Mounier-Kuhn syndrome

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[edit] Discussion of Mounier-Kuhn syndrome

  • Mounier-Kuhn syndrome is AKA tracheobronchomegaly
  • Mounier-Kuhn syndrome is a rare condition characterized by marked dilatation of the trachea and mainstem bronchi (peripheral airways are normal in caliber)
  • It is associated with atrophy of cartilaginous, muscular, and elastic components of the tracheal wall and may be seen in a variety of systemic diseases resulting in connective tissue abnormalities (i.e. Ehlers-Danlos syndrome).

[edit] Imaging Findings for Mounier-Kuhn syndrome

  • CT findings include a thinning of the tracheal wall and a tracheal diameter of more than 3 cm at 2 cm above the aortic arch.
  • Tracheal scalloping or frank diverticulosis can also be seen but is difficult to appreciate in cross section.
  • The trachea tends to collapse with forced expiration (tracheomalacia).

[edit] Images

Patient #1

[edit] See Also

[edit] External Links

Goldminer: Mounier-Kuhn syndrome

[edit] References for Mounier-Kuhn syndrome