Budd-Chiari syndrome and extensive inferior vena cava thrombosis treated with sequential interventional radiology and transjugular intrahepatic portosystemic shunting: A case report from Kenya

Abstract

Budd-Chiari syndrome is a rare disease characterized by the obstruction of hepatic venous outflow. Stepwise treatment options aimed to relieve obstruction and prevent complications of Budd-Chiari syndrome are medical therapy, interventional recanalization, and surgery. Aggressive interventions for complicated Budd-Chiari syndrome are placement of a transjugular intrahepatic portosystemic shunt, surgical shunting, or liver transplantation. Although literature suggests differences in the presentation and management between Europe and Asia, cases documenting successful use of stepwise management of Budd-Chiari syndrome in Sub-Saharan Africa are scarce. A 47-year-old male on treatment for chronic hepatitis B presented with abdominal pain and distension for 2 weeks and findings of gross ascites without stigmata of chronic liver disease. Laboratory investigations performed showed anemia, elevated transaminases, coagulopathy, and renal dysfunction. Abdominal ultrasound and computed tomography abdominal scan revealed filling defects in intrahepatic veins and inferior vena cava extending to bilateral renal and external iliac veins. Extensive workup for thrombophilia and myeloproliferative disorders was negative. The diagnosis was hepatic dysfunction secondary to inferior vena cava obstruction due to a thrombus in the setting of extensive inferior vena cava thrombosis, and heparin was initiated. However, due to lack of recanalization with anticoagulation, we performed aspiration thrombectomy, balloon angioplasty, and local thrombolysis. Transjugular intrahepatic portosystemic shunt procedure was subsequently done due to hepatic venous congestion and refractory ascites. He was discharged on oral anticoagulation. Imaging exams performed 4 months later showed patent inferior vena cava and transjugular intrahepatic portosystemic shunt, good flows in the portal vein and resolution of ascites.