Zérah M
Service de Neurochirurgie Pédiatrique, Groupe Hospitalier Necker Enfants Malades, Paris.
Neurochirurgie. 1999 Jun;45 Suppl 1:37-57.
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.
1985年至1997年期间，442名儿童接受了脊髓空洞症治疗。其中188名患有脊髓空洞症合并Chiari I畸形。65%的病例中，脊柱侧弯是首发症状。所有这些儿童均接受了后颅窝减压治疗，95%的患儿临床症状改善或病情稳定。仅44%的病例中脊柱侧弯保持不变或有所改善。还发现了许多其他导致颅颈交界区异常的原因（Chiari II畸形、软骨发育不全、黏多糖贮积症、颅缝早闭、Dandy-Walker畸形、后颅窝囊肿、产伤或其他导致颅内压升高的原因）。所有这些儿童均接受了后颅窝减压治疗，或尽可能针对病因进行治疗。现将其特征及结果予以介绍。文中还介绍了脊柱病因（脊髓纵裂、腰部脂肪瘤、终室、脊柱肿瘤……）。我们讨论了脊柱侧弯的治疗，尤其关注与孤立性脊髓空洞症相关的脊柱侧弯。
