Congenital Pulmonary Airway Malformation

Excerpt

Congenital pulmonary airway malformation (CPAM), one of the congenital lung diseases discussed under the umbrella term ‘congenital thoracic malformations,’ others being a bronchogenic cyst and pulmonary sequestration, is rare, but the most common developmental congenital anomaly of the lung. The malformation is due to abnormalities during embryogenesis and can occur at different stages during lung development, leading to anomalous bronchial morphogenesis. With new advancements and technological progress, CPAM is frequently diagnosed antenatally, and managed by a pediatric surgeon from early on. Infants with this diagnosis can present in a wide range of severity from being asymptomatic until later in life, to having respiratory distress while in the neonatal period.