Renal vascular hypertension in the context of Von Recklinghausen neurofibromatosis

Arterial hypertension in patients with neurofibromatosis is most often due to an associated pheochromcytoma. In rare cases the etiology of arterial hypertension is renovascular. Surgical treatment is mandatory when the stenosis is located in the proximal segment of the vessel. Angioplasty is hazardous in this setting due to the fibrotic nature of the culprit lesion. The authors report a case of ostial narrowing of the right renal artery in a16-year-old girl with severe arterial hypertension and neurofibromatosis the operation consisted of resection of the culprit lesion and reimplantation of the renal artery on the aorta Postoperatively her blood pressure returned to normal