Tracheobronchomegaly

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Tracheobronchomegaly
Illu bronchi lungs.jpg
Trachea and bronchi
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, also known as Mounier-Kühn syndrome, is a very rare congenital disorder of the lung primarily characterized by an abnormal widening of the upper airways.[1] 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.[1][2]

Diagnosis

Woodring et al. (1991) suggested the following diagnostic criteria for tracheomegaly in adults based on chest radiography:[3]

  • 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.[4][5][6]

References

  1. 1.0 1.1 Rjimati, M.; Serraj, M.; Elbiaze, M.; Benjelloun, MC.; Amara, B. (7 July 2021). "Mounier-Kuhn syndrome (Tracheobronchomegaly): Radiological diagnosis". Radiology Case Reports. 16 (9): 2546–2550. doi:10.1016/j.radcr.2021.06.021. ISSN 1930-0433. Archived from the original on 22 December 2023. Retrieved 20 December 2023.
  2. Ataya, Ali; Harman, Eloise (2017). Rare and Interesting Cases in Pulmonary Medicine. Academic Press. p. 158. ISBN 978-0-12-809767-0. Archived from the original on 2023-12-22. Retrieved 2023-12-20.
  3. Woodring J, et al. (1999). "Congenital tracheobronchomegaly (Mounier-Kuhn syndrome)". J Thorac Imaging. 6 (1).
  4. Smith DL, Withers N, Holloway B, Collins JV (August 1994). "Tracheobronchomegaly: an unusual presentation of a rare condition". Thorax. 49 (8): 840–1. doi:10.1136/thx.49.8.840. PMC 475137. PMID 8091335.
  5. KATZ I, LEVINE M, HERMAN P (December 1962). "Tracheobronchiomegaly. The Mounier-Kuhn syndrome". Am J Roentgenol Radium Ther Nucl Med. 88: 1084–94. PMID 13958486.
  6. Mounier-Kuhn P. Dilatation de la trachée: constatations radiographiques etbronchoscopiques. Lyon Med. 1932;150:106-9.

External links

Classification
External resources