2315 Early Detection and IR Intervention in a Case of Fulminant Budd-Chiari Syndrome

Abstract

INTRODUCTION: Budd-Chiari syndrome (BCS) is a relatively rare cause of abnormal LFTs, and infrequently progresses to fulminant hepatic failure. BCS requires a high degree of suspicion and can be easily confused with other causes of rapidly worsening liver enzymes. We present a case that illustrates successful management of fulminant hepatic failure by interventional radiology (IR) guided thrombolysis and thrombectomy, followed by TIPS placement in a rapidly decompensating 34-year-old woman who initially presented with mildly abnormal LFTs. CASE DESCRIPTION/METHODS: A 34-year-old white woman with past medical history of acid reflux, and family history of venous thromboembolism, presented to an outside hospital with a 5-day history of nausea, vomiting, diarrhea. She was subsequently was transferred to our facility for a higher level of care after worsening liver function testing and mentation. Patient had reported 5 days of epigastric tenderness, nausea, and abdominal distension prior to admission. Her LFTs continued to worsen, and clinically, she became progressively more hypotensive and tachycardic (BP 74/44, HR 112) requiring vasopressor support without significant improvement in her vital signs. Within 48 hours, she had acute onset of ascites and worsening encephalopathy. On arrival to our facility, she underwent CT abdomen/pelvis with and without IV contrast showing complete thrombosis of the right, left, and middle hepatic veins. Her liver function and transaminases continued to worsen prompting IR intervention. She underwent IR-guided thrombolysis and thrombectomy and TIPS with pre- and post- intervention pressures showing marked improvement in the transhepatic pressures (Table 1). (Pre- and post-intervention imaging in Figures 1 and 2). As part of her hypercoagulable workup, she was found to be deficient in protein C activity (46%). She had no OCP use for over 10 years. DISCUSSION: It was later discovered that patient’s mother had a history of saddle pulmonary embolism and other maternal relatives have had a pro-coagulative history, most of whom presented with significant lower extremity DVTs. This case illustrates that a high clinical suspicion is needed to make an early diagnosis of BCS, which can lead to early intervention and minimize the need for emergent liver transplantation. This case emphasizes the significant of a thorough history, and particularly a family history of venous thromboembolism.