Thirty-year nationwide research on epidemiology, treatment, and outcomes of congenital lung malformations

Congenital lung malformations (CLMs) may result in inflammation, malignancy, lung-dependent heart disease, and increased mortality. This study aimed to analyze the epidemiology of congenital lung malformations, changes in the prevalence, treatment patterns, and outcomes, including mortality. This nationwide retrospective research included all patients diagnosed with CLMs during 1986–2015. Histological findings, treatments, and primary outcomes were reviewed. Mortality due to CLMs was analyzed using official death certificates. The detection of CLMs increased from 1.9/100 000 live births (1990) to 23.1/100 000 live births (2015) (p < 0.0001). The detection of CPAM has increased while other CLMs have stayed unchanged. Altogether, 257 patients (77%) underwent surgery. Inflammation was identified in 53 (37.6%) histological specimens. No malignancies were detected. One adult death (previously undiagnosed) and four stillborn babies and eight neonatal deaths without surgery occurred. Two neonatal deaths occurred despite attempted surgery. The complication rate associated with operative management did not differ significantly between adults and children. The prevalence of CLMs has increased over the past 30 years, with a notable shift from sequestration and bronchogenic cysts to CPAM. The presence of inflammation in asymptomatic patients may support surgical management even in the absence of clinical symptoms.