ABSTRACT
Poland’s syndrome is a rare congenital anomaly consisting of a unilateral absence of the pectoralis major, ipsilateral muscle, hand anomaly and occasionally associated other malformations of the chest wall and breast. Many structural and functional abnormalities have been described in association with this syndrome. We report an incidentally diagnosed case in a 27-year-old male patient who presented to us with symbrachydactyly. In addition to this, anterior depression of 2nd, 3rd and 4th ribs and bifid (forked) 5th rib was present on radiological investigations. The body of sternum was short and deformed on the right side with absence of xiphoid process. All middle phalanges were absent on right hand. It is a rare variant of Poland’s syndrome.
Subject(s)
Adult , Fingers/abnormalities , Fingers/diagnostic imaging , Humans , Male , Muscular Diseases/complications , Pectoralis Muscles/abnormalities , Poland Syndrome/complications , Ribs/abnormalities , Syndactyly/complicationsABSTRACT
Poland’s syndrome is a rare congenital condition, characterized by the absence of the sternal or breastbone portion of the pectoralis major muscle, which may be associated with the absence of nearby musculoskeletal structures. We hereby report an 8‑year‑old boy with typical features of Poland syndrome, the first documented case from Uttar Pradesh, India.
Subject(s)
Child , Humans , India , Male , Pectoralis Muscles/abnormalities , Poland Syndrome/epidemiology , Poland Syndrome/genetics , Syndactyly/epidemiology , Syndactyly/geneticsABSTRACT
The Moebius syndrome is an infrequent symptomology in which the sixth and seventh cranial nerves are involved. Such involvement is translated in facial paralysis. There have been described around 500 cases in the world medical literature and some of them have received surgical treatment. Moebius syndrome has also received other names such as congenital nuclear aplasia, childlike nuclear aplasia, Oculofacial congenital paralysis and facial diplegia. Poland syndrome is another rare congenital abnormality of the chest wall, characterized by unilateral partial or total absence of the great pectoral muscle and ipsilateral symbrachydactyly. However, the Moebius-Poland syndrome is rarer. Its first case was recently reported in 2007 by Diego López de Lara et al. In this article we will report this infrequent case; the combination between both syndromes Moebuis and Poland in a three -month- old male patient.
El síndrome de Moebius es una sintomatología poco frecuente en la que los pares craneales sexto y séptimo están involucrados. Esta implicación resulta en parálisis facial. Se han descrito unos 500 casos en la literatura médica mundial y algunos de ellos han recibido tratamiento quirúrgico. Además el síndrome ha recibido otros nombres, tales como aplasia congénita nuclear, aplasia nuclear infantil, parálisis congénita oculofacial y diplejía facial. El síndrome de Poland es otra anomalía congénita muy poco frecuente de la pared torácica, caracterizado por ausencia unilateral parcial o total del músculo pectoral mayor y braquisindactilia ipsilateral. Sin embargo, el síndrome de Moebius-Poland es más raro, ya que el primer caso fue reportado recientemente en el año 2007 por Diego López de Lara et al. En este artículo se presentará este caso poco frecuente, que es una combinación entre ambos síndromes Moebius y Poland en un paciente masculino de tres meses de edad.
ABSTRACT
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.