Arthrogryposis of the upper extremity.

Patients with arthrogryposis multiplex congenita of the upper extremities can be helped a great deal by comparatively minor procedures. At the outset it is important to decide which patients can be helped and which patients cannot owing to the severity of disease or the social situation. It is also important, of course, to decide which patients do not require surgical or other methods of treatment. Once these decisions are made, one can safely begin in most patients with nonoperative measures, which consist of serial corrective cast applications starting at birth with changes every two to five days initially and then less often as the deformity is corrected. In patients who do not respond to this treatment, surgical intervention is necessary. We feel that surgery will be necessary in a great many of these patients. Surgery most often consists of soft tissue releases about the elbow, principally posterior capsulotomy, and triceps lengthening with or without tendon transfers to gain active motion. In the shoulder, surgery is rarely indicated, and when it is, this will be an osteotomy. In the wrist and hand, bone operations are almost always indicated because soft tissue procedures usually fail. These patients have two advantages in spite of their severe problems: They have normal sensation and they are usually of average or above average intelligence. Any improvement in the position of their extremities and any decrease in deformity will improve their functioning significantly.