分期前后方减压治疗合并颅底陷入的Chiari畸形

Treatment of Chiari malformation combined with basilar invagination by staged anterior and posterior decompression

目的合并颅底陷入的Chiari畸形患者常有前方脑干压迫，多数经前路减压即可使扁桃体上移，空洞缩小，但少数患者前路减压后Chiari畸形和脊髓空洞并不减轻，本文旨在探讨采用分期前后方减压治疗该类患者。方法9例患者经口咽入路齿状突磨除，观察1—2个月后发现Chiari畸形和脊髓空洞未减轻，遂行后方枕大孔减压，小脑扁桃体切除，硬膜扩大修补，枕颈或C。固定融合术。随访24—60个月（平均32个月）。结果本组无死亡或严重并发症患者，所有患者症状改善，8例Chiari畸形和脊髓空洞改善，1例无变化。结论合并颅底陷入的Chiari畸形患者应首先行经口咽入路齿状突磨除术，多数患者后方仅需固定即可，但有些患者颅底陷入症状缓解，而Chiari畸形依旧，需后方减压加固定手术。

Objective There is usually anterior compression of the brain stem in Chiari malformation patients combined with basilar invagination. After anterior decompression, many patients will present elevation of the cerebellar tonsil and resolution of syringomyelia. However, still in some patients, the Chiari malformation and syringomyelia remain unchanged after anterior decompression. This article is to discuss a staged anterior and posterior decompression strategy for the treatment of these patients. Methods 9 cases of Chiari malformation combined with basilar invagination were operated by transoral transpharyngeal odontoidectomy first, observed for 1 -2 months, the Chiari malformation and syringomyelia did not relieve, then posterior foramen magnum decompression, tonsillectomy and expansion of posterior fossa dura combined with occipito - cervical fusion or C1_2 fusion were performed. The patients were followed up for 24 - 60 months （ mean 32 months）. Results There was no mortality or severe complication. The clinical symptoms were improved in all patients. The Chiari malformation and syringomyelia were relieved in 8 patients, while unchange in 1 case. Conclusions For Chiari malformation patients combine with basilar invagination, anterior decompression with odontoidectomy should be performed first. In many patients, increase of posterior fossa volume, improved CSF dynamic may be achieved, posterior fusion will be enough. While in some other patients, only symptoms of basilar invagination may be improved after anterior decompression. So posterior decompression is still important.