Air Travel in Diffuse Cystic Lung Diseases

Patients with diffuse cystic lung diseases (DCLDs) require special consideration with respect to air travel. Gas exchange abnormalities may be present due to the diffuse parenchymal lung disease, and the decreased partial pressure of oxygen in the aircraft cabin increases the risk of in-flight hypoxemia. Additionally, in accordance with Boyle’s law, DCLD patients are at increased risk of development of spontaneous pneumothorax due to the expansion of the volume of parenchymal cysts in the setting of reduced cabin pressure of an aircraft. The risk of spontaneous pneumothorax associated with air travel has been examined in various survey-based studies conducted in patients with the three major DCLDs: lymphangioleiomyomatosis (LAM), Birt-Hogg-Dube syndrome (BHD), and pulmonary Langerhans cell histiocytosis (PLCH). In this chapter, we review the physiology behind the risks associated with air travel in DCLDs along with the available literature concerning risk assessment for flying in this patient population. We also briefly mention the potential physiologic consequences that underwater diving could have for DCLD patients.