颅颈交界区畸形的个体化手术治疗

Individual surgical treatment of craniocervical junction malformation

目的 探讨颅颈交界区畸形各种不同表现类型的个体化手术治疗方案.方法 对象为2011年1月至2013年12月河南省人民医院神经外科收治颅颈交界区畸形患者,共112例,其中小脑扁桃体下疝畸形65例（合并脊髓空洞58例,不合并脊髓空洞7例）,颅底陷入症（合并小脑扁桃体下疝畸形或合并寰枕融合）35例,复杂颅颈部畸形（寰枢椎脱位并寰枕融合或并小脑扁桃体下疝畸形或并颈椎分节不全）共22例.所有患者均有上颈髓神经受损症状.小脑扁桃体下疝畸形患者术前评价如寰枕关节稳定则行枕部小骨窗减压术,切除部分小脑扁桃体,松解后颅凹、上颈髓粘连,人工硬膜适当扩大修补缝合枕颈硬膜.颅底陷入症患者若神经受压表现在后方时,则取后路减压手术治疗.复杂颅颈部畸形伴寰枢椎脱位患者则行麻醉状态下的头颈牵引复柆,或前路松解后牵引复位,枕颈固定并植骨融合.结果 术后对所有患者进行了随访,症状改善明显者98例（87.51％）.症状无变化者10例（8.93％）.术后神经功能恶化者3例（2.67％）.死亡1例（0.89％）.结论 根据颅颈交界区畸形患者的具体临床表现,术前进行详细评估,采取不同的个体化手术方案,是治疗此类疾病的最佳方法.

Objective To explore the individual surgical treatment of various performance types of craniocervical junction malformation.Methods From January 2011 to December 2013,112 patients with craniocervical junction malformations were treated at our department,including Chiari malformation （n =65） （syringomyelia,n =58 and without syringomyelia,n =7）,basilar invagination disease （n =35） （with cerebellar tonsillar herniation malformation or occipitocervical fusion） and complex craniocervical malformation （n =22） （atlantoaxial dislocation with occipitocervical fusion or with chiari malformation or cervical insufficiency sub-section）.All of them had the symptoms of upper cervical nerve damage.For those with Chiari malformation,we evaluated atlanto-occipital joint stability preoperatively.If atlanto-occipital joint was stable,we performed small occipital bone window decompression,partial removal of cerebellar tonsils,loosening of posterior fossa,upper cervical adhesions,artificial dura appropriate sutured dural repair expanding neck pillow.For patients with basilar invagination,if nerve compression performance was in the rear,posterior decompression was performed.For those with complex craniocervical malformation with atlantoaxial dislocation,neck traction under anesthesia or traction after anterior release,then pillow neck fixation and fusion were performed.Results During follow-ups,the symptoms improved significantly （n =98,87.51％）.There were no symptomatic change （n =10,8.93％）,postoperative neurological deterioration （n=3,2.67％） and death （n =1,0.89％）.Conclusion According to specific clinical manifestations of craniocervical junction malformation patients,the best treatment is to perform individualized surgeries after thorough preoperative evaluations.