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

• 作者: Arturo Leonardo Delgado Bangasa Dumo Busisiwe Mrara Kopolo Mfuneko Matongo Ntsikelelo Mzayiya
• 来源期刊: 药剂与药理学:英文版
• 学科: 医学
• 关键词: ECTOPIA cordis MAJOR OMPHALOCELE STERNUM AGENESIS anterior diaphragmatic deficiency absence of PERICARDIUM persistence of the Ductus arteriosus
• 年，卷（期）: 2019,（12）
• 页码范围: 621-622
• 页数: 2页
• 分类号: R73

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.