Infantile pulmonary alveolar proteinosis with interstitial pneumonia: Bilateral simultaneous lung lavage utilizing extracorporeal membrane oxygenation and steroid therapy

An infant with refractory pulmonary alveolar proteinosis (PAP) associated w ith severe interstitial pneumonia is described. Although she was treated by bilateral simultaneous lung lavage utilizing extracorporeal membrane oxyge nation and steroid therapy, she died of progressive respiratory failure 28 days after admission. Histologic examination of lung autopsy specimen showe d only partial alveolar spaces to be filled with a dense PAS positive granu lar eosinophilic material and showed severe interstitial pneumonia with mar ked fibrosis of alveolar walls and interstitium. The lung lavage seemed to be effective for PAP because the effluent fluid sufficiently became clear a nd the PBX positive material was detected only in partial alveoli. The full venoarterial cardiopulmonary bypass with extracorporeal membrane oxygenati on seemed to be very useful to support bilateral lung lavage for small infa nts. The refractory symptoms and failure of treatment were resulted from th e association of severe interstitial pneumonia. In neonates or infants with PAP and severe interstitial pneumonia with poor response for steroid thera py, the lung transplantation should be considered.