Prenatal and Perinatal Outcomes in Fetuses With Macrocystic Congenital Pulmonary Airway Malformations: Thoracoamniotic Shunting Versus Expectant Management.

OBJECTIVE

Describe prenatal imaging characteristics, perinatal and postnatal outcomes in fetuses with isolated macrocystic congenital pulmonary airway malformations (CPAM) undergoing thoracoamniotic shunting (TAS) and expectant management.

METHODS

Retrospective cohort study of 123 fetuses with isolated macrocystic CPAM managed at the Ontario Fetal Center (1998-2024). Prenatal ultrasound features, perinatal outcomes, and postnatal surgical data were described for fetuses undergoing TAS and expectantly managed. Predictors of mortality were assessed in the TAS group.

RESULTS

TAS was performed in 30/123 (24%) of cases. Shunted fetuses had higher CPAM volume ratio (CVR) (1.5 vs. 0.5), larger cyst diameter (3.4 vs. 1.0 cm), mediastinal shift (58% vs. 7%), and hydrops (16.7% vs. 0%). Lesion size decreased after TAS in 80% of cases; however, survival was lower (85% vs. 100), and preterm delivery was more frequent (24% vs. 7%). Among shunted cases, CVR > 2 or persistent hydrops were associated with perinatal mortality. Neonatal respiratory support was required more often following TAS (86% vs. 25%). Most infants underwent postnatal resection, occurring earlier after TAS (0.1 vs. 7.0 months).

CONCLUSIONS

Macrocystic CPAM represents a high-risk subtype of congenital lung malformations, with a substantial proportion requiring fetal intervention, neonatal respiratory support, and postnatal surgical resection. These findings support tertiary surveillance and delivery.