Management of splanchnic vein thrombosisKey points
Laure Elkrief,
Audrey Payancé,
Aurélie Plessier,
Louis d’Alteroche,
Maxime Ronot,
Valérie Paradis,
Dominique Valla,
Pierre-Emmanuel Rautou
Affiliations
Laure Elkrief
Service d’Hépato-Gastroentérologie CHU de Tours, France
Audrey Payancé
Université Paris-Cité, Inserm, Centre de recherche sur l'inflammation, UMR 1149, Paris, France; Service d'Hépatologie, AP-HP, Hôpital Beaujon, DMU DIGEST, Centre de Référence des Maladies Vasculaires du Foie, FILFOIE, ERN RARE-LIVER, Clichy, France
Aurélie Plessier
Université Paris-Cité, Inserm, Centre de recherche sur l'inflammation, UMR 1149, Paris, France; Service d'Hépatologie, AP-HP, Hôpital Beaujon, DMU DIGEST, Centre de Référence des Maladies Vasculaires du Foie, FILFOIE, ERN RARE-LIVER, Clichy, France
Louis d’Alteroche
Service d’Hépato-Gastroentérologie CHU de Tours, France
Maxime Ronot
Université Paris-Cité, Inserm, Centre de recherche sur l'inflammation, UMR 1149, Paris, France; Service de radiologie, Hôpital Beaujon APHP.Nord, Clichy, France
Valérie Paradis
Université Paris-Cité, Inserm, Centre de recherche sur l'inflammation, UMR 1149, Paris, France; Service d’anatomie et cytologie pathologique, Hôpital Beaujon APHP.Nord, Clichy, France
Dominique Valla
Université Paris-Cité, Inserm, Centre de recherche sur l'inflammation, UMR 1149, Paris, France; Service d'Hépatologie, AP-HP, Hôpital Beaujon, DMU DIGEST, Centre de Référence des Maladies Vasculaires du Foie, FILFOIE, ERN RARE-LIVER, Clichy, France
Pierre-Emmanuel Rautou
Université Paris-Cité, Inserm, Centre de recherche sur l'inflammation, UMR 1149, Paris, France; Service d'Hépatologie, AP-HP, Hôpital Beaujon, DMU DIGEST, Centre de Référence des Maladies Vasculaires du Foie, FILFOIE, ERN RARE-LIVER, Clichy, France; Corresponding author. Address: Service d’Hépatologie, Hôpital Beaujon, 100 boulevard du Général Leclerc, 92110, Clichy, France; Tel.: +331 40 87 50 91, fax +331 40 87 44 35
Summary: The expression splanchnic vein thrombosis encompasses Budd-Chiari syndrome and portal vein thrombosis. These disorders have common characteristics: they are both rare diseases which can cause portal hypertension and its complications. Budd-Chiari syndrome and portal vein thrombosis in the absence of underlying liver disease share many risk factors, among which myeloproliferative neoplasms represent the most common; a rapid comprehensive work-up for risk factors of thrombosis is needed in these patients. Long-term anticoagulation is indicated in most patients. Portal vein thrombosis can also develop in patients with cirrhosis and in those with porto-sinusoidal vascular liver disease. The presence and nature of underlying liver disease impacts the management of portal vein thrombosis. Indications for anticoagulation in patients with cirrhosis are growing, while transjugular intrahepatic portosystemic shunt is now a second-line option. Due to the rarity of these diseases, studies yielding high-grade evidence are scarce. However, collaborative studies have provided new insight into the management of these patients. This article focuses on the causes, diagnosis, and management of patients with Budd-Chiari syndrome, portal vein thrombosis without underlying liver disease, or cirrhosis with non-malignant portal vein thrombosis.
