Gastrointestinal involvement . Secondary hypertension is a frequent finding in adults with neurofibromatosis. The cause may either be renovascular, or pheochromocytoma, or both. In affected patients under eighteen years of age, renovascular hypertension is a seven times more likely cause of hypertension than pheochromocytoma. Medical and endovascular treatments have, to date, had poor long-term outcomes.
Surgical reconstruction remains the therapy of choice.
Vascular anomalies . Almost all children with NF develop vascular lesions, most commonly involving the abdominal aorta, and the renal, internal carotid and vertebral arteries, although any artery may be affected. NF is the most common genetically determined renovascular disorder in children. The renal artery stenosis, resulting from fibromuscular dysplasia of the media, often begins at the origin of the artery from the aorta, and may extend into the intrarenal branches. Two or more neurofibromas of any type, or one plexiform neurofibroma.