Neurofibromatosis and hypertension

Area of Science:

• Nephrology
• Vascular Surgery
• Genetics

Background:

• Neurofibromatosis (von Recklinghausen's disease) is a genetic disorder that can affect blood vessels.
• Hypertension in young patients (<18 years) warrants investigation for underlying causes.
• Renal artery stenosis is a known cause of secondary hypertension.

Observation:

• A case of generalized neurofibromatosis associated with hypertension is presented.
• The patient had unilateral main artery stenosis, which was surgically repaired.
• Despite repair, hypertension persisted due to diffuse neurofibromatous lesions in smaller renal vessels.

Findings:

• Neurofibromatosis involving both large and small renal vessels can lead to resistant hypertension.
• Vascular neurofibromatosis is a progressive condition.
• Reconstructive surgery is preferred for renal vessel neurofibromatosis.

Implications:

• Consider neurofibromatosis in hypertensive patients under 18 with renal vascular lesions.
• Early diagnosis and management of vascular neurofibromatosis are crucial.
• Treatment strategies should address the diffuse nature of vascular involvement in neurofibromatosis.