Cerebrospinal Fluid Disorders
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Cerebrospinal Fluid Disorders Malucci_978-0824728335_TP.indd 1 12/2/09 11:15:52 AM Cerebrospinal Fluid Disorders Edited by Conor Mallucci The Walton Centre for Neurology and Neurosurgery Liverpool, UK Spyros Sgouros “Attikon” University Hospital University of Athens Athens, Greece Malucci_978-0824728335_TP.indd 2 12/2/09 11:15:53 AM fm IHBK047-Mallucci November 25, 2009 13:32 Char Count= Informa Healthcare USA, Inc. 52 Vanderbilt Avenue New York, NY 10017 C 2010 by Informa Healthcare USA, Inc. Informa Healthcare is an Informa business No claim to original U.S. Government works Printed in the United States of America on acid-free paper 10987654321 International Standard Book Number-10: 0-8247-2833-5 (Hardcover) International Standard Book Number-13: 978-0-8247-2833-5 (Hardcover) This book contains information obtained from authentic and highly regarded sources. Reprinted material is quoted with permission, and sources are indicated. A wide variety of references are listed. Reasonable efforts have been made to publish reliable data and information, but the author and the publisher cannot assume responsibility for the validity of all materials or for the consequence of their use. No part of this book may be reprinted, reproduced, transmitted, or utilized in any form by any electronic, mechanical, or other means, now known or hereafter invented, including photocopying, microfilming, and recording, or in any information storage or retrieval system, without written permission from the publishers. For permission to photocopy or use material electronically from this work, please access www.copyright.com (http://www.copyright.com/) or contact the Copyright Clearance Center, Inc. (CCC) 222 Rosewood Drive, Danvers, MA 01923, 978-750-8400. CCC is a not-for-profit organization that provides licenses and registration for a variety of users. For organizations that have been granted a photocopy license by the CCC, a separate system of payment has been arranged. Trademark Notice: Product or corporate names may be trademarks or registered trademarks, and are used only for identification and explanation without intent to infringe. Library of Congress Cataloging-in-Publication Data Cerebrospinal fluid disorders / edited by Conor Mallucci, Spyros Sgouros. p.;cm. Includes bibliographical references. ISBN-13: 978-0-8247-2833-5 (hardcover : alk. paper) ISBN-10: 0-8247-2833-5 (hardcover : alk. paper) 1. Intracranial hypertension. 2. Central nervous system–Disease. 3. Cerebrospinal fluid–Physiology. 4. Cerebrospinal fluid shunts. I. Mallucci, Conor. II. Sgouros, Spyros. [DNLM: 1. Intracranial Hypertension–cerebrospinal fluid. 2. Central Nervous System Diseases–cerebrospinal fluid. 3. Cerebrospinal Fluid–physiology. 4. Cerebrospinal Fluid Shunts. 5. Hydrocephalus. WL 203 C41395 2009] RC386.2.C47 2009 616.8–dc22 2009033086 For Corporate Sales and Reprint Permission call 212-520-2700 or write to: Sales Department, 52 Vanderbilt Avenue, 7th floor, New York, NY 10017. Visit the Informa Web site at www.informa.com and the Informa Healthcare Web site at www.informahealthcare.com fm IHBK047-Mallucci November 25, 2009 13:32 Char Count= Preface When asked what my interests are and in what age group I practice, I often reply that my interests lie in “hydrocephalus and CSF disorders from the fetus to old age.” Indeed it is hard to separate or “cut off” one’s interest when a child enters adulthood still carrying these conditions, and equally, it is hard to understand how one can “limit” one’s interest or “expertise” to just the brain or spine when clearly the cerebrospinal fluid dynami- cally affects both the compartments. The dilemma and debate continues as to whether we as neurosurgeons should limit our practice to diseases affecting children (and call ourselves pedi- atric neurosurgeons) or adults, but if there were ever a condition warranting a disease-specific approach as opposed to “age” specific, then it is those diseases that affect the dynamics of the cerebrospinal fluid. This book has been conceived therefore to cover the gap in the literature on CSF disorders in a comprehensive text, which addresses the issues of disorders of the CSF pathways from birth until old age as well as to explore and manage the effects of such disorders on the brain and spine. As neurosurgeons and health professionals, we continue to subspecialize into predomi- nantly pediatric and adult sectors, and, in the case of neurosurgery, into those with predomi- nantly cranial or spinal interests. In addition, healthcare delivery to children and adults often divides and segregates the two at times with poor provision to bridge the gap between the two. The result is disjointed knowledge and pockets of expertise in a variety of these areas with no unifying text to address these divisions. The losers are often the patients, stuck between the disciplines of pediatric care and adult-delivered services with little clarity on who the “experts” are to pick up their case. In creating this book, we attempt not only to cover the understanding, diagnosis, and management of the common conditions, such as hydrocephalus, syringomyelia, and idiopathic intracranial hypertension across all ages, but also to create a reference useful to neurosurgeons and related specialties, such as neurology, pediatrics, general medicine and practice, ophthal- mology, radiology, and emergency medicine, alike. We hope that the text will also provide a useful reference to nursing professionals, caregivers, and advisory bodies, as well as incorpo- rating the patient’s perspective. With the advent of the Internet and patients’ access to information, we felt it vital to create a book that will provide a reference for health professionals as well as the patients and families that we treat. Many of the conditions we treat are carried for life, and patients and families need considerable support in building up their own knowledge of their condition. We hope this text will help to guide them in attaining the understanding and support that they need. This book is divided into four distinct sections that will be useful to different readers from many disciplines. We have invited a panel of international experts recognized in their field to share their knowledge with us. In addition to the conventional author-led chapters, you will also find “boxes” included within a chapter but written by different authors, designed to give brief updates on the literature or specific techniques and also to give different viewpoints on the subject. The first section serves as a background to CSF disorders and diseases including the pathophysiology of the CSF pathways; a fascinating chapter on the history of the treatment of CSF pathways by Dr Jim Goodrich, including many figures from his private collection not available or seen by the general public; and chapters covering neuropsychology, imaging, and antenatal screening. fm IHBK047-Mallucci November 25, 2009 13:32 Char Count= vi Preface The second section focuses on disease specific discussion of all disorders that affect the brain and the spine in all ages, thus including not only the usual causes of hydrocephalus in children and adults but also conditions often missed out in books on hydrocephalus such as idiopathic intracranial hypertension, Chiari malformation, and syringomyelia. The third section concentrates on the treatment techniques that unify all these conditions, necessary to any surgeon declaring an interest in the management of them. In particular, the focus is on the evidence and literature for the techniques and choices we need to make. In the final section, we thought it appropriate to address topics not necessarily addressed in such texts or those of particular interest. Thus, you will see interesting chapters on fetal surgery, ethical considerations in managing CSF disorders, the management of hydrocephalus in the developing world, and the most importantly, a chapter on the patients perspective. Finally, we would like to acknowledge and remember Dr Patrick Hanlo and Dr Tony Hockley, authors of two of the chapters, who died suddenly before the completion of this book, and hence they did not have the chance to see their work come to fruition. Dr Hockley’s sudden death earlier this year has touched especially both of us, as he trained both of us in our early careers setting us on our paths. He in particular was a strong supporter of the idea behind the book, as he had recognized that these conditions, although often starting in childhood, often affect the patient and the family throughout the whole lifetime and had devoted his career in treating these patients with due compassion, care, and attention to detail so that they can enjoy a long and healthy quality of life. The color printing of the illustrations in this book was made possible with the generous unconditional sponsorship from the following companies: Aesculap AG; BBraun Aesculap UK; Christoph Miethke GmbH und Co. KG; and Vamvas Medical Greece. Conor Mallucci Spyros Sgouros fm IHBK047-Mallucci November 25, 2009 13:32 Char Count= Contents Preface .... v Contributors .... xi Section 1: Introduction 1. An Anatomical and Physiological Basis for CSF Pathway Disorders 1 Andrew Brodbelt and Marcus Stoodley Box Embryology of CNS with Emphasis on CSF Circulation 18 Sergio Cavalheiro, Jardel Mendonc¸a Nicacio,´ and Igor Vilela Faquini 2. Hydrocephalus: Historical Review of Its Treatment 22 James Tait Goodrich Box Treatment of Hydrocephalus in the Modern Era (1950s–Current Date) 63 Paul May and Spyros Sgouros 3. Imaging (Normal and Abnormal) 66 Charles Raybaud and Gahl Greenberg 4. Invasive Measurements of Cerebrospinal Fluid Pressure 110 Marek Czosnyka, Zofia Czosnyka, and John D. Pickard Box Intracranial Pressure (ICP) Sensors and Surgical Technique of ICP Bolt Insertion 116 Federico Di Rocco 5. The Neuropsychological Consequences of Hydrocephalus 121 Cathy Grant, Jo Iddon, Emily Talbot, Kristina Vella, and Arleta Starza-Smith 6. Antenatal Screening and Management of Hydrocephalus 131 Wan Tew Seow and George Seow Heong Yeo Section 2: Disease-Specific Discussion 7. Posthemorrhagic Hydrocephalus 141 Cheryl Muszinski 8.