Respiratory Strategies and Airway Management in Patients with Pulmonary Alveolar Proteinosis: A Review

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.