Pediatric Pulmonary Alveolar Proteinosis

  • Etiology: Disorder of surfactant turnover with abnormal intra-alveolar accumulation of surfactant-derived lipoproteinaceous material
  • Imaging CXR:
    — Classic is batwing pulmonary opacities in adults
    — Miliary pattern of diffuse pulmonary opacities is more common in children
    — May develop pulmonary fibrosis
  • Imaging CT: Crazy paving pattern
  • DDX:
  • Complications: Superimposed infection, pulmonary fibrosis
  • Treatment: Therapeutic whole-lung bronchoalveolar lavage, bilateral lung transplantation
  • Clinical:
    — Congenital form presents in neonatal period
    — May present as failure to thrive rather than respiratory distress

Radiology Cases of Pulmonary Alveolar Proteinosis

CXR of pulmonary alveolar proteinosis
CXR AP and lateral shows bilateral central pulmonary opacities in a near-batwing distribution.
CXR of pulmonary alveolar proteinosis
CXR AP shows bilateral diffuse and symmetrical interstitial infiltrates which are miliary in appearance.