Bilateral Dorsal Nail Curvature of the Thumb Distal Phalanx - A Case Report.

Congenital dorsal curvature of the distal phalanx has been previously described as 'reverse Kirner' or 'ski-jump' deformity. This report describes bilateral occurrence in the thumbs. A 13-year-old male presented with difficulty caring for his thumbnails and in picking up small objects. Examination showed dorsal curvature of the distal phalanges of both thumbs, with greater curvature of the right side. Radiographs showed wedge-shaped epiphyses and dorsal curvature without coronal plane deviation of the distal phalanges. There was objective and subjective decrease in function associated with lateral pinch and tripod grasp. The reported aetiopathogenesis for Kirner deformity cannot explain the observed dorsal curvature. The bilateral nature makes a secondary physeal cause unlikely and suggests an embryologic basis. Due to the noticeable deficits in function, operative intervention may be warranted. Level of Evidence: Level V (Therapeutic).

Spontaneous improvement of bilateral clinodactyly of the little finger with trapezoidal phalanx: a case report with a 15-year follow-up.

Clinodactyly can be produced by a longitudinal epiphyseal bracket that generates either a 'delta' or 'trapezoidal' phalanx. We present a case with a 15-year follow-up of bilateral clinodactyly of the little finger, to emphasize a 'wait-and-see' approach as self-remodelling of his phalanges occurred during growth.

Ulnar dimelia - a review of 24 cases.

Ulnar dimelia is a very rare unilateral congenital upper limb anomaly (CULA) affecting the whole extremity. Treatment remains difficult because of the complexity and multi-level involvement. Twenty-four cases with duplicated ulna, absent radius and polydactyly from seven European centres were reviewed according to a structured list of parameters. At first consultation, median age 8 months (1-178), the shoulder movement was good in 17 patients or poor in six, and the median passive elbow range of motion was 20° (0°-90°). The resting wrist position was flexed in 22/24 patients. Following stretching and splinting, elbow surgery included resection of the lateral proximal ulna in 11 patients and muscle transfers in six to improve passive movement and increase active elbow motion, respectively. Tendon transfers were performed in eight wrists and a pollicization or pseudo-pollicization in 23 patients. Overall, patients demonstrate acceptable function postoperatively. Guidelines for treatment of this severe CULA are presented.Level of evidence: IV.

Donor Foot Morbidity Following Nonvascularized Toe Phalanx Transfer Utilizing a New Reconstruction Technique.

BACKGROUNDS: Nonvascularized toe phalanx transfer is an accepted surgical option for short and hypoplastic digits in congenital hand abnormalities. However, one of the criticisms of this technique is the donor site morbidity. The purpose of this study was to evaluate donor foot morbidity after nonvascularized toe phalanx transfer using a new donor site reconstruction technique. METHODS: We retrospectively reviewed 116 nonvascularized toe phalanx transfers in 69 children between 2001 and 2020 in whom the donor foot was reconstructed with a new technique using iliac osteochondral bone graft with periosteum. Feet treated with an isolated donor proximal phalanx of the fourth toe were selected and morbidity was assessed both subjectively and objectively at a minimum of 2 years after surgery. Motion, stability, and alignment of the metatarsophalangeal joint were clinically evaluated. The relative length of the fourth toe to the third toe was measured on a roentgenogram. The satisfaction of the parents for overall function and appearance was evaluated using a visual analog scale. RESULTS: Ninety-four operated feet in 65 patients, including 43 boys and 22 girls, were included. The right foot was evaluated in 52 patients and the left foot in 42 patients. The mean age at operation was 2 years and the mean follow-up period was 7.6 years. Motion at the metatarsophalangeal joint was good at 69% with an average extension of 45 degrees and flexion of 25 degrees. Stability and alignment were good at 95% and 84%, respectively. Only 4 toes had gross instability and 4 toes with poor alignment required revision surgery. Sixty-two toes (66%) maintained proportional length and 9 toes were graded as short. Parental satisfaction was high for appearance as well as function. CONCLUSIONS: This newly described technique of using iliac osteochondral bone graft with periosteum to reconstruct toe phalanx donors provided satisfactory results. The function and appearance of the donor foot after a nonvascularized toe phalanx transfer was well preserved. LEVEL OF EVIDENCE: Level IV; therapeutic.

Recent Advancements in the Diagnosis and Treatment of Congenital Hand Differences.

Congenital hand and upper limb differences include a wide spectrum of structural abnormalities that affect functional, appearance, and psychosocial domains of affected children. Ongoing advances in the understanding and treatment of these differences continue to shape management. Over the past 10 years, new developments have been made in areas of molecular genetics, noninvasive treatments, surgical techniques, and outcome measures in several commonly seen congenital hand differences. Applying these advances in knowledge and management of congenital hand differences will enable surgeons to achieve the best outcomes possible for these children.

Camptodactyly: From Embryological Basis to Surgical Treatment.

Camptodactyly is a relatively rare hand deformity presenting as the proximal interphalangeal joint's nontraumatic and progressive flexion contracture. Most cases are limited to the fifth finger. The severity and type of camptodactyly should be considered to optimize treatment. Since many structures at the finger base can be involved in the pathogenesis of the deformity, surgical treatment for this particular type of deformity is challenging. This paper aims to bring insight into camptodactyly's pathogenesis and treatment options. We discuss the indication and pitfalls of surgical treatment options for particular camptodactyly types and present a case of a fourteen-year-old boy who was admitted to our department with proximal interphalangeal joint flexion contracture of the left fifth digit.

The unsolved problem of radial longitudinal dysplasia: how can we reliably prevent recurrence, preserve growth and optimize function?

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.

Long-Term Follow-Up of Ulnar Growth in Radial Dysplasia Treated by a Combination of Distraction and Radialization.

PURPOSE: To study the long-term results of radial club hand, regarding ulna growth, radial angulation, and volar subluxation using a 2-stage treatment protocol. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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. TYPE OF STUDY/LEVEL OF EVIDENCE: Therapeutic IV.

Factors affecting hand cosmesis and the aesthetic impact of surgery on congenital hand differences in Finland.

We assessed the appearance and cosmetic impact of surgery in congenitally different hands in Finland. A questionnaire was sent to 1165 respondents (786 female) with a mean age of 33 years (range 3-84). Participants were shown nine image pairs and seven pairs of pre- and postoperative images twice in a random order and asked to choose the more cosmetically pleasing one. We found that the appearance and number of fingers had an important aesthetic role, with higher number and more normal appearing digits consistently scoring higher than its counterpart (range 59-99%). Postoperative appearances were perceived as better than preoperative ones in syndactyly (98%), thumb duplication (92%), cleft hand (93%) and radial dysplasia (99%). Toe transfer and pollicization had little impact on cosmesis. This study demonstrated that surgery could improve cosmesis in congenitally different hands and overall, most respondents prefer an appearance that is as close as possible to normality. Level of evidence: IV.

A new incision in the correction of radial longitudinal deficiency: The double Y sliding flap approach.

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.

Mirror hand-ulnar dimelia: a single centre experience with 13 patients.

Mirror hand-ulnar dimelia (MHUD) is rare congenital disorder with a wide range of clinical and radiographic features. The current nomenclature is confusing and the existing classification systems focus mainly on morphology without providing much guidance for treatment. The goal of this study was to review the clinical and radiological features in 13 children with MHUD that were treated at a single institution. Our findings support the hypothesis that MHUD typically involves the entire upper limb, and the treatment plan should consider the predictors of function at each limb segment. A logical and comprehensive management algorithm is proposed.Level of evidence: IV.

Bilobed Flap in Hand Clinodactyly Reconstruction: Technique Description and Result Appraisal / Retalho bilobado na reconstrução das clinodactilias da mão: Descrição da técnica e avaliação do resultado

Abstract Objective Clinodactyly is a congenital hand deformity that is characterized by coronal angular deviation and may occur in thumbs or fingers. Surgical treatment is indicated for severe angulations. Among the described techniques, one of the options consists of bone alignment by wedge-shaped addition osteotomy of the anomalous phalanx. Such alignment maneuver creates a problem in skin cover, along with soft-tissue tension at the concave aspect of the deformity. Hence, some sort of skin flap is required for the adequate operative wound closure. We aim to demonstrate the aforementioned technique and to assess the results of bilobed flap in the treatment of hand clinodactyly. Methods Retrospective study conducted between January 2008 and January 2015. Five patients were included in the study, including nine operated digits. Surgical indication consisted of angular deviations ˃ 30o. Neither patients with thumb deformities nor those with deformities associated to syndromes were excluded from the study. We assessed the functional and cosmetic outcomes of the technique, as well as complications and the satisfaction rates of the family. Results All patients had satisfactory functional and cosmetic results, with a mean skin healing of 18.6 days. Among nine operated digits, only one of the patients presented vascular compromise at the distal portion of the first flap lobe, albeit without necrosis or the need for any additional procedure. Patients were followed up on a minimum of 12-month interval. No deformity recurred during the observation period. Conclusion Bilobed flap for the treatment of hand clinodactyly is a good option for skin cover after the osteotomy.

Resumo Objetivo A clinodactilia é uma deformidade congênita da mão, caracterizada por desvio angular coronal, podendo ocorrer em polegares ou dedos. O tratamento cirúrgico é indicado para angulações severas. Dentre as técnicas descritas, uma das opções consiste no alinhamento ósseo, através de osteotomia em cunha de adição da falange anômala. Essa manobra de alinhamento gera uma dificuldade de cobertura cutânea, juntamente com a tensão das partes moles no lado côncavo da deformidade. Sendo assim, algum tipo de retalho cutâneo é necessário para o fechamento adequado da ferida cirúrgica. Objetivamos demonstrar a técnica citada e avaliar os resultados do retalho bilobado no tratamento das clinodactilias da mão. Métodos Estudo retrospectivo entre janeiro de 2008 e janeiro de 2015. Foram incluídos cinco pacientes no estudo, nove dedos foram submetidos à cirurgia. Tiveram indicação cirúrgica os pacientes com desvios angulares nos dedos acima de 30 graus. Não foram excluídos do estudo os pacientes com deformidades no polegar, nem aqueles com deformidades associadas a síndromes. Foram avaliados os resultados funcionais e estéticos com o uso desta técnica, assim como as complicações e o grau de satisfação dos familiares. Resultados Todos os pacientes apresentaram resultados funcionais e estéticos satisfatórios, com cicatrização cutânea média de 18,6 dias. Entre os nove dedos submetidos à cirurgia, apenas um dos pacientes apresentou comprometimento vascular na porção distal do primeiro lobo do retalho, porém sem necrose ou necessidade de qualquer procedimento adicional. Os pacientes foram acompanhados durante um intervalo mínimo de 12 meses. Nenhuma deformidade ocorreu durante o período de observação. Conclusão O uso do retalho bilobado no tratamento das clinodactilias da mão é uma boa opção para cobertura cutânea após a realização de osteotomia.

[Developments in the Diagnosis and Therapeutic Strategy of the Apert Hand]. / Entwicklungen in der Diagnostik und Planung der Therapie bei der Apert-Hand.

The necessity for early surgical correction of Apert hands for the overall child development has been highlighted repeatedly in older literature. Nevertheless, uncertainties regarding the time and the scale of the initial surgical treatment still remain. While in former times there were no regular follow-ups after the syndactyly release, we now know that during growth bony changes will develop in the Apert hand requiring regular check-ups and, in some cases, revision surgeries. Affected parents need comprehensive clarification about a clear and time-efficient therapeutic concept. This review article describes our actual concept treating Apert hands.

Congenital Disorders of the Pediatric Thumb.

¼: Surgical timing for pediatric trigger thumb treatment is controversial for numerous reasons including the potential for spontaneous resolution, the possibility of bilateral involvement, and anesthesia concerns regarding the developing brain. Hence, a reasonable approach is to delay the surgical procedure until the patient is ≥3 years of age. ¼: Preaxial polydactyly is usually unilateral and sporadic, with the most common reconstruction method consisting of excision of the diminutive thumb with preservation and soft-tissue reconstruction of the dominant thumb. The surgical procedure is typically performed around the patient age of 1 year to decrease the risks of anesthesia but allow reconstruction prior to the development of a tip-to-tip pinch. ¼: Triphalangeal thumb and thumb hypoplasia are often found in the setting of systemic anomalies such as Holt-Oram syndrome, thrombocytopenia absent radius syndrome, Fanconi anemia, VACTERL (vertebral anomalies, anal atresia, cardiac anomalies, tracheoesophageal fistula, renal defects, and limb anomalies), and/or Blackfan-Diamond anemia. As such, patients should receive adequate workup for these entities. A surgical procedure should be performed only once patients have been medically cleared. ¼: The status of the carpometacarpal joint in thumb hypoplasia determines whether reconstruction with first web space deepening, collateral ligament stabilization, and opponensplasty compared with index pollicization is performed.

Surgical Treatment of Ulnar Cleft Hand with All Fingers - A Report of Two Patients.

An ulnar cleft hand is a rare congenital deformity presenting with a cleft between the ring and little finger. It may be associated with the absence of a finger. The goals of treatment are cleft closure, increasing the length of the hypoplastic little finger and preservation of function. We report two patients with ulnar cleft hand without missing fingers. Both underwent cleft closure. One was treated by lengthening of the fifth metacarpal with an external fixator, and the other by finger translocation. Both patients achieved a near normal length of the little finger and a reasonable functional and aesthetic outcome. Level of Evidence: Level V (Therapeutic).

The Hand in Distal Arthrogryposis.

Distal arthrogryposis is the second most common type of arthrogryposis after amyoplasia and is defined as arthrogryposis that affects hands and feet; it is mostly inherited in an autosomal-dominant fashion. This review discusses up-to-date background information, clinical features, and treatment of distal arthrogryposis in hands concentrating on camptodactyly, thumb-in-palm deformity, and windblown hand deformity, which are the most common and functionally limiting deformities. Treating these deformities should be individualized and follow a multidisciplinary approach. Most deformities can be initially treated nonoperatively, and if not responsive, operative treatment may be pursued to improve function. Surgery primarily aims to release soft-tissue contractures, rebalance muscle forces, and may need bony correction based on the deficits of each case. Current literature suggests that early treatment leads to better outcomes. However, reported cases are scarce, and no consensus or gold standard for treatment exists. Therefore, long-term (multicenter) studies are needed to assess outcomes and standardize the treatment of such deformities whenever possible.

The importance of embryology for parents of children with congenital hand differences.

This study aimed to determine whether embryology knowledge or explaining the possible developmental pathway error was important for parents of affected children, and to secondarily determine if there was a relationship between desired knowledge of embryology and disease severity, maternal age group or maternal level of education. Using a self-administered questionnaire, a significant proportion of responding parents considered knowledge of embryology important (32 out of 43). We found a significant association between the importance of embryology knowledge for parents and the disease severity. However, the importance and level of knowledge desired was not related to maternal age or level of education. This study demonstrated the importance of explaining the associated developmental errors in the congenital hand consultation, particularly in severe anomalies. Surgeons should familiarize themselves with embryology to provide an explanation as to why congenital hand differences happen, which may provide better psychological support for parents of these children.

Pollicization in the treatment of congenital severe hypoplasia and aplasia of the thumb: a systematic review.

Pollicization of the index is the treatment of choice for severe hypoplasia and aplasia of the thumb. After a historical overview, we present a systematic review of this procedure. The main steps of this procedure were reported by Dieter Buck-Gramcko in 1971 and are still relevant nowadays. Many refinements have been described over the last decades by different surgeons to address limitations related to bone stock, musculotendinous structures and skin incisions. However, considering the complexity of this procedure and the results in the literature, the functional and esthetic outcomes can still be improved thanks to basic research. Pollicization of the index is rarely done and is one of the most demanding surgical procedure in hand surgery.

Natural Progression and Symptomatic Management of Tarsal-Carpal Coalition Syndrome: A Case Report.

CASE: Tarsal-carpal coalition syndrome (TCCS) is a disorder identified by fusion of the carpals, tarsals, and phalanges of the hands and feet. We describe a case of an 11-year-old girl who has been followed at our outpatient clinic from the age of 8 months. CONCLUSION: Although patients with TCCS can experience a wide range of symptoms, the primary complaint arises from the foot deformity and associated pain. Using advanced imaging such as 3D computed tomography reconstruction and genetic testing, this report details the clinical, genetic, and radiographic characteristics of the disorder. We highlight the natural progression and symptomatic management of TCCS.