A Total Pleural Covering for Lymphangioleiomyomatosis Prevents Pneumothorax Recurrence

Abstract

BackgroundSpontaneous 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.MethodsSince 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.ResultsTPC 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%).ConclusionsTPC successfully prevented the recurrence of pneumothorax in LAM, was minimally invasive and rarely caused restrictive ventilatory impairment.

Novel clinical scoring system to identify patients with pneumothorax with suspicion for Birt–Hogg–Dubé syndrome

“…Based on these presentations, the conventional treatment approach in which all subpleural cysts and bullae are resected or subjected to pleuredosis is not feasible for the management of PTX in BHDS. Therefore, it is important for pulmonologists and thoracic surgeons to be aware of this rare cause of PTX and to consider a new therapeutic method such as the total pleural covering technique …”

Spontaneous pneumothorax in diffuse cystic lung diseases

“…Total Pleural Covering (TPC), which involves wrapping the visceral pleura in a bioabsorbable mesh, has shown promise in preventing pneumothorax without severe impairment of lung function or formation of severe pleural adhesions (96). In a recent study involving 43 patients with LAM, TPC resulted in a pneumothorax recurrence rate (26%) that was comparable to traditional pleurodesis (15, 96). Successful use of TPC has also been reported in PLCH, BHD, bronchiolitis obliterans, and Ehlers-Danlos Syndrome (97, 98).…”

Total pleural coverage followed by lung transplantation in patient with lymphangioleiomyomatosis

“…Since recurrent pneumothorax is one of the most common complications seen in LAM patients, chemical or mechanical were thickened, and minimal adhesion of the visceral to the parietal pleura was evident. e Elastica-Masson stain showed the thickened visceral pleura above the natural visceral pleura (arrow) pleurodesis has been recommended to prevent recurrence [3,4]. However, after pleurodesis, it was difficult to dissect the pleura and control intraoperative bleeding, because of severe adhesion.…”

“…Although animal experiments have been performed to check adhesion or visceral pleural thickness after pleurodesis [3], no report on a patient treated via TPC who later underwent lung transplantation has appeared. As shown here, TPC might be one of the good tools for prevention of recurred pneumothorax, making the thickened visceral pleura useful to describe our patient with LAM who underwent TPC followed by lung transplantation; examination of the extracted lung showed that our treatment reinforced the visceral pleura in the absence of dense adhesions.…”