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Surg Radiol Anat. 2013 Oct;35(8):639-46. doi: 10.1007/s00276-013-1083-7. Epub 2013 Feb 19.

Poland syndrome: from embryological basis to plastic surgery.

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  • 1Department of Pediatric Surgery, Faculty of Medicine, Children's Medical Hospital, Comenius University in Bratislava, Limbova Street 1, 833 40, Bratislava, Slovakia.

Abstract

Poland syndrome is a rare congenital anomaly described by Sir Alfred Poland over 170 years ago. Combination of unilateral aplasia of the sternocostal head of musculus pectoralis major, and an ipsilateral hypoplastic hand with simple syndactyly and short fingers is typical for this condition. It occurs more frequent among males, and is usually situated on the right hemithorax in the unilateral form. The pathogenesis of Poland syndrome is not clear. Most of the authors assume that the etiologic insult is vascular in nature. During the sixth week of gestation, not only the pectoral mass splits (future muscles of the thorax) and intervening tissue between the finger rays of hands starts to disappear but also the vascular differentiation from six aortic arches begins. In our paper we report two cases of children with Poland syndrome, who underwent surgical procedure in the Department of Pediatric Surgery, Comenius University in Bratislava, Slovakia. Our case reports are focused on pre-operatively and also post-operatively imaging (RTG, CT, and 3D CT imaging) of the affected thorax and arm, as well as the operative reconstruction technique of abnormal ribs. We also discussed the possible embryonic backgrounds of this anomaly as well as the importance of plastic surgery resulting in patients' normal life.

PMID:
23420344
DOI:
10.1007/s00276-013-1083-7
[PubMed - indexed for MEDLINE]
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