成人肺隔离症16例临床病理学分析

Clinical and pathological features of adult pulmonary sequestration:an analysis of 16 cases

目的总结分析肺隔离症(pulmonary sequestration,PS)的临床及病理学特征,提高对该病的认知和诊疗水平。方法回顾性分析2015年至2020年复旦大学附属华山医院收治的16例PS患者的临床、影像及病理资料。结果16例患者中,男性10例(62.5%),女性6例(37.5%),年龄22~66岁,平均年龄(47±12)岁。PS临床症状无特异性,主要表现为反复咳嗽、咳痰、发热及咯血。胸部CT检查表现为圆形及团块状的实性肿块影8例,囊状或蜂窝状影5例,片状致密影2例,梭形致密灶1例,术前经增强CT结合CT血管造影(CT angiography,CTA)确诊11例、误诊5例,误诊率为31.25%。所有患者均接受手术治疗,其中15例(93.75%)接受胸腔镜手术(video-assisted thoracoscopic surgery,VATS),1例(6.25%)接受开胸手术,术后均恢复良好。病变组织的病理学表现为肺组织发育不良,肺泡间隔呈迷路样,围成不规则蜂窝状囊腔改变,囊腔内见分泌物;周围慢性炎症反应与纤维化,可见畸形或者闭塞性的动静脉血管。结论PS临床表现无特异性,容易误诊,增强CT和CTA有助于术前诊断;治疗方法常为外科手术切除,术后病理表现为肺组织发育不良,呈慢性炎性反应伴纤维化;病理学改变需与慢性阻塞性肺病、支气管扩张症等疾病鉴别。

Objective To summarize the clinical and pathological characteristics of pulmonary sequestration(PS),improve the understanding of PS along with diagnosis and treatment.Methods The clinical,radiological,and pathological data of 16 patients with PS diagnosed and treated in Huashan Hospital,Fudan University from 2015 to 2020 were analyzed retrospectively.Results Among the 16 patients,10 were males(62.5%)and 6 were females(37.5%),aged 22 to 66 years old,with an average age of(47±12)years.Their clinical symptoms of PS were not specific,mainly including repeated cough,sputum,fever and hemoptysis.Chest CT examination showed round and massive solid mass shadows in 8 cases,cystic or honeycomb shadows in 5 cases,sheet-like dense shadows in 2 cases and fusiform dense lesions in 1 case.Eleven cases were diagnosed as PS by enhanced CT and CT angiography(CTA)and 5 cases were misdiagnosed.The misdiagnosis rate was 31.25%.All patients underwent surgical treatment,of which 15 cases(93.75%)underwent video-assisted thoracoscopic surgery(VATS)and 1 case(6.25%)underwent thoracotomy.All patients recovered well after surgery.The common pathological features of the lesions showed dysplasia of lung tissue,with irregular cyst and secretion,chronic inflammatory reaction with fibrosis in septa,and abnormal or occluded blood vessels.Conclusion The clinical manifestations of PS are non-specific and are easy to be misdiagnosed and missed.Enhanced CT and CTA are helpful for preoperative diagnosis.Surgical resection is a common treatment.Postoperative pathological manifestations include dysplasia of lung tissue,chronic inflammatory reaction with fibrosis,pathological changes need to be differentiated from chronic obstructive pulmonary disease,bronchiectasis and other diseases.