Clin Orthop Surg.  2012 Mar;4(1):36-44. 10.4055/cios.2012.4.1.36.

Changing Paradigms in the Treatment of Radial Club Hand: Microvascular Joint Transfer for Correction of Radial Deviation and Preservation of Long-term Growth

• de Jong JP
• Moran SL
• Vilkki SK

• KMID: 1245397
• DOI: http://doi.org/10.4055/cios.2012.4.1.36

Abstract

Radial longitudinal deficiency, also known as radial club hand, is a congenital deformity of the upper extremity which can present with a spectrum of upper limb deficiencies. The typical hand and forearm deformity in such cases consists of significant forearm shortening, radial deviation of the wrist and hypoplasia or absence of a thumb. Treatment goals focus on the creation of stable centralized and functionally hand, maintenance of a mobile and stable wrist and preservation of longitudinal forearm growth. Historically centralization procedures have been the most common treatment method for this condition; unfortunately centralization procedures are associated with a high recurrence rate and have the potential for injury to the distal ulnar physis resulting in a further decrease in forearm growth. Here we advocate for the use of a vascularized second metatarsophalangeal joint transfer for stabilization of the carpus and prevention of recurrent radial deformity and subluxation of the wrist. This technique was originally described by the senior author in 1992 and he has subsequently been performed in 24 cases with an average of 11-year follow-up. In this paper we present an overview of the technique and review the expected outcomes for this method of treatment of radial longitudinal deficiency.

Keyword

Radial club hand; Radial longitudinal deficiency; Treatment; Microvascular joint transplantation; MTP-joint transfer

MeSH Terms

Forearm/abnormalities/*surgery
Hand Deformities, Congenital/*surgery
Humans
Joints/*transplantation
Metatarsophalangeal Joint/surgery
Radius/abnormalities/*surgery

Figure

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  Fig. 1 The metatarsal phalangeal joint transfer procedure begins with the placement of an external distraction device. (A) An example of such a uniaxial distraction device placed on the ulnar aspect of the hand. (B) Anterior posterior radiograph showing distracter in place. It is helpful if the distal pins can be placed through the small and ring metacarpal bones. This patient has already undergone a pollicization procedure.

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  Fig. 2 Planning for distraction includes radiographic templating after the distraction device is in place. Image shows projected placement of bone shelf in addition to space required for wrist reconstruction. A construct of at least 40 mm is required for adequate joint reconstruction. Distraction is continued at a rate of 0.5 mm a day until a 1 cm space is seen between the distal unlar physis and the visible radiocarpal bones. Following adequate distraction metatarsal phalangeal joint transfer is performed.

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  Fig. 3 Toe has been divided and is ready for transfer. Image shows the skin which has been mobilized off of the distal and middle phalanx to provide skin coverage over the radial aspect of the wrist.

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  Fig. 4 Final appearance of the foot 2 years after toe harvest.

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  Fig. 5 Bony fixation is initially created with K-wires. Bent K-wires may be buried beneath the skin and used to further stabilize the bony construct. (A) An anterior posterior (AP) radiograph showing final construct. (B) An AP radiograph of the reconstruction following distraction device removal at 8 weeks with consolidation of metatarsal to ulna shaft. (C) Metatarsal phalangeal (MTP) joint longitudinal growth is followed to ensure the MTP joint maintains equivalent growth with distal ulnar physis.

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  Fig. 6 Flap is monitored by examination of the skin paddle, temperature evaluation of the skin paddle and (if possible) the placement of an implantable Doppler probe around one of the veins to the toe joint. Fig. 6 is an image of the arm at the time of the first dressing change at 5 days. The blue wire at right aspect of image represents the implantable Doppler probe wire.

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  Fig. 7 Thirteen-year follow-up of the left wrist reconstruction in a 16-year-old woman following vascularized metatarsal phalangeal joint transfer. Note minimal radial deviation of the wrist and ability to generate significant force through a centralized and stabile wrist joint. Wrist range of motion is 10 degrees of wrist extension and 90 degrees of wrist flexion. Grip strength is 10 kg compared to 30 kg in the contralateral hand (Images in Fig 7. from courtesy of S.K. Vilkki, MD).

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