Renovascular hypertension complicating neurofibromatosis

Three patients, ages 13 months, 13 years, and 16 years, with renovascular hypertension complicating neurofibromatosis underwent vascular reconstruction with good results. A review of these patients, plus 47 additional patients from the literature, constitutes the basis of this report. The standard intravenous pyelogram revealed abnormalities in only 46 per cent of the patients. Definitive diagnosis required angiography and selective renal vein renin determinations. Unilateral and bilateral lesions were seen with equal frequency. Associated arterial abnormalities were common. Medical treatment and balloon angioplasty failed to control the blood pressure in all patients. Surgical therapy, either nephrectomy or vascular reconstruction, cured or significantly improved 95 per cent of the patients. Although one primary goal of operation is renal salvage, 40 per cent of the patients in this review underwent nephrectomy to control their hypertension.