J Korean Soc Pediatr Nephrol.  2013 Oct;17(2):143-148.

A Case of Moyamoya Disease Associated with Neurofibromatosis Type 1 in Patients with Renal Artery Stenosis and Hypertension

• Seo YH
• Yim HE
• Yoo KH

Abstract

Neurofibromatosis type 1 (NF-1) is an autosomal dominant neurocutaneous disorder, which can affect different organs or systems of the body, including the cardiovascular system. One of the more serious aspects of the disease relates to arterial involvement. In particular, renal artery stenosis is one of the most common vascular abnormalities in patients with NF-1, and the manifestations vary, ranging from no symptoms to end-stage renal failure. Treatment usually consists of antihypertensive drugs, percutaneous transluminal angioplasty, or surgery. Other causes of hypertension should be ruled out and the patient followed up for close monitoring and proper management. We report a case of bilateral renal artery stenosis and hypertension in a patient with moyamoya disease associated with neurofibromatosis type 1. This report discusses the literature available on the current subject, its clinical features, diagnosis, and treatment.

Keyword

Renal artery stenosis; Hypertension; Nneurofibromatosis 1; Moyamoya disease

MeSH Terms

Angioplasty
Antihypertensive Agents
Cardiovascular System
Diagnosis
Humans
Hypertension*
Kidney Failure, Chronic
Moyamoya Disease*
Neurocutaneous Syndromes
Neurofibromatoses*
Neurofibromatosis 1*
Renal Artery Obstruction*
Renal Artery*
Antihypertensive Agents