Cerebral Palsy

Freeman Miller, MDCo-Director, Cerebral Palsy ProgramAlfred I. duPont Hospital for ChildrenNemours FoundationWilmington, Delaware

Cerebral Palsy

With CD-ROM

With 1116 Illustrations, 777 in Full Color

Illustrations by Erin Browne, CMI

Springer

Freeman Miller, MDCo-Director, Cerebral Palsy ProgramAlfred I. duPont Hospital for ChildrenNemours FoundationWilmington, DE 19899USA

Library of Congress Cataloging-in-Publication Data

Miller, Freeman.Cerebral palsy / Freeman Miller.

p. ; cm.Includes bibliographical references and index.ISBN 0-387-20437-7 (hc. : alk. paper)1. Cerebral palsied children—Rehabilitation. 2. Movement disorders in children. 3.

Cerebral palsy. I. Title.[DNLM: 1. Cerebral Palsy—rehabilitation—Child. 2. Cerebal Palsy—surgery—Child.

3. Disabled children—rehabilitation. WS 342 M647ca 2004]RJ496.C4M535 2004618.92′836—dc22 2003065734

ISBN 0-387-20437-7 Printed on acid-free paper.

© 2005 Springer Science+Business Media, Inc.All rights reserved. This work may not be translated or copied in whole or in part without thewritten permission of the publisher (Springer Science+Business Media, Inc., 233 Spring Street,New York, NY 10013, USA), except for brief excerpts in connection with reviews or scholarlyanalysis. Use in connection with any form of information storage and retrieval, electronic adap-tation, computer software, or by similar or dissimilar methodology now known or hereafterdeveloped is forbidden.The use in this publication of trade names, trademarks, service marks, and similar terms, evenif they are not identified as such, is not to be taken as an expression of opinion as to whetheror not they are subject to proprietary rights.While the advice and information in this book are believed to be true and accurate at the dateof going to press, neither the authors nor the editors nor the publisher can accept any legal re-sponsibility for any errors or omissions that may be made. The publisher makes no warranty,express or implied, with respect to the material contained herein.

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Cerebral palsy is a lifelong condition that affects the individual, family, andimmediate community. Therefore, the goal of allowing the individual withcerebral palsy to live life with the least impact of the disability requires com-plex attention to the individual and the family. Furthermore, society needsto be sensitive and to accommodate individuals with disabilities by limitingarchitectural impediments and providing accessible public transportationand communication. The educational system provides the key means forhelping the individual prepare to function in society to his or her maximumability. In many ways, the medical care system probably has the least signif-icant role in preparing the child with cerebral palsy to function optimally insociety. However, the medical care system is the place where parents firstlearn that their child has developmental issues outside the expected norm. Itis almost universally the place where parents also expect the child to be madenormal in our modern society. In earlier times, the parents would expecthealing to possibly come from the doctor, but also they would place hope forhealing in religion. As this belief in spiritual or miraculous healing has de-creased, a significant font of hope has decreased for parents of young chil-dren with disabilities.

The text aims to help the child with cerebral palsy to develop into anadult in whom the effects of the disability are managed so that they have theleast impact possible on adult function. This intention is in the context of thefact that the magnitude of improvement in the disability that occurs withideal management of the musculoskeletal system during growth may be onlya small improvement. Probably the more significant aspect of good muscu-loskeletal management through childhood is helping the child and family tomaintain realistic hope for the successful adult life of the growing child. Thisaim requires the medical practitioner to get to know the child and family andto communicate in a compassionate way realistic expectations of the child’sfunction. For many reasons, the greatest difficulty in providing this kind ofcare is the limited time practitioners have to spend with the individual pa-tient. There is also the sense, especially among orthopedic physicians, thatcerebral palsy cannot be cured (cannot make the child function normally),and thus it is a frustrating condition with which to work. The physician mustmaintain a balance between communicating hopelessness to the patient andfamily; and feeling the need to do something, usually a heel cord lengthening,because the parents are frustrated that the child is not progressing. All med-ical decisions, including a surgical option, should always consider both theshort- and long-term impact. With every decision the medical practitionershould ask, “What will be the impact of this recommendation by the timethe child is a mature adult?” This is the most difficult perspective, especially

Preface

for young practitioners with little experience. This text is intended to pro-vide this insight as much as possible.

Another issue is the poor scientific documentation of natural history andtreatment response in cerebral palsy, which has become clearer to me in thecourse of writing this book. With little scientifically based natural historyand few long-term studies, much of what is written in this text is expert-based observation. The goal of writing this is not to say that it is absolutefact, but to provide the starting point of gathering information with the hopethat others will be stimulated to ask questions and pursue research to proveor disprove the concepts.

The research, which is of help in treating children with cerebral palsy,needs to be planned and evaluated with consideration of its long-term im-pact on the child’s growth and development. All treatment should also con-sider the negative impact on the child. As an example, a number of moder-ately good studies have analyzed the impact of wearing ankle orthotics onthe young child. Although the orthotics may provide an immediate benefitby improving the child’s gait, there is probably no long-term benefit. Thus,if the child develops a strong sense of opposition to wearing the brace at 10years of age because of peer pressure, the brace wear cannot be justified ona cost–benefit analysis.

It is also important to consider the quality of the scientific evidence, rang-ing from double-blinded protocols to case reports, but it is equally importantnot to get hung up on this being the final answer. For example, excellentdouble-blinded studies show that botulinum toxin decreases spasticity andimproves gait for a number of months. Therefore, these studies need to beconsidered in the context of our goal, which is to give the child the maximumpossible function at full maturity. Because no evidence currently suggests thatbotulinum has either a negative or a positive effect on this long-term goal, thefamily and physician should decide together if botulinum injection has a pos-itive cost–benefit ratio, as its effects will last only for approximately 6 months.In comparison, no double-blind studies show that Achilles tendon lengthen-ings improve gait three or six months after the surgery, and no such studiesare needed because the goal of surgery is to make an improvement in gaitseveral years later and to have improvement at maturity. Most important isthat surgery create no disability at maturity. From this perspective, it wouldbe much more useful to have a good controlled case series with a 15-yearfollow-up than a double-blinded study with six months follow-up.

This book should stimulate research that will improve the knowledge basewhich is focused on the long-term outcome of treatments. However, just be-cause the scientific knowledge base is poor does not mean that we should notapply the best knowledge available to current patients. In addition to research,an individual professional can best extend his or her knowledge base throughpersonal experience. This means that the child and family should be followedover time by the same practitioner with good documentation. By far, my bestsource of information has been the children whom I have followed for 10 to20 years with videotapes every year or two. Practitioner experience is ex-tremely important for augmenting the relatively poor scientific knowledgebase for musculoskeletal treatment. Careful ongoing follow-up is also crucialto providing hope for the families and the individuals with cerebral palsy.

How to Use This Text and CDThe text of Cerebral Palsy is set up in three sections, with the first sectioncontaining 11 chapters that deal with specific aspects of cerebral palsy from

vi Preface

the perspective of managing the motor impairment. The first four chaptersaddress primarily global issues of the child and family. The fifth chapter pro-vides an overview of the medical and community care system and philosophy.The sixth chapter addresses the myriad options of equipment encountered inthe treatment of the musculoskeletal impairment, and the seventh addressesthe problems related to gait. The last four chapters are related to problemsencountered in anatomical regions. These chapters also include managementof the deformities and management of complications of medical treatment.These chapters are followed by treatment algorithms relevant to the issuesdiscussed within the chapter. Also included in the chapters are patient caseexamples. Many of the case examples have videos and are designated by avideo symbol. Each case has a unique name assigned to the patient and thevideo is accessed by opening the CD followed by opening the movies section,then opening the video with the same name. The second section of the bookincludes descriptions of techniques in rehabilitation often used with childrenwith cerebral palsy. The third section is a surgical atlas with the proceduresorganized by anatomical area.

The CD included with this text is opened with a Web browser. Because thedata on the CD is coded with XML and JAVA, only browsers released after2002, such as Netscape 7.0, Explorer 6.0 or Safari, will be fully able to accessthis data. Some of the text in the book is organized in topics and is displayedin the section entitled “Main.” All references on the CD have the abstractavailable on the CD by activating the link associated with the reference.Cases can also be activated from these references in the Main section. Thereis also a section called “Cases,” which lists all the cases by name as listed inthe text of the book. Following these cases are short quiz questions, whichcan be used to test understanding or study the material on line. There is alsoa section called “Quizzes,” which lists the quizzes by name of the cases. Thesequizzes can be opened and answered referring to the full case descriptions.The answers from the quizzes will be tabulated to keep a running total ofcorrect answers for each session. After a quiz is accessed, it will also changecolor to remind the reader that he has already reviewed that quiz. The sec-tion entitled “Decision Trees” is the treatment algorithms, which are presentat the end of each chapter in the book. These decision trees are set up so thatarea of interest is linked to the text in “Main” for further reading. The sectioncalled “Search” is an electronic index to search for specific subjects with in thechapter of the section “Main.” Because of space limitations, only individualchapters can be searched at one time. So if you want to search for “crutches,”you first should activate the Durable Medical Goods chapter, and then search.The results of the search allow you to directly link to the area of interest. Thesection “History” keeps a running history of the areas that have been assessed,so if you want to return to an area you were reading earlier in the session youcan open the history and it will allow you to return to that area. The section“About” includes information on the use of the CD and acknowledgments.

In summary, the CD includes videos, case study quizzes, and referenceabstracts, which are not inclnded in the book. The book includes significantportions of text not included on the CD, sections on rehabilitation techniques,and a surgical atlas. The book and the CD are intended to complement eachother but each can also be used alone.

AcknowledgmentsThe production of this book and CD was only possible because of an ex-tensive network of support that was available to me. The support of the

Preface vii

administration of the Nemours Foundation, especially the support of RoyProujansky and J. Richard Bowen in giving me time to work on this projectwas crucial. It was only through the generous support in caring for my pa-tients by my partners and staff, Kirk Dabney, Suken Shah, Peter Gabos,Linda Duffy, and Marilyn Boos, that I was able to dedicate time to writing.I am very grateful for the generous material provided by all the contributorsand for the extensive and extremely important role of the feedback given tome by the consultants. In spite of having an extremely busy practice, KirkDabney still found time to read all of the first section, making very valuableimprovements, and writing major sections of the upper extremity chapter.With his wide experience, Michael Alexander made an excellent contribu-tion in the editorial support of the section on rehabilitation. The task of writ-ing and editing would have been impossible without the dedicated work ofKim Eissmann, Linda Donahue, and Lois Miller. Production of the CD in-volved a significant amount of detailed editing and HTML coding, most ofwhich was performed by Linda Donahue. To add a personal touch to the cases,a unique name was assigned by Lois Miller. The CD required a great effort oftechnical programming to make it work intuitively on all computer formats.Tim Niiler patiently persisted with this frustrating task until it all worked.Videos were masked and formatted by Robert DiLorio. Production of thegraphics was a major effort in understanding the complex material in whichErin Browne excelled. This production would have been impossible withouther dedication to understanding the concepts and bringing them to visualclarity. I would also like to thank the staff of Chernow Editorial Services,especially Barbara Chernow. Without the long support through out the evo-lution of this book by Robert Albano and his staff at Springer, this projectwould also have been much more difficult. And finally, I am most gratefulfor the many families and children who have allowed me to learn from themwhat it is like to live with the many different levels of motor impairments.It is to the families and children that I dedicate this work in the hope that itwill lead to improved care and understanding by medical professionals.

Freeman Miller, MD

viii Preface

Preface........................................................................................ v

Contributors .............................................................................. xi

Section I Cerebral Palsy Management

1 The Child, the Parent, and the Goal .......................................... 3

2 Etiology, Epidemiology, Pathology, and Diagnosis ...................... 27

3 Patient Management .................................................................. 51

4 Neurologic Control of the Musculoskeletal System .................... 95

5 Therapy, Education, and Other Treatment Modalities ................ 151

6 Durable Medical Equipment ...................................................... 181

7 Gait ............................................................................................ 251

8 Upper Extremity ........................................................................ 387

9 Spine .......................................................................................... 433

10 Hip ............................................................................................ 523

11 Knee, Leg, and Foot.................................................................... 667

Section II Rehabilitation Techniques

1 Neurodevelopmental Therapy .................................................... 805Elizabeth Jeanson

2 Strengthening Exercises .............................................................. 806Diane Damiano

3 Balance Interventions.................................................................. 808Betsy Mullan

4 Electrical Stimulation Techniques .............................................. 809Adam J. Rush

5 Hippotherapy ............................................................................ 810Stacey Travis

6 Aquatic Therapy ........................................................................ 811Jesse Hanlon and Mozghan Hines

Contents

7 Assistive Devices ........................................................................ 818Mary Bolton

8 Seating Systems .......................................................................... 821Denise Peischl, Liz Koczur, and Carrie Strine

9 M.O.V.E.™ (Mobility Opportunities Via Education) Curriculum................................................................ 828Kristin Capone, Diana Hoopes, Deborah Kiser, and Beth Rolph

10 Occupational Therapy Extremity Evaluation.............................. 831Marilyn Marnie King

11 Intrathecal Baclofen Pumps ........................................................ 851Maura McManus

References .................................................................................. 858

Section III Surgical Techniques

1 Upper Extremity Procedures ...................................................... 865

2 Spinal Procedures ...................................................................... 894

3 Hip Procedures .......................................................................... 913

4 Knee Operative Procedures ........................................................ 955

5 Foot and Ankle Procedures ........................................................ 979

Index .......................................................................................... 1025

x Contents

Associate Editors

Kirk Dabney, MDMichael Alexander, MD

Contributors

Mary Bolton, PTKristin Capone, PT, MEdHenry Chambers, MDDiane Damiano, PhD Federico Fernandez-Palazzi, MDPatricia Fucs, MDJesse Hanlon, BS, COTA Mozghan Hines, LPTA Diana Hoopes, PTElizabeth Jeanson, PTMarilyn Marnie King, OTR/LDeborah Kiser, MS, PT Liz Koczur, MPTMaura McManus, MD, FAAPMR, FAAP

Betsy Mullan, PT, PCSDenise Peischl, BSEBeth Rolph, MPTAdam J. Rush, MDCarrie Strine, OTR/LStacey Travis, MPTJoaquin Xicoy-Forges, MD

Consultants

Steven Bachrach, MDJohn Henley, PhDDouglas Heusengua, PTHarry Lawall, CPOStephan T. Lawless, MDGary Mickalowski, CPO Edward Moran, CPOSusan Pressley, MSWJames Richards, PhD Mary Thoreau, MDRhonda Walter, MD

Contributors

SECTION I

Cerebral PalsyManagement