CLINICOPATHOLOGICAL FINDINGS IN CONGENITAL ANEURYSMS OF THE GREAT-VESSELS

We describe the clinical, histopathologic, and angiographic findings i n four children with congenital abnormalities of the great vessels of unknown cause, comprising either single or multiple arterial aneurysms , aortic/arterial dilatation, vessel tortuosity, or combinations of th ese abnormalities, Two children had early and severe respiratory distr ess due to aneurysmal compression of the trachea, All children had dif fuse dilatation of several arteries, and two children also had tortuos ity of multiple arteries. Progression of these abnormalities was clear ly evident in one child, in whom diffuse vessel irregularity and tortu osity affected intraabdominal, and intra and extra-cranial arteries, O ne child died at 5 years, while the other three have undergone success ful surgical repair in the first 3 months of Life and are now well, be tween age 2.5 and 7 years. The phenotype of each child appears unique but all have in common the rare finding of aneurysms of the aorta and main pulmonary artery, Congenital aortic aneurysms did not occur as an isolated finding in any of these children. (C) 1996 Wiley-Liss, Inc.