Mounier-Kuhn Syndrome (Tracheobronchomegaly): Etiology, Pathophysiology, Imaging, and Clinical Insights
Keywords: Mounier-Kuhn syndrome, Tracheobronchomegaly, Rare respiratory disorder, Bronchiectasis, Chronic lung disease, Pulmonology Introduction Mounier-Kuhn Syndrome (MKS), also known as tracheobronchomegaly , is an exceedingly rare respiratory disorder first described by Pierre Mounier-Kuhn in 1932. It is characterized by marked dilation of the trachea and main bronchi due to atrophy of the smooth muscle and elastic fibers in the airway wall . This results in recurrent respiratory infections, airway collapse, bronchiectasis, and progressive decline in pulmonary function. Although rare, the syndrome is increasingly recognized due to advancements in high-resolution imaging modalities such as CT. Given its clinical overlap with COPD , cystic fibrosis , and connective tissue disorders such as Marfan and Ehlers-Danlos syndrome, awareness of Mounier-Kuhn syndrome is crucial for accurate diagnosis and management. This column provides an expert-level comprehensive review of Mou...