马方综合征合并脊柱侧弯畸形的外科治疗

Surgical treatment for scoliosis in Marfan syndrome

目的 :探讨马方综合征合并脊柱侧弯的临床特点以及评价手术治疗的临床疗效。方法 :回顾性分析我院收治的 17例以脊柱侧弯畸形为主要表现的马凡综合征患者采用手术治疗的临床资料。结果 :所有病人手术成功 ,平均手术时间 3.2 (2 .3～ 3.9)h ;平均出血量 6 0 0 (40 0～ 890 )ml ;伤口引流量平均 35 0 (2 80～ 5 5 0 )ml ;术后平均身高183.5cm ,较术前平均增高 5cm ;平均矫正率 5 8% ,旋转矫正平均Ⅰ度。经 6个月至 6年的随访 ,矫正度丢失平均 6° ,无假关节形成及脊髓神经损伤等并发症的发生。结论 :马方综合征合并脊柱侧弯常见于婴幼儿和青少年 ,其侧弯较僵硬 ,角度较大 ,采用脊柱侧弯矫形植骨融合技术 。

Objective To study the clinical features of scoliosis in Marfan syndrome treated with surgery and to evaluate the clinical efficacy of the surgical treatment. Methods The clinical data of 17 cases of scoliosis in Marfan syndrome treated with surgery were retrospectively analyzed. Results The surgical treatment was successful in all the patients. The average operative time was 3.2 (2.3～3.9) hours; the mean bleeding volume was 600 (400～890) ml; the mean drainage volume was 350 (180～550) ml ; the average postoperative height was 183.5 (172.0～188.5) cm which was 5 cm higher than that before the operation; and the average correction rate was 58%. All the patients were followed up for 6 months～6 years (26 months). Six correction degrees were lost. No pseudoarthrosis and neurologic deficit symptom were found. Conclusion Scoliosis in Marfan syndrome is easily found in infants and adolescents. It is rigid and has larger curves. Orthopaedics can often get satisfactory clinical outcomes.