先天性多发性关节挛缩症合并脊柱侧凸的临床特征及疗效评价

目的 探讨先天性多发性关节挛缩症(arthrogryposis multiplex congenital,AMC)合并脊柱侧凸的临床特征和手术方式并评估疗效.方法 回顾性分析2001年11月至2006年8月在我院接受脊柱三维矫形手术治疗的AMC合并脊柱侧凸患者14例,男7例,女7例;年龄11～20岁,平均14.3岁.对侧凸类型进行分析并对其手术前后侧凸冠状面和矢状面Cobb角、骨盆倾斜角进行评价.结果 12例为长"C"型胸腰弯,5例合并先天性脊柱侧凸.14例患者主弯冠状面Cobb角术前平均79.1°,术后平均35.9°,平均矫正率为54.6%;10例合并胸椎前凸畸形者矢状位术前胸椎前凸Cobb角平均43.0°,术后胸椎后凸Cobb角平均16.4°;2例合并胸椎后凸畸形者矢状位术前胸椎后凸Cobb角平均91.5°,术后胸椎后凸Cobb角平均54.5°;3例术前骨盆倾斜角平均22.1°,术后平均13.3°.术后随访2.0～4.5年,平均29个月,末次随访时冠状面矫正丢失率平均6.8%.3例发生术中和术后并发症:1例后路矫形时发生大出血(4000m1);1例术后出现短暂性呼吸困难;1例二期后路内同定完成后出现完全性截瘫,术后3个月恢复正常.结论 AMC合并脊柱侧凸弯型以麻痹性神经肌源性长"C"型胸腰弯为主,可合并有先天性脊柱侧凸.脊柱三维矫形可以对AMC合并脊柱侧凸进行有效的矫正,但易发生并发症.

Scoliosis associated with arthrogryposis multiplex congenital: clinical caracteristics and surgical outcomes

Objective To evaluate the clinical characteristics and surgical outcomes of the scoliosis associated with arthrogryposis multiplex congenital (AMC). Methods This retrospective study included 14 cases (7 males and 7 females; average age, 14.3 years) with scoliosis associated with AMC from November 2001 to August 2006. The curve patterns and Cobb angles in the coronal and sagittal plane, and pelvic obliquity were evaluated before and after spinal surgery respectively. Results A long-C shaped thoracolum-bar curve was seen in 12 cases, and 5 cases were associated with congenital scoliosis. The mean Cobb angle of all the cases in the coronal plane improved from 79.1° to 35.9°. The mean correction rate was 54.6%. The mean Cobb angle of the thoracic lordosis in 10 cases was 43.0° preoperatively, and was corrected to thoracic kyphosis with a mean Cobb angle of 16.4°postoperatively. The mean Cobb angle of the thoracic kyphosis in 2 cases was improved from 91.5° to 54.5°. The mean pelvic obliquity in 3 cases was reduced from 22.1° to 13.3°. During a mean 29-month follow-up, the average loss of the frontal correction was 6.8%. Complications occurred in three cases: massive blood loss (4000 ml) in one case, a transient dyspnea in one case, and com-plete paraplegia after posterior correction with instrumentation in another case (recovered 3 months after op-eration). Conclusion A long-C shaped thoracolumbar curve, which is typical in scoliosis associated with paralytic neuromuscular disorders, is the most common pattern in scoliosis with AMC. And congenital verte-bral anomalies may also be found in AMC. Satisfactory correction can be achieved through three-dimension spinal correction, yet may result in perioperative complications.