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先天性肺气道畸形(congenital pulmonary airway malformations，CPAMs)即先天性囊性腺瘤样畸形，是最常见的先天性肺部发育畸形。但随着产前常规筛查的普及与影像诊断技术的提高，其发病率明显升高。目前针对CPAM的治疗策略尚未形成共识，肺叶切除仍是该病手术治疗的标准术式。近年来，胸腔镜解剖性肺段切除在国内逐渐开展。本文将结合国内外研究现状，从术前检查、手术技术、手术并发症、远期肺功能影响及CPAMs与肿瘤的关系等方面系统分析，为临床医生手术方案选择提供依据。“,”Congenital pulmonary airway malformations (CPAMs) (formerly congenital cystic adenomatoid malformation or CCAM) are the most common of all developmental lung malformations. With more routine use of antenatal ultrasonography and improved imaging techniques, there has been an increasing number of prenatally detected CPAMs. Though the treatment recommendation for CPAMs remains controversial, pulmonary lobectomy has been a gold standard operation at most caregiver centers. Recently thoracoscopic anatomic segmental resection is gradually gaining popularity in China. To provide rationales for clinicians to choose an ideal surgical option, the authors systematically analyzed the latest researches from multiple perspectives, including preoperative examinations, surgical techniques, surgical complications, long-term impact on lung function and the relationship between CPAMs and tumor.