Mounier-Kuhn Syndrome

(aka Tracheobronchomegaly)

Epidemiology

  • First reported in 1932
  • Probably congenital (familial clustering reported)
  • Sex: more common in men
  • Age: usually presents in 20 s-30 s

Physiology

  • Congenital abnormality of the trachea and main bronchi that is characterized by atrophy or absence of elastic fibers and thinning of the smooth muscle layer
  • Flaccid Airways: show marked collapsibility during a forced expiration or during a cough
    • Ineffective cough mechanism: causes retention of mucus, chronic colonization with bacteria, and recurrent pneumonias and eventual bronchiectasis

Diagnosis

  • FOB: striking dilation of intrathoracic trachea (mean diameter >31 mm) and major bronchi (mean diameter of right MSB >24 mm/left MSB >23 mm)
    • Flaccid Airways: which collpase during cough
  • CXR/Chest CT Pattern:
    • Redundant muculomembranous tissue between cartilaginous rings appear like multiple diverticuli along lateral trachea

Clinical

  • Severe Recurrent or Chronic Pulmonary Infection:
  • Bronchiectasis (see [[Bronchiectasis]])

Treatment

  • Treat Bronchiectasis: standard therapy
  • Bronchodilators: no documented therapeutic effect

References

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