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Extrahepatic Portal Vein Obstruction: Recent Portal Vein Thrombosis and Portal Cavernoma in the Absence of Cirrhosis

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Abstract

Extrahepatic portal vein obstruction (EHPVO) refers to the obstruction of the portal vein and/or right or left branches, extending or not to mesenteric or splenic veins, the obstruction being complete or partial. This chapter will focus on EHPVO, unrelated to a malignant invasion (and thus corresponding to thrombosis) in the absence of cirrhosis. After a recent occlusion of the portal venous lumen, a network of porto-portal collaterals develop which, collectively are denominated the portal cavernoma. In adults, one or multiple prothrombotic diseases are identified in 60% of the patients, the leading cause being myeloproliferative neoplasms (MPN), associated or not with a local cause in 30%. In children, etiological investigation usually fails to document an underlying condition. Manifestations range in severity from the absence of symptoms to intestinal infarction—the most dreaded immediate complication—and vary according to the time and site of obstruction. Complications of chronic EHPVO include portal hypertension related bleeding, symptomatic portal cholangiopathy and recurrent thrombosis.

Specific and sensitive EHPVO diagnosis criteria have been defined using contrast enhanced vascular imaging. In adults with recent portal vein thrombosis, immediate anticoagulation therapy, maintained for 3–6 months is recommended. In chronic EHPVO, anticoagulation is maintained or initiated in patients with a strong prothrombotic condition (based on personal and familial history of unprovoked deep vein thrombosis, and on findings of isolated or combined prothrombotic conditions), or past history suggesting intestinal ischemia or recurrent thrombosis on follow-up.

In patients with uncontrollable severe symptoms, resection of the necrotic gut in recent mesenteric infarction, and recanalization attempt to reestablish a physiological venous outflow or endoscopic treatment of symptomatic biliary strictures in portal cavernoma are the final steps of the management.

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Abbreviations

CT scan:

Computerized tomography scan

DOACS:

Direct oral anticoagulants

EHPVO:

Extrahepatic portal vein obstruction

HCC:

Hepatocellular carcinoma

HCA:

Hepatic cell adenoma

MPN:

Myeloproliferative neoplasm

MRI:

Magnetic resonance imaging

SIRS:

Systemic inflammatory response syndrome

TIPS:

Transjugular porto systemic shunt

PSVD:

Porto sinusoidal vascular disease

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Authors and Affiliations

  1. Centre de Référence des Maladies Vasculaires du Foie, Inserm U1149, Centre de Recherche sur l’Inflammation (CRI), Université Paris 7-Denis-Diderot, Paris, France

    Aurélie Plessier

  2. Fil-foie, ERN Rare Liver, Clichy, France

    Aurélie Plessier

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  1. Aurélie Plessier
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Editors and Affiliations

  1. CRI, UMR 1149, Université de Paris and Inserm, Paris, France

    Dominique Valla

  2. Hospital Clinic, Hepatic Hemodynamic Lab Hospital Clinic, Barcelona, Spain

    Juan Carlos Garcia-Pagan

  3. Gastroenterology and Hepatology, Ente Ospedaliero Cantonale, Lugano, Switzerland

    Andrea De Gottardi

  4. Université de Paris, Paris, France

    Pierre-Emmanuel Rautou

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Plessier, A. (2022). Extrahepatic Portal Vein Obstruction: Recent Portal Vein Thrombosis and Portal Cavernoma in the Absence of Cirrhosis. In: Valla, D., Garcia-Pagan, J.C., De Gottardi, A., Rautou, PE. (eds) Vascular Disorders of the Liver . Springer, Cham. https://doi.org/10.1007/978-3-030-82988-9_7

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