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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
Summary
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.
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