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Update on the classification, assessment of prognosis and therapy of Budd-Chiari syndrome
Marco Senzolo1, Evangelos C Cholongitas, David Patch
1Liver Unit, Royal Free Hospital, London, UK.
Insights
Budd-Chiari syndrome (BCS) involves hepatic venous outflow obstruction. Advances in diagnosis and treatment, including transjugular intrahepatic portosystemic shunts and anticoagulation, improve prognosis.
Area of Science:
- Hepatology
- Vascular Medicine
- Interventional Radiology
Background:
- Budd-Chiari syndrome (BCS) presents with varied symptoms and liver damage, complicating diagnosis and prognosis.
- Traditional BCS classification lacks prognostic utility, hindering therapy assessment.
- Hepatic venous outflow obstruction is the defining characteristic of BCS.
Purpose of the Study:
- To highlight recent advancements in the prognosis and therapy of Budd-Chiari syndrome.
- To emphasize the importance of identifying the venous obstruction site for patient outcomes.
- To discuss modern diagnostic and therapeutic strategies for BCS.
Main Methods:
- Simultaneous diagnosis and treatment in radiology departments using hepatic venography, transjugular liver biopsy, and pressure measurements.
- Interventional procedures including dilation/stenting of venous webs and transjugular intrahepatic portosystemic shunt (TIPS) placement.
- Routine anticoagulation therapy before and after interventions.
Main Results:
- Identification of the venous obstruction site significantly impacts prognosis.
- Portal vein thrombosis occurs in 20-30% of BCS cases.
- Transjugular intrahepatic portosystemic shunts have largely replaced surgical shunts.
- Liver transplantation is reserved for severe BCS forms.
Conclusions:
- Modern interventional radiology allows for single-session diagnosis and treatment of BCS.
- Anticoagulation is a crucial component of BCS management, regardless of thrombophilia.
- Advances in therapy offer improved outcomes for patients with Budd-Chiari syndrome.
Abstract:
Budd-Chiari syndrome (BCS) occurs as a result of obstruction of hepatic venous outflow at any level from the small hepatic veins to the junction of the inferior vena cava with the right atrium. Diagnosis can be difficult because of the wide spectrum of presentation of the disease and the varying severity of liver damage. The traditional classification of BCS--as fulminant, acute or chronic--is not prognostically useful. This makes assessing the benefit of therapy difficult, especially as there is no evidence from randomized studies. This article highlights advances in the prognosis and therapy of BCS. Identification of the site of venous obstruction has a major effect on prognosis. Portal-vein thrombosis occurs in 20-30% of cases, and acute presentation of BCS reflects an acute or chronic syndrome in 60% of BCS cases. BCS can be diagnosed and treated on a single occasion in the setting of the radiology department, with hepatic venography, transjugular liver biopsy, retrograde CO2 portography and inferior vena cava pressure measurements performed simultaneously with therapies such as dilation or stenting of webs in the inferior vena cava or hepatic veins, and placement of transjugular intrahepatic portosystemic shunts. Disruption of a portal vein thrombus can also be done during the same session. Surgical shunts have been superseded by the use of transjugular intrahepatic portosystemic shunts. Liver transplantation is reserved for fulminant and progressive chronic forms of BCS. Anticoagulation therapy must be used routinely, before and after specific therapy, regardless of whether a thrombophilic disorder is diagnosed.