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首页> 外文期刊>Revista chilena de pediatría >Asociación Sindromática: Poland, Goldenhar, Moebius, Klippel-Feil. Presentación de un Caso Clínico
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Asociación Sindromática: Poland, Goldenhar, Moebius, Klippel-Feil. Presentación de un Caso Clínico

机译：症状协会：波兰，Goldenhar，Moebius，Klippel-Feil。临床病例介绍

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摘要

Poland Syndrome is a congenital defect characterized by a unilateral absence of the clavicular and stem costal portion of the pectoral muscles associated to abnormalities of other muscles of the thoracic wall, ribs, breast and upper extremity. It is found in one of 20 to 32 thousand newborns. It is found sometimes associated to other syndromes, most often with Moebius Syndrome, and rarely with Goldenhar and Klippel-Feil. Due to the association, a common pathogenic cause has been postulated, that being an anomaly of vascularization during embryonic development. Clinical Case: A newborn male was seen who presented with Poland, Goldenhar, Moebius and Klippel-Feil Syndromes. Clinically, he presented left hemi facial microsomy, microtia, shortening and paralysis of the facial nerve; his neck was short and movement was limited due to C4-C5 fusion; agenesis of left pectorals, hypoplasia of left radius and hand. There were no known additional family cases, being thus, a sporadic syndromatic association.
机译：波兰综合症是一种先天性缺陷，其特征是单侧缺少胸壁的锁骨和干肋部分，与胸壁，肋骨，乳房和上肢其他肌肉的异常有关。在20至32,000名新生儿中发现了它。人们有时发现它与其他综合症有关，最常见的是Moebius综合症，很少与Goldenhar和Klippel-Feil合并。由于这种关联，已推测出常见的病因，这是胚胎发育过程中血管化的异常。临床病例：观察到一名新生男性，他患有波兰，戈登哈尔，莫比乌斯和克利佩尔·费尔综合征。临床上，他表现出左半面部面部切开，小口畸形，面神经缩短和麻痹。由于C4-C5融合，他的脖子短而活动受限;左胸骨发育不全，左radius骨和手发育不全。没有已知的其他家庭病例，因此是一个零星的综合症。

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