先天性小脑扁桃体下疝畸形合并脊髓空洞症的手术治疗(附14例报告)

Operative treatment for congenital tonsillar hernia deformity associated with syringomyelia:a report of 14 cases

目的 了解先天性小脑扁桃体下疝畸形合并脊髓空洞症的手术治疗效果。方法 对 14例先天性小脑扁桃体下疝畸形合并脊髓空洞症患者 ,根据不同的病情采用相应的手术方式进行治疗。其中 9例单纯行后颅窝和上颈椎椎板切除减压、枕大池成形术 ;5例行后颅窝减压、枕大池成形加脊髓空洞分流术。结果 优 11例 ,良 3例 ,有效率为 10 0 %。 14例术后随访 ,复查MRI示脊髓空洞较术前明显缩小 12例 ,空洞无明显改变 2例。结论 先天性小脑扁桃体下疝合并脊髓空洞有临床症状者 ,根据不同的病例情况采用相应的手术方式。

Objective To obtain the efficacy of surgery treatment for congenital tonsillar hernia deformity associated with syringomyelia. Methods 14 patients with congenital tonsillar hernia deformity associated with syringomyelia had been treated with correspondent surgery depending on their case condition. Of which 9 cases were given single depression of posterior cranial fossa and superior cervical vertebral lamina excision, and the plastic operation of great occipital foremen; but the rest of the 5 were given the depression of posterior cranial fossia, plastic operation of great occipital foremen and cavity shunt of spinal cord. Results The efficacy in 11 cases was excellent, and in 3 was best, the effective rate was 100%. The follow-up of over half a year by MRI examination found that the cavity of spinal cord shrunk obviously in 12 cases, but it did not change in 2 cases. Conclusions Patients with clinical signs of congenital tonsillar hernia deformity associated with syringomyelia should be given correspondent operative treatment depending on different case condition.