Budd-Chiari Syndrome and Portal Vein Thrombosis in Crohnʼs Disease

Abstract

Introduction: Untreated Crohn's disease (CD) is a prothrombotic state associated with venous thromboembolism and can present with portal vein thrombosis, deep venous thrombosis and pulmonary embolism (PE). There are a handful reported cases of CD presenting as hepatic vein thrombosis or Budd-Chiari syndrome. Case description: A 27 year old male presented with hematochezia, abdominal pain, anasarca with ascites, fatigue and hepatomegaly. A year prior to presentation he developed hematochezia and was seen at a different hospital where he underwent colonoscopy that was suggestive of inflammatory bowel disease but was lost to follow up and remained untreated and continued to have intermittent hematochezia and abdominal pain. On admission his Hb was 5.7 g/dL with elevated ALT (83 U/L) and AST (81 U/L) and albumin of 2.1 g/dL. Stool studies were negative for C difficile toxins, ova & parasites, enteric cultures but positive for leukocytes. Colonoscopy was performed that showed terminal ileitis and pancolitis with rectal pseudopolyp formation. EGD demonstrated duodenal ulcerations and inflammation of pylorus which on biopsy showed non-necrotizing granuloma formation that was consistent with a diagnosis of CD (Figure 1). His hypoalbuminemia was suspected to be due to protein-losing enteropathy from active enterocolitis.Figure 1Paracentesis of the ascitic fluid showed a serum:ascites albumin gradient of >1.4 which suggested portal hypertension. CT scan of abdomen demonstrated hepatomegaly with thrombi in the inferior vena cava, portal vein and right hepatic vein (Figure 2). Liver biopsy showed extensive perivenular sinusoidal dilation, areas of hepatocyte atrophy and drop-out, and mild centrizonal fibrosis (Budd-Chiari syndrome; Figure 3). He was then anti-coagulated with heparin drip and transitioned to apixiban. His anasarca improved with diuretics and CD was treated with prednisone and mesalamine at the time of discharge. Discussion: Active CD is a prothrombotic state that can lead to Budd-Chiari syndrome and subsequent hepatocyte injury as a result of venous congestion. Protein losing enteropathy can develop as a result of untreated CD leading to anasarca and loss of procoagulant factors which further increases the risk of venous thrombosis. Early recognition of thrombotic complications of CD and initiation of anticoagulation is recommended to prevent hepatocyte injury from venous congestion or fatal complications like development of PE.Figure 2Figure 3