Arnod-Chiari畸形合并颅底成角畸形的外科治疗

目的探讨Arnod-Chiari畸形合并无寰枢椎脱位的颅底成角畸形的治疗方法。方法30例Arnod-Chiari畸形患者合并颅底成角畸形但无完全寰枢椎脱位,MR显示脑干与颈脊髓腹侧明显受压,测量其颅底角小于120°,行枕下减压手术治疗同时纠正颅底角角度后内固定。结果30例患者术后半年复查MR均有人工枕大池的形成,脊髓空洞均缩小,脑干与颈脊髓之间夹角较术前明显增大,植骨已融合,颅底角大于130°。术后患者症状均得到明显改善。结论对Arnod-Chiari畸形合并颅底成角畸形患者进行枕下减压手术同时纠正颅底角角度并固定,可以解决脑干与颈脊髓腹侧受压问题,能达到缓解临床症状或阻止临床症状加重的目的。

Surgical treatment for Arnod-Chiari malformation combined with angulated base of skull

Objective To investigate the method of treatment for Arnod-Chiari malformation combined with deformed angulated base of skull and without occipitocervical dislocation.Methods Thirty patients with Arnod-Chiari malformation combined with deformed base of skull without complete occipitocervical dislocation were reviewed,magnetic resonance imaging showed brain stem and cervical spinal cord are compressed in these patients with the angle between brain stem and cervical spinal cord less than 120 degree.The infra-occipital decompression and internal fixation after rectification of cranial base angle were managed for these patients.Results Preoperative symptoms were improved in all patients afer 6 months,and magnetic resonance imaging showed artificial cisterna magna was enlarged,hollow of spinal cord was diminished,angle between brain stem and cervical spinal cord was more than 130 degree,and bone graft was fused in every patient.Conclusions The infra-occipital decompression and internal fixation are suitable methods for these patients suffered from Arnod-Chiari malformation combined with angulated malformation base of skull,which solve the compression for brain stem and cervical spinal cord and retrieved physiological function in region of occipital-neck.The management can improve the clinical symptoms or prevent aggravating of these symptoms in these patients.