Chiari I malformation associated with syringomyelia and scoliosis: a twenty-year review of surgical and nonsurgical treatment in a pediatric population

Abstract

Study design: Retrospective review of patients with Chiari I malformation with or without associated scoliosis.

Objectives: Determine the effect of decompression of Chiari I malformation with syringomyelia on stabilization or improvement of associated scoliosis.

Summary of background data: Chiari malformations are often associated with spinal deformities, including scoliosis. Studies have suggested a causal relation between syringomyelia and scoliosis.

Methods: Patients with Chiari I malformation and syringomyelia with or without scoliosis treated over the last 20 years were reviewed. Patients with any other anomalies were excluded. Scoliotic curves were classified by magnitude and curve type. All patients were treated with surgical decompression of the Chiari malformation with or without drainage of the syringomyelia.

Results: Twenty-five patients were identified, ranging in age from 19 months to 16.5 years. Nineteen patients (76%) had associated scoliosis. The majority of the patients with scoliosis (13 of 19) sought treatment for spinal deformity, and only 6 had for pain or neurologic symptoms. Eleven of 19 patients with scoliosis (58%) underwent fusion. Eight of 19 (42%) patients have not undergone fusion: 3 have experienced progress, 1 remains in a stable condition, and 4 have experienced improvement of curvature since undergoing decompression. The mean age of patients who experienced progress after decompression was 14.5 years, compared to 6 years for patients who experienced improvement.

Conclusion: Early decompression of Chiari I malformation with syringomyelia and scoliosis resulted in improvement or stabilization of the spinal deformity in 5 cases. Each of these patients underwent decompression before 8 years of age and before the curve was severe. However, this series represents a few patients demonstrating this trend, and further follow-up and investigation are warranted.