Club Hand Research Articles

Ulnar longitudinal deficiency: a rare case report and review

<p class="abstract">Ulnar hemimelia is a rare postaxial partial or complete longitudinal deficiency of ulna. It has an estimated incidence of 1/100,000-150,000 live births, with a male to female ratio of 3:2. There is usually ulnar deviation of hand and shortening of forearm. Radial head subluxation and fixed flexion deformity of the hand may be associated with it. Complex carpal, metacarpal, and digital abnormalities including absence of triquetrum, capitate and three fingered hand (tridactyly) are additional findings commonly found in association. Here, we present a case of a 17-year-old female with left sided ulnar club hand due to isolated partial ulnar aplasia.</p>

Expanded Goldenhar Complex with Multiple Rare Syndromic Associations

Goldenhar syndrome (GS) is the most severe form of the oculoauriculovertebral spectrum, a developmental disorder primarily involving the structures derived from the first and second branchial arches, intervening first pharyngeal pouch and branchial cleft characterized by microtia, facial asymmetry, ocular abnormalities, and abnormalities of the vertebral column. A multifactorial etiology involving genetic and environmental factors has been proposed and the majority of the cases are sporadic in occurrence. The clinical phenotype of GS is highly variable and multiple associated anomalies of various organ systems have been reported in the literature. The term “Expanded Goldenhar Complex” has been used when there are associated unusual extrafacial abnormalities involving major organ systems such as the central nervous system, cardiovascular system, and respiratory system. We report a case of Expanded Goldenhar Complex with multiple anomalies, some of which are very rarely reported associations. A 5-year-old boy presented with right-sided hemifacial microsomia, microtia, preauricular and malar skin tags, bilateral radial club hands, and scoliosis since birth. Imaging revealed additional abnormalities including aural atresia, unilateral pulmonary agenesis, dextrocardia, vertebral segmentation defects, left renal ectopia, and right undescended testis. Surgical corrections were undertaken for the right radial club hand and undescended testis and he is awaiting surgery for the left club hand. Complete imaging evaluation of children with dysmorphic facies and limb abnormalities helps in identifying the various associated organ anomalies and to arrive at an accurate syndromic diagnosis. These patients have to be managed by a multidisciplinary team of clinicians on a case-to-case basis and have a good prognosis if there are no major cardiac defects or intellectual disability.

Surgical and Protocolized Management of Distal Radius Nonunion

Abstract Introduction Nonunion of the distal radius is an extremely rare and serious complication with unpredictable outcomes. The aim of the present study is to analyze the radiological and functional results after a protocolized surgical treatment. Material and Methods A retrospective review of case series of patients with diagnosis of nonunion of the distal radius surgically treated from 2010 to 2016. The diagnosis of nonunion was made at 6 months without signs of consolidation. According to computed tomography (CT) scan images, we propose a classification of this complication as type I: no deformity or mild deformity, with subchondral metaphyseal bone stock beneath lunate facet greater than 10 mm, with no degenerative changes at the distal radioulnar joint (DRUJ); type II: moderate deformity or degenerative changes in the DRUJ, distinguishing between IIA and IIB based on the subchondral metaphyseal bone stock; and type III: degenerative changes at the midcarpal joint. In all cases, a reconstructive technique consisting of volar locking plate osteosynthesis with autologous tailored iliac crest graft was performed, hence none of the cases were classified as type III. Results In total, six cases were included. Mean follow-up: 58 months (range: 30–108 months). Consolidation was achieved in a mean period of 5 months (range: 3–9 months). Average active range of motion: flexion: 54° (range: 10°–80°); extension: 40° (range: 10°–85°); supination: 82.5° (range: 75°–85°); pronation 80° (range: 55–85°); radial deviation: 15° (range: 5°–20°); and ulnar deviation 15° (range: 5–20°). Mean strength: 24.15 Kg (6.9–35 Kg). The average score on the Visual Analog Scale (VAS) at rest was of 0.6 (0–2), and with movement, it was of 1.8 (0–4). The mean postoperative score on the Disabilities of the Arm, Shoulder and Hand (DASH) questionnaire was of 18.48 (range: 0–50.8). Average radial height: 9.65 mm (range: 7–12 mm); average radial angulation: 17° (10°–20°); volar tilt: 8.36° (range: 0.2°–21°); average ulnar variance: 1.52 mm (range: 0–3.5 mm). Complications: persistent nonunion, superficial wound infection, acquired residual ulnar club hand deformity, radiocarpal arthritis, and midcarpal arthritis. Nevertheless, no salvage techniques were needed or requested during the follow-up, with all patients in an active work situation or living an active life in complete normality. Conclusions The protocolized surgical treatment of pseudoarthrosis of the the distal radius, based on our proposed classification, offers good functional outcomes in the long-term follow-up.

Radial longitudinal deficiencies in children

Radial longitudinal deficiencies pose a unique challenge in children's orthopaedic surgery: a deformity at birth that can be the only visible sign of serious underlying health conditions. Treatment can be intensive and prolonged but children often cope extremely well with compensations for reduced upper limb function. The aim of this article is to describe essential knowledge of the condition, its associations and treatments. We provide a clinical and diagnostic framework to enable appropriate and timely investigations, management and referral where appropriate.

Prevalence of congenital limb defects in Uttarakhand state in India – A hospital-based retrospective cross-sectional study

BackgroundCongenital limb defects (CLD) occur when normal development of limbs is hampered during the intrauterine period. There is a paucity of literature regarding the prevalence and pattern of various congenital limb defects in India. The aim of this study is to assess the prevalence and pattern of various CLD using Swanson classification in the state of Uttarakhand, India. MethodsRetrospective study over a 6-year period was done. The prevalence of CLD was calculated by determining the live births from birth rate and total population from the 2011 census of the Government of India. ResultsA total of 316 cases with a mean age of 3.2 ± 2.3 years (1 day–12 years) were included. Radial clubhand (n = 9, prevalence = 0.4 per 10,000 live births) was the most common CLD in upper limb. Idiopathic clubfoot (CTEV) (n = 158, prevalence = 7 per 10,000 live births) was the most common CLD in lower limb. Failure of formation (n = 12) was the most common defect in the lower limb whereas failure of differentiation (n = 16) was common in the upper limb. DiscussionThis is the first study from any part of India depicting the prevalence of CLD, which can be used to formulate public health policies for early diagnosis and management of the same. ConclusionThe prevalence of CLD is 14 per 10,000 live births in Uttarakhand, India, which are comparable to various studies published from different parts of the world. Males are commonly involved with radial clubhand being common in the upper limb and CTEV being common in the lower limb.

A novel technique of ulna strut grafting for post-septic radial clubhand deformity: Case series of 4 patients with review of literature

BackgroundThough distal end radius fractures are one of the commonest fractures to occur, their nonunions are extremely uncommon. Out of these, post-septic defect nonunions with acquired radial clubhand deformity are even more rare and pose unique problems in management. We present a case series of 4 patients of post-septic radius nonunion with acquired radial clubhand deformity successfully treated with a novel technique of using Ulna Strut grafting for radius defect after ulnar shortening. All 4 patients have a good functional and cosmetic outcomes with radiological union at the cost of minimal limb length discrepancy of the forearm. MethodsThis is a case series of 4 patients with a retrospective study design and study duration of 3 years from August 2016 till March 2019.4 patients presenting to us with post-septic defect nonunions of radius with an acquired radial clubhand deformity were selected with a mean age of 19.75 years which included 2 males and 2 females. ResultsThe mean time for radiological union was 3.125 months. The average range of motion at the wrist joint was 35° of flexion and 50° of extension with average protonation being 47.5° and supination being 75°.There were no subsequent complications reported in these patients except for a minimal limb length discrepancy with satisfactory functional outcomes in all the patients. ConclusionThe use of Ulnar shortening and Ulna strut grafting for the treatment of defect nonunions of radius with acquired clubhand deformity yielded predictable results. It is a simple procedure that does not require additional training, with no additional morbidity of iliac crest or fibula bone grafting and had a minimum complication rate. The technique looks promising in the future, though a study on a larger population would further strengthen the predictability of this unique technique.

Radial club hand treated with soft-tissue distraction and subsequent pin stabilization: mid- to long-term results.

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.

Appearance of congenital hand anomalies

Background and objective:Impact of appearance of congenital hand anomalies has not previously been reported. The purpose of this study was to describe the common perception about how different congenitally malformed hands look.Methods:We developed a questionnaire in a game format to evaluate the appearance of different hands. Altogether 1450 (954 females) 4- to 84-year-old residents (296 children) of two European and one Asian (n = 102) country were asked to rate the appearance of different looking hands on a five-point pictorial Likert-type scale. Standardized photographs of the dorsal aspect of 17 different congenitally malformed non-operated hands and a normal hand were presented to respondents. Significance of age, gender, nationality, and profession of the respondents was assessed.Results:The respondents’ ranking order of the hands was nearly consistent. The normal hand (mean = 4.43, standard deviation = 0.85, Md = 5) and clinodactyly (mean = 4.37, standard deviation = 0.86, Md = 5) were perceived to have the best appearance. Symbrachydactyly (mean = 1.42, standard deviation = 0.68, Md = 1) and radial club hand (mean = 1.40, standard deviation = 0.68, Md = 1) received the lowest scores. Adults rated the appearance of hands higher than children regarding 14 hands, females higher than men regarding 15 hands, and Europeans higher than Asians in 4 hands (p < 0.05, respectively). Europeans rated four-finger hand (mean = 3.21, standard deviation = 1.18, Md = 3) better looking than six-finger hand (mean = 2.92, standard deviation = 1.18, Md = 3, p < 0.005), whereas Asians gave higher scores to six-finger hand (mean = 2.66, standard deviation = 1.26, Md = 3) compared to four-finger hand (mean = 2.51, standard deviation = 1.14, Md = 2). Medical doctors and nurses gave higher scores compared to the other profession groups, school children, and high school students in five hands (p < 0.05).Conclusions:A normal hand is perceived distinctly better looking than most congenitally different hands. Different malformations’ appearance was ranked very coherently in the same order despite of participants’ age, gender, nationality, or profession. Asians seem to prefer an additional digit to a four-finger hand.

Outcome of Centralization and Ulnarization of the Carpus with Ulnar Shortening Osteotomy on Functioning in Children with Radial Club Hand.

Radial club hand (RCH) is characterized by a wide array of hand and forearm anomalies. Various treatment approaches have been described depending upon the stages of RCH. The major drawback of these studies is that the effectiveness of these interventions was reported on clinical and radiological outcomes. With the increasing focus on patient-centered care nowadays, we wanted to identify the components associated with functioning and evaluate the effectiveness of two surgical procedures on functional outcomes using the International Classification of Functioning, Disability and Health (ICF)-based tools. We identified 14 children from our records (nine boys, five girls) with a mean age of 5.6years, classified as Bayne types III-IV and classified them into two groups; those who were operated by centralization (group 1) and ulnarization of the carpus with ulnar shortening osteotomy procedure (group 2). The outcomes were evaluated by the brief ICF core set for the child and youth with cerebral palsy up to the age of 5 and the brief ICF core set for hand conditions for a period of 1year after surgery. The results showed that both the operative techniques showed improvement in the structure component (s730-structure of upper extremity). ICF categories of d445-hand and arm use, d530-toileting, and d880-engagement in play showed a change in frequencies of more than 40% after surgery and were maintained till follow-up. However, categories related to muscle power functions (b730), muscle tone (b735), fine hand use (d440), hand and arm use (d445) and engagement in play (d880) showed no significant improvement (p > 0.05). There were no differences between both the surgical procedures in improving the outcomes (p > 0.05). We conclude that surgical techniques may be more appropriate to improve the cosmetic or structural appearance of the upper extremity than functioning.

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.&#x0D; 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.&#x0D; 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.&#x0D; 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%).&#x0D; 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.

Bilateral Radial Club Hands at the Komfo Anokye Teaching Hospital, Kumasi, Ghana: A Case Report.

Radial Club Hand (RCH) is a rare and complex congenital disorder of the pre-axial border of the upper extremity. It is a congenital longitudinal radial ray deficiency which presents as radial deviation of the hand and distal forearm in the shape of a golf club 1, 2. Radial ray deficiency (the most common type of longitudinal failure of formation) is a spectrum of malformations affecting the structures of the radial side of the forearm, including hypoplasia of the bones, joints, muscles, tendons, ligaments, nerves and blood vessels. The thumb dysfunction, wrist instability and short upper extremity that accompany this condition can cause significant functional impairment particularly in patients with bilateral involvement. Thus, the performance of activities of daily living such as accomplishing personal hygiene, fastening buttons and zippers becomes challenging 3.

Radial club hand management with initial gradual distraction by Ilizarov

Radial club hand management with initial gradual distraction by Ilizarov

Clinical spectrum of congenital tibial hemimelia in 35 limbs of 24 patients: A single center observational study from India

PurposeConsidering the paucity of reports on large series of patients with tibial hemimelia, we assessed the clinical spectrum of this rare congenital disorder in patients seen at a single Indian center over 10 years. MethodsRetrospective review of medical records of patients seen at single center in 10 years. ResultsThirty-five cases of TH, mostly Jones types Ia (18) and II (10), were diagnosed in 24 patients (13 had unilateral TH). Associated foot deformities included equinovarus (22), varus foot (10), absence of the medial row of toes (5) and polydactyly (3). Upper limbs anomalies included split-hand deformity (five patients) and radial club hand (two patients). Nine limbs of seven patients were surgically reconstructed. Modified orthosis was provided to seven patients, custom designed prosthesis fitment in six and amputation with prosthesis fitment in one. Patients presenting at adolescence or later were habituated to their deformity for indoor ambulation; families declined amputation. ConclusionReports of more TH cases will provide input to researchers to consider comprehensive rehabilitation for enhancing indoor and community ambulation.

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.&#x0D; Aim. We evaluated the results of CRCH treatment using microsurgical technique and external fixation.&#x0D; 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.&#x0D; 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.&#x0D; 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.

Clinical and functional results of radial club hand with centralization and pollicization using the second metacarpus: A clinical case series

IntroductionRadial 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. MethodsThere 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. ResultsThe 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. ConclusionEarly centralization and pollicization using second or third metacarpal bone can significantly restore the range of motion and function in patients with RCH.

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.

"Radial Club Hand, a Rare Congenital Abnormality: Report of Two Cases"

Congenital radial deficiency is an extremely rare congenital anomaly and its presentation may vary from mild hypoplasia to complete absence of radius. Club hand deformities are classified into two main categories radial and ulnar. Ulnar club hand is much less frequent than radial club hand. It is also known as radial club hand or radial dysplasia. We report two cases of rare congenital abnormality which can be helpful to the literature.

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.

Prenatal diagnosis of complex phenotype in a 13-week-old fetus with an interstitial multigene deletion 20q13.13.-q13.2 by chromosomal microarray

We report the first trimester three-dimensional ultrasonographic findings in a 13-week-old fetus with complex phenotype and a de novo 4.7 Mb multigene deletion encompassing chromosome region 20q13.13-q13.2 detected by chromosomal microarray. Fetal sonography detected radial-ray anomalies in the form of bilateral absence of thumbs and the left club hand deformity. The presence of single atrioventricular canal instead of the atrial septal defect typical for Holt-Oram syndrome pointed us to rather suspect the SALL4 related diseases. Central nervous system anomaly in the form of enlarged lateral brain ventricles with choroid plexus shifted backwards was not previously reported as a part of SALL4 related disorders. The pregnancy was terminated at 14 + 3 weeks of pregnancy and the autopsy confirmed ultrasonographic findings. Deleted region included 38 genes, where only SALL4, ADNP and KCNB1 heterozygote pathogenic variants were described to be cause of syndromic forms. Radial ray anomalies are common part of clinical picture of SALL4 related disorders. Despite the lack of prenatally described cases, we hypothesized that maldevelopment of lateral brain ventriculomegaly could be very early sonographic sign of disturbed ADNP expression causing Helsmoortel-Van der Aa syndrome, but in some extent also of KCNB1 related early-onset epileptic encephalopathy. Furthermore, the possible dosage-dependent influence of recessive genes located in this region cannot be also excluded. The use of genome-wide technologies enables the detection of subtle chromosomal imbalances and more precise familial genetic counseling regarding actual and future pregnancies.