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 o...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.展开更多
文摘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.
