Abstract
INTRODUCTION: Children with congenital lung disease (CLD) may suffer from long-term complications, such as impairments in lung growth, decreased total lung volume, recurrent lower respiratory tract infections and, in some cases, malignant transformation. OBJECTIVE AND METHODS: we described retrospective data on diagnostic process, clinical and functional data regarding a cohort of symptomatic and asymptomatic children with CLD followed in a single third level center in the last twenty years. RESULTS: 91 children were included in the study. Five classes of disease were examined. Bronchial tree and pulmonary abnormalities represent the most common anomalies. Despite the improved resolution of prenatal diagnosis, most of patients underwent chest CT scan to confirm the initial diagnostic suspicion. The most reported symptoms were wheezing, recurrent respiratory infections and acute respiratory failure. According to malformation type, patients underwent to surgery, endoscopic and/or medical treatment. Improvement of symptoms occurred faster in patients surgically and endoscopically treated. No statistical difference in the number of exacerbations before and after treatment was recorded, as well as no differences in spirometry values were observed among surgically and non-surgically treated children. No malignant transformation was observed in two patients with intra-lobar sequestration and hybrid lesion during the follow up period. CONCLUSION: the clinical presentation of congenital airway and lung disorders varies significantly depending on the type of malformation, making it challenging to standardize treatment strategies and follow-up programs. Based on our experience, prompt surgical or endoscopic intervention in early symptomatic children leads to faster symptom improvement and normal lung function in the follow-up period. However, further prospective studies are needed to better define optimal treatment strategies for these rare conditions, particularly for asymptomatic patients, for whom management approaches remain poorly established.