Poland syndrome with dextrocardia: Case report

Abstract

Poland syndrome is a rare congenital condition presenting with typical features including an absent costosternal head of pectoralis major and ipsilateral brachysyndactyly. There are many clinical variations of the syndrome including rib defects, absence of shoulder girdle muscle and breast hypoplasia or agenesis. Dextrocardia is rarely associated with Poland Syndrome with only 22 cases being previously reported in the worldwide
literature. Whereas ‘c1assical’ Poland syndrome is predominantly right sided, all cases associated with dextrocardia have been left sided. We report a further case of left sided Poland syndrome with dextrocardia which might have important implications for the understanding of the pathogenesis of this unusual condition.