Tracheobronchomegaly

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Tracheobronchomegaly
Other namesMounier-Kühn syndrome [1]
Specialty Pulmonology   OOjs UI icon edit-ltr-progressive.svg
Complications Recurrent pulmonary infections
Causesatrophy 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.
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]

Contents

Diagnosis

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

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-Kühn syndrome". Orphanet. Retrieved 14 April 2025.
  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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