Our Answer

Although the cytologic findings are in keeping with Pulmonary Alveolar Proteinosis(PAP) the visual appearance of the fluid was not classic for PAP (should be milky opaque). Therefore unequivocal diagnosis of PAP was not be made. There was consideration of bronchiolitis obliterans. Repeat endoscopy did not show reflux and the biopsy was non-specific. He was continued on Prednisone and also his Gastric Tube was changed to a Gastro-Jejunal one, though there were difficulties around the anaesthetic.

The BAL respiratory cultures grew H. influenza and bramarella catarrhalis.

He was referred to our service upon discharge. Whilst appearing well, he was unexpectedly hypoxic with SaO2's of 82%. He was constitutionally well and the SaO2 rapidly improved though he still required nocturnal supplemental oxygen. We reviewed his Chest radiograph which showed a diffuse ground glass appearance and discussed his CT with our radiologist who pointed out the characteristic appearance of PAP. On discussion with our pathologists, the gross appearance of the BAL on bronchoscopy in the presence of positive PAS was considered sufficient for the diagnosis of PAP, though the original samples were not available for review. Having already read the literature, his parents were convinced he had PAP. There were asking what should be done next.

What would you do?

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