An Interesting Case of Chronic Idiopathic Non-cirrhotic Portal Vein Thrombosis

Abstract

Purpose: Introduction: Portal vein thrombosis was first reported in 1868 by Balfour and Stewart. It is a rare condition that typically presents in non-cirrhotic patients. Inherited (Factor V Leiden and Prothrombin gene mutation G201210A, Protein C, S, Anti thrombin III deficiency) and acquired thrombophilias (Lupus Anticoagulant, myeloproliferative diseases, malignancy, surgery and trauma) account for majority of the cases of portal vein thrombosis. Doppler ultrasound studies are usually the initial test of choice. Case Report: 63 year old Hispanic Female with history of hypertension, Diabetes Mellitus, coronary artery disease presented with complaints of epigastric pain & bloody vomiting. She initially had epigastric discomfort & 2 episodes of hematemesis. She denied alcohol use. Abdominal examination was unremarkable except for mild epigastric tenderness. Laboratory analysis revealed hemoglobin of 10.5 mg/dl with normal liver function tests and aminotransferases. An upper gastrointestinal endoscopic examination was done which revealed grade 4 esophageal varices with fresh blood in the distal esophagus which were subsequently ligated. She was started on octreotide and propranolol. She had another episode of GI bleed requiring emergent endoscopic intervention. She underwent a liver biopsy. Pathology revealed focal mild portal fibrosis with mild micro & macro vesicular steatosis but no cirrhosis. A Doppler Ultrasound revealed portal vein thrombosis and concomitant portal hypertension. An extensive hypercoaguable work up was done which included protein C & S levels, anti thrombin III, Prothrombin gene mutation G20210A, Factor V Leiden, Lupus anticoagulant, Anticardiolipin antibodies, Homocysteine level & they were all negative. We also tested her blood for flow-cytometry for CD 55 and CD 59 but the test was normal and in the process effectively ruled out paroxysmal nocturnal hemoglobinuria. She also was also tested for the JAK 2 mutation. It was negative. We gave her the clinical diagnosis of chronic idiopathic non-cirrhotic portal vein thrombosis. She subsequently had a meso-caval shunt done to relieve the portal hypertension. She was started on warfarin anticoagulation 3 days later with close monitoring for bleeding. She clinically got better and was then discharged home. Discussion: Non-cirrhotic portal vein thrombosis can be acute or chronic. Acute cases need at least 3 months of anticoagulation. Chronic cases needs porto-systemic shunting. The use of anticoagulation in chronic cases should be decided upon on a case by case basis weighing the risk of bleeding versus thrombosis. We opted to give longterm anticoagulation to our patient to prevent re-thrombosis of the mesocaval shunt/graft.