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[Syringomyelia in children]
1Service de Neurochirurgie Pédiatrique, Groupe Hospitalier Necker Enfants Malades, Paris.
Insights
Posterior fossa decompression effectively treats syringomyelia in children, improving or stabilizing 95% of cases. However, scoliosis associated with syringomyelia often shows limited improvement after this treatment.
Area of Science:
- Pediatric Neurosurgery
- Spinal Surgery
- Neurology
Background:
- Syringomyelia is a serious spinal cord condition affecting children.
- Chiari I malformation is a common cause of pediatric syringomyelia.
- Scoliosis frequently presents as an initial symptom in pediatric syringomyelia cases.
Purpose of the Study:
- To analyze treatment outcomes for pediatric syringomyelia.
- To evaluate the effectiveness of posterior fossa decompression for syringomyelia.
- To assess scoliosis management in children with syringomyelia.
Main Methods:
- Retrospective review of 442 pediatric syringomyelia cases (1985-1997).
- Analysis of patients with syringomyelia associated with Chiari I malformation.
- Evaluation of treatment via posterior fossa decompression and other interventions.
Main Results:
- 95% of children with syringomyelia showed clinical improvement or stabilization after posterior fossa decompression.
- Scoliosis associated with syringomyelia improved or remained stable in only 44% of cases.
- Various craniovertebral and spinal anomalies were identified as causes of syringomyelia.
Conclusions:
- Posterior fossa decompression is highly effective for pediatric syringomyelia.
- Scoliosis management requires specific attention, especially in isolated syringomyelia cases.
- Comprehensive evaluation and treatment are crucial for pediatric syringomyelia with associated anomalies.
Abstract:
From 1985 to 1997, 442 children were treated for syringomyelia. One hundred and eighty eight had syringomyelia in association with Chiari I malformation. In 65% of the case scoliosis was the initial symptom. All these children were treated by posterior fossa decompression, 95% were clinically improved or stabilized. The scoliosis remained unchanged or improved in only 44% of the cases. Many other causes of craniovertebral junction anomalies have been found (Chiari II, achondroplasia, mucopolysaccharidosis, craniosynostosis, Dandy-Walker malformation, posterior fossa cysts, birth injuries, or other causes of raised intracranial pressure). All these children were treated by posterior fossa decompression or when possible treatment of the cause. Features and results are presented. Spinal causes (diastematomyelias, lumbar lipomas, ventricule terminalis, spinal tumors ...) are presented. Whe discuss the treatment of scoliosis with specific attention to scoliosis associated to isolated syringomyelia.