Mounier-Kuhn Syndrome: A Case Report

Abstract

Mounier-Kuhn syndrome is a rare clinical entity. It was described in 1932 to refer to the presence of tracheobronchomegaly. The clinical presentation is variable and it can occur in several different age groups. We present the case of a young man who was admitted to the hospital for an asthma exacerbation and was found to have tracheobronchomegaly on his CT scan. A review of the literature for this rare, but interesting, phenomenon is included.

Mounier–Kuhn syndrome: a case of tracheal smooth muscle remodeling

“…Moreover, several reports have described an association between Mounier–Kuhn syndrome and connective tissue diseases such as Ehlers–Danlos syndrome, Marfan syndrome, and cutis laxa 9, 10, 11, but an exact genetic etiology remains unknown. The reduction and/or atrophy of the elastic fibers of the tracheal posterior membrane are proposed to contribute to excessive tracheal collapse during expiration 12.…”

Das Mounier-Kuhn-Syndrom – eine seltene Ursache rezidivierender Bronchitiden

“…Allerdings wird bei milden klinischen Verläufen selten eine weiterführende bildgebende Diagnostik (CT oder Bronchoskopie) durchgeführt, sodass das Mounier-Kuhn-Syndrom in diesen Fällen oft nicht diagnostiziert wird. Milde klinische Verläufe werden dann als Exazerbation einer chronischen Bronchitis klassifiziert [6].…”

Mounier-Kuhn syndrome

“…Klinička slika može veoma da varira i, katkad, čak podseća na bronhijalnu astmu ili hroničnu opstrukcijsku bolest pluća 4,5 . Dijagnoza se obično postavlja na osnovu nalaza kompjuterizovane tomografije 6 .…”

“…U odsustvu znakova srčanog oboljenja, zatraženo je pulmološko ispitivanje, koje je dovelo do dijagnoze. Bolesnica nikada nije imala intenzivnije gušenje niti epizode bolesti koje bi potsećale na napad bronhijalne astme ili bronhoopstruciju drugog uzroka 4,5 . Njena bolest se pojavila kao sporadična, a nisu utvrđene druge bolesti niti poremećaji koji bi bili uzrok sekundarne traheobronhomegalije.…”