Congenital pulmonary airway malformations

**Definition:** A disease caused by failure of the bronchial structure to correctly develop during the antenatal period. This disease may present with severe respiratory distress in the newborn period, acute respiratory distress or infection later in life, or may be asymptomatic. This disease can be distinguished from other lesions and normal lung by polypoid projections of the mucosa, an increase in smooth muscle and elastic tissue within the cyst walls, an absence of cartilage in the cystic parenchyma, mucous secreting cells, and the absence of inflammation.

**Inclusions:** - Congenital honeycomb lung - Congenital polycystic disease of lung

**Exclusions:** - Cystic lung disease, acquired or unspecified