Aggressive Congenital Pulmonary Airway Malformations With Dominant Cysts

Congenital pulmonary airway malformations (CPAM) are multi-cystic masses in the thorax that contain abnormal lung tissue with highly variable presentations. Whereas some pose no considerable risk to the pregnant individual or fetus, larger or high-risk lesions carry significant morbidity and mortality that present unique diagnostic and management challenges. We present 2 cases of fetal CPAMs with dominant cysts that posed unique challenges during the pregnancy. The first had an early presentation at 13 weeks’ gestation, and the second had a rapidly increasing CPAM volume ratio. After careful consideration, both pregnant individuals underwent fetal interventions with placement of thoracoamniotic shunts. The subsequent care during the pregnancies and in the neonatal period was complex and required a multidisciplinary approach to care. These cases highlight the nuances of diagnosing and managing CPAMs in utero and after delivery. We also present a comprehensive review of CPAM literature to help guide management of the pregnancy and neonate. Optimal management requires multidisciplinary prenatal counseling, individualized delivery planning, and neonatal management.