Syndrome of idiopathic childhood aneurysms: a case report and review of the literature.

JVIR 2000; 11:997–1004 ANEURYSMS in childhood are rare. In the thoracic aorta, they are usually associated with coarctation of the aorta or congenital aortic valvular disease. Aneurysms of the abdominal aorta, especially if multiple, may be due to an inherent defect of collagen biosynthesis, in particular collagen III in the arterial form of Ehlers-Danlos syndrome. Rarer causes include infection, neurofibromatosis, trauma from previous umbilical artery catheterization, tuberous sclerosis, and inflammatory causes that include Kawasaki disease, polyarteritis nodosa, Takayasu disease, sarcoidosis, and giantcell arteritis. Rarely, no cause is identified and 14 cases of “idiopathic” aneurysms have been reported in the pediatric and surgical literature (1– 14) (Tables 1,2). These cases may represent a distinct clinical entity, that of a syndrome of idiopathic childhood aneurysms. We report a case of a 5-year-old patient with multiple and widespread aneurysm formation.