Treatment is identical for both partial and complete radial deficits. In the first few months after birth, the dislocated hand is treated with corrective plaster casts in an approach similar to that used for clubfoot. Although it is usually impossible to relocate the hand with conservative measures, serial stretching and immobilization in a cast keeps the radial soft tissue structures stretched. Aggressive stretching regimens by the parents and day and night bracing can be used to assist this correction.

Surgical centralization of the hand over the ulna improves both appearance and finger function. A careful evaluation of hand function, especially of the effects of wrist fixation on hand activity patterns, should always precede surgery. The length of the limb, elbow flexion, and the effect of the malformation on the patient’s ability to reach should be noted. Flexion in the radial digits is usually inadequate, and patients tend to favor the often normal ulnar digits. In unilateral defects, wrist flexion is not essential and the advantages of surgery may outweigh the disadvantage of a fixed wrist. In bilateral defects, however, fixation of both wrists, while improving finger function, can compromise relatively good patterns of function. This is especially likely if elbow and shoulder movements are insufficient to allow functional positioning of the hands.

Surgery can be done in the patient’s first or second year if great care is taken to preserve the ulnar growth plate. In the centralization procedure, the curved ulna is straightened with multiple osteotomies and the hand is centered over the ulna and held in position with an intramedullary wire extending into the metacarpal of the index, middle, or ring finger (see Plate 4-46). The ulnar growth plate will continue to grow if it is not injured and if the intramedullary wire is placed through its central portion. Pollicization of the index finger to replace the thumb on one hand is occasionally done if the defect is bilateral.

After surgery, the limb is immobilized in a plaster cast for 2 to 3 months. Day and night bracing continues for 3 more months, and continued night bracing may be necessary throughout the growing years. As the child grows, the intramedullary wire is replaced or advanced distally into the metacarpal. Despite wire fixation into the hand and bracing, recurrence of deformity is common and some hand surgeons have abandoned this procedure in favor of soft tissue reconstructions with tendon transfers.

Thumb Defects. If the thumb is absent, the index finger can be pollicized. A floating thumb can be amputated and the index finger pollicized, or the thumb can be lengthened by metacarpal osteotomy, distraction, and bone graft. A hypoplastic thumb may be treated with metacarpal distraction and bone graft and a tendon transfer to compensate for the hypoplastic thenar muscle. Rotational osteotomy may be indicated for the nonopposed thumb.

Tibial Deficiency. Complete tibial deficiency is a serious defect; the affected leg is short, the foot is in varus position, the great toe is absent, and the knee is unstable. The tibia is absent, while the fibula is present but may be bowed. Because the fibula is completely unstable, the limb cannot bear weight. Furthermore, the patella is usually absent with no quadriceps function. Treatment with surgery and prostheses is rarely successful. The recommended treatment is knee disarticulation amputation and fitting with an end-bearing socket prosthesis.