Mounier-Kuhn syndrome or tracheobronchomegaly: a case report and literature review

Abstract

Objective To improve the knowledge of tracheobronchomegaly (TBM, Mounier-Kuhn syndrome). Methods One patient with TBM was analyzed, and the related literatures were reviewed. Results ①TBM was an extremely rare disease, although the etiology was unknown, most scholars believed that TBM was a congenital disorder.②TBM was characterized by a congenital defect or atrophy of the elastic and smooth muscle tissue of the trachea and main bronchi. Pulmonary function tests showed obstructive ventilatory dysfunction.③The onset of TBM was slowly, and its most usual clinical manifestations included recurrent cough and sputum, and progressive dyspnea. Given the non-specific clinical pattern, the misdiagnosis rate of TBM is probably higher.④High-resolution CT scan of the chest was used for the diagnoses, which showed the trachea and the main bronchi marked dilatation.⑤Treatmeent was mainly supportive with chest physiotherapy and antibiotics. Conclusions TBM should be considered for patients who have recurrent pulmonary infection. A careful analysis of the chest radiograph of these patients is required. Raising awareness of the disease and diagnosis, it is important to help the disease diagnosis. Key words: Tracheobronchomegaly; Diagnosis; Therapy