S5773 Budd-Chiari Syndrome in a Patient of Ulcerative Colitis Treated With Transjugular Intrahepatic Portosystemic Shunt (TIPS): A Case Report

Introduction: Ulcerative colitis (UC) is a well-known hypercoagulable condition with a threefold increased risk of deep venous thrombosis (DVT) and pulmonary embolism. However, thrombosis of systemic veins is rare. Herein, we present a case of Budd-Chiari syndrome (BCS) treated with transjugular intrahepatic portosystemic shunt (TIPS) in a patient with UC. Case Description/Methods: A 24-year-old woman with a past medical history of UC presented to the emergency department with abdominal pain, vomiting, and bloody diarrhea. On physical examination, she was afebrile with blood pressure 122/92 mm Hg and pulse 135 bpm; her abdomen was soft and non-distended but tender in the right upper quadrant. Laboratory evaluation revealed a hemoglobin of 6.4 g/dl, white blood cell count of 14.7 cells/μL, lactic acid 6.1 mmol/L, aspartate aminotransferase 5858 U/L and alanine aminotransferase 3543 U/L, prothrombin time 37.3 seconds and international normalized ratio 3.82. Computed tomography (CT) of abdomen and pelvis showed hepatic congestion and thrombosis of hepatic veins, portal vein and splenic vein. Sigmoidoscopy revealed pancolitis. Her infectious work-up including blood cultures, viral hepatitis panel, Clostridium difficile infection screen, stool bacterial and viral PCR, and HIV were all negative. She was started on systemic steroids, empiric antibiotics and low-molecular-weight-heparin. For definitive treatment of fulminant hepatic failure, TIPS was performed which relieved the portal hypertension. The patient’s condition stabilized and she was discharged home. Discussion: Budd-Chiari syndrome is a rare but life-threatening manifestation of UC. The underlying mechanism may include an imbalance of procoagulant and anticoagulant factors in the blood induced by the widespread inflammatory processes. Extensive colonic disease, recent surgery and inherited and acquired disorders of thrombogenesis may predispose the patient to this condition. If the patient does not respond to initial anticoagulation, more aggressive treatment in the form of TIPS may be required to relieve hepatic congestion. A high index of suspicion is required for timely diagnosis and management of this rare condition.