先天性肌肉、肌腱缺如或发育不良11例报告

目的 报告国内较为罕见的肌肉、肌腱先天性缺如或发育不良病例的症状和治疗。方法 回顾并分析1996年8月至2002年8月我科所收治的11例18侧先天性肌肉肌腱缺如或发育不良患者病史。其中，涉及拇指影响拇指功能的8例14侧，涉及其他手指或腕关节的3例4侧。统计受累肌肉以大鱼际肌为主，肌腱移位为主要的治疗方法。结果 采用肌腱移位术后，外观得以改善，畸形均矫正，功能恢复较满意。结论 肌肉肌腱缺如或发育不良较为少见，其中以拇指大鱼际肌及拇长伸肌腱缺如最为常见。发病原因目前尚无确切的结论。选用合适的动力肌行移位术可使功能得到较好恢复，手术疗效满意。

Congenital absence or dysplasia of the muscle or tendon:a report of 11 cases

ObjectiveTo report an unusual anomaly of muscle and tendon in the upper limb and its treatment. Methods11 cases of absence o r dysplasia of muscle and tendon treated in our hospital from August 1996 to Aug ust 2002 were revie wed. Congenital absence of thenar muscles was seen in 14 hands of 8 patients, an d congenital dysplasia of fingers was seen in 4 hands of 3 patients. The most in volved muscles were the thenar muscles. Surgical reconstruction using tendon tra n sfer was the treatment of choice. ResultsIn all the patients t he lost function was restored and the deformity was corrected after the operations.Concl usionCongenital absence or dysplasia of muscle and tendon of the upper limb is a ra re clinical entity. Absence of the thenar muscles and extensor pollicis longus i s most commonly seen. Up to now the pathogenesis of the disease is unclear. Tend on transfer using proper muscle power can achieve functional restoration. The resu lt is satisfactory.