A 41-year-old woman was referred to our center for evaluation as a potential candidate for lung transplantation due to Mounier-Kühn syndrome with severe lung function decline. She developed cough and bronchorrhea at the age of 20 years along with dyspnea that progressed to functional class III.
Chest computed tomography showed bronchiectasis and significant airway dilation. The anteroposterior diameter of the trachea and the main bronchi were 53mm and 35mm, respectively.
We performed flexible bronchoscopy under local anesthesia in order to visualize the airway and obtain a sample for microbiological study. During the procedure, we observed severe distortion of the tracheobronchial anatomy with the formation of deep folds, tracheobronchomalacia, and abundant purulent secretions (see video in [Annex]). As far as we know, no dynamic images of cases of Mounier-Kühn of this magnitude have been published previously. Given the technical difficulties, the isolation of resistant Pseudomonas, and reports of poor progress after lung transplantation in these cases,1 we decided that the patient was not a suitable candidate to undergo the procedure.
Please cite this article as: Wainstein EJ, Las Heras MJ, Matías Castro H. Broncoscopia en un caso de síndrome de Mounier-Kühn. Arch Bronconeumol. 2020;56:46.
