Most BHD patients develop pulmonary cysts and although only 30-35% will suffer a pneumothorax the recurrence rate is very high (Toro et al., 2008). The standard treatment for recurrent pneumothorax is pleurodesis, sometimes accompanied with pleurectomy, which attaches the lung surface to the chest wall thereby reducing the risk of further air leaks. An alternative treatment pioneered by several independent groups of researchers and clinicians in Japan is pleural covering which reinforces the surface of the lung without attachment to the chest wall.
Every year in Japan over 12,000 patients with cystic lung diseases undergo surgery – mostly related to recurrent or intractable pneumothoraces. An average post-operative recurrence rate of 4-20% highlighted the need for alternative treatments. Pleurodesis is effective and widely used, but permanently attaching the lungs to the chest wall can reduce respiratory function and make subsequent thoracic surgery more difficult, so alternative non-adhesive treatments were of interest to clinicians and patients (Kurihara et al., 2010).
The pleural covering technique uses a bio-absorbable mesh – either regenerative oxidized cellulose (ROC) mesh or polyglycolic acid (PGA) felt – placed over the surface of the lung and attached using fibrin glue (Ueda et al., 2009, Kurihara et al., 2010). The mesh is absorbed into the pleura reinforcing the remaining lung tissue and unruptured cysts, and sealing air leaks. ROC membranes are less likely to adhere to the chest wall and are associated with fewer post-operative recurrences, compared to PGA felt (Uramoto & Tanaka, 2014).
The covering procedure was originally only used to reinforce excision and staple lines following bulbectomies, and found to reduce bullae regrowth at the staple lines and post-operative recurrence (Sakamoto et al., 2004). More extensive lung covering in LAM patients, to reinforce existing cysts, was found to prevent recurrent pneumothoraces without adhesion to the chest wall or reduction in lung function (Kurihara et al., 2008, Noda et al., 2010).
Data presented by Professor Kurihara at the Fifth BHD Symposium in 2013 (5th BHD Symposium Abstract 14) highlighted the efficacy of pleural covering in BHD patients – 45/46 patients treated with bilateral pleural covering had no post-operative pneumothoraces (<56-month follow-up). Additionally Okada et al., (2015) and Ebana et al., (2015) published pleural covering case studies detailing the treatment of four patients who have suffered no further pneumothoraces (<32 month follow-up). Removing all pulmonary cysts in BHD patients would significantly reduce lung function. Therefore instead the ruptured and thin-walled bulging cysts can be removed before reinforcing both the excision sites and remaining unruptured cysts (Okada et al., 2015).
Pleural covering has also been successful in a range of other cystic lung diseases including bronchiolitis obliterans, pulmonary eosinophilic granulomas (Noda et al., 2011), and AAT-deficiency (Kusu et al., 2012). Although currently not a common practice outside Japan, as more long-term case studies are reported and the efficacy of the treatment becomes clearer pleural covering could become a viable alternative to pleurodesis worldwide for patients suffering from recurrent pneumothorax. In particular the lack of adhesion to the chest wall would be advantageous in patients who are likely to require subsequent surgeries to the lungs, heart or oesophagus.
- Ebana H, Otsuji M, Mizobuchi T, Kurihara M, Takahashi K, Seyama K (2015). Pleural Covering Application for Recurrent Pneumothorax in a Patient with Birt-Hogg-Dubé Syndrome. Ann Thorac Cardiovasc Surg. Sep 11. PMID: 26370712.
- Kurihara M, Seyama K, Kumasaka T (2009). Preventing LAM Patients from Recurrent Pneumothorax – An Innovative Surgical Method without Adhesion: Total Pleural Covering Technique (TPC). ATS International Conference 2009 Abstract.
- Kurihara M, Kataoka H, Ishikawa A, Endo R (2010). Latest treatments for spontaneous pneumothorax. Gen Thorac Cardiovasc Surg. Mar;58(3):113-9. PMID: 20349299.
- Kusu T, Nakagiri T, Minami M, Shintani Y, Kadota Y, Inoue M, Sawabata N, Okumura M (2012). Null allele alpha-1 antitrypsin deficiency: case report of the total pleural covering technique for disease-associated pneumothorax. Gen Thorac Cardiovasc Surg. Jul;60(7):452-5. PMID: 22544422.
- Noda M, Okada Y, Maeda S, Sado T, Sakurada A, Hoshikawa Y, Endo C, Kondo T (2010). An experience with the modified total pleural covering technique in a patient with bilateral intractable pneumothorax secondary to lymphangioleiomyomatosis. Ann Thorac Cardiovasc Surg. Dec;16(6):439-41. PMID: 21263428.
- Noda M, Okada Y, Maeda S, Sado T, Sakurada A, Hoshikawa Y, Endo C, Kondo T (2011). A total pleural covering technique in patients with intractable bilateral secondary spontaneous pneumothorax: Report of five cases. Surg Today. Oct;41(10):1414-7. PMID: 21922367.
- Okada A, Hirono T, Watanabe T, Hasegawa G, Tanaka R, Furuya M (2015). Partial pleural covering for intractable pneumothorax in patients with Birt-Hogg-Dubé Syndrome. Clin Respir J. Jun 15. PMID: 26073198.
- Sakamoto K, Takei H, Nishii T, Maehara T, Omori T, Tajiri M, Imada T, Takanashi Y (2004). Staple line coverage with absorbable mesh after thoracoscopic bullectomy for spontaneous pneumothorax. Surg Endosc. Mar;18(3):478-81. PMID: 14752657.
- Toro JR, Wei MH, Glenn GM, Weinreich M, Toure O, Vocke C, Turner M, Choyke P, Merino MJ, Pinto PA, Steinberg SM, Schmidt LS, Linehan WM (2008). BHD mutations, clinical and molecular genetic investigations of Birt-Hogg-Dubé syndrome: a new series of 50 families and a review of published reports. J Med Genet. 208 Jun;45(6):321-31. Review. PMID: 18234728.
- Ueda S, Isogami K, Kobayashi S (2009). [Uncomplicated covering technique for preventing the recurrence in the thoracoscopic surgery for pneumothorax]. Kyobu Geka. 2009 May;62(5):381-4. Japanese. PMID: 19425378.
- Uramoto H, Tanaka F (2014). What is an appropriate material to use with a covering technique to prevent the recurrence of spontaneous pneumothorax? J Cardiothorac Surg. Apr 29;9:74. PMID: 24775221.