Revascularization in a 17-Year-Old Girl with Neurofibromatosis and Severe Hypertension Caused by Renal Artery Stenosis

Abstract

Renal artery stenosis caused by neurofibromatosis is a rare cause of renovascular hypertension. This hypertension can develop during childhood and is one of the leading causes of poor outcome. We report the case of a 17-year-old girl who was incidentally diagnosed with severe hypertension. During her examination for secondary hypertension, we reached a diagnosis of neurofibromatosis type 1 on the basis of a cluster of typical findings: optic nerve glioma, café au lait spots, nodular neurofibromas, and axillary freckling. Renal angiograms revealed a hemodynamically significant left renal artery stenosis (70%). Renal angioplasty with a self-expanding stent was performed one month later for rapidly progressive renal artery stenosis (90%) and uncontrolled blood pressure. Excellent blood pressure control resulted immediately and was maintained as of the 2-year follow-up evaluation. We think that percutaneous transluminal renal angioplasty can be effective in select patients who have neurofibromatosis type 1 and refractory hypertension caused by renal artery stenosis.

Keywords: Blood pressure monitoring, ambulatory/methods; hypertension, renovascular/diagnosis/etiology/radiography/therapy; neurofibromatosis 1/complications/metabolism/pathology; renal artery obstruction/complications/pathology/therapy; treatment outcome; vascular diseases/physiopathology.

Fig. 1.

Photographs show A) a flat, 7.5-cm-diameter, café au lait patch on the patient's left arm; and B) nodular cutaneous and subcutaneous fibromas on the midaxillary line and posterior thorax.

Fig. 2.

Magnetic resonance angiogram (post-contrast T1-weighted arterial time image, in axial view) shows an apparently normal left renal artery emerging from the aorta.

Fig. 3.

Renal angiogram shows extreme tortuosity and a 70%-to-80% stenosis in the mid segment of the left renal artery (arrow), poststenotic dilation of the vessel, and a small saccular pseudoaneurysm immediately after the stenosis (arrowhead).

Fig. 4.

One month after initial presentation, renal angiograms show A) a 90% left renal artery stenosis (arrow) and a saccular pseudoaneurysm (arrowhead), and B) the successful outcome of left renal artery angioplasty upon implantation of a self-expanding, drug-eluting stent (arrow).