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一例伴右旋移位的 Poland 综合征。

A case of Poland Syndrome associated with dextroposition.

机构信息

Neonatal Intensive Care Unit, Department of Clinical and Experimental Medicine, Pediatrics, University of Ferrara, Italy.

出版信息

Ital J Pediatr. 2010 Feb 20;36:21. doi: 10.1186/1824-7288-36-21.

Abstract

Classical Poland Syndrome (PS) is characterized by unilateral, partial or complete absence of the sternocostal head of the major pectoral muscle and brachysyndactyly of fingers on the same side. We report the case of a newborn infant with dextrocardia and PS located on the left side. This association is very rare: to date only 19 cases have been described in scientific literature. In all reported cases, as in the present, the Poland defect involved the left side and was associated to rib defects, whereas most cases of PS are on the right side and few have rib defects. This case supports the view that dextrocardia follows the loss of volume of the left hemithorax caused by Poland sequence and that the combination of PS and dextrocardia is not coincidental.

摘要

经典波兰综合征(PS)的特征为单侧、部分或完全缺失胸大肌胸肋部和同侧手指短并指。我们报告了一例左侧发生右旋心和 PS 的新生儿病例。这种关联非常罕见:迄今为止,科学文献中仅描述了 19 例。在所有报道的病例中,与本例一样,波兰缺陷涉及左侧,并伴有肋骨缺陷,而大多数 PS 病例发生在右侧,很少有肋骨缺陷。该病例支持以下观点,即右旋心是由波兰序列引起的左侧胸腔容量丧失的结果,并且 PS 和右旋心的组合并非巧合。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c897/2841204/055d375af3c1/1824-7288-36-21-1.jpg

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