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Osler-Weber-Rendu 综合征相关动静脉畸形:放射科医师须知

Arteriovenous Malformations in the Setting of Osler-Weber-Rendu: What the Radiologist Needs to Know.

机构信息

Department of Radiology, Dartmouth-Hitchcock Medical Center, Lebanon, NH, USA.

Department of Radiology, Dartmouth-Hitchcock Medical Center, Lebanon, NH, USA.

出版信息

Curr Probl Diagn Radiol. 2022 May-Jun;51(3):375-391. doi: 10.1067/j.cpradiol.2021.03.009. Epub 2021 Mar 11.

Abstract

Osler-Weber-Rendu, also known as Hereditary Hemorrhagic Telangiectasia, is an autosomal dominant disease with phenotypic manifestations that include pulmonary, cerebrospinal, hepatic, and other visceral arteriovenous malformations (AVMs). Pulmonary AVMs can result in hypoxemia, hemoptysis, or stroke due to paradoxical embolism. The mainstay of treatment is transcatheter embolization. Central nervous system and abdominal visceral AVMs contribute to morbidity of the disease. Radiologists should be familiar with the imaging manifestations and treatment algorithm of AVMs in Osler-Weber-Rendu to effectively guide patient care.

摘要

Osler-Weber-Rendu,也称为遗传性出血性毛细血管扩张症,是一种常染色体显性遗传疾病,其表型表现包括肺、脑脊髓、肝和其他内脏动静脉畸形(AVM)。肺 AVM 可导致低氧血症、咯血或因反常栓塞引起的中风。治疗的主要方法是经导管栓塞。中枢神经系统和腹部内脏 AVM 导致疾病的发病率。放射科医生应熟悉 Osler-Weber-Rendu 中 AVM 的影像学表现和治疗算法,以有效指导患者的治疗。

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