Prenatal Intervention in High-Risk CPAM: Postnatal Outcomes After Fetal versus Standard Surgery: A Propensity Score Matched Study

Abstract

Background

Congenital pulmonary airway malformation (CPAM) is a rare fetal lung anomaly characterized by cystic lesions that can impede lung development. While smaller lesions may remain asymptomatic and are managed postnatally, larger lesions can cause severe complications such as mediastinal shift and hydrops fetalis. Fetal surgery may be indicated in these cases. This study analyzed whether fetal surgical intervention affects outcomes of subsequent postnatal surgery in CPAM patients.

Methods

A retrospective single-center cohort study was conducted on pediatric patients treated for CPAM between January 2010 and August 2024. Patients were divided into two groups: those with prenatal surgical intervention and those treated with postnatal surgery only. Propensity score matching based on gestational age, gender, birth weight, and lesion volume ratio yielded 23 matched patients: 14 in the fetal surgery group and 9 in the control group. Baseline characteristics, type of fetal intervention, intraoperative, and long-term postoperative outcomes were assessed. Multivariable regression was performed to account for confounding variables.

Results

Among 179 identified patients, 23 were included after propensity score matching: 14 in the fetal surgery group and 9 in the standard postnatal surgery group. Baseline characteristics were balanced, except for significantly higher disease severity in the fetal surgery group (hydrops 69% vs. 0%, p < 0.001; mediastinal shift 93% vs. 33%, p = 0.001). Alcohol ablation was the most common fetal intervention; however, various other prenatal procedures were also performed, with most patients undergoing multiple interventions. Tendencies but no significant differences were found in primary outcomes, including mortality (15% vs. 0%, p = 0.26) and recurrence (29% vs. 0%, p = 0.18). Secondary outcomes such as ventilation duration and intensive care stay were longer in the fetal surgery group, but not statistically significant.

Conclusion

Despite higher baseline disease severity, patients who underwent fetal intervention showed postnatal outcomes comparable to those with less severe CPAM. Fetal surgery did not appear to adversely affect surgical recovery. These findings should be interpreted with caution but may support the selective use of prenatal intervention in high-risk cases and underscore the need for further research to refine both prenatal strategies and postnatal care.

Keywords

CPAM - lung malformations - hydrops fetalis - fetal surgery - minimally invasive surgery

Ethical Approval

This study was performed in accordance with the Helsinki Declaration as revised in 2013 and was approved by the institutional ethical committee (study ID 2025–811). Informed consent was obtained from their legal guardian(s).

Authors' Contributions

Conceptualization: M.K., T.K., and M.B. Methodology: M.K. and M.B. Validation: M.K., J.E., J.B., and M.B. Formal analysis: M.K., J.B., and M.B. Investigation: M.K. and T.N. Writing—original draft preparation: M.K. Writing—review and editing: J.E., R.M., T.S., T.K., and M.B. Supervision: M.B. and T.K.

Institutional Review Board Statement

The study was conducted according to the guidelines of the Declaration of Helsinki and approved by the Institutional Ethics Committee (ID 2025–811, February 25, 2025).

Informed Consent Statement

Informed consent was obtained from all subjects involved in the study.

Data Availability Statement

The data that support the findings of this study are available from the corresponding author upon reasonable request and with permission of our ethics committee.

^* These authors contributed equally to this work.

Publication History

Received: 26 April 2025

Accepted: 25 August 2025

Accepted Manuscript online:
27 August 2025

Article published online:
05 September 2025

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