Neurofibromatosis and renal artery stenosis: a case of familial incidence.

Hypertension as a result of proximal renal artery stenosis has been described in patients with neurofibromatosis, but a review of the English language literature showed no familial incidence of renovascular lesions in association with neurofibromatosis. We report a case of neurofibromatosis in a 24-year-old white woman with renovascular hypertension resulting from a proximal renal artery stenosis and poststenotic aneurysmal degeneration. Her sister, aged 38 years, presented similarly but without clinical evidence of neurofibromatosis. Both sisters had reversed saphenous vein bypass grafting for renal artery stenosis and showed no evidence of pheochromocytoma. Histologic examination of both surgical specimens revealed similar fibrodysplastic changes of the media and adventitia without abnormal neural tissue, findings consistent with those described in patients with neurofibromatosis.