CAJ | 학술논문

目的：探讨小儿肺囊性病变( cystic lung diseases, CLD)的形态学特征及临床干预时机。方法对125例CLD行常规HE及免疫组化染色,并复习相关文献。结果125例CLD中男性75例,女性50例,男女比为1．5∶1。年龄0~11．5岁,平均23．0个月,中位年龄15个月,小于1岁者60例(48．0%)。眼观：50例肺组织局部可见单房或多房囊肿,囊肿直径0．5~8．0 cm,囊腔与支气管不相通；18例肺切面呈蜂窝状,囊腔直径0．1~2．0 cm。26例肺组织实变,囊腔不明显。21例肺组织见较厚脓肿囊壁,内壁粗糙,可见脓苔。7例肺气肿肺组织按之有捻发音,切面见微囊。2例见肺组织内囊实性肿物,切面灰白色,鱼肉状。125例CLD中先天性肺支气管发育异常相关的囊肿共94例(75．2%),其中先天性肺气道畸形(congenial pulmonary air-way malformation, CPAM)59例(47．2%),其中1型29例(49．2%),2型18例(30．5%),4型12例(20．3%)；肺隔离症26例(20．8%),叶内型15例(57．7%),叶外型11例(42．3%),叶外型肺隔离症中5例合并 CPAM 2型；支气管源性囊肿8例(6．4%)；肠源性囊肿1例(0．8%)。获得性病变31例(24．8%),其中感染后肺脓肿21例,1例为真菌性脓肿；肺气肿改变7例；胸膜肺母细胞瘤共3例(Ⅰ型1例,Ⅱ型2例)。结论小儿CLD种类较多,结合影像学特征可明确诊断,选择合适的手术时机,可取得良好的治疗效果。
목적：탐토소인폐낭성병변( cystic lung diseases, CLD)적형태학특정급림상간예시궤。방법대125례CLD행상규HE급면역조화염색,병복습상관문헌。결과125례CLD중남성75례,녀성50례,남녀비위1．5∶1。년령0~11．5세,평균23．0개월,중위년령15개월,소우1세자60례(48．0%)。안관：50례폐조직국부가견단방혹다방낭종,낭종직경0．5~8．0 cm,낭강여지기관불상통；18례폐절면정봉와상,낭강직경0．1~2．0 cm。26례폐조직실변,낭강불명현。21례폐조직견교후농종낭벽,내벽조조,가견농태。7례폐기종폐조직안지유념발음,절면견미낭。2례견폐조직내낭실성종물,절면회백색,어육상。125례CLD중선천성폐지기관발육이상상관적낭종공94례(75．2%),기중선천성폐기도기형(congenial pulmonary air-way malformation, CPAM)59례(47．2%),기중1형29례(49．2%),2형18례(30．5%),4형12례(20．3%)；폐격리증26례(20．8%),협내형15례(57．7%),협외형11례(42．3%),협외형폐격리증중5례합병 CPAM 2형；지기관원성낭종8례(6．4%)；장원성낭종1례(0．8%)。획득성병변31례(24．8%),기중감염후폐농종21례,1례위진균성농종；폐기종개변7례；흉막폐모세포류공3례(Ⅰ형1례,Ⅱ형2례)。결론소인CLD충류교다,결합영상학특정가명학진단,선택합괄적수술시궤,가취득량호적치료효과。
Purpose To investigate the histopathological features of cystic lung diseases ( CLD) , and to discuss the timing of clinical interventions. Methods HE and immunohistochemical staining were performed and reviewed in 125 cases of CLD. Results 125 ca-ses of CLD aged from birth to 11 years and 6 month, with an average age of 23. 0 months, median age 15 months, of which 60 cases were less than 1 year (48. 0%). 75 cases were male and 50 cases female, with male to female ratio of 1. 5 ∶ 1. Grossly, 50 cases showed single or multiple cysts with the size 0. 5 ~8. 0 cm in diameter, which did not communicate with bronchial cavity. 18 cases showed honeycomb cysts with the diameter of 0. 1~2. 0 cm. 26 cases were solid lesions without visible cysts. 21 cases were observed lung abscess with thick and rough wall and pus inside. 7 cases of emphysema showed microcysts with crepitation. 2 cases were identi-fied cystic and solid masses, with fish-fresh like cut surface. Histopathologically, 94 cases (75. 2%) were related to congenital bron-chopulmonary dysplasia in 125 cases of CLD, in which there were 59 patients (47. 2%) of congenial pulmonary airway malformation (CPAM), including 29 cases of type 1 (49. 2%), 18 cases of type 2 (30. 5%), and 12 cases of type 4 (20. 3%), there were 26 ca-ses (20. 8%) of pulmonary sequestration, including 15 cases of intralobar type (57. 7%) and 11 of extralobar cases (42. 3%), 5 ca-ses were complicated with CPAM type 2, 8 cases were bronchial cyst (6. 4%) and 1 case of enteric cyst (0. 8%). Acquired lesions were detected in 31 cases (24. 8%), including 21 cases of infected lung abscess, 1 case of fungal abscess. 7 cases of emphysema, and 3 cases of pleuralpulmonary blastoma (typeⅠ1 case and typeⅡ2 cases). Conclusion Pediatric CLD is characterized as com-plexed categories. The prognosis depends on correct pathological diagnosis, combined with imaging evaluation and appropriate timing of surgery.