Fulminant Hepatic Failure from Budd-Chiari Syndrome Treated with TIPS

Abstract

Budd-Chiari syndrome (BCS) is a rare disease caused by hepatic venous outflow obstruction. Presentation can include fulminant hepatic failure. Few treatment options, including emergent surgical decompression and liver transplantation, are available for fulminant BCS. Anticoagulation is generally not effective. Previous case reports and small series have shown TIPS (transjugular intrahepatic portosystemic shunt) to have poor outcomes in fulminant BCS. We present a case of fulminant hepatic failure due to BCS successfully treated with TIPS. A 27 year old male with no past history presented with abdominal pain, jaundice, and elevated liver function tests (LFT's). His laboratory data revealed albumin 3.0 mg/dL, bilirubin 5.8 mg/dL, AST 674 U/L, ALT 434 U/L, and INR 2.62. Ultrasound (US) and CT scans revealed occluded hepatic veins, caudate lobe hypertrophy, and massive ascites. Venogram revealed completely thrombosed hepatic veins, and a stenotic intrahepatic inferior vena cava. Liver biopsy showed dilated sinusoids, peri-central vein (zone 3) necrosis and hemorrhage, and bridging fibrosis. The patient was listed for emergent liver transplantation. Due to worsening mental status with asterixis, worsening LFT's (bilirubin 8.0, AST 1245, ALT 672) and INR (4.16), an emergent TIPS was placed as a temporizing, decompressive measure. The TIPS was placed from the right hepatic vein stump to the right portal vein using a covered endoprosthesis. The hepatic venous pressure gradient decreased from 52 mmHg to 11 mmHg. Hypercoagulable work-up was negative. Three months after the TIPS placement (without the need for liver transplantation), the patient has returned to work. His TIPS is patent by US. Except for a bilirubin of 2.1 mg/dL and an INR of 1.75, his LFT's have normalized. Treatment options are limited for fulminant hepatic failure from BCS. Emergent surgical decompression is limited by the lack of local expertise, and significant morbidity and mortality. Emergent liver transplantation is limited by organ availability. Our case demonstrates that TIPS can be performed successfully and safely in a patient with fulminant hepatic failure from BCS. TIPS can be used as a temporizing bridge to liver transplantation, or possibly as definitive therapy.