LUNG - Congenital cystic disease

This consists of the right lung, cut to display the cystic areas replacing most of the lung tissue; the intervening lung, where apparent, appears collapsed; the cysts are trabeculated and appear to be derived from bronchi. There are scattered adhesions on the pleural surface.
This child was assessed in 1976 in Samoa, where he was found to have extensive bronchiectatic changes throughout the entire right lung, causing him to produce copious amounts of purulent sputum, resulting in stunting of growth. His left lung appeared essentially normal. Examination showed prominent clubbing of both fingers and toes, prominence of the left anterior chest, and dullness to percussion over the entire right chest. On the grounds that this lesion was bronchiectasis, the right lung was removed. Histology showed, however, a relatively slight inflammatory component, a uniform distribution of the lesions throughout the whole lung, and the absence of glands in the walls of the bronchi, and the lesion was regarded as an example of congenital cystic disease of the lung, with multiple bronchogenic cysts of peripheral type.