Fatal Hemorrhage as a Complication of Neurofibromatosis

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 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 aneurysms⁴, 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.

Get full access to this article

View all access options for this article.

References

Carrington G. , Bullitt J. : Hemorrhage in a Case of Von Recklinghausen's Disease. JAMA 87: 166 — 167. 1926

Heuer G. , Bell H. : Hemorrhage into and Infection of Large Tumors (Pachydermatoceles) of Von Recklinghausen's Disease. Ann Surg 94: 15-24,1931

Gordon S. : Hemorrhage Complicating Von Recklinghausen's Disease. Can Med Assoc J 27: 524-525, 1932

Knight J. , Cancilla P. : Neurofibroma involving the Superior Vena Cava with Formation of an Aneurysm . Arch Path 86: 427-430, 1968

Cornell S. , Kirkendall W. : Neurofibromatosis of the Renal Artery. Radiology 88: 24-28, 1967

Halpern M. , Currarino G. : Vascular Lesions causing Hypertension in Neurofibromatosis. New Eng J Med 273: 248-251, 1965

Recklinghausen F.V. : Uber die Multiplen Fibrome der Haut und ihre Beziehung zu den Multiplen Neuromen . Berlin (August Hirschwald) pp 3-18, 1882

Wilkins R. , Brody I. : Von Recklinghausen's Neurofibromatosis Arch Neurol 24: 374 — 377, 1971