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Hereditary hemorrhagic telangiectasia of liver: Pathophysiology with role of radiology in diagnosis and treatmentFinancial support and sponsorship Nil.
Hereditary hemorrhagic telangiectasia (HHT) or Osler–Weber–Rendu syndrome is a rare condition which can result in significant systemic and hepatobiliary abnormalities. Liver involvement in HHT consists primarily of the consequence of various intrahepatic shunts. Even though these vascular shunts are present in the majority of patients with HHT, symptoms occur only in minority with clear predilection to female gender. The symptoms and imaging findings of liver vascular malformations can be easily overlooked or misdiagnosed which can result in delay in treatment or potentially harmful vascular interventions. In this case report, we discuss the pathophysiology of HHT in liver involvement, role of imaging in diagnosis, and the possible role of interventional radiologist in the treatment.
KeywordsEmbolization for shunt reduction - hereditary hemorrhagic telangiectasia - intrahepatic shunts - liver vascular malformation
Received: 03 September 2019
Accepted: 11 February 2019
19 July 2021 (online)
© 2020. Indian Radiological Association. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial-License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/).
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