Surgical management of arthrogryposis in the upper extremity.

Arthrogryposis multiplex congenita is a rare congenital disorder in which the extremities are affected with severe resistant musculoskeletal contractures. In the upper extremity, these deformities typically consist of internal rotational contractures of the shoulder, extension deformity of the elbow, flexion and ulnar deviation of the wrist, thumb-in-palm deformity, and digital interphalangeal joint flexion contractures occurring singularly or in combination. At the Houston Unit of the Shriners Hospital for Crippled Children, during the interval from 1962 to 1982, 25 patients underwent 56 operative procedures to correct upper-extremity deformities due to arthrogryposis multiplex congenita. Functional improvement was observed clinically and reported subjectively by the patients or their parents in 75% of the cases.