Renovascular disease and hypertension in children with neurofibromatosis

Neurofibromatosis type 1 (NF1) is associated with vascular lesions, such as renal artery stenosis, and secondary hypertension. The real prevalence is largely unknown particularly in children. We observed 27 patients with NF1, mean age 12.8 years (range 4.2-24 years), for 2-10 years to assess the ass ociation of NF1 with vascular abnormalities and secondary hypertension. Pat ients were studied with angiography, 24-h blood pressure monitoring, a capt opril test, and Doppler ultrasonography of aorta and renal arteries. The pr evalence of hypertension was 18.5%; 61.5% of patients studied with angiogra phy had vascular lesions, half of whom were apparently normotensive. Howeve r; they had abnormal 24-h blood pressure monitoring, which was a first sign of poor blood pressure control. Those patients with severe hypertension (1 1.1%) were successfully treated with percutaneous transluminal angioplasty (PTA): stenosis recurred in 2 of 3 patients after a 2-year follow-up period , and was responsive to drugs. We conclude that hypertension is a frequent complication of NF1 in pediatric patients, it is usually secondary to typic al vascular lesions, and requires careful follow-up Ambulatory blood pressu re monitoring (24-h) is a sensitive method for detecting initial alteration s of the blood pressure pattern. PTA may be an effective treatment in this condition.