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

Abstract Background Birt-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. 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

Additional file 3: of A total pleural covering of absorbable cellulose mesh prevents pneumothorax recurrence in patients with Birt-Hogg-Dubé syndrome

Video S1. Total Pleural Covering (TPC) procedure of the right lungs in a Birt-Hogg-Dubé syndrome patient: With approximately 50% inflation of the lung, TPC using sheets of oxidized regenerated cellulose (ORC) mesh encompassed the entire surface of the lung. In detail, we covered the 1) upper lobe, 2) basal area in the lower lobe, 3) posterior mediastinal lung surface, 4) anterior mediastinal lung surfaces, 5) lateral lung surface, and 6) interlobar lung surface. (MPG 50006 kb

Additional file 2: of A total pleural covering of absorbable cellulose mesh prevents pneumothorax recurrence in patients with Birt-Hogg-DubĂŠ syndrome

Figure S2. Technical procedure for total pleural covering (TPC) of right lungs: Schemata depicting systematic covering of an entire visceral pleura by oxidized regenerated cellulose (ORC) mesh, which encloses the surface of the 1) upper lobe, 2) basal area in the lower lobe, 3) posterior mediastinal side of the upper and lower lobes, 4) anterior mediastinal side of the upper and middle lobes, 5) lateral side of the middle and lower lobes, and 6) interlobar surface of the lungs. (TIF 1440 kb

A Total Pleural Covering for Lymphangioleiomyomatosis Prevents Pneumothorax Recurrence

<div><p>Background</p><p>Spontaneous pneumothorax is a major and frequently recurrent complication of lymphangioleiomyomatosis (LAM). Despite the customary use of pleurodesis to manage pnenumothorax, the recurrence rate remains high, and accompanying pleural adhesions cause serious bleeding during subsequent lung transplantation. Therefore, we have developed a technique of total pleural covering (TPC) for LAM to wrap the entire visceral pleura with sheets of oxidized regenerated cellulose (ORC) mesh, thereby reinforcing the affected visceral pleura and preventing recurrence.</p><p>Methods</p><p>Since January 2003, TPC has been applied during video-assisted thoracoscopic surgery for the treatment of LAM. The medical records of LAM patients who had TPC since that time and until August 2014 are reviewed.</p><p>Results</p><p>TPC was performed in 43 LAM patients (54 hemithoraces), 11 of whom required TPC bilaterally. Pneumothorax recurred in 14 hemithoraces (25.9%) from 11 patients (25.6%) after TPC. Kaplan-Meier estimates of recurrence-free hemithorax were 80.8% at 2.5 years, 71.7% at 5 years, 71.7% at 7.5 years, and 61.4% at 9 years. The recurrence-free probability was significantly better when 10 or more sheets of ORC mesh were utilized for TPC (P = 0.0018). TPC significantly reduced the frequency of pneumothorax: 0.544 ± 0.606 episode/month (mean ± SD) before TPC vs. 0.008 ± 0.019 after TPC (P<0.0001). Grade IIIa postoperative complications were found in 13 TPC surgeries (24.1%).</p><p>Conclusions</p><p>TPC successfully prevented the recurrence of pneumothorax in LAM, was minimally invasive and rarely caused restrictive ventilatory impairment.</p></div

Characteristics of study population (n = 43).

<p>Characteristics of study population (n = 43).</p

Comparison of the frequency of pneumothorax episodes before and after TPC.

<p>A. The number of pneumothorax episodes was divided by the observation period (the months from the first pneumothorax episode to TPC or those after TPC). The frequency of pneumothorax (episodes/month) was significantly reduced after TPC (P<0.0001). B. A Poisson regression model was used to compare the number of pneumothorax episodes before TPC (open circle) and after TPC (closed circle) with log [observation period (years)] as offset and subjects (intercept) as random effect (P<0.0001). The estimated regression equation of the number of pneumothorax before TPC is e^{1.025 + 0.111×years} before TPC (dotted line) and e^{-2.606 + 0.337×years} after TPC (solid line).</p

Results of lung function tests after TPC.

<p>Results of lung function tests after TPC.</p

Schematic presentation of TPC workflow.

<p>TPC procedure for a LAM lung consists of 1) covering the lung’s entire visceral pleura with sheets of ORC mesh and 2) followed by spreading drops of fibrin glue. The entire procedure is performed under VATS.</p

Kaplan-Meier estimate of the probability of recurrence-free hemithorax after TPC.

<p>A. All hemithoraces that had TPC (n = 54). The probability of recurrence-free hemithorax after TPC is 80.8% at 2.5 years, 71.7% at 5 years, 71.7% at 7.5 years, and 61.4% at 9 years. Dotted lines indicate the range of 95% confidence interval. B. Comparison of pneumothorax recurrence among hemithoraces where 10 or more ORC sheets were applied for TPC (n = 39) and those where fewer than 10 ORC sheets were used (n = 15) (P = 0.0018). The probability of no recurrence of pneumothorax after TPC was analyzed by Cox regression analysis adjusted for patient’s age and surgical skill by year (era) when TPC was implemented (3 categorized era: 2003–2007, 2008–2011, and 2012–2014).</p