Galiwango G W, Swan M C, Nyende R, Hodges A M
Department of Plastic and Reconstructive Surgery, CoRSU Hospital, Kisubi, Kampala, P.O. Box 46, Kisubi, Uganda.
East Afr Med J. 2010 Nov;87(11):469-72.
Poland syndrome is a rare congenital condition presentingwith typical features including an absent costosternal head of pectoralis major andipsilateral 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 'classical' 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.
波兰综合征是一种罕见的先天性疾病,具有典型特征,包括胸大肌肋胸骨头缺如和同侧短指并指畸形。该综合征有许多临床变异,包括肋骨缺损、肩胛带肌肉缺如以及乳房发育不全或发育异常。右位心很少与波兰综合征相关,全球文献中此前仅报道过22例。虽然“经典”波兰综合征主要发生在右侧,但所有与右位心相关的病例均为左侧。我们报告了另一例伴有右位心的左侧波兰综合征病例,这可能对理解这种罕见疾病的发病机制具有重要意义。