Pulmonary alveolar proteinosis in extremis: the case for aggressive whole lung lavage with extracorporeal membrane oxygenation support.
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.
Sihoe
Alan D L
AD
Division of Cardiothoracic Surgery, The University of Hong Kong, Grantham Hospital, Hong Kong SAR, China.
Ng
Vivian M W
VM
Liu
Raymond W T
RW
Cheng
Lik-Cheung
LC
D000328:Adult; D018893:Bronchoalveolar Lavage / Q000379:methods; D001999:Bronchoscopy; D018450:Disease Progression; D015199:Extracorporeal Membrane Oxygenation / Q000379:methods*; D005260:Female; D005336:Fiber Optic Technology; D005500:Follow-Up Studies; D006801:Humans; D011649:Pulmonary Alveolar Proteinosis / Q000175:diagnosis* / Q000628:therapy*; D011659:Pulmonary Gas Exchange; D013902:Radiography, Thoracic; D012121:Respiration, Artificial / Q000379:methods; D018570:Risk Assessment; D012720:Severity of Illness Index; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome