Archivi giornalieri: 28 maggio 2007

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.