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波兰综合征伴右位心和肢体畸形的多模态影像学表现。

Multimodality imaging of Poland syndrome with dextrocardia and limb anomalies.

机构信息

Department of Radiology, Seattle Children's Hospital, Seattle, WA 98105, USA.

出版信息

Clin Nucl Med. 2012 Aug;37(8):815-6. doi: 10.1097/RLU.0b013e31824c602b.

Abstract

Poland syndrome is a rare disorder featuring unilateral anterior thoracic and upper extremity anomalies. These include hypoplasia of the pectoralis major costosternal head, absent pectoralis minor, breast hypoplasia, and absent upper rib cartilage. Pulmonary hypoplasia may occur secondarily. The postulated etiology is underdevelopment of the subclavian artery. Less than 50 cases of Poland syndrome with dextrocardia have been reported. Rib anomalies cause rightward cardiac displacement in fetal life. This case of left-sided Poland syndrome in a young girl is unique not only because of dextrocardia, but also because the condition preferentially afflicts the right side and males.

摘要

波兰综合征是一种罕见的疾病,表现为单侧前胸壁和上肢异常。这些异常包括胸大肌胸骨头发育不良、胸小肌缺失、乳房发育不全和上肋骨软骨缺失。可能会继发肺发育不全。推测的病因是锁骨下动脉发育不良。已报道的少于 50 例波兰综合征合并右位心病例。肋骨异常导致胎儿期心脏向右侧移位。这个年轻女孩左侧波兰综合征的病例不仅因为右位心,而且还因为这种情况更倾向于影响右侧和男性,是独特的。

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