Mediastinitis
Borislav A. Alexiev, M.D.
Allen P. Burke, M.D.
Terminology
Mediastinitis is defined as acute or chronic inflammation of the mediastinal structures.1,2,3,4,5,6,7 Two broad categories of acute mediastinitis are acute necrotizing mediastinitis and poststernotomy mediastinitis.8 Chronic mediastinitis has been arbitrarily subdivided into two categories: (1) granulomatous mediastinitis and (2) fibrosing or sclerosing mediastinitis.8
Incidence and Clinical
Poststernotomy mediastinitis has an incidence of 0.4% to 5% and a mortality of 16.5% to 47%.1 The most frequent pathogen is Staphylococcus aureus.1 Esophageal perforation, usually iatrogenic, is the second most frequent cause of acute mediastinitis, produced by common oropharyngeal flora, with a mortality rate of 20% to 60%, depending on the time of diagnosis.1 The third most frequent cause is descending necrotizing mediastinitis, the origin being an oral caries in 60% and betahemolytic streptococcus the causative agent in 71.5% of cases.1
Fibrosing (sclerosing) mediastinitis is a rare disorder characterized by the invasive proliferation of fibrous tissue within the mediastinum.2,4,9,10 Many (and perhaps most) cases in the United States are thought to be caused by an abnormal immunologic response to Histoplasma capsulatum infection.3,11,12 Additional infectious triggers implicated in the pathogenesis of fibrosing (sclerosing) mediastinitis include other fungal and mycobacterial organisms associated with granulomatous mediastinitis.5,6
Fibrosing (sclerosing) mediastinitis may be associated sarcoidosis,13 rheumatoid arthritis,13 radiotherapy for Hodgkin disease,13 and mast cell activation disease.11 Finally, there are rare immune-mediated (idiopathic) and drug-induced (e.g., methysergide) cases of fibrosing (sclerosing) mediastinitis.6 There is overlap between IgG4-related disease and fibrosing mediastinitis, both clinically and histologically.6 Interestingly, patients with idiopathic immune-mediated fibrosing (sclerosing) mediastinitis frequently have other disease manifestations such as retroperitoneal fibrosis or Riedel thyroiditis, all of which have been associated with the IgG4-related disease spectrum.6