CANTRELLS-SYNDROME - A CHALLENGE TO THE SURGEON

We present a case of partial Cantrell's syndrome with ventricular sept al defect, left ventricular diverticulum, dextrorotation of the heart, an anterior diaphragmatic defect, and a midline supraumbilical abdomi nal wall defect with omphalocele. At the age of 20 months, the patient underwent a successful cardiac surgical procedure. To detect risk fac tors and to define therapeutic strategies, we analyzed the spectrum an d the frequency of malformations described in 153 patients with Cantre ll's syndrome. Despite modem surgical standards, Cantrell's syndrome r epresents a challenge to the surgeon because of the wide spectrum of a nomalies, the severity of the abdominal and cardiac malformations, and the high mortality. (C) 1998 by The Society of Thoracic Surgeons.