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Portal Vein Thrombosis

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Vascular Liver Disease

Abstract

Portal vein obstruction is a common finding at necropsy where it is usually related to cirrhosis or malignancy of abdominal organs. Noncirrhotic, nonmalignant portal vein thrombosis is uncommon. The main risk factors for noncirrhotic nonmalignant portal vein thrombosis are inflammatory foci in the abdomen, abdominal surgery (particularly splenectomy), myeloproliferative diseases, and factor II gene mutation. Acute portal vein thrombosis usually manifests itself with abdominal pain and a systemic inflammatory reaction. In the most severe forms of acute portal vein thrombosis intestinal infarction may occur. Chronic portal thrombosis is characterized by cavernomatous transformation, and is associated with portal hypertension. Diagnosis is based on a high degree of clinical suspicion, and findings at imaging with CT or Doppler ultrasound. Recognition of underlying myeloproliferative disease has been facilitated by testing for V617F JAK2 mutation in peripheral blood. Therapy for acute portal vein thrombosis is based on early anticoagulation, which prevents extension of the thrombus in most patients, but achieves recanalization in less than 40% of them. Treatment for chronic portal vein thrombosis consists of pharmacological or endoscopic prophylaxis of bleeding from portal hypertension. Surgery can also be done in selected cases. Anticoagulation is logical in patients with underlying high-risk thrombophilia. Some findings suggest that anticoagulation could be of clinical benefit without increasing the risk of bleeding from portal hypertension.

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Valla, DC. (2011). Portal Vein Thrombosis. In: DeLeve, L., Garcia-Tsao, G. (eds) Vascular Liver Disease. Springer, New York, NY. https://doi.org/10.1007/978-1-4419-8327-5_12

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