Multimodality imaging of obliterative portal venopathy: what every radiologist should know

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.It is important to recognize that not all varices mean liver cirrhosis. Although liver cirrhosis constitutes the commonest cause of portal hypertension, we should be aware that portal hypertension can occur in the absence of liver cirrhosis—a condition termed as non-cirrhotic portal hypertension (NCPH).^1,2 NCPH represents a heterogeneous group of (primarily vascular) disorders where portal hypertension manifests amid absent liver cirrhosis. Pathologically, the insult is either pre- or intrahepatic involving the main portal vein or its smaller branches and/or the perisinusoidal area.^1–3Obliterative portal venopathy (OPV) represents an important cause of NCPH that is characterized by sclerosis and obliteration of the medium-sized portal venous branches leading to portal hypertension.^1–10 Liver biopsy characteristically shows phlebosclerosis and periportal and perisinusoidal fibrosis amid absent cirrhosis (Figure 1).^1–3 Although, the exact aetiology is contentious, infections and prothrombotic states have been implicated in eastern and western patients, respectively.^1,2 Additionally, xenobiotic exposure, autoimmune and genetic factors have also been incriminated.^1–4 Although the disease has a worldwide distribution, it continues to remain poorly understood primarily owing to its relative rarity.^1–3,5–8 Another potential reason is the use of diverse terminologies under which the entity has been described from various parts of the globe, such as non-cirrhotic portal fibrosis in India, idiopathic portal hypertension in Japan and hepatoportal sclerosis in the USA.Open in a separate windowFigure 1.(a) Atrophic small portal tract (arrow) showing absent portal vein [haematoxylin and eosin stain (HE), ×200]. (b) Two small portal tract (arrows) approximations (×100, HE). (c) Portal and central vein approximation (×100, HE). (d) Parenchymal extinction suggested by portal–portal and portal–central approximation (Masson''s trichrome stain, ×200).More recently, the disease has gained global attention because of escalating number of cases being reported in human immunodeficiency virus (HIV)-infected patients.^1–3,8–10 Also, US Food and Drug Administration has recently issued a warning regarding the potential association of OPV in patients with HIV on didanosine (antiretroviral therapy).³OPV primarily affects young patients usually in their third or fourth decades of life. The affected individuals typically present with clinically significant portal hypertension characterized by multiple episodes of well-controlled upper gastrointestinal (GI) bleed, massive splenomegaly and/or hypersplenism.^1–3 Advanced stages of the disease are often indistinguishable from liver cirrhosis especially on imaging. However, discrimination from cirrhosis is crucial in clinical practice because of differences in management. Management of OPV is primarily symptomatic, that is, focused on management of an acute episode of variceal bleed. The risk of rebleeding and bleeding-related mortality is low. Intriguingly, in contrast to liver cirrhosis, the liver function and liver structure remain normal or near normal until late in the disease process leading to a better prognosis and higher survival rates; the 10-year survival rate is around 86–95%.^1,2 Development of jaundice, ascites and hepatic encephalopathy is uncommon and if at all is seen only after an episode of GI bleeding.^1,2 Liver failure and the incidence of developing hepatocellular carcinoma are also much lower.^1–3,8–10 Nonetheless, in 20–33% of patients, the liver gradually atrophies and shows functional decompensation, occasionally needing liver transplantation.^1,2Although limited literature is available on the radiological manifestations of OPV, recent studies have suggested certain imaging manifestations to be more prevalent in OPV that can allow discrimination from cirrhosis. Moreover, use of newer techniques, including transient elastography, can allow prospective non-invasive diagnosis of OPV based upon the differential changes in liver and splenic stiffness. The aim of this review is to appraise the imaging findings of OPV described in the literature and illustrate them across a wide array of imaging modalities, including ultrasonography, CT, MRI and elastography, in a group of biopsy-proven cases of OPV diagnosed at our institute.