Multi-axial correction system in the treatment of radial club hand

Suneel B Bhat,Kriti Sehgal,B David Horn,Harish S Hosalkar,Atul F Kamath

doi:10.1007/s11832-009-0196-3

Abstract

BackgroundRadial club hand is a well-recognized congenital malformation characterized by hypoplasia of bone and soft tissue on the radial aspect of the forearm and hand. The modalities of treatment have traditionally varied from stretching casts with soft-tissue procedures to the use of multiple corrective osteotomies. These osteotomies can be stabilized by a variety of methods, including external fixators that allow the possibility of gradual distraction with neohistiogenesis. This current study outlines the usage of one such device (multi-axial correction system [MAC]) in the management of deformity associated with severe radial club hand.MethodsThree consecutive cases of unilateral or bilateral severe (Bayne type IV) congenital radial club hand were corrected using MAC fixation in the last 5 years. This is a retrospective review of all three cases. Data parameters included: patient demographics, presentation findings, degree of deformity, amount of correction/lengthening, length of procedure, length of treatment, and associated complications. The surgical technique is described in detail for the benefit of the readership.ResultsThe three patients with severe congenital radial club hand had a total of four limb involvements that underwent correction using osteotomies and usage of the MAC device for external fixation. All three patients underwent successful correction of deformity with the restoration of alignment, lengthening of forearm for improvement of function, and stabilization of the wrist (mean duration, mean lengthening, mean time to consolidation). The MAC system was well tolerated in all patients and associated complications were limited.ConclusionThe MAC fixator seems to be a good alternative modality of stabilization and correction for severe congenital radial club hand deformities. Its usage is fairly simple and it provides the ease of application of a mono-lateral fixator with far superior three-dimensional control, like the circular external fixator. We recommend that clinicians should add this modality to their armamentarium for the deformity correction of severe radial club hand and others in general.

Highlights

Longitudinal radial deficiency, or radial club hand, encompasses a collection of congenital deformities characterized by hypoplasia of bones, joints, muscles, tendons, ligaments, nerves, and vasculature on the radial aspect of the forearm [1, 2]
Most cases of radial deficiency are sporadic, and they are commonly associated with a number of congenital syndromes: thrombocytopenia absent radius (TAR) syndrome; vertebral defects, anal atresia, cardiac malformation, tracheoesophageal fistula, esophageal atresia, renal anomalies, and limb anomalies (VACTERL); Fanconi’s anemia; Holt-Oram syndrome; and trisomies 13 and 18 [1, 4]
We describe a series of three cases of severe radial club hand in which the multi-axial correction (MAC) fixation system was used to achieve successful deformity resolution and ulnar lengthening

Summary

Introduction

Longitudinal radial deficiency, or radial club hand, encompasses a collection of congenital deformities characterized by hypoplasia of bones, joints, muscles, tendons, ligaments, nerves, and vasculature on the radial aspect of the forearm [1, 2]. The modalities of treatment have traditionally varied from stretching casts with soft-tissue procedures to the use of multiple corrective osteotomies. These osteotomies can be stabilized by a variety of methods, including external fixators that allow the possibility of gradual distraction with neohistiogenesis. This current study outlines the usage of one such device (multi-axial correction system [MAC]) in the management of deformity associated with severe radial club hand

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