Birt-Hogg-Dubé症候群に伴う気胸に対して再発予防のためにTotal Pleural Coveringを施行した1例  [in Japanese] Pneumothorax secondary to Birt-Hogg-Dubé syndrome treated with the total pleural covering technique  [in Japanese]

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<p>Birt-Hogg-Dubé(BHD)症候群は顔面頭頸部皮疹,腎腫瘍,肺囊胞を特徴とする常染色体優性遺伝の疾患である.肺囊胞は両側多発性に存在することが特徴的で,気胸を繰り返すことが知られている.今回,画像所見と家族歴からBHD症候群を疑い,再発予防のためにTotal Pleural Coveringを施行した1例を報告する.症例は68歳,女性.前医で右気胸と診断され,保存的加療で改善しないため手術目的で本院紹介となった.右中下葉優位に両側多発肺囊胞を認め,気胸の家族歴を有することからBHD症候群に伴う続発性気胸を疑った.手術は胸膜表面の囊胞を切除した後にTotal Pleural Coveringを行った.術後に遺伝子検査を行いBHD症候群と診断された.BHD症候群に伴う気胸は多発肺囊胞を背景とし再発率が高い.BHD症候群が疑われた場合には再発予防を含めた手術を検討する必要がある.</p>

<p>We describe a 68-year-old woman with preoperatively suspected BHD syndrome, who underwent the total pleural covering technique to prevent recurrent pneumothorax. She presented with dry cough, and right pneumothorax was diagnosed. Since the lung collapse had not improved over the course of 1 month, surgical pulmonary fistula closure was required. Her daughter also had a history of pneumothorax. Chest CT showed a collapsed right lung and bilateral multiple lung cysts, predominantly in the middle and lower lobes. Owing to the characteristic pulmonary cysts and family history, BHD syndrome was primarily suspected. Although she had no previous episode of pneumothorax, we considered that the possibility of recurrent pneumothorax was high. Therefore, to prevent such recurrence, we performed resection of the pulmonary cysts and additional total pleural covering. Postoperatively, genomic testing confirmed the diagnosis of BHD syndrome. In cases of pneumothorax with BHD syndrome, resection of all the pulmonary cysts is difficult and the possibility of recurrence is high. Therefore, if BHD syndrome is suspected preoperatively, a surgical procedure to reduce the subsequent recurrence of pneumothorax is needed.</p>


  • The Journal of the Japanese Association for Chest Surgery

    The Journal of the Japanese Association for Chest Surgery 30(6), 726-730, 2016

    The Japanese Association for Chest Surgery


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