Objective To improve counseling on congenital lung malformations (CLM) by describing long‐term outcomes of children either operated on or managed by observation. Study design We analyzed lung function (spirometry), exercise tolerance (Bruce treadmill), and physical growth of 8‐year‐old children with CLM who participated in our longitudinal prospective follow‐up program. The data are shown as median standard deviation scores (SDS) with IQR, or estimated marginal means (95% CI) on the basis of general linear models. Results Twenty‐nine (48%) of the 61 children had required surgery at a median age of 108 (IQR: 8‐828) days, and 32 (52%) were managed by observation. In the surgery group, all lung function measurements (except for forced vital capacity [FVC]) were significantly below 0 SDS, with median FEV1 −1.07 (IQR: −1.70 to −0.56), FEV1/FVC −1.49 (−2.62 to −0.33), and FEF25%‐75% −1.95 (−2.57 to −0.63) (all P < 0.001). Children in the observation group had normal FEV1 and FVC, whereas FEV1/FVC (−0.81 (−1.65 to −0.14)) and FEF25%‐75% (−1.14 (−1.71 to −0.22)) were significantly below 0 SDS (both P < 0.001). Mean exercise tolerance was significantly below 0 SDS in both groups (observation: −0.85 (95% CI: −1.30 to −0.41); surgery: −1.25 (−1.69 to −0.80)); eight (28%) children in the observation group and ten (40%) in the surgery group scored <−1 SDS. Physical growth was normal in both groups. Conclusion Children with CLM may be at risk for reduced lung function and exercise tolerance, especially those who required surgery. As little pulmonary morbidity was found in children with asymptomatic CLM, this study supports a watchful waiting approach in this group.

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