脊柱侧弯并发Chiari畸形的手术治疗
作者：李展春，刘祖德，李占玉。
摘 要：［目的］探讨脊柱侧弯并发Chiari畸形的诊断和治疗方法。［方法］本组12例脊柱侧弯并发Chiari畸形的患者，其中有7例同时伴有脊髓空洞症，所有患者均先由神经外科行枕大孔扩大成形术或空洞分流术，二期行脊柱侧弯后路矫形术。［结果］Cobbs角术前平均71°，术后平均24°，矫正率为66%。脊柱矫形术后随访1~4 a，平均24 a，没有发生脱钩、断钉及假关节现象；没有出现新的神经损害表现；感觉障碍及腹壁浅反射减弱的症状没有加重。［结论］对于脊柱侧弯并发Chiari畸形、脊髓空洞症的患者，术前应完善检查、明确诊断；先进行枕大孔扩大成形术或空洞分流术，二期行脊柱侧弯后路矫形术，可以减少脊柱侧弯矫形时引起的神经损害并发症。
关键词：脊柱侧弯； Chiari畸形； 脊髓空洞症； 手术治疗。
Abstract：［Objective］To discuss the diagnosis and treatment method of scoliosis associated with Chiari malformation［Method］Twelve patients were suffered from scoliosis associated with Chiari malformation,in which there were 7 patients also complicated with syringomyeliaFirstly,posterior suboccipital craniectomy to enlarge foramen occipital magnum was performed,then the patients underwent the scoliosis correction with instrumentation［Result］Preoperatively,the mean Cobbs angle on the coronal plane was 71°,and 24° postoperatively,with an average correction of 66%During a followup from 1 year to 4 years,there were no release of the hook,no breaking of pedicle screw,no false articulation and no new nervous lesion,while few improving was found about the sensory disability and superficial abdominal hyporeflexia［Conclusion］We should first consummate the examination and make a definite diagnosis for the patients who were suffered from scoliosis associated with Chiari malformation and syringomyelia;posterior suboccipital craniectomy to enlarge foramen occipital magnum or syrinxsubarachnoid space shunting should be performed firstly to reduce nervous lesion during scoliosis correction surgery。
Key words：Scoliosis; Chiari malformation; Syringomyelia; Surgical treatment。
青少年脊柱侧弯大部分为特发性脊柱侧弯，而非特发性脊柱侧弯中并发Chiari畸形的病例则较为少见。Chiari畸形是胚胎期后脑先天性发育不良，在解剖上为小脑扁桃体等结构疝入上颈椎管内。临床上脊柱侧弯并发Chiari畸形的神经损害往往表现不明显，极易漏诊，或被脊柱侧弯的外观畸形所掩盖。X线片上无脊椎的结构性改变易被误诊为特发性脊柱侧弯，以致患者在接受脊柱侧弯矫形术时所承担神经系统并发症的风险远大于特发性脊柱侧弯〔1〕，所以，其诊断和治疗方法具有一定特殊性。本科于1999年1月~2003年12月，共收治脊柱侧弯并发Chiari畸形的患者12例，作者对这些患者进行了手术治疗，现报道如下。
1 临床资料。
11 一般资料。
本组12例，男7例，女5例；年龄7～17岁，平均13岁；病程2～8 a。本组患者均以脊柱侧弯为首诊原因，体检发现腹壁反射减弱，消失或不对称6例，浅感觉减退3例，肌力减退1例，肢体发育不对称（两侧肢体不等长）和病理反射阳性各1例。体检发现异常后，MRI检查确认为Chiari畸形，其中7例同时伴有脊髓空洞存在。脊柱侧弯部位为左胸弯9例，胸腰双弯2例，胸腰弯1例，其中8例为后凸型胸弯。Cobbs角38°~94°，平均71°；胸椎后凸23°~59°，平均47°。
12 手术方法。
本组患者均先由神经外科行枕大孔扩大成形术和空洞分流术，3～9个月后进行脊柱侧弯后路矫形术。5例并发Chiari畸形的患者行枕大孔扩大成形术，7例同时并发Chiari畸形和脊髓空洞症的患者中有4例空洞和脊髓的比例小于50％行枕大孔扩大成形术，另3例空洞和脊髓的比例大于50％行空洞分流术。脊柱侧弯后路矫形术使用CD Horizon M8 9例，TRSH 3例。手术均在体感诱发电位（SEP）全程监测下操作。