Renovascular hypertension associated with neurofibromatosis: a case report

Although vascular involvement in type 1 neurofibromatosis (NF1) is rare, it may lead to renal artery stenosis and renovascular hypertension (RVH). RVH may be treated using antihypertensive drugs, percutaneous transluminal renal angioplasty (PTRA), surgical reconstruction of the renal artery, or nefrectomy. In NF1 the results of PTRA are less predictable than in cases of fibromuscular dysplasia and atherosclerosis. We report a case of RVH associated with NF1. Despite administration of multiple antihypertensive drugs blood pressure remained uncontrolled. Selective left renal arteriography demonstrated two consecutive high-grade stenotic lesions with post-stenotic aneurysmal dilatation treated successfully with balloon dilatation. During the ensuing 2 year follow up complete normalization of blood pressure was observed. This case illustrates that endovascular therapy may be beneficial and should be considered a reasonable first option in these patients. However vascular involvement in NF may be progressive and therefore always requires continuing follow up.