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Nonmalignant portal vein thrombosis in adults

Nature Clinical Practice Gastroenterology & Hepatology volume 3, pages 505–515 (2006)Cite this article

Abstract

Portal vein thrombosis (PVT) consists of two different entities: acute PVT and chronic PVT. Acute PVT usually presents as abdominal pain. When the thrombus extends to the mesenteric venous arches, intestinal infarction can occur. Chronic PVT is usually recognized after a fortuitous diagnosis of hypersplenism or portal hypertension, or when there are biliary symptoms related to portal cholangiopathy. Local risk factors for PVT, such as an abdominal inflammatory focus, can be identified in 30% of patients with acute PVT; 70% of patients with acute and chronic PVT have a general risk factor for PVT, most commonly myeloproliferative disease. Early initiation of anticoagulation therapy for acute PVT is associated with complete and partial success in 50% and 40% of patients, respectively. A minimum of 6 months' anticoagulation therapy is recommended for the treatment of acute PVT. For patients with either form of PVT, permanent anticoagulation therapy should be considered if they have a permanent risk factor. In patients with large varices, β-adrenergic blockade or endoscopic therapy seems to prevent bleeding as a result of portal hypertension, even in patients on anticoagulation therapy. In patients with jaundice or recurrent biliary symptoms caused by cholangiopathy, insertion of a biliary endoprosthesis is the first treatment option. Overall, the long-term outcome for patients with PVT is good, but is jeopardized by cholangiopathy and transformation of underlying myeloproliferative disease into myelofibrosis or acute leukemia.

Key Points

  • Acute or chronic portal vein thrombosis (PVT) suggests the presence of one or several underlying prothrombotic disorders

  • Myeloproliferative disease that lacks the usual diagnostic features is the most frequent prothrombotic disorder in patients with PVT

  • Major complications comprise intestinal infarction, chronic portal hypertension, and portal cholangiopathy, while liver function is preserved

  • In most patients with acute PVT, complete or partial recanalization is achieved with early anticoagulation therapy

  • Permanent anticoagulation should be considered in patients with permanent prothrombotic disorders who present with acute or chronic PVT

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Figure 1: Acute thrombosis of the portal vein.
Figure 2: Acute thrombosis of the right branch of the portal vein.
Figure 3: Acute thrombosis of the main portal vein.
Figure 4: Acute portal vein thrombosis.
Figure 5: Venous intestinal ischemia.
Figure 6: Mesenteric vein and portal vein gas.
Figure 7: Cavernous transformation of the portal vein in a patient with chronic portal vein thrombosis.
Figure 8: Portal cholangiopathy in a patient with chronic portal vein thrombosis.
Figure 9: Portal vein thrombosis in a patient with cirrhosis.

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Acknowledgements

We are most indebted to Valérie Vilgrain (Service de Radiologie), Marie-Hélène Denninger (Service d'Immunohématologie), Dominique Cazals-Hatem (Service d'Anatomie et Cytologie Pathologiques), and Jean Brière and Jean-Jacques Kiladjian (Service d'Hématologie), Hôpital Beaujon, Clichy, for their collaboration. Supported in part by the fifth framework program of the European Union (EN-Vie QLG1-CT-2002-16786), GIS Maladies Rares, and French Ministère de la Santé (Centre de référence des Maladies Vasculmaires du Foie).

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  1. Service d'Hépatologie, Hôpital Beaujon, Clichy and in the Service d'Hépatogastroentérologie, Hôpital Saint Camille, Bry-sur-Marne

    Bertrand Condat & Dominique Valla

  2. Professor of Hepatology and Head of the Service d'Hépatologie, Hôpital Beaujon, Clichy, France

    Bertrand Condat & Dominique Valla

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Correspondence to Dominique Valla.

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Condat, B., Valla, D. Nonmalignant portal vein thrombosis in adults. Nat Rev Gastroenterol Hepatol 3, 505–515 (2006). https://doi.org/10.1038/ncpgasthep0577

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