BACKGROUND: Neurofibromatosis type 1 (NF1) is one of the most frequently inherited diseases. Vascular manifestations have been reported, mostly concerning stenosis of the renal artery or spontaneous rupture of single arteries. Also, venous aneurysms have been reported. METHODS: We present clinical and pathological findings of a young patient with NF1 with lethal bleeding due to spontaneous rupture of a lumbal and renal artery. RESULTS: High fragility of several arteries as well as veins in the abdominal and pelvic arterial and venous system resulted in a destroyed vessel structure caused by invading neurogen fibres. CONCLUSIONS: In rare cases, NF1 is associated with a severe systemic vasculopathy concerning the arterial and venous vascular system.
BACKGROUND:Neurofibromatosis type 1 (NF1) is one of the most frequently inherited diseases. Vascular manifestations have been reported, mostly concerning stenosis of the renal artery or spontaneous rupture of single arteries. Also, venous aneurysms have been reported. METHODS: We present clinical and pathological findings of a young patient with NF1 with lethal bleeding due to spontaneous rupture of a lumbal and renal artery. RESULTS: High fragility of several arteries as well as veins in the abdominal and pelvic arterial and venous system resulted in a destroyed vessel structure caused by invading neurogen fibres. CONCLUSIONS: In rare cases, NF1 is associated with a severe systemic vasculopathy concerning the arterial and venous vascular system.