Tracheobronchomegaly Explained

Tracheobronchomegaly
Synonyms:	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
Diagnosis:	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]}

Notes and References

1. Web site: RESERVED . INSERM US14-- ALL RIGHTS . Orphanet: Mounier Kühn syndrome . www.orpha.net . 26 May 2019 . en.
2. Schwartz . W . Rossoff L . Tracheobronchomegaly . Chest . 106 . 5 . 1589–1590 . American College of Chest Physicians . Nov 1994 . PDF . 2008-02-27 . 7956426 . 10.1378/chest.106.5.1589 . 2020-01-09 . https://web.archive.org/web/20200109170937/http://www.chestjournal.org/cgi/reprint/106/5/1589 . dead .
3. Menon B, Aggarwal B, Iqbal A . Mounier-Kuhn syndrome: report of 8 cases of tracheobronchomegaly with associated complications . South Med J . 101 . 1 . 83–87 . 2008 . 18176298 . 10.1097/SMJ.0b013e31815d4259 .
4. J Thorac Imaging . Woodring J, etal . Congenital tracheobronchomegaly (Mounier-Kuhn syndrome). 6. 1 . 1999 .
5. 8091335 . 475137 . 49 . 8 . Tracheobronchomegaly: an unusual presentation of a rare condition . August 1994 . Smith DL, Withers N, Holloway B, Collins JV . Thorax . 840–1 . 10.1136/thx.49.8.840.
6. 13958486 . 88 . Tracheobronchiomegaly. The Mounier-Kuhn syndrome . December 1962 . KATZ I, LEVINE M, HERMAN P . Am J Roentgenol Radium Ther Nucl Med . 1084–94.
7. Mounier-Kuhn P. Dilatation de la trachée: constatations radiographiques etbronchoscopiques. Lyon Med. 1932;150:106-9.