Pulmonary Alveolar Proteinosis in Extremis: The Case for Aggressive Whole Lung Lavage with Extracorporeal Membrane Oxygenation Support

Abstract

Pulmonary alveolar proteinosis (PAP) is a rare disorder in which lipoproteinaceous material is deposited in the alveoli, compromising gaseous exchange. We report the case of a 29-year-old female patient presenting with the most extreme case of PAP yet reported. She successfully managed by aggressive bilateral whole lung lavage (WLL) in a single sitting using extracorporeal membrane oxygenation (ECMO) support. Despite critical hypercarbia and ventilator-dependence for 12 days before lavage, the patient experienced rapid recovery of pulmonary function after WLL and ECMO could be discontinued on-table. Aggressive WLL with ECMO support can be safe and effective even in the most severe cases of PAP. © 2006 Australasian Society of Cardiac and Thoracic Surgeons and the Cardiac Society of Australia and New Zealand.