Syringomyelia: Diagnosis and Treatment

ISBN-13: 9783642626517 / Angielski / Miękka / 2012 / 195 str.

Jorg Klekamp; Madjid Samii

Opis

Syringomyelia has fascinated neurologists and neurosurgeons for decades, if not for centuries. The slowly progressing cystic cavitations of the spinal cord have led scientists and clinicians to various pathophysiological hypotheses and treatment strategies. Until recently, no clear concept existed as to what caused a syrinx and how and when a particular patient should be treated. The introduction of magnetic resonance imaging (MRI) has revolutionized our view of syringomyelia. For the first 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 with a noninvasive method. This has led to a huge amount of information not available to previous scientists. Nevertheless, pathophysiology and treatment are still contro- versial. The aim of this book is to give a guideline on how to approach a patient with a syrinx. Based on clinical experience, we have modified our strategies repeatedly in an 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 a solid basis for our decision making. Clinical courses, surgical indications, tech- niques, and postoperative results are discussed for each of the different pathologies known to be associated with syringomyelia. In this manner, we hope to give a co- herent overview on all aspects of syringomyelia, which should help physicians to counsel and treat patients with this fascinating but also potentially devastating dis- ease.

Spis treści

1 Introduction.- 1.1 Definitions of Cord Cavitations.- 1.2 History of Syringomyelia.- 1.2.1 Review of Pathophysiological Hypotheses.- 1.2.1.1 Dysraphic Hypothesis.- 1.2.1.2 Neoplastic Hypothesis.- 1.2.1.3 Inflammatory Hypothesis.- 1.2.1.4 Ischemic Hypothesis.- 1.2.1.5 Hematomyelic Hypothesis.- 1.2.1.6 Secretory Hypothesis.- 1.2.1.7 Transudation Hypothesis.- 1.2.1.8 Hydrodynamic Hypothesis.- 1.2.1.9 Pressure Dissociation Hypothesis.- 1.2.1.10 Transmedullary Hypothesis.- 1.2.1.11 Edema Hypothesis.- References.- 2 The Pathophysiology of Syringomyelia.- 2.1 Anatomical Background.- 2.2 Fluid Movements in the Extracellular and Subarachnoid Spaces.- 2.3 Tethered Cord and Extracellular Space.- 2.4 Pathophysiological Concept.- 2.5 Disorders Associated with Syringomyelia.- References.- 3 Syringomyelia Associated with Diseases at the Craniocervical Junction.- 3.1 Chiari Malformations.- 3.1.1 Clinical Presentation.- 3.1.2 Neuroradiology.- 3.1.2.1 Craniocervical Junction.- 3.1.2.2 Spine and Spinal Cord.- 3.1.3 Neurophysiology.- 3.1.4 Surgical Management.- 3.1.4.1 Hydrocephalus and Chiari Malformation.- 3.1.4.2 Decompression for Chiari I Malformation.- 3.1.4.3 Decompression for Chiari II Malformation.- 3.1.4.4 Basilar Invagination and Chiari Malformation.- 3.1.4.5 Craniocervical Instability and Chiari Malformation.- 3.1.4.6 Degenerative Changes of the Spine and Chiari Malformation.- 3.1.5 Postoperative Outcome.- 3.1.5.1 Complications.- 3.1.5.2 Radiological Results for Chiari I Malformation.- 3.1.5.3 Clinical Results for Chiari I Malformation.- 3.1.5.4 Postoperative Results for Chiari II Malformation.- 3.1.6 Management of Clinical Recurrences of Chiari Malformations.- 3.1.7 Conclusions for Patients with Chiari Malformations.- References.- 3.2 Rhombencephalic Malformations.- 3.2.1 Dandy-Walker Malformation.- 3.2.2 Atresia of Foramina of Luschka and Magendie.- References.- 3.3 Foramen Magnum Arachnoiditis.- 3.3.1 Clinical Presentation.- 3.3.2 Neuroradiology.- 3.3.3 Surgical Management.- 3.3.4 Postoperative Outcome.- References.- 3.4 Intracranial Tumors.- References.- 4 Syringomyelia Associated with Diseases of the Spinal Canal.- 4.1 Syringomyelia Related to Spinal Arachnoid Scarring.- 4.1.1 Clinical Presentation.- 4.1.1.1 Posttraumatic Syringomyelia.- 4.1.1.2 Postinflammatory Syringomyelia.- 4.1.2 Neuroradiology.- 4.1.3 Neurophysiology.- 4.1.4 Surgical Management.- 4.1.4.1 Arachnolysis and Decompression of the Spinal Canal.- 4.1.4.2 Surgical Strategy for Patients with Additional Degenerative Spinal Disease, Kyphotic Angulations, or Spinal Instabilities.- 4.1.4.3 Implantation of a Syrinx Shunt.- 4.1.5 Postoperative Outcome.- 4.1.5.1 Complications.- 4.1.5.2 Radiological Results.- 4.1.5.3 Clinical Results.- 4.1.6 Management of Clinical Recurrences of Spinal Arachnoid Scarring.- 4.1.7 Conclusions for Patients with Spinal Arachnoid Scarring.- References.- 4.2 Syringomyelia Related to Tumors of the Spinal Canal.- 4.2.1 Intramedullary Tumors.- 4.2.1.1 Clinical Presentation.- 4.2.2.2 Neuroradiology.- 4.2.1.3 Surgical Management159.- 4.2.1.4 Postoperative Outcome.- 4.2.2 Extramedullary and Epidural Tumors.- References.- 4.3 Syringomyelia Related to Spinal Dysraphism.- 4.3.1 Clinical Presentation.- 4.3.2 Neuroradiology.- 4.3.3 Surgical Management.- 4.3.4 Postoperative Outcome.- References.- 4.4 Syringomyelia Related to Diseases of the Spine.- 4.4.1 Degenerative Diseases.- 4.4.2 Spinal Scoliosis.- References.

Blurb

Magnetic resonance imaging (MRI) has revolutionized our understanding of many neurological diseases. This is the first textbook on syringomyelia by a single team of authors since the introduction of this imaging modality. Based on a pathophysiological concept which is presented in the opening chapters of this book, we provide guidelines for diagnosis and surgical treatment of patients with syringomyelia. Syringomyelia is a manifestation of cerebrospinal fluid (CSF) flow obstruction, spinal cord tethering, or a spinal cord tumor. Therefore, an underlying pathology characterized by at least one of these features can be identified for every patient. Each entity which may lead to syringomyelia is described in a separate chapter, covering clincial presentations, neuroradiological features, neurophysiology, surgical indications and techniques and a detailed analysis of postoperative results. Special sections are devoted to patients in whom surgical treatment failed initially.This book should help any physician dealing with a patient with syringomyelia to initiate the appropriate diagnostic test and studies to identify the underlying disorder. Based on the analyses of postoperative outcomes for each of these disorders, the patient may be advised to either undergo surgery or to continue conservative treatment. Neurosurgeons are given precise guidelines for surgical indications and strategies. Microsurgical techniques are illustrated step by step and include detailed intraoperative photographs.