Pentalogy of Cantrell with Total Ectopia Cordis and a Major Omphalocele-A Case Report  

作　　者：Arturo Leonardo Delgado Kopolo Mfuneko Matongo Bangasa Dumo Ntsikelelo Mzayiya Busisiwe Mrara 

机构地区：[1]Department of Surgery,Walter Sisulu University/Nelson Mandela Academic Hospital,Umtata 5099,Eastern Cape,South Africa

出　　处：《Journal of Pharmacy and Pharmacology》2019年第12期621-622,共2页药剂与药理学（英文版）

摘　　要：Ectopia cordis(EC)is a rare malformation due to failure of maturation of the midline mesodermal components of the chest and abdomen.It can be defined as 0.1%of congenital heart diseases,and it could present isolated or could belong to the spectrum of the Pentalogy of Cantrell(PoC),which is a rare congenital disorder first described in 1958 by Cantrell.We are reporting a rare case of total ectopia cordis,associated to a major omphalocele,total agenesis of the sternum,anterior diaphragmatic deficiency,absence of pericardium,and persistence of the Ductus arteriosus,making therefore these features compatible with a full spectrum of the Pentalogy of Cantrell,encouraging us to report this case.

关 键 词：ECTOPIA cordis MAJOR OMPHALOCELE STERNUM AGENESIS anterior diaphragmatic deficiency absence of PERICARDIUM persistence of the Ductus arteriosus 

分 类 号：R73[医药卫生—肿瘤]