Sections View Full Chapter Figures Tables Videos Annotate Full Chapter Figures Tables Videos Supplementary Content + Download Section PDF Listen ++ For further information, see CMDT Part 16-15: Hepatic Venous Outflow Obstruction (Budd-Chiari Syndrome) + Key Features Download Section PDF Listen +++ +++ Essentials of Diagnosis ++ Right upper quadrant pain and tenderness Ascites Imaging study showing occlusion/absence of flow in the hepatic vein(s) or inferior vena cava Similar clinical picture in sinusoidal obstruction syndrome but major hepatic veins are patent +++ General Considerations ++ Cases in India, China, and South Africa Often the result of occlusion of the hepatic portion of the inferior vena cava, presumably due to prior thrombosis Clinical presentation is mild but the course is frequently complicated by hepatocellular carcinoma Sinusoidal obstruction syndrome Occlusion of terminal venules, which mimics Budd-Chiari syndrome clinically May occur in patients who have undergone hematopoietic stem cell transplantation, particularly those with pretransplant serum aminotransferase elevations or fever during cytoreductive therapy with cyclophosphamide, azathioprine, carmustine, busulfan, etoposide, or gemtuzumab ozogamicin Also common in those receiving high-dose cytoreductive therapy or high-dose total body irradiation Can be caused by Comfrey or "bush teas" (pyrrolizidine alkaloids) +++ Etiologies ++ Hypercoagulable state Caval webs Myeloproliferative neoplasms, eg, polycythemia vera Right-sided heart failure or constrictive pericarditis Neoplasm compressing the hepatic vein Paroxysmal nocturnal hemoglobinuria Behçet syndrome Blunt abdominal trauma Oral contraceptives or pregnancy + Clinical Findings Download Section PDF Listen +++ +++ Symptoms and Signs ++ Presentation is most commonly subacute but may be fulminant, acute, or chronic Tender, painful hepatic enlargement Jaundice; splenomegaly; and ascites With chronic disease, bleeding varices and hepatic encephalopathy may be evident Hepatopulmonary syndrome may occur +++ Differential Diagnosis ++ Cholecystitis Shock liver Cirrhosis Hepatic congestion from right-sided heart failure Metastatic cancer involving the liver + Diagnosis Download Section PDF Listen +++ +++ Laboratory Tests ++ Liver biochemical test abnormalities are nonspecific Jaundice may or may not be present Very high serum alanine aminotransferase/aspartate aminotransferase levels (ALT/AST) (> 1000 units/L) suggest occlusion of hepatic and portal veins Signs of decompensated liver disease (low albumin, coagulopathy) indicate poor prognosis +++ Imaging Studies ++ Hepatic imaging studies may show a prominent caudate lobe, since its venous drainage may be occluded Contrast-enhanced, color or pulsed-Doppler ultrasonography Screening test of choice Has a sensitivity of 85% for detecting evidence of hepatic venous or inferior vena caval thrombosis MRI with spin-echo and gradient-echo sequences and intravenous gadolinium injection allows visualization of the obstructed veins and collateral vessels Direct venography can delineate caval webs and occluded hepatic veins Concomitant splanchnic vein thrombosis may be found in 4–21% of cases +++ Diagnostic Procedures ++ Percutaneous or transjugular liver biopsy Frequently shows a characteristic centrilobular congestion and fibrosis and often multiple large regenerative nodules Often contraindicated in sinusoidal obstruction syndrome because of thrombocytopenia, and the diagnosis is based on clinical findings + Treatment Download Section PDF Listen +++ +++ Medications ++ Lifelong anticoagulation and treatment of the underlying myeloproliferative disease is often required Low-molecular-weight heparins are preferred over unfractionated heparin because of a high rate of heparin-induced thrombocytopenia with the latter Warfarin is also acceptable, but direct oral anticoagulants have not been well studied Infusion of a thrombolytic agent into recently occluded veins has been attempted with success Defibrotide An adenosine receptor agonist that increases endogenous tissue plasminogen activator levels Used to prevent and treat sinusoidal obstruction syndrome; however, it has no benefit in severe disease Given as an intravenous infusion every 6 hours for a minimum of 21 days Serious adverse effects include hypotension and hemorrhage Expense may limit its use +++ Surgery ++ Transjugular intrahepatic portosystemic shunt (TIPS) is preferred over surgical decompression (side-to-side portacaval, mesocaval, or mesoatrial shunt) which, in contrast to TIPS, has not been proven to improve long-term survival Consider liver transplantation for Acute liver failure Cirrhosis with hepatocellular dysfunction Failed portosystemic shunt +++ Therapeutic Procedures ++ Treat ascites with salt restriction and diuretics (see Ascites) Treatable causes of Budd-Chiari syndrome should be sought Prompt recognition and treatment of an underlying hematologic disorder may avoid the need for surgery Rarely, thrombolytic therapy may be attempted within 2 weeks of acute hepatic vein thrombosis In cases of unresolved hepatic congestion, placement of a TIPS may be feasible, although late TIPS dysfunction is common Balloon angioplasty, in some cases with placement of an intravascular metallic stent, is preferred for inferior vena caval web and is being performed increasingly when there is a short segment of thrombosis in the hepatic vein + Outcome Download Section PDF Listen +++ +++ Complications ++ Hepatic failure Cirrhosis Spontaneous bacterial peritonitis (less common than in cirrhosis alone) +++ Prognosis ++ The overall 5-year survival rate is 50–90% with treatment (but < 10% without intervention) Adverse prognostic factors Older age High Child-Pugh score Ascites Encephalopathy Elevated serum total bilirubin Prolonged prothrombin time Elevated serum creatinine Concomitant portal vein thrombosis Histologic features of acute liver disease superimposed on chronic liver injury A 3-month mortality may be predicted by the Rotterdam score, which is based on encephalopathy, ascites, prothrombin time, and serum bilirubin A serum ALT level at least 5-fold above the upper limit of normal on presentation, which is indicative of hepatic ischemia, also predicts a poor outcome (particularly when the ALT level decreases slowly) Risk of hepatocellular carcinoma is increased; risk factors include Cirrhosis Combined hepatic vein and inferior vena cava obstruction Long-segment inferior vena cava block +++ When to Admit ++ All patients + References Download Section PDF Listen +++ + +Hernández-Gea V et al. Current knowledge in pathophysiology and management of Budd-Chiari syndrome and non-cirrhotic non-tumoral splanchnic vein thrombosis. J Hepatol. 2019 Jul;71(1):175–99. [PubMed: 30822449] + +Valla DC. Budd-Chiari syndrome/hepatic venous outflow tract obstruction. Hepatol Int. 2018 Feb;12(Suppl 1):168–80. [PubMed: 28685257] + +Van Wettere M et al. Diagnosis of Budd-Chiari syndrome. Abdom Radiol (NY). 2018 Aug;43(8):1896–907. [PubMed: 29285598]