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

ABSTRACT

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.