Portal cavernoma or chronic non cirrhotic extrahepatic portal vein obstruction - 03/09/20

Doi : 10.1016/j.clinre.2020.03.016

Laure Elkrief ^a,^⁎ , Pauline Houssel-Debry ^b,^{^{1
Joint first authors.}}, Oanez Ackermann ^c,^g, Stéphanie Franchi-Abella ^d,^g, Sophie Branchereau ^e, Dominique Valla ^f,^g,^h, Sophie Hillaire ^h, Danielle Dutheil ^g,^i,^j, Aurélie Plessier ^f,^g,^h, Virginia Hernandez-Gea ^k, Christophe Bureau ^g,^l
^a Department of Gastroenterology and Hepatology, Trousseau Hospital, University Hospital of Tours, Avenue de la République, 37170 Chambray-lès-Tours, France
^b Department of liver disease, Pontchaillou Hospital, University Hospital of Rennes, 2, rue Henri le Guilloux 35033 Rennes cedex 9, France
^c Department of Pediatric Hepatology, Bicêtre Hospital, AP–HP, 78, rue du Général Leclerc 94270 Le Kremlin Bicêtre, France
^d Department of Pediatric Radiology, Bicêtre Hospital, AP–HP, 78, rue du Général Leclerc 94270 Le Kremlin Bicêtre, France
^e Department of Pediatric Visceral Surgery, Bicêtre Hospital, AP–HP, 78, rue du Général Leclerc 94270 Le Kremlin Bicêtre, France
^f Department of Hepatology, DHU Unity, Beaujon Hospital, AP–HP, 100, boulevard du Général Leclerc 92118 Clichy, France
^g French Network for Rare Liver Diseases (FILFOIE), Saint-Antoine Hospital, APHP, 184, rue du Faubourg Saint-Antoine, 75012 Paris, France
^h Reference center of vascular liver diseases, European Reference Network (ERN) Rare-Liver, Hamburg, Germany
ⁱ Department of Internal Medicine, Foch Hospital, 40, rue Worth, 92150 Suresnes, France
^j Association of patients with vascular liver diseases (AMVF), Beaujon Hospital, Department of Hepatology, 100, boulevard du Général Leclerc 92118 Clichy, France
^k Hepatic Hemodynamic Laboratory, Liver Unit, Hospital Clínic, Institut de IDIBAPS and CIBERehd, Barcelona, Spain
^l Department of Gastroenterology and Hepatology, Rangueil Hospital, University Hospital of Toulouse, 1, avenue du Professeur Jean Poulhès, 31400 Toulouse, France

^⁎Corresponding author.

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Resumen

Chronic non cirrhotic extrahepatic portal vein obstruction (EHPVO) refers to the cavernomatous transformation of the portal vein (the so-called “portal cavernoma”) which occurs following acute thrombosis of the portal vein in the absence of recanalization. In adults, EHPVO mainly occurs following thrombosis, while in children it may be related to congenital malformations and/or neonatal umbilical venous catheterization. However, 50% of the cases of EHPVO remain idiopathic [1]. Risk factors and associated diseases should be investigated (chapter 1). Indeed, the presence of a thrombophilic alteration, in particular myeloproliferative neoplasm impacts prognosis and determine a causal treatment.

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Keywords : Portal cavernoma, Cavernoma cholangiopathy, Variceal

Abbreviations : CT, EHPVO, INR, MPN, MRI, PVT, TIPS, VKA

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Introduction

What are the manifestations and how can we perform the diagnosis?

What are the manifestations?

What complications can occur?

How is the diagnosis obtained?

What treatments are available for chronic non cirrhotic extrahepatic portal vein obstruction?

Anticoagulation

How can portal hypertension-related complications be managed?

How should portal cavernoma cholangiopathy be managed?

When to perform surgery?

Recommendations

Disclosure of interest

Exportación

Vol 44 - N° 4

P. 491-496 - septembre 2020 Regresar al número

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Ischemic cholangiopathy: An update
Odile Goria, Isabelle Archambeaud, Caroline Lemaitre, Danielle Dutheil, Aurélie Plessier, Pierre-Emmanuel Rautou, Virginia Hernandez-Gea, Dominique Valla

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Portal cavernoma or chronic non cirrhotic extrahepatic portal vein obstruction - 03/09/20

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