Background: Pulmonary alveolar proteinosis is a rare disorder characterized by a large accumulation of lipoproteinaceous material within the alveoli. This causes respiratory failure due to a restriction of gas exchange and changes in the ventilation/perfusion ratio. Treatment methods include noninvasive pharmacological approaches and invasive procedures, such as whole-lung lavage under general anesthesia.
Methods: Based on the literature search using free-term key words, we have analyzed published articles concerning the perioperative management of adult and pediatric patients with pulmonary alveolar proteinosis.
Results and discussion: In total, 184 publications were analyzed. Only a few manuscripts were related to anesthetic, respiratory, and airway management in patients suffering from pulmonary alveolar proteinosis. Airway should be strictly separated using a double-lumen tube. Respiratory strategies involve the use of manual clapping, continuous positive airway pressure, high-frequency jet ventilation of the affected lung, and employment of venovenous extracorporeal membrane oxygenation in the most serious of cases.
Conclusion: The goal of this review is to summarize the current published information about an anesthetic management strategy with a focus on airway management, ventilation, and oxygenation techniques in PAP patients.