Robert L. Lebowitz, M.D. Children' s Hospital, Harvard Medical School Boston, Massachusetts

4 Year-old Girl with Hypertension

A 4 year-old girl presented with hypertension. Ultrasonography (Figure 1) showed some anechoic areas in the lower pole of the left kidney. Superficially, this resembled lower pole hydronephrosis in a duplex kidney, possibly due to reflux. The color Doppler examination suggested renal artery stenosis. A radionuclide cystogram was normal. Left renal angiography (Figure 2) and (Figure 3) showed an aneurysm of the main left renal artery with several branch stenoses and delayed perfusion into a thinned lower pole, mimicking lower pole scarring from infection. She underwent left nephrectomy. The pathologic diagnosis was fibromuscular dysplasia.