Fibrosing mediastinitis and consequent superior vena cava syndrome -
A case report
Department of Thoracic & Cardiovascular Surgery, M.D. Anderson Cancer Center, Houston, Texas, USA
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Case Report
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Abstract
Fibrosing mediastinitis is a rare, chronic inflammatory disease that has several implicated etiologies. We describe a case of a
41-year old woman who presented with the classic signs and symptoms of superior vena cava syndrome. Imaging revealed a
diffuse infiltrative mediastinal process, which on biopsy was consistent with fibrosing mediastintis.
Key words
Fibrosing mediastintis; superior vena cava (SVC) syndrome
J Thorac Dis 2012;4(4):428-430. DOI: 10.3978/j.issn.2072-1439.2012.01.01 |
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Introduction
Fibrosing mediastintis, also known as sclerosing mediastinitis,
is a rare disease characterized by diffuse fibrosis of the soft
tissues of the mediastinum which can lead to significant
morbidity secondary to obstruction of central structures.
The pathogenesis is uncertain, but can be a consequence
of granulomatous disease, most frequently histoplasmosis.
Other contributing factors include tuberculosis, sarcoidosis,
autoimmune and other fungal diseases. |
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Case report
A 41-year old diabetic, non-smoker presented with a 8 month
history of progressive swelling of her face, neck and upper
extremities. This was accompanied by headaches, distortion of
vision, dizziness on bending over, facial flushing and occasional
hemoptysis. Physical examination revealed the classic findings of
superior vena cava obstruction with severe edema in the face and
upper extremities, and obvious venous collateralization across the
chest.
A chest computed tomography demonstrated a 5 cm × 5 cm × 6.5 cm diffuse infiltrative process within the anterior mediastinum,
occluding the superior vena cava and extending into the right
hilar and subcarinal region (Figure 1). This process engulfed the
take-off of all the branch vessels of the aorta and truncus anterior.
A Positron Emission Tomography (PET) did not demonstrate any
abnormal hypermetabolism in the patient’s body.
Figure 1. Diffuse infiltrative process (indicated by arrows)
within the anterior mediastinum extending along the superior
vena cava into the right hilar and subcarinal region with
calcifications.
Laboratory tests including a panel of antibodies for
autoimmune processes, immunohistochemistry analysis for
lymphoma, fungal serology, sputum smears and cultures for acid
fast bacilli were all negative. A mediastinoscopy was performed.
Biopsy of the ‘rock hard’ mediastinal mass and paratracheal
nodes was consistent with fragments of dense fibrous collagen
and adipose tissue, with no evidence of malignancy. A pathology
review at our institution was consistent with a diagnosis of
fibrosing mediastintis.
A trial of itraconazole has been initiated and a superior vena
cava stent has been placed via trans-jugular access (Figure 2).
The patient’s severe edema and symptoms started to recede
within a day of the interventional procedure.
Figure 2. Trans-jugular passage of Kumpe catheter into the
Superior Vena cava -- balloon angioplasty and placement of
Viabahn covered stent.
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Discussion
Fibrosing mediastintis, also known as sclerosing mediastinitis,
is a rare disease characterized by accumulation and proliferation
of dense acellular collagen and fibrous tissue. The process
encases central structures in the mediastinum such as the
superior vena cava, trachea, esophagus, pulmonary vasculature
and nerves. Superior vena cava obstruction is the most frequent
complication (1), but involvement of the pulmonary artery,
phrenic nerve, recurrent laryngeal nerve, and pulmonary veins
have also been reported (2). Involvement of the proximal
coronary ostia with myocardial ischemia and infarction, has also been described (3). Cardiology consultation should be
considered in patients with chest pain.
The pathogenesis of the disease still remains elusive. It has
been seen in patients as young as 7 years of age (4). In patients
with histoplasmosis, it has been postulated that prolonged
seepage of some soluble antigen or other substance from the
involved lymph nodes causes unchecked fibrosis (5).
CT and MR imaging techniques play a vital role in the
diagnosis, work up and monitoring of the disease. They also play
a vital role in guiding either surgical or percutaneous therapy
by assessing the extent of mediastinal involvement (6). Biopsy
samples obtained by percutaneous needle technique may be
insufficient to rule out malignancy. Open biopsy with extensive
sampling is frequently required to establish a definitive diagnosis
and rule out other infiltrative lesions of the mediastinum,
including lymphoma, carcinoma, and desmoid tumors (7,8).
Steroid therapy and use of antifungal agents are generally not
useful in cases of extensive fibrosis (9). However, Inoue and
colleagues suggest that the serum IgG4 level is a good selection
indicator for steroid therapy in sclerosing mediastinitis (10).
Most reports advocate minimal intervention unless symptoms
become life- threatening (7).
The relief of superior vena cava syndrome is important in
the palliative management of such a patient. Intravascular
stents are a promising option for relief of non-neoplastic
SVC obstruction (11). Restenosis of the stent is a frequent
complication in patients with fibrosing mediastinitis. Shortand
midterm success can be achieved, but progressive fibrosing
mediastinitis remains a difficult clinical problem with repeat
dilation of stents and/or additional stent placement necessary
to maintain optimal stent patency and improvement in clinical
symptomatology (12).
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Acknowledgements
Disclosure: The authors declare no conflict of interest.
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References
Cite this article as: Kant S, Walsh GL. Fibrosing mediastinitis and
consequent Superior Vena Cava syndrome - A case report. J Thorac Dis
2012;4(4):428-430. doi: 10.3978/j.issn.2072-1439.2012.01.01
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