Pentalogy of Cantrell Associated with Ectopia Cordis: A Case Report

Abstract

Pentalogy of Cantrell is a congenital anomaly of the median mesodermal constructions with a poor prognosis. It is characterized by defects of the anterior diaphragm, the lower sternum, the abdominal wall, pericardium, and various congenital heart malformations. We present a case of ectopia cordis and Pentalogy of Cantrell in a newborn of a healthy 35-year-old woman with no history of embryotoxic exposure or smoking. The infant was first diagnosed with the anomaly in the second trimester of pregnancy and was delivered at 35 weeks of gestational age via a caesarian section. Shortly after birth, he was transferred to the neonatal intensive care unit (NICU) due to progressive respiratory failure, which ultimately, along with septicemia, led to infant death on the second day. In conclusion, the Pentalogy of Cantrell should be appropriately assessed for effective prenatal counseling and postnatal management with a multidisciplinary team; since infant survival assessment and early diagnosis give the parents the option of terminating the pregnancy. Plain Language Summary Pentalogy of Cantrell (POC) is a congenital anomaly with a poor prognosis, especially in developing countries. This syndrome is a collection of five congenital midline birth anomalies (cardiac malformations, partial absence of the pericardium, anterior diaphragm hernia, lower sternum defects, and abdominal wall defects) that present a distinctive challenge for surgeons. We present a case of ectopia cordis and POC diagnosed in the second trimester and delivered by the caesarian section from a healthy 35-year-old gravid 3 with no history of embryotoxic exposure or smoking. Shortly after delivery, the baby was transferred to the neonatal intensive care unit, and mechanical ventilation was assembled due to progressive respiratory failure. Unfortunately, his general condition deteriorated , and he died on the second day. In conclusion, the POC should be appropriately assessed for effective prenatal counseling and postnatal management with a multidisciplinary team.