ABDOMINAL AORTIC-ANEURYSMS IN CHILDREN

Abdominal aortic aneurysms are rare in children. Causes include mycoti c aneurysms, vasculitides (eg, Takayasu's arteritis), connective tissu e diseases (eg, Marfan's syndrome, Ehlers-Danios syndrome, and tuberou s sclerosis) and traumatic false aneurysms. Four cases are described. Case 1 was a 12-year-old boy who presented with an acute unheralded ru pture of the subdiaphragmatic aorta accompanied by lower limb paralysi s and ischemia. Attempted repair failed because of extensive friabilit y of the large arteries. Histological evaluation confirmed cystic medi al necrosis despite Marfanoid phenotype. Cases 2 and 3 were boys aged 12 and 11 with Takayasu's arteritis who presented with hypertensive en cephalopathy and heart failure. Although both had involvement of the o rigins of the renal arteries, one aneurysm was predominantly suprarena l and the other infrarenal. Currently both children are being managed successfully with antihypertensive therapy. Case 4 was a 5-year-old gi rl who presented with hypertension and a pulsatile abdominal mass afte r treatment of infective endocarditis 18 months previously. Arteriogra phy and three-dimensional computed tomography confirmed an aneurysm (6 x 5 x 4 cm) arising from the aorta and involving the right renal arte ry, Aneurysmectomy, removal of a small ischemic right kidney, and Gore -Tex grafting resulted in cure of the hypertension and uneventful reco very. The present series confirms that rupture is a fatal complication , renovascular complications are common, and medical control of hypert ension is an essential part of management. Management strategies need to he highly individualized, and may be successful without surgical in tervention, Close clinical and ultrasound follow-up of those managed n onoperatively is essential. Copyright (C) 1996 by W.B. Saunders Compan y.