肺毛细血管瘤病与肺静脉闭塞症临床及CT特点  

The clinical and CT features of pulmonary capillary hemangiomatosis and pulmonary veno-occlusive disease

作　　者：黎剑宇[1] 曾庆思[1] LI Jian-yu;ZENG Qing-si(Department of Radiology,the First Affiliated Hospital of Guangzhou Medical University,Guangzhou 510120,China)

机构地区：[1]广州医科大学附属第一医院放射CT/DR科,广州510120

出　　处：《放射学实践》2020年第11期1419-1423,共5页Radiologic Practice

摘　　要：目的:分析及总结肺毛细血管瘤病(PCH)与肺静脉闭塞症(PVOD)的临床及CT影像特征。方法:回顾性分析广州医科大学附属第一医院2013-2019年经临床或病理确诊的6例PCH或PVOD患者的病例资料,其中男4例,女2例,总结其临床表现及CT征象特点,并复习相关文献对该病进行归纳分析。结果:PCH和PVOD临床症状以胸闷、气促、干咳为主,少或无咳痰、咯血。常伴有肺功能受损,以弥散功能严重下降为主,平均一氧化碳弥散量(DLCO)占预测值的百分比[DLCO(%Pred)]为(31.0±7.3)%。心脏彩超及右心导管均提示肺动脉高压。胸部CT征象常表现为多发斑片磨玻璃影、多发磨玻璃小结节影、小叶间隔增厚、肺门及纵隔淋巴结肿大、肺动脉主干增宽及右心增大等肺动脉高压表现,心包积液等,部分可见支气管动脉增粗。其中PCH以多发磨玻璃小结节影为主,小叶间隔增厚、肺门及纵隔淋巴结肿大等表现较轻,PVOD以多发斑片磨玻璃影为主,小叶间隔增厚、肺门及纵隔淋巴结肿大等表现较明显。结论:PCH和PVOD是罕见的肺血管疾病,其临床症状、肺功能检查、胸部CT表现具有一定特征。Objective:To analyze and summarize the clinical and CT features of pulmonary capillary hemangiomatosis(PCH)and pulmonary veno-occlusive disease(PVOD).Methods:The data of 6 patients with PCH or PVOD diagnosed in the First Affiliated Hospital of Guangzhou Medical University from 2013 to 2019 were analyzed retrospectively,including 4 males and 2 females.The clinical manifestations and CT features of 6 cases were summarized asn analyzed with relevant literature.Results:PCH and PVOD were rare and easily confused with idiopathic pulmonary artery hypertension.The main clinical symptoms were chest tightness,shortness of breath and dry cough,with little or no expectoration and hemoptysis.It was often accompanied by impairment of lung function,mainly with decrease of diffusion function.Echocardiography and right cardiac catheterization indicated pulmonary hypertension.CT findings were often manifested as multiple ground glass opacity,multiple ground glass nodules,interlobular septum,enlarged hilar and mediastinal lymph nodes,pulmonary hypertension such as widened main pulmonary artery and enlarged right heart,pericardial effusion,etc.,some could be manifested as thickened bronchial artery.PCH and PVOD were mainly composed of multiple ground glass nodules and multiple ground glass opacity respectively.Thickened interlobular septum and enlarged hilar and mediastinal lymph nodes of PVOD were more severe than those of PCH.Conclusion:PCH and PVOD are rare pulmonary vascular diseases.There clinical symptoms,pulmonary function test and chest CT have some characteristics.

关 键 词：肺毛细血管瘤病 肺静脉闭塞症 肺动脉高压 体层摄影术 X线计算机 

分 类 号：R814.42[医药卫生—影像医学与核医学] R816.4[医药卫生—放射医学]