Combined retroperitoneal and mediastinal fibrosis associated with variceal haemorrhage

Fibrosing mediastinitis is a rare benign disorder caused by proliferation of acellular collagen and fibrous tissue within the mediastinum. Although many cases are idiopathic, many (and perhaps most) cases in the United States are thought to be caused by an abnormal immunologic response to Histoplasma capsulatum infection. Affected patients are typically young and present with signs and symptoms of obstruction or compression of the superior vena cava, pulmonary veins or arteries, central airways, or esophagus. There may be two types of fibrosing mediastinitis: focal and diffuse. The focal type usually manifests on computed tomographic (CT) or magnetic resonance (MR) images as a localized, calcified mass in the paratracheal or subcarinal regions of the mediastinum or in the pulmonary hila. The diffuse type manifests on CT or MR images as a diffusely infiltrating, often noncalcified mass that affects multiple mediastinal compartments. CT and MR imaging play a vital role in the diagnosis and management of fibrosing mediastinitis.

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Fibrosing mediastinitis mimicking as chronic pulmonary thromboembolism

Sinha¹,

Kundaragi

Kale³

et al. 2020

BJR|case reports

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Fibrosing mediastinitis is an uncommon, benign, progressive disorder caused by proliferation of fibrous tissue within mediastinum resulting in encasement of vital mediastinal broncho-vascular structures. Due to its rarity and variable clinical presentation, it is often misdiagnosed. We are presenting a case of fibrosing mediastinitis in an Ethiopian origin young male presenting with pulmonary hypertension due to simultaneous occlusion of pulmonary vein and arteries, clinically misdiagnosed as chronic pulmonary thromboembolism.

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Combined retroperitoneal and mediastinal fibrosis associated with variceal haemorrhage

Cited by 3 publications

References 7 publications

Œdème aigu du poumon, une nouvelle complication de la médiastinite sclérosante

Œdème aigu du poumon, une nouvelle complication de la médiastinite sclérosante

Fibrosing Mediastinitis

Fibrosing mediastinitis mimicking as chronic pulmonary thromboembolism

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