Shrestha Santosh Man, Kage Masayoshi, Lee Byung Boong
Liver Foundation Nepal, Kathmandu, Nepal.
Department of Diagnostic Pathology, Kurume University Hospital, Fukuoka, Japan.
Hepatol Res. 2017 Jun;47(7):603-615. doi: 10.1111/hepr.12869. Epub 2017 May 9.
Hepatic vena cava syndrome, also known as membranous obstruction of inferior vena cava (IVC), was considered a rare congenital disease and classified under Budd-Chiari syndrome. It is now recognized as a bacterial infection-induced disease related to poor hygiene. Localized thrombophlebitis of the IVC at the site close to hepatic vein outlets is the initial lesion which converts on resolution into stenosis or complete obstruction, the circulatory equilibrium being maintained by development of cavo-caval collateral anastomosis. These changes persist for the rest of the patient's life. The patient remains asymptomatic for a variable period until acute exacerbations occur, precipitated by bacterial infection, resulting in deposition of thrombi at the site of the lesion and endophlebitis in intrahepatic veins. Large thrombus close to hepatic vein outlets results in ascites from hepatic venous outflow obstruction, which is followed by development of venocentric cirrhosis. Endophlebitis of intrahepatic veins results in ischemic liver damage and development of segmental stenosis or membrane. Acute exacerbations are recognized clinically as intermittent jaundice and/or elevation of aminotransferase or ascites associated with neutrophil leukocytosis and elevation of C-reactive protein; sonologically, they are recognized as the presence of thrombi of different ages in IVC and thrombosis of intrahepatic veins. Development of liver cirrhosis and hepatocellular carcinoma is related to severity or frequency of acute exacerbations and not to duration or type of caval obstruction. Hepatic vena cava syndrome is a common co-morbid condition with other liver diseases in developing countries and it should be considered in differential diagnosis in patient with intermittent elevation serum bilirubin and or aminotransferase or development of ascites and cirrhosis.
肝静脉综合征，也称为下腔静脉膜性梗阻（IVC），曾被认为是一种罕见的先天性疾病，并归类于布加综合征。现在它被认为是一种与卫生条件差相关的细菌感染性疾病。靠近肝静脉出口处的下腔静脉局部血栓性静脉炎是初始病变，病变消退后会转变为狭窄或完全梗阻，通过腔静脉侧支吻合的形成来维持循环平衡。这些变化会在患者余生持续存在。患者在一段可变的时间内保持无症状，直到因细菌感染引发急性加重，导致病变部位血栓形成和肝内静脉内膜炎。靠近肝静脉出口处的大血栓会导致肝静脉流出道梗阻引起腹水，随后发展为中心性肝硬化。肝内静脉内膜炎会导致缺血性肝损伤和节段性狭窄或膜的形成。急性加重在临床上表现为间歇性黄疸和/或转氨酶升高或腹水，伴有中性粒细胞增多和C反应蛋白升高；在超声检查中，表现为下腔静脉内不同时期血栓的存在以及肝内静脉血栓形成。肝硬化和肝细胞癌的发生与急性加重的严重程度或频率有关，而与腔静脉梗阻的持续时间或类型无关。肝静脉综合征在发展中国家是一种与其他肝脏疾病常见的共病情况，在血清胆红素和/或转氨酶间歇性升高或出现腹水和肝硬化的患者的鉴别诊断中应予以考虑。
