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Presyrinx in children with Chiari malformations

S Goh1, C L Bottrell, A H Aiken

  • 1School of Medicine, University of California San Francisco, San Francisco, CA 94143, USA. Suzanne.goh@ucsf.edu

Neurology
|June 21, 2008
PubMed

Insights

Presyrinx, a reversible spinal cord edema, is linked to Chiari malformations in children. Surgical decompression can restore cerebrospinal fluid (CSF) flow and resolve symptoms.

Area of Science:

  • Pediatric Neurology
  • Neuroradiology
  • Neurosurgery

Background:

  • Presyrinx is a rare, reversible spinal cord edema caused by abnormal cerebrospinal fluid (CSF) flow dynamics.
  • Previous reports of pediatric presyrinx are limited, with only three cases documented.
  • This study details the clinical and radiologic features of six pediatric patients diagnosed with presyrinx.

Purpose of the Study:

  • To describe the clinical and radiologic characteristics of presyrinx in a pediatric cohort.
  • To investigate the association between presyrinx and Chiari malformations.
  • To evaluate the efficacy of surgical decompression for presyrinx.

Main Methods:

  • Retrospective review of pediatric spine MRI reports from January 1995 to April 2007.
  • Identification of six patients with the radiologic diagnosis of presyrinx.
  • Analysis of neuroimaging, clinical symptoms, treatment, and outcomes.

Main Results:

  • Four patients had Chiari I malformation, and two had Chiari II malformation.
  • MRI findings included T2 prolongation, mild T1 prolongation, and cord enlargement without cavitation.
  • Cine phase-contrast MRI showed diminished or absent CSF flow at the foramen magnum in three patients.
  • Five patients underwent surgical decompression, leading to restored CSF flow and presyrinx resolution in three post-operative imaging studies.
  • Two patients with myelopathy symptoms experienced resolution after surgery.

Conclusions:

  • Chiari I and II malformations can cause pediatric presyrinx by obstructing CSF flow at the craniocervical junction.
  • Presyrinx should be considered in the differential diagnosis of pediatric myelopathy, especially in those with risk factors for abnormal CSF flow.
  • Surgical intervention can effectively resolve presyrinx and associated myelopathic symptoms.
Abstract

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