Introduction The treatment of extralobar pulmonary sequestration (ELS) remains divergent. This study aims to demonstrate the characters of ELS in children for optimal clinical management in the future. Material and Methods A retrospective analysis was conducted for ELS patients' treatment in our center from January 2013 to April 2020. Results In total, 85 patients were included, containing 70 upper‐diaphragmatic, 7 intra‐diaphragmatic, and 8 infra‐diaphragmatic ELS. Eight patients' pathology results showing inflammation without symptoms preoperation and two patients had chest pain for torsion. All the upper‐diaphragmatic and intra‐diaphragmatic ELS patients accepted thoracoscopic surgery resection. The intraoperative operation time and blood loss volume of intra‐diaphragmatic ELS were significantly more than that of the upper‐diaphragmatic (40.14 ± 9.92 vs. 23.07 ± 6.79 min; 9.29 ± 3.45 vs. 3.18 ± 4.94 ml; all p < .05). No chest tubes were inserted in both subgroups. No complications were found in the postoperative follow‐up of operative ELS patients at least 3 months. A total of eight infra‐diaphragmatic ELS patients except for one (7/8) had conservative therapy and follow‐up by the outpatient clinic or phone call eventually. There were no symptoms occurring and no size increasing of observation infra‐diaphragmatic ELS. Conclusions The ELS has the potential risk of infection and torsion in this study. Thoracoscopic surgery might be optimal management of upper‐diaphragmatic ELS for its minimal invasion and low perioperative risks, which could be developed into a day operation with safe and quick recovery. The intra‐diaphragmatic and infra‐diaphragmatic ELS need a larger sample size and multiple center data to get a better management approach.
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