Mounier-Kuhn syndrome, also known as tracheobronchomegaly, is a rare congenital disorder characterized by the abnormal dilation and weakening of the trachea and main bronchi. This condition is named after French physicians Pierre Mounier-Kuhn and Pierre Kuhn, who first described the syndrome in the 1930s.
In individuals with Mounier-Kuhn syndrome, the tracheal and bronchial walls become flaccid and excessively stretched, leading to significant enlargement of these airway structures. This abnormality is a result of insufficient or absent elastic fibers, which are responsible for maintaining the integrity and elasticity of the trachea and bronchi. The weakened airways may develop sac-like dilations called diverticula, further exacerbating the condition.
Common symptoms of Mounier-Kuhn syndrome include chronic cough, recurrent respiratory infections, shortness of breath, and excessive production of sputum. Affected individuals may also experience difficulty in expectorating mucus due to impaired airway clearance. In severe cases, respiratory failure and respiratory distress may occur.
The exact cause of Mounier-Kuhn syndrome is not fully understood, although genetic factors are believed to play a role. The condition is typically diagnosed through imaging studies such as computed tomography (CT) scan or bronchoscopy, which visualize the enlarged airways.
Management of Mounier-Kuhn syndrome focuses on alleviating symptoms and preventing complications. Treatment options may involve bronchodilators to improve airflow, mucolytic agents to thin out mucus, antibiotics to treat respiratory infections, and chest physiotherapy to aid in mucus clearance. In some cases, surgical interventions such as airway stenting or tracheoplasty may be necessary to improve respiratory function and
