Table Of ContentR. Shane Tubbs
W. Jerry Oakes
Editors
The Chiari
Malformations
123
The Chiari Malformations
R. Shane Tubbs (cid:129) W. Jerry Oakes
Editors
The Chiari Malformations
Editors
R. Shane Tubbs, MS, PA-C, PhD W. Jerry Oakes, MD
Section of Pediatric Neurosurgery Section of Pediatric Neurosurgery
Children’s of Alabama Children’s of Alabama
Birmingham , AL Birmingham , AL
USA USA
ISBN 978-1-4614-6368-9 ISBN 978-1-4614-6369-6 (eBook)
DOI 10.1007/978-1-4614-6369-6
Springer New York Heidelberg Dordrecht London
Library of Congress Control Number: 2013941396
© Springer Science+Business Media New York 2013
This work is subject to copyright. All rights are reserved by the Publisher, whether the whole or
part of the material is concerned, speci fi cally the rights of translation, reprinting, reuse of
illustrations, recitation, broadcasting, reproduction on micro fi lms or in any other physical way,
and transmission or information storage and retrieval, electronic adaptation, computer software,
or by similar or dissimilar methodology now known or hereafter developed. Exempted from this
legal reservation are brief excerpts in connection with reviews or scholarly analysis or material
supplied speci fi cally for the purpose of being entered and executed on a computer system, for
exclusive use by the purchaser of the work. Duplication of this publication or parts thereof is
permitted only under the provisions of the Copyright Law of the Publisher’s location, in its
current version, and permission for use must always be obtained from Springer. Permissions for
use may be obtained through RightsLink at the Copyright Clearance Center. Violations are liable
to prosecution under the respective Copyright Law.
The use of general descriptive names, registered names, trademarks, service marks, etc. in this
publication does not imply, even in the absence of a speci fi c statement, that such names are
exempt from the relevant protective laws and regulations and therefore free for general use.
While the advice and information in this book are believed to be true and accurate at the date of
publication, neither the authors nor the editors nor the publisher can accept any legal responsibility
for any errors or omissions that may be made. The publisher makes no warranty, express or
implied, with respect to the material contained herein.
Printed on acid-free paper
Springer is part of Springer Science+Business Media (www.springer.com)
We would like to dedicate this book to the patients who have
collectively given us knowledge on the spectrum of Chiari
malformation. Additionally, this book is written in the memory
of Dr. Cornelis Joachimus van Houweninge Graftdijk who
pioneered decompression for hindbrain herniation and his son
Mr. Willem Aris van Houweninge Graftdijk and granddaughter
Ms. Evelien van Houweninge Graftdijk who helped remind us
of his story.
We thank our wives and children for their patience and support
of our academia.
R. Shane Tubbs
W. Jerry Oakes
Foreword
The history of the Chiari malformation is long and interesting with the inter-
est growing in each succeeding year. Thought to be rare when fi rst described
by Chiari in 1890, the increasing recognition can be partly attributed to the
advent of magnetic resonance imaging. But instead of clari fi cation, the visu-
alization and accurate description made possible by this diagnostic tool has
only brought forth new manifestations of the process and new controversies
over management. The titles of the 34 chapters in this volume exemplify and
are meant to clarify these controversies. Who to treat, when to treat, and how
to treat are the primary problems, but the numerous variations of the theme
such as syringomyelia, scoliosis, and associated bony abnormality are of
great importance.
All these issues are presented and discussed and various solutions are
brought forward. The authors are the world’s experts.
John A. Jane, Sr., MD, PhD, FRCS(C)
vii
Contents
1 Introduction and Classification of the Chiari
Malformations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1
R. Shane Tubbs and W. Jerry Oakes
2 The Chiari Malformations: A Historical Context. . . . . . . . . . . 5
R. Shane Tubbs and W. Jerry Oakes
3 Embryology of the Craniocervical Junction
and Posterior Cranial Fossa . . . . . . . . . . . . . . . . . . . . . . . . . . . . 13
Mohammadali M. Shoja, R. Shane Tubbs, and W. Jerry Oakes
4 Embryology and Pathophysiology of the
Chiari I and II Malformations . . . . . . . . . . . . . . . . . . . . . . . . . . 55
Mohammadali M. Shoja, R. Shane Tubbs, and W. Jerry Oakes
5 Surgical Anatomy of the Craniocervical Junction Relevant
to Chiari Malformations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 73
R. Shane Tubbs, Mohammadali M. Shoja, Marios Loukas,
and W. Jerry Oakes
6 Epidemiology of the Chiari I Malformation . . . . . . . . . . . . . . . 83
John D. Heiss
7 Genetics of the Chiari I and II Malformations . . . . . . . . . . . . . 93
Christina A. Markunas, Allison E. Ashley-Koch,
and Simon G. Gregory
8 Pathology of Chiari I and II Malformations. . . . . . . . . . . . . . . 103
Lucy B. Rorke-Adams
9 Pathology of Syringomyelia Due to Chiari Malformations . . . 121
Daniel Keith Harrison
10 Associated Disorders of Chiari Type I Malformations. . . . . . . 127
R. Shane Tubbs and W. Jerry Oakes
11 Chiari-Like Malformation in Dogs. . . . . . . . . . . . . . . . . . . . . . . 135
Dominic J. Marino and Curtis W. Dewey
12 Radiology of the Chiari Malformations. . . . . . . . . . . . . . . . . . . 153
Abby E. Deans and A. James Barkovich
ix