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[马凡综合征合并脊柱或心血管疾病患者漏斗胸的外科治疗]

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

作者信息

Tanaka Yusuke, Matsumoto Isao, Saito Daisuke, Yoshida Shuhei, Takata Munehisa, Tamura Masaya, Takemura Hirofumi

机构信息

Department of General, Thoracic and Cardiovascular Surgery, Kanazawa University, Kanazawa, Japan.

出版信息

Kyobu Geka. 2020 Mar;73(3):163-168.

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.

摘要

患有脊柱侧弯或心血管疾病的马凡综合征患者通常需要手术治疗。如果这些患者同时患有漏斗胸,在心血管手术期间进行脊柱畸形矫正或胸壁闭合手术可能会因胸廓变窄导致胸腔器官受压,进而引起血流动力学和呼吸功能障碍。心血管并发症是马凡综合征患者的预后因素,在这些情况下通常需要进行手术治疗。在我们涉及4例患者的病例系列中,2例患有漏斗胸和脊柱侧弯的患者先接受了努氏手术,6个月后成功进行了安全的脊柱矫正手术。1例合并瓦氏窦扩张的患者也接受了努氏手术,并在心血管手术前行开胸手术。另1例患有漏斗胸且心脏状况急性恶化的患者,采用AO钢板进行胸骨抬高并同时进行心血管手术。对于患有漏斗胸并伴有脊柱侧弯或心血管疾病的马凡综合征患者,有必要根据病情考虑同时修复或分期修复。

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