Thione Alessandro, Cavadas Pedro C, Espadero Alberto Perez, Rubi Carlo G
Clinica Cavadas, Reconstructive Surgery, Hospital de Manises, Valencia, Spain.
Plast Reconstr Surg Glob Open. 2017 May 23;5(5):e1292. doi: 10.1097/GOX.0000000000001292. eCollection 2017 May.
Amyoplasia congenita, or "classic distal arthrogryposis," is the most common disorder among the congenital, non-progressive, multiple joint contractural conditions named arthrogryposis. The cause remains unknown, and it occurs sporadically. Abnormal neurological examination indicates that movement in utero was diminished as a result of an abnormality of the central or peripheral nervous system, the motor end plate, or muscle. The absence of central neural pathology indicates the origin in akinetic fetal condition. Three weeks are enough to cause muscle weakness and joint fibrosis. Joint contractures in amyoplasia are often rigid and refractory to nonoperative treatment such as passive stretching. Surgery is focused on each patient's need respecting adaptive maneuvers to accomplish daily tasks. We present a case in which pectoral major muscle had no strength for pinching; a trapezius muscle transfer was planned to obtain an interbrachial pinch useful for grasping.
先天性肌发育不全，或“典型远端关节挛缩症”，是先天性、非进行性、多关节挛缩病症（统称为关节挛缩症）中最常见的疾病。病因尚不清楚，且为散发性。异常的神经系统检查表明，由于中枢或周围神经系统、运动终板或肌肉异常，子宫内的活动减少。中枢神经病理学检查未发现异常，提示其起源于胎儿运动不能状态。三周时间足以导致肌肉无力和关节纤维化。先天性肌发育不全中的关节挛缩通常较为僵硬，对被动拉伸等非手术治疗无效。手术针对每位患者的需求，注重采用适应性动作来完成日常任务。我们报告一例病例，该患者胸大肌无力进行捏取动作；计划进行斜方肌转移术，以获得有助于抓握的双臂间捏取动作。
