Bilateral Whole Lung Lavage in Hereditary Pulmonary Alveolar Proteinosis in a 4-year-old Child Using Extracorporeal Membrane Oxygenation

Abstract

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.

Ten-year experience of whole lung lavage in pediatric Pulmonary Alveolar Proteinosis

“…Although we observed a higher rate of SAE in our smaller PAP patients that underwent SLT-based WLL, our approach using sequential blocking of the lavaged lung may also be of interest to other centers. One case report mentioned the use of two SLT for WLL in a PAP patient with atypical anatomy 6 , and another report recently described WLL in pediatric PAP supported by ECMO therapy 13 . The authors reported that their patient was too hypoxic and too small for DLT intubation, hence underwent cannulation and ECMO-treatment for WLL.…”