Patients with hereditary haemorrhagic telangiectasia (HHT) are known to suffer from cerebral arteriovenous malformations (CAVMs). In this review, we explore existing literature for bleeding risk, interventional therapy and neuroradiological features in HHT-related CAVMs. Studies estimate the annual intracerebral haemorrhage rate of CAVMs in HHT patients to be 0.667-1.014%. The clinician must balance bleeding risk and the non-negligible procedural risks of interventional therapy. We recommend, in agreement with European guidelines, that screening of asymptomatic HHT patients should only be carried out after careful information.

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