马凡综合征合并脊柱侧凸的手术矫治

Orthopedic procedure of scoliosis associated with Marfan′s syndrome

目的 评价脊柱后路矫形内固定植骨融合术治疗马凡综合征合并脊柱侧凸的治疗效果。方法 马凡综合征合并脊柱侧凸患者12例，进行脊柱后路矫形内固定植骨融合术，术前脊柱侧凸主弯Cobb角62°～90°（71°±6°）。结果 手术时间3．4～4．8（4．2±0．4）h，出血量550～920（690±117）ml。全部患者均获随访，时间0．9～4．8年，未发生神经及其它系统并发症。术后主弯Cobb角24°～37°（29°±3°），矫正率51％～65％（59％±4％），植骨全部融合，无假关节形成及断钉、脱钩发生。结论 脊柱后路矫形内固定植骨融合术治疗马凡综合征合并脊柱侧凸，对侧凸矫形疗效满意。

Objective To evaluate the effect of orthopedic procedure with the operation of posterior correction, fixation and fusion for scoliosis associated with Marfan＇s syndrome. Methods 12 cases of scoliosis associated in Marfan＇s syndrome were treated by posterior operation of correction, fixation and fusion. The preoperative major Cobb＇s angle was 62°～90° （71°±6°）. Results Theoperationtimewas3.4～4.8（4.2±0.4）handtheamount of blood loss was 550 ～ 920（ 690± 117 ） ml. All the patients were followed up for 0.9 ～ 4. 8 year and there was no neurologic or other complications. The postoperative Cobb angle was 24°～ 37°（29°± 3°） and the correction rate was 51% ～ 65% （59%～4% ）. The solid fusion was obtained, while no pseudoartieulation formation and breakage of the internal fixation was found. Conclusions The operation of posterior correction, fixation and fusion could often get satisfactory effect to scoliosis associated in Marfan＇s syndrome.