Marfan症候群に合併した漏斗胸とAnnulo-aortic ectasiaの一期的手術

One stage operation for annulo-aortic ectasia and pectus excavatum caused by Marfan's syndrome

　患者は32歳，男性。主訴は息切れの増強と前胸部痛。家族歴は母親と兄が心疾患により死亡し，妹も腹部大動脈瘤により人工血管置換術を行っている。身長は197cm，体重は62kgと長身痩躯で，高度の胸郭変形と関節過伸展，クモ状指，水晶体の亜脱臼などを認め，Marfan様の体格を呈していた。聴診上，大動脈弁領域にto and fro murmurを聴取し，大動脈造影にIV度のARを認めた。手術は胸部正中皮膚切開を行い，胸骨を第2肋間にて横断，胸骨—肋骨複合体を摘出したのち胸骨翻転術を行った。大動脈瘤は直径10cmで洋梨状の拡大を示し，27mm，Björk-Shiley弁付きcomposite graftを用い，定型的なBentall手術を行った。一期的手術により良好な手術野が得られること，術後安定した循環，呼吸機能となることなどの点において，今後も奨められるべき術式と考えられる。

One stage operation was successfully performed on a 32-year-old man with annulo-aortic ectasia (AAE) and pectus excavatum caused by Marfan's syndrome. In the Bentall's operation for AAE, median sternotomy is usually carried out. In this case it was difficult to obtain a favorable viewing field due to an extremely deformed sternum and significant displacement of the heart into the left thoracic cavity. By removing the sternum en bloc, as a cost-sterno complex, the surgery was performed safely with an excellent operative field. In this surgery combined with the sternal turnover, the amount of intra and post operative bleeding was the same as occurs in other open heart surgeries.