International Journal of Clinical Case Reports 2013, Vol.3, No.8, 39
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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. A six month follow up was
unremarkable (Figure 1; Figure 2; Figure 3).
Figure 1 Scout image showing right mediastinal mass
Figure 2 Pseudoaneurysm
Figure 3 Hematoma
Discussion
The incidence of vascular lesions in NF1 i s reported
as 3.6% (Rasmussen et a l., 2001). Neurofibromin is
expressed in blood vessel endothelial and smooth
muscle cells and NF1 vasculopathy may result from
an alteration of neurofibromin function in these cells
(
Hamilton and Friedman, 2001). Lessened NF1 gene
function, especially in response to arterial injury, leads
to intimal thickening and decreased luminal size
(
Lasater et al., 2010). As a result of the fragility and
dysplastic nature of t he arteries, spontaneous rupture
may occur over time. Another pathogenic mechanism
that has been endorsed is direct invasion of vessels by
adjacent neurofibromata, neurofibromasarcomas and
schwannomas (Chew et al., 2001).
Bleeding from arterial aneurysms and pseudoaneury-
sms can be lethal. When such situations are encoun-
tered, both surgical and medical treatment options are
available. In hemodynamically unstable situations,
emergent andaggressive surgical intervention is
recommended. Whereas, if the patient is hemody-
namically stable, a more conservative approach with
endovascular embolization or nonoperative manage-
ment may suffice (Pezzetta et al., 2003). Thevessels
associated with NF
-
1
are friable making surgical
control a difficult task. Therefore, whenever a patient
is reasonably stable, urgent angiography with percu-
taneous embolization may be employed and thus far
this approach has shown a superior outcome to surgi-
cal intervention (Teitelbaum et al., 1998; Kipfer et al.,
2001;
Morello et al., 2001; Dominguez et al., 2002).
Radiation is said t o have deleterious effects on the
vascular endothelium such as fibrinoid necrosis and
subendothelial edema in arteries (Hasle ton et al.,
1985).
Therefore, it is pertinent to mention radiation
was used to treat our patient for breast cancer. It is not
clear if there was an association between radiation and
the development of an internal mammary artery
aneurysm in this patient.
A high index of suspicion is required to abort the
vascular complications of NF1. The possibility of
aneurysmal rupture in a patient with neurofibromato-
sis1 presenting with an acute hemoglobin drop should
be entertained as one of the differential diagnoses.
Surveillance with CT angiography in patients with
NF1 and other condi tions that increase the ris k of
vasculopathy is recommended.
Authors’ contribution
All authors contributed significantly in drafting the manuscript.
The three authors read and approved the final manuscript.