Idiopathic portal vein thrombosis not related to hepatic disease or malignancy

Acute portal vein thrombosis (PVT) is a rare disorder defined by the sudden occlusion of the portal vein, which could be partial or complete. Prothrombotic states, inherited or acquired, are thought to be the cause in patients without cirrhosis or malignancy. However, the aetiology of some cases remains idiopathic despite a multidisciplinary diagnostic approach. The initial diagnostic modality to confirm PVT is either contrast-enhanced abdominal (CT) or MRI; as it can identify predisposing factors, and detect evidence of complications. Eliciting the underlying aetiology is critical to guide overall management and prevent future recurrence. The purpose of treatment is to stop thrombus extension and achieve portal vein patency by anticoagulation to optimise outcomes. Herein, we present an unusual case of spontaneous PVT in a young woman. We will also discuss the evaluation of patients without obvious aetiology.