CAJ | 학술논문

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。
목적：탐토선천성폐기도기형(CPAM)적림상、영상、병리특정급예후。방법：회고성분석73례인동CPAM적림상병리특정병복습상관문헌。결과：환인년령1 d~15세，평균년령4.3세，남녀발병궤솔기본상동。의조Stocker조직병이학분형，73례CPAM중Ⅰ형49례，Ⅱ형22례，Ⅲ형2례。균위단측병변，루급좌측29，우측44례，합병심장기형6례，합병주망막낭종2례，반은형척주렬급갑상선발육불량1례，반뇌발육락후、뇌적수1례，반신모세포류1례。영상학방면제2례Ⅲ형CPAM현시폐실변양개변외，여병례흉부X선혹CT균표현위단개혹다개함기대낭；혹다발대소상근봉와상소낭，낭내미견폐문리。73례균행환측폐협절제술，수방시간2~25개월불등，제2례Ⅲ형환인사망외，여환인회복량호。결론：CPAM시일충교한견적발생우폐적선천성낭성선류양기형，가통과영상검사발현，학진의거병리조직학검사，통과수술완정절제환측폐협，예후교호。
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 classiifed as Stocker type I, 22 cases as type II, 2 cases as type III. All were unilateral lesion and involvement on the letf side of the 29 cases, on the right side of 44 cases. Of the 73 cases studied, the associated malformations included cardiac anomalies(6 cases), arachnoid cyst (2 cases), invisible spinal biifda and thyroid dysplasia (1 case), brain development backwardness and hydrocephalus (1 case), wilm’s tumor (1 case). All of the 73 cases performed X-ray or CT examination: except mass-like change for 2 cases, the others showed single or multiple large air cystic lesions; honeycomb-like small cystic lesions, the cyst lack of lung marking. All cases were underwent lobectomy, Follow-up time ranges from 2~25 months, except 2 cases died of type III, the rest of 71 cases recovered well.Conclusion:CPAM is a relatively rare pulmonary lesion of congenital cystic adenomatoid malformation. Imaging analysis is a valuable tool to suggest CPAM, while deifnite diagnosis requires pathologic examination. Surgical excision, commonly lobectomy is recommended.