Marfan症候群合併自然気胸手術症例の検討

Evaluation of Patients of Spontaneous Pneumothorax Associated with Marfan Syndrome who Underwent Thoracoscopic Surgery

要旨　Marfan症候群は自然気胸を合併する頻度が高いとされている．今回，Marfan症候群合併自然気胸に対して外科的治療を施行した4例(5側)について臨床的検討を行った．年齢28.6±16.4歳，性別は男性・女性各2例(50.0%)で，異時性両側気胸1例であった．全例が心血管疾患を有しており，BMI 18.5以下の痩せであった．再発例が1側(20.0%)で，初発が4側(80.0%)であった．手術適応は，気漏持続3側(60.0%)，再発気胸1側(20.0%)，ブラの存在1例(20.0%)であった．術式は全例が胸腔鏡下ブラ切除のみであった．重篤な術後合併症はなく，平均観察期間12.9カ月で再発例はなかった．Marfan症候群は心血管合併症を有することが多いが，術前に評価を行い，安全に手術を遂行することができた．Marfan症候群患者の自然気胸に対する胸腔鏡下ブラ切除術は，術後合併症，再発の観点から有用な手技と考えられた．

　Patients with Marfan syndrome sometimes experience spontaneous pneumothorax. Four patients with spontaneous pneumothorax, including one patient with bilateral lesion-associated Marfan syndrome who underwent surgery were evaluated. A past history of pneumothorax was seen in 1 patient. Age of the patients was 28.6±16.4 years old and they consisted of two men and two women. All patients had cardiovascular disease and body-mass index was less than 18.5. Indication for operation in five lesions was prolonged air leak in 3 lesions, recurrent pneumothorax in 1 lesion and bullae in computed tomography in 1 lesion. The operative procedure for pneumothorax was thoracoscopic bullectomy without additional treatment such as covering with absorbable artificial materials in any of the patients. Operation time, intraoperative bleeding and postoperative hospital stay was 88.4±24.5 minutes, 4.00±8.94 grams and 6.60±2.07 days, respectively. Severe postoperative complications were not seen. As for recurrence, the mean duration of observation was 12.9 months and no recurrent pneumothorax had occurred. Although patients with Marfan syndrome often complicate cardiovascular disease, these cases showed they can undergone surgery for spontaneous pneumothorax without severe perioperative complications after evaluation for cardiovascular disease. Thoracoscopic surgery for spontaneous pneumothorax associated with Marfan syndrome is considered a useful method in view of perioperative complication and postoperative recurrence.