Pneumothorax secondary to Birt-Hogg-Dubé syndrome treated with the total pleural covering technique

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  • Kitazawa Shinsuke
    Department of General Thoracic Surgery, Tsukuba University Hospital
  • Kobayashi Naohiro
    Department of General Thoracic Surgery, Tsukuba University Hospital
  • Araki Kentaro
    Department of General Thoracic Surgery, Tsukuba University Hospital
  • Kikuchi Shinji
    Department of General Thoracic Surgery, Tsukuba University Hospital
  • Goto Yukinobu
    Department of General Thoracic Surgery, Tsukuba University Hospital
  • Sato Yukio
    Department of General Thoracic Surgery, Tsukuba University Hospital

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Other Title
  • 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

Abstract

<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>

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