Pentalogy of Cantrell with Ectopia Cordis: CT Findings

A 14-month-old girl with pentalogy of Cantrell, a very rare congenital syndrome characterized by an epigastric omphalocele and malformations of the heart, sternum, pericardium, and diaphragm, underwent echocardiography and multidetector computed tomography before surgical repair of these deformities was attempted. These tests revealed multiple cardiovascular and noncardiovascular abnormalities. After surgery, the patient's cardiovascular status was stable. Although studies have shown that echocardiography, multidetector computed tomography, and magnetic resonance imaging may each play a role in the diagnosis and management of this condition, there are few data available to support the use of one imaging modality over another.