Renovascular hypertension associated with neurofibromatosis: two cases andreview of the literature

The authors report two cases of renovascular hypertension associated with n eurofibromatosis. A 19-year-old woman was admitted to our hospital with a c omplaint of abdominal pain and blood pressure of 180/120 mmHg. Examination revealed cafe-au-lait: spots over her chest and extremities. Peripheral pla sma renin activity (PRA) under basal conditions was 2.8 ng/ml/h and increas ed to 12.6 ng/ml/h after administration of 50 mg captopril. Plasma and urin ary catecholamines were normal. Selective renal angiography showed left ane urysmal dilatation of the segmentary branch and right renal artery stenosis with multiple aneurysmal affecting different branches. Blood pressure was controlled by multiple drugs, including P-blockers and angiotensin-converti ng enzyme inhibitor. Another patient, a 20-year-old woman, was admitted bec ause of severe arterial hypertension, numerous cafe-au-lait: spots, scolios is, and mass over the right arm. PRA from the right renal vein was extremel y elevated, and selective angiography demonstrated bilateral renal artery s tenosis. Aortorenal bypass was performed successfully.