Caroli病(先天性肝内胆管囊性扩张症),这些知识点你知道吗?

2022-06-30 华夏影像诊断中心 华夏影像诊断中心

这些诊治经验值得收藏~

术语

定义

  • 最初分为两种

    • Caroli病(“单纯”型):先天性、多发性的肝内大胆管(IHBDs)的囊性扩张,不伴其他肝异常

    • Caroli综合征(“复杂”型):更常见,以肝内大胆管的囊状扩张、肝纤维化、门静脉高压为特征

影像

一般特征

  • 最佳诊断线索

    • 肝内多发大小不等的囊与胆道树相通

    • 中心点征:增强CT上扩张的肝内胆管内可见小强化点(门静脉小分支)

  • 位置

    • 囊状扩张的肝内胆管可以弥漫分布,也可局限于肝叶或肝段

      • Caroli综合征常常弥漫累及全肝

      • Caroli病通常主要累及某一肝段,偶尔会分布于肝叶(肝左叶多见)

        • 以肝叶分布的病例,囊肿数目通常<10

  • 大小

    • 囊的大小差异很大(毫米至厘米)

  • 形态

    • 节段性囊状扩张的肝内胆管间可间隔正常或扩张的胆管

      • 每个囊均与胆道树相通

    • 胆总管的大小通常认为是正常的

      • 肝外胆管扩张程度可达50%,可能是由既往的胆管炎、结石移动或并发的胆总管囊肿引起的

  • 被认为是纤维多囊性肝病的一种变异

    • 其他变异包括先天性肝纤维化,常染色体显性(ADPKD)和隐性(ARPKD)多囊性肝肾疾病,胆管错构瘤,胆总管囊肿

  • 胆管囊肿的Todani分类

    • Caroli病为第V型胆管囊肿:肝内胆管囊状扩张

平片表现

  • 内镜逆行性胰胆管造影

    • 肝内大胆管(弥漫,肝叶或肝段)扩张(囊状扩张较梭形扩张更常见),伴交替的胆管狭窄

    • 肝内胆管结石和沉淀物常见,表现为充盈缺损

    • 合并胆管炎的病例,ERCP上可见与胆道树相通的胆管脓肿

CT表现

  • 大小不等的囊,无强化,与胆道树相通

    • 分布多样化(弥漫,肝叶或肝段分布)

  • 强化的门静脉小分支被囊状扩张的胆管包绕,在增强CT上表现为中心点征

    • 强化的“点”可位于扩张胆管的中心,也可以在其边缘

  • 平扫CT更容易发现肝内胆管高密度结石

  • 与合并的肝纤维化相关的其他征象

    • 肝左叶外侧段增大,右叶缩小(与其他原因所致的肝纤维化一致)

    • 肝左叶内侧段正常或增大(显著特征,其他原因所致的肝纤维化不常见)

  • 其他与常见的肾异常相关的表现

    • 常染色体隐性遗传性多囊肾病(ARPKD),常染色体显性遗传性多囊肾病(ADPKD),髓质海绵肾,髓质囊性病

MR表现

  • 肝内不同分布的囊性病变,T1低信号,T2高信号

    • MRCP能更清楚地显示囊与胆道树相通

    • 因为与胆道树相通,使用肝胆特异性对比剂(如钆塞酸二钠)时,囊性病变于肝胆期可充盈对比剂

    • 增强扫描T1WI可见中心点征:扩张的肝内胆管内可见强化的门静脉小分支

      • T2WI或MRCP上,扩张胆管中心的门静脉小分支表现为低信号

    • 肝内胆管结石表现为扩张胆管内无强化的低信号

超声表现

  • 肝内胆管扩张:超声难以显示囊性病变与胆道树之间的交通

  • 扩张的胆管腔可见完全或不完全的横膈膜(管内桥征)

  • 门静脉小分支可以部分或完全由扩张的肝内胆管包绕(超声的中心点征)

  • 胆管内结石表现为后方伴声影的结节状回声

成像推荐

  • 最佳影像方案

    • MRCP

鉴别诊断

常染色体显性遗传性多囊肝病

  • 多发肝囊肿(>10,通常数百个)

  • 不伴胆管扩张,且囊与胆道树无交通

    • 胆管造影时囊不显影

  • 常合并肾囊肿

胆管错构瘤

  • 肝内小胆管胆管板畸形(纤维多囊病的变异)

  • 肝内无数的厘米以下的小结节,囊性或囊实混杂,实性成分强化与肝实质呈等密度

  • 胆管错构瘤不与胆道树相通

原发性硬化性胆管炎

  • 肝内、肝外胆管狭窄;狭窄、正常胆管、扩张胆管交替

  • 胆管扩张程度较Caroli病轻,扩张的胆管不会呈囊状

  • 经常进展为肝硬化和肝功能衰竭

上行性胆管炎

  • 由胆道梗阻所致的感染(胆总管结石最常见)

  • 可引起肝内胆道脓肿,脓肿与胆管相通

  • 不同于Caroli病,脓肿壁厚并且形态不规则,周围肝实质水肿

复发性化脓性胆管炎

  • 以胆道系统充满胆色素结石为特点,导致胆道梗阻和胆管炎反复发作

    • 结石可呈铸型伴胆管腔扩张

  • 肝内、肝外胆管同时扩张,常呈柱状(而非囊状)

  • 常见于东南亚人群,可能与寄生虫性和细菌性胆管炎有关

病理

一般特征

  • 病因

    • 发病机制并不明确,可能与胆管板的异常重构有关

      • Caroli病:较大胆管畸形

      • Caroli综合征:大胆管和小胆管畸形

        • 小胆管的胆管板异常是肝纤维化的原因

  • 遗传学

    • 常染色体隐性遗传(常染色体显性遗传罕有报道)

    • 由染色体6的PKHD1基因(多囊肾肝病基因)突变所致(编码纤维囊)

      • 与ARPKD相同的基因基础,是同一基因病变的不同表现

  • 相关异常

    • 髓质海绵肾和肾纤维化

    • 常染色体隐性多囊肝肾疾病(很少与ADKPD相关)

    • 肝纤维化、肝囊肿、胆管错构瘤

    • 胆管囊肿

临床问题

临床表现

  • 最常见的体征/症状

    • 早期常无症状,童年或成年早期可出现症状

    • 有Caroli病或Caroli综合征的患者可能出现与胆道疾病有关的症状,通常是反复发作的胆管炎引起的发热和腹部疼痛

      • 胆道梗阻可导致胆汁淤积(黄疸、瘙痒等)

    • Caroli综合征可出现肝纤维化的症状(例如门静脉高压)

  • 诊断主要基于影像表现(尤其MRCP和ERCP),活检并不常规推荐

人群分布特征

  • 年龄

    • 与生俱来,尽管可能数年内无症状

      • 80%的患者30岁之前出现症状

    • Caroli病:症状通常在二三十岁时出现

    • Caroli综合征:婴儿或儿童期就可出现症状

      • 年轻时出现伴发的肾异常

  • 性别

    • 男:女=1:1

自然病史及预后

  • 并发症

    • 复发性胆管炎和(或)肝脓肿

      • 胆管扩张→胆汁淤积一胆管内胆泥/结石形成→胆管炎

    • 结石形成(95%)(大部分是胆红素结石)

    • 继发性胆汁性肝硬化和(或)门静脉高压

    • 胆管癌(7%风险)

  • 预后

    • 取决于疾病的严重程度和共存的肾疾病

    • Caroli病的远期预后较差

治疗

  • 对胆管炎/脓毒症和结石的支持治疗

    • 抗生素治疗胆管炎

    • 括约肌切开术取出肝外胆管结石

    • 肝内胆管结石采取碎石术

    • 口服胆汁盐可为一种选择治疗方式,尤其是对难治性的肝内结石[如熊去氧胆酸(UDCA)]

  • 可以通过体外引流或胆肠吻合术进行胆道减压

  • 手术是某些患者的选择

    • 如果扩张的胆管局限在肝叶或肝段内,肝叶或肝段切除术是可行的

    • 全肝弥漫病变可以考虑肝移植

      • 与其他适应证的肝移植结局类似

 

诊断要点

关注点

  • 需要将Caroli病与其他与肝多发囊肿或胆管扩张相关的肝病鉴别

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本文摘自《消化影像诊断学》

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    2022-08-11 wincls
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    2022-09-10 lsj637
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    2023-06-10 wolongzxh
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    2022-07-18 12157a75m71暂无昵称

    学习了 carali病

    0

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    2022-07-02 huirong
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