eMedicine Specialties > Orthopedic Surgery > Hand & Upper Extremity
Radial Clubhand: Treatment
Updated: Feb 5, 2009
Treatment
Medical Therapy
Medical treatment is directed at any of the above-listed associated syndromes. Appropriate treatment for these conditions requires referral to pediatric subspecialists.
Correction of radial clubhand requires a combination of nonoperative and operative management that begins shortly after birth. Passive stretching of the taut radial structures is instructed at the initial visit. This stretching is performed at each diaper change and at bedtime. A stiff elbow with limited motion also is stretched during this time. Splint fabrication is difficult in the newborn, especially with a shortened forearm. Therefore, splint use is delayed until the forearm is long enough to accommodate a splint.
Surgical Therapy
Centralization of the wrist on the ulna is the standard treatment to correct radial deviation. This procedure is performed in patients aged approximately 1 year. Surgery at this time allows for improvement in forearm length and provides a foundation for the development of motor function within the hand. This timing also allows additional reconstruction for thumb hypoplasia at a relatively early time. Such early intervention takes advantage of the ability of the immature brain to adjust. Children with bilateral deficiencies that affect both the forearm and thumb require staged treatment to gain maximal use of the reconstructed limbs.
Tendon and/or bony procedures are performed simultaneously to better align the forearm and to balance the wrist. Tendon transfers are used to attempt to correct the muscular imbalance and include advancing the extensor carpi ulnaris to increase its moment arm for ulnar deviation and transfer of the flexor carpi ulnaris to the extensor carpi ulnaris. Other, less common options are transfer of the index and long flexor digitorum superficialis around the ulna to the dorsum of the wrist, transfer of the flexor and extensor carpi radialis to the ulnar side, and proximal advancement of the hypothenar muscles to the ulna.
Bony correction consists of a closing-wedge osteotomy when there is considerable (ie, >30°) ulnar bow. Bony reconstruction of the distal radius is more difficult to perform; an attempt is made to provide osseous support to the radial side of the carpus. Initial efforts consisted of nonvascularized transfer of a bone graft (tibia, fibula). These techniques were unsuccessful, as continued ulnar growth resulted in loss of support. However, innovative procedures involving microsurgical transfer of a vascularized bone graft along with its growth plate (fibula, second toe) have been encouraging.10
Preoperative Details
The main preoperative emphasis is placed on the status of soft tissues. Stretching and splinting of the taut radial structures is required prior to surgery. Failure to elongate the tight radial side limits the ability to centralize the wrist on the ulna. Preliminary soft-tissue lengthening with an external fixator is a viable option in cases recalcitrant to stretching, such as in older children or patients with a recurrent deformity.11,12
Preoperative measurements of the degree of active and passive motion of the digits and wrist are recorded. Radiographs in the anteroposterior and lateral projection, including the elbow and hand, are obtained. The degree of ulnar bow is calculated from the lateral radiograph as the angle between the proximal and distal ulna. Angulation of more than 30° usually requires corrective osteotomy at the time of centralization to realign the forearm.
Intraoperative Details
The basic goals of treatment in radial deficiency are as follows8 :
- Correct the radial deviation of the wrist
- Balance the wrist on the forearm
- Maintain wrist and finger motion
- Promote growth of the forearm
- Improve the function of the extremity
The digital abnormalities also require consideration during formulation of a treatment plan, as stiff fingers and a deficient thumb will hamper prehension and create an additional functional handicap. Centralization remains the principal procedure to realign the carpus onto the distal ulna and is indicated in types II, III, and IV radial deficiencies. Contraindications for surgical intervention are a limited life expectancy in a child, mild deformity with adequate support for the hand (type 1), an elbow extension contracture that prevents the hand from reaching the mouth, and, in adults, adjustment to the deformity.
Centralization is performed at about age 1 year. Multiple surgical approaches have been described, with 2 incisions providing adequate exposure and release of contracted tissues. A radial zigzag incision is performed along the fold between the hand and forearm. This design allows adequate exposure and Z-plasty skin lengthening after centralization. The anomalous dorsal branch of the enlarged median nerve must be identified in the skin fold at the wrist. Aberrant preaxial musculotendinous units and anomalous contracted fibrous bands are released to allow adequate passive correction of the carpus centered over the ulna.
A second incision is performed, beginning dorsally at the midline of the wrist and extending ulnarly in a transverse and elliptical fashion to the volar midline. This design provides exposure to the carpus and allows for excision of redundant ulnar tissue (see Image 3). The flexor carpi ulnaris and ulnar neurovascular structures are identified and protected. The carpus is exposed by a transverse arthrotomy, and redundant fibrous tissue is excised from the ulnocarpal joint. The carpus is then reduced onto the distal ulna for centralization.
Ulnar incision to centralize carpus and proximal incision for corrective osteotomy.
Failure to achieve centralization requires repeat examination for any persistent contracted or fibrotic radial tissue. In severe cases, adequate reduction cannot be achieved, and alternative surgical means are necessary, such as carpectomy, limited shaving of the distal ulna epiphysis while avoiding injury to the growth plate, or shortening osteotomy of the ulna to reduce soft-tissue tension. Another option is application of an external fixator, followed by postoperative distraction and delayed formal centralization.
Soft-tissue stabilization and balancing are performed with dorsal capsular reefing, distal advancement or reefing of the extensor carpi ulnaris insertion, and transfer of the flexor carpi ulnaris to the dorsum. These manipulations redirect the palmar and radial deviating forces to resist recurrence of deformity.
The wrist is held reduced by a Kirschner wire (K-wire), which is placed through the carpus and third metacarpal and into the ulnar shaft. If the ulnar angulation is more than 30°, a diaphyseal closing-wedge osteotomy is performed at the apex of the deformity. The osteotomy is secured with the same K-wire used to maintain centralization. Additional K-wires may be used for added stability.
Numerous technical modifications and advancements have been proposed to sustain a well-aligned wrist position, including correction of the ulnar bow, radialization or overcorrection of the carpus, tendon transfer, capsular plication, and prolonged pin fixation. Even microvascular free toe transfer to support the radial side of the wrist with a growing part has been advocated.10 The toe proximal phalanx is fused to the base of the second metacarpal, and the proximal metatarsal is affixed to the side of the distal ulna. This joint transplantation avoids direct manipulation of the ulnocarpal joint, and the transfer grows at a rate similar to that of the adjacent ulna. Unfortunately, no method reliably and permanently corrects the radial deviation, balances the wrist, and allows continued growth of the forearm.8,13 The maintenance of the carpus at the end of the ulna without sacrificing wrist mobility or stunting forearm growth remains a daunting task.14
Postoperative Details
Immediate active and passive digital motion is initiated, along with measures to reduce swelling. The timing of K-wire removal is controversial. At least 8-10 weeks of fixation is required prior to removal. Some authors recommend 6 months of fixation prior to removal.
Following K-wire extraction, a splint is made and removed for exercises, with gradual weaning from the splint over the next 4-6 weeks. A nighttime splint regimen is encouraged until skeletal maturity is reached.
Follow-up
Patients with radial deficiencies require follow-up into adulthood. The evaluation should include not only the status of the centralization but also any additional operative and nonoperative needs.15 The shortened extremity with diminished motion may not be able to accomplish certain functions. These tasks often can be carried out with the use of assistive devices. In addition, as the child ages, distraction osteogenesis may be an option to increase forearm length.
Complications
Complications are common following centralization and can occur at the time of surgery or during the postoperative or follow-up period. Many of these complications are minor and do not impact the overall outcome. These include pin-tract irritation and a transient diminution in finger motion.
Recurrence is the most common source of failure after centralization, and the cause appears to be multifactorial (Image 6). Operative causes of failure include the inability to obtain complete correction at surgery, inadequate radial soft-tissue release, and failure to balance the radial force. Postoperative reasons consist of early pin removal, poor postoperative splint use, and the natural tendency of the shortened forearm and hand to deviate in a radial direction for hand-to-mouth use.
Recurrence after centralization.
The application of sophisticated techniques, such as distraction osteogenesis and microsurgery, to the treatment of radial clubhand introduces additional potential complications, such as fracture of the regenerate bone, digital stiffness from lengthening, and vascular thrombosis of the microsurgical anastomosis.
More on Radial Clubhand |
| Overview: Radial Clubhand |
| Workup: Radial Clubhand |
 Treatment: Radial Clubhand |
| Follow-up: Radial Clubhand |
| Multimedia: Radial Clubhand |
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Keywords
radial clubhand, radial deficiency, pre-axial deficiency, preaxial deficiency, forearm deformity, hand deformity, congenital hand deformity, thumb hypoplasia, Holt-Oram syndrome, cardiac septal defects, thrombocytopenia with absent radius, TAR, Fanconi anemia, aplastic anemia, VACTERL syndrome, cleft palate, clubfoot, kyphosis, scoliosis, torticollis, rib deformities, congenital spinal deformity
