Clinical Perinatal Management of High-Risk Fetal Congenital Pulmonary Airway Malformation

Abstract Objectives: To summarize the clinical experience in managing high-risk fetuses with congenital pulmonary airway malformation (CPAM). Design: A retrospective analysis of integrated management clinical data was conducted for high-risk fetuses with CPAM treated at the Third Affiliated Hospital of Guangzhou Medical University and Guangdong Women and Children Health Hospital from 1st July 2015 to 30th June 2021. Data were collected before, during, and after birth. Setting: Third Affiliated Hospital of Guangzhou Medical University and Guangdong Women and Children Health Hospital. Patients: A total of 51 patients were included in the study. Intervention: Prenatal maternal glucocorticoid (GC) treatment was administered in 27 cases. Measurements and Main Results: Prenatal data showed the first diagnosis at 22.90±2.34 weeks' gestation with a prenatal ultrasound congenital pulmonary airway malformation volume ratio (CVR) value of 3.08±0.91. Lesions were diagnosed in the left lung in 27 cases (52.9%), with microcystic changes present in 12 cases (23.5%). Mediastinal shift was observed in 23 cases (45.1%), with 20 cases (39.2%) exhibiting fetal hydrops. Prenatal maternal glucocorticoid (GC) treatment was administered in 27 cases, with a survival rate of 81.4% in the GC group and 95.8% in the non-GC group, showing no statistical difference (P=0.116). Postnatal data indicated an average gestational age of 37.98±2.63 weeks, with 13.7% of patients having a 5-minute Apgar score of less than 8. Neonatal surgery was performed in 17.6% of patients, all of which were lobectomies. The survival rate for high-risk CPAM infants was 88.2%, with 70.6% showing no respiratory symptoms after birth. The presence of fetal hydrops, microcystic type, and mediastinal shift did not significantly increase the risk of death. Over the 6-year follow-up period, 12 cases (26.7%) developed pectus excavatum. Conclusions: Managing high-risk congenital cystic adenomatoid malformation (CCAM) fetuses during the perinatal period remains challenging, but the overall prognosis is generally reasonable. Glucocorticoids may have a role in improving the outcome of high-risk CPAM fetuses, although the statistical significance is still uncertain.