目的通过对小儿先天性肺囊性腺瘤样畸形(CCAM)的临床特征、发病机制及鉴别诊断的分析,探讨患儿CCAM的影像学特点,以提高对该病的影像学认识。方法回顾性分析2011年3月-2013年3月3例经手术、病理证实CCAM患儿的临床资料、胸部CT影像学特点,复习相关文献,并对3例患儿随访2年。结果 3例CCAM患儿均累及单侧单一肺叶,且位于下叶,其中左肺下叶2例,右肺下叶1例;2例可见多个含气大囊,1例见多发蜂窝样小囊。依照Stocker组织病理学分型,3例患儿中Ⅰ型2例,Ⅱ型1例。手术证实病变均为单侧,并侵犯一个肺叶,不与支气管相通。所有患儿治愈出院,术后随访2年结果显示恢复良好,无复发。结论影像学检查为发现和诊断CCAM的主要依据,其CT表现具有特征性,根据CT表现可推测其病理分型,熟悉其临床及CT特征有助于提高对该病的诊断。 Objective To investigate the clinical features, pathogenesis and differential diagnosis of congenital cystic adenomatoid deformity (CCAM) in children and explore the imaging features of CCAM in children so as to enhance the imaging knowledge of the disease. Methods The clinical data of 3 children with CCAM confirmed by operation and pathology from March 2011 to March 2013 were retrospectively analyzed. The CT features of chest CT were reviewed and the related literatures were reviewed. Three children were followed up for 2 years. Results All three cases of CCAM involved unilateral single lobe and were located in the lower lobe, including 2 cases in the left lower lobe and 1 case in the right lower lobe. There were 2 cases of multiple air-containing capsules and 1 case of multiple-cell-like small cells bag. According to Stocker’s histopathological classification, there were 2 cases of type I and 1 case of type II in 3 cases. Surgery confirmed unilateral lesions, and violations of a lobe, not with bronchial communication. All children were cured and discharged. The follow-up of 2 years showed good recovery and no recurrence. Conclusions Imaging examination is the main basis for the detection and diagnosis of CCAM. The CT findings are characteristic. According to the CT findings, the pathological classification can be inferred. Familiar with the clinical and CT features can help to improve the diagnosis of the disease.