Surgical treatment of three cases of Cantrell's syndrome

Abstract

This ten-year retrospective study was designed to examine the morbidity and mortality of three cases of Cantrell's syndrome between 1998 and 2008. The three patients showed different degrees of Cantrell's pentalogy including abdominal ectopia cordis, thoracic-abdominal ectopia cordis and left ventricular diverticulum. Of the three, the 5-month-old boy suffering from complicated congenital heart disease with abdominal ectopia cordis received a successful single stage repair and reconstruction of the abdominal wall. The 33-week-old premature girl with thoracic-abdominal ectopia cordis underwent two stage correction of tetraology of Fallot. The 4-year-old girl underwent ectomy of left ventricular diverticulum and thoracoabdominal wall repair. Twenty-four to thirty-five months follow-up were satisfactory. We hold that two-stage repair are technically feasible for Cantrell's syndrome, especially for those with complex congenital heart diseases. Post-operative ventilatory support and multiple post-operative care should be prolonged. Malnutrition, infection and arrhythmia are central problems in medical care and surgery should be considered if there was progressive heart failure or hemodynamic instability.