Challenging anticoagulation cases: Acute extensive portal vein thrombosis in a patient without cirrhosis - Evidence-based management of a rare clinical entity

Acute non-cirrhotic and non-malignant portal vein thrombosis (aPVT) is a rare and heterogenous condition. Current guidelines recommend early initiation of therapeutic anticoagulation to prevent extension of thrombosis, and favor recanalization. Although not formally defined, a poor outcome in the acute setting would include thrombosis extension with progression to intestinal infarction. Patients are also at risk of negative long-term outcomes related to complications of portal hypertension, such as variceal bleeding, ascites, and portal cholangiopathy. Identifying patients at risk of these events despite early initiation of anticoagulation remains challenging. Trials comparing treatment strategies in those failing standard therapy with meaningful radiological and clinical endpoints, whether in the short or long term, are desperately needed. The objective of this review will be to discuss a real-life clinical case and propose a treatment approach for aPVT based on the available evidence. We will mainly focus on management strategies including anticoagulation, prognostic factors, and options beyond anticoagulation, such as thrombolysis, thrombectomy, and transjugular intrahepatic portosystemic shunts. This review will not cover tumor portal vein thrombosis or thrombosis associated with cirrhosis.