Abstract

Congenital cystic pulmonary diseases represent a spectrum of closely related anomalies that arise during an early stage of embryonic lung bud maturation. Eleven cases of congenital cystic pulmonary diseases were reveiwed and classified based on embryologically oriented histogenesis, which was proposed by Buntain et al. (1974), and is known to be most reasonable. The results were summarized as follows : 1) Among 11 cases of congenital cystic pulmonary diseases, 8 were classified as bronchogenic cysts and 3 were as cystic adenomatoid malformation, and none of the patients with congenital cystic pulmonary disease was classified as pulmonary sequestration or congenital lobar emphysema. 2) In cases of bronchogenic cysts, which were solitary in all, 5 were located at mediastinum, 2 in the lung and one at subcutaneous tissue of the anterior neck. The cysts were lined uniformly by pseudostratified ciliated columnar epithelium. Smooth muscle fibers, submucosal mucous glands and cartilage tissue were frequently observed in the wall of the cysts. 3) The three cases of congenital cystic adenomatoid malformation were subclassified into 2 cases of type Ⅰ and 1 case of type Ⅱ by Stocker's classification(1977). Situs inversus was combined in a case of type Ⅰ congenital cystic adenomatoid malformation as an associated cogenital malformation, which might be the first case reported in the literature. The cysts were lined by ciliated columnar or cuboidal epithelium. Smooth muscle fiber and increased elastic fibers were also observed in the cyst wall.