Pulmonary alveolar proteinosis requiring "hybrid" extracorporeal life support, and complicated by acute necrotizing pneumonia

Abstract

Pulmonary alveolar proteinosis (PAP), which was first described by Rosen in 1958, is a rare disease characterized by impaired surfactant metabolism that provokes the accumulation of proteinaceous material in the alveoli. PAP is usually an auto-immune disease though, less commonly, may be congenital or secondary to another underlying disorder, such as infection, an immunodeficiency or a haematological disease. A positive diagnosis can be made with the appearance of "crazy-paving" on a computed tomography scan, with a milky fluid bronchial aspiration. A cytological examination will also show eosinophilic material and pink stained periodic acid-Schiff-positive material. A whole lung lavage is the most widely accepted therapy for hypoxemic PAP. Herein, we report the case of a 27-year-old woman admitted into our intensive care unit for hypoxemic PAP that was complicated by a pulmonary embolism. Because the patient presented with refractory hypoxemia associated with a brief cardiac arrest, femoral veno-arterial extracorporeal life support (ECLS) was rapidly inserted. Under ECLS, the patient subsequently developed "Harlequin syndrome", which was managed using an original and minimally invasive method. A whole lung lavage as well as prone positioning was effectively performed under ECLS, and resulted in substantial improvement in oxygenation. The patient could be discharged from the hospital 40 days later.