Spontaneous Hemomediastinum Associated with Neurofibromatosis 1: Report of a Case  

Hilary Ambrose Enuh1 , Oluwaseyi Bamidale Adekunle1 , Jay Mbeng Nfonoyim2
1 Department of medicine, Richmond University Medical Center, Staten Island, New York
2 Critical Care Unit, Richmond University Medical Center, Staten Island, New York
Author    Correspondence author
International Journal of Clinical Case Reports, 2013, Vol. 3, No. 8   doi: 10.5376/ijccr.2013.03.0008
Received: 24 May, 2013    Accepted: 30 May, 2013    Published: 03 Jun., 2013
© 2013 BioPublisher Publishing Platform
This is an open access article published under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Abstract

A 59 years old female with a past medical history of neurofibromatosis, malignant peripheral nerve sheath tumor on chemotherapy was admitted for acute respiratory failure secondary to health care associated pneumonia. During the admission, she was suddenly found to be tachycardiac with decreased right sided breath sounds and a 3 mg/dl drop in hemoglobin. A chest x-ray showed a widened mediastinum. Computed tomography (CT) revealed right paramediastinal hematoma and a pseudo-aneurysm arising from the right internal mammary artery. She received four units of packed red blood cells. In line with her health care proxy�s decision, surgical intervention was withheld and she was conservatively managed for two weeks. She gradually improved, was discharged with a stable hemoglobin of 10.2 g/dl. Spontaneous hemomediastinum is an uncommon and often life threatening event. Neurofibromatosis 1 (NF1, von Recklinghausen�s disease) is a rare cause of effusion of blood into the mediastinum. The intercostal, subclavian and bronchial arteries are the commonest vessels involved in mediastinal hematomas. Only one case of internal mammary artery involvement has been documented so far. Here, we describe a case of spontaneous mediastinal hemorrhage as a result of pseudoaneurysmal rupture of the right internal mammary artery.

Keywords
Neurofibromatosis; Spontaneous hemomediastinum; Internal mammary artery rupture
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