The Chiari Malformations

377 Pages·2013·14.786 MB·English

by  R. Shane Tubbs MS, PA-C, PhD, W. Jerry Oakes MD (auth.), R. Shane Tubbs, W. Jerry Oakes (eds.)

R. 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
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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