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与右位相关的波兰综合征变体。

A variant of Poland syndrome associated with dextroposition.

作者信息

Mutlu Hakan, Sildiroglu Onur, Basekim Cinar C, Kizilkaya Esref

机构信息

Gulhane Military Medical Academy, Haydarpasa Teaching Hospital, Department of Radiology, Istanbul, Turkey.

出版信息

J Thorac Imaging. 2007 Nov;22(4):341-2. doi: 10.1097/01.rti.0000184831.84395.05.

Abstract

Poland syndrome is characterized by partial or complete absence of pectoralis muscles, ipsilateral syndactyly, and occasionally other malformations of the anterior chest wall and breast. Other associated anomalies, including dextrocardia, renal malformations, and vertebral anomalies, have been reported in rare cases. The condition is more frequent among males, and usually occurs on the right hemithorax in the unilateral form. The syndrome is believed to be caused by a genetic disorder that reduces the embryonal circulation in the subclavian and vertebral arteries. We report a 20-year-old man with Poland syndrome on the left hemithorax, which is rarely associated with dextroposition.

摘要

波兰综合征的特征是胸肌部分或完全缺失、同侧并指畸形,偶尔还伴有前胸壁和乳房的其他畸形。在罕见病例中还报告了其他相关异常,包括右位心、肾脏畸形和脊柱畸形。这种情况在男性中更为常见,通常以单侧形式出现在右半胸。该综合征被认为是由一种遗传疾病引起的,这种疾病会减少锁骨下动脉和椎动脉的胚胎循环。我们报告了一名20岁男性,患有左侧胸壁波兰综合征,这种情况很少与右位心相关。

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