Bronchography in the recognition of congenital cystic bronchiectasis

Congenital cystic bronchiectasis is an uncommon disease. Most of the reported cases have been in infants and young children. This paper presents four cases of older children and younger adults who had congenital cystic bronchiectasis. Distribution of the disease is lobar or may even involve an entire lung. Bronchography plays an important role in the diagnosis of congenital cystic bronchiectasis. The three important bronchographic signs of congenital cystic bronchiectasis are: (1) absence of contrast filling of the bronchial glands duct; (2) contrast filling of numerous cysts which communicate directly with the bronchial tree; and (3) nonvisualization of normal lung parenchyma within the area of involvement.