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Tracheobronchomegaly

Orpha number ORPHA3347
Prevalence of rare diseases <1 / 1 000 000
Inheritance
  • Autosomal recessive
Age of onset Adulthood
ICD 10 code -
MIM number
Synonym(s) Mounier-Kuhn syndrome

Summary

Tracheobronchomegaly is a disorder of unknown cause defined by dilatation of trachea and large bronchi. It has occasionally been familial or associated with a connective tissue disorder such as Ehlers-Danlos syndrome. It manifests in adult patients with tracheobronchial irritation and recurring bronchopulmonary infections due to ineffective coughing. Diagnosis is made by chest X-ray (posteroanterior and lateral), but more specifically by CT imaging on which the tracheal diameter can be measured. Tracheobronchomegaly can also result from parietal fibrosing pulmonary diseases that apply traction to the tracheal walls. *Author: Pr. J.F. Cordier (April 2002)*

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