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Tracheobronchomegaly
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Tracheobronchomegaly

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Tracheobronchomegaly
Other names Mounier-Kühn syndrome
Specialty Pulmonology Edit this on Wikidata
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.

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