Congenital Lung Malformations

Abstract

The development of the foregut, including the division of the esophagus from the tracheobronchial tree and the patterning and differentiation of the pulmonary anlage remains incompletely understood. Nonetheless, the last decade has made remarkable progress toward developing a more sophisticated model of the dynamic interactions of endoderm and mesoderm that give rise to the tracheobronchial tree, lung, and esophagus. Congenital cystic lesions such as congenital pulmonary airway malformation (alternatively congenital pulmonary adenomatoid malformation) (CPAM), bronchopulmonary sequestration (BPS), bronchogenic cysts, foregut duplications, and congenital lobar emphysema (CLE) arise from discrete perturbations within this interaction.