Treatments

Chest tube thoracostomy

A thoracostomy is a surgical procedure where a hollow plastic tube is inserted between the ribs under local anesthesia to drain air from the chest cavity. The process can take several days. Usually a suction device is used to suck out the air, which speeds up the process. Removing the air pressing on the lung often means the collapsed lung can re-inflate on its own.

Sometimes thoracostomy is combined with another type of treatment, such as pleurodesis.

Pleurodesis and pleurectomy

Pleurodesis sticks the outside surface of the lung to the chest wall. This stops air or fluid being able to build up on the outside of the lung, which prevents the lung from collapsing again.

Pleurodesis sticks the outside surface of the lung to the chest wall. This stops air or fluid being able to build up on the outside of the lung, which prevents the lung from collapsing again.

There are two types of pleurodesis, chemical and mechanical, and they both work by irritating the outside of the lung, so it sticks to the chest wall. Chemical pleurodesis uses chemicals, most commonly talc (magnesium silicate), to irritate the lung. Chemical pleurodesis can be performed during surgery or via a chest tube, if you already have one in place. In mechanical pleurodesis, a surgeon uses a piece of gauze to gently scratch the surface of the lung, thus causing it to stick to the chest wall.

Additionally, pleurectomy can be used to remove the lining between the chest wall and the lung, making it easier for the the lung to stick to the chest wall. BHD pulmonary experts from the Netherlands have recently suggested that combined pleurectomy and pleurodesis may be the best way to stop BHD patients having multiple pneumothoraces (1).

Lung resection, blebectomy and bullectomy

Lung resection is the surgical removal of a part of a lung or a whole lung. Lung blebs and bullae are small and large air sacs on the surface of the lung respectively. A blebectomy or bullectomy is the removal of these lung cysts.

Following these procedures, your lung function may decrease to some extent due to the removal of lung tissue. If your surgeon suggests any of these treatments, make sure your surgeon knows you have BHD syndrome and may have more lung collapses or develop more cysts in the future. If possible, get a second opinion so you can make an informed choice. In some cases though, removal of cysts or lung tissue may be the best option.

Pleural Covering

Pleural covering is a relatively new surgical technique that involves covering either all of the lung (total pleural covering, TPC) or the just the lower areas of the lung (lower pleural covering, LPC) with a mesh which dissolves into the surface of the lung and strengthens the tissue. A study published in 2018 from Japan enrolled a total of 81 pneumothorax patients diagnosed with BHD from 2010-2017 (2). LPC significantly reduced the frequency of pneumothorax episodes but was not able to completely prevent pneumothorax recurring. However, in this study, TPC was able to completely prevent the recurrence of pneuomothorax. One caveat to this was that the median follow up period after TPC was significantly shorter than LPC. Further studies into the long-term effects of this procedure are warranted.

References

1. Christiaan Johannesma P, Houweling AC, Van Waesberghe JHTM, Van Moorselaar RJJA, Starink TM, Menko FH, et al. The pathogenesis of pneumothorax in Birt-Hogg-Dubé syndrome: A hypothesis. Respirology [Internet]. 2014 Nov 1 [cited 2021 May 19];19(8):1248–50. Available from: https://pubmed.ncbi.nlm.nih.gov/25302759/

2. Mizobuchi T, Kurihara M, Ebana H, Yamanaka S, Kataoka H, Okamoto S, et al. A total pleural covering of absorbable cellulose mesh prevents pneumothorax recurrence in patients with Birt-Hogg-Dubé syndrome. Orphanet J Rare Dis [Internet]. 2018 May 15 [cited 2021 May 19];13(1):1–7. Available from: https://doi.org/10.1186/s13023-018-0790-x

Publication date: December 2014
Review date: May 2021