脊椎・心血管疾患を合併するMarfan症候群患者における漏斗胸の手術

Surgical Treatment of Pectus Excavatum in Patients with Marfan Syndrome Associated with Vertebral or Cardiovascular Disease

Marfan症候群では，脊椎変形疾患や心血管疾患などの合併症に対して手術加療が必要になることが多い．漏斗胸を伴う場合は，脊椎矯正による胸郭の狭小化や心大血管手術時の閉胸操作による心肺の圧迫などが起因となって，術中・術後に血行動態の悪化や呼吸障害が起こる可能性があることに注意を要する．われわれは，漏斗胸を併発した4例のMarfan症候群における漏斗胸ならびに脊椎・心血管合併症に対する手術経験について検討を行ったので，文献的考察を加えて報告する．

Surgical treatment is often required in patients with Marfan syndrome presenting with scoliosis or cardioaortic disease. If these patients present with pectus excavatum, surgery for spinal deformity correction or chest wall closure during cardioaortic surgery can cause thoracic organ compression secondary to narrowing of the thorax resulting in hemodynamic and respiratory compromise. Cardiovascular complications serve as prognostic factors in patients with Marfan syndrome, and surgical treatment is often required in these cases. In our case series involving 4 patients, 2 patients with pectus excavatum and scoliosis underwent a Nuss procedure followed by safe and successful spinal correction surgery 6 months later. A Nuss procedure was also performed in a patient complicated with sinus of Valsalva dilatation, and thoracotomy was performed before cardioaortic surgery. Another patient with a complication of pectus excavatum and acute deterioration of cardiac status underwent concomitant repair with sternal elevation using an AO plate and cardioaortic surgery. In patients with Marfan syndrome presenting with pectus excavatum complicated by scoliosis or cardioaortic disease, it is necessary to consider concomitant or staged repair depending on the disease condition.