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Tracheobronchomegaly
Tracheobronchomegaly
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Tracheobronchomegaly | |
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Other names | Mounier-Kühn syndrome |
Specialty | Pulmonology |
Complications | Recurrent pulmonary infections |
Causes | atrophy of elastic fibers in the trachea and main bronchi, leading to thinning of the smooth muscle layer |
Diagnostic method | CT Chest. Tracheobroncheal flaccidity, dilatation, and/or collapse. |
Frequency | 300 cases have been reported to date |
Tracheobronchomegaly is a very rare congenital disorder of the lung primarily characterized by an abnormal widening of the upper airways. The abnormally widened trachea and mainstem bronchi are associated with recurrent lower respiratory tract infection and copious purulent sputum production, eventually leading to bronchiectasis and other respiratory complications.
Diagnosis
Woodring et al. (1991) suggested the following diagnostic criteria for tracheomegaly in adults based on chest radiography:
- Adult Males: Tracheal transverse diameter > 25 mm and sagittal diameter > 27 mm.
- Adult Females: Tracheal transverse diameter > 21 mm and sagittal diameter > 23 mm.
History
The term "Mounier-Kuhn syndrome" derives from the characterization of the condition by Prof. Pierre-Louis Mounier-Kuhn in 1932, while the name "tracheobronchomegaly" was introduced by Katz et al. in 1962.
External links
Classification | |
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External resources |
Congenital malformations and deformations of respiratory system
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Upper RT |
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Lower RT |
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