A total pleural covering of absorbable cellulose mesh prevents pneumothorax recurrence in patients with Birt-Hogg-Dube syndrome

作者：Mizobuchi Teruaki; Kurihara Masatoshi; Ebana Hiroki; Yamanaka Sumitaka; Kataoka Hideyuki; Okamoto Shouichi; Kobayashi Etsuko; Kumasaka Toshio; Seyama Kuniaki

来源：Orphanet Journal of Rare Diseases, 2018, 13(1): 78.

DOI：10.1186/s13023-018-0790-x

• Birt-Hogg-Dube syndrome
• Pneumothorax
• Pleural covering
• Multiple cystic lung disease

Background: Birt-Hogg-Dube syndrome (BHDS) is a recently recognized inherited multiple cystic lung disease causing recurrent pneumothoraces. Similarly to the lesions in patients with lymphangioleiomyomatosis (LAM), the pulmonary cysts are innumerable and widely dispersed and cannot all be removed. We recently described a total pleural covering (TPC) that covers the entire visceral pleura with oxidized regenerated cellulose (ORC) mesh. TPC successfully prevented the recurrence of pneumothorax in LAM patients. The purpose of this study was to evaluate the effect of an ORC pleural covering on pneumothorax recurrence in BHDS patients.
Results: This retrospective study enrolled a total of 81 pneumothorax patients with the diagnosis of BHDS who underwent 90 covering surgeries from January 2010 to August 2017 at Tamagawa Hospital. During the first half of the study period, a lower pleural covering (LPC) which covered the affected area with ORC mesh was mainly used to treat 38 pneumothoraces. During the second half of the study period, TPC was primarily performed for 52 pneumothoraces. All the thoracoscopic surgeries were successfully performed without serious complications (>= Clavien-Dindo grade III). The median follow-up periods after LPC/TPC were 66/34 months, respectively.
Pneumothorax recurrence rates after LPC at 2.5/5/7.5 years postoperatively were 5.4/12/42%, respectively; none of the patients who had underwent TPC developed postoperative pneumothorax recurrence (P= 0.032).
Conclusions: TPC might be an effective option for surgical treatment of intractable pneumothorax in patients with BHDS.

• 出版日期2018-5-15
• 单位河北医科大学