Congenital Radial Club Hand Research Articles

Bone-periosteal-muscle flap for ulnar lengthening in children with congenital radial club hand

Background. Congenital radial club hand is characterized by the underdevelopment of all forearm structures. Ulnar bone shortening ranges from 24.7% to 50.0% compared to the intact limb. The aim of the study was to evaluate the outcomes of ulnar lengthening by distraction osteogenesis in patients with congenital radial club hand type IV who underwent osteotomy with the formation of a bone-periosteal-muscle flap, and to compare these results with the treatment outcomes of the patients who had standard (oblique) ulnar osteotomy. Methods. The main group consisted of 20 patients who underwent osteotomy with the formation of a bone-periosteal-muscle flap during ulnar lengthening between 2019 and 2022. The control group included 19 patients (22 forearms) who underwent oblique ulnar osteotomy between 1998 and 2018. The following indicators were evaluated: length of the regenerate, distraction time, correction period, fixation index, osteosynthesis index, and complications. Results. A lengthening of 4.1 cm was achieved (30.7% of the initial ulnar bone length). The correction of angular deformity was 71.4%. Greater correction was achieved with osteotomy in the proximal ulna. In the subgroup with proximal segment osteotomy, the distraction and osteosynthesis indices were 25.6 and 25.7 days/cm, respectively. In the mid-third osteotomy group, these indices were 42.3 and 42.6 days/cm, respectively. Complications were limited to inflammatory phenomena in 30% of cases. All patients in the main group exhibited successful regenerate formation. Thus, the bone fragment with a periosteal-muscle pedicle serves as an additional source of osteogenesis during distraction. Conclusions. This study demonstrates the appropriateness of osteotomy with the formation of a bone-periosteal-muscle flap in children with congenital radial club hand. This technique allows for greater deformity correction, a shortened regenerate formation period, and a reduction in complications.

Acquired Radial Club Hand: An Algorithm to Manage Radial Deficiency.

Introduction Loss of radius either due to trauma or infection results in a deformity resembling a congenital radial club hand. This deformity results in difficulty to perform hand functions and cosmetic appearanceand is called acquired radial club hand. There are a few case reports for the treatment of this severe deformity, but there are no proper guidelines for the management of this disease. From our experience, we decided to provide treatment guidelines for acquired radial club hand. Objectives To evaluate the outcome of radial deformity treatment in acquired radial club hand injuries and develop a treatment algorithm. Patients and methods It is a case series study of 11 patients with acquired radial club hand. It was conducted at a tertiary care hospital in Pakistan, from year 2016 to 2022. Basic principles of management of infection and trauma were followed. For the treatment of radial deformity, different options were opted according to the type of deformity, following the principles of treatment of congenital radial club hand. The outcome was graded on functional activity, pain, and bony union. Results Out of 11 patients, 36.36% showed excellent results, 27.27% showed good results, 27.27% showed fair results, and 9.09% showed poor results. Results were excellent in all patients with avascularized bone graft and distraction lengthening, with or without the Darrach procedure. Of the patients in whom distraction lengthening was performed, one patient showed excellent results while the other patient achieved similar results after the Darrach procedure of ulnar shortening. In the case of one bone formation by radioulnar synostosis, the results were variable. Two of the patients showed good outcomes while the other two had fair outcomes. Results in the case of ulnar centralization were mixed with good, fair, and poor results in one patient each. After three months of follow-up, 87% of the patients showed fair to excellent results. Conclusion With our experience, we recommend an algorithm for the treatment of acquired radial club hand.

Use the Orto-SUV frame in children with congenital radial clubhand: A clinical case

BACKGROUND: The shortening of the forearm in children with congenital radial clubhand and multi-plane deformity of the ulna is challenging to treat by surgery. The literature shows single studies using the Ortho-SUV frame for the correction of forearm deformity in multiple exostosis chondrodysplasia and patients with posttraumatic deformities.
 CLINICAL CASE: A 17-year-old patient with congenital radial clubhand who had a relapse of hand deviation undergo correction of forearm deformity. The elimination of radial hand deviation and ulnar deformity correction in the distal part with subsequent elongation were performed.
 DISCUSSION: No data on the use of the Ortho-SUV frame for the correction of ulnar multiplanar deformity in radial clubhand are available. Indicators, i.e., fixation index, osteosynthesis index, lengthening, and complications, were compared with the indicators used for ulnar elongation with a small angle of radial deviation of the hand.
 CONCLUSIONS: Personalized plan of forearm deformity correction based on the anatomical features of the congenital radial clubhand and X-ray indicators allowed achieving good cosmetic and functional results.

Method of radioulnar superposition in the treatment of patients with congenital radial club hand

New technique for the treatment of congenital club foot in patients with great malformation of the radius in the distal forearm is presented. Surgical intervention consists of 2 stages. First the distraction device is applied to correct the radius deviation and simultaneously to descent the hand distally together with hypoplastic radius. On the second stage the distraction device is taken off, partial resection of the distal ulnar epiphysis and resection of the proximal end of the hypoplastic radius is performed with following reposition of the ends. This method radioulnar superposition enables to eliminate the club hand, to preserve the wrist joint and to restore its physiologic functions.

Mistakes in the treatment of congenital radial clubhand in children

There were 194 patients, aged 8 months-16 years, with congenital talipomanus radioflexa who had 254 deformity of upper extremity. Operative treatment was performed on 232 exptremities. Good results were achieved in 69%), satisfactory in 22%o of cases. In 9%o of patients (21 upper extremities) partial or complete relapses of deformity requiring repeated surgical correction were detected. Failed outcomes were due to inadequate preoperative planning, technical and methodic mistakes. Analysis showed if those errors were avoided, the significant decrease of deformity relapses rate could be achieved.

Lengthening of the ulna by external fixation in children with congenital radial club hand

Background. Congenital radial club hand is characterized by the radial deviation of the hand, the longitudinal underdevelopment of the forearm, and the dysfunction of the upper limb. The shortening of the ulna is observed in all types of congenital radial club hand. The average shortening of the ulna surgical treatment was 33.3% compared to the intact contralateral side.
 Aim. This study aimed to evaluate the results of ulna elongation by the method of external fixation, depending on the level of osteotomy, in patients with congenital radial club hand type IIIIV.
 Materials and methods. The treatment results of 36 patients with congenital radial club hand type IIIIV from 1998 to 2018 were analyzed. The average age of the patients was 7.4 years 3.5 years. The patients were divided into three groups, depending on the level of ulnar osteotomy. Shortening of the ulna, correction of the angle of deformity of the ulna, radial deviation of the hand, period of correction, elongation obtained, index of fixation and osteosynthesis, and associated complications were analyzed.
 Results. The observation period was an average of 5.8 years. Before surgical treatment, the ulna was 33.3% shorter, while after surgery, it was 16%. Before surgery, the angle of deformation was 20.5 14.8, while after surgery, it was 7.4 5.6; this gives an angle of deformity correction of 63.9%. The elongation of the ulna was 3.2 1.1 cm. In patients who underwent proximal osteotomy, the resulting elongation was 32% and 18.4% more, respectively, than in patients who underwent an osteotomy in the middle and distal sections of the ulna. In group 1, the correction period was 24.4% and 28.9% more than in groups 2 and 3, respectively. The index of fixation in group 1 was 53.6%, which was 45.7% less than in groups 1 and 3. Postoperative complications included a false joint (15%), inflammation (10%), and forearm deformities (7.5%).
 Conclusions. In patients with congenital radial club hand type IIIIV, the optimal part of an ulna osteotomy is the proximal section. With a hand deviation of more than 20, osteotomy is performed in the distal section with simultaneous correction of the deformity.

Lengthening of radius in patients with congenital radial club hand, type II

Background. Congenital radial club hand (CRCH) is characterized by longitudinal underdevelopment of the forearm and hand on the radial surface. Underdevelopment can range from hypoplasia to aplasia of the radius. More than 50 methods to correct the forearm deformities, depending on the degree of radius underdevelopment, have been proposed.
 Aim. We evaluated the results of CRCH treatment using microsurgical technique and external fixation.
 Methods. We analyzed 16 patients (age, 4.6 0.9 years) with CRCH type II, according to the classification of Bayne and Klug, treated between 1994 and 2017. The patients were divided into two groups: Group 1 were patients undergoing microsurgical autotransplants of the epimetaphyseal second metatarsal bone with growth plate to the position of the radius defect and group 2 were patients treated by lengthening of the radius with external fixation. We analyzed the types of deformities, size of the radius defects, and range of motion in upper limb joints before the stage of the lengthening. External fixation index and number of complications also were determined. The type and number of recurrent deformities and timing of their detection were analyzed.
 Results. The observation period ranged from 12 months to 10 years (average, 3.8 years). In group 1, good results were obtained in 62.5% of cases. After transplantation of the metatarsal bone growth plate, the work of the growth plate continued, characterized by increasing radius length in the later observation period. In group 2, good results were obtained in 50% of cases. Clinical and X-ray examinations showed recurrent hand deviation and radius shortening, which required repeated radius lengthening.
 Conclusion. Microsurgical transplantation of the second metatarsal bone with growth plate is accepted more in reconstruction of the radial bone in patients with CRCH type II due to creation of a growth zone in the distal part of the radius. Radius lengthening via external fixation is applicable while maintaining the distal epimetaphysis and normal transverse dimensions of the radial bone.

Recurrence rate of radial deviation following the centralization surgery of radial club hand.

Background: Congenital radial club hand (RCH), as a rare congenital deformity of the upper extremity, is characterized by a wide spectrum of malformations including radial deviation. Centralization surgery is the standard treatment for severe cases that have been associated with a high rate of recurrence. This study reports the long-term results and recurrence rate of radial deviation following the centralization surgery of RCH. Methods: The medical records of 13 congenital RCH patients (16 hands), who underwent centralization surgery, were reviewed retrospectively. Hand-forearm angle (HFA), hand-forearm position (HFP), and ulnar bow (UB) were used to assess forearm angles. Results: The mean age of the patients was 19.4±8.9 months, and their mean follow-up was 62.1±39.9 months. The mean HFA correction was 29.4°±23.9°, the mean HFA recurrence was 13.3°±13.7°, the mean correction of HFP was 13.4±7.3 mm, and the mean recurrence of HFP was 1.4±2.8 mm. The mean UB showed 7.6°±12.5° correction immediately after surgery and a further 3.6°±7.3° at the last follow-up (overall 11.2°±17.6°). A number of 12 out of 13 parents were completely satisfied with the results. Conclusion: According to our results, an acceptable long-term result is expected after the centralization surgery of RCH. However, the risk of the recurrent radial deviation is high and needs to be optimized in future investigations.

Vascularized second metatarsophalangeal joint transfer for radial deficiency - an update.

Vascularized second metatarsophalangeal joint transfer offers a possibility to reconstruct the radial support which is lacking in radial dysplasia. Our experience from 1987 to 2017 with 34 congenital radial club hand reconstructions have allowed a possibility for long-term evaluation of the method. Compared with conventional methods, second metatarsophalangeal joint transfer results in better wrist mobility and does not restrict typical ulnar growth. The balance of the wrist remains good until age 11. Thereafter, the growth of the vascularized bone graft transfer matches only partially the distal ulnar growth in adolescence, resulting in mild recurrence of radial deviation. A new option to create a two-bone forearm in selected Bayne-Klug Type III radial dysplasia cases will allow a relatively good pro-supination ability. Potentially, a proximal fibular epiphyseal transfer could be a future solution. Currently, a safe harvest of the proximal fibula at childhood remains controversial.

Skin necrosis following ilizarov application in an adolescent with radial club hand

Congenital radial club hand is a well-recognized congenital malformation of the upper extremity. It is characterized by radial deviation of the hand and shortening of the forearm. Soft-tissue distraction with an Ilizarov fixator followed by ulnar centralization in a staged approach for the Type IV radial club hand is well described in the literature. In this report, we describe a unique complication following Ilizarov application for soft-tissue distraction in a patient with Type IV radial club hand. A longitudinal skin necrosis appeared at the radial side of the forearm and hand that healed spontaneously after the cessation of distraction.

Comparative analysis of the effectiveness of the incision designs by Bayne and Evans in the correction of congenital radial clubhand in children

Introduction. In 1733, Petit first described the deformation of the forearm, congenital radial clubhand. A large number of modifications in the surgical treatment of this deformation have been developed. In the available literature, there is no comparative analysis of the applied designs of the sections.
 Aim. The goal is a comparative analysis of the effectiveness of the use of dorsal rotation flap by Evans, and incision designs by Bayne, in the correction of congenital radial clubhand in children.
 Material and methods. Between 2013 and 2016, block randomization of 40 children with congenital radial clubhand was used and grouped by procedures. Surgical correction was performed using two different incision designs (the first group by Bayne and the second by Evans). The analysis of the early postoperative complications was performed. The evaluation in the late postoperative period of soft tissue condition of the forearm using a pinch test, Vancouver scale, and a visual analog scale was carried out.
 Results. Based upon gender, patients were divided with male predominance in both groups with 7 girls and 13 boys in the first group, and 8 girls and 12 boys in the second group. In the immediate postoperative period, marginal necrosis was observed in the first group (35%). In the late postoperative period, the study showed an advantage of using dorsal rotation flap by Evans versus the incision designs by Bayne.
 Conclusion. Using incision designs by Evans with the formation of a dorsal rotation flap provided a more aesthetic and functional result.

Post-osteomyelitic-acquired radial club hand deformity in children treated by centralization of ulna.

Radial club hand deformity acquired post haematogenous osteomyelitis of radius bone is a very rare disease. Resulting in functional and cosmetic deficit of upper limb which is similar to congenital cases. For a long-time various surgeons attempted to reconstruct the deformity by bone grafting, plating, Ilizarov, monorail external fixator, callus distraction and so on. Keeping in mind that creating single bone forearm we proposed centralization of ulna on wrist to correct the deformity. In our institute, we conducted a rare study involving small number of patients ( n = 5), between February 2013 and November 2106. The study participants comprised four male children and one female child whose average age was 1.8 months. Participant inclusion criteria were no active infection, no distal end of radius (cartilage remnant) and only haematogenous osteomyelitis. Exclusion criteria were congenital radial club hand and active infection. Decrease in the angle of radial deviation (radiologically) preoperatively from 54° to 5° and angle of volar flexion from 34° to 4° were measured. Preoperatively no ulnar angulations were observed. Length of ulna remains similar to the opposite side. No neurovascular complication noted and full range of movement regained. Our case series is unique in terms of massive bone loss, that is, distal metaphysic and epiphysis, so we followed the statement of Ono et al. and did centralization of ulna over carpus and achieved good functional and cosmetic outcome at the cost of loss of motion at the wrist, concluding that treatment is primarily considered as salvage only.

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.

Challenging management of congenital bilateral radial club hand: A case report

Congenital radial club hand (CRCH) is a terminal deficiency characterized by the radial deviation of the hand, marked shortening of the forearm, and generalized underdevelopment of the extremity with an incidence rate of 1 per 100,000 live births. Both sexes are nearly or equally affected. Challenges that the disease poses to the patient are multifaceted, ranging from medical, social, cosmetic and functional issues which also are the multidimensional treatment modalities. We report a case of a 2-month-old baby with the severe case of bilateral CRCH. The patient had the centralization of the left ulna with Kwire stabilization. About 160d of correction was achieved. He did well and was discharged after two weeks at the orthopedic clinic for follow-up. Congenital radial club hand though uncommon may be amenable to surgery. Centralization is still the treatment of choice in severe cases and early intervention will offer a better outcome.

Congenital isolated right radial club hand

Congenital radial club hand (RCH) is an uncommon congenital anomaly characterized by various degrees of deficiency along the preaxial or radial side of the extremity. We present one such case of Type 4 congenital isolated RCH who presented to a tertiary care center in the Middle East.

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.

Centralisation progressive de la main dans la main bote radiale congénitale

The therapeutic objective of a congenital radial clubhand is to obtain, at the end of the growth, a functional hand, that is a hand with a thumb, in the axis of the forearm with the most cosmetic aspect as possible. The challenge lies in the fact that the hand is not in the axis of the forearm. Thus, some authors realize a surgical centralization or radialization by doing a direct release of the retracted soft tissues. On the other hand, it is possible to correct the hand without any invasive surgery by using an external fixator allowing to correct the hand progressively. The Taylor Spatial Frame fixator is a system of external fixation which allows, by using a software, to realize this correction accurately.

Pre-Centralization Soft Tissue Distraction for Bayne Type IV Congenital Radial Deficiency in Children

There are several surgical options for the treatment of severe congenital radial deficiency. The aim of the current study was to evaluate the results of a staged protocol consisting of gradual soft tissue distraction with an Ilizarov external fixator followed by centralization of the ulna. Three patients (four extremities) with Bayne type IV radial club hand were treated at an average age of 18 months. At a follow-up of 26 months, an average correction of 72 degrees in the hand-forearm angle and an improvement of 19 mm in the hand-forearm position were obtained. There were no cases of neurovascular compromise, wound infection, carpal resection, or ulnar shortening. All families were pleased with the results. These findings suggest that a staged surgical approach is a technically feasible alternative in the treatment of severe congenital radial club hand deformity in young children.

Residual deformity in congenital radial club hands after previous centralisation of the wrist

We used the Ilizarov method in seven patients with severe congenital radial club hands who had had previous wrist surgery, to correct residual shortening and bowing of the ulna together with recurrent wrist deformity. The mean age at operation was 6.5 years. The mean ulnar shortening was 5.3 cm and the mean angular deformity 42°. The mean length gained was 51% of the original ulna. The mean healing index was 46.9 days (29.8 to 64.0). The ratio of the length of the lengthened ulna to the normal side improved on average from 64% to 95%. The angular deformity was initially completely corrected in six out of seven patients. The length ratio, however, decreased to 83% at the final follow-up. In four patients, the angular deformity partially recurred. We recommend correction of congenital radial club hand by staged procedures. The first is centralisation and stabilisation of the wrist and the second lengthening of the ulna and correction of the angular deformity using the Ilizarov method.