Tracheobronchomegaly

Tracheobronchomegaly
Other namesMounier-Kühn syndrome[1]
SpecialtyPulmonology Edit this on Wikidata
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. ^ RESERVED, INSERM US14-- ALL RIGHTS. "Orphanet: Mounier Kühn syndrome". www.orpha.net. Retrieved 26 May 2019.{{cite web}}: CS1 maint: numeric names: authors list (link)
  2. ^ Schwartz, W; Rossoff L (Nov 1994). "Tracheobronchomegaly". Chest. 106 (5). American College of Chest Physicians: 1589–1590. doi:10.1378/chest.106.5.1589. PMID 7956426. Archived from the original (PDF) on 2020-01-09. 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.

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