Congenital cystic adenomatoid malformation, intralobar sequestration, extralobar sequestration, bronchopulmonary foregut malformation, bronchial atresia, and lobar emphysema generally are treated as separate entities, but there are commonalities among them.
To clarify the identity of congenital cystic adenomatoid malformation in relation to other cystic diseases.
We studied 10 consecutive cases of surgically resected cystic lung disease in children. We studied the bronchial and vascular trees by mapping bronchi, pulmonary blood vessels, systemic blood vessels, the size and configuration of cysts, and associated scarring.
The cystic lesions proved to be the following: 1 case of congenital cystic adenomatoid malformation, 2 cases of bronchial atresia with congenital cystic adenomatoid malformation, 1 hybrid case of congenital cystic adenomatoid malformation and intralobar sequestration, 1 case of intralobar sequestration with an aberrant hilum, 3 cases of intralobar sequestration with bronchial obliteration, 1 case of lung abscess, and 1 case of lobar emphysema. Congenital cystic adenomatoid malformation was the sole pathologic diagnosis in 1 case, but 7 other specimens had cystic adenomatoid change to various degrees. There was no bronchial connection between the cystic lesions and the patient's airway in 8 cases. The bronchial tree was absent in the cystic lung in 2 cases. Bronchus tapered into scar near the cystic lesion in 4 cases. Only 1 case had no significant bronchial abnormality.
The high incidence of cystic adenomatoid change in cystic lung disease associated with an abnormality of the bronchial tree suggests that cystic adenomatoid change may develop together with and be related to other congenital or acquired conditions in the lung.