Key result
Neurofibromatous involvement of the adventitia and media of a major artery led to fatal arterial rupture in a patient with Von Recklinghausen's disease.
Case Report (n=1)
Neurofibromatosis can involve the adventitia and media of major blood vessels, potentially leading to fatal arterial rupture.
Highlights fatal arterial rupture risk in neurofibromatosis; leaves open whether targeted screening improves survival.
Rupture of a major artery occurred in a patient with Von Recklinghausen's disease. The adventitia and media of the artery in the region of the rupture were invaded by neurofibroma. Numerous cases of hemorrhage into neurofibromata have been reported 1 2 , 3 , several of these with a fatal outcome. Cases have been reported describing neurofibromatous involvement of the adventitia of blood vessels with the devel opment of aneurysms 4 , 5 and hypertension 5,6 . In 1882, Frederick Von Reckling hausen reported a case of neurofibromatosis with rupture of a pulmonary artery aneurysm 7, 8. Unfortunately, microscopic examination of the vessel was not performed. We report a fatal case of rupture of a major artery secondary to neurofibromatous involvement of the adventitia and media of the vessel.
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Leier et al. (1972) conducted a case report in Von Recklinghausen's disease (Neurofibromatosis) (n=1). Neurofibromatous involvement of blood vessels was evaluated on Fatal rupture of a major artery. Neurofibromatous involvement of the adventitia and media of a major artery led to fatal arterial rupture in a patient with Von Recklinghausen's disease.
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