肝血管畸形的诊断和治疗

摘要: 肝血管畸形是一类以肝内动脉、静脉或门静脉系统结构异常为特征的先天性或获得性疾病，临床上可表现为高动力循环症、门静脉高压症和门腔分流并发症。该类疾病的发病率较低，临床表现缺乏特异性，尤其易与肝硬化门静脉高压症相混淆，从而导致诊断与治疗的延迟。本文重点介绍肝血管畸形所致的常见临床表现及其鉴别诊断，并阐述3种典型的肝血管畸形疾病，旨在为临床诊治提供思路和参考。
- 肝血管畸形 /
- 病理状态，体征和症状 /
- 诊断 /
- 治疗学
Abstract: Hepatic vascular malformations are a group of congenital or acquired disorders characterized by structural abnormalities of the intrahepatic arterial， venous， or portal venous systems， with the clinical manifestations of hyperdynamic circulation， portal hypertension， and complications of portosystemic shunting. Due to the low incidence rate and nonspecific clinical manifestations of such disorders， they are easily confused with cirrhotic portal hypertension， leading to the delay in diagnosis and treatment. This article introduces the common clinical manifestations and differential diagnosis of hepatic vascular malformations and elaborates on three typical types of hepatic vascular malformations， in order to provide ideas and references for clinical diagnosis and treatment.
- Hepatic Vascular Malformation /
- Pathological Conditions， Signs and Symptoms /
- Diagnosis /
- Therapeutics

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注：动脉期肝动脉扩张，肝静脉早显，多发胆管扩张及胆汁瘤。

图 1 肝血管畸形患者的三期CT血管造影（动脉期）

Figure 1. Triphasic CT angiography （arterial phase） in a patient with hepatic vascular malformations

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注：动脉期门静脉早显。

图 2 HAPF患者CT影像

Figure 2. CT image of a patient with hepatic artery-portal fistula

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注： a， Ⅰa型； b， Ⅰb型； c， Ⅱ型；IVC，下腔静脉；SV，脾静脉；SMV，肠系膜上静脉；PV，门静脉；SHUNT，分流道/分流血管；CEPS，先天性肝外门体分流。

图 3 经典的CEPS分类

Figure 3. Classic classification of CEPS

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表 1 肝动静脉畸形鉴别诊断

Table 1. Differential diagnosis of hepatic arteriovenous malformations

项目	肝动脉-肝静脉畸形	肝动脉-门静脉瘘
临床表现	高动力循环	门静脉高压
影像学	动脉期肝静脉早显；肝动脉、肝静脉迂曲扩张；有时伴胆管扩张	动脉期门静脉早显；肝动脉及门静脉扩张；常合并门静脉血栓
合并症和周边表现	高输出量心衰；腹痛“窃血”症状：胆管缺血、肠道缺血	腹水；食管胃底静脉曲张；门静脉血栓
常见病因	遗传性出血性毛细血管扩张症	原发性：基因突变；继发性：外伤、肝硬化、肝癌和化疗药使用
治疗方式	无症状者观察；有症状者针对并发症处理；肝移植	药物及对症治疗；介入栓塞/分流；肝移植

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表 2 门-体静脉分流及其鉴别

Table 2. Portosystemic shunt and its differential diagnosis

项目	先天性肝内门体分流	先天性肝外门体分流（Abernethy畸形）	自发性门体分流
临床表现	无症状	可无症状或仅轻度肝功能异常，直至出现门体分流相关的严重并发症	继发于肝硬化门静脉高压
合并症	无	肝性脑病、肺动脉高压、肝肺综合征、肝细胞癌或肝细胞腺瘤	肝性脑病、腹腔积液、食管胃底静脉曲张
发病年龄	婴儿发病<2岁自发闭合	常于20岁左右诊断，但也有部分患者在50岁后仍无症状^［4］	中年及以后发病
病理特点	不易合并恶性肿瘤	典型表现是门静脉分支缺如或发育不良，并伴有肝窦充血和动脉分支粗大^［5-6］	肝硬化病史及病理表现

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