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Research in Pediatrics & Neonatology

Pentalogy of Cantrell With Total Ectopia Cordis and A Major Omphalocele. A Case Report

Submission: October 14, 2019; Published: October 21, 2019

ISSN : 2576-9200
Volume3 Issue5


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 a 0.1% of congenital heart diseases, it could present isolated or belonging to the spectrum of the Pentalogy of Cantrell (PoC), this 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.

Keywords: Ectopia cordis; Major Omphalocele; Sternum agenesis; Anterior diaphragmatic deficiency; Absence of pericardium; Persistence of the Ductus arteriosus

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