Fibrosing mediastinitis (FM) is a rare disease of slowly progressive fibrosis that encases major mediastinal structures. Complications include obstruction of mediastinal vasculature, including main pulmonary arteries, which may lead to life-threatening hemodynamic compromise. In North America, the disease is usually a sequela of histoplasmosis. Because Histoplasma capsulatum (H. capsulatum) is found in the Ohio River Valley, physicians in Pennsylvania must be able to recognize and diagnose the disease.
The patient is a 59 year-old female who was seen for follow-up in April 2013. She originally presented to the outpatient office in 2008 with chronic chest pain and shortness of breath. At that time her chest pain was constant, stabbing, non-pleuritic, located over the right anterior chest wall with radiation to the back, and was relieved with narcotic pain medications. She also had dyspnea on exertion. She described these symptoms as having been present for two years and noted that they had been getting progressively worse. On review of symptoms, she also noted light headedness with standing, occasional palpitations, and a dry cough. She denied weight loss, fevers, chills, hemoptysis, or lower extremity edema.
Her past medical history was significant for a pulmonary embolism at the age of 18, treated with warfarin, multiple episodes of pneumonia from 1999 to 2007, and a diagnosis of superior vena cava (SVC) syndrome in 2008. Her social history was significant for a 10 pack year history of cigarette smoking. Her family history was significant for her father with myocardial infarction at age 50.
On her most recent physical exam her vitals were temperature 99.1°F, blood pressure 110/82 mm Hg, pulse of 72 beats per minute, and oxygen saturation of 97% on room air. She was without cervical lymphadenopathy and lacked neck or face swelling. Her lungs were clear to auscultation, and auscultation of her heart revealed regular rate and rhythm and normal heart sounds with no murmurs, rubs or gallops. There was no cyanosis, clubbing or edema of the extremities. Her skin exam was notable for prominent veins of the upper chest wall and dilated tortuous abdominal wall veins.
SVC syndrome was diagnosed in 2008 when she presented to her primary care provider with left neck swelling. CT of her neck at the time showed a heterogeneous density in the left external jugular vein which was thought to be a thrombus; however, upper and lower extremity Dopplers were negative for acute thrombus. Further evaluation with CT of her chest with contrast showed SVC occlusion with dense calcification, suggestive of chronic inflammatory or post-radiation etiology (Figure 1). The patient, however, denied any history of radiation exposure or central vein catheterization. Interestingly, the CT of the chest also showed a chronic calcified occlusion of the right upper lung (RUL) pulmonary artery. She also had RUL ground-glass opacities with a calcified granuloma (Figure 2), and calcified mediastinal and hilar nodes, prominent venous collateral formation in the chest wall and mediastinum, and calcifications of her spleen (Figures 3,4).
Molnar, MD, Esther
"A Case of Fibrosing Mediastinitis,"
The Medicine Forum:
Vol. 15, Article 11.
Available at: http://jdc.jefferson.edu/tmf/vol15/iss1/11