Anesthetic management of a patient with Osler-Weber-Randu disease]

Osler-Weber-Randu disease (Osler disease) is an autosomal dominant disease, sometimes known as hereditary hemorrhagic telangiectasia with its family history. It is not a popular disease and approximately seventy families are known as inheritance lineage in Japan. We experienced anesthetic management of a 49-yr-old woman with Osler disease. She was diagnosed to have the brain abscess following fever and clouding of consciousness for several days and was scheduled for the removal of the abscess. A chest X-ray revealed pulmonary arteriovenous fistula in the right middle lung field. The patient had had life threatening frequent massive bleeding from her nose and/or stomach for the past several years. Her brother and children also had the same symptoms. In addition to Osler disease, the patient had the prolonged coagulation time that was supposed to the due to chronic liver disease. Therefore, fresh frozen plasma (5 units) and platelet concentrates (10 units) were infused during the surgery. Intraoperative blood loss was about 700 grams. We had no difficulties in management of bleeding and respiratory controls during anesthesia.