Air Travel-Related Spontaneous Pneumothorax in Diffuse Cystic Lung Diseases
Purpose of Review
Spontaneous pneumothorax (SP) is a common manifestation of patients with diffuse cystic lung diseases (DCLDs) such as lymphangioleiomyomatosis (LAM), pulmonary Langerhans cell histiocytosis (PLCH), and Birt-Hogg-Dubé syndrome (BHD). Air travel may pose an additional risk for the development of SP. Here, we summarize the literature pertaining to air travel-related SP in DCLDs in order to assist patients and clinicians in appropriate decision-making with regard to air travel.
Several recent studies have estimated that the per-flight risk of SP in patients with DCLDs is approximately 1%, with disease-specific risk estimates of 1.1–2.6% in LAM, 0–0.63% in BHD, and 0.37% in PLCH.
In general, it should be safe for most patients with DCLDs to undertake air travel. Patients should be counseled to seek medical attention and not board the airplane in the presence of sudden/new onset chest pain and/or dyspnea prior to boarding the plane.
KeywordsAir travel Birt-Hogg-Dubé syndrome Diffuse cystic lung disease Lymphangioleiomyomatosis Pulmonary Langerhans cell histiocytosis Spontaneous pneumothorax
N.G. received funding and support from the NIH, NIH grant number: U54HL127672, to conduct some of the research reported in this article.
Compliance with Ethical Standards
Conflict of Interest
Nishant Gupta and Nikolai Wajda declare no conflict of interest.
Human and Animal Rights and Informed Consent
This article does not contain any studies with human or animal subjects performed by any of the authors.
Papers of particular interest, published recently, have been highlighted as: • Of importance
- 1.International Air Transport Association (IATA). Annual Review 2017. IATA website. http://www.iata.org/media/annual-report-2017/index.html. Accessed August 30, 2017.
- 7.Federal Aviation Administration. 14CFR25.841 (a). US Government Publishing Office Website. https://www.gpo.gov/fdsys/pkg/CFR-1999-title14-vol1/pdf/CFR-1999-title14-vol1-sec25-841.pdf. Accessed August 30, 2017.
- 18.Franz DN, Brody A, Meyer C, et al. Mutational and radiographic analysis of pulmonary disease consistent with lymphangioleiomyomatosis and micronodular pneumocyte hyperplasia in women with tuberous sclerosis. Am J Respir Crit Care Med. 2001;164(4):661–8. https://doi.org/10.1164/ajrccm.164.4.2011025.CrossRefPubMedGoogle Scholar
- 22.Avila NA, Chen CC, Chu SC, et al. Pulmonary lymphangioleiomyomatosis: correlation of ventilation-perfusion scintigraphy, chest radiography, and CT with pulmonary function tests. Radiology. 2000;214(2):441–6. https://doi.org/10.1148/radiology.214.2.r00fe41441.CrossRefPubMedGoogle Scholar
- 23.Avila NA, Dwyer AJ, Murphy-Johnson DV, Brooks P, Moss J. Lymphangioleiomyomatosis: correlation of qualitative and quantitative thin-section CT with pulmonary function tests and assessment of dependence on pleurodesis. Radiology. 2002;223(1):189–97. https://doi.org/10.1148/radiol.2231010315.CrossRefPubMedGoogle Scholar
- 27.Hayashida M, Seyama K, Inoue Y, Fujimoto K, Kubo K. Respiratory Failure Research Group of the Japanese Ministry of Health, Labor, and Welfare. The epidemiology of lymphangioleiomyomatosis in Japan: a nationwide cross-sectional study of presenting features and prognostic factors. Respirology. 2007;12(4):523–30. https://doi.org/10.1111/j.1440-1843.2007.01101.x.CrossRefPubMedGoogle Scholar
- 31.Zbar B, Alvord WG, Glenn G, et al. Risk of renal and colonic neoplasms and spontaneous pneumothorax in the Birt-Hogg-Dubé syndrome. Cancer Epidemiol Biomark Prev. 2002;11(4):393–400.Google Scholar
- 33.• Johannesma, et al. Risk of spontaneous pneumothorax due to air travel and diving in patients with Birt-Hogg-Dubé syndrome. Springerplus. 2016;5(1):1506. https://doi.org/10.1186/s40064-016-3009-4. Survey-based study of 145 BHD patients estimating the risk of SP related to air travel in patients with BHD. CrossRefPubMedPubMedCentralGoogle Scholar
- 35.• Gupta N, Kopras EJ, Henske EP, James LE, El-Chemaly S, Veeraraghavan S, et al. Spontaneous pneumothoraces in patients with Birt–Hogg–Dubé syndrome. Ann Am Thorac Soc. 2017;14(5):706–13. https://doi.org/10.1513/AnnalsATS.201611-886OC. Survey of 104 BHD patients describing the risk of spontaneous pneumothorax attributed to air travel, as well as the burden of spontaneous pneumothoraces and the utility of pleurodesis in patients with BHD. CrossRefPubMedPubMedCentralGoogle Scholar
- 36.Hoshika Y, Kataoka H, Kurihara M, Ando K, Sato T, Seyama K, et al. Features of pneumothorax and risk of air travel in Birt-Hogg-Dubé syndrome. Am J Respir Crit Care Med. 2012;185:A4438.Google Scholar
- 39.Hartman TE, Tazelaar HD, Swensen SJ, Muller NL. Cigarette smoking: CT and pathologic findings of associated pulmonary diseases. Radiographics. 1997;17(2):377–90. https://doi.org/10.1148/radiographics.17.2.9084079.CrossRefPubMedGoogle Scholar
- 41.Singla A, Kopras EJ, McCormack FX, Gupta N. Management of spontaneous pneumothorax and safety of air travel in patients with pulmonary Langerhans cell histiocytosis. Am J Respir Crit Care Med. 2017;195:A1591.Google Scholar
- 42.Cheatham ML, Safcsak K. Air travel following traumatic pneumothorax: when is it safe? Am Surg. 1999;62:1160–4.Google Scholar
- 44.International Air Transport Association (IATA). IATA Medical Manual, February 2017. IATA website. http://www.iata.org/publications/Documents/medical-manual.pdf. Accessed Sept 21, 2017.
- 45.Aerospace Medical Association Medical Guidelines Task Force. Medical guidelines for airline travel, 2nd ed. Aviat Space Environ Med. 2003;74(5 Suppl):A1–19.Google Scholar
- 47.Gupta N, Langenderfer D, McCormack FX, Schauer DP, Eckman MH. Chest computed tomographic image screening for cystic lung diseases in patients with spontaneous pneumothorax is cost effective. Ann Am Thorac Soc. 2017;14(1):17–25. https://doi.org/10.1513/AnnalsATS.201606-459OC.CrossRefPubMedPubMedCentralGoogle Scholar