It is a syndrome complex that consists of an upper anterior abdominal wall defect with an ectopia cordis. The cardinal features are upper anterior abdominal wall defect, lower anterior sternal defect, anterior defect of the diaphragm, diaphragmatic pericardial defect and associated congenital cardiac anomalies. The prognosis of the condition is poor, and if at all a favourable outcome always depends on the severity of the cardiac anomalies that coexisted in that condition. The exact pathology of the condition is not identified, however many associations have been described.
