A Case Report on Osler -Weber Rendu Disease

Abstract

Osler weber rendu disease, also known as hereditary hemorrhagic telengiectasia is a rare autosomal dominant genetic disorder that leads to abnormal blood vessel formation in skin, mucous membrane and in organs such as lungs, liver and brain. A 68 year old postmenopausal lady was admitted to our hospital following multiple episodes of hematemesis and malena for past 5 days. Upper GI endoscopy revealed multiple telengiectasia with active spurting, gastric polyp. Patient was treated with 1 unit of PRBC transfusion for anemia, Inj.Tranexamic acid 500 mg IV tid, Inj. Ondanseteron 4 mg IV for hematemesis. Tab. Thalidomide 100 mg hs have been given for treating hereditary hemorrhagic telengiectasia. The treatment is only palliative, with no consensus on the best treatment option. It is essential to promote control of the disease as long as possible.