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先天性肺气道畸形(congenital pulmonary airway malformation，CPAM)是产前检查发现最常见的胎儿先天性呼吸道畸形。长期以来对于产前诊断CPAM的疾病转归和无症状患儿是否需要手术治疗的问题一直存在争议。因此为了权衡手术效益和风险，了解CPAM的自然病程发展和转归就显意义重大。本综述结合CPAM新的分类体系、产前和产后评估技术和最新的大样本研究，探讨CPAM的自然预后和治疗策略。“,”Congenital pulmonary airway malformation (CPAM) is the most common fetal congenital airway malformation during prenatal examination. For a long time, there has been a lot of controversy about the outcomes of prenatal diagnosis of CPAM and whether or not asymptomatic cases require surgery. For balancing the benefits and risks of CPAM, it is of great significance to understand the natural history and outcomes of CPAM. This review focused upon the natural prognosis and treatment strategy of CPAM based upon a new classification system, prenatal and postpartum assessment techniques and the latest large sample studies.