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Pentalogy of
Cantrell with Total Ectopia Cordis and a Major Omphalocele—A Case Report
Arturo Leonardo Delgado, Kopolo Mfuneko Matongo, Bangasa Dumo, Ntsikelelo Mzayiya and Busisiwe Mrara
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DOI:10.17265/2328-2150/2019.12.003
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.
Ectopia cordis, major omphalocele, sternum agenesis, anterior diaphragmatic deficiency, absence of pericardium, persistence of the Ductus arteriosus.
Arturo L. Delgado, et al. 2019. “Pentalogy of Cantrell with Total Ectopia Cordis and a Major Omphalocele—A Case Report.” Journal of Pharmacy and Pharmacology 7 (12): 621-622.