Tracheobronchomegaly
| Tracheobronchomegaly | |
|---|---|
| Other names | Mounier-Kühn syndrome[1] |
| Specialty | Pulmonology |
| 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 | <500 reported cases as of 2025[1] |
Tracheobronchomegaly is a rare lung condition characterised by abnormal widening of the trachea and main bronchi, typically presenting with no symptoms, or a long-standing cough or recurrent chest infections.[1] There may be copious purulent sputum production, eventually leading to bronchiectasis and other respiratory complications.[1]
It may be acquired secondary to another lung disease or medical lung procedure, but when no cause is identified it is presumed congenital and is known as Mounier-Kühn syndrome.[1]
Diagnosis is by medical imaging and excluding other causes of long-term cough.[1] Typically the trachea appears wide on chest X-ray.[1]
Signs and symptoms
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.[2]
Diagnosis
Diagnosis is by medical imaging and excluding other causes of long-term cough.[1] Typically the trachea appears wide on chest X-ray.[1]
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
- ^ a b c d e f g h i Sharma, Shivang; Kuperberg, Stephen J. (February 2025). "State-of-the-art narrative review: Mounier-Kuhn syndrome and tracheobronchomegaly". Respiratory Medicine. 237 107914. doi:10.1016/j.rmed.2024.107914. ISSN 1532-3064. PMID 39710278.
- ^ 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.
- ^ 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.