Objective: The clinical implications of a postnatal chest X‐ray (CXR) in asymptomatic children with a prenatally diagnosed congenital lung malformation (CLM) are uncertain. We assessed the justification for the postnatal use of CXR in these children. Methods: We included patients with CLM confirmed through chest computed tomography angiography or histopathological analysis who were asymptomatic at birth, underwent routine postnatal CXR, and participated in our standard of care prospective structured longitudinal follow‐up program. Children with major associated morbidities were excluded. Primary outcomes were the positive and negative predictive values (PPV and NPV) of CXR findings for symptom development at 4 weeks and 6 months of age. Secondarily, we sought to establish whether CXR findings were associated with undergoing additional diagnostics during the initial observational hospital stay or prolonged postnatal hospital admission. Results: Among 121 included patients, CXR showed no abnormalities in 35 (29%), nonspecific abnormalities in 23 (19%), and probable CLM in 63 (52%). The PPV of CXR in relation to symptom development at 4 weeks and 6 months was 0.05 and 0.25, respectively. Corresponding NPVs were 0.96 and 0.91. An association was identified between CXR findings and undergoing further diagnostics during the initial observational hospital stay ( p = .047). Additional diagnostic findings did not influence clinical management. CXR findings were not associated with prolonged initial hospital stay ( p = .40). Conclusion: The routine practice of postnatal CXR in asymptomatic patients with prenatally diagnosed CLM can be omitted, as CXR findings do not influence subsequent clinical management.

Objective: We aimed to determine if the incidence of lower respiratory tract infections (LRTI) among children with asymptomatic, observationally managed congenital lung abnormalities (CLA) differed from that of symptomatic patients who underwent surgery. Second, we sought to compare the pre‐ and post‐resection incidence of LRTI in patients who underwent surgery.Methods: This retrospective cohort study included patients born between 1999 and 2021 with CLA confirmed by CT scan who were enrolled in a prospective longitudinal follow‐up program. The LRTI incidence rates at 1, 2, 5, 8, and 12 years were compared between surgically and observationally managed patients using incidence rate ratios (IRR). Differences in pre‐ and post‐resection LRTI incidence rates among patients who underwent CLA‐related surgery were assessed through IRR.Results: Among 217 included patients, 81 (37%) had undergone surgery and 136 (63%) had been observationally managed. The LRTI incidence rates did not significantly differ at any follow‐up moment between the surgical and observational groups. Among the children who underwent CLA‐related surgery, the pre‐resection LRTI incidence rates were significantly higher than the post‐resection LRTI incidence rates (IRR of 3.57, 95% confidence interval: [2.00; 6.33], p < .001).Conclusion: We could not demonstrate differences in LRTI incidence throughout childhood between patients with surgically and observationally managed CLA. We recommend discussing cases of LRTI in patients with CLA in a multidisciplinary setting, using additional diagnostics such as chest X‐ray to screen for CLA involvement, enabling a well‐considered decision on surgical resection of the lesion.

Background: Congenital lung malformations (CLM) are developmental anomalies that may predispose to respiratory morbidity. Their association with asthma remains unclear, largely due to small series and the lack of population‐based controls. Objective: To evaluate asthma prevalence and related outcomes in children with CLM compared to population controls. Methods: We conducted a population‐based case‐control study linking CLM patients from a surgical registry to provincial health data. Each CLM patient was linked to up to ten date‐of‐birth‐matched controls. Asthma was defined by ICD‐9/10 codes from age ≥5 years. Outcomes included asthma prevalence, time to first diagnosis, recurrent medical encounters, and pharmacotherapy patterns. Analyses yielded risk ratios (RR), adjusted hazard ratios (aHR), and adjusted incidence rate ratios (aIRR). Results: Forty‐nine CLM cases and 486 controls were included. Asthma was diagnosed in 19 (38.8%) CLM cases versus 76 (15.6%) controls (RR = 2.48, 95% CI [1.65–3.73]; p < 0.001). The RR did not differ between surgically and conservatively managed CLM cases (RR = 2.87, 95% CI [0.45–18.32]; p = 0.234). CLM cases had a threefold higher hazard of asthma (aHR = 3.24, 95% CI [1.84–5.71]; p < 0.001). Female cases were particularly affected (aHR = 3.92, 95% CI [1.98–7.74]; p < 0.001), with no difference observed in males (aHR = 1.98, 95% CI [0.90–4.37]; p = 0.092). Annually, CLM patients accumulated 71% more asthma‐related encounters (aIRR = 1.71, 95% CI [1.28–2.25]; p < 0.001). Conclusions: Children with CLM are at increased risk of asthma, especially females, with earlier onset and more frequent encounters. These findings highlight the need for long‐term respiratory follow‐up and objective validation of asthma diagnoses in this population.

Congenital lung malformations (CLM) comprise various developmental respiratory tract anomalies. Long-term outcomes in adulthood remain poorly defined because structured follow-up often ends after childhood. We aimed to determine the clinical, functional, and imaging characteristics of adults with CLM. A retrospective study was carried out in adults with CLM followed up at our center. We assessed clinical characteristics, spirometry measures (FEV1, FVC, FEV1/FVC, and MMEF25-75), adult body plethysmography (TLC, RV, RV/TLC), and longitudinal volumetric quantification of CLM size on computed tomography (CT). Thirty patients with CLM were included (14 diagnosed prenatally, 16 referred). Seventeen (57%) underwent surgery at a median age of 4 years (interquartile range 0-24.5). Adult symptoms occurred in 17 (57%) patients, with 86% of episodes managed conservatively. Two pulmonary malignancies (pleuropulmonary blastoma and adenocarcinoma) were identified in referrals, aged 24 and 31 respectively. Childhood spirometry showed reduced mean z-scores for FEV1 (-1.1 ± 1.6; p = 0.02) and FEV1/FVC (-1.3 ± 1.6; p = 0.01), as well as scores below the lower limit of normal (LLN; z-score < -1.64) for MMEF25-75 (-1.7 ± 1.4; p < 0.001). In adulthood, mean z-scores remained negative for all indices, with FEV1 (-1.7 ± 1.5; p < 0.001) and MMEF25-75 (-1.8 ± 1.3; p < 0.001) averaging below the LLN, while body plethysmography outcomes were mixed. Longitudinal volumetric CT analysis revealed that lesion size increased in 8 patients, fluctuated in 2, and decreased in 6. Adults with CLM show persistent functional impairments and heterogeneous lesion progression on imaging. These findings mandate a standardized, multimodal surveillance protocol beyond childhood to proactively monitor for signs of late-onset disease progression.

For many decades, congenital lung malformations (CLM) were mainly regarded as rare childhood anomalies. However, tracking the natural course of unresected lesions has revealed that affected individuals may experience complications in both childhood and adulthood, including recurrent infection, breathlessness and – albeit rarely – malignant transformation. Accordingly, accurately determining the prevalence of these symptoms and the incidence of malignant transformation is essential for counselling the growing cohort of asymptomatic adolescents and adults with coincidentally discovered CLM, as well as their families.

We aimed to synthesize current knowledge on pulmonary malignancies in adults with congenital lung malformations (CLMs), focusing on clinical presentation, treatment approaches, and outcomes.