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.

Oculodentodigital dysplasia.

Oculodentodigital dysplasia is a rare, autosomal dominant disorder with high penetrance and variable expressivity, caused by mutations in the connexin 43 or gap junction protein alpha‑1 gene. It has been diagnosed in fewer than 300 people worldwide with an incidence of around 1 in 10 million. It affects many parts of the body, particularly eyes (oculo), teeth (dento), and fingers and/or toes (digital). The common clinical features include facial dysmorphism with thin nose, microphthalmia, syndactyly, tooth anomalies such as enamel hypoplasia, anodontia, microdontia, early tooth loss and conductive deafness. Other less common features are abnormalities of the skin and its appendages, such as brittle nails, sparse hair, and neurological abnormalities. To prevent this syndrome from being overlooked, awareness of possible symptoms is necessary. Early recognition can prevent blindness, dental problems and learning disabilities. Described here is the case of a 21‑year‑old male who presented to the ophthalmology outpatient department with a complaint of bilateral progressive loss of vision since childhood.

Fibrodysplasia Ossificans Progressiva with Rare Familial Inheritance Pattern: A Case Series.

Fibrodysplasia ossificans progressive (FOP)also termed as “stone man syndrome” is an autosomal dominant disorder manifested in the form of congenital malformation of the great toes with progressive ossification of the skeletal muscle and soft tissues in a specific pattern. It is a rare disorder, usually as sporadic and isolated and very few cases have been reported. Often FOP is missed or diagnosed very late, both of which leads to rapid progression and reduced life expectancy. We report first of such a case series in India with the hereditary transmission in a family, affecting 3 persons. Our index case was the first daughter 17 years female who presented with stiff joints, worsening over time and her father 45 years male was also affected with his major joints fused in different positions, and her younger sister 13 years female was in the early stage of disease. Further investigations such as serum calcium, phosphorous, X-ray, computed tomography, and bone scan confirmed it as cases of FOP. We present our detailed study of the interventions, their effectiveness, the role of drugs given to reduce symptoms in FOP, and this would aid the physicians and healthcare workers to suspect/diagnose the disease.

The Prevalence and Genetic Pattern of Clinodactyly in Korean Populations / 체질인류학회지

Clinodactyly, as rare congenital malformation, refers to a curvature of a digit in a radial or ulnar direction in the coronal plane. The abnormality is inherited as an autosomal dominant trait. And its frequency was low, however, it was higher by accompanying other congenital anomaly. In present study, the frequency and genetic characteristics of clinodactyly were investigated. In 100 family (382 peoples), clinodactyly was found in 4.7% (n=18). All clinodactyly were bilateral and it was more frequent in female (6.8%) than male (2.6%), without statistical difference (p=0.056). Its inheritance was autosomal dominant trait in 80% (4/5) families, however, one family did not have any inheritance pattern. We described the frequency and clinical implication of clinodactyly, and this description will be lead to an improved understanding of its spectrum and inheritance.

Bilateral proximal delta phalanges of hand: a rare case report with review of literature.

Delta phalanx or longitudinal bracketed epiphysis is a rare congenital anomaly that affects phalanges in the hand more commonly than toes. It results from a defective secondary ossification that extends longitudinally along the diaphysis and brackets the diaphysis and metaphysis, causing restricted longitudinal growth and resultant angular deformities. Although rare, longitudinal epiphyseal bracket most commonly manifests in the hands as clinodactyly and in the feet as hallux varus. We report a case of 18 year old male patient with isolated bilateral multiple proximal delta phalanges of hand. Keywords:

Congenital Clinodactyly of the Thumb.

Aims: To present five patients with thumb clinodactyly and the results of surgical treatment in two patients who were followed till skeletal maturity. Case Presentation: Four patients (five thumbs) exhibited an ulnar interphalangeal angulation and were all associated with a triphalangeal thumb, while in only one patient a radial deviation associated with a longitudinal epiphyseal bracket of the proximal phalanx was noted. Three patients (four thumbs) received primary surgical treatment for thumb polydactyly. A three-phalangeal thumb was retained in all. Two of these thumbs were also treated surgically for clinodactyly. Removal of the accessory delta phalanx and reconstruction of the soft tissues was performed in a 3-year-old girl. Follow-up at 18 years of age revealed reduced size of the thumb, limitation of flexion and a secondary radial interphalangeal deviation. A closing wedge osteotomy of the shaft of the proximal phalanx, leaving the delta phalanx undisturbed, was performed in a 12-year-old girl. Follow-up at 18 years of age revealed no loss of thumb alignment but not improved function. Discussion: The aim of surgical reconstruction of thumb clinodactyly is to create a painless thumb of adequate mobility, stability, alignment and size. Removal of an angulated middle phalanx in a very young child should be associated with a ligament reconstruction from the soft tissues. In an older child or adult, an osteotomy of the shaft of the proximal phalanx or fusion of the oblique joint and realignment with bone resection may be indicated. The real value of a surgical procedure may be evaluated only when the satisfactory result remains unimpaired beyond skeletal maturity. Conclusion: Surgical reconstruction of thumb clinodactyly may require specific challenges and techniques to obtain a painless, properly aligned, stable thumb with improved function that will retain the satisfactory result beyond skeletal maturity.

The Correction of Clinodactyly on Thumb by Bone Graft from Deformed Metacarpal Region-Previous Correction of Polydactyly: Case Report

Clinodactyly is defined as an angulation of a digit in the radio-ulnar plane. This anomaly can be congenital, dominantly inherited, or acquired due to trauma or inflammation. Although the deformity usually causes little functional impairment, correction is made because of cosmetic problems. Male subject, with polydactyly on thumb(Wassel's type VII) received first surgery at the age of one. And at the age of six, abnormal growth on the radial side of the first metacarpal bone and ulnar deviation of the distal phalanx of the thumb at the interphalangeal joint had developed. The authors used the growing bony segment from the first metacarpal bone as a bone graft for the correction of clinodactyly on thumb. Z-plasty incision was made on the concave(ulnar) side of thumb and a wedge osteotomy was made on the distal phalanx. The bone graft was inserted into the gap of the distal phalanx of the thumb and fixed it with K-wires. Deformity of the metacarpal bone and clinodactyly on thumb was corrected effectively without donor site morbidity with noticible growth of the grafted metacarpal bone 12 months after surgery.