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Introduction: Osler-Rendu-Weber syndrome or hereditary hemorrhagic telangiectasia affects between 1/5000 and 1/8000 people. It is characterized by presence of recurrent epistaxis, mucocutaneous telangiectasia and visceral arteriovenous malformations. It is a genetic disease with autosomal dominant transmission inducing an endothelial cells hyper-proliferation., Case Report: A 68-year-old women with Osler-Rendu-Weber syndrome was referred for management of general impairment with confusional syndrome and hyperthermia. Various examinations have allowed us to conclude at diagnosis of brain abscess with ventriculitis probably favored by right-left shunt secondary to pulmonary arteriovenous malformations. Evolution was favorable after antibiotic treatment and endovascular embolization., Conclusion: In case of brain abscess without obvious promoting factor, don't forget to looking for a right-left shunt providing septic or aseptic emboli. Furthermore, diagnosis of Rendu-Osler-Weber syndrome should be considered presence of telangiectasias and/or epistaxis., (Copyright © 2020. Published by Elsevier Masson SAS.)

Introduction: Hereditary hemorrhagic telangiectasia (HHT), is a rare, hereditary vascular dysplasia, characterized by recurrent epistaxis, mucocutaneous telangiectasias and visceral arteriovenous malformations. The vascular endothelial growth factor VEGF seems to play a crucial role in the pathogenesis of this disease. Recently bevacizumab, a humanized monoclonal VEGF inhibitor, has shown promise in treating patients with HHT., Case Report: A 66-year-old man, having HHT since the age of 30 years with recurrent epistaxis related to telangiectasia at the nasal septum and chronic iron deficiency anemia requiring frequent blood transfusions with iron infusions. The assessment of his disease showed septal perforation, telangiectasis in the proximal jejunum and terminal ileum, and pulmonary arteriovenous malformations. There was no improvement, despite iron infusions, repeated blood transfusions and cauterization. The patient was treated with bevacizumab at a dose of 5mg/kg/infusion every 2 weeks and was given 6 cycles. Bevacizumab, was effective without side effects., Discussion: It has been hypothesized that HHT is related to an imbalanced state between antiangiogenic factors and proangiogenic factors. Mutations of 3 genes are actually identified in HHT: ENG, ACVRL1, MADH4. The management of patients with HHT currently based on screening for visceral arteriovenous malformations and symptomatic measures are often disappointing. However, the angiogenic nature of this disease suggests an interesting therapy by using angiogenesis inhibitor. Therefore, bevacizumab was introduced as a potential therapy for HHT. Some clinical cases or small series report the efficacy of bevacizumab, in HHT with recurrent epistaxis, refractory iron deficiency anemia, gastrointestinal bleeding and also in liver vascular malformations with high cardiac output failure., Conclusion: The use of modulators of angiogenesis such as bevacizumab is a possible therapeutic target in HHT., (Copyright © 2014 Société nationale française de médecine interne (SNFMI). Published by Elsevier SAS. All rights reserved.)

Hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu syndrome) is a development disorder of the vasculature characterized by telangiectases and arteriovenous malformations in specific locations. Among monogenic disorders, it is one of the most common, though affected individuals are widely underdiagnosed. The most common features of this disorder, nosebleeds, and telangiectases on the lips, hands, and oral mucosa are often quite subtle. Mutations in at least five genes may result in hereditary hemorrhagic telangiectasia, but mutations in two genes (ENG and ACVRL1/ALK1) account for approximately 85% of cases. Optimal management requires understanding the specific clinical patterns of these vascular malformations, especially their locations and timing during life. Therapeutic modulation of angiogenesis may be an effective therapy., (Copyright © 2013 Société nationale française de médecine interne (SNFMI). Published by Elsevier SAS. All rights reserved.)