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目的:探讨改良寰枕减压术联合枕颈内固定术治疗Chiari畸形Ⅰ型合并脊髓空洞症及寰枢椎脱位的临床疗效。方法:四川省德阳市人民医院神经外科自2011年1月至2019年10月采用改良寰枕减压术联合枕颈内固定术治疗Chiari Ⅰ畸形合并脊髓空洞症及寰枢椎脱位患者25例，现回顾性分析患者的临床资料，比较患者手术前后脊髓背侧脑脊液流速峰值、电生理监测结果、寰齿间距(ADI)、脊髓空洞直径和芝加哥Chiari畸形预后量表(CCOS)评分的变化。结果:患者术后脊髓背侧脑脊液流速峰值[(3.25±0.47) cm/s]高于术前脊髓背侧脑脊液流速峰值[(2.13±0.19) cm/s]，差异有统计学意义(n P<0.05)。与术前比较，术后6个月患者的ADI值降低，脊髓空洞直径降低，CCOS评分增加，电生理监测异常者所占比例降低，差异均有统计学意义(n P<0.05)。术后无新发神经功能损害、感染、脑脊液漏、瘫痪、呼吸衰竭及死亡患者。n 结论:应用改良寰枕减压术联合枕颈内固定术治疗Chiari畸形Ⅰ型合并脊髓空洞症及寰枢椎脱位，能有效地起到寰枕区减压及寰枢椎解剖复位的作用，脊髓空洞缓解率高、症状体征改善明显、术后并发症少。“,”Objective:To investigate the clinical effect of modified atlantooccipital decompression combined with occipitocervical internal fixation on Chiari type I malformation combined with syringomyelia and atlantoaxial dislocation.Methods:Twenty-five patients with Chiari I malformation combined with syringomyelia and atlantoaxial dislocation accepted by modified atlantooccipital decompression combined with occipitocervical internal fixation in our hospital from January 2011 to March 2019. The clinical data of these patients were retrospectively analyzed. The changes of peak velocity of cerebrospinal fluid in the dorsal part of the spinal cord, electrophysiological results, atlantodental interval (ADI) values, sizes of syringomyelia, and Chicago Chiari outcome scale (CCOS) scores before and after operation were compared.Results:The peak velocity of cerebrospinal fluid in the dorsal spinal cord after surgery ([3.25±0.47] cm/s) was statistically higher in these patients than that before surgery ([2.13±0.19] cm/s,n P<0.05). As compared with the preoperative results, ADI values, sizes of syringomyelia, and proportion of patients with abnormal electrophysiological monitoring at 6 months after surgery were significantly decreased, and CCOS scores at 6 months after surgery were significantly increased (n P<0.05). There were no new nerve function damage, infection, cerebrospinal fluid leakage, paralysis, respiratory failure or death.n Conclusions:Modified atlantooccipital decompression combined with occipitocervical internal fixation plays effective role in atlantooccipital decompression and atlantoaxial anatomical reduction in patients with Chiari malformation type I combined with syringomyelia and atlantoaxial dislocation. The remission rate of syringomyelia is high, the symptoms and signs improve obviously, and the postoperative complications are less.