Budd-Chiari syndrome and acute portal vein thrombosis: management by a transjugular intrahepatic portosystemic shunt (TIPS) and portal vein interventions via a TIPS.

Acute portal vein thrombosis (PVT) is a devastating complication of Budd-Chiari syndrome (BCS). Conservative approach, anticoagulation, systemic or transarterial thrombolysis, and urgent liver transplantation were applied in this scenario but with poor results. We present and discuss an approach to treat BCS complicated by acute PVT. Two young female patients presented with acute liver failure, rapidly progressive tense ascites, renal- and respiratory failure. The diagnosis of chronic BCS complicated by acute PVT was confirmed with ultrasound Doppler. Initial treatment was supportive. Right portal vein localization was by transarterial portogram or by computed tomography-guided microcoil placement. Transjugular intrahepatic portosystemic shunt (TIPS) was performed and included Wallstents and a Jograft in one case and Viatorr stentgraft that was extended later with a Hemobahn stentgraft in another. Mechanical clot removal from the portal system was performed in the primary procedure and in a revision procedure in the following few days. Stents were placed precisely with no extension into the inferior vena cava or deeply into the main portal vein. Patients were fully anticoagulated and patency was assessed by ultrasound Doppler. The procedures were performed on days 5 and 10 following admission. In both cases, successful thrombectomies were revised and maintained. Partial occlusion of the TIPS and reaccumulation of ascites were reversed with repeated procedure. Both patients were discharged without ascites and normal liver function. In conclusion, urgent TIPS and portal vein thrombectomy via TIPS are emerging therapeutic options that offer a safe and effective treatment to patients with BCS complicated by acute portal vein thrombosis.