Portal cavernoma or chronic non cirrhotic extrahepatic portal vein obstruction

Laure Elkrief; Pauline Houssel-Debry; Oanez Ackermann; Stéphanie Franchi-Abella; Sophie Branchereau; Dominique Valla; Sophie Hillaire; Danielle Dutheil; Aurélie Plessier; Virginia Hernandez-Gea; Christophe Bureau

doi:10.1016/j.clinre.2020.03.016

Portal cavernoma or chronic non cirrhotic extrahepatic portal vein obstruction

Clin Res Hepatol Gastroenterol. 2020 Sep;44(4):491-496. doi: 10.1016/j.clinre.2020.03.016. Epub 2020 Aug 17.

Affiliations

¹ Department of Gastroenterology and Hepatology, Trousseau Hospital, University Hospital of Tours, Avenue de la République, 37170 Chambray-lès-Tours, France. Electronic address: laure_elkrief@yahoo.fr.
² Department of liver disease, Pontchaillou Hospital, University Hospital of Rennes, 2, rue Henri le Guilloux 35033 Rennes cedex 9, France.
³ Department of Pediatric Hepatology, Bicêtre Hospital, AP-HP, 78, rue du Général Leclerc 94270 Le Kremlin Bicêtre, France; French Network for Rare Liver Diseases (FILFOIE), Saint-Antoine Hospital, APHP, 184, rue du Faubourg Saint-Antoine, 75012 Paris, France.
⁴ Department of Pediatric Radiology, Bicêtre Hospital, AP-HP, 78, rue du Général Leclerc 94270 Le Kremlin Bicêtre, France; French Network for Rare Liver Diseases (FILFOIE), Saint-Antoine Hospital, APHP, 184, rue du Faubourg Saint-Antoine, 75012 Paris, France.
⁵ Department of Pediatric Visceral Surgery, Bicêtre Hospital, AP-HP, 78, rue du Général Leclerc 94270 Le Kremlin Bicêtre, France.
⁶ Department of Hepatology, DHU Unity, Beaujon Hospital, AP-HP, 100, boulevard du Général Leclerc 92118 Clichy, France; French Network for Rare Liver Diseases (FILFOIE), Saint-Antoine Hospital, APHP, 184, rue du Faubourg Saint-Antoine, 75012 Paris, France; Reference center of vascular liver diseases, European Reference Network (ERN) Rare-Liver, Hamburg, Germany.
⁷ Reference center of vascular liver diseases, European Reference Network (ERN) Rare-Liver, Hamburg, Germany.
⁸ French Network for Rare Liver Diseases (FILFOIE), Saint-Antoine Hospital, APHP, 184, rue du Faubourg Saint-Antoine, 75012 Paris, France; Department of Internal Medicine, Foch Hospital, 40, rue Worth, 92150 Suresnes, France; Association of patients with vascular liver diseases (AMVF), Beaujon Hospital, Department of Hepatology, 100, boulevard du Général Leclerc 92118 Clichy, France.
⁹ Hepatic Hemodynamic Laboratory, Liver Unit, Hospital Clínic, Institut de IDIBAPS and CIBERehd, Barcelona, Spain.
¹⁰ French Network for Rare Liver Diseases (FILFOIE), Saint-Antoine Hospital, APHP, 184, rue du Faubourg Saint-Antoine, 75012 Paris, France; Department of Gastroenterology and Hepatology, Rangueil Hospital, University Hospital of Toulouse, 1, avenue du Professeur Jean Poulhès, 31400 Toulouse, France.

PMID: 32819872
DOI: 10.1016/j.clinre.2020.03.016

Abstract

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.

Keywords: Cavernoma cholangiopathy; Portal cavernoma; Variceal.

MeSH terms

Chronic Disease
Humans
Hypertension, Portal / diagnosis*
Hypertension, Portal / therapy*
Portal Vein / abnormalities*
Practice Guidelines as Topic

Supplementary concepts

Portal Vein, Cavernous Transformation Of