儿童先天性肺气道畸形73例临床病理分析概要.PDFVIP

临床与病理杂志 2015, 35(10) J Clin Path ol R e s 1773 doi: 10.3978/j.issn.2095-6959.2015.10.014 ·论著 · View this article at: /10.3978/j.issn.2095-6959.2015.10.014 儿童先天性肺气道畸形73例临床病理分析 舒艳，朱坤，汤宏峰，赵曼丽，杨敏，赵云，刘蕾，李俊平 (浙江大学医学院附属儿童医院病理科，杭州 310013) [摘　要] 目的：探讨先天性肺气道畸形 (CPAM) 的临床、影像、病理特征及预后。方法：回顾性分析73例儿 童 CPAM 的临床病理特征并复习相关文献。结果：患儿年龄 1 d~15岁，平均年龄4.3 岁，男女发病 几率基本相同。依照 Stocker组织病理学分型，73例 CPAM 中Ⅰ型49 例，Ⅱ型22例，Ⅲ型2 例。均为 单侧病变，累及左侧29 ，右侧44 例，合并心脏畸形6例，合并蛛网膜囊肿2 例，伴隐形脊柱裂及甲 状腺发育不良 1例，伴脑发育落后、脑积水 1例，伴肾母细胞瘤 1例。影像学方面除2 例Ⅲ型 CPAM 显示肺实变样改变外，余病例胸部X 线或 CT均表现为单个或多个含气大囊；或多发大小相近蜂窝 状小囊，囊内未见肺纹理。73例均行患侧肺叶切除术，随访时间2~25个月不等，除2 例Ⅲ型患儿死 亡外，余患儿恢复良好。 结论：CPAM是一种较罕见的发生于肺的先天性囊性腺瘤样畸形，可通 过影像检查发现，确诊依据病理组织学检查，通过手术完整切除患侧肺叶，预后较好。 [关键词] 肺气道畸形；儿童；肺叶切除术；预后 Congenital pulmonary airway malformation of the lung in children: a clinicalpathological analysis of 73 cases SHU Yan, ZHU Kun, TANG Hongfeng, ZHAO Manli, YANG Min, ZHAO Yun, LIU Lei, LI Junping (Department of Pathology, Children’s Hospital, Zhejiang University School of Medicine, Hangzhou 310013, China) Abstract Objective: To explore the clinical, imaging, pathological feature and prognosis of congenital pulmonary airway malformation (CPAM). Methods: Retrospect analysis the clinical pathologic features of 73 cases of CPAM and review related literatures. Results: Patients’ age ranges from 1 day to 15 years, with mean age 4.3 years old. CPAM has no sex predilection. Histologic examination showed that 49 cases were classified as Stocker type I, 22 cases as type II, 2 cases as type III. A