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.

Macrodactilia / Macrodactylie / Macrodactyly

Se presenta un paciente con macrodactilia progresiva del segundo y el tercer dedos de la mano derecha al cual se le realizó una amputación en rayo del segundo y tercer dedos con transposición de la base del cuarto metacarpiano a la base del tercero. El paciente presentó una evolución posoperatoria favorable con una estética y función de la mano aceptables(AU)

We present a male patient with progressive macrodactyly of the second and third fingers of the right hand. A ray amputation was made in the second and third fingers with transposition from the base of the fourth metacarpal to the base of the third. The patient had favorable postoperative evolution, with acceptable aesthetic and hand function(AU)

Un patient atteint de macrodactylie progressive du deuxième et troisième doigt de la main droite, ayant subi une amputation du deuxième et troisième rayon digital et une transposition de la base du quatrième métacarpien à la base du troisième, est présenté. Il a eu une évolution postopératoire favorable, avec esthétique et fonction acceptables(AU)

Macrodactilia: estudo retrospectivo de quatro casos / Macrodactyly: a retrospective study of four cases

INTRODUÇÃO: A macrodactilia é uma anomalia rara e de etiologia desconhecida. Os primeiros casos foram descritos, em 1821, por Klein. Representa aproximadamente 1% de todas as anomalias congênitas. Surge no nascimento e caracteriza-se pelo crescimento dos dedos das mãos, dos pés ou de todo o membro; entretanto, pode se apresentar mais tardiamente, com os sintomas de compressão de nervo, podendo associar-se à síndrome do túnel do carpo. MÉTODOS: Estudo retrospectivo de quatro casos de macrodactilia atendidos no Hospital da Santa Casa Misericórdia de Campo Grande, MS, nos últimos 10 anos. RESULTADOS: Descrevemos quatro casos de macrodactilia, sendo três em quirodáctilos e um acometendo primeiro pododáctilo. Todos os pacientes tratados com procedimentos cirúrgicos, um dos casos com amputação de falanges e metacarpo. CONCLUSÕES: É recomendada a amputação como opção cirúrgica em alguns casos e o tratamento precoce da síndrome do túnel do carpo quando presente.

INTRODUCTION: Macrodactyly is a rare anomaly of unknown etiology. The first cases were described in 1821 by Klein. It represents approximately 1% of all congenital anomalies. It appears at birth and is characterized by excessive growth of the fingers, toes, or of the entire limb; however, its appearance may be delayed, with symptoms of nerve compression, and may present with carpal tunnel syndrome. METHODS: Retrospective study of four cases of macrodactyly treated at the Hospital da Santa Casa Misericórdia in Campo Grande, MS, in the last 10 years RESULTS: We describe four cases of macrodactyly: three in the fingers and one affecting the first toe. All patients were treated with surgical procedures, one with amputation of phalanges and metacarpals. CONCLUSIONS: Amputation is a surgical option recommended in some cases, as is the early treatment of carpal tunnel syndrome.

Retalho tenar adipofascial reverso para cobertura do nervo mediano hipertrofiado em macrodactilia / The reverse adipofascial thenar flap for coverage of the hypertrophied median nerve in macrodactyly

A macrodactilia é uma anomalia congênita infrequente, caracterizada pelo crescimento desproporcional dos tecidos ósseo, gorduroso, nervoso, vascular e dérmico nos dedos das mãos ou dos pés. Existem muitas teorias sobre a sua etiopatogenia, sendo a mais aceita a hiperestimulação por fatores de crescimento enviados através dos nervos. Foram descritos alguns casos associados com a síndrome do túnel do carpo. Apresenta-se um caso clínico de síndrome do túnel do carpo por hipertrofia do nervo mediano, evidenciando um aumento de conteúdo dentro do retináculo flexor, o qual foi tratado cirurgicamente com sucesso pela realização de uma retinaculotomia do ligamento anular do carpo junto a um retalho de transposição tenar adipofascial reverso da região tenariana hipertrofiada visando à cobertura do nervo mediano na região do punho.

Macrodactyly is a rare congenital anomaly characterized by the disproportionate growth of bone, fat, nervous, vascular, and dermal tissue in the digits . There are many different theories about its etiopathogenesis, the most accepted being a hyperstimulation by growth factors conducted through nerves. A few cases have been described in conjunction with carpal tunnel syndrome. Here, a clinical case of carpal tunnel syndrome due to hypertrophy of the median nerve is presented, showing an increase of content within the flexor retinaculum. Successful surgical treatment was accomplished by conducting a retinaculotomy of the anterior annular ligament along with a reverse transposition adipofascial flap of the hypertrophied thenar region for coverage of the median nerve at the wrist.

Resultados da correção da sindactilia na mão com a técnica de Skoog / Results of the correction of syndactyly of the hand by using Skoog's technique

Introdução: A sindactilia é uma anomalia congênita comum da mão, tem incidência de aproximadamente um em 2.000 nascimentos, predomínio no sexo masculino e é bilateral em 50% dos casos. Objetivo é descrever uma série de casos de pacientes com sindactilia nas mãos, operados no Hospital Sarah Brasília, com a técnica de Skoog modificada e o uso de órtese. Métodos: Realizou-se a técnica de Skoog modificada para correção de sindactilia. No pós-operatório, foi confeccionada órtese punho-mão e dedos, e procedeu-se à avaliação do resultado da cicatriz conforme a Escala de Vancouver. Resultado: Durante cinco anos, avaliaram-se 45 pacientes, sendo 30 (66%) homens e 15 mulheres, com idade de um a quatro anos (média de dois anos - 60%), 37% dos casos mostraram-se associados a síndromes, 50% eram simples e bilateral. As complicações observadas nas sindactilias simples foram encontradas em 8% dos pacientes e caracterizaram-se por retração cicatricial, cicatriz visível e alteração ungueal; três casos (6%) foram reoperados para liberação de retração cicatricial com zetaplastia e confecção de neocomissura e quatro casos demandaram realinhamento digital, observado nas sindactilias complexas. Conclusão: Com esta técnica, obtiveram-se 92% de resultados funcionais, 8% de complicações relacionadas à técnica cirúrgica e três reoperações para liberação de retração cicatricial, por meio de zetaplastia e aprofundamento da comissura com novo retalho e enxerto de pele Em relação à avaliação quanto à Escala de Vancouver, o item predominante foi a pigmentação existente entre os dedos devido ao enxerto de pele.

Introduction: Syndactyly is a congenital anomaly of the hand, occurring in approximately 1 in every 2000 births. It is more common in male patients and is present in both hands in 50% of the cases. The objective of this study was to describe a series of cases of patients with hand syndactyly, operated at the Sarah Hospital in Brasília using modified Skoog's technique and orthosis. Methods: The modified Skoog's technique was performed to correct syndactyly. Wrist-hand and finger orthosis was applied postoperatively, and the appearance of the scar was assessed according to the Vancouver Scar Scale. Results: Forty-five patients were assessed during five years, of whom 30 (66%) were male and 15 were female, all aged between 1 and 4 years (mean age: 2 years [60%]); 37% of cases were associated with syndromes and 50% were simple bilateral cases. Complications observed in simple syndactyly occurred in 8% of patients and included scar retraction, visible scars, and ungual changes; three patients (6%) underwent revision surgery with z-plasty and confection of a new web space commissure to correct scar retraction, and four patients required finger realignment, observed in complex syndactyly. Conclusion: This technique resulted in 92% good functional outcomes, 8% complications associated with the surgical technique, and three revision procedures with z-plasty and confection of the web space commissure with a new flap and skin graft to correct scar retraction. The Vancouver scale was used to assess scar quality, and pigmentation between the fingers (due to skin graft) had the highest score.

Evaluation of treatment for camptodactyly: retrospective analysis on 40 fingers / Avaliação do tratamento da camptodactilia: análise retrospectiva de 40 dígitos

OBJECTIVE: to retrospectively assess the results from cases treated in the hand surgery service, starting from a preestablished protocol; and to conduct a critical analysis on the results achieved, with separation of the cases into their respective subgroups. METHODS: twenty-three patients and a total of 40 fingers were evaluated between January 2004 and December 2011. We correlated the altered anatomical structures found in the cases that underwent the surgical procedure and its results, with regard to both conservative and surgical treatment, emphasizing the main indications. RESULTS: the results were analyzed using the Sierget method of the Mayo Clinic. CONCLUSION: we observed that the cases of camptodactyly of the little finger alone in the flex-ible form (>60◦) that underwent surgical treatment uniformly presented excellent results. In the rigid forms, our observations indicated that there were benefits comprising gains of extension and correction of the deformity. However, the range of motion with active flexion in the proximal interphalangeal joint was always partial. With evolution over time, some cases presented some loss of the gain previously achieved, which corroborates the need for continual vigilance during the follow-up, with systematic use of braces until the final phase of skeletal growth...

OBJETIVOS: avaliar, retrospectivamente, os resultados dos casos tratados no serviço de Cirurgia da Mão, a partir de um protocolo preestabelecido; e fazer uma análise crítica dos resultados alcançados, com a separação dos casos em seus respectivos subgrupos. MÉTODOS: foram avaliados 23 pacientes, num total de 40 dígitos, de janeiro de 2004 a dezembro de 2011. Relacionamos as estruturas anatômicas alteradas encontradas nos casos que foram submetidos a procedimento cirúrgico e seus resultados, tanto no tratamento conservador como no cirúrgico, e enfatizamos suas principais indicações. RESULTADOS: os resultados foram analisados pelo método de Sierget da clínica Mayo. Conclusão: observamos que os casos de camptodactilia isolada do dedo mínimo na forma flexível, > 60◦, que foram submetidos a tratamento cirúrgico de maneira uniforme apresentaram resultados excelentes. Nas formas rígidas, nossas observações indicam benefícios com ganho de extensão e correção da deformidade. Entretanto, o arco de movimento com flexão ativa na interfalângica proximal (IFP) é sempre parcial. Com o tempo de evolução, alguns casos apresentaram alguma perda do ganho previamente alcançado, o que corrobora a permanente vigilância necessária no acompanhamento, com uso sistemático de órteses, até a fase final do crescimento esquelético...

Bandas amnióticas constrictivas y derivación oportuna: a propósito de 2 casos clínicos / Constriction band syndrome and the right time for intervention: two clinical cases

Introducción: El síndrome de bridas amnióticas (SBA) es una afección infrecuente de etiología controversial con manifestaciones muy heterogéneas. Las extremidades son las más frecuentemente afectadas, y puede asociarse a otras malformaciones. El recién nacido con SBA rara vez va a requerir de cirugía inmediata, sin embargo, la evaluación por el especialista y la indicación quirúrgica debe ser oportuna y no postergada innecesariamente, lo cual podría significar la pérdida de la extremidad. Objetivo: Comunicar 2 casos de SBA que afectan la extremidad superior, en los cuales la intervención oportuna providencial resultó en un favorable pronóstico. Caso clínico: Reportamos dos casos, donde hubo una derivación tardía de los pacientes por mitos preconcebidos tales como "que era necesario completar el crecimiento para realizar un tratamiento definitivo" (caso 2) o "que no había ningún tratamiento que ofrecerle frente a la severidad de la malformación del recién nacido" (caso 1). En ambos pacientes las bridas constrictivas fueron liberadas en forma exitosa con el uso de múltiples zetoplastias, sin complicaciones en la evolución postoperatoria. Conclusión: Se discute y enfatiza la importancia del diagnóstico, derivación y tratamiento precoz de ésta patología.

Introduction: The amniotic band syndrome is an infrequent affection, of controversial aetiology, with extreme heterogeneous manifestations. Extremities are most frequently compromised and may be associated to other malformations. These newborns rarely need immediate surgery nevertheless the surgical indication must be timely and not postponed, which could mean loss of the extremity. Objective: To report 2 patients with the amniotic band syndrome, in whom casual early interventions were associated to a favorable prognosis. Case reports: We present two patients with an important delay in the referral to the specialist because of the preconceived myths that either full growth was necessary for definitive surgery (case 2) or that no possible treatment could be offered due to the severity of the diagnosis (case 1). All constricting bands were successfully and effectively released with multiple Z plasties with uneventful post operative evolution. Conclusion: We discuss and emphasize the importance of early diagnosis and intervention.

Avaliação do tratamento da camptodactilia: análise retrospectiva de 22 pacientes, em um total de 39 dígitos

Camptodactilia é uma deformidade congênita caracterizada por uma postura em flexão na IFP, geralmente encontrada no dedo mínimo. Afeta aproximadamente 1% da população. A causa primária para esta deformidade é ainda motivo de discussão, não havendo consenso na literatura mundial. Se não for tratada haverá uma piora progressiva em cerca de 80% dos casos. Essa patologia, de aparência relativamente simples, não é fácil de ser tratada. O problema reside no fato da existência de várias formas de apresentação clínica, o que faz com que não haja um modelo único de tratamento efetivo. O objetivo deste estudo é avaliar, retrospectivamente, os resultados dos casos tratados no serviço de Cirurgia da Mão do INTO-MS-RJ, a partir de um protocolo pré estabelecido; e realizar uma análise crítica dos resultados alcançados, separando os casos em seus respectivos subgrupos. Foram avaliados 22 pacientes, num total de 39 dígitos, de janeiro de 2004 a dezembro de 2012. Relacionamos as estruturas anatômicas alteradas encontradas nos casos que foram submetidos a procedimento cirúrgico e seus resultados, tanto no tratamento conservador, como para os cirúrgicos, enfatizando suas principais indicações. Os resultados foram analisados pelo método de Sierget e col da clínica Mayo. Observamos que os casos de camptodactilia isolada do dedo mínimo na forma flexível, >60º, que foram submetidos a tratamento cirúrgico, de maneira uniforme apresentaram resultados excelentes. Nas formas rígidas, nossas observações indicam benefícios com ganho de extensão e correção da deformidade, entretanto o arco de movimento com flexão ativa na IFP é sempre parcial. Com o tempo de evolução alguns casos apresentaram alguma perda de ganho previamente alcançado, o que corrobora a permanente vigilância necessária no acompanhamento, com uso sistemático de órteses, até a fase final do crescimento esquelético.

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

Radial longitudinal deficiency, also known as radial club hand, is a congenital deformity of the upper extremity which can present with a spectrum of upper limb deficiencies. The typical hand and forearm deformity in such cases consists of significant forearm shortening, radial deviation of the wrist and hypoplasia or absence of a thumb. Treatment goals focus on the creation of stable centralized and functionally hand, maintenance of a mobile and stable wrist and preservation of longitudinal forearm growth. Historically centralization procedures have been the most common treatment method for this condition; unfortunately centralization procedures are associated with a high recurrence rate and have the potential for injury to the distal ulnar physis resulting in a further decrease in forearm growth. Here we advocate for the use of a vascularized second metatarsophalangeal joint transfer for stabilization of the carpus and prevention of recurrent radial deformity and subluxation of the wrist. This technique was originally described by the senior author in 1992 and he has subsequently been performed in 24 cases with an average of 11-year follow-up. In this paper we present an overview of the technique and review the expected outcomes for this method of treatment of radial longitudinal deficiency.

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

Radial longitudinal deficiency, also known as radial club hand, is a congenital deformity of the upper extremity which can present with a spectrum of upper limb deficiencies. The typical hand and forearm deformity in such cases consists of significant forearm shortening, radial deviation of the wrist and hypoplasia or absence of a thumb. Treatment goals focus on the creation of stable centralized and functionally hand, maintenance of a mobile and stable wrist and preservation of longitudinal forearm growth. Historically centralization procedures have been the most common treatment method for this condition; unfortunately centralization procedures are associated with a high recurrence rate and have the potential for injury to the distal ulnar physis resulting in a further decrease in forearm growth. Here we advocate for the use of a vascularized second metatarsophalangeal joint transfer for stabilization of the carpus and prevention of recurrent radial deformity and subluxation of the wrist. This technique was originally described by the senior author in 1992 and he has subsequently been performed in 24 cases with an average of 11-year follow-up. In this paper we present an overview of the technique and review the expected outcomes for this method of treatment of radial longitudinal deficiency.

Free Fat Graft for Congenital Hand Differences

BACKGROUND: Free fat graft has been used for the treatment of congenital hand differences. However, there have been a few reports about the outcome of that treatment. In this study, the outcome of free fat grafts for congenital hand and foot differences was investigated. METHODS: Fourteen bones with longitudinal epiphyseal bracket, 3 wrists with Madelung deformity, and 5 cases of osseous syndactyly were treated with free fat graft with osteotomy, physiolysis, or separation of osseous syndactyly. Of the fourteen bones with longitudinal epiphyseal bracket, 9 were treated with open wedge osteotomy with free fat graft and 5 with physiolysis and free fat graft. The Madelung deformity was treated with physiolysis with free fat graft. For osseous syndactyly, syndactyly release with free fat graft was performed five times on four hands. RESULTS: In the fourteen cases with longitudinal epiphyseal bracket, lateral deviation improved in all except two cases after surgery. The average lateral deviation angle changed from 32.5 degrees before surgery to 15.2 degrees after surgery. The average improvement of the lateral deviation angle was 12.2 degrees in the osteotomy group and 20.6 degrees in the physiolysis group. The mean ratio of improvement of the lateral deviation angle to the lateral deviation angle before surgery was 39.4% in the osteotomy group and 51.2% in the physiolysis group. The Madelung deformity improved after surgery in two cases but there was no improvement in one case. For these conditions, the results were not good enough when surgery was done after age 13 or at age four for severely hypoplastic brachymesophalangy. Of the 5 cases of osseous syndactyly, reunion of the separated bones occurred in one case. The grafted free fat should be deep enough to cover the osteotomy site of the bones to prevent reunion of the separated bones. CONCLUSIONS: Physiolysis and free fat graft performed during the growth period can correct the deviation due to longitudinal epiphyseal bracket and Madelung deformity. Free fat graft is also useful to prevent reunion of the bones after separation of osseous syndcatyly, if the grafted fat is securely filled into the space between the separated bones.

Free Fat Graft for Congenital Hand Differences

BACKGROUND: Free fat graft has been used for the treatment of congenital hand differences. However, there have been a few reports about the outcome of that treatment. In this study, the outcome of free fat grafts for congenital hand and foot differences was investigated. METHODS: Fourteen bones with longitudinal epiphyseal bracket, 3 wrists with Madelung deformity, and 5 cases of osseous syndactyly were treated with free fat graft with osteotomy, physiolysis, or separation of osseous syndactyly. Of the fourteen bones with longitudinal epiphyseal bracket, 9 were treated with open wedge osteotomy with free fat graft and 5 with physiolysis and free fat graft. The Madelung deformity was treated with physiolysis with free fat graft. For osseous syndactyly, syndactyly release with free fat graft was performed five times on four hands. RESULTS: In the fourteen cases with longitudinal epiphyseal bracket, lateral deviation improved in all except two cases after surgery. The average lateral deviation angle changed from 32.5 degrees before surgery to 15.2 degrees after surgery. The average improvement of the lateral deviation angle was 12.2 degrees in the osteotomy group and 20.6 degrees in the physiolysis group. The mean ratio of improvement of the lateral deviation angle to the lateral deviation angle before surgery was 39.4% in the osteotomy group and 51.2% in the physiolysis group. The Madelung deformity improved after surgery in two cases but there was no improvement in one case. For these conditions, the results were not good enough when surgery was done after age 13 or at age four for severely hypoplastic brachymesophalangy. Of the 5 cases of osseous syndactyly, reunion of the separated bones occurred in one case. The grafted free fat should be deep enough to cover the osteotomy site of the bones to prevent reunion of the separated bones. CONCLUSIONS: Physiolysis and free fat graft performed during the growth period can correct the deviation due to longitudinal epiphyseal bracket and Madelung deformity. Free fat graft is also useful to prevent reunion of the bones after separation of osseous syndcatyly, if the grafted fat is securely filled into the space between the separated bones.

Our experience with macrodactyly: a rare congenital anomaly

To present our experience with [Macrodactyly] a rare congenital anomaly and its associated anomalies. Macrodactyly is an increase in the size of one or several fingers or toes. The overgrowth is limited to or predominantly affects the digits. It is characterized by an increase in all mesenchymal elements particularly fibro-adipose tissue. It does not appear to be an inherited condition and is thought to be caused by abnormal nerve supply, abnormal blood supply or abnormal humeral mechanisms. Pathologically, they are benign, soft tissue growths. Macrodactyly is commonly an isolated condition but other congenital anomalies are associated with it. It can be static or a progressive disorder. Soft tissue debulking, phalangectomies, ray resection, ostetomies and arthrodesis of interphalangeal joints are different modes of treatment. Descriptive case series. This study was conducted in Plastic Surgery Unit of Hayatabad Medical Complex Peshawar and Orthopaedic unit of Khyber Teaching Hospital, Peshawar from April 2007 to December 2009. A total of 32 patients were registered during the study period. Patients were admitted through out patient department, written informed consent was obtained from all individuals. Detailed history was taken, every patients was assessed clinically and radiologically. All patients were followed for recurrence. 2 patients were lost in follow up and the study was completed on 30 patients. Mean age of the patients was 13.7 years. Out of 30 patients, 19 were male and 11 were female. Hands were involved in 20 patients and feet in 10 patients. There was no bilateral hands or feet involvement. Eighteen patients had progressive and 12 patients have static macrodactyly. Seventeen patients had isolated macrodactyly while in 13 patients macrodactyly was associated with other congenital anomalies most commonly syndactyly. Most commonly involved digit was index finger in hand and big toe in foot. Macrodactyly is a rare congenital anomaly but cosmetic and functional disability of the patient is significant. Although it is mostly isolated but a significant number of cases were associated with other congenital anomalies which necessitate further research in this field

Macrodactilia na mão: relato de caso / Hand macrodactyly: a case report

Os autores descrevem a evolução de um caso de macrodactilia digital progressiva na mão ao longo de 10 anos e os programas cirúrgicos e de reabilitação utilizados. Criança do sexo feminino foi admitida no Hospital de Reabilitação aos 10 meses de idade, apresentava macrodactilia digital progressiva no II e III quirodáctilos da mão esquerda. Evoluiu com síndrome do túnel do carpo, atrofia e perda da oponência do polegar. Aos dois anos, foi submetida a amputação do III raio; aos seis anos, a encurtamento do II dedo, epifisiodese e, aos nove anos, a retinaculotomia dos flexores, amputação do II raio e restauração da oponência do polegar com a transferência do extensor próprio do índex. Foram necessários vários procedimentos e métodos de tratamento para o alívio dos sintomas de compressão do nervo mediano e resultado final funcional satisfatório. Os autores recomendam a amputação como opção cirúrgica e o tratamento precoce da síndrome do túnel do carpo.

The authors describe the progress over a ten year period of a single hand macrodactyly digital progressive case and corresponding surgical and rehabilitation procedures used. The female child was first evaluated at the hospital at the age of 10 months, with macrodactyly digital progressive at I and II fingers of the left hand. Afterwards she developed carpal tunnel syndrome, atrophy and lost of thumb opposition. The third ray was amputated at age 2. At age 6 she was realized finger shortening, physeal arrest and at age 9 a carpal tunnel release, II ray resection and proprius extensor tendon opponensplasty. Many interventions and treatment methods were required to relieve the compression symptoms of the median nerve and satisfactory final functional result. The authors recommend amputation as a surgical option and early carpal tunnel release.

Alargamiento progresivo del antebrazo en el niño con minifijador externo RALCA® / Progressive lengthening of the forearm in children with RALCA® external minifixators

Se realizó un estudio de intervención cuasiexperimental con 8 pacientes portadores de acortamiento del antebrazo, operados en el Complejo Científico Ortopédico Internacional Frank País, entre enero de 2002 y febrero de 2004, y a los cuales se les efectuó un alargamiento progresivo del antebrazo con minifijadores externos RALCA®. 5 pacientes presentaron afecciones bilaterales y fue la mano zamba congénita la patología más frecuente con 5 casos, cuatro de ellos bilaterales. En 11 de los 13 antebrazos operados fue necesario realizar osteotomías para corregir las deformidades angulares y en todos se utilizó un alambre guía intramedular. La centralización de la muñeca fue la técnica asociada que más se empleó. Se logró un promedio de 3,2 cm de elongación y se obtuvo una mejoría significativa en los movimientos de flexo extensión del codo y de la muñeca. La complicación más frecuente fueron las parestesias durante el tiempo que duró la elongación ósea. En el 100 por ciento de los casos tanto pacientes como familiares manifestaron satisfacción con los resultados estéticos y funcionales.

A quasi-experimental interventional study was undertaken among 8 patients carriers of shortening of the forearm operated on at Frank País International Orthopedic Complex between January 2002 and February 2004. Progressive lengthening of the forearm with RALCA® external minifixators was performed. 5 patients presented bilateral affections and the congenital club hand proved to be the most common pathology with 5 cases, 4 of them bilateral. In 11 of the 13 forearms operated on, it was necessary to carry out osteotomy to correct the angular deformities and in all of them it was used an intramedullary guide wire. The centralization of the wrist was the most used associated technique. An average elongation of 3.2 cm was attained. A significant improvement of the flexo-extension movements of the elbow and wrist was observed. The most frequent complication was paresthesia during the time the bone elongation lasted. In 100 percent of the cases, both relatives and patients expressed their satisfaction with the aesthetic and functional results.

Index finger pollicisation for a congenital thumb hypoplasia and aplasia-surgical techniques

Thumb hypoplasia and aplasia are disabling congenital deformities and in the more severe cases [Grades IV and V] the only possibility is pollicisation Some Grade III hypoplasia cases are more severe with absence of the trapezio-metacarpal joint and most of the first metacarpal. These cases are best treated by amputation and pollicisation. The authors describe in detail pollicisation of the index and its pitfalls

Tratamento cirúrgico das sindactilias congênitas da mão pela técnica de Bauer / Surgical treatment of congenital hand syndactyly using Bauer's technique

Objetivo: Analisar os resultados clínico-funcionais do tratamento das sindactilias congênitas da mão pela técnica de Bauer et al, inicialmente descrita para a correção das sindactilias simples totais. Métodos: Foram estudadas 13 crianças, totalizando 21 mãos, submetidas a tratamento cirúrgico pela técnica de Bauer et al, todas acompanhadas por um período médio de 14 meses após a cirurgia. Entre os pacientes, sete (54 por cento) eram do sexo masculino e seis (46 por cento) do feminino; cinco (38 por cento) tinham acometimento unilateral e oito (62 por cento), bilateral. Entre as mãos, oito (38 por cento) apresentavam sindactilia simples e 13 (62 por cento), sindactilias complexas. Foram utilizadas, segundo a técnica, incisões em linha quebrada para a separação dos dedos, um retalho da vizinhança para a reconstrução da comissura e um enxerto de pele total retirado da região inguinal para cobertura da área paracomissural. Resultados: Adotados na sua interpretação os critérios propostos por Friedhofer et al, que levam em consideração a comissura, os retalhos digitais, os enxertos paracomissurais, a cicatriz e a funcionabilidade da mão, os autores obtiveram 15 mãos com bom resultado (72 por cento,) três regulares (14 por cento) e três com mau resultado (14 por cento) durante o acompanhamento. Conclusão: O emprego da técnica de Bauer proporcionou, tanto nos casos de sindactilia simples como complexas, percentual significativo de bons resultados e índice baixo de complicações

Tratamiento quirúrgico en braquisindactilia: formación de pinza eficiente / Surgical treatment in braquisindactily: building a useful forceps

The therapy for hand malformations is challenging especially in aphalangia in symbrachydactyly. Ten cases of symbrachydactyly are presentd which are seek to obtain a useful forceps of the hand, treated with two surgical techniques: I. Resection of nubbins and Z-plasties of the interdigital space II. Nonvascularized extraperiostic graft of the first phalanx of the fourth toe to a beam of the hand. Surgical techniques, complications and resultd are described.

El tratamiento de las malformaciones congénitas de mano es un desafío especialmente en braquisindactilia. Se presentan 10 casos de braquisindactilia en los cuales se busca conseguir una pinza útil en la mano, tratados con dos técnicas quirúrgicas: 1. Resección de los mamelones digitales y zetoplastias del espacio interdigital logrado. II. Injerto extra perióstico no vascularizado de la 1° falange del 4° ortejo a un rayo de la mano. Se describe la técnica quirúrgicas, sus complicaciones y resultados.