摘要
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 experience of diagnosis and treatment of congenital cystic adenomatoid malformation (CCAM) of the lung in children. Methods Between March 2001 and December 2007, 23 children with CCAM confirmed by operation and pathology in Beijing Children' s Hospital were analyzed retrospectively. All of 23 cases underwent chest X-ray and CT scan examination before operation. Results Of 23 cases, 15 were males and 8 were females with a mean age of 4.5 years at operation, including 2 newborns. The preoperative diagnosis was based on history, clinical presentation, and radiological findings and 4 patients were misdiagnosed. There are four kinds of image appearances detected in the chest radiology. The preoperative diagnosis rate of image examination approached to 82.6 %. Histopathological examination showed fifteen cases were classified as Stocker type I lesion and eight as type II , without malignancy. All patients with CCAM got complete cure by operation. Lesions were unilateral and involved single lobe in all but one patient. The postoperative follow-up showed an excellent recovery. Conclusion Early diagnosis and prompt management are required for CCAM in children. Imaging examination is a valuable method to hint CCAM, while the definite diagnosis depends on pathological examination. Stocker' s subtypes can be predicted from CT findings. Surgical resection, commonly lobectomy, is the main treatment of choice in all cases of CCAM in order to prevent recurrent infection and the potential neoplastic transformation. The operation can obtain excellent results.
出处
《中国实用儿科杂志》
CSCD
北大核心
2009年第7期539-541,共3页
Chinese Journal of Practical Pediatrics
关键词
先天性肺囊性腺瘤样畸形
儿童
手术
congenital cystic adenomatoid malformation
children
operation