摘要： 目的  总结合并寰枢椎脱位的复杂颅颈交界区畸形经后路减压复位内固定术的临床经验。方法  回顾分析18 例合并寰枢椎脱位的复杂颅颈交界区畸形患者（先天性寰枢椎脱位15 例、经口腔入路齿状突磨除术后症状加重致枕颈失稳1 例、外伤所致2 例）的临床资料。施行经后路减压复位钉棒内固定术，术中行体感诱发电位及肌电图监测，根据日本骨科协会（JOA）17 分评分系统和影像学改善程度评价手术疗效。结果  术后影像学检查显示，18 例中16 例钉棒内固定系统和寰枢椎复位良好，1 例复位不良；骨性融合良好16 例，欠佳1 例。术后临床表现均不同程度好转，1 例突发呼吸骤停死亡。术后平均随访6.62 个月（3 ~ 28 个月），JOA 平均评分为11.62 ± 3.23，与手术前评分（7.51 ± 3.82）相比，差异具有统计学意义（t = -5.476，P = 0.004）。结论  经后路减压、复位、钉棒内固定术治疗合并寰枢椎脱位的颅颈交界区畸形临床疗效良好，能够减少患者痛苦、避免再次手术，值得临床推广应用。

关键词: 脱位, 内固定术（非MeSH 词）, 寰枢关节, 寰枕关节, 诱发电位, 躯体感觉, 肌电描记术

Abstract: Objective To explore the clinical effect of posterior reduction and screw-rod (plate) internal fixation technique for the treatment of atlantoaxial dislocation in complex craniovertebral junction region malformation. Methods Clinical data of 18 patients suffered from atlantoaxial dislocation with complex craniovertebral junction region malformation were analysed retrospectively, including 15 cases of congenital atlantoaxial dislocation, 1 case with aggravated symptoms of cervico-occipital unstability after anterior odontoid process grinding, 2 cases with trauma-induced dislocation. All patients underwent posterior decompressive reduction and screw-rod internal fixation. During surgical procedure, senory evoked potential (SEP) and electromyography (EMG) monitorings were used. Japanese Orthopaedic Association (JOA) score and imaging were used to evaluate the surgical effect. Results Postoperative imaging examination showed that fixed system and atlantoaxial reduction were good in 16 cases and bad in 1 cases. The bony fusion was good in 16 cases and bad in 1 case. The patients' clinical symptoms were improved to different degree, but one patient suddenly occurred respiratory arrest and died. Patients were followed up for 3 to 28 months (mean 6.62 months). After operation the average JOA score was 11.62 ± 3.23, while before operation it was 7.51 ± 3.82. The difference was significant (t = -5.476, P = 0.004). Conclusion Posterior decompressed reduction and screw-rod (plate) internal fixation for atlantoaxial dislocation with complex craniovertebral junction region malformation is save and effective. It will be widely used in the future.

Key words: Dislocations, Internal fixation (not in MeSH), Atlanto-axial joint, Atlanto-occipital joint, Evoked potential, somatosensory, Electromyography