Prognosis of pentalogy of Cantrell

Abstract

Regarded as a lethal condition, pentalogy of Cantrell is a rare congenital syndrome that presents in its complete form the following five defects: defect of the abdominal wall closure at the superior part, sternal fusion defect, cardiac malformations, defect of the diaphragm, and defect of the pericardium. Using data from reported cases of survivors who have received complex treatments for the anomalies, this article aims at describing the possible ways of monitoring these pregnancies and of predicting prenatal and postnatal outcomes. The optimal treatment conditions require supervision by multidisciplinary teams in a third-level maternity with neonatal intensive care units and maternal-fetal departments and neonatal surgery department. Moreover, establishing the chronology for the corrections of the defects, solving anaesthetic problems and postnatal ventilator support are necessary for the correct treatment of this syndrome.