A case-series of pulmonary alveolar proteinosis treated with bilateral simultaneous whole lung lavage: A novel treatment modality.

Pulmonary Alveolar Proteinosis (PAP) is a rare disease, 3.2-6.7 cases per million. It is caused by PAS positive lipoprotein accumulation in the airspace which increases the alveolar-arterial gradient resulting in hypoxemia. Given the rare nature of this disorder, the lack of procedure standardization, and the predominantly unilateral approach taken: this study demonstrates the outcomes and efficacy of a unique bilateral approach used in this patient population. Methods: Four patients were identified by chart review having biopsy proven PAP treated with Bilateral Simultaneous Whole Lung Lavage (BSWLL) between 2007 and 2013. A total of eight procedures were performed. These patients were followed, and their characteristics and outcomes are detailed in Table 1. The procedures were performed in the operating room with general anesthesia and paralysis. Patients\u27 were intubated with 13 French double lumen endotracheal tubes (ETT), and these were connected to a custom tubing system for lavage after isolation of the lungs from each other. Isolation was verified by applying water seal to one lung and incrementally adjusting the pressure to the opposite lung: this was verified in each lung to ensure no airleak was present. The first lung to be lavaged would be clamped and degassed over 5 minutes prior to instillation of 2 liters of normal saline at 37 °C at which point respiratory therapy would manually percuss the chest. 1L would be removed and then 1 L would be instilled. This was repeated until the fluid removed visually cleared. At this point all remaining fluid was drained, and the process would be repeated on the opposite lung. Results: Eight BSWLL procedures were done in four patients. The patients suffered no significant adverse events. Average procedure time was 3.73 hours. Mean residual fluid was 874.4 mL. Right lungs required an average of 20.2 L while Left lungs averaged 23.1 L. SpO2 went from a mean of 94% on 100% FiO2 pre-lavage to 95% immediately post-lavage to 96% on room air at first follow up appointment. There were no intraoperative complications and minimal post-op complications. Hospital stay after the procedure ranged from 0-2 days, mean of 0.87. DLCO increased from a mean of 14.9 to 16.8 Conclusions: WLL is the accepted standard of care for PAP, but the current treatment with alternating unilateral WLL requires relatively frequent procedures. This study demonstrates BSWLL could be a safe and effective treatment procedure for PAP. (Figure Presented)