Non-casual Association Between Congenital Pulmonary Airway Malformations/Primary Lung Hypoplasia and Congenital Diaphragmatic Hernia (CDH)

摘要

Congenital pulmonary airway malformations (CPAM), formerly congenital cystic adenomatoid malformations, represent the most common congenital developmental anomaly of the lung (1). This malformation is due to defects which can occur at different (embryological or fetal) stages during lung and tracheobronchial development (1). As reported by Leblanc et al. (2), CPAM classification is traditionally based on the proposition made by Stocker on the histological and morphological findings of lung lesions obtained via surgery. Stocker et al. (3) initially classified into three groups: type 1 with single or multiple large cysts of size (2 cm) containing mucus cells; type 2 with multiple medium sized cysts (2 cm), and possibly other anomalies associated; type 3 with bulky lesions with solid appearance and often mediastinal shift. Later, based on the site of lesion origin, this classification was expanded into five types by Stocker et al. (4), including also type 0 or acinar dysplasia, often lethal, and type 4 that is an acinar malformation lesion with varying sized cysts, all lined by type 1 and 2 alveolar cells with no mucous cells.