Search

Abstract Background We developed a 2-stage, MTP (metatarsophalangeal) joint- plus ADM (abductor digiti minimi) tendon-transfer, procedure for treatment of hypoplastic thumb. This method is intended to achieve both structural and functional goals of reconstruction. Structurally, it preserves a five-digit hand with minimal donor site complications. Functionally, it provides a functioning opposable thumb. Case presentation The case series included 7 patients with type IV hypoplastic thumb. At the first stage non-vascularized joint (not bone) was transplanted. In the second stage abductor digiti minimi tendon was transferred. Patients were followed for a median 5-yr period (range: 37–79 months). Functional outcome was assessed using a modified Percival assessment tool. Participants aged 17 to 36 months at the time of surgery with (2 male, 4 female). All patients were able to grasp large and small objects after the procedure. The thumb tip could actively move to touch the tips of index (2 patients) middle, ring, and little fingers (all patients) in an ulnar ward sequence and vice versa. All patients attained the ability to do lateral, palmar, and tripod pinch. As for donor site complications, none of the patients were found to have difficulty walking or keeping their balance. Conclusions An alternative surgical procedure was developed to reconstruct a hypoplastic thumb. We obtained a good functional and cosmetic outcome with few donor site complications. Future studies will be needed to determine the long-term outcomes, to refine the selection criteria and to examine the necessity of additional procedure at the older ages.

Purpose: Publications evaluating the results of the ulna lengthening in congenital radial deficiency are based only on small groups of subjects which yield statistical studies of low scientific value. The aim was to examine the effectiveness of ulna lengthening in radial longitudinal deficiency and determine the number and quality of complications based on one of the most numerous study groups described in the literature. Methods: The material consists of a study group with 31 upper limbs of unmatured patients diagnosed with type III and IV radial longitudinal deficiency. The study group was evaluated based on the parameters known from the literature. The difficulties during elongation were classified according to Paley's classification. Results: The study group contained patients with a mean age of 9 years, and the number of boys and girls was comparable. Ulna length significantly increased after elongation compared to the initial bone length. The patient's age didn't affect the ulna lengthening, and the amount of elongation didn't significantly affect the total stabilization period. However, the total stabilization time increased with increasing patient age. Difficulties affected more than half of the cases. Conclusions: Ulna elongation in congenital radial deficiency results in significant lengthening of the ulna, and thus the entire forearm, compared to the initial bone length. This technique has a high percentage of difficulty, so its use should be considered after cautious discussion with the parents and patients. [ABSTRACT FROM AUTHOR]

Background: We developed a 2-stage, MTP (metatarsophalangeal) joint- plus ADM (abductor digiti minimi) tendon-transfer, procedure for treatment of hypoplastic thumb. This method is intended to achieve both structural and functional goals of reconstruction. Structurally, it preserves a five-digit hand with minimal donor site complications. Functionally, it provides a functioning opposable thumb. Case presentation: The case series included 7 patients with type IV hypoplastic thumb. At the first stage non-vascularized joint (not bone) was transplanted. In the second stage abductor digiti minimi tendon was transferred. Patients were followed for a median 5-yr period (range: 37–79 months). Functional outcome was assessed using a modified Percival assessment tool. Participants aged 17 to 36 months at the time of surgery with (2 male, 4 female). All patients were able to grasp large and small objects after the procedure. The thumb tip could actively move to touch the tips of index (2 patients) middle, ring, and little fingers (all patients) in an ulnar ward sequence and vice versa. All patients attained the ability to do lateral, palmar, and tripod pinch. As for donor site complications, none of the patients were found to have difficulty walking or keeping their balance. Conclusions: An alternative surgical procedure was developed to reconstruct a hypoplastic thumb. We obtained a good functional and cosmetic outcome with few donor site complications. Future studies will be needed to determine the long-term outcomes, to refine the selection criteria and to examine the necessity of additional procedure at the older ages. [ABSTRACT FROM AUTHOR]

Congenital radial longitudinal dysplasia remains an 'unsolved problem' in hand surgery. The challenges presented by the skeletal deficiency of the distal radius and soft tissue dysplasia of the severe radial longitudinal deficiency have been addressed by a number of techniques that aim to stabilize the position of the hand relative to the forearm and optimize forearm growth and hand function. Analysis of hand function and position in these children is difficult because of the abnormal 'wrist' mechanics, and the published results of the techniques used to date often lack a standardized approach and importantly the perception of function from the patient's perspective. The existing data is reviewed and compared with the results of cohorts from two major congenital upper limb centres. Soft tissue distraction prior to radialization or centralization may offer benefit in ulnar growth and forearm length but there is a need for further research into the long-term functional outcomes of the various techniques available to determine the optimal choice for these children. Level of evidence: V [ABSTRACT FROM AUTHOR]

Radial longitudinal deficiency is a rare skeletal anomaly characterized by a defect in the development of structures that form the radial half of the forearm. The disorder is associated with a large spectrum of preaxial abnormalities. It is shown that the thumb and preaxial carpal bones are almost always hypoplastic or absent in almost all types of radial longitudinal deficiency. Congenital dislocation of the dysplastic radial head may accompany this rare deformity. Herein, we present a 20-year-old male patient with radial longitudinal deficiency who had a markedly hypoplastic radius but had a thumb and carpal bones with normal size, shape, and joint relations. Further, the right radial longitudinal deficiency of our patient was unusually accompanied by left congenital radial head dislocation. Our case shows that, although rare, radial longitudinal deficiency can present without any carpal and thumb abnormalities. And the current case also shows that a contralateral sided congenital radial head dislocation may accompany radial longitudinal deficiency.

(1) Background: Patients treated with the two previous generations of ulnarization developed a bump related to the ulnar head becoming prominent on the radial side of the hand. To finally remedy this problem, a third generation of ulnarization was developed to keep the ulnar head contained. While still ulnar to the wrist center, the center of the wrist remains ulnar to the ulnar head, with the ulnar head articulating directly with the trapezoid and when present the trapezium. (2) Methods: Between 2019 and 2021, 22 radial club hands in 17 patients were surgically corrected with this modified version of ulnarization. (3) Results: In all 17 patients, the mean HFA (hand–forearm-angle) correction was 68.5° (range 12.2°–88.7°). The mean ulna growth was 1.3 cm per year (range 0.2–2 cm). There were no recurrent radial deviation deformities more than 15° of the HFA. (4) Conclusions: This new version of ulnarization may solve the problem of the ulna growing past the carpus creating a prominent ulnar bump. The results presented are preliminary but promising. Longer-term follow-up is needed to fully evaluate this procedure. [ABSTRACT FROM AUTHOR]

Radial club hand deformities are commonly treated with arthrolysis to allow centralization of the ulna. In this retrospective cohort study of 31 hands in 28 patients, we aimed to assess the outcomes of correction using progressive distraction and subsequent percutaneous pinning of the wrist with a corrective ulnar osteotomy. Mean follow-up time was 7 years (range 2 to 20). The angulation of the hand–forearm complex was decreased after each step of the procedure. Mean correction of the angulation was 64°, and the residual total forearm–hand angulation was 12° after completion of the surgery. At the time of bony maturity (four patients), all wrists had fused. Fifty-eight reoperations were required in 31 wrists because of pin migration or breakage, and in addition 18 secondary osteotomies of the ulna were performed. From this study we conclude that distraction and pinning provide satisfactory and stable realignment of the wrist to correct the deformity, but this treatment has drawbacks regarding the high number of reoperations and the loss of wrist mobility. Level of evidence: IV [ABSTRACT FROM AUTHOR]

Radial 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.Three 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.The 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.The 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.

(1) Background: Patients treated with the two previous generations of ulnarization developed a bump related to the ulnar head becoming prominent on the radial side of the hand. To finally remedy this problem, a third generation of ulnarization was developed to keep the ulnar head contained. While still ulnar to the wrist center, the center of the wrist remains ulnar to the ulnar head, with the ulnar head articulating directly with the trapezoid and when present the trapezium. (2) Methods: Between 2019 and 2021, 22 radial club hands in 17 patients were surgically corrected with this modified version of ulnarization. (3) Results: In all 17 patients, the mean HFA (hand–forearm-angle) correction was 68.5° (range 12.2°–88.7°). The mean ulna growth was 1.3 cm per year (range 0.2–2 cm). There were no recurrent radial deviation deformities more than 15° of the HFA. (4) Conclusions: This new version of ulnarization may solve the problem of the ulna growing past the carpus creating a prominent ulnar bump. The results presented are preliminary but promising. Longer-term follow-up is needed to fully evaluate this procedure.

• Radial club hand (RCH) is a rare congenital deformity leading in several functional and psychological problems. • Treatment of RCH should begin as soon as possible after birth. • Treatment of patients with RCH by primary traction and centralization and pollicization surgery, can greatly improve the deformity. • Early centralization and pollicization can significantly restore the range of motion and function in patients with RCH. Radial club hand (RCH) is a rare congenital deformity leading in several functional and psychological problems. However, our knowledge about the long-term functional outcomes of treating RCH is limited. In current study, we investigated the outcomes of centralization and pollicization using second or third metacarpal bone in RCH patients. There were 15 hands (13 patients) with RCH underwent centralization and pollicization using second or third metacarpal bone or tendon transfer. The patients aged 1.2 ± 1 years at the time of the surgery. On early postoperative x-rays, the forearm-hand angle was measured. The patients were followed for 6.2 ± 2.3 years. At the final visit, disabilities of arm, shoulder and hand (DASH) score was completed. Furthermore, forearm-hand angle and range of motion of both wrists in sagittal and coronal planes were measured. The mean of forearm-hand angle increased significantly. In 11 wrists, forearm-hand angle increased only 10 degrees of less. The range of operated wrist was improved in sagittal and coronal planes. The relative range of wrist motion in patients with unilateral deformity in sagittal and coronal planes was 83 ± 11 percent and 61 ± 12 percent. Three patients developed skin necrosis. Early centralization and pollicization using second or third metacarpal bone can significantly restore the range of motion and function in patients with RCH. [ABSTRACT FROM AUTHOR]

Introduction: Radial club hand is characterized by radial deviation of the hand, as a result of hypoplasia or absence of the radius. Material and methods: A descriptive, retrospective, cross-sectional and observational study of active patients diagnosed with radial club hand at the Shriners-Mexico Hospital was carried out. Results: We studied 71 patients and 92 limbs, the prevalence was 0.08%, 64.7% were men and 35.3% women. We observed 22 patients with involvement of the right thoracic extremity, 28 left and 21 bilateral. It was found that 93% of the patients had an associated syndrome. The most common type of radial longitudinal dysplasia was type 1. 58 limbs did not have a thumb. The State of Mexico is the most affected and 91.6% had a guardian with maximum high school education. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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 appearance and 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., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2023, Khan et al.)

To study the long-term results of radial club hand, regarding ulna growth, radial angulation, and volar subluxation using a 2-stage treatment protocol. From 1998 to 2009, 39 radial club hands (32 patients) were treated with distraction, radialization, and a bilobed flap. Long-term follow-up was available in 13 patients (17 hands; average 12.6 years, range 9–16 years). All 17 hands were classified as Bayne and Klug grade 3 or 4. The average age at distraction was 12 months (SD 5.3). The average age at radialization was 14 months (SD 5.8). At final follow-up, the average ulna length on the involved side was 69.3% of the uninvolved contralateral side in the unilateral cases. In the 4 bilateral cases, the average ulna length was 62% of the ulna length of a cohort of normal children. The transverse diameter of the ulna in the posteroanterior view was 79%, and in the lateral view 99%, of the radius on the contralateral side in the unilateral cases. The average radial deviation improved from 82° to 8° and the average volar subluxation improved from 20° to 12°. However, in 4 hands recurrent volar subluxation and required revision surgery. This approach to treatment was associated with consistent results in the correction of the radial angulation, volar subluxation, and ulna growth in long-term follow-up. Volar subluxation may result in a requirement for revision. Therapeutic IV. [ABSTRACT FROM AUTHOR]

Congenital radial longitudinal dysplasia remains an 'unsolved problem' in hand surgery. The challenges presented by the skeletal deficiency of the distal radius and soft tissue dysplasia of the severe radial longitudinal deficiency have been addressed by a number of techniques that aim to stabilize the position of the hand relative to the forearm and optimize forearm growth and hand function. Analysis of hand function and position in these children is difficult because of the abnormal 'wrist' mechanics, and the published results of the techniques used to date often lack a standardized approach and importantly the perception of function from the patient's perspective. The existing data is reviewed and compared with the results of cohorts from two major congenital upper limb centres. Soft tissue distraction prior to radialization or centralization may offer benefit in ulnar growth and forearm length but there is a need for further research into the long-term functional outcomes of the various techniques available to determine the optimal choice for these children. Level of evidence: V.

One concern in the surgical treatment of radial longitudinal deficiency (RLD) is certainly the skin incision. Over the years many different types have been proposed and used. We propose a new skin incision technique: a double Y sliding flap with the main body along the dorsal wrist crease, followed by raising a proximal and a distal flap providing wide access to the dorsal surface of the wrist joint. After correction of the wrist deformity, skin triangles are resected on the radial and ulnar sides of the incision. Then the skin of the distal flap is slid radially and proximally, filling the defect left by the resected radial triangle, while the proximal flap is slid in the opposite direction, enabling transverse closure along the ulnar side of the incision. The final scar comprises a central body along the dorsal wrist crease, and a radial branch. The aim of this study was to analyze the clinical results of this new double Y sliding flap approach for the surgical treatment of type III and IV RLD. We retrospectively reviewed medical records of surgical correction of RLD using our new incision, between January 2016 and December 2018 in our department of hand surgery. Endpoints comprised correction of redundant skin, scar appearance, and complications. Twelve limbs in 9 patients treated with this double Y sliding flap approach were reviewed: correction of redundant skin was systematic, only 2 limbs showed postoperative complications (1 case of notable edema and 1 of delayed wound healing), and scar aspect was graded good in 11 of the 12 cases. The double Y sliding flap was safe, with minimal complications, adequate skin restoration, wide exposure of the wrist, and esthetically good scar. LEVEL OF EVIDENCE: IV., (Copyright © 2022 SFCM. Published by Elsevier Masson SAS. All rights reserved.)

Congenital radial club hand is an intercalary or terminal deficiency characterized by radial deviation of the hand, marked shortening of the forearm, and generalized underdevelopment of the extremity. We performed centralization of the ulna in 12 forearms of 10 patients with severe congenital radial club hands. The mean follow up was 42 months after operation (24 to 68 months). The mean age at the operation was 16.8 months (7 to 42 months). The mean angular deformity before operation was 100 degrees (75 to 135). The angular deformity was corrected in all patients initially, but in the latest follow up the mean residual angular deformity was 19.58 degrees (5 to 45). The mean correction of angular deformity was 79.57 degrees (55 to 120). Range of motion in the wrist increased significantly after operation in both flexion-extension range and radial-ulnar deviation range (P

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.

To report the histopathological, electron microscopic, and immunohistochemical findings of tissue samples obtained from patients with radial longitudinal deficiency (RLD) and investigate the contribution of abnormality in soft tissues as a secondary driver of deformity in RLD. Specimens from radial-sided muscles and tendons were obtained at the time of surgery (either radialization or centralization) from 14 patients with 16 limbs affected with Bayne type 3 and type 4 RLD. The specimens were evaluated using light microscopy, electron microscopy, and immunohistochemical examination. Among the 16 frozen muscle specimens, 6 (37%) showed normal muscle, while 10 (63%) showed the presence of atrophic fibers. The 6 cases with normal muscle showed no abnormality in fiber type distribution. Six patients showed predominance of type 1 muscle fibers. None of the specimens had myofibroblasts; 4 of 16 specimens had mast cells, and 9 of 16 specimens showed the presence of platelet derived growth factor–positive cells. Features of myofibroblasts (the presence of basal lamina, intercellular junctions, or pinocytic vesicles) were not identified in any specimen on electron microscopy. The histopathological, electron microscopic, and immunohistochemical findings, in particular the absence of myofibroblasts, in tissue samples obtained from patients with RLD, do not support the assumption of abnormality in soft tissues as a secondary driver of deformity in RLD. This study provides a preliminary insight into a possible role of soft tissues in the development of the deformity in RLD. [ABSTRACT FROM AUTHOR]

The Baller-Gerold syndrome is a rare syndrome with very few cases published in literature. Craniosynostosis and radial aplasia are striking features, easy to diagnose. However, there are many differential diagnoses. Often, the question raised is whether the Baller-Gerald syndrome is a distinct entity. We report a patient with findings of craniosynostosis and radial aplasia consistent with the diagnosis of the Baller-Gerold syndrome. Genotypic heterogeneity could possibly underlie the phenotypic variability exhibited by these cases.

the baller-gerold syndrome is a rare syndrome with very few cases published in literature. craniosynostosis and radial aplasia are striking features, easy to diagnose. however, there are many differential diagnoses. often, the question raised is whether the baller-gerald syndrome is a distinct entity. we report a patient with findings of craniosynostosis and radial aplasia consistent with the diagnosis of the baller-gerold syndrome. genotypic heterogeneity could possibly underlie the phenotypic variability exhibited by these cases.

The risk and consequences of an elbow or a wrist contracture are lower during a forearm lengthening than during a lower limb lengthening. This kind of complication can mostly be avoided by an active and intensive regimen of physiotherapy. However, there are some challenges to deal with in treating the disorder multiple exostoses and the radial club hand, including the lack of consensus on the best treatment for multiple exostoses. However, it is important to realize that the evolution of multiple exostoses can lead to a radial head dislocation which will damage the pronation and the supination range of motion. As this motion can be poor even without a radial head dislocation as a result of the radius being longer than the ulna, an interesting technique can be to lengthen the ulna to limit this phenomenon. In radial club hand, the main problem is the deviation of the hand requiring a centralization. The best treatment for centralization of the hand is to do a progressive correction with an external fixator. Thereafter, it is possible to lengthen the forearm, but this indication is mainly cosmetic in the unilateral radial club hand. [ABSTRACT FROM AUTHOR]

Objectives: Brachial plexus block in combination with general anaesthesia is used for surgical correction of radial club hand. We carried out this retrospective study to assess the feasibility and efficacy of brachial plexus block in children with radial club hand. Intraoperative and postoperative analgesic requirements were the primary outcome measures analysed. Methodology: We conducted a retrospective audit and collected data on the intraoperative and postoperative analgesic requirements in 24 patients anaesthetised for orthopedic treatment of this congenital deformity in our children's hospital over a period of 5 years. Results: Transarterial axillary block was administered to 8 patients; nerve stimulator guided supraclavicular block was used in 4 patients; and 12 were administered anesthesia without administration of brachial plexus block. Intraoperative additional supplementation of intravenous opioid was required in 12.5%, 50% and 75% of patients administered axillary block, supraclavicular block and no block (only general anesthesia) respectively. The mean time interval for administration of first dose of analgesic in postoperative period was 4.5, 3.6 and 0.75 hours respectively. Permanent diaphragmatic paralysis was reported in one patient with supraclavicular block. No complication was reported in the group with axillary block. Conclusion: Supplementation of general anesthesia with transarterial axillary brachial plexus block provides adequate perioperative analgesia in children with radial club hand operated for centralisation of ulna. [ABSTRACT FROM AUTHOR]

VACTERL association is a useful acronym for a condition characterised by the sporadic, non-random association of specific birth defects of multiple organ systems.We present one such case which had congenital abnormalities of renal,skeletal and cardiac system.

Background: In children with hypoplasia or aplasia of the radius (radial longitudinal deficiency) manual activity limitations may be caused by several factors; a short and bowed forearm, radial deviation of the wrist, a non-functional or absent thumb, limited range of motion in the fingers and impaired grip strength. The present study investigates the relation between these variables and activity and participation in children with radial dysplasia. Methods: Twenty children, age 4-17 years, with radial longitudinal dysplasia Bayne type II-IV were examined with focus on the International Classification of Functioning and Health, version for Children and Youth (ICF-CY) context. Body function/structure was evaluated by measures of range of motion, grip strength, sensibility and radiographic parameters. Activity was examined by Box and Block Test and Assisting Hand Assessment (AHA). Participation was assessed by Children's Hand-use Experience Questionnaire (CHEQ). Statistical correlations between assessments of body function/ structure and activity as well as participation were examined. Results: The mean total active motion of wrist (49.6°) and digits (447°) were less than norms. The mean hand forearm angle was 34° radially. Ulnar length ranged from 40 to 80% of age-related norms. Grip strength (mean 2.7 kg) and Box and Block Test (mean 33.8 blocks/minute) were considerably lower than for age-related norms. The mean score for the AHA was 55.9 and for CHEQ Grasp efficiency 69.3. The AHA had significant relationship with the total range of motion of digits (p = 0.042). Self-experienced time of performance (CHEQ Time) had significant relationship with total active motion of wrist (p = 0.043). Hand forearm angle did not show any significant relationship with Box and Block Test, AHA or CHEQ. Conclusion: In radial longitudinal deficiency total range of motion of digits and wrist may be of more cardinal importance to the child's activity and participation than the angulation of the wrist. [ABSTRACT FROM AUTHOR]

We compared the radiological and functional outcomes in patients with radial longitudinal deficiency treated surgically with those managed nonoperatively and correlated the ease of performance of activities with radiological and functional parameters in the two groups. A retrospective analysis was done in 446 type 3 and 4 hands (modified Bayne classification) over 20 years comparing the subjective assessment, objective and functional scores in nonoperatively managed hands (Group 1; n = 137) to hands managed with centralization or radialization (Group 2; n = 309). The subjective, objective and functional measures were significantly better in Group 2. Improved alignment, finger and wrist range of motion and grip strength were related to improvement in functional score. Surgical treatment of radial longitudinal deficiency provides improvement in cosmesis, function and ease of performance of activities when compared with nonoperatively managed hands. [ABSTRACT FROM PUBLISHER]

In patients with radial club hand (RCH), there is absence of the radius and severe hypoplasia of the thumb, so both wrist stabilization and thumb reconstruction are essential. When wrist stabilization is performed, recurrence of angulation is a problem. When thumb reconstruction is done, preservation of at least one major dorsal vein is important to prevent necrosis of the reconstructed thumb. We executed a strategic approach to RCH, which aimed to prevent both recurrence of angulation and necrosis of the reconstructed thumb. [ABSTRACT FROM AUTHOR]

Radial 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. Three 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. The 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. The 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. [ABSTRACT FROM AUTHOR]

Preliminary soft tissue distraction using an external fixator before centralisation and tendon transfer of the flexor and extensor carpi ulnaris to the little finger metacarpal was used to treat Bayne’s type III and IV deformities in 18 hands of 14 patients with radial club hands. Treatment with external fixator was started at a mean age of 8 (range 3–30) months. In 16 of 18 hands, the surgical treatment was completed before 10 months of age. Adequate soft tissue stretching was achieved before centralisation using fractional distraction with the external fixator in the majority of hands. After an average follow-up period of 31 months, there were seven good, eight satisfactory and one unsatisfactory result. [ABSTRACT FROM PUBLISHER]

Background: Radial club hand is a congenital deformity in which the radius is absent, the metacarpophylangeal and interphalayngeal joints are fused and muscles arising from the radius are absent. This can alter the motor response to peripheral nerve stimulation. The classical motor response to median nerve stimulation (contractions of the fingers) cannot be elicited, hence fine twitches in the hand, at the wrist or in the surgical area or pronation–supination at a current of 0.5 mA were taken as endpoints. The aim of this study was to assess the feasibility of achieving successful infraclavicular coracoid approach block in the intra- and postoperative periods when ideal responses to electrical nerve stimulation are absent because of congenital anomalies. Methods: Thirty-five patients aged 8 months to 2 years, weighing 7–12 kg were selected and the blocks were performed under light general anesthesia. Fentanyl 2 μg·kg−1was used as rescue analgesic in case of block failure. Postoperatively CHEOPS pain score was recorded at h1, h4 h6, h8, and h10 postoperatively. Tramadol 2 mg·kg−1 was given in case of inadequate pain relief (CHEOPS >4). Results: In no patient could we elicit the finger flexion response which is typical of median nerve stimulation. Intraoperatively, one out of 35 children (3.3%) showed reaction to pain. In the postoperative period two out of 35 (6%) children had high pain scores and were given tramadol. Analgesia lasted for 6.5 ± 1.50 h. Conclusions: Despite the limitation of absence of an ideal response to nerve stimulation in radial club hand, endpoints such as fine twitches in the hand, at the wrist or in the surgical area or pronation–supination were sufficient to achieve a successful block. This block gives satisfactory intra- and postoperative analgesia. [ABSTRACT FROM AUTHOR]

Purpose: For treatment evaluation of children with radius deficiencies (RDs), standardized assessment of hand function in performing activities is required. Instruments to measure hand function have been developed for other diagnoses. The current study aimed to find additional evidence for validity, reliability, and usefulness of these instruments for children with RDs. Methods: In this study, 20 children with RDs (aged 4–12 years) participated; 16 were boys, and 13 children were unilaterally affected. Children were assessed using the Assisting Hand Assessment, the Unilateral Below Elbow Test, the Prosthetic Upper Extremity Functional Index, and ABILHAND-Kids. Construct and convergent validity of the instruments were studied focusing on predefined hypotheses and relationships with other instruments and the therapist’s global assessment. Test-retest reliability was assessed in 10 children by means of the intraclass correlation coefficients and the smallest detectable differences. Results: For children with RDs, the Assisting Hand Assessment and the Prosthetic Upper Extremity Functional Index appeared to be the most valid function test and questionnaire according to the relationships found with type of RD, functional hand grips, and the therapist’s global assessment of hand function. Regarding test-retest reliability, intraclass correlation coefficients ranged from 0.82 to 0.91, and smallest detectable differences were acceptably small. Conclusions: The current results contribute to the evidence that the instruments, especially the Assisting Hand Assessment and the Prosthetic Upper Extremity Functional Index, provide valid and reliable results in children with RDs. Type of study/level of evidence: Diagnostic I. [Copyright &y& Elsevier]