Budd-Chiari syndrome
Budd-Chiari syndrome is obstruction of hepatic venous outflow, usually hepatic vein thrombosis, causing hepatomegaly, ascites, and right-upper-quadrant pain. Diagnosis is by Doppler ultrasound; first-line treatment is anticoagulation, escalating to angioplasty, stenting, or TIPS for progressive disease.
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What this article covers
- Overview
Budd-Chiari syndrome is obstruction of hepatic venous outflow, most often thrombosis of one or more of the three major hepatic veins, with or without extension into the suprahepatic inferior vena cava (IVC).
- Epidemiology
Budd-Chiari syndrome is uncommon and occurs mainly in young to middle-aged adults with an underlying prothrombotic state rather than in older patients with atherosclerotic risk factors.
- Risk factors
Myeloproliferative neoplasm: evidence of an underlying myeloproliferative neoplasm, most often polycythemia vera, is found in approximately 50% of patients, even with a normal complete blood count.
- Pathophysiology
Thrombosis of the hepatic veins obstructs outflow from the hepatic sinusoids.
- Clinical features
Abdominal pain: dull, constant right-upper-quadrant or epigastric pain from hepatic capsular stretch, present in most symptomatic patients.
- Differential diagnosis
Congestive hepatopathy from right heart failure: also causes tender hepatomegaly and ascites, but jugular venous distension is present and hepatic vein Doppler shows patent, reversed flow rather than absent flow.
- Diagnosis
Establishing test: the diagnosis is established by Doppler ultrasonography demonstrating absent or abnormal flow in one or more major hepatic veins together with hepatic congestion; this is the…
- Management
First, initiate therapeutic anticoagulation once diagnosed and there is no active major bleeding: unfractionated heparin IV bolus 80 units/kg, then infusion titrated to an aPTT of 1.5 to 2.5 times…
- Complications
Progressive hepatic fibrosis and cirrhosis: sustained congestion produces centrilobular fibrosis over months if outflow is not relieved.
- Prognosis
Outcomes depend on how quickly outflow is reestablished and on control of the underlying prothrombotic disorder.
- Prevention
Indefinite anticoagulation after the index diagnosis is the principal measure to prevent rethrombosis in patients with an ongoing prothrombotic state.
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