S5633 Liver Let Die: A Malignant Budd-Chiari Crisis

Introduction: Budd-Chiari syndrome (BCS) is a rare condition caused by hepatic venous outflow obstruction, classified as primary (intrinsic venous thrombosis) or secondary (external compression or invasion by malignancy or other pathology). Clinical presentation varies from subacute ascites to fulminant hepatic failure, making early diagnosis essential. This case highlights rapidly progressive secondary BCS in a patient with untreated hepatitis C virus (HCV), cirrhosis, and malignancy, complicated by hepatic infarction, hemorrhagic ascites, and multi-organ failure. Case Description/Methods: A 71-year-old woman with hypertension, untreated HCV, and a history of non-small cell lung cancer (status post left upper lobectomy in 2019), presented with 5 days of abdominal pain and distension. She met criteria for sepsis and was treated with intravenous fluids and broad-spectrum antibiotics (vancomycin and piperacillin-tazobactam). Labs showed a white blood cell count of 33.4 × 10⁹/L, platelet count 158 × 10⁹/L, international normalized ratio (INR) 5.3, total bilirubin 5.1 mg/dL, alanine aminotransferase 133 U/L, aspartate aminotransferase 269 U/L, albumin 2.5 g/dL, and lactic acid 6.0 mmol/L. Computed tomography (CT) of the abdomen and pelvis (AP) demonstrated cirrhotic-type colopathy, abnormal liver architecture, and pulmonary nodules suspicious for metastases. Blood and urine cultures showed no growth. Despite intravenous vitamin K and antibiotics, INR and lactic acid worsened to 7.1 and 9.9 mmol/L respectively. Ultrasound showed a cirrhotic liver with reversed (hepato-fugal) portal venous flow. CT angiography revealed thrombus in the hepatic veins and hypoattenuation of the liver concerning for infarction. Fresh frozen plasma was perfused, leading to improvement in INR to 3.1. The patient was transferred to a tertiary center for trans-jugular intrahepatic portosystemic shunt (TIPS) placement, inferior vena cava venography (further confirming BCS), and mechanical thrombectomy. Paracentesis yielded 1,700 mL of bloody ascitic fluid with malignant cells. She developed refractory septic and hypovolemic shock and passed away following transition to comfort care. Discussion: This case highlights the rapid progression and high mortality associated with secondary BCS in patients with underlying malignancy and chronic liver disease. Findings such as hepatofugal flow and hepatic infarction warrant urgent hepatology and interventional radiology involvement. Despite timely intervention, prognosis remains poor in advanced disease.