Radial Clubhand Treatment & Management

Updated: Apr 20, 2018
  • Author: Scott H Kozin, MD; Chief Editor: Harris Gellman, MD  more...
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Treatment

Approach Considerations

The objective of treatment in radial clubhand is to reduce the functional deficit incurred by a short or absent radius, a short ulna, an abnormal muscular anatomy, and a radial deviation of the wrist. Type I radial clubhands have minor radial deviation of the wrist, which creates less of a functional problem than types II, III, and IV (see Presentation, Classification). In those children with considerable absence of the radius, the wrist assumes severe radial deviation that increases to 90° over time. This further compromises the flexor and extensor tendons, creating functional difficulty.

Thumb hypoplasia also requires consideration in the formulation of a treatment plan for radial clubhand. An absent or deficient thumb inhibits use of the hand. Reconstruction or pollicization is necessary to optimize hand function. [25]  Thumb reconstruction is usually delayed until after forearm treatment. Centralization is indicated in radial clubhand types II, III, and IV, in which there is severe radial wrist deviation and insufficient support of the carpus.

Contraindications for surgical intervention are mild (type I) deformity in children and elbow extension contractures that prevent the hand from reaching the mouth if the deformity at the wrist is corrected. Surgery is also contraindicated for adults who have adjusted to their deformity.

Numerous modifications and advances have been made in the technique of centralization. Improved methods have been developed to balance the wrist with additional tendon transfers or overcorrection of the wrist into ulnar deviation (ie, radialization). Better attempts at stretching the soft tissue with distraction techniques and bone-lengthening procedures also are used today. [26, 27, 28] In addition, microsurgical transfer of a viable growth plate (fibula, second toe) to the radial side of the forearm provides a support of the radial carpus that continues to grow over time.

A successful centralization still results in a shortened forearm segment secondary to altered growth of the ulna. The short forearm is both a cosmetic and a functional problem for the teenager with radial deficiency. Lengthening of the ulna can be accomplished by using distraction osteogenesis. Uniplanar and multiplanar devices have been employed depending on the deformity, forearm size, and surgeon preference.

Successful lengthening results in functional improvement because an increased volume of space becomes available for the hand, though complications are common. [29]  Restoration of near-equal forearm length promotes use of the extremity during activities of daily living. [30]  However, serious complications can occur with lengthening procedures in children with radial deficiencies. [31] The appropriate indications and age for surgery and amount of length obtainable remain profound questions.

These procedural changes represent new concepts to correct radial clubhand. These technologic advances in limb lengthening and microsurgery add innovative methods to better correct the deformity and provide osseous support. Follow-up results of vascularized second metatarsophalangeal joint transfer to stabilize the carpus are encouraging with respect to motion and less recurrence.

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Medical Therapy

Medical treatment is directed at any of the aforementioned 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. Instruction in passive stretching of the taut radial structures is provided 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. Serial casting can also be used to gradually stretch the tight radial structures.

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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 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 bony procedures are performed simultaneously to provide better alignment of 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 of the ulna consists of a closing-wedge osteotomy when there is considerable (ie, >30°) ulnar bow. Bony reconstruction of the distal radius is more difficult; 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 efforts were unsuccessful; 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. [32, 33]

Preparation for surgery

The main preoperative emphasis is placed on the status of soft tissues. Stretching and splinting of the taut radial structures is required before 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 (eg, in older children or patients with a recurrent deformity). [34, 35]  A uniplanar or multiplanar device can be used (see the images below).

Uniplanar external fixator. Uniplanar external fixator.
Multiplanar external fixator. Multiplanar external fixator.

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 (see the image below). Angulation of more than 30° usually necessitates corrective osteotomy at the time of centralization to realign the forearm.

Construction of lines to calculate ulna curvature, Construction of lines to calculate ulna curvature, hand-forearm angle, and total angulation.

Operative details

The basic goals of treatment are as follows [16] :

  • Correct radial deviation of the wrist
  • Balance the wrist on the forearm
  • Maintain wrist and finger motion
  • Promote growth of the forearm
  • Improve function of the extremity
  • Enhance limb appearance for social and emotional benefit

The digital abnormalities also require consideration during formulation of a treatment plan, in that 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 (see the image below).

Ulnar incision to centralize carpus and proximal i Ulnar incision to centralize carpus and proximal incision for corrective osteotomy.

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. If taut radial structures remain, 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.

If there has been preliminary external fixator distraction, a single incision may be made, 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 excision of redundant ulnar tissue (see the image below).

Single incision following preliminary external fix Single incision following preliminary external fixation.

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.

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. [32]  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. [16, 36]  Maintenance of the carpus at the end of the ulna without sacrificing wrist mobility or stunting forearm growth remains a daunting task. [37]

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Postoperative Care

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 before removal. Some authors recommend 6 months of fixation before removal.

After K-wire extraction, a splint is made and removed for exercises, with gradual weaning from the splint over the following 4-6 weeks. A nighttime splint regimen is encouraged until skeletal maturity is reached.

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Complications

Complications are common after 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 (see the images below) is the most common source of failure after centralization, and the cause appears to be multifactorial. 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. Recurrence after centralization.
Marked recurrence of radial deviation. Marked recurrence of radial deviation.
Ilizarov device applied for correction of recurren Ilizarov device applied for correction of recurrent deformity.

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.

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Long-Term Monitoring

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. [38]  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 (see the image below) may be an option to increase forearm length.

Forearm lengthening with distraction osteogenesis. Forearm lengthening with distraction osteogenesis.
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