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Review
. 2023 May 27;15(5):675-687.
doi: 10.4254/wjh.v15.i5.675.

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

Luca Ielasi  1   2 , Matteo Tonnini  1   3 , Fabio Piscaglia  1   3 , Ilaria Serio  3
Affiliations
Review

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

Luca Ielasi et al. World J Hepatol. .

Abstract

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 malformations; Hereditary hemorrhagic teleangiectasia; Liver; Rendu-Osler-Weber syndrome.

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Conflict of interest statement

Conflict-of-interest statement: All authors declare no conflict of interest.

Figures

Figure 1
Figure 1
Ultrasound findings in hereditary hemorrhagic teleangiectasia. A: Hepatic artery dilation; B: Peripheral hepatic hypervascularization; C: Pulsatile flow in right portal branch related to arteriovenous malformation.
Figure 2
Figure 2
Multiple arteriovenous malformations and enlarged hepatic artery in contrast-enhanced computed tomography scan.
See this image and copyright information in PMC

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