目的总结小儿先天性肺囊性腺瘤样畸形(CCAM)的临床特点及诊治经验。方法回顾性分析2001年3月至2007年12月在北京儿童医院经手术和病理检查确诊为CCAM的23例患儿的临床资料。所有患儿术前均摄X线胸片并行胸部CT扫描检查。结果23例患儿中男15例,女8例。进行手术治疗时的平均年龄4.5岁,其中包括新生儿2例。术前诊断主要依靠病史和影像学检查。术前误诊4例,术前影像学检查确诊率为82.6%。依照Stocker组织病理学分型,23例患儿中Ⅰ型15例,Ⅱ型8例,没有恶变。23例均经手术治疗,证实1例为双侧病变,余均为单侧病变和累及单叶。全部病例痊愈出院,随访结果显示23例均恢复良好。结论小儿CCAM临床表现无特异性。影像学检查是发现该病的有效手段,确诊依据组织学检查,组织病理学分型可依据胸部CT结果得出大致判断。手术治疗是CCAM主要治疗手段,肺叶切除是标准术式并可取得良好的效果。 Objective To summarize the clinical features and diagnosis and treatment of congenital cystic adenomatoid deformity (CCAM) in children. Methods The clinical data of 23 children with CCAM confirmed by surgery and pathology from March 2001 to December 2007 in Beijing Children’s Hospital were analyzed retrospectively. All children underwent preoperative chest X-ray chest CT scan. Results Among the 23 cases, 15 were male and 8 were female. The mean age at surgery was 4.5 years, including 2 in newborns. Preoperative diagnosis depends mainly on medical history and imaging studies. 4 cases were misdiagnosed before operation, the diagnosis rate of preoperative imaging examination was 82.6%. According to Stocker’s histopathological classification, 15 cases of type I and 8 cases of type II had no malignant transformation. Twenty-three patients were surgically treated, and 1 patient was confirmed as bilateral lesion, with unilateral lesions and unilateral lobes. All cases were cured and discharged, the follow-up results showed that 23 cases recovered well. Conclusion The clinical manifestations of CCAM in children are not specific. Imaging examination is to find an effective means of the disease, diagnosis based on histological examination, histopathological classification based on chest CT findings can be roughly judged. Surgical treatment is the main treatment of CCAM, lobectomy is a standard technique and can achieve good results.