Tracheobronchiomegaly (Mounier-Kuhn syndrome)

Tracheobronchiomegaly is a rare congenital disorder due to defective development of elastic and muscle tissues in the upper airways. The characteristic radiologic features shown by bronchography include marked dilatation of the tracheobronchial system and diffuse saccular bronchiectasis. These findings and recurrent episodes of pneumonia lead to a progressive loss of pulmonary function. Even with vigorous medical therapy the prognosis is poor and severe respiratory failure eventually develops.