Fatal Hemorrhage as a Complication of Neurofibromatosis

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Abstract
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 reported1 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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