Psuedo Budd-Chiari Syndrome: An Uncommon Presentation of Very Common Disease

Abstract

Introduction Alcoholic steatohepatitis and liver cirrhosis are the most common diagnosis in gastroenterology. Here we are writing about a different disease with a similar presentation to this common condition. Before considering this diagnostic possibility, it is important to rule out this common diagnosis. Case description A 25 year old female with Past Medical History of alcoholic liver cirrhosis presented with bright red bleeding per rectum of 2 days duration. She had approximately 15-20 bloody bowel movements, which were associated with lower abdominal cramps. In addition, she was feeling fatigued and occasionally dizzy. On physical examination, her vitals were normal except tachycardia. On examination, she looked pale. Few spider-angioma were present over face and body. Rest of the examination was non-contributory. Pertinent laboratory findings included Hemoglobin 3.6, WBC 16 thousand, INR 1.4, PT 15.2, APTT 29.1, T Bili 1.7, ALP 242, and Albumin 2.2. Initially, she was treated with 3U of Packed Red Blood Cells. Ultrasonography of abdomen with Doppler showed reverse flow within main, right and left portal veins and splenic veins. Left, middle and right hepatic veins were not well visualized. Diagnostic hepatic venography demonstrated decreased flow in the hepatic veins proximal to the Inferior Vena Cava confluence without any visible clot. Hepatic veins to Inferior Vena Cava pressure gradient was 5 mmHg. CT of Abdomen confirmed hepatomegaly, caudate lobe hypertrophy, features of cirrhosis and portal hypertension. She had an endoscopy showing grade 2 esophageal varices and type1 isolated gastric varices. As she continued to bleed, she underwent a colonoscopy which did not show any pathology. Eventually, she was considered for TIPS procedure for ongoing bleeding. Further, liver biopsy showed steatohepatitis with bridging fibrosis to early cirrhosis (Stage 3-4). And her hyper-coagulation panel and myeloproliferative workup were negative. After this extensive workup and management, she recovered well and was discharged in stable condition. Discussion Pseudo-Budd Chiari Syndrome is one of the rare diagnosis in the field of GI. Until this date only 4 cases have been described in the literature and only one case had decompensated liver cirrhosis. As radiological diagnosis may be false positive, arteriography and/or liver biopsy should always be considered. Possible reversibility with alcohol abstinence makes this clinical entity a must for physicians to know.Figure: US abdomen with color Doppler showing decreased flow in the hepatic veins proximal to the IVC.Figure: EGD showing esophageal varices.Figure: Normal colonoscopy.