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Pediatric syringomyelia with chiari malformation: its clinical characteristics and surgical outcomes
K Hida1, Y Iwasaki, I Koyanagi
1Department of Neurosurgery, University of Hokkaido, School of Medicine, Sapporo, Japan.
Insights
Pediatric syringomyelia, often linked to Chiari Type I malformation, presents unique symptoms like scoliosis. Surgical interventions, including foramen magnum decompression, effectively treat syringomyelia and improve associated scoliosis in children.
Area of Science:
- Pediatric Neurology
- Neurosurgery
- Spinal Surgery
Background:
- Pediatric syringomyelia differs clinically from adult forms, with spinal dysraphism presenting distinct characteristics.
- This study focuses on pediatric syringomyelia associated with Chiari Type I malformation, excluding spinal dysraphism.
- The research aims to define clinical features and assess surgical outcomes for pediatric syringomyelia, particularly regarding scoliosis.
Observation:
- The study analyzed 16 pediatric patients (ages 3-15) with syringomyelia and Chiari Type I malformation.
- Commonly observed symptoms included nystagmus, motor weakness, sensory disturbances, and a high incidence of scoliosis (13/16 patients).
- No patients in this cohort had spinal dysraphism.
Findings:
- Surgical interventions, foramen magnum decompression (FMD) and syringo-subarachnoid (S-S) shunting, were performed.
- Post-surgery, motor function improved in 7/8 patients, and sensory disturbances improved in 9/10.
- Significant reduction in syrinx size was noted on MRI; scoliosis showed improvement in 5, stabilization in 5, and deterioration in 3 patients.
Implications:
- Pediatric syringomyelia exhibits a lower incidence of pain and sensory issues but a higher prevalence of scoliosis compared to adult cases.
- Surgical treatment is effective in improving or stabilizing scoliosis in pediatric patients with syringomyelia.
- These findings highlight the importance of surgical management for pediatric syringomyelia and its associated spinal deformities.
Background:
Most reports regarding pediatric syringomyelia have focused not only on Chiari malformation, but also on spinal dysraphism. However, the clinical characteristics of syringomyelia with spinal dysraphism are quite different from those of syringomyelia due to Chiari Type I malformation. The objectives of this study were to identify clinical characteristics of pediatric syringomyelia and to determine whether surgery prevents or corrects the scoliosis associated with syringomyelia.
Methods:
We reviewed the records of 16 pediatric patients with syringomyelia and Chiari Type I malformation. The patients' ages ranged from 3 to 15 years, with mean age of 9.8 years. None of the patients had spinal dysraphism.
Results:
Nystagmus was observed in 2 of the 16 patients, motor weakness in 8 patients, sensory disturbance in 10 patients, and scoliosis in 13 patients. As the initial surgical procedure, foramen magnum decompression (FMD) was performed in seven patients and syringo-subarachnoid (S-S) shunting in nine patients. The motor function improved in 7 of the 8 affected patients, and the sensory disturbance improved in 9 of the 10 affected patients. The magnetic resonance images obtained after the surgery revealed marked decrease of the syrinx size in all patients. Of the 13 patients with scoliosis, 5 showed improvement, 5 stabilization, and 3 deterioration.
Conclusions:
Compared with adolescent and adult syringomyelia, pediatric syringomyelia shows a much lower incidence of sensory disturbance and pain, but quite a high incidence of scoliosis. Surgery is effective in improving or stabilizing scoliosis in these patients.