Antenatal Diagnosis and Management of Congenital Pulmonary Airway Malformation: Current Perspectives

Congenital pulmonary airway malformations (CPAM) are rare, heterogeneous lung lesions resulting from abnormal development of the pulmonary airways, parenchyma, and/or vasculature, likely due to obstruction. This review provides a comprehensive update on what is known about CPAM—classification, incidence, pathophysiology, antenatal diagnostic, prenatal management, and pregnancy outcomes.

CPAMs represent the most common congenital thoracic malformation, accounting for 30–47% of fetal thoracic lesions. The incidence has risen in recent decades, largely due to widespread antenatal ultrasound and enhanced diagnostic imaging. Early antenatal diagnosis enables timely counseling, perinatal management, and postnatal intervention. While infants with symptomatic lesions require surgical resection shortly after birth, the optimal timing of surgery for asymptomatic lesions remains controversial, despite concerns about potential malignant transformation and the risk of recurrent pulmonary infections. The antenatal course of CPAMs varies widely, ranging from complete regression to life-threatening hydrops fetalis, intrauterine death, and neonatal morbidity. A significant advancement in prenatal diagnosis is the CPAM Volume Ratio (CVR), which reliably predicts prognosis and guides clinical management. A CVR greater than 1.6 is associated with a high risk for developing hydrops. Corticosteroids have become the first-line therapy in hydropic fetuses or fetuses determined to be at risk for developing hydrops because of CPAM volume ratio (CVR) >1.6. However, a standardized consensus on the timing and frequency of antenatal therapy is still lacking.

This review provides clinically relevant information to antenatal diagnosis, effectively counsel patients, and management with an evidence-based approach. It emphasizes the ongoing need for innovation and further research into management strategies.