Arora A, Sarin S K
1 Department of Radiology, Institute of Liver and Biliary Sciences, New Delhi, India.
Br J Radiol. 2015 Feb;88(1046):20140653. doi: 10.1259/bjr.20140653. Epub 2014 Dec 16.
Obliterative portal venopathy (OPV) is an important cause of non-cirrhotic portal hypertension, which is often erroneously misdiagnosed as cryptogenic cirrhosis. It has a worldwide distribution with majority of cases hailing from the Asian subcontinent. However, recently the disease has gained global attention particularly because of its association with human immunodeficiency virus infection and use of antiretroviral drug therapy (didanosine). As the name suggests, the disorder is characterized by sclerosis and obliteration of the intrahepatic portal vein branches (with attendant periportal fibrosis) leading to portal hypertension amid intriguingly little liver dysfunction. It primarily affects young adults who present with clinically significant portal hypertension in the form of episodes of variceal bleed; however, contrasting liver cirrhosis, the liver function and liver structure remain normal or near normal until late in the disease process. Radiological findings during advanced disease are often indistinguishable from cirrhosis often warranting a liver biopsy. Nevertheless, recent studies have suggested that certain imaging manifestations, if present, can help us to prospectively suggest the possibility of OPV. At imaging, OPV is characterized by a wide range of intrahepatic and/or extrahepatic portal venous abnormalities with attendant changes in liver and splenic volume and stiffness. We shall, through this pictorial review, appraise the literature and illustrate the germane radiological manifestations of OPV that can be seen using different imaging modalities including ultrasonography, CT, MRI, elastography and hepatic haemodynamic studies.
闭塞性门静脉病(OPV)是非肝硬化门静脉高压的重要原因,常被误诊为隐源性肝硬化。该病在全球范围内均有分布,大多数病例来自亚洲次大陆。然而,近年来,这种疾病受到了全球关注,尤其是因为它与人类免疫缺陷病毒感染及抗逆转录病毒药物治疗(去羟肌苷)有关。顾名思义,该疾病的特征是肝内门静脉分支硬化和闭塞(伴有门静脉周围纤维化),导致门静脉高压,而肝功能损害却出奇地轻微。它主要影响年轻成年人,这些患者会出现临床上显著的门静脉高压,表现为静脉曲张出血发作;然而,与肝硬化不同的是,在疾病晚期之前,肝功能和肝脏结构仍保持正常或接近正常。疾病晚期的影像学表现通常与肝硬化难以区分,往往需要进行肝活检。尽管如此,最近的研究表明,如果出现某些影像学表现,有助于我们前瞻性地提示OPV的可能性。在影像学上,OPV的特征是肝内和/或肝外门静脉出现广泛异常,同时肝脏和脾脏体积及硬度发生变化。我们将通过这篇影像综述评估相关文献,并阐述使用不同成像方式(包括超声、CT、MRI、弹性成像和肝脏血流动力学研究)可观察到的OPV相关影像学表现。