J. Klekamp . M. Samii Syringomyelia

Springer-Verlag Berlin Heidelberg GmbH

Jorg Klekamp Madjid Samii

• In omyelia Diagnosis and Treatment

With Contributions by Cordula Matthies to the Text on Neurophysiology

With 113 Figures in 456 Separate Illustrations, Some in Colour, and 36 rabies

Springer

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Privat-Dozent Dr. JORG KLEKAMP

Professor Dr. MADJlD SAM II

Klinikum Hannover Nordstadt Neurochirurgische Klinik Haltcnhoffstrasse 4 1 30167 Hannover, Germany

e-mail: JoergKlekamp@t-online.de

ISBN 978-3-642-62651-7

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Syringom)'t'lia: diagnosi5 aoo trtatmmt J laerg Klekamp, Madjid Samii. p.;cm.

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For Our Families

Preface

Syringomyelia has fascinated neurologists and neurosurgeons for decades, if notfor centuries. The slowly progressing cystic cavitations of the spinal cord have ledscientists and clinicians to various pathophysiological hypotheses and treatmentstrategies. Until recently, no clear concept existed as to what caused a syrinx andhow and when a particular patient should be treated. The introduction of magneticresonance imaging (MRI) has revolutionized our view of syringomyelia. For thefirst time, we are able to diagnose a syrinx before it produces clinical symptoms,and we can follow the course of the syrinx before and after surgical treatment witha noninvasive method. This has led to a huge amount of information not availableto previous scientists. Nevertheless, pathophysiology and treatment are still contro­versial.

The aim of this book is to give a guideline on how to approach a patient with asyrinx. Based on clinical experience, we have modified our strategies repeatedly inan attempt to improve clinical results. We have developed a treatment concept,evolved out of 20 years of clinical and experimental work, which has proven to be asolid basis for our decision making. Clinical courses, surgical indications, tech­niques, and postoperative results are discussed for each of the different pathologiesknown to be associated with syringomyelia. In this manner, we hope to give a co­herent overview on all aspects of syringomyelia, which should help physicians tocounsel and treat patients with this fascinating but also potentially devastating dis­ease.

Finally, we wish to acknowledge the tremendous impact that cooperation withUlrich Batzdorf at the University of California in Los Angeles had on our work inthis field over many years. Fruitful discussions and this ongoing cooperationhelped to improve treatment strategies. Clinical and also experimental studies wereperformed together, which led to several publications. Most of the statistics of thisbook are based on the collaborative research of the Departments of Neurosurgeryat the Klinikum Hannover Nordstadt and the University of California.

Hannover, June 2001 [org KlekampMadjid Samii

Acknowledgements. We wish to express our thanks to Dr. C. Matthies for her eval­uation of neurophysiological results for patients with syringomyelia. Her protocol,outlined in Chap. 3.1.3., was of tremendous help during surgery. Drs. R.-H. Pra­witz, A. Majewski, and Prof. B. Terwey provided excellent neuroradiological infor­mation for surgical planning and postoperative controls for our patients. Their ex­pertise had a strong impact on our understanding of neuroradiological features ofpatients with an often malformed and severely altered anatomy. Prof. B. Terweyprovided the excellent phase-contrast cine MRI studies.

Contents

1 Introduction 1

1.1 Definitions of Cord Cavitations 1

1.2 History of Syringomyelia 41.2.1 Review of Pathophysiological Hypotheses 7

1.2.1.1 Dysraphic Hypothesis 7

1.2.1.2 Neoplastic Hypothesis 7

1.2.1.3 Inflammatory Hypothesis 7

1.2.1.4 Ischemic Hypothesis 8

1.2.1.5 Hematomyelic Hypothesis 8

1.2.1.6 Secretory Hypothesis 9

1.2.1.7 Transudation Hypothesis 9

1.2.1.8 Hydrodynamic Hypothesis 9

1.2.1.9 Pressure Dissociat ion Hypothesis 10

1.2.1.10 Transmedullary Hypothesis 11

1.2.1.11 Edema Hypothesis 11

References 11

2 The Pathophysiology of Syringomyelia 17

2.1 Anatomical Background 17

2.2 Fluid Movements in the Extracellular andSubarachnoid Spaces 18

2.3 Tethered Cord and Extracellular Space 18

2.4 Pathophysiological Concept 19

2.5 Disorders Associated with Syringomyelia 21

References 22

3 Syringomyelia Associated with Diseasesat the Craniocervical Junction 27

j.i Chiari Malformations 27

3.1.1 Clinical Presentation 30

3·1.2 Neuroradiology 34

3·1.2.1 Craniocervical Junction 34

3.1.2.2 Spine and Spinal Cord 37

3·1.3 Neurophysiology 39

J.l.4 Surgical Management 43

3.1.4.1 Hydrocephalus and Chiari Malformation 43

3.1.4.2 Decompre ssion for Chiari I Malformation 46

3.1.4.3 Decompression for Chiari II Malformation 52

3.1.4.4 Basilar Invagination and Chiari Malformation 56

J.l.4.5 Craniocervical Instability and Chiari Malformation 59

3.23.2 .1

3·2.2

3·33·3·13·3·23·3-33·3-4

301·5301·5·1301·5·2301·5·33·1.5·43·1.6

301·7

X Contents

3.1.4.6 Degenerative Changes of the Spineand Chiari Malformation 59Postoperative Outcome 62Complications 62Radiological Results for Chiari I Malformation 65Clinical Results for Chiari I Malformation 65Postoperative Results for Chiari II Malformation 72Management of Clinical Recurrences of Chiari Malformations 73Conclusions for Patients with Chiari Malformations 84References 85

Rhombencephalic Malformations 92Dandy-Walker Malformation 92Atresia of Foramina of Luschka and Magendie 94References 97

Foramen Magnum Arachnoiditis 98Clinical Presentation 98Neuroradiology 101Surgical Management 101Postoperative Outcome 103References 104

3.4 Intracranial Tumors 106References 109

4 Syringomyelia Associated with Diseases of the Spinal Canal III

4.1 Syringomyelia Related to Spinal Arachnoid Scarring 111

4.1.1 Clinical Presentation 1124.1.1.1 Posttraumatic Syringomyelia 1124.1.1.2 Postinflammatory Syringomyelia 1154.1.2 Neuroradiology 1164.1.3 Neurophysiology 1264.1.4 Surgical Management 1274.1.4.1 Arachnolysis and Decompression of the Spinal Canal 1274.1.4.2 Surgical Strategy for Patients with Additional Degenerative Spinal Disease,

Kyphotic Angulations, or Spinal Instabilities 1334.143 Implantation of a Syrinx Shunt 1334.1.5 Postoperative Outcome 1374.1.5-1 Complications 1374.1.5-2 Radiological Results 1374.1.5.3 Clinical Results 1384.1.6 Management of Clinical Recurrences

of Spinal Arachnoid Scarring 1454.1.7 Conclusions for Patients with Spinal Arachnoid Scarring 152

References 152

4.2 Syringomyelia Related to Tumors of the Spinal Canal 1574.2.1 Intramedullary Tumors 1574.2.1.1 Clinical Presentation 1584.2.1.2 Neuroradiology 1584.2.1.3 Surgical Management 1594.2.1.4 Postoperative Outcome 1654.2.2 Extramedullary and Epidural Tumors 170

References 172

4.3 Syringomyelia Related to Spinal Dysraphism 174

4.3.1 Clinical Presentation 174

4.3.2 Neuroradiology 176

4.3.3 Surgical Management 181

4.3.4 Postoperative Outcome 184

References 187

4.4 Syringomyelia Related to Diseases of the Spine 188

4.4.1 Degenerative Diseases 188

4.4.2 Spinal Scoliosis 190

References 192

Subject Index 193

Contents XI

General Information

Each chapter of this book has been written in a way that it can be read as a separatesection without the need to go through previous ones. Pre- and postoperative neu­rological symptoms are analyzed according to a scoring system (Table 1) [3]. Addi­tionally, the overall clinical condition is documented according to the Karnofskyscore [2]. Success of treatment requires at least the stopping of a progressive neuro­logical course. When a patient develops progressive neurological symptoms aftersurgery, this is defined as a clinical recurrence. Using clinical recurrences as an in­dicator for treatment failure, long-term results are documented with survival statis­tics [i]. Unlike the majority of publications on syringomyelia, which present onlypercentages of patients with postoperative improvement, stabilization, or dete­rioration, this method allows accounting for varying follow-up times and gives amuch more realistic picture of postoperative results.

The overwhelming majority of operations illustrated in this book were per­formed in the prone position. All intraoperative photographs are oriented in thesame fashion: cranial to the left, caudal to the right. When the semi-sitting posi­tioning was used, this is mentioned in the figure legend and the photograph is pre­sented according to the surgeon 's view.

Table 1. Neurological scoring system

Score Sensory dist., Motor weakness Gait ataxia Bladder function Bowel functionswallowing,pain,dysesthesias

References1. Kaplan EL, Meier P (1958) Nonparametric estimation from incomplete observa­

tions. JAm Stat Assoc 53: 457-4812. Karnofsky DA, Burchenal JH (1949) The clinical evaluation of chemotherapeutic

agents in cancer. In: MacLeod CM (ed) Evaluation of chemotherapeutic agents.Columbia University Press, New York, pp 191-205

3· Klekamp J, Samii M (1993) Introduction of a score system for the clinical evalua­tion of patients with spinal processes. Acta Neurochir (Wien) 123: 221-223