Season 9 Case 13

Season 9 Jan 31, 2021

Hx: 50 yo male with cough

how about this?

Answer: Mounier-Kuhn syndrome (aka tracheobronchomegaly)

-cystic dilation of trachea&bronchi

-absence/atrophy of elastin fibers&smooth muscle

-?congenital (not agreed upon)

-assoc w Ehlers-Danlos

Mounier-Kuhn Syndrome:

-consider when trachea >3cm (above aortic arch)

-bronchi >2cm (R) or >1.5cm (L)

-can have tracheal diverticula

-dynamic imaging shows change in airway diameter ( increased w inspiration due to weakness)

-yields chronic productive cough/infection and COPD symptoms

chest

Neuro