Abstract

The clinical, radiological, and physiological findings in 16 patients with extensive bronchiectasis due to deficiency in bronchial cartilage are discussed. These features do not occur in other types of bronchiectasis and comprise a distinctive syndrome.

The main clinical features are persistent cough and wheezy breathing. The symptoms are first noticed in infancy often after mild respiratory infection, but at times after measles or pneumonia. Most of the children develop chest deformity and clubbed fingers, and are small in stature.

The radiological findings are pulmonary hyperinflation, air-filled dilated bronchi, and, bronchographically, the bronchi balloon during inspiration and collapse with expiration.

Physiologically the outstanding features are gross increase in residual lung volume, severe impairment of maximum expiratory flow rates, especially at low lung volumes, a marked decrease in conductance in the upstream segment, poor static elastic recoil, and dynamic compliance which is frequency dependent.

It is postulated that this syndrome is due primarily to extensive developmental deficiency of bronchial cartilage, with secondary recurrent respiratory infection.