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Shunt malfunction causing acute neurological deterioration in 2 patients with previously asymptomatic Chiari malformation Type I

Authors :
Robert E. Elliott
David H. Harter
Howard L. Weiner
Donato Pacione
Jeffrey H. Wisoff
Stephen P. Kalhorn
Source :
Journal of Neurosurgery: Pediatrics. 4:170-175
Publication Year :
2009
Publisher :
Journal of Neurosurgery Publishing Group (JNSPG), 2009.

Abstract

Patients with symptomatic Chiari malformation Type I (CM-I) typically exhibit a chronic, slowly progressive disease course with evolution of symptoms. However, some authors have reported acute neurological deterioration in the setting of CM-I and acquired Chiari malformations. Although brainstem dysfunction has been documented in patients with CM-II and hydrocephalus or shunt malfunction, to the authors' knowledge only 1 report describing ventriculoperitoneal (VP) shunt malfunction causing neurological deterioration in a patient with CM-I exists. The authors report on their experience with the treatment of previously asymptomatic CM-I in 2 children who experienced quite different manifestations of acute neurological deterioration secondary to VP shunt malfunction. Presumably, VP shunt malfunction created a positive rostral pressure gradient across a stenotic foramen magnum, resulting in tetraparesis from foramen magnum syndrome in 1 patient and acute ataxia and cranial nerve deficits from syringobulbia in the other. Although urgent shunt revisions yielded partial recovery of neurological function in both patients, marked improvement occurred only after posterior fossa decompression.

Details

ISSN :
19330715 and 19330707
Volume :
4
Database :
OpenAIRE
Journal :
Journal of Neurosurgery: Pediatrics
Accession number :
edsair.doi...........8b8ed7068d7f8b5dd62fcd56bb53d780