TIPS with a Twist - The Real Life Management of a Case of Budd-Chiari-related Acute Liver and Subsequent Multiple Organ Failure

Introduction: Budd-Chari syndrome (BCS) is a rare condition defined by the obstruction of hepatic venous outflow. BCS is a relatively infrequent cause of acute liver failure (ALF), accounting for less than 1% of cases. Treatment for acute BCS consists of a stepwise approach, requiring anticoagulation, angioplasty, transjugular intrahepatic portosystemic shunt (TIPS), and liver transplantation.

Case report: We present the case of a 31-year-old female patient with BCS, which led to ALF and subsequent multiple organ failure, which was successfully treated with TIPS and endovascular coil placement. Initial diagnostic workup revealed the complete obstruction of the hepatic venous outflow, spleno-mesenteric confluent thrombosis, and biochemical criteria of ALF. Her condition rapidly deteriorated towards multiple organ failure. At one point, the MELD score was 42, while the SOFA score predicted a mortality rate of >95%. Following continuous venovenous hemodiafiltration with cytokine adsorbent filters, TIPS was inserted, resulting in a portal pressure gradient (PPG) of 14 mmHg. Following TIPS, the patient had persistent ascites and later presented an episode of gastric variceal bleeding with endoscopic and surgical treatment failure. TIPS revision with further dilation led to a final PPG of 6 mmHg. During the procedure, selective embolization by coil placement of the spleno-gastric collateral circulation ultimately resolved the variceal bleeding. In the aftermath, the patient had complete organ failure remission and was successfully discharged with no ascites, encephalopathy, or significant impairment regarding daily life activities.

Conclusion: In the rare setting of BCS complicated with ALF and portal hypertension-related complications, TIPS and endovascular embolization provide a unique, effective, and against-all-odd solution.