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.
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