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Diagnostic Imaging Spine - NoRestriction PDF
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IAGNOSTIC IMAGING
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IAGN8STIC IMAGING
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Jeffrey S. Ross, MD
Staff Neuroradiologist
Head, Radiology Research
Cleveland Clinic Foundation
Kevin R. Moore, MD
Adjunct Assistant Professor of Radiology
Section of Neuroradiology
University of Utah, School of Medicine
Pediatric Neuroradiologist
Primary Children's Medical Center
Salt Lake City, UT
Julia Crim, MD
Chief of Musculoskeletal Radiology
Associate Professor of Radiology
University of Utah, School of Medicine
Salt Lake City, UT
Mark Z. Chen, MD
Department of Radiology
Hoag Memorial Hospital
Newport Beach, CA
Assistant Clinical Professor
Department of Radiology
UCSF School of Medicine
Gregory L. Katzman, MD
Associate Professor, Neuroradiology
Adjunct Associate Professor, Medical Informatics
University of Utah, School of Medicine
Chief, Radiology VA Hospital
Salt Lake City, UT
iii·
AMI RSYS'"
Names you know, content you trust'"
111
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III
AMIRSYS®
Names you know, content you trust»
First Edition
Text - Copyright Jeffrey S. Ross, MD 2004
All rights reserved. No part of this publication may be reproduced, stored in a retrieval system, or transmitted, in any form or media
or by any means, electronic, mechanical, photocopying, recording, or otherwise, without prior written permission from Amirsys Inc.
ISBN: 0-7216-2880-X
ISBN: 0-8089-2315-3 (International English Edition)
In the cases where drugs or other chemicals are prescribed, readers are a,dvised to check the Product information currently provided by the manufacturer of each drug to be
administered to verify the recommen~ed dose, the method and duration of administration, and contraindications. It is the responsibility of the treating physician relying on
experience and knowledge of the patient to determine dosages and the best treatment for the patient.
To the maximum extent permitted by applicable law. Amirsys provides the Product AS lS AND WlTH ALL FAULTS, AND HEREBY DISCLAIMS ALL WARRANTIES AND
CONDITIONS. WHETHER EXPRESS, IMPLIED OR STATUTORY. INCLUDING BUT NOT LIMlTED TO. ANY (IF ANY) IMPLIED WARRANTIES OR CONDITIONS OF
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Amirsys disclaims all warranties of any kind if the Product was customized. repackaged or altered in any way by any third party.
Diagnostic imaging. Spine / Jeffrey S. Ross ... let al.].- 1st ed.
p. em.
Includes index.
ISBN 0-7216-2880-X
1. Spine-Imaging. I. Title: Spine. II. Ross, Jeffrey S. Oeffrey Stuart)
RD768.D535 2005
616.7'30754-dc22
2004057457
IV
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The fear of the Lord is the beginning of knowledge, but fools despise wisdom and discipline.
Proverbs 1: 7
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We at Amirsys and Elsevier are proud to present Diagnostic Imaging: Spine, the fourth volume in our acclaimed
Diagnostic Imaging series. This precedent-setting,.image- and graphic-packed series began with David Stoller's DI:
Orthopaedics. The next two volumes, DI: Brain and DI: Head and Neck are now joined by Jeff Ross and Michael
Brant-Zawadzki's DI: Spine. The current practice of neuroradiology demands expertise in all three core areas. Spine,
the last in the neuroradiology "triumvirate," covers the major (and most important minor) diagnoses that affect the
axial skeleton, intradural space, spinal cord and peripheral nerves.
Again, the unique bulleted format of the Diagnostic Imaging series allows our authors to present approximately
•
twice the information and four times the images per diagnosis compared to the old-fashioned traditional prose
textbook. All the DI books follow the same format, which means the same information is in the same place: Every
time! In every organ system. The innovative visual differential diagnosis "thumbnail" that provides an at-a-glance
look at entities that can mimic the diagnosis in question has been highly popular. "Key Facts" boxes provide a
succinct summary for quick, easy review.
In summary, Diagnostic Imaging is a product designed with you, the reader, in mind. Today's typical practice
settings demand efficiency in both image interpretation and learning. We think you'll find the new Spine volume a
highly efficient and wonderfully rich resource that will complete your core neuroradiology reference books. Enjoy!
Anne G. Osborn, MD •
H. Ric Harnsberger, MD
CEO, Amirsys Inc.
• •
Vll
•
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FOREWORD
Signs and symptoms referable to the spine are among the most common reasons for seeking medical attention
and stimulus for imaging. While most symptoms are related to self-limiting disorders, they occur across a backdrop of
morphologic changes that range from subtle to extraordinarily complex in terms of appearances and differentials.
Sorting these considerations out requires more than deciding between normal and abnormal, but also normal and
unusual, abnormal but not relevant, abnormal and not much we can do and abnormal and not a moment to lose.
Where and when to go next requires one to sort through differential considerations and subsequent options, whether
imaging, intervention or tincture of time. The imager in this environment requires a blend of experience and
expertise in interpretation, technical knowledge, judgment and common sense. While noble and sought after
qualities we all wish to possess in some abundance, in today's world of rapidly evolving technology and new
knowledge, we all need quick, accurate and easy to acquire supplemental intelligence. Hence the need for a usable,
compact up to date reference that will enable our decision-making. Such a reference companion becomes invaluable
in our efforts to move from observation to wisdom.
What lies within is the result of selection, common sense afld thoughtful labor. The review of the literature is
current and the layout is practical and logical. Not only are the primary disease considerations well illustrated, but
the differential considerations as well. A format extremely helpful in the decision making process. This is not a text
in the traditional sense and the verbiage has been trimmed to the absolute minimum. As one who as always been
drawn to dense prose, the format was at first unsettling. If the absence of punctuation and complete sentence is
offensive, this text is not for you. If you are fond of word smithing in your medical texts, this format is not for you. If,
however, you want data nuggets in lieu of word streams and real life illustrations in lieu of descriptions, you are home
free. Succinct bullets, copious illustrations and images are the format. One need not wade through paragraphs to
extract information about the disease, differentials, genetics, imaging findings and technical considerations. These
are conveyed more succinctly and efficiently than one could possibly expect.
While this is not the text you will curl up with next to a fire on a cold winter night, it is the text you will always
keep within arms reach in the reading room.
Michael T. Modic, MD
Chairman, Division of Radiology
Cleveland Clinic Foundation
IX
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PREFACE
It's about time! Hard to comprehend that 10 years have gone by since I last was co-author on a major spine
imaging text. Bifocals, gray hair and innumerable MRI's of degenerated disc disease have occurred, and have been
read in the interim. Hanging of film hardcopies has become a rare and rather quaint experience. Voice recognition is
now a dictation mainstay. "Hanging chad" is now a part of my vocabulary.
Spine MR imaging has also undergone a gentler evolution typical of a mature technology and sits squarely at the
forefront of spinal diagnosis. CT is enjoying another resurgence given multi-detector technology and ease of use of
3D workstations and PACS systems.
With the publication of DI spine, my distinguished co-authors and I are pleased to be part of an evolution in
educational textbooks. Gone is the prose, replaced by a tight outline format allOWing not only a dense amount of
information for each diagnosis, but also the ability to rapidly scan the material for areas of particular interest by well-
defined subheadings. There is the highest priority on images, with space for multiple examples of a given pathologic
diagnosis.
Diagnoses have been divided into sections, with easy color coding and numbering for quick reference.
Differential considerations are highlighted by smaller thumbnail images. Really stressed for time? Try the key facts
box for each diagnosis. Integral to the book are the over 130 illustrations, superbly rendered by James Cooper.
Each diagnosis is meant to stand by itself, yet maintain a close integration within the section. The number of
unique diagnoses is exhaustive giving the reader an in depth coverage of all the essential diagnoses in a given area of
the spine. With this highly structured approach to spine information, the production of a second edition is possible in
a much shorter time interval than seen with my previous spine textbook.
I am indebted to my co-authors and to the entire Amirsys team for their dedication and diligence in prodUcing
this work. I hope you find this book not only visually pleasing but of real and timely use in your practice.
Jeffrey S. Ross, MD
Xl
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ACKNOWLEDGMENTS
Illustrations
James A. Cooper, MD
Image/Text Editing
Angie D. Mascarenaz
Kaerli Main
Case Management
Cassie L. Dearth
David Harnsberger
Roth LaFleur
Production Lead
Melissa A. Morris
Xlll
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SECTIONS
PART I
Congenital
Congenital and Developmental Disorders [j]
PART II
Trauma, Degeneration, and Inflammatory Arthritides
Trauma [I]
Degenerative Disease and Inflammatory Arthritides [1]
PART III
Infection and Inflammatory Disorders
Infection [j]
Inflammatory and Autoimmune [I]
PART IV
Neoplasms, Cysts and Other Masses
Neoplasms [j]
Non-Neoplastic Cysts and Tumor Mimics [I]
PART V
Vascular and Systemic Disorders
Vascular Lesions [1]
Spinal Manifestations of Systemic Diseases [1]
PART VI
Peripheral Nerve and Plexus
Plexus and Peripheral Nerve Lesions rn
PART VII
Spine Post-Procedural Imaging
Post-Procedural Imaging and Complications [I]
PART VIII
Potpourri
Gamuts [I]
xv
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TABLE OF CONTENTS
Anomalies of Notochord
PART I Development
Congenital Diastematomyelia 1-1-56
Kevin R. Moore, MD
Neurenteric Cyst 1-1-60
SECTION 1 Kevin R. Moore, MD
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Intradural Extramedullary
Meningioma IV-I-78
Michael Brant-Zawadzki, MD, FACR
Hemangiopericytoma IV-I-82
Michael Brant-Zawadzki, MD, FACR
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Schwannoma IV-1-86
Mark Z. Chen, MD PART V
Neurofibroma IV-1-90
Mark Z. Chen, MD
Vascular and Systemic Disorders
Malignant Nerve Sheath Tumors IV-1-94
Mark Z. Chen, MD
CSF Disseminated Metastases IV-1-98 SECTION 1
Michael Brant-Zawadzki, MD, FACR Vascular Lesions
Intramedullary Introduction and Overview
Astrocytoma, Spinal Cord IV-I-I02 Vascular Anatomy V-1-2
Michael Brant-Zawadzki, MD, FACR Jeffrey S. Ross, MD
Ependymoma, Cellular, Spinal Cord IV-l-106
Mark Z. Chen, MD Vascular lesions
Ependymoma, Myxopapillary, Spinal Cord IV-l-lIO Type I DAVF V-1-6
Mark Z. Chen, MD Jeffrey S. Ross, MD
Hemangioblastoma, Spinal Cord IV-l-lI4 Type II AVM V-l-lO
Jeffrey S. Ross, MD Jeffrey S. Ross, MD
Spinal Cord Metastases IV-l-lI8 Type III AVM V-1-14
Michael Brant-Zawadzki, MD, FACR Jeffrey S. Ross, MD
Paraganglioma IV-1-122 Type IV AVF V-1-18
Jeffrey S. Ross, MD Jeffrey S. Ross, MD
Melanocytoma IV-1-126 Cavernous Malformation, Spinal Com V-1-22
Jeffrey S. Ross, MD Jeffrey S. Ross, MD
Spinal Cord Infarction V-1-26
Jeffrey S. Ross, MD
SECTION 2 Subarachnoid Hemorrhage V-1-30
Non-Neoplasti~ Cysts and Tumor Jeffrey S. Ross, MD
Mimics Spontaneous Epidural Hematoma V-1-34
Mark Z. Chen, MD
Cysts Subdural Hematoma V-1-38
Arachnoid Cyst IV-2-2 Mark Z. Chen, MD
Mark Z. Chen, MD Superficial Siderosis V-1-42
Posterior Sacral Meningocele IV-2-6 Jeffrey S. Ross, MD
Mark Z. Chen, MD
Perineural Root Sleeve Cyst IV-2-10
Mark Z. Chen, MD SECTION 2
Syringomyelia IV-2-14 Spinal Manifestations of Systemic
Kevin R. Moore, MD Diseases
Non-Neoplastic Masses and Tumor Paget Disease V-2-2
Mark Z. Chen, MD
Mimics
Hyperparathyroidism V-2-6
Epidural Lipomatosis IV-2-18 Julia Crim, MD
Mark Z. Chen, MD
Renal Osteodystrophy V-2-8
Heterogeneous Fatty Marrow IV-2-22
Julia Crim, MD
Julia Crim, MD
Hyperplastic Vertebral Marrow V-2-10
Langerhans Cell Histiocytosis IV-2-24 Mark Z. Chen, MD
Kevin R. Moore, MD
Myelofibrosis V-2-14
Acquired Epidermoid Tumor IV-2-28 Julia Crim, MD
Mark Z. Chen, MD
Extramedullary Hematopoiesis V-2-16
Fibrous Dysplasia IV-2-30 Mark Z. Chen, MD
Julia Crim, MD
Tumoral Calcinosis V-2-20
Kummel Disease IV-2-32 Jeffrey S. Ross, MD
Julia Crim, MD
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SECTION 1 SECTION 1
Plexus & Peripheral Nerve Post-Procedural Imaging and
Lesions Complications
Introduction and Overview Post-Interventional Procedure
Normal Plexus and Nerve Anatomy VI-I-2 Imaging and Complications
Kevin R. Moore, MD
Myelography Complications VII-I-2
Gregory 1. Katzman, MD
Plexus & Peripheral Nerve lesions Vertebroplasty Complications VII-I-6
Superior Sulcus Tumor VI-I-6 Michael Brant-Zawadzki, MD, FACR
Jeffrey S. Ross, MD
CSF Leakage Syndrome VII-I-IO
Muscle Denervation VI-I-IO Michael Brant-Zawadzki, MD, FACR
Julia Crim, MD
Brachial Plexus Traction Injury VI-I-12 Post-Operative Imaging and
Kevin R. Moore, MD
VI-I-16
Complications
Traumatic Neuroma
Julia Crim, MD Hardware Failure VII-I-14
Mark Z. Chen, MD
Thoracic Outlet Syndrome VI-I-18
Kevin R. Moore, MD Accelerated Degeneration VII-I-18
Jeffrey S. Ross, MD
Idiopathic Brachial Plexus Neuritis VI-I-22
Julia Crim, MD Post-Laminectomy Spondylolisthesis VII-I-22
Jeffrey S. Ross, MD
Radiation Plexopathy VI-I-26
Kevin R. Moore, MD Peridural Fibrosis VII-I-24
Jeffrey S. Ross, MD
Hypertrophic Neuropathy VI-I-30
Kevin R. Moore, MD Pseudomeningocele VII-I-28
Jeffrey S. Ross, MD
Peripheral Nerve Tumor VI-I-34
Julia Crim, MD Bone Graft Complications VII-I-32
Jeffrey S. Ross, MD
Peripheral Neurolymphomatosis VI-I-38
Kevin R. Moore, MD Recurrent Vertebral Disc Herniation VII-I-34
Jeffrey S. Ross, MD
Radial Neuropathy VI-I-40
Julia Crim, MD Post-Operative Infection VII-I-38
Gregory 1. Katzman, MD
Femoral Neuropathy VI-I-42
Kevin R. Moore, MD Post-Operative Spinal Complications VII-I-42
Jeffrey S. Ross, MD
Ulnar Neuropathy VI-I-44
Kevin R. Moore, MD Failed Back Surgery Syndrome VII-I-46
Jeffrey S. Ross, MD
Suprascapular Nerve Entrapment VI-I-48
Julia Crim, MD Metal Artifact VII-I-SO
Jeffrey S. Ross, MD
Median Nerve Entrapment VI-I-SO
Julia Crim, MD
Common Peroneal Neuropathy VI-I-52 Post-Radiation and Chemotherapy
Kevin R. Moore, MD Complications
Posterior Tibial Nerve Entrapment VI-I-54 Radiation Myelopathy VII-I-54
Julia Crim, MD Michael Brant-Zawadzki, MD, FACR
Post Irradiation Vertebral Marrow VII-I-58
Mark Z. Chen, MD
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PART VIII
Potpourri
SECTION 1
Gamuts
Secondary Acute Transverse Myelitis VIII-I-2
Mark Z. Chen, MD
Back Pain in Children VIII-I-6
Mark Z. Chen, MD
Kyphosis VIII-I-IO
Julia Crim, MD
Peripheral Neuropathy VIII-I-12
Julia Crim, MD
Pathologic Vertebral Fracture VIII-I-14
Julia Crim, MD
Dense Vertebral Body VIII-I-18
Julia Crim, MD
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IAGNOSTIC IMAGING
I
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SPINE EMBRYOLOGY
1
2
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3D graphic shows normal formation of neural plate 3D graphic shows normal neural tube closure (top) and
(top) and neural groove (bottom). Cutaneous dysjunction (bottom). Cutaneous epithelium (orange),
epithelium (orange), neural crest (red), neural ectoderm neural crest (red), neural ectoderm (green), and
(green), and notochord (gray). notochord (gray).
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SPINE EMBRYOLOGY
DIFFERENTIAL DIAGNOSIS
1
Notochord anomalies Caudal cell mass anomalies 3
• Split notochord syndromes; dorsal enteric • Caudal regression syndrome
cyst/sinus/fistulJ/diverticulum, neurenteric cyst • Filum fibrolipoma
• Diastematomyelia • Tight filum terminale (tethered cord syndrome)
• Terminal myelocystocele
Premature dysjunction anomalies • Anterior sacral meningocele
• Intradural spinal lipoma (rostral to filum) • Sacrococcygeal teratoma
• Lipomyelocele
• Lipomyelomeningocele Vertebral formation and segmentation
• Terminal lipoma anomalies
Nondysjunction anomalies • Hemivertebra, butterfly vertebra, block vertebra,
"Klippel-Feil syndrome"
• Myelocele
• Myelomeningocele Malformations of unconfirmed mechanism
• Dorsal dermal sinus • Simple (dorsal) meningocele
• Cervical myelocystocele • Lateral meningocele
o Transient communication of amnion through • Day 30: Neural tube development below caudal
notochordal canal to yolk sac (canal of Kovalevsky - neuropore commences within undifferentiated caudal
neurenteric canal) cell mass (of primitive streak)
• End of 3rd week: Neural fold/groove formation o Less precise process than neurulation
(neurulation) o Cysts develop in caudal cell mass, coalesce into
o Neural folds elongate, fuse in midline ~ neural tube ependymal lined tubular structure, unite with rostral
• Fusion occurs at multiple sites simultaneously neural tube
o Temporary openings at cranial and caudal ends o Transient ventriculus terminalis appears (day 43 to
(neuropores) 48)
• Normally close day 25 ~ 27, signaling completion o Retrogressive differentiation ~ caudal conus
of neurulation medullaris, filum terminale
o Notochord induces surrounding paraxial mesoderm • Days 40-60: Vertebra undergo chondrification (within
derived from primitive streak ~ paired somite blocks specific centers)
(myotomes, sclerotomes) • About 3rd gestational month, spinal cord extends
• Myotomes ~ paraspinal muscles, skin cover nearly entire length of developing spinal column
• Medial sclerotomes ~ vertebral body, o During further development, vertebral column and
intervertebral disc, meninges, spinal ligaments dura elongate faster than spinal cord, producing
• Lateral sclerotomes ~ posterior spinal elements apparent shift of terminal cord to higher level
o Neuroepithelial cells (neuroblasts) around central • Conus near adult level by infancy
tube form mantle layer ~ spinal cord gray matter o Lower spinal nerves (cauda equina) descend
• Dorsal neuroblasts ~ sensory neurons obliquely to exit through corresponding vertebral
• Ventral neuroblasts ~ motor neurons foramen
• Intermediate horn neuroblasts ~ sympathetic • After chondrification, vertebral ossification
autonomic nervous system neurons commences (4 distinct centers in vertebral body,
o Outermost spinal cord layer forms marginal layer, vertebral arches), continues through birth into young
myelinates ~ spinal cord white matter adulthood
o Central neuroepithelial cells differentiate into
ependymal cells along central canal
o Neural crest cells appear along each side of neural
ICLINICAL IMPLICATIONS
groove ~ spinal sensory ganglia (DRG), autonomic
ganglia, Schwann cells, meninges (pia, arachnoid), Clinical Importance
pigment cells, adrenal medulla • Abnormal neurenteric canal ~ persistent
• Day 24: Sclerotomal resegmentation begins, continues enteric-spinal canal connection (canal of Kovalevsky)
through 5th week o Dorsal-enteric anomalies (neurenteric cyst) spectrum
o Caudal half of superior vertebra combines with • Failure of correct neural groove closure 2° teratogen or
rostral half of inferior vertebra after split at faulty notochordal induction ~ neural tube defect
sclerotomal cleft ~ "new" vertebral body disorders
• Intersegmental arteries trapped in center of new o Myelocele, myelomeningocele
vertebral body o Lower extremity paralysis, associated brain
o Notochord within vertebral body degenerates; anomalies (callosal dysgenesis, Chiari 2
intervertebral notochordal remnant ~ vertebral disc malformation)
nucleus pulposus • Failure of vertebral formation, segmentation ~
segmentation and fusion anomalies (SFAs)
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SPINE EMBRYOLOGY
1
4
Axial graphic demonstrates type 7 diastematomyelia Axial T2WI MR depicts classic type 7 diastematomyelia,
with large osseous spur connecting vertebral body to with ossified spur dividing dural sac, spinal cord (note,
posterior elements. Spinal cord is divided into two syringomyelic left hemicord).
heimicords, each within a separate dural tube (right
hemicord has syrinx).
o Butterfly vertebra, hemivertebra, block vertebra • Vertebral formation and segmentation disorders
o Scoliosis, respiratory compromise, "Klippel-Feil o Hemivertebra, butterfly vertebra, block vertebra
syndrome" • Malformations of unconfirmed mechanism
• Incomplete primitive streak regression may leave o Simple (dorsal) meningocele
caudal remnant ~ sacrococcygeal teratoma o Lateral meningocele
• Notochordal remnant within posterior vertebral body,
clivus, or sacrum may undergo malignant
degeneration ~ chordoma ISELECTED REFERENCES
1. Larsen WJ: Human embryology. 3rd ed. New York:
Churchill-Livingstone. 47-59, 2001
ICUSTOM DIFFERENTIAL DIAGNOSIS I 2. Jinkins JR: Atlas of neuroradiologic embryology, anatomy,
and variants. Philadelphia. Lippincott Williams & Wilkins.
Pertinent differential diagnosis 1-38,2000
3. Barkovich A: Pediatric Neuroimaging. 3rd ed. Philadelphia:
• Notochordal anomalies Lippincott-Raven 621-665, 2000
o Neural plate incompletely splits from notochord 4. Tortori-Donati P et al: Spinal dysraphism: a review of
• Split notochord syndrome (dorsal enteric neuroradiological features with embryological correlations
cyst/sinus/fistula/diverticulum) and proposal for a new classification. Neuroradiology.
• Diastematomyelia 42(7):471-91, 2000
• Neurulation anomalies 5. Moore K et al: The developing human; clinically oriented
o Premature dysjunction embryology. Philadelphia: W.B.Saunders Co. 53-63, 1993
• Dysjunction prior to neural tube closure permits 6. Pang D: Sacral agenesis' and caudal spinal cord
malformations. Neurosurgery. 32(5): 755-78, discussion
perineural mesenchyme access to neural groove 778-9, 1993
and ependymal lining - differentiation into fat, 7. DeLaPaz RL: Congenital anomalies of the lumbosacral
incomplete neural tube closure spine. Neuroimaging Clinics of North America.
• Intradural spinal lipoma (rostral to filum), 3(3):439-40, 1993
lipomyelo(meningo)cele 8. Pang D et al: Split cord malformation: Part I: A unified
o Nondysjuction theory of embryogenesis' for double spinal cord
• Produces focal ectodermal/neuroectodermal tract malformations. Neurosurgery. 31(3):451-80, 1992
and prevents mesenchymal migration - focal or 9. Sadler TW: Langman's medical embryology. 5th ed.
Baltimore: Williams and Wilkins, 1985
widespread dysraphism
10. Naidich TP et al: Diastematomyelia: hemicord and
• Myelo(meningo)cele, dorsal dermal sinus, cervical meningeal sheaths; single and double arachnoid and dural
myelocystocele tubes. AJNR Am J Neuroradiol. 4(3):633-6, 1983 '
• Caudal cell mass anomalies
o Faulty caudal cell mass canalization and
retrogressive differentiation
o Insult occurs prior to 4th gestational week
o Filum fibrolipoma, tight filum terminale (tethered
cord syndrome), caudal regression syndrome,
terminal myelocystocele, anterior sacral
meningocele
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SPINE EMBRYOLOGY
IIMAGE GALLERY
1
5
Typical
(Left) Axial graphic shows
lipomyelomeningocele
(premature dysjunction).
Neural placode (red)
attaches to overlying
(skin-covered) fat. Note
posterior dysraphism from
incomplete neural tube
closure. (Right) Axial TlWI
MR shows wide posterior
dysraphism (black arrows),
insertion of rotated neural
placode (white arrow) into a
dorsal lipomatous mass.
Typical
(Left) Axial T2WI MR
demonstrates severe caudal
regression syndrome, with
absence of sacrum and
small, dysplastic iliac wings
(arrows). (Right) Axial Tl WI
MR reveals filum fibrolipoma
(arrow) producing clinical
tethered cord symptoms.
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CHIARI II MALFORMATION
1
6
Sagittal graphic shows characteristic findings of Chiari /I Sagittal TlWI MR depicts classic small posterior fossa
malformation, including callosal dysgenesis, tectal with beaked tectum (arrow), vermian ectopia (curved
beaking, small posterior fossa, vermian ectopia, and arrow), and a small syrinx (open arrow).
medullary kinking.
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CHIARI II MALFORMATION
Key Facts
1
Terminology • Intracranial CSF hypotension 7
• Synonyms: CM 2, Arnold-Chiari malformation type 2 • Severe, chronic shunted hydrocephalus (congenital)
(AC2), Chiari type II Pathology
• Virtually 100% associated with neura(tube closure • Secondary to sequelae of CSF leakage through open
defect (NTD), usually lumbar myelomeningocele spinal dysraphism during gestation (4th fetal week)
(MMC)
Clinical Issues
Imaging Findings • Usually presents within context of known MMC
• Small posterior fossa (PF) • Chiari II malformation most common cause of death
• "Cascade" or "waterfall" of cerebellum/brainstern inMMC
downwards
• Medulla "heaps up" over cord tethered by dentate Diagnostic Checklist
ligaments => cervicomedullary "kink" (70% ) • Low torcular herophili indicates small posterior fossa
• CT or MR showing towering cerebellum, downward
Top Differential Diagnoses vermian displacement, +/- brainstem compression
• Chiari I malformation diagnostic of Chiari II
• Chiari III malformation
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CHIARI II MALFORMATION
1 • Open dysraphism (MMC) ~ 100% (lumbar> >
cervical)
• Surgical management
o Chiari decompression with resection of posterior
8 • Posterior arch C1 anomalies (66%) foramen magnum, C1 ring
• Hydrosyringomyelia (20-90%) o CSF diversion/shunting
• Diastematomyelia (5%) o Fetal MMC repair in selected patients may
• Klippel-Feil syndrome ameliorate Chiari II severity
• Cervical myelocystocele
o Brain/skull
• Corpus callosum (CC) dysgenesis (90%), IDIAGNOSTIC CHECKLIST
aqueductal stenosis, rhombencephalosynapsis,
gray matter malformations, absent septum Consider
pellucidum, fused forniceal columns • Brain/spinal axis MR to detect presence of Chiari II,
• Lacunar skull (hickenschadel) assess severity, look for complications
Gross Pathologic & Surgical Features Image Interpretation Pearls
• Small PF ~ contents shift down into cervical spinal • Low torcular herophili indicates small posterior fossa
canal • CT or MR showing towering cerebellum, downward
o Cerebellar hemispheres/tonsils "wrap" around vermian displacement, +/- brainstem compression
medulla diagnostic of Chiari II
o Pons/cranial nerve roots often elongated
o Compressed/elongated/low IV ventricle ~ pouch in
cervical canal ISELECTED REFERENCES
o +/- Hydrosyringomyelia 1. MeLone DG et al: The Chiari II malformation: cause and
impact. Childs Nerv Syst. 19(7-8):540-50, 2003
Microscopic Features 2. Tubbs RS et al: Chiari II malformation and occult spinal
• Purkinje cell loss, sclerosis within herniated tissues dysraphism. Case reports and a review of the literature.
Pediatr Neurosurg. 39(2):104-7, 2003
3. Sener RN et al: Rhombencephalosynapsis and a Chiari II
ICLINICAL ISSUES malformation. J Comput Assist Tomogr. 27(2):257-9, 2003
4. McDonnell GV et al: Prevalence of the
Presentation Chiari/hydrosyringomyelia complex in adults with spina
• Most common signs/symptoms bifida: preliminary results. Eur J Pediatr Surg. 10 Suppl
1:18-9,2000
o Neonate: MMC, enlarging head circumference +/- 5. Ojemann JG et al: Prepontine lesions with chiari II
hydrocephalus symptoms malformation. Report of two cases. Pediatr Neurosurg.
o Older child/adult: Clinical hydrocephalus, 33(3):113-7,2000
symptoms referable to tethered cord (MMC repair), 6. Northrup H et al: Spina bifida & other neural tube defects.
o All age groups: Varying degrees of lower extremity CUff Probl Pediatr 30:313-32, 2000
paralysis/sphincter dysfunction/bulbar signs 7. MeLone DG: Hydromyelia in a child with chiari II. Pediatr
• Clinical profile Neurosurg. 32(6):328, 2000
o Usually presents within context of known MMC 8. Tulipan N: Intrauterine MMC repair reverses hindbrain
herniation. Pediatr Neurosurg 31 (3): 13 7-42, 1999
• Infants: Enlarging head circumference 9. Piatt JH Jr et al: The Chiari II malformation: lesions
• Child/adult: Known Chiari II malformation, signs discovered within the fourth ventricle. Pediatr Neurosurg.
of hydrocephalus/shunt failure +/- bulbar 30(2):79-85, 1999
symptoms 10. Nishino A et al: Cervical myelocystocele with Chiari II
• Laboratory malformation: magnetic resonance imaging and surgical
o Fetal screening: i (){-feto protein treatment. Surg Neurol. 49(3):269-73, 1998
11. La Marca F et al: Presentation and management of
Demographics hydromyelia in children with Chiari type-II malformation.
• Age: Usually presents at birth with MMC +/- Pediatr Neurosurg. 26(2):57-67, 1997
hydrocephalus 12. Cama A et al: Chiari complex in
children--neuroradiological diagnosis, neurosurgical
• Gender: M = F treatment and proposal of a new classification (312 cases).
Natural History & Prognosis Eur J Pediatr Surg. 5 Suppl 1:35-8, 1995
13. Shuman RM: The Chiari malformations: a constellation of
• Chiari II malformation most common cause of death anomalies. Semin Pediatr Neurol. 2(3):220-6, 1995
inMMC 14. Ruge JR et al: Anatomical progression of the Chiari II
o Brainstem compression/hydrocephalus, intrinsic malformation. Childs Nerv Syst. 8(2):86-91, 1992
brainstem "wiring" defects 15. Nishimura T et al: Brain stem auditory-evoked potentials in
• Progression of spinal neurological deficits is rare; meningomyelocele. Natural history of Chiari II
suspect hydrocephalus, associated undiagnosed spinal malformations. Childs Nerv Syst. 7(6):316-26, 1991
deformity (diastematomyelia), tethered cord 16. Peach B: Arnold-Chiari malformation with normal spine.
Arch Neurol. 10:497-501, 1964
Treatment
• Folate supplement for pregnant mothers
(pre-conception ~ 6 weeks post conception)
significantly decreases MMC risk
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CHIARI II MALFORMATION
IIMAGE GALLERY 1
9
Variant
(Left) Sagittal T2WI MR
shows relatively mild
posterior fossa changes in
conjunction with long
segment (holocord)
syringomyelia (arrows).
(Right) Sagittal T2WI MR
reveals characteristic Chiari /I
posterior fossa findings in
conjunction with long
segment syringomyelia.
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MYELOMENINGOCELE
1
10
Sagittal graphic shows a patulous dural sac without skin Sagittal TlWI MR depicts a large unrepaired lumbar
covering protruding through a dysraphic defect. Inset myelomeningocele, with neural elements protruding
shows wide open dysraphism, with nerve roots dangling into the sac (Courtesy R. Boyer, MOJ.
from ventral placode.
DDx: Myelomeningocele
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MYELOMENINGOCELE
Key Facts
1
Terminology Pathology 11
• Synonyms: MMC, meningomyelocele, open spinal • Red, exposed neural placode leaking CSF protrudes
dysraphism (OSD), spina bifida aperta, spina bifida through osseous midline defect
cystica
• Posterior spinal defect lacking skin covering =} neural Clinical Issues
tissue, CSF, and meninges exposed to air • Stable post-operative deficit expected ~ best possible
outcome
Imaging Findings
• Best diagnostic clue: Wide osseous dysraphism, Diagnostic Checklist
low-lying cord/roots, post-operative skin closure • Imaging untreated MMC rarely indicated
changes • MMC patients frequently have other CNS
abnormalities; neurological deterioration requires
Top Differential Diagnoses assessment of entire craniospinal axis
• Dorsal meningocele • Low-lying cord on MR imaging does not always
• Closed (occult) spinal dysraphism equate to clinical tethering
• Post-operative pseudomeningocele
• Open neural arch, flared laminae, protruding o Dysjunction = normal neural tube separation from
myelomeningocele sac, and Chiari II brain overlying ectoderm during neural tube closure
findings ("lemon" sign, "banana" sign, o Open neural tube defects (NTD) arise from
hydrocephalus) dysjunction failure =} neural placode
• Anterior placode surface =} external pia mater cord
Other Modality Findings surface
• Sagittal cine phase contrast MR =} diminished conus • Posterior placode surface =} internal neural tube
pulsation may indicate tethering ependyma
Imaging Recommendations o Placode may be segmental or terminal
• Best imaging tool: MR imaging • Segmental (lumbar, thoracolumbar, thoracic) =}
spinal cord continues distally
• Protocol advice
o Obstetrical ultrasound: Initial MMC diagnosis, • Terminal (lumbosacral, sacral) =} placode at end of
spinal cord
delivery planning (Caesarian section), triaging for
possible fetal surgery • Genetics
o Pax 3 paired box gene derangements
o Head CT: Hydrocephalus evaluation
o MRI: Sagittal and axial T1 WI and T2WI; must o Methylene-tetra-hydrofolate-reductase (MTHFR)
mutations associated with abnormal folate
include entire sacrum
metabolism
• MTHFR mutations + folate deficiency =} increased
risk ofNTD
I DIFFERENTIAL DIAGNOSIS o Trisomy 13, 18 (14% of NTD fetuses)
Dorsal meningocele • Etiology
• Meninges protrude through dorsal dysraphism into o Lack of complex carbohydrate molecule expression
subcutaneous fat on neuroectodermal cell surface =} failed neural tube
• Skin covered, usually does not contain neural closure
elements o NTD deficits worsened by chronic mechanical
injury, amniotic fluid chemical trauma
Closed (occult) spinal dysraphism • Epidemiology
• Dorsal osseous dysraphism; cord may be low-lying o Incidence 0.44-0.6/1,000 live births
• Skin or other cutaneous derivative (e.g., lipoma) covers o Maternal folate deficiency, obesity, anti-epileptic
neural elements therapy (lowers folate bioavailability)
• Associated abnormalities
Post-operative pseudomeningocele o Kyphoscoliosis: Neuromuscular imbalance +/-
• History, clinical exam permit distinction vertebral segmentation anomalies
• Look for surgical laminectomy defect, absence of spina • Developmental (65%): Neuromuscular imbalance,
bifida osseous changes anterior displacement of spinal extensor muscles
• Congenital (30%): Congenital osseous anomalies
(hemivertebrae, bony bar)
IPATHOLOGY o Diastematomyelia, dermal sinus (31-46%)
• Cord split above (31%), below (25%), or at (22%)
General Features MMC level
• General path comments • Hemimyelocele variant (10%) ~ asymmetric
deficits
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MYELOMENINGOCELE
1 o Syrinx (30-75%)
o Chiari II malformation ("" 100%), hydrocephalus
• MMC closure < 48 hours to stabilize neural deficits,
prevent infection
12 requiring shunting (80%) o Some tertiary centers perform in-utero surgical
o Orthopedic abnormalities (80%); 2° to muscular repair; may lessen Chiari II, neurological deficit
imbalance severity
• Subsequent management revolves around treating
Gross Pathologic & Surgical Features post-operative complications
• Red, exposed neural placode leaking CSF protrudes o Cord untethering, hydrocephalus management,
through osseous midline defect treatment of kyphoscoliosis
• Spinal cord always physically tethered; +/- clinical
neurologic deterioration
Microscopic Features IDIAGNOSTIC CHECKLIST
• Purkinje cell loss, sclerosis of herniated posterior fossa Consider
tissues related to Chiari II malformation • Imaging untreated MMC rarely indicated
• MMC patients frequently have other CNS
abnormalities; neurological deterioration requires
ICLINICAL ISSUES assessment of entire craniospinal axis
Presentation • Cord re-tethering = most common spinal cause of
delayed deterioration
• Most common signs/symptoms
o Stable neurological deficits expected following Image Interpretation Pearls
closure • Low-lying cord on MR imaging does not always equate
• Neurological deterioration => imaging evaluation to clinical tethering
for tethered cord, dural ring constriction, cord
ischemia, or syringohydromyelia
• Clinical profile ISELECTED REFERENCES
o Newborn with midline raw, red, exposed neural
1. Johnson MP et al: Fetal myelomeningocele repair:
placode
short-term clinical outcomes. Am] Obstet Gynecol.
• Lesion level determines severity of neurological 189(2):482-7, 2003
deficits 2. Hirose S et al: Fetal surgery for myelomeningocele: panacea
• Hydrocephalus 2° to Chiari II malformation or peril? World] Surg. 27(1):87-94, 2003
o Post-operative: Fixed paraparesis and sensory deficits 3. Rintoul NE et al: A new look at myelomeningoceles:
concomitant with defect level, large heads functional level, vertebral level, shunting, and the
(hydrocephalus), neurologically induced orthopedic implications for fetal intervention. Pediatrics.
disorders, neurogenic bladder (90%), +/- 109(3):409-13,2002
kyphoscoliosis 4. Ertl-Wagner B et al: Fetal magnetic resonance imaging:
indications, technique, anatomical considerations and a
• Laboratory findings: Elevated maternal serum alpha review of fetal abnormalities. Eur Radiol. 12(8):1931-40,
fetoprotein (AFP) 2002
5. Trivedi] et al: Clinical and radiographic predictors of
Demographics scoliosis in patients with myelomeningocele. ] Bone Joint
• Age: Always present at birth Surg Am. 84-A(8):1389-94, 2002
• Gender: M < F (1:3) 6. Bowman RM et al: Spina bifida outcome: a 25-year
• Ethnicity: 1 Frequency in Irish/Welsh populations prospective. Pediatr Neurosurg. 34(3):114-20, 2001
(4-8x), families with other affected children (7-15x) 7. Tortori-Donati P et al: Magnetic resonance imaging of
spinal dysraphism. Top Magn Reson Imaging.
Natural History & Prognosis 12(6):375-409, 2001
• Stable post-operative deficit expected ~ best possible 8. Unsinn KM et al: US of the spinal cord in newborns:
outcome spectrum of normal findings, variants, congenital
anomalies, and acquired diseases. Radiographies.
o Hydrocephalus, tethered cord determine prognosis
20(4):923-38, 2000
for deterioration 9. Tortori-Donati Pet al: Spinal dysraphism: a review of
o Neurological deterioration suggests complication neuroradiological features with embryological correlations
• Tethering by scar or second (unrecognized) and proposal for a new classification. Neuroradiology.
malformation ~ most common 42(7):471-91, 2000
• Constricting post-operative dural ring 10. Barkovich A: Pediatric Neuroimaging. 3rd ed. Philadelphia:
• Cord compression by epidermoid/dermoid tumor Lippincott-Raven, 627-34, 2000
or arachnoid cyst 11. Shurtleff DB et al: Epidemiology of tethered cord with
meningomyelocele. Eur] Pediatr Surg 7 suppll: 7-11, 1997
• Cord ischemia 12. Shaw GM et al: Risk of neural tube defect-affected
• Syringohydromyelia (29-77%) pregnancies among obese women. ]AMA. 275(14):1093-6,
• Chiari II malformation.is the most common cause of 1996
death in MMC patients 13. MeLone DG et al: Complications of myelomeningocele
closure. Pediatr Neurosurg. 17(5):267-73, 1991-92
Treatment
• Folate supplementation to pregnant/conceiving
women
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MYELOMENINGOCELE
IIMAGE GALLERY 1
13
Typical
(Left) Sagittal T2WI MR
displays the typical
post-operative appearance
following MMC closure, with
patulous sac, tethered cord,
and skin covering. (Right)
Coronal ultrasound real time
in utero shows dorsal open
dysraphism with complex
cystic myelomeningocele
(arrows) (Courtesy A.
Kennedy, MD).
Variant
(Left) Sagittal T2WI MR
depicts a variant
post-operative appearance
following MMC closure.
Atrophic distal cord is
low-lying with terminal syrinx
(arrow) (Courtesy C.
Hedlund, DO). (Right)
Sagittal T2WI MR in a patient
with repaired lumbosacral
myelomeningocele reveals
severe caudal regression in
conjunction with atrophic
tethered cord (Courtesy C.
Hedlund, DO).
Variant
(Left) Sagittal T2WI MR
shows a sacral
myelomeningocele
herniating through a wide
dysraphic defect. Sac is not
skin covered (Courtesy R.
Boyer, Mo). (Right) Sagittal
T7WI MR reveals a thoracic
myelomeningocele with
dysmorphic cord and syrinx
covered by skin (following
operative closure).
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LIPOMYELOMENINGOCELE
1
14
Sagittal graphic shows a low-lying conus with syrinx Sagittal T7WI MR displays typical features of LMMC
terminating in a large dorsallipomyelomeningoce/e. with tethered spinal cord terminating in a large lipoma
contiguous with subcutaneous fat through a large
dysraphic defect.
DDx: lipomyelomeningocele
.. ... .
•
.
.to" 0
~
."
j
/1'
Terminal Lipoma Intradural Lipoma Dorsal Meningocele
...
Myelomeningocele
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LIPOMYELOMENINGOCELE
Key Facts
1
Terminology Pathology 15
• Synonyms: LMMC, lipomyelocele (LMC), • Cord always tethered
lipomyeloschisis • Lipoma may be asymmetric (40%), rotating placode
and elongating roots on one side, shortening on
Imaging Findings other
• Best diagnostic clue: Subcutaneous fatty mass
contiguous with neural placode/lipoma through Clinical Issues
posterior dysraphism • Soft midline or paramedian skin covered mass above
• Ventral neural placode/tethered cord +/- terminal buttocks
hydromyelia, cord myeloschisis • Cutaneous stigmata (50%); hemangioma, dimple,
dermal sinus, skin tag, hairy patch
Top Differential Diagnoses
• Terminal lipoma Diagnostic Checklist
• Intradural (juxtamedullary) lipoma • Determination of re-tethering following surgery
• Dorsal meningocele primarily clinical diagnosis; use imaging to search for
• Myelocele/myelomeningocele complications
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LIPOMYELOMENINGOCELE
1 Gross Pathologic & Surgical Features IDIAGNOSTIC CHECKLIST
16 • Canal enlarged at placode, spina bifida level
Consider
• Lipoma may envelop both dorsal and ventral nerve
roots, dorsal nerve roots only, or filum • Presence/absence of placode rotation important
terminale/conus pre-operative information for surgeon
• Dura deficient at spina bifida zone ~ does not close Image Interpretation Pearls
neural tube • Determination of re-tethering following surgery
o Attached laterally to neural placode, posterior to primarily clinical diagnosis; use imaging to search for
dorsal nerve roots as they emerge from cord complications
• Lipoma may be asymmetric (40%), rotating placode
and elongating roots on one side, shortening on other
o Predisposes to operative injury, incomplete tether ISELECTED REFERENCES
release
1. McNeely PD et al: Ineffectiveness of dietary folic acid
Microscopic Features supplementation on the incidence of
• Dorsal placode surface adjacent to lipoma has no lipomyelomeningocele: pathogenetic implications. J
ependymal lining; covered by connective tissue mixed Neurosurg. 100(2):98-100, 2004
2. Schoenmakers MA et al: Long-term outcome of
with islands of glial cells, smooth muscle fibers neurosurgical untethering on neurosegmental motor and
ambulation levels. Dev Med Child Neurol. 45(8):551-5,
2003
j CLINICAL ISSUES 3. Tortori-Donati P et al: Magnetic resonance imaging of
spinal dysraphism. Top Magn Reson Imaging.
Presentation 12(6):375-409,2001
• Most common signs/symptoms: Back/leg pain, 4. Bulsara KR et al: Clinical outcome differences for
scoliosis, lower extremity paraparesis, sacral sensory lipomyelomeningoceles, intraspinal lipomas, and lipomas
loss, limb atrophy, orthopedic foot deformity, of the filum terminale. Neurosurg Rev. 24(4):192-4, 2001
5. Lee JH et al: Combined anomaly of intramedullary
bladder/bowel dysfunction
arteriovenous malformation and lipomyelomeningocele.
• Clinical profile AJNR Am J Neuroradiol. 21(3):595-600, 2000
o Soft midline or paramedian skin covered mass above 6. Barkovich AJ: Pediatric Neuroimaging. 3rd ed.
buttocks Philadelphia, Lippincott, Williams, and Wilkins. 641-646,
• Lumbosacral mass: Clinical attention within 6 2000
months 7. Kim SY et al: Prenatal diagnosis of lipomyelomeningocele.
• No mass: Present when neurological deficits J Ultrasound Med. 19(11):801-5,2000
manifest (5 years ~ adulthood) 8. Cochrane DD et al: The patterns of late deterioration in
o Cutaneous stigmata (50%); hemangioma, dimple, patients with transitionallipomyelomeningocele. Eur J
Pediatr Surg. 10 Suppl 1:13-7, 2000
dermal sinus, skin tag, hairy patch 9. Iskandar BJ et al: Congenital tethered spinal cord
Demographics syndrome in adults. J Neurosurg. 88(6):958-61, 1998
10. Wu HY et al: Long-term benefits of early neurosurgery for
• Age: Infancy (most common) ~ adulthood lipomyelomeningocele. J Urol. 160(2):511-4, 1998
• Gender: F > M 11. Colak A et al: Recurrent tethering: a common long-term
problem after lipomyelomeningocele repair. Pediatr
Natural History & Prognosis Neurosurg. 29(4):184-90, 1998
• Potentially irreversible progressive neurological 12. Ball W: Pediatric Neuroradiology. Philadelphia,
impairment (cord tethering, enlarging lipoma) Lippincott-Raven, 724-725, 1997
o Lipoma grows with infant 13. Chreston J et al: Sonographic detection of
o Bladder dysfunction usually persists if not operated lipomyelomeningocele: a retrospective documentation. J
early Clin Ultrasound. 25(1):50-1, 1997
14. Sutton LN: Lipomyelomeningocele. Neurosurgery Clinics
• ~ 45% of children neurologically normal at diagnosis of North America 6:325-38, 1995
o 16-88% develop neurological symptoms if left 15. Kanev PM et al: Reflections on the natural history of
untreated lipomyelomeningocele. Pediatr Neurosurg. 22(3):137-40,
o Most symptomatic patients progress if untreated 1995
• Neurologically intact patients at surgery usually 16. Herman JM et al: Analysis of 153 patients with
remain intact on long term follow-up myelomeningocele or spinal lipoma reoperated upon for a
• Post-operative exam should not deteriorate with tethered cord. Presentation, management and outcome.
longitudinal growth; neurological decline raises Pediatr Neurosurg. 19(5):243-9, 1993
17. Kanev PM et al: Management and long-term follow-up
suspicion of re-tethering, warrants repeat imaging
review of children with lipomyelomeningocele, 1952-1987.
• Symptomatic re-tethering common; weeks to years J Neurosurg. 73(1):48-52, 1990
after initial surgery
o Median time between initial procedure and
reoperation for re-tethering ~ 52 months
Treatment
• Early prophylactic surgery « 1 year of age) to untether
cord, resect lipoma, and reconstruct dura
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LIPOMYELOMENINGOCELE
IIMAGE GALLERY 1
17
Typical
(Left) Sagittal NECT
following myelography
shows a dilated dural sac
and low-lying cord
terminating in a large
hypodense lipoma (arrow).
Subcutaneous fat is
contiguous with lipoma
through spina bifida. (Right)
Anteroposterior myelography
depicts splitting
("myeloschisis") of a
low-lying spinal cord within
the dilated distal dural sac.
Typical
(Left) Sagittal TTWI MR
shows a lipomyelocele with
tethered cord terminating in
a large lipoma contiguous
with subcutaneous fat
(Courtesy R. Boyer, Mo).
(Right) Axial T2WI MR in
LMMC reveals dilation of the
distal dural sac, with
low-lying cord (arrow)
terminating in lipoma
contiguous with
subcutaneous fat through
dorsal dysraphism.
Variant
(Left) Sagittal TT WI MR
shows a tethered cord
terminating in a
Iipomye/omeningocele.
Dural sac protrudes through
dysraphic defect with lipoma
to merge into subcutaneous
fat (Courtesy C. Hedlund,
00). (Right) Axial TTWI MR
depicts a
lipomyelomeningoce/e with
rotated neural placode, dural
sac, and lipoma protruding
through dorsal defect into
subcutaneous tissues
(Courtesy C. Hedlund, 00).
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SPINAL LIPOMA
1
18
Axial graphic shows incomplete closure of the dorsal Sagittal TlWI MR shows a fatty intradural mass
spinal cord around a dorsal juxtamedullary conus encircling the conus! producing focal canal
lipoma, encompassing the dorsal roots. enlargement. A small amount of fat is also present
within the filum terminale (arrow).
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SPINAL LIPOMA
Key Facts
1
Terminology • Arise from premature separation (dysjunction) of 19
• Spinal lipoma intimately associated with spinal cord cutaneous ectoderm from neuroectoderm during
(intradural) or distal cord/filum insertion (terminal) neurulation
o Intradural lipoma
• Lobulated ovoid/rounded intradural hyperintense Filum fibrolipoma
mass intimately associated with cord • Common; 4-6% population, mostly asymptomatic but
• +/- Canal widening, local dysraphism symptomatic patients present with "tethered cord"
• i Signal intensity on fat saturation sequences • Hyperintense/hypodense mass in filum +/- tethering,
o Terminal lipoma low-lying conus
• Hyperintense mass attached to distal cord/filum; Dermoid cyst
extends through lumbosacral dysraphism ~
• Mixed density/signal intensity mass; lack of
subcutaneous fat homogeneous hyperintensity +/- dermal sinus help to
• Thin, "stretched" cord usually tethered +/- syrinx distinguish
• T2WI
o Similar signal intensity, imaging appearance to
TlWI
I PATHOLOGY
o +/- Spinal cord compression (intradural) ~
hyperintense cord signal General Features
• STIR: i Signal intensity confirms fat • General path comments
• T1 C+: No lipoma enhancement o Three main types of spinal lipomas: Intradural,
Ultrasonographic Findings lipomyelo(meningo)cele/terminallipoma, and filum
• Real Time: Echogenic intraspinal mass +/- reduced lipoma
o Composed of normal fat
conus motion
• Fat cells t in size considerably during infancy; tiny
Other Modality Findings lipomas in neonates may grow substantially
• Sagittal PC MRI: Decreased conus motion ~ tethered during infancy
cord • Conversely, lipomas i in size if patient loses
weight
Imaging Recommendations • Etiology
• Best imaging tool: MR imaging o Arise from premature separation (dysjunction) of
• Protocol advice cutaneous ectoderm from neuroectoderm during
o Ultrasound in infants for screening; confirm with neurulation
MRI if positive • Surrounding mesenchyme enters ependyma-lined
o Sagittal, axial T1WI to define lipoma(s) extent and central spinal canal, impeding closure of neural
relationship to neural placode, adjacent tissues folds ~ open placode
• Image through tip of thecal sac to avoid missing • Mesenchyme differentiates into fat
fibrolipoma or terminal lipoma • Similar mechanism ~ dermal sinus tract; explains
their frequent association
• Epidemiology
IDIFFERENTIAL DIAGNOSIS o Intradural lipoma (4%)
• < 1% of primary intraspinal tumors
Lipomyelocele/lipomyelomeningocele o Lipomyelo(meningo)cele (includes terminal lipoma)
• Skin covered (closed) neural placode-lipoma complex (84%)
contiguous with subcutaneous fat through dysraphic o Filum lipoma (12%)
defect • Associated abnormalities
• Mass often palpable +/- cutaneous stigmata
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SPINAL LIPOMA
1 o Intradural lipoma: +/- Localized dysraphism at
lipoma level; segmentation anomalies rare Natural History & Prognosis
20 o Terminal lipoma: Sacral hypogenesis, anorectal • Small lipomas may grow dramatically during infancy
malformations, genitourinary (GU) malformations • Symptomatic patients unlikely to improve
(5-10%), terminal diastematomyelia, epidermoid, spontaneously without intervention
dermal sinus, angioma, arachnoid cyst o Caveat: Lipomas may shrink if patient loses weight
• Sacral anomalies much more likely if GU, Treatment
anorectal malformations present (;:0: 90%) • Surgical resection, untethering of cord (if applicable)
Gross Pathologic & Surgical Features • Weight loss may be conservative management method
• Intradural lipoma to avoid surgery in highly selected obese patients
o Partially encapsulated sessile (55%) or exophytic
(45%) juxtamedullary fatty mass entirely enclosed
within dural sac I DIAGNOSTIC CHECKLIST
o Midline spinal cord "open"; subpial lipoma nestled
Consider
between open lips
• Follow-up even small lipomas in neonates; they may
• Terminal lipoma
o Delicately encapsulated fatty mass attached to
grow significantly!
cord/filum; frequently contiguous with Image Interpretation Pearls
subcutaneous fat through dorsal lumbosacral • Profound hypodensity on CT myelography and T1WI
dysraphism hyperintensity distinctively characteristic of lipoma
• Rotation uncommon (unlike LMMC) • Use chemical fat-saturation or inversion recovery MR
o Cord almost always tethered; stretched and thinned technique to confirm fat content
+/- hydrosyringomyelia (20%)
Microscopic Features
• Homogeneous mass of mature fat separated into ISELECTED REFERENCES
globules by strands of fibrous tissue 1. Bulsara KR et al: Clinical outcome differences for
o +/- Calcification, ossification, muscle fibers, nerves, lipomyelomeningoceles, intraspinal lipomas, and lipomas
glial tissue, arachnoid, ependyma of the filum terminale. Neurosurg Rev. 24(4):192-4, 2001
2. Tortori-Donati P et al: Magnetic resonance imaging of
spinal dysraphism. Top Magn Reson Imaging.
12(6):375-409, 2001
ICLINICAL ISSUES 3. Arai H et al: Surgical experience of 120 patients with
lumbosacral lipomas. Acta Neurochir (Wien).
Presentation 143(9):857-64,2001
• Most common signs/symptoms 4. van Leeuwen R et al: Surgery in adults with tethered cord
o Cervical, thoracic intradural lipoma: Slow ascending syndrome: outcome study with independent clinical
mono- or paraparesis, spasticity, cutaneous sensory review. J Neurosurg. 94(2 Suppl):205-9, 2001
loss, deep sensory loss 5. Barkovich A: Pediatric neuroimaging. 3rd ed. Philadelphia,
Lippincott-Raven. 636-640, 2000
o Lumbosacral intradural lipoma: Flaccid lower
6. Unsinn KM et al: US of the spinal cord in newborns:
extremity paralysis, sphincter dysfunction spectrum of normal findings, variants, congenital
o Terminal lipoma: Bowel/bladder dysfunction, lower anomalies, and acquired diseases. Radiographies.
extremity weakness/sensory abnormality, foot 20(4):923-38,2000
deformity, scoliosis 7. Iskandar BJ et al: Congenital tethered spinal cord
o Symptoms may be exacerbated by pregnancy syndrome in adults. J Neurosurg. 88(6):958-61, 1998
• Clinical profile 8. Endoh M et al: Spontaneous shrinkage of lumbosacral
o Intradural lipoma: Patient complains of weakness lipoma in conjunction with a general decrease in body fat:
case report. Neurosurgery. 43(1):150-1; discussion 151-2,
and sensory anomalies referable to lesion level
1998
• Overlying skin usually looks normal; no 9. Ball W: Pediatric Neuroradiology. Philadelphia,
cutaneous stigmata Lippincott-Raven, 723-724, 1997
o Terminal lipoma: Patient presents with clinical 10. Byrne RW et al: Operative resection of 100 spinal lipomas
appearance of "Tethered cord syndrome" and in infants less than 1 year of age. Pediatr Neurosurg.
(frequently) cutaneous stigmata 23(4):182-6; discussion 186-7, 1995
11. Fujiwara F et al: Intradural spinal lipomas not associated
Demographics with spinal dysraphism: a report of four cases.
• Age Neurosurgery. 37(6):1212-5, 1995
o 3 age peaks for presentation 12. Aoki N: Rapid growth of intraspinal lipoma demonstrated
by magnetic resonance imaging. Surg Neurol. 34(2):107-10,
• < 5 years (24%)
1990
• 2nd ~ 3rd decades (55%)
• 5th decade (16%)
• Gender
o Intradural: M :0; F
o Terminal: M < F
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SPINAL LIPOMA
IIMAGE GALLERY 1
21
(Left) Sagittal Tl WI MR
shows an intradural lipoma
(arrows) adherent to the
dorsal cervical spinal cord
(Courtesy G. Hedlund, DO).
(Right) Sagittal TlWI MR
depicts a large intradural
thoracic lipoma which
invaginates into the dorsal
cord surface and remodels
the central spinal canal
(Courtesy R. Boyer, MO).
Typical
(Left) Sagittal TlWI MR
demonstrates a large
intradural juxtamedullary
conus lipoma with low-lying
conus at the L3 level
(Courtesy G. Hedlund, DO).
(Right) Lateral ultrasound
real time shows an
echogenic dorsal mass
(arrows) representing a
juxtamedullary lipoma
tethering the conus tip (open
arrow) at L3 (Courtesy G.
Hedlund, DO).
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.. '
. ~ ~. ~~
~"-""'-
. J0·~J
"-J l' ·v
Anteroposterior radiography shows incomplete fusion of Axial bone CT depicts incomplete fusion of the 51
the 51 posterior ring. A small ossicle resides within the posterior elements. Ossicle (arrow) within defect is an
osseous defect (arrow). occasionally noted variant.
ITERMINOlOGY MR Findings
Abbreviations and Synonyms • TlWI
o Normal dural sac, conus position, and filum
• Synonym: "Spina bifida occulta" thickness
Definitions o No abnormal neural, dural, or lipomatous tissue
• Spinous process/lamina fusion failure without within posterior osseous defect
underlying neural or dural abnormality • T2WI: Same as T1WI
Ultrasonographic Findings
• Real Time: Posterior element defect; normal conus
I IMAGING FINDINGS level and periodic motion
General Features Imaging Recommendations
• Best diagnostic clue: Incomplete lumbosacral posterior • Best imaging tool
element fusion o Plain films most economical screening tool
• Location: Lumbosacral junction (L5 > Sl) > > cervical o MR best for definitive exclusion of significant
(C1 > C7 > Tl), thoracic underlying abnormality
• Size: Defect usually small • Protocol advice
• Morphology: Posterior elements do not fuse in o Ultrasound for infants with sacral dimples or other
midline; margins are rounded, well-corticated, and cutaneous stigmata
may overlap o Sagittal and axial T1WI, T2WI best screen for
Radiographic Findings tethered cord, neural anomalies
• Radiography: Unfused spinous process/lamina
approximate at midline
CT Findings
• NECT: No abnormal neural, dural, or lipomatous
tissue within posterior osseous defect
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Key Facts
1
Terminology • Location: Lumbosacral junction (L5 > Sl) > > cervical 23
• Synonym: "Spina bifida occulta" (C1 > C7 > Tl), thoracic
• Spinous process/lamina fusion failure without • Normal dural sac, conus position, and filum thickness
underlying neural or dural abnormality • No abnormal neural, dural, or lipomatous tissue
within posterior osseous defect
Imaging Findings
• Best diagnostic clue: Incomplete lumbosacral
Top Differential Diagnoses
posterior element fusion • Closed spinal dysraphism (CSD)
• Surgical laminectomy defect
• Normal progression of ossification
Microscopic Features
• Histologically normal corticated bone IIMAGE GALLERY
ICLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Usually asymptomatic if no cutaneous stigmata
o Occasionally skin dimple on back
• Dimples within gluteal cleft almost always
pilonidal sinuses; need no further evaluation
• Dimples above gluteal cleft have higher incidence
of associated abnormalities; merit further
evaluation with MRI
• Clinical profile: Patient presents with low back or leg (Left) Axial T1 WI MR reveals incidental incomplete posterior fusion
pain; finding is incidentally noted during imaging (arrow). Osseous margins are rounded and well-corticated. (Right)
Anteroposterior radiography depicts incomplete fusion of the 57
Demographics laminae. This variation demonstrates rotation and overlap of the
• Age: All ages; more common < 40 years of age posterior elements.
• Gender: F > M
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DORSAL DERMAL SINUS
1
24
Sagittal graphic shows a dermal sinus extending from Sagittal TlWI MR shows a typical dermal sinus (black
skin surface into spinal canal to terminate in an arrow) entering at L4/5 and coursing cranially (white
epidermoid. A skin dimple with capillary angioma and arrows), terminating at the conus. A Vitamin E capsule
hairy tuft marks sinus opening. marks the skin opening.
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Key Facts
1
Terminology Pathology 25
• Synonyms: DST, dermal sinus tract • Focal incorporation of cutaneous ectoderm into
neural ectoderm during disjunction at a
Imaging Findings circumscribed point only ~ focal segmental adhesion
• Best diagnostic clue: Hypointense sinus tract
superimposed on hyperintense subcutaneous fat Clinical Issues
• Osseous findings range from normal ~ groove in • Progressive neurological deterioration from cord
lamina/spinous process ~ multilevel dysraphism tethering, (epi)dermoid enlargement, mass effect on
• Dorsal dural tenting indicates ~ dural penetration spinal cord or cauda equina, sequelae of
• Lumbosacral sinus ~ tethered cord, low-lying conus meningitis/abscess
• Thoracic, cervical sinuses ~ normal conus position
Diagnostic Checklist
Top Differential Diagnoses • Dorsal dermal sinus must be differentiated from
• Low sacrococcygeal midline dimple simple sacral dimple or pilonidal sinus
• Pilonidal sinus • Maintain a high index of suspicion for all dimples
• (Epi)dermoid tumor without dermal sinus above intergluteal fold
o +/- Tethered cord • Small « 5 mm), low « 2.5 cm from anus), extend
• Lumbosacral sinus ~ tethered cord, low-lying inferiorly or horizontally toward coccyx
conus • Usually no associated masses, other cutaneous
• Thoracic, cervical sinuses ~ normal conus stigmata
position
Pilonidal sinus
• T2WI
o Sinus tract hypointense to subcutaneous fat • Common; nearly always incidental
o +/- Hyperintense (epi)dermoid cyst • Low ostium, do not enter spinal canal
o +/- Nerve root clumping (adhesive arachnoiditis) (Epi)dermoid tumor without dermal sinus
• DWI: +/- Hyperintense epidermoid cyst • No skin stigmata or sinus tract
• Tl C+: +/- Intra/extramedullary abscess, infectious or
chemical arachnoiditis
Ultrasonographic Findings IPATHOLOGY
• Real Time General Features
o Shows entire length of tract from skin to spinal cord
• Subcutaneous tract slightly hypoechoic, hard to • General path comments
detect o Three clinically important types of sinus tracts
• Echogenic subarachnoid tract clearly • Low sacral or coccygeal dermal sinuses form
demonstrated within anechoic CSF embryologically differently; always terminate in
o +/- Low-lying conus, thick filum, j nerve mobility, sacral or coccygeal fascia and never extend into
intrathecal mass subarachnoid space
• Pilonidal sinus: Low ostium, do not enter spinal
Other Modality Findings canal
• Sagittal phase contrast cine MRI • Congenital dorsal dermal sinus + atypical dimple
o Loss of normal conus pulsation (large (> 5 mm), remote from anus (> 2.5 cm),
o Increased contrast between free-flowing CSF, solid combined with other lesions)
epidermoid tumor o Midline dimple/pit is one of most common referrals
to pediatric neurosurgeons
Imaging Recommendations • Regardless of depth, dimples below top of
• Best imaging tool: MR imaging intergluteal crease end blindly and never extend
• Protocol advice intraspinally
o Sagittal and axial T1WI, T2WI • Sinus opening dermatomallevel correlates with
• Adjust window/level to best delineate metameric cord level of attachment
subcutaneous tract • Etiology
o Ultrasound supplements MR in infants < 1 year; use o Focal incorporation of cutaneous ectoderm into
MR to confirm positive ultrasound study neural ectoderm during disjunction at a
circumscribed point only ~ focal segmental
adhesion
I DIFFERENTIAL DIAGNOSIS • Spinal cord ascends relative to spinal canal,
stretches adhesion into a long, tubular tract
low sacrococcygeal midline dimple • Epidemiology
• 2-4% of infants o Low sacrococcygeal dimple: 2-4% of all infants
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Variant
(Left) Sagiltal TlWI MR
demonstrates a thoracic
dermal sinus tract (arrow)
terminating in an intradural
dermoid tumor. The dura is
tented outward at point of
sinus tract entry. (Right)
Axial T2WI MR reveals a
double "tram track" sinus
tract (arrows) terminating in
a hyperintense intradural
dermoid tumor. The sinus
tractenters the dural sac
through a dysraphic defect.
Variant
(Left) Sagittal TlWI MR
depicts a dermoid tumor
(black arrows) within the
spinal canal, filling the sinus
tract nearly to the cutaneous
opening (white arrow) and
tethering the low-lying conus
(open arrow). (Right) Axial
TI WI MR reveals a
heterogeneous intraspinal
dermoid tumor associated
with dermal sinus tract.
Hyperintense tumor
elements reflect fat content.
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Sagittal TlWI MR shows a hypointense intradural Sagittal TlWI MR shows a mixed signal intensity
epidermoid within the cauda equina that displaces intra-extradural dermoid that tethers the spinal cord and
nerve roots. Arrows demarcate rostral and caudal expands outward into a dermal sinus (skin opening
extent. indicated by arrow).
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Key Facts
1
Terminology Top Differential Diagnoses 29
• Synonym: (Epi)dermoid cyst • Arachnoid cyst
• Neurenteric cyst
Imaging Findings
• 40% intramedullary, 60% extramedullary; extradural Pathology
rare • Congenital (100% dermoid, 60% epidermoid)
• Dermoid: Well-demarcated isodense mass +/- regions • Acquired (40% epidermoid)
of fat hypodensity, calcification
• Epidermoid: Well-circumscribed hypodense mass Clinical Issues
with attenuation similar to CSF +/- calcification (rare) • Slowly progressive compressive
• Dermoid: Tl hypo- to hyperintense signal intensity radiculopathy/myelopathy
mass Diagnostic Checklist
• Epidermoid: Usually T1 isointense to CSF, • Diagnosis requires high index of suspicion; look for
occasionally mildly hyperintense mass effect on regional structures
• DWI: Distinguishes epidermoid (hyperintense) from • FLAIR and DWI may distinguish epidermoid from
arachnoid cyst (isointense to CSF) CSF, arachnoid cyst
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DERMOID AND EPIDERMOID TUMORS
1 Gross Pathologic & Surgical Features
• Radiotherapy not established for the treatment of
epidermoid cysts
30 • Epidermoid o May be an alternative to palliative operation or for
o Striking white pearly sheen capsule; may be smooth, patients who cannot undergo surgery
lobulated, or nodular
o Cyst filled with creamy, waxy, pearly material
o Either easy to shell out or firmly affixed to regional
structures (result of local inflammation)
IDIAGNOSTIC CHECKLIST
• Dermoid Consider
o Well-demarcated smooth mass; wall may be • Dermoid is a congenital lesion and presents during
thickened by skin appendages or calcifications childhood/adolescence
o Cyst filled with thick cheesy, buttery, yellowish • Epidermoid may be congenital or acquired, and
material usually arises later during 3rd to 5th decade
Microscopic Features Image Interpretation Pearls
• Epidermoid • Epidermoids/dermoids are frequently difficult to
o Outer connective tissue capsule lined with stratified diagnosis on CT and MRI
squamous epithelium; calcification rare o Diagnosis requires high index of suspicion; look for
o Centrally contains desquamated epithelial keratin, mass effect on regional structures
cholesterol crystals; positive staining with antibodies o FLAIR and DWI may distinguish epidermoid from
to EMA and cytokeratin CSF, arachnoid cyst
• Dermoid
o Uni- or multilocular; outer connective tissue capsule
lined with stratified squamous epithelium ISELECTED REFERENCES
containing hair follicles, sebaceous glands, and
1. Ziv ET et ali Iatrogenic intraspinal epidermoid tumor: two
sweat glands cases and a review of the literature. Spine. 29(1):EI5-8,
o +/- Inflammation (if ruptured) 2004
o Centrally contains desquamated epithelial keratin, 2. Bretz A et ali Intraspinal epidermoid cyst successfully
lipid material treated with radiotherapy: case report. Neurosurgery.
53(6):1429-31; discussion 1431-2, 2003
3. Ferrara P et ali Intramedullary epidermoid cyst presenting
ICLINICAL ISSUES with abnormal urological manifestations. Spinal Cord.
41(11):645-8,2003
Presentation 4. Amato VG et ali Intramedullary epidermoid cyst:
preoperative diagnosis and surgical management after MRI
• Most common signs/symptoms introduction. Case report and updating of the literature. J
o Asymptomatic Neurosurg Sci. 46(3-4):122-6, 2002
o Slowly progressive compressive 5. Graham D et ali Greenfield's Neuropathology. 7th ed.
radiculopathy/myelopathy London, Arnold. 964-966, 2002
o Other signs/symptoms 6. Scarrow AM et ali Epidermoid cyst of the thoracic spine:
• Cauda equina syndrome case history. Clin Neurol Neurosurg. 103(4):220-2,2001
• Acute chemical meningitis secondary to rupture 7. Barkovich A: Pediatric neuroimaging. 3rd ed. Philadelphia,
and discharge of inflammatory cholesterol crystals Lippincott-Raven. 669-70, 2000
8. Kikuchi K et ali The utility of diffusion-weighted imaging
into CSF with navigator-echo technique for the diagnosis of spinal
• Meningitis; most common in association with epidermoid cysts. AJNR Am J Neuroradiol. 21(6):1164-6,
dermal sinus 2000
9. Potgieter S et ali Epidermoid tumours associated with
Demographics lumbar punctures performed in early neonatal life. Dev
• Age Med Child Neurol. 40(4):266-9, 1998
o Dermoids usually cause symptoms before 20 years of 10. Machida T et ali Acquired epidermoid tumour in the
age thoracic spinal canal. Neuroradiology. 35(4):316-8, 1993
o Epidermoids are slower growing; symptoms usually 11. Toro VE et ali MRI of iatrogenic spinal epidermoid tumor. J
arise in early adult hood (3rd to 5th decade) Comput Assist Tomogr. 17(6):970-2, 1993
12. Lunardi P et ali Long-term results of the surgical treatment
• Gender of spinal dermoid and epidermoid tumors. Neurosurgery.
o Dermoids M = F 25(6):860-4, 1989
o Epidermoids M > F 13. Shikata J et ali Intraspinal epidermoid and dermoid cysts.
Surgical results of seven cases. Arch Orthop Trauma Surg.
Natural History & Prognosis 107(2):105-9, 1988
• Symptoms slowly progress if untreated 14. Phillips J et ali Magnetic resonance imaging of intraspinal
• Complete surgical resection offers best opportunity for epidermoid cyst: a case report. J Comput Tomogr.
good neurologic outcome 11(2):181-3, 1987
o Incomplete resection frequently recurs; malignant
transformation very rare
Treatment
• Standard treatment is complete surgical excision
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Typical
(Left) Coronal TI WI MR
depicts a small intradural
dermoid tumor with intrinsic
hyperintense signal from fat
(arrow). (Right) Sagittal
NECT shows a mixed
intradural/extradural
dermoid tumor with osseous
remodeling producing a
large palpable sacral mass
(arrows) in a patient with
Currarino triad (Courtesy C.
Hedlund, DO).
Variant
(Left) Sagitta!. TlWI MR
shows a low-lying tethered
cord with filum lipoma,
ending in a large sacral
dermoid tumor. The dermoid
displays mixed isointense
and hyperintense (fat) signal
intensity. (Right) Sagittal
TlWI MR reveals a large
hypointense extradural
epidermoid tumor. Fat
capping at the poles
(arrows) confirms epidural
location (Courtesy M.
Huckman, MO).
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Sagittal T2WI MR shows wedge-shaped conus Sagittal T1WI MR demonstrates a low-lying conus
termination and sacral hypogenesis, characterisUc of terminating in a large lipoma (arrow). These findings in
group 1 CRS patients. conjunction with sacral hypogenesis are typical of group
2 CRS patients.
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CAUDAL REGRESSION SYNDROME
Key Facts
1
Terminology • Occult intrasacral meningocele (aIM) 33
• Synonyms: CRS, sacral agenesis, lumbosacral Pathology
dysgenesis • Most severe cases ~ sirenomelia ("mermaid")
Imaging Findings • Most cases sporadic
• Best diagnostic clue: Lumbosacral dysgenesis with • 1/7,500 births (milder forms> severe forms)
abnormal distal spinal cord • 15-20% are infants of diabetic mothers; 1% of
• Spectrum ranging in severity from absent coccyx to offspring from diabetic mothers affected
lumbosacral agenesis • Genitourinary abnormalities (24%)
• Group 1: Blunt spinal cord termination above L1; Diagnostic Checklist
central canal may be prominent • MR imaging most useful imaging tool for
• Group 2: Elongated conus with thick filum +/- characterizing abnormalities and surgical planning
intraspinal lipoma • Caudal spine anomalies should be sought out in
Top Differential Diagnoses patients with genitourinary or anorectal anomalies
• Tethered spinal cord and vice versa
• Closed spinal dysraphism
o Group 1: Blunt spinal cord termination above L1; o Lower extremity deformities, lumbosacral ageneSiS,
central canal may be prominent anorectal abnormalities, renal/pulmonary
o Group 2: Elongated conus with thick filum +/- hypoplasia characteristic
intraspinal lipoma • Most severe cases ~ sirenomelia ("mermaid")
• 20% ~ tethering subcutaneous lesions (group 2)
Imaging Recommendations • Genetics
• Consider ultrasound for infant screening o Most cases sporadic
• MR imaging to confirm ultrasound findings, treatment o Dominantly inherited form recently described;
planning defect in the HLBX9 homeobox gene (chromosome
o Sagittal MRI to demonstrate extent of lumbosacral 7)
deficiency, distal spinal cord morphology, and • HLBX9 also expressed in pancreas ~ possible
presence/absence of tethering association between diabetes hyperglycemia and
o Axial MRI to detect osseous spinal narrowing, caudal regression
hydromyelia, other associated lesions • Etiology
o Normal caudal spine development ~ canalization
and retrogressive differentiation
IDIFFERENTIAL DIAGNOSIS • Anorectal and genitourinary structures form
contemporaneously in close anatomic proximity
Tethered spinal cord o Insult prior to fourth gestational week ~ caudal cell
• Low-lying spinal cord +/- thickened or fatty filum, no mass developmental abnormalities
caudal dysgenesis • Hyperglycemia, infectious, toxic, or ischemic
• Difficult to clinically discern from mild sacral insult postulated to impair spinal cord, vertebral
dysgenesis formation
o +/- Associated imaging abnormalities may help • Signaling defects by retinoic acid and sonic
distinguish hedgehog during blastogenesis and gastrulation
Closed spinal dysraphism • Abnormal neural tube, notochord development ~
• Dorsal dysraphism without severe vertebral column impaired migration of neurons and mesodermal
agenesis cells
• Ex: Lipomyelomeningocele (lipomyeloschisis) • Epidemiology
o 1/7,500 births (milder forms> severe forms)
Occult intrasacral meningocele (aIM) o 15-20% are infants of diabetic mothers; 1% of
• Sacrum thinned and remodelled, sometimes imitating offspring from diabetic mothers affected
caudal regression o Association with VACTERL (10%), omphalocele,
exstrophy bladder, imperforate anus, spinal
anomaluies (10%), and Currarino triad syndromic
!PATHOLOGY complexes
• Associated abnormalities
General Features o Tethered cord
• General path comments • ;::; 100% of CRS patients with conus terminating
o Sequela of caudal cell mass dysplasia with spectrum below L1
of severity • Thickened filum (65%) +/- dermoid or lipoma
o Other spinal anomalies
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CAUDAL REGRESSION SYNDROME
1 • Vertebral anomalies (22%), diastematomyelia,
terminal hydromyelia (10%), myelomeningocele
• Level of vertebral aplasia correlates with motor but
not sensory level
34 (35-50%), lipomyelomeningocele (10-20%),
terminal myelocystocele (15%), anterior sacral Demographics
meningocele • Age
o Congential cardiac defects (24%), pulmonary o Severe cases identified in utero (obstetrical
hypoplasia ultrasound) or at birth
o Genitourinary abnormalities (24%) o Mild cases may not be identified until adulthood
• Renal agenesis/ectopia, hydronephrosis, Mullerian • Gender: M = F
duct malformations, urinary bladder Natural History & Prognosis
malformation
• Variable depending on severity
o Anorectal anomalies (particularly anal atresia)
• Higher level of anal atresia =} more severe Treatment
lumbosacral dysgenesis, genitourinary anomalies • Surgical untethering if clinically symptomatic
o Orthopedic abnormalities; extreme cases =} lower • Surgical release/duraplasty may improve neurological
extremity fusion (sirenomelia) function in patients with distal spinal canal stenosis
Gross Pathologic & Surgical Features • Orthopedic procedures to improve lower extremity
functionality
• Severity of vertebral dysgenesis, presence/absence of
tethering, and osseous canal diameter impact surgical
planning
IDIAGNOSTIC CHECKLIST
Microscopic Features
• Findings typical for tissue content
Consider
• MR imaging most useful imaging tool for
Staging, Grading or Classification Criteria characterizing abnormalities and surgical planning
• Group 1: More severe caudal dysgenesis with
high-lying, club-shaped cord terminus (decreased
Image Interpretation Pearls
number of anterior horn cells) • Caudal spine anomalies should be sought out in
o Distal cord hypoplasia with wedging seen in all patients with genitourinary or anorectal anomalies
patients with partial or complete dysgenesis and and vice versa
termination of spinal cord above L1
o Termination of the conus above L1 highly correlated
with sacral malformations ending at Sl or above ISELECTED REFERENCES
• Group 2: Less severe dysgenesis with low-lying, 1. Nievelstein RA et al: Magnetic resonance imaging in
tapered, distal cord tethered by tight filum, lipoma, children with anorectal malformations: embryologic
lipomyelomeningocele, or terminal myelocystocele implications.] Pediatr Surg. 37(8):1138-45, 2002
2. Barkovich A: Pediatric neuroimaging. 3rd ed. Philadelphia,
o Conus termination below L1 highly correlated with
Lippincott-Raven, 650-53, 2000
sacral malformations ending at S2 or below 3. Tortori-Donati P et al: Spinal dysraphism: a review of
o Tethering is thus more common in milder sacral neuroradiological features with embryological correlations
dysgenesis and proposal for a new classification. Neuroradiology
42(7): 471-91, 2000
4. Catala M: Embryogenesis. Why do we need a new
ICLINICAL ISSUES explanation for the emergence of spina bifida with lipoma?
Childs Nerv Syst. 13(6):336-40, 1997
Presentation 5. Ball W: Pediatric Neuroradiology. Philadelphia,
Lippincott-Raven, 735-36, 1997
• Most common signs/symptoms Heij HA et al: Abnormal anatomy of the lumbosacral
6.
o Neurogenic urinary bladder dysfunction (nearly all region imaged by magnetic resonance in children with
patients) anorectal malformations. Arch Dis Child. 74(5):441-4,
o Sensorimotor paresis (group 2 > group 1) 1996
• Severity of motor deficit> sensory 7. Long FR et al: Tethered cord and associated vertebral
• Sacral sensory sparing common, even in severe anomalies in children and infants with imperforate anus:
cases evaluation with MR imaging and plain radiography.
o Neurologically asymptomatic (group 1 > group 2) Radiology. 200(2):377-82, 1996
8. Nievelstein RA et al: MR of the caudal regression
• Clinical profile syndrome: embryologic implications. A]NR Am]
o Broad spectrum ranging from neurologically normal
Neuroradiol. 15(6):1021-9, 1994
=} complete lumbosacral agenesis and fusion of
9. Pang D: Sacral agenesis and caudal spinal cord
lower extremities (sirenomelia) malformations. Neurosurgery 32(5): 755-78, discussion
• Always accompanied by narrow hips, hypoplastic 778-9,1993
gluteal muscles, and shallow intergluteal cleft 10. Barkovich A] et al: The wedge-shaped cord terminus: a
o Symptomatic patient presentation ranges from mild radiographic sign of caudal regression. A]NR Am]
foot disorders =} complete lower extremity paralysis Neuroradiol. 10(6):1223-31, 1989
and distal leg atrophy
• Motor level usually higher than sensory level
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IIMAGE GALLERY 1
35
Typical
(Left) Sagittal T2WI MR
shows a low-lying, tethered
conus and mild sacral
hypogenesis (four sacral
vertebrae). Findings are
diagnostic of mild group 2
CRS (Courtesy R. Boyer,
MO). (Right) Sagittal T7WI
MR demonstrates a small
lipoma at the filum terminus
(arrow), in conjunction with
low-lying tethered conus and
mild sacral hypogenesis (four
sacral vertebrae).
Variant
(Left) Sagittal T2WI MR in a
CRS patient following
myelomeningocele repair
shows wedge-shaped conus
(group 1) and severe sacral
dysgenesis. (Right) Coronal
T7 WI M R depicts marked
pelvic hypogenesis (group 1
CRS).
Variant
(Left) Anteroposterior
radiography shows left
hemisacral caudal dysgenesis
(Courtesy R. Boyer, MO).
(Right) Anteroposterior
radiography depicts marked
pelvic hypogenesis and
pubic deficiency correlating
with clinical bladder
exstrophy and lower
extremity
under-development
(Courtesy C. Hedlund, DO).
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Sagittal graphic shows a low-lying hydromyelic tethered Sagittal Tl WI M R shows an elongated, attenuated spinal
cord with fibrolipoma inserting into a terminal lipoma. cord terminating directly into a small terminal lipoma
Lipoma is conuguous with subcutaneous fat through (arrow). No distinguishable filum is detected.
dorsal dysraphism.
ITERMINOlOGY CT Findings
• NECT: Stretched, thinned cord with low-lying conus,
Abbreviations and Synonyms
thickened filum +/- fibrolipoma, dysraphism, vertebral
• Synonyms: "Tethered cord syndrome (TCS)", "tight segmentation and fusion anomalies
filum terminale syndrome"
MR Findings
Definitions
• TIWI
• Symptoms and imaging findings referable to low-lying o Thickened filum +/- hyperintense lipoma
conus medullaris tethered by a short, thick filum o Filum> 2 mm (L5-S1, axial MRI)
terminale
o +/- Low-lying conus; may be difficult to distinguish
from thickened filum
o Dorsal positioning of conus medullaris, filum
IIMAGING FINDINGS terminale in thecal sac
General Features • Noted even in prone position; normally cord falls
• Best diagnostic clue: Conus ends below L2 inferior into anterior 2/3 of canal when prone
endplate; tethered by thickened filum +/- fibrolipoma, • T2WI
o Findings similar to Tl WI
terminal lipoma
o +/- Hyperintense dilatation of conus central canal 2°
• Location: Thoracolumbar junction ~ sacrum
to hydromyelia or myelomalacia (25%)
• Size: Filum thickened (> 2 mm at L5-S1, axial MRI)
o Fatty filum ~ chemical shift artifact
• Morphology: Attenuated conus terminates lower than
o Dural sac widened; dorsal dura tense, tented
normal
posteriorly by thickened filum
Radiographic Findings
Ultrasonographic Findings
• Radiography
o May be normal, but nearly always shows localized • Real Time: +/- Low-lying conus, thickened filum
dysraphism or incomplete posterior fusion terminale, reduced or absent spinal cord movement
o +/- Scoliosis (20%)
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Key Facts
1
Terminology Pathology 37
• Synonyms: "Tethered cord syndrome (TCS)", "tight • Widened dural sac; thickened filum closely applied to
filum terminale syndrome" or indistinguishable from dural sac
• Symptoms and imaging findings referable to
low-lying conus medullaris tethered by a short, thick Clinical Issues
filum terminale • Low back and leg pain; worst in morning,
exacerbated by exertion
Imaging Findings • Urinary bladder dysfunction
• Best diagnostic clue: Conus ends below L2 inferior
endplate; tethered by thickened filum +/- Diagnostic Checklist
fibrolipoma, terminal lipoma • Tethered cord syndrome is a clinical diagnosis;
• Size: Filum thickened (> 2 mm at L5-S1, axial MRI) imaging role is detection of low-lying conus/thick
filum, associated anatomic abnormalities for surgical
Top Differential Diagnoses decision making
• Normal variant low-lying conus • Measure filum thickness at L5/S1; stretching at more
• Open or closed spinal dysraphism rostral levels may erroneously thin filum into
• Post-surgical low-lying conus "normal" size range
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TETHERED SPINAL CORD
1 o Low back and leg pain; worst in morning,
ISELECTED REFERENCES
exacerbated by exertion
38 o Gait spasticity, weakness, muscular atrophy 1. Yamada et al: Neurosurg Focus 16(2), 2004
o j Sensation, abnormal lower extremity reflexes 2. Haro H et al: Long-term outcomes of surgical treatment for
o Urinary bladder dysfunction tethered cord syndrome. J Spinal Disord Tech. 17(1):16-20,
2004
o Orthopedic foot abnormalities (usually clubfoot) 3. Selcuki M et al: Is a filum terminale with a normal
o Cutaneous stigmata (::s: 50%); simple dimple, hairy appearance really normal? Childs Nerv Syst. 19(1):3-10,
tuft, or hemangioma 2003
• Clinical profile 4. Unsinn KM et al: US of the spinal cord in newborns:
o Adults and children present differently spectrum of normal findings, variants, congenital
• Adults: Pain first (2° degenerative changes), anomalies, and acquired diseases. Radiographics.
followed later by weakness +/- incontinence 20(4):923-38, 2000
• Children: Incontinence, scoliosis, weakness 5. Barkovich A: Pediatric neuroimaging. 3rd ed. Philadelphia,
Lippincott-Raven. 648-650, 2000
Demographics 6. Yamada S et al: Adult tethered cord syndrome. ] Spinal
Disord. 13(4):319-23,2000
• Age: Symptomatic presentation most common during 7. Selcuki M et al: Management of tight filum terminale
rapid somatic growth (adolescent growth spurt, school syndrome with special emphasis on normal level conus
age 4 to 8 years), or in elderly 2° kyphosis medullaris (NLCM). Surg Neurol. 50(4):318-22; discussion
• Gender: M = F 322, 1998
8. Souweidane MM et al: Retethering of sectioned
Natural History & Prognosis fibrolipomatous filum terminales: report of two cases.
• Progressive, irreversible neurological impairment Neurosurgery. 42(6):1390-3, 1998
o Majority of patients show improvement or 9. Iskandar B] et al: Congenital tethered spinal cord
stabilization of neurological deficits following syndrome in adults.] Neurosurg. 88(6):958-61, 1998
surgical untethering; motor weakness (12-60%), 10. Sharif Set al: "Tethered cord syndrome"--recent clinical
sensory dysfunction (40-60%), pain (50-88%), experience. Br] Neurosurg. 11(1):49-51, 1997
11. Yundt KD et al: Normal diameter of filum terminale in
bladder dysfunction (19-67%) children: in vivo measurement. Pediatr Neurosurg.
o Better outcome if symptom duration shorter or 27(5):257-9, 1997
conus moves to more normal level following surgery 12. Beek FJ et al: Sonographic determination of the position of
• Post-operative symptom recurrence rare; prompts the conus medullaris in premature and term infants.
consideration for re-tethering Neuroradiology. 38 Suppll:SI74-7, 1996
13. Gilmore RL et al: The clinical neurophysiology of tethered
Treatment cord syndrome and other dysraphic syndromes, in Yamada
• Symptomatic patients: Early prophylactic surgery S (ed): Tethered Cord Syndrome. Park Ridge, IL: AANS,
o Resect tethering mass, release cord, and repair dura 167-182, 1996
• Asymptomatic patients with radiographic tethering: 14. Beek FJ et al: Sonographic determination of the position of
the conus medullaris in premature and term infants.
Management controversial Neuroradiology. 38 Suppll:SI74-7, 1996
o Some advocate prophylactic surgery =} low 15. Brunelle F et al: Lumbar spinal cord motion measurement
morbidity, prognosis for asymptomatic patients with phase-contrast MR imaging in normal children and in
better than symptomatic children with spinal lipomas. Pediatr Radiol. 26(4):265-70,
o Others advocate prophylactic surgery only for 1996
asymptomatic adults who lead physically active 16. Yamada S et al: Pathophysiology of tethered cord
lifestyles syndrome. Neurosurg Clin N Am. 6(2):311-23, 1995
17. Warder DE et al: Tethered cord syndrome: the low-lying
and normally positioned conus. Neurosurgery.
34(4):597-600; discussion 600, 1994
I DIAGNOSTIC CHECKLIST 18. Warder DE et al: Tethered cord syndrome and the conus in
a normal position. Neurosurgery. 33(3):374-8, 1993
Consider 19. Chestnut R et al: The Vater association and spinal
• Clinical tethering may occur despite normal conus dysraphia. Pediatr Neurosurg. 18(3):144-8, 1992
level 20. Raghavan N et al: MR imaging in the tethered spinal cord
o Tethered cord syndrome is a clinical diagnosis; syndrome. A]R Am] Roentgenol.152(4):843-52, 1989
imaging role is detection of low-lying conus/thick 21. Wilson DA et al: John Caffey award. MR imaging
filum, associated anatomic abnormalities for surgical determination of the location of the normal conus
medullaris throughout childhood. AJR Am J Roentgenol.
decision making 152(5):1029-32, 1989
• Distinguish cord tethering 2° tight/thickened filum 22. Kang JK et al: Effects of tethering on regional spinal cord
terminale from tethering due to other lesions blood flow and sensory-evoked potentials in growing cats.
Childs Nerv Syst. 3(1):35-9, 1987
Image Interpretation Pearls 23. Pang D et al: Tethered cord syndrome in adults. J
• Measure filum thickness at L5/S1; stretching at more Neurosurg. 57(1):32-47, 1982
rostral levels may erroneously thin filum into "normal"
size range
• Determine conus level using axial images; cauda
equina may obscure conus tip on sagittal images =}
imitate elongated conus
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(Left) Sagittal T1 WI M R
depicts a low-lying, tethered
conus with thickened fatty
filum (arrow). (Right) Axial
T1WI MR shows a small
filum lipoma in conjunction
with filum thickening in a
symptomatic TCS patient.
Typical
(Left) Axial T2WI MR of TCS
patient demonstrates
chemical shift artifact
(arrow) confirming fat
content in thickened filum.
(Right) Sagittal T2WI MR
shows a low-lying conus at
U with thickened filum and
dorsal positioning of tethered
cord related to dermal sinus
tract (Courtesy C. Hedlund,
00).
Typical
(Left) Sagittal ultrasound real
time depicts a low-lying
conus (arrow) with
echogenic thickening of the
filum (open arrows)
corresponding to filum
lipoma. Conus movement
was reduced (Courtesy R.
Boyer, Mo). (Right) Sagittal
T1WI MR reveals a low-lying
conus at U, with thickened
fatty filum. The cord/conus is
displaced dorsally (Courtesy
R. Boyer, Mo).
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TERMINAL MYELOCYSTOCELE
1
40
Sagittal graphic displays a low-lying, hydromyelic spinal Sagittal TlWI MR depicts a low-lying hydromyelic cord
cord piercing an expanded subarachnoid space traversing meningocele, expanding into a large terminal
(meningocele), to terminate in a myelocystocele. cyst. Arrow marks dorsal fibrous band (Courtesy R.
Boyer, MO).
Y•
,..'lc. .- .
.
" .
-
..
11 _ _ . ,t. ;:JI
~:
Ant. Meningocele Dorsal Meningocele Sacrococc. Teratoma Myelomeningocele
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TERMINAL MYELOCYSTOCELE
Key Facts
1
Terminology Pathology 41
• Synonym: Terminal syringocele • Skin covered lumbosacral dysraphism
• Complex spinal malformation ~ hydromyelic • Arachnoid lined meningocele directly contiguous
low-lying tethered spinal cord traversing a with spinal subarachnoid space
meningocele to terminate in myelocystocele • Low-lying hydromyelic cord traverses meningocele,
expands into large contiguous ependymal-lined
Imaging Findings terminal cyst
• Best diagnostic clue: Hydromyelic tethered cord
traversing dorsal meningocele to terminate in a Clinical Issues
dilated terminal ventricle cyst • Presents at birth with large skin-covered back mass
• Lumbosacral dysraphism • Majority neurologically intact at diagnosis
Top Differential Diagnoses Diagnostic Checklist
• Anterior sacral meningocele • Early diagnosis and surgery ~ best chance for normal
• Simple dorsal meningocele neurological outcome
• Sacrococcygeal teratoma • Non-neurological prognosis largely linked to severity
• Myelomeningocele of associated anomalies
o Axial plane: Bifid hydromyelic dorsal spinal cord • Low-lying hydromyelic cord traverses
within meningocele meningocele, expands into large contiguous
ependymal-lined terminal cyst
Imaging Recommendations o Terminal cyst does not communicate directly with
• Best imaging tool: MRI subarachnoid space
• Protocol advice o Some speculate ~ most severe manifestation of
o Sagittal MR imaging for diagnosis and estimating persistent terminal ventricle (ventriculus terminalis)
length of hydromyelia, sizing cysts, and identifying spectrum
associated abnormalities • Etiology
o Axial MR imaging to clarify extent of rachischisis, o Postulated to result from deranged secondary
evaluate associated anomalies neurulation of caudal cell mass; typically associated
with other caudal tail-fold malformations
• CSF unable to normally exit from early neural
I DIFFERENTIAL DIAGNOSIS tube ~ terminal ventricle balloons into cyst ~
disrupts overlying dorsal mesenchyme but not
Anterior sacral meningocele superficial ectoderm
• Anterior meningocele protrudes thorough an enlarged • Posterior elements do not form normally ~ spina
sacral neural foramen into pelvis bifida with intact skin
Simple dorsal meningocele • Terminal ventricle dilatation ~ distends
• Dorsal meningocele protrudes thorough focal arachnoid cord lining ~ meningocele
dysraphism • Cyst bulk prevents cord ascent ~ tethered cord
• Cord rarely tethered or hydromyelic • After arachnoid formation, progressive distention
of distal cord causes caudal bulge below end of
Sacrococcygeal teratoma meningocele into extra-arachnoid space and
• Similar clinical appearance of skin mass cephalically to expand distal cord, producing
• Distinguish by presence of solid tumor elements flaring of distal spinal cord
within cysts, calcifications • Disruption of caudal motor segments ~
progressive symptoms present at birth or that
Myelomeningocele appear later
• Open spinal dysraphism; no skin covering, clinically • Epidemiology
obvious o Rare: 1-5% of skin covered lumbosacral masses
• Much more common in cloacal exstrophy patients
o Sporadic; no familial incidence
IPATHOLOGY o Postulated associations with teratogens retinoic acid,
hydantoin, ioperamid hydrochloride
General Features o No known association with diabetes mellitus (unlike
• General path comments caudal regression syndrome)
o Classic pathological triad • Associated abnormalities
• Skin covered lumbosacral dysraphism o Cloacal exstrophy, imperforate anus, omphalocele,
• Arachnoid lined meningocele directly contiguous pelvic deformities, equinovarus, ambiguous
with spinal subarachnoid space hypoplastic genitalia/and renal abnormalities
o Syndromal associations
• Caudal regression syndrome
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TERMINAL MYELOCYSTOCELE
1 • OElS syndrome constellation (omphalocele,
exstrophy of the bladder, imperforate anus, and
o Operation soon after diagnosis is indicated to
prevent progreSSion of neurological abnormalities,
42 spinal anomalies) cyst growth
o Chiari I and II malformations, hydrocephalus, and o Persistent neurological deficits usually permanent
vertebral segmentation anomalies (rare) • Overall prognosis is mainly related to other associated
anomalies (OEIS constellation)
Gross Pathologic & Surgical Features
• Lumbosacral dysraphism with hypoplastic, widely Treatment
everted laminae • Early diagnosis and surgical repair maximizes
• Proximal (smaller, rostral) sac resembles typical probability of a normal neurological outcome
meningocele, with inner surface lined by arachnoid • Delayed recognition and operation increase odds of
and thick fibrous layer onset, progression of lower extremity paresis
• Distal spinal cord herniates under fibrous band
between medial ends of most cephalic widely bifid
lamina, traverses meningocele ~ terminal cyst (larger, IDIAGNOSTIC CHECKLIST
caudal)
o Cord narrowed by fibrous band where it exits spinal Consider
canal, then widens distally due to concurrent • Early diagnosis and surgery ~ best chance for normal
hydromyelia neurological outcome
o Distal spinal nerve roots arise from ventral surface of • Non-neurological prognosis largely linked to severity
intra-arachnoid segment of spinal cord, traverse of associated anomalies
meningocele, and re-enter spinal canal before
Image Interpretation Pearls
exiting at their root sleeves or via bony clefts
• Tethered hydromyelic cord traversing meningocele to
Microscopic Features terminate in a separate caudal cyst is unique imaging
• Meningocele lined by arachnoid, thick fibrous layer finding diagnostic of terminal myelocystocele
• Terminal cyst lined by ependyma, dysplastic glia;
directly contiguous with central cord canal
• Outer cord surface pia, arachnoid continuous with ISELECTED REFERENCES
meningocele 1. Lemire RJ et al: Tumors and malformations of the caudal
spinal axis. Pediatr Neurosurg. 38(4):174-80, 2003
2. Kumar R et al: Terminal myelocystocele. Indian J Pediatr.
ICLINICAL ISSUES 69(12):1083-6,2002
3. Midrio P et al: Prenatal diagnosis of terminal
Presentation myelocystocele in the fetal surgery era: case report.
• Most common signs/symptoms Neurosurgery. 50(5):1152-4; discussion 1154-5, 2002
4. Unsinn KM et al: US of the spinal cord in newborns:
o Presents at birth with large skin-covered back mass spectrum of normal findings, variants, congenital
• Skin appears normal or exhibits hemangioma, anomalies, and acquired diseases. Radiographies.
nevus, or hypertrichosis 20(4):923-38, 2000
• Rare patients without back mass present later with 5. Cartmill M et al: Terminal Myelocystocele: an unusual
progressive neurological deficits presentation. Pediatr Neurosurg. 32(2):83-5, 2000
o Majority neurologically intact at diagnosis 6. Barkovich A: Pediatric neuroimaging. 3rd ed. Philadelphia,
o Later presentation or untreated lesion may develop Lippincott-Raven, 653-654, 2000
progressive lower extremity paresis 7. Tortori-Donati P et al: Spinal dysraphism: a review of
neuroradiological features with embryological correlations
• Clinical profile and proposal for a new classification. Neuroradiology
o Usually neurologically intact at birth but may 42(7): 471-91, 2000
present with lower extremity sensorimotor deficits 8. Choi S et al: Long-term outcome of terminal
o Large mass obliterates intergluteal cleft, extends myelocystocele patients. Pediatr Neurosurg. 32(2):86-91,
upward from perineum for a variable distance 2000
o +/- Concurrent midline cecal and paramedian 9. Meyer SH et al: Terminal myelocystocele: important
bladder exstrophy, other visible anomalies differential diagnosis in the prenatal assessment of spina
bifida.] Ultrasound Med. 17(3):193-7, 1998
Demographics 10. Byrd SE et al: Imaging of terminal myelocystoceles. ] Natl
• Age: Infancy Med Assoc. 88(8):510-6, 1996
11. Byrd SE et al: MR of terminal myelocystoceles. Eur] Radiol.
• Gender: F > M 20(3):215-20, 1995
Natural History & Prognosis 12. Peacock W] et al: MagnetiC resonance imaging in
myelocystoceles. Report of two cases. ] Neurosurg.
• Normal intellectual potential 70(5):804-7, 1989
• Mass size and deficits tend to progress with time, may 13. MeLone DG et al: Terminal myelocystocele. Neurosurgery.
be partially or totally reversible with prompt surgical 16(1):36-43, 1985
repair 14. Lemire R] et al: Skin-covered sacrococcygeal masses in
o Main goals of neurosurgical intervention are to infants and children. J Pediatr. 79(6):948-54, 1971
reduce mass size and un-tether cord
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TERMINAL MYELOCYSTOCELE
IIMAGE GALLERY 1
43
Variant
(Left) Coronal T7 WI MR
shows a low-lying spinal
cord (arrows) traversing a
large dorsolateral
meningocele that atypically
extends into the left flank
(Courtesy R. Boyer, MO).
(Right) Axial CECT following
myelography shows wide
posterior dysraphism (black
arrows). The spinal cord
(white arrow) traverses a
large eccentric dorsolateral
meningocele (Courtesy R.
Boyer, MO).
Variant
(Left) Lateral myelography
demonstrates the
hydromyelic low-lying cord
(open arrow) passing
through the dorsal
meningocele (white arrows)
to terminate in a small
caudal terminal cyst (black
arrow). (Right)
Anteroposterior myelography
shows the meningocele's
(white arrows) relationship
to wide dysraphism and
terminal cyst (black arrow).
Surgical wires indicate prior
bladder exstrophy repair.
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ANTERIOR SACRAL MENINGOCELE
1
44
Sagittal graphic (left) depicts characteristic anterior Sagittal T2WI MR shows a large anterior sacral cyst,
sacral "scimitar" remodeling by large anterior cyst. contiguous with the meninges, extending through an
Coronal graphic (right) shows cyst origin through an enlarged neural foramen (Courtesy C. Hedlund, 00).
enlarged neural foramen.
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ANTERIOR SACRAL MENINGOCELE
Key Facts
1
Terminology • Neurenteric cyst 45
• Anterior sacral meningocele (ASM) • Cystic neuroblastoma
• Sacral meninges herniate anteriorly into the pelvis Pathology
through focal erosion or hypogenesis of sacral +/- • S% of retrorectal tumors
coccygeal vertebral segments • Associated abnormalities: Anorectal anomalies,
Imaging Findings epidermoid/dermoid tumor or other tethering lesion,
• Best diagnostic clue: Presacral cyst, contiguous with Currarino triad
thecal sac, protruding through an anterior osseous Diagnostic Checklist
defect • MR imaging to identify and characterize cyst location
• Widened sacral canal and neural foramina and contents, confirm abnormal ultrasound findings
• Curved "scimitar" sacrum on lateral projection • Continuity of cyst with thecal sac necessary to ensure
Top Differential Diagnoses diagnosis
• Sacrococcygeal teratoma (SGT) • Soft tissue mass or calcification implies tumor
• Sacral chordoma • Presence/absence of neural tissue within cyst
• Ovarian cyst important for surgical planning
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ANTERIOR SACRAL MENINGOCELE
1 Microscopic Features
3. Clatterbuck RE, Jackman Sv, Kavoussi LR, Long DM.
Laparoscopic treatment of an anterior sacral meningocele. J
46 • Characteristic features of dural sac tissue; may reveal Neurosurg (Spine). 92: 246, 2000
signs of prior inflammation 4. Shamoto H et al: Anterior sacral meningocele completely
occupied by an epidermoid tumor. Childs Nerv Syst.
15(4):209-11, 1999
ICLINICAL ISSUES 5. Fitzpatrick MO, Taylor WAS. Anterior sacral meningocele
associated with a rectal fistula. Case report and review of
Presentation the literature. J Neurosurg (Spine). 91: 124-127, 1999
6. Voyvodic F et al: Anterior sacral meningocele as a pelVic
• Most common signs/symptoms complication of Marfan syndrome. Aust N Z J Obstet
o Constipation, urinary frequency, incontinence, Gynaecol. 39(2):262-5, 1999
dysmenorrhea, dyspareunia, low back/pelvic pain 7. Ball W: Pediatric Neuroradiology. Philadelphia,
• 2° to pressure on pelvic viscera Lippincott-Raven, 737, 1997
o Other signs/symptoms 8. Lee SC et al: Currarino triad: anorectal malformation,
• Sciatica, diminished rectal/detrusor tone, sacral bony abnormality, and presacral mass--a review of 11
cases. J Pediatr Surg. 32(1):58-61, 1997
numbness/paresthesias in lower sacral
9. Gaskill SJ et al: The Currarino triad: its importance in
dermatomes (nerve root pressure) pediatric neurosurgery. Pediatr Neurosurg. 25(3):143-6,
• Intermittent positional high or low pressure 1996
headaches due to fluid shifts between ASM and 10. Kochling J et al: The Currarino syndrome--hereditary
spinal subarachnoid space transmitted syndrome of anorectal, sacral and presacral
• Superinfection +/- meningitis (uncommon) anomalies. Case report and review of the literature. Eur J
• Clinical profile Pediatr Surg. 6(2):114-9, 1996
o Most present with urinary or bowel complaints; 11. Funayama CA et al: Recurrent meningitis in a case of
congenital anterior sacral meningocele and agenesis of
ASM is discovered during imaging evaluation
sacral and coccygeal vertebrae. Arq Neuropsiquiatr.
o Rare finding in headache patient 53(4):799-801, 1995
12. Raftopoulos C et al: Anterior sacral meningocele and
Demographics Marfan syndrome: a review. Acta Chir Belg. 93(1):1-7, 1993
• Age: Onset of symptoms in 2nd to 3rd decade 13. Chamaa MT et al: Anterior-sacral meningocele; value of
• Gender magnetic resonance imaging and abdominal sonography.
o M = F (children) A case report. Acta Neurochir (Wien). 109(3-4):154-7, 1991
o M < F (adults) 14. North RB et al: Occult, bilateral anterior sacral and
intrasacral meningeal and perineurial cysts: case report and
Natural History & Prognosis review of the literature. Neurosurgery. 27(6):981-6, 1990
• Good prognosis following successful repair IS. McGuire RA Jr et al: Anterior sacral meningocele. Case
report and review of the literature. Spine. 15(6):612-4, 1990
Treatment 16. Brem H et al: Neonatal diagnosis of a presacral mass in the
• Open posterior trans-sacral approach, patching of presence of congenital anal stenosis and partial sacral
meningocele - dural sac connection agenesis. J Pediatr Surg. 24(10):1076-8, 1989
17. Lee KS et al: The role of MR imaging in the diagnosis and
o Easier if cyst does not contain neural elements,
treatment of anterior sacral meningocele. Report of two
permitting simple ligation of cyst neck cases. J Neurosurg. 69(4):628-31, 1988
• Endoscopic approach =} lower morbidity 18. Martin B et al: MR imaging of anterior sacral meningocele.
J Comput Assist Tomogr. 12(1):166-7, 1988
19. Dyck P et al: Anterior sacral meningocele. Case report. J
IDIAGNOSTIC CHECKLIST Neurosurg. 53(4):548-52, 1980
20. Sherman et al. Anterior Sacral Meningocele. Am J Surg
Consider 79:743-747,1950
• Ultrasound for infant screening 21. Bryant T. Case of deficiency of the anterior part of the
sacrum with a thecal sac in the pelvis, similar to the tumor
• MR imaging to identify and characterize cyst location of spina bifida. Lancet. 1: 258-360,1937
and contents, confirm abnormal ultrasound findings 22. Fiumara F et al: Purulent meningitis due to spontaneous
• Bone CT to demonstrate characteristic osseous anterior sacral meningocele perforation. Case report
findings that permit a specific diagnosis
Image Interpretation Pearls
• Continuity of cyst with thecal sac necessary to ensure
diagnosis
• Soft tissue mass or calcification implies tumor
• Presence/absence of neural tissue within cyst
important for surgical planning
ISELECTED REFERENCES
1. Rigante D et al: Anterior sacral meningocele in a patient
with Marfan syndrome. Clin Neuropathol. 20(2):70-2, 2001
2. Barkovich A: Pediatric neuroimaging. 3rd ed. Philadelphia,
Lippincott-Raven, 654-55, 2000
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ANTERIOR SACRAL MENINGOCELE
IIMAGE GALLERY 1
47
Variant
(Left) Sagittal T2WI MR
shows a multilocular
presacral cyst extending
through an enlarged neural
foramen into pelvis
(Courtesy R. Boyer, MO).
(Right) Axial T2WI MR with
chemical fat-saturation
reveals multicystic nature
(arrows) of this surgically
confirmed ASM.
Variant
(Left) Sagittal T2WI MR
reveals sacral hypogenesis
with anterior sacral
meningocele in a patient
with Currarino triad
(Courtesy R. Boyer, MO).
(Right) Sagittal T2WI MR
demonstrates classic
remodeled "scimitar"
sacrum and large anterior
presacral cyst (Courtesy G.
Hedlund,oO).
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OCCULT INTRASACRAL MENINGOCELE
1
48
Sagittal T2WI MR shows a typical occult intrasacral Axial T2WI MR depicts smooth enlargement of sacral
meningocele, with smooth remodeling and enlargement canal by an extradural cyst, with displacement of nerve
ofsacral canal. Arrow marks the superior margin ofcyst. roots anteriorly (arrows). No neural elements are
present within cyst.
ITERMINOLOGY CT Findings
Abbreviations and Synonyms • NECT
o CSF density cyst enlarges sacral canal
• Synonyms: Occult intrasacral meningocele (aIM), o Nerve roots displaced by extradural cyst
sacral meningeal cyst, type IB meningeal cyst • CECT: No cyst enhancement
Definitions • Bone CT
• Spinal extradural arachnoid cyst localized to sacrum o Smooth remodeling, enlargement of sacral canal
o No expansion or remodeling of neural foramina
MR Findings
IIMAGING FINDINGS • Tl WI: CSF signal intensity extradural sacral cyst
General Features adjacent to distal thecal sac
• Best diagnostic clue: Smooth, cystic enlargement of • T2WI
o Cyst follows CSF signal intensity
central sacral canal
o No neural elements within cyst
• Location: Sacrum
• DWI: Hypointense signal intensity excludes
• Size: Expands but does not transgress margins of
epidermoid tumor
sacrum
• Tl C+: No cyst enhancement
• Morphology: Sacral extradural meningeal cyst; no
internal neural elements Ultrasonographic Findings
Radiographic Findings • Real Time: Hypoechoic intrasacral cyst without
intrinsic neural elements
• Radiography: Sacral canal enlargement and posterior
vertebral scalloping Other Modality Findings
• Myelography • CSF cine phase contrast flow sensitive imaging may
o Enlargement of distal sacral canal, extradural help define cyst walls by depicting differential CSF
compression of distal contrast-filled thecal sac pulsation
• +/- Contrast opacification of cyst (if isthmus large
enough)
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ICLINICAL ISSUES
IPATHOLOGY
Presentation
General Features • Most common signs/symptoms
• General path comments o Asymptomatic; incidental discovery on MRI
o Extradural arachnoid cyst o Symptomatic: Chronic low back pain, sciatica,
• No herniation of meninges, hence not a true perineal paresthesias, and bladder dysfunction
meningocele o Less common signs/symptoms
• No neuronal elements within cyst • Intermittent, severe lower back pain
o Cyst is connected to thecal sac by a pedicle that • Atypical bowel symptoms, severe constipation,
permits contiguous CSF flow and stool incontinence
o CSF pulsation +/- raised intraspinal pressure (across • Tethered cord syndrome
stenotic pedicle) pressure erodes and remodels sacral • Clinical profile
canal
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IDIAGNOSTIC CHECKLIST
Consider
• MRI best modality for diagnosis of sacral cyst,
pre-operative planning
• Definitive cyst characterization based on operative
inspection, histological examination
Image Interpretation Pearls
• Classic appearance is an intrasacral cyst producing
smooth sacral canal expansion with outward
displacement of nerve roots
• aIM centered in midline; center over neural foramen
implies Tarlov cyst
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OCCULT INTRASACRAL MENINGOCELE
IIMAGE GALLERY 1
51
Typical
(Left) Sagittal T2WI MR
demonstrates large sacral
meningocele with extensive
remodeling of sacral canal.
(Right) Axial T2WI MR
shows marked enlargement
and remodeling of sacrum by
a large intrasacral
meningocele. No neural
elements reside within the
extradural cyst, and posterior
elements are intact.
Typical
(Left) Axial bone CT
follOWing myelography
shows a large intrasacral
meningocele with contrast
opacification indicating
contiguity with thecal sac.
Posterior elements are intact
but markedly thinned.
(Right) Axial NECT follOWing
myelography shows a large
extradural cyst with sacral
remodeling that anteriorly
displaces nerve root sleeves
(arrows). Mild contrast
uptake indicates contiguity
with thecal sac.
Variant
(Left) Sagittal T2WI MR
shows a large intrasacral
meningocele in conjunction
with low-lying tethered cord
and syringomyelia. Arrows
mark rostral and caudal cyst
margins. (Right) Axial TIWI
MR reveals an intrasacral
meningocele that anteriorly
displaces the bilateral sacral
nerve roots (black arrows).
Also noted is a left 51 Tarlov
cyst (open arrow).
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SACROCOCCYGEAL TERATOMA
1
52
Sagittal graphic depicts a typical large, heterogeneous, Sagittal T2WI MR shows a large (MP type II) mixed
partially cystic sacrococcygeal teratoma located anterior cysUc/solid tumor, with mostly solid internal portion and
to the sacrum, without osseous invasion. mostly cystic external portion (Courtesy R. Boyer, MO).
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SACROCOCCYGEAL TERATOMA
Key Facts
1
Terminology • Malignant risk t with age at diagnosis, higher surgical 53
• Synonyms: SGT, germ cell tumor of coccyx subtype, male gender, presence of necrosis or
hemorrhage
Imaging Findings • Epidemiology: 1:35,000 to 40,000 births
• Complex mixed attenuation cystic/solid pelvic mass
with enhancement of solid portions
Clinical Issues
• Calcifications (::; 60%); small punctate foci ~ well • Most common: Neonate with large benign sacral
formed teeth, bones tumor noted prenatally or at delivery
• Less common: Newborn with buttock asymmetry or
Top Differential Diagnoses delayed presentation at > 1 month with presacral
• Anterior sacral meningocele (ASM) tumor
• Chordoma
• Dermoid tumor
Diagnostic Checklist
• Exophytic rhabdomyosarcoma • AAP type influences prognosis, treatment approach
• Heterogeneous sacral tumor +/- calcification, cysts,
Pathology hemorrhage suggests diagnosis
• Most common newborn tumor
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SACROCOCCYGEAL TERATOMA
1 o Type I: Primarily external (47%) ~ best prognosis
o Type II: Dumbbell shape, equal external/internal
IDIAGNOSTIC CHECKLIST
54 portions (34%) Consider
o Type III: Primarily internal within abdomen/pelvis
• Imaging goals: Imperative to confirm diagnosis,
(9%) determine intra/extrapelvic extent and size,
o Type IV: Entirely internal; no visible external relationship to adjacent structures, and
component (10%) ~ worst prognosis presence/absence of metastatic disease
• AAP type influences prognosis, treatment approach
ICLINICAL ISSUES Image Interpretation Pearls
• Heterogeneous sacral tumor +/- calcification, cysts,
Presentation hemorrhage suggests diagnosis
• Most common signs/symptoms
o Back/pelvic mass in a newborn
• Exophytic masses easily diagnosed, but internal ISELECTED REFERENCES
(type III, IV) occult ~ delayed diagnosis
1. Hirose S et al: Fetal surgery for sacrococcygeal teratoma.
o In-utero presentation
Clin Perinatol. 30(3):493-506, 2003
• Polyhydramnios, high output cardiac failure with 2. Graf JL et al: Fetal sacrococcygeal teratoma. World J Surg.
hydrops, hepatomegaly, placentomegaly 27(1):84-6, 2003
o Urinary retention, constipation (larger tumors) 3. Axt-Fliedner R et al: Prenatal diagnosis of sacrococcygeal
• Clinical profile teratoma: a review of cases between 1993 and 2000. Clin
o Most common: Neonate with large benign sacral Exp Obstet Gynecol. 29(1):15-8, 2002
tumor noted prenatally or at delivery 4. Perrelli L et al: Sacrococcygeal teratoma. Outcome and
o Less common: Newborn with buttock asymmetry or management. An analysis of 17 cases. J Perinat Med.
30(2):179-84, 2002
delayed presentation at > 1 month with presacral Avni FE et al: MR imaging of fetal sacrococcygeal teratoma:
5.
tumor diagnosis and assessment. AJR Am J Roentgenol.
• Laboratory: Serum Ol- fetoprotein (AFP), B-human 178(1):179-83,2002
chorionic gonadotropin (HCG) levels post-operative 6. Monteiro M et al: Case report: sacrococcygeal teratoma
tumor markers with malignant transformation in an adult female: CT and
MRI findings. Br J Radiol. 75(895):620-3, 2002
Demographics 7. Paek BW et al: Radiofrequency ablation of human fetal
• Age: 50-70% diagnosed in-utero or during first days of sacrococcygeal teratoma. Am J Obstet Gynecol.
life, 80% by 6 months, and fewer than 10% beyond 2 184(3):503-7, 2001
years 8. Barkovich A: Pediatric neuroimaging. 3rd ed. Philadelphia,
Lippincott-Raven, 655, 2000
• Gender: M < F 4:1 9. Holterman AX et al: The natural history of sacrococcygeal
Natural History & Prognosis teratomas diagnosed through routine obstetric sonogram: a
single institution experience. J Pediatr Surg. 33(6):899-903,
• Prognosis excellent in benign tumors 1998
o Mature or mostly cystic ~ good prognOSiS 10. Ball W: Pediatric Neuroradiology. Philadelphia,
o Immature teratomas ~ greater risk of malignancy if Lippincott-Raven, 736-37, 1997
not totally resected 11. Schropp KP et al: Sacrococcygeal teratoma: the experience
• Malignant tumors have variable prognOSiS; later of four decades. J Pediatr Surg. 27(8):1075-8; discussion
diagnosis (> 1st birthday), internal location ~ worse 1078-9, 1992
prognosis 12. Wells RG et al: Imaging of sacrococcygeal germ cell tumors.
• Fetal mortality rate 20-65% (2° to polyhydramnios, Radiographics. 10(4):701-13, 1990
13. Noseworthy J et al: Sacrococcygeal germ cell tumors in
tumor hemorrhage, high-output cardiac failure) childhood: an updated experience with 118 patients. J
o Hydrops fetalis portends grim prognosis; > 30 weeks Pediatr Surg. 16(3):358-64, 1981
gestation ~ 25% mortality, < 30 weeks gestation ~ 14. Schey WL et al: Clinical and radiographic considerations of
93% mortality sacrococcygeal teratomas: an analysis of 26 new cases and
• Postpartum morbidity attributable to associated review of the literature. Radiology. 125(1):189-95, 1977
congenital anomalies, tumor mass effects, recurrence, 15. Altman RP et al: Sacrococcygeal teratoma: American
and intra-operative/post-operative complications Academy of Pediatrics Surgical Section Survey-1973. J
Pediatr Surg. 9(3):389-98, 1974
Treatment 16. Huddart SN et al: Sacrococcygeal teratomas: the UK
• Surgery alone curative if entire benign tumor and Children's Cancer Study Group's experience. I. Neonatal.
coccyx removed; recurs if coccyx not resected
• Cytoreduction surgery + radiation, chemotherapy may
be palliative for malignant tumors; early diagnosis,
resection best chance for cure
• Fetal surgery, (RFA) offered at some specialized centers;
good outcome reqUires careful patient selection
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SACROCOCCYGEAL TERATOMA
IIMAGE GALLERY 1
55
Typical
(Left) Sagittal T2WI MR
shows a predominately
cystic external (AAP /)
teratoma that displaces the
pelvic viscera rostrally
(Courtesy C. Hedlund, DO).
(Right) Anteroposterior
radiography shows a large
external sacral teratoma
(AAP type 7) (Courtesy R.
Boyer, MO).
· .. .r:".~··i'·
. .' J:.~ , f. t
(Left) Sagittal T7 C+ MR
reveals a predominately
internal (AAP III)
heterogeneous enhancing
'~i
Hedlund, DO).
;~.~~
Typical
4Wii
.(Left) Coronal ultrasound
real time of a fetus reveals a
mixed echogenicity
solid/cystic sacral mass
(Courtesy A. Kennedy, MO).
(Right) Sagittal T2WI MR
fetal MR depicts a
heterogeneous solid/cystic
sacral mass (AAP type II)
with concurrent
polyhydramnios (Courtesy A.
Kennedy, MO).
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DIASTEMATOMYELIA
1
56
Coronal graphic demonstrates type I SCM with large Coronal T7WI MR (Type I SCM) demonstrates an
osseous marrow-filled spur dividing syringomyelic ossified septum dividing the two hemicords (arrows).
tethered spinal cord into two hemicords contiguous Scoliosis and fusion of the posterior elements is also
with thoracic syrinx. apparent.
Type /I Diastem. Meningocele Manque High Hair Patch Low Hair Patch
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DIASTEMATOMYELIA
Key Facts
1
Terminology Pathology 57
• Synonyms: SCM, split cord malformation, "diastem" • "Split notochord syndrome" spectrum
• Sagittal division of spinal cord into two hemicords, • Epidemiology: 5% of congenital scoliosis
each with one central canal, dorsal horn, and ventral • Congenital spinal deformities (85%)
horn • Nerve roots may become adherent to dura and tether
cord =;> "meningocele manque"
Imaging Findings
• Location: Thoracolumbar cleft (85% between T9 and Clinical Issues
Sl) > > upper thoracic, cervical clefts • Cutaneous stigmata high on back indicates
• Hemicords usually reunite above and below cleft diastematomyelia level (> 50%); "fawn's tail" hair
• Split cord; one or two dural tubes patch most common
• Osseous spur often visible; fibrous spurs are
frequently occult Diagnostic Checklist
• Vertebral segmentation anomalies • Search for DSM in patients with cutaneous stigmata,
intersegmental fusion of posterior elements, clinical
Top Differential Diagnoses tethered cord
• Duplicated spinal cord (diplomyelia)
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DIASTEMATOMYELIA
1 o Bony or cartilaginous spur at inferior cleft, • ~ 90% of patients stabilize or improve following
originating from lamina or vertebral body surgery
58 o Vertebral anomalies more severe (block or butterfly o Caveat: Scoliosis rarely affected by surgical
vertebrae, hemivertebrae, and posterior spina bifida) untethering
o Hydromyelia common .
• Single dural tube, subarachnoid space (50%) Treatment
o No bony spur; nearly always have a fibrous band • Surgical tethered cord release, spur resection, and dural
coursing thru inferior cleft inserting into dura ~ repair for progressive symptoms, prophylactic
tethers cord precursor to scoliosis surgery
o Vertebral anomalies less severe (usually butterfly
vertebrae)
o Nerve roots may become adherent to dura and IDIAGNOSTIC CHECKLIST
tether cord =} "meningocele manque"
Consider
Microscopic Features • Scrutinize images for presence of spur; type I DSM
• Single ependymal lined central canal, ventral generally has more severe symptoms and anomalies,
horn/root, and dorsal horn/root per hemicord worse prognosis
• Search for DSM in patients with cutaneous stigmata,
Staging, Grading or Classification Criteria intersegmental fusion of posterior elements, clinical
• Pang type I SCM tethered cord
o Separate dural sac, 'arachnoid space surrounds each
hemicord Image Interpretation Pearls
o Osseous/fibrous spur • Segmentation anomalies with intersegmental laminar
o More commonly symptomatic than type II fusion virtually pathognomonic for diastematomyelia
• Pang type II SCM
o Single dural sac, arachnoid space
o No osseous spur; +/- adherent fibrous bands tether I SELECTED REFERENCES
cord 1. Balci S et al: Cervical diastematomyelia in
o Rarely present with symptoms unless hydromyelia, cervico-oculo-acoustic (Wildervanck) syndrome: MRI
tethering findings. Clin DysmorphoL 11(2):125-8,2002
2. Tortori-Donati P et al: Magnetic resonance imaging of
spinal dysraphism. Top Magn Reson Imaging.
ICLINICAL ISSUES 3.
12(6):375-409, 2001
Barkovich A: Pediatric neuroimaging. 3rd ed, Philadelphia,
Presentation Lippincott-Raven, 655-65, 2000
4. Balci S et al: Diastematomyelia in two sisters. Am J Med
• Most common signs/symptoms Genet. 86(2):180-2, 1999
o Clinically indistinguishable from other causes of 5. Ersahin Y et al: Split spinal cord malformations in children.
tethered cord J Neurosurg. 88(1):57-65, 1998
o Cutaneous stigmata high on back indicates 6. Frymoyer et al: The Adult Spine. Vol 2. 2nd ed.
diastematomyelia level (> 50%); "fawn's tail" hair Philadelphia, Lippincott-Raven. 1923-1926, 1997
patch most common 7. Miller A et al: Evaluation and treatment of
o Other signs/symptoms diastematomyelia. J Bone Joint Surg Am. 75(9): 1308-17,
1993
• Progressive kyphoscoliosis in older children, 8. Pang D et al: Split cord malformation: Part I: A unified
adults theory of embryogenesis for double spinal cord
• Orthopedic foot problems (50%); especially malformations. Neurosurgery. 31(3):451-80, 1992
clubfoot 9. Pang D et al: Split cord malformation: Part II: Clinical
• Urologic dysfunction syndrome. Neurosurgery 31(3): 481-500, 1992
• Clinical profile 10. Kaffenberger DA et al: Meningocele manque: radiologic
o Mild cases normal +/- cutaneous stigmata findings with clinical correlation. AJNR Am J NeuroradioL
o Severe cases ~ kyphoscoliosis, neurological and 13(4):1083-8, 1992
11. Naidich TP et al: Diastematomyelia: hemicord and
musculoskeletal abnormalities meningeal sheaths; single and double arachnoid and dural
Demographics tubes. AJNR Am J NeuroradioL 4(3):633-6, 1983
12. Harwood-Nash DC et al: Diastematomyelia in 172
• Age: Diagnosis in childhood; adult presentation children: the impact of modern neuroradiology. Pediatr
uncommon Neurosurg. 16(4-5):247-51, 1990-91
• Gender 13. Russell NA et al: Diastematomyelia in adults. A review.
o Pediatric: F > > M (80-94%) Pediatr Neurosurg. 16(4-5):252-7, 1990-91
o Adult: M < F 1:3.4
Natural History & Prognosis
• Stable or progressive disability if untreated
o Late onset or previously stable patients may become
symptomatic following relatively minor back injury
or surgery requiring spinal manipulation
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DIASTEMATOMYELIA
IIMAGE GALLERY 1
59
Typical
(Left) Axial bone CT
following myelography
shows dural tube division by
an osseous septum (type 7
SCM). The left lamina is
dysraphic. (Right) Axial
T2WI MR shows two
hemicords within individual
dural tubes, separated by an
osseous spur (type I SCM).
The left hemicord
demonstrates syringomyelia.
Variant
(Left) Axial 71* eRE MR
·shows a rare cervical type"
SCM. The two hemicords
share a single dural tube.
(Right) Sagittal T7WI MR
(type 7 SCM) depicts an
osseous spur (arrow) at L7
vertebral level that splits,
tethers cord. The conus
resides at L3 level.
Variant
(Left) Anteroposterior
radiography (type I SCM)
depicts widening of the
spinal canal, with a midline
osseous spur (arrows)
(Courtesy of R. Boyer, MO).
(Right) Axial T7 WI MR
depicts a thin fibrous septum
(open arrow) dividing two
hemicords (type" SCM). A
dorsal nerve root (arrow)
affixes right hemicord to the
dorsal dural sac
(meningocele manque).
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NEURENTERIC CYST
1
60
Sagittal graphic shows a large mediastinal enteric cyst Coronal bone CT (30 reformat) shows extensive
extending into the ventral spinal canal through a patent vertebral segmentation anomalies, multiple abnormal
canal of Kovalevsky to produce cord compression. fused ribs, and a large patent canal of Kovalevsky
(Courtesy S. Blaser; Mo).
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NEURENTERIC CYST
Key Facts
1
Terminology Pathology 61
• Synonyms: Spinal enterogenous cyst, spinal enteric • Single smooth unilocular (rarely multilocular) cyst
cyst, spinal dorsal-enteric cyst containing clear or proteinaceous fluid (milky,
cream-colored, yellowish, xanthochromic)
Imaging Findings • Demonstrable cyst connection with spinal cord,
• Best diagnostic clue: Intraspinal cyst + vertebral vertebrae, or both; dorsal spinal-enteric tract traverses
abnormalities (persistent canal of Kovalevsky, cartilage-lined canal of Kovalevsky through small
segmentation and fusion anomalies) dysplastic vertebra
• Location: Thoracic (42%) > cervical (32%) > > lumbar
spine, intracranial/basilar cisterns (rare) Clinical Issues
• Focal osseous canal enlargement, widening of • Back/radicular pain
interpedicular distance • Progressive paraparesis/paresthesias
Top Differential Diagnoses Diagnostic Checklist
• Arachnoid cyst • Degree of cord compression, size of cyst, and severity
• (Epi)dermoid cyst of associated anomalies determine disease course and
• Anterior thoracic meningocele prognosis
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NEURENTERIC CYST
1 Gross Pathologic & Surgical Features
o Drainage, partial resection if complete excision not
possible
62 • Single smooth unilocular (rarely multilocular) cyst o Subtotal excision ~ recurrence
containing clear or proteinaceous fluid (milky,
cream-colored, yellowish, xanthochromic)
o Demonstrable cyst connection with spinal cord, IDIAGNOSTIC CHECKLIST
vertebrae, or both; dorsal spinal-enteric tract
traverses cartilage-lined canal of Kovalevsky through Consider
small dysplastic vertebra • Degree of cord compression, size of cyst, and severity
of associated anomalies determine disease course and
Microscopic Features
prognosis
• Thin-walled cyst lined by simple, pseudostratified,
stratified cuboidal, or columnar epithelium +/- ciliated Image Interpretation Pearls
epithelium, goblet cells • Imaging appearance reflects cyst composition
• Ultrastructurally similar to brain Rathke cleft cyst, • Look for associated mediastinal or abdominal cysts,
colloid cyst connecting fistulae, or vertebral anomalies
Staging, Grading or Classification Criteria
• World Health Organization (WHO) classification: ISELECTED REFERENCES
"Other malformative tumors and tumor-like lesions"
• Three types based on histology (Wilkins and Odum) 1. Agrawal D et al: Intramedullary neurenteric cyst presenting
o Type A: Single layer or pseudostratified epithelium as infantile paraplegia: a case and review. Pediatr
Neurosurg. 37(2):93-6, 2002
resembling respiratory (17%) or gastrointestinal 2. Kapoor V et al: Neuroradiologic-pathologic correlation in a
(50%) epithelium, or both (33%) neurenteric cyst of the clivus. A]NR Am] Neuroradiol.
o Type B: Type A + mucous or serous glands, smooth 23(3):476-9, 2002
muscle connective tissue, lymphoid tissue or 3. Graham D et al: Greenfield's Neuropathology. 7th ed.
nervous tissue London, Arnold. 968-969, 2002
o Type C: Type A + ependymal or other glial elements 4. Kumar R et al: Intraspinal neurenteric cysts--report of three
paediatric cases. Childs Nerv Syst. 17(10):584-8,2001
5. Lippman CR et al: Intramedullary neurenteric cysts of the
ICLINICAL ISSUES spine. Case report and review of the literature. ] Neurosurg.
94(2 Suppl):305-9, 2001
6. Filho FL et al: Neurenteric cyst of the craniocervical
Presentation junction. Report of three cases. ] Neurosurg. 94(1
• Most common signs/symptoms Suppl):129-32,2001
o Back/radicular pain 7. Kadhim H et al: Spinal neurenteric cyst presenting in
o Progressive paraparesis/paresthesias infancy with chronic fever and acute myelopathy.
o Gait disturbance Neurology. 54(10):2011-5, 2000
o Meningitis 8. Barkovich A: Pediatric neuroimaging. 3rd ed. Philadelphia,
• Mollaret ~ rupture into CSF Lippincott-Raven. Pages 655-658, 2000
9. Paleologos TS et al: Spinal neurenteric cysts without
• Bacterial ~ seeding by enteric organisms associated malformations. Are they the same as those
o Other signs/symptoms presenting in spinal dysraphism? Br] Neurosurg.
• Chronic fever and myelopathy 2° to secretion of 14(3):185-94, 2000
TNF-()( (usually infants) 10. Lee SH et al: Thoracic neurenteric cyst in an adult: case
• Congenital mirror movements of hands (very rare, report. Neurosurgery. 45(5):1239-42; disscussion 1242-3,
seen in conjunction with diastematomyelia) 1999
• Clinical profile 11. Ellis AM et al: Intravertebral spinal neurenteric cysts: a
o Children present more commonly with cutaneous unique radiographic sign--"the hole-in-one vertebra". J
Pediatr Orthop. 17(6):766-8, 1997
stigmata, spinal dysraphism symptoms 12. Atlas S. Magnetic Resonance Imaging of the Brain and
o Adults present primarily with pain, myelopathy Spine. 2nd ed. Philadelphia, Lippincott-Ravin. 1314-1316,
1996
Demographics 13. Prasad VS et al: Cervico-thoracic neurenteric cyst:
• Age clinicoradiological correlation with embryogenesis. Childs
o 2nd ~ 4th decades (range 8 days ~ 72 years) Nerv Syst. 12(1):48-51, 1996
• Cysts with associated malformations present 14. Gao PY et al: Neurenteric cysts: pathology, imaging
earlier than isolated cysts spectrum, and differential diagnosis. International Journal
• Gender: M:F = 3:2 to 2:1 of Neuroradiology 1:17-27,1995
15. Brooks BS et al: Neuroimaging features of neurenteric cysts:
Natural History & Prognosis analysis of nine cases and review of the literature. AJNR
• Some asymptomatic ~ diagnosis at autopsy Am J Neuroradiol. 14(3):735-46, 1993
• Most show progressive neurological deterioration 16. Kropp J et al: Neurenteric cyst diagnosed by
technetium-99m pertechnetate sequential scintigraphy. J
• Significant symptomatic improvement following Nucl Med. 28(7):1218-20, 1987
resection in many patients 17. Wilkins RH et al: Spinal intradural cysts. in Handbook of
clinical neurology. 20:55-102, 1976
Treatment
• Primary treatment goal is complete surgical excision
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NEURENTERIC CYST
IIMAGE GALLERY 1
63
Typical
(Left) Sagittal bone CT
(Klippel-Feil syndrome)
following myelography
shows a sagittal vertebral
cleft (arrow) in conjunction
with a ventral cervical
neurenteric cyst. Mild cord
compression is present.
(Right) Sagittal T2WI MR
demonstrates a ventral
neurenteric cyst (arrow) in
conjunction with C2
vertebral cleft. Cyst produces
mild ventral cord
compression.
Variant
(Left) Sagittal T1 WI M R
shows a dorsal cervical
neurenteric cyst associated
with cord myeloschisis and
spinal canal remodeling.
(Right) Axial T1 WI MR
depicts cervical dorsal
diaschisis with associated
dorsal neurenteric cyst.
Spinal canal remodeling
focally widens canal at cyst
level. Cyst contents are
slightly hyperintense to CSF.
Variant
(Left) Sagittal T1WI MR
shows a large "dumbbell"
neurenteric cyst extending
from the mediastinum into
the central canal through a
patent canal of Kovalevsky
causing cord compression
(Courtesy S. Blaser, MO).
(Right) Sagittal T1 C+ MR
shows an atypical
multilocular enhancing
dorsal neurenteric cyst
causing marked cord
compression.
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FAILURE OF VERTEBRAL FORMATION
1
64
Coronal graphic shows segmented "balanced" LI and Anteroposterior radiography demonstrates segmented
L4 hemivertebra and a TIl butterfly vertebra. "balanced" right T7 and left TIl hemivertebrae
(arrows) with resultant focal scoliotk curves.
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FAILURE OF VERTEBRAL FORMATION
Key Facts
1
Terminology Pathology 65
• Synonyms: Vertebral dysplasia, segmentation .and • Abnormal vertebrae may be supranumerary or replace
fusion anomaly (SFA), "disorganized spine" normal vertebral body
• Partial or complete failure of vertebral formation • More severe SFA ~ higher likelihood of associated
visceral anomalies
Imaging Findings
• +/- Scoliosis Clinical Issues
• Sagittal or coronal vertebral clefts, hemivertebra, • Abnormal spine curvature +/- neural deficits, limb or
butterfly vertebra visceral abnormalities
• +/- Lipoma, tethered cord, diastematomyelia, spinal
cord compression Diagnostic Checklist
• Look for concurrent segmentation failure, other
Top Differential Diagnoses neural and visceral anomalies
• Vertebral fracture • Type of deformity determines propensity for scoliosis
• Inherited spinal dysplasias progression
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FAILURE OF VERTEBRAL FORMATION
1 o Lateral hemivertebra: One chondral center does not
develop; ossification center subsequently fails to
• Surgical resection and/or fusion to arrest/reverse
kyphoscoliosis in moderate to severe cases
66 develop on that side
o Sagittal cleft ("butterfly") vertebra: Separate
ossification centers form (but fail to unite) in each IDIAGNOSTIC CHECKLIST
paired paramedian chondrification center
o Coronal cleft vertebra: Formation and persistence of Consider
separate ventral and dorsal ossification centers • Syndromal origin or association ~ important to look
o Posterior hemivertebra: Later failure during for and characterize visceral anomalies
ossification stage
Image Interpretation Pearls
• Epidemiology
o Isolated or syndromal, singular or multiple • Look for concurrent segmentation failure, other neural
o Incidence increased with parental consanguinity, and visceral anomalies
concurrent multisystem anomalies • Type of deformity determines propensity for scoliosis
• Associated abnormalities progression
o Dysraphism, split notochord syndromes
• Diastematomyelia, syrinx, tethered cord/fatty
filum, congenital tumor, visceral organ anomalies ISELECTED REFERENCES
o Other developmental vertebral abnormalities 1. Arlet V et al: Congenital scoliosis. Eur Spine].
• Partial duplication (supernumerary hemivertebra) 12(5):456-63, 2003
• Segmentation failure (block vertebra, posterior 2. Isono M et al: Limited dorsal myeloschisis associated with
element dysraphism, pediculate bar, neural arch multiple vertebral segmentation disorder. Pediatr
Neurosurg. 36(1):44-7, 2002
fusion)
3. Suh SW et al: Evaluating Congenital spine deformities for
o Visceral abnormalities; 610/0 of congenital scoliosis intraspinal anomalies with magnetic resonance imaging. ]
patients Pediatr Orthop 21(4): 525-31, 2001
4. Kim Y] et al: Surgical treatment of congenital kyphosis.
Gross Pathologic & Surgical Features Spine. 26(20):2251-7, 2001
• Normal bone density unless concurrent metabolic 5. ]askwhich D et al: Congenital scoliosis. Curr Opin Pediatr
abnormality 12(1): 61-6, 2000
• Surgical observations of vertebral configuration 6. McMaster M] et al: Natural history of congenital kyphosis
matches imaging findings and kyphoscoliosis. A study of one hundred and twelve
patients.] Bone Joint Surg Am 81(10): 1367-83, 1999
Microscopic Features 7. Tortori-Donati P et al: Segmental spinal dysgenesis:
• Normal bone histology unless concurrent metabolic neuroradiologic findings with clinical and embryologic
abnormality correlation. A]NR Am] Neuroradiol. 20(3):445-56, 1999
8. Hayman LA et al: Bony spine and costal elements, Part II.
Int] Neurorad. 4(1):61-68, 1998
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FAILURE OF VERTEBRAL FORMATION
IIMAGE GALLERY 1
67
Variant
(Left) Axial bone CT depicts
a partial sagittal cleft of a
thoracic vertebra. This
deformity would not be
expected to produce
scoliosis. (Right)
Anteroposterior myelography
shows T7 2 hypoplasia (open
arrows) and aplasia of L1,
producing spinal stenosis
(arrows) and lateral cord
compression (Courtesy R.
Boyer, MO).
Variant
(Left) Anteroposterior
radiography demonstrates
segmental lumbar
hypogenesis of L2 and
agenesis of U (Courtesy R.
Boyer; MO). (Right) Lateral
radiography of lumbar
segmental spinal dysgenesis
shows hypogenesis of L2
(arrow) & agenesis of U,
w/posterior positioning of
the lumbar spine relative to
the sacrum (Courtesy R.
Boyer, MO).
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PARTIAL VERTEBRAL DUPLICATION
1
68
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\ l'
.....
.di.'
Anteroposterior radiography depicts a supernumerary Coronal TlWI MR shows a typical left thoracic
hemivertebra (arrow) between the right L2 and L3 supernumerary hemivertebra (arrow) producing
vertebral levels that produces a convex right scoliotk minimal scoliosis.
curve.
ITERMINOLOGY CT Findings
• Bone CT: Supranumerary lateral hemivertebra
Abbreviations and Synonyms
• Synonym: Supranumerary vertebra MR Findings
Definitions • TIWI: Supranumerary lateral hemivertebra +/-
dysraphism, spinal cord anomalies
• Partial duplication of vertebral column produces one • T2WI: Same as TlWI
or more supranumerary ("extra") vertebra
Imaging Recommendations
• Plain films for counting, quantitating scoliosis
IIMAGING FINDINGS • Multiplanar MR imaging to evaluate vertebral
anatomy, assess for associated abnormalities
General Features • CT with 3D reconstructions - demonstrates
• Best diagnostic clue: Atypical scoliosis (sharply hemivertebra AND scoliosis
angulated, single curve, or focal) with one or more
"extra" hemivertebra
• Location: Thoracolumbar> cervical I DIFFERENTIAL DIAGNOSIS
• Size: Hemivertebra is smaller than normal vertebra
• Morphology: "Extra" hemivertebra frequently Butterfly vertebra
associated with focal scoliotic curve • Two pedicles; looks like "bilateral hemivertebra" with
central cleft
Radiographic Findings • Separate bilateral ossification centers form, fail to
• Radiography unite
o Supranumerary hemivertebra with scoliosis
o Plain films best for "counting" to definitively localize Vertebral fracture
abnormal vertebral level • Pathologic or traumatic; history critical
o Long-cassette erect weightbearing radiographs • Noncorticated irregular margins +/- soft tissue edema,
quantitate scoliosis cord injury
• Two pedicles at level, unlike hemivertebra
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Key Facts
1
Terminology • TlWI: Supranumerary lateral hemivertebra +/- 69
• Synonym: Supranumerary vertebra dysraphism, spinal cord anomalies
• Partial duplication of vertebral column produces one Top Differential Diagnoses
or more supranumerary ("extra") vertebra • Butterfly vertebra
Imaging Findings • Vertebral fracture
• Plain films best for "counting" to definitively localize Diagnostic Checklist
abnormal vertebral level • Supranumerary hemivertebra "missing" a pedicle on
• Bone CT: Supranumerary lateral hemivertebra hypoplastic side; distinguishes from fracture
I , ~t
o More severe cases detected in infancy or childhood
o Milder cases detected in adolescence during school (Left) Coronal TlWI MR shows a small left supranumerary lumbar
scoliosis checks, by pediatrician, or incidentally hemivertebra (arrow). (Right) Coronal TlWI MR centered at a left
during imaging lumbar supranumerary hemivertebra (open arrow) shows normal
• Gender: M = F right pedicles above and below hemivertebra (arrows) but not at
hemivertebra level.·
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VERTEBRAL SEGMENTATION FAILURE
1
70
Coronal bone CT with 30 reformat shows hypogenesis Coronal bone CT depicts bilateral interpedicular bars,
and fusion of multiple mid-thoracic vertebrae. with fusion of the posterior elements and ribs at multiple
Developmental asymmetry produces rotational scoliotic levels (Courtesy G. Hedlund, 00).
curvature to the left.
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o T2WI to evaluate spinal cord pathology, o Mildest (and most common) form is indeterminate
compression (transitional) vertebrae at thoracolumbar,
• CT to characterize osseous structures lumbosacral transition
o 3D CT useful for pre-operative planning • Genetics
o Deranged Pax-l gene expreSSion => abnormal
notochord signaling in developing vertebral column
I DIFFERENTIAL DIAGNOSIS o Many syndromes associated with SFA
• Klippel-Feil (cervical SFA): Common, gene locus
Juvenile chronic arthritis 8q22.2
• Difficult to distinguish from cervical block vertebra • Spondylothoracic dysplasia Oarcho-Levin):
• Search for other affected joints, appropriate history Uncommon, thoracic spine fusions in "crab-like"
array with multiple rib fusions
Surgical fusion
• Etiology
• No "waist" at disc space level, facets infrequently o Block vertebra: Segmentation failure of two or more
ankylosed vertebral somites
• Surgical history key to making diagnosis o Posterior neural arch anomalies: Failure to unite in
Chronic sequelae of discitis the midline => dysraphism +/- unilateral pedicle
• Cortical endplate margins irregular, "waist" absent aplasia/hypoplasia
• Search for history of prior spinal infection • Epidemiology
o Isolated or syndromal, singular or multiple
Ankylosing spondylitis o SFAs account for 18% of scoliosis; incidence higher
• Delicate contiguous syndesmophytes ("bamboo spine") with multisystem abnormalities, parental
+ symmetric SI joint disease consanguinity
• HLA· B27 positive (95%) • Associated abnormalities
o Other neuraxis anomalies (40%)
• Dysraphism, split notochord syndrome
IPATHOLOGY • Partial or complete failure of formation (vertebral
aplasia, hemivertebra, butterfly vertebra)
General Features • Partial duplication (supernumerary hemivertebra)
• General path comments o Scoliosis
ci Normal embryology: Vertebral formation occurs o Renal, gastrointestinal (20%), congenital cardiac
over three sequential periods defects (10%)
• Membrane development: Segmental formation of
medial sclerotome (vertebral bodies) and lateral Gross Pathologic & Surgical Features
myotome (paraspinal muscles) • Normal bone density unless concurrent metabolic
• Chondrification: Sclerotomes separate transversely abnormality
and join with adjacent sclerotomal halves => • Surgical observations of vertebral configuration
paired chondrification sites develop in vertebral matches imaging findings
bodies and neural arches Microscopic Features
• Ossification: Chondral skeleton ossifies from
• Normal bone histology unless concurrent metabolic
single ossification center
abnormality
o Segmentation and fusion anomalies result from
aberrant vertebral column formation
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VERTEBRAL SEGMENTATION FAILURE
1 Staging, Grading or Classification Criteria ISELECTED REFERENCES
72 • Block vertebra - failure of ~ 2 vertebral somites to 1. Arlet V et al: Congenital scoliosis. Eur Spine].
segment 12(5):456-63, 2003
o Combined vertebrae may be normal height, short, 2. Cornier AS et al: Controversies surrounding Jarcho-Levin
or tall syndrome. Curr Opin Pediatr. 15(6):614-20,2003
o Disc space frequently rudimentary or absent 3. Isono M et al: Limited dorsal myeloschisis associated with
multiple vertebral segmentation disorder. Pediatr
o Frequent association with hemivertebra/absent Neurosurg. 36(1):44-7, 2002
vertebra above or below block level, posterior 4. Suh SW et al: Evaluating Congenital spine deformities for
element fusion intraspinal anomalies with magnetic resonance imaging. J
• Posterior neural arch anomalies Pediatr Orthop 21(4): 525-31, 2001
o Failure to unite in the midline ~ dysraphism (+/- 5. Kim YJ et al: Surgical treatment of congenital kyphosis.
unilateral pedicle aplasia/hypoplasia) Spine. 26(20):2251-7, 2001
o Unfused spinous processes; L5, Sl > C1 > C7 > T1 > 6. Nagashima H et al: No neurological involvement for more
lower thoracic spine than 40 years in Klippel-Feil syndrome with severe
hypermobility of the upper cervical spine. Arch Orthop
o Multiple level posterior fusion ~ congenital
Trauma Surg. 121(1-2):99-101, 2001
vertebral bar 7. Jaskwhich D et al: Congenital scoliosis. Curr Opin Pediatr
12(1): 61-6, 2000
8. Yildiran A et al: Semantic and nosological confusions on
ICLINICAL ISSUES multiple vertebral segmentation defects. Pediatr Neurosurg.
33(3):168, 2000
Presentation 9. McMaster MJ et al: Natural history of congenital kyphosis
• Most common signs/symptoms and kyphoscoliosis. A study of one hundred and twelve
o Asymptomatic patients. J Bone Joint Surg Am 81(10): 1367-83, 1999
o Kyphoscoliosis 10. McMaster MJ: Congenital scoliosis caused by a unilateral
failure of vertebral segmentation with contralateral
o Other signs/symptoms hemivertebrae. Spine. 23(9):998-1005, 1998
• Neural deficits (usually myelopathic), limb or 11. Anderson PJ et al: The cervical spine in Crouzon syndrome.
visceral anomalies Spine. 22(4):402-5, 1997
• Respiratory failure (rare, 2° impeded chest 12. Lopez BC et al: Inadequate PAX-l gene expression as a
movement due to severe scoliosis, rib cage fusion) cause of agenesis of the thoracolumbar spine with failure
• Clinical profile: Asymptomatic incidental detection or of segmentation. Case report. J Neurosurg. 86(6):1018-21,
presents for evaluation of abnormal spine curvature 1997
13. AsIan Y et al: Multiple vertebral segmentation defects. Brief
Demographics report of three patients and nosological considerations.
• Age: Severe cases detected during infancy/childhood; Genet Couns. 8(3):241-8, 1997
14. Mortier GR et al: Multiple vertebral segmentation defects:
mild cases may present as adults
analysis of 26 new patients and review of the literature. Am
• Gender: M "" F; dependent on syndromal association J Med Genet. 61(4):310-9, 1996
15. Jansen BR et al: Discitis in childhood. 12-35-year follow-up
Natural History & Prognosis of 35 patients. Acta Orthop Scand. 64(1):33-6, 1993
• Scoliosis frequently progressive 16. Lee CK et al: Isolated congenital cervical block vertebrae
o Unilateral unsegmented bar with contralateral below the axis with neurological symptoms. Spine.
hemivertebrae ~ rapidly progressive, severely 6(2):118-24, 1981
deforming congenital scoliosis 17. Mayfield JK et al: Congenital kyphosis due to defects of
o Isolated block vertebra rarely produce scoliosis anterior segmentation. J Bone Joint Surg Am.
• Abnormal segments may continue to fuse 62(8):1291-301, 1980
Treatment
• Conservative in mild cases (orthotics, observation)
• Surgical fusion to arrest/reverse kyphoscoliosis in
moderate to severe cases
I DIAGNOSTIC CHECKLIST
Consider
• Clinical manifestations variable, determined by type of
SFA and syndromal association
Image Interpretation Pearls
• Coronal MRI, AP radiography best for detecting and
characterizing SFAs, "counting" to determine abnormal
vertebral levels
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Variant
(Left) Sagittal T2WI MR
(VACTERL syndrome) shows
hypogenesis and fusion of
multiple thoracic and lumbar
vertebrae (arrows)
producing kyphotic gibbus
deformity (Courtesy R.
Boyer, MO). (Right) Sagittal
T2WI MR depicts multilevel
vertebral segmentation
failure with fusion of the
facets and laminae (arrows)
into an interlaminar bar
(Courtesy R. Boyer, MO).
Variant
(Left) Sagittal T2WI MR
shows partial fusion of the
anterior L3 and L4 vertebral
bodies (arrow), a mild
variation of congenital block
vertebra (Courtesy R. Boyer,
MOJ. (Right) Coronal T1WI
MR shows fusion of right L1
hemivertebra (open arrow)
with the L2 vertebral body
(arrow), producing focal
scoliosis (CourtesyR. Boyer,
MOJ.
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KLIPPEL-FElL SPECTRUM
1
74
Sagittal graphic shows congenital fusion of the C5-6 Sagittal TlWI MR (KFS type l) shows congenital CVj
vertebrae and spinous processes, with characteristk segmentation and fusion anomalies involving nearly the
rudimentary disc space and distinctive "waist" typical of entire cervical spine. Diastematomyelic upper cervical
congenital fusion. cord is notably thinned.
~'~'
"
...iiI..
' .~
...'
..
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KLIPPEL-FElL SPECTRUM
Key Facts
1
Terminology Pathology 75
• Synonyms: KFS, Klippel-Feil syndrome • Congenital cervical fusion 2° to failure of normal
segmentation of cervical somites (3rd ~ 8th weeks)
Imaging Findings • Sporadic; familial genetic component with variable
• Location: C2-3 (SO%) > CS-6 (33%) > CVJ, upper expression identified in many patients
thoracic spine
• Morphology: Vertebral body narrowing ("wasp waist") Clinical Issues
at fused rudimentary disc space +/- fusion of posterior • Classic triad (33-S0%): Short neck, low posterior
elements hairline, and limited cervical motion
• +/- Omovertebral bone • Many patients have normal clinical appearance
despite severity of involvement
Top Differential Diagnoses
• Juvenile chronic arthritis Diagnostic Checklist
• Surgical fusion • Much KFS morbidity and nearly all mortality related
• Chronic sequelae of discitis to visceral system dysfunction
• Ankylosing spondylitis • Look for instability, progressive degenerative changes,
cord/brainstem compression
o Osseous findings same as T1WI; normal marrow o "Klippel-Feil syndrome" colloquially used for all
signal patients with cervical congenital fusion anomalies
o +/- Cord or nerve root compression, syringomyelia, regardless of extent
brainstem abnormalities, myeloschisis o Congenital cervical fusion 2° to failure of normal
segmentation of cervical somites (3rd ~ 8th weeks)
Imaging Recommendations • Genetics
• Best imaging tool o Sporadic; familial genetic component with variable
o Radiography to evaluate and follow instability, expression identified in many patients
degenerative changes • C2/3 fusion (type 2) ~ autosomal dominant with
o MRI to exclude cord compression, detect variable penetrance
degenerative changes • CS/6 fusion (type 2) ~ autosomal recessive
• Protocol advice o SGMI (chromosome 8) ~ 1st Klippel-Feil gene
o Serial neutral and flex/extend plain radiographs to identified; gene expression overlaps all 3 KFS types
detect progressive instability, degenerative disease • Etiology
o Multiplanar MRI to evaluate canal compromise, cord o No universally accepted etiology; embryonic insult
compression, soft tissue degenerative changes postulated between 4th ~ 8th weeks
o Ultrasound or CECT to detect and characterize • Putative environmental causative factors include
associated visceral organ abnormalities teratogens, maternal alcoholism
• Association with other syndromes (fetal alcohol,
Goldenhar, Wildervanck -
IDIFFERENTIAL DIAGNOSIS cervical!occulo/acoustic)
• Epidemiology: 1/42,000 births
Juvenile chronic arthritis • Associated abnormalities
• Difficult to distinguish from cervical block vertebra o Hemivertebrae, butterfly vertebrae, spina bifida
• Search for other affected joints, appropriate history o Scoliosis (usually congenital) +/- kyphosis (60%)
Surgical fusion o Odontoid dysplasia, basilar impression, C1
• No disc space "waist", facets infrequently ankylosed assimilation, occipito-cervical instability
o Syringomyelia, diastematomyelia (20%), Chiari 1
• Surgical history key to making diagnosis
malformation (8%), neurenteric cyst or dermoid
Chronic sequelae of discitis (rare)
• Irregular endplate margins, no "waist", +/- kyphosis o Cervicomedullary neuroschisis +/- synkinesis (20%)
• Confirm history of prior spinal infection o Sprengel deformity +/- omovertebral bone (lS-30%);
unilateral or bilateral
Ankylosing spondylitis o Sensorineural hearing loss (30%), GU tract
• Delicate contiguous syndesmophytes ("bamboo spine") abnormalities (3S%), congenital heart disease (14%),
+ symmetric SI joint disease upper extremity deformity, facial anomalies
• HLA - B27 positive (9S%)
Gross Pathologic & Surgical Features
• Surgical observations correlate to imaging findings
IPATHOLOGY Microscopic Features
General Features • Histologically normal bone, disc
• General path comments
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KLIPPEL-FElL SPECTRUM
1 Staging, Grading or Classification Criteria IDIAGNOSTIC CHECKLIST
76 • Type 1 (9%): Massive fusion of cervical, upper thoracic
spine ~ severe neurological impairment, frequent Consider
associated abnormalities • Much KFS morbidity and nearly all mortality related to
• Type 2 (84%): Fusion of one or more cervical vertebral visceral system dysfunction
interspaces Image Interpretation Pearls
• Type 3 (7%): Fusions involve cervical and lower • Look for instability, progressive degenerative changes,
thoracic/lumbar vertebra cord/brainstem compression
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KLIPPEL-FElL SPECTRUM
IIMAGE GALLERY 1
77
Variant
(Left) Sagittal T2WI MR (KFS
type 3) shows congenital
L2/3 fusion with narrow
rudimentary disc space and
normal AP canal diameter.
(Right) Sagittal T7WI MR
(KFS type 7) shows
atlanto-occipital assimilation,
dens hypoplasia, and partial
segmentation failure at
C7/T7 (Courtesy C.
Hedlund,DO).
Variant
(Left) Axial T7WI MR (KFS
type 7) depicts an
omovertebral bone (arrows)
connecting the lamina to the
scapula (Courtesy C.
Hedlund, 00). (Right)
Sagittal T7WI MR (KFS type
7) shows multilevel cervical
segmentation failure with
two hyperintense intradural
lipomas affixed to the spinal
cord (Courtesy B. Chong,
MO).
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Sagittal graphic shows an os odontoideum and enlarged Sagittal T2WI MR depicts a large os odontoideum in
anterior Cl ring suggesting a congenital origin. No static conjunction with enlargement of the anterior Cl ring
instability or CVj stenosis in this case. (arrow). Static alignment is normal.
ITERMINOlOGY CT Findings
Abbreviations and Synonyms • CECT
o +/- Enhancing granulation tissue mass ("pannus")
• Synonyms: CVJ anomalies, craniocervical junction • Patients with reducible abnormalities have largest
(cq) variants or anomalies pannus
Definitions • Bone CT
• Anatomical variations of skull base-cervical spine o Similar findings to plain radiographs; sagittal and
articulation coronal reformats minimize effect of overlapping
structures
MR Findings
I IMAGING FINDINGS • TIWI: Osseous anomalies +/- soft tissue granulation
General Features tissue, cord compression, hindbrain anomalies
• T2WI: Similar to TIWI; best shows spinal cord status
• Best diagnostic clue: Flattening or malformation of
• TI C+: +/- Enhancing "pannus"
clivus, anterior CI ring, or odontoid process
• Location: Craniovertebral junction (CVJ) Imaging Recommendations
• Size: Variable with specific anatomical variation • Best imaging tool: Bone CT with multiplanar reformats
• Morphology: Variable: Platybasia, segmentation and • Protocol advice
fusion anomalies, CI or C2/dens anomalies o Dynamic flex/extend plain films to demonstrate
Radiographic Findings biomechanics, uncover instability
o Multiplanar TIWI, T2WI to evaluate cord, soft
• Radiography: Platybasia, basilar invagination, CI ring tissues; flex/extend MRI optimally delineates effect
assimilation, asymmetrical CI/2 articulation,
of position on neural compression
backward tilt, hypoplasia/aplasia, or os odontoideum o CT with multiplanar reformats to evaluate osseous
of odontoid process, C2/3 fusion (Klippel-Feil)
structures for surgical planning
• Fluoroscopy: Variable instability of CO/I, Cl/2
articulations with dynamic flexion and extension
.~
\. ".,7'3
'alii .-
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. fc
A1ucopolysaccharidos~ Psoriatic Arthritis juv. Chronic Arthritis Type II Dens Fx
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CRANIOVERTEBRAL JUNCTION VARIANTS
1 o C1 assimilation ("occipitalized C1"): Segmentation
failure ~ fibrous or osseous union between 1st Treatment
80 spinal sclerotome and 4th occipital sclerotome • Conservative approach initially unless unstable or
• +/- Occipitocervical synostosis; most C1 ring neural deficits
assimilations asymptomatic, more likely to be o Traction, cervical orthosis, activity restriction
symptomatic if retro-odontoid AP canal diameter • Symptomatic, refractory to conservative management
< 19mm o Skeletal traction to distinguish reducible from
o C1 malformation: Aplasia, hypoplasia, cleft C1 arch, irreducible abnormalities, relieve symptoms
"split atlas" (anterior and posterior arch rachischisis) pre-operatively
o Association with Klippel-Feil, basilar invagination, o Correction of underlying biomechanical
Chiari 1 malformation abnormality with decompression +/- fusion
• C2 anomalies: C1/2 segmentation failure, dens
dysplasia
o Majority confined to odontoid process; partial IDIAGNOSTIC CHECKLIST
(hypoplasia) ~ complete absence (aplasia),
ossiculum terminal persistens, os odontoideum
Consider
• Ossiculum terminale persistens: Ossification • Look for combinations of anomalies based on known
failure of terminal ossicle ~ incidental "notch" in association patterns
dens tip • Impact of diagnosis must be customized to individual
• Os odontoideum: Well-defined ossicle at dens tip patient to develop best treatment approach
+ anterior C1 arch enlargement Image Interpretation Pearls
o Odontoid anomalies in association with • Dynamic flex-extend imaging determines stability,
ligamentous laxity ~ atlantoaxial instability; most redUcibility of abnormality
common in Down syndrome, Morquio syndrome, • CT with reformats indispensable to evaluate osseous
Klippel-Feil syndrome, and skeletal dysplasias abnormalities
• Incompetence of cruciate ligament ~ C1/2
instability, possible neurologic deficit or death
ISELECTED REFERENCES
ICLINICAL ISSUES 1. Naderi S et al: Anatomical and computed tomographic
analysis of C1 vertebra. Clin Neurol Neurosurg.
Presentation 105(4):245-8,2003
2. Perez-Vallina JR et al: Congenital anomaly of
• Most common signs/symptoms craniovertebral junction: atlas-dens fusion with C1 anterior
o Posterior occipital headache exacerbated by arch cleft. J Spinal Disord Tech. 15(1):84-7, 2002
flexion/extension 3. Taitz C: Bony observations of some morphological
o Sub-occipital neck pain (85%); may clinically mimic variations and anomalies of the craniovertebral region.
"basilar migraine" Clin Anat. 13(5):354-60, 2000
o Myelopathy, brainstem/cranial nerve deficits, 4. Smoker WR: MR imaging of the craniovertebral junction.
weakness, lower extremity ataxia Magn Reson Imaging Clin N Am. 8(3):635-50, 2000
5. Kim FM: Developmental anomalies of the craniocervical
o Vascular symptoms (15-20%); TIA, vertigo, visual
junction and cervical spine. Magn Reson Imaging Clin N
symptoms with rotation or head manipulation Am. 8(3):651-74, 2000
• Clinical profile 6. Iwata A et al: Foramen magnum syndrome caused by
o Usually normal clinical appearance; obvious clinical atlanto-occipital assimilation. J Neurol Sci. 154(2):229-31,
dysmorphism implies syndromal association 1998
o Symptomatic presentation following mild trauma 7. Menezes AH: Craniovertebral junction anomalies:
common diagnosis and management. Semin Pediatr Neurol.
4(3):209-23, 1997
Demographics 8. Menezes AH: Craniovertebral junction anomalies. In:
• Age: Infancy ~ late adulthood depending on severity Tindall et al (ed): The Practice of Neurosurgery. Baltimore,
Williams and Wilkins, 2729-2740, 1996
• Gender: M = F
9. Menezes AH: Primary craniovertebral anomalies and the
Natural History & Prognosis hindbrain herniation syndrome (Chiari I): data base
analysis. Pediatr Neurosurg. 23(5):260-9, 1995
• Neurological symptoms usually gradual onset with
10. Erbengi A et al: Congenital malformations of the
localizing signs; some patients asymptomatic craniovertebral junction: classification and surgical
throughout life treatment. Acta Neurochir (Wien). 127(3-4):180-5, 1994
• Occasionally neurological presentation is fulminant ~ 11. Smoker WR: Craniovertebral junction: normal anatomy,
quadriplegia, sudden death craniometry, and congenital anomalies. Radiographics.
• Undetected anomalies at risk for injury during minor 14(2):255-77, 1994
trauma, anesthesia 12. Chamberlain W: Basilar impression (platybasia). Yale J BioI
• Early diagnosis permits treatment before symptoms or Med 11:487, 1938-1939
permanent neurological sequelae
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Variant
(Left) Sagittal bone CT shows
'"\. r \ variant Cl assimilation into
~)\
the clivus and C2 spinous
n
between anterior C1 and
odontoid process (Courtesy
G. Hedlund, DO). (Right)
Axial bone CT shows a
congenital cleft of both
j1.,
. •
.
I
/
, (
(
( \
(, I
anterior and posterior Cl
rings ("split atlas"), with
rounded corticated margins
that distinguish from fracture
(Courtesy G. Hedlund, DO).
•
Variant
(Left) Lateral radiography
shows mild dens hypoplasia.
Anterior Cl ring enlargement
suggests a congenital origin.
Static alignment is normal,
but dynamic imaging would
be required to exclude
instability. (Right) Sagittal
bone CT shows os
odontoideum, with
hypertrophy of C1 anterior
ring, platybasia, clival-os
pseudoarticulation, and
subluxation of the COIC l/os
complex indicating static
C 1/2 instability.
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LIMBUS VERTEBRA
1
82
Disc herniation underneath the non-ossified apophysis Lateral radiography shows a well corticated fragment
in a pediatric patient (left) mimics a vertebral "defect", (arrow) at anterosuperior L4 vertebral margin with
while subsequent ossification (right) produces a typical matching vertebral donor site, typical of limbus vertebra
adult appearance. (LV).
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LIMBUS VERTEBRA
Key Facts
1
Terminology • Schmorl node 83
• Synonyms: LV, slipped vertebral apophysis • Anterior osteophyte fragment
• Calcified disc herniation
Imaging Findings
• Best diagnostic clue: Small corticated bone fragment
Clinical Issues
matching osseous defect of anterosuperior vertebral • No further investigative procedures necessary
margin following diagnosis
• Children: Lucent defect in anterosuperior corner of • LV may evolve into anterior Schmorl node in some
vertebral body patients
• Adults: Triangular corticated bone fragment Diagnostic Checklist
• Best imaging tool: Plain radiographs usually • LV is sequel of remote injury in an immature skeleton
diagnostic • Important to exclude other causes of back pain in a
• MRI helpful (if necessary) to assess for edema or patient with LV
inflammation in context of acute symptoms • LV fragment matches size of vertebral body defect
Top Differential Diagnoses
• Acute vertebral fracture
o T2 hyperintense disc extends between fragment and o Caused by herniation of nucleus pulposus beneath
vertebral body ring apophysis (junction of cartilaginous endplate,
• Caveat: Disc protrusion may be hypointense in bony rim)
adults with degenerative disc disease, but usually • Occurs prior to fusion of apophysis to vertebral
resembles intrinsic signal intensity within body
remainder of intervertebral disc • Apophysis remains separate from vertebral body
• STIR: Mild hyperintense marrow edema (if acute) o Anterosuperior vertebral margin may be more
frequently affected because of adjacent vertebral
Other Modality Findings body size difference (superior < inferior)
• Discography: Intradiscal contrast flows between • During flexion, the anterior disc is forced down
fragment and vertebral body, confirming diagnosis into superior end plate of larger inferior vertebra
Imaging Recommendations • Pushes nucleus pulposis through the cartilage
plate, isolating apophysis from main vertebral
• Best imaging tool: Plain radiographs usually diagnostic
body
• Protocol advice
o Plain radiographs to establish diagnosis • Etiology
o Superior and inferior surface of developing vertebral
o MRI helpful (if necessary) to assess for edema or
body is covered by thin, peripherally thickened
inflammation in context of acute symptoms
cartilage plate ~ cartilaginous marginal ridge
o Endochondral ossification of marginal ridge begins
at age 7-9 years ~ forms rim apophysis (RA)
I DIFFERENTIAL DIAGNOSIS o RA separated from vertebral body by this thin
Acute vertebral fracture cartilage layer until apophysis fuses to vertebral
• LV commonly misdiagnosed as fracture body at age 18-20 years
• Look for trabecular smudging, soft tissue swelling • Relative weak point in disc/vertebral body until
fusion occurs
Schmorl node • LV occurs when nucleus pulposus herniates
• Schmorl node herniates more centrally into vertebral between RA and vertebral body, permanently
end plate than LV isolating a small segment of vertebral rim
o Outermost fibers of anulus fibrosus (Sharpey fibers)
Anterior osteophyte fragment embedded into RA fasten disc to spine
• Osteophyte fractures off, mimicking LV • Epidemiology
• Usually no matching donor site on adjacent vertebral o Common radiologic finding in adults; relatively less
body common in children
o Trauma appears to be responsible for many cases
Calcified disc herniation
• LV more common in active, athletic children;
• No matching vertebral body defect traumatic insult may not be remembered
• Location usually not characteristic of LV
• Associated abnormalities
o Scheuermann Disease
o Schmorl nodes
IPATHOLOGY
Gross Pathologic & Surgical Features
General Features • Small corticated bone fragment, separated from
• General path comments vertebral body by intervening disc material
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LIMBUS VERTEBRA
1 Microscopic Features 3.
fractures. Neurol Res. 24(2):139-44, 2002
Bonic EE et al: Posterior limbus fractures: five case reports
84 • Cancellous bone, hyaline cartilage, and acellular and a review of selected published cases. ] Manipulative
hyaline tissue (disc) Physiol Ther. 21(4):281-7, 1998
• Basophilic degeneration, hemorrhagic foci in hyaline 4. Swischuk LE et al: Disk degenerative disease in childhood:
cartilage common Scheuermann's disease, Schmorl's nodes, and the limbus
vertebra: MRI findings in 12 patients. Pediatr Radiol.
28(5):334-8, 1998
5. Martinez-Lage]F et al: Avulsed lumbar vertebral rim plate
ICLINICAllSSUES in an adolescent: trauma or malformation? Childs Nerv
Syst. 14(3):131-4, 1998
Presentation 6. Talha A et al: Fracture of the vertebral limbus. Eur Spine].
• Most common signs/symptoms 6(5):347-50, 1997
o Chronic back pain 7. Carreon LY et al: Neovascularization induced by anulus
o Occasionally incidental finding and its inhibition by cartilage endplate. Its role in disc
o Inciting event rarely recognized or remembered absorption. 1;22(13):1429-34, 1997
8. Henales V et al: Intervertebral disc herniations (limbus
• Clinical profile vertebrae) in pediatric patients: report of 15 cases. Pediatr
o Variable symptomatology depending on location Radiol. 23(8):608-10, 1993
• Anterior defect ~ symptoms usually minor; 9. Epstein NE: Lumbar surgery for 56 limbus fractures
stiffness or loss of physiologic lordosis emphasiZing noncalcified type III lesions. Spine.
• Posterior defect ~ symptoms mimic nerve root, 17(12):1489-96, 1992
cord compression 10. Cao LB et al: Imaging study of lumbar posterior marginal
o Physical exam generally shows decreased range of intraosseous node. An analysis of 36 cases. Chin Med]
motion, +/- kyphosis, or pain on spinous process (Engl). 105(10):866-9, 1992
11. Epstein NE et al: Limbus lumbar vertebral fractures in 27
palpation
adolescents and adults. Spine. 16(8):962-6, 1991
Demographics 12. Goldman AB et al: Posterior limbus vertebrae: a cause of
radiating back pain in adolescents and young adults.
• Age: Adults> children, adolescents Skeletal Radiol. 19(7):501-7, 1990
• Gender: M > F 13. Hsu K et al: High lumbar disc degeneration. Incidence and
etiology. Spine. 15(7):679-82, 1990
Natural History & Prognosis 14. Banerian KG et al: Association of vertebral end plate
• No further investigative procedures necessary fracture with pediatric lumbar intervertebral disk
following diagnosis herniation: value of CT and MR imaging. Radiology.
o Symptoms usually resolve over months to years 177(3):763-5, 1990
• LV may evolve into anterior Schmorl node in some 15. McCarron RF: A case of mistaken identity, limbus annulare
patients mimics fracture. Orthop Rev. 16(3):173-5, 1987
16. Kerns S et al: Annulus fibrosus calcification in the cervical
Treatment spine: radiologic-pathologic correlation. Skeletal Radiol.
• Conservative management 15(8):605-9, 1986
o Analgesics 17. Yagan R: CT diagnosis of limbus vertebra. ] Comput Assist
Tomogr. 8(1):149-51, 1984
o Physical therapy 18. Kozlowski K: Anterior intervertebral disc herniations in
o Limitation of physical activity during acute phase children: unrecognised chronic trauma to the spine.
• Surgical management not indicated Australas Radiol. 23(1):67-71, 1979
19. Ghelman B et al: The limbus vertebra: an anterior disc
herniation demonstrated by discography. Am]
IDIAGNOSTIC CHECKLIST Roentgenol. 127(5):854-5, 1976
Consider
• LV is sequel of remote injury in an immature skeleton
• Important to exclude other causes of back pain in a
patient with LV
Image Interpretation Pearls
• Diagnosis of LV is less difficult in adults than children
o Presence of characteristic, well formed triangular
osseous fragment
• LV fragment matches size of vertebral body defect
o Helps distinguish LV from other differential
considerations such as calcified disc or osteophyte
ISELECTED REFERENCES
1. Mupparapu M et al: Radiographic diagnosis of Limbus
vertebra on a lateral cephalometric film: report of a case.
Dentomaxillofac Radiol. 31(5):328-30, 2002
2. Mendez]S et al: Limbus lumbar and sacral vertebral
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LIMBUS VERTEBRA
IIMAGE GAllERY 1
85
Variant
(Left) Lateral radiography
shows LV at both L4 and L5
(arrows). L5 shows
development of Schmorl
node changes posterior to
the corticated fragment.
(Right) Sagittal T2WI MR
demonstrates L4 and L5 LV
(arrows), with variant
Schmorl node changes
posterior to the L5 corticated
fragment.
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CONJOINED NERVE ROOTS
1
86
Composite graphic shows two nerve roots (left L5 and Anteroposterior myelography demonstrates typical
57) exiting the dural sac through a single composite conjoined root configuration, with an aberrant enlarged
dural sleeve and neural foramen (left 57). root sleeve (arrow) containing two roots (L5, 57).
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Key Facts
1
Terminology • Synovial cyst 87
• Synonym: Composite nerve root sleeve Pathology
• Asymmetric anomalous origin of an enlarged nerve • Symptomatic (but undiagnosed) conjoined nerve
root sleeve containing two nerve roots roots are one cause of failed back syndrome
Imaging Findings • Most commonly unilateral
• Best diagnostic clue: Enlarged root sleeve containing Clinical Issues
two roots originating midway between expected • Usually asymptomatic
positions of two contributing nerve roots • Symptomatic patients present with radiculopathy
• Lumbar spine> > cervical, thoracic spine
• L4/S > LS/Sl > L3/4 Diagnostic Checklist
• Enlarged neural foramen • Important anatomical variant to identify prior to
• Ipsilateral hypoplastic or absent pedicle spinal surgery to prevent inadvertent nerve injury
• Dorsal root ganglia contrast-enhancement and
Top Differential Diagnoses enlargement of foraminal neural structures may lead
• Nerve sheath tumor (NST) to misdiagnosis as nerve sheath tumor
• Disc herniation
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CONJOINED NERVE ROOTS
1 ICLINICAL ISSUES 6. Chu CR et al: Cervical conjoined nerve root variant:
preoperative imaging and surgical conformation. Case
88 report.] Neurosurg. 80(3):548-51, 1994
Presentation 7. Phillips LH et al: The frequency of intradural conjoined
• Most common signs/symptoms lumbosacral dorsal nerve roots found during selective
o Usually asymptomatic dorsal rhizotomy. Neurosurgery 33(1): 88-90, discussion
o Symptomatic patients present with radiculopathy 90-1, 1993
• Conjoined nerves prone to compression by discs, 8. Firooznia H et al: Normal correlative anatomy of the
lateral bony stenosis along lateral recess lumbosacral spine and its contents. Neuroimaging clinics
of North America 3(3): 411-24, 1993
• Herniated disc beneath the bifid root ~ pain,
9. Gomez]G et al: Conjoined lumbosacral nerve roots. Acta
disability, signs of entrapment 2° to fixation of Neurochir (Wien). 120(3-4):155-8, 1993
conjoined root within lateral recess, between 10. Okuwaki T et al: Conjoined nerve roots associated with
pedicles lumbosacral spine anomalies. A case report. Spine.
• Clinical profile: Patient presents with cervical or 16(11):1347-9, 1991
lumbar radiculopathy 11. Maiuri F et al: Anomalies of the lumbosacral nerve roots.
Neurol Res. 11(3):130-5, 1989
Demographics 12. Goffin] et al: Association of conjoined and anastomosed
• Age: No age predilection nerve roots in the lumbar region. A case report. Clin
• Gender: M = F Neurol Neurosurg. 89(2):117-20, 1987
13. Torricelli P et al: CT diagnosis of lumbosacral conjoined
Natural History & Prognosis nerve roots. Findings in 19 cases. Neuroradiology.
• Asymptomatic patients require no treatment 29(4):374-9, 1987
14. Peyster RG et al: Computed tomography of lumbosacral
• Rare symptomatic patients referable to conjoined root
conjoined nerve root anomalies. Potential cause of
level may require surgical therapy false-positive reading for herniated nucleus pulposus.
• Symptomatic undiagnosed conjoined roots are one Spine. 10(4):331-7, 1985
cause of failed back syndrome 15. Hoddick WK et al: Bony spinal canal changes that
o Nerve root anomalies should be searched for in cases differentiate conjoined nerve roots from herniated nucleus
of failed disc surgery pulposus. Radiology. 154(1):119-20, 1985
16. Rosner M] et al: Anomalous exit of the C-6 nerve root via
Treatment the C-6, C-7 foramen. Neurosurgery. 14(6):740-3, 1984
• Conservative non-operative observation unless 17. Gebarski SS et al: "Conjoined" nerve roots: a requirement
symptomatic for computed tomographic and myelographic correlation
• Surgical decompression in selected patients with for diagnosis. Neurosurgery. 14(1):66-8, 1984
18. Cail WS et al: Conjoined lumbosacral nerve roots.
documented concordant radiculopathy
Diagnosis with metrizamide myelography. Surg Neurol.
o Hemilaminectomy with foraminotomy, 20(2):113-9,1983
pediculectomy, and discectomy best alleviate 19. Neidre A et al: Anomalies of the lumbosacral nerve roots.
symptomatology Review of 16 cases and classification. Spine. 8(3):294-9,
1983
20. Coughlin]R et al: Metrizamide myelography in conjoined
IDIAGNOSTIC CHECKLIST lumbosacral nerve roots.] Can Assoc Radiol. 34(1):23-5,
1983
Consider 21. Helms CA et al: The CT appearance of conjoined nerve
roots and differentiation from a herniated nucleus
• Important anatomical variant to identify prior to pulposus. Radiology. 144(4):803-7, 1982
spinal surgery to prevent inadvertent nerve injury 22. White]G 3rd et al: Surgical treatment of 63 cases of
• Exclude differential diagnoses such as nerve sheath conjoined nerve roots.] Neurosurg. 56(1):114-7, 1982
tumor, disc herniation, or synovial cyst 23. Epstein]A et al: Conjoined lumbosacral nerve roots.
Management of herniated discs and lateral recess stenosis
Image Interpretation Pearls in patients with this anomaly.] Neurosurg. 55(4):585-9,
• Dorsal root ganglia contrast-enhancement and 1981
enlargement of foraminal neural structures may lead 24. Bouchard]M et al: Preoperative diagnosis of conjoined
to misdiagnosis as nerve sheath tumor roots anomaly with herniated lumbar disks. Surg Neurol.
10(4):229-31, 1978
ISELECTED REFERENCES
1. Bottcher] et al: Conjoined lumbosacral nerve roots:
current aspects of diagnosis. Eur Spine], 2003
2. Haijiao W et al: Diagnosis of lumbosacral nerve root
anomalies by magnetic resonance imaging. ] Spinal Disord.
14(2):143-9,2001
3. Savas R et al: Hypoplastic lumbar pedicle in association
with conjoined nerve root MRI demonstration. Comput
Med Imaging Graph. 22(1):77-9, 1998
4. Sener RN: Sacral pedicle agenesis. Comput Med Imaging
Graph. 21(6):361-3, 1997
5. Prestar F]: Anomalies and malformations of lumbar spinal
nerve roots. Minim Invasive Neurosurg. 39(4):133-7, 1996
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CONJOINED NERVE ROOTS
IIMAGE GALLERY 1
89
Typical
(Left) Axial T2WI MR
demonstrates a large right
nerve root sleeve, arising
rostral to expected level,
containing two different
nerve roots (arrows). (Right)
Sagittal T1WI MR depicts
conjoined L5 and 5 / nerve
roots (arrows) exiting
independently through their
appropriate neural foramen,
the most common variation
of conjoined nerve root
anatomy.
Variant
(Left) Sagittal T1 C+ MR
shows normally enhancing
ORC at all levels except left
L5/S/ (white arrow). Two
enhancing ORCs are seen
within the 5/ foramen (black
arrows), an uncommon
conjoined root variation.
(Right) Axial TI WI MR of
conjoined left L5 and 5/
nerve roots reveals both
ORCs within the left 5/
neural foramen (arrow), an
uncommon conjoined root
anatomical variation.
Variant
(Left) Sagittal TlWI MR
shows two nerve roots
(arrows) transiting a single
aberrant enlarged neural
foramen. (Right) Axial T1 WI
MR depicts both L5 and 5/
nerve root ORCs (arrows)
transiting a single enlarged
neural foramen.
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Sagittal T7WI MR shows linear high signal at L2-3 level Axial T7 WI MR at LJ-4 level shows punctate high signal
(arrow). There is no thickening of filum, and conus is within dorsal thecal sac (arrow).
normal in position.
ITERMINOlOGY MR Findings
Abbreviations and Synonyms • TlWI
o Linear high signal oriented in cranio-caudal
• Synonyms: Fibrolipoma of filum terminale, fatty filum direction on sagittal images
terminale, "fat in the filum" • May occur anywhere from below conus to sacrum
Definitions o Dorsal half of thecal sac in position of filum
• Asymptomatic presence of fat within otherwise terminale
normal size filum terminale o Low signal on fat suppressed sequence
• No tethered cord, conus normal position • T2WI: Tracks fat signal
• Symptomatic patient implies diagnosis of intraspinal • T1 C+: No enhancement
lipoma, not asymptomatic fibrolipoma Imaging Recommendations
• Best imaging tool: MR imaging: Tl WI shows typical fat
appearance, normal conus position and morphology
IIMAGING FINDINGS • Protocol advice: Add fat suppressed sequence if
General Features uncertain of TlWI signal etiology
• Best diagnostic clue: Linear fat signal within filum
terminale on Tl WI
• Location: Filum terminale (conus level to sacrum)
IDIFFERENTIAL DIAGNOSIS
• Size: 1-5 mm Intraspinal lipoma
• Morphology: Linear, "stripe-like" high signal on T1WI • Larger lipomatous mass (> few mm)
CT Findings • Thickening of filum terminale (> 2 mm)
• NECT • Low lying or indistinct conus
o Punctate fat attenuation in dorsal aspect of lumbar Tethered cord
thecal sac • Thick filum, indistinct conus termination, with
o No associated dysraphism smooth transition to filum
• CECT: No enhancement
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BONE ISLAND
1
92
Sagittal T7 C+ MR demonstrates focal area of low signal Sagittal STIR MR shows lesion with low T2 signal, with
in L1 vertebral body, without enhancement. well-defined margins and no adjacent marrow
hyperintensity.
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BONE ISLAND
Key Facts
1
Terminology • Most common in pelvis, femur, ribs 93
• Synonyms: Enostosis, sclerotic island, calcified island, • Size: 1 mm to several em
compact island • Usually normal
• Asymptomatic focal areas of bony sclerosis Pathology
Imaging Findings • General path comments: Normal compact bone
• Best diagnostic clue: Small focal areas of sclerosis, • Etiology: Developmental
with brush-like margins • Epidemiology: Spine involvement in up to 14% of
• Any bone may be involved subjects
ICLINICAL ISSUES
Presentation
• Most common signs/symptoms: Asymptomatic
• Clinical profile: Incidental finding in patient imaged
for back pain
(Left) Axial TlWI MR shows focal low Tl signal in right 51 vertebral
Demographics body without epidural soft tissue. (Right) Sagittal T1WI MR shows 51
• Age: All age groups vertebral lesion as focal Tl hypointensity.
• Gender: M = F
Natural History & Prognosis
• Stable lesions
o Rarely will increase or decrease in size over time
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VENTRICULUS TERMINALIS
1
94
SagittLJl graphic demonstrates uncomplicated fusiform SagittLJl T2WI MR shows mild dilatation of the central
dilatation of the distal central cord canal, typical of canal (arrows) limited to the conus, typical of classic
ventriculus terminalis. ventriculus terminalis.
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VENTRICULUS TERMINALIS
Key Facts
1
Terminology Pathology 95
• Synonyms: Terminal ventricle, fifth ventricle • Simple CSF filled cavity of conus medullaris
• Normal cord microscopic histology
Imaging Findings
• Best diagnostic clue: Mild cystic dilatation of distal Clinical Issues
central spinal cord canal without cord signal • Incidental finding on imaging performed for
abnormality or enhancement unrelated indications
• Size: 2-4 mm (transverse); rarely exceeds 2 cms in • Clinical profile: Patient is asymptomatic or presents
length with nonspecific neurological symptoms
• Conus terminates at normal level (T12 ~ L2)
Diagnostic Checklist
Top Differential Diagnoses • Most important imaging goal is to distinguish from
• Transient dilatation of the central canal cystic cord neoplasm or syrinx
• Hydrosyringomyelia • Isolated mild dilatation of distal central canal in a
• Cystic spinal cord neoplasm normally located conus is nearly always an incidental
• Myelomalacia normal finding
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VENTRICULUS TERMINALIS
1 ICLINICAL ISSUES ISELECTED REFERENCES
96 1. Dullerud R et al: MR imaging of ventriculus terminalis of
Presentation the conus medullaris. A report of two operated patients
• Most common signs/symptoms and a review of the literature. Acta Radiol. 44(4):444-6,
o Incidental finding on imaging performed for 2003
unrelated indications 2. Celli P et al: Cyst of the medullary conus: malformative
• Usually identified during sciatica work-up persistence of terminal ventricle or compressive dilatation?
o Rarely becomes abnormally dilated and Neurosurg Rev. 25(1-2):103-6, 2002
symptomatic, necessitating treatment 3. Kriss VM et al: Sonographic appearance of the ventriculus
terminalis cyst in the neonatal spinal cord. ] Ultrasound
• Bilateral sciatica
Med. 19(3):207-9,2000
• Lower extremity weakness 4. Tortori-Donati P et al: Spinal dysraphism: a review of
• Urinary retention neuroradiological features with embryological correlations
• Clinical profile: Patient is asymptomatic or presents and proposal for a new classification. Neuroradiology.
with nonspecific neurological symptoms 42(7):471-91,2000
5. Unsinn KM et al: US of the spinal cord in newborns:
Demographics spectrum of normal findings, variants, congenital
• Age: Most commonly identified under age 5; may anomalies, and acquired diseases. Radiographics.
occur at any age 20(4):923-38, 2000
• Gender: M = F 6. Truong BC et al: Dilation of the ventriculus terminalis:
sonographic findings.] Ultrasound Med. 17(11):713-5,
Natural History & Prognosis 1998
• No effect on mortality or morbidity 7. Matsubayashi R et al: Cystic dilatation of ventriculus
o Most commonly either stable in size or regresses; terminalis in adults: MRI. Neuroradiology. 40(1):45-7, 1998
8. Agrillo U et al: Symptomatic cystic dilatation of V
size progression not reported ventricle: case report and review of the literature. Eur Spine
• Rare symptomatic surgical cases show symptom ].6(4):281-3, 1997
improvement post-operatively 9. Unsinn KM et al: Sonography of the ventriculus terminalis
in newborns. A]NR Am] Neuroradiol. 17(5):1003-4, 1996
Treatment 10. Tindall et al: The Practice of Neurosurgery. Baltimore,
• No treatment indicated for asymptomatic incidental Williams and Wilkins, 2797, 1996
finding 11. Unsinn KM et al: Ventriculus terminalis of the spinal cord
o MRI follow-up if deemed necessary based on clinical in the neonate: a normal variant on sonography. A]R Am]
findings Roentgenol. 167(5):1341, 1996
• Surgical decompression and management of associated 12. Rypens F et al: Atypical and equivocal sonographic features
of the spinal cord in neonates. Pediatr Radiol.
abnormalities in rare symptomatic cases
25(6):429-32, 1995
o Cyst fenestration +/- cyst shunting to the 13. Coleman LT et al: Ventriculus terminalis of the conus
subarachnoid space, pleural cavity, or peritoneal medullaris: MR findings in children. A]NR Am]
cavity Neuroradiol. 16(7):1421-6, 1995
14. Kriss VM et al: The ventriculus terminalis of the spinal cord
in the neonate: a normal variant on sonography. A]R Am]
I DIAGNOSTIC CHECKLIST Roentgenol. 165(6):1491-3, 1995
15. Iskander B] et al. Terminal hydrosyringomyelia and occult
Consider spinal dysraphism.] Neurosurg 81:513-19, 1994
16. Choi BH et al: The ventriculus terminalis and filum
• Most important imaging goal is to distinguish from
terminale of the human spinal cord. Hum Pathol.
cystic cord neoplasm or syrinx 23(8):916-20, 1992
• Asymptomatic patients require no further imaging 17. Sigal R et al: Ventriculus terminalis of the conus
evaluation medullaris: MR imaging in four patients with congenital
• Symptomatic patients should be monitored using dilatation. A]NR Am] Neuroradiol. 12(4):733-7, 1991
clinical and MR follow-up unless degree of cyst 18. Stewart DH]r et al: Surgical drainage of cyst of the conus
expansion prompts surgical drainage medullaris. Report of three cases. ] Neurosurg.
33(1):106-10, 1970
Image Interpretation Pearls
• Isolated mild dilatation of distal central canal in a
normally located conus is nearly always an incidental
normal finding
o Calcification, septations, nodules, enhancement, or
eccentric location all argue against ventriculus
terminalis and prompt further evaluation
o Beware of truncation or phase ghosting artifact
mimicking a dilated terminal ventricle or syrinx
• Exclude other unsuspected abnormalities that may
predispose to syrinx or cord tethering before
attributing finding to incidental dilatation of
ventriculus terminalis
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VENTRICULUS TERMINALIS
IIMAGE GALLERY 1
97
Typical
(Left) Sagittal T2WI MR
shows mild focal dilation of
the terminal ventricle limited
to the conus, without cord
signal abnormality that
would imply a pathologic
lesion. (Right) Sagittal T7 C+
MR shows no abnormal
enhancement of the terminal
ventricle.
Variant
(Left) Sagittal T2WI MR
demonstrates cystic
enlargement of the terminal
ventricle without other spinal
cord abnormalities. (Right)
Axial T7 C+ MR
demonstrates cystic
ventriculus terminalis
enlargement. Absence of
contrast enhancement helps
to exclude cystic neoplasm.
Variant
(Left) Sagittal T7 C+ MR
shows a large hypointense
ventriculus terminalis cyst
within a normally located
distal conus. The remainder
of the spinal cord was
normal (not shown). (Right)
Axial T7 C+ MR
demonstrates a mildly
eccentric hypointense
ventriculus terminalis cyst.
Absence of contrast
enhancement helps exclude
cystic neoplasm.
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CHIARI I MALFORMATION
1
98
Sagittal graphic demonstrates pointed "peg-like" tonsils Sagittal T2WI MR depicts classic Chiari I changes.
extending below foramen magnum, elongating the Elongated, "peg-shaped" tonsils extend below Cl ring
normally positioned IVth ventricle. (arrow), and display characteristic oblique cerebellar
folia ("sergeant's stripes").
ITERMINOlOGY CT Findings
• NECT: Small posterior fossa =} low torcular, "crowded"
Abbreviations and Synonyms foramen magnum, tonsillar ectopia, flattened spinal
• Synonyms: CM 1, Arnold-Chiari 1 malformation, ACI cord
Definitions • Bone CT
• Elongated, "peg-shaped" cerebellar tonsils extend o Often normal; abnormal cases =} short clivus, CV]
below foramen magnum into cervical spinal canal segmentation/fusion anomalies, small posterior
fossa
MR Findings
IIMAGING FINDINGS • TlWI
General Features o Pointed (not rounded) tonsils ~ 5 mm below
foramen magnum
• Best diagnostic clue: Pointed cerebellar tonsils ~ 5 mm
o "Tight foramen magnum" with small/absent cisterns
below foramen magnum +/- syringohydromyelia
o +/- Elongation of IVth ventricle, hindbrain
(14-75%)
anomalies
• Location: Craniovertebral junction (CV])
• Size: Tonsils> 5 mm below foramen magnum • T2WI
o Oblique tonsillar folia (like "sergeant's stripes")
• Morphology: Low-lying, pointed "peg-like" tonsils with
o +/- Short clivus =} "apparent" descent of 4th
oblique vertical sulci, elongated but normally located
ventricle, medulla
IVth ventricle
o +/- Syringohydromyelia (14-75%)
Radiographic Findings Other Modality Findings
• Radiography: Either normal or short clivus, CV]
• Cine phase contrast (PC) MR: Disorganized CSF
segmentation/fusion anomalies, small posterior fossa
pulsation, 1 brainstem/cerebellar tonsil motion =} 1
(PF)
peak systolic velocity, j flow through foramen
• Myelography: Low-lying tonsils efface CSF at foramen
magnum
magnum
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CHIARI I MALFORMATION
Key Facts
1
Terminology Pathology 99
• Synonyms: CM 1, Arnold-Chiari 1 malformation, • Diagnostic criteria: Herniation of at least one
ACI cerebellar tonsil> 5 mm or herniation of both tonsils
3 mm to 5 mm below a line connecting the basion
Imaging Findings with opisthion
• Best diagnostic clue: Pointed cerebellar tonsils ~ 5
mm below foramen magnum +/- syringohydromyelia Clinical Issues
(14-75%) • Clinical profile: Clinical CMl syndrome: Headache,
• "Tight foramen magnum" with small/absent cisterns pseudotumor-like episodes, Meniere disease-like
• +/- Elongation of IVth ventricle, hindbrain anomalies syndrome, lower cranial nerve and spinal cord signs
• Oblique tonsillar folia (like "sergeant's stripes") • Increasing ectopia + time ~ t likelihood symptoms
Top Differential Diagnoses Diagnostic Checklist
• Normal tonsillar displacement below foramen • Unless tonsils> 5 mm and/or pointed, probably not
magnum clinically significant Chiari I malformation
• Acquired tonsillar herniation ("acquired Chiari 1") • Tonsillar herniation> 12 mm nearly always
symptomatic
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CHIARI I MALFORMATION
1 Microscopic Features
• Symptomatic patients: PF decompression/ resection of
posterior arch C1, +/- duraplasty, cerebellar tonsil
100 • Tonsillar softening or sclerosis with Purkinje/granular resection
cell loss o > 90% ~ brainstem signs
Staging, Grading or Classification Criteria o > 80% ~ hydrosyringomyelia
o +/- Scoliosis arrest
• Diagnostic criteria: Herniation of at least one
• Direct shunting of symptomatic syrinx obsolete; goal
cerebellar tonsil> 5 mm or herniation of both tonsils
3 mm to 5 mm below a line connecting the basion to restore normal CSF flow at foramen magnum
with opisthion
o Herniation of both tonsils 3-5 mm below foramen
magnum + syrinx, cervicomedullary kinking, IVth IDIAGNOSTIC CHECKLIST
ventricular elongation, or pointed tonsils ~ Consider
congenital CM 1 • Unless tonsils> 5 mm and/or pointed, probably not
o Tonsil herniation :0; 5 mm does not exclude CM 1 clinically significant Chiari I malformation
Image Interpretation Pearls
ICLINICAL ISSUES • Tonsillar herniation> 12 mm nearly always
symptomatic
Presentation
• Most common signs/symptoms
o :0; 50% asymptomatic (especially if :0; 5 mm inferior ISELECTED REFERENCES
displacement)
o Symptomatic patients present with constellation of 1. Milhorat TH et al: Tailored operative technique for Chiari
type I malformation using intraoperative color Doppler
findings ultrasonography. Neurosurgery. 53(4):899-905; discussion
• Sudden death (rare) 905-6,2003
• Suboccipital headache, cranial nerve palsy, ocular 2. Tubbs RS et al: Surgical experience in 130 pediatric patients
disturbances, otoneurologic dysfunction, cord with Chiari I malformations. J Neurosurg. 99(2):291-6,
motor or sensory abnormalities, gait disturbance, 2003
neuropathic joint 3. Eule JM et al: Chiari I malformation associated with
• Tonsillar herniation> 12 mm nearly always syringomyelia and scoliosis: a twenty-year review of
symptomatic; "" 30% with tonsils 5-10 mm below surgical and nonsurgical treatment in a pediatric
population. Spine. 27(13):1451-5, 2002
foramen magnum asymptomatic 4. Genitori L et al: Chiari type I anomalies in children and
• CM 1 patients with syrinx nearly always present adolescents: minimally invasive management in a series of
with symptoms referable to syrinx; if the syrinx 53 cases. Childs Nerv Syst. 16(10-11):707-18,2000
extends into medulla, bulbar symptoms 5. Milhorat TH et al: Chiari I malformation redefined: clinical
predominate and radiographic findings for 364 symptomatic patients.
o Trauma is a common precipitating event for Neurosurgery. 44(5):1005-17, 1999
symptom onset (24%) 6. Nishikawa M et al: Pathogenesis of Chiari malformations:
• Clinical profile: Clinical CM1 syndrome: Headache, A morphometric study of the posterior cranial fossa. J
Neurosurg 86: 40-7, 1997
pseudotumor-like episodes, Meniere disease-like 7. Menezes AH: Primary craniovertebral anomalies and the
syndrome, lower cranial nerve and spinal cord signs hindbrain herniation syndrome (Chiari 1): Data base
analysis. Pediatric Neurosurg 23: 260-69, 1995
Demographics 8. Caldemeyer KS et al: Chiari I malformation: association
• Age: 10 months ~ 65 years; syrinx, congenital CVj with hypophosphatemic rickets and MR imaging
anomalies hasten presentation appearance. Radiology. 195(3):733-8, 1995
• Gender: F > M (3:2) 9. Stovner LJ et al: Posterior cranial fossa dimensions in the
Chiari I malformation: relation to pathogenesis and
Natural History & Prognosis clinical presentation. Neuroradiology. 35(2):113-8, 1993
• Natural history not clearly understood 10. Elster AD et al: Chiari I malformations: clinical and
o Many patients asymptomatic for prolonged periods radiologic reappraisal. Radiology. 183(2):347-53, 1992
o Increasing ectopia + time ~ t likelihood symptoms 11. Stovner LJ et al: Syringomyelia in Chiari malformation:
relation to extent of cerebellar tissue herniation.
• Children respond better to treatment than adults Neurosurgery. 31(5):913-7; discussion 917, 1992
• Degree of tonsillar herniation correlates with clinical 12. Pillay PK et al: Symptomatic Chiari malformation in adults:
severity a new classification based on magnetic resonance imaging
with clinical and prognostic significance. Neurosurgery.
Treatment 28(5):639-45, 1991
• Treating asymptomatic patients is controversial 13. Barkovich AJ et al: Significance of cerebellar tonsillar
o "Radiographic" CM 1 (no syrinx), no corresponding position on MR. AJNR Am J Neuroradiol. 7(5):795-9, 1986
clinical signs/symptoms ~ conservative
management
o Asymptomatic Chiari I + syrinx ~ consider
decompression
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CHIARI I MALFORMATION
IIMAGE GALLERY 1
101
Variant
(Left) Sagittal T1WI MR
(congenital CM 7) reveals
large ectopic tonsils
obliterating basilar CSF
spaces. Associated findings
include short, flattened clivus
and cervical syringomyelia
(arrow). (Right) Sagittal
T1WI MR depicts moderate
tonsillar ectopia associated
with variant focal cystic
syringomyelia.
Variant
(Left) Sagittal T1WI MR
shows "peg-like" tonsils
descending inferiorly to _
posterior C 7 level and a large
cervical syringomyelia.
(RighI) Axial T2WI MR
displays medullary extension
(arrow) of cervical
syringomyelia
(syringobulbia) in a Chiari 7
patient with clinical
brainstem symptoms.
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LATERAL MENINGOCELE
1
102
Axial graphic shows a large left lateral thoracic Coronal T2WI MR (Marfan syndrome) shows multiple
meningocele producing pedicular erosion, transverse lateral thoracic meningoceles (arrows).
process remodelling, and widening of the neural
foramen.
-:-' \
~_..c:u~\t.....
I~~'_~'~ ).. ~~~
~.'
-,.. ',.,.
~~, '"
'. _.'4.._
~
, 'J ~ •
~_ ..
\ . - 'I".
._---;..--,.
-.1
.
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LATERAL MENINGOCELE
Key Facts
1
Imaging Findings • Foregut duplication cyst 103
• Best diagnostic clue: CSF signal/density meningeal Pathology
protrusion through neural foramen into adjacent • Strong association with neurofibromatosis type 1
intercostal/extrapleural space (NFl) - 85%
• Thoracic> lumbar spine
• R > Li 10% bilateral Clinical Issues
• Wide neural forameni +/- pedicular thinning, • Age: Most commonly present during 4th ~ 5th
posterior vertebral scalloping (usually) decades of life
• Tl C+: No enhancementi distinguishes from nerve • Gender: M = F
sheath tumor or inflammation (CIDP) • Most remain asymptomatic unless very large or
scoliosis causes symptoms
Top Differential Diagnoses
• Nerve sheath tumor Diagnostic Checklist
• Radicular (meningeal) cyst • Lateral meningocele prompts search for
• Chronic inflammatory demyelinating history/stigmata of Nfl or connective tissue disorder
polyneuropathy
o CSF signal intensity (hypointense) mass in o Cyst separate from dural sac, unlike meningocele
contiguity with thecal sac • Nerve root definable as discrete structure within or
o Pedicular thinning, neural foraminal widening +/- adjacent to cyst
posterior vertebral scalloping
• T2WI: CSF signal intensity (hyperintense) mass in Chronic inflammatory demyelinating
contiguity with thecal saCi rarely see neural elements polyneuropathy
within meningocele • Solid fusiform nerve root enlargement, enhancement
• Tl C+: No enhancementi distinguishes from nerve • Clinical and laboratory findings characteristic
sheath tumor or inflammation (CIDP)
Foregut duplication cyst
Ultrasonographic Findings • Bronchogenic most commoni may contain GI mucosa
• Real Time o Seldom contiguous with neural foramen
o Posterior mediastinal or lumbar hypoechoic • Proximity to spinal canal +/- vertebral anomalies =
paraspinal cystic mass contiguous with an expanded neurenteric cyst
spinal canal
• Displaces and compresses adjacent spinal cord
o Ultrasound is primary diagnostic tool in utero, IPATHOLOGY
screening newborn infants
• Pulsed Doppler: No vascular flow pattern General Features
• Color Doppler: Avascular hypoechoic mass • General path comments: Dural sac diverticulum,
pedicular erosion, neural foraminal widening, and
Imaging Recommendations posterior vertebral scalloping
• Best imaging tool: MR imaging • Genetics
• Protocol advice o Strong association with neurofibromatosis type 1
o Consider sonography for newborn screeningi (NFl) - 85%
follow-up with MRI to clarify positive ultrasound • Most common posterior mediastinal mass
study o Less common with Ehlers-Danlos, Marfan
o MR imaging for diagnosis, pre-operative planning syndromes
o Bone CT to evaluate pedicles, vertebral bodies • Etiology
(particularly if surgery is contemplated) o Meningocele 2° to primary meningeal dysplasia
• Meningeal weakness permits dural sac to focally
stretch in response to repetitive CSF pulsation ~
IDIFFERENTIAL DIAGNOSIS enlarges neural foramina
Nerve sheath tumor • Secondary osseous remodeling permits further
herniation
• Less hyperintense than CSF on T2WI, higher signal o Posterior vertebral scalloping with dural dysplasia ~
intensity than CSF on TlWI same etiology
• Contrast enhancement implies tumor • Epidemiology: Uncommon in NFl and inherited
o Caveats: Some schwannomas may appear cystic, and connective tissue disorders, but substantially more
some neurofibromas minimally enhance common than occurence as an isolated lesion
Radicular (meningeal) cyst • Associated abnormalities
• CSF signal intensity/density cyst within neural o Occasionally isolated finding
foramen
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LATERAL MENINGOCELE
1 o +/- Co-existent lumbar and thoracic lateral
meningoceles Image Interpretation Pearls
104 o +/- Findings specific to hereditary disorder • MR shows nonenhancing CSF signal intensity/density
• Nfl: Dural ectasia, nerve sheath tumors, CNS mass extending through an enlarged neural foramen
neoplasms, pheochromocytomas, interstitial
pulmonary fibrosis, skin and subcutaneous
neurofibromas ISELECTED REFERENCES
• Marfan syndrome: Dural ectasia, vascular 1. Kubota M et al: Lateral thoracic meningocele presenting as
dissection/aneurysm, lens dislocation, joint laxity a retromediastinal mass. Br] Neurosurg. 16(6):607-8, 2002
2. Baysefer A et al: Lateral intrathoracic meningocele
Gross Pathologic & Surgical Features associated with a spinal intradural arachnoid cyst. Pediatr
• Scalloping of pedicles, laminae, and vertebral bodies Neurosurg. 35(2):107-10, 2001
adjacent to meningocele 3. Zibis AH et al: Unusual causes of spinal foraminal
• Enlarged central spinal canal, neural foramina widening. Eur Radiol. 10(1):144-8,2000
4. Unsinn KM et al: US of the spinal cord in newborns:
• Cord position variable; usually displaced away from spectrum of normal findings, variants, congenital
meningocele anomalies, and acquired diseases. Radiographies.
• Scoliosis convex toward meningocele 20(4):923-38,2000
5. Barkovich A: Pediatric neuroimaging. 3rd ed. Philadelphia,
Microscopic Features Lippincott-Raven. 398, 667, 2000
• Dura/arachnoid lined outpouching of thecal sac 6. Chen SS et al: Multiple bilateral thoracic meningoceles
without neurofibromatosis: a case report. Zhonghua Yi Xue
Za Zhi (Taipei). 61(12):736-40, 1998
I CLINICAL ISSUES 7. Gripp KW et al: Lateral meningocele syndrome: three new
patients and review of the literature. Am] Med Genet.
Presentation 70(3):229-39, 1997
8. Strollo DC et al: Primary mediastinal tumors: part II.
• Most common signs/symptoms
Tumors of the middle and posterior mediastinum. Chest.
o Asymptomatic (most common) 112(5):1344-57, 1997
o Nonspecific motor or sensory symptoms referable to 9. Ball W: Pediatric Neuroradiology. Philadelphia,
cord/nerve root compression Lippincott-Raven. 727, 1997
o Other signs/symptoms 10. Gibbens DT et al: Chest case of the day. Lateral thoracic
• Respiratory embarrassment (neonates); very large meningocele in a patient with neurofibromatosis. A]R Am]
meningocele fills thoracic cavity Roentgenol. 156(6):1299-300, 1991
• Clinical profile 11. Nakasu Y et al: ThoraciC meningocele in
neurofibromatosis: CT and MR findings. ] Comput Assist
o Asymptomatic patient (incidental discovery)
Tomogr. 15(6):1062-4, 1991
o +/- Scoliosis evaluation 12. Chee CP: Lateral thoracic meningocele associated with
o Nfl: Cutaneous cafe-au-Iait spots,cutaneous and neurofibromatosis: total excision by posterolateral
subcutaneous neurofibromas, +/- kyphoscoliosis extradural approach. A case report. Spine. 14(1):129-31,
o Connective tissue disorder: Frequently tall, joint 1989
hypermobility, lens dislocation, +/- normal 13. Richaud]: Spinal meningeal malformations in children
intelligence, +/- scoliosis (without meningoceles or meningomyeloceles). Childs
Nerv Syst. 4(2):79-87, 1988
Demographics 14. Maiuri F et al: Lateral thoracic meningocele. Surg Neurol.
• Age: Most commonly present during 4th =:> 5th 26(4):409-12, 1986
decades of life 15. Weinreb]C et al: CT metrizamide myelography in multiple
bilateral intrathoracic meningoceles. ] Comput Assist
• Gender: M = F Tomogr. 8(2):324-6, 1984
Natural History & Prognosis 16. Erkulvrawatr S et al: Intrathoracic meningoceles and
neurofibromatosis. Arch Neurol. 36(9):557-9, 1979
• Most remain asymptomatic unless very large or 17. Booth AE: Lateral thoracic meningocele. ] Neurol
scoliosis causes symptoms Neurosurg Psychiatry. 32(2):111-5, 1969
• Most static in size; occasionally grow slowly 18. Bunner R: Lateral intrathoracic meningocele. Acta Radiol.
• May disappear after hydrocephalus shunting 51(1):1-9, 1959
• Excellent prognosis after surgical resection
Treatment
• Options, risks, complications
o Surgical ligation of dural sac neck, resection of
meningocele
o Correction/stabilization of scoliosis
IDIAGNOSTIC CHECKLIST
Consider
• Lateral meningocele prompts search for
history/stigmata of NFl or connective tissue disorder
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LATERAL MENINGOCELE
IIMAGE GALLERY 1
105
Variant
(Left) Axial T2Wf MR
(neurofibromatosis type 7)
shows a large left lateral
lumbar meningocele
(arrows) in conjunction with
extensive dural dysplasia
(open arrows) and marked
left pedicular erosion. (Right)
Axial NECT following
myelography (NFl) shows a
large left lateral lumbar
meningocele (arrows) and
extensive dural dysplasia
(open arrows) with marked
left pedicular erosion.
Variant
(Left) Lateral myelography
(neurofibromatosis type 1)
shows marked dural
dysplasia with posterior
vertebral scalloping (arrows)
and multiple lumbar
meningoce/es. (Right)
Anteroposterior myelography
(neurofibromatosis type 7)
demonstrates multiple
bilateral lumbar
meningoceles (arrows).
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DORSAL SPINAL MENINGOCELE
1
106
Sagittal T2WI MR shows a skin covered simple lumbar Axial T2WI MR demonstrates a simple dorsal
dorsal meningocele extending through a dorsal meningocele protruding through a single-level posterior
dysraphic defect into subcutaneous fat. dysraphic defect into subcutaneous fat.
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DORSAL SPINAL MENINGOCELE
Key Facts
1
Terminology Pathology 107
• Synonyms: Simple meningocele, simple spinal • Meningocele is always skin covered; skin may be
meningocele, posterior meningocele dysplastic or ulcerated
Imaging Findings Clinical Issues
• Best diagnostic clue: Skin covered dorsal dural sac • Palpable skin-covered mass or incidental discovery
protruding thorough posterior osseous defect dUring imaging for other indications
• Best imaging tool: MR imaging best shows dural sac
characteristics, associated vertebral or spinal cord Diagnostic Checklist
anomalies • Ultrasound for infants to determine cord level,
presence or absence of neural elements in
Top Differential Diagnoses meningocele
• Lipomyelomeningocele • MR is best modality to definitively characterize
• Terminal myelocystocele meningocele, evaluate for other spinal abnormalities
• Myelomeningocele • Imaging goals are lesion detection, determination of
simple or complex morphology, and exclusion of
other spinal anomalies
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IDIAGNOSTIC CHECKLIST
Consider
• Ultrasound for infants to determine cord level,
presence or absence of neural elements in
meningocele
• MR is best modality to definitively characterize
meningocele, evaluate for other spinal abnormalities
Image Interpretation Pearls
• Imaging goals are lesion detection, determination of
simple or complex morphology, and exclusion of
other spinal anomalies
o Critical to determine whether neural elements reside
within sac prior to surgery
• Meningocele may be most obvious abnormality that
masks other less obvious but clinically more important
lesions
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Variant
(Left) Sagittal T2WI MR
depicts a large
cervicothoracic dorsal
meningocele extending into
the subcutaneous tissues
through a thin dysraphic
isthmus (Courtesy 5. Blaser,
MO). (Right) Axial T2WI MR
shows a large dorsal
meningocele passing through
a narrow posterior
dysraphism (Courtesy 5.
Blaser, MO).
Variant
(Left) Sagittal ultrasound real
time depicts a low-lying cord
(open arrow) and the rostral
portion (arrow) of a large
dorsal meningocele
(Courtesy R. Boyer, MO).
(Right) Transverse
ultrasound shows a small
dorsal meningocele sac
(arrow) extending through a
wide posterior osseous
defect (open arrows)
(Courtesy R. Boyer, MO).
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NEUROFIBROMATOSIS TYPE 1
1
110
Coronal graphic shows bilateral spinal nerve root and Coronal STIR MR demonstrates characteristic extensive
brachial plexus neurofibromas. Intramedullary cervical bilateral hyperintense nerve root and brachial plexus
glial tumor produces focal cord expansion, cystic central neurofibromas. Small cutaneous neurofibromas
canal dilatation. (arrows) are also visible.
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NEUROFIBROMATOSIS TYPE 1
Key Facts
1
Terminology Pathology 111
• Synonyms: NFl, von Recklinghausen disease, • Plexiform neurofibroma is the hallmark of NFl
peripheral neurofibromatosis
Clinical Issues
Imaging Findings • Skeletal deformity common (25-40%)
• Best diagnostic clue: Kyphoscoliosis +/- multiple • Palpable spinal or cutaneous mass
nerve root tumors, plexiform neurofibroma, dural • Pigmentation anomalies (cafe-au-Iait, axillary
ectasia/lateral meningocele freckling, Lisch nodules) ~ 90% NFl patients
Top Differential Diagnoses Diagnostic Checklist
• Neurofibromatosis type 2 (NF2, central • Multiple nerve sheath tumors, ~ 1 plexiform
neurofibromatosis) neurofibroma, bizarre kyphoscoliosis with deformed
• Chronic inflammatory demyelinating vertebra - consider NFl
polyneuropathy (CIDP) • Absence of visible stigmata does not exclude NFl
• Congenital hypertrophic polyradiculoneuropathies • Characteristic PNF imaging appearance best displayed
using fat-saturated T2WI or STIR MR imaging
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NEUROFIBROMATOSIS TYPE 1
1 o Localized NF (90% all NF)
• Most common NF in both NFl, non-NFl patients
• NF growth usually slow; rapid growth associated with
pregnancy, puberty, or malignant transformation
112 • Cutaneous and deep nerves, spinal nerve roots
• NFl: Larger, multiple, more frequently involve Treatment
large deep nerves (sciatic nerve, brachial plexus) • Conservative observation; intervention dictated by
• Malignant transformation rare clinical symptomatology, appearance of neoplasm
o Diffuse NF • Surgical resection of symptomatic localized NF, spinal
• Infiltrating subcutaneous tumor; rarely affects cord tumors
spinal nerves, majority (90%) unassociated with • PNF invasive, rarely resectable; observation +/-
NFl biological or chemotherapeutic (thalidomide,
o Plexiform NF (pathognomonic for NFl) antihistamines, maturation agents, antiangiogenic
• Diffuse enlargement of major nerve drugs) intervention
trunks/branches ~ bulky rope-like ("bag of • Spinal fusion reserved for symptomatic or severe
worms") nerve expansion with adjacent tissue kyphoscoliosis
distortion
• Commonly large, bilateral, multilevel with
predilection for sciatic nerve, brachial plexus IDIAGNOSTIC CHECKLIST
• ~ 5% risk malignant degeneration ~ sarcoma
Consider
Microscopic Features • Multiple nerve sheath tumors, ~ 1 plexiform
• Neoplastic Schwann cells + perineural fibroblasts grow neurofibroma, bizarre kyphoscoliosis with deformed
along nerve fascicles vertebra ~ consider Nfl
o Collagen fibers, mucoid/myxoid matrix, tumor, • Absence of visible stigmata does not exclude NFl
nerve fascicles intermixed
Image Interpretation Pearls
o S-100 positive, mitotic figures rare unless malignant
degeneration • Characteristic PNF imaging appearance best displayed
using fat-saturated T2WI or STIR MR imaging
Staging, Grading or Classification Criteria
• Consensus Development Conference on
Neurofibromatosis (NIH, 1987) 2 or more of the ISELECTED REFERENCES
following criteria 1. Cardona S et al: Evaluation of F18-deoxyglucose positron
o > 6 cafe-au-Iait spots measuring ~ 15 mm in adults emission tomography (FDG-PET) to assess the nature of
or 5 mm in children, ~ 2 neurofibromas of any type neurogenic tumours. Eur J Surg Oncol. 29(6):536-41, 2003
or ~ 1 plexiform neurofibroma, axillary or inguinal 2. Iannicelli E et al: Radiol Med (Torino). English, Italian.
freckling, optic glioma, two or more Lisch nodules MID: Apr;103(4):332-43, 2002
(iris hamartomas), distinctive bony lesion (sphenoid 3. Packer RJ et al: Plexiform neurofibromas in NFl: toward
biologic-based therapy. Neurology. 58(10):1461-70, 2002
wing dysplasia, thinning of long bone +/- 4. Simoens WA et al: with radiologist's experience? Eur
pseudoarthrosis, first-degree relative with NFl Radiol. 11(2):250-7,2001
5. Lin J et al: Cross-sectional imaging of peripheral nerve
sheath tumors: characteristic signs on CT, MR imaging,
I CLINICAL ISSUES and sonography. AJR Am J Roentgenol. 176(1):75-82, 2001
6. Solomon SB et al: Positron emission tomography in the
Presentation detection and management of sarcomatous transformation
• Most common signs/symptoms in neurofibromatosis. Clin Nucl Med. 26(6):525-8, 2001
o Skeletal deformity common (25-40%) 7. Ferner RE et al: Evaluation of (18)fluorodeoxyglucose
positron emission tomography «18)FDG PET) in the
• Focal or acute angle kyphoscoliosis +/- detection of malignant peripheral nerve sheath tumours
myelopathy arising from within pleXiform neurofibromas in
• Extremity bowing or overgrowth neurofibromatosis 1. J Neurol Neurosurg Psychiatry.
o Palpable spinal or cutaneous mass 68(3):353-7, 2000
o Pigmentation anomalies (cafe-au-Iait, axillary 8. von Deimling A et al: Neurofibromatosis Type 1. In:
freckling, Lisch nodules) ~ 90% NFl patients Kleihues P, Cavanee W (eds), Pathology and genetics of
• Clinical profile tumors of the nervous system. Lyon, IARC Press, 216-218,
o Severity of clinical appearance highly variable 2000
o Classic NFl triad: Cutaneous lesions, skeletal 9. Mukonoweshuro W et al: Neurofibromatosis type 1: the
role of neuroradiology. Neuropediatrics. 30(3):111-9, 1999
deformity, and mental deficiency 10. Murphey M et al: From the archives of the AFIP. Imaging
Demographics of musculoskeletal neurogenic tumors:
radiologic-pathologic correlation. Radiographies.
• Age: Childhood diagnosis; minimally affected patients 19(5):1253-80, 1999
may be diagnosed as adults
• Gender: M = F
• Ethnicity: 1 Frequency in Arab-Israeli populations
Natural History & Prognosis
• Kyphosis, scoliosis frequently progressive
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NEUROFIBROMATOSIS TYPE 1
IIMAGE GALLERY 1
113
Typical
(Left) Axial T7 C+ MR shows
bilateral nerve root localized
neurofibromas; the left
tumor extends along the
dorsal primary ramus
(arrow) into paraspinal
muscle. (Right) Sagittal T2WI
MR depicts multiple ventral
intradural, extramedullary
neurofibromas producing
spinal cord compression
(arrow).
Variant
(Left) Coronal STIR MR
reveals a large plexiform
neurofibroma extending
along the left paraspinal
sympathetic chain,
remodeling the adjacent
vertebral bodies and
contributing to scoliosis.
(Right) Axial NECT following
myelography shows marked
dural dysplasia and left
lumbar lateral meningocele.
Posterior vertebral scalloping
and erosion/hypoplasia of
left pedicle are characteristic.
Variant
(Left) Sagittal T2WI MR
shows a holocord
astrocytoma extending into
the brainstem with large
neoplastic syrinx. Vertebral
canal widening mimics that
produced by dural dysplasia.
(Right) Lateral radiography
demonstrates bizarre acute
angle cervical kyphosis with
marked dural ectasia and
neural foraminal
enlargement, highly
characteristic of NFl.
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NEUROFIBROMATOSIS TYPE 2
1
114
\ .. ;~. ",
,l'~
li;/ J4:f~rr"'
1."",:;"
.r" '.t ,','
Drop Metastases Drop Metastases Hemangiobfastomas
r! ~
....,1 ~,......
NS Meningioma
"
i ~
'\
. ~
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NEUROFIBROMATOSIS TYPE 2
Key facts
1
Terminology Pathology 115
• Mnemonic for NF2 tumors = MISME; multiple • 22q12 deletion correlates with loss of NF2 gene
inherited schwannomas, meningiomas, & product "Merlin" (aka Schwannomin)
ependymomas
Clinical Issues
Imaging Findings • Nearly half initially present with hearing loss
• Best diagnostic clue: Multiple spinal tumors of • Up to 45% with extramedullary tumors exhibit
various histologic types signs/symptoms of cord compression
• Best imaging tool: MRI + gadolinium • Few patients require therapeutic intervention for
• Protocol advice: Contrast-enhanced imaging is best intramedullary tumors which often remain quiescent
method for detecting lesions regardless of size • Intradural, extramedullary tumors frequently lead to
surgical intervention
Top Differential Diagnoses
• Metastases Diagnostic Checklist
• Hemangioblastomas • Presence of multiple & different pathologic types of
• Non-syndromic schwannoma, meningioma, spinal tumors is highly suggestive of NF2
ependymoma • Screen using MRI C+ of brain & entire spine
o Ependymomas
• Iso- to slightly hyperintense Lymphoma
• Centrally located with cord expansion • May coat spinal cord surface
• T2WI
o Schwannomas
• Well-delineated rounded hyperintense mass IPATHOLOGY
• May undergo hemorrhage or cystiC degeneration
General Features
o Meningiomas
• May be heterogeneous • General path comments: NF2 mostly associated with
• Best defines high occurrence of cord compression tumors of Schwann cells & meninges
o Ependymomas: Hyperintense; centrally located with • Genetics
cord expansion o Inherited autosomal dominant syndrome
o Ill-defined hyperintense lesion may be low grade o 22q12 deletion correlates with loss of NF2 gene
astrocytoma product "Merlin" (aka Schwannomin)
o Any intramedullary lesion may have syrinx • Etiology
o Chromosomal 22 deletion with eventual
• Tl C+
o Schwannomas: Enhance intensely; homogeneously inactivation of Merlin functionality
when small, heterogeneously when large & cystic • 1st event is 22 chromosomal loss
o Meningiomas: Enhance intensely (often to a lesser • "Second hit theory": Remaining single NF2 copy is
degree than schwannomas) & homogeneously mutated; vast majority are null mutations
o Ependymomas: Enhancing centrally located mass • Results in truncated, poorly or non-functional
Merlin protein
Imaging Recommendations • Epidemiology
• Best imaging tool: MRI + gadolinium olin 50,000 live births worldwide
• Protocol advice: Contrast-enhanced imaging is best o Intradural spinal tumors are present in up to 65%
method for detecting lesions regardless of size patients at initial presentation for imaging
• 84% have intramedullary tumors
• 87% have intradural extramedullary tumors
IDIFFERENTIAL DIAGNOSIS Gross Pathologic & Surgical Features
Metastases • Multiple tumors of different histiologic types
• Originate from leptomeninges Microscopic Features
• Eccentrically placed, rarely within center of cord
• Merlin associates at cell cytoskeleton near plasma
Hemangioblastomas membrane & inhibits cell proliferation, adhesion, and
• Often associated with von-Hippel Lindau migration
• Originate from leptomeninges • Merlin monoclonal antibody immunohistochemistry
• May have associated cyst exhibits consistent immunostaining of Schwann cells
• NF2 histopathology no different than that of
Non-syndromic schwannoma, meningioma, nonsyndromic tumors; exception is the multiplicity
ependymoma Staging, Grading or Classification Criteria
• Usually solitary • Definite diagnosis of NF2
• Imaging appears identical to syndromic tumors o Bilateral CN8 (vestibular) schwannomas
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NEUROFIBROMATOSIS TYPE 2
1 o 1st degree relative with NF2 & either unilateral early
Natural History & Prognosis
onset vestibular schwannoma (age < 30 years) or any
116 2 • Many lead relatively normal lifespans
• Meningioma, glioma, schwannoma, juvenile • Few patients require therapeutic intervention for
posterior subcapsular lenticular opacity intramedullary tumors which often remain quiescent
• Presumptive diagnosis of NF2 • Intradural, extramedullary tumors frequently lead to
o Early onset unilateral CNS schwannomas (age < 30 surgical intervention
years) and one of the following o Percentage of patients with extramedullary tumors
• Meningioma, glioma, schwannoma, juvenile who undergo surgery is about 5x higher than the
posterior subcapsular lenticular opacity percentage of patients with intramedullary tumors
o Multiple meningiomas (> 2) and unilateral o Higher surgical rate is result of high number of
vestibular schwannoma or one of the following tumors & frequent occurrence of cord compression
• Glioma, schwannoma, juvenile posterior o Schwannomas are present more often & in higher
subcapsular lenticular opacity numbers than meningiomas and have more surgical
• Germline study findings support genotype-phenotype procedures overall
correlation of tumor grading o Meningiomas account for a disproportionate
o Nonsense & frame shift mutations number of symptomatic lesions
• Usually associated with severe disease phenotype • Meningiomas comprise"" 12% of extramedullary
• A higher percentage of these patients have tumors yet account for 37% of extramedullary
intramedullary tumors tumors requiring excision
• Have higher numbers of all spinal tumors; • Suggests NF2 meningiomas are more aggressive
intramedullary & extramedullary Treatment
• Younger at the onset of symptoms, diagnosis of • Tumor resection is mainstay of NF2 treatment
NF2, and presentation for imaging
• Intramedullary tumors: Monitor with regular imaging
o Missense mutations & large gene deletions are found
& appropriate clinical correlation
with mild phenotype o Relatively indolent course of most intramedullary
o Splice-site mutations; severe or mild phenotype
tumors & anticipated burden from other
• Dependent on intron involved and its affect on spinal/intracranial tumors must be considered ~
protein functionality standard aggressive management may not be
• Imaging classification of NF2 spinal tumors warranted, even in symptomatic patient
o Intradural, extramedullary tumors
• Intradural, extramedullary tumors: Early removal of
• Schwannomas: Round & located near a nerve root rapidly growing or symptomatic tumors
• Meningiomas: Flat surface of dural attachment • First-degree relatives need to be evaluated
o Three cardinal features of intramedullary tumors
• Central location within cord parenchyma
• Intense enhancement IDIAGNOSTIC CHECKLIST
• Multiplicity, often too many to count
Consider
• Presence of multiple & different pathologic types of
ICLINICAL ISSUES spinal tumors is highly suggestive of NF2
Presentation Image Interpretation Pearls
• Most common signs/symptoms • Screen using MRI C+ of brain & entire spine
o Nearly half initially present with hearing loss • Imaging follow-up of patients with spinal tumors
o Up to 45% with extramedullary tumors exhibit should be based on knowledge of tumor location,
signs/symptoms of cord compression number, & suspected histologic type
• Varies depending on location
• Weakness & sensory loss at or below level
• Spasticity, pain, loss of bowel/bladder control ISELECTED REFERENCES
• Clinical profile 1. Patronas N] et al: Intramedullary and spinal canal tumors
o Detection rates for genetic testing"" 65% in patients with neurofibromatosis 2: MR imaging findings
o Juvenile subcapsular lens opacities common and correlation with genotype. Radiology. 218(2):434-42,
o Retinal & choroidal hamartomas in 10-20% 2001
o Cafe-au-lait spots < 50% 2. Evans DG et al: Neurofibromatosis type 2. ] Med Genet.
o Minimal to no cutaneous neurofibromas 37(12):897-904,2000
o Cutaneous schwannomas in 2/3rds 3. den Bakker MA et al: Expression of the neurofibromatosis
type 2 gene in human tissues. ] Histochem Cytochem.
Demographics 47(11):1471-80, 1999
4. Kluwe L et al: Identification of NF2 germ-line mutations
• Age
o Genetic disease present at conception and comparison with neurofibromatosis 2 phenotypes.
Hum Genet. 98(5):534-8, 1996
o Become symptomatic 2nd-3rd decades 5. Mautner VF et al: The neuroimaging and clinical spectrum
• Gender: M = F of neurofibromatosis 2. Neurosurgery. 38(5):880-5;
• Ethnicity: No racial predilection discussion 885-6, 1996
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NEUROFIBROMATOSIS TYPE 2
IIMAGE GALLERY 1
117
Typical
(Left) Sagittal TT C+ MR
demonstrates numerous
rounded homogenously
enhancing schwannomas
(arrows). Contrast increases
conspicuity. Note close
approximation of each lesion
to nerve root. (Right) Axial
TT C+ MR shows typical
rounded homogeneously
enhancing appearance of an
intradural extramedullary
schwannoma.
(Left) Coronal TT C+ MR
shows round enhancing
schwannomas in close
approximation to nerve roots
(arrows), enhancing
meningioma with broad
dural base (open arrow), &
dumbbell schwannoma
(curved arrow). (Right)
Coronal T2WI MR
demonstrates large cystic
hyperintense
dumbbell-shaped
schwannoma extending
intradural to extradural
through neural foramen.
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DURAL DYSPLASIA
1
118
Sagittal graphic demonstrates scalloping of the posterior Sagittal T2WI MR (neurofibromatosis type 1) shows
vertebral bodies with central canal enlargement. Also classic posterior vertebral scalloping and patulous dural
shown (inset) are bilateral lumbar lateral meningoce/es. sac from L2 through 52 .
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DURAL DYSPLASIA
Key Facts
1
Terminology Pathology 119
• Synonym: Dural ectasia • General path comments: Genetic predisposition =?
• Patulous dural sac with posterior vertebral scalloping primary meningeal dysplasia =? weakness in
meninges =? expansion, secondarily remodeling of
Imaging Findings posterior vertebral body and pedicular thinning =?
• Best diagnostic clue: Smooth "C" shaped scalloping of further dural sac expansion
posterior vertebral bodies with patulous dural sac • NFl: Autosomal dominant (chromosome 17q12)
• Location: Lumbar> cervical, thoracic • Marfan: Autosomal dominant
• Posterior vertebral scalloping, expansion of osseous • Homocystinuria: Autosomal recessive
spinal canal, patulous dural sac, +/- kyphoscoliosis • Ehler-Danlos: Autosomal dominant
• +/- Pedicular thinning, lateral meningocele(s)
Clinical Issues
Top Differential Diagnoses • Moderate to severe back pain> 50% of Marfan
• Congenital vertebral dysplasia patients; presence, degree of dural ectasia associated
• Spinal tumor or syrinx with back pain
• Cauda equina syndrome of ankylosing spondylitis
(AS)
a Not useful for imaging spine deformity; primary a Homocystinuria: Autosomal recessive
utility is detecting associated vascular anomalies a Ehler-Danlos: Autosomal dominant
a Ascending aortic aneurysm ("tulip bulb • Etiology
configuration") suggests Marfan syndrome a Nfl: Primary mesenchymal disorder
a Marfan syndrome: Primary connective tissue defect
Imaging Recommendations unknown
• MRI shows osseous abnormalities well a Ehlers-Danlos: > 10 different types of collagen
• Additionally, MRI is most useful modality to exclude synthesis defects
syrinx or tumor as cause of canal enlargement before a Homocystinuria: Cystathionine beta-synthetase
attributing to dural ectasia deficiency
• Epidemiology
a Nfl: 1/4,000, 50% new mutations; dural ectasia
I DIFFERENTIAL DIAGNOSIS common
Congenital vertebral dysplasia a Marfan: 1/5,000 (United States); dural ectasia
present> 60% patients
• Achondroplasia, mucopolysaccharidosis, osteogenesis a Homocystinuria: 1/344,000 worldwide; dural
inperfecta (tarda) dysplasia less common than Marfan
• Search for appropriate family history, clinical stigmata a Ehler-Danlos: 1/400,000 (worldwide); dural dysplasia
Spinal tumor or syrinx less common than Marfan
• Astrocytoma, ependymoma, nerve sheath tumor, • Associated abnormalities
syrinx a Lateral thoracic or lumbar meningocele, anterior
• Characteristic imaging findings lead to correct sacral meningocele
diagnosis a Kyphoscoliosis
a Joint hypermobility, lens abnormalities, aneurysm,
Cauda equina syndrome of ankylosing arterial dissection (connective tissue disorders)
spondylitis (AS) a Peripheral and central neoplasms (NFl)
• Irregular lumbar canal expansion Gross Pathologic & Surgical Features
• Proposed etiology for dural ectasia; proliferative • Enlarged CSF thecal sac, remodeled posterior vertebral
inflammatory synovium =? cauda equina symptoms bodies
• Imaging and clinical stigmata of AS typically present • Dura in ectatic areas is extremely thin, fragile
Microscopic Features
IPATHOLOGY • Dural thinning in ectatic areas
General Features
• General path comments: Genetic predisposition =? ICLINICAL ISSUES
primary meningeal dysplasia =? weakness in meninges
=? expansion, secondarily remodeling of posterior Presentation
vertebral body and pedicular thinning =? further dural • Most common signs/symptoms
sac expansion a Back pain +/- radiculopathy
• Genetics·
a NFl: Autosomal dominant (chromosome 17q12)
a Marfan: Autosomal dominant
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DURAL DYSPLASIA
1 • Moderate to severe back pain> 50% of Marfan
patients; presence, degree of dural ectasia
o Pseudoarthrosis, CNS/PNS tumors => Nfl
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DURAL DYSPLASIA
IIMAGE GALLERY 1
121
Variant
(Left) Sagittal T2WI MR
(neurofibromatosis type 1)
shows extensive scalloping of
the posterior cervical
vertebral bodies and clivus
(Courtesy C. Hedlund, 00).
(Right) Axial bone CT
demonstrates posterior
vertebral remodeling
(arrows) and right pedicle
thinning. The left pedicle
demonstrates stress fracture
with pseudoarthrosis (open
arrow).
Variant
(Left) Sagittal T2WI MR
(Madan syndrome) shows
striking posterior vertebral
scalloping and dural ectasia.
(Right) Axial T2WI MR
(Madan syndrome) depicts
dural ectasia producing
marked posterior vertebral
scalloping (arrow),
associated with large
bilateral lumbar
meningoceles (open arrows).
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CONGENITAL SPINAL STENOSIS
1
122
Sagittal graphic shows marked congenital AP narrowing Sagittal T1WI MR demonstrates moderate congenital AP
of the central spinal canal. canal diameter reduction spanning L3 through the
sacrum,
Achondroplasia Mucopolysaccharidosis
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CONGENITAL SPINAL STENOSIS
Key Facts
1
Terminology • Neurogenic claudication: Radiating leg pain with 123
• Synonyms: "Short pedicle" syndrome, congenital walking, relieved by rest, bending forward
short pedicles, developmental spinal stenosis • Cauda equina syndrome (rare): Bilateral leg weakness,
urinary retention due to atonic bladder
Imaging Findings • Progressive myelopathy (spinal cord dysfunction) or
• Location: Lumbar> cervical> thoracic spine reversible acute neurologic deficits ("stingers")
• Short thick pedicles, "trefoil-shaped" lateral recesses,
and laterally directed laminae
Diagnostic Checklist
• Patients present at a younger age than typical for
Top Differential Diagnoses degenerative spine disease
• Acquired spinal stenosis • Congenital spinal stenosis may not produce
• Inherited spinal stenosis symptoms until superimposed acquired degenerative
changes accumulate
Clinical Issues • Recognition of short thick pedicles, AP canal
• Low back pain, radiating leg pain (unilateral or diameter reduction key to diagnosis
bilateral), +/- bladder and bowel dysfunction
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CONGENITAL SPINAL STENOSIS
1 • Neurogenic claudication: Radiating leg pain with
walking, relieved by rest, bending forward
ISELECTED REFERENCES
124 • Cauda equina syndrome (rare): Bilateral leg 1. Brigham CD et al: Permanent partial cervical spinal cord
weakness, urinary retention due to atonic bladder injury in a professional football player who had only
congenital stenosis. A case report. J Bone Joint Surg Am.
o Cervical
85-A(8):1553-6, 2003
• Radiating arm pain or numbness 2. Oguz H et al: Measurement of spinal canal diameters in
• Progressive myelopathy (spinal cord dysfunction) young subjects with lumbosacral transitional vertebra. Eur
or reversible acute neurologic deficits ("stingers") Spine]. 11(2):115-8, 2002
• Clinical profile 3. Allen CR et al: Transient quadriparesis in the athlete. Clin
o Symptomatic cervical or lumbar stenosis symptoms Sports Med. 21(1):15-27, 2002
at younger age than typical of degenerative stenosis 4. Boockvar JA et al: Cervical spinal stenosis and
• These patients typically lack complicating medical sports-related cervical cord neurapraxia in children. Spine.
26(24):2709-12; discussion 2713,2001
problems (diabetes or vascular insufficiency)
5. Shaffrey CI et al: Modified open-door laminoplasty for
o Athlete presents with temporary neurological deficit treatment of neurological deficits in younger patients with
following physical contact that subsequently congenital spinal stenosis: analysis of clinical and
resolves radiographic data.J Neurosurg. 90(4 Suppl):170-7, 1999
6. Bey T et al: Spinal cord injury with a narrow spinal canal:
Demographics utilizing Torg's ratio method of analyzing cervical spine
• Age: Symptoms may arise in teens; more commonly in radiographs. J Emerg Med. 16(1):79-82, 1998
4th-5th decade 7. Yoshida M et al: Indication and clinical results of
• Gender: M ~ F laminoplasty for cervical myelopathy caused by disc
• Ethnicity: No ethnic or racial predilection herniation with developmental canal stenosis. Spine.
23(22):2391-7, 1998
Natural History & Prognosis 8. To~g JS et al: Suggested management guidelines for
• May be incidental finding in younger patients participation in collision activities with congenital,
developmental, or postinjury lesions involVing the cervical
o Borderline cases usually asymptomatic until
spine. Med Sci Sports Exerc. 29(7 Suppl):S256-72, 1997
superimposed acquired spinal degenerative disease 9. Azhar MM et al: Congenital spine deformity, congenital
occurs stenosis, diastematomyelia, and tight filum terminale in a
• Many patients eventually develop symptomatic spinal workmen's compensation patient: a case report. Spine.
stenosis 21(6):770-4, 1996
o Minor superimposed acquired abnormalities (bulge, 10. Ducker TB: Post-traumatic progressive cervical myelopathy
herniation, osteophyte) cause severe neurologic in patient with congenital spinal stenosis. J Spinal Disord.
symptoms 9(1):76; discussion 77-81, 1996
11. Torg JS: Cervical spinal stenosis with cord neurapraxia and
• Early surgical treatment important for best outcome
transient quadriplegia. Sports Med. 20(6):429-34, 1995
o Surgical decompression usually relieves symptoms 12. Lemaire JJ et al: Lumbar canal stenosis. Retrospective study
effectively of 158 operated cases. Neurochirurgie. 41(2):89-97, 1995
o Long term pain relief following surgery is common 13. Smith MG et al: The prevalence of congenital cervical
spinal stenosis in 262 college and high school football
Treatment players. J Ky Med Assoc. 91(7):273-5, 1993
• Lumbar: Decompressive laminectomy, posterior 14. Major NM et al: Central and foraminal stenosis of the
foraminotomy at involved levels lumbar spine. Neuroimaging Clinics of North America
o Risk of complications increases with more levels of 3(3):559-561, 1993
decompression, diabetes, and long term steroid use 15. Moss A G et al: Computed tomography of the body. Vol
two, Philadelphia: W.B.Saunders Company 496-500, 1992
• Cervical: Posterior cervical laminectomy or
16. Scher AT: Spinal cord concussion in rugby players. Am J
laminoplasty Sports Med. 19(5):485-8, 1991
o Same risk factors for complication as lumbar 17. Torg JS: Cervical spinal stenosis with cord neurapraxia and
decompression transient quadriplegia. Clin Sports Med. 9(2):279-96, 1990
18. Dauser RC et al: Symptomatic congenital spinal stenosis in
a child. Neurosurgery. 11(1 Pt 1):61-3, 1982
IDIAGNOSTIC CHECKLIST 19. Reale F et al: Congenital stenosis of lumbar spinal canal:
comparison of results of surgical treatment for this and
Consider other causes of lumbar syndrome. Acta Neurochir (Wien).
• Patients present at a younger age than typical for 42(3-4):199-207, 1978
20. Dharker SR et al: Congenital stenosis of the lumbar canal.
degenerative spine disease A study of 60 cases. Neurol India. 26(1):1-6, 1978
• Congenital spinal stenosis may not produce symptoms 21. Hinck VC et al: Congenital stenosis of the cervical spinal
until superimposed acqUired degenerative changes canal. Northwest Med. 67(3):268-74, 1968
accumulate
Image Interpretation Pearls
• Recognition of short thick pedicles, AP canal diameter
reduction key to diagnosis
• Look for superimposed degenerative changes
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CONGENITAL SPINAL STENOSIS
IIMAGE GALLERY 1
125
Variant
(Left) Sagittal T2WI MR
shows marked congenital
stenosis at C3 due to
abnormally short pedicles.
Cord T2 hyperintensity
(arrow) corresponds to
clinical myelopathy. (Right)
Axial bone CT demonstrates
marked AP canal reduction
attributable to
developmentally short
pedicles and laterally
oriented laminae.
Variant
(Left) Sagittal T2WI MR
reveals mild congenital AP
canal narrowing exacerbated
by C4/5 disc herniation that
produces spinal cord T2
hyperintensity (arrow)
corresponding to clinical
myelopathy. (Right) Axial
graphic depicts congenital
cervical AP spinal narrowing.
The pedicles are thick and
laterally directed with
resultant flattening of
laminae.
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SCOLIOSIS
1
126
Anteroposterior bone CT 3D reformatted image of Axial bone CT 3D reformation shows the rotation of the
idiopathic scoliosis shows rotation as well as scoliosis to apical vertebra (open arrow) compared to the terminal
be present. Arrow on apical vertebra; open arrows on vertebrae (arrow). 3D CT is valuable in surgical
terminal vertebrae. planning.
DDx: Scoliosis
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SCOLIOSIS
Key Facts
1
Terminology • Scoliosis due to congenital syndromes without 127
• General term for any lateral curvature of the spine vertebral anomalies
• Scoliosis due to infection
Imaging Findings • Scheuermann disease
• Location: Most commonly thoracic or thoracolumbar • Scoliosis due to tumor
• Standing PA radiograph of the full thoracic and • Scoliosis due to trauma
lumbar spine on single cassette • Degenerative scoliosis
• Method of Cobb is standard for measuring scoliosis • Compensatory scoliosis
• Lateral radiograph to show sagittal plane
abnormalities
Clinical Issues
• MRI to screen for bone, cord abnormalities • Idiopathic scoliosis asymptomatic
• Fusion for rapidly progressive curves, curves> 40°
Top Differential Diagnoses
Diagnostic Checklist
• Idiopathic scoliosis
• Neuromuscular scoliosis • Short-curve scoliosis usually has underlying
• Congenital scoliosis abnormalities
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SCOLIOSIS
1 Scoliosis due to trauma
o Greatest along concave aspect of scoliosis
• Severe scoliosis
128 • Usually see post-traumatic deformity o Respiratory compromise
• Stress fracture may be occult cause o Neurologic symptoms
Scoliosis due to radiation o Instability
• Usually avoided today by radiation port placement Treatment
• Radiation to entire vertebra rather than a portion • Options, risks, complications
preferred o Observation for minor curves
Degenerative scoliosis o Bracing for curves> 25°
o Fusion for rapidly progressive curves, curves> 40°
• Develops in adults
• Degenerative disc disease, facet arthropathy seen
• Can also have secondary degenerative disease from
idiopathic scoliosis IDIAGNOSTIC CHECKLIST
Neuropathic spine Consider
• Rapidly developing spine deformity • Short-curve scoliosis usually has underlying
• Bony destruction seen on radiography abnormalities
Compensatory scoliosis
• Due to limb length inequality ISELECTED REFERENCES
• Can be diagnosed on spine radiographs by position of 1. Arlet V et al: Congenital scoliosis. Eur Spine].
iliac crests 12(5):456-63, 2003
2. Daffner SD et al: Adult degenerative lumbar scoliosis. Am]
Positional scoliosis Orthop. 32(2):77-82; discussion 82,2003
• Poor positioning by radiology technologist 3. Jones KB et al: Spine deformity correction in Marfan
• Present on supine radiographs syndrome. Spine. 27(18):2003-12, 2002
• Resolves on upright radiographs 4. Ahn UM et al: The etiology of adolescent idiopathic
scoliosis. Am] Orthop. 31(7):387-95, 2002
Iatrogenic scoliosis 5. Cassar-Pullicino VN et al: Imaging in scoliosis: what, why
• Rib resection and how? Clin Radiol. 57(7):543-62, 2002
• Level above lumbar fusion 6. Berven S et al: Neuromuscular scoliosis: causes of deformity
and principles for evaluation and management. Semin
• Hardware failure
Neurol. 22(2):167-78, 2002
7. Lenke LG et al: Curve prevalence of a new classification of
operative adolescent idiopathic scoliosis: does classification
IPATHOLOGY correlate with treatment? Spine. 27(6):604-11, 2002
8. Geijer H: Radiation dose and image quality in diagnostic
General Features radiology. Optimization of the dose-image quality
• Etiology: Causes listed above relationship with clinical experience from scoliosis
• Epidemiology: Common radiography, coronary intervention and a flat-panel digital
detector. Acta Radiol Suppl. 43(427):1-43, 2002
Gross Pathologic & Surgical Features 9. Do T: Orthopedic management of the muscular
• Deformity of trunk visible on physical examination dystrophies. Curr Opin Pediatr. 14(1):50-3, 2002
10. RedIa S et al: Magnetic resonance imaging of scoliosis. Clin
Staging, Grading or Classification Criteria Radiol. 56(5):360-71, 2001
11. Thomson]D et al: Scoliosis in cerebral palsy: an overview
• Etiology
and recent results.] Pediatr Orthop B. 10(1):6-9, 2001
• Direction of curve Lowe TG et al: Etiology of idiopathic scoliosis: current
12.
• Severity of curve trends in research.] Bone Joint Surg Am. 82-A(8):1157-68,
2000
13. ]askwhich D et al: Congenital scoliosis. Curr Opin Pediatr.
ICLINICAL ISSUES 12(1):61-6, 2000
14. Mohaideen A et al: Not all rods are Harrington - an
Presentation overview of spinal instrumentation in scoliosis treatment.
• Most common signs/symptoms Pediatr Radiol. 30(2):110-8, 2000
o Visible deformity 15. Roach ]W: Adolescent idiopathic scoliosis. Orthop Clin
o Idiopathic scoliosis asymptomatic North Am. 30(3):353-65, vii-viii, 1999
16. Oestreich AE et al: Scoliosis circa 2000: radiologic imaging
o Painful scoliosis indicates underlying abnormality
perspective. II. Treatment and follow-up. Skeletal Radiol.
Demographics 27(12):651-6, 1998
17. Oestreich AE et al: Scoliosis circa 2000: radiologic imaging
• Age: Usually presents in childhood or adolescence perspective. I. Diagnosis and pretreatment evaluation.
• Gender: Idiopathic M:F = 1:7 Skeletal Radiol. 27(11):591-605, 1998
18. Kim HW et al: Spine update. The management of scoliosis
Natural History & Prognosis in neurofibromatosis. Spine. 22(23):2770-6, 1997
• Most scoliosis is mild
• May progress rapidly, especially during growth spurts
• Degenerative disc disease common
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SCOLIOSIS
IIMAGE GALLERY 1
129
Typical
(Left) Coronal bone CT
shows method of Cobb.
Angle between endplates of
terminal vertebrae is 98.5
degrees. Patient with
neuromuscular scoliosis due
to cerebral palsy. (Right)
Anteroposterior radiography
shows scoliosis due to
Scheuermann kyphosis.
Curvature is mild.
Undulation of endplates can
be seen (arrows) but is more
readily apparent on lateral
radiograph.
(Left) Posteroanterior
radiography shows apparent
levoscoliosis. However, right
iliac crest (arrow) is higher
than left, indicating scoliosis
is compensatory for limb
length inequality. (Right)
Anteroposterior radiography
shows mild scoliosis in
patient with
neurofibromatosis. Scoliosis
is related to abnormal
mesenchymal development
not tumor.
Variant
(Left) 30 CT shows complex
kyphoscoliosis in Marfan
syndrome. Open arrow is on
T1 vertebra, white arrow at
L5. Curved white arrow is at
apex of kyphosis, curved
black arrow at apex of
thoracic scoliosis. (Right) AP
30 CT shows multiple curves
in patient with Marfan
syndrome. Four separate
curves (arrows) are visible.
Secondary degenerative
changes are evident in the
lumbar spine.
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IDIOPATHIC SCOLIOSIS
1
130
Coronal graphic shows S-eurved scoliosis. Marked Posteroanterior radiography shows thoracic
rotation is evident at apices of the curves, and is dextroscoliosis and thoracolumbar levoscoliosis. Arrows
reflected in rib deformity. point to end vertebrae, open arrow to transitional
vertebra.
ITERMINOlOGY • Morphology
o Rotation as well as lateral curvature of spine always
Abbreviations and Synonyms present
• S-curve scoliosis o Most common pattern is thoracic dextroscoliosis
with compensatory lumbar levoscoliosis
Definitions o Usually see two curvatures, sometimes three
• Scoliosis of undetermined etiology, without o Thoracic levoscoliosis uncommon ~ increased
underlying bony or neuromuscular abnormalities association with underlying abnormalities
• Primary curvature: Curvature with greatest angulation
• Secondary curvature: One more compensatory
Radiographic Findings
curvatures above or below the primary curvature • Radiography
• Flexible curvature: Curvature which resolves when o Long, standing PA radiograph shows thoracic and
patient bends to contralateral side lumbar spine together
• Structural curvature: Curvature which does not change o Lift used to equalize limb lengths if needed
when patient bends to contralateral side o Wedging of vertebrae on concave aspect of curve
common, does not indicate spinal anomaly
o Curvature measured using the method of Cobb on
IIMAGING FINDINGS frontal radiograph
o Kyphosis, lordosis measured on lateral radiograph by
General Features angle between endplates
• Best diagnostic clue: Smooth, S-shaped spinal o Severity of rotation can be only very approXimately
curvature of thoracic and lumbar spine assessed
• Location o Lateral bending films distinguish between flexible
o Thoracic, lumbar most common and structural components of curve
o Occasionally involves cervical spine in addition • Primary curve usually structural
• Size • Secondary curve usually flexible, but may become
o Curvature of at least 10 degrees structural over time
o Can progress to > 90 degrees o Lateral radiograph important to show kyphosis and
lordosis
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IDIOPATHIC SCOLIOSIS
Key Facts
1
Terminology • Scoliosis due to tumor 131
• Scoliosis of undetermined etiology, without • Congenital scoliosis
underlying bony or neuromuscular abnormalities • Scoliosis due to infection
• Scoliosis due to congenital syndromes
Imaging Findings • Limb length inequality
• Best diagnostic clue: Smooth, S-shaped spinal • Degenerative scoliosis
curvature of thoracic and lumbar spine
• Rotation as well as lateral curvature of spine always
Pathology
present • Epidemiology: F:M = 7:1
• Most common pattern is thoracic dextroscoliosis with • Spondylolysis at LS common
compensatory lumbar levoscoliosis Diagnostic Checklist
• Usually see two curvatures, sometimes three • Levoscoliosis of thoracic spine has higher incidence
• Thoracic levoscoliosis uncommon ~ increased of syringomyelia, spinal cord tumors
association with underlying abnormalities • If scoliosis is painful, look for underlying cause;
Top Differential Diagnoses idiopathic scoliosiS is not painful
• Neuromuscular scoliosis
Imaging Recommendations
• Best imaging tool: Radiography
• Protocol advice: MRI should include coronal T1 WI or
PDWI to evaluate vertebral bodies
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IDIOPATHIC SCOLIOSIS
1 I PATHOLOGY Treatment
132 • Options, risks, complications
General Features o Observation used for curves < 20-25°
• Genetics: Strong familial component with o Bracing first treatment option
multifactorial inheritance o Fusion for severe or rapidly progressive scoliosis
• Etiology: unknown, probably multifactorial • Generally reserved for scoliosis of about 40° or
• Epidemiology: F:M = 7:1 greater
• Associated abnormalities • Posterior fusion with fixation rods
o Spondylolysis at L5 common • Most fixation rods currently in use are not
• Important to identify pre-operatively to plan Harrington rods; use generic term
fusion levels • Anterior fusion from lateral aspect of vertebral
Gross Pathologic & Surgical Features bodies
• Lateral curvature plus rotation o Complications of fusion include
• Concave side of scoliosis often mildly hypoplastic • Failure of fusion
• Thoracic kyphosis and lumbar lordosis often • Deformity, degenerative changes adjacent to fused
straightened or reversed levels
• Infection
Microscopic Features o Crankshaft phenomenon
• Bone normal histologically • Posterior fusion in skeletally immature patients
may act as a tether posteriorly
Staging, Grading or Classification Criteria • Growth continues anteriorly
• Infantile type: Onset before age of 4 • Results in lordosis, progressive scoliosis
o Rare; usually resolves unless severe • Change of > 10° diagnostic
• Juvenile type: Onset ages 4 to 9
o Uncommon; almost always progresses
• Adolescent type: Onset 10 years to skeletal maturity IDIAGNOSTIC CHECKLIST
o Most common type; usually diagnosed at puberty
• Since scoliosis usually will not progress after skeletal Consider
maturity, assessment of bone age important • Levoscoliosis of thoracic spine has higher incidence of
• Skeletal maturity assessed by Risser method, based on syringomyelia, spinal cord tumors
apophysis of iliac wing
o Apophysis not present = stage 0 Image Interpretation Pearls
o Apophysis covers lateral 25% iliac wing = stage 1: • If scoliosis is painful, look for underlying cause;
Bone age 13 y, 8 mo (f); 14 y, 7 mo (m) idiopathic scoliosis is not painful
o Apophysis covers lateral 50% iliac wing = stage 2:
Bone age 14 y, 6 mo (f); 15 y, 7 mo (m)
o Apophysis covers 75% iliac wing = stage 3: Bone age ISELECTED REFERENCES
15 y, 2 mo (f); 16 y, 2 mo (m) 1. Bagchi K et al: Hardware complications in scoliosis surgery.
o Apophysis covers entire iliac wing = stage 4: Bone Pediatr Radiol. 32(7):465-75, 2002
age 16 y, 2 mo (f); 17 y, 0 mo (m) 2. RedIa S et al: Magnetic resonance imaging of scoliosis. Clin
o Apophysis fused = stage 5: Bone age 18 y, 1 mo (f); Radiol. 56(5):360-71, 2001
18 y, 6 mo (m) 3. Lowe TG et al: Etiology of idiopathic scoliosis: current
trends in research. J Bone Joint Surg Am. 82-A(8):1157-68,
• Risser method less accurate than hand radiographs
2000
4. Mohaideen A et al: Not all rods are Harrington - an
overview of spinal instrumentation in scoliosis treatment.
ICLINICAL ISSUES Pediatr Radiol. 30(2):110-8, 2000
5. Roach ]W: Adolescent idiopathic scoliosis. Orthop Clin
Presentation North Am. 30(3):353-65, vii-viii, 1999
• Most common signs/symptoms 6. Oestreich AE et al: Scoliosis circa 2000: radiologic imaging
o Asymptomatic perspective. II. Treatment and follow-up. Skeletal Radiol.
o Diagnosed on physical exam with patient leaning 27(12):651-6, 1998
forward 7. Oestreich AE et al: Scoliosis circa 2000: radiologic imaging
perspective. I. Diagnosis and pretreatment evaluation.
Demographics Skeletal Radiol. 27(11):591-605, 1998
8. Lee CS et al: The crankshaft phenomenon after posterior
• Age: Onset in infancy, childhood or adolescence Harrington fusion in skeletally immature patients with
• Gender: F:M = 7:1 thoracic or thoracolumbar idiopathic scoliosis followed to
maturity. Spine. 22(1):58-67, 1997
Natural History & Prognosis 9. Cordover AM et al: Natural history of adolescent
• Curve often progresses during growth spurts thoracolumbar and lumbar idiopathic scoliosis into
• Curve usually will not progress after skeletal maturity adulthood.] Spinal Disord. 10(3):193-6, 1997
unless greater than 40-50 degrees 10. Little DG et al: The Risser sign: a critical analysis.] Pediatr
• Respiratory compromise seen with severe scoliosis Orthop. 14(5):569-75, 1994
• Degenerative disc disease, sacroiliac arthritis develop
in adults
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IDIOPATHIC SCOLIOSIS
IIMAGE GALLERY 1
133
(Left) Anteroposterior
radiography shows failure of
posterior fusion for scoliosis.
Left image shows
appearance immediately
postoperatively, right shows
subsequent displacement of
multiple laminar hooks.
(Right) Coronal bone CT
shows fracture (arrow) of
unilateral posterior fixation
rod. Failed bony fusion is
readily apparent as a cleft
(open arrows) in the
otherwise solid fusion mass.
Variant
(Left) Anteroposterior
radiography shows a 40 year
old female with untreated
idiopathic scoliosis and
superimposed degenerative
lumbar scoliosis. (Right)
Posteroanterior radiography
shows thoracolumbar
levoscoliosis and lumbar
dextroscoliosis. Left lateral
listhesis at L7 -L2 (arrows) is
due to superimposed
degenerative arthritis.
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Key Facts
1
Terminology • Scoliosis due to syndromes 135
• Spinal curvature secondary to vertebral anomalies • Scoliosis due to trauma
• Scoliosis due to radiation
Imaging Findings • Limb length inequality
• Location: Most common in thoracic spine, but can • Idiopathic kyphosis
occur at any level • Scheuermann kyphosis
• Usually short curve scoliosis • Kyphosis due to tuberculosis
• May have multiple curves if multiple anomalies • Juvenile chronic arthritis
present
• Screening with full spine PA and lateral radiographs
Pathology
• Vertebral anomalies often difficult to see; use • Abnormalities due either to failure of development
coned-down radiographs, MR or CT to clarify and/or failure of segmentation
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Typical
(Left) Sagittal T2WI MR
depicts a coronal L4
vertebral body cleft (arrow)
in young child. There is as
yet no growth deformity and
the child may be monitored
clinically. (Right) Coronal
T2WI MR shows butterfly
thoracic vertebra in young
child with multiple
segmentation anomalies. No
definite disc is seen below
this vertebra, reflecting
incomplete segmentation.
Typical
(Left) Anteroposterior
radiography shows failure of
segmentation of multiple
thoracic vertebrae. Right
sided hemivertebra (arrow)
is present. There may be
fusion of left-sided ribs.
(Right) Anteroposterior
radiography shows butterfly
vertebra (arrow). Right side
is fused to vertebra below,
while left side has disc space
above and below, indicating
high risk for childhood
progression.
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NEUROMUSCULAR SCOLIOSIS
1
138
Anteroposterior radiography shows a mild, C-curve Anteroposterior radiography shows severe C-curve
dextroscoliosis extending from upper thoracic to levoscoliosis in patient with cerebral palsy and
midlumbar region in a cerebral palsy patient. paraplegia. Pelvic tilt reflects patient's sitting position.
Note decreased lung volumes.
Definitions
• Scoliosis due to neurologic or myopathic diseases IDIFFERENTIAL DIAGNOSIS
Idiopathic scoliosis
IIMAGING FINDINGS • S-shaped curve
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NEUROMUSCULAR SCOLIOSIS
Key Facts
1
Terminology • Congenital scoliosis 139
• Scoliosis due to neurologic or myopathic diseases • Scoliosis related to syndromes
IPATHOLOGY Treatment
• Options, risks, complications
General Features o Bracing for minor curves; usually does not halt
• General path comments: Secondary to neurogeniC progreSSion
causes or myopathy o Surgical fusion has higher complication rate than
• Etiology other scolioses
o Neurogenic causes
• Cerebral palsy
• Spinal cord tumor IDIAGNOSTIC CHECKLIST
• Syringomyelia
• Traumatic paralysis Consider
• Poliomyelitis • Screening full spine MRl to exclude spinal cord or
• Myelomeningocele bony abnormalities
• Charcot-Marie-Tooth disease
o Myopathic causes
• Duchenne muscular dystrophy ISELECTED REFERENCES
• Spinal muscular atrophy 1. Do T: Orthopedic management of the muscular
• Friedreich ataxia dystrophies. Curr Opin Pediatr. 14(1):50-3,2002
• Arthrogryposis multiplex congenita 2. Berven S et al: Neuromuscular scoliosis: causes of deformity
• Epidemiology: 20% of cerebral palsy patients and principles for evaluation and management. Semin
Neurol. 22(2):167-78, 2002
• Associated abnormalities Thomson JD et al: Scoliosis in cerebral palsy: an overview
3.
o Syringomyelia, spinal dysraphism, tethered cord and recent results. J Pediatr Orthop B. 10(1):6-9, 2001
o Kyphosis or lordosis 4. Redia S et al: Magnetic resonance imaging of scoliosis. Clin
Radiol. 56(5):360-71, 2001
Gross Pathologic & Surgical Features
• Rigid curve
Staging, Grading or Classification Criteria IIMAGE GALLERY
• Minor curve: < 20 degrees
• Significant curve: > 20 degrees
ICLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Rapidly progressive curvature
o Other signs/symptoms
• Respiratory compromise
Demographics
• Age: Onset in infancy or childhood; may progress in (Left) Lateral radiography shows loss of thoracic kyphosis and lumbar
adulthood lordosis in cerebral palsy patient. Vertebral body rotation is reflected
• Gender: M = F in posterior displacement of ribs on one side (arrow). (Right)
Anteroposterior radiography shows long segment fixation of scoliosis.
in cerebral palsy patient. Failure of fusion is more common than with
idiopathic scoliosis, & close imaging follow-up is prudent.
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IDIOPATHIC KYPHOSIS
1
140
Lateral radiography shows marked kyphosis of the Lateral radiography shows patient with 35 degrees of
upper thoracic spine in 30 year old patient. Premature kyphosis, near the upper limits of normal. Note that
degenerative disc disease is present, with minimal normal kyphosis is greatest in the upper thoracic spine.
wedging of vertebral bodies.
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IDIOPATHIC KYPHOSIS
Key Facts
1
Terminology Top Differential Diagnoses 141
• Synonyms: Postural kyphosis, round back deformity • Scheuermann kyphosis
• Congenital kyphosis
Imaging Findings • Post-traumatic kyphosis
• Best diagnostic clue: Thoracic kyphosis greater than
approximately 40 degrees Pathology
• Location: Upper to mid-thoracic spine • Curve usually flexible
• Smooth, nonangular curvature • Curve may become structural if left unaddressed
ICLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Asymptomatic
o Stooped shoulders
Demographics (Left) Lateral radiography shows idiopathic kyphosis compounded in
• Age: Adolescents an older patient by degenerative disc disease. (Right) Lateral
• Gender: More common in females radiography shows physiologic wedging (arrows), a normal
phenomenon, at Tl2 and L1. Overall thoracic curvature was normal.
Natural History & Prognosis
• Premature development of thoracic degenerative disc
disease
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SCHMORL NODE
1
142
Sagittal T7WI MR shows focal undersurface end plate Sagittal T7 C+ MR with fat-saturation shows diffuse
defect filled by disc material with low signal intensity of marrow enhancement surrounding disc extending
adjacent marrow suggesting recent event in patient with through inferior end plate defect in patient with focal
focal back pain. pain.
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SCHMORL NODE
Key Facts
1
Terminology • Limbus vertebrae 143
• Intravertebral disc herniation • Bone island
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SCHMORL NODE
1 o Most cases found incidentally as chronic, "burned
out" lesions
symptomatic and asymptomatic individuals. Eur Spine J.
4(1):56-9, 1995
14. Takahashi K et al: A large painful Schmorl's node: a case
144 • Clinical profile
report. J Spinal Disord. 7(1):77-81, 1994
o Teenager involved in axial-loading sports IS. Jensen MC et al: Magnetic resonance imaging of the
o Acute onset of localizing pain lumbar spine in people without back pain. N Engl J Med.
331(2):69-73, 1994
Demographics 16. Hamanishi C et al: Schmorl's nodes on magnetic resonance
• Age: Adolescents and young adults imaging. Their incidence and clinical relevance. Spine.
• Gender: M > F, up to 9:1 ratio 19(4):450-3, 1994
17. Magnetic resonance imaging of the lumbar spine in people
Natural History & Prognosis without back pain: N EnglJ Med. JuI14i331(2):69-73, 1994
• Self-limited 18. Sward L: The thoracolumbar spine in young elite athletes.
• Good, unless systemic osteoporosis leads to recurrent Current concepts on the effects of physical training. Sports
compression fractures Med. 13(5):357-64, 1992
19. Walters G et al: Magnetic resonance imaging of acute
Treatment symptomatic Schmorl's node formation. Pediatr Emerg
• Observational, with pain management in symptomatic Care. 7(5):294-6, 1991
cases 20. Sward L et al: Back pain and radiologic changes in the
thoraco-lumbar spine of athletes. Spine. 15(2):124-9, 1990
21. Mclain R et al: An unusual presentation of a Schmorl's
node. Report of a case. Spine. 15(3):247-50, 1990
IDIAGNOSTIC CHECKLIST 22. Roberts S et al: Biochemical and structural properties of the
cartilage end-plate and its relation to the intervertebral
Consider disc. Spine. 14(2):166-74, 1989
• Follow-up MRI in cases of unexplained VB edema and 23. McFadden KD et al: End-plate lesions of the lumbar spine.
localized pain Spine. 14(8):867·9, 1989
24. Yasuma T et al: Schmorl's nodes. Correlation of X-ray and
Image Interpretation Pearls histological findings in postmortem specimens. Acta
• Always contiguous with parent disc Patho!]pn. 38(6):723-33, 1988
25. Kagen S et al: Focal uptake on bone imaging in an
asymptomatic Schmorl's node. Clin Nucl Med. 13(8):615-6,
ISELECTED REFERENCES 26.
1988
Kornberg M: MRI diagnosis of traumatic Schmorl's node. A
1. Yamaguchi T et al: Schmorl's node developing in the case report. Spine. 13(8):934-5, 1988
lumbar vertebra affected with metastatic carcinoma: 27. Malmivaara A et al: Plain radiographic, discographic, and
correlation magnetic resonance imaging with histological direct observations of Schmorl's nodes in the
findings. Spine. 28(24):E503-5, 2003 thoracolumbar junctional region of the cadaveric spine.
2. Peng B et al: The pathogenesis of Schmorl's nodes. J Bone Spine. 12(5):453-7, 1987
Joint Surg Br. 85(6):879-82, 2003 28. Saluja G et al: Schmorl's nodes (intravertebral herniations
3. Hauger 0 et al: Giant cystic Schmorl's nodes: imaging of intervertebral disc tissue) in two historic British
findings in six patients. AJR Am J Roentgenol. populations. J Anat. 145:87-96, 1986
176(4):969-72,2001 29. Lipson SJ et al: Symptomatic intravertebral disc herniation
4. Wagner AL et al: Relationship of Schmorl's nodes to (Schmorl's node) in the cervical spine. Ann Rheum Dis.
vertebral body endplate fractures and acute endplate disk 44(12):857-9, 1985
extrusions. AJNR Am J Neuroradiol. 21(2):276-81, 2000 30. Resnick D et al: Intravertebral disk herniations:
5. Wagner AL et al: Relationship of Schmorl's nodes to cartilaginous (Schmorl's) nodes. Radiology. 126(1):57-65,
vertebral body endplate fractures and acute endplate disc 1978
extrusions. AJNR 21: 276-81, 2000 31. Deeg H]: Schmorl's nodule. N Eng!] Med. 298(1):57, 1978
6. Grive E et al: Radiologic findings in two cases of acute 32. Resnick D et al: Intervertebral disc abnormalities associated
Schmorl's nodes. AJNR AmJ Neuroradiol. 20(9):1717-21, with vertebral metastasis: observations in patients and
1999 cadavers with prostatic cancer. Invest Radiol. 13(3):182-90,
7. Silberstein M et al: Spinal Schmorl's nodes: sagittal 1978
sectional imaging and pathological examination. Australas 33. Resnick D et al: Intravertebral disc herniations:
Radiol. 43(1):27-30, 1999 Cartilaginous (Schmorl's) nodes. Radiology 126: 57-65,
8. Swischuk LE et al: Disk degenerative disease in childhood: 1978
Scheuermann's disease, Schmorl's nodes, and the limbus 34. Hilton RC et al: Vertebral end-plate lesions (Schmorl's
vertebra: MRI findings in 12 patients. Pediatr Radiol. nodes) in the dorsolumbar spine. Ann Rheum Dis.
28(5):334-8, 1998 35(2):127-32, 1976
9. Seymour R et al: Magnetic resonance imaging of acute 35. Smith DM: Acute back pain associated with a calcified
intraosseous disc herniation. Clin Radiol. 53(5):363-8, 1998 Schmorl's node: a case report. Clin Orthop. (117):193-6,
10. Tribus CB: Scheuermann's kyphosis in adolescents and 1976
adults: diagnosis and management. J Am Acad Orthop 36. Boukhris R et al: Schmorl's nodes and osteoporosis. Ciin
Surg. 6(1):36-43, 1998 Orthop. 0(104):275-80, 1974
11. Fahey V et al: The pathogenesis of Schmorl's nodes in
relation to acute trauma. An autopsy study. Spine.
23(21):2272-5, 1998
12. Stabler A et al: MR imaging of enhancing intraosseous disk
herniation (Schmorl's nodes). AJR Am J Roentgenol.
168(4):933-8, 1997
13. Takahashi K et al: Schmorl's nodes and low-back pain.
Analysis of magnetic resonance imaging findings in
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SCHMORL NODE
IIMAGE GALLERY 1
145
Typical
(Left) Sagittal TI C+ MR with
fat-saturation shows diffuse
enhancement of marrow
around focal disc incursion
into superior end plate of L4
in patient with recent axial
load trauma, non-Iateralizing
pain. (Right) Sagittal TlWI
MR shows small, blister-like
incursion into superior end
plate. Note low signal border
suggestive of condensed
bone. No history of recent
trauma, non-focal pain.
Typical
(Left) Axial NEeT following
myelogram shows sclerotic
rim of bone surrounding low
density material within the
vertebral body just above
interspace. (Right) Sagittal
TI WI MR shows small focal
node of disc encroaching
into lower thoracic vertebral
body's superior endplate.
Note minimal "mirror"
change in inferior end plate
of suprajacent vertebral
body.
Variant
(Left) Sagittal TlWI MR (L)
and TI C+ (R) (fat-saturated)
sections depict focal disc
incursion into upper
endplate of L2; fat signal
(ossified disc) fades with
fat-saturation (as do L5-57
type /I changes). (Right)
Sagittal NECT shows
sclerotic margins bordering a
small, focal disc incursion
into inferior end plate of a
low- thoracic vertebral body.
Note calcified node in the
vertebral body immediately
below.
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SCHEUERMANN DISEASE
1
146
Sagittal graphic shows herniation of disc material Sagittal bone CT shows multiple Schmorl nodes,
through vertebral endplates creating focal subcortical irregular vertebral endplates, and anterior wedging of
bone defects. Undulation of endplates reflects bony multiple thoracic vertebral bodies typical of
reparative process. Scheurmann disease.
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SCHEUERMANN DISEASE
Key Facts
1
Terminology Pathology 147
• Kyphosis secondary to multiple Schmorl nodes ~ • Chronic repetitive trauma in a skeletally immature
vertebral body wedging person
• Disc extrusions through weakened regions of
Imaging Findings vertebral end plates
• Best diagnostic clue: Three or more wedged thoracic • Prevalence: 0.4-8%
vertebrae with irregular endplates • Peak incidence: 13-17 years
• 75% thoracic
• 15% have scoliosis as well as kyphosis Clinical Issues
• Thoracic spine pain and tenderness worsened by
Top Differential Diagnoses activity
• Postural kyphosis
• Wedge compression fractures Diagnostic Checklist
• Congenital kyphosis • Schmorl nodes without anterior wedging are not
• Osteogenesis imperfecta tarda indicative of Scheuermann disease
• Ankylosing spondylitis (AS) • Radiography may show undulating endplates rather
• Spondyloepiphyseal dysplasia tarda (SED) than discrete Schmorl nodes
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SCHEUERMANN DISEASE
1 o Abnormal chondrocytes
o Loose cartilage matrix
I DIAGNOSTIC CHECKLIST
148 o Diminished number or thickness of collagen fibers Image Interpretation Pearls
o Increased proteoglycan content • Schmorl nodes without anterior wedging are not
• Osteonecrosis, osteochondrosis not seen indicative of Scheuermann disease
• Radiography may show undulating endplates rather
than discrete Schmorl nodes
ICLINICAL ISSUES
Presentation
• Most common signs/symptoms
ISELECTED REFERENCES
o Kyphosis 1. Soo CL et al: Scheuermann kyphosis: long-term follow-up.
o Other signs/symptoms Spine]. 2(1):49-56, 2002
2. Stotts AK et al: Measurement of spinal kyphosis:
• Thoracic spine pain and tenderness worsened by
implications for the management of Scheuermann's
activity kyphosis. Spine. 27(19):2143-6, 2002
• Neurologic symptoms from kyphosis or from disc 3. Wenger DR et al: Scheuermann kyphosis. Spine.
herniations 24(24):2630-9, 1999
• Fatigue 4. Swischuk LE et al: Disk degenerative disease in childhood:
Scheuermann's disease, Schmorl's nodes, and the limbus
Demographics vertebra: MRI findings in 12 patients. Pediatr Radiol.
• Age: Develops in adolescence, may present later in life 28(5):334-8, 1998
• Gender: Slight male predominance 5. Tribus CB: Scheuermann's kyphosis in adolescents and
adults: diagnosis and management. J Am Acad Orthop
Natural History & Prognosis Surg. 6(1):36-43, 1998
• Increases in magnitude during adolescent growth spurt 6. Platero D et al: Juvenile kyphosis: effects of different
• Mild progression after growth is complete variables on conservative treatment outcome. Acta Orthop
Belg. 63(3):194-201, 1997
• Severe deformity uncommon
7. Chiu KY et al: Cord compression caused by multiple disc
• Kyphosis greater than 70 degrees has poor functional herniations and intraspinal cyst in Scheuermann's disease.
result Spine. 20(9):1075-9, 1995
• Premature disc degeneration 8. Murray PM et al: The natural history and long-term
• Disc herniations related to degeneration, mechanical follOW-Up of Scheuermann kyphosis. J Bone Joint Surg Am.
stress from spine deformity 75(2):236-48, 1993
9. Mandell GA et al: Bone scintigraphy in patients with
Treatment atypical lumbar Scheuermann disease. J Pediatr Orthop.
• Observation 13(5):622-7, 1993
o Indications 10. Tala VT et al: Postural kyphosis and Scheuermann's disease.
Seminars in Spine Surgery 4:216-24, 1992
• Growth still remains
11. Farsetti Pet al: Juvenile and idiopathic kyphosis.
• Kyphotic deformity less than 50 degrees Long-term follow-up of 20 cases. Arch Orthop Trauma
o Elimination of the specific strenuous activity Surg. 110(3):165-8, 1991
o Analgesics 12. Findlay A et al: Dominant inheritance of Scheuermann's
o Spine exercises juvenile kyphosis. J Med Genet. 26(6):400-3, 1989
o Follow-up until growth-plate fuses, age 25 13. Gilsanz V et al: Vertebral bone density in Scheuermann
• Brace treatment disease. J Bone Joint Surg Am. 71(6):894-7, 1989
o Indications 14. Paajanen H et al: Disc degeneration in Scheuermann
• At least one year of growth remains disease. Skeletal Radiol. 18(7):523-6, 1989
15. Blumenthal SL et al: Lumbar Scheuermann's. a clinical
• < 70 degree kyphosis series and classification. Spine 12:929-32, 1987
• At least partial correction of kyphosis on 16. Ippolito E et al: Juvenile kyphosis; histological and
hyperextension histochemical studies. J Bone Joint Surg 63: 175-82, 1981
• Surgical treatment 17. Fon GT et al: Thoracic kyphosis: range in normal subjects.
o Uncommonly felt to be necessary AJR AmJ Roentgenol. 134(5):979-83, 1980
o Indications
• > 75 degree kyphosis in a skeletally immature
person
• > 60 degree kyphosis in mature person
• Excessive pain
• Neurologic deficit
o Posterior instrumentation and fusion
o Anterior and posterior fusion for more severe
kyphosis
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SCHEUERMANN DISEASE
IIMAGE GALLERY 1
149
Variant
(Left) Lateral radiography
reveals focal kyphosis
secondary to isolated
thoracolumbar Scheuermann
disease. Scalloped contour of
endplates distinguishes from
multiple compression
fractures. (Right) Sagittal
bone scan SPECT images
show increased radionuclide
uptake throughout lumbar
spine in adolescent patient
with lumbar Scheuermann
disease.
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THANATOPHORIC DWARFISM
1
150
Anteroposterior radiography of a term infant shows Lateral radiography demonstrates short ribs and
short ribs, bell-shaped thorax, and platyspondyly. Lungs hypoplastic sternum. Vertebral morphology is often
are hypoinflated. difficult to seen on CXR because of obscuration by
pulmonary opacities.
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THANATOPHORIC DWARFISM
Key Facts
1
Terminology Top Differential Diagnoses 151
• Lethal short-limbed dysplasia • Achondroplasia
• Osteogenesis imperfecta congenita
Imaging Findings
• Best diagnostic clue: Severe dwarfism with narrow Clinical Issues
chest and short ribs • Most common signs/symptoms: Respiratory distress
• Morphology: Severe flattening of vertebral bodies in neonate with dwarfism
• Best imaging tool: Antenatal ultrasound • Usually fatal shortly after birth; may survive several
years
ICLINICAL ISSUES
Presentation
• Most common signs/symptoms: Respiratory distress in
neonate with dwarfism
Demographics
• Age: Congenital
• Gender: M = F (Left) Lateral radiography depicts the cloverleaf skull abnormality
which is often associated with thanatophoric dysplasia, as well as
Natural History & Prognosis other congenital syndromes. (Right) Lateral radiography shows
• Usually fatal shortly after birth; may survive several platyspondyly'and wide intervertebral disc spaces. No other vertebral
years anomalies are present. Mineralization appears normal.
Treatment
• Options, risks, complications
o Ventriculoperitoneal shunt
o Tracheostomy
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ACHONDROPLASIA
1
152
DDx: Achondroplasia
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ACHONDROPLASIA
Key Facts
1
Terminology Top Differential Diagnoses 153
• Autosomal dominant dwarfism affecting spine and • Pseudoachondroplasia
extremities • Hypochondroplasia
• Rhizomelic dwarfism; most severe growth disturbance • Diastrophic dysplasia
in proximal portions of limbs • Spondyloepiphyseal dysplasia (SED) congenita
• Thanatophoric dysplasia
Imaging Findings • Osteogenesis imperfecta (01)
• Lumbar interpedicular distance progressively
decreases in caudad direction Pathology
• Vertebral bodies slightly flattened with short pedicles • Autosomal dominant
• +/- Bullet-shaped vertebral bodies • Epidemiology: Most common nonlethal skeletal
• Thoracolumbar kyphosis dysplasia (1:26,000 live births)
• Bony spinal stenosis due to degenerative disease
superimposed on short pedicles Diagnostic Checklist
• Best imaging tool: Single AP "babygram" at birth • Most likely cause of short limbed dwarfism with
shows skull, spine, pelvic abnormalities normal ossification detected in 3rd trimester
o Growth disturbance evident in third trimester • Similar to but milder than achondroplasia
ultrasound
o Ossification normal Diastrophic dysplasia
o Homozygous form can be diagnosed in second • Facial features marked by micrognathia
trimester • Flattened vertebral bodies; narrowed interpediculate
distances
CT Findings • Scoliosis and kyphosis
• NECT: Communicating hydrocephalus (uncommon) • Cl-2 instability
• Bone CT • Subluxations in extremities ("hitchhiker's thumb")
o Mildly flattened vertebral bodies • Clubfoot
o Bullet shaped vertebral bodies
o Posterior vertebral body scalloping Spondyloepiphyseal dysplasia (SED)
o Bony spinal stenosis due to degenerative disease congenita
superimposed on short pedicles • Facial features and skull normal
o Deformity may lead to stress fractures • Severe vertebral body flattening
o Small foramen magnum • Cl-2 instability and odontoid hypoplasia
• Delayed ossification of epiphyses
MR Findings • Flattened epiphyses
• Morphologic changes as described for CT • Shortened long bones
• Disc herniations common • Absent pubic ossification in infants
• Compression of cervicomedullary junction, spinal
cord, nerve roots ~ myelomalacia Thanatophoric dysplasia
• Lethal dwarfism
Imaging Recommendations • Severe platyspondyly
• Best imaging tool: Single AP "babygram" at birth shows • Long, narrow trunk
skull, spine, .pelvic abnormalities • Bell-shaped thorax
• Protocol advice • May have "cloverleaf" skull
o 2-3 mm multidetector CT
o Coronal and sagittal reformations for surgical Osteogenesis imperfecta (01)
planning • Infantile form has short, thick bones, multiple
o Axial, sagittal MR through foramen magnum in all . fractures
infants and children to assess for stenosis • Milder forms present in childhood or adulthood with
short stature, platyspondyly, osteopenia, fractures,
bone deformities
IDIFFERENTIAL DIAGNOSIS
Pseudoachondroplasia IPATHOLOGY
• Facial features and skull normal
• Usually detected in early childhood rather than at General Features
birth • Genetics
• Vertebral body flattening variably seen o Autosomal dominant
o Gene mapped to chromosome 4p16.3
Hypochondroplasia o Same allele as hypochondroplasia, thanatophoric
• Midface hypoplasia dwarfism
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ACHONDROPLASIA
1 o Homozygous form rare, much more severe than
heterozygous
o Kyphosis may be preventable by not allowing
hypotonic infant to sit unassisted
154 • Etiology o Surgical decompression of foramen magnum in
o Usually a spontaneous mutation severe cases; usually symptoms resolve
o Fibroblast growth factor receptor abnormality o Surgical decompression for spinal stenosis
• Epidemiology: Most common nonlethal skeletal o Bracing, fusion for thoracolumbar kyphosis
dysplasia (1:26,000 live births) • Extraspinal: Ilizarov limb lengthening procedure
• Associated abnormalities controversial
o Sleep apnea
o Sudden infant death
o Chest wall deformity may lead to respiratory IDIAGNOSTIC CHECKLIST
difficulties
o Limb lengthening procedures can lead to neurologic Consider
symptoms • Most likely cause of short limbed dwarfism with
normal ossification detected in 3rd trimester
Gross Pathologic & Surgical Features
• Spinal stenosis Image Interpretation Pearls
o Premature fusion of neural arch synchondroses • Stress fracture or disc herniation may present acutely
o Short pedicles, thickened laminae and can be evaluated with CT or MR
o Disc bulges and herniations contribute to stenosis • Spine imaging must include foramen magnum to
o Can occur at all spinal levels evaluate for stenosis
o Significant cause of morbidity in adults
• Foramen magnum stenosis
o Found in almost all children with achondroplasia ISELECTED REFERENCES
o Often resolves functionally as child grows 1. Tanaka N et al: The comparison of the effects of short-term
• Thoracolumbar kyphosis growth hormone treatment in patients with
o Related to hypotonia in infants achondroplasia and with hypochondroplasia. Endocr ].
o Often resolves but may develop fixed deformity 50(1):69-75,2003
o Seen in 30% of adults 2. Park HW et al: Correction of lumbosacral hyperlordosis in
achondroplasia. Clin Orthop. (414):242-9, 2003
Microscopic Features 3. Thomeer RT et al: Surgical treatment of lumbar stenosis in
• Disruption of growth plate enchondral ossification achondroplasia.] Neurosurg. 96(3 Suppl):292-7, 2002
4. Gordon N: The neurological complications of
achondroplasia. Brain Dev. 22(1):3-7, 2000
5. Lemyre E et al: Bone dysplasia series. Achondroplasia,
ICLINICAL ISSUES hypochondroplasia and thanatophoric dysplasia: review
and update. Can Assoc Radiol]. 50(3):185-97, 1999
Presentation 6. Keiper GL]r et al: Achondroplasia and cervicomedullary
• Most common signs/symptoms compression: prospective evaluation and surgical
o Dwarfism, characteristic facies evident at birth treatment. Pediatr Neurosurg. 31(2):78-83, 1999
o Other signs/symptoms 7. Lachman RS: Neurologic abnormalities in the skeletal
• Infants hypotonic dysplasias: a clinical and radiological perspective. Am]
• Sleep apnea in infants and children due to Med Genet. 69(1):33-43, 1997
8. Taybi H, Lachman RS: Radiology of syndromes, metabolic
compression of cord at foramen magnum disorders, and skeletal dysplasias. 4th ed. St. Louis, Mosby.
• Thoracolumbar kyphosis 748-55, 1996
• Spinal stenosis 9. Hall ]G: Information update on Achondroplasia. Pediatrics.
• Clinical profile 95(4):620, 1995
o Rhizomelic dwarfism apparent at birth 10. Ryken TC et al: Cervicomedullary compression in
o Frontal bossing and depressed nasal bridge achondroplasia.] Neurosurg. 81(1):43-8, 1994
characteristic 11. Hecht]T et al: Neurologic morbidity associated with
o Intelligence normal achondroplasia.] Child Neurol. 5(2):84-97, 1990
12. Kao SC et al: MR imaging of the craniovertebral junction,
o Obesity a common clinical problem cranium, and brain in children with achondroplasia. A]R
o Probably overdiagnosed clinically Am] Roentgenol. 153(3):565-9, 1989
13. Fortuna A et al: Narrowing of thoraco-Iumbar spinal canal
Demographics in achondroplasia.] Neurosurg Sci. 33(2):185-96, 1989
• Age: Congenital; diagnosis usually made in infancy 14. Thomas IT et al: Magnetic resonance imaging in the
• Gender: M = F assessment of medullary compression in achondroplasia.
Am] Dis Child. 142(9):989-92, 1988
Natural History & Prognosis 15. Beighton P et al: Gibbal achondroplasia.] Bone Joint Surg
• High morbidity from spinal stenosis Br. 63-B(3):328-9, 1981
Treatment
• Options, risks, complications
o Growth hormone can promote growth of long
bones
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ACHONDROPLASIA
IIMAGE GALLERY 1
155
Typical
(Left) Lateral radiography
demonstrates mild vertebral
body flattening and posterior
scalloping with short
pedicles in an adult patient.
(Right) Lateral radiography
shows platyspondyly in a
neonate. Endplates are
notched, an abnormality
which resolves in childhood.
(Left) Anteroposterior
radiography shows
characteristic findings at
birth. Vertebral bodies are
flat, interpediculate distance
narrows caudally, and
deformity of pelvis and long
bones is evident. (Right)
Sagittal T2WI MR
demonstrates foramen
magnum stenosis in a
neonate with compression of
the cervicomedullary
junction.
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MUCOPOLYSACCHARIDOSES
1
156
Sagittal T2WI MR (Hurler-Scheie, MPS I-HIS) shows Axial T2WI MR (Hurler-Scheie, MPS I-HIS) depicts
marked hypointense ligamentous thickening (arrows) classic marked hypointense ligamentous thickening
and craniocervical junction stenosis. (arrows).
DDx: Mucopolysaccharidosis
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MUCOPOLYSACCHARIDOSES
Key Facts
1
Terminology • Achondroplasia 157
• Synonyms: MPS, lysosomal storage disorder, • Down syndrome
"gargoylism" • Spondyloepiphyseal dysplasia
• Inherited lysosomal storage disorders Pathology
• Specific enzyme deficiency => inability to breakdown • Odontoid hypoplasia, GAG deposition, +/-
specific glycosaminoglycans (GAG) ligamentous laxity with reactive change produce soft
Imaging Findings tissue mass around dens
• Best diagnostic clue: Dens hypoplasia, craniovertebral Clinical Issues
junction (CVJ) stenosis, and thickened dural ring at • Premature death the rule; rate of deterioration
foramen magnum depends on specific enzymatic deficiency
• Platyspondyly, anterior beaking + thoracolumbar
gibbus deformity (MPS I-H and IV) Diagnostic Checklist
• Dens hypoplasia with mass and dural thickening
Top Differential Diagnoses suggests MPS syndrome
• GMI gangliosidosis
• Mucolipidosis III (pseudo-Hurler polydystrophy)
• T2WI
o Hypoplastic dens and peri-odontoid soft tissue mass Down syndrome
o Hypointense, thickened dura +/- cyst formation due • +/- Dens hypoplasia without soft tissue dens mass or
to meningeal thickening (MPS I-H, II) marrow deposition features
o +/- Cord compression with hyperintense signal • Distinguish using genetic and clinical information
abnormality Spondyloepiphyseal dysplasia
• Tl C+: No abnormal enhancement, even if mass • Autosomal dominant; presents at birth
present
• Flattening of vertebral bodies, dens hypoplasia,
• MRS: Brain proton MRS may show diminished
scoliosis
NAA/choline ratio, elevated glutamine/glutamate and
• Minimal hand and foot involvement
inositol peak areas
Imaging Recommendations
• Best imaging tool: MR imaging I PATHOLOGY
• Protocol advice General Features
o Spine MR imaging to elucidate cause/site of cord
compression • General path comments
o GAG accumulates in most organs and ligaments
o Plain radiographs to characterize osseous spine and
limb abnormalities • Coarse facies (hence the name "gargoylism")
o Flexion-extension radiographs or fluoroscopy to
• Hepatosplenomegaly, umbilical hernia,
detect craniovertebral instability • Skeletal dysotosis multiplex, joint contractures
• Arterial wall (mid-aortic stenosis), cardiac valve
thickening
IDIFFERENTIAL DIAGNOSIS • Upper airway obstruction (38%); very difficult
intubations
GM1 gangliosidosis o Odontoid hypoplasia, GAG deposition, +/-
• Shares features of vertebral beaking, upper lumbar ligamentous laxity with reactive change produce
gibbus, and dens hypoplasia soft tissue mass around dens
• Distinguish on clinical, genetic criteria o Biconvex ovoid, bullet shaped or rectangular
vertebral bodies, vertebral beaking, posterior
Mucolipidosis III (pseudo-Hurler vertebral slip (MPS IV), large disc protrusions (MPS
polydystrophy) VI)
• Shares features of vertebral beaking, upper lumbar • Genetics: Autosomal recessive (except MPS II Hunter's;
gibbus, and dens hypoplasia X-linked recessive)
• Distinguish on clinical, genetic criteria • Etiology: Inherited lysosomal enzyme deficiency
• Epidemiology: MPS I-H (1:10,000 births), MPS IV
Achondroplasia (1:40,000 births) most common
• Autosomal dominant disorder of enchondral bone
formation Gross Pathologic & Surgical Features
• Short broad pedicles and thickened laminae => spinal • CV] stenosis => neurovascular compression, altered
stenosis CSF dynamics => hydrocephalus, hydrosyringomyelia
• Distinguish on genetic, clinical criteria • Dilated enlarged laminae medullary cavities
• Thickened dura may appear normal at surgery
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MUCOPOLYSACCHARIDOSES
1 Microscopic Features
o Surgical (more severe symptoms)
• Posterior occipitocervical
158 • Epidural/dural mucopolysaccharide deposition with decompression/stabilization
elastic and collagenous proliferation • Transoral odontoid resection/posterior
Staging, Grading or Classification Criteria stabilization for symptomatic dens mass
o Bone marrow transplant (BMT) or intravenous
• Hurler (MPS I-H), Hurler-Scheie (MPS I-H/S):
(){-L-iduronidase (4pI6.3) recombinant human enzyme
• Decreases GAG accumulation in organs;
• Hunter (MPS II): Iduronate 2-sulfatase (Xq28)
ameliorates some but not all manifestations
• Sanfillipo (MPS III): Heparin N-sulfatase (17q2S.3)
• Morquio (MPS IV): Galactose 6-sulfatase (16q24.3)
• Scheie (MPS V)
• Maroteaux-Lamy (MPS VI): Arylsulfatase B (Sqll-q13) IDIAGNOSTIC CHECKLIST
• Sly (MPS VII) Consider
• Successful diagnosis requires combination of clinical,
imaging, and genetic/biochemical information
ICLINICAL ISSUES
Image Interpretation Pearls
Presentation • Dens hypoplasia with mass and dural thickening
• Most common signs/symptoms suggests MPS syndrome
o Gradual subtle progressive myelopathy (often falsely • Vertebral beaking pattern may permit specific
attributed to lower extremity deformities) diagnosis
• Common with MPS I-H, II, III, and VII;
uncommon with MPS IV, I H/S, VI unless
associated with musculoskeletal deformities
• Reduced exercise tolerance; may be earliest
ISELECTED REFERENCES
symptom 1. Chirossel]P et al: Management of craniocervical junction
dislocation. Childs Nerv Syst. 16(10-11):697-701,2000
o Clinical neurologic symptoms attributable to brain
2. Barkovich A: Pediatric neuroimaging. 3rd ed. Philadelphia,
GAG deposition, myelination abnormalities, spinal Lippincott-Raven, 121-22,2000
deformities, and peripheral nerve entrapment 3. Kim FM et al. Neuroimaging of scoliosis in childhood.
• Clinical profile Neuroimaging Clinics of North America. 9(1):213-14,1999
o Coarse facies with macroglossia, bushy eyebrows, 4. Levin TL et al: Lumbar gibbus in storage diseases and bone
flat nasal bridge (mild in MPS VI, VII) dysplasias. Pediatr Radiol. 27(4): 289-94, 1997
o Corneal clouding (except MPS II) 5. Piccirilli CB et al: Cervical kyphotic myelopathy in a child
o Significant mental retardation (except in MPS I H/S, with Morquio syndrome. Childs Nerv Syst. 12(2):114-6,
1996
lIb, IV) 6. Vinchon M et al: Cervical myelopathy secondary to Hunter
o Joint contractures, dysostosis multiplex (dominates syndrome in an adult. A]NR Am] Neuroradiol.
in MPS IV, VI) 16(7):1402-3, 1995
• Laboratory diagnosis: Measurement of specific urinary 7. Crockard HA et al: Craniovertebral junction anomalies in
glycosaminoglycans inherited disorders: part of the syndrome .or caused by the
• Clinical diagnostic algorithm disorder? Eur] Pediatr. 154(7):504-12, 1995
o Age, 10, +/- corneal clouding, urinary GAG 8. Blaser S et al: Neuroradiology of lysosomal disorders.
Neuroimaging Clin NA 4:283-98, 1994
excretion, and specific clinical findings
9. Stevens]M et al: The odontoid process in
Demographics Morquio-Brailsford's disease. The effects of occipitocervical
fusion.] Bone Joint Surg BI. 73(5):851-8, 1991
• Age: Usually diagnosed in childhood; occasional mild 10. Murata R et al: MR imaging of the brain in patients with
cases diagnosed as adults mucopolysaccharidosis. A]NR Am] Neuroradiol.
• Gender: M = F except MPS II (boys only ~ X-linked) 10(6):1165-70, 1989
11. Nelson] et al: Clinical findings in 12 patients with MPS IV
Natural History & Prognosis A (Morquio's disease). Further evidence for heterogeneity.
• Premature death the rule; rate of deterioration Part I: Clinical and biochemical findings. Clin Genet.
depends on specific enzymatic deficiency 33(2):111-20, 1988
• Slowly progressive cord compression ~ quadriparesis, 12. Banna M et al: Compressive meningeal hypertrophy in
sensory loss without treatment mucopolysaccharidosis. A]NR Am] Neuroradiol.
o Surgical results poor when performed late in disease 8(2):385-6, 1987
13. Kulkarni MV et al: Magnetic resonance imaging in the
course diagnosis of the crania-cervical manifestations of the
• High spinal cord compression is major spinal cause of mucopolysaccharidoses. Magn Reson Imaging. 5(5):317-23,
MPS complications and death 1987
o Apnea and sudden death may follow relatively 14. Edwards MK et al: CT metrizamide myelography of the
minor trauma cervical spine in Morquio syndrome. A]NR Am]
Neuroradiol. 3(6):666-9, 1982
Treatment
• Options, risks, complications
o Conservative (mild symptoms): External spinal
bracing
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MUCOPOLYSACCHARIDOSES
IIMAGE GALLERY 1
159
Typical
(Left) Sagittal NECT
(Morquio, MPS IV) shows
typical short posterior C7
arch, odontoid hypoplasia,
and mildly hyperdense
pannus (arrows) in an 11
year old male with clinical
cord compression symptoms.
(Right) Sagittal T2WI MR
(Morquio, MPS IV)
demonstrates odontoid
hypoplasia with markedly
hypointense pannus
producing cord compression
and abnormal cord
hyperintensity at the
craniocervical junction.
Variant
(Left) Lateral radiography
(Hurler, MPS I-H) shows a
hypoplastic thoracic
vertebral body (arrow) with
central beaking producing
gibbus deformity (Courtesy
R. Boyer, Mo). (Right)
Sagittal T2WI MR (Hurler,
MPS I-H) shows variant
dural dysplasia and
pronounced degenerative
disc changes (Courtesy R.
Boyer, Mo).
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SICKLE CELL
1
160
Infarcts due to sickle cell tend to affect central portion of Lateral radiography shows central depression of the
vertebral body, causing focal collapse with preservation vertebral endplates, with normal height of anterior and
of height peripherally, yielding an "H-shaped" vertebra. posterior vertebral margins.
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SICKLE CELL
Key Facts
1
Terminology Pathology 161
• Synonyms: Sickle cell anemia, hemoglobin HbSS • 1% of African-Americans homozygous HbSS, 8-13%
disease, HbSC disease, SS disease HbSA (asymptomatic)
Imaging Findings Clinical Issues
• Best diagnostic clue: H-shaped vertebral bodies • Most common signs/symptoms: Sickle cell crisis
• High incidence of infections
Top Differential Diagnoses • Pneumococcal septicemia, meningitis
• Thalassemia
ICLINICAL ISSUES
Presentation
• Most common signs/symptoms: Sickle cell crisis
• Clinical profile (Left) Axial T1 C+ MR with fat-saturation shows peripheral
o Recurrent crises, jaundice, growth retardation, stroke enhancement (arrow) of infarct of lumbar spinous process. Angular
contour of enhancement differs from rounded morphology of
o High incidence of infections
infection. (Right) Sagittal STIR MR displays low signal in vertebral
• Pneumococcal septicemia, meningitis bodies due to marrow hyperplasia. High signal in multiple spinous
processes (arrows) is consistent with bone infarctions.
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OSTEOPETROSIS
1
162
Posteroanterior radiography shows severe sclerosis ofall Lateral radiography shows sclerosis of vertebral bodies,
visualized bones. Cortices are thickened and medullary ribs and sternum, creating a "rugger jersey" appearance
cavities are narrow. similar to renal osteodystrophy.
~
.~.
--.-."
..
-' , . '
~.' ".
Normal Neonate Renal Osteodystrophy Blastic Metastasis Paget Disease
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OSTEOPETROSIS
Key Facts
1
Terminology • Delayed form: Thickened bone cortex, 163
• Synonym: "Marble bone disease" "bone-within-bone" appearance
• Heterogeneous grouping of hereditary osteoclast Top Differential Diagnoses
disorders • Normal newborn
Imaging Findings • Renal osteodystrophy
• Best diagnostic clue: Diffuse increase in bone density • Blastic metastatic disease
• Infantile form: Dense bones, marrow space • Paget disease
obliteration
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OCHRONOSIS
1
164
o Extraspinal findings
ITERMINOlOGY • Premature osteoarthritis of axial skeleton large
Abbreviations and Synonyms joints
• Synonyms: Alkaptonuria, alcaptonuria • Calcification of pubic symphysis fibrocartilage
• Chondrocalcinosis rare in peripheral skeleton
Definitions • +/- Involvement of ear cartilage
• Deposition of homogentisic acid and its metabolites
secondary to absence of homogentisic acid oxidase Imaging Recommendations
enzyme • Best imaging tool: Radiography
• Ochronosis: Abnormal pigmentation caused by
deposition of homogentisic acid
• Alkaptonuria: Homogentisic acid in urine I DIFFERENTIAL DIAGNOSIS
Degenerative disc disease
• Degenerated discs may calcify
IIMAGING FINDINGS • Discogenic vertebral body sclerosis
General Features Ankylosing spondylitis
• Best diagnostic clue: Calcified intervertebral discs • Calcification of annular ligament (not disc) + facet
• Location: Lumbar spine> thoracic> cervical ankylosis
• Morphology: Linear intervertebral disc calcification
Osteoarthritis
Radiographic Findings • May have identical appearance in appendicular
• Radiography skeleton large joints
o Calcified intervertebral discs
o Premature degenerative disc disease Gout
o Ankylosis of spine • Rare in spine; positive cases show endplate
o Osteopenia destruction, tophi
o Scoliosis and kyphosis
o Sacroiliac osteoarthritis
DDx: Ochronosis
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OCHRONOSIS
Key Facts
1
Terminology • Premature degenerative disc disease 165
• Synonyms: Alkaptonuria, alcaptonuria • Ankylosis of spine
• Deposition of homogentisic acid and its metabolites • Osteopenia
secondary to absence of homogentisic acid oxidase • Scoliosis and kyphosis
enzyme • Sacroiliac osteoarthritis
ICLINICAL ISSUES
Presentation (Left) Anteroposterior radiography shows osteoarthritis-like changes
• Most common signs/symptoms due to ochronosis of sacroiliac joints. Chondrocalcinosis is present in
o Dark urine pubic symphysis (arrow) (Courtesy M. Gelman, MO). (Right)
o Other signs/symptoms Anteroposterior radiography shows joint space narrowing and
minimal chondrocalcinosis (white arrow). Contour of femoral head is
• Back pain
irregular (black arrow) (Courtesy M. Gelman, MO).
• Arthritis of large peripheral joints
Demographics
• Age: Arthritis usually develops in adulthood
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Sagittal T7WI MR shows marked dural ectasia eroding Sagittal T2WI MR shows homogeneously high signal
sacrum in patient with Marfan syndrome. intensity CSF in Marfan padent with severe dural ectasia.
o Scoliosis
ITERMINOLOGY • Usually S-curve, same appearance as idiopathic
Abbreviations and Synonyms scoliosis
• Most common types are Marfan, Ehlers-Danlos (EDS), • Sometimes complex curve
Stickler syndrome • Kyphosis often present
• Ehlers-Danlos also known as cutis hyperelastica o Osteopenia
• Often said to be absent in Marfan syndrome, but
Definitions documented by bone densitometry
• Group of congenital disorders with similar imaging o Marfan syndrome
findings • Increased anterior translation CI-C2
• Rotary subluxation CI-C2
• Increased height of odontoid process
IIMAGING FINDINGS • Basilar invagination
• Biconcave vertebrae
General Features o Ehlers-Danlos syndrome
• Best diagnostic clue: Dural ectasia • Platyspondyly
• Location: Most commonly lumbar spine and sacrum
CT Findings
Radiographic Findings • Bone CT
• Radiography o Dural ectasia
o Tremendous overlap in appearance of connective • Width of dural sac> at LS than L4
tissue disorders • Scalloping posterior margins vertebral bodies
• Rely on physical examination, other organ system • Anterior sacral meningocele
findings for specific diagnosis • Perineural cysts
o Dural ectasia • CT myelogram '
• Scalloping posterior margins vertebral bodies o Delineates extent of ectasia
• Widened interpediculate distance o May show diverticula in spinal canal
• Thinned, eroded pedicles • Spinal cord compression can occur
• Erosion of sacrum o Contrast diluted by large CSF space
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Key Facts
1
Terminology • Protocol advice: Include entire sacrum on sagittal 167
• Most common types are Marfan, Ehlers-Danlos (EDS), images
Stickler syndrome Top Differential Diagnoses
• Group of congenital disorders with similar imaging • Neurofibromatosis
findings • Nerve sheath tumor
Imaging Findings • Tarlov cyst
• Tremendous overlap in appearance of connective • Osteogenesis imperfecta (01)
tissue disorders Pathology
• Dural ectasia • Variety of genetic defects in connective tissue
• Scalloping posterior margins vertebral bodies • Aortic root dilation
• Widened interpediculate distance • Mitral valve prolapse
• Scoliosis • Unusually tall, slender body habitus
• Osteopenia • Joint hypermobility
• Best imaging tool: MRI • Arachnodactyly
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CONNECTIVE TISSUE DISORDERS
1 • Marfan: Fibrillin-1 protein important component
of elastic + nonelastic connective tissues
o Average life expectancy < 40 if untreated
• Bone and joint injuries common because of
168 • EDS I-III linked to collagen type V mutations hypermobility
(COLSA1 ~ A3 genes)
• EDS IV linked to collagen type III abnormality Treatment
• EDS VII linked to collagen type I abnormality • Options, risks, complications
• Stickler syndrome: Most involve mutations of o Surgery for scoliosis has increased complication rate
COL2A1 gene compared to idiopathic scoliosis
• Epidemiology o Surgical treatment of aortic dilation allows near
o Ehlers-Danlos: 1:20,000 normal life expectancy
o Marfan: 1:10,000 ~ 20,000 • Lifespan for Marfan syndrome t from 32 years in
• Associated abnormalities 1972 ~ 61 years in 1996
o Marfan, Ehlers Danlos and Stickler syndromes share o Surgical treatment for Ehlers-Danlos complicated by
following skin and connective tissue fragility
• Aortic root dilation • Injuries can be reduced by lifestyle alteration to avoid
• Mitral valve prolapse overly stressing joints
• Unusually tall, slender body habitus
• Joint hypermobility
• Arachnodactyly I DIAGNOSTIC CHECKLIST
• Blue sclerae Image Interpretation Pearls
o Marfan
• Ectopia lentis • Check for aortic dilation on all thoracic spine MR and
• Retinal detachment CT of scoliosis
• Pectus carinatum or excavatum
• Protrusio acetabulae
• Leg length discrepancy ISELECTED REFERENCES
o Ehlers Danlos syndrome 1. Giampietro PF et al: Marfan syndrome: orthopedic and
• Hyperelastic, fragile skin genetic review. CUff Opin Pediatr. 14(1):35-41, 2002
• Fragile blood vessels 2. Jones KB et al: Leg-length discrepancy and scoliosis in
Marfan syndrome. J Pediatr Orthop. 22(6):807-12, 2002
• Aneurysms of multiple vessels
3. Nallamshetty L et al: Plain radiography of the lumbosacral
• Peripheral neuropathy spine in Marfan syndrome. Spine]. 2(5):327-33, 2002
• Triphalangeal thumbs 4. Rose PS et al: Thoracolumbar spinal abnormalities in
• Radioulnar synostosis Stickler syndrome. Spine. 26(4):403-9, 2001
• Some types have ectasia, perforation of bowel 5. Ahn NU et al: Dural ectasia and conventional radiography
o Stickler Syndrome distinguished by in the Marfan lumbosacral spine. Skeletal Radiol.
• Midface hypoplasia, micrognathia 30(6):338-45,2001
6. Herzka A et al: Atlantoaxial rotatory subluxation in
Staging, Grading or Classification Criteria patients with Marfan syndrome. A report of three cases.
• Many types of Ehlers-Danlos Spine. 25(4):524-6, 2000
7. Carter N et al: Bone mineral density in adults with Marfan
syndrome. Rheumatology (Oxford). 39(3):307-9, 2000
8. Letts M et al: The spinal manifestations of Stickler's
ICLINICALISSUES syndrome. Spine. 24(12):1260-4, 1999
9. Villeirs GM et al: Widening of the spinal canal and dural
Presentation ectasia in Marfan's syndrome: assessment by CT.
• Most common signs/symptoms Neuroradiology. 41(11):850-4, 1999
o Dural ectasia often asymptomatic 10. Hobbs WR et al: The cervical spine in Marfan syndrome.
o Cord symptoms if thoracic spine dural ectasia Spine. 22(9):983-9, 1997
o High prevalence back pain in patients with 11. Raft ML et al: Joint hypermobility syndromes. Curr Opin
connective tissue disorders Rheumatol. 8(5):459-66, 1996
12. Raftopoulos C et al: Anterior sacral meningocele and
o Other signs/symptoms Marfan syndrome: a review. Acta Chir Belg. 93(1):1-7, 1993
• Headaches, sciatica 13. Kozlowski K et al: Lumbar platyspondyly--characteristic
• Clinical profile: "Marfanoid" body habitus not specific sign of Ehlers-Danlos syndrome. Skeletal Radiol.
to Marfan syndrome 20(8):589-90, 1991
Demographics
• Age
o Usually becomes apparent in early childhood
o May not present until adulthood
• Gender
o Marfan: M = F
o Ehlers-Danlos: More common in males
Natural History & Prognosis
• Premature death from cardiac disease common
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Typical
(Left) Axial CT myelogram
shows contrast filling
bilateral nerve root sleeve
cysts in patient with Madan
syndrome. (Right) Axial CT
myelogram shows large,
multilocular presacral
meningocele (arrows) in
patient with Madan
syndrome. Note dilution of
contrast due to size of
meningocele.
Typical
(Left) Sagittal STIR MR
shows dural diverticula
(arrows) in thoracic spine.
(Right) Axial CT myelogram
shows large dural
diverticulum (arrow)
compressing spinal cord in
patient with Madan
syndrome.
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OSTEOGENESIS IMPERFECTA
1
170
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...,
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..
....
. .
,
,
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.,~
Spond. Dysplasia Thanatophoric Achondroplasia Osteoporosis
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OSTEOGENESIS IMPERFECTA
Key Facts
1
Terminology • Osteoporosis 171
• Synonyms: Osteogenesis imperfecta (01) "brittle bone Pathology
disease" • Nearly all cases autosomal dominant
• Genetic disorder of type 1 collagen resulting in bone • Inherited or spontaneous mutation
fragility • Epidemiology: 4/100,000 births
• Terms "congenita" and "tarda" no longer used
Clinical Issues
Imaging Findings
• Multiple fractures
• Best diagnostic clue: Severe osteopenia and multiple • Scoliosis
fractures • +/- Deafness, blue sclerae
• Vertebral fractures, vertebra plana, "codfish vertebrae" • Severe forms previously lethal now may survive to
• Classified into 4 types based on clinical, genetic and adulthood
radiographic criteria (see below) • Fractures often less common after puberty
Top Differential Diagnoses • Growth retardation varies with severity of disease
• Nonaccidental trauma (NAT)
• Dwarfisms
o Achondroplasia
MR Findings o Spondyloepiphyseal dysplasia (SED)
• Kyphoscoliosis
• Hydromyelia Osteoporosis
• Abnormal marrow signal intensity due to fractures • Thinned bone cortices, accentuation of primary
• May be used to augment ultrasound for antenatal trabeculae, resorption of secondary trabeculae
diagnosis • Radiography insensitive for diagnosis; better evaluated
by bone densitometry
Ultrasonographic Findings • Codfish vertebrae, compression fractures
• Real Time • Causes
o Antenatal diagnosis in 2nd trimester o Senile
o Poorly ossified skull o Cushing disease
o Short ribs o Hyperparathyroidism
o Short, deformed limbs o Premature menopause
Nuclear Medicine Findings o Cerebral palsy
o Paralysis
• Bone Scan: Positive at fracture sites
o Disuse
Other Modality Findings o Malabsorption syndromes
• Bone densitometry with dual energy x-ray
absorptiometry (DEXA) or CT
o Used to aid diagnosis in mild forms, follow response IPATHOLOGY
to treatment
General Features
Imaging Recommendations • General path comments
• Best imaging tool: Radiography o Abnormal type I collagen
• Protocol advice: Low kVp technique to compensate for o Type I collagen found in bone, skin, sclerae
osteoporosis • Genetics
o Nearly all cases autosomal dominant
o Inherited or spontaneous mutation
IDIFFERENTIAL DIAGNOSIS • Etiology: Numerous type I collagen mutations =;>
"brittle" bone
Nonaccidental trauma (NAT) • Epidemiology: 4/100,000 births
• Normal bone mineral density • Associated abnormalities
• Fractures may otherwise appear identical o Blue sclerae
• Careful history, family evaluation needed o Early hearing loss·
• Genetic testing may be useful o Brittle teeth
o Thin, fragile skin
Dwarfisms o Joint laxity
• Loss of vertebral body height o Respiratory, cardiac problems
• Short stature
• Scoliosis Gross Pathologic & Surgical Features
• Bone density variable • Thin, eggshell-like bone cortices
• Common causes • Decreased medullary trabeculae
o Thanatophoric dwarfism
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OSTEOGENESIS IMPERFECTA
1 • Recent or healed fractures
Treatment
• Bowing, angulation of bones
172 • Overgrowth of epiphyses • Conservative management
o Bisphosphonates used for medical therapy with
Microscopic Features some success
• Lack of organized trabeculae o Prolonged immobilization avoided because of
• Prominent osteoid seams resultant worsening of osteoporosis
• Morphologically normal osteoblasts, increased in • Surgical management
number o Intramedullary rod placement for long bone
• Growth-plate fragmentation fractures
o Due to trauma • Least invasive internal fixation for rapid
o Results in "popcorn" calcifications in metaphyses mobilization
seen on radiographs • Helps prevent progression of deformity, further
o Contributes to growth disturbance fractures
o Spinal fusion for scoliosis
Staging, Grading or Classification Criteria o High risk of hardware mechanical failure
• Type I: Most common type
o Thin, gracile long bones
o High fracture risk in childhood, decreases after
puberty
IDIAGNOSTIC CHECKLIST
o Kyphoscoliosis, wormian bones Consider
o Blue sclerae • Main differential diagnosis is nonaccidental trauma
• Type II: Often lethal early in life
o Short, broad, deformed bones at birth due to
multiple fractures ISELECTED REFERENCES
• Type III: Autosomal recessive; rare
1. Falk MJ et al: Intravenous bisphosphonate therapy in
o Fractures at birth, kyphoscoliosis children with osteogenesis imperfecta. Pediatrics.
• Type IV: Similar to type I 111(3):573-8, 2003
o Kyphoscoliosis, wormian bones 2. Teng SW et al: Initial experience using magnetic resonance
o Sclerae usually normal in adulthood, may be blue in imaging in prenatal diagnosis of osteogenesis imperfecta
childhood type II: a case report. Clin Imaging. 27(1):55-8, 2003
• Recently types V-VII have been proposed, not 3. Zeitlin L et al: Modern approach to children with
distinguishable radiographically osteogenesis imperfecta. J Pediatr Orthop B. 12(2):77-87,
o These types do not localize to genes for type I 2003
4. Iwamoto J et al: Increased bone resorption with decreased
collagen; etiology unknown activity and increased recruitment of osteoblasts in
osteogenesis imperfecta type I. J Bone Miner Metab.
20(3):174-9,2002
ICLINICAL ISSUES 5. Cole WG: Advances in osteogenesis imperfecta. Clin
Orthop. (401):6-16,2002
Presentation 6. Ward LM et al: Osteogenesis imperfecta type VII: an
• Most common signs/symptoms autosomal recessive form of brittle bone disease. Bone.
o Multiple fractures 31(1):12-8,2002
o Scoliosis 7. Marlowe A et al: Testing for osteogenesis imperfecta in
cases of suspected non-accidental injury. J Med Genet.
o +/- Deafness, blue sclerae 39(6):382-6,2002
o Short stature secondary to fractures, kyphoscoliosis, 8. Glorieux FH et al: Osteogenesis imperfecta type VI: a form
growth plate abnormalities of brittle bone disease with a mineralization defect. J Bone
• Clinical profile Miner Res. 17(1):30-8,2002
o Diagnosis suggested by radiographs, confirmed with 9. Glorieux FH et al: Type V osteogenesis imperfecta: a new
• Skin biopsy form of brittle bone disease. J Bone Miner Res.
• Genetic testing 15(9):1650-8, 2000
• Caveat: Both tests may be false negative 10. Mulpuri K et al: Intramedullary rodding in osteogenesis
imperfecta. J Pediatr Orthop. 20(2):267-73, 2000
Demographics 11. Bischoff H et al: Type I osteogenesis imperfecta: diagnostic
difficulties. Clin Rheumatol. 18(1):48-51, 1999
• Age 12. Widmann RF et al: Spinal deformity, pulmonary
o Often evident at birth compromise, and quality of life in osteogenesis imperfecta.
o Mild cases may present in adulthood Spine. 24(16):1673-8, 1999
• Gender: M = F 13. Moore MS et al: The role of dual energy x-ray
absorptiometry in aiding the diagnosis of pediatric
Natural History & Prognosis osteogenesis imperfecta. Am J Orthop. 27(12):797-801,
• Severe forms previously lethal now may survive to 1998
adulthood 14. Kocher MS et al: Osteogenesis imperfecta. J Am Acad
• Fractures often less common after puberty Orthop Surg. 6(4):225-36, 1998
• Growth retardation varies with severity of disease 15. Tongsong T et al: Prenatal diagnosis of osteogenesis
. imperfecta type II. lnt J GynaecolObstet. 61(1):33-8, 1998
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OSTEOGENESIS IMPERFECTA
IIMAGE GALLERY 1
173
(Left) Anteroposterior
radiography shows 1 year
old with type /I disease.
Scoliosis, not present at
birth, has developed. Left
humerus is gracile, right is
broad; both show fractures.
(Right) Lateral radiography
demonstrates flattened
vertebrae throughout lumbar
spine. Disc spaces are much
taller than vertebrae.
(Left) Anteroposterior
radiography reveals typical
type I osteogenesis
imperfecta pelvic deformity,
with gracile bones and
severe protrusio acetabulae.
(Right) Lateral radiography
(type 1 osteogenesis
imperfecta) shows cupping
of vertebral endplates
throughout lumbar spine.
Sacral insufficiency fracture
is also evident (arrow).
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SECTION 1: Trauma
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SPINE ANATOMY
1
2
Lateral graphic of lumbar spine shows disc, vertebral Sagittal TlWI MR shows normal cervical spine vertebral
body, and exWng root relationships. Note normal marrow signal slightly hyperintense to disc, with normal
anterior longitudinal ligament (arrows) and interspinous alignment and prevertebral soft tissues.
ligaments (open arrow).
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SPINE ANATOMY
1
DIFFERENTIAL DIAGNOSIS
Extradural disease • Infectious/meningitis 3
• Trauma/fracture • Subdural/subarachnoid hemorrhage
• Congenital segmentation anomalies Intramedullary disease
• Degenerative disc disease/herniation • Infectious - primary viral or bacterial infection
• Facet degenerative arthropathy/central stenosis • Demyelinating diseases
• Metastatic disease
• Astrocytoma
• Primary bone tumors • Ependymoma
• Disc space infection/vertebral osteomyelitis/epidural • Hemangioblastoma
abscess • Metastatic disease
• Epidural hemorrhage • Infarction
Intradural extramedullary disease • Vascular malformations
• Schwannoma • Metabolic (BIZ deficiency)
• Meningioma • Trauma
• Leptomeningeal metastatic disease
• Granulomatous disease (sarcoid)
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SPINE ANATOMY
1
4
Normal axis anterior view showsodontoid with'articlliar Sagitta(vi~W through CU articiJ!ation. shows apical
facet (arrow), superior articulating process (open ligament (arrow), crudate liga'ment (black arrow), and ".
arrow), body (black arrow), transverse process (curved tectorial membrane (open arrow) being superior
arrow). extension of PLL.
to
o At C2 level, artery loops and turns lateral ascend o Dura - dense tough covering, corresponding to
in C1 transverse foramen meningeal layer ofcranial dura
o Turns medial crossing on top of C1 ingroove • Epidural space filled with fat, loose connective
. 0 Artery then perforates the dura and arachnoid at tissue,' veins
lateral edge of posterior occipitoatlantalmembrane, • Dura continues with spinal nerves through
coursing ventrally on the medulla foramen to fuse with epineurium
• Vertebral column blood supply .• Cephalic attachment at foramen magnum, caudal
o Paired segmental arteries (intercostals, lumbar attachment at back of coccyx
arteries) arise from aorta and extend dorsolaterally o Afachnoid c middle covering; thin and delicate,
around middle of vertebral body' . . continuous with ,cranial arachnoid
o Near transverse process segmental, artery divides into • Separated fromdura by potential subdural space
lateral and dorsal branches o Pia - inner covering of delicate connective tissue
o Lateral branch supplies dorsal musculature closely applied to cord
o Dorsal branch • LongitudinaUibers laterally concentrated as
• Passes lateral to foramen giving off branch(es) denticulate'ligaments lying between posterior and
proViding major vascular supply to bone and . anterior roots, .and attach at 21 points to dura
vertebral canal contents •. Longitudil1.alfibers concentrateddorsally as
• Posterior central branch supplying disc: and septum posticum attaching dorsal cord to dorsal
vertebral body midline dura
• Prelaminal branch supplies inner surface of arch
and ligamenta flava, regional epiduraLtissue
• Neural branch entering neural foraniensupplies I CUSTOM DIFFERENTIAL DIAGNOSIS I
pia, arachnoid, cord
• Postlaminar branch supplies musculature Disease 'localization .
overlying lamina and brane:hesto bone • Extradura'l disease encompasses disease of vertebral
• Nerves bodies, arch, facets, paravertebral and epidural soft
o Spinal nerves arranged in 31pairs grouped tissues anddisc/aIlllulus complex
regionally: 8 cervical, 12 thoracic,S lumbar,S sacral, • Intraduralextramedllllary disease encompasses dura,
1 coccygeal arachnoid and intrathecal nerve roots, vessels
o Ascensus spinalis - apparent developmental rising of • Irttramedllllaryericompasses intrinsic spinal cord
cord related to differential spinal growth' . abnormalities of grey and white matter, vessels
• Course of nerve roots becomes longer and more
oblique at lower segments "."
.• C1 nerve from C1 segment and exits above Cl SELECTED REFERENCES
• C8 nerve from C7 segment and exits~ C7-TI I, WhiteML:CerviCalspine: MR imaging techniques and
• T6 nerve from TS segment and exits~. T6-T7 anatorny.Magn Reson Imaging Clin N Am. 8(3):453-70,
• T12 nerve from T8 segment and exits~T12-L1 2000 .
• L2 nerve from TID segment and exits =0> Ll-L2 2. Bowen BC et al: Vascular anatomy and disorders of the
• S3 nerve from T12 segmeniand exitsS3foramen lumbar spine and spinal cord: Magn Reson Imaging Clin N
Am. 7(3):555-71,1999
• Meninges
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SPINE ANATOMY
IIMAGE GALLERY
1
5
Typical
(Left) Sagittal TIWI MR
shows normal parasagittal
anatomy. Note smooth
articulation of superior
(arrow) and inferior (open
arrow) articular facets,
occipital condyle
relationship to CI (curved
arrow). (Right) Axial TlWI
MR shows normal anatomy
at I.ST. Superior facet is
anterior (arrow), and inferior
facet posterior (open arrow).
Vertebral artery at neural
foramenallevel shows flow
void (curved arrow).
Typical
(Left) Axial graphic shows
normal lumbar disc level,
with annulus fibrosus (white
arrow), nucleus pulposus
(black arrow), facets (open
arrow), transverse process
(curved arrow). (Right)
Posterior view of cervical
spine with posterior elements
removed shows relationship
of uncovertebral joints
(arrows), PLL (open arrow),
and vertebral arteries in
transverse foramen.
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SPINE FRACTURE CLASSIFICATION-MODELS
1
6
Sagittal graphic shows unstable cervical hyperflexion Lateral radiography shows C4-5 flexion facet dislocation
injury involving anterior· and posterior longitudinal with bilateral "jumped" facets. There is disruption of all
ligaments, disc and interspinous ligaments, epidural three columns by this injury.
hemorrhage/cord compression.
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SPINE FRACTURE CLASSIFICATION-MODELS
DIFFERENTIAL DIAGNOSIS
1
Clinical implications • Stable injuries can be treated non-operatively 7
• Denis classification unstable injuries includes • Not all unstable injuries need operative treatment
• 1) Three column disruption Odontoid fractures
• 2) > 5(YVt) collapse of anterior cortex • Mortality rate of elderly with odontoid fractures up to
• 3) > 25 degrees focal kyphosis 25li'b
• 4) Extending neurologic deficit • Type I odontoid fractures should be evaluated for
Thoracolumbar fractures occipitoatlantal instability
• Compression or wedging fracture involve only • Nonunion of odontoid bony injuries uncommon,
anterior column, stable except for type II odontoid fractures
• Burst fracture involve anterior, middle +/- posterior • Type III odontoid fractures show broad surface of
columns, unstable or possibly unstable trabecular bone and good prognosis for healing
• Seat belt or Chance fractures involve posterior and
middle columns, unstable
• Fracture dislocation involve all columns and are
highly unstable
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Sagittal graphic of thoracolumbar junction shows. Sagittal graphic showing Chance (seat belt) fracture of
compression (wedge) fractures involving primarily thoracolumbar junction extending in horizontal plane
anterior column, with normal middle and posterior through body and posterior elements (three column
columns. involvement). .
compromise
o Fat suppressed T2WI, STIR for definition of posterior
ligamentous disruption
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ATLANlTO··()CCIPITAllOISLOCATION·,··
, • - , -, - - - < ~ -
1
10
Sagittal graphic shows fatal atlanta-occipital dissociation, Lateral radiography shows increased distance between
with cord transection at craniocervical junction. C7 is basion and Cl, & abno(mal Powers' ratio. Prevertebral
displaced posteriorly relative to skull base. soft tissues are too wide, even allowing for possible
bleeding related to tubes.
,.
DDx: Craniocervical Juhcti0rJ Instability·
l~~ ~
.. ,<?.-
.~~,
- .,
Occipital Fracture Down Syndrome AO Dislocation OcCipital Fracture
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ATLANTO-OCCIPITAL DISLOCATION
Key Facts
1
Terminology Pathology 11
• Disruption of stabilizing ligaments between occiput, • Etiology: High speed motor vehicle accident
Cl
Clinical Issues
Imaging Findings • Often immediately fatal
• Increased distance from basion to odontoid
• Powers' ratio> 1.15
Diagnostic Checklist
• Best imaging tool: CT scan with coronal + sagittal • Only sign of injury on radiographs may be widened
reformations prevertebral soft tissues
ICLINICAL ISSUES
(Left) Coronal bone CT shows disruption of both occiput-CI (open
Presentation arrows) and CI-C2 (arrows) facet joints. (Right) Sagittal STIR MR
• Most common signs/symptoms shows disrupted apical ligament (open arrow) and anterior
o Cranial nerve injuries atlanta-occipital membrane (curved arrow). Tectorial membrane
o Peripheral motor deficit (arrow) appears intact.
Demographics
• Age: More common in children
Natural History & Prognosis
• Often immediately fatal
• High incidence neurologic deficits in survivors
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OCCIPITAL CONDYLE FRACTURE
1
12
Axial bone CT demonstrates minimally displaced right Axial bone CT shows nondisplaced left occipital
occipital condyle fracture without comminution condyle fracture (arrow) as involvemeiJt from skull base
(arrow). fracture (open arrows).
. -
Accessory Centers Osteomyeiitis Metastasis Rheumatoid Arthritis
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OCCIPITAL CONDYLE FRACTURE
Key Facts
1
Terminology Clinical Issues 13
• Occipital condylar Fx 2° high-energy blunt trauma • Presenting symptoms usually related to severity of
head injury rather than to OCFx itself
Imaging Findings • Cranial nerve (CN) deficit(s) (up to 30%)
• Best diagnostic clue: OCFx on CT • No specific predictors of OCFx
• Morphology: Linear, comminuted, or avulsion type • Generally inconsistent
Fx • Head injury is main determinant of outcomes
• CT: Thin-section with sagittal, coronal reformats • Surgical therapy is controversial
• MRI: Sagittal & coronal STIR • Follow-up CT is recommended 10-12 weeks after
Top Differential Diagnoses injury to document fracture healing
• Accessory ossification center(s) Diagnostic Checklist
• Marrow space abnormality • Suspect OCFx in all patients sustaining high-energy
Pathology blunt trauma to head, upper cervical spine
• May be as high as 19% of patients with high-energy • Search for OCFx in presence of unexplained neck
blunt trauma pain, lower CN deficit(s)
• Via nerve root compression or stretching o MRI: Sagittal & coronal STIR
• Direct brain stem injury or vascular compromise
Radiographic Findings IDIFFERENTIAL DIAGNOSIS
• Radiography
o Very insensitive (0/51 in one study) due to Accessory ossification center(s)
• AP ~ superimposition of maxilla and occiput • Anterior to OCD
• Lateral ~ superimposition of mastoid processes • Well corticated
• Open mouth ~ difficult or impossible to obtain in
unconscious, intubated, severely injured patients Marrow space abnormality
o May see prevertebral soft tissue swelling • Infectious: Osteomyelitis
• Fluoroscopy • Neoplastic: Primary or metastatic neoplasms
o Flexion/extension fluoroscopy to assess for • Inflammatory: Rheumatoid arthritis
ligamentous injury, instability
• Only after triaging injury with CT
• Displacement of a Fx during flexion/extension IPATHOLOGY
could result in grave consequences
General Features
CT Findings • Etiology
• CTA: Evaluate vertebrobasilar patency/injury o High-energy blunt trauma, most often MVA
• Bone CT o Deployment of air bags has been associated with
o Uni- or bilateral OCFx, occipitoatloid subluxation craniocervical junction injury
o Associated skull base Fx • Epidemiology
• Extension to hypoglossal and/or jugular foramen o May be as high as 19% of patients with high-energy
o Associated C1 or C2 Fx(s) blunt trauma
• True incidence, prevalence remains unknown
MR Findings o Increasingly being reported likely related to
• STIR Widespread use of CT & MR imaging
o Occipitoatloid joint subluxation, alar lig. disruption • Associated abnormalities
o Marrow edema o Subdural/epidural hematoma, subarachnoid
o Prevertebral or nuchal ligament edema hemorrhage, intra-axial contusion
o Cord edema or hemorrhage o Cervical spine fracture, usually C1 or C2
o Foramen magnum extradural, subdural hemorrhage o Thoracic, abdominal, & limb injuries
• MRA: Evaluate vertebrobasilar patency/injury
Staging, Grading or Classification Criteria
Angiographic Findings • Anderson & Montesano classification (1988)
• Conventional: Replaced by CTA, MRA o Type I: Least common
Imaging Recommendations • Produced by axial load injury with a component
• Best imaging tool of ipsilateral flexion; considered an impaction
o Bone CT for Fx OCFx
o MRI for soft tissues • Results in a comminuted Fx without displacement
• Protocol advice o Type II
oCT: Thin-section with sagittal, coronal reformats • Basilar skull fracture involving occipital condyle,
usually from a direct blow to skull
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OCCIPITAL CONDYLE FRACTURE
1 • Linear Fx that may involve both OC
• Intact tectorial membrane & alar ligaments
o 35% who survive to discharge have poor outcomes
at 1 month requiring continued nursing support,
14 preserve stability tube feeding, or tracheostomy care; 35% of these
o Type III: Most common (75%) gain functional independence with rehabilitation
• Avulsion Fx of inferomedial OC, usually result of o Remaining 65% have good outcomes becoming
severe contralateral flexion & rotation independently mobile & self-caring within 1 month
• May have medially displaced Fx fragment, & • Limited & painful mobility of craniocervical junction
partial or complete disruption of tectorial can persist after OCFx despite good osseous
membrane & contralateral alar ligament consolidation, especially when type III
• Inferior clivus may also be involved o Children generally have good osseous consolidation
• Tulli classification (1997) with normal & painless craniocervical mobility
o Type 1: Most common: Nondisplaced OCFx; stable
o Type 2A: Displaced OCFx with intact ligaments; Treatment
stable • By A & M classification
o Type 2B: Displaced OCFx with anyone or move o Type I & II OCFx are considered stable (tectorial
criteria of radiographic instability membrane & contralateral alar ligament intact)
• > 8° of axial occipitoatloid rotation to one side • Treated conservatively ~ semirigid or rigid collar
• > 1 mm of occipitoatloid translation o Type III OCFx is a potentially unstable injury if
• > 7 mm of overhang of CIon C2 ligamentous injury present
• > 45° of axial rotation of C1-C2 to one side • Assess for instability ~ flexion/extension
• > 4 mm of C1-C2 translation • Treated with rigid collar, halo traction vest, or
• < 13 mm distance between posterior body of C2 to surgical fixation
posterior ring of C1 • By Tulli classification
• Avulsed transverse ligament with MR evidence of o Type 1: Require no specific treatment
ligamentous disruption o Type 2A: May be treated with a rigid collar
• Hanson classification (2001) o Type 2B: Surgical instrumentation or halo traction
o Uses A & M classification as a base • Surgical therapy is controversial
o Subdivides type III into stable & unstable o Possible indications ~ neurovascular decompression
and/or stabilization
o Usually posterior fusion (occipitoatlantoaxial
ICLINICAL ISSUES arthrodesis)
• Follow-up CT is recommended 10-12 weeks after
Presentation injury to document fracture healing
• Most common signs/symptoms
o Presenting symptoms usually related to severity of
head injury rather than to OCFx itself IDIAGNOSTIC CHECKLIST
o Cranial nerve (CN) deficit(s) (up to 30%)
• One or more CN9-12 (most often 12) Consider
• 40% manifest days to months after trauma, • Suspect OCFx in all patients sustaining high-energy
possibly fragment migration or callus formation blunt trauma to head, upper cervical spine
• Collet-Sicard syndrome = full 9th through 12th Image Interpretation Pearls
CN deficits • Search for OCFx in presence of unexplained neck pain,
o Spasmodic torticollis from concomitant atlantoaxial lower CN deficit(s)
rotatory fixation
o Dysphagia from retropharyngeal hematoma
o Hemi- or quadriparesis ISELECTED REFERENCES
o Vertebrobasilar ischemia
o High cervical pain, impaired skull mobility 1. Momjian S et al: Occipital condyle fractures in children.
Case report and review of the literature. Pediatr Neurosurg.
• Clinical profile 38(5):265-70,2003
o No specific predictors of OCFx 2. Hanson JA et al: Radiologic and clinical spectrum of
o Generally inconsistent occipital condyle fractures: retrospective review of 107
o Most patients have mild/moderate j GCS consecutive fractures in 95 patients. AJR Am J Roentgenol.
178(5):1261-8,2002
Demographics 3. Hadley et al: Occipital condyle fractures. Neurosurgery.
• Age: Infants to elderly 50(3 Suppl):S114-9, 2002
• Gender: M:F = 3:1 4. Demisch S et al: The forgotten condyle: Delayed
hypoglossal nerve palsy caused by fracture of the occipital
Natural History & Prognosis condyle. Clin Neurol Neurosurg. 100(1):44-5, 1998
• Functional recovery after unilateral CN deficit(s) is 5. Tuli S et al: Occipital condyle fractures. Neurosurgery.
generally good 41(2):368-76; discussion 376-7, 1997
• Head injury is main determinant of outcomes 6. Bloom AI et al: Fracture of the occipital condyles and
associated craniocervicalligament injury: incidence, CT
imaging and implications. Clin Radiol. 52(3):198-202, 1997
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OCCIPITAL CONDYLE FRACTURE
IIMAGE GALLERY 1
15
Typical
(Left) Sagittal bone CT
reformat demonstrates
minimally displaced right
occipital condyle fracture
without comminution
(arrow). (Right) Sagittal STIR
MR in the same case shows
hyperintense edema along
occipital condyle fracture
(arrow) as well as associated
hyperintense soft tissue
injury to both ligaments &
muscle (open arrows).
Typical
(Left) Axial bone CT shows
nondisplaced left occipital
condyle fracture (arrow).
(Right) Coronal bone CT
reformat demonstrates
nondisplaced left occipital
condyle fracture (arrow).
Typical
(Left) Coronal bone CT
reformat demonstrates
nondisplaced left occipital
condyle fracture (arrow)
contiguous with hypoglossal
canal/skull base fracture (not
shown). (Right) Coronal
bone CT reformat shows
hypoglossal canal
involvement (arrows) from
contiguous occipital condyle
& skull base fracture (not
shown).
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JEFFERSON C1 FRACTURE
1
16
Axial bone CT shows fracture of Cl arch and avulsion of Coronal reformation shows avulsion Fx of C 7 pillar
inner lateral mass at attachment of transverse ligament (arrow) at attachment of transverse ligament, thus
(arrow). instability. Lateral offset of Cl vs. C2 pillars: Cl arch
fracture seen on axial cut.
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JEFFERSON C1 FRACTURE
Key Facts
1
Terminology Top Differential Diagnoses 17
• Atlas burst fracture • Pseudospread of the atlas in children
• Compression fracture of Cl arch • Congenital variants, clefts, malformations of atlas
• Rotational malalignment of atlas, axis pillars
Imaging Findings • ~ Generally seen unilaterally, with rotation and
• Fluoroscopy: Subluxation if unstable abduction of head
• CT defines components of fracture to best advantage
• May see various patterns of arch disruption Diagnostic Checklist
• Look for avulsion fragment off inner pillar at • Routine CT of cervical spine in every trauma victim
insertion of transverse ligament complaining of severe neck pain
• ~ Indicates instability • Careful not to overcall 1-2 mm offset of C1lateral
• Any lateral spread of C1 pillars on open-mouth x-ray masses vs. those of C2 on open mouth view in
view requires CT infants
• Evaluate entire cervical spine (and even upper
thoracic) as associated fractures occur in 24-48% of
cases
• PD/Intermediate: Prevertebral soft tissue swelling • 97% of posterior clefts are midline, 3% through sulcus
• MRA of vertebral artery
o May show vertebral artery occlusion • Various deficiencies of arch development can be seen
o MRA useful if delayed cerebellar or brainstern signs • Most are partial hemiaplasias of posterior arch
appear • Clefts, congenital defects show smooth or
well-corticated edges
Angiographic Findings
• Conventional: Only needed if CTA or MRA findings Rotational malalignment of atlas, axis pillars
are inconclusive • ~ Generally seen unilaterally, with rotation and
abduction of head
Imaging Recommendations
• Any lateral spread of Cl pillars on open-mouth x-ray
view requires CT !PATHOlOGY
• CT bone reconstruction algorithm details sites of
fracture General Features
o Distinguishes well-corticated margins of congenital • General path comments
clefts from jagged edges of fracture defect o Rough-edge fragmentation of Cl arch at one or
• Thin-section (1 mm) cuts mandatory, reformations more sites
very helpful o Typically a stable fracture, unless transverse
• Evaluate integrity of VBA foramina ligament avulsed, more than 7 mm offset a clue to
• Evaluate entire cervical spine (and even upper such avulsion and instability
thoracic) as associated fractures occur in 24-48% of • Unstable fractures occur if transverse or posterior
cases longitudinal ligaments disrupted
• Or if anterior arch is severely comminuted
• And are more often associated with severe
IDIFFERENTIAL DIAGNOSIS neurological deficits, lower levels of injury
o Combined fractures occur, especially those of C2
Pseudospread of the atlas in children • Etiology
• Common finding in many children 3 months to four o Axial compressive force applied to skull vertex
years of age evaluated for minor trauma o Force transmitted down through occipital condyles
• Seen in 90% or more of two year olds onto C1 pillars with head + neck rigidly erect
• Caused by disparity in growth rates of the atlas and • Epidemiology
axis o C1 fractures represent 6% of all vertebral injuries
• Jefferson fracture rare in young children - greater o One-third of Cl fractures conform to classic burst
plasticity, synchondroses of Cl arch serve as "buffer" Jefferson fracture
Congenital variants, clefts, malformations of o Rare in infants, young children
• Associated abnormalities
atlas o Fractures at other levels
• May show 1-2 mm offset of Cl pillars from those of o VBA injury; dissection or occlusion
C2
• Clefts found in 4% of posterior arches, 0.1 % of
anterior arches
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JEFFERSON C1 FRACTURE
1 ICLINICAL ISSUES 11. Gehwiler JA et al: Malformations of the atlas simulating
the Jefferson fracture. AJNR 4:187-90, 1983
18 12. Jefferson G: Fracture of the atlas vertebra. Report of four
Presentation cases and a review of those previously recorded. Br J Surg
• Most common signs/symptoms 7:407-11, 1920
o Upper neck pain after compression trauma (e.g.,
diving)
o Other signs/symptoms
• Neurologic signs uncommon, unless unstable
fracture or associated fractures are present, or if
VBA injured
• Clinical profile
o Upper neck pain
o Trauma victim
o Fracture can be missed on plain films
Natural History & Prognosis
• Stable fracture with healing in majority of isolated
cases
Treatment
• Immobilization, fusion if gross instability
IDIAGNOSTIC CHECKLIST
Consider
• Routine CT of cervical spine in every trauma victim
complaining of severe neck pain
• Evaluate lower levels for additional Fxs (present in
25-50% of cases)
Image Interpretation Pearls
• Well-corticated edges of midline Cl arch defects are
likely congenital clefts
• Careful not to overcall 1-2 mm offset of Cllateral
masses vs. those of C2 on open mouth view in infants
ISELECTED REFERENCES
1. Lustrin ES et al: Pediatric cervical spine: normal anatomy,
variants, and trauma. Radiographies. 23(3): 539-60, 2003
2. Torreggiani WC et al: Musculoskeletal case 20. Jefferson
fracture (Cl burst fracture). Can J Surg. 45(1): 16, 65-6,
2002
3. Harris JH: The cervicocranium: its radiographic assessment.
Radiology 218:337-51,2001
4. Connor SE et al: Congenital midline cleft of the posterior
arch of atlas: a rare cause of symptomatic cervical canal
stenosis. Eur Radiol. 11(9): 1766-9, 2001
5. Sharma A et al: Partial aplasia of the posterior arch of the
atlas with an isolated posterior arch remnant: findings in
three cases. AJNR Am J Neuroradiol. 21(6): 1167-71, 2000
6. Guiot B et al: Complex atlantoaxial fractures. J Neurosurg.
91(2 Suppl): 139-43, 1999
7. Haakonsen M, Gudmundsen TE, Histol O. Midline anterior
and posterior atlas clefts may simulate a Jefferson fracture.
A report of 2 cases. Acta Orthop Scand. 66(4):369-71, 1995
8. Currarino G et al: Congenital defects of the posterior arch
of the atlas: a report of seven cases including an affected
mother and son. AJNR Am J Neuroradiol. 15(2):249-54,
1994
9. Kesterson L et al: Evaluation and treatment of atlas burst
fractures (Jefferson fractures). J Neurosurg. 75(2):213-20,
1991
10. Lee C et al: Unstable Jefferson variant atlas fractures: an
unrecognized cervical injury. AlNR Am J Neuroradiol.
12(6):1105-10, 1991
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JEFFERSON C1 FRACTURE
IIMAGE GALLERY 1
19
Typical
(Left) Axial bone CT shows
fractures of anterior C1 arch
(arrows). (Right) Coronal
reformation, bone windows,
shows lateral offset of C 1 vs.
C2 lateral mass. Axial slices
showed anterior & posterior
left sided C 1 arch fractures,
right side of arch was intact.
Variant
(Left) Lateral radiography
shows fractures of C 1, C2,
C5 (arrows) in patient with
diving injury (axial load and
flexion). Note prevertebral
swelling. (Right) Axial bone
CT shows anterior arch
components of jefferson
fracture in this patient with
multiple fractures (C 1
fracture being subtle on
radiography). Lower slices
showed C2 & C5 fractures.
Typical
(Left) Axial bone CT window
shows left-sided Cl arch
fractures. (Right) Axial bone
CT shows developmental
midline defect (arrow) in Cl
arch; note sclerotic,
corticated edges. Foramen
magnum level includes
occipital condyles (curved
arrows) & tip of dens (open
arrow).
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ODONTOID C2 FRACTURE
1
20
Anterior graphic shows three fractures through C2: Type Lateral radiography shows defect at base of dens and
I odontoid (arrow); type 11/ odontoid (open arrow) & prevertebral swelling (arrow), with posterior
horizontal axis body fracture (curved arrow). displacement of dens and CI arch vs. C2 (curved
arrow). Note osteopenia.
MR Findings
IIMAGING FINDINGS
• TlWI
General Features o Loss of dens/C2 body contiguity
• Best diagnostic clue: Lateral radiograph shows anterior o Replacement of normal marrow's high signal from
or posterior displacement of Cl arch vs. CZ, + swelling fat, by low signal of edema
of prevertebral soft tissue o Displacement of dens from C2 body, typically
anterior subluxation
Radiographic Findings • T2WI: High signal of edema in marrow, + prevertebral
• Radiography soft tissues
o Classic imaging appearance: Lucent linear defect
through base of dens, with posterior displacement of Nuclear Medicine Findings
dens arch of Cl relative to CZ body/arch • Bone Scan: Can be used to detect old fracture with
o Open-mouth frontal radiograph depicts transverse non-union
or oblique defect through dens Imaging Recommendations
o May be difficult to depict in elderly with
• Plain x-rays (especially lateral, open-mouth views)
osteoporosis
initially suggest need for CT if any of above
• Fluoroscopy: Used to evaluate stability of fusion mentioned clues are present
CT Findings.
• NECT: Soft tissue swelling anterior to C2 in acute cases
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ODONTOID C2 FRACTURE
Key Facts
1
Terminology Top Differential Diagnoses 21
• Traumatic bony disruption of odontoid process • Congenital nonunion of odontoid tip (Os
odontoideum)
Imaging Findings .• Metastatic or other pathologic C2 fracture
• Best diagnostic clue: Lateral radiograph shows
anterior or posterior displacement of C1 arch vs. C2, Pathology
+ swelling of prevertebral soft tissue • Three ossification centers form C2
• Classic imaging appearance: Lucent linear defect • Os odontoideum represents unfused ossification
through base of dens, with posterior displacement of center atop dens C2
dens arch of C1 relative to C2 body/arch • Type III fracture follows embryologic line of union
• Open-mouth frontal radiograph depicts transverse or between dens and body of C2
oblique defect through dens • Type I: Avulsion of tip
• May be difficult to depict in elderly with osteoporosis • Type II: Transverse fracture at base of dens
• Axial (source) images may miss fracture if its plane • Type III: Fracture extending into body of C2
parallels slice orientation
• Thin-section (1 mm) axial slices with bone • Type III fracture follows embryologic line of union
reconstruction algorithm with fastest possible scan between dens and body of C2
times for optimal reformation into sagittal, coronal • Etiology
planes o Caused by sudden forward or backward movement
• MRI with T1WI in sagittal/coronal planes (3 mm of the head, with neck rigidly erect & articulations
slices), T2WI in sagittal plane to evaluate canal size, locked
cord injury o Osteoporosis in elderly predisposes to type II
• GRE imaging to detect blood in cord if myelopathy is fracture + nonunion
present
Staging, Grading or Classification Criteria
• Type I: Avulsion of tip
IDIFFERENTIAL DIAGNOSIS • Type II: Transverse fracture at base of dens
• Type III: Fracture extending into body of C2
Congenital nonunion of odontoid tip (Os
odontoideum)
• Well-corticated ossification center above rudimentary
ICLINICAL ISSUES
dens Presentation
• No soft tissue swelling • Most common signs/symptoms
• No history of trauma, or pain o Neck pain
Congenital variation - 3rd occipital condyle o Other signs/symptoms
• One of several anomalous bony structures which may • Myelopathy
form at foramen magnum as remnant of first • Clinical profile
sclerotome o Often in elderly, osteoporotic patients
• This midline bony peg off anterior lip of foramen o Fluctuating long tract signs, spasticity may be the
magnum may articulate to dens, simulate odontoid only presentation in older patients whose initial
fracture trauma was minor & not well diagnosed
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ODONTOID C2 FRACTURE
1 o Nonunion uncommon after treatment with traction
followed by bracing
22
IDIAGNOSTIC CHECKLIST
Consider
• T2WI serve to verify edematous marrow, and better
depict associated soft tissue edema in prevertebral
space (missing in chronic nonunion)
• T1WI sagittal and coronal images best depict the
disruption of bone contiguity, and degree of
displacement
o Marrow shows signal loss from edema in acute cases,
normal signal indicates chronic nonunion
• Flexion/extension films or fluoroscopy for evaluating
stability
Image Interpretation Pearls
• Sclerotic margins of ununited dens indicate chronic
non-union of old fracture
ISELECTED REFERENCES
1. Muller EJ et al: Non-rigid immobilisation of odontoid
fractures. Eur Spine J. 12(5):522-5, 2003
2. v Ludinghausen M et al: The third occipital condyle, a
constituent part of a median occipito-atlanto-odontoid
joint: a case report. Surg Radiol Anat. 24(1):71-6, 2002
3. Rao PV: Median (third) occipital condyle. Clin Anat.
15(2):148-51,2002
4. Sanderson SP et al: Fracture through the C2 synchondrosis
in a young child. Pediatr Neurosurg. 36(5):277-8, 2002
5. Martin-Ferrer S: Odontoid fractures. J Neurosurg. 95(1
Suppl):158-9,2001
6. Weisskopf M et al: CT scans versus conventional
tomography in acute fractures of the odontoid process. Eur
Spine J. 10(3):250-6, 2001
7. Sasso RC: C2 dens fractures: treatment options. J Spinal
Disord. 14(5):455-63, 2001
8. Teo EC et al: Biomechanical study of C2 (Axis) fracture:
effect of restraint. Ann Acad Med Singapore. 30(6):582-7,
2001
9. Vaccaro AR et al: Contemporary management of adult
cervical odontoid fractures. Orthopedics. 23(10):1109-13;
quiz 1114-5, 2000
10. Ziai WC et al: A six year review of odontoid fractures: the
emerging role of surgical intervention. Can J Neurol Sci.
27(4):297-301,2000
11. Govender S et al: Fractures of the odontoid process. J Bone
Joint Surg Br. 82(8):1143-7, 2000
12. Swischuk LE et al: Is the open-mouth odontoid view
necessary in children under 5 years? Pediatr Radiol.
30(3):186-9, 2000
13. Brant-Zawadzki M et al:CT in the evaluation of spine
trauma. AJR. 136: 369, 1981
14. Charlton OP et al: Roentgenographic evaluation of cervical
spine trauma. JAMA. 242:1073-5, 1979
15. Anderson et al: Fractures of the odontoid process of the
axis. J Bone and Joint Surg 56A: 1663-74, 1974
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ODONTOID C2 FRACTURE
IIMAGE GALLERY 1
23
Typical
(Left) Axial bone CT shows
fracture at base of dens
(arrow); oblique plane of
anatomy vs. slice orientation
makes for confusing finding.
(Right) Sagittal bone CT
reformation shows true plane
of fracture, displacement of
dens & C 1 arch posteriorly to
better advantage.
Variant
(Left) Sagittal bone CT
reformation shows fracture
through base of dens, with
permeative bony destruction
of dens, C2 body, and
extensive prevertebral soft
tissue mass due to metastatic
disease. (Right)
Anteroposterior radiography
(open mouth) shows
transverse type /I fracture
only vaguely (arrow) due to
osteopenia.
Variant
(Left) Sagittal TlWI MR
shows fracture at base of
dens with low signal margins
indicating bony sclerosis, &
normal C2 marrow signal
(also seen on T2WI),
indicative of chronic fracture
nonunion. (Right) Lateral
radiography shows sclerotic
bony defect at base of dens.
Note lack of prevertebral
swelling, no subluxation
suggesting fibrous fusion.
Nonunion of old fracture
versus os odontoideum.
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BURST FRACTURE, C2
1
24
Axial bone CT demonstrates comminuted C2 burst Coronal bone CT reformat reveals comminuted C2
fracture with centrifugal displacement of fragments and burst fracture with laterally displaced fragments &
involvement of bilateral transversarium foramen. involvement of bilateral transversarium foramen.
o Frontal radiograph
ITERMINOlOGY • May see vertical Fx line within C2 body, either
Abbreviations and Synonyms midline or eccentric
• Fracture (Fx) • Magnitude indicated by degree of lateral fragment
• Dispersion or axial loading Fx displacement, disruption of Luschka joints
o Lateral radiograph
Definitions • Vertebral body comminution
• Compression injury often with displaced fracture • Posteriorly retropulsed fragments
fragments causing cord compromise • Intact facets
o Highly associated with hangman fracture • Focal prevertebral soft tissue swelling> 6 mm
• 100% by one report . o Associated hangman
• Bilateral pars Fx
• C2/3 anterolisthesis
IIMAGING FINDINGS • Disruption Cl/2 spinolaminar line
• Disruption C2/3 posterior vertebral line
General Features • Myelography: Demonstrates canal narrowing from
• Best diagnostic clue: Comminuted fracture of C2 body fragment retropulsion
with multiple fragments dislocated anteroposteriorly
• Location: C2 body CT Findings
• Size: May involve entire vertebral body • CTA
• Morphology: Minimal to severe deformity o Quickly, easily evaluates for vertebral artery injury if
Fx involves transversarium foramen
Radiographic Findings • Normal
• Radiography • Mass effect impinging luminal diameter
o Variable range • Dissection
• Minimal compression with minimal fragment • Occlusion
displacement • CT Bone
• Severe comminution o Axial images
• Marked posteriorly retropulsed fragments
-..
DDx: C2 Burst Fracture Mimics
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,
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BURST FRACTURE, C2
Key Facts
1
Terminology Top Differential Diagnoses 25
• Compression injury often with displaced fracture • Odontoid type 3 Fx
fragments causing cord compromise • Pathologic Fx
• Highly associated with hangman fracture • Flexion teardrop Fx
Imaging Findings Pathology
• Best diagnostic clue: Comminuted fracture of C2 • Associated abnormalities: Hangman Fx, cord
body with multiple fragments dislocated contusion/hematoma, vertebral artery injury
anteroposteriorly
• Morphology: Minimal to severe deformity Clinical Issues
• Posteriorly retropulsed fragments • Symptoms are variable from no deficit to quadriplegia
• Focal prevertebral soft tissue swelling> 6 mm • Age: C-spine injury: 80% of patients are 18-25 yrs
• May have acute cord injury • Gender: C-spine injury: M:F = 4:1
• Best imaging tool: CT Bone • Considered unstable injury given high frequency of
• CT: Acquire axially with sagittal & coronal reformats association with hangman fractures
• MRI: Add sagittal STIR & GRE • Steroids if neurologic symptoms & < 8 hrs in onset
• There is no standard surgical therapy
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BURST FRACTURE, C2
1 • Abnormal flexed alignment
• Anteroinferior body "teardrop" fragment
o May have transient symptoms
o With cord injury
26 • Distraction of posterior column • Spinal shock: Flaccidity, areflexia, loss of anal
o Facet subluxation/distraction sphincter tone, fecal incontinence, priapism, loss
o Spinous process splaying of bulbocavernosus reflex
• Most severe & unstable C-spine type of Fx • Neurogenic shock: Hypotension, paradoxical
bradycardia, flushed/dry/warm peripheral skin
• Autonomic dysfunction: Ileus, urinary retention,
IPATHOLOGY poikilothermia
• Clinical profile: C-spine injury: Clinical evaluation is
General Features unreliable - 46% sensitivity
• General path comments
o Since spinal canal diameter is greatest at C2, cord Demographics
injury from retropulsed fragment compression is the • Age: C-spine injury: 80% of patients are 18-25 yrs
exception • Gender: C-spine injury: M:F = 4:1
o Considered a "major" Fx type • High risk activities: Diving, equestrian activities,
• Etiology football, gymnastics, skiing, hang gliding
o MVAs, falls cause most C2 fractures
Natural History & Prognosis
o Mechanism of burst
• Compression forces cause axial loading • Considered unstable injury given high frequency of
association with hangman's fractures
• C2/3 nucleus pulposus is driven through C2
inferior endplate, into vertebral body Treatment
• Sudden "explosive" pressure 1 causes vertebral • Traction with cervical tongs if> 25% loss of height,
body comminution fragment retropulsion, neurologic deficit
• Fragments undergo centrifugal dispersion • Steroids if neurologic symptoms & < 8 hrs in onset
• Posteriorly retropulsed fragments enter canal & • Surgical treatment
may impact, compress cord o There is no standard surgical therapy
o Hangman: Hyperextension injury • Per Congress of Neurological Surgeons standards
• Epidemiology o Spontaneous interbody fusion between C2 & C3 can
o 25% of C2 fractures cannot be classified as odontoid occur - conservative immobilization
or hangman Fxs - vertebral body, laminae, spinous o Consider surgical C2/3 anterior interbody fusion
processes
• C2 burst is an uncommon Fx
o Up to 10% of unconscious patients presenting to the
ED following MVA have C-spine pathology
ISELECTED REFERENCES
1. Congress of Neurological Surgeons: Isolated fractures of the
o 1/3 C-spine injuries involve C2
axis in adults. Neurosurgery. 50(3 Suppl):SI25-39, 2002
o C-spine injuries cause estimated 6,000 deaths & 2. Daffner RH et al: A new classification for cervical vertebral
5,000 new cases of quadriplegia each yr injuries: influence of CT. Skeletal Radiol. 29(3):125-32,
• Associated abnormalities: Hangman's Fx, cord 2000
contusion/hematoma, vertebral artery injury 3. Fujimura Y et al: Classification and treatment of axis body
fractures. J Orthop Trauma. 10(8):536-40, 1996
Gross Pathologic & Surgical Features 4. Lozano-Requena JA et al: Sagittal fracture of the second
• Comminuted Fx cervical vertebral body. Int Orthop. 18(2):114-5, 1994
• Extruded blood, cellular debris, fragments of tissue 5. Burke JT et al: Acute injuries of the axis vertebra. Skeletal
envelope Fx site in hematoma Radiol. 18(5):335-46, 1989
• Inflammation begins within hrs & continues for 6. Hadley MN et al: Acute axis fractures: a review of 229 cases.
J Neurosurg. 71(5 Pt 1):642-7, 1989
several days until replaced by developing soft callus Baba H et al: Burst fracture of the body of the axis; a case
7.
Microscopic Features report Uapanese). Orthop Surg TraumatoI32:1659-62, 1989
8. Jakim I et al: Transverse fracture through the body of the
• Periosteal disruption axis. J Bone Joint Surg Br. 70(5):728-9, 1988
• Cortical bone displacement & separation 9. Guerra J Jr et al: Vertebral burst fractures: CT analysis of
• Disruption of vessels within Haversian canals at Fx site the retropulsed fragment. Radiology. 153(3):769-72, 1984
• Extrusion of marrow contents into Fx gap 10. Kobayashi K: Axis body fracture Uapanese). Seikei Geka
28:1145-1153, 1977
Staging, Grading or Classification Criteria 11. Brashear R Jr et al: Fractures of the neural arch of the axis.
• Classification of axis body fractures: Avulsion, A report of twenty-nine cases. J Bone Joint Surg Am.
transverse, burst, & sagittal 57(7):879-87, 1975
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Symptoms are variable from no deficit to
quadriplegia
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BURST FRACTURE, C2
IIMAGE GALLERY 1
27
Typical
(Left) Sagittal bone CT
reformat reveals centrifugal
displacement of C2 burst
fracture fragments with
associated retropulsion into
the spinal canal. (Right)
Sagittal T1 WI MR
demonstrates hypointensity
within fracture marrow as
well as retropulsed fragment
entering canal. Cord signal
intensity isointense appears
normal.
Typical
(Left) Sagittal T2WI MR
better demonstrates
retropulsed fragment
entering canal effacing
anterior CSF Cord
edemalcontusion
hyperintensity can be seen.
(Right) Sagittal STIR MR
confirms cord hyperintensity
from contusion as well as
hyperintense soft tissue
ligamentous injury anteriorly
and posteriorly (arrows).
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HANGMAN'S C2 FRACTURE
1
28
Lateral radiography shows lucent gap in pedicles of C2, Sagittal T2WI MR shows the anterior subluxation of C2
its laminal line aligned with C3, but its body and C1 vb, edema in the disc interspace and anteriorly,
anteriorly displaced vs. C3. preservation of anteroposterior canal dimension and
cord integrity.
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HANGMAN'S C2 FRACTURE
Key Facts
1
Terminology • Additional fracture levels seen in 33% of cases, C1 29
• Traumatic spondylolisthesis of axis (TSA) most commonly
• Canal at C2 enlarged
Imaging Findings • Any anterior subluxation of C2 vs. C3 on lateral x-ray
• Anterior displacement of C2 vb, C1, & skull vs. C3 on deserves CT
lateral x-ray • Evaluate entire cervical spine (and even upper
• Classic imaging appearance: Defects through pedicles thoracic) as associated fractures occur in 33% of cases
of C2, C2 vertebral body (vb) anterior to C3 vb, • MR if neurologic symptoms are present
laminae aligned
• Axial slice shows disrupted ring of C2
Top Differential Diagnoses
• Variation of fracture sites, including extension into • Pseudosubluxation
body, not uncommon • Rotatory subluxation of C2-C3
• Dens typically spared • Primary spondylolysis
• Extension into vertebral artery foramen raises
possibility of vertebral artery (VBA) damage
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HANGMAN'S C2 FRACTURE
1 • Cerebellar findings suggest stroke due to vertebral
artery dissection or occlusion
spondylolysis: a patient and a brief review. Pediatr Radiol.
21(5):367-8, 1991
30 • Clinical profile 14. Burke JT et al: Acute injuries of the axis vertebra. Skeletal
Radiol. 18(5):335-46, 1989
o Pain upper neck after trauma
15. Fielding JW et al: Traumatic spondylolisthesis of the axis.
o Neurological sequelae in minority of traumatic Clin Orthop. (239):47-52, 1989
cases, as canal wide here and further decompressed 16. Mivris SE et al: Hangman's fracture: radiologic assessment
by fracture in 27 cases. Radiology 163: 713-7, 1987
o Nevertheless, neurological sequelae occur in 25% 17. Baumgarten M et al: Computed axial tomography in CI-C2
o Vertebral artery injury may cause delayed trauma. Spine. 10(3):187-92, 1985
neurological signs 18. Hadley MN et al: Axis fractures: a comprehensive review of
management and treatment in 107 cases. Neurosurgery.
Natural History & Prognosis 17(2):281-90, 1985
• Depends on presence of neurological damage 19. Sherk HH et al: Clinical and pathologic correlations in
• Delayed stroke a possibility if vertebral artery damaged traumatic spondylolisthesis of the axis. Clin Orthop.
(174):122-6, 1983
• Accelerated degenerative changes 20. Bucholz RW: Unstable hangman's fractures. Clin Orthop.
Treatment (154):119-24, 1981
21. Pepin JW et al: Traumatic spondylolisthesis of the axis:
• Immobilization Hangman's fracture. Clin Orthop. (157):133-8, 1981
• Fusion 22. Seljeskog EL et al: Spectrum of the hangman's fracture. J
Neurosurg. 45(1):3-8, 1976
23. Marar BC: Fracture of the axis arch. "Hangman's fracture"
IDIAGNOSTIC CHECKLIST of the cervical spine. Clin Orthop. (106):155-65, 1975
24. Williams TG: Hangman's fracture. J Bone Joint Surg Br.
Consider 57(1):82-8, 1975
• Check vb alignment, soft tissue thickness (4 mm or
less) on lateral x-ray and get CT if abnormal
• Evaluate transverse foramen for integrity; get MRA if
any question to exclude vertebral artery injury
Image Interpretation Pearls
• C2-C3 vb anterior subluxation with laminalline
normally aligned requires CT even without fracture
shown on x-ray
ISELECTED REFERENCES
1. Ranjith RK, Mullett JH, Burke TE. Hangman's fracture
caused by suspected child abuse. A case report. J Pediatr
Orthop B. 11(4):329-32, 2002
2. Management of combination fractures of the atlas and axis
in adults. Neurosurgery. 50(3 Suppl):SI40-7, 2002
3. Harrop JS, Vaccaro A, Przybylski G]. Acute respiratory
compromise associated with flexed cervical traction after
C2 fractures. Spine. 15;26(4):E50-4, 2001
4. Isolated fractures of the axis in adults. Neurosurgery. 50(3
Suppl):SI25-39,2002
5. Samaha C et al: Hangman's fracture: the relationship
between asymmetry and instability. J Bone Joint Surg Br.
82(7):1046-52, 2000
6. Williams JP 3rd et al: CT appearance of congenital defect
resembling the Hangman's fracture. Pediatr Radiol.
29(7):549-50, 1999
7. Guiot B et al: Complex atlantoaxial fractures. J Neurosurg.
91(2 Suppl):139-43, 1999
8. Agrillo U et al: Hangman's fracture. Spine. 24(22):2412,
1999
9. Greene KA et al: Acute axis fractures. Analysis of
management and outcome in 340 consecutive cases. Spine.
22(16):1843-52, 1997
10. Nunez DB et al: Cervical spine trauma: How much more do
we learn by routinely using helical CT? Radiographics 16:
1307-18, 1996
11. Starr JK et al: Atypical hangman's fractures. Spine.
18(14):1954-7, 1993
12. James R et al: The occurrence of cervical fractures in
victims of judicial hanging. Forensic Sci Int. 54(1):81-91,
1992
13. Parisi M et al: Hangman's fractur~ or primary
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HANGMAN'S C2 FRACTURE
I IMAGE GALLERY 1
31
Typical
(Left) Axial bone CT at level
of pedicles (7 mm section
does not include laminae)
shows their cleavage
(arrows) from body of C2.
Note relationship of fracture
line to VBA foramen (curved
arrow). (Right) Lateral
radiography in flexion shows
exaggerated gap at level of
pedicular fracture and
maximal subluxation. Note
increased anteroposterior
canal dimension at level of
fracture.
Variant
(Left) Sagittal bone CT
reformation shows anterior
displacement of C2 vb, C 1,
skull; C2 arch stays with C3-
finding typical of
Hangman's. (Right) Axial
bone CT shows transverse
right C2 pedicle fracture,
sagittal left pedicle fracture
entering vertebral foramen
(arrow).
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HYPERFLEXION INJURY, CERVICAL
1
32
Sagittal graphic shows flexion injury at C4-5 with Sagittal radiography shows focal kyphosis, separation of
subluxation, disc herniation, ligamentous disruption, spinous processes, and anterior subluxation of facets at
cord compression & epidural hemorrhage. C5-6 in patient with pain, no neurologic defect. Note
wide canal.
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HYPERFLEXION INJURY, CERVICAL
Key Facts
1
Terminology Pathology 33
• Flexion force disrupting capsular and posterior • Anatomy of C-spine predisposes to instability once
ligaments, with anterior vertebral ligaments are disrupted
displacement/angulation • Associated abnormalities: Central cord syndrome
Imaging Findings Diagnostic Checklist
• Best diagnostic clue: Focal kyphosis, abnormal • Fluoroscopy with appropriate history of severe
vertical separation of facets/spinous processes flexion injury, but only minimal separation on static
films
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HYPEREXTENSION INJURY, CERVICAL
1
34
l.a.teral radiography shows ill-defined defect in C2 arch Axial bone CT windowed section shows disruption of
(arrow), with slight anterior C2 vb displacement. Note both laminae just anterior to spinous process in this
old C7 spinous process (clay shoveler) Fx (open arrow), padent with traumatic hyperextension.
from previous trauma.
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HYPEREXTENSION INJURY, CERVICAL
1
Key Facts
• Must obtain CT once plain film findings suggest 35
Terminology
fracture
• Fracture of the laminae due to forceful posterior
• MR vital if neurologic signs present to evaluate cord
displacement of head and/or upper cervical spine
injury, compression
Imaging Findings
Top Differential Diagnoses
• Best diagnostic clue: Bony defect through posterior
arch with posterior element malalignment • Whiplash fracture
• Rotational fracture-subluxation
• Bone CT
• High-resolution algorithm best for non-displaced • Clay shoveler fracture
fracture • Congenital cleft
• Arch defect best shown on axial views Diagnostic Checklist
• Sagittal reformation shows listhesis, facet • CT in any case, particularly when shoulder density
relationships precludes evaluation of cervico-thoracic junction
• Shows cord edema if myelopathy present • Using CTA or MRA to exclude vertebral artery injury
• T2* GRE: Best depicts associated hemorrhagic cord
contusion
Whiplash fracture
• Associated vb fracture ICLINICAL ISSUES
• Malalignment more common
Presentation
Rotational fracture-subluxation • Most common signs/symptoms: Severe neck pain with
• May have associated laminar fracture transient or lasting myelopathic signs
• Clinical profile: History of trauma with extension
Clay shoveler fracture components and/or axial loading (e.g., diving)
• Spinous process avulsion from sudden severe force
placed on ligamentum nuchae Natural History & Prognosis
• Laminae typically spared • Depends on presence/degree of neurologic
compromise
Congenital cleft • Good if no neurologic damage and stabilization
• Typically midline achieved
• Accelerated degenerative disease may be seen
• Neurologic recovery can occur if only mild edema is
seen in cord acutely
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HYPEREXTENSION INJURY, CERVICAL
1 • Fixed neurologic deficit if cord shows hemorrhagic
contusion
the evaluation of posterior cervical spine fractures. Spine.
24(8):771-4, 1999
13. Makan P: Neurologic compromise after an isolated laminar
36 • Progression of fixed deficit superiorly if post-traumatic
fracture of the cervical spine. Spine. 24(11):1144-6, 1999
syrinx appears late 14. Kinoshita H: Pathology of hyperextension injury of the
cervical spine: a case report. Spinal Cord. 35(12):857-8,
Treatment 1997
• If no neurological injury, treatment is aimed at IS. Plezbert JA et al: Fracture of a lamina in the cervical spine. J
immobilization (halo) and correction of any deformity Manipulative Physiol Ther. 17(8):552-7, 1994
• If neurologic signs present, and compression found, 16. Kinoshita H: Pathology of hyperextension injuries of the
acute decompression cervical spine. Paraplegia. 32(6):367-74, 1994
• If cord is edematous, high dose steroids (first 24 hours) 17. Lukhele M: Fractures of the vertebral lamina associated
with unifacet and bifacet cervical spine dislocations. S Afr J
may help
Surg. 32(3):112-4, 1994
• If cord demonstrates hemorrhage or transection, 18. Plezbert JA et al: Fracture of a lamina in the cervical spine. J
immobilization to prevent further deformity Manipulative Physiol Ther. 17(8):552-7, 1994
19. Kang JD et al: Sagittal measurements of the cervical spine
in subaxial fractures and dislocations. An analysis of two
IDIAGNOSTIC CHECKLIST hundred and eighty-eight patients with and without
neurological deficits. J Bone Joint Surg Am. 76(11):1617-28,
Consider 1994
• CT in any case, particularly when shoulder density 20. Kiwerski J: Hyperextension-dislocation injuries of the
precludes evaluation of cervico-thoracic junction cervical spine. Injury. 24(10):674-7,1993
21. Silberstein M et al: Prevertebral swelling in cervical spine
• Evaluation of upper thoracic spine for associated injury: identification of ligament injury with magnetic
fractures, as non-contiguous fractures can be seen resonance imaging. Clin Radiol. 46(5):318-23, 1992
• Using CTA or MRA to exclude vertebral artery injury 22. Goldberg AL et al: Hyperextension injuries of the cervical
spine. Magnetic resonance findings. Skeletal Radiol.
Image Interpretation Pearls 18(4):283-8, 1989
• Subtle malalignment of posterior laminalline on 23. Barquet A et al: An unusual extension injury to the cervical
lateral radiograph should prompt CT spine. A case report. J Bone Joint Surg Am. 70(9):1393-5,
• Multiple, non-contiguous fractures may be seen 1988
24. Edeiken-Monroe B et al: Hyperextension dislocation of the
cervical spine. AJR Am J Roentgenol. 146(4):803-8, 1986
ISELECTED REFERENCES 25. Coin CG et al: Diving type injury of the cervical spine:
Contribution of CT to management. J Comput Assist
1. Ranger GS: "Radiographic clearance of blunt cervical spine Tomogr. 3: 362-5, 1979
injury: plain radiograph or computed tomography scan?", 26. Calenoff L et al: Multiple level spinal injuries: importance
by Griffen MM, et al. J Trauma. 56(2):457; author reply of early recognition. AJR Am J Roentgenol. 130(4):665-9,
457,2004 1978
2. Berlin L: CT versus radiography for initial evaluation of 27. Penning L: Functional pathology of the cervical spine.
cervical spine trauma: what is the standard of care? AJR Am Baltimore, Williams and Wilkins, 1968
J Roentgenol. 180(4):911-5,2003
3. Cothren CC et al: Cervical spine fracture patterns
predictive of blunt vertebral artery injury. J Trauma.
55(5):811-3,2003
4. Hayes KC et al: Retropulsion of intervertebral discs
associated with traumatic hyperextension of the cervical
spine and absence of vertebral fracture: an uncommon
mechanism of spinal cord injury. Spinal Cord.
40(10):544-7,2002
5. Maiman DJ et al: Preinjury cervical alignment affecting
spinal trauma. J Neurosurg. 97(1 Suppl):57-62, 2002
6. Hanson JA et al: Cervical spine injury: a clinical decision
rule to identify high-risk patients for helical CT screening.
AJR Am J Roentgenol. 174(3):713-7,2000
7. Matar LD et al: "Spinolaminar breach": an important sign
in cervical spinous process fractures. Skeletal Radiol.
29(2):75-80, 2000
8. Biffl WL et al: The devastating potential of blunt vertebral
arterial injuries. Ann Surg. 231(5):672-81, 2000
9. Klein GR et al: Efficacy of magnetic resonance imaging in
the evaluation of posterior cervical spine fractures. Spine.
24(8):771-4, 1999
10. Zhu Q et al: Traumatic instabilities of the cervical spine
caused by high-speed axial compression in a human
model. An in vitro biomechanical study. Spine.
24(5):440-4, 1999
11. Brady WJ et al: ED use of flexion-extension cervical spine
radiography in the evaluation of blunt trauma. Am J Emerg
Med. 17(6):504-8, 1999
12. Klein GR et al: Efficacy of magnetic resonance imaging in
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Typical
(Left) Sagittal bone CT shows
displaced laminar fragment
at C3, midline, as well as
slight anterior subluxation of
C2 on C3; both C2 and C3
showed laminar fractures on
axial views. (Right) Axial
NECT shows blood within
and behind the canal in a
patient rendered paraplegic
by extension trauma. Note
disrupted bony arch.
Typical
(Left) Axial NECT shows
"sprung" C7 arch in patient
with paralysis after extension
trauma. (Right) Axial NECT
shows fractures of T4
laminae in patient who also
showed mid-cervical
extension fracture.
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HYPERFLEXION-ROTATION INJURY, CERVICAL
1
38
Lateral graphic shows disrupted facet and posterior Sagittal Oblique radiography shows offset of facets at
ligaments, with facet subluxation. C5-6 (arrow), inferior C5 facet perched on superior C6
facet.
ITERMINOLOGY CT Findings
Abbreviations and Synonyms • CTA: Done to detect vertebral artery dissection or
occlusion
• Jumped facet(s)
• Bone CT
• Rotatory subluxation of vertebral bodies (vb), posterior
a Normally, lower vb facet (superior facet) is anteriorly
elements in the cervical spine (C-spine)
oriented in respect to its upper neighbor's inferior
Definitions facet
• Traumatic disruption of cervical spine (ligaments a Axial: "Naked" inferior facet; its subjacent neighbor,
alone, or together with bony elements) causing facet superior facet of the lower vb, not seen at joint
subluxation interface
a Sagittal: Affected lateral mass "perched" on or
jumped anterior to subjacent partner
IIMAGING FINDINGS a May see fracture of facet tip
a May see fracture of vb if significant flexion
General Features component
• Best diagnostic clue: Malalignment of adjacent facets a Axial views show rotated relationship between lower
and spinous processes on lateral x-ray, with focal vb with its upper neighbor
vertebral body angulation
MR Findings
• Location: Typically mid- or lower C-spine
• TlWI
Radiographic Findings a Shows the malalignment
• Radiography a Insensitive to associated fractures
a Altered adjacent facet relationship on lateral • T2WI
radiograph a Depicts edema of disrupted ligaments
a Spinous process malalignment, esp. vertical offset on a Demonstrates cord compromise, contusion when
anteroposterior view present
a Focal vb kyphosis • STIR: More sensitive to fracture than TlWI, but not as
sensitive as CT
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HYPERFLEXION-ROTATION INJURY, CERVICAL
Key Facts
1
Terminology • Posterior laminalline (point of junction of the 39
• Jumped facet(s) laminae) should show the same gentle lordotic curve
• Rotatory subluxation of vertebral bodies (vb), as anterior + posterior vb edges on lateral view
posterior elements in the cervical spine (C-spine) • Prevertebral soft tissue should show Vz thickness of AP
vb diameter or less
Imaging Findings • AP x-ray should show regular spacing between
• 5 view cervical spine series starts work-up spinous processes, all vertically aligned in midline
• CT with thin axial sections & reformations • Disc space height loss can be clue (in absence of
recommended with serious trauma, or if patient degenerative changes)
cannot cooperate
• Always evaluate the following relationships when Pathology
examining C-spine trauma x-rays • Rotatory shear forces tear capsular, annular,
• Anterior vb edges should trace gentle C-like lordosis longitudinal ligaments; allows even intact bony
• Posterior edges of vb should parallel the anterior elements to sublux
curve
• Facets should align on lateral and oblique views
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HYPERFLEXION-ROTATION INJURY, CERVICAL
1 Natural History & Prognosis
for management and neurologic outcome. AJR Am J
Roentgenol. 163(5):1165-9, 1994
40 • Dictated by type and degree of neurological deficit 16. Willis BK et al: The incidence of vertebral artery injury
• Mild cord contusion can regress after midcervical spine fracture or subluxation.
• Cord hematoma heralds grave prognosis Neurosurgery. 34(3):435-41; discussion 441-2, 1994
17. Shapiro SA: Management of unilateral locked facet of the
Treatment cervical spine. Neurosurgery. 33(5):832-7; discussion 837,
1993
• Traction
18. Hadley MN et al: Facet fracture-dislocation injuries of the
• Decompression and stabilization as necessary cervical spine. Neurosurgery. 30(5):661-6, 1992
• Fusion 19. Roy-Camille R et al: Treatment of lower cervical spinal
injuries--C3 to C7. Spine. 17(10 Suppl):S442-6, 1992
20. Beyer CA et al: Unilateral facet dislocations and
IDIAGNOSTIC CHECKLIST fracture-dislocations of the cervical spine: a review.
Orthopedics. 15(3):311-5, 1992
Consider 21. Myers BS et al: The role of torsion in cervical spine trauma.
• CT in any case of malalignment of vbs, facets on Spine. 16(8):870-4, 1991
radiograph of C-spine 22. Young JW et al: The laminar space in the diagnosis of
rotational flexion injuries of the cervical spine. AJR Am J
Image Interpretation Pearls Roentgenol. 152(1):103-7, 1989
23. Rorabeck CH et al: Unilateral facet dislocation of the
• Check facet surface relationships on every lateral
cervical spine. An analysis of the results of treatment in 26
radiograph patients. Spine. 12(1):23-7, 1987
• Obtain sagittal reformations through both left + right 24. Yetkin Z et al: Uncovertebral and facet joint dislocations in
lateral mass columns on every CT done for spine cervical articular pillar fractures: CT evaluation. AJNR Am J
trauma Neuroradiol. 6(4):633-7, 1985
• Look for associated vascular injury 25. Brant-Zawadzki M et al: Trauma, Computed Tomography
of the Spine and Spinal Cord. Newton TH, Potts DG,
Clavadal Press, 149-86, 1983
ISELECTED REFERENCES 26. O'Callaghan JP et al: CT of facet distraction in flexion
injuries of the thoracolumbar spine: the "naked" facet. AJR
1. Vaccaro AR et al: Is magnetic resonance imaging indicated Am J Roentgenol. 134(3):563-8, 1980
before reduction of a unilateral cervical facet dislocation? 27. Pick RY et al: C7--T1 bilateral facet dislocation: a rare lesion
Spine. 27(1):117-8, 2002 presenting with the syndrome of acute anterior spinal cord
2. Crawford NR et al: Unilateral cervical facet dislocation: injury. Clin Orthop. (150):131-6, 1980
injury mechanism and biomechanical consequences. 28. Ravichandran G: Traumatic single facet subluxation of
Spine. 27(17):1858-64; discussion 1864, 2002 cervical spine without neurological damage. A new clinical
3. Hart RA: Cervical facet dislocation: when is magnetic sign. Arch Orthop Trauma Surg. 92(2-3):221-4, 1978
resonance imaging indicated? Spine. 27(1):116-7, 2002 29. Scher AT: Unilateral locked facet in cervical spine injuries.
4. Sim E et al: In vitro genesis of subaxial cervical unilateral AJR Am J Roentgenol. 129(1):45-8, 1977
facet dislocations through sequential soft tissue ablation. 30. BabcockJL: Cervical spine injuries. Diagnosis and
Spine. 26(12):1317-23, 2001 classification. Arch Surg. 111(6):646-51, 1976
5. Lingawi SS: The naked facet sign. Radiology. 219(2):366-7, 31. Holdsworth FW et al: Fractures, Dislocations and
2001 Fracture-Dislocations of the Spine. Journal of Bone and
6. Razack N et al: The management of traumatic cervical Joint Surgury. 45B: 6-20, 1963
bilateral facet fracture-dislocations with unicortical
anterior plates. J Spinal Disord. 13(5):374-81,2000
7. Daffner RH et al: A new classification for cervical vertebral
injuries: influence of CT. Skeletal Radiol. 29(3):125"32,
2000
8. Argenson C et al: Traumatic rotatory displacement of the
lower cervical spine. Bull Hosp Jt Dis. 59(1):52-60, 2000
9. An HS et al: Cervical Spine Trauma. Spine. Vol 23:2713-29,
1998
10. Andreshak JL et al: Management of unilateral facet
dislocations: a review of the literature. Orthopedics.
20(10):917-26, 1997
11. Halliday AL et al: The management of unilateral lateral
mass/facet fractures of the subaxial cervical spine: the use
of magnetic resonance imaging to predict instability.
Spine. 22(22):2614-21, 1997
12. Leite CC et al: MRI of cervical facet dislocation.
Neuroradiology. 39(8):583-8, 1997
13. Korres DS et al: The significance of rotation in
fracture-separation of the articular pillar of a lower cervical
vertebra. A clinical and cadaveric study. Acta Orthop Scand
Suppl. 275:17-20, 1997
14. Sim E: Vertical facet splitting: a special variant of rotary
dislocations of the cervical spine. J Neurosurg.
82(2):239-43, 1995
15. Shanmuganathan K et al: Rotational injury of cervical
facets: CT analysis of fracture patterns with implications
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HYPERFLEXION-ROTATION INJURY, CERVICAL
IIMAGE GALLERY 1
41
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BURST FRACTURE, CERVICAL
1
42
Sagittal bone CT window reformation shows vertical Axial bone CT shows fracture plane through C7
cleavage plane of C7 burst fracture. Pedicles + arch vertebral body, which extended from superior to inferior
were intact. end plate.
ITERMINOLOGY MR Findings
• TlWI: Low signal and loss of height of vb
Abbreviations and Synonyms • T2WI
• Shattered vertebral body (vb) o High signal, loss of vb height with soft tissue edema
• Teardrop fracture in prevertebral region .
Definitions o May see canal compromise, even cord compression
• Comminuted fracture (Fx) of vb extending through if retropulsed fragment present
both endplates due to axial compression • T2* GRE: May show cord contusion
Angiographic Findings
• Conventional: Only done if vertebral artery foramen
IIMAGING FINDINGS fractured, or signs of vertebrobasilar insufficiency
General Features suggest dissection
• Best diagnostic clue: Wedged lower cervical vb with Imaging Recommendations
end plate defect, focal gibbus on lateral radiograph • Always obtain CT if Fx of cervical vb seen on
• Location: Typically mid- or lower cervical spine radiographs
Radiographic Findings • Thin-section axial slices with sagittal reformations,
bone and soft tissue windows
• Radiography: Cleavage of vb extending through end
• MR vital if neurologic symptoms/signs present
plate with focal flexion or gibbus deformity
• MRA or CTA if Fx involves vertebral artery foramen
CT Findings
• NECT: Fragmented vb without posterior element Fx
• Bone CT IDIFFERENTIAL DIAGNOSIS
o Fracture lines extend through end plates, anterior
and/or posterior vb margins
Benign compression fracture
• No endplate-to-endplate comminution
• No retropulsion of fragments or pedicular widening
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BURST FRACTURE, CERVICAL
Key Facts
1
Terminology Top Differential Diagnoses 43
• Comminuted fracture (Fx) of vb extending through • Benign compression fracture
both endplates due to axial compression • Flexion fracture
Imaging Findin'gs Clinical Issues
• Best diagnostic clue: Wedged lower cervical vb with • Severe neck pain after trauma with axial load
end plate defect, focal gibbus on lateral radiograph • Radiculopathy or myelopathy may be present
IPATHOLOGY 2.
Spine. 27(2):160-6, 2002
Kiwerski JE: Early anterior decompression and fusion for
crush fractures of cervical vertebrae. lnt Orthop.
General Features 17(3):166-8, 1993
• General path comments: Instability of anterior/middle 3. Schweighofer F et al: Complications after an unusual
column procedure in the treatment of a cervical spine burst
• Etiology: Axial compression, mild flexion combine to fracture. A ca.se report. Langenbecks Arch Chir.
produce the Fx 377(4):235-6, 1992
4. Ripa DR et al: Series of ninety-two traumatic cervical spine
Gross Pathologic & Surgical Features injuries stabilized with anterior ASIF plate fusion .
technique. Spine. 16(3 Suppl):S46-55, 1991 .
• Fragmented vertebral body 5. Cabanela ME et al: Anterior plate stabilization for bursting
teardrop fractures of the cervical spine. Spine.
13(8):888-91, 1988
ICLINICAL ISSUES 6. Mazur JM et al: Unrecognized spinal instability associated
with seemingly "simple" cervical compression fractures.
Presentation Spine. 8(7):687-92, 1983
• Most common signs/symptoms 7. Johnson JL et al: Nonoperative treatment of the acute
o Severe neck pain tear-drop fracture of the cervical spine. Clin Orthop.
o Other signs/symptoms (168):108-12, 1982
• Myelopathy or radiculopathy if canal or foraminal
compromise by fragments
• Clinical profile IIMAGE GALLERY
o Severe neck pain after trauma with axial load
o Radiculopathy or myelopathy may be present
Natural History & Prognosis
• Self-limited unless neurologic injury becomes
permanent
Treatment
• Surgical stabilization and (if needed) canal
decompression
o Bed rest may be sufficient with immobilization if
comminution is minimal
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HYPEREXTEN:SION':'ROTATION; CERVICAL
1
44
Axial bone CT shows linear fracture extending from Axial CECT shows disrupted left vertebral artery (arrow),
lateral mass into left vertebral foramen in patient after & left lateral mass fracture after vehicular accident.
motor vehicle accident CT angiogram showed Adjacent slice showed fracture line into foramen.
dissected vertebral artery.
DDx: .postetiorElementJractures
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HYPEREXTENSION-ROTATION, CERVICAL
Key Facts
1
Terminology • NECT: Fractured pedicle, articular pillar or laminar 45
• Variation of hyperextension fracture strut
• Unilateral facet or laminar fracture due to • CECT: May show absent filling of vertebral artery
hyperextension + rotation combined, typically with • May see edematous annulus, or ligaments
ligament disruption • Effusion within facet joint
• Will show cord edema or contusion, if present
Imaging Findings
• Best diagnostic clue: Unilateral posterior element
Diagnostic Checklist
fracture . • MRA or CTA in any case of unilateral posterior
element fracture
IDIAGNOSTIC CHECKLIST
Consider
• MRA or CTA in any case of unilateral posterior (Left) Axial bone CT after intrathecal contrast injection shows
element fracture unilateral left laminar fracture in patient with rotational extension
Image Interpretation Pearls injury. (Right) Sagittal oblique CT reformation shows fragmented
lateral mass (arrow) in patient with rotational extension injury.
• MRI best evaluates associated disc extrusion, cord
injury
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1
46
Anterior graphic shows disrupted left facets & ligaments Sagittal bone CT reformation shows vertical cleavage of
from lateral flexion injury C7 lateral mass. Axial view showed fracture of
uncovertebral process. AP radiograph showed slight
rotation.
'~\-,
/.
IJ'
:,."
"Jumped" Facet "Floating" Pillar Flexion-Extension
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LATERAL FLEXION INJURY, CERVICAL
Key Facts
1
Terminology Top Differential Diagnoses 47
• Articular mass fracture, pillar fracture • Flexion/rotation fracture
• Fracture isolation of lateral mass
Imaging Findings
• Slight malalignment maybe only clue on lateral view Diagnostic Checklist
• Sagittal fracture of pillar • CT in any serious spine injury or persistent
• Associated transverse process fracture, or vb fracture post-traumatic neck pain
IDIAGNOSTIC CHECKLIST
Consider
• CT in any serious spine injury or persistent
post-traumatic neck pain
(Left) Axial bone CT shows fractures of right lateral mass,'
Image Interpretation Pearls uncovertebral process (arrow) and transverse process at C7. (Right)
Anteroposterior radiography shows step-off at C6-7 in spinous
• Unilateral displacement of one facet versus its processes alignment, separation of left uncovertebral joint,
neighbor suggests fracture compression of C7 right lateral mass suggesting its fracture (shown by
CT).
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, ,
POSTERIOReO'I.l.JMNINJLJIIY",' eERVICAI.
1
48
Axial NECT shows disrupted posterior arch, with blood Axial bone CT shows unilateral fracture of left lateral
in epidural space & within posterior ligaments indicative mass (arrow) a stable fracture within the posterior
of severe posterior column injury. column. Note congenital cleft at laminal line (curved
arrow).
ITERMINOlOGY CT Findings
• NECT: Fx of laminae or articular pillar, may see blood
Abbreviations and Synonyms in epidural space
• Vertebral body (vb) • Bone CT
• Posterior longitudinal ligament (PLL) o High-resolution algorithm better at detecting
• Fracture (Fx) non-displaced Fx
Definitions MR Findings
• Posterior column: Spinal architecture beyond PLL,
• T2WI
anulus, posterior vb margin o Increased signal in muscles, ligaments
o Includes facets, their joint ligaments, laminae, o Depicts cord edema when contusion present
ligamentum flavum, interspinous ligaments, spinous • STIR: Best for showing soft tissue edema, marrow
processes edema of fractured vb or arch
Imaging Recommendations
IIMAGING FINDINGS • Best imaging tool: CT with soft tissue + bone windows,
reformations
General Features • Protocol advice: 1 mm slice thickness, bone algorithm
• Best diagnostic clue: Disrupted alignment, reconstruction of CT slices, isotropic reformations
relationships of posterior arch in adjacent vertebral
levels
Radiographic Findings I DIFFERENTIAL DIAGNOSIS
• Radiography Multicolumn fractures with element of
o Fracture lines through laminae, facets, or spinous
process posterior column involvement
o Malaligned posterior elements • Flexion-extension Fx
• Flexion-rotation Fx
• Extension-rotation Fx
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• Jumped facets
ISELECTED REFERENCES
1. Maiman D] et al: Preinjury cervical alignment affecting
IPATHOLOGY 2.
spinal trauma.] Neurosurg. 97(1 Suppl):57-62, 2002
Matar LD et al: "Spinolaminar breach": an important sign
General Features in cervical spinous process fractures. Skeletal Radial.
29(2):75-80, 2000
• General path comments 3. Brady WJ et al: ED use of flexion-extension cervical spine
o Tear of ligaments bridging spinous processes + radiography in the evaluation of blunt trauma. Am] Emerg
laminae Med. 17(6):504-8, 1999
o Fractures of laminae, facets, or spinous processes 4. Zhu Q et al: Traumatic instabilities of the cervical spine
o Posterior column fracture thought to be generally caused by high-speed axial compression in a human
stable, as anterior/middle columns intact and model. An in vitro biomechanical study. Spine.
prevent subluxation 24(5):440-4, 1999
• Exception: If capsular ligaments torn, facets 5. Plezbert]A et al: Fracture of a lamina in the cervical spine.]
Manipulative Physiol Ther. 17(8):552-7, 1994
and/or laminae both fractured, rotational 6. Goldberg AL et al: Hyperextension injuries of the cervical
instability may exist spine. Magnetic resonance findings. Skeletal Radiol.
• Associated abnormalities: May have associated 18(4):283-8, 1989
disruption of middle or anterior column, which results 7. Denis, F: The three column spine and its significance in the
in instability classification of acute thoracolumbar spinal injuries. Spine.
8(8):817-31, 1983
ICLINICAL ISSUES
IIMAGE GALLERY
Presentation
• Most common signs/symptoms
o Neck pain
o Other signs/symptoms
• Neurological disturbance
• Clinical profile: Post-traumatic neck pain and loss of
mobility
Treatment
• Options, risks, complications
o Immobilization
o Surgical fusion
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ANTERIOR ·COMPRESSION<EiRACTI,JRE, THORACIC
1
50
Sagittal graphic shows 2 compression fractures. Most Sagittal graphic shows 2 addiUonal types of compression
common type is compression of superior endplate. fracture. Open arrow shows common type involving
Compression of inferior endplate alone is rare. both endplates. Arrow shows rare type with coronally
oriented cleft.
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ANTERIOR COMPRESSION FRACTURE, THORACIC
Key Facts
1
Terminology • Scheuermann kyphosis 51
• Vertebral body fracture compressing anterior cortex, • Physiologic vertebral wedging
sparing middle/posterior columns Pathology
Imaging Findings • Axial load with or without flexion component
• Best diagnostic clue: Wedge-shaped vertebral body • Two distinct populations: Major trauma, insufficiency
• May occur at multiple levels, contiguous or fracture
noncontiguous Clinical Issues
• May extend to posterior cortex of vertebral body • Patients with normal bone mineral density heal well
• Fractures of posterior elements absent with conservative management
• Best imaging tool: CT best to differentiate from • Osteoporotic patients may have progreSSive fracture
Chance, burst fractures
Diagnostic Checklist
Top Differential Diagnoses • Compression fracture involving inferior endplate
• Compression-distraction injury (Chance fracture) with normal superior endplate raises suspicion for
• Burst fracture pathologic fracture
• Pathologic fracture due to tumor
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ANTERIOR COMPRESSION FRACTURE, THORACIC
1 • Associated abnormalities patients: gUidelines for diagnosis and imaging. Injury.
34(6):426-33,2003
o Other spine fractures, contiguous or not contiguous
52 o Fractures of the pelvis + lower extremities 5. Haba H et al: Diagnostic accuracy of magnetic resonance
imaging for detecting posterior ligamentous complex
injury associated with thoracic and lumbar fractures. ]
Neurosurg. 99(1 Suppl):20-6, 2003
ICLINICAL ISSUES 6. Phillips FM: Minimally invasive treatments of osteoporotic
vertebral compression fractures. Spine. 28(15):S45-53, 2003
Presentation 7. Sheridan R et al: Reformatted visceral protocol helical
• Most common signs/symptoms computed tomographic scanning allows conventional
o Acute trauma with focal back pain radiographs of the thoracic and lumbar spine to be
o Insidious back pain in elderly patients eliminated in the evaluation of blunt trauma patients. ]
o Other signs/symptoms Trauma. 55(4):665-9, 2003
• Radiculopathy 8. Hiwatashi A et al: Increase in vertebral body height after
vertebroplasty. A]NR Am] Neuroradiol. 24(2):185-9, 2003
• Kyphotic deformity 9. Sapkas GS et al: Thoracic spinal injuries: operative
Demographics treatments and neurologic outcomes. Am] Orthop.
32(2):85-8, 2003
• Age 10. Wintermark M et al: Thoracolumbar spine fractures in
o Bimodal patients who have sustained severe trauma: depiction with
o Young trauma patients and multi-detector row CT. Radiology. 227(3):681-9, 2003
o Elderly osteoporotic patients 11. Haba H et al: Diagnostic accuracy of magnetic resonance
imaging for detecting posterior ligamentous complex
Natural History & Prognosis injury associated with thoracic and lumbar fractures. ]
• Patients with normal bone mineral density heal well Neurosurg. 99(1 Suppl):20-6, 2003
with conservative management 12. Wittenberg RH et al: Noncontiguous unstable spine
• Increased risk of premature degenerative disc disease fractures. Spine. 27(3):254-7, 2002
in young patients 13. Zhou X] et al: Characterization of benign and metastatic
vertebral compression fractures with quantitative diffusion
• Osteoporotic patients may have progressive fracture MR imaging. A]NR Am] Neuroradiol. 23(1):165-70, 2002
o Chronic pain 14. Lane]M et al: Minimally invasive options for the
• Kyphotic deformity often progressive in osteoporotic treatment of osteoporotic vertebral compression fractures.
patients Orthop Clin North Am. 33(2):431-8, viii, 2002
• Patients with 1 osteoporotic compression fracture at 15. Baur A et al: Acute osteoporotic and neoplastic vertebral
increased risk for other fractures compression fractures: fluid sign at MR imaging.
• May have delayed onset of neurologic symptoms Radiology. 225(3):730-5, 2002
16. Robertson A et al: Spinal injury patterns resulting from car
Treatment and motorcycle accidents. Spine. 27(24):2825-30, 2002
• Options, risks, complications: Conservative 17. Parisini P et al: Treatment of spinal fractures in children
and adolescents: long-term results in 44 patients. Spine.
management usually successful 27(18):1989-94, 2002
• Vertebroplasty & kyphoplasty used for chronic pain, 18. Robertson A et al: Spinal injury patterns resulting from car
kyphotic deformity and motorcycle accidents. Spine. 27(24):2825-30, 2002
o Usually affords immediate pain relief 19. Parisini P et al: Treatment of spinal fractures in children
o May increase risk of compression fractures of and adolescents: long-term results in 44 patients. Spine.
adjacent vertebrae 27(18):1989-94, 2002
• Bisphosphonates, calcitonin decrease pain & risk of 20. O'Connor PA et al: Spinal cord injury following
further osteoporotic fracture osteoporotic vertebral fracture: case report. Spine.
27(18):E413-5, 2002
21. Lindsay R et al: Risk of new vertebral fracture in the year
following a fracture. ]AMA. 285(3):320-3, 2001
IDIAGNOSTIC CHECKLIST 22. Holmes]F et al: Epidemiology of thoracolumbar spine
injury in blunt trauma. Acad Emerg Med. 8(9):866-72, 2001
Image Interpretation Pearls 23. Vaccaro AR et al: Post-traumatic spinal deformity. Spine.
• Compression fracture involving inferior endplate with 26(24 Suppl):Sl11-8, 2001
normal superior endplate raises suspicion for 24. van Beek E] et al: Upper thoracic spinal fractures in trauma
pathologic fracture patients - a diagnostic pitfall. Injury. 31(4):219-23, 2000
25. Kerttula LI et al: Post-traumatic findings of the spine after
earlier vertebral fracture in young patients: clinical and
MRI study. Spine. 25(9):1104-8, 2000
ISELECTED REFERENCES 26. Rechtine GR 2nd et al: Treatment of thoracolumbar
1. Folman Y et al: Late outcome of nonoperative trauma: comparison of complications of operative versus
management of thoracolumbar vertebral wedge fractures. ] nonoperative treatment.] Spinal Disord. 12(5):406-9, 1999
Orthop Trauma. 17(3):190-2, 2003
2. Wintermark M et al: Thoracolumbar spine fractures in
patients who have sustained severe trauma: depiction with
multi-detector row CT. Radiology. 227(3):681-9, 2003
3. Naves M et al: The effect of vertebral fracture as a risk
factor for osteoporotic fracture and mortality in a Spanish
population. Osteoporos Int. 14(6):520-4,2003
4. Hsu]M et al: Thoracolumbar fracture in blunt trauma
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ANTERIOR COMPRESSION FRACTURE, THORACIC
I IMAGE GALLERY 1
53
Typical
(Left) Anteroposterior
fluoroscopy shows widening
of paraspinous line (arrow)
due to hematoma from
compression fracture.
Scoliosis was due to muscle
spasm. (Right) Coronal bone
CT shows mild endplate
depression and linear bands
of sclerosis at T7 0 and T7 1
(arrows) from mild
compression fractures at
both levels. Normal
trabecular pattern excludes
tumor.
Typical
(Left) Sagittal T7 WI M R
shows cup-shaped
depression (arrow) of
superior vertebral cortex in
osteoporotic patient. Low
signal intensity fracture line is
immediately beneath cortex.
(Right) Sagittal STIR MR
shows T72 burst (arrow)
compared to T7 0, T7 1
compressions (open arrows).
Note band-like configuration
of edema, & preserved
posterior cortices of
compression fractures.
Variant
(Left) Lateral radiography
shows T3 compression
fracture (arrow). Fractures at
this level are difficult to see
on routine radiography due
to overlap from shoulders, +
swimmer's view is needed.
(Right) Sagittal STIR MR
shows chronic compression
fracture. Bone marrow
edema is absent.
Transversely oriented callus
(arrow) is very low signal
intensity. Focal kyphosis is
present.
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LATERAL COMPRESSIONFRJ\CTURE, THORACIC
1
54
Coronal graphic illustrates a right lateral thoracic Coronal bone CT reformat reveals right lateral T5
compression fracture. Note associated endplate injuries compression & left lateral T6 compression fractures.
to both vertebral bodies above + below. Posterior vertebral body & facets were intact (not
shown).
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LATERAL COMPRESSION FRACTURE, THORACIC
Key Facts
1
Terminology • Thoracic VCF may be result of single event trauma or 55
• Thoracic vertebral body Fx with! vertebral height, ongoing chronic microfractures
predominantly lateral location • 11% of VCF are lateral
• Tl C+ IPATHOLOGY
o Both benign + malignant processes may enhance!
o Paraspinal, epidural enhancing tissue suggests General Features
malignant pathology • General path comments
• MRS: Vertebral body percent fat fraction is o Anatomic-pathologic correlation
significantly i in weakened bone controls • Thoracic cage provides i structural stability to TS
Nuclear Medicine Findings • Kyphosis & extensive overlap of articulating facets
• Bone Scan: i Activity on bone scan highly predictive limits extension injuries
of positive outcome after vertebroplasty • Trauma forces translate into flexion vector
• Fluorine-18 deoxyglucose (FDG) PET • Results in wedging or compression deformities
o No i uptake in acute vertebral fractures originating • Posterior elements, ligaments often remain intact
from osteoporosis or preclinical osteoporosis o VCF occur when combined axial & bending loads
o High FDG uptake is characteristic for malignant & exceed strength of vertebral body
inflammatory processes o Lateral TS VCF correlated with seat belt injury
o FDG-PET may have potential value differentiating • Genetics
osteoporotic from pathologic VCF o Inherited bone disorders (Le., NFl, Gaucher)
o Vitamin D receptor polymorphism may influence
Imaging Recommendations bone mineral density & osteoporotic
• Best imaging tool • Etiology
o CT > > radiography o Typically result of fleXion, axial forces, or both
o MRI for neurologic deficit(s) o Trauma, osteopenia, metabolic bone disease, genetic
• Protocol advice disorders, neoplasm (primary or metastatic)
oCT: Sagittal & coronal reformats • Metastatic vertebral lesions account for 39% of
o MRI: STIR & fat-saturation Tl C+ bony mets in patients with primary neoplasms
• Myeloma appears similar to benign VCF
• Benign vertebral lesions occur in approximately
IDIFFERENTIAL DIAGNOSIS 1/3 of cancer patients
o Seat belt injury
Compression Fx • Result of shoulder-lap belt assemblies
• Anterior without lateral component • Results in anterolateral wedge compression of
thoracolumbar vertebra
Chance Fx ("seat belt Fx")
• Lateral compression occms on side opposite
• Horizontal splitting of spinous process, laminae, restrained shoulder
pedicles, superior vertebral body • Posterior elements may be disrupted contralateral
• Disc and facet distraction with ligament disruption to anterolateral body compression
Burst Fx • Postulated mechanism, referred to as "roll-out
• Abnormal posterior vertebral body wall phenomenon" = flexion & rotation about axis of
• Fragment retropulsion into spinal canal shoulder strap
o Thoracic VCF may be result of single event trauma
or ongOing chronic microfractures
• Epidemiology
o 11% of VCF are lateral
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Typical
(Left) Anteroposterior
radiography shows right
lateral T5 compression
fracture (arrows) which was
verified with CT imaging.
(Right) Axial bone CT
demonstrates right lateral
circumferential arc of bone
fragments from thoracic
lateral compression fracture.
Note intact posterior
vertebral body & facets.
Typical
(Left) Coronal bone CT
reformat demonstrates left
lateral thoracic compression
fracture. posterior vertebral
body & facets were intact
(not shown). (Right) Axial
bone CT shows left lateral
circumferential arc of bone
fragments from thoracic
lateral compression fracture.
Note intact posterior
vertebral body & pedicles.
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BURST THORACOLUMBAR FRACTURE
1
58
Lateral radiography shows wedge compression of L1 Axial bone CT, same case as on left, shows comminuted
vertebral body. Pedicles were widened on AP view. vertebral body Fxs, outward rotation of pedicles,
Patient had jumped off garage roof, experienced neck retropulsed fragment not seen on x-ray.
and lower back pain.
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Key Facts
1
Terminology • Widened pedicles on anteroposterior view 59
• Unstable compression fracture (Fx) • Wedge-shaped vb on lateral v view
• Comminuted fracture of vertebral body (vb) • May see malalignment
extending through both superior and inferior • Posterior displacement of bone into canal
endplates • Low signal within wedged vb
• High signal within disrupted vb, surrounding soft
Imaging Findings tissue
• Location: Typically mid- or lower thoracic spine, • May see cord contusion
upper lumbar spine
IPATHOLOGY
ISELECTED REFERENCES
1. Verlaan JJ et al: Operative compared with nonoperative
General Features treatment of a thoracolumbar burst fracture without
neurological deficit. J Bone Joint Surg Am. 86-A(3):649-50;
• General path comments: Typically due to vertical force author reply 650-1, 2004
trauma (jumping, landing on buttock) 2. Kim NH et al: Neurologic injury and recovery in patients
• Etiology: Trauma with burst fracture of the thoracolumbar spine. Spine.
• Associated abnormalities 24(3):290-3; discussion 294, 1999
o Epidural hematoma 3. Saifuddin A et al: The role of imaging in the diagnosis and
o Cord contusion management of thoracolumbar burst fractures: current
concepts and a review of the literature. Skeletal Radiol.
Gross Pathologic & Surgical Features 25(7):603-13, 1996
• Disrupted vb 4. McAffee PC et al: The unstable burst fracture. Spine 7:
• Column instability 365-73, 1982
5. Holdsworth FW: Fractures, dislocations, and
Microscopic Features fracture-dislocations of the spine. J Bone and Joint Surg Br.
• Microtrabecular disruption, hemorrhage 45: 6-20, 1963
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FACET-LAMINAFR.J\CTURE,.THORACIC
1
60
Axial bone CT shows comminuted fractures involving a Axial bone CT with sagittal reformation shows fractures
thoracic vertebral body and its posterior elements. through vertebral body & lamina (arrow).
Septic Facet Arthritis Septic Facet Arthritis Septic Facet Arthritis Metastases
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FACET-LAMINA FRACTURE, THORACIC
Key Facts
1
Terminology • Paraspinal and intraspinal hematoma 61
• Neural (vertebral) arch fracture • Thin-section bone CT most effective in characterizing
posterior column fractures
Imaging Findings • MRI best for cord evaluation
• Best diagnostic clue: Cortical disruption/discontinuity • Sagittal reformation from axial CT
through laminae, facet joints of thoracic vertebrae • Sagittal STIR or T2WI with fat suppression for
• 60% of thoracolumbar fractures occur between T12 posterior ligamentous injury
and L2
• Widened paraspinalline
Top Differential Diagnoses
• Increased interpediculate distance • Septic facet arthritis and osteomyelitis
• Facet subluxation/dislocation • Pathologic fracture
• Vertebral body comminution Diagnostic Checklist
• Vertebral subluxation/dislocation • Look for paraspinal soft tissue hematoma as clue to
• Hyperintense marrow edema spinal fractures on CT
• Posterior soft tissue edema
• Cord edema
• Retropulsed bony fragments within spinal canal • Demonstrates extent of canal compromise
o Vertebral subluxation/dislocation • Shows horizontal fractures through posterior
• "Double body" sign: Overlapping vertebrae on elements
axial imaging o Sagittal STIR or T2WI with fat suppression for
posterior ligamentous injury
MR Findings
• TlWI
o Acute intraspinal hemorrhage hyperintense II?IFFERENTIAL DIAGNOSIS
o Hypointense fracture lines
o Separated osseous fragments Septic facet arthritis and osteomyelitis
• T2WI • Marrow edema in facet articular processes & adjacent
o Hyperintense marrow edema laminae on MRI
o Posterior soft tissue edema • Fluid intensity within facet joint
• Ligamentous tear with widened interspinous gap • Surrounding soft tissue edema, enhancement and
o Cord edema abscess
• Swelling
• Compression Pathologic fracture
• Distraction • Underlying lytic or sclerotic lesion on CT
• STIR: Hyperintense marrow edema accentuated • Bony expansion
• Herniated disc • Soft tissue mass
• Paraspinal and intraspinal hematoma • More commonly associated with vertebral body
Ultrasonographic Findings
• Disrupted supraspinous and interspinous ligaments I PATHOLOGY
o Difficult to visualize between TlO and T12 due to
overlapping spinous processes General Features
Nuclear Medicine Findings • Etiology
o Motor vehicle accidents
• Bone Scan o Falls
o Focal increased uptake of radiotracer in posterior o Sports
elements o Penetrating injuries
• Limited role in acute trauma: Positive 24 hours • Epidemiology
after fracture o Prevalence of thoracolumbar injuries
• Useful in occult injuries with chronic back pain • ApproXimately 6% in blunt trauma patients
Imaging Recommendations • Associated abnormalities
o Intracranial hemorrhage
• Best imaging tool
o Thin-section bone CT most effective in o Other fractures
characterizing posterior column fractures o Injury to visceral organs
o MRI best for cord evaluation o Vascular trauma
• Must be included in imaging workup of patients • Anatomy
with neurologic deficit o Neural (vertebral) arch
• Protocol advice • Pedicles, laminae, articular processes, base of
o Sagittal reformation from axial CT spinous process
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FACET-LAMINA FRACTURE, THORACIC
1 o Posterior ligamentous complex
• Ligamentum flavum, facet joint capsule,
o Incomplete cord injury may achieve neurologic
recovery
62 interspinous ligament, supraspinous ligament
o Three column concept of thoracic spine Treatment
• Anterior column: Anterior longitudinal ligament • Initial IV high-dose steroids if cord injury present
(ALL), anterior one half of vertebral body and • Conservative treatment if mechanically stable
annulus fibrosis o One or two column injuries
• Middle column: Posterior longitudinal ligament o Stabilization with bracing
(PLL), posterior one half of vertebral body and o Early mobilization
annulus fibrosis • Surgery to prevent instability and deformity, preserve
• Posterior column: Neural arch, facet joints, neural function
spinous process, transverse process, ligamentous o Indicated in three column injuries, kyphosis> 20
complex degrees, and progressive neurologic deficits
• Mechanism of injury o Anterior and/or posterior fusion
o Neural arch often fractured in following o Spinal decompression may be indicated
mechanisms
• Extension
• Flexion-distraction: Horizontal fracture through IDIAGNOSTIC CHECKLIST
vertebral body, PLL, posterior elements
Consider
• Flexion-rotation: Ruptured posterior ligamentous
complex, fractures, dislocation • Use bone windows to evaluate entire spine on total
body trauma CT
• Shear: All ligaments disrupted with listhesis in all
three directions o Retro-reconstruction to thinner collimation if spinal
o Neural arch usually not involved in follOWing fractures detected
• Sagittal and coronal reformation through area of
mechanisms
interest
• Flexion: Posterior ligaments may rupture
o Evaluate spinal canal for hematoma & cord
• Compression: Except in severe burst fractures
compromise
• 75% of thoracolumbar fractures
Staging, Grading or Classification Criteria Image Interpretation Pearls
• Mechanical stability of fractures depend on number of • Look for paraspinal soft tissue hematoma as clue to
columns involved spinal fractures on CT
• Stable: One or two columns injured
o Anterior column: Compression fracture
o Anterior and middle columns: Most burst fractures ISELECTED REFERENCES
o Middle and posterior columns: Chance 1. Sheridan R et al: Reformatted visceral protocol helical
flexion-distraction injury computed tomographic scanning allows conventional
• Unstable: Three columns involved radiographs of the thoracic and lumbar spine to be
eliminated in the evaluation of blunt trauma patients. J
o Some burst and Chance fractures
Trauma. 55(4):665-9, 2003
o All fracture dislocations 2. Haba H et al: Diagnostic accuracy of magnetic resonance
imaging for detecting posterior ligamentous complex
injury associated with thoracic and lumbar fractures. J
ICLINICAL ISSUES Neurosurg. 99(1 Suppl):20-6, 2003
Wintermark M et al: Thoracolumbar spine fractures in
3.
Presentation patients who have sustained severe trauma: depiction with
• Most common signs/symptoms multi-detector row CT. Radiology. 227(3):681-9, 2003
o Back pain 4. Sapkas GS et al: Thoracic spinal injuries: operative
treatments and neurologic outcomes. Am J Orthop.
o Neurologic deficits 32(2):85-8, 2003
o Other signs/symptom 5. Moon SH et al: Feasibility of ultrasound examination in
• Multisystemic injury posterior ligament complex injury of thoracolumbar spine
fracture. Spine. 27(19):2154-8, 2002
Demographics 6. Trivedi JM: Spinal trauma: therapy--options and outcomes.
• Age: Mean age between 25-40 years Eur J Radiol. 42(2):127-34, 2002
• Gender 7. Holmes JF et al: Epidemiology of thoracolumbar spine
o M:F ratio injury in blunt trauma. Acad Emerg Med. 8(9):866-72, 2001
• 9:1 in motorcycle accidents 8. Denis F: Spinal instability as defined by the three-column
• 3:2 in car accidents spine concept in acute spinal trauma. Clin Orthop.
(189):65-76, 1984
• Ethnicity: No race predilection
9. Denis F: The three column spine and its significance in the
Natural History & Prognosis classification of acute thoracolumbar spinal injuries. Spine.
8(8):817-31,1983
• Residual neurologic deficits in 15-20% of
thoracolumbar trauma
o Complete paraplegia at time of injury rarely
improves despite therapy
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FACET-LAMINA FRACTURE, THORACIC
IIMAGE GALLERY 1
63
Typical
(Left) Axial bone CT with
sagittal reformation shows
horizontal fracture through
thoracic vertebral body,
pedicle, and lamina (Chance
flexion-distraction injury).
(Right) Sagittal T2WI MR
with fat suppression shows
ruptured posterior
ligamentous complex
(arrow). There is marrow
edema with mild anterior
wedging in vertebral body.
Typical
(Left) Sagittal T2WI MR with
fat suppression shows
comminuted vertebral
fractures. Retropulsed bony
fragments severely narrow
central canal. (Right) Axial
T2WI MR shows
comminuted vertebral body
& arch fractures with central
canal stenosis.
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CHANCE FRACTURE, THORACIC
1
64
Sagittal graphic shows soft tissue Chance injury through Sagittal graphic shows bony Chance injury through
PLL, ligamentum flavum, facet joints (not shown) & vertebral body, pedicles (not shown) and spinous
interspinous ligaments, with cord contusion. process with cord contusion.
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CHANCE FRACTURE, THORACIC
Key Facts
1
Terminology • Distraction injury 65
• Flexion-distraction injury • Burst fracture
• Traumatic compression fracture
Imaging Findings • Osteoporotic compression fracture
• Best diagnostic clue: Wedging anterior vertebral body
+ increased interspinous distance or spinous process
Pathology
fracture • Less common than burst & compression fractures
• Usually occurs at Tll-L3 Clinical Issues
• Occasionally at mid-thoracic spine • Long term kyphotic deformity common without
• Disruption of posterior longitudinal ligament (PLL), surgical fusion
interspinous ligaments
• Anterior longitudinal ligament (ALL) usually intact Diagnostic Checklist
• More likely to be disrupted in midthoracic injuries • Consider Chance fracture whenever radiography
than lower thoracic shows severe compression fracture in patient with
normal bone density
Top Differential Diagnoses
• Shear injury
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CHANCE FRACTURE, THORACIC
1 • Usually see medial rib, transverse process fractures
Treatment
in mid-thoracic Chance
66 • Etiology • Options, risks, complications: Osteoligamentous &
o Motor vehicle accident or fall ligamentous injuries usually require spinal fusion
o Anterior compression, posterior distraction around
fulcrum
o Injury patterns depend on position of fulcrum IDIAGNOSTIC CHECKLIST
o Classic pattern: Lap seat belt
• Seat belt fulcrum anterior to vertebral column
Image Interpretation Pearls
• Compression anterior column, distraction • Consider Chance fracture whenever radiography
posterior column shows severe compression fracture in patient with
• May have compression failure of anterior column, normal bone density
or functionally intact column
o Midthoracic Chance fractures have fulcrum in
middle column ISELECTED REFERENCES
• Posterior column tension failure 1. Sapkas GS et al: Thoracic spinal injuries: operative
• Anterior column compression failure treatments and neurologic outcomes. Am] Orthop.
o Anterior longitudinal ligament (ALL) is usually 32(2):85-8, 2003
intact 2. Wintermark M et al: Thoracolumbar spine fractures in
patients who have sustained severe trauma: depiction with
• In severe fractures, ALL may be stripped from multi-detector row CT. Radiology. 227(3):681-9, 2003
vertebral body 3. Hsu]M et al: Thoracolumbar fracture in blunt trauma
• Epidemiology patients: guidelines for diagnosis and imaging. Injury.
o Less common than burst & compression fractures 34(6):426-33,2003
o Decreased prevalence after institution of automobile 4. Haba H et al: Diagnostic accuracy of magnetic resonance
shoulder belts imaging for detecting posterior ligamentous complex
• Associated abnormalities: 65% have significant injury associated with thoracic and lumbar fractures.]
abdominal injuries (bowel most common) Neurosurg. 99(1 Suppl):20-6, 2003
5. Sheridan R et al: Reformatted visceral protocol helical
Staging, Grading or Classification Criteria computed tomographic scanning allows conventional
radiographs of the thoracic and lumbar spine to be
• Osseous Chance fracture eliminated in the evaluation of blunt trauma patients. ]
o Fracture of vertebral body, Trauma. 55(4):665-9, 2003
o Posterior element fractures: Pedicles, transverse 6. Uu Y] et al: FleXion-distraction injury of the
processes, laminae, spinous process thoracolumbar spine. Injury. 34(12):920-3, 2003
• Ligamentous Chance injury 7. Bouliane M] et al: Instability resulting from a missed
o Intervertebral disc Chance fracture. Can] Surg. 44(1):61-2,2001
o Facet dislocation 8. Beaunoyer M et al: Abdominal injuries associated with
o Rupture interspinous ligaments thoraco-Iumbar fractures after motor vehicle collision. ]
Pediatr Surg. 36(5):760-2, 2001
• Osteoligamentous Chance injury 9. Levine OS et al: Chance fracture after pedicle screw
o Variable combination of fracture + ligament injury fixation. A case report. Spine. 23(3):382-5; discussion 386,
1998
10. Greenwald TA et al: Pediatric seatbelt injuries: diagnosis
ICLINICAL ISSUES and treatment of lumbar flexion-distraction injuries.
Paraplegia. 32(11):743-51, 1994
Presentation 11. Anderson PA et al: Flexion distraction and chance injuries
• Most common signs/symptoms to the thoracolumbar spine.] Orthop Trauma. 5(2):153-60,
o Back pain folloWing high speed injury 1991
12. Reid AB et al: Pediatric Chance fractures: association with
o Other signs/symptoms intra-abdominal injuries and seatbelt use. ] Trauma.
• Neurologic injury may be present 30(4):384-91, 1990
• Clinical profile 13. Denis F: Spinal instability as defined by the three-column
o High speed motor vehicle accident spine concept in acute spinal trauma. Clin Orthop.
o Fall (189):65-76, 1984
o Has been reported through pedicle screw at lower 14. Rogers LF: The roentgenographic appearance of transverse
level of surgical fusion or chance fractures of the spine: the seat belt fracture. Am]
Roentgenol Radium Ther Nucl Med. 111(4):844-9, 1971
Natural History & Prognosis
• Long term kyphotic deformity common without
surgical fusion
• Osseous injury
o Acutely unstable
o May require fixation if reduction cannot be
maintained
• Osteoligamentous, ligamentous injury
o Poor prognosis for healing unless fusion performed
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CHANCE FRACTURE, THORACIC
IIMAGE GALLERY 1
67
Typical
(Left) Lateral radiography
shows anterior compression
of T11 (arrow), widely
distracted fracture of pars
interarticularis (open arrow).
(Right) Anteroposterior
radiography shows loss of
vertebral body height at T11,
& horizontal fractures
through right pedicle
(arrow), left pars
interarticularis (open arrow).
Variant
(Left) Sagittal STIR MR
shows mid-thoracic Chance.
There are extensive
interspinous ligament injuries
(open arrows). ALL is
partially disrupted at fracture
site (arrow). (Right) Sagittal
T1WI MR shows 50% height
loss and diffuse low signal
intensity in T6 vertebra. ALL
is amorphous (arrow) &
attached to anterior vertebral
fragment.
Typical
(Left) Sagittal bone CT shows
comminuted fracture of
anlerior portion of T12
vertebral body (arrow), &
horizontal fracture through
spinous process (open
arrow). (Right) Coronal bone
CT shows T12 Chance
fracture extending through
right pars interarticularis &
laminae (arrows).
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DISTRACTION FX, LOW THORACIC
1
68
Sagittal graphic shows thoracic complex fracture with Sagittal bone CT reformation, intrathecal contrast,
anterior compression, posterior ligament disruption with shows T7 1-12 chance Fx with fragment off T7 2, anterior
distraction. subluxation T7 1/12, no caudal flow of contrast & canal
kink.
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Key Facts
1
Terminology Pathology 69
• Seat belt fracture; Chance fracture; flexion-distraction • Lap belt restraint of torso during violent deceleration
fracture in car accident
• Stabilizing ligaments (anterior, posterior longitudinal,
Imaging Findings capsular, ligamenta flavum) are torn with this
• Radiography: Anterior wedging of low thoracic or mechanism
upper lumbar vb with focal kyphosis, facet and vb • Up to 65% have intra-abdominal injury, especially
subluxation bowel
• Neurological damage in 30%
IDIAGNOSTIC CHECKLIST
Consider
• Always check for associated intra-abdominal damage (Left) Axial bone CT consecutive slice set shows block of intrathecal
contrast at TlI-12 Chance Fx. Note naked facets (arrows) of TIl,
Image Interpretation Pearls jumped anterior to Tl2 (open arrows) in paraplegic post MVA.
• > 15 degrees of kyphosis ~ indicates instability (Right) Sagittal bone CT shows jumped TI I- I 2 facets in lap-belted
MVA victim with paraplegia resulting from Chance Fx. Tl2 showed
anterior wedging on mid-sagittal view, which also showed kyphotic
canal kink.
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ANTERIOR COMPRESSION FRACTURE, LUMBAR
1
70
Lateral radiography shows compression fractures at T12 Sagittal bone CT shows mul6ple compression fractures
and L 1; normal bone mineral density. Note anterior with differing features: Cup-like depression L7 (curved
cortical break (arrow) and sclero6c trabecular arrow), sclero6c band L3 (open arrow), sclero6c line L5
compression (open arrow). (arrow).
ITERMINOlOGY CT Findings
Abbreviations and Synonyms • Bone CT
o Band of sclerosis due to trabecular impaction
• Wedge fracture, wedge compression fracture o Endplate depression
Definitions o +/- Stepoff anterior cortex
• Fracture of anterior cortex of vertebral body without o Rarely see extension of fracture to posterior cortex,
displacement of posterior wall or involvement of but posterior displacement absent
neural arch MR Findings
• TlWI: Low signal intensity, band-like or triangular
configuration
IIMAGING FINDINGS • T2WI
General Features o High signal intensity, band-like or triangular
configuration
• Best diagnostic clue: Loss of height of anterior portion
o Obscured by fatty marrow unless fat-saturation pulse
of vertebral body
used
• Location
o Trauma: Most common upper lumbar • STIR
o Greatest conspicuity of edema
o Osteoporosis: Any level
o Differentiate acute from old fractures
Radiographic Findings Imaging Recommendations
• Radiography • Best imaging tool
o Depression of vertebral endplate, usually superior
o Earliest diagnosis by MRI
only
o CT may better distinguish from pathologic fracture
• May be cup-shaped or angular
• Protocol advice: Sagittal TlWI, STIR
o +/- Stepoff anterior cortex
o Variant: Coronal fracture of vertebral body
o Abnormalities much more prominent on lateral
than anteroposterior radiography
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ANTERIOR COMPRESSION FRACTURE, LUMBAR
Key Facts
1
Terminology • Earliest diagnosis by MRI 71
• Fracture of anterior cortex of vertebral body without • CT may better distinguish from pathologic fracture
. displacement of posterior wall or involvement of Top Differential Diagnoses
neural arch • Chance fracture
Imaging Findings • Burst fracture
• Depression of vertebral endplate, usually superior • Pathologic fracture due to tumor
only • Physiologic wedging
• Variant: Coronal fracture of vertebral body • Scheuermann disease
ICLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Post-traumatic back pain
o Other signs/symptoms
• Insidious onset back pain
Natural History & Prognosis
• Heals well in young patients (Left) Lateral radiography shows compression fractures at L2 & L3
o Increased incidence disc degeneration from skiing accident. L3 vertebral fracture is less common pattern,
with vertical fracture (arrows) as well as endplate depression. (Right)
• May progress in osteoporotic patients Sagittal STIR MR shows compression fractures from Ti2 to L5.
Configuration of edema is band-like or triangular. There is cupping of
superior endplates but no discrete fracture lines.
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Lateral radiography shows L2 lateral compression Anteroposterior radiography confirms left lateral
fracture. The lateral nature is evident by one normal compression fracture of L2 (arrow).
superior end-plate (arrow) & one abnormal superior
endplate (open arrow).
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LATERAL COMPRESSION FRACTURE, LUMBAR
Key Facts
1
Terminology Pathology 73
• Lumbar vertebral body Fx with ~ vertebral height, • Lateral VCF of thoracolumbar spine is correlated with
predominantly lateral seat belt injury
• 11% of VCF are lateral
Imaging Findings • LS VCF is most common spine fracture, comprising
• Best diagnostic clue: Lateral wedge deformity of nearly 50% of all spine fractures
affected lumbar spine (LS) vertebral body
• Location: 50-65% VCF are between T12 & L2 Clinical Issues
• Arc of irregular bone fragments circumferentially • Many experience severe & prolonged pain that can
from vertebral body markedly alter activities of daily living
• Acute: STIR best visualizes hyperintense marrow • Main VCF risk factor is osteoporosis
edema which CAN extend into pedicles • Early intervention with vertebroplasty may be
• CT > > radiography indicated given low complication rates
Top Differential Diagnoses Diagnostic Checklist
• Burst Fx • VCF in elderly patient with unrelenting back pain
• Chance Fx ("seat belt Fx") • Up to 20% of VCF are multiple ~ screen entire spine!
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LATERAL COMPRESSION FRACTURE, LUMBAR
1 o Frequency of radiographic evidence of VCF increases
from 500/100,000 person years (py) in women aged
Complications reported include transitory fever,
o
transient worsening of pain, radiculopathy, rib Fx,
74 SO-54 yrs to 2,960/100,000 py in women> 85 yrs cement pulmonary embolism, infection, & spinal
o In the US, VCF account for 150,000 hospital cord compression
admissions, 161,000 physician office visits, & > 5 o With osteoporotic VCF, complication rate is 1-2%
million restricted activity days annually o With malignant tumors, the complication rate t 2°
• Associated abnormalities to cement leakage resulting from vertebral body
o Immobility makes more susceptible to pneumonia, destruction caused by the malignant process
deep vein thrombosis, & pulmonary embolism o MR is preferred for guiding vertebroplasty as it offers
o Patients with osteoporotic VCF have significantly anatomic detail and concurrent information about
lower vital capacity and forced expiratory volume other abnormalities (Le., spinal stenosis)
o If tumor is suspected, it may be appropriate to
Microscopic Features precede vertebroplasty with biopsy after the cannula
• Inflammatory phase: Hematoma, osteocytic death, is placed but before cement is injected
intense inflammatory response • Vertebroplasty cement presence does not hinder
• Reparative phase: Hematoma organized by the therapeutic result of subsequent or
fibrovascular tissue, callus forms on collagen matrix concomitant radiation or chemotherapy
• Remodeling phase: Resorption of unnecessary callus, • Surgery when neurologic dysfunction and/or
trabecular proliferation along lines of stress instability occurs as a result of VCF
• Vertebroplasty
o Direct injection of polymethylmethacrylate into
ICLINICAL ISSUES collapsed body
o Alleviates pain & prevents further collapse
Presentation • Achieves 90% efficacy in pain relief
• Many experience severe & prolonged pain that can o Highly efficacious regardless of Fx age, even with
markedly alter activities of daily living severe deformity (> 70%)
o 84% of VCF are painful, on average lasting 4-6 wks
o May see opacifying intravertebral clefts
o Subgroup of patients has subacute or chronic pain
o Antecedent venography does not improve
that is refractory to conservative therapy effectiveness or safety
• Numbness, tingling, & weakness may indicate nerve • Kyphoplasty = vertebroplasty variation in which an
compression inflatable balloon is first placed inside compressed
• May be asymptomatic vertebral body into which cement is instilled
Demographics
• Age IDIAGNOSTIC CHECKLIST
o Osteoporotic: t Frequency with age
o Traumatic: Usually younger patient population Consider
• Gender • VCF in elderly patient with unrelenting back pain
o M:F "" 2:1
o Yet male incidence is high contradicting common Image Interpretation Pearls
misconception that osteoporosis is women's health • up to 20% of VCF are multiple ~ screen entire spine!
problem
Natural History & Prognosis
• Main VCF risk factor is osteoporosis
ISELECTED REFERENCES
o Severity of osteoporosis is worsened by smoking, 1. lung HS et al: Discrimination of metastatic from acute
physical inactivity, use of prednisone & other osteoporotic compression spinal fractures with MR
imaging. Radiographies. 23(1):179-87, 2003
medications, poor nutrition
2. Schmitz A et al: FDG-PET findings of vertebral compression
o In males all above risk factors apply as well as low fractures in osteoporosis: preliminary results. Osteoporos
testosterone levels Int. 13(9):755-61, 2002
• Women with VCF have higher mortality rates as 3. Baur A et al: Acute osteoporotic and neoplastic vertebral
compared to age-matched control subjects without Fx compression fractures: fluid sign at MR imaging.
o Women with 1 or more VCF have a 1.23-fold greater Radiology. 225(3):730-5, 2002
age-adjusted mortality rate 4. Herneth AM et al: Vertebral metastases: assessment with
o Mortality rises as the number of VCF increases; ADC. Radiology. 225(3):889-94, 2002
women with 2: 5 Fxs have a > twofold increase 5. Kaufmann T] et al: Age of fracture and clinical outcomes of
percutaneous vertebroplasty. A]NR Am] Neuroradiol.
Treatment 22(10):1860-3, 2001
6. Miniaci A et al: Anterolateral compression fracture of the
• Conservative therapy usually consists of analgesic use, thoracolumbar spine. A seat belt injury. Clin Orthop.
rest, & external bracing (240):153-6, 1989
o Immobilization accelerates bone loss, which may
contribute to further VCF
• Early intervention with vertebroplasty may be
indicated given low complication rates
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LATERAL COMPRESSION FRACTURE, LUMBAR
IIMAGE GALLERY 1
75
Typical
(Left) Lateral radiography
shows L 1 lateral compression
Fx. (Right) Anteroposterior
radiography confirms right
lateral compression fracture
of L1 (arrow).
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• Vertically oriented
o May have compression fractures at adjacent levels Chance fracture
• Anterior vertebral body compression
CT Findings • Horizontal fractures posterior elements OR
• Bone CT • Separation of spinous processes
o Retropulsed bone fragments in spinal canal
• Percent narrowing of spinal canal should be Pathologic fracture due to tumor
reported • More commonly compression fracture, but sometimes
o Vertically oriented posterior element fractures burst fracture
o Widening interpediculate distance • Mass may be obscured by fracture and hematoma
• Can be diagnosed on routine CT scan of abdomen in • CT may show trabecular, cortical destruction
multitrauma patients • MR may show tumor at other levels
• Multidetector abdomen CT scan obViates need for Fracture-dislocation
dedicated spine CT
• Due to shear injury or distraction
o Expedites care of patients with multiple injuries
already undergoing abdomen CT • Vertebra displaced relative to vertebra above or below
o Thin slices can be fused for evaluation of abdomen,
unfused for spine
o Small field of view coronal and sagittal images I PATHOLOGY
reformatted from original data set General Features
MR Findings • General path comments
• T1WI: Visualization of retropulsed fragments o Position of vertebral fragments in canal on CT not
• T2WI true measure of displacement at time of injury
o Evaluate position of conus relative to fracture • Fragments tend to move forward after initial
o Evaluate cord contusion posterior excursion
• STIR: Cord abnormalities more conspicuous o Posterior element fracture may be greenstick fracture
• Posterior element fractures often difficult to see on all in adolescents
sequences o Degree of neurologic injury with lumbar burst
highly dependent on position of conus
Imaging Recommendations o Lower lumbar burst fractures may have minimal or
• Best imaging tool: CT for surgical planning no neurologic signs
• Protocol advice • Etiology
o 1-3 mm overlapping helical images o Most commonly due to falls from height
o Coronal, sagittal reformations o Twisting or lateral flexion component may be
present
• Epidemiology
IDIFFERENTIAL DIAGNOSIS o Construction workers
o Mountain climbers
Compression fracture o Sky divers
• Posterior vertebral body cortex intact o Skiers
• Posterior element fractures absent o Sometimes osteoporotic insufficiency fracture
• Less than 40-50% loss of height in patients with o Occasionally pathologic fracture due to tumor
normal bone density • Associated abnormalities
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ICLINICAL ISSUES 7.
treatment and follow-up. Eur Spine]. 12(3):255-60, 2003
Wood K et al: Operative compared with nonoperative
treatment of a thoracolumbar burst fracture without
Presentation neurological deficit. A prospective, randomized study. ]
• Most common signs/symptoms Bone Joint Surg Am. 85-A(5):773-81, 2003
o Traumatic back pain 8. Wintermark M et al: Thoracolumbar spine fractures in
o Other signs/symptoms patients who have sustained severe trauma: depiction with
multi-detector row CT. Radiology. 227(3):681-9, 2003
• Lower extremity neurologic deficits
9. Nguyen HV et al: Osteoporotic vertebral burst fractures
Natural History & Prognosis with neurologic compromise. ] Spinal Disord Tech.
16(1):10-9,2003
• Fragments in spinal canal remodel over time
10. Parisini P et al: Treatment of spinal fractures in children
o Canal narrowing decreases significantly in size and adolescents: long-term results in 44 patients. Spine.
within one year following injury 27(18):1989-94, 2002
o Best seen on CT scan, but can be evaluated on lateral 11. McLain RF et al: Segmental instrumentation for thoracic
radiography and thoracolumbar fractures: prospective analysis of
construct survival and five-year follow-up. Spine].
Treatment 1(5):310-23,2001
• Options, risks, complications 12. Aydinli U et al: Dural tears in lumbar burst fractures with
o Systemic steroids generally given at time of injury greenstick lamina fractures. Spine. 26(18):E410-5, 2001
o Conservative management for 13. Lalonde F et al: An analysis of burst fractures of the spine
in adolescents. Am] Orthop. 30(2):115-20, 2001
• Mild fractures 14. Dai LY: Remodeling of the spinal canal after
• Lower lumbar fractures thoracolumbar burst fractures. Clin Orthop. (382):119-23,
• Long term outcomes similar to fusion in 2001
neurologically intact patients 15. Kuklo TR et al: Measurement of thoracic and lumbar
o Laminectomy and posterior fusion used if there is fracture kyphosis: evaluation of intraobserver,
• Neurologic dysfunction interobserver, and technique variability. Spine. 26(1):61-5;
• Greater than 20-30 degree kyphosis discussion 66, 2001
• Subluxation of facet joints 16. Carl AL et al: Anterior dural laceration caused by
thoracolumbar and lumbar burst fractures. ] Spinal Disord.
• Greater than 50% loss of vertebral body height
13(5):399-403, 2000
IDIAGNOSTIC CHECKLIST
Consider
• High association with lower extremity & pelvis
fractures
Image Interpretation Pearls
• Plain radiographic diagnosis possible in most cases
o Look carefully at lateral radiograph for retropulsed
fragments
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Typical
(Left) Coronal bone CT
shows comminution of
vertebral body & loss of
height. Paraspinous
hematoma (arrows) is
evident. (Right) Coronal
bone CT shows widened
interpediculate distance
(arrows) at level of burst
fracture. Large fragments of
bone are present in spinal
canal (open arrow).
Variant
(Left) Coronal bone CT
shows burst fracture of L4
with right lateral
compression due to axial
load plus lateral flexion.
Fracture extends to both
superior and inferior
vertebral endplates. (Right)
Sagittal bone CT shows L4
burst fracture with superior
and inferior endplate
involvement and retropulsion
of inferior portion of
posterior cortex (arrow).
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Axial bone CT shows an L3 superior facet fracture Axial bone CT obtained two months later demonstrates
(arrow) in a young hockey player who experienced interval healing (arrow) following brace therapy and
sudden onset low back pain while playing (Courtesy abstaining from play (Courtesy William 0. Shaffer, MO).
W/lIiam 0. Shaffer, MO).
.~
£,,>
.
\f ~."-.. t'~
:~.
... ..'~l ~~'
.
"', ./
~J
'. \
\
Bilateral Pars Fx Bilateral Pars Fx Pars Fx Pars Fx
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,,""
• Clinical profile
~
o Athlete: Acute pain after inciting event, often
hidden out of desire to "keep playing" ./
o Post-Op: New pain pattern, local tenderness, pain on
unusual movements, relief with recumbency suggest
facet Fx versus recurrent disk herniation
Natural History & Prognosis (Left) Axial bone CT demonstrates bilateral superior lumbar facet
• Majority heal with conservative therapy fractures (arrows). (Right) Sagittal bone CT reformat shows lumbar
superior facet fracture (arrow).
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Anteroposterior radiography shows vertically oriented Anteroposterior radiography shows zone 2 fracture due
fracture through zone 1 of the sacrum (arrows). to lateral compression. Note angular deformity of
Mechanism of injury was anteroposterior force or arcuate lines on left (arrows) compared to normal right
open-book. side (open arrows).
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SACRAL TRAUMATIC FRACTURE
1 o Vertical shear (fall from a height)
• Usually vertically oriented fracture
o Posterior pelVic pain or tenderness follOWing high
velocity trauma
84 • Vertical displacement may be present or may have o Other signs/symptoms
spontaneously reduced • Neurologic deficit
• Fractures of L5 transverse processes sometimes • Bowel, bladder symptoms
present
• Epidemiology: Common injury Natural History & Prognosis
• Associated abnormalities • Unstable injuries require open reduction internal
o Lumbosacral junction injury fixation (ORIF)
• Seen in up to 1/3 of sacral fractures with pelVic • Neurologic injury
ring involvement o Sl, S2 deficits tend to improve within 1 year
• Facet joint injury or L5-S1 disc injury regardless of treatment
o Fracture of L5 transverse process indicates unstable o Dural laceration may lead to cerebrospinal fluid leak
fracture Treatment
• Fracture due to avulsion of lumbosacral ligament • Options, risks, complications
o Fractures anterior pelvic ring
o Bed rest, slow progression to weightbearing if stable
o Lumbar spine fractures o Stabilization of unstable fractures with transalar
• Spine fracture with neurologic deficit may cause screws
sacral fracture to be overlooked clinically
o Role of surgical decompression controversial
o Neurologic injuries in 40% of displaced fractures • Neurologic recovery improved with early
• Nerve roots or sacral plexus compared to late decompression
• Transection or stretch injury o Neural canal or neural foraminal obstruction may be
o Vascular injuries removed
• Therapeutic embolization may be needed o Significant post-operative infection rate
• Active bleeding often seen on contrast-enhanced
CT scan at time of trauma
o Injury bladder or urethra
o Lower extremity fractures
I DIAGNOSTIC CHECKLIST
o Degloving injury of posterior soft tissues Image Interpretation Pearls
Staging, Grading or Classification Criteria • High velocity injuries in adults always involve anterior
pelvic ring when sacrum is fractured
• Denis classification
o Divides fracture by involvement of 3 different zones • If see L5 transverse process fracture, suspect sacral fx
• Zone 1: Lateral to neural foramina
o 50% of sacral fractures
o Vertical or oblique orientation ISELECTED REFERENCES
o Neurologic deficit in 6-24% 1. Bellabarba C et al: Midline sagittal sacral fractures in
o L5 nerve root may be entrapped in fracture anterior-posterior compression pelvic ring injuries. ]
• Zone 2: Through neural foramina Orthop Trauma. 17(1):32-7, 2003
2. Rubel IF et al: Description of a rare type of posterior pelvis
o 34% of sacral fractures traumatic involvement: the green-stick fracture of the
o Vertical or oblique sarcum. Pediatr Radiol. 31(6):447-9, 2001
o Neurologic deficit in 28% 3. Verlaan JJ et al: Traumatic lumbosacral dislocation: case
o Traumatic "far out syndrome" report. Spine. 26(17):1942-4, 2001
• L5 root caught between sacral alar fragment and 4. Kim MY et al: Transverse sacral fractures: case series and
transverse process literature review. Can] Surg. 44(5):359-63, 2001
• Zone 3: Through spinal canal 5. Leone A et al: Lumbosacral junction injury associated with
o 16% of sacral fractures unstable pelVic fracture: classification and diagnosis.
Radiology. 205(1):253-9, 1997
o Transverse zone 3 fracture 6. Templeman D et al: Internal fixation of displaced fractures
• 35% have associated avulsion of nerve roots of the sacrum. Clin Orthop. (329):180-5, 1996
• "Sacral burst fracture": Transverse fracture with 7. Rodriguez-Fuentes AE: Traumatic sacrolisthesis 51-52.
retropulsion of bone into spinal canal Report of a case. Spine. 18(6):768-71, 1993
• Fracture-dislocation may occur through 8. Albert T] et al: Concomitant noncontiguous thoracolumbar
rudimentary disc at Sl-S2 and sacral fractures. Spine. 18(10):1285-91, 1993
• Neurologic deficit in 57-60% 9. Gibbons K] et al: Neurological injury and patterns of sacral
• May have fracture below lumbosacral fusion fractures.] Neurosurg. 72(6):889-93, 1990
10. Denis F et al: Sacral fractures: an important problem.
o Vertical zone 3 fracture Retrospective analysis of 236 cases. Clin Orthop.
• Majority have neurologic deficit 227:67-81,1988
• Bowel, bladder, sexual dysfunction
ICLINICAL ISSUES
Presentation
• Most common signs/symptoms
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SACRAL TRAUMATIC FRACTURE
IIMAGE GALLERY 1
85
(Left) Anteroposterior
radiography shows vertical
shear zone 2 fracture
through 51 & 52 neural
foramina (arrows). L5
transverse process fracture
(open arrow) indicates
pelvic instability. (Right)
Axial bone CT shows
extension of sacral fracture
into 51 neural foramen
(arrow).
Variant
(Left) Lateral radiography
shows displaced traumatic
fracture occurring at 5112
level (arrows), below spinal
fusion. This fracture can also
occur as an insufficiency
fracture. (Right) Sagittal
bone CT shows traumatic
fracture (arrows) at 57152
level, below fusion. Fracture
is through rudimentary disc
at this level.
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SACRAL INSUFFICIENCY FRACTURE
1
86
Sagittal graphic depicts the characterisUc "H" shaped Coronal TlWI MR shows bilateral (L > R) ill-defined
sacral insufficiency fractures, with bilateral vertical sacral sacral marrow hypointensities adjacent to sacroiliac
alar and horizontal upper sacral fractures. joints.
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•, .
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. ~""I' .
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o CT coronal reformation
MR Findings
• Tl WI: Hypointense linear or ill-defined marrow signal
• T2WI: Hyperintense linear or ill-defined marrow signal I DIFFERENTIAL DIAGNOSIS
• STIR: Patchy hyperintense marrow
• Tl C+: Curvilinear fracture lines better delineated Sacral metastases
• Sacral marrow edema • More discrete in morphology
o Unilateral or bilateral • Random distribution
o Ill-defined and irregular • Invasion of adjacent soft tissue and neural foramina
o Adjacent to sacroiliac joints • Other sites involved
• Not involving or crossing sacroiliac joints
o Seen within three weeks after symptom onset Primary sacral neoplasm
o Resolves after three months • Large and solitary
o Associated cortical disruption • Bony expansion
• Presence of discrete fracture lines • Cortical breakthrough
o Usually visualized between three weeks and three • Soft tissue invasion
months
o Parallel to sacroiliac joints
Sacral osteomyelitis
• Lack of bony expansion • Cutaneous and subcutaneous abnormalities present
o Soft tissue edema
• Absent soft tissue mass
o Abscess
• Intact sacroiliac joints
• Sensitive and specific • Ill-defined marrow edema
• May be overlooked on MRI of lumbar spine o Contiguous with subcutaneous abnormality
o If no coronal sequence o Extending across sacroiliac joints
o If only limited axial imaging through sacrum o Associated with sacroiliitis
• No fracture lines
Nuclear Medicine Findings
• Bone Scan
Sacroiliitis
o Bilateral or unilateral sacral radiotracer uptake with • Signal alteration within sacroiliac joints
or without a horizontal component • Erosions at joints
o Characteristic "H" shaped pattern of radiotracer • Joint ankylosis
uptake only present in 19% of the cases • Marrow edema also present on iliac side of sacroiliac
o Sensitive but not specific joints
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SACRAL .liNSlJFFICIENC¥FRACTURE
1 • Etiology
o Predisposing factors Natural History & Prognosis
88 • Osteoporosis • Favorable outcome and good prognosis
• Rheumatoid arthritis o Symptoms resolve in as early as one month
• Renal osteodystrophy • Usually within 6-9 months
• Endogenous or exogenous corticosteroid excess • May be limited by co-morbidities
• Radiation therapy Treatment
• Other causes of osteopenia
• Conservative
• Paget disease o Bed rest
o Minor trauma
o Protected weight bearing
• Often elicited after the diagnosis o Analgesics
o No precipitating event in many instances
o Physical therapy
• Epidemiology o Treatment of underlying osteoporosis
o Incidence 0.14-1.8%
• Sacroplasty
o More common in women
o Injection of polymethylmethacrylate
• Associated abnormalities
o Vertebral compression fractures
o Other pelvic insufficiency fractures
• Acetabular roof
I DIAGNOSTIC CHECKLIST
• Pubic rami Consider
• Parasymphysial pubic bone • Including coronal T1WI on all lumbar spine MRI
Microscopic Features Image Interpretation Pearls
• Organizing hematoma • Make sure to evaluate sacrum on lumbar spine MRI
• Trabecular condensation • Consider sacral insufficiency fractures when marrow
• New bone and cartilage matrix edema adjacent to sacral iliac joints present
• Remodeling by osteoblasts and osteoclasts
I SELECTED REFERENCES
I CLINICAL ISSUES
1. Pommersheim W et al: Sacroplasty: a treatment for sacral
Presentation insufficiency fractures. A]NR Am] Neuroradiol.
24(5):1003-7, 2003
• Most common signs/symptoms 2. White]H et al: Imaging of sacral fractures. Clin Radiol.
o Acute or subacute pain 58(12):914-21,2003
• Lowback 3. Dasgupta B et al: Sacral insufficiency fractures: an
• Buttock unsuspected cause of low back pain. Br] Rheumatol.
• Hip 37(7):789-93, 1998
• Groin 4. Peh WC et al: Vacuum phenomena in the sacroiliac joints
• Often in multiple sites and in association with sacral insufficiency fractures.
Incidence and significance. Spine. 22(17):2005-8, 1997
o Sacral tenderness
5. Grangier C et al: Role of MRI in the diagnosis of
o Radiculopathy insufficiency fractures of the sacrum and acetabular roof.
o Signs and symptoms mimic multiple medical Skeletal Radio 26:517-24, 1997
conditions 6. Grasland A et al: Sacral insufficiency fractures: an easily
• Degenerative disc disease overlooked cause of back pain in elderly women. Arch
• Spinal stenosis Intern Med 156:668-74, 1996
• Vertebral compression fracture 7. Peh WC et al: Imaging of pelvic insufficiency
• Hip arthritis fractures. Radiographies 16:335-48, 1996
8. Stabler A et al: Vacuum phenomena in insufficiency
• Metastases fractures of the sacrum. Skeletal Radiology 24:31-35, 1995
o High clinical suspicion required to make the 9. Peh WC et al: Sacral insufficiency fractures. Spectrum of
diagnosis radiological features. Clin Imaging. 19(2):92-101, 1995
• Women with postmenopausal osteoporosis 10. Baker R]]r et al: Sacral insufficiency fracture: half of an "H".
• Elderly men with senile osteoporosis Clin Nucl Med. 19(12):1106-7, 1994
• Clinical profile 11. West SG et al: Sacral insufficiency fractures in rheumatoid
o Pain exacerbated by activity arthritis. Spine. 19(18):2117-21, 1994
o Relieved by rest 12. Blomlie V et al: Radiation-induced insufficiency fractures
of the sacrum: evaluation with MR imaging.. Radiology.
Demographics 188(1):241-4, 1993
• Age: Sixth through eighth decades of life 13. Weber M et al: Insufficiency fractures of the sacrum.
Twenty cases and review of the literature. Spine.
• Gender 18(16):2507-12, 1993
o Elderly men 14. Kayes Ket al: Radiologic case study. Sacral insufficiency
o Postmenopausal women fracture. Orthopedics. 14(7):817-8,820,1991
• Ethnicity: No racial predilection
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SACRAL INSUFFICIENCY FRACTURE
IIMAGE GALLERY 1
89
Typical
(Left) Coronal T2WI MR
shows bilateral (L > R) sacral
marrow edema with
left-sided cortical disrupdon
(arrow). Bilateral sacroiliac
joints are intact. (Right)
Coronal bone scan shows
characteristic "H" pattern of
radiotracer uptake in
sacrum.
Typical
(Left) Axial TlWI MR shows
left-sided sacral marrow
edema extending to, but not
involving, sacroiliac joint.
Anterior cortical disruption is
present (arrow). (Right) Axial
T1 C+ MR shows bilateral
sacral marrow enhancement
with associated subtle
cortical disruptions (arrows),
sparring sacroiliac joints.
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TRAUMATIC DISC HERNIATION
1
90
Sagittal T1WI MR shows C5-6 flexion injury with Sagittal T1WI MR shows C5-6 flexion injury with disc
subluxation, prevertebral hemorrhage (arrow), disc extrusion compressing cord with cord hemorrhage &
extrusion touching ventral cord (open arrow). edema.
• Myelography
ITERMINOLOGY o Extradural mass effacing anterior CSF-filled
Abbreviations and Synonyms subarachnoid space
o May see associated cord flattening
• Herniated nucleus pulposus (HNP)
o Non-standard terminology o Distortion or obliteration of adjacent nerve root
• Traumatic disc herniation (tHNP) sleeve(s)
• Spinal cord injury (SCI) CT Findings
Definitions • NECT
• Disc herniation induced by trauma o Soft tissue density projecting into spinal canal
o Obliteration of adjacent epidural fat
o Narrowing of the disc space
IIMAGING FINDINGS • CTA: Quick screening for traumatic vascular injury
• Bone CT
General Features o tHNP often obscured by associated fractures,
• Best diagnostic clue: Herniation of disc material most epidural hematoma
evident on T2WI o May also see locked facets, joint subluxation, Fxs
• Location MR Findings
o Cervical spine most frequently
o 50% of tHNP occur at level of injury or one level • TlWI
o Disc material effacing anterior CSF perhaps
below/above
compressing cord or cauda equina
• Size: Small to large
• Young: Hydrated mid-intensity disc material
• Morphology: Appearance is the same as disc
• Older: Desiccated hypointense material
herniation unrelated to trauma
• T2WI
Radiographic Findings o Disc material effacing anterior CSF perhaps
• Radiography compressing cord or cauda equina
o Insensitive for disc pathology • Young: Hydrated hyperintense disc material, may
o Associated traumatic bone injuries be more hyperintense than normal
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TRAUMATIC DISC HERNIATION
Key Facts
1
Terminology Pathology 91
• Disc herniation induced by trauma .. Pre-existing disc degeneration likely
• Epidemiology: Seen in 3-9% of all SCI patients,
Imaging Findings 33-48% of unstable cervical spine injuries
• Best diagnostic clue: Herniation of disc material most
evident on T2WI Clinical Issues
• 50% of tHNP occur at level of injury or one level • Radicular syndrome (pain & segmental neurologic
below/above deficit) ("" 65%)
• Morphology: Appearance is the same as disc • Myeloradiculopathy ("" 30%)
herniation unrelated to trauma • Medullary symptoms ("" 7%)
• MRI is modality of choice • Anterior cord syndrome is highly associated with
• CT Bone depicts associated osseous injuries better traumatic disc herniation
• Good prognosis: Radiculopathy
Top Differential Diagnoses • Poor prognosis: Myelopathy
• Nontraumatic HNP
• Epidural abscess, phlegmon Diagnostic Checklist
• Epidural tumor • tHNP in any patient who fails closed reduction
• Older: Desiccated hypointense material o Discs allow movement between vertebral bodies &
o Hyperintense cord contusion from compression transmit loads from one vertebral body to the next
o Stripping of posterior longitudinal ligament o Annulus & nucleus disperse axial load pressure
• STIR: Associated ligamentous or soft tissue injury o When an axial load is applied to nucleus it shortens
• T2* GRE & radially expands exerting pressure on annulus
o Susceptibility from hemorrhage o Annular resistance opposes this outward pressure
o Thin-section for cervical spine foraminal evaluation o Axial loads up to 40 kg cause only 1 mm of vertical
• MRA: Quick screening for traumatic vascular injury compression & 0.5 mm of radial expansion
o During movement the annulus acts as ligament
Angiographic Findings restraining movement & partially stabilizing
• Conventional: Replaced by CTA, MRA interbody joint
Imaging Recommendations • Genetics
o Collagen type II gene deficiency, vitamin D receptor
• Best imaging tool
o MRI is modality of choice intragenic polymorphism both map to same region
o CT Bone depicts associated osseous injuries better on chromosome 12q13
• Protocol advice • Strong association with disc degeneration &
o Best seen with sagittal T2WI herniation
o Add GRE to assess for cord hemorrhage • Established for nontraumatic HNP, however most
o STIR for ligamentous & soft tissue injury agree that pre-existing disc degeneration
predisposes to tHNP
• Thus same genes likely play some role in tHNP
I DIFFERENTIAL DIAGNOSIS • Etiology
o Trauma-induced disruption of annulus ~ nucleus
Nontraumatic HNP pulposus herniation
• Disc herniation with little or no trauma o Pre-existing disc degeneration likely
• Appears similar to tHNP on imaging • Epidemiology: Seen in 3-9% of all SCI patients,
33-48% of unstable cervical spine injuries
Epidural abscess, phlegmon • Associated abnormalities
• Usually from discitis o Bone Fxs, subluxations, dislocations
• Not as focal, may extend> 1 spinal segment o Cord contusion, hemorrhage
• Majority enhances, unlike disc material o Ligamentous, soft tissue injury
o Brain injury
Epidural tumor
• Usually back pain without trauma history Microscopic Features
• Often enhances diffusely • Fibrovascular tissue surrounds herniated disc material
• Look for extension from adjacent bony metastasis • Vessels extend into annulus fibrosus but not endplate
• Scattered cartilage fragments & macrophages localize
around disc margin
!PATHOlOGY • Findings are similar to degenerated herniated discs &
suggest an absorptive process
General Features
• General path comments
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TRAUMATI(} DISC HERNIATION
1 Staging, Grading or Classification Criteria
o There is no practice standard
• Surgical treatment often in association with repair of
92 • Herniation = presence of nuclear material within or associated injuries
beyond the confines of an annular tear
o Protrusion = focal or asymmetric disc extension
beyond the interspace with a broader base than any
other dimension of protrusion
IDIAGNOSTIC CHECKLIST
o Extrusion = focal or asymmetric disc extension Consider
beyond the interspace with base of origin narrower • tHNP in any patient who fails closed reduction
than the diameter of extruding material itself
o Free fragment = herniation (extrusion) that has no
connection with original disc
• Modifiers
ISELECTED REFERENCES
1. Martinez-Lage JF et al: Lumbar disc herniation in early
o Contained = restricted beneath an intact posterior childhood: case report and literature review. Childs Nerv
longitudinal ligament Syst. 19(4):258-60,2003
o Noncontained = herniation extends through 2. Tohme-Noun C et al: Imaging features of traumatic
defective posterior longitudinal ligament dislocation of the lumbosacral joint associated with disc
herniation. Skeletal Radiol. 32(6):360-3, 2003
3. Hayes KC et al: Retropulsion of intervertebral discs
I CLINICAL ISSUES associated with traumatic hyperextension of the cervical
spine and absence of vertebral fracture: an uncommon
Presentation mechanism of spinal cord injury. Spinal Cord.
40(10):544-7,2002
• Most common signs/symptoms 4. Hadley MN: Initial closed reduction of cervical spine
o Radicular syndrome (pain & segmental neurologic fracture-dislocation injuries. Neurosurgery. 50(3
deficit) ("" 65%) Suppl):S44-50, 2002
o Myeloradiculopathy ("" 30%) 5. Zortea M et al: Genetic mapping of a susceptibility locus
o Medullary symptoms ("" 7%) for disc herniation and spastic paraplegia on 6q23.3-q24.1.
o Anterior cord syndrome is highly associated with J Med Genet. 39(6):387-90, 2002
traumatic disc herniation 6. Fuentes S et al: Traumatic thoracic disc herniation. Case
illustration. J Neurosurg. 95(2 Suppl):276, 2001
• Injury to anterior 2/3 of cord
7. Gray L et al: Thoracic and lumbar spine trauma. Semin
• Pain & temperature affected Ultrasound CT MR. 22(2):125-34, 2001
• Touch & vibration intact 8. Dai L et al: Central cord injury complicating acute cervical
o Brown-Sequard syndrome disc herniation in trauma. Spine. 25(3):331-5; discussion
• Injury to 1/2 cord 336,2000
• Ipsilateral loss of touch & motor function 9. Lee JY et al: Histological study of lumbar intervertebral disc
• Contralateral loss of pain & temperature herniation in adolescents. Acta Neurochir (Wien).
• Often superimposed on central cord syndrome 142(10):1107-10,2000
o Central cord syndrome 10. Benedetti PF et al: MRI findings in spinal ligamentous
injury. AJRAmJ Roentgenol. 175(3):661-5,2000
• Upper extremities involvement> lower 11. Katzberg RW et al: Acute cervical spine injuries: prospective
• Older population, seen with spondylosis w/o Fx MR imaging assessment at a level 1 trauma center.
• Historically associated with central hematoma, Radiology. 213(1):203-12, 1999
predominantly white matter injury 12. Vaccaro AR et al: Magnetic resonance evaluation of the
o Cauda equina syndrome: Pelvic & sacral pain due to intervertebral disc, spinal ligaments, and spinal cord before
compression of cauda equina spinal nerve roots and after closed traction reduction of cervical spine
• Clinical profile: SCI patient, often with numerous dislocations. Spine. 24(12):1210-7, 1999
injuries 13. Bucciero A et al: Myeloradicular damage in traumatic
cervical disc herniation. J Neurosurg Sci. 42(4):203-11,
Demographics 1998
14. Rumana CS et al: Brown-Sequard syndrome produced by
• Age: SCI: Mean age = 30.5 yrs, median = 26 yrs; 50% cervical disc herniation: case report and literature review.
are 16-30 Surg Neurol. 45(4):359-61, 1996
• Gender: SCI: > 80% male 15. Carreon LY et al: Histologic changes in the disc after
• Ethnicity: SCI: More common in non-Caucasians cervical spine trauma: evidence of disc absorption. J Spinal
• Timing of SCI Disord. 9(4):313-6, 1996
o Highest rate in July, lowest in February 16. Doran SE et al: Magnetic resonance imaging
o 39% occur on Saturday or Sunday documentation of coexistent traumatic locked facets of the
cervical spine and disc herniation. J Neurosurg.
Natural History & Prognosis 79(3):341-5, 1993
• Good prognosis: Radiculopathy
• Poor prognosis: Myelopathy
Treatment
• A number of large clinical series have failed to
establish a relationship between tHNP & subsequent
neurological deterioration with attempted closed
traction-reduction in awake patients
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TRAUMATIC DISC HERNIATION
IIMAGE GALLERY 1
93
Typical
(Left) Sagittal TlWI MR
demonstrates large L4/5
traumatic disc herniation as
an extruded fragment
posterior to L4 vertebral
body (arrow). (Right) Axial
T2WI MR confirms L4/5
traumatic disc herniation
(arrow).
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1
94
Sagittal graphic of young adult shows acute apophyseal Sagittal bone CT reformation (L) and source axial cut
ring fracture involving posterior inferior corner with (R) show sclerosed anterior margin of superior end plate
displacement, associated hemorrhage & thecal sac with truncated cornet; fragments displaced anteriorly
compression. (arrows).
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APOPHYSEAL RING FRACTURE
IIMAGE GALLERY 1
97
Typical
(Left) Sagittal bone CT
reformation shows small
bone fragment displaced
posteriorly from truncated
superior S 7 endplate. Note
sclerosis of donor site, &
associated disc protrusion.
(Right) Axial bone CT shows
concentric bony rim
displaced from superior
margin of S 7, with sclerosis
of donor site.
Typical
(Left) Sagittal FLAIR MR
demonstrates edema
subjacent to a distorted
anterosuperior vb margin,
typical of a subacute Limbus
L5 injury. Note more chronic
appearance of Limbus vb
two levels above (arrow).
(Right) Sagittal TI WI MR
shows two limbus vb (LJ,
L5). The lower one
demonstrates subjacent
edema seen as low signal.
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RHABDOMYOLYSIS
1
98
Axial T2WI MR shows diffuse hyperintensity of dorsal Axial T7 C+ MR shows diffuse and irregular muscle
right paraspinal muscles with sharp ventral margin. enhancement (arrows), with central nonenhancing
Patient was in prolonged right decubitus position for component.
surgery
ITERMINOLOGY MR Findings
• TlWI: Isointense to hypointense Tl signal from
Abbreviations and Synonyms
affected muscle groups
• Rhabdomyolysis (RML), myonecrosis • T2WI: Diffuse increased T2 signal with margins
Definitions confined by facial boundaries
• Clinical and biochemical syndrome resulting from • STIR: Increased T2 signal
damage of integrity of skeletal muscle, with release of • Tl C+
toxic muscle cell components into circulation a May show patchy contrast enhancement
a Severe disease with myonecrosis shows peripheral
enhancement with no central enhancement
IIMAGING FINDINGS Nuclear Medicine Findings
General Features • Bone Scan
a Bone scan shows widespread, diffuse accumulation
• Best diagnostic clue: Increased T2 signal within
of the radioisotope in the affected muscles
affected skeletal muscle group
a Tc99-MDP 95% sensitive in early active stage (first
• Location: Skeletal muscle
10 days)
• Size: Large: May involve multiple paraspinal muscle
groups . Imaging Recommendations
• Morphology: Diffuse abnormal signal • Best imaging tool
CT Findings a Bone scan to total body extent of damage
a MRI for extent of focal muscle damage
• NECT • Protocol advice: T2WI in multiple planes
a Normal to slight low attenuation by CT
a Skeletal muscle calcification may occur early in
disease course
• CECT: Normal to slight enhancement
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RHABDOMYOLYSIS
Key Facts
1
Terminology Pathology 99
• Rhabdomyolysis (RML), myonecrosis • Common pathway of RML may be a disturbance in
• Clinical and biochemical syndrome resulting from myocyte calcium homeostasis
damage of integrity of skeletal muscle, with release of • Elevated serum creatine kinase (CK) 5x normal value,
toxic muscle cell components into circulation 100% sensitive
• Genetic etiologies include glycolytic enzyme
Imaging Findings deficiencies (McArdle disease)
• Best diagnostic clue: Increased T2 signal within • Major etiologies include trauma (38%), including
affected skeletal muscle group prolonged immobilization for surgical procedures,
• T1WI: Isointense to hypointense T1 signal from prolonged seizures, direct muscle injury
affected muscle groups
• T2WI: Diffuse increased T2 signal with margins Clinical Issues
confined by facial boundaries • Hydration with isotonic crystalloid
• Bone scan shows widespread, diffuse accumulation of • Treatment of acute renal failure (mannitol, diuretics)
the radioisotope in the affected muscles • Life threatening complications include acute renal
failure, hyperkalemia and cardiac arrest
• Episodes of hyperthermia/rhabdomyolysis
IDIFFERENTIAL DIAGNOSIS triggered by exposure to volatile anesthetics
Hemorrhage/hematoma o Neuroleptic malignant syndrome
• Tl WI MR: Darker than fat • Drug related development of hyperthermia,
• T2WI MR: Brighter than fat muscle rigidity, fluctuating consciousness, and
• Surrounding edema autonomic instability
• Phenothiazines, butyrophenones, other
Infection/abscess antipsychotics/antidepressants
• Reticular to circumscribed subcutaneous edema • Underlying defect may be presynaptic
• Peripheral enhancement with abscess • Etiology
o Multiple etiologies
Myositis ossificans • Major etiologies include trauma (38%), including
• Localized soft tissue ossifications with circumscribed prolonged immobilization for surgical procedures,
pattern prolonged seizures, direct muscle injury
Soft tissue tumor • Ischemia (14%), including compression and
vascular occlusion
• Heterogeneous enhancement
• Polymyositis (8%)
• Prominent vessels
• Drug overdose (7%), and wide variety of drugs
• Bone sclerosis/periosteal reaction (including statins)
• Exertion (6%), marathon running
IPATHOLOGY • Burns
• Infections, both bacterial and viral
General Features • Wide variety of toxins, including alcohol
• General path comments • Metabolic disorders such as diabetic ketoacidosis
o Common pathway of RML may be a disturbance in and hypothyroidism
myocyte calcium homeostasis o Accumulation of cell lysis products =} microvascular
o Elevated serum creatine kinase (CK) 5x normal damage, capillary leak, increased
value, 100% sensitive intracompartmental pressures, reduced tissue
• CK-MM isoenzyme predominates perfusion and ischaemia
o Urine + hemoglobin/myoglobin, negative does not • Epidemiology: 25 cases per 2.5 million population
exclude diagnosis Gross Pathologic & Surgical Features
• "Coke colored" urine • Swelling, edema of muscle groups
• Genetics
o Multiple genetic predispositions Microscopic Features
o Genetic etiologies include glycolytic enzyme • Disruption of myofibrillar architecture and wide
deficiencies (McArdle disease) spread vacuolization
• Autosomal recessive • Late: Atrophy
o Abnormal lipid metabolism (carnitine deficiency)
o Malignant hyperthermia Staging, Grading or Classification Criteria
• Autosomal dominance with variable penetrance • Risk factors for acute renal failure due to RML
• Defect in receptor of calcium release channel of o Presence of sepsis
sarcoplasmic reticulum o Age> 70
o Volume depletion
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RHABDOMYOlYSIS
1 o Initial uric acid levels elevated
7.
noninfectious causes. South Med]. 95(5): 542-4, 2002
Bocca G et al: Compartment syndrome, rhabdomyolysis
o Degree of elevation of CPK, potassium, phosphorus
100 and risk of acute renal failure as complications of the
lithotomy position. J Nephro!. 15(2): 183-5, 2002
ICLINICAL ISSUES 8. WexlerRK: Evaluation and treatment of heat-related
illnesses. Am Fam Physician. 65(11): 2307-14, 2002
9. Criner JA et al: Rhabdomyolysis: the hidden killer. Medsurg
Presentation Nurs. 11(3): 138-43, ISS, 2002
• Most common signs/symptoms 10. Lappa A et al: Successful treatment of a complicated case of
o Muscle pain (50%) neuroleptic malignant syndrome. Intensive Care Med.
o Acute renal failure with myoglobinuria (30%) 28(7): 976-7, 2002
o Disseminated intravascular coagulation (DIe) near 11. Evans M et al: The myotoxicity of statins. Curr Opin
universal finding, although rarely symptomatic Lipido!. 13(4): 415-20, 2002
12. Atmaca H et al: Rhabdomyolysis associated with
o Metabolic acidosis
gemfibrozil-colchicine therapy. Ann Pharmacother. 36(11):
o Hyperkalemia 1719-21,2002
o Hypovolemia 13. Kikuno N et al: Traumatic rhabdomyolysis resulting from
o Other signs/symptoms continuous compression in the exaggerated lithotomy
• Tense muscle compartment may be compartment position for radical perineal prostatectomy. Int J Uro!. 9(9):
syndrome 521-4,2002
• Clinical profile: Muscle pain with tense swollen 14. Kuang Wet al: Rhabdomyolysis after laparoscopic donor
compartment following local trauma nephrectomy. Urology. 60(5): 911, 2002
15. Bolego C et al: Safety considerations for statins. Curr Opin
Demographics Lipido!. 13(6): 637-44, 2002
16. ModiJR et al: Fluvastatin-induced rhabdomyolysis. Ann
• Age: Any age group Pharmacother. 36(12): 1870-4, 2002
• Gender: M > F 17. Kratz A et al: Effect of marathon running on hematologic
Natural History & Prognosis and biochemical laboratory parameters, including cardiac
markers. Am J Clin Patho!. 118(6): 856-63, 2002
• 5% mortality rate 18. Black C et al: Etiology and frequency of rhabdomyolysis.
Pharmacotherapy. 22(12): 1524-6, 2002
Treatment
19. Barahona MJ et al: Hypothyroidism as a cause of
• Options, risks, complications rhabdomyolysis. Endocr]. 49(6): 621-3,2002
o Hydration with isotonic crystalloid
o Treatment of acute renal failure (mannitol, diuretics)
• Life threatening complications include acute renal
failure, hyperkalemia and cardiac arrest
IDIAGNOSTIC CHECKLIST
Consider
• Requires a high index of suspicion
• Clinical and laboratory findings give diagnosis, with
imaging secondary role
• Statins cause < < 1 death/million users due to
rhabdomyolysis
Image Interpretation Pearls
• Imaging findings nonspecific
• Clinical history, lab values critical
ISELECTED REFERENCES
1. Getachew E et al: Pathologic quiz case: a man with
exertion-induced cramps and myoglobinuria. McArdle
disease (glycogenosis type V or myophosphorylase
deficiency). Arch Pathol Lab Med. 127(9):1227-8,2003
2. Torres-Villalobos G et al: Pressure-induced rhabdomyolysis
after bariatric surgery. Obes Surg. 13(2):297-301, 2003
3. Wiltshire JP et al: Lumbar muscle rhabdomyolysis as a
cause of acute renal failure after Roux-en-Y gastric bypass.
Obes Surg. 13(2):306-13, 2003
4. Kisakol G et al: Rhabdomyolysis in a patient with
hypothyroidism. Endocr]. 50(2):221-3, 2003
5. Masaoka S et al: Malignant lymphoma in skeletal muscle
with rhabdomyolysis: a report of two cases. J Orthop Sci.
7(6): 688-93, 2002
6. Blanco JR et al: Rhabdomyolysis of infectious and
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RHABDOMYOLYSIS
IIMAGE GALLERY 1
Typical 101
Typical
(Left) Axial NECT shows
enlargement of the right
paraspinal muscles, without
abnormal attenuation. There
is irregular reticulation of
subcutaneous fat due to
edema. (Right) Coronal T1
C+ MR shows irregular
enhancement of right dorsal
paraspinal muscles (arrows),
in contrast to normal left
muscle signal intensity.
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Parasagittal STIR shows splenius capitis muscle belly Parasagittal STIR shows diffuse paraspinal lumbar
contusion & associated fascial injury (arrows). Also note muscle hyperintense strain (arrows) with hypointense
prevertebral tissue & facet ligament injuries (open hemorrhage within muscle fiber disruption (open
arrows). arrow).
o Strain:
ITERMINOLOGY • Grade 1 & 2: Edema +/- blood; initially center of
Definitions affected muscle - entire muscle involvement
• Strain = muscle fiber disruption from indirect forces • Grade 3: Edema & muscle discontinuity, may form
• Sprain = ligamentous injury hematoma
• Tl C+: May see enhancement acutely
Imaging Recommendations
IIMAGING FINDINGS • Best imaging tool: MRI
General Features • Protocol advice: T2 + fat suppressing technique
• Best diagnostic clue: Muscle T2 hyperintensity
• Morphology: Laceration, contusion, strain
IDIFFERENTIAL DIAGNOSIS
CT Findings
• NECT: Contusion: May form dense myositis ossificaIl-s
Denervation-related muscle atrophy
• Acutely appears similar to injury
MR Findings • Unlike injury becomes fatty replaced
• TlWI Neoplasm, inflammation, or infection
o Contusion: 1 Circumference
o Laceration: Muscle defect, often transverse, usually • Appear similar to acute injury
with hemorrhage • Fails to resolve with time
o Strain: Grade 3: Muscle discontinuity with
hemorrhage at torn edge, may form hematoma
o Muscle may herniate through fascial rent IPATHOLOGY
• STIR General Features
o Contusion: Hyperintense edema
o Contusion: Feathery interstitial pattern from fluid • Etiology
o Most commonly from MVA; also athletic injuries,
tracking between muscle fibers
blow from falling objects, direct injury
o Laceration: Edema, hemorrhage
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o Mechanism • Rhabdomyolysis
• Hyperextension> hyperflexion, lateral flexion o Fever, intense pain, weakness
• Excessive stretch and/or tension o Myoglobulin may induce renal failure
o Contusion
• Muscle compression from direct trauma Treatment
• Common in contact sports (aka "charleyhorse") • Mobilization & activity leads to less scar in healing
• Muscle can still function, even with severe injury contusion with more rapid recovery of tensile strength
o Laceration: Result of penetrating trauma • As healing progresses, rehabilitation activity may
o Strain: Injury from single violent force become more aggressive
• Epidemiology • Soft collar may be used for no more than 2-4 wks
o Lacerations are rare • Brief therapy with analgesics +/- muscle relaxants
o Contusions more frequent
• Associated abnormalities: TMJ, cervical nerve-root,
esophageal, tracheal, vascular injuries ISELECTED REFERENCES
1. Jinkins JR: Lumbosacral interspinous ligament rupture
Microscopic Features associated with acute intrinsic spinal muscle degeneration.
• Contusion: Hematoma formation, inflammation, JBR-BTR. 86(4):226-30, 2003
connective tissue scar replacing muscle fibers 2. Hayashi N et al: Accuracy of abnormal paraspinal muscle
• Laceration repair: Dense connective tissue, not normal findings on contrast-enhanced MR images as indirect signs
muscle fibers, with j ability to generate tension of unilateral cervical root-avulsion injury. Radiology.
• Strain: Muscle fiber disruption 223(2):397-402,2002
3. Vaccaro AR et al: Magnetic resonance imaging analysis of
o Usually myotendinous junction (weakest aspect)
soft tissue disruption after flexion-distraction injuries of
o Often accompanied by hemorrhage, hematoma the subaxial cervical spine. Spine. 26(17):1866-72, 2001
o Damaged muscle fiber necrosis & breakdown
• Proliferation of inflammatory cells
• Inelastic scar predisposes to recurrent injury
o May form myositis ossificans dUring later healing
IIMAGE GALLERY
Staging, Grading or Classification Criteria ..
T14~~
• Strain
o 1st degree: Microscopic muscle fiber disruption;
similar appearance to contusion
Presentation (Left) Sagittal STIR shows splenius capitis strain (arrow) containing
• Most common signs/symptoms: Pain; symptoms may fiber disruption (not shown). Also note interspinous/supraspinous
ligament (open arrows) and prevertebral injuries (curved arrow).
be delayed 2-3 days
(Right) Parasagittal STIR reveals diffuse hyperintense muscle injury
• Clinical profile: Palpation often reveals spasm including trapezius (white arrow), spleniUS capitis, & other muscles.
Natural History & Prognosis There are also diffuse fascial, ligament, & subcutaneous injuries.
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Axial graphic of lumbar body shows right pedicle Axial NEeT shows well-defined coronal oriented
screws, altering biomechanics to produce left pedicle fracture through right pedicle (arrow), with bone
stress fracture. production reflecting a chronic lesion. There is an old
appearing fracture of left lamina.
ITERMINOlOGY CT Findings
Abbreviations and Synonyms • NECT
o Coronal oriented fracture through pedicle
• Pedicle stress fracture (PSF) o Associated bony sclerosis
• Pediculolysis • +/- Association with adjacent sclerosis
Definitions • Sclerosis may be extensive, & mimic more
• Fracture of spinal pedicle not related to direct acute aggressive lesion
trauma o Look for contralateral posterior element abnormality
• Pedicle fracture
• Spondylolysis
IIMAGING fiNDINGS • Pedicle aplasia, hypoplasia
MR Findings
General Features
• Best diagnostic clue: Linear low attenuation on CT • TlWI
o Coronal or vertically oriented linear low signal
through pedicle, well-defined margins through pedicle
• Location: Lumbar pedicle> cervical> thoracic o May show larger area of low signal in pedicle due to
• Size: Variable fracture size through pedicle, from
bony sclerosis
partial to complete with separation o Contralateral posterior element abnormality
• Morphology o Osteophyte and bony hyperplasia may complicate
o Linear fracture, vertically oriented diagnosis by masking fracture site
o May be associated with sclerosis, hyperostosis o Variable pedicle marrow signal
Radiographic Findings • Low pedicle signal on TlWI due to sclerosis or
• Radiography edema
o Linear fracture through pedicle • Chronic lesions may show fatty Tl marrow signal
o Adjacent bony sclerosis in pedicle adjacent to fracture site
• T2WI
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Key Facts
1
Terminology • Chronic lesions may show fatty Tl marrow signal in 105
• Pedicle stress fracture (PSF) pedicle adjacent to fracture site
• Pediculolysis Pathology
• Fracture of spinal pedicle not related to direct acute • Etiology: Altered biomechanics leads to stress fracture
trauma
Clinical Issues
Imaging Findings • Back pain, radiculopathy if associated bony
• Best diagnostic clue: Linear low attenuation on CT hypertrophy and nerve compression
through pedicle, well-defined margins • Symptomatic treatment for pain
• Coronal oriented fracture through pedicle • Surgical pedicle screw fixation of fracture
• Look for contralateral posterior element abnormality
• Osteophyte and bony hyperplasia may complicate Diagnostic Checklist
diagnosis by masking fracture site • Look for contralateral posterior element disease such
• Variable pedicle marrow signal as pedicle hypoplasia, or aplasia
• Low pedicle signal on TlWI due to sclerosis or edema • Look for causative abnormality above or below
fracture site
o Coronal or vertically oriented linear low signal • Enhancement of soft tissue and peripheral
through pedicle enhancement of epidural abscess
o High signal intensity in adjacent pedicle due to
edema, repair Paget disease
• STIR • Bone expansion with thickened cortex
o Coronal or vertically oriented linear low signal • No bone destruction
through pedicle • Thickened trabeculae, heterogeneous marrow signal
o High signal intensity in adjacent pedicle due to may simulate fracture site on MR
edema, repair Pseudoarthrosis
• T2* GRE: Precise fracture site may be better defined on
• Horizontal (axial) directed defect through prior
gradient echo images
surgical fusion site
• Tl C+: Patchy adjacent reactive marrow enhancement
• Pseudoarthrosis involving intervertebral disc level and
Nuclear Medicine Findings posterior elements in a horizontal plane
• Bone Scan
o Increased uptake from fracture site
o Increased uptake from associated abnormalities such !PATHOLOGY
as contralateral spondylolysis
General Features
Imaging Recommendations • General path comments: Fracture with new bone
• Best imaging tool: CT shows fracture, displacement, production, callus
contralateral abnormality • Etiology: Altered biomechanics leads to stress fracture
• Protocol advice: Thin slice CT with multiplanar • Epidemiology
reformats o Related to prior spinal fusion surgery
o Remote trauma may also be involved
• Associated abnormalities: Prior spinal fusion, with or
IDIFFERENTIAL DIAGNOSIS without instrumentation
Metastatic disease Gross Pathologic & Surgical Features
• Rounded mass lesion with enhancement • Mixture of reparative processes, bone formation,
• Multiple lesions collagen deposition, fibrosis
• Associated pathologic fracture Microscopic Features
• Bone destruction
• Typical chronic fracture
Primary bone tumor
• Osteoblastoma, ABC, giant cell
• Mass lesion within pedicle, with expansion, ICLINICAL ISSUES
enhancement
• Bone destruction or remodeling
Presentation
• Most common signs/symptoms
Infection/septic facet o Back pain, radiculopathy if associated bony
• Abnormal low signal from facet, with irregular hypertrophy and nerve compression
adjacent soft tissue extension o Other signs/symptoms
• Joint effusion • May be asymptomatic finding
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IDIAGNOSTIC CHECKLIST
Consider
• Look for contralateral posterior element disease such
as pedicle hypoplasia, or aplasia
ISELECTED REFERENCES
1. Hollenberg GM et ali Imaging of the spine in sports
medicine. Curr Sports Med Rep. 2(1):33-40, 2003
2. Bose B: Fracture of Sl-2 after L4-S1 decompression and
fusion. Case report and review of the literature. J
Neurosurg. 99(3 Suppl):31O-2, 2003
3. Slipman CW et ali Sacral stress fracture in a female field
hockey player. Am J Phys Med Rehabil. 82(11):893-6, 2003
4. Sirvanci M et ali Pedicular stress fracture in lumbar spine.
Clin Imaging. 26(3): 187-93,2002
5. Shah MK et ali Sacral stress fractures: an unusual cause of
low back pain in an athlete. Spine. 27(4):E104-8, 2002
6. Kraft DE: Low back pain in the adolescent athlete. Pediatr
Clin North Am. 49(3):643-53, 2002
7. Reitman CA et ali Lumbar isthmic defects in teenagers
resulting from stress fractures. Spine]. 2(4):303-6, 2002
8. Fourney DR et ali Early sacral stress fracture after reduction
of spondylolisthesis and lumbosacral fixation: case report.
Neurosurgery. 51(6):1507-10; discussion 1510-1, 2002
9. Macdessi SJ et ali Pedicle fracture after instrumented
posterolateral lumbar fusion: a case report. Spine. 26(5):
580-2,2001
10. Chong VF et ali Pedicular stress fracture in the lumbar
spine. Australas Radiol. 41(3): 306-7, 1997
11. Heggeness MH et ali The trabecular anatomy of
thoracolumbar vertebrae: implications for burst fractures. J
Anat. 191 (Pt 2): 309-12, 1997
12. Gunzburg R et ali Stress fracture of the lumbar pedicle.
Case reports of "pediculolysis" and review of the literature.
Spine. 16(2): 185-9, 1991
13. Traughber PD et ali Bilateral pedicle stress fractures: SPECT
and CT features. J Comput Assist Tomogr. 15(2): 338-40,
1991
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Variant
(Left) NEeT Sagittal oblique
shows lucent fracture line
through C5-6 left pedicle
(arrow) with sclerotic
margins. Patient was post
C5·6 discectomy & failed
fusion. (Right) Axial TlWI
MR shows chronic right
pedicle fracture as linear low
signal with large amount of
bony hyperplasia (arrows).
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POST-TRAUMATIC SYRINX
1
108
Sagittal T2WI MR demonstrates a syrinx which formed Sagittal T1 C+ MR shows no abnormal enhancement
following a C5/6 disc herniaUon. associated with syrinx which formed following C5/6
disc herniaUon. Same case as on left.
• Myelography
ITERMINOlOGY o Arachnoiditis & adhesions at level of injury
Abbreviations and Synonyms o Focal changes in cord size
• Post-traumatic syrinx (PTS) o Delayed CT myelography
• Syringomyelia, Syringohydromyelia • Syrinx fills in with delayed scanning
• May change with position, characteristically j in
Definitions size when moving from prone to supine
• Cystic cord cavity that may (hydromyelia) or may not
(syringomyelia) communicate with central canal
CT Findings
• Artificial distinction ~ many use syringohydromyelia • NECT
o Cavitation with central CSF hypodensity
o Normal or atrophied cord
IIMAGING FINDINGS o May be difficult to appreciate central cavity on
unenhanced imaging
General Features • Bone CT
• Best diagnostic due: Cystic expansile cord lesion o May see posterior vertebral body scalloping in
• Location: Rostral to injury site in 81%, caudal in 4%, longstanding lesions
both directions in 15% MR Findings
• Size: Average length 6 cm; range 5 mm to entire cord
• TlWI: Focal hypointensity
• Morphology
o Longitudinal spinal cord cleft with CSF imaging • T2WI
o Focal hyperintensity
characteristics
• Myelomalacia precedes overt syrinx formation =
o Frequently has a fusiform "beaded" appearance
"presyrinx state"
Radiographic Findings o May begin as small cysts ~ coalesce into larger cysts
• Radiography o Adjacent cord may be normal or atrophied
o Spinal deformities: Fx, dislocations, kyphotic or • May appear to be an "expansile" lesion, a relative
lordotic changes finding in presence of cord atrophy
o Flexion/extension evaluate spinal stability • CSF Flow
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POST-TRAUMATIC SYRINX
Key Facts
1
Imaging Findings Pathology 109
• Best diagnostic clue: Cystic expansile cord lesion • Trauma most common cause of secondary syrinx
• Location: Rostral to injury site in 81%, caudal in 4%, • PTS incidence 0.3-4% of spinal cord injuries
both directions in 15%
• Frequently has a fusiform "beaded" appearance Clinical Issues
• Arachnoiditis & adhesions at level of injury • Most common symptom = pain which may increase
• May begin as small cysts ~ coalesce into larger cysts with coughing, sneezing, or straining
• Adjacent cord may be normal or atrophied • Classic presentation: Severe pain unrelieved by
• Best imaging tool: T2WI, T1 C+ MR analgesics, ascending disassociated sensory loss
• Untreated syrinx is progressive
Top Differential Diagnoses • Heralded by changing or new symptoms
• Gibbs artifact • Shunting can arrest neurologic progression
• Myelitis • Followed post-operatively by physical exam & MRI
• Spinal cord tumor
• Myelomalacia Diagnostic Checklist
• Idiopathic • Main imaging goal is to exclude associated or
causative lesions
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POST-TRAUMATIC SYRINX
1 • Tubular spinal cord cavitation surrounded by dense
glial fibril wall (Rosenthal fibers)
o Syringoperitoneal or syringosubarachnoid
o Shunt procedural goals
110 • Most form in gray matter between dorsal horns & • Associated scar dissection ~ promotes normal
posterior columns local CSF dynamics
o Predominantly affecting crossing pain & • Cyst drainage ~ ! abnormal intramedullary
temperature fibers pressure
• Hyalinized thickened vessels o Outcomes
• Aberrant nerve fibers in cavity wall • Pain or motor recovery occurs in "" 50%
• Perivascular (Virchow Robin) spaces may be enlarged • Sensory symptoms improve in 33%
• Adhesions often present tethering cord with local • Fails to halt symptom progression in 10%
scarring • Nearly 50% shunts non-functional at 6 yrs
• Microinfarction is another possible cause o Often performed with somatosensory evoked
• Enlargement secondary to cavity fluid motion in potential monitoring to assess cord function during
response to pressure changes procedure
• Followed post-operatively by physical exam & MRI
o Syrinx reduction postoperative MRI correlates with
ICLINICALISSUES satisfactory clinical outcome in 85%
• Other surgical considerations
Presentation o Laser syringotomy
• Most common signs/symptoms o Decompressive laminectomy with subarachnoid
o Spasticity, hyperhidrosis, pain, sensory loss, space reconstruction
automotive hyperreflexia o Spinal cord transection
o May result in neurologic deterioration years after o Pedicled omental graft transposition
injury
• Delays from 6 mos to 35 yrs reported
• Mean interval ranges 9-13 yrs IDIAGNOSTIC CHECKLIST
o Cervical: Rarely CN deficit(s), 25% have bulbar signs
o Rarely new onset urinary dysfunction, neuropathic Image Interpretation Pearls
joint • Main imaging goal is to exclude associated or causative
• Clinical profile lesions
o Most common symptom = pain which may increase
with coughing, sneezing, or straining
• Axial> radicular pain ISELECTED REFERENCES
o Classic presentation: Severe pain unrelieved by 1. Vannemreddy SS et al: Posttraumatic syringomyelia:
analgesics, ascending disassociated sensory loss predisposing factors. Br J Neurosurg. 16(3):276-83,2002
• Loss to pain & temperature with preservation to 2. Brugieres P et al: CSF flow measurement in syringomyelia.
touch AJNR 21(10): 1785-92, 2000
o Uncommon in childhood 3. Fischbein NJ et al: The "presyrinx" state: a reversible
• Most childhood syrinx is simple hydromyelia or myelopathic condition that may precede syringomyelia.
Chiari-associated AJNR 20(1): 7-20, 1999
4. Perrouin-Verbe B et al: Post-traumatic syringomyelia and
• More likely than adults to present with scoliosis post-traumatic spinal canal stenosis: a direct relationship:
o EMG ~ t in polyphasic spikes with t in motor unit review of 75 patients with a spinal cord injury. Spinal
action potential duration Cord. 36(2):137-43, 1998
5. Jinkins JR et al: MR of parenchymal spinal cord signal
Demographics change as a sign of active advancement in clinically
• Age: May occur at any age; average age at the time of progressive posttraumatic syringomyelia. AJNR Am J
injury"" 28 yrs (range, 16-67) Neuroradiol. 19(1):177-82, 1998
• Gender: M > F 6. Kramer KM et al: Posttraumatic syringomyelia: a review of
21 cases. Clin Orthop. (334):190-9, 1997
Natural History & Prognosis 7. Schurch B et al: Post-traumatic syringomyelia (cystic
• Variable; dependent on underlying etiology myelopathy): a prospective study of 449 patients with
• PTS onset earlier with t age, cervical & thoracic levels, spinal cord injury. J Neurol Neurosurg Psychiatry.
dislocated Fx, spinal instrumentation without 60(1):61-7, 1996
8. Sgouros S et al: Management & outcome of posttraumatic
decompression, complete SCI syringomyelia. J Neurosurg. 85(2):197-205, 1996
• Untreated syrinx is progressive 9. Silberstein M et al: Delayed neurologic deterioration in the
• As cavity enlarges patient with spinal trauma: role of MR imaging. AJNR Am J
o Involves previously unaffected neural tissue Neuroradiol. 13(5):1373-81, 1992
o Results in progressive myelopathy 10. Bronskill MJ et al: Syrinx-like artifacts on MR images of the
o Heralded by changing or new symptoms spinal cord. Radiology. 166(2):485-8, 1988
• Muscle wasting +/- spasticity
• Neuropathic Charcot joints
Treatment
• Shunting can arrest neurologic progression
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POST-TRAUMATIC SYRINX
IIMAGE GALLERY 1
111
Typical
(Left) Sagittal T2WI MR
demonstrates syrinx that
followed cervical spine
trauma with fracture (not
shown); susceptibility can be
seen from anterior fusion
hardware (arrows). (Right)
Axial T2WI MR confirms
central location of this
post-traumatic syrinx.
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Sagittal T2WI MR demonstrates hyperintense cord Axial T2WI MR shows foci of hypointense hemorrhage
contusion with focus of hypointense hemorrhage (arrows) within cord contusion. Note retropulsed
(arrow) following thoracic spine burst fracture. fragments (open arrow) following thoracic spine burst
fracture.
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Key Facts
1
Terminology • Spinal cord tumor 113
• Traumatic cord edema, swelling, and/or hemorrhage • Myelomalacia
Microscopic Features
I DIFFERENTIAL DIAGNOSIS • Cord microcirculation alterations maximize 24-48 hrs
Myelitis • Intramedullary edema +/- hemorrhage involving
central gray matter
• Demyelinating disease (e.g., MS), ADEM, lateral o Edema peaks 3-6 days, subsides by 1-2 weeks
myelitis • Extends outward with severity of trauma
• All may enhance
• Hemorrhagic necrosis & liquification may occur
Spinal cord tumor • Demyelination & cystic myelomalacia ensues
• Astrocytoma, ependymoma, hemangioblastoma o Reactive gliosis, vascular proliferation at the margins
• Cord expansion; nearly always enhancing Staging, Grading or Classification Criteria
Cavernous malformation • American Spinal Injury Association (ASIA)
• Best seen with GRE o Grade A: No motor or sensory function preserved in
• May be multiple sacral segments S4-S5
• May be asymptomatic o Grade B: Sensory, but not motor function is
preserved below neurologic level; sacral S4-S5
Myelomalacia function preserved
• Cord volume loss, gliosis o Grade C: Motor function preserved below neurologic
• Do not see CSF signal central cavitation on T1WI level, > 50% key muscles below have muscle grade <
3/5
o Grade D: Motor function preserved below
IPATHOLOGY neurologic level, > 50% key muscles below have
muscle grade> 3/5
General Features o Grade E: Normal
• Etiology o (neurologic level of injury = most caudal cord
o MVA (45%), falls (22%), acts of violence (16%), segment with normal sensory & motor function on
sports injury (13%) both sides of body)
• Most common act of violence is shooting • Kulkarni classification
• Most common sports injury results from diving
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Typical
(Left) Sagittal STIR MR
demonstrates hyperintense
cord contusion with a
suggestion of hypointense
hemorrhage. Also note
prevertebral (arrows) &
ligamentous (open arrows)
injuries. (Right) Axial T2*
GRE MR confirms
hemorrhage (arrow) within
cord contusion.
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Sagittal T2WI MR shows flame-shaped high signal in Sagittal n* CRE MR of patient with cord edema seen
cord centered on slight C3-4 disc protrusion. Note high on T2WI shows no evidence of hemorrhage in the cord;
signal in anterior longitudinal ligament (arrow). note poor sensitjvity of CRE image to edema.
ITERMINOLOGY CT Findings
• NECT: May show disc protrusion or extrusion
Abbreviations and Synonyms
• Bone CT
• Acute traumatic central cord syndrome (ATCCS) o Spondylosis or congenital canal stenosis
• Spinal cord injury without radiographic abnormality o Typically no fracture
(SCIWORA) o May be normal
• Synonyms
o Spinal cord concussion MR Findings
o Transient traumatic cord apraxia • T1 WI: Slight cord swelling
• T2WI
Definitions o High signal
• Acute post-traumatic paralysis affecting arms> legs, o Typically C3-4 and/or CS-61evel
with bladder dysfunction, variable sensory loss o Disc protrusion or extrusion
o Narrow canal
• T2* GRE
IIMAGING FINDINGS o High intraparenchymal cord signal
General Features o Low signal indicates hemorrhage, and predicts
permanent injury (not central cord syndrome)
• Best diagnostic clue: T2WI shows high signal in cord o Spondylosis exaggerated
• Location: Predominates at C3-4 through CS-6 levels o Loss of CSF signal (narrow canal and/or swollen
Radiographic Findings cord)
• Radiography Imaging Recommendations
o Typically negative • MRI in any case of post-traumatic cord signs is
o May show spondylosis or congenitally diminished
mandatory
canal diameter
• Myelography
o Done only if no MRI available
o Tight canal
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CENTRAL SPINAL CORD SYNDROME
Key Facts
1
Terminology • Spur, disc or ligamentous ossification predispose 117
• Acute traumatic central cord syndrome (ATCCS) • May have normal canal
• Acute posttraumatic paralysis affecting arms> legs, • Most often seen in young athletes
with bladder dysfunction, variable sensory loss • Primary injury to lateral corticospinal tract with loss
ofaxons; predominantly white matter injury
Imaging Findings • No hemorrhage
• Best diagnostic clue: T2WI shows high signal in cord • Central grey intact in acute, reversible cases
• Location: Predominates at C3-4 through C5-6 levels
Clinical Issues
Top Differential Diagnoses • Complete resolution to slight residual weakness and
• Cord contusion spasticity
• Cord compression or extensive edema on MRI
Pathology indicate worse prognosis
• Hyperextension force • In general, conservative therapy produces equal
• Compression of cervical cord by buckling of outcomes to surgical intervention
ligamenta flava
• With pre-existing canal narrowing
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·, C_EN_T_RA
__L_SP_I_N_A_L_C_O_R_D_SY_N
__D_R_O_M_E _
1 • Leg spasticity as residual
• Clinical profile: Post-traumatic cord signs in patient
5. Jimenez 0 et al: A histopathological analysis of the human
cervical spinal cord in patients with acute traumatic central
118 with underlying congenital or acquired spinal stenosis cord syndrome. Spinal Cord. 38(9):532-7, 2000
6. Newey ML et al: The long-term outcome after central cord
• Variability of clinical post-traumatic cord syndromes
syndrome: a study of the natural history. J Bone Joint Surg
which resolve to considerable degree within hours to Br. 82(6):851-5, 2000
days 7. An HS: Cervical Spine trauma. Spine 23:2713-29, 1998
o Anterior cervical cord syndrome 8. Chen TY et al: Efficacy of surgical treatment in traumatic
• Immediate complete paralysis, altered sensation central cord syndrome. Surg Neurol. 48(5):435-40;
• Preserved Vibratory/positional sense discussion 441, 1997
o Posterior cervical cord syndrome 9. Massaro F et al: Acute traumatic central cord syndrome.
• Pain and paresthesias in neck, upper arms and Acta Neurol (Napoli). 15(2):97-105, 1993
10. Martin D et al: MRI-pathological correlations in acute
trunk
traumatic central cord syndrome: case report.
• Symmetric and burning sensation Neuroradiology. 34(4):262-6, 1992
• May have mild paresis of arms 11. Quencer RM et al: Acute traumatic central cord syndrome:
MRI-pathological correlations. Neuroradiology.
Demographics 34(2):85-94, 1992
• Age: Children typically show some abnormality on 12. ZWimpfer TJ et al: Spinal cord concussion. J Neurosurg
radiographs, adults more often exhibit spinal cord 72:894-900, 1990
injury without radiographic abnormality 13. Riviello JJ Jr et al: Delayed cervical central cord syndrome
• Gender: No differences after trivial trauma. Pediatr Emerg Care. 6(2):113-7, 1990
• Ethnicity: Asians have higher incidence of ossified 14. Fox JL et al: Central spinal cord injury: magnetic resonance
posterior longitudinal ligament as predisposition imaging confirmation and operative considerations.
Neurosurgery. 22(2):340-7, 1988
Natural History & Prognosis 15. Torg JS et al: Neurapraxia of the cervical spinal cord with
transient quadriplegia. J Bone Joint Surg (Am)68:1354-70,
• Complete resolution to slight residual weakness and
1986
spaSticity 16. Merriam WF et al: A reappraisal of acute traumatic central
• Generally complete or near complete reversal of the cord syndrome. J Bone Joint Surg Br. 68(5):708-13, 1986
acute paralysis
• Prognosis is age related
o Most under age of fifty recover completely
o Those over age of seventy usually have significant
residual deficits
• Cord compression or extensive edema on MRI indicate
worse prognosiS
Treatment
• Initial stabilization if spinal instability suspected
• Decompression if focal stenosis from disc or focal spur
• Steroid therapy in first 24 hours may have a role
• In general, conservative therapy produces equal
outcomes to surgical intervention
I DIAGNOSTIC CHECKLIST
Consider
• Any cord signs after trauma are indication for MRI
Image Interpretation Pearls
• Hemorrhage on MRI in cord heralds lack of recovery
ISELECTED REFERENCES
1. Hendey GW et al: Spinal cord injury without radiographic
abnormality: results of the National Emergency
X-Radiography Utilization Study in blunt cervical trauma. J
Trauma. 53(1):1-4, 2002
2. Collignon F et al: Acute traumatic central cord syndrome:
magnetic resonance imaging and clinical observations. J
Neurosurg. 96(1 Suppl):29-33, 2002
3. Dai L: Magnetic resonance imaging of acute central cord
syndrome: correlation with prognosis. Chin Med Sci].
16(2):107-10,2001
4. Kothari P et al: Injury to the spinal cord without
radiological abnormality (SCIWORA) in adults. J Bone Joint
Surg Br. 82(7):1034-7, 2000
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Typical
(Left) Axial bone CT shows
C2 fracture in anteroinferior
edge of the vertebral body
(arrow) caused by surfing
injury in patient with central
cord syndrome. (Right)
Sagittal bone CT reformation
shows C2 fracture of
anteroinferior vertebral body
to better advantage (arrow)
than did axial slices in this
patient with central cord
syndrome after a surfing
injury.
Typical
(Left) Axial T2* CRE MR
shows high signal in cord in
patient whose mild disc
protrusion resulted in canal
stenosis, central cord
syndrome. (Right) Axial T2*
CRE MR shows low signal
from hemorrhage (due to
magnetic susceptibility
effects) in cord: Irreversible
paralysis following spinal
trauma.
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SPINAL CORD HERNIATION
1
120
Sagittal graphic shows focal dural defect in thoracic Axial CECT myelogram (L) and sagittal T2WI MR (R)
spine allowing cord herniation. Note distinctive cord show cord distortion, anterior displacement into
kink. extradural cavity (arrows), with focal expansion of
dorsal sac.
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SPINAL CORD HERNIATION
Key Facts
1
Terminology Top Differential Diagnoses 121
• Ventral cord herniation • Arachnoid cyst
• Herniation of spinal cord through defect in dura of • Adhesions
ventral canal • Epidural hematoma
• Epidural empyema
Imaging Findings
• Best diagnostic clue: Focal anterior displacement of Pathology
cord with expansion of dorsal subarachnoid space • General path comments: Defect or diverticulum in
• Location: Typically in mid-thoracic spine ventral dural sheet with cord herniation into it
• Focal cord deformity • Several proposed mechanisms ~
• Cord displaced anteriorly against posterior edge of • Congenital weakening of ventral dural fibers
vertebral body • Damage to ventral dura by disc herniation or other
• Increased dorsal subarachnoid sac mechanism
• May see secondary collection of contrast in extradural • Abnormal adhesion of cord to anterior dural sleeve
sac progressively wears down dura leading to herniation
• Best imaging tool: CT post-myelography
o Leg weakness
Adhesions o Other signs/symptoms
• Simulates appearance of arachnoid cyst • Brown-Sequard syndrome
• Cord adhesions usually present with herniation • Spasticity
Epidural hematoma • Sphincter dysfunction
• Clinical profile: Unexplained chronic, progressive leg
• Can produce focal cord displacement
• If subacute, will show high signal on Tl WI
pain with onset of myelopathy
• Usually more diffuse Demographics
Epidural empyema • Age: Most reported cases in middle-aged individuals
• Gender: No preference
• Usually more extensive
• TlWI show enhancement • Ethnicity: All
Natural History & Prognosis
• Progressive neurologic symptoms and signs until
I PATHOLOGY diagnosis made
General Features Treatment
• General path comments: Defect or diverticulum in • Options, risks, complications
ventral dural sheet with cord herniation into it o Surgery
• Etiology • Repair of dural defect
o Several proposed mechanisms ~ • Repositioning of cord
• Congenital weakening of ventral dural fibers • Excision of arachnoid cyst if present
• Damage to ventral dura by disc herniation or • Enlargement of dural defect with surgery, freeing
other mechanism cord also successful
• Abnormal adhesion of cord to anterior dural • Symptoms improve or resolve after successful surgery
sleeve progressively wears down dura leading to
herniation
• Large nerve sleeve diverticulum IDIAGNOSTIC CHECKLIST
• Epidemiology: Rare
• Associated abnormalities: Arachnoid cyst Consider
• Any focal cord displacement or subarachnoid space
Gross Pathologic & Surgical Features deformity as possible herniation
• Ventral dural defect or diverticulum • Rare, but important to recognize sice represents
• Cord herniated into defect curable cause of myelopathy
• Adhesions
• May see arachnoid cyst Image Interpretation Pearls
• Delayed filling of extradural sac on CT after
myelography with focal filling defect contiguous with
ICLINICAL ISSUES cord = herniation
Presentation
• Most common signs/symptoms
o Chronic leg pain
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SPINAL CORD HERNIATION
1 I SELECTED REFERENCES
122 1. Sasaoka R et al: Idiopathic spinal cord herniation in the
thoracic spine as a cause of intractable leg pain: case report
and review of the literature. ] Spinal Disord Tech.
16(3):288-94,2003
2. Barbagallo GM et al: Thoracic idiopathic spinal cord
herniation at the vertebral body level: a subgroup with a
poor prognosis? Case reports and review of the literature. ]
Neurosurg. 97(3 Suppl):369-74, 2002
3. Miyaguchi M et al: Idiopathic spinal cord herniation
associated with intervertebral disc extrusion: a case report
and review of the literature. Spine. 26(9):1090-4, 2001
4. Aizawa T et al: Idiopathic herniation of the thoracic spinal
cord: report of three cases. Spine. 26(20):E488-91, 2001
5. Eguchi T et al: Spontaneous thoracic spinal cord
herniation--case report. Neurol Med Chir (Tokyo).
41(10):508-12,2001
6. Watanabe M et al: Surgical management of idiopathic
spinal cord herniation: a review of nine cases treated by
the enlargement of the dural defect.] Neurosurg. 95(2
Suppl):169-72, 2001
7. Pereira P et al: Idiopathic spinal cord herniation: case
report and literature review. Acta Neurochir (Wien).
143(4):401-6,2001
8. Wada E et al: Idiopathic spinal cord herniation: report of
three cases and review of the literature. Spine.
25(15):1984-8, 2000
9. Ewald C et al: Progressive spontaneous herniation of the
thoracic spinal cord: case report. Neurosurgery.
46(2):493-5; discussion 495-6, 2000
10. Tekkok IH: Spontaneous spinal cord herniation: case report
and review of the literature. Neurosurgery. 46(2):485-91;
discussion 491-2, 2000
11. Marshman LA et al: Idiopathic spinal cord herniation: case
report and review of the literature. Neurosurgery.
44(5):1129-33, 1999
12. Vallee B et al: Ventral transdural herniation of the thoracic
spinal cord: surgical treatment in four cases and review of
literature. Acta Neurochir (Wien). 141(9):907-13, 1999
13. Dix]E et al: Spontaneous thoracic spinal cord h,erniation
through an anterior dural defect. A]NR Am] Neuroradio!.
19(7):1345-8, 1998
14. Henry A et al: Tethered thoracic cord resulting from spinal
cord herniation. Arch Phys Med Rehabi!. 78(5):530-3, 1997
15. Baur A et al: Imaging findings in patients with ventral
dural defects and herniation of neural tissue. Eur Radio!.
7(8):1259-63, 1997
16. Sioutos P et al: Spontaneous thoracic spinal cord
herniation. A case report. Spine. 21(14):1710-3,1996
17. Kumar R et al: Herniation of the spinal cord. Case report. ]
Neurosurg. 82(1):131-6, 1995
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Typical
(Left) Axial T2WI MR shows
defect in normal dural
(arrows) sac, with extension
of cord (open arrow)
through it. (Right) Axial
T7WI MR shows distorted,
anteriorly displaced cord.
Surgery confirmed the dural
defect in patient with slowly
progressive leg weakness.
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Posterior graphic shows bilateral lumbar posterior Axial bone CT myelogram shows facet joint subluxation,
element fractures. Right fracture has caused dural tear, fracture fragments, with leak of intrathecal dye (arrows).
allowing nerve root entrapment with CSF leak. StabJ1ization surgery verified suspected herniated nerve
roots.
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LUMBAR FRACTURE WITH DURAL TEAR
Key Facts
1
Terminology Pathology 125
• Traumatic dural laceration with nerve root (NR) • CSF leak seen (if looked for) in up to 60% of
entrapment thoracolumbar Fxs
• When neurological deficit present with lumbar Fx,
Imaging Findings 25% have dural tear
• Intrathecal dye necessary to demonstrate dural tear • L3 burst and laminar fx particularly common level to
• Dural tear warns surgeon to look for NR if posterior demonstrate CSF leak, NR herniation (65% in one
fusion planned series)
......
Treatment
• Treatment is aimed at immobilization, fixation (rods)
and closing dura
o If neurologic signs present, care taken not to
transect NR on approach
~rJ ~
•
.
~.
~ ;?I
'
IDIAGNOSTIC CHECKLIST --
Consider
• CT with intrathecal contrast injection for evaluating (Left) Anteroposterior radiography demonstrates "burst" L7 Fx with
any patient with neurological signs & burst fracture widened pedicles. CT myelography demonstrated CSF leak prior to
prior to surgery stabilization surgery where suspected nerve root herniation was
verified. (Right) Sagittal bone CT myelogram reformation shows
comminuted fracture and leak of contrast (arrows) prior to surgical
fixation which verified herniated nerve roots .
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EPIDURAL~SUBI)URAtHEMATOMA
1
126
Sagittal graphic of cervico-thoracic juncdon shows Sagittal T2WI MR (L) and T1WI MR (R) show lobulated
extensive dorsal epidural & ventral subdural hemorrhage collections pushing cord anteriorly Patient had acute
with cord compression. pain and leg weakness after minor exertion.
ITERMINOlOGY CT Findings
• NECT: High density within canal
Abbreviations and Synonyms
• CTA: May show arteriovenous malformation as rare
• Intraspinal hemorrhage cause
o Epidural hematoma (EDH)
o Subdural hematoma (SDH) MR Findings
Definitions • TIWI
o Hypo-, iso- or hyperintense (depending on age)
• Extravasation of blood into the epidural or subdural eccentric, multilocular or inhomogeneous
compartment of the spine multisegmental collection
• May conform symmetrically to subdural space
cinching the CSF space if in subdural
IIMAGING FINDINGS compartment
General Features • T2WI: Long mass in extraaxial compartment with
inhomogeneous low (if acute), or high signal (if
• Best diagnostic clue: Long segmental extra-axial mass
subacute) intensity features
encasing or displacing cord or cauda equina
• PD/lntermediate: Iso- to high signal of long fusiform
• Location: Anywhere along spinal canal
or oval mass in epidural space
• Size • T2* GRE: Accentuates low signal of tubular,
o Variable, typically multisegmental
multisegmental extra-axial collection
• Rarely focal, as when associated with focal fracture
• Tl C+: Marginal enhancement of collection
or disc extrusion
• Morphology: Typically fusiform, oval, or tubular Angiographic Findings
Radiographic Findings • Conventional
o Usually negative when searching for source
• Myelography: Effaced or constricted subarachnoid • Rarely, may show AVM or vascular tumor as
space
source
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EPIDURAL-SUBDURAL HEMATOMA
Key Facts
1
Terminology • T2WI: Long mass in extraaxial compartment with 127
• Epidural hematoma (EDH) inhomogeneous low (if acute), or high signal (if
• Subdural hematoma (SDH) subacute) intensity features
• Extravasation of blood into the epidural or subdural Top Differential Diagnoses
compartment of the spine • Epidural abscess/empyema
Imaging Findings • Epidural lipomatosis
• Best diagnostic clue: Long segmental extra-axial mass • Epidural tumor
encasing or displacing cord or cauda equina Pathology
• Hypo-, iso- or hyperintense (depending on age) • Minor exertion, e.g., sit-ups with Valsalva
eccentric, multilocular or inhomogeneous • Therapeutic anticoagulation
multisegmental collection • Instrumentation
• May conform symmetrically to subdural space
cinching the CSF space if in subdural compartment Clinical Issues
• Intense, knife-like pain
• Extremity weakness
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EPIDURAL-SUBDURAL HEMATOMA
1 Demographics
15. Dardik A et al: Subdural hematoma after thoracoabdominal
aortic aneurysm repair: an underreported complication of
128 • Age: Majority of afflicted aged 55-70 spinal fluid drainage?] Vasc Surg. 36(1):47-50, 2002
• Gender: 2/3 men 16. Sung]M et al: Acute spontaneous spinal epidural
• Ethnicity: All hematoma in a hemodialysis patient with a bleeding
tendency. Nephron. 91(2):358-60, 2002
Natural History & Prognosis 17. Tseng SH et al: Cervical epidural hematoma after spinal
• 40% recover completely manipulation therapy: case report.] Trauma. 52(3):585-6,
2002
Treatment 18. Reitman CA et al: Subdural hematoma after cervical
epidural steroid injection. Spine. 27(6):E174-6, 2002
• Options, risks, complications
19. Uber-Zak LD et al: Neurologic complications of sit-ups
o Surgical associated with the Valsalva maneuver: 2 case reports. Arch
o Non-surgical option exists for those with minor Phys Med Rehabil. 83(2):278-82, 2002
neurological signs
IDIAGNOSTIC CHECKLIST
Consider
• Contrast injection & fat-saturation for complete
characterization
o Active bleed may enhance vividly
Image Interpretation Pearls
• CT may help identify hemorrhage when MRI
confusing
ISELECTED REFERENCES
1. Groen R]: Non-operative treatment of spontaneous spinal
epidural hematomas: a review of the literature and a
comparison with operative cases. Acta Neurochir (Wien).
146(2):103-10,2004
2. Liao CC et al: Experience in the surgical management of
spontaneous spinal epidural hematoma.] Neurosurg. 100(1
Suppl):38-45, 2004
3. Kirwan R et al: Nontraumatic acute and subacute
enhancing spinal epidural hematoma mimicking a tumor
in a child. Pediatr Radiol, 2004
4. Steinmetz MP et al: Successful surgical management of a
case of spontaneous epidural hematoma of the spine
during pregnancy. Spine]. 3(6):539-42, 2003
5. Uribe] et al: Delayed postoperative spinal epidural
hematomas. Spine]. 3(2):125-9, 2003
6. Lang SA et al: Spinal epidural hematoma and epidural
analgesia. Can] Anaesth. 50(4):422-3, 2003
7. Chang FC et al: Contrast enhancement patterns of acute
spinal epidural hematomas: a report of two cases. A]NR Am
J Neuroradiol. 24(3):366-9, 2003
8. Muthukumar N: Chronic spontaneous spinal epidural
hematoma -- a rare cause of cervical myelopathy. Eur Spine
]. 12(1):100-3,2003
9. Kreppel D et al: Spinal hematoma: a literature survey with
meta-analysis of 613 patients. Neurosurg Rev. 26(1):1-49,
2003
10. Houten ]K et al: Paraplegia after lumbosacral nerve root
block: report of three cases. Spine]. 2(1):70-5, 2002
11. Clark MA et al: Spinal epidural hematoma complicating
thrombolytic therapy with tissue plasminogen activator--a
case report.] Emerg Med. 23(3):247-51, 2002
12. Stoll A et al: Epidural hematoma after epidural block:
implications for its use in pain management. Surg Neurol.
57(4):235-40,2002
13. Ravid S et al: Spontaneous spinal epidural hematoma: an
uncommon presentation of a rare disease. Childs Nerv
Syst. 18(6-7):345-7,2002
14. Allison E] ]r et al: Spinal epidural haematoma as a result of
warfarin/fluconazole drug interaction. Eur] Emerg Med.
9(2):175-7,2002
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EPIDURAL-SUBDURAL HEMATOMA
IIMAGE GALLERY 1
129
(Left) Sagittal Tl C+ MR
depicts peripherally
enhancing tubular collection
dorsal to cord in patient with
osteoporotic compression
fracture following minor
trauma. (Right) Sagittal T2WI
MR depicts large low signal
collection dorsal to cord in
patient with osteoporotic
fracture following minor
trauma.
Variant
(Left) Sagittal TlWI MR
shows high signal SOH
conforming to subdural
space in patient with platelet
dysfunction, recent fall,
onset of pain and leg
weakness. Note cinching of
CSF space. (Right) Axial
NECT depicts hyperdense
SOH cinching butterfly
shaped low density CSF
space in patient with fall,
platelet dysfunction, onset of
pain & leg weakness.
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VERTEBRAL ARTERY DISSECTION
1
130
Coronal MRA shows diffuse bilateral vertebral artery Axial TlWI MR with fat suppression shows crescentic
narrowing due to bilateral vertebral artery dissections hyperintense intramural hematomas in bilateral vertebral
(arrows). arteries, with significantly narrowed flow voids (arrows).
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VERTEBRAL ARTERY DISSECTION
Key Facts
1
Terminology Pathology 131
• Hemorrhage into vessel wall with subsequent stenosis • Causes 5-20% of all infarcts in young & niiddle-aged
or pseudoaneurysm patients
• Associated abnormalities: Multi-vessel involvement
Imaging Findings up to two thirds of cases
• Best diagnostic clue: Intramural hematoma (IH):
Crescentic Tl hyperintensity surrounding diminished Clinical Issues
flow void • Clinical profile: Neurologic symptoms may be
• Location: Most common between C2 & Cl vertebrae delayed up to one week or longer in 61% of cases
• Tapered or abruptly occluded lumen • Resolution or significant improvement of
• Irregular luminal surface angiographic stenosis in 90% within first 2 to 3
• "Pearl and string" Sign: Focal dilatation followed by months
narrowing
• Double lumen Diagnostic Checklist
• Aneurysmal dilation • Axial TlWI with fat-saturation to look for IH
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VERTEBRALARTEIlY DISSECTION
1 • Coughing, sneezing, roller-coaster ride • Rate of recurrent VAD: 8%
o 50% within first month
• Prolonged or sudden neck hyperextension or
132 rotation may be precipitating factor o 1% per year
• Epidemiology
o Estimate of 1 to 1.5 per 100,000 Treatment
o VAD responsible for 0.4-2.5% of all cerebrovascular • May not be indicated in absence of neurologic
accidents symptoms
• Causes 5-20% of all infarcts in young & • Anticoagulation, unless SAH present
middle-aged patients • Antiplatelet therapy
• 40% of posterior fossa ischemic strokes • If persistent ischemia, recurrent embolic events, or
• Carotid artery dissection 3-5x more common than intracranial hemorrhage
VAD o Surgical ligation
• Associated abnormalities: Multi-vessel involvement up o Endovascular occlusion with coils or balloons
to two thirds of cases o Stent-assisted angioplasty
• Pathophysiology
o Intimal tear or ruptured vasa vasorum leading to
intramural hematoma I DIAGNOSTIC CHECKLIST
• Subintimal hematoma cases complete or partial
vascular occlusion
Consider
• Subadventitial dissection results in • Axial TlWI with fat-saturation to look for IH
pseudoaneurysm: Potential rupture & SAH in Image Interpretation Pearls
intracranial VAD • Variable size and position of vertebral artery within
o Vascular stasis leading to emboli formation transverse foramina as a normal variant on CTA
Microscopic Features
• Hematoma within tunica media of vessel wall
o Compressing intima, distending adventitia ISELECTED REFERENCES
o May rupture back into vascular lumen: Double 1. Cohen JE et al: Endovascular management of symptomatic
lumen . vertebral artery dissection achieved using stent angioplasty
• Underlying atherosclerosis uncommon and emboli protection device. Neurol Res. 25(4):418-22,
2003
2. Dziewas R et al: Cervical artery dissection--clinical features,
risk factors, therapy and outcome in 126 patients. J Neurol.
ICLINICAL ISSUES 250(10):1179-84,2003
3. Sanelli PC et al: Normal variation of vertebral artery on CT
Presentation angiography and its implications for diagnosis of acquired
• Most common signs/symptoms pathology. J Comput Assist Tomogr. 26(3):462-70, 2002
o Posterior neck pain 4. Hasan I et al: Vertebral artery dissection in children: a
• Common in adults comprehensive review. Pediatr Neurosurg. 37(4):168-77,
• Infrequent in children: 12% 2002
o Occipital headaches 5. Schievink WI: Spontaneous dissection of the carotid and
vertebral arteries. N Engl J Med 344:898-906, 2001
• 70% of patients 6. Nakatsuka M et al: Three-dimensional computed
o Neurologic deficits tomographic angiography in four patients with dissecting
• Up to 85% of patients aneurysms of the vertebrobasilar system. Acta Neurochir
• Lateral medullary syndrome of Wallenberg (Wien). 142(9):995-1001,2000
• Ipsilateral Horner syndrome 7. Ahmad HA et al: Cervicocerebral artery dissections. J Accid
• Cerebellar signs Emerg Med. 16(6):422-4, 1999
• Clinical profile: Neurologic symptoms may be delayed 8. Barinagarrementeria F et al: Causes and mechanisms of
up to one week or longer in 61 % of cases cerebellar infarction in young patients. Stroke.
28(12):2400-4, 1997
Demographics 9. Bartels E et al: Evaluation of extracranial vertebral artery
dissection with duplex color-flow imaging. Stroke.
• Age 27(2):290-5, 1996
o Mean age: 44.9 years 10. Provenzale JM: Dissection of the internal carotid and
• Range 16-76 years vertebral arteries: Imaging features. AJR165: 1099-104,
• Gender 1995
o Male to female ratio 11. Schievink WI et al: Recurrent spontaneous cervical-artery
• 6.6 to 1 in children dissection. N Engl] Med. 330(6):393-7, 1994
• 1.3 to 1 in adults 12. Levy C et al: Carotid and vertebral artery dissection:
Three-dimensional time-of-flight MR angiography and MR
Natural History & Prognosis imaging versus conventional angiography. Radiology
• Spontaneously healing or recanalization in most cases 190:97-103, 1994
13. Hart RG et al: Cerebral infarction in young adults: a
• Resolution or significant improvement of
practical approach. Stroke. 14(1):110-4, 1983
angiographic stenosis in 90% within first 2 to 3
months
• Recurrent ischemic rate of 15%
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Typical
(Left) Coronal CTA shows
diffusely narrowed right
vertebral artery with minimal
contrast in its lumen
(arrows). (Right) Axial TI WI
MR with fat suppression
shows a narrowed flow void
in right vertebral artery,
surrounded by hyperintense
intramural hematoma
(arrow).
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CAROTID'AilTERY"DISSECTION'"
1
134
3D graphic illustrates internal carotid dissection with Common carotid DSA shows dissection at ICA origin as
clot filling the false lumen (arrows). Injury of adjacent near total loss of flow & distal string sign (arrows);
sympathetic ganglia (open arrow) can cause Horner dissection stops at skull base without petrous extension
syndrome, (open arrow).
ITERMINOlOGY MR Findings
Abbreviations and Synonyms • T1WI
o Eccentric crescentic thrombus within arterial wall
• Internal carotid (lCA), common carotid (CCA) • Most often hyperintense (subacute)
Definitions • Best seen with fat-saturation technique applied
• Traumatic-induced injury to carotid intima resulting • DWI: Secondary ischemia: Restricted brain diffusion
in blood dissecting into media creating false lumen ·MRA
o "String sign" (76%)
o Abrupt luminal normalization upon entry into
IIMAGING FINDINGS carotid canal (42%)
o Intimal flap (29%)
General Features o Dissecting aneurysm: Saccular near skull base (40%)
• Best diagnostic clue: Tapering luminal narrowing o Uncommon: Double lumen
• Location o Bilateral in 15%
o ICA at or near the bifurcation is most common o Infrequently extends intracranially
o ICA between bifurcation & base of skull Ultrasonographic Findings
o CCA proximal to bifurcation
• Duplex sonography less reliable than MRA as
o ICA at base of skull is least common
dissection is usually distal to region that can be seen
o Rarely extends intracranially
well on sonograms
• Size: Extends a few centimeters
• May see echogenic intimal flap or echogenic thrombus
• Morphology: "Sting sign" = tapered luminal stenosis • Doppler waveforms may show highly resistive,
CT Findings obstructive pattern
• NECT: Secondary ischemia: Hypodense brain Angiographic Findings
parenchyma
• Conventional
• CTA o Replaced by CTA for quick trauma screening
o Quick & easily accessible
o Requires invasive contrast bolus administration
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CAROTID ARTERY DISSECTION
Key Facts
1
Terminology • High risk associations: MVA, boxing, hanging, 135
• Traumatic-induced injury to carotid intima resulting chiropractic manipulation, coughing/sneezing
in blood dissecting into media creating false lumen • Accounts for 0.4-2.5% of all strokes in general
population but 5-20% of strokes among young
Imaging Findings patients « 45 yrs)
• Best diagnostic clue: Tapering luminal narrowing
• Morphology: "Sting sign" = tapered luminal stenosis
Clinical Issues
• Best imaging tool: Neck CTA • Most common symptom is unilateral anterior
headache (86%) .
Top Differential Diagnoses • Many spontaneously heal in 7-30 days
• Atherosclerotic narrowing • Primary therapy: Anticoagulation for 3-6 mos
• Spontaneous dissection • Consider endovascular treatment
• External compression
Diagnostic Checklist
Pathology • Hyperintense hematoma mimics flow on routine
• Precise mechanism unclear; four mechanisms of MRA ~ add axial fat-saturation TlWI if dissection
craniocervical injury have been described suspected
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CAROTID ARTERY DISSECTION
1 • Type 3 = intraoral trauma (classically children
running with items in their mouth) Demographics
136 • Type 4 = skull base Fx with intrapetrous • Age: Most are 35-50 yrs
thrombosis • Gender: All carotid dissection ~ M:F = 1.5:1
o Suggested that severe abdominal injuries may cause Natural History & Prognosis
carotid injury by transmission of a pressure wave
• Prone to elaborate microemboli ~ TIA or stroke
along the aorta
o Cerebral ischemia is most serious consequence
o High risk associations: MVA, boxing, hanging,
• Many spontaneously heal in 7-30 days
chiropractic manipulation, coughing/sneezing
• Dissecting aneurysms remain stable, ~ in size, or
o If in setting of minimal/mild trauma, consider resolve, but do not 1 in size
underlying arteriopathy: FMD, Marfan,
• Rarely result in death
Ehler-Danlos, cystic medial necrosis
• Blunt carotid artery injury has poorer prognosis
• Epidemiology o Associated with 20-40% mortality & permanent
o Traumatic carotid dissection becoming increasingly neurologic deficit in 40-80%
recognized
o Accounts for 0.4-2.5% of all strokes in general Treatment
population but 5-20% of strokes among young • Primary therapy: Anticoagulation for 3-6 mos
patients « 45 yrs) o In cases of residual aneurysm, persisting severe
o Dissecting aneurysms occur in 25-35% of patients stenosis, or underlying arteriopathy, long term
with carotid dissection aspirin therapy is often used
• Associated abnormalities: TIA, stroke, subarachnoid • If embolic events occur while anticoagulated
hemorrhage o Consider endovascular treatment
• Circumvents need for blood flow occlusion
Gross Pathologic & Surgical Features
required during surgical bypass
• Vessel tortuosity may indicate structural wall anomaly • Long term efficacy & durability of stent placement
predisposing to external mechanical injury for carotid dissection remains to be determined
Microscopic Features o Surgical options
• Penetration of blood through intima into carotid wall • Extracranial to intracranial bypass
• This splits media creating a false lumen • Bypass grafting
• Dissects for varying distances • Ligation of internal carotid artery for
• Dissecting aneurysm = intramural hematoma pseudoaneurysm repair
expanding into adventitia • Obtain follow-up study after a few months to evaluate
o Distinguished from pseudoaneurysm by presence of healing
retaining adventitial layer
I DIAGNOSTIC CHECKLIST
I CLINICAL ISSUES Image Interpretation Pearls
Presentation • Hyperintense hematoma mimics flow on routine MRA
~ add axial fat-saturation T1WI if dissection suspected
• Most common signs/symptoms
o Most common symptom is unilateral anterior
headache (86%)
o Other signs/symptoms I SELECTED REFERENCES
• TIA or stroke (58%) 1. Payton TF et al: Traumatic dissection of the internal carotid
• Postganglionic Horner syndrome (52%) artery. Pediatr Emerg Care. 20(1):27-9, 2004
• Bruit (48%); neck pain (25%) 2. Pride GL]r et al: Stent-coil treatment of a distal internal
• Amaurosis fugax (12%) carotid artery dissecting pseudoaneurysm on a redundant
• Lower cranial nerve deficit(s) (12%) loop by use of a flexible, dedicated nitinol intracranial
stent. A]NR Am] Neuroradiol. 25(2):333-7, 2004
• May be asymptomatic 3. Malek AM et al: Endovascular management of extracranial
o Delayed neurologic event in patient with history of carotid artery dissection achieved using stent angioplasty.
trauma is typical A]NR Am] Neuroradiol. 21(7):1280-92, 2000
• Symptoms onset can range 4 hrs to 75 days 4. Djouhri H et al: MR angiography for the long-term
• Clinical profile follow-up of dissecting aneurysms of the extracranial
o May not have any external sign of injury internal carotid artery. A]R Am] Roentgenol.
o May also be masked or misinterpreted in setting of 174(4):1137-40,2000
associated head or neck trauma 5. Nusbaum AO et al: Isolated vagal nerve palsy associated
with a dissection of the extracranial internal carotid artery.
o Postganglionic Horner syndrome (aka A]NR Am] Neuroradiol. 19(10):1845-7, 1998
oculosympathetic paresis) 6. Leclerc X et al: Helical CT for the follow-up of cervical
• Ptosis of upper eyelid & miosis internal carotid artery dissections. A]NR Am] Neuroradiol.
• Elevation of lower eyelid (upside-down ptosis) 19(5):831-7, 1998
• Loss of ipsilateral sweating of the face (anhidrosis) 7. Opeskin K: Traumatic carotid artery dissection. Am J
Forensic Med Pathol. 18(3):251-7, 1997
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CAROTID ARTERY DISSECTION
IIMAGE GALLERY 1
137
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Superselected spinal OSA demonstrates single large Supine radiograph obtained during myelography reveals
arterial feeder (arrow), compact nidus (open arrow), & numerous dilated veins resulting from a traumatic dural
dilated venous outflow (curved arrow) of traumatic AVF.
duralAVF.
ITERMINOLOGY MR Findings
• TlWI: Perimedullary or epidural flow voids from
Abbreviations and Synonyms enlarged draining veins
• Arteriovenous fistula (AVF)
• T2WI
• Traumatic dural AV fistula (tDAVF) o Unlike spontaneous DAVF, often no cord
• Post-traumatic paraspinal (AVF) enlargement/hyperintensity from congestive
Definitions myelopathy
o Perimedullary or epidural flow voids from enlarged
• Traumatic induced arteriovenous fistula
draining veins
• May compress cord or nerve roots
IIMAGING FINDINGS I • Tl C+
o May 1 conspicuity of enlarged draining veins
General Features o Rarely demonstrates nidus
• Best diagnostic clue: DSA demonstration of AVF nidus • MRA
with enlarged draining veins o Both time of flight & contrast techniques have been
• Location described
o Dural or extradural o Performed as adjunct to MRI for helping guide DSA
o Any segment; cervical> lumbar Angiographic Findings
• Size • Conventional
o Small nidus
o "Gold standard" for diagnosis and defining tDAVF
o May have extensive draining vein engorgement
o Angiographic protocol for evaluation of a suspected
• Morphology tDAVF includes selective injections of both external
o Small, tight AVF nidus
carotid + vertebral arteries, thyrocervical trunks, &
o Serpiginous veins
paired segmental arteries
Radiographic Findings o Demonstrates solitary AVF nidus & perimedullary or
• .Myelography: Serpiginous dye column defects from epidural enlarged draining veins
enlarged perimedullary or epidural draining veins • Angiographic appearance is same as sDAVF
,
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IPATHOLOGY
General Features IIMAGE GALLERY
• Etiology
o Usually caused by direct penetrating wounds
• Stabbing, gunshot, or major trauma with bone Fx
• Epidemiology: Rare lesions; much rarer than
spontaneous DAVF
ICLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Radiculopathy as a result of enlarged epidural veins
o Back pain
(Left) T2WI MR reveals flow voids on cord surface of dilated veins
Demographics from traumatic OAVF. Unlike spontaneous OAVF, traumatic often has
• Gender: Unlike spontaneous DAVF, tDAVF not not formed cord engorgement/congestion at time of patient
presentation. (Right) Embolization material can be seen (arrows)
associated with males
following successful interventional therapy of a traumatic dural AVF.
Natural History & Prognosis
• Rapidly progressive without treatment
Treatment
• MRI with MRA provides improved screening for dural
AVF gUiding subsequent DSA studies by helping target
fistula level
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Degenerative Diseases
Degenerative Disc Disease 11-2-6
Degenerative Endplate Changes 11-2-10
Instability 11-2-14
Vertebral Disc Bulge 11-2-18
Vertebral Disc Anular Tear 11-2-22
Intervertebral Disc Herniation, Cervical 11-2-26
Intervertebral Disc Herniation} Thoracic 11-2-30
Intervertebral Disc Herniation, Lumbar 11-2-34
Foraminal Disc Extrusion 11-2-38
Spondylolisthesis 11-2-42
Spondylolysis 11-2-44
Facet Arthropathy, Cervical 11-2-48
Facet Arthropathy, Lumbar 11-2-52
Facet Joint Synovial Cyst 11-2-56
Acquired Spinal Stenosis} Lumbar 11-2-60
Acquired Spinal Stenosis, Cervical 11-2-64
Degenerative Scoliosis 11-2-68
Neurogenic (Charcot) Arthropathy 11-2-72
DISH 11-2-76
OPLL 11-2-80
Ossification Ligamentum Flavum 11-2-84
Inflammatory Arthritides
Adult Rheumatoid Arthritis 11-2-88
Juvenile Chronic Arthritis 11-2-92
Seronegative Spondyloarthropathy 11-2-96
Gout 11-2-100
CPPD 11-2-102
Hemodialysis Spondyloarthropathy 11-2-104
Calcific Tendinitis, Longus Coli 11-2-106
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2
2
Sagittal graphic shows cervical disk extrusion, with the Sagittal T2WI MR shows C5-6 disc extrusion where the
base of herniation smaller than component within base of herniation (arrows) is smaller than rest of
epidural space. epidural component (curved arrows).
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DEGENERATIVE DISC DISEASE NOMENCLATURE
DIFFERENTIAL DIAGNOSIS
Methods of defining disc disease - 1 Locations
• Normal • Craniocaudal - disc level
• Bulge • Craniocaudal - infra-pedicular level
• Herniation (small, medium, large) • Craniocaudal - pedicular level
Methods of defining disc disease - 2
•
•
Craniocaudal - supra-pedicular level
Axial - central
2
• Normal • Axial - right (or left) central 3
• Bulge • Axial - right (or left) sub-articular
• Herniation (size measurement in 3 planes) • Axial - right (or left) foraminal
Methods of defining disc disease - 3 • Axial - right (or left) extra-foraminal
• Normal Canal compromise - axial images
• Bulge • Compromise < 1/3 is "mild"
• Protrusion • Compromise between 1/3 and 2/3 of canal is
• Extrusion "moderate"
• Extrusion with free fragment • Compromise of > 2/3 is severe
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DEGENERATIVE DISC DISEASE NOMENCLATURE
2
4
Axial view of lumbar disc showing right-left location Sagittal view for level classification: S (suprapedicular),
classification: C (centra/), S (sub-articular), F P (pedicular), I (infrapedicular), 0 (disc).
(foramina/), E (extra-foramina/).
o Displacement of nuclear material to a more o Type II: Hyperintense on TlWI, isointense on T2WI
peripheral site in the disc space (adipose tissue)
o Form of internal disc disruption o Type III: Hypointense on T1 WI and T2WI
o Avoid use of "herniation" since that implies (discogenic sclerosis)
extension beyond disc space • Non standard disc terms to avoid
• Limbus fracture o Disc material beyond the interspace (DEBIT)
o Traumatic separation of bone from edge of vertebral o Herniated nucleus pulposus (material other than
ring apophysis nucleus may be herniated)
o May have associated disc herniation o Rupture (implied traumatic etiology)
• Limbus vertebrae o Prolapse (synonymous with protrusion)
o Separation of rim of vertebral body ring apophysis
which may be traumatic or developmental
o Also called "rim lesion" I CUSTOM DIFFERENTIAL DIAGNOSIS I
• Spondylosis
o Shortened form of "spondylosis deformans", a Bulging disc
specifically defined sub-classification of • Disc tissue circumferentially beyond edges of
degeneration apophyses involving> 180 degrees of circumference
o Most commonly used in generic sense for any (50-100% of disc circumference)
degenerative changes of spine including osteophytic • Bulge is term that needs a differential diagnosis
enlargement of apophyseal bone • Bulge may be normal variant
• As in "cervical spondylosis" • Bulge associated with advanced disc degeneration
• Sub-ligamentous • Bulge associated with vertebral body remodeling
o Underneath the PLL (related to osteoporosis, trauma)
o Distinct meaning from "sub-anular" (where disc • Bulge in response to loading or angular motion with
herniation is contained by anulus) ligamentous laxity
o When distinction between sub-anular and • Bulge as fake out from central sub-ligamentous disc
sub-ligamentous cannot be made, then use term herniation
"sub-capsular"
• Peridural membrane
o Delicate membrane attaching undersurface of PLL I SELECTED REFERENCES
extending laterally and posteriorly encircling canal 1. Fardon DF et al: Nomenclature and classification of lumbar
outside dura disc pathology. Recommendations of the Combined task
• Sub-membranous Forces of the North American Spine Society, American
o When characterizing herniation infers that displaced Society of Spine Radiology, and American Society of
material is extruded beyond anulus and PLL, with Neuroradiology. Spine. 26(5):£93-£113, 2001
only peridural membrane investing the material 2. Milette PC: The proper terminology for reporting lumbar
intervertebral disk disorders. A]NR Am J Neuroradiol.
• Degenerative endplate changes (Modic changes)
18(10):1859-66, 1997
o MR signal abnormalities involving vertebral body
endplates related to degenerative disc disease
o Type I: Hypointense on Tl WI, hyperintense on
T2WI (fibrovascular marrow + edema)
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2
6
Sagittal graphic shows degeneration of L4·5 and l.5-S1 Sagittal T2WI MR shows low signal at L4-5, l.5-S1
intervertebral discs with loss of disc height, associated compared to normal disc signal at LJ-4. Loss of disc
type 1/ fatty endplate change, and osteophyte formation. height at L4-5, disc herniation at l.5-S1 (open arrow),
and anular tear at L4-5 (arrow).
'~,
:,:.~
.......
- .
Disc Space Infection Hemodialysis Spondy. Ankylosing Spondylitis
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2
10
Sagittal T2WI MR shows type I degenerative endplate Sagittal T7WI MR shows classic type /I endplate
changes as increased signal at L5-S7. There is disc changes due to fatty marrow replacement at L4-5.
degeneration with loss of signal from intervertebral disc.
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Key Facts
Terminology Pathology
• Vertebral endplate change, modic changes • Type I: Replacement with fibrovascular marrow
• Type I, II, III endplate change • Type II: Replacement by fatty marrow
• MR signal abnormalities involving vertebral body • Type III: Replacement by bony sclerosis with little
endplates related to degenerative disc disease residual marrow
• Type I: Hypointense on T1WI, hyperintense on T2WI • Type I: Present in 4% of patients undergoing MR for 2
• Type II: Hyperintense on TlWI, isointense on T2WI disc disease
11
• Type III: Hypointense on T1WI and T2WI • Type II: Present in 16% of patients undergoing MR
for disc disease
Top Differential Diagnoses • Type III: Least common, approximately 1%
• Disk space infection
• Hemodialysis spondyloarthropathy Clinical Issues
• Seronegative spondyloarthropathy • Relationship of type I endplate changes to low back
• Metastatic disease pain (discogenic pain) controversial
• Gout
• PET
o Hypometabolic with degenerative endplate changes Gout
o Hypermetabolic with disc space infection or • Rarely vertebral involvement with low signal
vertebral osteomyelitis involVing endplates, similar to disc space infection
o PET accurate and specific to differentiate
degenerative endplate change from infection
• Gallium Scan I PATHOLOGY
o No increased uptake with degenerative endplate
changes
General Features
• General path comments
Imaging Recommendations o Replacement of normal hematopoietic marrow
• Best imaging tool: MRI • Type I: Replacement with fibrovascular marrow
• Protocol advice: Sagittal TlWI and T2WI defines • Type II: Replacement by fatty marrow
lesions and classification • Type III: Replacement by bony sclerosis with little
residual marrow
• Genetics: None known
IDIFFERENTIAL DIAGNOSIS • Etiology
o Unknown
Disk space infection • Type I: Probably reflects sequelae of acute disc
• Low signal involving endplates similar to type I degeneration
change • Type I: Present in 30% of chymopapain treated
• Endplate destruction discs (good model for acute disc degeneration)
• Hyperintense intervertebral disc on T2WI • Type II: Probably reflects sequelae of chronic disc
• Paravertebral or epidural soft tissue phlegmon/abscess degeneration
• Epidemiology
Hemodialysis spondyloarthropathy o Adult with disc degeneration
• Low signal on T1 WI involVing endplates similar to o Type I: Present in 4% of patients undergoing MR for
pyogenic disc space infection disc disease
• May be indistinguishable from pyogenic disc space o Type II: Present in 16% of patients undergoing MR
infection for disc disease
Seronegative spondyloarthropathy o Type III: Least common, approximately 1%
• Inflammatory endplate changes (Anderson lesions) • Associated abnormalities
may mimic type I degenerative endplates o Disc degeneration
• Typical squaring of vertebral bodies • Loss of disc space height
• Late changes with fusion of bodies and posterior • Loss of signal from disc on T2WI
elements Gross Pathologic & Surgical Features
Metastatic disease • Disc degeneration with dessication of disc, bony
• Focal low signal on TlWI with bone destruction ± endplate eburnation
epidural extension Microscopic Features
• Low signal does not parallel endplates as in type I • Type I: Spindle cells, capillaries (vascularized fibrous
change tissue) with prominent interstitial space, thickened
trabeculae with new bone production
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IDIAGNOSTIC CHECKLIST
Consider
• Disc space infection if high signal within disc on T2WI
• Disc degeneration may also show increased signal
within disc on T2WI due to cracks, fissures
Image Interpretation Pearls
• Discrimination of early disc space infection and severe
type I endplate changes may be difficult or impossible
• Degenerative endplate changes due not show
paravertebral enhancing soft tissue, which is often
present with disc space infection
• Use axial post-contrast fat suppressed T1 WI to better
define paravertebral soft tissues
ISELECTED REFERENCES
1. Senegas]: Mechanical supplementation by non-rigid
fixation in degenerative intervertebral lumbar segments:
the Wallis system. Eur Spine]. 11 Suppl2: S164-9, 2002
2. Stumpe KD et al: FDG positron emission tomography for
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(Left) Sagittal T7 WI MR
shows low signal involving
L4-5 endplates (arrow), with
vacuum phenomena within
several disc spaces. Low
signal endplate margins
appear intact. (Right) Sagittal
T2WI MR shows high signal
from L4-5 endplates. The
disc at L4-5 is low signal
reflecting disc degeneration.
Note that endplates are
intact.
(Left) Sagittal T7 C+ MR
shows prominent
enhancement of the type I
change at L4-5 (arrows).
Mild linear enhancement
due to disc degeneration
present within the disc itself.
(Right) Sagittal T2WI MR
shows type I endplate
changes at C5-6 level with
increased signal. Slight
increased signal present
within disc itself due to disc
degeneration with fissuring.
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INSTABILITY
2
14
Sagittal TlWI MR shows anterolisthesis L4 on L5, with Sagittal TI C+ MR shows 1055 of disc height at LJ-4, with
1055 of disc height and vacuum phenomenon. retrolisthesis. There is pronounced
Retrolisthesis present at L5-S7 with marked 1055 of disc contrast-enhancement of type I degenerative endplate
height. changes (arrows).
t ';' ~,.
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INSTABILITY
Key Facts
Terminology Pathology
• Loss of spine motion segment stiffness, where applied • Translational instability occurs 5-6x more often in
force produces greater displacement than normal, women, 3x more common in African-American
with pain/deformity women than Caucasian women
Imaging Findings Clinical Issues 2
• Best diagnostic clue: Deformity which increases with • Nonspecific symptoms
motion and increases over time • Low back pain with occasional radiation 15
• Various parameters used for degenerative instability • Nonspecific clinical findings leads to use of
by plain films radiographic surrogate findings
• Dynamic slip> 3 mm in flexion/extension
• Static slip of 4.5 mm or greater Diagnostic Checklist
• Angulation> 10-15° suggests need for surgical • Radiographic assessment begins with standing AP and
intervention lateral views of lumbar spine with flexion/extension
• Traction spurs views
• Vacuum phenomenon
o Fractures
MR Findings o Infection (especially anterior column involvement)
• T1WI o Primary bone and metastatic tumors (vertebral body
o Anterolisthesis, retrolisthesis, lateral translation destruction, neural compression, post resection)
o Nonspecific changes of degenerative disc disease o Isthmic spondylolisthesis (L5-S1 progressive
o Controversial as to role in defining instability: Type deformity in children)
I degenerative endplate changes o Scoliosis
• T2WI: Loss of disc signal ± disc space height o Degenerative instabilities
• STIR: Type I endplate changes may be more evident on • Axial rotational (recurrent pain worse with
this sequence twisting); plain films show malaligned spinous
• T1 C+ processes and pedicle rotation
o Nonspecific enhancement of disc due to • Translational (degenerative spondylolisthesis);
degenerative disc disease plain films show spondylolisthesis, traction spurs,
o Enhancement of type I degenerative endplate vacuum phenomenon
changes • Retrolisthesis (posterior displacement of cephalad
Imaging Recommendations body, loss of posterior disc height, facet
• Best imaging tool: Flexion/extension plain films subluxation); plain films show increased
• Protocol advice: MR findings useful as secondary tool retrolisthesis with extension
for degeneration, endplate changes, stenosis and • Degenerative scoliosis (low back pain +/-
herniation claudication, radiculopathy); imaging shows
central and foraminal stenosis
• Post laminectomy (resection of 50% of bilateral
IDIFFERENTIAL DIAGNOSIS facets alters segmental stiffness); plain films show
new or worsening segmental motion
Pseudoarthrosis • Post fusion (altered biomechanics from fusion, but
• Abnormal low T1 signal extending through disc, little correlation between radiographic findings
posterior elements and ligaments and clinical symptoms)
• Etiology: Multifactorial
Infection • Epidemiology
• Endplate destruction, disc T2 hyperintensity o Translational instability occurs 5-6x more often in
women, 3x more common in African-American
Tumor women than Caucasian women
• Enhancing soft tissue mass o Facet joint angulation has been suggested as cause
Post-operative • Associated abnormalities: Clinical and imaging
relationship of disc degeneration to instability
• Following multilevel laminectomy or facetectomy
controversial
Gross Pathologic & Surgical Features
IPATHOLOGY • Findings of degenerative disc disease
General Features Microscopic Features
• General path comments • Same as degenerative disc disease
o Many causes 'of spinal instability
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INSTABILITY
ICLINICAL ISSUES ISELECTED REFERENCES
1. Tay BB et al: Indications, techniques, and complications of
Presentation lumbar interbody fusion. Semin Neurol. 22(2):221-30, 2002
• Most common signs/symptoms 2. Kristof RA et al: Degenerative lumbar
o Nonspecific symptoms spondylolisthesis-induced radicular compression:
o Low back pain with occasional radiation nonfusion-related decompression in selected patients
2 o Physical signs suggesting instability include palpable
step off deformity ± or spinous process lateral
without hypermobility on flexion-extension radiographs.]
Neurosurg. 97(3 Suppl):281-6, 2002
deviation 3. Haughton VM et al: Measuring the axial rotation of lumbar
16 vertebrae in vivo with MR imaging. A]NR Am]
o Paraspinal muscle spasms
Neuroradiol. 23(7):1110-6, 2002
o Nonspecific clinical findings leads to use of 4. Pitkanen MT et al: Segmental lumbar spine instability at
radiographic surrogate findings fleXion-extension radiography can be predicted by
• Clinical profile: Elderly female with nonspecific back conventional radiography. Clin Radiol. 57(7):632-9, 2002
pain with evidence of L4-5 spondylolisthesis by plain 5. Kuroki H et al: Clinical results of posterolateral fusion for
films degenerative lumbar spinal diseases: a follow-up study of
more than 10 years.] Orthop Sci. 7(3):317-24,2002
Demographics 6. Chen W] et al: Surgical treatment of adjacent instability
• Age: > 50 years after lumbar spine fusion. Spine. 26(22):E519-24, 2001
• Gender: M < F for degenerative anterior slip, especially 7. Bendo]A et al: Importance of correlating static and
at L4-5 dynamic imaging studies in diagnosing degenerative
lumbar spondylolisthesis. Am] Orthop. 30(3):247-50, 2001
Natural History & Prognosis 8. Nizard RS et al: Radiologic assessment of lumbar
intervertebral instability and degenerative
• 20% of subjects have instability resolve over 10 year
spondylolisthesis. Radiol Clin North Am. 39(1):55-71, v-vi,
time period 2001
• Increased flexion by plain films does not adversely 9. Gibson]N et al: Surgery for degenerative lumbar
affect long term clinical outcome spondylosis. Cochrane Database Syst Rev. (2):CD001352,
• L5-S 1 isthmic spondylolisthesis rarely unstable in 2000
adults 10. Gibso~]N et al: Surgery for degenerative lumbar
spondylosis. Cochrane Database Syst Rev. (3):CD001352,
Treatment 2000
• Conservative medical treatment of pain including 11. Mochida] et al: How to stabilize a single level lesion of
exercise, physical therapy, NSAIDs degenerative lumbar spondylolisthesis. Clin Orthop.
(368):126-34, 1999
• Surgical treatment
12. Nakai S et al: Long-term follow-up study of posterior
o Posterolateral fusion (+/- pedicle screw fixation) lumbar interbody fusion.] Spinal Disord. 12(4):293-9, 1999
• Disadvantages include variable satisfactory 13. Herkowitz HN et al: Management of degenerative disc
outcomes (16-95%), pseudoarthrosis (14-70%), disease above an L5-S1 segment requiring arthrodesis.
reoperation rates (25%) Spine. 24(12):1268-70, 1999
o Interbody fusions via anterior, posterior or 14. Whitecloud TS 3rd et al: Operative treatment of the
circumferential approaches degenerated segment adjacent to a lumbar fusion. Spine.
• Posterior lumber interbody fusion (PLIF) 19(5):531-6, 1994
15. Murata M et al: Lumbar disc degeneration and segmental
• Anterior lumbar interbody fusion (ALIF)
instability: a comparison of magnetic resonance images
• Transforaminallumbar interbody fusion (TLIF) and plain radiographs of patients with low back pain. Arch
• Risks: ALIF (retrograde ejaculation, cage displacement, Orthop Trauma Surg. 113(6):297-301, 1994
iatrogenic herniation), PLIF (arachnoiditis, epidural 16. Boden SD et al: Lumbosacral segmental motion in normal
hemorrhage, cage displacement) individuals. Have we been measuring instability properly?
Spine. 15(6):571-6, 1990
17. Abumi K et al: Biomechanical evaluation of lumbar spinal
I DIAGNOSTIC CHECKLIST stability after graded facetectomies. Spine. 15(11):1142-7,
1990
Consider 18. Weiler P] et al: Numerical [corrected] analysis of the load
capacity of the human spine fitted with L-rod
• Findings of "mechanical back pain" present with instrumentation. Spine. 15(12):1285-93, 1990
degenerative instability 19. Hayes MA et al: Roentgenographic evaluation of lumbar
• Specificity of plain films limited by high prevalence of spine flexion-extension in asymptomatic individuals.
lumbar spondylosis in general population Spine. 14(3):327-31, 1989
20. ]ohnsson KE et al: Postoperative instability after
Image Interpretation Pearls decompression for lumbar spinal stenosis. Spine.
• Radiographic assessment begins with standing AP and 11(2):107-10, 1986
lateral views of lumbar spine with flexion/extension 21. Fryrnoyer ]W et al: Segmental instability. Rationale for
views treatment. Spine. 10(3):280-6, 1985
22. Gertzbein SD et al: Centrode patterns and segmental
instability in degenerative disc disease. Spine. 10(3):257-61,
1985
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INSTABILITY
IIMAGE GALLERY
(Left) Sagittal Tl C+ MR
shows upper thoracic
retrolisthesis (arrows),
following multilevel
laminectomy/debridement
for infection. (Right) Sagittal
Tl C+ MR shows progressive
deformity and retrolisthesis
(arrows) at operative site,
marked thecal sac
compromise, and extensive
pseudomeningocele.
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VERTEBRAL DISC BULGE
2
18
Sagittal T2WI MR shows bulging discs at L4-5 and L5-s7 Axial T2WI MR with fat suppression shows broad-based
with associated hypointensities. Minimal height loss is disc bulge at L4-5, indendng ventral thecal sac.
present at L4-5.
• Myelography
ITERMINOLOGY o Smooth indentation on anterior thecal sac
Abbreviations and Synonyms • Central canal and subarticular recesses usually not
• Anular bulge compromised
• Unless short pedicles also present
Definitions o Bulging disc accentuated by upright myelography
• Generalized extension of disc beyond edges of • Especially at L4-5
vertebral ring apophyses
CT Findings
• NECT
o Smooth broad-based radial disc bulge
IIMAGING FINDINGS o Vacuum disc phenomenon
General Features • Bone CT
• Best diagnostic clue: Circumferential disc "expansion" o Better osseous detail with sagittal and coronal
beyond confines of vertebral endplates reformation
• Location MR Findings
o Cervical: C5-6 and C6-7 most common
• TlWI: Hypointense bulging disc
o Lumbar: L4-5 and L5-S1 most common
• T2WI
• Size: Short radius of extension: :s; 3 mm
o Hypointense bulging disc
• Morphology • Dessication and fibrosis
o Broad-based
• Initially seen as linear hypointensity
• > 50% of disc circumference
• Mild hypointensity likely related to normal aging
Radiographic Findings • Tl C+
• Radiography o Enhancing anular tears
o Disc height loss o Enhancing fibrovascular marrow in adjacent
o Endplate sclerosis and spurring vertebral endplates
o Facet arthropathy • Disc height loss
o Degenerative spondylolisthesis • Anular tear
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VERTEBRAL DISC BULGE
Key Facts
Terminology • Anular tear
• Anular bulge • Extension of contrast into anulus
• Generalized extension of disc beyond edges of • T1WI and T2WI MRI with sagittal and axial planes
vertebral ring apophyses Top Differential Diagnoses
Imaging Findings • Disc protrusion
• Ossification of posterior longitudinal ligament
2
• Best diagnostic clue: Circumferential disc "expansion"
beyond confines of vertebral endplates 19
Pathology
• Size: Short radius of extension: :s: 3 mm • Genetics: Genetic predisposition to disc degeneration
• > 50% of disc circumference • Epidemiology: Up to 39% of asymptomatic adults
• Smooth indentation on anterior thecal sac have bulging discs
• Central canal and subarticular recesses usually not
compromised Clinical Issues
• Bulging disc accentuated by upright myelography • 82% without progression after one year
• Hypointense bulging disc • > 80-90% success rate with conservative treatment
• Disc height loss
• Endplate spurs
• Ligamentum flavum hypertrophy
I DIFFERENTIAL DIAGNOSIS
• Facet arthropathy Disc protrusion
Nuclear Medicine Findings • < 50% of disc circumference
• Bone Scan • May exceed 3 mm in radius
o Uptake of radiotracer in endplates and posterior • Associated central canal, subarticular or foraminal
elements narrowing
• Degenerative changes Ossification of posterior longitudinal
Other Modality Findings ligament
• Discography • Most common in cervical spine (70%)
o Indications • Continuous, segmental, mixed or retrodiscal
• Failed conservative therapy • Hypointense on T1 WI and T2WI
• Normal or equivocal MRI findings • Vertical extension
• To correlate disc pathology with symptoms • Radiolucent line between ossified ligament and
• In absence of disc herniation posterior margin of vertebral body on CT
o Pain responses
• No pain, dissimilar pain, similar pain, exact pain Vertebral endplate spur
• Concordant response: Absent painful response in • Often associated with bulging disc
control disc • Continuous with vertebral endplate
• Exact pain in symptomatic disc • Focal or diffuse
o Extension of contrast into anulus • Marrow signal may be present
• Concentric or circumferential tear
• Radial tear
o Dallas discogram description IPATHOLOGY
• Grade 0: Contrast confined within nucleus
pulposus
General Features
• Grade 1: Inner third of anulus • Genetics: Genetic predisposition to disc degeneration
• Grade 2: Middle third • Etiology
• Grade 3: Outer third o Environmental factors
• Focal or < 30° of disc circumference • Heavy lifting
• Grade 4 (April and Bogduk): Outer third of anulus • Twisting
• > 30° of disc circumference • Flexion and extension
o Extension of contrast beyond anulus • Acute trauma
• Grade 5 (Schellhas) o Age
• Full thickness tear o Poor posture
• Focal or diffuse o Repetitive micro trauma
• Endplate injury
Imaging Recommendations • Rim lesion: Horizontal tear in outer anulus at its
• Best imaging tool attachment to ring apophysis
o T1WI and T2WI MRI with sagittal and axial planes o Interruption of nutritional supply to disc
o Only discography can identify symptomatic disc o Decreasing water content in nucleus pulposus
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VERTEBRAL DISC ANULAR TEAR
2
22
Sagittal T2WI MR shows small foci of high intensity Axial T2WI MR shows crescentic, focal high signal in
(arrows) in the far left lateral disc margins of U-4 and U-4 disc (arrow), in anulus within foraminal zone.
L4-5Ievels. Note signal loss ofparent discs. Sagittal T2WI showed an identical focus
cross-referenced to this site.
ITERMINOlOGY CT Findings
Abbreviations and Synonyms • CECT
o CECT can show enhancement of disc margin
• Anular fissure; anular defect; high intensity zone (HIZ) o CT post discography shows intradiscal dye at
in posterior anulus posterior margin of or leaking through anulus
o Term "tear" inaccurately implies acute traumatic
etiology, rather than the typical cause of repetitive MR Findings
stress • T1WI: Contrast-enhancing nidus in disc margin
• T2WI: High signal zone at edge of disc which has low
Definitions intrinsic signal
• Disruption of concentric collagenous fibers comprising • T1 C+: Focally enhancing nidus in posterior disc
the anulus fibrosus margin
Other Modality Findings
IIMAGING FINDINGS • Discography demonstrates contrast leak from central
site of injection through anulus
General Features • Discography is more a provocative test (symptom
• Best diagnostic clue: Abnormal signal focus (HIZ) at simulation), rather than a diagnostic imaging
posterior disc margin on MRI modality
• Location: Posterior margin of lumbar, thoracic, or o Separates symptomatic tears (internal disc
cervical disc disruption syndrome-IDD) from incidental ones
• Size: Typically a 1-2 mm focus o If typical pain reproduced
• Morphology: Well-circumscribed o No double-blind prospective studies exist to verify
• Typically seen with degenerating disc IDD theory
• Classic imaging appearance o Several studies show poor correlation between
o Focal HIZ in the anulus on T2WI with low signal of presence of tear and concordant symptoms at
parent disc discography
o Contrast-enhancement on T1WI
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VERTEBRAL DISC ANULAR TEAR
Key Facts
Terminology • MRI shows anular tears in majority of asymptomatic
• Anular fissure; anular defect; high intensity zone individuals
(HIZ) in posterior anulus • Associated abnormalities: Disc protrusion or
extrusion (herniation)
Imaging Findings
• Focal HIZ in the anulus on T2WI with low signal of Clinical Issues
• Chronic back pain or sciatica in absence of
2
parent disc
• Contrast-enhancement on TIWI mechanical nerve root compromise 23
• Tl C+: Focally enhancing nidus in posterior disc • Controversial role of anular tear
margin Diagnostic Checklist
• Discography is more a provocative test (symptom • Other sources of low back pain or disc pathology;
simulation), rather than a diagnostic imaging e.g., disc infection
modality • Incidentally seen focal high intensity zone in anulus
Pathology of otherwise normal disc is essentially a normal
• Autopsy demonstrates high prevalence of tears finding
• Direct association with disc degeneration
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VERTEBRAL DISC ANULAR TEAR
o Source of enhancement
Image Interpretation Pearls
Staging, Grading or Classification Criteria • Incidentally seen focal high intensity zone in anulus
• Concentric tears are essentially an aging variant of otherwise normal disc is essentially a normal
• Rim tears and radial tears may have clinical finding
consequences in some cases
• Dallas discogram description
2 o Grade 0: Contrast confined within nucleus pulposus
o Grade 1: Inner third of anulus
I SELECTED REFERENCES
1. Munter FM et al: Serial MR Imaging of Annular Tears in
24 o Grade 2: Middle third Lumbar Intervertebral Disks. AJNR Am J Neuroradiol.
o Grade 3: Outer third 23(7):1105-9,2002
o Focal or < 30° of disc circumference 2. Slipman CW et al: Side of symptomatic annular tear and
o Grade 4: (April and Bogduk): Outer third anulus site of low back pain: is there a correlation? Spine.
o > 30° of disc circumference 26(8):EI65-9, 2001
• Extension of contrast beyond anulus 3. Weishaupt D et al: Painful Lumbar Disk Derangement:
Relevance of Endplate Abnormalities at MR Imaging.
o Grade 5 (Schellhas) Radiology. 218(2):420-7, 2001
o Full thickness tear 4. Schollmeier G et al: Observations on fiber-forming
o Focal or diffuse collagens in the anulus fibrosus. Spine. 25(21): 2736-41,
2000
5. Mercer S et al: The ligaments and annulus fibrosus of
ICLINICAL ISSUES human adult cervical intervertebral discs. Spine. 24(7):
619-26; discussion 627-8, 1999
Presentation 6. Ahn JM et al: Peripheral focal low signal intensity areas in
• Most common signs/symptoms the degenerated annulus fibrosus on T2-weighted fast spin
echo MR images: correlation with macroscopic and
o Back or radicular pain
microscopic findings in elderly cadavers. Skeletal Radiol.
• Recurrent meningeal nerve and ventral ramus of 28(4):209-14, 1999
somatic spinal nerve are sources of innervation 7. Saifuddin A et al: The value of lumbar spine magnetic
• Pain may be radicular in distribution resonance imaging in the demonstration of anular tears.
• Anular disruption may allow inflammatory Spine. 23(4):453-7, 1998
substances to leak from nucleus 8. Ito M et al: Predictive signs of discogenic lumbar pain on
• Most anular tears are asymptomatic magnetic resonance imaging with discography correlation.
o Other signs/symptoms Spine. 23(11):1252-8; discussion 1259-60, 1998
9. Stadnik TW et al: Annular tears and disk herniation:
• Reproducibility of pain with discography
prevalence and contrast enhancement on MR images in
• Clinical profile the absence of low back pain or sciatica. Radiology.
o Chronic back pain or sciatica in absence of 206(1):49-55, 1998
mechanical nerve root compromise 10. Vernon-Roberts B et al: Pathogenesis of tears of the anulus
o Controversial role of anular tear investigated by multiple-level transaxial analysis of the
• Most tears are incidental findings Tl2-Ll disc. Spine 22(22): 2641-6, 1997
11. Wood KB et al: Magnetic resonance imaging of the
Demographics thoracic spine. Evaluation of asymptomatic individuals. J
• Age Bone Joint Surg Am. 77(11):1631-8, 1995
o Up to 25% of normal individuals under 20 show 12. Bogduk N et al: The innervation of the cervical
intervertebral discs. Spine. 13(1): 2-8, 1988
anular defects
13. Yu SW et al: Tears of the anulus fibrosus: correlation
o Majority of adult individuals harbor anular defects between MR and pathologic findings in cadavers. AJNR Am
• Gender: No gender differences J Neuroradiol. 9(2):367-70, 1988
14. Pooni]S et al: Comparison of the structure of human
Natural History & Prognosis intervertebral discs in the cervical, thoracic and lumbar
• Most are asymptomatic or self-limited regions of the spine. Surg Radiol Anat. 8(3): 175-82, 1986
o Scar endpoint of inflammation 15. Johnson EF et al: The distribution and arrangement of
• Some felt to be cause of chronic back pain and sciatica elastic fibres in the intervertebral disc of the adult human.
• Good in most symptomatic cases J Anat. 135 (Pt 2): 301-9, 1982
• Up to 1/3 of chronic pain sufferers have recurrent
symptoms despite any therapy
Treatment
• Symptomatic pain relief with NSAIDs
• Chronic pain may prompt patients to undergo fusion
as last resort
IDIAGNOSTIC CHECKLIST
Consider
• Other sources of low back pain or disc pathology; e.g.,
disc infection
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VERTEBRAL DISC ANULAR TEAR
IIMAGE GALLERY
Typical
(Left) Axial T2WI MR shows
semilunar high signal in the
posterolateral anulus within
the L4-5 foramen (arrow),
the low signal of the disc
indicative of its
... ~.
degeneration. (Right) Sagittal
..<~
the foraminal margin of the
":(.~; -ill
"'~. ~
L4-5 disc.
itt..•,'- . _, .
':'t;-,"";. .." _.,.• t.:• • •"",. • ,.'
·.Y~.$!l'.
r -=-,":
~;:.
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INTERVERTEBRAL DISC HERNIATION, CERVICAL
Key Facts
Terminology • Greatest diameter of extrusion in any plane>
• Abbreviation: Cervical disc herniation (CDH) distance between edges of base in same plane
• Synonyms: Protruded disc, extruded disc, free • Sequestered or free fragment: Extruded disc without
fragment, sequestered disc contiguity to parent disc
• Nonstandard: Prolapse, herniated nucleus pulposus
(HNP), rupture
Top Differential Diagnoses
• OPLL
2
Imaging Findings • Osteophyte 27
• Best diagnostic clue: Small mass in spinal canal, • Tumor
contiguous with intervertebral disc • Abscess
• Protrusion is herniated disc with broad-base at parent Clinical Issues
disc • Clinical symptoms affected by level, location and size
• Greatest diameter of protrusion in any plane < of herniation
distance between edges of base in same plane • Neck pain and stiffness, with radiating pain to
• Extrusion is herniated disc with narrow or no base at shoulder and upper extremity
parent disc
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INTERVERTEBRAL DISC HERNIATION, CERVICAL
• Anulus fibrosus • Compressive cervical myelopathy with herniation
o Fibrocartilage, with collagen fibers in concentric and spondylosis treated by corpectomy with strut
lamellae graft or with multilevel laminectomy
Microscopic Features
• Degeneration shows cracks, fissures within nucleus; I DIAGNOSTIC CHECKLIST
endplate fragmentation; granulation tissue; thickening
2 endplate trabeculae Consider
• OPLL if extradural disease low in signal on all
28 sequences
ICLINICAL ISSUES
Image Interpretation Pearls
Presentation • Define herniation by level, size, location (central,
• Most common signs/symptoms paracentral, lateral), effect on thecal sac and cord,
o Clinical symptoms affected by level, location and migration away from disc level
size of herniation • Combination of axial T1WI and gradient echo T2*
o Neck pain and stiffness, with radiating pain to better define disc contour due to variable disc signal
shoulder and upper extremity • FLAIR insensitive in the spine for intrinsic cord signal
o May include paresthesia, hyperesthesia and abnormality such as contusion, myelomalacia
weakness o Use fast STIR as additional sequence for cord disease
• Neck pain (90%) combined with standard fast spin echo T2WI
• Paresthesia (89%)
• Radicular pain (65%)
• Weakness (15%) ISELECTED REFERENCES
o Central herniations produce cord compression; 1. Furusawa N et al: Herniation of cervical intervertebral disc:
paracentrals compress ipsilateral hemicord immunohistochemical examination and measurement of
o Lateral herniations compress anterior cervical root nitric oxide production. Spine. 26(10): 1110-6, 2001
giving weakness without pain 2. Fardon DF et al: Nomenclature and classification of lumbar
o Lateral herniations compress root and dorsal root disc pathology. Recommendations of the Combined task
ganglion causing pain Forces of the North American Spine Society, American
• Clinical profile Society of Spine Radiology, and American Society of
Neuroradiology. Spine. 26(5): E93-E113, 2001
o 4th-5th decades
3. Campi A et al: Comparison of MRI pulse sequences for
o Risk factors include increasing age; heavy physical investigation of lesions of the cervical spinal cord.
work; work dissatisfaction; depression; history of Neuroradiology. 42(9): 669-75, 2000
headache 4. Grob D: Surgery in the degenerative cervical spine. Spine.
23(24): 2674-83, 1998
Demographics 5. Ahlgren BD et al: Cervical Radiculopathy. Orthop Clin
• Age: Adult population North Am. 27(2): 253-63, 1996
• Gender: M > F 6. Jensen MC et al: Magnetic resonance imaging of the
lumbar spine in people without back pain. N Engl] Med.
Natural History & Prognosis 331(2): 69-73, 1994
• Acute radiculopathy usually self-limited disorder with 7. Radhakrishnan K et al: Epidemiology of cervical
full recovery expected radiculopathy. A population-based study from Rochester,
• Chronic nonspecific pain much more variable Minnesota, 1976 through 1990. Brain. 117 (Pt 2): 325-35,
1994
o 90% back to work by 3 months
8. Sze G et al: Fast spin-echo MR imaging of the cervical
o Continuous or recurrent pain affects 30-40% spine: influence of echo train length and echo spacing on
image contrast and quality. A]NR Am] Neuroradiol. 14(5):
Treatment 1203-13, 1993
• Options, risks, complications 9. Jahnke RW et al: Cervical stenosis, spondylosis, and
o Conservative treatment herniated disc disease. Radiol Clin North Am. 29(4):
• Exercise and physical conditioning 777-91, 1991
• Nonsteroidal anti-inflammatory drugs 10. Boden SD et al: Abnormal magnetic-resonance scans of the
• Transcutaneous electrical stimulation cervical spine in asymptomatic subjects. A prospective
• Epidural steroid injection investigation.] Bone Joint Surg Am. 72(8): 1178-84, 1990
11. Modic MT et al: Imaging of degenerative disease of the
• Opioids cervical spine. Clin Orthop. (239): 109-20, 1989
• Atypical analgesics such as antidepressants, 12. Brown BM et al: Preoperative evaluation of cervical
antiepileptic drugs, neuroleptics radiculopathy and myelopathy by surface-coil MR imaging.
o Multiple surgical approaches without clear A]R Am] Roentgenol. 151(6): 1205-12, 1988
consensus 13. Simon]E et al: Diskogenic disease of the cervical spine.
• Anterior discectomy with fusion (with or without Semin Roentgenol. 23(2): 118-24, 1988
anterior plating and screw fixation) 14. Crandall PH et al: Cervical spondylotic myelopathy.]
• Anterior discectomy without fusion Neurosurg. 25(1): 57-66, 1966
• Hemilaminectomy and foraminotomy
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INTERVERTEBRAL DISC HERNIATION, CERVICAL
IIMAGE GALLERY
Typical
(Left) Sagittal T7 WI MR
shows extrusion displacing
cervical cord. High signal
inferior to COH is epidural
2
fat (arrow). Isointense signal
superior to herniation is 29
epidural plexus (open
arrow). (Right) Sagittal T2WI
MR shows small central
protrusion as low signal
effacing ventral thecal sac
but not distorting the cord.
Type I degenerative endplate
changes are present as high
signal at C5-6.
Typical
(Left) Axial T2* CRE MR
shows small focal protrusion
touching ventral cord.
Herniation is < 25% of the
disc margin. (Right) Axial
T7 WI M R shows
broad-based extrusion
severely impinging upon
cord. Herniation is > 25%
and < 50% of disc
circumference.
(Left) Sagittal T7 C+ MR
shows C4-5 extrusion
impinging upon cord.
Smaller C5-6 protrusion also
effaces ventral cord.
Peripheral enhancement
related to epidural plexus
and granulation tissue.
(Right) Axial T2* CRE MR
shows left sided extrusion
(arrow) effacing left side of
thecal sac and cord, and
extending towards left neural
foramen.
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INTERVERTEBRAL DISC HERNIATION, THORACIC
2
30
Axial T2· eRE MR through the upper thoracic spine Sagittal TlWI MR with contrast shows large hypointense
shows large left sided extrusion (arrow) compressing the mass contiguous with disc space reflecting calcified disc
left side of thoracic cord (open arrow). extrusion. Prominent distended epidural veins "tent"
around herniation.
I
.; J
"
I'
.~' '~;
FI . I
Meningioma Chondroid Tumor Multiple Myeloma Infection
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INTERVERTEBRAL DISC HERNIATION, THORACIC
• Cord injury/vascular injury
Gross Pathologic & Surgical Features • Migrated disc fragment missed
• Nucleus pulposus • Intradural disc extension missed
o Gelatinous material with high water content and • Lack of adequate visualization during surgery
few collagen fibers
o Water content decreases with age
• Anulus fibrosus IDIAGNOSTIC CHECKLIST
2 o Fibrocartilage, with collagen fibers in concentric
lamellae Consider
32 • Calcification (65%)
Microscopic Features
• Multiple herniation (14%)
• Degeneration shows cracks, fissures within nucleus; • Intradural herniations (7%)
endplate fragmentation; granulation tissue; thickening
endplate trabeculae Image Interpretation Pearls
• T2WI critical since herniation may not be visible on
T1WI due to calcification
ICLINICAL ISSUES • Check and recheck herniation level, counting from C2
and L5 levels
Presentation • Combination of axial T1 WI and gradient echo T2*
• Most common signs/symptoms better define disc contour due to variable disc signal
o Protean symptomatology
o Pain: Axial, localized or radicular (76%)
o Myelopathy ISELECTED REFERENCES
• Motor impairment (60%)
1. Dickman CA et al: Reoperation for herniated thoracic discs.
• Hyperreflexia, spasticity (58%)
J Neurosurg. 91(2 Suppl): 157-62, 1999
• Sensory impairment (61%) 2. Levi N et al: Thoracic disc herniation. Unilateral
• Bladder dysfunction (24%) transpedicular approach in 35 consecutive patients. J
o Other signs/symptoms Neurosurg Sci. 43(1): 37-42; discussion 42-3, 1999
• May rarely present as abdominal pain 3. Stillerman CB et al: Experience in the surgical management
• Clinical profile of 82 symptomatic herniated thoracic discs and review of
o 5th decade the literature. J Neurosurg. 88(4): 623-33, 1998
o Prior trauma history 4. Korovessis PG et al: Three-level thoracic disc herniation:
case report and review of the literature. Em Spine]. 6(1):
o History of Scheuermann
74-6, 1997
Demographics 5. Wood KB et al: The natural history of asymptomatic
thoracic disc herniations. Spine. 22(5): 525-9; discussion
• Age: Adult population 529-30, 1997
• Gender: M = F 6. Wood KB et al: Magnetic resonance imaging of the
thoracic spine. Evaluation of asymptomatic individuals. J
Natural History & Prognosis Bone Joint Surg Am. 77(11): 1631-8, 1995
• Asymptomatic population 7. Whitcomb DC et al: Chronic abdominal pain caused by
o Thoracic disc herniation (37%) thoracic disc herniation. Am J Gastroenterol. 90(5): 835-7,
o Disc bulge (53%) 1995
o Anular tear (58%) 8. Boukobza M et al: Thoracic disc herniation and spinal cord
• Thoracic disc herniations compression. MRI and gadolinium-enhancement. J
o As in cervical and lumbar spine, high percentage of Neuroradiol. 20(4): 272-9, 1993
9. Dietze DD Jr et al: Thoracic disc herniations. Neurosurg
herniations remain without major neurologic Clin N Am. 4(1): 75-90, 1993
compromise 10. Brown CW et al: The natural history of thoracic disc
o 20-30% may require surgery herniation. Spine. 17(6 Suppl): S97-102, 1992
o Small herniations (0-10% canal compromise) tend to 11. Parizel PM et al: Gd-DTPA-enhanced MR in thoracic disc
remain stable in size, and remain asymptomatic herniations. Neuroradiology. 31(1): 75-9, 1989
o Large herniations (> 20% canal compromise) tend to 12. Blumenkopf B: Thoracic intervertebral disc herniations:
decrease in size diagnostic value of magnetic resonance imaging.
Neurosurgery. 23(1): 36-40, 1988
Treatment 13. Ross JS et al: Thoracic disk herniation: MR imaging.
• Options, risks, complications Radiology. 165(2): 511-5, 1987
14. Benson MK et al: The clinical syndromes and surgical
o Thoracic disc surgery uncommon, represents 1-2%
treatment of thoracic intervertebral disc prolapse. J Bone
of all disc surgery Joint Surg Br. 57(4): 471-7, 1975
o Surgery for intractable pain, neurologic dysfunction 15. Carson J et al: Diagnosis and treatment of thoracic
o Variety of surgical approaches intervertebral disc protrusions. J Neurol Neurosurg
• Transthoracic Psychiatry. 34(1): 68-77, 1971
• Transfacet pedicle sparring
• Lateral extracavitary
• Transpedicular
o Causes of surgical failure for thoracic herniations
• Misidentified level
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INTERVERTEBRAL DISC HERNIATION, THORACIC
IIMAGE GALLERY
(Left) Sagittal T2WI MR
shows small focal
midthoracic disc protrusions, 2
effacing thecal sac but not
deforming thoracic cord.
(Right) Axial Tl C+ MR 33
shows large thoracic
calcified extrusion as low
signal ventral epidural mass
(arrow) effacing cord (open
arrow).
(Left) Sagittal Tl WI MR
shows large extrusion at T2-3
level (arrow) effacing thecal
sac. Second smaller disc
protrusion present at T7-8
(open arrow). (Right) Sagittal
T2WI MR shows large
thoracic disc extrusion
compressing anterior cord
(arrow). There are sloping
margins to lesion related to
displaced posterior
longitudinal ligament and
veins.
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2
34
Sagittal T2 WI MR shows a disc extrusion at L5-S 7. A disc Sagittal T2WI MR shows a "tongue-like" disc protrusion
protrusion is present at L4-5. Note the "mushroom" at LS-S 7. The greatest cranial-caudal dimension of
appearance of extrusion. protruded disc is the same as that of its base.
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INTERVERTEBRAL DISC HERNIATION, LUMBAR
IIMAGE GALLERY
Typical
(Left) Sagittal T2WI MR
shows a disc fragment (open
arrows) migrated caudally
from its parent disc at L5-S I,
with a thin attachment
2
(arrow). (Right) Sagittal T1 37
C+ MR shows migrated disc
fragment (arrow) posterior to
S 1 segment demonstrating
mild peripheral
enhancement.
Typical
(Left) Axial T2WI MR shows
a left subarticular disc
protrusion (arrow) at L5-S1
distorting thecal sac and
obliterating subarticular
recess. The traversing left S 1
nerve root is impinged.
(Right) Axial T1WI MR
shows a free disc fragment
(arrow) to the left of thecal
sac at L5-S I.
Typical
(Left) Sagittal T1WI MR
shows a foraminal disc
extrusion (arrow) at L4-5
obliterating perineural fat.
The exiting L4 nerve root is
impinged. (Right) Sagittal T1
C+ MR with fat suppression
shows intra vertebral disc
herniation (arrow) at
superior endplate of L4 with
surrounding marrow
enhancement.
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FORAMINAL DISC EXTRUSION
2
38
Sagittal T7WI MR shows L5-s1 foraminal disc extrusion Axial T7WI MR shows a left L4-5 foraminal disc
(open arrows), displacing exiting L5 nerve root (arrow). extrusion (open arrow), contacting exiting left L4 nerve
root (arrow).
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FORAMINAL DISC EXTRUSION
• Rim lesion: Horizontal tear in outer anulus at its • Cortisone ± lidocaine
attachment to ring apophysis • 60-80% asymptomatic after 1 year
o Interruption of nutritional supply to disc • Surgery
o Decreasing water content in nucleus pulposus o Indications
• Proteoglycans replaced by fibrocartilage and • Failed conservative therapy after 6-8 weeks
fibrosis • Progressive neurologic deficits
• Increasing stiffness o Lumbar
2 • Decreasing intra-disc pressure • Inter-Iaminal approach with partial medial
o Ineffective cushioning facetectomy
40 o Unequal force distribution to anulus • Pars interarticularis fenestration preserves facet
• Concentric tear ± radial tear ~ disc herniation integrity
• Epidemiology o Cervical
o Uncommon • Anterior or posterior approach
• 5-10% of all disc herniations • Laminotomy-foraminotomy
• Associated abnormalities • Discectomy
o Spondylolisthesis • Stabilization
o Scoliosis
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Typical
(Left) Sagittal T2WI MR
shows L4-5 disc Foraminal
disc herniation with
associated anular tear
(arrow). (Right) Axial
PO/Intermediate MR shows
a large right foraminal disc
herniation (arrows), effacing
perineural Fat. The exiting
nerve root cannot be
visualized.
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SPONDYLOLISTHESIS
2
42
Sagittal bone CT shows grade 4 spondylolisthesis due to Anteroposterior radiography shows "Napoleon's hat"
spondylolysis (open arrow). Chronicity of displacement sign. Hat is inverted; crown (arrows) is anterior cortex of
is evident by bony remodeling (arrow). vertebral body, and brim (open arrows) is transverse
process.
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SPONDYLOLISTHESIS
Key Facts
Terminology Top Differential Diagnoses
• Displacement of vertebral body, described relative to • Physiologic motion
inferior vertebra • Displaced vertebral body fracture
Imaging Findings Pathology
• Evaluate percentage or grade of listhesis on neutral, • Spondylolysis 2
flexion and extension lateral • Trauma at sites other than pars interarticularis
• Protocol advice: Thin-slice CT with sagittal • Degenerative 43
reformations • Postsurgical
Physiologic motion
• Slight subluxations normal in young patients ISELECTED REFERENCES
1. Lamm Met al: Acute traumatic L5-S1 spondylolisthesis. J
Displaced vertebral body fracture Spinal Disord Tech. 16(6):524-7, 2003
• Fracture often best seen on CT 2. Pneumaticos SG et al: Scoliosis assodated with lumbar
spondylolisthesis: a case presentation and review of the
literature. Spine]. 3(4):321-4, 2003
!PATHOlOGY 3. Bassewitz H et al: Lumbar stenosis with spondylolisthesis:
current concepts of surgical treatment. Clin OrthoP.
General Features (384):54-60, 2001
• Etiology 4. Nizard RS et al: Radiologic assessment of lumbar
intervertebral instability and degenerative
o Spondylolysis spondylolisthesis. Radiol Clin North Am. 39(1):55-71, v-vi,
• Acute trauma or fatigue fracture 2001
o Trauma at sites other than pars interarticularis 5. Cinotti G et al: Predisposing factors in degenerative
• Shear injury spondylolisthesis. A radiographic and CT study. lnt
• Fractures of posterior elements Orthop. 21(5):337-42, 1997
o Degenerative 6. Herkowitz HN: Spine update. Degenerative lumbar
• May be related to alignment of facet joints spondylolisthesis. Spine. 20(9):1084-90, 1995
o Postsurgical
• Destabilization of spine at wide laminectomy
• Late complication above or below fusion IIMAGE GAllERY
o Tumor
o Infection
o Neuropathic arthropathy
• Associated abnormalities: Scoliosis may develop
Staging, Grading or Classification Criteria
• Grade I: < 25% displacement vertebral body
• Grade II: 25-50% displacement vertebral body
• Grade III: 50-75% displacement vertebral body
• Grade IV: 75-100% displacement vertebral body
• Grade V: Spondyloptosis
I CLINICAL ISSUES (Left) Sagittal T7WI MR shows L5151 spondylolisthesis in patient run
over by a truck. Mixed low and high signal hematoma is present
Presentation behind L5 vertebral body and between posterior elements L5 and S 1
• Most common signs/symptoms: Back pain, (arrows). (Right) Sagittal STIR MR shows traumatic L5-51
radiculopathy, neurogenic claudication spondylolisthesis with large hematoma (arrow) deviating nerve roots
posteriorly. Posterior ligamentous disruption also evident (open
arrow).
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SPONDYLOLYSIS
2
44
Sagittal T2WI MR shows a well corticated linear defect Axial T2WI MR shows bilateral L5 pars defects with
in L5 pars interardcularis (arrow). elongated anterior-posterior dimension of the spinal
canal.
DDx: Spondylolisthesis
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SPONDYLOLYSIS
Key Facts
Terminology • Focally decreased signal in PIon sagittal and axial
• Isthmic spondylolysis imaging
• Defects in pars interarticularis (PI) thought to result • STIR: Marrow edema adjacent to pars defects
from repetitive stress injury • SPECT increases sensitivity
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SPONDYLOLYSIS
o 5-7% in general population o Have worsening slippage
• 22-44% in competitive athletes in diving, weight o To preserve neural function and prevent instability
lifting, wrestling • Posterior or posterolateral fusion
• Associated abnormalities • Stabilization with fusion or cast immobilization
o Scoliosis • Possible decompression
o 50% with spoqdylolisthesis
oSpina bifida occulta
2 o Scheuermann disease IDIAGNOSTIC CHECKLIST
46 Microscopic Features Image Interpretation Pearls
• New bone and cartilaginous matrix if healing present • Look for integrity of pars interarticular on sagittal MRI
• Fibrocartilaginous tissue within pars defects if not o At the level of facet joints
healed
ISELECTED REFERENCES
ICLINICAL ISSUES 1. McTimoney CA et al: Current evaluation and management
Presentation of spondylolysis and spondylolisthesis. Curr Sports Med
Rep. 2(1):41-6, 2003
• Most common signs/symptoms 2. Merbs CF: Asymmetrical spondylolysis. Am] Phys
o Chronic low back pain Anthropol. 119(2):156-74, 2002
• Usually presents during adolescent growth spurt 3. Logroscino G et al: Spondylolysis and spondylolisthesis in
o Other symptoms the pediatric and adolescent population. Childs Nerv Syst.
• Back spasm 17(11):644-55,2001
• Hamstring tightness 4. Standaert C] et al: Spondylolysis. Phys Med Rehabil Clin N
Am. 11(4):785-803,2000
• Radiculopathy and cauda equina syndrome
5. Saifuddin A et al: The value of lumbar spine MRI in the
• Gait disturbance assessment of the pars interarticularis. Clin Radiol.
• Clinical profile: Pain exacerbated by rigorous activities 52(9):666-71, 1997
• 80% without symptoms 6. Ulmer J et al: MR Imaging of Lumbar Spondylolysis: The
Importance of Ancillary Observations. A]R. 169:233-9,
Demographics 1997
• Age: 10-20 years 7. Ohmori K et al: Vertebral slip in lumbar spondylolysis and
• Gender: M:F = 2-3:1 spondylolisthesis. Long-term follow-up of 22 adult
• Ethnicity patients.] Bone Joint Surg Br. 77(5):771-3, 1995
o In 30-40% of Eskimos 8. Ulmer ]L et al: Distinction between degenerative and
o Higher incidence in Caucasian males: 6.4% isthmic spondylolisthesis on sagittal MR images:
importance of increased anteroposterior diameter of the
Natural History & Prognosis spinal canal ("wide canal sign"). A]R Am] Roentgenol.
163(2):411-6, 1994
• Progressive spondylolisthesis occurs during skeletal
9. Jinkins JR et al: Spondylolysis, spondylolisthesis, and
immaturity associated nerve root entrapment in the lumbosacral spine:
o More common in females MR evaluation. A]R Am] Roentgenol. 159(4):799-803, 1992
o Risk of progression: 3-28% 10. Reynolds R: Spondylolysis and Spondylolisthesis. Seminars
• Little slippage with horizontal sacrum in Spine Surgery. 4:235-47, 1992
o Lumbosacral angle ~ 100° 11. Danielson BI et al: Radiologic progression of isthmic
• Progressive spondylolisthesis with vertical sacrum lumbar spondylolisthesis in young patients. Spine.
o Lumbosacral angle < 100° 16(4):422-5,1991
• Progression from grade 1 spondylolisthesis: Superior 12. Frennered AK et al: Natural history of symptomatic isthmic
low-grade spondylolisthesis in children and adolescents: a
vertebral body subluxed by one-fourth of a vertebral seven-year follow-up study.] Pediatr Orthop. 11(2):209-13,
body 1991
• To grade 2: Subluxation by half a vertebral body 13. Rossi F et al: Lumbar spondylolysis: occurrence in
• To grade 3: Subluxation by three-fourths of a vertebral competitive athletes. Updated achievements in a series of
body 390 cases.] Sports Med Phys Fitness. 30(4):450-2, 1990
• To grade 4: Subluxation by whole width of a vertebral 14. Johnson D et al: MR Imaging of the Pars Interarticularis.
body AJR. 152:327-32, 1989
15. Rossi F: Spondylolysis, spondylolisthesis and sports. ]
Treatment Sports Med Phys Fitness. 18(4):317-40, 1978
• Conservative measures in patients with grade 1 and 2 16. Wiltse LL et al: Classification of spondylolisis and
spondylolisthesis. Clin Orthop. (117):23-9, 1976
spondylolisthesis 17. Wiltse LL: Spondylolisthesis. West] Med. 122(2):152-3,
o Modification of activity 1975
o Anti-inflammatory medications
o Epidural steroid injection
o Back brace
o Physical therapy
o Two-thirds success rate of symptomatic relief
• Surgical interventions in patients who
o Fail conservative treatment
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SPONDYLOLYSIS
IIMAGE GALLERY
Typical
(Left) Lateral radiography
shows a defect and mild
angulation in L5 pars
interarticularis (arrow).
There is disc height loss at
2
L5-S7 with associated 47
sclerotic endplate changes.
(Right) Coronal oblique
radiography shows a thin
lucency (arrow) in right L5
pars interarticularis with
adjacent bony sclerosis.
Typical
(Left) Sagittal bone CT with
sagittal reformation shows a
L5 pars defect without
anterolisthesis. (Right) Axial
bone CT at L5 shows a
left-sided pars defect.
Right-sided pars fracture has
healed.
Typical
(Left) Sagittal TlWI MR
shows a defect in L4 pars
interarticularis (arrow).
Fractures at this level are
much less common than
those at L5. (Right) Axial
bone scan with SPECT shows
increased radiotracer uptake
in bilateral L5 pars
interarticularis.
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2
48
Lateral radiography shows severe facet hypertrophic Axial NECT shows severe left facet degenerative
degenerative arthropathy at C2-3 (arrows). arthropathy with foraminal stenosis (arrow). "Vacuum
phenomenon" present in left facet joint.
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FACET ARTHROPATHY, CERVICAL
Key Facts
Terminology • Inflammatory arthritides
• Facet arthrosis, degenerative facet disease, • Paget disease
degenerative joint disease • Myositis ossificans
• Osteoarthritis of synovially-lined apophyseal joints • Tumor
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Typical
(Left) Axial NECT shows
severe left facet degenerative
arthropathy and foraminal
stenosis. "Vacuum
phenomenon" present in
2
facet joint and uncovertebral 51
joint (arrows). (Right) Axial
NECT shows severe,
exuberant right facet
degenerative arthropathy.
Typical
(Left) Axial CECT following
myelography shows bilateral
foraminal degenerative
change, stenosis with
relatively mild impingement
upon thecal sac. (Right)
Axial T2* eRE MR shows
severe right uncovertebral
hypertrophic degenerative
arthropathy with severe
foraminal stenosis (arrows).
Typical
(Left) Coronal NECT shows
multilevel severe facet
hypertrophic degenerative
arthropathy with foraminal
stenosis (arrows). (Right)
Sagittal NECT shows
hypertrophic facet
degenerative arthropathy
with "vacuum
phenomenon" (arrows).
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2
52
Axial TlWI MR shows marked facet hypertrophic Axial T2WI MR shows bilateral facet hypertrophy and
degenerative arthropathy with enlarged facets (arrows), small bilateral facet effusions (arrows).
producing foraminal stenosis (open arrows).
, ...,
..
~~.Z.
..... )
'.:.~.
. '" I .
,
. ' •.
Septic Facet Tumoral Calcinosis Tumor
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o Inflammatory soft tissue changes surrounding facet • Consider CT myelography if MRI contraindications or
joint are common MRI does not adequately demonstrate facet
• May appear aggressive even in absence of relationship to neural foramina
infection
MR Findings IDIFFERENTIAL DIAGNOSIS
• TlWI
o Joint space narrowing, thinning of articular cartilage Septic facet
• Conversely, some patients show variable synovial • T2 hyperintensity extending into soft tissues, epidural
thickening abscess/phlegmon
• Irritation of the synovium can produce synovial
hyperplasia with paradoxical joint space widening Inflammatory arthritides
• T2WI • Look for ankylosis/erosions involving facet and SI
o Osteophyte proliferation narrowing neural foramina joints
o Foraminal compromise often combination of disease
Paget disease
• Facet hypertrophic degenerative arthropathy,
particularly superior articular facet extending into • Thickened cortex, trabeculae, bony expansion
lumbar foramen Tumor
• Ligamentous buckling/thickening • Metastasis, lymphoma
• Disc bulge/osteophyte extending into inferior • Soft tissue enhancing mass, bone destruction
portion of lumbar foramen
• STIR: May show marrow hyperintensity due to edema, Tumoral calcinosis
type I endplate-like changes • Large, lobulated calcific mass, no enhancing soft tissue
• T2* GRE component (rare)
o Shows osseous changes well
o Tends to overemphasize the degree of foraminal or
central canal narrowing IPATHOLOGY
• Tl C+
o Enhancing inflammatory soft tissue changes General Features
surrounding facet joint not uncommon • General path comments
• May appear aggressive even in absence of o Normal bone mineralization (in contrast to
infection rheumatoid arthritis)
o Degenerative (hypertrophic) inflammatory changes
Nuclear Medicine Findings in synovial joints
• Bone Scan: Increased uptake with degenerative disc • Progression is similar to that seen in other joints
disease, facet arthrosis except for early focal full-thickness cartilage
Imaging Recommendations necrosis
o Joint traction during subluxation may produce gas
• Plain films useful to demonstrate presence and severity in joint ("vacuum phenomenon")
of facet degenerative changes o Associated with synovial cysts, degenerative disc
• Sagittal and axial TlWI and T2WI best demonstrate
disease, canal stenosis
degenerative facet compression of adjacent thecal sac
and fat-filled neural foramina
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FACET ARTHROPATHY, LUMBAR
IIMAGE GALLERY
(Left) Sagittal Tl WI MR
shows L4-5, L5-sl facet
degenerative arthropathy 2
with hypertrophy and low
signal. (Right) Sagittal Tl C+
MR shows enhancement of 55
the L4-5, L5-sl facets
(arrows) due to degenerative
arthropathy.
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FACET JOINT SYNOVIAL CYST
2
56
Axial graphic depicts right facet joint synovial cyst. Fluid Sagittal T2WI MR shows a slightly lobulated
expands right facet joint, extending into subarticular predominantly hyperintense mass in the spinal canal at
recess in a loculated collection, there is mass effect on U-5_
the thecal sac.
o Lobulated
ITERMINOLOGY o Sharply marginated
Abbreviations and Synonyms Radiographic Findings
• Facet joint ganglion cyst • Radiography
Definitions o Degenerative changes
• Synovial cyst formed from degenerative facet joint • Disc space loss
• Vacuum disc phenomenon
• Endplate sclerosis and spurring
IIMAGING FINDINGS • Facet arthropathy
• Spondylolisthesis without spondylolysis
General Features • Lateral listhesis
• Best diagnostic clue: Posterolateral extradural cystic • Scoliosis
mass communicating with facet joint • Worst at the level of synovial cyst
• Location • Myelography
o Posterolateral to thecal sac o Circumscribed posterolateral extradural mass
o Adjacent to facet joint o Central canal and subarticular narrowing
o Lumbar spine: 90% o High sensitivity
• 70-80% at L4-5 o Low specificity
• L3-4 and L5-S 1: Less common CT Findings
o Uncommon
• Cervical and thoracic spine • NECT
o Difficult to detect because of fluid density
• Foraminal o Increased density from hemorrhage
• Far lateral extra-foraminal
o Mural calcifications
• Bilateral
o Intra-cystic gas
• Size: 1-2 cm o Facet joint gas
• Morphology
• CECT: Mild rim-enhancement
o Round
• Bone CT
••
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r! -A'
I \Ct_'\;<
.~~,"."
•
~!~[\ .
•
~
Free Disc Fragment Ganglion Cyst Schwannoma Periarticuiar Abscess
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FACET JOINT SYNOVIAL CYST
Key Facts
Terminology Top Differential Diagnoses
• Synovial cyst formed from degenerative facet joint • Extruded disc fragment
• Ganglion cyst
Imaging Findings • Nerve sheath tumor
• Best diagnostic clue: Posterolateral extradural cystic
mass communicating with facet joint Pathology 2
• Lumbar spine: 900/0 • Invariably associated with degenerative disc and facet
• 70-80% at L4-S disease 57
• Increased density from hemorrhage
• Mural calcifications Clinical Issues
• Hyperintense • Chronic low back pain
• Low intensity rim • Acute pain from hemorrhage
• Tl C+: Enhancing wall • May spontaneously regress
• Well circumscribed • High post-surgical success rate in symptomatic
• Variable central canal, subarticular, or lateral recess patients
narrowing • Laminectomy with cyst excision
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FACET JOINT SYNOVIAL CYST
• Greatest mobility at L4-S
o Synovial proliferation Image Interpretation Pearls
o Cyst formation • Well-circumscribed T2 hyperintense mass
• Associated abnormalities • Thin hypointense rim
o Degenerative spondylolisthesis • Posterolateral to thecal sac
o Rheumatoid arthritis • Abutting facet joint
o Calcium pyrophosphate deposition disease
2 Gross Pathologic & Surgical Features ISELECTED REFERENCES
58 • Encapsulated 1. Houten JK et al: Spontaneous regression of symptomatic
• Serous, mucinous, ± hemorrhagic fluid lumbar synovial cysts. Report of three cases. J Neurosurg.
• White myxoid material 99(2 Suppl):235-8, 2003
2. Swartz PG et al: Spontaneous resolution of an intraspinal
Microscopic Features synovial cyst. AJNR Am J Neuroradiol. 24(6):1261-3, 2003
• Fibrous connective tissue 3. Gadgil AA et al: Bilateral symptomatic synovial cysts of the
• Inflammatory cell infiltration lumbar spine caused by calcium pyrophosphate deposition
• Calcific deposits disease: a case report. Spine. 27(19):E428-31, 2002
• Lined with hypervascular synovium 4. Phuong LK et al: Far lateral extraforaminallumbar synovial
cyst: report of two cases. Neurosurgery. 51(2):505-7;
discussion 507-8, 2002
5. Metellus P et al: An unusual presentation of a lumbar
I CLINICAL ISSUES synOVial cyst: case report. Spine. 27(1l):E278-80, 2002
6. Gishen P et al: Percutaneous excision of a facet joint cyst
Presentation under CT guidance. Cardiovasc Intervent Radiol.
• Most common signs/symptoms 24(5):351-53,2001
o Chronic low back pain 7. Tillich M et al: Symptomatic intraspinal synovial cysts of
o Other signs/symptoms the lumbar spine: correlation of MR and surgical findings.
• Acute pain from hemorrhage Neuroradiology. 43(12):1070-5, 2001
8. Bureau NJ et al: Lumbar facet joint synovial cyst:
• Radicular symptoms
percutaneous treatment with steroid injections and
• Neurogenic claudication distention--clinical and imaging follow-up in 12 patients.
• Cauda equina syndrome Radiology. 221(1):179-85, 2001
• Myelopathy if cervical or thoracic 9. Banning CS et al: Patient outcome after resection of lumbar
juxtafacet cysts. Spine. 26(8):969-72, 2001
Demographics 10. Trummer M et al: Diagnosis and surgical management of
• Age: ~ 60 intraspinal synOVial cysts: report of 19 cases. J Neurol
• Gender: More common in females Neurosurg Psychiatry. 70(1):74-7,2001
• Ethnicity: No racial predilection 11. Apostolaki E et al: MR imaging of lumbar facet joint
synovial cysts. Eur Radiol. 10(4):615-23,2000
Natural History & Prognosis 12. Bandiera S et al: Hemorrhagic synovial lumbar cyst: a case
• May spontaneously regress report and review of the literature. Chir Organi Mov.
• High post-surgical success rate in symptomatic 84(2):197-203, 1999
patients 13. Howington JD et al: Intraspinal synovial cysts: 10-year
experience at the Ochsner Clinic. J Neurosurg. 91(2
Treatment Suppl):193-9, 1999
14. Jonsson B et al: Lumbar nerve root compression by
• Conservative intraspinal synovial cysts. Report of 8 cases. Acta Orthop
o Bed rest Scand. 70(2):203-6, 1999
o Analgesics 15. Parlier-Cuau C et al: Symptomatic lumbar facet joint
o Epidural and facet injection synovial cysts: clinical assessment of facet joint steroid
o Bracing injection after 1 and 6 months and long-term follow-up in
• Laminectomy with cyst excision 30 patients. Radiology. 210(2):509-13, 1999
o In setting of intractable pain 16. Sampson MA et al: Acute extradural compression due to an
o To relieve nerve compression and central stenosis intraspinal synovial cyst: CT and myelogram appearances.
Clin Radiol. 41(6):433-4, 1990
o Hemilaminectomy and flavectomy also performed 17. Liu SS et al: Synovial cysts of the lumbosacral spine:
• Less permanent diagnosis by MR imaging. AJR Am J Roentgenol.
o Percutaneous cyst aspiration 154(1):163-6, 1990
• Under CT guidance 18. Jackson DE et al: Intraspinal Synovial Cysts: MR Imaging.
o Steroid injection into cyst Radiology 170 (2): 527-30, 1989
• Half to two-thirds of patients have excellent 19. Onofrio BM et al: Synovial cysts of the spine.
symptomatic relief after 6 months Neurosurgery. 22(4):642-7, 1988
20. Wang AM et al: Synovial cysts of the lumbar spine: CT
evaluation. Comput Radiol. 11(5-6):253-7, 1987
IDIAGNOSTIC CHECKLIST
Consider
• Thin-section sagittal T2WI may show communication
between facet joint and adjacent synovial cyst
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Typical
(Left) Axial TI WI M R shows
a large fluid intensity mass
(arrow) within left lateral
aspect of spinal canal with
significant mass effect.
2
(Right) Axial TlWI MR with 59
fat suppression shows a
hyperintense extradural mass
within right
subarticularllateral recess,
compatible with a
hemorrhagic synovial cyst.
Typical
(Left) Axial T2WI MR shows
a well-circumscribed
hyperintense mass with a
hypointense rim abutting left
L4-5 facet joint, narrowing
the central canal. (Right)
Axial T2WI MR shows
bilateral subarticular
hyperintense masses
(arrows) at L4-5, consistent
with bilateral facet synovial
cysts.
Typical
(Left) Axial CECT shows a
right-sided circumscribed
juxta-articular mass (arrow),
slightly hyperdense than
CSF, with a thin rim of mild
enhancement, causing
subarticular stenosis. (Right)
Axial bone CT after
myelography with coronal
reformation shows an
extradural mass abutting
right L4-5 facet joint,
distorting thecal sac.
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ACQUIRED SPINAL STENOSIS, LUMBAR
2 ~ ~ ~
~~ ~.~
60
. y , '\ .
·
,I,
,
~
;t,', ri', ~'t'IIJ,.I
., ~
'T
.
, ~~."" ~J
_ ..~.J
j ~- : ,
Axial TlWI MR shows severe central canal stenosis due Axial T1WI MR shows central canal stenosis primarily
to bulging anulus (arrows) and posterior facet related to facet and ligamentous hypertrophy/thickening
hypertrophic degenerative arthopathy combining to with little ventral epidural disease,
compress the thecal sac.
,,~ .~
.~
Epidural Abscess Herniation Metastatic Paget Disease
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ACQUIRED SPINAL STENOSIS, LUMBAR
Key Facts
Terminology • Bilateral lower extremity pain, paresthesia, and
• Spondylosis, central canal stenosis, lumbar canal weakness
stenosis • Exacerbation by prolonged standing and walking
• Spinal canal narrowing in lumbar spine • Relieve of pain by squatting or sitting (flexion) in
80%
• Secondary to multifactorial degenerative changes,
being progressive and dynamic process • Degree of spinal stenosis on imaging may not 2
correlate with symptomatology
Imaging Findings • Majority of symptomatic patients stable over months 61
• Best diagnostic clue: Trefoil appearance of lumbar to years (40-70%)
spinal canal on axial imaging • History of back pain < 5 years and leg pain < 6
• Location: Most common in lower lumber spine where months have success with nonoperative treatment
there is most mobility (L4-5) • Operative treatment primarily surgical decompression
• Sagittal diameter of lumbar canal less than 12 mm with laminectomies and fusion
relative stenosis • > 70% complete relief of preoperative symptoms
• Other surgery: Expansive laminoplasty or fenestration
Clinical Issues procedure
• Chronic low back pain
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ACQUIRED SPINAL STENOSIS, LUMBAR
IIMAGE GALLERY
Typical
(Left) Axial T2WI MR shows
severe central canal stenosis
with marked facet
arthopathy. The thecal sac 2
has assumed a trefoil
appearance. (Right) Sagittal 63
T2WI MR shows severe
central stenosis L4-5 due to
marked posterior
ligamentum flavum
thickening (arrow).
Hyperintensity within
interspinous ligament
denotes degeneration (open
arrow).
Typical
(Left) Axial T2WI MR shows
severe central stenosis with
disc bulge, facet arthropathy,
ligamentous thickening.
Right synovial cyst further
compresses thecal sac
(arrow). Right facet effusion
(open arrow). (Right) Sagittal
T2WI MR shows canal
stenosis at LJ-4 with bulging
disc, ligamentous thickening
(arrow). Serpentine areas of
low signal within cephalad
thecal sac due to redundant
nerve roots (open arrows).
Typical
(Left) Axial T1 C+ MR shows
central canal stenosis with
facet arthropathy and
ligamentous thickening.
There is diffuse enhancement
of the intrathecal roots
(arrow). (Right) Sagittal T1
C+ MR shows focal
enhancement of the roots at
the stenotic L4-5 level.
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ACQUIRED SPINAL STENOSIS, CERVICAL
2
64
Sagittal graphic shows disc degeneration with Sagittal STIR MR shows severe central canal stenosis at
osteophyte formation causing central stenosis and cord C3-4, C4-5 with marked narrowing of subarachnoid
compression at multiple levels (cervical spondylosis), space, Hyperintensity within cord at C4-5 reflects
contusion/myelomalacia,
• Myelography
ITERMINOlOGY o Obliterated subarachnoid space at disc levels
Abbreviations 'and Synonyms o Multiple horizontal "bars" or ridges on AP view due
to subarachnoid space narrowing
• Acquired spinal stenosis, cervical (ASSC)
o Cord widened on AP view due to compression
• Cervical spondylosis; cervical spondylotic myelopathy
o Multiple levels of root cutoff
Definitions
CT Findings
• Spinal canal and neural foraminal narrowing in
• NECT
cervical spine secondary to multifactorial degenerative
o Disc-osteophyte complex protruding into the canal
changes
and compressing thecal sac and cord
o Uncovertebral and facet joint hypertrophy
o Narrowing of the neural foramina
IIMAGING FINDINGS
MR Findings
General Features
• Best diagnostic clue: Completely effaced cerebral • TlWI
o Completely effaced subarachnoid space at disc levels
spinal fluid in cervical spine at disc levels in cervical spine ~ "washboard spine"
• Location: Cervical spine, ventral epidural, centered at o Variable degree of cord compression
disc levels o Disc disease: Disc herniations, osteophytes, disc
• Size: Sagittal diameter of cervical canal < 13 mm bulges
• Morphology
o Extradural degenerative change compreSSing thecal • T2WI
o Intramedullary T2 hyperintensity represents
sac and cord myelomalacia, demyelination, or edema
o Congenitally short pedicles often contribute to o Narrowing of subarachnoid space due to ventral and
acquired spinal stenosis dorsal epidural disease
Radiographic Findings • Ventral low T2signal due to disc/osteophyte
• Radiography: Disc space height loss, osteophyte complex
extending into canal, multilevel degenerative change
DDx: Myelopathy
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ACQUIRED SPINAL STENOSIS, CERVICAL
Key Facts
Terminology • Dorsal low T2 signal due to ligamentous thickening
• Acquired spinal stenosis, cervical (ASSC) • Tl C+: Cord myelomalacia, contusion may enhance
• Cervical spondylosis; cervical spondylotic after gadolinium
myelopathy Clinical Issues
• Spinal canal and neural foraminal narrowing in
cervical spine secondary to multifactorial
• ASSC has no pathognomic symptoms or signs
• Spastic paraparesis commonly seen
2
degenerative changes • Natural history of insidious onset, periods of static 65
Imaging Findings disability, and episodic worsening
• Best diagnostic clue: Completely effaced cerebral • New signs and symptoms (75%)
spinal fluid in cervical spine at disc levels • Slow steady progression of disability (20%)
• Intramedullary T2 hyperintensity represents • Rapid onset with long period of stability (5%)
myelomalacia, demyelination, or edema Diagnostic Checklist
• Narrowing of subarachnoid space due to ventral and • OPLL if confluent anterior epidural disease
dorsal epidural disease • MRI of cervical spine may overestimate neural
• Ventral low T2 signal due to disc/osteophyte complex foraminal stenosis
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ACQUIRED SPINAL STENOSIS, CERVICAL
• Spastic paraparesis commonly seen o Airway obstruction
• Upper extremity numbness, weakness o Adjacent level degeneration
("myelopathic hand") o Reduced mobility
• Gait disturbance, loss of position and vibration
sense
• Chronic neck pain radiating to occiput and upper IDIAGNOSTIC CHECKLIST
extremities
2 o Cord syndromes Consider
• Transverse: Severe spasticity, sphincter • OPLL if confluent anterior epidural disease
66 involvement, Lhermitte
Image Interpretation Pearls
• Motor system: Spasticity but minimal sensory
disturbance • MRI of cervical spine may overestimate neural
• Central cord: Severe motor and sensory foraminal stenosis
disturbance, worse for upper extremities o Short TE on axial T2* images decreases this artifact
• Brown-Sequard: Contralateral sensory and
ipsilateral motor deficits
• Brachialgia cord syndrome: Lower motor neuron ISELECTED REFERENCES
~ upper extremity involvement, upper motor 1. Mayr MT et al: Cervical spinal stenosis: outcome after
neuron ~ lower extremity involvement, radicular anterior corpectomy, allograft reconstruction, and
pain instrumentation. ] Neurosurg. 96(1 Suppl): 10-6, 2002
2. Tani S et al: Laminoplasty with preservation of posterior
• Clinical profile: Neck pain, "heaviness" in arms, ataxic
cervical elements: surgical technique. Neurosurgery. 50(1):
gait, hyperreflexia, extensor plantar reflex 97-101; discussion 101-2, 2002
Demographics 3. Edwards CC 2nd et al: Corpectomy versus laminoplasty for
multilevel cervical myelopathy: an independent
• Age: > 50 matched-cohort analysis. Spine. 27(11): 1168-75,2002
• Gender: M > F 4. Geck M] et al: Surgical options for the treatment of cervical
spondylotic myelopathy. Orthop Clin North Am. 33(2):
Natural History & Prognosis 329-48, 2002
• Natural history of insidious onset, periods of static 5. Handa Y et al: Evaluation of prognostic factors and clinical
disability, and episodic worsening outcome in elderly patients in whom expansive
o New signs and symptoms (75%) laminoplasty is performed for cervical myelopathy due to
o Slow steady progression of disability (20%) multisegmental spondylotic canal stenosis. A retrospective
o Rapid onset with long period of stability (5%) comparison with younger patients. ] Neurosurg. 96(2
Suppl): 173-9,2002
• Continued pain, disability and poor outcomes
6. Epstein N: Posterior approaches in the management of
(30-50%) cervical spondylosis and ossification of the posterior
• More favorable outcome if treated early longitudinal ligament. Surg Neurol. 58(3-4): 194-207;
• Severity and duration of pre-operative neurologic discussion 207-8,2002
deficits predictive of the degree of neurologic recovery 7. Vue WM et al: The Torg--Pavlov ratio in cervical
spondylotic myelopathy: a comparative study between
Treatment patients with cervical spondylotic myelopathy and a
• Cervical spine nonspondylotic, nonmyelopathic population. Spine.
o Medications 26(16): 1760-4,2001
• Anti-inflammatories, narcotics, antidepressants, 8. Emery SE: Cervical spondylotic myelopathy: diagnosis and
muscle relaxants treatment.] Am Acad Orthop Surg. 9(6): 376-88, 2001
9. Fouyas IP et al: Surgery for cervical radiculomyelopathy.
o Soft collar immobilization and traction Cochrane Database Syst Rev. (3): CD001466, 2001
o Physical therapy 10. Satomi K et al: Short-term complications and long-term
• Isometrics results of expansive open-door laminoplasty for cervical
• Aerobic conditioning stenotic myelopathy. Spine]. 1(1): 26-30, 2001
• Flexibility exercises 11. Kawakami M et al: A comparative study of surgical
o Depending on site of compression approaches for cervical compressive myelopathy. Clin
• Anterior corpectomy or interbody arthrodesis Orthop. (381): 129-36, 2000
12. Alfieri KM et al: MR imaging of spinal stenosis. Applied
• Posterior decompression with laminectomies
Radiology. August:18-26, 1997
• Open-door laminaplasties with or without 13. Amunosen T et al: Lumbar spinal stenosis: clinical and
foraminotomies radiologic features. Spine 20:1178-86, 1995
• Prognostic factors for good surgical outcome 14. Modic MT et al: Imaging of degenerative disease of the
o Young age cervical spine. Clin Orthop 239:109-20, 1989
o < 1 year of symptoms
o Unilateral motor deficit
o Presence of Lhermitte sign
• Post-operative complications
o Neurologic deterioration (5%)
o Vertebral artery injury
o Graft extrusion or fracture
o Instrumentation failure or migration
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ACQUIRED SPINAL STENOSIS, CERVICAL
IIMAGE GALLERY
Typical
(Left) Sagittal T1WI MR
shows severe multilevel disc
degeneration with severe 2
central canal stenosis at
0-4, C4-5 with cord
compression. Note 67
calcification/ossification of
C2-3 disc. (Right) Sagittal
T2WI MR shows severe
central stenosis with
obliteration of subarachnoid
space at C3-5, C4-5 with
ventral disc/osteophyte
complexes.
Typical
(Left) Axial T2* eRE MR
shows marked central
stenosis with large ventral
disc/osteophyte (arrow).
There is bilateral foraminal
stenosis. (Right) Sagittal
T2WI MR shows narrowing
at 0-4 through C5-6 with
disc/osteophyte complex
and hyperintense cord
contusion/myelomalacia
(arrows). Type I
degenerative endplate
change at C4-5 (open
arrow).
(Left) Sagittal T1 C+ MR
shows enhancement of cord
contusion (arrowj. Extradural
disease is of low signal and
not well defined on T1 W
sequence. (Right) Sagittal
STIR MR shows severe
multilevel stenosis, worst at
C2-3, 0-4. Cord
hyperintensity present at
both levels. Congenital
fusion of C4 and C5 bodies
accelerates degenerative
process.
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DEGENERATIVE SCOLIOSIS
2
68
Coronal TlWI MR shows moderate levoscoliosis Sagittal T2WI MR in the same patient shows 1055 of
centered at LJ-4. There is right lateral listhesis of L7 on lumbar lordosis with multi-level disc space narrowing
L2, and L2 on LJ. Disc height 1055 and degenerative and degenerative endplate changes.
endplate changes are present.
DDx: Scoliosis
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DEGENERATIVE SCOLIOSIS
Key Facts
Terminology • Variable central stenosis
• Lateral curvature in spine due to degenerative disc • Subarticular recess stenosis
and facet disease in older patients • Neural foraminal stenosis
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DEGENERATIVE SCOLIOSIS
o May have implications on disease progression
Dysplasias
• Neurofibromatosis type 1 (NFl), Marfan syndrome, Treatment
Ehlers-Danlos syndrome • Non-operative
• Posterior vertebral scalloping o Physical therapy
o Dural ectasia o NSAIDs
• High thoracic acute curvature in Nfl o Epidural injection
2 o Associated with wedge-shaped vertebra, block
vertebra, spina bifida occulta
o Brace
• Surgery
70 o Preserves neural function and prevents instability
o Decompression
!PATHOLOGY • Alone or with fusion
• Posterior or in combination with anterior fusion
General Features • With instrumentation
• General path comments: Degenerative disease of spine
• Etiology
o Degenerative disc at L3-4 and L4-5
o Facet arthropathy
IDIAGNOSTIC CHECKLIST
o Loss of lordosis Image Interpretation Pearls
o Instability in sagittal and coronal plane • Presence of extensive degenerative disc disease and
• Leading to spondylolisthesis and scoliosis facet arthropathy indicates degenerative scoliosis
• Epidemiology: Prevalence around 6%
• Associated abnormalities
o Osteoarthritis ISELECTED REFERENCES
• Hip and knee 1. Tsuchiya K et al: Application of multi-detector row helical
• May be unilateral scanning to postmyelographic CT. Eur Radiol.
o Spondylosis 13(6):1438-43, 2003
• Cervical and thoracic 2. Bartynski WS et al: Lumbar root compression in the lateral
o No association with osteoporosis recess: MR imaging, conventional myelography, and CT
myelography comparison with surgical confirmation. AJNR
Am J Neuroradiol. 24(3):348-60, 2003
ICLINICAL ISSUES 3. Gupta MC: Degenerative scoliosis. Options for surgical
management. Orthop Clin North Am. 34(2):269-79, 2003
4. Daffner SD et al: Adult degenerative lumbar scoliosis. Am J
Presentation Orthop. 32(2):77-82; discussion 82, 2003
• Most common signs/symptoms 5. Murata Y et al: Changes in scoliotic curvature and lordotic
o Low back pain angle during the early phase of degenerative lumbar
o Other signs/symptoms scoliosis. Spine. 27(20):2268-73, 2002
• Radiculopathy 6. Willen J et al: The diagnostic effect from axial loading of
• Neurogenic claudication the lumbar spine during computed tomography and
magnetic resonance imaging in patients with degenerative
• Deformity and waist asymmetry
disorders. Spine. 26(23):2607-14, 2001
• Gait disturbance 7. McPhee IE et al: The surgical management of degenerative
• Clinical profile lumbar scoliosis. Posterior instrumentation alone versus
o Pain worse with prolonged spinal extension two stage surgery. Bull Hosp Jt Dis. 57(1):16-22, 1998
o Radiculopathy not reliably relieved by flexion 8. Duance VC et al: Changes in collagen cross-linking in
• In contrast to spinal stenosis without scoliosis degenerative disc disease and scoliosis. Spine.
23(23):2545-51, 1998
Demographics 9. Pritchett JW et al: Degenerative symptomatic lumbar
• Age: Sixth decade scoliosis. Spine. 18(6):700-3, 1993
• Gender: M < F 10. Thornbury JR et al: Disk-caused nerve compression in
• Ethnicity: More common in Caucasians patients with acute low-back pain: diagnosis with MR, CT
myelography, and plain CT. Radiology. 186(3):731-8, 1993
Natural History & Prognosis 11. OgilVie JW: Adult scoliosis: evaluation and nonsurgical
treatment. Instr Course Lect. 41:251-5, 1992
• Curvature may regress Aebi M: Correction of degenerative scoliosis of the lumbar
12.
o Especially in early course of disease spine. A preliminary report. Clin Orthop. (232):80-6, 1988
• Risk factors for curve progression 13. Grubb SA et al: Degenerative adult onset scoliosis. Spine.
o Cobb angle> 30 degrees 13(3):241-5, 1988
o Laterallisthesis > 6 mm 14. Gillespy T 3rd et al: Progressive senile scoliosis: seven cases
o Grade 2 or 3 vertebral rotation of increasing spinal curves in elderly patients. Skeletal
o Inter-crest line through L5 or L4-5 disc space Radiol. 13(4):280-6, 1985
• 3 degrees per year 15. Robin GC et al: Scoliosis in the elderly: a follow-up study.
Spine. 7(4):355-9, 1982
• 73% of patients
• Biochemical changes in cross-link profile of
intervertebral discs
o Tissue remodeling
o Increased tissue turnover
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DEGENERATIVE SCOLIOSIS
IIMAGE GALLERY
Typical
(Left) Coronal T1WI MR
shows mild levoscoliosis
centered at L2-3. There is left
laterallisthesis of L4 on L5
2
with multi-level degenerative
disc and endplate disease. 71
(Right) Coronal T1WI MR
shows dextroscoliosis
centered at L2-3.
Asymmetric disc space
narrowing and degenerative
endplate changes are
evident.
Typical
(Left) Anteroposterior
radiography shows lumbar
rotatory levoscoliosis with
associated disc height 1055
and endplate sclerosis. There
is left laterallisthesis of L4 on
5. (Right) Lateral radiography
shows 1055 of lumbar lordosis
with multi-level disc space
1055 and degenerative
endplate changes, worst at
L2-3.
Typical
(Left) Axial bone CT with
coronal reformation shows
mild levoscoliosis at L4-5
with vacuum disc
phenomenon and right-sided
endplate sclerosis. There is
disc height 1055 at U-4 and
L4-5. (Right) Sagittal T1WI
MR shows grade 7
anterolisthesis of L4 on 5.
Degenerative disc and
endplate disease is present at
L2-3, L4-5, and L5-57.
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NEUROGENIC (CHARCOT) ARTHROPATHY
2
72
Sagittal graphic shows arthropathy centered at 3 Lateral radiography shows neuropathic spine secondary
adjacent discs with endplate erosions, bony debris, and to traumatic paraplegia. Note bony debris (arrows)
deformity sclerosis, and destructive arthropathy with
malalignment.
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• STIR: Similar to T2WI but more pronounced edema • Amorphous calcifications in intraosseous and soft
• Tl C+ tissue cold abscesses
o Enhancement may mimic infection, but often more • Cultures often negative
diffuse • Pulmonary tuberculosis may be absent
o Enhancement centers on disc, facet joints • Rely on histology rather than culture for diagnosis
o Fluid collections tend not to enhance as much as an
abscess Soft tissue sarcoma: Malignant fibrous
o Chronic, inactive neuropathic joint shows little or histiocytoma or osteosarcoma
no enhancement • Reactive spindle cells in soft tissue mass of Charcot
joint may be misdiagnosed as sarcoma by unwary
Nuclear Medicine Findings pathologists
• Bone Scan: Positive on all three phases • Centered in soft tissues
• PET: Uptake with FDG • Soft tissue sarcomas rarely destroy intervertebral discs
• Gallium scan or facet joints
o Positive
• WBC scan Bone tumor
o Positive in active neuropathic joint • Rare to involve two adjacent vertebrae
• Tends to spare disc space
Imaging Recommendations
• Best imaging tool: CT: Preserved bone density, bony Degenerative disc disease (DOD) with
debris best seen on CT, helps distinguish from instability
infection
• Severe DDD may show endplate erosion, especially if
• Protocol advice: Thin-section CT with reformatted instability present
images in coronal and sagittal planes
• Erosion similar to very early neuropathic joint
however, other signs of neuropathic joint absent
• DDD slowly progressive
IDIFFERENTIAL DIAGNOSIS
Pyogenic infection
• Bones usually osteopenic but occasionally see IPATHOLOGY
sclerosing variant General Features
• Bone destruction extends across disc space
• General path comments
• May see cloaca, sequestrum in bone o Occurs when there is preserved mobility with
• Paraspinous soft tissue abscess shows impaired proprioception and pain
rim-enhancement o Because proprioception is impaired, joint is stressed
• Uncommon to destroy both discs and facet joints beyond normal range of motion
except in patients with failed fusion o Because pain is diminished or absent, patient does
• Fever, elevated ESR and CRP useful not self protect following minor injury
• Cultures negative in 50% o Abnormal joint motion results in
Atypical infection: Tuberculosis, fungi • Disruption of joint capsule and stabilizing
• Bone density preserved ligaments
• Paraspinous soft tissue mass • Microfractures and/or major fractures
o Fractures fail to heal because of continued motion
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NEUROGENIC (CHARCOT) ARTHROPATHY
o Motion at fracture leads to
• Exuberant, nonbridging peripheral callus
IDIAGNOSTIC CHECKLIST
formation Consider
• Joint subluxation and/or dislocation • Distinction between neuropathic joint and infection is
• Soft tissue reparative response one of most difficult problems radiographically
• Nonunited bone fragments (bone debris) • Important differential diagnostic consideration for
• Etiology
2 o Diabetes Mellitus: Most common cause
infection when patient
o Has predisposing cause for neuropathic joint
• Diabetic neuropathy in chronic diabetes mellitus o Has no systemic signs of infection
74 o Neurosyphilis: Originally described by Charcot o Has negative cultures (percutaneous or open)
• More commonly involves hips, knees than spine o Has preserved bone density, disorganization and
o Following traumatic paraplegia bone debris
• Paraplegic patients who are physically active put
stress on insensate spine Image Interpretation Pearls
• Seen> 5 years following traumatic paraplegia • Only two processes can destroy a joint in a month:
• Below level of loss of sensation Infection and neurogenic arthropathy
• Often presents as instability in wheel chair • Nuclear medicine studies unreliable in differentiation
o Congenital insensitivity to pain of neuropathic joint from infection
o Other causes or neuropathic joint occur in
peripheral joints but not in spine
Gross Pathologic & Surgical Features
ISELECTED REFERENCES
• Bone sclerosis due to 1. Wagner SC et al: Can imaging findings help differentiate
spinal neuropathic arthropathy from disk space infection?
o Affected level continues to be stressed, Le., no disuse
Initial experience. Radiology. 214(3):693-9, 2000
osteopenia 2. Palestro CJ et al: Radionuclide imaging in orthopedic
o Callus formation infections. Semin Nucl Meei. 27(4):334-45, 1997
o Bone debris 3. Standaert C et al: Charcot spine as a late complication of
traumatic spinal cord injury. Arch Phys Med Rehabil.
Microscopic Features 78(2):221-5, 1997
• Bone and cartilage fragments 4. Arnold PM et al: Surgical management of lumbar
• Histiocytes and lymphoplasmacytic infiltrate neuropathic spinal arthropathy (Charcot joint) after
• Fibrosis, granulation tissue without acute traumatic thoracic paraplegia: report of two cases. J Spinal
inflammation Disord. 8(5):357-62, 1995
• Mimics chronic infection 5. Park YH et al: Imaging findings in spinal
neuroarthropathy. Spine. 19(13):1499-504, 1994
6. Heggeness MH: Charcot arthropathy of the spine with
resulting paraparesis developing during pregnancy in a
ICLINICAllSSUES patient with congenital insensitivity to pain. A case report.
Spine. 19(1):95-8, 1994
Presentation 7. Montgomery TJ et al: Traumatic neuropathic arthropathy
• Most common signs/symptoms of the spine. Orthop Rev. 22(10):1153-7, 1993
o Instability of spine 8. Harrison MJ et al: Spinal Charcot arthropathy.
o Spine deformity Neurosurgery. 28(2):273-7, 1991
o Some cases are painless 9. Crim JR et al: Spinal neuroarthropathy after traumatic
paraplegia. AJNR Am J Neuroradiol. 9(2):359-62, 1988
o Pain may be present 10. Kalen V et al: Charcot arthropathy of the spine in
• Common misconception is that pain must be long-standing paraplegia. Spine. 12(1):42-7, 1987
absent to make diagnosis of neuropathic joint 11. Fishel B et al: Multiple neuropathic arthropathy in a
• Pain is, however, less severe than expected for patient with syphilis. Clin Rheumatol. 4(3):348-52, 1985
severity of joint destruction
• Clinical profile
o Middle-aged diabetic patient with nonspecific back
pain
o Paraplegic patient complaining of instability while
seated
Natural History & Prognosis
• Treated with rigid internal fixation and fusion of
affected spinal levels
• Most cases do well following fusion
• May become superinfected
Treatment
• Options, risks, complications
o Fusion with rigid internal fixation
o Can also be treated with bracing, but success limited
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Typical
(Left) Sagittal STIR MR
shows neuropathic joint at
L2-3; paraplegic patient.
Chronic fluid collection
(arrow) was sterile.
Granulation tissue (open
arrow) was present
posteriorly. (Right) Sagittal
bone CT shows neuropathic
joint in paraplegic patient.
Note preserved bone
density, bony debris
(arrows) and gross instability
of all three columns.
Typical
(Left) Lateral radiography
shows endplate destruction
(arrow), preserved bone
density and instability in
diabetic patient. Multiple
cultures were negative.
Patient did well following
fusion. (Right) Axial bone CT
shows nonunited fractures,
bone debris, preserved bone
density, and destruction of
facet joints (arrows) in
diabetic patient with L5-S7
neuropathic joint.
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DISH
2
76
Sagittal graphic shows bulky flowing ossificauon of the Axial bone CT of the thoracic spine demonstrates
anterior longitudinal ligament extending over more than characteristic right-sided flowing ALL ossificauon. The
four conuguous vertebra. Disc spaces are relatively left side adjacent to the aorta is typically spared.
preserved.
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DISH
Key Facts
Terminology Pathology
• Diffuse idiopathic skeletal hyperostosis (DISH), senile • ! Spine mobility 2° ALL ossification; relative
ankylosing hyperostosis, asymmetrical skeletal protection from degenerative disc disease & facet
hyperostosis, Forestier disease arthropathy
Imaging Findings Clinical Issues 2
• Best diagnostic clue: Flowing anterior vertebral • Frequently incidental finding; patient may complain
ossification with relatively minimal degenerative disc of intermittent spinal stiffness, restricted mobility 77
disease, facet arthropathy, and absent facet ankylosis • Pain related to associated enthesitis, tendinitis
• Location: Thoracic spine (100%) > cervical (65-80%), • Anterior osteophytes may compress esophagus ~
lumbar spine (68-90%); R> L dysphagia
• No canal stenosis unless concurrent OPLL
Diagnostic Checklist
Top Differential Diagnoses • DISH is largely asymptomatic and incidentally
• Spondylosis detected
• Ankylosing spondylitis • Look carefully for signs of associated OPLL ~
• Psoriatic or reactive (Reiter) arthritis myelopathy 2° to central canal stenosis
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DISH
Gross Pathologic & Surgical Features I DIAGNOSTIC CHECKLIST
• Bulky anterior vertebral ossification with normal
cortical bone, marrow appearance Consider
• DISH is largely asymptomatic and incidentally
Microscopic Features detected
• Normal-appearing Haversian bone, marrow within o Look carefully for signs of associated OPLL =>
2 ossified ligament myelopathy 2° to central canal stenosis
Staging, Grading or Classification Criteria Image Interpretation Pearls
78 • Three primary diagnostic criteria for DISH • Flowing ossification classically R > L
o Flowing anterior ossification extending over at least • Discs/facet joints relatively preserved, no SI joint
4 contiguous vertebral bodies erosion or ankylosis
o No apophyseal or SI joint ankylosis • May co-exist with degenerative spondylosis
o Relativelyminimal degenerative disc changes, no
facet ankylosis
ISELECTED REFERENCES
1. Mader R: Diffuse idiopathic skeletal hyperostosis: a distinct
ICLINICAL ISSUES clinical entity. Isr Med Assoc]. 5(7):506-8, 2003
2. Belanger TA et al: Diffuse idiopathic skeletal hyperostosis:
Presentation musculoskeletal manifestations. J Am Acad Orthop Surg.
• Most common signs/symptoms 9(4):258-67,2001
o Frequently incidental finding; patient may complain 3. Le Hir PX et al: Hyperextension vertebral body fractures in
of intermittent spinal stiffness, restricted mobility diffuse idiopathic skeletal hyperostosis: a cause of
intravertebral fluidlike collections on MR imaging. AJR Am
• Often worse in morning, after prolonged sitting,
J Roentgenol. 173(6):1679-83, 1999
or during cold winter weather 4. Papakostas K et al: An unusual case of stridor due to
• May be relieved by mild activity osteophytes of the cervical spine: (Forestier's disease). J
o Pain related to associated enthesitis, tendinitis LaryngolOtol. 113(1):65-7, 1999
o Other signs/symptoms 5. Ehara S et al: Paravertebral ligamentous ossification: DISH,
• Anterior osteophytes may compress esophagus => OPLL and OLP. Eur J Radiol. 27(3):196-205, 1998
dysphagia 6. Cammisa M et al: Diffuse idiopathic skeletal hyperostosis.
• Stridor (extremely rare) requiring tracheostomy Eur J Radiol. 27 Suppl1:S7-11, 1998
7. Weinfeld RM et al: The prevalence of diffuse idiopathic
• Clinical profile: Usually incidental observation in
skeletal hyperostosis (DISH) in two large American
asymptomatic Caucasian patient imaged for unrelated Midwest metropolitan hospital populations. Skeletal
reasons Radiol. 26(4):222-5, 1997
8. Mata S et al: A controlled study of diffuse idiopathic
Demographics skeletal hyperostosis. Clinical features and functional
• Age: Middle-age => older adults; uncommon before age status. Medicine (Baltimore). 76(2):104-17, 1997
SO, common in elderly 9. Van Dooren-Greebe RJ et al: Prolonged treatment with oral
• Gender: M:F = 2:1 retinoids in adults: no influence on the frequency and
• Ethnicity severity of spinal abnormalities. Br J Dermatol. 134(1):71-6,
o Caucasian> > African-American, Native-American or 1996
Asian populations 10. McCafferty RR et al: Ossification of the anterior
longitudinal ligament and Forestier's disease: an analysis of
o ! Incidence in osteoporotic Caucasian populations
seven cases. J Neurosurg. 83(1):13-7, 1995
Natural History & Prognosis 11. Nesher G et al: Rheumatologic complications of vitamin A
and retinoids. Semin Arthritis Rheum. 24(4):291-6, 1995
• Nearly always incidental finding without additive 12. Burkus JK et al: Hyperextension injuries of the thoracic
morbidity or mortality spine in diffuse idiopathic skeletal hyperostosis. Report of
o Severe cases may rarely produce dysphagia four cases. J Bone Joint Surg Am. 76(2):237-43, 1994
o May be mildly increased predisposition to fracture 2° 13. Mata S et al: Chest radiographs as a screening test for
decreased flexibility diffuse idiopathic skeletal hyperostosis. J Rheumatol.
o 1 Risk of heterotopic ossification after total hip 20(11):1905-10, 1993
arthroplasty 14. Ramos-Remus C et al: Radiologic features of DISH may
mimic ankylosing spondylitis. Clin Exp Rheumatol.
Treatment 11(6):603-8, 1993
• Vast majority of cases incidental => merit conservative 15. Schlapbach P et al: Diffuse idiopathic skeletal hyperostosis
(DISH) of the spine: a cause of back pain? A controlled
(observational) management study. Br J Rheumatol. 28(4):299-303, 1989
• Consider osteophyte resection if severe symptoms 16. Resnick D et al: Association of diffuse idiopathic skeletal
directly referable to DISH hyperostosis (DISH) and calcification and ossification of
the posterior longitudinal ligament. AJR Am J Roentgenol.
131(6):1049-53, 1978
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DISH
IIMAGE GALLERY
Typical
(Left) Sagittal bone CT to the
right of midline reveals
flowing ALL ossification over 2
more than 4 contiguous
levels, typical of DISH.
(Right) Sagittal T1WI MR 79
right parasagittal slice shows
bulky flowing ALL
ossification (arrows)
spanning more than four
vertebral levels, but minimal
disc abnormality typical of
DISH.
Variant
(Left) Lateral radiography
shows a large anterior
ossified mass that is
discontinuous at several disc
spaces, a variation implying
some degree of continued
cervical spine mobility.
(Right) Lateral radiography
depicts typical ALL
ossification (DISH, arrows)
as well as concurrent PLL
ossification (OPLL, open
arrows).
Variant
(Left) Sagittal T1WI MR
shows bulky anterior flowing
ossification (arrows)
consistent with DISH. Fatty
marrow is responsible for
high T1 signal intensity.
(Right) Axial T2* eRE MR
demonstrates large bulky
ossification (arrows) of ALL,
displacing the aerodigestive
tract anteriorly.
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OPLL
2
80
Sagittal graphic shows flowing multilevel ossification Sagittal T2WI MR demonstrates bulky mulUfocal PLL
within the posterior longitudinal ligament (PLL) ossification producing severe central canal stenosis and
producing canal narrowing, cord compression. cord compression at upper cervical, cervicothoracic
juncUon.
· L,\'A
, :· ; - · ,
' . . . .:~~
,i'
,:
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OPLL
Key Facts
Terminology Pathology
• Ossification of posterior longitudinal ligament • Epidemiology: 2-4% prevalence in Japan; much less
common elsewhere
Imaging Findings • Bulky posterior ligamentous ossification with normal
• Best diagnostic clue: Flowing multilevel ossification cortical bone, marrow
posterior to vertebral bodies, with relatively minimal 2
degenerative disc disease, absent facet ankylosis Clinical Issues
• Location: Midcervical (C3-CS) > mid-thoracic (T4-T7) • Symptomatic myelopathy almost universal if canal 81
• Characteristic "upside down T" or "bowtie" diameter < 6 mm; rare if > 14 mm
configuration on axial images • t Risk for developing progressive myelopathy if >
60% canal stenosis, t cervical range of motion
Top Differential Diagnoses
• Spondylosis Diagnostic Checklist
• Calcified herniated disc • Overly "thick" hypointense PLL on MRI strongly
• Meningioma suggestive of OPLL
• Cervical peridural calcification of hemodialysis • Requires high index of suspicion, careful observation
to diagnose on plain radiographs
• May show high signal intensity if significant • Hemodialysis patient +/- myelopathy, sensory
marrow fat content abnormalities
• T2WI • Requires dural resection in addition to
o PLL findings similar to T1 WI laminectomy/laminoplasty
o +/- Cord hyperintensity 2° myelomalacia, edema
• T2* GRE
o Hypointense signal intensity within PLL ossification IPATHOLOGY
o Caveat: Exaggerates severity of stenosis 2° to
magnetic susceptibility effects General Features
• General path comments
Imaging Recommendations o Idiopathic PLL ossification predominately described
• Best imaging tool: Multiplanar MR imaging in Japanese > other Asian populations, less common
• Protocol advice in Caucasians
o Sagittal T1 WI, T2WI to evaluate spinal cord o More recently described early-stage "OPLL in
compression, extent of ligamentous ossification evolution" noted in both Asian, Caucasian
o Axial T2WI images to determine stenosis severity, populations
confirm cord signal abnormality • Hypertrophied PLL with punctate calcifications
o CT with sagittal reformats to confirm MR diagnosis, rather than smooth, flowing ossific mass
clarify extent of ossification for surgical planning • Difficult to distinguish from more common
spondylosis at this early stage
o OPLL patients have higher bone mineral density
IDIFFERENTIAL DIAGNOSIS (BMD) than age-matched controls
• Possible predisposition for excessive bone
Spondylosis deposition
• Usually centered at disc interspace; rarely contiguous • Genetics: Chromosome 6 XI collagen (alpha) 2 gene
across 4 or more vertebral levels (COLl1A2) =;> abnormal N-propeptide, OPLL
• More substantial facet, disc degenerative changes than susceptibility
OPLL • Etiology
• Absence of characteristic "T-shaped" PLL ossification o Not conclusively determined
Calcified herniated disc o Postulated etiologies include infectious agents,
• Focal calcified "mass" centered at a single disc space autoimmune disorders, or trauma
level • Epidemiology: 2-4% prevalence in Japan; much less
• Lacks characteristic "T-shaped" PLL ossification common elsewhere
• Associated abnormalities
Meningioma o Diffuse idiopathic skeletal hyperostosis (DISH)
• Avidly enhancing dural based mass + dural "tail", o Enthesial, tendinous, and ligamentous ossifications
smooth margins (if concurrent DISH)
• Often T2 hypointense 2° to calcification
• Lacks characteristic "T-shaped" PLL ossification
Gross Pathologic & Surgical Features
• Bulky posterior ligamentous ossification with normal
Cervical peridural calcification of cortical bone, marrow
hemodialysis • OPLL may transgress dura
• Curvilinear dural calcification; may be circumferential
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OPLL
Microscopic Features ISELECTED REFERENCES
• Histologically normal Haversian bone, marrow within 1. Matsunaga S et al: Clinical course of patients with
ossified ligament ossification of the posterior longitudinal ligament: a
minimum lO-year cohort study. J Neurosurg. 100(3
Suppl):245-8, 2004
I CLINICAL ISSUES 2. Shiraishi T et al: Cervical peridural calcification in patients
2 Presentation
undergoing long-term hemodialysis. Report of two cases.
100(3 Suppl): 284-6, 2004
3. Kamizono J et al: Occupational recovery after open-door
82 • Most common signs/symptoms type laminoplasty for patients with ossification of the
o Incidental observation in asymptomatic patient posterior longitudinal ligament. Spine. 28(16):1889-92,
o Symptomatic patients present with myelopathy 2003
referable to stenosis level 4. Epstein NE et al: In vitro characteristics of cultured
• Symptomatic myelopathy almost universal if posterior longitudinal ligament tissue. Spine. 27(1):56-8,
canal diameter < 6 mm; rare if > 14 mm 2002
• Canal diameter 6-14 mm variable presentation; 5. Epstein N: Diagnosis and surgical management of cervical
greater cervical mobility ~ more likely to be ossification of the posterior longitudinal ligament. Spine].
2(6):436-49, 2002
symptomatic 6. Matsunaga S et al: Trauma-induced myelopathy in patients
• Clinical profile: Classic presentation is Japanese with ossification of the posterior longitudinal ligament. J
patient with progressive quadriparesis or paraparesis Neurosurg. 97(2 Suppl):172-5, 2002
7. Matsunaga S et al: J Neurosurg. 96(2 Suppl):168-72, 2002
Demographics 8. Epstein NE: Identification of ossification of the posterior
• Age: Usually age> SO years; rare < 30 years longitudinal ligament extending through the dura on
• Gender: M:F = 2: 1 preoperative computed tomographic examinations of the
• Ethnicity: 1 Prevalence in Japanese population cervical spine. Spine. 26(2):182-6, 2001
9. Matsunaga S et al: Quality of life in elderly patients with
Natural History & Prognosis ossification of the posterior longitudinal ligament. Spine.
• Mild cases asymptomatic ~ incidental discovery 26(5):494-8, 2001
• Spastic paresis ~ paralysis (17-22%) 10. Sakou T et al: Recent progress in the study of pathogenesis
of ossification of the posterior longitudinal ligament. J
o Patients with mild OPLL at diagnosis rarely develop
Orthop Sci. 5(3):310-5, 2000
severe canal stenosis on follow-up 11. Yamauchi T et al: Bone mineral density in patients with
o Patients presenting with myelopathy very likely to ossification of the posterior longitudinal ligament in the
clinically progress cervical spine. J Bone Miner Metab. 17(4):296-300, 1999
o Mild trauma may exacerbate myelopathy 12. Koga H et al: Genetic mapping of ossification of the
• 1 Risk for developing progressive myelopathy if > 60% posterior longitudinal ligament of the spine. Am J Hum
canal stenosis, 1 cervical range of motion Genet. 62(6):1460-7, 1998
o j Spinal mobility appears to protect spinal cord from 13. Ehara S et al: Paravertebral ligamentous ossification: DISH,
OPLL and OLP. Eur J Radiol. 27(3):196-205, 1998
injury
14. Matsunaga S et al: The natural course of myelopathy
Treatment caused by ossification of the posterior longitudinal
ligament in the cervical spine. Clin Orthop. (305):168-77,
• Asymptomatic patients: Careful observation, 1994
non-operative management 15. Epstein NE: The surgical management of ossification of the
• Symptomatic or high grade stenosis patients: Anterior posterior longitudinal ligament in 43 north americans.
(corpectomy) or posterior (laminectomy or Spine. 19(6):664-72, 1994
laminoplasty) decompression 16. Epstein NE: Ossification of the posterior longitudinal
ligament in evolution in 12 patients. Spine. 19(6):673-01,
1994
IDIAGNOSTIC CHECKLIST 17. Epstein N: The surgical management of ossification of the
posterior longitudinal ligament in 51 patients. J Spinal
Consider Disord. 6(5):432-54; discussion 454-5, 1993
• Look carefully for OPLL on lateral plain films; confirm
with multiplanar CT or MRI
Image Interpretation Pearls
• Search for flOWing multilevel ossification behind
vertebral bodies
• Overly "thick" hypointense PLL on MRI strongly
suggestive of OPLL
• Requires high index of suspicion, careful observation
to diagnose on plain radiographs
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OPLL
I IMAGE GALLERY
Variant
(Left) Sagittal bone CT shows
extensive flowing PLL
ossification that severely
narrows the central canal.
Also noted is concurrent ALL
ossification (DISH). (Right)
Axial bone CT reveals bulky
"upside-down T" PLL
ossification that severely
narrows the central canal at
C2. Mild concurrent ALL
ossification (arrow) is typical
of DISH.
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OSSIFICATION LIGAMENTUM FLAVUM
2
84
Sagittal NEG shows dorsal curvilinear ossification Axial TlWI MR reveals focal ossification within the
within ligamentum flavum (arrows) producing mild ligamentum f1avum (arrow). Hyperintense signal
central canal narrowing. intensity similar to regional fat indicates fatty marrow.
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OSSIFICATION LIGAMENTUM FLAVUM
Key Facts
Terminology Pathology
• Ossification of ligamentum flavum (OLF), ossification • Pathogenesis probably related to hydroxyapatite
of the vertebral arch ligaments (OVAL), ligamentum (HAD) or calcium pyrophosphate deposition (CPPD)
flavum "pseudogout" in ligament ~ calcification, ossification
Imaging Findings Clinical Issues 2
• Best diagnostic clue: Linear thickening of ligamentum • Incidental observation on imaging study ordered for
flavum with imaging characteristics similar to other reasons 85
adjacent vertebral marrow ossification • Chronic thoracic myelopathy
• Symmetric, bilateral> unilateral • Duration of pre-operative symptoms = most
• Thin curvilinear "calcification" ventral to lamina important predictor of long term post-operative
• +/- Dorsal thecal sac impression outcome
• "V-shape" on axial images
Diagnostic Checklist
Top Differential Diagnoses • Consider OLF in a patient presenting with progressive
• Facet arthrosis myelopathy, particularly if Japanese or North African
• Meningioma descent
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OSSIFICATION LIGAMENTUM FLAVUM
4. Seichi A et al: Image-guided resection for thoracic
!ClINICAlISSUES ossification of the ligamentum flavum. J Neurosurg. 99(1
Suppl):60-3, 2003
Presentation 5. Muthukumar N et al: Tumoral calcium pyrophosphate
• Most common signs/symptoms dihydrate deposition disease of the ligamentum flavum.
o Incidental observation on imaging study ordered for Neurosurgery. 53(1):103-8; discussion 108-9, 2003
other reasons 6. Mizuno J et al: Unilateral ossification of the ligamentum
flavum in the cervical spine with atypical radiological
2 o Chronic thoracic myelopathy
• Ambulation difficulty, weakness, back pain, and appearance. J Clin Neurosci. 9(4):462-4, 2002
7. Akhaddar A et al: Thoracic spinal cord compression by
86
lower extremity paresthesias ligamentum flavum ossifications. Joint Bone Spine.
• Posterior column findings usually first followed by 69(3):319-23,2002
progressive spastic paraparesis 8. Li KK et al: Myelopathy caused by ossification of
o Back pain +/- radicular symptoms figamentum flavum. Spine. 27(12):E308-12, 2002
• Clinical profile: Symptomatic patients present 9. Vasudevan A et al: Ossification of the ligamentum flavum.
similarly to other cause of myelopathy J Clin Neurosci. 9(3):311-3, 2002
10. Hirai T et al: Ossification of the posterior longitudinal
Demographics ligament and ligamentum flavum: imaging features. Semin
• Age: 4th ~ 6th decade Musculoskelet Radiol. 5(2):83-8, 2001
11. Trivedi P et al: Thoracic myelopathy secondary to ossified
• Gender: M > F
ligamentum flavum. Acta Neurochir (Wien). 143(8):775-82,
• Ethnicity: Symptomatic presentation more common in 2001
Japanese, North African descent> > Caucasian> 12. Xiong L et al: CT and MRI characteristics of ossification of
African-American the ligamenta flava in the thoracic spine. Eur Radiol.
11(9):1798-802,2001
Natural History & Prognosis 13. Muthukumar N et al: Calcium pyrophosphate dihydrate
• Most patients remain asymptomatic deposition disease causing thoracic cord compression: case
• Minority of patients manifest progressive myelopathy; report. Neurosurgery. 46(1):222-5, 2000
symptoms may improve or stabilize following surgery 14. Yamagami T et al: Calcification of the cervical ligamentum
o Persistent post-operative spasticity suggests flavum--case report. Neurol Med Chir (Tokyo). 40(4):234-8,
irreversible cord injury 2000
15. Ono K et al: Pathology of ossification of the posterior
• Duration of pre-operative symptoms = most important
longitudinal ligament and ligamentum flavum. Clin
predictor of long term post-operative outcome Orthop. (359):18-26, 1999
Treatment 16. Wang PN et al: Ossification of the posterior longitudinal
ligament of the spine. A case-control risk factor study.
• Conservative observation in asymptomatic, mild cases Spine. 24(2):142-4; discussion 145, 1999
• Posterior decompression (laminectomy or 17. Ehara S et al: Paravertebral ligamentous ossification: DISH,
laminoplasty), ligamentum flavum resection in OPLL and OLP. Eur J Radiol 27(3): 196-205, 1998
symptomatic cases 18. al-Orainy IA et al: Ossification of the ligament flavum. Eur
J Radiol. 29(1):76-82, 1998
19. Ido K et al: Surgical treatment for ossification of the
IDIAGNOSTIC CHECKLIST posterior longitudinal ligament and the yellow ligament in
the thoracic and cervico-thoracic spine. Spinal Cord.
Consider 36(8):561-6, 1998
20. Fam AG: Calcium pyrophosphate crystal deposition disease
• Consider OLF in a patient presenting with progressive and other crystal deposition diseases. Curr Opin
myelopathy, particularly if Japanese or North African Rheumatol. 7(4):364-8, 1995
descent 21. Imai S et al: Cervical radiculomyelopathy due to deposition
• Important to exclude co-existent OPLL, other of calcium pyrophosphate dihydrate crystals in the
myelopathic lesions ligamentum flavum: historical and histological evaluation
of attendant inflammation. J Spinal Disord. 7(6):513-7,
Image Interpretation Pearls 1994
• Distinguish from facet arthropathy by location in 22. Oelamarter RB et al: Lumbar spinal stenosis secondary to
ligamentum flavum, observation above or below facet calcium pyrophosphate crystal deposition (pseudogout).
Clin Orthop. (289):127-30, 1993
joint level
23. Gomez H et al: Myeloradiculopathy secondary to
pseudogout in the cervical ligamentum flavum: case report.
Neurosurgery. 25(2):298-302, 1989
ISELECTED REFERENCES 24. Kawano N et al: Calcium pyrophosphate dihydrate crystal
1. Takeuchi A et al: Thoracic paraplegia due to missed deposition disease in the cervical ligamentum flavum. J
thoracic compressive lesions after lumbar spinal Neurosurg. 68(4):613-20, 1988
decompression surgery. Report of three cases. J Neurosurg. 25. Berghausen EJ et al: Cervical myelopathy attributable to
100(1 Suppl):71-4, 2004 pseudogout. Case report with radiologic, histologic, and
2. Miyakoshi N et al: Factors related to long-term outcome crystallographic observations. Clin Orthop. (214):217-21,
after decompressive surgery for ossification of the 1987
ligamentum flavum of the thoracic spine. J Neurosurg. 26. Resnick 0 et al: Vertebral involvement in calcium
99(3 Suppl):251-6, 2003 pyrophosphate dihydrate crystal deposition disease.
3. Ben Hamouda K et al: Thoracic myelopathy caused by Radiographic-pathological correlation. Radiology.
ossification of the ligamentum flavum: a report of 18 cases. 153(1):55-60, 1984
J Neurosurg. 99(2 Suppl):157-61, 2003
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OSSIFICATION LIGAMENTUM FLAVUM
IIMAGE GALLERY
Typical
(Left) Axial bone CT shows
bilateral linear ligamentum
flavum ossification in an
asymptomatic patient
imaged for lumbar trauma.
2
(Right) Sagittal bone CT in a 87
patient with severe
degenerative disc disease
demonstrates fine linear OLF
(arrow) incidentally noted
during work-up for chronic
neck pain.
Typical
(Left) Sagittal TlWI MR
reveals focal nodular OLF in
the thoracic ligamentum
flavum (arrow), mildly
narrowing the central canal.
(Right) Axial T2WI MR
depicts unilateral
hypointense ossification
(arrow) within the left
ligamentum flavum
producing mild narrowing of
the lateral spinal canal and
neural foramen.
Typical
(Left) Axial bone CT reveals
-.--=-_·--~~-=;~"=-tl
~.- ~ - ....~.
asymmetric bilateral OLF
(arrows) co-existing with
. _.. ..
. -- . mild facet arthropathy.
:.-~
--: (Right) Axial bone CT
,- - - ::... obtained for trauma shows
focal nodular ossification
- ~._-
~ (arrow) in the OLF, an
~ -~.~'.-.'.-.
incidental asymptomatic
finding.
L~·
:'-iI
1
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ADULT RHEUMATOID ARTHRITIS
2
88
Axial and sagittal graphics show erosion of dens by Coronal bone CT shows severe erosions at base of dens
pannus. Rupture of transverse ligament of dens is (arrows) and right C7-C2 facet joint (open arrows).
evident on axial graphic. Note mass effect on spinal Early erosion seen at left C 7-C2 facet joint.
cord.
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ADULT RHEUMATOID ARTHRITIS
Key Facts
Terminology Top Differential Diagnoses
• Inflamed and thickened synovium is called pannus • Seronegative spondyloarthropathy
• Juvenile chronic arthritis
Imaging Findings • Degenerative disc disease
• Never involves spine without hands and/or feet
• Cervical spine: Approximately 60% of RA patients Pathology 2
• Rarely involves sacroiliac joints and lumbar spine • Rheumatoid factor may be negative initially
• CI-C2 instability in 33% of all RA patients • Epidemiology: 1% of population has rheumatoid 89
• Atlanto-axial subluxation in 5% of patients with arthritis
cervical RA
• Instability may also be present at lower levels of Clinical Issues
cervical spine • Morning pain and stiffness
• Cervical spine radiographs in flexion/extension to • Radiculopathy common with cervical spine
assess for instability involvement
• MR imaging in patients with cord symptoms
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ADULT RHEUMATOID ARTHRITIS
I PATHOLOGY Demographics
General Features • Age: Occurs at all ages, most common in middle-age
• Gender: M:F = 2-3:1
• General path comments
o Erythrocyte sedimentation rate, C-reactive protein Natural History & Prognosis
elevated • May develop radiculopathy, myelopathy
2 o Rheumatoid factor may be negative initially
• Eventually positive in up to 95% of RA patients
• Increased morbidity, mortality with craniocervical
junction instability
90 • False positive RF seen in elderly patients, systemic
lupus erythematosus, sarcoid, cryoglobulinemia, Treatment
cirrhosis, endocarditis • Medical treatment
o Polyarticular synovial inflammation leads to o Disease modifying agents (DMARDS) are anti tumor
articular destruction necrosis factor
• Genetics: Strong hereditary component • Can dramatically slow disease progression
• Etiology: Common arthritis of unknown etiology o Corticosteroids, methotrexate
• Epidemiology: 1% of population has rheumatoid • Surgical treatment
arthritis o C1/2 transarticular surgical fusion for atlantoaxial
• Associated abnormalities subluxation
o Lungs: Interstitial lung disease, rheumatoid lung o Transoral odontoid resection for dens/pannus cord
nodules, pleural effusions compression
o Soft tissues: Rheumatoid nodules
Gross Pathologic & Surgical Features IDIAGNOSTIC CHECKLIST
• Upper cervical abnormalities are most common
manifestation of cervical spine RA Image Interpretation Pearls
o Pannus erodes transverse atlantoaxial ligament • Flexion-extension views often technically inadequate
• Permits subluxation of the anterior C1 ring due to lack of patient motion
relative to the dens o Do not misinterpret as stability
• Often asymptomatic but can result in cord
compression when neck flexed
o Cranial settling, atlantoaxial subluxation ISELECTED REFERENCES
• Due to erosion of occipital cOll'dylar-C1 facet 1. Neva MH et al: Early and extensive erosiveness in
joints and occiput-C1ligaments peripheral joints predicts atlantoaxial subluxations in
patients with rheumatoid arthritis. Arthritis Rheum.
Microscopic Features 48(7):1808-13,2003
• Thickened synovium containing plasma cells, 2. Emery P et al: Role of biologics in early arthritis. Clin Exp
multinucleated giant cells, polymorphonuclear Rheumatol. 21(5 SuppI31):S191-4, 2003
leukocytes, lymphocytes 3. Casey AT et al: Rheumatoid arthritis of the cervical spine:
current techniques for management. Orthop Clin North
Staging, Grading or Classification Criteria Am. 33(2):291-309, 2002
• 1987 American Rheumatology Association Criteria 4. Roche CJ et al: The rheumatoid cervical spine: signs of
o Morning stiffness at least 1 hr, 6 weeks instability on plain cervical radiographs. Clin Radiol.
o Swelling 3 or more joints, 6 weeks 57(4):241-9,2002
5. Riew KD et al: Diagnosing basilar invagination in the
o Swelling wrist, MCP or PIP joints, 6 weeks rheumatoid patient. The reliability of radiographic criteria.
o Symmetrical swelling J Bone Joint Surg Am. 83-A(2):194-200, 2001
o Hand x-ray changes 6. Reijnierse M et al: Neurologic dysfunction in patients with
o Subcutaneous nodules rheumatoid arthritis of the cervical spine. Predictive value
• Presence of any four of the seven criteria yields 93% of clinical, radiographic and MR imaging parameters. Eur
sensitivity, 90% specificity Radiol 11(3): 467-73, 2001
7. Riise T et al: High mortality in patients with rheumatoid
arthritis and atlantoaxial subluxation. J Rheumatol.
28(11):2425-9,2001
ICLINICAL ISSUES 8. Neva MH et al: Prevalence of radiological changes in the
cervical spine-a cross sectional study after 20 years from
Presentation presentation of rheumatoid arthritis. J RheumatoI27(1):
• Most common signs/symptoms 90-3,2000
o Morning pain and stiffness 9. Zoli A et al: Craniocervical junction involvement in
o Radiculopathy common with cervical spine rheumatoid arthritis: a clinical and radiological study. J
involvement Rheumatol. 27(5):1178-82, 2000
o Other signs/symptoms 10. Janssen H et al: MR imaging of arthritides of the cervical
• Pathologic fracture spine. Magn Reson Imaging Clin N Am. 8(3):491-512, 2000
• Cervical myelopathy
• Peripheral nerve compression, especially in carpal
tunnel
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ADULT RHEUMATOID ARTHRITIS
I IMAGE GALLERY
Typical
(Left) Lateral radiography
shows CI-2 subluxation
(arrow) in flexed position.
Subaxial disease also
evident, causing C3-4
2
uncovertebral (open arrow) 91
and facet (curved arrow)
erosions. (Right) Lateral
radiography shows that CI-2
subluxation (arrow)
improves but does not
resolve when neck is
extended. This indicates
amount of space-occupying
pannus between CI and C2
preventing reduction.
Typical
(Left) Sagittal T1 WI MR
shows heterogeneous signal
intensity pannus (arrows)
surrounding dens, eroding
clivus and compressing cord
both anteriorly and
posteriorly. Dens is severely
eroded. (Right) Sagittal T2WI
MR shows heterogeneous
signal intensity pannus
(arrows). There is spinal cord
compression at CI-2 and
C3-4. C3-4 subluxation is
from uncovertebral, facet
erosions (not shown).
Variant
(Left) Axial bone CT shows
pathologic oblique fracture
of dens (open arrows) in
longstanding RA. Note
odontoid erosions (arrow).
(Right) Sagittal T2WI MR
shows pathologic oblique
fracture of dens (arrow).
Pannus formation and
hematoma are seen
surrounding dens, and there
is prevertebral hematoma.
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JUVENILE CHRONIC ARTHRITIS
2
92
Lateral radiography shows multiple vertebral fusions in Lateral radiography shows adult-type juvenile onset RA,
adult patient with history of JCA. Fused vertebrae are tall with odontoid erosion and multilevel facet erosions
and narrow. Odontoid process is eroded, and there is resulting in "stepladder" subluxations ofcervical spine.
C/-2 subluxation.
LI
'
.....
Congenital Fusion Physiologic Sublux. Down Syndrome Osteogen. Imperf.
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JUVENILE CHRONIC ARTHRITIS
Key Facts
Terminology • Pannus intermediate to high signal intensity
• Juvenile chronic arthritis OCA) Top Differential Diagnoses
• A spectrum of idiopathic inflammatory arthropathies • Congenital spinal fusion
occurring in childhood • Physiologic subluxations
Imaging Findings •
•
Down syndrome
Osteogenesis imperfecta
2
• Best diagnostic clue: Cervical spine subluxations and
growth disturbance 93
Pathology
• Cervical vertebral fusions • JCA is divided into 5 categories
• Growth disturbances • Oligoarticular JRA
• Multilevel vertebral subluxations • Still disease
• Erosions • Seronegative polyarticular JRA
• Visualization of bone erosions • Juvenile onset adult RA
• Pannus low signal intensity • Juvenile onset AS
• Cartilage erosions visible much earlier than bony
erosions
CT Findings
• Bone CT IDIFFERENTIAL DIAGNOSIS
o Can be used for early diagnosis of erosions
o Radiation considerations have led to preference for Congenital spinal fusion
MRI • Isolated anomaly or in association with syndromes
such as VACTERL
MR Findings • No systemic symptoms
• TlWI • Often have kyphosis, scoliosis
o Visualization of bone erosions • May have Sprengel deformity
o Pannus low signal intensity
• T2WI Physiologic subluxations
o Cartilage erosions visible much earlier than bony • Normal vertebral morphology
erosions • Subluxations usually less than 2 mm
o Pannus intermediate to high signal intensity
o Joint effusions: Facet and sacroiliac joints
Down syndrome
• Vertebral subluxations
• STIR
o Pannus intermediate to high signal intensity • Normal vertebral morphology
o Joint effusions: Facet and sacroiliac joints Osteogenesis imperfecta
• Tl c+ • Subluxations
o Avid enhancement at erosions, in pannus • Diffuse osteopenia
o Contrast excreted into synOVial joints by 10 minutes • Vertebra plana and fractures
post injection • No systemic symptoms
• Evaluation of pannus must be performed
immediately post injection
Nuclear Medicine Findings
• Bone Scan
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JUVENILE CHRONIC ARTHRITIS
[PATHOLOGY Demographics
General Features • Age
o Younger children often have systemic disease (Still)
• Etiology: Unknown o Older children and adolescents present with joint
• Epidemiology: Patients with severe hand JCA usually pain
also have cervical spine involvement • Gender: Females more common than males except for
2 • Associated abnormalities
o Young children may have fever, anemia,
juvenile onset AS
94
hepatosplenomegaly Natural History & Prognosis
o Secondary amyloidosis may be present • Growth disturbances lead to premature osteoarthritis
• Causes nephropathy • Often "burns out" as children approach adulthood
o Duration of symptoms in childhood correlated to
Gross Pathologic & Surgical Features risk of continued disease in adulthood
• Because cartilage thicker in children, bone erosions are • Psychosocial issues from chronic disease in childhood
less commonly seen than in adults
• Growth disturbance is often the most outstanding Treatment
feature • Options, risks, complications: NSAIDs, steroids,
• Fusion of joints common in appendicular skeleton as methotrexate, anti tumor necrosis factor (TNF)
well as spine medications
• Periostitis may be seen around joints
Microscopic Features IDIAGNOSTIC CHECKLIST
• Thickened synovium containing plasma cells,
multinucleated giant cells,PMNs, lymphocytes Consider
Staging, Grading or Classification Criteria • Underdiagnosed; consider in child with vague, chronic
articular or neck pain
• JCA is divided into 5 categories ~
• Oligoarticular JRA
o 40% of patients
o RF negative
ISELECTED REFERENCES
o Involves 1 to several large joints (e.g., knee, hip, 1. Kekilli E et al: Cervical involvement in juvenile-onset
shoulder, elbow) ankylosing spondylitis with bone scintigraphy. Rheumatol
Int. 24(3):164-5, 2004
• Still disease 2. Laiho K et al: The cervical spine in juvenile chronic
o 20% of patients arthritis. Spine J. 2(2):89-94, 2002
o RF negative 3. Burgos-Vargas R et al: A short-term follow-up of enthesitis
o Under 5 years old and arthritis in the active phase of juvenile onset
o Systemic disease, growth disturbance spondyloarthropathies. Clin Exp Rheumatol. 20(5):727-31,
o Fever, anemia, hepatosplenomegaly 2002
• Seronegative polyarticular JRA 4. Haapasaari J et al: MRI diagnosis and successful treatment
o 25% of patients of upper cervical spine synovitis in a patient with juvenile
chronic arthritis. Clin Exp Rheumatol. 20(2):256-7, 2002
o Adult distribution, but seronegative
5. Roche C] et al: The rheumatoid cervical spine: signs of
• Juvenile onset adult RA instability on plain cervical radiographs. Clin Radiol.
o 5% of patients 57(4):241-9,2002
o RF positive 6. Laiho K et al: The cervical spine in mutilant juvenile
• Juvenile onset AS chronic arthritis. Joint Bone Spine. 68(5):425-9, 2001
o 10% of patients 7. Prieur AM et al: Prognostic factors in juvenile idiopathic
o Almost all HLA-B27 positive arthritis. Curr Rheumatol Rep. 3(5):371-8, 2001
o Usually adolescent males 8. Cunnane G: Amyloid precursors and amyloidosis in
inflammatory arthritis. Curr Opin Rheumatol. 13(1):67-73,
o Primarily involves SUs, lower extremity joints
2001
o Enthesopathy a prominent feature 9. Janssen H et al: MR imaging of arthritides of the cervical
spine. Magn Reson Imaging Clin N Am. 8(3):491-512, 2000
10. Zoli A et al: Craniocervical junction involvement in
ICLINICAL ISSUES rheumatoid arthritis: a clinical and radiological study. J
Rheumatol. 27(5):1178-82, 2000
Presentation 11. Ansell BM: Prognosis in juvenile arthritis. Adv Exp Med
• Most common signs/symptoms BioI. 455:27-33, 1999
o Vague, pauciarticular pain 12. Husby G: Treatment of amyloidosis and the
o Limited neck movement rheumatologist. State of the art and perspectives for the
future. ScandJ Rheumatol. 27(3):161-5, 1998
o Peripheral tenosynovitis may be presenting 13. Schneider R et al: Systemic onset juvenile rheumatoid
complaint arthritis. Baillieres Clin Rheumatol. 12(2):245-71, 1998
o Other signs/symptoms 14. Tucker LB: Juvenile rheumatoid arthritis. Curr Opin
• Still disease: Fever, anemia, hepatosplenomegaly Rheumatol. 5(5):619-28, 1993
• Juvenile onset AS: Chronic low back pain, limited
motion
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IIMAGE GALLERY
(Left) Anteroposterior
radiography shows sclerosis
and poor definition of 51 2
joints (arrows) indicating
sauoiliitis in 17 yo male with
low back and left knee pain. 95
Knee MRlshowed
inflammatory arthropathy.
(Right) Axial bone CT shows
left 51 joints erosions (curved
arrow) and bilateral effusions
(arrows) in child with
inflammatory arthropathy.
Typical
(Left) Lateral radiography
shows posterior fusion at
C2-3 (open arrow) due to
jRA. Dens is large and
appears fused to clivus
(arrow). Subluxations are
seen at C3-4 and C4-5.
(Right) Sagittal T2WI MR
shows enlargement of dens,
which abuts clivus. Lateral
images showed fused facet
joints at C2-3.
Typical
(Left) Axial bone CT shows
erosions due to jCA,
involVing facet joints
(arrows), costotransverse
joints (open arrows) and left
sternoclavicular joint (curved
arrow). (Right) Lateral
radiography shows surgical
fusion from occiput to C4 for
instability from jCA. Note
mandibular hypoplasia and
temporomandibular joint
prostheses.
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SERONEGATIVE SPONDYLOARTHROPATHY
2
96
Coronal graphic shows Materal sacroiliac erosions and Anteroposterior radiography shows bilateral sacroiliitis
flowing syndesmophytes along lateral margins of lumbar (arrows), with joint widening and multiple small
vertebrae. erosions, as well as reactive sclerosis. Diffuse ankylosis
lumbar spine also seen.
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SERONEGATIVE SPONDYLOARTHROPATHY
Key Facts
Terminology • MR/CT in combination to evaluate bone and cord
• RF (rheumatoid factor) negative inflammatory status following trauma
arthritis and enthesopathy affecting the spine and Top Differential Diagnoses
sacroiliac joints • Rheumatoid arthritis
Imaging Findings •
•
Juvenile chronic arthritis
Infectious sacroiliitis
2
• Best diagnostic clue: SI joint erosion or ankylosis
• Sacroiliac joint (SI]) erosive arthritis • Diffuse idiopathic skeletal hyperostosis (DISH) 97
• Comer erosions vertebral bodies • Retinoid therapy
• Ossification of outer fibers anulus fibrosis Clinical Issues
• Ossification of paraspinous ligaments • Low back pain, greatest in morning
• Kyphotic deformity thoracic and lumbar spine • Pathologic fracture
• Fractures through syndesmophytes and/or vertebral • Most cases mild, limited to Sl]s
bodies • Some progress to extensive spine ankylosis over
• Start with plain films; use CT if plain films are period of years
negative
• May be bulky; more common in psoriatic and • T2WI: High signal intensity bone marrow at early
reactive arthropathy comer and SI] erosions
• Distinguished from osteophytes by origin from • STIR: High signal intensity bone marrow at early
mid vertebral body rather than endplate comer and SI] erosions
o Kyphotic deformity thoracic and lumbar spine • Tl C+: Avid enhancement in active disease
o Facet joint arthritis • Marrow in disc spaces (later stages), preservation of
• Erosions progress to fusion central canal
o Atlantoaxial subluxation
o Fractures through syndesmophytes and/or vertebral Nuclear Medicine Findings
bodies • Bone Scan: Positive 3-phase bone scan in active disease
• Often difficult to see because of osteopenia, spinal Imaging Recommendations
deformity
• Start with plain films; use CT if plain films are
• Unstable; involve all spinal columns negative
o Accelerated degenerative disc disease in unfused • MR/CT in combination to evaluate bone and cord
regions due to increased stress status follOWing trauma
• May mimic discitis or neuropathic disease
o Other joints of axial skeleton may show erosions
progressing to fusion IDIFFERENTIAL DIAGNOSIS
• Costotransverse, costovertebral joints
• Sternoclavicular, acromioclavicular joints Rheumatoid arthritis
• Pubic symphysis • Rarely involves SI]s
o Peripheral arthritis • Primarily involves cervical spine
• Erosions • Bony ankylosis not a feature
• Periosteal new bone at entheses; "whiskered"
appearance Juvenile chronic arthritis
• Psoriatic: Affects hands> feet • Onset less than age 18
• Reactive, AS: Affects feet more than hands • Growth disturbances of vertebrae
o Bone mineral density • Bony fusions
• Normal early in course of disease • Juvenile onset ankylosing spondylitis usually limited
• Diffuse osteopenia in areas of ankylosis to Sl]s, peripheral enthesopathy
CT Findings Infectious sacroiliitis
• Bone CT • Unilateral
o Highly sensitive to early sacroiliac erosions
o Commonly shows involvement of costotransverse Diffuse idiopathic skeletal hyperostosis
and costovertebral joints (DISH)
o Useful to evaluate fractures • Flowing ossification paraspinous ligaments
MR Findings • Sacroiliac joints normal
• TIWI Retinoid therapy
o Low signal intensity bone marrow at comer and SI] • Flowing ossification paraspinous ligaments
erosions • Sacroiliac joints normal
o Joint erosions and ankylosis
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SERONEGATIVE SPONDYLOARTHROPATHY
• Most common at cervicothoracic or
IPATHOLOGY thoracolumbar junction
General Features o Other signs/symptoms
• General path comments: RF negative, elevated ESR • Fatigue, low grade fever
• Genetics • Rarely, cauda equina syndrome (due to synovial
o Strong association withHLA - B27 haplotype cysts)
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SERONEGATIVE SPONDYLOARTHROPATHY
IIMAGE GALLERY
Typical
(Left) Lateral radiography
shows shiny corner (arrow),
vertebral squaring,
ossification of outer fibers of
anulus fibrosus (open
2
arrow), and anterior 99
longitudinal ligament in
psoriatic arthritis. (Right)
Anteroposterior radiography
shows bulky lateral
paraspinous ligamentous
ossifications (arrows) in
psoriatic arthritis.
Typical
(Left) Sagittal T1 WI M R
shows C6-7
fracture-dislocation (arrow)
through syndesmophytes
and posterior ligaments in
AS. Thin, low signal intensity
syndesmophytes are visible
in remainder of visualized
spine. (Right) Sagittal STIR
MR shows C6-7 fracture
(arrow) through
syndesmophyte in AS.
Hematoma posteriorly (open
arrow) indicates interspinous
ligament rupture.
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GOUT
2
100
Sagittal PO/Intermediate MR shows punched out Sagittal T2WI MR shows endplate destruction at C6-7
vertebral body erosions at multiple levels. There is (open arrow). Intraosseous tophus (arrow) is low signal
endplate destruction at C6-7 (open arrow). Curved intensity and almost indistinguishable from the
arrow points to prevertebral tophus. surrounding marrow.
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SECTION 1: Infe<:tions
Infections
Pyogenic Osteomyelitis 11I-1-6
Granulomatous Osteomyelitis 11I-1-10
Osteomyelitis, CI-C2 11I-1-14
Septic Facet Joint Arthritis 11I-1-18
Epidural Abscess 11I-1-22
Subdural Abscess 11I-1-26
Paraspinal Abscess 11I-1-30
Viral Myelitis 11I-1-34
HIV 11I-1-38
Myelitis-Cord Abscess 11I-1-42
Spinal Meningitis 11I-1-46
Echinococcus 11I-1-50
Schistosomiasis 11I-1-54
Cysticercosis 11I-1-58
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INFECTIONS, PATHWAYS OF SPREAD
1
2
Sagittal graphic shows lumbar disc space infection, Sagittal T1 C+ MR shows pyogenic disc space infection
vertebral body osteomyelitis with endplate destruction at L4-5 with endplate destruction (arrows), irregular
and marrow edema. There are ventral and dorsal enhancement of bodies and disc, and epidural
abscess collections. phlegmon (open arrow).
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INFECTIONS, PATHWAYS OF SPREAD
DIFFERENTIAL DIAGNOSIS
1
Disc space irregularity • Hemorrhage 3
• Pyogenic disc space infection • Empyema
• TB, fungal disc space infection Meningeal/leptomeningeal
• Type I degenerative endplate changes • Bacteria infection
• Hemodialysis spondyloarthropathy • Viral infection (CMV)
• Gout • Autoimmune (AIDP, elDp)
• Seronegative spondyloarthropathy • Leptomeningeal metastatic disease
Epidural mass • Arachnoiditis
• Metastatic disease Cord
• Abscess/phlegmon • Primary tumor (astrocytoma, ependymoma with
• Disc herniation/osteophyte ring-enhancement)
• Primary bone tumor • Demyelinating disease may give ring-enhancement,
• Hemorrhage cord edema
Subdural mass • Bacterial or parasitic infection (rare)
• Postoperative benign effusion
• Spinal tuberculosis classification (Mehta 2001) o Venous route classically through Batson plexus
o Stable anterior lesions without kyphotic deformity • Longitudinal network of valveless veins running
• Treated with anterior debridement and strut parallel to spinal column
grafting • Lie outside of thoracoabdominal cavity
o Global lesions, kyphosis, instability • Communicate with multiple venous systems:
• Treated with posterior instrumentation, anterior Spine, vena cava, portal, azygos, intercostal,
strut grafting pulmonary and renal
o High risk for transthoracic surgery (medical or • Flow direction in Batson plexus variable due to
anesthetic complications) variable intrathoracic or intraabdominal pressure
• Treated with posterior decompression, • Pharyngovertebral plexus serves same purpose
instrumentation • CSF spread
o Isolated posterior lesions o Cranial ¢;> spinal
• Treated with posterior decompression • Extension
o Spine ~ psoas muscle
o Extension to mediastinum
I ANATOMY-BASED IMAGING ISSUES I o Extension through dura ~ meningitis
Key Concepts or Questions Other Imaging Issues
• Direct extension • Grisel syndrome ~ unilateral or bilateral subluxation
o Bone in contact with adjacent infection giving CIon C2, associated with an infectious condition in
osteomyelitis the head or neck
o Typical route for intramedullary spinal cord abscess o Associated with head/neck infection
• Infection through congenital dysraphism/dermal
sinus mechanism of infection ~ 40% of
intramedullary abscesses I CLINICAL IMPLICATIONS
• Lymphatic spread
o Limited in importance relative to hematogenous Clinical Importance
spread • Spine = 2-5% of all osteomyelitis sites
• Hematogenous spread • Axial spine pain most common presentation
o Major pathway of infection spread to axial skeleton • Progressive, although may have insidious onset
• Major pathway of intramedullary spinal cord • Pain may be constant, without relief from rest
abscess in adults • Fever variable, and present in < 50% of cases
o Arterial or venous route (controversial as to relative o High grade fever in < 5%
importance) • Motor/sensory deficits in 10-15%
o Arterial route important in spine infection spread o Rare intramedullary abscess always present with
• Metaphyseal bone near anterior longitudinal motor and/or sensory neurological deficits
ligament has end arteriole network making it • Delay in diagnosis common
susceptible to bacterial seeding o Intramedullary abscess fatal 8%, persistent
• Distal nonanastomosing vessels have slow flow, neurologic deficit 70%
and occlusion leads to avascular necrosis • Lab abnormalities
• Segmental arteries usually supply two adjacent o Erythrocyte sedimentation rate positive in > 90%
vertebral bodies and intervening disc, giving o C reactive protein also elevated
typical disc space infection pattern
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INFECTIONS, PATHWAYS OF SPREAD
1
4
Sagittal graphic shows dermal sinus extending from skin Sagittal T1W C+ MR shows extensive dorsal epidural
surface to conus, with conus abscess,edema. abscess· throughout thoracic spine with cord
compression. Note extensive metal artifact from prior
anterior/posterior fixation.
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INFECTIONS, PATHWAYS OF SPREAD
IIMAGE GALLERY
1
5
Typical
(Left) Axial CECT shows
direct extension of infection
from mycotic aortic
aneurysm (arrow) into
vertebral body (open arrow),
and left psoas muscle
(curved arrow). (Right) Axial
Tl C+ MR shows direct
extension of infection from
mycotic aortic aneurysm
(arrow), erosion of ventral
vertebral body and
osteomyelitis (open arrow).
There is also direct extension
into psoas.
Typical
(Left) Axial Tl C+ MR shows
extension of tuberculous
spondylitis from vertebral
body into psoas muscles
bilaterally, forming large
abscesses. Enhancing
phlegmon also present in
ventral epidural space.
(Right) Sagittal Tl C+ MR
shows multifaceted
appearance of TB, with areas
mimicking pyogenic
infection (arrow), focal
lesions like metastatic
disease (open arrow), and
dural enhancement (curved
arrow).
Typical
(Left) Sagittal Tl C+ MR with
fat suppression shows
extensive subdural empyema
with peripheral
enhancement (arrows)
throughout the cervical
spine, and extending along
clivus (open arrow). (Right)
Axial Tl WI M R of lumbar
spine shows left septic facet
(arrows) with left facet bone
destruction and infection
extending into adjacent soft
tissues.
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PYOGENIC OSTEOMYELITIS
1
6
Sagittal T2WI MR shows hyperintense marrow in three Sagittal T1 C+ MR shows enhancing vertebrae, disc,
consecutive thoracic vertebrae with loss of endplate and epidural phlegmon, compatible with
definition. Intervertebral discs appear hyperintense and spondylodiscitis. Another infectious focus is present
narrowed. more caudally (arrow).
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PYOGENIC OSTEOMYELITIS
Key Facts
1
Terminology • Paraspinal ± epidural infiltrative soft tissue ± 7
• Pyogenic spondylodiscitis loculated fluid collection
• Bacterial suppurative infection of vertebrae and • Variable central canal narrowing
intervertebral disc • CECT: Enhancing disc, marrow, and paravertebral
soft tissue
Imaging Findings • Cord compression with ill-defined T2 hyperintensity
• Best diagnostic clue: Ill-defined hypointense vertebral
marrow on TlWI with loss of endplate definition on
Top Differential Diagnoses
both sides of the disc • Degenerative endplate changes
• Lumbar (48%) > thoracic (35%) > cervical spine • Tuberculous vertebral osteomyelitis
(6.5%) • Spinal neuropathic arthropathy
• 2 adjacent vertebrae with intervening disc • Chronic hemodialysis spondyloarthropathy
• Disc space narrowing Clinical Issues
• Loss of vertebral endplate cortex • Acute or chronic back pain
• Ill-defined marrow signal alteration • Focal spinal tenderness
• Vertebral collapse • Fever
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PYOGENIC OSTEOMYELITIS
1 o Hyperintense on T2WI
o Post-gadolinium enhancement
o Fever
o Other signs/symptoms
8 • Non-contiguous vertebral involvement • Myelopathy if cord compromised
• Posterior elements commonly effected • Elevated erythrocyte sedimentation rate,
• Disc space preserved C-reactive protein, and white cell count
• Clinical profile: Average duration of symptoms for 7
weeks before diagnosis
IPATHOLOGY Demographics
General Features • Age
• General path comments: Along a spectrum of o Bimodal distribution
suppurative infection involving disc, vertebrae, and • Pediatric patients
adjacent soft tissue • 60-70s
• Etiology • Gender: Slight male predominance
o Predisposing factors Natural History & Prognosis
• Intravenous drug use
• Immunocompromised state • Vertebral collapse
• Chronic medical illnesses such as renal failure, • Irreversible neurological deficits
cirrhosis, cancer, diabetes • Mortality rate of 2-12%
o Staphylococcus aureus is the most common • Favorable outcome with resolution of symptoms if
prompt diagnosis and treatment
pathogen
o Residual functional deficits may be present in 15%
• Escherichia coli most common within
gram-negative bacilli of patients
• Salmonella more common in patients with sickle • Recurrence due to incomplete treatment: 2-8%
cell disease Treatment
o Bacteremia from an extraspinal primary source • Early empiric antibiotics with broad spectrum
• Most common route of infection coverage until causative pathogen isolated
• GU or GI tract, lungs, cardiac, mucous/cutaneous o Coverage should be effective against staphylococci,
sources gram negatives, and anaerobes
• Vascularized subchondral bone adjacent to • Organism specific parenteral antibiotics for 6-8 weeks
endplate seeded primarily • Spinal immobilization with bracing for 6-12 weeks
• Secondary infection of intervertebral disc and • Surgical treatment
adjacent vertebra o Laminectomy, debridement, ± stabilization
• Intervertebral disc first site of infection in children o Especially if epidural abscess, instability present
due to presence of vascularity
o Direct inoculation from penetrating trauma, surgical
intervention, or diagnostic procedures IDIAGNOSTIC CHECKLIST
• Epidural injection/catheter
o Extension from adjacent infection in paraspinal soft Image Interpretation Pearls
tissues • Diffusely enhancing disc, adjacent vertebral marrow,
• Diverticulitis, appendicitis, inflammatory bowl and soft tissue with endplate erosion highly suggestive
disease of vertebral osteomyelitis
• Pyelonephritis
• Epidemiology: 2-7% of osteomyelitis in US
• Associated abnormalities
o Spinal meningitis
ISELECTED REFERENCES
1. Ledermann HP et al: MR imaging findings in spinal
o Myelitis infections: rules or myths? Radiology. 228(2):506-14, 2003
Gross Pathologic & Surgical Features 2. Love C et al: Diagnosing spinal osteomyelitis: a
comparison of bone and Ga-67 scintigraphy and magnetic
• Necrotic bone resonance imaging. Clin Nucl Med. 25(12):963-77, 2000
• Suppurative soft tissue 3. Wagner SC et al: Can imaging findings help differentiate
spinal neuropathic arthropathy from disk space infection?
Microscopic Features Initial experience. Radiology. 214(3):693-9, 2000
• Bone/disc fragments 4. Carragee E]: The clinical use of magnetic resonance
• Leukocytes, micro-organisms, cellular debris imaging in pyogeniC vertebral osteomyelitis. Spine.
• Vascular proliferation 22(7):780-5, 1997
5. Dagirmanjian A et al: MR Imaging of Vertebral
Osteomyelitis Revisited. A]R. 167:1539-43, 1996
ICLINICAL ISSUES 6. Thrush A et al: MR Imaging of Infectious Spondylitis.
A]NR. 11:1171-80, 1990
7. Modic MT et al: Vertebral osteomyelitis: assessment using
Presentation MR. Radiology. 157(1):157-66, 1985
• Most common signs/symptoms
o Acute or chronic back pain
o Focal spinal tenderness
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PYOGENIC OSTEOMYELITIS
IIMAGE GALLERY 1
9
(Left) Sagittal T1 WI M R
shows C6 and C7 vertebral
endplate erosion with
associated hypointense
marrow and intervertebral
disc space narrowing. (Right)
Sagittal T2WI MR
demonstrates hyperintensity
in C6 vertebra, extending
into disc space and C7
vertebra. Prevertebral
phlegmon is also present.
Typical
(Left) Sagittal T2WI MR
shows vertebral collapse,
endplate erosion, and
hyperintense narrowed disc
space at L7-2, compatible
with discitis and vertebral
osteomyelitis. (Right) Axial
T1 C+ MR with fat
suppression reveals
enhancing paravertebral
phlegmon at LI-2.
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GRANULOMATOUS OSTEOMYELITIS
1
10
Sagittal graphic through lumbar spine depicts multifocal Sagittal STIR MR shows classic TS. There is an anterior
granulomatous osteomyelitis. Frank abscesses are intraosseous abscess in L5 with ventral extension.
present at L3-4 disc space and between spinous process Subligamentous spread of infection is present (arrows),
of L2 and L3. reaching anterior L4.
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GRANULOMATOUS OSTEOMYELITIS
Key Facts
1
Terminology • T2WI: Hyperintense marrow, disc, soft tissue 11
• Tuberculous spondylitis (TS): Pott disease infection
• Granulomatous infection of spine and adjacent soft • Marrow, subligamentous, discal, dural enhancement
tissue typically caused by tuberculosis or brucellosis • Diffuse or peripherally enhancing soft tissue
• Increased radionuclide uptake in spine and paraspinal
Imaging Findings soft tissue
• Gibbus vertebrae with relatively intact intervertebral
discs and large paraspinal abscesses in TS Top Differential Diagnoses
• Size: Multiple (non)contiguous vertebrae • PyogeniC spondylitis (PS)
• EndpIate irregularity and osteolysis • Fungal spondylitis
• Vertebral sclerosis • Spinal metastases
• Fusion across disc space in late TS and BS Clinical Issues
• NECT: Calcifications of chronic paravertebral • Chronic back pain
abscesses: TS > BS • Focal tenderness
• Osseous destruction
• Fever
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GRANULOMATOUS OSTEOMYELITIS
1 IPATHOLOGY o TS: Most prevalent in 50s
o BS: More common in 60s
12 General Features • Gender
• General path comments: Granulomatous destruction oM=FinTS
of spinal column with adjacent soft tissue infection o M:F = 2.4:1 in BS
• Etiology Natural History & Prognosis
o TS
• Prognosis depends on early diagnosis and institution
• Hematogenous spread or through lymphatics from of appropriate therapy
pulmonary origin • If untreated
• Initial inoculum in anterior vertebral body o Progressive vertebral collapse
• Spread to (non)adjacent vertebral bodies beneath o Irreversible neurologic deficits
longitudinal ligaments o Death
• Sparing of intervertebral disc due to lack of • With proper treatment
proteolytic enzymes o Favorable outcome with resolution of symptoms
• Paraspinal, subarachnoid dissemination of disease • Especially with early presentation and lack of
o BS neurologic deficits or spinal deformity
• Access to spine via hematogenous dissemination
• Direct extension to adjacent discs and vertebrae Treatment
o Other pathogens causing granulomatous • Antibrucellar medications highly effective
osteomyelitis (Streptomycis, Madurella) uncommon o Surgical debridement rarely indicated
• Epidemiology • Long term antituberculous medication for at least one
o Rising incidence of tuberculosis in past two decades year
o TS in less than 1% of patients with tuberculosis • Surgical decompression in setting of neurologic
o Concomitant pulmonary tuberculosis in about 10% deficits ± spinal deformity
of patients o Indicated in 10-25% of TS
o TS more aggressive in children o Laminectomy and debridement in absence of
• Kyphosis and cord compression more common vertebral destruction
o Brucellosis uncommon in US: 100-200 cases per year o Debridement and fusion if spinal deformity present
• Prevalent in Mediterranean, South and Central
America, Middle East with reported incidence
between 6-58% IDIAGNOSTIC CHECKLIST
• Associated abnormalities
o Intramedullary abscess Image Interpretation Pearls
o Arachnoiditis • Thoracic spondylitis with posterior element
involvement and large paraspinal abscesses suggestive
Microscopic Features ofTS
• Both TS and BS show caseating granulomas and • BS should be in differential diagnosis of lumbar
nonspecific inflammatory reaction spondylitis at L4 with associated bilateral sacroiliitis
• Acid fast bacilli isolated < 50% of time
• Brucellar species very difficult to culture
ISELECTED REFERENCES
1. Akman S et al: Magnetic resonance imaging of tuberculous
ICLINICAL ISSUES spondylitis. Orthopedics. 26(1):69-73, 2003
2. Narlawar RS et al: Isolated tuberculosis of posterior
Presentation elements of spine: magnetic resonance imaging findings in
• Most common signs/symptoms 33 patients. Spine. 27(3):275-81, 2002
o Chronic back pain 3. Gouliamos AD et al: MR imaging of tuberculous vertebral
o Focal tenderness osteomyelitis: pictorial review. Em Radiol. 11(4):575-9,
o Fever 2001
o Other signs/symptoms 4. Hadjipavlou AG et al: The effectiveness of gallium citrate
Ga 67 radionuclide imaging in vertebral osteomyelitis
• Paraparesis
revisited. Am] Orthop. 27(3):179-83, 1998
• Kyphosis 5. Sharif HS et al: Granulomatous spinal infections: MR
• Sensory disturbance imaging. Radiology. 177(1):101-7, 1990
• Bladder and bowel dysfunction 6. Sharif HS et al: Brucellar and tuberculous spondylitis:
• Clinical profile comparative imaging features. Radiology. 171(2):419-25,
o Gradual, insidious onset of symptoms results in 1989
diagnostic delay 7. Smith AS et al: MR imaging characteristics of tuberculous
• Mean delay of 14 weeks spondylitis vs vertebral osteomyelitis. A]NR. 10:619-25,
o Fever relatively infrequent in TS compared to PS 1989
o Neurologic deficits more common with TS compared
to BS and PS
Demographics
• Age
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GRANULOMATOUS OSTEOMYELITIS
IIMAGE GALLERY 1
13
(Left) Sagittal T1 WI MR of a
patient with TS shows a
well-defined intraosseous
lesion in L5, slightly
hypointense compared to
marrow, with surrounding
edema. There is ventral
subligamentous spread
(arrow). (Right) Sagittal
T2WI MR shows L5
intraosseous lesion, which is
hyperintense. Anterior
vertebral cortex of L5
appears eroded.
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OSTEOMYELITIS, C1-C2
1
14
Sagittal graphic shows osteomyelitis involving odontoid Coronal TlWI MR shows craniocervical osteomyelitis
with bone destruction, extension to anterior arch of Cl, with extensive soft tissue destruction involving CO-CI,
epidural abscess formation (open arrow). CI·C2 articulations (arrows) with lateral subluxation of
cranium.
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OSTEOMYELITIS, C1-C2
Key Facts
1
Imaging Findings Pathology 15
• Best imaging tool: MRI with contrast ± MRA; shows • Staph aureus most common organism in USA
bone involvement, prevertebral soft tissues, epidural
space, vertebral arteries Clinical Issues
• IV antibiotics, followed by prolonged oral antibiotic
Top Differential Diagnoses administration
• CI-C2 osteoarthritis • Surgical decompression/stabilization
• Rheumatoid arthritis
• Chordoma Diagnostic Checklist
• Primary bone tumor • Grisel syndrome: Inflammatory, non-traumatic
• Nasopharyngeal carcinoma subluxation of Cl-C2 following peripharyngeal
• Extension of skull base osteomyelitis infection
• Degenerative endplate changes • Attributed to laxity of transverse/alar ligaments by
• Odontoid fracture hyperemia
• Hemodialysis spondyloarthropathy • Associated with otitis media, pharyngitis,
• Gout retropharyngeal abscess, upper respiratory infections
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OSTEOMYELITIS, C1-C2
1 • May be indistinguishable from pyogenic disc space
IDIAGNOSTIC CHECKLIST
infection
16 Consider
Gout
• Rarely vertebral involvement with low signal • Grisel syndrome: Inflammatory, non-traumatic
involving endplates, similar to disc space infection subluxation of CI-C2 following peripharyngeal
infection
o Attributed to laxity of transverse/alar ligaments by
IPATHOLOGY hyperemia
o Associated with otitis media, pharyngitis,
General Features retropharyngeal abscess, upper respiratory infections
• General path comments o Present with pain, head tilt, restricted neck
o Staph aureus most common organism in USA movement
o Mycobacterium tuberculosis most common o Head position classically 20° tilt, 20° rotation giving
worldwide "cock-robin" position
o Brucella, Pseudomonas, Serratia & Candida Image Interpretation Pearls
organisms common in IV drug addicts,
• Severe Cl-2 subluxation: Check posterior circulation
immunocompromised patients
for infarcts due to vertebral compression, check for
o Infection starts as septic arthritis of Cl-2
medullary compression
• Etiology
o Hematogenous seeding to capillary ends/end
arterioles in subchondral regions
o Many vascular anastomoses
ISELECTED REFERENCES
• Pharyngovertebral plexus drains posterosuperior 1. Krishnan A et al: Craniovertebral junction tuberculosis: a
nasopharynx inferiorly to basiocciput penetrating review of 29 cases. J Comput Assist Tomogr. 25(2): 171-6,
2001
atlanto-occipital membrane
2. Magliulo G et al: Osteomyelitis of the skull base with
• Pharyngovertebral plexus ¢} lymphatics atypical onset and evolution. Ann Otol Rhinol Laryngol.
• Periodontoidal venous plexus ¢} 109(3): 326-30, 2000
pharyngovertebral veins 3. Chan LL et al: Imaging of mucormycosis skull base
• Batson plexus allows seeding from viscera osteomyelitis. AJNR Am J Neuroradiol. 21(5): 828-31, 2000
• Epidemiology 4. Subburaman N et al: Skull base osteomyelitis interpreted as
o Uncommon; most cervical infections CS & C6 malignancy. J Laryngol Otol. 113(8): 775-8, 1999
region 5. Chong VF et al: The retropharyngeal space: route of
o Risk factors include diabetes, drug abuse, tumour spread. Clin Radiol. 53(1): 64-7, 1998
6. Swift AC et al: Skull base osteitis following fungal sinusitis.
endocarditis, immunocompromise J Laryngol Otol. 112(1): 92-7, 1998
• Associated abnormalities: CI-C2 subluxation, 7. Jones DC et al: Oropharyngeal morbidity following
cervicomedullary junction compression; vertebral transoral approaches to the upper cervical spine. Int J Oral
artery compression Maxillofac Surg. 27(4): 295-8, 1998
8. Baker LL et al: Atlanto-axial subluxation and cervical
osteomyelitis: two unusual complications of
ICLINICAL ISSUES adenoidectomy. Ann Otol Rhinol Laryngol. 105(4):295-9,
1996
Presentation 9. Lam CH et al: Conservative therapy of atlantoaxial
osteomyelitis. A case report. Spine. 21(15):1820-3, 1996
• Most common signs/symptoms 10. Gormley W et al: Spontaneous atlantoaxial osteomyelitis:
o Neck pain, stiffness, limited range of motion, no longer a rare case? Case report. Neurosurgery.
dysphagia' 35(1):132-5; discussion 135-6, 1994
o Other signs/symptoms 11. Wetzel FT et al: Grisel's syndrome. Clin Orthop.
• Triad of fever, pain, neurologic deficit in minority (240):141-52, 1989
of cases 12. Wilson BC et al: Nontraumatic subluxation of the
• Cl-2 subluxation, medulla compression and atlantoaxial joint: Grisel's syndrome. Ann Otol Rhinol
motor/sensory deficit Laryngol. 96(6):705-8, 1987
13. Zigler JE et al: Pyogenic osteomyelitis of the occiput, the
• Vertebral artery compression with posterior atlas, and the axis. A report of five cases. J Bone Joint Surg
circulation infarction Am. 69(7):1069-73, 1987
Demographics
• Age: Adult> child
• Gender: No gender preference
Treatment
• LV. antibiotics, followed by prolonged oral antibiotic
administration
• Surgical decompression/stabilization
o Variable approaches; anterior debridement and
posterior cervical-occipital arthrodesis
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OSTEOMYELITIS, C1-C2
IIMAGE GALLERY 1
17
(Left) Sagittal TI C+ MR
shows c/·2 osteomyelitis
with extensive enhancement
of C2 body, odontoid with
extensive prevertebral
phlegmon (arrows). There is
atlanto-axial subluxation and
cord compression. (Right)
Sagittal T2WI MR shows
increased signal from
odontoid/body of C2 in this
case of C/-2 osteomyelitis.
Anterior arch of C/ is
involved by phlegmon
(arrow), with C/-2
subluxation and cord
compression.
Variant
(Left) Sagittal T2WI MR
shows tuberculous
spondylitis involVing C/-2
articulation, with C/-2
subluxation (arrow), dorsal
(open arrow) and ventral
(curved arrow) abscesses
(Courtesy R. Harnsberger,
MO). (Right) Sagittal TlWI
MR shows tuberculous
osteomyelitis involving C2
body (open arrow),
extending ventral to C/-2
articulation into prevertebral
soft tissues (arrows).
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SEPTIC FACET JOINT ARTHRITIS
1
18
Axial graphic through lumbar vertebra demonstrates Axial T2WI MR with fat suppression through lumbar
right facet joint osteomyelitis and abscess, extending into vertebra shows fluid intensity expanding left facet joint &
subarticular recess and posterior paraspinal muscle. extending posteriorly. There is hyperintense edema in
surrounding soft tissue.
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SEPTIC FACET JOINT ARTHRITIS
Key Facts
1
Terminology • Uptake of technetium 99m diphosphonate in 19
• Pyogenic facet joint infection posterior elements
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SEPTIC FACET JOINT ARTHRITIS
1 • Facet joint may be preserved • Clinical profile
o Acute back pain mimics disc herniation
20 o Difficult to distinguish from spondylodiscitis
[PATHOLOGY clinically
General Features Demographics
• General path comments: Hematogenous infection of • Age: 50-60s
facet joint and adjacent soft tissue • Gender: Slight male predominance
• Genetics: No genetic predisposition • Ethnicity: No racial predilection
• Etiology Natural History & Prognosis
o Predisposing factors
• Intravenous drug abuse • Osseous destruction
• Immunocompromised state • Spinal instability
• Diabetes, cirrhosis, renal failure, cancer, other • Progressive neurologic deterioration
chronic medical illnesses • Sepsis and death
o Staphylococcus aureus most common • Favorable outcome with intravenous antibiotics alone:
o Bacteremia from an extraspinal primary source 71%
• Most common route of infection • Success rate improved if combined with percutaneous
• GU or GI tract, lungs, or cutaneous source drainage: 85%
o Direct inoculation from penetrating trauma, surgical Treatment
intervention, or diagnostic procedures • Intravenous antibiotics
• Facet joint injection • Percutaneous drainage
o Extension from adjacent infection in paraspinal soft
• Surgery
tissues o Decompressive laminectomy
• Diverticulitis, appendicitis, inflammatory bowl o Indicated when epidural abscess and neurological
disease deficits present
• Renal abscess
• Epidemiology
o Uncommon entity I DIAGNOSTIC CHECKLIST
• < 50 reported cases in literature
• 4-5% of infectious spondylitis in one series Consider
• Associated abnormalities • Imaging guided facet joint aspiration if blood culture
o Spondylodiscitis negative
o Epidural abscess: 25%
• Cord compression Image Interpretation Pearls
• Foraminal narrowing • Enhancing facet joint with juxtaarticular, paraspinal,
o Paraspinal abscess or epidural phlegmon or abscess characteristic of
o Spinal meningitis septic facet joint arthritis
Gross Pathologic & Surgical Features
• Necrotic bone ISELECTED REFERENCES
• Suppurative soft tissue
1. Coscia MF et al: Pyogenic lumbar facet joint arthritis with
Microscopic Features intradural extension: a case report. J Spinal Disord Tech.
15(6):526-8,2002
• Bone/synovial fragments 2. Muffoletto AJ et al: Hematogenous pyogenic facet joint
• Leukocytes, micro-organisms, cellular debris infection. Spine. 26:1570-6, 2001
• Granulation tissue 3. Muffolerro AJ et al: Hematogenous pyogenic facet joint
infection of the subaxial cervical spine. A report of two
cases and review of the literature. J Neurosurg. 95(1
ICLINICAL ISSUES Suppl):135-8, 2001
4. Rombauts PA et al: Septic arthritis of a lumbar facet joint
Presentation caused by Staphylococcus aureus. Spine. 25:1736-8, 2000
• Most common signs/symptoms 5. Fujiwara A et al: Septic arthritis of a lumbar facet joint:
o Acute or chronic back pain report of a case with early MRI findings. J Spinal Disord.
11(5):452-3, 1998
o Focal tenderness 6. Ergan M et al: Septic arthritis of lumbar facet joint. A
o Fever review of six cases. Rev Rhum Engl Ed. 64:386-95, 1997
o Other signs/symptoms 7. Baltz MS et al: Lumbar facet joint infection associated with
• Neurological impairment in 38% of cases epidural and paraspinal abscess. Clin Orthop. (339):109-12,
• Radiculopathy 1997
• Paraparesis 8. Swayne LC et al: Septic arthritis of a lumbar facet joint:
• Sensory disturbance detection with bone SPECT imaging. J Nucl Med.
30(8):1408-11, 1989
• Sphincter dysfunction
• Elevated erythrocyte sedimentation rate,
C-reactive protein, white count
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SEPTIC FACET JOINT ARTHRITIS
IIMAGE GALLERY 1
21
Typical
(Left) Axial T2WI MR with fat
suppression through a
lumbar vertebra shows fluid
in left facet joint with
posterior extension and
loculation. Edema is present
in surrounding soft tissue.
(Right) Axial T1 C+ MR with
fat suppression shows
enhancement in left facet
joint with posterior abscess
(arrow). Enhancement also
present in epidural and
paraspinal soft tissue.
Typical
(Left) Axial T2WI MR
through lumbar vertebra
shows asymmetric
hyperintensity in right facet
joint. (Right) Axial T2WI MR
demonstrates a phlegmon
expanding right facet joint
with extension (arrows) into
posterior paraspinal muscle.
Typical
(Left) Axial PO/Intermediate
MR through a cervical
vertebra shows
hyperintensity in left facet
joint with adjacent marrow
edema. Blood culture was
positive for E. coli. (Right)
Axial bone CT through a
cervical vertebra shows
osseous destruction in right
facet joint with low density,
surrounding bony debris,
compatible with septic facet
arthritis.
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EPIDURAL ABSCESS
1
22
Sagittal graphic through lumbar spine demonstrates Sagittal T1 C+ MR through thoracic spine shows discitis
vertebral osteomyelitis with intervertebral abscess and osteomyelitis with contiguous enhancing ventral
extending ventrally and dorsally, narrowing central epidural phlegmon, narrowing central canal.
canal.
11
A. •
.~ \~
Metastasis Hematoma
,~
Disc Fragment Epi. Lipomatosis
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EPIDURAL ABSCESS
Key Facts
1
Terminology • Peripherally enhancing necrotic abscess 23
• Extradural spinal infection with abscess formation Top Differential Diagnoses
Imaging Findings • Extradural metastasis
• Best diagnostic clue: Findings of spondylodiscitis, • Epidural hematoma
with adjacent enhancing epidural phlegmon ± • Extruded/migrated disc
peripherally enhancing fluid collection Pathology
• Posterior epidural space (80%) > anterior (20%) > • Staphylococcus aureus is most common pathogen
circumferential (caudal to S2) • Mycobacterium tuberculosis is next most frequent
• Lower thoracic and lumbar> cervical and upper cause: 25%
thoracic • Cord symptoms likely from combination of
• Size: Multi-segmental compressive & ischemic effects
• Tl WI: Iso- to hypointense to cord
• T2WI: Hyperintense Clinical Issues
• Homogeneously or heterogeneously enhancing • Acute or subacute spinal pain and tenderness
phlegmon • Fever
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EPIDURAL ABSCESS
1 o Extension from adjacent infection in paraspinal soft
tissues
o Co-morbidities
o Duration between onset of neurologic deficits and
24 • Diverticulitis, appendicitis, pyelonephritis time of surgical intervention
o Anterior cranial-caudal epidural extension by • Early diagnosis & institution of treatment improve
tracking beneath posterior longitudinal ligament prognosis
o Tuberculous infection tends to spread beneath • Mortality rate 12-30%
anterior longitudinal ligament
• Intervertebral discs spared Treatment
o Cord symptoms likely from combination of • Emergent surgical decompression with drainage of
compressive & ischemic effects abscess
• Cord ischemia from compromised epidural venous o Even in absence of initial neurologic compromise
plexuses • Rapid progression of neurologic deficits can occur
• Epidemiology: 0.2 to 2.8 cases per 10,000 despite appropriate medical therapy
• Associated abnormalities • Early empiric antibiotics with broad spectrum
o Discitis coverage until causative pathogen isolated
o Osteomyelitis o Coverage should be effective against staphylococci,
o Paraspinal abscess gram negatives, and anaerobes
o Septic facet arthritis • Organism specific intravenous antibiotics followed by
o Spinal instability long term parenteral antibiotics for 6-8 weeks
• Medical therapy alone if
Microscopic Features o Substantial operative risks
• Leukocytes, micro-organisms, cellular debris o Extensive cranial-caudal involvement of spinal canal
• Granulation tissue o Paralysis> 3 days
• Increased vascularity
IDIAGNOSTIC CHECKLIST
ICLINICALISSUES
Image Interpretation Pearls
Presentation • Epidural soft tissue with homogeneous or peripheral
• Most common signs/symptoms enhancement plus adjacent spondylodiscitis
o Acute or subacute spinal pain and tenderness characteristic of SEA
o Fever
o Other signs/symptoms
• Radiculopathy ISELECTED REFERENCES
• Paraparesis 1. Eastwood]D et al: Diffusion-weighted imaging in a patient
• Paresthesia with vertebral and epidural abscesses. A]NR Am]
• Loss of bladder and bowel control Neuraradiol. 23(3):496-8, 2002
• Paralysis 2. Varma R et al: Imaging of pyogenic infectious
• Clinical profile spondylodiskitis. Radiol Clin North Am. 39(2):203-13,
o In patients with septicemia or chronic illness 2001
neurologic symptoms may be obscured by systemic 3. Reihsaus E et al: Spinal epidural abscess: a meta-analysis of
915 patients. Neurasurg Rev. 23(4):175-204; discussion
complaints 205, 2000
o Lumbar SEA mimics disc herniation 4. Ruiz A et al: MR imaging of infections of the cervical spine.
o Abscess from hematogenous spread progresses Magn Reson Imaging Clin N Am. 8(3):561-80, 2000
rapidly 5. Tung GA et al: Spinal epidural abscess: correlation between
• Abscess from discitis/osteomyelitis tends to MRI findings and outcome. Neuroradiology. 41(12):904-9,
smolder 1999
6. Sampath P et al: Spinal epidural abscess: a review of
Demographics epidemiology, diagnosis, and treatment. ] Spinal Disord.
12(2):89-93, 1999
• Age
o All ages reported 7. Mackenzie AR et al: Spinal epidural abscess: the importance
of early diagnosis and treatment. ] Neural Neurosurg
o Peak incidence in 60s and 70s
Psychiatry. 65:209-12, 1998
• Gender: M:F := 1:0.56 8. Khanna RK et al: Spinal epidural abscess: evaluation of
• Ethnicity: No racial predilection factors influencing outcome. Neurosurgery. 39(5):958-64,
1996
Natural History & Prognosis 9. Numaguchi Y et al: Spinal epidural abscess: evaluation with
• Irreversible neurologic deficit and death if untreated or gadolinium-enhanced MR imaging. Radiographies.
delay in treatment 13(3):545-59; discussion 559-60, 1993
• Prognosis influenced by 10. Nussbaum ES et al: Spinal Epidural Abscess: A Report of 40
o Age Cases and Review. Surg Neural. 38:225-31, 1992
• Children with better prognosis than adults 11. Sandhu FS et al: Spinal epidural abscess: evaluation with
contrast-enhanced MR imaging. A]NR Am] Neuroradiol.
o Severity of initial neurologic deficits
12(6):1087-93, 1991
• Thecal compression> 50% associated with poor
prognosis
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EPIDURAL ABSCESS
IIMAGE GALLERY 1
25
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SUBDURAL ABSCESS
1
26
I . r •
." ll. 4. ···.·_f
.. ~.;-/. I~~
. \ ,. \ ~ '.
'-II Y
'I·.~~~~ ~.r .... ..
Sagittal T7 C+ MR through thoracolumbar spine shows
~~.~
Axial T7 C+ MR confirms subdural location of this
an extensive ventral intraspinal rim-enhancing, fluid collection, compatible with subdural abscess.
collection (arrows). Enhancing nerve roots are also present, due to spinal
meningitis.
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SUBDURAL ABSCESS
Key Facts
1
Terminology Pathology 27
• Spinal subdural empyema • Staphylococcus aureus most common
• Collection of pus in "potential" space between dura
and arachnoid Clinical Issues
• Fever
Imaging Findings • Neck or back pain
• Best diagnostic clue: Intradural extramedullary • Paraparesis, quadriparesis
ring-enhancing fluid collection on axial imaging • Paresthesia, sensory level
• Intermediate signal intensity (SI) • Laminectomy and durotomy for abscess drainage and
• Effaced CSF irrigation
• Heterogeneous, diffuse subdural enhancement
• Rim-enhancing fluid collection Diagnostic Checklist
• (Rim)-enhancing intradural extramedullary collection
Top Differential Diagnoses suspicious for SSA
• Spinal epidural abscess • Especially if outlined by epidural fat and CSF on axial
• Subdural hematoma imaging
• Spinal meningitis
• Epidural fat, enhancing dura and subdural abscess, • Smooth or nodular leptomeningeal or nerve root
CSF, cord enhancement
• Cord displacement • ± Cord edema
o ± Impingement
o ± Edema CSF leakage syndrome
• From prior spinal trauma, diagnostic or interventional
Nuclear Medicine Findings procedure, or spontaneous
• Gallium Scan • Low opening pressure on lumbar puncture
o Increased intraspinal uptake of gallium citrate (Ga • Diffuse smooth dural thickening and enhancement
67) o Likely from dural venous engorgement
• Improved sensitivity with SPECT • May see cerebellar tonsillar descent and effaced
pre-pontine space in posterior fossa
Imaging Recommendations
• Best imaging tool: Sagittal and axial TlWI and T2WI Idiopathic hypertrophic spinal
MR pachymeningitis
• Protocol advice • Unknown etiology
o Intravenous gadolinium increases sensitivity in
• Diagnosis of exclusion
detecting SSA • Fibrous inflammatory thickening of dura mater
• Axial imaging confirms subdural location • Thickened dura iso- to hypointense on TlWI and
T2WI
o Marked post-contrast-enhancement
IDIFFERENTIAL DIAGNOSIS
Spinal epidural abscess
• Difficult to distinguish clinically
IPATHOLOGY
• Lower thoracic and lumbar spine General Features
• Typically associated with spondylodiscitis • General path comments: Suppuration of spinal
• Intermediate SI on TlWI, hyperintense on T2WI subdural space
o Diffuse or rim-enhancement
• Genetics: No genetic predilection
• Variable mass effect on spinal cord
• Etiology
Subdural hematoma o Predisposing factors
• Other than trauma, lumbar puncture in patients with • Intravenous drug abuse
coagulopathy is the most common cause • Immunocompromised state
• More common in lumbar or thoracolumbar region • Alcoholism
• Predominantly hypointense on T2WI or gradient echo • Diabetes, cirrhosis, renal failure, or other chronic
o Isointense on TlWI medical illnesses
• No post-gadolinium enhancement o .Mechanism of inoculation
• Hematogenous dissemination from extraspinal
Spinal meningitis focus of infection
• Hematogenous dissemination of bacteria from • Mucous/cutaneous source most common
extraspinal source • Contiguous spread from adjacent abscess
• Increased CSF intensity on TlWI • Direct inoculation through trauma or
• Diffuse CSF enhancement interventional procedures
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SUBDURAL ABSCESS
1 • Lumbar puncture, cervical/lumbar discogram
Treatment
• Unexplained source of infection
28 o Common pathogens • Supportive care with hydration and pain management
• Staphylococcus aureus most common • Intravenous dexamethasone to decrease inflammatory
• Gram negative bacilli reaction and brain/cord edema
o Cord symptoms likely from a combination of • Percutaneous imaging guided catheter drainage
compressive and ischemic effects • Surgery
• Epidemiology o Laminectomy and durotomy for abscess drainage
o Rare and irrigation
o About 50 reported cases of SSA in the literature o Recommended in all surgical candidates
• Associated abnormalities • Empiric intravenous antibiotics with Gram-positive
o Spinal meningitis cocci coverage until positive culture results
o Spinal epidural abscess
o Usually not associated with vertebral osteomyelitis
• Anatomy I DIAGNOSTIC CHECKLIST
o Subdural space
Image Interpretation Pearls
• "Potential" space between dura mater and
arachnoid membrane • (Rim)-enhancing intradural extramedullary collection
o Factors contributing to low incidence of SSA suspicious for SSA
compared cranial subdural abscess o Especially if outlined by epidural fat and CSF on
• Lack of venous sinuses axial imaging
• Epidural space may act as a filter
• Centripetal blood flow in the spine vs. centrifugal
blood flow in the brain ISELECTED REFERENCES
1. Ozates M et al: Spinal subdural tuberculous abscess. Spinal
Gross Pathologic & Surgical Features Cord. 38(1):56-8, 2000
• Turbid CSF 2. Schneider P et al: Spinal subdural abscess in a pediatric
• Frank pus patient: a case report and review of the literature. Pediatr
Emerg Care. 14(1):22-3, 1998
3. Martin RJ et al: Neurosurgical care of spinal epidural,
subdural, and intramedullary abscesses and arachnoiditis.
I CLINICAL ISSUES Orthop Clin North Am. 27(1):125-36, 1996
4. Sathi S et al: Spinal subdural abscess: successful treatment
Presentation with limited drainage and antibiotics in a patient with
• Most common signs/symptoms AIDS. Surg Neurol. 42(5):424-7, 1994
o Fever 5. Levy ML et al: Subdural empyema of the cervical spine:
o Neck or back pain clinicopathological correlates and magnetic resonance
o Tenderness imaging. Report of three cases. J Neurosurg. 81(1):160,
o Other signs/symptoms 1994
• Paraparesis, quadriparesis 6. Krauss WE et al: Infections of the dural spaces. Neurosurg
Clin N Am. 3(2):421-33, 1992
• Paresthesia, sensory level 7. Bartels RH et al: Spinal subdural abscess. Case report. J
• Gait disturbance Neurosurg. 76(2):307-11, 1992
• Meningismus 8. Harries-Jones R et al: Meningitis and spinal subdural
• Hyporeflexia empyema as a complication of sinusitis. J Neurol
• Bladder or bowl dysfunction Neurosurg Psychiatry. 53(5):441, 1990
• Clinical profile 9. Hershkowitz S et al: Spinal empyema in Crohn's disease. J
o Triad of fever, neck or back pain, and cord Clin Gastroenterol. 12(1):67-9, 1990
compression 10. Lownie SP et al: Spinal subdural empyema complicating
cervical discography. Spine. 14(12):1415-7, 1989
• 38% of reported cases 11. Butler EG et al: Spinal subdural abscess. Clin Exp Neurol.
o Source of infections such as cellulitis, furuncles, 25:67-70, 1988
dental abscess may be present 12. Knudsen LL et al: Computed tomographic myelography in
o Difficult to distinguish from spinal epidural abscess spinal subdural empyema. Neuroradiology. 29(1):99, 1987
clinically 13. Harris LF et al: Subdural empyema and epidural abscess:
recent experience in a community hospital. South Med J.
Demographics 80(10):1254-8, 1987
• Age: All ages reported 14. Theodotou B et al: Spinal subdural empyema: diagnosis by
• Gender: M:F = 1:2 spinal computed tomography. Surg Neurol. 21(6):610-2,
• Ethnicity: No racial predilection 1984
15. Reddy DR et al: Pneumococcal spinal subdural abscess (a
Natural History & Prognosis case report). J Postgrad Med. 19(4):190-2, 1973
• If left untreated, progressive neurologic deterioration 16. Hirson C: Spinal subdural abscess. Lancet. 2(7424):1215-7,
1965
o Sepsis, death
• 20% survival rate if intravenous antibiotics only
• 82% survival rate if surgical treatment
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SUBDURAL ABSCESS
I IMAGE GALLERY 1
29
Typical
(Left) Axial T2WI MR
through lumbar spine shows
a left-sided intraspinal
hyperintense collection
compressing cauda equina.
(Right) Axial Tl C+ MR with
fat suppression shows the
same fluid collection with
penphe~/enhancemen~
proven to be a subdural
abscess at surgery.
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PARASPINAL ABSCESS
1
30
Axial graphic through a lumbar disc space demonstrates Axial Tl C+ MR with fat suppression in patient with
an extensive abscess infiltrating bilateral psoas muscles tuberculous spondylitis shows intraosseous & right psoas
and epidural space. Abnormal retroperitoneal lymph abscesses, plus prevertebral phlegmon, abscess.
nodes are also present.
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PARASPINAL ABSCESS
Key Facts
1
Terminology • Real Time: Anechoic or hypoechoic intramuscular 31
• Infection of soft tissues surrounding spine collection
• Power Doppler: Hyperemia and increased vascularity
Imaging Findings surrounding abscess
• Best diagnostic clue: Paravertebral enhancing • Increased uptake on In-Ill WBC scan
phlegmon or peripherally enhancing liquified
collection
Top Differential Diagnoses
• Ill-defined infiltrative paraspinal soft tissue • Neoplasm, primary or metastatic
• Focal or diffuse muscle enlargement • Retroperitoneal hematoma
• Obliterated soft tissue fascial plane • Extramedullary hematopoiesis
• Low density intra-muscular collection Pathology
• Intra-abscess gas • General path comments: Suppuration of paraspinal
• Calcified psoas abscesses characteristic of tuberculous soft tissue from direct extension or hematogenous
paraspinal abscess dissemination of pathogens
• T2WI: Hyperintense
• TI C+: Diffuse or peripheral enhancement
o Hyperintense on T2WI
MR Findings • Lymphoma with intermediate signal intensity (SI)
• TIWI • Desmoid, calcifications with low SI
o Iso- to hypointense o Post-gadolinium enhancement
• Difficult to distinguish from normal musculature • Necrotic component mimics abscess
• T2WI: Hyperintense • Variable vertebral involvement
• PD/lntermediate: Hyperintense o Intervertebral discs typically spared
• STIR: Hyperintense
• T2* GRE: Iso- to hyperintense Retroperitoneal hematoma
• TI C+: Diffuse or peripheral enhancement • Infiltrative soft tissue
• Features of spondylitis o Hyperdense on CT
o Hyperintense disc on T2WI o Hypointense on TIWI and T2WI in acute stage
o Endplate erosion o Hyperintense on TIWI in later stage
o Marrow edema o ± Mild enhancement
o Enhancing disc and marrow • Diffuse muscular enlargement
• Intraspinal extension with cord compression o Fluid-fluid level present if anti-coagulated
Ultrasonographic Findings Extramedullary hematopoiesis
• Real Time: Anechoic or hypoechoic intramuscular • Along thoracic and lumbar spine
collection • Paravertebral mass
• Power Doppler: Hyperemia and increased vascularity o Homogeneous and well-defined
surrounding abscess o Iso- to hypointense on TlWI
o Iso- to hyperintense on T2WI
Nuclear Medicine Findings o ± Mild enhancement
• Gallium Scan • Diffuse vertebral marrow hypointensity
o Increased radionuclide uptake o Relatively hyperintense intervertebral discs spared
• WBC Scan
o Increased uptake on In-Ill WBC scan
Imaging Recommendations IPATHOLOGY
• Best imaging tool: Sagittal and axial TIWI and T2WI General Features
MRI with gadolinium • General path comments: Suppuration of paraspinal
• Protocol advice soft tissue from direct extension or hematogenous
o T2WI with fat suppression or STIR improves dissemination of pathogens
detection of early paraspinal inflammation • Genetics: No genetic predisposition
o Post-gadolinium Tl WI with fat suppression also
• Etiology
improves lesion conspicuity o Most common pathogens
• Staphylococcus aureus
• Mycobacterium tuberculosis
IDIFFERENTIAL DIAGNOSIS • Escherichia coli
Neoplasm, primary or metastatic o Fungal infection rare
• More common in immunocompromised host
• Discrete or infiltrative soft tissue mass
o Predisposing factors
o Isointense to muscle on Tl WI
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PARASPINAL ABSCESS
1 • Intravenous drug abuse
• Immunocompromised state
• Analgesic medications
• Percutaneous catheter drainage
32 • Diabetes mellitus, alcoholism, cirrhosis, chronic • Surgical debridement
renal failure, and other chronic medical illnesses o Spinal stabilization may be required if instability
o Direct extension from adjacent infection present
• Spondylodiscitis
• Septic facet arthritis
• Appendicitis I DIAGNOSTIC CHECKLIST
• Diverticulitis
• Inflammatory bowel disease Image Interpretation Pearls
• Perinephric abscess • Peripherally enhancing collection in paravertebral soft
o Transcutaneous infection of deep tissue tissue with associated spondylitis characteristic of PA
• Trauma
• Epidural injection or catheter placement
• Facet joint injection ISELECTED REFERENCES
• Spine or GI surgery 1. Martinez V et al: Tuberculous paravertebral abscess. Lancet.
o Hematogenous spread from distant sites 363(9409):615, 2004
o Primary PA uncommon 2. Ledermann HP et al: MR imaging findings in spinal
• Epidemiology: > 90% cases of tuberculous spondylitis infections: rules or myths? Radiology. 228(2):506-14, 2003
associated with intra- or paraspinal abscess 3. Gouliamos AD et al: MR imaging of tuberculous vertebral
• Associated abnormalities: Epidural abscess osteomyelitis: pictorial review. Eur Radiol. 11(4):575-9,
2001
Gross Pathologic & Surgical Features 4. Hill JS et al: A Staphylococcus aureus paraspinal abscess
associated with epidural analgesia in labour. Anaesthesia.
• Necrotic soft tissue with thick green brown fluid
56(9):873-8,2001
Microscopic Features 5. Veillard E et al: Prompt regression of paravertebral and
epidural abscesses in patients with pyogenic discitis.
• Leukocytes, micro-organisms, cellular debris Sixteen cases evaluated using magnetic resonance imaging.
• Granulation tissue, increase vascularity Joint Bone Spine. 67(3):219-27, 2000
6. Tzen KY et al: The role of 67Ga in the early detection of
spinal epidural abscesses. Nucl Med Commun.
ICLINICAL ISSUES 21(2):165-70,2000
7. Cook NJ et al: Paraspinal abscess follOWing facet joint
Presentation injection. Clin Rheumatol. 18(1):52-3, 1999
• Most common signs/symptoms 8. Raj V et al: Paraspinal abscess associated with epidural in
o Fever labour. Anaesth Intensive Care. 26(4):424-6, 1998
9. Staatz G et al: Spondylodiskitic abscesses: CT-guided
o Back pain and tenderness
percutaneous catheter drainage. Radiology. 208(2):363-7,
o Other signs/symptoms 1998
• Lower extremity pain 10. Pascaretti C et al: Epidural involvement in nontuberculous
• Paraspinal muscle spasm disk space infections. Incidence by magnetic resonance
o If epidural component present imaging, impact and prognosis. Rev Rhum Engl Ed.
• Weakness 64(10):556-61, 1997
• Paresthesia 11. Cecil M et al: Paraspinal pyomyositis, a rare cause of severe
• Sphincter dysfunction back pain: case report and review of the literature. Am J
Orthop. 26(11):785-7, 1997
• Clinical profile: Diagnosis may be delayed due to
12. Bertol V et al: Neurologic complications of lumbar epidural
chronic and insidious symptomatology analgesia: spinal and paraspinal abscess. Neurology.
Demographics 48(6):1732-3, 1997
13. Baltz MS et al: Lumbar facet joint infection associated with
• Age: 50-60s epidural and paraspinal abscess. Clin Orthop. (339):109-12,
• Gender: M:F = 3:1 1997
• Ethnicity: No racial predilection 14. Dagirmanjian A et al: MR imaging of vertebral
osteomyelitis revisited. AJR. 167:1539-43, 1996
Natural History & Prognosis 15. Nussbaum ES et al: Spinal tuberculosis: a diagnostic and
• Depends on host immune response management challenge. J Neurosurg. 83:243-7, 1995
o May be contained with early treatment 16. Shanley D]: Tuberculosis of the spine: imaging features.
o Overwhelming sepsis leading to death in debilitated AJR Am J Roentgenol. 164(3):659-64, 1995
host 17. Pegues DA et al: Infectious complications associated with
temporary epidural catheters. Clin Infect Dis. 19(5):970-2,
• Progressive neurological impairment if spondylitis ± 1994
epidural abscess present 18. Post MJ et al: Magnetic resonance imaging of spinal
• Dependent on infection. Rheum Dis Clin North Am. 17(3):773-94, 1991
o Co-morbidities 19. Smevik B et al: Computed tomography of a tuberculous
o Extent of spinal involvement paravertebral abscess. Z Kinderchir. 43(6):430-2, 1988
o Degree of neurologic compromise 20. Lee JK et al: Psoas muscle disorders: MR imaging.
Radiology. 160(3):683-7, 1986
Treatment
• Long term intravenous antibiotics
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PARASPINAL ABSCESS
IIMAGE GALLERY 1
33
Typical
(Left) Coronal Tl C+ MR
with fat suppression in
patient with tuberculous
spondylitis demonstrates
intraosseous abscess
spanning two vertebrae and
right psoas abscess. (Right)
Axial Tl C+ MR with fat
suppression in patient with
pyogenic spondylitis shows
circumferential enhancing
paravertebral phlegmon with
epidural extension,
narrowing spinal canal.
Typical
(Left) Axial T2WI MR
through thoracic spine shows
enlarged bilateral
paravertebral soft tissues
with multiple hyperintense
collections. (Right) Axial
TlWI MR shows centrally
nonenhancing abscesses
within bilateral enhancing
phlegmon. Epidural
extension is present (arrow).
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VIRAL MYELITIS
1
34
Sagittal T2WI MR shows long, edematous cervical cord Sagittal T7 C+ MR shows ill-defined enhancement of
segment in patient with onset of progressive weakness enlarged cord in patient with two day history of
and radieulopathy over two days. Consitent with ATM. progressive weakness and radieulopathy. Consitent with
ATM.
ITERMINOlOGY MR Findings
Abbreviations and Synonyms • TlWI
o Expanded cord, fills canal
• Acute transverse myelitis, viral, ATM o May see central low signal simulating syrinx, but
Definitions intensity higher than CSF
• Acute inflammatory insult of spinal cord due to direct • T2WI: Diffuse increase in signal intensity through
viral infection or post-viral immunologic attack involved segment
• Tl C+: Variable, non-focal enhancement of involved
cord segment
IIMAGING FINDINGS Imaging Recommendations
General Features • Best imaging tool: MRI
• Protocol advice: T2Wl with thin (3 mm) sagittal
• Best diagnostic clue: Swollen, edematous cord with
sections; Tl C+ to exclude focal lesion as cause of cord
segmental contiguous involvement
edema
• Location: Cervical, thoracic segments; isolated conus
involvement rare
• Size: From one segment to extensive cord involvement
• Morphology: Fusiform expansion of cord
IDIFFERENTIAL DIAGNOSIS
Radiographic Findings "Idiopathic" transverse myelitis
• Myelography: Focal or diffuse cord enlargement • Identical clinical picture
• No etiology found
CT Findings • Up to 40% of cases preceded by upper respiratory tract
• CECT infection
o May see vague contrast-enhancement • Presence of CSF lymphocytes and neutrophils
o Intrathecal contrast (post-myelogram) outlines indicative of some type of inflammation in most cases
symmetrically swollen cord • Typically long segment of cord involvement by
swelling, edema, vague diffuse enhancement
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VIRAL MYELITIS
Key Facts
1
Terminology • Multiple sclerosis (MS) 35
• Acute inflammatory insult of spinal cord due to direct • Acute disseminated encephalomyelitis (ADEM)
viral infection or post-viral immunologic attack • Neuromyelitis optica
• Spinal arteriovenous malformation (AVM)
Imaging Findings • Arteritis
• Best diagnostic clue: Swollen, edematous cord with • Acute cord infarct
segmental contiguous involvement
• Expanded cord, fills canal
Pathology
• May see central low signal simulating syrinx, but • Since eradication of polio, other enteroviruses most
intensity higher than CSF common etiology
• T2WI: Diffuse increase in signal intensity through • Herpes
involved segment Diagnostic Checklist
• T1 C+: Variable, non-focal enhancement of involved • Long, segmental cord enlargement and edema
cord segment without focal lesions strong diagnostic clue in
Top Differential Diagnoses patients with acute onset of myelopathy
• "Idiopathic" transverse myelitis
• "Idiopathic" likely because of incomplete evaluation • May be related to recent aortic aneurysm surgery or
and/or follow-up aortic dissection (vertebral artery dissection if cervical)
• May represent collateral damage of immune attack • More often due to atherosclerotic occlusion of ventral
following an infection already mopped up by host's spinal artery (of Adamkiewicz)
defenses • Conus involvement common (due to single ventral
spinal artery)
Multiple sclerosis (MS)
• up to 33% may have isolated cord lesions (no brain Mycoplasma myelitis
lesions) • Coincident with a mycoplasma pneumonitis
• Most lesions are focal (1-2 segments), may be multiple
• 20% demonstrate monosegmental involvement
• Acute lesions exhibit focal enhancement with short IPATHOLOGY
segment edema
• No peripheral nervous system involvement General Features
• 90% of cases show oligoclonal bands • General path comments: Swollen, soft cord
• Etiology
Acute disseminated encephalomyelitis o Since eradication of polio, other enteroviruses most
(ADEM) common etiology
• Mimic of multiple sclerosis • Coxsackie, echovirus, hepatitis, rubella, measles,
• Related to vaccination or immune insult mumps
• Monophasic illness o Herpes
• Ebstein-Barr, varicella zoster, cytomegalus virus,
Neuromyelitis optica herpes simplex, herpes virus 6, herpes B (monkey
• Myelitis and optic neuritis virus)
• Often a manifestation of ADEM, or systemic lUpus o Retrovirus
erythematosus • HTLV I and II, HIV
• In immunocompromised hosts
Spinal arteriovenous malformation (AVM) o Other
• High-flow AVM may cause acute myelopathy due to • Influenza, rabies, West Nile
bleed or venous thrombosis • Epidemiology
• Subacute onset of myelopathy, including edema, cord o 1/100,000 per year
swelling may be due to "steal" & resulting ischemia o More common in immunocompromised
• Dural AVM may cause stuttering symptoms or acute • Associated abnormalities
myelopathy (due to venous thrombosis ~ o Peripheral neuropathy
Foix-Alajouanine syndrome) o Encephalitis
Arteritis Gross Pathologic & Surgical Features
• Small vessel vasculitis may have no imaging • Edematous, boggy cord
abnormality • May have necrosis
Acute cord infarct Microscopic Features
• Acute stroke-like presentation • Lymphocytic infiltrate
• Motor signs predominate • Vascular thickening
• Demyelination
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VIRAL MYELITIS
1 ICLINICAL ISSUES unrelated bone marrow transplantation. Bone Marrow
Transplant. 24(12):1355-8, 1999
36 9. Nakajima H et al: Herpes simplex virus myelitis: clinical
Presentation manifestations and diagnosis by the polymerase chain
• Most common signs/symptoms reaction method. Eur Neurol. 39(3):163-7, 1998
o Limb weakness 10. Haanpaa M et al: CSF and MRI findings in patients with
o Areflexia acute herpes zoster. Neurology. 51(5):1405-11, 1998
o Other signs/symptoms 11. Ku B et al: Acute transverse myelitis caused by Coxsackie
• Sensory dysesthesia virus B4 infection: a case report. J Korean Med Sci.
13(4):449-53, 1998
• Pain 12. Salonen 0 et al: Myelitis associated with influenza A virus
• Clinical profile infection. J Neurovirol. 3(1):83-5, 1997
o Acute onset of weakness following febrile illness or 13. de Silva SM et al: Zoster myelitis: improvement with
upper respiratory tract infection antiviral therapy in two cases. Neurology. 47(4):929-31,
o Reaches nadir at 1 week 1996
o CSF shows elevated mononuclear counts, protein 14. Moulignier A et al: AIDS-associated cytomegalovirus
level infection mimicking central nervous system tumors: a
o Specific diagnosis through viral titers or polymerase diagnostic challenge. Clin Infect Dis. 22(4):626-31, 1996
15. Ebo DG et al: Herpes zoster myelitis occurring during
chain reaction on CSF samples treatment for systemic lupus erythematosus. J Rheumatol.
Demographics 23(3):548-50, 1996
16. Breningstall GN et al: Acute transverse myelitis and
• Age: Typically younger patients brainstem encephalitis associated with hepatitis A
• Gender: No preference infection. Pediatr Neurol. 12(2):169-71, 1995
• Ethnicity: All 17. Campi A et al: Acute transverse myelopathy: spinal and
cranial MR study with clinical follow-up. AJNR Am J
Natural History & Prognosis Neuroradiol. 16(1):115-23, 1995
• Most cases leave lasting residual; significant minority 18. Gilden DH et al: Varicella-zoster virus myelitis: an
resolve expanding spectrum. Neurology. 44(10):1818-23, 1994
• Disease course one to twelve weeks 19. Caldas C et al: Case report: transverse myelitis associated
with Epstein-Barr virus infection. Am J Med Sci.
Treatment 307(1):45-8, 1994
• Supportive; steroids and antiviral agents often used, 20. Kyllerman MG et al: PCR diagnosis of primary herpesvirus
variable efficacy type I in poliomyelitis-like paralysis and respiratory tract
disease. Pediatr Neurol. 9(3):227-9, 1993
21. Chang CM et al: Postinfectious myelitis, encephalitis and
encephalomyelitis. Clin Exp Neurol. 29:250-62, 1992
IDIAGNOSTIC CHECKLIST 22. Cumming W]: Myelitis and toxic, inflammatory and
infectious disorders. Curr Opin Neurol Neurosurg.
Consider 5(4):549-53, 1992
• Obtaining brain MRI to exclude multiple sclerosis, 23. Hwang YM et al: A case of herpes zoster myelitis: positive
ADEM magnetic resonance imaging finding. Eur Neurol.
31(3):164-7, 1991
Image Interpretation Pearls 24. Iwamasa T et al: Acute ascending necrotizing myelitis in
• Long, segmental cord enlargement and edema without Okinawa caused by herpes simplex virus type 2. Virchows
focal lesions strong diagnostic clue in patients with Arch A Pathol Anat Histopathol. 418(1):71-5, 1991
acute onset of myelopathy 25. Dawson DM et al: Acute nontraumatic myelopathies.
Neurol Clin. 9(3):585-603, 1991
26. Power C et al: Pathological and molecular biological
features of a myelopathy associated with HTLV-l infection.
ISELECTED REFERENCES CanJ Neurol Sci. 18(3):352-5, 1991
1. Jeha LE et al: West Nile virus infection: a new acute 27. Wiley CA et al: Acute ascending necrotizing myelopathy
paralytic illness. Neurology. 61(1):55-9, 2003 caused by herpes simplex virus type 2. Neurology.
2. Zandman-Goddard G et al: Transverse myelitis associated 37(11):1791-4, 1987
with chronic hepatitis C. Clin Exp Rheumatol. 21(1):111-3, 28. Awerbuch G et al: Demonstration of acute post-viral
2003 myelitis with magnetic resonance imaging. Pediatr Neural.
3. Fujimoto H et al: Epstein-Barr virus infections of the 3(6):367-9, 1987
central nervous system. Intern Med. 42(1):33-40, 2003
4. Karacostas D et al: Cytomegalovirus-associated transverse
myelitis in a non-immunocompromised patient. Spinal
Cord. 40(3):145-9, 2002
5. Gorson KC et al: Nonpoliovirus poliomyelitis simulating
Guillain-Barre syndrome. Arch Neurol. 58(9):1460-4, 2001
6. McMinn P et al: Neurological manifestations of enterovirus
71 infection in children during an outbreak of hand, foot,
and mouth disease in Western Australia. Clin Infect Dis.
32(2):236-42, 2001
7. Andersen 0: Myelitis. Curr Opin Neurol. 13(3):311-6,2000
8. Gruhn B et al: Successful treatment of Epstein-Barr
virus-induced transverse myelitis with ganciclovir and
cytomegalovirus hyperimmune globulin following
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VIRAL MYELITIS
IIMAG E GALLERY 1
37
Typical
(Left) Sagittal T2WI MR
shows focal lesion in lower
cervical cord which
demonstrates mild
expansion. Patient had onset
of weakness shortly after
viral illness. (Right) Sagittal
Tl C+ MR demonstrates
ill-defined focus of
enhancement in region of
cord which showed high
signal on T2WI: Patient
developed weakness shortly
after onset of viral illness.
Typical
(Left) Sagittal TlWI MR
shows large cervicothoracic
cord filling canal, central low
signal. Patient with leg
weakness, peripheral
neuropathy. (Right) Sagittal
T2WI MR depicts expanded,
edematous cervico-thoracic
cord in patient with
weakness, peripheral
neuropathy.
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HIV
1
38
Sagittal T2WI MR demonstrates intramedullary Axial T2WI MR shows hyperintensity within posterior
hyperintensity within posterior cervical cord in patient cervical cord in patient with HIV myelopathy (Courtesy
with HIV myelopathy (Courtesy Florian P. Thomas, MD, Florian P. Thomas, MD, PhD).
PhD).
• T2WI
ITERMINOlOGY -I
o May be normal
Abbreviations and Synonyms o Hyperintensity either diffusely or involving WM
• Synonyms: AIDS or HIV ~ myelopathy or myelitis tracts laterally & symmetrically
o Cord atrophy
Definitions • STIR: Foci of hyperintensity
• Myelopathy resulting from primary HIV infection • T1 C+: Visible lesions may enhance
Imaging Recommendations
IIMAGING FINDINGS • Best imaging tool: MRI C+
General Features
• Best diagnostic clue: Spinal cord T2 hyperintensity IDIFFERENTIAL DIAGNOSIS
which may show patchy enhancement
• Location: Thoracic> cervical; mid to low thoracic cord
B12 deficiency
with t rostral involvement as disease progresses • May appear identical to HIV myelopathy
• Morphology • Negative HIV test
o Most common: Atrophy (72%) Varicella Zoster virus
o Common • Intrinsic myelopathy
• Diffuse nonspecific T2 hyperintensity of spinal • PCR-positive for virus in CSF
cord with no definite pattern (29%)
• Atrophy + diffuse intrinsic abnormality (14%) CMV myelitis
o Classic: T2 hyperintensity involVing white matter • Cause of HIV-related polyradiculopathy
(WM) tracts laterally & symmetrically • MRI may show nerve root & conus leptomeningeal
thickening, enhancement
MR Findings • Characteristic intranuclear inclusions
• T1WI
o May be normal Multiple sclerosis
o Cord atrophy • Similar appearance
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HIV
Key Facts . .
1
liermmo
° I ~ • HTLVI (human T-cell leukemIa/lymphoma VIrus) 39
ogy .. • L m homa
• Synonyms: AIDS or HIV ~ myelopathy or myelItIs y p
• Myelopathy resulting from primary HIV infection Pathology
° dO • General path comments: Diagnosis is one of
Imaging Fm mgs exclusion based on clinical, laboratory, & radiologic
• Best diagnostic clue: Spinal cord T2 hyperintensity findings
which may show patchy enhancement • Most common cause of spinal cord disease in AIDS
• Location: Thoracic> cervical; mid to low thoracic patients is AIDS-associated myelopathy with
cord with 1 rostral involvement as disease progresses
• Best imaging tool: MRI c+ prevalence of 20-55%
Clinical Issues
Top Differential Diagnoses • Insidious onset of progressive spastic paraparesis with
• B12 deficiency ataxia, urinary problems & sensory loss
• Varicella Zoster virus
• CMV myelitis Diagnostic Checklist
• Multiple sclerosis • Need to exclude other treatable causes of myelopathy
• Transverse myelitis
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HIV
1 • In children, spinal cord WM is also effected • May be related to polymorphism of transmethyl
o Diffuse demyelination with axonal loss & a more cycle enzyme genes
40 prominent inflammatory infiltrate
o Typical VM is rarely observed Natural History & Prognosis
o Difference may be from different myelin structure or • Prognosis is poor
alternative pathologic mechanisms in children Treatment
• In rare cases there is evidence of partial remyelination
• MRI is essential in exclusion of other extrinsic or
• Pathology changes observed in brains of patients with
intrinsic processes, but there are caveats
AIDS are morphologically different with myelin pallor,
o Normal-appearing spinal cord is often seen in
microglial infiltrates, & reactive astrocytosis
patients with mild & severe myelopathy
Staging, Grading or Classification Criteria o Cord atrophy & signal abnormality are found in
• Criteria for diagnosis of AIDS-associated myelopathy both mildly & severely myelopathic patients
• (1) Male or female> 18 yrs with HIV-l infection • Historically no known treatment
• (2) AIDS-associated myelopathy, with or without o Treatment studies are underway using highly active
neuropathy & dementia antiretroviral therapy (HAART)
o (A) At least two of the following symptoms • Now less common with HAART
• Paresthesia &/or numbness in lower extremities or • Estimated < 10% of patients with AIDS now
all four limbs develop spinal cord pathology
• Weakness of limbs o Improvement with L-methionine has been reported
• Unsteady, stiff, or uncoordinated gait
• Sensation of electric shock through back and legs
on neck flexion IDIAGNOSTIC CHECKLIST
• Urinary frequency, urgency, incontinence, or
retention
Consider
• Fecal incontinence or retention • Need to exclude other treatable causes of myelopathy
• Sexual dysfunction with male erectile impairment Image Interpretation Pearls
o (B) At least two of the following signs • MRI is often noncontributory but may reveal
• Reduction in vibratory and/or position sensation unsuspected coexisting conditions or other
• Brisk deep tendon reflexes explanation for patient symptoms
• Abnormal plantar response • Spinal cord atrophy is the most common abnormal
• Lhermitte sign MR finding typically involving the thoracic cord with
• Spastic, ataxic, or ataxospastic gait or without cervical cord involvement
• (3) Signs & symptoms of AIDS-associated myelopathy
for at least 6 weeks
• (4) Abnormal somatosensory evoked potentials I SELECTED REFERENCES
• (5) No other determinable cause for spinal cord disease
by serologic & CSF studies 1. Portegies P et al: Guidelines for the diagnosis and
management of neurological complications of HIV
infection. Eur J Neurol. 11(5):297-304, 2004
2. Di Rocco A: HIV-associated Myelopathy. Curr Treatment
ICLINICAL ISSUES Options Infect Dis. 5:457-465, 2003
3. Di Rocco A: Diseases of the spinal cord in human
Presentation immunodeficiency virus infection. Semin Neurol.
• Most common signs/symptoms 19(2):151-5, 1999
o Insidious onset of progressive spastic paraparesis 4. Chong J et al: MR findings in AIDS-associated myelopathy.
with ataxia, urinary problems & sensory loss AJNR AmJ Neuroradiol. 20(8):1412-6, 1999
• Occurs in later stages of disease 5. Quencer RM: AIDS-associated myelopathy: clinical severity,
MR findings, and underlying etiologies. AJNR Am J
• May need to urinate every 2-3 hours Neuroradiol. 20(8):1387-8, 1999
• Difficulty in obtaining or maintaining an erection 6. Weiser B et al: Human immunodeficiency virus type 1
• Stiffness in legs develops, often accompanied by expression in the central nervous system correlates directly
cramps & spasms ~ walking becomes difficult with extent of disease. Proc Natl Acad Sci USA.
• Weakness with degrees of paralysis develops 87(10):3997-4001, 1990
• In final stages, there may be severe paralysis of 7. Eilbott DJ et al: Human immunodeficiency virus type 1 in
legs & loss of urinary control with incontinence spinal cords of acquired immunodeficiency syndrome
• Loss of anal sphincter control ~ incontinence patients with myelopathy: expression and replication in
macrophages. Proc Natl Acad Sci USA. 86(9):3337-41,
• Painless initially, in later stages cramps & spasms
1989
may cause serious discomfort or pain
• Arms & hands generally not effected
o May have acute myelitic syndrome occurring shortly
after seroconversion
• Clinical profile
o Very low CD4 + lymphocyte counts
o Often have vitamin B12 deficiency
• Relationship to pathogenesis not yet understood
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HIV
IIMAG E GALLERY 1
41
Typical
(Left) Micropathology, low
power with Luxol fast blue
stain, shows extensive
spongifarm changes within
white matter from vacuolar
myelopathy (Courtesy
Robert E. Schmidt, MO).
(Right) Micropathology, high
power with Luxol fast blue
stain, shows marked
vacuolation within white
matter from HIV myelopathy
(Courtesy Robert E. Schmidt,
MO).
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MYELITIS-CORD ABSCESS
1
42
Sagittal graphic shows irregular abscess cavity in cervical Sagittal T7 C+ MR shows extensive irregular
cord, with cord expansion and edema. enhancement involving cerebellum, cervical cord
reflecting Streptococcal abscesses (Courtesy }. Illes,
MOJ.
ITERMINOLOGY MR Findings
Abbreviations and Synonyms • TlWI
o Ill-defined low signal from expanded cord
• Intramedullary spinal cord abscess (ISCA) o Abscess cord may show focal low signal
Definitions • T2WI: Increased T2W signal from abscess core and
• Infection of spinal cord with necrosis surrounding edema; cord expansion
o Pyogenic infection most common • PD/Intermediate: Increased signal from abscess and
surrounding edema
• STIR: Good definition of cord edema
IIMAGING FINDINGS • DWI
o May show positive diffusion (reduced ADC) similar
General Features to brain abscess
• Best diagnostic clue: Ring-enhancing mass within • Lack of diffusion restriction does not exclude
cord, with appropriate clinical history of abscess
inflammation/infection • Tl C+: Irregular ring-enhancing lesion with cord
• Location:· Intramedullary expansion
• Size: Variable, usually less than 2 cm Imaging Recommendations
• Morphology: Round or oblong irregular area of
• Best imaging tool: MRI with contrast defines cord
ring-enhancement, with associated edema
abnormality, edema, enhancing abscess
Radiographic Findings • Protocol adVice: Sagittal, axial TlW, T2W images,
• Myelography: Nonspecific findings of symmetrical post-contrast sagittal, axial TlW
cord enlargement, or myelographic block from
intramedullary lesion
CT Findings
• NECT: Normal, or ill-defined cord irregularity and
expansion
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MYELITIS-CORD ABSCESS
Key Facts
1
Terminology Pathology 43
• Intramedullary spinal cord abscess (ISCA) • Routes of infection: Majority of cases
idiopathic/cryptogenic
Imaging Findings • Hematogenous spread (10%)
• Best diagnostic clue: Ring-enhancing mass within • Contiguous spread from adjacent infection (25% of
cord, with appropriate clinical history of these cases from dermal sinus tract)
inflammation/infection • Direct implantation sucK as trauma, or surgical
Top Differential Diagnoses • Multiple organisms involved
• Acute transverse/viral myelitis Clinical Issues
• Hypervascular cord neoplasms • Often present with signs/symptoms of structural cord
• Multiple sclerosis lesion, rather than infection
• Type I dural fistula • Mixed neurologic deficits including fever, pain,
• Cavernous malformation motor deficit, sensory disturbance, and sphincter
• Cord infarction dysfunction
• Intradural/extramedullary tumors
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MYELITIS-CORD ABSCESS
1 o Often present with signs/symptoms of structural
cord lesion, rather than infection 6.
Neurol Sci. 28(4): 354-6, 2001
Elmac I et al: Cervical spinal cord intramedullary abscess.
44 o Mixed neurologic deficits including fever, pain, Case report.] Neurosurg Sci. 45(4): 213-5; discussion 215,
2001
motor deficit, sensory disturbance, and sphincter
7. Ruiz A et al: MR imaging of infections of the cervical spine.
dysfunction Magn Reson Imaging Clin N Am. 8(3): 561-80, 2000
o Other signs/symptoms 8. Desai KI et al: Holocord intramedullary abscess: an unusual
• Incontinence, meningismus case with review of literature. Spinal Cord. 37(12): 866-70,
• Triad of fever, pain, neurologic deficit in 25% 1999
o Delay in diagnosis common (time from symptom 9. Mukunda BN et al: Solitary spinal intramedullary abscess
onset to diagnosis> 30 days) caused by Nocardia asteroides. South Med]. 92(12):
1223-4, 1999
• Fever < 50%
10. Murphy K] et al: Spinal cord infection: myelitis and abscess
o Children
formation. A]NR Am] NeuroradioL 19(2): 341-8, 1998
• Paralysis (60%) 11. Sverzut]M et al: Spinal cord abscess in a heroin addict: case
• Fever (> 50%) report. Neuroradiology. 40(7): 455-8, 1998
• Urinary retention (30%) 12. Chan CT et al: Intramedullary abscess of the spinal cord in
• Back pain (28%) the antibiotic era: clinical features, microbial etiologies,
• Nausea/vomiting (10%) trends in pathogenesis, and outcomes. Clin Infect Dis.
• 20% recover without neurologic sequelae 27(3): 619-26, 1998
13. Derkinderen P et al: Intramedullary spinal cord abscess
Demographics associated with cervical spondylodiskitis and epidural
• Age: Adults and children (mean age 34 years) abscess. Scand] Infect Dis. 30(6): 618-9, 1998
14. Friess HM et al: MR of staphylococcal myelitis of the
• Gender: M:F = 3: 1
cervical spinal cord. A]NR Am] NeuroradioL 18(3): 455-8,
• Ethnicity: None known 1997
Natural History & Prognosis 15. Martin R] et al: Neurosurgical care of spinal epidural,
subdural, and intramedullary abscesses and arachnoiditis.
• Associated with high mortality and morbidity Orthop Clin North Am. 27(1): 125-36, 1996
• Mortality of 24% reported from years 1944-1977 16. Banuelos AF et al: Central nervous system abscesses due to
o Mortality of 8% in antibiotic era (1977-1997) Coccidioides species. Clin Infect Dis. 22(2): 240-50, 1996
• Quick surgical drainage and aggressive intravenous 17. Chu]Y et al: Listeria spinal cord abscess--clinical and MRI
antibiotics required findings. Can J NeuroI Sci. 23(3): 220-3, 1996
• Persistent neurologic deficit with successful treatment 18. Lindner A et al: Magnetic resonance image findings of
> 70% spinal intramedullary abscess caused by Candida albicans:
case report. Neurosurgery. 36(2): 411-2, 1995
Treatment 19. pfadenhauer K et al: Spinal IJ;1anifestation of
neurolisteriosis.] NeuroL 242(3): 153-6, 1995
• Early surgical drainage followed by antibiotic therapy
20. Bartels RH et al: Intramedullary spinal cord abscess. A case
o Laminectomy with myelotomy and drainage report. Spine. 20(10): 1199-204, 1995
o LV. antibiotic coverage 4-6 weeks, with 2-3 21. King S] et al: MRI of an abscess of the cervical spinal cord
additional months oral antibiotics in a case of Listeria meningoencephalomyelitis.
• Surgically treated mortality approximately 14% Neuroradiology. 35(7): 495-6, 1993
IDIAGNOSTIC CHECKLIST
Consider
• Ampicillin empiric coverage in idiopathic cases for L.
monocytogenes
• Serial WBe count, ESR, enhanced MRI, important for
post-operative follow-up, detection of recurrence
Image Interpretation Pearls
• MR brain to exclude concomitant brain abscess
ISELECTED REFERENCES
1. Berger]R et al: Infectious myelopathies. Semin NeuroL
22(2): 133-42, 2002
2. Krishnan A et al: Craniovertebral junction tuberculosis: a
review of 29 cases.] Comput Assist Tomogr. 25(2): 171-6,
2001
3. Chidambaram B et al: Intramedullary abscess of the spinal
cord. Pediatr Neurosurg. 34(1): 43-4, 2001
4. Durmaz R et al: Multiple nocardial abscesses of cerebrum,
cerebellum and spinal cord, causing quadriplegia. Clin
Neurol Neurosurg. 103(1): 59-62, 2001
5. Rossi FH et al: Listeria spinal cord abscess responsive to
trimethoprim-sulfamethoxazole monotherapy. Can]
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MYELITIS-CORD ABSCESS
IIMAGE GALLERY 1
45
Typical
(Left) Sagittal Tl WI M R with
extensive cerebellar, cervical
cord Strep abscesses shows
diffuse cord expansion, loss
of CSF signal, and poor
definition of 4th ventricle
due to edema (Courtesy j.
JIles, MO). (Right) Sagittal
T2WI MR shows diffuse T2
hyperintensity involving
cerebellum, brain stem,
cervical cord from diffuse
Strep abscesses (Courtesy}.
Illes, MO).
Typical
(Left) Sagittal TlWI MR
shows diffuse cord
enlargement filling the thecal
sac from C7 to C7 level.
Abscess cavity at C6-7 level
is not defined without
contrast (Courtesy j. Illes,
MO). (Right) Sagittal Tl C+
MR shows irregular
ring-enhancement of cord at
C6 & C7 levels reflecUng
focal cord abscess (Courtesy
}. JIles, MO).
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SPINAL MENINGITIS
1
46
ITERMINOlOGY CT Findings
• NECT: Increased CSF density
Abbreviations and Synonyms
• CECT: Enhancing CSF ± meninges
• Infectious arachnoiditis
MR Findings
Definitions
• TIWI
• Infection of spinal cord leptomeninges and o Increased CSF intensity
subarachnoid space o Indistinct cord-CSF interface
o Irregular cord outline
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SPINAL MENINGITIS
Key Facts
1
Terminology Top Differential Diagnoses 47
• Infectious arachnoiditis • Carcinomatous meningitis
• Infection of spinal cord leptomeninges and • Sarcoidosis
subarachnoid space • Lumbar arachnoiditis
• Guillain-Barre syndrome
Imaging Findings • Intracranial hypotension
• Location: All spinal segments involved
• NECT: Increased CSF density Clinical Issues
• Increased CSF intensity • Acute onset of fever, chills, headache, + altered level
• Indistinct cord-CSF interface of consciousness
• Irregular cord outline
• Clumped nerve roots
• Smooth or nodular meningeal enhancement
• Homogenously enhancing CSF
• Smooth or nodular nerve root enhancement
• ± Segmental or focal intramedullary enhancement
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SPINAL MENINGITIS
1 • 2 months to 12 years: Haemophilus influenzae, • Neck stiffness
Streptococcus pneumoniae, & Neisseria • Paraparesis
48 meningitides • Paresthesia
• Adults: Above, plus streptococci + staphylococci • Gait disturbance
o Subacute meningitis: Symptoms develop in 1-7 days • Urinary bladder dysfunction
• Mostly viral (e.g., HIV-related CMV • Clinical profile: Milder symptoms with protracted
radiculomyelitis), some bacterial (e.g., Lyme course in tuberculous or fungal meningitis
disease)
o Chronic meningitis: Fluctuating symptoms for > 7 Demographics
days • Age
• Tuberculosis o Newborns
• Syphilis o Infants: Peak age 3-8 months
• Fungal: Coccidioidomycosis, cryptococcosis, and o Adults: 20s; 60s
aspergillosis • Gender
o Mechanism of inoculation o No gender preference among adults
• Hematogenous dissemination from extraspinal o M:F = 3:1 in neonates
focus of infection Natural History & Prognosis
• Contiguous spread from adjacent spondylodiscitis,
• Prognosis depends on severity of disease, causative
spinal epidural abscess
pathogen, patient age + co-morbidities
• Direct inoculation through trauma or o Mortality of bacterial meningitis: 20-90%
interventional procedures
• Depending on initial neurologic impairment &
• Unexplained source of infection: Probably bacteria
rate of progression
colonized in nasopharynx
• Chronic disabilities include paralysis, seizures,
o Pathophysiology of bacterial meningitis
deafness, etc.
• Initial acute inflammatory exudate in o Viral meningitis generally less severe
subarachnoid space
• Full recovery expected within 2 weeks in most
• Toxic mediators potentiate inflammatory response
cases
• Increased permeability of blood-cord barrier
• Influx of inflammatory cells Treatment
• Spinal cord swelling and edema likely due to • Supportive care with hydration and pain management
ischemia from vasculitis, venous congestion; • Intravenous dexamethasone to decrease inflammatory
and/or direct infection reaction and brain/cord edema
• Epidemiology: Incidence of bacterial meningitis: 2-3 • Empiric intravenous antibiotics based on suspected
per 100,000 organisms in each age group
• Associated abnormalities • Organism specific intravenous antibiotics
o Spondylodiscitis • Preventive oral antibiotics for close contacts of
o Spinal epidural abscess patients with Neisseria meningitides
o Subdural empyema
o Subarachnoid cysts
• Fibrin deposition results in loculation of I DIAGNOSTIC CHECKLIST
subarachnoid space
o Myelitis Image Interpretation Pearls
o Cord abscess • Imaging often negative in early spinal meningitis
o Syringomyelia • Increased CSF SI on T1WI with diffuse
• Blocked CSF flow results in increased pressure post-gadolinium enhancement suggestive of spinal
within cord and subsequent central canal meningitis
expansion
Microscopic Features ISELECTED REFERENCES
• Cellular debris, inflammatory cells, and
micro-organisms 1. Hunter ]V et al: Neuroimaging of central nervous system
infections. Semin Pediatr Infect Dis. 14(2):140-64,2003
• Tuberculous meningitis 2. Kaplan SI.: Clinical presentations, diagnosis, and
o Small tubercles consist of epithelioid cells, prognostic factors of bacterial meningitis. Infect Dis Clin
Langerhan giant cells, & foci of caseation North Am. 13(3):579-94, vi-vii, 1999
3. Meltzer CC et al: MR imaging of the meninges. Part I.
Normal anatomic features and nonneoplastic disease.
ICLINICAL ISSUES 4.
Radiology. 201(2):297-308, 1996
Post MD et al: Magnetic resonance imaging of spinal
Presentation infection. Rheum Dis Clin North Am. 17:773-94, 1991
• Most common signs/symptoms 5. Chang KH et al: Tuberculous arachnoiditis of the spine:
Findings on Myelography, CT, and MR imaging. A]NR.
o Acute onset of fever, chills, headache, + altered level
10:1255-62, 1989
of consciousness
o Other signs/symptoms
• Generalized convulsions
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SPINAL MENINGITIS
IIMAGE GALLERY 1
49
Typical
(Left) Sagittal T7 WI MR
through thoracic spine shows
diffusely increased CSF signal
intensity, obscuring cord-CSF
interface. (Right) Sagittal
T2WI MR with fat
suppression demonstrates
hyperintense CSF with areas
of subtle low intensities
(arrows) due to bacterial
meningitis. Mild mass effect
on thoracic cord is
suggested.
Typical
(Left) Sagittal T7 C+ MR with
fat suppression demonstrates
diffusely enhancing CSF,
with mild heterogeneity. CSF
culture confirmed bacterial
meningitis. (Right) Sagittal T7
C+ MR with fat suppression
through lumbar spine shows
enhancing CSF with
indistinct, enhancing nerve
roots.
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'ECHINOCOCCUS
1
50
Sagittal graphic of lumbar spine shows cystic mass Sagittal T2WI MR shows lobulated extradural
involving posterior elements with extension into dorsal heterogeneous T2 hyperintense mass (with permission
epidural space, thecal sac & cauda equina compression, Athanassopoulou et ai, MRI in spinal hydatid disease,
{online case 7855}, EuromultimediaJ,
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-~-"
e::
..i..:/J;,~ ':\~:. t~\'.'
.
~
Chondrosarcoma Osteosarcoma Renal Cell Met Chordoma
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ECHINOCOCCUS
Key Facts
1
Terminology Imaging Findings 51
• Synonyms: Hydatidosis, hydatid disease, hydatid cyst • Best diagnostic clue: Multiloculated, multiseptated T2
disease hyperintense vertebral body/posterior element mass
• Disease caused by cyst stage of infestation by without significant enhancement in endemic area for
tapeworm of genus echinococcus echinococcus
• Liver, lung involvement most common, bone • Location: Thoracic spine most common
involvement rare
• E. granulosus = causative agent of Hydatid disease in Pathology
man/other mammals • Intramedullary cyst
• Dog as definite host; parasitized by adult tapeworm • Intradural/extramedullary
• E. multilocularis = causative agent of alveolar hydatid • Extradural intraspinal
disease in man/other mammals • Vertebral disease
• E. vogeli = polycystic form in Central & South • Paravertebral
America Diagnostic Checklist
• Hydatid cyst fluid highly allergenic, cyst rupture may
cause anaphylaxis
MR Findings
• TIWI IPATHOLOGY
o CSF signal multiseptated/multicystic lesions
o Degenerated cysts may be isointense to muscle General Features
• T2WI • General path comments
o CSF signal multiseptated/multicystic lesions o Bone involvement rare: Incidence ranges from
o Epidural extension of soft tissue with cord 0.5-2% of all cases of echinococcosis
compression • Vertebrae most common bone involved
o Degenerated cysts may show low signal relative to • Vertebrae> long bone epiphyses> ilium> skull >
CSF ribs
• STIR: CSF signal multiseptated/multicystic lesions o No pericyst formation in bone involvement
• Tl C+: Minimal or no enhancement of large cystic • Parasite expands by exogenous proliferation with
lesions irregular pattern
• Bone resorption due to pressure erosion & necrosis
Imaging Recommendations o Typical involvement of vertebral bodies, contiguous
• Best imaging tool ribs & paravertebral tissue
o MR shows bone, epidural, paravertebral extension • Disc space generally spared
and degree of neural compromise • Etiology
o CT shows bone morphology, degree of bone o Usual cause is accidental ingestion of dog feces
destruction containing echinococcal eggs
• Protocol advice: Sagittal, axial Tl WI + T2WI, o Cysts slowly grow in vertebral body/posterior
post-contrast sagittal, axial TlWI elements with subsequent extension into
pre/paravertebral region, ribs, epidural space with
cord compression
IDIFFERENTIAL DIAGNOSIS • Epidemiology
o Echinococcus common in Southern South America,
Primary bone neoplasm Mediterranean, Middle East, Central Asia, Africa
• Osteosarcoma, chondrosarcoma, ABC, giant cell, o US: California, Arizona, New Mexico, Utah
chordoma
Gross Pathologic & Surgical Features
Metastatic disease • Spherical, fluid filled hollow cyst, containing multiple
• Cystic metastases; such as renal cell, thyroid protoscolices, brood capsules, daughter cysts
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ECHINOCOCCUS
1 Microscopic Features
5. Stabler A et al: Imaging of spinal infection. Radiol Clin
North Am. 39(1):115-35, 2001
52 • Outer laminated hyaline wall 6. Hassan FO et al: Primary pelVic hydatid cyst: an unusual
• Inner nucleated germinal layer, studded with cause of sciatica and foot drop. Spine. 26(2):230-232, 2001
developing brood capsules 7. Bruschi F et al: Immunochemical and molecular
o Protoscolices formed within brood capsules characterization of vertebral hydatid fluid. Scand J Infect
Dis. 31(3):322-3, 1999
Staging, Grading or Classification Criteria 8. Savas R et al: Spinal cord compression due to costal
Echinococcus multilocularis. Comput Med Imaging Graph.
• Five categories of spine involvement
23(2):85-8, 1999
o Intramedullary cyst 9. Normelli HC et al: Vertebral hydatid cyst infection
o Intradural!extramedullary (Echinococcus granulosus): a case report. Eur Spine J.
o Extradural intraspinal 7(2):158-61, 1998
o Vertebral disease 10. Mazyad MA et al: Spinal cord hydatid cysts in Egypt. J
o Paravertebral Egypt Soc Parasitol. 28(3):655-8, 1998
11. von Sinner WN et al: Case report 833: Primary spinal
echinococcosis (Echinococcus granulosus) of lumbosacral
spine with destruction of the left pedicles of L3-5 and
1 CLINICAL ISSUES extension of a large paraspinal cystic mass into the spinal
canal. Skeletal Radiol. 23(3):220-3, 1994
Presentation 12. Sharif HS: Role of MR imaging in the management of
• Most common signs/symptoms spinal infections. AJR Am J Roentgenol. 158(6):1333-45,
o Back pain, myelopathy, radiculopathy depending 1992
upon anatomic location of infestation 13. Dernevik L et al: Management of dumbbell tumours.
o Other signs/symptoms Reports of seven cases. Scand J Thorac Cardiovasc Surg.
• Progressive weakness, numbness of extremities 24(1):47-51, 1990
14. Richards KS et al: Effect of albendazole on human hydatid
Demographics cysts: an ultrastructural study. HPB Surg. 2(2):105-12;
• Age: Any age discussion 112-3, 1990
15. Charles RW et al: Echinococcal infection of the spine with
• Gender: No gender predilection
neural involvement. Spine. 13(1):47-9, 1988
• Ethnicity: Related to endemic geographic locations 16. Bhargava S: Radiology--including computed
Natural History & Prognosis tomography--of parasitic diseases of the central nervous
system. Neurosurg Rev. 6(3):129-37, 1983
• Indolent, progressive course without treatment 17. Braithwaite PA et al: Vertebral hydatid disease: radiological
assessment. Radiology. 140(3):763-6, 1981
Treatment 18. Kaufman DM et al: Infectious agents in spinal epidural
• Oral treatment with albendazole or mebendazole abscesses. Neurology. 30(8):844-50, 1980
• Surgical resection for neural compression 19. Karvounis PC et al: Intradural spinal echinococcus
• Recurrence rate of spinal hydatid cysts after surgery simulating lumbar disc protrusion. Neurochirurgia (Stuttg).
18% 20(2):58-60, 1977
IDIAGNOSTIC CHECKLIST
Consider
• Hydatid cyst fluid highly allergenic, cyst rupture may
cause anaphylaxis
• Disease is indolent with latent period of multiple years
before cysts reach symptomatic size
Image Interpretation Pearls
• Lack of significant enhancement is critical differential
feature of echinococcal cysts
ISELECTED REFERENCES
1. Hadjipavlou AG et al: Effectiveness and pitfalls of
percutaneous transpedicle biopsy of the spine. Clin
Orthop. (411):54-60, 2003
2. El Kohen A et al: Multiple hydatid cysts of the neck, the
nasopharynx and the skull base revealing cervical vertebral
hydatid disease. Int J Pediatr Otorhinolaryngol.
67(6):655-62,2003
3. Karadereler S et al: Primary spinal extradural hydatid cyst
in a child: case report and review of the literature. Eur
Spine J. 11(5):500-3, 2002
4. Sener RN et al: Multiple, primary spinal-paraspinal hydatid
cysts. Eur Radiol. 11(11):2314-6,2001
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ECHINOCOCCUS
IIMAGE GALLERY 1
53
(Left) Sagittal T7 WI M R
shows nonspecific lobulated
vertebral mass involving
posterior elements (with
permission Athanassopoulou
et ai, MRI in spinal hydatid
disease, {online case 18SS},
Euromultimedia). (Right)
Sagittal T7WI MR shows
intermediate signal mass
involving thoracic vertebral
body (with permission
Athanassopoulou et ai, MRI
in spinal hydatid disease,
{online case 18SS},
Euromultimedia).
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SCHISTOSOMIASIS
1
54
Sagittal graphic shows schistosomiasis involving conus Sagittal T2WI MR shows diffuse cord T2 hyperintensity
medullaris with granulomatous inflammatory response. centered on conus medullaris, with moderate cord
expansion in patient with schistosomiasis (Courtesy R.
Mendonca, MO).
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SCHISTOSOMIASIS
Key Facts
1
Terminology • Pyogenic abscess 55
• Synonym: Bilharziasis • Viral or idiopathic transverse myelitis
• CNS infection from parasitic trematodes (blood • Spinal dural fistula
flukes) of genus Schistosoma • Sarcoidosis
• Infect particular species fresh water snails within Pathology
endemic areas • Vascular obstruction secondary to granuloma
Imaging Findings formation with intense inflammatory reaction,
• Best diagnostic clue: Myelopathy, cord enhancement, ischemic necrosis
edema in patient from endemic area Clinical Issues
Top Differential Diagnoses • 3 Clinical patterns of cord involvement
• Multiple sclerosis • Myelitic
• ADEM • Granulomatous/pseudotumoral
• Intramedullary neoplasm • Radicular/myeloradicular
• Infarction • Oral treatment with antihelmintics: Praziquantel and
• Tuberculosis oxamniquine
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SCHISTOSOMIASIS
1 o Prevalence of CNS involvement varies from 1-30%
of infected individuals
• End-stage hepatosplenic disease, varices, pulmonary
hypertension, CNS disease associated with high
56 o Spinal cord schistosomiasis one of most common mortality
causes of non-traumatic myelopathies in endemic
areas Treatment
• Associated abnormalities • Oral treatment with antihelmintics: Praziquantel and
o Egg retention & granuloma formation in bowel oxamniquine
causes bloody diarrhea, cramping, inflammatory • Steroids to decrease inflammatory response
colon polyps
o Urinary tract involvement leads to dysuria,
hematuria, bladder polyps, ulcers, obstructive IDIAGNOSTIC CHECKLIST
uropathy, increased risk of bladder squamous
carcinoma
Consider
o Unshed eggs =} portal circulation =} hepatic • Patients with spinal involvement may have no other
perisinusoidal obstruction, portal hypertension, clinical evidence of schistosomiasis
splenomegaly, ascites
Microscopic Features ISELECTED REFERENCES
• Large necrotic-exudative granulomas 1. Cohen-Gadol AA et al: Spinal cord biopsy: a review of 38
cases. Neurosurgery. 52(4): 806-15; discussion 815-6,2003
Staging, Grading or Classification Criteria 2. Samandouras G et al: Schistosoma haematobium
• CSF bilharzia enzyme-linked immunosorbent assay presenting as an intrinsic conus tumour. Br] Neurosurg.
(ELISA) test 16(3): 296-300, 2002
o Antibody testing cannot distinguish active infection 3. Olson S et al: Spinal schistosomiasis. ] Clin Neurosci. 9(3):
from inactive infection 317-20,2002
• Urine, stool analysis 4. ]unker] et al: Cervical intramedullar schistosomiasis as a
rare cause of acute tetraparesis. Clin Neurol Neurosurg.
• Colon biopsy
103(1): 39-42, 2001
5. Leite CC et al: Clinics in diagnostic imaging (52). Spinal
cord schistosomiasis. Singapore Med]. 41(8): 417-9, 2000
ICLINICAL ISSUES 6. Mazyad MA et al: Spinal cord schistosomiasis and
neurologic complications.] Egypt Soc Parasitol. 29(1):
Presentation 179-82, 1999
• Most common signs/symptoms 7. Bennett G et al: Schistosomal myelitis: findings at MR
o CNS involvement frequently asymptomatic imaging. Eur] Radiol. 27(3): 268-70, 1998
o Fever, malaise, arthralgias, diarrhea 8. Murphy K] et al: Spinal cord infection: myelitis and abscess
o 3 Clinical patterns of cord involvement formation. A]NR Am] Neuroradiol. 19(2): 341-8, 1998
9. Pittella]E: Neuroschistosomiasis. Brain Pathol. 7(1):
• Myelitic 649-62, 1997
• Granulomatous/pseudotumoral 10. Ueki K et al: Schistosoma mansoni infection involVing the
• Radicular/myeloradicular spinal cord. Case report.] Neurosurg. 82(6): 1065-7, 1995
o Spine involvement generally as transverse myelitis 11. Haribhai HC et al: Spinal cord schistosomiasis. A clinical,
pattern, or spinal cord infarction laboratory and radiological study, with a note on
• Transverse myelitis therapeutic aspects. Brain. 114 (Pt 2): 709-26, 1991
• Tetraparesis, paraparesis 12. Bloom K et al: Paraplegia from schistosomiasis. Paraplegia.
28(7): 455-9, 1990
• Anterior spinal artery occlusion
13. Bac 0] et al: Schistosomiasis in ectopic or unusual sites. A
• Radiculopathy report of 5 cases. S Afr Med]. 72(10): 717-8, 1987
o Other signs/symptoms 14. Scrimgeour EM et al: Involvement of the central nervous
• Bowel, bladder dysfunction system in Schistosoma mansoni and S. haematobium
• Clinical profile infection. Brain. 108 (Pt 4):1023-38, 1985
o CSF pleocytosis, elevated protein content, 15. Queiroz LS et al: Massive spinal cord necrosis in
oligoclonal IgG bands schistosomiasis. Arch Neurol. 36(8): 517-9, 1979
o Stool smear examination with quantitative 16. Siddom ]A: Schistosomiasis and anterior spinal artery
occlusion. Am] Trop Med Hyg. 27(3): 532-4, 1978
Kato-Katz oogram if suspected schistosomiasis
17. Norfray]F et al: Schistosomiasis of the spinal cord. Surg
Demographics Neurol. 9(1): 68-71, 1978
18. Lechtenberg R et al: Schistosomiasis of the spinal cord.
• Age: Any age (peak in 2nd decade) Neurology. 27(1): 55-9, 1977
• Gender: M > F
• Ethnicity: No ethnic predisposition, infection related
to location of endemic schistosomiasis
Natural History & Prognosis
• Most patients improve with appropriate antihelmintic
drug treatment
• Acute schistosomiasis up to 25% mortality
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SCHISTOSOMIASIS
IIMAGE GALLERY 1
57
(Left) Sagittal Tl C+ MR
shows patchy enhancement
of distal thoracic cord &
conus due to schistosomiasis
(Courtesy R. Mendonca,
MO). (Right) Sagittal TlWI
MR shows mild diffuse
cord/conus enlargement
with schistosomiasis
infection (Courtesy A. Aul,
MO).
(Left) Sagittal Tl C+ MR
shows patchy conus
enhancement due to
schistosomiasis infection.
Ovoid intradural lesion
below conus presumed
granuloma (Courtesy A. Au/,
MOJ. (Right) Sagittal Tl C+
MR shows shows ovoid
intradural lesion below
conus level presumed to be
schistosomiasis
granulomatous inflammatory
mass (Courtesy A. Aul, MOJ.
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CYSTICERCOSIS
1
58
Axial graphic shows multiple subarachnoid cysts Sagittal T1 C+ MR of cervical spine shows complex
(cysticerci) distorting nerve roots, with multiple multicysticisolid mass with cord enhancement in this
additional lesion within muscles. patient with intramedullary cysticercosis (Courtesy R.
Mendonca, MOJ.
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CYSTICERCOSIS
Key Facts
1
Terminology Pathology 59
• CNS parasitic infection caused by pork tapeworm, • Cysticercosis most common worldwide parasitic
Taenia solium infection
• Classified as extraspinal (vertebral body) or • CNS infection in 60-90% of cases
intraspinal (extradural, subarachnoid, intramedullary) • Thickened, opaque arachnoid, pachymeningitis
• Subarachnoid or intramedullary cysts with cleavage
Top Differential Diagnoses plane from normal tissue; surrounding soft yellowish
• Pyogenic abscess granulation tissue
• Granulomatous disease
• Neoplasm Clinical Issues
• Arachnoid cyst • Spastic paraparesis, quadriparesis, flaccid paralysis
• Other parasitic infections • Sensory level
• Syrinx • Bladder dysfunction
• Schwannoma • Enzyme-linked immunoelectrotransfer blot (EITB)
reportedly> 90% sensitivity
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CYSTICERCOSIS
1 • Epidemiology
o Cysticercosis most common worldwide parasitic
o Steroids to decrease inflammation
o Oral treatment with praziquantel or albendazole
60 infection . o Postoperative course of anticysticercal necessary
o CNS infection in 60-90% of cases since generalized disease with focal spinal
o Endemic in Mexico, Central & South America, manifestation
eastern Europe, Asia, India, Africa
Gross Pathologic & Surgical Features IDIAGNOSTIC CHECKLIST
• Thickened, opaque arachnoid, pachymeningitis
• Subarachnoid or intramedullary cysts with cleavage Consider
plane from normal tissue; surrounding soft yellowish • Spinal cysticercosis often accompanied by intracranial
granulation tissue disease
Microscopic Features Image Interpretation Pearls
• Cyst wall has 3 layers: Outer (cuticular layer), middle • Only spinal findings may be chronic inflammatory
cellular (pseudoepithelial) layer, inner reticular arachnoiditis ~ altered CSF flow dynamics ~
(fibrillary) layer syringomyelia
• Inflammatory reaction with edema, adjacent gliosis
• Cyst wall degeneration with calcification
• Adjacent acute, chronic inflammatory cell infiltrate ISELECTED REFERENCES
giving "cysticercal abscess", arachnoiditis
1. Parmar H et al: MR imaging in intramedullary cysticercosis.
Neuroradiology. 43(11): 961-7, 2001
2. Del Brutto OH et al: Proposed diagnostic criteria for
ICLINICAL ISSUES neurocysticercosis. Neurology. 57(2): 177-83,2001
3. Mathuriya SN et al: Intramedullary cysticercosis: MRI
Presentation diagnosis. Neurol India. 49(1): 71-4,2001
• Most common signs/symptoms 4. Rahalkar MD et al: The many faces of cysticercosis. Clin
o Commonly asymptomatic Radiol. 55(9): 668-74, 2000
o Spine symptoms 5. Zee CS et al: Imaging of neurocysticercosis. Neuroimaging
CUn N Am. 10(2): 391-407, 2000
• Spastic paraparesis, quadriparesis, flaccid paralysis
6. Garg RK: Neurocysticercosis. Postgrad Med]. 74(872):
• Sensory level 321-6, 1998
• Bladder dysfunction 7. Sawhney 1M et al: Uncommon presentations of
• Neck, low back pain neurocysticercosis. J Neurol Sci. 154(1): 94-100, 1998
o Other signs/symptoms: Brain involvement 8. Leite CC et al: MR imaging of intramedullary and
• Seizure, headache, hydrocephalus, focal intradural-extramedullary spinal cysticercosis. AJR Am J
neurologic deficit, stroke Roentgenol. 169(6): 1713-7, 1997
• Clinical profile 9. Mohanty A et al: Spinal intramedullary cysticercosis.
o Diagnosed confirmed by ELISA of serum or CSF Neurosurgery. 40(1): 82-7, 1997
10. Corral I et al: Intramedullary cysticercosis cured with drug
• > 70% sensitivity, highly specific for cysticercal treatment. A case report. Spine. 21(19): 2284-7, 1996
antigens; sensitivity 1 if assay performed on CSF 11. Creasy JL et al: Magnetic resonance imaging of
• Increased sensitivity as number of lesion increase, neurocysticercosis. Top Magn Reson Imaging. 6(1): 59-68,
if assay performed on CSF 1994
• 2/3 spinal NCC reported with negative ELISA 12. Isidro-Llorens A et al: Spinal cysticercosis. Case report and
• Enzyme-linked immunoelectrotransfer blot (EITB) review. Paraplegia. 31(2): 128-30, 1993
reportedly> 90% sensitivity 13. Zee CS et a1: CT myelography in spinal cysticercosis. J
o Peripheral blood eosinophilia 25% Comput Assist Tomogr. 10(2): 195-8, 1986
14. Akiguchi I et al: Intramedullary spinal cysticercosis.
o CSF: Nonspecific protein elevation, lymphocytosis
Neurology. 29(11): 1531-4, 1979
Demographics 15. Firemark HM: Spinal cysticercosis. Arch Neurol. 35(4):
250-1, 1978
• Age: Any age 16. Kurrein F et al: Cysticercosis of the spine. Ann Trop Med
• Gender: M = F Parasitol. 71(2): 213-7, 1977
• Ethnicity: In US, Hispanic & Asian patients common
~ endemic area immigration
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CYSTICERCOSIS
IIMAGE GALLERY 1
61
Typical
(Left) Sagittal T7 C+ MR of
cervical spine shows
complex intramedullary
multicystic mass with patchy
peripheral enhancement in
cysticercosis (Courtesy R.
Mendonca, MO). (Right)
Sagittal T2WI MR of cervical
spine shows heterogeneous
T2 signal from expanded
upper cervical cord in
intramedullary cysticercosis
(Courtesy R. Mendonca,
MO).
Typical
(Left) Sagittal T2WI MR
shows well-defined T2
hyperintense cyst in thoracic
cord with mild focal cord
expansion but no adjacent
edema in this patient with
intramedullary cysticercosis
(Courtesy H. Shah, MO).
(Right) Axial T7 C+ MR
shows focal, well-defined
nonenhancing cyst in
thoracic cord with mild focal
cord expansion in this
patient with intramedullary
cysticercosis (Courtesy H.
Shah, MO).
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GUILLAIN-BARRE SYNDROME
2
2
Axial T1 C+ MR in patient with ascending paralysis Sagittal T1 C+ MR (with fat-saturation) of an 8 year old
demonstrates preferential enhancement of ventral shows diffuse pial enhancement of conus, and
cauda equina (arrows). thickened, enhancing cauda equina roots. Note normal
conus size.
(t'.'
.~/"~~~
~ ClOP Meningitis Blood Induced Metastases
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GUILLAIN-BARRE SYNDROME
Key Facts
Terminology • Physiological nerve root enhancement
• Autoimmune post-infectious or post-vaccinial acute Pathology
inflammatory demyelination of peripheral nerves, • Inflammatory (postulated autoimmune or viral)
nerve roots, cranial nerves demyelination
Imaging Findings • Usually follows recent viral illness 2
• Avid enhancement of cauda equina Clinical Issues
• Roots may be slightly thickened, not nodular 3
• Acute flaccid paralysis or
• Axial images show preferential contrast accentuation • Distal paraesthesia followed by rapid ascending
of ventral roots in cauda paralysis
Top Differential Diagnoses • Most patients somewhat better by 2-3 months
• Miller-Fischer syndrome (MFS) • Mortality up to 8%
• Chronic polyneuropathies Diagnostic Checklist
• Vasculitic neuropathy • Clinical setting of nerve root enhancement in cauda
• Carcinomatous or lymphomatous meningitis critical in differential diagnosis
• Chemical or post-surgical arachnoiditis
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GUILLAIN-BARRE SYNDROME
Staging, Grading or Classification Criteria I DIAGNOSTIC CHECKLIST
• Spectrum of disease extends to subacute inflammatory
demyelinating polyradiculoneuropathy (SIDP)
Consider
• SIDP a bridge to chronic inflammatory demyelinating • Clinical setting of nerve root enhancement in cauda
polyradiculopathy (CIDP) critical in differential diagnosis
o Both entities exhibit slower onset, protracted course, Image Interpretation Pearls
2 exacerbations, lesser cranial nerve involvement • Anterior cauda nerve root and pial conus
enhancement without significant enlargement or
4 nodularity is strong clue
ICLINICAL ISSUES
Presentation
I SELECTED REFERENCES
• Most common signs/symptoms
o Classically presents with "ascending paralysis" 1. Cheng BC et al: Guillain-Barre syndrome in southern
o Ascent up to brainstem may involve cranial nerves Taiwan: clinical features, prognostic factors and
therapeutic outcomes. Eur] Neurol. 10(6):655-62, 2003
• Respiratory paralysis requiring ventilator in severe
2. Chang YW et al: Spinal MRI of vincristine neuropathy
cases mimicking Guillain-Barre syndrome. Pediatr Radiol.
o Other signs/symptoms 33(11):791-3, 2003
• Sensory loss common but less severe 3. Molinero MR et al: Epidemiology of childhood
• Numbness tingling Guillain-Barre syndrome as a cause of acute flaccid
• Distal paraesthesias rapidly followed by "ascending paralysis in Honduras: 1989-1999.] Child Neurol.
paralysis" 18(11):741-7,2003
o Frequently bilateral and symmetric 4. Oh S] et al: Subacute inflammatory demyelinating
o May require prolonged respiratory support in severe polyneuropathy. Neurology. 61(11):1507-12, 2003
5. Haber P et al: Influenza vaccination and Guillain Barre
cases syndrome. Clin Immunol. 109(3):359; author reply 360-1,
• Autonomic disturbances 2003
• Cranial nerve involvement common 6. Wilmshurst]M et al: Lower limb and back pain in
o Facial nerve involved in up to 50% of cases Guillain-Barre syndrome and associated contrast
o Ophthalmoparesis in 10-20% of cases enhancement in MRI of the cauda equina. Acta Paediatr.
90(6):691-4, 2001
Demographics 7. Kehoe M: Guillain-Barre syndrome--a patient gUide and
• Age: Typically children, young adults nursing resource. Axone. 22(4):16-24, 2001
• Gender: No preference 8. Duarte] et al: Hypertrophy of multiple cranial nerves and
• Ethnicity: All spinal roots in chronic inflammatory demyelinating
neuropathy.] Neurol Neurosurg Psychiatry. 67(5):685-7,
Natural History & Prognosis 1999
9. Berciano]: MR imaging in Guillain-Barre syndrome.
• Acute flaccid paralysis or
Radiology. 211(1):290-1, 1999
• Distal paraesthesia followed by rapid ascending 10. Vroomen PC et al: The clinical significance of gadolinium
paralysis enhancement of lumbar disc herniations and nerve roots
o Most often bilateral, symmetric on preoperative MRI. Neuroradiology. 40(12):800-6, 1998
• Cranial nerve involvement common 11. Byun WM et al: Guillain-Barre syndrome: MR imaging
o Facial nerve in 50% findings of the spine in eight patients. Radiology.
o Ophthalmoparesis in < 10% 208(1):137-41, 1998
• Clinical nadir at 4 weeks 12. Iwata F et al: MR imaging in Guillain-Barre syndrome.
Pediatr Radiol. 27(1):36-8, 1997
• Most patients somewhat better by 2-3 months 13. Gorson KC et al: Prospective evaluation of MRI
o 30-50% have persistent symptoms at 1 year lumbosacral nerve root enhancement in acute
o Permanent deficits in 5-10% Guillain-Barre syndrome. Neurology. 47(3):813-7, 1996
• 2-10% relapse 14. Perry]R: MRI in Guillain-Barre syndrome. Neurology.
o 6% develop chronic course resembling CIDP 45(5):1024-5, 1995
o Earliest stages of SIDP, CIDP may be 15. Bertorini T et al: Contrast-enhanced magnetic resonance
indistinguishable clinically from Guillain-Barre imaging of the lumbosacral roots in the dysimmune
• Mortality up to 8% inflammatory polyneuropathies. ] Neuroimaging.
5(1):9-15, 1995
Treatment 16. Georgy BA et al: MR of the spine in Guillain-Barre
• Medical management with plasma exchange or syndrome. A]NR Am] Neuroradiol. 15(2):300-1, 1994
17. Crino PB et al: Magnetic resonance imaging of the cauda
intravenous gamma globulin equina in Guillain-Barre syndrome. Neurology 44(7):
• No proven benefit from corticosteroid administration 1334-6, 1994
• Intensive care management in severe cases 18. Patel H et al: MRI of Guillain-Barre syndrome. ] Comput
Assist Tomogr. 17(4):651-2, 1993
19. ]inkins]R et al: MRI of benign lumbosacral nerve root
enhancement. Semin Ultrasound CT MR. 14(6):446-54,
1993
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GUILLAIN-BARRE SYNDROME
IIMAGE GALLERY
Typical
(Left) Sagittal T7 C+ MR in
32 year old woman
recuperating from ascending
lower extremity paralysis
2
shows enhancement of
conus pial surface, and 5
thickened, enhancing cauda
equina. (Right) Axial T7 C+
MR in the same case shows
enhancement and slight
thickening of ventral cauda
equina roots.
(Left) Coronal T7 C+ MR
with fat-saturation shows
enhancement of spinal
nerves and lumbar plexus
roots in child with CBS
(Courtesy S. Blaser, MO).
(Right) Sagittal T7 C+ MR
shows striking enhancement
of cauda equina and surface
of conus simulating
carcinomatous meningitis in
patient with CBS (Courtesy
S. Blaser, MO).
Variant
(Left) Sagittal T7 C+ MR off
midline shows enhancing
cervical roots in child with
CBS (Courtesy S. Blaser,
MO). (Right) Axial T7 C+
MR in patient with CBS
demonstrates preferential
enhancement of dorsal
roots-an atypical finding
(Courtesy S. Blaser, MO).
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ClOP
2
6
Axial T2WI MR shows marked enlargement of the Sagittal T7 C+ MR demonstrates diffuse pial thickening
lumbar/sacral nerve roots (black arrows) and and enhancement extending into the cauda equina
lumbosacral trunk (arrows). nerve roots. Clinical course distinguished from
Cuillain-Barre (AIDP).
ITERMINOlOGY CT Findings
• NECT: Isodense nerve enlargement
Abbreviations and Synonyms • CECT: Mild to moderate nerve enhancement
• Chronic inflammatory demyelinating polyneuropathy
(CIPD), chronic inflammatory demyelinating MR Findings
polyradiculoneuropathy • TIWI: Isointense enlargement of cauda equina and
proximal peripheral nerves
Definitions • T2WI: Enlargement, abnormal hyperintensity of
• Chronic acquired, immune-mediated demyelinating intradural and extradural spinal nerves/branches
neuropathy characterized by relapsing or progressive
• FLAIR
muscle weakness +/- sensory loss o No utility for spine imaging
o Sagittal FLAIR may reveal hyperintense brain lesions
similar to multiple sclerosis (MS)
IIMAGING FINDINGS • Tl C+: Mild to moderate nerve enhancement
General Features Ultrasonographic Findings
• Best diagnostic clue: Enlargement and abnormal T2 • Real Time: Hypoechoic, hypertrophic nerves
hyperintensity of nerve roots, plexi, or peripheral
nerves Imaging Recommendations
• Location • T2WI, enhanced coronal and axial TlWI sequences
o Spinal nerve roots and peripheral nerves with fat suppression best delineate nerve lesions
(extraforaminal > intradural) • Brain MRI to detect subclinical CNS demyelination
o Lumbar> cervical, brachial plexus,
thoracic/intercostal> cranial nerve
• Size: Nerve size varies small - very large IDIFFERENTIAL DIAGNOSIS
• Morphology: Focal or diffuse fusiform enlargement of
cauda equina, nerve roots/plexi, and peripheral nerves Guillain-Barre (AIDP)
• Pial, nerve root enhancement similar to CIDP
. ....' .....
..~- .. _ 1110..,
...,..
.
- . .
Guil/an-Barre (AID?)
~Charcot-Marie-Tooth Neurofibromatosis 7 Lateral Meningocele
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ClOP
Key Facts
Terminology • Inherited demyelinating neuropathy
• Chronic inflammatory demyelinating • Neurofibromatosis type 1
polyneuropathy (CIPD), chronic inflammatory • Lateral meningocele
demyelinating polyradiculoneuropathy Pathology
• Chronic acquired, immune-mediated demyelinating
neuropathy characterized by relapsing or progressive
• Hallmarks of CIDP: Enlarged nerves with "onion
bulb" formations, demyelination
2
muscle weakness +/- sensory loss
7
Clinical Issues
Imaging Findings • Mixed sensorimotor neuropathy
• Best diagnostic clue: Enlargement and abnormal T2 • Symmetric proximal and distal weakness, sensory loss
hyperintensity of nerve roots, plexi, or peripheral • Clinical course: Chronic progressive, step-wise
nerves progressive, or recurrent behavior
• Sagittal FLAIR may reveal hyperintense brain lesions
similar to multiple sclerosis (MS) Diagnostic Checklist
• Consider CIDP in differential of nerve root/peripheral
Top Differential Diagnoses nerve enlargement
• Guillain-Barre (AIDP)
• Distinguish from CIDP by onset duration, clinical • Connective tissue disease, diabetes mellitus, IgG
course or IgA monoclonal gammopathy, hepatitis C
o Acute onset of ascending paralysis with relative infection, Sjogren syndrome, inflammatory bowel
sensory preservation disease, human immunodeficiency virus (HIV),
and lymphoma
Inherited demyelinating neuropathy • Although precise relationship of secondary
• Charcot-Marie-Tooth, Dejerine-Sottas neuropathies diseases to CIDP is unclear, neuropathy frequently
• Genetic testing, clinical phenotype help distinguish responds to therapy-like idiopathic CIDP
from CIDP • Epidemiology: Prevalence 2-7.7/100,000 U.S.
Neurofibromatosis type 1 population
• Associated abnormalities: +/- Concurrent CNS
• Diffuse nerve root enlargement, enhancement
demyelination
• Genetic testing, distinctive clinical stigmata
distinguish from CIDP Gross Pathologic & Surgical Features
lateral meningocele • Extensive fusiform nerve enlargement +/- gross onion
bulb formations
• CSF density/signal intensity (not solid) +/- foraminal
enlargement, dural ectasia Microscopic Features
• Usually co-existing NFl or connective tissue disorder • Hallmarks of CIDP: Enlarged nerves with "onion bulb"
(Marfan syndrome) formations, demyelination
o Macrophage, T cell infiltration ~ perivascular
inflammatory infiltrates, nerve demyelination and
I PATHOLOGY remyelination
o "Onion bulb" formation 2° to excessive Schwann cell
General Features
process proliferation ~ segmental repetitive
• General path comments demyelination/remyelination
o Acquired, immune-mediated multifocal
demyelinating neuropathy affecting primarily spinal
nerves, plexi, and proximal nerve trunks ICLINICAL ISSUES
• Putative pathogenic mechanisms broadly resemble
MS; considered by some to be peripheral Presentation
counterpart of MS • Most common signs/symptoms
• Etiology o Mixed sensorimotor neuropathy
o Exact pathogenesis of CIDP unclear; involves both o Symmetric proximal and distal weakness, sensory
cellular and humoral immune factors loss
• Chemokines released into CSF from inflamed o Other signs/symptoms
spinal nerve roots ~ attract T cells, monocytes, • Occasional pure motor neuropathy
and macrophages to PNS and precipitate release of • Symptomatic lumbar stenosis 2° to nerve root
proinflammatory enzymes hypertrophy
• Activated CD4 T cells, antimyelin • Clinical profile
antibodies/complement cross-react with myelin o CIDP is usually a clinical diagnosis based on
and PNS antigens presence of progressive weakness/sensory loss and
o Idiopathic or secondary (CIDP with concurrent response to corticosteroid therapy
disease)
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ClOP
o +/- Concurrent CNS demyelination (frequently Guillain-Barre syndrome. J Neurol. 250(8):913-6, 2003
subclinical) 8. Fee DB et al: Resolution of chronic inflammatory
o Laboratory abnormalities demyelinating polyneuropathy-associated central nervous
system lesions after treatment with intravenous
• Abnormal EMG/NCV: Keyelectrophysiologic
immunoglobulin. J Peripher Nerv Syst. 8(3):155-8, 2003
features =} nerve conduction block, slowed 9. Cocito D et al: Different clinical, electrophysiological and
conduction velocities suggestive of demyelination immunological features of ClOP associated with
• 1 CSF protein paraproteinaemia. Acta Neurol Scand. 108(4):274-80, 2003
2 o Diagnosis relies primarily on clinical, 10. Oguz B et al: Diffuse spinal and intercostal nerve
electrophysiologic examination supplemented by involvement in chronic inflammatory demyelinating
8 nerve biopsy polyradiculoneuropathy: MRI findings. Eur Radiol. 13
o ~ 50% of CIDP patients have atypical features; Suppl 4:L230-4, 2003
11. Magda P et al: Comparison of electrodiagnostic
asymmetry, distal weakness, sensory loss only
abnormalities and criteria in a cohort of patients with
Demographics chronic inflammatory demyelinating polyneuropathy.
Arch Neurol. 60(12):1755-9, 2003
• Age: Adult> childhood 12. Costello F et al: Childhood-onset chronic inflammatory
• Gender: M = F demyelinating polyradiculoneuropathy with cranial nerve
involvement. J Child Neurol. 17(11):819-23,2002
Natural History & Prognosis 13. Saperstein DS et al: Clinical spectrum of chronic acquired
• Clinical course: Chronic progressive, step-wise demyelinating polyneuropathies. Muscle Nerve.
progressive, or recurrent behavior 24(3):311-24,2001
o Mildly affected patients tend to recover; severely 14. Cros D: Peripheral Neuropathy. First ed. Philadelphia:
affected patients more likely to have chronic Lippincott Williams & Wilkins: 432, 2001
symptoms or mortality related to CIDP 15. Sabatelli M et al: Pure motor chronic inflammatory
o Prolonged remission/cure is exception, not rule demyelinating polyneuropathy. J Neurol. 248(9):772-7,
2001
o Atypical and secondary CIDP respond similarly to
16. Taniguchi N et al: Sonographic detection of diffuse
classic CIDP peripheral nerve hypertrophy' in chronic inflammatory
• Average disease duration = 7.5 years demyelinating polyradiculoneuropathy. J Clin Ultrasound.
28(9):488-91, 2000
Treatment 17. Van den Bergh PY et al: Chronic demyelinating
• Immunomodulation or immunosuppression therapy hypertrophic brachial plexus neuropathy. Muscle nerve
o Prednisolone therapy, plasmapheresis, or 23(2): 283-8, 2000
intravenous immunoglobulin (IVIG) 18. Duarte J et al: Hypertrophy of multiple cranial nerves and
spinal roots in chronic inflammatory demyelinating
neuropathy. J Neurol Neurosurg Psychiatry. 67(5):685-7,
I DIAGNOSTIC CHECKLIST 19.
1999
Midroni G et al: MRI of the cauda equina in ClOP: clinical
Consider correlations. J Neurol Sci. 170(1):36-44, 1999
20. Mizuno K et al: Chronic inflammatory demyelinating
• Consider CIDP in differential of nerve root/peripheral polyradiculoneuropathy with diffuse and massive
nerve enlargement peripheral nerve hypertrophy: distinctive clinical and
magnetic resonance imaging features. Muscle Nerve.
Image Interpretation Pearls 21(6):805-8, 1998
• MRI findings imperfectly correlate with clinical disease 21. Kuwabara S et al: Magnetic resonance imaging at the
activity/severity, laboratory findings demyelinative foci in chronic inflammatory demyelinating
polyneuropathy. Neurology. 48(4):874-7, 1997
22. Simmons Z et al: Chronic inflammatory demyelinating
ISELECTED REFERENCES polyradiculoneuropathy in children: I. Presentation,
electrodiagnostic studies, and initial clinical course, with
1. Saperstein DS et al: Current concepts and controversy in comparison to adults. 20(12):1569-75, 1997
chronic inflammatory demyelinating polyneuropathy. 23. Van Es HW et al: Magnetic resonance imaging of the
Curr Neurol Neurosci Rep. 3(1):57-63, 2003 brachial plexus in patients with multifocal motor
2. Rodriguez-Casero MV et al: Childhood chronic neuropathy. Neurology. 48(5):1218-24, 1997
inflammatory demyelinating polyneuropathy with central 24. Goldstein JM et al: Nerve root hypertrophy as the cause of
nervous system demyelination resembling multiple lumbar stenosis in chronic inflammatory demyelinating
sclerosis. Neuromuscul Disord. 13(2):158-61, 2003 polyradiculoneuropathy. Muscle Nerve. 19(7):892-6, 1996
3. Ropper AH: Current treatments for ClOP. Neurology. 60(8 25. Schady W et al: Massive nerve root enlargement in chronic
Suppl 3):S16-22, 2003 inflammatory demyelinating polyneuropathy. J Neurol
4. Toyka KV et al: The pathogenesis of ClOP: rationale for Neurosurg Psychiatry. 61(6):636-40, 1996
treatment with immunomodulatory agents. Neurology.
60(8 Suppl 3):52-7, 2003
5. Haq RU et al: Chronic inflammatory demyelinating
polyradiculoneuropathy in diabetic patients. Muscle Nerve.
27(4):465-70,2003
6. Press R et al: Aberrated levels of cerebrospinal fluid
chemokines in Guillain-Barre syndrome and chronic
inflammatory demyelinating polyradiculoneuropathy. J
Clin Immunol. 23(4):259-67, 2003
7. Odaka M et al: Patients with chronic inflammatory
demyelinating polyneuropathy initially diagnosed as
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ClOP
IIMAGE GALLERY
Typical
(Left) Axial T2WI MR reveals
bilateral symmetric
enlargement, hyperintensity 2
of cervical nerve roots and
brachial plexus (arrows).
(Right) Axial T2WI MR 9
shows diffuse thickening and
hyperintensity of thoracic
nerve roots and paraspinal
intercostal nerves.
Typical
(Left) Sagittal T2WI MR
depicts enlarged lumbar
nerve roots extending into
extraforaminal ventral
primary rami (arrows).
(Right) Sagittal FLAIR MR of
the brain in a ClOP patient
shows a typical
paraventricular
demyelinating lesion (arrow)
similar to those seen in
multiple sclerosis patients.
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ARACHNOIDITIS, LUMBAR
2
10
Axial graphic of a lumbar vertebra demonstrates nerve Axial T2WI MR shows centrally and peripherally
roots plastered against the periphery of thecal sac, with clumped nerve roots.
multiple adhesions compartmentalizing arachnoid
space.
CT Findings
IIMAGING FINDINGS • NECT
o Post-myelography
General Features • "Empty thecal sac sign"
• Best diagnostic clue: Absence of discrete nerve roots in • Absent nerve root sleeve filling
thecal sac • Nerve roots adherent to thecal sac
• Location • Clumped nerve roots forming soft tissue mass(es)
o Lumbar spine • Irregular contrast collections
• Cauda equina o Thickened retracted dura
• Morphology o Distorted thecal sac
o Intrathecal cords • CECT: Enhancing nerve roots and dura
o "Empty sac" • Bone CT
o Soft tissue mass o Rare calcifications of nerve roots or soft tissue mass
• Arachnoiditis ossificans
Radiographic Findings
• Radiography: Evidence of prior lumbar surgery MR Findings
• Myelography • TIWI
o Type I o ± Residual Pantopaque (high signal droplets)
• "Featureless sac sign" o Indistinct cord due to increased cerebral spinal fluid
• Homogeneous filling of thecal sac (CSF) intensity
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ARACHNOIDITIS, LUMBAR
Key Facts
Terminology • Minimal to mild cord, nerve root, and dural
• Chronic adhesive arachnoiditis enhancement
• Post-inflammatory adhesion and clumping of nerve • Findings extend over at least two lumber vertebrae
roots Top'Differential Diagnoses
Imaging Findings • Spinal stenosis
• Cauda equina neoplasms
2
• Best diagnostic clue: Absence of discrete nerve roots
in thecal sac • Intradural metastases 11
• Absent or blunted nerve root sleeve filling Pathology
• Flow of myelographic contrast may be obstructed • Now more commonly associated with prior lumbar
• Clumped nerve roots forming cords surgery
• Nerve roots adhering to walls of thecal sac • 6-16% of postoperative patients
• "Empty thecal sac sign"
• Soft tissue mass filling most of thecal sac (type III) Clinical Issues
• Intradural cysts • Chronic low back pain
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ARACHNOIDITIS, LUMBAR
o Myelography alone elicits an intrathecal o 1.8% in one series
inflammatory response
o Intrathecal hemorrhage Treatment
• Shown to irritate meninges in animal models • Intrathecal steroid injection
• Dorsal thoracic arachnoiditis more common in • Spinal cord stimulation
subarachnoid hemorrhage o Treatment of choice when pain is the predominant
o Other causes symptom
2 • Spinal anesthesia o Immediate success rate (pain relief): > 70%
o Intermediate success rate: > 50%
• Degenerative disc disease
12 o Pathophysiology o Long term success rate: > 30%
• Infectious meningitis: Vascular congestion, • Pain rehabilitation
inflammatory exudate • Laminectomy with microlysis of adhesions
• Fibrin deposition o Reserved for patients with progressive neurologic
• Fibrocytes proliferate deficits
• Perineural and leptomeningeal fibrosis o 50% initial success rate
• Nerve roots adhere to one another, thecal sac o Effectiveness decreases over time
• CSF loculation
• Epidemiology
o Uncommon I DIAGNOSTIC CHECKLIST
• < 1,000 cases reported in literature over 50 years
Image Interpretation Pearls
o Clinical syndrome more common
• 6-16% of post-operative patients • Absent discrete nerve roots in thecal sac with
clumping or "empty sac sign" highly suggestive of
• Associated abnormalities
o Syringomyelia lumbar arachnoiditis
• Not lined by ependyma
• Alteration in CSF circulation
• Increased amount of CSF entering cord ISELECTED REFERENCES
• Symptoms higher in cord 1. Ross JS: Magnetic resonance imaging of the postoperative
spine. Semin Musculoskelet Radiol. 4(3):281-91, 2000
Gross Pathologic & Surgical Features 2. Petty PG et al: Symptomatic lumbar spinal arachnoiditis:
• Inflammatory, collagenous mass fact or fallacy? J Clin Neurosci. 7(5):395-9, 2000
• Maybe calcified 3. Long DM: Chronic adhesive spinal arachnoiditis:
• Posterior nerve roots involved more commonly pathogenesis, prognosis, and treatment. Neurosurgery
Quarterly. 2:296-318, 1992
Microscopic Features 4. Sklar EM et al: Complications of epidural anesthesia: MR
appearance of abnormalities. Radiology. 181(2):549-54,
• Collagen formation 1991
• Chronic lymphocytic infiltration 5. Johnson CE et al: Benign lumbar arachnoiditis: MR
• Small foci of calcifications imaging with gadopentetate dimeglumine. AJR Am J
Roentgenol. 155(4):873-80, 1990
6. Delamarter RB et al: Diagnosis of lumbar arachnoiditis by
ICLINICALISSUES magnetic resonance imaging. Spine. 15:304-10, 1990
7. Caplan LR et al: Syringomyelia and arachnoiditis. J Neurol
Presentation Neurosurg Psychiatry. 53(2):106-13, 1990
• Most common signs/symptoms 8. Augustijn P et al: Chronic spinal arachnoiditis following
intracranial subarachnoid haemorrhage. Clin Neurol
o Chronic low back pain
Neurosurg. 91(4):347-50, 1989
o Radicular or non-radicular leg pain 9. Sklar E et al: Acquired spinal subarachnoid cysts:
• Simulates spinal stenosis and polyneuropathy evaluation with MR, CT myelography, and intraoperative
o Other signs/symptoms sonography. AJNR Am.] Neuroradiol. 10(5):1097-104,1989
• Paraparesis 10. Ross JS et al: MR imaging of lumbar arachnoiditis. AjR.
• Hypoesthesia 149:1025-32, 1987
• Gait disorder 11. Burton CV et al: Causes of failure of surgery on the lumbar
• Bladder and bowel dysfunction spine. Clin Orthop. (157):191-9, 1981
12. Quiles M et al: Lumbar adhesive arachnoiditis. Etiologic
• Clinical profile and pathologic aspects. Spine. 3(1):45-50, 1978
o No defining clinical symptomatology 13. Quencer RM et al: The postoperative myelogram.
o Radiological findings may be present without Radiographic evaluation of arachnoiditis and
clinical symptoms dural/arachnoidal tears. Radiology. 123(3):667-79, 1977
14. Jorgensen J et al: A clinical and radiological study of
Demographics chronic lower spinal arachnoiditis. Neuroradiology.
• Gender: No gender predilection 9(3):139-44, 1975
• Ethnicity: No racial predilection
Natural History & Prognosis
• Symptoms usually static
o Fluctuate in severity
• Small percentage with progressive neurologic deficits
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ARACHNOIDITIS, LUMBAR
IIMAGE GALLERY
Typical
(Left) Axial T2WI MR shows
compartmentalized nerve
roots and loculated CSF from
intrathecal adhesions.
Post-surgical changes involve
posterior elements. (Right)
Axial T2WI MR shows
centrally clumped nerve
roots forming a mass.
Posterior thecal sac is
thickened (arrow) from
adherent nerve roots.
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ARACHNOIDITIS OSSIFICANS, LUMBAR
2
14
Axial bone CT with sagittal reformation shows two Sagittal T1WI MR shows a conglomerate of lumbar
intraspinal thin linear calcifications at L5-S 7. nerve roots consistent with arachnoiditis. A subtle linear
intrathecal hypointensity (arrow) corresponds to a
calcification on CT
ITERMINOLOGY MR Findings
• Clumped nerve roots forming cords and mass(es)
Definitions • Focal signal alteration distinct from nerve root
• Intradural ossification associated with conglomerate
post-inflammatory adhesion and clumping of lumbar o Variable signal intensity
nerve roots • Hypo- or hyperintense on T1 WI and T2WI
o Hyperintensity represents fatty marrow
o May exert mass effect on conus and cauda equina
IIMAGING FINDINGS o No post-gadolinium enhancement
General Features • Variable nerve root enhancement
• Intraspinal cysts and loculations
• Best diagnostic clue: Focal calcific density on CT or
hyperintensity on T1WI and T2WI within lumbar Imaging Recommendations
nerve root aggregate • Best imaging tool: Axial thin-seCtion bone CT with
• Location: Lumbar spine sagittal reformation
• Morphology
o Thin, linear
o Mass-like, globular I DIFFERENTIAL DIAGNOSIS
Radiographic Findings Retained Pantopaque
• Radiography: Intraspinal ossification • Hyperintense on TlWI
• Myelography: Intrathecal contrast may obscure • Iso- to hypointense on T2Wl
calcifications • Differentiated from calcification by CT
CT Findings Intrathecal primary neoplasm, metastases
• Bone CT • More vigorous enhancement of mass, nerve roots, or
o Calcific densities within clumped nerve roots focal nodules
o Calcifications may surround conus medullaris,
cauda equina
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ARACHNOIDITIS OSSIFICANS, LUMBAR
Key Facts
Terminology • Hypo- or hyperintense on TlWI and T2WI
• Intradural ossification associated with • No post-gadolinium enhancement
post-inflammatory adhesion and clumping of lumbar Pathology
nerve roots • Osseous metaplasia from chronic inflammation and
Imaging Findings fibrosis 2
• Best diagnostic clue: Focal calcific density on CT or Diagnostic Checklist
hyperintensity on T1 WI and T2WI within lumbar 15
• Thin-section bone CT when heterogeneous
nerve root aggregate intrathecal signal present in setting of arachnoiditis
• Mass or nodules isointense on TlWI and hyperintense • Simulates spinal stenosis and polyneuropathy
on T2WI
Natural History & Prognosis
Spinal stenosis • Possible progressive neurologic impairment
• Clumped nerve roots
• Degenerative changes prominent Treatment
• No intrathecal calcification • Decompressive laminectomy over entire length of
ossification with some reported success
Calcified dural plaques • No therapeutic advantage in resecting intradural
• Thin, patchy dural calcifications ossifications
• Asymptomatic
IDIAGNOSTIC CHECKLIST
[PATHOLOGY
Consider
General Features • Thin-section bone CT when heterogeneous intrathecal
• Etiology signal present in setting of arachnoiditis
o Prior trauma, lumbar surgery, subarachnoid
hemorrhage, myelography, spinal anesthesia
o Pathophysiology of lumbar arachnoiditis ISELECTED REFERENCES
• Fibrin deposition 1. Faure A et al: Arachnoiditis ossificans of the cauda equina.
• Proliferating fibroblasts Case report and review of the literature. J Neurosurg. 97(2
• Perineural and leptomeningeal fibrosis Suppl):239-43, 2002
o Ossification results from 2. Frizzell B et al: Arachnoiditis ossificans: MR imaging
• Intrathecal hematoma features in five patients. AJR 177:461-4, 2001
• Incorporated bone fragments 3. Long OM: Chronic adhesive spinal arachnoiditis:
• Osseous metaplasia from chronic inflammation pathogenesis, prognosis, and treatment. Neurosurgery
Quarterly. 2:296-318, 1992
and fibrosis
• End-stage chronic arachnoiditis
• Epidemiology: Rare
• Associated abnormalities: Syringomyelia I IMAGE GALLERY
Gross Pathologic & Surgical Features
• Calcified inflammatory, collagenous mass
Microscopic Features
• Osteoblastic proliferation with osseous metaplasia
• Mature bone with osseous marrow and trabeculae
I CLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Low back pain (Left) Axial T2WI MR confirms the focal intrathecal hypointensity
o Other signs/symptoms (arrow) at dorsal aspect of clumped nerve roots. (Right) Axial
• Radicular or non-radicular leg pain PO/Intermediate MR shows the same focal hypointensity (arrow),
• Paraparesis corresponding to calcification on CT.
• Bladder and bowel dysfunction
• Clinical profile
o No defining clinical symptomatology
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SARCOIDOSIS
2
16
Sagittal graphic depicts muluple intramedullary sarcoid Sagittal T2WI MR (left) shows diffuse 1M T2
granulomas in brainstem and lIpper cervical cord. hyperintensities with nodular isointensities. Sagittal T7
C+ MR (right) shows nodular and mass-like
enhancement (Courtesy A. Osborn, MO).
• Morphology: Variable
ITERMINOLOGY
Radiographic Findings
Abbreviations and Synonyms • Myelography: Nonspecific cord expansion
• Neurosarcoidosis
CT Findings
Definitions • NECT: Cord expansion
• Noncaseating granulomatous disease of spine and • Bone CT
spinal cord o Multiple lytic lesions in spine
• Sclerotic margins
o Sclerotic or mixed lytic and sclerotic lesions also
IIMAGING FINDINGS possible
General Features MR Findings
• Best diagnostic clue: Combination of leptomeningeal • TIWI
and peripheral intramedullary (1M) mass-like o Fusiform cord enlargement
enhancement suggestive of spinal sarcoidosis o Cord atrophy in late stages
• Location o Iso- to hypointense lesions
o Intramedullary
• T2WI
• Cervical o Hyperintensity
• Upper thoracic • Focal or diffuse
o Extramedullary intradural • STIR: Lesions hyperintense
• Cauda equina • Tl C+
• Dural involvement without segmental o Enhancing dural masses
predilection • Variable cord and nerve root compression
o Extradural o Leptomeningeal enhancement
o Vertebral • Smooth or nodular
• Lower thoracic and upper lumbar spine • 60% of cord lesions
• Size: Variable • Including cauda equina
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SARCOIDOSIS
Key Facts
Terminology Top Differential Diagnoses
• Noncaseating granulomatous disease of spine and • Intramedullary neoplasm
spinal cord • Multiple sclerosis
Imaging Findings Pathology
• Best diagnostic clue: Combination of leptomeningeal • General path comments: Noncaseating 2
and peripheral intramedullary (1M) mass-like granulomatous inflammation
enhancement suggestive of spinal sarcoidosis • Clinical CNS involvement in patients with 17
• Multiple lytic lesions in spine sarcoidosis: 5%
• Fusiform cord enlargement • Intraoperative frozen-sections may be misinterpreted
• Cord atrophy in late stages for gliomas
• Hyperintensity
• Enhancing dural masses Diagnostic Checklist
• Leptomeningeal enhancement • Protean imaging manifestations
• Peripheral 1M enhancement • Mimicking multiple spinal pathologies
• Mass-like 1M enhancement • Invariable presence of systemic disease helps make
the diagnosis and avoid spinal cord biopsy
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SARCOIDOSIS
o Imaging evidence of CNS involvement: 10% o Azathioprine
o CNS involvement at autopsy: 15-25% o Methotrexate
o Isolated neurosarcoidosis: 1.5% o Cyclosporine
• Associated abnormalities • Low dose radiation
o Intracranial sarcoidosis o 1-3,000 rads over days to weeks
o Systemic sarcoidosis
• Pathophysiology of 1M involvement
2 o Leptomeningeal granulomatous inflammation IDIAGNOSTIC CHECKLIST
o Central intramedullary spread via perivascular space
18 • Cord enlargement and faint enhancement Image Interpretation Pearls
o Focal or multifocal enhancement • Protean imaging manifestations
• Normal cord contour o Mimicking multiple spinal pathologies
o Resolved inflammation o Invariable presence of systemic disease helps make
• No enhancement the diagnosis and avoid spinal cord biopsy
• Normal or atrophic cord
Gross Pathologic & Surgical Features ISELECTED REFERENCES
• Granuloma resembles neoplasm
1. Smith JK et al: Imaging manifestations of neurosarcoidosis.
• Intraoperative frozen-sections may be misinterpreted AJR Am J Roentgenol. 182(2):289-95, 2004
for gliomas 2. Bose B: Extramedullary sarcoid lesion mimicking
intraspinal tumor. Spine J. 2(5):381-5, 2002
Microscopic Features 3. Dubey N et al: Role of fluorodeoxyglucose positron
• Epithelioid cell noncaseating granulomas emission tomography in the diagnosis of neurosarcoidosis.
o Surrounded by lymphocytes and mononuclear J Neurol Sci. 205(1):77-81, 2002
phagocytes 4. Gullapalli D et al: Neurologic manifestations of sarcoidosis.
o Negative stains for infectious agents Neurol Clin. 20(1):59-83, vi, 2002
o Granulomas smaller compared to those in systemic 5. Bode MK et al: Isolated neurosarcoidosis - MR findings and
disease pathologic correlation. Acta Radiol. 42(6):563-7, 2001
6. Vinas FC et al: Spinal cord sarcoidosis: a diagnostic
• Fewer multinucleated giant cells dilemma. Neurol Res. 23(4):347-52, 2001
• Areas of neural tissue infarction 7. Maroun FB et al: Sarcoidosis presenting as an
• Perivascular lymphocytic infiltrate intramedullary spinal cord lesion. Can J Neurol Sci.
o Also involving adventitia of vessels 28(2):163-6, 2001
8. Hayat GR et al: Solitary intramedullary neurosarcoidosis:
role of MRI in early detection. J Neuroimaging.
ICLINICAL ISSUES 9.
11(1):66-70, 2001
Yasui K et al: Correlation of magnetic resonance imaging
Presentation findings and histopathology of lesion distribution of spinal
cord sarcoidosis at post-mortem. Neuropathol Appl
• Most common signs/symptoms Neurobiol. 26(5):481-7, 2000
o Lower extremity weakness 10. Koike H et al: Differential response to corticosteroid
o Other signs/symptoms therapy of MRI findings and clinical manifestations in
• Paresthesia spinal cord sarcoidosis. J Neurol. 247(7):544-9, 2000
• Radiculopathy 11. Dumas JL et al: Central nervous system sarcoidosis:
• Bladder and bowel dysfunction follow-up at MR imaging during steroid therapy.
• Clinical profile: Acute to subacute onset Radiology. 214(2):411-20, 2000
12. Christoforidis GA et al: MR of CNS sarcoidosis: correlation
Demographics of imaging features to clinical symptoms and response to
• Age: 20-40 years old treatment. AJNR AmJ Neuroradiol. 20(4):655-69,1999
13. Hashmi M et al: Diagnosis and treatment of intramedullary
• Gender spinal cord sarcoidosis. J Neurol. 245(3):178-80, 1998
o Male> female in spinal sarcoidosis 14. Jallo GI et al: Intraspinal sarcoidosis: diagnosis and
o Slight female predominance in systemic sarcoidosis management. Surg Neurol. 48(5):514-20; discussion 521,
• Ethnicity 1997
o Sarcoidosis relatively more common in Northern 15. Lexa FJ et al: MR of sarcoidosis in the head and spine:
Europeans and African-Americans spectrum of manifestations and radiographic response to
o Rare in Asians steroid therapy. AJNR 5:973-82, 1994
16. Junger SS et al: Intramedullary spinal sarcoidosis: clinical
Natural History & Prognosis and magnetic resonance imaging characteristics.
• Favorable response to corticosteroid therapy Neurology. 43(2):333-7, 1993
o Two-thirds of neurosarcoidosis 17. Seltzer S et al: CNS sarcoidosis: evaluation with
contrast-enhanced MR imaging. AJNR Am J Neuroradiol.
• One-third with remitting relapsing course 12(6):1227-33, 1991
Treatment 18. Nesbit GM et al: Spinal cord sarcoidosis: a new finding at
MR imaging with Gd-DTPA enhancement. Radiology.
• Intravenous ± oral corticosteroids 173(3):839-43, 1989
o Chronic steroid therapy may be required 19. Terunuma H et al: Sarcoidosis presenting as progressive
• Immunosuppressive therapy myelopathy. Clin Neuropathol. 7(2):77-80, 1988
o Cyclophosphamide
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SARCOIDOSIS
IIMAGE GALLERY
Typical
(Left) Sagittal T1 C+ MR
shows diffuse mildly
heterogenous enhancement
involving mid and dorsal
aspect of cervical cord from
C2 to T2-3. (Right) Sagittal
T1 C+ MR with fat
suppression shows "multiple
vertebral sarcoid" lesions
with homogeneous
enhancement.
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MULTIPLE SCLEROSIS, SPINAL CORD
2
20
Sagittal graphic depicts multiple demyelinating plaques Sagittal STIR MR shows a hyperintense demyelinating
in cervical cord. plaque within thoracic cord without significant cord
expansion.
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MULTIPLE SCLEROSIS, SPINAL CORD
IIMAGE GALLERY
(Left) Sagittal T2WI MR of
cervical cord shows an
ill-defined intramedullary 2
hyperintense lesion at C5-6.
(Right) Axial T2WI MR of
cervical cord in another 23
patient shows a poorly
defined wedge-shaped
mildly hyperintense plaque
within right lateral aspect of
the cord.
Variant
(Left) Sagittal C+ MR shows
diffuse cord expansion,
extensive mulUlevel cord
enhancement in acute
exacerbation of MS. (Right)
Sagittal T2W MR shows
diffuse cord enlargement &
hyperintensity throughout
cervical segment into upper
thoracic cord in case of
acute MS exacerbation.
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ADEM, SPINAL CORD
2
24
Sagittal T2WI MR shows mulUple focal lesions with high Sagittal T1 C+ MR exhibits patch enhancement
signal intensity throughout thoracic cord in paUent throughout thoracic spine (which showed muWple focal
suffering from lower extremity weakness, paresthesias, high signal lesions on T2WI) in paUent with ADEM.
after viral illness.
ITERMINOLOGY MR Findings
• Tl WI: Focal low signal and slight cord swelling
Abbreviations and Synonyms
• T2WI
• Acute disseminated encephalomyelitis (ADEM) o Multifocal flame shaped white matter lesions with
Definitions slight cord swelling
o Dorsal white matter more voluminous
• Para/postinfectious immune mediated inflammatory
o May see gray matter involvement
disorder of the white matter
• PD/Intermediate: Sometimes depict the lesions more
sensitively
IIMAGING FINDINGS • T2* GRE: Same as T2WI, not as sensitive
• Tl C+
General Features o Variable enhancement, depending on stage of
• Best diagnostic clue: Multifocal white matter lesions disease
with relatively little mass effect or vasogenic edema o Punctate, ring-shaped, or fluffy enhancement
• Location o May see nerve enhancement
o Anywhere in the spinal cord white matter Imaging Recommendations
o Brain almost always involved
• Best imaging tool: Pre- and post-contrast MRI
• Size: Punctate to segmental • Protocol advice: Include brain MRI whenever
• Morphology: Plump lesions with feathery edges suspicious cord lesions found
Radiographic Findings
• Myelography: May see focal cord swelling
IDIFFERENTIAL DIAGNOSIS
CT Findings
• CECT: May see (multi)focal intramedullary Multiple sclerosis
enhancement • Indistinguishable on single study
• BrainMRI may help differentiate (cranial nerve
involvement indicates ADEM)
",.
"
'~""."
.,,-; .. J
.
.
~
Dural AVM Cord Infarct Transverse Myelitis MS
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ADEM, SPINAL CORD
Key Facts ..
Terminology • Viral or idiopathic transverse myelItIs
• Acute disseminated encephalomyelitis (ADEM) Pathology
• Para/postinfectious immune mediated inflammatory • Autoimmune process prodUcing inflammatory
disorder of the white matter reaction
Imaging Findings . Clinical Issues 2
• Best diagnostic clue: Multifocal white matter leslOns • Paresis
with relatively little mass effect or vasogenic edema 25
• Cranial nerve palsies
• Anywhere in the spinal cord white matter • Abnormal CSF
• Brain almost always involved • Usually no oligoclonal bands
• Multifocal flame shaped white matter lesions with • Age: Typically childhood or young adult
slight cord swelling • 50-60% recover completely
• Variable enhancement, depending on stage of disease
Diagnostic Checklist
Top Differential Diagnoses • Re-scanning if initially negative
• Multiple sclerosis • Caution: ADEM can mimic cord neoplasm!
• Immune-mediated vasculitis
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ADEM, SPINAL CORD
17. Murthy]M et al: Clinical, electrophysiological and
Natural History & Prognosis magnetic resonance imaging study of acute disseminated
• 50-60% recover completely encephalomyelitis.] Assoc Physicians India. 47(3):280-3,
• Neurologic sequelae in 30-40% 1999
• 10% mortality 18. Murthy]M: MRI in acute disseminated encephalomyelitis
following Semple antirabies vaccine. Neuroradiology.
• May see striking cord atrophy in chronic phase
40(7):420-3, 1998
Treatment 19. Kumada S et al: Encephalomyelitis subsequent to
2 • Options, risks, complications
mycoplasma infection with elevated serum anti-Gal C
antibody. Pediatr Neurol. 16(3):241-4, 1997
26 o Immunomodulation, supportive 20. Patel SP et al: Neuropsychiatric features of acute
o Plasmapheresis in fulminant case disseminated encephalomyelitis: a review. ]
Neuropsychiatry Clin Neurosci. 9(4):534-40,1997
21. Kinoshita A et al: Inflammatory demyelinating
I DIAGNOSTIC CHECKLIST polyradiculitis in a patient with acute disseminated
encephalomyelitis (ADEM).] Neurol Neurosurg Psychiatry.
Consider 60(1):87-90, 1996
• Re-scanning if initially negative 22. Kleiman M et al: Acute disseminated encephalomyelitis:
response to intravenous immunoglobulin. ] Child Neurol.
o Typically delay between clinical onset and
10(6):481-3, 1995
appearance of imaging findings 23. Baum PA et al: Deep gray matter involvement in children
with acute disseminated encephalomyelitis. A]NR Am]
Image Interpretation Pearls Neuroradiol. 15(7):1275-83, 1994
• Check thin-section intracranial slices on Tl C+ for
cranial nerve enhancement
• Caution: ADEM can mimic cord neoplasm!
ISELECTED REFERENCES
1. Garg RK: Acute disseminated encephalomyelitis. Postgrad
Med]. 79(927):11-7,2003
2. Miravalle A et al: Encephalitis complicating smallpox
vaccination. Arch Neurol. 60(7):925-8, 2003
3. Dale RC: Acute disseminated encephalomyelitis. Semin
Pediatr Infect Dis. 14(2):90-5, 2003
4. Idrissova ZhR et al: Acute disseminated encephalomyelitis
in children: clinical features and HLA-DR linkage. Eur ]
Neurol. 10(5):537-46,2003
5. Khong PL et al: Childhood acute disseminated
encephalomyelitis: the role of brain and spinal cord MRI.
Pediatr Radiol. 32(1):59-66, 2002
6. Au WY et al: Acute disseminated encephalomyelitis after
para-influenza infection post bone marrow
transplantation. Leuk Lymphoma. 43(2):455-7, 2002
7. Murthy SN et al: Acute disseminated encephalomyelitis in
children. Pediatrics. 110(2 Pt 1):e21, 2002
8. Khong PL et al: Childhood acute disseminated
encephalomyelitis: the role of brain and spinal cord MRI.
Pediatr Radiol. 32(1):59-66, 2002
9. Inglese M et al: Magnetization transfer and diffusion tensor
MR imaging of acute disseminated encephalomyelitis.
A]NR Am] Neuroradiol. 23(2):267-72, 2002
10. Arya SC: Acute disseminated encephalomyelitis associated
with poliomyelitis vaccine. Pediatr Neurol. 24(4):325, 2001
11. Lin WC et al: Sequential MR studies of a patient with white
matter disease presenting psychotic symptoms: ADEM
versus single-episode MS. Kaohsiung] Med Sci.
17(3):161-6,2001
12. Miyazawa R et al: Plasmapheresis in fulminant acute
disseminated encephalomyelitis. Brain Dev. 23(6):424-6,
2001
13. Bastianello S: Magnetic resonance imaging of MS-like
disease. Neurol Sci. 22 Suppl 2:S103-7, 2001
14. Tsuru T et al: Acute disseminated encephalomyelitis after
live rubella vaccination. Brain Dev. 22(4):259-61, 2000
15. Singh S et al: Acute disseminated encephalomyelitis and
multiple sclerosis: magnetic resonance imaging
differentiation. Australas Radiol. 44(4):404-11, 2000
16. Pradhan S et al: Intravenous immunoglobulin therapy in
.acute disseminated encephalomyelitis.] Neurol Sci.
165(1):56-61, 1999
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ADEM, SPINAL CORD
IIMAGE GAllERY
(Left) Sagittal T1 C+ MR
shows a vaguely enhancing
lesion in cervical cord of 2
patient with ADEM who had
numerous cord lesions on
T2WI. (Right) Sagittal 27
PO/Intermediate MR exhibits
confluent ill-defined
intramedullary lesions in
patient with ADEM.
Variant
(Left) Sagittal T2WI MR
shows focal cord lesion in
patient with extremity
weakness and cranial nerve
findings after a viral illness.
(Right) Axial T2WI MR
shows lesion in dorsal aspect
of cord in patient with
ADEM.
Variant
(Left) Sagittal T1 C+ MR
shows enhancing focal lesion
in distal cord. Patient
developed leg weakness and
cranial nerve signs after viral
illness. (Right) Axial T1 C+
MR shows focal enhancing
lesion in distal cord in
patient with ADEM.
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IDIOPATHIC ACUTE TRANSVERSE MYELITIS
2
28
Sagittal T2WI MR shows diffuse intramedullary Sagittal T1 C+ MR in the same patient does not show
hyperintensity with mild cord expansion from C2-] to significant post-gadolinium enhancement. Subtle
C7. intramedullary hypointensity is present.
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VITAMIN 812 DEFICIENCY, SPINAL CORD
2
32
Sagittal graphic shows dorsal column demyelination Axial T2WI MR reveals T2 hyperintensity confined to
secondary to Vitamin B 12 deficiency. Abnormality is the dorsal columns (arrows) in an "inverted V"
characteristically located in the dorsal spinal columns. configuration characteristic of B 12 deficiency.
ITERMINOlOGY MR Findings
• T1 WI: Mild spinal cord enlargement, dorsal spinal
Abbreviations and Synonyms cord hypointensity
• Subacute combined degeneration, combined system
• TZWI
disease o Hyperintensity confined to dorsal +/-lateral
Definitions columns
• Vitamin BIZ deficiency produces selective • Longitudinal dorsal cord TZ signal abnormality
degeneration of dorsal +/-lateral spinal cord columns • Axial image => "inverted V" or "inverted rabbit
ears" within dorsal spinal cord
• TI C+: +/- Mild dorsal column enhancement
IIMAGING FINDINGS Other Modality Findings
General Features • Brain MR imaging
o No specific findings in adults
• Best diagnostic clue: Mild spinal cord enlargement,
o May see severe brain atrophy that improves
abnormal TZ hyperintensity within dorsal columns following parenteral BIZ therapy in infants
• Location: Dorsal spinal cord columns +/-lateral
columns Imaging Recommendations
• Size: Imaging abnormality confined to abnormal • Multiplanar TZWI to confirm localization in dorsal
spinal tracts columns, exclude cord infarct or spondylosis
• Morphology: Mild spinal cord enlargement, abnormal
signal intensity within dorsal +/-lateral columns only
CT Findings IDIFFERENTIAL DIAGNOSIS
• NECT Spinal cord infarction
o Mild spinal cord enlargement in florid cases • Hyperacute presentation, motor> sensory symptoms
o Usually normal • Predominantly ventral cord or central gray matter
signal changes
Spinal Cord Infarct Spinal Cord Contusion Multipre Sclerosis Virar Myefitis
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Key Facts
Terminology • Acute transverse myelitis
• Subacute combined degeneration, combined system Pathology
disease • Methylmalonic acid accumulation believed to cause
• Vitamin BI2 deficiency produces selective myelin toxicity => dorsolateral spinal cord
degeneration of dorsal +/-lateral spinal cord columns demyelination (myelin sheaths + axons)
• Megaloblastic anemia
2
Imaging Findings
• Best diagnostic clue: Mild spinal cord enlargement, 33
Clinical Issues
abnormal T2 hyperintensity within dorsal columns • Insidious subacute course of clinical symptom onset;
• Axial image => "inverted V" or "inverted rabbit ears" functional disturbances increase fairly rapidly -
within dorsal spinal cord severe disability within weeks to months
Top Differential Diagnoses Diagnostic Checklist
• Spinal cord infarction • Neurologic findings may precede anemia
• Spinal cord contusion • Imaging changes may not completely resolve
• Inflammatory demyelination follOWing treatment
• Infectious myelitis
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IDIAGNOSTIC CHECKLIST
Consider
• Neurologic findings may precede anemia
• Imaging changes may not completely resolve
following treatment
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VITAMIN 812 DEFICIENCY, SPINAL CORD
I IMAGE GALLERY
Typical
(Left) Axial T I WI MR shows
"inverted V" hypointensity
confined to dorsal columns
(arrows). (Right) Axial T2WI
MR reveals T2 hyperintensity
within dorsal columns typical
of B 12 deficiency
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SECTION 1: Neoplasms
Extradural
Blastic Osseous Metastases IV-I-6
Lytic Osseous Metastases IV-I-lO
Hemangioma IV-I-I4
Osteoid Osteoma IV-I-I8
Osteoblastoma IV-I-22
Aneurysmal Bone Cyst IV-I-26
Giant Cell Tumor IV-I-30
Osteochondroma IV-I-34
Chondrosarcoma IV-I-38
Osteosarcoma IV-I-42
Chordoma IV-I-46
Ewing Sarcoma IV-I-SO
Lymphoma IV-I-54
Leukemia IV-I-58
Plasmacytoma IV-I-62
Multiple Myeloma IV-I-66
Neuroblastic Tumor IV-I-70
Angiolipoma IV-I-74
Intradural Extramedullary
Meningioma IV-I-78
Hemangiopericytoma IV-I-82
Schwannoma IV-I-86
Neurofibroma IV-I-90
Malignant Nerve Sheath Tumors IV-I-94
CSF Disseminated Metastases IV-I-98
Intramedullary
Astrocytoma, Spinal Cord IV-I-102
Ependymoma, Cellular, Spinal Cord IV-I-106
Ependymoma, Myxopapillary, Spinal Cord IV-I-110
Hemangioblastoma, Spinal Cord IV-I-1l4
Spinal Cord Metastases IV-I-1I8
Pa~aganglioma IV-I-I22
Melanocytoma IV-I-I26
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NEOPLASMS, PATHWAYS OF SPREAD
1
2
Axial graphic shows hematogenously disseminated lytic Axial T7WI MR shows large metastatic paravertebral
metastatic lesion to thoracic vertebral body and pedicle, mass lesion involving thoracic body, with extension to
with subsequent direct epidural tumor extension and posterior elements, chest wall and epidural tumor with
cord compression. cord compression (arrow).
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NEOPLASMS, PATHWAYS OF SPREAD
DIFFERENTIAL DIAGNOSIS
1
Direct extension of tumor Lymphatic spread 3
• Primary bone tumor mimic metastasis direct • Limited in importance relative to hematogenous
extension: Sarcoma, chordoma, ABC, osteoblastoma spread
• Infection may show destruction, soft tissue
involvement, thecal sac compression
CNS tumor types with subarachnoid spread
• Medulloblastoma, PNET
Hematogenous spread of tumor • Ependymoma
• Heterogeneous nonmalignant marrow signal may ·"'Pineal tumors
occur with chronic anemia, osteoporosis • Astrocytoma (glioblastoma)
• Mottled marrow signal classic sign of multiple
myeloma
Spread along CSF pathways from
hematogenous dissemination
CSF spread of tumor • Cord and leptomeningeal metastatic disease from
• Granulomatous disease (sarcoid, TB) can mimic lung, breast cancer
leptomeningeal mets
• Bacterial meningitis may show irregular
leptomeningeal enhancement
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NEOPLASMS, PATHWAYS OF SPREAD
1
4
Coronal graphic of thoracolumbar junction shows Axial T7 WI with contrast shows large paravertebral mass
metastatic neuroblastoma with paravertebral mass, extending into canal and displacing cord (arrow) from
multilevel epidural tumor extension. neuroblastoma.
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NEOPLASMS, PATHWAYS OF SPREAD
IMAGE GALLERY 1
5
(Left) Sagittal T1 C+ MR
shows bulky nodular and
linear enhancement of
leptomeninges in this patient
with CSF spread of
supratentorial glioblastoma.
(Right) Axial T1 C+ MR in
same case shows large cystic
and solid enhancing mass
involving corpus callosum,
with intraventricular
extension.
(Left) Sagittal T1 C+ MR
shows focal epidural mass
with cord compression from
metastatic melanoma. Route
of dissemination is
hematogenous. (Right)
Sagittal T1 C+ MR in
extensive metastatic breast
carcinoma shows foci of
enhancement from
leptomeningeal (arrows) and
intramedullary (open arrow)
metastases. Note multiple
vertebral body mets.
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Sagittal graphic of thoracic spine shows multiple Sagittal TI WI MR in this patient with prostate carcinoma
metastatic lesions in vertebral bodies, posterior elements shows diffuse low signal throughout all vertebral bodies
with dorsal + ventral epidural extension, cord and posterior elements from metastatic disease.
compression.
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BLASTIC OSSEOUS METASTASES
Key Facts
1
Terminology • Inhomogeneous marrow 7
• Extension of primary tumor to spine, where bone Pathology
production exceeds bone destruction • Osteosclerotic primaries include prostate, carcinoid,
Imaging Findings bladder, nasopharynx, medulloblastoma,
• Sclerosis may occur as discrete nodular appearance, neuroblastoma
mottled areas, to larger regions of diffuse increased • Mixed lytic and blastic primaries include lung, breast,
density cervix, ovarian
• MR image entire spinal axis Diagnostic Checklist
• Radionuclide studies for screening of entire skeleton • Defining response to therapy of sclerotic lesion
Top Differential Diagnoses difficult since osteolytic conversion (tumor
• Hemangioma progression) and fading (good response) may look
• Renal osteodystrophy identical
• Paget disease • Metastatic "ivory vertebral body" seen with prostate,
• Hematopoietic malignancy lymphoma, myeloma, chordoma
• Benign (osteoporotic) compression fracture
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BLASTIC OSSEOUS METASTASES
1 I PATHOLOGY Demographics
8 • Age: Children and adults
General Features • Gender: Predilection varies with specific tumor
• General path comments: Marrow initially infiltrated,
trabeculae destroyed, then cortex Natural History & Prognosis
• Etiology • Relentless, progressive; pathologic fracture, ESCC may
o Hematogenous dissemination (arterial or venous via ensue
Batson plexus) > perineural, lymphatic, CSF spread • Varies with histology of primary lesion
o Marrow infiltration precedes osseous
destruction/sclerosis Treatment
• Posterior vertebral body first, then pedicle • Pain relief with opioid medications, steroids for cord
o Primary tumor edema, pain
• Osteosclerotic primaries include prostate, • Radiation therapy
carcinoid, bladder, nasopharynx, • Surgical decompression, stabilization
medulloblastoma, neuroblastoma • Vertebroplasty
• Mixed lytic and blastic primaries include lung, • Embolization
breast, cervix, ovarian
• Epidemiology
o Vertebral metastases IDIAGNOSTIC CHECKLIST
• 10-40% of patients with systemic cancer
• Account for 40% of all bone metastases Consider
o 90% of prostate metastases involve spine, with • Defining response to therapy of sclerotic lesion
lumbar 3x more often than cervical difficult since osteolytic conversion (tumor
o Epidural spinal cord compression in 5% of adults progression) and fading (good response) may look
with systemic cancers (70% solitary, 30% multiple identical
sites) Image Interpretation Pearls
Gross Pathologic & Surgical Features • Metastatic "ivory vertebral body" seen with prostate,
• Softened, eroded bone +/- adjacent soft tissue mass lymphoma, myeloma, chordoma
Microscopic Features
• Varies with histology of primary, osteoclastic/blastic I SELECTED REFERENCES
response 1. Wahl RL: Current status of PET in breast cancer imaging,
staging, and therapy. Semin Roentgenol. 36(3):250-60,
Staging, Grading or Classification Criteria 2001
• Surgical stratification (Tomita et al) 2. Tomita K et al: Surgical strategy for spinal metastases.
o Grade of malignancy (slow growth, 1 point =;. rapid Spine. 26(3):298-306, 2001
growth, 4 points) 3. Castillo M et al: Diffusion-weighted MR imaging offers no
o Visceral metastases (no metastases, 0 points =;. advantage over routine noncontrast MR imaging in the
untreatable, 4 points) detection of vertebral metastases. A]NR. 21: 948-53, 2000
o Bone metastases (solitary, 1 point =;. multiple 2 4. Chamberlain MC et al: Epidural spinal cord compression.
Neuro-oncol. 1: 120-3, 1999
points) 5. Vanel D et al: MRI of bone metastases. Eur Radiol. 8:
o Total 2-3 points suggest wide excision for long term 1345-51, 1998
control 6. Ontell FK et al: Blastic osseous metastases in ovarian
o TotalS-IO points indicate nonoperative supportive carcinoma. Can Assoc Radiol]. 46(3):231-4,1995
care 7. Olson EM et al: Osseous metastasis in medulloblastoma:
MRI findings in an unusual case. Clin Imaging.
15(4):286-9, 1991
I CLINICAL ISSUES 8. Thrall JH et al: Skeletal metastases. Radiol Clin North Am.
25(6):1155-70, 1987
Presentation 9. Galasko CS: Mechanisms of lytic and blastic metastatic
disease of bone. Clin Orthop. (169):20-7, 1982
• Most common signs/symptoms 10. Kongtawng T et al: Radiographic evaluation of treatment of
o Progressive axial, referred, or radicular pain advanced carcinoma of the prostate. South Med J.
• Pain associated with cord compression not 71(3):247-50, 1978
relieved by recumbent position 11. Joffe N et al: Osteoblastic bone metastases secondary to
o Epidural tumor extension may cause paralysis, adenocarcinoma of the pancreas. Clin Radial. 29(1):41-6,
sensory loss, incontinence 1978
o Compression fracture 12. Lokich JJ: Osseous metastases: radiographic monitoring of
therapeutic response. Oncology. 35(6):274-6, 1978
o Other signs/symptoms
• Hypercalcemia
• Focal tenderness
• Soft tissue mass
• Clinical profile: Elderly male with prostate carcinoma,
new onset leg weakness, back pain
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Typical
(Left) Anteroposterior bone
scan shows marked
abnormal uptake in thoracic
spine, multiple ribs in this
patient with prostate
carcinoma. (Right) Sagittal
T7WI MR shows low signal
throughout multiple bodies
from prostate carcinoma.
There is confluent
involvement of upper
thoracic spine with epidural
extension, cord compression
(arrow).
Typical
(Left) Axial T7WI MR shows
diffuse low signal within
thoracic body and posterior
elements from prostate
carcinoma. Epidural
extension severely
compresses cord (arrows).
(Right) Sagittal T7WI MR
shows diffuse hypointensity
within all lumbar vertebral
bodies and posterior element
due to blastic metastases.
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LYTIC OSSEOUS METASTASES
1
10
Sagittal T1WI MR shows collapse of LJ with diffuse low Sagittal T2WI MR shows severe collapse of LJ with slight
signal. There is convex posterior margin to LJ, with heterogeneous increased signal. Convex posterior body
severe cauda equina compression (arrow) and anterior margin and thecal sac compression are well-defined.
paravertebral soft tissue.
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LYTIC OSSEOUS METASTASES
Key Facts
1
Terminology Pathology 11
• Extension of primary tumor to spine, where bone • Lytic metastases include: Breast, lung, kidney,
destruction exceeds bone production thyroid, oropharyngeal and nasopharyngeal, GI tract,
bladder, uterine, ovarian, melanoma, chordoma,
Imaging Findings paraganglioma
• Radiography requires 50-70% bone destruction for • Expansile osteolytic lesions with kidney, thyroid
detection, and> 1 cm metastasis
• Tc99m SPECT has high sensitivity with larger lesions, • Spine most frequent site of skeletal metastases
cortical involvement
• MR scan entire spinal axis Clinical Issues
• Pain: Progressive axial, referred, or radicular
Top Differential Diagnoses • Epidural tumor extension may cause paralysis,
• Hematopoietic malignancy sensory loss, incontinence
• Benign (osteoporotic) compression fracture
• Normal heterogeneous marrow Diagnostic Checklist
• Avascular necrosis • Only accurate investigation to establish presence and
site of compressive lesion is MR
o Cortex (especially posterior), pedicle destroyed • Radionuclide studies for screening of entire skeleton
o Intervertebral discs generally spared
o May cause pathologic fracture with
paraspinal/epidural mass I DIFFERENTIAL DIAGNOSIS
• Usually involves more than one quadrant
• "Draped curtain" sign = tumor spreads posteriorly Hematopoietic malignancy
into epidural space with relative midline sparing • Plasmacytoma, multiple myeloma (MM), lymphoma,
• T2WI leukemia
o Hypointense ~ hyperintense to normal marrow • Radionuclide studies negative/equivocal in 25% of
o "Target sign" ~ hyperintense rim surrounding MM
hypointense metastasis on T2WI • Diffuse marrow involvement more common than
• STIR: High intensity lesion in posterior metastasis
vertebra/pedicle
• DWI: Hyperintense (efficacy controversial)
Benign (osteoporotic) compression fracture
• T1 C+: Lesions diffusely enhance (may mask lesions if • May be difficult to distingUish acute osteoporotic Fx
no fat suppression used) (DWI may be helpful)
o 1/3 of Fxs in patients with known primary tumor are
Nuclear Medicine Findings benign
• Bone Scan o 1/4 of Fxs in apparently osteopenic patients are from
o SPECT superior to planar imaging malignant disease
o Tc99m SPECT has high sensitivity with larger • Marrow signal with late subacute/chronic benign
lesions, cortical involvement fractures similar to normal marrow (suppresses on
o Uptake in regions of bone production, not uptake STIR)
directly by tumor
o Predict level of neural compression < 20%
Normal heterogeneous marrow
o Bone scan false negatives most common with • Focal/irregular, patchy fatty marrow in older patients
myeloma, leukemia, anaplastic carcinomas • Intact pedicle, posterior vertebral cortex and no
epidural soft tissue
• PET
o FDG-PET has better specificity, but lower sensitivity Avascular necrosis
for detecting malignant bone metastases when • Vacuum cleft below endplate, usually anterior
compared with bone scan vertebral body
o FDG PET/CT has better specificity for detection of
malignant involvement of the spine than does FDG
PET alone IPATHOLOGY
Other Modality Findings General Features
• Biochemical markers indicate presence/extent of
skeletal metastases • General path comments
o Marrow initially infiltrated, trabeculae destroyed,
Imaging Recommendations then cortex
• MR scan entire spinal axis o May cause pathologic fracture (breast, lung), cord
o Standard MRI + STIR or fat-suppressed T2WI (scan compression
entire spine) o Both bone resorption and bone formation often
o Contrast-enhanced, fat-saturated T1WI present
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LYTIC OSSEOUS METASTASES
1 o Lytic lesions
• Lytic metastases include: Breast, lung, kidney,
ICLINICAL ISSUES
12 thyroid, oropharyngeal and nasopharyngeal, GI Presentation
tract, bladder, uterine, ovarian, melanoma, • Most common signs/symptoms
chordoma, paraganglioma o Pain: Progressive axial, referred, or radicular
• Expansile osteolytic lesions with kidney, thyroid o Epidural tumor extension may cause paralysis,
metastasis sensory loss, incontinence
• Etiology o Compression fracture
o Hematogenous dissemination (arterial or venous via o Other signs/symptoms
Batson plexus) > perineural, lymphatic, CSF spread • Hypercalcemia, focal tenderness, soft tissue mass
o Marrow infiltration precedes osseous destruction • Clinical profile: Back pain, weakness in patient with
• Posterior vertebral body first, then pedicle known primary tumor
o Primary tumor (adults)
• Lung, breast, prostate, lymphoma, renal most Demographics
common spinal metastases • Age
• Unknown primary in 15"25% o Children and adults
o Primary tumor (children): Sarcomas (Ewing, o Middle age to elderly most common
neuroblastoma), hematologic malignancies • Gender: Varies with specific tumor type
• Epidemiology
o Spine most frequent site of skeletal metastases Natural History & Prognosis
o Vertebral metastases • Spine metastases found in 5-10% of cancer patients
• 10-40% of patients with systemic cancer • Relentless, progressive; pathologic fracture, ESCC may
• Account for 40% of all bone metastases ensue
o Epidural spinal cord compression (ESCC) in 5% of • Varies with histology of primary lesion
adults with systemic cancers (70% solitary, 30% Treatment
multiple sites)
o ESCC occurs in 5% of children with malignant solid • Radiation therapy
• Surgical decompression, stabilization
tumors
• Invades canal via neural foramen • Vertebroplasty, embolization
• Circumferential cord compression common
o MRI of entire spine in patients with suspected
epidural extension show multiple sites in one-third I DIAGNOSTIC CHECKLIST
of patients Consider
Gross Pathologic & Surgical Features • Only accurate investigation to establish presence and
• Softened, eroded bone +/- adjacent soft tissue mass site of compressive lesion is MR
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HEMANGIOMA
1
14
Sagittal graphic of thoracolumbar junction shows the Axial NEeT shows typical corduroy pattern of thickened
typical striated pattern of hemangioma, with thickened trabeculae and intervening low attenuation fat.
bony trabeculae. There is no extraosseous extension or
thecal sac compromise.
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HEMANGIOMA
Key Facts
1
Terminology • MR best demonstrates aggressive characteristics 15
• Benign vertebral body vascular tumor • Sagittal and axial T1WI images most useful to
• Incidental lesion identified on imaging performed for characterize composition
unrelated reasons • Axial T2WI and enhanced T1WI best for
• Radiographic diagnostic criteria are lesion growth, characterizing epidural extent and cord compromise
bone destruction, vertebral collapse, absence of fat in (aggressive lesions)
lesion, and active vascular component • Axial bone algorithm CT is most useful for
• Rarer presentation (clinical or radiographic) is characteristic features that distinguish hemangioma
"aggressive hemangioma" from metastatic lesion
• May extend epidurally and cause cord compression Pathology
Imaging Findings • Slow growing
• Best diagnostic clue: Well-circumscribed, hypodense • Capillary, cavernous, or venous origin
lesion with coarse vertical trabeculae ("white polka • Most common spinal axis tumor
dots") on axial CT • 10-12% of adult population
• Both CT and MR can permit a specific diagnosis • 25-30% multiplei particularly in thoracic spine
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HEMANGIOMA
1 Microscopic Features Image Interpretation Pearls
16 • Benign lesions show mature, thin-walled, • Classic hemangioma: Hypodense lesion (CT) with
endothelium-lined capillary and cavernous sinuses coarse, vertically-oriented trabeculae; hyperintense
interspersed among sparse, osseous trabeculae and (MRI) on both T1WI and T2WI
fatty stromata
• Aggressive lesions contain less fat and more vascular
stroma ISELECTED REFERENCES
Staging, Grading or Classification Criteria 1. Bandiera S et al: Symptomatic vertebral hemangioma: the
treatment of 23 cases and a review of the literature. Chir
• Aggressive lesion features: Typical location between Organi Mov. 87(1): 1-15,2002
T-3 and T-9, involvement of the entire vertebral body, 2. Murugan L et al: Management of symptomatic vertebral
extension to neural arch, an expanded cortex with hemangiomas: review of 13 patients. Neurollndia. 50(3):
indistinct margins, irregular honeycomb pattern, soft 300-5,2002
tissue mass 3. Gabal AM: Percutaneous technique for sclerotherapy of
vertebral hemangioma compressing spinal cord.
Cardiovasc Intervent Radiol. 25(6): 494-500, 2002
4. Miszczyk L et al: The efficacy of radiotherapy for vertebral
I CLINICAL ISSUES hemangiomas. Neoplasma. 48(1): 82-4, 2001
Presentation 5. Bas T et al: Efficacy and safety of ethanol injections in 18
cases of vertebral hemangioma: a mean follow-up of 2
• Most common signs/symptoms years. Spine. 26(14): 1577-82,2001
o Benign hemangiomas are incidentally discovered 6. Baudrez V et al: Benign vertebral hemangioma:
o Symptomatic (aggressive) hemangiomas present MR-histological correlation. Skeletal Radiol. 30(8): 442-6,
with intense, localized spinal pain, myelopathy 2001
and/or radiculopathy from osseous expansion, 7. Cross JJ et al: Imaging of compressive vertebral
pathologic fracture, and/or epidural extension hemangiomas. Em Radiol. 10(6): 997-1002, 2000
8. Doppman JL et al: Symptomatic vertebral hemangiomas:
• Clinical profile: Middle-aged women with new onset
treatment by means of direct intralesional injection of
thoracic region pain ethanol. Radiology. 214(2): 341-8, 2000
Demographics 9. Lee S et al: Extraosseous extension of vertebral
hemangioma, a rare cause of spinal cord compression.
• Age: Peak incidence fourth to sixth decades Spine. 24(20): 2111-4, 1999
• Gender: M = F; aggressive lesions slightly more 10. Pastushyn AI et al: Vertebral hemangiomas: diagnosis,
common in women management, natural history and clinicopathological
correlates in 86 patients. Surg Neurol. 50(6): 535-47, 1998
Natural History & Prognosis 11. Sakata K et al: Radiotherapy of vertebral hemangiomas.
• Benign (fatty) hemangiomas - incidental lesions, Acta Oncol. 36(7): 719-24, 1997
excellent prognosis 12. Griffith JF et al: Clinics in diagnostic imaging (25).
• Aggressive vascular hemangiomas - variable depending Aggressive vertebral haemangioma. Singapore Med]. 38(5):
on size of lesion, degree of epidural extension, and 226-30, 1997
13. Jayakumar PN et al: Symptomatic vertebral haemangioma:
presence/absence of cord compression
endovascular treatment of 12 patients. Spinal Cord. 35(9):
Treatment 624-8,1997
14. Ide C et al: Vertebral haemangiomas with spinal cord
• Annual neurological and radiological examinations for compression: the place of preoperative percutaneous
patients with hemangiomas associated with pain vertebroplasty with methyl methacrylate. Neuroradiology.
• Regular monitoring for patients with asymptomatic 38(6): 585-9, 1996
lesions unnecessary unless pain develops at the 15. Guedea F et al: The role of radiation therapy in vertebral
appropriate spinal level hemangiomas without neurological signs. lnt Orthop.
• Aggressive hemangiomas 18(2): 77-9, 1994
o Direct intralesional injection of ethanol (post 16. Fox MW et al: The natural history and management of
ethanol treatment body collapse reported symptomatic and asymptomatic vertebral hemangiomas. J
Neurosurg. 78(1): 36-45, 1993
complication) 17. Laredo JD et al: Vertebral hemangiomas: fat content as a
o Vertebroplasty in conjunction with embolization sign of aggressiveness. Radiology. 177(2):467-72, 1990
and surgery as needed 18. Nguyen JP et al: Vertebral hemangiomas presenting with
o Post-operative radiation therapy in patients neurologic symptoms. Surg Neurol. 27(4):391-7, 1987
following subtotal tumor resection 19. Nicola N et al: Vertebral hemangioma: retrograde
embolization-stabilization with methyl methacrylate. Surg
Neurol. 27(5):481-6, 1987
I DIAGNOSTIC CHECKLIST 20. Ross JS et al: Vertebral hemangiomas: MR imaging.
Radiology. 165(1):165-9, 1987
Consider 21. Laredo JD et al: Vertebral hemangiomas: radiologic
evaluation. Radiology. 161(1):183-9, 1986
• Paget if vertebral body expansion; should have no
epidural soft tissue
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HEMANGIOMA
IIMAGE GALLERY 1
17
Typical
(Left) Sagittal NECT shows
thickened vertically oriented
trabeculae, with intervening
fatty stoma involving L4
body. (Right) Sagittal Tl WI
MR shows well-defined high
signal from the Tl body
consistent with hemangioma.
There is no epidural soft
tissue extension.
Typical
(Left) Sagittal TlWI MR
shows fatty signal intensity
throughout the L2 body with
linear low signal striations
due to thickened trabeculae.
(Right) Sagittal Tl WI MR in
patient 6 months following
alcohol treatment of L4
hemangioma shows severe
L4 collapse, with posterior
bony retropulsion and
compression of the thecal
sac (arrow).
Variant
(Left) Axial NECT shows
thickened, linear trabeculae
within L3 body. There is
severe thecal sac
compression from soft tissue
epidural extension, and bony
retropulsion (arrows). (Right)
Sagittal T2WI (left side)
shows upper thoracic
hemangioma with
extra osseous extension into
epidural space, and cord
compression (arrow).
Enhanced T7 WI (right side)
shows enhancing epidural
tissue.
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OSTEOID OSTEOMA
1
18
Axial graphic shows small, highly vascular tumor nidus Axial bone CT shows sclerotic C7 00 nidus (arrow).
of osteoid osteoma in left lamina, surrounded by dense Lesion is sharply demarcated, and surrounded by
reactive bone. sclerosis (open arrow). Diagnosis missed on MR, made
on radiograph, confirmed on CT.
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, ~~:-.
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~~
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OSTEOID OSTEOMA
Key Facts
1
Terminology • Unilateral absent pedicle or pars interarticularis 19
• Benign osteoid-producing tumor < 1.5 cm in size • Sclerotic metastasis, lymphoma
• The tumor is often called a "nidus" to distinguish it • Osteomyelitis
from the surrounding sclerotic, reactive bone Pathology
Imaging Findings • Epidemiology: 12% of all benign skeletal neoplasms
• Best diagnostic clue: Small radiolucent tumor nidus Clinical Issues
with surrounding sclerosis • Most common signs/symptoms: Night pain relieved
• 10% of 00 occur in spine by aspirin, NSAIDs
• Central, lucent tumor nidus often obscured by • 70% have scoliosis related to muscle spasm, concave
reactive sclerosis on side of tumor
• Focal scoliosis concave on side of tumor • Gait disturbance, muscle atrophy, torticollis
Top Differential Diagnoses sometimes seen
• Osteoblastoma • Majority occur in second decade of life
• Stress fracture of pedicle or lamina
• Unilateral spondylolysis
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OSTEOID OSTEOMA
1 • CT usually shows endplate destruction or destructive
arthritis of facet joints
• CT-guided percutaneous excision
• Thermo/photocoagulation
20 • Sequestrum tends to have irregular shape • Conservative observation (patients with well
• Often presents with night pain relieved by aspirin, controlled symptoms)
NSAIDs
Ewing sarcoma IDIAGNOSTIC CHECKLIST
• Diffuse edema may involve adjacent vertebral bodies
and ribs Consider
• Pleural thickening, effusion • Important cause of painful scoliosis in a child or
• Centered in vertebral body young adult
• No nidus on CT scan
Image Interpretation Pearls
• Thin-section CT most accurate in visualizing nidus
IPATHOLOGY • Edema of adjacent bones on MRI mimics extension of
infection across discs and joints
General Features
• Etiology: Benign tumor of osteoblastic origin
• Epidemiology: 12% of all benign skeletal neoplasms ISELECTED REFERENCES
Gross Pathologic & Surgical Features 1. Uu PT et al: Imaging of osteoid osteoma with dynamic
gadolinium-enhanced MR imaging. Radiology.
• Sharply-demarcated, round, pink-red mass (nidus) 227(3):691-700,2003
• Nidus can be shelled out from surrounding, sclerotic 2. Scuotto A et al: Unusual manifestation of vertebral osteoid
reactive bone which does not contain tumor osteoma: case report. Eur Radiol. 12(1):109-12,2002
3. Davies M et al: The diagnostic accuracy of MR imaging in
Microscopic Features osteoid osteoma. Skeletal Radiol. 31(10):559-69, 2002
• Web of osteoid trabeculae showing variable amounts 4. Lefton DR et al: Vertebral osteoid osteoma masquerading
of mineralization as a malignant bone or soft-tissue tumor on MRI. Pediatr
• Vascular, fibrous connective tissue Radiol. 31(2):72-5, 2001
• Similar histologically to osteoblastoma 5. Cove]A et al: Osteoid osteoma of the spine treated with
percutaneous computed tomography-guided
• No malignant potential
thermocoagulation. Spine. 25(10):1283-6, 2000
• Reactive zone may contain lymphocytes and 6. Radcliffe SN et al: Osteoid osteoma: the difficult diagnosis.
plasmocytes Eur] Radiol. 28(1):67-79, 1998
7. Gangi A et al: Percutaneous laser photocoagulation of
spinal osteoid osteomas under CT gUidance. A]NR Am]
ICLINICAL ISSUES Neuroradiol. 19(10):1955-8, 1998
8. Assoun] et al: Osteoid osteoma: MR imaging versus CT.
Presentation Radiology. 191(1):217-23, 1994
• Most common signs/symptoms: Night pain relieved by 9. Zambelli PY et al: Osteoid osteoma or osteoblastoma of the
aspirin, NSAIDs cervical spine in relation to the vertebral artery. ] Pediatr
Orthop. 14(6):788-92, 1994
• Clinical profile 10. Greenspan A: Benign bone-forming lesions: osteoma,
o 70% have scoliosis related to muscle spasm, concave osteoid osteoma, and osteoblastoma. Clinical, imaging,
on side of tumor pathologic, and differential considerations. Skeletal Radiol.
o Gait disturbance, muscle atrophy, torticollis 22(7):485-500, 1993
sometimes seen 11. Woods ER et al: Reactive soft-tissue mass associated with
osteoid osteoma: correlation of MR imaging features with
Demographics pathologic findings. Radiology. 186(1):221-5, 1993
• Age 12. Klein MH et al: Osteoid osteoma: radiologic and pathologic
o Majority occur in second decade of life correlation. Skeletal Radiol. 21(1):23-31, 1992
o Has been reported as late as seventh decade, but very 13. Raskas DS et al: Osteoid osteoma and osteoblastoma of the
spine.] Spinal Disord. 5(2): 204-11, 1992
rare Afshani E et al: Common causes of low back pain in
14.
• Gender: M:F = 2-3:1 children. Radiographies. 11(2): 269-91, 1991
15. Kransdorf M] et al: Osteoid osteoma. Radiographies.
Natural History & Prognosis
11(4):671-96, 1991
• Resection is curative in most cases 16. Crim]R et al: Widespread inflammatory response to
o Entire nidus must be removed or recurrence is osteoblastoma: the flare phenomenon. Radiology.
probable 177(3):835-6, 1990
o Radionuclide labeling can be used to localize
intraoperatively
• Spontaneous healing has been reported
Treatment
• Open excision
o Proximity to vertebral artery should be noted in
cervical 00
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OSTEOID OSTEOMA
IIMAGE GALLERY 1
21
(Left) Anteroposterior
radiography shows focal,
short-curve levoscoliosis in
young man with neck pain.
Sclerosis at C6 on right
suggested possible diagnosis
of 00. (Right) Sagittal T1 WI
MR images of cervical spine
show focal low signal from
C5 vertebral body & pedicle
from marrow reactive edema
is this patient with osteoid
osteoma.
Typical
(Left) Posteroanterior bone
scan (3 hour image) shows
intense, focal uptake on right
at T7 7 (arrow) and scoliosis
with concavity on side of
00. (Right) Axial bone CT
shows 00 nidus centered in
lamina (white arrow).
Reactive sclerosis extends
into vertebral body (black
arrow).
Typical
(Left) Axial NECT shows
pleural thickening (open
arrow) adjacent to 00 nidus
(arrow). Patient had pleuritic
chest pain. (Right) Axial
bone CT shows thick,
uniform reactive periosteal
reaction (arrow) in rib
adjacent to 00 of adjacent
lamina (not shown). Mild
pleural thickening is also
visible adjacent to rib.
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OSTEOBLASTOMA
1
22
Axial graphic shows expansile, highly vascular Axial T2WI MR shows OB (arrows) of L5 with
osteoblastoma arising in right lamina, and impinging on characteristic heterogeneous signal intensity. Bony
exiting nerve root. matrix is low signal. There is edema in the adjacent
vertebral body.
DDx: Osteoblastoma
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Osteoid Osteoma ABC Metastasis Osteosarcoma
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OSTEOBLASTOMA
Key Facts
1
Terminology • Inflammatory response may spread far beyond lesion 23
• Osteoblastoma (OB); giant osteoid osteoma • May have aneurysmal bone cyst component
• Benign tumor forming osteoid • Peritumoral edema may obscure lesion, mimic
malignancy or infection on MRI
Imaging Findings
• 40% of osteoblastomas occur in the spine
Top Differential Diagnoses
• Originate in neural arch • Osteoid osteoma (00)
• Matrix often not visible on spine radiographs, better • Aneurysmal bone cyst (ABC)
seen on CT • Osteogenic sarcoma (OGS)
• Scoliosis in 50-60% of cases Clinical Issues
• May be occult on radiographs; obtain CT in every • 90% in 2nd-3rd decades of life
case of young patient with painful scoliosis • 10-15% recurrence for typical OB
• Well-circumscribed, expansile lesion of neural arch • 50% recurrence for aggressive OB
• Often extends into vertebral body
• Narrow zone of transition, sclerotic rim
• Variable amount of mineralization
o Aggressive osteoblastoma
• Cortical breakthrough, wide zone of transition
IDIFFERENTIAL DIAGNOSIS
o Matrix mineralization Osteoid osteoma (00)
• Variable amount of mineralization •Smaller « 1.5 cm)
• May see small, irregular trabeculae • Round nidus with surrounding sclerotic bone
• Can be difficult to distinguish from calcified • Same age group
cartilage of chondrosarcoma, enchondroma • Pain usually more intense
o Inflammatory response may spread far beyond • Scoliosis common
lesion
• Widespread, ill-defined sclerotic bone around Aneurysmal bone cyst (ABC)
lesion • Expansile lesion of posterior elements
• Periosteal reaction adjacent ribs • ABC component present in 10-15% of OB
• Pleural thickening effusion • ABC may also be isolated, or associated with other
• Ossification of ligamentum flavum tumors
• Multiple blood-filled cavities with fluid-fluid levels
MR Findings • Matrix absent in ABC without OB
• TlWI: Low/intermediate signal
• T2WI Metastasis
o Intermediate/high signal intensity • Older patients
o Low signal areas of bone matrix • Usually destroys cortex rather than expanding
o May have aneurysmal bone cyst component • May be expansile, especially in renal cell carcinoma
• Fluid-fluid levels • Involves posterior elements and/or vertebral body
o Extensive peritumoral edema common ("flare
phenomenon") Osteogenic sarcoma (OeS)
o Peritumoral edema may involve adjacent bones and • Sarcoma containing bone matrix
soft tissues • Rare in spine
o Peritumoral edema may obscure lesion, mimic • More aggressive appearance on radiographs, CT
malignancy or infection on MRI • Wider zone of transition
o Pleural effusions common with thoracic OB • Cortical breakthrough rather than cortical expansion
• T1 C+ • Involves neural arch and/or vertebral body
o Variable enhancement Chordoma
o Peritumoral edema may enhance
• Involves vertebral body rather than posterior elements
Angiographic Findings • Common in sacrum, rare in vertebrae
• Intense tumor blush • No matrix: Purely lytic tumor
Nuclear Medicine Findings Infection
• Bone Scan: Positive 3 phase bone scan • MRI appearance of OB may mimic infection because of
inflammatory change in adjacent bones
Imaging Recommendations • Distinction between OB & infection easily made on CT
• CT with sagittal, coronal reformations
Fibrous dysplasia
• Rare in spine
• Expansile lesion of posterior elements
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OSTEOBLASTOMA
1 • May be lytic, or contain "ground glass" matrix or tiny
trabeculae of bone
• Gender: M:F = 2-2.5:1
24
Natural History & Prognosis
Chondrosarcoma • Grow slowly
• Rare in spine • 10-15% recurrence for typical OB
• Involves vertebral body and/or posterior elements • 50% recurrence for aggressive OB
• May have fairly unaggressive features on imaging
studies Treatment
• Cartilage calcification in arcs and rings • Curettage with bone graft or methylmethacrylate
placement
• Pre-operative embolization may be useful
IPATHOLOGY
General Features I DIAGNOSTIC CHECKLIST
• General path comments
o Prostaglandins released by tumor cause extensive Image Interpretation Pearls
peritumoral edema • Aggressive OB is difficult on imaging and histology to
o Pain usually less intense than with 00 distinguish from osteogenic sarcoma
o Wide zone of transition suspicious for osteogenic
Gross Pathologic & Surgical Features sarcoma
• Friable, highly vascular tumor • OB is important cause of painful scoliosis
• Red color due to vascularity
• Well demarcated from surrounding host bone
Microscopic Features
ISELECTED REFERENCES
1. Biagini R et al: Osteoid osteoma and osteoblastoma of the
• Prominent osteoblasts sacrum. Orthopedics. 24(11):1061-4, 2001
• Rims of osteoblasts along trabeculae 2. Okuda S et al: Ossification of the ligamentum flavum
• Vascular fibrous stroma associated with osteoblastoma: a report of three cases.
• Woven bone matrix of variable quantity Skeletal Radiol. 30(7):402-6, 2001
• Cartilage absent 3. Shaikh MI et al: Spinal osteoblastoma: CT and MR imaging
• ABC component found in 10-15% with pathological correlation. Skeletal Radiol. 28(1):33-40,
• "Aggressive" OB 1999
o Same features as above, plus 4. Saifuddin A et al: Osteoid osteoma and osteoblastoma of
the spine. Factors associated with the presence of scoliosis.
o More nuclear pleomorphism
Spine. 23(1):47-53, 1998
o Epithelioid osteoblasts 5. Cheung FM et al: Diagnostic criteria for pseudomalignant
Staging, Grading or Classification Criteria osteoblastoma. Histopathology. 31(2):196-200, 1997
6. Murphey MD et al: Primary tumors of the spine:
• Classic osteoblastoma Radiologic-pathologic correlation. RadioGraphies. 16:
• Aggressive osteoblastoma 1131-58,1996
o Borderline lesion with osteosarcoma 7. Greenspan A: Benign bone-forming lesions: osteoma,
o Locally aggressive but does not metastasize osteoid osteoma, and osteoblastoma. Skeletal Radiol.
o Sometimes called pseudomalignant OB 22(7):485-500, 1993
8. Boriani S et al: Osteoblastoma of the spine. Clin Ortho ReI
Res. 278: 37-45, 1992
9. Nemoto 0 et al: Osteoblastoma of the spine. Spine. 15:
ICLINICAL ISSUES 1272-80, 1990
10. CrimJR et al: Widespread inflammatory response to
Presentation osteoblastoma: the flare phenomenon. Radiology.
• Most common signs/symptoms 177(3):835-6, 1990
o Dull, localized pain 11. MirraJM et al: Bone tumors: Clinical, radiologic and
o Scoliosis concave on side of tumor pathologic correlations. vol. 1. 2nd ed. Philadelphia, Lea &
o Neurologic symptoms due to compression of cord, Feibiger. 399-430, 1989
nerve roots
• Painful scoliosis
o Possible causes in addition to OB include
o Trauma
o 00, other tumors
o Infection
o Spondylolysis
o Renal or retroperitoneal abnormality
Demographics
• Age
o 90% in 2nd-3rd decades of life
o Have been diagnosed in patients up to 7th decade of
life
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OSTEOBLASTOMA
I IMAGE GALLERY 1
25
(Left) Anteroposterior
radiography shows focal
levoscoliosis secondary to
upper thoracic OB. Tumor is
not visible. Note focal
pleural thickening (arrow), a
secondary sign of OB.
(Right) Posteroanterior bone
scan shows intense uptake at
OB of thoracic spine, with
surrounding "flare" of less
intense uptake in multiple
adjacent bones. Flare reflects
inflammatory response
around tumor.
Variant
(Left) Axial bone CT shows
aggressive OB of LS centered
in pedicle, with extensive
cortical breakthrough and
soft tissue ossification. Low
grade OCS could also have
this appearance. (Right)
Coronal bone CT shows OB
with 50ft tissue mass in spinal
canal. Mass originated in
lamina. It contains immature
ossification (arrows) and is
poorly marginated,
mimicking OCS.
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ANEURYSMAL BONE CYST
1
26
Axial graphic shows aneurysmal bone cyst with Axial T2WI MR shows ABC with multiple fluid-fluid
expansile, multicystic mass in posterior vertebral body & levels due to layering blood products. Lesion is sharply
pedicle extending into epidural space. Fluid-fluid levels demarcated, with a narrow zone of transition.
are characteristic.
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ANEURYSMAL BONE CYST
Key Facts
1
Terminology • Fluid-fluid levels caused by hemorrhage, blood 27
• Aneurysmal bone cyst (ABC) product sedimentation
• Expansile benign neoplasm containing thin-walled, • Tumor matrix absent
blood-filled cavities • Commonly extends into epidural space, may severely
narrow spinal canal
Imaging Findings
• 10-30% of ABC occur in spine/sacrum
Top Differential Diagnoses
• Arise in neural arch • Osteoblastoma
• 75-90% extend into vertebral body • Telangiectatic osteogenic sarcoma (OGS)
• Balloon-like expansile remodeling of bone • Metastases
• Centered in neural arch, extends into vertebral body • Giant cell tumor (GCT)
• Absent pedicle sign: Expansion of pedicle results in Clinical Issues
loss of pedicle contour on AP radiographs • Back pain, most severe at night
• Cortical thinning • Scoliosis
• Focal cortical destruction common • Neurologic signs and symptoms from root and/or
cord compression
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ANEURYSMAL BONE CYST
1 • Etiology
o 3 theories
• Radiation therapy may predispose to
radiation-induced sarcoma
28 o 1: Results from trauma + local circulatory
disturbance
o 2: Underlying tumor induces vascular process IDIAGNOSTIC CHECKLIST
(venous obstruction or AV fistulae)
• 5-35% of ABC associated with other tumor, most Consider
commonly GCT or OB • "Absent pedicle" sign caused by: ABC, osteoblastoma,
o 3: Neoplasm with cytogenetic abnormalities lytic OGS, metastasis, trauma, congenital absence of
• More than 1/2 cases show abnormality in pedicle
chromosomes 17p or 16
Image Interpretation Pearls
• Epidemiology: 1-2% of primary bone tumors are ABC
• Evaluation of pedicles on AP radiograph should be
Gross Pathologic & Surgical Features part of routine on every patient
• Spongy, red mass
• Multiple blood-filled spaces
ISELECTED REFERENCES
Microscopic Features
1. Lomasney LM et al: Fibrous dysplasia complicated by
• Typical aneurysmal bone cyst formation affectipg multiple cervical
o Cystic component predominates vertebrae. Skeletal Radiol. 32(9):533-6, 2003
• Cavernous blood-filled cysts of variable sizes 2. Pogoda P et al: Aneurysmal bone cysts of the sacrum.
• Lined by fibroblasts, giant cells, histiocytes, Clinical report and review of the literature. Arch Orthop
hemosiderin Trauma Surg. 123(5):247-51, 2003
o Solid components 3. Garneti N et al: Cervieal spondyloptosis caused by an
• Septations interposed between blood-filled spaces aneurysmal bone cyst: a case report. Spine. 28(4):£68-70,
2003
• Contain bland stroma with fibrous tissue, reactive
4. Chan MS et al: Spinal aneurysmal bone cyst causing acute
bone, giant cells cord compression without vertebral collapse: CT and MRI
• Solid ABC is rare variant findings. Pediatr Radiol. 32(8):601-4, 2002
o 5-8% of all ABCs 5. Lam CH et al: Nonteratomatous tumors in the pediatric
o Solid component predominates sacral region. Spine. 27(11):£284-7, 2002
o Propensity for spine 6. Papagelopoulos PJ et al: Treatment of aneurysmal bone
cysts of the pelVis and sacrum. J Bone Joint Surg Am.
83-A(11):1674-81,2001
7. Boriani S et al: Aneurysmal bone cyst of the mobile spine:
ICLINICAL ISSUES report on 41 cases. Spine. 26(1):27-35, 2001
8. DiCaprio MR et al: Aneurysmal bone cyst of the spine with
Presentation familial incidence. Spine. 25(12):1589-92, 2000
• Most common signs/symptoms 9. Bush CH et al: Treatment of an aneurysmal bone cyst of
o Back pain, most severe at night the spine by radionuclide ablation. AJNR Am J
o Other signs/symptoms Neuroradiol. 21(3):592-4, 2000
• Scoliosis 10. Ozaki T et al: Aneurysmal bone cysts of the spine. Arch
• Neurologic signs and symptoms from root and/or Orthop Trauma Surg. 119(3-4):159-62, 1999
cord compression 11. Papagelopoulos PJ et al: Aneurysmal bone cyst of the spine.
Management and outcome. Spine. 23(5):621-8, 1998
• Pathologic fracture 12. de Kleuver M et al: Aneurysmal bone cyst of the spine: 31
• Clinical profile: Young patient with back pain of cases and the importance of the surgical approach. J
insidious onset Pediatr Orthop B. 7(4):286-92, 1998
13. Murphey MD et al: Primary tumors of the spine:
Demographics Radiologic-pathologic correlation. RadioGraphies.
• Age: 80% less than 20 years old 16:1131-58, 1996
• Gender: Slightly more common in females 14. Koci TM et al: Aneurysmal bone cyst of the thoracic spine:
• Familial incidence has been reported evolution after particulate embolization. AJNR Am J
Neuroradiol. 16(4 Suppl):857-60, 1995
Natural History & Prognosis 15. Kransdorf MJ et al: Aneurysmal bone cyst: Concept,
• Long term history of untreated ABC variable controversy, clinical presentation, and imaging. AJR. 164:
o Grows initially, then usually stabilizes 573-80, 1995
o No malignant degeneration
• Recurrence rate 20-30% (increased if incomplete
excision)
Treatment
• Embolization
o Curative as sole therapy in some cases
o Can also be used pre-operatively
• Surgical excision
• May require instrumentation for stabilization of spine
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IIMAGE GALLERY 1
29
(Left) Anteroposterior
radiography shows ABC of
T72 with "absent pedicle"
sign (open arrow). Arrows
show superior and inferior
extent of tumor, which
involved vertebral body as
well as neural arch. (Right)
Sagittal T2WI MR shows
ABC of T7 2, with vertebral
body involvement (arrow)
and extension into spinal
canal (open arrow). Lesion
was centered on pedicle (not
shown).
Typical
(Left) Axial bone CT shows
ABC of C2, causing
ballooning of posterior
elements and sharply
demarcated lytic region in
vertebral body. (Right) Axial
NECT shows ABC of C2,
with multiple fluid-fluid
levels (arrows) due to
layering of blood products.
Typical
(Left) Anteroposterior
radiography shows ABC
causing balloon-like
expansion of L2 transverse
process (open arrows) and
pedicle. Sharply-demarcated
lytic lesion extends into
vertebral body (arrow).
(Right) Anteroposterior
angiography shows ABC of
L2 with draping of vessels
around lesion periphery, as
well as regions of
neovascularity centrally.
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Anteroposterior radiography shows sacral GCT (curved Axial bone CT shows sacral GCT involving 51 and 52
arrows). Recognition of lytic sacral lesions is more neural foramina. There is focal cortical destruction
difficult because of similar appearance of overlying (arrow). Zone of transition is narrow.
bowel gas (open arrow).
ITERMINOLOGY CT Findings
Abbreviations and Synonyms • Bone CT
o Features described for radiography more clearly seen
• Giant cell tumor (GCT) o May contain fluid attenuation regions due to
Definitions necrosis or focal aneurysmal bone cyst (ABC)
• Locally aggressive neoplasm composed of component
o Absence of matrix best seen on noncontrast CT
osteoclast-like giant cells
MR Findings
• Tl WI: Low-intermediate signal intensity
IIMAGING FINDINGS • T2WI
General Features o Intermediate-high signal intensity
o Fluid-fluid levels if ABC present
• Best diagnostic clue: Lytic lesion in vertebral body or
o Use to evaluate extraosseous component of tumor
sacrum
• STIR: Intermediate-high signal intensity
• Location
o Spine: 3% of all GCT • Tl C+
o Heterogeneous enhancement
o Centered in vertebral body
o Areas of necrosis common
o Sacrum: 4% of all GCT
• Hemosiderin may be seen, low signal on Tl WI and
o Rarely involves multiple sites T2WI
Radiographic Findings Nuclear Medicine Findings
• Radiography • Bone Scan: Positive all three phases
o Lytic, expansile lesion
o Narrow zone of transition Imaging Recommendations
o Margin usually not sclerotic • Best imaging tool
o Matrix absent, but may have residual bone o CT scan for diagnosis
trabeculae o MR for evaluation of spinal canal, nerve roots
o May have cortical breakthrough
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GIANT CELL TUMOR
Key Facts
1
Terminology • Osteogenic sarcoma (OGS) 31
• Locally aggressive neoplasm composed of • Osteoblastoma
osteoclast-like giant cells • Myeloma
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GIANT CELL TUMOR
1 o Difficult histologic diagnosis
IDIAGNOSTIC CHECKLIST
o Anaplasia and pleomorphism of giant cells
32 o Distinguished from giant cell rich OGS by lack of Image Interpretation Pearls
osteoid formation by tumor cells • Sacral GCT may be occult on radiographs; have low
Staging, Grading or Classification Criteria threshold for CT in patients with atypical sacral pain
., Patients treated with radiation must be followed with
• Typical GCT
o Grading systems have been proposed, but not MRI because of risk of recurrence, sarcomatous
predictive of behavior degeneration
• Malignant GCT
o High risk of local recurrence and metastasis to bone,
lung, liver ISELECTED REFERENCES
1. Bertoni F et al: Malignancy in giant cell tumor of bone.
Cancer. 97(10):2520-9, 2003
ICLINICAL ISSUES 2. Lackman RD et al: The treatment of sacral giant-cell
tumours by serial arterial embolization. J Bone Joint Surg.
Presentation 84(B):873-7,2002
3. Garcia-Bravo A et al: Secondary tetraplegia due to giant-cell
• Most common signs/symptoms: Back pain of insidious tumors of the cervical spine. Neurochirurgie. 48(6):527-32,
onset, greatest at night 2002
• Clinical profile 4. Murphey MD et al: From the archives of AFIP. Imaging of
o Pathologic fracture in 30% giant cell tumor and giant cell reparative granuloma of
o Limited range of motion of joint adjacent to affected bone: radiologic-pathologic correlation. Radiographics.
area 21(5):1283-309, 2001
5. Meis JM et al: Primary malignant giant cell tumor of bone:
Demographics "Dedifferentiated" giant cell tumor. Mod Pathol. 2:541-6,
• Age 1995
o 80% 3rd to 5th decade of life 6. Schutte HE et al: Giant cell tumor in children and
o In spine, peak incidence 2nd and 3rd decades adolescents. Skeletal Radiol. 22:173-6, 1993
7. Manaster BJ et al: Giant cell tumor of bone. Radiol Clin
o Rare before skeletal maturity North Am. 31:299-323, 1993
• Gender: M:F = 1-2.5 in spine (more marked female 8. Tubbs WS et al: Benign giant-cell tumor of bone with
preponderance than in appendicular skeleton) pulmonary metastases: Clinical findings and radiologic
appearance of metastases in 13 cases. Am J Roentgenol.
Natural History & Prognosis 158:331-4, 1992
• Locally aggressive: 12-50% recurrence rate 9. Aoki J et al: Giant cell tumors of bone containing large
• Can undergo sarcomatous transformation, amounts of hemosiderin: MR-pathologic correlation. J
spontaneously or in response to radiation therapy Comput Assist Tomogr. 15:1024-7, 1991
• Pulmonary implants: 1-2% 10. Bridge JA et al: Cytogenetic findings and biologic behavior
o Occurs in patients with stage 3 disease of giant cell tumors of bone. Cancer. 65:2697-703, 1990
11. Mirra JM et al: Bone tumors: Clinical, radiologic and
o Within 3 years after removal of primary GCT
pathologic correlations. vol. 1. 2nd ed. Philadelphia, Lea
o Histologically identical to primary GCT &Feibiger. 942-1019, 1989, 1989
o Self limited growth potential 12. Carrasco CH et al: Giant cell tumors. Orthop Clin North
o May regress spontaneously Am. 20:395-405, 1989
o Resection is curative
• Primary malignant GCT (rare)
o Poor prognosis
o Metastasis to lung, liver, bone
Treatment
• Curettage alone associated with high rate of recurrence
• Cryotherapy or thermocoagulation used to reduce
recurrence
• Curettage defect filled with methylmethacrylate or
bone graft
• Radiation only for cases of unresectable tumor
o Risk of sarcoma years after treatment
• Arterial embolization
o Used pre-operatively
o Used for therapy for unresectable tumor
• Surgical resection of pulmonary implants
o Good prognosis following resection
• Malignant GCT treated with wide resection,
chemotherapy, radiation
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GIANT CELL TUMOR
I IMAGE GALLERY 1
33
Variant
(Left) Axial NECT shows
malignant sacral GCT later
metastasizing to bone, liver
and lung. Note extensive
cortical breakthrough, ST
mass (arrow), and
permeative appearance in
left sacral ala (curved
arrow). (Right) Sagittal STIR
MR shows malignant sacral
GCT (arrows). Clues
indicating tumor is more
aggressive than most GCT
include larger ST mass and
more extensive cortical
destruction
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OSTEOCHONDROMA
1
34
Axial graphic shows a typical osteochondroma Coronal T2WI MR shows a large left T1 transverse
(exostosis) protruding into the central canal, producing process osteochondroma. Hyperintense cartilage cap
spinal cord compression. thickness > 1.5 cm suggests chondrosarcoma, but
benign pathology found at surgery.
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OSTEOCHONDROMA
Key Facts
1
Terminology • Metabolic and connective tissue diseases 35
• Synonyms: OC, osteocartilaginous exostosis, exostosis Pathology
• Hereditary multiple exostoses (HME) = familial • Narrow stalk (pedunculated) or broad attachment
osteochondromatosis, diaphyseal aclasis base (sessile) contiguous with vertebral cortex,
Imaging Findings medullary space
• Best diagnostic clue: Sessile or pedunculated osseous Clinical Issues
"cauliflower" lesion with marrow/cortical continuity • Asymptomatic incidental diagnosis on radiography
with parent vertebra • Palpable mass
• Cervical (50%, C2 predilection) > thoracic (T8 > T4 > • Myelopathy; onset often follows trauma
other levels) > lumbar> > sacrum
• Spinous/transverse processes> vertebral body Diagnostic Checklist
• Multiplicity ~ consider HME
Top Differential Diagnoses • Interpret cartilage thickness in context of patient age
• Chondrosarcoma • Cartilage cap> 1.5 cm in adults raises concern for
• Osteoblastoma malignant transformation (chondrosarcoma)
• Aneurysmal bone cyst (ABC)
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OSTEOCHONDROMA
1 o Radiation-induced OC
• Most common benign radiation-induced tumor; Treatment
36 prevalence 6-24% • Conservative management in asymptomatic patients
• Radiation dose 1,500-5,500 cGYi occur at • Surgical excision, deformity correction in symptomatic
treatment field periphery, latent period 3-17 y, patients
patients generally < 2 yo at time of XRT
• Pathologically and radiographically identical to
other exostoses IDIAGNOSTIC CHECKLIST
• Epidemiology Consider
o Prevalence of solitary OC unknown since many
asymptomatic • Multiplicity - consider HME
o Prevalence of HME 1:50,000 to 1:100,000 in Western • Interpret cartilage thickness in context of patient age
populations; up to 1:1,000 in ethnic Chamorros Image Interpretation Pearls
Gross Pathologic & Surgical Features • Cartilage cap> 1.5 cm in adults raises concern for
• Osseous excrescence with cortex, medullary cavity malignant transformation (chondrosarcoma)
contiguous with parent bone • Radiologic features pathognomonic, reflect pathologic
• Cartilage cap ranges from thick bosselated glistening appearance
blue-gray surface (young patients) to several
millimeters thick or entirely absent (adults)
ISELECTED REFERENCES
Microscopic Features 1. Taitz J et al: Osteochondroma after total body irradiation:
• Mature cartilaginous, cancellous, and cortical bone an age-related complication. Pediatr Blood Cancer.
• Cartilage cap histology reflects classic growth plate 42(3):225-9, 2004
zones 2. Fiecht! JF et al: Spinal osteochondroma presenting as
atypical spinal curvature: a case report. Spine.
28(13):E252-5,2003
ICLINICAL ISSUES 3. Jones KB et al: Of hedgehogs and hereditary bone tumors:
re-examination of the pathogenesis of osteochondromas.
Iowa Orthop J. 23:87-95, 2003
Presentation 4. Sharma MC et al: Osteochondroma of the spine: an
• Most common signs/symptoms enigmatic tumor of the spinal cord. A series of 10 cases. J
o Asymptomatic incidental diagnosis on radiography Neurosurg Sci. 46(2):66-70; discussion 70, 2002
o Palpable mass 5. Pierz KA et al: Hereditary multiple exostoses: one center's
o Myelopathy; onset often follows trauma experience and review of etiology. Clin Orthop.
o Other signs/symptoms (401):49-59, 2002
• Cranial nerve deficits (dysphagia, hoarseness), 6. Jose Alcaraz Mexia M et al: Osteochondroma of the
thoracic spine and scoliosis. Spine. 26(9):1082-5, 2001
pharyngeal mass, scoliosis, radiculopathy 7. Wuyts W et al: Molecular basis of multiple exostoses:
• Clinical profile mutations in the EXTI and EXT2 genes. Hum Mutat.
o Many solitary OC patients present with painless 15(3):220-7,2000
slowly growing mass, but may manifest identical 8. Ratliff J et al: Osteochondroma of the C5 lamina with cord
symptoms to HME patients compression: case report and review of the literature.
o HME patients more often symptomatic; many Spine. 25(10):1293-5, 2000
exostoses produce impingement, other symptoms 9. Murphey MD et al: Imaging of osteochondroma: variants
referable to OC location and complications with radiologic-pathologic correlation.
Radiographies. 20(5):1407-34, 2000
• Mechanical pain - 2° to bursitis over exostosis, 10. Silber JS et al: A solitary osteochondroma of the pediatric
surrounding tendon/muscle/neural irritation, OC thoracic spine: a case report and review of the literature.
stalk fracture, or infarct/ischemic necrosis Am] Orthop. 29(9):711-4, 2000
11. Govender S et al: Osteochondroma with compression of
Demographics the spinal cord. A report of two cases. ] Bone Joint Surg Br.
• Age 81(4):667-9, 1999
o Peak age = 10-30 Y 12. Okuyama K et al: Huge solitary osteochondroma at TIl
o Most HME patients diagnosed by age 5 y, virtually level causing myelopathy: case report. Spinal Cord.
all by 12 Y 35(11):773-6, 1997
• Gender: M:F = 3:1 13. Mikawa Y et al: Cervical spinal cord compression in
hereditary multiple exostoses. Report of a case and a review
Natural History & Prognosis of the literature. Arch Orthop Trauma Surg. 116(1-2):112-5,
• Post-surgical local recurrence rate < 2% 1997
14. Murphey MD et al: From the archives of the AFIP. Primary
• Benign lesions; no propensity for metastasis tumors of the spine: radiologic pathologic correlation.
• Malignant transformation < 1% solitary lesions; 3-5% Radiographies. 16(5):1131-58, 1996
HME 15. Morikawa M et al: Osteochondroma of the cervical spine:
o Markers for possible malignant degeneration MR findings. Clin Imaging. 19: 275-8, 1995
• Growth or new pain after skeletal maturity,
cartilage cap thickness (> 1.5 cm in adults)
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OSTEOCHONDROMA
IIMAGE GALLERY 1
37
Typical
(Left) Axial bone CT shows a
typical exostosis protruding
into central spinal canal.
Marrow contiguity with
vertebral body is typical.
(Right) Axial Tl C+ MR
shows a typical exostosis
protruding into central spinal
canal, producing cord
compression. Marrow
enhances minimally, similar
to parent vertebral body.
Typical
(Left) Coronal TlWI MR
demonstrates a large left Tl
osteochondroma, with
internal marrow high signal
and hypointense
cartilaginous cap. (Right)
Axial Tl WI MR shows a large
left T1 osteochondroma
presenting with brachial
plexopathy in an HME
patient. Tumor fills left
C7/T1 foramen (arrows).
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CHONDROSARCOMA
1
38
Sagittal graphic of lumbosacral junction shows large soft Axial Tl C+ MR shows large mass with peripheral
tissue mass destroying bone, with presacral and epidural irregular enhancement arising from left side of thoracic
extension. vertebral body. There is extension of tumor to left
epidural space (arrow).
• Humerus: 10%
ITERMINOlOGY o Craniofacial bones: 2%, can arise from laryngeal
Abbreviations and Synonyms cartilage
o Spine and sacrum: 5%
• Conventional chondrosarcoma, dedifferentiated
o Soft tissues (extraskeletal chondrosarcoma)
chondrosarcoma, mesenchymal chondrosarcoma
• Size: 1-40 cm, mean: 10 cm
Definitions • Morphology: Ill-defined lytic lesion with or without
• Malignant tumor of connective tissue, characterized chondroid matrix
by formation of cartilage matrix by tumor cells
Radiographic Findings
• Radiography
IIMAGING FINDINGS o Lytic mass with or without chondroid matrix
o Medullary (central) chondrosarcoma: 85%
General Features • Expansion of medullary cavity
• Best diagnostic clue: LytiC mass with or without • Thickening of cortex with endosteal scalloping
chondroid matrix, cortical disruption and extension • Popcorn-like ring and arc calcification
into soft tissues • Cortical disruption with extension into soft tissue
• Location in advanced stages
o Flat bones o Exostotic (peripheral) chondrosarcoma
• Pelvis: 25% • Thickening of cortex and soft tissue mass at site of
• Ilium: 15% attachment to bone
• Pubis and ischium: 9% • Chondroid matrix
• Scapula: S% • Late destruction of bone
o Ribs and sternum: 12% o Dedifferentiated chondrosarcoma: 10%
o Metaphysis/diaphysis of long bones, may extend • Ill-defined, lytic lesion in continuity with
into epiphysis cartilaginous tumor
• Femur: 15% • Abrupt transition between cartilaginous tumor
• Tibia: 5% and dedifferentiated lytic component
o Mesenchymal chondrosarcoma: < 1%
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CHONDROSARCOMA
Key Facts
1
Imaging Findings Pathology 39
• Best diagnostic clue: Lytic mass with or without • Can occur as primary chondrosarcoma or as
chondroid matrix, cortical disruption and extension malignant degeneration of osteochondroma or
into soft tissues enchondroma
• Chondroid matrix mineralization of "rings and arcs" • Third most common primary malignant bone tumor
(characteristic) • 10-25% of all primary bone sarcomas
• Extraskeletal chondrosarcoma: 2% of all soft tissue
Top Differential Diagnoses sarcomas
• Lymphoma/metastatic disease
• Chondroblastoma/low grade chondroid lesion Clinical Issues
• Osteogenic sarcoma (OGS) • Dull aching pain at rest, may be severe at night
• Malignant fibrous histiocytoma (MFH) • Soft tissue swelling, mass
• Myositis ossificans • Prognosis depends on location, size, and histologic
grade
• Overall 5 year survival: 48-60%
• Wide surgical excision
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CHONDROSARCOMA
1 • Accumulation of p53 protein, may be indicator of
poor prognosis
• Duration of symptoms - several months to years
• Clinical profile
40 • Etiology o Soft tissue swelling, mass
o Can occur as primary chondrosarcoma or as o If lesion is close to joint, restricted range of motion
malignant degeneration of osteochondroma or
enchondroma Demographics
o Exostotic (peripheral) chondrosarcoma • Age: 20-90 years, peak: 40-60 years
• Malignant degeneration of hereditary multiple • Gender: M:F = 2:1
exostoses, Ollier disease Natural History & Prognosis
• Can arise in cartilage cap of a previously benign
• Prognosis depends on location, size, and histologic
osteochondroma grade
• Epidemiology o Overall 5 year survival: 48-60%
o Third most common primary malignant bone tumor o 5 year survival 90% for grade 1,81% for grade 2,
o 10-25% of all primary bone sarcomas 29% for grade 3 tumors
o Extraskeletal chondrosarcoma: 2% of all soft tissue
• Recurrence 5-10 years after surgery
sarcomas o Recurrence can be associated with increased
Gross Pathologic & Surgical Features histologic grade and more aggressive behavior
• Lobulated tumor composed of translucent hyaline • Metastases (lung, lymph nodes, liver, kidneys, brain):
nodules (resemble normal cartilage) 66% (grade 3 chondrosarcomas)
o Mineralization at periphery of nodules • Degeneration into fibrosarcoma, malignant fibrous
• Focal ivory-like areas represent extensive enchondral histiocytoma, osteosarcoma in 10%
ossification • Pelvic chondrosarcoma may invade bladder or colon
• Hemorrhagic necrosis, especially in high grade tumors • Pathologic fractures rare
• Central lesions erode and destroy cortex with Treatment
extension into surrounding soft tissue
• Wide surgical excision
o Cortical disruption earlier in flat bones due to
• Limited role for chemotherapy or radiation therapy
narrow medullary cavity o For high grade chondrosarcomas
• Extraosseous component grows on outer bone surface • Biopsies must be planned with future tumor excision
o Encircles affected bone in mind
Microscopic Features
• Irregular-shaped lobules of cartilage
• May be separated by narrow, fibrous bands IDIAGNOSTIC CHECKLIST
• Chondrocytes arranged in clusters, can be
Consider
mononuclear or multinucleated
• Matrix: Mature hyaline cartilage or myxoid stroma • Enlarging or painful enchondroma or
• Immunohistochemistry: Positive for 5-100 protein and osteochondroma suspicious for sarcomatous
vimentin transformation
• Clear cell chondrosarcoma may be indistinguishable
• Dedifferentiated chondrosarcoma: Two components
from benign chondroid lesions (chondroblastoma,
that coexist in one tumor
o High grade sarcoma +/- heterologous elements enchondroma)
(MFH,OGS) • Always consult orthopedic surgeon before biopsy of
o Low grade cartilaginous lesion possible chondrosarcoma
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CHONDROSARCOMA
IIMAGE GALLERY 1
41
(Left) Sagittal T1 WI MR
shows large soft tissue mass
involving left paravertebral
region, extending over
multiple levels. (Right)
Sagittal T2WI MR in same
patient shows lobulated
mass of increased signal.
There is typical linear
striations within the mass
consistent with chondroid
lesion.
Typical
(Left) Axial T2WI MR shows
a large, lobulated
hyperintense mass involving
thoracic body (arrows) with
epidural extension (open
arrow). (Right) Sagittal STIR
MR shows hyperintense
mass involving upper
thoracic body and posterior
elements. There is a large
amount of dorsal epidural
extension with cord
compression.
Typical
(Left) Axial NEeT shows
destruction of medial rib,
and calcific matrix (arrow)
within left paravertebral soft
tissue mass. (Right) Axial
NECT shows a large, partially
calcified mass adjacent to
the right sacrum, consistent
with a soft tissue
chondrosarcoma (Courtesy
O. Stoller, MO).
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OSTEOSARCOMA
1
42
Axial graphic shows secondary osteosarcoma arising in Lateral radiography shows metastatic oes to lumbar
Pagetic vertebral body, destroying cortex & invading spine. Multiple deposits of amorphous bone matrix are
adjacent soft tissues. Tumor has wide zone of transition. visible in vertebral bodies and pedicles.
Definitions CT Findings
• Sarcoma containing osteoid produced directly by the • CECT: Contrast administration tends to obscure bone
malignant cells matrix and is not recommended
• Matrix (material produced by tumor cells) is • Bone CT
immature, woven osteoid o Best visualization of bone matrix, wide zone of
transition, soft tissue mass
o Moth eaten bone destruction
IIMAGING FINDINGS MR Findings
General Features • TlWI
o Low signal intensity: Mineralized tumor
• Best diagnostic clue: Aggressive lesion forming
o Low-intermediate signal intensity: Solid,
immature bone
nonmineralized tumor
• Location
o 4% of all primary OGS occur in spine and sacrum • T2WI
o Low signal intensity: Mineralized tumor
o 79% arise in posterior elements
o High signal intensity: Nonmineralized tumor, soft
o 17% involve 2 adjacent spinal levels
tissue mass
o 84% invade spinal canal
o Fluid/fluid levels seen in telangiectatic OGS
• Size: Several centimeters • Appearance similar to other sarcomas; use CT for
Radiographic Findings matrix visualization
• Radiography Nuclear Medicine Findings
o Wide zone of transition
o Permeative appearance • Bone Scan
o Increased uptake on all three phases
o Cortical breakthrough and soft tissue mass
.. .
Metastasis
,• , .
~.
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OSTEOSARCOMA
Key Facts
1
Terminology • Best imaging tool: CT scan 43
• Sarcoma containing osteoid produced directly by the Top Differential Diagnoses
malignant cells • Sclerotic metastasis
Imaging Findings . • Osteoblastoma (OB)
• 4% of all primary OGS occur in spine and sacrum • Aneurysmal bone cyst (ABC)
• 79% arise in posterior elements • Osteomyelitis
• 17% involve 2 adjacent spinal levels • Ewing sarcoma
• 84% invade spinal canal • Chondrosarcoma
• Wide zone of transition • Lymphoma
• Permeative appearance Clinical Issues
• Cortical breakthrough and soft tissue mass • Insidious onset of back pain
• 80% have bone matrix seen on radiographs and/or • Median survival 23 months in recent series
CTscan • Lower survival rate in sacral tumors
• 20% have lytic appearance without visible bone
matrix
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OSTEOSARCOMA
1 Gross Pathologic & Surgical Features
• Lower survival rate in sacral tumors
• Survival rate lower than for peripheral OGS due to
44 • Heterogeneous mass with ossified and non-ossified difficulty of surgical resection
components • Metastases: Bone, lung, liver
• Ossified areas: Yellow-white, firm, may be as hard as • 3% of 10 year survivors of all OGS develop a second
cortical bone malignancy
• Less ossified areas: Soft, tan, with foci of hemorrhage
and necrosis Treatment
• Cortical breakthrough with often large extraosseous • Surgical resection with wide margins
tumor mass • Adjuvant and neoadjuvant chemotherapy
• Necrosis common • Biopsies must be planned with future tumor excision
in mind
Microscopic Features
• Pluripotential neoplasm
• Malignant cells produce some osteoid in all subtypes,
but it may be difficult to find
IDIAGNOSTIC CHECKLIST
• Classic: High degree anaplasia, high mitotic rate Image Interpretation Pearls
• Tumor cells may be spindle or round, size varies from • All telangiectatic OGS are lytic on radiographs and CT,
small to giant but not all lytic OGS are telangiectatic
• Telangiectatic OGS
o Dilated vascular channels lined by multinucleated
giant cells ISELECTED REFERENCES
o Stroma forms osteOid, which may not be a
1. Ilaslan H et al: Primary vertebral osteosarcoma: imaging
prominent feature findings. Radiology. 230(3):697-702, 2004
Staging, Grading or Classification Criteria 2. Brenner W et al: PET imaging of osteosarcoma. J Nucl Med.
44(6):930-42, 2003
• Classified by predominant cell type histologically 3. Ozaki T et al: Osteosarcoma of the spine: experience of the
o Osteoblastic (conventional), chondroblastic, Cooperative Osteosarcoma Study Group. Cancer.
fibroblastic, telangiectatic, small cell, giant cell, 94(4):1069-77,2002
epithelioid 4. Bredella MA et al: Value of FOG PET in conjunction with
o Surface OGS not reported in spine MR imaging for evaluating therapy response in patients
• Surgical staging for malignant musculoskeletal tumors with musculoskeletal sarcomas. AJR Am J Roentgenol.
o Stage Ia: Low grade, intracompartmental 179(5):1145-50,2002
o Stage Ib: Low grade, extracompartmental 5. Aung L et al: Second malignant neoplasms in long-term
survivors of osteosarcoma: Memorial Sloan-Kettering
o Stage lIa: High grade, intracompartmental Cancer Center Experience. Cancer. 95(8):1728-34, 2002
o Stage lIb: High grade, extracompartmental 6. Yamamoto T et al: Sacral radiculopathy secondary to
o Stage IIIa: Low or high grade, intracompartmental, multicentric osteosarcoma. Spine. 26(15):1729-32, 2001
metastases 7. Iwata A et al: Osteosarcoma as a second malignancy after
o Stage IIIb: Low or high grade, extracompartmental, treatment for neuroblastoma. Pediatr Hematol Oncol.
metastases 18(7):465-9,2001
8. Bramwell VH: Osteosarcomas and other cancers of bone.
Curr Opin Oncol. 12(4):330-6,2000
9. Vuillemin-Bodaghi V et al: Multifocal osteogenic sarcoma
ICLINICAL ISSUES in Paget's disease. Skeletal Radiol. 29(6):349-53, 2000
10. Unni KK: Osteosarcoma of bone. J Orthop Sci. 3(5):287-94,
Presentation 1998
• Most common signs/symptoms 11. Rosenberg ZS et al: Osteosarcoma: Subtle, rare, and
o Insidious onset of back pain misleading plain film features. Am J Roentgenol.
o Pain greatest at night 165:1209-14, 1995
o Radicular pain 12. Tigani 0 et al: Vertebral osteosarcoma. Ital J Orthop
o Paraplegia Traumatol. 14(1):5-13, 1988
13. Barwick KW et al: Primary osteogenic sarcoma of the
• Clinical profile vertebral column: a clinicopathologic correlation of ten
o Pathologic fracture patients. Cancer. 46(3):595-604, 1980
o Pulmonary metastases common, can cause
pneumothorax (calcifying)
o Increased serum alkaline phosphatase
Demographics
• Age
o Spine OGS has peak incidence in 4th decade
o Later peak incidence than for appendicular OGS
o Age range 8-80 years in one large series
• Gender: M = F
Natural History & Prognosis
• Median survival 23 months in recent series
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OSTEOSARCOMA
IIMAGE GALLERY 1
45
Typical
(Left) Axial bone CT shows
amorphous ossification in
sacrum and left ilium,
crossing sacroiliac joint. 5T
mass is also densely ossified,
a distinguishing feature of
OC5. (Right) Anteroposterior
specimen radiography shows
resection of OC5 involving
sacrum and ilium. Left 51
neural foramen (arrow) lies
medial to tumor, which
crosses 51 joint (open arrow).
(Left) Anteroposterior
radiography shows OC5
metastatic to spine, with
extensive blastic lesions.
Ossified metastatic foci are
also seen in the liver (arrow).
(Right) Bone scan shows
metastatic OC5 to spine,
ribs, pleural space, and
pelvic lymph nodes from
femoral primary.
Variant
(Left) Anteroposterior
radiography shows lytic OC5
at left transitional vertebra
(curved arrows). Right
pedicle is normal (arrow),
left pedicle is absent (open
arrow). (Right) Axial T1 C+
MR shows telangiectatic
OCS of sacrum (arrows).
Fluid filled nonenhancing
regions are present, as well
as enhancing nodules.
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CHORDOMA
1
46
Sagittal graphic of cervical spine shows extradural soft Sagittal T2WI MR shows hyperintense mass involving
tissue mass with epicenter in posterior. aspect of C2 C3 body with extensive epidural extension and cord
body, causing bone destruction and epidural extension compression.
with cord compression.
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CHORDOMA
Key Facts
1
Terminology • May extend into epidural/perivertebral space, 47
• Malignant tumor arising from notochord remnants compress cord
• May extend along nerve roots, enlarge neural
Imaging Findings foramina
• Mass is hyperintense to discs on T2WI, with multiple • Amorphous intratumoral Ca++
septa
• Histologic identification of physaliphorous cell Pathology
confirms diagnosis • 3 types described
• Location: Sacrococcygeal> spheno-occipital> > • Typical: Lobules, sheets, and cords of clear cells with
vertebral body intracytoplasmic vacuoles (physaliphorous cells);
• Size: Several cm at presentation abundant mucin
• Morphology: Midline lobular soft tissue mass with • Chondroid: Hyaline cartilage (usually
osseous destruction spheno-occipital region)
• May extend into disc, involve 2 or more adjacent • Dedifferentiated: Sarcomatous elements (rare, highly
vertebrae malignant)
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CHORDOMA
1 Demographics
45(4):427-34, 2001
10. Arnautovic KI et al: Surgical seeding of chordomas. ]
48 • Age: Peak incidence 5th-6th decades (rare in children) Neurosurg. 95(5):798-803, 2001
• Gender: M:F = 2:1 spine (no gender predilection in 11. Scheil S et al: Genome-wide analysis of sixteen chordomas
sacral chordoma) by comparative genomic hybridization and cytogenetics of
• Ethnicity: Rare in African-Americans the first human chordoma cell line, U-CH1. Genes
Chromosomes Cancer. 32(3):203-11, 2001
Natural History & Prognosis 12. Bosma]] et al: En bloc removal of the lower lumbar
vertebral body for chordoma. Report of two cases. ]
• Slow-growing Neurosurg. 94(2 Suppl):284-91, 2001
• Distant metastases 5-40% (lung, liver, lymph nodes, 13. Crapanzano]P et al: Chordoma: a cytologic study with
bone) histologic and radiologic correlation. Cancer. 93(1):40-51,
• Poor prognostic factors 2001
o Large size 14. McMaster ML et al: Chordoma: incidence and survival
o Subtotal resection, local recurrence patterns in the United States, 1973-1995. Cancer Causes
o Microscopic necrosis Control. 12(1):1-11, 2001
o Ki-67 index> 5% 15. Bergh P et al: Prognostic factors in chordoma of the sacrum
and mobile spine: a study of 39 patients. Cancer.
• 5 year survival up to 67-84%
88:2122-34, 2000
• 10 year survival 40% 16. Tai PT et al: Management issues in chordoma: a case series.
Treatment Clin Oncol (R ColI Radiol). 12(2):80-6, 2000
17. Gelabert-Gonzalez M et al: Intradural cervical chordoma.
• Surgical resection with adjuvant XRT Case report.] Neurosurg Sci. 43(2):159-62, 1999
o En bloc resection yields best outcome 18. Cheng EY et al: Lumbosacral chordoma. Prognostic factors
• Recurrence common and treatment. Spine. 24(16):1639-45, 1999
o Local (90%) 19. Wippold F] 2nd et al: Clinical and imaging features of
o Regional lymph nodes (5%) cervical chordoma. A]R Am] Roentgenol. 172(5):1423-6,
o Distant metastases (5%); lung, bone 1999
o 5 year survival after relapse (5-7%) 20. York]E et al: Sacral chordoma: 40-year experience at a
major cancer center. Neurosurgery. 44(1):74-9; discussion
79-80,1999
21. Logroscino CA et al: Chordoma: long-term evaluation of
IDIAGNOSTIC CHECKLIST 15 cases treated surgically. Chir Organi Mov.
83(1-2):87-103, 1998
Consider 22. Ng SH et al: Cervical ecchordosis physaliphora: CT and MR
• High signal intensity mass on T2WI with septations, features. Br] Radiol. 71(843):329-31, 1998
little enhancement is chordoma/chondroid tumor 23. Murphy]M et al: CT and MRI appearances of a thoracic
chordoma. Eur Radiol. 8(9):1677-9, 1998
Image Interpretation Pearls 24. Boriani S et al: Primary bone tumors of the spine.
• Recurrence (seeding) along operative tract is not Terminology and surgical staging. Spine. 22(9):1036-44,
uncommon, modify field-of-view to include operative 1997
25. Catton C et al: Chordoma: long-term follow-up after
approach
radical photon irradiation. Radiother Oncol. 41(1):67-72,
1996
26. Murphey MD et al: From the archives of the AFIP. Primary
ISELECTED REFERENCES tumors of the spine: radiologic pathologic correlation.
1. Smolders D et al: Value of MRI in the diagnosis of Radiographics. 16(5):1131-58, 1996
non-clival, non-sacral chordoma. Skeletal Radiol. 27. Boriani S et al: Chordoma of the spine above the sacrum.
32(6):343~50, 2003
Treatment and outcome in 21 cases. Spine. 21(13):1569-77,
2. Noel G et al: Radiation therapy for chordoma and 1996
chondrosarcoma of the skull base and the cervical spine. 28. Klekamp] et al: Spinal chordomas--results of treatment
Prognostic factors and patterns of failure. Strahlenther over a 17-year period. Acta Neurochir (Wien). 138(5):514-9,
Onkol. 179(4):241-8, 2003 1996
3. Baratti D et al: Chordoma: natural history and results in 28 29. Fagundes MA et al: Radiation therapy for chordomas of the
patients treated 'at a single institution. Ann Surg Oncol. base of skull and cervical spine: patterns of failure and
10(3):291-6, 2003 outcome after relapse. Int] Radiat Oncol BioI Phys.
4. Leone A et al: Chordoma of the low cervical spine 33(3):579-84, 1995
presenting with Horner's syndrome. Eur Radiol. 12 Suppl 30. Hall WA et al: Sacrococcygeal chordoma metastatic to the
3:S43-7, 2002 brain with review of the literature. ] Neurooncol.
5. Bayar MA et al: Spinal chordoma of the terminal filum. 25(2):155-9, 1995
Case report. ] Neurosurg. 96(2 Suppl):236-8, 2002 31. Wojno K] et al: Chondroid chordomas and low-grade
6. Carpentier A et al: Suboccipital and cervical chordomas: chondrosarcomas of the craniospinal axis. An
the value of aggressive treatment at first presentation of immunohistochemical analysis of 17 cases. Am] Surg
the disease.] Neurosurg. 97(5):1070-7, 2002 Pathol. 16(12):1144-52, 1992
7. Steenberghs] et al: Intradural chordoma without bone
involvement. Case report and review of the literature. ]
Neurosurg. 97(1 Suppl):94-7, 2002
8. Delank KS et al: Metastasizing chordoma of the lumbar
spine. Eur Spine]. 11(2):167-71,2002
9. Soo MY: Chordoma: review of clinicoradiological features
and factors affecting survival. Australas Radiol.
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CHORDOMA
IIMAGE GALLERY 1
49
Variant
(Left) Sagittal TlWI MR
shows large atypically
hyperintense mass involving
clivus and extending into
C/-2junction. Tumor mass
effect compresses medulla
and cervical cord. (Right)
Axial T2WI MR shows typical
high signal mass with
multiple low signal
septations. There are
bilateral tonsillar infarcts
from mass effect (arrows).
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EWING SARCOMA
1
50
Sagittal graphic shows vertebral body replaced by Axial bone CT shows ill-defined sacral Ewing sarcoma
Ewing sarcoma, resulting in mild collapse. Tumor (open arrows). Cortex anteriorly is irregular, with tumor
extends into adjacent soft tissues through small extending through cortical perforaUons into soft tissues
perforations in bone cortex. (curved arrows).
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EWING SARCOMA
Key Facts
1
Terminology • Osteomyelitis 51
• Round cell sarcoma of bone • Osteogenic sarcoma (OGS)
• Other primary sarcomas and metastatic disease
Imaging Findings
• Centered in vertebral body or sacrum
Pathology
• Permeative/moth eaten bone destruction • Primitive neuroectodermal tumor (PNET) is closely
• Wide zone of transition related tumor
• Tiny perforations of cortex rather than extensive loss • 6th most common malignant bone tumor
or cortical bone radiographically Clinical Issues
• 50% have extraosseous, noncalcified, soft tissue mass • Localized pain
• MRI best shows involvement of adjacent bones & soft • Fever, leukocytosis, elevated ESR (simulating
tissues, which can be underestimated on CT scan osteomyelitis)
Top Differential Diagnoses • Prognosis worse in spinal than peripheral Ewing
• Primitive neuroectodermal tumor (PNET) sarcoma because of difficulty of surgical resection
• Langerhans cell histiocytosis • Significant risk of second malignancy
• Other small round cell tumors
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EWING SARCOMA
1 o Annual incidence of Ewing sarcoma all locations:
3/1,000,000 Caucasian children < 15 years old
I DIAGNOSTIC CHECKLIST
52 o 6th most common malignant bone tumor Image Interpretation Pearls
Gross Pathologic & Surgical Features • Always consider osteomyelitis and other small round
• Grayish white tumor cell tumors in imaging differential diagnosis
• Poorly demarcated
• Areas of hemorrhage, cyst formation, necrosis
ISELECTED REFERENCES
Microscopic Features 1. Bacci G et al: Long-term outcome for patients with
• Small, round cells (2-3 times larger than lymphocytes), non-metastatic Ewing's sarcoma treated with adjuvant and
meager cytoplasm neoadjuvant chemotherapies. 402 patients treated at
• Cell outlines indistinct Rizzoli between 1972 and 1992. Eur J Cancer. 40(1):73-83,
• Round nuclei, frequent indentations, high mitotic rate 2004
• Solid sheets of cells divided into irregular masses by 2. Caksen H et al: A case of metastatic spinal Ewing's sarcoma
misdiagnosed as brucellosis and transverse myelitis. Neurol
fibrous strands
Sci. 24(6):414-6, 2004
• Features which distinguish PNET from Ewing sarcoma 3. Fuchs B et al: Complications in long-term survivors of
o PNET forms rosettes of cells Ewing sarcoma. Cancer. 98(12):2687-92, 2003
o PNET is positive for 2 or more neuroectodermal 4. Burchill SA: Ewing's sarcoma: diagnostic, prognostic, and
markers therapeutic implications of molecular abnormalities. J Clin
o Distinction most reliably made with Fluorescence in Pathol. 56(2):96-102, 2003
Situ Hybridization (FISH) 5. Bacci G et al: Therapy and survival after recurrence of
Ewing's tumors: the Rizzoli experience in 195 patients
treated with adjuvant and neoadjuvant chemotherapy
from 1979 to 1997. Ann Oncol. 14(11):1654-9,2003
ICLINICAL ISSUES 6. Fuchs B et al: Ewing's sarcoma and the development of
secondary malignancies. Clin Orthop. (415):82-9, 2003
Presentation 7. Hawkins DS et al: Evaluation of chemotherapy response in
• .Most common signs/symptoms pediatric bone sarcomas by [F-18]-fluorodeoxy-D-glucose
o Localized pain positron emission tomography. Cancer. 94:3277-84, 2002
o Fever, leukocytosis, elevated ESR (simulating 8. Goktepe AS et al: Paraplegia: an unusual presentation of
osteomyelitis) Ewing's sarcoma. Spinal Cord. 40(7):367-9, 2002
o Vertebra plana 9. Hoffer FA: Primary skeletal neoplasms: osteosarcoma and
ewing sarcoma. Top Magn Reson Imaging. 13(4):231-9,
o Neurologic symptoms ranging from radiculopathy
2002
to paralysis 10. Paulussen M et al: Ewing tumour: incidence, prognosis and
Demographics treatment options. Paediatr Drugs. 3(12):899-913, 2001
11. Devoe K et al: Immunohistochemistry of small round-cell
• Age tumors. Semin Diagn Pathol. 17:216-24,2000
o 90% of all Ewing sarcoma patients present before 20 12. Llombart-Bosch A et al: Histology, immunohistochemistry,
years and electron microscopy of small round cell tumors of
o Second (smaller) peak at age 50 years bone. Semin Diagn Pathol. 13:153-70, 1996
o Spine and sacral lesions often in older patients than 13. Eggli KD et al: Ewing'S sarcoma. Radiol Clin North Am.
peripheral Ewing sarcoma 31:325-37, 1993
14. Downing JR et al: Detection of the (11;22)(q24;qI2)
• Gender: M:F = 2: 1
translocation of Ewing's sarcoma and peripheral
Natural History & Prognosis neuroectodermal tumor by reverse transcription
polymerase chain reaction. Am J Pathol. 143: 1294-300,
• Metastases to lung, regional lymph nodes, and other
1993
bones in 30% at presentation IS. Boyko OB et al: MR imaging of osteogeniC and Ewing's
• Prognosis worse in spinal than peripheral Ewing sarcoma. AmJ Roentgenol. 148:317-22, 1987
sarcoma because of difficulty of surgical resection
• Significant risk of second malignancy
• Current treatments yield long term survival> 50% of
patients with localized disease at presentation
• Complications common> 5 years after treatment
o Local recurrence
o Metastases
o Treatment complications
o Second malignancies
Treatment
• Surgery or radiotherapy; without chemotherapy
universally fatal
• Neoadjuvant chemotherapy given prior to surgery
• Surgical resection with wide margins
• Radiation therapy for surgically inaccessible lesions,
stage III disease, poor response to chemotherapy
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EWING SARCOMA
IIMAGE GALLERY 1
53
Typical
(Left) Axial STIR MR shows
sacral Ewing sarcoma with
heterogeneous signal
intensity mass (arrows)
extending through cortical
perforations into presacral
50ft tissues. Patient presented
with radieulopathy. (Right)
Axial T7 C+ MR shows sacral
Ewing sarcoma containing
areas of necrosis (arrows)
within enhancing mass. Note
extension of tumor into left
51 neural foramen (open
arrow).
Typical
(Left) Axial T2WI MR shows
metastatic Ewing sarcoma of
thoracic vertebra, spinal
canal, adjacent rib, and
thoracic cavity. Note cortical
thinning and indistinctness in
areas of tumor permeation
(arrow). (Right) Axial T1 C+
MR shows metastatic Ewing
sarcoma of thoracic vertebra,
spinal canal, adjacent rib,
and thoracic cavity. There is
moderate, heterogeneous
enhancement.
Variant
(Left) Axial bone CT shows
sclerotic Ewing sarcoma of
sacrum (curved arrow) and
ilium (open arrow). Sacral
involvement was better seen
on subsequent MR. Tumor
extends into iliacus muscle
(arrow). (Right) Coronal
STIR MR shows Ewing tumor
(arrows) in sacrum,
paraspinous 50ft tissues, and
along right sciatic nerve. MR
appearance is typical, but
lesion showed atypical
sclerosis on CT scan.
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LYMPHOMA
1
54
Sagittal T2WI MR demonstIates a focal lymphoma Axial T1 C+ MR shows extensive epidural lymphoma
deposit in the spinal epidural space. Note hypointense also involving adjacent stIuctures (paraspinal muscles,
dura interposed between tumor and cord (arrow). pleura, rib).
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LYMPHOMA
Key Facts
1
Terminology Pathology 55
• Lymphoreticular neoplasms with wide variety of • Exact causes are unknown
specific diseases & cellular differentiation • NHL > > Hodgkin disease (HD); 80-90% are B cell
• CNS lymphoma> 85% NHL (B cell> > > T cell)
Imaging Findings • Hodgkin disease rare
• Epidural: Enhancing epidural mass +/- vertebral
involvement Clinical Issues
• Osseous: Bone destruction ("ivory" vertebra, rare) • Most common presenting symptom = back pain
• Leptomeningitic: Smooth/nodular pial enhancement • Intramedullary = myelopathy (weakness, numbness)
• Intramedullary: Poorly-defined enhancing mass • Generally poor prognosis for CNS lymphoma
• Best imaging tool: MRI + C • Best prognosis with primary osseous lymphoma
• XRT +/- chemotherapy
Top Differential Diagnoses
• Epidural disease that may mimic epidural lymphoma Diagnostic Checklist
• Osseous lymphoma mimics • Lymphoma is a great mimicker
• Lymphomatous meningitis mimics
• Intramedullary lymphoma mimics
o Epidural: Isointense homogeneous epidural mass o Discriminates between patients who achieve
(often multisegmental +/- extends through complete response & those who show no or only
foramina) partial response to induction therapy
o Osseous: Hypointense to normal marrow (+/- o Prognosis can be predicted during treatment, as
epidural extension) soon as after 1 cycle of chemotherapy
o Leptomeningitic: Thick nerve roots +/- focal nodules o Recurrent disease can be diagnosed early during
(isointense with cord) follow-up
o Intramedullary: Mass usually isointense to spinal
cord Imaging Recommendations
• T2WI • Best imaging tool: MRI + C
o Epidural: Iso/hyperintense to cord • Protocol advice
o Osseous: Variable; iso/hyperintense o Fat-saturated TlWI
o Leptomeningitic: Thick nerve roots +/- focal nodules o STIR may be helpful
(isointense with cord)
o Intramedullary: Hyperintense with surrounding
edema IDIFFERENTIAL DIAGNOSIS
• STIR: Osseous: May show T2 characteristics better
Epidural disease that may mimic epidural
• Tl C+
o Epidural: Enhances intensely, uniformly lymphoma
o Osseous: Diffuse uniform enhancement • Hematoma: Heterogeneous> homogeneous signal
o Leptomeningitic: Roots enhance • Abscess: Rim> solid enhancement, central low signal
o Intramedullary: Variable: Patchy/confluent, common
infiltrating/discrete • Metastasis: Epidural met without bone involvement
• Dynamic contrast-enhanced MRI rare
o Demonstrates 1 bone marrow enhancement in
patients with lymphoproliferative diseases with Osseous lymphoma mimics
diagnostic accuracy of 99% • Metastasis: Destructive, +/- soft tissue mass
o Assess degree of diffuse bone marrow infiltration • Eosinophilic granuloma: Vertebra plana; younger
o j After treatment in all patients who respond to patients
treatment; but not in 2/3 patients who did not lymphomatous meningitis mimics
respond to treatment • Other neoplastic/granulomatous or infectious
Nuclear Medicine Findings meningitides
• Bone Scan: Increased uptake Intramedullary lymphoma mimics
• PET: Fluorine··18 fluorodeoxyglucose (FDG) PET has • Ependymoma: Hemorrhage, cysts common
proven useful for staging, monitoring treatment • Astrocytoma: Multisegmental; cysts common
response, predicting treatment outcomes, & risk • Metastasis: Usually round, more sharply delineated
stratifying lymphoma patients
• Gallium scan
o 67Ga scintigraphy has a high sensitivity & IPATHOLOGY
specificity for diagnosis of bone lymphoma
General Features
• Etiology
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LYMPHOMA
1 o Exact causes are unknown
o CNS lymphoma may be primary or secondary
• Best prognosis with primary osseous lymphoma
o 5-10 yr survival rates == 91-87%
56 (hematogenous or direct geographic extension)
o Risk factors Treatment
• Chemical exposure such as pesticides, fertilizers, • XRT +/- chemotherapy
or solvents o Markedly sensitive to chemotherapy/XRT
• Epstein-Barr virus plays role in o Intrathecal for leptomeningitic
immunocompromised • +/- Surgery
• Infection with HTLV-l
• AIDS/transplant immunocompromised patients
• Family history of NHL, although no hereditary I DIAGNOSTIC CHECKLIST
pattern has been established
Consider
• Epidemiology
o NHL > > Hodgkin disease (HD); 80-90% are B cell • Lymphoma is a great mimicker
• CNS lymphoma> 85% NHL (B cell> > > T cell)
• Hodgkin disease rare
o Primary epidural == 1-7% of NHL; 10-30% of epidural ISELECTED REFERENCES
malignancies 1. Westin EH et al: Lymphoma and myeloma in older
o Secondary epidural in 5% of patients with systemic patients. Semin Oncol. 31(2):198-205, 2004
lymphoma 2. Rahmouni A et al: Bone marrow with diffuse tumor
infiltration in patients with lymphoproliferative diseases:
o Primary osseous == 3-4% of malignant bone tumors
dynamic gadolinium-enhanced MR imaging. Radiology.
o Bone marrow involvement in 25-50% NHL patients, 229(3):710-7, 2003
5-15% HD 3. Israel 0 et al: Bone lymphoma: 67Ga scintigraphy and CT
o Epidural/vertebral involvement related to for prediction of outcome after treatment. J Nucl Med.
hematogenous metastatic involvement or local 43(10):1295-303, 2002
spread from adjacent lymph nodes 4. Vazquez E et al: Neuroimaging in pediatric leukemia and
o Intramedullary == 3% of CNS lymphoma lymphoma: differential diagnosis. Radiographies.
o Leptomeningitic nearly always occurs as spread from 22(6):1411-28, 2002
5. Guermazi A et al: Extranodal Hodgkin disease: spectrum of
intracranial lymphoma disease. Radiographies. 21(1):161-79, 2001
o Most common malignancy of the epidural space 6. Koeller KK et al: Neoplasms of the spinal cord and filum
o Lymphoma represents an increasingly important terminale: Radiologic-Pathologic correlation.
cause of morbidity & mortality in older patients RadioGraphies. 20:1721-49, 2000
• Associated abnormalities: Multisystem/multiorgan 7. Mulligan ME et al: Imaging features of primary lymphoma
involvement of bone. AJR. 173:1691-7, 1999
8. White LM et al: MR imaging of primary lymphoma of
Gross Pathologic & Surgical Features bone: variability of T2-weighted signal intensity. AJR Am J
• Varies from discrete mass to poorly-marginated Roentgenol. 170(5):1243-7, 1998
infiltrative disease 9. Caruso PA et al: Primary intramedullary lymphoma of the
spinal cord mimicking cervical spondylotic myelopathy.
Microscopic Features AJR Am J Roentgenol. 171(2):526-7, 1998
• Neoplastic lymphocytes in marrow, meninges 10. Moulopoulos LA et al: Magnetic resonance imaging of the
bone marrow in hematologic malignancies. Blood.
• Pack perivascular spaces 90(6):2127-47, 1997
Staging, Grading or Classification Criteria 11. Boukobza M et al: Primary vertebral and spinal epidural
non-Hodgkin's lymphoma with spinal cord compression.
• NHL: Ann Arbor staging system Neuroradiology. 38:333-7, 1996
• Hodgkin: Cotswold system (modified Ann Arbor 12. Yankelevitz DF et al: Effect of radiation therapy on thoracic
staging system) and lumbar bone marrow: evaluation with MR imaging.
AJR AmJ Roentgenol. 157(1):87-92, 1991
13. Lim V et al: Spinal cord pial metastases: MR imaging with
ICLINICAL ISSUES gadopentetate dimeglumine. AJR Am J Roentgenol.
155(5):1077-84, 1990
Presentation 14. Lim V et al: Spinal cord pial metastases: MR imaging with
gadopentetate dimeglumine. AJNR AmJ Neuroradiol.
• Most common signs/symptoms
11(5):975-82, 1990
o Most common presenting symptom == back pain 15. Beres Jet al: Spinal epidural lymphomas: CT features in
o Intramedullary == myelopathy (weakness, numbness) seven patients. AJNR Am J Neuroradiol. 7(2):327-8, 1986
Demographics
• Age: Adults; peak == 4th-7th decade
• Gender: Slight male predominance
• Ethnicity: All affected
Natural History & Prognosis
• Cord compression occurs in up to 5-10% of systemic
lymphomas
• Generally poor prognosis for CNS lymphoma
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LYMPHOMA
IIMAGE GAllERY 1
57
(Left) Sagittal TI C+ MR
demonstrates ill-defined
intramedullary enhancing
lymphoma (arrow). (Right)
Axial T2WI MR reveals
hyperintensity of
intramedullary lymphoma.
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LEUKEMIA
1
58
Sagittal T7WI MR in CML patient demonstrates marked Sagittal T7 C+ MR depicts avid diffuse marrow
diffuse marrow hypointensity relative to adjacent discs. enhancement refleCting CML marrow infiltration.
Incidental disc herniation (arrow) was etiology of neck Normal marrow would be expected to minimally
pain prompting imaging. enhance.
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LEUKEMIA
Key Facts
1
Terminology • Ewing sarcoma 59
• Acute or chronic myeloid or lymphoid white blood Pathology
cell neoplasia with spinal involvement as component • Strong association with chromosomal abnormalities
of systemic disease
Clinical Issues
Imaging Findings • Chronic leukemia may be asymptomatic
• Best diagnostic clue: Diffuse osteopenia with multiple • Symptomatic patients present with fever, t ESR,
vertebral fractures +/- lytic spine lesions hepatosplenomegaly, lymphadenopathy, joint
• Radiographs may look normal, even if extensive effusions, petechial and retinal hemorrhage, anemia,
disease frequent infections
• Granulocytic sarcoma ~ focal lytic mass
Diagnostic Checklist
Top Differential Diagnoses • MRI sensitivity for leptomeningeal metastases < <
• Metastases laboratory CSF evaluation
• Langerhans cell histiocytosis (LCH) • Consider leukemia in a patient with unexplained
• Osteomyelitis compression fractures
• Lymphoma
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LEUKEMIA
1 • Associated abnormalities
IDIAGNOSTIC CHECKLIST
o Long bone periostitis (12-25%), "leukemic"
60 metaphyseal lines Consider
o Focal destruction of flat/tubular bones, pathologic • Marrow infiltration in child with osteoporosis raises
fractures suspicion for leukemia
o Opportunistic infections, second malignancies • MRI sensitivity for leptomeningeal metastases < <
o Chemotherapy-related complications laboratory CSF evaluation
Gross Pathologic & Surgical Features Image Interpretation Pearls
• Hyperemic/hemorrhagic bone marrow with • Consider leukemia in a patient with unexplained
destruction of bony trabeculae, bone infarction compression fractures
Microscopic Features
• Diffuse bone marrow infiltration by
poorly-differentiated hematologic cells ISELECTED REFERENCES
o ALL: Infiltrates of small blue cells 1. Kuhn J et al: Caffey's Pediatric Diagnostic Imaging. 10th
o AML: Auer rods (condensed lysosomal cytoplasmic ed. Philadelphia, Mosby. Pages 710-711, 2004
rod shaped structures) diagnostic 2. Laffan EE et al: Whole-body magnetic resonance imaging: a
o CLL: Mature lymphocytes, < 55% atypical cells useful additional sequence in paediatric imaging. Pediatr
Radiol. 34(6):472-80, 2004
o CML: Leukocytosis with increase in basophils, 3. Chang YW et al: Spinal MRI of vincristine neuropathy
eosinophils, neutrophils; Philadelphia chromosome mimicking Guillain-Barre syndrome. Pediatr Radiol.
t(9;22) 33(11):791-3, 2003
4. Beckers R et al: Acute lymphoblastic leukaemia presenting
with low back pain. Eur J Paediatr Neurol. 6(5):285-7,2002
ICLINICAL ISSUES 5. Vazquez E et al: Neuroimaging in pediatric leukemia and
lymphoma: differential diagnosis. Radiographies.
Presentation 22(6):1411-28,2002
• Most common signs/symptoms 6. Buckland ME et al: Spinal chloroma presenting with
o Localized or diffuse bone pain triplegia in an aleukaemic patient. Pathology. 33(3):386-9,
2001
o Recurrent para-articular arthralgias (75%) 7. Carriere B et al: Vertebral fractures as initial signs for acute
• Clinical profile lymphoblastic leukemia. Pediatr Emerg Care. 17(4):258-61,
o Chronic leukemia may be asymptomatic 2001
o Symptomatic patients present with fever, 1 ESR, 8. Shalaby-Rana E et al: (99m)Tc-MDP scintigraphic findings
hepatosplenomegaly, lymphadenopathy, joint in children with leukemia: value of early and delayed
effusions, petechial and retinal hemorrhage, anemia, whole-body imaging. J Nucl Med. 42(6):878-83, 2001
frequent infections 9. Yavuz H et al: Transverse myelopathy: an initial
presentation of acute leukemia. Pediatr Neurol.
Demographics 24(5):382-4, 2001
10. Lo LD et al: Are L5 fractures an indicator of metastasis?
• Age Skeletal Radiol. 29(8):454-8, 2000
o ALL: Peak 2-10 y 11. Kim HJ et al: Spinal involvement of hematopoietic
o AML: Peak> 65 y malignancies and metastasis: differentiation using MR
o CML: Rare in childhood « 5%), peak> 40 y imaging. Clin Imaging. 23(2):125-33, 1999
o CLL: Peak 50-70 y 12. Sandhu GS et al: Granulocytic sarcoma presenting as cauda
• Gender: M:F = 2:1 equina syndrome. Clin Neurol Neurosurg. 100(3):205-8,
1998
Natural History & Prognosis 13. Aisenberg J et al: Bone mineral density in young adult
• 5 year survival (all leukemias): 25-30% survivors of childhood cancer. J Pediatr HematoI Oncol.
o Children with ALL: 90% complete remission, 80% 5 20(3):241-5, 1998
year disease free survival 14. Novick SL et al: Granulocytic sarcoma (chloroma) of the
sacrum: initial manifestation of leukemia. Skeletal Radiol.
o Adults with ALL: Remission in 60-80%, 20-30% 5 27(2):112-4, 1998
year disease free survival 15. Deme S et al: Granulocytic sarcoma of the spine in
o AML: 45% 5 year survival nonleukemic patients: report of three cases. Neurosurgery.
o CLL: 6 years median survival 40(6):1283-7, 1997
o CML: 5 years median survival 16. Wu CY et al: Detection of dural involvement by magnetic
resonance imaging in adult patients with acute
Treatment leukemias--preliminary experience. Ann Hematol.
• Chemotherapy 70(5):243-9, 1995
o Induction phase, consolidation phase, maintenance 17. Heinrich SD et al: The prognostic significance of the
therapy phase skeletal manifestations of acute lymphoblastic leukemia of
o Intrathecal chemotherapy for CNS involvement childhood. J Pediatr Orthop. 14(1):105-11, 1994
• Radiation therapy
• Bone marrow transplant
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LEUKEMIA
I IMAGE GALLERY 1
61
(Left) Sagittal T7 WI MR
depicts diffuse abnormal
marrow replacement
characterized by marrow
signal hypointense relative to
adjacent discs. (Right)
Lateral radiography shows
osteopenia only in a
pediatric patient with
systemic leukemia.
Variant
(Left) Sagittal Tl C+ MR with
fat-saturation in a
symptomatic AML patient
shows extensive
leptomeningeal neoplastic
enhancement replacing
normal hypointense CSF
signal. (Right) Axial T2WI
MR with fat-saturation of the
T7 0 vertebra shows a
hyperintense focal leukemic
metastasis extending into the
left pedicle, without epidural
extension (Courtesy R.
Boyer, MOJ.
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PLASMACYTOMA
1
62
Sagittal graphic shows collapse of thoracic vertebral Sagittal STIR MR shows C7 severe collapse (vertebra
body due to tumor infiltration with retropulsion of plana) with posterior bony retropulsion and cord
anterior & posterior margins. Dorsal retropulsion & compression. There is no associated soft tissue mass.
tumor causes cord compression.
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PLASMACYTOMA
Key Facts
1
Terminology • Benign (osteoporotic) compression fracture 63
• Solitary monoclonal plasma cell tumor of bone or • Vertebral hemangioma (VH)
soft tissue, with no evidence of multiple myeloma Pathology
(MM) elsewhere • Most patients SBP reflects early (stage I) multiple
Imaging Findings myeloma (MM), the malignant plasma cell disorder
• Best diagnostic clue: TI hypointense marrow with Clinical Issues
low-signal, curvilinear areas • Age: Mean age = 55 years (younger than age of
• Location: Vertebral body = most common site of SBP patients with MM)
• Bone scintigraphy unreliable for staging and • SBPs typically have indolent course (median survival
monitoring = 10 years)
• FDG-PET reliably detects active MM • Definitive local radiotherapy (4,000 cGy)
• Standard MR + STIR, scan entire spine
Diagnostic Checklist
Top Differential Diagnoses • Caution: Must exclude second unanticipated lesion
• Multiple myeloma (33% of cases)
• Metastasis
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PLASMACYTOMA
1 • Monosomy 13 correlated with poor prognosis,
especially combined with elevated ~2
• Some asymptomatic patients with stage I MM not
treated until more aggressive disease demonstrated at
64 microglobulin, concomitant chromosome 11 clinical follow-up
aberration
• Etiology
o Most patients SBP reflects early (stage I) multiple IDIAGNOSTIC CHECKLIST
myeloma (MM), the malignant plasma cell disorder
o Some patients stable for decades, suggesting Consider
relationship to MGUS • Caution: Must exclude second unanticipated lesion
• Epidemiology (33% of cases)
o Solitary bone plasmacytic lesions represent 3-5% of
monoclonal gammopathies
Image Interpretation Pearls
o Spine is most common site • Classic imaging appearance = hypointense vertebra
o 20% have solitary lesions in rib, sternum, clavicle, (Tl WI) with cortical "infoldings," curvilinear low
scapula signal areas
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PLASMACYTOMA
IIMAGE GALLERY 1
65
(Left) Sagittal T7 WI M R
shows severe collapse at C7
level with well-defined
margins. There is
retropulsion of posterior
vertebral body with cord
compression (arrow). (Right)
Sagittal T7 C+ MR shows
severe collapse of central
portion of C7 body with
diffuse enhancement of
ventral and dorsal
retropulsed segments. There
is cord compression due to
posterior retropulsion.
Typical
(Left) Axial T7 C+ MR shows
plasmacytoma involving C7
body with diffuse axial
expansion (open arrows),
and retropulsion of posterior
vertebral body compressing
thecal sac and cord (arrow).
(Right) Sagittal T2WI MR
shows collapse/anterior
wedging deformity of lower
thoracic vertebral body,
showing nonspecific mild
signal heterogeneity. Mild
posterior retropulsion effaces
ventral thecal sac.
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MULTIPLE MYELOMA
1
66
Sagittal T1 C+ MR of lumbar spine shows innumerable Sagittal T2WI MR shows diffuse marrow hypointensity
small enhancing vertebral myelomatous lesions. with severe compression fracture at U. Fractures also
involve superior endpla tes ofT12, L4, and LS.
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MULTIPLE MYELOMA
Key Facts
1
Terminology • MRI or MDCT more sensitive than plain films or 67
• Multifocal malignant proliferation of monoclonal bone scintigraphy in MM staging
plasma cells within bone marrow Top Differential Diagnoses
Imaging Findings • Metastases
• Best diagnostic clue: Multi-focal, diffuse, or • Osteoporosis
heterogeneous T1 hypointensity Pathology
• Diffuse osteopenia: 85% • Most common primary tumor of bone
• Multiple lytic lesions: 80% • Incidence: 3-4 per 100,000 persons per year
• Focal marrow involvement
• Diffuse marrow involvement Clinical Issues
• Variegated pattern • Bone pain: 75%
• 67% of vertebral fractures appear benign • 20% asymptomatic at presentation
• Bone scintigraphy detects 10% of lesions • Median survival rate of 3-5 years with chemotherapy
• MM lesions metabolically active
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MULTIPLE MYELOMA
1 • 27% of biopsied bone tumors
o Incidence: 3-4 per 100,000 persons per year Treatment
68 • Supportive care
Gross Pathologic & Surgical Features o Analgesia
• Confluent or well-circumscribed, red-gray, soft tumor o Osteoclast inhibiting agents: Bisphosphates
replacing cancellous bone o Erythropoietin
o Vertebroplasty
Microscopic Features • Local radiation
• Aggregates of neoplastic plasma' cells • Chemotherapy
o Infiltration and replacement of normal marrow o Melphalan and prednisone
• Myeloma cells o Thalidomide
o Eccentric, round, hyperchromatic nuclei with • Transplants
"cartwheel" distribution of chromatin o Autologous hematopoietic stem cell: Patients < 78
Staging, Grading or Classification Criteria years old with newly diagnosed MM
o Allogeneic bone marrow: Treatment related
• Durie-Salmon staging system: Based on Hgb and serum
mortality up to 50%
calcium levels, monoclonal protein burden, and
number of bone lesions
o Stage I: Low tumor mass
o Stage II: Intermediate tumor mass I DIAGNOSTIC CHECKLIST
o Stage III: High tumor mass Consider
o Each stage divided into A (serum creatinine < 2
• MM when multi-focal, diffuse, or heterogeneous Tl
mg/dl) or B (serum creatinine> 2 mg/dl) marrow hypointensity present
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MULTIPLE MYELOMA
I IMAGE GALLERY 1
69
Typical
(Left) Axial TI C+ MR with
fat suppression through
thoracic spine shows
multiple small enhancing
vertebral and rib lesions. A
larger right rib lesion is
partially visible (arrow).
(Right) Coronal PET with
FOG-18 demonstrates the
same metabolically active
right rib lesion.
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NEUROBLASTIC TUMOR
1
70
Coronal graphic shows a vascular paraspinal NB Coronal T7 C+ MR shows an enhancing left paraspinal
originating on the right, with spread through contiguous NB with intraspinal spread through the neural foramina,
neural foramina across midline to the left (stage III). causing mild cord displacement (Courtesy C. Hedlund,
00).
ITERMINOlOGY CT Findings
• CECT: Enhancing paraspinal mass +/- epidural
Abbreviations and Synonyms extension, finely stippled calcifications
• Neuroblastic tumors (NT) = ganglioneuroma (GN),
• Bone CT
ganglioneuroblastoma (GNB), and neuroblastoma (NB) o Widened neural foramina & intercostal spaces,
Definitions pedicle erosion, adjacent rib splaying (GN, GNB) or
• Embryonal tumors derived from neural crest cells destruction (NB)
MR Findings
• TlWI
IIMAGING FINDINGS o Hypointense ~ isointense paraspinal mass +/-
General Features epidural extension through neural foramina
o +/- Hypointense marrow replacement
• Best diagnostic clue: Abdominal or thoracic paraspinal
mass +/- intraspinal extension, calcification • T2WI: Hypointense ~ hyperintense paraspinal mass
+/- epidural extension, spinal cord compression
• Location: Abdominal (40% adrenal, 25% paraspinal
• Tl C+: Variable enhancement +/- internal
ganglia) > thoracic (15%) >, pelvic (5%) > cervical
hemorrhage, necrosis
(3%); miscellaneous (12%)
• Size: Variable: 1-10 cm diameter Ultrasonographic Findings
• Morphology: Marrow replacement, "dumbbell" • Real Time: Mixed echogenicity paraspinal mass;
paraspinal-intraspinal tumor calcifications ~ posterior acoustic shadowing
Radiographic Findings Nuclear Medicine Findings
• Radiography • Bone Scan: 99mTc MDP uptake in osseous metastatic
o Widened paraspinal soft tissues +/- scoliosis lesions
o +/- Stippled abdominal or mediastinal calcifications • PET: Avid FDG uptake
• MIBG (metaiodobenzylguanidine)
o Uptake by sympathetic catecholaminergic cells
~
.
., ..
...........
-
.. ~
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NEUROBLASTIC TUMOR
Key Facts
1
Terminology • Lymphoma 71
• Neuroblastic tumors (NT) = ganglioneuroma (GN), • Wilms tumor
ganglioneuroblastoma (GNB), and neuroblastoma Pathology
(NB) • Arise from primordial neural crest cell derivatives
Imaging Findings along sympathetic chain
• Best diagnostic clue: Abdominal or thoracic Clinical Issues
paraspinal mass +/- intraspinal extension, • Abdominal mass/pain, bone pain, fatigue, weight
calcification loss, blanching subcutaneous nodules
• Location: Abdominal (40% adrenal, 25% paraspinal • Paraparesis/paraplegia (cord compression)
ganglia) > thoracic (15%) >, pelvic (5%) > cervical
(3%); miscellaneous (12%) Diagnostic Checklist
• Infants more often present with thoracic, cervical
Top Differential Diagnoses tumors; older children with abdominal tumors
• Ewing sarcoma • Critical to recognize epidural extension prior to
• Vertebral metastasis surgery
• Nerve sheath tumor
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NEUROBLASTIC TUMOR
1 • Evans anatomic staging for NB (prognosis - % survival)
o Locoregional (combines stages 1-3) Treatment
72 • 1: Confined to organ of interest (90%) • Options include chemotherapy, surgery, radiation,
• 2: Extension beyond organ but not crossing steroids
midline (75%)
• 3: Extension crossing midline (include vertebral
column) (30%) IDIAGNOSTIC CHECKLIST
o Stage 4: Systemic, widespread distal metastases
(10%)
Consider
o Stage 4S: < 1 yrs at diagnosis, metastatic disease • NB presentation depends on patient age, primary site,
confined to skin, liver, and bone marrow, may metastatic burden, metabolically active products
spontaneously regress (nearly 100% survival) • Infants more often present with thoracic, cervical
tumors; older children with abdominal tumors
Image Interpretation Pearls
ICLINICAL ISSUES • Critical to recognize epidural extension prior to
Presentation surgery
• Most common signs/symptoms
o Abdominal mass/pain, bone pain, fatigue, weight
loss, blanching subcutaneous nodules
ISELECTED REFERENCES
o Paraparesis/paraplegia (cord compression) 1. Tateishi U et al: Adult neuroblastoma: radiologic and
o Other signs/symptoms clinicopathologic features. ] Comput Assist Tomogr.
27(3):321-6, 2003
• Diarrhea (VIP syndrome) Rha SE et al: Neurogenic tumors in the abdomen: tumor
2.
• Proptosis, periorbital/conjunctival ecchymoses types and imaging characteristics. Radiographies.
("raccoon eyes") 23(1):29-43, 2003
• Opsoclonus-myoclonus-ataxia (OMA) 3. Pfluger T et al: Integrated imaging using MRI and 1231
paraneoplastic syndrome (2-3%) metaiodobenzylguanidine scintigraphy to improve
• Horner syndrome (cervical NB) sensitivity and specificity in the diagnosis of pediatric
o Laboratory findings neuroblastoma. A]R Am] Roentgenol. 181(4):1115-24,
• > 90% 1 urine homovanillic acid (HVA) and/or 2003
4. Siegel M] et al: Staging of neuroblastoma at imaging: report
vanillylmandelic acid (VMA)
of the radiology diagnostic oncology group. Radiology.
• 1 Serum neuron-specific enolase (NSE), lactic 223(1):168-75,2002
dehydrogenase (LDH), ferritin 5. Lonergan G] et al: Neuroblastoma, ganglioneuroblastoma,
• Clinical profile and ganglioneuroma: radiologic-pathologic correlation.
o Kerner-Morrison syndrome ~ intractable secretory Radiographies. 22(4):911-34, 2002
diarrhea 2° VIP secretion (GN, GNB > NB) 6. Meyer ]S et al: Imaging of neuroblastoma and Wilms'
o Pepper syndrome: Infant with overwhelming liver tumor. Magn Reson Imaging Clin N Am. 10(2):275-302,
metastatic NB ~ respiratory compromise 2002
7. Geoerger B et al: Metabolic activity and clinical features of
o "Blueberry muffin baby": Infant with subcutaneous primary ganglioneuromas. Cancer. 91(10):1905-13, 2001
metastatic NB 8. Kushner BH et al: Extending positron emission
o Hutchinson syndrome: Widespread bone metastasis tomography scan utility to high-risk neuroblastoma:
~ bone pain, limping, pathologic fractures fluorine-18 fluorodeoxyglucose positron emission
o NAT mimic: Metastatic retrobulbar neuroblastoma tomography as sole imaging modality in fOllow-up of
~ rapidly progressive painless proptOSiS, periorbital patients.] Clin Oncol. 19(14):3397-405,2001
ecchymosis 9. Joshi V]: Peripheral neuroblastic tumors: Pathological
classification based on recommendations of international
Demographics neuroblastoma committee. Pediatr and Dev Path. 3:184-99,
• Age: 40% < 1 yr, 35% 1-2 yr, and 25% > 2 yr at 2000
diagnosis; rare after age 10 yr 10. Schwab et al: Neuroblastic tumors of the adrenal gland and
sympathetic nervous system, Kleihues and Cavenee (eds):
• Gender: M > F (1.3:1) Pathology and Genetics of tumors of the nervous system.
Natural History & Prognosis IARC press, Lyon. 153-161,2000
11. Sofka CM et al: Magnetic resonance imaging of
• GN prognosis excellent after surgical resection neuroblastoma using current techniques. Magn Reson
• GNB prognosis dependent on proportion of GN, NB Imaging. 17(2):193-8, 1999
• NB 5 year survival"" 83% for infants, 55% for children 12. Perel Y et al: Clinical impact and prognostic value of
1-5 years, and 40% for children> 5 years metaiodobenzylguanidine imaging in children with
o Favorable prognostic indicators: Locoregional, stage metastatic neuroblastoma. ] Pediatr Hematol Oncol.
4s, I n-myc amplification, hyperdiploid DNA 21(1):13-8, 1999
o Unfavorable prognostic indicators: Stage 4 disease, 13. Brodeur GM et al: International criteria for diagnosis,
staging, and response to treatment in patients with
ocular involvement, HER2/neu oncogene neuroblastoma.] Clin Oncol. 6(12):1874-81,1988
over-expression, 1 NSE/LDH/serum ferritin, 1 urine
HVA/VMA, Ip chromosomal deletion
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NEUROBLASTIC TUMOR
I IMAGE GALLERY 1
73
(Left) Anteroposterior
radiography shows large
posterior mediastinal mass
(ganglioneuroma) without
rib or vertebral destruction
(Courtesy R. Boyer, MO).
(Right) Axial T7 C+ MR
reveals large left (surgically
proven) paraspinal
ganglioneuroma. No
vertebral or neural foramina I
invasion was detected
(Courtesy C. Hedlund, DO).
Typical
(Left) Axial NECT depicts
large left posterior
mediastinal mass with
stippled internal calcification.
Surgical pathology revealed
ganglioneuroblastoma
(Courtesy R. Boyer, MO).
(Right) Coronal T7 C+ MR
demonstrates
heterogeneously enhancing
necrotic thoracic
neuroblastoma without
epidural invasion. The large
mass produces atelectasis of
adjacent lung (Courtesy C.
Hedlund, DO).
Typical
(Left) Coronal 1-123 MIBC
coronal SPECT scan
demonstrates typical avid
uptake within large left
paraspinal neuroblastoma
(arrow) (Courtesy B. Reid,
MOJ. (Right) Sagittal T7 C+
MR shows extensive blastic
enhancing extradural
neuroblastoma metastases to
the axial skeleton in child
(Courtesy C. Hedlund, DO).
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ANGIOLIPOMA
1
74
Sagittal T1WI MR shows long segment heterogeneous Sagittal T2WI MR shows heterogeneous slight
T1 hyperintense mass within posterior thoracic epidural hyperintensity from dorsal epidural mass extending over
space causing cord compression. Scattered foci of low multiple thoracic segments. Cord compression is well
signal are present (arrows). identified (arrows).
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ANGIOLIPOMA
Key Facts
1
Terminology • T1 C+: Fat suppressed T1WI most useful for lesion 75
• Angiolipoma (AL), vascular lipoma, hemangiolipoma, definition, showing heterogeneous enhancement
fibromyolipoma Pathology
• Benign tumor of adipose and vascular elements • May occur as focal or infiltrating forms
Imaging Findings • Infiltrating form more common in anterior epidural
• Best diagnostic clue: Hyperintense mass on space, may destroy adjacent bone
unenhanced T1 WI, showing enhancement on fat • Focal form more common in posterior thoracic
suppressed Tl WI epidural space, no bone destruction
• Spine involvement uncommon: Extradural> > Clinical Issues
intramedullary • Nonspecific back pain
• Extend over 1-4 vertebral body segments • Progressive paraparesis
• CT shows extradural mass of fat attenuation (-20 to • Surgical excision for painful, symptomatic lesions
-60 HU), with scattered soft tissue reticulation
• Hyperintense on T1WI, with inhomogeneous signal Diagnostic Checklist
• Angiolipomas do not show vascular flow voids
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ANGIOLIPOMA
1 o Consistent marker of angiolipomas is presence of
fibrin microthrombi in vascular channels associated
with extension into the spinal canal: a case report. Korean
J Radiol. 1(4): 212-4, 2000
76 with disrupted endothelial cells 4. Bailey D et al: Dorsal thoracic cord compression from a
spinal angiolipoma: case report and brief comment. Conn
• Vessels variously described as sinusoids, thin-walled,
Med. 64(5): 267-9, 2000
thick walled with smooth muscle proliferation 5. Labram EK et al: Revisited: spinal angiolipoma--three
• Mitoses and pleomorphism not present additional cases. Br J Neurosurg. 13(1): 25-9, 1999
• Features vary 6. Oge HK et al: Spinal angiolipoma: case report and review of
o Predominately lipomatous with few small literature. J Spinal Disord. 12(4): 353-6, 1999
angiomatous regions 7. Klisch J et al: Radiological and histological findings in
o Predominately vascular with small lipomatous spinal intramedullary angiolipoma. Neuroradiology. 41(8):
component 584-7,1999
8. Sciot R et al: Cytogenetic analysis of subcutaneous
angiolipoma: further evidence supporting its difference
from ordinary pure lipomas: a report of the CHAMP Study
ICLINICAL ISSUES Group. AmJ Surg Pathol. 21(4):441-4,1997
9. Weiss SW: Lipomatous tumors. Monogr Pathol. 38:207-39,
Presentation 1996
• Most common signs/symptoms 10. Provenzale JM et al: Spinal angiolipomas: MR features.
o Nonspecific back pain AJNR AmJ Neuroradiol. 17(4): 713-9, 1996
o Other signs/symptoms: Cord compression 11. Fletcher CD et al: Correlation between clinicopathological
• Progressive paraparesis features and karyotype in lipomatous tumors. A report of
178 cases from the Chromosomes and Morphology
• Symptoms may progress with pregnancy (CHAMP) Collaborative Study Group. Am J Pathol.
• Acute onset of symptoms may occur due to 148(2):623-30, 1996
degeneration, or hemorrhage into lesion 12. Shibata Y et al: Thoracic epidural angiolipoma--case report.
• Clinical profile: Slowly progressive paraparesis with Neurol Med Chir (Tokyo). 33(5): 316-9, 1993
back pain 13. Jelinek JS et al: Liposarcoma of the extremities: MR and CT
findings in the histologic subtypes. Radiology.
Demographics 186(2):455-9, 1993
• Age 14. Pagni CA et al: Spinal epidural angiolipoma: rare or
o 5th decade, although may occur in children or unreported? Neurosurgery. 31(4): 758-64; discussion 764,
adults 1992
o Mean age 42 years 15. Stranjalis G et al: MRI in the diagnosis of spinal extradural
angiolipoma. Br J Neurosurg. 6(5): 481-3, 1992
• Gender: M < F 16. Weill A et al: Spinal angiolipomas: CT and MR aspects. J
Natural History & Prognosis Comput Assist Tomogr. 15(1): 83-5, 1991
17. Quint DJ et al: Epidural lipomatosis. Radiology.
• Slowly progressive 169(2):485-90, 1988
• Bone infiltration associated with more aggressive 18. Matsushima K et al: Spinal extradural angiolipoma: MR
behavior and worse prognosis and CT diagnosis. J Comput Assist Tomogr. 11(6):1104-6,
1987
Treatment 19. Dixon AY et al: Angiolipomas: an ultrastructural and
• Surgical excision for painful, symptomatic lesions clinicopathological study. Hum Pathol. 12(8):739-47, 1981
o Focal form low rate of recurrence following excision 20. HOWARD WR et al: Angiolipoma. Arch Dermatol.
o Infiltrative form 50% recurrence rate 82:924-31, 1960
• Partial resection may give good symptomatic relief in
infiltrative form
• Degree of hypointensity on T1 WI predictive of degree
of vascularity encountered at surgery
I DIAGNOSTIC CHECKLIST
Consider
• Not to be confused with angiomyolipoma, which is
seen in tuberous sclerosis, and occurs in kidney
Image Interpretation Pearls
• Angiolipomas do not show vascular flow voids
ISELECTED REFERENCES
1. Gelabert-Gonzalez M et al: Spinal extradural angiolipoma,
with a literature review. Childs Nerv Syst. 18(12): 725-8,
2002
2. Amlashi SF et al: Spinal epidural angiolipoma. J
Neuroradiol. 28(4): 253-6, 2001
3. Choi JY et al: Angiolipoma of the posterior mediastinum
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ANGIOLIPOMA
IIMAGE GALLERY 1
77
(Left) Sagittal T1 C+ MR
shows enhancing
heterogeneous signal
epidural mass extending over
multiple thoracic segments,
compressing the cord
anteriorly. Adjacent bone
intact. (Right) Axial T2* CRE
MR shows intermediate fat
signal mass within the
posterior epidural space,
displacing cord anteriorly
(arrow). Small amount of
heterogeneous signal present
on right (open arrow).
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MENINGIOMA
1
78
Axial CECT done for abdominal pain, incidentally Sagittal T1 C+ MR shows homogeneously enhancing
depicts calcified meningioma within lower thoracic oval lesion in low thoracic canal. Note dural base,
spinal canal (arrow). well-circumscribed margins.
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MENINGIOMA
Key Facts
1
Terminology Top Differential Diagnoses 79
• Slow growing, benign tumor originating from and • Schwannoma
based on the dura mater • Lymphoma
• Extradural masses
Imaging Findings
• Enhancing intradural/extramedullary mass + dural Pathology
"tail" • Most are solitary, sporadic
• T1 WI: Isointense (to spinal cord) • Thoracic (80%) > > cervical (16%) > lumbar (4%)
• Majority are isointense to cord • NF2
• Prominent enhancement • Multiple meningiomatosis
• Myelography, CT myelography • Firm, well demarcated, lobulated/rounded mass with
• Sharp meniscus of contrast caps lesion (classic for dural attachment
intradural, extramedullary mass) • Occasionally cystic
• Ipsilateral subarachnoid space widened (cord, roots
displaced away from mass)
o Sharp meniscus of contrast caps lesion (classic for • Site frequency roughly proportional to surface
intradural, extramedullary mass) area of dura in spine
o Ipsilateral subarachnoid space widened (cord, roots • Genetics
displaced away from mass) o Almost all have chromosome 22 abnormalities
o NF2
Imaging Recommendations • Multiple meningiomatosis
• Contrast-enhanced MRI • Familial clear cell meningioma syndrome
• CT (if densely calcified) • Multiple meningiomas
• Etiology
o Arise from arachnoid cap cell rests
IDIFFERENTIAL DIAGNOSIS o Genetic predisposition in women, NF2
o Associated with prior radiation
Schwannoma
• Epidemiology
• Very hyperintense on T2WI o Second most common intradural extramedullary
• Cystic change, hemorrhage more common tumor
• No dural attachment
• 25% of primary spinal tumors
• Very rare posterior to cord o F:M = 4:1
Other intradural extramedullary masses • Associated abnormalities: Bony sclerosis
• Paraganglioma (rare) Gross Pathologic & Surgical Features
• Epidermoid (signal usually = CSF) • Firm, well demarcated, lobulated/rounded mass with
• Arachnoid cyst (like CSF, doesn't enhance) dural attachment
• Intradural metastasis (often multiple) • Expands centripetally within dural sac
• Intradural tumors below cordi e.g., cauda equina • May rarely have dumbbell morphology simulating
ependymoma, drop metastases neurinoma
Lymphoma • Occasionally cystic
• Solitary intradural mass uncommon Microscopic Features
Extradural masses • Most are "typical" meningiomas
• Can simulate meningioma if big, cannot be o Common histologic subtypes
compartmentalized • Most common = psammomatous type with Ca++
o Chordoma concretions
o Cyst • Meningothelial
• Fibrous
• Transitional
!PATHOLOGY o Less common
• Angiomatous
General Features • Microcystic
• General path comments • Clear cell
o Most are solitary, sporadic • Choroid
o Intracranial: Spine meningiomas = 8:1 • Lipomatous
o Thoracic (80%) > > cervical (16%) > lumbar (4%) • Hemorrhagic
• Rare
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MENINGIOMA
1 o Atypical (increased mitoses, cellularity, etc.)
o Anaplastic (malignant) 6.
177(6):1479-81,2001
Louis DN et al: Meningiomas. In Kleihues P, Cavanee WK
• Angioblastic, difficult to distinguish from (eds), Tumours of the Nervous System, 176-84, IARC Press,
80
2000
hemangiopericytoma 7. Naderi S: Spinal meningiomas. Surg Neurol. 54(1):95, 2000
• Can metastasize or be multicentric 8. Klekamp J et al: Surgical results for spinal meningiomas.
Surg Neurol. 52: 552-62, 1999
Staging, Grading or Classification Criteria 9. Yoshiura T et al: Cervical spinal meningioma with unusual
• > 95% WHO grade I MR contrast enhancement. AJNR Am J Neuroradiol.
19(6):1040-2, 1998
10. Sato N et al: Extradural spinal meningioma: MRI.
ICLINICAL ISSUES Neuroradiology. 39(6):450-2, 1997
11. Borden NM: Aggressive angioblastic meningioma with
Presentation multiple sites in the neural axis. AlNR Am J Neuroradiol.
• Most common signs/symptoms 16(4):793-4, 1995
12. Akeson Pet al: Radiological investigation of
o Pain
neurofibromatosis type 2. Neuroradiology. 36(2):107-10,
o Other signs/symptoms 1994
• Motor/sensory deficit 13. Souweidane MM et al: Spinal cord meningiomas.
• Gait disturbance Neurosurg Clin N Am. 5(2):283-91, 1994
• Clinical profile: Female with onset of myelopathy 14. Kabus D et al: Metastatic meningioma.
Hemangiopericytoma or angioblastic meningioma? Am J
Demographics Surg Pathol. 17(11):1144-50, 1993
• Age 15. Kannuki S et al: Coexistence of intracranial and spinal
o Peak incidence 5th/6th decade meningiomas--report of two cases. Neurol Med Chir
o Under 50, more commonly genetic, worse prognosis (Tokyo). 31(11):720-4, 1991
16. Solero CL et al: Spinal menigiomas: Review of 174 operated
• Gender: > 80% female
cases. Neurosurg. 125: 153-60, 1989
Natural History & Prognosis 17. Levy WJ Jr et al: Spinal cord meningioma. J Neurosurg.
57(6):804-12, 1982
• Slow growing, compresses but doesn't invade adjacent
structures
• Excellent prognosis with complete excision
o Possible in 96%
• Recurrence rate up to 40% at 5 years in patients with
incomplete resection, en plaque, and infiltrative
meningioma
Treatment
• Complete surgical resection
• +/- XRT (subtotal resection, aggressive tumors)
IDIAGNOSTIC CHECKLIST
Consider
• Angiogram for distinguishing meningioma,
neurofibroma
Image Interpretation Pearls
• If tumor is dorsal to cord, almost always meningioma,
not neurinoma (nerve roots are anterolaterally located)
ISELECTED REFERENCES
1. Cohen-Gadol AA et al: Spinal meningiomas in patients
younger than 50 years of age: a 21-year experience. J
Neurosurg. 98(3 Suppl):258-63, 2003
2. Covert S et al: Magnetic resonance imaging of
intramedullary meningioma of the spinal cord: case report
and review of the literature. Can Assoc Radiol].
54(3):177-80,2003
3. Lucey BP et al: Spinal meningioma causing diffuse
leptomeningeal enhancement. Neurology. 60(2):350-1,
2003
4. Doita M et al: Recurrent calcified spinal meningioma
detected by plain radiograph. Spine. 26(11):E249-52, 2001
5. Eastwood JD et al: Diffusion-weighted MR imaging in a
patient with spinal meningioma. AJR Am J Roentgenol.
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MENINGIOMA
IIMAGE GAllERY 1
81
Variant
(Left) Sagittal T2WI MR in
patient with family history of
NF2 shows extrameduHary
multicystic mass, which
enhanced. Despite similarity
to neurinoma, dorsal
location favors meningioma -
proven at surgery. (Right)
Sagittal T7 C+ MR shows
enhancing multicystic mass
(family history of NF2):
Nonspecific appearance.
Dorsal extra-axial location
favors meningioma
(surgicaHy proven), though
cysts unusual.
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HEMANGIOPERICYTOMA
1
82
Sagittal T1 C+ MR shows enhancing, dural based Axial T1 C+ MR shows paraspinous, vividly enhancing
intraspinal mass invading the posterior elements. mass associated with intraspinal dural component.
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HEMANGIOPERICYTOMA
Key Facts
1
Terminology Top Differential Diagnoses 83
• Hypervascular neoplasm arising from pericytes • Meningioma
• Chordoma and other primary bone malignancies
Imaging Findings • Neurinoma
• Best diagnostic clue: Vividly enhancing lesion • Aggressive hemangioma
expanding/eroding spinal canal, with large soft tissue • Vascular metastases
component
• Dural based if primary Pathology
• Epicenter in bone if metastatic • Vascular neoplasm consisting of capillaries outlined
• TlWI: Hypointense multilobular mass by intact basement membrane
expanding/eroding bone and canal • Basement membrane separates capillaries from
• T2WI: Mild to moderate hyperintensity spindle shaped tumor cells in the extravascular area
• Tl C+: Vivid, homogeneous enhancement • Undifferentiated malignancy, but may see dual
• Conventional: Marked hypervascularity in arterial differentiation towards hemangiopericytoma and
phase with abnormal irregular vessels meningioma
Angiosarcoma Treatment
• Very rarely in spine • Options, risks, complications: Resection, radiation
• More haphazard boundaries
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HEMANGIOPERICYTOMA
1 IDIAGNOSTIC CHECKLIST Neuropathol (Berl). 21(3):253-7, 1972
84 Consider
• Angiography and embolization for operative planning
Image Interpretation Pearls
• Malignant meningioma more likely to show invasive
finger like processes as opposed to lobules of
hemangiopericytoma
ISELECTED REFERENCES
1. Woitzik J et al: Delayed manifestation of spinal metastasis:
a special feature of hemangiopericytoma. Clin Neurol
Neurosurg. 105(3):159-66,2003
2. Musacchio M et al: Posterior cervical
haemangiopericytoma with intracranial and skull base
extension. Diagnostic and therapeutic challenge of a rare
hypervascular neoplasm. J Neuroradiol. 30(3):180-7, 2003
3. Cox DP et al: Myopericytoma of the thoracic spine: a case
report. Spine. 28(2):E30-2, 2003
4. Akhaddar A et al: Thoracic epidural hemangiopericytoma.
Case report. J Neurosurg Sci. 46(2):89-92; discussion 92,
2002
5. Tayoro K et al: [Imaging of meningeal
hemangiopericytomas] J Radiol. 83(4 Pt 1):459-65, 2002
6. Ijiri K et al: Primary epidural hemangiopericytoma in the
lumbar spine: a case report. Spine. 27(7):E189-92, 2002
7. Betchen S et al: Intradural hemangiopericytoma of the
lumbar spine: case report. Neurosurgery. 50(3):654-7, 2002
8. Nonaka M et al: Metastatic meningeal
hemangiopericytoma of thoracic spine. Clin Neurol
Neurosurg. 100(3):228-30, 1998
9. Lin YJ et al: Primary hemangiopericytoma in the axis bone:
case report and review of literature. Neurosurgery.
39(2):397-9; discussion 399-400, 1996
10. Murphey MD et al: From the archives of the AFIP.
Musculoskeletal angiomatous lesions: radiologic-pathologic
correlation. Radiographics. 15(4):893-917, 1995
11. Kozlowski K et al: Primary sacral bone tumours in children
(report of 16 cases with a short literature review). Australas
Radiol. 34(2):142-9, 1990
12. Bridges LR et al: Haemangiopericytic meningioma of the
sacral canal: a case report. J Neurol Neurosurg Psychiatry.
51(2):288-90, 1988
13. Cuccurullo L et al: Hemangiopericytoma of the cervical
spine. A case report. Acta Neurol (Napoli). 6(6):472-81,
1984
14. Beadle GF et al: Treatment of advanced malignant
hemangiopericytoma with combination adriamycin and
DTIC: a report of four cases. J Surg Oncol. 22(3):167-70,
1983
15. Muraszko KM et al: Hemangiopericytomas of the spine.
Neurosurgery. 10(4):473-9, 1982
16. Cappabianca P et al: Hemangiopericytoma of the spinal
canal. Surg Neurol. 15(4):298-302, 1981
17. Stern MB et al: Hemangiopericytoma of the cervical spine:
report of an unusual case. Clin Orthop. (151):201-4, 1980
18. Anderson C et al: Skeletal metastases of an intracranial
malignant hemangiopericytoma. Report of a case. J Bone
Joint Surg Am. 62(1):145-8, 1980
19. Subbarao K et al: Primary malignant neoplasms. Semin
Roentgenol. 14(1):44-57, 1979
20. Harris DJ et al: Hemangiopericytoma of the spinal canal.
Report of three cases. J Neurosurg. 49(6):914-20, 1978
21. Hart S et al: Haemangiopericytoma of the pre-sacral space.
Br J Surg. 60(7):583-4, 1973
22. Baxter PJ et al: Haemangiopericytoma. Light and electron
microscopy studies of a cerebral secondary tumour. Acta
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HEMANGIOPERICYTOMA
IIMAGE GALLERY 1
85
Typical
(Left) Axial NECT shows
large isodense lesion
expanding and eroding the
sacral canal and neural
foramen in patient with
previously treated
intracranial
hemangiopericytoma. (Right)
Axial TlWI MR shows
replacement of right arch
and vb by isointense lesion,
encasement of vertebral
artery. Lesion showed high
signal on T2WI and vivid
enhancement. Patient had
intracranial primary.
Typical
(Left) Axial T2* GRE MR
shows high signal lesion
expanding right arch of C4
and encasing vertebral
artery. Metastatic
hemangiopericytoma. (Right)
Axial Tl C+ MR shows
enhancing expansile lesion
within arch and vb of C4.
Metastatic
hemangiopericytoma.
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SCHWANNOMA
1
86
Axial graphic portrays a right-sided "dumbbell" shaped Axial T2WI MR with fat suppression shows a
spinal nerve root schwannoma, enlarging neural "dumbbell" shaped schwannoma in right intervertebral
foramen and compressing spinal cord. Both intra- and foramen.
extradural components are present.
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SCHWANNOMA
Key Facts
1
Terminology • 75% hyperintense 87
• Neurinoma, neurilemmoma • Intense enhancement
• Neoplasm of nerve sheath in peripheral nervous Top Differential Diagnoses
system • Neurofibroma (NF)
Imaging Findings • Meningioma
• Best diagnostic clue: Well-circumscribed, "dumbbell" • Myxopapillary ependymoma
shaped, enhancing spinal mass Pathology
• 70-75% intradural extramedullary • Sporadic: Most common
• Most common intradural extramedullary mass • Inactivation of NF2 gene
• 15% completely extradural • 30% of primary spine tumors
• 15% "dumbbell"
• Enlarged intervertebral foramen Clinical Issues
• Thinned pedicle • Clinical profile: Pain associated with movement
• Isodense to cord, nerve roots
• Iso/hypointense relative to cord, roots
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SCHWANNOMA
1 • Etiology
o Inactivation of NF2 gene Treatment
88 • Encoding merlin protein • Total surgical resection
• Epidemiology o Involved nerve root sacrificed
o 30% of primary spine tumors
o Typically solitary unless part of inherited tumor
syndrome IDIAGNOSTIC CHECKLIST
• Associated abnormalities
o NF2
Consider
• Spinal meningiomas • Schwannoma when solitary enhancing "dumbbell"
• Intramedullary ependymomas shaped spinal lesion present
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SCHWANNOMA
IIMAGE GAllERY 1
89
(Left) Coronal Tl C+ MR
with fat suppression shows
an enhancing intradural
schwannoma deviating
thoracic cord to the left
(arrows). (Right) Axial Tl C+
MR with fat suppression
shows an intradural
extramedullary
homogeneously enhancing
schwannoma compressing
spinal cord.
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NEUROFIBROMA
1
90
Axial graphic portrays bilateral lobulated plexiform Coronal T2WI MR shows bilateral multi-level thoracic
neurofibromas in NFl. There is erosion of the left spinal neurofibromas in a patient with NFl.
pedicle.
• Morphology
ITERMINOlOGY o Three NF morphologic features
Abbreviations and Synonyms • Localized
• Neurofibroma (NF) • Diffuse
• Nerve sheath tumor • Plexiform
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NEUROFIBROMA
Key Facts
1
Terminology • Malignant peripheral nerve sheath tumor (MPNST) 91
• Localized, diffuse, or plexiform neoplasm of nerve metabolically active
sheath Top Differential Diagnoses
Imaging Findings • Schwannoma
• Best diagnostic clue: Bulky multilevel spinal nerve • Spinal meningioma
root tumors in patient with stigmata of • Meningocele
neurofibromatosis type 1 (Nfl) Pathology
• Localized • 5% of all benign soft tissue tumors
• Diffuse • 90% of NFs occur as sporadic, solitary tumors
• Plexiform • 13-65% of NFl patients have spinal NFs
• Isodense to spinal cord
• T1WI: Similar signal intensity (SI) to cord and nerve Clinical Issues
roots • Clinical profile: Rapid growth of NF suggestive of
• "Target sign" malignant transformation
• Hypointense septations throughout plexiform NF
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NEUROFIBROMA
1 o 90% of NFs occur as sporadic, solitary tumors
o 13-65% of NFl patients have spinal NFs
o high recurrence rate
• MR surveillance of asymptomatic spinal NFs in NFl of
92 • 57% single lesions doubtful benefit
o 3-5% of NFl patients develop MPNST
o 50-60% of MPNSTs are associated with NFl
• Associated abnormalities I DIAGNOSTIC CHECKLIST
o NFl
• Short segment thoracic scoliosis ± kyphosis: Image Interpretation Pearls
40-60% • All spinal nerve root tumors are NFs in NFl
• Vertebral anomalies • They are schwannomas or mixed tumors in NF2
• Meningocele • Solitary spinal lesion most likely schwannoma
• Dural ectasia
• Intramedullary astrocytomas
I SELECTED REFERENCES
Microscopic Features
1. Khong PL et al: MR imaging of spinal tumors in children
• No true capsule with neurofibromatosis 1. AJR Am J Roentgenol.
• Neoplastic Schwann cells + fibroblasts 180(2):413-7, 2003
• Tumor, nerve fascicles intermixed 2. Cardona S et al: Evaluation of Fl8-deoxyglucose positron
o Presence ofaxons characteristic of NFs emission tomography (FDG-PET) to assess the nature of
• Collagen common neurogenic tumours. Eur J Surg Oncol. 29(6):536-41, 2003
o Rare in schwannoma 3. Simoens WA et al: MR features of peripheral nerve sheath
• Mucoid/myxoid matrix tumors: can a calculated index compete with radiologist's
experience? Eur Radiol. 11: 250-7, 2001
o Rare hemorrhage, fatty degeneration, or vascular
4. Woodruff JM et al: Neurofibroma. In: Kleihues P, Cavenee
change WK (eds), Tumors of the Nervous System, 167-8, IARC
• Mitotic figures rare in NFs Press, 2000
o High mitotic rate in MPNSTs 5. Ferner RE et al: Evaluation of (18)fluorodeoxyglucose
• Immunohistochemistry positron emission tomography «18)FDG PET) in the
o S-100 and anti-leu 7 positive detection of malignant peripheral nerve sheath tumours
arising from within plexiform neurofibromas in
Staging, Grading or Classification Criteria neurofibromatosis 1. J Neurol Neurosurg Psychiatry.
• NFs: WHO grade I 68(3):353-7, 2000
• MPNSTs: WHO grade III/IV 6. Thakkar SD et al: Spinal tumours in neurofibromatosis type
1: an MRI study of frequency, multiplicity and variety.
Neuroradiology. 41(9):625-9, 1999
7. Murphey MD et al: Imaging of musculoskeletal neurogenic
ICLINICAL ISSUES tumors: Radiologic-pathologic correlation. RadioGraphies.
19: 1253-80, 1999
Presentation 8. Klekamp J et al: Surgery of spinal nerve sheath tumors with
• Most common signs/symptoms special reference to neurofibromatosis. Neurosurgery.
o Pain 42(2):279-89; discussion 289-90, 1998
o Weakness 9. Yagi T et al: Intramedullary spinal cord tumour associated
o Sensory deficit with neurofibromatosis type 1. Acta Neurochir (Wien).
139(11):1055-60, 1997
• Associated with plexiform NFs
10. Shariff SY et al: Intraspinal tumours in children--clinical
o Other signs/symptoms presentation. Ir Med]. 90(7):264-5, 1997
• Myelopathy 11. Grossman RI et al: Neuroradiology The Requisites. 489-90.
• NFl patients: Cafe-au-Iait spots, skin fold Mosby-Year Book, Inc., 1994
freckling, cutaneous NFs, Lisch nodules 12. Tatagiba M et al: Involvement of spinal nerves in
• Clinical profile: Rapid growth of NF suggestive of neurofibromatosis. Neurosurg Rev. 17(1):43-9, 1994
malignant transformation 13. Shu HH et al: Neurofibromatosis: MR imaging findings
involving the head and spine. AJR Am J Roentgenol.
Demographics 160(1):159-64, 1993
• Age: Peak presentation: 20-30 y 14. Clinchot DM et al: The spectrum of neurofibromatosis:
• Gender: No gender predilection neurologic manifestations with malignant transformation.
J Am Paraplegia Soc. 16(2):81-3, 1993
• Ethnicity: No racial predilection 15. Egelhoff]C et al: Spinal MR findings in neurofibromatosis
Natural History & Prognosis types 1 and 2. AlNR Am] Neuroradiol. 13(4):1071-7, 1992
16. Ros PR et al: Plexiform neurofibroma of the pelvis: CT and
• Slow-growing MRI findings. Magn Reson Imaging. 9(3):463-5, 1991
• Malignant transformation to MPNSTs 17. Li MH et al: MR imaging of spinal neurofibromatosis. Acta
o Rapid growth of NFs Radiol. 32(4):279-85, 1991
o Rare with sporadic NFs
o 3-5% of plexiform NFs
Treatment
• Resection of sporadic/solitary NF
o > 80% cure rate
• Plexiform NFs difficult to resect
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NEUROFIBROMA
IIMAGE GALLERY 1
93
Typical
(Left) Axial T1 WI MR
through thoracic spine shows
an isointense mass within left
neuroforamen. A second
mass is present more laterally
and dorsally (arrow). (Right)
Axial T1 C+ MR shows two
neurofibromas with diffuse
enhancement.
Typical
(Left) Axial T2WI MR with fat
suppression shows an
infiltrating neurofibroma in
left malar soft tissue. Bilateral
parapharyngeal
neurofibromas are evident
(arrows). (Right) Axial T2WI
MR shows plexiform
neurofibromas involving
sacral plexus bilaterally with
intraspinal extension (arrow).
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MALIGNANT NERVE SHEATH TUMORS
1
94
Axial T1WI MR in a patient with NFl shows a large Axial T1 C+ MR shows right paraspinous MPNST with
isointense mass within right paraspinous muscle (open heterogenous enhancement. The left neurofibroma also
arrow). A second smaller mass resides in left enhances. In addition, multiple intrathecal
neuroforamen (arrow). neurofibromas are present (arrows).
,
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-
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Ulnar Schwannoma
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Liposarcoma
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- "*
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MALIGNANT NERVE SHEATH TUMORS
Key Facts
1
Terminology Top Differential Diagnoses 95
• Malignant lesion of neural origin involving spinal • Benign peripheral nerve sheath tumor
root, neural plexus, peripheral nerve, or end organs • Soft tissue sarcoma
Imaging Findings Pathology
• Best diagnostic clue: Large infiltrative, often • Incidence in general population: 0.001 %
hemorrhagic, soft tissue mass related to neurovascular • 50-60% of MPNST patients have NFl
bundle
• Intervertebral foraminal widening Clinical Issues
• Heterogeneous areas correspond to hemorrhage and • Involves major nerve trunks
necrosis • Sudden enlargement of preexisting neurofibroma in
• TlWI: Soft tissue mass isointense to muscle NFl
• T2WI: Hyperintense compared to surrounding fat • Local recurrence: 26-65%
• Tl C+: Marked enhancement • Metastases: 20-65%
• Difficult to distinguish from benign spinal • Surgical resection
schwannomas • Improved resectability with distal lesions
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MALIGNANT NERVE SHEATH TUMORS
1 • No true capsule o Younger age
• Tan-gray, fleshy
96 • Areas of hemorrhage and necrosis Treatment
• Infiltrates surrounding soft tissue • Surgical resection
• Spreads along perineurium and epineurium o Improved resectability with distal lesions
• 20% in paraspinal MPNST vs. 95% in extremity
Microscopic Features lesions
• Fasciculated hyperchromatic mitotically active spindle o Amputation often necessary
cells o Resection with wide margins
• Epithelioid and mesenchymal differentiation: 12-27% • Margin of at least 3 cm recommended
o Due to pluripotent nature of neural crest • Pre- or post-operative radiation therapy
o Epithelial glands, foci of mature bone and cartilage • Chemotherapy for metastatic disease
o Features of chondrosarcoma, rhabdomyosarcoma o Effectiveness unproven
(malignant Triton tumor), osteosarcoma,
angiosarcoma
• Immunohistochemistry stains for S-100 protein, Leu-7, IDIAGNOSTIC CHECKLIST
Vimentin, and neuron specific enolase
Consider
Staging, Grading or Classification Criteria • Malignant transformation when preexisting
• Surgfcal staging neurofibroma enlarges
o Stage I: Low grade
o Stage II: High grade
o Stage III: Low or high grade, metastases ISELECTED REFERENCES
• Within each Stage: A = intracompartmental; B =
1. Baehring JM et al: Malignant peripheral nerve sheath
extracompartmental tumor: the clinical spectrum and outcome of treatment.
• Low grade: Perineurial cell differentiation Neurology. 61(5):696-8, 2003
• High grade: High mitotic rate, ± necrosis, sarcomatous 2. Su W et al: Malignant peripheral nerve sheath tumor cell
divergent differentiation invasion is facilitated by Src and aberrant CD44 expression.
Glia. 42:350-8, 2003
3. Asavamongkolkul A et al: Malignant peripheral nerve
I CLINICAL ISSUES sheath tumor with neurofibromatosis type 1: a 2-case
report and review of the literature. J Med Assoc Thai.
Presentation 84(2):285-93, 2001
4. Weiss SW et al: Enzinger and Weiss's soft tissue tumors. 4th
• Most common signs/symptoms ed. St.Louis MO, CV Mosby, 1209-63,2001
o Local or radicular pain 5. Ferner RE et al: Evaluation of (18) fluorodeoxyglucose
o Enlarging soft tissue mass positron emission tomography ((18) FDG PET) in the
o Paraparesis detection of malignant peripheral nerve sheath tumors
o Paresthesia arising from within pleXiform neurofibromas in
• Clinical profile neurofibromatosis 1. J Neurol Neurosurg Psychiatry.
o Involves major nerve trunks 68:353-7, 2000
6. Woodruff JM: Pathology of tumors of the peripheral nerve
• Sciatic nerve, brachial or lumbosacral plexus
sheath in type 1 neurofibromatosis. Am J Med Genet.
o Sudden enlargement of preexisting neurofibroma in 89(1):23-30, 1999
NFl 7. Beggs I: Pictorial review: Imaging of peripheral nerve
o Usually presents as stage lIb lesion: 85% tumors. Clin Radiol. 52:8-17,1997
8. Kransdorf MJ et al: Imaging of soft tissue tumors, 1st ed.
Demographics Philadelphia PA, W.B. Saunders. 240-54, 1997
• Age 9. Celli P et al: Primary spinal malignant schwannomas:
o 40-50 years clinical and prognostic remarks. Acta Neurochir (Wien).
o If associated with Nfl: 26-42 years 135(1-2):52-5, 1995
• Gender 10. Bass JC et al: Retroperitoneal plexiform neurofibromas: CT
o M=F findings. AmJ Roentgenol. 163:617-20, 1994
11. Suh JS et al: Peripheral (extracranial) nerve tumors:
o In NFl: M > F Correlation of MR imaging and histologic findings.
Natural History & Prognosis Radiology. 183:341-6, 1992
12. Stull M et al: Magnetic resonance appearance of peripheral
• Local recurrence: 26-65% nerve sheath tumors. Skeletal Radiol. 20:9-14,1991
o 71% in primary spinal malignant schwannoma 13. Riccardi VM et al: Neurofibrosarcoma as a complication of
• Metastases: 20-65% von Recklinghausen neurofibromatosis. Neurofibromatosis.
o Lung, bone, pleura, liver 2:152-65, 1989
• 5 year survival: 10-50%
• NFl patients have worse prognosis
o Higher recurrence rate
o Shorter survival
• Better prognosis associated with
o Diameter < 5 cm
o Gross total resection
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MALIGNANT NERVE SHEATH TUMORS
I IMAGE GALLERY 1
97
Typical
(Left) Coronal STIR MR
shows an intraspinal mass
extending through
neuroforamen with a large
extra-spinal component
(arrow). A round left-sided
retroperitoneal mass is also
present. (Right) Coronal T7
C+ MR shows the same
enhancing MPNST with
intra- and extra-spinal
components. The left
retroperitoneal neurofibroma
is predominantly cystic with
linear enhancement.
Typical
(Left) Axial T7 C+ MR shows
a large lobulated
heterogeneously enhancing
retroperitoneal MPNST,
displacing abdominal aorta
and liver. (Right) Axial T7 C+
MR shows an enhancing
neurofibrosarcoma (open
arrow) eroding left vertebral
body and posterior element.
A left retrocrural
neurofibroma (arrow) also
enhances.
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CSF DISSEMINATED METASTASES
1
98
o Intramedullary nodule(s)
!TERMINOLOGY • Rarely due to CSF spread, impossible to
Abbreviations and Synonyms distinguish from hematogenous met(s) to cord
• Leptomeningeal carcinomatosis Radiographic Findings
• Leptomeningeal tumor spread • Myelography
• Drop metastases o Myelography, CT myelography
Definitions • "Filling defects"
• Spread of malignant tumors through the subarachnoid CT Findings
space • NECT: Often normal; +/- bony/extradural tumor
present
• CECT
IIMAGING FINDINGS o May be normal
General Features o Enhancement of cauda equina
• Best diagnostic clue: Smooth/nodular enhancement MR Findings
along cord, roots • TlWI
• Location: Any point along CSF pathway (including o Metastases usually isointense with cord, roots
central canal) o Extensive disease may fill thecal sac, elevating
• Size: Variable normally low CSF signal
• Morphology • CSF in sac has "ground glass" appearance
o Four basic patterns • Nerve roots appear blurred, "smudged"
• Solitary focal mass at bottom of thecal sac or • T2WI
along cord surface o Focal metastases usually isointense with cord, roots
• Diffuse, thin, sheet-like coating of cord/roots (hypointense to CSF)
("carcinomatous meningitis") o Thickened nerve roots
• "Rope-like" thickening of cauda equina • Tl C+
• Multifocal discrete nodules along cord/roots o Obvious enhancement
:l ~
if ,,
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~,,,
,,;
~
'".. -
'y
, , '.
,
.
Post-Operative 1/3 Meningitis Subdural tmpyema .. .
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CSF DISSEMINATED METASTASES
Key Facts
1
Terminology • Pyogenic meningitis (clinical/laboratory findings 99
• Leptomeningeal carcinomatosis helpful)
• Drop metastases • Granulomatous meningitis
• Chemical meningitis
Imaging Findings • Congenital hypertrophic polyradiculoneuropathies
• Obvious enhancement
Clinical Issues
• Pattern varies
• "Sugar coating" of cord, roots • Most common signs/symptoms: Severe pain,
• Single/multiple enhancing nodular masses polyradiculopathy
• Round/ovoid intramedullary mass, often with • Typically seen in advanced cancer cases
ring-like pattern Diagnostic Checklist
• Image entire neuraxis! • MRI more sensitive than CSF cytology; especially
• Contrast-enhanced, fat suppressed TIWI fat-saturated T1 C+
Top Differential Diagnoses • Post-operative enhancement of meninges can mimic
• Multifocal primary tumor leptomeningeal tumor spread
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IDIAGNOSTIC CHECKLIST
Consider
• MRI more sensitive than CSF cytology; especially
fat-saturated Tl C+
Image Interpretation Pearls
• Post-operative enhancement of meninges can mimic
leptomeningeal tumor spread
ISELECTED REFERENCES
1. Lindsay A et al: Spinal leptomeningeal metastases
following glioblastoma multiforme treated with
radiotherapy.] Clin Neurosci. 9(6): 725-8,2002
2. Singh SK et al: MR imaging of leptomeningeal metastases:
comparison of three sequences. A]NR Am] Neuroradiol.
23(5): 817-21, 2002
3. Straathof CS et al: The diagnostic accuracy of magnetic
resonance imaging and cerebrospinal fluid cytology in
leptomeningeal metastasis.] Neurol. 246(9): 810-4, 1999
4. Collie DA et al: Imaging features of leptomeningeal
metastases. Clin Radiol. 54(11): 765-71, 1999
5. van der Ree TC et al: Leptomeningeal metastasis after
surgical resection of brain metastases. ] Neurol Neurosurg
Psychiatry. 66(2): 225-7, 1999
6. Gomori]M et al: Leptomeningeal metastases: evaluation by
gadolinium enhanced spinal magnetic resonance imaging.
] Neurooncol. 36(1): 55-60, 1998
7. Kallmes DF et al: High-dose gadolinium-enhanced MRI for
diagnosis of meningeal metastases. Neuroradiology. 40(1):
23-6,1998
8. Formaglio F et al: Meningeal metastases: clinical aspects
and diagnosis. Ita!] Neurol Sci. 19(3): 133-49, 1998
9. Chamberlain MC et al: Carcinomatous meningitis
secondary to breast cancer: predictors of response to
combined modality therapy.] Neurooncol. 35(1): 55-64,
1997
10. Chamberlain MC: Comparative spine imaging in
leptomeningeal metastases. ] Neurooncol. 23(3): 233-8,
1995
11. Heinz R et al: Detection of CSF metastasis: CT myelography
or MR? A]NR. 16: 1147-51, 1995
12. Freilich R] et al: Neuroimaging and cerebrospinal fluid
cytology in the diagnosis of leptomeningeal metastasis.
Ann Neurol. 38(1): 51-7, 1995
13. Watanabe M et al: Correlation of MRI and clinical features
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IIMAGE GALLERY 1
101
(Left) Sagittal Tl C+ MR
shows thickened, enhancing
surface of the cord in patient
with metastatic breast
cancer, severe pain and
cervical polyradiculopathy.
(Right) Axial Tl C+ MR at
the level of the conus shows
diffuse enhancing rind of
tumor in patient with
metastatic melanoma.
Sagittal images depicted
extensive spread along entire
canal.
Variant
(Left) Axial Tl C+ MR
demonstrates enhancing
nodule on posterolateral
cord surface (arrow) in
patient recently treated for
pineal germinoma, indicating
single "drop" metastasis:
Resolved with radiation.
(Right) Sagittal TlWI MR
with fat-saturation depicts a
plaque-like mass on cord
surface (arrow) in patient
with recently treated pineal
germinoma, new cervical
radiculopathy due to "drop"
met.
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Sagittal graphic of cervical spine shows solid mass with Sagittal T7 C+ MR shows enhancing, exophytic cord
cystic component within cervical cord. mass. Note cystic, low signal component inferiorly
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ASTROCYTOMA, SPINAL CORD
Key Facts
1
Terminology Top Differential Diagnoses 103
• Intramedullary glioma • Ependymoma
• Other neoplasms
Imaging Findings • Metastasis (older patients)
• Best diagnostic clue: Enhancing infiltrating mass • Hemangioblastoma
expanding cord • Syringohydromyelia
• Location: Cervical> thoracic • Autoimmune or inflammatory myelitis
• Occasionally asymmetric, even exophytic • Cord ischemia/infarction
• Cord expansion • Dural vascular malformation
• Usually < 4 segments
• Occasionally multisegmental, even holocord (more Pathology
common with pilocytic astrocytoma) • Second most common cord neoplasm
• +/- Cyst/syrinx (fluid slightly hyperintense to CSF) • 80-90% low grade
• T2Wl: Hyperintense on PD, T2WI
• Almost always enhances Clinical Issues
• Focus of enhancement is target for biopsy • Slow onset of myelopathy
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ASTROCYTOMA, SPINAL CORD
1 Gross Pathologic & Surgical Features Image Interpretation Pearls
104 • Expanded cord • Axial, sagittal fat-saturated T1WI after contrast to
exclude dural or pial lesion inciting syringomyelia
Microscopic Features
• Fibrillary astrocytoma
o Increased cellularity, variable atypia/mitoses ISELECTED REFERENCES
o Parenchymal infiltration
1. Sun B et al: MRI features of intramedullary spinal cord
• Pilocytic astrocytoma ependymomas. J Neuroimaging. 13(4):346-51, 2003
o Rosenthal fibers, glomeruloid/hyalinized vessels 2. Saito R et al: Symptomatic spinal dissemination of
o Low prevalence of nuclear atypia/mitoses malignant astrocytoma. J Neurooncol. 61(3):227-35, 2003
3. Santi M et al: Spinal cord malignant astrocytomas.
Staging, Grading or Classification Criteria Clinicopathologic features in 36 cases. Cancer.
• 80-90% low grade 98(3):554-61,2003
o Fibrillary astrocytoma = WHO II 4. Arslanoglu A et al: MR imaging characteristics of
o Pilocytic astrocytoma = WHO I pilomyxoid astrocytomas. AJNR Am J Neuroradiol.
o Ganglioglioma, mixed gliomas also occur 24(9):1906-8, 2003
• 10-15% high grade 5. Brotchi]: Intrinsic spinal cord tumor resection.
o Most are anaplastic astrocytomas (WHO III) Neurosurgery. 50(5):1059-63, 2002
6. Kim MS et al: Intramedullary spinal cord astrocytoma in
o Glioblastoma (WHO IV) uncommon adults: postoperative outcome. J Neurooncol. 52(1):85-94,
2001
7. Constantini S et al: Radical excision of intramedullary
ICLINICAL ISSUES spinal cord tumors: surgical morbidity and long-term
follow-up evaluation in 164 children and young adults. J
Presentation Neurosurg (Spine 2). 93: 183-93, 2000
• Most common signs/symptoms 8. Houten JK et al: Spinal cord astrocytomas: presentation,
o Slow onset of myelopathy management and outcome. J Neurooncol. 47: 219-4, 2000
o Other signs/symptoms 9. Lowe GM: Magnetic resonance imaging of intramedullary
spinal cord tumors.J Neurooncol. 47(3):195-210, 2000
• May cause painful scoliosis 10. Miller DC: Surgical pathology of intramedullary spinal cord
• Clinical profile: Insidious onset of myelopathy in neoplasms. J Neurooncol. 47(3):189-94, 2000
adolescent or young adult 11. Strik HM et al: A case of spinal glioblastoma multiforme:
immunohistochemical study and review of the literature. J
Demographics Neurooncol. 50(3):239-43, 2000
• Age 12. Nishio S et al: Spinal cord gliomas: management and
o Most common intramedullary tumor in outcome with reference to adjuvant therapy. J Clin
children/young adults Neurosci. 7(1):20-3,2000
o 60% of IMSCTs in children are astrocytomas, 30% 13. Houten JK et al: Spinal cord astrocytomas: presentation,
ependymomas management and outcome. J Neurooncol. 47(3):219-24,
2000
• Gender: M > F = 1.3:1 14. Houten JK et al: Pediatric intramedullary spinal cord
Natural History & Prognosis tumors: special considerations. J Neurooncol. 47(3):225-30,
2000
• Most are slow-growing 15. Baleriaux DL: Spinal cord tumors. Eur Radiol. 9(7):1252-8,
• Malignant tumors may cause rapid neurologic 1999
deterioration 16. Bourgouin PM et al: A pattern approach to the differential
• Survival varies with tumor histology/grade, & gross diagnosis of intramedullary spinal cord lesions on MR
total resection imaging. AJR AmJ Roentgenol. 170(6):1645-9, 1998
o 80% 5 year for low grade; 30% for high grade 17. Squires LA et al: Diffuse infiltrating astrocytoma of the
• Post-operative neurologic function determined largely cervicomedullary region: clinicopathologic entity. Pediatr
by degree of pre-operative deficit Neurosurg. 27(3):153-9, 1997
18. Minehan KJ et al: Spinal cord astrocytoma: pathological
Treatment and treatment considerations. J Neurosurg. 83: 590-5, 1996
19. Nemoto Y et al: Intramedullary spinal cord tumors:
• Obtain tissue diagnosis significance of associated hemorrhage at MR imaging.
• Microsurgical resection (low grade tumors) Radiology. 182(3):793-6, 1992
o Intraoperative US, evoked potentials helpful 20. Li MH et al: MR imaging of spinal intramedullary tumors.
• Adjuvant therapy Acta Radiol. 32(6):505-13, 1991
o No evidence that XRT, chemotherapy improve long 21. Scotti G et al: Magnetic resonance diagnosis of
term outcome intramedullary tumors of the spinal cord. Neuroradiology.
29(2):130-5, 1987
IDIAGNOSTIC CHECKLIST
Consider
• MR at first suggestion of myelopathy
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ASTROCYTOMA, SPINAL CORD
IIMAGE GAllERY 1
105
(Left) Sagittal Tl WI MR
shows exophytic mass
expanding high cervical
cord, with cystic component
inferiorly. (Right) Sagittal
T2WI MR shows high signal
intensity of exophytic high
cervical mass, cystic
component inferiorly, and
diffuse abnormal signal
increase in lowest
component.
Typical
(Left) Sagittal Tl WI MR
shows fusiform expansion of
cord at C7 -C2, and
isointense enlargement of
cord below. (Right) Sagittal
Tl C+ MR shows cystic
expansion of cord at Cl-C2,
enhancing lesion below, and
isolhypointense cord below.
Biopsy was targeted to
enhancing lesion, proving
astrocytoma.
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Coronal graphic depicts an ependymoma centered in Sagittal T1 C+ MR shows solid enhancing component of
cervical cord with mild cord expansion. Cranial and ependymoma at C6 level. Note the rostral + caudal
rostral cyst as well as hemorrhagic products are nonenhancing tumor associated cysts.
associated with this mass.
• Myelography
ITERMINOlOGY o Fusiform cord enlargement
Abbreviations and Synonyms o Partial or complete block of intrathecal contrast
• Cord ependymoma CT Findings
Definitions • NECT
o Spinal canal widening
• Neoplasm of ependyma lining spinal cord central
canal • Thinned pedicles
• Widened interpediculate distance
• Posterior vertebral scalloping
IIMAGING FINDINGS • CECT: Symmetrically enlarged spinal cord with
well-circumscribed enhancement
General Features MR Findings
• Best diagnostic clue: Circumscribed, enhancing cord
mass with hemorrhage • TlWI
o Isointense or slightly hypointense to spinal cord
• Location: Cervical> thoracic> conus o Hemorrhage hyperintense
• Size o Cord atrophy may be present
o Multisegmental • Correlates with surgical morbidity
• Typically 3-4 segments
• T2WI
• Morphology o Hyperintense
o Well-circumscribed o Polar (rostral or caudal) or intratumoral cysts:
o Symmetric cord expansion 50-90%
o May have exophytic component
• Similar to CSF intensity
Radiographic Findings o Syrinx
• Radiography o Focal hypointensity: Hemosiderin
o Central canal widening: 20% • "Cap sign": Hemosiderin at cranial or caudal
o Posterior vertebral scalloping margin
o Scoliosis • 20-64% of cord ependymomas
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EPENDYMOMA, CELLULAR, SPINAL CORD
1 o Second most common primary spinal cord tumor in
children
• Chemotherapy for failed surgery and radiotherapy
o Unproven benefit
108 • Associated abnormalities
o Subarachnoid hemorrhage
o Superficial siderosis IDIAGNOSTIC CHECKLIST
o NF2
• Schwannomas Image Interpretation Pearls
• Meningiomas • Associated peripheral hemorrhage suggestive of cord
ependymoma
Gross Pathologic & Surgical Features
• Soft red or grayish-purple mass
o Small blood vessels on tumor surface ISELECTED REFERENCES
• Well-circumscribed
1. Chamberlain MC: Ependymomas. Curr Neurol Neurosci
o May be encapsulated
Rep. 3(3):193-9, 2003
• Cystic change common 2. Sun B et al: MRI features of intramedullary spinal cord
• Hemorrhage at tumor periphery ependymomas.] Neuroimaging. 13(4):346-51, 2003
3. Carter M et al: Genetic abnormalities detected in
Microscopic Features ependymomas by comparative genomic hybridisation. Br]
• Perivascular pseudorosettes Cancer. 86(6):929-39, 2002
• True ependymal rosettes less common 4. ]euken]W et al: Correlation between localization, age, and
• Moderate cellularity with low mitotic activity chromosomal imbalances in ependymal tumours as
• Occasional nuclear atypia detected by CGH.] Patho!. 197(2):238-44, 2002
5. Chang UK et al: Surgical outcome and prognostic factors of
• Rare to no mitoses
spinal intramedullary ependymomas in adults. ]
• Immunohistochemistry: Positive for GFAP, S-100, Neuroonco!. 57(2):133-9, 2002
vimentin 6. Hanbali F et al: Spinal cord ependymoma: radical surgical
resection and outcome. Neurosurgery. 51(5):1162-72;
Staging, Grading or Classification Criteria discussion 1172-4, 2002
• Most are WHO grade II 7. Choi]Y et al: Intracranial and spinal ependymomas: review
• Rare: WHO grade III ofMR images in 61 patients. Korean] Radio!. 3(4):219-28,
o Anaplastic ependymoma 2002
8. Hirose Y et al: Chromosomal abnormalities subdivide
ependymal tumors into clinically relevant groups. Am]
ICLINICAL ISSUES 9.
Patho!. 158(3):1137-43,2001
Wiestler OD et al: Ependymoma. In Kleihues P, Cavanee
Presentation WK (eds), Pathology & Genetics of Tumors of the Central
Nervous System, 72-7. IARC Press, 2000
• Most common signs/symptoms 10. Lagares A et al: Spinal cord ependymoma presenting with
o Neck or back pain acute paraplegia due to tumoral bleeding. ] Neurosurg Sci.
o Other signs/symptoms 44(2):95-7; discussion 97-8, 2000
• Progressive paraparesis 11. Koeller KK et al: Neoplasms of the spinal cord and filum
• Paresthesia terminale: Radiologic-pathologic correlation. Radiographies
• Clinical profile 20: 1721-49,2000
o Delay in diagnosis due to slow growth 12. Miyazawa N et al: MRI at 1.5 T of intramedullary
ependymoma and classification of pattern of contrast
• Average duration of symptoms before diagnosis:
enhancement. Neuroradiology. 42(11):828-32, 2000
2.5 yrs 13. Mazewski C et al: Karyotype studies in 18 ependymomas
Demographics with literature review of 107 cases. Cancer Genet
Cytogenet. 113(1):1-8, 1999
• Age 14. Graf M et al: Extraneural metastasizing ependymoma of
o 35-45 yo the spinal cord. Pathol Oncol Res. 5(1):56-60, 1999
• Mean age at presentation: 39 yo 15. Bourgouin PM et al: A pattern approach to the differential
• Gender: Slight female predilection diagnosis of intramedullary spinal cord lesions on MR
• Ethnicity: No racial predilection imaging. A]R Am] Roentgeno!. 170(6):1645-9, 1998
16. Kahan H et al: MR characteristics of histopathologic
Natural History & Prognosis subtypes of spinal ependymoma. A]NR 17: 143-50, 1996
• Less pre-operative neurologic deficit at presentation, 17. Fine M] et al: Spinal cord ependymomas: MR imaging
better post-operative outcome features. Radiology. 197(3):655-8, 1995
18. Waldron]N et al: Spinal cord ependymomas: a
• Thoracic tumors have worse surgical outcome retrospective analysis of 59 cases. Int] Radiat Oncol BioI
• Rarely metastasis Phys. 27(2):223-9, 1993
o Lung, skin, kidney, lymph nodes 19. Nemoto Y et al: Intramedullary spinal cord tumors:
• 5 year survival: 85% significance of associated hemorrhage at MR imaging.
Radiology. 182(3):793-6, 1992
Treatment
• Surgical resection
o Gross total resection in > 85% of cases
• Radiotherapy for subtotal resection or recurrent
disease
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Typical
(Left) Sagittal T2WI MR of
cervical spine shows a
hyperintense expansile
intramedullary mass with
caudal edema (arrow). A
cystic component is present
in the medulla (open arrow).
(Right) Sagittal T7 C+ MR in
the same patient shows a
heterogenously enhancing
mass from C2-5. Biopsy
reveals an ependymoma.
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Sagittal graphic shows cauda equina myxopapillary Sagittal T2WI MR (left) and TI C+ MR (right) show a
ependymoma, enlarging spinal canal & remodeling predominantly cystic cauda equina mass with
vertebral cortex. Mass is vascular, w/old intratumoral associated subacute hemorrhage (arrows). A ME was
hemorrhage & acute SAH. found at surgery (Courtesy A. Osborn, MO).
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EPENDYMOMA, MYXOPAPILLARY, SPINAL CORD
Key Facts
1
Terminology Top Differential Diagnoses 111
• Slow growing glioma arising from ependymal cells of • Nerve sheath tumor (NST)
filum terminale • Intradural metastases
• Acquired epidermoid
Imaging Findings
• Best diagnostic clue: Enhancing cauda equina mass Pathology
with hemorrhage • ME most common subtype to hemorrhage
• Almost exclusively in conus, filum terminale, cauda • 90% of filum terminale tumors
equina • Acute non-aneurysmal subarachnoid hemorrhage
• Well circumscribed • Superficial siderosis
• Isodense intradural mass
• +/- Bony canal expansion Clinical Issues
• Hyperintensity due to accumulation of mucin • Back pain
• Hypointensity at tumor margin: Hemosiderin • Average duration of symptoms prior to diagnosis: 2 yr
• Tl C+: Intense enhancement
MR Findings Meningioma
• TlWI • Usually isointense with cord on Tl and T2WI
o Usually isointense with cord • More common in thoracic, cervical spine
o Hyperintensity due to accumulation of mucin o Conus/filum location unusual
• T2WI • Hemorrhage uncommon
o Almost always hyperintense to cord • Bony changes rare
o Hypointensity at tumor margin: Hemosiderin
• 70% of intradural spine tumors with hemorrhage Paraganglioma
are ependymomas • Rare tumor of cauda equina
o May see flow voids • May be highly vascular
• STIR: Hyperintense o Indistinguishable from myxopapillary ependymoma
• Tl C+: Intense enhancement • Usually smaller
• Rare: Destructive extradural sacrococcygeal lesion
Imaging Recommendations I PATHOLOGY
• Best imaging tool: Sagittal and axial T2WI and TlWI
MRI with gadolinium General Features
• Protocol advice: Always include the conus in patients • General path comments
with back pain o Four epemdymoma subtypes: Cellular,
myxopapillary, clear-cell, tanycytic
• Cellular most common intramedullary tumor
IDIFFERENTIAL DIAGNOSIS subtype
• ME most common subtype to hemorrhage
Nerve sheath tumor (NST) • 10-40% of MEs are multiple
• Large, multilevel NST may be indistinguishable • Genetics
• Small tumor associated with nerve root rather than o Spinal ependymomas genetically different from
filum terminale intracranial ependymomas
• Usually extends through neural foramina o No consistent alterations
• Hemorrhage less common • Tumor genetics: Extra copies of chromosomes 9
and 18 have been reported
Intradural metastases • Etiology
• Smooth or nodular enhancement along conus and o ME originates from ependymal cells of filum
nerve roots terminale
• Enhancing masses o Subcutaneous sacrococcygeal ME arises from
o Usually multiple coccygeal medullary vestige
Acquired epidermoid • Ependyma lined cavity of caudal neural tube at
the postanal pit
• Hypointense on Tl WI
• Hyperintense on T2WI: Similar to CSF • Epidemiology
o Ependymoma: 4% of all primary central nervous
• No enhancement
system neoplasms in adults
• Typically small
• 30% of ependymomas are spinal
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EPENDYMOMA, MYXOPAPILLARY, SPINAL CORD
1 o Cord ependymoma: Most common primary spinal o Gross total resection in > 85% of cases
cord tumor in adults • Radiotherapy in recurrence or subtotal resection
112 • 60% of primary spinal cord neoplasms • Adjuvant therapy for multifocallesions
o ME: 27-30% of all ependymomas
• Most common tumor of conus, filum terminale,
cauda equina IDIAGNOSTIC CHECKLIST
• 90% of filum terminale tumors
• Associated abnormalities Consider
o Acute non-aneurysmal subarachnoid hemorrhage • Scanning up to at least mid-thoracic spine if conus
o Superficial siderosis lesion found
Gross Pathologic & Surgical Features Image Interpretation Pearls
• Soft, lobulated, ovoid • T1 hyperintense, enhancing, hemorrhagic mass
• Grayish white surface associated with filum terminale highly suggestive of
• Noninfiltrating, often encapsulated ME
• May be highly vascular
Microscopic Features ISELECTED REFERENCES
• Spindle, columnar, cuboidal tumor cells with radial Mahler-Araujo MB et al: Structural genomic abnormalities
1.
perivascular arrangement of chromosomes 9 and 18 in myxopapillary
• Fibrous, mucoid matrix ependymomas. J Neuropathol Exp Neurol. 62(9):927-35,
• Cysts, hemorrhage, calcifications common 2003
• Absent, low mitotic activity 2. Sun B et al: MRI features of intramedullary spinal cord
o MIB 0.4-1.6q1o ependymomas. J Neuroimaging. 13(4):346-51, 2003
• Immunohistochemistry: GFAP, S-100, vimentin 3. Akpolat N et al: Sacrococcygeal extraspinal ependymoma: a
positive case report. TurkJ Pediatr. 45(3):276-9, 2003
4. Hanbali F et al: Spinal cord ependymoma: radical surgical
o Cytokeratin negative
resection and outcome. Neurosurgery. 51(5):1162-72;
Staging, Grading or Classification Criteria discussion 1172-4, 2002
5. Choi]Y et al: Intracranial and spinal ependymomas: review
• WHO grade I1IV of MR images in 61 patients. Korean] Radiol. 3(4):219-28,
• May have local seeding 2002
o Subarachnoid dissemination 6. Bavbek M et al: Lumbar myxopapillary ependymoma
• No malignant degeneration mimicking neurofibroma. Spinal Cord. 39(8):449-52, 2001
7. Wiestler OD et al: Myxopapillary ependymoma. In
Kleihues P, Cavenee WK (eds): Tumors of the Central
ICLINICAL ISSUES 8.
Nervous System, 78-9. IARC Press, 2000
Wager M et al: Cauda equina tumors: a French multicenter
Presentation retrospective review of 231 adult cases and review of the
literature. Neurosurg Rev. 23(3):119-29; discussion 130-1,
• Most common signs/symptoms 2000
o Back pain 9. Chung JY et al: Subcutaneous sacrococcygeal
o Other signs/symptoms myxopapillary ependymoma. A]NR Am J Neuroradiol.
• Paraparesis 20(2):344-6, 1999
• Radiculopathy 10. Asazuma T et al: Ependymomas of the spinal cord and
• Bladder and bowel dysfunction: 20-25% cauda equina: An analysis of 26 cases and a review of the
• Clinical profile literature. Spinal Cord. 37(11):753-9, 1999
o Symptoms mimic disc herniation 11. Rickert CH et al: Ependymoma of the cauda equina. Acta
Neurochir (Wien). 141(7):781-2, 1999
o Delay in diagnosis due to slow tumor growth
12. Friedman DP et al: Neuroradiology case of the day.
• Average duration of symptoms prior to diagnosis: Radiographics. 18: 794-8, 1998
2 yr 13. Yamada CY et al: Myxopapillary ependymoma of the filum
terminale. A]R Am J Roentgenol. 168(2):366, 1997
Demographics 14. Kahan H et al: MR characteristics of histopathologic
• Age subtypes of spinal ependymoma. A]NR Am] Neuroradiol.
o Broad age range: Reported at all ages 17(1):143-50, 1996
• Peak: 30-40 yo 15. Kline M] et al: Extradural myxopapillary ependymoma:
• Gender: M:F = 2:1 report of two cases and review of the literature. Pediatr
• Ethnicity: No racial predilection Pathol Lab Med. 16(5):813-22, 1996
16. Wippold F] II et al: MR imaging of myxopapillary
Natural History & Prognosis ependymoma. A]R. 165: 1263-7, 1995
• Late recurrence, distant metastases uncommon after 17. Shen WC et al: Ependymoma of the cauda equina
presenting with subarachnoid hemorrhage. A]NR Am J
complete resection Neuroradiol. 14(2):399-400, 1993
• Risk of local recurrence if incomplete resection 18. Parenti G et al: Primary cauda equina tumors. J Neurosurg
• Excellent prognosis with complete resection Sci. 37(3):149-56, 1993
Treatment
• Resection
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(Left) Sagittal T7 C+ MR in
same patient demonstrates
homogeneous enhancement
in the cauda equina mass,
proven to be a myxopapillary
ependymoma. (Right)
Sagittal T7WI MR with fat
suppression in another
patient shows an intensely
enhancing myxopapillary
ependymoma in lumbosacral
thecal sac.
Typical
(Left) Sagittal
PO/Intermediate MR shows
a subtle mass filling thecal
sac from L4 to L5, with a
linear hypointense signal
(arrow). Post-surgical
changes are evident
posteriorly. (Right) Sagittal
T7 C+ MR in same patient
demonstrates the mass with
diffuse enhancement. Biopsy
showed a myxopapillary
ependymoma.
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HEMANGIOBLASTOMA, SPINAL CORD
1
114
Sagittal graphic shows focal intramedullary mass in Sagittal T7 C+ MR shows two small enhanCing
cervical cord with prominent feeding vessels, associated intradural nodules on dorsal pial surface of cervical
cyst, and cord expansion/edema. cord.
!TERMINOLOGY CT Findings
• NECT: Intramedullary mass +/- expanded/remodeled
Abbreviations and Synonyms spinal canal
• Capillary hemangioblastoma (HB); von Hippel-Lindau • CECT: May demonstrate enhancing nodule
syndrome (VHL) • CTA: May show enlarged feeding vessels, enhancing
Definitions mass
• Low grade, capillary rich neoplasms of cerebellum and MR Findings
spinal cord that occur sporadically or in setting of von
• TlWI
Hippel-Lindau syndrome o Depends on lesion size, presence of syrinx
o Small
• Isointense with cord (may be invisible unless
IIMAGING FINDINGS hemorrhage has occurred)
General Features • Well-delineated syrinx (hypointense) present in >
50%
• Best diagnostic clue: Intramedullary mass with
o Large
serpentine "flow voids"
• Mixed hypo/isointense
• Location • Lesions = > 2.5 cm almost always show "flow
o Subpial
voids" (enlarged feeding arteries and/or draining
o Posterior aspect of the spinal cord, often associated
veins)
with intraspinal cyst
o Rarely anterior aspect of cord • T2WI
o Small lesions usually uniformly hyperintense
• Size o +/- Peritumoral edema
o Few mm to several cm
o Syrinx fluid often slightly hyperintense to CSF
o Multiple tumors (often small) in VHL
o Mixed hyperintense (flow voids, hemorrhage
• Morphology: Round, well-defined margins
common)
o May show extensive, long segment cord edema
without syrinx
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Key Facts
1
Terminology • Hypervascular cord neoplasms 115
• Low grade, capillary rich neoplasms of cerebellum • Intradural/extramedullary tumors
and spinal cord that occur sporadically or in setting Pathology
of von Hippel-Lindau syndrome • 1-5% of all spinal cord neoplasms
Imaging Findings • 75% of spinal HBs are sporadic (25% VHL-associated)
• Best diagnostic clue: Intramedullary mass with • Often multiple (VHL usually has one large +/- many
serpentine "flow voids" small HBs)
• Subpial • Cerebellar HBs, retinal angiomas,
• Posterior aspect of the spinal cord, often associated pheochromocytoma, renal cell carcinoma,
with intraspinal cyst angiomatous or cystic lesions of the kidneys,
• May show extensive, long segment cord edema pancreas, and epididymis
without syrinx • HBs are WHO grade I
• T1 C+
o Small Intradural/extramedullary tumors
• Subpial nodule (often on surface of dorsal cord) • Meningioma/schwannoma rare associated syrinx,
• Well-demarcated, intense, homogeneous uncommon flow voids
enhancement • Paraganglioma (filum> > cord but may be
o Large indistinguishable)
• Heterogeneous enhancement
• If syrinx present, wall doesn't enhance
IPATHOLOGY
Angiographic Findings
• Conventional General Features
o Intense, prolonged vascular stain • General path comments
o +/- A-V shunting o VHL phenotypes
o Enlarged spinal arteries (anterior> posterior) supply • Type 1 = without pheochromocytoma
mass • Type 2A = with pheochromocytoma, renal cell
carcinoma (RCe)
Other Modality Findings • Type 2B = with pheochromocytoma, no RCC
• Intraoperative sonography may be useful in locating • Genetics
nodule o Familial HB (VHL)
Imaging Recommendations • Autosomal dominant
• Chromosome 3p, other gene mutations common
• Contrast-enhanced MR • VEGF highly expressed
o Scan brain, entire spine in patients with
• Erythropoietin often upregulated
known/suspected VHL
• Tumors arise after the loss or inactivation of wild
• DSA for large lesions/pre-operative embolization
type allele in a cell
• 20% of VHL families no deletion or mutation can
be detected
I DIFFERENTIAL DIAGNOSIS o Sporadic HB (unknown origin)
Arteriovenous malformation (AVM) • Etiology: Suppressor gene product (VHL protein)
• Cord often normal/small, gliotic causes neoplastic transformation
• Syrinx, focal nodule absent • Epidemiology
o 1-5% of all spinal cord neoplasms
Cavernous malformation o 75% of spinal HBs are sporadic (25%
• Mottled or speckled pattern of prior hemorrhage, VHL-associated)
hemosiderin rim o Often multiple (VHL usually has one large +/- many
• Minimal enhancement small HBs)
• Associated abnormalities
Hypervascular cord neoplasms o VHL
• Ependymoma (mass centrally located; no syrinx) • Cerebellar HBs, retinal angiomas,
• Astrocytoma (usually not hypervascular; peritumoral pheochromocytoma, renal cell carcinoma,
edema common) angiomatous or cystic lesions of the kidneys,
• Vascular metastasis (known primary, e.g., renal cell pancreas, and epididymis
carcinoma)
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Typical
(Left) Anteroposterior
angiography shows enlarged
anterior spinal artery feeding
hypervascular mass at the
the thoracic lumbar junction
(arrow). (Right) Sagittal T1
C+ MR shows focal
enhancing intradural mass
with superior cyst (arrow)
within conus.
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Metastatic involement of skeleton dorsal, epidural space Sagittal T1 C+ MR (L) and sagittal T2WI MR (R) show
and cord: Note the hemorhagic intramedullary focal enhancing oval lesion in cord with edema above
metastasis expanding the cord. and below. Patient had breast metastases and
developed paresis.
ITERMINOlOGY CT Findings
• CECT: May rarely depict hypervascular mets as
Abbreviations and Synonyms enhancing intraspinal foci (e.g., from hypernephroma)
• Intramedullary spinal cord metastatic disease
• ISCM, ISM MR Findings
Definitions • TIWI
o Enlarged cord
• Metastatic lesion from primary carcinoma in another o Rarely, syrinx cavity
organ (including brain)
• T2WI
o Focal high signal
o Diffuse edema
IIMAGING FINDINGS o Rarely syrinx
General Features o Rarely, low signal due to hemorrhagic metastasis
(e.g., from thyroid, melanoma)
• Best diagnostic clue: Focal, enhancing cord lesion(s)
• PD/Intermediate: As T2WI
with extensive edema
• T2* GRE: Highlights hemorrhagic focus
• Location
• TI C+: Focal enhancement
o Any cord segment
o Conus least common Ultrasonographic Findings
• Size: Typically small « 1.S cm) • Real Time
• Morphology o Helps in surgical removal
o Well-circumscribed o Focal hyperechoic lesion
o Spherical or oval
Angiographic Findings
Radiographic Findings • Conventional: Helps differentiate AVM or
• Myelography hemangioblastoma from metastasis
o May miss the lesion in substantial minority of cases
o When positive, depicts focal cord swelling
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SPINAL CORD METASTASES
1 ICLINICAL ISSUES 9.
102(4):249-254, 2000
Ateaque A et al: Intramedullary spinal cord metastases
120 from a hypernephroma 11 years follOWing the diagnosis
Presentation and treatment of the primary lesion. Br] Neurosurg.
• Most common signs/symptoms 14(5):474-6,2000
o Rapidly progressive flaccid paraparesis 10. Keung YK et al: Secondary syringomyelia due to
o Other signs/symptoms intramedullary spinal cord metastasis. Case report and
• Sphincter disturbance review of literature. Am] Clin Oncol. 20(6):577-9,1997
11. Phuphanich S et al: Magnetic resonance imaging of syrinx
• Pain
associated with intramedullary metastases and
• Hypesthesia
leptomeningeal disease.] Neuroimaging. 6(2):115-7, 1996
• Brown-Sequard syndrome 12. Honma Y et al: Intramedullary spinal cord and brain
• Clinical profile metastases from thyroid carcinoma detected 11 years after
o Most cases have known primary CA and develop initial diagnosis--case report. Neurol Med Chir (Tokyo).
sudden myelopathy 36(8):593-7, 1996
o In 20% of ISCM, this is first presentation of cancer 13. Schiff D et al: Intramedullary spinal cord metastases:
clinical features and treatment outcome. Neurology.
Demographics 47(4):906-12, 1996
• Age: Any, typically over 50 14. Connolly ES]r et al: Intramedullary spinal cord metastasis:
• Gender: No gender preference report of three cases and review of the literature. Surg
Neurol. 46(4):329-37; discussion 337-8, 1996
Natural History & Prognosis 15. Shibamoto Y et al: Spinal metastasis from occult
• ISCM heralds poor prognosis intracranial germinoma mimicking primary intramedullary
tumour. Neuroradiology. 36(2):137-8, 1994
• Minority survive more than one year 16. Raco A et al: Intramedullary metastasis of unknown origin:
Treatment a case report. Neurosurg Rev. 15(2):135-8, 1992
17. Tognetti F et al: Metastases of the spinal cord from remote
• Options, risks, complications neoplasms. Study of five cases. Surg Neurol. 30(3):220-7,
o Steroids, chemotherapy, radiation therapy 1988
o Surgery an option in solitary cases 18. Van Velthoven V et al: Intramedullary spread of a cerebral
• Goal of therapy is stabilization or reversal of oligodendroglioma. Surg Neurol. 30(6):476-81, 1988
myelopathy, and preservation of ambulation 19. Winkelman MD et al: Intramedullary spinal cord
metastasis. Diagnostic and therapeutic considerations.
Arch Neurol. 44(5):526-31, 1987
Foster a et al: Syrinx associated with intramedullary
I DIAGNOSTIC CHECKLIST 20.
metastasis.] Neurol Neurosurg Psychiatry. 50(8):1067-70,
Consider 1987
• Full craniospinal imaging when focal cord lesion
found
Image Interpretation Pearls
• Edema out of proportion to focal small cord lesion
suggests metastasis, even if solitary
ISELECTED REFERENCES
1. Aryan HE et al: Intramedullary spinal cord metastasis of
lung adenocarcinoma presenting as Brown-Sequard
syndrome. Surg Neurol. 61(1):72-6, 2004
2. Reddy P et al: Intramedullary spinal cord metastases: case
report and review of literature. ] La State Med Soc.
155(1):44-5, 2003
3. Ogino M et al: Successful removal of solitary
intramedullary spinal cord metastasis from colon cancer.
Clin Neural Neurosurg. 104(2):152-6,2002
4. Potti A et al: Intramedullary spinal cord metastases (ISCM)
and non-small cell lung carcinoma (NSCLC): clinical
patterns, diagnosis and therapeutic considerations. Lung
Cancer. 31(2-3):319-23, 2001
5. Fakih M et al: Intramedullary spinal cord metastasis (ISCM)
in renal cell carcinoma: a series of six cases. Ann Oncol.
12(8):1173-7, 2001
6. Mortimer N et al: Intramedullary spinal cord metastasis.
Lancet Oncol. 2(10):607, 2001
7. Mathur S et al: Late intramedullary spinal cord metastasis
in a patient with lymphoblastic lymphoma: case report. ]
Clin Neurosci. 7(3):264-8, 2000
8. Schijns OE et al: Intramedullary spinal cord metastasis as a
first manifestation of a renal cell carcinoma: report of a
case and review of the literature. Clin Neurol Neurosurg.
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SPINAL CORD METASTASES
IIMAGE GAllERY 1
121
(Left) Anteroposterior
myelography demonstrates
focal swelling of the conus in
paretic patient with
subsequently found breast
metastases from primary
tumor treated 10 years ago.
(Right) Sagittal CT
reformation post
myelography shows focally
swollen conus in paretic
patient with history of breast
cancer treated 10 years ago.
Metastases elsewhere were
found after conus biopsy.
Variant
(Left) Sagittal T2WI MR
shows diffuse swelling of
cervico-thoracic cord with
high signal and focal low
signal zones indicating
hemorrhage. Patient had
metastatic lung
adenocarcinoma. (Right)
Sagittal T1 C+ MR shows
diffuse cervico-thoracic cord
swelling and focal enhancing
lesion at T2 in patient with
metastatic adenocarcinoma
of the lung.
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PARAGANGLIOMA
1
122
Sagittal TlWI MR shows well-defined intradural Sagittal Tl C+ MR shows intradural extramedullary mass
extramedullary mass spanning multiple cervical markedly enhancing.
segments, with cord compression.
ITERMINOlOGY CT Findings
Abbreviations and Synonyms • NECT
o Large tumors may show bony remodeling, even
• Paraganglioma (PG), chemodectoma, glomus tumor erosion
(terminology based on anatomic site) o Rare presentation = destructive intraosseous mass
Definitions (usually in sacrum)
• Extra adrenal, spinal paraganglioma composed of • CECT: May demonstrate enhancing mass below
chromaffin cells (groups qf cells associated to the conus/along filum
autonomous system) MR Findings
• TlWI
o Imaging features nonspecific (vascular intradural
IIMAGING FINDINGS extramedullary mass)
General Features o Well-delineated round/ovoid/lobulated mass
o Isointense, mixed isointense/hypointense compared
• Best diagnostic clue: Vascular cauda equina mass
to cord
• Location o Prominent "flow voids" common
o Spine is rare extra-adrenal site of paraganglioma
o Most common in cauda equina • T2WI
o Hyperintense +/- blood products, hemosiderin rim
. 0 Rarely occur cervical, thoracic spine
or "cap"
• Size: 10 =? > SO mm o Prominent flow voids
• Morphology: Well-defined intradural extramedullary
mass • Tl C+
o Intense homogeneous enhancement
Radiographic Findings • Rare: Demonstrates multiple "uphill" intradural
• Myelography metastases
o Smooth/lobulated intradural extramedullary mass Angiographic Findings
o +/- Serpentine filling defects (large arteries, draining
• Conventional: Hypervascular mass
veins)
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PARAGANGLIOMA
Key Facts
1
Terminology Pathology 123
• Paraganglioma (PG), chemodectoma, glomus tumor • General path comments: Can be difficult to
(terminology based on anatomic site) distinguish PG from ependymoma using only light
• Extra adrenal, spinal paraganglioma composed of microscopy, standard H&E stains
chromaffin cells (groups of cells associated to the • PGs originate from neural crest cells associated with
autonomous system) segmental or collateral autonomic ganglia
("paraganglia") throughout body
Imaging Findings • "APUD" cell tumors (Amine Precursor Uptake and
• Best diagnostic clue: Vascular cauda equina mass Decarboxylation)
• Morphology: Well-defined intradural extramedullary • Most neural crest tumors occur in adrenal medulla
mass (pheochromocytoma)
Top Differential Diagnoses • 80-90% of extra-adrenal paragangliomas occur
in/near carotid body, jugular bulb
• Myxopapillary ependymoma
• Spine is uncommon site
• Schwannoma
• Meningioma
• Metastasis
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PARAGANGLIOMA
1 • Electron microscopy shows dense core neurosecretory
7.
report of three cases.] Korean Med Sci. 16(4):522-6, 2001
Masuoka] et al: Germline SDHD mutation in
granules
124 paraganglioma of the spinal cord. Oncogene.
Staging, Grading or Classification Criteria 20(36):5084-6, 2001
• WHO grade I 8. Lalloo ST et al: Clinics in diagnostic imaging (68).
• Rare aggressive, malignant spinal paragangliomas have Intradural extramedullary spinal paraganglioma. Singapore
Med]. 42(12):592-5, 2001
been reported
9. Hamilton MA et al: Metastatic paraganglioma causing
spinal cord compression. Br] Radiol. 73(872):901-4,2000
10. Sundgren P et al: Paragangliomas of the spinal canal.
ICLINICAL ISSUES Neuroradiology. 41(10):788-94, 1999
11. Herman M et al: Paraganglioma of the cauda equina: case
Presentation report and review of the MRI features. Acta Univ Palacki
• Most common signs/symptoms Olomuc Fac Med. 141:27-30, 1998
o Spinal paragangliomas have little/no secretory 12. Ashkenazi E et al: Paraganglioma of the filum terminale:
activity case report and literature review. ] Spinal Disord.
11(6):540-2, 1998
o Most common symptom = back/lower extremity 13. Herman M et al: Paraganglioma of the cauda equina: case
pain report and review of the MRI features. Acta Univ Palacki
o Symptom duration varies from days to years Olomuc Fac Med. 141:27-30, 1998
o Other signs/symptoms 14. Hopster D] et al: Widespread neuroendocrine malignancy
• Sensory/motor loss, bowel/bladder dysfunction within the central nervous system: a diagnostic
• Clinical profile: Middle-aged patient with myelopathy, conundrum.] Clin Pathol. 50(5):440-2, 1997
intradural extramedullary enhancing mass 15. Moran CA et al: Primary spinal paragangliomas: a
clinicopathological and immunohistochemical study of 30
Demographics cases. Histopathology. 31(2):167-73, 1997
16. Faro SH et al: Paraganglioma of the cauda equina with
• Age associated intramedullary cyst: MR findings. A]NR Am]
o Range from 13 =;> 70 years
Neuroradiol. 18(8):1588-90, 1997
o Average = 45-50 years 17. Roche PH et al: Cauda equina paraganglioma with
• Gender: M = F subsequent intracranial and intraspinal metastases. Acta
Neurochir (Wien). 138(4):475-9, 1996
Natural History & Prognosis 18. Rees]H et al: Paragangliomas of the cauda equina. I]NR 2:
• Slow-growing, generally benign behavior 242-50, 1996
• Varies with tumor location (generally excellent for 19. Fitzgerald LF et al: Paraganglioma of the thoracic spinal
spinal PGs) cord. Clin Neurol Neurosurg. 98(2):183-5, 1996
• Recurrence < 5% after gross total removal 20. Campbell L et al: Improved detection of disseminated
pheochromocytoma using post therapy 1-131 MIBG
Treatment scanning. Clin Nucl Med. 21(12):960-3, 1996
• May require pre-operative embolization 21. Brodkey]A et al: Metastatic paraganglioma causing spinal
• Surgical excision usually curative cord compression. Spine. 20(3):367-72, 1995
22. Patel SR et al: A IS-year experience with chemotherapy of
patients with paraganglioma. Cancer. 76(8):1476-80, 1995
23. Steel TR et al: Paraganglioma of the cauda equina with
IDIAGNOSTIC CHECKLIST associated syringomyelia: case report. Surg Neurol.
42(6):489-93, 1994
Consider 24. Boukobza M et al: Paraganglioma of the cauda equina:
• MR features nonspecific magnetic resonance imaging. Neuroradiology.
35(6):459-60, 1993
Image Interpretation Pearls 25. Singh RV et al: Paraganglioma of the cauda equina: a case
• Prominent flow voids with intradural extramedullary report and review of the literature. Clin Neurol Neurosurg.
enhancing lesion most often ependymoma 95(2):109-13, 1993
26. Massey V et al: Treatment of metastatic chemodectoma.
Cancer. 69(3):790-2, 1992
ISELECTED REFERENCES 27. Pande AK: Malignant pheochromocytoma. Int] Cardiol.
34(3):346-8, 1992
1. Lazaro B et al: Malignant paraganglioma with vertebral 28. Cybulski GR et al: Spinal cord compression from a thoracic
metastasis: case report. Arq Neuropsiquiatr. 61(2B):463-7, paraganglioma: case report. Neurosurgery. 28(2):306-9,
2003 1991
2. Jeffs G] et al: Functioning paraganglioma of the thoracic 29. Yoshida A et al: Paraganglioma of the cauda equina. A case
spine: case report. Neurosurgery. 53(4):992-4; discussion report and review of the literature. Acta Pathol ]pn.
954-5,2003 41(4):305-10, 1991
3. U-King-Im]M et al: Vertebral metastatic chemodectoma: 30. North CA et al: Multiple spinal metastases from
imaging and therapeutic octreotide. Case report. ] paraganglioma. Cancer. 66(10):2224-8, 1990
Neurosurg. 97(1 Suppl):106-9, 2002 31. Parnell AP et al: Extradural metastases from
4. Houten]K et al: Thoracic paraganglioma presenting with paraganglionomas, report of two cases. Clin Radiol.
spinal cord compression and metastases. ] Spinal Disord 39(1):65-8, 1988
Tech. 15(4):319-23,2002 32. Sato Y et al: Hippel-Lindau disease: MR imaging. Radiology.
5. Kim SH et al: Oncocytoma of the spinal cord. Case report. ] 166(1 Pt 1):241-6, 1988
Neurosurg. 94(2 Suppl):310-2, 2001
6. Shin]Y et al: MR findings of the spinal paraganglioma:
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PARAGANGLIOMA
IIMAGE GALLERY 1
125
(Left) Sagittal T1 WI MR
shows isointense mass filling
thecal sac and obliterating
normal CSF signal. Large
flow voids present along
superior margin (arrow).
(Right) Sagittal T2WI MR
shows mass of increased T2
signal, filling the lumbar
thecal sac. Flow voids again
prominent along superior
margin (arrow).
(Left) Sagittal T1 C+ MR
shows enhancement of
intradural mass extending
throughout lumbar spine to
thoracic junction (arrows).
Multiple feeding vessel flow
voids present at superior
margin (open arrow). (Right)
Sagittal T1 C+ MR shows
extensive feeding vessels
with prominent flow voids
throughout the thoracic
spine (arrows). Superior
aspect of enhancing
intradural tumor also seen
(open arrow).
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MELANOCYTOMA
1
126
Sagittal TlWI MR shows intramedullary thoracic mass Sagittal T2WI MR shows heterogeneous solid and cystic
with heterogeneous Tl hyperintensity (arrows). mass intramedullary mass involving thoracic spine.
Multple foci of low signal present within lesion.
ITERMINOlOGY CT Findings
• NECT: Nonspecific intradural mass, poorly visualized
Abbreviations and Synonyms byCT
• Primary melanocytoma of cord, meningeal
melanocytoma MR Findings
• TIWI: Isointense to hyperintense intradural lesion
Definitions
• T2WI: Isointense to hypointense to normal cord
• Pigmented primary neoplasm of the CNS, involving • T2* GRE: May show blooming of low signal related to
cord or meninges melanin susceptibility effect
• Distinct from other melanotic lesions such as • TI C+: Heterogeneous enhancement
meningioma, schwannoma, melanoma
Imaging Recommendations
• Best imaging tool: MR C+ of entire spine, brain to
IIMAGING FINDINGS exclude additional lesions and possiblity of metastatic
melanoma
General Features • Protocol advice: TI, T2WI and post-contrast T1 WI
• Best diagnostic clue: Intradural enhancing mass
showing T1 hyperintensity, not fat suppressing
• Location: Intradural mass, either IDIFFERENTIAL DIAGNOSIS
intradural/extramedullary or rarely intramedullary
• Size: May extend over several vertebral body segments Hemorrhagic cord neoplasm
• Morphology: Heterogeneous signal mass lesion • Ependymoma may show hemorrhage, hemosiderin
• Astrocytoma (usually not hypervasculari peritumoral
Radiographic Findings edema common)
• Myelography: Nonspecific intradural mass, may show • Vascular metastasis such as renal cell carcinoma
block with larger lesion
Cavernous malformation
• Mottled or speckled pattern of prior hemorrhage,
hemosiderin rim
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MELANOCYTOMA
Key Facts
1
Terminology • Arteriovenous malformation 127
• Pigmented primary neoplasm of the CNS, involving • Pigmented intradural/extramedullary tumors
cord or meninges Pathology
• Distinct from other melanotic lesions such as • General path comments: Melanotic lesions probably
meningioma, schwannoma, melanoma constitute spectrum from well-differentiated
Top Differential Diagnoses melanocytoma => overtly malignant melanoma
• Hemorrhagic cord neoplasm • Immunoreactive for antimelanoma antibody HMB-45
• Cavernous malformation and S-100 protein, vimentin antibodies and negative
for epithelial membrane antigen
o' ....
~
,-IIitr,;'" .-
Presentation
• Most common Signs/symptoms
o Progressive back pain, extremety numbness,
weakness (Left) Sagittal Tl C+ MR shows heterogeneous enhancement of
o Other signs/symptoms intramedullary thoracic lesion. (Right) Axial TlWI MR shows slightly
hyperintense intramedullary mass within thoracic cord.
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Cysts
Arachnoid Cyst IV-2-2
Posterior Sacral Meningocele IV-2-6
Perineural Root Sleeve Cyst IV-2-10
Syringomyelia IV-2-14
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ARACHNOID CYST
2
2
Sagittal graphic demonstrates a type III intradural Axial bone CT following myelography shows dorsal
arachnoid cyst in mild dorsal thoracic canal, with epidural contrast containing cystic mass displacing
moderate mass effect on spinal cord. thecal sac, characteristic of an extradural AC. Spinal
canal is mildly expanded.
• Morphology
ITERMINOlOGY o Well-circumscribed
Abbreviations and Synonyms o Oval, elongated
• Arachnoid cyst (AC) o "Dumbbell" lesion: Extension through neural
• Spinal meningeal cyst (MC) foramen
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ARACHNOID CYST
Key Facts
Terminology • Typically CSF intensity
• Spinal meningeal cyst (Me) • Tl C+: No enhancement
• Intraspinal extramedullary loculated cerebral spinal • Variable mass effect on spinal cord
fluid (CSF) collection Top Differential Diagnoses
Imaging Findings • Idiopathic spinal cord herniation
• Facet synovial cyst
2
• Best diagnostic clue: Nonenhancing extramedullary
loculated CSF intensity collection displacing cord or • Dural ectasia 3
nerve roots Pathology
• Extradural or intradural extramedullary • Primary AC: Diverticulum of dura, arachnoid, or
• +/- Bony canal expansion nerve root sheath
• Axial post-myelography CT may delineate • Nabors classification of spinal MC
communication between extradural AC and
subarachnoid space Clinical Issues
• Contrast filled intradural AC difficult to visualize • Pain may worsen with Valsalva maneuver
• Suggested by mass effect on cord and nerve roots • Laminectomy with complete cyst wall resection
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ARACHNOID CYST
• Post-inflammatory or surgical adhesions
compartmentalize subarachnoid space Treatment
• Epidemiology: Primary extradural AC relatively rare • Laminectomy with complete cyst wall resection
• Associated abnormalities • If complete resection not possible
o Kyphoscoliosis o Wide marsupialization of cyst
o Syrinx o Shunting
o Spinal dysraphism
2 Gross Pathologic & Surgical Features IDIAGNOSTIC CHECKLIST
4 • Smooth-walled cystic mass
• Clear, colorless fluid Consider
• Thin-section axial post-myelography CT to define
Microscopic Features communication between extradural AC and
• Primary AC ± arachnoid lining inner cyst wall subarachnoid space
o Sometimes duplicated
o Variable connective tissue also present Image Interpretation Pearls
o Typically avascular • Nonenhancing thoracic extramedullary CSF intensity
o No significant inflammatory changes collection highly suggestive of spinal AC
• Secondary AC formed by fibrous connective tissue
Staging, Grading or Classification Criteria ISELECTED REFERENCES
• Nabors classification of spinal MC
1. Wang MY et al: Intradural spinal arachnoid cysts in adults.
o Type I: Extradural MC without spinal nerve root Surg Neurol. 60(1):49-55; discussion 55-6, 2003
fibers 2. Takeuchi A et al: Spinal arachnoid cysts associated with
• A: Extradural MC/AC syringomyelia: report of two cases and a review of the
• B: Sacral meningocele literature. J Spinal Disord Tech. 16(2):207-11,2003
o Type II: Extradural MC with spinal nerve root fibers 3. Abou-Fakhr FS et al: Thoracic spinal intradural arachnoid
• Tarlov perineural cyst cyst: report of two cases and review of literature. Eur
• Spinal nerve root diverticulum Radiol. 12(4):877-82,2002
4. Krings T et al: Diagnostic and therapeutic management of
o Type III: Intradural MC
spinal arachnoid cysts. Acta Neurochir (Wien).
• Intradural AC 143(3):227-34; discussion 234-5,2001
• Acquired intradural AC also known as 5. Lee HJ et al: Symptomatic spinal intradural arachnoid cysts
subarachnoid cyst in the pediatric age group: description of three new cases
and review of the literature. Pediatr Neurosurg.
35(4):181-7,2001
ICLINICAL ISSUES 6. Watters MR et al: Transdural spinal cord herniation:
imaging and clinical spectra. AJNR Am J Neuroradiol.
Presentation 19(7):1337-44, 1998
7. Silbergleit R et al: Imaging of spinal intradural arachnoid
• Most common signs/symptoms cysts: MRI, myelography and CT. Neuroradiology.
o Most patients asymptomatic 40(10):664-8, 1998
• Especially type II MC 8. Congia S et al: Myelographic and MRI appearances of a
o Other signs/symptoms thoracic spinal extradural arachnoid cyst of the spine with
• Pain extra- and intraspinal extension. Neuroradiology.
• Paraparesis 34(5):444-6, 1992
• Paresthesia 9. Rabb CH et al: Spinal arachnoid cysts in the pediatric age
• Hyperreflexia group: an association with neural tube defects. J Neurosurg.
77(3):369-72, 1992
• Bladder and bowel incontinence 10. Gindre-Barrucand T et al: Magnetic resonance imaging
• Clinical profile contribution to the diagnosis of spinal cord compression
o Pain may worsen with Valsalva maneuver by a subdural arachnoid cyst. Neuroradiology. 33(1):87-9,
• Increased intracystic pressure 1991
11. Dietemann JL et al: Thoracic intradural arachnoid cyst:
Demographics possible pitfalls with myelo-CT and MR. Neuroradiology.
• Age 33(1):90-1, 1991
o May present at any age 12. Boisserie-Lacroix M et al: The value of MRI in the study of
• Type I thoracic AC more common in adolescents spinal extradural arachnoid cysts. Comput Med Imaging
• Others more common in adults Graph. 14(3):221-3, 1990
• Gender: No gender predilection 13. Sklar E et al: Acquired spinal subarachnoid cysts:
Evaluation with MR, CT myelography, and intraoperative
• Ethnicity: No racial predilection sonography. AJNR. 10:1097-104, 1989
Natural History & Prognosis 14. Gray L et al: MR imaging of thoracic extradural arachnoid
cysts. JACT. 4:664-8, 1988
• Worsening neurologic deficits with enlarging cyst 15. Nabors MW et al: Updated assessment and current
• Excellent prognosis classification of spinal meningeal cysts. J Neurosurg.
o Complete relief of symptoms> 80% 68(3):366-77, 1988
• Degree of cord atrophy predictive of neurological
outcome
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ARACHNOID CYST
IIMAGE GALLERY
Typical
(Left) Sagittal
PO/Intermediate MR through
thoracic spine demonstrates
a dorsal epidural fluid
intensity mass with mild
mass effect. (Right) Axial
T2WI MR in same patient
shows expanded spinal
canal. Dorsal cystic mass
proved to be an extradural
AC at surgery.
(Left) Sagittal TI C+ MR
through thoracic spine shows
a nonenhancing dorsal
epidural fluid intensity mass
outlined by surrounding fat.
There is mass effect on spinal
cord (Courtesy M. Modic,
MOJ. (Right) Axial TlWI MR
in same patient shows dorsal
extradural AC enlarging and
extending through neural
foramina, and compressing
spinal cord (Courtesy M.
Modic, MOJ.
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POSTERIOR SACRAL MENINGOCELE
2
6
Sagittal T2WI MR demonstrates a meningocele at LS, Axial T2WI MR shows a midline defect in vertebral arch
extending into posterior subcutaneous tissue. Filum of L5. There is direct communication between thecal
terminale appears thickened (arrow), tethered at the sac, which contains sacral nerve roots, and the
neck of herniation sac. meningocele.
Definitions CT Findings
• PSM: Posterior herniation of dural sac through • NECT
vertebral arch defect in lumbosacral spine o Cerebral spinal fluid (CSF) density
• Spina bifida: Spinal dysraphism involving bony o Variable extension into posterior subcutaneous soft
elements of spine tissue
o Non-union of neural arch> unfused vertebral body • Covered by skin
• CECT: No enhancement
• Bone CT
I IMAGING FINDINGS o Deficient posterior elements in one or two spinal
segments
General Features MR Findings
• Best diagnostic clue: Focal outpouching of thecal sac
• TlWI: Isointense to CSF
through defect in posterior arch of a vertebra
• T2WI: Isointense to CSF
• Location: Lumbosacral> cervical or thoracic
• PD/Intermediate: Isointense to CSF
• Size: Variable
• STIR: CSF intensity
• Morphology • Tl C+: No enhancement
o Well-circumscribed
• Communicates with subarachnoid space
o Round or elongated
• Typically without neural elements
o Thin-walled
o Nerve root may enter and exit meningocele before
o Cystic mass
leaving neural foramen
Radiographic Findings o Filum terminale may extend into sac
• Radiography • Midline defect in vertebral posterior elements
o Spectrum of bony abnormalities
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POSTERIOR SACRAL MENINGOCELE
Key Facts
Terminology • Midline defect in vertebral posterior elements
• PSM: Posterior herniation of dural sac through Top Differential Diagnoses
vertebral arch defect in lumbosacral spine • Myelomeningocele
Imaging Findings • Lipomyelomeningocele
• Best diagnostic clue: Focal outpouching of thecal sac • Distal sacral or intrasacral meningocele 2
through defect in posterior arch of a vertebra Pathology
• Lumbosacral> cervical or thoracic 7
• PSM: Least common form of spina bifida
• Cystic mass
• Cerebral spinal fluid (CSF) density Clinical Issues
• Variable extension into posterior subcutaneous soft • Subcutaneous mass may be palpable
tissue • Usually asymptomatic
• T2WI: Isointense to CSF
• T1 C+: No enhancement Diagnostic Checklist
• Communicates with subarachnoid space • Careful evaluation of spinal cord to exclude
• Typically without neural elements associated anomalies
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POSTERIOR SACRAL MENINGOCELE
• Myelomeningocele
IDIAGNOSTIC CHECKLIST
• PSM
oSpina bifida occulta: 17% Consider
• Non-union of vertebral arch • Careful evaluation of spinal cord to exclude associated
• Asymptomatic anomalies
• Cutaneous findings overlying bony defect: Skin
dimpling, patch of hair Image Interpretation Pearls
2 • L5, SI, Cl most common • Skin covered posterior outpouching of thecal sac
• Spinal dysraphism: Open or closed through lumbosacral bony defect characteristic of PSM
8 o Open spinal dysraphism
• Myelomeningocele
• Myeloschisis ISELECTED REFERENCES
• Hemimyelo(meningo)cele 1. Barazi SA et al: High and low pressure states associated
o Closed spinal dysraphism: ± Back mass with posterior sacral meningocele. Br J Neurosurg.
• With mass: PSM, lipomyelo(meningo)cele 17(2):184-7,2003
• Without mass: Simple or complex 2. Khanna AJ et al: Magnetic resonance imaging of the
• Simple: Tight filum terminale, intradural lipoma, pediatric spine. J Am Acad Orthop Surg. 11(4):248-59,2003
etc. 3. Dick EA et al: Spinal ultrasound in infants. Br J Radiol.
• Complex: Split cord malformation, caudal 75(892):384-92, 2002
regression, neurenteric cyst, etc. 4. Evans A et al: Magnetic resonance imaging of intraspinal
cystic lesions: a pictorial review. Curr Probl Diagn Radiol.
31(3):79-94,2002
5. Diel J et al: The sacrum: pathologic spectrum,
ICLINICAL ISSUES multimodality imaging, and subspecialty approach.
Radiographies. 21(1):83-104, 2001
Presentation 6. Ersahin Y et al: Is meningocele really an isolated lesion?
• Most common signs/symptoms Childs Nerv Syst. 17:487-90, 2001
o If large, may see posterior lumbosacral contour bulge 7. Tortori-Donati P et al: Magnetic resonance imaging of
o Subcutaneous mass may be palpable spinal dysraphism. Top Magn Reson Imaging.
o Other signs/symptoms 12(6):375-409,2001
8. Tortori-Donati P et al: Spinal dysraphism: a review of
• Neurologic symptoms if cord tethered neuroradiological features with embryological correlations
• Back pain and proposal for a new classification. Neuroradiology.
• Scoliosis 42(7):471-91,2000
• Lower extremity weakness 9. Northrup H et al: Spina bifida and other neural tube
• Sensory loss defects. Curr Probl Pediatr. 30(10):313-32, 2000
• Gait disturbance 10. Egelhoff JC: MR imaging of congenital anomalies of the
• Bladder and bowel dysfunction pediatric spine. Magn Reson Imaging Clin N Am.
• Decreased deep tendons reflexes 7(3):459-79, 1999
11. Cornette L et al: Closed spinal dysraphism: a review on
• Clinical profile diagnosis and treatment in infancy. Eur J Paediatr Neurol.
o Usually asymptomatic 2(4):179-85,1998
o Symptoms, if present, develop after first few years of 12. Babcook CJ: Ultrasound evaluation of prenatal and
life neonatal spina bifida. Neurosurg Clin N Am. 6(2):203-18,
1995
Demographics 13. Barkovich AJ: Pediatric Neuroimaging. 2nd ed. 491-496,
• Age 1995
o Usually presents in first two decades of life 14. Byrd SE et al: Developmental disorders of the pediatric
o May present as late as fourth decade spine. Radiol Clin North Am. 29:711-52, 1991
15. Brunberg JA et al: Magnetic resonance imaging of spinal
• Gender: M = F
dysraphism. Radiol Clin North Am. 26(2):181-205, 1988
• Ethnicity: Spina bifida: Hispanics, Caucasians> Asians,
16. Fitz CR: Diagnostic imaging in children with spinal
African-Americans disorders. Pediatr Clin North Am. 32(6):1537-58, 1985
Natural History & Prognosis 17. Raghavendra BN et al: Sonography of the spine and spinal
cord. Radial Clin North Am. 23(1):91-105, 1985
• Good prognosis 18. Bale PM: Sacrococcygeal developmental abnormalities and
• Surgical complications tumors in children. Perspect Pediatr Pathol. 8(1):9-56, 1984
o Meningitis 19. Levine E et al: Computed tomography of sacral and
o Cord tethering perisacrallesions. Crit Rev Diagn Imaging. 21(4):307-74,
• May present later in life 1984
20. Campbell S et al: Ultrasound in the diagnosis of spina
Treatment bifida. Lancet. 1(7915):1065-8, 1975
• Surgical resection of PSM
• Closure of dura
• Myocutaneous flap
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POSTERIOR SACRAL MENINGOCELE
IIMAGE GAllERY
Typical
(Left) Sagittal Tl WI MR
shows a posterior
meningocele at L5. Focal fat
(arrow) is present in the
filum terminale, tethered at
2
the neck of herniation sac. 9
Distal spinal canal is
widened. (Right) Axial Tl WI
MR shows meningocele and
midline defect in neural arch
of L5.
(Left) Sagittal
PO/Intermediate MR
demonstrates simple
meningocele in lower lumbar
spine. It is
well-circumscribed and of
CSF intensity. (Right) Sagittal
TlWI MR shows
meningocele connected to
distal thecal sac through a
thin pedicle (arrow).
Typical
(Left) Transverse ultrasound
real time reveals parallel
sacral laminae with
associated midline osseous
defect. (Right) Axial T2WI
MR in another patient
demonstrates deficient
posterior elements in upper
sacrum, with meningocele
covered by skin and
subcutaneous tissue.
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PERINEURAL ROOT SLEEVE CYST
2
10
Axial NEeT post-myelography through L5 shows Sagittal T2WI MR through lumbar spine demonstrates a
contrast filling a left perineural cyst. well-circumscribed hyperintense Tarlov cyst remodeling
posterior aspect of 52. Intrinsic low intensity represents
CSFflow.
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PERINEURAL ROOT SLEEVE CYST
Key Facts .
Terminology • Flow sensitive sequence may demonstrate sIgnal loss
• Spinal nerve root diverticulum within cyst
• Type II extradural meningeal cyst (MC) Top Differential Diagnoses
• Dilatation of arachnoid and dura of spinal posterior • Meningocele
nerve root sheath, containing nerve fibers • Facet synovial cyst 2
Imaging Findings Pathology
• Best diagnostic clue: Cerebral spinal fluid (CSF) 11
• Common, incidental, and usually asymptomatic
density/intensity masses enlarging sacral neural • No reliable imaging method to differentiate
foramina symptomatic from asymptomatic lesions
• S2 and S3 nerve roots most commonly involved • Rupture may cause spontaneous intracranial
• Widened canal hypotension
• Enlarged foramen • Nabors classification of spinal MC
• Posterior vertebral scalloping
• Opacification of cyst by intrathecal contrast
• Tl C+: No enhancement
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PERINEURAL ROOT SLEEVE CYST
Gross Pathologic & Surgical Features Treatment
• Origin at junction of dorsal nerve root and its • Medical treatment
ganglion o Anti-inflammatory medication
• Spinal nerve root traverses through cyst or within cyst o Physical therapy
wall • Temporary relief with cyst aspiration
• ± Communication with subarachnoid space o Usually several months
2 Microscopic Features
• Sacral laminectomies with microsurgical cyst
fenestration and imbrication
12 • Outer wall with epineurium lined by arachnoid • Complete cyst/nerve root resection
• Inner wall lined with pia mater
• Fibrocollagenous tissue also present
• Old hemorrhage I DIAGNOSTIC CHECKLIST
• Nerve fibers (75%) or ganglionic cells (25%) within
cyst or cyst wall Consider
• Delayed CT (30-60 minutes) after intrathecal contrast
Staging, Grading or Classification Criteria injection to visualize filling of perineural cyst
• Nabors classification of spinal MC
• Type I: Extradural MC without spinal nerve root fibers Image Interpretation Pearls
o IA: Extradural MC • Sacral CSF intensity mass enlarging neural foramen
• Also known as extradural arachnoid cyst characteristic of perineural root sleeve cyst
• Relatively rare
• Dorsal or dorsal-lateral lower thoracic spine
• May extend into neural foramina
o IE: Occult sacral meningocele
ISELECTED REFERENCES
1. Kumar K et al: Symptomatic spinal arachnoid cysts: report
• Type II: Extradural MC with spinal nerve root fibers of two cases with review of the literature. Spine.
o Tarlov perineural cyst 28(2):E25-9,2003
o Spinal nerve root diverticulum 2. Caspar W et al: Microsurgical excision of symptomatic
• Type III: Intradural MC sacral perineurial cysts: a study of 15 cases. Surg Neurol.
o Also known as intradural arachnoid cyst 59(2):101-5; discussion 105-6, 2003
o Posterior aspect of midthoracic spine 3. Acosta FL et al: Diagnosis and management of sacral Tarlov
cysts. Case report and review of the literature. Neurosurg
Focus. 15 (2):Article 15, 2003
4. Voyadzis JM et al: Tarlov cysts: a study of 10 cases with
ICLINICAL ISSUES review of the literature. J Neurosurg. 95(1 Suppl):25-32,
2001
Presentation 5. Mummaneni PV et al: Microsurgical treatment of
• Most common signs/symptoms symptomatic sacral Tarlov cysts. Neurosurgery. 47(1):74-8;
o Majority asymptomatic: > 80% discussion 78-9, 2000
o Other signs/symptoms 6. ArunKumar MJ et al: Sacral nerve root cysts: A review on
• Low back or sacral pain pathophysiology. Neurol India. 47(1):61-4, 1999
• Radicular or perineal pain 7. Paulsen RD et al: Prevalence and percutaneous drainage of
cysts of the sacral nerve root sheath (Tarlov cysts). AJNR.
• Paresthesia 15:293-7, 1994
• Weakness 8. Davis SW et al: Sacral meningeal cysts: Evaluation with MR
• Bladder or bowel dysfunction imaging. Radiology. 187:445-8, 1993
• Clinical profile 9. Araki Y et al: MRI of symptomatic sacral perineural cyst.
o Symptoms simulate disc herniation and spinal Radiat Med. 10(6):250-2, 1992
stenosis 10. Nabors MW et al: Updated assessment and current
o Symptoms may worsen with postural changes and classification of spinal meningeal cysts. J Neurosurg.
Valsalva maneuvers 68:366-77, 1988
11. Goyal RN et al: Intraspinal cysts: a classification and
Demographics literature review. Spine. 12(3):209-13, 1987
12. Willinsky RA et al: Computed tomography of a sacral
• Age: 30-40 yo perineural cyst. J Comput Assist Tomogr. 9(3):599-601,
• Gender: M = F 1985
• Ethnicity: No racial predilection 13. Dastur HM: The radiological appearances of spinal
extradural arachnoid cysts. J Neurol Neurosurg Psychiatry.
Natural History & Prognosis 26:231-235, 1963
• Progressive cyst enlargement 14. Abbott KH et al: The role of perineurial sacral cysts in the
• Cyst recurs following aspiration or partial resection sciatic and sacrococcygeal syndromes. A review of the
• Symptomatic relief after complete resection: > 70% literature and report of 9 cases. J Neurosurg. 14:5-21, 1957
o Better outcome associated with 15. Tarlov 1M: Perineural cysts of the spinal nerve roots. Arch
• Cysts < 1.5 cm Neurol Psychiatry. 40:1067-1074, 1938
• Radicular pain
• Bladder/bowel dysfunction
• Worsening symptoms with Valsalva maneuver
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PERINEURAL ROOT SLEEVE CYST
IIMAGE GALLERY
Typical
(Left) Axial T2WI MR
through L5 shows a CSF
intensity mass in left lateral
recess. (Right) Axial Tl C+
MR demonstrates left lateral
2
recess lesion without 13
enhancem,ent, compatible
with a Tarlov perineural cyst.
Typical
(Left) Axial T2WI MR with fat
suppression shows bilateral
thoracic paraspinal
hyperintense masses.
Left-sided mass
communicates with and is of
same intensity as CSF.
Right-sided mass is more
hyperintense. (Right) Axial
Tl C+ MR with fat
suppression demonstrates
nonenhancing nature of
cysts, consistent with
bilateral nerve sheath
diverticula.
Typical
(Left) Axial TlWI MR
through thoracic spine shows
a left foraminal CSF intensity
perineural cyst. (Right) Axial
T2WI MR with fat
suppression shows bilateral
sacral Tarlov perineural
cysts, remodeling neural
foramina.
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SYRINGOMYELIA
2
14
Sagittal graphic demonstrates a large, sacculated, Sagittal T1Wf MR depicts a large longstanding
"beaded" syrinx extending to the conus. sacculated syrinx associated with severe tonsillar ectopia
(Chiari I, arrow) and mild posterior cervical vertebral
scalloping.
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. I
.
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Ventriculus Terminalis Hemangioblastoma Astrocytoma Myelomalacia
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SYRINGOMYELIA
Key Facts
Terminology Pathology
• Synonyms: Hydromyelia, syringohydromyelia, syrinx • Longitudinally oriented CSF-filled cavity +/-
• Hydromyelia = cystic central canal dilatation surrounding gliosis, myelomalacia
• Syringomyelia = cystic spinal cord cavity not
contiguous with central cord canal Clinical Issues
• "Cloak-like" pain and temperature sensory loss with 2
Imaging Findings preservation of position sense, proprioception, light
• Best diagnostic clue: Expanded spinal cord with touch 15
dilated, beaded, or sacculated cystic cavity • Distal upper extremity weakness, gait instability
• Sagittal images demonstrate longitudinal extent • Mechanical spinal pain, radicular pain, spastic
• Axial images confirm syrinx topology, clarify paraparesis, scoliosis
relationship to adjacent structures
Diagnostic Checklist
Top Differential Diagnoses • Despite septated appearance, large syrinx cavities
• Ventriculus terminalis usually contiguous
• Cystic spinal cord tumor • Contrast administration essential to exclude tumor in
• Myelomalacia complicated cavitary lesions
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SYRINGOMYELIA
o Cavity lined by ependyma; length influenced by
age-related central canal stenosis IDIAGNOSTIC CHECKLIST
• Noncommunicating (isolated) central canal dilation Consider
below syrinx-free cord segment • Syrinx etiology influences treatment approach
o Variable distance below IVth ventricle; cavity • Despite septated appearance, large syrinx cavities
margins defined by adjacent central canal stenosis usually contiguous
o +/- Paracentral dissection into parenchyma, gliosis
2 • Extracanalicular (parenchymal) syrinx non-contiguous Image Interpretation Pearls
with central canal • Simple syringomyelia rarely enhances or produces
16 o Watershed spinal cord area +/- myelomalacia, diagnostic dilemma
paracentral dissection lined by glial or fibroglial • Contrast administration essential to exclude tumor in
tissue, rupture into spinal subarachnoid space, complicated cavitary lesions
central chromatolysis, neuronophagia, Wallerian
degeneration
ISELECTED REFERENCES
1. Aryan HE et al: Syringocephaly. J Clin Neurosci.
ICLINICAL ISSUES 11(4):421-3,2004
2. Piatt JH Jr: Syringomyelia complicating myelomeningocele:
Presentation review of the evidence. J Neurosurg. 100(2 Suppl):101-9,
• Most common signs/symptoms 2004
o "Cloak-like" pain and temperature sensory loss with 3. Kyoshima K et al: Spontaneous resolution of
preservation of position sense, proprioception, light syringomyelia: report of two cases and review of the
touch literature. Neurosurgery. 53(3):762-8; discussion 768-9,
o Distal upper extremity weakness, gait instability 2003
4. Brodbelt AR et al: Post-traumatic syringomyelia: a review. J
o Other signs/symptoms
Clin Neurosci. 10(4):401-8,2003
• Mechanical spinal pain, radicular pain, spastic 5. Ozerdemoglu RA et al: Scoliosis associated with
paraparesis, scoliosis syringomyelia: clinical and radiologic correlation. Spine.
• Cranial neuropathy (2° to syringobulbia) 28(13):1410-7,2003
• Clinical profile 6. Klekamp J et al: Syringomyelia associated with foramen
o Symmetrically enlarged central cavity asymptomatic magnum arachnoiditis. J Neurosurg. 97(3 Suppl):317-22,
or presents with nonspecific neurological signs 2002
(spasticity, weakness, segmental pain) 7. Holly LT et al: Slitlike syrinx cavities: a persistent central
canal. J Neurosurg. 97(2 Suppl):161-5, 2002
o Paracentral cavitation associated with combination
8. Klekamp J: The pathophysiology of syringomyelia-
of long tract and segmental signs referable to level, historical overview and current concept. Acta Neurochir
side, and quadrant of spinal cord cavitation (Wien). 144(7):649-64,2002
9. Vinas FC et al: Spontaneous resolution of a syrinx. J Clin
Demographics Neurosci. 8(2):170-2, 2001
• Age: Usual presentation in late adolescence or early 10. Klekamp J et al: Spontaneous resolution of Chiari I
adulthood; uncommon in childhood malformation and syringomyelia: case report and review of
• Gender: M = F the literature. Neurosurgery. 48(3):664-7, 2001
11. Unsinn KM et al: US of the spinal cord in newborns:
Natural History & Prognosis spectrum of normal findings, variants, congenital
• Variable; dependent on underlying etiology anomalies, and acquired diseases. Radiographics.
o Chronic, slow progression usual; occasional acute 20(4):923-38, 2000
12. Brugieres P et al: CSF flow measurement in syringomyelia.
course
AJNR. 21(10): 1785-92, 2000
o Spontaneous resolution rare 13. Levy LM: MR imaging of cerebrospinal fluid flow and
• +/- Reversibility after Chiari decompression, mass spinal cord motion in neurologic disorders of the spine.
removal, adhesion lysis, or cord untethering Magn Reson Imaging Clin NAm. 7(3):573-87, 1999
o Long term improvement < 50% 14. Fischbein NJ et al: The "presyrinx" state: a reversible
o Sensory disturbances, dysesthesias, pain more likely myelopathic condition that may precede syringomyelia.
to improve than motor weakness or gait disturbance AJNR AmJ Neuroradiol. 20(1):7-20,1999
• CSF diastolic flow determines cyst size, biological 15. Milhorat TH et al: Intramedullary pressure in
behavior syringomyelia: clinical and pathophysiological correlates of
syrinx distension. Neurosurgery. 41(5):1102-10, 1997
o Diastolic flow velocity! after successful surgery; 16. Tanghe HL: Magnetic resonance imaging (MRI) in
persistent 1 implies poor outcome syringomyelia. Acta Neurochir (Wien). 134(1-2):93-9, 1995
17. Milhorat TH et al: Pathological basis of spinal cord
Treatment cavitation in syringomyelia: analysis of 105 autopsy cases. J
• Primarily address underlying causative etiology Neurosurg. 82(5):802-12, 1995
o Correct osseous deformities, decompress/untether
spinal cord, lyse adhesions
o Consider syrinx drainage with indwelling catheter
only if not possible to restore normal cord CSF
dynamics
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SYRINGOMYELIA
I IMAGE GALLERY
Typical
(Left) Axial T2WI MR
demonstrates mild central
canal dilatation
(hydromyelia) in conjunction 2
with Chiari 7 malformation
(not shown). (Right) Sagittal 17
T2WI MR shows thoracic
cord syringohydromyelia
extending into conus
medullaris. Extensive cord
involvement distinguishes
from ventriculus terminalis.
Variant
(Left) Axial T2WI MR
demonstrates asymmetric
syrinx extension into right
medulla (syringobulbia) with
surrounding gliosis (arrow).
(Right) Sagittal T7WI MR
depicts a large sacculated
syrinx extending entire length
of cord into conus. The
syrinx cavity is contiguous
despite septated appearance
(Courtesy R. Boyer, MO).
Variant
(Left) Sagittal T2WI MR
shows an extensive
post-traumatic syrinx in
conjunction with healed
thoracolumbar burst
fracture. (Right) Axial T2WI
MR of post-traumatic syrinx
shows extensive irregular
cord T2 hyperintensity with
asymmetric extension into
dorsal cord (arrow).
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EPIDURAL LIPOMATOSIS
2
18
Sagittal graphic illustrates confluent abundant epidural Sagittal TlWI MR shows prominent dorsal epidural fat
fat in dorsal thoracic canal, with mild mass effect on from TI to T9, narrowing central canal. Compression
spinal cord. fractures involve T4 through T8 vertebrae. Patient has
Cushing disease.
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EPIDURAL LIPOMATOSIS
Key Facts
Terminology Top Differential Diagnoses
• Excessive accumulation of intraspinal fat causing • Subacute epidural hematoma
cord compression and neurologic deficits • Spinal angiolipoma
• Epidural metastasis, lymphoma
Imaging Findings
• Best diagnostic clue: Abundant epidural fat in Pathology 2
mid-thoracic and distal lumbar spinal canal • Long term exogenous steroid administration
compressing thecal sac • Excessive endogenous steroid production 19
• Thoracic spine: 58-61%
• Lumbar spine: 39-42% Clinical Issues
• Epidural fat ~ 7 mm thick in thoracic spine • Weakness: > 85%
• "Y" shaped configuration to lumbar thecal sac on • Back pain: 64%
axial imaging • > 80% with post-surgical symptomatic relief
• Increased fat density in spinal canal Diagnostic Checklist
• Homogeneous
• Excessive extradural fat in dorsal midthoracic spine
• Mass effect on thecal sac and nerve roots and caudal lumbar spine diagnostic of SEL
Imaging Recommendations
IPATHOLOGY
• Best imaging tool: Sagittal and axial T1WI and T2WI General Features
MRI • General path comments
• Protocol advice: Fat suppression to confirm adipose o Increased fat tissue in spinal canal
tissue o Typically in thoracic and lumbar regions
• Genetics: No genetic predisposition
• Etiology
I DIFFERENTIAL DIAGNOSIS o Long term exogenous steroid administration
• 75% of reported cases
Subacute epidural hematoma • Treatment of obstructive airways disease, systemic
• Hyperintense on T1WI lUpus erythematosus, etc.
o Hyperintense even with fat suppression • No definite correlation between dosage and
• Heterogeneous with hypointense foci on T2WI duration of steroid use and development of SEL
• Acute onset of symptoms • Mean dosage between 30 mg/d and 100 mg/d for
Spinal angiolipoma 5-11 years in reported cases
o Association with epidural steroid injection also
• Benign neoplasm with varying proportion of adipose
and vascular elements reported
o Excessive endogenous steroid production
• Mid-thoracic region most common
• Infiltrative type invades surrounding bone • Cushing disease
• Hyperintense on T1 WI • Hypothyroidism
o Slightly or moderately hypointense to epidural fat • Other endocrinopathies
o Heterogeneous due to vascular elements o General obesity
• Iso- to slightly hypointense to epidural fat on T2WI o Idiopathic
• Diffuse post-contrast-enhancement • Epidemiology: Uncommon entity
o Better visualized with fat suppression • Associated abnormalities
o Vertebral compression fractures
Epidural metastasis, lymphoma • Steroid related osteoporosis
• Hypointense to epidural fat on T1WI and T2WI o Syrinx
o Soft tissue signal intensity • Anatomy
• Vertebral and paraspinal involvement o Meningovertebralligaments in lumbar spine
o Not limited to spinal canal • Median, paramedian, lateral aspects of ventral and
• Avid post-gadolinium enhancement dorsal epidural space
• Anchor dura to spinal canal
Epidural abscess • May form septum, partitioning epidural space
• Most common in ventral epidural space • May explain triangular or "Y" shaped
o Associated with discitis/osteomyelitis configuration of lumbar thecal sac on axial
• Posterolateral spinal canal from septic facet joint imaging
• Hypointense on T1WI
• Hyperintense on T2WI
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EPIDURAL LIPOMATOSIS
Gross Pathologic & Surgical Features ISELECTED REFERENCES
• Abundant adipose tissue external to thecal sac 1. Fassett DR et al: Spinal epidural lipomatosis: a review of its
• No capsule causes and recommendations for treatment. Neurosurg
Focus. 16(4):Article 11, 2004
Microscopic Features 2. Payer M et al: Idiopathic symptomatic epidural lipomatosis
• Hypertrophied fat cells of the lumbar spine. Acta Neurochir (Wien). 145(4):315-20;
2 3.
discussion 321, 2003
Geers C et al: Polygonal deformation of the dural sac in
lumbar epidural lipomatosis: anatomic explanation by the
20 ICLINICAL ISSUES presence of meningovertebralligaments. AJNR Am J
Presentation Neuroradiol. 24(7):1276-82, 2003
4. Dumont-Fischer 0 et al: Spinal epidural lipomatosis
• Most common signs/symptoms revealing endogenous Cushing's syndrome. Joint Bone
o Weakness: > 85% Spine. 69(2):222-5, 2002
o Other signs/symptoms 5. Lisai P et al: Cauda Equina Syndrome Secondary to
• Back pain: 64% Idiopathic Spinal Epidural Lipomatosis. Spine. Vol
• Sensory loss 26:307-9,2001
• Polyradiculopathy 6. Citow JS et al: Thoracic epidural lipomatosis with
associated syrinx: case report. Surg Neurol. 53(6):589-91,
• Altered reflexes
2000
• Incontinence 7. McCullen GM et al: Epidural lipomatosis complicating
• Ataxia lumbar steroid injections. J Spinal Disord. 12(6):526-9,
• Clinical profile: Gradual progression of symptoms 1999
8. Robertson SC et al: Idiopathic spinal epidural lipomatosis.
Demographics Neurosurgery. 41(1):68-74; discussion 74-5, 1997
• Age 9. Fiirgaard B et al: Spinal epidural lipomatosis. Case report
o Mean age at presentation: 43 yo and review of the literature. Scand J Med Sci Sports.
• Slightly younger for thoracic SEL 7(6):354-7, 1997
• Older (50s) for lumbar SEL 10. Benamou PH et al: Epidural lipomatosis not induced by
• Gender: M > F corticosteroid therapy. Three cases including one in a
patient with primary Cushing's disease (review of the
• Ethnicity: No racial predilection
literature). Rev Rhum Engl Ed. 63(3):207-12, 1996
Natural History & Prognosis 11. Hierholzer J et al: Epidural lipomatosis: case report and
literature review. Neuroradiology. 38(4):343-8, 1996
• Excellent 12. Kumar K et al: Symptomatic Epidural Iipomatosis
o > 80% with post-surgical symptomatic relief Secondary to Obesity. J Neurosurg. Vol 85: 348-50, 1996
o Pre-surgical low steroid dose and idiopathic SEL 13. Provenzale JM et al: Spinal angiolipomas: MR features.
have better prognosis AJNR AmJ Neuroradiol. 17(4):713-9, 1996
• Post-surgical mortality: 22% 14. Zentner J et al: Spinal epidural lipomatosis as a
o Immunocompromised state from chronic steroid use complication of prolonged corticosteroid therapy. J
Neurosurg Sci. 39(1):81-5, 1995
Treatment 15. Beges C et al: Epidural lipomatosis. Interest of magnetic
• Correction of underlying endocrinopathies resonance imaging in a weight-reduction treated case.
• Discontinuing exogenous steroids Spine. 19(2):251-4, 1994
16. Kuhn MJ et al: Lumbar epidural lipomatosis: the "Y" sign of
• Weight reduction in case of general obesity
thecal sac compression. Comput Med Imaging Graph.
• Surgical intervention 18(5):367-72, 1994
o Indicated when cord compression and radiculopathy 17. Preul MC et al: Spinal angiolipomas. Report of three cases. J
present Neurosurg. 78(2):280-6, 1993
o Multilevel laminectomy 18. Roy-Camille R et al: Symptomatic spinal epidural
o Fat debulking lipomatosis induced by a long-term steroid treatment.
o Posterolateral fusion to maintain stability Review of the literature and report of two additional cases.
Spine. 16(12):1365-71, 1991
19. Healy ME et al: Demonstration by magnetic resonance of
IDIAGNOSTIC CHECKLIST symptomatic spinal epidural lipomatosis. Neurosurgery.
21(3):414-5, 1987
20. Randall BC et al: Epidural lipomatosis with lumbar
Consider radiculopathy: CT appearance. J Comput Assist Tomogr.
• Fat suppression to distinguish SEL from epidural 10(6):1039-41, 1986
hematoma
Image Interpretation Pearls
• Excessive extradural fat in dorsal midthoracic spine
and caudal lumbar spine diagnostic of SEL
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EPIDURAL LIPOMATOSIS
[IMAGE GALLERY
(Left) Sagittal TI WI M R
shows increased extradural
fat in lumbar canal,
narrowing thecal sac. (Right)
Axial TlWI MR shows
excessive epidural fat
surrounding thecal sac, with
a triangular configuration.
(Left) Sagittal TI WI M R of
thoracic spine demonstrates
abundant dorsal extradural
fat, narrowing central canal.
(Right) Axial TlWI MR
shows increased epidural fat
compressing thecal sac and
flattening thoracic cord
(Courtesy M. Modic, MO).
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HETEROGENEOUS FATTY MARROW
2
22
Sagittal T1WI MR shows stippled appearance of normal Sagittal T1WI MR shows marbled appearance of fatty
hematopoietic and fatty marrow in 70 year old child. and erythropoietic marrow in middle-aged adult
ITERMINOlOGY CT Findings
Abbreviations and Synonyms • Bone CT
o Normal trabecular pattern preserved
• Red marrow reconversion
MR Findings
Definitions
• TlWI
• Irregularly distributed fatty, hematopoietic and fibrous o High signal intensity fat intermingled with low to
elements in bone marrow. intermediate signal intensity red marrow
• Red marrow: Hematopoietic marrow o Red marrow equal or higher signal than disc
• Yellow marrow: Fatty marrow • T2WI: Heterogeneity less apparent than on TlWI, STIR
• STIR: Low signal intensity fat intermingled with
intermediate signal intensity marrow
IIMAGING FINDINGS • DWI: Not shown to be of use in distingUishing red
General Features marrow from tumor
• Tl C+: Erythropoietic marrow enhances slightly
• Best diagnostic clue: Red marrow signal intensity equal
• In phase and out of phase gradient echo imaging
or higher than intervertebral disc on TlWI
o Contribution of fat and water to signal cycle in and
• Location out of phase with increasing TE
o Vertebral bodies
o Erythropoietic marrow contains fat and water: Low
o Yellow marrow often distributed around central
signal on out of phase images
perforating vein
o Neoplasm usually doesn't contain fat: High signal
o Yellow marrow adjacent to endplates in
on out of phase images
degenerative disc disease o No large series to validate this concept
o After bone marrow transplantation: Fat centrally in
• Early myeloma has same appearance as red
vertebral body, red marrow at periphery
marrow
o Loss of red marrow in regions of radiation therapy
• Morphology: Stippled or marbled appearance of Nuclear Medicine Findings
vertebral body • Bone Scan: Normal
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HETEROGENEOUS FATTY MARROW
Key Facts
Terminology • Yellow marrow adjacent to endplates in degenerative
• Irregularly distributed fatty, hematopoietic and disc disease
fibrous elements in bone marrow • After bone marrow transplantation: Fat centrally in
vertebral body, red marrow at periphery
Imaging Findings • Loss of red marrow in regions of radiation therapy
• Best diagnostic clue: Red marrow signal intensity
Top Differential Diagnoses
2
equal or higher than intervertebral disc on T1 WI
• Yellow marrow often distributed around central • Multiple myeloma, leukemia 23
perforating vein • Metastasis
• Myelofibrosis
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LANGERHANS CELL HISTIOCYTOSIS
2
24
Sagittal graphic shows diffuse vertebral marrow Sagittal T7 C+ MR in a child shows an avidly enhancing
infiltration with pathologic fracture producing vertebra destructive C2 mass with posterior epidural spread. The
plana sparing the disc spaces. Epidural extension adjacent disc space is spared, typical of LCH (Courtesy
produces ventral cord compression. C. Hedlund, DO).
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LANGERHANS CELL HISTIOCYTOSIS
• Skin lesions without other site of involvement • Vertebral height restoration varies 18.2% ~ 63.8%
• Monostotic lesion +/- diabetes insipidus (Dr), before, 72.2% ~ 97% after skeletal maturity
adjacent lymph node involvement, or rash
• Polyostotic lesions involving several bones or :0: 2 Treatment
lesions in one bone +/- Dr, adjacent lymph node • Conservative management initially with observation
involvement, or rash +/- bracing
o Extensive LCH • +/- Surgical intervention (partial intralesional
2 • Visceral organ involvement +/- bone lesions, DI, curettage, fusion), external beam radiotherapy,
chemotherapy, steroids in patients with neurological
adjacent lymph node involvement, and/or rash
26 without lung, liver, or hemopoietic organ system deficits, conservative treatment failure
dysfunction
• Visceral organ involvement +/- bone lesions, DI,
adjacent lymph node involvement, and/or rash IDIAGNOSTIC CHECKLIST I.
with lung, liver, or hemopoietic organ system
dysfunction .
Consider
• Vertebra plana much more common in children than
adults
• Unexplained vertebral compression fractures in
I CLINICAL ISSUES children or adolescents merit evaluation to exclude
Presentation LCH or leukemia
• Most common signs/symptoms Image Interpretation Pearls
o Asymptomatic
• Vertebra plana with relative absence of epidural mass,
o Localized pain 2° medullary expansion or pathologic
disc abnormalities classic for LCH
fracture, reduced mobility, swelling, fever,
leukocytosis
o Other signs/symptoms
• Myelopathy or radiculopathy
ISELECTED REFERENCES
• Clinical profile 1. Kuhn J et al: Caffey's Pediatric Diagnostic Imaging. 10th
o EG (70%) ed. Philadelphia, Mosby. Page 704, 2004
2. Simanski C et al: The Langerhans' cell histiocytosis
• Localized form (bone lesions only), older children (eosinophilic granuloma) of the cervical spine: a rare
(5-15 y), good prognosis diagnosis of cervical pain. Magn Reson Imaging.
• Back pain, stiffness, scoliosis, neurologic 22(4):589-94, 2004
complications, fever, leukocytosis 3. Bavbek M et al: Spontaneous resolution of lumbar vertebral
o Hand-Schiiller-Christian disease (20%) eosinophilic granuloma. Acta Neurochir (Wien).
• Chronic disseminated osseous and visceral lesions, 146(2):165-7, 2004
younger children (1-5 y), intermediate prognosis 4. Puertas EB et al: Surgical treatment of eosinophilic
(fatal 10-30%) granuloma in the thoracic spine in patients with
neurological lesions. J Pediatr Orthop B. 12(5):303-6, 2003
• Triad of diabetes insipidus, exophthalmos, bone 5. Fernando Ugarriza L et al: Solitary eosinophilic granuloma
destruction of the cervicothoracic junction causing neurological
o Letterer-Siwe disease (10%) deficit. Br J Neurosurg. 17(2):178-81, 2003
• Acute form, rapid visceral organ dissemination, 6. Graham D et al: Greenfield's Neuropathology. 7th ed.
infants « 3 y), poor prognosis London, Arnold. 1017-1018,2002
• Fever, cachexia, anemia, hepatosplenomegaly, 7. Bertram C et al: Eosinophilic granuloma of the cervical
lymphadenopathy, rash, gum hyperplasia spine. Spine. 27(13):1408-13, 2002
8. Plasschaert F et al: Eosinophilic granuloma. A different
• Most patients die :s 1-2 years behaviour in children than in adults. J Bone Joint Surg Br.
Demographics 84(6):870-2,2002
9. Kamimura M et "al: Eosinophilic granuloma of the spine:
• Age early spontaneous disappearance of tumor detected on
o Predominantly affects children, adolescents, or magnetic resonance imaging. Case report. J Neurosurg.
young adults, but may occur at any age 93(2 Suppl):312-6, 2000
• Most severe manifestations peak at younger ages 10. Reddy PK et al: Eosinophilic granuloma of spine in adults:
• Gender: M:F = 2: 1 a case report and review of literature. Spinal Cord.
38(12):766-8, 2000
Natural History & Prognosis 11. Yeom JS et al: Langerhans' cell histiocytosis of the spine.
• Variable depending on presentation age, extent of Analysis of twenty-three cases. Spine. 24(16):1740-9, 1999
systemic disease 12. Kandoi M et al: Rapidly progressive polyostotic
o Spontaneous remission of lesions common, but eosinophilic granuloma involving spine: a case report.
relapse or reactivation possible Indian J Med Sci. 52(1):22-4, 1998
13. Raab P et al: Vertebral remodeling in eosinophilic
o Lesions usually begin to regress after"" 3 months, granuloma of the spine. A long-term follow-up. Spine.
may take up to 2 years to resolve 23(12):1351-4, 1998
o Vertebra plana prognosis favorable for symptomatic
improvement, vertebral height restoration with
conservative orthopedic treatment
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LANGERHANS CELL HISTIOCYTOSIS
IIMAGE GALLERY
Typical
(Left) Sagittal T7 C+ MR
shows pathological vertebral
collapse with enhancing
epidural mass. Vertebral
2
endplates and adjacent discs
are intact (Courtesy 5. Blaser 27
MO, and A. /liner, MO).
(Right) Lateral radiography
demonstrates marked C7
vertebral compression
(" vertebra plana" J without
endplate destruction or
kyphosis.
Typical
(Left) Anteroposterior
radiography shows left lateral
collapse of the T7 2 vertebra
producing acute angle
scoliosis. Vertebral endplates
and pedicles are preserved
(Courtesy R. Boyer, MO).
(Right) Coronal T7 C+ MR
depicts lateral T7 2 vertebral
collapse with focal scoliosis
and small paraspinal
enhancing mass. Vertebral
endplates and adjacent disc
spaces are preserved
(Courtesy R. Boyer, MOJ.
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ACQUIRED EPIDERMOID TUMOR
2
28
Sagittal graphic depicts a small well-circumscribed Sagittal T2WI MR through lumbar spine shows a
pearly white tumor within cauda equina, compatible well-circumscribed lesion, hyperintense to CSF,
with an epidermoid. adherent to cauda equina.
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ACQUIRED EPIDERMOID TUMOR
Key Facts
Terminology • II WI: Isointense to CSF or cord
• Acquired intraspinal mass arising from iatrogenically • T2WI: Iso- to hyperintense to CSF
implanted epithelial elements • Tl C+: No or faint peripheral enhancement
!ClINICALISSUES
Presentation
• Most common signs/symptoms
o Low back pain
o Other signs/symptoms
• Radicular pain
• Paraparesis (Left) Sagittal T7 WI M R through lumbar spine demonstrates a subtle
• Paresthesia intradural lesion (arrow) distal to conus medullaris, slightly
hyperintense to CSF. (Right) Sagittal T7 C+ MR shows the same lesion
• Sphincter dysfunction without enhancement (arrow). An epidermoid was found at surgery.
Demographics
• Age: All ages reported
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FIBROUS DYSPLASIA
2
30
Anteroposterior and posteroanterior bone scans show Anteroposterior radiography shows polyostouc FD.
polyostotic fibrous dysplasia. Note scoliosis and Arrow shows femoral lesion. Right L5 pedicle is involved
multifocal involvement of spine (arrows). (open arrow), but this might be missed without posiUve
BS in this region.
~.
"
..
~.
.
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FIBROUS DYSPLASIA
Key Facts
Terminology • Best imaging tool: CT scan, 1-3 mm sections
• McCune Albright Syndrome: Polyostotic FD, Top Differential Diagnoses
precocious puberty, cafe-au-lait skin lesions • Aneurysmal bone cyst (ABC)
Imaging Findings • Osteoid osteoma
• Best diagnostic clue: Mildly expansile lesion with • Osteoblastoma 2
"ground glass" bone matrix Pathology
• Spine involvement almost always with polyostotic 31
• Endocrine abnormalities
disease
Neurofibromatosis
• Scoliosis, cafe-au-Iait spots IIMAGE GALLERY
• Peripheral nerve sheath tumors
·1 PATHOLOGY
General Features
• Genetics: Sporadic mutation in GNAS1 gene
• Epidemiology
o Solitary lesions common
o Polyostotic form less common
• Associated abnormalities
o Endocrine abnormalities
• Hyperparathyroidism
• Acromegaly (Left) Lateral radiography shows lytic lesions at C3 and C4 due to FO
• Premature puberty and associated ABC. Balloon-like expansion of C4 reflects ABC
• Phosphaturia component (arrow) (Courtesy L. Lomasney, MO). (Right) Sagittal
T7WI MR shows FO (arrows) and associated ABC (open arrow)
Gross Pathologic & Surgical Features involving C3 and C4. Blood products in ABC component result in
• White or tan lesion with gritty consistency high signal intensity on T7 WI.
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KUMMELL DISEASE
2
32
Anteroposterior radiography shows loss of height of L1 Coronal bone CT shows gas within collapsed, sclerotic
vertebral body and sclerosis beneath endplate. A thin vertebral body. Cas is also present in adjacent disc
band ofgas is visible (arrows). spaces.
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KUMMELL DISEASE
Key Facts
Terminology Top Differential Diagnoses
• Delayed, post-traumatic collapse of vertebral body • Infection
• Nontraumatic bone infarction
Imaging Findings • Gas within degenerated discs
• Location: Thoracic or lumbar vertebral body
• Loss of height, sclerosis of vertebral body
• Gas in metastasis
2
• Narrow, horizontally oriented band of gas in vertebral
body 33
• Best imaging tool: CT scan
IIMAGE GALLERY
ICLINICAL ISSUES
Presentation
• Most common signs/symptoms: Pain, kyphosis
Demographics
• Age: Usually elderly, osteoporotic patients
• Gender: More common in females
Natural History & Prognosis
• ProgreSSive vertebral body collapse if untreated
Treatment
• Options, risks, complications: VeJ:tebroplasty or
kyphoplasty relieves pain (Left) Sagittal T2WI MR shows band-like gas in vertebral body
(arrow), and gas in intervertebral discs (open arrows). Findings less
conspicuous and less specific than on CT. (Right) Sagittal T1 WI M R
shows fluid filled vertebral cleft 5 months following fracture,
precursor to gas-filled cleft of KOmmell disease.
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SECTION 1
Vascular Lesions
Introduction and Overview
Vascular Anatomy V-I-2
Vascular lesions
Type I DAVF V-I-6
Type IIAVM V-I-lO
Type III AVM V-I-14
Type IV AVF V-I-18
Cavernous Malformation, Spinal Cord V-I-22
Spinal Cord Infarction V-I-26
Subarachnoid Hemorrhage V-I-30
Spontaneous Epidural Hematoma V-I-34
Subdural Hematoma V-I-38
Superficial Siderosis V-I-42
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VASCULAR ANATOMY
1
2
Coronal oblique graphic shows source of· thoracic Anteroposterior angi~graphy of left T9 intercostal artery
vascular supply from aorta (arrow) derived from shows classic hairpin turn of anterior spinal artery
segmental feeders (open arrow): ASA (curved black (Adamkiewicz) (arrow).
arrow), PSA shown (curved white arrow).
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VASCULAR ANATOMY
1
DIFFERENTIAL DIAGNOSIS 3
Anterior spinal artery Prominent intradural vessels and mimics
• Descends in midline without interruption form • Normal variant (particularly dorsal veins, &. near
foramen magnulll to filum terminale conus)
• Reinforced by segmental feeders • CSF flow dephasing mimics vessel flow voids
• Gives rise to sulcal (central) branches supplying (particularly dorsal thoracic)
anterior 2/3 of cord • Collateral flow (lVC occlusion)
• Dominant thoracic anterior radicular = artery of • Enlarged nerve roots with chronic inflammatory
Adamkiewicz (left side, T9-T12 origin most common) demyelinating polyneuropathy (eIDP)
• Central canal stenosis with nerve root redundancy
Posterior spinal arteries • Tumor: Hemangioblastoma, schwan noma,
• Paired longitudinal system on dorsal cord medial to meningioma, ependymoma, paraganglioma
dorsal roots • Spinal dural fistulas (type 1)
• Plexiform and variable venous network between two • Spinal cord arteriovenous malformations (types 11, Ill)
dorsal arteries • Spinal intradural-peri medullary fistula (type IV)
• Supply posterior 1/3 of cord
• "Pauci-segmental" fewer vessels « 5) with larger • Central group provides return for anterior horns &.
caliber surrounding white matter => drain into central
• "Pluri-segmental" more vessels with smaller caliber veins in anterior median fissure => form anterior
o Dominant thoracic anterior radicular = artery of median vein
Adamkiewicz • Peripheral dorsal &. lateral cord drainage via small
• Left side origin (73%) valveless radial vein plexus => coronal venous
• T9-T12 origin (62%) plexus on cord surface => epidural venous plexus
• Lumbar origin (26%) of Batson
• T6-T8 (12%) • Epidural plexus connects with superior, inferior
o Posterior thoracic cord feeders => 9-12 (average 8) vena cavae, azygos &. hemiazygos systems,
• No right-left lateralization preference intracranial dural sinuses
• Vessel caliber 150-400 IJ o 30-70 medullary radicular veins
• Variable reporting of "great posterior radicular • No anterior or posterior dominance of veins
artery" o Anterior median vein continues caudally along
• Lumbosacral &. pelvic filum terminale to end of dural sac
o 0-1 major cord feeders o Coronal &. median veins drain => medullary veins
o Anterior spinal artery ends at conus with which leave intradural space at root sleeve into
communicating branches "rami cruciantes" to epidural plexus
posterior spinal artery • Medullary veins have functional valve-like
o Posterior division of iliac artery => inferior &. mechanism at dural margin preventing epidural
superior lateral sacral branches => spinal arteries via reflux into intradural space
anterior sacral foramina • No intradural valves
o Anterior division iliac artery => inferior gluteal artery
=> supplies sciatic nerve
o Posterior division internal iliac artery => iliolumbar IEMBRYOLOGY
artery artery supplies femoral nerve at iliac wing
level Embryologic Events
• Cord nutrient vessels • Spinal cord vessels originate from capillary network on
o Central &. peripheral systems ventrolateral surface of cord connected with segmental
o Central => ASA &. flow centrifugal aortic branches
o Peripheral => PSA, pial plexus &. flow centripetal • Two primitive longitudinal systems formed
o Dense capillary network in gray matter of cord • End of 2nd month ventrolateral systems transformed
• Veins into longitudinal solitary anteromedian anterior spinal
o Parallels spinal arteries artery
• Very symmetrical pattern of venous drainage • Plexus-like pattern remains more prominent on dorsal
(compared with highly asymmetric arterial side
supply) with minimal anterior-posterior, right-left, • ASA formation followed by variable regression of
segmental variation segmental feeders (initially 31), completed by 4th
o Two sets of intrinsic radial draining veins drain into month
anastomoses on cord surface o Reduction most pronounced in thoracic &. lumbar
areas
• Segmental arteries persist as intercostal and lumbar
arteries
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VASCULAR ANATOMY
1
4
Oblique coronal graphic of thoraticcord supply shows Coronal oblique graphic shows detail of dominant
anterior (arrow) & posterior (open arrow) radicular anterior spinal· artery in .anterior sulcus (arrow),· & two
arteries which anastomose toASA, PSA. posterior spinal arteries (open arrows).
I SELECTED REFERENCES
1. Caglar S et al: Extraforaminallumbar arterial anatomy.
Surg Neurol. 61(1):29-33; discussion 33, 2004
2. Williams GM et al: Preoperative selective intercostal
angiography in patients undergoing thoracoabdominal
aneurysm repair.] Vase Surg. 39(2):314-21, 2004
3. Marinkovie S et al: Microsurgical anatomy of the
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VASCULAR ANATOMY
I IMAGE GALLERY 1
5
Typical
(Left) Axial graphic shows
intercostal (arrow) arising
from aorta, giving spinal
branch (curved arrow),
dividing into ventral (open
white arrow), & dorsal (open
black arrow)
radiculomedullary arteries.
(Right) Axial graphic shows
ventral radiculomedullary
artery (RM) (arrow)
supplying anterior spinal
artery (curved arrow), &
posterior RM artery
supplying paired posterior
spinal arteries (open arrows).
Typical
(Left) Axial graphic shows
ventral (black arrow) &
dorsal (curved black arrow)
epidural plexus, basivertebral
vein (open black arrow),
ventral external plexus
(curved white arrow), IVC
(open white arrow). (Right)
Axial graphic shows normal
coronal plexus on cord
surface (white arrow),
ventral (open arrow) &
dorsal (curved arrow)
epidural plexus, basivertebral
vein (black arrow).
(Left) Anteroposterior
angiography of R vertebral
shows prominent segmental
feeder (arrow), so-called
"artery of cervical
enlargement" supplying
anterior spinal artery (open
arrows) (Courtesy M. Ahmed,
MO). (Right) Coronal
oblique CTA shows normal
hairpin turn of artery of
Adamkiewicz (arrow) in
thoracic spine.
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TYPE I DAVF
1
6
Sagittal Oblique graphic of thoracic cord shows site of Sagittal T2WI MR shows multiple flow voids on dorsal
type I dural fistula at dural root sleeve level (arrow), surface of cord due to enlarged veins. Extensive
with secondary dilatation of intradural venous plexus. increased signal in cord reflecting edema from venous
hypertension (arrows).
ITERMINOLOGY CT Findings
Abbreviations and Synonyms • CECT
o Enlarged distal spinal cord
• Synonyms: Dural fistula; type I spinal vascular o Enhancing pial veins on cord surface
malformation; radiculomeningeal fistula o Much more difficult to diagnose on routine CT than
• Abbreviations: DAVF, dural AVF, type I AVM MR imaging
Definitions • CTA
• Spinal arteriovenous (AV) fistula, present within dura, o Nidus in neural foramen shows focal enhancement,
with intradural distended draining veins with prominent intradural draining dilated veins
o Postprocessing with multiplanar reformats
mandatory
IIMAGING FINDINGS MR Findings
General Features • TlWI
o Cord enlarged, hypointense
• Best diagnostic clue: Abnormally enlarged,
• Abnormal small enhancing vessels on cord pial
hyperintense distal cord covered with dilated pial
surface
veins
• Location • T2WI
o Cord enlarged, hypointense
o Intradural, extramedullary flow voids from
• Multiple small abnormal vessel flow voids (dilated
distended draining veins
pial veins) on cord pial surface
o Most commonly occur at level of conus
• Edema spares cord periphery
• Size: Variable • Edema has "flame shaped" margins superiorly and
• Morphology: Serpentine vessels on cord surface inferiorly
Radiographic Findings • Low signal cord periphery on T2WI consistent
• Myelography: Multiple curvilinear filling defects due with venous hypertensive myelopathy
to dilated veins • STIR: Cord enlarged, hypointense
• Tl C+
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TYPE I DAVF
Key Facts
1
Terminology • MRA: Dynamic contrast-enhanced MRA capable of 7
• Spinal arteriovenous (AV) fistula, present within dura, defining dilated intradural veinsi may guide catheter
with intradural distended draining veins angiography
• Fistula may arise anywhere from vertebral artery to
-Imaging Findings internal iliac superiorly
• Best diagnostic clue: Abnormally enlarged, • CTA technically less demanding than MRAi is capable
hyperintense distal cord covered with dilated pial of superior resolution of nidus location, draining
veins veins
• Cord enlarged, hypointense • Use selective spinal arteriography to confirm
• Multiple small abnormal vessel flow voids (dilated diagnosis and direct treatment planning
pial veins) on cord pial surface
• Edema spares cord periphery
Clinical Issues
• Edema has "flame shaped" margins superiorly and • Middle-aged male with progressive lower extremity
inferiorly weakness exacerbated by exercise
o Multiple enhancing serpentine veins on cord surface • May be associated with prominent feeding intradural
o May show patchy, ill-defined enhancement within vessels
cord
• MRA: Dynamic contrast-enhanced MRA capable of Collateral venous flow
defining dilated intradural veinsi may guide catheter • IVC occlusion may cause prominent
angiography epidural/intradural veins
• Occasionally MR imaging normal or demonstrates • Check for small or ill-defined IVC, or metal artifact in
only abnormal cord signal IVC region due to filter
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TYPE I DAVF
1 o 80% of patients are male
o Usually presents in 5th or 6th decade (range 20-80)
o T2WI cord edema decreases over 1-4 months
following successful embolization
8 o Improved cord appearance on MR following
Gross Pathologic & Surgical Features treatment does not necessarily correlate with
• Most commonly occurs at thoracolumbar level (T5-L3) improved symptoms
• Usually located either adjacent to intervertebral
foramen or within dural root sleeve
• Arterial supply arises from dural branch of radicular I DIAGNOSTIC CHECKLIST
artery
• Intradural vein drains directly into cord pial veins Consider
• Frequently poor correlation between location of AV • Other type of spinal vascular malformation if evidence
shunt and clinical level of spinal dysfunction of intramedullary flow voids
• Rarely, clinically manifests as "subacute necrotizing • CTA effective for defining nidus, draining veins with
myelopathy" ("Foix-Alajounine syndrome") high spatial resolution
• Cervical type I AVM may drain intracranially with
Microscopic Features
high flow, varix formation and SAH
• True arteriovenous fistula, no intervening small vessel
network Image Interpretation Pearls
• Imaging & clinical findings are frequently subtle or
Staging, Grading or Classification Criteria
nonspecific; early diagnosis requires high level of
• Previously categorized as type IV spinal arteriovenous suspicion
malformation
• Anson-Spetzler classification into type IA (single
feeder) and lIB (multiple feeders) ISELECTED REFERENCES
1. Spetzler RF et al: Modified classification of spinal cord
ICLINICAL ISSUES 2.
vascular lesions. J Neurosurg (Spine 2).96: 145-156,2002
Van Dijk JM et al: Multidisciplinary management of spinal
Presentation dural arteriovenous fistulas: clinical presentation and
long-term follow-up in 49 patients. Stroke. 33(6): 1578-83,
• Most common signs/symptoms 2002
o Most common presentation is progressive lower 3. Farb RI et al: Spinal dural arteriovenous fistula localization
extremity weakness with both upper + lower motor with a technique of first-pass gadolinium-enhanced MR
neuron involvement angiography: initial experience. Radiology. 222(3): 843-50,
o Other signs/symptoms 2002
• Additional symptoms include back pain, 4. Mascalchi M et al: Spinal vascular malformations: MR
bowel/bladder dysfunction, impotence angiography after treatment. Radiology. 219(2): 346-53,
2001
• Thoracolumbar fistula location spares upper Song JK et al: Surgical and endovascular treatment of spinal
5.
extremities dural arteriovenous fistulas: long-term disability
• Very rarely presents with subarachnoid assessment and prognostic factors. J Neurosurg. 94(2
hemorrhage Suppl): 199-204, 2001
• Clinical profile 6. Hurst RW et al: Peripheral spinal cord hypointensity on
o Middle-aged male with progressive lower extremity T2-weighted MR images: a reliable imaging sign of venous
weakness exacerbated by exercise hypertensive myelopathy. AJNR AmJ Neuroradiol. 21(4):
o Time from symptom onset to diagnosis often 781-6,2000
7. Bowen BC et al: MR angiography of the spine. Magn Reson
delayed Imaging Clin N Am. 6(1): 165-78, 1998
Demographics 8. Anson J et al: Classification of spinal arteriovenous
malformations and implications for treatment. BNI
• Age: Usually presents in 5th or 6th decade Quarterly. 8: 2-8, 1992
• Gender: M > F
Natural History & Prognosis
• Slowly progressive clinical course over several years
leading to paraplegia
• Cord ischemia/venous congestive myelopathy is
reversible if treated early, but may become irreversible
when untreated
• Bowel/bladder dysfunction & impotence rarely
improve, even after successful obliteration of fistula
Treatment
• Endovascular fistula occlusion with permanent
embolic agents
• Surgical fistula obliteration
o 40-600/0 improve follOWing obliteration of fistula,
40-50% stabilize myelopathic symptoms
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TYPE I DAVF
IIMAGE GALLERY 1
9
(Left) Sagittal Tl C+ MR
shows multiple enhancing
serpentine vessels on
thoracic cord surface, both
ventral and dorsal, due to
enlarged draining veins.
(Right) Sagittal T2WI MR
shows enlarged draining
veins as multiple flow voids
surrounding cord. High T2
signal in cord tapers at both
ends, and tends to spare
periphery typical of venous
hypertensive edema.
Typical
(Left) Sagittal T2WI MR
shows typical intradural flow
voids from distended &
enlarged draining veins, with
cord edema (arrow). (Right)
Sagittal T2WI MR shows
diffuse thoracic cord edema
& enlargement, but no
prominent flow voids within
thecal sac. Angiography
revealed left Tl:? fistula.
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TYPE· II AVM
1
10
Sagittal oblique graphic of thoracic cord shows focal, Sagittal CTA shows focal enhanClilg nidus within cord at
compact intramedullary nidus of type /I arteriovenous C6level, with anterior and posterior draimiJg veins.
malformation (arrow).
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TYPE II AVM
Key Facts . .
1
Terminology • Type IV: Ventral fistula (venous vances dIsplace, 11
• Synonyms: Glomus AVM distort cord)
• Type I: Dural arteriovenous fistula (DAVF) • Type II: Supplied by anterior spinal artery (ASA) or
• Type II: Intramedullary glomus type AVM (similar to posterior spinal artery (PSA); nidus drains to coronal
brain AVM) venous plexus (on cord surface) which in turn drains
• Type III: Juvenile-type AVM (intramedullary, anterograde to extradural space
extramedullary) Pathology
• Type IV: Intradural extra/perimedullary AVF • Associated abnormalities: Aneurysms of feeding
Imaging Findings vessels in type II AVMs (40%)
• Type I: Flow voids with cord T2 hyperintensity Clinical Issues
• Type II: Intramedullary nidus (may extend to dorsal • Good outcome in type II (glomus) and IV
subpial surface) (perimedullary)
• Type III: Nidus may have extramedullary and • Type II: Surgical resection, + pre-op embolization
extraspinal extension (aneurysms, nidus)
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TYPE II AVM
1 • Type IV: Associated with Rendu-Osler-Weber
syndrome Treatment
12 • Etiology • Type I: Embolization or surgical resection
o Type II: Compact nidus, high flow, aneurysms • Type II: Surgical resection, + pre-op embolization
common (20-44%) (aneurysms, nidus)
o Type III: Large diffuse nidus, cord ischemia, venous • Type III: Complete resection generally not possible,
hypertension palliative therapy
o Type IV: Congenital; may be acquired after trauma; • Type IV: Embolization or surgical resection depending
(A) venous hypertension, (B, C) arterial steal, (C) upon staging and feeding vessels
cord compression
• Epidemiology
o Intramedullary (type II, III): 15-20% of spinal AVMs IDIAGNOSTIC CHECKLIST
o AVMs account for 10% of spinal masses
Consider
• Associated abnormalities: Aneurysms of feeding vessels
in type II AVMs (40%) • CTA as localization tool prior to catheter angiography
o Can define malformation type, location of ASA
Gross Pathologic & Surgical Features
Image Interpretation Pearls
• Type I: Peripheral direct fistula in dura
• Type II: Compact intramedullary nidus lacks normal • Definition of intramedullary involvement critical for
capillary bed classification, prognosis, treatment options
o No parenchyma within nidus; (nidus may have pial
extension)
• Type III: Large, complex intramedullary lesion, normal ISELECTED REFERENCES
neural parenchyma inside nidus (may involve 1. Spetzler RF et al: Modified classification of spinal cord
extramedullary, extradural) vascular lesions. J Neurosurg (Spine 2) 96: 145-156,2002
• Type IV: Direct fistula between ASA/PSA & draining 2. Mascalchi M et al: Spinal vascular malformations: MR
angiography after treatment. Radiology. 219(2): 346-53,
vein, no nidus 2001
Microscopic Features 3. Bemporad JA et al: Magnetic resonance imaging of spinal
cord vascular malformations with an emphasis on the
• Type II: Abnormal vessels with variable wall thickness, cervical spine. In Neuropathic basis for imaging.
internal elastic lamina Neuroimaging Clinics of North America 11:111-29, 2001
• Reactive change in surrounding tissue: Gliosis, cytoid 4. Mascalchi M et al: Contrast-enhanced time-resolved MR
bodies, Rosenthal fibers; hemosiderin deposition angiography of spinal vascular malformations. J Comput
common; +/- Ca++ Assist Tomogr. 23(3): 341-5, 1999
5. Hasegawa M et al: The efficacy of CT arteriography for
Staging, Grading or Classification Criteria spinal arteriovenous fistula surgery: technical note.
• Newest classification of AVMs into extra-intradural; Neuroradiology. 41(12): 915-9, 1999
intradural (intramedullary, compact, diffuse, conus; 6. Morgan MK: Outcome from treatment for spinal
subtyped by flow, size) arteriovenous malformation. Neurosurg Clin N Am.
10(1):113-9, 1999
7. Bao YH et al: Classification and therapeutic modalities of
spinal vascular malformations in 80 patients.
ICLINICAL ISSUES Neurosurgery. 40(1):75-81, 1997
8. Hasuo K et al: Contrast-enhanced MRI in spinal
Presentation arteriovenous malformations and fistulae before and after
• Most common signs/symptoms embolisation therapy. Neuroradiology. 38(7):609-14, 1996
o Type I: Progressive myelopathy 9. Mourier KL et al: Intradural perimedullary arteriovenous
o Type II: SAH most common symptom; pain, fistulae: results of surgical and endovascular treatment in a
myelopathy series of 35 cases. Neurosurgery. 32(6):885-91; discussion
891, 1993
o Type III: Progressive neurologic decline (weakness), 10. Theron J et al: Spinal arteriovenous malformations:
SAH advances in therapeutic embolization. Radiology.
o Type IV: Progressive conus/cauda equina syndrome, 158(1):163-9,1986
SAH 11. Horton JA et al: Embolization of intramedullary
• Clinical profile: Type II: Adult with acute arteriovenous malformations of the spinal cord. AJNR Am J
subarachnoid hemorrhage, myelopathy Neuroradiol. 7(1):113-8, 1986
• Pregnancy associated with worsening symptoms 12. Scialfa G et al: Embolization of vascular malformations of
the spinal cord. J Neurosurg Sci. 29(1):1-9, 1985
Demographics 13. Riche MC et al: Embolization of spinal cord vascular
malformations via the anterior spinal artery. AJNR Am J
• Age: Adult
Neuroradiol. 4(3):378-81, 1983
• Gender: M = F
Natural History & Prognosis
• Good outcome in type II (glomus) and IV
(perimedullary)
• Poor prognosis for juvenile (type III) AVM
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TYPE II AVM
IIMAGE GALLERY 1
13
(Left) Anteroposterior
angiography of right
vertebral injection shows
feeding vessels supplying
compact AVM nidus
involving cervical cord.
(Right) Axial T1 C+ MR
shows enhancing vessels
within right side of the
cervical cord, and extending
to pial surface (arrows).
(Left) Sagittal T1 WI MR
shows multiple flow voids
within cord at C6 level
(arrow), extending to dorsal
pial surface where they
merge with draining veins
(open arrow) (Right) Sagittal
T1 C+ MR with fat
suppression shows multiple
punctate & linear areas of
enhancement within cord
from AVM nidus, flow voids
dorsal to cord surface from
draining veins.
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Sagittal oblique graphic of thoracic cord shows large Sagittal T7 C+ MR shows multiple flow voids in cervical
complex intramedullary nidus of type 11/ arteriovenous cord, and along leptomeninges in this type 11/ AVM
malformaUon (arrows), with mulUple feeding vessels. Focal enhancement is within nidal aneurysm (arrow)
(Courtesy j. Egelhoff, DO).
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TYPE III AVM
Key Facts
1
Terminology • Type IV: Pial fistula (venous varices displace, distort 15
• Synonyms: Juvenile AVM; intramedullary - cord)
extramedullary AVM • MRA: Dynamic enhanced MRA capable of defining
• Type I: Dural arteriovenous fistula (DAVF) feeding enlarged arteries, nidus, and enlarged
• Type II: Intramedullary glomus type AVM (similar to draining veins
brain AVM) Pathology
• Type III: Juvenile-type AVM (large complex • Associated abnormalities: Type III associated with
intramedullary, extramedullary AVMs) Cobb syndrome (metameric vascular malformation
• Type IV: Intradural extra/perimedullary AVF with triad of spinal cord, skin, bone)
Imaging Findings Clinical Issues
• Type I: Flow voids with cord hyperintensity • Poor prognosis for juvenile (type III) AVM
• Type II: Intramedullary nidus (may extend to dorsal • Type III: Complete resection generally not possible,
subpial surface) palliative therapy
• Type III: Nidus may have extramedullary and
extraspinal extension
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Typical
(Left) Sagittal nWI MR of
cervical spine shows long
segment of multiple
intramedullary flow voids
extending onto cord surface
(Courtesy]. Egelhoff, 00).
(Right) Anteroposterior
angiography of type 1/1 AVM
with extradural involvement
(Cobb syndrome) shows
large draining veins both
intradural (arrows) and
extradural (open
arrows) (Courtesy ]. Egelhoff,
00).
Typical
(Left) Sagittal CTA with bone
subtraction shows
intramedullary nidus (arrow)
nearly encompassing entire
bony canal. (Right) Coronal
CTA shows nidus within left
side of cord, with multiple
feeding vessels (arrows).
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TYPE IVAVF
1
18
Coronal oblique graphic of thoracic cord shows Sagittal T2WI MR shows mulUple serpentine
intradural site of arteriovenous fistula (type IV) on dorsal intradural/extramedullary flow voids dorsal to thoracic
cord surface with diffuse venous engorgment (arrow). cord, plus focal cord abnormality (arrow) due to high
flow aneurysm.
Normal CSF Motion Type I Dural Fistula Tortuous Roots Intradural Tumor
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TYPE IV AVF
Key Facts
1
Terminology • Type III: Nidus may have extramedullary and 19
• Synonyms: Perimedullary fistula; type IV spinal extraspinal extension
vascular malformation • Type IV: Ventral fistula (venous varices displace,
• Type I: Dural arteriovenous fistula (DAVF) distort cord)
• Type II: Intramedullary glomus type AVM (similar to • Type IV: Feeding vessel from ASA or PSA connects
brain AVM) directly with spinal vein (no nidus)
• Type III: Juvenile-type AVM (intramedullary, Clinical Issues
extramedullary) • Type IV-A tends to surgical resection since normal
• Type IV: Intradural extra/perimedullary AVF (types A, caliber ASA is inaccessible to embolization
~q . • Type IV-B have dilated feeding vessels allowing
Imaging Findings embolization, or surgery
• Type I: Flow voids with cord hyperintensity • Type IV-C have high flow state, and most emendable
• Type II: Intramedullary nidus (may extend to dorsal to embolization
subpial surface)
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TYPE IV AVF
1 o Sporadic or syndromic • Gender: M = F
• Type I: None
20 • Type II: Associated with cutaneous angiomas, Natural History & Prognosis
Klippel-Trenaunay-Weber, Rendu-Osler-Weber • Type I: 40-60% improve following obliteration of
syndromes fistula, 40-50% stabilize myelopathic symptoms
• Type III: Associated with Cobb syndrome • Type II, IV: Generally good outcome
(metameric vascular malformation involving triad • Type III: Poor prognosis
of spinal cord, skin, bone) Treatment
• Type IV: Associated with Rendu-Osler-Weber • Type IV: Embolization or surgical resection based upon
syndrome anatomy and size: (A) surgical resection, (B) surgical
• Etiology resection or embolization, (C) embolization
o Type IV: Congenital o Type IV-A tends to surgical resection since normal
o May be acquired after trauma caliber ASA is inaccessible to embolization
• Epidemiology o Type IV-B have dilated feeding vessels allowing
o Intramedullary (type II, III): 15-20% of spinal AVM embolization, or surgery
o Type IV: 10-20% of spinal AVMs o Type IV-C have high flow state, and most
o AVMs account for < 10% of spinal masses emendable to embolization
• Associated abnormalities: Type IV: Associated with
Rendu-Osler-Weber syndrome
Gross Pathologic & Surgical Features IDIAGNOSTIC CHECKLIST
• Type I: Peripheral direct fistula in dura Consider
• Type II: Compact intramedullary nidus lacks normal
capillary bed; no parenchyma within nidus; (riidus • CTA as localization tool prior to catheter angiography
o Can define malformation type, location of ASA
may have pial extension)
• Type III: Large, complex intramedullary lesion, normal
neural parenchyma inside nidus (may involve
extramedullary, extradural)
ISELECTED REFERENCES
• Type IV: Direct fistula between ASA/PSA & draining 1. Hida K et al: Corpectomy: a direct approach to
vein, no nidus perimedullary arteriovenous fistulas of the anterior cervical
spinal cord.] Neurosurg. 96(2 Suppl): 157-61, 2002
Microscopic Features 2. Rodesch G et al: Classification of spinal cord arteriovenous
• Abnormal vessels with variable wall thickness, internal shunts: proposal for a reappraisal--the Bicetre experience
with 155 consecutive patients treated between 1981 and
elastic lamina
1999. Neurosurgery. 51(2): 374-9; discussion 379-80, 2002
• Reactive change in surrounding tissue: Gliosis, cytoid 3. Spetzler RF et al: Modified classification of spinal cord
bodies, Rosenthal fibers; hemosiderin deposition vascular lesions.] Neurosurg. 96(2 Suppl): 145-56,2002
common; +/- Ca++ 4. Mascalchi M et al: Spinal vascular malformations: MR
angiography after treatment. Radiology. 219(2): 346-53,
Staging, Grading or Classification Criteria 2001
• IV-A: Small AVF with slow flow, mild venous 5. Vates GE et al: Conus perimedullary arteriovenous fistula
enlargement with intracranial drainage: case report. Neurosurgery.
• IV-B: Intermediate AVF, dilated feeding arteries; high 49(2): 457-61; discussion 461-2,2001
flow rate 6. Sugiu K et al: Successful embolization of a spinal
perimedullary arteriovenous fistula with cellulose acetate
• IV-C: Large AVF, dilated feeding arteries; dilated,
polymer solution: technical case report. Neurosurgery.
tortuous veins 49(5): 1257-60; discussion 1260-1, 2001
7. Sure U et al: Spinal type IV arteriovenous malformations
(perimedullary fistulas) in children. Childs Nerv Syst.
ICLINICAL ISSUES 16(8): 508-15, 2000
8. Grote EH et al: Clinical syndromes, natural history, and
Presentation pathophysiology of vascular lesions of the spinal cord.
• Most common signs/symptoms Neurosurg Clin N Am. 10(1): 17-45, 1999
o Type I: Progressive myelopathy 9. Hida K et al: Results of the surgical treatment of
o Type II: SAH most common symptom; pain, perimedullary arteriovenous fistulas with special reference
to embolization. ] Neurosurg. 90(4 Suppl): 198-205, 1999
myelopathy 10. Mascalchi M et al: Contrast-enhanced time-resolved MR
o Type III: Progressive neurologic decline (weakness), angiography of spinal vascular malformations. ] Comput
SAH Assist Tomogr. 23(3): 341-5, 1999
o Type IV: Progressive conus/cauda equina syndrome, 11. Bao YH et al: Classification and therapeutic modalities of
SAH spinal vascular malformations in 80 patients.
• Type IV-A with slow flow, venous hypertension Neurosurgery. 40:75-81, 1997
primary cause of symptoms, no SAH 12. Ricolfi F et al: Giant perimedullary arteriovenous fistulas of
the spine: clinical and radiologic features and endovascular
• Types IV-B, C show arterial steal and cord
treatment. AlNR Am] Neuroradiol. 18(4): 677-87,1997
compression from venous varices, SAH
Demographics
• Age: Child and adult
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TYPE IV AVF
IIMAGE GALLERY 1
21
Typical
(Left) Sagittal T1 C+ MR
shows multiple enhancing
intradural vessels
surrounding distal cord.
(Right) Sagittal T2WI MR
shows high signal within
distal cord due to edema, &
more focal low signal
reflecting compressing
perimedullary vessels.
(Left) Anteroposterior
angiography shows vascular
malformation supplied by
radicular artery on pial
surface of cord. Shunting is
at cord level (type IV), not at
dural level (type I). (Right)
Coronal CTA shows similar
pattern to vascular
malformation, fed by right
radicular artery (arrow) with
dilated veins on the dorsal
cord (open arrow).
(Left) Sagittal T1 WI MR
shows multiple serpentine
flow voids dorsal to the
thoracic cord from the
intradural type IV fistula.
(Right) Anteroposterior
angiography shows the
fistular fed by right radicular
artery, with multiple dilated
perimedullary veins.
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CAVERNO'USMA[FORMAT.-ON,"SPINAL CORD
1
22
Sagittal T7WI MR (left) and T2WI (right) show well Axial T2* GRE MR shows associated multiple low signal
defined lesion with typical heterogeneous signal of intracranial cavernous malformations in patient with
cavernous malformation. Note prominent hemosiderin spinal cord cavernous malformation.
rim on T2WI (arrow).
DDx: Hemorrhagic~C:ordlesions
AVM Ependymoma'
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CAVERNOUS MALFORMATION, SPINAL CORD
Key Facts
1
Terminology Pathology 23
• Cavernous malformation (CM) • Thoracic (50%)
• Synonyms include cavernous angioma, cavernous • Cervical (40%)
hemangioma, "cryptic" vascular malformation • Conus (10%)
• Vascular lesion with lobulated, thin sinusoidal • Multiple (familial) CM syndrome (20%)
vascular channels, no interspersed neural tissue • Autosomal dominant, variable penetrance
• Familial CMs at high risk for hemorrhage, forming
Imaging Findings new lesions
• Best diagnostic clue: Locules of blood with fluid-fluid
levels surrounded by very T2 hypointense rim Clinical Issues
• Location: Spinal cord uncommon site, 3-5% of all • Sensorimotor deficits, progressive paraparesis
cavernous malformations • Gender: M:F = 1:2
Top Differential Diagnoses Diagnostic Checklist
• Intramedullary neoplasm • Scan brain for additional lesions!
• AVM
• Multiple sclerosis
• Astrocytoma: Multisegmental enhancing mass o Cranial irradiation suspected risk factor for cerebral
(hemorrhage uncommon) CMs
• Hemangioblastoma: Vascular nodule; "flow voids"
common Gross Pathologic & Surgical Features
• Metastatis: Enhancing mass with edema, hemorrhage • Discrete, lobulated blue-reddish brown
("mulberry-like") nodule
AVM • Pseudocapsule (gliotic, hemosiderin-stained cord)
• Enlarged flow voids with vascular nidus
• DSA reveals enlarged feeding arteries, nidus, early Microscopic Features
draining veins • Vascular spaces with single layer of endothelial cells
• Vascular spaces separated by collagenous walls without
Multiple sclerosis smooth muscle or elastin
• Clinical course of spinal cavernous malformations • Histologic features of arteries, capillaries and vein
resembles multiple sclerosis with chronicity, generally lacking
exacerbations • No intervening neural tissue between vascular spaces
• No blood byproducts/hemosiderin in MS o Ca++ rare (common in brain cavernous
malformations)
o Blood products in different stages of evolution
!PATHOlOGY o Areas of partial & complete thrombosis
• No true surrounding capsule
General Features o Surrounding neural tissue shows gliosis, atrophy,
• General path comments hemosiderin, calcium & iron deposits
o Identical to intracranial cavernous malformations
o Location
• Thoracic (50%)
• Cervical (40%)
ICLINICAL ISSUES
• Conus (10%) Presentation
• Genetics • Most common signs/symptoms
o Multiple (familial) CM syndrome (20%) o Sensorimotor deficits, progressive paraparesis
• Autosomal dominant, variable penetrance o Four clinical patterns
• Mutation in chromosomes 3, 7q • Multiple episodes of neurological deterioration,
(Hispanic-Americans) intermittent recovery
o Familial CMs at high risk for hemorrhage, forming • Slowly progressive neurological decline
new lesions • Sudden symptom onset with rapid decline
• Familial characterized by higher frequency (hours-days)
multiple CMs, infratentoriallocation, lower age at • Mild symptoms with acute onset, gradual decline
clinical presentation (weeks-months)
• Etiology: Angiogenically immature lesions with o Extremely rare: Subarachnoid hemorrhage
endothelial proliferation, increased neoangiogenesis; • Clinical profile: Young adult with sudden paraplegia
VEGF, I3FGF, TGF(){ expressed
• Epidemiology Demographics
o 10-30% multiple, familial • Age
o 70% of spinal CMs in females o Between 3rd and 6th decade
• Range = 12-88 years
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CAVERNOUS MALFORMATION, SPINAL CORD
1 • Peak = 4th decade
• Gender: M:F = 1:2
Suppl):65-70, 2000
12. Vishteh AG et al: Patients with spinal cord cavernous
24 malformations are at an increased risk for multiple
Natural History & Prognosis neuraxis cavernous malformations. Neurosurgery.
• Broad range of dynamic behavior (may progress, 45(1):30-2; discussion 33, 1999
13. Moriarity]L et al: The natural history of cavernous
enlarge, regress)
malformations. Neurosurg Clin N Am. 10(3):411-7, 1999
• De novo lesions may develop (especially in familial 14. Chabert E et al: Intramedullary cavernous malformations.]
CM syndrome) Neuroradiol. 26(4):262-8, 1999
• Clinical course varies from slow progression to acute 15. Zevgaridis D et al: Cavernous haemangiomas of the spinal
quadriplegia cord. A review of 117 cases. Acta Neurochir (Wien).
• Bleeding rate - 1-5% per lesion per year 141(3):237-45, 1999
16. Duke B] et al: Cavernous angiomas of the cauda equina:
Treatment case report and review of the literature. Surg Neurol.
• Conservative management if asymptomatic 50(5):442-5, 1998
o Follow with serial MRI 17. Naim-Ur-Rahman et al: Intramedullary ossified cavernous
• Surgical resection for symptomatic lesions angioma of the spinal cord: Case report. Br] Neurosurg.
12(3):267-70, 1998
o Long pre-treatment duration (- 3 years) 18. Huffmann BC et al: Treatment strategies and results in
o Post-operative outcome related to pre-operative spinal vascular malformations. Neurol Med Chir (Tokyo).
neurologic status 38 Suppl:231-7, 1998
o 66% improve, 28% stable, 6% deterioration 19. Padovani R et al: Cavernous angiomas of the spinal district:
post-operatively surgical treatment of 11 patients. Eur Spine]. 6(5):298-303,
1997
20. Turjman F et al: MRI of intramedullary cavernous
IDIAGNOSTIC CHECKLIST haemangiomas. Neuroradiology. 37(4):297-302, 1995
21. Harrison M] et al: Symptomatic cavernous malformations
Consider affecting the spine and spinal cord. Neurosurgery.
37(2):195-204; discussion 204-5, 1995
• Other hemorrhagic lesions if no classic speckled 22. Spetzger U et al: Cavernous angiomas of the spinal cord
pattern, or hemosiderin margins clinical presentation, surgical strategy, and postoperative
results. Acta Neurochir (Wien). 134(3-4):200-6, 1995
Image Interpretation Pearls 23. Canavero S et al: Spinal intramedullary cavernous
• Heterogeneous mass ("locules" of blood with "popcorn" angiomas: a literature meta-analysis. Surg Neurol.
appearance) surrounded by dark rim (hemosiderin) 41(5):381-8, 1994
• Scan brain for additional lesions! 24. Acciarri N et al: Surgical treatment of spinal cavernous
• Screen with GRE angiomas.] Neurosurg Sci. 37(4):209-15, 1993
25. Acciarri N et al: Spinal cavernous angioma: a rare cause of
subarachnoid hemorrhage. Surg Neurol. 37(6):453-6, 1992
26. Bourgouin PM et al: Multiple occult vascular
ISELECTED REFERENCES malformations of the brain and spinal cord: MRI diagnosis.
1. Sandalcioglu IE et al: Intramedullary spinal cord cavernous Neuroradiology. 34(2):110-1, 1992
malformations: clinical features and risk of hemorrhage.
Neurosurg Rev. 26(4):253-6, 2003
2. Musunuru K et al: Widespread central nervous system
cavernous malformations associated with cafe-au-Iait skin
lesions. Case report.] Neurosurg. 99(2):412-5, 2003
3. Nagib MG et al: Intramedullary cavernous angiomas of the
spinal cord in the pediatric age group: a pediatric series.
Pediatr Neurosurg. 36(2):57-63, 2002
4. Chen DH et al: Cerebral cavernous malformation: novel
mutation in a Chinese family and evidence for
heterogeneity.] Neurol Sci. 196(1-2):91-6, 2002
5. Mottolese C et al: Central nervous system cavernomas in
the pediatric age group. Neurosurg Rev. 24(2-3):55-71;
discussion 72-3, 2001
6. Balaban H et al: Multiple spinal intramedullary cavernous
angioma: case report. Clin Neurol Neurosurg. 103(2):120-2,
2001
7. Sure U et al: Endothelial proliferation, neoangiogenesis,
and potential de novo generation of cerebrovascular
malformations.] Neurosurg 94:972-7, 2001
8. Clatterbuck RE et al: The nature and fate of punctate (Type
IV) cavernous malformations. Neurosurg 49:26-32, 2001
9. Deutsch H et al: Pediatric intramedullary spinal cavernous
malformations. Spine. 26(18):E427-31, 2001
10. Zacharia IT et al: Co-eXisting spinal cord and brain
cavernous angiomas.] Assoc Physicians India. 49:835-7,
2001
11. Deutsch H et al: Spinal intramedullary cavernoma: clinical
presentation and surgical outcome. ] Neurosurg. 93(1
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CAVERNOUS MALFORMATION, SPINAL CORD
IIMAGE GALLERY 1
25
Typical
(Left) Sagittal TIWI MR
shows focal high signal
within cord at C2 due to
hemorrhage, which extends
inferiorly to C2-3. Superior
portion has weI/-defined
margins. (Right) Sagittal
T2WI MR shows
heterogeneous signal from
lesion in cord at C2, with
peripheral low signal
(hemosiderin).
Typical
(Left) Axial T2* GRE MR
shows cavernous
malformation as patchy low
signal within cervical cord
(arrow). (Right) Sagittal
TI WI MR (left side) shows
mixed signal within thoracic
cord with weI/-defined
margins. Hemorrhage
extends inferiorly into distal
cord (arrows). T2* GE study
(right side) shows blooming.
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SPINAL CORD INFARCTION
1
26
Sagittal TlWI MR (left side) is unremarkable. STIR MR Axial T2WI MR shows central hyperintensity involving
(right side) shows hyperintense foci in thoracic bodies gray matter of cord (arrow), typical for infarction. Low
(arrows) & cord (open arrows) due to bone + signal foci surrounding cord is CSF pulsation artifact
parenchymal infarcts. (open arrows).
ITERMINOLOGY MR Findings
• TlWI
Abbreviations and Synonyms o Sight cord expanSion, & decreased signal
• Spinal cord infarction (SCI); cord ischemia o Early may have no significant Tl signal abnormality
Definitions o Atrophy in late stage
o Focal hemorrhage conversion may occur with
• Permanent tissue loss in spinal cord due to vessel
occlusion, typically radicular branch of vertebral artery hyperintensity on TlWI & hypointensity on T2WI
o May see large vessel abnormalities such as aortic
(cervical cord) or aorta (thoracic & lumbar cord)
aneurysm or dissection
• T2WI
o T2 hyperintensity in gray matter, gray matter with
IIMAGING FINDINGS adjacent white matter, or entire cross sectional area
General Features of cord
• Best diagnostic clue: Focal hyperintensity on T2WI in o Marrow T2 hyperintensity in anterior vertebral body
slightly expanded cord or in deep medullary portion near endplate may be
• Location: Most frequent in thoracic cord because of present due to vertebral body infarct
arterial border zone • STIR
• Size: Variable, but usually> 1 vertebral body segment o Nonspecific increased signal in cord
• Morphology: Central hyperintensity on T2WI o Marrow hyperintensity may be present due to
vertebral body infarct
CT Findings • DWI: Shows abnormal restricted diffusion (as in brain
• NECT: Noncontributory with infarct)
• CTA • Tl C+: May show patchy ill-defined enhancement in
o Generally noncontributory subacute phase
o Wide variability in presence of intercostals arteries • MRA: Noncontributory since resolution of dynamic
with aortic aneurysm with thrombosis enhanced MRA not sufficient to define ASA
o Definition of anterior spinal artery (ASA) possible,
but technically challenging
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SPINAL CORD INFARCTION
Key Facts
1
Terminology • T2 hyperintensity in gray matter, gray matter with 27
• Spinal cord infarction (SCI); cord ischemia adjacent white matter, or entire cross sectional area of
• Permanent tissue loss in spinal cord due to vessel cord
occlusion, typically radicular branch of vertebral • Marrow T2 hyperintensity in anterior vertebral body
artery (cervical cord) or aorta (thoracic & lumbar or in deep medullary portion near endplate may be
cord) present due to vertebral body infarct
• DWI: Shows abnormal restricted diffusion (as in brain
Imaging Findings with infarct)
• Best diagnostic clue: Focal hyperintensity on T2WI in • T2WI sagittal and axial, 3 mm slice thickness; DWI of
slightly expanded cord cord
• Location: Most frequent in thoracic cord because of
arterial border zone Top Differential Diagnoses
• Focal hemorrhage conversion may occur with • Multiple sclerosis (MS)
hyperintensity on TlWI & hypointensity on T2WI • Spinal cord neoplasm
• May see large vessel abnormalities such as aortic • Idiopathic transverse myelitis
aneurysm or dissection • Type I dural fistula
o Embryology-anatomy
Angiographic Findings • Seven to eight of 62 (31 pairs) radicular arteries
• Conventional: Spinal artery occlusion supply spinal cord in three territories
Imaging Recommendations • Cervicothoracic territory includes the cervical cord
• T2WI sagittal and axial, 3 mm slice thickness; DWI of & first two or three thoracic segments, supplied by
cord anterior spinal artery from vertebral artery +
branches of the costocervical trunk
• Midthoracic territory includes fourth to eighth
IDIFFERENTIAL DIAGNOSIS thoracic segments, supplied by radicular branch
from aorta at T7 level
Multiple sclerosis (MS) • Thoracolumbar territory includes remainder of
• Peripheral in location thoracic segments + lumbar cord, supplied by
• Less than two vertebral segments in length artery of Adamkiewicz
• Less than half cross-sectional area of cord • Artery of Adamkiewicz usually originates from
• 90% incidence of associated intracranial lesions 9th, 10th, 11th, or 12th intercostal artery (75%),
• Relapsing & remitting clinical course less commonly from higher intercostal artery or
lumbar artery
Spinal cord neoplasm • Radicular arteries form one anterior & two
• Cord expansion invariably present posterior spinal arteries
• Diffuse or nodular contrast enhancement • Anterior spinal arteries + branches supply gray
• Extensive peri-tumoral edema matter + an adjacent mantle· of white matter
• Associated cystic changes • Posterior spinal arteries + branches supply
• Slower clinical onset one-third to one-half of periphery of cord
• Genetics
Idiopathic transverse myelitis o Rare: CADASIL (cerebral autosomal dominant
• Lesion centrally located arteriopathy with subcortical infarcts and
• 3 to 4 segments in length leukoencephalopathy)
• Occupying more than two-thirds of cord's • Mapped to chromosome 19q12
cross-sectional area • Subarachnoid vessel abnormalities including
• No associated intracranial lesions concentric thickening of media and adventitia
• Onset not quite as sudden • Etiology .
Type I dural fistula o Idiopathic
o Atherosclerosis
• May rarely show only cord expansion and edema on
T2WI, without prominent vessels o Thoracoabdominal aneurysm
• Typical findings include prominent enlarged o Aortic surgery
serpentine pial veins on cord surface o Systemic hypotension
o Infection
o Embolic disease
o Spinal arteriovenous malformation
I PATHOLOGY o Vasculitis
General Features o Dissection (vertebral or aortic)
• General path comments o Decompression sickness
o Coagulopathy
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SPINAL CORD INFARCTION
1 o Post anesthesia complication (epidural injection,
celiac plexus block) Image Interpretation Pearls
28 • Epidemiology: Rare, usually patients> SO • Classic imaging appearance: T2 hyperintensity
• Associated abnormalities: Atherosclerotic risk factors involving the anterior horn cells
such as hypertension, diabetes, cigarette smoking • Check for aortic aneurysm or dissection
I DIAGNOSTIC CHECKLIST
Consider
• Associated vertebral infarction seen as T2
hyperintensity allowing specific diagnosis of
nonspecific cord signal change
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SPINAL CORD INFARCTION
IIMAGE GALLERY 1
29
Typical
(Left) Axial OWl MR shows
restricted diffusion (high
signa/) from central gray
matter of cord (arrow).
Remainder of extramedullary
tissues low in signal due to
high b value utilized. (Right)
Axial T2WI MR shows
increased signal from central
gray matter, sparing cord
periphery. Patient also had
positive diffusion study for
acute infarct.
Typical
(Left) Sagittal T2WI MR in a
patient following aortic
aneurysm surgery shows
increased signal within distal
cord/conus due to infarct.
(Right) Sagittal T2WI MR
following multilevel
corpectomy (open arrow)
for spondylosis shows diffuse
hyperintensity + enlargement
of cervical cord due to
infarct (arrows).
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SUBARACflNOID·HEMQRRHAGE
1
30
Sagittal T7 C+ MR shows variable appearance of spinal Sagittal T2WI MR shows spinal subarachnoid
subarachnoid hemorrhage with nonenhancing clot in hemorrhage with intermediate signal linear clot in distal
caudal thecal sac (arrows) & mild inflammatory nerve thecal sac (arrows).
root enhancement (open arrow).
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SUBARACHNOID HEMORRHAGE
Key Facts
1
Terminology Imaging Findings 31
• Hemorrhage into spinal subarachnoid space from • Best diagnostic clue: Fluid-fluid level within thecal
variety of etiologies sac
• Trauma (> 50%) • Scan whole spinal axis, + brain if unknown etiology
• Lumbar puncture, epidural or intradural catheter
placement Pathology
• Aneurysmal SAH with spinal extension • Incidence of SAH 6/100,000 people annually
• Spinal arteriovenous malformations • 85% relate to aneurysm rupture
• Spinal ependymoma • Non-aneurysmal perimesencephalic hemorrhage 10%
• Spinal neurinoma • Primary spinal SAH 1%
• Bleeding diatheses Clinical Issues
• Anticoagulant therapy • Spinal SAH characterized by sudden headache, acute
• Infection (pneumococcal meningitis, herpes)
sciatic pain, xanthochromic CSF, meningeal
irritation, sensory deficit or paralysis
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SUBARACHNOID HEMORRHAGE
1 Staging, Grading or Classification Criteria
11. Berlis A et al: Subarachnoid haemorrhage due to cervical
spinal cord haemangioblastomas in a patient with von
32 • Hunt and Hess scale for SAH Hippel-Lindau disease. Acta Neurochir (Wien).
o I: Asymptomatic 145(11):1009-13; discussion lOB, 2003
o II: Severe headache or nuchal rigidity, no neurologic 12. Wells JB et al: Subarachnoid hemorrhage presenting as
deficit post-dural puncture headache: a case report. Mt Sinai J
Med. 69(1-2):109-10, 2002
o III: Drowsy, minimal neurologic deficit 13. Wityk RJ et al: Neurovascular complications of marfan
o IV: Stuporous, hemiparesis syndrome: a retrospective, hospital-based study. Stroke.
o V: Coma, decerebrate posturing 33(3):680-4, 2002
14. Fahy BG et al: Current concepts in neurocritical care.
Anesthesiol Clin North America. 20(2):441-62, viii, 2002
ICLINICAL ISSUES 15. Pai SB et al: Post lumbar puncture spinal subarachnoid
hematoma causing paraplegia: a short report. Neurol India.
Presentation 50(3):367-9,2002
16. Ruelle A et al: Spinal subarachnoid bleeding of unknown
• Most common signs/symptoms
etiology. Case reports. J Neurosurg Sci. 45(1):53-7, 2001
o Acute back or radicular pain ± signs of cord 17. Hausmann et al: Coagulopathy induced spinal intradural
compression (numbness, weakness) extramedullary hematoma: report of three cases and review
• Spinal SAH characterized by sudden headache, of the literature. Acta Neurochir (Wien). 143(2):135-40,
acute sciatic pain, xanthochromic CSF, meningeal 2001
irritation, sensory deficit or paralysis 18. Hosoda H et al: Mediastinal neurilemmoma complicated
• Massive spinal SAH may give acute cord with spinal subarachnoid hemorrhage. Jpn J Thorac
compression, paraplegia, fecal & urinary Cardiovasc Surg. 49(6):384-7, 2001
incontinence 19. Cihangiroglu M et al: Spinal subarachnoid hemorrhage
complicating oral anticoagulant therapy. Eur J Radiol.
Demographics 39(3):176-9,2001
20. Cruickshank AM: ACP Best Practice No 166: CSF
• Age: Adult> children spectrophotometry in the diagnosis of subarachnoid
• Gender: Predilection related to underlying etiology haemorrhage. J Clin Pathol. 54(11):827-30, 2001
• Ethnicity: Predilection related to underlying etiology
I DIAGNOSTIC CHECKLIST
Consider
• CT brain> 95% sensitive to SAH during first 24 hours
o Sensitivity at 1 week 50%
I SELECTED REFERENCES
1. Pancu D et al: EPs do not accept the strategy of "lumbar
puncture first" in subarachnoid hemorrhage. Am J Emerg
Med. 22(2):115-7, 2004
2. Yahiro T et al: Pseudoaneurysm of the thoracic
radiculomedullary artery with subarachnoid hemorrhage.
Case report. J Neurosurg. 100(3 Suppl):312-5, 2004
3. Koch C et al: Dural arteriovenous fistula of the lumbar
spine presenting with subarachnoid hemorrhage. Case
report and review of the literature. J Neurosurg. 100(4
Suppl):385-91, 2004
4. Shah KH et al: Incidence of traumatic lumbar puncture.
Acad Emerg Med. 10(2):151-4,2003
5. Kastenbauer S et al: Pneumococcal meningitis in adults:
spectrum of complications and prognostic factors in a
series of 87 cases. Brain. 126(Pt 5):1015-25, 2003
6. Chatterjee T et al: Pneumococcal meningitis masquerading
as subarachnoid haemorrhage. MedJ Aust. 178(10):505-7,
2003
7. Yamaguchi S et al: Spinal subdural hematoma: a sequela of
a ruptured intracranial aneurysm? Surg Neurol.
59(5):408-12; discussion 412,2003
8. Bertalanffy H et al: Isolated paramedullary
hemangioblastoma originating from the first cervical nerve
root: case report. Spine. 28(1O):E191-3, 2003
9. Rabinov JD et al: Endovascular management of
vertebrobasilar dissecting aneurysms. AJNR Am J
Neuroradiol. 24(7):1421-8, 2003
10. Nozaki K et al: Spinal intradural extramedullary cavernous
angioma. Case report. J Neurosurg. 99(3 Suppl):316-9, 2003
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SUBARACHNOID HEMORRHAGE
IIMAGE GALLERY 1
33
Typical
(Left) Sagittal T7 WI MR (left)
and T2WI MR (right) shows
CSF fluid level (arrow)
related to subarachnoid
hemorrhage (intracranial
aneurysm rupture). (Right)
Sagittal T7 C+ MR (left)
shows poor discrimination of
conus/cauda equina but no
intradural enhancement.
T2WI MR (right) shows
subarachnoid hemorrhage as
low signal (post lumbar
catheter placement).
Typical
(Left) Sagittal T7 WI MR (left)
show high signal
subarachnoid hemorrhage
layering dependently within
caudal thecal sac (arrow).
T2WI (right) shows blood as
decreased signal relative to
CSF. (Right) Axial NECT
following posterior fossae
tumor surgery shows
extensive subarachnoid
blood as intradural high
attenuation (arrow). Note
intrathecal post-operative
gas.
Typical
(Left) Axial T7WI MR shows
subarachnoid blood as
diffuse increased signal
within thecal sac, with
preserved dural margin & no
extra dural mass. Normal
nerve roots are defined by
high signal blood. (Right)
Sagittal T7WI MR shows
extensive subarachnoid
hemorrhage as increased
signal throughout lumbar
thecal sac (arrows), outlining
conus & cauda equina.
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SPONTANEOUS EPIQURAI..HEMATOMA
1
34
Sagittal T7WI MR through cervicothoracic spine shows Sagittal T2WI MR in same case demonstrates subacute
two lentiform shaped hyperintense ventral epidural hyperintense epidural hematomas (arrows). Padent had
hematomas with central stenosis. Thoracic dorsal no history of trauma, & underwent emergent
epidural fat is noted (arrows). laminectomy & evacuation of hematomas.
• Myelography
ITERMINOLOGY I o Block of cerebral spinal fluid
Abbreviations and Synonyms • Post-myelography CT confirms epidural location
• Spontaneous spinal epidural hematoma: SSEDH CT Findings
Definitions • NECT
o Hyperdense epidural mass
• Accumulation of hemorrhage between dura & spine
o Variable cord compression
not caused by significant trauma or iatrogenic
o Sagittal reformation better evaluates craniocaudal
procedures
extent
MR Findings
IIMAGING FINDINGS .... • TIWI
o Variable signal intensity with age of SSEDH
General Features
o Acute: < 48 hrs
• Best diagnostic clue: Extradural multisegmental Tl
• Isointense > hypo- or hyperintense
hyperintense fluid collection
o Subacute and chronic
• Location • Hyperintense> isointense
o Thoracic, lumbar> cervical
o Fat suppressed T1 WI to distinguish blood vs. fat
• Cervicothoracic region more common in children
o Dorsal> ventral> circumferential
• T2WI
o Heterogeneously hyperintense
• Size: Multi-level> single level o Hypointense foci due to deoxyhemoglobin or
• Morphology fibrous septae in epidural fat
o Broad-based
• PD/Intermediate: Diffusely hyperintense
o Lentiform or biconvex
• T2* GRE: Hyperintense with prominent hypointense
o Outlined by dorsal epidural fat cranially and
deoxyhemoglobin
rostrally
• Tl C+
Radiographic Findings o Focal enhancement in acute SSEH represents active
• Radiography: Noncontributory extravasation of contrast-enhanced blood
Metastasis Lipomatosis
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SPONTANEOUS EPIDURAL HEMATOMA
Key Facts
1
Terminology Top Differential Diagnoses 35
• Accumulation of hemorrhage between dura & spine • Epidural metastasis, lymphoma
not caused by significant trauma or iatrogenic • Disc extrusion, migration
procedures
Pathology
Imaging Findings • General path comments: Epidural venous
• Best diagnostic clue: Extradural multisegmental Tl hemorrhage ± minimal trauma
hyperintense fluid collection
• Thoracic, lumbar> cervical Clinical Issues
• Dorsal> ventral> circumferential • Acute onset of neck, back pain
• Hyperdense epidural mass • Rapid progression of neurologic impairment
• Variable signal intensity with age of SSEDH • Favorable clinical and imaging outcome with
• T2* GRE: Hyperintense with prominent hypointense conservative management
deoxyhemoglobin • Severity and duration of neurologic deficit predictive
• Focal enhancement in acute SSEH represents active of post-operative neurologic recovery
extravasation of contrast-enhanced blood
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SPONTANEOUS EPIDURAL HEMATOMA
IIMAGE GALLERY 1
37
(Left) Sagittal T7 WI MR
through thoracic spine shows
two subacute hyperintense
SSEDHs, one ventral (arrow)
and one dorsal (open
arrow). Isointense foci are
present at rostral aspect of
dorsal SSEDH. (Right)
Sagittal T2WI MR shows
multisegmental epidural
hematomas (arrows), which
are hyperintense. Spinal
stenosis and cord
compression are evident.
(Left) Sagittal T7 WI MR
through thoracic spine
demonstrates an extensive
homogenous hyperintense
dorsal epidural collection
(arrows). (Right) Axial T7 WI
MR with fat-saturation in
same patient demonstrates
persistent hyperintensity,
compatible with a subacute
epidural hematoma.
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SUBDURAL-HEMATOMA
1
38
Sagittal TlWI MR through lumbosacral spine shows Axial TlWI MR confirms the subdural location of both
distal ventral and dorsal intraspinal hyperintense ventral and dorsal subacute hematomas, causing severe
collections (arrows)_ The dorsal collection is outlined by central stenosis.
cauda equina and epidural fat
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SUBDURAL· HEMATOMA
Key Facts
1
Terminology Top Differential Diagnoses 39
• Accumulation of blood between dura, arachnoid • Epidural hematoma
• Subdural abscess
Imaging Findings • CSF leakage syndrome
• Best diagnostic clue: Intradural collection
hyperintense on TlWI, predominantly hypointense Pathology
on T2WI or gradient-echo imaging • Iatrogenic cause factor in 2/3 of those with abnormal
• Thoracolumbar> lumbar or lumbosacral> cervical coagulation parameters
• Clumped, loculated masses of hemorrhagic
density/intensity Clinical Issues
• Outlined by epidural fat & relatively low density cord • Acute onset of neck or back pain
• Heterogeneous, variable signal intensity depending • Radicular pain
on duration of SSDH • May resolve spontaneously
• T2* GRE: Significant hypointense portion due to • Decompressive laminectomy with clot evacuation
deoxyhemoglobin
• Tl C+: ± Slight enhancement
• STIR: Hyperintense
• T2* GRE: Significant hypointense portion due to Spinal meningitis
deoxyhemoglobin • Normal or increased CSF intensity on TlWI
• Tl C+: ± Slight enhancement • Indistinct cord-CSF interface
• Variable mass effect on spinal cord and cauda equina • Diffusely enhancing CSF
o Hyperintense cord edema on T2WI • Smooth or nodular meningeal/nerve root
enhancement
Imaging Recommendations • ± Cord edema/swelling
• Best imaging tool
o Sagittal and axial T2WI and T1 WI MR Idiopathic hypertrophic spinal
o Spinal angiography not indicated unless findings of pachymeningitis
arteriovenous malformation present • Diagnosis of exclusion
• Unknown etiology
• Fibrous inflammatory thickening of dura mater
IDIFFERENTIAL DIAGNOSIS • Thickened dura iso- to hypointense on TlWI and
T2WI
Epidural hematoma o Marked post-contrast-enhancement
• Thoracolumbar spine most common
• "Capping" of hematoma by dorsal epidural fat on
sagittal imaging IPATHOLOGY
• Directly adjacent to osseous structures with effacement
of epidural fat General Features
o Not limited by dura • General path comments
• Iso- to hyperintense on TlWI acute, hyperintense o Embryology-anatomy
subacute to chronic • Inner dura (dural border cell layer) structurally
• Hyperintense on T2WI with hypointense weaker than outer layer
deoxyhemoglobin • May tear during trauma
• ± Central or peripheral enhancement • Creating potential space within dural border cell
layer
Subdural abscess
• SSDH forms between strong external dura and
• Thoracolumbar spine most common arachnoid
• Multi-segmental dural/subdural thickening • True spinal subdural space demonstrated in
• Obliterated CSF human autopsy studies
• Isointense on TlWI, hyperintense on T2WI o Other proposed mechanisms of SSDH formation
• Diffuse vs. rim-enhancement • Rupture of valveless radiculomedullary veins that
CSF leakage syndrome cross subdural and subarachnoid spaces
• Spontaneous or from prior spinal trauma, diagnostic • Rupture of extra-arachnoidal vessels on inner
or interventional procedure, or spontaneous dural surface
• Low opening pressure on lumbar puncture • Genetics: No genetic predilection
• Dural venous engorgement and enhancement • Etiology
• Diffuse smooth meningeal thickening, enhancement o Trauma
• May see cerebellar tonsillar descent, effaced o Bleeding diathesis: 54% of reported cases
pre-pontine space in posterior fossa • Anticoagulation
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SUBDURAL HEMATOMA
1 • Coagulopathy: Hemophilia, leukemia,
thrombocytopenia, polycythemia, etc.
• Concurrent subarachnoid hemorrhage portends poor
outcome
40 o Iatrogenic cause factor in 2/3 of those with
abnormal coagulation parameters Treatment
• Lumbar puncture • Decompressive laminectomy with clot evacuation
• Spinal anesthesia o Indicated with severe & progressive deterioration of
o Neoplasm neurologic symptoms
o Arteriovenous malformation • Non-operative management
o Post-operative complication o In cases of mild symptoms at presentation
o Spontaneous: 15% o In patients with rapid neurologic recovery
• Epidemiology: Much less common than spinal o Analgesics
epidural hematoma o ± Intravenous dexamethasone to decrease
• Associated abnormalities inflammatory reaction and cord edema
o In the setting of trauma
• Vertebral fracture-dislocation
• Posttraumatic disc herniation IDIAGNOSTIC CHECKLIST
• Hemorrhagic cord contusion: Hyperintense on Consider
T2WI with focal hypointensity
o Subarachnoid and epidural hemorrhage • NECT to confirm intradural location + hyperdense
acute hematoma if MR findings equivocal
Gross Pathologic & Surgical Features
Image Interpretation Pearls
• Subdural (partially) organized blood clot under
pressure • Intradural extramedullary collection hyperintense on
o Bluish tense dura TlWI and predominantly hypointense on T2WI or
gradient-echo imaging without significant
• ± Subarachnoid/epidural hemorrhage
enhancement characteristic of SSDH
Microscopic Features
• Hemosiderin
• Fibrin and histiocytes ISELECTED REFERENCES
• Little or no fibroadipose tissue 1. Hung KS et al: Traumatic spinal subdural hematoma with
spontaneous resolution. Spine. 27(24):E534-8, 2002
2. Boukobza M et al: Spinal subdural haematoma: a study of
ICLINICAL ISSUES three cases. Clin Radiol. 56(6):475-80, 2001
3. Adler MD et al: Acute hemorrhagic complication of
Presentation diagnostic lumbar puncture. Pediatr Emerg Care.
17(3):184-8,2001
• Most common signs/symptoms
4. Hausmann et al: Coagulopathy induced spinal intradural
o Acute onset of neck or back pain extramedullary haematoma: report of three cases and
o Radicular pain review of the literature. Acta Neurochir (Wien).
o Other signs/symptoms 143(2):135-40,2001
• Headache 5. Maeda M et al: Nonsurgical treatment of an upper thoracic
• Meningismus spinal subdural hemorrhage. Spinal Cord. 39(12):657-61,
• Paraplegia 2001
• Paresthesia 6. Abla AA et al: Spinal chronic subdural hematoma.
Neurosurg Clin N Am. 11(3):465-71, 2000
• Bladder/bowel dysfunction
7. Egede LE et al: Spinal subdural hematoma: a rare
• Diminished deep tendon reflexes complication of lumbar puncture. Case report and review
• Clinical profile of the literature. Md Med]. 48(1):15-7, 1999
o Neurologic impairment may be delayed 8. Domenicucci M et al: Nontraumatic acute spinal subdural
• Onset of neurologic deficits varies from hours to > hematoma: report of five cases and review of the literature.
1 week J Neurosurg. 91(1 Suppl):65-73, 1999
o Chronic SSDH may not have significant pain 9. Longatti PL et al: Spontaneous spinal subdural hematoma.
o Mimics subarachnoid hemorrhage clinically Journal of Neurosurgical Sciences. 38:197-9,1994
10. Donovan MJ et al: Acute spinal subdural hematoma: MR
Demographics and CT findings with pathologic correlates. AJNR.
15:1895-905, 1994
• Age 11. Haines DE et al: The "subdural" space: a new look at an
o All ages affected
outdated concept. Neurosurgery. 32(1):111-20, 1993
o Slightly increased incidence in those 2: 60
12. Calhoun JM et al: Spontaneous spinal subdural hematoma:
• Gender: M = F case report and review of the literature. Neurosurgery.
• Ethnicity: No racial predilection 29(1):133-4, 1991
13. Spanu G et al: Spinal subdural haematoma: a rare
Natural History & Prognosis complication of lumbar puncture. Case report and review
• May resolve spontaneously of the literature. Neurochirurgia (Stuttg). 31(5):157-9, 1988
• Favorable clinical and imaging outcome in those
suitable for conservative management
• > 40% of patients undergoing surgery have near
complete neurologic recovery
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SUBDURAL HEMATOMA
IIMAGE GALLERY 1
41
Typical
(Left) Sagittal Tl WI M R
through thoracolumbar spine
in patient with leukemia +
recent lumbar puncture
demonstrates diffuse subtle
ventral intraspinal collection
(arrows), iso- to slightly
hyperintense. (Right) Sagittal
T2WI MR shows same
ventral collection, with
loculation and prominent
hypointense areas.
Typical
(Left) Axial T2WI MR
through lumbar vertebra
shows subdural location of
this acute hematoma, with
mass effect in thecal sac.
(Righi) Sagittal TlWI MR
through lumbosacral spine in
another patient shows
extensive ventral and
minimal dorsal (arrow)
hyperintense subdural
hematomas.
Typical
(Left) Sagittal TlWI MR in
patient with chronic neck
pain demonstrates diffuse
ventral and dorsal intraspinal
hyperintense collections,
with intracranial extension.
(Right) Axial TlWI MR
through cervical vertebra
shows thin circumferential
hyperintense chronic
subdural hematoma.
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SUPERFICIAL SIDEROSIS
1
42
Axial T2WI MR shows multiple areas of curvilinear low Sagittal T2WI MR of conus (left) & cervical cord (right)
signal coating surface of brainstem & cerebellar folia shows diffuse low signal involVing surface of cord, with
(arrows) due to hemosiderin deposition. relative hyperintensity of normal central cord.
ITERMINOLOGY MR findings
• TIWI: Usually normal, occasional low signal on cord
Abbreviations and Synonyms surface
• Synonyms: Central nervous system siderosis,
• T2WI
hemosiderosis o Linear low signal involVing cord surface, nerve roots
• Abbreviation: Superficial siderosis (SS), sensorineural o Look for typical surface involvement of cerebellum
hearing loss (SNHL) brainstem '
Definitions • T2* GRE
• Recurrent subarachnoid hemorrhage (multiple o Linear low signal involving cord surface, nerve roots
etiologies) causing hemosiderin deposition on cord, o Findings more pronounced than with T2WI, TlWI
nerve surface • Tl C+: No enhancement of leptomeninges
Imaging Recommendations
• Best imaging tool
I IMAGING FINDINGS o MR brain/spine
General features o Diagnosis of siderosis necessitates search for
underlying cause of repetitive subarachnoid
• Best diagnostic clue: Diffuse hypointensity of
hemorrhage
cord/brain surface on T2WI, gradient echo images
• Protocol advice
• Location: Cerebellum, brainstem, cerebrum, cord, oBrian MR: TlWI, T2WI, FLAIR, post-contrast TlWI,
cranial nerves
T2* GRE .
• Size: Linear low signal < few mm in thickness o Spine: Scan total spine with sagittal T2WI, T2* GRE,
• Morphology: Curvilinear, following surface contour of
post-contrast Tl WI
involved CNS structure
CT findings
• NECT: Cord, brain volume loss
• CECT: No enhancement
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SUPERFICIAL SIDEROSIS
Key Facts
1
Terminology Pathology 43
• Synonyms: Central nervous system siderosis, • Hemosiderin coating surface of cerebellum,
hemosiderosis brainstem, spinal cord
• Recurrent subarachnoid hemorrhage (multiple • Extends to & involves pial, subpial, subependymal
etiologies) causing hemosiderin deposition on cord, surfaces
nerve surface • Variable neuronal loss, reactive gliosis, demyelination
• Rare, insidious, progressive abnormality
Imaging Findings
• Best diagnostic clue: Diffuse hypointensity of Clinical Issues
cord/brain surface on T2WI, gradient echo images • Bilateral sensorineural hearing loss (95%)
• Ataxia (88%)
Top Differential Diagnoses • Pyramidal signs (76%)
• MR artifact • Dementia (20%)
• Subdural hemorrhage • Nystagmus (19%)
• Cord central T2 hyperintensity • Idiopathic (25-46%)
• Cord peripheral T2 hypointensity in dural fistula
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SUPERFICIAL SIDEROSIS
1 • Dementia (20%) 5. Padberg M et al: Cerebral siderosis: deafness by a spinal
tumor.] Neurol. 247(6):473, 2000
• Nystagmus (19%)
44 • Anosmia (17%) 6. Bostantjopoulou S et al: Superficial CNS siderosis and
spinal pilocytic astrocytoma. Neurology. 55(3):450, 2000
o Poor correlation of MR findings with clinical
7. Pelak VS et al: Evidence for preganglionic pupillary
symptoms involvement in superficial siderosis. Neurology.
o Cause of recurrent subarachnoid hemorrhage found 53(5):1130-2, 1999
in 50% 8. Manfredi M et al: Superficial siderosis of the central
• Post-operative change with friable surgical cavity nervous system and anticoagulant therapy: a case report.
(hemispherectomy) Ital] Neurol Sci. 20(4):247-9,1999
• Traumatic nerve root avulsion 9. Lemmerling Met al: Secondary superficial siderosis of the
• Neoplastic hemorrhage (35%); ependymoma most central nervous system in a patient presenting with
sensorineural hearing loss. Neuroradiology. 40(5):312-4,
common tumoral cause
1998
• Arteriovenous malformation 10. Schievink WI et al: Surgical treatment of superficial
• Aneurysm siderosis associated with a spinal arteriovenous
• Cavernous malformation (0.5-0.9% of population malformation. Case report.] Neurosurg. 89(6):1029-31,
affected) 1998
o Idiopathic (25-46%) 11. Pyhtinen] et al: Superficial siderosis in the central nervous
• Clinical profile system. Neuroradiology. 37(2):127-8, 1995
o Classic description: Sensorineural hearing loss} 12. Lai MT et al: Superficial siderosis of the central nervous
system: a case with an unruptured intracranial aneurysm. ]
cerebellar ataxia, dementia, myelopathy
LaryngolOtol. 109(6):549-52, 1995
(uncommon to see all manifestations) 13. Bonito V et al: Superficial siderosis of the central nervous
o CSF analysis system after brachial plexus injury. Case report. ]
• Xanthochromia Neurosurg. 80(5):931-4, 1994
• Elevated RBC count 14. Pribitkin EA et al: Superficial siderosis of the central
• Elevated iron & ferritin levels nervous system: an underdiagnosed cause of sensorineural
• May be normal hearing loss and ataxia. Am] Otol. 15(3):415-8, 1994
15. River Y et al: Superficial hemosiderosis of the central
Demographics nervous system. Mov Disord. 9(5):559-62, 1994
• Age: Adult and children (average age 50 years) 16. Bracchi M et al: Superficial siderosis of the CNS: MR
• Gender: M:F = 3:1 diagnosis and clinical findings. A]NR Am] Neuroradiol.
14(1):227-36,1993
• Ethnicity: No predilection 17. Kumar A et al: Posterior fossa surgery: an unusual cause of
Natural History & Prognosis superficial siderosis. Neurosurgery. 32(3):455-7; discussion
457, 1993
• Preclinical phase often prolonged (> 10 years) 18. Grunshaw ND et al: Superficial siderosis of the central
o Reports of SS developing> 30 years following initial nervous system--diagnosis by magnetic resonance imaging.
surgical procedure Clin Radiol. 48(3):186-8, 1993
19. Parnes SM et al: Superficial siderosis of the central nervous
Treatment system: a neglected cause of sensorineural hearing loss.
• Treat underlying abnormality by surgical or Otolaryngol Head Neck Surg. 107(1):69-77, 1992
endovascular means
o AVM, aneurysm, tumor
• Cochlear implant for SNHL
• Iron/copper chelates unsuccessful for treatment
IDIAGNOSTIC CHECKLIST
Consider
• Superficial siderosis is secondary to repetitive
subarachnoid hemorrhage
o Look for underlying etiology in brain/spine
ISELECTED REFERENCES
1. Messori A et al: The importance of suspecting superficial
siderosis of the central nervous system in clinical practice. ]
Neurol Neurosurg Psychiatry. 75(2):188-90,2004
2. Anderson NE: Late complications in childhood central
nervous system tumour survivors. Curr Opin Neurol.
16(6):677-83, 2003
3. Yoshida S et al: Superficial siderosis from spinal teratoma.
Lancet. 360(9345):1539, 2002
4. Polidori MC et al: Superficial siderosis of the central
nervous system: a 70-year-old man with ataxia, depression
and visual deficits. Gerontology. 47(2):93-5, 2001
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SUPERFICIAL SIDEROSIS
IIMAGE GALLERY 1
45
Typical
(Left) Sagittal T2WI MR
shows large filum terminale
ependymoma with drop
metastases (arrows),
superficial siderosis of cauda
equina with linear low signal
(open arrow). (Right) Sagittal
T2WI MR of recurrent
ependymoma (open arrows)
shows superficial siderosis
extending cephalad along
cord (arrows).
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PAGET DISEASE
2
2
Axial graphic of Pagetic vertebra demonstrates enlarged Axial NECT shows thoracic body with coarse .&
vertebral body, pedicles, and left facet with trabellular thickened trabeculae, focal areas or fat attenuation.
thickening and increased fatty marrow.
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PAGET DISEASE
Key Facts
Terminology Top Differential Diagnoses
• Osteitis deformans • Osteoblastic metastases
• Chronic metabolic disorder of abnormal bone • Vertebral hemangioma
remodeling in adult skeleton
Pathology
Imaging Findings • Possible viral etiology 2
• Best diagnostic clue: Enlarged vertebra with • 3-4% of adults over 40
trabecular coarsening and cortical thickening • Sarcomatous transformation: < 1% 3
• Location: Lumbar spine most common: L3, L4 • Uncommon in skull & spine relative to high
• "Picture frame" vertebra prevalence of disease in axial skeleton
• Diffusely dense "ivory" vertebra
• TlWI: Hypointense cortex + thickened trabeculae Clinical Issues
• Variable degree of spinal stenosis + foraminal • Deep, dull bone pain
narrowing • Disease process may be halted with return of normal
• Diffusely increased uptake in enlarged vertebra osseous architecture after medical treatment
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PAGET DISEASE
• Osteosarcoma: 50-60% • Poor prognosis when sarcomatous degeneration
• Fibrosarcoma/MFH: 20-25% present
• Chondrosarcoma: 10% o 3 year mortality rate: > 90%
• Histologic distinction of secondary sarcoma not
critical due to high degree of anaplasia Treatment
• Osseous destruction, enhancing soft tissue mass • Analgesic medications
with necrosis, metastases • Medications to inhibit osteoclastic bone resorption &
2 Gross Pathologic & Surgical Features
relieve pain
o Bisphosphates: Alendronate (Fosamax), Pamidronate
4 • Newly formed bone abnormally enlarged, deformed, (Aredia), etc.
weak o Calcitonin
o Mithramycin
Microscopic Features • Surgical decompression of spinal stenosis
• Active phase: Osteolytic phase
o Aggressive bone resorption
o Giant osteoclasts with multiple nuclei and inclusion IDIAGNOSTIC CHECKLIST
bodies
o Osteoclastic> osteoblastic activity Image Interpretation Pearls
o Replacement of hematopoietic bone marrow by • Expanded and square vertebra with cortical thickening
fibrous connective tissue and central lucency ("picture frame") highly suggestive
o Increased vascular channels of Paget disease
• Mixed blastic-lytic phase
o Increasing osteoblastic activity eventually surpasses
bone resorption
o Disorganized trabecular pattern
ISELECTED REFERENCES
1. Spieth ME et al: Positron emission tomography and Paget
• "Mosaic" or "jigsaw": Multiple remodeling lines disease: hot is not necessarily malignant. Clin Nucl Med.
o "Pumice" bone 28(9):773-4, 2003
• Weak bone lacking normal interconnections 2. Saifuddin A et al: Paget's disease of the spine: unusual
• Inactive quiescent phase features and complications. Clin Radiol. 58(2):102-11, 2003
o Decreased bone turnover 3. Whitehouse RW: Paget's disease of bone. Semin
• Diminishing osteoblasts, osteoclasts Musculoskelet Radiol. 6(4):313-22, 2002
o Loss of excessive vascularity 4. Smith SE et al: From the archives of the AFIP. Radiologic
spectrum of Paget disease of bone and its complications
o Fatty marrow
with pathologic correlation. Radiographies.
22(5):1191-216,2002
5. Vande Berg BC et al: Magnetic resonance appearance of
ICLINICAL ISSUES uncomplicated Paget's disease of bone. Semin
Musculoskelet Radiol. 5(1):69-77, 2001
Presentation 6. Hadjipavlou AG et al: Paget's disease of the spine and its
• Most common signs/symptoms management. Eur Spine]. 10(5):370-84,2001
o Deep, dull bone pain 7. Poncelet A: The neurologic complications of Paget's
o Other signs/symptoms disease. J Bone Miner Res. 14 SuppI2:88-91, 1999
8. Boutin RD et al: Complications in Paget disease at MR
• Asymptomatic: 20% imaging. Radiology. 209:641-51, 1998
• Myelopathy, cauda equina syndrome from canal 9. Cook GJ et al: Fluorine-18-FDG PET in Paget's disease of
narrowing bone. J Nucl Med. 38(9):1495-7, 1997
• Radiculopathy from foraminal stenosis 10. Klein RM et al: Diagnostic procedures for Paget's disease.
• Elevated serum alkaline phosphatase, uriD" Radiologic, pathologic, and laboratory testing. Endocrinol
hydroxyproline Metab Clin North Am. 24(2):437-50,1995
• Clinical profile 11. Mirra JM et al: Paget's disease of bone: review with
o Sarcomatous transformation emphasis on radiologic features, Part 1. Skeletal Radiol.
24(3):163-71, 1995
• Severe pain at site of previous asymptomatic 12. Mirra JM et al: Paget's disease of bone: review with
disease with large lytic lesion + soft tissue mass emphasis on radiologic features, Part II. Skeletal Radiol.
24(3):173-84, 1995
Demographics 13. Hadjipavlou A et al: Malignant transformation in Paget
• Age disease of bone. Cancer. 70(12):2802-8, 1992
o 55-85 yo 14. Ryan MD et al: Spinal manifestations of Paget's disease.
• Rare < 40 yo Aust N ZJ Surg. 62(1):33-8, 1992
• Gender: M:F = 3-4:2 15. Roberts MC et al: Paget disease: MR imaging findings.
• Ethnicity Radiology. 173:341-5, 1989
o Caucasians of Northern Europeans descent 16. Vellenga CJ et al: Bone scintigraphy and radiology in
o Rare in Asians and African-Americans Paget's disease of bone: a review. Am J Physiol Imaging.
3(3):154-68, 1988
Natural History & Prognosis 17. Frame B et al: Paget disease: A review of current knowledge.
Radiology. 141:21-4, 1981
• Disease process may be halted with return of normal
osseous architecture after medical treatment
o Long term remission expected
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PAGET DISEASE
IIMAGE GALLERY
Typical
-------------- (Left) Anteroposterior
radiography of
thoracolumbar shows a
diffusely dense "ivory" L7
vertebra. Differential
2
diagnoses also include 5
lymphoma and sclerotic
metastasis. (Right) Axial
TlWI MR in another patient
through a lumbar vertebra
shows coarse and irregular
trabeculae with mild
vertebral expansion
(Courtesy M. Modic, MD).
Typical
(Left) Sagittal TlWI MR
through lumbar spine
demonstrates abnormal L2-4
vertebrae. There is mild
vertebral enlargement with
coarse trabeculae. Increased
fatty marrow is present in L3
and L4. (Right) Axial T2WI
MR in same patient
demonstrates a diffusely
coarse and haphazard
trabecular pattern, in
vertebral body and posterior
elements.
Typical
(Left) Sagittal TlWI MR of
lumbar spine shows a slightly
enlarged L2 vertebra in
anterior-posterior dimension.
Increased fatty marrow and
mild compression are
present. (Right) Sagittal
TlWI MR shows low signal
with thickened cortex from
Tl body and spinous
process. Fatty marrow signal
and expansion most obvious
for spinous process.
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HYPERPARATHYROIDISM
2
6
Coronal graphic shows severe hyperparathyroidism with Sagittal bone CT shows diffuse osteopenia and endplate
extensive endplate erosions and thinned trabeculae. erosion (arrow).
DDx: Hyperparathyroidism
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HYPERPARATHYROIDISM
Key Facts
Terminology • Soft tissue calcifications
• Increased levels of hyperparathyroid hormone Top Differential Diagnoses
Imaging Findings • Multiple myeloma
• Osteopenia • Renal osteodystrophy
• Cortical thinning •
•
Senile osteoporosis
Seronegative spondyloarthropathy
2
• Subperiosteal bone resorption
• Sacroiliac joint erosions 7
• Rare: Brown tumor (osteoclastoma)
ICLINICAL ISSUES
Presentation
(Left) Anteroposterior radiography shows subperiosteal bone
• Most common signs/symptoms: Bone pain, renal colic resorption at middle phalanges (arrows), and resorption of terminal
Natural History & Prognosis tufts (open arrows). (Right) Lateral radiography shows diffuse,
nonspecific osteopenia. Endplate erosions (arrows) are clue to
• Many cases remain mild, stable diagnosis.
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RENAL OSTEODYSTROPHY
2
8
Sagittal graphic shows thickened, sclerouc vertebral Lateral radiography shows blurring of trabeculae. and
endplates. Trabeculae are scanty and irregular in ill-defined sclerosis adjacent to vertebral endplates in
morphology. young patient on hemodialysis. Note premature
aortoiliac atherosclerosis.
~
. _'.~'. . ' ..
-.,..-- .. ."
., .- • f . .1. ;~ • ' •.
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RENAL OSTEODYSTROPHY
Key Facts
Terminology • Changes of hyperparathyroidism
• Bony changes due to chronic, end-stage renal disease • Changes of osteomalacia/rickets
• Secondary hyperparathyroidism (HPT), osteomalacia, Top Differential Diagnoses
bone sclerosis, aluminum toxity contribute to • Primary hyperparathyroidism
findings •
•
Osteomalacia and rickets
Dialysis arthropathy
2
Imaging Findings
• Osteosclerosis and/or osteopenia • Osteopetrosis 9
• Rugger jersey spine • Paget disease
• Discogenic sclerosis
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HYPERPLASTIC VERTEBRAL MARROW
2
10
Sagittal T7WI MR in a patient with sickle cell anemia Sagittal T2WI MR in same patient shows homogeneous
demonstrates biconcave vertebrae with diffuse marrow marrow hypointensity. compatible with hyperplastic
hypointensity. Intervertebral discs appear hyperintense cellular marrow.
compared to marrow.
• Cortical thinning
ITERMINOLOGY • "Hair-on-end" striations in calvarium
Abbreviations and Synonyms • Biconcave vertebrae in sickle cell anemia
• Marrow reconversion CT Findings
• Hypercellular marrow expansion • Bone CT
Definitions o H-shaped vertebrae due to endplate infarction in
• Physiologic process where fatty marrow is converted to sickle cell anemia
red marrow in response to systemic stress MR Findings
• TlWI
o Intermediate to low signal intensity (SI)
I IMAGING FINDINGS • Where fatty marrow is expected
General Features • Similar to, hypointense, or slightly hyperintenseto
muscle
• Best diagnostic clue: Intervertebral discs hyperintense
• Normal vertebral marrow SI significantly higher
compared to vertebral marrow on TlWI
than muscle
• Location
o Calvarium, spine, ribs, pelvis initially involved • T2WI
o Intermediate to low SI
• Followed by extremities
• Similar to, but not higher than, muscle on
• Size: Entire spinal column typically affected fat-suppressed T2WI
• Morphology • STIR: Similar, but not hyperintense, compared to
o Diffuse, homogeneous
muscle
o Patchy, geographic • Tl C+: Mild post-gadolinium enhancement
Radiographic Findings Nuclear Medicine Findings
• Radiography • Tc-99m diphosphonate or Tc-99m sulfur colloid scan
o Hemolytic anemias
o Sites of increased uptake due to marrow expansion
• Medullary expansion in ribs, calvarium, long
• Distal appendicular skeleton
bones
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• Calvarium
Myeloproliferative disorder
Other Modality Findings • Polycythemia vera, chronic myelogenous leukemia,
• lron-oxide-enhanced MR marrow imaging primary thrombocythemia, myelofibrosis with
o Normal and hypercellular reconverted marrow myeloid metaplasia
accumulate supermagnetic iron oxides • Uncontrolled proliferation of hematopoietic stem cells
• Significant signal loss on STIR 45-60 minutes after • May overlap, evolve into one another, or transform
iron oxide infusion into acute leukemia
o May be useful in distinguishing tumor infiltration • MR appearance overlaps with hyperplastic normal
from marrow reconversion marrow
Imaging Recommendations Marrow edema
• Best imaging tool • Trauma, osteomyelitis, marrow surrounding tumors
o MRI modality of choice in marrow imaging • Hypointensity on TlWI
• High sensitivity in detecting increased marrow o Typically less than muscle SI
cellularity on Tl WI • Focal or multisegmental hyperintensity on STIR
• Protocol advice • Associated cortical disruption
o STIR or T2WI MRI with fat-saturation • Soft tissue and intraspinal hematoma in trauma
• To distinguish hematopoietic elements from • Discitis intraspinal ± phlegmon/abscess in
marrow edema or neoplastic infiltration osteomyelitis
o Pre-, post-gadolinium TlWI with fat suppression
also helpful
I PATHOLOGY
I DIFFERENTIAL DIAGNOSIS General Features
• General path comments
Normal hematopoetic marrow o Circulatory demands exceed available cellular
• Children up to - 10 years marrow
• Recruitment of yellow marrow to produce red
Marrow infiltration/replacement marrow
• Leukemia, lymphoma, multiple myeloma, or o Embryology-anatomy
metastases • At birth, marrow space in entire skeleton occupied
• Diffuse or focal Tl hypointensity with red marrow
o Multifocal disease more easily distinguished from • Conversion of red to yellow marrow occurs in first
hyperplastic normal marrow two decades of life
o Diffuse marrow infiltration may appear very similar • From peripheral and distal to central and
• SI usually great than muscle on STIR or fat suppressed proximal
T2WI • Adult marrow pattern by 25 years of age
• Post-gadolinium enhancement • Red marrow (40% fat, 40% water, 20% protein)
• Increased metabolic activity on PET scan predominantly in axial skeleton, proximal humeri
• Pathologic compression fractures and femora
• Epidural/paravertebral tumor extension
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HYPERPLASTIC VERTEBRAL MARROW
• With age, red marrow decreases from 58% of total
marrow volume in 1st decade to 29% by 8th ICLINICAL ISSUES
decade Presentation
• Amount of fatty marrow (80% fat, 15% water, 5% • Most common signs/symptoms
protein) increases o May not have symptoms referable to spine
• Also to replace bone loss o Other signs/symptoms related to anemia,
o Process of reconversion starts in axial skeleton
2 • Beginning with calvarium, then vertebrae, ribs,
hypoxemia
• Skin and mucous membrane pallor
sternum, and pelvis • Weakness and fatigue
12 • Followed by extremities, from proximal to distal • Dyspnea
• Epiphyses usually spared unless extreme needs • Congestive heart failure
present
• Extensive reconversion in appendicular skeleton Demographics
suggests severe anemia or neoplastic involvement • Age
of axial skeleton o All ages reported
o Adults < 40 yo have better marrow reconversion o More evident in adults> 25 yo
capacity than older adults • Ethnicity
• Etiology o No racial predilection except certain anemias
o Iron deficiency anemia • Sickle cell disease: African-Americans
• Most common cause of anemia • Thalassemia: Races along Eastern Mediterranean
• Chronic blood loss Sea; Greek, Italian, Persian, etc.
• Increased requirements (pregnancy, adolescence
etc.) Natural History & Prognosis
• Inadequate diet • Dependent on underlying etiology causing marrow
o Anemia of chronic disease reconversion
• Second most common cause of anemia Treatment
• Infections (e.g., AIDS), inflammatory disease (e.g.,
rheumatoid arthritis), cancer most common • Treatment of underlying disease
• Interferons, interleukins from monocytes inhibit • Transfusions
erythropoietin production, erythroid proliferation • Recombinant erythropoietin
• Inadequate release of iron from reticulum cells to
plasma
• Increased destruction of red blood cells by
I DIAGNOSTIC CHECKLIST
reticuloendothelial system Consider
o Hemolytic anemia
• Fat-suppressed T2WI (or STIR) & TlWI with
• Accelerated destruction of red blood cells gadolinium when hypointense marrow present on
• Sickle cell disease, thalassemia, etc. TlWI
o Chronic hypoxemia
• Cyanotic heart disorder, heart failure Image Interpretation Pearls
• Heavy smoking: > 1 pack per day • Diffuse marrow T1 hypointensity without marked
• High altitude post-gadolinium enhancement or cortical disruption
• Obesity suggests hyperplastic marrow
• Marathon athletes
o Hematopoietic growth factors
• E.g., granulocyte colony-stimulating factor ISELECTED REFERENCES
• Adjunct in chemotherapy 1. Oaldrup-Link HE et al: Iron-oxide-enhanced MR imaging of
• May simulate marrow tumor progression bone marrow in patients with non-Hodgkin's lymphoma:
• Associated abnormalities differentiation between tumor infiltration and
o Increased marrow cellularity in diaphyses of long hypercellular bone marrow. Em Radiol. 12(6):1557-66,
bones 2002
o Osteonecrosis in sickle cell anemia 2. Plecha OM: Imaging of bone marrow disease in the spine.
Semin Musculoskelet Radiol. 4(3):321-7, 2000
Microscopic Features 3. Bam A et al: MRI gadolinium enhancement of bone
• Capillary proliferation marrow: age-related changes in normals and in diffuse
• Increased sinusoids neoplastic infiltration. Skeletal Radiol. 26(7):414-8, 1997
• Expanded cellular marrow 4. Carroll KW et al: Useful internal standards for
distinguishing infiltrative marrow pathology from
• Anemia of chronic disease hematopoietic marrow at MRI. J Magn Reson Imaging.
o Normal or increased reticuloendothelial iron storage 7(2):394-8, 1997
• Iron deficiency anemia 5. Poulton TB et al: Bone marrow reconversion in adults who
o Decreased reticuloendothelial iron storage are smokers: MR Imaging findings. AJR Am J Roentgenol.
161(6):1217-21, 1993
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HYPERPLASTIC VERTEBRAL MARROW
IIMAGE GALLERY
Typical
(Left) Coronal T7WI MR
through lumbar spine in a
patient with anemia of
chronic disease shows
homogeneously hypointense
2
marrow, similar to muscle. 13
(Right) Sagittal T2WI MR in
the same patient shows
intermediate to low marrow
signal intensity. Degenerative
changes are present at
anterior and superior
endplates of U-5.
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MYELOFIBROSIS
2
14
Sagittal T1WI MR shows uniform low signal intensity Sagittal STIR MR shows marrow signal intensity almost
marrow replacement in patient with primary equal to cortical bone in patient with primary
myelofibrosis (Courtesy L. Steinbach, MOJ. myelofibrosis (Courtesy L. Steinbach, MOJ.
DDx: Myelofibrosis
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MYELOFIBROSIS
Key Facts
Terminology Top Differential Diagnoses
• Histomorphologic pattern of fibrosis in bone marrow • Leukemia and lymphoma
which may be associated with various disorders • Erythropoietic marrow
• Sclerotic metastases
Imaging Findings
• Best diagnostic clue: Very low signal intensity in bone Diagnostic Checklist 2
marrow on all sequences of MRI • Cannot exclude concomitant tumor by MRI; biopsy
needed for diagnosis 15
ICLINICAL ISSUES
Presentation
• Most common signs/symptoms
o Anemia
o Other signs/symptoms
• Splenomegaly due to extramedullary
hema topoiesis
(Left) Sagittal bone CT shows diffusely increased bone density in
Demographics myelofibrosis associated with Hodgkin lymphoma. Individual
• Age: Primary: 50-70 trabeculae are not discernible in dense bone meshwork. (Right)
Sagittal T2WI MR shows decreased bone marrow signal intensity in
Natural History & Prognosis myelofibrosis associated with Hodgkin lymphoma.
• Variable, depending on causative disease entity
Treatment
• Options, risks, complications: Androgens, prednisone,
thalidomide, bone marrow transplants have some
effectiveness
IDIAGNOSTIC CHECKLIST
Image Interpretation Pearls
• Cannot exclude concomitant tumor by MRI; biopsy
needed for diagnosis
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EXTRAMEDULLARY HEMATOPOIESIS
2
16
Sagittal graphic of extramedullary hematopoiesis depicts Coronal T7 C+ MR with fat suppression in patient with
abundant hematopoietic marrow in lumbar vertebrae, beta-thalassemia shows mildly enhancing bilateral
extending into prevertebral and dorsal epidural space. paraspinal masses, proven extramedullary
hematopoiesis at biOpsy.
J'~
Metastasis Lymphoma Phlegmon Hematoma
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EXTRAMEDULLARY HEMATOPOIESIS
Key Facts
Terminology • Diffuse vertebral marrow hypointensity on all
• Epidural ± paravertebral proliferation of sequences
hematopoietic tissue in response to profound chronic Top Differential Diagnoses
anemia • Epidural/paraspinal metastasis
Imaging Findings • Spinal epidural lymphoma 2
• Best diagnostic clue: Minimally enhancing isointense Pathology
thoracic intra- or paraspinal masses with associated 17
• Ectopic hematopoietic rests stimulated in response to
diffuse marrow hypointensity chronic anemic states
• Mid thoracic> cervical, lumbar • Spinal involvement most common after
• Soft tissue density hepatosplenic EMH
• TlWI: Isointense to cord
• Tl C+: Minimal, mild, or moderate Clinical Issues
• Variable mass effect on spinal cord • Cord compression from epidural EMH should be
• Nerve root compression suspected in patients with chronic anemia who
present with neurologic complaints
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EXTRAMEDULLARY HEMATOPOIESIS
Gross Pathologic & Surgical Features IDIAGNOSTIC CHECKLIST
• Discrete flesh colmed mass
Image Interpretation Pearls
Microscopic Features • Spinal EMH should be considered when thoracic
• Resembles bone marrow on biopsy epidural/paraspinal isointense masses are present in
o Trilineage hematopoiesis patients with hemoglobinopathies or
2 • Erythroid and granulocytic precursors
• Megakaryocytes
myeloproliferative disorders
18
ISELECTED REFERENCES
ICLINICAL ISSUES 1. Salehi SA et al: Spinal cord compression in
beta-thalassemia: case report and review of the literature.
Presentation Spinal Cord. 42(2):117-23, 2004
• Most common signs/symptoms 2. Koch CA et al: Nonhepatosplenic extramedullary
o Back ± radicular pain hematopoiesis: associated diseases, pathology, clinical
o Other signs/symptoms course, and treatment. Mayo Clin Proc. 78(10):1223-33,
• Paraparesis 2003
3. Chehal A et al: Hypertransfusion: a successful method of
• Sensory deficit treatment in thalassemia intermedia patients with spinal
• Gait disturbance cord compression secondary to extramedullary
• Bladder, bowel dysfunction hematopoiesis. Spine. 28(13):E245-9, 2003
• Diminished deep tendon reflexes 4. Cario H et al: Treatment with hydroxyurea in thalassemia
• Anemia, pancytopenia intermedia with paravertebral pseudotumors of
• Clinical profile: Cord compression from epidural EMH extramedullary hematopoiesis. Ann Hematol. 81(8):478-82,
should be suspected in patients with chronic anemia 2002
who present with neurologic complaints 5. Chourmouzi D et al: MRI findings of extramedullary
haemopoiesis. Eur Radiol. 11(9):1803-6,2001
Demographics 6. Alorainy IA et al: MRI features of epidural extramedullary
• Age: More common in adults hematopoiesis. Eur J Radiol. 35(1):8-11, 2000
7. Aydingoz U et al: Spinal cord compression due to epidural
• Gender: No gender predilection extramedullary haematopoiesis in thalassaemia: MRI.
• Ethnicity Neuroradiology. 39(12):870-2, 1997
o Some hemoglobinopathies more common in certain 8. Guermazi A et al: Imaging of spinal cord compression due
ethnic groups to thoracic extramedullary haematopoiesis in
• Sickle cell disease: African-Americans myelofibrosis. Neuroradiology. 39(10):733-6, 1997
• Thalassemia: Races along Eastern Mediterranean 9. Dibbern DAJr et al: MR of thoracic cord compression
Sea; Greek, Italian, Persian, etc. caused by epidural extramedullary hematopoiesis in
o Myeloproliferative disorders: No ethnic predilection myelodysplastic syndrome. AJNR Am J Neuroradiol.
18(2):363-6, 1997
Natural History & Prognosis 10. Kalina P et al: Cord compression by extramedullary
hematopoiesis in polycythemia vera. A]R Am J Roentgenol.
• Excellent prognosis: Resolution of symptoms 3-7 days
164(4):1027-8, 1995
after radiotherapy 11. Papavasiliou C: Clinical expressions of the expansion of
o Overall prognosiS limited by underlying the bone marrow in the chronic anemias: the role of
hemoglobinopathy or myeloproliferative disorder radiotherapy. Int J Radiat Oncol Bioi Phys. 28(3):605-12;
1994
Treatment 12. Kalina P et al: MR of extramedullary hematopoiesis causing
• Intravenous steroids cord compression in beta-thalassemia. A]NR Am J
o Decrease cord edema Neuroradiol. 13(5):1407-9, 1992
• Radiation therapy 13. Konstantopoulos K et al: A case of spinal cord compression
o Treatment of choice by extramedullary haemopoiesis in a thalassaemic patient:
a putative role for hydroxyurea? Haematologica.
• Ectopic hematopoietic tissue extremely
77(4):352-4, 1992
radio-sensitive 14. Kaufmann T et al: The role of radiation therapy in the
o Alone or in conjunction with surgery management of hematopoietic neurologic complications
o Risk of marrow suppression in thalassemia. Acta Haematol. 85(3):156-9, 1991
• Decompressive laminectomy with surgical resection 15. Gouliamos A et al: Low back pain due to extramedullary
o Indicated when significant myelopathy present hemopoiesis. Neuroradiology. 33(3):284-5, 1991
• Transfusions 16. Papavasiliou C et al: Masses of myeloadipose tissue:
o When radiation or surgery contraindicated radiological and clinical considerations. Int J Radiat Oncol
o Hematopoietic tissues regress as stress of anemia Bioi Phys. 19(4):985-93, 1990
relieved
o Frequent recurrences
• Hydroxyurea
o Increases production of hemoglobin-F
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EXTRAMEDULLARY HEMATOPOIESIS
I IMAGE GAllERY
Typical
(Left) Axial TlWI MR in a
patient with beta-thalassemia
shows bilateral paraspinal
masses, isointense to cord.
Bilateral rib marrow
2
expansion is evident 19
(arrows). (Right) Axial Tl C+
MR demonstrates moderate
enhancement of bilateral
paraspinal masses in patient
with beta-thalassemia.
Typical
(Left) Axial T2* eRE MR
shows paravertebral masses,
isointense to spinal cord.
Biopsy reveals EMH. (Right)
Axial CECT in patient with
EMH shows bilateral mildly
enhancing paravertebral
masses. A vessel traverses
through left paraspinal mass.
Bilateral pleural effusions are
present.
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TUMORAL CALCINOSIS
2
20
Coronal TlWI MR shows large lobulated masses of low Sagittal TlWI MR shows multiple, well-defined low
signal involving posterior elements at L4, L5, with no signal lesions encompassing posterior elements at L4-5.
associated intermediate or soft tissue component. There is moderate canal stenosis due to tumoral
calcinosis mass effect.
, ~~1'1 "1'.
~
. . I
~\
. ~ I'•
. "<.).I.~~;
'.- . .,
,~ \
-~~:!
Baastrup Synovial Cyst Osteosarcoma Septic Facet
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TUMORAL CALCINOSIS
Key Facts
Terminology Top Differential Diagnoses
• Benign periarticular soft tissue hyperplasia, • Calcium pyrophosphate deposition disease
calcification • Primary bone tumor
• Synovial chondromatosis
Imaging Findings
• Predilection for large joints
•
•
Neuropathic joint
Baastrup disease 2
• Hip • Synovial cyst
• Shoulder 21
• Infection/septic facet
• Elbow
• Spinal involvement uncommon Pathology
• Calcific mass with clustered calcific aggregates • Most cases spontaneous and sporadic
surrounding joint • Autosomal recessive and dominant patterns
• Occasional bone remodeling occasionally found
• No evidence of bone destruction • Unknown - several theories
• No significant soft tissue mass which is not calcified
Diagnostic Checklist
• Tumoral calcinosis is diagnosis of exclusion
• T2* GRE: Low signal from expansile dorsal element • "Kissing" spinous processes
lobulated masses • Cystic degeneration of interspinous ligament with
• Tl C+ high signal on T2WI
o Minimal enhancement
o No associated enhancing soft tissue component Synovial cyst
• Well-defined mass arising from ventral margin of facet
Nuclear Medicine Findings joint/ligamentum flavum
• Bone Scan: Markedly increased uptake • Central high signal on T2WI
• Peripheral low signal on T2WI, peripheral
Imaging Recommendations enhancement
• Best imaging tool: CT shows calcific lesions, extent, • Commonly associated with joint effusion
presence or absence of bone destruction
• Protocol advice: Axial thin section CT, multiplanar Infection/septic facet
reformats • Abnormal low signal from facet, with irregular
adjacent soft tissue extension
• Joint effusion
IDIFFERENTIAL DIAGNOSIS • Bone destruction
• Peripheral enhancement of epidural abscess
Calcium pyrophosphate deposition disease
• Calcification involving ligamentum flavum
• May compress dorsal thecal sac I PATHOLOGY
• Confined to ligamentum flavum
• No facet joint involvement General Features
• General path comments
Primary bone tumor o Calcific masses about large joints, with spine
• Ill-defined margins, infiltrative involvement uncommon
• Large soft tissue component separate from calcific o Need to exclude other causes of soft tissue
mass calcification
• Enhancement of soft tissue component • Collagen vascular disease, hypervitaminosis D,
• Bone destruction chronic renal disease
Synovial chondromatosis • Genetics
• Usually affects larger joints as does tumoral calcinosis o Most cases spontaneous and sporadic
o Autosomal recessive and dominant patterns
• Monoarticular involvement, unknown cause
occasionally found
• Middle aged patients, M < F
o Cases of familial hyperphosphatemia with tumoral
• Progressive periarticular swelling and pain
• Multiple intra-articular densities on CT, plain film calcinosis
o Some overlap of clinical features with
Neuropathic joint pseudoxanthoma elasticum (PXE)
• Bone destruction, as well as bone proliferation • PXE shows degenerated dermal elastic fibers
• PXE shows vasculopathy, and there are rare cases
Baastrup disease of peripheral aneurysm formation in tumoral
• Degeneration of interspinous ligament, ligamentum calcinosis
flavum • Etiology
• Thickening, buckling of dorsal spinal ligaments
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TUMORAL CALCINOSIS
o Unknown - several theories o Benign nature
• Inborn error of phosphorus metabolism o Potential for confusion with malignant lesions such
• "Traumatic theory": Microtrauma of connective as sarcoma
tissues around the joints, bleeding + formation of • No enhancement
rudimentary bursae initial event
• Variation of calcium pyrophosphate deposition
disease ISELECTED REFERENCES
2 o Laboratory values generally normal 1. Martinez S: Tumoral calcinosis: 12 years later. Semin
• Elevated renal phosphate absorption Musculoskelet Radiol. 6(4): 331-39, 2002
22 • Elevated serum I, 25-dihydroxy-vitamin D levels 2. Blay P et al: Vertebral involvement in hyperphosphatemic
• Epidemiology: 2nd to 3rd decades of life tumoral calcinosis. Bone. 28(3): 316-8, 2001
• Associated abnormalities 3. Durant DM et al: Tumoral calcinosis of the spine: a study
o Chronic renal failure of 21 cases. Spine. 26(15): 1673-9, 2001
o Hyperphosphatemia occasionally 4. Matsukado K et al: Tumoral calcinosis in the upper cervical
spine causing progressive radiculomyelopathy--case report.
o CPPD
Neurol Med Chir (Tokyo). 41(8): 411-4, 2001
Gross Pathologic & Surgical Features 5. Watanabe A et al: Tumoral calcinosis of the lumbar
meninges: case report. Neurosurgery. 47(1): 230-2, 2000
• Calcific mass, with solid & "milk of calcium" 6. Reginato AJ et al: Familial and clinical aspects of calcium
components pyrophosphate deposition disease. Curr Rheumatol Rep.
1(2): 112-20, 1999
Microscopic Features 7. Adams WM et al: Familial tumoral calcinosis: association
• Hydroxyapatite + calcium pyrophosphate dihydrate with cerebral and peripheral aneurysm formation.
crystals identified Neuroradiology. 41(5):351-5, 1999
• Chronic inflammatory cells, multinucleated giant 8. Kokubun S et al: Tumoral calcinosis in the upper cervical
cells, granulation tissue spine: a case report. Spine. 21(2): 249-52, 1996
9. Ohashi K et al: Idiopathic tumoral calcinosis involVing the
cervical spine. Skeletal Radiol. 25(4): 388-90, 1996
10. Geirnaerdt MJ et al: Tumoral calcinosis. Skeletal Radiol.
ICLINICAL ISSUES 24(2):148-51, 1995
11. Yamaguchi T et al: Successful treatment of
Presentation hyperphosphatemic tumoral calcinosis with long-term
• Most common signs/symptoms acetazolamide. Bone. 16(4 Suppl):247S-250S, 1995
o Painless enlargement of joint 12. McGuinness FE: Hyperphosphataemic tumoral calcinosis
o Other signs/symptoms in Bedouin Arabs--clinical and radiological features. Clin
• Usually lesions at other large synovial joints when Radiol. 50(4):259-64, 1995
spinal involvement present 13. McGregor DH et al: Nonfamilial tumoral calcinosis
• Clinical profile: Painless growth about large joint in associated with chronic renal failure and secondary
hyperparathyroidism: report of two cases with
adult
clinicopathological, immunohistochemical, and electron
Demographics microscopic findings. Hum Pathol. 26(6):607-13, 1995
14. Steinbach LS et al: Tumoral calcinosis:
• Age: Adult radiologic-pathologic correlation. Skeletal Radiol.
• Gender: M = F 24(8):573-8, 1995
• Ethnicity: African-American> Caucasian 15. Shaffrey CI et al: Tumoral calcium pyrophosphate
dihydrate deposition disease mimicking a cervical spine
Natural History & Prognosis neoplasm: case report. Neurosurgery. 37(2): 335-9, 1995
• Progressive 16. Noyez JF et al: Tumoral calcinosis, a clinical report of
• Multiple locations may be involved eleven cases. Acta Orthop Belg. 59(3):249-54, 1993
17. Calloway DM et al: Combined modality treatment for
Treatment tumoral calcinosis. Orthop Rev. 22(3):365-9, 1993
• Options, risks, complications 18. Tezelman S et al: Tumoral calcinosis. Controversies in the
o Symptomatic treatment etiology and alternatives in the treatment. Arch Surg.
o Wide surgical excision 128(7):737-44; discussion 744-5, 1993
19. Peller PJ et al: Extraosseous Tc-99m MDP uptake: a
o Recurrence common
pathophysiologic approach. Radiographies. 13(4):715-34,
o Evaluate for underlying metabolic abnormality 1993
o Drug treatment with phosphorus deprivation by oral 20. Slavin RE et al: Familial tumoral calcinosis. A clinical,
aluminum hydroxide, or acetazolamide histopathologic, and ultrastructural study with an analysis
of its calcifying process and pathogenesis. Am J Surg
Pathol. 17(8):788-802, 1993
IDIAGNOSTIC CH~CKLIST 21. Fam AG: Calcium pyrophosphate crystal deposition disease
and other crystal deposition diseases. Curr Opin
Consider Rheumatol. 4(4):574-82, 1992
• Tumoral calcinosis is diagnosis of exclusion
• Look for lesions in other large joints by CT
Image Interpretation Pearls
• Recognition of this entity important
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TUMORAL CALCINOSIS
I IMAGE GALLERY
Typical
(Left) Axial NEeT shows
lobulated masses of high
attenuation centered around
left L5-S 1 facet joint,
extending into dorsal
2
epidural space (arrows). 23
(Right) Axial T1WI MR in the
same patient shows
extensive lobulated low
signal surrounding the left
L5-S 1 facet joint. There is
mass effect upon left side of
the thecal sac (arrow).
(Left) Sagittal T1 WI M R
'~-.s( shows lobulated low signal
mass involving posterior
elements from L4-5 to L5-S1
. t, ", .\" level, with well-defined
.
margin. Lesion expansion
.t l
of low signal from tumoral
mass of posterior elements at
..~~
L4-5, and L5-S1.
Variant
(Left) Sagittal T1WI MR
shows well-defined low
signal mass involving
ligamentum flavum, with
severe compression of dorsal
thecal sac at L4-5 (arrow).
(Right) Axial T2WI MR in the
same patient shows the low
signal mass in the midline of
the dorsal elements, effacing
the dorsal thecal sac. There
are bilateral facet effusions.
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Coronal graphic demonstrates right brachial plexus in Coronal STIR MR depicts normal mildly hyperintense
relation to vertebra, vertebral and subclavian arteries. brachial plexus roots/rami (arrows). C5 & C6 form the
C5, C6, & C7 rami exit above same-numbered vertebra, superior (upper) trunk, C7 the middle trunk, and CB &
CB & T7 below. T7 the inferior (lower) trunk.
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NORMAL PLEXUS AND NERVE ANATOMY
DIFFERENTIAL DIAGNOSIS 1
Normal nerve/plexus HMSN 3
• ]\Jorrnal nerve SiZl" course, contour, internal • Charcot-Marie-Tooth, Dejerine-Sottas
architectulT
Inflammatory disease
Nerve/plexus mass • Infectious neuritis (usual Iv viral)
• Metastasis (systemic or local extension) • Tabes dorsalis (Syphilis)
• Nerve sheath tumor • l.eprosy
• l"eurolymphomatosis • Sa rcoidosis
• Guillain-Barre syndrome
Trauma • ClDP
• Stretch/avulsion injury (traction) • Idiopathic (Parsonage-Turner syndrome)
• l.aceration (fracture fragment, projectile)
• Direct compression (hematoma, fracture) Vascular insult
• Ischemic neuropathy (peripheral vascular disease,
Entrapment syndrome vascular trauma)
• RetinaculaI' or muscular compression at specific • Vasculitis (diabetic, Churg-Strauss, polyarteritis
locations nodosa, Wegener granulomatosis)
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NORMAL PLEXUS AND NERVE ANATOMY
1 '.'J'.'.','-~-U.
'~I>"
4
JI~:l.: .,.
.' Ill/A'~
~~.
~
~ ..~-af'
'.~'"
.'"
'!'i~'"~T\.
'':.
'. .• ' ...
.~-
'. 'I .
t
1t ,\"
, f." .. -
~
--'" ~ .
. ~' ~. ~
.' .
I· ' /
J :
Coronal STIR MR demonstrates normal lower (C7, CS, Sagittal oblique STIR MR shows normal anatomy &
T!) brachial plexus roots/rami (arrows) sequentially internal fasicular architecture of superior, middle, and
forming trunks, divisions, and cords. Normal brachial inferior trunks (arrows). Normal fascicles are regular and
plexus courses retroclavicular into the axilla. slightly hyperintense (STIR, fat-saturated T2WI).
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NORMAL PLEXUS AND NERVE ANATOMY
I IMAGE GALLERY
1
5
Typical
(Left) Coronal graphic
reveals normal anatomy of
the bilateral lumbar and
sacral plexi. The lumbosacral
trunk (LST) crosses the sacral
ala and joins the 57-53
ventral rami to form the
sacral plexus. (Right) Sagittal
graphic shows the
lumbosacral trunk and sacral
rami coalescing into the
sacral plexus along the
ventral piriformis muscle
surface.
Typical
(Left) Coronal fat-saturated
T2WI MR reveals normal
longitudinal sacral plexus &
sciatic nerve anatomy
(arrows) as they course
through the greater sciatic
foramen (CSF) into the
thigh. (Right) 3D graphic
depicts normal peripheral
nerve anatomy. The nerve is
surrounded by epineurium.
The perineurium surrounds
the fascicles, each of which
contains many endoneurium
wrapped axons.
Typical
(Left) Sagittal TlWI MR
oblique shows normal sciatic
nerve internal architecture
(arrows) at piriformis muscle
(open arrow). Note
isointense fascicles within
hyperintense fibrofatty
connective tissue. (Right)
Sagittal oblique fat-saturated
T2WI MR shows normal
sciatic nerve (arrows) at
piriformis muscle (open
arrow). Mildly hyperintense
fascicles interspersed with
hypointense fibrofatty
connective tissue.
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Anteroposterior radiography of chest shows right apical Coronal TlWI MR shows large left apical soft tissue
lung mass, with rib destruction from bronchogenic mass involving left first rib, left side of upper thoracic
carcinoma. vertebral bodies with epidural extension.
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SUPERIOR SULCUS TUMOR
Key Facts
1
Terminology • CT shows mediastinal nodes, pulmonary, hepatic or 7
• Synonym: Pancoast tumor adrenal metastases
• Benign or malignant tumor extending to superior Top Differential Diagnoses
thoracic inlet with severe shoulder/arm pain along • Metastatic disease
C8, TI, T2 nerve trunks, Horner syndrome, weakness
• Other thoracic tumors besides NSCLC
+ atrophy of intrinsic hand muscles (Pancoast • Hematologic neoplasms
syndrome) • Infections
• Non-small cell lung carcinoma (NSCLC) most • Radiation fibrosis
frequent cause
Clinical Issues
Imaging Findings • Median survival with pre-operative radiation and
• Mediastinoscopy performed regardless of PET positive resection 22 months, with 5 year survival 27%
nodes since N2, 3 node involvement is negative • Classic approach is preoperative radiotherapy
prognostic factor followed by surgical resection
• MR demonstrates apical soft tissue involvement & • Pain palliation with radiation alone successful in 75%
relationship to brachial plexus/subclavian vessels
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ICLINICAL ISSUES
Presentation
ISELECTED REFERENCES
1. Detterbeck FC et al: Lung cancer. Special treatment issues.
• Most common signs/symptoms
Chest. 123(1 Suppl):244S-258S, 2003
o Unrelenting pain in shoulder/arm in C8-T2 2. Kichari JR et al: MR imaging of the brachial plexus: current
distribution imaging sequences, normal findings, and findings in a
• Pain in upper chest wall with first intercostal spectrum of focal lesions with MR-pathologic correlation.
nerve involvement CUff Probl Diagn Radiol. 32(2):88-101, 2003
o Other signs/symptoms 3. Detterbeck FC: Changes in the treatment of Pancoast
• Horner syndrome (ptOSiS, anhidrosis, miosis) tumors. Ann Thorac Surg. 75(6):1990-7, 2003
• Atrophy of hand muscles 4. Dartevelle P et al: Surgical management of superior sulcus
o Tumors are peripheral in lung location tumors. Oncologist. 4(5):398-407, 1999
5. Ducic Y et al: A logical approach to the thoracic inlet: the
• Pulmonary symptoms such as cough, hemoptysis, Dartevelle approach revisited. Head Neck. 21(8):767-71,
dyspnea are uncommon early in disease course 1999
6. Jones DR et al: Pancoast tumors of the lung. CUff Opin
Demographics Pulm Med. 4(4):191-7, 1998
• Age: Adult 7. Kuhlman JE et al: CT and MR imaging evaluation of chest
• Gender wall disorders. Radiographies. 14(3):571-95, 1994
o M>F
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Typical
(Left) Coronal PET (FOG) CT
fusion shows intense glucose
uptake involving the right
upper lobe mass, extending
beyond the chest wall
(arrows), as well as
metastatic right hi/ar
lymphadenopathy (open
arrow). (Right) Sagittal TI WI
MR shows extension of the
upper lobe bronchogenic
carcinoma through chest
wall involving upper ribs
(open arrow) posterior to
subclavian vessels (arrow).
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., •
.. -:
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MUSCLE DENERVATION, SPINE
Key Facts
1
Terminology Top Differential Diagnoses 11
• Muscle signal/density alteration and volume change • Muscle trauma or disuse atrophy
following denervation • Muscle inflammation or infection
• Radiation myopathy
Imaging Findings
• Best diagnostic clue: Asymmetric muscle volume loss Diagnostic Checklist
with fatty replacement ~ chronic denervation • Acute and chronic denervation changes commonly
• Size: Enlarged 2° muscle edema (acute) or atrophied co-exist on MR imaging, and may persist for
(chronic) considerable time periods
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BRACHIAL PLEXUS TRACTION INJURY
1
12
Coronal graphic demonstrates avulsion of left C5 - C8 Coronal STIR MR reformat shows multiple
nerve roots with avulsion pseudomeningoceles. pseudomeningoceles (arrows) and "nerve retraction
ball" (open arrows). Attenuated, partially avulsed nerve
rami are indicated by curved arrows.
ITERMINOLOGY CT Findings
Abbreviations and Synonyms • NECT
o Difficult CT diagnosis
• Synonyms: Brachial plexus stretch injury, brachial o +/- Pseudomeningocele(s), paraspinal hematoma
plexus avulsion, avulsion pseudomeningocele o Most useful following myelography
Definitions • Bone CT
• Stretch injury or avulsion of ~ 1 cervical roots, o +/- Vertebral fracture(s)
brachial plexus MR Findings
• TlWI
o Enlargement, hypo- to isointense signal of neural
IIMAGING FINDINGS elements (stretch injury)
o Dilated hypointense CSF signal intensity within
General Features
empty thecal diverticulum (avulsion)
• Best diagnostic clue: Lateral CSF-containing dural sac
outpouching(s) devoid of neural elements • T2WI
o Enlargement, hyperintense signal of neural elements
• Location: Brachial plexus, pre- or post-ganglionic
(stretch injury)
nerve roots
o Hyperintense CSF signal intensity within empty
• Morphology thecal diverticulum (avulsion)
o Stretch injury: Enlargement or attenuation of
o +/- Spinal cord edema, myelomalacia, syringomyelia
stretched (but contiguous) plexus elements
• Central spinal cord edema in acute stage 2° root
o Avulsion injury: Attenuated or disrupted proximal
avulsion
roots/rami within or immediately distal to
o Denervation changes in posterior cervical paraspinal
diverticulum +/- retracted distal nerve roots, nerve
muscles (especially multifidus) ~ pre-ganglionic
"retraction ball"
injury
Radiographic Findings • STIR: Similar to fat-saturated T2WI
• Myelography: +/- Dilated empty root sleeves, • Tl C+
CSF/contrast leak, spinal cord "divot" at avulsion site
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BRACHIAL PLEXUS TRACTION INJURY
Key Facts 1
Terminology Pathology 13
• Synonyms: Brachial plexus stretch injury, brachial • Majority of adult brachial plexus palsies are
plexus avulsion, avulsion pseudomeningocele posttraumatic injuries 2° high-energy force
• Stretch injury or avulsion of ~ 1 cervical roots,
brachial plexus Clinical Issues
• Pain, paralysis of ipsilateral limb +/- phrenic nerve
Imaging Findings palsy
• Best diagnostic clue: Lateral CSF-containing dural sac • Clinical examination cannot reliably distinguish
outpouching(s) devoid of neural elements between pre- and post-ganglionic injuries
Top Differential Diagnoses Diagnostic Checklist
• Nerve sheath tumor • MRI may distinguish pre- from post-ganglionic injury
• Lateral meningocele for surgical planning, prognostication
• Nerve root sleeve cyst • Search for soft tissue trauma (edema, hemorrhage) or
• CIDP fracture to support diagnosis
• Muscle denervation pattern predicts abnormal nerves
o Enhancement absent in acute injury; chronic may o Injury level critical to treatment planning, prognosis
show enhancing scar tissue • Pre-ganglionic injury: Lesion central to dorsal root
o Enhancing muscle implies denervation ganglion, worse prognosis
• Post-ganglionic injury: Injury peripheral to
Imaging Recommendations ganglion, better prognosis
• Best imaging tool: High-resolution MR imaging (MR • Etiology
neurography) o Majority of adult brachial plexus palsies are
• Protocol advice posttraumatic injuries 2° high-energy force
o Coronal, sagittal oblique T1WI and STIR MR • Forced abduction or downward displacement of
o CT myelography if MR inconclusive arm, gunshot wound
• Lacerations (knives, glass, automobile metal, chain
saw, or animal bite)
IDIFFERENTIAL DIAGNOSIS o In infants, brachial plexus palsy 2° excessive traction
on plexus during difficult (breech or forceps)
Nerve sheath tumor delivery
• Plexiform neurofibroma (NFl), solitary nerve sheath • Epidemiology
tumor mimics root avulsion(s) o Strong association with off-road vehicle, motorcycle
• + Tumoral enhancement accidents
• Look for clinical, genetic, or imaging stigmata of NFl • Incidence follows trauma epidemiological patterns
Lateral meningocele • Associated abnormalities
• Marfan syndrome, Ehlers-Danlos syndrome, NFl o +/- Co-existing soft tissue or vascular injury,
• Lateral meningocele mimics pseudomeningocele, but vertebral fractures
contains neural elements o +/- Muscle denervation; pattern predicts abnormal
• Clinical history/stigmata +/- dural dysplasia nerve roots
distinguish from trauma o Transdural spinal cord herniation (rare)
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BRACHIAL PLEXUS TRACTION INJURY
1 • Type 2: Dura torn at mouth of foramen, DRG
more or less displaced
o Nerve bypass or graft, reconstruction with
neurolysis, nerve graft, nerve transfer, functioning
14 • Type 3: Dura not torn, DRG not displaced muscle/tendon transfer
• Type 4: Dura not torn, DRG not displaced, either o Amputation of non-functional or painful "flail arm"
ventral or dorsal roots intact o Dorsal root entry zone (DREZ) lesion palliation for
intractable pain
ICLINICAL ISSUES
I DIAGNOSTIC CHECKLIST
Presentation
• Most common signs/symptoms Consider
o Pain, paralysis of ipsilateral limb +/- phrenic nerve • Familiarity with normal brachial plexus anatomy
palsy essential for MR interpretation
• Complete brachial plexus avulsion produces • MRI may distinguish pre- from post-ganglionic injury
useless "flail arm" for surgical planning, prognostication
• Incomplete paralysis common with complete root
avulsion(s) because of redundant muscle Image Interpretation Pearls
innervation from multiple roots • Search for soft tissue trauma (edema, hemorrhage) or
o Clinical examination cannot reliably distinguish fracture to support diagnosis
between pre- and post-ganglionic injuries • Muscle denervation pattern predicts abnormal nerves
• Clinical profile
o Erb-Duchenne palsy
• Upper plexus injury (CS, C6 roots, upper trunk) ~ I SELECTED REFERENCES
proximal muscle weakness 1. Carlstedt T et al: Restoration of hand function and so
• Direct blow to shoulder, birth traction injury called "breathing arm" after intraspinal repair of C5-T1
o Middle radicular syndrome brachial plexus avulsion injury. Case report. Neurosurg
• C7 root, middle trunk ~ radial nerve Focus. 16(5):E7, 2004
abnormalities 2. Haerle M et al: Management of complete obstetric brachial
plexus lesions.] Pediatr Orthop. 24(2):194-200, 2004
o Klumpke palsy 3. Ferraresi S et al: Reinnervation of the biceps in C5-7
• Lower plexus injury (C8, Tl roots, lower trunk) ~ brachial plexus avulsion injuries: results after distal bypass
distal muscle weakness surgery. Neurosurg Focus. 16(5):E6, 2004
• Fall with overhead grasp slowing descent 4. Kim DH et al: Mechanisms of injury in operative brachial
plexus lesions. Neurosurg Focus. 16(5):E2, 2004
Demographics 5. Filler AG et al: MR neurography and muscle MR imaging
• Age: Young to middle-age adult> infants, children for image diagnosis of disorders affecting the peripheral
• Gender: M > F; follows trauma epidemiology nerves and musculature. Neurol CHn. 22(3):643-82, 2004
6. Chen H] et al: Combined dorsal root entry zone lesions
Natural History & Prognosis and neural reconstruction for early rehabilitation of
• Variable; generally poor in avulsion, better with brachial plexus avulsion injury. Acta Neurochir Suppl.
stretch injury + nerve contiguity 87:95-7, 2003
7. Bertelli]A et al: Brachial plexus avulsion injury repairs with
• Post-ganglionic better prognosis than pre-ganglionic
nerve transfers and nerve grafts directly implanted into the
injury spinal cord yield partial recovery of shoulder and elbow
• Obstetrical injury better prognosis than adult stretch movements. Neurosurgery. 52(6):1385-9; discussion
injuries 1389-90, 2003
• Integrity of nerve roots critical for surgical decision 8. DaSilva VR et al: Upper thoracic spinal cord herniation
making, prognostication after traumatic nerve root avulsion. Case report and review
o Stretched (but contiguous) nerves may recover some of the literature.] Neurosurg. 99(3 Suppl):306-9, 2003
function, while avulsed nerve roots produce 9. Gei AF et al: Brachial plexus paresis associated with fetal
irreversible sensory and motor deficits neck compression from forceps. Am] Perinatol.
20(6):289-91, 2003
o Pre-ganglionic avulsions usually not amenable to 10. Doi K et al: Cervical nerve root avulsion in brachial plexus
surgical repair; function of some denervated muscles injuries: magnetic resonance imaging classification and
restored with nerve transfers from accessory nerves, comparison with myelography and computerized
cervical plexus, or intercostal nerves tomography myelography.] Neurosurg. 96(3
o Post-ganglionic avulsions repaired with excision of Suppl):277-84, 2002
damaged segment and nerve autograft between 11. Dubuisson AS et al: Brachial plexus injury: a survey of 100
nerve ends consecutive cases from a single service. Neurosurgery.
51(3):673-82; discussion 682-3, 2002
Treatment 12. Hems TE et al: The role of magnetic resonance imaging in
• Conservative management the management of traction injuries to the adult brachial
plexus.] Hand Surg [Br]. 24(5):550-5,1999
o Physical therapy, rehabilitation
• Surgical management
oRe-anastomosis of avulsed roots to spinal cord not
generally advocated, but new microsurgical
techniques prompting re-review
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BRACHIAL PLEXUS TRACTION INJURY
IIMAGE GALLERY 1
15
Typical
(Left) Coronal T2WI MR
demonstrates multiple left
sided nerve root avulsions
with extradural fluid
displacing cord to right
(Courtesy P Chapman,
MO). (Right) Axial T2WI MR
shows left sided CB nerve
root avulsion
pseudomeningocele with
extradural fluid displacing
cord and thecal sac to the
right (Courtesy P Chapman,
MO)
Typical
(Left) Axial STIR MR shows a
large right CB avulsion
pseudomeningocele (arrow).
No internal neural elements
identified. Normal left ventral
primary ramus indicated
with open arrow for
comparison. (Right) Axial
bone CT following
myelography depicts
contrast surrounding dural
sac and leaking through right
neural foramen (arrow) into
scalene muscles along
brachial plexus.
Variant
(Left) Axial T2WI MR with
fat-saturation shows left
brachial plexus obstetrical
traction injury (arrows) in a
infant fol/owing shoulder
dystocia & difficult vaginal
birth (Courtesy G. Hedlund,
DO). (Right) Axial T2WI MR
shows avulsion
pseudomeningocele of T1
(curved arrow) with marked
enlargement of the C8 (open
arrow) and T1 (arrow)
ventral primary rami
secondary to stretch injury.
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TRAUMATIC NEUROMA
1
16
Coronal STIR MR shows enlargement of sciatic nerve, Coronal T7 C+ MR shows avid contrast-enhancement
with a bulbous mass (arrow) at tip of severed nerve, by traumatic neuroma (arrow) of sciatic nerve. Nerve
representing traumatic neuroma. Note spiculated proximal to focal neuroma shows slight enhancement
margins of mass due to scarring. also.
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TRAUMATIC NEUROMA
Key Facts
1
Terminology Imaging Findings 17
• Amputation neuroma: Subtype of traumatic neuroma • Best diagnostic clue: Mass arising in nerve at stump or
following traumatic or surgical amputation site of nerve injury
• Morton neuroma: Traumatic neuroma between
metatarsal heads Top Differential Diagnoses
• Non-neoplastic nerve growth 2° major or minor • Benign peripheral nerve tumor
trauma . • Malignant peripheral nerve sheath tumor (MPNST)
• Perineural cyst, ganglion cyst
• Soft tissue metastasis or recurrent malignancy
ICLINICAL ISSUES (Left) Axial STIR MR shows traumatic ulnar nerve neuroma (open
arrow) in patient follOWing total elbow arthroplasty (curved arrow).
Presentation Images are diagnostic despite metal artifact. (Right) Axial T7 C+ MR
• Most common signs/symptoms: Severe pain or shows heterogeneous enhancement of traumatic neuroma of sciatic
paresthesias n. in patient status post amputation for osteogenic sarcoma.
Enhancement outlines nerve fascicles.
Treatment
• Steroid injection, physical therapy, resection
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THORACIC OUTLET SYNDROME
1
~
18
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·~.",~"tl,'"'~~ .'~i,'~ t i'. ' '., ',.
:,{~. ,..,
,~
.
'J"j ."',
'~I
-
'
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~
i , * t.
'<':: .' ,"' :--.,........'~"
-- oJ •
.
;0 'Y...
l'
Coronal graphic of thoracic outlet demonstrates brachial Coronal STIR MR in a professional musician (drummer)
plexus compression + subclavian artery aneurysm with symptomatic TOS shows abnormal brachial plexus
arising secondary to cervical rib and anterior scalene T2 hyperintensity at the thoracic outlet.
muscle compression.
Definitions MR Findings
• Neural, venous, and/or arterial compressive syndrome • TlWI: Often normal; abnormal cases ~ brachial
at thoracic outlet (TO) plexus (BP) compression or distortion, abnormal
vascular flow void at TO
• T2WI
IIMAGING FINDINGS o +/- Focal abnormal brachial plexus T2
hyperintensity in TO
General Features o +/- Brachial plexus compression or distortion,
• Best diagnostic clue scalene muscle inflammation or fibrosis, abnormal
o Radiography: Cervical rib in symptomatic patient vascular flow voids within TO
o MRI: Neural or vascular compression within • STIR: Similar to fat-saturated T2WI
interscalene or costocervical tunnels • Tl C+: +/- Focal enhancement at TO
• Location: Interscalene, costoclavicular space, • MRA
retropectoralis minor space (subcoracoid tunnel) o +/- SCA compression, aneurysm on MRA
o Positional occlusion or narrowing of SCA with arm
Radiographic Findings hyperabduction, external rotation
• Radiography • MRV: +/- Subclavian vein compression or thrombosis,
o Cervical radiographs usually normal positional occlusion or narrowing of SCV
• +/- Cervical rib, elongated C7 transverse process
Ultrasonographic Findings
CT Findings • Real Time: Muscular or osseous compression of
• CECT: Usually normal; +/- abnormal soft tissue brachial plexus, SCA, or SCV
process, vascular anomaly within TO • Color Doppler: +/- SCA aneurysm, SCV thrombosis
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THORACIC OUTLET SYNDROME
Key Facts
1
Terminology Pathology 19
• Thoracic outlet syndrome (TOS) • Cervical ribs, abnormal transverse processes, fibrous
• Neural, venous, and/or arterial compressive syndrome bands, scalene compression of TO contents
at thoracic outlet (TO) • Neural injury with fibrosis, axonal loss
Imaging Findings Clinical Issues
• +/- Cervical rib, elongated C7 transverse process • "True" neurological TOS: Intermittent arm pain,
• +/- brachial plexus compression or distortion, scalene numbness, and weakness with hyperabduction,
muscle inflammation or fibrosis, abnormal vascular external rotation
flow voids within TO • Vascular TOS: Paresthesias 2 to ischemia
0
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THORACIC OUTLET SYNDROME
1 Gross Pathologic & Surgical Features ISELECTED REFERENCES
20 • Vascular compression, SCA aneurysm, SCV thrombosis 1. Charon JP et al: Evaluation of MR angiographic technique
may be noted at surgery in the assessment of thoracic outlet syndrome. CUn Radiol.
• Nerves may be swollen and edematous, or atrophic 59(7):588-95, 2004
and fibrotic 2. Samarasam I et al: Surgical management of thoracic outlet
• Cervical ribs, abnormal transverse processes, fibrous syndrome: a lO-year experience. ANZJ Surg. 74(6):450-4,
2004
bands, scalene compression of TO contents
3. Konstantinou DT et al: Klippel-Feil syndrome presenting
Microscopic Features with bilateral thoracic outlet syndrome. Spine.
29(9):E189-92, 2004
• Neural injury with fibrosis, axonal loss 4. Ambrad-Chalela E et al: Recurrent neurogenic thoracic
outlet syndrome. AmJ Surg. 187(4):505-10,2004
5. Yanaka K et al: Diagnosis of vascular compression at the
ICLINICAL ISSUES thoracic outlet using magnetic resonance angiography. Eur
. Neurol. 51(2):122-3, 2004
Presentation 6. Demondion X et al: Thoracic outlet: assessment with MR
• Most common signs/symptoms imaging in asymptomatic and symptomatic populations.
o Paresthesias, numbness in forearm/ hand Radiology. 227(2):461-8, 2003
o Pain in shoulder, proximal upper extremity ~ neck 7. Reid JR et al: Thoracic outlet syndrome with subclavian
aneurysm in a very young child: the complementary value
o Obliteration of brachial, radial pulses with arm of MRA and 3D-CT in diagnosis. Pediatr Radiol. 32(1):22-4,
hyperabduction and elevation 2002
• Clinical profile 8. Athanassiadi K et al: Treatment of thoracic outlet
o "True" neurological TOS: Intermittent arm pain, syndrome: long-term results. WorldJ Surg. 25(5):553-7,
numbness, and weakness with hyperabduction, 2001
external rotation 9. Demondion X et aJ: Thoracic outlet: anatomic correlation
• Lower trunk (C8, Tl) ~ upper, middle trunks with MR imaging. AJR AmJ Roentgenol. 175(2):417-22,
• Nearly always 2° to fibrous band extending from 2000
10. Hagspiel KD et al: Diagnosis of vascular compression at the
cervical rib compressing lower trunk thoracic outlet using gadolinium-enhanced high-resolution
• Hand wasting, concordant EMG findings ultrafast MR angiography in abduction and adduction.
o "Disputed" neurological IDS: No consistent clinical Cardiovasc Intervent Radiol. 23(2):152-4, 2000
or laboratory findings 11. Dymarkowski S et al: Three-dimensional MR angiography
o Vascular TOS: Paresthesias 2° to ischemia in the evaluation of thoracic outlet syndrome. AJR Am J
• More diffuse than neurological TOS Roentgenol. 173(4):1005-8, 1999
• +/- Obliteration of brachial, radial pulses 12. Esposito MD et al: Thoracic outlet syndrome in a thrOWing
athlete diagnosed with MRI and MRA. J Magn Reson
Demographics Imaging. 7(3):598-9, 1997
• Age: Adult> > children; average age at diagnosis 26 yrs 13. Remy-Jardin M et al: Functional anatomy of the thoracic
outlet: evaluation with spiral CT. Radiology. 205: 843 -851,
• Gender: M < F 1997
Natural History & Prognosis 14. Matsumura JS et al: Helical computed tomography of the
normal thoracic outlet. J Vasc Surg. 26: 776-783, 1997
• Symptomatic patients rarely recover spontaneously 15. Longley DG et al: Thoracic outlet syndrome: evaluation of
without treatment the subclavian vessels by color duplex sonography. AJR Am
• Post-operative prognosis of vascular TOS better than J Roentgenol. 158:623-630, 1992
neurological TOS
Treatment
• Initial conservative treatment with physical therapy
• Consider TOS decompression/1st rib resection for
conservative treatment failures
IDIAGNOSTIC CHECKLIST
Consider
• TOS probably end result of diverse symptomatic
etiologies rather than a single disease process
. Image Interpretation Pearls
• SCA aneurysm, SCV thrombosis, brachial plexus
compression with abnormal T2 hyperintensity at TO
strongly suggest TOS
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THORACIC OUTLET SYNDROME
IIMAGE GALLERY 1
21
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IDIOPATHIC BRACHIAL PLEXUS' NEURITIS
1
22
Coronal STIR MR shows diffusely increased signal Sagittal oblique STIR MR shows abnormal signal
intensity in brachial plexus (arrows) due to idiopathic intensity in brachial plexus (arrows) due to idiopathic
brachial neuritis. brachial neuritis.
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IDIOPATHIC BRACHIAL PLEXUS NEURITIS
Key Facts
1
Terminology • Diabetic neuropathy 23
• Immune-mediated neuropathy of brachial plexus • Pancoast tumor
• Myositis
Imaging Findings
• Any muscle innervated by brachial plexus
Pathology
• Most common: Rotator cuff, deltoid, biceps, triceps • Often associated with viral or bacterial infection
• Uncommon: Brachialis, forearm muscles, diaphragm, • Can occur post-vaccination
serratus anterior (long thoracic nerve) • Can also be post-traumatic or post-surgical
• Sometimes bilateral Clinical Issues
Top Differential Diagnoses • Sudden onset of pain, followed by weakness,
• Cervical radiculopathy paresthesias
• Suprascapular nerve entrapment • May have sensory abnormalities only
• Brachial plexus neoplasm Diagnostic Checklist
• Brachial plexus or cervical nerve root avulsion • Abnormal muscle signal often involves more than
• Radiation neuritis/myositis one peripheral nerve distribution
• Quadrilateral space syndrome
• Diffuse, homogeneous high signal within muscle • Mass seen in spinoglenoid or suprascapular notch
bellies
• Tendons normal in appearance Brachial plexus neoplasm
• No evidence of mass • Presents with pain, muscle weakness
• Often affects muscles from two or more different • Mass seen on MRI of brachial plexus
peripheral nerves • Usually metastasis; primary tumor uncommon
• E.g., infraspinatus (suprascapular nerve) + teres Brachial plexus or cervical nerve root
minor (axillary nerve)
• PD/Intermediate: Similar findings to T2WI, but high avulsion
signal in nerves and muscles less pronounced • Severe, high velocity injury
• STIR: Similar findings to T2WI, but high signal in • Loss of nerve continuity, fluid around nerve course
nerves and muscles more prominent seen on MRI
• T1 C+: Mild, diffuse enhancement of nerves and Radiation neuritis/myositis
affected muscles
• Diffusely enlarged nerves showing increased T2 signal
Other Modality Findings • Diffusely enlarged muscles showing increased T2
• Nerve conduction studies show nerve abnormalities signal
• Geographic distribution
Imaging Recommendations • History of radiation therapy
• Best imaging tool: MR imaging
• Protocol advice Quadrilateral space syndrome
o T1WI, T2WI or STIR along long and short axes of • Mass between teres minor and teres major muscles
symptomatic muscles • Compresses axillary nerve, affects deltoid, teres minor
o MRI of shoulder should have 16-18 cm FOV on muscles
coronal images
Diabetic neuropathy
• Include spinoglenoid notch, quadrilateral space to
exclude mass in these regions • Usually distal, symmetric
o Imaging of brachial plexus excludes brachial plexus • Sometimes involves one or more proximal muscles
mass Pancoast tumor
• Not needed if patient gives characteristic history, • Bronchogenic carcinoma at lung apex ("superior
unless symptoms do not resolve sulcus")
• May directly extend to involve brachial plexus
IDIFFERENTIAL DIAGNOSIS Myositis
• Diffuse T2 signal abnormalities in muscle
Cervical radiculopathy • Edema in surrounding fat
• Evaluate with cervical spine MRI
• Most commonly see involvement of single nerve root Muscle injury
• Brachial neuritis involves multiple nerve roots • Heterogeneous signal abnormalities in muscle belly
• Severe cases may have denervation changes in affected • Blood products often evident
muscles • Surrounding soft tissues usually show
edema/hematoma
Suprascapular nerve entrapment
• Affects infraspinatus +/- supraspinatus muscles
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IDIOPATHIC BRACHIAL PLEXUS NEURITIS
1 Rotator cuff tear
• Brachial plexus MRI to exclude other causes in atypical
cases
24 • Fluid in tendon defect on MRI o Muscles outside of shoulder girdle
• Chronic, large tears show muscle atrophy o Respiratory compromise
Burner/stinger syndrome o Pure sensory loss
• Common athletic injury in contact sports Image Interpretation Pearls
• Pain, paresthesias, muscle weakness • Abnormal muscle signal often involves more than one
• Direct blow to brachial plexus in supraclavicular peripheral nerve distribution
region • Distribution of signal abnormalities helps to
• Alternatively caused by traction to brachial plexus or distinguish from nerve entrapment syndromes
cervical nerve root
• Usually transient but may persist for weeks
• MRI may be performed to exclude nerve avulsion ISELECTED REFERENCES
1. Safran MR: Nerve injury about the shoulder in athletes,
part 2: long thoracic nerve, spinal accessory nerve,
IPATHOLOGY burners/stingers, thoracic outlet syndrome. Am J Sports
Med. 32(4):1063-76, 2004
General Features 2. Safran MR: Nerve injury about the shoulder in athletes,
• Etiology part 1: suprascapular nerve and axillary nerve. Am J Sports
o Often associated with viral or bacterial infection Med. 32(3):803-19, 2004
• EBY, CMY, HIV among viral infections 3. Gourie-Devi M et al: Long-term follow-up of 44 patients
o Can occur post-vaccination with brachial monomelic amyotrophy. Acta Neurol Scand.
o Can also be post-traumatic or post-surgical 107(3):215-20, 2003
• Surgery may not be in same region (e.g., hip 4. Janes SE et al: Brachial neuritis folloWing infection with
Epstein-Barr virus. Eur J Paediatr Neurol. 7(6):413-5, 2003
arthroplasty has been reported as cause) 5. Cruz-Martinez A et al: Neuralgic amyotrophy: variable
• Epidemiology: M > F expression in 40 patients. J Peripher Nerv Syst.
7(3):198-204,2002
Gross Pathologic & Surgical Features 6. Gonzalez-Alegre P et al: Idiopathic brachial neuritis. Iowa
• Perineural edema, edema in affected muscles Orthop J. 22:81-5, 2002
7. Carroll KW et al: Magnetic resonance imaging of the
Microscopic Features shoulder: a review of potential sources of diagnostic errors.
• Perineural inflammatory infiltrate Skeletal Radiol. 31(7):373-83, 2002
• Thinning of myelin sheath 8. Watson BV et al: Isolated brachialis wasting: an unusual
• Axonal degeneration presentation of neuralgic amyotrophy. Muscle Nerve.
24(12):1699-702,2001
9. Simon JP et al: Parsonage-Turner syndrome after total-hip
arthroplasty. J Arthroplasty. 16(4):518-20,2001
ICLINICAL ISSUES 10. Antoniou J et al: Suprascapular neuropathy. Variability in
Presentation the diagnosis, treatment, and outcome. Clin Orthop.
(386):131-8,2001
• Most common signs/symptoms 11. Miller JD et al: Acute brachial plexus neuritis: an
o Sudden onset of pain, followed by weakness, uncommon cause of shoulder pain. Am Fam Physician.
paresthesias 62(9):2067-72, 2000
o May have sensory abnormalities only 12. Bredella MA et al: Denervation syndromes of the shoulder
o Long thoracic nerve involvement: Winging of girdle: MR imaging with electrophysiologic correlation.
scapula Skeletal Radiol. 28(10):567-72, 1999
o Phrenic nerve involvement: Shortness of breath 13. Helms CA et al: Acute brachial neuritis (Parsonage-Turner
syndrome): MR imaging appearance--report of three cases.
Demographics Radiology. 207(1):255-9, 1998
• Age: May occur at any age
• Gender: M > F
Natural History & Prognosis
• Most cases resolve in 3 months to 2 years
Treatment
• Options, risks, complications: Physical therapy to
preserve range of motion
IDIAGNOSTIC CHECKLIST
Consider
• Shoulder MRI in patient with shoulder weakness and
sensory loss, normal cervical spine MRI
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IDIOPATHIC BRACHIAL PLEXUS NEURITIS
IIMAGE GALLERY 1
25
Typical
(Left) Sagittal T2WI MR
shows diffusely abnormal
signal intensity in the .
infraspinatus and
supraspinatus muscles due to
idiopathic brachial neuritis.
(Right) Axial
PO/Intermediate MR shows
mild increase in signal
intensity throughout
infraspinatus (arrows).
Deltoid is slightly higher
signal than subscapularis due
to signal drop-off from
surface coil.
Typical
(Left) Coronal T1WI MR
shows uniform, smooth
enlargement of visualized
portions of right brachial
plexus (arrow) in patient
with brachial neuritis,
compared to normal left side
(open arrow). (Right)
Coronal STIR MR shows
uniform, tubular nerve
enlargement (arrow) and
increased signal intensity
which is primarily at the
periphery of the nerves.
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RADIATION PLEXOPATHY
1
26
Coronal graphic shows diffuse edema of brachial plexus Coronal STIR MR (breast cancer post-XRT with vague
elements in breast cancer patient with skeletal symptoms) shows mild diffuse brachial plexus T2
metastases following radiation therapy. hyperintensity without focal nodular involvement.
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RADIATION PLEXOPATHY
Key Facts
1
Terminology Pathology 27
• Synonyms: Radiation plexitis, XRT plexitis, • Connective tissue response spectrum from edema,
radiation-induced fibrosis (RIF) acute inflammation ~ chronic inflammation,
• Post-radiation brachial plexus (BP) or lumbosacral progressive fibrosis, & neovascularization, disruption
plexus inflammation of vascular supply to nerve
Imaging Findings Clinical Issues
• Best diagnostic clue: Smooth, diffuse T2 • Pain, paresthesia, motor deficits of ipsilateral
hyperintensity +/- enhancement of multiple plexus extremity
elements
Diagnostic Checklist
Top Differential Diagnoses • Important to correctly diagnosis to avoid
• Malignant brachial plexus infiltration inappropriate additional radiation therapy
• Plexiform neurofibroma • May be difficult to distinguish XRT plexitis from
• Plexus traction injury recurrent tumor on imaging studies
• Smooth T2 hyperintensity, linear enhancement of
upper BP favors XRT plexitis > malignancy
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RADIATION PLEXOPATHY
1 ICLlNICALISSUES ISELECTED REFERENCES
28 1. Bajrovic A et al: Is there a life-long risk of brachial
Presentation plexopathy after radiotherapy of supraclavicular lymph
• Most common signs/symptoms nodes in breast cancer patients? Radiother Oncol.
o Pain, paresthesia, motor deficits of ipsilateral 71(3):297-301,2004
extremity 2. Schierle C et al: Radiation-induced brachial plexopathy:
• BP plexopathy usually referable to upper (CS-7) > review. Complication without a cure. ] Reconstr Microsurg.
lower BP (C8-Tl) 20(2): 149-52, 2004
• Lumbosacral plexopathy most commonly affects 3. Lu L et al: Diagnosis and operative treatment of
radiation-induced brachial plexopathy. Chin] Traumatol.
sciatic nerve distribution
5(6):329-32, 2002
• Pain often relatively mild compared to malignant 4. Ferrante MA et al: Electrodiagnostic approach to the
plexopathy patient with suspected brachial plexopathy. Neurol Clin.
o Other signs/symptoms 20(2):423-50, 2002
• Horner syndrome (rare; more common with 5. Johansson S et al: Dose response and latency for
malignant plexopathy) radiation-induced fibrosis, edema, and neuropathy in
• Clinical profile breast cancer patients. Int] Radiat Oncol Bioi Phys.
o Unilateral BP plexopathy following radiotherapy for 52(5):1207-19,2002
6. Fathers E et al: Radiation-induced brachial plexopathy in
breast cancer, Pancoast tumor, systemic metastasis
women treated for carcinoma of the breast. Clin Rehabil.
o Bilateral brachial plexopathy after mantle 16(2):160-5, 2002
radiotherapy for Hodgkin disease 7. Rubin DI et al: Arteritis and brachial plexus neuropathy as
o Lumbosacral plexopathy following radiotherapy for delayed complications of radiation therapy. Mayo Clin
prostate, cervical cancer Proc. 76(8):849-52,2001
8. Cros D: Peripheral Neuropathy. First ed, Philadelphia:
Demographics Lippincott Williams & Wilkins. 185-7,2001
• Age: Age at onset parallels demographics of initial 9. Johansson S et al: Timescale of evolution of late radiation
cancer diagnosis + development time for radiation injury after postoperative radiotherapy of breast cancer
plexopathy patients. Int] Radiat Oncol Bioi Phys. 48(3):745-50, 2000
• Gender: M < F (reflects pertinent cancer 10. Wittenberg KH et al: MR imaging of nontraumatic brachial
plexopathies: frequency and spectrum of findings.
demographics) Radiographies. 20(4):1023-32, 2000
Natural History & Prognosis 11. Johansson S et al: Brachial plexopathy after postoperative
radiotherapy of breast cancer patients--a long-term
• Variable; related to dose delivered to plexus, severity of follow-up. Acta Oncol. 39(3):373-82, 2000
nerve injury 12. Churn M et al: Early onset of bilateral brachial plexopathy
o Some patients resolve spontaneously during mantle radiotherapy for Hodgkin's disease. Clin
o Others slowly progress despite treatment Oncol (R Coil Radiol). 12(5):289-91,2000
• Risk of brachial plexopathy remains for considerable 13. Qayyum A et al: Symptomatic brachial plexopathy
portion of patient's life following treatment for breast cancer: utility of MR
• All patients have potential to develop delayed imaging with surface-coil techniques. Radiology.
214(3):837-42,2000
symptoms, worsening of grade 14. Wadd N] et al: Brachial plexus neuropathy following
Treatment mantle radiotherapy. Clin Oncol (R Coil Radiol).
10(6):399-400, 1998
• Avoid inappropriate additional radiation therapy 15. Wouter van Es H et al: Radiation-induced brachial
• Corticosteroids +/- combined pentoxifylline and plexopathy: MR imaging. Skeletal Radiol. 26(5):284-8, 1997
tocopherol (Vit E) therapy 16. Bowen BC et al: Radiation-induced brachial plexopathy:
• No traditional role for surgery MR and clinical findings. A]NR Am] Neuroradiol.
o Transferred myocutaneous flap + BP neurolysis may 17(10):1932-6, 1996
improve blood supply to fibrotic brachial plexus 17. Iyer RB et al: MR imaging of the treated brachial plexus.
• Palliative long-term narcotics, sympathectomy for A]R Am] Roentgenol. 167(1):225-9, 1996
pain relief
IDIAGNOSTIC CHECKLIST
Consider
• Important to correctly diagnosis to avoid
inappropriate additional radiation therapy
Image Interpretation Pearls
• May be difficult to distinguish XRT plexitis from
recurrent tumor on imaging studies
• Smooth T2 hyperintensity, linear enhancement of
upper BP favors XRT plexitis > malignancy
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RADIATION PLEXOPATHY
IIMAGE GAllERY 1
29
Typical
(Left) STIR MR Sagittal
oblique (radiation therapy
for breast cancer, acute
symptoms) depicts diffuse
enlargement, fascicular
swelling, abnormal T2
hyperintensity of three BP
cords (arrows). (Right) T7
C+ MR Sagittal oblique
(radiation therapy for breast
cancer, acute symptoms)
demonstrates diffuse
enlargement, mild
enhancement of abnormal
BP cords (arrows).
Typical
(Left) Coronal STIR MR
(radiation therapy for breast
cancer, acute symptoms)
shows diffuse linear T2
hyperintensity of upper
brachial plexus (arrows).
(Right) Coronal T7 C+ MR
(radiation therapy for breast
cancer, acute symptoms)
reveals patchy mild
enhancement of upper
brachial plexus elements.
Typical
(Left) Coronal STIR MR
(radiation therapy for
cervical cancer, chronic
symptoms) shows mild left
lumbosacral plexus T2
hyperintensity (arrows)
surrounded by hyperintense
fibrovascular scar tissue.
(Right) Coronal T7 C+ MR
(radiation therapy for
cervical cancer, chronic
symptoms) shows mild
surrounding fibrovascular
scar tissue enhancement of
lumbosacral plexus .
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HYPERTROPHIC NEUROPATHY
1
30
Axial T1 WI M R (focal hypertrophic neuropathy, path Axial STIR MR (focal HN) confirms mild T2
proven) demonstrates focal enlargement of Ubial hyperintensity, paroal loss of normal fascicular
division (arrow) of sciaUc nerve. Histology revealed architecture (arrow). Fascicles adjacent to mass are
typical "onion-bulb" formaUons. abnormally enlarged and T2 hyperintense.
ITERMINOlOGY CT Findings
• CECT: Enlarged nerves +/- enhancement
Abbreviations and Synonyms
• Synonyms: Hereditary motor-sensory neuropathy MR Findings
(HMSN), hypertrophic neuropathy (HN), • T1WI
Charcot~Marie-Tooth (CMT) syndrome o Fusiform Tl hypointense mass(es) of peripheral
nerves +/- cauda equina nerve roots
Definitions o +/- Abnormal muscle Tl hyperintensity, volume loss
• Hereditary disorder characterized by focal or diffuse (chronic denervation ~ fatty atrophy)
peripheral nerve enlargement
• T2WI
o HMSN I (Charcot-Marie-Tooth syndrome type I, o Fusiform Tl hyperintense mass(es) of peripheral
CMT 1) nerves +/- cauda equina nerve roots
o HMSN II (neuronal type peroneal muscular atrophy, o Internal fascicular architecture disrupted over
CMT2) abnormal segment
o HMSN III (Dejerine-Sottas disease (DSD), o +/- Abnormal muscle hyperintensity, swelling on
hypertrophic neuropathy of infancy, congenital fat-saturated T2WI (acute denervation)
hypomyelinated neuropathy) • STIR: Similar to fat saturated T2WI
• Tl C+: +/- Abnormal enhancement of enlarged nerves
(most commonly HMSN I, III)
IIMAGING FINDINGS
Other Modality Findings
General Features • Nerve conduction velocities (NCVs)
• Best diagnostic clue: Focal or diffuse peripheral nerve o HMSN I: Severe diffuse motor NCV slOWing
enlargement + distal extremity atrophy o HMSN II: Relatively normal motor NCV,
• Location: Peripheral nerves +/- spinal nerve roots reduced/absent sensory nerve action potentials
Radiographic Findings o HMSN Ill: Markedly reduced motor NCV
• Myelography: +/- Nerve root thickening, myelographic
0
block 2 spinal stenosis
" ..
'_.~:,. ~
Guillain-Barre
~.~. Plexiform BP Traction Injury
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HYPERTROPHIC NEUROPATHY
Key Facts
1
Terminology • Nerve sheath tumor 31
• Synonyms: Hereditary motor-sensory neuropathy • Traumatic nerve stretch injury
(HMSN), hypertrophic neuropathy (HN), Pathology
Charcot-Marie-Tooth (CMT) syndrome • Enlargement of peripheral nerves +/- spinal nerve
• Hereditary disorder characterized by focal or diffuse roots, "onion-bulb" formations common in HMSN I,
peripheral nerve enlargement III but uncommon in HMSN II
Imaging Findings • Histologic features of demyelination, remyelination
• Best diagnostic clue: Focal or diffuse peripheral nerve Clinical Issues
enlargement + distal extremity atrophy • Similarly affected relatives common
• Internal fascicular architecture disrupted over • Normal life expectancy
abnormal segment
Diagnostic Checklist
Top Differential Diagnoses • Consider HMSN when abnormally enlarged
• Guillain-Barre syndrome peripheral nerves or nerve roots identified on MR
• Chronic inflammatory demyelinating imaging
polyneuropathy (CIDP)
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HYPERTROPHIC NEUROPATHY
1 o HMSN II: Primary axonal pathology, axon loss +
Wallerian degeneration Natural History & Prognosis
32 o HMSN III: Severe hypomyelination/demyelination, • Normal life expectancy
axonal loss, myelin sheath thinning • Slowly progressive condition; disability severity highly
variable
Staging, Grading or Classification Criteria
• HMSN types lA, IE (CMT lA, lB) Treatment
• HMSN type II (neuronal type peroneal muscular • Conservative: Injury prevention (lifestyle adjustment,
atrophy) avoidance of occupational injury), treatment of
• HMSN type III (hypertrophic neuropathy of infancy, acqUired neuropathies, physical therapy, ankle-foot
Dejerine-Sottas disease) orthoses, anti-inflammatory medications
• HMSN type 4 (hypertrophic neuropathy associated • Surgical: Correction of orthopedic deformities
with phytanic acid excess)
• HMSN type 5 (associated with spastic paraplegia)
• HMSN type 6 (associated with optic atrophy) IDIAGNOSTIC CHECKLIST
• HMSN type 7 Consider
• MR imaging currently plays a limited role in the
ICLINICAL ISSUES work-up of HMSN
• Genetic consultation for patients with fusiform nerve
Presentation enlargement identified on MR imaging
• Most common signs/symptoms Image Interpretation Pearls
o HMSN I
• Consider HMSN when abnormally enlarged peripheral
• Similarly affected relatives common
nerves or nerve roots identified on MR imaging
• Distal extremity muscle weakness/atrophy (motor
> sensory), foot deformities (pes cavus, hammer
toes), j tendon reflexes, +/- palpable extremity
nerve masses
ISELECTED REFERENCES
• Relative lack of pain (in contrast to acquired 1. Kretzer RM et al: Hypertrophic neuropathy of the cauda
neuropathies) equina: case report. Neurosurgery. 54(2):515-8; discussion
518-9,2004
o HMSN II: Often minimally symptomatic
2. Graham D et al: Greenfield's Neuropathology. 7th ed.
o HMSN III: Clinical symptoms similar but more London, 628-632, 2002
severe than HMSN I, earlier presentation 3. Moore KR et al: The value of MR neurography for
• Delayed motor development, progressive muscular evaluating extraspinal neuropathic leg pain: a pictorial
weakness of legs and arms essay. A]NR Am] Neuroradiol. 22(4):786-94, 2001
o Other signs/symptoms 4. Cellerini M et al: MR imaging of the cauda equina in
• Back/lower extremity radicular pain +/- hereditary motor sensory neuropathies: correlations with
myelopathy sural nerve biopsy. A]NR Am] Neuroradiol. 21(10):1793-8,
2000
• Sensory loss, focal tenderness, dysesthesias
5. Keller MP et al: Inherited peripheral neuropathy. Semin
• +/- Palpable nerve mass Neurol. 19(4):353-62, 1999
• Cold feet, hair loss, leg edema 6. Hahn M et al: Hypertrophied cauda equina presenting as
• Clinical profile intradural mass: case report and review of literature. Surg
o HMSN type I: Distal extremity weakness, muscle Neurol. 49(5):514-8; discussion 518-9, 1998
atrophy (legs> arms) 7. Calore EE et al: Hypertrophic motor and sensory
• Sensation often normal until adulthood neuropathy type I (Charcot-Marie-Tooth disease):
• Hyporeflexia or areflexia, orthopedic foot ultrastructural study of sural nerve biopsy in members of a
family. Pathologica. 86(3):279-83, 1994
deformities, scoliosis (37-50%), tremor (~ 25%)
8. Vasilescu C: Hereditary motor and sensory neuropathy.
o H.MSN type II: Peripheral nerves not clinically Clinical, genetic and electrodiagnostic studies. Rom]
enlarged, lower extremity> hand weakness, sensory Neurol Psychiatry. 31(3-4):207-19, 1993
loss in distal extremities, foot deformities « HMSN 9. Gemignani F et al: Peroneal muscular atrophy with
I) hereditary spastic paraparesis (HMSN V) is pathologically
o HMSN type III: Infant with hyporeflexia, palpable heterogeneous. Report of nerve biopsy in four cases and
enlarged peripheral nerves, sensory loss +/- ataxia review of the literature. Acta Neuropathol (Bed).
83(2):196-201, 1992
Demographics 10. Chou SM: Immunohistochemical and ultrastructural
classification of peripheral neuropathies with onion-bulbs.
• Age Clin Neuropathol. 11(3):109-14, 1992
o HMSN I: Onset in 1st decade typical, delayed in
11. Choi SK et al: MR imaging in hypertrophic neuropathy: a
some patients until young or mid adulthood case of hereditary motor and sensory neuropathy, type I
o HMSN II: Often diagnosed later in life; symptoms (Charcot-Marie-Tooth). Clin Imaging. 14(3):204-7, 1990
commonly begin during 2nd decade 12. Bird TD: Hereditary motor-sensory neuropathies.
o HMSN III: Onset typically during infancy or early Charcot-Marie-Tooth syndrome. Neurol Clin. 7(1):9-23,
childhood 1989
• Gender: M = F
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HYPERTROPHIC NEUROPATHY
IIMAGE GALLERY 1
33
Typical
(Left) Axial Tl C+ MR with
fat-saturation (focal
hypertrophic neuropathy)
shows focal enhancement in
tibial division of sciatic nerve
(arrow). Adjacent peroneal
division (open arrow) is
uninvolved. (Right) Coronal
Tl C+ MR with fat-saturation
demonstrates fusiform
enlargement and
enhancement of left C7 root
(arrow).
Variant
(Left) Coronal TlWI MR
(Charcot-Marie-Tooth)
demonstrates diffuse bilateral
enlargement of spinal nerve
roots and lumbar plexus in a
patient imaged for
symptomatic scoliosis.
(Right) Axial T2WI MR
(Charcot-Marie-Tooth)
depicts cauda equina and
bilateral spinal nerve root
hypertrophy.
Variant
(Left) Axial T2WI MR
(Charcot-Marie-Tooth)
demonstrates prominent
enlargement of cauda equina
and bilateral spinal nerves.
(Rig/It) Axial Tl C+ MR
(Charcot-Marie-Tooth)
shows prominent
enlargement without
abnormal enhancement of
sacral spinal nerve roots.
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Coronal TlWI MR shows perineuroma of peroneal n. Coronal T2WI MR shows perineuroma of peroneal n.
(arrow) found during evaluation for peroneal n. palsy. Mass is multilobulated and heterogeneous in signal
Mass was contiguous with n. in all imaging planes. intensity (arrow). TI C+ (not shown) revealed avid
enhancement.
ITERMINOlOGY MR Findings
Abbreviations and Synonyms • TlWI
o Low signal intensity mass
• Peripheral nerve sheath tumor previously used term o May see high signal fatty atrophy in innervated
but less accurate description muscles (mm.)
• Malignant peripheral nerve sheath tumor (MPNST)
• T2WI
replaces earlier terms: Malignant schwannoma, o Heterogeneous, intermediate to high signal intensity
neurofibrosarcoma, neurogenic sarcoma o May see denervation edema, diffuse high signal in
Definitions innervated mm. .
• A grouping of benign and malignant primary tumors • STIR: Same as T2WI but more pronounced
of peripheral nerves (nn.) • Tl C+: Avidly enhance with contrast
• Schwannoma often contains areas of cystic
degeneration
IIMAGING FINDINGS • MPNST usually shows extensive central necrosis
.
..,...
,.
,' .
. - , -.
.:")'
;
- ~». 'J
~:
Traumatic Neuroma Hemangioma Neuritis Sarcoma (MFH)
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o Neurofibroma
Hemangioma • Plexiform neurofibroma: Arborizing tumor of
• Enlarged vessels may mimic multilobulated peripheral nerves occurring in neurofibromatosis
neurofibroma o Nerve sheath myxoma (neurothekeoma)
• Draining veins visible o Perineuroma
Neuritis • Malignant tumors
o Malignant peripheral nerve sheath tumor (MPNST)
• Increased T2 signal in affected n., muscles, without
o Lymphoma
mass
Other soft tissue sarcomas
• Not centered along n. ICLINICAL ISSUES
Presentation
!PATHOlOGY • Most common signs/symptoms
o Palpable, painless mass
General Features o Other signs/symptoms
• General path comments: Malignant degeneration seen • May cause neurologic symptoms
in neurofibroma, but rare in schwannoma
Demographics '
• Associated abnormalities: 5-13% of patients with
neurofibromatosis develop MPNST • Age
o Localized neurofibroma usually ages 20-30
Gross Pathologic & Surgical Features o Nerve sheath myxoma, perineuroma: Childhood to
• Schwannoma: Encapsulated; solid areas, edematous early adulthood
areas, cysts o Schwannoma and MPNST ages 20-50
• Neurofibroma: Not encapsulated, glistening, tan-white Treatment
• Nerve sheath myxoma: Gelatinous
• Perineuroma: Circumscribed white mass • Options, risks, complications
• MPNST: Usually large, white-tan, hemorrhage and o Some amenable to surgical excision
necrosis o Radiation, chemotherapy for MPNST
Microscopic Features
• Schwannoma: 2 types of cells, Antoni A (spindle cells) I DIAGNOSTIC CHECKLIST
and Antoni B (tumor cells surrounded by flUid)
• Neurofibroma: All elements of peripheral n. proliferate Image Interpretation Pearls
• Nerve sheath myxoma: Myxoid material, cells • Often can see tapered transition to normal contiguous
arranged in bundles n. at margin of mass
• Perineuroma: Elongated cells in parallel bundles;
storiform growth
• MPNST: Serpentine shape of tumor cells, arranged in I SELECTED REFERENCES
whorls or palisades 1. Rosai]: Surgical Pathology, vol 2. 9th ed. Edinburgh,
Mosby. 2629-2662, 2004
Staging, Grading or Classification Criteria 2. Skubitz KM, Skubitz AP. Characterization of sarcomas by
• Benign tumors means of gene expression. J Lab CUn Med. 144(2):78-91,
o Schwannoma (neurilemoma) 2004
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PERIPHERAL NERVE TUMOR
1 3. Lin Y, Daniel S, Forte V. Is a plexiform neurofibroma
pathognomonic of neurofibromatosis type I?
36 Laryngoscope. 114(8):1410-4, 2004
4. He Y, Zhang Z, Tian Z, Zhang C, Zhu H. The application of
magnetic resonance imaging-guided fine-needle aspiration
cytology in the diagnosis of deep lesions in the head and
neck. J Oral Maxillofac Surg. 62(8):953-8, 2004
5. Forthman CL, Blazar PE. Nerve tumors of the hand and
upper extremity. Hand Clin. 20(3):233-42, 2004
6. Beekman R, Slooff WB, Van Oosterhout MF, Lammens M,
Van Den Berg LH. Bilateral intraneural perineurioma
presenting as ulnar neuropathy at the elbow. Muscle
Nerve. 30(2):239-43, 2004
7. Robertson TC, Buck DA, Schmidt-Ullrich R, Powers CN,
Reiter ER. Isolated plexiform neurofibroma: treatment with
three-dimensional conformal radiotherapy. Laryngoscope.
114(7):1139-42, 2004
8. Shapiro SL. Endoscopic decompression of the
intermetatarsal nerve for Morton's neuroma. Foot Ankle
Clin. 9(2):297-304, 2004
9. Stamatis ED, Karabalis C. Interdigital neuromas: current
state of the art--surgical. Foot Ankle Clin. 9(2):287-96, 2004
10. Kim DH, MurovicJA, Tiel RL, Kline DG. Management and
outcomes in 318 operative common peroneal nerve lesions
at the Louisiana State University Health Sciences Center.
Neurosurgery. 54(6):1421-8; discussion 1428-9, 2004
11. Stemmer-Rachamimov AO, Louis DN, Nielsen GP,
Antonescu CR, Borowsky AD, Bronson RT, Burns DK,
Cervera P, McLaughlin ME, Reifenberger G, Schmale MC,
MacCollin M, Chao RC, Cichowski K, Kalamarides M,
Messerli SM, McClatchey AI, Niwa-Kawakita M, Ratner N,
Reilly KM, Zhu Y, Giovannini M. Comparative pathology
of nerve sheath tumors in mouse models and humans.
Cancer Res. 15;64(10):3718-24, 2004
12. Dellon AL, Kim J, Ducic 1. Painful neuroma of the posterior
cutaneous nerve of the forearm after surgery for lateral
humeral epicondylitis. J Hand Surg [Am]. 29(3):387-90,
2004
13. Velagaleti GY, Miettinen M, Gatalica Z. Malignant
peripheral nerve sheath tumor with rhabdomyoblastic
differentiation (malignant triton tumor) with balanced
t(7;9)(ql1.2;p24) and unbalanced translocation
der(16)t(I;16)(q23;q13). Cancer Genet Cytogenet.
149(1):23-7,2004
14. Hochman MG, Zilberfarb JL. Nerves in a pinch: imaging of
nerve compression syndromes. Radiol Clin North Am.
42(1):221-45,2004
15. Beaulieu S, Rubin B, Djang D, Conrad E, Turcotte E, Eary
JF. Positron emission tomography of schwannomas:
emphasizing its potential in preoperative planning. AJR
Am J Roentgenol. 182(4):971-4, 2004
16. HuangJH, Zaghloul K, Zager EL. Surgical management of
brachial plexus region tumors. Surg Neurol. 61(4):372-8,
2004
17. Arun D, Gutmann DH. Recent advances in
neurofibromatosis type 1. Curr Opin Neurol. 17(2):101-5,
2004
18. Mohan H, Nada R, Tahlan A, Punia RS, Mukherjee KK.
Peripheral nerve sheath tumours--a short series with some
uncommon variants. Indian J Pathol Microbiol.
46(2):204-6, 2003
19. Weiss SW, GoldblumJR: Soft Tissue Tumors. 4th ed. St
Louis, Mosby. 1111-1263,2001
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• . '~."l' ~,
, .7 . . ~:;.::}'til -
,I''''.
"W"
'0 ,
"r'"1'
"<
(Left) Coronal STIR MR
shows MPNST (arrow) of
thigh. Mass is heterogeneous
in signal intensity.
I ~ _',...
Appearance is nonspecific,
¢.~
and location adjacent to
if' , .• vessels is best clue to
'''~ ~
,0' '.
11,
",
Typical
(Left) Coronal STIR MR
shows neurofibromatosis of
sciatic nerve, forming a
multilobulated cascade of
oval, heterogeneous signal
intensity masses, (Right)
Coronal T7 C+ MR shows
neurofibromatosis diffusely
affecting sciatic n. Tumors
show variable enhancement
pattern,
Variant
(Left) Axial CECT shows
neurofibroma centered in
right L4 neural foramen,
extending into abdomen,
and eroding bone, Patient
had vague pain for 14 years
before undergoing
radiography, which showed
mass, (Right) Sagittal T2WI
MR through lumbar vertebral
body and pedicle shows
heterogeneous signal
intensity in neurofibroma
(arrows) causing unusually
extensive bone erosion of 2
adjacent spinal levels,
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PERIPHERAL NEUROLYMPHOMATOSIS
1
38
Coronal STIR MR shows L2-L4 dorsal root ganglia Axial·T7 C+ MR in neurolymphomatosis reveals
enlargement (arrows) & abnormal thickening & thickening & abnormal enhancement of infiltrated right
hyperintensity of the right lumbar plexus (open arrows). lumbar plexus (arrows).
[TERMINOLOGY MR Findings
• TlWI: Hypo- to isointense nerve/plexus thickening
Abbreviations and Synonyms • T2WI: Abnormal nerve T2 hyperintensity,
• Synonyms: Peripheral neurolymphomatosis (NL), disruption/distortion of normal fascicular morphology
Perineural lymphomatosis • STIR: Abnormal nerve T2 hyperintensity,
Definitions disruption/distortion of normal fascicular morphology
• Perineural plexus or peripheral nerve lymphomatous • Tl C+: Variable enhancement
infiltration Imaging Recommendations
• Best imaging tool: MR imaging
• Protocol advice: Multiplanar high-resolution MRI
IIMAGING FINDINGS (Tl WI, fat-saturated T2WI or STIR, fat-saturated Tl
General Features C+)
• Best diagnostic clue: Diffusely infiltrating plexus or
peripheral nerve lesion in patient with lymphoma
• Location: Plexus or peripheral nerve
IDIFFERENTIAL DIAGNOSIS
• Morphology Peripheral nerve metastasis
o Abnormal plexus/nerve thickening • Usually more focal; signal intensity mimics primary
o Disruption/distortion of normal fascicular nerve tumor
morphology • Clinical history of known malignancy makes diagnosis
Radiographic Findings CIDP
• Radiography: Usually normal unless bone metastases • Fusiform nerve/plexus thickening
CT Findings • Often bilateral, symmetriC
• CECT: Often normal; abnormal cases show fusiform or • Clinical, history, and laboratory findings distinguish
nodular thickening + variable enhancement Hypertrophic neuropathy
• Fusiform nerve/plexus thickening
DDx: Neurolymphomatosis
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PERIPHERAL NEUROLYMPHOMATOSIS
Key Facts
1
Terminology • CIDP 39
• Synonyms: Peripheral neurolymphomatosis (NL), • Hypertrophic neuropathy
Perineural lymphomatosis • Nerve/plexus stretch injury
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RADIAL NEUROPATHY
1
40
Axial TlWI MR shows enlargement of left radial nerve Axial STIR MR shows enlargement, marked T2
(arrow) as it crosses humeral spiral groove in Parkinson hyperintensity of left radial nerve (arrow) as it crosses
patient who awoke with radial nerve deficit humeral spiral groove. Abnormal Signal intensity equals
that of regional vessels.
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RADIAL NEUROPATHY
Key Facts
1
Terminology Top Differential Diagnoses 41
• Radial neuropathy (RN), radial nerve entrapment, • Median neuropathy
posterior interosseous nerve (PIN) syndrome • Ulnar neuropathy
• Lateral epicondylitis
Imaging Findings
• Best diagnostic clue: Focal radial nerve enlargement, Diagnostic Checklist
abnormal T2 hyperintensity • Radial nerve palsy is common complication of
• Most commonly entrapped at mid humeral shaft or humeral fracture
fibrous arch of Frohse above elbow
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FEMORAL NEUROPATHY
1
42
Coronal STIR MR shows a hyperintense enlarged Axial STIR MR shows marked right femoral nerve
femoral nerve at right femoral canal. Symptoms arose enlargement, fascicular hyperintensity within iliopsoas
following inguinal herniorrhaphy, and exploration groove (arrow). Normal left femoral nerve indicated by
revealed suture (arrow) around nerve. open arrow.
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FEMORAL NEUROPATHY
Key Facts
1
Terminology Top Differential Diagnoses 43
• Synonyms: Femoral neuropathy, femoral • Neoplastic femoral nerve infiltration
mononeuropathy, femoral nerve (FN) palsy • Nerve sheath tumor
• Femoral nerve (FN) entrapment or injury 2° to direct • Lumbosacral disk syndromes
trauma, compression, stretch injury, or ischemia • Lumbar plexopathy
Imaging Findings Diagnostic Checklist
• Best diagnostic clue: Femoral nerve enlargement, • Look carefully for lesion or hematoma in iliopsoas
abnormal T2 hyperintensity groove or femoral canal
I CLINICAL ISSUES
Presentation
IIMAGE GALLERY
• Most common signs/symptoms
o Acute symptom onset, pain/weakness in FN
distribution, diminished/absent knee jerk reflex,
thigh muscle atrophy
o Severe back/groin pain (retroperitoneal hematoma)
Demographics
• Age: All ages
• Gender: M = F
Natural History & Prognosis
• Recovery is rule over few days ~ months in most cases
Treatment (Left) Axial T7 WI MR of both legs in femoral neuropathy patient
• Physical therapy, avoidance of excessive hip shows marked volume loss and fatty replacement of right quadriceps
muscles (chronic denervation). (Right) Axial STIR MR in femoral
abduction/external rotation, knee bracing, treatment
neuropathy patient shows marked right quadriceps muscle volume
of causative mass lesions 1055, T2 hyperintensity indicating component of acute (ongoing)
denervation on top of chronic denervation.
IDIAGNOSTIC CHECKLIST
Consider
• Femoral neuropathy is uncommon
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ULNAR NEUROPATHY
1
44
Axial TlWI MR in patient with clinically symptomatic Axial T2WI MR with fat-saturation of ulnar nerve at
ulnar neuropathy shows focal enlargement of ulnar medial epicondyle (arrow) shows focal enlargement, T2
nerve (arrow) within cubital tunnel. hyperintensity, loss of internal architecture
(axonotmesis).
1!~
~ ~'
',.'.~
I' '- .-
I'
. -.
t,
~~.,
~·Y ~
;.' .
UN Transection Brachial Plexitis Plexiform NF Perineural Vein
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ULNAR NEUROPATHY
Key Facts
1
Terminology • Medial epicondylitis 45
• Synonyms: Ulnar nerve entrapment, cubital tunnel Pathology
syndrome • Edematous/indurated UN +/- thickened retinaculum,
• Partial fixation, compression, or distortion of ulnar fibrous stranding
nerve (UN) • +/- Anconeus epitrochlearis, enlarged medial triceps
Imaging Findings head
• Best diagnostic clue: UN enlargement +/- T2 Clinical Issues
hyperintensity at cubital or Guyon tunnel in • Pain, paresthesias in ulnar nerve distribution
symptomatic patient
Diagnostic Checklist
Top Differential Diagnoses • MR imaging is more sensitive for acute than chronic
• Acute direct nerve trauma neuropraxia
• Idiopathic brachial plexitis (Parsonage-Turner • Focal ulnar nerve enlargement, abnormal T2
syndrome) hyperintensity at retinaculum just distal to medial
• Nerve sheath tumor epicondyle diagnostic for UN neuropathy
• Enlarged perineural vein
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ULNAR NEUROPATHY
1 Gross Pathologic & Surgical Features
o In situ decompression brings rapid relief, but
transposition may bring longer symptom remission
46 • Edematous/indurated UN +/- thickened retinaculum,
fibrous stranding
• +/- Anconeus epitrochlearis, enlarged medial triceps IDIAGNOSTIC CHECKLIST
head
Consider
Microscopic Features
• MR imaging is more sensitive for acute than chronic
• Nerve edema +/- inflammatory cells, fibrous neuropraxia
infiltration, fascicular atrophy/loss • Search for anatomic abnormalities (enlarged medial
Staging, Grading or Classification Criteria head of triceps, anconeus muscle, osteophyte,
• Three severities of nerve injury thickened retinaculum)
o Neuropraxic injury (mildest) Image Interpretation Pearls
• Focal myelin sheath damage without axonal • Focal ulnar nerve enlargement, abnormal T2
disruption hyperintensity at retinaculum just distal to medial
• Contiguous swollen/T2 hyperintense nerve epicondyle diagnostic for UN neuropathy
fascicles
o Axonotmetic injury (intermediate)
• Axon/myelin sheath disruption; subsequent ISELECTED REFERENCES
Wallerian degeneration leaves Schwann cells and
endoneurium intact 1. Matsuzaki H et al: Long-term clinical and neurologic
• Homogeneously increased T2 signal intensity, loss recovery in the hand after surgery for severe cubital tunnel
syndrome.] Hand Surg [Am]. 29(3):373-8, 2004
of fascicular architecture at injury site 2. Hochman MG et al: Nerves in a pinch: imaging of nerve
o Neurotmetic injury (most severe) compression syndromes. Radiol Clin North Am.
• Functional +/- anatomical transection 42(1):221-45, 2004
• Axon, myelin sheath disruption with 3. Park GY et al: The ultrasonographic and electrodiagnostic
discontinuity of some/all of surrounding findings of ulnar neuropathy at the elbow. Arch Phys Med
connective tissues ~ Wallerian degeneration, Rehabil. 85(6):1000-5, 2004
regeneration failure 4. Thornton R et al: Magnetic resonance imaging of sports
• Cannot always distinguish from axonotmesis injuries of the elbow. Top Magn Reson Imaging.
14(1):69-86,2003
5. Kim DH et al: Surgical outcomes of 654 ulnar nerve lesions.
] Neurosurg. 98(5):993-1004, 2003
I CLINICAL ISSUES 6. Murata K et al: Causes of ulnar tunnel syndrome: a
retrospective study of 31 subjects.] Hand Surg [Am].
Presentation 28(4):647-51,2003
• Most common signs/symptoms 7. Dellon AL et al: Results of the musculofasciallengthening
o Range of symptoms; mild transient paresthesias of technique for submuscular transposition of the ulnar nerve
4th and fifth digits ~ claw hand/digits, intrinsic at the elbow.] Bone Joint Surg Am. 85-A(7):1314-20, 2003
muscle atrophy 8. Matev B: Cubital tunnel syndrome. Hand Surg.
8(1): 127-31, 2003
o +/- Severe elbow/wrist pain radiating proximally or
9. Kato H et al: Cubital tunnel syndrome associated with
distally medial elbow Ganglia and osteoarthritis of the elbow.]
• Clinical profile Bone]oint Surg Am. 84-A(8):1413-9, 2002
o Pain, paresthesias in ulnar nerve distribution 10. Chung CB et al: Magnetic resonance imaging of the upper
o Throwing athletes with elbow pain of medial extremity: advances in technique and application. Clin
epicondylitis + paresthesias Orthop. (383):162-74, 2001
11. Arle JE et al: Surgical treatment of common entrapment
Demographics neuropathies in the upper limbs. Muscle Nerve.
• Age: Adults> children 23(8):1160-74,2000
12. Martinoli C et al: US of nerve entrapments in osteofibrous
• Gender: M ::; F
tunnels of the upper and lower limbs. Radiographies. 20
Natural History & Prognosis Spec No:S199-213; discussion S213-7, 2000
13. Ruocco M] et al: MR imaging of ulnar nerve entrapment
• Rest, avoidance of provocative activities may produce
secondary to an anomalous wrist muscle. Skeletal Radiol.
remission in mild cases 27(4):218-21, 1998
• More severe cases show variable response to surgical 14. Chiou H] et al: Cubital tunnel syndrome: diagnosis by
decompression high-resolution ultrasonography. ] Ultrasound Med.
o Chronic pain, muscle weakness, atrophy portend 17(10):643-8, 1998
worse prognosis 15. Britz GW et al: Ulnar nerve entrapment at the elbow:
correlation of magnetic resonance imaging, clinical,
Treatment electrodiagnostic, and intraoperative findings.
• Conservative: Physical therapy, avoidance of Neurosurgery. 38(3):458-65; discussion 465, 1996
provocative maneuvers 16. Zeiss] et al: The ulnar tunnel at the wrist (Guyon's canal):
• Surgical: Osteophyte or anconeus resection, normal MR anatomy and variants. A]R Am] Roentgenol.
158(5):1081-5, 1992
decompression +/- nerve transposition
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ULNAR NEUROPATHY
IIMAGE GALLERY 1
47
Typical
(Left) Axial T1 C+ MR with
fat-saturation depicts uniform
enhancement of left ulnar
nerve (arrow) at elbow with
loss of internal architecture
(axonotmesis). (Right) Axial
STIR MR shows normal
appearance of ulnar nerve
(arrow) despite chronic
clinical symptoms.
Variant
(Left) Axial T1WI MR shows
mild abnormal UN rounding
(arrow) with associated
perineural inflammatory
changes of subcutaneous fat
(mild neuropraxic injury).
(Right) Axial STIR MR shows
rounding of UN at elbow
(arrow) with mild
hyperintensity of several
fascicles (mild neuropraxic
injury).
Variant
(Left) Axial T1 WI M R distal
to medial epicondyle shows
focal UN rounding (arrow)
and anconeus epitrochlearis
muscle (open arrow)
covering cubital tunnel.
(Right) Axial STIR MR distal
to medial epicondyle shows
focal ulnar nerve rounding
. (arrow) & mild fascicular
hyperintensity (neuropraxic
injury).
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SUPRASCAPULAR NERVE ENTRAPMENT
1
48
Coronal graphic shows suprascapular nerve coursing Coronal T2WI MR shows cyst extending from torn
through suprascapular notch (open arrow) & labrum into spinoglenoid notch (open arrow). Increased
spinoglenoid notch (arrow). signal intensity in infraspinatus muscle (arrow) reflects
denervation.
ITERMINOlOGY MR Findings
Abbreviations and Synonyms • TlWI
o Low signal intensity mass or venous varicosities in
• Spinoglenoid or suprascapular notch impingement suprascapular or spinoglenoid notch
Definitions o Fatty atrophy in affected mm. if chronic
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SUPRASCAPULAR NERVE ENTRAPMENT
Key Facts
1
Imaging Findings • Rotator cuff tendinopathy 49
• Best diagnostic clue: Mass compressing nerve, and Pathology
abnormal signal in innervated mm. • Paralabral cyst due to tear of posterosuperior glenoid
• Occurs at suprascapular or spinoglenoid notch labrum
Top Differential Diagnoses • Venous varicosities
• Cervical radiculopathy • Tumor compressing nerve
• Parsonage-Turner syndrome (brachial neuritis)
• Rotator cuff tear
ISELECTED REFERENCES
IPATHOLOGY 1. Carroll KW et al: Enlarged spinoglenoid notch veins
General Features causing suprascapular nerve compression. Skeletal Radiol.
32(2):72-7, 2003
• General path comments 2. Tung GA et al: MR imaging and MR arthrography of
o Suprascapular nerve: CS and C6 nerve roots paraglenoid labral cysts. AJR Am J Roentgenol.
• Through suprascapular notch 174(6):1707-15,2000
• Innervates supraspinatus mm., extends deep to 3. Fabre T et al: Entrapment of the suprascapular nerve. J
mm. Bone Joint Surg Br. 81(3):414-9, 1999
• Through spinoglenoid notch 4. Zehetgruber H et al: Suprascapular nerve entrapment. A
• Innervates infraspinatus mm. meta-analysis. lnt Orthop. 26(6):339-43,
2002;26(6):339-43.
• Etiology
o Paralabral cyst due to tear of posterosuperior glenoid
labrum
o Venous varicosities IIMAGE GALLERY
o Tumor compressing nerve
• Epidemiology: Overhead athletes
Gross Pathologic & Surgical Features
• Cyst often entered at arthroscopy via torn labrum
• Open surgery: Tense, slightly blue cyst visible
• Nerve: Normal, or may show attenuation or
enlargement
Microscopic Features
• Cyst thin-walled; may be septated
ICLINICAL ISSUES (Left) Sagittal T2WI MR shows cyst in spinoglenoid notch (open
arrow) and denervation changes of infraspinatus muscle (arrow).
Presentation Supraspinatus muscle (curved arrow) is normal signal intensity.
• Most common signs/symptoms: Weakness, pain (Right) Coronal T7 WI M R shows infraspinatus muscle atrophy
(arrows) due to longstanding impingement.
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MEDIAN NERVE ENTRAPMENT
1
50
Axial STIR MR shows abnormal signal in median nerve Axial TlWI MR shows mild enlargement of median
(open arrow) due to infiltration of nerve by sarcoid. nerve (open arrow) due to sarcoidosis. Flexor
Flexor retinaculum (curved arrows) is' normal in retinaculum (curved arrows) is normal. Tr = trapezium
appearance. Tr = trapezium.
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MEDIAN NERVE ENTRAPMENT
Key Facts
1
Terminology Top Differential Diagnoses 51
• Carpal tunnel syndrome: Entrapment at carpal tunnel • Cervical Radiculopathy
• Pronator syndrome: Entrapment at pronator teres • Tenosynovitis
• Peripheral nerve sheath tumor
Imaging Findings
• Best diagnostic clue: Increased signal in nerve on STIR Clinical Issues
MRI • Numbness, tingling along course of nerve
• Positive Tinel sign
ICLINICAL ISSUES
Presentation
• Most common signs/symptoms (Left) Axial T2WI MR shows persistent abnormal signal in median
o Numbness, tingling along course of nerve nerve (arrow) at level of proximal metacarpals follOWing incomplete
o Positive Tinel sign carpal tunnel release. (Right) Coronal graphic shows fibrous band of
o Tenderness at pronator region or carpal tunnel flexor retinaculum overlying median nerve (yellow). Nerve is
edematous. Flexor tendons covered by tendon sheaths are shown
Demographics deep to the median nerve.
• Gender: Carpal tunnel: F:M = 3:1
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COMMON PERONEAL NEUROPATHY
1
52
Axial T2WI MR with fat-saturation shows CPN fascicular Axial T1WI MR reveals mild CPN fascicular enlargement
enlargement, abnormal T2 hyperintensity (arrow) at (arrow) + anterior compartment muscle volume loss
fibular head & acute anterior compartment muscle with fatty atrophy (open arrows) representing more
denervation (open arrow). chronic denervation change.
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..•.
. J.! ....:
..
. ..{',n.
Presentation .... ~ '
,f,
~.'
~.
.Jo,.'
• Most common signs/symptoms: Foot drop, sensory ,-
abnormality along anterolateral leg
• Clinical profile: Foot drop +/- history of acute or
\
chronic trauma to lateral knee
Demographics
• Age: Adults> > children, infants
Natural History & Prognosis
• Variable but frequently good recovery following (Left) Axial TlWI MR shows isointense fascicular enlargement of left
conservative management CPN at fibular head (arrow). (Right) Axial STIR MR confirms
• Decompression in recalcitrant cases may produce fascicular enlargement, abnormal T2 hyperintensity of left CPN at
improvement even after 6-12 months delay fibular head (arrow).
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POSTERIOR TIBIAL NERVE ENTRAPMENT
1
54
Sagittal graphic shows posterior tibial nerve (open Coronal bone CT shows coalition variant with os
arrow) coursing beneath flexor retinaculum (curved sustentaculi (open arrow) narrowing tarsal tunnel. Note
arrows) between flexor hallucis longus and flexor quadratus plantae atrophy (curved arrow) compared to
digitorum longus tendons. normal contralateral side.
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Key Facts
1
Terminology • Calcaneus stress fracture 55
• Synonym: Tarsal tunnel syndrome • Sciatica
• Talocalcaneal coalition
Imaging Findings • Peripheral neuropathy
• Distinguishes between different etiologies
• Fatty atrophy of intrinsic plantar mm.
Clinical Issues
• Vague heel pain, dysesthesias
Top Differential Diagnoses
• Plantar fasciitis
Diagnostic Checklist
.. Symptom vagueness => difficult clinical diagnosis
I PATHOLOGY
ISELECTED REFERENCES
General Features 1. Hochman MG et al: Nerves in a pinch: imaging of nerve
• General path comments compression syndromes. Radial Clin North Am.
o Posterior tibial n. between flexor digitorum longus, 42(1):221-45, 2004
flexor hallucis longus mm. 2. Kinoshita M et al: Tarsal tunnel syndrome associated with
an accessory muscle. Foot Ankle Int. 24(2):132-6, 2003
o Innervates intrinsic plantar mm. of foot, also
3. Labib SA et al: Heel pain triad (HPT): the combination of
sensory plantar fasciitis, posterior tibial tendon dysfunction and
o Passes through fibro-osseous tunnel in hindfoot tarsal tunnel syndrome. Foot Ankle Int. 23(3):212-20, 2002
• Beneath medial malleolus and sustentaculum tali 4. Oh SJ et al: Entrapment neuropathies of the tibial
o Divides into 3 branches (posterior tibial) nerve. Neurol Clin. 17(3):593-615, vii,
• Medial plantar, lateral plantar, medial calcaneal 1999
• Etiology 5. Lau JT et al: Tarsal tunnel syndrome: a review of the
o Overuse syndrome in runners literature. Foot Ankle Int. 20(3):201-9, 1999
o Space occupying lesion in tarsal tunnel (majority of
patients)
• Tumors: Nerve sheath, lipoma, rarely sarcoma IIMAGE GALLERY
• Non-neoplastic: Venous varicosities,
tenosynovitis, ganglion cyst, accessory mm.
o Fracture
o Talocalcaneal coalition: Bony overgrowth can
compress nerve
o Valgus hindfoot: Stretches n.
Gross Pathologic & Surgical Features
• Nerve enlargement or flattening
'ICLINICALISSUES
Presentation (Left) Axial TlWI MR shows anomalous flexor digitorum longus m.
• Most common signs/symptoms (arrow) compressing tarsal tunnel. Note atrophy of quadratus plantae
o Vague heel pain, dysesthesias (curved arrow). (Right) Coronal T2WI MR shows ganglion cyst (open
o Tinel sign: Tingling along course of nerve when arrow) in tarsal canal. Curved arrow shows flexor hallucis longus 1.;
nerve is tapped straight arrow shows posterior tibial neurovascular bundle.
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Post-Interventional Procedure
Imaging and Complications
Myelography Complications VII-I-2
Vertebroplasty Complications VII-I-6
CSF Leakage Syndrome VII-I-IO
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MYELOGRAPHY COMPLICATIONS
1
2
Coronal graphic shows complications of C1-2 puncture Axial CT following attempted myelogram shows focal
with cord injury from needle, + subarachnoid blood. contrast within cord substance + small amount of
Note close proximity to caudal loop of PICA (arrow). epidural gas.
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MYELOGRAPHY COMPLICATIONS
Key Facts
1
Terminology • Cord injury: MRI cord contusion 3
• Any unexpected sequela following myelography • Iatrogenic epidermoid: CSF intensity/density lesion
• Major complications: Cord injury, anaphylaxis, • Replacing needle stylet before withdrawing helps to
arachnoiditis, CSF leak, contrast reaction prevent headache
• Minor complications: PDPH, transient neurologic Pathology
sequela, incorrect injection, contrast reaction • Incidence of headache with 22 gauge atraumatic
• Delayed complications: Iatrogenic epidermoid needle is 5% vs. 30% for 20 gauge bevelled needles
Imaging Findings Clinical Issues
• Most common complications (e.g., headache) have • Most common complication: Headache
no imaging findings • Usually begins within 3 days and lasts 3-5 days
• CSF leak: Radionuclide cisternography collection • PDPH: Treatment for headache is supportive
• Intracranial hydrocephalus: "Sagging" midbrain
• Aseptic meningitis: Meningeal enhancement
• Arachnoiditis: Clumped nerve roots, empty thecal
sac, mild cauda equina enhancement
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MYELOGRAPHY COMPLICATIONS
1 • Majority are late complications of spinal puncture
during the early neonatal period Treatment
4 • Have been discovered 2-23 yrs following apparent • Contrast reaction: Standard care
time of implantation • PDPH: Treatment for headache is supportive
• Epidemiology o Non-narcotic analgesics, hydration, & time
o PDPH o Bed rest has been shown to be of no benefit
• Occurs in 36-58% of myelograms performed with o There is no clinical evidence that maintaining a
22-gauge QUincke needles supine position before/headache onset is effective
• Incidence of headache with 22 gauge atraumatic o Epidural blood patch
needle is 5% vs. 30% for 20 gauge bevelled needles • Rarely necessary
• Incidence with 24 gauge atraumatic needle is • Perform for PDPH persisting longer tl}an one week
1-2% but requires aspiration if CSF is needed • Place 15-30 cc of autologous blood within
o Arachnoiditis: Rare, dehydration increases incidence extradural space adjacent to puncture site
o Iatrogenic epidermoid • Very effective ~ produces remission in 89% of
• Epidermoid cysts are uncommon ~ 0.5-1% of all cases, and a further 8% relieved by a 2nd injection
spinal tumors; acquired tumors account for"" 40% • CSF leak
• Incidence significantly ~ after nonstylet needles o Large volume blood patch (60 cc) is often successful
replaced by tight-fitting stylet needles o Surgical closure of dural tear is option of last resort
• Associated abnormalities • Iatrogenic epidermoid: Complete excision is usually
o Subdural hematoma: Rare possible and is curative
o Nerve rootlet avulsion: Rare
Microscopic Features IDIAGNOSTIC CHECKLIST
• CSF leak: Aseptic inflammatory reaction
• Iatrogenic epidermoid: Contains keratin, cholesterol Consider
crystals, desquamed epithelial cells • Premyelography MRI screening recommended for
patients with spinal canal stenosis, severe spondylosis,
& myelopathy of any cause
ICLINICAL ISSUES
Presentation ISELECTED REFERENCES
• Most common signs/symptoms 1. Ziv ET et al: Iatrogenic intraspinal epidermoid tumor: two
o Most common complication: Headache cases & review of the literature. Spine. 29(1):EI5-8, 2004
• Usually begins within 3 days and lasts 3-5 days 2. Turnbull DK et al: Post-dural puncture headache:
o Nausea, vomiting, hearing loss, tinnitus, vertigo, pathogenesis, prevention and treatment. Br] Anaesth.
dizziness, paresthesias 91(5):718-29,2003
o Seizures: Dehydration increases incidence 3. Park]C et al: Iatrogenic spinal epidermoid tumor. A
o Visual disturbances such as diplopia or cortical complication of spinal puncture in an adult. Clin Neurol
Neurosurg. 105(4):281-5,2003
blindness have been reported
4. Peterman SB: Postmyelography headache: a review.
o Cranial nerve palsies are not uncommon Radiology. 200(3):765-70, 1996
o Transient neurologic sequela (e.g., expressive 5. Nakakoshi T et al: Aseptic meningitis complicating iotrolan
dysphasia); rare myelography. A]NR Am] Neuroradiol. 12(1):173, 1991
o Iatrogenic epidermoid: Progressive radicular & back 6. Robertson H] et al: Cervical myelography: survey of modes
pain, gait abnormalities, sphincter disturbances of practice and major complications. Radiology.
174(1):79-83, 1990
Demographics 7. Katoh Y et al: Complications of lateral CI-2 puncture
• Age: PDPH: More common in younger age patients myelography. Spine. 15(11):1085-7, 1990
• Gender: PDPH: M:F = 1:2 8. Kostiner AI et al: Outpatient lumbar myelography with
metrizamide. Radiology. 155(2):383-5, 1985
• Body Habitus: Lower body mass index is a significant
9. Sarno ]B: Transient expressive (nonfluent) dysphasia after
factor for PDPH in some series metrizamide myelography. A]NR Am] Neuroradiol.
Natural History & Prognosis 6(6):945-7, 1985
10. Baker F] et al: Aseptic meningitis complicating metrizamide
• PDPH myelography. A]NR Am] Neuroradiol. 3(6):662-3, 1982
o 72% resolve within 7 days, 87% in 6 mos 11. Brodey PA et al: Nerve rootlet avulsion. A complication of
o Decrease risk by using myelography using the Chynn needle. Radiology.
• Smaller gauge, atraumatic (blunt) needles 125(3):734, 1977
• Paramedian approach 12. Kennedy TF et al: Nerve rootlet avulsion as a complication
• Parallel orientation of needle bevel to dural fibers of myelography with the Cualtico needle. Radiology.
119(2):389, 1976
• Replacing stylet before withdrawing
o IV hydration, no removal of CSF, experience of
myelographer, & inpatient status have been
inconsistently associated with lower PDPH rates
o When performed as an outpatient procedure does
not have increased incidence of side effects or risk
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MYELOGRAPHY COMPLICATIONS
IIMAGE GALLERY 1
5
Typical
(Left) Sagittal CT
myelography sagittal
reformat reveals an incorrect
epidural + subdural
injection. Often the study is
still diagnostic as pathology
may efface the epidural
contrast (arrow). (Right)
Sagittal CT myelography
reformat demonstrates a
mixed compartment
injection; normal intradural
contrast is present as is a
lentiform-shaped subdural
collection (arrow).
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VERTEBROPLASTY COMPLICATIONS
1
6
Axial bone CT shows cement in epidural space behind Axial bone CT shows cement in foramen (arrows). Slice
vertebral body of anterior spinal canal (arrows). Patient above showed bulk of cement in vertebral body. Patient
was asymptomatic after procedure. suffered pain and radiculopathy after procedure
(Courtesy 5. Dunnagan, MD).
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VERTEBROPLASTY COMPLICATIONS
Key Facts
1
Imaging Findings • Use of intrathecal dye can help prevent cement 7
• Extravasation of cement into spinal canal extravasation when visualization of vb borders
• Extravasation of cement into foramen suboptimal
• Extravasation of cement into vertebral plexus • Pre- and post-contrast MRI used for detecting
• Cement conforms to shape of vein, slowly flows osteomyelitis, "bounceback" fracture
towards central veins on fluoro Clinical Issues
• Pulmonary embolization of cement • Extravasation of cement into canal
• Vertebral Osteomyelitis • Most often asymptomatic
• "Bounce back" fracture
• CT is best for defining cement extension beyond vb, Diagnostic Checklist
into foramen, venous and pulmonary embolization • CT in any case of unexplained symptomatology
• MR best for detecting osteomyelitis, early signs of follOWing procedure
"bounce back" fracture
• Extravasation or embolization of cement - use post
procedural plain films
• Bone or wide soft tissue windows especially useful
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VERTEBROPLASTY COMPLICATIONS
1 o Embolization of cement into vein or lungs percutaneous vertebroplasty. ] Vase Interv Radiol.
14(6):683-96,2003
• Most often asymptomatic
8 • Shortness of breath, chest pain in minority of 15. Sarzier]S et al: Intrathecal injection of contrast medium to
pulmonary emboli prevent polymethylmethacrylate leakage during
percutaneous vertebroplasty. A]NR Am] Neuroradiol.
• Compromised pulmonary function seen rarely 24(5):1001-2,2003
• Cardiopulmonary collapse and death reported 16. Hodler] et al: Midterm outcome after vertebroplasty:
• Patients with advanced pulmonary compromise predictive value of technical and patient-related factors.
not good candidates for aggressive vertebroplasty Radiology. 227(3):662-8, 2003
o Osteomyelitis 17. Yeom]S et al: Leakage of cement in percutaneous
• Delayed onset of back pain transpedicular vertebroplasty for painful osteoporotic
• Elevated sedimentation rate, C-reactive protein, compression fractures. ] Bone Joint Surg Br. 85(1):83-9,
white cell count 2003
18. Evans A] et al: Vertebral compression fractures: pain
• Positive blood cultures reduction and improvement in functional mobility after
• Positive aspirate or bone biopsy percutaneous polymethylmethacrylate vertebroplasty
o "Bounce back" fractures retrospective report of 245 cases. Radiology. 226(2):366-72,
• Delayed onset of new back pain 2003
19. Shapiro S et al: Surgical removal of epidural and intradural
polymethylmethacrylate extravasation complicating
IDIAGNOSTIC CHECKLIST percutaneous vertebroplasty for an osteoporotic lumbar
compression fracture. Case report.] Neurosurg. 98(1
Consider Suppl):90-2, 2003
20. Uppin AA et al: Occurrence of new vertebral body fracture
• CT in any case of unexplained symptomatology after percutaneous vertebroplasty in patients with
following procedure osteoporosis. Radiology. 226(1):119-24, 2003
21. Moreland DB et al: Vertebroplasty: techniques to avoid
complications. Spine]. 1(1):66-71,2001
I SELECTED REFERENCES
1. Stricker K et al: Severe hypercapnia due to pulmonary
embolism of polymethylmethacrylate during
vertebroplasty. Anesth Analg. 98(4):1184-6, table of
contents, 2004
2. Appel NB et al: Percutaneous vertebroplasty in patients
with spinal canal compromise. A]R Am] Roentgenol.
182(4):947-51,2004
3. Lin EP et al: Vertebroplasty: cement leakage into the disc
increases the risk of new fracture of adjacent vertebral
body. A]NR Am] Neuroradiol. 25(2):175-80, 2004
4. Brown DB et al: Treatment of chronic symptomatic
vertebral compression fractures with percutaneous
vertebroplasty. A]R Am] Roentgenol. 182(2):319-22, 2004
5. Tsai IT et al: Polymethylmethacrylate cement dislodgment
following percutaneous vertebroplasty: a case report. Spine.
28(22):E45 7-60, 2003
6. Baroud G et al: Biomechanical explanation of adjacent
fractures following vertebroplasty. Radiology. 229(2):606-7;
author reply 607-8,2003
7. Mathis ]M: Percutaneous vertebroplasty: complication
avoidance and technique optimization. A]NR Am]
Neuroradiol. 24(8):1697-706, 2003
8. Chiiders]C ]r: Cardiovascular collapse and death during
vertebroplasty. Radiology. 228(3):902; author reply 902-3,
2003
9. Kang ]D et al: Cement augmentation of osteoporotic
compression fractures and intraoperative navigation:
summary statement. Spine. 28(15):S62-3, 2003
10. Gangi A et al: Percutaneous vertebroplasty: indications,
technique, and results. Radiographies. 23(2):e10, 2003
11. Kelekis AD et al: Radicular pain after vertebroplasty:
compression or irritation of the nerve root? Initial
experience with the "cooling system". Spine. 28(14):E265-9,
2003
12. Mousavi P et al: Volumetric quantification of cement
leakage following percutaneous vertebroplasty in
metastatic and osteoporotic vertebrae. ] Neurosurg. 99(1
Suppl):56-9, 2003
13. Koessler M] et al: Fat and bone marrow embolism during
percutaneous vertebroplasty. Anesth Analg. 97(1):293;
author reply 294, 2003
14. Stallmeyer M] et al: Optimizing patient selection in
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VERTEBROPLASTY COMPLICATIONS
IIMAGE GALLERY 1
9
Typical
(Left) Lateral radiography
shows large volume of
cement beyond vertebral
body in disc interspace and
beyond, between two other
vertebral bodies also treated
which contain cement within
their confines. (Right)
Sagittal bone CT reformation
shows cement in
prevertebral vein (arrow),
extending into renal vein
(open arrow), the two foci
were connected on
contiguous slices. Patient
was asymptomatic.
Typical
(Left) Axial CECT shows
cement embolus bifurcating
into tertiary branches of
pulmonary artery (arrow)
following vertebroplasty in
asymptomatic patient.
(Right) Sagittal T2WI MR
shows high signal
surrounding cement several
weeks after vertebroplasty in
patient on chronic steroid
therapy. Aspiration showed
staph. aureus infection
(Courtesy 5. Dunnagan,
MD).
Typical
(Left) Axial bone CT shows
column of cement extending
into spinal canal,
compressing contrast-filled
thecal sac. (Right) Axial bone
CT shows mushroom of
cement (arrow) extravasating
into thecal sac filled with
contrast (Courtesy 5.
Dunnagan, MD).
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CSF LEAKAGE SYNDROME
1
10
Anteroposterior myelography shows numerous nerve Coronal T1 C+ MR shows hyperemic, thickened dura,
sleeve cysts, one of which leaks (amorphous contrast effacement of suprasellar cistern due to downward
collection, arrows). Patient had SIH, with postural brain displacement in SIH patient with CSF leak from
headaches and subdural effusion. ruptured nerve sleeve cyst.
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CSF LEAKAGE SYNDROME
1 o LP shows low CSF pressure +/- pleocytosis, increased
protein
patients with spontaneous intracranial hypotension. Surg
Neurol. 58(3-4):280-4; discussion 284-5, 2002
12 o Other signs/symptoms 10. Black P: Cerebrospinal fluid leaks follOWing spinal surgery:
• Cranial nerve palsies use of fat grafts for prevention and repair. Technical note. J
Neurosurg. 96(2 Suppl):250-2, 2002
• Visual disturbance
11. Chen CJ et al: Spinal MR findings in spontaneous
• Rare: Severe encephalopathy with disturbed intracranial hypotension. Neuroradiology.
consciousness, death 44(12):996-1003, 2002
• Clinical profile: Onset of postural headache after 12. Schrijver I et al: Spontaneous spinal cerebrospinal fluid
spinal surgery, or lumbar puncture leaks and minor skeletal features of Marfan syndrome: a
microfibrillopathy. J Neurosurg 96: 483-489, 2002
Natural History & Prognosis 13. Yousry I et al: Cervical MR imaging in postural headache:
• 75% resolve spontaneously within 3 months (dural MR signs and pathophysiological implications. AJNR
thickening, venous engorgement disappear; fluid 22(7):1239-50, 2001
collections resorbed) 14. Dillon WP: Spinal manifestations of intracranial
hypotension. AJNR Am J Neuroradiol. 22(7):1233-4, 2001
• 20-25% persistent leak, chronic headaches
15. Patel MR et al: CT-guided percutaneous fibrin glue therapy
• Generally excellent of cerebrospinal fluid leaks in the spine after surgery. AJR
• Rare: Coma, death from intracranial herniation AmJ Roentgenol. 175(2):443-6,2000
16. O'Carroll CP et al: The syndrome of spontaneous
Treatment intracranial hypotension. Cephalalgia. 19(2):80-7, 1999
• Conservative Rx to restore CSF volume (fluid 17. Atkinson JL et al: Acquired Chiari I malformation
replacement, bed rest) secondary to spontaneous spinal cerebrospinal fluid
• Other leakage and chronic intracranial hypotension syndrome in
o Autologous blood patch seven cases. J Neurosurg. 88(2):237-42, 1998
o Epidural saline infusion 18. Moayeri NN et al: Spinal dural enhancement on MRI
associated with spontaneous intracranial hypotension. J
o Surgery if large dural tear, ruptured diverticulum or
Neurosrug 88: 912-8, 1998
Tarlov cyst 19. Rabin BM et al: Spontaneous intracranial hypotension:
Spinal MR findings. AJNR 19: 1034-9, 1998
20. Vakharia SB et al: Magnetic resonance imaging of
IDIAGNOSTIC CHECKLIST cerebrospinal fluid leak and tamponade effect of blood
patch in postdural puncture headache. Anesth Analg.
Consider 84(3):585-90, 1997
• Intracranial cisternography to look for congenital 21. McCormack BM et al: Pseudomeningocele/CSF fistula in a
defects in middle fossa, or temporal bone patient with lumbar spinal implants treated with epidural
blood patch and a brief course of closed subarachnoid
Image Interpretation Pearls drainage. A case report. Spine. 21(19):2273-6, 1996
• "Sagging" brain, posterior fossa with diffuse dural 22. Maycock NF et al: Post-laminectomy cerebrospinal fluid
fistula treated with epidural blood patch. Spine.
thickening in patient evaluated for headache should 19(19):2223-5, 1994
suggest CSF leak 23. Lee KS et al: Postlaminectomy lumbar pseudomeningocele:
report of four cases. Neurosurgery. 30(1):111-4, 1992
24. Waisman M et al: Postoperative cerebrospinal fluid leakage
ISELECTED REFERENCES after lumbar spine operations. Conservative treatment.
Spine. 16(1):52-3, 1991
1. Kelley GR et al: Sinking brain syndrome: craniotomy can
25. Primeau M et al: Spinal cerebrospinal fluid leak
precipitate brainstem herniation in CSF hypovolemia.
demonstrated by radioisotopic cisternography. Clin Nucl
Neurology. 62(1):157, 2004
Med. 13(10):701-3, 1988
2. Paolini S et al: Intraspinous postlaminectomy
26. Hanakita J et al: Spinal cord compression due to
pseudomeningocele. Eur Spine J. 12(3):325-7, 2003
postoperative cervical pseudomeningocele. Neurosurgery.
3. Luetmer PH et al: DynamiC CT myelography: a technique
17(2):317-9, 1985
for localizing high-flow spinal cerebrospinal fluid leaks.
27. Morris RE et al: Traumatic dural tears: CT diagnosis using
A]NR AmJ Neuroradiol. 24(8):1711-4, 2003
metrizamide. Radiology. 152(2):443-6, 1984
4. Rothrock JF: Low pressure headache and pseudosubdural
28. TeplickJG et al: CT Identification of postlaminectomy
hematomas. Headache. 43(9):1009, 2003
pseudomeningocele. AJR AmJ Roentgenol. 140(6):1203-6,
5. Eross EJ et al: Prolonged neurologic complication and MRI
1983
abnormalities consequent to intracranial hypotension.
29. Findler G et al: Continuous lumbar drainage of
Headache. 43(4):415, 2003
cerebrospinal fluid in neurosurgical patients. Surg Neurol.
6. Schievink WI et al: Recurrent spontaneous spinal
8(6):455-7, 1977
cerebrospinal fluid leak associated with "nude nerve root"
syndrome: case report. Neurosurgery. 53(5):1216-8;
discussion 1218-9, 2003
7. Schievink WI et al: Recurrent spontaneous spinal
cerebrospinal fluid leaks and intracranial hypotension: a
prospective study. J Neurosurg. 99(5):840-2, 2003
8. Whiteley W et al: Spontaneous intracranial hypotension
causing confusion and coma: a headache for the
neurologist and the neurosurgeon. Br J Neurosurg.
17(5):456-8,2003
9. Fujimaki H et al: Cerebrospinal fluid leak demonstrated by
three-dimensional computed tomographic myelography in
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Typical
(Left) Axial T2WI MR shows
sacral nerve sleeve cysts
remodeling canal, loculated
fluid in presacral space along
the course of sacral roots
consistent with CSF leak.
(Right) Sagittal bone CT
reformation with intrathecal
dye shows CSF leak from
subOCCipital site of Chiari
decompression (arrow).
Note markedly
hypertrophied veins anterior
to the cervical cord.
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HARDWARE FAILURE
1
14
lateral radiography flexion (left) and extension (right) Axial bone CT with sagittal reformation in same patient
views in patient with screw bridging type /I odontoid confirms nonunion at the base of odontoid (arrow) with
fracture reveal motion between arch of C7 and the peri-implant lucency (curved arrow),. compatible with
screw. hardware failure.
• Disengaged hook
ITERMINOLOGY • Broken, dislodged rod
Abbreviations and Synonyms • Intervertebral cage/allograft migration
o Pseudoarthrosis
• Implant or metal failure
• Lucency between bone graft and adjacent vertebra
Definitions • Sclerosis and rounding of unfused bones
• Mechanical breakdown or malfunction of hardware o Development or progreSSion of vertebral
malalignment
• Fluoroscopy
IIMAGING FINDINGS o Pseudoarthrosis on flexion/extension views
• 4 mm of translation or > 10° of angular motion
General Features between adjacent vertebrae
• Best diagnostic clue: Fractured or malpositioned • Up to 3 mm of translation considered normal
metallic implant
• Location: Any spinal segment where instrumentations CT Findings
occur • Bone CT
o Peri-implant lucency (suggests loosening)
Radiographic Findings o Over-extension beyond posterior vertebral cortex by
• Radiography cervical bicortical screws
o Cervical spine o Medial cortex penetration by suboptimally placed
• Loose cannulated screw bridging type II odontoid lumbar pedicle screw
fracture o Occult osseous fractures
• Broken, extruded screw o Osseous (non)union
• Fractured, ventrally displaced plate • Pseudoarthrosis
• Dislodged allograft
• Broken, detached posterior cervical wire MR Findings
o Thoracolumbar and lumbosacral spine • MRI generally does not depict hardware location or
• Broken sublaminar or subpars wire integrity well
• Bent, loose, or fractured pedicle screw
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HARDWARE FAILURE
Key Facts
1
Terminology • Plain films excellent in evaluating vertebral 15
• Implant or metal failure alignment, hardware integrity, fusion status
• Mechanical breakdown or malfunction of hardware • MRI to identify soft tissue complications or spinal
cord injury
Imaging Findings
• Best diagnostic clue: Fractured or malpositioned
Pathology
metallic implant • 2-45% reoperation rate for implant failure
• Broken, extruded screw Clinical Issues
• Fractured, ventrally displaced plate • Osseous fusion may be expected even if hardware
• Bent, loose, or fractured pedicle screw fails
• Broken, dislodged rod
• Intervertebral cage/allograft migration Diagnostic Checklist
• Pseudoarthrosis on flexion/extension views • Important not only to evaluate hardware failure but
• 4 mm of translation or > 10° of angular motion also to look for complicating instability or osseous
between adjacent vertebrae fracture
• Peri-implant lucency (suggests loosening)
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HARDWARE FAILURE
1 • Epidemiology
o 2-45% reoperation rate for implant failure Image Interpretation Pearls
16 o Hardware failure in scoliosis surgery • Important not only to evaluate hardware failure but
• 31% with anterior approach also to look for complicating instability or osseous
• 1% with posterior approach fracture
o Unconstrained cervical fusion system • Important to consider original indication for fusion in
• 22-46% failure rate vs. 18% in constrained system suspected implant failure
o Lumbar fusion with unconstrained pedicle screw o Failed fusion for trauma may indicate unsuspected
system ligamentous injury
o Failed fusion for neoplasm may indicate tumor
• 22% failure rate
• 75% due to pseudoarthrosis recurrence or progression
• Associated abnormalities
o Pseudoarthrosis
o Spinal instability ISELECTED REFERENCES
o Osseous fractures 1. Singh SK et al: Occipitocervical reconstruction with the
o Dural laceration Ohio Medical Instruments Loop: results of a multicenter
o Nerve injury evaluation in 30 cases. ] Neurosurg. 98(3 Suppl):239-46,
2003
2. Bagchi K et al: Hardware complications in scoliosis surgery.
ICLINICAL ISSUES 3.
Pediatr Radiol. 32(7):465-75, 2002
Deckey]E et al: Loss of sagittal plane correction after
removal of spinal implants. Spine. 25(19):2453-60, 2000
Presentation 4. Glassman SD et al: The durability of small-diameter rods in
• Most common signs/symptoms lumbar spinal fusion.] Spinal Disord. 13(2):165-7,2000
o May be incidental finding 5. Apfelbaum RI et al: Direct anterior screw fixation for recent
o Other signs/symptoms and remote odontoid fractures. ] Neurosurg 93(2 Suppl):
• Pain 227-36,2000
6. Geiger F et al: Complications of scoliosis surgery in
• Tenderness
children with myelomeningocele. Eur Spine]. 8(1):22-6,
• Weakness 1999
• Paresthesia 7. Wetzel FT et al: Hardware failure in an unconstrained
• Radiculopathy lumbar pedicle screw system. A 2-year follow-up study.
• Clinical profile Spine. 24(11):1138-43, 1999
o Hardware failure in early post-operative period 8. Masferrer Ret al: Efficacy of pedicle screw fixation in the
• Indication of continued gross spinal instability treatment of spinal instability and failed back surgery: a
o Development or progression of neurologic 5-year review.] Neurosurg. 89(3):371-7, 1998
symptoms 9. Lowery GL et al: The significance of hardware failure in
anterior cervical plate fixation. Patients with 2- to 7-year
• Osseous nonunion and/or hardware failure should
follow-up. Spine. 15;23(2):181-6; discussion 186-7, 1998
be suspected 10. Wellman B] et al: Complications of posterior articular mass
plate fixation of the subaxial cervical spine in 43
Demographics consecutive patients. Spine. 23(2):193-200, 1998
• Age: Mostly adults 11. Shapiro SA et al: Spinal instrumentation with a low
• Gender: No sex predilection complication rate. Surg Neurol. 48(6):566-74, 1997
12. Paramore CG et al: Radiographic and clinical follow-up
Natural History & Prognosis review of Caspar plates in 49 patients.] Neurosurg.
• Osseous fusion may be expected even if hardware fails 84(6):957-61, 1996
o Broken hardware need not be removed if spine 13. Blumenthal S et al: Complications of the Wiltse Pedicle
clinically, radiographically stable Screw Fixation System. Spine. 18(13):1867-71, 1993
• Fibrous union without radiographic osseous fusion 14. Slone RM et al: Spinal fixation. Part 3. Complications of
may be satisfactory spinal instrumentation. Radiographies. 13(4):797-816,
1993
o Best demonstrated on dynamic flexion extension
15. Guidera K] et al: Cotrel-Dubousset instrumentation. Results
views in 52 patients. Spine. 18(4):427-31, 1993
• Repeat surgical fusion may be necessary 16. McLain RF et al: Early failure of short-segment pedicle
o Especially if hardware failure occurs early instrumentation for thoracolumbar fractures. A
preliminary report.] Bone]oint Surg Am. 75(2):162-7,
Treatment 1993
• Conservative observation 17. Wilkinson RH et al: Radiographic evaluation of the spine
• Surgical revision to prevent nonunion and instability after surgical correction of scoliosis. A]R Am] Roentgenol.
133(4):703-9,1979
IDIAGNOSTIC CHECKLIST
Consider
• Consulting references describing expected hardware
appearance
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HARDWARE FAILURE
IIMAGE GALLERY 1
17
Typical
(Left) Anteroposterior
radiography in a patient with
CO-2 fusion shows
asymmetric screw
placement. (Right) Lateral
radiography demonstrates
dorsal extrusion of left
transarticular screw from CI
and C2 lateral masses.
Typical
(Left) Axial bone CT through
lumbar spine demonstrates
subtle lucency medial to the
left pedicle screw. A fracture
is present at the base of left
pedicle due to failed
hardware. (Right) Axial bone
CT with sagittal reformation
through lumbosacral spine
shows ventral extrusion of an
intervertebral allograft at
L5-51.
(Left) Anteroposterior
radiography of lumbosacral
spine in a patient with fusion
from L4-51 shows subtle
discontinuity in the right rod
(arrow). (Right) Lateral
radiography confirms the rod
fracture (arrow) with slight
overlap oUts ends.
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ACCELERATED DEGENERATION
1
18
Sagittal graphic shows solid L4-5 interbody fusion and Sagittal TI WI MR shows patient post C4-TI fusion with
laminectomy. Severe disc degeneration at 13-4 with anterior plate/screws. There is herniation above fusion
spondylolisthesis, loss of disc height, osteophytes and site at C3-4 (arrow) with impingement upon cord, and
central stenosis. increased kyphosis.
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ACCELERATED DEGENERATION
Key Facts
1
Terminology • Increased mobility in these remaining mobile 19
• Spinal "transitional" degenerative syndrome, segments is hypothesized to cause accelerated
accelerated segmental degeneration degenerative pathologic changes
• Accelerated degeneration of disc space/facets at • Segmental motion above or below fusion increases
level(s) adjacent to surgical fusion • Intradiscal pressure above or below fusion increases
• Lower lumbar fusions show more stress on adjacent
Imaging Findings levels than higher lumbar fusions
• Also occurs adjacent to congenital segmentation
anomalies
Clinical Issues
• May be asymptomatic
Pathology • Mechanical pain
• Produced by aberrant biophysical stresses from • Radicular or myelopathic symptoms referable to
altered normal spinal motion level(s) above or below a surgical fusion
• Wolff law; living tissue responds to chronic changes • Difficult to distinguish natural history of disc
in stresses & strains degeneration from accelerated degeneration
following fusion
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ACCELERATED DEGENERATION
1 o Increased mobility in these remaining mobile
I DIAGNOSTIC CHECKLIST
segments is hypothesized to cause accelerated
20 degenerative pathologic changes Consider
• Segmental motion above or below fusion increases • Pseudoarthrosis if extensive signal abnormality
• Intradiscal pressure above or below fusion involVing anterior and posterior elements in a
increases horizontal fashion
o More common with multilevel fusion, but also seen
folloWing single level fusion Image Interpretation Pearls
• Lower lumbar fusions show more stress on • Presence of prior surgical fusion, instrumentation
adjacent levels than higher lumbar fusions • Look for transitional lumbosacral vertebral body
• Epidemiology
o More common after surgical fusion than following
decompression only ISELECTED REFERENCES
o Bertolotti syndrome refers to association of back 1. Polikeit A et al: Factors influencing stresses in the lumbar
pain with lumbosacral transitional vertebrae spine after the insertion of intervertebral cages: finite
• Disc bulge/herniation 9x more common at element analysis. Eur Spine]. 12(4):413-20, 2003
interspace immediately above transitional vertebra 2. Eck]C et al: Biomechanical study on the effect of cervical
than at any other level spine fusion on adjacent-level intradiscal pressure and
segmental motion. Spine. 27(22):2431-4, 2002
Gross Pathologic & Surgical Features 3. Gertzbein SD et al: Disc herniation after lumbar fusion.
• Disc space height loss, disc bulge or herniation, central Spine. 27(16):E373-6, 2002
stenosis, facet osteoarthritic change 4. Iseda T et al: Serial changes in signal intensities of the
adjacent discs on T2-weighted sagittal images after surgical
Microscopic Features treatment of cervical spondylosis: anterior interbody fusion
• Disc dehydration, fissuring, anular disruption, disc versus expansive laminoplasty. Acta Neurochir (Wien).
143(7):707-10,2001
protrusion or extrusion
5. Miyakoshi N et al: Outcome of one-level posterior lumbar
Staging, Grading or Classification Criteria interbody fusion for spondylolisthesis and postoperative
intervertebral disc degeneration adjacent to the fusion.
• Spondylolisthesis may be graded I-IV Spine. 25(14):1837-42, 2000
6. Maiman D] et al: Biomechanical effect of anterior cervical
spine fusion on adjacent segments. Biomed Mater Eng.
ICLINICAL ISSUES 9(1):27-38, 1999
7. Farcy]P: Review of surgical cases which have deteriorated
Presentation over time. Bull Acad Nat! Med 183(4): 775-82, 1999
• Most common signs/symptoms 8. Eck]C et al: Adjacent-segment degeneration after lumbar
o May be asymptomatic fusion: a review of clinical, biomechanical, and radiologic
o Mechanical pain studies. Am] Orthop 28(6): 336-40,1999
9. Hambly MF et al: The transition zone above a lumbosacral
o Radicular or myelopathic symptoms referable to
fusion. Spine. 23(16):1785-92, 1998
level(s) above or below a surgical fusion 10. Balderston RA et al: Magnetic resonance imaging analysis
o Herniation above site of fusion < 2% of lumbar disc changes below scoliosis fusions. A
• Clinical profile: Prior surgical fusion, with new and prospective study. Spine. 23(1):54-8; discussion 59, 1998
progressing pain in different distribution than 11. Chow DH et al: Effects of short anterior lumbar interbody
pre-operative pain fusion on biomechanics of neighboring unfused segments.
Spine. 21(5):549-55, 1996
Demographics 12. Wu W et al: Degenerative changes folloWing anterior
• Age: Adult cervical discectomy and fusion evaluated by fast spin-echo
• Gender: M = F MR imaging. Acta Radiol 37(5): 614-7, 1996
13. Penta M et al: Magnetic resonance imaging assessment of
Natural History & Prognosis disc degeneration 10 years after anterior lumbar interbody
• Difficult to distinguish natural history of disc fusion. Spine. 20(6):743-7, 1995
14. Kim YE et al: Effect of disc degeneration at one level on the
degeneration from accelerated degeneration following
adjacent level in axial mode. Spine. 16(3):331-5, 1991
fusion 15. Dennis S et al: Comparison of disc space heights after
• Progressively worsens anterior lumbar interbody fusion. Spine. 14(8):876-8, 1989
o Highly variable course 16. Elster AD: Bertolotti's syndrome revisited. Transitional
• Decreased signal within adjacent discs on T2WI may vertebrae of the lumbar spine. Spine. 14(12):1373-7, 1989
occur as early as 12 months following fusion 17. Lee CK: Accelerated degeneration of the segment adjacent
• Below scoliosis fusion chance of herniation up to 34%, to a lumbar fusion. Spine. 13(3):375-7, 1988
chance of disc signal abnormality 50% 18. Kurowski P et al: The relationship of degeneration of the
intervertebral disc to mechanical loading conditions on
Treatment lumbar vertebrae. Spine. 11(7):726-31, 1986
19. Lin PM et al: Posterior lumbar interbody fusion. Clin
• Surgical decompression and fusion at adjacent
Orthop. (180):154-68, 1983
symptomatic levels
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ACCELERATED DEGENERATION
IIMAGE GALLERY 1
21
Typical
(Left) Sagittal T7 WI M R
shows patient post C3-5
fusion with graft. There is
degeneration of C5-6 with
central stenosis (arrow).
Congenital fusion of C6-7
further increases
biomechanical stress on
C5-6. (Right) Sagittal T2WI
MR shows patient post
fusion ofC3-5. Degeneration
at C5-6 with central stenosis
and narrowed subarachnoid
space. Hyperintensity within
cervical cord is due to
myelomalacia (arrow).
Typical
(Left) Anteroposterior
myelography (L side) shows
block to contrast at L1-2
(arrow) at upper margin of
posterior fusion. Lateral view
(R side) shows contrast
block, & severe L1-2
degeneration (open arrow).
(Right) Sagittal T2WI MR (L
side), and T7WI C+ (R side)
with lumbar pedicle screws
giving artifact. There is
peripheral enhancing disc
extrusion (arrows) below
fusion site with thecal sac
compression.
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POST-LAMINECTOMY SPONDYLOLISTHESIS
1
22
Sagittal NEeT reformat shows increased kyphotic Sagittal TlWI MR shows upper thoracic body collapse,
deformity, pseudoarthrosis at thoracolumbar junction severe retrolisthesis with was progressive following
following multilevel laminectomy for intramedullary multilevel laminectomy. There is a large post-operative
ependymoma. pseudomeningocele.
Definitions CT Findings
• Post-operative loss of spine motion segment stiffness, • NECT: Nonspecific post-operative changes with
where applied force produces greater displacement malalignment
than normal, with pain/deformity MR Findings
• T1 WI: Anterolisthesis, retrolisthesis, lateral translation
• T2WI: Loss of disc signal, disc space height in adult
IIMAGING FINDINGS with disc degeneration
General Features • T1 C+: In adult, nonspecific enhancement of disc due
to degenerative disc disease
• Best diagnostic clue: New post-operative deformity
which increases with motion, and increases over time Imaging Recommendations
• Location: Any spinal motion segment, most common • Best imaging tool: Flexion/extension plain films
involving cervical spine in children
• Size: Displacement may vary from few mm to width of
vertebral body IDIFFERENTIAL DIAGNOSIS
• Morphology: Displacement of vertebral body with
respect to adjacent body Infection
• Endplate destruction, disc T2 hyperintensity
Radiographic Findings
• Radiography Tumor
o Various parameters used for degenerative instability • Enhancing soft tissue mass destroying vertebral
by plain films body/posterior elements
• Dynamic slip> 3 mm in flexion/extension
• Static slip of 4.5 mm or greater
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POST-LAMINECTOMY SPONDYLOLISTHESIS
Key Facts
1
Terminology Pathology 23
• Post-operative loss of spine motion segment stiffness, • Predisposing factors for post-operative instability
where applied force produces greater displacement • Children> adults
than normal, with pain/deformity • No preoperative lordosis in cervical spine
• Surgical disruption of facets
Imaging Findings • C2 laminectomy ± increased risk
• Best diagnostic clue: New post-operative deformity • More laminectomy levels ± increased risk
which increases with motion, and increases over time
ICLINICAL ISSUES
Presentation
• Most common signs/symptoms: Neck, back pain with
later appearance of new neurologic symptoms
• Clinical profile: Early good operative result with
progressive axial spine pain, new neurologic
symptoms
Demographics
• Age: Children and adults
• Gender: No gender predilection (Left) Sagittal T1 WI MR shows spondylolisthesis of L4 on L5 following
laminectomy. with disc degeneration. (Right) Sagittal myelography
Natural History & Prognosis shows anterolisthesis of vertebral body at upper margin of lumbar
• Generally progressive fusion with accelerated disc degeneration.
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PERIDURAL FIBROSIS
1
24
Axial T1WI MR in patient following posterior lumbar Axial T1 C+ MR in same patient following posterior
interbody fusion (PUF) shows extensive low signal lumbar interbody fusion (PUF) shows diffuse
surrounding thecal sac (arrows). Artifact present in disc enhancement of extensive epidural fibrosis with minimal
space from fusion cages. thecal sac deformity.
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PERIDURAL FIBROSIS
Key Facts
1
Terminology • Extent of fibrosis possibly related to extent of surgical 25
• Scar formation within epidural space after lumbar dissection
surgery • Scar tissue compresses, irritates, puts abnormal
• Part of failed back surgery syndrome (FBSS) traction on nerve roots
• Estimated 1 million spinal operations worldwide each
Top Differential Diagnoses year
• Recurrent disc herniation • Peridural fibrosis implicated in 3-5% of cases as cause
• Epidural abscess/phlegmon of FBSS
• Pseudomeningocele
• Post-operative hemorrhage
Clinical Issues
• Arachnoiditis • Low back or radicular pain
• Numbness
Pathology • Weakness
• Post-operative scarring is part of normal reparative • Worse reoperative success rates for only epidural
mechanism fibrosis found at surgery, without disc herniation
• May be asymptomatic; contribution to clinical
symptoms controversial
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PERIDURAL FIBROSIS
1 Microscopic Features I SELECTED REFERENCES
26 • Invasion of post-operative hematoma by dense fibrous 1. Gerszten PC et al: Low-dose radiotherapy for the inhibition
tissue originating within deep surface of paravertebral of peridural fibrosis after reexploratory nerve root
musculature decompression for postlaminectomy syndrome. J
• Fibrous tissue can extend to thecal sac, adhere to dura Neurosurg. 99(3 Suppl):271-7, 2003
and roots 2. Van Goethem JW et al: Review article: MRI of the
postoperative lumbar spine. Neuroradiology. 44(9):723-39,
2002
3. Anderson SR: A rationale for the treatment algorithm of
ICLINICAL ISSUES failed back surgery syndrome. Curr Rev Pain. 4(5):395-406,
2000
Presentation 4. Anderson VC et al: Failed back surgery syndrome. Curr Rev
• Most common signs/symptoms Pain. 4(2):105-11, 2000
o Low back or radicular pain 5. Coskun E et al: Relationships between epidural fibrosis,
o Numbness pain, disability, and psychological factors after lumbar disc
o Weakness surgery. Eur Spine J. 9(3):218-23,2000
6. Pearce JM: Aspects of the failed back syndrome: role of
o 10% rate of recurrent back/radicular pain 6 months
litigation. Spinal Cord. 38(2):63-70, 2000
after discectomy 7. Samy Abdou M et al: Epidural fibrosis and the failed back
o Causes of recurrent pain surgery syndrome: history and physical findings. Neurol
• Recurrent disc herniation Res. 21 Suppl1:S5-8, 1999
• New herniation at another level 8. Devulder J et al: Spinal cord stimulation: a valuable
• Peridural fibrosis treatment for chronic failed back surgery patients. J Pain
• Clinical profile: Adult with gradual onset low back Symptom Manage. 13(5):296-301, 1997
pain following initially successful disc surgery 9. Robertson JT: Role of peridural fibrosis in the failed back: a
review. Eur Spine J. 5 Suppl 1:S2-6, 1996
Demographics 10. Van Goethem JW et al: MRI after successful lumbar
discectomy. Neuroradiology. 38 Suppl1:S90-6, 1996
• Age: Adult 11. Fritsch EW et al: The failed back surgery syndrome:
• Gender: M = F reasons, intraoperative findings, and long-term results: a
Natural History & Prognosis report of 182 operative treatments. Spine. 21(5):626-33,
1996
• Chronic, indolent course of FBSS 12. Ross JS et al: Association between peridural scar and
• 30-35% success rate for repeat surgery (range 12-100%) recurrent radicular pain after lumbar discectomy: Magnetic
• Worse reoperative success rates for only epidural resonance evaluation. Neurosurgery 38: 855-61, 1996
fibrosis found at surgery, without disc herniation 13. Georgy BA et al: Fat-suppression contrast-enhanced MRI in
• 50-70% success rate for spinal cord stimulation the failed back surgery syndrome: a prospective study.
Neuroradiology. 37(1):51-7, 1995
Treatment 14. Kim SS et al: Revision surgery for failed back surgery
• Symptomatic treatment with physical therapy, wide syndrome. Spine. 17(8):957-60, 1992
variety of pain medication 15. North RB et al: Failed back surgery syndrome: 5-year
follow-up in 102 patients undergoing repeated operation.
• Periradicular injection of corticosteroids and local
Neurosurgery. 28(5):685-90; discussion 690-1, 1991
anesthetics 16. Ross JS et al: MR imaging of the postoperative lumbar
• Spinal cord stimulation by implanted electrodes spine: assessment with gadopentetate dimeglumine. AJR
• Surgical lysis of scar tissue rarely performed AmJ Roentgenol. 155(4):867-72, 1990
17. Bundschuh CV et al: Distinguishing between scar and
recurrent herniated disk in postoperative patients: value of
I DIAGNOSTIC CHECKLIST contrast-enhanced CT and MR imaging. AJNR Am J
Neuroradiol. 11(5):949-58, 1990
Consider 18. Ross JS et al: MR imaging of the postoperative lumbar
spine: assessment with gadopentetate dimeglumine. AJNR
• Identification of only epidural fibrosis in FBSS patient
AmJ Neuroradiol. 11(4):771-6, 1990
is contraindication to reoperation, yields poor 19. Djukic S et al: Magnetic resonance imaging of the
reoperative result postoperative lumbar spine. Radiol Clin North Am.
• Best reoperative result in patients with herniation at 28(2):341-60, 1990
new level 20. Hueftle MG et al: Lumbar spine: postoperative MR imaging
• Intermediate operative success results in patients with with Gd-DTPA. Radiology 167: 817-24, 1988
new herniation at previously operated level, better
than scar only, worse than disc at non-operative level
Image Interpretation Pearls
• Classic imaging appearance: Enhancing epidural soft
tissue surrounding the typically enlarged nerve root
• Primary function of imaging is to exclude recurrent
disc herniation or other cause of FBSS (e.g.,
conus/filum tumor)
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PERIDURAL FIBROSIS
I IMAGE GALLERY 1
27
Typical
(Left) Axial TI C+ MR shows
left hemilaminectomy defect
(open arrow). Small amount
of epidural fibrosis
surrounding left 57 root
diffusely enhances, as does
operative defect in posterior
anulus (arrow). (Right) Axial
TlWI MR shows right
laminectomy defect, and
extensive epidural fibrosis
surrounding the right lateral
and dorsal aspect of thecal
sac, and exiting root
(arrows).
Typical
(Left) Axial T7 C+ MR shows
right hemilaminectomy
defect, and diffuse
enhancement of epidural
fibrosis surrounding the right
lateral aspect of thecal sac.
(Right) Axial TI C+ MR
shows right
hemilaminectomy defect,
and enlargement of exiting
root sleeve on right, with
peripheral enhancement due
to scar cicatrization (arrow).
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PSEUDOMENINGOCELE
1
28
Sagittal graphic shows changes of L4-5 interbody fusion Axial CT post myelography shows contrast collecuon at
with cage, Large well-defined CSF collection extends laminectomy/fusion site in posterior soft tissues
into dorsal soft tissue from thecal sac, from site of (arrows), There is incorporation of hardware into the
operative dural tear, collecuon,
,,' ,
.~ '. ' . " "
.
..
.<.t •
, ~'~;~
: .~
Epidural Abscess Hemorrhage Osteosarcoma Rhabdomyolysis
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PSEUDOMENINGOCELE
Key Facts
1
Terminology Top Differential Diagnoses 29
• Dural dehiscence, pseudocyst • Paraspinous abscess
• Spinal cyst contiguous with thecal sac, not lined with • post-operative hematoma
meninges • Soft tissue tumor
• Rhabdomyolysis
Imaging Findings
• Best diagnostic clue: CSF filled spinal axis cyst with Pathology
supportive post-operative or post-traumatic ancillary • Epidemiology: 0.19 to 2% of patients undergoing
findings lumbar laminectomies
• Most common location is dorsal to thecal sac at • Reactive fibrous tissue forms cyst capsule
lumbar laminectomy site
• Fat-saturated T2WI best sequence to demonstrate Clinical Issues
pseudomeningocele and to localize dural • Commonly asymptomatic
communication • Nonspecific back pain
• Sagittal plane useful for diagnosis and to tailor thin • Occasionally extends superficially, palpable within
section axial imaging for definitive localization subcutaneous tissues
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PSEUDOMENINGOCELE
1 • Iatrogenic dural laceration with CSF leak
• May also see after dural graft following spinal
• May need to widen window on MR studies to visualize
subcutaneous component due to surface coil signal
30 tumor resection inhomogeneity
• Epidemiology: 0.19 to 2% of patients undergoing
lumbar laminectomies
• Associated abnormalities: May rarely show peripheral ISELECTED REFERENCES
calcification/ossification 1. Phillips CD et al: Depiction of a postoperative
Gross Pathologic & Surgical Features pseudomeningocele with digital subtraction myelography.
AJNR AmJ Neuroradiol. 23(2):337-8, 2002
• CSF-containing collection 2. Elbiaadi-Aziz N et al: Cerebrospinal fluid leak treated by
aspiration and epidural blood patch under computed
Microscopic Features tomography guidance. Reg Anesth Pain Med. 26(4):363-7,
• No true meningeal covering 2001
• Reactive fibrous tissue forms cyst capsule 3. Bosacco SJ et al: Evaluation and treatment of dural tears in
lumbar spine surgery: a review. Clin Orthop 389: 238-47,
2001
ICLINICALISSUES 4. Jinkins JR et al: The postsurgical lumbosacral spine.
Magnetic resonance imaging evaluation follOWing
Presentation intervertebral disk surgery, surgical decompression,
intervertebral bony fusion, and spinal instrumentation.
• Most common signs/symptoms Radiol din North Am 39(1): 1-29, 2001
o Commonly asymptomatiC 5. Ross JS: Magnetic resonance imaging of the postoperative
o Nonspecific back pain spine. Semin Musculoskelet Radiol 4(3): 281-91, 2000
o Headache if associated with CSF hypotension 6. Stambough JL et al: Subarachnoid drainage of an
o Occasionally extends superficially, palpable within established or chronic pseudomeningocele. J Spinal Disord.
subcutaneous tissues 13(1):39-41,2000
o Meningitis/abscess if extends to/communicates with 7. Saunders RL et al: Four-level cervical corpectomy. Spine.
skin surface 23(22):2455-61, 1998
8. McCormack BM et al: Pseudomeningocele/CSF fistula in a
• Clinical profile: Adult patient with palpable
patient with lumbar spinal implants treated with epidural
subcutaneous mass overlying lumbar laminectomy site blood patch and a brief course of dosed subarachnoid
drainage. A case report. Spine. 21(19):2273-6, 1996
Demographics
9. Hosono N et al: Postoperative cervical pseudomeningocele
• Age: Adult or children with herniation of the spinal cord. Spine. 20(19):2147-50,
• Gender: M = F 1995
10. Aldrete JA et al: Postlaminectomy pseudomeningocele. An
Natural History & Prognosis unsuspected cause of low back pain. Reg Anesth.
• May be asymptomatic 20(1):75-9, 1995
• post-operative defects may close spontaneously if 11. Shapiro SA et al: Closed continuous drainage of
small, but can require treatment cerebrospinal fluid via a lumbar subarachnoid catheter for
• Cervical root avulsion with pseudomeningocele treatment or prevention of cranial/spinal cerebrospinal
usually leaves permanent neurological deficits fluid fistula. Neurosurgery. 30(2):241-5, 1992
12. Lee KS et al: Postlaminectomy lumbar pseudomeningocele:
Treatment report of four cases. Neurosurgery. 30(1):111-4, 1992
13. Murayama S et al: Magnetic resonance imaging of
• Closure of underlying dural defect when possible
post-surgical pseudomeningocele. Comput Med Imaging
o Surgical repair for large defects Graph. 13(4):335-9, 1989
o Subarachnoid catheter drainage occasionally 14. Tuvia J et al: Visualization of a pseudomeningocele on
successful isotope cisternography. Clin Nud Med. 13(12):889-91,
o Blood patch may be effective for small defects 1988 '
15. Schumacher HW et al: Pseudomeningocele of the lumbar
spine. Surg Neurol. 29(1):77-8, 1988
IDIAGNOSTIC CHECKLIST 16. TeplickJG et al: CT Identification of postlaminectomy
pseudomeningocele. AJR AmJ Roentgenol. 140(6):1203-6,
Consider 1983
17. Helle TL et al: Postoperative cervical pseudomeningocele as
• Relationship of post-operative pseudomeningocele to a cause of delayed myelopathy. Neurosurgery. 9(3):314-6,
patients pain difficult 1981
• Causes of pain related to pseudomeningocele include 18. Eismont FJ et al: Treatment of dural tears associated with
o Tension of cyst against nerve roots spinal surgery. J Bone Joint Surg Am. 63(7):1132-6, 1981
o Entrapment of nerve roots
o Periradicular fibrosis
Image Interpretation Pearls
• Classic imaging appearance: CSF signal/density
collection contiguous with thecal sac in correct
clinical context
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PSEUDOMENINGOCELE
IIMAGE GALLERY 1
31
Typical
(Left) Axial T1 WI M R shows
ovoid CSF signal collection
within dorsal soft tissues at
prior laminectomy site
(arrow). There is a left
pedicle screw causing
artifact (open arrow). (Right)
Axial T2WI MR shows large
hyperintense fluid collection
within dorsal right
hemilaminectomy site
extending into subcutaneous
tissues.
Typical
(Left) Sagittal T2WI MR
shows hyperintense fluid
collection at L4/ L5 levels.
Focal low signal within
collection is flow dephasing
from CSF movement into
collection at dural tear
(arrow). (Right) Axial T2WI
MR shows hyperintense fluid
collection within dorsal soft
tissues at laminectomy site.
There are scattered pieces of
bone graft material within
the fluid collection (arrows).
Typical
(Left) Sagittal T1WI MR
shows large
pseudomeningocele
extending over the L2 to L5
levels with multiple
loculations. Note the
subcutaneous extension at
several levels (arrows).
(Right) Sagittal T2WI MR
shows extensive
hyperintense dorsal
pseudomeningocele in this
patient following multilevel
lumbar laminectomy. There
are multiple loculations and
extension into subcutaneous
tissues.
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BONE GRAFT COMPLICATIONS
1
32
Sagittal T7 WI M R shows interbody fusions spanning Sagittal T2WI MR shows 3 level interbody fusion. Three
C3-4 through C5-6. Craft material is displaced levels of grafts are placed posteriorly with effacement of
posteriorly at all three levels abutting ventral cord with thecal sac and cord (arrows). Note retropharyngeal
compression (arrows). hematoma (open arrow).
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Key Facts
1
Terminology Pathology 33
• Synonyms: Graft migration, graft displacement, graft • Graft migration occurs in 7% of cases after anterior
extrusion cervical corpectomy
• 2 level corpectomy and fusion; 3 level (50%)
Top Differential Diagnoses • Graft migration rate 1 with more levels of fusion
• Abscess • Graft migration rate 1 with fusion ending at C7
• Hemorrhage
• Infarction
• Pseudoarthrosis
ICLINICAL ISSUES
Presentation
• Most common signs/symptoms (Left) Sagittal T7WI MR shows pseudoarthrosis through L5-S1 level
o May be asymptomatiC, with only radiographic with linear low signal from disc level through posterior elements
abnormality (arrows). Sold fusion at L4-5. (Right) Sagittal T2WI MR in patient with
o New post-operative pain/focal deficit post-operative infection shows collapse of graft at C5-6 with kyphosis
o Rare respiratory distress and subluxation (arrow), with cord compression. Note prevertebral
edema (open arrow).
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RECURRENT VERTEBRAL DISC HERNIATION
1
34
Axial graphic of lumbar spine shows left laminectomy Axial T2WI MR shows right sided recurrent extrusion
. defect. Large right central herniation is present (arrow) as focal low signal mass displacing right 51 root
compressing thecal sac & roots adjacent to site of prior & right lateral margin of thecal sac. Note normal left 51
discectomy root (open arrow).
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IDIAGNOSTIC CHECKLIST
Consider
• Best reoperative result in patients with herniation at
new level away from operation site
• Intermediate operative success results in patients with
new herniation at previously operated level, better
than scar only, worse than disc at non-operative level
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RECURRENT VERTEBRAL DISC HERNIATION
IIMAGE GAllERY 1
37
Typical
(Left) Axial T1 WI M R shows
right sided recurrent
herniation as focal epidural
mass of intermediate signal
with compression of thecal
sac. Exiting right S 1 root is
obscured by the herniation.
(Right) Axial T1 C+ MR
shows typical central lack of
enhancement of herniation
(arrow), with peripheral
enhancement of granulation
tissue (open arrow).
(Left) Sagittal T1 C+ MR
shows recurrent disc
extrusion as centrally
non enhancing mass with
peripheral enhancing
granulation tissue migrating
superiorly from L4-5 level
(arrow). (Right) Sagittal
T2WI MR shows large
recurrent extrusion (free
fragment) migrating
cephalad from L4-5 parent
disc level (arrow). Note
posterior laminectomy defect
(open arrow).
Variant
(Left) Sagittal T1 C+ MR
shows recurrent
nonenhancing herniation
mixed with enhancing
fibrosis at L4-5 (arrow).
Severe multilevel disc
degeneration is present with
type /I endplates. (Right)
Sagittal T2WI MR shows
large recurrent L4-5 extrusion
effacing thecal sac, and
migrating inferiorly behind
L5 body.
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POST-OPERATIVE INFECTION
1
38
Sagittal T1 C+ MR demonstrates a large abscess Sagittal STIR MR shows a large abscess posteriorly with
posteriorly with an epidural component (open arrows) an epidural component (open white arrows) as well as
as well as disc enhancement (arrow). fluid signal in an adjacent disc (white arrow).
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POST-OPERATIVE INFECTION
Key Facts
1
Terminology • Local extension infection 39
• Infectious sequelae following operative procedures • Neoplasm
o Phlegmon: Paravertebral soft tissue mass o Negative predictive value, sensitivity, specificity, &
(intermediate signal intensity) accuracy for diagnosing post-operative spine
o Abscess: Hypointense collections within phlegmon infection are 100%, 100%,81%, & 86% respectively
• T2WI • WBC Scan
o Edema: Hyperintense marrow o Often useless 2° to high uptake of labeled leukocytes
o Discitis: Disc hyperintensity or fluid intensity with in normal hematopoietically active bone marrow
loss of disc height • 99mTc-ciprofloxacin SPECT
o Facetitis: Hyperintense facet fluid collection o Sensitive for post-operative spine infections
o Phlegmon: Hyperintense paravertebral soft tissue o SPECT sensitivity much higher than planar imaging
o Abscess: Hyperintense collections within phlegmon o Specificity is limited, especially in recently operated
o Arachnoiditis: Nerve root clumping, adherence to patients « 6 months)
thecal sac ("empty sac sign")
• STIR: Best to visualize hyperintense marrow edema Imaging Recommendations
• Tl C+ • Best imaging tool: MRI C+; consider PET in presence of
o Discitis: Disc enhancement hardware susceptibility
o Facetitis: Facet enhancement • Protocol advice
o Phlegmon: Enhancing soft tissue o STIR best images marrow edema
o Abscess: Fluid within enhancing phlegmon o Fat suppressed techniques i infection conspicuity
o Osteomyelitis: Marrow enhancement
o Arachnoiditis: Nerve root clumping & enhancement
• Chronic MRI findings IDIFFERENTIAL DIAGNOSIS
o Anterior wedging of affected vertebrae with fusion
across narrowed/abliterated disc space
Hematogenous-seeded infection
o Normal marrow fat destroyed without restoration • Most often bacterial (usually Staphylococcus aureus)
• MRI may be non-diagnostic in the presence of • IV drug abusers at higher risk
hardware implanted during causative procedure • Often single level or skips levels
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POST-OPERATIVE INFECTION
1 o Most common etiologic agent is Staph. aureus o Possible use of closed suction-irrigation system
o Three possible causes of a delayed infection o Intravenous organism-specific antibiotic treatment
40 • Intra-operative seeding & spinal immobilization
• Metal fretting causing a sterile inflammatory • Prophylaxis
response o One pre-operative administration of Cefotiam has
• Stimulating low-virulent organisms to fester with been shown effective for I rate of post-operative
hematogenous seeding wound infections in lumbar disc surgery
o Risk factors for spinal surgical site infection: • Rate of infection 0.2 % after antibiotic
Increasing age, post-operative incontinence, administration vs. 2.8 % without prophylaxis
posterior approach, surgery for tumor resection, o Intraoperative antibiotic prophylaxis has been
morbid obesity, increased blood loss, longer advocated by some
operative times, number of operated levels • Scoliosis surgery
o Scoliosis surgery o Lower thoracic & thoracolumbar: Modified extended
• Closure of thoracolumbar wounds after scoliosis latissimus dorsi myocutaneous flap to close and
surgery often difficult due to tautness & lack of supply blood to wounds
usable tissue o Lumbosacral: Addition of gluteus maximus muscle
• Resulting dead space containing metallic fixation flap to obtain covering caudal extent
devices is predisposed to infection o Allows for wound closure & drastically I the risk of
• Epidemiology post-operative infection
o Overall uncommon; 0.2-3% spinal surgeries o Effective for closure & increased blood supply to
o Postprocedural discitis: Incidence ~ 0.2% already infected wounds
o Scoliosis surgery: Post-operative infection due to lack
of vascularized tissue & presence of metallic
hardware near the wound is common I DIAGNOSTIC CHECKLIST
Consider
!ClINICAL ISSUES • Consider PET as an alternative to MRI
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POST-OPERATIVE INFECTION
IIMAGE GAllERY 1
41
Typical
(Left) Axial bone CT reveals
lucency around infected
right screw prior to hardware
removal. (Right) Axial T2WI
MR demonstrates fluid
collected (arrows) around
infected right screw prior to
hardware removal. Note F5E
technique minimizes
susceptibility yielding
diagnostic scan.
Typical
(Left) Axial fat-saturation Tl
C+ MR reveals facetitis &
widespread infection
complicating right L5/S 1
right facet injection in
rheumatoid patient. (Right)
Axial bone CT in same
patient demonstrates
erosions from facetitis
complicating right L5/S1
right facet injection in
rheumatoid patient.
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Sagittal T2WI MR shows C4-Cl strut graft placement + Sagittal TlWI MR shows strut graft placed from C4-Cl
anterior plate for spondylosis (open arrow). Cord with anterior plate. Cord margin is indistinct from near
compression results from acute epidural hematoma isointense acute epidural hemorrhage (arrows).
(arrows) dorsal to operative site.
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(Left) Sagittal TI WI M R
shows multilevel carpectomy
+ strut graft placed for
spond0os6. Cord shows
fusiform enlargement with
slight TI hypointensity
(arrows) from acute
infarction. (Right) Sagittal
T2WI MR shows anterior
corpectomy + strut graft,
posterior multilevel
laminectomy. Cord shows
fusiform enlargement and T2
hyperintensity (arrow) from
acute infarction.
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Axial graphic of lumbar spine shows post-operative Axial T7WI MR shows laminectomy at LS-51. There is
change with bilateral pedicle screws and left peripheral clumping of roots in caudal thecal sac due to
laminectomy defect. There is clumping of nerve roots arachnoiditis (arrow), note droplet of T7 hyperintense
reflecting arachnoiditis. Pantopaque (open arrow).
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Treatment
• Conservative measures
o Physiotherapy, back strengthening exercises
o Transcutaneous electrical nerve stimulation
o Epidural steroids
• Spinal cord stimulators
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FAILED BACK SURGERY SYNDROME
IIMAGE GALLERY 1
49
Typical
(Left) Axial T1 C+ MR shows
abnormal central clumping
of cauda equina at L3 level,
reFlecting arachnoiditis.
There is no signiFicant
intradural enhancement.
(Right) Sagittal T1 C+ MR
shows central clumping of
intrathecal roots into a
central rope-like mass,
simulating low lying cord.
Note multilevel laminectomy
defect, degenerative disc
space enhancement.
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METAL ARTIFACT
1
50
Axial T7WI MR shows metal artHact from bilateral Axial T7WI MR shows metal artifact from pedicle
pedicle screws. There is typical spatial mismapping with screws. Frequency encode direction is oriented AP;
peripheral high signal (arrows). Frequency encoding minimizing artifact in transverse direction, and
direction is right to left. spreading artifact along AP direction.
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METAL ARTIFACT
Key Facts
1
Terminology • Plastic (best) < titanium < < tantalum < stainless steel 51
• Magnetic susceptibility < cobalt chrome (worst)
• Phenomena where material becomes partially • MRI general methods to minimize metal artifact
magnetized in presence of applied external magnetic • Fast spin echo> conventional spin echo> gradient
field echo
• Geometric distortion + signal loss secondary to • Larger field of view
dephasing • Higher readout bandwidth
• Smaller voxel size
Imaging Findings • Appropriate geometric orientation of frequency
• CT: Metal causes severe x-ray attenuation (missing encode direction
data) in selected planes • Lower magnet field strength
• Missing data or hollow projections cause classic
"starburst" or streak artifacts during image Top Differential Diagnoses
reconstruction • Bone/osteophyte
• Materials with lower x-ray attenuation coefficients • Gas
produce less artifactual distortions • Hemorrhage
• Disc herniation
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METAL ARTIFACT
1 IPATHOLOGY 7. Frazzini VI et al: Internally stabilized spine: optimal choice
of frequency-encoding gradient direction during MR
52 imaging minimizes susceptibility artifact from titanium
General Features vertebral body screws. Radiology. 204(1):268-72, 1997
• General path comments: Post cervical 8. Doran SE et al: Internal fixation of the spine using a
discectomy/fusion, metallic susceptibility artifacts are braided titanium cable: clinical results and postoperative
produced by microscopic amounts of nickel, copper, magnetic resonance imaging. Neurosurgery. 38(3):493-6;
and zinc discussion 496-7, 1996
• Etiology: In anterior cervical discectomies, sufficient 9. Ortiz 0 et al: Postoperative magnetic resonance imaging
with titanium implants of the thoracic and lumbar spine.
metals to produce artifacts are deposited only by Neurosurgery. 38(4):741-5, 1996
contact of metal drill bits + suction tips 10. Petersilge CA et al: Optimizing imaging parameters for MR
• Epidemiology: S% of post-operative cervical evaluation of the spine with titanium pedicle screws. A]R
discectomy cases have sufficient metal artifact to Am] Roentgenol. 166(5):1213-8, 1996
obscure thecal sac 11. Shellock FG: MR imaging and cervical fixation devices:
evaluation of ferromagnetism, heating, and artifacts at 1.5
Tesla. Magn Reson Imaging. 14(9):1093-8, 1996
ICLINICAL ISSUES 12. Tominaga T et al: Magnetic resonance imaging of titanium
anterior cervical spine plating systems. Neurosurgery.
Presentation 36(5):951-5, 1995
13. Tartaglino LM et al: Metallic artifacts on MR images of the
• Most common signs/symptoms: Typically postoperative spine: reduction with fast spin-echo
asymptomatic, ancillary finding of surgical procedure techniques. Radiology. 190(2):565-9, 1994
14. Vaccaro AR.et al: Metallic spinal artifacts in magnetic
Demographics resonance imaging. Spine. 19(11):1237-42, 1994
• Age: Any age 15. Yoshino MT et al: Metallic postoperative artifacts on
• Gender: No gender predilection cervical MR. A]NR Am] Neuroradiol. 14(3):747-9, 1993
16. Toro VE et al: MR artifacts after anterior cervical
diskectomy and fusion: a cadaver study. ] Comput Assist
!DIAGNOSTIC CHECKLIST 17.
Tomogr. 17(5):696-9, 1993
Peterman SB et al: Magnetic resonance artifact in the
Consider postoperative cervical spine. A potential pitfall. Spine.
16(7):721-5, 1991
• Following anterior cervical discectomy/fusion small
amount of metal artifact is typical at fusion site
o Contact between the drill burr and suction tip
produced artifacts from metal flakes in soft tissues
• MRI pedicle screw artifact size correlates with
reduction in ratio of FOV to number of pixels in
frequency-encoding direction
Image Interpretation Pearls
• Minimize pedicle screw artifact by orienting frequency
encode gradient parallel to screw long axis, using FSE
technique
• Slice thickness 3-4 mm adequate, thinner sections
yield little artifact reduction
ISELECTED REFERENCES
1. Chang SD et al: MRI of spinal hardware: comparison of
conventional Tl-weighted sequence with a new metal
artifact reduction sequence. Skeletal Radiol. 30(4):213-8,
2001
2. Viano AM et al: Improved MR imaging for patients with
metallic implants. Magn Reson Imaging. 18(3):287-95,
2000
3. Henk CB et al: The postoperative spine. Top Magn Reson
Imaging. 10(4):247-64, 1999
4. Rudisch A et al: Metallic artifacts in magnetic resonance
imaging of patients with spinal fusion. A comparison of
implant materials and imaging sequences. Spine.
23(6):692-9, 1998
5. Wang]C et al: A comparison of magnetic resonance and
computed tomographic image quality after the
implantation of tantalum and titanium spinal
instrumentation. Spine. 23(15): 1684-8, 1998
6. Taber KH et al: Pitfalls and artifacts encountered in clinical
MR imaging of the spine. Radiographics. 18(6):1499-521,
1998
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METAL ARTIFACT
IIMAGE GALLERY 1
53
(Left) Sagittal T1 WI M R
shows metal artifact from
L4-5, L5-S1 interbody fusion
with cages. Artifact remains
confined to vertebral body
margins, allowing definition
of epidural disease. (Right)
Sagittal T2* GRE MR shows
extensive metal susceptibility
artifact in patient with L4-5,
L5-S1 interbody fusion with
cages.
Typical
(Left) Axial T2WI MR shows
susceptibility artifact from
left pedicle screw with
frequency encode direction
oriented right-left. Note
typical spatial mismapping
with increase signal (arrows).
(Right) Axial T2WI MR
shows metal artifact from left
pedicle screw. Frequency
encode direction is oriented
Ap, stretching artifact along
that direction, minimizing
right-left direction artifact.
Typical
(Left) Axial T2* GRE MR
shows focal low signal in
anterior epidural region
reflecting metal artifact from
tiny metal shard, not visible
on plain films or CT,
mimicking osteophyte.
(Right) Axial T1 WI MR
shows screw malposition.
Linear metal artifact from
vertebral body screw
following thoracic fusion
shows artifact is posterior
within body, overlapping
thecal sac and cord (arrow).
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RADIATION MYELOPATHY
1
54
Sagittal T2WI MR shows diffusely enlarged hyperintense Sagittal Tl C+ MR in the same patient shows patchy
cervical cord in patient treated previously for enhancement within long segment of enlarged cord,
nasopharyngeal cancer. T1 C+ MR showed focal low signal above, below. T2WI showed diffuse high
enhancement. signal throughout cord.
DDx: Myelopathy
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RADIATION MYELOPATHY
Key Facts
1
Terminology • Enhancing focus becomes site of focal atrophy in 55
• Chronic progressive radiation myelitis (CPRM) serial scans
• Delayed radiation myelopathy (DRM) Top Differential Diagnoses
• Radiation necrosis • Transverse myelitis
Imaging Findings • Multiple sclerosis
• Mainly in white matter of lateral spinothalamic • Astrocytoma
tracts, dorsal columns Pathology
• Clinical signs may reflect longer segment of damage • Radiation damage to cord
than shown on MR • Typically with doses over 50 Grey (Gy)
• Spindle-shaped cord swelling with irregular, focal • Fractionation schedule - more than 2 Gy per
rind of enhancement treatment
• Focal cord atrophy in late stage • Demyelination in lateral, dorsal tracts
• Ring or irregular enhancement surrounded by • Hyalinosis of intramedullary vessel walls
edematous cord
• Necrosis
• Enhancing focus may be absent
• Swollen astrocytes
I DIFFERENTIAL DIAGNOSIS • Endothelial damage
Transverse myelitis • Hyalinosis of intramedullary vessel walls
• More rapid onset of myelopathy • Necrosis
• Predisposing factors of infection, vaccination • Local calcium deposits
Multiple sclerosis
• Multifocal ICLINICAL ISSUES
• Patchy high signal foci on T2WI
• Sensory symptoms at onset Presentation
• Waxing, waning signs and symptoms • Most common signs/symptoms
o Progressive numbness and weakness
Cord infarct o Other signs/symptoms
• Acute onset of paralysis • Sphincter dysfunction
• Grey matter involvement predominates • Clinical profile
o Onset of weakness, numbness one month to several
Astrocytoma years following fractionated radiotherapy
• Very slow onset and progression o Radiotherapy of lung or nasopharyngeal cancer;
Syrinx mantle lymphoma therapy especially with
• More insidious onset intrathecal methotrexate
• Discrete cavity Demographics
• Age: Any
• Gender: No predilection
!PATHOlOGY
Natural History & Prognosis
General Features • Progressive, without significant improvement in most
• General path comments: Swollen, boggy cord cases
• Etiology
o Radiation damage to cord Treatment
o Typically with doses over 50 Grey (Gy) • Options, risks, complications: Hyperbaric oxygen
o May see in cases below 50 Gy helpful in some early cases
o Fractionation schedule - more than 2 Gy per
treatment
o Concurrent chemotherapy may be a predisposing IDIAGNOSTIC CHECKLIST
factor, especially if intrathecal
• Epidemiology: Unusual complication Consider
• Checking radiation field, fractionation schedule
Gross Pathologic & Surgical Features (especially if > 2 Gy per treatment)
• Edematous, enlarged cord
Image Interpretation Pearls
Microscopic Features • Late stage shows focal atrophy at sites of previous
• Demyelination in lateral, dorsal tracts contrast enhancement
• Lipid ladened microphages
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RADIATION MYELOPATHY
1 • Fatty replacement of marrow in vertebral bodies
within treatment field
24. Godwin-Austen RB et al: Observations on radiation
myelopathy. Brain. 98(4):557-68, 1975
56 • Pathologic changes in cord not always visible on MRI 25. Burns RJ et al: Pathology of radiation myelopathy.] Neural
Neurosurg Psychiatry. 35(6):888-98, 1972
ISELECTED REFERENCES
1. Warscotte L et al: Concurrent spinal cord and vertebral
bone marrow radionecrosis 8 years after therapeutic
irradiation. Neuroradiology. 44(3):245-8, 2002
2. Okada S et al: Pathology of radiation myelopathy.
Neuropathology. 21(4):247-65, 2001
3. Maddison P et al: Clinical and MRI discordance in a case of
delayed radiation myelopathy. ] Neurol Neurosurg
Psychiatry. 69(4):563-4, 2000
4. Maddison P et al: Clinical and MRI discordance in a case of
delayed radiation myelopathy. ] Neurol Neurosurg
Psychiatry. 69(4):563-4, 2000
5. Macbeth F: Radiation myelitis and thoracic radiotherapy:
evidence and anecdote. Clin Oncol (R Coli Radiol).
12(5):333-4, 2000
6. Calabro F et al: MRI of radiation myelitis: a report of a case
treated with hyperbaric oxygen. Eur Radiol. 10(7):1079-84,
2000
7. Nieder C et al: Radiation myelopathy: new perspective on
an old problem. Radiat Oncol Investig. 7(4):193-203, 1999
8. Rampling R et al: Radiation myelopathy. Curr Opin
Neurol. 11(6):627-32, 1998
9. Alfonso ER et al: Radiation myelopathy in over-irradiated
patients: MR imaging findings. Eur Radiol. 7(3):400-4, 1997
10. Koehler PJ et al: Delayed radiation myelopathy: serial
MR-imaging and pathology. Clin Neurol Neurosurg.
98(2):197-201, 1996
11. Wong CS et al: Radiation myelopathy following single
courses of radiotherapy and retreatment. Int J Radiat Oncol
Bioi Phys. 30(3):575-81, 1994
12. Hirota S et al: Chronological observation in early radiation
myelopathy of the cervical spinal cord:
gadolinium-enhanced MRI findings in two cases. Radiat
Med. 11(4):154-9, 1993
13. Schultheiss TE et al: Invited review: permanent radiation
myelopathy. Br J Radiol. 65(777):737-53, 1992
14. Dische S: Accelerated treatment and radiation myelitis.
Radiother Oneal. 20(1):1-2, 1991
15. ]eremic B et al: Incidence of radiation myelitis of the
cervical spinal cord at doses of 5500 cGy or greater. Cancer.
68(10):2138-41, 1991
16. Bloss]D et al: Radiation myelitis: a complication of
concurrent cisplatin and 5-fluorouracil chemotherapy with
extended field radiotherapy for carcinoma of the uterine
cervix. Gyneeol Oneal. 43(3):305-8, 1991
17. Magrini SM et al: Neurological damage in patients
irradiated twice on the spinal cord: a morphologic and
electrophysiologieal study. Radiother Oncol. 17(3):209-18,
1990
18. Marcus RB]r et al: The incidence of myelitis after
irradiation of the cervical spinal cord. Int J Radiat Oneal
Bioi Phys. 19(1):3-8, 1990
19. Dische S et al: Radiation myelitis and survival in the
radiotherapy of lung cancer. Int J Radiat Oncol Bioi Phys.
15(1):75-81, 1988
20. Goldwein JW: Radiation myelopathy: a review. Med
Pediatr Oncol. 15(2):89-95, 1987
21. Hatlevoll R et al: Myelopathy following radiotherapy of
bronchial carcinoma with large single fractions: a
retrospective study. IntJ Radiat Oneal Bioi Phys. 9(1):41-4,
1983
22. Fitzgerald RH Jr et al: Chronic radiation myelitis.
Radiology. 144(3):609-12, 1982
23. Worthington BS: Diffuse cord enlargement in radiation
myelopathy. Clin Radiol. 30(1):117-9, 1979
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RADIATION MYELOPATHY
IIMAGE GALLERY 1
57
Typical
(Left) Axial T2WI MR shows
obvious signal elevation in
cord. Patient developed
weakness and numbness
several months after neck
irradiation. (Right) Sagittal
T2WI MR shows fusiform
expansion and cord edema
in patient after previous neck
irradiation.
(Left) Sagittal T7 C+ MR
shows spindle shaped
enhancement in thoracic
cord; fatty marrow
replacement also noted in
patient following previous
radiation therapy for
vertebral metastases. (Right)
Sagittal T2WI MR shows
increased signal within cord
in patient previously treated
for skeletal metastases.
Variant
(Left) Sagittal T7 WI MR
shows focal low signal within
atrophic cord. Patient was
paraplegic several years after
chest irradiation. Note fat
signal in vertebral bodies.
(Right) Sagittal T2WI MR
shows atrophic cord, with
signal elevation, in
paraplegic patient several
years after chest irradiation.
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Sagittal TlWI MR shows homogeneous post irradiation Sagittal TlWI MR (left) in a patient with previous
fatty marrow in thoracic spine, with clear cranial and lumbar irradiation shows diffuse hyperintense marrow,
rostral demarcation (arrows), corresponding to except in collapsed L2 vertebra. Fatty marrow is of
radiotherapy portal. intermediate Sian T2WI (right).
ITERMINOLOGY MR Findings
Definitions • TlWI
o Very high SI
• Transformation of vertebral marrow into fatty marrow o Increased SI also in marrow outside radiation field
after therapeutic irradiation • From scatter dose of radiation
• T2WI: Intermediate SI
• PD/Intermediate: Intermediate SI
IIMAGING FINDINGS • STIR
General Features o High SI in acute phase
o Low SI thereafter
• Best diagnostic clue: Marrow signal intensity (SI)
within radiotherapy portal similar to subcutaneous fat • Tl C+
on TlWI . o Post-gadolinium enhancement in acute phase
• Probably related to vascular congestion
• Location: Corresponds to site of irradiation
o No enhancement thereafter
• Size: Corresponds to extent of radiation field
• Decreased vascularity and fibrosis
• Morphology • First three weeks
o Homogeneous marrow SI
o No change or early marrow Tl hyperintensity
• Heterogeneity due to residual or recurrent marrow
• Hyperintensity suggestive of hemorrhage ± fatty
disease
replacement
o Sharp demarcation between irradiated vs. untreated
o Hyperintensity on STIR
nlarrow
• Indicating marrow edema and necrosis
Radiographic Findings • Peak incidence nine days after irradiation
• Radiography: Osteopenia, compression fractures may • Subsequent gradual decrease in SI
be present • Three to six weeks
o Heterogeneous mottled pattern on Tl WI
CT Findings o Increasing fat intensity within central vertebral
• Bone CT marrow
o Compression fractures • Surrounding basivertebral vein
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POST IRRADIATION VERTEBRAL MARROW
Key Facts
1
Terminology • PET: Decreased marrow metabolic activity with 59
• Transformation of vertebral marrow into fatty FDG-18
marrow after therapeutic irradiation • MRI is the modality of ch~ice in marrow imaging
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POST IRRADIATION VERTEBRAL MARRnW
IIMAGE GALLERY 1
61
(Left) SagittalT7WI MR
demonstrates smooth
marrow hyperintensity in
thoracic vertebrae caudal to
TI-2 (arrow), which is the
upper boundary of
radiotherapy field. (Right)
Sagittal T7WI MR in another
patient who received pelvic
radiation shows fatty marrow
replacement in L4 and L5
vertebrae and sacrum.
Non-irradiated marrow in
upper lumbar spine appears
heterogeneous..
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SECTION 1: Gamuts
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Sagittal T2WI MR in a patient with acute, progressive Sagittal T1 C+ MR in same patient does not
myelopathy shows diffuse homogeneous hyperintensity demonstrate significant post-gadolinium enhancement
involving lower brain stem & entire cervical cord with Findings are compatible with acute transverse myelitis.
slight cord swelling.
ITERMINOlOGY MR Findings
Abbreviations and Synonyms • TlWI
o Normal
• Noncompressive myelopathy o Focal or diffuse hypointensity
• Acute transverse myelitis (ATM) o Mild hyperintensity due to petechial hemorrhage
Definitions • T2WI
o Focal or diffuse hyperintensity
• Inflammatory disorder of spinal cord associated with
many etiologies o Swollen cord
• STIR: Hyperintense
• Tl C+
o Variable enhancement
IIMAGING FINDINGS
• None
General Features • Focal, diffuse, heterogeneous
• Best diagnostic clue: Hyperintense lesion on T2WI • May be mass-like
with mild cord expansion without significant • Peripheral/meningeal
enhancement • Cord swelling and enhancement typically resolve
• Location: Thoracic> cervical> conus medullaris o Corresponding to clinical improvement
• Size: Segmental, multi-segmental, or holocord • Cord atrophy may be present in later stage
• Morphology: Fairly well-defined signal abnormality Imaging Recommendations
Radiographic Findings • Best imaging tool
• Myelography o Sagittal and axial T1 WI & T2WI MRI with
o Cord swelling gadolinium
o Cerebral spinal fluid flow may be impeded • 40% of clinical ATM with normal MRI findings
CT Findings
• NECT: Mild cord enlargement
• CECT: Focal, diffuse, or heterogeneous enhancement
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SECONDARY ACUTE TRANSVERSE MYELITIS
1 • Direct cord involvement ± autoimmune
phenomenon
• Clinical profile: Progression to severe neurologic
deficits within days
4 • Epstein-Barr virus, Cytomegalovirus
• Herpes zoster, varicella-zoster virus Demographics
• HIV, HTLV-1 • Age
• Enteroviruses o All ages can be effected
• Non-viral pathogens: Syphilis, Lyme disease, • Peak incidence: 10-19 and 30-39 yo
mycoplasma pneumoniae, schistosomiasis • Gender
o Post-vaccination o Female predominance in collagen vascular disease
• Autoimmune response with small vessel o Other etiologies without significant gender
vasculopathy predilection
• Polio, rubella, rabies, smallpox, influenza, Natural History & Prognosis
hepatitis B vaccinations, etc.
• 30-50% with complete recovery after treatment
o Post irradiation
• Dose of 45-50 Gy in ' 1.8-2 Gy fractions Treatment
considered safe • Collagen vascular disease
• Toxic radiation effeCt may be potentiated by o Corticosteroids
concurrent chemotherapy o Immunosuppressive therapy
• Symptoms develop 6-30 months after therapeutic • Cyclophosphamide, azathioprine, chlorambucil
irradiation o Plasmapheresis
• MRI may be normal even if symptoms present o Intravenous immunoglobulin
o Arterio-venous malformation (AVM) • Infectious etiologies
• Chronic ischemia/venous stasis o Anti-viral, anti-bacterial medications
• Course more indolent compared to other o Corticosteroids
etiologies • Post irradiation myelitis
o Para-neoplastic syndrome o Hyperbaric oxygen treatment
• May be initial presentation of underlying
neoplasm
• Autoantibodies against proteins in spinal cord IDIAGNOSTIC CHECKLIST
• Lung, breast, hepatocellular carcinoma, etc.
• Lymphoma, leukemia, multiple myeloma Consider
• Epidemiology: 4.6 new cases of transverse myelitis per • Brain MRI to exclude intracranial lesions associated
million people per year in US with MS or ADEM
• Associated abnormalities
o Optic neuritis in SLE
o Fatty marrow in post irradiation ISELECTED REFERENCES
o Abnormal surface vessels in cord AVM
1. Harzheim M et al: Discriminatory features of acute
Microscopic Features transverse myelitis: a retrospective analysis of 45 patients. ]
Neurol Sci. 217(2):217-23,2004
• Necrosis of gray and white matter 2. Sherer Y et al: Transverse myelitis in patients with
• Destruction of neurons, axons, and myelin antiphospholipid antibodies--the importance of early
• Astrocytic gliosis diagnosis and treatment. Clin Rheumatol. 21(3):207-10,
• Perivascular lymphocytic infiltrate with vascular 2002
occlusion 3. Transverse Myelitis Consortium Working Group: Proposed
diagnostic criteria and nosology of acute transverse
myelitis. Neurology. 59(4):499-505, 2002
4. Goebels N et al: Extensive myelitis associated with
ICLINICAL ISSUES Mycoplasma pneumoniae infection: magnetic resonance
Presentation imaging and clinical long-term follow-up. J Neurol.
248(3):204-8, 200]
• Most common signs/symptoms 5. Scotti G et al: Diagnosis and differential diagnosis of acute
o Sensory deficit transverse myelopathy. The role of neuroradiological
• Loss of pain and temperature sensation investigations and review of the literature. Neurol Sci. 22
• Well-defined upper level SuppI2:S69-73,2001
• Ascending paresthesia in bilateral upper/lower 6. Kovacs B et al: Transverse myelopathy in systemic lupus
extremities erythematosus: an analysis of 14 cases and review of the
literature. Ann Rheum Dis. 59(2):120-4, 2000
• Band-like dysesthesia 7. Tartaglino LM et al: MR imaging in a case of
o Other signs/symptoms: postvaccination myelitis. A]NR Am J Neuroradiol.
• Back ± radicular pain 16(3):581-2, 1995
• Paraplegia/quadriplegia 8. Austin SG et al: The role of magnetic resonance imaging in
• Bladder and bowel urgency, frequency, and acute transverse myelitis. Can] Neurol Sci. 19(4):508-11,
retention 1992
• Hypotonia and hyporeflexia initially
• Spasticity and hyperreflexia over time
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SECONDARY ACUTE TRANSVERSE MYELITIS
IIMAGE GALLERY 1
5
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Sagittal reformation from an axial bone CT demonstrates Sagittal T2WI MR shows grade 4 anterolisthesis of L5 on
L5 pars defect without anterolisthesis. 51 secondary to bilateral L5 spondylolysis. There is
severe central canal narrowing at superior endplate of
51.
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Key Facts
1
Terminology • Slipped vertebral ring apophysis 7
• Back pain in children & adolescents with or without • Discitis and osteomyelitis
underlying organic cause • Scheuermann disease
• Juvenile calcific discitis
Imaging Findings • Idiopathic scoliosis
• Location: Lumbar segment most common in • Neoplasms
nonspecific back pain • Tethered cord
• Plain films and bone scan part of initial imaging • Ankylosing spondylitis (AS)
work-up
• MRI is the modality of choice if neurologic symptoms
Pathology
present • General path comments: Up to 60% of back pain in
children without underlying etiology
Top Differential Diagnoses • Emotional, psychosocial, and conduct problems
• Sports injuries linked to development of low back pain without
• Spondylolysis organic cause
• Spondylolisthesis
• Lumbar disc herniation
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Typical
(Left) Sagittal TI C+ MR with
fat suppression in infant
shows enhancing disc &
endplates at L5-s1, with
associated prevertebral,
epidural enhancement,
compatible with discitis,
osteomyelitis. (Right) Sagittal
TlWI MR in patient with
worsening back pain and
progressive neurologic
symptoms demonstrates
thickened filum terminale,
tethered by intraspinal
lipoma.
Typical
(Left) Sagittal TI C+ MR in
child with leukemia shows
moderate compression of L5
vertebra with mild
compression in upper
lumbar & lower thoracic
vertebrae, compatible with
leukemic infiltration. (Right)
Sagittal T2WI MR in child
with back pain shows
expansile mass involving the
conus, with central cystic
change. Cellular
ependymoma was found at
surgery.
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KYPHOSIS
1
10
Sagittal 30 CT myelogram shows iatrogenic gibbus Sagittal bone CT reformation shows gibbus deformity
deformity due to infection above level of fusion. Arrow and multiple vertebral fusions due to tuberculosis of the
shows intact intervertebral cage below level of spine (Pott disease).
deformity.
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KYPHOSIS
Key Facts
1
Terminology • Lumbar spine has normal lordosis 25-35° 11
• Increased apex dorsal curvature of spine in sagittal Pathology
plane
• Traumatic
• Gibbus deformity: Extreme, angular focal kyphosis
• Congenital
Imaging Findings • Developmental
• Best diagnostic clue: Increased Cobb angle on lateral • Infectious
radiograph • Neoplastic
• Thoracic spine has normal kyphosis < 40° • Iatrogenic
• Arthritis
I CLINICAL ISSUES
Demographics (Left) Sagittal bone CT reformation shows congenital kyphosis due to
• Age fusion of three vertebrae. This isolated, previously undiagnosed
o Tumor: Any age; type of tumor varies with age anomaly in 30 yo patient has led to premature 000 of lower lumbar
o Child: Consider neurogenic, congenital causes spine. (Right) Sagittal T1WI MR shows iatrogenic kyphosis folloWing
wide cervical laminectomy. Anterolisthesis has developed at C3/4
o Adolescent or young adult: Consider Scheuermann, and there is bone marrow edema of C4 (arrow) related to abnormal
postural, post-traumatic motion.
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PERIPHERAL NEUROPATHY
1
12
Axial PO/Intermediate MR of elbow shows enlarged Axial STIR MR shows abnormal signal in the anterior
ulnar n. (arrow) due to cubital tunnel syndrome, the muscles of the leg (arrows), which are innervated by the
second most common entrapment neuropathy in the peroneal n. Images at level of knee showed tumor of
upper extremity. peroneal n.
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PERIPHERAL NEUROPATHY
Key Facts
1
Terminology Imaging Findings 13
• Primary disorder of one or more peripheral nerves • Best diagnostic clue: Abnormal T2 signal intensity in
(nn.) muscles innervated by effected nerve
• Mononeuropathy: Single nerve (n.) effected
• Multiple mononeuropathies: Multiple adjacent nn. Top Differential Diagnoses
effected • Radiculopathy
• Polyneuropathy: Effects multiple nn.; usually distal} • Myositis
symmetric involvement
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Sagittal STIR MR shows burst fracture of Til due to Sagittal TI WI MR shows pathologic Til burst fracture
breast cancer metastasis. Diffuse marrow infiltration is due to metastasis. Uniform marrow replacement helps
evident. Fracture line traverses vertebral body (arrow). distinguish it from traumatic fracture.
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Key Facts
1
Terminology • MR vs. CT scan still debatable as to best tool 15
• Fracture occurring through bone weakened by tumor • MR scan to evaluate rounded areas of tumor, pattern
or infection of enhancement
• CT scan to evaluate for trabecular, cortical
Imaging Findings destruction
• Best diagnostic clue: Trabecular and/or cortical
destruction
Top Differential Diagnoses
• Rounded area of marrow replacement and trabecular • Osteoporotic compression fracture
destruction • Neuropathic joint
• May replace entire vertebral body marrow • Idiopathic kyphosis
• Usually can be distinguished on MR from band-like • Scheuermann disease
signal abnormalities due to nonpathologic fracture • Osteogenesis imperfecta
• Fracture may be compression or burst Pathology
• Soft tissue paraspinous mass not a specific finding • Epidemiology: Pathologic fracture may be presenting
• Can be mimicked by hematoma from nonpathologic sign of tumor, especially breast carcinoma
fracture
o Soft tissue paraspinous mass not a specific finding o Intraosseous hematoma can mimic tumor
• Can be mimicked by hematoma from • Pathologic fractures in myeloma often mimic
nonpathologic fracture osteoporotic compression fractures
o Use to evaluate symptomatic patients after fracture
fixation Nuclear Medicine Findings
• Artifact from most metallic fixators does not • Bone Scan
prevent diagnostic CT scan o Increased activity at fracture line
• Thin sections, overlapping, multidetector helical o Underlying lesion may show increased, normal or
CT needed decreased uptake
• PET
MR Findings o Fracture shows increased uptake with or without
• TlWI underlying lesion
o Rounded or diffuse low signal intensity marrow o Accuracy improved by PET/CT
replacement
o Very low signal intensity fracture line mayor may Imaging Recommendations
not be visible • Best imaging tool
• T2WI o MR vs. CT scan still debatable as to best tool
o Rounded or diffuse high signal intensity marrow • MR scan to evaluate rounded areas of tumor,
replacement pattern of enhancement
o May be same signal intensity as fatty marrow • CT scan to evaluate for trabecular, cortical
o Very low signal intensity fracture line destruction
o Increase conspicuity of lesion by using fat-saturation
sequences
• PD/Intermediate: Tumor may be indistinguishable I DIFFERENTIAL DIAGNOSIS
from normal marrow
Osteoporotic compression fracture
• STIR
o Rounded or diffuse high signal intensity marrow • Thinned trabeculae without focal destruction
• Corresponds to low signal area on TlWI • Cortical destruction is absent
o Low signal may be seen in sclerotic metastases • Often involves multiple levels
o Very low signal intensity fracture line • Paraspinous hematoma mimics tumor mass
• DWI Neuropathic joint
o Initially promising results with DWI not confirmed
• Fractures
on subsequent studies • Bone debris
o No increase in accuracy compared to Tl WI
• Preserved bone density
• Tl C+ • Subluxations of vertebral bodies
o Enhancement around fracture line and of • Centered on discs and facet joints
underlying lesion • Soft tissue mass
o Enhancement usually more rounded than in
nonpathologic fracture Idiopathic kyphosis
• Nonpathologic fractures enhance in band-like • Mild wedging of several adjacent upper thoracic
configuration vertebrae
• Majority of cases can be distinguished from benign • Angular deformity endplates or anterior cortex absent
fracture by above criteria
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General Features
• General path comments I DIAGNOSTIC CHECKLIST
o Risk of fracture from tumor depends on
• Tumor type: Lytic tumor higher risk than blastic Consider
• Tumor volume • May be presenting sign of malignancy
• Pedicle involvement
• Amount of cortical destruction, bowing of
vertebral margins ISELECTED REFERENCES
• Load on vertebra 1. Even-Sapir E et al: Assessment of malignant skeletal
• Etiology disease: initial experience with 18F-fluoride PET/CT and
o Most common primary tumors comparison between 18F-fluoride PET and 18F-fluoride
• Malignant: Ewing sarcoma, lymphoma, leukemia PET/CT.] Nucl Med. 45(2):272-8, 2004
• Benign: Hemangioma, Langerhans cell 2. Roth SE et al: Metastatic burst fracture risk prediction using
histiocytosis, fibrous dysplasia, unicameral bone biomechanically based equations. Clin Orthop.
(419):83-90, 2004
cyst
3. Fayad LM et al: Sacral fractures: a potential pitfall of FDG
• Borderline: Giant cell tumor, chordoma positron emission tomography. A]R Am] Roentgenol.
• Vertebra plana manifestation of fracture 181(5):1239-43, 2003
o Most common metastatic tumors 4. Castillo M: Diffusion-weighted imaging of the spine: is it
• Breast, lung, renal, gastrointestinal tumors reliable? A]NR Am] Neuroradiol. 24(6):1251-3, 2003
• More common in lytic than blastic tumors 5. Bertuna G et al: Marked osteoporosis and spontaneous
o Osteomyelitis vertebral fractures in children: don't forget, it could be
• Involvement of 2 adjacent vertebral bodies and leukemia. Med Pediatr Oncol. 41(5):450-1, 2003
6. Lieberman I et al: Vertebroplasty and kyphoplasty for
intervening disc
osteolytic vertebral collapse. Clin Orthop. (415
• Fracture may be extensive Suppl):SI76-86,2003
o Radiation 7. Mahnken AH et al: Multidetector CT of the spine in
• Bone sclerotic, fragmented multiple myeloma: comparison with MR imaging and
• Sharp demarcation of abnormal bone in radiation radiography. A]R Am] Roentgenol. 178(6):1429-36,2002
field from normal bone outside it 8. Castillo M et al: Diffusion-weighted MR imaging offers no
o Paget disease advantage over routine noncontrast MR imaging in the
• Usually occurs in lytic phase detection of vertebral metastases. A]NR Am] Neuroradiol.
• Epidemiology: Pathologic fracture may be presenting 21(5):948-53,2000
9. Lo LD et al: Are L5 fractures an indicator of metastasis?
sign of tumor, especially breast carcinoma Skeletal Radiol. 29(8):454-8, 2000
Gross Pathologic & Surgical Features 10. Shih TT et al: Solitary vertebral collapse: distinction
between benign and malignant causes using MR patterns. ]
• Tumor or infection permeating bone Magn Reson Imaging. 9(5):635-42, 1999
11. Baur A et al: Diffusion-weighted MR imaging of bone
Microscopic Features marrow: differentiation of benign versus pathologic
• Fracture callus can mimic tumors compression fractures. Radiology. 207(2):349-56, 1998
12. Lecouvet FE et al: Vertebral compression fractures in
multiple myeloma. Part 1. Distribution and appearance at
ICLINICAL ISSUES MR imaging. Radiology. 204(1):195-9, 1997
13. Cuenod CA et al: Acute vertebral collapse due to
Presentation osteoporosis or malignancy: appearance on unenhanced
• Most common signs/symptoms and gadolinium-enhanced MR images. Radiology.
199(2):541-9, 1996
o Back pain, greatest at night
14. Moulopoulos LA et al: MR prediction of benign and
o Fracture with history of no trauma or minor trauma malignant vertebral compression fractures. ] Magn Reson
o Other signs/symptoms Imaging. 6(4):667-74, 1996
• Nerve or cord compression 15. An HS et al: Can we distinguish between benign versus
malignant compression fractures of the spine by magnetic
Demographics resonance imaging? Spine. 20(16):1776-82, 1995
• Age: Usually in older patients
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PATHOLOGIC VERTEBRAL FRACTURE
IIMAGE GALLERY 1
I
17
Typical
~f ~"')
(Left) Sagittal bone CT shows
prostate cancer metastases
and pathologic fracture
.! (arrow), Sclerotic metastases
have lower rate of fracture
than lytic ones, but are at
increased risk compared to
i· '\'.
• CT with soft tissue window
shows vertebra plana from
metastatic disease, Note
tumor extension into spinal
...
• ') t
' . ",. ' canal (arrow).
\
. ,',. '''t "'~"
, ,~
Variant
(Left) Sagittal T7 C+ MR
shows L7 fracture (arrow)
with band-like enhancement
suggesting benign etiology.
Enhancement of other
lesions allowed diagnosis of
malignancy. Patient with
myeloma. (Right) Sagittal
bone CT shows pathologic
L7 fracture (arrow) due to
myeloma, Trabecular
destruction well seen
adjacent to fracture. Multiple
other lytic lesions visible, as
well as T7 pathologic
fracture.
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DENSE- VERTEBRAL BODY
1
18
Lateral radiography shows "ivory vertebra"· of L3 due to Sagittal bone CT shows sclerotic metastases with
prostate metastasis. Less severe involvement seen at L1. fractures and smudgy, amorphous increased density.
r,,~t ,.
~(
...... 1
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DENSE VERTEBRAL BODY
Key Facts
1
Terminology Pathology 19
• Osteosclerosis • Tumors and tumor-like conditions
• Increased density of 1 or more vertebral bodies • Systemic disorders
compared to normal patients • Iatrogenic
• Infection
Imaging Findings • Healing fracture
• Best imaging tool: CT scan • Neuroarthropathy
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INDEX
A dural dysplasia vs., I:l-1l8i, 1:1-119
failure of vertebral formation vs., I:I-64i
ABC. See Aneurysmal bone cyst (ABC). kyphosis vs., VIII:l-lOi
Abscess. See also Empyema; Phlegmon. mucopolysaccharidoses vs., 1:1-156i, 1:1-157
acquired lumbar spinal stenosis vs., 1I:2-60i, II:2-61 thanatophoric dwarfism vs., I:I-150i, 1:1-150
angiolipoma vs., IV:I-74i, IV: 1-75 Acoustic neurofibromatosis, bilateral. See
bone graft complications vs., VII:I-32i, VII:I-33 Neurofibromatosis type 2.
cervical intervertebral disc herniation vs., II:2-27 AcqUired spinal stenosis
epidural, III: 1-22 to III: 1-24, I1I:1-2Si cervical, II:2-64 to II:2-66, II:2-67i
differential diagnosis, III:I-22i, III:I-23 congenital spinal stenosis vs., I:I-122i, 1:1-123
•
epidural-subdural hematoma vs., II:1-126i, II:1-127 lumbar, II:2-60 to II:2-62, II:2-63i
extramedullary hematopoiesis vs., V:2-16i, V:2-17 ADEM. See Encephalomyelitis, acute disseminated
hematoma vs., V:I-34i, V:I-35 (ADEM).
lipomatosis vs., IV:2-18i, IV:2-19 Adhesions, spinal cord herniation vs., II:I-121
lumbar intervertebral disc herniation vs., 11:2- Adolescents, back pain in, VIII: 1-6 to VIII: 1-8, VIII:I-9i
34i, II:2-35 AIDS-associated polyneuropathy. See also HIV
lymphoma vs., IV:I-55 myelopathy.
peridural fibrosis vs., VII:I-25 CSF disseminated metastases vs., IV:I-99
post-operative infection vs., VII:1-38i Alcaptonuria/Alkaptonuria. See Ochronosis.
pseudomeningocele vs., VII:1-28i, VII:I-29 ALL. See Leukemia.
spinal cord herniation vs., 1I:1-120i, II:I-121 Amputation
subdural abscess vs., III:1-26i, III: 1-27 post-operative peripheral neuropathy vs., VIII: 1-12
traumatic disc herniation vs., 1I:1-90i, II:I-91 traumatic neuroma due to, VI:I-16 to VI:I-17
facet joint synovial cyst vs., 1I:2-S6i, II:2-57 peripheral nerve tumor vs., VI:I-34i
in failed back surgery syndrome, VII:I-47 Amyotrophy, neuralgic. See Brachial plexus neuritis,
myelitis-cord, III: 1-42 to III: 1-44, I1I:l-4Si idiopathic.
paraspinal, III: 1-30 to III: 1-32, I1I:1-33i Aneurysmal bone cyst (ABC), IV: 1-26 to IV: 1-28, IV:I-29i
pseudomeningocele vs., VII: 1-29 back pain in children vs., VIII: 1-7
paravertebral, extramedullary hematopoiesis vs., differential diagnosis, IV:I-26i, IV:I-27
V:2-16i, V:2-17 echinococcus vs., III: 1-51
post-operative spinal complications vs., VII:1-42i, fibrous dysplasia vs., IV:2-30i, IV:2-31
VII: 1-44 giant cell tumor vs., IV:1-30i, IV:I-31
psoas, traumatic spinal muscle injury vs., 1I:1-102i osteoblastoma vs., IV:1-22i, IV: 1-23
pyogenic osteochondroma vs., IV:1-34i, IV:I-35
cysticercosis vs., III:1-58i, III:I-59 osteosarcoma vs., IV:I-42i, IV:I-43
schistosomiasis vs., III:I-55 pseudomeningocele vs., VII: 1-29
recurrent vertebral disc herniation vs., VII:1-34i, Angioblastic meningioma. See Hemangiopericytoma.
VII:I-35 Angiolipoma, IV:I-74 to IV: 1-76, IV:I-77i
retropharyngeal, calcific longus coli tendinitis vs., Angioma, cavernous. See Cavernous angioma.
1I:2-106i Ankylosing spondylitis. See Spondyloarthropathy,
rhabdomyolysis vs., 1I:1-98i, II:I-99 seronegative.
subdural, III: 1-26 to III: 1-28, III:1-29i Anterior compression fracture. See Compression
subdural hematoma vs., V:1-38i, V:1-39 fractures.
Accelerated degeneration, VII: 1-18 to VII: 1-20, VII:I-2li Anterior sacral meningocele. See Meningocele, anterior
Accessory ossification center(s) sacral.
OCCipital condyle fracture vs., 1I:1-12i, II:I-13 Anular bulge, vertebral disc, II:2-18 to II:2-20, II:2-2li
sacral traumatic fracture vs., 1I:1-82i, II: 1-83 Anular tear, vertebral disc, II:2-22 to II:2-24, 1I:2-2Si
Achondroplasia, 1:1-152 to 1:1-154, 1:1-155i AO dislocation. See Atlanto-occipital dislocation.
congenital scoliosis and kyphosis vs., I:I-134i, 1:1-135 Apophysis
congenital spinal stenosis vs., I:I-122i, 1:1-123 ring fracture, II:I-94 to II:I-96, 1I:1-97i
differential diagnosis of, I:1-152i, 1:1-153
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INDEX
slipped vertebral ring, back pain in children vs., of hemodialysis. See Spondyloarthropathy, of
VIII: 1-7 hemodialysis.
Apraxia, transient traumatic cord, 1I:1-116 to 1I:1-118, seronegative. See Spondyloarthropathy, seronegative.
1I:1-119i Arthrosis, facet, ossification ligamentum flavum, 11:2-
Arachnoid cysts, IV:2-2 to IV:2-4, IV:2-5i 84i, 1I:2-85
cysticercosis vs., III: 1-59 Articular mass fracture (lateral cervical flexion injury),
differential diagnosis, IV:2-2i, IV:2-3 1I:1-46 to 1I:1-47
epidermoid tumors vs., I:1-28i, 1:1-29 Artifacts
neurenteric cyst vs., I:1-60i, 1:1-61 CSF flow, type IV AVF vs., V:1-18i, V:I-19
spinal cord herniation vs., 1I:1-12Oi Gibbs, post-traumatic syrinx associated with, 11:1-
Arachnoiditis 108i, II: 1-109
chemical or post-surgical, Guillain-Barre syndrome metal, VII:l-50 to VII: 1-52, VII:1-53i
vs., 111:2-3 MRI, superficial siderosis vs., V:1-42i, V:I-43
infectious. See Meningitis. vertebral artery dissection vs., 1I:1-130i, 1I:1-131
lumbar, 111:2-10 to 111:2-12, III:2-13i Ascending paralysis. See Guillain-Barre syndrome.
spinal meningitis vs., 1I1:1-46i, 111:1-47 Aseptic meningitis, myelography complications vs.,
myelography complications vs., VII: 1-2i, VII: 1-3 VII: 1-3
peridural fibrosis vs., VII:1-24i, VII:I-25 ASM. See Meningocele, anterior sacral.
Arachnoiditis ossificans, lumbar, 111:2-14 to 111:2-15 Aspergillosis, superior sulcus tumor vs., VI:I-7
Arch fracture; See Hyperextension injury, cervical. Asphyxiating thoracic dysplasia, thanatophoric
Arnold-Chiari malformations. See Chiari entries. dwarfism vs., 1:1-150
Arteriovenous fistulas (AVFs) Astrocytoma, IV:I-I02 to IV: 1-104, IV:1-105i
dural acquired cervical spinal stenosis vs., 1I:2-64i
traumatic, II: 1-138 to II: 1-139 cavernous malformation of spinal cord vs., V:1-22i,
type I (DAVF), V:I-6 to V: 1-8, V:1-9i V: 1-23
differential diagnosis, V:1-6i, V:I-7 CSF disseminated metastases vs., IV:I-99
myelitis-cord abscess vs., III:1-42i, 111:1-43 cysticercosis vs., III: 1-59
schistosomiasis vs., III: 1-54i, III: 1-5 5 differential diagnosis, IV:1-102i, to IV: 1-103
spinal cord infarction vs., V:1-26i, V:I-27 dural dysplasia vs., 1:1-119
type IV AVF vs., V:1-18i, V:I-19 ependymoma vs., IV:1-102i, IV:I-I03, IV:1-106i,
type IV, V:I-18 to V: 1-20, V:1-2li IV: 1-107
differential diagnosis, V:1-18i, V:I-19 hemangioblastoma of spinal cord vs., IV:I-115
type II AVM vs., V:1-lOi, V: 1-11 idiopathic acute transverse myelitis vs., 11I:2-28i
type III AVM vs., V:1-14i, V:I-15 intramedullary lymphoma vs., IV:I-55
Arteriovenous malformations (AVMs) melanocytoma vs., IV:1-126i, IV:I-126
acute disseminated encephalomyelitis vs., 1I1:2-24i, myelitis-cord abscess vs., 1I1:1-42i, 111:1-43
111:2-25 radiation myelopathyvs., VII:I-55
astrocytoma of spinal cord vs., IV:1-102i, IV:l-103 sarcoidosis vs., 11I:2-16i
cavernous malformation of spinal cord vs., V:1-22i, schistosomiasis vs., 1I1:1-54i
V:I-23 secondary acute transverse myelitis vs., VIII:1-2i
dural arteriovenous fistula type I vs., V:I-7 spinal cord infarction vs., V:1-26i
hemangioblastoma of spinal cord vs., IV:1-114i, spinal cord metastases vs., IV:1-118i, IV:I-119
IV: 1-115 syringomyelia vs., IV:2-14i
melanocytoma vs., IV:1-126i, IV:I-127 type II AVM vs., V:1-10i
spinal cord metastases vs., IV:I-119 type III AVM vS., V:1-14i, V:I-15
traumatic dural arteriovenous fistula vs., 1I:1-139i type IV AVF vs., V:I-19
type II, V:l-IO to V:I-12, V:1-13i ventriculus terminalis vs., 1:1-95
type III, V:I-14 to V:I-16, V:1-17i Asymmetric ligamentum flavum hypertrophy, facet joint
type II AVM vS., V:1-10i, V:l-11 synovial cyst vs., II: 2-5 7
viral myelitis vs., III: 1-35 Atherosclerotic disease
Arteritis, viral myelitis vs., 111:1-35 carotid artery dissection vs., 1I:1-134i, II: 1-135
Arthritis. See Juvenile chronic arthritis; Osteoarthritis; vertebral artery dissection vs., 1I:1-130i, 1I:1-131
Psoriatic arthritis; Rheumatoid arthritis; Septic facet Atlanto-axial osteomyelitis (CI-C2 osteomyelitis), III:l-
joint arthritis. 14 to III:I-17, III:1-18i
Arthropathy Atlanto-occipital dislocation, II:I-I0 to II: 1-11
facet joints Atlas
cervical, 1I:2-48 to 1I:2-50, 1I:2-5li congenital anomalies, Jefferson Cl fracture vs., 11:1-
lumbar, 1I:2-52 to 1I:2-54, 1I:2-55i 16i,II:l-17
in gout. See Gout. pseudospread, in children, Jefferson Cl fracture vs.,
neurogenic. See Neurogenic (Charcot) arthropathy. 1I:1-16i, 1I:1-17
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INDEX
rotational malalignment, Jefferson C1 fracture vs., ulnar neuropathy vs., VI:1-4S
I1:1-17 Brachial plexus traction injury, VI:1-12 to VI:1-14,
Atlas burst fracture Oefferson C1 fracture)} I1:1-16 to I1:1- VI:1-1Si
18,II:1-19i differential diagnosis, VI:1-12i} VI: 1-13
Atrophy, denervation-related muscle hypertrophic neuropathy vs., VI:1-30i} VI:1-31
differential diagnosis, VI:1-lOi idiopathic brachial plexus neuritis vs., VI:1-22i,
traumatic spinal muscle injury vs.,11:1-102i VI:1-23
Autoimmune diseases. See Inflammatory and peripheral neurolymphomatosis vs., VI:1-38i} VI:1-39
autoimmune diseases. radiation plexopathy vs., VI:1-26i} VI:1-27
Avascular necrosis thoracic outlet syndrome vs., VI:1-18i, VI:1-19
lytic osseous metastases vs., IV:1-10i, IV:1-ll Breast metastasis
post-traumatic. See Kummell disease. astrocytoma of spinal cord vs., IV:1-102i
AVE See Arteriovenous fistulas (AVFs). radiation plexopathy vs., VI:1-26i} VI:1-27
AVMs. See Arteriovenous malformations (AVMs). Brittle bone disease. See Osteogenesis imperfecta.
Avulsion pseudomeningocele. See Brachial plexus Broken wires, VII:1-14i
traction injury. Brown tumor of hyperparathyroidism, giant cell tumor
Axis pillars, rotational malalignment of, Jefferson C1 vs.,IV:1-31
fracturevs., I1:1-17 Burner/stinger syndrome, idiopathic brachial plexus
neuritis vs., VI: 1-24
Burst fractures. See also Compression fractures.
B C2, I1:1-24 to II: 1-26, II:1-27i
cervical, II: 1-42 to II: 1-43
Baastrup disease, tumoral calcinosis vs., V:2-20i, V:2-21
Back pain, in children, VIII: 1-6 to VIII: 1-8, VIII:1-9i
Basilar impression, acquired, craniovertebral junction
cervical injuries vs.
hyperflexion injury, II:1-32i, II:1-32
hyperflexion-rotation injury, II:1-38i} II:1-39
II
variants vs., 1:1-78i, 1:1-79 III
Beam hardening artifact, VII: 1-50 Jefferson C1 fracture, II:1-16 to II:1-18, II:1-19i
Bilharziasis. See Schistosomiasis. lumbar, II:1-76 to II: 1-78, II:1-79i
Blastic metastatic disease, osteopetrosis vs., 1:1-162i lumbar fractures vs.
Blastic osseous metastases, IV: 1-6 to IV: 1-8, IV:1-9i anterior compression fracture, II:1-70i, II:1-71
"Block vertebra," 1:1-70 to 1:1-72, 1:1-73i lateral compression fracture, II:1-73i
Bone, metal artifact vs'} VII:1-S1 thoracic fractures vs.
Bone cyst, aneurysmal. See Aneurysmal bone cyst (ABC). anterior compression fracture, II:1-Sli
Bone graft complications} VII: 1-32 to VII: 1-33 Chance fracture, II:1-64i} II:1-6S
post-operative spinal complications vs., VII: 1-43 lateral compression fracture, II:1-S4i, II:1-SS
Bone infarct low thoracic distraction fracture, II:1-68i
bone island vs., 1:1-93 thoracolumbar, II:1-S8 to II:1-S9
nontraumatic, Kummell disease vs., IV:2-32i} IV:2-33 vertebral, failure of vertebral formation vs., 1:1-64i
Bone island, 1:1-92 to 1:1-93 Butterfly vertebra, partial vertebral duplication vs., I: 1-
differential diagnosis, 1:1-92i} 1:1-93 68-69
giant, osteosarcoma vs., IV:1-43
Schmorl node vs., 1:1-142i} 1:1-143
Bone marrow. See Marrow. c
Bone neoplasms/tumors. See also specific types. Calcaneal stress fractures, posterior tibial nerve
neurogenic (Charcot) arthropathy vs., II:2-72i, II:2-73 entrapment vs., VI:1-S4i
primary Calcific discitis, back pain in children vs., VIII:1-7
C1-C2 osteomyelitis vs., III: 1-15 Calcific longus coli tendinitis, II:2-106 to I1:2-107
echinococcus vs., III:1-SOi} III:1-Sl hemodialysis spondyloarthropathy vs., II:2-104
hemangiopericytoma vs., IV:1-82i} IV:1-83 Calcification of hemodialysis, cervical peridural, OPLL
pedicle insufficiency fracture vs., II:1-104i} II:1-10S vs., II:2-80i, II:2-81
superior sulcus tumor vs., VI:1-6i Calcified disc fragment, apophyseal ring fracture vs.,
tumoral calcinosis vs., V:2-21 II: 1-95
"Bounce back" fractures, due to vertebroplasty, VII:1-7 Calcified disc herniation
Brachial plexus neoplasms/tumors limbus vertebra vs., 1:1-83
idiopathic brachial plexus neuritis vs., VI:1-23 OPLL vs., II:2-80i, II:2-81
malignant infiltrates, radiation plexopathy vs., VI:1- Calcified dural plaques, lumbar arachnoiditis ossificans
26i} VI:1-27 vs., III:2-14i} III:2-1S
superior sulcus tumor vs., VI:1-7 Calcified island. See Bone island.
Brachial plexus neuritis, idiopathic, VI:1-22 to VI:1-24, Calcinosis, tumoral, V:2-20 to V:2-22, V:2-23i
VI:1-2Si differential diagnosis, V:2-20i} V:2-21
differential diagnosis, VI:1-22i} VI: 1-23 to VI:1-24 lumbar facet arthropathy vs., II:2-42i, I1:2-S3
suprascapular nerve entrapment vs., VI:1-48i} VI:1-49
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INDEX
Calcium pyrophosphate dihydrate deposition disease intracranial hypotension. See Hypotension,
(CPPD), II:2-102 to II:2-103 intracranial CSF.
differential diagnosis, II:2-102i, II:2-103 normal pulsations, dural arteriovenous fistula type I
hemodialysis spondyloarthropathy vs., II:2-104 vs., V:1-6i, V:1-7
ochronosis vs., 1:1-164i, 1:1-165 Cerebrospinal fluid (CSF) leakage syndrome, VII: 1-10 to
tumoral calcinosis vs., V:2-21 VII:1-12, VII:1-13i
Capillary hemangioblastoma. See Hemangioblastoma of differential diagnosis, VII:l-10i, VII: 1-11
spinal cord. myelography complications vs., VII: 1-3
Carcinomatosis, myelography complications vs., VII:1-2i subdural abscess vs., III:1-26i, III:1-27
Carcinomatous meningitis subdural hematoma vs., V:1-38i, V:1-39
Guillain-Barre syndrome vs., III:2-2i, III:2-3 Cervical spine
lumbar arachnoiditis vs., III:2-11 acqUired stenosis, II:2-64 to 11:2-66, II:2-67i
spinal meningitis vs., III: 1-47 congenital spinal stenosis vs., 1:1-122i, 1:1-123
Carotid artery dissection, II:1-134 to II: 1-136, II:1-137i C1-C2 osteomyelitis, III:l-14 to III:1-17, III:1-18i
Carpal tunnel syndrome. See Median nerve entrapment. facet arthropathy, 11:2-48 to 11:2-50, II:2-Sli
Cauda equina neoplasms intervertebral disc herniation, 11:2-26 to 11:2-28,
CSF disseminated metastases vs., IV: 1-99 II:2-29i
ependymoma, meningioma vs., IV:1-78i nerve root avulsion, idiopathic brachial plexus
lumbar arachnoiditis vs., III:2-11 neuritis vs., VI:1-22i, VI:I-23
Cauda equina syndrome, of ankylosing spondylitis, peridural calcification of hemodialysis, OPLL vs.,
dural dysplasia vs., 1:1-1l8i, 1:1-119 II:2-80i, 11:2-81
Caudal cell mass anomalies radiculopathy
anterior sacral meningocele, 1:1-44 to 1:1-46, 1:1-47i idiopathic brachial plexus neuritis vs., VI:1-23
caudal regression syndrome, 1:1-32 to 1:1-34, 1:1-3Si median nerve entrapment vs., VI:l-SOi
occult intrasacral meningocele, 1:1-48 to 1:1-50, I:l-Sli suprascapular nerve entrapment vs., VI:1-48i,
sacrococcygeal teratoma, 1:1-52 to 1:1-54, l:l-SSi VI:1-49
terminal myelocystocele, 1:1-40 to 1:1-42, 1:1-43i trauma
tethered spinal cord, 1:1-36 to 1:1-38, 1:1-39i C2 burst fracture, 11:1-24 to 11:1-26, II:1-27i
Caudal regression syndrome, 1:1-32 to 1:1-34, 1:1-3Si cervical burst fracture, 11:1-42 to 11:1-43
Cavernous angioma Hangman's C2 fracture, 11:1-28 to 11:1-30, II:1-3li
central spinal cord syndrome vs., II:1-1l6i, II:1-117 hyperextension injury, 11:1-34 to 11:1-36, II:1-37i
type II AVM vs., V:l-10i, V:1-11 hyperextension-rotation injury, II: 1-44 to II: 1-45
type III AVM vs., V:1-14i, V:1-15 hyperflexion injury, 11:1-32 to 11:1-33
Cavernous malformation, spinal cord, V: 1-22 to V: 1-24, hyperflexion-rotation injury, 11:1-38 to 11:1-40,
V:1-2Si II:1-4li
differential diagnosis, V:1-22i, V:1-22 to V: 1-23 Jefferson C1 fracture, 11:1-16 to 11:1-18, II:1-19i
hemangioblastoma of spinal cord vs., IV:1-115 lateral flexion injury, 11:1-46 to 11:1-47
melanocytoma vs., IV:1-126i, IV:1-126 odontoid C2 fracture, 11:1-20 to 11:1-22, 11:1-231
myelitisccord abscess vs., III:1-43 posterior column injury, 11:1-48 to 11:1-49
spinal cord contusion-hematoma vs., II:1-1l2i, Chance fracture
II:1-113 lumbar
CCA. See Carotid artery dissection. burst fracture vS' II:1-76i, 11:1-77
J
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INDEX
giant cell tumor vs., IV:I-30i, IV:1-31 anterior lumbar, II:1-70 to 11:1-71
hemangiopericytoma vs., IV:1-82i, IV:1-83 anterior thoracic, II: 1-50 to II: I-52, II: 1-S3i
meningioma vs., IV:1-78i Scheuermann disease vs., I:1-146i, 1:1-147
osteoblastoma vs., IV: 1-23 Congenital disorders, 1:1-1 to I: 173. See also
sacral, anterior sacral meningocele vs., 1:1-44i, specific disorder.
1:1-45 Conjoined nerve roots, 1:1-86 to 1:1-88, 1:1-89i
sacrococcygeal teratoma vs., l:l-S2i, 1:1-53 Connective tissue disorders. See also Ehlers-Danlos
CIDP. See Polyneuropathy, chronic inflammatory syndrome; Marfan syndrome.
demyelinating (CIDP). description, 1:1-166 to 1:1-168, I:1-169i
Clay shoveler fracture, cervical hyperextension osteochondroma vs., IV:1-34i, IV:I-35
injury vs., II:1-34i, II:1-35 Contusion-hematoma, spinal cord, II:1-112 to II:l-
Clefts 114,II:1-115i
congenital, cervical hyperextension injury vs., central spinal cord syndrome vs., II:I-116i,
1I:1-34i, II:1-35 II:1-117
Jefferson C1 fracture vs., 1I:1-16i, II:1-17 differential diagnosis, II: 1-112i, II: 1-113
CM. See Chiari entries. myelography complications vs., VII:I-2i,
Collateral venous flow, DAVF type 1vs., V:I-6i, VII: 1-3
V:1-7 vitamin B12 deficiency vs., III:2-32i, III:2-33
Common carotid artery dissection, II:I-134 to II:l- Conus, low-lying
136,1I:1-137i normal variant, tethered spinal cord vs., 1:1-37
Common peroneal neuropathy, VI:I-52 to VI:1-53 post-operative, tethered spinal cord vs., 1:1-37
Compact island. See Bone island. CPN (common peroneal neuropathy), VI:1-S2 to
Composite nerve root sleeve, 1:1-86 to 1:1-88, VI:I-53
1:1-89i CPPD. See Calcium pyrophosphate dihydrate
Compression, external, carotid artery dissection deposition disease (CPPD).
vs., 1I:1-134i, 11:1-135 Craniocervical dissociation, II: 1-10 to II: 1-11
Compression-distraction injury. See Chance Craniovertebral junction variants, 1:1-78 to 1:1-80,
fracture. 1:1-8li
Compression fractures. See also Burst fractures. CRS (caudal regression syndrome), 1:1-32 to 1:1-34,
acute, Schmorl node vs., 1:1-142i, 1:1-143 1:1-3Si
anterior Cryptococcus, superior sulcus tumor vs., VI:1-7
lumbar, II:1-70 to 11:1-71 CSF. See Cerebrospinal fluid (CSF).
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dorsal, 1:1-24 to 1:1-26, I:I-Z7i intervertebral
neurenteric cyst vs., 1:1-60i cervical, 1I:2-Z6 to II:Z-Z8, II:Z-Z9i
Dermoid/epidermoid tumors, 1:1-28 to 1:1-30, lumbar, 11:2-34 to 11:2-36, II:Z-37i
1:1-3li thoracic, 11:2-30 to 11:2-32, II:Z-33i
acquired epidermoid, IV:2-28 to IV:2-29 intravertebral. See Schmorl node.
myxopapillary ependymoma of spinal cord lumbar, back pain in children vs., VIII: 1-7
vs., IV:l-11Oi, IV:l-l11 lumbosacral, femoral neuropathy vs., VI:1-4Zi,
congenital, acquired epidermoid tumor vs., VI:I-43
IV:Z-Z8i metal artifact vs., VII: 1-51
differential diagnosis, I:I-Z8i, 1:1-29 nontraumatic, traumatic disc herniation vs.,
filum terminale fibrolipoma vs., 1:1-90i, 1:1-91 II:1-90i, 11:1-91
iatrogenic, myelography complications vs., post-operative spinal complications vs., VII:1-
VII: 1-3 42i, VII: 1-43
neurenteric cyst vs., 1:1-6Oi recurrent vertebral, VII:1-34 to VII:1-36,
sacrococcygeal teratoma vs., 1:1-5Zi, 1:1-53 VII:1-37i
spinal lipoma vs., 1:1-18i, 1:1-19 peridural fibrosis vs., VII:1-Z4i, VII:I-25
without dermal sinus, dorsal dermal sinus vs., traumatic, II: 1-90 to II: 1-92, II:1-93i
1:1-Z4i, 1:1-25 Disc migration, epidural hematoma vs., V:1-34i,
Desmoid, paraspinal abscess vs., 1l1:1-30i, III:I-31 V:I-35
Developmental spinal stenosis, 1:1-122 to 1:1-124, Disc protrusion. See also Disc extrusion.
1:1-125i focal, anular tear of vertebral disc vs., II:2-22i,
Diabetic neuropathy, idiopathic brachial plexus
neuritis vs., VI:I-23
Diastematomyelia, 1:1-56 to 1:1-58, 1:1-59i
11:2-23
vertebral disc bulge vs., II:Z-18i, 11:2-19
Disc space infection
II
Diastrophic dwarfism accelerated degeneration vs., VII:1-18i, vii
achondroplasia vs., 1:1-153 VII:I-19
congenital scoliosis and kyphosis vs., 1:1-135 degenerative disc disease vs., II:2-6i, 11:2-7
neuromuscular scoliosis vs., I: 1-138 degenerative endplate changes vs., H:2-10i,
Diffuse idiopathic skeletal hyperostosis (DISH), 11:2-11
11:2-76 to 11:2-78, II:2-79i Disc tear, anular, 11:2-22 to 11:2-24, II:2-2Si
Diplomyelia, diastematomyelia vs., l:l-S6i Discitis
Disc bulge, vertebral, 11:2-18 to 11:2-20, II:2-2li anular tear of vertebral disc vs., II:2-22i, 11:2-23
Disc calcification/ossification back pain in children vs., VIII:I-7
apophyseal ring fracture vs., II:I-94i, 11:1-95 calcific juvenile, back pain in children vs.,
calcified fragment, apophyseal ring fracture vs., VIII: 1-7
11:1-95 chronic sequelae of
Disc disease, degenerative, 11:2-6 to 11:2-8, II:2-9i Klippel-FeU spectrum vs./ I:1-74i, 1:1-75
blastic osseous metastases vs., IV:1-6i vertebral segmentation failure vs., I:1-70i,
CPPD vs., II:2-102i, 11:2-103 1:1-71
differential diagnosis, II:2-6i, 11:2-7 hematogenous, post-operative infection vs.,
Kummell disease vs., IV:2-32i, IV:2-33 VII:1-38i
neurogenic (Charcot) arthropathy vs., II:2-72i, Schmorl node vs., I:1-142i, 1:1-143
11:2-73 Discogenic sclerosis, renal osteodystrophy vs.,
ochronosis vs., 1:1-164i V:2-8i, V:2-9
rheumatoid arthritis vs., II:2-89 DISH (diffuse idiopathic skeletal hyperostosis),
Disc extrusion. See also Disc herniation; Disc 11:2-76 to 11:2-78, II:2-79i
protrusion. Disorganized spine. See Vertebral formation and
epidural hematoma vs., V:I-34i, V:I-35 segmentation anomalies.
extruded fragment Dissection
epidural abscess vs., III:1-ZZi, III:I-23 carotid artery, 11:1-134 to 11:1-136, II:1-137i
facet joint synovial cyst vs., II:2-S6i, 11:2-57 vertebral artery, 11:1-130 to 11:1-132, II:l-133i
schwannoma vs., rV:1-86i, IV:I-87 Distraction fracture
foraminal, 11:2-38 to 11:2-40, II:Z-4li low thoracic, II: 1-68 to II: 1-69
Disc herniation thoracic Chance fracture vs., II:1-64i, 11:1-65
acqUired lumbar spinal stenosis vs., II:2-60i, Disuse atrophy, muscle denervation vs., VI:1-1Oi
11:2-61 Diverticulum, spinal nerve root, foraminal disc
calcified extrusion vs., I1:2-38i, 11:2-39
limbus vertebra vs., 1:1-83 Dorsal dermal sinus, 1:1-24 to 1:1-26, 1:1-Z7i
OPLL vs., II:Z-80i, 11:2-81 Dorsal-enteric cyst. See Neurenteric cyst.
conjoined nerve roots vs., 1:1-86i, 1:1-87
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type II pseudomeningocele vs., VII:I-28i, VII:I-29
hemangioma vs., IV:I-14i, IV:I-15 spinal cord herniation vs., 1I:1-I20i, II:I-121
Schmorl node vs., I:l-I42i, 1:1-143 subdural abscess vs., III:I-26i, III:I-27
Enostosis. See Bone island. traumatic disc herniation vs., 1I:1-90i, II:I-91
Enteric cyst. See Neurenteric cyst. Epidural empyema
Entrapment. See Nerve entrapment. epidural-subdural hematoma vs., II:I-I26i,
Eosinophilic granuloma, back pain in children vs., II:I-127
VIII:I-6i, VIII:I-7 spinal cord herniation vs., 1I:1-I20i, II:I-121
Ependymoma, IV:I-I06 to IV: 1-108, IV:I-109i Epidural fibrosis. See Peridural fibrosis.
astrocytoma vs., IV:l-102i, IV: 1-103, IV:I- Epidural hematoma
106i,IV:I-I07 epidural abscess vs., III:I-22i, III:I-23
caudal epidural-subdural, II: 1-126 to II: 1-128, II: I-129i
lumbar arachnoiditis vs., III:2-11 extramedullary hematopoiesis vs., V:2-I6i,
meningioma vs., IV:I-78i V:2-17
cavernous malformation of spinal cord vs., lymphoma vs., IV:I-55
V:I-22i spinal cord herniation vs., II:I-I20i, II:I-121
cellular, spinal cord, IV: 1-106 to IV: 1-108, spontaneous, V:I-34 to V:I-36, V:I-37i
IV:I-I09i subacute, epidural lipomatosis vs., IV:2-I8i,
cysticercosis vs., 1I1:I-S8i, III:I-59 IV:2-19
differential diagnosis, IV:I-106i, IV:I-I07 subdural hematoma vs., V:I-38i, V: 1-39
dural dysplasia vs., 1:1-119 Epidural hemorrhage
hemangioblastoma of spinal cord vs., IV:l-
114i,IV:I-115
intramedullary lymphoma vs., IV: 1-55
acquired lumbar spinal stenosis vs., II:2-61
angiolipoma vs., IV:I-74i
metal artifact vs., VII: I-SOi, VII: 1-5 1
II
lumbar arachnoiditis ossificans vs., III:2-I4i ix
post-operative spinal complications vs.,
melanocytoma vs., IV:l-126i VII:I-43
myelitis-cord abscess vs., III:I-43 subarachnoid hemorrhage vs., V:I-30i, V:I-31
myxopapillary, IV: 1-110 to IV: 1-112, IV:I-113i Epidural lipomatosis, IV:2-18 to IV:2-20, IV:2-2li
acquired epidermoid tumor vs., IV:2-28i angiolipoma vS., IV:I-75
CSF disseminated metastases vs., IV: 1-99 epidural abscess vs., III:I-22i, III:I-23
differential diagnosis, IV:I-llOi, IV:l-l11 epidural fat, epidural hematoma vs., V:I-34i,
myelography complications vs., VII:I-2i, V:I-35
VII: 1-3 epidural-subdural hematoma vs., II:I-I26i,
paraganglioma vs., IV:I-123i, IV:I-124 11:1-127
schwannoma vs., IV:I-87 Epidural lymphoma
non-syndromic, neurofibromatosis type 2 vs., lumbar intervertebral disc herniation vs., 1I:2-34i
1:1-115 mimics of, IV:I-S4i, IV:I-55
spinal cord contusion-hematom~vs., II:I-112i Epidural metastases
spinal cord metastases vs., IV:I-119 epidural hematoma vs., V:I-34i, V:I-35
type III AVM vs., V:I-14i, V:I-15 epidural lipomatosis vs., IV:2-I8i, IV:2-19
type IV AVF vs., V:I-19 extramedullary hematopoiesis vs., V:2-I6i,
ventriculus terminalis vs., 1:1-95 V:2-17
Epicondylitis lumbar intervertebral disc herniation vs., 11:2-
lateral, radial neuropathy vs., VI:I-4Oi 34i, II:2-35
medial, ulnar neuropathy vs., VI:I-45 lymphoma vs., IV:I-55
Epidermoid tumors. See Dermoid/epidermoid Epidural-subdural hematoma, II:I-126 to II:I-128,
tumors. II:I-I29i
Epidural abscess, III: 1-22 to III: 1-24, III:I-2Si Epidural tumor
differential diagnosis, III: 1-22i, III: 1-23 epidural-subdural hematoma vs., 1I:1-I26i,
epidural hematoma vs., V:I-34i, V:I-35 II:I-127
epidural lipomatosis vs., IV:2-I8i, IV:2-19 metastatic, lumbar intervertebral disc
epidural-subdural hematoma vs., II:I-I26i, herniation vs., 1I:2-34i, II:2-35
II:I-127 recurrent vertebral disc herniation vs., VII:I-
extramedullary hematopoiesis vs., V:2-I6i, 34i, VII: 1-35
V:2-17 traumatic disc herniation vs., II:I-90i, II:I-91
lumbar intervertebral disc herniation vs., 11:2- Erythropoietic marrow, myelofibrosis vs., V:2-I4i
34i, II:2-35 Ewing sarcoma, IV:I-50 to IV: 1-52, IV:I-S3i
lymphoma vS., IV:I-5S back pain in children vs., VIII:I-7
peridural fibrosis vs., VII:I-25 differential diagnosis, IV:I-SOi, IV:I-51
post-operative infection vs., VII:I-38i
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INDEX
Langerhans cell histiocytosis vs., IV:2-24i, recurrent vertebral disc herniation as, VII:1-34
IV:2-25 to VII:1-36, VII:1-37i
leukemia vs., IV: 1-59 Failure of vertebral formation/segmentation. See
neuroblastic tumor vs., IV:1-70i, IV:l-71 Vertebral formation and segmentation
osteoid osteoma vs., IV: 1-20 anomalies.
osteosarcoma vs., IV: 1-43 Fatty filum terminale. See Filum terminale
Exostosis. See Osteochondroma. fibrolipoma.
Extension-rotation fractures, cervical posterior Fatty marrow. See also Marrow.
column injury vs., 1I:1-48i focal
External compression, carotid artery dissection vs., anular tear of vertebral disc vs., 1I:2-22i,
1I:1-134i, 11:1-135 11:2-23
Extramedullary hematopoiesis, V:2-16 to V:2-18, hemangioma vs., IV:1-14i, IV:1-15
V:2-19i Schmorl node vs., 1:1-143
paraspinal abscess vs., III:1-30i, III:1-31 heterogeneous, IV:2-22 to IV:2-23
Extravasation of cement. See Cement, extravasation of. normal, post irradiation vertebral marrow vs
Extruded disc. See Disc extrusion. VII:1-58i, VII:1-S9 .,
ossification with, anular tear of vertebral disc
vs., II:2-22i, 11:2-23
F post irradiation vertebral marrow, VII:1-58 to
VII:1-60, VII:1-6li
Facet joints
Femoral nerve metastasis, femoral neuropathy vs.,
arthropathy
VI:1-42i
cervical, 11:2-48 to 11:2-50, 1I:2-5li
Femoral neuropathy, VI:1-42 to VI:1-43
lumbar, 11:2-52 to 11:2-54, 1I:2-55i
Fibrolipoma. See Filum terminale fibrolipoma.
arthrosis, ossification ligamentum flavum vs.,
Fibromuscular disease
1I:2-84i, 11:2-85
carotid artery dissection vs., 1I:1-134i, 11:1-135
fractures
vertebral artery dissection vs., II:l-13Oi, 11:1-131
healing, cervical facet arthropathy vs., 11:2-49
Fibromyolipoma (angiolipoma), IV:1-74 to IV:l-
lumbar facet-posterior fracture, 11:1-80 to
76,IV:1-77i
11:1-81
Fibrosis
thoracic facet-lamina fracture, 11:1-60 to
peridural, VII: 1-24 to VII: 1-26, VII:1-27i
1I:1-62,1I:1-63i
differential diagnosis, VII:1-24i, VII:1-25
jumped facets
lumbar intervertebral disc herniation vs.,
in cervical hyperflexion-rotation injury,
II:2-34i, 11:2-35
11:1-38 to 11:1-40, 1I:1-4li
recurrent vertebral disc herniation vs.,
in cervical lateral flexion injuries, 1I:1-46i
VII:1-24i, VII:1-25, VII:1-34i, VII:1-35
in cervical posterior column injury, II:1-48i
radiation, VI:1-26 to VI:1-28, VI:1-29i
osteoarthritis, septic facet joint arthritis vs.,
superior sulcus tumor vs., VI:1-6i, VI:1-7
III:1-18i, III:1-19
Fibrous dysplasia, IV:2-30 to IV:2-31
osteophyte, foraminal disc extrusion vs., 11:2-
osteoblastoma vs., IV:1-23-24
38i, 11:2-39
Fibrous histiocytoma, malignant
septic chondrosarcoma vs., IV:1-38i, IV:1-39
cervical facet arthropathy vs., 1I:2-48i,
neurogenic (Charcot) arthropathy vs., 1I:2-72i,
II:2-49 11:2-73
lumbar facet arthropathy vs., II:2-42i, 11:2-53
Fifth ventricle. See Ventriculus terminalis.
tumoral calcinosis vs., V:2-20i, V:2-21
Filum terminale fibrolipoma, 1:1-90 to 1:1-91
septic arthritis, III:1-18 to III: 1-20, III:1-2li
differential diagnosis, I:1-90i, 1:1-90 to 1:1-91
differential diagnosis, III:1-18i, III:1-19
spinal lipoma vs., I:1-18i, 1:1-19
facet joint synOVial cyst vs., II:2-56i, 11:2-57
tethered spinal cord vs., I:1-36i
thoracic facet-lamina fracture vs., II:1-60i,
Filum terminale syndrome, tight. See Tethered
11:1-61
spinal cord.
synovial cyst, 11:2-56 to 11:2-58, I1:2-59i
Fistula, arteriovenous. See Arteriovenous fistulas
arachnoid cyst vs., IV:2-li, IV:2-3
(AVFs).
perineural root sleeve cyst vs., IV:2-10i,
Flexion fractures
IV: 2-11
anterior end plate corner, apophyseal ring
septic facet joint arthritis vs., III:1-18i,
fracture vs., II:1-94i, 11:1-95
III:1-19
cervical burst fracture vs., II:1-42i, 11:1-43
Failed back surgery syndrome, VII: 1-46 to VII: 1-
cervical lateral flexion injury vs., II:1-46i
48, VII:1-49i flexion-extension, cervical posterior column
injury vs., II:1-48i
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INDEX
flexion-rotation, cervical posterior column chordoma vs., IV:1-46i, IV:1-47
injury vs., 1I:1-48i differential diagnosis, IV:1-30i, IV:1-31
teardrop fracture, C2 burst fracture vs., 11:1- echinococcus vs., III:1-51
24i, II: 1-25-26 malignant, osteosarcoma vs., IV: 1-43
Flexion injuries pseudomeningocele vs., VII: 1-29
flexion-distraction. See Chance fracture, Gibbs artifact, post-traumatic syrinx associated
thoracic. with, 1I:1-108i, II:1-109
flexion-rotation, cervical hyperflexion injury Glioma. See also Paraganglioma.
vs., 1I:1-32i, II: 1-32 back pain in children vs., VIII: 1-7
lateral cervical, II:1-46 to II:1-47 intramedUllary. See Astrocytoma.
"Floating" lateral mass, cervical lateral flexion post-traumatic syrinx vs., 1I:1-108i
injury vs., 1I:1-46i, II:1-47 Glomus arteriovenous malformations, type II, V:1-
Focal fatty marrow. See Fatty marrow, focal. 10 to V:1-12, V:1-13i
Foramen, cement extravasation into, VII:1-6i, Glomus tumor. See Paraganglioma.
VII:1-7 GM1 gangliosidosis, mucopolysaccharidoses vs.,
Foraminal disc extrusion, II:2-38 to II:2-40, II:2-4li 1:1-156i,I:1-157
Foregut duplication cyst, lateral meningocele vs., "Gorgoylism." See Mucopolysaccharidoses.
1:1-102i,I:1-103 Gout, II:2-100 to II:2-101
Fracture-dislocation, lumbar burst fracture vs., C1-C2 osteomyelitis vs., III:1-16
1I:1-76i, 11:1-77 calcific longus coli tendinitis vs., 1I:2-106i,
Fracture isolation of lateral mass, cervical lateral II:2-107
flexion injury vs., 1I:1-46i, II:1-47
Fractures. See Traumatic injuries; specific type of
fracture.
CPPD vs., II:2-104
degenerative endplate changes vs., II:2-11
differential diagnosis, 1I:2-100i, II:2-100 to
II
Fungal infection xi
II:101
neurogenic (Charcot) arthropathy vs., II:2-72i, hemodialysis spondyloarthropathy vs., II:2-104i
II:2-73 ochronosis vs., 1:1-164
spondylitis, granulomatous osteomyelitis vs., rheumatoid arthritis vs., II:2-89
III:1-11 Graft complications. See Bone graft complications.
Fusion Granulocytic sarcoma. See Leukemia.
congenital spinal, juvenile chronic arthritis vs., Granuloma
1I:2-92i, 11:2-93 eosinophilic, back pain in children vs., VIII:1-
posterior element incomplete, 1:1-22-23 6i, VIII: 1-7
surgical inflammatory, spinal cord metastases vs.,
Klippel-Feil spectrum vs., 1:1-74i, 1:1-75 IV: 1-119
vertebral segmentation failure vs., 1:1-70i, Granulomatous disease
1:1-71 cysticercosis vs., III:1-59
Fusion cages, accelerated degeneration vs., VII: 1- meningitis, Guillain-Barre syndrome vs., 111:2-
18i, VII:1-19 2i, III:2-3
osteomyelitis, III: 1-10 to III:1-12, III:l-13i
Guillain-Barre syndrome, III:2-2 to III:2-4, III:2-5i
G CIDP vs., III:2-6i, III:2-6 to III:2-7
Ganglioglioma, astrocytoma of spinal cord vs., CSF disseminated metastases vs., IV: 1-99
IV:1-103 differential diagnosis, III:2-2i, III:2-3
Ganglion cysts hypertrophic neuropathyvs., VI:1-30i, VI:1-31
common peroneal neuropathy vs., VI:1-52i spinal meningitis vs., 1I1:1-46i, III:1-47
facet joint synovial cyst vs., II:2-56i, II:2-57
recurrent vertebral disc herniation vs., VII:1-35
traumatic neuroma vs., VI:1-16i, VI:1-17 H
Gangliosidosis, GM1, mucopolysaccharidoses vs., Hair patch
1:1-156i,I:1-157 high, diastematomyelia vs., 1:1-56i
Gas, intrathecal, metal artifact vs., VII:1-50i, low, diastematomyelia vs., 1:1-56i
VII:1-51 Hangman's C2 fracture, II: 1-28 to II: 1-30, 1I:1-3li
GCT. See Giant cell tumor. Hardware failure, VII:1-14 to VII:1-16, VII:1-17i
Germ cell tumor of coccyx. See Sacrococcygeal Hardware malposition, VII:1-46i
teratoma. HbSC disease. See Sickle cell.
Giant bone island, osteosarcoma vs., IV:1-43 Hemangioblastoma of spinal cord, IV: 1-114 to
Giant cell tumor, IV:1-30 to IV: 1-32, IV:1-33i IV: 1-116, IV:1-117i
aneurysmal bone cyst vs., IV:1-26i, IV: 1-27 astrocytoma vs., IV:1~103
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INDEX
cellular ependymoma vs., IV:I-I06i, IV: 1-107 differential diagnosis, V:1-38i, V:1-39
contusion-hematoma vs., 1I:1-112i epidural hematoma vs., V:1-35
CSF disseminated metastases vs., IV: 1-99 spinal meningitis vs., III: 1-47
cysticercosis vs., III:1-S8i, III:1-59 subdural abscess vs., I11:1-26i, III:1-27
differential diagnosis, IV:1-1l4i, IV:1-115 Hematopoiesis, extramedullary, V:2-16 to V:2-18,
metastases vs., IV:1-1l8i, IV:1-119 V:2-19i
myelitis-cord abscess vs., III: 1-43 paraspinal abscess vs., III:1-30i, III:1-31
neurofibromatosis type 2 vs., I:1-1l4i, 1:1-115 Hemocystinuria, connective tissue disorders vs.,
syringomyelia vs., IV:2-14i 1:1-167
Hemangiolipoma (angiolipoma), IV:1-74 to IV:1- Hemodialysis
76,IV:1-77i cervical peridural calcification due to, OPLL
Hemangioma, IV:1-14 to IV:1-16, IV:1-17i vs., 1I:2-80i, II:2-81
aggressive, hemangiopericytoma vs., IV:1-82i, spondyloarthropathy due to, II:2-104 to II:2-105
IV: 1-83 C1-C2 osteomyelitis vs., III:1-15 to III:1-16
blastic osseous metastases vs., IV:1-6i, IV:1-7 calcific longus coli tendinitis vs., II:2-107
differential diagnosis, IV:1-14i, IV:1-15 CPPD vs., 1I:2-102i, II:2-103
peripheral nerve tumor vs., VI:1-34i, VI:1-35 degenerative disc disease vs., 1I:2-6i, II:2-7
superior sulcus tumor vs., VI:1-7 degenerative endplate changes vs., 1I:2-10i,
vertebral II: 2-11
Paget disease vs., V:2-2li, V:2-3 differential diagnosis, 1I:2-104i
plasmacytoma vs., IV: 1-63 gout vs., 1I:2-100i, II:2-100
post irradiation vertebral marrow vs., VII:l- ochronosis vs., I:1-164i, 1:1-165
S8i, VII:1-59 pyogenic osteomyelitis vs., III: 1-7
Hemangiopericytoma, IV: 1-82 to IV: 1-84, IV:1-8Si renal osteodystrophy vs., V:2-9
superior sulcus tumor vs., VI:1-7 rheumatoid arthritis vs., 1I:2-88i, II:2-89
Hematogenous-seeded infection, post-operative Hemoglobin HbSS disease. See Sickle cell.
infection vs., VII:1-38i, VII: 1-39 Hemorrhage
Hematologic neoplasms. See also specific type. bone graft complications vs., VII:1-32i, VII: 1-33
blastic osseous metastases vs., IV:1-7 cervical intervertebral disc herniation vs., II:2-27
Langerhans cell histiocytosis vs., IV:2-25 epidural
lytic osseous metastases, IV:l-lOi, IV:1-11 acqUired lumbar spinal stenosis vs., II:2-61
superior sulcus tumor vs., VI:1-7 angiolipoma vs., IV:1-74i
Hematoma metal artifact vs., VII: l-SOi, VII: 1-5 1
angiolipoma vs., IV:1-74i, IV:1-75 post-operative spinal complications vs.,
contusion-hematoma of spinal cord, II: 1-112 to VII: 1-43
II:1-114,1I:1-11Si subarachnoid hemorrhage vs., V:1-30i,
central spinal cord syndrome vs., 1I:1-116i, V:1-31
II:1-117 failed back surgery syndrome vs., VII:1-46i,
CSF leakage syndrome vs., VII:I-IOi, VII: 1-11 VII:1-47
epidural intramedullary, subarachnoid hemorrhage vs.,
epidural abscess vs., III:1-22i, III:1-23 V:1-30i, V:1-31
epidural-subdural, II:1-126 to II:1-128, intraspinal (epidural-subdural hematoma), II:1-
1I:1-129i 126 to II:1-128, 1I:1-129i
extramedullary hematopoiesis vs., V:2-16i, paravertebral, subarachnoid hemorrhage vs.,
V:2-17 V:1-3Oi
lymphoma vs., IV:1-55 post-operative, peridural fibrosis vs., VII:1-24i,
spinal cord herniation vs., 1I:1-120i, II:1-121 VII: 1-25
spontaneous, V:1-34 to V:1-36, V:1-37i recurrent vertebral disc herniation vs., VII:1-35
subacute, epidural lipomatosis vs., IV:2-18i, rhabdomyolysis vs., II:1-99
IV:2-19 subarachnoid, V:1-30 to V:1-32, V:1-33i
subdural hematoma vs., V:1-38i, V:1-39 differential diagnosis, V:1-30i, V:1-31
malignant nerve sheath tumors vs., IV:1-94i, filum terminale fibrolipoma vs., I:1-90i,
IV: 1-95 1:1-91
post-operative, pseudomeningocele vs., VII:l- underlying etiology of, superficial siderosis
28i, VII: 1-29 vs., V:1-43
retroperitoneal, paraspinal abscess vs., 111:1- subdural
30i, III: 1-31 angiolipoma vs., IV:1-74i
rhabdomyolysis vs., II:1-99 post-operative spinal complications vs.,
spontaneous epidural, V:1-34 to V: 1-36, V:1-37i VII:1-42i, VII:1-43
subdural, V:1-38 to V:1-40, V:1-4li
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subarachnoid hemorrhage vs., V:I-30i, Hypertrophic polyradiculoneuropathies,
V:I-31 congenital
superficial siderosis vs., V:I-42i, V:I-43 CSF disseminated metastases vs., IV:I-99
thoracic intervertebral disc herniation vs., neurofibromatosis type 1 vs., I:l-110i, 1:1-111
II:2-31 Hypervitaminosis A or D, osteopetrosis vs., 1:1-162
Hemosiderosis (superficial siderosis), V:I-42 to Hypochondroplasia, achondroplasia vs., I:l-1S~i
V:I-44, V:I-4Si Hypoplastic arch, Jefferson Cl fracture vs., 11:1-161
Hereditary motor-sensory neuropathy. See Hypotension, intracranial CSF
Hypertrophic neuropathy. Chiari I malformation vs., 1:1-98i, 1:1-99
Herniation Chiari II malformation vs., 1:1-6i, 1:1-7
disc. See Disc herniation. myelography complications vs., VII:I-3
spinal cord spinal meningitis vs., III:I-47
idiopathic, arachnoid cyst vs., IV:2-li, IV:2-3 spontaneous. See Cerebrospinal fluid (CSF)
traumatic, 11:1-120 to II:I-122, II:I-123i leakage syndrome.
tonsillar, acquired, Chiari I malformation vs.,
I:I-98i, 1:1-99
Histiocytoma, fibrous malignant I
chondrosarcoma vs., IV:I-38i, IV:I-39
IATM (idiopathic acute transverse myelitis), 11I:2-
neurogenic (Charcot) arthropathy vs., II:2-72i, 28 to III:2-30, III:2-3li
II:2-73
ICA (carotid artery dissection), 11:1-134 to 11:1-136,
Histiocytosis, Langerhans cell, IV:2-24 to IV:2-26, 1I:1-137i
IV:2-27i
differential diagnosis, IV:2-24i, to IV:2-25
Ewing sarcoma vs., IV:I-51
Idiopathic acute transverse myelitis (IATM), 11I:2-
28 to III:2-30, III:2-3li
Idiopathic brachial plexus neuritis. See Brachial
II
leukemia vs., IV:l-S8i, IV:I-59 xiii
plexus neuritis, idiopathic.
HIV myelopathy, 11I:1-38 to III: 1-40, III:l-4li
Idiopathic kyphosis. See Kyphosis, idiopathic.
AIDS-associated polyneuropathy, IV: 1-99
Idiopathic scoliosis. See Scoliosis, idiopathic.
HTLVI (human T-cell leukemia/lymphoma), HIV
Implant (hardware) failure, VII:I-14 to VII:I-16,
myelitis vs., III:I-39
VII:I-17i
Hurler syndrome
Infarction of spinal cord. See Spinal cord
dural dysplasia vs., I:l-118i
infarction.
failure of vertebral formation vs., I:I-64i
Infection, III: 1-6 to III-I-6l. See also Abscess;
Hydatid disease. See Echinococcus.
Empyema; Phlegmon.
Hydrocephalus, severe chronic shunted, Chiari II
acquired lumbar spinal stenosis vs., 1I:2-60i,
malformation vs., 1:1-7
11:2-61
Hydromyelia. See Syringomyelia.
arachnoiditis. See Arachnoiditis.
Hydrosyringomyelia. See Syringohydromyelia.
atypical, neurogenic (Charcot) arthropathy vs.,
Hyperextension injury, cervical, 11:1-34 to 11:1-36,
1I:2-72i, 11:2-73
II:1-37i
congenital scoliosis and kyphosis vs., 1:1-135
Hyperextension-rotation injury, cervical, 11:1-44
disc space
to 11:1-45
accelerated degeneration vs., VII:I-18i,
Hyperflexion injury, cervical, 11:1-32 to 11:1-33
VII:I-19
Hyperflexion-rotation injury, cervical, 11:1-38 to
degenerative disc disease vs., 1I:2-6i, 11:2-7
1I:1-40,II:I-4li
degenerative endplate changes vs., 1I:2-10i,
cervical posterior column injury vs., II:I-48i,
11:2-11
11:1-49
failed back surgery syndrome vs., VII:1-46i,
Hyperostosis, diffuse idiopathic skeletal (DISH),
VII: 1-47
II:2-76 to II:2-78, 1I:2-79i
granulomatous osteomyelitis, III: 1-10 to 11I:1-
Hyperparathyroidism, V:2-6 to V:2-7
12, III:I-13i
brown tumor of, giant cell tumor vs., IV:I-31
hematogenous-seeded, post-operative infection
differential diagnosis, V:2-6i, V:2-7
vs., VII:I-38i, VII:I-39
primary, renal osteodystrophy vs., V:2-8
hemodialysis spondyloarthropathy vs., II:2-104i
Hyperplastic vertebral marrow, V:2-10 to V:2-12,
idiopathic kyphosis vs., 1:1-141
V:2-13i
instability vs., 1I:2-1Si, 11:2-16
multiple myeloma vs., IV:1-66i, IV: 1-67
Kummell disease vs., IV:2-32i
Hypertrophic neuropathy, VI:I-30 to VI:I-32,
local extension, post-operative infection vs.,
VI:I-33i
VII: 1-39
differential diagnosis, VI: 1-30i, VI: 1-31
meningitis. See Meningitis.
neurofibromatosis type 1 vs., l:l-11Oi, 1:1-111
myelitis. See Myelitis.
peripheral neurolymphomatosis vs., VI:I-38i
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INDEX
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L differential diagnosis, I:I-14i, 1:1-15
dorsal spinal meningocele vs., I:I-14i, 1:1-15,
Laminectomy defect, surgical, posterior element I:I-106i, 1:1-107
incomplete fusion vs., 1:1-23 filum terminale fibrolipoma vs., I:I-90i, 1:1-91
Langerhans cell histiocytosis, IV:2-24 to IV:2-26, myelomeningocele vs., I:1-10i
IV:2-27i posterior element incomplete fusion vs., I:1-22i
differential diagnosis, IV:2-24i, to IV:2-25 posterior sacral meningocele vs., IV:2-6i, IV:2-7
Ewing sarcoma vs., IV:1-51 spinal lipoma vs., I:1-18i, 1:1-19
leukemia vs., IV:1-S8i, IV:1-59 tethered spinal cord vs., I:1-36i
Lateral compression fracture Liposarcoma, angiolipoma vs., IV: 1-75
lumbar, II: 1-72 to II:I-74, 1I:1-7Si LMMC. See Lipomyelomeningocele.
thoracic, II:1-54 to II:1-56, 1I:1-S7i Longitudinal ligament, posterior, ossification
Lateral flexion injury, cervical, II:1-46 to II:1-47 (OPLL), II:2-80 to II:2-82, 1I:2-83i
Lateral meningocele. See Meningocele, lateral. acquired cervical spinal stenosis vs., 1I:2-64i,
Leptomeningeal metastases II:2-65
neurofibroma vs., IV:I-90i, IV:1-91 cervical intervertebral disc herniation vs., 11:2-
sarcoidosis vs., III:2-16i 26i, II:2-27
Leukemia, IV:1-58 to IV: 1-60, IV:I-6li differential diagnosis, 1I:2-80i, II:2-81
back pain in children vs., VIII:1-6i, VIII:I-7 epidural-subdural hematoma vs., 1I:1-126i,
blastic osseous metastases vs., IV:1-7 II:1-127
differential diagnosis, IV: 1-S8i, IV: 1-59 metal artifact vs., VII:1-SOi
Ewing sarcoma vs., IV:1-51
heterogeneous fatty marrow vs., IV:2-22i,
IV:2-23
vertebral disc bulge vs., 1I:2-18i, II:2-19
Longus coli tendinitis, calcific, II:2-106 to II:2-107
hemodialysis spondyloarthropathy vs., II:2-104
II
Langerhans cell histiocytosis vs., IV:2-24i xv
Lumbar canal stenosis, type IV AVF vs., V:I-18i,
myelofibrosis vs., V:2-14i V:1-19
sickle cell vs., I:I-16Oi Lumbar spine
Ligamentum flavum acqUired stenosis, II:2-60 to II:2-62, 1I:2-63i
hypertrophy, asymmetric, facet joint synovial congenital spinal stenosis vs., I:I-122i,
cyst vs., II:2-57 1:1-123
ossification, II:2-84 to II:2-86, 1I:2-87i anterior compression fracture, II:1-70 to II:1-71
Limb length inequality arachnoiditis, III:2-10 to III:2-12, III:2-13i
congenital scoliosis and kyphosis vs., 1:1-135 spinal meningitis vs., III:1-46i, III:1-47
idiopathic scoliosis vs., 1:1-131 arachnoiditis ossificans, III:2-14 to III:2-15
neuromuscular scoliosis vs., 1:1-139 burst fractures, II:1-76 to II:1-78, 1I:1-79i
Limbus vertebra, 1:1-82 to 1:1-84, I:I-8Si thoracolumbar, II:1-58 to II:1-59
Schmorl node vs., 1:1-143 facet arthropathy, II:2-52 to II:2-54, 1I:2-SSi
Lipocalcinogranulomatosis (tumoral calcinosis), facet-posterior fracture, II: 1-80 to II: 1-81
V:2-20 to V:2-22, V:2-23i fracture, with dural tear, II:1-124 to II:1-125
Lipoma, 1:1-18 to 1:1-20, I:I-2li intervertebral disc herniation, II:2-34 to II:2-
angiolipoma vs., IV:I-74i, IV:I-75 36, 1I:2-37i
differential diagnosis, I:I-18i, 1:1-19 lateral compression fracture, II: 1-72 to II:1-74,
filum terminale fibrolipoma, 1:1-90 to 1:1-91 1I:1-7Si
intradural (juxtamedullary) plexopathy, femoral neuropathy vs., VI: 1-43
acquired epidermoid tumor vs., IV:2-28i, Lumbosacral disc syndromes, femoral neuropathy
IV:2-29 vs., VI:I-42i, VI:I-43
lipomyelomeningocele vs., I:I-14i, 1:1-15 Lumbosacral dysgenesis. See Caudal regression
intraspinal, filum terminale fibrolipoma vs., syndrome.
I:I-90i Lung neoplasms
terminal non-small cell lung carcinoma, VI:I-7
lipomyelomeningocele vs., I:1-14i, 1:1-15 radiation plexopathy vs., VI:I-26i, VI:1-27
myelomeningocele vs., I:l-IOi LV. See Limbus vertebra.
Lipomatosis, epidural, IV:2-18 to IV:2-20, IV:2-2li Lymphangioma, superior sulcus tumor vs., VI:I-7
angiolipoma vs., IV:I-75 Lymphocytic leukemia. See Leukemia.
epidural abscess vs., 11I:1-22i, III:I-23 Lymphoma, IV:I-54 to IV:1-56, IV:l-S7i
epidural fat, epidural hematoma vs., V:I-34i, astrocytoma of spinal cord vs., IV:I-103
V:1-35 back pain in children vs., VIII:1-7
epidural-subdural hematoma vs., 1I:1-126i, blastic osseous metastases vs., IV:I-7
II:I-127 chondrosarcoma vs., IV:I-39
Lipomyelomeningocele, 1:1-14 to 1:1-16, I:I-17i chordoma vs., IV:I-46i, IV:I-47
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INDEX
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perineural root sleeve cyst vs., IV:2-10i, cavernous malformation of spinal cord vs., V:1-
IV: 2-11 22i, V:I-23
sacrococcygeal teratoma vs., 1:1-52i, 1:1-53 cervical facet arthropathy vs., I1:2-48i, 11:2-49
terminal myelocystocele vs., 1:1-4Oi, 1:1-41 chondrosarcoma vs., IV:1-38i, IV:I-39
anterior thoracic, neurenteric cyst vs., 1:1-61 chordoma vs., IV:1-46i, IV:I-47
distal intrasacral/sacral CSF disseminated, IV: 1-98 to IV: 1-100, IV:I-I0li
perineural root sleeve cyst vs., IV:2-10i, degenerative endplate changes vs., I1:2-10i,
IV: 2-11 11:2-11
posterior sacral meningocele vs., IV:2-6i, drop, neurofibromatosis type 2 vs., I:1-114i
IV:2-7 echinococcus vs., III:I-50i, III:I-51
dorsal spinal, 1:1-106 to 1:1-108, 1:1-109i epidural
lipomyelomeningocele vs., 1:1-14i, 1:1-15, epidural hematoma vs., V:I-34i, V:I-35
1:1-106i, 1:1-107 epidural lipomatosis vs., IV:2-18i, IV:2-19
myelomeningocele vs., 1:1-10i, 1:1-11 extramedullary hematopoiesis vs., V:2-16i,
occult intrasacral meningocele vs., 1:1-49i, . V:2-17
1:1-48 lumbar intervertebral disc herniation vs.,
posterior element incomplete fusion vs., I1:2-34i, 11:2-35
1:1-22i lymphoma vs., IV:I-55
lateral, 1:1-102 to 1:1-104, 1:1-105i Ewing sarcoma vs., IV:I-51
brachial plexus traction injury vs., VI:1- extradural, epidural abscess vs., III:I-22i, III:I-23
12i, VI:I-13 femoral neuropathy vs., VI:I-42i
CIDP vs., III:2-6i, III:7
differential diagnosis of, 1:1-102i, 1:1-103
lateral thoracic, perineural root sleeve cyst vs.,
gas due to, Kummell disease vs., IV:2-32i, IV:2-33
giant cell tumor vs., IV:I-30i, IV:I-31
granulomatous osteomyelitis vs., I1I:I-I0i,
II
IV:2-lOi,IV:2-11 111:1-11 xvii
neurofibroma vs., IV:1-90i, IV:I-91 hemangioblastoma of spinal cord vs., IV:I-114i
occult, tethered spinal cord vs., 1:1-36i hemorrhagic, type III AVM vs., V:I-14i, V:I-15
occult intrasacral, 1:1-48 to 1:1-50, 1:1-5li heterogeneous fatty marrow vs., IV:2-22i,
caudal regression syndrome vs., 1:1-32i, IV:2-23
1:1-33 hyperplastic vertebral marrow vs., V:2-10i,
differential diagnosis, I: 1-48i, I: 1-49 V:2-11
perineural root sleeve cyst vs., IV:2-10i, idiopathic brachial plexus neuritis vs., VI:l-
IV:2-11 . 22i, VI:I-23
perineural root sleeve cyst vs., IV:2-10i, IV:2-11 intradural
posterior sacral, IV:2-6 to IV:2-8, IV:2-9i acquired epidermoid tumor vs., IV:2-28i
perineural root sleeve cyst vs., IV:2-10i, lumbar arachnoiditis vs., I1I:2-10i, III:2-11
IV:2-11 myxopapillary ependymoma of spinal cord
pseudomeningocele vs., VII: 1-29 vs., IV:I-110i, IV:l-l11
schwannoma vs., IV:1-86i, IV:I-87 sarcoidosis vs., III:2-16i, III:2-17
simple dorsal, terminal myelocystocele vs., 1:1- intramedullary lymphoma vs., IV: 1-55
40i,I:I-41 intrathecal primary neoplasm, lumbar
Meningocele manque, diastematomyelia vs., 1:1-56i arachnoiditis ossificans vs., I11:2-14i
Mesothelioma, superior sulcus tumor vs., VI:I-7 Langerhans cell histiocytosis vs., IV:2-25
Metabolic disorders, osteochondroma vs., IV:1- leptomeningeal
34i, IV: 1-35 neurofibroma vs., IV:I-90i, IV:I-91
Metal artifact, VII: 1-50 to VII: 1-52, VII:1-53i sarcoidosis vs., I1I:2-16i
Metal (hardware) failure, VII:I-14 to VII:I-16, leukemia vs., IV:l-S8i, IV:l-S9
VII:1-17i lytic osseous, IV:I-I0 to IV:I-12, IV:1-13i
Metastatic disease meningitis vs., III:1-46i
acqUired lumbar spinal stenosis vs., I1:2-60i, multiple myeloma vs., IV:I-66i, IV:I-67
11:2-61 neuroblastoma
aneurysmal bone cyst vs., IV:1-26i, IV:I-27 Ewing sarcoma vs., IV:I-51
astrocytoma of spinal cord vs., IV:1-102i, leukemia vs., IV:1-S8i, IV:l-S9
IV: 1-103 neurofibromatosis type 2 vs., I:1-114i
back pain in children vs., VIII:I-7 occipital condyle fracture vs., I1:1-12i, 11:1-13
blastic, osteopetrosis vs., I:1-162i odontoid C2 fracture vs., I1:1-20i, 11:1-21
blastic osseous, IV: 1-6 to IV: 1-8, IV:1-9i osteoblastic, Paget disease vs., V:2-2li, V:2-3
bone island vs., 1:1-92i, 1:1-93 osteoblastoma vs., IV:1-22i, IV:I-23
CI-C2 osteomyelitis vs., III:1-14i, III:I-15 paraganglioma vs., IV:1-123i, IV:I-124
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INDEX
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INDEX
spinal cord metastases vs., IV: 1-119 radiation, VII:1-54 to VII:I-56, VII:1-S7i
mycoplasmal, viral myelitis vs., III:I-35 Myeloproliferative disorder, hyperplastic vertebral
post-traumatic syrinx vs., II: 1-109 marrow vs., V:2-10i, V:2-11
spinal cord contusion-hematoma vs., II:I-I13 Myelosclerosis. See Myelofibrosis.
spinal cord multiple sclerosis vs., III:2-20i, Myonecrosis. See Rhabdomyolysis.
III:2-21 Myositis
viral, III: 1-34 to III: 1-36, III:1-37i idiopathic brachial plexus neuritis vs., VI:1-23
acute, myelitis-cord abscess vs., III:1-42i, muscle denervation vs., VI:1-1Oi
III: 1-43 peripheral neuropathy vs., VIII:1-12i
ADEM vs., III:2-25 radiation, idiopathic brachial plexus neuritis
schistosomiasis vs., III:1-54i, III:I-55 vs., VI:1-22i, VI:I-23
Myelitis, transverse Myositis ossificans
acute cervical facet arthropathy vs., II:2-48i, II:2-49
Guillain-Barre syndrome vs., III:2-3 chondrosarcoma vs., IV:1-38i, IV:1-39
secondary acute transverse myelitis vs., rhabdomyolysis vs., II:1-98i, II:1-99
VIII:1-2i, VIII:I-3 superior sulcus tumor vs., VI:I-7
vitamin B12 deficiency vs., III:2-33 Myxopapillary ependymoma. See Ependymoma,
central spinal cord syndrome vs., II:1-116i, myxopapillary.
II:I-117
HIV myelitis vs., III:I-39
idiopathic N
acute (IATM), III:2-28 to III:2-30, III:2-3li
cellular ependymoma of spinal cord vs./
IV:I-I06i,IV:I-I07
Nasopharyngeal carcinoma, CI-C2 osteomyelitis
vs., III:I-15
NCe. See Cysticercosis.
II
xix
sarcoidosis vs./ III:2-17 Necrosis, avascular
schistosomiasis vs./ III:I-54i, III:I-55
lytic osseous metastases, IV:1-10i, IV:l-11
spinal cord infarction vs., V:I-27
post-traumatic. See Kummell disease.
viral myelitis vs., III:I-34i, III: 1-34 to III: 1-35
Neoplasms/tumors, IV:I-6 to IV:I-127. See also
radiation myelopathyvs., VII:I-54i, VII:I-55
Metastatic disease; Pathologic fractures;
secondary acute, VIII: 1-2 to VIII: 1-4, VIII:I-5i
specific neoplasm or tumor.
Myelitis-cord abscess, III: 1-42 to III: 1-44, III: 1-4Si
back pain in children vs., VIII:I-7
Myelocele, lipomyelomeningocele vs., 1:1-14i,
cervical facet arthropathy vs., II:2-48i, II:2-49
1:1-15
cervical intervertebral disc herniation vs., II:2-27
Myelocystocele, terminal, 1:1-40 to 1:1-42, 1:1-43i
cysticercosis vs., III:1-S8i, III:I-59
dorsal spinal meningocele vs., I:I-107i, 1:1-106
degenerative scoliosis vs., II:2-68i, II:2-69
Myelofibrosis, V:2-14 to V:2-15
extradural, IV:l-l to IV: 1-77
differential diagnosis, V:2-14i
failed back surgery syndrome vs., VII:I-47
heterogeneous fatty marrow vs., IV:2-23
instability vs., II:2-15i, II:2-16
osteopetrosis vs., 1:1-163
intradural/extramedullary, IV:I-78 to IV:I-I00.
Myelogenous leukemia. See Leukemia.
See also specific neoplasms.
Myelography complications, VII: 1-2 to VII: 1-4,
acquired epidermoid tumor vs., IV:2-28i,
VII:l-Si
IV:2-29
Myeloma
hemangioblastoma of spinal cord vs.,
Ewing sarcoma vs., IV:l-SOi, IV:I-51
IV:1-115
giant cell tumor vs., IV:I-31
meningioma vs., IV:I-79
Myelomalacia
myelitis-cord abscess vs., III:1-43
post-traumatic syrinx vs., II:I-I09
pigmented, melanocytoma vs., IV:l-127
spinal cord contusion-hematoma vs., II:I-113
syringomyelia vs., IV:2-14i, IV:2-15 type II AVM vs., V:1-10i, V:l-11
type III AVM vs., V:1-15
ventriculus terminalis vs., 1:1-94i, 1:1-95
with prominent vascular supply, type IV
Myelomeningocele, 1:1-10 to 1:1-12, l:l-13i
AVF vs., V:1-18i, V:1-19
differential diagnosis, 1:1-10i, 1:1-11
intramedullary, IV:I-I02 to IV:l-127. See also
dorsal spinal meningocele vs., I:1-107i, 1:1-106
specific neoplasms.
lipomyelomeningocele vs., I:1-14i, 1:1-15
posterior sacral meningocele vs., IV:2-6i, IV:2-7 cavernous malformation of spinal cord vs.,
V:1-22i
terminal myelocystocele vs., I:l-4Oi, 1:1-41
tethered spinal cord vs., 1:1-37 sarcoidosis vs., III:2-16i, III:2-17
schistosomiasis vs., III:I-55
Myelopathy
HIV-related, III: 1-38 to III: 1-40, III:l-4li spinal cord multiple sclerosis vs., III:2-20i,
III:2-21
noncompressive, VIII: 1-2
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INDEX
type II AVM vs., V:l-10i, V:l-11 traumatic neuroma vs'} VI:I-16i} VI:I-17
type III AVM vs'} V:I-14i} V:I-15 myxopapillary ependymoma of spinal cord vs.,
type IV AVF vs., V:I-19 IV:l-l11
intrathecal primary} lumbar arachnoiditis neuroblastic tumor vs., IV: 1-71
ossificans vs., 1I1:2-14i perineural root sleeve cyst vs'} IV:2-11
lumbar facet arthropathy vs., 1I:2-42i} 11:2-53 peripheral
neurogenic (Charcot) arthropathy vs., 1I:2-72i} benign, traumatic neuroma vs'} VI:I-17
11:2-73 malignant, traumatic neuroma vs., VI:I-16i}
perineural root sleeve cyst vs'} IV:2-11 VI:I-17
post-laminectomy spondylolisthesis vs., VII: 1- median nerve entrapment vs., VI:I-50i,
22i} VII: 1-23 VI:I-51
post-operative infection vs., VII: 1-39 peripheral neuropathy vs., VIII:I-12i
primary, paraspinal abscess vs., III:I-31 ulnar neuropathy vs., VI:I-44i} VI:I-45
recurrent, traumatic neuroma vs., VI:I-16i} Nerve trauma} acute direct, ulnar neuropathy vs.,
VI:I-17 VI:I-44i} VI:I-45
sacral insufficiency fracture vs., 1I:1-86i} 11:1-87 Neural (vertebral) arch fracture, II: 1-60 to II: 1-62,
sacral traumatic fracture vs., 1I:1-82i} 11:1-83 II:I-63i
thoracic intervertebral disc herniation vs., 11:2- Neuralgic amyotrophy. See Brachial plexus neuritis,
30i} 11:2-31 idiopathic.
traumatic spinal muscle injury vs., 11:1-102 Neurenteric cyst} 1:1-60 to 1:1-62, I:I-63i
with paramagnetic effects, filum terminale anterior sacral meningocele vs., I:I-44i} 1:1-45
fibrolipoma vs'} 1:1-91 differential diagnosis, I:I-60i} 1:1-61
Nerve entrapment epidermoid tumors vs., I:I-28i} 1:1-29
lumbar fracture with dural tear} 11:1-124 to Neurilemmoma. See Schwannoma.
11:1-125 Neurinoma. See Schwannoma.
median, VI:I-50 t9 VI:I-51 Neuritis
radial neuropathy vs'} VI:I-40 brachial plexus, idiopathic, VI:I-22 to VI:I-24,
posterior tibial, VI:I-54 to VI:I-55 VI:I-25i
suprascapular, VI:I-48 to VI:I-49 inflammatory, neurofibroma vs., IV:I-90i,
idiopathic brachial plexus neuritis vs'} VI:l- IV:I-91
22i} VI: 1-23 neoplastic, neurofibroma vs., IV:I-90i} IV:I-91
ulnar nerve, VI:I-44 to VI:I-46, VI:I-47i peripheral nerve tumor vs., VI:I-34i} VI:I-35
Nerve root(s) radiation, idiopathic brachial plexus neuritis
avulsion of vs., VI:I-22i} VI:I-23
arachnoid cyst vs., IV:2-3 viral, common peroneal neuropathy vs.,
perineural root sleeve cyst vs., IV:2-11 VI:I-52i
conjoined, 1:1-86 to 1:1-88} I:I-89i Neuroblastoma} IV:I-70 to IV:I-72} IV:I-73i
diverticulum of. See Perineural root sleeve cyst. cystic, anterior sacral meningocele vs., I:I-44i}
physiological enhancement} Guillain-Barre 1:1-45
syndrome vs., III:2-3 differential diagnosis, IV:I-7Oi
sleeve cyst. See Perineural root sleeve cyst. Langerhans cell histiocytosis vs., IV:2-25
thick, CSF disseminated metastases vs., IV:I-99 leukemia vs., IV:I-58i} IV:I-59
tortuous, DAVF type I vs., V:I-6i} V:I-7 metastatic
Nerve sheath tumors. See also Neurofibroma. back pain in children vs., VIII: 1-7
benign peripheral, malignant nerve sheath Ewing sarcoma vs., IV:I-51
tumors vs., IV: 1-95 Neurocysticercosis, III: 1-58 to III: 1-60, III:I-6li
brachial plexus traction injury vs'} VI:I-12i, Neuroectodermal tumor, primitive (PNET), Ewing
VI:I-13 sarcoma vs., IV:I-50i} IV:I-51
common peroneal neuropathy vs., VI:I-52i Neurofibroma, IV: 1-90 to IV: 1-92, IV:I-93i. See
conjoined nerve roots vs'} I:I-86i} 1:1-87 also Nerve sheath tumors.
connective tissue disorders vs., 1:1-167 conjoined nerve roots vs., I:I-86i} 1:1-87
dural dysplasia vs., 1:1-119 differential diagnosis, IV:I-90i} IV:I-91
facet joint synovial cyst vs'} 1I:2-56i} 11:2-57 extramedullary hematopoiesis vs., V:2-17
femoral neuropathy vs., VI:I-42i lateral meningocele vs., I:I-I02i, 1:1-103
hypertrophic neuropathy vs., VI:I-31 plexiform
lateral meningocele vs., I:I-I02i} 1:1-103 femoral neuropathy vs., VI:I-42i
lumbar intervertebral disc herniation vs., 11:2- hypertrophic neuropathy vs., VI:I-30i}
34i} 11:2-35 VI:I-31
malignant peripheral, IV:I-94 to IV:I-96, pseudomeningocele vs., VII: 1-29
IV:I-97i radial neuropathy vs., VI:I-4Oi
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INDEX
radiation plexopathy vs., VI:1-26i, VI:I-27 polyneuropathy. See Polyneuropathy, chronic
thoracic outlet syndrome vs., VI:1-18i inflammatory demyelinating (CIDP).
schwannoma vs., IV:1-86i, IV:I-87 polyradiculoneuropathy. See
spinal cord metastases vs., IV: 1-119 Polyradiculoneuropathy.
Neurofibromatosis radial, VI: 1-40 to VI: 1-41
congenital scoliosis and kyphosis vs., 1:1-135 ulnar, VI:I-44 to VI: 1-46, VI:1-47i
connective tissue disorders vs., I:1-166i, 1:1-167 vasculitic, Guillain-Barre syndrome vs., III:2-3
fibrous dysplasia vs., IV:2-31 Neurosarcoidosis. See Sarcoidosis.
neuromuscular scoliosis vs., 1:1-138 Neurulation abnormalities, 1:1-6 to 1:1-30
Neurofibromatosis type 1, 1:1-110 to 1:1-112, Chiari II malformation, 1:1-6 to 8, I:I-9i
I:1-113i dermoid and epidermoid tumors. See Dermoid/
CIDP vs., III:2-6i, III:7 epidermoid tumors.
Neurofibromatosis type 2, 1:1-114 to 1:1-116, dorsal dermal sinus, 1:1-24 to 1:1-26, I:1-27i
1:1-117i lipomyelomeningocele. See
differential diagnosis, I:1-114i, 1:1-115 Lipomyelomeningocele.
hemangioblastoma of spinal cord vs., IV:1-114i myelomeningocele. See Myelomeningocele.
neurofibromatosis type 1 vs., I:1-110i, 1:1-111 posterior element incomplete fusion, 1:1-22 to
Neurofibrosarcoma. See Peripheral nerve sheath tumors. 1:1-23
Neurogenic (Charcot) arthropathy, 11:2-72 to 11:2- spinal lipoma, 1:1-18 to 1:1-20, I:1-2li
74,1I:2-75i Newborn, normal, osteopetrosis vs., I:1-162i
CIDP vs., III:2-6i, III: 7 NF. See Neurofibromatosis entries.
CSF disseminated metastases vs., IV: 1-99
differential diagnosis, 1I:2-72i, 11:2-73
gout vs., 1I:2-100i, 11:2-100
Non-neoplastic cysts. See Cysts.
Non-small cell lung carcinoma, VI:I-7
Notochord developmental anomalies
II
xxi
hemodialysis spondyloarthropathy vs., 1I:2-104i diastematomyelia, 1:1-56 to 1:1-58, 1:1-59i
pathologic vertebral fracture vs., VIII:1-1Si neurenteric cyst, 1:1-60 to 1:1-62, I:1-63i
pyogenic osteomyelitis vs., III: 1-7
scoliosis vs., 1:1-128
tumoral calcinosis vs., V:2-21 o
Neurogenic sarcoma. See Peripheral nerve sheath
OB. See Osteoblastoma.
tumors.
Oc. See Osteochondroma.
Neurolymphomatosis
Occipital condyle
femoral neuropathy vs., VI: 1-42i
fractures, 11:1-12 to 11:1-14, 1I:1-1Si
peripheral, VI:I-38 to VI:I-39
atlanto-occipital dislocation vs., 1I:1-10i,
Neuroma, traumatic, VI:I-16 to VI:I-17
11:1-10
peripheral nerve tumor vs., VI:I-34i
third, odontoid C2 fracture vs., 1I:1-20i, 11:1-21
Neuromuscular disease
Occult intrasacral meningocele. See Meningocele.
degenerative scoliosis vs., 1I:2-68i, 11:2-69
Ochronosis, 1:1-164 to 1:1-165
idiopathic kyphosis vS., 1:1-141
CPPD vs., 1I:2-102i, 11:2-104
Scheuermann disease vs., 1:1-147
Odontoid C2 fracture, 11:1-20 to 11:1-22, 1I:1-23i
Neuromuscular scoliosis. See Scoliosis,
dens type II, craniovertebral junction variants
neuromuscular.
vs.,I:1-78i
Neuromyelitis optica
Odontoid fracture
secondary acute transverse myelitis vs., VIII:I-3
CI-C2 osteomyelitis vs., III:I-15
viral myelitis vs., III:I-35
type 3, C2 burst fracture vs., 1I:1-24i, 11:1-25
Neuropathic arthropathy/joint. See Neurogenic
Odontoid process, congenital nonunion of,
(Charcot) arthropathy.
odontoid C2 fracture vs., 1I:1-20i, 11:1-21
Neuropathy
OGS. See Osteosarcoma.
common peroneal, VI:I-52 to VI:I-53
01. See Osteogenesis imperfecta.
diabetic, idiopathic brachial plexus neuritis vs.,
OIM. See Meningocele, occult intrasacral.
VI:I-23
00. See Osteoid osteoma.
femoral, VI: 1-42 to VI: 1-43
OPLL. See Longitudinal ligament, posterior,
hypertrophic, VI:I-30 to VI:I-32, VI:1-33i
ossification (OPLL).
differential diagnosis, VI:1-30i, VI:I-31
Os odontoideum, odontoid C2 fracture vs., 11:1-
neurofibromatosis type 1 vs., I:1-110i, 1:1-
20i,1I:1-21
111
Osseous metastases
peripheral neurolymphomatosis vs., VI:1-38i
blastic, IV:I-6 to IV: 1-8, IV:1-9i
inherited demyelinating, CIDP vs., III:2-6i, III:7
lytic, IV:I-I0 to IV:I-12, IV:l-13i
peripheral, VIII:I-12 to VIII: 1-13
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INDEX
Osseous tumor mimics, lymphoma vs., IV:1-S4i, dural dysplasia vs., 1:1-119
IV:1-55 Scheuermann disease vs., 1:1-147
Ossification Osteogenic sarcoma (OGS). See Osteosarcoma.
disc, apophyseal ring fracture vs., 1I:1-94i, Osteoid osteoma, IV:I-18 to IV: 1-20, IV:1-2li
11:1-95 back pain in children vs., VIII:1-6i, VIII: 1-7
ligamentum flavum, 11:2-84 to 11:2-86, 1I:2-87i bone island vs., 1:1-93
normal progression of, posterior element differential diagnosis, IV:1-18i, IV:I-19 to
incomplete fusion vs., 1:1-23 IV: 1-20
paraspinalligament, calcific longus coli fibrous dysplasia vs., IV:2-30i, IV:2-31
tendinitis vs., 1I:2-106i, 11:2-107 idiopathic scoliosis vs., 1:1-130i
posterior longitudinal ligament (OPLL), 11:2-80 osteoblastoma vs., IV:1-22i, IV: 1-23
to 11:2-82, 1I:2-83i Osteomalacia, renal osteodystrophy vs., V:2-9
acquired cervical spinal stenosis vs., 11:2- Osteomyelitis
64i, 11:2-65 back pain in children vs., VIII: 1-7
cervical intervertebral disc herniation vs., cervical
1I:2-26i, 11:2-27 CI-C2, 111:1-14 to 111:1-17, III:1-18i
differential diagnosis, 1I:2-80i, 11:2-81 calcific longus coli tendinitis vs., 1I:2-106i,
epidural-subdural hematoma vs., 1I:1-126i, 11:2-107
11:1-127 Ewing sarcoma vs., IV:1-SOi, IV:I-51
metal artifact vs., VII:1-S0i gout vs., 1I:2-100i, 11:2-100
vertebral disc bulge vs., 1I:2-18i, 11:2-19 granulomatous, III: 1-10 to III: 1-12, 11I:1-13i
with fatty marrow, anular tear of vertebral disc hemodialysis spondyloarthropathy vs., 1I:2-104i
vs., 1I:2-22i, 11:2-23 hyperplastic vertebral marrow vs., V:2-1Oi
Ossification center(s), accessory Langerhans cell histiocytosis vs., IV:2-24i,
occipital condyle fracture vs., 1I:1-12i, 11:1-13 IV:2-25
sacral traumatic fracture vs., II: 1-82i, II: 1-83 leukemia vs., IV:1-S8i, IV:1-59
Osteitis deformans. See Paget disease. occipital condyle fracture vs., 1I:1-12i, 11:1-13
Osteoarthritis osteoid osteoma vs., IV:1-18i, IV:I-19 to IV: 1-20
CI-C2 osteomyelitis vs., III:I-15 osteosarcoma vs., IV:1-42i, IV:I-43
facet joint, septic facet joint arthritis vs., 111:1- pyogenic, III: 1-6 to III: 1-8, III:1-9i
18i, III:I-19 sacral insufficiency fracture vs., 1I:1-86i, 11:1-87
ochronosis vs., 1:1-164 scoliosis vs., 1:1-126i, 1:1-127
sacral insufficiency fracture vs., 1I:1-86i, 11:1-87 skull base, CI-C2 osteomyelitis vs., III:I-15
Osteoblastic metastases, Paget disease vs., V:2-2li, thoracic facet-lamina fracture vs., 1I:1-60i,
V: 2-3 11:1-61
Osteoblastoma, IV: 1-22 to IV: 1-24, IV:1-2Si tubercular, Langerhans cell histiocytosis vs.,
aneurysmal bone cyst vs., IV:1-26i, IV:I-27 IV:2-24i
back pain in children vs., VIII:1-6i, VIII:I-7 tubercular vertebral, pyogenic osteomyelitis
degenerative scoliosis vs., 1I:2-68i, 11:2-69 vs., 1I1:1-6i, III:1-7
differential diagnosis, IV:1-22i, IV: 1-23 to vertebral, due to vertebroplasty, VII:I-7
IV: 1-24 Osteonecrosis, blastic osseous metastases vs.,
fibrous dysplasia vs., IV:2-30i, IV:2-31 IV:1-6i
giant cell tumor vs., IV:1-30i, IV:I-31 Osteopetrosis, 1:1-162 to 1:1-163
osteochondroma vs., IV:1-34i, IV:I-35 myelofibrosis vs., V:2-14i
osteoid osteoma vs., IV:1-18i, IV:I-19 renal osteodystrophy vs., V:2-8i, V:2-9
osteosarcoma vs., IV:1-42i, IV:I-43 sickle cell vs., 1:1-160i, 1:1-161
Osteochondroma, IV:I-34 to IV:I-36, IV:1-37i Osteophyte
Osteodystrophy, renal. See Renal osteodystrophy. cervical intervertebral disc herniation vs., 11:2-
Osteogenesis imperfecta, 1:1-170 to 1:1-172, 1:1-173i 26i, 11:2-27
achondroplasia vs., 1:1-1S3i endplate, foraminal disc extrusion vs., 1I:2-38i,
congenital scoliosis and kyphosis vs., 1:1-135 11:2-39
connective tissue disorders vs., 1:1-166i, 1:1-167 fragment, anterior, limbus vertebra vs., 1:1-82i,
differential diagnosis, 1:1-170i, 1:1-171 1:1-83
idiopathic scoliosis vs., 1:1-130i, 1:1-131 metal artifact vs., VII: 1-51
juvenile chronic arthritis vs., 1I:2-92i, 11:2-93 posterior, apophyseal ring fracture vs., 11:1-95
neuromuscular scoliosis vs., 1:1-138 recurrent vertebral disc herniation vs., VII:1-35
pathologic vertebral fracture vs., VIII:1-1Si, thoracic intervertebral disc herniation vs., 11:2-31
VIII: 1-16 Osteopoikilosis, bone island vs., 1:1-93
thanatophoric dwarfism vs., 1:1-150 Osteoporosis
Osteogenesis imperfecta tarda multiple myeloma vs., IV:1-66i, IV:I-67
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INDEX
osteogenesis imperfecta vs., I:I-17li pseudomeningocele vs., VII: 1-29
senile, hyperparathyroidism vs., V:2-6i, V:2-7 Paraspinalligament ossification, calcific longus
Osteoporotic compression fractures. See coli tendinitis vs., II:2-106i, II:2-107
Compression fractures, osteoporotic. Paravertebral abscess, extramedullary
Osteosarcoma, IV: 1-42 to IV: 1-44, IV:I-4Si hematopoiesis vs., V:2-16i, V:2-17
chondrosarcoma vs., IV:I-38i, IV:I-39 Paravertebral hemorrhage, subarachnoid
differential diagnosis, IV: 1-42i, IV: 1-43 hemorrhage vs., V:I-3Oi
echinococcus vs., 1I1:1-SOi, III:I-51 Pars interarticularis
Ewing sarcoma vs., IV:I-51 fracture (spondylolysis), lumbar facet-posterior
giant cell tumorvs., IV:I-31 fracture, II: 1-80 to II: 1-81
neurogenic (Charcot) arthropathy vs., 1I:2-72i, unilateral absent, osteoid osteoma vs., IV:I-18i,
II:2-73 IV:I-19
osteoblastoma vs., IV:I-22i, IV: 1-23 Parsonage-Turner syndrome. See Brachial plexus
pseudomeningocele vs., VII:I-28i, VII: 1-29 neuritis, idiopathic.
telangiectatic, aneurysmal bone cyst vs., IV:l- Partial vertebral duplication, I: 1-68-69
26i,IV:I-27 Pathologic fractures
tumoral calcinosis vs., V:2-2Oi anterior thoracic compression fracture vs.,
Osteosclerosis (dense vertebral body), VIII:I-18 to 1I:1-5li
VIII: 1-19 C2 burst fracture vs., 1I:1-24i, II:I-25
Osteosclerotic metastases. See Metastatic disease, compression fractures
sclerotic. burst thoracolumbar fracture vs., 1I:1-S8i,
Ovarian cyst, anterior sacral meningocele vs., 1:1-
44i,I:I-45
II:I-59
cervical burst fracture vs., 1I:1-42i, II:I-43
thoracic Chance fracture vs., II:I-65
II
xxiii
lumbar anterior compression fracture vs., 11:1-
p 70i,II:1-71
PA. See Paraspinal abscess. lumbar burst fracture vs., 1I:1-76i, II:I-77
Pachymeningitis, idiopathic hypertrophic odontoid C2 fracture vs., 1I:1-20i, II:I-21
spinal meningitis vs., III:I-47 thoracic facet-lamina fracture vs., 1I:1-60i,
subdural abscess vs., III:I-27 II:I-61
subdural hematoma vs., V:I-39 vertebral, VIII: 1-14 to VIII: 1-16, VIII:I-17i
Pachymeningopathies, CSF leakage syndrome vs., Pedicle, unilateral absent, osteoid osteoma vs.,
VII: 1-11 IV:I-18i, IV:I-19
Paget disease, V:2-2 to V:2-4, V:2-Si Pedicle insufficiency fracture, II:I-I04 to II:I-I06,
acqUired lumbar spinal stenosis vs., 1I:2-60i, 1I:1-107i
II:2-61 Pelvic ring (sacral traumatic) fractures, II:I-82 to
blastic osseous metastases vs., IV:I-6i, IV:I-7 II:I-84,1I:1-8Si
cervical facet arthropathy vs., 1I:2-48i, II:2-49 Peridural fibrosis, VII: 1-24 to VII: 1-26, VII:I-27i
differential diagnosis, V:2-2i, V:2-3 differential diagnosis, VII:I-24i, VII: 1-25
hemangioma vs., IV:I-14i, IV:I-15 lumbar intervertebral disc herniation vs., 11:2-
lumbar facet arthropathy vs., II:2-53 34i, II:2-35
osteopetrosis vs., I: 1-162i recurrent vertebral disc herniation vs., VII:I-
pedicle insufficiency fracture vs., 1I:1-104i, 24i, VII:I-25, VII:I-34i, VII:I-35
II: 1-105 Perimedullary fistula. See Arteriovenous fistulas
plasmacytoma vs., IV:I-62i, IV:I-63 (AVFs), type IV.
renal osteodystrophy vs., V:2-8i, V:2-9 Perineural lymphomatosis (peripheral
Pancoast tumor. See superior sulcus tumor. neurolymphomatosis), VI:I-38 to VI:I-39
Pantopaque, retained, lumbar arachnoiditis Perineural root sleeve cyst, IV:2-10 to IV:2-12,
ossificans vs., 11I:2-14i IV:2-13i
Paraganglioma, IV:I-122 to IV:I-124, IV:I-12Si brachial plexus traction injury vs., VI:I-12i,
myxopapillary ependymoma of spinal cord vs., VI:I-13
IV: 1-11 1 differential diagnosis, IV:2-10i, IV:2-11
Paralysis, ascending. See Guillain-Barre syndrome. foraminal disc extrusion vs., 1I:2-38i, II:2-39
Parasitic diseases traumatic neuroma vs., VI:I-17
cysticercosis, III: 1-58 to III: 1-60, III:I-6li Perineural vein, enlarged, ulnar neuropathy vs.,
differential diagnosis, III: 1-5 1, III: 1-59 VI:I-44i, VI:I-45
echinococcus, III: 1-50 to III: 1-52, 1I1:1-S3i Peripheral nerve metastasis, peripheral
schistosomiasis, III: 1-54 to III: 1-56, 1I1:1-S7i neurolymphomatosis vs., VI:I-38i
Paraspinal abscess, III: 1-30 to III: 1-32, III:I-33i Peripheral nerve sheath tumors
benign, traumatic neuroma vs., VI:I-17
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INDEX
malignant, traumatic neuroma vs., VI:1-16i, peripheral nerve tumor vs., VI:1-34i
VI:1-17 PNET (primitive neuroectodermal tumor), Ewing
median nerve entrapmentvs., VI:l-SOi, VI:1-51 sarcoma vs., IV:l-SOi, IV:1-S1
Peripheral nerve tumors, VI:1-34 to VI:1-36, Pneumonia, staphylococcal, superior sulcus tumor
VI:1-37i vs., VI:1-7
Peripheral neurofibromatosis. See Polyneuropathy, chronic inflammatory
Neurofibromatosis type l. demyelinating (CIDP), III:Z-6 to III:2-8, III:Z-
Peripheral neurolymphomatosis, VI:1-38 to 9i. See also Polyradiculoneuropathy.
VI:1-39 brachial plexus traction injury vs., VI:l-13
Peripheral neuropathy. See Neuropathy. CSF disseminated metastases vs., IV: 1-99
Perivertebral venous plexus, cement extravasation differential diagnosis, III:Z-6i, III:2-6 to III:2-7
into, VII: 1-7 Guillain-Barre syndrome vs., 11I:2-Zi, III:2-3
Peroneal neuropathy, common, VI:1-52 to VI:1-53 hypertrophic neuropathy vs., VI:1-30i, VI:1-31
Phlegmon lateral meningocele vs., I:I-IOZi, 1:1-103
CSF leakage syndrome vs., VII: 1-11 neurofibroma vs., IV:1-91
epidural, extramedullary hematopoiesis vs., neurofibromatosis type 1 vs., l:l-11Oi, 1:1-111
V:2-16i, V:2-17 peripheral neurolymphomatosis vs., VI:1-38i
paravertebral, extramedullary hematopoiesis Polyradiculoneuropathy
vs., V:Z-16i, V:2-17 acute inflammatory demyelinating. See
peridural fibrosis vs., VII:I-Z4i, VII:1-25 Guillain-Barre syndrome.
traumatic disc herniation vs., 1I:1-90i, 11:1-91 congenital hypertrophic
Pigmented tumors, intradural/extramedullary, CSF disseminated metastases vs., IV: 1-99
melanocytoma vs., IV:1-127 neurofibromatosis type 1 vs., 1:1-110i,
Pillar fracture (lateral cervical flexion injury), 11:1- 1:1-111
46 to 11:1-47 Post-procedure complications, VII:1-1 to VII: 1-61
Pilonidal sinus, dorsal dermal sinus vs., 1:1-25 accelerated degeneration, VII: 1-18 to VII: 1-20,
Plantar fasciitis, posterior tibial nerve entrapment VII:I-Zli
vs., VI:1-S4i bone graft complications, VII: 1-32 to VII: 1-33
Plaques, calcified dural, lumbar arachnoiditis post-operative spinal complications vs.,
ossificans vs., III:Z-14i, III:2-15 VII: 1-43
Plasmacytoma, IV: 1-62 to IV: 1-64, IV:1-6Si CSF leakage syndrome. See Cerebrospinal fluid
aneurysmal bone cyst vs., IV:1-27 (CSF) leakage syndrome.
blastic osseous metastases vs., IV: 1-7 failed back surgery syndrome, VII: 1-46 to
differential diagnosis, IV:1-6Zi, IV:1-63 VII:1-48, VII:1-49i
lytic osseous metastases vs., IV: 1-11 recurrent vertebral disc herniation as, VII:1-
superior sulcus tumor vs., VI:1-7 34 to VII: 1-36, VII:1-37i
Plexiform neurofibroma. See Neurofibroma, hardware failure, VII:1-14 to VII:1-16, VII:l-l7i
plexiform. infection, VII: 1-38 to VII: 1-40, VII:l-4li
Plexopathy instability vs., II:Z-1Si, 11:2-16
lumbar, femoral neuropathyvs., VI:1-43 metal artifact, VII: 1-50 to VII:1-52, VII:l-S3i
radiation, VI:1-26 to VI:1-Z8, VI:I-Z9i myelography complications, VII: 1-2 to VII: 1-4,
thoracic outlet syndrome vs., VI:1-18i, VII:l-Si
VI:1-19 peridural fibrosis. See Peridural fibrosis.
Plexus lesions, VI:I-6 to VI:1-55 post irradiation vertebral marrow, VII: 1-58 to
brachial plexus. See Brachial plexus entries. VII:1-60, VII:1-6li
muscle denervation, spine, VI:1-10 to VI: 1-11 post-laminectomy spondylolisthesis, VII: 1-22
neoplasms, thoracic outlet syndrome vs., VI: 1-19 to VII: 1-23
peripheral neurolymphomatosis, VI:1-38 to pseudomeningocele. See Pseudomeningocele.
VI:1-39 radiation myelopathy, VII: 1-54 to VII: 1-56,
radiation plexopathy, VI:1-26 to VI:1-28, VII:l-S7i
VI:I-Z9i recurrent vertebral disc herniation, VII:1-34 to
thoracic outlet syndrome vs., VI:1-18i, VII:1-36, VII:1-37i
VI:1-19 spinal, VII:1-42 to VII:1-44, VII:I-4Si
superior sulcus tumor, VI:1-6 to VI:1-8, VI:1-9i vertebroplasty complications, VII: 1-6 to VII: 1-
idiopathic brachial plexus neuritis vs., 8, VII:1-9i
VI:1-23 Postdural puncture headache (PDPH), VII: 1-2 to
thoracic outlet syndrome vs., VI:1-18i VII:1-4, VII:l-Si
thoracic outlet syndrome, VI:1-18 to VI: 1-20, Posterior column injury
VI:I-Zli cervical, 11:1-48 to 11:1-49
traumatic neuroma, VI:1-16 to VI:1-17
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INDEX
thoracic facet-lamina fracture, 11:1-60 to 11:1- facet joint. See Septic facet joint arthritis.
62, II:1-63i meningitis, CSF disseminated metastases vs.,
Posterior element IV: 1-98i, IV: 1-99
cervical hyperextension injury, 11:1-34 to 11:1- neurogenic (Charcot) arthropathy vs., II:2-72i,
36, II:1-37i 11:2-73
incomplete fusion, 1:1-22 to 1:1-23 osteomyelitis, III: 1-6 to 111:1-8, 1II:1-9i
Posterior longitudinal ligament. See Longitudinal spondylitis, granulomatous osteomyelitis vs.,
ligament, posterior, ossification (OPLL). lII:l-lOi, III:l-11
Posterior meningocele. See Meningocele, dorsal
spinal; Meningocele, posterior sacral.
Posterior tibial nerve entrapment, VI: 1-54 to VI: 1-55 Q
Postural kyphosis. See Kyphosis, idiopathic. Quadrilateral space syndrome, idiopathic brachial
Pott disease (granulomatous osteomyelitis), III: 1-10 plexus neuritis vs., VI:I-23
to III:I-12, 1II:1-13i
Primitive neuroectodermal tumor (PNET), Ewing
sarcoma vs., IV:l-S0i, IV:I-51
Pronator syndrome. See Median nerve entrapment.
R
Prostate metastases, bone island vs., I:I-92i Radial neuropathy, VI:I-40 to VI:I-41
Protrusion. See Disc protrusion. Radiation
Pseudo-Hurler polydystrophy, congenital scoliosis and kyphosis vs., 1:1-135
mucopolysaccharidoses vs., 1:1-157 fibrosis due to, VI:I-26 to VI:I-28, VI:I-29i
Pseudoachondroplasia, achondroplasia vs., 1:1-153
Pseudoarthrosis
accelerated degeneration vs., VII:1-18i, VII:I-19
superior sulcus tumor vs., VI:1-6i, VI:I-7
myelopathy due to, VII:I-54 to VII:I-56,
VII:l-S7i
II
bone graft complications vs., VII:I-33 myopathy due to, muscle denervation vs., xxv
failed back surgery syndrome vs., VII: 1-47 VI:l-11
instability vs., II:2-1Si, 11:2-16 myositis due to, idiopathic brachial plexus
pedicle insufficiency fracture vs., II: 1-104i, neuritis vs., VI:1-22i, VI:I-23
11:1-105 neuritis due to, idiopathic brachial plexus
Pseudocyst. See Pseudomeningocele. neuritis vs., VI:1-22i, VI:I-23
Pseudogout. See Calcium pyrophosphate dihydrate peripheral neuropathy vs., VIII:1-12i
deposition disease (CPPD). plexopathy due to, VI:I-26 to VI:I-28, VI:I-29i
Pseudomeningocele, VII: 1-28 to VII: 1-30, VII:I-3li thoracic outlet syndrome vs., VI:l-18i,
avulsion. See Brachial plexus traction injury. VI:I-19
bone graft complications vs., VII:I-32i post irradiation vertebral marrow, VII:I-58 to
CSF leakage syndrome vs., VII:l-10i, VII: 1-11 VII:I-60, VII:1-6li
differential diagnosis, VII:1-28i, VII:I-29 scoliosis due to, I:1-126i, 1:1-128
failed back surgery syndrome vs., VII:1-46i idiopathic scoliosis vs., 1:1-131
peridural fibrosis vs., VII: 1-25 neuromuscular scoliosis vs., 1:1-138
post-operative spinal, hemangioma vs., IV:I-14i, IV:I-15
myelomeningocele vs., I:I-I0i, 1:1-11 Radicular (meningeal) cyst, lateral meningocele
posterior sacral meningocele vs., IV:2-6i, vs., I:I-102i, 1:1-103
IV:2-7 Radiculopathy
Pseudospread of atlas, in children, Jefferson Cl cervical
fracture vs., II:1-16i, II:I-17 idiopathic brachial plexus neuritis vs.,
Pseudosubluxation, hangman's C2 fracture vs., VI:I-23
II:I-28i, 11:1-29 median nerve entrapment vs., VI:l-SOi
Psoas abscess, traumatic spinal muscle injury vs., suprascapular nerve entrapment vs., VI:l-
II:1-102i 48i, VI: 1-49
Psoriatic arthritis peripheral neuropathy vs., VIII:l-12i
craniovertebral junction variants vs., I:1-78i, Reactive (Reiter's) arthritis, DISH vs., II:2-76i,
1:1-79 11:2-77
DISH vs., II:2-76i, 11:2-77 von Recklinghausen disease. See
Pulmonary embolization of cement, VII:1-6i, Neurofibromatosis type 1.
VII:I-7 Recurrent vertebral disc herniation, VII:I-34 to
Pycnodysostosis, osteopetrosis vs., 1:1-162 VII:I-36, VII:1-37i
Pyogenic abscess description, VII:I-34 to VII:I-36
cysticercosis vs., III:l-S8i, III:I-59 failed back surgery syndrome as, VII:I-46 to
schistosomiasis vs., III: 1-55 VII: 1-48, VII:1-49i
Pyogenic infections peridural fibrosis vs., VII:1-24i, VII: 1-25
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INDEX
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INDEX
burst thoracolumbar fracture vs., 1I:1-S8i, compensatory, differential diagnosis, 1:1-128
II:1-59 congenital, 1:1-134 to 1:1-136, 1:1-137i
congenital scoliosis and kyphosis vs., 1:1-135 degenerative scoliosis vs., 1I:2-68i, 11:2-69
differential diagnosis, 1:1-146i, 1:1-147 differential diagnosis, 1:1-126i, 1:1-127
idiopathic kyphosis vs., 1:1-140i, 1:1-140 to idiopathic scoliosis vs., 1:1-130i, 1:1-131
1:1-141 neuromuscular scoliosis vs., 1:1-138i, 1:1-138
idiopathic scoliosis vs., I: 1-131 without vertebral anomalies, 1:1-127
kyphosis vs., VIII:1-lOi degenerative, 11:2-68 to 11:2-70, 1I:2-7li
low thoracic distraction fracture vs., 1I:1-68i differential diagnosis, I: 1-128
lumbar anterior compression fracture vs., 11:1- idiopathic scoliosis vs., 1:1-131
70i,1I:1-71 differential diagnosis, 1:1-126i, 1:1-127 to 1:1-28
neuromuscular scoliosis vs., 1:1-138i, 1:1-138 iatrogenic, I: 1-128
pathologic vertebral fracture vs., VIII:1-1Si, idiopathic, 1:1-130 to 1:1-132, 1:1-133i
VIII:1-16 back pain in children vs., VIII:1-7
scoliosis vs., 1:1-127 congenital scoliosis and kyphosis vs., 1:1-
Schistosomiasis, III:1-54 to III:1-56, III:1-S7i 134i,I:1-135
cysticercosis vs., III:1-59 degenerative scoliosis vs., 1I:2-68i, 11:2-69
Schmorl node, 1:1-142 to 1:1-144, 1:1-14Si. See also differential diagnosis, 1:1-127, 1:1-130i,
Scheuermann disease. 1:1-131
apophyseal ring fracture vs., 1I:1-94i, 11:1-95 neuromuscular scoliosis vs., 1:1-138i
bone island vs., 1:1-92i, 1:1-93 infectious
differential diagnosis, 1:1-142i, 1:1-143
limbus vertebra vs., 1:1-82i, 1:1-83
Schwannoma, IV:1-86 to IV: 1-88, IV:1-89i
congenital scoliosis and kyphosis vs., 1:1-135
differential diagnosis, 1:1-126i, 1:1-127
idiopathic scoliosis vs., 1:1-131
II
xxvii
acqUired epidermoid tumor vs., IV:2-28i neuromuscular scoliosis vs., 1:1-138
conjoined nerve roots vs., 1:1-86i, 1:1-87 neuromuscular, 1:1-138 to 1:1-139
cysticercosis vs., 11I:1-S8i, III:1-59 congenital scoliosis and kyphosis vs., 1:1-135
differential diagnosis, IV:1-86i, IV:1-87 differential diagnosis, 1:1-127
dural arteriovenous fistula type I vs., V:1-6i idiopathic scoliosis vs., 1:1-130i, 1:1-131
facet joint synovial cyst vs., 1I:2-46i neuropathic spine vs., 1:1-128
femoral neuropathyvs., VI:1-42 positional, differential diagnosis, 1:1-128
filum terminale fibrolipoma vs., 1:1-91 radiation-induced
foraminal disc extrusion vs., 1I:2-38i, 11:2-39 congenital scoliosis and kyphosis vs., 1:1-135
hemangiopericytoma vs., IV:1-82i, IV:1-83 differential diagnosis, 1:1-126i, 1:1-128
malignant nerve sheath tumors vs., 1V:1-94i idiopathic scoliosis vS., 1:1-131
melanocytoma vs., IV:1-127 neuromuscular scoliosis vs., 1:1-138
meningioma vs., IV:1-78i, IV: 1-79 traumatic
myelitis-cord abscess vs., III: 1-43 congenital scoliosis and kyphosis vs., 1:1-135
neurofibroma vs., IV:1-90i, IV:1-91 differential diagnosis, 1:1-128
non-syndromic, neurofibromatosis type 2 vs., idiopathic scoliosis vs., 1:1-131
1:1-115 tumor-induced
paraganglioma vs., IV:1-123i, IV:1-124 differential diagnosis, 1:1-126i, 1:1-127
radial neuropathy vs., VI:1-4Oi idiopathic scoliosis vs., 1:1-130i, 1:1-131
superior sulcus tumorvs., VI:1-7 neuromuscular scoliosis vs., 1:1-138i, 1:1-138
SCI. See Spinal cord infarction. SEA. See Epidural abscess.
Sciatica, posterior tibial nerve entrapment vs., Seat belt fracture. See Chance fracture, thoracic.
VI:1-54 Secondary acute transverse myelitis, VIII: 1-2 to
Scleroderma, osteochondroma vs., IV:1-34i VIII:1-4, VIII:1-Si
Sclerosis. See also Multiple sclerosis. SED. See Spondyloepiphyseal dysplasia.
discogenic, renal osteodystrophy vs., V:2-8i, Segmental degeneration, accelerated, VII: 1-18 to
V:2-9 VII:1-20, VII:1-2li
osteosclerosis (dense vertebral body), VIII:1-18 Segmental instability. See Instability.
to VIII:1-19 Segmentation and fusion anomalies. See Vertebral
tuberous, fibrous dysplasia vs., IV:2-30i, formation and segmentation anomalies.
IV:2-31 Septic facet joint
Sclerotic island. See Bone island. cervical facet arthropathy vs., 1I:2-48i, 11:2-49
Sclerotic metastases. See Metastatic disease, lumbar facet arthropathy vs., 1I:2-42i, 11:2-53
sclerotic. pedicle insufficiency fracture vs., 1I:1-104i,
SCM. See Diastematomyelia. 11:1-105
Scoliosis, 1:1-126 to 1:1-128, 1:1-129i tumoral calcinosis vs., V:2-20i, V:2-21
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INDEX
Septic facet joint arthritis, III:1-18 to III: 1-20, diastematomyelia, 1:1-56 to 1:1-58, I:l-S9i
III:I-2li duplicated (diplomyelia), diastematomyelia vs.,
differential diagnosis, III:I-18i, III:1-19 I:l-S6i
facet joint synovial cyst vs., 1I:2-S6i, II:2-57 herniation of
thoracic facet-lamina fracture vs., 1I:1-60i, idiopathic, arachnoid cyst vs., IV:2-li,
II:1-61 IV:2-3
Seronegative spondyloarthropathy. See traumatic, II:1-120 to II:1-122, 1I:1-123i
Spondyloarthropathy, seronegative. ischemia. See Spinal cord infarction.
SGT. See Sacrococcygeal teratoma. multiple sclerosis. See Multiple sclerosis.
Shear injury, thoracic Chance fracture vs., 11:1- myelitis-cord abscess, III: 1-42 to III: 1-44,
Mi, II: 1-65 III:1-4Si
"Short pedicle" syndrome. See Stenosis, spinal, peripheral T2 hypointensity in, superficial
congenital. siderosis vs., V:1-42i, V:1-43
Sickle cell, 1:1-160 to 1:1-161 post-operative complications, VII: 1-42 to VII: 1-
differential diagnosis, I:I-160i, 1:1-161 44, VII:l-4Si
Kummell disease vs., IV:2-32i tethered. See Tethered spinal cord.
muscle denervation vs., VI:1-10 transient dilatation of central canal,
Siderosis, superficial, V: 1-42 to V:1-44, V:I-4Si ventriculus terminalis vs., I:1-94i, 1:1-95
Simple meningocele. See Meningocele, dorsal vitamin B12 deficiency, III:2-32 to III:2-34,
spinal. III:2-3Si
Sinus, dorsal dermal, 1:1-24 to 1:1-26, I:I-27i Spinal cord infarction, V: 1-26 to V:1-28, V:I-29i
Slipped vertebral apophysis. See Limbus vertebra. acute, viral myelitis vs., III:1-35
Soft tissue sarcoma ADEM vs., III:2-24i, III:2-25
malignant nerve sheath tumors vs., IV:I-94i, astrocytoma of spinal cord vs., IV:1-103
IV: 1-95 bone graft complications vs., VII:1-32i,
neurogenic (Charcot) arthropathy vs., 1I:2-72i, VII:1-33
II:2-73 cellular ependymoma of spinal cord vs., IV:l-
peripheral nerve tumor vs., VI:I-34i, VI:1-35 106i,IV:1-107
Soft tissue tumors central spinal cord syndrome vs., 1I:1-116i,
metastatic, traumatic neuroma vs., VI: 1-17 II:1-117
pseudomeningocele vs., VII: 1-29 differential diagnosis, V:1-26i, V:1-27
rhabdomyolysis vs., II: 1-98i, II: 1-99 IATM vs., III:2-28i, III:2-29
Spina bifida aperta. See Myelomeningocele. myelitis-cord abscess vs., III:1-43
Spina bifida cystica. See Myelomeningocele. radiation myelopathy vs., VII:1-S4i, VII:1-55
Spina bifida occulta (posterior element incomplete sarcoidosis vs., III:2-17
fusion), 1:1-22 to 1:1-23 schistosomiasis vs., III:1-55
Spinal canal secondary acute transverse myelitis vs.,
cement extravasation into, VII:I-6i, VII: 1-6 to VIII: 1-3
VII: 1-7 spinal cord multiple sclerosis vs., III:2-20i,
lumbar, type IV AVF vs., V:I-18i, V:1-19 III: 2-21
Spinal cord. See also Spinal cord infarction; Spinal superficial siderosis vs., V:1-42i
cord neoplasms. vitamin B12 deficiency vs., III:2-32i
ADEM. See Encephalomyelitis, acute Spinal cord neoplasms
disseminated (ADEM). acqUired cervical spinal stenosis vs., II:2-65
astrocytoma. See Astrocytoma. back pain in children vs., VIII:1-7
cavernous malformation. See Cavernous cystic, ventriculus terminalis vs., I:I-94i, 1:1-95
malformation, spinal cord. dural arteriovenous fistula type I vs., V:I-6i,
central spinal cord syndrome, II:1-116 to II:1- V:1-7
118,1I:1-119i ependymoma. See Ependymoma.
central T2 hyperintensity of, superficial hemangioblastoma. See Hemangioblastoma of
siderosis vs., V:1-43 spinal cord.
contusion-hematoma, II: 1-112 to II: 1-114, 11:1- hemorrhagic, melanocytoma vs., IV:I-126i,
115i IV:1-126
central spinal cord syndrome vs., 1I:1-116i, hypervascular
II:1-117 hemangioblastoma of spinal cord vs.,
differential diagnosis, 1I:1-112i, II: 1-113 IV:l-115
myelography complications vs., VII:I-2i, myelitis-cord abscess vs., III:1-43
VII: 1-3 IATM vs., III:2-29
vitamin B12 deficiency vs., III:2-32i, melanocytoma, IV:1-126 to IV:1-127
III:2-33 metastatic, IV: 1-118 to IV:1-120, IV:1-12li
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INDEX
post-traumatic syrinx vs., 11:1-109 CPPD vs., II:2-102i, 11:2-103
secondary acute transverse myelitis vs., degenerative disc disease vs., II:2-6i, 11:2-7
VIII: 1-3 degenerative endplate changes vs., II:2-10i,
spinal cord contusion-hematoma vs., 11:1-113 11:2-11
spinal cord infarction vs., V:I-26i, V:I-27 differential diagnosis, II:2-104i
syringomyelia vs., IV:2-15 gout vs., II:2-100i, 11:2-100
Spinal fusion. See Fusion. ochronosis vs., 1:1-164i, 1:1-165
Spinal manifestation of systemic disease, V:2-2 to pyogenic osteomyelitis vs., III: 1-7
V:2-23 renal osteodystrophy vs., V:2-9
extramedullary hematopoiesis, V:2-16 to V:2- rheumatoid arthritis vs., II:2-88i, 11:2-89
18, V:2-19i seronegative, 11:2-96 to 11:2-98, II:2-99i
paraspinal abscess vs., III: 1-30i, III: 1-31 back pain in children vs., VIII:I-8
hyperparathyroidism, V:2-6 to V:2-7 cauda equina syndrome of, dural dysplasia
brown tumor of, giant cell tumorvs., IV:I-31 vs., 1:1-118i, 1:1-119
differential diagnosis, V:2-6i, V:2-7 congenital scoliosis and kyphosis vs., 1:1-135
primary, renal osteodystrophy vs., V:2-8 connective tissue disorders vs., 1:1-167
hyperplastic vertebral marrow, V:2-10 to V:2- CPPD vs., 11:2-103
12, V:2-13i degenerative disc disease vs., II:2-6i, 11:2-7
myelofibrosis, V:2-14 to V:2-15 differential diagnosis, II:2-96i, 11:2-97
differential diagnosis, V:2-14i DISH vs., II:2-76i, 11:2-77
heterogeneous fatty' marrow vs., IV:2-23 gout vs., 11:2-100
osteopetrosis vs., 1:1-163
Paget disease. See Paget disease.
renal osteodystrophy. See Renal osteodystrophy.
hemangiopericytoma vs., IV: 1-83
hyperparathyroidism vs., V:2-6i, V:2-7
idiopathic kyphosis vs., 1:1-140i, 1:1-141
II
tumoral calcinosis, V:2-20 to V:2-22, V:2-23i xxix
Klippel-Feil spectrum vs., I:I-74i, 1:1-75
lumbar facet arthropathy vs., II:2-42i, kyphosis vs., VIII:l-lOi
11:2-53 ochronosis vs., 1:1-164
Spinal muscles. See Muscles. posterior tibial nerve entrapment vs., VI: 1-55
Spinal neoplasms/tumors. See also Spinal cord rheumatoid arthritis vs., II:2-88i, 11:2-89
neoplasms. Scheuermann disease vs., 1:1-146i, 1:1-147
benign, back pain in children vs., VIII:I-7 vertebral segmentation failure vs., 1:1-70i,
dural dysplasia vs., 1:1-118i, 1:1-119 1:1-71
idiopathic scoliosis vs., 1:1-130i, 1:1-131 Spondylocostal dysostOSiS, thanatophoric
malignant, back pain in children vs., VIII: 1-7 dwarfism vs., 1:1-150i, 1:1-150
metastatic Spondylodiscitis, pyogenic, III: 1-6 to III: 1-8, III: 1-9i
granulomatous osteomyelitis vs., III:I-I0i, Spondyloepiphyseal dysplasia
111:1-11 achondroplasia vs., l:l-lS3i
pyogenic osteomyelitis vs., III:I-6i, III:I-7 mucopolysaccharidoses vs., l:l-lS6i, 1:1-157
to III: 1-8 sickle cell vs., 1:1-160i, 1:1-161
Spinal nerve roots. See Nerve root(s). thanatophoric dwarfism vs., I:l-ISOi, 1:1-150
Spinal stenosis. See Stenosis, spinal. Spondyloepiphyseal dysplasia tarda, Scheuermann
Spinoglenoid notch impingement. See disease vs., 1:1-146i, 1:1-147
Suprascapular nerve entrapment. Spondylolisthesis, 11:2-42 to 11:2-44, II:2-4Si
Split cord malformation. See Diastematomyelia. back pain in children vs., VIII:I-6
Spondylitis degenerative, spondylolysis vs., 1I:2-44i, 11:2-45
ankylosing. See Spondyloarthropathy, differential diagnosis, II:2-42i, 11:2-43
seronegative. dysplastic, spondylolysis vs., II:2-44i, 11:2-45
fungal, granulomatous osteomyelitis vs., III: 1-11 iatrogenic, spondylolysis vs., II:2-44i, 11:2-45
pyogenic, granulomatous osteomyelitis vs., isthmic, post-laminectomy spondylolisthesis
III:l-lOi, 111:1-11 vs., VII: 1-23
seronegative. See Spondyloarthropathy, pathologic, spondylolysis vs., II:2-44i, 11:2-45
seronegative. post-laminectomy, VII: 1-22 to VII: 1-23
tuberculous primary, hangman's C2 fracture vs., 11:1-29
granulomatous osteomyelitis, III: 1-10 to traumatic
111:1-12, III:I-13i axial (Hangman's C2 fracture), 11:1-28 to
pyogenic osteomyelitis vs., III:I-6i 1I:1-30,II:I-3li
Spondyloarthropathy spondylolysis vs., II:2-44i, 11:2-45
of hemodialysis, 11:2-104 to 11:2-105 with spondylolysis, accelerated degeneration
CI-C2 osteomyelitis vs., III:I-15 to III: 1-16 vs., VII:I-19
calcific longus coli tendinitis vs., 11:2-107 Spondylolysis,II:2-44 to 11:2-46, II:2-47i
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INDEX
back pain in children vs., VIII:I-6 subarachnoid hemorrhage vs., V:1-30i, V:I-31
differential diagnosis, 1I:2-44i, II:2-45 superficial siderosis vs., V:1-42i, V:I-43
lumbar facet-posterior fracture vs., II:I-80 to Subluxation
II:I-81 CI-C2, due to rheumatoid arthritis, odontoid
mimics, on sagittal MR, II:2-45 C2 fracture vs., II:I-21
spondylolisthesis vs., 1I:2-42i physiologic, juvenile chronic arthritis vs., 11:2-
unilateral, osteoid osteoma vs., IV:I-19 92i, 11:2-93
with spondylolisthesis, accelerated pseudosubluxation, hangman's C2 fracture vs.,
degeneration vs., VII:I-19 1I:1-28i, II:I-29
Spondylosis. See also Disc disease, degenerative. rotary, C2-C3, hangman's C2 fracture vs., 11:1-
acquired spinal. See Stenosis, spinal, acquired. 28i, II: 1-29
DISH vs., 1I:2-76i, II:2-77 rotation fracture-subluxation, cervical
OPLL vs., 1I:2-80i, II:2-81 hyperextension injury vs., 1I:1-34i, II:I-35
Sports injuries. See also Traumatic injuries. traumatic anterior (cervical hyperflexion
back pain due to, in children, VIII: 1-6 injury), II:1-32 to II: 1-33
idiopathic brachial plexus neuritis vs., VI:I-24 Superficial siderosis, V: 1-42 to V: 1-44, V:l-4Si
SS disease. See Sickle cell. Superior sulcus tumor, VI:I-6 to VI:I-8, VI:1-9i
SSA. See Subdural abscess. differential diagnosis, VI:1-6i, VI:I-7
SSDH. See Hematoma, subdural. idiopathic brachial plexus neuritis vs., VI:I-23
Stenosis, spinal thoracic outlet syndrome vs., VI:1-18i
acquired Supernumerary vertebra (partial vertebral
cervical, II:2-64 to II:2-66, 1I:2-67i duplication),I:1-68-69
congenital spinal stenosis vs., 1:1-122i, Suprascapular nerve entrapment, VI:I-48 to VI: 1-49
1:1-123 idiopathic brachial plexus neuritis vs., VI:l-
lumbar, II:2-60 to II:2-62, 1I:2-63i 22i, VI:I-23
congenital, 1:1-122 to 1:1-124, 1:1-12Si Susceptibility artifact, VII: 1-50
inherited, congenital spinal stenosis vs., 1:1- Synovial chondromatosis, tumoral calcinosis vs.,
122i,I:I-123 V:2-21
lumbar arachnoiditis ossificans vS., 1I1:2-14i, Synovial cysts
III:2-15 conjoined nerve roots vs., I:1-86i, 1:1-87
lumbar arachnoiditis vs., 1I1:2-lOi, III:2-11 facet joint, II:2-56 to II:2-58, 1I:2-S9i
lumbar canal, type IV AVF vs., V:I-18i, V:I-19 arachnoid cyst vs., IV:2-li, IV:2-3
Stress fractures differential diagnosis, 1I:2-S6i, II:2-57
calcaneal, posterior tibial nerve entrapment vs., perineural root sleeve cyst vs., IV:2-10i,
VI:l-S4i IV:2-11
kyphosis vs., VIII:l-lOi septic facet joint arthritis vs., 1I1:1-18i,
idiopathic,I:1-141 III:I-19
laminar, osteoid osteoma vs., IV:1-18i, IV:I-19 meningioma vs., IV:1-78i
pedicle, II:I-I04 to II: 1-106, 1I:1-107i recurrent vertebral disc herniation vs., VII:1-
osteoid osteoma vs., IV:1-18i, IV:I-19 34i, VII: 1-35
sacral insufficiency fractures, II: 1-86 to II: 1-88, tumoral calcinosis vs., V:2-20i, V:2-21
1I:1-89i Syringocele. See Myelocystocele, terminal.
traumatic sacral fracture vs., 1I:1-82i, II:I-83 Syringohydromyelia
Subarachnoid hemorrhage, V:I-30 to V:I-32, astrocytoma of spinal cord vs., IV:I-I03
V:1-33i idiopathic acute transverse myelitis (IATM) vS.,
differential diagnosis, V:1-30i, V:I-31 III:2-28i, III:2-29
filum terminale fibrolipoma vs., 1:1-90i, 1:1-91 secondary acute transverse myelitis vs., VIII:1-
underlying etiology of, superficial siderosis vs., 2i, VIII: 1-3
V: 1-43 spinal cord multiple sclerosis vs., 1I1:2-20i,
Subdural abscess, III: 1-26 to III: 1-28, III:1-29i III:2-21
subdural hematoma vs., V:1-38i, V:I-39 ventriculus terminalis vs., 1:1-94i, 1:1-95
Subdural hematoma, V:1-38 to V: 1-40, V:l-4li Syringomyelia, IV:2-14 to IV:2-16, IV:2-17i
differential diagnosis, V:1-38i, V:I-39 acquired cervical spinal stenosis vs., 1I:2-64i,
epidural hematoma vs., V:I-35 II:2-65
spinal meningitis vs., III:I-47 cysticercosis vs., III: 1-59
subdural abscess vs., 1I1:1-26i, III:I-27 differential diagnosis, IV:2-14i, IV:2-15
Subdural hemorrhage dural dysplasia vs., 1:1-118i, 1:1-119
angiolipoma vs., IV:1-74i idiopathic (nontraumatic), post-traumatic
post-operative spinal complications vs., VII:1- syrinx vs., 1I:1-108i, II:I-109
42i, VII: 1-43
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INDEX
post-traumatic syrinx, II:I-108 to II: 1-110, acquired, Chiari I malformation vs., 1:1-98i,
II:l-11li 1:1-99
radiation myelopathy vs., VII:l-S4i, VII:I-55 below foramen magnum, Chiari I malformation
Syrinx. See Syringomyelia. vs., I:I-98i, 1:1-99
Tortuous spinal nerve root, dural arteriovenous
fistula type I vs., V:1-6i, V:I-7
T TOS. See Thoracic outlet syndrome.
Talocalcaneal coalition, posterior tibial nerve Transverse myelitis. See Myelitis, transverse.
entrapment vs., VI:l-S4i, VI:I-54 to VI:I-55 Traumatic injuries, II:I-I0 to II: 1-139
Tadov cyst anterior compression fracture
aneurysmal bone cyst vs., IV:I-27 lumbar, II:I-70 to 11:1-71
connective tissue disorders vs., I:I-166i, 1:1-167 thoracic, II:I-50 to 11:1-52, II:l-S3i
occult intrasacral meningocele vs., 1:1-49i, 1:1-48 apophyseal ring fracture, II:I-94 to II: 1-96,
perineural. See also Perineural root sleeve cyst. II:I-97i
posterior sacral meningocele vs., IV:2-7 atlanto-occipital dislocation, II:I-I0 to II: 1-11
Tarsal tunnel syndrome, VI:I-54 to VI:I-55 burst fractures. See Burst fractures.
TCS. See Tethered spinal cord. carotid artery dissection, II: 1-134 to II: 1-136,
Tear drop fractures, hyperflexion-rotation injury, II:1-137i
cervical vs., II:1-38i, II:I-39 central spinal cord syndrome, II:l-116 to II:l-
Telangiectatic osteogenic sarcoma, aneurysmal 118,II:1-119i
bone cyst vs., IV:1-26i, IV:I-27 Chance fracture, thoracic. See Chance fractures
Tenosynovitis, median nerve entrapment vs., VI:l-
SOi, VI:I-51
Teratoma. See Sacrococcygeal teratoma.
thoracic.
common peroneal neuropathy vs., VI: 1-53
compression fractures. See Compression
'
II
xxxi
Terminal lipoma. See Lipoma, terminal. fractures.
Terminal myelocystocele. See Myelocystocele, terminal. craniovertebral junction variants vs., 1:1-79
Terminal ventricle. See Ventriculus terminalis. degenerative scoliosis vs., II:2-68i, II:2-69
Tethered spinal cord, 1:1-36 to 1:38, I:1-39i disc herniation, II:I-90 to 11:1-92, II:I-93i. See
back pain in children vs., VIII:I-8 also Disc herniation.
caudal regression syndrome vs., 1:1-32i, 1:1-33 distraction fracture, low thoracic, II: 1-68 to
differential diagnosis, I:1-36i, 1:1-37 II:I-69
filum terminale fibrolipoma vs., I:I-9Oi epidural-subdural hematoma, II:I-126 to II:l-
post-operative release of, I:1-36i 128, II:1-129i
post-operative spinal complications vs., VII:l- facet-lamina fracture, thoracic, II:I-60 to II:l-
42i, VII: 1-44 62, II: 1-63i
Thalassemia, sickle cell vs., I:1-160i, 1:1-161 facet-posterior fracture, lumbar, II: 1-80 to II:I-81
Thanatophoric dwarfism, 1:1-150 to 1:1-151 hangman's C2 fracture, II: 1-28 to II:1-30,
achondroplasia vs., I:l-lS3i II:1-3li
Thoracic outlet syndrome, VI:I-18 to VI: 1-20, hyperextension injury, cervical, II:I-34 to II:l-
VI:1-2li 36, II:1-37i
Thoracic spine hyperextension-rotation injury, cervical, II:l-
anterior compression fracture, II:I-50 to II:l- 44 to II: 1-45
52, II:l-S3i hyperflexion injury, cervical, II:I-32 to II:I-33
Chance fracture. See Chance fracture, thoracic. hyperflexion-rotation injury, cervical, II:I-38
distraction fracture, low thoracic, II:I-68 to to II: 1-40, II:1-4li
II:I-69 idiopathic kyphosis vs., 1:1-141
facet-lamina fracture, II: 1-60 to II: 1-62, II: 1-63i Jefferson Cl fracture, II:I-16 to II:I-18, II:1-19i
intervertebral disc herniation, II:2-30 to II:2- lateral compression fracture
32, II:2-33i lumbar, 1I:1-72 to II:I-74, II:1-7Si
lateral compression fracture, II:I-54 to II:I-56, thoracic, II:I-54 to II:1-56, II:l-S7i
II:l-S7i lateral flexion injury, cervical, II: 1-46 to II:1-47
tumors, superior sulcus tumor vs., VI:I-7 lumbar fracture with dural tear, II:I-124 to
Thoracolumbar fracture, burst, II: 1-58 to II: 1-59 II:I-125
Tibial nerve entrapment, posterior, VI:I-54 to neuroma, V1:1-16 to V1:1-17
VI:I-55 peripheral nerve tumor vs., VI:1-34i
Tight filum terminale syndrome. See Tethered nonaccidental, osteogenesis imperfecta vs.,
spinal cord. 1:1-171
Tonsillar herniation occipital condyle fracture, II:I-12 to II:I-14,
II:l-1Si
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INDEX
atlanto-occipital dislocation vs., II:1-10i, Vascular lesions, V:I-6 to V:I-45. See also specific
II:I-I0 lesion.
odontoid C2 fracture, II: 1-20 to II: 1-22, II: 1-231 Vascular lipoma. See Angiolipoma.
dens type II, craniovertebral junction Vascular metastases
variants vs., 1:1-78i hemangioblastoma of spinal cord vs., IV: 1-115
pedicle insufficiency fracture, 11:1-104 to II:l- hemangiopericytoma vs., IV: 1-83
106,II:1-107i melanocytoma vs., IV:1-126i, IV:I-126
post-traumatic syrinx, II:l-108 to II:I-110, Vasculitis, immune-mediated, ADEM vs., III:2-25
II:1-11li Vasculitis neuropathy, Guillain-Barre syndrome
posterior column injury, cervical, 11:1-48 to vs., III:2-3
11:1-49 Vasospasm, vertebral artery dissection vs., II:I-131
rhabdomyolysis, 11:1-98 to II:I-I00, II:1-lOli Venous plexus
sacral fractures, II:I-82 to 11:1-84, II:1-8Si enlarged, CSF leakage syndrome vs., VII: 1-11
insufficiency, II:I-86 to II: 1-88, II:1-89i perivertebral, cement extravasation into,
scoliosis due to. See Scoliolis, traumatic. VII:I-7
spinal cord contusion-hematoma. See Spinal Ventral cord herniation, II:I-120 to II:I-122,
cord, contusion-hematoma. II:1-123i
spinal cord herniation, II:I-120 to II:I-122, Ventriculitis terminalis, post-traumatic syrinx vs.,
II:1-123i II:1-108i, II:I-I09
spinal muscles, II:I-I02 to II:I-I03 Ventriculus terminalis, 1:1-94 to 1:1-96, 1:1-97i
idiopathic brachial plexus neuritis vs., syringomyelia vs., IV:2-14i, IV:2-15
VI:I-23 Vertebra(e)
sports injuries butterfly, partial vertebral duplication vs., 1:1-
back pain due to, in children, VIII: 1-6 68-69
idiopathic brachial plexus neuritis vs., dysplasia, congenital, dural dysplasia vs., 1:1-
VI:I-24 118i,I:I-119
thoracic outlet syndrome vs., VI:1-18i, VI:I-19 endplate changes. See Endplate changes,
ulnar neuropathy vs., VI:1-44i, VI:I-4S degenerative.
vertebral artery dissection, II: 1-130 to II: 1-132, endplate spur, vertebral disc bulge vs., II:2-18i,
II:1-133i II:2-19
Tuberculosis fractures. See Vertebral fractures.
echinococcus vs., III: 1-51 fusion of. See Fusion.
neurogenic (Charcot) arthropathy vs., II:2-72i, hemangioma
II:2-73 Paget disease vs., V:2-2li, V:2-3
post-operative infection vs., VII:1-38i, VII: 1-39 plasmacytoma vs., IV: 1-63
Scheuermann disease vs., 1:1-147 post irradiation vertebral marrow vs., VII:1-
schistosomiasis vs., III: 1-55 S8i, VII: 1-59
spinal cord metastases vs., IV: 1-119 limbus, 1:1-82 to 1:1-84, 1:1-85i
superior sulcus tumor vs., VI:I-7 marrow. See Marrow.
vertebral osteomyelitis due to, pyogenic metastatic disease
osteomyelitis vs., III:1-6i, III:I-7 hemangioma vs., IV:I-15
Tuberculous spondylitis (granulomatous neuroblastic tumor vs., IV:1-70i, IV:I-71
osteomyelitis), III: 1-10 to III: 1-12, III:1-13i partial duplication, I: 1-68-69
Tuberous sclerosis, fibrous dysplasia vs., IV:2-30i, physiologic wedging, anterior thoracic
IV:2-31 compression fracture vs., II:1-Sli
Tumoral calcinosis, V:2-20 to V:2-22, V:2-23i tubercular osteomyelitis, pyogenic
lumbar facet arthropathy vs., II:2-42i, II:2-53 osteomyelitis vs., III:l-6i, III:I-7
Tumors. See Neoplasms/tumors. Vertebral arch ligament (ligamentum flavum)
ossification, II:2-84 to II:2-86, II:2-87i
Vertebral artery dissection, II:I-130 to II: 1-132,
u II:1-133i
Vertebral body
Ulnar neuropathy, VI:I-44 to VI:I-46, VI:1-47i
dense, VIII:I-18 to VIII:I-19
radial neuropathy vs., VI:1-4Oi
fracture, spondylolisthesis vs., II:2-43
shattered (cervical burst fracture), 11:1-42 to
v 11:1-43
tumor, recurrent vertebral disc herniation vs.,
VAD. See Vertebral artery dissection. VII:I-35
Varicella Zoster virus, HIV myelopathy vs., Vertebral discs. See Disc entries.
III:1-38i Vertebral formation and segmentation anomalies
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INDEX
w
Wedge compression fractures
anterior
lumbar, II:1-70 to II:1-71
thoracic, II:1-50 to II: 1-52, 1I:1-53i
Scheuermann disease vs., I:I-146i, 1:1-147
Wedging, physiologic, lumbar anterior
compression fracture vs., 1I:1-70i, II:l-71
Whiplash fracture
cervical hyperextension injury vs., 1I:1-34i,
II:1-35
cervical hyperflexion injury vs., 1I:1-32i,
II:1-33
hyperflexion-rotation injury, cervical vs., 11:1-
38i, II:1-39
Wilms tumor, neuroblastic tumor vs., IV: 1-71
Wires, broken, VII:1-14i
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