摘要目的 总结先天性肺气道畸形(CPAM)的临床特点、影像、病理特征及治疗方法.方法 回顾2012年4月至2017年11月胸外科收治的283例病理诊断为单纯的先天性肺气道畸形且除外合并其他畸形同期手术的病例,总结分析其临床资料、手术方法、手术情况及术后病理分型.结果 283例均顺利完成手术,无严重手术并发症及二次开胸.其中男167例,女116例,男∶女=1.44∶1;年龄0.77～166.00个月,中位年龄20.67个月;手术时间25～220 min,中位时间75 min,出血量1～ 250ml,中位出血量5 ml.其中开胸手术80例,胸腔镜手术203例,中转开胸19例,中转率9.36％.术后病理:病理分型0型无、1型57例、2型194例、3型5例、4型9例,1、2混合型9例,1、3混合型2例,2、3混合型5例,2、4混合型1例,3、4混合型1例.结论 目前,先天性肺囊性腺瘤样畸形被重新命名为CPAM并且被重新分型.CPAM可通过影像检查发现,确诊依据病理组织学检查,通过手术完整切除肺部病变,预后较好.胸腔镜微创手术较开胸手术减轻手术创伤,伤口小而美观,建议推广.

abstractsObjective The purpose of the study is introducing the concept and classification of congenital pulmonary airway malformation(CPAM),and summarizing the clinical features,imaging,pathological features and treatment methods of congenital pulmonary airway malformation.Methods 283 cases were retrospectively analyzed from April 2012 to November 2017,which were treated by thoracic surgery,pathological diagnosis were isolated congenital pulmonary airway abnormalities except combination of other deformity surgery at the same time.we analyze the clinical data,surgical methods,operation and postoperative pathology classification.Results All the 283 cases were successfully performed without severe surgical complications and secondary thoracotomy.There were 167 males and 116 females,male∶ female =1.44∶1;Age:0.77-166 months,median age 20.67 months;The operation time was 25-220 min,median time was 75min,and the bleeding volume was 1-250 ml,and the median blood volume was 5 ml.Among them,80 patients underwent thoracotomy,203 cases of thoracoscopic surgery,19 cases were transferred.Postoperative pathology 57 cases of type 1,194 cases of type 2,5 cases of type 3,9 cases of type 4.Conclusion CCAM has been renamed as congenital pulmonary airway malformation (CPAM) and has been reclassified.CPAM can be detected by imaging examination,and the diagnosis is based on histopathological examination,and the prognosis is better after the complete surgical resection of lung lesions.Thoracoscopic minimally invasive surgery could make less surgical trauma,small and beautiful wound,which is suggested to be promoted widely.