Pregnancy in patient with hereditary hemorrhagic telangietasia (osler-weber-rendu disease) and pulmonary arteriovenous malformations: case report

Abstract

Background: Hereditary hemorrhagic telangiectasia, also known as Osler-Weber-Rendu disease is autosomal dominant disorder that affects blood vessels throughout the body (causing vascular dysplasia) and results in a tendency for bleeding. It manifested by mucocutaneous telangiectases and arteriovenous malformations in different organs, including the lungs (pulmonary arteriovenous malformations-PAVM). During the pregnancy due to the hormonal and hemodynamic changes bleeding and pulmonary symptoms become more severe, sometimes leading to lifetreating complications. Case presentation:We report the case of a woman who developed severe dyspnoe, cough, haemoptysis, nose bleedings during 34-week of pregnancy. Diagnosis of hereditary hemorrhagic telangictasia (Osler-Weber-Rendu disease) was established. The lung symptoms were regard to pulmonary arteriovenous malformations. Tests revealed signs of disseminated intravascular coagulation (DIC), acquired von Willebrand syndrome (aVWs). She was successful delivered by cesarean section a then underwent a surgical treatment of pulmonary arteriovenous malformations.