Whole Lung Lavage of Nine Children with Pulmonary Alveolar Proteinosis: Experience in a Tertiary Lung Center

BACKGROUND: Pulmonary alveolar proteinosis (PAP) is a rare disease in children, characterized by intra-alveolar accumulation of large amounts of surfactant proteins, which severely reduce gas exchange. Whole lung lavage (WLL) is the preferred technique for the treatment of severe PAP. CASE PRESENTATION: This report presents nine pediatric cases with advanced PAP who underwent WLL under general anesthesia during a 9 year period. One patient was treated with multiple unilateral WLL without employing cardiopulmonary bypass (CPB) and eight cases were treated by simultaneous lavage of both lungs using partial CPB. CONCLUSION: Our experience suggested that partial CPB was useful to support oxygenation during WLL in small children with severe PAP in whom lung separation and selective lavaging of each lung were impracticable.