重度僵硬性脊柱侧后凸合并Chiari畸形的截骨手术治疗及疗效观察

Efficacy of osteotomy in the treatment of severe rigid kyphoscoliosis associated with Chiari malformation

背景：重度僵硬性脊柱侧后凸畸形合并Chiari畸形（Chiari malformation, CM）的手术治疗难度大，风险较高，目前国内相关临床研究较少。目的：探讨重度僵硬性脊柱侧后凸畸形合并CM的后路全脊椎截骨术（posterior vertebral col-umn resection, PVCR）治疗及近期临床疗效。方法：回顾性分析2011年2月至2015年2月收治的17例重度僵硬性脊柱侧后凸畸形合并CM患者的临床资料，其中男7例，女10例；年龄14-27岁，平均（19.7±7.3）岁。其中11例患者一期后颅窝减压术（posterior fossa decompression, PFD）术前存在神经损害症状。术前冠状位主弯Cobb角88°-135°，平均109.8°±21.6°；矢状位后凸Cobb角80°-128°，平均92.7°±11.9°。所有患者均已完成一期PFD，准备二期行PVCR，记录患者术中出血量、手术时间、术后2周及末次随访时的影像学改变。结果：二期PVCR手术时间为286-570 min，平均（369.5±102.1） min；出血量为1700-4300 ml，平均（2195.8±1092.4）ml。二期PVCR后全部获得随访，随访时间为8-39个月，平均（14.7±3.5）个月。随访期间无断钉断棒发生，术后未出现新的神经损害并发症。术后2周冠状位主弯Cobb角25°-70°，平均46.2°±10.7°，矫正率为（54.3±11.5）%；术后2周矢状面后凸Cobb角为38°-65°，平均45.7°±12.4°，矫正率为（52.6±12.8）%，与术前相比较均有统计学差异（P＜0.05）。末次随访冠状位Cobb角为30°-82°，平均48.7°±11.3°，丢失率为（3.8±2.6）%；矢状位Cobb角为40°-75°，平均47.9°±13.2°，丢失率为（5.7±3.4）%，与术后2周相比较均无统计学差异（P＞0.05）。结论：对于重度僵硬性脊柱侧后凸合并CM患者，采用一期PFD、二期PVCR可获得满意的矫形效果，具有较高的手术安全性。

Background：It is difficult and high-risk of surgical treatment for severe rigid kyphoscoliosis associated with Chi-ari malformation （CM）, and there are few domestic clinical researches on it. Objective：To research the therapeutic regime of posterior vertebral column resection in severe rigid kyphoscoliosis combined with CM and its short-term clinical efficacy. Methods：Seventeen patients with severe and rigid kyphoscoliosis associated with CM treated from February 2011 to Febru-ary 2015 were enrolled in this retrospective study. There were 7 males and 10 females with an average age of （19.7 ± 7.3） years （range, 14-27 years）. Neurologic deficit syndrome existed in 11 patients before operation. The mean preoperative ma-jor coronal Cobb angle was 109.8° ± 21.6° （range, 88°-135° ）. The mean kyphosis Cobb angle was 92.7° ± 11.9° （range, 80°-128°）. Posterior vertebral column resection （PVCR） was prepared after posterior fossa decompression （PFD） in all patients. Intraoperative blood loss, operation time and imaging changes （preoperatively, 2 weeks postoperatively and at the last fol-low-up） were recorded. Results：The average operation time of PVCR was （369.5±102.1） min （range, 286-570 min）. The av-erage blood loss was （2195.8 ± 1092.4） ml （range, 1700-4300 ml）. The averageperiod of follow-up was （14.7 ± 3.5） months （range, 8-39 months） in all the patients. There was no instrument failure or new neurologic deficits at the final follow-up. Two weeks after PVCR, the major coronal Cobb angle was 46.2°±10.7° （range 25°-70°） with the average correction rate be-ing （54.3±11.5）%;the kyphosis Cobb angle was 45.7°±12.4° （range 38°-65°） with the correction rate being （52.6±12.8）%. There were significant differences in the major coronal Cobb angle and kyphosis Cobb angle before and after PVCR （P〈0.05）. At the final follow-up, the coronal Cobb angle and kyphosis Cobb angle was 48.7°±11.3°（range 30°-82°） and 47.9°± 13.2° （range 40°-75° ）, respectively;the average lost rate was （3.8 ± 2.6）%and（5.7 ± 3.4）%. No significant differences were found in the coronal Cobb angle or kyphosis Cobb angle 2 weeks after PVCR and at the last follow-up （P〉0.05）. Conclu-sions：Two-staged surgery is safe and effective for severe rigid kyphoscoliosis associated with CM.