Abstract
Fibrosing mediastinitis is a rare, often debilitating and potentially lethal disease characterized by an exuberant fibroinflammatory response within the mediastinum. Patients typically present with insidious symptoms related to compression of adjacent structures including the esophagus, heart, airways, and cardiac vessels. Fibrosing mediastinitis is most often triggered by Histoplasmosis infection; however, antifungal and anti-inflammatory therapies are largely ineffective. While structural interventions aimed at alleviating obstruction can provide significant palliation, surgical interventions are challenging with high mortality and clinical experience with percutaneous interventions is limited. Here, we will review the presentation, natural history, and treatment of fibrosing mediastinitis, placing particular emphasis on catheter-based therapies.
Keywords:
histoplasmosis; pulmonary artery stenosis; pulmonary artery stent; pulmonary vein stenosis; pulmonary vein stent.
© 2019 Wiley Periodicals, Inc.
MeSH terms
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Adolescent
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Adult
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Aged
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Airway Obstruction / diagnostic imaging
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Airway Obstruction / microbiology
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Airway Obstruction / mortality
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Airway Obstruction / therapy*
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Bronchoscopy* / adverse effects
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Bronchoscopy* / instrumentation
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Bronchoscopy* / mortality
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Child
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Endovascular Procedures* / adverse effects
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Endovascular Procedures* / instrumentation
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Endovascular Procedures* / mortality
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Female
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Histoplasmosis / diagnostic imaging
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Histoplasmosis / microbiology
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Histoplasmosis / mortality
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Histoplasmosis / therapy*
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Humans
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Male
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Mediastinitis / diagnostic imaging
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Mediastinitis / microbiology
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Mediastinitis / mortality
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Mediastinitis / therapy*
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Middle Aged
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Pulmonary Veno-Occlusive Disease / diagnostic imaging
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Pulmonary Veno-Occlusive Disease / mortality
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Pulmonary Veno-Occlusive Disease / therapy*
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Risk Factors
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Sclerosis / diagnostic imaging
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Sclerosis / microbiology
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Sclerosis / mortality
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Sclerosis / therapy*
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Stenosis, Pulmonary Artery / diagnostic imaging
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Stenosis, Pulmonary Artery / mortality
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Stenosis, Pulmonary Artery / therapy*
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Stents
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Treatment Outcome
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Young Adult