During a routine physical examination, a 4-year-old girl was found to have a blood pressure of 170/110 mm Hg.
Six months earlier, right facial paresis developed that cleared completely. She was a well, happy child. Her blood pressure varied between
200/100 and 160/100 mm Hg. Popliteal pressures were about 10 mm Hg higher. Ocular fundi showed hypertensive retinopathy with blurred discs and macular exudates. She had multiple café au lait spots, varying in size from 0.5 cm to several centimeters, on the torso and the extremities. There were no nodules. She had thoracic scoliosis (Fig 1).
Results of multiple urinalyses were normal, with specific gravities up to 1,023. Urine cultures were sterile. Serum chemistry levels, including BUN and creatinine were normal. Creatinine clearance was 60 ml/mn/1.73sq m. Catecholamine, vanillylmandelic acid, and urinary and blood cortisol levels were normal. A left renal arteriogram was done (Fig 2). The peripheral vein