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目的:探讨支气管肺前肠畸形的诊断方法及治疗方案。方法:收集北京儿童医院2000年10月至2019年6月共17例支气管肺前肠畸形患儿的临床特点及诊疗过程。其中，男10例，女7例，年龄范围为2个月至11岁，平均年龄为3.22岁。临床表现包括咳嗽14例、进食后呛咳8例、发热5例、胸痛1例、咯血1例。17例患儿行增强CT检查均发现隔离肺，其中右下隔离肺8例、左下隔离肺7例、左上隔离肺1例、右颈部隔离肺1例；肺内隔离肺13例，肺外隔离肺4例。所有患儿中，11例为上消化道造影检查发现，1例为超声检查发现，5例为术中发现隔离肺与胃或食管异常通道；其中隔离肺与下段食管瘘13例，隔离肺与上段食管瘘2例，隔离肺与胃瘘2例。结果:支气管肺前肠畸形患儿的分类为Ⅲ型14例、Ⅱ型2例、ⅠB型1例。1例患儿确诊后未在我院继续治疗，余16例患儿行手术治疗。其中，行胸腔镜手术6例，开胸手术9例，颈部手术1例；对肺内隔离肺行肺叶切除8例，对肺外隔离肺行肺叶切除4例，因肺发育不良行全肺切除3例，肺内隔离肺行肺叶楔形切除1例。手术中出血量范围为2～60 ml，手术时间范围为50～200 min。术后出现重症肺炎而放弃治疗1例；术后出现食管瘘1例；14例术后无并发症、预后良好。结论:手术前早期、准确的诊断，对于支气管肺前肠畸形手术方式的选择和预后的判断有着重要的意义。“,”Objective:To explore the diagnosis and treatment of bronchopulmonary foregut malformation (BPFM).Methods:Clinical characteristics, diagnosis and treatment were reviewed for 17 BPFM children from October 2000 to June 2019. There were 10 boys and 7 girls with an average age of 3.22 (1/6-11) years. The clinical manifestations included cough (n=14), choking after eating (n=8), fever (n=5), chest pain (n=1) and hemoptysis (n=1). On enhanced computed tomography (CT), the sites of pulmonary sequestration were right lower (n=8), left lower (n=7), left upper (n=1) and right neck (n=1). It was intrapulmonary (n=13) and extrapulmonary (n=4). Detection was made by upper gastrointestinal contrast examination (n=11), ultrasonography (n=1) and operation (n=5). Pulmonary sequestration was associated with lower esophageal fistula (n=13), upper esophageal fistula (n=2) and gastric fistula (n=2).Results:The clinical types were ⅠB (n=1), Ⅱ (n=2) and Ⅲ (n=14). Except for one child giving up treatment, 16 cases were operated. The procedures included thoracoscopy (n=6), thoracotomy (n=9) and neck surgery (n=1). Lobectomy was performed for intrapulmonary pulmonary sequestration (n=8), extrapulmonary pulmonary pulmonary sequestration (n=4), hypoplastic lung (n=3) and intrapulmonary pulmonary sequestration (n=1). The amount of blood loss ranged from 2 to 60 ml. The operative duration was 50 to 200 min. One child abandoned treatment because of severe postoperative pneumonia. Postoperative esophageal fistula occurred in 1 case while the remainder had a decent prognosis without any complication.Conclusions:Early and accurate preoperative diagnosis is vital for selecting surgical approaches and predicating the prognosis of BPFM.