Respiratory Conditions Associated with Tracheobronchomegaly (Mounier-Kuhn Syndrome): A Study of Seventeen Cases

Abstract

Background: Mounier-Kuhn syndrome (MKS) is a rare disorder characterized by enlargement of the trachea and main bronchi and associated with recurrent respiratory tract infections. Objective: This multicenter, retrospective study was carried out to describe respiratory conditions associated with tracheobronchomegaly. Methods: Nine institutions involved in the ‘Groupe d'Endoscopie de Langue Française' (GELF) participated in this study. A standard form was used to record patient characteristics, treatments and follow-up from medical charts. Results: Seventeen patients, 53% male, aged 58 ± 18 years at diagnosis were included. Recurrent infections revealed MKS in 88% of cases. Main comorbid conditions were diffuse bronchiectasis in 88% of patients and tracheobronchomalacia in 67% of cases. The exacerbation rate was 1.5 exacerbations/patient/year. The main non-respiratory morbid condition was gastroesophageal reflux disease in 29% of cases. Interventional bronchoscopy was performed in seven patients (41%), consisting of laser (n = 2) and tracheal stenting (n = 5). Complications related to stents were observed in 80% of cases with a mean stent duration of 8 months. Four deaths, including three due to respiratory causes, occurred during follow-up. Conclusions: This is the largest series of MKS reported in the literature, showing that bronchiectasis and tracheobronchomalacia are the main associated morbid conditions that constitute a challenge for treatment.

Tracheobronchoplasty followed by bilateral lung transplantation for Mounier-Kuhn syndrome

“…The malacia leads to an inability to clear secretions and a high rate of bronchiectasis (88%), tracheal diverticulae (53%), and emphysema (55%). 3 Patients usually present in adulthood with recurrent LRTI, shortness of breath, and chronic cough. Chest radiography or tomography allows diagnosis of tracheomegaly.…”

“…Flexible bronchoscopy then confirms enlargement at rest and expiratory obstruction. 3 Dynamic compute tomography can also be used as a diagnostic adjunct although awake bronchoscopy is the diagnostic gold standard.…”

An Accidental Discovery of Tracheobronchomegaly: a Case Report

“…The clinical manifestations of TBM lack specificity; furthermore, cough, expectoration, and progressive dyspnea are common symptoms; in addition, some patients could be asymptomatic [5]. Chest CT, which plays an important role in the diagnosis of TBM, indicates the following in patients with this condition: ➀ obvious dilation of the trachea and main bronchi; ➁ thinning and softening of the tracheal walls and their expansion to form diverticula; and ➂ confinement of lesions mainly to the secondary bronchi, while the boundary between the dilated trachea, main bronchi, and the surrounding tissues remains clear [2,6,7]. According to the diagnostic criterion formulated by Menon [1], in men, when the transversal diameter and sagittal diameter of the trachea are larger than 25 mm and 27 mm, respectively, and the diameter of the left-main bronchus and right-main bronchus are larger than 18.4 mm and 21 mm, respectively, and in women, when the transversal diameter and sagittal diameter of the trachea are larger than 21 mm and 23 mm, respectively, and the diameter of left-main bronchus and right-main bronchus are larger than 17.4 mm and 19.8 mm respectively, the possibility of TBM should be considered.…”

Anesthesia for a patient with unexpected giant tracheobronchomegaly

“…The usual clinical features include a productive, chronic cough with copious, purulent sputum, low-grade fever, and symptoms consistent with chronic respiratory tract infection (dyspnea, hoarseness, and loud cough) [3]. It is characterized by flaccid and markedly dilated trachea and main bronchi on inspiration with narrowing or collapse on expiration or cough [45].…”