摘要目的 根据寰枕畸形伴发脊髓型颈椎病患者的不同临床特征、影像学特点采取不同手术方式组合对颈脊髓进行减压,并评价探讨其疗效和预后.方法 北京大学第三医院自2002年1月至2007年7月共收治22例寰枕畸形伴发脊髓型颈椎病患者,在了解患者首发症状及病程演变情况后,以日本矫形外科学会(JOA)评分系统对手术前后体征进行分析比较,常规行X片、CT、MRI等影像学检查.一期以不同术式组合(枕颈减压、枕大池成形3例,枕颁减压、枕大池成形和空洞穿刺引流1例,枕颈减压、单开门椎管扩大成形5例,枕颈减压、局限性单侧椎板切除减压1例,枕颈减压、钛板内固定1例,枕颈减压+颈前路间盘摘除、植骨、钛板内固定1例,枕颈减压、枕大池成形+单开门椎管扩大成形3例,枕颈减压、枕大池成形+局限性单侧椎板切除减压4例,枕颈减压、枕大池成形和空洞穿刺引流+局限性单侧椎板切除减压3例)对两种病变造成的颈脊髓等神经系统压迫进行允分减压.术后全部患者均随访调查,并以改善、稳定、进展时患者术后中期状况进行评价.结果 术后效果优6例(手术前后JOA分值差≥2),好13例(JOA分值差=1),一般3例(JOA分值差为0).随访2～48月,进一步改善20例,稳定2例.结论 根据不同临床表现、体征和影像学特点,采取不同术式组合一期手术治疗寰枕畸形伴发脊髓型颈椎病可获良好的治疗效果.

abstractsObjective To present a clinical and radiological evaluation of 22 consecutive patients with two kinds of cranio-cervical junction malformation who were managed in a one-stage operation with different approaches and methods. Methods Admitted into the Third Affiliated Hospital of Peking University from January 2002 to July 2007, 22 consecutive patients with two kinds of cranio-cervical junction disorders (congenital atlanto-occipital malformation accompanied by acquired cervical spondylotic myelopathy) underwent clinical, radiological and MRI evaluations. Clinical assessment consisted of a detailed history and neurological assessment and JOA grading. According to their clinical symptoms and imaging findings, the patients were treated with different operative approaches and methods (posterior occipitocervical decompression and/or cistema magna reconstruction for atlanto-occipital malformation, and anterior cervical microdiscectomy with titanium intervertebralcage fusion or expansive open-door laminoplasty for cervical spondylotic myelopathy, and cavitary drainage for syringomyelia). All of the patients were observed by follow-up to evaluate their post-operative conditions. Results Of the 22 cases, the operative outcome was graded as excellent(difference between pre- and post-operative JOA scores was more than 2) in 6 cases, as good (the difference was 1) in 13, as unchanged (the difference was 0) in 3. In the follow-up of 2-48 months, 20 cases get further better, and 2 cases became stable. Conclusion The patients with atlanto-occipitalmalformation and cervical spondylotic myelopathy can be treated by one-stage operation with different approaches and methods according to their clinical manifestations and signs and imaging features. The treatment can reach excellent clinical and radiological outcomes and low morbidity.