Successful management of fibrosing mediastinitis with severe vascular compromise: Report of two cases and literature review

Abstract

Fibrosing mediastinitis is a rare disorder characterized by the invasive proliferation of fibrous tissue within the mediastinum. This fibrosis can result in compression of intrathoracic structures including the pulmonary vasculature leading to clinical symptoms and complications like pulmonary hypertension. Here, we present two cases of young patients with fibrosing mediastinitis complicated by pulmonary artery stenosis requiring medical and endovascular management with excellent results.

Idiopathic fibrous mediastinitis with refractory pleural effusion: a case report and literature review

“…1 , 3 , 8 FM may be an immune-mediated hypersensitivity to fungi or other antigens. 3 , 7 There are many known causes of FM. Granulomatous FM is associated with infections such as histoplasmosis, tuberculosis, blastomycosis, and cryptococcosis or with noninfectious diseases such as sarcoidosis.…”

“…11 , 14 , 17 , 18 Statistically, 75% to 95% of the lesions are focal, while 5% to 25% are bilateral. 1 , 4 , 7 The severity of stenosis of the involved vessels can be noninvasively evaluated by echocardiography, CT angiography, or magnetic resonance imaging. Although magnetic resonance imaging is inferior to CT in terms of evaluating calcification, it can accurately identify invasive mediastinal soft tissue lesions in FM and invasion of adjacent structures.…”

“…Medical interventions include steroids, methotrexate, and rituximab; autoimmune FM can be treated with corticosteroids or immunosuppressants. 7 , 15 , 20 Antifungal and antituberculosis therapy for FM with evidence of an active granuloma is not efficacious and is not currently recommended. 1 In one report, the symptoms of idiopathic FM improved significantly after treatment with high-dose steroids and rituximab.…”

“…1 In one report, the symptoms of idiopathic FM improved significantly after treatment with high-dose steroids and rituximab. 7 Patients with severe vascular occlusion with hemodynamic impairment are treated with stenting and surgical intervention (bypass occlusion). Endovascular therapy is feasible and shows good safety and technical success.…”

“…At the time of this writing, continued hormone therapy was effective for our patient. Hormone therapy has been mentioned in several previous reports, 7 , 22 but the underlying mechanism of its efficacy is rarely mentioned. We speculate that hormone therapy may reduce the concentration of inflammatory factors in the mediastinal tissue and inhibit collagen fibrosis.…”