Introduction I
MEDICAL RADIOLOGY
Diagnostic Imaging
Softcover Edition
Editors:
A. L. Baert, Leuven K. Sartor, Heidelberg
Introduction III
cmyk W. S. Müller-Forell (Ed.)
Imaging of Orbital and Visual
Pathway Pathology
With Contributions by
E. Boltshauser · S. Kollias · W. Lieb · E. Martin · W. S. Müller-Forell · S. Pitz U. Schwarz · W. Wichmann
Foreword by
K. Sartor
Preface by
N. Pfeiffer
With 433 Figures in 1368 Separate Illustrations, 66 in Color
123
IV Introduction
PD Wibke S. Müller-Forell, MD Institute of Neuroradiology
Medical School University of Mainz Langenbeckstrasse 1
55101 Mainz Germany
Medical Radiology · Diagnostic Imaging and Radiation Oncology Series Editors: A. L. Baert · L. W. Brady · H.-P. Heilmann · F. Molls · K. Sartor Continuation of
Handbuch der medizinischen Radiologie Encyclopedia of Medical Radiology
ISBN 3-540-27988-1 Springer-Verlag Berlin Heidelberg New York ISBN 978 3-540-27988-4 Springer-Verlag Berlin Heidelberg New York
Library of Congress Cataloging-in-Publication Data
Imaging of orbital and visual pathway pathology / W. S. Müller-Forell (ed.) ; with contributions by E. Boltshauser ... [et al.] ; foreword by A. L. Baert.
p. ; cm. – (Medical radiology)
Includes bibliographical references and index.
ISBN 3540633022 (hardcover; alk. paper) ISBN 3540279881 (softcover; alk. paper) 1. Eye--Imaging. 2. Visual pathways--Imaging. I. Müller-Forell, W. S. (Wibke S.), 1949- II. Series.
[DNLM: 1. Orbital Diseases--pathology. 2. Diagnostic Imaging. 3. Orbit--pathology. 4.
Orbital Diseases--diagnosis. 5. Orbital Diseases--radiography. 6. Visual Pathways--pathology. WW 202 I31 2002]
RE79.I42 I534 2002
617.7’0754--dc21 2001049328
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Introduction V
cmyk
Dedicated to
Professor Dr. Sigurd Wende
1924–1991
An extraordinary neuroradiologist, a challenging teacher
and a wonderful paternal friend.
Introduction VII
Foreword
Most information about the external world enters the human mind via the visual system;
when seeing and looking are impaired, important aspects of life may elude us or the world becomes disturbingly distorted. While the globe may be likened to a camera, it is the brain that constructs an image of the world. It does this by making sense of the sig- nals it receives from the retina, in which it is helped by data stored in memory as well as data gathered through other modes of perception. The retinal signals, induced by pho- tons hitting photoreceptor cells, travel fi rst by way of the optic nerves, optic chiasm and optic tracts to relay stations, the lateral geniculate bodies, then via the optic radiations to the back of the occipital lobes; some signals, however, are shunted from the lateral geniculate bodies to the brainstem. The primary visual cortex needs the adjacent “asso- ciation” cortex to provide a fi rst rendition of the image of the world. Further input from other cortical and subcortical areas completes the image, for example, by adding an affective quality to it. Most „images“ are fi nally elevated to the level of conscious- ness but some remain subconscious, although not necessarily without important conse- quences. This (parallel) processing of visual signals is accompanied by and related to neural mechanisms that precisely coordinate eye (and head/body) movement. A system this complex may become impaired in many different places and almost invariably will become impaired sooner or later. Pathologies that impair the system‘s anatomy and function may be intrinsic, that is, confi ned to the optic pathways, or extrinsic, i.e., arising in neighboring structures. Since the visual system extends from the anterior circumfer- ence of the globe to the tip of the occipital pole, there is a tremendous potential for intrinsic and extrinsic pathologies.
There was a time when the term “monograph” frequently indicated not only that the book thus classifi ed dealt with just one subject but also that it was written by just one person; being an expert on a certain subject was synonymous with knowing everything there was to know about this subject. Today, because of the literally exponential growth of knowledge in almost all areas of medicine, it generally takes several or many experts to cover even “small” subjects. Furthermore, it is no longer considered suffi cient to deal with a subject from one – let us say the radiologist’s – point of view alone. How can one satisfactorily write on modern imaging of the visual system without asking the neuro-ophthalmologist – who best understands the system‘s function and who also uses some imaging – or the clinical scientist experienced in the pertinent pathology to par- ticipate? Even with regard to radiologic imaging the (neuro-)radiologist would be ill advised to focus on morphology alone, ignoring the tremendous progress that clinically practicable fMRI has made in recent years.
Consequently, Dr. Müller-Forell, a neuroradiologist, has teamed up with several inter- nationally renowned experts from related clinical fi elds to realize her ambitious project, a comprehensive treatise on imaging of the visual system “front to back”. As there is
VIII Introduction
cmyk
nothing of similar scope on the market, this represents a most welcome and timely endeavor. The resulting book, thoroughly organized and an admirable feat, conveys invaluable information on methodology, normal anatomy, and orbital and intracranial pathology, as well as on normal and abnormal function. I am convinced that the book‘s superior quality will ensure its warm reception by all clinicians interested in this impor- tant topic, including radiologists, ophthalmologists, neurologists, neurosurgeons, and otorhinologists.
Heidelberg Klaus Sartor
Introduction IX
Preface
As an ophthalmologist, I feel greatly honored to have been asked to write a preface for this book. Orbital and visual pathway pathology is a fi eld comprising a multitude of medical disorders that overlap the fi elds of ophthalmology, otorhinolaryngology, neurosurgery, general medicine and, of course, the diagnostic disciplines, especially neuro radiology. Diagnosis of many of these disorders is highly challenging. Fortunately, with the development and refi nement of imaging techniques, especially CT and MRI, tremendous advances have been accomplished. Yet even with these new methods at hand diagnosis is usually far from being straightforward. This book, edited by Wibke Müller-Forell with contributions from herself, E. Boltshauser, S. Kollias, W. Lieb, E. Martin, S. Pitz, U. Schwarz and W. Wichmann, will greatly facilitate this task.
The general part not only describes ophthalmologic imaging techniques in a detailed, state-of-the-art way but also displays the anatomy of the visual pathway from the outer orbit to the visual cortex. The images are of extremely high quality, and many will fi nd anatomical details that they have not discovered previously. Much and successful effort was also put into the summary of the relevant neuro-ophthalmology, and this will prove to be helpful for many readers. The same is even more true for the chapter on functional magnetic resonance imaging. This relatively new technique needs to be understood so that it can be used to its full extent.
The special part with its display of orbital and intracranial pathology of the optic pathway is exhaustive. I found it diffi cult to fi nish this preface, because even when proof- reading the book it turned out to be a compulsive page-turner. It is richly illustrated throughout with real cases, often comprising a photo of the patient, a case history, and a display of the adequate imaging methods, accompanied by the description of the treat- ment and histological results. While it is a systematic book, the material is arranged in such a way that the cases can even be read as a quiz. I found many cases similar to those I have seen in my practice, but I also found cases that taught me what I perhaps should have diagnosed, but did not, in the past. Especially helpful is the chapter on optic path- way pathology in children. It is, I believe, unique and will be extremely helpful for those who see children with neuro-ophthalmological problems.
The reader will soon discover that this book is based upon a tremendous amount of clinical experience and knowledge of real cases of the kind that arise daily from the inter- disciplinary approach and co-operation between neuroradiologists and other clinicians as mentioned above. Few – if any – have such a fundus of clinical experience at their fi ngertips. I am sure that this book will enormously help everybody who is entrusted with the clinical problem of diffi cult-to-diagnose diseases of the orbit and visual pathway.
I wish the book well, and I am sure it will be received with great enthusiasm.
Mainz Norbert Pfeiffer
Introduction XI
cmyk
Imaging of the pathology of the entire visual system has thus far been given room for only a limited discussion in a small number of comprehensive neuroradiological text- books. This is the fi rst textbook and atlas dealing with the diseases of the entire visual pathway. The title of this book emphasizes that the subject is not restricted to the orbit, but is extended to the pathologies which may affect the visual pathway from the lens to the striate cortex of the occipital lobe. The book provides a context for the history and/or clinical symptomatology of individually involved parts of the visual system and the corresponding pathological fi ndings, to ensure that all physicians involved in the treatment of disorders of the visual system, regardless of whether they are neuroradi- ologists, ophthalmologists, oto-/rhinolaryngologists, neurosurgeons or neurologists will fi nd this textbook an invaluable source of practical and theoretical knowledge.
In the fi rst section of the book particular attention is given to the most important current imaging methods, including ultrasound, computer tomography and magnetic resonance imaging, although here the focus is less on the physical aspects dealt with in greater depth in purely neuroradiological textbooks (for interested readers the respec- tive radiological textbooks are indicated in the reference section). Even though the pur- pose of this book is to assist referring physicians in their decision as to which method is best suited to provide the most specifi c information to their questions, we are not offer- ing any ready-made “cooking recipes”, because each individual patient requires an indi- vidual examination protocol. Another chapter of this book is devoted to the detailed dis- cussion of imaging CT- and MR-anatomy of the orbit and the intracranial/intracerebral visual pathway. The chapter on neuroophthalmology is designed to provide comprehen- sive knowledge of this complex fi eld and the great variety of related diagnostic criteria.
The following chapter discusses the most current developments in functional MR-imag- ing (fMRI) of the visual system, as well as indications for applications of the method, and results obtained in its use.
In the fi rst chapter of the special part an overview of the complexity of visual impair- ment in newborns and children is presented, a fi eld which is generally discussed only in special pediatric or neuroradiological textbooks. The focus of this special part of the book is the on the presentation of individual patient histories, symptoms and imaging (-in some cases- histological) fi ndings. Equal importance is given to both the discus- sion of the accurate diagnosis and the illustration of various imaging modalities. CTs in relevant windows (soft tissue, bone) and different, multiple MR-sequences are presented to demonstrate different diagnostic criteria in different patients with similar clinical symptoms, their relevance and results. The anatomy of the visual pathway is meticu- lously characterized in the presentation of the course of the pathology from the orbit, the prechiasmatic and postchiasmatic intracranial regions, to the occipital lobe, though the occurrence of redundant histological diagnoses is inevitable. Since the patients chosen
Introduction
XII Introduction
for presentations are from our own patient population, on the other hand, some histolo- gies may be missing. The reason for this is that especially in the intracranial space there is no exclusivity of all possible pathologies.
I feel deeply indebted to a large number of people for their support, on a personal as well as on a practical level. First and foremost I wish to thank Prof. Dr. Fritz Heuck for his patience and constant support over the past few years, and for his fi rm conviction that this work was to come to successful completion. My dear friend Dr. Renate Gustorf- Aeckerle merits many, many thanks for her active role the initiation of the book and for her continued encouragement all through this project. It gives me great pleasure to thank all my co-authors for their substantial efforts and contributions. In particular the wonderful active cooperation of Dr. Susanne Pitz deserves special thanks, as does the prolonged daily cooperation with Prof. Dr. Wolfgang Lieb. I am very pleased and indeed proud that the extended period of time I was given the opportunity to spend at the Institute of Neuroradiology of the University Hospital of Zurich (USZ) resulted in the fruitful cooperation with the following well-known and respected specialists: Prof.
Dr. Boltshauser and Prof. Dr. Martin-Fiori of the Children’s University Hospital, Prof.
Dr. Wichmann of the Department of Neuroradiology at the Klinik im Park, Priv. Doz.
Dr. Spyros Kollias, Institute of Neuroradiology, and Priv. Doz. Dr. Urs Schwarz from the Clinic of Neurology of the USZ, who all contributed in such a wonderful way to the pres- ent tome.
There are many individuals without whose contributions to the realisation of this atlas would not have been possible: Special thanks are in order for both the excellent work and the remarkably friendly cooperation of Mrs. Keuchel and her colleagues Mrs.
Soldevilla and Mrs. Nessler from the photographic laboratory of the Clinic of Radiol- ogy, University of Mainz. The expert realization of a consistent lay-out for the diagrams by Mr. Stefan Kindel, graphic artist at the Clinic of Neurosurgery of the University of Mainz, was of immense help. I would not have wanted to miss his prompt initiative, and his constructive, positive and always friendly cooperation. Special thanks are also due to Mrs. Gisela Rumsey, who corrected the manuscript in an admirable effort. The help of Mrs. Ursula Davis from Springer Verlag was overwhelming, especially since her support was not only needed, but provided in a particularly sympathetic and amicable manner that I will never forget.
Last but not least, I would like to offer grateful thanks to my beloved husband, Dr.
Hans-Joachim Forell, for his constant, loving and patient support, and for his tacit con- sent that this work was to be dedicated not to him, but to a common fatherly friend. His silent contribution is invaluable, especially since he had to spend many, many evenings and weekends alone, accompanied only by Felix (our cat).
Mainz Wibke S. Müller-Forell
Introduction XIII
cmyk Contents
General Part. . . 1
1 Ophthalmologic Imaging Methods Wolfgang E. Lieb, Wibke S. Müller-Forell, Werner Wichmann . . . 3
1.1 Color Doppler Ultrasonography of the Eye and Orbit Wolfgang Lieb . . . 3
1.2 Computed Tomography Wibke S. Müller-Forell . . . 15
1.3 Magnetic Resonance Imaging (MRI) Werner Wichmann . . . 18
2 Anatomy Werner Wichmann and Wibke S. Müller-Forell . . . 25
3 Neuro-ophthalmology: A Short Primer Urs Schwarz. . . 61
4 Functional Magnetic Resonance Imaging of the Human Visual System Spyros S. Kollias . . . 107
Special Part. . . 125
5 Optic Pathway Pathology in Children Eugen Boltshauser and Ernst Martin. . . 127
6 Orbital Pathology Wibke S. Müller-Forell and Susanne Pitz . . . 147
7 Intracranial Pathology of the Optic Pathway Wibke S. Müller-Forell. . . 341
Subject Index . . . 437
List of Anatomic Structures . . . 437
List of Acronyms . . . 445
List of Contributors . . . 447
Ophthalmologic Imaging Methods 1
General Part
