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Tracheobronchomegaly

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Tracheobronchomegaly
Other namesMounier-Kühn syndrome[1]
ComplicationsRecurrent pulmonary infections
Causesatrophy of elastic fibers in the trachea and main bronchi, leading to thinning of the smooth muscle layer
Diagnostic methodCT Chest. Tracheobroncheal flaccidity, dilatation, and/or collapse.
Frequency300 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.[2] 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.[3]

Diagnosis

Woodring et al. (1991) suggested the following diagnostic criteria for tracheomegaly in adults based on chest radiography:[4]

  • 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.[5][6][7]

References

  1. Mounier-Kuhn P. Dilatation de la trachée: constatations radiographiques etbronchoscopiques. Lyon Med. 1932;150:106-9.

External links

Classification
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