Tracheobronchomegaly
| Tracheobronchomegaly | |
|---|---|
 | |
| Trachea and bronchi | |
 | |
| 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, also known as Mounier-Kühn syndrome, is a very rare congenital disorder of the lung primarily characterized by an abnormal widening of the upper airways.[1] 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.[1][2]
Diagnosis
Woodring et al. (1991) suggested the following diagnostic criteria for tracheomegaly in adults based on chest radiography:[3]
- 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.[4][5][6]
References
- ↑ 1.0 1.1 Rjimati, M.; Serraj, M.; Elbiaze, M.; Benjelloun, MC.; Amara, B. (7 July 2021). "Mounier-Kuhn syndrome (Tracheobronchomegaly): Radiological diagnosis". Radiology Case Reports. 16 (9): 2546–2550. doi:10.1016/j.radcr.2021.06.021. ISSN 1930-0433. Archived from the original on 22 December 2023. Retrieved 20 December 2023.
- ↑ Ataya, Ali; Harman, Eloise (2017). Rare and Interesting Cases in Pulmonary Medicine. Academic Press. p. 158. ISBN 978-0-12-809767-0. Archived from the original on 2023-12-22. Retrieved 2023-12-20.
- ↑ Woodring J, et al. (1999). "Congenital tracheobronchomegaly (Mounier-Kuhn syndrome)". J Thorac Imaging. 6 (1).
- ↑ 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.
- ↑ 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.
- ↑ Mounier-Kuhn P. Dilatation de la trachée: constatations radiographiques etbronchoscopiques. Lyon Med. 1932;150:106-9.
External links
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