ABSTRACT

Cantrell’s Pentalogy (CP) is a rare congenital syndrome characterized by defects of lower sternum, anterior diaphragm and pericardium with complete or partial ectopia cordis, an omphalocele and congenital intracardiac defects. Few cases manifesting all the defects have been reported, howe-ver, only a small number have survived as the outcome mainly depends on the cardiac malforma-tion. Herein, we report a case of most severe form of CP manifesting all defects and associated with complete thoracoabdominal ectopia cordis to discuss the challenges in management of this uncommon entity.

Keywords: Ectopia cordis, Cantrell`s pentalogy, omphalocele