Fraser F C, Teebi A S, Walsh S, Pinsky L
Department of Human Genetics, McGill University, Montreal, Quebec, Canada.
Am J Med Genet. 1997 Dec 12;73(2):194-6. doi: 10.1002/(sici)1096-8628(19971212)73:2<194::aid-ajmg16>3.0.co;2-l.
We report on two cases of Poland sequence (defect of the pectoralis major and hand on the same side) with dextrocardia, and review the literature on such patients. In all 16 reported cases, the Poland defect was on the left side, and associated with a rib defect, whereas most cases of Poland sequence involve the right side, and few have a rib defect. The dextrocardia appeared to be a dextroposition, and was not associated with other cardiac defects, whereas isolated dextrocardias (without situs inversus) frequently are. These observations suggest that the dextrocardia associated with Poland sequence is usually secondary to it.
我们报告两例伴有右位心的波兰序列征(同侧胸大肌和手部缺陷)病例,并回顾有关此类患者的文献。在所有16例已报告病例中,波兰序列征缺陷位于左侧,并伴有肋骨缺陷,而波兰序列征的大多数病例累及右侧,且很少有肋骨缺陷。右位心似乎是心脏右旋,且与其他心脏缺陷无关,而孤立性右位心(无内脏反位)通常与之相关。这些观察结果表明,与波兰序列征相关的右位心通常是其继发表现。