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Current guidelines for diagnosis and management of hepatic involvement in hereditary hemorrhagic teleangiectasia

Luca Ielasi1,2, Matteo Tonnini1,3, Fabio Piscaglia1,3

  • 1Department of Medical and Surgical Sciences, University of Bologna, Bologna 40138, Italy.

World Journal of Hepatology
|June 12, 2023
PubMed

Hereditary hemorrhagic teleangiectasia (HHT), also known as Rendu-Osler-Weber syndrome, is the most common cause of hepatic vascular malformations in adults. Different vascular shunts (arteriovenous, arterioportal or portovenous) lead to different clinical manifestations. Even though no hepatic-related symptoms are reported in the majority of cases, the severity of liver disease could lead to refractory medical conditions, in some cases requiring liver transplantation. The aim of this manuscript is to provide an updated overview of the current evidence regarding the diagnosis and treatment of HHT liver involvement and liver-related complications.

Keywords:
Hepatic vascular malformationsHereditary hemorrhagic teleangiectasiaLiverRendu-Osler-Weber syndrome

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