小儿先天性囊性肺疾病传统上分为先天性囊性腺瘤样畸形、支气管源性囊肿、肺隔离症和先天性肺叶气肿。近年来,产前超声广泛开展和对该类疾病病理研究的深入,对这类疾病有了新的认识。回顾文献发现,以往的定义和分类不再合适,现综述小儿先天性囊性肺疾病的研究进展并提出新的分类方法。 Congenital cystic lung disease in children is traditionally divided into congenital cystic adenomatoid deformity, bronchogenic cysts, pulmonary sequestration and congenital lobar emphysema. In recent years, prenatal ultrasound is widely carried out and pathological studies of these diseases in-depth, have a new understanding of these diseases. Review the literature found that the previous definition and classification is no longer appropriate, are reviewed in children with congenital cystic lung disease research advances and proposed a new classification method.