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J Vasc Surg. 2005 Mar;41(3):539-43.

Renal artery stenosis and aneurysms associated with neurofibromatosis.

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Division of Vascular Surgery, Department of Surgery, State University of New York at Stony Brook, Stony Brook, NY 11794, USA.


Renal artery lesions associated with neurofibromatosis may involve stenosis and aneurysm formation at all levels of the renal artery to the intraparenchymal branches, and usually are associated with hypertension. A 13-year-old boy with type I neurofibromatosis and severe hypertension presented with multiple aneurysms and multiple stenotic lesions in the renal artery and segmental arteries. The patient underwent ex-vivo renal artery repair with autologous hypogastric artery and autotransplantation to the iliac fossa and was clinically improved. The characteristic histologic findings are presented. A review of the recent literature comparing different treatment modalities for renovascular hypertension in children with neurofibromatosis suggests that surgery remains the best treatment alternative.

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