S5326 Fatal Outcome of a Rare TIPS-Biliary Fistula: Lessons From Recurrent Infection

Introduction: Transjugular intrahepatic portosystemic shunt (TIPS) placement is a well-established intervention for managing complications of portal hypertension, including refractory ascites and variceal bleeding. While TIPS-related infections are rare, recurrent episodes should prompt investigation for structural complications. We present a unique case of recurrent TIPS-associated infection caused by a fistulous connection between the TIPS and biliary tree—an exceedingly rare and underreported complication. Case Description: A 33-year-old man with chronic portal vein thrombosis due to homozygous MTHFR mutation and a history of TIPS placement (2/2020) for portal hypertension presented with fever, abdominal pain, emesis, and diarrhea. He had a history of recurrent TIPS-associated infections (TIPSitis) beginning 8 months post-placement, despite chronic antibiotic suppression and multiple TIPS revisions (12/2020, 1/2021, 4/2025). He also required biliary stenting for obstruction from venous collaterals. Blood cultures across hospitalizations grew Streptococcus viridans, Citrobacter, Enterococcus faecalis, and Candida species. Prior endoscopic retrograde cholangiopancreatography (ERCP) in 3/2024 raised concern for a TIPS-biliary fistula, though contrast extravasation was not seen. During this admission, magnetic resonance cholangiopancreatography revealed an occluded TIPS and biliary dilation near a suspected erosion site. ERCP confirmed contrast extravasation from the biliary tree into the TIPS. A fully covered metal stent was placed as a temporizing measure while he underwent evaluation for multivisceral transplantation. He later died from an upper gastrointestinal bleed. Discussion: TIPS-biliary fistula is an exceptionally rare but serious complication, with only 3 similar cases reported—all requiring transplantation. These fistulas provide a persistent route for microbial translocation, leading to recurrent infections refractory to antibiotics. While fistula formation may occur acutely due to bile duct injury, delayed onset is likely related to stent-induced pressure necrosis or ischemia from thrombosis and repeated revisions. No standardized treatment exists; covered stent placement may offer temporary control. In patients with recurrent, polymicrobial, or fungal TIPSitis, clinicians should maintain high suspicion for TIPS-biliary fistula. Early recognition is key to guide timely intervention and consideration for transplant.