Pentalogy of cantrell in Ile-Ife: A case report and review of the literature

Pentalogy of Cantrell is a rare congenital malformation. The aetiology is not well understood, but genetic and sporadic forms have been reported. It is a combination of five congenital defects: the heart, pericardium, diaphragm, sternum and anterior abdominal wall. Only a few cases of pentalogy of Cantrell have been reported in Nigeria. Prenatal diagnosis is possible at the beginning of the second trimester using ultrasonography. Success in the management of this condition requires expertise and a multidisciplinary approach. We report the case of a 7-day-old boy, who was referred to our hospital in Ile-Ife with a chest wall defect at birth and ectopia cordis, diaphragmatic hernia seen on ultrasound and multiple heart defects seen on echocardiography. The diagnosis of pentalogy of Cantrell was made. The severity of the cardiac defects in this case and the late referral contributed to the poor outcome of the reported case. Other difficulties in the management of this case peculiar to our environment are highlighted.