Treatment of Multiple Pulmonary Arteriovenous Fistulas with Therapeutic Embolization in Osler-Rendu-Weber Syndrome

Osler-Rendu-Weber 증후군 환자에서 색전요법을 이용한 다발성 폐동정맥루 치험 1예

Hereditary hemorrhagic telangiectasia(Osler-Rendu-Weber Syndrome) is characterized by telangiectasia of the skin and mucous membranes and intermittent bleeding from vascular abnormalities. About 20% of patients with this syndrome have pulmonary arteriovenous fistulas. Pulmonary arteriovenous fistula is uncommon malformation which has an abnormal connection between the pulmonary capillary bed, in which venous blood in the pulmonary artery is shunted through the fistula into the pulmonary vein without exposure to alveolar oxygen and result in unoxygenated, desaturated systemic arterial blood, polycythemia, cyanosis and clubbing. Death often results from cerebral abscess and rupture of the malformation with massive hemorrhage. Therapeutic intervention is recommended for all symptomatic patients because of the risk of those serious complications. Treatment options include surgery and transcatheter obliteration with steel coils or detachable balloons. Therapeutic embolization has the advantages that multiple bilateral pulmonary arteriovenous fistulas can be occluded and also that the procedure can be repeated if necessary. Recently we experienced a case of the multiple bilateral pulmonary arteriovenous fistulas associated with telangiectatic change of hepatic artery and multiple angiodysplasia on the gastric mucosa in 41 years old female patient who had mild dyspnea of exertion(NYHA class II). clubbing finger, severe iron deficiency anemia. She was treated with embolization technique using steel coils and iron replacement. After the therapeutic embolization. significant improvement of dyspnea of exertion with disappearance of multiple pulmonary nodule on follow-up simple chest x-ray was noted. During the subsequent six months follow-up period, she had the improvement of symptoms and iron deficiency anemia.

저자들은 최근 가족력은 없지만 폐동정맥루와 간동맥혈관 조영술 및 위점막에서 관찰된 혈관기형을 동반한 Osler-Rendu-Weber 증후군 1예에서 폐동정맥루에 대해 금속코일을 이용한 색전요법을 시행하여 특별한 합병증 없이 증상의 개선을 경험하였기에 문헌 고찰과 함께 보고하는 바이다.