Bayne L G
Clin Orthop Relat Res. 1985 Apr(194):68-73.
Arthrogryposis of the upper extremity is easy to diagnose. The shoulders, when affected, are adducted and internally rotated; they are thin, and very little girdle muscle is noted. The elbows are usually straight, and extension contractures are present. The hand and wrist are clublike; the wrist is contracted in flexion, with slight ulnar deviation. The thumb is usually adducted and flexed in a palmar direction. The small joints of the fingers are stiff, and frequently the fingers are ulnar deviated. Early treatment consists of passive stretching of the contracted parts by either plaster casts or splints. If successful, this treatment is followed by functional splinting. If stretching is not successful, then surgical release of contracted major joints or parts can be helpful. Tendon transfers are used to give a dynamic force to aid correction of the deformity and provide useful motion. Surgical correction of small joints of the hand has not proved too successful and frequently will decrease mobility even further. The goal in treating upper extremity deformities in arthrogryposis is to provide one extremity that can be brought to the mouth for feeding and hygiene and one that can be used to push up from a sitting position or to be used with a crutch if necessary. Hand function can be improved by careful evaluation and planned procedures that are consistent with the above goals.
上肢关节挛缩症易于诊断。肩部若受影响，会内收并内旋；肩部较瘦，几乎看不到肩胛带肌。肘部通常伸直，存在伸展挛缩。手和腕部呈棒状；腕部屈曲挛缩，伴有轻度尺侧偏斜。拇指通常内收并向手掌方向屈曲。手指的小关节僵硬，手指常向尺侧偏斜。早期治疗包括用石膏管型或夹板对挛缩部位进行被动伸展。若治疗成功，后续进行功能性夹板固定。若伸展治疗不成功，则对挛缩的主要关节或部位进行手术松解可能会有帮助。肌腱转移用于提供动力，以辅助矫正畸形并提供有用的活动。手部小关节的手术矫正尚未证明非常成功，而且常常会进一步降低活动度。治疗关节挛缩症上肢畸形的目标是使一只上肢能够举到嘴边用于进食和清洁，另一只上肢能够用于从坐姿撑起身体，必要时可与拐杖配合使用。通过仔细评估和符合上述目标的计划性手术，手部功能可得到改善。
