The Prognosis of Type 3 Congenital Pulmonary Airway Malformations May Not Be Poor: Antenatal Sonographic Evaluation and Postnatal Outcomes of Non-Cardiac Thoracic Malformations in a Large Cohort

Abstract

Background:To analyze the prenatal sonographic findings, prevalence, and outcomes of non-cardiac thoracic malformations (NCTMs). Method:This study included a retrospective analysis of electronic health records from February 2014 to March 2021. The follow-up sonographic examinations of the fetuses with diagnoses of NCTM and postnatal clinical follow-up were retrieved from electronically stored files and reports.Results: Out of 19,640 fetuses, 25 fetuses were found to have NCTM with a prevalence of 0.12%. The most common NCTM was congenital pulmonary airway malformation (CPAM) (n = 8, 32%), followed by pleural effusion (PE), pulmonary hypoplasia (PH), and congenital diaphragmatic hernia (CDH) (4 cases each, 16%), bronchopulmonary sequestration (BPS) (n = 3, 12%), congenital lymphangioma (CL), and pectus excavatum (PEx) (1 case each, 4%). All type 3 CPAM cases and BPS cases resolved spontaneously in utero, whereas all type 2 CPAM (n = 2) cases required surgery in the neonatal period and one of the type 1 cases resulted in fetal hydrops. All PE, PEx, and PH cases, and 25% of the CDH cases (n = 1) were associated with underlying abnormalities. All cases with PH (n = 4), all PE cases associated with hydrops (n = 2, 50%) resulted in exitus in utero. Conclusion:NCTMs are rare congenital anomalies with a prevalence of 0.12%. The most common type of NCTM was found as CPAM. All cases of type 3 CPAM and BPS showed spontaneous resolution in utero. The prognosis of type 3 CPAM may be favorable.