Perinatal Management of High-Risk Congenital Lung Malformations: A Multicenter Analysis.

BACKGROUND

Congenital lung malformations (CLMs) are often diagnosed prenatally, with high-risk cases potentially benefiting from perinatal intervention. We report outcomes following perinatal intervention for CLMs across a national multicenter cohort.

METHODS

Retrospective analysis of prenatally diagnosed CLMs (2016-2024) from a 17-center consortium was performed. Prenatal characteristics, procedural details, and outcomes were analyzed for patients undergoing ex utero intrapartum treatment (EXIT), cesarean section-to-immediate resection (STR), or emergent resection within 24 hours of birth (ER).

RESULTS

Of 1756 CLM patients, 25 (1.4%) underwent perinatal intervention: 15 EXIT (0.9%), 4 STR (0.2%), and 6 ER (0.3%). Among EXIT cases, 11 (73%) underwent EXIT-to-resection and 4 (27%) EXIT-to-airway. Most lesions were large, with a median congenital pulmonary airway malformation volume ratio (CVR) at delivery of 2.3 (IQR 1.57-2.70). Mediastinal shift was universal (100%), while hydrops was present in 33%. Congenital pulmonary airway malformation (CPAM) was the most common pathology-based diagnosis among EXIT patients (87%). Procedural survival was 15/15 (100%) for EXIT, 4/4 (100%) for STR, and 5/6 (83%) for ER. Thirty-day survival was 13/15 (87%) for EXIT, 4/4 for STR, and 3/6 for ER. ECMO was used in 2/15 EXIT, 1/4 STR, and 1/6 ER neonates. Median NICU length of stay for survivors was 30 days (EXIT), 24 days (STR), and 25 days (ER).

CONCLUSION

Perinatal intervention for CLM remains rare. These findings provide contemporary multicenter data on EXIT, STR, and ER and may help inform prenatal counseling and delivery planning. Further study is warranted to refine patient selection for perinatal intervention and delivery modification.