CTICasesPentalogy of Cantrell
Imaging findings
Chest CT in a newborn infant demonstrates ectopia cordis, with the heart herniating through a large midline anterior chest wall defect. Associated findings include an omphalocele, a diaphragmatic defect, a pericardial defect, and complex congenital heart disease (pulmonary atresia with a large VSD and multiple major aortopulmonary collateral arteries / MAPCAs).
Key takeaways
Pentalogy of Cantrell is a rare congenital syndrome characterized by a combination of midline defects: (1) an omphalocele or upper abdominal wall defect, (2) a lower sternal defect, (3) a diaphragmatic defect, (4) a diaphragmatic pericardial defect, and (5) congenital intracardiac anomalies. Ectopia cordis is a classic association, and the prognosis is extremely poor, depending on the severity of the cardiac anomalies.
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