Clinical decision making in acute Budd Chiari Syndrome.

A 51-year-old male firefighter with a remote history of deep vein thrombosis following ankle fracture was hospitalized at our center for a 2 week history of jaundice, ascites and right upper quadrant abdominal pain. His bloodwork on admission was significant for ALT and AST 100 times the upper limit of normal, bilirubin 30 mg/dL (normal 322), hemoglobin 16.8 mg/dL (normal 13.5-16), platelets 570 x 109 cells/mL (normal 150-400 x 109) and INR 1.5 (normal 0.9-1.1). CT scan revealed caudate lobe hypertrophy, compression of the inferior vena cava (ICV) and opacification of the hepatic veins (Figure 1), consistent with a diagnosis of Budd Chiari Syndrome (BCS). Further work-up revealed Janus kinase 2 mutation, confirming polycythemia ruba vera. He was started on anticoagulation and diuretics, but had no improvement after 5 days. A hepatic venogram showed total obstruction of the 3 hepatic veins, and severe IVC stenosis; thus, the portal vein could not be accessed to perform a transjugular intrahepatic portosystemic shunt (TIPS). Liver biopsy showed zone 3 occlusion and cell dropout, consistent with venous outflow obstruction. A surgical side-toside portocaval shunt was performed on day 7 of admission. Due to marked hypertrophy of the caudate lobe, the distance from the portal vein to IVC was excessive at 4.1 cm, necessitating a prosthetic shunt. However, postoperatively, he had worsening liver function with new onset of progressive hepatic encephalopathy and acute renal injury. He was therefore promptly listed for transplantation for fulminant liver failure. After 6 days, he underwent ABO incompatible liver transplantation (ILT); a veno-venous bypass was required before the hepatectomy. The patient received plasmapheresis and rituximab following the ILT. He gradually recovered, and remains stable at 12 months post-ILT, maintained on coumadin and tacrolimus. BCS is a rare condition defined as hepatic venous outflow obstruction originating anywhere from the small hepatic veins to the right atrium. The classic presentation is a triad of abdominal pain, ascites and hepatomegaly, but there is a wide spectrum of clinical manifestations from asymptomatic to acute liver failure. Most patients with BCS have an underlying thrombophilic disorder, namely myeloproliferative syndrome.1 Five-year survival rates from diagnosis of Budd Chiari range from 50 to 80%.1 Age, response of ascites to diuretics, Child score and serum creatinine