Cantrell’s pentalogy
Pentalogy of Cantrell is a rare congenital malformation characterized by midline defects, resulting from defective development in the septum transversum. The constellation of findings includes deficiency of the diaphragmatic pericardium, lower sternum, anterior diaphragm, supraumbilical abdominal wall often allowing an omphalocele as well as an intrapericardial diaphragmatic hernia, and a cardiac lesion, most often a ventricular septal defect. In severe cases, the heart herniates through the diaphragmatic defect, causing thoracoabdominal ectopia cordis. Other associated congenital cardiac lesions may include an atrial septal defect, pulmonary valve stenosis, tetralogy of Fallot, dextrocardia, anomalous pulmonary venous connection, tricuspid atresia, and truncus arteriosus.




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