ABSTRACT
Congenital lung abnormalities are uncommon and diverse in their presentations. This chapter discusses the classical lesions including congenital lobar emphysema (CLE), congenital pulmonary airway malformation (CPAM), pulmonary sequestration, and bronchogenic cysts, along with several more uncommon lung anomalies. The pulmonary vascular system develops in parallel to the lung parenchyma. As the lung buds form, a pulmonary vascular plexus arises. CLE distention causes compression of adjacent normal lung parenchyma and can result in mediastinal shift and cardiorespiratory compromise. CLE is rarely diagnosed on prenatal ultrasound. Similar to CLE, polyalveolar lung results in expiratory air trapping and lobar overdistension with respiratory compromise. Physiologic consequences of CPAM can be seen antepartum and occur secondary to mediastinal shift and compression of normal lung tissue. Bronchogenic cysts are typically thick-walled, unilocular lesions, which are composed of smooth muscle, cartilage, and mucous glands lined by ciliated columnar epithelium. They are usually simple and contain fluid or mucus.
