Medicine:Tracheobronchomegaly
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| Tracheobronchomegaly | |
|---|---|
| Other names | Mounier-Kühn syndrome[1] |
| Complications | Recurrent pulmonary infections |
| Causes | atrophy 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. |
| Frequency | 300 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
- ↑ RESERVED, INSERM US14-- ALL RIGHTS. "Orphanet: Mounier Kühn syndrome" (in en). https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=en&Expert=3347. Retrieved 26 May 2019.
- ↑ 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. http://www.chestjournal.org/cgi/reprint/106/5/1589. Retrieved 2008-02-27.
- ↑ "Mounier-Kuhn syndrome: report of 8 cases of tracheobronchomegaly with associated complications". South Med J 101 (1): 83–87. 2008. doi:10.1097/SMJ.0b013e31815d4259. PMID 18176298.
- ↑ "Congenital tracheobronchomegaly (Mounier-Kuhn syndrome)". J Thorac Imaging 6 (1). 1999.
- ↑ "Tracheobronchomegaly: an unusual presentation of a rare condition". Thorax 49 (8): 840–1. August 1994. doi:10.1136/thx.49.8.840. PMID 8091335.
- ↑ "Tracheobronchiomegaly. The Mounier-Kuhn syndrome". Am J Roentgenol Radium Ther Nucl Med 88: 1084–94. December 1962. PMID 13958486.
- ↑ Mounier-Kuhn P. Dilatation de la trachée: constatations radiographiques etbronchoscopiques. Lyon Med. 1932;150:106-9.
External links
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