Mounier-Kuhn syndrome

J Bronchology Interv Pulmonol. 2014 Apr;21(2):145-9. doi: 10.1097/LBR.0000000000000068.

Abstract

Mounier-Kuhn syndrome or congenital tracheobronchomegaly is a rare clinical and radiologic condition. It is characterized by a tracheal and bronchial dilation. Fewer than 100 cases have been reported in the medical literature since the original description in 1932. The first utilization of bronchoscopy for diagnosis of this condition was recorded by Lemoine only in 1949. The cause of this condition is not clearly understood; however, histopathologic findings of atrophy of smooth muscles and elastic tissue in the trachea and main bronchi have been observed. Tracheobronchomegaly can be associated with tracheal and bronchial diverticuli.

Publication types

  • Case Reports

MeSH terms

  • Aged
  • Atrophy
  • Bronchiectasis / diagnostic imaging
  • Delayed Diagnosis
  • Diagnosis, Differential
  • Diagnostic Techniques, Respiratory System*
  • Dilatation, Pathologic
  • Diverticulum / diagnostic imaging*
  • Humans
  • Male
  • Occupational Diseases / diagnosis*
  • Pulmonary Disease, Chronic Obstructive / diagnosis*
  • Radiography
  • Rare Diseases / diagnostic imaging*
  • Rare Diseases / pathology
  • Rare Diseases / physiopathology
  • Recurrence
  • Respiratory Tract Infections / etiology
  • Tracheobronchomegaly / diagnostic imaging*
  • Tracheobronchomegaly / pathology
  • Tracheobronchomegaly / physiopathology
  • Treatment Failure