Tracheobronchomegaly

{{Infobox medical condition (new)

| name = Tracheobronchomegaly

| synonyms = Mounier-Kühn syndrome{{cite web |author= |title=Mounier-Kühn syndrome |url=https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=en&Expert=3347 |website=Orphanet |access-date=14 April 2025 |language=en}}

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| complications = Recurrent pulmonary infections

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| causes = atrophy of elastic fibers in the trachea and main bronchi, leading to thinning of the smooth muscle layer

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| diagnosis = CT Chest. Tracheobroncheal flaccidity, dilatation, and/or collapse.

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| frequency = 300 cases have been reported to date

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Tracheobronchomegaly is a very rare congenital disorder of the lung primarily characterized by an abnormal widening of the upper airways.{{cite journal |last=Schwartz |first=W |author2=Rossoff L |title=Tracheobronchomegaly |journal=Chest |volume=106 |issue=5 |pages=1589–1590 |publisher=American College of Chest Physicians |date=Nov 1994 |url=http://www.chestjournal.org/cgi/reprint/106/5/1589 |format=PDF |accessdate=2008-02-27 |pmid=7956426 |doi=10.1378/chest.106.5.1589 |archive-date=2020-01-09 |archive-url=https://web.archive.org/web/20200109170937/http://www.chestjournal.org/cgi/reprint/106/5/1589 |url-status=dead }} 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.{{cite journal |vauthors=Menon B, Aggarwal B, Iqbal A |title=Mounier-Kuhn syndrome: report of 8 cases of tracheobronchomegaly with associated complications |journal=South Med J |volume=101 |issue=1 |pages=83–87 |year=2008 |pmid=18176298 |doi=10.1097/SMJ.0b013e31815d4259 }}

Diagnosis

Woodring et al. (1991) suggested the following diagnostic criteria for tracheomegaly in adults based on chest radiography:{{cite journal| journal=J Thorac Imaging |vauthors=Woodring J, etal | title=Congenital tracheobronchomegaly (Mounier-Kuhn syndrome)| volume=6| issue=1 | year=1999 }}

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.{{cite journal | pmid = 8091335 | pmc=475137 | volume=49 | issue=8 | title=Tracheobronchomegaly: an unusual presentation of a rare condition |date=August 1994 | vauthors=Smith DL, Withers N, Holloway B, Collins JV | journal=Thorax | pages=840–1 | doi=10.1136/thx.49.8.840}}{{cite journal | pmid = 13958486 | volume=88 | title=Tracheobronchiomegaly. The Mounier-Kuhn syndrome |date=December 1962 | vauthors=KATZ I, LEVINE M, HERMAN P | journal=Am J Roentgenol Radium Ther Nucl Med | pages=1084–94}}Mounier-Kuhn P. Dilatation de la trachée: constatations radiographiques etbronchoscopiques. Lyon Med. 1932;150:106-9.