Pulmonary alveolar proteinosis (PAP) is a rare pulmonary disease. Diagnosis is established by bronchoalveolar lavage (BAL), which has macroscopic ‘milky appearance’, and in the presence of typical computed tomography, findings are diagnostic of PAP but, however, the feature of periodic acid–Schiff-positive eosinophilic proteinaceous fluid raises the confidence of the diagnosis. We report late-onset PAP in a 10-year-old girl who had acid fast bacilli on an initial BAL examination, but was subsequently diagnosed as PAP.

A bstract The hereditary form of pulmonary alveolar proteinosis (PAP) is an uncommon entity. We report a case of PAP due to colony-stimulating factor 2 receptor alpha (CSF2RA) gene mutation. The standard of care includes whole lung lavage (WLL). We faced two challenges: Firstly, a severely hypoxemic patient, and secondly, the nonavailability of appropriate size of double-lumen endotracheal tube for pediatric patients for a WLL while permitting single-lung ventilation. Hence, we performed WLL using venovenous extracorporeal membrane oxygenation (VV ECMO) with a successful outcome. The patient has been discharged and is off oxygen support since more than a year. There are only a few case reports of children having hereditary PAP treated with WLL using ECMO in Indian and Western literature. How to cite this article Prabhudesai P, Khosla I, Kulkarni S, Arya MK, Pandey A, Yadav N. Bilateral Whole Lung Lavage in Hereditary Pulmonary Alveolar Proteinosis in a 4-year-old Child Using Extra corporeal Membrane Oxygenation. Indian J Crit Care Med 2021;25(9):1069–1072.