International Journal of Clinical Case Reports 2013, Vol.3, No.8, 39
-
41
39
Research Report Open Access
Spontaneous Hemomediastinum Associated with Neurofibromatosis 1: Report of a Case
Hilary Ambrose Enuh
1
,
Oluwaseyi Bamidale Adekunle
1
,
Jay Mbeng Nfonoyim
2
1
Department of medicine, Richmond University Medical Center, Staten Island, New York
2
Critical Care Unit, Richmond University Medical Center, Staten Island, New York
Corresponding author email:
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
Copyright: © 2013 Enuh et al. 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.
Preferred citation for this article as:
Enuh et al., 2013, Spontaneous Hemomediastinum Associated with Neurofibromatosis 1: Report of a Case, International Journal of Clinical Case Reports, Vol.3,
No.8 39-41 (doi: 10.5376/ijccr.2013.03.0008)
Abstract
A 59 years old f emale with a p ast medical h istory of n eurofibromatosis, 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 rar e cause of effusion of b lood into the mediastinum. Th e intercostal, sub clavian 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
Background
Aside from thoracic trauma and rupture of t he great
vessels, spontaneous hemomediastinum is an uncom-
mon and often life threatening event. Neurofibroma-
tosis 1 (NF1, von Recklinghausen’s disease) is a rare
cause of effusion of blood into the mediastinum. NF1
is the most common of at least eight clinical pheno-
types of neurofibromatosis occurring in about 1 out of
2 000
~5 000 individuals (Hung et al., 2012; Rasmus-
sen and Friedman, 2000). It is an autosomal dominant
disorder which arises from mutation of the gene at
chromosome 17q11.2, which is responsible for neurof-
ibromin production (Gottfried et al., 2006). This leads
to cell proliferation and inhibited apoptosis throughout
the body, especially in the nervous system (Cawthon
et al., 1990). Vascular lesions associated with NF1are
infrequent, but c an be fatal, and are characterized by
stenosis, occlusion, aneurysms, pseudoaneurysms and
rupture or fistul a formation in sm all, medium, and
large arteries (Seow et al ., 2007). The intercostal,
subclavian and bronchial arteries are the commonest
vessels involved in mediastinal hematomas (Miyazaki
et al., 2011). Only one case of i nternal mammary
artery involvement has been doc umented so far
(
Conlon et al., 2007). Here, we describe a case of
spontaneous mediastinal hemorrhage as a res ult of
pseudoaneurysmal rupture of the right internal
mammary artery.
Case Summary
A 59 years old female with a past m edical history of
neurofibromatosis, malignant peripheral nerve sheath
tumor on chemotherapy, right sided breast carcinoma
status post resection and radiation was admitted for
acute respiratory failure s econdary to health care
associated pneumonia. She was intubat ed and care d
for in the intensive care unit.
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 tomogra-
phy (CT) revealed a 9.1 cm by 6.1 cm right paramedi-
astinal hematoma and a pseudo-aneurysm arising from
the right internal mammary artery with a 5 cm hema-
toma just inf erior to it. She received four units of
packed red blood cells. In line with her health care