病例分析:纤维多囊性肝病的典型影像学表现  

Case analysis:Imaging manifestations of fibropolycystic liver diseases

作　　者：汪媛媛 罗春燕[1] 陈卫霞[1] 秦韵 WANG Yuanyuan;LUO Chunyan;CHEN Weixia;QIN Yun(Department of Radiology,West China Hospital,Sichuan University,Chengdu 610041,P.R.China)

机构地区：[1]四川大学华西医院放射科,成都610041

出　　处：《中国普外基础与临床杂志》2024年第9期1059-1062,共4页Chinese Journal of Bases and Clinics In General Surgery

摘　　要：纤维多囊性肝病(fibropolycystic liver diseases,FLDs)是一类罕见的先天性胆管板畸形相关的遗传性疾病,包括胆管错构瘤、先天性肝纤维化、多囊性肝病、Caroli病及胆总管囊肿。该疾病临床关注度低,影像学表现多样,容易误诊和漏诊。笔者通过本案例,总结该类疾病的典型影像学征象及其潜在的病理学机制,旨在加深读者对该疾病的认识,从而降低该疾病的漏诊率和误诊率。Fibropolycystic liver diseases(FLDs)is a rare genetic disorder,including bile duct hamartomas,congenital hepatic fibrosis,polycystic liver disease,Caroli’s disease,and choledochal cysts.Fibropolycystic liver diseases has received little clinical attention and exhibits a variety of imaging manifestations,leading to a high likelihood of missed diagnosis and misdiagnosis.Through this case,we delineate the characteristic imaging manifestations of the disease and its underlying pathological mechanisms.Our objective is to enhance readers'comprehension of the disease and thereby reduce the rate of missed diagnosis and misdiagnosis of the disease.

关 键 词：纤维多囊性肝病 胆管错构瘤 先天性肝纤维化 CAROLI病 影像学征象 

分 类 号：R575[医药卫生—消化系统] R445.2[医药卫生—内科学]