Pediatric Chiari malformation Type 0: a 12-year institutional experience
Joshua J Chern1, Amber J Gordon, Martin M Mortazavi
1Pediatric Neurosurgery, Children's Hospital, Birmingham, Alabama 35233, USA.
Journal of Neurosurgery. Pediatrics
|July 5, 2011
Summary
Chiari 0 (C0) malformations, characterized by syringomyelia without tonsillar ectopia, show significant symptom improvement after posterior fossa decompression. Careful diagnosis is crucial for this rare subgroup.
Area of Science:
- Neurosurgery
- Pediatric Neurology
- Cerebrospinal Fluid Dynamics
Background:
- Syringomyelia can occur without classic Chiari malformation Type I (CM-I).
- Previous work identified "Chiari 0" (CM-0) cases with syringomyelia and normal CM-I imaging.
- Abnormal cerebrospinal fluid (CSF) flow at the foramen magnum was implicated in CM-0 etiology.
Purpose of the Study:
- To report on the 12-year follow-up of pediatric patients diagnosed with CM-0.
- To evaluate the efficacy of posterior fossa decompression in this specific patient subgroup.
Main Methods:
- A retrospective analysis of 12 years of surgical cases.
- Identification of patients with syringomyelia and no evidence of CM-I.
- Surgical treatment involved posterior fossa decompression and duraplasty.
Main Results:
- Fifteen patients (3.7%) were identified with CM-0.
- Physical barriers to CSF flow were observed at the foramen magnum in many patients.
- Significant reduction in syringomyelia was noted postoperatively in most patients.
Conclusions:
- CM-0 represents a small but distinct subgroup within Chiari malformations.
- Accurate patient selection and exclusion of other syrinx etiologies are critical for successful treatment.
- Posterior fossa decompression can ameliorate the clinical course of carefully selected CM-0 patients.
