Syringomyelia as a presenting feature of shunt dysfunction: Implications for the pathogenesis of syringomyelia

The pathogenesis of syringomyelia continues to be an enigma. The patency of the central canal and its role in the pathogenesis of communicating syringomyelia continues to elicit controversy. The case reported here provides an opportunity to retest some of the hypotheses of syringomyelia. A 33 year o...

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Main Author: Natarajan Muthukumar
Format: Article
Language:English
Published: Wolters Kluwer Medknow Publications 2012-01-01
Series:Journal of Craniovertebral Junction and Spine
Subjects:
Online Access:http://www.jcvjs.com/article.asp?issn=0974-8237;year=2012;volume=3;issue=1;spage=26;epage=31;aulast=Muthukumar
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author Natarajan Muthukumar
author_facet Natarajan Muthukumar
author_sort Natarajan Muthukumar
collection DOAJ
description The pathogenesis of syringomyelia continues to be an enigma. The patency of the central canal and its role in the pathogenesis of communicating syringomyelia continues to elicit controversy. The case reported here provides an opportunity to retest some of the hypotheses of syringomyelia. A 33 year old female presented with sensory disturbances over the left upper extremity and trunk and was diagnosed to have panventriculomegaly with communicating syringomyelia. She was initially treated with ventriculoperitoneal shunting. As there was no change in her neurological status following shunt, this was followed by foramen magnum decompression with excision of an arachnoid veil covering the fourth ventricular outlet. She had clinical and radiological improvement after foramen magnum decompression. Five months later she had reappearance of the symptoms of syringomyelia and was found to have shunt dysfunction and holocord syrinx. She improved following shunt revision. This case is being reported to highlight the following points: 1. In patients with communicating syringomyelia and hydrocephalus, shunt dysfunction can present with symptoms of syringomyelia without the classical clinical features of shunt dysfunction, 2. In patients with communicating syringomyelia, the central canal of the spinal cord acts as an "exhaust valve" for the ventricular system, and, 3. studies about the patency of the central canal are reviewed in the context of this case and the role of the central canal in the pathogenesis of communicating syringomyelia is reviewed.
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spelling doaj.art-66be774db0124cf0a3009f61c9dc62ba2022-12-22T00:37:19ZengWolters Kluwer Medknow PublicationsJournal of Craniovertebral Junction and Spine0974-82372012-01-0131263110.4103/0974-8237.110125Syringomyelia as a presenting feature of shunt dysfunction: Implications for the pathogenesis of syringomyeliaNatarajan MuthukumarThe pathogenesis of syringomyelia continues to be an enigma. The patency of the central canal and its role in the pathogenesis of communicating syringomyelia continues to elicit controversy. The case reported here provides an opportunity to retest some of the hypotheses of syringomyelia. A 33 year old female presented with sensory disturbances over the left upper extremity and trunk and was diagnosed to have panventriculomegaly with communicating syringomyelia. She was initially treated with ventriculoperitoneal shunting. As there was no change in her neurological status following shunt, this was followed by foramen magnum decompression with excision of an arachnoid veil covering the fourth ventricular outlet. She had clinical and radiological improvement after foramen magnum decompression. Five months later she had reappearance of the symptoms of syringomyelia and was found to have shunt dysfunction and holocord syrinx. She improved following shunt revision. This case is being reported to highlight the following points: 1. In patients with communicating syringomyelia and hydrocephalus, shunt dysfunction can present with symptoms of syringomyelia without the classical clinical features of shunt dysfunction, 2. In patients with communicating syringomyelia, the central canal of the spinal cord acts as an "exhaust valve" for the ventricular system, and, 3. studies about the patency of the central canal are reviewed in the context of this case and the role of the central canal in the pathogenesis of communicating syringomyelia is reviewed.http://www.jcvjs.com/article.asp?issn=0974-8237;year=2012;volume=3;issue=1;spage=26;epage=31;aulast=MuthukumarShunt dysfunctionchiari malformationforamen magnum decompressionsyringomyeliacentral canal
spellingShingle Natarajan Muthukumar
Syringomyelia as a presenting feature of shunt dysfunction: Implications for the pathogenesis of syringomyelia
Journal of Craniovertebral Junction and Spine
Shunt dysfunction
chiari malformation
foramen magnum decompression
syringomyelia
central canal
title Syringomyelia as a presenting feature of shunt dysfunction: Implications for the pathogenesis of syringomyelia
title_full Syringomyelia as a presenting feature of shunt dysfunction: Implications for the pathogenesis of syringomyelia
title_fullStr Syringomyelia as a presenting feature of shunt dysfunction: Implications for the pathogenesis of syringomyelia
title_full_unstemmed Syringomyelia as a presenting feature of shunt dysfunction: Implications for the pathogenesis of syringomyelia
title_short Syringomyelia as a presenting feature of shunt dysfunction: Implications for the pathogenesis of syringomyelia
title_sort syringomyelia as a presenting feature of shunt dysfunction implications for the pathogenesis of syringomyelia
topic Shunt dysfunction
chiari malformation
foramen magnum decompression
syringomyelia
central canal
url http://www.jcvjs.com/article.asp?issn=0974-8237;year=2012;volume=3;issue=1;spage=26;epage=31;aulast=Muthukumar
work_keys_str_mv AT natarajanmuthukumar syringomyeliaasapresentingfeatureofshuntdysfunctionimplicationsforthepathogenesisofsyringomyelia