Tracheobronchomegaly

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Mounier-Kuhn syndrome
Classification and external resources
ICD-9 748.3
OMIM 275300
DiseasesDB 31501
MeSH D014137

Mounier-Kuhn syndrome (also called tracheobronchomegaly) 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.[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: Trachel transverse diameter > 21 mm and sagittal diameter > 23 mm.

History

The term "Mounier-Kuhn syndrome" derives from the characterization of the condition by Mounier-Kuhn in 1932, while the name "tracheobronchomegaly" was introduced by Katz et al. in 1962.[4][5][6]

References

  1. ↑ Schwartz, W; Rossoff L (Nov 1994). "Tracheobronchomegaly" (PDF). Chest (American College of Chest Physicians) 106 (5): 1589–1590. doi:10.1378/chest.106.5.1589. PMID 7956426. Retrieved 2008-02-27. 
  2. ↑ 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. 
  3. ↑ Woodring J et al (1999). "Congenital tracheobronchomegaly (Mounier-Kuhn syndrome)". J Thorac Imaging 6 (1). 
  4. ↑ Smith DL, Withers N, Holloway B, Collins JV (August 1994). "Tracheobronchomegaly: an unusual presentation of a rare condition". Thorax 49 (8): 840–1. PMC 475137. PMID 8091335. 
  5. ↑ 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. 
  6. ↑ Mounier-Kuhn P. Dilatation de la trachée: constatations radiographiques etbronchoscopiques. Lyon Med. 1932;150:106-9.
Congenital malformations and deformations of respiratory system (Q30–Q34, 748)
Upper RT
Lower RT
Trachea and bronchus

M: RES

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noco (c, p)/cong/tumr, sysi/epon, injr

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