Ileum-like congenital elongation of the proximal descending aorta

A case of extraordinary congenital elongation of the proximal descending aorta giving origin to multiple aortic loops, packed in the left-postero-superior mediastinum, is presented. Clinical findings, that is, mid-late systolic ejection murmur, a delayed peak of the femoral pulse, blood pressure in the legs slightly lower than in the arms, were considered noncontributory from the diagnostic point of view, since they may also be present in mild aortic coarctation and in aortic kinking. Chest roentgenogram and fluoroscopy showing 2 pulsating masses protruding to the left of the proximal descending aorta suggested the presence of a more complex aortic malformation. Only angiography provided a correct diagnosis and detected some associated abnormalities as well.