An overview of current treatment methods for Budd-Chiari syndrome

作者：Seijo Susana; Carlos Garcia Pagan Juan

来源：Expert Opinion on Orphan Drugs, 2014, 2(2): 147-157.

DOI：10.1517/21678707.2014.872560

• angioplasty
• Budd-Chiari syndrome
• hepatic vein thrombosis
• liver transplantation
• portal hypertension
• transjugular intrahepatic portosystemic shunt

Introduction: The Budd-Chiari Syndrome (BCS) is a rare and life-threatening disorder caused by the obstruction of hepatic venous outflow. The clinical presentation of BCS can range from the absence of symptoms to the development of end-stage liver disease or fulminant liver failure.
Areas covered: This review provides an overview of the available treatments for BCS. Long-term anticoagulation is mandatory in all patients with BCS. The need for an additional intervention, such as hepatic vein angioplasty, thrombolysis, transjugular intrahepatic portosystemic shunt, surgical shunts or liver transplantation depends on the severity of symptoms and response to treatment. Due to the low prevalence of the disease, knowledge of management of BCS is mostly based on retrospective series and expert opinion and hampered the development of randomized controlled trials.
Expert opinion: Outcome of BCS has improved in the last decades, mainly due to the increased suspicion and early treatment instauration; the frequent recognition of an underlying systemic prothrombotic disorder and its ensuing adequate treatment; the widespread use of long-term anticoagulation and the implementation of a stepwise management strategy based on the clinical response to the previous step of treatment. Further studies are needed to better define treatment failure and the optimal timing for scaling in treatment.

• 出版日期2014-2