Abstract

Spontaneous pneumothorax (SP) is a common manifestation of patients with diffuse cystic lung diseases (DCLDs) such as lymphangieoleiomyomatosis (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 regards 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.

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