Pentalogy of Cantrell; complete expression in a nine‑month‑old‑boy

Abstract

Pentalogy of Cantrell is a rare congenital malformation whose exact cause is not completely understood; it is characterized by defects in the anterior abdominal and thoracic walls consisting of omphalocoele, diaphragmatic defect, ectopia cordis, intracardiac defects and sternal clefts. The complex has variable clinical expression with complete and incomplete expressions reported. We, therefore, report a case of complete manifestation of the pentad in a 9‑month‑old boy.

Keywords: Cantrell, ectopia cordis, omphalocoele, pentalogy, sternal cleft

Nigerian Medical Journal | Vol. 54 | Issue 3 | May-June | 2013