Multisystem vascular dysplasia: Osler-Weber-Rendu disease: unraveling the cause of the bleeding

Abstract

Osler-Weber-Rendu disease (OWR), also known as multisystem vascular dysplasia or hereditary hemorrhagic telangiectasia, is a rare autosomal dominant disorder, characterized by the development of vascular dysplasias (VD) such as telangiectasias and arteriovenous malformations of mucocutaneous or visceral location, which show a tendency to bleeding, and can affect any organ or system, with epistaxis being the most frequent initial clinical presentation. Although the definitive diagnosis is based on genetic studies, due to their low availability and high cost, they have led to the use of imaging methods to identify findings that guide clinical suspicion. Three cases are presented with a diagnosis of OWR and imaging findings of hepatic, gastrointestinal and pulmonary DV levels.