First Report of Fulminant Budd-Chiari Syndrome Treated by Direct Intrahepatic Portocaval Shunt in Vietnam

Abstract

Budd-Chiari syndrome (BCS) is a rare disorder caused by hepatic venous outflow obstruction that can lead to acute liver failure proposing liver transplantation or transjugular intrahepatic portosystemic shunt. However, the transjugular intrahepatic portosystemic shunt is not always successful due to the entire hepatic vein thrombosis while transplantation is not unfailingly feasible. In these situations, the direct intrahepatic portosystemic shunt (DIPS) is a viable alternative that may ameliorate portal hypertension in these patients. We described a case of a 21-year-old male with fulminant hepatic failure owning to BCS with a 4-day history of abdominal pain and nausea. Laboratory workup, including viral, autoimmune etiologies JAK2 mutation, Factor V Leiden, antiphospholipid antibody syndrome, was negative. The patient's clinical status worsened with hepatic encephalopathy stage II despite administering anticoagulation. Thus, the patient underwent urgently DIPS after unaccessible to the creation of a transjugular intrahepatic portosystemic shunt and impossible to transplantation. The patient's health was improved and discharged. Fulminant Budd-Chiari is a rare disease to be demanding prompt treatment. While transplantation or transjugular intrahepatic portosystemic shunt is failed, the DIPS is considered an alternative candidate associated with clinical improvement.

Keywords: Budd-Chiari syndrome; Direct intrahepatic portosystemic shunt; Fulminant hepatic failure.