Congenital pulmonary airway malformations: prenatal markers, perinatal outcomes, and long-term follow-up experience.

OBJECTIVE

The aim of this study was to evaluate prenatal characteristics, associated anomalies, and perinatal and postnatal outcomes of fetuses diagnosed with congenital pulmonary airway malformation at a tertiary referral center.

METHODS

This retrospective study analyzed data from 56 fetuses diagnosed prenatally with congenital pulmonary airway malformation by ultrasonography between January 2015 and December 2024. Maternal age, gestational age at diagnosis and delivery, lesion localization and ultrasonographic type, congenital pulmonary airway malformation volume ratio, presence of hydrops fetalis and associated anomalies, karyotype results, perinatal losses, postnatal surgical requirement, and long-term outcomes were evaluated.

RESULTS

The median gestational age at diagnosis was 23 weeks (range: 15-32). Lesions were located in the right lung in 58.9% and in the left lung in 41.1% of cases. Mixed lesions were most common (44.6%), followed by microcystic (30.4%) and macrocystic (25.0%) types. Congenital pulmonary airway malformation volume ratio >1.6 was observed in 17.9% of cases, and hydrops fetalis developed in 12.5%. Associated structural anomalies were detected in 12.5% of fetuses. Pregnancy termination, intrauterine fetal demise, and neonatal death occurred in 7.1, 1.8, and 1.8% of cases, respectively. Spontaneous lesion regression was observed in 62.5% of cases, while only 12% of live-born infants required postnatal surgery. During a mean follow-up of 72.5 months (range: 22-96), no congenital pulmonary airway malformation-related mortality was observed.

CONCLUSION

Most fetuses with prenatal congenital pulmonary airway malformation diagnosis have favorable perinatal and long-term outcomes. High congenital pulmonary airway malformation volume ratio and hydrops fetalis are key predictors of adverse outcomes, whereas conservative management is safe and effective in asymptomatic cases.