摘要目的 探讨枕颈内固定并植骨融合治疗先天性复杂寰枕畸形的疗效.方法 回顾性分析21例复杂寰枕畸形患者,其中17例行后颅窝减压+枕颈植骨固定融合术,4例行经口咽寰枢椎间软组织及骨性组织切除减压,齿状突复位减压+后路后颅窝枕骨大孔减压术+枕颈植骨固定融合术；术后随访3个月至3年.结果 寰枢脱位并存在延颈髓腹、背侧受压者,选择经口咽寰枢椎间软组织及骨性组织切除减压齿状突复位减压+后路后颅窝枕骨大孔减压术+枕颈植骨固定融合术,疗效满意.硬脑膜扩大修补与否及小脑扁桃体切除术与否与最终远期疗效无关；伴有多节段脊髓空洞症者行脊髓空洞“T”管蛛网膜下腔引流.本组治愈14例,有效7例.结论 针对Chiari畸形解剖变异大的特点以及合并症形成的机制,选择不同的手术方案,提高手术治疗的远期疗效.

abstractsObjective To investigate the effects of occipitocervical fixation and bone grafting fusion for the treatment of complicated congenital occipitocervical malformations.Methods A retrospective analysis of 21 cases of complicated occipitocervical malformation patients was performed.Seventeen cases underwent posterior cranial decompression bone grafting and fixation of occipital cervical fusion.Four cases underwent soft tissue and bony tissue resection,decompression of odontoid process reset decompression,posterior foramen magnum decompression,bone grafting and fixation of occipital cervical fusion.All patients were followed up by 3 months to 3 years.Results For the patient with atlantoaxial dislocation and the presence of intra-axial ventral,dorsal compression,good effects could be achieved through soft tissue and bony tissue resection,posterior foramen magnum decompression,bone grafting and fixation of occipital cervical fusion.There was no relationship between dural matter repair,cerebellum tonsil resection and final effects.For the patient with segmental syringomyelia,spinal cord subarachnoid drainage should be done.Conclusions Different operation scheme should be considered according to the anatomic variation and formation mechanism of complicated occipitocervical malformations to improve the long-term surgical effects.