Hand assessment and management of arthrogryposis multiplex congenita

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.