Treatment Of Radial Club Hand Research Articles

The Paley ulnarization of the carpus with ulnar shortening osteotomy for treatment of radial club hand.

Recurrent deformity from centralization and radialization led to the development in 1999 of a new technique by the author called ulnarization. This method is performed through a volar approach in a vascular and physeal sparing fashion. It biomechanically balances the muscle forces on the wrist by dorsally transferring the flexor carpi ulnaris (FCU) from a deforming to a corrective force. The previous problems of a prominent bump from the ulnar head and ulnar deviation instability were solved by acutely shortening the diaphysis and by temporarily fixing the station of the carpus to the ulnar head at the level of the scaphoid. This is the first report of this modified Paley ulnarization method, which the author considers a significant improvement over his original procedure.

Management of congenital radial club hand by gradual correction

Introduction Severe congenital radial club hand is aesthetically unacceptable. This paper represents our experience in treating early and late diagnosed cases using gradual distraction by Ilizarov external fixator. Methods We treated 34 cases of congenital radial club hand with an age ranged from 1 to 15 years. There were 20 girls and eight bilateral cases. Three had been treated on both sides. So, we have treated 37 limbs. Nine cases had been operated before. Centralization alone was done in 12 cases and followed by lengthening in eight cases. Ulnar lengthening and gradual correction of wrist deformities were done for the rest of cases. The patients were followed clinically and radiographically with the following parameters: hand forearm angle, range of motion, daily functional activities, extent of lengthening achieved, and cosmetic improvement. Results The follow up ranged from 1 to 10 years. The magnitude of lengthening achieved ranged from 5 to 11 cm. The average healing index was 52.02 days/cm with cosmetic appearance satisfaction in all cases. Complications included; pin tract infection in 24 cases, flexion contractures of the elbow and fingers in 26 cases [which mostly disappeared during follow up], and spontaneous ulnocarpal fusion in 2 cases. Two cases suffered fracture in the regenerate zone. Conclusions The use of the Ilizarov method with gradual distraction of bone and soft tissues in treatment of radial club hand was effective in forearm lengthening with functional and cosmetic improvement.

Occurrence of radial club hand in children with different syndromes

Aim. Clinical analysis of congenital radial club hand as part of some genetic syndromes as well as the optimization of methods of non-surgical and surgical treatment of patients with this pathology. Material and Methods. From 2007 to 2014, we conducted a survey of 170 children with congenital radial club hand. Among them, 32 patients were diagnosed (18.8%) with different syndromes. We assessed the degree to which the radius was underdeveloped among this group of patients as well as the management features of patients according to various comorbidities. Results. The assessment identified Holt-Oram syndrome in 17 children (nine boys and eight girls; 53.1%) and TAR-syndrome in nine children (four boys and five girls; 28.1%). VACTERL syndrome was detected in four male patients (12.5%) and Nagera syndrome was observed in two children (one boy and one girl; 6.25%). Surgical treatment of radial club hand in patients with genetic syndromes is the same as that of the patients with isolated congenital radial club hand: a single- or two-stage correction of the hand relative to the ulna with subsequent reconstruction of the rays of the hand. The duration of treatment of such patients did not significantly differ compared to the patients with isolated congenital radial club hand. Conclusion. Congenital radial club hand, identified as part of genetic syndromes, requires a comprehensive examination to diagnose comorbidities, observation, and treatment by specialists to determine the optimal age for surgical correction of the existing strain of the upper limb.

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

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.

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

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.

Management of radial clubhand with gradual distraction followed by centralization

Background:Treatment of radial clubhand has progressed over the years from no treatment to aggressive surgical correction. Various surgical methods of correction have been described; Centralization of the carpus over the distal end of the ulna has become the method of choice. Corrective casting prior to centralization is an easy and effective method of obtaining soft tissue stretching before any definitive procedure is undertaken. Moreover, it helps put the limb in a correct position. The outcome of deformity correction by serial casting / JESS distractor followed by centralization is discussed.Materials and Methods:In a prospective study, of 17 cases with 18 radial clubhands of Heikel's Grade III and IV (with average age 11 months (range 20 days – 24 months) with M:F of 2.6:1, were treated by gradual soft tissue stretching using corrective cast (14 cases) and JESS distraction (4 cases), followed by centralization (16 cases) or radialization (2 cases) and tendon transfers.Results:The average correction attained during the study was 71° of radial deviation and 31° of volar flexion. The average third metacarpal to distal ulna angle in anteroposterior and lateral view at final follow-up was 7° in both views. Angle of movement at elbow showed a small increase from 99° to 101° during the follow-up period. However, the range of movement at fingers showed increase in stiffness during the follow-up. No injury occurred to the distal ulnar epiphysis during the operative intervention. The results at the final follow-up, at the end of 2 years were graded on the basis of the criteria of F.W. Bora, and of Bayne and Klug. Considering the criteria of F.W. Bora, satisfactory result was shown by nine of the 18 hands (50%) while 16 out of 18 hands (89%) showed good or satisfactory result based on deformity criteria of Bayne and Klug.Conclusion:The management of radial clubhand by gradual corrective cast or JESS distractor followed by centralization and tendon transfers in children is an acceptable method of treatment with consistently satisfactory results, both functional and cosmetic.