Current Pulmonology Reports

, Volume 7, Issue 2, pp 56–62 | Cite as

Air Travel-Related Spontaneous Pneumothorax in Diffuse Cystic Lung Diseases

  • Nikolai Wajda
  • Nishant Gupta
Pleural Diseases and Mesothelioma (G Lee, Section Editor)
Part of the following topical collections:
  1. Topical Collection on Pleural Diseases & Mesothelioma


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.

Recent Findings

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.


Air 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.

Supplementary material

13665_2018_199_MOESM1_ESM.wav (22.7 mb)
ESM 1 (WAV 23207 kb)


Papers of particular interest, published recently, have been highlighted as: • Of importance

  1. 1.
    International Air Transport Association (IATA). Annual Review 2017. IATA website. Accessed August 30, 2017.
  2. 2.
    Peterson DC, Martin-Gill C, Guyette FX, et al. Outcomes of medical emergencies on commercial airline flights. N Engl J Med. 2013;368(22):2075–83. Scholar
  3. 3.
    Sand M, Bechara FG, Sand D, Mann B. Surgical and medical emergencies on board European aircraft: a retrospective study of 10189 cases. Crit Care. 2009;13(1):R3. Scholar
  4. 4.
    Coker RK, Shiner RJ, Partridge MR. Is air travel safe for those with lung disease? Eur Respir J. 2007;30(6):1057–63. Scholar
  5. 5.
    Taveira-DaSilva AM, Burstein D, Hathaway OM, Fontana JR, Gochuico BR, Avila NA, et al. Pneumothorax after air travel in lymphangioleiomyomatosis, idiopathic pulmonary fibrosis and sarcoidosis. Chest. 2009;136(3):665–70. Scholar
  6. 6.
    Cottrell JJ. Altitude exposures during aircraft flight. Chest. 1988;92(1):81–4. Scholar
  7. 7.
    Federal Aviation Administration. 14CFR25.841 (a). US Government Publishing Office Website. Accessed August 30, 2017.
  8. 8.
    Steagall WK, Glasgow CG, Hathaway OM, et al. Genetic and morphologic determinants of pneumothorax in lymphangioleiomyomatosis. Am J Physiol Lung Cell Mol Physiol. 2007;293(3):L800–8. Scholar
  9. 9.
    Hu X, Cowl CT, Baqir M, Ryu JH. Air travel and pneumothorax. Chest. 2014;145(4):688–94. Scholar
  10. 10.
    Nicholson TT, Sznajder JI. Fitness to fly in patients with lung disease. Ann Am Thorac Soc. 2014;11(10):1614–22. Scholar
  11. 11.
    Ahmedzai, et al. British thoracic society standards of care committee. Managing passengers with stable respiratory disease planning air travel. British thoracic society recommendations. Thorax. 2011;66(Suppl 1):i1–i30. Scholar
  12. 12.
    Baumann MH. Pneumothorax and air travel. Lessons learned from a bag of chips. Chest. 2009;136(3):655–6. Scholar
  13. 13.
    Harknett EC, Chang WY, Byrnes S, et al. Use of variability in national and regional data to estimate the prevalence of lymphangioleiomyomatosis. Q J Med. 2011;104(11):971–9. Scholar
  14. 14.
    Ryu JH, Moss J, Beck GJ, et al. The NHLBI lymphangioleiomyomatosis registry: characteristics of 230 patients at enrollment. Am J Resp Crit Care Med. 2006;173(1):105–11. Scholar
  15. 15.
    Osborne JP, Fryer A, Webb D. Epidemiology of tuberous sclerosis. Ann N Y Acad Sci. 1991;615:125–7.CrossRefPubMedGoogle Scholar
  16. 16.
    Costello LC, Hartman TE, Ryu JH. High frequency of pulmonary lymphangioleiomyomatosis in women with tuberous sclerosis complex. Mayo Clin Proc. 2000;75(6):591–4. Scholar
  17. 17.
    Moss J, Avila NA, Barnes PM, et al. Prevalence and clinical characteristics of lymphangioleiomyomatosis (LAM) in patients with tuberous sclerosis complex. Am J Respir Crit Care Med. 2001;164(4):669–71. Scholar
  18. 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. Scholar
  19. 19.
    Muzykewicz DA, Sharma A, Muse V, Numis AL, Rajagopal J, Thiele EA. TSC1 and TSC2 mutations in patients with lymphangioleiomyomatosis and tuberous sclerosis complex. J Med Genet. 2009;46(7):465–8. Scholar
  20. 20.
    Cudzilo CJ, Szczesniak RD, Brody AS, et al. Lymphangioleiomyomatosis screening in women with tuberous sclerosis. Chest. 2013;144(2):578–85. Scholar
  21. 21.
    Crausman RS, Lynch DA, Mortensen RL, et al. Quantitative CT predicts the severity of physiologic dysfunction in patients with lymphangioleiomyomatosis. Chest. 1996;109(1):131–7. Scholar
  22. 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. Scholar
  23. 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. Scholar
  24. 24.
    Chu S, et al. Comprehensive evaluation of 35 patients with lymphangioleiomyomatosis. Chest. 1999;115(4):1041–52. Scholar
  25. 25.
    Almoosa KF, et al. Management of pneumothorax in lymphangioleiomyomatosis: effects on recurrence and lung transplantation complications. Chest. 2006;129(5):1274–81. Scholar
  26. 26.
    Young LR, Almoosa KF, Pollock-BarZiv S, Coutinho M, McCormack FX, Sahn SA. Patient perspectives on management of pneumothorax in lymphangioleiomyomatosis. Chest. 2006;129(5):1267–73. Scholar
  27. 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. Scholar
  28. 28.
    Pollock-BarZiv S, Cohen MM, Downey GP, Johnson SR, Sullivan E, McCormack FX. Air travel in women with lymphangioleiomyomatosis. Thorax. 2007;62(2):176–80. Scholar
  29. 29.
    Gonano C, Pasquier J, Daccord C, Lazor R. Risk of pneumothorax due to air travel in pulmonary lymphangioleiomyomatosis. Respiration. 2017;94:150–6. Scholar
  30. 30.
    Gupta N, Vassallo R, Wikenheiser-Brokamp KA, McCormack FX. Diffuse cystic lung disease. Part II. Am J Respir Crit Care Med. 2015;192:17–29. Scholar
  31. 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
  32. 32.
    Toro JR, Pautler SE, Stewart L, et al. Lung cysts, spontaneous pneumothorax, and genetic associations in 89 families with Birt-Hogg-Dubé syndrome. Am J Respir Crit Care Med. 2007;175(10):1044–53. Scholar
  33. 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. Survey-based study of 145 BHD patients estimating the risk of SP related to air travel in patients with BHD. CrossRefPubMedPubMedCentralGoogle Scholar
  34. 34.
    Furuya M, Yao M, Tanaka R, et al. Genetic, epidemiologic and clinicopathologic studies of Japanese Asian patients with Birt-Hogg-Dubé syndrome. Clin Genet. 2016;90(5):403–12. Scholar
  35. 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. 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. 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
  37. 37.
    Vassallo R, Harari S, Tazi A. Current understanding and management of pulmonary Langerhans cell histiocytosis. Thorax. 2017;72(10):937–45. Scholar
  38. 38.
    Bonelli FS, Hartman TE, Swensen SJ, Sherrick A. Accuracy of high-resolution CT in diagnosing lung diseases. AJR Am J Roentgenol. 1998;170(6):1507–12. Scholar
  39. 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. Scholar
  40. 40.
    Mendez JL, Nadrous HF, Vassallo R, et al. Pneumothorax in pulmonary Langerhans cell histiocytosis. Chest. 2004;125(3):1028–32. Scholar
  41. 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. 42.
    Cheatham ML, Safcsak K. Air travel following traumatic pneumothorax: when is it safe? Am Surg. 1999;62:1160–4.Google Scholar
  43. 43.
    Tam A, Singh, Gupta S, et al. Air travel after biopsy-related pneumothorax: is it safe to fly? J Vasc Interv Radiol. 2011;22(5):595–602. Scholar
  44. 44.
    International Air Transport Association (IATA). IATA Medical Manual, February 2017. IATA website. Accessed Sept 21, 2017.
  45. 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
  46. 46.
    Postmus P, Johannesma, et al. In-flight pneumothorax: diagnosis may be missed because of symptom delay. Am J Respir Crit Care Med. 2014;190(6):704–5. CrossRefPubMedGoogle Scholar
  47. 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. Scholar

Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Division of Pulmonary, Critical Care, and Sleep MedicineUniversity of CincinnatiCincinnatiUSA

Personalised recommendations