Renovascular disease in children

Abstract

A 4-year-old girl with neurofibromatosis and a one-year history of hypertension was admitted for repeat renal arteriography and for renal vein renin determinations. Neurofibromatosis, manifested by cafe-aulait spots, cutaneous neurofibromata, and neurolipomas. had been diagnosed at age 6 months during evaluation of a large head circumference without hydrocephalus. Physical examination and growth and development were otherwise normal. The family history was negative for neurofibromatosis and hypertension. One year prior to admission, her blood pressure was 130—140/90 mm Hg during a routine checkup; she was asymptomatic at that time. Laboratory evaluation included normal urinalysis (specific gravity, 1.022; pH, 5.0; no albumin; no blood; no glucose; no ketones; and an unremarkable sediment). Urine culture showed no growth. Serum creatinine was 0.4 mg/dl; BUN, 12 mg/dl; sodium, 139 mEq/liter; potassium, 3.5 mEq/liter; chloride, 103 mEq/liter; carbon dioxide, 24 mmol/liter; calcium, 9.5 mg/dl; phosphate, 4.7 mg/dl; and blood sugar, 108 mgldl. A radionuclide renal scan showed equal function bilaterally, and an ultrasound examination of her kidneys and suprarenal areas was normal. A 'spot VMA and plasma catecholamines also were normal; peripheral plasma renin activity was 4.0 ng/ml/hour. Renal arteriography revealed an ostial stenosis of the left renal artery. Renal vein renin levels were not obtained. She was treated with a combination of hydralazine, nifedipine, and enalapril, but her blood pressure failed to return to normal. Accordingly, elective repair of the ostial stenosis was performed using a left end-to-side splenorenal artery shunt. Hypertension persisted despite the apparently satisfactory revascularization procedure. Blood pressure