Tracheobronchomegaly

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]

Tracheobronchomegaly
Other namesMounier-Kühn syndrome[1]
SpecialtyPulmonology 
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

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. RESERVED, INSERM US14-- ALL RIGHTS. "Orphanet: Mounier Kühn syndrome". www.orpha.net. Retrieved 26 May 2019.
  2. Schwartz, W; Rossoff L (Nov 1994). "Tracheobronchomegaly" (PDF). Chest. American College of Chest Physicians. 106 (5): 1589–1590. doi:10.1378/chest.106.5.1589. PMID 7956426. Retrieved 2008-02-27.
  3. Menon B, Aggarwal B, Iqbal A (2008). "Mounier-Kuhn syndrome: report of 8 cases of tracheobronchomegaly with associated complications". South Med J. 101 (1): 83–87. doi:10.1097/SMJ.0b013e31815d4259. PMID 18176298.
  4. Woodring J, et al. (1999). "Congenital tracheobronchomegaly (Mounier-Kuhn syndrome)". J Thorac Imaging. 6 (1).
  5. 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.
  6. 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.
  7. Mounier-Kuhn P. Dilatation de la trachée: constatations radiographiques etbronchoscopiques. Lyon Med. 1932;150:106-9.


This article is issued from Wikipedia. The text is licensed under Creative Commons - Attribution - Sharealike. Additional terms may apply for the media files.