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

Abstract

BackgroundBirt-Hogg-Dubé 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.ResultsThis 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).ConclusionsTPC might be an effective option for surgical treatment of intractable pneumothorax in patients with BHDS.Electronic supplementary materialThe online version of this article (10.1186/s13023-018-0790-x) contains supplementary material, which is available to authorized users.

Pathology of Birt–Hogg–Dubé syndrome: A special reference of pulmonary manifestations in a Japanese population with a comprehensive analysis and review

“…Pleurodesis appears to reduce the risk of recurrent pneumothorax . We and another Japanese group reported that bioabsorbable pleural coverings successfully protected patients from intractable pneumothoraces . The latter method may enable patients to safely undergo VATS years later if they develop other lung diseases.…”

Birt–Hogg–Dubé syndrome

“…In one retrospective series, the risk of relapse was significantly reduced from >60% with conservative treatment to around 30% after pleurodesis [67]. In another retrospective series reporting the efficacy of surgical pleurodesis with a cellulose mesh covering the visceral pleura, total pleural covering was significantly more effective than lower pleural covering, with 0% versus 42% recurrence rate at 7.5 years [130]. It is important to inform patients about the risk and symptoms of pneumothorax, and to recommend medical assessment in case of new respiratory symptoms such as dyspnoea or chest pain.…”

Video Assisted Thoracoscopic Surgery (VATS) of Lymphangiomyomatosis (LAM) with Multiple Pulmonary Bullae in a Woman: A Case Report and Literature Review

“…Therefore, we searched and screened 8 articles on the treatment of LAM with thoracoscopic surgery. We meta-analyzed the pneumothorax recurrence rate of LAM in 8 studies [9,[11][12][13][14][15][16] (Table 2).…”

“…We conducted a meta-analysis of 8 case reports and experimental studies [9,[11][12][13][14][15][16] showed that VATS treatment of pneumothorax, the recurrence of pneumothorax was reduced compared with conservative treatment (non-surgical treatment control group) [67/174, 95% CI:-0.44[-0.52,-0.36], P<0.00001], statistics also have research meaning. Among them, I 2 >50% (I 2 =81%), we conducted statistical bias analysis.…”