Management and follow-up of congenital lung malformations

INTRODUCTION: Congenital lung malformations (CLM)—including congenital pulmonary airway malformation (CPAM), bronchopulmonary sequestration (PS), congenital lobar overinflation (CLO), bronchogenic cyst (BC), and isolated congenital bronchial atresia (BA)—have shown increased prenatal diagnosis rates with the more effective use of antenatal imaging methods such as ultrasound (US) and magnetic resonance imaging (MRI). However, the optimal management of these lesions remains unclear. We aimed to investigate the diagnostic processes and management approaches in patients with CLM diagnosed at our clinic.
METHODS: This retrospective, cross-sectional study included 46 patients aged 0–18 years who were diagnosed with CLM between April 2018 and March 2025. Data on the time and method of diagnosis, presenting complaints, whether surgery was performed, and imaging modalities were recorded.
RESULTS: The median age of the patients was 60 months (IQR 62). In the prenatal diagnosis group, the median age at first presentation was 38 months (min–max=1–156) for asymptomatic patients and 6 months (min–max=0.2–132) for symptomatic patients. Sixty-three percent of cases were diagnosed prenatally, and 37% were diagnosed postnatally. Sixty-nine percent (20/29) of prenatally diagnosed cases were asymptomatic at birth. Most radiological lesions were unilobar and unilateral and did not show mediastinal shift. The most commonly affected lobe was the right lower lobe. CPAM (43.5%) was the most common diagnosis, followed by PS (17.8%). Advanced imaging, in addition to ultrasound and chest X-ray, was performed in 20% of the CPAM group and 37.5% of the PS group.
DISCUSSION AND CONCLUSION: The majority of our CLM cases were diagnosed prenatally. In prenatally diagnosed cases that are asymptomatic postnatally, the types and timing of diagnostic tests vary on a case-by-case basis, and the timing of surgical treatment remains uncertain. We believe that further prospective studies and stronger scientific evidence are needed in this field and that management decisions should be based on an individualized benefit–risk assessment.

Keywords: Computed tomography, congenital lung malformations, diagnosis, surgical treatment.