Polisindactilia preaxil. Presentación de caso / Preaxile polysyndactyly. Case presentation / Polissindactilia do preáxil. Apresentação do caso

RESUMEN La polisindactilia es un defecto congénito que tiene lugar en las manos o en los pies, o en ambos al mismo tiempo y que consiste en la existencia de múltiples dedos supernumerarios además de la fusión de tejido blando y/o óseo entre dedos adyacentes, generando la ausencia total o parcial del espacio entre dos dedos. Se presenta el caso de una paciente femenina de 35 años de edad. No presenta ningún antecedente patológico personal de interés, ni alergias medicamentosas conocidas. Presenta una sindactilia compleja en el dedo pulgar de la mano derecha, con polidactilia preaxil, acompañado de disminución de la movilidad en las articulaciones de este dedo. Esta paciente voluntariamente y con consentimiento informado forma parte del proyecto de investigación "Defectos congénitos del esqueleto apendicular de la institución." Este defecto afecta el 1.2% de los recién nacidos y el 10 % de esta aparición ocurre en las extremidades superiores, siendo la duplicación del pulgar la polidactilia más común de la mano. La polisindactilia es una entidad con muy poca frecuencia, es más común en la población blanca y asiática, en esta entidad es importante el tratamiento temprano para evitar deformidades en el dedo afectado, Idealmente se debe realizar la cirugía entre los 10 y 12 meses de edad.

ABSTRACT Polysyndactyly is a congenital defect that occurs in the hands or feet, or both at the same time and that consists of the existence of multiple supernumerary fingers in addition to the fusion of soft tissue and / or bone between adjacent fingers, generating the total or partial absence of the space between two fingers. The case of a 35-year-old female patient is presented. He does not present any personal pathological history of interest, or known drug allergies. It presents a complex syndactyly in the thumb of the right hand, with preaxial polydactyly, accompanied by decreased mobility in the joints of this finger. This patient voluntarily and with informed consent is part of the research project "Congenital defects of the appendicular skeleton of the institution." This defect affects 1.2% of newborns and 10% of this appearance occurs in the upper extremities, being the duplication of the thumb the most common polydactyly of the hand. Polysyndactyly is an entity with very little frequency, it is more common in the white and Asian population, in this entity early treatment is important to avoid deformities in the affected finger, Ideally, surgery should be performed between 10 and 12 months of age.

RESUMO A polissindactilia é um defeito congênito que ocorre nas mãos ou nos pés, ou ambos ao mesmo tempo e que consiste na existência de múltiplos dedos supranumerários além da fusão de tecidos moles e / ou osso entre os dedos adjacentes, gerando o total ou parcial ausência de espaço entre dois dedos. É apresentado o caso de uma paciente de 35 anos de idade. Ele não apresenta nenhuma história patológica pessoal de interesse ou alergia a medicamentos conhecida. Apresenta sindactilia complexa no polegar da mão direita, com polidactilia pré-axial, acompanhada de diminuição da mobilidade nas articulações deste dedo. Este paciente voluntariamente e com consentimento informado faz parte do projeto de pesquisa "Defeitos congênitos do esqueleto apendicular da instituição". Esse defeito atinge 1,2% dos recém-nascidos e 10% desse aparecimento ocorre nas extremidades superiores, sendo a duplicação do polegar a polidactilia da mão mais comum. A polissindactilia é uma entidade com muito pouca frequência, é mais comum na população branca e asiática, nesta entidade o tratamento precoce é importante para evitar deformidades no dedo afetado. Idealmente, a cirurgia deve ser realizada entre 10 e 12 meses de idade.

Plane-shaped flap for reconstruction of the web space in polysyndactyly of the fifth toe fused with the fourth toe / 中华医学美学美容杂志

Objective@#To discuss the application of plane-shaped flaps for reconstruction of the web space in polysyndactyly of the fifth toe fused with the fourth toe.@*Methods@#A total of 62 cases (81 feet) with polysyndactyly of the fifth toe fused with the fourth toe were involved in the study, 53 of which were incomplete and 9 were complete fusion of the fourth and fifth toe. The fifth toe showed various degrees of fibular clinodactyly and minor deformities. Excision of polydactyly and then using arthroplasty or osteotomy correction deviation was for fibular clinodactyly. All plane-shaped flaps were used for reconstruction of the web space deeply, and the lateral sides of toes were closed with flaps and the distal soft tissues of polydactyly were used lengthening the reconstructed fifth toe. Brachydactylias were improved, and nail folds were reconstructed. Thus, the appearances reached the normal level.@*Results@#All the webs were reconstructed primarily without skingraft. After 12 months of follow-up, these reconstructed web space were satisfied with good appearances of width, depth and gradient. The fibular clinodactyly was completely corrected, and brachydactylias were corrected in various degrees. Nail folds reached the normal ones.@*Conclusions@#Plane-shaped flap in polysyndactyly of the fifth toe fused with the fourth toe for reconstruction of the web space is satisfactory. The reconstructed webs not only achieves good appearance, but also avoids skin grafts. It is one of the best choices of the polysyndactyly treatment.

Analysis of Congenital Postaxial Polydactyly of the Foot Using Magnetic Resonance Imagings / 대한정형외과학회잡지

PURPOSE: We aimed to evaluate the magnetic resonance imaging (MRI) findings of congenital postaxial polydactyly of the foot. MATERIALS AND METHODS: Three-hundred and forty-seven feet of 288 patients who underwent congenital postaxial polydactyly or polysyndactyly correction were divided into five subtypes according to the radiographic shapes of deformity origins (widened metatarsal head, bifid, fused duplicated, incompletely duplicated, or completely duplicated). MRIs were assessed to determine whether they unrevealed areas were fused or separated. MRI was also used to assess cases with radiographic phalangeal aplasia. RESULTS: Huge variations were noted in MRIs. Fusion or separation at the base or head between original and extra digits were observed, and MRI effectively depicted phalangeal aplastic areas. CONCLUSION: MRI evaluations of congenital postaxial polydactyly of the foot are useful for determining the anatomical statuses which were not visualized by plain radiography (level of evidence: 3).

Crossed Polydactyly and Greig Cephalopolysyndactyly Syndrome.

Greig cephalopolysyndactyly syndrome is a rare genetic disorder, with an autosomal dominant inheritance and consisting of a triad of polysyndactyly, macrocephaly and hypertelorism. Crossed polydactyly is a finding characteristically associated with this syndrome. We report a one and half year old male child who presented with classic clinical features and family history diagnostic of the above syndrome.

Nationwide Estimation for Incidence at Birth of Congenital Polydactyly and Syndactyly in Korean

INTRODUCTION: The aim was to estimate the incidence at birth of congenital polydactyly and syndactyly to use medical utilization database of the National Health Insurance Corporation in Korea. MATERIAL & METHOD: Base population are selected from the children born in 1994(n=728,515). The diseases were identified by disease code system; ICD-9 755(Other congenital limb-anomalies), ICD-10 Q69(Polydactyly), ICD-10 Q70(Syndactyly), ICD-10 Q66(Other congenital feet-deformities), ICD-10 Q74(Other upper limb-anomalies). The suspicious cases of polydactyly and syndactyly are selected from the base population with those criteria(n=3,497). The 669 cases, 16 university hospitals and 16 hospitals have been examined the name and location of anomaly through medical record review. The diagnostic accuracy by Disease-code system was calculated. The incidence rate at birth was estimated by the class of polydactyly/syndactyly, adjusted for the diagnostic accuracy. RESULT: The incidence per 100,000 birth are estimated; at hand, polydactyly, 93(95% confidence interval 86-100), syndactyly, 29(25-33), polysyndactyly, 13(11- 16); at foot, polydactyly, 40(36-45), syndactyly, 22(19-26), polysyndactyly, 14(12 - 17); at multifocal area(hand & foot), polydactyly, 1(0.6 -2), syndactyly, 2(0.9-3), polysyndactyly, 3(2 - 4). CONCLUSION: These incidence rates may be underestimated and show the minimum incidence rates. The incidences must be discussed in comparison with incidences through registry or prospective follow-up study of congenital anomalies.