ABSTRACT: Tracheobronchomegaly or Mounier-Kuhn syndrome is a rare disorder characterized by marked dilatation of the trachea and main bronchi, bronchiectasis, and recurrent respiratory tract infections. The etiology of this disorder is... more
ABSTRACT: Tracheobronchomegaly or Mounier-Kuhn syndrome is a rare disorder characterized by marked dilatation of the trachea and main bronchi, bronchiectasis, and recurrent respiratory tract infections. The etiology of this disorder is uncertain. It is thought to be due to defective development of elastic and muscle tissues in the upper airways. Patients usually present with symptoms related to infection and bronchiectasis. Symptoms can range from chronic cough to severe respiratory failure requiring mechanical ventilation. However, some patients may be asymptomatic. The diagnosis is usually made on the basis of the characteristic computerized thomography scan findings. Treatment was mainly supportive with chest physiotherapy and antibiotics during infectious exacerbations. 19 years old and 36 kg weigh male patient was admitted with shortness of breath and recurrent lung infections. Chest X-ray showed marked tracheal widening, bilateral pneumothorax, and bronchiectasis. Trachea diameter was measured of 27 mm in the thoracic tomography. Because of the rarity, we report the case of a patient who was diagnosed with Mounier-Kuhn syndrome with bilateral pneumothorax (a previously non reported association) and developing respiratory failure even with simple exercise.
