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Tracheobronchomegaly

Field	Value
name	Tracheobronchomegaly
synonyms	Mounier-Kühn syndrome
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

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. There may be copious purulent sputum production, eventually leading to bronchiectasis and other respiratory complications.

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.

Diagnosis is by medical imaging and excluding other causes of long-term cough. Typically the trachea appears wide on chest X-ray.

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.

Diagnosis

Diagnosis is by medical imaging and excluding other causes of long-term cough. Typically the trachea appears wide on chest X-ray.

Woodring et al. (1991) suggested the following diagnostic criteria for tracheomegaly in adults based on chest radiography:

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.

References

(February 2025). "State-of-the-art narrative review: Mounier-Kuhn syndrome and tracheobronchomegaly". Respiratory Medicine.
(2008). "Mounier-Kuhn syndrome: report of 8 cases of tracheobronchomegaly with associated complications". South Med J.
(1999). "Congenital tracheobronchomegaly (Mounier-Kuhn syndrome)". J Thorac Imaging.
(August 1994). "Tracheobronchomegaly: an unusual presentation of a rare condition". Thorax.
(December 1962). "Tracheobronchiomegaly. The Mounier-Kuhn syndrome". Am J Roentgenol Radium Ther Nucl Med.
Mounier-Kuhn P. Dilatation de la trachée: constatations radiographiques etbronchoscopiques. Lyon Med. 1932;150:106-9.

Wikipedia Source

This article was imported from Wikipedia and is available under the Creative Commons Attribution-ShareAlike 4.0 License. Content has been adapted to SurfDoc format. Original contributors can be found on the article history page.

congenital-disorders-of-respiratory-system rare-diseases syndromes trachea bronchus-disorders

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