NEUROFIBROMATOSIS AND ARTERIAL ANEURYSMS

Neurofibromatosis (NF) is a common autosomal dominant disease characte rized by the development of hamartomatous or neoplastic lesions due to the proliferation of neural crest cells. An association of aneurysmal arterial lesions with NF, which may have catastrophic complications, has been rarely reported. Our recent experiences with the diagnosis an d management of three male NF patients with aneurysms is described. A 19-year-old man with refractory hypertension due to unilateral, comple x, unreconstructable renal artery aneurysms was successfully treated b y nephrectomy. Histopathology demonstrated intramural renal artery Sch wann cell proliferation. A 44-year-old patient underwent ligation of a ruptured superior mesenteric artery aneurysm, Finally, a femoral-popl iteal artery saphenous vein bypass graft with aneurysm exclusion was p erformed in a 58-year-old man with a 3.5 cm asymptomatic popliteal art ery aneurysm. In NF, the underlying pathology in large arteries is int ramural Schwann cell proliferation with secondary fibrosis. Mesodermal dysplasia may affect small arteries resulting in stenosis, post-steno tic dilatation, or aneurysmal degeneration. Clinicians should be aware of the unusual association of NF with aneurysms, particularly the occ ult development of visceral and renal artery aneurysms. These lesions are subject to sudden rupture with potentially devastating consequence s, and they mandate a high index of suspicion in NF patients.