Cystic Hygroma (Lymphatic Malformation)
Fig. 12.5 Posterior cervical lymph node (reactive)
abscess in the neck. Penetration of abscess
through the deep fascia can give rise to e­ xpanding
abscess beneath the superficial fascia called “col-
lar stud” abscess. Incision and drainage of cold
abscess should be avoided to prevent nonhealing
sinus. Antigravity needle aspiration of pus can be
performed for the diagnosis. Rupture of such an
abscess results in a chronic discharging sinus in
the neck (Fig. 12.6b). Large lymph nodal mass
due to Hodgkin lymphoma may cause “bull
neck” appearance (Fig. 12.7a).
a b
Fig. 12.6 Cervical
tuberculous
lymphadenitis (note
multiple large matted
nodes). (b) Multiple
tubercular sinus neck
77
Management includes investigation with com-
plete blood count, ESR, chest X-ray, Mantoux
test, and fine-needle aspiration cytology to clinch
the diagnosis. Those with indeterminate lesion
may need surgical excision biopsy. Tubercular
lesions are treated with antitubercular therapy.
Infection due to atypical mycobacterium and
drug-resistant tubercular lesions may require
therapeutic surgical excision.
Sternomastoid tumor (Fig. 12.8a, b) generally
presents in the first few weeks of life with a firm
mass of 1–3 cm in size in the substance of the
sternomastoid muscle. Etiology is uncertain;
trauma during delivery with hematoma within the
sternomastoid muscle is a probable cause.
Management is conservative with gentle neck
exercise and reassurance to the family. The lesion
usually resolves within a few weeks.
Congenital Torticollis
Fibrosis and contracture of the sternomastoid
muscle lead to torticollis (Fig. 12.9a, b) and
­tilting of the head to the opposite side. The asso-
ciated problems are facial asymmetry (hemihy-
poplasia), scoliosis, craniofacial deformity, and
atrophy of the ipsilateral trapezius muscle in
uncorrected cases. Ophthalmic checkup to rule
out squint and cervical spine X-ray to rule our
bony vertebral anomaly are mandatory. Treatment
78 12 Neck Cysts, Sinuses, Lymphadenopathy, and Torticollis

a b

Fig. 12.7 (a) Bull neck due to Hodgkin lymphoma. (b) Acute myeloid leukemia

a b

Fig. 12.8 (a, b) Sternomastoid tumor

Suggested Reading
a b
Fig. 12.9 (a, b) Torticollis
should initially be conservative with neck
physiotherapy.
Indications for Surgery
• Persistent deformity limiting head rotation
after failed conservative treatment
• Facial asymmetry and hemifacial hypoplasia
• Older patients above 1 year of age
Surgery: An incision of 3–4 cm is given in
the skin crease 1 cm above the clavicle to divide
the sternal and clavicular heads of the sterno-
mastoid muscle. The muscle is best divided at
its lower third. The spinal accessory nerve and
other vascular structures are protected. The
79
thickened investing cervical fascia is also
divided from the midline extending posteriorly
up to the anterior border of the trapezius muscle.
The procedure can be done by endoscopic tenot-
omy through an axillary incision (stealth
surgery).
Suggested Reading
Coran AG, Caldamone A, Scott Adzich N, Krummel TM,
Laberge JM, editors. Pediatric surgery. 7th ed. Oxford:
Elsevier Limited; 2012.
Holcomb GW III, Murphy PJ, Ostile DJ, editors.
Ashcraft’s pediatric surgery. 6th ed. Philadelphia, PA:
Saunders/Elsevier; 2014.
Part 4
Thorax
 Chest Wall Deformity
13

Chest wall deformities can cause functional and/

or cosmetic problems in children. Chest wall
deformities are common, occurring in about in
1 in 1000 people, with a male-to-female ratio of
5:1. It can be classified as follows:

• Pectus excavatum
• Pectus carinatum
• Sternal defects
• Poland syndrome
• Diffuse skeletal disorders with thoracic
involvement

Of these, the excavatum deformity is the most

common and will be described in detail in this
chapter.

Pectus Excavatum Fig. 13.1 Pectus excavatum

It is a congenital chest wall disorder wherein

there is a depression of the sternum and the lower
costal cartilages, hence the chest appears funnel
shaped (Fig. 13.1). The defect is often asymmet-
ric, and there may be costal bulge where the ribs
dip posteriorly to meet the depressed sternum.
It is the most common chest wall deformity with
marked male predominance. A family history is
present in nearly one third, and associated scoliosis
is present in one fourth. The etiology is not clear,
but mechanical factors, inherent defect in the cos-
tochondral cartilage, and genetic causes have been
implicated in the pathogenesis of this deformity.
Though it is usually well tolerated in infancy,
many patients experience precordial pain with
exertion or even pain at the site of deformity. Others
experience palpitations and syncope, frequent
chest infections, and asthma-like symptoms.
These patients are investigated with the fol-
lowing investigations:
• Chest radiograph.
• CT scan will show the severity of defect and the
compression of the heart. Also it is helpful in
calculating indices, which may be helpful in

© Springer Nature Singapore Pte Ltd. 2018 83

S. Roy Choudhury, Pediatric Surgery, https://doi.org/10.1007/978-981-10-6304-6_13
84
B
A Open surgical repair consists of modifications
Haller index - A/B
Fig. 13.2 Haller index
treatment planning. Haller index is the most
commonly used index (Fig. 13.2). It is calcu-
lated as transverse diameter of the thoracic cav-
ity divided by the distance between the sternum
and the vertebrae at the level of maximal defect.
• Echocardiography: the stroke volume is
decreased. Echo will also demonstrate mitral
valve prolapse.
• Pulmonary function test: all parameters are
decreased to about 80% of the normal pre-
dicted values.
Patients with mild to moderate deformity are
started on a deep breathing, aerobic exercise, and
a posture program and evaluated yearly. Patients
with severe chest deformities are evaluated fur-
ther with CT of the chest and cardiac and pulmo-
nary function test. Surgery is indicated if two or
more of the following criteria are positive:
• Evidence of cardiac or pulmonary compres-
sion on CT and Haller index > 3.25.
• Cardiac evaluation demonstrating displace-
ment/compression/murmur/conduction
defects/mitral valve prolapse.
• Pulmonary function test demonstrates any
sign of obstructive/restrictive lung disease.
• Progressive and symptomatic deformity.
13 Chest Wall Deformity
• Recurrent deformities following a failed
repair.
The ideal age for surgery is just before puberty.
of the older Ravitch procedure. The open proce-
dure has the following steps:
• Transverse or chevron incision on the chest
• Reflection of the pectoral and abdominal wall
muscle of the sternum and the deformed ribs
till the costochondral junction
• Separation of the perichondrium of the
deformed ribs
• Excision of the deformed ribs from the ster-
num to few millimeters proximal to the costo-
chondral junction
• Anterior osteotomy of the sternum and frac-
ture of the posterior table of sternum
• The use of strut to stabilize the sternum in the
anterior position
• Closure of muscles and superficial layers
The newer minimally invasive technique
(Nuss procedure) involves inserting one or two
curved steel bars under the sternum in order to
brace the anterior chest under thoracoscopic
guidance. The bar is inserted under the sternum
and is rotated 180° to elevate the sternum. The
bar is subsequently removed after 2–4 years.
Pectus Carinatum
It is abnormal protrusion of the sternum and the
costal cartilages. The treatment if needed is simi-
lar to that in pectus excavatum with excision of
deformed cartilage and osteotomy of the ster-
num, placing it in a normal position.
Suggested Reading
Poland Syndrome
The most consistent feature of this syndrome is
the absent or attenuated sternocostal component
of the pectoralis major muscle. There is associ-
ated variable involvement of the hand, breast, or
chest wall (ribs).
Sternal Defects
Include defects like ectopia cordis, cleft/bifid
sternum, etc.
85
 iffuse Skeletal Disorders
D
with Thoracic Involvement
It includes the syndromes like asphyxiating tho-
racic dystrophy, spondylothoracic dysplasia, etc.
Suggested Reading
Coran AG, Caldamone A, Scott Adzich N, Krummel TM,
Laberge JM, editors. Pediatric surgery. 7th ed. Oxford:
Elsevier; 2012.
Holcomb GW III, Murphy PJ, Ostile DJ, editors.
Ashcraft’s pediatric surgery. 6th ed. Philadelphia, PA:
Saunders/Elsevier; 2014.
 Mediastinal Masses in Children
Mediastinal masses often pose a diagnostic chal-
lenge to the clinician. The mediastinum is ana-
tomically divided into superior, anterior, middle,
and posterior compartments (Fig. 14.1).
Cysts and Tumors of the
Mediastinum in Children
1. Anterior mediastinum (Figs. 14.2, 14.3, and
14.4):
Lymphomas
Teratoma
Germ cell tumor
Cystic hygroma
Thymic masses
2. Middle mediastinum (Figs. 14.5 and 14.6):
Vascular lesions
Lymphadenopathy
Pericardial cysts and tumors
3. Posterior mediastinum (Figs. 14.7 and 14.8):
Neurogenic tumors (most common)
Neurofibroma, neurofibrosarcoma, neuroblas-
toma, ganglioneuroma, ganglioneuroblas-
toma, pheochromocytoma, paraganglioma
Foregut duplication cysts
Lymphoma is the most common anterior
mediastinal mass in children followed by germ
cell tumors like teratoma and dermoid. In infants,
thymic masses are also common.
© Springer Nature Singapore Pte Ltd. 2018
S. Roy Choudhury, Pediatric Surgery, https://doi.org/10.1007/978-981-10-6304-6_14
14
Neurogenic tumors are the most common mass
in the posterior mediastinum. Neuroblastoma is
common in young children. Ganglioneuroma is
common in older children. Approximately 50% of
mediastinal masses are malignant in children
compared to 15% in adults.
Clinical Presentation
Antenatal detection of some lesions is possible
with fetal ultrasound.
Anterior/middle mediastinal masses: Nearly
half of these children are asymptomatic, and the
diagnosis is incidental upon imaging for nonspe-
cific symptoms. Anterior and middle mediastinal
masses in infants present with respiratory dis-
tress, stridor, and cyanosis. Older children pres-
ent with cough, dyspnea, orthopnea, and rarely
hemoptysis. Few children may present with supe-
rior vena cava syndrome (Fig. 14.9). Infants may
also present with sternal bulging especially if a
large mass like teratoma is present.
Posterior mediastinal masses can be quite large
yet asymptomatic, often discovered in a chest
radiograph done for other indications. Some chil-
dren may present with dyspnea, cough, dysphagia,
pain, or respiratory distress secondary to compres-
sion by the mass. If the tumor extends through
the spinal foramina, then features of spinal cord
compression may be present. Horner’s syndrome
(miosis, ptosis, anhydrosis) can be present if there
87
88 14 Mediastinal Masses in Children

Fig. 14.1 Anatomical

divisions of the
mediastinum
Trachea

Superior

Anterior

Middle

Posterior

is involvement of sympathetic ganglia chain. Few

cases of neuroblastoma can present with paraneo-
plastic syndrome (opsoclonus-­myoclonus, hyper-
calcemia, etc.).

Evaluation/Imaging

• Chest X-Ray can reveal widening of mediasti-

num with deviation of trachea or bronchus.
Fig. 14.2 Anterior mediastinal mass (acute lymphatic
Calcification may be noted in teratoma or neu-
leukemia) roblastoma. Bony destruction can be seen in
Evaluation/Imaging 89

a b

Fig. 14.3 Mediastinal mass with chest wall extension, acute lymphatic leukemia, (a) clinical, (b) CT scan

Fig. 14.4 CT scan – thymolipoma

Fig. 14.6 CT scan of the chest showing enlarged lymph

Fig. 14.5 Lymphangioma in the neck with mediastinal nodes in the (a) pretracheal, (b) subcarinal, and right para-
extension tracheal region (Hodgkin lymphoma)
90 14 Mediastinal Masses in Children

Fig. 14.7 Posterior mediastinal mass (neurogenic tumor)

Fig. 14.9 Mediastinal mass causing respiratory distress

with features of superior vena cava obstruction

Fig. 14.8 (a, b) CT scan showing esophageal duplication

cyst
Suggested Reading
malignant infiltrative tumors. Vertebral abnor-
malities may suggest foregut duplication cyst.
• Ultrasound is useful in detecting nature of the
mass (solid or cystic) and vascularity of the
lesion.
• Contrast-enhanced computed tomography
(CECT) is the investigation of choice which
gives the important information about the
lesion like extent, organ of origin, calcifica-
tion, and lymphadenopathy.
• MRI is more useful to see the extent of vascu-
lar lesions.
• Image-guided fine-needle aspiration cytology
(FNAC) or biopsy is helpful for tissue diagnosis.
• PET scan is useful to determine the extent of
disease and metastasis from various primary
tumors.
• Tumor markers (alpha-fetoprotein, beta HCG,
VMA, HVA, etc.) help in supporting the
diagnosis.
Management
Management depends on the type of the lesion and
91
symptoms. Malignant germ cell tumors and neu-
roblastomas require multimodal management
which may include surgical excision, c­ hemotherapy
and radiotherapy. Foregut duplications, teratomas
and some of the thymic masses are best treated
with surgical removal; lymphomas are usually
treated with chemotherapy. Symptomatic lymph-
angiomas or hemangiomas may occasionally
require surgical resection. Metastatic lesions are
generally treated with appropriate chemotherapy.
Suggested Reading
Coran AG, Caldamone A, Scott Adzich N, Krummel TM,
Laberge JM, editors. Pediatric surgery. 7th ed. Oxford:
Elsevier; 2012.
 Empyema Thoracis
Empyema thoracis is defined as accumulation of
pus in the pleural cavity. Management of pleural
empyema depends on the clinical condition of the
patient and stage of the disease.
Pathology
This generally follows acute bacterial or less
commonly viral pneumonias. Other relatively
less common causes of empyema include:
• Penetrating thoracic trauma
• Esophageal perforations due to iatrogenic
injury or corrosive/ foreign body ingestion
• Infection following thoracotomy
• Extension of subdiaphragmatic or paraverte-
bral abscess
Predisposing conditions are chronic pulmo-
nary diseases, diabetes mellitus, long-term ste-
roid therapy, immune suppression, congenital
heart disease, and recurrent aspirations.
In nearly half of the cases of pneumonia, there
is formation of excess pulmonary interstitial fluid
which transudates across the visceral pleura and is
sterile. This is known as a parapneumonic effu-
sion (PPE). This fluid is sterile and can be man-
aged with antibiotics without any need of
drainage. Once this fluid gets infected, it leads to
complicated parapneumonic effusion and pro-
gresses to frank pus formation in the pleural cav-
ity i.e., empyema. Once the fluid gets infected, it
© Springer Nature Singapore Pte Ltd. 2018
S. Roy Choudhury, Pediatric Surgery, https://doi.org/10.1007/978-981-10-6304-6_15
15
needs drainage by either insertion of chest tube or
in selected cases by thoracocentesis. The differen-
tiation between simple PPE and complicated PPE
depends on analysis (Lights criteria) of the fluid
to determine whether it is a transudate or exudate
(protein levels) and to see features suggestive of
bacterial infection (low pH, less glucose, increased
LDH, or positive Gram staining).
There are three recognized stages of the disease:
Stage I Exudative phase. Bacterial infection
of the parapneumonic effusion leads to the exu-
dative phase of the empyema. This stage lasts for
24–72 h and then progresses to the next stage.
Stage II Fibrinopurulent phase. A pyogenic
membrane is formed; fibrin strands form septa-
tions and loculations. This is the most common
pathological form encountered in children. This
stage lasts for 7–10 days and requires more
aggressive treatment like intercostal tube drain-
age (ICD).
Stage III Organization phase. A fibrous rind
(thick pleural peel) encases the collapsed lung
and prevents it from re-expanding. This stage
usually occurs within 2–4 weeks after initial pre-
sentation (Fig. 15.1a, b). Treatment often requires
surgical intervention.
Microbiology
Staphylococcus aureus being the predominant
pathogen in the developing countries. Other organ-
isms being Streptococcus pneumoniae, various
93
94 15 Empyema Thoracis

a b

Fig. 15.1 CT scan of the chest, organized empyema with (a) thick pleural peel and (b) collapsed lung

gram-negative organisms (e.g., Enterobacteriaceae empyema classically shows a predominance of

such as Klebsiella and Pseudomonas aeruginosa),
Bacteroides, and rare species of Streptococci,
Proteus, Salmonella, and Yersinia. Tuberculous
empyema, fungal empyemas, Mycoplasma,
Legionella pneumophila, adenovirus, and influ-
enza are rare.
polymorphonuclear leukocytes.
Sputum (if child is able to expectorate) should
be sent for Gram staining and culture.
Radiological Investigations

Chest radiograph: With minimal effusion, there

will be blunting of costophrenic angle. With pro-
Clinical Features gressive collection of fluid in the hemithorax, there

Initial features are similar to that of pneumonia

like cough, breathlessness, fever, malaise, fetor,
loss of appetite, lethargy, pleuritic chest pain, and
abdominal pain. Parapneumonic effusion should
be suspected if there is tachycardia and tachy-
pnea, decreased chest expansion, stony dullness
to percussion, reduced or absent breath sounds,
and tracheal deviation.

Investigations

Hemogram will reveal leukocytosis with

increased neutrophils and raised ESR. Mantoux
test should be performed in suspected cases of
tuberculosis.
Pleural fluid aspirate should be sent for urgent
microscopy, Gram stain, culture (bacterial and
tubercular), and differential cell count.
Cytological examination of the pleural fluid in an Fig. 15.2 X-ray of the chest, left empyema
Medical Therapy 95

Fig. 15.4 CT showing pleural collection with thick peel,

collapsed lung

Fig. 15.3 X-ray of the chest right empyema

will be increased haziness and deviation of the tra-

chea to the opposite side (Figs. 15.2 and 15.3).
Chest ultrasonography (USG): Radiograph
may not be able to correctly document the fluid in
the chest as both collapse and consolidation, and
fluid in the chest will be seen as haziness. USG is
helpful in differentiating between fluid and lung
collapse or consolidation. Moreover it can help in
staging the empyema by detecting echogenicity
in fluid, loculation, and pleural thickening.
Additionally, USG can guide for thoracocentesis
and insertion of chest drain into the area of pus
collection. Fig. 15.5 CT showing pleural thickening and loculated
CECT chest: It is useful if any surgical inter- collection
vention like decortications is contemplated.
Thickening of parietal and visceral pleura with
intervening collection gives the pleural split sign ciated with acute lobar pneumonia, and these
suggestive of chronic empyema. Thickened pleu- usually resolve with antibiotics alone. Lack of
ral rind, pus collection, consolidated lung, and clinical improvement after 48 h suggests that
associated lung pathology such as a lung abscess either the choice of antibiotic is inappropriate or
or tumor are better delineated (Figs. 15.4, 15.5, that there is an associated pus collection.
and 15.6a, b).

Management
The management includes treatment of the
underlying pneumonia with antibiotics. For
empyema per se apart from antibiotics, drainage
is needed and is the mainstay of the treatment.
Small sympathetic effusions are commonly asso-
Medical Therapy
Supportive medical therapy includes oxygen,
fluids, nutrition, analgesia, respiratory physio-
therapy, antipyretics and analgesics, appropri-
ate antibiotics based on the sensitivity of
organisms isolated from blood, or pleural aspi-
rate culture.
96
a b
Fig. 15.6 (a) Consolidation of lung, (b) note bronchovascular markings within the consolidation (c.f. empyema)
Drainage
Intercostal tube drainage (ICD) with adequate
size tube (16–24 Fr) depending on the age and
size of the patient, to be inserted under local or
general anesthesia, through the 5th intercostal
space in the midaxillary line. A bubbling chest
drain implies a bronchopleural fistula. If this is
associated with a residual pneumothorax, then
low-pressure suction should be applied. Addition
of fibrinolytic agents like tissue plasminogen
activator (tPA) or urokinase instilled intrapleu-
rally may be effective in early fibrinopurulent
stage of the disease.
 uidelines for Managing
G not developed.
the Intercostal Tube
• Underwater seal bottle should be kept upright
and below the level of the chest.
• Daily records must be kept of the volume of
fluid drained along with temperature, pulse,
and fluid balance charts.
• Medical personnel giving instructions for
chest drain clamping should note:
Bubbling chest drains should never be
clamped.
If chest pain or breathlessness develops, the
chest drain should be unclamped immediately.
15 Empyema Thoracis
• The chest tube removal depends on clinical
and radiological improvement and decrease in
drainage (<1–2 mL/kg/24 h).
• Procedure for ICD removal to be followed:
Older children to perform a Valsalva
maneuver.
Younger children: removal during expira-
tion. This is important as during expiration
there is positive intrathoracic pressure so air is
not sucked inside while removing the chest
tube.
A sterile occlusive dressing should be applied
to the drain wound and left undisturbed for 48 h.
A chest radiograph should be taken shortly after
drain removal to ensure that a pneumothorax has
Surgical Management (Table 15.1)
Indications
1. Clinical non-improvement after adequate
intravenous antibiotics and drainage
2. Cases in which thick, septate pleural collec-
tion cannot be aspirated by needle or drained
by tube thoracostomy (ICD)
3. Advanced stage empyema
Surgical Management 97

Table 15.1 Management of empyema

Clinical and radiological
evidence of empyema

Early Fibrinopurulent Organised

Exudative (Stage 1) (Stage 2) (Stage 3)

Instillation of tPA in
IV antibiotics and
chest tube (3 doses Open or VATS
drainage (aspiration or
12 hours apart with Decortication
chest tube insertion)
one hour dwell)

Lack of clinical
improvement in 4-5
days

Ultrsound chest

If no pleural collection
Persistent Pleural
then continue
disease then VATS
antibotics

VATS video-assisted thoracoscopic decortications

Debridement (Fig. 15.8a, b). Thoracoscopic decortication for

Open surgical debridement or video-assisted tho-
racoscopic decortication (VATS) is effective in
the fibrinopurulent stage of the disease. There are
evidences that early surgical debridement is asso-
ciated with quicker recovery and shorter hospital
stay.
Decortication: Involves blunt and sharp dis-
section to remove the thickened parietal and vis-
ceral pleura, which surround and encase the lung.
This is required for the organized (Stage III)
stage of the disease (Fig. 15.7a, b).
VATS for Empyema
This is indicated in early empyema (within
1–2 weeks). Advantages of thoracoscopic
debridement include less pain, early recovery,
better cosmesis, and shorter hospital stay
thick pleural peel is more technically
demanding.
Persistent bronchopleural fistula usually
results from necrotizing pneumonia and lung
abscess. Treatment consists of adequate chest
drainage, decortication with suture closure of the
fistula, or a muscle flap thoracoplasty (flap taken
from serratus anterior muscle).
Complications of Empyema
The pus can track along a vessel to the chest wall
and present as a fluctuating chest swelling called
“empyema necessitans.” An empyema may rup-
ture into the airway or the pericardium or spread
to the brain or bone in children. Decrease in func-
tional lung volume and chest wall collapse with
crowding of ribs are often associated with chronic
empyema.
98 15 Empyema Thoracis

a b

Fig. 15.7 Open decortication. (a) Thickened parietal pleura with underlying pus collection, (b) removed thick parietal pleura

Fig. 15.8 Comparison

of scar in (a) open a b
surgery versus (b) VATS
for empyema

2009. Pediatric thoracic surgery, 1, Springer, London.

Suggested Reading Coran AG, Caldamone A, Scott Adzich N, Krummel TM,
Laberge JM, editors. Pediatric surgery. 7th ed. Oxford:
Parikh DH, Crabbe DCG, Auldist AW, Rotherberg SS. Elsevier; 2012.
 Congenital Diaphragmatic Hernia
and Eventration 16

Congenital diaphragmatic hernia (CDH) occurs in somal anomalies, tracheobronchial anomalies,

1 per 2000–5000 live birth, and one third remains and neural tube defects. The survival of isolated
stillborn. Associated anomalies are significant CDH is significantly better than those with asso-
cause of poor outcome. Antenatal diagnosis helps ciated non-hernia-related anomalies.
in prognostication and planning the management.

 mbryology of Congenital
E
Site of Defect Diaphragmatic Hernia (CDH)
(Fig. 16.1)
Left-sided defect occurs in 80%, right-sided
defect in 20%, and bilateral CDH cases are rare. • Anterior central tendon develops from septum
transversum
• The dorsolateral crura evolves from the esoph-
Associated Anomalies ageal mesentery
• The dorsolateral portion develops from pleu-
Non-hernia-related anomalies occurs in 35% roperitoneal membranes
cases of postnatally diagnosed CDH which • Muscular portion develops from the thoracic
include congenital heart defects (63%), chromo- intercostal muscle group
Septum transverserum

Pleuro- Esophageal
peritoneal mesentery
membrane

Fig. 16.1 Development of the diaphragm

© Springer Nature Singapore Pte Ltd. 2018 99

S. Roy Choudhury, Pediatric Surgery, https://doi.org/10.1007/978-981-10-6304-6_16
100
Complete closure of pleuroperitoneal canal
occurs by 8th week of gestation. Midgut returns
to abdomen by 9th–10th week. If the canal
remains patent during this period (failure of mus-
cular fusion), then CDH occurs.
Types of Diaphragmatic Hernia
• Posterolateral foramen of Bochdalek hernia
(most common) (Fig. 16.2a, b)
• Foramen of Morgagni (between sternal and
costal attachment) hernia (Fig. 16.3a, b)
• Esophageal hiatus hernia
• Eventration of the diaphragm, i.e., elevation of
the dome of the diaphragm with the presence
of thin muscle fibers
• Traumatic
Pathology
In 90% cases, CDH occur without a hernia sac,
while a sac is present in 10% cases.
CDH usually contains the bowel, spleen, and
left lobe of liver.
Pathophysiology
Lung development (see Chap. 16) determines
prognosis in CDH patients. Pulmonary hypo-
plasia and decrease pulmonary vascular bed
lead to pulmonary hypertension, persistence of
fetal circulation with right-to-left shunt, and
a b
Fig. 16.2 (a) Chest
X-ray, left congenital
diaphragmatic hernia.
(b) Left CDH at surgery
16 Congenital Diaphragmatic Hernia and Eventration
desaturation. Factors which predispose to respi-
ratory distress are hypoxia, acidosis, hypother-
mia, and stress.
 athogenesis of Respiratory Distress
P
in CDH
• Pulmonary hypertension
–– Extensive muscularization of the pulmo-
nary vessels causes persistent pulmonary
hypertension (PPH of the new born).
–– Decreased cross-sectional area of the pul-
monary vascular bed.
• Reduced airway branching
• Underdeveloped alveoli and surfactant
deficiency
Pulmonary hypertension results in right-to-­
left shunt which results in poorly oxygenated
blood to reach peripheral circulation and ulti-
mately to the lungs. This causes further constric-
tion of pulmonary arterioles resulting in increased
pulmonary vascular resistance. This results in a
vicious cycle with rapidly deteriorating hypoxia,
hypercarbia, and acidosis.
Diagnosis
Antenatal diagnosis can be made with ultrasound
scan in 40–90% cases showing the presence of
bowel loops at the level of heart compressing on
the lung (see Chap. 4). Polyhydramnios is pres-
ent in 80% cases.
Eventration of the Diaphragm 101

Antenatal Management mediastinal shift with paucity of abdominal

gas shadow (Fig. 16.2a). Other imaging
Discussed in Chap. 4. ­s tudies like oral contrast study, ultrasonogra-
phy, or CT scan are occasionally required to
differentiate CDH from any cystic lung
Postnatal Management diseases.

Clinical presentation is with different degrees of

respiratory distress and a scaphoid abdomen. In
newborn patients, delayed appearance of symp-
toms indicates better lung reserve and hence bet-
ter prognosis.
X-Ray Chest
X-ray chest is useful for the diagnosis which
shows bowel loops in the thoracic cavity and
Eventration of the Diaphragm
Eventration of the diaphragm refers to elevation
of the dome of the diaphragm into the thoracic
cavity (Fig. 16.4a,b). In contrast to CDH, the
diaphragm retains its attachment to the costal
margins. Eventration of the diaphragm can be
congenital or acquired due to injury to the
phrenic nerve. The clinical ­presentation is with
respiratory symptoms. Symptomatic patients

a b

Fig. 16.3 Intrapericardial hernia through the foramen of Morgagni, (a) barium study and (b) laparoscopic view

a b

Fig. 16.4 (a) Chest

X-ray, left eventration of
the diaphragm. (b) Right
eventration of the
diaphragm
102 16 Congenital Diaphragmatic Hernia and Eventration

require surgical repair generally in the form of • Preoperative strategy:

plication of the diaphragm using nonabsorbable
suture either by open or thoracoscopic approach.
Management of CDH (Table 16.1)
Initial stabilization: Bag and mask ventilation to
be avoided as it will distend the stomach or bowel
present in thoracic cavity!
• CDH is a physiological emergency and not sur-
gical emergency. Delayed surgical repair after
stabilization (resolution/stabilization of pulmo-
nary hypertension) is the preferred treatment.
–– Resuscitation – endotracheal intubation for
ventilation.
–– Nasogastric aspiration to decompress the
abdominal contents.
–– Establish umbilical venous and arterial
lines.
–– Ventilation with either high rates and mod-
est airway pressures or low rates and
higher pressures. Goal is to maintain
­preductal PaO2 > 60 mmHg and
PaCO2 < 60 mmHg.
–– Sedation and reduced handling to prevent
sudden reactive pulmonary hypertension.
–– Muscle paralysis not always indicated.

Table 16.1 Management algorithm for CDH

CDH diagnosed Antenatally

after birth Diagnosed CDH

Stable child with no Unstable child with Intubate and

respiratory distress Respiratory Distress Ventilate

Surgery NG tube insertion

Sedation
Minimal Handling
Umblical vessel
catheterization
Fluid balance
Vasopressors if needed
Pharmacotherpay if
persistent pulmonary
hypertensin

Consider
High frequency ventilation
iNO

Stable Consider
ECMO
Suggested Reading
–– Adequate fluid balance to maintain periph-
eral perfusion. Vasoactive drugs can be
added if needed.
• Pharmacological therapy:
• Currently used drugs in CDH with the aim to
decrease pulmonary hypertension:
–– Phosphodiesterase 5 inhibitors – sildenafil
–– Calcium channel blockers
–– Prostacyclin derivatives
–– Endothelin receptor antagonist
–– Nitric oxide (inhaled nitric oxide, iNO)
Failure to stabilize on conventional ventilation
will require the following measures in sequence
(Table 16.1):
a. Inhaled nitric oxide (iNO) causes pulmonary
vasodilatation.
b. High-frequency ventilation (HFV) avoids
barotrauma.
c. Extracorporeal membrane oxygenation
(ECMO) helps by resting the lung.
The CDH EURO Consortium Consensus is as
follows
Physiological stabilization, which is loosely
defined as 24 h of the following:
• Mean arterial blood pressure normal for gesta-
tional age off inotropes
• Preductal saturation levels of >85% SaO2 in
an FiO2 of <50% off iNO
• Lactate <3 mmol/L
• Urine output >2 mL/kg/h
• No signs of PH
103
Operation
Surgical approach for repair of CDH may be
abdominal or thoracic or minimally invasive tho-
racoscopic or laparoscopic approach. The dia-
phragm should be repaired with nonabsorbable
suture or synthetic patch (PTFE or Prolene mesh)
in case of a large defect. Abdominal wall muscle
flap may also be used.
 rognostic Factors (Fetal
P
and Postnatal)
• Lung-to-head ratio (LHR in fetus) less than
one carries extremely poor prognosis.
• Liver position in the chest is associated with
poor outcome.
• Gestational age (early herniation associated
with poor prognosis).
• Stomach position (in the chest associated with
poor prognosis).
• Polyhydramnios (poor prognosis).
• Cardiac anomaly (poor prognosis).
Suggested Reading
Coran AG, Caldamone A, Scott Adzich N, Krummel TM,
Laberge JM, editors. Pediatric surgery. 7th ed. Oxford:
Elsevier; 2012.
Holcomb GW III, Murphy PJ, Ostile DJ, editors. Ashcrafts
pediatric surgery. 6th ed. Philadelphia, PA: Saunders,
Elsevier; 2014.
Lally PK. Congenital diaphragmatic hernia – current sta-
tus. Semin Pediatr Surg. 2007;16(2):79–134.
 Congenital Lung Lesions
These are congenital lesions which affect the
lung and the mediastinum, and surgical removal
is almost always needed for their treatment.
Types
The commonly used terminology for congenital
lung lesions:
Congenital cystic adenomatoid malformation
(CCAM).
Congenital lobar emphysema (CLE).
Pulmonary sequestration (PS).
Foregut duplication cysts/bronchogenic cysts.
Bronchopulmonary foregut malformation (BPFGM)
is the recently used terminology to encompass all
these anomalies.
Embryology
Lung grows from the third week of gestation till 8
years. Development of lung occurs in five stages.
(1) Embryonic stage starts from the third week of
gestation when the lung starts to grow as a diver-
ticulum from the foregut at the level of laryngo-
tracheal groove which continues till the 6th week.
(2) Pseudoglandular stage extends from 7th to
16th week of gestation when bronchial airways
develop. Conducting airways, i.e., trachea to ter-
minal bronchiole, grow till 16 weeks. (3)
Canalicular stage is between 16 and 24 weeks
© Springer Nature Singapore Pte Ltd. 2018
S. Roy Choudhury, Pediatric Surgery, https://doi.org/10.1007/978-981-10-6304-6_17
17
when alveolar airspaces develop. Type 1 and type
2 pneumocytes (type 2 are precursor of surfac-
tant) begin to differentiate, and gas exchange
becomes functionally possible at the end of this
stage. (4) Saccular stage is between 24 weeks and
term when maturation of alveoli and development
of pulmonary vasculature occur. (5) Alveolar
stage extends from birth to 8 years when alveolar
maturation and multiplication continues.
Tracheoesophageal separation occurs at end
of 6th week. Primitive lung bud formed by the
end of 7th week with 5 major lobes, 3–5 orders of
bronchi. Congenital lung lesions have embryo-
logical, clinical, and histopathological overlap.
Thus it is proposed that they have a common eti-
ology like obstruction of the fetal airway. The
severity, timing, and duration of obstruction
result in various lesions.
Prenatal ultrasonography has resulted in
increased detection of congenital lung lesions.
 ntenatal Diagnosis and Treatment
A
(See Chap. 4)
 ongenital Cystic Adenomatoid
C
Malformation
Incidence is 1 per 8000 to 1 per 35,000 live births.
Embryo-Pathology
CCAM results from an excessive proliferation of
terminal respiratory bronchioles that forms cysts
105
106 17 Congenital Lung Lesions

Table 17.1 Classification of CCAM

Stocker Adzick
Cyst size I > 2 cm Macrocystic >5 mm
II < 2 cm Microcystic <5 mm
III solid
Associated anomalies II Microcystic
Prognosis
Favorable I Macrocystic
Unfavorable III Microcystic
Echogenicity
Solid (echogenic) III Microcystic
Cystic (echolucent) I, II Macrocystic

Table 17.2 Stocker new classification of CPAM
Types Origin Prognosis
0 Tracheobronchial Poor
1 Bronchial/ Good prognosis
bronchiolar
2 Bronchiolar Other malformations,
poor prognosis
3 Bronchiolar/ Solid appearance, poor
alveolar prognosis
4 Distal acinar/ Large cysts, good
peripheral prognosis
The Stocker new classification describes five types on the
basis of their site of origin namely, type 0—tracheo-
bronchical, type 1—bronchial/bronchiolar, type 2—bron-
chiolar, type 3—bronchiolar/alveolar, type 4—distal
acinar/peripheral
of various sizes. Grossly, CCAM is a discrete
intrapulmonary mass that contains cysts ranging
in diameter from 1 mm to over 10 cm. There may
be small connections with the tracheobronchial
tree. It generally involves one lobe of the lung
with preference for lower lobes. Stoker
(Table 17.1) classified them into three types
based primarily on the cyst size. The new Stoker
classification renamed them as congenital
Pulmonary Airway Malformation (CPAM) and
added two more subtypes (type 0 and 4) based on
the location of the development of the malforma-
tion. Type 0 of tracheobronchial origin has solid
appearance with poor prognosis and type 4 of
distal acinar origin with large cysts carry better
prognosis (Table 17.2). Adzick classified prena-
tally detected lesions into two types; macrocystic
type with size 5 mm or more and microcystic
type which appear as a solid echogenic mass on
sonography (Table 17.1). The macrocystic type
has better prognosis than large solid appearance
microcystic lesion.
Antenatal diagnosis
The lesions may be incidentally detected on rou-
tine antenatal sonography. Large-sized lesion
with massive pulmonary involvement may result
in fetal hydrops. Fetal MRI can differentiate
CCAM from other thoracic lesions.
Prognosis
Large microcystic lesions (solid) are more often
associated with hydrops and worse prognosis.
Other prognostic factors are: Lung to thorax
transverse ratio (L/T) and CCAM volume ratio
(CVR; CCAM volume/head circumference).
If CVR is >1.6, then 80% would develop hydrops.
Management of CCAM
The outcome of antenatally diagnosed CCAM
varies from fetal demise to spontaneous regres-
sion (40% cases). For symptomatic fetuses in
early gestation 24–32 weeks, in utero surgery can
salvage. For asymptomatic and late gestation
fetuses, treatment after delivery is recommended.
Chest X-ray may pick up the lesion however;
chest CT scan is more accurate in the diagnosis
(Figs. 17.1a, b, 17.2a, b, 17.3 and 17.4). Surgical
treatment is by lobectomy of the involved lobe
(Figs. 17.5 and 17.6). For asymptomatic lesions,
a period of observation may be justified as com-
plete spontaneous regression is known in 20% of
cases. Elective surgical resection of the lesion is
recommended between 3 and 6 months of age for
asymptomatic lesions as there are long-term risks
Antenatal Diagnosis and Treatment 107

a b

Fig. 17.1 (a) CT Microcystic CCAM with hydropneumothorax. (b) CT Macrocystic CCAM

a b

Fig. 17.2 (a, b) CT showing right lower lobe and left lingula CCAM

Fig. 17.4 Infected CCAM left upper lobe, note solid

appearance

Fig. 17.3 Mini-intensity projection reconstruction reveals

the lobar distribution of the lesion (CCAM)
108 17 Congenital Lung Lesions

Fig. 17.7 X-ray chest showing LUL CLE, note broncho-

Fig. 17.5 Microcystic CCAM at surgery, solid vascular markings within the lesion
appearance
nary lobe due to air trapping, with concomitant
compression of the remaining lung tissue, and dis-
placement of the mediastinum by herniation of the
affected lobe into the opposite side of the chest.

Embryo-Pathology
Fig. 17.6 Macrocystic CCAM (large cyst)
of development of malignancy (bronchoalveolar
carcinoma, rhabdomyosarcoma).
Congenital Lobar Emphysema
Incidence in children is 1 per 20,000 to 1 per 30,000.
CLE is characterized by hyperinflation of a pulmo-
Primary pathology could be bronchomalacia with
focal cartilaginous deficiency causing obstruc-
tion of developing airway with check valve type
of obstruction leading to air trapping. Connection
to the airways is present.
Clinical presentation: One fourth present at
time of birth with respiratory distress, 50% pres-
ent by 1 month, and almost all cases present by 6
months of age. Associated congenital heart dis-
ease in 15% cases hence, echocardiography is
recommended for all cases. Lung lobe involve-
ment in terms of frequency are, left upper lobe
(LUL–40–50%), followed by right middle lobe
(RML–30–40%), and followed by right upper
lobe (RUL–20%).
Investigations: X-ray chest features are;
hyperinflation of the affected lobe, mediastinal
shift to opposite side, compression or atelectasis
of rest of the lung, and chest opacity due to
reduced clearance of lung fluid from affected
lobe after birth. Presence of scant lung markings
within the radiolucent area on a plain X-ray of
the chest with collapse of the adjacent lobe dif-
Pulmonary Sequestration
Fig. 17.8 CT scan showing LUL CLE
Fig. 17.9 Chest X-ray showing right pneumothorax, note
collapsed right lung
ferentiates CLE from pneumothorax (Figs. 17.7,
17.8 and 17.9). CT chest is needed to rule out any
extramural or intramural airway obstruction as
they can also cause hyperinflation.
Management
Lobectomy of the affected lobe is the surgical
treatment of choice (open vs. thoracoscopic)
(Fig. 17.10). Prognosis is excellent, and complete
recovery of lung volume and function in has been
seen in the long-term follow-up. Conservative
109
Fig. 17.10 Appearance of CLE at surgery
management may be reserved for older infants
with minimal symptoms showing radiological
improvement over time.
Pulmonary Sequestration
Definition and Types
They are mass of nonfunctioning lung tissue,
which lack communication with the tracheo-
bronchial tree and are supplied by ananomalous
systemic artery. They are rare occuring in
10–30% of the cystic bronchopulmonary foregut
malformations. In intralobar pulmonary seques-
tration (ILPS), the aberrant lung tissue is local-
ized within the normal lung, with common
visceral pleura (Fig. 17.11). An extralobar pul-
monary sequestration (ELPS) is separated from
the rest of the lung and has its own visceral
pleura. ILPS are two to three times more fre-
quent than ELPS, occur more frequently in
males, and are more localized to the left poste-
rior mediastinum. ELPS can occur anywhere in
the mediastinum, even intra-abdominally.
Diagnosis: The most frequent presentation of
ILPS is recurrent pulmonary infection, however,
ELPS is usually an incidental finding on chest
X-rays. Few cases can also present with high out-
110
Fig. 17.11 CT showing intralobar sequestration left
lower lobe
put cardiac failure due to hyperdynamic circulation
through the anomalous systemic blood supply.
ELPS is often confused with neuroblastoma, and in
some cases excision and biopsy is needed for defin-
itive diagnosis. ELPSs are frequently associated
with other congenital anomalies like congenital
diaphragmatic hernia, tracheoesophageal fistula,
pectus excavatum, and congenital heart disease.
Treatment: Surgical treatment for ILPS
involves a lobectomy; however, for ELPS a
sequestrectomy is often possible. Identification
of the supplying systemic artery is important dur-
ing surgical removal. Few cases of asymptomatic
ELPS with absence of significant systemic blood
supply can be given a trial of observation.
Bronchopulmonary Foregut
Malformations (Bronchogenic Cysts,
Esophageal Duplication Cysts,
Neuroenteric Cysts)
They are usually single unilocular cyst present
in mediastinum, with no contact with the air-
way. They can be differentiated from CCAM as
17 Congenital Lung Lesions
Fig. 17.12 CT of neurenteric cyst; note vertebral
abnormality
they usually have cartilage on histopathologi-
cal examination, whereas CCAM has abun-
dance of smooth muscle and elastin fibers in its
walls.
Embryo-Pathology
They result from an abnormal budding of ventral
foregut at 4–8 weeks of gestation. They arise
from trachea, bronchus, or other airways.
Connection with the parent structure is lost. In
neurenteric cyst, concomitant vertebral deformi-
ties are present (Fig. 17.12).
Diagnosis is by X-ray or CT scan of the chest
(Figs. 17.13a, b and 17.14). The findings are
smooth, spherical, paratracheal, or hilar solid
mass lesion, no calcifications, presence of air
fluid level, mediastinal shift, and honeycombing
in multilocular cysts which is rare.
Treatment is by thoracotomy or thoracoscopic
excision of the lesion. Care should be taken to
prevent injury to the adjacent airway or esopha-
gus as they may share a common muscular wall.
Suggested Reading 111

a b

Fig. 17.13 (a) Chest X-ray bronchogenic cyst. (b) Chest CT bronchogenic cyst

Suggested Reading
Holcomb GW III, Murphy PJ, Ostile DJ, editors. Ashcrafts
pediatric surgery. 6th ed. Philadelphia, PA: Saunders/
Elsevier; 2014.
Coran AG, Caldamone A, Scott Adzich N, Krummel TM,
Laberge JM, editors. Pediatric surgery. 7th ed. Oxford:
Elsevier Limited; 2012.
Choudhury SR, Chadha R, Mishra A, Kumar V, Singh V,
Dubey NK. Lung resections in children for congenital
and acquired lesions. Pediatr Surg Int. 2007;23:851–9.

Fig. 17.14 CT of esophageal duplication cyst

 Esophageal Atresia
Esophageal atresia and tracheoesophageal fistula
(EA/TEF) once considered a fatal congenital
anomaly has now the chance of survival in above
90% cases due to improvement in anesthetic and
neonatal intensive care. Successful anastomosis
of the neonate’s esophageal ends tests the skill of
a pediatric surgeon.
Incidence of EA/TEF is 2–4 per 10,000 live
births, with male preponderance and which has a
sporadic occurrence.
Embryology
Failure of complete separation of the tracheo-
bronchial tree from the esophageal tube results in
EA/TEF. Both of these structures develop from
the embryonic foregut.
Associated anomalies are present in 50–70%
cases.
VACTERL association consists of vertebral,
anorectal, cardiac, tracheoesophageal, renal and
radial anomalies, and limb defects and is present
in 15% cases. At least three of the above anoma-
lies should be present in the VACTERL
association.
 renatal Ultrasonographic Diagnosis
P Chest X-ray with a no. 8 or 10 feeding tube in situ
(Sensitivity 42%)
Small or absent gastric bubble with polyhydram-
nios suggests esophageal atresia.
© Springer Nature Singapore Pte Ltd. 2018
S. Roy Choudhury, Pediatric Surgery, https://doi.org/10.1007/978-981-10-6304-6_18
18
 ypes of Esophageal Atresia
T
(Gross, Fig. 18.1)
(a) Esophageal atresia without fistula (EA (8%))
(b) Esophageal atresia with upper pouch fistula
(0.6%)
(c) Esophageal atresia with lower pouch fistula
(most common (85%))
(d) Esophageal atresia with upper and lower
pouch fistula (1.4%)
(e) H-type tracheoesophageal fistula (4%)
Clinical Presentation
• Excessive salivation in a newborn baby.
• Feeding is associated with regurgitation,
chocking, coughing, cyanosis, and respiratory
distress.
• Inability to pass a nasogastric tube.
• Abdominal distension (in cases of tracheo-
esophageal fistula).
• Aspiration of saliva and gastric juice causing
pneumonia.
Diagnosis
passed orally or nasally shows arrest at T3–T4
(thoracic) vertebral level which is approximately
10 cm from the mouth. Presence of air in abdo-
men indicates presence of TEF (Fig. 18.2).
113
114 18 Esophageal Atresia

a (8%) b (0.6%) c (85%) d (1.4%) e (4%)

Fig. 18.1 Types of esophageal atresia (Gross)

Gasless abdomen suggests pure esophageal

atresia without fistula (EA).

Other Investigations
• Echocardiography to rule out cardiac
anomalies
• Ultrasonography to look for renal anomalies

Features of Esophageal Atresia (EA) Without

TEF (Pure Esophageal Atresia)
• Clinically flat-looking abdomen and gasless
abdomen on plain X-ray (Fig. 18.3a, b).
• The gap between the esophageal ends is
usually long (more than two vertebral
bodies).
• Often requires staged operation to restore
esophageal continuity.
Fig. 18.2 TEF, note the coiled feeding tube in the upper
esophageal pouch

a b

Fig. 18.3 Esophageal

atresia. (a) Flat
abdomen. (b) Gasless
abdomen on X-ray
Management 115

Management (Table 18.1) pouch minimizes the risk of tracheal

Preoperative Treatment
• A double-lumen Replogle tube (sump cathe-
ter) inserted nasally or orally for continuous
drainage of saliva from the upper esophageal
aspiration.
• Nursing in head-up position helps minimizing
gastric juice reflux.
• Intravenous fluid, broad-spectrum antibiotics
and vitamin K to be initiated. Special attention to
be paid on chest care including physiopherapy.

Table 18.1 Management algorithm of EA/TEF

Suspected Esophageal
atresia ± TEF

look for associated

Look for VACTERL
malformations (ECHO,
association
USG)

Attempt insertion of 10F

oral catheter and confirm
with Chest X Ray

Bowel gas present

(TEF)

Right posterolateral
thoracotomy

Anastomosis feasible Anastomosis not Bowel gas absent (Pure

then Primary repair feasible ( Long Gap) EA)

Cervical esophagostomy +gastrostomy followed

by esophageal substitution later
Delayed esophageal anastomosis (Pure EA)
Esophageal lengthening procedure
Primary esophageal subsitution
116 18 Esophageal Atresia

Operation

The operation can be done by thoracoscopy or

thoracotomy. The open technique involves a right
posterolateral thoracotomy with an extrapleural
approach with division of fistula and primary
end-to-end esophageal anastomosis. For neo-
nates in whom echocardiography shows right
aortic arch, some surgeons prefer left-sided
thoracotomy.

Steps of Surgery

• Approach is by right posterolateral thoracot-

omy through the 4th intercostal space Fig. 18.4 Surgical division of fistula (short arrow) and
• An extrapleural approach is preferred with esophageal anastomosis (long arrow)
pleura reflected medially which helps in lung
retraction
Table 18.2 Survival of esophageal atresia/tracheoesoph-
• Azygous vein is ligated and divided ageal fistula
• Ligation and division of TE fistula
Group Waterstone Survival (%)
(Fig. 18.4) is performed
A BW > 2500 g, otherwise well 100
• Generous mobilization of upper esophageal
B BW 2000–2500 g and well or 85
pouch helps in reducing the gap between the higher weight with moderate
ends for anastomosis associated anomalies
• An end-to-end anastomosis of both the esoph- C BW < 2000 g or higher with 65
ageal pouches performed with fine interrupted severe congenital anomalies
sutures (Fig. 18.4) Okamoto modification of Spitz
Group classification Survival (%)
Pure atresia and long-gap atresia—requires I BW > 2000 g, no major cardiac 100
staged operation (see below). anomaly
II BW < 2000 g, no major cardiac 81
anomaly
III BW > 2000 g and major 72
 rognostic Factors for Esophageal
P cardiac anomaly
Atresia (Table 18.2) IV BW < 2000 g and major 27
cardiac anomaly
Postoperative Complications

The common complications are anastomotic
leak, stricture (Fig. 18.5), recurrent TEF, GE
reflux, and tracheomalacia. A minor anastomotic
leak ofter resolves under observation. A major
anastomotic leak may require surgical interven-
tion by either reanastomosis or more often by a
diversion procedure.
TEF Without Atresia (H-Type TEF)
H-type TEF presents with history of coughing
and chocking during feeding and recurrent
pneumonia. Diagnosis can be made on con-
trast swallow esophagography (Fig. 18.6) and
on bronchoesophagoscopy. Contrast esopha-
TEF Without Atresia (H-Type TEF)
Fig. 18.5 Anastomotic stricture post TEF repair
Fig. 18.6 Contrast esophagogram showing H-type TEF
117
Fig. 18.7 Cervical ligation of H-type TEF
gogram is performed with child in prone posi-
tion, the contrast is injected into distal
esophagus through a feeding tube. The ­contrast
is injected, while the tube is gradually with-
drawn. Surgical treatment consists of ligation
and division of fistula (Fig. 18.7), generally
through a neck incision approach or rarely by
a thoracotomy.
Long-Gap Esophageal Atresia
• All isolated esophageal atresia without TEF
generally are long gap.
• EA/TEF with long gap is considered when
primary esophageal anastomosis is not feasi-
ble even after extensive mobilization.
Management Options for Long-Gap
Esophageal Atresia
• Primary anastomosis—extensive esophageal
mobilization (Scharli’s lesser curve elonga-
tion technique).
• Delayed primary anastomosis. The principle
is based on rapid spontaneous growth of the
esophageal ends, making an end-to-end anas-
tomosis possible after 4–6 weeks (Prem puri).
118
Fig. 18.8 (a) Cervical esophagostomy with feeding gastrostomy. (b) Gastric tube and
gastric pull-up for esophageal replacement
• Intraoperative myotomy (Livaditi) and flap
techniques (Gough).
• Intra-extra-thoracic suture traction elongation
technique (Focker).
• Multistage extra-thoracic elongation tech-
nique (Kimura).
• Esophagostomy and gastrostomy in the new-
born. Delayed bridging of the esophagus with
the stomach or colon (Fig. 18.8a, b).
• Primary gastric transposition in the newborn
(the stomach pulled up in the chest and anas-
tomosed directly to the esophagus).
18 Esophageal Atresia
Suggested Reading
Coran AG, Caldamone A, Scott Adzich N, Krummel TM,
Laberge JM, editors. Pediatric surgery. 7th ed. Oxford:
Elsevier Limited; 2012.
Pierro A. Oesophageal atresia. Semin Pediatr Surg.
2009;18(1):1–57.
Roy Choudhury S, Ashcraft KW, Sharp RJ, Murphy JP,
Snyder CL, Sigalet DL. Survival of esophageal atresia
patients: influence of birth weight, cardiac anomaly,
and late respiratory complications. J Pediatr Surg.
1999;34:70–4.
Choudhury SR. Surgical diseases of the esophagus in chil-
dren. J Indian Assoc Pediatr Surg. 2016;21:96–7.
 Esophageal Replacement
Substitution of the esophagus is required in chil-
dren when the native esophagus becomes unavail-
able or nonfunctional. There is no ideal substitute
for the esophagus.
I ndications for Esophageal
Substitution in Children
Esophageal replacement in children is most com-
monly required in those suffering from esopha-
geal atresia. This surgery is needed in the
following situations:
• Failed anastomosis in esophageal atresia with
tracheoesophageal fistula
• Wide-gap esophageal atresia
A trial of delayed primary repair after 6–12
weeks in these circumstances is often successful,
wherein a gastrostomy is made to feed the child
and repeated or continuous upper pouch suction
performed to prevent aspiration. This principle is
based on rapid growth of the esophagus in the
first few months of life. However, in limited-­
resource centers, delayed primary repair is often
unsuccessful because of prolonged hospital stay
and worsening of chest condition due to aspira-
tion of saliva.
Other indications of esophageal replacement
in children include the following:
© Springer Nature Singapore Pte Ltd. 2018
S. Roy Choudhury, Pediatric Surgery, https://doi.org/10.1007/978-981-10-6304-6_19
19
• Esophageal strictures either corrosive or peptic
• Achalasia, tumors, etc.
• Scleroderma
• Epidermolysis bullosa
• Diffuse esophageal candidiasis in an immune-­
compromised child
Criteria for an Ideal Substitute for the
Esophagus
• Must function as an efficient conduit
• Should grow with the child and last lifelong
• Technique should be simple and adaptable
• Minimal reflux or resistant to reflux
• Should not cause respiratory or cardiac
embarrassment
• Minimal cosmetic deformity
• Easily manageable postoperative complications
Timing of surgery: There is no ideal time for
surgery. Operations done at 6–12 months of age
are tolerated well than done at the neonatal
period. This can be explained as most of these
conduits do not have peristaltic activity and
depend on gravity for clearance of food to the
stomach. At around 1 year of age, the child
spends significant time in upright position which
helps in clearance of the neoesophagus. An ideal
patient should be nutritionally well preserved.
It is very important to be aware of following
issues during or after the initial diversion surgery
as it may affect the ultimate outcome.
119
120 19 Esophageal Replacement

• Siting of gastrostomy: It should be sited in • Jejunal interposition

the antrum of the stomach and away from the
greater curvature to make it available for gas-
tric tube formation.
• Sham feeding: Patients on esophagostomy
should be encouraged to swallow feeds which
will come out of the cervical esophagostomy
but will aid in early recovery of feeding and
swallowing functions.
• The surgeon should be prepared to perform
an alternate procedure if the desired proce-
dure cannot be performed, e.g., abnormal
blood supply and unprepared or unsuitable
organ.
Routes of Substitution (Table 19.1)
The following three routes are used:
• Transpleural
• Retrosternal
• Trans-hiatal posterior mediastinal
 ifferent Replacement Procedures
D retrosternal). Esophagogastric anastomosis is done
(Table 19.2)
• Gastric transposition
• Gastric tube
• Colon interposition
• Fundal tube esophagoplasty
Gastric Transposition (Figs. 19.1 and 19.2)
Operative Technique
The stomach is mobilized with preservation of the
right gastric and right gastroepiploic vessels and
the gastrostomy site closed. Esophageal stump is
transected and closed. The highest point of the
fundus identified for pull-up provides the maxi-
mum length. The second part of duodenum is
Kocherized, and pyloromyotomy or pyloroplasty
done (may not be necessary). For trans-­ hiatal
route, a posterior mediastinal tunnel is made over
the vertebral body. Cervical esophagostomy is
mobilized, and the posterior mediastinal tunnel
made just anterior to the prevertebral fascia keep-
ing the finger over the vertebral body all along the
dissection. The abdominal and neck fingers are
advanced till they meet and widened at 2–3 finger
breadth, and the tunnel is completed. The stomach
is pulled through the thorax into the neck through
the desired route (posterior mediastinal or
in the neck. The pyloric antrum is fixed to the dia-
phragm. A feeding jejunostomy is performed. A
contrast swallow esophagogram (Fig. 19.3) done
between the 8th to 10th postoperative day is fol-
lowed by initiation of oral feeding.

Table 19.1 Comparison of the three routes of esophageal replacement

Route Advantage Disadvantage
Retrosternal No major structures encountered in this Longest route from the neck to the
route abdomen
Ease of procedure Angulation of the graft
When other routes are unavailable due to Problems with assess if cardiac
fibrosis surgery is required
Transpleural Convenience and ease of the procedure Displacement of the lung
Requires thoracotomy
Posterior mediastinal Most direct route Mediastinum may be unavailable
Organ contained in the mediastinum because of previous surgery, fibrosis,
Little or no lung compression or inflammation
Thoracotomy not always required
Different Replacement Procedures 121

Table 19.2 Advantages and disadvantages of various procedures

Method Advantages Disadvantages
Colon Adequate length Precarious blood supply
Reflux seldom occurs Graft necrosis
High incidence of leaks and strictures
Multiple anastomosis
Redundancy over the long term
Slow transit of food
Gastric tube Adequate length Very long suture line
Good blood supply High incidence of leaks and strictures
Size of conduit appropriate Reflux leading to Barrett’s syndrome
Rapid transit
Jejunum Appropriate size Very precarious blood supply
Retention of peristaltic activity
Length can be a problem
Three anastomosis
Free jejunal graft Appropriate size Specialized technique for microvascular
Good peristaltic activity anastomosis
Prolonged operating time
Precarious blood supply
High failure rate
Stomach Adequate length easily attained Bulk of stomach in thorax
Excellent blood supply Reflux common early on
Single anastomosis Affects growth/pulmonary function
Ease of procedure Severe post-op tachycardia

a b

Fig. 19.2 Gastric pull-up

Complications of gastric transposition (see

Fig. 19.1 (a) Gastric tube and (b) gastric pull-up for
esophageal replacement Table 19.3).
122 19 Esophageal Replacement

Postoperative Care Following Gastric

Transposition
• Strict monitoring of vitals in an intensive care
unit setup
• Elective ventilation for 24–48 h
• Nasogastric aspiration to prevent acute gastric
dilatation
• Pain relief
• Early jejunostomy feeding
• Chest physiotherapy

Gastric Tube Interposition (Figs. 19.1,

19.4, 19.5, and 19.6)

1. Antiperistaltic based on the left gastroepiploic

artery (reverse gastric tube)
2. Isoperistaltic based on the right gastroepiploic
artery

Techniques
• Tube constructed from about 2.5 cm above
pylorus on 22–28 Fr tube based on either the
left or the right gastroepiploic artery
Fig. 19.3 Oral contrast study following gastric pull-up • Tube pulled up retrosternally or through pos-
terior mediastinum
• Anastomosis is made with the upper esopha-
Table 19.3 Complications of gastric transposition gus in the neck which may be done primarily
The early complications: or delayed by 4–6 weeks to reduce the chance
 • Pneumothorax of leak
 • Tracheal, major vessel injury
 • Severe tachycardia
 • Respiratory compromise  omplications of Gastric Tube
C
 • Venous compression (See Table 19.4)
 • Acute gastric dilatation
 • Anastomotic leak Colon Interposition
 • Cardiac Arrhythmia
Late complications: This is still a popular procedure done in the pedi-
 • Anastomotic stricture atric age group. Preoperative bowel preparation
 • Swallowing problem is required. Barium enema should be done to
 • Delayed gastric emptying rule out rotational anomalies, polyposis, or other
 • Jejunostomy tube complication colonic diseases. Depending upon the choice of
 • Dumping syndrome the surgeon and anatomy of the vascular arcade
 • Anemia of the colon, the suitable part of the colon is iso-
 • Gastroesophageal reflux lated and cleaned with irrigation. It is passed
Different Replacement Procedures
Fig. 19.4 Gastric fundal tube (Rao)
Fig. 19.5 Reverse gastric tube
Fig. 19.6 Contrast study following reverse gastric tube
123
Table 19.4 Complications of Gastric Tube
 • Pneumonitis
 • Mediastinitis may occur because of leak from
long suture line
 • Cervical anastomotic leak
 • Stricture (30–40%)
 • GER
 • Ulcer
 • Bleeding
behind the stomach and the pylorus and through
the desired route to the neck for cervicocolic
anastomosis above and cologastric anastomosis
in the gastric antrum below. Colocolic anasto-
mosis is performed to restore continuity of the
colon.
 ifferent Techniques of Using the Colon
D
• Right colon—isoperistaltic or retroperistaltic
(popular in the United States)
• Left colon or transverse colon (popular in
Europe)
• Right colon substernal route
• Left colon retrohilar route (Waterston)
• Left colon trans-hiatal posterior mediastinal
route (Hamza)
Contraindications of Colonic Interposition
• Incomplete marginal arterial arcade
• Associated high anorectal malformation and
pouch colon
• Any other colonic disease
Principles of Waterston Operation
• Preservation of lower esophageal stump and
gastroesophageal junction (Ashcraft)
• Colon mobilization through diaphragmatic
incision
• Isoperistaltic segment based on the left colic
artery
• Left sixth space thoracotomy Complication
of Colonic Interposition Procedures
(Table 19.5)
124 19 Esophageal Replacement

Table 19.5 Complications after colonic interposition Suggested Reading

 • Necrosis of graft
 • Anastomotic leak (30%) Coran AG, Caldamone A, Scott Adzich N, Krummel TM,
Laberge JM, editors. Pediatric surgery. 7th ed. Oxford:
 • Esophago-cutaneous fistula
Elsevier; 2012.
 • Transient malabsorption Holcomb GW III, Murphy PJ, Ostile DJ, editors. Ashcrafts
 • Strictures (20%) pediatric surgery. 6th ed. Philadelphia, PA: Saunders/
 • Gastric outlet obstruction Elsevier; 2014.
 • Mediastinitis, empyema Spitz L, Coran C, Teitelbaum DH, Pierro A, Tan
HL. Operative pediatric surgery. 7th ed. Boca Raton:
 • Redundancy CRC Press; 2013.
 • Halitosis Choudhury SR, Yadav PS, Khan NA, Shah S, Debnath
 • Graft-related complications: ulcer, colitis, PR, Kumar V, Chadha R. Pediatric esophageal sub-
hemorrhage, polyp stitution by gastric pull-up and gastric tube. J Indian
Assoc Pediatr Surg. 2016;21:110–4.
 Part 5
Abdomen
 Acute Abdomen in Children
Acute abdomen is defined as a sudden onset of
severe pain in the abdomen due to some intra-
abdominal pathology and often requires surgical
intervention. Acute abdomen is one of the most
common and distressing problems faced by the
clinicians treating children. The following ques-
tions are frequently raised while evaluating a
child with acute abdomen:
• The need for hospital admission versus home
treatment
• When to refer for surgical consultation
• Whether analgesics are to be prescribed or not
• When is the appropriate time for any
intervention
 elevant Clinical History
R Characteristics of Pain
in the Evaluation of a Child
with Acute Abdomen
• Length of the current illness
• Previous similar episodes
• Periodicity: is the pain constant, getting better,
or worse
• Any related aggravating factor
• Associated with any bilious vomiting, diar-
rhea, or urinary symptoms
© Springer Nature Singapore Pte Ltd. 2018
S. Roy Choudhury, Pediatric Surgery, https://doi.org/10.1007/978-981-10-6304-6_20
20
Clinical Examination
• Being patient and gentle with the patient and
parents provides a comfortable environment.
• Elicit for tenderness: local or general, same
site, any pointing sign.
• Nonspecific pain is vague, central, and colicky
in nature. Pain due to appendicitis is localized
and worsens over time.
• Localized tenderness, guarding, rigidity, and
rebound tenderness are important signs indi-
cating underlying inflammation.
• Examination of hernial sites.
• Hospital admission, “active observation,” and
repeat examination in doubtful cases.
• Characteristics of visceral pain are dull, poorly
localized, and central.
• Characteristics of parietal pain are sharp,
localized, cough, or movement that aggravates
pain.
• Referred pain follows the same dermatome.
• Foregut pain is localized to epigastric region.
• Midgut pain is localized to periumbilical region.
• Hindgut pain is localized to pelvic region.
127
128
 ed Flag Signs for Acute Abdominal
R • The most common surgical cause of acute
Pain in Children
• Bilious vomiting
• History of intra-abdominal surgery
• Features of peritonitis (tenderness, guarding,
rigidity, and rebound tenderness) (Fig. 20.1)
• Blood in stool
• Blood in vomitus
• Features of intestinal obstruction
• Abdominal distension
• Systemic signs
Etiology: Points to Remember
• Nonspecific abdominal pain is the most com-
mon cause of acute abdomen in children.
• The most common medical cause of acute
abdomen in children is gastroenteritis.
Fig. 20.1 Distended abdomen, bilious nasogastric aspi-
rate, anxious facial appearance of peritonitis
a b
Fig. 20.2 (a, b) Abdominal X-ray showing pneumoperitoneum
20 Acute Abdomen in Children
abdomen in children is acute appendicitis.
• Non-gastrointestinal causes of acute abdomen
are urinary tract infection, urinary stones,
pneumonia, Henoch-Schonlein purpura,
hemolytic disorder, and diabetic ketoacidosis.
I nvestigations for Evaluation
of Acute Abdomen in Children
(a) Urine microscopy: can help in diagnosing
urinary tract infection or renal trauma.
(b) CBC, electrolytes, amylase, lipase, and liver
function tests: Increased leukocyte counts
are suggestive of some infective process.
Serum amylase and lipase are useful in diag-
nosis of acute pancreatitis. Liver function
test is useful in diagnosing hepatitis and bili-
ary obstruction.
(c) Abdominal and chest X-ray (Figs. 20.2a, b
and 20.3): Chest X-Ray is helpful in detecting
pneumonia or empyema. ­Pneumoperitoneum
can also be detected by presence of free air
under the diaphragm.
Abdominal radiograph should include
both supine and upright films. Both domes of
the diaphragm and the pelvis should be
included in the films. Relevant findings are
the following:
• Intestinal obstruction: multiple air fluid
levels, dilated bowel loops
Summary of Common Causes of Acute Abdomen Seen in Practice 129

Table 20.1 Causes of acute abdomen in children

Infants <2 years
• Colic
• Infantile dyschezia
• Gastroesophageal reflux
• Non-gastrointestinal causes
• Intussusception
• Malrotation and volvulus
• Incarcerated hernia with bowel necrosis
• Ovarian torsion
• Acute gastroenteritis
• Abdominal trauma
• Constipation
• Toxin ingestion
Fig. 20.3 Abdominal X-ray showing filling defects in the Children (2–18 years)
intestine, features suggestive of worm obstruction • Acute gastroenteritis
• Urinary tract infection and urolithiasis
• Necrotizing enterocolitis: dilated bowel
• Constipation
loops, pneumatosis intestinalis, fixed bowel
• Streptococcal pharyngitis
loop, and portal venous gas
• Food poisoning
• Meconium ileus/peritonitis: soap-bubble
• Mesenteric lymphadenitis
appearance and calcification in the right • Appendicitis
iliac fossa • Intestinal obstruction
• Perforation: pneumoperitoneum • Pneumonia
• Malrotation with volvulus: gasless abdomen • Abdominal trauma
• Testicular torsion
(d) Ultrasonography: It is useful in workup of • Pancreatitis
children with acute abdomen as it is readily • Henoch-Schonlein purpura
available and noninvasive investigation. It is • Acute cholecystitis
useful in detecting fluid in the abdomen • Abdominal migraine
(echogenic fluid suggestive of perforation or Adolescents (12–18 years)
pus), intussusception, appendicitis, and • Dysmenorrhea
pathology of the solid visceral organs. • Mittelschmerz
Ultrasound is also useful in assessing the • Pelvic inflammatory disease
genitourinary causes like renal stone, tumor, • Threatened abortion
ovarian or testicular torsion, etc. • Ectopic pregnancy
(e) CT/MRI/contrast enema/swallow or follow-­
through is needed in special cases.
(f) Laparoscopy is indicated if all other investi- • Mesenteric adenitis: accompanied by viral
gations are equivocal and doubt of hollow URI, high-grade fever, and colicky central
viscus injury persists (Table 20.1). abdominal pain.
• Worm infestation is common in developing
countries.
Summary of Common Causes • Acute appendicitis: abdominal pain followed
of Acute Abdomen Seen in by low-grade fever and vomiting. Localized
Practice tenderness over the McBurney’s point can be
elicited.
• Gastroenteritis: vomiting precedes pain, diar- • Constipation.
rhea predominates. • Intussusception.
130 20 Acute Abdomen in Children

• Intestinal obstruction (Meckel’s, bands, her-

nia, worm obstruction) (Fig. 20.4).
• Intestinal perforation (typhoid, TB)
(Figs. 20.5), gangrene (Fig. 20.4).
• Tubercular abdomen.
• Gall bladder and biliary disease (acute hepati-
tis), liver abscess, acute cholecystitis, compli- Fig. 20.4 Worm obstruction with gangrene of intestine
cated choledochal cyst (Figs. 20.6 and 20.7).
• Pancreatitis, splenic abscess.
• Ovarian and pelvic pathology.
• Urolithiasis and urinary tract infection.

I ndications for Surgical

Consultation in Cases of Acute
Abdomen

• Severe pain with progressive deterioration

• Bile-stained or feculent vomiting
• Involuntary abdominal guarding, rigidity
• Severe tenderness, rebound tenderness
• Marked distension with diffuse tympany Fig. 20.5 Typhoid perforation of the ileum
• Signs of acute fluid, blood loss
• Abdominal trauma or history of previous surgery
• Abdominal pain without obvious etiology

Current search of literature does not preclude

the use of analgesics in acute abdomen.

Management

The general principles of management include

the following:

• Resuscitation of the child Fig. 20.6 Perforated gall bladder, managed by

• The first focus should be on establishing
hemodynamic stability. Proper hydration,
nasogastric drainage, monitoring urine output,
analgesic, and antibiotics as required for the
underlying condition should be instituted. A
period of stabilization is necessary before any
surgical intervention.
cholecystectomy
• Identification of cases which need urgent sur-
gical intervention.
• Treatments of different causes of acute abdo-
men are discussed in the relevant sections
(chapters).
Suggested Reading 131

Suggested Reading
Hutson JM, O’Brien M, Woodward AA, Beasley
SW. Jone’s clinical pediatric surgery diagnosis and
management. 6th ed. Oxford: Blackwell; 2008.
Coran AG, Caldamone A, Scott Adzich N, Krummel TM,
Laberge JM, editors. Pediatric surgery. 7th ed. Oxford:
Elsevier Limited; 2012.
Williams NS, Bulstrode CJK, O’Connell PR, editors.
Bailey & Love’s short practice of pediatric surgery.
26th ed. Abingdon: Taylor & Francis Group; 2013.

Fig. 20.7 Choledochal cyst with perforation, managed

by cyst excision and hepaticojejunostomy
 Umbilical Anomalies
Disorders of the umbilicus are frequently encoun-
tered in pediatric surgery. An understanding of
the embryology of the abdominal wall formation
and umbilicus is important for identifying and
properly treating these conditions.
 mbryology of Formation
E List of Anomalies
of the Umbilicus
During 6–8 weeks of fetal development, the gastro-
intestinal tract herniates into the umbilical cord due
to rapid bowel growth called physiological midgut
hernia. The intestine returns in the abdomen by
10–12 weeks and undergoes a 270° counterclock-
wise rotation and fixation. Normally the umbilical
ring closes by birth due to the developing abdomi-
nal wall. At birth the umbilical cord contains one
umbilical vein, two umbilical arteries, the fibrous
remnants of the urachus, and omphalomesenteric
duct (vitellointestinal duct) (Table 21.1).
After ligation of the cord, the vessels throm-
bosed, and the cord dries and sloughs off, leaving
a granulating surface that heals by cicatrization,
and is covered by epithelium. This is followed by
scar contraction and retraction of the umbilicus.
Clamping of the cord should be done few cen-
timeters away from the base of the umbilical ring
to avoid any injury to bowel, should a small
umbilical hernia persists. It is a common practice
to apply topical antimicrobial agents to prevent
infection of the umbilical stump especially when
© Springer Nature Singapore Pte Ltd. 2018
S. Roy Choudhury, Pediatric Surgery, https://doi.org/10.1007/978-981-10-6304-6_21
21
proper cord care is lacking. Normally the cord
separates and falls off between 3 days and 2
months. Delayed cord separation may be due to
neutrophil functional disorder or immunological
problem.
• Umbilical granuloma/umbilical polyp
• Patent vitellointestinal duct (VID), Meckel’s
diverticulum/band
• Patent urachus/urachal cyst, remnants
• Umbilical hernia
• Exomphalos minor, major
• Gastroschisis
• Exstrophy bladder/cloacal exstrophy
• Acquired (sinus, fistula, infection)
Common Presentations
Umbilical Hernia (Fig. 21.1)
Umbilical hernia can be differentiated from
omphalocele as they are never present at birth
and develop once the cord stump falls off due to
weakness of the umbilical cicatrix. Small hernia
at birth can be observed for spontaneous closure
which generally occurs between 2 and 3 years of
age by cicatrization of the umbilical ring.
Reassurance of the family during counseling is
133
134 21 Umbilical Anomalies

Table 21.1 Embryological structures of the umbilicus and their fate

Embryological structure
Urachus (connects the bladder to
the allantois)
Omphalomesenteric duct/vitelline
duct (connects the midgut to the
yolk sac)
Omphalomesenteric arteries
Omphalomesenteric veins
Umbilical arteries
Umbilical veins
Fate
Obliterates and forms the median
umbilical ligament
Obliterates and in 2% of population
it forms Meckel’s diverticulum
Most regress, fuse to form the
celiac, superior and inferior
mesenteric arteries
Plexus around the duodenum
becomes the superior mesenteric
and portal vein
Obliterate after birth
Right obliterates during fetal life;
left connected to ductus venosus,
obliterates after birth
Pathologic condition/remnant
Patent urachus, sinus, cyst
Patent omphalomesenteric duct, sinus,
cyst, band, or polyp
Dominant artery may accompany
Meckel’s diverticulum, fibrous band
from the umbilicus to the bowel
mesentery
Preduodenal portal vein
Medial umbilical ligaments
Falciform ligament

umbilical defect with a taped coin is of no benefit

and should be avoided.

 atent Vitellointestinal Duct (PVID)

P
(Fig. 21.2a, b)

Depending upon the degree of the obliteration of

the duct, it may have various presentations:

• Umbilical sinus: Only the part near the umbi-

Fig. 21.1 Umbilical hernia
important. Persistence of the hernia after 2–3
years of age or a large defect requires surgical
repair. Operation involves a small sub-umbilical
incision with dissection around the neck of the
sack and fascial approximation for closure of the
defect, with preservation of the umbilicus.
Although obstructed umbilical hernia is rare, it
needs repair even if reduced. Occlusion of the
licus is patent and usually presents with
umbilical discharge.
• Patent VID: The whole duct is patent. This
is suspected in patients pressing with the
history of discharge of fecal matter and/or
gas from the umbilicus which may be asso-
ciated with prolapsed of intestine from the
umbilicus. Treatment requires surgical
exploration, disconnection of the duct rem-
nant from ileum and often anastomosis of
the native bowel.
• Umbilical band: Usually asymptomatic but
child may present with volvulus or obstruc-
tion due to twisting of intestines around the
band.
• Meckel’s diverticulum is discussed in Chap. 34
If the diagnosis is uncertain, then ultrasonog-
raphy or sinogram may help. If the diagnosis is
still uncertain, then umbilical exploration is
Infected Umbilical Stump 135

Fig. 21.2 (a) Patent

VID, (b) Prolapsed VID
a b

needed. Treatment requires surgical exploration,

disconnection of the duct remnant from the
ileum, and often anastomosis of the native bowel.

Umbilical Mass (Figs. 21.3 and 21.4a, b)

The parents may complain of a reddish mass pro-

truding from the umbilicus. The differentials
include umbilical granuloma, umbilical polyp,
and rarely patent vitellointestinal duct. The latter
is very obvious due to appearance of intestinal
mucosa. Umbilical granuloma is due to forma-
tion of granulation tissue at the umbilical stump
and has rough surface, whereas an umbilical
polyp represents remnant of vitellointestinal duct
and has a glistening red mucosal surface.
Management
Umbilical granuloma (Fig. 21.4a, b) is the most
common umbilical problem in infants which
presents with an irregular pale mass at the umbi-
licus. They usually respond to chemical cauter-
ization by topical application of silver nitrate or
trichloroacetic acid. Care should be taken to
avoid touching any undesired site. More than one
application may be required. Pedunculated
lesions can be tied off at the base and can be
allowed to fall off. Crushing the base of the gran-
uloma with a fine-tip artery forceps is curative
and can be done as an outpatient procedure with-
Fig. 21.3 Umbilical polyp
out anesthesia. Persistent discharge calls for sur-
gical exploration.
Umbilical polyps (Fig. 21.3) are cherrylike
mass at the umbilicus with smooth mucosa-lined
surface. This may have underlying remnant of
vitellointestinal duct in 30–60% of cases and
need evaluation by ultrasonography or laparos-
copy. Surgical exploration and excision of rem-
nants along with the polyp is curative.
Infected Umbilical Stump
Infection of the umbilical stump presents with
erythema and purulent discharge from the umbi-
licus. Cleaning of the area with spirit and appli-
cation of local antiseptic (0.2% mercurochrome
lotion) often resolves the infection. Systemic
antibiotics may be necessary.
136 21 Umbilical Anomalies

Fig. 21.4 (a, b)

Umbilical granuloma a b

 atent Urachus or Urachal Cyst

P
Remnants

They may present as passage of urine from the

umbilicus. Retraction of umbilicus on passage of
urine is also a sign. An ultrasound examination of
the urinary tract may detect a urachal cyst.
Micturating cystourethrogram is usually not indi-
cated unless there is suspicion of posterior ure-
thral valve. Surgical intervention is necessary as
there cysts are liable to get infected or develop
malignancy in the long term.
For exomphalos minor (Fig. 21.5), see Chap.
22 on abdominal wall defects.

Suggested Reading
Coran AG, Caldamone A, Scott Adzich N, Krummel
TM, Laberge JM, editors. Pediatric Surgery. 7th ed.
Oxford: Elsevier; 2012.

Fig. 21.5 Exomphalos minor

 Abdominal Wall Defects
Formation of the abdominal wall involves a
complex embryological process, and any aber-
ration of that results in various abdominal wall
defects. Antenatal diagnosis of such defects and
associated malformations help in identifying
prognostic factors influencing outcome.
Types
• Gastroschisis
• Omphalocele
• Hernia of the umbilical cord
• Exstrophy/cloacal exstrophy
Definition
Omphalocele is a large defect >4 cm through the
umbilicus, covered with a sac (amniotic mem-
brane) containing the intestines, liver, and occa-
sionally spleen and gonad.
Gastroschisis has no covering membrane; the
defect is usually to the right side of the umbili-
cus; the bowel is usually thickened, matted, and
edematous due to exposure to the amniotic fluid.
Hernia of the umbilical cord (due to failure of
midgut to return to the abdominal cavity) is a
small defect containing only the intestine and
covered with a membrane. The recti muscles
meet cranially in midline at xiphoid.
© Springer Nature Singapore Pte Ltd. 2018
S. Roy Choudhury, Pediatric Surgery, https://doi.org/10.1007/978-981-10-6304-6_22
22
Incidence
Omphalocele was considered the most com-
mon abdominal defect with an incidence of
1–2/5000 live births. Recently, gastroschisis
has become more common with an incidence
of 1–2.5/5000 live births. This increase has
been attributed to increase premature deliver-
ies and better survival of preterm babies as this
disease is more common in preterm babies.
Both these defects affect male more often than
female.
Other epidemiological associations seen are:
• Omphalocele: Both young and advanced age
group on mothers and maternal obesity
• Gastroschisis: Young maternal age, low-­
socioeconomic status, and primigravida
status
Embryology
Embryogenesis of the Defects
The anterior body wall is formed by proper for-
mation and fusion of the caudal, lateral, and
cephalic folds. Isolated omphaloceles represents
a lateral fold defects and are always at the umbi-
licus. The rectus muscles often insert far apart on
the costal margins. The aberrations occur early in
137
138
the embryogenesis thereby affecting other organ
systems as well, so children with omphalocele
frequently have associated anomalies. Cephalic
fold is related to the heart and septum transver-
sum therefore defects result in ectopia cordis or
the pentalogy of Cantrell. Similarly caudal fold
defects cause bladder and cloacal exstrophy as
caudal folds are related to the developing bladder.
Gastroschisis is a defect that lies in the failure of
the umbilical coelom to develop. Umbilical coe-
lom normally accommodates the developing
intestines which grows faster than what the
abdominal cavity would accommodate. The
intestine therefore ruptures through the abdomi-
nal wall. The classical location of the defect just
to the right of the umbilicus can be explained as
this site is relatively unsupported as a result of
resorption of the right umbilical vein at about
4 weeks’ gestation. In gastroschisis, the bowel is
usually thickened, matted, edematous, and cov-
ered with a fibrinous peel. This transformation in
the bowel is considered a postnatal event as the
bowel is normal looking at birth, and probably
exposure of the bowel to the air and mesenteric
venous compression at the umbilical ring results
in this thickened matted bowel. Other proposed
theories for gastroschisis include partial blockage
of the right omphalomesenteric artery and rup-
ture of hernia of umbilical cord, but these theo-
ries have not been widely accepted.
Umbilical cord hernia is simple failure of the
midgut to return to the peritoneal cavity at
10–12 weeks. Thus the defect contains midgut
and is covered by a membrane. Such hernias are
much smaller than omphalocele and have a better
outlook.
Antenatal Management
of Abdominal Wall Defects
Accurate prenatal diagnosis is important for
planning the management and in explaining the
prognosis. Omphalocele is seen as a protrusion of
viscera including the liver from the umbilicus
which is covered by a sac. In gastroschisis no sac
is seen covering the viscera. In hernia of the
umbilical cord, there is absence of the liver in the
22 Abdominal Wall Defects
sac. Alpha-fetoprotein and acetylcholine esterase
are also elevated in these abdominal wall defects
provided open neural defects have been ruled out.
Chromosomal analysis and fetal echocardiogra-
phy is recommended in the antenatal period, and
if the abnormalities detected are incompatible
with life, then the fetus is terminated.
Sensitivity of ultrasound and maternal serum
alpha-fetoprotein screening is approximately
80%. Ruptured omphalocele can be differenti-
ated from gastroschisis by the presence of the
liver in the defect and large size of the defect.
Counseling about fetal morbidity and mortal-
ity should include details of the following:
• Nature of primary defect
• Associated anomalies
• Genetic syndromes
If there are no associated lethal defects, then
pregnancy is carried till term with close
monitoring:
• Serial ultrasound evaluation every 2–4 weeks
to evaluate fetal growth, amniotic fluid
volume.
• Fetal physiological assessment by biophysical
profile and nonstress monitoring in the third
trimester due to the increased risk of intrauter-
ine death.
• Consultation at a tertiary level center with
maternal fetal medicine, neonatology, and
pediatric surgery.
Although there are occasional reports of
bowel injury during normal vaginal delivery,
there is no data to favor or disfavor normal vagi-
nal delivery. The mode is best decided by the
treating obstetrician in consultation with the
pediatric surgeon.
Associated Abnormalities
Gastroschisis
• The most common abnormalities associated
are gastrointestinal like intestinal atresia
which can occur in up to 30% patients. Other
organ systems are rarely involved.
Surgical Repair 139

• Gastroesophageal reflux and undescended tes- Post-delivery Management

tis is also common in gastroschisis. of Omphalocele and Gastroschisis
• Higher risk of a stillborn baby is also associ-
ated with gastroschisis. The principles of management of abdominal
defects are as follows:
Omphalocele
• Multiple anomalies are seen in 67–88% of • Reduce the viscera.
fetuses with omphalocele. • Close the defect.
• Cardiac anomalies are present in 50% and gas- • Identify and treat the associated
trointestinal anomalies in 40%. Musculoskeletal, abnormalities.
genitourinary, and central nervous system • Supportive nutrition till the child is able to
anomalies are commonly associated as well. accept full enteral feeds.
• Chromosomal anomalies are associated in • To recognize and treat wound or bowel-related
30–40%, trisomy 13, 18, and 21, sex chromo- complications.
some abnormalities: Turner syndrome (45X),
Klinefelter syndrome (47, XXY), and trip-
loidy (69XXX). Initial Management
• Chromosomal anomalies are more likely with
omphaloceles containing the bowel only. • Nasogastric tube decompression.
• Genetic syndromes that are associated with • Rectal stimulation to evacuate meconium.
omphalocele: Beckwith-Wiedemann syn- • IV hydration (150 ml/kg, larger amount of fluid
drome, pentalogy of Cantrell, and bladder required for gastroschisis than omphalocele).
cloacal exstrophy. • Urethral catheter is inserted for urinary output
• Half of the fetuses are lost. charting.
• Temperature maintenance.
Clinical differences between omphalocele and gas- • Cardiology evaluation and echocardiography.
troschisis (Figs. 22.1a, b and 22.2a, b) • The exposed bowel in gastroschisis needs to
be covered by a bowel bag, or a makeshift
arrangement can be done by using a blood
Omphalocele Gastroschisis transfusion bag or urine collection bag.
Location Umbilical ring Lateral to cord, • It is necessary to ensure that the defect in gas-
right side
troschisis is not very narrow to compromise
Fascial defect Variable Small (<4 cm)
(4–10 cm)
the vascularity of the eviscerated bowel. Any
Sac Present, may be Absent, no
traction of the mesentery of the bowel is also
ruptured remnants avoided.
Cord attachment On to the sac Normal
Intestinal Usually normal Edematous,
appearance thickened Surgical Repair
Herniated liver Often present Unusual
Abdominal cavity Small Usually near Following options can be used for closure of the
normal
defect:
Associated Common Rare
anomalies (40–80%)
• Reduction and primary closure of defect
Bowel Uncommon Greater risk
strangulation (Fig. 22.3a, b)
Bowel atresia Rare Common • Without prosthetic mesh.
Associated Rare Common • With prosthetic mesh: The mesh can result
intellectual in complications and will require removal
Anomalies later.
140 22 Abdominal Wall Defects

a b

Fig. 22.1 (a) Gastroschisis (exposed bowel, no sac). (b) Gastroschisis (rare left-sided defect)

a b

Fig. 22.2 (a, b) Omphalocele with sac containing bowel and liver; note the attached umbilical cord
General Principles of Abdomen Closure 141

a b

Fig. 22.3 (a) Scar following conventional closure. (b) Cosmetic scar following closure of gastroschisis

• Gradual reduction of bowel and closure of

defect
• Preformed silastic silo (can be applied bed-
side and is held in place by a preloaded
spring)
• Custom-made silo (silo made by a pros-
thetic mesh sutured to the fascial defect)
(Fig. 22.4)

The silo is reduced by gravity and/or by grad-

ually decreasing the size of silo. When the bowel
can be completely reduced easily, primary clo-
sure can be done.
Fig. 22.4 Silo closure of gastroschisis

• Creation of ventral hernia and delayed repair

• Only skin closure over the defect
• Eschar formation over the omphalocele sac
either spontaneously or by application of
mercurochrome or silver sulfadiazine
(Fig. 22.5a, b)
Delayed closure is often difficult as the viscera
“grows into the hernia” making it larger and
difficult to reduce as the age of the child pro-
gresses. Tissue expanders may be needed to
increase the capacity of the abdomen to accom-
modate viscera and to achieve proper skin
cover.
 eneral Principles of Abdomen
G
Closure
• Reduce the bowel first and the liver last as the
liver tends to keep the reduced bowel in place.
• To aid in reduction, it is helpful if the bowel
can be decompressed by gastric aspiration,
rectal washes, and milking.
• If closure is still difficult, the abdominal wall
can be gently stretched by inserting a finger
into the defect and stretching the wall by
sweeping the finger posteriorly to anteriorly.
142
a b
Fig. 22.5 (a) Mercurochrome (0.2%) painting. (b) Healing by epithelization following escharification treatment
• The defect should be closed with interrupted
suture.
• Relaxing incisions can be given over the
abdominal wall fascia if the closure seems
under tension.
• It is necessary to ensure that the abdominal
closure is not under extreme tension, other-
wise abdominal compartment syndrome may
result. The following parameters favor silo
formation instead of primary closure:
–– Mean airway pressure more than 25 cm of
water
–– Intravesical pressure more than 20 cm of
water
–– Saphenous intravenous line unable to flow
by gravity
• Signs of abdominal compartment syndrome
like persistent tachycardia, oliguria,
increased ventilator requirements, and tense
abdomen should be detected early. Such
patients need urgent decompression and silo
formation.
 mphalocele Care During Surgical
O
Closure
• Omphalomesenteric duct remnants are fre-
quently present.
• Hepatic veins above and bladder below are at
risk of injury.
22 Abdominal Wall Defects
• Kinking of the hepatic veins following forced
reduction.
• Sac is often adhered to the liver. Attempt to
separate the sac from the liver may cause
excessive bleeding and better be left attached.
Complications
• Decreased venous return
• Abdominal compartment syndrome
• Decreased pulmonary compliance
• Renal failure
• Necrotizing enterocolitis
 anagement of Intestinal Atresias
M
with Gastroschisis (Incidence 10%)
• Delayed repair of atresia 4–6 weeks after
­primary abdominal wall closure
• Initial ostomy (for distal atresia or perfora-
tion) and delayed bowel anastomosis
• Primary bowel anastomosis for healthy bowel
(rare)
 anagement of Postoperative
M
Dysmotility of Bowel
• Bowel motility may take a long time to recover.
• Start early enteral small-volume (trophic)
feeding as tolerated.
Bladder and Cloacal Exstrophy
• Central venous line with TPN needs to be
instituted and continued as required.
• Prokinetic drugs like erythromycin, metoclo-
pramide, and domperidone have variable
success.
Cryptorchidism is associated with gastroschi-
sis with an incidence of 15–30%.
Replacement of the herniated testis into the
abdominal cavity will result in normal testicular
descent into the scrotum in the majority of cases.
Beckwith-Wiedemann Syndrome
• Macroglossia, gigantism, omphalocele, and
visceromegaly. Incidence 1 in 13,700 births.
Mode of inheritance: controversial.
• Ultrasound findings: macroglossia, umbilical
anomalies, polyhydramnios, enlarged pla-
centa, omphalocele, visceromegaly involving
the liver, kidney, and spleen.
• Complications: BWS increased risk of birth
trauma, hypoglycemia, congestive heart fail-
ure, and respiratory distress.
• The prognosis is poor.
Pentalogy of Cantrell
• Omphalocele, ectopic heart, pericardial or
pleural effusions, intracardiac abnormalities,
a b
Fig. 22.6 (a, b) Cloacal exstrophy: prolapsed ileum in the midline (elephant trunk appearance), hemi-bladder on two
sides and exomphalos
143
ascites, two-vessel cord, scoliosis, craniofa-
cial abnormalities.
• Chromosomal anomalies such as trisomy 13
and 18 and Turner syndrome may be
associated.
• Most cases have a fatal outcome.
Amniotic Band Syndrome
• Spectrum of constrictions, amputations, and
slash deformities due to amniotic bands.
• 1 in 200 births. Sonographic findings:
Multiple, asymmetric, and bizarre craniofa-
cial, limb, and abdominal wall defects. Large
body wall defects omphaloceles, gastroschi-
sis, and bladder exstrophy.
• Prognosis: Most infants with severe craniofa-
cial deformities have fatal outcome, while
infants with isolated limb defects have favor-
able prognosis.
 ladder and Cloacal Exstrophy
B
(Fig. 22.6a, b)
• Midline defects infraumbilical ventral wall
causing herniation of the bladder and hindgut.
• 1 in 33,000 live births, cloacal exstrophy 1 in
200,000–400,000 live births.
• Sonographic findings infraumbilical abdominal
wall defect with an irregular soft tissue mass.
144 22 Abdominal Wall Defects

• Non-visualization of the normal fluid-filled

urinary bladder, low umbilical cord
insertion.
• Associated with OEIS complex: omphalocele,
bladder exstrophy, imperforate anus, and spi-
nal anomalies.
• The prognosis of bladder exstrophy is favor-
able, but cloacal exstrophy is not favorable.

Prune belly syndrome consists of deficiency of

abdominal muscles, hydroureteronephrosis, and
cryptorchidism (Fig. 22.7).

Prognosis and Outcome

• The clinical outcome of antenatally diagnosed

abdominal diseases appears to be favorable
Fig. 22.7 Prune belly syndrome with postnatal treatment (Table 22.1).
• Fetal karyotyping should be offered in case of
a fetal abdominal wall defect.

Table 22.1 Algorithm

of antenatal management
of abdominal wall Abdominal wall
defects defects

Antenatal Postnatal
diagnosis diagnsis

associated fatal no major

abnormalities abnormality

close
consider monitoring post natal
termination management
term delivery
Suggested Reading
• Early and close prenatal consultation of the
obstetrician, neonatologist, and the pediatric
surgeon will favorably influence the perinatal
outcome.
• Survival of newborns with gastroschisis is
above 90% with excellent long-term
outcome.
• Major causes of death are prematurity, sepsis,
and bowel ischemia.
• Omphalocele is associated with higher mor-
tality (30–70%), especially those with
145
a­ssociated chromosomal, cardiac, or other
major anomalies.
Suggested Reading
Puri P, Hollwarth ME, editors. Pediatric surgery, diagno-
sis and management. Heidelberg: Springer; 2009.
Coran AG, Caldamone A, Scott Adzich N, Krummel TM,
Laberge JM, editors. Pediatric surgery. 7th ed. Oxford:
Elsevier; 2012.
 Abdominal Tuberculosis
in Children
Abdominal tuberculosis (ATB) in children is still
prevalent in the developing nations with the
reported incidence of 0.9–1.95% of all pediatric
hospital admissions. The diagnosis of ATB in
children is elusive and challenging as there are no
distinct clinical signs or symptoms or any spe-
cific diagnostic test. The final diagnosis is often
made after invasive surgical interventions like
laparotomy or laparoscopy. Abdominal tubercu-
losis is generally due to infection by
Mycobacterium tuberculosis and rarely due to
Mycobacterium bovis or other atypical mycobac-
teria. It could be an isolated disease or in associa-
tion with extraintestinal tuberculosis. The source
of infection could be due to ingestion of tubercle
bacilli or hematogenous or contiguous spread
from other focus. The damage to the tissue occurs
through Type IV hypersensitivity reaction where
the tubercular bacillus activates the immune sys-
tem and results in formation of epithelioid granu-
lomas with caseous necrosis of the tissues. The
order of involvement of various sites is the ileum,
ileocecal region, colon, jejunum, rectum, duode-
num, stomach, and esophagus.
 ypes of Abdominal Tuberculosis
T (Fig. 23.3a–c).
in Children
1. Tuberculosis of mesenteric lymph nodes
2. Peritoneal tuberculosis
(a) Wet: ascitic
(b) Dry: plastic, adhesive type
© Springer Nature Singapore Pte Ltd. 2018
S. Roy Choudhury, Pediatric Surgery, https://doi.org/10.1007/978-981-10-6304-6_23
23
(c) Miliary tuberculosis: granular peritoneal
surface (Fig. 23.1a, b)
(d) Omental: mass, cake, miliary nodules
(Fig. 23.2)
3. Intestinal tuberculosis
(a) Ulcerative
(b) Hypertrophic
(c) Ulcerohypertrophic
(d) Miliary
4. Tuberculosis of solid organs like the liver,
spleen, etc.
Clinical Features
Symptoms of abdominal tuberculosis are often
nonspecific like abdominal pain, anorexia, fever,
weight loss, diarrhea, vomiting, constipation, and
cough.
Presenting signs could be variable like abdom-
inal distension, visible peristalsis, palpable lump,
doughy abdomen, ascites, umbilical involvement,
hepatosplenomegaly, enterocutaneous fistula,
and lymphadenopathy. Umbilical involvement in
intra-abdominal tuberculosis can be in the form
of puckering, inflammation, and fecal fistula
Diagnosis
In children who present with subacute intestinal
obstruction, tuberculosis should always be
147
148
a b
Fig. 23.1 (a, b) Miliary tuberculosis on the bowel
Fig. 23.2 Disseminated tuberculosis on the peritoneal
surface and liver (laparoscopic view)
c­ onsidered in the differential diagnosis especially
if there is previous history of tuberculosis in the
child or his family. The diagnosis essentially relies
on demonstrating the acid-fast bacilli (AFB) in tis-
sue. Since abdominal tuberculosis is a paucibacil-
lary disease, often AFB cannot be demonstrated in
the tissue or the sputum. The presence of caseating
granuloma with epithelioid giant cells in the tissue
specimen of a patient with high suspicion of tuber-
culosis can be considered diagnostic. In the era of
image-guided biopsy and availability of laparos-
copy, a genuine attempt should be made to achieve
a histopathological diagnosis of tuberculosis
before initiating antitubercular therapy.
23 Abdominal Tuberculosis in Children
Investigations
Hematological findings may be nonspecific but
may show anemia with raised ESR. ELISA test
for detecting the antitubercular antibody are non-
specific and should not be performed.
A positive tuberculin (Mantoux) test does not dif-
ferentiate between an active or inactive infection.
Abdominal fluid examination will reveal an
exudative fluid with increased lymphocytes.
Rarely AFB can be seen on a smear although
they may be cultured from the ascitic fluid.
Increased levels of enzyme adenosine deami-
nase (more than 33 U/L) in ascitic fluid have
high sensitivity and specificity for detecting
tuberculosis; however, the test is not freely
available. The Gene X pert MTB/RIF
(Myccobacterium Tuberculosis/ Resistance to
Rifampicin) is a nucleic acid amplification test
and detects MTB by its DNA sequencing
(Cepheid Inc. Synnyvale, CA, USA). It has been
applied for analysis of sputum sample in the
diagnosis of pulmonary tuberculosis with high
sensitivity and specificity. It is a rapid test
(approximately 2 hours) and can diagnose MTR
TB. However few reports of Gene X pert MTB/
RIF assay for the diagnosis of abdominal tuber-
culosis are now available. Ascitic fluid has a low
yield of tubercular bacilli, hence has a poor
detection rate.
Complications
a b
Fig. 23.3 Umbilical involvement in intra-abdominal tuberculosis: (a) puckering, (b) inflammation (c) fecal fistula
Radiological Investigations
Chest radiograph may show sign of previous or
active tubercular infection.
Abdominal radiograph may show multiple air
fluid levels if the child has intestinal obstruction
due to stricture or adhesions. Ascitic form may
reveal a gasless abdomen with diffuse haziness
due to ascitic fluid. Rarely calcification may be
seen.
Ultrasound of the abdomen can be useful in
the diagnosis, and the following findings may be
seen in abdominal tuberculosis:
• Presence of echogenic or septate fluid in the
abdomen
• Thickening and nodularity of the peritoneum
• Thickening of the mesentery with increased
echogenicity
• Enlarged mesenteric or retroperitoneal lymph
nodes (size >1 cm)
• Thickened and dilated bowel loops
• Matted thickened bowel loops especially in
the ileocecal region
Barium meal follow through (BMFT) is rarely
necessary for the diagnosis.
• It may demonstrate an ileal stricture with
proximal dilatation of the bowel loops.
• Pulled-up deformed cecum.
• Abnormal ileocecal valve area.
• Irregular mucosal folds with ulceration.
• Loss of normal ileocecal angle.
149
c
Contrast-enhanced computed tomographic
scan (CECT) is a useful adjunct for detecting the
following intra-abdominal abnormalities:
• Thickened dilated bowel loops
• Ileocecal thickening
• Dense peritoneal fluid
• Thickened omentum, mesentery, and peritoneum
• Hypodense lesions in solid organs suggestive
of miliary tuberculosis
Histopathological Diagnosis
An enlarged peripheral lymph node if present can
be subjected to FNAC or biopsy.
Image-guided biopsy or laparoscopy can be
done to retrieve tissue samples for histo- or cyto-
pathological examination.
Histopathological hallmarks for diagnosis of
tuberculosis are caseating epithelioid cell gran-
uloma, Langhans giant cells, and acid-fast
bacilli.
Often an indirect evidence of the disease is the
response to antituberculous therapy.
Complications
The complication of abdominal tuberculosis
includes intestinal obstruction from adhesion or
stricture, intestinal perforation, intraperitoneal
abscess, cocoon formation, fistulization, hemor-
rhage, malabsorption, and malnutrition.
150
Treatment
Medical treatment consists of antituberculous
chemotherapy. As per Revised National
Tuberculosis Control Programme (RNTCP),
abdominal tuberculosis is category 1 (severe
form of extrapulmonary tuberculosis). Four drugs
are recommended with isoniazid, 5 mg/kg/day;
rifampicin, 10 mg/kg/day; ethambutol, 15 mg/kg/
day; and pyrazinamide, 20–25 mg/kg/day for
2 months, followed by two drugs, isoniazid and
rifampicin with the same dose as above for a total
of 6–9 months.
For strict compliance direct observation of
drug intake (DOTS) regime is Category – 1: 2
(H3 R3 Z3 E3) 4 (H3 R3) means initial 2 months
of therapy with isoniazid, rifampicin, pyrazin-
amide, and ethambutol in a thrice weekly sched-
ule followed by INH and rifampicin for a period
of 4 months in a thrice weekly schedule.
Indications for Surgery
• Complications arising out of tuberculosis like
bowel stricture, intestinal perforation,
obstruction, or massive uncontrolled
hemorrhage
• Diagnostic biopsy by open or laparoscopic
approach
Surgical Procedures
Findings of disseminated tuberculosis on the
bowel and peritoneal surface with adhesions at
23 Abdominal Tuberculosis in Children
surgery are best left alone with only taking peri-
toneal biopsy for confirmation of the diagnosis.
Extensive bowel handing and adhesiolysis in
such situations should be avoided as there are risk
of bowel injury and resultant fecal fistula leading to
prolonged morbidity. Postoperative a­ ntituberculous
therapy should be started. Patients on intravenous
fluids can be given injection with streptomycin and
ciprofloxacilin before switching over to oral antitu-
berculous therapy.
Stricture involving a short segment of the
bowel can be managed with stricturoplasty mean-
ing the stricture is opened longitudinally and
closed transversely. More extensive strictures
may require resection and end-to-end anastomo-
sis of the intestine.
Localized intestinal perforation can be man-
aged with closure or resection and anastomosis
of the bowel if the edges are found healthy. There
is a high chance of postoperative leak and fecal
fistula formation. Hence, for grossly diseased
bowel with peritonitis, an initial bowel ostomy
(ileostomy) could be an extremely useful salvage
procedure. Subsequent closure of the stoma can
be electively performed on a later date after an
adequate period of therapy with antituberculous
drugs.
Suggested Reading
World Health Organization. Guidance for national tuber-
culosis programmes on the management of tuberculo-
sis in children. 2nd ed. WHO.
Pant N, Choudhury SR, Gupta A, et al. Umbilical signs
of peritoneal tuberculosis in children. Indian J Pediatr.
2012;79:1192.
 Splenic and Liver abscess
24

Splenic abscess (SA) is rare in children. The
causes of SA are diverse. The current management
of SA in children aims at splenic preservation.
Etiology of Splenic Abscess
cal definition although exposing the patient to
higher radiation. US not only clinches the
­diagnosis in SAs but also helps in guiding aspira-
tion of the abscess and to follow-up the progress
during recovery.

The causes or associations of SA are hematoge- Management of Splenic Abscess

nous spread of infection from other parts of the
body, immunocompromised state, hematological
disorders, malignancy, trauma, and diabetes.
Enteric fever, tuberculosis, and parasitic diseases
are more frequently reported etiologic factors
from developing countries.
Clinical presentation is generally with fever,
anorexia, and pain in the left upper quadrant.
Occasionally patient may have referred pain to
the left shoulder due to irritation of the left
phrenic nerve.
The management of SA is still elusive. Various
treatment modalities like antibiotic therapy, per-
cutaneous drainage, open drainage, and partial or
total splenectomy, alone or in various combina-
tions, have been reported. Historically total sple-
nectomy was advocated for SA. Preservation of
the spleen is important in children because of its
immunologic function.

Imaging Studies

Cases of SA are now diagnosed more often due to

widespread use of imaging modalities with ultraso-
nography (US) and computerized tomography
(CT) scan. The ultrasound features include
hypoechoic lesion in the spleen with irregular mar-
gin and echogenic contents. Similarly, CT scan
shows a hypodense area in the spleen (Figs. 24.1
and 24.2a, b). There may be single or multiple SAs.
CT scan has been reported to have a higher
sensitivity than US and provides better anatomi- Fig. 24.1 CT scan showing solitary splenic abscess

© Springer Nature Singapore Pte Ltd. 2018 151

S. Roy Choudhury, Pediatric Surgery, https://doi.org/10.1007/978-981-10-6304-6_24
152
a b
Fig. 24.2 (a) Multiple splenic abscesses (b) CT scan showing multiple splenic abscesses
Antibiotic therapy and percutaneous aspiration
or drainage are now recommended as the first line
of choice in the treatment of both s­ olitary and mul-
tiple SAs in children. Splenectomy may be
reserved for patients who fail aspiration or drain-
age or in cases of rupture of a SA. Many children
respond favorably to conservative treatment with
intravenous antibiotics only (abscess size <3 cm).
For larger abscess (abscess size >3 cm), US-guided
percutaneous needle aspiration (PNA) is safe, free
from complication, and usually curative. Even per-
cutaneous drainage with pigtail catheter has been
reported with high therapeutic success rate at low
costs compared to surgery. Splenic preservation
has an added advantage in children. Percutaneous
catheter drainage has its inherent problems like
catheter slippage, blockage, and infection.
Liver Abscess
Liver abscess (LA) in children is more commonly
reported from the developing countries.
Incidence
The incidence of LA varies from different coun-
tries. Overall, pyogenic liver abscess (PLA) is
more common (80%) than amebic liver abscess
(ALA) in children. ALA is mostly endemic in
Southeast Asia, Egypt, and South Africa.
24 Splenic and Liver abscess
Etiopathogenesis
Predisposing Factors
Historically perforated appendicitis was the most
frequently identified cause of liver abscess in chil-
dren. In the developed countries, LAs are seen most
often in children with major debilitating diseases,
granulocyte dysfunction, sickle cell disease, helmin-
thic infections, and congenital or acquired immuno-
suppression. However in endemic zones
malnutrition, poor hygienic conditions, and parasitic
diseases like helminthiasis are predisposing factors.
Several congenital and obstructive diseases of
the biliary tract can lead to bacterial proliferation
and multiple abscesses. Liver trauma is also a
predisposing factor. A large number of LA with-
out any apparent cause have been labeled as cryp-
togenic. The abscess content may be sterile
because of prior antibiotic therapy and inade-
quate culture techniques especially with regard to
anaerobic organisms.
Pathophysiology
The portal of entry of bacteria and other organ-
ism in the liver is either through biliary tract, the
portal vein, or the hepatic artery. Pyogenic liver
abscess is usually single and located in the right
lobe of the liver (75%) due to preferential portal
venous flow to the right lobe. Multiple pyogenic
abscesses and involvement of both lobes are not
uncommon (25%). Rupture of liver abscess can
occur in both lobes.
Investigations 153

Amebic liver abscess also most frequently Clinical Presentation

affects the right lobe of the liver. The center of the
abscess contains thick, reddish-brown pus, simi-
lar to anchovy or chocolate sauce. Secondary
bacterial infection may occur converting the pus
to green or yellow in color. Amoebae may be
identified in the abscess wall scrapings. It is often
not possible to differentiate a pyogenic from an
amebic liver abscess either clinically or radio-
logically. A mixed microbial infection is more
commonly reported from India.
Causative Organisms
The usual presenting features of LA in children
are fever, abdominal pain, and tender hepatomeg-
aly (Table 24.1). Subdiaphragmatic irritation or
pleura-pulmonary spread of LA may lead to pain
in the right shoulder and/or cough. A frequent
associated finding in children is pleural effusion
or empyema due to contagious spread. An insidi-
ous presentation is more common although some
cases may present with fulminant sepsis or an
acute abdomen due to rupture or contagious
spread of the abscess.

Staphylococcus aureus is the most common Investigations

pathogenic organism isolated in LA followed by
E. coli, Klebsiella, Enterobacter, Salmonella
typhi, and anaerobes; however, tubercular LA is
rare. Patients with leukemia are prone to fungal
hepatic and splenic microabscesses.
Complete blood counts may show leukocytosis
and a raised erythrocyte sedimentation rate
(ESR). Liver enzymes like serum alkaline
phosphatase, serum glutamate-oxaloacetate

Table 24.1 Diagnostic protocol for liver abscess

Diagnostic protocol

fever abdominal pain vomiting diarrhoea

Tenderness in right hypochondrium Heptomegaly

USG-abdomen-hypoechoic lesions in liver X-ray chest-any pleual/pericardial effusion

Aspiration of pus: Final Diagnosis-Liver abscess

Categorization of patient for protocol based treatment

154
t­ransaminase (SGOT), and serum glutamate-
pyruvate transaminase (SGPT) levels may be
raised. Coagulation parameters (prothrombin
time, international normalized ratio) may be
deranged. Blood cultures are less sensitive than
pus aspirate cultures. The cultures are often nega-
tive due to prior antibiotic administration.
Demonstration of trophozoites in aspirates of pus
from amebic LA is difficult as they are usually
located in the wall of the abscess. Although sero-
logic tests like indirect hemagglutination anti-
body (IHA) titers and enzyme-linked
immunosorbent assay (ELISA) are sensitive
markers to diagnose amebic infection, they have
low specificity in endemic zones. Ultrasound
(US) is widely used for the diagnosis. Contrast-
enhanced computed tomography (CECT) scan
may be required for better anatomical localiza-
tion of the abscess and guides on planning a
drainage procedure (Figs. 24.3 and 24.4a, b). On
magnetic resonance imaging (MRI), LA appears
hypointense on T1-weighted and hyperintense in
T2-weighted sequences.
 reatment of Liver Abscess
T
(Table 24.2)
The treatment of liver abscess constitutes antimi-
crobial therapy and drainage of pus collection.
Fig. 24.3 CT Scan showing a large liver abscess
24 Splenic and Liver abscess
Medical Treatment
Antimicrobial treatment alone may be successful
in some cases, however, combined with percuta-
neous or open surgical drainage, constitute the
mainstay of treatment. Conservative manage-
ment is effective for smaller abscesses (<5 cm),
but for larger abscesses drainage of the pus is
necessary. Percutaneous needle aspiration (PNA)
under US guidance is the best treatment modality
for noncomplicated larger abscesses (>5 cm).
Prior correction of coagulopathy is important.
The choice of antimicrobial agents should pro-
vide broad-spectrum coverage directed toward
the most commonly involved pathogens.
Therefore, a combination of an antistaphylococ-
cal drug, an anti-anaerobic, and anti-­ amebic
a
Fig. 24.4 (a) Chest X-ray showing air in liver abscess
cavity (b) CT scan showing liver abscess with air in it
Open Surgical Drainage 155

Table 24.2 Treatment protocol for liver abscess cases with ruptured LA or failed percutaneous
Treatment protocol aspiration/drainage.
Patient selection Type of treatment PNA along with antimicrobial therapy has
– All uncomplicated cases Medical management been found to be effective in a large percentage of
– Abscess cavity <5 cm only (combination of patients with uncomplicated LA and, hence,
– Abscess with solid higher-­generation should be considered the first line of treatment.
appearance on USG cephalosporin,
– Multiple small lesions aminoglycoside, and Repeated aspirations of liquefied contents as
metronidazole) guided by ultrasonography may be necessary.
– Febrile even after Percutaneous needle Percutaneous drainage (PD) is indicated when:
48–72 h of adequate aspiration with medical
medical treatment with management (repeated (a) Large abscess with impending ruptures or
liquefied abscess aspirations at certain
– Abscess cavity >5 cm interval may be ruptured into the parietal wall (Fig. 24.5a, b)
required) (b) Thick pus in solitary cavity not sufficiently
– Large abscess with Percutaneous aspirated by wide-bore needle
impending rupture or continuous catheter
pointing to the parietal drainage with medical Our preference of PD is with wide-bore (>10
wall management
Fr size) catheter as they provide better drainage
– Abscess ruptured into Open surgical drainage/
peritoneal cavity laparoscopic drainage than pigtail catheters. It is perceived that PD
– Abscess cavity not involves reduced risks as compared to open sur-
accessible by gical drainage, eliminates the need for general
percutaneous drainage anesthesia, requires short hospitalization, and has
– Ongoing sepsis even
after antibiotic therapy better acceptance by patients. However PD cath-
and percutaneous eters are associated with complications like
drainage blockage, infection, and dislodgment.

drug, along with an aminoglycoside or cephalo-

sporin against gram-negative bacilli, is a good
initial choice for antimicrobial therapy. Since a
mixed microbial infection is common, such a
combination therapy is very effective in the treat-
ment. Isolation of organisms and their antibiotic
sensitivities should guide the final choice of anti-
microbial drug therapy. Currently 4–6 weeks of
antimicrobial therapy is recommended for soli-
tary lesions that have been adequately drained.
Multiple LA may require up to 12 weeks of ther-
apy. Both the clinical and radiographic progress
of the patient should guide the duration of antimi-
crobial therapy, although radiological canges
may persist longer even after recovery.
Surgical Treatment
With the refinement of image-guided techniques,
percutaneous aspiration and drainage have
become the accepted standards of management
of LA with open surgical drainage reserved for
Open Surgical Drainage
Open surgical treatment has an important role to
play in the management of LA in cases of:
(a) Ruptured abscess with peritonitis
(b) Failed percutaneous drainage, especially in
severely sick patients with ongoing sepsis
(c) Inaccessible area for image-guided drainage
Laparoscopic drainage can be a less invasive
and effective alternative to open surgical drainage.
In our experience with treating a large number
of LA patients, differentiation between the two
types of LA is often not possible and does not
seem to have much of clinical significance. A
mixed microbial infection is more likely, and a
combination of antimicrobial therapy against
pyogenic and amebic pathology results in satis-
factory outcome.
For suspected fungal abscess, systemic anti-
fungal agents should be initiated after the abscess
156
a b
Fig. 24.5 Left lobe liver abscess, (a) ruptured in the parietal abdominal wall, (b) CT scan showing two liver abscesses
with the left lobe abscess ruptured into the parietal wall
has been drained percutaneously or surgically.
Initial drug therapy of choice for fungal LA is
currently amphotericin B and fluconazole.
Complications and Prognosis
The presence of jaundice, liver failure, acute
abdomen and sepsis, bilirubin levels >3.5 mg/dl,
deranged prothrombin time, international nor-
malized ratio (PT/INR), encephalopathy, and
hypoalbuminemia (<2 g/dl) are indicators of poor
prognosis. Pleural effusion and empyema fre-
quently complicate liver abscess. Majority on
these patients respond to aspiration or tube drain-
24 Splenic and Liver abscess
age of pleural collection. A mortality rate of 3%
has been reported in a large series from India;
however, there are no long-term morbidities in
the survivors.
Suggested Reading
Zinner MJ, Ashley SW. Maingot’s abdominal operation.
12th ed. New York: McGraw Hill; 2013.
Roy Choudhury S, Khan NA, Saxena R, et al. Protocol
based management of 154 cases of pediatric liver
abscess. Pediatr Surg Int. 2017;33:165–72.
Roy Choudhury S, Debnath PR, Jain P, Kushwa AK, Puri
A, Chadha R, Sonkar P. Conservative management of
isolated splenic abscess in children. J Pediatr Surg.
2010;45:372–5.
 Lesions of the Pancreas and Spleen
Pancreatic lesions in children are diverse and
could be due to developmental and acquired con-
ditions affecting the gland.
Diseases of the Pancreas in Children
• Annular pancreas (see duodenal obstruction)
• Pancreas divisum and ductal anomalies
• Acute pancreatitis
• Chronic pancreatitis
• Pancreatic cysts and pseudocysts
• Neoplasms
• Endocrine abnormality and persistent hyper-
insulinemic hypoglycemia of infancy
• Cystic fibrosis
Acute Pancreatitis
Acute pancreatitis is an acute inflammation of the
pancreas and is clinically defined as the presence
of abdominal pain with elevated pancreatic
enzymes. The various possible causes are listed
below.
 auses of Acute Pancreatitis
C between 50 and 85%.
in Children
Idiopathic
Choledocholithiasis
© Springer Nature Singapore Pte Ltd. 2018
S. Roy Choudhury, Pediatric Surgery, https://doi.org/10.1007/978-981-10-6304-6_25
25
Infections: mumps, rubella, and Coxsackie B
virus
Traumatic, post-liver transplantation
Congenital anomalies: pancreas divisum, chole-
dochal cyst, and pancreaticobiliary malunion
Drugs: azathioprine, tetracycline, l-asparagi-
nase, and valproic acid
Metabolic: hypercalcemia and hypertriglyceri­
demia
Diagnosis
Diagnosis depends on clinical, biochemical, and
radiological investigation. The diagnosis of acute
pancreatitis is entertained if two of these are sug-
gestive of pancreatitis.
Clinical: Acute pancreatitis has a wide spec-
trum of presentation. It usually presents with epi-
gastric pain, nausea, vomiting, anorexia, and
abdominal distension. In severe cases the presen-
tation is that of a multisystem organ failure.
Biochemical: The most useful test is elevated
pancreatic enzymes, amylase and lipase, which
are usually elevated more than three times the
normal value. Though they are the most reliable
biochemical marker, still their sensitivities range
Radiological: Ultrasonography is the initial
preferred investigation and will show edema of
the pancreas with or without peripancreatic fluid
collection. It also detects the presence of dilated
pancreatic or biliary ducts and calculus.
157
158 25 Lesions of the Pancreas and Spleen

Magnetic resonance cholangiopancreatography hypertension due to splenic vein thrombosis

(MRCP) provides better delineation of the anat- or due to pancreatic pseudoaneurysm result-
omy. Endoscopic retrograde cholangiopancrea- ing in hematemesis, hemobilia, or
tography (ERCP) is usually reserved for hemoperitoneum.
therapeutic interventions like sphincterotomy,
stenting, etc.
Diagnosis

Treatment Imaging by means of USG, CECT, or MRCP

reveals a small atrophic pancreas with calcifi-
Medical management is successful in majority of cation and a dilated duct. The pancreatic duct
the patients. Conservative treatment includes nil may have multiple strictures and stones
by mouth, nasogastric aspiration, intravenous (Fig. 25.1).
hydration, and parenteral hyperalimentation to Surgical treatment for chronic relapsing pan-
provide rest to the inflamed gland. Drugs like creatitis includes:
analgesics, antibiotics, histamine receptor block-
ers, proton pump inhibitors, somatostatin ana- • Distal pancreatectomy: if the pathology is
logues, and glucagon are also used. Surgical limited to the tail of the pancreas
debridement of the pancreas may be required for • Sphincterotomy (pancreas divisum) and
pancreatic necrosis or infection. sphincteroplasty
Anatomical abnormalities may require surgi- • Longitudinal pancreaticojejunostomy
cal correction: (Puestow) (Fig. 25.2) and distal pancreaticoje-
junostomy (Duval)
• Biliary pancreatitis: ERCP and stone removal • Pancreatectomy
are recommended followed by cholecystec-
tomy after 2–4 weeks of resolution of the
disease.
• Sphincter of Oddi dysfunction: ERCP and
sphincterotomy may be curative.
• Choledochal cyst and pancreatobiliary mal-
union: Excision of cyst and hepaticojejunos-
tomy should be done.
• Pancreas divisum: The condition can be
treated with endoscopic or surgical dilatation
of the minor papilla or sphincterotomy.

Chronic Pancreatitis

Chronic pancreatitis results from repeated inflam-

mation of the pancreas which results in irrevers-
ible progressive damage to the pancreas.
Causes in children are trauma, hereditary and
metabolic disorders, congenital anomalies like
pancreas divisum, and choledocholithiasis.
The most common presentation is with
persistent or recurrent abdominal pain. Other
uncommon presentation can be due to portal Fig. 25.1 Multiple pancreatic calculi
Persistent Hyperinsulinemic Hypoglycemia of Infancy (PHHI) 159

Fig. 25.3 Large upper abdominal lump due to pseudo-

cyst of the pancreas

Fig. 25.2 Longitudinal pancreaticojejunostomy

(Puestow) for chronic pancreatitis with dilated duct con-
taining multiple stones

Pancreas Divisum

In this condition the minor pancreatic duct of

Santorini becomes the main drainage for the pan-
creas, and the major duct of Wirsung becomes
attenuated or rudimentary. Treatment consists of
adequate drainage of the duct of Santorini (minor
papilla) by endoscopic sphincterotomy. In case of
restenosis and a dilated pancreatic duct, longitudi-
nal pancreaticojejunostomy should be considered. Fig. 25.4 CT scan showing pancreatic pseudocyst

Pseudocyst of the Pancreas

Pseudocyst of the pancreas is collection of fluid

usually in the lesser sac, walled off by fibrous tis-
sue and lacking an epithelium lining. It often
complicates the course of acute pancreatitis.
Causes in children include trauma, pancreati-
tis, and obstruction of the duct. An upper abdomi-
nal lump may be the presentation (Fig. 25.3).
Ultrasonography and CT scan are useful in mak-
ing the diagnosis (Fig. 25.4). Fig. 25.5 Open cystogastrostomy
Conservative treatment is offered for acute
cases. Generally after a period of 6 weeks, the cyst
either disappears or the cyst wall becomes mature. Persistent Hyperinsulinemic
Persistent pseudocysts after 6 weeks and cyst diam- Hypoglycemia of Infancy (PHHI)
eter larger than 5 cm will require surgical drainage
by either cystogastrostomy (open or endoscopic) or PHHI is a rare but very important cause of severe
cystojejunostomy (Fig. 25.5) depending on the neonatal hypoglycemia. Timely recognition and
proximity of the cyst wall to the viscera. An treatment of this entity become important due to
infected pseudocyst requires external drainage. the fact that severe and frequent hypoglycemia
160
may lead to severe neurological damage or even
a life-threatening event. Near-total pancreatec-
tomy may be required for patients with intracta-
ble hypoglycemia despite medical treatment.
This may result in diabetes mellitus or recurrent
postoperative hypoglycemia.
Diagnosis of PHHI
• Nonketotic hypoglycemia (blood sugar levels
<36 mg/dL), both fasting and postprandial.
The definition of hypoglycemia will depend
upon the gestational age in the case of
neonates.
• Hyperinsulinemia (plasma insulin level
>3 mU/L).
• Need for high rate of glucose infusion
(>10 mg/kg/min) in order to maintain blood
glucose levels more than 54 mg/dL.
• Rise in blood glucose levels in response to
administration of glucagon. Usually the blood
sugar levels rise by 18–36 mg/dL following
administration of 0.5 mg of glucagon by
subcutaneous or intramuscular route. Most
­
other hypoglycemic conditions in infancy
induce a starvation-like state with exhaustion
of the liver glycogen. In such situations, there
is a failure to increase the blood glucose con-
centration after administration of glucagon.
Pathology
The defect is with excess secretion of insulin by
the β-cells due to mutation in the gene encoding
for the ATP-activated K+ channel or the sulfonyl-
urea (SUR) receptors.
Investigations
Biochemical investigations are done to confirm
the diagnosis of PHHI (see above).
Another parameter that affects the manage-
ment of PHHI is whether the disease is local or
diffuse. Rarely MRI can show the presence of
25 Lesions of the Pancreas and Spleen
focal disease. Other ways to confirm the pres-
ence of focal disease is through interventional
radiology where either the selective sampling of
the pancreatic veins or by stimulation of the pan-
creas by specific cannulation of its artery and
detecting insulin in the blood. PET-CT scan
using l-dopa may also help to localize a focal
disease.
Medical management includes high level of
glucose infusion along with drugs. Of the follow-
ing drugs, diazoxide is the first-line drug fol-
lowed by somatostatin.
• Drugs which inhibit insulin secretion (diazoxide,
somatostatin, epinephrine, diphenylhydantoin, and
calcium channel blockers)
• Drugs which antagonize the insulin effect of body
tissues (epinephrine, glucagon, and growth hormone)
• Drugs which destroy islet cells (alloxan)
Surgical treatment is indicated in:
1. Failure of medical management
2. Presence of a focal lesion which is amenable
to surgical resection
3. Poor compliance to medical management
For focal lesions localized excision can be
done. For diffuse lesion near-total pancreatec-
tomy (95–98%) is recommended wherein all of
the pancreas barring the pancreatic tissue
between the duodenum and the common bile
duct is left.
Splenic Disorders in Children
Splenic Cyst
These are epithelial-lined cysts often called epi-
dermoid cysts. Small cysts >5 cm may be
observed. Larger cysts <5 cm or symptomatic
cysts require treatment. Aspiration and sclerosis
may be successful; however, better treatment
option is excision of the cyst wall and
­marsupialization of the remaining cyst either by
open or by laparoscopic approach.
Suggested Reading 161

Splenectomy Persistent monocytosis

Indications for Splenectomy Vaccination and prophylaxis before splenec-

Hereditary spherocytosis: persistence of reticulo-
cyte count more than 10% with moderate to
severe anemia
Immune thrombocytopenic purpura: symp-
tomatic thrombocytopenia unresponsive to medi-
cal management
Sickle cell disease: recurrent or single major
episode of sequestration
Thalassemia: blood transfusion requirement
more than 250 mL/kg/year
Gaucher’s disease: associated with massive
splenomegaly with hypersplenism
Trauma (accidental or operative), rare
Severe hypersplenism with portal hyper­
tension
Hematological Changes Following
Splenectomy
1. Erythrocytes
Howell-Jolly bodies (nuclear fragments)
Heinz bodies (hemoglobin deposits)
Pappenheimer bodies (iron deposits)
Target cells
Spur cells (acanthocytes)
2. Platelets
Transient thrombocytosis
3. Leukocytes
Transient leukocytosis
Persistent lymphocytosis
tomy: For elective splenectomy vaccinations
against pneumococ­cus, haemophilus influenzae
type b, and meningococ­cus are recommended.
All vaccination should be completed 2 weeks
prior to splenectomy except in emergency situa-
tion where vaccination can be given 2 weeks
after. Annual Influenza vaccine is also recom-
mended for asplenic patients. In trauma patients
vaccination can be given in the postoperative
period. Oral antibiotic prophylaxes with penicil-
lin, erythromycin, amoxicillin, or co-amoxiclav
are effective and generally indicated for chil-
dren until the age of 10–15 years.
 omplications of Splenectomy
C
Immediate complications: Hemorrhage, hemateme-
sis, gastric/pancreatic injury, and basal atelectasis.
Delayed complications: Postsplenectomy sep-
ticemia due to bacterial infection from pneumo-
coccus, haemophilus influenzae type b,
meningococcus, and Escherichia coli.
Overwhelming postsplenectomy sepsis (OPSI) is
a major concern during the first 2–3 years of sple-
nectomy in children.
Suggested Reading
Zinner MJ, Ashley SW. Maingot’s abdominal operation.
12th ed. New York: McGraw Hill; 2013.
Puri P, Hollwarth ME, editors. Pediatric surgery, diagno-
sis and management. Heidelberg: Springer; 2009.
 Infantile Obstructive
Cholangiopathy 26

Physiologic jaundice of the newborn is a com- Table 26.1 Causes of conjugated hyperbilirubinemia in
mon, benign, and self-limiting condition. Jaundice infancy
persisting beyond 2 weeks of age with predomi- Extrahepatic causes Intrahepatic causes
nantly conjugated hyperbilirubinemia should • Extrahepatic • Idiopathic neonatal hepatitis
raise a suspicion for pathological condition and biliary atresia • Toxic: TPN, drug induced
• Choledochal cyst • Genetic/chromosomal:
merits detailed workup to differentiate between
• Bile duct stenosis trisomy 18, 21
biliary atresia and neonatal hepatitis (Table 26.1). • Spontaneous • Infectious: TORCH,
The term “infantile obstructive cholangiopathy” perforation of the hepatitis
was suggested by Landing to encompass neonatal bile duct • Metabolic, endocrine
• Cholelithiasis disorders
hepatitis, biliary atresia, and choledochal cyst as a
• Inspissated bile/ • Miscellaneous: Alpha-1-­
spectrum of manifestations. mucus plug antitrypsin deficiency,
Persistent hyperbilirubinemia (>10 mg/dL and • Extrinsic Alagille syndrome, Carolis
lasting for more than 2 weeks) should be urgently compression of the disease, Byler’s disease,
bile duct congenital hepatic fibrosis
evaluated. Initial evaluation should include
detailed history and physical examination, and
biochemical and hematological testing. Children
with acholic stools and yellowish discoloration of Etiology
urine with predominantly conjugated hyperbiliru-
binemia (>20% of total or 1.5 mg/dL) need to be • Occult virus infection by reovirus 3, cyto-
investigated for obstructive cholangiopathy. megalovirus (CMV), rotavirus C, human pap-
illomavirus, and retroviruses probably result
in an immunologic reaction triggered by
Extra Hepatic Biliary Atresia viral-­
induced neoantigens on the biliary
epithelium
Extrahepatic biliary atresia (EHBA) is a rare • Developmental failure of recanalization of
obstructive condition of the bile ducts causing biliary ducts
neonatal jaundice. • Ductal plate malformation theory in which
Incidence is 1 in 10,000 to 1 in 16,700 live births the poorly supported ducts rupture and
with female preponderance, M:F, 1.4 to 1.7:1. the resulting intense fibrosis results in
Associated congenital anomalies are present obliteration
in 11–20% cases. Polysplenia is present in 7.5% • Environmental toxin exposure
cases.

© Springer Nature Singapore Pte Ltd. 2018 163

S. Roy Choudhury, Pediatric Surgery, https://doi.org/10.1007/978-981-10-6304-6_26
164 26 Infantile Obstructive Cholangiopathy

I IIa

IIb III

Types of extrahepatic biliary atresia

Fig. 26.1 Types of EHBA

Based on the etiopathogenesis, EHBA can be
classified into four groups:
1. Syndromic biliary atresia (embryonic type)-
where associated congenital anomalies are
found, such as an interrupted inferior vena
cava, preduodenal portal vein, intestinal mal-
rotation, situs inversus, cardiac defects, and
polysplenia. This type is called BASM syn-
drome (biliary atresia splenic malformation
syndrome). They account for 10–20% cases
with poor outcome.
2. Cystic EHBA-associated with cystic dilata-
tion of the biliary tree.
3. Cytomegalovirus-associated EHBA with
raised IgM anti CMV antibodies.
4. Isolated (non-syndromic) EHBA: Most com-
mon group.
Investigations
Out of these varieties the first two occur dur-
ing prenatal period. However the other two
groups have a prenatal or postnatal onset.
Classification
Morphologic classification of biliary atresia
(proposed by the Japanese Society of Pediatric
Surgeons)
Principal types (Fig. 26.1):
–– Type I: atresia of common bile duct (approx. 5%)
–– Type II: atresia of hepatic duct (approx. 2%)
–– Type III: atresia of bile duct at the porta hepa-
tis (>90%)
Early in the course of biliary atresia, the liver
is enlarged, firm, and green. The gallbladder may
be small and filled with white mucus, or it may be
completely atretic. With progression of the dis-
ease, the liver becomes hard and nodular. There
are characteristic microscopic features which
help to differentiate biliary atresia from other
forms of neonatal hepatitis.
Diagnosis of Biliary Atresia
Clinical Features
Neonates present with persistent jaundice along
with acholic stools, passage of dark-stained
urine, and hepatomegaly.
The baby with biliary atresia generally looks
active, is not acutely ill, has a good body weight,
and progressively develops acholic stools, jaun-
dice, and hepatomegaly. In comparison, patients
with medical cause of cholestasis (neonatal hepa-
titis, prematurity) are generally more sick looking,
low on body weight, and jaundiced since birth.
165
Investigations
Laboratory investigations as shown below.
Laboratory Investigations for Neonatal
Obstructive Cholangiopathy
• Liver function tests
–– Conjugated hyperbilirubinemia >2 mg/dL
or direct component >20% of the total.
–– Elevated transaminase and alkaline phos-
phatase level.
–– Gamma-glutamyl transpeptidase (GGT)
levels above 250 units/L are suggestive of
biliary atresia.
• Complete workup to rule out metabolic causes
includes
–– Complete hemogram
–– Coagulation profile (PT/INR)
–– Serology for hepatitis A, B, and C
–– TORCH titers
–– Alpha1 antitrypsin level
–– Serum lipoprotein-X: levels (>300 mg%)
–– Thyroid function test
–– Urine for reducing sugars
–– Urinary aminoacidogram
• Radiological investigations
–– Ultrasonography should be the first diag-
nostic imaging study done to jaundiced
infants to evaluate the presence of a gall-
bladder and identify intra- or extrahepatic
bile duct dilatation and echogenicity of
liver parenchyma. Atretic bile ducts in the
porta hepatis produce the characteristic
triangular cord sign. Abnormal gall blad-
der size, wall, and mucosa (gall bladder
ghost triad) and increased diameter of the
hepatic artery are other suggestive fea-
tures. The postprandial contraction of the
gallbladder eliminates the possibility of
biliary atresia. In late cases features of
portal hypertension can be seen. The over-
166 26 Infantile Obstructive Cholangiopathy

Fig. 26.3 Cholangiogram through gall bladder showing

distal bile duct patency, proximal biliary atresia (absence of
Fig. 26.2 Liver nuclear scan showing no excretion of dye intrahepatic biliary radicals, clips are post-open surgery)
in the gut suggestive of biliary atresia

with oral phenobarbital (5 mg/kg) for up to

Table 26.2 Histopathology of neonatal hepatis and 5 days before the procedure to enhance the
extrahepatic biliary atresia
sensitivity of the hepatic scintigraphy. The
Idiopathic neonatal Extrahepatic biliary presence of the radioisotope in the GI tract
hepatitis atresia
excludes the diagnosis of BA (Fig. 26.2).
Intracytoplasmic and Intracytoplasmic and
canalicular cholestasis canalicular cholestasis
• Percutaneous liver biopsy for histopathology
Disarranged architecture Preserved architecture has a diagnostic accuracy of more than 90%.
Giant cell formation Rare giant cells Microscopically, few findings are very charac-
Inflammatory infiltrate in Discrete inflammatory teristic of EHBA; therefore, percutaneous liver
the portal space infiltrate
Little fibrosis Portal and periportal
biopsy can be used to aid in diagnosis if an
Extramedullary fibrosis experienced pathologist is available. Findings
hematopoiesis Ductal proliferation characteristic of EHBA include bile duct pro-
Bile thrombus in the liferation (most important), portal fibrosis, and
interlobular ducts
absence of sinusoidal fibrosis. Other associ-
ated findings include bile stasis, periportal
all accuracy of ultrasound in the diagnosis inflammation, and giant cells (Table 26.2).
or elimination of biliary atresia is now • Duodenal aspiration for bile: The presence
above 90%. of the yellow bilirubin pigment in the aspi-
–– Magnetic resonance cholangiopancreatog- rate of duodenal content excludes the diag-
raphy (MRCP) has been recently used in nosis of BA.
diagnosis of biliary atresia and has been • Endoscopic retrograde cholangiopancreatography.
proved to be reliable.
–– Radionuclide scintigraphy. Surgical cholangiography confirms the diag-
Hepatic scintigraphy with hepatobiliary nosis and is the gold standard investigation for
iminodiacetic acid or diisopropyl iminodi- confirmation of biliary atresia (Fig. 26.3). Those
acetic acid. (HIDA, DISIDA): HIDA is infants with radiographic evidence of patent
short form for the radiotracer earlier used biliary tract (extra- and intrahepatic) have no
and has become synonymous with hepato- BA (Fig. 26.4). Laparoscopic operative cholan-
biliary scintigraphy even though Tc99 giogram offers minimally invasive technique for
mebrofenin is the most common radiophar- the diagnosis. Biliary hypoplasia is a rare cause
maceutical used. Patients are “primed” of persistent neonatal conjugated hyperbilirubi-
Results and Prognosis Following Kasai Portoenterostomy
Fig. 26.4 Cholangiogram showing patent biliary tree.
Note patent intrahepatic biliary radicals above and flow of
dye into the intestine
nemia. Diagnosis is done by cholangiogram
showing hypoplastic biliary tree. Management
is conservative as the result of Kasai operation
for biliary hypoplasia is poor.
Management
Surgical Kasai portoenterostomy is still the gold
standard operation for biliary atresia. The patients
are managed as per Fig. 26.5. Kasai procedure
consists of removing the obliterated extrahepatic
biliary system (Fig. 26.6) and anastomosing the
most proximal part (portal plate) to a Roux-en-Y
jejunal loop (Fig. 26.7). The dissection of the
fibrous biliary tree should be as far as the entrance
of the anterior branch of the right hepatic artery
on the right side and as far as the entrance of the
umbilical vein into the left portal vein on the left
side. The fibrous cone is sharply transected up to
the posterior surface of the portal vein keeping a
wide raw area for the anastomosis. The length of
the Roux-en-Y limb is kept at least 40 cm to pre-
vent cholangitis. Liver biopsy is mandatory to
define the degree of fibrosis present. Best results
167
are seen if the surgery is performed within
60 days of life. Hence early identification and
referral of suspected case are important. The out-
come worsens with progression of time and
almost invariably poor beyond 120 days of life.
The postoperative management protocol and
prognostic factors are given below.
Postoperative Management Protocol
• Nasogastric drainage until intestinal function
returns
• Intravenous antibiotics till oral antibiotics are
initiated
• Drain removal before discharge
• Prolonged use of antibiotics and choleretics to
prevent postoperative cholangitis
• Ursodeoxycholic acid (UDCA)—10–15 mg/
kg/day
• Fat-soluble vitamins vitamin ADEK
drops—1 mL/day, oral/injectable vitamin K
for long term
• Prednisolone—2 mg/kg/day divided twice
daily; taper over 2–3-week period
Almost three-fourths of patients will develop
portal hypertension in spite of adequate postop-
erative bile flow. They will manifest esophageal
varices, hypersplenism, and ascites. Hepatic
transplantation is reserved for those patients with
failed portoenterostomy, progressive liver failure,
or late referral to surgery. Other complications
include cholangitis, hepatopulmonary syndrome,
and pulmonary hypertension (due to intrapulmo-
nary shunting).
 esults and Prognosis Following
R
Kasai Portoenterostomy
More than two thirds of patients show initial
good biliary drainage (25–86%).
One third (35%) of total (half of that achiev-
ing good bile drainage) continue with sustained
bile flow and remains free from long-term
sequelae.
One third (35%) of total (half of those achiev-
ing good bile drainage) will show insidious
development of progressive liver failure with
return of jaundice in the long term.
168 26 Infantile Obstructive Cholangiopathy

Fig. 26.5 Management

Suspected Biliary atresia
of suspected
extrahepatic children
with biliary atresia

Plan
Per Operative Cholangiogram
and proceed

Gall Bladder atretic Gall Bladder not atretic

Absence of bile on aspiration on Presence of bile on aspiration on

cholangiogram failure of contrast cholangiogram passage of the
to pass in the duodenum or to contrast in the duodenum or
pass proximally in hepatic ducts proximally in hepatic ducts

Biliary atresia ruled out

Biliary atresia confirmed. and no further surgical
Proceed with Kasai portoenterostomy intervention needed

Fig. 26.6 Hilum of the liver in biliary atresia showing

atretic gall bladder (silk thread) and atretic bile duct in the
porta hepatis (arrow)

Fig. 26.7 Roux-en-Y hepaticoportoenterostomy (Kasai)

Suggested Reading
One third (15–30%) of total will never
drain bile after portoenterostomy and will
progress to liver failure and will need early
transplantation.
The Prognostic Factors for Biliary Atresia
• The serum bilirubin value at 3 months after
surgical correction can be used to predict
long-term survival.
• Type I and II biliary atresias generally have
good prognosis if treated early.
• Type III biliary atresia, the presence of larger
bile ductules at the porta hepatis (>150 mm in
diameter), is associated with a better
prognosis.
• Syndromic biliary atresias have worse out-
comes in terms of both clearance of jaundice
and overall mortality.
• Concomitant CMV infection fare less well
after a portoenterostomy.
 iver Transplantation in Biliary
L Associated pancreaticobiliary duct malformation
Atresia
Biliary atresia is the most common indication for
liver transplantation in children, 70–80% of the
patients eventually requiring transplantation.
The indications for liver transplantation in
postoperative biliary atresia patients are:
1. No bile drainage at all, because major clinical
deterioration will be inevitable.
2. The presence of signs of developmental retar-
dation or their sequelae if they become
uncontrollable.
3. Decreased bile drainage with progressive liver
failure.
169
4. A high hepatic artery resistance index mea-
sured on Doppler ultrasonography is an indi-
cation for relatively urgent transplantation.
Five-year survival after liver transplantation
for biliary atresia is currently 80–90%.
 ther Rare Causes of Surgical
O
Jaundice in Infants
Inspissated bile syndrome is due to impaction of the
bile duct by thick viscid bile or sludge. The causes
include dehydration, hemolysis, cystic fibrosis, total
parenteral nutrition, or defect in bile secretion. It may
at times be difficult to differentiate from biliary atre-
sia. It is generally a self-limiting condition. However,
persistent obstruction may require intraoperative
cholangiography and flushing the biliary tree.
Idiopathic perforation of bile duct is a rare entity.
It occurs due to weakness of the bile duct wall usually
at the cystic duct and common bile duct junction.
may be present. Presentation can be with jaundice,
clay-colored stool, ascites, and malnutrition. Surgical
drainage of the area with cholecystostomy generally
leads to healing of the perforation.
Suggested Reading
Coran AG, Caldamone A, Scott Adzich N, Krummel TM,
Laberge JM, editors. Pediatric surgery. 7th ed. Oxford:
Elsevier; 2012.
Spitz L, Coran C, Teitelbaum DH, Pierro A, Tan HL.
Operative pediatric surgery. 7th ed. Boca Raton, FL:
CRC Press; 2013.
Kliegman R, Stanton B, Geme JS, Nelson SN. Textbook
of pediatrics. 20th ed. Philadelphia, PA: Saunders,
Elsevier; 2015.
 Portal Hypertension in Children
Portal hypertension is defined as:
• Portal venous pressure >11 mmHg
• Splenic pulp pressure >16 mmHg
• Increase in hepatic venous pressure gradient
(wedged hepatic venous pressure minus free
hepatic venous pressure, normal range is
7–8 mm of Hg).
The investigations to determine the above
pressures are not performed clinically so they are
irrelevant for the diagnosis of portal hyperten-
sion. Therefore, patients with splenomegaly and
presence of esophageal varices with supportive
radiological findings can be labeled as having
portal hypertension.
Etiology and Types
• Cirrhotic
• Noncirrhotic
–– Prehepatic: Extrahepatic portal venous
obstruction (EHPVO), common in children
–– Intrahepatic
Presinusoidal: Portal vein sclerosis, con-
genital hepatic fibrosis
Sinusoidal: Fatty liver, nodular regenera-
tive hyperplasia
Postsinusoidal: Veno-occlusive disease
–– Posthepatic Budd-Chiari syndrome
© Springer Nature Singapore Pte Ltd. 2018
S. Roy Choudhury, Pediatric Surgery, https://doi.org/10.1007/978-981-10-6304-6_27
27
While intrahepatic causes are more common
in developed countries, in a developing country
like India, extrahepatic portal venous obstruction
(EHPVO) is the most common cause of prehe-
patic portal hypertension in children. It may
result from perinatal omphalitis, umbilical
venous cannulation, intra-abdominal sepsis, and
dehydration. Patients with EHPVO usually have
normal liver function but with significant effects
of portal hypertension like variceal hemorrhage
and hypersplenism. Biliary atresias followed by
post-total parenteral nutrition-induced cholesta-
sis are the most common causes of cirrhosis lead-
ing to portal hypertension in children. Congenital
hepatic fibrosis, focal biliary cirrhosis, alpha1-­
antitrypsin deficiency, chronic active hepatitis,
and cystic fibrosis are other causes of portal
hypertension in children. These children have
abnormal liver function with enlarged firm liver,
ascites, jaundice, and malnutrition. Budd-Chiari
syndrome is due to obstruction of hepatic veins.
This could be due to webs or in association with
coagulation disorders (protein C, protein S, and
antithrombin 3 deficiency), malignant disease,
oral contraceptives, and autoimmune disorders.
Presentation
The usual presenting features are bleeding esoph-
ageal varices, splenomegaly with hypersplenism
(Fig. 27.1), growth retardation, and ascites.
171
172
Fig. 27.1 Massive splenomegaly due to EHPVO
Variceal hemorrhage occurs from submucosal
plexus of the lower esophagus due to portosys-
temic collaterals around the distal esophagus.
Retroperitoneal, periumbilical (Fig. 27.2), and
hemorrhoidal collaterals can develop.
Children tolerate variceal bleeding much bet-
ter than adults, and the overall outcome in chil-
dren is much better than in adults.
Diagnosis
• Doppler ultrasonography is accurate in mak-
ing the diagnosis. It gives information about
direction, velocity, and waveform of the portal
blood flow. In cases of EHPVO, portal vein
cavernoma can be seen. It can also confirm
patency of inferior mesenteric and splenic
veins.
• MRI and CT angiography are more specific in
making the anatomical diagnosis. They are
also helpful in determining patency of major
vessels before planning shunt procedures.
27 Portal Hypertension in Children
Fig. 27.2 Abdominal wall collateral venous dilatation
due to portal hypertension
• Endoscopy is done to visualize, grade, and
manage esophageal varices. Management of
varices by either sclerotherapy or banding can
be done at the same time.
Grading of Varices
• Grade I: present but small and collapsible
• Grade II: tortuous vein filling less than one
third of esophageal lumen
• Grade III: tortuous vein filling more than one
third of esophageal lumen
• Grade IV: varices with stigmata (cherry red
spots and “varices on varices”)
Management
Management of Variceal Bleeding
• Resuscitation
–– Insertion of two large-bore IV lines and
commencement of intravenous fluid vol-
ume resuscitation.
–– Sending investigations; hemogram, coagu-
lation profile, renal and liver function tests.
–– Transfusion of RBC to maintain hemoglo-
bin of 10 g/dl; avoiding overtransfusion.
Surgical Management
• Medical management
–– Correction of coagulopathy with injection
of vitamin K and fresh frozen plasma
infusion.
–– Starting octreotide: bolus dose of 1 μg/kg
(max 50 μg) followed by infusion at the rate
of 1–3 μg/kg/h (max 50 μg) and continue till
24 h after bleeding ceases and then tapering it.
–– Keeping nil by mouth.
–– If needed then balloon tamponade with
Sengstaken-Blakemore tube may be
applied.
–– Histamine 2 receptor blocker drugs.
• Treatment of varices: Upper GI endoscopy
within 24 h to confirm and treat bleeding vari-
ces by either endoscopic sclerotherapy or
banding.
• For uncontrolled bleeding TIPS (transjugular
intrahepatic portosystemic shunt) or portosys-
temic shunt should be considered.
Primary Prophylactic Treatment
(Treatment Before Bleed)
• Beta-blockers reduce portal venous pressure
and decrease chances of first episode of bleed
and subsequent bleed.
• Role of prophylactic banding is unclear in
children.
TIPS is also indicated in patient with recurrent
variceal bleeding who awaits liver transplanta-
tion. The debate that whether a patient after con-
trol of initial variceal bleeding is best managed
with serial endoscopic variceal management or
portosystemic shunt is still ongoing.
Surgical Management
Indications of Surgical Intervention
• Failed medical management (failure to achieve
control of variceal bleeding after two sessions
of endoscopy)
• Severe symptomatic hypersplenism
• Massive symptomatic splenomegaly
173
• Lack of access to expert endoscopy therapy
• Gastric bleeding or other ectopic variceal
bleeding
• Rare blood group
• Portal biliopathy
• Few selected cases of Budd-Chiari syndrome
where obstruction does not extend into the
inferior vena cava (IVC)
• Intraparenchymal liver disease requires liver
transplantation.
• Budd-Chiari syndrome is due to hepatic
venous web causing venous obstruction.
Occasionally the obstruction extends to IVC.
• Treatment of Budd-Chiari syndrome includes:
• Percutaneous angioplasty
• Shunt surgery
–– In cases without significant IVC obstruc-
tion, portosystemic shunt surgery can be
considered.
–– In the presence of significant IVC obstruc-
tion, meso-atrial shunt becomes an option.
Surgical options for portal hypertension in
children are:
• Shunt operations
• Non-shunt procedures
Shunts
• Portosystemic shunts
–– Nonselective: diverts a large portion of
mesenteric blood away from the liver
Total
• Lienorenal shunt (central): Side-to-­side
lienorenal shunt without splenectomy
(Mitra) or end to side lienorenal shunt
with splenectomy (Fig. 27.3)
• Portacaval shunt (end to side/side to
side)
• Mesocaval shunt with large-diameter
graft
Partial
• Sarfeh shunt in which attempt is made
to drop mesenteric pressure below
12 cm of water so as to decompress vari-
ces but still maintain some hepatopetal
flow. Usually a graft of 8 mm diameter
is used.
174 27 Portal Hypertension in Children

Shunts for portal hypertension

SV
PV
IVC

SV

IVC PV

Shunt LRV

LRV

Distal splenorenal (Warren) shunt Proximal lienorenal shunt

with splenectomy

PV

shunt

SV
PV

IVC IVC LRV

Porto caval shunt Side-to-side lienorenal (Mitra)

shunt without splenectomy

Fig. 27.3 Different shunts for portal hypertension (IVC-inferior vena cava, PV-portal vein, SV-splenic Vein, LRV-left
renal vein)

–– Selective shunt: preserve majority of portal/ • Distal splenorenal shunt (Warren shunt;
mesenteric flow to the liver while shunting Fig. 27.3)
blood from high-pressure gastroesophageal –– Bypass shunts (Rex shunt): an internal
varices to low-pressure systemic circulation jugular venous graft is used to connect the
Suggested Reading 175

a b
Short gastric
vessels
Branch of
Inferior
left
phrenic vessels
gastroepiploic
Paraesophageal vessels
vessels
Left
gastric vessels

Fig. 27.4 (a, b) Esophagogastric devascularization procedure

left portal vein to mesenteric vein. This can

be performed in EHPVO. The prerequisites Complications of Portal
are normal liver, patent left portal vein Hypertension
at Rex fissure (umbilical fissure), and a
suitable vein in mesenteric circulation to
­ • Ectopic varices
function as inflow tract. This is a physiologi- • Growth retardation
cal shunt as it maintains portal venous blood • Hypersplenism
flow into the liver; however, this shunt is • Portal biliopathy
technically demanding. • Ascites
• Hepatorenal syndrome
Complications of shunt surgery include shunt • Hepatopulmonary syndrome
block and risk of encephalopathy due to shunting • Encephalopathy (due to spontaneous porto-
away of blood from the liver.Non-shunt operations systemic shunt)
such as the Sugiura procedure (splenectomy with
esophagogastric devascularization with esopha- Management algorithm of portal hypertension
geal transaction and anastomosis) are successful in children.
for managing variceal bleeding. They are consid-
ered particularly when a shuntable splenic or mes-
enteric vein is not available and in cases of massive Suggested Reading
splenomegaly with severe hypersplenism where
splenectomy becomes necessary. Splenectomy Coran AG, Caldamone A, Scott Adzich N, Krummel TM,
Laberge JM, editors. Pediatric surgery. 7th ed. Oxford:
with gastroesophageal decongestion, as originally
Elsevier; 2012.
described by Hassab for managing bilharzial cir- Zinner MJ, Ashley SW. Maingot’s abdominal operation.
rhosis with portal hypertension, has the merits 12th ed. New York: McGraw Hill; 2013.
over the Sugiura procedure that thoracotomy and Roy Choudhury S, Chadha R, Sharma AK, Anand
VK, Patwari AK. Surgical management of massive
esophageal transaction are avoided, thereby elimi-
splenomegaly and severe hypersplenism secondary
­
nating the possibility of esophageal leakage to extrahepatic portal venous obstruction in children.
(Fig. 27.4a, b). Surg Today. 2007;37:19–23.
 Choledochal Cyst
Choledochal cyst is a rare congenital dilatation of
the various ducts of the biliary system. The inci-
dence varies from 1 in 100,000 to 150,000 in the
Western population and is reported to be as high
as 1 in 1000 in Japan.
Etiology
The etiology is related to an abnormal pancreatic-­
biliary junction (common channel theory) caus-
ing reflux of pancreatic enzymes into the common
bile duct (trypsin and amylase). This pancreatico-
biliary ductal malunion (PBM) usually exceeds
10 mm, compared with the normal common
channel length of 5 mm in children. These ducts
join well outside the duodenal wall and therefore
are not surrounded by normal sphincter mecha-
nism. Other theories which do not find favor
include congenital weakness of the common bile
duct and distal obstruction of the bile duct.
Classification of Choledochal Cyst
Choledochal cysts are classified (Todani classifi-
cation) depending on the morphology and local-
ization (Fig. 28.1)
Type I saccular or diffuse fusiform dilatation of
the extrahepatic bile duct
Type II diverticulum of the extrahepatic bile
duct
© Springer Nature Singapore Pte Ltd. 2018
S. Roy Choudhury, Pediatric Surgery, https://doi.org/10.1007/978-981-10-6304-6_28
28
Type III choledochocele (dilatation of the terminal
common bile duct within the duodenal wall)
Type IV multiple cysts of the extra- and intrahe-
patic bile ducts (IVa) or multiple extrahepatic
duct cysts (IVb)
Type V single or multiple intrahepatic cysts (Carolis
disease when associated with hepatic fibrosis)
Pathology
The cyst is lined by discontinuous columnar epi-
thelium. Squamous metaplasia can be present.
The cyst wall is thick, fibrous and often inflamed.
Long standing cyst can result in cirrhotic changes
in the liver. Features of portal hypertension can
develop.
Presentation
• Prenatal detection is possible by antenatal ultra-
sonography in some cases.
• Infants usually present with features of
obstructive jaundice, failure to thrive, and
fever. Infants develop jaundice more fre-
quently, causing diagnostic dilemma with bili-
ary atresia.
• In children abdominal pain is the most com-
mon symptom. Obstructive jaundice if present
is intermittent as compared to persistent jaun-
dice in infants. The classic triad of jaundice,
pain, and right hypochondrial mass is a rare
177
178 28 Choledochal Cyst

Fig. 28.1 Anatomical

types of choledochal
cyst

Type Ia Type Ib Type Ic

Type II Type III Type IV

Type V

clinical presentation. Few patients may pres-

ent with complications like cholangitis, pan-
creatitis, or biliary peritonitis. Older children
usually present with abdominal pain and mass.
Jaundice is less severe and intermittent.

Diagnosis

Choledochal cyst is confirmed by imaging with

Fig. 28.2 CT scan showing a choledochal cyst
ultrasound and/or CT scan (Fig. 28.2). These
investigations provide information about the
size and position of cyst, status of proximal
ducts, vascular abnormality, and hepatic
echotexture. Hepatic scintigraphy with HIDA
Management
Fig. 28.3 MRCP: Choledochal cyst with stones and
dilated hepatic ducts; note the pancreatic duct
(or DISIDA) scan can be contributory by show-
ing a hepatic filling defect which gradually fills
with radiotracer on delayed images, although
they are rarely required for the diagnosis.
Recently MR cholangiopancreatography
(MRCP) is the investigation of choice as it pro-
vides excellent anatomical delineation of the
biliary tree (Figs. 28.3 and 28.4).
Management
Surgical management consists of cyst excision
and Roux-en-Y hepaticojejunostomy or hepati-
coduodenostomy reconstruction (Fig. 28.5).
Intraoperative endoscopy or cholangiography
helps in identifying any anatomical or pathologi-
cal abnormalities like stone, sludge, stricture, etc.
which can be appropriately dealt during the sur-
gery. Recently laparoscopic excision is being
performed wherever expertise is available.
Forme fruste choledochal cyst (FFCC) is charac-
terized by absent/minimal dilatation of the extrahe-
patic bile duct and pancreaticobiliary malunion with
179
Fig. 28.4 MRCP: Multiple CBD stones, pancreatic duct
is delineated
features of fever, jaundice, and recurrent abdominal
pain. The normal diameter of the common bile duct
in children ranges between 2 and 6 mm. FFCC is
associated with a diameter above 6 mm and below
10 mm. Most patients with FFCC have a long com-
mon channel with partial obstruction of the terminal
common bile duct. Management consists of cyst
excision and Roux-en-y hepaticojejunostomy.
Choledochocele is an extremely rare variant
of choledochal cysts classified as type III by
Alonso-Lej. The cystic dilatation occurs in the
distal portion of the common bile duct. Patients
with choledochocele can develop intermittent
colicky abdominal pain, obstructive jaundice,
and recurrent bouts of pancreatitis. Diagnosis is
suggested by ultrasonography and confirmed
with HIDA scan, MRCP, or ERCP. Endoscopic
management (papillotomy) of choledochocele
can be done for a small lesion. Surgical manage-
ment entails excision of the duodenal luminal
portion of the cyst leaving the medial portion
containing the ampulla intact. Large choledocho-
cele may require excision of cyst with hepatico-
enterostomy and pancreatic sphincteroplasty.
180
Fig. 28.5 Choledochal cyst with gall bladder (intra
operative)
Type II choledochal cyst requires excision of
the diverticulum and reconstruction of the com-
mon bile duct.
Type IV choledochal cyst: Biliary drainage is
improved by excision of extrahepatic duct and
28 Choledochal Cyst
Roux-en-Y hepaticojejunostomy. Debris and cal-
culi should be removed from dilated intrahepatic
ducts. Hepatic duct stricture should be incorpo-
rated in biliary-enteric anastomosis.
Type V choledochal cyst: If the disease is con-
fined to a particular lobe of the liver, then hepatic
lobectomy can be curative. If both the lobes are
involved and child remains symptomatic even
after hepaticojejunostomy, then liver transplanta-
tion may be needed.
Residual cyst can cause problems like stric-
ture, cholangitis, stone formation, pancreatitis,
biliary cirrhosis, and malignancy. Long-term fol-
low-­up after surgery is necessary.
Suggested Reading
Coran AG, Caldamone A, Scott Adzich N, Krummel TM,
Laberge JM, editors. Pediatric surgery. 7th ed. Oxford:
Elsevier; 2012.
Spitz L, Coran C, Teitelbaum DH, Pierro A, Tan
HL. Operative pediatric surgery. 7th ed. Boca Raton:
CRC Press; 2013.
 Lesions of the Stomach
29

Peptic Ulcer Secondary Disease

• Most commonly secondary to systemic dis-
Peptic ulcer is defined as an ulcer in the gastro- eases like major trauma, major burns (Curling
duodenal region due to imbalance in acid-­ ulcer), sepsis, shock, or head injury (Cushing
producing and acid-protective mechanisms. ulcer).
The incidence in children is 5 per 100,000. • Other common causes can be chemical (drugs
like corticosteroids or NSAIDs).
• These occur secondary to decreased mucosal
Pathology blood flow, disruption of mucosal barrier, and
stress-related luminal hyperacidity
The peptic ulcer disease in children can be pri- • Usually there are multiple superficial erosions
mary or secondary. Primary disease occurs in the in the stomach. However, Cushing and Curling
absence of any major systemic illness. ulcer may present as single deep ulcer which
is likely to bleed or perforate.
Primary Disease
• Genetic predisposition is common.
• Causes can be due to gastric hypersecre- Clinical Presentation
tion. Helicobacter pylori is now considered
the most likely cause for peptic ulcer Primary and secondary peptic ulcers have differ-
disease. ent presentations though there may be an overlap.
• H. pylori produces urease which hydrolyzes Primary peptic ulcer disease may present as
urea to ammonia and bicarbonate. Ammonia chronic recurrent abdominal pain usually in the
is cytotoxic, whereas bicarbonate increases epigastric region. Vomiting or refusal to feeds is
the pH and disrupts the mucus barrier. H. common in infants. Nocturnal awakening due to
pylori also produces a cytotoxin which can pain is also common and differentiates from
directly damage the gastric epithelium. recurrent psychogenic abdominal pain. Massive
• Usually have an ulcer in the gastric antrum or gastrointestinal bleeding and gastroduodenal per-
the duodenum. foration are uncommon presentations.

© Springer Nature Singapore Pte Ltd. 2018 181

S. Roy Choudhury, Pediatric Surgery, https://doi.org/10.1007/978-981-10-6304-6_29
182 29 Lesions of the Stomach

Secondary peptic ulcer disease frequently

presents with massive gastrointestinal bleeding,
perforation, or shock in a child with predisposing
condition.

Diagnosis

Upper gastrointestinal endoscopy with gastric

biopsy for detecting H. pylori is the standard tool
for diagnosis.
H. pylori can also be detected by a breath test
after giving radiolabeled urea. Stool antigen
detection is also a helpful investigation to detect
H. pylori.
Barium meal studies may be useful but are
less sensitive than endoscopy.

Treatment

The treatment of primary peptic ulcer disease Fig. 29.1 Ingested coin in the stomach
involves eradication of H. pylori infection. This
can be achieved by giving two antibiotics (amox-
icillin, clarithromycin or metronidazole) for 2
weeks and a proton pump inhibitor for 4 weeks.
Surgical treatment is needed in complicated
cases with massive uncontrollable gastrointesti-
nal hemorrhage or in cases with gastroduodenal
perforation.

Foreign Bodies and Bezoars

Foreign bodies in the stomach are frequently

encountered in children. Once they pass the
esophagus (cricopharyngeus), smooth foreign
bodies (e.g., coin, Fig. 29.1) generally pass
through the rest of the intestinal tract. Watchful
expectancy with reassurance is required. The
exceptions are sharp objects and button batteries
ingested along with a magnet. They should be
removed with endoscopic assistance.
Bezoars are solid mass of indigestible mate-
rial that accumulated in the gastrointestinal tract.
Phytobezoars are made up of vegetable matter,
whereas trichobezoars (Fig. 29.2) are made up of
swallowed hair (Rapunzel syndrome).
Fig. 29.2 Trichobezoar
Pharmacobezoars occur due to accumulation of
drugs with limited solubility.
The risk factors for bezoar are delayed gastric
emptying and decreased acid secretion in the
stomach.
Small bezoars may pass on their own or can be
removed endoscopically; however, large bezoars
require open or laparoscopic approach for
removal.
Gastric Volvulus 183

Fig. 29.3 (a, b) Upper

gastrointestinal contrast a b
study showing gastric
volvulus

Gastric Volvulus

It occurs when the attachments of the ligaments

of stomach are lax or absent. It can be associated
with eventration of the diaphragm, hiatal hernia,
or diaphragmatic hernia. The age group involves
neonates and young infants. Depending upon the
axis of rotation, two varieties are known:

• Organo axial: the stomach rotates on the lon-

gitudinal axis, and the greater curvature usu-
ally comes anterior to the lesser curvature
(Fig. 29.3a, b). They are often associated with
diaphragmatic defects and have higher
chances of necrosis.
• Mesenterio axial type: the stomach rotates
around the line joining the lesser and the
greater curvature. The antrum usually passes
anterior. They usually present with chronic
symptoms and necrosis is uncommon.

Classical presentation consists of a triad of

symptoms of upper abdominal pain or distension,
nonproductive attempts of vomiting/retching,
and inability to pass a nasogastric tube. This triad
is called Borchardt triad.
Radiology with upper gastrointestinal contrast
study helps in the diagnosis (Figs. 29.3 and 29.4).
Fig. 29.4 Gastric volvulus with herniation in the right chest
Surgical correction by gastrostomy or gastropexy
either open or laparoscopic is generally
successful.
184
Fig. 29.5 (a) Massive
a b
pneumoperitoneum due
to gastric perforation.
(b) Total gastric necrosis
Pyloric Atresia
It presents with features of gastric outlet
obstruction. Plain X-ray shows large “single
bubble” of the distended stomach. Associations
with epidermolysis bullosa and other gastroin-
testinal anomalies have been reported, and the
outcome is poor. Surgical correction requires
gastroduodenostomy.
Gastric Perforations and Necrosis
Gastric perforations most commonly occur in
premature neonates. They are due to idiopathic or
iatrogenic or peptic ulcer disease. Most perfora-
tions are idiopathic and are called spontaneous
gastric perforations. Isolation of fungus from the
perforation sites has been reported suggesting a
possible cause. The most common setting is in a
child with perinatal stress. It is proposed that
decreased enteric blood supply causes ischemia
and makes the stomach more prone to
­perforations. Iatrogenic causes can be overzeal-
ous bag and mask ventilation, esophageal intuba-
tion and ventilation, and injury during insertion
of nasogastric tube.
29 Lesions of the Stomach
Clinical Presentation
The most common presentation is of sudden
severe abdominal distension due to massive
pneumoperitoneum (Fig. 29.5a, b) resulting in
respiratory distress. Other findings of sepsis or
peritonitis may also be present.
Management requires resuscitation followed
by surgical repair. If the abdominal distension
causes significant respiratory compromise, the
free air can be aspirated. Alternatively, a soft
plastic cannula or glove drain can be inserted in
the abdominal cavity for temporary stabilization
before definitive surgery.
 astric Duplication and Gastric
G
Teratoma
See chap. 34 and 45
Suggested Reading
Coran AG, Caldamone A, Scott Adzich N, Krummel
TM, Laberge JM. Pediatric surgery. 7th ed. Oxford:
Elsevier; 2012.
 Gastroesophageal Reflux,
Achalasia Cardia, Congenital
Esophageal Stenosis
Gastroesophageal reflux (GER) is defined as
involuntary regurgitation of gastric contents into
the esophagus. It is very common in infants.
Almost all newborn babies vomit due to physio-
logical GER. However in 90% infant symptoms
resolve by 9–10 months of age due to maturity of
the lower esophageal sphincter (LES). Persistence
of symptoms beyond 18 months (<2%) is associ-
ated with increased risk of complications.
It is important to differentiate between “physi-
ological” from “pathological” GER and gastro-
esophageal reflux disease (GERD) which is
frequently associated with reflux-related compli-
cations. Pathological GER is said to be present
whenever reflux results in major symptoms or
complications.
Factors Preventing GER
Anatomic Factors
(a) Acute angle of His
(b) Lower esophageal sphincter tone
(>10–30 mmHg)
(c) The length of intra-abdominal esophagus
(d) Mucosal rosette in the lower esophagus
(e) The phrenoesophageal membrane
(f) The pinch-cock effect exerted by the dia-
phragmatic crura
© Springer Nature Singapore Pte Ltd. 2018
S. Roy Choudhury, Pediatric Surgery, https://doi.org/10.1007/978-981-10-6304-6_30
30
Physiological Factors
(a) Effective peristalsis of the distal esophagus
to clear the refluxed gastric contents (second-
ary peristalsis)
(b) Prompt and effective gastric emptying
Any dysfunction of the above mechanisms
may result in GER. The presence of transient
lower esophageal sphincter relaxation (tLESR)
accounts for 94% of reflux episodes in children
and adults. The most common factor which pre-
disposes to GER in patients who do not have
associated disease like esophageal atresia or con-
genital diaphragmatic hernia is tLESR which is
currently believed to be the main mechanism of
gastroesophageal reflux.
Neurologically impaired children (e.g. cere-
bral palsy, Down syndrome) and those following
repair of esophageal atresia have an increased
incidence of GER.
Factors Predisposing to GER
• Wide or obtuse angle of His
• Associated hiatus hernia (Fig. 30.1)
• Increased resistance at the gastric outlet with
raised intragastric pressure and delayed gas-
tric emptying
185
186 30 Gastroesophageal Reflux, Achalasia Cardia, Congenital Esophageal Stenosis

Differential Diagnosis

Vomiting due to mechanical, infective, allergic

(eosinophilic esophagitis), or neurological causes
should be excluded.

Investigations

• Upper gastrointestinal (UGI) imaging series

(Fig. 30.1)
• Gastric scintigraphic scan (Fig. 30.2)
• Esophagogastroscopy and esophageal
biopsy
• Continuous esophageal pH monitoring
• Esophageal manometry
• Intraluminal esophageal electrical impedance

Upper gastrointestinal contrast study provides

information about any anatomical abnormality
Fig. 30.1 Sliding hiatal hernia with gastroesophageal like hiatus hernia, esophageal stricture, gastric
reflux outlet obstruction, and malrotation. In addition
information about esophageal motility, peristal-
sis, and complication of GERD like stricture and
Symptoms of GER ulceration can also be obtained.
Gastric scintigraphic scan is done with a
• Vomiting, poor weight gain, increased cry, meal-/milk-containing radiotracer which is
sleep disturbance, decreased appetite, abdom- given preferably through a nasogastric tube and
inal or chest pain the child is observed under camera (Fig. 30.2).
• Wheezing, stridor, recurrent respiratory infec- The advantage of scan over traditional radio-
tion, sore throat, hoarseness, chronic cough, graphic study is that the child can be observed
water brash under camera for longer periods, but the ana-
• Acute life-threatening event (ALTE), apnea, tomical details are inadequate. Another advan-
bradycardia tage of this study is that late scans can show
• Sandifer syndrome – posturing with opisthot- traced activity in lung fields suggestive of
onus or torticollis aspiration.
Upper gastrointestinal endoscopy provides
macroscopic and histological diagnosis of
Complications of GER esophagitis. Based on endoscopy the severity of
reflux can be graded into Grade I–V. Most
• Failure to thrive importantly Barrett’s esophagus can be
• Erosive esophagitis identified.
• Esophageal stricture formation Twenty-four-hour esophageal pH monitor-
• Chronic respiratory disease ing is the gold standard for the diagnosis of
• Barrett’s esophagus GER. However nonacid reflux cannot be
Surgical Treatment
Fig. 30.2 Technetium
scan showing GER
d­ iagnosed. Simultaneous monitoring of esoph-
ageal and gastric pH (dual probe) improves
the accuracy of assessing GER. Scoring sys-
tem for esophageal pH monitoring includes
percent of time pH < 4 for total period (called
the reflux index), number of reflux episodes
lasting 5 min or longer, duration of the longest
reflux episode, and total number of reflux epi-
sodes. Based on reflux index, GER can be
classified as mild, moderate, and severe
grades. Threshold values for reflux index are
4% in children and 3% in infants fed primarily
on milk.
Esophageal manometry is not routinely per-
formed as it requires a cooperative and quiet
child. It is still helpful in doubtful cases as it
may show the presence of tLESR, gives infor-
mation about LES (length and tone), and also
predicts children who may have postoperative
dysphagia because of abnormal or inadequate
peristalsis.
Intraluminal impedance studies are increas-
ingly being used. This investigation is able to
detect nonacid reflux in addition to acid reflux
and with development of combined impedance
and pH monitoring study will replace simple pH
monitoring in the near future.
187
Treatment
Medical
The effective medical management consists of
upright posturing after feeding, elevating the
head end of the bed, prone position for infants
over 6 months, and small frequent meals with
thickening by adding cereal. The positioning of
child in prone or left lateral position has shown to
decrease the episodes of tLESR.
Pharmacologic agents include reduction of
gastric acid secretion (histamine 2 receptor
blockers, proton pump inhibitors), lansoprazole,
and prokinetic drugs (domperidone, metoclo-
pramide). A novel proposed therapy is adminis-
tration of GABAB agonists like baclofen for
decreasing tLESR.
Surgical Treatment
Surgical treatment of GER should be considered
for the following patients:
• Failure of adequate medical treatment to alle-
viate symptoms of GER.
188 30 Gastroesophageal Reflux, Achalasia Cardia, Congenital Esophageal Stenosis
• Those with ALTE related to gastroesophageal
reflux; however, a period of medical therapy
under close monitoring conditions should be
attempted in many cases prior to recommend-
ing a surgical approach.
• Patients with complications of gastroesopha-
geal reflux, such as aspiration, stricture of the
esophagus, or Barrett’s esophagus.
• Patients with neurologic impairment that requires
feeding gastrostomy who are found to have patho-
logic reflux and who remain on medication.
• Patients with chronic reflux and recurrence of
anastomotic stricture after repair of esopha-
geal atresia.
• Patients with pathological anatomy like a hia-
tus hernia.
Neurologically impaired patients or those
with inability to tolerate feeds also need an
accompanying gastrostomy.
Fundoplication
Fundoplication operation helps to prevent acid reflux
by creating a valve and a high-pressure zone at the
lower esophagus. Increasing the length of the
Restore
intraabdominal
segment of
esophagus
Approximate
diaphagmatic
crurae
Fig. 30.3 Principles of
fundoplication operation
abdominal esophagus and bringing it to higher intra-
abdominal pressure zone further prevent reflux of
gastric contents into the esophagus. Additionally
narrowing the hiatus by crural approximation, resto-
ration of the angle of His, and reduction of hiatus
hernia reduces GER (Fig. 30.3). Increasingly fundo-
plications are being performed laparoscopically.
The commonly performed fundoplication pro-
cedures are shown in Fig. 30.4.
Nissen fundoplication involves a 360° com-
plete wrap of the gastric fundus around the lower
esophagus along with narrowing the hiatus by
crural suturing. In the Thal fundoplication, a
270° partial wrap of the gastric fundus around the
lower anterior esophagus is created. The Toupet
procedure uses 180° posterior wrap. Depending
on the severity of the condition, the wrap can be
tight (neurologically impaired children) or floppy
(GER post esophageal atresia repair). A complete
tight wrap is associated with higher incidence of
gas bloat and dysphagia, whereas a partial or
floppy wrap may be associated with vomiting.
Laparoscopic and robotic surgery has now almost
replaced open surgery with the advantage of early
recovery and discharge from the hospital.
Other complications of fundoplication are
dumping syndrome and bowel obstruction; the
Reduce hiatal
hernia when
present
Wrap fundus
around LES to
reinforce antireflux
barrier
Achalasia Cardia
Fig. 30.4 The
commonly performed
techniques of Normal
stomach
fundoplication
latter has a higher incidence with open than with
laparoscopic surgery.
The chance of failure of surgery is higher in
neurologically impaired children than neurologi-
cally normal children. Therefore total esophago-
gastric disassociation with esophagojejunostomy
can be an option in neurologically impaired chil-
dren with severe GER.
Several endoscopic procedures like Stretta
(radio-frequency energy to the esophagogastric
junction), endoscopic fundoplication to create
mucosal valves, and endoscopic injection at
esophagogastric junction have been proposed,
but the experience in children is limited.
Achalasia Cardia
Achalasia cardia is a pathological condition
characterized by failure of relaxation of the
distal esophagus and the esophagogastric junc-
tion during deglutition. There is progressive
dilatation of the esophagus leading to hypope-
ristalsis and further dilatation. The cause of
189
Dor
Nissen Toupet
this condition is unknown, but the entity is
reported to occur in families. The problem lies
with the intrinsic innervations of the esophagus
wherein there is decreased production of nitric
oxide (NO) which prevents relaxation of the
LES. A similar condition is seen in Chagas dis-
ease in which due to degeneration of the intrin-
sic innervations of the esophagus, there is
decreased motility and progressive esophageal
dilatation. LES in this condition may be hypo-
tonic rather than hypertonic.
This disease is very rare in children. The pre-
sentation is with difficulty in swallowing along
with regurgitation, vomiting, recurrent chest
infection, and near-miss sudden infant death syn-
drome. The differential diagnosis includes con-
genital esophageal stenosis, gastroesophageal
reflux, milk allergy, duplication cyst of the esoph-
agus and the stomach, and diffuse esophageal
spasm. The diagnosis can be made on barium
esophagogram showing dilated esophagus with
smooth narrowing of the distal esophagus and
esophagogastric junction also called “bird beak”
sign (Fig. 30.5). Manometry will show failure of
190 30 Gastroesophageal Reflux, Achalasia Cardia, Congenital Esophageal Stenosis
Fig. 30.5 Contrast
swallow esophagogram
showing smooth
tapering lower end,
achalasia cardia
relaxation of the esophagogastric junction with
swallowing with reduced esophageal peristalsis.
It is at times difficult to differentiate congenital
esophageal stenosis from achalasia cardia. A
nasogastric tube can be negotiated through the lat-
ter but not in the former condition.
Treatment includes pneumatic dilatation,
pharmacotherapy with isosorbidedinitrate and
nifedipine, or local injection of botulinum toxin.
Definitive surgical therapy is Heller’s cardiomy-
otomy preferably with additional fundoplication
(Thal-Dor) which is also feasible by a laparo-
scopic approach.
 ongenital Esophageal Stenosis
C Brunicardi FC, Andersen DK, Billiar TR, Dunn LD,
(CES)
This condition presents with dysphagia during the
weaning period. Close differential diagnosis is
with achalasia cardia; radiography and manome-
try can help in differentiating between the two
conditions. There are three types: esophageal
membrane (EM) or web, fibromuscular stenosis
(FMS), and tracheobronchial remnants (TBR).
TBR is the most common form involving the
lower third of the esophagus and requires surgical
excision of the narrow segment. Histopathological
demonstration of t­racheobronchial cartilage con-
firms the diagnosis of TBR. The EM and FMS
forms can be managed by endoscopic dilatation or
excision.
Suggested Reading
Hunter JG, Jeffrey B. Schwartz’s principles of surgery.
10th ed. New York: McGraw Hill; 2014.
Coran AG, Caldamone A, Scott Adzich N, Krummel TM,
Laberge JM, editors. Pediatric surgery. 7th ed. Oxford:
Elsevier; 2012.
 Pyloric Stenosis
Infantile hypertrophic pyloric stenosis (IHPS) is
a condition due to hypertrophy and hyperplasia
of the gastric pyloric muscles leading to gastric
outlet obstruction in infants.
The incidence is 1–3 per1000 live births in the
Western population. This disease is less common
among Asian population.
IHPS commonly affect the first-born male child.
In familial occurrence, the mother is found to be
more commonly affected than the father, and boys
are affected four times more commonly than girls.
Associated anomalies are rare. The etiology is
unknown. Some probable theories include increased
acidity which results in pylorospasm that further
increases stasis. Gastrin production increases sec-
ondary to stasis and alkaline milk feeds. This further
increases the acidity and pylorospasm. Other prob-
able causes are excess substance P, decreased neu-
rotrophins, deficient nitric oxide synthase, abnormal
innervation, spasm of the pylorus, and failure of
relaxation of the pylorus similar to achalasia cardia.
Genetic causes are supported by disease predisposi-
tion in males and certain ABO blood types.
Pathology
The hypertrophied muscles of the circular layer
compress on the mucosa resulting in a narrow
pyloric channel. The pylorus becomes thickened
with narrow elongated pyloric canal and the stom-
ach becomes dilated due to pyloric obstruction.
© Springer Nature Singapore Pte Ltd. 2018
S. Roy Choudhury, Pediatric Surgery, https://doi.org/10.1007/978-981-10-6304-6_31
31
Clinical Features
The classical presentation of IHPS is non-bil-
ious projectile vomiting, in a full-term baby
between 3 and 6 weeks of age. In preterm
babies, the disease usually presents 2 weeks
later. The vomiting becomes forceful after feed-
ing and coffee ground in color due to gastritis.
Additionally, there are failure to thrive, weight
loss, hunger, constipation, and features of
dehydration.
Significant examination findings are palpation
of a mobile ovoid mass “olive,” in the right upper
quadrant especially induced by a test feed, and
visible gastric peristalsis (Fig. 31.1).
Differential Diagnosis
The differential diagnosis includes overfeeding,
gastroesophageal reflux/delayed gastric empty-
ing, acute gastroenteritis, raised intracranial pres-
sure, antral web, pyloric/gastric duplication, and
extrinsic compression.
Investigations
Biochemical investigations to look for any altera-
tions are essential (see below). Ultrasonography
is useful in the diagnosis. The positive findings in
USG include presence of doughnut or target or
191
192
Fig. 31.1 Visible gastric peristalsis due to pyloric
obstruction
Fig. 31.2 Open pyloromyotomy
bull’s-eye sign, pyloric muscle thickness >4 mm,
and pyloric channel length >17 mm. Pyloric
diameter of more than 14 mm is also considered
diagnostic.
An upper gastrointestinal contrast study is
infrequently required. To aid in the diagnosis of
pyloric stenosis, it is necessary for the contrast to
pass beyond the pylorus. The features suggestive
of pyloric stenosis are large stomach with delayed
emptying, string sign (a thin string of contrast
passes through the pylorus), and shoulder sign
(the hypertrophied muscle bulges into the stom-
ach lumen).
31 Pyloric Stenosis
Biochemical Alterations
Recurrent vomiting with loss of gastric juice
results in hypokalemic, hypochloremic, and meta-
bolic alkalosis. In a compensatory effort, aldoste-
rone causes renal tubular absorption of Na+ and
water in exchange of K+ and H+ which are further
depleted. Bicarbonate is absorbed to compensate
for low chloride level. Thus, metabolic alkalosis
with paradoxical aciduria results.
Management
Resuscitation is done with normal saline boluses
of 10–20 ml/kg body weight as required followed
by 5 or 10% dextrose in 0.45% saline (N/2) at 1.5
times maintenance rate with added potassium
(20–30 mEq/L) after ensuring adequate urine out-
put. It often takes 24–48 h to correct the metabolic
and electrolyte imbalance before the patient can
be taken for surgery. Adequate resuscitation is
utmost important as pyloric stenosis is not a surgi-
cal emergency. Serum bicarbonate of less than
28 mEq/dL, serum chloride of more than
100 mEq/dL and normal serum sodium and potas-
sium levels with adequate urine output denote
adequate resuscitation to allow safe anesthesia.
Surgery
The standard treatment is pyloromyotomy after
Ramstedt described in 1911 (Fig. 31.2).
Laparoscopic pyloromyotomy is recently
becoming popular because of less postoperative
pain, early recovery, and better cosmetic result
(Fig. 31.3a, b).
Pyloromyotomy is done under general anesthe-
sia via a small upper transverse or umbilical
(Bianchi) incision. The thickened pylorus is iden-
tified by its attachment to the omentum and trans-
verse colon and delivered into the wound. The
pyloric serosa is gently incised over an avascular
area and then the muscle is split with a spreader
from the pyloroduodenal junction (prepyloric
vein) to the antrum of the stomach, thereby allow-
ing the intact mucosa to bulge out into the wound.
Suggested Reading 193

a b

Fig. 31.3 (a) Laparoscopic pyloromyotomy with a knife. (b) Pyloric incision with a hook cautery

The completeness of pyloromyotomy is judged by incomplete pyloromyotomy, wound infection, and

free movement of the split edges of the pylorus, persistent vomiting due to gastroesophageal reflux.
and mucosal integrity is checked with injection of
air or saline into the stomach through a nasogastric
tube. The results are very satisfactory. Postoperative Suggested Reading
feeding can be started after 6–8 h of surgery and
gradually build as tolerated. Complications of Coran AG, Caldamone A, Scott Adzich N, Krummel TM,
Laberge JM (eds). Pediatric surgery. 7th ed. Oxford:
pyloromyotomy include mucosal perforation Elsevier; 2012.
which can be repaired and patched with omentum,
 Neonatal Intestinal Obstruction
32

Neonatal intestinal obstruction has an incidence of Varieties

1 in 2000 live births. Congenital atresia and steno-
sis (duodenum, jejunum, ileum, colon, and multi-
ple sites) constitute the majority of cases. Other
causes include malrotation, volvulus, meconium
ileus, Hirschsprung disease, and anorectal malfor-
mations. Prenatal sonography showing echogenic
bowel, dilated bowel, and polyhydramnios is sug-
gestive of intestinal anomalies.
Duodena atresia
• Type I membrane, wind sock diaphragm
(Fig. 32.1)
• Type II atresia with intervening fibrous cord
• Type III atresia with gap
Duodenal stenosis/partial obstruction

• Web with a hole

Intestinal Atresia and Stenosis • Annular pancreas (Fig. 32.2)

Pyloric Atresia

It is rare and familial and has an association with

epidermolysis bullosa. Single large gastric bub-
ble in X-ray is diagnostic. A Heineke-Mikulicz
pyloroplasty for a pyloric web or Billroth 1
­gastroduodenostomy for solid pyloric atresia is
recommended. Gastrojejunostomy should be
­
avoided.

Duodenal Atresia and Stenosis

They are caused by failure of recanalization dur-

ing the embryonic development. Atresia is more
common than stenosis (20% cases). Fig. 32.1 Duodenal web

© Springer Nature Singapore Pte Ltd. 2018 195

S. Roy Choudhury, Pediatric Surgery, https://doi.org/10.1007/978-981-10-6304-6_32
196
• Preduodenal portal vein
• Ladd’s band in malrotation
Associations
Prematurity, growth retardation, other malforma-
tions (50%) including malrotation, heart disease,
and Down syndrome (35%).
Antenatal Diagnosis
Antenatal detection of maternal polyhydramnios
(50%), growth retardation, and double bubble
appearance on ultrasonography (Fig. 32.3) is
Fig. 32.2 Annular pancreas
Fig. 32.3 Antenatal scan showing duodenal atresia
32 Neonatal Intestinal Obstruction
suggestive of duodenal obstruction and prompt
for fetal karyotype study. Management is best
done after delivery.
Diagnosis
Clinical presentation includes bilious vomiting,
high nasogastric bilious aspirate, and electrolyte
imbalance. Bile-stained vomiting in neonates is
almost always due to organic cause.
Pain X-ray shows double bubble (gastric and
duodenal) with distal paucity of gas (Fig. 32.4).
Atresia generally occurs at the level of ampulla of
Vater. In 85% of cases, the ampulla is present in
proximal bowel giving rise to bilious aspirates,
Fig. 32.4 Duodenal atresia (double bubble)
Intestinal Atresia and Stenosis 197

and the rest 15% of cases, it is present in the

distal bowel and the aspirates are non-bilious.

Treatment

Initial management comprises of nasogastric

decompression, correction of fluid and electro-
lyte imbalance, and looking for any associated
anomaly (Down syndrome, congenital heart
disease).

Surgery
Fig. 32.5 Dilated loops of bowel with visible peristalsis
Duodenoduodenostomy is the operation of choice.
A wide diamond-shaped anastomosis between the
two ends of the duodenum (Kimura) has a high
success rate. Any membrane should be divided
taking caution for not damaging the ampulla of
Vater on its medial side. Transanastomotic feed-
ing tubes are useful for starting early enteral
feeding.

Jejunoileal Atresia and Stenosis

The probable etiology is localized intrauterine

vascular accident.

Clinical Presentation

Antenatal Diagnosis
The presence of polyhydramnios, echogenic
bowel, and dilated and thickened bowel is sug-
gestive of bowel pathology. One third of jejunoil-
eal atresias are correctly diagnosed by antenatal
USG.
Postnatal Diagnosis
Clinical presenting features are bilious vomiting,
abdominal distension, jaundice, and failure to
pass meconium. Occasionally, small amount of
meconium may be passed. Features of intestinal
obstruction develop with prominent abdominal
wall veins and dilated loops of bowel with visible
peristalsis (Fig. 32.5).
Fig. 32.6 High jejunal atresia (white arrow pointing to a
third air-fluid level)
Radiological Diagnosis
Diagnosis can usually be made on erect and
supine skiagram of the abdomen. Dilated loops
of bowel with air-fluid levels suggest obstruction.
Further distal obstruction is associated with
larger number of air-fluid levels (Figs. 32.6, 32.7,
and 32.8).
198
Fig. 32.7 Mid-ileal atresia. Abdominal X-ray erect and
supine showing dilated small bowel loops with air-fluid
levels; note absence of gas in the pelvis. Features are sug-
gestive of intestinal obstruction
Types (Grosfeld classification) (Fig. 32.9):
I—Mucosal (membranous) atresia with intact
bowel wall and mesentery (Fig. 32.10).
II—Blind ends are separated by a fibrous
cord.
IIIa—Blind ends are separated by a mesen-
teric defect (V-shaped gap) (Fig. 32.11).
IIIb—Apple peel atresia.
IV—Multiple atresia (string of sausages).
In newborn, differentiation of large bowel
from small bowel on plain X-ray is difficult as the
bowel characteristics are not seen. Contrast
enema may be useful to differentiate low small
bowel obstruction from colonic obstruction.
Differential diagnosis of distal small bowel
obstruction includes ileal atresia, meconium
ileus, and total colonic aganglionosis.
32 Neonatal Intestinal Obstruction
Fig. 32.8 Distal ileal atresia. Note multiple air-fluid lev-
els. Features are suggestive of intestinal obstruction
Treatment
Preoperative preparations are done with nasogas-
tric aspiration, intravenous hydration, mainte-
nance of temperature, and injection of vitamin K
and antibiotics.
Surgery
Resection of the atretic part and end-to-end
anastomosis is performed. The proximal bowel
may be very distended and atonic. This atonic
portion needs to be resected. If resection of the
dilated segment is not possible, then tapering
can be considered. It is absolutely imperative to
rule out any distal obstruction by milking the
bowel or flushing with saline through a tube
(Fig. 32.12).
Intestinal Atresia and Stenosis 199

Fig. 32.9. Classification Types of intestinal atresias

of intestinal atresia

I II

IIIa IIIb

IV

Fig. 32.11 Small bowel atresia, note V-shaped gap in the

Fig. 32.10 Jejunal web mesentery

Malrotation with Volvulus born period with bilious vomiting (resembles

This is associated with extrinsic duodenal
obstruction due to a band from the colon
(Ladd’s band) going across the second part of
the duodenum. It usually presents in the new-
duodenal atresia).
Presentation
• Malrotation without volvulus presents with
bilious vomiting in an otherwise normal child.
200
Fig. 32.12 Small bowel atresia with intraluminal pellets
which needs emptying by flushing through an enterotomy
Fig. 32.13 Malrotation with volvulus (arrow pointing at
the twist)
Fig. 32.14 Midgut volvulus with gangrene
32 Neonatal Intestinal Obstruction
• Malrotation with acute volvulus (Figs. 32.13 and
32.14): Irritable sick child, abdominal pain,
abdominal distension with tenderness, and guard-
ing. This is a surgical emergency as the gangrene
of the twisted bowel can set in. Bleeding per rec-
tum may be present in late presenters.
• Malrotation with chronic volvulus: Chronic
abdominal pain and malnutrition.
Diagnosis and Management
Abdominal X-ray: Double bubble sign will be
present in cases with significant duodenal
obstruction. Small bowel loops will present pri-
marily on the right side.
USG of the abdomen: Can detect presence of
volvulus (whirlpool sign) and can also suggest
malrotation by examining the relationship of the
superior mesenteric artery and vein. The vein is
to the left of the artery in malrotation.
Contrast upper GI study: Duodenojejunal
junction present on the right side of vertebral
column. “Z”-shaped duodenojejunal loop is fre-
quently present. Corkscrew appearance is pres-
ent in case of volvulus (Fig. 32.15).
Operative procedure (Ladd’s procedure)
involves counterclockwise derotation of bowel,
division of Ladd’s band, widening the base of the
mesentery, placing small bowel on the right side
and large bowel on the left side of the abdomen,
and an appendectomy (optional).
Meconium Ileus
This condition is a neonatal manifestation of cystic
fibrosis inherited as an autosomal recessive genetic
defect. The intestine becomes impacted with thick,
viscid, inspissated meconium due to lack of pan-
creatic enzymes (Fig. 32.16). The neonate presents
with features of intestinal obstruction.
Abdominal X-ray
• Air-fluid levels are generally absent in abdom-
inal X-ray as the thick viscid meconium does
not form air-fluid level.
• Meconium mixed with air can give ground-­
glass appearance in the right lower quadrant.
Intestinal Atresia and Stenosis 201

Fig. 32.15 Upper

gastrointestinal contrast
study showing
corkscrew appearance of
the duodenum
suggestive of
malrotation

Contrast enema may show a microcolon Surgical treatment is required if conservative

(Fig. 32.17).
Low level of fecal trypsin, high level of
sodium chloride in the sweat, and DNA analysis
for cystic fibrosis clinch the diagnosis. Of these
tests, sweat chloride cannot be performed in a
neonate because of insufficient amount of sweat
produced for analysis.
Nearly 40% of cases may be complicated with
volvulus, atresia, or meconium peritonitis. An
enema with gastrografin is diagnostic (Fig. 32.17)
as well as therapeutic as it may d­isplace the
obstructing meconium and relieve the obstruc-
tion owing to its high osmolarity and detergent
action. Simple meconium ileus often responds to
enemas using N-acetyl cysteine or gastrografin.
treatment fails and in complicated meconium ileus.
An enterotomy with manual evacuation of the meco-
nium, resection of a segment of dilated ileum, and
end-to-side anastomosis with a distal venting ileos-
tomy stoma (Bishop-­Koop operation) is required for
irrigation of bowel in the postoperative period
(Fig. 32.18). Additional irrigation with N-acetyl cys-
teine may help in evacuation of viscid meconium.
Meconium Plug Syndrome
This is a condition where there is delayed or diffi-
cult passage of neonatal first meconium due to
202 32 Neonatal Intestinal Obstruction

Fig. 32.16 Meconium ileus

Fig. 32.17 Barium enema showing meconium ileus with

microcolon

a Mikulicz resection b Bishop-Koop resection

c Santuli and Blanc d Tube enterostomy

Fig. 32.18 Surgical procedures for meconium ileus

Suggested Reading
Fig. 32.19 Intraperitoneal calcification due to meconium
peritonitis
thick sticky plug of meconium with a firm head
which often dislodges following rectal stimulation
or an enema. Once the meconium plug is passed,
203
the obstruction gets relieved. However, persistence
of symptoms calls for further investigations as this
could be associated with cystic fibrosis, small left
colon syndrome, Hirschsprung disease, hypothy-
roidism, maternal narcotic addiction, and neuronal
intestinal dysplasia. A sweat chloride test to rule
out cystic fibrosis and a suction rectal biopsy to
exclude Hirschsprung’s disease are reasonable.
Meconium Peritonitis
Peritoneal calcification on abdominal X-ray indi-
cates intrauterine perforation with meconium
peritonitis (Fig. 32.19). Large single air-fluid
level indicates a giant meconium pseudocyst
associated with meconium peritonitis. Treatment
is always surgical (see above).
Suggested Reading
Coran AG, Caldamone A, Scott Adzich N, Krummel TM,
Laberge JM, editors. Pediatric surgery. 7th ed. Oxford:
Elsevier Limited; 2012.
Ladd AP, Rescorla SJ, Grosfeld JL, editors. Handbook
of pediatric surgical patient care. 1st ed. Singapore:
World Scientific Publishing Company; 2014.
 Necrotizing Enterocolitis
33

Necrotizing enterocolitis (NEC) is characterized ENTERAL FEEDING

by varying grades of intestinal injury due to
diverse causes.
Necrotizing enterocolitis is the commonest Immature GI tract, Disruption of Pathogenic Bacterial
abdominal emergency in newborns. The inci- mucosal barriers Invasion

dence is 1–3 per 1000 live births, but increases to

30 per 1000 live births for low birth weight
neonates. NEC

Etiology
Necrotizing enterocolitis commonly affects
premature babies. Factors suspected for initia-
tion of the disease are enteral feeding, intesti-
nal ischemia, and enteric microorganisms
(Fig. 33.1). Progression of the disease is medi-
ated by mediators of inflammation like tumor
necrosis factor-α (TNF-α), platelet-activating
factor (PAF) and nitric oxide (NO). Although
multiple factors may affect the development
of NEC, the final effector pathway remains the
same that a premature newborn with immature
gut or a mature newborn with some prena-
tal stress with altered gut barrier or immunity
are affected. A virulent organism then takes
advantage of this factors and sets in motion an
inflammatory cascade mediated by a number of
cytokines which ultimately result in ischemia
and necrosis of the bowel.
Hypoxic-Ischaemic Injury
Fig. 33.1 Pathogenesis of NEC
single segment or can have multiple skip lesions.
The most fulminant form is NEC totalis in which
more than 75% of the bowel is involved.
Presentation
1. Initially, nonspecific physiological signs like
temperature instability, apnea, bradycardia,
and lethargy are noticed.
2. Signs suggestive of gastrointestinal involve-
ment include abdominal distension, bleeding
per rectum, and feed intolerance with high
pre-feed aspirates.
3. Progressive disease results in abdominal
redness and tenderness (Fig. 33.2), abdomi-
­

NEC usually involves the terminal ileum fol- nal mass, abdominal wall crepitus, and
lowed by the colon. The disease can involve a pneumoperitoneum.

© Springer Nature Singapore Pte Ltd. 2018 205

S. Roy Choudhury, Pediatric Surgery, https://doi.org/10.1007/978-981-10-6304-6_33
206 33 Necrotizing Enterocolitis

4. Laboratory signs include acidosis, thrombo-

cytopenia, neutropenia, and disseminated
intravascular coagulation.
5. Radiological signs include intestinal dilatation,
persistent dilated loop, pneumatosis intestina-
lis, ascites, portal venous gas, and pneumoperi-
toneum (football sign) indicating intestinal
perforation (Figs. 33.3a, b and 33.4a, b).

Classification

Severity of the disease is classified according to

Bell’s classification as stage I (suspected), stage Fig. 33.2 Abdominal wall erythema and gastrointestinal
II (definite), and stage III (advanced) (Table 33.1). bleeding (NG tube) due to NEC

Fig. 33.3 (a, b)

Pneumoperitoneum with
a b
scrotal air

a b

Fig. 33.4 (a) NEC with pneumatosis

intestinalis. (b) Pneumoperitoneum
Solitary Intestinal Perforation (SIP) 207

Table 33.1 Modified Bell’s staging for NEC

Stage Clinical finding
I—Suspected Apnea, bradycardia,
temperature instability
IIa—Definite Apnea, bradycardia,
temperature instability
IIb Thrombocytopenia, mild
metabolic acidosis
IIIa—Advanced Mixed acidosis, oliguria,
hypotension, coagulopathy
IIIb Shock, deterioration in
laboratory values and vital signs
Radiological finding
Gas pattern of mild ileus
increased gastric residuals
Ileus gas pattern with one or
more dilated loops, focal
pneumatosis
Widespread pneumatosis,
ascites, portal venous gas
Prominent bowel loops,
worsening ascites, no free air
Pneumoperitoneum
Gastrointestinal finding
Occult blood in stool, mild
abdominal distention
Grossly bloody stools,
prominent abdominal
distention, absent bowel
sounds
Abdominal wall edema with
palpable loops and tenderness
Worsening wall edema,
erythema, and induration
Perforated bowel

Treatment pneumoperitoneum secondary to NEC. Some

In suspected cases, initial medical management
consists of withdrawal of enteral feeding, nasogas-
tric tube decompression, and intravenous fluid and
initiation of parenteral nutrition. Close clinical,
radiological, and biochemical monitoring is neces-
sary. Serial abdominal radiographs are needed to
monitor the progress of the disease. Broad-
spectrum antibiotics are instituted. Once the child
stabilizes and adequate period of bowel rest has
passed (7–10 days) in cases of NEC, oral feeds are
initiated and gradually progressed.
neonates may become stable by PD itself and can
be managed conservatively, whereas neonates
who fail to stabilize within 24–48 h of drainage
need rescue laparotomy.
Surgery for focal disease involves limited
bowel resection with either end-to-end anastomo-
sis or enterostomy (stoma), depending on the sta-
tus of the retained bowel. Whereas for extensive
bowel involvement, a second look exploration
after a period of 24–48 h is an option, checking
for any viable bowel that could be preserved
(Fig. 33.5). Extensive NEC needing massive
bowel resection results in short bowel syndrome.

Indications for Surgery

(a) Presence of pneumoperitoneum
(b) Bowel necrosis
While pneumoperitoneum is relatively easy to
diagnose, the markers to detect intestinal necrosis
are limited. So various surrogate markers of
intestinal gangrene can be considered as relative
indications for surgery are fixed dilated loop of
bowel on serial X-rays, palpable mass, abdomi-
nal wall erythema, portal venous gas, positive
peritoneal fluid cultures, progression of illness
with worsening thrombocytopenia, acidosis, and
cardiovascular collapse.
Peritoneal drainage (PD) as a temporizing
procedure is often beneficial especially in
extremely low birth weight babies (<1000 g) with
Solitary Intestinal Perforation (SIP)
Solitary or isolated intestinal perforation (SIP) is
currently considered a separate entity from
NEC. It is seen in very premature babies with
perforation usually involving the terminal ileum
spearing the rest of the bowel (Fig. 33.6).
Radiologically prominent pneumoperitoneum
and absence of pneumatosis is suggestive of
SIP. Surgical treatment includes peritoneal drain-
age only or drainage followed by laparotomy in
unresponsive patients. Intestinal perforation may
be managed by repair or resection of the involved
segment. The outcome of SIP appears to be better
than NEC.
Outcome: The mortality rate is high in NEC
(15–30%).
208
Fig. 33.5 Patchy bowel necrosis
Fig. 33.6 Sigmoid colon perforation
Complications
Stoma-Related Complications:
• Post-NEC stricture (Fig. 33.7) that may be
focal or multiple, occurs as a result of healing
of NEC.
• Cholestatic liver disease due to prolonged
TPN.
• Short bowel syndrome in cases of extensive
bowel loss.
• Growth and neurodevelopmental problems.
33 Necrotizing Enterocolitis
Fig. 33.7 Bowel stricture due to NEC
Prevention of NEC
Interventions to increase gut immunity and matu-
ration include maternal glucocorticoid supple-
mentation and administering breast milk and oral
immunoglobulins.
Measures to prevent injury to gut mucosa are:
• Feeding regimes
• Trophic feeds to be initiated early
• Low volume feeds
• Gradual increase in fees
• Colonization by commensal bacteria by use of
probiotics
• Controlling inflammation by use of anti-­
inflammatory molecules like PAF (platelet-­
aggregating factor) antagonist or degrading
enzymes
Suggested Reading
Coran AG, Caldamone A, Scott Adzich N, Krummel TM,
Laberge JM, editors. Pediatric surgery. 7th ed. Oxford:
Elsevier; 2012.
Puri P, Hollwarth ME. Pediatric surgery, diagnosis and
management. Heidelberg: Springer; 2009.
 Meckel Diverticulum and
Duplications of the Intestine 34

Meckel diverticulum is a true diverticulum, i.e., it

contains all the layers of the bowel wall and is
present in the distal small bowel.

Embryology

Meckel diverticulum is a remnant of the embry-

onic vitellointestinal duct (see chapter 21).

Pathology
Meckel diverticulum can be described by a rule
of 2’s: It is present in 2% of population, usually
2 in..long and 2 cm in diameter, usually discov-
ered around 2 years of age, and two times more
common in males (only symptomatic cases),
has two types of heterotopic mucosa (gastric
and pancreatic), and is situated 2 ft from the
ileocecal valve (Fig. 34.1). It possesses all
three coats of the intestine and has independent
blood supply. The mucosa may contain het-
erotopic epithelium (gastric, colonic, or pan-
creatic epithelium) in 20% cases, which may
cause ulceration and bleeding at the base of the
diverticulum.
Fig. 34.1 Laparoscopic view of Meckel diverticulum
Presentation
Meckel diverticulum remains asymptomatic in
most cases with a 4–6% lifetime risk of becoming
symptomatic. Meckel’s diverticulum may be dis-
covered incidentally at laparotomy or present with
symptoms, which are intestinal bleeding, abdomi-
nal pain, and intestinal obstruction or perforation.
Children with symptomatic Meckel’s present with
hemorrhage (40%), intestinal obstruction (30%),
diverticulitis (20%), and umbilical discharge (6%)
(Figs. 34.2, 34.3, and 34.4).

© Springer Nature Singapore Pte Ltd. 2018 209

S. Roy Choudhury, Pediatric Surgery, https://doi.org/10.1007/978-981-10-6304-6_34
210 34 Meckel Diverticulum and Duplications of the Intestine

• Intestinal obstruction: Can be due to the diver-

ticulum acting as a lead point and causing
intussusception, internal hernia, volvulus
around a band from the diverticulum to the
umbilicus (Fig. 34.3), and bowel prolapse
through the umbilicus.
• Inflammation: Heterotopic mucosa is com-
monly present in an inflamed diverticulum.
Many cases present with symptoms sugges-
tive of appendicitis. Therefore, the finding of a
normal appendix in a child being operated
Fig. 34.2 Perforated Meckel diverticulum at open with presumptive diagnosis of appendicitis
surgery should prompt for the examination to look for
the presence of Meckel’s diverticulum.

Diagnosis

Diagnosis is related to presentation. Bleeding

Fig. 34.3 Meckel diverticulum with band to umbilicus
resulting in intestinal obstruction due to volvulus
Meckel diverticulum may be diagnosed on occa-
sion by 99mTc sodium pertechnetate scan
(Fig. 34.5). Various drugs are used to promote
uptake of the radionuclide (pentagastrin) or
retention of the drug in the ectopic mucosa (H2
receptor blockers) and the intestine (glucagon).
This test has a high false-negative predictive
value. Angiography is often useless to demon-
strate the site of bleeding as it requires bleeding
more than 0.5 ml/min which is often not the case.
Persistent bleeding requires laparotomy or lapa-
roscopy even if Meckel scan is negative.
Diverticulitis or perforation presents with find-
ings similar to appendicitis. Obstruction second-
ary to intussusception or volvulus around a band
is diagnosed by clinical findings, ultrasonogra-
phy and X-ray.

Treatment

Symptomatic Meckel diverticulum warrants

Fig. 34.4 Gangrenous Meckel diverticulum removal. Since the heterotopic mucosa is often
present at the base of the diverticulum, the base
• Bleeding: Usually, a brick red- or maroon-­ along with a portion of normal adjoining intes-
colored stools are passed but if bleeding is tine needs to be removed. Wedge resection can
severe, then, fresh blood may be passed. be performed in most patients. In cases where
Bleeding occurs due to the presence of ectopic the base is unhealthy or is very wide, resection
mucosa (usually gastric) at the base of the and end-to-end anastomosis of the bowel are
diverticulum. ­performed. Asymptomatic Meckel ­diverticula
Duplications of Intestine 211

Fig. 34.5 Hot spot in nuclear scan suggestive of Meckel

diverticulum
Fig. 34.6 Cystic duplication of the intestine
identified incidentally should be removed if
narrow mouthed or upon palpation there is
questionable ectopic (gastric or pancreatic)
mucosa.

Duplications of Intestine

They are congenital intestinal anomalies which

have the following three characteristics:

1. Presence of well-developed smooth muscle
2. An epithelial lining representing gastrointesti-
nal mucosa
3. Intimate association with some part of alimen-
tary tract
Incidence: They usually present before the age
of 2 years, incidence being 1 in 4500 live births.
Fig. 34.7 Tubular duplication of the intestine (note dou-
ble bowel with two lumens)
mouth to the anus. They could be asymptomatic
and detected incidentally; however, symptom-
atic ones can present with obstruction or bleed-
ing due to the presence of ectopic gastric or
pancreatic mucosa.

Etiology
• Persistence of neurenteric canal
• Partial or abortive twinning
• Diverticula of embryonic intestine and recana-
lization defects
They are associated with vertebral, spinal,
and genitourinary anomalies in 40–50% of
patients. Two types are seen, cystic or tubular
(Figs. 34.6 and 34.7). They may be found any-
where in the gastrointestinal tract from the
Investigations
• Sonography may show an outer and inner tube
with different echogenicity (gut signature
sign) and is useful in the diagnosis.
• CECT scan is also very helpful particularly
for knowing the extension in cases of
­thoracoabdominal duplications and for detect-
ing associated vertebral anomalies.
• Tc-99m pertechnetate scan can detect ectopic
gastric mucosa.
212
a b
Fig. 34.8 (a, b) Chylous cyst of the intestine
Fig. 34.9 Lymphangioma of the intestine
Treatment
Surgical removal is the treatment of choice for
duplications. They share a common blood supply
with the native intestine. So resection of the
­associated normal intestine is also needed. Large
thoracoabdominal duplication can be removed in a
staged manner. If the whole cyst cannot be removed
due to its close relation with the organ, its lining
should be removed leaving the outer wall. Long
colonic duplications can also be managed by con-
necting the colons both proximally and distally.
Mesenteric Cyst
Incidence: They are rare lesions and account for
1 in 20,000 admissions to a pediatric hospital.
34 Meckel Diverticulum and Duplications of the Intestine
Classification
• Lymphatic origin (chylous or chylolymphatic
cyst, Figs. 34.8a, b and 34.9)
• Mesothelial origin
• Duplication cyst
• Teratoma
• Non-pancreatic pseudocyst
They may become symptomatic with recurrent
pain and features of obstruction. Surgical resection
of the lesion along with the involved bowel and
anastomosis results in complete cure. In about 10%
of cases, the lesion is very extensive which pre-
cludes complete excision. In these cases, partial
excision with marsupialization of the remaining
intestine is performed. The cyst lining in these cases
should be destroyed by electrocautery, or tincture of
iodine, or hypertonic saline. Extensive lymphangi-
oma of the intestine is extremely rare (Fig. 34.9).
Suggested Reading
Coran AG, Caldamone A, Scott Adzich N, Krummel TM,
Laberge JM, editors. Pediatric surgery. 7th ed. Oxford:
Elsevier; 2012.
Puri P, Hollwarth ME. Pediatric surgery, diagnosis and
management. Heidelberg: Springer; 2009.
 Intussusception
Intussusception is defined as telescoping of a por-
tion of the gut into another immediately adjacent
to it, usually from proximal to distal. Very rarely
retrograde intussusception can occur, e.g., through
gastrojejunostomy. It is one of the most common
causes of intestinal obstruction in infants.
Etiology
1. Idiopathic variety is common in children occur-
ring in 90% of cases, often associated with pre-
ceding viral illness in 20–30% of cases.
2. Gastroenteritis often precipitates it.
3. Probable explanation is that the change in diet
is associated with alteration in gut flora lead-
ing to inflammation of Payer’s patches which
swells up and acts as a leading point. Seasonal
occurrences with upper respiratory infection,
adenovirus, and rotavirus infection are seen.
4. Pathological lead pint (PLP) is frequently
present in older children and adults. The most
common PLP is inverted Meckel’s diverticu-
INTUSSUSCEPIENS
Fig. 35.1 Pathology of
intussusception
© Springer Nature Singapore Pte Ltd. 2018
S. Roy Choudhury, Pediatric Surgery, https://doi.org/10.1007/978-981-10-6304-6_35
35
lum followed by intestinal polyps (inflammatory
fibroid polyp, hamartomatous polyp of Peutz-
Jeghers syndrome).
Age: Usually occurs in infants aged between 6
and 9 months, associated with change in diet
(weaning).
Generally occurs in the last 50 cm of small
bowel where maximum aggregation of Payer’s
patches is present.
Pathology (Figs. 35.1, 35.2, and 35.3)
Intussuscepiens: Outer (covering) tube
Intussusceptum: Inner (entering) tube
Apex: Tip of the inner tube (lead point)
Neck: Entry point.
Mechanical bowel obstruction results due to
obstruction of the lumen. The mesenteric vessels
get strangulated which results in local edema
and venous congestion. The venous congestion
causes oozing of blood and mucus from the intus-
INTUSSUSCEPTUM
APEX
NECK
213
214
Fig. 35.2 Intussusception
Fig. 35.3 Ruptured intussusception with gangrenous
head
susceptum resulting in characteristic red currant
jelly stools. Finally, ischemia sets in which results
in bowel necrosis in cases with late presentation.
Apex is the most vulnerable site for gangrene.
Types with Frequency of Occurence
Ileoileal, 5%; ileocolic, 77%; ileoileocolic, 12%;
colocolic, 2%; multiple, 1%; retrograde, 0.2%;
others, 2.8%.
Clinical Features
Usually, a lusty male child aged between 6 and
9 months presents with sudden onset of paroxys-
mal abdominal pain, screaming, drawing up legs,
vomiting, and facial pallor. In between attacks,
the child becomes listless and drawn. This gradu-
ally becomes normal till the next bout of pain.
Initially, the baby passes normal stool and then
35 Intussusception
the character changes to blood and mucous
termed “red currant jelly.”
Examination in a quiet environment with
warm hand will help to detect a sausage-shaped
lump with concavity toward the umbilicus and
emptiness of the right iliac fossa called “sign de
dance.” On rectal examination, the apex of the
intussusception may be felt and red currant jelly
staining on the finger can be seen. Features of
intestinal obstruction with vomiting, abdominal
distension, and visible bowel loops develop with
progression of time.
Diagnosis
(a) Abdominal X-ray findings include increased
bowel gas shadow, absence of cecal gas, and
multiple air-fluid levels. The presence of a
normal gas-filled colon nearly rules out
intussusceptions.
(b) Ultrasonography can detect the mass with
characteristic “target sign” (loop of bowel
within loop). Pathological lead point can also
be seen.
(c) Contrast enema with air or barium can demon-
strate the head as a filling defect called “claw
sign” (not seen in ileoileal intussusception).
Differential Diagnosis
(a) Acute enterocolitis: Generally associated
with passage of fecal matter with blood (in
contrast with only blood and mucous in cases
with intussusception).
(b) Henoch-Schonlein purpura: Intussusception
may be precipitated.
(c) Prolapse of the rectum: Examination of the
finger insinuated between the mass and rectal
wall will halt at the vault.
Treatment
1. Initial treatment starts with keeping the patient
nil by the mouth and hydration with intrave-
nous fluids. A nasogastric tube needs to be
inserted if the child is vomiting or the abdo-
men is distended.
Treatment
2. Radiological reduction can be done in the fol-
lowing ways:
• Hydrostatic reduction
–– Using water soluble contrast introduced
through a rectal catheter under fluoroscopic
guidance
–– Alternatively using saline introduced
through a rectal catheter under ultrasono-
graphic guidance
• Pneumatic reduction: Introducing air
through a rectal catheter under fluoroscopic
guidance
During hydrostatic reduction the rule of 3’s is
followed keeping the can at a height at 3 ft, for
3 min, and for maximum three attempts.
Air-enema reduction technique: Applying
air pressure up to 80–100 mm of Hg, with the
help of a three-way Foley catheter inserted
rectally and attached to an air insufflation/
pressure gauze device under fluoroscopic
control.
The success rate is close to 80–90%, even
higher in early cases.
Absolute contraindications for enema reduc-
tion are features of peritonitis, suspected perfo-
a b
Fig. 35.4 (a, b) Inverted Meckel diverticulum causing intussusception
215
ration, and shock. In cases with prolonged
obstruction, the chance of success is poor.
Signs of success for enema reduction are pas-
sage of flatus and feces and contrast seen entering
in small bowel loops during the procedure.
3. Operative reduction by laparotomy or lapa-
roscopy (when radiological reduction is con-
traindicated or failed): Operative steps are
identification of the sausage-shaped mass,
starting squeezing from the apex, avoiding
pulling as it can cause rupture, and compres-
sion with warm saline pack which reduces
edema, thus helping in reduction.
In difficult cases pressure squeeze to reduce
edema of the ileocecal valve (Copes method)
may be attempted.
Bowel resection is required for irreducible and
gangrenous intussusception.
If a lead point is found like Meckel’s divertic-
ulum (Fig. 35.4a, b) or polyp (Fig. 35.5), then
resection is recommended.
4. Post reduction recurrence rate is between 2%
and 3%.
216 35 Intussusception

Suggested Reading
Coran AG, Caldamone A, Scott Adzich N, Krummel TM,
Laberge JM, editors. Pediatric surgery. 7th ed. Oxford:
Elsevier; 2012.

Fig. 35.5 Intussusception with a large polyp as lead point

 Anorectal Malformations
The structural abnormalities associated with ano-
rectal malformation or the commonly used termi-
nology “imperforate anus” are now better
understood; however, the surgical management
of this condition is still evolving.
Embryology
The lower part of the hindgut forms the cloaca which
divides into urogenital sinus and post-­allantoic gut
by the development of the urorectal septum. Rupture
of the cloacal membrane opens the anus to the exte-
rior. Any interference with formation of urorectal
septum or growth of mesenchymal tissue around
cloaca results in anorectal malformation.
Incidence
Incidence is 1 in 4500 live births; slight male pre-
ponderance is present.
Associated Abnormalities
Associated abnormalities are present in 50–70%
of cases. Spinal malformations, renal and cardiac
abnormalities are common; hence, a child with
ARM should be evaluated by renal and spinal
ultrasound along with echocardiography. A naso-
gastric tube should be passed to rule out associ-
ated esophageal atresia.
© Springer Nature Singapore Pte Ltd. 2018
S. Roy Choudhury, Pediatric Surgery, https://doi.org/10.1007/978-981-10-6304-6_36
36
Types
Traditionally anorectal malformations have been
classified as high, intermediate, and low
­(wingspread) depending on the termination of the
gut above or below the pelvic floor (supralevator
or infralevator) (Fig. 36.1).
 sual Characteristics of High/
U
Intermediate Anorectal
Malformations (HARM)
Anorectum ends in fistula with the urinary
tract in males and vagina or vestibule in females.
Deficient sphincter muscles.
Abnormal bony sacrum.
Higher incidence of associated anomalies.
Poor outcome in terms of achieving continence.
Treatment is more challenging.
 sual Characteristics of Low
U
Anorectal Malformations (LARM)
Anorectum ends in fistulous connection to the
exterior.
Good sphincter muscles.
Normal sacrum.
Better chance of achieving continence.
Simpler treatment.
In male, high anomaly is common (90%) with
the lower end of the rectum ending in a fistulous
217
218 36 Anorectal Malformations

Male perineal fistula Recto-bulbar fistula Recto-prostatic fistula Recto-bladder neck fistula

Female perineal fistula Vestibular fistula Cloaca

Fig. 36.1 Anatomical types of male (above) and female (below) anorectal malformations

communication with the urethra. In female patients, Clinical Presentation

low anomaly with fistulous communication to the
exterior is common (90%). Imperforate anus with- In the newborn
out a fistulous communication is rare (5%) and has
a high association with Down syndrome. 1. Sign of LARM: Meconium filled tract and
meconium discharging perineal fistula are
definite signs of LARM (Fig. 36.2a–d).
Classification (Krikenbech) 2. Female patient with vestibular or perineal fis-
tula may present late as they often decompress
Major clinical groups through the fistula (Figs. 36.3 and 36.4).
Perineal canal or H type rectovaginal fistula
Perineal (cutaneous) fistula (Fig. 36.5) presents with passage of fecal mat-
Rectourethral fistula ter per vaginum in addition to the anal
Bulbar passage.
Prostatic 3. Sign of HARM: Meconium in urine (recto-­
Rectovesical fistula urinary fistula) in males and single perineal
Vestibular fistula opening (cloaca) in females are diagnostic of
Cloaca HARM (Figs. 36.6a, b, 36.7, 36.8, and 36.9).
No fistula Presence of flat bottom is suggestive of
Anal stenosis HARM (Figs. 36.6 and 36.7).
4. Children with Down syndrome and ARM are
Rare/regional variants almost always associated with HARM with-
out fistula.
Pouch colon 5. Look for associated malformations (men-
Rectal atresia/stenosis tioned above).
Rectovaginal fistula
H-type fistula
Others
Clinical Presentation 219

a b

c d

Fig. 36.2 (a–d) Low anorectal malformations in male. Note meconium filled tract (a–c), fistulous opening (c), skin tag (d)
220 36 Anorectal Malformations

Fig. 36.4 Anterior perineal (ectopic) anus

Fig. 36.3 Ano vestibular fistula (arrow)

a b

Fig. 36.5 (a, b) H-type fistula (perineal canal). (a) Opening in vagina, (b) anal opening
Clinical Presentation 221

a b

Fig. 36.6 (a) HARM, no anal opening, flat perineum. (b) Sigmoid divided colostomy

Fig. 36.7 Absence of anal opening, female HARM Fig. 36.8 Small appearing genitalia in Cloaca
222 36 Anorectal Malformations

Fig. 36.9 Genital appearance in Congenital Pouch Colon

(new born)

Investigations

Invertogram is recommended after 18 h of birth for

allowing the bowel air to reach the lower rectum. It
Fig. 36.10 Invertogram showing low ARM
is better avoided in cases with associated tracheo-
esophageal fistula due to the risk of aspiration.
Radio-opaque marker is placed at the bottom, the
child is held upside down with slight flexion at the
hip joint, and greater trochanters are aligned. The
lower limit of the gas shadow is evaluated along
with an attempt to identify o­bvious associated
abnormality like sacral agenesis. Earlier this
shadow was interpreted in relation to bony land-
marks of the pelvis like pubococcygeal line (PC
line) and I line. PC line is the line joining the pubis
to coccyx and I line is the line parallel to PC line
but passing through the lower border or ischial
tuberosity. Termination above PC line indicates
HARM and below suggests intermediate or
LARM (Figs. 36.10 and 36.11).
Now a days cross-table prone X-ray (Rao) with
buttocks of the baby elevated is preferred over an
invertogram (Fig. 36.12). Relation of rectal gas to
coccyx is given paramount importance in deciding Fig. 36.11 Invertogram showing high ARM
the management. If rectal gas is above the coccyx,
then colostomy is performed, but if it is below, width of the abdomen suggests ­congenital pouch
then primary repair is preferred. Distance of bowel colon, a variant of HARM more commonly seen
gas shadow more than 1 cm from the perineum is (15% of ARM) in northern India (Figs. 36.13 and
taken as intermediate or high anomaly. A large air- 36.14). Ultrasonography may also be applied to
fluid shadow occupying more than 50% of the determine the level of termination of the rectum.
Management 223

Management (Figs. 36.15 and 36.16)

Initial stabilization is done with withdrawal

of feeds, nasogastric aspiration, intravenous
fluids, antibiotics, injection of vitamin K,
and thermoregulation. Additional investiga-
tions to rule out associated abnormalities
include USG of spine, genitourinary and an
echocardiography.
Fig. 36.12 Cross table prone lateral view (Narasimha
Rao) showing HARM

a b

Fig. 36.13 (a, b) Large air-fluid level suggesting congenital pouch colon

a b

Colovesical fistula
Pouch

Fig. 36.14 (a) Congenital Pouch Colon, (b) rectum terminating in high urinary tract fistula
224 36 Anorectal Malformations

Rule out associated anomalies

like esophageal atresia
(VACTERL)
Perineal examination

Perineal fistula or other

evidence of low anomaly Flat perineum, meconium in
(meconium filled tract, covered urine
anus)

Invertogram/cross lateral
Anoplasty/ Limited PSARP
prone view after 24 h

Rectal gas shadow above

Rectal gas shadow below
coccyx, unstable patient,
coccyx, stable patient
pouch colon

Primary PSARP
Colostomy
with/without colostomy

Definitive surgery
PSARP

Colostomy closure
after 3 months

Fig. 36.15 Algorithm of management for male anorectal malformations

For low anomalies an early correction is pos-
sible if the child is stable without any major
associated abnormalities. The different surgi-
cal techniques are:
Cutback anoplasty
Anal dilation for anal stenosis
Anoplasty for male low ARM or limited pos-
terior sagittal ano-rectoplasty
Female patients with vestibular fistula. The
different techniques for female vestibular/
perineal fistula are anal transposition or
anterior or posterior sagittal ano-rectoplasty
For high anomalies the preferred treatment is ini-
tial colostomy followed by staged correction.
The different definitive surgical procedures
for correction of ano rectal anomalies are:
Posterior sagittal ano-rectoplasty (PSARP),
abdomino-perineal, abdomino-sacro-perineal,
and laparoscopic-assisted pull-through.
Currently, PSARP as described by Pena is the
most commonly performed operation Distal
cologram is invaluable in determining the
level of distal pouch and presence of urinary
fistula. Pressure augmented distal cologram
often fills up the the bladder and urethra
Management
Fig. 36.16 Algorithm
of management for
female anorectal
malformations
Three openings,
commonest
(Vestibular
fistula
Perineal fistula)
Decompressing
PSARP
±colostomy
through the fistula, thus eliminating the need
for an MCU (Fig. 36.17a–d). USG for renal
track is recommended.
Steps of PSARP operation for male patients
• Catheterization of the bladder
• Posterior midline (sagittal) incision extending
from the mid-sacrum to the perineum
• Division of the parasagittal fibers, muscle
complex, and levator ani muscles in the
midline; may need to split the coccyx with
the cautery in the midline for the cases with
the rectum ending in a high-level fistula
• Identification of the rectum and securing the
lower end with stay sutures
• Opening the rectum in the midline, identifying
the rectourethral fistula from inside
• Separation of the rectourethral fistula with an
initial anterior submucosal dissection fol-
lowed by full-thickness separation of the rec-
tum from the urethra
• Closure of the urethral fistula
• Adequate mobilization of the rectum for
bringing it to the perineum
• The rectum is placed within the muscle com-
plex, levator muscle approximated
• Completion of the anoplasty
225
Perineal examination
Two openings One opening
(Rectovaginal (Cloaca,
fistula or Pouch colon)
Vaginal agenesis)
Distended
Colostomy
abdomen
Definitive
surgical
reconstruction
Colostomy
closure 2–3
months later
For recto-bladder neck fistula cases (very high
ending), abdominal and/or laparoscopic approach
for division of the fistula and pull-through of the
anorectum is recommended.
Steps of PSARP operation for female patients
• Catheterization of the bladder.
• Posterior sagittal incision with division of the
muscles in the midline as above.
• Separation of the rectum from the vagina with
meticulous dissection.
• Reconstruction of the perineal body.
• The rectum is placed within the muscle com-
plex and anoplasty completed.
Management of Cloaca
The length of the common channel should be
assessed by endoscopy. Those patients with a
common channel length of less than 3 cm can be
managed by posterior sagittal approach as
described above. The rectum is separated from
the urogenital sinus and mobilized for perform-
ing an anoplasty. A total or partial urogenital
sinus mobilization (TUM/PUM) can aid in
bringing the urethra and vagina to the perineum.
Patients with a common channel length of more
than 3 cm require more extensive procedure
226 36 Anorectal Malformations

a b

c d

Fig. 36.17 (a, b) Distal cologram, rectourethral fistula (arrow). (c, d) Distal cologram, cloaca (black arrow showing
common channel). Note cervical impression above vagina (white arrow) (R-Rectum. V-Vagina, B-Bladder)

including a laparotomy and often vaginal Primary PSARP for imperforate anus can be
replacement with a segment of bowel. undertaken with

Anal dilation is started 2 weeks after the • Experienced surgeon

definite repair and colostomy can be closed • Good birth weight babies
after 6 weeks if adequate healing has taken • No associated anomalies
place. • Bowel gas shadow extending below the coccyx
Suggested Reading
Advantages of primary PSARP
• Avoids repeated surgeries and anesthesia
• Avoids colostomy complications
• Avoids recurrent UTI
• Avoids scars
• Early development of ano-cerebro-cortical reflux
Advantages of laparoscopic-assisted anorectal
pull-through (LAARP)
• Avoid division and weakening of external
sphincter
• Diminishing perirectal scarring
• Good visualization of fistula
• Precise placement of the rectum within the
external sphincter
227
Assessment of Fecal Continence
The following functions are assessed:
(a) Voluntary bowel movement
(b) Soiling
(c) Constipation
Several scoring systems (Kelly, Krikenbech)
for continence assessment are available in chil-
dren above 3 years. Management of fecal incon-
tinence includes bowel management program as
discussed in chapter 40.
Suggested Reading
Coran AG, Caldamone A, Scott Adzich N, Krummel TM,
Laberge JM, editors. Pediatric surgery. 7th ed. Oxford:
Elsevier; 2012.
Holschneider AM, Hutson JM, editors. Anorectal mal-
formations in children embryology, diagnosis, sur-
gical treatment, follow-up. Heidelberg: Springer;
2006.
 Acquired Anorectal Disorders
37

Acquired anorectal problems are common cause

for visit to the doctor. The common acquired ano-
rectal disorders in children are:

• Perianal abscess
• Fistula-in-ano
• Anal fissure
• Anal tag and hemorrhoids
• Rectal prolapse

Perianal Abscess and Fistula-in-Ano

Perianal or perirectal abscesses are not uncom-

mon during infancy. They are often seen in males
as it is proposed that imbalance in androgen
causes production of abnormal anal glands that
are prone to infection.
The abscess typically presents as a firm, tender
mass in the perianal region often with discharging
pus (Fig. 37.1). Sitz baths and antibiotics are pre-
scribed if the abscess does not appear to be fluctu-
ant. Approximately one third of abscesses thus
treated resolve, rest require incision and drainage
usually performed as an outpatient procedure.
As many as 40–50% of perianal abscesses
leads to the formation of a fistula-in-ano. The
child usually presents after two or more flare-ups
of a perianal abscess that either continues to drain
or forms a small pustule that ruptures, only to
form again. The fistula is commonly located lat-
Fig. 37.1 Ruptured perianal abscess
eral to the anus rather than in the midline
(Fig. 37.2). The preferred surgical procedure for
fistula-in-ano is fistulotomy. With the patient
under general anesthesia, the anus is gently seri-
ally dilated. A fine malleable probe is inserted
through the fistula and gently advanced until it is
visualized to exit the base of the involved crypt.
An incision is made along the probe and is deep-
ened until the whole fistula track is laid open over
the probe. The base of the exposed fistula is
denuded of granulation tissue with a curette. The
wound is left open to heal with the aid of sitz bath
and maintenance of local hygiene. Recently,
conservative management of fistula is being
­

© Springer Nature Singapore Pte Ltd. 2018 229

S. Roy Choudhury, Pediatric Surgery, https://doi.org/10.1007/978-981-10-6304-6_37
230 37 Acquired Anorectal Disorders

the anal sphincter. Most anal fissures will heal by

these measures. In symptomatic or non-­
responding cases, chemical sphincterotomy can
be performed by topical application of glyceryl
trinitrate ointment or local injection of botulinum
toxin.
Surgical management is reserved for children
who fail conservative management, and options
available are fissurectomy, anal stretching, or lat-
eral sphincterotomy.
An anal fissure in an older child or a teenager
is often associated with chronic inflammatory
bowel disease, usually Crohn’s disease in the
Western world.

Fig. 37.2 Fistula-in-ano; note the lateral site

Anal Tag and Hemorrhoids

advocated in infants as many of these lesions heal A perianal skin tag may result from a healed fis-
spontaneously. Acute flare-ups are managed sure (Fig. 37.3). It is generally of no consequence
accordingly during conservative management. and can be managed conservatively. The large
one can be bothersome and can affect adequate
perianal hygiene. In these cases, local excision is
Anal Fissure reasonable. Hemorrhoids are extremely uncom-
mon in the pediatric population. Children with
Anal fissure is a longitudinal tear or an ulcer in portal hypertension are likely to develop hemor-
the distal anal canal. The cause in children is a rhoids but are usually asymptomatic. The usual
period of constipation followed by hard bulky presentation is of bleeding, pain, prolapse, and
stool that results in a posterior midline tear in the itching. Most children respond to conservative
anoderm below the mucocutaneous junction. The measures like sitz bath, management of constipa-
discomfort in having a bowel movement associ- tion, and high roughage diet. Surgical therapy is
ated with the fissure often leads to further consti- rarely necessary.
pation, which, in turn, aggravates the fissure with
each stool and prevents healing. This theory now
is considered oversimplification of the pathology.
The alternate theory proposes that the raised
sphincter pressure results in ischemia in the
posterior midline anal canal which ulcerates
­
leading to anal fissure. The diagnosis is made by
the ­history of blood streaking on the stool, the
child’s crying during bowel movements, and the
recognition of a tear in the distal anal canal.
Management of these patients includes sitz
baths and an osmotic stool softener such as lactu-
lose, and local analgesic (2–5% lignocaine jelly).
Sitz baths promote good anal hygiene and relax Fig. 37.3 Anal tag
Rectal Prolapse 231

Rectal Prolapse a

Rectal prolapse is a relatively common problem

in young children and causes great distress to the
child and the parents. Prolapse can range from an
intermittent mucosal prolapse that occurs during
defecation and reduces spontaneously to full-­
thickness prolapse of the rectum, which requires
manual reduction.
Rectal prolapse in children is likely precipi-
tated by:
b
• Weakness of the pelvic supporting muscula-
ture (levator mechanism).
• Loosely attached rectal submucosa to the
underlying muscularis.
• Straining during defecation because of pro-
tracted diarrhea or constipation.
• Parasitic infection may also promote prolapse.
• Decreased anal tone due to previous surgery.

Rectal prolapse can be classified as follows:

• Partial: Only the mucosa protrudes for 1–3 cm.

Radial folds are seen (Fig. 37.4a).
• Full thickness: Whole thickness of the rectal
wall prolapses. Concentric mucosal folds are
often seen (Fig. 37.4b).

Straining during defecation and long periods

of sitting on the toilet, because of protracted diar-
rhea or constipation (which may occur in cystic
fibrosis), allow stretching of the pelvic dia-
phragm, resulting in prolapse which may not
improve spontaneously. On the other hand, pro-
lapse associated with malnutrition and diarrhea
often improves with time.
Presentation of rectal prolapse is often with or
without concomitant bleeding. The prolapse
either reduces spontaneously as the child gets off
the toilet or the parent pushes it back in. Many
children very quickly learn to reduce their own
prolapse. It generally does not recur until the next
stool. It is uncommon for the child to be able to
produce the prolapse in the examining room.
Occasionally, the prolapse can be demonstrated
Fig. 37.4 (a) Mucosal rectal prolapse with radial folds.
(b) Full-thickness rectal prolapse with concentric folds in
a malnourished child
on attempted defecation. The typical prolapse is a
rosette of mucosa, sometimes slightly longer pos-
teriorly than anteriorly (Fig. 37.4a, b). One should
be able to slip a finger alongside the prolapse and
feel the sulcus 1–2 cm up inside the anus. It should
be differentiated from intussusceptions of the sig-
moid colon. A deeper sulcus suggests intussus-
ception rather than rectal prolapse (see Chap. 35).
232
Management of Rectal Prolapse
Acute prolapse needs immediate replacement. If
the prolapsed bowel does not stay inside, then but-
tocks should be taped after reduction. A change in
defecation habits and provision of stool soften-
ers that eliminates the persistent urge to defecate
may allow the pelvic musculature to resume its
normal tone. Giardiasis or parasitic infections
should be looked for in the stool examination and
adequately managed. Treatment of constipation
or diarrheal disorder should take precedence in
children. Similarly, improvement of nutritional
status is also important. In older children, pelvic
floor strengthening exercises should be taught. It
is necessary to give a period of conservative man-
agement of children with prolapse to allow spon-
taneous resolution.
Sclerotherapy (with the following compounds:
hypertonic saline, 25%; glucose; sodium tetra-
decyl sulfate; 5% phenol in almond oil) injected
into the submucosal plane above the dentate line
produces an inflammatory response and scar that
prevents the rectum from sliding downward. The
injection procedure sometimes requires to be
repeated.
37 Acquired Anorectal Disorders
 perative Treatment for Rectal
O
Prolapse
In children who have failed conservative ther-
apy or sclerotherapy, and in patients with full-­
thickness prolapse, one of the operative fixation
techniques as mentioned below may be needed:
(a) Circum anal suturing (Thiersch wiring)
(b) Mucosal excision and suturing (for prolapse
secondary to previous anorectal surgery)
(c) Open posterior rectopexy (fixing the rectum
with the presacral pelvic fascia)
(d) Laparoscopic rectopexy
Suggested Reading
Coran AG, Caldamone A, Scott Adzich N, Krummel
TM, Laberge JM, editors. Pediatric Surgery. 7th ed.
Oxford: Elsevier; 2012.
Holschneider AM, Hutson JM, editors. Anorectal malfor-
mations in children embryology, diagnosis, surgical
treatment, follow-up. Heidelberg: Springer; 2006.
Wexner SD, Duthie GS, editors. Constipation—etiol-
ogy, evaluation and management. 2nd ed. Heidelberg:
Springer; 2006.
 Hirschsprung Disease
Hirschsprung disease (HD) is a functional
obstruction caused by the absence of ganglion
cells in the distal colon or involving more proxi-
mal bowel.
Harold Hirschsprung presented the first
description in autopsy finding in1886. However,
the actual pathology of aganglionosis was real-
ized by Orvar Swenson in 1946.
Incidence
The incidence is 1 in 4500–7000 live births.
There is a male preponderance with male to
female ratio of 4:1. In long segment disease, the
male to female ratio becomes equal, i.e., 1:1.
Pathology
Hallmark of Histological Diagnosis
• Absence of ganglion cells in the Auerbach’s
(intermuscular) and Meissner’s (submucosal)
plexus
• Hypertrophied nerve fibers extending into the
submucosa which may be seen in hematoxylin
and eosin staining or better with acetylcholin-
esterase staining
© Springer Nature Singapore Pte Ltd. 2018
S. Roy Choudhury, Pediatric Surgery, https://doi.org/10.1007/978-981-10-6304-6_38
38
Cause
• Failure of neural crest cell migration
cranio-caudally
• Absence of neurotrophic factors or cellular
adhesion molecules
• Mutation of RET proto-oncogene (familial
variety has association with MEN 2B)
• Deficient nitric oxide synthase (NO)
Gross Pathology (Fig. 38.1b)
• Proximal ganglionic intestine dilates and
hypertrophies.
• Distal aganglionic bowel appears normal/
collapsed.
• Transition zone between ganglionic and agan-
glionic bowel appears like a funnel.
• Extent of the disease: Rectosigmoid disease is
common and seen in 80% of cases. Long seg-
ment HD (aganglionosis extending to the
descending colon and more proximal colon)
(Fig. 38.2a, b) is rare.
• Variations: Total colonic aganglionosis (TCA)
in 8% of cases, total bowel aganglionosis
(TBA), and ultrashort segment HD.
233
234
a b
Fig. 38.1 (a) Massively distended abdomen. (b) Abdominal X-ray showing distended colon with distal “cut off”
(gasless pelvis) in a patient with Hirschsprung disease
Pathophysiology
Enteric nervous system: consists of extrinsic and
intrinsic pathways.
Extrinsic system consists of:
(a) Cholinergic: with acetylcholine (ACE) as the
neurotransmitter—resulting in excitatory/
contractile state of bowel
(b) Adrenergic: with adrenaline and noradrena-
line as neurotransmitter—which are mainly
inhibitory and some excitatory
Intrinsic System
Nonadrenergic/noncholinergic: transmitted by
nitric oxide (NO) which is inhibitory resulting in
bowel relaxation.
Decrease in NO (inhibitory) and increase in cho-
linergic and adrenergic excitatory function
lead to loss of balance and a persistent con-
tractile state of the involved aganglionic seg-
ment which causes obstruction.
38 Hirschsprung Disease
Clinical Features
Newborn to infant age group presents with:
• History of delayed passage of meconium
(>48 h after birth)
• Constipation, abdominal distension
(Fig. 38.1 (a)), poor feeding, emesis, and con-
stipation followed by diarrhea due to
enterocolitis
• Per rectal examination revealing tight anus
and gripping of the finger with explosive pas-
sage of flatus and feces
Diagnosis
• A plain abdominal X-ray may show colonic
distension with distal “cut off” (Fig. 38.1(b))
• Barium enema—The diagnostic features are
narrow distal bowel (aganglionic), transition
zone, and dilated proximal bowel (gangli-
Clinical Features 235

a b

Dilated
ganglionic bowel

Cone tranzition
zone

Narrow aganglionic
bowel

Fig. 38.2 Rectosigmoid transition (a) Barium enema. (b) Appearance at surgery. (c) Diagrammatic representation
236
onic) (Fig. 38.2a). There is also significant
contrast retention in delayed films. No bowel
wash should be given prior to the study, the
contrast should be allowed to run by gravity
through a rectal catheter, thereby avoiding
overdistension, and a lateral film and delayed
films up to 24 h should be taken. The study
should terminate once the transition zone and
proximal dilated bowel are demonstrated.
The transition zone may be absent in the fol-
lowing scenarios:
–– Neonate
–– Ultrashort segment HD or total colonic
aganglionosis
–– Rectal examination or wash performed
prior to enema
a b
Fig. 38.3 (a) Barium
enema, long segment HD.
(b) Long segment HD at
surgery
38 Hirschsprung Disease
• Manometry—The role is for
–– Screening or exclusion of HD because of
inconsistent results and operator variability
–– Useful in exclusion of HD in older child
and diagnosis of ultrashort segment HD (as
transition zone is absent on barium enema
but manometry may be positive)
The presence of recto-anal inhibitory reflex
(RAIR) (normally distension of rectum is associ-
ated with relaxation of anal sphincter) excludes
HD. The absence of RAIR is also seen in internal
sphincter achalasia.
• Rectal biopsy—This is the gold standard
for the diagnosis. Biopsy may be obtained
by suction rectal biopsy, punch biopsy, or
open biopsy. Biopsy should be taken at least
Clinical Features
Table 38.1 Clinical
difference between idiopathic
Soiling (encopresis)
constipation and Hirschsprung
disease Per rectal examination
Stool in rectal ampulla
Obstructive symptoms
Enterocolitis
Stool retentive behavior
Contrast examination
Fig. 38.4 Idiopathic constipation (note dilated rectal
ampulla up to the anal verge with shelf)
1–1.5 cm above the dentate line as there is
paucity of ganglion cells up to 1 cm above
the dentate line. Three specimens are taken
between 2 and 5 cm above the dentate line.
A strip of full-thickness rectal biopsy con-
taining the muscle layers may serve as a
myectomy for the treatment for ultrashort
HD. Histological finding of agangliono-
sis (staining with hematoxylin and eosin or
237
Idiopathic constipation Hirschsprung disease
Common Unusual
Dilated ampulla Narrow (finger gripping)
Common Unusual
Rare Common
Never Possible
Common Rare
Dilated ampulla (Fig. 38.4) Narrow rectum
calretinin) with hypertrophied nerve bun-
dles confirms the diagnosis. Suction rectal
biopsy specimens which contain submucosa
require acetylcholine esterase staining for
identification of hypertrophied nerve fibers.
Intraoperative frozen section biopsy helps in
the confirmation of the diagnosis and identi-
fication of the proximal ganglionic bowel for
the purpose of pull-­through surgery.
Differential Diagnosis
• Meconium ileus, distal ileal atresia, low ano-
rectal malformation, and small left colon
syndrome.
• Other causes of delayed passage of meconium
which includes prematurity, meconium plug,
sepsis and electrolyte imbalance, hypothyroid,
intestinal neuronal dysplasia, and intestinal
pseudoobstruction.
• Functional constipation and encopresis (fecal
retention with liquid soiling) is a common dif-
ferential diagnosis (Table 38.1).
Other forms of the HD
• Late presentation even in adulthood is possi-
ble in this disease.
• Ultrashort segment disease is rare.
• Total colonic aganglionosis constitute 3–12%
of cases.
Management (Fig. 38.5)
Initial stabilization is done with gentle rectal
washout, intravenous hydration, and often intra-
venous antibiotics if enterocolitis is suspected.
238 38 Hirschsprung Disease

Treatment of enterocolitis due to HD is by fre- (b) One-stage transanal endorectal pull-through

quent rectal washouts with oral/intravenous anti-
biotics (depending upon the severity of the
condition).
Surgical Treatment
(a) Staged operation
Staged operation consists of initial siting
of a stoma at the level of ganglionic bowel
called leveling colostomy, followed by pull-­
through operation after 3–6 months. The
level of aganglionosis can be determined by
intraoperative frozen section biopsy.
Types of pull-through procedures (Fig.
38.6)
Duhamel (Martin modified) procedure:
Retrorectal pull-through
Swenson procedure: Abdominal transanal
pull-through
Soave (Boley) procedure: Endorectal
pull-through
with or without laparoscopy assistance
(Fig. 38.7a, b)
(c) Anorectal myectomy for ultrashort HD
(d) Operations for total colonic aganglionosis:
Martin modification of Duhamel operation or
Kimura modification (long colo-ileal side-to-
side anastomosis) with pull-through
The principle of Swenson operation is
based on removal of the aganglionic bowel
with end-to-­end anastomosis of the ganglionic
bowel above the anal sphincter. It involves
abdominopelvic circumferential dissection of
the rectum followed by eversion of the rectum
to complete the circumferential anastomosis
from below.
In the Duhamel pull-through operation, the
proximal ganglionic colon is pulled behind the
rectum in a retrorectal plane and anastomosed at
1 cm above the dentate line. The common wall is
then divided with a linear cutter stapler, thereby
leaving a posterior circumference of ganglionic

Fig. 38.5 Management

algorithm for Hirschsprung
disease (HD) Suspected HD, History,
Barium enema

• Obstructed • Decompressing on washes or

• Large dilated colon despite dilatation
rectal washes • Bowel diameter satisfactory
• Long segment HD for pull through

Leveling colostomy Rectal Biopsy

with histopathological One stage pull through
confirmation Frozen section confirmation

Staged Pull through

• Duhamel
• Swensen
• Soave
Suggested Reading 239

2 cm
1 cm 1 cm 1 cm

Duhamel Swenson Soave (ERPT)

Fig. 38.6 Pull-through operations for Hirschsprung disease

a b

Fig. 38.7 (a) Endorectal pull-through. (b) Endorectal completed anastomosis

and anterior circumference of aganglionic bowel.
Since the operation involves minimum pelvic
dissection, hence the results in terms of bowel
function and continence are satisfactory.
In the Soave operation, the aganglionic bowel
is removed and the proximal ganglionic colon is
pulled through a rectal muscular cuff retained
following a submucosal endorectal dissection.
The pulled-through bowel is anastomosed cir-
cumferentially above the dentate line.
The results of the three operations are
comparable.
Suggested Reading
Coran AG, Caldamone A, Scott Adzich N, Krummel
TM, Laberge JM. Pediatric surgery. 7th ed. Oxford:
Elsevier; 2012.
Holschneider AM, Hutson JM. Hirschprung’s Disease and
allied disorder. Heidelberg: Springer; 2006.
 Bleeding Per Rectum
39

The etiology and presentation of bleeding per Table 39.1 Causes of bleeding per rectum in children
rectum in children are diverse. The degree can Neonate
vary from mild to severe bleeding. The common Anal fissure
causes can be grouped according to the age of the Necrotizing enterocolitis
presentation (Table 39.1). Malrotation with volvulus
Hirschsprung disease with enterocolitis
Infant up to 3 years
 pproach to a Child with Rectal
A Anal fissure
Bleeding Intussusception
Milk protein allergy
A detailed history and physical examination Duplications of intestine
will provide important information regarding Gangrene of bowel secondary to volvulus,
the possible cause and site of bleeding. A per- intussusception, internal hernia, etc.
rectal examination should follow to identify Infectious diarrhea
any local cause like rectal polyp or anal fissure. Eosinophilic enteropathy
Staining of the examining finger gives evidence Thrombocytopenia
regarding the presence of any fresh or altered Child age above 3 years
Polyps
blood.
Meckel diverticulum
The management guidelines for massive gas-
Anal fissure
trointestinal bleeding involve:
Infectious colitis
Henoch-Schonlein purpura
• Resuscitation and stabilization of the patient’s
Hemolytic uremic syndrome
general condition
Eosinophilic enteropathy
• Identification and management of causes which
Inflammatory bowel disease
may require urgent surgical intervention
Vascular lesions

Resuscitation requires prompt insertion of

intravenous line, fluid resuscitation along with
blood and blood products as necessary in a child
with shock.
Bleeding Meckel diverticulum, vascular mal-
formations, typhoid ulcer, and duplications can
present as an emergency. Conditions that may
require urgent surgical intervention comprise pri-
marily of those that lead to bowel necrosis. These
children are sick at presentation with tense dis-
tended abdomen and features of peritonism. The

© Springer Nature Singapore Pte Ltd. 2018 241

S. Roy Choudhury, Pediatric Surgery, https://doi.org/10.1007/978-981-10-6304-6_39
242
cause of intestinal gangrene can be internal
­herniation, volvulus, band obstruction with fixed
loop, and necrotizing enterocolitis.
In India, gastrointestinal infections are the
leading cause of bleeding per rectum in children.
Hence, in a stable patient without any obvious
cause of bleeding, a course of combination anti-
bacterial and antiparasitic drug therapy often
settles the issue.
Anal fissure is the most common cause of rec-
tal bleeding in the first 2 years of life. The condi-
tion has been discussed in detail in chapter 37.
Polyps of the Colon and Rectum
The most common cause of lower gastrointestinal
bleed in school going children is rectal polyp. The
characteristic of bleeding from rectal polyp is
painless fresh blood passed in drops both before
and after defecation. Juvenile polyps are the com-
monest cause of these and account for 80% of
childhood polyps with a characteristic histological
finding of cluster of mucoid lobes surrounded by
flattened mucus-secreting glandular cells. There is
no malignant potential. These polyps are most
commonly seen in children between 3–10 years.
Majority (85%) of children have a solitary polyp.
The most common c­omplaint is rectal bleeding
and occasionally the polyps may prolapse out of
the rectum. Diagnosis is made by rectal examina-
tion and/or endoscopy. Removal by endoscopy or
by suture ligation of the stalk is the treatment of
choice. Lymphoid polyps make up about 15% of
childhood polyps. They begin to appear during the
first year of life, peak at about the third year of life,
and diminish in number by 5 years of age. These
are multiple and may present with mild chronic
blood loss. Diagnosis is made by barium enema,
endoscopy, and biopsy. No treatment is necessary
since they will regress spontaneously. Presence of
multiple rectal polyps (more than five) or rectal
polyp in a child with family history of juvenile
polyposis coli suggests the possibility of polyposis
coli. Juvenile polyposis coli with an autosomal
dominant inheritance are premalignant; hence,
close surveillance and timely removal of the
affected colon is recommended.
39 Bleeding Per Rectum
Other rare disorders which have multiple
intestinal polyps are Peutz-Jeghers syndrome
(hamartomatous polyps) and familial adenoma-
tous polyposis coli.
In Peutz-Jeghers syndrome, polyps are asso-
ciated with melanin hyperpigmentation of the
lips and oral mucosa. The polyps are usually
multiple and hamartomatous. They can involve
any part of the gastrointestinal tract, but the
majorities are limited to the jejunum and ileum.
Chronic blood loss and anemia in a child with
repeated bouts of colicky abdominal pain sec-
ondary to actual or incipient intussusception are
typical. Diagnosis is made by gastrointestinal
contrast studies or endoscopy. Gastrointestinal
malignancy has been reported in 2–3% of
patients. Females with Peutz-Jeghers syndrome
seem predisposed to develop ovarian tumors,
usually in adolescence. Similarly testicular
tumors are common in boys. Treatment depends
on the severity of symptoms and extent of
involvement. All polyps should be removed by
either endoscopy or open surgery, and close sur-
veillance including breast, pelvic, and testicular
examination is recommended.
Familial adenomatous polyposis (FAP) coli
have a genetic basis of inheritance with suscepti-
bility for malignant transformation in all cases.
Affected patients have hundreds of adenomatous
colonic polyps with virtually all patients develop-
ing adenocarcinoma of colon by the third decade
of life. In Gardner’s syndrome which has an auto-
somal dominant inheritance, the premalignant
adenomatous colonic polyps are associated with
multiple osteomas, fibromas, and epidermoid
cysts. Management involves total colectomy, rec-
tal mucosectomy, and ileoanal anastomosis pref-
erably with a J pouch.
Suggested Reading
Kliegman R, Stanton B, Geme JS, Nelson SN. Textbook
of pediatrics. 2nd ed. Saunders, Philadelphia: Elsevier;
2015.
Leilli JL Jr. Chapter 93: Polypoid diseases of the gastroin-
testinal tract. In: Coran AG, Scott Adzich N, Krummel
TM, Laberge JM, editors. Pediatric surgery. 7th ed.
Oxford: Elsevier; 2012. p. 1177–85.
 Constipation and Fecal
Incontinence
Constipation
Constipation is a common problem in childhood
with a prevalence of about 3–4%. Constipation
can be defined as delay or difficulty in passing
stools, which is present for more than 2 weeks.
The most widely accepted definition is as per
Rome III definitions in which a child with pres-
ence of two of more of the following symptoms is
considered to have constipation:
• Two or less defecations per week
• At least one episode of incontinence per week
• History of excessive stool retention or reten-
tive posturing
• History of painful or hard bowel movements
• Large fecal mass in the rectum
• History of large-diameter stools that may
obstruct the toilet
Presence of two or more of these symptoms
for more than 1 month in a child <4 years or for
more than 2 months in a child >4 years is consid-
ered diagnostic for constipation.
Causes of Constipation
A. Functional or idiopathic constipation
(commonest)
B. Organic causes
(a) Anorectal malformation
(b) Pelvic mass
© Springer Nature Singapore Pte Ltd. 2018
S. Roy Choudhury, Pediatric Surgery, https://doi.org/10.1007/978-981-10-6304-6_40
40
(c) Neurogenic bladder secondary to spinal
cord trauma or spinal cord birth defects
(d) Intestinal neural disorders
• Hirschsprung disease
• Intestinal neuronal dysplasia
• Visceral myopathies or neuropathies
(e) Metabolic or hormonal conditions
• Hypothyroidism
• Hypercalcemia
• Hypokalemia
(f) Drugs
• Opiates
• Phenobarbiturates
• Antacids
Since the most common cause of constipation
is functional, it needs to be differentiated from
other causes, which may require surgical inter-
vention or a different set of medications. The
­following signs and symptoms alert the ­physician
that the constipation is not idiopathic and needs
further investigations or referral:
• Constipation in a neonate
• Delayed passage of meconium after 48 h
• Family history of Hirschsprung disease
• Blood in stools
• Failure to thrive
• Bilious vomiting
• Marked abdominal distension
• Anal abnormality (abnormal location, stenosis)
• Findings suggestive of neurogenic cause
–– Absent anal or cremasteric reflex
243
244
–– Decreased lower limb power/tone/reflex
–– Spina bifida (hair/swelling in lumbosacral
spine)
• Delayed milestones (suggestive of hypothy-
roidism/developmental causes)
Management of Constipation
Management of functional constipation
• Normal fiber intake (increase roughage in
diet)
• Normal fluid intake
• Normal physical activity
• Proper toilet training (child should be made to
sit on the pot after a meal and with proper
posture)
The treatment of constipation is in three phases
• Fecal disimpaction: If a large fecal mass is
obstruction of the rectum, it needs to be
removed by polyethylene glycol (PEG). In
case PEG is unavailable, rectal enemas or
even manual fecal disimpaction may be neces-
sary. Rectal glycerin suppository at regular
interval is very helpful.
• Maintenance therapy: PEG at the dose of
0.4–0.8 mg/kg/day is preferred; otherwise,
lactulose at the dose of 1–2 mL/kg/day can
also be given. The maintenance therapy is
continued for at least 2 months provided that
the child is free from constipation for at least
1 month.
• Tapering off: The medications are gradually
tapered.
Causes of Fecal Incontinence
in Children
1. Anorectal malformations
2. Hirschsprung’s disease
3. Spina bifida
40 Constipation and Fecal Incontinence
4. Pelvic and sacrococcygeal tumors
5. Trauma
 rue Fecal Incontinence Versus
T
Fecal Pseudoincontinence
(Encopresis)
True fecal incontinence patients are those who
lack the ability of voluntary bowel movements.
Fecal pseudoincontinence or encopresis is gener-
ally due to fecal impaction with overflow or spill-
age, and once their bowel is evacuated and
constipation treated, they may be able to have
voluntary bowel movement.
It is important to differentiate those patients
with use of contrast enema:
A. Tendency toward constipation and dilated
colon from those with
B. Tendency toward diarrhea with nondilated
or narrow colon or narrow colon
Bowel management program for patients with
tendency toward constipation and dilated colon
includes:
(a) Dietary modification: Avoid constipating
diet, more high-fiber diet (fruits, vegetables,
juices, cereals, pulses, honey) that act as
laxatives.
(b) Laxatives.
(c) Large-volume enemas.
Bowel management program for patients with
tendency toward diarrhea with nondilated or
narrow colon includes:
(a) Constipating diet (avoidance of diarrhea-­
promoting food)
(b) Small-volume enemas
(c) Oral drugs: Loperamide, Imodium, and pec-
tin (slowing the bowel)
Bowel management program is more success-
ful in constipated patients than in those with diar-
rhea and short colon. The process of bowel
Suggested Reading 245

a b c

Fig. 40.1 (a) Home enema application with can. (b) Patient education for enema therapy. (c) Antegrade continence
enema (ACE) through an appendix stoma

is empty. A patient should remain clean for 24 h

management in patients with fecal incontinence
with an effective treatment. Home administration
is one of trial and error. With the management
of enema prepared with saline (or salt and water)
protocol, most of the patients have success and
and soap and applied through simple enema can
can live reasonably normally with a good quality
is cheap and effective (Fig. 40.1a, b). As the child
of life.
grows older especially in patients with spina
bifida, rectal irrigations may not be possible as it
often needs assistance particularly in wheelchair-
Enema therapy
bound patients. Therefore, an antegrade conti-
nence enema (ACE) through an abdominal stoma
The idea of enema therapy is to empty the colon
(appendix or cecostomy, Fig. 40.1c) may be
so as to keep the patient clean for 24 h. The type,
more practical than rectal enema administration.
dose, frequency, and duration of enemas will
Constipation with megasigmoid and megarectum
vary from patient to patient. The common com-
responds to sigmoid resection.
mercially available enema is phosphate enema.
A more effective therapy can be achieved by
adding glycerine (5–30 mL) and soap 10–15 g
to normal saline 500–1000 mL (or salt added to Suggested Reading
water). Volume of enema can vary from small
Holschneider A.M., Hutson J.M. (eds.). Anorectal mal-
(<500 mL) to large (>500 mL) depending on age
formations in children embryology, diagnosis, surgical
and weight of the patient. The whole process of treatment, follow-up. 2006 Springer Heidelberg.
enema administration and subsequent empty- Kliegman R, Stanton B, Geme JS, Nelson SN. Textbook
ing of the colon can be completed in less than of pediatrics. 20th ed. Saunders, Philadelphia:
Elsevier; 2015.
1 h preferably given in the morning. The enema
Wexner SD, Duthie GS, editors. Constipation—tiology,
should be received for about 15 minutes, hold for evaluation and management. 2nd ed. Heidelberg:
10 min, and then sit on the toilet till the bowel Springer; 2006.
 Stoma Care
Stomas or ostomies are artificially created opening
of a hollow organ on the body surface. They can be
temporary or permanent. It is important to plan for
sitting the stomas in areas without interfering with
the normal activities like wearing clothes.
Stomas in Pediatric Patients
The commonly performed stomas in children are
(Fig. 41.1):
(a) Esophagostomy for saliva drainage
(Fig. 41.2a, b)
(b) Gastrostomy for feeding (tube) (Figs. 41.3
and 41.4)
(c) Jejunostomy for feeding (tube)
(d) Ileostomy for fecal diversion (Fig. 41.5)
(e) Colostomy for fecal diversion (Figs. 41.6,
41.7 and 41.8)
(f) Vesicostomy for urinary diversion (Fig. 41.9)
(g) Ureterostomy for urinary diversion
Stoma Related Complications
Colostomy and ileostomy are frequently per-
formed in children for fecal diversion. The stoma
effluent may vary from liquid to semisolid to
solid depending on the level and duration. The
further distal the stoma in the bowel, the more
solid the output is. The common stoma-related
© Springer Nature Singapore Pte Ltd. 2018
S. Roy Choudhury, Pediatric Surgery, https://doi.org/10.1007/978-981-10-6304-6_41
41
problems are bleeding from the mucocutaneous
junction leading to anemia, skin excoriation, pro-
lapse, retraction, stenosis, and loose motion with
dehydration (Figs. 41.10, 41.11, 41.12, 41.13,
and 41.14). High effluent output causes more
water and electrolyte loss from the body, associ-
ated with peristomal skin excoriation. Proximal
stomas with liquid output are more difficult to
manage than distal stomas with solid bowel out-
put. Several types of stoma collection bags are
available for collection of the effluent (Fig. 41.15).
They can be simply tied around the abdomen or
fixed with Stomahesive paste and powder.
Management of Stomas
Simple application of cotton cloth with frequent
changing and gentle cleaning of the site appear to
be effective in majority of our patients as they are
generally small, and their parents handle it very
effectively. It is necessary that the cotton should
not be kept directly on the stoma as it will become
adherent to it and may cause bleeding while
removal. To prevent trauma it is necessary to keep
a paraffin gauge or a cotton cloth dipped in oil
over the stoma and then cover it with a pad or
other cloth. The surrounding skin can be pro-
tected with application of coconut oil or any
emollient cream and a paste of zinc oxide with
calamine (Siloderm) or a Stomahesive paste
(karaya gum, adept). Weak steroid (betametha-
sone) and antifungal (clotrimazole) creams are
247
248 41 Stoma Care

Fig. 41.1 Showing different sites of stomas in

pediatric patients
Esophagostomy

Gastrostomy

Jejunostomy

Transverse
colostomy

Sigmoid
colostomy

Ureterostomy

Vesicostomy
Management of Stomas 249

a b

Fig. 41.2 (a) Esophagostomy and gastrostomy (tube). (b) Esophagostomy

a b

Fig. 41.3 Well-­managed gastrostomy tube for feeding

250 41 Stoma Care

Fig. 41.4 Gastrostomy feeding in newborn

Fig. 41.6 Divided sigmoid colostomy (double stoma)

Fig. 41.5 Ileostomy (end stoma)

Fig. 41.7 Sigmoid end colostomy; note stoma site skin

excoriation
Management of Stomas 251

Fig. 41.10 Colostomy prolapse (pouch colon)

Fig. 41.8 Transverse loop colostomy

Fig. 41.11 Colostomy prolapse (distal stoma), a com-

mon complication

Fig. 41.12 Divided stoma (ileostomy and mucous fis-

Fig. 41.9 Vesicostomy tula), with skin excoriation
252
Fig. 41.13 Perianal excoriation following ileal pull-through
Fig. 41.14 Prolapsed vesicostomy
effective in managing troublesome peristomal
skin excoriation. While cleaning the stoma, a
nonabrasive moist cloth should be used to mini-
mize the damage to the stoma. It is necessary to
keep the peristomal area dry by frequent cleaning
and application of powder/paste. Excessive stoma
output with dehydration needs medical attention
with adequate fluid and electrolyte replacement.
41 Stoma Care
Fig. 41.15 Simple stoma collection bag
Minor prolapse can be managed by simple reduc-
tion; however, stoma retraction or stenosis may
require surgical revision.
Gastrostomy Management
An ideal device that can be used for creating a
gastrostomy is a gastrostomy button. Because of
the prohibitive cost of the button and limited
availability, Malecot catheter or Foley catheter is
effectively used in resource-poor centers. The
common problem faced by gastrostomy is
enlargement of the stoma with pericatheter leak-
age of feeds. This can be prevented by the use of
Malecot catheter rather than Foley catheter. The
triangular shape of the bulb of Malecot (rather
than the spherical shape in Foley) prevents
enlargement of the site. Also a nipple can be
placed over the stoma and the catheter passed
through it. This nipple limits the movement of the
catheter and enlargement of the stoma. Side holes
are made in the nipple, allowing air to pass
through and keep the area dry.
Suggested Reading
Breckman B. Stoma care and rehabilitation. Oxford:
Elsevier Churchill Livingstone; 2005.
 Part 6
Pediatric Oncology
 Wilms’ Tumor
Wilms’ Tumor (WT)
Wilms’ tumor is the commonest renal malig-
nancy of childhood and the second most common
non-central nervous system solid tumor after
neuroblastoma (Table 42.1).
Epidemiology
The incidence of pediatric renal tumors is about
8 per million in children less than 15 years of age.
Wilms’ tumor accounts for 91% of pediatric renal
malignancies. The mean age of presentation is
3 years. The incidence of bilateral WT is 5–10%.
A family history is found in 1–2% of the cases.
Etiopathogenesis
Wilms’ tumor results from of series of step which
are still not well understood. Nephrogenic rests
are cluster of primitive metanephric cells derived
for renal stem cells which are sometimes present
in normal kidneys in intralobular or perilobular
location. These rests are more often seen in
patients of unilateral Wilms’ tumor (30–40%)
and in all patients with bilateral Wilms’ tumor.
Therefore, these rests are considered as precursor
to Wilms’ tumor.
A large number of foci of genetic abnormalities
are identified in children with Wilms’ tumor which
may predispose these patients to Wilms’ tumor.
© Springer Nature Singapore Pte Ltd. 2018
S. Roy Choudhury, Pediatric Surgery, https://doi.org/10.1007/978-981-10-6304-6_42
42
Table 42.1 Pathologic classification of pediatric renal
tumors
Wilms’ tumor
• Favorable histology Wilms’ tumor
• Unfavorable histology Wilms’ tumor
 – Diffuse anaplasia
 – Focal anaplasia
Congenital mesoblastic nephroma
Cystic partially differentiated nephroblastoma
Clear cell sarcoma
Malignant rhabdoid tumor of the kidney
Renal cell sarcoma
Renal adenocarcinoma
Renal neurogenic tumors
Renal teratoma
Molecular genetics. Heterozygosity of Wilms’
tumor (WT) gene and several chromosomal loci
have been mapped. WT-1 (11p13) is a tumor sup-
pressor gene and is deleted in children with
WAGR and mutated in children with Denys-­
Drash syndrome.
There is loss of heterozygosity (LOH) of
WT-2 gene (11p15) in association with familial
form of Beckwith-Wiedemann syndrome (BWS)
with specific loss of maternal allele.
The National Wilms’ Tumor Study (NWTS)-5
has reported that (LOH) at the 16q and 1p loci is
associated with a poorer prognosis with high
chances of relapse. The p53 tumor suppressor
gene has been found in 75% of patients with ana-
plastic histology.
255
256 42 Wilms’ Tumor

Clinical Presentation (a) Blastemal: Seen as round blue cells

(b) Stromal
The commonest presentation is with an asymptom- (c) Epithelial elements (aberrant glomeruli and
atic abdominal mass (Fig. 42.1a). Associated fea- tubules).
tures could be microscopic or gross hematuria, Favorable histology Wilms’ tumor: Triphasic
malaise, weight loss, anemia, fever, hypertension, or monophasic
intraperitoneal rupture of tumor with abdominal cri- Unfavorable histology: Anaplastic (focal or
sis, tumor thrombus involving IVC with dilated diffuse)
abdominal veins, varicocele, and cardiac thrombus. Clear cell sarcoma (bone-metastasizing tumor)
Association. Hypospadias, undescended tes- and rhabdoid tumor (brain-metastasizing tumor)
tis, aniridia, isolated hemihypertrophy, Denys-­ are no more considered as Wilms’ tumor and clas-
Drash syndrome (male pseudohermaphroditism, sified as a separate entity.
Wilms’ tumor, end-stage renal disease), Beck-
with-Wiedemann syndrome (macroglossia, vis-
ceromegaly, hypoglycemia, umbilical defects), Investigations
and WAGR (Wilms’ tumor, aniridia, genitoruri-
nary, mental retardation) syndrome. Imaging is performed with: chest X-ray, abdomi-
nal ultrasonography with Doppler, and contrast-
enhanced CT scan of the abdomen and chest
Pathology (Figs. 42.2a, b and 42.3).
Features to look for on imaging:
Gross appearance (Fig. 42.1b) of the tumor is
grayish white; the cut surface is fleshy with cystic • Nature of mass
areas, necrosis, and hemorrhage. • Organ of origin and its extension
Microscopy shows heterogenous tissue, aber- • Presence of functioning contralateral renal tissue
rant glomeruli, and bone, cartilage, muscle, and • Presence of any bilateral disease
adipose tissue. • Presence of IVC/renal vein thrombus
Presence of three elements (Triphasic) is • Presence of distance metastasis
hallmark of the histopathology of Wilms’ tumor:

a b

Fig. 42.1 (a) Left

Wilms’ tumor. (b) Gross
appearance of
nephroureterectomy
specimen of Wilms’
tumor
Staging 257

Fig. 42.2 (a) CT scan

a b
showing left Wilms’
tumor. (b) CT scan
showing neuroblastoma

Fig. 42.3 CT showing

pulmonary metastasis

Differential diagnosis. Neuroblastoma (Table Staging

42.2), congenital mesoblastic nephroma, cystic
nephroma, cystic partially differentiated nephro- Staging system utilized by the Children’s
blastoma, cystic Wilms’ tumor, teratoid WT, and Oncology Group (COG):
renal cell carcinoma. I – Tumor limited to the kidney and com-
pletely excised without rupture
or biopsy. Surface of the renal capsule is intact.
Table 42.2 Comparison of presentation of Wilms’ tumor
II – Tumor extends through the renal capsule
with neuroblastoma
or has invaded the renal sinus, but is completely
Neuroblastoma Wilms’ tumor
removed with no microscopic involvement of the
Younger age group Slightly older age group:
margins.
(<2 years of age) Peak 3–4 years of age
III – Residual tumor is confined to the abdo-
Calcification very Calcification uncommon:
common: 90% 10–15% men and not from hematogenous spread. Also
Encases vascular Displaces adjacent included in Stage III are cases with tumor
structures but does not structures without involvement of the abdominal lymph nodes, rup-
invade them insinuating between them ture of the tumor, transected tumor thrombus,
Elevates the aorta away Extension into IVC/renal peritoneal implants, and microscopic or grossly
from the vertebral vein
column
positive resection margins. Inoperable tumors are
Poorly marginated, Well-circumscribed also included in Stage III.
surface nodular IV – Hematogenous metastases at any site
Crossing midline Uncommon, except very (lung, liver, bone, brain) or lymph node metasta-
large tumors ses beyond the abdomen.
Kidney displaced Claw sign with the kidney V – Bilateral renal involvement at the time of
involved diagnosis.
258
Management
Multimodal management of Wilms’ tumor is the
standard of care with use of surgical methods,
radiotherapy, and chemotherapy.
There are two basic protocols followed inter-
nationally:
• The Children’s Oncology Group (COG) rec-
ommends abdominal exploration in children
with unilateral Wilms’ tumor for staging and
excision/biopsy. The treatment is then based
on histopathology and staging.
• The International Society of Pediatric Oncology
(SIOP) recommends preoperative chemother-
apy in these children without any preoperative
histopathological/cytological diagnosis.
For children presenting with a large or unre-
sectable tumor, a slightly modified COG protocol
is followed. Cytological diagnosis in these chil-
dren is achieved by fine needle aspiration cytol-
ogy (FNAC), and preoperative chemotherapy is
given. Patients with smaller tumors are managed
as per COG protocol.
Surgical Treatment
Goals of surgery in Wilms’ tumor:
• Complete en bloc removal of specimen:
Nephroureterectomy for unilateral Wilms’
tumor. Tumorectomy/partial nephrectomy for
bilateral tumors or tumors in solitary kidney.
• Avoid rupture/spillage of the tumor.
• Adequate lymph node sampling.
• Staging of the tumor.
• Marking of the renal bed to aid in subsequent
radiotherapy.
The Essential Steps of the Operation
The abdomen is opened through a large supraumbili-
cal transverse incision, and assessment is done for the
extent of the tumor. With the presence of CECT
showing a normal contralateral kidney, routine explo-
ration of contralateral kidney is not recommended.
42 Wilms’ Tumor
The attention is now diverted toward the
tumor. The colon is lifted medially off the Gerota
fascia. It is preferable to take vascular control of
the renal vessels before mobilizing the kidney to
prevent vascular dissemination of the tumor. Due
to large tumor size, this is often not possible.
Tumor should be handled gently to avoid spill
especially if the tumor has areas of necrosis. The
renal vein, IVC, and the ureter should be exam-
ined for the presence of tumor extension. The
ureter should be ligated and divided as low as
possible. The tumor should be removed en bloc
without transection of the tumor.
Adrenal glands can be spared if not involved;
however for upper polar lesion, they may need
to be removed. Lymph node sampling should be
performed, and all lymph nodes visible or pal-
pable should be removed from iliac, para-aor-
tic, and celiac areas. Lastly the tumor bed is
marked with clips to aid in future radiotherapy.
 umor with Vena Caval/Atrial
T
Thrombus
Tumor growth into the suprahepatic vena cava
and atrium requires cardiopulmonary bypass for
surgical removal. Extension above the level of
the hepatic veins or into the right atrium can be
managed with preoperative chemotherapy to
facilitate shrinkage of the intravascular throm-
bus, thereby obviating the need for cardiopulmo-
nary bypass for removal of the tumor thrombus.
If the tumor can be localized and controlled
below the atrium, resection without the use of
cardiopulmonary bypass is possible.
Bilateral Wilms’ Tumor
(Figs. 42.4 and 42.5)
Children with bilateral tumors are generally
younger than those with unilateral lesions with a
mean age of 25 versus 44 months. Preservation of
renal parenchyma is a critical issue for these chil-
dren. The COG does not recommend biopsy for
bilateral tumors. These patients are started neo-
adjuvant chemotherapy for 6 weeks and are then
Chemotherapy
reassessed, so as to increase the chances of renal
salvage. In patients with polar tumors with one
third or less renal parenchyma involved, partial
nephrectomy may be carried out. Tumorectomy
with some margin can be performed for small
hilar tumors.
Rhabdoid tumors have remained the most
resistant to cure of all pediatric renal tumors.
NWTS-5 used an intensive therapy with carbo-
platinum, etoposide, and cyclophosphamide.
Unfortunately, no improvement in survival
occurred.
Chemotherapy
Pre-op chemotherapy (CT)
Preoperative treatment of Wilms’ tumor is gener-
ally accepted in certain circumstances like occur-
Fig. 42.4 CT bilateral Wilms’ tumor
a b
Fig. 42.5 (a) Above
case managed with (a)
right nephrectomy, (b)
left partial nephrectomy
259
rence of Wilms’ tumor in a solitary kidney,
unresectable tumors, bilateral renal tumors,
tumor in a horseshoe kidney, intravascular exten-
sion of the tumor above the intrahepatic vena
cava, and respiratory distress from extensive met-
astatic tumor. Pretreatment biopsy/cytology
should be obtained. Reevaluation for operability
is to be done after 5 weeks with a fresh CT scan.
Reevaluation for resectability in bilateral Wilms’
tumor is done at 6 and 12 weeks if needed.
COG Chemotherapy Protocol
Treatment of favorable histology (FH) tumors:
• Very low-risk tumors: <2 years of age, <550 g
tumor weight, Stage I, any loss of heterozy-
gosity (LOH) status
• Low risk: Stage I or II, no LOH at 1p and 16q
• Standard risk: Stage I or II with LOH or
Stages III and IV without LOH at 1p and 16q
• High risk: Stages III and IV with LOH at 1p
and 16q
Very low-risk tumors: Surgery only. They do
not require chemotherapy and can be kept on
observation.
Stages I and II, low risk: Surgery plus chemo-
therapy with EE4A regimen, no radiotherapy.
Stages I and II, standard risk: Surgery plus
chemotherapy DD4A regimen, no radiotherapy.
Stage III, standard risk: Surgery plus chemo-
therapy DD4A with abdominal/flank radiotherapy.
Stage III, high risk: Surgery plus chemother-
apy DD4A switched to regimen M after 6 weeks
with abdominal/flank radiotherapy.
260 42 Wilms’ Tumor

Stage IV, complete response of metastatic lesion Regimen of postoperative therapy as per SIOP protocol
by 6 weeks: DD4A regimen without chest radio- Stage II + and 28 weeks DAM/
therapy (abdominal irradiation as per local stage). III VCR/EPI + RT
tumor bed
Stage IV, incomplete response of metastatic
High grade 34 weeks EPI/IF/
lesion by 6 weeks: DD4A for initial 6 weeks fol-
VP16/
lowed by regimen M and chest radiotherapy CARBO + RT
(abdominal irradiation as per local stage). Metastatic IV As per the local
Treatment of Unfavorable Histology (UH) stage for tumor +
Tumors (Anaplastic) treatment of
metastases—RT
and/or excision
All patients should receive flank irradiation:
DAM dactinomycin, VCR vincristine, EPI epirubicin, IF
• Stage I: focal or diffuse are treated with DD4A. ifosfamide, VP16 etoposide, CARBO carboplatin, RT
• Stage II and III focal: DD4A. radiotherapy
• Stage II and III diffuse: UH-1 regimen.
• Stage IV focal or diffuse: UH-1 regimen (if
poor response then UH-2) and lung irradiation.
Prognosis and Outcome
Chemotherapy regimen:
• EE4A: Vincristine and dactinomycin for The prognostic factors are stage, tumor histol-
18 weeks. ogy, and LOH at 1p and 16q. In Stage I favorable
• DD4A: Vincristine, dactinomycin, and adria- histology, age and tumor weight are also impor-
mycin for 24 weeks. tant. With the importance of LOH-positive
• M: Vincristine, dactinomycin, and adriamycin patient being recognized as a poor prognostic
with additional cyclophosphamide and etoposide. factor, the Children’s Oncology Group has
• UH-1: Vincristine, adriamycin, cyclophospha- started with more intensive chemotherapy for
mide, carboplatin, and etoposide. these patients.
• UH-2: UH-1 with additional vincristine and The current 5-year survival in Stage I and
irinotecan. Stage II is >90%, and Stage III and Stage IV is
close to 73%.
Management of
WT pre-op as per
SIOP protocol
Clinical staging Congenital Mesoblastic Nephroma
Localized 4 weeks of Surgical staging
DAM/VCR This is a common renal tumor in infants (below
Metastatic 6 weeks of (Histological 1 year). It is the commonest renal tumor in neo-
DAM/VCR/ diagnosis) nates and infants. It presents with an abdominal
EPI
mass in newborn and infants. It has distinct his-
DAM dactinomycin, VCR vincristine, EPI epirubicin
tological appearances and different from Wilms’
Regimen of postoperative therapy as per SIOP protocol tumor. The classic variant is characterized by
Stage Treatment uniform spindle-shaped cells arranged in bun-
Localized Stage I, low None dles with scattered foci of entrapped normal
grade glomeruli and tubules. The cellular variant pres-
Stage I, 18 weeks DAM/ ents late and has a tendency to infiltrate and local
intermediate VCR recurrence. Complete surgical excision is cura-
grade +
anaplasia tive and radiotherapy or chemotherapy is not
Stage II—(no 28 weeks DAM/ effective.
lymph nodes) VCR/EPI
Suggested Reading 261

Suggested Reading Coran AG, Caldamone A, Scott Adzich N, Krummel TM,

Laberge JM, editors. Pediatric surgery. 7th ed. Oxford:
Elsevier; 2012.
Carachi R, Grosfeld JL, Azmy AF. The surgery of
Poplak DG, Pizzo PA. Principles and practice of pediatric
childhood tumours. 2nd ed. Heidelberg: Springer;
oncology. 7th ed. Philadelphia: Wolters Kluwer; 2016.
2008.
 Neuroblastoma
Neuroblastoma is the most common extracranial
solid tumor in infants and children, accounting
for 6–10% of all childhood cancers. The overall
incidence is 1 per 10,000 children. The incidence
is highest in the first year of life. Neuroblastomas
arise from primordial neural crest cells, and occur
in the adrenal medulla or anywhere along the
sympathetic ganglia, most notably in the retro-
peritoneum and posterior mediastinum.
Pathology
Neuroblastomas are made up of immature neu-
roblasts; they are small uniform cells with dense,
hyperchromatic nuclei and scant cytoplasm.
Differentiated cells have a more mature ganglion
cell appearance with well-defined nucleoli and
eosinophilic cytoplasm. Characteristic features
of neuroblastoma include presence of neuropil
and arrangement in rosettes. Immunohisto­
chemistry with specific marker (neuron-specific
enolase, chromogranin) can help in diagnosis
of doubtful cases. The Shimada classification,
modified later as the International Neuroblastoma
Pathology Classification, is based on the bio-
logic behavior and prognosis of tumors. Neuro­
blastoma typically represents poorly differentiated
tumor, whereas ganglioneuroma is its well-
differentiated, benign counterpart. Ganglioneuro­
blastoma represents both, having features of
immature, poorly differentiated neuroblasts and
matured ganglion cells. Based upon the type of
© Springer Nature Singapore Pte Ltd. 2018
S. Roy Choudhury, Pediatric Surgery, https://doi.org/10.1007/978-981-10-6304-6_43
43
tumor, degree of differentiation, age, and per-
centage of mitotic/karyorrhectic cells, the tumor
is classified as favorable or unfavorable histo­
vlogy.
Prognostic Factors (Table 43.1)
Favorable prognostic indicators are:
(a) Age less than 18 months.
(b) Clinical stages 1, 2, and 4S.
(c) N-myc non-amplification.
(d) Differentiation, low mitosis-karyorrhexis
index (defined as fewer than 100 mitotic or
karyorrhectic cells per 5000 cells), and
stroma-rich tumors.
(e) The presence of hyperdiploid DNA content
is associated with early tumor stage and
improved prognosis.
(f) The high expression level of tyrosine kinase
receptor for nerve growth factor (NGF) has
also shown strong predictability for favor-
able outcome.
(g) Higher CD44 expression correlates with less
aggressive tumor behavior and improved
survival.
Unfavorable prognostic factors are:
(a) Age more than 18 months.
(b) 1p36 loss of heterozygosity (LOH) and
unbalanced 11q LOH.
(c) Tumors with diploid DNA content are found
in approximately two thirds of a­ dvanced-­stage
263
264 43 Neuroblastoma

Table 43.1 Current prognostic factors for neuroblastoma

Favorable Poor
Clinical
Age <18 months >18 months
Site Non-adrenal Adrenal
Thoracic, pelvic
Stage І, ІІ, ІVS ІІІ, ІV
Histological Differentiation Poorly differentiated
Low mitosis-karyorrhexis index (defined as fewer than High MKI
100 mitotic or karyorrhectic cells per 5000 cells) Stroma poor
Stroma-rich tumors
Biological
N-myc Non-amplification Amplification (>10 copies)
DNA content Hyperdiploid Diploid, tetraploid
1p Del – Present
LOH 1p36 – Present
TrkA Present –
TrkB – Present
NGF Present –
CD44 Elevated Lack
Ferritin – Elevated
NS enolase – Elevated
LDH – Elevated (>1500 U/ml)
17q gain – Present
MDR-1 – Present
P-Glycoprotein – Elevated
NM-23 – Present
Bcl-2 – Present
Caspase Elevated –

neuroblastomas and are often resistant to bone metastasis and an advanced

chemotherapeutic options. Biochemical
markers of importance include neuron-­
specific enolase (NSE), ferritin, and lactate
dehydrogenase.
(d) High levels of NSE tend to have advanced-­
stage neuroblastomas and poor survival rates.
(e) High ferritin level is frequently seen in
patients who have advanced-stage disease
and correlates with worse overall outcome.
(f) Increased levels of serum lactate dehydroge-
nase (>1500 U/mL) are associated with rapid
cellular turnover and poor prognosis.
(g) Low expression of tyrosine kinase receptor is
associated with N-myc amplification and
advanced-stage neuroblastomas.
(h) Abdominal mass, thoracic mass, compres-
sion effects, and bone pain with dramatic
recent change in activity level may indicate
neuroblastoma.
Clinical Presentation
The presentation can be in the following ways:
• Due to large primary tumour—Cervical, tho-
racic, abdominal, or pelvic mass. Cervical masses
are ­apparent at an early stage. Most common site
is the adrenal (approx. 50% cases, Fig. 43.1).
• Due to local extension or compression by the
primary tumor (Fig. 43.2):
–– Cervical: Horner syndrome due to involve-
ment or origin for stellate ganglion
–– Intra spinal extension: Motor and sensory
deficits
–– Compression of bladder/rectum by pelvic
tumor: Difficulty in passing urine and stool
Clinical Presentation 265

• Due to metastases: –– Pancytopenia—Involvement of bone

–– Skin nodules especially in Stage 4S (blue-
berry muffin-like spots)
–– Proptosis/periorbital ecchymosis (raccoon
eyes, Fig. 43.3)
marrow
–– Hepatomegaly—Liver metastases
–– Bone pain—Bone metastases
• Paraneoplastic syndrome:
–– Secretory diarrhea: Caused by secretion of
vasoactive intestinal peptide (VIP) by
tumor cells
–– Opsoclonus myoclonus syndrome “danc-
ing eyes, dancing feet”: Caused by pres-
ence of anti-neuronal antibody

Fig. 43.2 Dilated veins due to IVC obstruction from

Fig. 43.1 CT scan showing left adrenal neuroblastoma abdominal neuroblastoma

a b

Fig. 43.3 “Raccoon eye” neuroblastoma ((a) pre- and (b) post-chemotherapy)
266
Screening
Screening is done by 24-h urinary VMA (vanil-
lylmandelic acid) and HVA (homovanillic acid)
levels. Neonatal screening which was performed
in Japan, Germany, etc. has been stopped because
of overdiagnosis of neuroblastoma and unneces-
sary treatment.
Diagnosis
Radiological investigations:
1. X-ray, ultrasound, contrast CT (Fig. 43.4),
and MRI
2. Nuclear medicine investigation
Radiolabeled metaiodobenzylguanidine • N-myc: Gene is present on chromosome 2 and
(MIBG) scan is highly specific and sensitive for
evaluating bone and bone marrow disease. FDG-­
PET (positron emission tomography) scan can
help in localization of metastasis or viable tumor
after therapy. Role of bone scan when MIBG is Table 43.2 International Neuroblastoma Staging System
being performed is controversial. It is still Stage Definition
included in the workup of a patient with 1
neuroblastoma.
Fig. 43.4 CT scan, abdominal neuroblastoma; note
tumor crossing the midline, encasing and lifting the aorta
with fine calcifications
43 Neuroblastoma
Histopathological investigation:
–– FNAC/biopsy of the primary or secondary
(for diagnosis) and genetic testing (for
prognostication)
–– Bone marrow aspiration and biopsy from
bilateral posterior iliac spines
Other tests:
Twenty-four-hour urinary HVA (homovanillic
acid) and VMA (vanillylmandelic acid)
Staging (Table 43.2)
Prognosis/Risk stratification
• Age.
• Stage.
• Histology.
its amplification (>10 copies) is associated
with poor prognosis.
Localized tumor with complete gross excision,
with or without microscopic residual disease;
negative ipsilateral lymph nodes
2A Localized tumor with incomplete gross
excision; negative ipsilateral nonadherent
lymph nodes
2B Localized tumor with or without complete
gross excision; positive ipsilateral nonadherent
lymph nodes; negative contralateral lymph
nodes
3 Unresectable unilateral tumor infiltrating
across the midline, with or without regional
lymph node involvement, or localized
unilateral tumor with contralateral regional
lymph node involvement, or midline tumor
with bilateral extension by infiltration
(unresectable) or by lymph node involvement
4 Any primary tumor with dissemination to
distant lymph nodes, bone, bone marrow, liver,
skin, or other organs (except as defined for
Stage 4S)
4S Localized primary tumor (as defined for Stage
I, IIA, or IIB) with dissemination limited to
skin, liver, and bone marrow (limited to
infants <1 year old)
Suggested Reading
• Diploidy: Near-diploid and near-tetraploid
tumors have worse prognosis as compared to
near triploid tumors.
• Allelic deletions on chromosomes 1p and 11q
associated with poor prognosis.
Principles of Treatment
Based on these prognostic factors, the tumor is
classified as low-, intermediate-, and high-risk
group (Table 43.3).
A combined modality of surgery, chemother-
apy, and radiotherapy based on disease stage and
risk group is used for neuroblastoma.
Low-Risk Tumors
Surgery alone is sufficient. Chemotherapy is not
needed. Stage IVS may require no treatment at
all if it is low risk.
Intermediate-Risk Tumors
Chemotherapy regimen comprising cyclophos-
phamide, doxorubicin, carboplatin, and etopo-
side is given.
Radiotherapy is reserved for progressive dis-
ease and unresectable primary tumor.
Surgery: The most complete tumor resection
is performed while preserving full organ and neu-
rological function even if it necessitates leaving
residual disease over vital structures.
High-Risk Tumors
They require intensive induction chemotherapy,
myeloablative consolidation, and chemotherapy
267
Table 43.3 International Neuroblastoma Risk Group
Staging System
Stage Description
L1 Localized tumor not involving vital structures
as defined by the list of image-defined risk
factors and confined to one body compartment
L2 Locoregional tumor with presence of one or
more image-defined risk factors
M Distant metastatic disease (except stage MS)
MS Metastatic disease in children younger than
18 months with metastases confined to skin,
liver, and/or bone marrow
In summary, for low-risk patients, surgical excision of the
tumor is usually curative and avoids the risks associated
with chemotherapy. Intermediate risk patients are usually
treated with surgery and standard chemotherapy. The poor
prognosis in high-risk patients justifies a much more
intense treatment regimen, including combination chemo-
therapy followed by complete surgical excision (if possi-
ble), radiation therapy to achieve local control, or even
myeloablative treatments with bone marrow rescue.
with stem cell rescue. 13-cis-retinoic acid is admin-
istered for promoting differentiation of tumor cells.
Targeted treatment for minimal residual disease
like MIBG therapy, targeted immunotherapy are
also done.
Surgical resection of residual tumor should be
done if possible.
Recently a pre-treatment risk group staging
has been proposed with risk-based pretreatment
classification. The detail of this classification is
beyond the scope of this review.
Suggested Reading
Coran AG, Caldamone A, Scott Adzich N, Krummel TM,
Laberge JM, editors. Pediatric surgery. 7th ed. Oxford:
Elsevier; 2012.
Poplak DG, Pizzo PA. Principles and practice of pediatric
oncology. 7th edition, Philadelphia: Wolters Kluwer;
2016.
 Hepatoblastoma and Other
Liver Masses in Children
Liver masses in the pediatric age group could be
due to various benign and malignant conditions
(Table 44.1). Hepatoblastoma is the commonest
primary malignant liver tumor in infants and
children.
In newborns the commonest liver mass is
infantile hepatic hemangioma. Hepatoblastoma
is seen between 4 months and 4 years of age,
whereas hepatocellular carcinoma is seen in older
children. Among the benign tumors, mesenchy-
mal hamartoma and focal nodular hyperplasia are
seen in young infants and children.
Hepatoblastoma
The reported incidence from the United States is
1.2 per million populations. There is male pre-
ponderance, with M:F, 1.7:1. There are associa-
tions with Beckwith-Wiedemann syndrome,
prematurity, Gardner syndrome, and familial pol-
yposis coli. Hepatoblastoma affects right lobe in
50%, left lobe in 15%, and central location in
27% cases.
Pathology
Hepatoblastoma is a tumor with divergent patterns
of differentiation which range from fetal hepato-
cytes to embryonal cells with ­differentiated tissues
© Springer Nature Singapore Pte Ltd. 2018
S. Roy Choudhury, Pediatric Surgery, https://doi.org/10.1007/978-981-10-6304-6_44
44
like muscles, connective tissue, and bone.
Depending upon the presence of these different
cell types, hepatoblastoma is classified as follows:
Types
Pure fetal
Embryonal/mixed fetal and embryonal
Macrotrabecular
Small cell undifferentiated
Mixed epithelial and mesenchymal pattern
With teratoid features
Without teratoid features
Pure fetal type has the best prognosis, whereas
the small cell undifferentiated type has the worst
prognosis.
Presentation
The usual mode of presentation is an asymptom-
atic abdominal mass in the right upper quadrant
(Fig. 44.1) in infants. Nonspecific symptoms
include low-grade fever, anorexia, and failure to
thrive. Large tumor may rarely result in portal
hypertension due to obstruction of portal vein,
ascites due to obstruction of portal vein and
hepatic veins, and obstructive jaundice due to
compression of bile duct.
269
270 44 Hepatoblastoma and Other Liver Masses in Children

Table 44.1 Liver masses in children

Benign tumors
Mesenchymal hamartoma
Infantile hemangioma
Focal nodular hyperplasia
Hepatic adenoma
Teratoma
Malignant tumors Nontumor masses
Hepatoblastoma Parasitic: Hydatid disease
Hepatocellular carcinoma Abscess: Bacterial/amoebic
Sarcomas Cystic lesions
Kaposiform hemangioendothelioma Vascular malformations
Metastatic tumors

borns. AFP is also a useful prognostic tool as

hepatoblastomas with AFP <100 are considered
to be undifferentiated with poor prognosis.
Platelet count is often raised in hepatoblas-
toma due to secretion of thrombopoietin by tumor
cells. Thrombocytosis may help in supporting the
diagnosis of hepatoblastoma in doubtful cases.

Imaging Studies

• Ultrasonography (USG) is the first imaging

modality of choice to identify the site of origin
of the mass.
• Contrast-enhanced computed tomography
(CECT) scan (Fig. 44.2a, b) or MRI of the
abdomen helps in determining the extent of
tumor involvement. Triphasic CT scan can
demonstrate the vascular anatomy of the liver
in relation to the tumor. Evaluation of the
chest by CT is also needed as the lung is the
most common site for metastases.
• Most hepatoblastomas are positive on FDG-­
Fig. 44.1 Abdominal mass due to right lobe PET scan; therefore, PET scan may be useful
hepatoblastoma to assess extent of disease and response to
chemotherapy.
Diagnosis

Biochemical Marker Histopathology/Cytology

Alpha-fetoprotein (AFP) is raised in most cases Fine needle aspiration cytology/percutaneous

of hepatoblastoma. AFP level is a useful marker
for diagnosis and for monitoring treatment as it
comes to normal level in 4–6 weeks after com-
plete resection of the tumor. One should also
remember that neonates have an elevated alpha-­
fetoprotein level at birth which comes down to
adult level at 6–8 months. Normal values of AFP
may range from 40,000 ng/mL in a term newborn
to more than 100,000 ng/mL in premature new-
needle biopsy is necessary to obtain tissue diag-
nosis in children planned for upfront chemother-
apy. But due to inherent complications of
percutaneous biopsy, some groups have recom-
mended that children between 6 months and
3 years of age with serum AFP more than 1000 ng/
mL and more than three times the normal for age
can be presumed to have ­hepatoblastoma, and
biopsy can be avoided for these patients.
Infantile Hepatic Hemangioma 271

a b

Fig. 44.2 (a) CT scan, right lobe hepatoblastoma. (b) CT scan, left lobe hepatoblastoma

Staging strategy is variable among various groups. A bal-

anced approach may be that PRETEXT I and II
Management is currently based on the PRETEXT tumors without E, V, P, and M can be considered
staging (pretreatment extent of disease) as deter- for primary resection. PRETEXT III and IV or
mined by the International Society of Pediatric presence of E, V, P, and M need neoadjuvant che-
Oncology Liver Tumor Group (Fig. 44.3). motherapy. Complete surgical resection provides
PRETEXT 1, with three adjoining sectors free the best chance for cure (Figs. 44.4 and 44.5). For
(tumor in only one sector) large and advanced tumors, chemotherapy with
PRETEXT 2, with two adjoining sectors free reduction of tumor size followed by surgical
(two sectors involved) excision provides the best form of therapy.
PRETEXT 3, in which one sector or two non-­ Several chemotherapeutic agents are used, most
adjoining sectors are free (tumor involves two or commonly, cisplatin, adriamycin, vincristine, and
three sectors) 5-fluorouracil. The most commonly used chemo-
PRETEXT 4, in which no sector is free (tumor therapy is cisplatin alone or in combination with
in all four sectors) adriamycin (PLADO regime, cisplatin 25 mg/m2/
Extension or involvement by the tumor is day IV infusion for 3 days, and adriamycin 20 mg/
highlighted by one or more of the following: m2/day IV infusion for 3 days at thrice weekly inter-
val) depending upon whether the tumor is classified
• V: Inferior vena cava/all three hepatic veins as standard risk or high risk by its characteristics.
• M: Metastases Those patients with unresectable or multi-
• P: Porta focal hepatoblastoma who do not respond to
• E: Extrahepatic spread chemotherapy and selected cases of PRETEXT
• F: Multifocal IV tumors can be considered for upfront liver
• C: Caudate lobe transplantation.
Hepatocellular carcinoma, undifferentiated
Advanced hepatoblastoma as determined by embryonal sarcoma, and rhabdomyosarcoma of
PRETEXT Stage III or above with involvement the biliary tree are other rare malignant condi-
of more than two sectors of the liver poses a sig- tions affecting children.
nificant challenge for surgical resection.

Management Infantile Hepatic Hemangioma

Treatment of hepatoblastoma involves combina- Infantile hepatic hemangioma (IHH) is the most
tion of chemotherapy and surgery. The treatment common benign solid liver tumor in children
272
Fig. 44.3 Staging of
hepatoblastoma
I II
III
Fig. 44.4 Large right lobe hepatoblastoma
at surgery
seen in the first year of life. They can be small
focal lesion or large diffuse lesion. In a triad of
hepatomegaly, congestive heart failure and
­anemia, and cutaneous hemangiomas has been
found in 80% of the cases. Thrombocytopenia,
consumptive coagulopathy (Kasabach-Merritt
syndrome), and congenital hypothyroidism are
other features of this lesion. Various syndromes
such as Osler-­Weber-­Rendu, Klippel-Trenaunay-
Weber, Ehlers-Danlos, Beckwith-Wiedemann,
diaphragmatic hernia, trisomy 21, transposition
of the great arteries, and extranumerary digits
44 Hepatoblastoma and Other Liver Masses in Children
SIOPEL Pre-T-Ext Staging
IV
have been ­ associated with infantile hepatic
hemangioendotheliomas. Computed tomography
(CT), with intravenous contrast, classically
shows diffusely enhancing lesion (Fig. 44.6).
MRI with intravenous gadolinium improves the
accuracy of diagnosis.
Management
These lesions tend to grow in the first year of life
and then begin to regress spontaneously.
Infantile Hepatic Hemangioma 273

are often successful. Localized lesions can be

resected with good outcome. Any residual lesion
after successful conservative treatment should be
resected as malignant transformation to angiosar-
coma is a possibility.

Mesenchymal Hamartoma of Liver

This is the third most common solid tumor and

the second most common benign tumor of the
liver. It presents with an abdominal mass, respi-
ratory distress, and congestive cardiac failure.
Antenatal diagnosis by ultrasonography is

Fig. 44.5 Resected specimen of hepatoblastoma (right

hepatectomy)

Fig. 44.6 CT scan showing hemangioendothelioma of

liver; note diffuse vascular lesion with peripheral
enhancement

Asymptomatic lesions should be observed.

Patients with features of congestive cardiac fail-
ure, coagulopathy, or respiratory distress need
intervention. Digitalis and diuretics are used for
heart failure. Steroids in the form of prednisolone
2–3 mg/kg/day and vincristine have been suc-
cessfully used. Interferon alpha has fallen in dis-
repute due to complication like spastic diplegia.
Propranolol is a new addition to drugs effective Fig. 44.7 (a, b) CT scan, mesenchymal hamartoma of
in this disease. Embolizations for large lesions liver, one large cyst, and multiple small lesions
274 44 Hepatoblastoma and Other Liver Masses in Children

Fig. 44.8 Excision and marsupialization for a massive

mesenchymal hamartoma of the liver

sometimes possible. Pathologically these are

large well-circumscribed lesion with cystic and
solid areas mainly involving the right lobe of the
liver. CT, USG, and MRI are all helpful in the
diagnosis (Fig. 44.7a, b), and serum alpha-feto-
protein level in often raised. Complete surgical b
excision is the treatment of choice as transfor-
mation to an undifferentiated embryonal sar- Fig. 44.9 (a) CT scan, giant liver cyst in newborn. (b)
Liver cyst at surgery
coma is a possibility. For large lesions temporary
marsupialization (Fig. 44.8) may be done fol-
lowed by close follow-up and complete surgical Congenital Giant Liver Cyst
excision in the future.
(Fig. 44.9a, b) is a rarity in children. It can be
managed by with simple cyst excision.
Focal Nodular Hyperplasia

This tumor occurs relatively late with a mean age
of 7 years and female preponderance. Association
with oral contraceptives has been documented in
adults. Pediatric focal nodular hyperplasia often
shows regression; hence, an expectant treatment
may be offered. Symptomatic cases and those
with persistent lesions and doubtful diagnosis are
better removed surgically.
Suggested Reading
Carachi R, Grosfeld JL, Azmy AF. The surgery of
childhood tumours. 2nd ed. Heidelberg: Springer;
2008.
Coran AG, Caldamone A, Scott Adzich N, Krummel TM,
Laberge JM, (eds). Pediatric surgery. 7th ed. Oxford:
Elsevier; 2012.
Poplak DG, Pizzo PA. Principles and practice of Pediatric
Oncology. 7th ed. Philadelphia: Wolters Kluwer;
2016.
 Germ Cell Tumors
45

Germ cell tumors (GCT) can be gonadal or

extragonadal in origin. These lesions are pre-
sumed to originate from the primordial germ
cell. Overall incidence of GCT is about 1 in
100,000 children less than 15 years of age. The
following are the features of germ cell tumors
in children:

• Extragonadal site predominates compared

with gonadal locations and accounts for about
60% cases (Figs. 45.1 and 45.2). Of the
extragonadal sites, sacrococcygeal site is the Fig. 45.1 Testicular germ cell tumor; note inguinal
commonest, the other sites being the head and orchidectomy
neck, brain, mediastinum, and retroperito-
neum.
• Ovarian germ tumors account for 30%, and
testicular germ cell tumors account for the
remaining 10% of the cases.
• Overall 80% of GCT are benign, and the
remaining 20% are malignant.
• The most common benign germ cell tumors
are teratomas.
• The most common malignant histology is
yolk sac tumor, which has alpha-fetoprotein
(AFP) as a sensitive marker.

Pathology

The pathological types are dysgerminoma (or

seminoma), yolk sac (endodermal sinus) tumor,
embryonal carcinoma, polyembryoma, chorio- Fig. 45.2 Bilateral ovarian germ cell tumor (left larger
carcinoma, teratoma, and mixed GCT. In order of than right)

© Springer Nature Singapore Pte Ltd. 2018 275

S. Roy Choudhury, Pediatric Surgery, https://doi.org/10.1007/978-981-10-6304-6_45
276
a b
Fig. 45.3 (a) CT scan showing intra-abdominal mass, mature teratoma, (b) surgically removed teratoma
Fig. 45.4 Large pelvic teratoma in a newborn removed
surgically
frequency, the most common are teratoma, endo-
dermal sinus tumor (yolk sac tumor), germinoma,
and mixed GCT.
Choriocarcinoma, embryonal carcinoma, and
polyembryomas are rarely seen. Most childhood
germ cell tumors are benign, comprising of
mature and immature teratomas (Figs. 45.3a, b
and 45.4). Yolk sac tumor (also called endoder-
mal sinus tumor) is the most common malignant
45 Germ Cell Tumors
histologic variant in infancy and childhood.
Immature teratomas contain primitive neuroepi-
thelium, and based on that, they are histologically
graded between I and III (Norris).
The most important predictor of tumor recur-
rence in pediatric immature teratomas is the pres-
ence of microscopic foci of yolk sac tumor.
Because of their size, they may be missed by the
pathologic sampling process.
Clinical Presentation
Ovarian tumors – Presentation is usually with a
painless mass in the lower abdomen. Approximately
10% of these patients may present with features of
torsion of the ovary.
Testicular tumors – Usually present with
painless mass in the scrotum. Occasionally
pain and inflammatory signs may be present,
and differentiation from benign conditions like
torsion testis, epididymo-orchitis, or hema-
toma becomes necessary.
Sacrococcygeal teratoma (SCT) – Is the most
common neoplasm of the fetus and newborn with
an incidence of 1 in 20,000–40,000 live births
and has a female predominance. Most SCTs are
histologically benign; however, approximately
one fifth exhibit malignant features.
Investigations
a b
Fig. 45.5 (a, b) Sacrococcygeal teratoma
A mass is the usual presentation (Fig. 45.5a,
b). Antenatal diagnoses are also frequently
made with the increasing use of antenatal ultra-
sound scan. The tumor is present in the relation
of the coccyx and usually protrudes externally
and may extend across the pelvis into the
abdomen.
Altman classification has been described
based on its local extent.
Type I lesion has only external component.
Type II lesion has primarily external compo-
nent but also has pelvic extension.
Type III lesion has little external compo-
nent and has significant abdomino-pelvic
extension.
Type IV lesion does not have external
component.
Risk of malignancy is highest in type IV
lesions, probably because they present late.
SCT in newborn is predominantly benign.
Older children and intrapelvic presentation are
associated with higher rate of malignancy.
Investigations
Radiological examinations are done to look for
local extent and for metastases. In infants of less
277
than 2 months of age with sacrococcygeal tera-
toma, metastatic workup can be avoided, as
chances of malignancy are very less. For evalua-
tion of local extent of the disease, a CECT is pre-
ferred, the only exception being testicular tumor in
which ultrasonography of the scrotum is sufficient.
Teratomas often show calcification, which is seen
as chunky radiopaque shadows in a plain
radiograph.
Metastatic workup is done by CECT of the
thorax and the abdomen to detect retroperitoneal
lymph node metastases or lung metastases.
Biochemical markers: Serum alpha-­
fetoprotein (AFP) and β human chorionic
gonadotropin (βhCG) are markers for malig-
nant germ cell tumors. AFP is raised in yolk sac
tumors, which are the most common malignant
germ cell tumors in children. The presence of
raised levels of these biomarkers after five half-
lives even in the absence of any radiological
evidence denotes the presence of residual
malignant disease. However, it must be remem-
bered that AFP is normally elevated in neonates
and comes to adult values at approximately
8 months of age. Choriocarcinoma, although
less common, has human chorionic gonadotro-
pin (hCG) as an easily identifiable serum
marker.
278
Staging
Stage I – Complete resection at any site, coccy-
gectomy for sacrococcygeal site, negative tumor
margins
Stage II – Microscopic residual, lymph nodes
negative
Stage III – Lymph node involvement with
metastatic disease. Gross residual tumor or
biopsy only; retroperitoneal nodes negative or
positive
Stage IV – Distant metastases, including the
liver and lung (Figs. 45.6 and 45.7a, b).
Fig. 45.6 Chest X-ray showing metastatic germ cell
tumor
a b
Fig. 45.7 (a, b) CT scan chest, metastatic germ cell tumor
45 Germ Cell Tumors
Treatment
Excision of the tumor is the primary treatment.
However, germ cell tumors are extremely chemo-
sensitive tumors and respond very well to cisplatin-
based chemotherapy like PEB (cisplatin, etoposide,
and bleomycin). Therefore, extensive surgery
which may affect organ function or cause signifi-
cant deformity is discouraged, and tumor reduction
prior to surgery by administrating chemotherapy
(neoadjuvant chemotherapy) is preferred. Complete
surgical excision alone is sufficient as treatment.
This is true for even immature teratoma in children
regardless of histological grade. However, for
immature teratoma grade III, some advocate addi-
tional chemotherapy due to the high risk of recur-
rence and malignant transformation.
Surgery
• Sacrococcygeal teratoma. Coccygectomy
should be performed along with excision of
the sacrococcygeal teratoma.
• Testicular tumor is removed through an ingui-
nal incision (Fig. 45.1). The cord is taken
under control before manipulation of the scro-
tal tumor. High inguinal orchidectomy is per-
formed by ligating the cord as high as possible
near the deep inguinal ring. Scrotal incision
should be strictly avoided to eliminate any
Suggested Reading 279

chance of lymphatic spread of the tumor to the Table 45.1 Management guidelines for germ cell tumors
groin lymph nodes. Low risk Surgery and observation
• Ovarian tumors. For proper staging of the Stage I gonadal
tumor, the following steps are taken: All immature teratomas
–– Contralateral ovary is always inspected, Intermediate risk Surgery and PEB
Stage II–IV testes ×3 cycles
and any suspicious areas are biopsied. Stage II–III ovary
–– Ascitic fluid if present is sent for cytology; Stage I–II extragonadal
otherwise, peritoneal wash fluid is submit- High risk Surgery and PEB
ted for cytology. Stage IV ovary ×4 cycles
–– Examine peritoneal surface and the liver Stage III–IV extragonadal
and excise suspicious lesions. PEB = cisplatin, etoposide, bleomycin
–– The ovary is removed without violating the
tumor capsule.
–– Examine omentum and remove if adherent
or involved.
–– Inspection of retroperitoneal lymph nodes
and biopsy of enlarged nodes.
–– The fallopian tube is removed only if
adherent to the tumor.
–– Purely cystic lesion can be considered for
ovarian-preserving cystectomy without
spilling the cyst content.

Chemotherapy

Stage I gonadal germ cell tumors and immature

teratomas at any site do not require postoperative
chemotherapy. All other tumors depending upon
their site and stage of the tumor need 3–4 cycles
of chemotherapy (Table 45.1). Fig. 45.8 Fetus in fetu

Fetus in Fetu
This is characterized by organized musculoskel-
etal, vertebral, and organ structures resembling a
fetus (Fig. 45.8). Currently, it is considered as a
mature teratoma. The other theory suggests it is
a parasitic twin fetus resulting from a monozy-
gotic twin pregnancy. Complete surgical removal
is the treatment.
Suggested Reading
Carachi R, Grosfeld JL, Azmy AF. The surgery of child-
hood tumours. 2nd ed. Heidelberg: Springer; 2008.
Coran AG, Caldamone A, Scott Adzich N, Krummel TM,
Laberge JM, editors. Pediatric surgery. 7th ed. Oxford:
Elsevier; 2012.
Poplak DG, Pizzo PA. Principles and practice of Pediatric
Oncology 7th edition. Philadelphia: Wolters Kluwer;
2016.
 Soft Tissue Sarcomas
Sarcomas are malignant tumors of mesenchymal
cell origin. They are named after the normal tis-
sue they resemble, e.g., rhabdomyosarcoma from
skeletal muscle, leiomyosarcoma from smooth
muscle, fibrosarcoma and malignant fibrous his-
tiocytoma from connective tissue, liposarcoma
from fatty tissue, angiosarcoma from blood ves-
sels, synovial sarcoma from synovial tissue, and
neurofibrosarcoma from nerve tissue. Other rare
soft tissue sarcomas include extraosseous
Ewing’s tumor, peripheral neuroectodermal
tumors, epithelioid sarcoma, hemangiopericy-
toma, and alveolar soft part sarcoma. Soft tissue
sarcomas comprise of a great variety of tumors.
These tumors are broadly classified as rhabdo-
myosarcoma (RMS) and non-­rhabdomyosarcoma
soft tissue sarcomas (NRSTS). NRSTS com-
prises of a heterogenous group of tumors like
synovial sarcoma, fibrosarcoma, and desmoplas-
tic round cell tumor. RMS is the most common
sarcoma in children and is discussed in detail
here.
Rhabdomyosarcoma
RMS accounts for 4.5% of all cases of childhood
tumors. They are the third most common cause of
extracranial solid tumors of childhood after neu-
roblastoma and Wilms’ tumor. RMS is a malig-
nant tumor of mesenchymal origin, included in
the group of small blue round cell tumors of
© Springer Nature Singapore Pte Ltd. 2018
S. Roy Choudhury, Pediatric Surgery, https://doi.org/10.1007/978-981-10-6304-6_46
46
childhood along with neuroblastoma, non-Hodg-
kin’s lymphoma, soft tissue Ewing’s sarcoma,
and primitive neuroectodermal tumors (PNET).
The children with RMS usually present at
young age (2–6 years) or at adolescence (10–
15 years). RMS in younger children is often of
embryonal variety that involves the trunk,
whereas RMS in adolescents is usually of alveo-
lar variety that involves the extremities.
Genetics
Specific genetic aberrations can be seen in most
cases with RMS.
• Embryonal RMS: Loss of heterozygosity in
11p15 locus in 80% of tumors
• Alveolar RMS: Translocation involving chromo-
some 13 and chromosome 1 or 2 in 75% of cases
 istological Variants of Childhood
H
RMS
Embryonal RMS: The cells may be differenti-
ated with eosinophilic cytoplasm or may have
appearance of small round cell tumor. In alveo-
lar RMS, as the name implies, some of the cells
are outlined by fibrous septa, whereas the cells in
the center are loosely clumped giving the appear-
ance of the alveolus. Botryiod sarcoma is a
281
282
Fig. 46.1 Rhabdomyosarcoma of leg
variety of embryonal RMS commonly involving
the genito-urinary system of young children. The
name botryoid came
from its similarity to “bunch of grapes”. In diffi-
cult cases, immunohistochemistry with actin,
desmin, myogenin, and/or MyoD1 may help in
making a definitive diagnosis.
1. Favorable prognosis RMS: Embryonal (botry-
oid, spindle cell variety).
2. Poor prognosis RMS: Alveolar (including
solid variant) and undifferentiated type.
Embryonal variety is commonest (50%), fol-
lowed by alveolar type (20%). Botryoid variety
occurs in 5% of cases.
Sites of Origin
1. Head and neck—35%
2. Genitourinary—26%
3. Extremities—20% (Fig. 46.1)
4. Metastatic—15%
5. Others—4%
The head and neck (except parameningeal
location), orbit, and genitourinary site of origin
46 Soft Tissue Sarcomas
(except bladder and prostate) are considered
favorable sites for RMS. Other sites are consid-
ered unfavorable with poor prognosis.
Diagnosis
Diagnosis is by direct open biopsy or FNAC or
excisional biopsy depending upon the location
and the size of the lesion.
Workup for local extent of involvement: MRI
is useful for lesions involving the limb, pelvis, or
the paraspinal location; for other sites, CECT
may be sufficient.
Metastatic workup: Local lymph node status
needs to be assessed clinically and radiologically.
In children more than 10 years of age with
extremity RMS or paratesticular RMS, the
regional lymph nodes should be examined patho-
logically as only clinical or radiological evalua-
tion gives high false-negative results.
• Bone marrow aspiration and biopsy from
bilateral posterior superior iliac spine.
• CECT chest for lung metastases.
• Skeletal survey and bone scan for bony
metastases.
Rhabdomyosarcoma 283

• Recently, FDG-PET scan is increasingly being regime is still considered as first-line therapy for
used to determine the extent of the disease. most RMS except in extremely low-­ risk cases
where two drug therapies (VA) are sufficient.

Staging • Vincristine: 1.5 mg/m2 iv weekly.

• Actinomycin D: 1350mcg/m2 iv single dose.
Intergroup RMS Study group staging system: • Cyclophosphamide: 2.2 g/m2 iv single dose
Group 1: Localized disease, completely resected over 1 hr with mesna at least 2 h prehydration
Group 2: Microscopic residual, completely and 6 h posthydration.
resected with nodes, nodes involved with micro- • Other chemotherapeutic regimens for advanced-
scopic residual stage RMS have incorporated doxorubicin and
Group 3: Incomplete resection the topoisomerase inhibitor, irinotecan.
• Group 4: Distant metastasis
Radiotherapy (RT)
• Candidates for RT primarily include those
Treatment of RMS with group 2 (microscopic residual) or group
3 (gross residual) disease.
Surgery • In group 2 disease, low-dose radiation (40 Gy
Primary goal of surgical intervention for RMS is at 1.5–1.8 Gy/fraction) is associated with local
wide and complete resection of the primary tumor control rates of at least 90%.
tumor with a surrounding rim of normal tissue. A • For patients with group 3 disease, radiation
circumferential margin of 0.5 cm is considered doses are more commonly 50 Gy.
adequate. • Radiotherapy is usually planned 9–12 weeks
PRE (pretreatment re-excision) is a wide re-­ after initiating chemotherapy.
excision of the previous operative site with ade-
quate margins of normal tissue performed prior Second-Look Operation
to the initiation of adjuvant therapy within It is performed after partial chemotherapy to
6 weeks of primary surgery. PRE is most com- either achieve better locoregional control by exci-
monly performed on extremity and trunk lesions. sion of the residual lesion or to confirm complete
Lymph node sampling: Regional lymph node response to chemotherapy. It is often useful to
disease (N-1) is present in 23% of all RMS patients, excise the lesion as soon as it becomes ­amenable
predominantly in primary tumor sites, such as the to resection provided that no significant func-
perineum, retroperitoneum, extremity, bladder/ tional compromise is done to the organ of origin.
prostate, parameningeal, and paratesticular. Lymph
node sampling is mandatory in extremity RMS and • During, or after completing, adjuvant thera-
paratesticular RMS >10 years of age. If lymph pies, patients with RMS are reimaged with CT
nodes beyond the regional lymph nodes are or MRI. If residual tumor remains, or if the
involved, then they are considered distant metasta- outcome of therapy remains in doubt, a second-­
ses and are classified in group/stage 4 disease. look operation may be considered.
Positive lymph node status is an indepen- • Most effective in extremity and truncal lesions.
dently poor prognostic factor for both failure-free
survival and overall survival. Prognostic Factors
The most important factors are the following:
Chemotherapy
Standard therapeutic regimens consist of a combi- • Site of origin.
nation of vincristine, actinomycin D, and cyclo- • The clinical group (takes into account the
phosphamide (VAC). Despite multiple trials using lymph node status and the completeness of the
newer agents for treatment of high-risk RMS, VAC excision, distant metastases).
284 46 Soft Tissue Sarcomas

• Pretreatment staging (TNM)—it takes into
account the size of tumor, site, distant metas-
tases, and lymph node status.
• Histology.
Based on this, the patients can be classi-
fied as:
High risk: All stage 4 tumors, overall 5-year
survival <50%
Intermediate risk: All alveolar tumors, embry-
onal RMS group 3 at unfavorable site, overall
5-year survival 80%
Low risk: All other tumors, overall 5-year sur-
vival >90%
Favorable prognostic factors include:
removal is the mainstay of treatment. For unre-
sectable tumor or where complete resection is not
feasible without causing serious loss of tissue or
function, neoadjuvant radiation and chemother-
apy must be added. Poor prognostic factors are
tumor size >5 cm, microscopic positive margins,
high-grade histology tumors, and intra-­abdominal
primary site. Metastatic disease may be present
in 15% of cases, the usual sites being the lung,
bone, liver, and rarely regional lymph nodes. The
best results are obtained in cases with successful
wide surgical resection (2 cm margin) with
tumor-free margins.

(a) Embryonal/botryoid histology

(b) Primary tumor sites in the orbit and non-
parameningeal head/neck region and geni-
tourinary excluding the bladder/prostate
regions
(c) A lack of distant metastases at diagnosis
(d) Complete gross removal of tumor at the time
of diagnosis
(e) Tumor size less than or equal to 5 cm
(f) Age less than 10 years at the time of diagno-
sis and time to relapse

 rinciples of Management of Non-­

P
rhabdomyomatous Soft Tissue
Sarcomas (Figs. 46.2 and 46.3)
Adequate tissue must be obtained by excisional
or incisional biopsy (Tru-Cut needle biopsy may
be a suitable alternative). The biopsy site should
be included in the subsequent wide local exci-
sion. Multimodality treatment offers the best
chance of successful outcome. Complete surgical Fig. 46.2 Non-rhabdomyomatous sarcoma over the back
Suggested Reading 285

Fig. 46.3 Ewing

sarcoma involving the
gluteal region

Coran AG, Caldamone A, Scott Adzich N, Krummel TM,

Suggested Reading Laberge JM, editors. Pediatric surgery. 7th ed. Oxford:
Elsevier; 2012.
Carachi R, Grosfeld JL, Azmy AF. The surgery of child- Poplak DG, Pizzo PA. Principles and practice of pediatric
hood tumours. 2nd ed. Heidelberg: Springer; 2008. oncology: Wolters Kluwer; 2016.
 Part 7
Pediatric Urology
 Hydronephrosis
47

Hydronephrosis is defined as aseptic dilatation Obstructive pathology in the bladder or urethra

of the pelvicalyceal system of the kidney due to usually results in bilateral hydroureteronephrosis.
partial or complete obstruction to the outflow of
urine. Vesicoureteric reflux can also cause • Bladder pathology
hydronephrosis. –– Neurogenic bladder
If it gets secondarily infected, then it is called –– Voiding dysfunction
pyonephrosis. –– Bladder diverticula
• Urethral pathology (usually males)
–– Posterior urethral valve (males only)
Causes of hydronephrosis (Fig. 47.1) –– Anterior urethral valve
–– Polyp
• Physiological hydronephrosis –– Stricture
• Pelviureteric junction (PUJ) obstruction –– Meatal stenosis
–– Intramural—stone, polyp –– Phimosis
–– Mural—congenital (commonest cause in
children)
–– Extramural—vessel, retrocaval ureter Pathology of Hydronephrosis

Associated with hydroureter (hydroureterone- Kidneys with extrarenal pelvis – obstruction

phrosis) leads to initial pelvic dilatation later followed by
pressure atrophy of the renal parenchyma (gener-
• Ureteral pathology ally late damage of renal parenchyma).
–– Stricture Kidneys with intrarenal pelvis – obstruction
–– Stone leads to more parenchymal atrophy, resulting in a
–– Polyp thin-walled hydronephrotic sac (early damage of
–– External compression—pelvic tumors renal parenchyma).
–– Ectopic ureter Significant obstruction leads to tubular dam-
• Ureterovesical junction pathology age, glomerular damage, inflammation, and
–– Vesicoureteric reflux fibrosis. Several chemical mediators of inflam-
–– Ureterovesical junction obstruction mation have been identified like interleukin-5
–– Ureterocoele and eotaxin-2. Activation of renin-angiotensin

© Springer Nature Singapore Pte Ltd. 2018 289

S. Roy Choudhury, Pediatric Surgery, https://doi.org/10.1007/978-981-10-6304-6_47
290 47 Hydronephrosis

Fig. 47.1 Usual sites of obstruction in

children

Pelviureteric
junction

Ureterovesical
junction

Posterior urethra

system appears to play a significant role in Pathology (see above)

obstruction. The expressions of growth factor
TGFβ upregulation have been demonstrated in Most cases are due to the presence of an ady-
obstructed kidneys. namic segment at the PUJ which results in func-
tional obstruction.

Pelviureteric Junction Obstruction Presentation

Incidence is 1 in 1250 live births. Male/female
ratio is 2:1, and 10–20% cases are bilateral.
Incidence of antenatal detection of hydronephrosis
is around 0.5 to 1% of all pregnancies. Most of
these are physiological or transient hydronephro-
sis which resolves by birth or in the early postnatal
period. More than 90% of antenatally diagnosed
hydronephrosis resolves over a period of time.
• Antenatally detected HDN: Ultrasono­
graphic fetal renal pelvic antero-posterior
(AP) d­ iameter > 4mm in the second trimester
or > 7mm in the third trimester. Significant
obstruction is suspected when pelvic AP
diameter is >10mm with parenchymal thin-
ning. Unilateral hydronephrosis is commoner
than bilateral cases.
• Insidious onset, dull aching pain, palpable
lump.
• Attack of acute renal colic.
Imaging
• Acute colic followed by lump and then pas-
sage of large volume of urine – disappearance
of pain and swelling—Dietl’s crisis
Imaging
• USG (anatomy) – The features to look for are
pelvic dilatation (AP diameter), calyceal cup-
ping, clubbing, narrow PUJ, thickness of
renal parenchyma, status of the ureter (nor-
mal or dilated), and any associated pathology.
Differential diagnosis is with multicystic dys-
plastic kidney (MCKD). In PUJ obstruction,
the dilated pelvis is medial in location and the
pelvis communicates with the dilated calyces.
MCKD is characterized by a small dysplastic
kidney with multiple non communicating
cysts and often an atretic ureter.
• Intravenous pyelography (IVP) (anat-
omy + function) – Poor function, delayed
nephrogram, delayed uptake and excretion,
and pelvicalyceal dilatation. IVP is now rarely
performedfor the diagnosis of PUJ obstruction
in children.
• Gd-MRU (gadolinium-enhanced magnetic res-
onance urography) – Provides information on
anatomy and function. Advantage over CT scan
Time
Activity
Normal
Lasix Injection
Fig. 47.2 Renogram
patterns Dilated nonobstructed
291
is less radiation, and disadvantage is need for
anesthesia. This appears to be the future inves-
tigation of choice for the upper urinary tract.
Dynamic Isotope Renography (Figs. 47.2,
47.3, and 47.4)
• This is currently the investigation of choice
along with USG for evaluation of hydrone-
phrosis. Commonly used isotopes for renal
function and drainage are Tc 99-labeled DTPA,
MAG3, and LLEC for scan. These isotopes are
filtered by the glomeruli and not absorbed by
tubules, and since they are Tc labeled, thus
they are traced by gamma camera.
• Diuretic renogram can differentiated between an
obstructed from a non obstructed system
(Fig. 47.2). After injection of the radioisotope
and a diuretic, a T 1/2 of <10 min is normal,
where as a T 1/2 of >20 min is obstruction and T
1/2 between 10–20 minutes is an equivocal result.
• Adequate hydration should be ensured. Tc
DTPA scan can be applied to calculate GFR
from clearance of DTPA (modified Gates
method).
• CT scan is rarely necessary for the diagnosis
of hydronephrosis (Fig. 47.5); high radiation
exposure is a disadvantage.
Obstructed
Equivocal
292 47 Hydronephrosis

Left Kidney
kidney a b
Right 800
RK
kidney 700
Left
background Counts/second 600

Right 500
background 400
300 LK
200
100
0
0 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21

Fig. 47.3 (a) Renogram curve showing obstructed pattern in the right kidney. (b) Renogram showing right pelviure-
teric junction obstruction

Fig. 47.4 (a, b) a

Renogram showing Composite frame Curve unit time Right kidney
obstructed left kidney bkgd. corrected
in minutes
(pelviureteric junction) Left kidney
bkgd. corrected
30.9 k
27.8 k
24.7 k
21.6 k
Counts

18.5 k
15.4 k
9.3 k
6.2 k
3.1 k
0.0 k
0 4 7 10 13 16 19 22 25 27 32
b
Pre void Post void

• Whitaker test—Infusion of fluid via nephros- • Retrograde pyelography (RGP)—

tomy with measurement of pelvic pressure Occasionally required for doubtful cases.
(rarely performed).
Obstructive Uropathy in Children
Fig. 47.5 CT scan showing giant right hydronephrosis
• Micturating cystourethrogram (MCUG) is
recommended for bilateral hydronephrosis to
rule out reflux or lower tract pathology.
Obstructive Uropathy in Children
Antenatally Diagnosed
Hydronephrosis
Incidence is 0.25–1%. The survival rate for unilat-
eral hydronephrosis is 100%, and only 20–25%
will require an operation by 4 years of age. Other
hydronephroses due to transient pelvic dilatation
will resolve over time. Similarly, only 5% of bilat-
eral cases require an intervention, and spontane-
ous resolution is expected in the majority of cases.
Society of pediatric urology (SFU) hydrone-
phrosis grading system (Table 47.1).
Management
Asymptomatic children with preserved renal func-
tion with stable hydronephrosis can be observed
and monitored with serial USG and renal scintigra-
phy. The indications for surgery are as follows:
Indications for surgery
• Increasing hydronephrosis on follow-up ultra-
sonography (increase in renal pelvic AP diam-
eter 20 mm and above).
• Parenchymal thinning.
• Obstructive pattern on diuretic renogram
(Figs. 47.3 and 47.4).
• Decreasing renal function (<40% differential
renal function on diuretic renogram).
293
Table 47.1 SFU hydronephrosis grading system
SFU Pattern of renal sinus splitting (IVP, USG
grade appearance)
0 No splitting
1 Slight splitting of the central renal complex
without calyceal involvement, normal
parenchyma
2 Splitting of the central renal complex with
extension to nondilated calyces
3 Wide splitting of the renal pelvis, dilated
outside the renal border, calyces uniformly
dilated, normal parenchyma
4 Large, dilated calyces (may appear convex);
thinning of the parenchyma to 50% of the
ipsilateral (normal) kidney
• Palpable lump.
• Recurrent UTI.
• Pain.
• In solitary kidney with PUJ obstruction or
bilateral PUJO, surgery can be considered
(although few clinicians have managed con-
servatively by monitoring GFR).
Renal conservation is the aim. If the differen-
tial function is >10% in isotope renogram, one
should try to preserve the kidney. If differential
function is <10% in isotope renogram, some rec-
ommend percutaneous nephrostomy (PCN) and
await recovery of renal function. However, PCN
increases the chances of infection and high
chance of early dislodgement and makes the sub-
sequent pyeloplasty more difficult.
Nephrectomy should rarely be required in
children for hydronephrosis due to pelviureteric
junction obstruction.
 yeloplasty Operation (Open or
P
Laparoscopic or Robotic)
Dismembered (Anderson-Hynes) pyeloplasty
(Figs. 47.6 and 47.7).
Principals of pyeloplasty operation include a
wide, dependent, and watertight pelviureteric
anastomosis after removal of the narrow
­pelviureteric junction. The use of fine absorbable
suture, transanastomotic stent (DJ or n­ ephrostent),
nephrostomy (optional), and perinephric drain is
preferred.
294 47 Hydronephrosis

• Non-dismembered (Culp flap, Foley Y-V flap, are treated conservatively with serial ultrasound
spiral flap) pyeloplasty. scan as the MCKD may regress in size over a
• Endopyelotomy. period of time. Symptomatic cases with large
• Laparoscopic and robotic pyeloplasty are
currently favored minimally invasive
techniques.

Multicystic dysplastic kidney is a developmen-

tal disorder characterized by replacement of the
renal parenchyma with multiple small cysts
(Figs. 47.8 and 47.9). This condition usually
affects one side and needs to be differentiated
from the PUJ obstruction (see above) and other
forms of renal cystic diseases like polycystic
renal disease. Associated urinary defects could
be atretic ureter and vesicoureteric reflux.
Multicystic dysplastic kidneys are poorly func-
tional, and bilateral cases are incompatible with
life. Unilateral cases with normal opposite ­kidney Fig. 47.7 Pelviureteric junction obstruction at surgery

PUJ Obstruction Dismembered pyeloplasty Completed pyeloplasty

Fig. 47.6 Steps of Anderson-Hynes pyeloplasty

a b

Fig. 47.8 (a) Multicystic dysplastic kidney on ultrasonography, (b) specimen with atretic ureter
Obstructive Uropathy in Children 295

Post-natal evaluation-
CBC, SE, KFT, urine
analysis, blood
pressure, bladder
palpable/lump abdomen
No
hydronephrosis

Unilateral Bilateral Hydronephrosis

Hydronephrosis Start antibiotic prophylaxis

SFU grade I & II- SFU grade III & IV-

follow with serial start antibiotic
USG 4 monthly for 1 prophylaxis, direutic VCUG at 1
year, if stable review DTPA/MAG 3 week if
SOS or manage as renography urine C/S
per SFU grading sterile

Differential function Non obstructive-VCUG

> 40% observe, Normal stop antibiotics
repeat USG and follow with USG 4
renography after monthly till 1 year
4 months- if
deteriorates consider
operative intervention

VCUG normal, no
Posterior uretheral valves reflux. SFU grade I VU Reflux-
Valve ablation, & II, serial USG continue antibiotic
vesicostomy or supra scans 4 monthly till prophylaxis,
vesical diversion I year, if deteriorates further VUR
manage as per SFU management
grade

Fig. 47.9 Algorithm of management for antenatally detected hydronephrosis

296
lump, recurrent UTI, or hypertension may require
surgical removal. Management of antenatally
diagnosed hydronephrosis is shown in Fig. 47.9.
Suggested Reading
47 Hydronephrosis
Belman BA, King LR, Kramer SA, editors. Clinical pedi-
atric urology. 4th ed. USA: Martin Dunitz; 2002.
Coran AG, Caldamone A, Scott Adzich N, Krummel TM,
Laberge JM, editors. Pediatric surgery. 7th ed. Oxford:
Elsevier; 2012.
Thomas DF, Duffy PG, Rickwood AMK, editors.
Essentials of pediatric urology. 2nd ed. London:
Informa Healthcare; 2008.
 Inguinal Hernia, Hydrocele, Acute
Scrotum and Varicocele
Inguinal Hernia
The incidence of inguinal hernia in children is
0.8–4.4%. In premature babies, the incidence is
higher, 16–25%. The usual age of presentation is
during the first year of life and the male-to-female
ratio is 3:1.
Pathogenesis: Presence of patent processus
vaginalis (PPV) leads to inguinal hernia. If the
sac and the internal opening are large, then a her-
nia results; if they are small, then a hydrocele
results. Indirect inguinal hernia is the commonest
type in children.
Side: Right side is affected in 60%, left side in
30%, and bilateral in 10% cases.
Factors contributing to development of her-
nia: Although most of the cases are isolated, but
the following conditions have high association
with inguinal hernia.
undescended testis, exstrophy of bladder, asci-
tes, VP shunt, peritoneal dialysis, exomphalos,
cystic fibrosis, and connective tissue disorders.
Contents: Contents of the hernia sac can be
bowel, omentum, ovary and fallopian tube (only
in females), appendix and cecum or fallopian
tubule in sliding hernia, a Meckel’s diverticulum
(Littre’s hernia), and a portion of the circumfer-
ence of the intestine (Richter’s hernia).
Types:
Complete (sac extending up to the bottom of
the scrotum), funicular (sac extending up to
© Springer Nature Singapore Pte Ltd. 2018
S. Roy Choudhury, Pediatric Surgery, https://doi.org/10.1007/978-981-10-6304-6_48
48
root of the scrotum), bubonocele (sac extend-
ing just outside the external ring), hydrocele of
the cord, and communicating hydrocele
(Fig. 48.1).
Clinical Features
Most of the hernias present with asymptomatic
swelling in the groin which appears on crying
and straining and decreases in size during sleep-
ing or relaxed position (Figs. 48.2 and 48.3). A
proper history from the parents or referring phy-
sician or a photograph is generally reliable
toward the diagnosis. Straining, crying, and
Valsalva maneuver can make the hernia visible.
Silk glove sign in the spermatic cord indicates
presence of a hernia sac. This sign is positive if
the examiner while rubbing the cord side to side
on the pubic tubercle gets the feeling of rubbing
two pieces of silk together. Reducibility with a
gargling sound is characteristic of bowel in the
hernia sac.
Differential Diagnosis
Hydrocele (Fig. 48.4): The swelling is scrotal, it
is possible to get above the swelling, and fluctua-
tion and transillumination tests are positive (in
babies even hernia may be transilluminant).
297
298 48 Inguinal Hernia, Hydrocele, Acute Scrotum and Varicocele

Types of inguinal hernia

Peritoneal
cavity

DIR

Superficial
inguinal ring
Obliterated
VAS processus
vaginalis

Testis

Normal Inguinal hernia Bubonocele

(funicular)

Complete Hydrocele Communicating

inguinal hernia of cord hydrocele

Fig. 48.1 Types of inguinal hernia

Fig. 48.2 Left inguinal hernia, male Fig. 48.3 Bilateral inguinal hernia, female
Surgical Procedure
Fig. 48.4 Right hydrocele
Hydrocele is irreducible except in c­ ommunicating
hydrocele, which may reduce slowly during
sleep.
Encysted hydrocele of the cord: The swelling
is generally limited within the spermatic cord. It
is above the testis with some mobility except
while pulling on the testis it becomes fixed (trac-
tion test). It is fluctuant and transilluminant.
Femoral hernia: The swelling is below and
lateral to the pubis tubercle.
Undescended testis: The testis in the inguinal
canal can be felt as a firm mobile structure. Pain
and typical sickening sensation on pressure (tes-
ticular sensation) are characteristic, and the scro-
tum remains empty.
Lipoma of the cord
Female: Hydrocele of the canal of Nuck
Treatment
Surgery is the recommended treatment for inguinal
hernia in children. There is no role of waiting in
case of a patent processus vaginalis. For premature
babies, due to increased risk of incarceration, surgi-
299
cal repair is recommended before their discharge
from the hospital, after attaining weight of 2 kg.
Surgical Procedure
Herniotomy, which is ligation and division of the
patent processus vaginalis: The hernial sac is iso-
lated, ligated, and divided. It is always useful to
inspect the proximal hernial sac before ligation to
detect any sliding hernia. If the sliding hernia is
present, then the sac can be ligated distal to the
contents, and the deep ring can be tightened
(Bevan repair). In females the deep ring can be
closed after the herniotomy.
The operation can be done as a day-care case
except in premature babies (post-conceptional
age <60 weeks) who should be observed over-
night due to high risk of postoperative apnea.
Unilateral versus bilateral groin exploration is a
controversial issue. The mean contention is that con-
tralateral patent processus vaginalis (CPPV) is pres-
ent in approximately 30–50% of children undergoing
herniotomy. Of these children, 30–50% may develop
a contralateral hernia, so routine contralateral ingui-
nal exploration may benefit only 15–20% of cases.
Hence contralateral exploration for clinical unilat-
eral inguinal hernia is generally not recommended
as in 75% of cases it is unnecessary.
With introduction of laparoscopy, this CPPV
can be managed without any significant increase
in operative time or cost.
Contralateral exploration is still recommended
in children with ventriculoperitoneal shunt who
present with hernia as because of increased intra-­
abdominal fluid, it is very likely that the contra-
lateral side will also become symptomatic.
Similarly, for CAPD (peritoneal dialysis) cathe-
ter insertion, PPV can be ruled out by herniogra-
phy (contrast is injected in the peritoneal cavity
and the child is kept in leg down position for
15 min to rule out any contrast in the hernia sac).
If patent processus vaginalis is demonstrated,
then herniotomy is performed.
In patients associated with inherited disorders
of the connective tissue, herniorrhaphy is per-
formed as recurrence rate of herniotomy alone is
very high.
300 48
Laparoscopic correction is gaining popularity.
In girls laparoscopy allows visualization of the
internal genitalia. Another advantage is that
asymptomatic CPPV can be diagnosed and man-
aged in the same sitting.
Complications
Incarceration, i.e., irreducible hernia: The con-
tents get trapped distal to the deep inguinal ring
(more common) or the superficial inguinal ring.
This is more common in the first 6 months and is
rare after 4–5 years of age.
Obstruction, i.e., blockage of the lumen of the
entrapped intestine: This results in intestinal
obstruction.
Strangulation, i.e., obstruction with vascular
compromise: These children present with acute
tender inguinoscrotal swelling with or without
features of peritonitis.
Most incarcerated hernias in children can be
successfully reduced by sustained gentle com-
pression following analgesia. It is necessary to
observe the child for 24 h after a successful
reduction for features of peritonitis as occasion-
ally gangrenous bowel can also be reduced.
Surgery is done after 48 h after the reduction to
allow resolution of edema. If manual reduction
fails, then surgical reduction is required.
Hydrocele
Types of Hydrocele
Vaginal, infantile, congenital (communicating),
and hydrocele of the cord.
Diagnosis
The most common presentation is an asymptom-
atic scrotal swelling which on examination is
fluctuant, transilluminant, and nonreducible and
one can get above the swelling (Fig. 48.4). If the
swelling can be reduced or there is significant
change in size of swelling during the day, then
Inguinal Hernia, Hydrocele, Acute Scrotum and Varicocele
the communication of the hydrocele with the
peritoneal cavity is present (communicating
hydrocele).
Hydrocele of the cord: For hydrocele swelling
above the testis, mobile traction test fixes it due
to attachment to the cord.
Treatment of Hydrocele
Majority of congenital hydroceles resolve spon-
taneously by 2 years of age. Surgery is indicated
if the hydrocele persists beyond 2 years of age
and in cases of communicating hydrocele with
PPV. The operation comprises of herniotomy
(ligation of processus vaginalis) with drainage of
the distal hydrocele sac fluid.
Postoperative Complications
Scrotal swelling, iatrogenic undescended testis,
recurrence, injury to the vas deferens, testicular
atrophy, and intestinal injury.
Causes of Acute Scrotum in Children
Torsion of the appendix of testis
Torsion of the testis
Epididymitis, epididymo-orchitis
Idiopathic scrotal edema
Trauma
Incarcerated inguinal hernia
Acute hydrocele
 orsion of the Appendix Testis
T
(Hydatid of Morgagni)
Incidence is four times more common than tor-
sion testis. Generally occurs in 7–10-year-old
boys. Onset of pain is usually gradual but may be
accompanied with nausea and vomiting.
Tenderness at the upper pole of the testis with the
“blue dot” sign is characteristic. Spontaneous
involution due to ischemic necrosis of the appen-
dix can happen. Treatment consists of supportive
Hydrocele
therapy with analgesics, limited mobility, and
warm compresses. Surgery is only indicated if
torsion of the testis cannot be ruled out. At scrotal
exploration, excision of the twisted appendix is
performed.
Torsion of the Testis
In this condition, the testis rotates upon its pedi-
cle resulting to a compromise of its blood
supply.
There are two peak age groups, neonatal and
pubertal.
Predisposing factors for torsion are:
(a) Inversion of the testis is the commonest. Lie
of the testis is transverse/horizontal or upside
down.
(b) High investment of the tunica vaginalis “bell
clapper” deformity: Because of which, the tes-
tis hangs free and can twist within the tunica
vaginalis. Many children with this deformity
have a transverse lie of testis in the scrotum.
(c) Rapid cremaster muscle contraction may
also cause rotation of the testis.
(d) It is also proposed the pubertal testis with
increased vascularity is more prone to torsion.
Intravaginal and extravaginal torsion
Neonatal torsion is generally extravaginal
(twist of the spermatic cord), and pubertal torsion
is intravaginal due to “bell clapper” deformity. In
neonates, the loose tissue around the testis allows
the testis along with its tunica vaginalis to twist.
a b
Fig. 48.5 (a, b) Acute
epididymo-orchitis
301
During postneonatal age, once the tunica vagina-
lis is fixed in the scrotum, extravaginal torsion
cannot occur.
Presenting Features
Acute torsion presents with sudden onset of
excruciating pain in the groin and lower abdomen
associated with nausea and vomiting. Trauma,
straining, and physical activity are precipitating
factors. Palpation of abnormal lie of the testis and
a thickened tender twisted spermatic cord are
characteristic.
Acute epididymo-orchitis clinically resembles
torsion of the testis (Fig. 48.5a, b).
Elevation of the testis reduces pain in
epididymo-­orchitis, whereas it makes it worse in
case of torsion. Associated urethritis, urinary
tract infection (E. coli), and viral infection are
common in epididymo-orchitis. In mumps orchi-
tis, the cord in general is not thickened.
Real-scale ultrasonographic appearance of
whirlpool sign of the cord with decreased blood
flow in color Doppler is characteristic of testicu-
lar torsion.
Radionuclide study with technetium 99
can also show whether the testis is perfused or
not.
Idiopathic scrotal edema presents at
4–12 years of age with red swollen scrotum with-
out much pain or tenderness. The swelling may
extend to the perineum, groin, and penis. It is
probably due to allergic reaction and generally
subsides in a day or two.
302 48 Inguinal Hernia, Hydrocele, Acute Scrotum and Varicocele

Management (Fig. 48.6) testicular atrophy and subfertility due to abnor-

These investigations are of doubtful utility in
children who are prepubertal, as the testis vol-
ume is small and accuracy of these tests is
limited.
In suspected cases, immediate surgical
exploration is needed. Exploration is done
through a midline scrotal incision. The
involved testis is derotated, and the testis is
covered with warm sponge. The involved tes-
tis is assessed for viability and managed as
below.
mally raised scrotal temperature. Most are noted
in the adolescence or early adulthood. The left
side is affected in 95% of cases. The causes for
increased involvement of the left side are many,
but the primary cause is the lack of valves in the
left testicular vein. Obstruction of the left testic-
ular vein due to renal tumor can cause
varicocele.

• Necrotic testis—orchidectomy. A nonviable

testis may be removed with prior consent
(Fig. 48.7).
• Viable testis—orchidopexy.
• Doubtful viability—for age >10 years, orchi-
dectomy because of risk of alloimmunization
secondary to damage to blood-testis barrier,
and for patients age <10 years testis may be
preserved.

Varicocele

Varicocele is due to abnormal dilatation of the

veins of the pampiniform plexus. It can lead to Fig. 48.7 Gangrenous testis due to acute tortion

History
History and
and physical
physical
examination
examination

Suggestive of acute torsion with

Suspected torsion
short duration

Scrotal exploration Colour

Colour Doppler
Doppler ultrasound
ultrasound

No blood flow Blood flow normal or increased

Fig. 48.6 Algorithm of

evaluation and
Exploration Observation
management of acute
scrotum in children
Suggested Reading 303

Clinical Features ligament. However, the most preferred surgery

is mass ligation of the testicular vessel above
The scrotum on the affected side hangs lower the inguinal ligament (Palomo operation). This
than normal, and on palpation, there is a “bag of can be done very effectively by laparoscopy.
worms” feel of the dilated vessels (Fig. 48.8). On This procedure ensures that all the veins are
lying down, the veins empty by gravity. The ipsi- ligated. Collaterals maintain the vascular sup-
lateral testis may be smaller and softer in long-­ ply to the testis. Alternative treatment is embo-
standing varicocele. lization of the testicular vein under radiology
Diagnosis is often made on Doppler ultra- control.
sound, but the accuracy in children may be lim- Postoperative complications include recur-
ited due to smaller size of the testis. rence, reactive hydrocele, and testicular atrophy
Indications for treatment are chronic pain, dis- (rare). The recurrence rate after surgery is very
comfort, or demonstrable testicular atrophy. In high due to filling up of the collateral veins.
adults, demonstration of associated subfertility is
also an indication for surgery.
Operative treatment comprise of ligation of Suggested Reading
the testicular vein preferably above the inguinal
Coran AG, Caldamone A, Scott Adzich N, Krummel TM,
Laberge JM, editors. Pediatric surgery. 7th ed. Oxford:
Elsevier; 2012.
Hadidi AT, Azmi A, editors. Hypospadias surgery. 1st ed.
Heidelberg: Springer; 2004.
Kavoussi LR, Novick AC, Partin AW, Peters
CA. Campbell–Walsh urology. 10th ed. Philadelphia:
Elsevier Saunders; 2012.

Fig. 48.8 Left varicocele

 Undescended Testis
Absence of normal testis in the scrotum in male
individual is generally termed undescended testis
(UDT). The pathological abnormalities involving
such gonads influence the outcome.
Embryology
Gonadal tissue located on the embryo’s genital
ridge begins differentiation into a testis during
sixth and seventh intrauterine life under the influ-
ence of the testis-determining SRY gene. Sertoli
cells produce Mullerian inhibitory factor (MIF)
causing regression of the Mullerian duct struc-
tures. Leydig cells start producing testosterone
by the ninth week which stimulate development
of Wolffian structures including the epididymis
and vas deferens. From seventh to eighth month,
the testis descends through the inguinal canal
into the scrotum.
Factors Influencing Testicular
Descent
 ubernaculum: This is a mucofibrous structure
G region; the rest are impalpable (25%).
attached to the testis and scrotum and guides the
descent of the testis into the scrotum. Epididymis
also plays a role in the descent.
© Springer Nature Singapore Pte Ltd. 2018
S. Roy Choudhury, Pediatric Surgery, https://doi.org/10.1007/978-981-10-6304-6_49
49
There are two phases of testicular descent:
• Transabdominal descent (8–15 weeks)—In
this phase the testis descends from the retro-
peritoneum to the deep inguinal ring. It is
because of regression of the cranial suspen-
sory ligament (under influence of androgens)
and thickening of the gubernaculum (under
the influence of insulin-like factor).
• Inguinoscrotal migration (28–35 weeks)—
This is under androgenic control and maybe
due to release of calcitonin gene-related pep-
tide from the genitofemoral nerve. Migration
may be aided by transmission of intra-­
abdominal pressure through patent processus
vaginalis (PPV).
Incidence
Undescended testis (UDT) is found in 30% of
preterm babies, 4% of term babies, and 1% at
1 year of age. A descent of testis can occur post-
natally but is rare after the age of 3 months. Two
thirds of UDT are palpable in the inguinal
Associated anomalies include PPV, epididymal
anomalies, hypospadias and urinary tract
abnormalities.
305
306
Classification
1. True UDT: Sites could be intra-abdominal,
intracanalicular or pre-scrotal in the superfi-
cial inguinal pouch (most common).
2. Retractile UDT: Due to cremasteric reflex
which is active between the ages of 3 and
9 years, the testis gets drawn up.
3. Ectopic UDT: (Sites) perineum, contralateral
scrotum, or femoral region (Fig. 49.1)
4. Ascended testis: Once descended, however,
and gone back up, this could be primary or
more commonly iatrogenic following hernia
or hydrocele surgery or trauma.
Fig. 49.1 Crossed testicular ectopia
49 Undescended Testis
Terminologies
• Cryptorchid—Testis neither resides nor can
be manipulated into the scrotum.
• Ectopic—Aberrant course of the testis.
• Retractile—Testis can be manipulated into the
scrotum where it remains without tension.
• Gliding—Testis can be manipulated into the
upper scrotum but retracts when released.
• Ascended—Previously descended and then
“ascends” spontaneously. It occurs because of
failure of spermatic cord to lengthen ade-
quately, while the length of inguinal canal
increase as the child is growing.
• Emergent (peeping) testis—Intracanalicular
testis which is usually not palpable because of
overlying musculature but can be squeezed
through the superficial inguinal ring and be
palpated.
From the management point of view, the most
important classification is:
• Palpable testis
• Non-palpable testis (around 25% cases)
Clinical Features
The right side is affected in 50%, left in 30%,
and bilateral in 20% cases (Figs. 49.2a, b and
49.3).
Careful history and physical examination is
essential for diagnosis and planning the man-
agement. Gentle examination in a comfortable
room, both supine and standing position, is
necessary.
Gliding test: This is done by rolling the fingers
of one hand from the deep to the superficial ring
and trying to push the subcutaneous structures
towards the scrotum using gel or powder and the
fingers of the other hand at the root of the ­scrotum
Complications 307

Fig. 49.2 (a, b) Right

undescended testis; note a b
the empty scrotum

t­ raction; however the later does not remain in the

scrotum. Pathological changes are seen in UDT
but not in retractile testis. Ipsilateral hemiscro-
tum is poorly developed in true undescended
testis.

 ifferentiation of Low UDT Vs.

D
Retractile Testis

HCG stimulation test—Intramuscular injection

of HCG may help in the descent of retractile tes-
tis; however a true UDT will not descend.

 ifferentiation of Anorchia
D
Fig. 49.3 Bilateral empty scrotum, undescended testis
(Bilateral Absence of Testis)
Vs. Intra-abdominal Testis
trying to grab or role the testis. Also examination (Cryptorchidism)
in a sitting or squatting position and gentle
abdominal squeeze to push the testis in to the HCG stimulation test: Baseline testosterone,
inguinal canal improve palpation. FSH, and LH levels are measured. Elevated base-
Both retractile testis and low UDT may be line FSH level indicates anorchia. If baseline
manipulated into the scrotum. The former FSH/LH levels are normal, then elevation of
remains in the scrotum temporarily without serum testosterone level following HCG
308
s­timulation (2000 IU intramuscular daily for
3 days) indicates the presence of functioning tes-
ticular tissue. However a negative response does
not exclude the presence of testis; hence an
exploration is indicated.
Radiological Localization
US scan, CT and MRI are generally unnecessary
for localization of testis. MRI is superior and
may be useful in obese children, and gadolinium
MR angiography has high sensitivity for localiza-
tion. For impalpable testis, laparoscopy is the
current procedure of choice.
Complications
Infertility
In spite of histological abnormalities in UDT and
the contralateral descended testis, fertility rate of
men with unilateral UDT is normal. Bilateral
UDT is associated with 50% infertility.
Malignancy
The risk is 10–60 times higher than normal in
UDT cases. Orchidopexy done at early age
reduces the risk and facilitates testicular
a b
Fig. 49.4 (a) Testicular
torsion in undescended
testis. (b) Vascular
insufficiency due to
torsion
49 Undescended Testis
examination and cancer detection.
Contralateral normally descended testis also
has a risk of developing malignancy. Risk is
highest in intra-­
abdominal testis. The most
frequent malignant tumor associated with
UDT is seminoma.
Others
Pain, hernia, torsion (Fig. 49.4a, b) epididymo-­
orchitis, and atrophy.
Treatment
The current recommended age for surgery is
between 6 months and 1 year. Early surgery is
indicated for an associated inguinal hernia. In
postpubertal men with unilateral palpable UDT,
if the testis appears normal, then orchidopexy
should be done; if the testis appears abnormal
(soft, small, atrophic), then orchidectomy is rec-
ommended (Fig. 49.5). Orchidectomy is best for
unilateral intra-abdominal testis in postpubertal
men because of the high cancer risk. Prior con-
sent for orchidectomy and post-removal histo-
pathological examination is mandatory.
Retractile testes are more common than true
undescended testis and require no treatment but
reassurance to the parents that by puberty the tes-
tis will get heavier and stay in the scrotum.
Surgical Treatment
Fig. 49.5 Atrophic UDT in the inguinal canal should be
removed and sent for histopathology
The role of hormonal treatment, like low-dose
HCG (Buserelin), is not standardized and currently
not in use, except in some European countries.
Surgical Treatment
Operative treatment depends on palpability of the
testis:
(a) For palpable testis: Open orchidopexy is the
standard treatment (Fig. 49.6).
309
Fig. 49.6 Open orchidopexy; note the separation of the
intact sac from the spermatic cord structures
(b) For non-palpable testis: Currently laparos-
copy is the best procedure (Fig. 49.7a, b).
Orchidopexy for palpable testis is relatively
easier. The following maneuvers may be
employed to achieve adequate length of the vas
and vessels so that the testis can reach the sub-­
dartos pouch in the scrotum:
1. Dissect the processus vaginalis completely
free from the cord.
2. Incise the deep inguinal ring and mobilize the
cord structures retroperitoneally.
3. Divide all the retroperitoneal attachments.
4. Divide the floor of the inguinal canal and fas-
cia transversalis.
5. Divide the deep epigastric vessels (Prentiss
maneuver) to achieve a straighter route to the
scrotum.
If testis still cannot be brought down while
performing open orchidopexy, the following
maneuvers can be performed:
• Staged orchidopexy: it includes initial mobili-
zation and pexing the testis at the lowest
310 49 Undescended Testis

Fig. 49.7 (a, b)

a b
Laparoscopic view of
peeping testis

Table 49.1 Surgical man- Laparoscopy

agement of impalpable testis

Blind ending Vessels crossing Intra abdominal

vessels internal ring testis

Absent testis Inguinal Laparoscopic

no further action exploration orchidopexy or
orchidectomy

Orchidopexy or
orchidectomy

­ ossible site, and after a period of 6 months, a

p Fowler-Stephens (F-S) Orchidopexy
second operation to bring the testis down to The principle is high ligation and division of
the scrotum is often successful. testicular vessels which helps in lengthening the
• Fowler-Stephens (see below) procedure. cord to bring down the intra-abdominal testis.
• Microvascular anastomosis of testicular ves- The vascular supply of the testis will depend on
sels to the deep inferior epigastric vessels. the collateral circulation coming from a. inferior
vesical artery, b. artery to vas, and c. cremasteric
Laparoscopic orchidopexy for non-palpable artery around a peritoneal covering of the testis.
testis (Table 49.1). A two-stage operation after an interval of
Depending on the laparoscopic findings, the 6–12 months gives better result than single-stage
following five scenarios are feasible: F-S orchidopexy because of better development
of collateral circulation to the testis. A low liga-
• Blind-ending vas and vessels—Indicating tion of testicular vessels (Koffs) has also been
absent testis; hence no intervention is required. shown to be effective in achieving a good collat-
Blind-ending vessels are a sure indication of eral circulation for the testis.
absent testis.
• Atrophic intra-abdominal testis—Orchidectomy.
• Intra-abdominal testis adequately mobi- Suggested Reading
lized—Laparoscopy-assisted orchidopexy.
• Intra-abdominal testis very high—Fowlers-­ Coran AG, Caldamone A, Scott Adzich N, Krummel TM,
Stephens procedure. Laberge JM, editors. Pediatric surgery. 7th ed. Oxford:
Elsevier; 2012.
• Vas and vessels entering the inguinal ring— Belman BA, King RL, Kramer SA (eds). Clinical pediat-
Inguinal exploration. ric urology. 4th ed. USA: Martin Dunitz; 2002.
 Hypospadias and Epispadias
50

Hypospadias (b) Genital tubercle forms the body of the penis.

Hypospadias is a birth defect where the opening of
the urethra is on the under surface (ventral surface)
of the penis (Figs. 50.1, 50.2, 50.3, 50.4, and 50.5).
Development of the Penile Urethra
(a) This occurs between the 7th and 14th week
of development.
Mesenchymal tissue forms the corporal bod-
ies, bucks fascia, dartos fascia, and corpus
spongiosum.
(c) Urethral folds form on either side of urethral
groove on the ventral side of the genital
tubercle to form the penile urethra. Glandular
urethra formed either by invagination of the
ectodermal glandular tissue or by endoder-
mal cellular differentiation.
(d) Two genital swellings fuse to form the scrotum.

Fig. 50.1 Types of

hypospadias
Glanular
Anterior (50%)
Subcoronal
Distal penile

Middle (30%)
Midshaft

Proximal penile
Penoscrotal

Scrotal Posterior (20%)

Perineal

© Springer Nature Singapore Pte Ltd. 2018 311

S. Roy Choudhury, Pediatric Surgery, https://doi.org/10.1007/978-981-10-6304-6_50
312 50 Hypospadias and Epispadias

Fig. 50.2 Coronal hypospadias Fig. 50.3 Mid-penile hypospadias

Fig. 50.4 Proximal penile hypospadias with chordee

Fig. 50.5 Penoscrotal

hypospadias with
chordee, hooded prepuce
Principles of Repair 313

Types of hypospadias (according to the loca- Anatomical Defect

tion of the meatus) (Figs. 50.1, 50.2, 50.3, and
50.4): Apart from the abnormal location of the meatus,
other defects are also present in hypospadias:
Anterior 50% cases
Glandular (a) Ventral chordee – ventral curvature of the
Coronal (Fig. 50.2) shaft of the penis
Middle 30% cases (b) Absent or splayed corpus spongiosum
Distal penile (c) Hooded prepuce due to deficiency of prepuce
Mid-penile (Fig. 50.3) ventrally (Fig. 50.4)
Proximal penile (Fig. 50.4a, b)
Posterior 20% cases Circumcision should not be done in cases of
Penoscrotal (Fig. 50.5a, b) hypospadias, as the preputial skin is often
Scrotal required for the repair.
Perineal

Incidence
Incidence of hypospadias is 1 in 300 boys; it is
one of the most common urinary anomalies in
boys.
Etiology
Etiology is not clear but is multifactorial.
Proposed defects are genetic (familial), defect in
androgen stimulation, enzymatic defects, and
environmental factors.
Associated Anomalies
Undescended testis, inguinal hernia, enlarged
prostatic utricle, and upper urinary tract
anomalies.
Cases of severe penoscrotal hypospadias,
hypospadias with undescended testis, or micro-
penis require karyotyping and other i­ nvestigations
to exclude DSD anomalies (see Chap. 58 on dis-
orders of sexual differentiation, DSD).
Ideal Time of Repair
Between 6 and 15 months of age, ideally before
school-going age.
Objectives of Repair
1. Straight penis (orthoplasty)
2. Urethral meatus at the tip and good caliber
neourethra (urethroplasty).
3. Symmetric conical glans (glansplasty).
4. Satisfactory configuration of the meatus (meato-
plasty).
5. Satisfactory cosmetic skin cover (preputio-
plasty).
Principles of Repair
Single-stage repair is ideal whenever possible.
Decisions regarding the choice of the opera-
tion and stages depend on the anatomy of the
defect.
1. Size of the penis: Very small phallus may
require preoperative testosterone stimulation
(injection testosterone enanthate 25 mg IM,
three injections at three weekly interval or
topical testosterone cream application) to
increase the size.
2. Severity of the defect. More proximal hypo-
spadias requires staged operation versus the
distal ones, which are often managed, by
single-­stage operation.
3. Quality of the urethral plate. Good urethral
plate can be used for urethral tube formation
in one-stage repair.
314 50 Hypospadias and Epispadias

Hypospadias

No Chordee
Persistent chordee despite
or
thorough degloving and/or
Chordee corrected with Preservation of
Nesbitt plication
the urethral plate

Urethral plate transection

Good glans configuration Poor glans configuration
and Staged repair

Snodgraft
MAGPI Foreskin rotation graft
Inner prepucial graft
Snodgrass (TIP) Bracka Inner prepucial free graft
Duckett tube
GAP/Pyrimid Duckett tube
Onlay

Fig. 50.6 Algorithm of surgical management of hypospadias (MAGP, meatal advancement and glanuloplasty; GAP,
glans approximation procedure; TIP, tubularized incised plate urethroplasty)

4. Anatomy of the glans: Good size and broad
glans are better for glansplasty versus narrow
conical glans which may require a skin graft.
5. Chordee. Severe chordee may require staged
operation with chordee correction as the ini-
tial stage. Fortunately most chordee are
­corrected on degloving the penis, and mild
residual chordee can be taken care by dorsal
plication (modified Nesbit’s procedure).
Commonly Practiced Operations
The operation may be done in one or two stages
(Fig. 50.6).
Single-Stage Urethroplasty
For single-stage urethroplasty, the most com-
monly practiced procedure is Snodgrass TIPS
(tubularized incised plate urethroplasty) opera-
tion. It is necessary to have a supple urethral plate
and no residual chordee after degloving for this
procedure to be performed. Other procedures,
which may be helpful depending upon the case,
provided chordee is absent or has been corrected,
are as follows:
• MAGPI (meatal advancement and glanulo-
plasty) procedure: Suitable for anterior hypo-
spadias, mobile urethra, no thinning of distal
ventral urethra, web of tissue in glans distal to
the meatus.
• GAP (glans approximation procedure):
Suitable for cases with deep navicular fossa,
no web of tissue in glans.
• Mathieu procedure: Suitable for coronal or
distal penile hypospadias with good perime-
atal mobile skin, shallow navicular fossa.
• Duckett’s onlay urethroplasty: In this proce-
dure the inner preputial skin island flap is
brought ventrally and sutured to the urethral
plate. This procedure is useful in cases of mid-
dle hypospadias where the urethral plate is
narrow for TIPS urethroplasty.
Two-Stage Operations
In cases of posterior hypospadias, the chordee
persists even after degloving. In this scenario the
Types of Epispadias 315

urethral plate needs to be divided for straighten- persistent chordee, sexual dysfunctional prob-
ing the penis. Most surgeons then prefer a staged lems, and psychological problems.
approach of chordee correction followed by a
second-stage urethroplasty.
Epispadias
• Byars flap: After chordee correction, the pre-
putial skin is brought ventrally as a pedicle The meatus is present on the dorsal surface of the
flap in the first stage, and after 6 months, this penis and is often associated with dorsal
is tubularization to form the urethra. chordee.
• Bracka’s two-stage urethroplasty: After chor- It almost always forms the part of classical
dee correction inner preputial, free skin graft bladder exstrophy; isolated epispadias are com-
is used to cover the ventral raw area. A second paratively rare.
stage tubularization is done after 6–9 months. Incidence: 1 in 120,000 in boys and 1 in
• In some cases Duckett’s tube urethroplasty can 500,000 in girls
be performed in which the tube is fashioned
from the inner preputial skin and brought to the
ventral surface to bridge the gap between the Types of Epispadias
proximal meatus and the tip of the glans.
Asopa’s inner preputial onlay flap urethroplasty Isolated
is a useful alternative procedure in such cases.
• Glandular (Fig. 50.7)
• Penile
Complications • Penopubic (Fig. 50.8)

Wound infection, fistula formation, meatal steno- With exstrophy-epispadias complex (more com-
sis, urethral stricture, scarring, poor cosmesis, mon), see Chap. 52 on exstrophy bladder.

Fig. 50.7 Glandular epispadias Fig. 50.8 Epispadias (penopubic)

316
Depending upon the urinary continence, it
can be classified as:
• Incontinent epispadias (always wet): Bladder
neck is also deficient and often needs bladder
neck reconstruction in addition to epispadias
repair.
• Continent epispadias (dry): Only epispadias
repair may suffice.
Treatment
Principles of repair are similar to hypospadias
repair (see above). The techniques available are:
50 Hypospadias and Epispadias
• Cantwell-Ransley repair (preferred)
• Mitchell’s penile disassembly technique
Suggested Reading
Hadidi AT, Azmi A, editors. Hypospadias surgery. 1st ed.
Heidelberg: Springer; 2004.
Kavoussi LR, Novick AC, Partin AW, Peters CA.
Campbell—Walsh urology. 10th ed. Philadelphia:
Elsevier Saunders; 2012.
 Prepuce and Circumcision
51

Prepuce or foreskin is a double-layered cover

over the glans penis in male or clitoris in
female. The prepuce of a child is a frequent
cause of concern in the family and the doctor.

Physiology of Foreskin

At birth the prepuce is almost always nonretractable

due to congenital adhesion between the glans penis
and the prepuce (Fig. 51.1). This developmental
nonretractility of the foreskin is often wrongly
termed phimosis. Preputial separation continues
after birth and even at 5 years of age; some degree
of preputial adherence persists in boys. The fore- Fig. 51.1 Preputial adhesion
skin, if left alone, becomes fully and easily retract-
able by physical maturity in majority of boys.
“Ballooning” of the foreskin during micturition is a
common phenomenon in the period of early child-
hood when the foreskin is still nonretractile, but  anagement of Nonretractable
M
when substantial preputial separation has taken Foreskin
place. Ballooning in early childhood calls for no
treatment as it is both transient and self-limiting, Traditionally inability to retract the foreskin over
resolving as the prepuce becomes more retractable. the glans has been termed as phimosis. A consider-
Pathological ballooning is one that persists after able number of patients attend clinic due to paren-
voiding and causing urinary obstruction. tal anxiety for narrow preputial opening. A large
The foreskin typically protects the glans. The proportion of them are due to physiological nar-
prepuce has a rich somatosensory innervation rowing as described above. It is important to dif-
forming an important component of the penile ferentiate this from pathological narrowing of the
erogenous tissue and thus sexual satisfaction. foreskin.

© Springer Nature Singapore Pte Ltd. 2018 317

S. Roy Choudhury, Pediatric Surgery, https://doi.org/10.1007/978-981-10-6304-6_51
318 51 Prepuce and Circumcision

Indications for Intervention may be performed in the out patient department

Presence of associated dysuria, balanitis or scar-
ring needs intervention. Recently steroids have
been shown to be effective in the management of
phimosis. Twice daily application of a topical ste-
roid (concentration 0.05–0.1%) for a period of
4 weeks may show response in up to 90% chil-
dren with recurrence rate of 17%. The practice of
forceful retraction of the foreskin in young boys
with narrow preputial opening should be avoided
as it can result in more scarring and pathological
phimosis. However, in older children with persis-
tent prepucial adhesion, retraction of the foreskin
after gentle separation from the underlying glans
with satisfactory results.
Indications for Circumcision
Failure to respond to steroids and presence of sig-
nificant scarring of the foreskin are the indica-
tions for ­ circumcision. Pathological phimosis
constitutes the indication for circumcision in
boys. This is characterized by scarred, nonre-
tractable foreskin in older children (above
2 years) which fissures and bleeds on attempted
retraction (Fig. 51.2a–d). Recurrent balanopos-
thitis is also an indication for circumcision.

a b

c d

Fig. 51.2 (a–d) Pathological phimosis, note the scarred nonretractable foreskin
Paraphimosis
Preputioplasty may be an alternative to circumci-
sion for narrow preputial opening.
Balanitis xerotica obliterans
(BXO) is a rare pathological condition of the fore-
skin, with a peak incidence in boys aged 9–11 years.
The etiology of BXO is unknown. The preferred
treatment is circumcision. For milder forms of
BXO, the application of a potent topical steroid
(e.g., 0.05% mometasone furoate, 0.05% clobeta-
sol propionate, or 0.05% betamethasone cream)
may ameliorate local symptoms and result in an
improvement in the appearances of the foreskin.
Unnecessary circumcisions should be avoided,
particularly for physiological phimosis in boys
under 5 years old. All parents of boys with hypo-
spadias, as well as those with epispadias or
ambiguous genitalia, and buried penis should be
advised against circumcision as the foreskin may
be required for reconstructive purposes. A family
history of bleeding disorder is a relative contrain-
dication to circumcision.
 oints to Remember in the
P insect bite (Fig. 51.5), or allergic reaction.
Management of Narrow Prepuce
• The foreskin is generally unretractable at
birth; it spontaneously becomes fully and eas-
ily retractable in boys by puberty.
• Conservative treatment should be offered for
the nonretractable foreskin in infants.
a b
Fig. 51.3 (a–c) Smegma collection
319
• Effective treatments for the nonretractable fore-
skin include the use of topical steroid therapy.
• Recurrent balanoposthitis may require prepu-
tioplasty or circumcision.
• Pathological phimosis due to balanitis xerot-
ica obliterans is rare under the age of 5.
• Pathological phimosis and BXO are definitive
indications for circumcision.
Smegma Collection
This is a common problem which presents with a
whitish mass under the foreskin (Fig. 51.3a–c).
In most cases the child is asymptomatic, and par-
ents can be reassured that with retraction of pre-
puce, this collection will resolve. Treatment is by
retraction of the foreskin and cleaning the collec-
tion and maintaining local hygiene with regular
cleaning.
Acute balanoposthitis (Fig. 51.4):
Inflammation of the prepuce is called posthitis,
and that of the glans is called balanitis. This is
treated with topical and or systemic antibiotics
and maintaining local hygiene.
Edema of the foreskin could be due to trauma,
Paraphimosis
Paraphimosis (Fig. 51.6) results from a tight fore-
skin which after forceful retraction over the glans
becomes non-reducible. The foreskin and glans
c
320
Fig. 51.4 Acute balanoposthitis
Fig. 51.5 Penile foreskin edema due to insect bite
51 Prepuce and Circumcision
Fig. 51.6 Paraphimosis
swell up due to venous and lymphatic obstruc-
tion. This is a painful condition. Reduction is
aided by manual compression with application of
ice to reduce the edema. Reduction of paraphi-
mosis is painful and should be done under local
anesthesia. A dorsal slit in emergency may be
necessary.
Suggested Reading
Kavoussi LR, Novick AC, Partin AW, Peters CA. Campbell
–Walsh urology. 10th ed. Philadelphia, PA: Elsevier
Saunders; 2012.
 Exstrophy of Bladder
Bladder exstrophy is a defect which forms a part
of midline abdominopelvic fusion abnormalities
known as exstrophy-epispadias complex. At one
end of this spectrum lies epispadias with cloacal
exstrophy being the most severe form.
Incidence is 1 in 50,000 live births, with male/
female ratio of 4:1.
Embryology
Exstrophy of the bladder results from incomplete
development of the lower abdominal wall due to
delayed rupture of the cloacal membrane.
Persistence of cloacal membrane prevents medial
mesenchymal growth. If the urorectal septum is
incomplete at the time of rupture of cloacal mem-
brane, then cloacal exstrophy results. Usually the
urorectal septum is complete before the rupture
of membrane, therefore bladder exstrophy
results.
Antenatal Diagnosis
Absent fluid-filled urinary bladder and low-set
umbilical cord are the suggestive findings on
ultrasonography. Other suggestive findings
include lower abdominal bulge, anteriorly dis-
placed scrotum with small phallus, and abnormal
widening of iliac crests.
© Springer Nature Singapore Pte Ltd. 2018
S. Roy Choudhury, Pediatric Surgery, https://doi.org/10.1007/978-981-10-6304-6_52
52
Defect
Anatomical defects involve muscular, facial,
bony, and urogenital defects.
The common defect to all of exstrophy-­
epispadias complex is diastasis of pubic bones
along with lower abdominal muscle defect due to
divergent recti. The other features of bladder
exstrophy are:
• Absent anterior abdominal wall and bladder
wall.
• Posterior bladder plate everted and exposed.
• Absent umbilicus with varying degree of
exomphalos.
• Levator ani is also divergent leading to weak-
ness of pelvic floor.
• Anteriorly placed anus.
• Bilateral inguinal hernia and rectal prolapse
are common.
• In males epispadias is present with an
everted urethral plate with short flat glans
(Fig. 52.1). Testes are normally descended
with rudimentary prostate and seminal vesi-
cles. Dorsal chordee with ventral prepuce is
present. Corporal bodies are shorter and the
base of the penis, and scrotum are widely
separated.
• In female patients bifid clitoris with diver-
gence of mons and labia is present (Fig. 52.2).
The introitus is anteriorly displaced.
321
322
Fig. 52.1 Exstrophy bladder, male
Fig. 52.2 Exstrophy bladder, female
Due to chronic inflammation, the exposed
mucosa becomes polypoidal, and squamous
metaplasia may occur (Fig. 52.3) resulting in
recurrent stone formation and even malignant
change in the long term.
Exstrophy Variants
They account for 10% of cases and include cov-
ered exstrophy (Fig. 52.4), pseudoexstrophy,
duplicate exstrophy, and superior vesical fis-
sure. They have muscular, fascial and bony
defect similar to classical exstrophy of the
bladder.
52 Exstrophy of Bladder
Fig. 52.3 Late presentation in female: note epithelializa-
tion of the bladder plate
Fig. 52.4 Superior vesical fissure (variant of exstrophy
bladder)
Cloacal Exstrophy (Fig. 52.5)
Exstrophy of the cloaca is characterised by
exposed and prolapsed intestine in the center of
the defect, hemi-­bladder on both sides and varying
degree of exomphalos (elephant trunk deformity).
Management
Staged Repair Is Widely Practiced
1. Neonate: Early surgical closure of the bladder
and abdominal wall defect and pubic bone
approximation with or without osteotomyis
Suggested Reading
Fig. 52.5 Cloacal exstrophy
recommended. Osteotomy achieves median
pubic bone approximation and decreases the
chances of postoperative dehiscence of the
repair. However, it does not affect the long-
term continence. Osteotomy may not be
needed for pubic approximation if the child is
operated within 72 h of life as the ligaments
are lax under the influence of relaxin.
2. Epispadias repair is generally done at 6 months
to 1 year of age (Cantwell-Ransley repair).
3. Bladder neck repair (BNR) is done by
4–5 years of age as by this time the bladder
has grown to allow BNR. The child is old
enough to physically and psychologically able
to participate in formal voiding program.
Bladder augmentation with catheterizable
stoma after 5 years of age may be needed if blad-
der volume remains small.
One-stage combined exstrophy-epispadias
repair is regaining popularity. In these patients
bladder closure, epispadias repair, and bladder
neck repair are performed in a single operation.
In cases of failed repair or where repair is con-
sidered difficult due to a small patch of bladder,
urinary diversion (ureterosigmoidostomy, ileal
323
conduit, colonic conduit), even continent urinary
diversion (Indiana pouch, Mainz II pouch), may
be considered.
Long-term complications of ureterosigmoid-
ostomy are stricture, hyperchloremic acidosis,
and tumor (adenocarcinoma).
Cloacal Exstrophy
It requires multistage repair with bowel ostomy,
bladder closure, bladder neck reconstruction,
bladder augmentation, and anorectal recon-
struction. Most children are reared as females
as the size of phallus is very small. Most chil-
dren survive with permanent stoma with clean
intermittent catheterization of the urinary
reservoir.
 omplications and Follow-Up
C
for Exstrophy Bladder
• Urinary incontinence almost invariably
occurs. However with a combination of surgi-
cal repair of bladder and bladder neck, bladder
augmentation, and clean intermittent catheter-
ization, urinary continence (significant dry
interval) can be achieved in 80% cases.
• Sexual dysfunction. Generally fertility rate is
low. However successful pregnancy in female
and fertility in male have been reported.
• Psychosocial problems.
• Urinary stone formation.
Long-term follow-up and care are required.
Suggested Reading
Barry Belman A, King LR, Kramer SA, editors. Clinical
pediatric urology. 4th ed. Boca Raton, FL: Martin
Dunitz; 2002.
Kavoussi LR, Novick AC, Partin AW, Peters
CA. Campbell–Walsh urology. 10th ed. Philadelphia,
PA: Elsevier Saunders; 2012.
 Urinary Tract Infection
Urinary tract infection (UTI) is one of the com-
monest disorders in children. In young infants the
clinical picture is often nonspecific, and thus
diagnosis is missed or delayed. In the first
12 months of life, urinary infections are more
common in boys, but thereafter UTIs occur pre-
dominantly in girls. P-fimbriated E. coli are par-
ticularly potent pathogens by virtue of their
adherence properties. Other common infecting
organisms are Proteus vulgaris, Klebsiella,
Enterobacter, and Pseudomonas. The presence
of a foreskin may be a risk factor in boys with
recurrent UTIs and those with urinary tract
abnormalities.
Clinical Features in Suspected UTI
Infants younger than 3 months of age usually
present with fever, vomiting, lethargy, and irrita-
bility. In older children, specific features like dys-
uria, frequency, abdominal pain, loin tenderness,
hematuria, cloudy urine, or foul smelling urine
may be present. Urine sampling is required in
following conditions:
• Signs and symptoms of UTI
• Unexplained fever >38 °C or higher for more
than 24 h
• Children with fever with an alternative site of
infection who remain unwell despite adequate
treatment
© Springer Nature Singapore Pte Ltd. 2018
S. Roy Choudhury, Pediatric Surgery, https://doi.org/10.1007/978-981-10-6304-6_53
53
Urine Sampling
Collection: A clean catch midstream sample is
preferred. For children who are unable to void on
command, urine is obtained by urethral catheter-
ization or suprapubic aspiration. While perform-
ing suprapubic aspiration, the urine container
should be ready as this procedure may itself initi-
ate voiding. Urine sampling from urine collecting
bags should be avoided because of high false-­
positive rates.
Urine dipsticks: They are useful screening
investigations. The leukocyte esterase depends
on the presence of WBC in the urine, whereas the
nitrite test detects the presence or absence of coli-
form bacteria in the urine.
Urine microscopy: Significant pyuria is
defined as >10 WBC/mm3 in an uncentrifuged
specimen and is supposed to be more sensitive.
Usually, centrifuged urine is tested, and the pres-
ence of 5 WBCs/high power field (equivalent to
25 WBC/mm3) is considered significant. The
urine sample should be tested within 1 h if stored
at room temperature or within 4 h if refrigerated.
Urine culture: Criterion for the bacteriologi-
cal diagnosis of urinary infection is a pure growth
of >105 bacterial colony-forming units (CFUs)
per mL. The recent guidelines of the American
Academy of Pediatrics in 2011 have suggested
modification of this criteria as 50,000 CFU/mL
and the presence of pyuria. In specimens obtained
by suprapubic aspiration, lower values may be
325
326 53 Urinary Tract Infection

significant, as is a growth of >500–1000 Gram-­ Further investigations include MCU

positive organisms. Urine samples which cannot (Micturating cystourethrogram) and DMSA scan
be cultured within 4 h should be refrigerated, or depending on the ultrasound abnormality, age of
boric acid should be added to it. the patient, and severity of the infection.
Urine samples should be sent for culture in: MCU is performed in children with abnormal
ultrasound result, atypical or recurrent urinary
• Systemically unwell children of all ages tract infections. The threshold for doing MCU
• All children under the age of 3 years should be low for children below 6 months and
• Single positive result for nitrite or leukocyte for all children with severe/atypical or recurrent
esterase UTI (NICE protocol) or those with urinary symp-
• Recurrent UTI toms. Prophylactic antibiotic cover should be
given before performing MCU.
Children who do not respond to treatment DMSA scan is performed to diagnose renal
within 24–48 h, suspect acute pyelonephritis/ scars and is best performed 4–6 months after uri-
upper urinary tract infection. nary tract infection. It is recommended in cases
with atypical or recurrent infections.

 ymptoms of Lower Urinary Tract

S
Infection Treatment

Symptoms of lower urinary tract infection
include frequency/nocturia, dysuria, secondary
enuresis, suprapubic pain, and hesitancy.
Children with only urinary symptoms without
systemic signs and symptoms are considered to
have lower urinary tract infection.
 ymptoms of Upper Urinary Tract
S Dysfunctional voiding and constipation are fre-
Infection
Upper urinary tract infection is suspected if fever
>38 °C is present with bacteriuria or children
with fever <38 °C and bacteriuria with additional
finding of loin pain or tenderness.
Management: Ultrasound is recommended in
all children below 6 months, febrile infants, and
for older children if the UTI is severe or atypical
or recurrent. Definition of atypical UTI is the
presence of:
• Septicemia
• Seriously ill child
• Poor urine stream
• Deranged renal function test
• Failure to respond to treatment with antibiot-
ics within 48 h
• Infection with non-E. coli organisms
Oral antibiotics are preferred if tolerated.
Children who are “toxic” and cannot take oral
medication are given intravenous antibiotics till
they are well enough for oral antibiotics. For
upper urinary tract infection, antibiotics are given
for 7–14 days and for lower urinary tract infec-
tion for 3 days. Correction of any anatomical
abnormality of the urinary tract should follow.
quent causes of UTI in young girls and should be
treated with increasing intake of oral fluids, com-
plete bladder emptying with voiding training, and
avoidance of constipation. Children who have
bilateral renal abnormalities, impaired kidney
function, high blood pressure, and/or proteinuria
should receive monitoring and appropriate man-
agement by a specialist to slow the progression of
chronic kidney disease.
Suggested Reading
Belman BA, King RL, Kramer SA, editors. Clinical pediatric
urology. 4th ed. Boca Raton, FL: Martin Dunitz; 2002.
Kavoussi LR, Novick AC, Partin AW, Peters
CA. Campbell–Walsh urology. 10th ed. Philadelphia,
PA: Elsevier/Saunders; 2012.
Kliegman R, Stanton B, Geme JS, Schor N. Nelson
textbook of pediatrics. 20th ed. Philadelphia, PA:
Saunders/Elsevier; 2015.
 Vesicoureteric Reflux (VUR)
Vesicoureteric reflux (VUR) is retrograde flow of
urine from the bladder into the upper urinary
tract. If reflux extends in to the renal papilla, then
it is called intrarenal reflux.
Incidence
VUR is identified in 25% cases of antenatally
diagnosed hydronephrosis which brings its inci-
dence to 1 in 2000–5000 live births. VUR is a
heritable disorder with high incidence in siblings
of up to 50%.
Etiology
Mechanisms which prevent reflux across uretero-
vesical junction are as follows:
• Natural tone of ureter muscle allows mild pas-
sive closure of intramural ureter.
• Flap valve mechanism: As the bladder dis-
tends, there is progressive obliquity of the
intramural and submucosal ureter. Increase in
intravesical pressure causes compression of
this portion of ureter against the detrusor
muscle.
• Trigone also contracts during micturition
anchoring the ureteric orifice and preventing
its lateral displacement. This aids in flap valve
mechanism.
© Springer Nature Singapore Pte Ltd. 2018
S. Roy Choudhury, Pediatric Surgery, https://doi.org/10.1007/978-981-10-6304-6_54
54
• The ratio of the length of submucosal ureteral
tunnel to ureteric diameter is critical. This
ratio is 5:1 in children without VUR and is
less in patients with VUR.
Disruptions of these mechanisms result in
VUR. Possible reasons are:
• Abnormal distal origin of the ureteric bud
from the mesonephric duct results in ureteric
lateral ectopia and decreased length of submu-
cosal ureter.
• Abnormal development of trigone can also
result in lateral ectopia and poor anchorage of
ureteric orifice.
Both the absolute and relative lengths of the
submucosal tunnel tend to increase with age, thus
accounting for the spontaneous resolution of
VUR over time.
Classification
Primary and Secondary VUR
Primary VUR is due to an anatomical abnormal-
ity of the vesicoureteric junction.
Secondary VUR is due to increased intravesi-
cal pressure and abnormal bladder function in
association with neuropathic bladder and poste-
rior urethral valve.
327
328 54 Vesicoureteric Reflux (VUR)

VUR is known to run in families. In females it
usually presents late (3–7 years), is of low grade
(1–3), and is associated with dysfunctional void-
ing. In males it presents early (0–2 years) and is
usually of high grade (3–5), and anatomical fac-
tors are often associated.
including urinary frequency and urgency, pro-
longed voiding intervals, daytime wetting, peri-
neal/penile pain, holding maneuvers (posturing
to prevent wetting), and constipation/encopresis.
Investigations

Reflux Nephropathy Ultrasound may be normal in low-grade reflux.

Abnormal findings include:
Acquired renal scarring is the consequence of
symptomatic upper tract infection associated • Hydroureteronephrosis
with VUR and intrarenal reflux (IRR). Congenital • Scarred contracted kidney
nephropathy and renal dysplasia may contribute
a significant proportion of cases. MCU remains the gold standard investigation
Asymptomatic bacteriuria, a relatively com- for the diagnosis and evaluation of VUR
mon finding in older girls, does not give rise to (Figs. 54.1 and 54.2). MCU also helps in
renal scarring. To pose a risk of renal scarring, recognizing secondary VUR by detecting
­
urinary infection must be sufficiently severe to
give rise to symptomatic pyelonephritis.
Vesicoureteric reflux per se does not cause renal
damage, but when associated with urinary tract
infection, it results in acute pyelonephritis and
subsequently renal damage. Another factor is that
even in primary VUR, 18% of patients may have
voiding dysfunction. The incidence of UTI and
resolution of VUR are affected by the presence of
voiding dysfunction.

Presentation of VUR

Antenatal

Antenatally diagnosed bilateral hydroureterone-

phrosis merits postnatal follow-up and evaluation
for VUR.

Postnatal

• Symptomatic urinary tract infection, loin pain

due to pyelonephritis
• Hypertension
• Renal insufficiency

Symptoms indicative of bladder/bowel dys- Fig. 54.1 MCU: Bilateral VUR with trabeculated
function should be sought in the initial ­evaluation, bladder
Management of VUR
Fig. 54.2 MCU, Bilateral Grade V VUR
p­ osterior urethral valve or neurogenic bladder. It
also helps in grading of VUR.
Direct cystography—Radionuclide is instilled
in the bladder through an catheter or by direct
injection, catheter, and then the presence of reflux
is looked for under gamma camera. The advan-
tage of this investigation is limited radiation
exposure as compared to standard MCU and
increased duration for which bladder can be
observed for reflux. Hence this test can be used
for follow-up purposes. The disadvantages are
lack of information about grade of reflux and
bladder and urethral anatomy.
DMSA scan is very sensitive for detection
of scar associated with VUR and determina-
tion of differential renal function. VUR asso-
ciated with normal DMSA scan findings is
generally low-grade reflux with a high sponta-
neous resolution rate.
Indirect cystography with intravenous MAG3
may be used for follow-up investigation,
329
p­articularly in older children. Urodynamics is
mandatory in children with voiding dysfunction
and neurogenic bladder for diagnosis and moni-
toring of response to treatment.
Grades of VUR
International classification of vesicoureteral
reflux (Fig. 54.3):
Grade I: Ureter only.
Grade II: Ureter, pelvis, calices, no dilatation,
and normal calyceal fornices.
Grade III: Mild or moderate dilatation and/or
tortuosity of ureter and mild or moderate dilata-
tion of the pelvis, but no or slight blunting of the
fornices.
Grade IV: Moderate dilatation and/or tortuos-
ity of ureter and mild dilatation of renal pelvis
and calices and complete obliteration of sharp
angle of fornices but maintenance of papillary
impressions in majority of calices.
Grade V: Gross dilatation and tortuosity of
ureter, gross dilatation of renal pelvis and calices,
and papillary impressions are no longer visible in
majority of calices.
Management of VUR
Conservative (Medical Management)
• Continuous antibiotic prophylaxis: Antibiotics
are given at ¼–½ of full dose, e.g., trime-
thoprim 1–2 mg/kg/day as a single nighttime
dose. Nighttime dosing is preferred as there is
residual urine for prolonged bladder in the
night.
• Regular urine examination (microscopy and
culture sensitivity).
• Treatment of bladder dysfunction:
–– Regular voiding, double or triple voiding
–– Anticholinergics like oxybutynin if bladder
pressures are high
–– α-Blocker therapy
–– Biofeedback therapy to help in relaxation
of sphincter while voiding.
• Treatment of constipation if present.
330
Fig. 54.3 Grades of
vesicoureteric reflux
Follow-Up Management
General evaluation, including monitoring of
blood pressure, height, and weight, is recom-
mended annually.
The follow-up interval is determined by the
likelihood of resolution; for higher grades of
VUR, resolution is less likely, and therefore a
longer interval of follow-up is appropriate.
Generally yearly USG, MCU, and DMSA are
recommended.
I ndications for Surgical
Intervention
Functional
Symptomatic breakthrough urinary tract infec-
tion despite medical management.
Anatomical
–– VUR that occurs in conjunction with abnor-
malities such as paraureteric diverticulum or
ureteric duplication has a lower tendency to
resolve than uncomplicated primary reflux
and needs surgical intervention.
54 Vesicoureteric Reflux (VUR)
–– High-grade reflux (Grade IV or V) persisting
after 12 months of age requires surgical
interventions.
–– Persistent reflux that has attained full somatic
potential needs surgical intervention to pre-
vent acute pyelonephritis during pregnancy.
Surgical Management (Fig. 54.4)
Endoscopic injection treatment: Dextranomer-­
hyaluronic acid copolymer
Injection therapy (Deflux) is effective for low-
grade reflux (Grades I–III) (Fig. 54.5a, b). In this
procedure, an inert foreign material is injected at the
ureteral orifice to aid in correcting reflux by
improved backing of the intramural ureter, fixation
of ureterovesical junction at trigone, and by decreas-
ing the caliber of the distal ureter. Injection thepary
can bring high grade reflux to a low grade one.
Surgical: Ureteric reimplantation is (Cohen
transtrigonal, Politano-Leadbetter or thotopic,
Lich-Gregoir extravesical laparoscopic, or
robotic) effective for high-grade reflux (Grades
IV–V) with 95% success rate. Most of these
techniques rely on obtaining 4–5:1 ratio of the
submucosal tunnel to ureteral diameter. In
cases where the ureter is very dilated, the ure-
ter needs to be tapered.
Suggested Reading 331

Injection • Dextranomer/ hyaluronic acid coploymer

therapy injection (DEFLUX)

• Cohens reimplantation
Open surgery • Leadbetter-Polintano repair
• Lich-Gregoir repair and many more

• Extravesical laparoscopic/robotic uretreral

Minimally
reimplantation
Invasive Surgery
• Vesicoscopic ureteral reimplantation

Fig. 54.4 Summary of interventional management of VUR

a b

Fig. 54.5 (a) MCU: Left VUR. (b) Resolution of VUR following DEFLUX injection

Suggested Reading
Belman BA, King RL, Kramer SA, editors. Clinical pedi-
atric urology. 4th ed. Boca Raton, FL: Martin Dunitz;
2002.
Kavoussi LR, Novick AC, Partin AW, Peters
CAk. Campbell–Walsh urology. 10th ed. Philadelphia,
PA: Elsevier/Saunders; 2012.
 Neurogenic Bladder
Neurogenic or neuropathic bladder is defined as
abnormalities in bladder function secondary to
defects in its innervations. In addition to functional
voiding disturbances, it can adversely affect the
function of the kidneys, which is a serious concern.
 tiology of Childhood Neuropathic
E ron lesion.
Bladder
1. Spina bifida and tethered cord and
hydrocephalus
2. Spinal cord trauma, infarction, and tumors
3. Transverse myelitis
4. Tumors, sacrococcygeal teratoma, and
neuroblastoma
Physiology of Bladder Function
1. Filling and storage
2. Emptying
3. Voiding at will
 ypes of Neural Lesion with
T hold urine and urine is stored under high pres-
Neurogenic Bladder
Suprasacral cord lesions (contractile bladder)
where the conus medullaris is intact, voiding
occurs solely by detrusor contractions, almost
© Springer Nature Singapore Pte Ltd. 2018
S. Roy Choudhury, Pediatric Surgery, https://doi.org/10.1007/978-981-10-6304-6_55
55
always accompanied by detrusor sphincter
dyssynergia. These are upper motor neuron
lesions.
Sacral cord lesions (acontractile bladder)
where the conus medullaris is destroyed, voiding
occurs either by overflow or by raising intra-­
abdominal pressure. These are lower motor neu-
Intermediate bladder dysfunction is char-
acterized by a combination of detrusor hyper-
reflexia, typically generating low pressures,
and some degree of sphincteric incompetence.
Neuropathic bladder with meningomyelocele
is mostly intermediate in nature.
 econdary Upper Renal Tract
S
Complications
Such complications occur due to obstruction or
reflux and infection.
The type of neurogenic bladder can be classi-
fied as per Table 55.1
Disorders of storage include increased detru-
sor tone which limits the ability of bladder to
sures. This can be due to:
• Nonelastic bladder
• Hyperreflexia or overactive bladder
333
334 55 Neurogenic Bladder

Table 55.1 Summary

Disorder Cause Treatment
of pathophysiology and
management of Anticholinergics
neurogenic bladder Increased detrusor tone
Bladder augmentation

Disorder of storage α Sympathomimetic

Incompetent urethral drugs
closing mechanism
Bladder neck
reconstruction

Cholinergic drugs
Underactive detrusor Clean intermittent
catheterization
Disorder of
evacuation Clean intermittent
catheterization
Detrusor-sphincteric
dyssynergia α Sympatholytic
Biofeedback techniques

Another reason for the bladder inability to
hold urine can be poor outflow resistance at rest.
This can be due to:
• Incompetent bladder neck
• Incompetent external sphincter
Disorders of evacuation include inability of
the bladder to contract effectively to empty. This
can be due to:
• Areflexic bladder (no contraction)
• Hypoactive bladder (unsustained contraction)
implies either sphincteric competence or a blad-
der that is virtually empty due to gross sphinc-
teric incompetence.
Conus reflex (anocutaneous, glans bulbar):
Positive conus reflexes carry an excellent prog-
nosis for continence. Most patients with positive
reflexes become dry on clean intermittent cath-
eterization (CIC) alone or in combination with
an anticholinergic drug; few patients may need
augmentation cystoplasty or other procedures.
Sacral sensory sparing (perineal sensation)
is a favorable sign.

Another factor that results in failure of evacu-

Clinical Assessment in a Newborn
ation of bladder is detrusor sphincter dyssyner-
gia. In this disorder the bladder outlet fails to
Bladder expressibility, the absence of conus
relax while detrusor is contracting.
reflex, and no sacral sensory sparing are signs of
a neuropathic bladder.
Assessment

Bladder expressibility (expressing out urine by History

suprapubic pressure): An expressible bladder is
pathognomonic of bladder neuropathy with Detailed history of the following are taken:
some measure of sphincteric incompetence. A
• Bladder/bowel habits
bladder that cannot be expressed, by contrast,
Investigations 335

• Pattern of incontinence: 5. Urodynamics is used to identify the “unsafe”

–– Dry periods bladder, i.e., bladder which can result in
–– Stream renal damage. Intravesical pressure of more
–– Dribbling than 40 cm H2O can cause renal damage.
• Urinary tract infections Parameters recorded are:
• Bowel incontinence (a) Bladder capacity – normal bladder capac-
ity in ounces is:
i. <2 years age: Age (years) × 2 + 2
Examination ii. >2 years of age: Age (years) ÷ 2 + 6
Bladder volume can be calculated in ml by:
• Anthropometry and blood pressure For 0–2 years, capacity = weight (kg) × 7
• Spine examination For 2–12 years, capacity = (age + 2) × 30
• Abdomen: (b) Compliance can be assessed by pressure-­
–– Bladder palpable/compressible specific volumes, i.e., bladder volume at
–– Palpable fecaloma bladder pressures of 10, 20, and 30 cm
–– Renal lump H2O, respectively. In normal children,
• Perineum: more than 99% of bladder capacity can be
–– Bulbocavernosus reflex stored at 30 cm H2O pressure. Baseline
–– Anal tone pressures exceeding 20 cm H2O within
–– Ammoniacal dermatitis the physiological range of capacity on
Lower limb muscle charting and reflexes conventional slow-fill urodynamics
should be regarded as being “unsafe.”
(c) Leak point pressure: Pressure above
Investigations 40 cm H2O is dangerous for upper tract
(d) Postvoid residue
1. Ultrasonography (USG) of urinary tract: (e) Uroflowmetry
(a) Presence of renal pelvic/ureteral
dilatation
(b) Cortical thickness and renal length
(c) Bladder wall thickness
(d) Postvoid residue – very important to
determine
2. DMSA scan for renal functional assessment
and the presence of scars.
3. Micturating cystourethrography (MCUG):
(a) Bladder outline usually neurogenic blad-
der is elongated with sacculations giving
a “Christmas tree” appearance (Fig. 55.1).
(b) Vesicoureteric reflux.
(c) Bladder neck incompetence – funnel-­
shaped bladder neck during filling phase.
(d) Postvoid residue.
4. MRI of lumbosacral spine in children more
than 3 months of age (Fig. 55.2). In children
less than 3 months of age, spinal ultrasonogra- Fig. 55.1 MCU: Neurogenic bladder (sacculations and
phy can provide necessary information. Christmas tree appearance) with VUR
336 55 Neurogenic Bladder

a b c

Fig. 55.2 (a–c) Neurogenic bladder due to (a) spinal deformity and (b) tethered cord, (c) MRU showing small bladder
with VUR

Management (Table 55.1)

Goals:

1. To preserve renal function

2. To prevent progressive bladder damage
3. To achieve social continence by measures the
patient may practice independently.

General Principles of Management

High Postvoid Residue

It happens in cases of detrusor under activity or

dyssynergia. Clean intermittent catheterization
(CIC) is the most effective way. Children who
find it difficult to catheterize their urethra due to
neurological deficiency or sensate urethra can
be managed by creating an appendicovesicos-
tomy (Mitrofanoff procedure). Appendix is used
as a continent conduit between the bladder and
the skin (Fig. 55.3). Children with borderline
residues can be managed with double voiding
(voiding in quick succession).
Fig. 55.3 Mitrofanoff stoma
Detrusor Over Activity
Anticholinergics like oxybutynin and tolterodine
are the first line of treatment. Children who fail
this pharmacological treatment are candidates for
bladder augmentation. Recently botulinum toxin
injection (repetitive) has been reported to be
effective for such bladder.
Augmentation Cystoplasty
Poor Bladder Outlet Resistance
Sympathomimetics like pseudoephedrine or
phenylpropanolamine can be used, but efficacy is
limited. Surgical options include:
• Bladder neck reconstruction/tightening
• Bladder neck suspension using fascial slings
especially in females
• Artificial urethral sphincter
• Bladder neck closure with Mitrofanoff stoma
for CIC
Dyssynergia
CIC is helpful. Biofeedback can be used to
encourage children to relax their sphincter while
voiding.
Functional Voiding Disorders
Children without any obvious neurological lesion
may present with various urinary symptoms like
incontinence, urgency, frequency, recurrent uri-
nary tract infection, and constipation. Detailed
evaluation of the urinary tract with ultrasonogra-
phy should be done. Those children without any
obvious lesion may be managed with behavioral
therapy, voiding training with triple voiding (void-
ing in quick succession), detrusor relaxation with
anticholinergics, biofeedback to help sphincter
relaxation, and treatment of constipation.
 lean Intermittent Catheterization
C considered unsafe for the upper renal tract. A
(CIC) in Children
Indications
1. Neurogenic bladder
2. Following bladder augmentation
3. Continent urinary diversion
337
4. Myogenic failure of bladder like in some PUV
patients
Technique
CIC should be demonstrated and taught to the
patient/parent in the hospital or clinic which is to
be followed at home. Proper hygiene is to be
maintained with thorough hand wash and clean-
ing of the perineum/genitalia with soap and
water. For pediatric patients, size 6–10 Fr feeding
tubes can be used. Liberal amount of water-­
soluble jelly (2% lignocaine) is to be applied to
the part. Once the catheter is introduced into the
bladder, urine should be allowed to drain com-
pletely, which can be checked by pressing on the
lower abdomen before removing the catheter.
The catheter can be cleaned by rinsing in soap
and water and then dried under sunlight. It can be
stored in a dry stainless steel container. Catheter
can also be cleaned by boiling in water for
20 minutes before use. One catheter can be used
for 1–2 weeks; however, it should be discarded if
it gets dirty or any damage is noted. Generally
CIC is recommended for every 3–4 hourly inter-
val to keep the patient dry and to protect the
upper urinary tract from any pressure effect. For
patients with high risk of upper tract damage, a
nighttime indwelling catheter is recommended.
Augmentation Cystoplasty
This procedure is required for high pressure low
complaint and small volume bladder which is
segment of colon (colocystoplasty) or small
bowel (enterocystoplasty) can be used for this
purpose. To ensure complete emptying of the
bladder following augmentation, either CIC peri-
urethrally or catheterizable stoma (Mitrofanoff
principle) like appendicovesicostomy should be
considered.
338 55 Neurogenic Bladder

Suggested Reading treatment, long term followup. 1st ed. Heidelberg:

Springer; 2006.
Blaivas J, Chancellor MB, Weiss J, Verhaaren M. Atlas of
Esposito C, Guys JM, Gough D, Savanelli A, editors.
urodynamics. 2nd ed. Oxford: Blackwell; 2007.
Pediatric neurogenic bladder dysfunction: diagnosis,
 Posterior Urethral Valves
Posterior urethral valves (PUV) are the most
common cause of urinary obstruction in male.
This condition only affects the boys as the etiol-
ogy is congenital and related to opening of the
ejaculatory ducts into the posterior urethra. The
pathology often involves the urinary tract above
the obstruction that determines the outcome.
Incidence is 1 in 5000–8000 male live births.
Embryology and Types
Young classified posterior urethral valves into
three types: Type I is the usual type (>95%) that
arises from the distal end of the verumontanum
and extends distally and anteriorly to attach to
the anterior urethral wall. Type III valve is a cir-
cular membrane distal to the verumontanum
with central hole. Type II is described as leaflets
that extend upward from the verumontanum is
nonobstructive folds of mucosa and of no clini-
cal significance. Dewan and Ransley’s anatomi-
cal and endoscopic studies point to a single
configuration comprising an obliquely orientated
congenital obstructive posterior urethral mem-
brane (COPUM) with a variable sized eccentric
aperture located within it.
Type I: The valves occur due to abnormal
insertion of the ejaculatory ducts in the anterior
wall of the urethra. When these ducts migrate to
the posterior wall of the urogenital sinus, Type I
valve results. Type III valve results from failure
© Springer Nature Singapore Pte Ltd. 2018
S. Roy Choudhury, Pediatric Surgery, https://doi.org/10.1007/978-981-10-6304-6_56
56
of complete canalization of the membranous
urethra.
The valvular obstruction develops as a result
of abnormal embryogenesis at the confluence of
mesonephric ducts and the urogenital sinus
membrane.
Pathophysiology
Early embryonic obstruction leads to abnormali-
ties in bladder wall components with an increase
in the collagen element and renal dysplasia
(Fig. 56.1a, b). Obstruction in later gestation
results in the typical sequelae of chronic bladder
outflow obstruction and raised intravesical pres-
sure, without renal dysplasia. The long-term prog-
nosis in an individual with this disorder is
determined by a combination of the degree of
early abnormal development, involving the ure-
thra, bladder, kidneys, and ureters, and the severity
of late secondary effects of outflow obstruction on
the bladder and the upper urinary tract. Predicting
long-term outcomes is usually difficult.
Presentation
Fetal Diagnosis
Prenatal USG can suggest the diagnosis. The USG
features are distended hypertrophied b­ladder,
339
340 56 Posterior Urethral Valves

Fig. 56.1 (a) MCU:

a b
Neonatal PUV,
trabeculated bladder,
dilated posterior urethra,
left VUR. (b) VURD
(dysplastic kidney)

bilateral hydroureteronephrosis, oligohydramnios, Neonates

and a “keyhole” sign. When the condition is
detected early at 16–20 weeks, the prognosis is
likely to be poor, especially if oligohydramnios is
also present. In pregnancies which proceed to
term, a severely affected infant often demonstrates
features of Potter’s syndrome at birth (characteris-
tic Potter’s facies, skeletal “molding” deformities),
and death supervenes in the early newborn period
as a result of pulmonary hypoplasia. In the absence
of second trimester oligohydramnios, the func-
tional prognosis is linked to the severity of dilata-
tion and other prognostic features on second
trimester ultrasound. In cases where dilatation
develops later in gestation, the prognosis is gener-
ally good. In addition to dilatation and renal dys-
plasia (bright kidneys), other findings detected by
fetal ultrasound include lung hypoplasia, urinary
ascites, and perinephric urinomas.
Symptoms are due to bladder outlet obstruction
and renal failure. Listlessness, poor feeding, irrita-
bility, and failure to thrive are common. The uri-
nary stream is usually poor. The bladder is palpable
in most instances; the kidneys may also be palpa-
ble. Urinoma and urinary ascites occur as an occa-
sional complication due to leakage from the
kidney.
Infants and Older Children
They present with urinary symptoms like poor
stream, dribbling, urinary tract infection, enure-
sis, and voiding dysfunction. Additionally fea-
tures due to renal failure may be present.
Management
Fig. 56.2 MCU showing PUV (arrow) with trabeculated bladder
Investigations
Ultrasonography (USG) is the initial investigation
of choice. USG features include bilateral upper
tract dilatation, thickening of the bladder wall,
with significant postvoid residual urine, and dilata-
tion of the posterior urethra. Occasional perirenal
urinoma or urinary ascites may also be detected.
Micturating cystourethrography (MCU) is the
investigation of choice for the definitive diagno-
sis (Fig. 56.2). It should be performed with a
strict aseptic precaution ensuring a sterile urine
culture. MCU will show a dilated posterior ure-
thra; often a defect caused by the valve can also
be appreciated in the posterior urethra. The blad-
der is irregular in outline with trabeculations and
sacculations (Fig. 56.2). Vesicoureteric reflux
may also be present. The urinary stream is poor,
and there is significant postvoid residue. Bladder
neck hypertrophy may also be present.
Other conditions closely mimicking PUV are
bladder diverticulum in neonates causing urethral
341
obstruction (Fig. 56.3a, b), stone, and some ure-
thral anomalies (Fig. 56.4a, b).
Management (Table 56.1)
Fetal Management (see chapter 4,
Antenatal Diagnosis and Fetal Therapy)
The different options are as follows:
1. Termination of pregnancy: Early detection of
severe urinary tract dilatation, echogenic kid-
neys, and early oligohydramnios (before
20 weeks) indicate poor outcome.
2. Preterm delivery: For rapidly progressing
late-onset urinary tract dilatation (after
24 weeks) with decreasing amniotic fluid vol-
ume may precipitate fetal distress.
3. Vesicoamniotic shunt: In the presence of early
obstruction and preserved renal function, a
vesicoamniotic shunt may be considered. It
helps in restoring amniotic fluid volume and
342 56 Posterior Urethral Valves

Fig. 56.3 (a–c) MCU: a b

Neonatal bladder
diverticulum causing
urethral obstruction

Fig. 56.4 (a) Urethral

a b
stone. (b). Congenital
anterior urethral
diverticulum with
obstruction

therefore lung development and prevents pul- should also be performed within 24–72 h to con-
monary hypoplasia. firm the diagnosis of PUV. The steps of manage-
ment include:

Neonatal Treatment 1. A catheter drainage of the bladder, achieving

optimal stabilization of renal function, correc-
Newborn with suspected PUV should undergo tion of fluid, and electrolyte imbalance fol-
ultrasound postnatally within 24–48 h. MCU lowed by valve ablation.
Assessment of Renal Function
2. Endoscopic valve ablation in stable patients:
A resectoscope is inserted in the posterior ure-
thra, and the valve is incised at 5, 7, and 12
o’clock (Fig. 56.5) positions.
3. Patients in whom valve ablation is not feasible
or the child with severe urosepsis, persistent
acidosis and markedly impaired renal func-
tion may benefit from temporary urinary
diversion. The choice of the diversion can be a
vesicostomy or a supravesical diversion.
Definite advantages of one over the other have
not been proven.
4. Vesicostomy can be considered if there is
nonavailability of instrument and in very
small birth weight babies. In cases with per-
sistent acidosis and urosepsis, those who do
not respond to adequate urinary catheter
drainage, the benefit from vesicostomy may
be limited as the ureters are dilated and tor-
tuous. These ureters may be the reason for
Fig. 56.5 Endoscopic
view of incision of
posterior urethral valve Endoscopic view of incision of PUV
343
lack of response to catheter drainage and
can only be addressed by supravesical
drainage (ureterostomy).
5. Supravesical diversion (ureterostomy, pyelos-
tomy). They are indicated if initial catheter
drainage shows no improvement or further
deterioration of renal function. The only con-
cern with supravesical drainage is the lack of
bladder cycling because of which the bladder
may not grow adequately. Refluxing ureteros-
tomy (ureterostomy of the refluxing poorly
functioning side) maximizes drainage from
the ipsilateral kidney and at the same time
maintains bladder cycling.
Assessment of Renal Function
A baseline DMSA scan and glomerular filtration
rate (GFR) study should be performed.
Bladder neck
Verumontanum
Posterior urethral
valve
Cold knife/hook
344
Assessment of Bladder Function
Urodynamic study (UDS) of the bladder is indi-
cated to determine the bladder capacity and com-
pliance of bladder so that medical therapy if
indicated can be initiated.
PUV needs long-term follow-up, as relief of
the obstruction by incision of the valves is not the
end of management. Persistent urinary tract dila-
tation along with noncompliant bladder (valve
bladder syndrome) and VUR may be present
which may compromise renal function in the
long term. Adequate management of voiding dys-
function and UTI needs the use of antibiotics,
anticholinergics (oxybutynin), alpha sympathetic
blockers like prazosin, or CIC (clean intermittent
catheterization).
Predictors of Poor Outcome
Prenatal
1. Maternal oligohydramnios, regardless of the
gestational age
2. Early detection on prenatal ultrasound of
“bright” echogenic kidneys and pelvicalyceal
dilatation
a b
Fig. 56.6 (a, b) PUV
before and after
endoscopic valve
ablation; note smooth-
walled bladder and
normal posterior urethra
56 Posterior Urethral Valves
Postnatal
1. Presentation in the first 12 months of life (if
undetected prenatally). GFR at 1 year of
age: higher values associated with better
outcome
2. Proteinuria: if present is associated with poor
outcome
3. The nadir of serum creatinine achieved at 1 year:
>1 mg/dl is associated with poor outcome
4. Bilateral vesicoureteric reflux
5. Impaired continence at and above 5 years of
age
Other mechanisms of renal protection are uri-
nary ascites, perinephric urinoma, bladder
diverticulum, and patent urachus.
Indicators of Good Renal Function
1. Presentation in later childhood.
2. Protection of the upper tracts by a “pop-off”
phenomenon. Of these, the most common is
unilateral high-grade vesicoureteric reflux in
which the affected ipsilateral kidney has neg-
ligible or zero function where the contralateral
kidney is normal called vesicoureteric reflux
disease (VURD syndrome).
Prognosis and Outcome 345

Fig. 56.7 MCU before a b

and after valve ablation
(a) PUV with VUR (b)
smooth bladder,
disappearance of VUR

There is some protective value of the “pop-­off”

phenomenon (vesicoureteric reflux). Other less  ladder Management in PUV
B
common forms of “pop-off” mechanism include Patients
urinary ascites, perinephric urinoma, and a large
bladder diverticulum. Vesicoureteric reflux Bladder dysfunction occurs in 50-75% of patients
resolves in 30–60% of cases following valve abla-with PUV. The urodynamic patterns are: a.
tion (Fig. 56.6a, b), one third remains stable, and
decreased compliance/small capacity bladder in
one third requires intervention to prevent further
infants, b. detrussor hyper-reflexia in older chil-
deterioration. Resolution of VUR is less likely in
dren and c. myogenic failure in post-pubertal
the presence of bladder dysfunction or when func-boys. Bladder dysfunction in PUV patients is
tion in the affected renal unit is poor (Fig. 56.7).
managed with anticholinergics like oxybutynin,
tolterodine or others and clean intermittent cath-
eterization. A small contractile bladder with
Follow-Up Management severe upper tract dilatation despite medical
treatment should be treated with bladder
PUV patients should be under close and long-­ augmentation.
term follow-up. The following parameters should
be closely monitored during each visit:
Prognosis and Outcome
1. Height and weight
2. Blood pressure Bladder dysfunction continues in two thirds
3. Urinary tract ultrasonography of the boys despite valve treatment. Untreated
4. Urine protein bladder will lead to progressive upper tract
5. Serum creatinine and electrolytes deterioration leading to renal failure (“valve
bladder”). The combination of renal dyspla-
Tests done as indicated are: sia, bladder dysfunction, obstruction, and
infection leads to chronic renal failure in late
1. Ultrasound KUB childhood or adult life in one third to half of
2. MCU the patients.
3. Isotope renography (DMSA)
4. Urodynamics
346 56 Posterior Urethral Valves

Table 56.1 Algorithm of management of PUV

New born with PUV

Resisucitate- ABG,
CBC, SE, KFT, urine C/S,
USG KUB and VCUG
Uretheral catherization
Sequential renal
function assessment

No improvement in serum creatnine on Reduction of serum

urinary catheter drainage for 2 weeks creatnine baby stable
Increased upper tract dilatation
Persistent infection

Supravesical diversion Stable good weight baby Premature remature small

ureterostomy/pyelostomy
KFT normal
Valve ablation with
closure of diversion
Neonatal resectohscope available
Primary valve ablation
Follow up
Somatic growth,
BP, urine analysis, USG, MCUG
Radionucliotide imaging
infant/inapropriate
or unavailable instruments/massive
urine leakage/massive VU Reflux
Blocksom’s Cutaneous vesicostomy
OR
Suprapubic drainage
KFT normal
Valve ablation with
closure of vesicostomy

Suggested Reading
Belman BA, King RL, Kramer SA, editors. Clinical pedi-
atric urology. 4th ed. Boca Raton, FL: Martin Dunitz;
2002.
Kavoussi LR, Novick AC, Partin AW, Peters
CA. Campbell–Walsh urology. 10th ed. Philadelphia,
PA: Elsevier Saunders; 2012.
 Urinary Incontinence in Children,
Ectopic Ureter and Ureterocele
Urinary incontinence (loss of control) or enuresis
occurs due to diverse causes in children. The
incontinence may be nocturnal, diurnal, or both
below.
 auses of Urinary Incontinence
C entirely by day, or both by day and night?
In Children
Organic causes of urinary incontinence in
children:
Urinary infection (intermittent leakage)
Neuropathic (continuous/intermittent leakage)
Bladder outflow obstruction (intermittent leakage)
Structural (continuous leakage)
Exstrophy/epispadias
Ectopic ureter (girls)
Congenital short urethra (girls), wide bladder
neck seen with congenital pouch colon
Urogenital sinus anomaly (girls)
Functional enuresis
Detrusor instability (dysfunctional voiding)
Primary nocturnal enuresis
© Springer Nature Singapore Pte Ltd. 2018
S. Roy Choudhury, Pediatric Surgery, https://doi.org/10.1007/978-981-10-6304-6_57
57
Diagnosis
A properly taken history aids in making the
diagnosis:
1. Does the wetting occur entirely by night,
Only nighttime wetting is due to functional
nocturnal enuresis.
2. Is the problem primary (i.e., lifelong) or sec-
ondary (i.e., of onset following toilet training
and after a period when the child was dry)?
Secondary problems are due to dysfunctional
voiding disorder.
3. In the case of daytime wetting, does this occur
continuously or only intermittently?
Continuous day and night wetting since birth
suggests a structural anomaly like ectopic ureter,
exstrophy epispadias, or incompetent bladder
neck. In girls continuous incontinence with nor-
mal voiding strongly suggests the possibility of
an ectopic ureter.
A thorough physical examination should be
done particularly looking for any anatomical
defect, e.g., spine and neurological examination.
347
348 57 Urinary Incontinence in Children

Investigations tract dilatation, bladder wall thickening, and

presence of significant residual urine indicat-
1. Urine culture and sensitivity to rule out uri- ing neurogenic bladder.
nary tract infection. 3. Intravenous urography (IVU) or MR urogra-
2. Ultrasound: significant findings may include phy (Figs. 57.1a and 57.2a, c).
duplication anomalies (hydronephrosis, 4. X-ray and MRI spine for suspected neuro-
hydroureter, and dysplastic kidney), upper genic bladder dysfunction.

Fig. 57.1 (a) IVU

a b
showing nonfunctioning
kidney on the right side
in a girl child with
urinary incontinence
(right ectopic ureter).
(b) DMSA scan showing
no function on the right
side

a b c

Fig. 57.2 (a–c) Dysplastic kidney with ectopic ureter (left). (a) IVU showing poorly functioning left kidney in a girl
with incontinence of urine, (b) Retrograde urogram through ectopic ureteric opening, (c) Operative specimen of left
nephroureterectomy, note small dysplastic kidney
Ectopic Ureters and Ureteroceles
5. Nuclear renal scan (Fig. 57.1b).
6. Urodynamic examination for suspected neu-
rogenic bladder.
7. Examination under anesthesia and cystoure-
throscopy (Fig. 57.3).
8. Laparoscopy occasionally helps in localiza-
tion of ectopic kidney.
Fig. 57.3 Ectopic ureteric opening in the vulva
a b
Fig. 57.4 Y duplication with dysplastic kidney with VUR. (a) MCU, (b) intraoperative appearance
349
Ectopic Ureters and Ureteroceles
Ureteral duplication anomalies are common
abnormalities and have a reported incidence of
0.7%. Most cases of duplications are asymptom-
atic and require no treatment. In some cases, they
result in ectopic ureters and ureteroceles which
often require surgical intervention. Usually the
upper moiety of the duplex system is involved.
Duplications can be classified as complete if
both the ureters open separately in the genitouri-
nary tract. If the ureters combine distally and
open through a common orifice, then incomplete
duplication results (Fig. 57.4a, b).
Ectopic ureter: The ureter opens at an ectopic
caudal location like urethra in males or genital
tract in females. They are two to three times more
common in females. In females, majority of ecto-
pic ureters are associated with duplex system
(duplication), whereas in males majority are
associated with a single system (Figs. 57.4, 57.5,
57.6, and 57.7). There are two distinct forms of
EU, one draining a duplex kidney and the other
connected to a single kidney, the latter being
termed single-system ectopic ureter (SSEU)
(Figs. 57.1a, b and 57.2a, b).
350
Fig. 57.5 Upper pole duplex with ectopic ureter (hemi-
nephroureterectomy operation)
Fig. 57.6 Duplex with ectopic ureter (IVP)
57 Urinary Incontinence in Children
Ureterocele
Ureterocele is an abnormal dilatation of the intra-
vesical terminal ureter. Like ectopic ureters, they
are more common in females (4:1). In females,
majority of them are associated with duplex sys-
tem (duplication), whereas in males majority are
associated with a single system. They are classi-
fied as intravesical—confined within the bladder
(Fig. 57.8a, b) or ectopic—where some portion
extends into the bladder neck or the urethra
(Fig. 57.8c). Cecoureterocele is a variety of ecto-
pic ureterocele which extends into the urethra
with its orifice into the bladder.
Embryology
Ureteral duplication results when the ureteral bud
divides after origin from mesonephric duct
(incomplete duplication) or two separate ureteric
buds arise from the mesonephric duct (complete
duplication).
Ectopic ureter: Caudal ectopia results if the
ureteric bud arises more proximal than usual
location. In males the mesonephric duct is incor-
porated in the proximal urethra and bladder tri-
gone, so the ectopic ureter opens proximal to
urethral sphincter; hence, there is no inconti-
nence. In females, the mesonephric remnants are
in close relation with anterolateral wall of the
vagina. Therefore in females the ectopic ureter
may open in the vagina and result in
incontinence.
Ureterocele: Etiology remains unclear, but
abnormal muscular development of terminal ure-
ter has been implicated.
Presentation
Both these conditions may present with recurrent
urinary tract infections, failure to thrive, and
abdominal mass and pain.
Ectopic ureter in males can present as urgency,
frequency, epididymo-orchitis, and pelvic pain.
Females may present with continuous dribbling
of urine with normal voiding pattern and vaginal
discharge.
Ectopic Ureters and Ureteroceles 351

Fig. 57.7 Duplex with

a b
upper pole ectopic
ureter. (a) Sling on
normal lower pole
ureter, (b) dilated upper
pole ectopic ureter

a b c

Fig. 57.8 (a) MCU showing ureterocele (Filling defect). (b) Intraoperative appearance of intravesical ureterocele. (c)
Prolapsed ureterocele in a newborn female (arrow)

Ureterocele may present with bladder outlet
obstruction in either sex or as an intralabial mass
in females (ectopic or cecoureterocele)
(Fig. 57.8c).
In ectopic ureter, the ureter can often be traced
very low in pelvis suggesting ectopic insertion.
Ureterocele can be readily identified in a par-
tially filled bladder as a thin-walled cystic protru-
sion in the posterolateral wall of the bladder.

Investigations
Imaging modalities include USG, IVU, MCU,
MR urography, and nuclear imaging (Figs. 57.1,
57.2, and 57.6)
Ultrasonography
Both ureterocele and ectopic ureter may present
with hydroureteronephrosis. The duplex system
may also be apparent with affected upper moiety
especially in females.
Micturating Cystourethrogram
Ureterocele may be seen as a filling defect posi-
tioned in the area of trigone. If images are taken
once the bladder has been filled significantly, then
ureterocele may be effaced and in some cases be
everted, giving appearance of a diverticulum.
Presence of ipsilateral reflux in the lower pole sys-
tem is often seen because of associated lateral
ectopia and distortion of the trigone.
Ectopic ureters often demonstrate VUR. VUR
may also be detected in lower pole system.
352
Intravenous Urography
It is a useful investigation in these cases espe-
cially if the pathology is not defined by USG,
MCU, and renogram.
Ureterocele with functioning renal moiety
may demonstrate contrast filled “cobra head” at
ureterovesical junction. If the associated moiety
is nonfunctioning, then the ureterocele may
appear as a filling defect when the bladder fills by
contrast secretion from the other side.
Ectopic ureter with a functioning associated
moiety may be seen to extend very low down in
the pelvis and open in proximal urethra in males
or genital tract in females.
Few findings may suggest presence of a non-
functioning (occult) duplex with an ectopic ure-
ter like “dropping lily sign” (inferior and lateral
displacement of lower pole by the dilated upper
pole system), lateral displacement of lower sys-
tem ureter, and missing calyx.
Renal Scintigraphy
It helps in determining if any functioning renal
parenchyma is present and guides treatment
planning.
Fig. 57.9 Transureteroureterostomy (upper
pole duplex ureter)
57 Urinary Incontinence in Children
CT Urography and MRI Urography
They help in defining anatomy especially when
the involved renal moiety is nonfunctioning.
Examination under anesthesia and cysto-
urethrography are useful in the diagnosis and
localization of ectopic ureter (Fig. 57.3).
Treatment (Table 57.1)
Surgical
EU associated with a dysplastic nonfunctioning
kidney requires nephroureterectomy (hemi or
complete depending on the anatomy) (Figs. 57.4,
57.5, and 57.7). EU with a functioning kidney
merits renal preservation with either ureteric
reimplantation or transureteroureterostomy
(Fig. 57.9). The result of surgery is very gratify-
ing in unilateral ectopic ureter as the chance to
achieve complete dryness is very high. The same
is not true for bilateral cases due to abnormal tri-
gone, small bladder, and insufficiency of the
bladder neck (Fig. 57.10).
Management of other causes of urinary incon-
tinence is discussed in relevant chapters.
Treatment 353

Fig. 57.10 Bilateral

ectopic ureter opening in
the vagina, note small
bladder

Table 57.1 Management algorithm of ectopic ureter and ureterocele

Ectopic ureter
Minimal or
Nonfunctioning Salvagable function
moiety/ kidney

Duplex system Single system

Single system
Upper pole Ureteric Duplex system
Nephrouretectomy
heninephrectomy reimplantation

No associated VUR
Associated VUR
Ureteropyelostomy
Common sheath
Distal reimplantation
ureteroureterostomy

Ureterocele
Single system Duplex system
minimal or non
Functioning Minimal or non functioning
functioning Salvagable renal function
kidney moiety
kidney

Selective
No VUR
cystoscopic
Nephrectomy (contralateral or VUR present No VUR VUR present
punctre of
ipsilateral)
ureterocele

ureterocele
Heminephrectomy In addition to Uretropyelostomy
If VUR appears then
heminephrectomy,
excision,
ureterocele excision, alone issufficient or
ureterocele ureteroureterosto
detrusor
detrusor repair and
ureteric excision and my with subtotal repair and
reimplantation is ureteric excision of ureter ureteric
needed reimplantation reimplantation
may be needed is needed
354 57 Urinary Incontinence in Children

Suggested Reading
Belman BA, King RL, Kramer SA, editors. Clinical pediatric
urology. 4th ed. Boca Raton, FL: Martin Dunitz; 2002.
Choudhury SR, Chadha R, Bagga D, Puri A, Debnath
PR. Spectrum of ectopic ureters in children. Pediatr
Surg Int. 2008;24:819–23.
Kavoussi LR, Novick AC, Partin AW, Peters
CA. Campbell–Walsh urology. 10th ed. Philadelphia,
PA: Elsevier/Saunders; 2012.
 Disorders of Sexual Differentiation
The term “disorders of sex development (DSD)”
represents a group of congenital conditions
with atypical development of chromosomal,
gonadal, or anatomical sex. DSD has replaced
the old terminology of intersex disorders or
pseudohermaphroditism.
Development of Gonads and
Internal Genitalia (Table 58.1)
Urogenital ridge forms on the ventromedial
aspect of intermediate mesoderm and comprises
of the undifferentiated gonad, mesonephros,
Wolffian ducts (mesonephric duct), and Mullerian
ducts (paramesonephric duct). Primitive germ
cells migrate to this ridge, and the differentiation
of the gonad to testis or ovary begins.
The internal genital development depends on
the ipsilateral gonad, and external genital devel-
opment depends on the presence of testosterone
and dihydrotestosterone (DHT). In the absence of
testis, the Mullerian structures will develop into
the uterus and fallopian tube, while the Wolffian
ducts will involute. In the absence of androgens,
the external genitalia develop into female-like
genitalia which is the default pathway. However,
if a testis is present, the internal genital duct will
develop along male lines: an epididymis, vas, and
seminal vesicle will form, whereas the Mullerian
structures will involute in the presence of
© Springer Nature Singapore Pte Ltd. 2018
S. Roy Choudhury, Pediatric Surgery, https://doi.org/10.1007/978-981-10-6304-6_58
58
Mullerian inhibiting substance. In the presence of
androgens, the external genitalia will develop into
male-like genitalia. Besides the ovary and testis,
patients with DSD can also have two other types
of gonads: the streak gonad and the ovotestis.
Abnormalities in testosterone or DHT synthesis
(due to enzyme 5α-reductase deficiency) affect
the development of external genitalia and may
result in hypospadias, due to failure of the urethra
to tubularize to the end of the glans of penis.
Common Types of DSDs
• 46,XX DSD (female pseudohermaphroditism)
• 46,XY DSD (male pseudohermaphroditism)
• Ovotesticular DSD (true hermaphroditism)
• Disorders of gonadal differentiation (includes
syndromes of gonadal dysgenesis)
46,XX DSD (Female
Pseudohermaphroditism)
It occurs because of exposure of the female
fetus to androgens. The causes are:
• Congenital adrenal hyperplasia (most com-
mon) (Figs. 58.1 and 58.2)
• Placental aromatase deficiency and maternal
ovarian and adrenal tumors
355
356 58 Disorders of Sexual Differentiation

Table 58.1 Normal development of gonads and genitalia

Structure Male Female
Gonad Testis (needs presence of SRY gene present on Y Ovary was considered default pathway
chromosome) in the absence of Y chromosome, but
recent evidence suggests need of both X
chromosomes for normal development
Internal genitalia Mullerian ducts involutes due to secretion of MIS Wolffian ducts involutes in absence of
(Mullerian inhibiting substance) androgens and Mullerian ducts develop
Wolffian ducts develop in the presence of in the absence of MIS
testosterone
Both MIS and testosterone are secreted by testis
External genitalia Develop in the presence of dihydrotestosterone Default pathway in the absence of
(DHT) androgens

Functional defect in any of the five enzymatic steps required for cortisol synthesis,
most commonly 21-hydroxylase (involved in 90-95% of cases) and 11-hydroxylase
level

Decreased production of Accumulation of virilizing

adrenal hormones precursors

Clitoral hypertrophy
↓Glucocorticoids ↓Mineralocorticoids
Urogenital sinus
(common opening of urethra and
vagina)
Labioscrotal folds are enlarged
Increased Internal genitalia is normal as it has
↓ Na, ↑ K
secretion of already formed before fetal adrenal
ACTH and MSH
Salt wasting
glands starts functioning
ABSENT GONADS ON PALPATION

ACTH further
increases
steroidogenesis
and MSH
casuses
increased
pigmentation

Fig. 58.1 Pathogenesis and clinical features of congenital adrenal hyperplasia. ACTH adrenocoticotropic hormone,
MSH melanocyte-stimulating hormone
46,XX DSD (Female Pseudohermaphroditism)
• Exogenous androgens administered during
pregnancy
Congenital Adrenal Hyperplasia
Congenital adrenal hyperplasia (CAH) is the
most common type of 46,XX DSD in which
there is an enzymatic block in synthesis of adre-
nal corticosteroids and mineralocorticoids. The
adrenals are thereby stimulated by increased
ACTH. This results in accumulation of the pre-
cursors proximal to the enzymatic defect which
are then converted to potent androgens which
causes the typical virilization seen in affected
females. The most common enzyme deficiency
Fig. 58.2 Congenital adrenal hyperplasia, virilized
female
a b
Fig. 58.3 Genitogram
in a CAH patient. (a)
Vagina with cervical
impression (arrow). (b)
dye filling of cervical
canal (arrow)
357
is 21-­ hydroxylase (90–95%) followed by
11-hydroxylase. This is an autosomal recessive
genetic disorder.
The phenotypic picture varies from mild clito-
ral enlargement alone to complete masculiniza-
tion with urethral meatus at the tip of the phallus
(Fig. 58.2). Prenatal diagnosis is based on finding
the disease gene on the short arm of chromosome
6. The diagnosis can be confirmed by chorionic
villous sampling. Antenatal management in a
fetus suspected with congenital disorder starts
with administration of dexamethasone from 5 to
6 weeks of gestation until the results of the
genetic testing are known. Dexamethasone is
stopped in affected males and unaffected females.
In affected female the drug is continued till term.
After birth, management consists of replacement
steroid therapy. A genitogram helps in the identi-
fication of the vagina (Fig. 58.3a, b) and in deter-
mining the length of the common urogenital
sinus which is confirmed on panendoscopy (cys-
tourethroscopy and vaginoscopy). For masculin-
ized genitalia, a cutback or flap vaginoplasty (for
low vaginal entry) with clitoral recession at
6–18 months of age is recommended.
Children with high vaginal entry proximal to the
urethral external sphincter will require more
extensive reconstruction by either total urogeni-
tal sinus mobilization (TUM) or bowel vagino-
plasty. Long-term surgical results of female
children show a­dequate sexual identification,
358 58 Disorders of Sexual Differentiation

reproduction, intellectual functioning, and accept-
able genitalia.
46,XY DSD (Male
Pseudohermaphroditism)
 ndrogen Insensitivity Syndrome
A Complete androgen insensitivity syndrome
(AIS), Male DSD (Testicular
Feminization Syndrome)
AIS patients are genetic male with 46,XY karyo-
type with external genitalia having the female-
like appearance. They are inherited as X-linked
recessive condition. AIS is mainly of two variet-
ies: complete and partial (Fig. 58.4). In the com-
plete variety, the external genitalia looks like
normal female.
Partial androgen insensitivity results when
there is partial resistance at the level of androgen
receptor. This results in suboptimal response to
androgens which affects the development of
external genitalia and also interferes with descent
of the testis. Patients with this disorder present
with severe hypospadias often associated with
undescended testis (Fig. 58.5). A close differen-
tial diagnosis is 5α-reductase type 2 deficiency
(Fig. 58.4). They can be reared as male with hor-
monal replacement therapy (androgens).
(CAIS) is indicated when the external genitalia is
that of a normal female. However they are
brought to notice by amenorrhea at puberty or a
hernia containing testis in the hernial sac.
Evaluation should include karyotype, hormonal
assays, pelvic ultrasound, urethrovaginogram, and
gonadal biopsy in some cases. These patients will
never menstruate or bear children as they do not
have a uterus or fallopian tube due to the presence
of MIS from the testis. Malignant degeneration
(germ cell tumors) of the gonads is increased (20–
30%) especially with ­intra-­abdominal testis. Early
gonadectomy is advised to decrease the possible
development of malignancy, avoid the latter

46 XY DSD (Male pseudohermaphroditism)

(Mullerian Structures are always absent due to MIS)

Decreased
production of Insensitive androgen receptors
Testosterone due to 5α reductase type 2
enzymatic defects deficiency
↓Dihydrotestosterone
-17-hydroxylase Complete insenstivity (DHT)
- 3β-hydroxysteroid Partial Insenstivity
(Testicular
dehydrogenase (Reifenstein
feminization
-17βhydroxysteroid syndrome)
syndrome)
dehydrogenase severe hypospadias
with undescended
Bilateral symmetric testis
Bilateral symmetric undescended small Penile growth at
Normal female
undescended small testis puberty due to
external genitalia
testis production of DHT
Severe hypospadias
Severe hypospadias from 5α reductase
type 2

Fig. 58.4 Types and clinical features of various types of 46,XY DSD. MIS Mullerian inhibiting substance
Disorders of Gonadal Dysgenesis 359

Fig. 58.5 Male DSD.

a b
(a) Micropenis. (b)
Perineal hypospadias

p­ sychological trauma to the older child, and elimi-

nate risk of losing the patient during follow-up.
However, delayed orchidectomy has the advantage
of pubertal breast development due to production
of estrogen from aromatization of high level of tes-
tosterone. They require hormonal replacement
therapy (estrogen) later in life. Vaginal reconstruc-
tion is planned when the patient wishes to be sexu-
ally active.
46 XX male is a rare disorder with 90% being
SRY positive. They usually present with small
testis and infertility. They are managed with tes-
tosterone replacement therapy.
Fig. 58.6 Mixed gonadal dysgenesis (note asymmetric
gonad)

Disorders of Gonadal Dysgenesis

Mixed Gonadal Dysgenesis
Mixed gonadal dysgenesis (MGD) is an intersex
disorder which has unilateral streak gonad and a
dysgenetic testis on the other side that is often
palpable. The chromosomal abnormality is
45,XO/46,XY mosaicism. On physical examina-
tion, the patients have ambiguous genitalia and
small phallus with significant chordee. Often the
urethral and vaginal opening can be seen sepa-
rately; otherwise a urogenital sinus may be pres-
ent with a wide meatus. The dysgenetic testis is
often palpable on one side; the other side being a
streak is never palpable (Fig. 58.6). The dysge-
netic testis might show prepubertal tubules lined
by a few spermatogonia and immature Sertoli
cells. Female gender assignment is usually pre-
ferred, but male assignment is an alternative in
instances of extreme virilization. Dysgenetic
gonads with the presence of a Y chromosome or
a translocated fragment have a significant risk of
developing malignant gonadoblastoma (though
seminoma and dysgerminoma can occur). Hence
bilateral gonadectomy should be done at an early
age. The risk is very high in children with pure
gonadal dysgenesis. The child with female gen-
der assignment will need clitoral recession and
vaginoplasty in early infancy. If male gender is
assigned, then various types of hypospadias
repair can be done, gonads replaced with prosthe-
ses, the pre-penile scrotum reconstructed and
Mullerian structures removed. They should be
screened for Wilms tumor.
Swyer syndrome is 46 XY pure gonadal dys-
genesis in externally female with bilateral streak
gonads. Treatment is early gonadectomy with
hormonal replacement therapy.
360
 votesticular DSD (True
O The presentation is often because of hernia or
Hermaphrodite)
These patients have both testicular and ovarian tis-
sue. The most common configuration is of ovary on
one side and ovotestis on the other side. The ovo-
testis is usually palpable, and the degree of descent
is proportional to the testicular tissue in the gonad.
Other gonadal combinations are also possible. The
Mullerian structures on the side of the testicular tis-
sue are regressed but are retained on the other side
along with a midline uterus. A urogenital sinus
defect is present along with hypertrophied clitoris.
The phenotype shifts more toward male phenotype
when the quantity of testicular tissue increases. The
karyotype is usually 46,XX (74%), but 46,XX/XY
(19%) and 46,XY (6%) are also seen. Biopsy of an
ovotestis should be performed by opening the
gonad widely and taking a deep biopsy as the tes-
ticular tissue is central and ovarian tissue is polar.
For true hermaphrodites raised as males, repair of
hypospadias, orchidopexy, and removal of ovarian
tissue are done. Hermaphrodites raised as females
undergo clitoroplasty, vaginoplasty, and removal of
testicular tissue. Testicular tumors like seminomas,
gonadoblastomas, and dysgerminomas have been
described
Persistent Mullerian Duct Syndrome
This disorder is characterized by the presence of
Mullerian structures (uterus, fallopian tubules,
and upper 2/3 of the vagina) in a phenotypic and
genetic male. This disorder is caused by the
absence of Mullerian inhibiting substance (MIS)
or insensitivity of its receptors. It is an autosomal
recessive disorder. The patients may present in
the following three forms:
• Female form: Bilateral undescended gonads
located in the pelvis on either side in the broad
ligament.
• Hernia uteri inguinalis: One testis is located in
the hernial sac along with the fallopian tubules
and/or uterus.
• Transverse testicular ectopia: Both the testes
along with Mullerian remnants are located in
the hernial sac.
58 Disorders of Sexual Differentiation
undescended testis, and the Mullerian structures
are an intraoperative surprise. In the era of laparos-
copy, these abnormalities are more often diag-
nosed. The treatment comprises of excision of
Mullerian remnants with careful preservation of
vas deferens and orchidopexy. Often staged orchi-
dopexy (two-stage Fowler-Stephens) is needed.
Principles of diagnosis and management of
DSD
• A multidisciplinary approach with geneticists,
neonatologists, pediatric and adult endocri-
nologists, gynecologists, pediatric surgeons/
urologists, psychologists, ethicists, and social
workers.
• Goal of DSD treatment is the long-term physi-
cal, psychological, and sexual well-being of
the patient (“patient centered”).
• When recognized in the neonatal period, situ-
ation should be explained to the parents fully
and kindly. Registering and naming the new-
born should be delayed as long as necessary.
Summary
Diagnosis (Table 58.1)
DSD should be suspected in the following
circumstances:
• Genital ambiguity
• Female genitalia with palpable gonad/clitoro-
megaly or posterior fusion of labia minora
• Male genitalia with micropenis, severe hypospa-
dias, and hypospadias with undescended testis
• Older patients with delayed/incomplete
puberty, virilization in female, primary amen-
orrhea, and gynecomastia in males
Important points
• Presence of palpable gonads rules out 46,XX
DSD
• Presence of Mullerian structures (cervix or
uterus) rules out 46,XY DSD
• CAH should be suspected in every case with
ambiguous genitalia and nonpalpable gonads.
Suggested Reading 361

Table 58.2 Rapid diagnosis of common DSD disorders management. Female gender assignment is the
Y chromosome Y chromosome rule. In cases with significant masculinization,
present absent clitoroplasty and repair of urogenital sinus are
Symmetrical 46,XY DSD 46,XX DSD needed.
gonads For other DSD conditions, the sex assignment
Asymmetrical Mixed gonadal True depends on the following:
gonads dysgenesis hermaphrodite
(MGD) (ovotesticular
DSD) • Parental and patient preference. It is prefera-
ble to delay the surgical genital reconstruction
Plasma 17-hydroxyprogesterone (17-OHP) is if possible till the patient can give valid
raised in CAH due to enzymatic block. It is a consent.
useful marker for diagnosis and monitoring • Ease of genital reconstruction. Phallus of less
response to treatment. than 1.5 cm at birth is preferably considered
for female genitoplasty.
In addition to the standard karyotyping, a
rapid diagnosis can also be made by clinical
examination along with determination of Y chro- Components of Female Genitoplasty
mosome by PCR (polymerase chain reaction) or (Either or all of the Following)
FISH (fluorescent in situ hybridization) test. On
clinical examination, position of gonads should • Clitoroplasty
be noted carefully. If one gonad is above and the • Repair of urogenital sinus including total or
other below the superficial inguinal ring, then partial urogenital mobilization (TUM/PUM)
they are considered asymmetric. If both gonads • Vaginal replacement
are present above or below the superficial ingui- • Removal of testicular tissue
nal ring, then they are called symmetric
(Table 58.2).
Components of Male Genitoplasty
(Either or all of the Following)
Common investigations ordered in
the diagnosis of DSD • Orchidopexy
• Hypospadias repair
• Repair of penoscrotal transposition
• Pelvic ultrasonography—to look for gonads • Removal of ovarian tissue
and Mullerian structures • Removal of Mullerian structures is not needed
• Karyotype till they are symptomatic
• Genitogram—retrograde contrast study • Some patients may need testosterone injection
through the apparent urethral opening at puberty to help development of secondary
• MRI pelvis—for Mullerian structures sexual characteristics
• Diagnostic laparoscopy including gonadal
biopsy
Suggested Reading

Summary of Treatment of DSD
Congenital adrenal hyperplasia (CAH) is treated
with oral hydrocortisone with/without fludrocor-
tisone. Minor clitoromegaly reverses on medical
Belman BA, King RL, Kramer SA, editors. Clinical pedi-
atric urology. 4th ed. Boca Raton, FL: Martin Dunitz;
2002.
Kavoussi LR, Novick AC, Partin AW, Peters
CA. Campbell–Walsh urology. 10th ed. Philadelphia,
PA: Elsevier/Saunders; 2012.
 Gynecological and Breast
Disorders in Children
In this chapter, common gynecological and breast-
related problems in children are discussed.
Ovarian Cysts
Neonatal ovarian cysts are common finding, and
most female neonates develop small ovarian
cysts in response to maternal estrogens. In about
one-fifth of them, the cyst may be more than
1 cm in diameter. After birth the levels of gonad-
otropins reach prepubertal level by 2–3 years of
age and result in gradual resolution of the ovarian
cyst. Therefore most simple ovarian cyst can be
managed expectantly in neonates.
Management of Ovarian Cysts
The management of ovarian cyst depends on the
age of presentation, size, and echogenicity of its
contents. As a general rule most ovarian cysts in
neonates should be managed expectantly.
Simple Cyst
• Cysts less than 2 cm are not pathological.
• Cysts less than 5 cm can be managed expec-
tantly with close follow-up.
• Cysts more than 5 cm can be considered for
intervention as they have more chances of
© Springer Nature Singapore Pte Ltd. 2018
S. Roy Choudhury, Pediatric Surgery, https://doi.org/10.1007/978-981-10-6304-6_59
59
undergoing torsion. Aspiration can be per-
formed, but fenestration of the cyst (either
laparoscopically or open) is preferred.
Complex cyst is the one, which has a multi-­
septate appearance on ultrasonography. This can be
due to either bleeding into the cyst or torsion.
Complex cysts can cause bleeding, rupture, and
intestinal obstruction and hence should be consid-
ered for cystectomy with preservation of maximal
ovarian tissue. Presence of solid elements inside the
cyst on imaging should arouse suspicion of neopla-
sia, and appropriate investigations should be done.
Ovarian Cyst in Prepubertal Child
It is very uncommon (2–3%) during the prepu-
bertal period. A single cyst of less than 1 cm can
be considered non-pathological and ignored, but
a larger cyst needs evaluation. Presence of preco-
cious puberty should be ruled out.
Cysts can be managed conservatively if its
ovarian origin is definite, alpha-fetoprotein
(AFP) level is normal, simple cyst and the child is
asymptomatic. The cyst should decrease in
3–6 months’ time. If it does not decrease, then
intervention is warranted.
Simple cyst can be managed by cystectomy or
fenestration. For complex cysts, cystectomy or
oopherectomy (as differentiation from mature
teratoma is difficult) is preferred.
363
364
Ovarian Cysts in Adolescence
Ovarian cysts are again very common in adoles-
cence and are secondary to either dysfunctional
ovulation with persistent follicle or due to persis-
tent corpus luteum. Complex cysts are subjected
to surgery as differentiation from teratomas is
often difficult. The management is similar to that
of management of ovarian cyst in a prepubertal
child.
Ovarian Torsion
Ovarian torsion results from partial or complete
twisting of the ovarian pedicle on its axis caus-
ing vascular compromise, congestion, and hem-
orrhagic infarction (Fig. 59.1a). Ovarian torsion
is a surgical emergency as delay in treatment
can lead to loss of the organ. Clinically, most
children present with an abrupt onset of low
abdominal pain, low-grade fever, nausea, and
vomiting sometimes mimicking symptoms of
acute appendicitis. Although ovarian torsion can
occur at any age, it is commonly seen in prepu-
bertal girls. Most ovarian torsions are associated
with a concomitant ovarian cystic or solid mass.
Ultrasound is the imaging study of choice. An
enlarged solid ovarian mass with peripheral
cysts noted at US suggests the diagnosis of tor-
sion. In neonates with ovarian torsion, ultra-
a b
Fig. 59.1 (a) Twisted gangrenous ovary. (b) Twisted ovarian cyst in a neonate
59 Gynecological and Breast Disorders in Children
sound studies generally show a cystic mass
(Fig. 59.1b), whereas a prepubertal child may
have a complex mass. CT scan may be required
to clarify the nature of the mass. Color Doppler
ultrasonography is useful to confirm the absence
of ovarian blood flow in torsion. Management is
by either laparoscopy or surgical exploration.
The removal of a mass carrying a twisted ovary
is recommended therapy. Untwisting of torsion
and observation in case of absence of a mass
(normal ovaries) are accepted modes of treat-
ment. Contralateral oophoropexy in cases of
torsion of a normal adnexa is advisable.
Labial Adhesions in Infants
Labial adhesion is a common pediatric gyneco-
logic problem occasionally confused with imper-
forate hymen or absent vagina thus causing a
great degree of parental anxiety. Most children
present between 1 and 2 years of age. It has been
proposed that low estrogen level results in labial
adhesions. This explains rarity of this condition
in neonates who are protected by maternal estro-
gens. Other causes include local inflammation,
irritation, trauma, and sexual abuse. A small
opening near the clitoris is always present
through which urine escapes. This seldom causes
symptoms except recurrent UTI if it covers the
urethral meatus. Another presentation can be
Vaginal Conditions 365

Fig. 59.2 (a, b) Labial

a b
adhesion, before and
after separation

wetting of the undergarments due to escape of Presentation

urine in vestibule once the child gets up after • Primary amenorrhea
voiding. • Lower abdominal pain due to bleeding in the
Treatment consists of applying estrogenic rudimentary uterus in response to hormones
cream (0.1%) for 2 weeks. Manual separation is • Prepubertal presentation is often due to absent
a simple minor procedure and can be done in vaginal opening
the outpatient department with an artery for- • Vestibular anus with vaginal agenesis
ceps or even with the nozzle of an ointment
tube (Fig. 59.2a, b). Adhesion may recur. Differentiation from complete androgen insen-
Unless the urethral meatus is covered, there is sitivity syndrome (CAIS) is essential (Table 59.1).
no reason to be further aggressive in manage-
ment. Prolonged use of estrogenic cream can Treatment
cause precocious sexual development and It is best managed during the postpubertal period
should be discouraged. when the presence and functional status of the uterus
can be easily ascertained. The presence of a uterus
with cervix and proximal vagina can be considered
Vaginal Conditions for pull-through vaginoplasty. In other patients, hor-
mones can suppress the rudimentary uterus and a
Mayer-Rokitansky-Küster-Hauser neo-vagina created with a bowel segment.
Syndrome (MRKH Syndrome)
OHVIRA Syndrome
It is defined as the presence of vaginal agenesis Obstructed hemivagina with ipsilateral renal agen-
with rudimentary uterus and normal ovaries, esis or dysplastic kidney is due to Mullerian and
often associated with renal and skeletal abnor- renal anomaly. The goal of management relies on
malities. The Incidence of MRKH syndrome is
1 in 4000–5000 live female births.
Table 59.1 Difference between MRKH syndrome and
Embryology and pathology: This is due to CAIS
failure of Mullerian ducts to reach the urogeni-
MRKH
tal sinus during the development of the embryo. Features syndrome CAIS
The uterus is often rudimentary with normal Karyotype 46XX 46XY
ovaries. Rarely normal uterus and cervix may be Vaginal opening Absent in most Present in most
present. Vagina is completely absent in most Uterus and ovary Present Both absent
with small vaginal pouch present in about Testis and Absent Present
20–25% children. Associated renal abnormali- testosterone
ties include renal agenesis or ectopia, whereas Associated renal/ Present Absent
skeletal abnormalities may manifest as spine, skeletal defects
limb, or rib defects. Pubic hair at puberty Present Absent
366
identification of the pathological anatomy, surgical
treatment of the vaginal septum, and removal of
dysplastic kidney with ectopic ureter.
Vaginal Septum
Septate vagina is a congenital condition where
two vaginal canals are created by the septum
completely or partially (Fig. 59.3). The septate
vagina may go unnoticed until puberty. They may
be associated with other urogenital anomalies
like cloaca or congenital pouch colon. Physical
examination including endoscopy is diagnostic.
Most of these patients have two cervices. The
septa can be divided around pubertal age, during
surgical correction of associated anomaly or in
symptomatic patients.
Transverse vaginal septum is a congenital
condition of females that can block the passage
of vaginal secretions causing primary amenor-
rhea, hematocolpos, and cyclic pelvic pain. The
septum can be found in the upper, middle, or
lower vagina with varying thickness, upper
vagina being the commonest. Physical examina-
tion along with pelvic ultrasound is diagnostic.
Surgical resection is the treatment of choice and is
usually undertaken after menarche. Postoperative
dilation may be necessary to prevent restenosis and
dyspareunia.
Hemivagina on both sides in the vestibule is
often seen in girls with congenital pouch colon
(Fig. 59.4). They are associated with double
uterus and double cervix. The lower part of the
septum can be divided in adolescent girls to allow
sexual penetration.
Fig. 59.3 Septate vagina
59 Gynecological and Breast Disorders in Children
Vaginal bleeding in the female infant is not
uncommon and often a cause of concern. There
are several causes which need careful evaluation
to establish the diagnosis. Maternal withdrawal
stimulation is the commonest cause of vaginal
bleeding in newborns during the first 2 weeks of
life. This is a self-limiting condition; hence,
observation and reassurance suffice. Vulvo­vaginitis
in children caused by ascending enteric organ-
isms associated with poor hygiene or fungal
infections is managed with appropriate antimi-
crobial therapy. Foreign bodies in the vagina can
produce serosanguineous discharge. Physical
examination with endoscopy will aid in the diag-
nosis Tumors of the genital tract associated with
vaginal bleeding include vascular malformation
involving the genital tract, rhabdomyosarcoma
(the most common malignant tumor of the low
genital tract in young females), endodermal sinus
tumors of the vagina, and endometrial carcino-
mas. Functional ovarian (Sertoli-Leydig cell
tumor) or adrenal tumors that produce estrogen
can be associated with sexual precocity and vagi-
nal bleeding. Prolapsed urethral mucosa through the
Fig. 59.4 Hemivagina in a case of congenital pouch
colon (arrow)
Vaginal Conditions
urethral meatus can present as a friable polypoid
lesion with bleeding. Genital injury is a common
cause of vaginal bleeding in young girls.
Imperforate Hymen
Inspection of female genitalia at birth should be
done by the caretakers to look for any anatomical
genital abnormalities like imperforate hymen
(Fig. 59.5a, b). Obstructing lesions may present
with hydrocolpos and/or metrocolpos (Fig. 59.6)
which can be picked up by antenatal or postnatal
ultrasound. Several anatomical variations of
hymen exist. Micro-perforation is often present
allowing egress of secretions and blood. Infant
and adolescent girls may present with recurrent
urinary tract infection or abdominal mass due to
hydrometrocolpos. Amenorrhea, with recurrent
abdominal pain, can occur with the onset of
Fig. 59.5 (a)
a b
Imperforate hymen
(New born). (b) After
incision
Fig. 59.6 Hydrocolpos (arrow) due to cloaca with vagi-
nal obstruction
367
puberty. Careful genital examination with
ultrasonography clinches the diagnosis. In
­
asymptomatic patients, surgical treatment should
be deferred until puberty when the estrogen surge
will help to prevent further scarring of the surgi-
cal incision. Early surgical incision in a low
estrogenic environment may result in scarring
and recurrence of obstruction. Removal of an
elliptical piece of excess hymenal tissue is pre-
ferred to cruciate incision due to high chances of
obstruction with the latter.
Hymenal tag often presents as a protruding
mass in newborn and infants causing signifi-
cant parental anxiety. This is a simple condi-
tion (Fig. 59.7), and management requires
observation with reassurance to the family.
Normal hymen can have different appearances
(Fig. 59.8).
Fig. 59.7 Hymenal tag
368 59 Gynecological and Breast Disorders in Children

Transplacental hormonal influence in neonates

may cause hyperplasia of breast tissue with pre-
disposition to infection (mastitis neonatorum)
(Fig. 59.11a, b). The condition is due to mater-
nal prolactin hormone, the level of which is
maximum in the fetus at the time of birth. The
milky discharge is the normal response to the
hormone. Infection or abscess formation needs
appropriate treatment with antibiotics and
drainage. Sometimes there may be complaint of
intermittent episodes of bleeding through the
nipple of the baby. In infants it is a benign, self-

Fig. 59.8 Hymenal membrane

Breast Disorders

Breast disorders in children of either sex are

mostly benign. Premature hyperplasia (the-
larche) in females is the most common breast
lesion in children causing significant concern in
the family. It occurs around the age of 8–10 years
as a disk-shaped concentric asymptomatic sub- Fig. 59.9 Virginal hypertrophy of breast, should be dif-
areolar mass. Occasionally it is associated with ferentiated from a giant fibroadenoma
pain in the breast. It remains static until changes
occur in the opposite breast 6–12 months later.
It can regress spontaneously or stay until puberty
arrives. The management is by counseling and
reassurance to the family. Virginal hypertrophy
is rapid breast enlargement after puberty due to
estrogen sensitivity (Fig. 59.9). If symptomatic,
management is by reduction mammoplasty.

Mastitis

Breast enlargement is commonly seen in new-

born babies (Fig. 59.10). This may often be
associated with clear or milky nipple discharge. Fig. 59.10 Neonatal breast enlargement (physiological)
Breast Disorders 369

Fig. 59.11 (a) Neonatal

a b
mastitis. (b) Breast
abscess formation

limited condition that should be managed con-

servatively. The main reason of bloody nipple
discharge in older children is mammary ductal
ectasia, which is an anomaly of ductal develop-
ment. Surgical procedures should be avoided,
because injury to the breast bud may cause per-
manent damage.

Breast Fibroadenoma

Fibroadenoma is a common benign tumor found

in the breast of adolescent girls. It is also consid-
ered the most common discrete solid mass found Fig. 59.12 Fibroadenoma breast (note circumareolar
within the adolescent breast tissue called “breast cosmetic incision for its removal)
mouse.” Most girls harboring a fibroadenoma are
between 13 and 16 years of age; the tumor is slow
growing and tends to develop in the upper outer
quadrant. The risk of malignancy is extremely
low. The tumor is usually solitary, discrete with
an average diameter of 2–4 cm with marginal
increase in size during menstruation. At physical
exam the mass feels like a well-circumscribed,
movable, “rubbery” nodule. Fibroadenomas may
be related to an exaggerated local response to the
estrogenic effects of puberty. Sonography of the
breast is helpful in making a diagnosis in doubt-
ful cases. The tumor looks well circumscribed,
hyperechoic, and homogenous on ultrasound.
Fine needle aspiration cytology is helpful in
establishing the diagnosis. Management of grow-
ing, symptomatic, or anxiety-producing fibroad-
enoma is surgical excision through a periareolar
Fig. 59.13 Accessory nipple
incision to preserve cosmesis (Fig. 59.12).
Fibroadenomas more than 5 cm in diameter are
called giant fibroadenomas and should be rapid growth leading to often a fungating mass.
excised. Cystosarcoma phyllodes or phyllodes Although predominantly benign, they are locally
tumor is a giant breast tumor in females with aggressive, and 10–15% are malignant.
370
Fig. 59.14
Gynecomastia. (a)
a b
Unilateral. (b) Bilateral
gynecomastia
Supernumerary (Accessory) Nipple
Accessary nipple (polythelia) can occur anywhere
along the milk line from the axilla to the pubis
(Fig. 59.13). They are most frequently located in
the axilla or below a normally developed breast.
About one third have more than one location of
supernumerary breast tissue. In some cases, a true
accessory breast termed polymastia can develop.
Surgery for polythelia is performed for cosmetic
reasons. Polymastia should be excised due to
potential swelling during later pregnancy.
Gynecomastia
It is a condition characterized by breast enlargement
in boys which occurs due to excessive estrogen or
decreased testosterone levels (Fig. 59.14a, b). Mostly
(65%) it is physiological due to hormonal fluctua-
tions during puberty. It is more often seen in obese
boys probably because of increased estrone, pro-
duced by aromatization of androstenedione in fatty
tissue. In others, there could be an underlying cause
for the gynecomastia, such as tumors secreting
estrogen/human chorionic gonadotrophin, liver
dysfunction, hyperthyroidism, or inadvertent
administration of exogenous estrogen. Decreased
testosterone levels can occur in Klinefelter’s
syndrome, androgen insensitivity, or defects in
­
59 Gynecological and Breast Disorders in Children
t­estosterone biosynthesis. Also it is caused by some
drugs such as cimetidine. A pathologic cause should
be suspected when either the puberty is early
(tumors) or late (gonadal dysfunction), when there
is rapid breast growth, or if there is nipple
discharge.
Physiologic gynecomastia usually resolves
in 75% of boys within 2 years and takes slightly
longer for boys who are obese. Hence the most
appropriate therapy is reassurance. Surgery is
reserved for cases where the breast size does
not decrease over the period of a year or if
there is excessive fibrosis of the breast tissue.
Surgery is indicated in those boys where there
is risk of malignant degeneration; e.g., boys
with Klinefelter’s syndrome should be consid-
ered for a prophylactic mastectomy. For idio-
pathic gynecomastia, a subareolar mastectomy
with preservation of nipple and areola or lipo-
suction of the breast tissue is the treatment of
choice.
Suggested Reading
Chadha R, Roy Choudhury S, Pant N, Jain V, Puri A,
Acharya H, Zargar NU, Kundal A. The anoma-
lous clinical anatomy of Congenital Pouch Colon in
Females. J Pediatr Surg. 2011;46:1593–602.
Coran AG, Caldamone A, Scott Adzich N, Krummel TM,
Laberge JM, editors. Pediatric surgery. 7th ed. Oxford:
Elsevier; 2012.
 Part 8
Miscellaneous
 Recent Advances in Pediatric
Surgery
Over the last five decades, the understanding and
management of pediatric surgical diseases have
gone through a sea of changes. Recent advances
in the diagnosis and treatment may be considered
under the following headings:
1. Antenatal diagnosis and management
2. Fetal interventions
3. Progress in neonatal surgery
4. Advances in pediatric urology/gastroentero-
logy
5. Thoracic/oncology surgery
6. Minimal access/robotic surgery
7. Pediatric organ transplantation
8. Tissue engineering and stem cell therapy
Antenatal diagnosis and fetal interventions are
discussed in Chap. 4.
Progress in Neonatal Surgery
Esophageal Atresia
Survival was poor in the 1960s. With improve-
ment of neonatal surgical critical care, neonates
weighing 1500 g currently have a predicted sur-
vival of 97%. Use of thoracoscopy for repair for
esophageal atresia is a new advancement.
Advances in tissue engineering may help chil-
dren with long gap esophageal atresia.
© Springer Nature Singapore Pte Ltd. 2018
S. Roy Choudhury, Pediatric Surgery, https://doi.org/10.1007/978-981-10-6304-6_60
60
Hirschsprung Disease
The understanding of molecular genetics of
Hirschsprung disease has significantly changed
with identification of several gene loci like RET
proto-oncogene which have been found to be asso-
ciated with familial and long-segment Hirschsprung
disease. The management is also changing from
multistage operations to modern single-stage oper-
ation aided with laparoscopy. New trend is toward
performing a single-stage procedure in the new-
born period with the aid of frozen section diagnosis
of the level of aganglionosis and with laparoscopic
assistance. Moreover this procedure can be per-
formed transanally with or without laparoscopy.
Use of immunohistochemistry and application of
calretinin stain also helps in making diagnosis of
Hirschsprung’s disease in difficult cases.
Imperforate Anus
The pioneering contribution of Peña in the surgi-
cal management of anorectal malformation by
the posterior sagittal approach (PSARP) has
resulted in better understanding of the anatomy
with superior surgical outcome. Single-stage pro-
cedures in the newborn period are associated
with better chance of continence and lesser num-
ber of operative procedures. Laparoscopic-
assisted anorectoplasty has better cosmetic and
373
374
functional outcome. Total urogenital sinus mobi-
lization is helpful for correction of various cloa-
cal and urogenital abnormalities. Molecular
biological researches aimed at identifying the
genetic basis of anorectal malformations are cur-
rently ongoing.
Congenital Diaphragmatic Hernia
Better understanding of the pathophysiology has
resulted in paradigm shift of the management
strategies. In utero intervention is recommended
in fetuses with low LHR (lung-to-head ratio less
than 1). Addition of high-frequency ventilation,
inhaled nitric oxide, and ECMO has resulted in
improved outcome.
Intestinal Atresias/Abdominal Wall
Defects
Successful program of total parenteral nutrition
in newborn patients with the advances in liver
and bowel transplantation has resulted in
improved outcome.
 ecent Advances on Organ
R
Transplantation
Improvement in immunosuppressive therapy and
better surgical outcome have resulted in overall
improved results in pediatric organ transplanta-
tion. It has now been realized that donor pre-
treatment with antileukocyte antibody followed
by minimal postoperative immunosuppression
will exhaust the donor immune response to the
allograft and result in long-term tolerance.
The Pittsburgh group has reported the survival
of 55% of patients undergoing intestinal
transplantation.
Liver transplantation in children continues to
achieve excellent results. Survival rate excess of 80%
has been recently reported from Birmingham, UK. It
is now being increasingly used to improve survival in
patients with EHBA, neonatal cholestasis, and multi-
focal or PRETEXT IV hepatoblastoma.
60 Recent Advances in Pediatric Surgery
The use of split liver transplantation (whole
liver divided into two grafts) has led to a further
increase in the donor pool.
Living related donor transplantation is now
successfully done in several centers in India.
 ecent Advances in Pediatric
R
Tumors
With improvement in multimodality manage-
ment (radiotherapy, chemotherapy, surgery,
bone marrow transplantation, stem cell therapy)
of pediatric solid tumors, the survival rates have
improved. There is a continuous effort to
improve survival of children while limiting the
complications with focus on achieving long-
term good quality of life. Markers which affect
prognosis have been identified in most tumors,
and the treatment delivered is fine-tuned depend-
ing upon the presence or absence of these mark-
ers to achieve optimal outcome.
The current 5-year survival of Wilm’s tumor is
>90% in Stage I and Stage II.
The survival of hepatoblastoma is 65–70%.
Treatment of rhabdomyosarcoma has
improved 5-year survival to >75%.
Neuroblastoma still presents a major challenge.
Short Bowel Syndrome
The recent advances in the management of short
bowel syndrome includes:
• Drugs and stimulants
Applicable for improving short bowel syn-
drome are growth hormone, glutamine, glucagon-­
like peptide (GLP 2) and somatostatin
(octreotide). The function of interstitial cells of
Cajal as the gut pacemaker has been identified.
• Bowel lengthening techniques
Bianchi, Kimura, and STEP (serial transverse
enteroplasty) procedures are being applied to
increase the absorptive surface of the bowel.
• Tissue engineering (see below)
• Bowel transplantation
Advances in Imaging
 pplication of Newer
A and heart valves have been cultured. Engineering
Understandings
• Antiangiogenesis and suppression in the
treatment of cancer and use of beta blockers
in the treatment of hemangiomas are in clini-
cal use.
• Scarless healing of fetus is an advantage for
fetal surgery like fetal meningomyelocele
repair.
• Gene replacement therapy may become suc-
cessful in management of metabolic and
genetic diseases.
• Application of nanotechnology to improve the
delivery system of drugs, vaccines, and con-
trast materials for imaging studies.
Harvesting Stem Cells
Stem cells are primaliry of two types, embryonic
or adult type. They have the potential to differen-
tiate in to various types of cell lines, hence, can
be pleuripotent or multipotent. Bone marrow is a
rich sorce of stem cells and are used clinically for
various conditions.
Embryonic Stem Cells
Fresh sample of amniotic fluid contains many
cells cast off by the fetus. The cells that are still
living appear white and constitute just 1.8% of
the sample.
Cord blood stem cells can be preserved for use
in the future.
Tissue Engineering
Tissue engineering is a promising technology for
supply of different organs and tissue for clinical
use. Harvested stem cells are used over a biode-
gradable scaffold to form various tissues.
Complex tissue and organ building is still a chal-
lenge at present. At present thin tissues with low
metabolic activity like the skin, bone, cartilage,
375
of more complex tissues like the esophagus or
intestine is still under experiment.
 olecular Genetics and Gene
M
Therapy
Majority of pediatric surgical diseases are due to
unidentified multifactorial inheritance. The chance
of recurrence of a disease in such a disorder is
higher than the general population, especially in
severe form of the disease and if both parents are
affected. The current importance lies in early diag-
nosis of genetic disorders and more informed
counseling of parents. Gene therapy is currently
not available for pediatric surgical disorders.
However better understanding of viral vectors and
genomics may allow us to find some clinical appli-
cation for single-gene disorder to begin with.
Advances in Imaging
Ultrasonography
3D ultrasonography: The computer reconstructs
a 3D image from either 2D array or 1D array with
data collected overtime.
4D ultrasonography: This allows 3D recon-
struction in real time. The utility of this ultra-
sound is limited at present to antenatal ultrasound
to look for aberrations.
Multidetector Computed
Tomography (MDCT)
In contrast to conventional CT, MDCT uses mul-
tiple parallel rows of detectors that acquire data
by rotating around the patient. It has the follow-
ing advantages over the conventional CT:
• Shorter acquisition time.
• Thinner or thicker sections can be created
from raw data.
• Improved 3D reconstruction.
376 60 Recent Advances in Pediatric Surgery

 lectron Beam Computed

E repair of esophageal atresia, pyeloplasty and
Tomography (EBCT) other urological procedures.

EBCT has a large stationary X-ray tube that

partly covers the patient and obviates the need to
move the X-ray source and the detector. It is
therefore able to capture images ten times faster
than MDCT and has been used by cardiologists
to acquire images in a beating heart. It also limits
the amount of radiation to the child.
Magnetic Resonance Imaging
• Use of higher field strength (3.0 tesla as com-
pared to 1.5 tesla).
• Ultrafast MRI.
• Functional MRI allows detecting neuronal
activity by detecting changes in blood flow to
different parts of the brain.
 nergy Sources in Application
E
for Surgery
• Ultrasonic cutting and coagulation (Harmonic
scalpel) uses ultrasound energy at very high
frequency (55,000 Hz) which divides the tis-
sue with minimal collateral damage.
• Ligasure.
• Enseal: advanced bipolar with vessel sealing
up to 7mm.
 obot in Pediatric Surgery: Da Vinci
R
System
Advantage of Robotic Surgery

1. 3D visualization
 ET (Positron Emission Tomography)
P 2. Instruments with 6° of freedom
Scan 3. Motion scaling
4. Tremor reduction
This is able to detect the functional activity in the 5. Intuitive instrument control
tissue. Tissues which are metabolically active are
picked up, so it is useful in detecting malignancy
and infection. Positron-emitting radionuclide is Limitation
taken up by the active cell which allows them to be
detected. PET-CT allows correlation of the areas 1. Cost
of increased uptake with the images on CT scan. 2. Technical limitation of instrument size for
pediatric patients
3. Training
 inimal Access Surgery (MAS)
M 4. Currently robotic urological operations are
in Pediatric Surgery (See Chapter 61) reported in children

MAS is very useful for the following conditions:
intra-abdominal testis, intersex anomalies, VATS
for empyema, lymph node and tumor biopsy,
tubercular abdomen, and cholecystectomy.
MAS has some advantage in duplications,
appendix, Hirschsprung disease, pyloromyot-
omy, and management of high anorectal malfor-
mations by pull-through.
Advanced MAS procedures are lap splenec-
tomy, choledochal cyst excision, fundoplication,
Subcutaneous Endoscopic (Stealth)
Surgery: Instruments inserted in cosmetically
insignificant location like axilla are used to oper-
ate on areas with marked cosmetic consider-
ations like the neck. This technique can be used
for torticollis and subcutaneous lesions of the
neck.
NOTES (natural orifice transluminal endo-
scopic surgery): Transvaginal, transrectal, or
transgastric routes can be used to perform
Suggested Reading
intra-­abdominal endoscopic surgery, e.g., trans-
vaginal cholecystectomy.
SILS (single-incision laparoscopic sur-
gery): A single incision is used to insert a com-
pound port which allows insertion of a camera
and two instruments to perform surgery.
Use of virtual reality as simulators for endoscopic
surgery training.
377
Suggested Reading
Gupta DK, Sharma S, Azizkhan RG, editors. Pediatric
surgery—diagnosis and management. 1st ed. New
Delhi, India: Jaypee Brothers; 2009.
Coran AG, Caldamone A, Scott Adzich N, Krummel
TM, Laberge JM, editors. Pediatric surgery. 7th ed.
Elsevier: Oxford; 2012.
 Minimally Invasive Surgery
in Children 61

The current understanding of minimally invasive Physiological Changes in MIS

surgery (MIS) is surgical interventions involving
the least possible physical trauma to the patient, Pneumoperitoneum is a complex process and
particularly surgery performed using an endo- affects many systems:
scope passed through a tiny incision.
• Acid-base metabolism
• Respiratory mechanics
Advantages of MISs • Cardiovascular effects
• Renal effects
1. Better cosmesis
• Neurological effects
2. Better analgesia
• Physiological changes
3. Less adhesions
4. Decreased ileus Acid-base metabolism:
5. Magnified field of vision
6. Early recovery and shorter hospital stay
1. Increased ETCO2
2. Increased pCO2
Disadvantages of Pediatric MIS 3. Decreased pH
4. ETCO2 returns to baseline by 6–8 min
1. Additional expense due to setup and 5. Minute ventilation increases 15–20% for
equipments normocarbia
2. Prolonged procedures
3. Lack of tactile evaluation
4. Loss of depth perception Cardiovascular Changes
5. Alteration of physiology in small patients
6. Steep learning curve for advanced techniques High CO2 causes direct arteriolar dilatation and
myocardial depression. It indirectly increases
Learning curve improves with practice (simu- catecholamines thereby increasing heart rate,
lator); operating time decreases to “nearly that” blood pressure, and cardiac output. High intra-­
of open procedures with increased experience abdominal pressure is associated with decreased
and newer technology. venous return.

© Springer Nature Singapore Pte Ltd. 2018 379

S. Roy Choudhury, Pediatric Surgery, https://doi.org/10.1007/978-981-10-6304-6_61
380 61 Minimally Invasive Surgery in Children

Renal Effects  asic Concepts: VATS (Video-­

Reversible oliguria due to renal vein compres-
sion, direct parenchymal compression, and no
changes in serum creatinine level
Neurological Effects
Increased intracranial pressure due to hypercar-
bia, increased intra-abdominal pressure
B
Assisted Thoracoscopic Surgery)
Exposure
Double lung ventilation
Collapse of lung by low pressure (4–8 mm
Hg) of CO2
Retraction of lung by retractors
Single lung ventilation (not always required)
Basic Instruments for MIS (Fig. 61.1)

Other Effects 1. Telescope

2. Camera
1. Hypothermia 3. Monitor
2. Pneumothorax 4. Light source
3. Pneumomediastinum 5. Fiber-optic cable
4. Gas embolism 6. Gas insufflator
5. Subcutaneous emphysema 7. CO2 cylinder
8. Recorder
9. Suction irrigation unit
Precautions in Pediatric MIS 10. Hand instruments
11. Ports of various sizes from 1.8 to 12 mm
1. Empty bladder – catheterize for infraumbili- 12. Hand instruments (Fig. 61.2):
cal procedure. (a) Forceps
2. Empty stomach – for supraumbilical proce- (b) Scissors
dure. (c) Needle holders
3. Positioning the table as required. (d) Knife
4. Open method of primary port placement (e) Retractors
(Hassen’s technique) is preferred in children (f) Clip applicators
over Veress needle insertion. (g) Endo staplers
5. Ergonomics: Proper port placement in chil- 13. Electrocautery
dren is important as small working space is a 14. Harmonic
challenge. 15. Ligasure
6. Port fixation: Rubber sleeve on the port – pre- 16. Telescope—0–30°
vents gas leak, fixation of the port with a stitch 17. Clip applicator
prevents port migration. 18. Endo stapler
7. Pneumoperitoneum: Keep the pressure mini-
mum.
Energy Source Devices
Although no absolute values can be recom-
mended, pressures in the following ranges are Electrocoagulation
considered to be safe: 6–8 mmHg for infants,
8–12 mmHg for small children, and 12–15 mmHg Monopolar
for older children and adolescents Bipolar
Energy Source Devices 381

Fig. 61.1 Basic laparoscopic equipment set

Fig. 61.2 Basic laparoscopic instruments for pediatric MIS

382 61 Minimally Invasive Surgery in Children

 armonic Scalpel (Ultrasonic Cutting

H Table 61.1 Current applications of pediatric MIS
and Coagulation): Advantages 1 Impalpable UDT
2 Hernia
1. Minimum charring and desiccation 3 Appendectomy
2. Reduced need for ligatures 4 Cholecystectomy
3. Coagulation/cutting at lower temperatures 5 Pyloric stenosis
4. Less lateral thermal damage 6 Lap-assisted pull through
5. Heat development in the tissue rather than the 7 Intersex anomaly
instrument 8 Biopsy
6. No electrical circuit through the patient 9 Ovarian cyst, Intra-abdominal mass
10 Recurrent abdominal pain/bleeding
11 Tubercular abdomen
 igasure (Pressure and Energy
L 12 Empyema (VATS)
for Vessel Sealing) 13 Lung biopsy (VATS)
14 Eventration (VATS)
1. Combination of pressure and energy 15 CDH (VATS)
2. Melts the collagen and elastin Contraindications of MIS
3. Forms a permanent, plastic-like seal 1 Bleeding disorder
4. Effective for vessels up to 7 mm in diameter 2 Unstable hemodynamic status
3 Massive abdominal distension
5. Lateral thermal spread is minimal (1–2 mm)
4 Severe cardiac disease
5 Pulmonary insufficiency
6 Adhesions
I ndications for MIS in Pediatric
7 Abdominal wall sepsis
Surgery
8 Polytrauma
For success in pediatric minimally invasive surgery,
They can be divided into three groups the following points should be remembered:
(Table 61.1): (a) Proper case selection and safety of the procedure
(b) Readiness to convert
1. MAS is very useful for the following condi- (c) Constant practice to improve skills and spectrum
tions: intra-abdominal testis, intersex anoma- in pediatric MIS
lies, VATS for empyema, thoracoscopy and (d) Stay informed about new techniques, tools, and
repair of diaphragm, lung biopsy, lymph node indications for MIS
and tumor biopsy, tubercular abdomen, chole- Robotic surgery (see chapter 60)
cystectomy, and fundoplication.
2. MAS have some advantage in duplications,
appendix, Hirschsprung disease, pyloromyot-
omy, pyeloplasty and pull-through operation Suggested Reading
for high anorectal malformations.
LeBlanc KA. Management of laparoscopic surgical com-
3. Advanced MAS procedures: lap splenectomy, plications. 2nd ed. USA: Marcel Dekker; 2004.
choledochal cyst excision, gastric pull-up, repair Godbole P, editor. Pediatric endourological technique. 1st
of esophageal atresia and lung lobectomies. ed. Heidelberg: Springer; 2007.
 Short Cases in Pediatric Surgery
62

Pediatric surgery covers a large number of condi-
tions which are not organs specific; hence, those
commonly encountered conditions seen in prac-
tice are discussed in this chapter.
Tongue-Tie or Ankyloglossia
Ankyloglossia or tongue-tie is a common con-
genital disorder involving the lingual frenulum
characterized by restriction of movement of
tongue tip which cannot be protruded beyond the
lower incisor teeth. It varies in degree, from a
mild form to a severe form in which the tongue is
completely tethered to the floor of the mouth.
Examination requires a tongue depressor in a
young child or asking to protrude the tongue in
older children to check for tethering and tongue
mobility. If the frenulum is short and thick, it
may restrict the mobility of the tongue
(Fig. 62.1a–c). Minor degree of ankyloglossia
where the tongue easily protrudes out of the teeth
margin may be observed for speech development.
In older children, speech and articulation may be
affected, although the correlation is not well doc-
umented and it is difficult to predict about which
patient the difficulty will occur. It is often a cause
for serious concern to the family. The frenulum is

a b c

Fig. 62.1 (a–c) Tongue-tie. (a, b) On protrusion, (c) on retraction of tongue

© Springer Nature Singapore Pte Ltd. 2018 383

S. Roy Choudhury, Pediatric Surgery, https://doi.org/10.1007/978-981-10-6304-6_62
384
thin and relatively avascular in neonates and
young infants; it can often be incised with a sharp
scissor as an outpatient procedure. Surgical treat-
ment in older patients may require a short general
anesthetic for surgical division (frenulectomy).
Ranula
A ranula is a type of mucocele found on the floor
of the mouth. The appearance is blue, translu-
cent, fluctuant dome-shaped swelling (Fig. 62.2).
It is generally due to the collection of salivary
mucin from the salivary gland duct as a result of
minor trauma. Small asymptomatic lesions can
be observed. Larger lesions may require surgery.
Treatment involves marsupialization of the cyst
wall to the floor of the mouth or removal of the
sublingual gland.
BCG Lymph Adenitis
Suppurative lymphadenitis occurs in 0.1–1% of
BCG vaccine recipients (Fig. 62.3). Most com-
monly they resolve spontaneously, but chemo-
Fig. 62.2 Ranula
62 Short Cases in Pediatric Surgery
Fig. 62.3 BCG adenitis
therapy may be needed occasionally. Aspiration
of the cold abscess may be required. Surgical
excision of a suppurative draining node is rarely
necessary.
Tubercular Lymphadenitis
Lymph node tuberculosis usually occurs in cervi-
cal, axillary, and inguinal region. Jones and
Campbell staging of superficial tuberculous
lymphadenitis are as follows:
Stage I: Enlarged, firm, mobile discrete nodes
showing nonreactive hyperplasia
Stage II: Larger rubbery nodes fixed to sur-
rounding tissues due to periadenitis
Stage III: Central softening due to abscess
formation
Stage IV: Collar stud abscess
Stage V: Rupture and sinus formation (under-
mined edges with discolored margins)
Management is by antituberculous drugs
(ATT) and surgical excision in minority of
cases.
Chemotherapy is nearly always curative in cases
of superficial lymphadenitis before caseation and
sinus formation (Figs. 62.4 and 62.5). Surgery is
limited to those patients who fail to show improve-
ment even after an adequate course of chemother-
apy or who have discomfort from enlarged tense
Presacral Cleft
Fig. 62.4 Tubercular sinus in the groin
Fig. 62.5 Tubercular sinus in the neck
a b
Fig. 62.6 (a, b) Caput succedaneum
385
and fluctuant nodes and with atypical mycobacte-
rial infections often resistant to ATT.
Caput Succedaneum
Caput succedaneum (Fig. 62.6a, b) is seen in
some cases of birth trauma, vacuum, and forceps
delivery due to rupture of blood vessels within
the dense connective tissue underneath the skin.
Clinically the swelling crosses the suture lines as
there is subcutaneous fluid collection. Purpura
and ecchymosis of the overlying skin are com-
mon. The bleeding is usually minimal due to the
tamponading effect of the dense connective tis-
sue and usually resolves spontaneously within
few days.
A cephalhematoma is collection of blood
between the periosteum and the skull bone. Since
it is a subperiosteal swelling, its boundaries are
limited by individual bone which is in contrast to
caput which is subcutaneous extraperiosteal fluid
collection with poorly defined margins.
Both conditions are treated with expectant
observation and reassurance to the family.
Injection of vitamin K is recommended to correct
coagulation disorder in the new born.
Presacral Cleft
The presentation is with an asymptomatic depres-
sion at the lower end of the coccyx. They are
harmless and should be left alone (Fig. 62.7).
386 62 Short Cases in Pediatric Surgery

Fig. 62.7 (a, b)

a b
Presacral cleft

Syndactyly

Syndactyly is a condition where two or more dig-

its are fused together (Figs. 62.8 and 62.9a, b). It
may affect the hand or the foot. Surgical release
is only necessary to gain movement of function-
ing digits. Ideal timing for surgery is between 6
and 12 months.
Polydactyly or multiple digits (Fig. 62.10): The
world record for polydactyly in a child is recently
reported from Uttar Pradesh India with 34 digits.
A digit attached with a simple skin tag (accessory
digit) can be easily removed (Fig. 62.11).

Ludwig’s Angina

Ludwig’s angina is cellulitis affecting the floor of

the mouth which can cause airway obstruction
(Fig. 62.12). Aggressive treatment with hospital-
ization, intravenous antibiotics, and incision and
drainage may be necessary. Airway obstruction
may require a tracheostomy.
Amniotic Band
Fig. 62.8 Syndactyly hand
amputation of body parts. The bands can be
released with Z-plasties after 2 years of age or as
an emergency procedure if they are associated
with neurocirculatory compromise.

Amniotic bands (also called Streeter bands) are Mucous Cyst

embryonic strands of tissue which crisscross
from one wall of the amniotic sac to the other and
thus can constrict any part of the developing
fetus. This can interfere with the normal develop-
ment of that part (Fig. 62.13). They can result in
Mucous cyst of oral cavity (Fig. 62.14) is a com-
mon problem. Management is generally by
observation only. Rarely surgical marsupializa-
tion is required.
Mucous Cyst 387

Fig. 62.9 Syndactyly

a b
(a) hand and (b) foot

Fig. 62.10 Polydactyly

with syndactyly (28
fingers and toes in one
patient)

Fig. 62.12 Ludwig’s angina

Fig. 62.11 Accessory digit

388
Fig. 62.13 Amniotic band
Treatment of preauricular tag and preauricular
sinus (Figs. 62.15 and 62.16): They are best left
alone.
Ganglion Cyst
A ganglion cyst or synovial cyst is due to leak-
age of synovial fluid from the joint into the sur-
rounding tissue usually tendon sheath. They are
firm swelling fixed to the underlying tendon
sheath (Fig. 62.17a, b). The first line of manage-
ment of ganglion cyst is observation and reas-
surance to the family regarding its benign
nature. Chance of spontaneous resolution is
62 Short Cases in Pediatric Surgery
Fig. 62.14 Mucous cyst lower lip
Fig. 62.15 Preauricular tag
very high. Historically Bible (heavy book) was
used to rupture the cyst into the tissue followed
by absorption of the fluid. Symptomatic ones
may be treated with needle aspiration, with
injection of local steroid (kenacort), and with or
without hyalase (hyaluronidase), although there
are high chances of recurrence. Surgical exci-
sion may be necessary.
Ingrown Toe Nail 389

Pyogenic Granuloma

Pyogenic granulomas (Fig. 62.18) are friable

vascular lesions that present as rapidly growing
papules or nodules and hence may bleed pro-
fusely after minor trauma. The term “pyogenic
granuloma” is a misnomer. These lesions are vas-
cular lesions and are not infective in etiology.
Treatment with surgical excision and skin sutur-
ing, electrocoagulation, or CO2 laser ablation is
equally curative, although sometimes they may
recur.

Ingrown Toe Nail

Ingrowing toe nail (Fig. 62.19) is a painful condi-

tion due to overgrowth of the nail into one or both
sides of the nail bed resulting in inflammation
and granuloma formation. Treatment is by surgi-
cal excision of the nail (half or total) along with
the germinal layer from the nail bed.
Fig. 62.16 Preauricular sinus

Fig. 62.17 (a, b)

a b
Ganglion cyst on the
wrist

Fig. 62.18 Pyogenic

granuloma foot
390 62 Short Cases in Pediatric Surgery

Fig. 62.19 Ingrown toe nail

Dermoid Cyst

Dermoid cysts are seen either in the midline of Fig. 62.20 Angular dermoid cyst
the body or along the lines of embryonic fusion
(Fig. 62.20). Treatment is by complete surgical
excision.

Wound Granuloma

Treatment of wound granuloma (Fig. 62.21) is

by chemical cauterization with copper sulfate
or silver nitrate or trichloroacetic acid.

Keloid

Management of keloid (Fig. 62.22) includes

intralesional steroids (triamcinolone), surgical
excision, cryosurgery, radiation therapy, inter-
feron alpha, and pulse dye laser treatment.
Fig. 62.21 Chest tube site wound granuloma
Suggested Reading 391

a b

Fig. 62.22 (a) Keloid over leg, (b) keloid over chest

Suggested Reading
Hutson JM, O’Brien M, Woodward AA, Beasley
SW. Jone’s clinical pediatric surgery diagnosis and
management. 6th ed. Oxford: Blackwell; 2008.
 Picture Plates (Miscellaneous)
63

Fig. 63.2 Connective tissue disorder

Fig. 63.1 Virilizing adrenal tumor (isosexual precocious

puberty)

© Springer Nature Singapore Pte Ltd. 2018 393

S. Roy Choudhury, Pediatric Surgery, https://doi.org/10.1007/978-981-10-6304-6_63
394 63 Picture Plates (Miscellaneous)

Fig. 63.5 Phocomelia

Fig. 63.3 Waardenburg-Shah syndrome (white hair with

Hirschsprung’s disease)

Fig. 63.4 Sirenomelia

(mermaid)
63 Picture Plates (Miscellaneous) 395

Fig. 63.6 Osteogenesis a b

imperfect (a) blue
sclera, (b) limb fractures

Fig. 63.7 Giant melanocytic nevus

396 63 Picture Plates (Miscellaneous)

Fig. 63.8 Human tail

Fig. 63.9 Wet gangrene

Fig. 63.10 Talipes

equinovarus
63 Picture Plates (Miscellaneous) 397

Fig. 63.11 Complete penoscrotal transposition

a b

Fig. 63.12 (a, b) Aphalia with urethral agenesis

a b

Fig. 63.13 (a, b) Buried penis

398 63 Picture Plates (Miscellaneous)

Fig. 63.16 Ectopic scrotum

Fig. 63.14 Buried penis due to obesity

Fig. 63.15 Diphallus

a b

Fig. 63.17 Precautious puberty (a) male with (b) gynecomastia

63 Picture Plates (Miscellaneous) 399

Fig. 63.18 Chest X-ray of hydatid cyst lung

Fig. 63.20 CT scan showing hydatid cyst lung (floating

membrane)

Fig. 63.19 Hydatid cyst lung, note white laminated

membrane

Fig. 63.21 CT scan hydatid cyst lung

400 63 Picture Plates (Miscellaneous)

a b

Fig. 63.22 (a, b) CT scan multiple hydatid cysts of liver

Fig. 63.23 Hydatid cyst of liver at operation, note the

white laminated membrane

Fig. 63.24 Hydatid cyst of liver removed surgically

63 Picture Plates (Miscellaneous) 401

Fig. 63.25 CT scan hydatid cyst of kidney

Fig. 63.26 Prune belly syndrome (lax abdominal wall,

cryptorchidism, dilated urinary tract) (note Foley catheter
in the patent urachus)