Congenital surfactant protein B deficiency--emphasis on imaging.

BACKGROUND AND OBJECTIVES: Surfactant protein B deficiency has become increasingly recognized as a cause of severe prolonged respiratory distress. Little has been written about the imaging appearance and the role of imaging in diagnosis.
MATERIALS AND METHODS: Three newborn infants with severe respiratory distress exhibited prolonged diffuse pulmonary opacification radiographically.
RESULTS: Diffuse ground-glass opacity and markedly prominent interlobular septa suggesting alveolar proteinosis were present on early thin-section chest computed tomographic (CT) images. Fibrotic changes with prominent interlobular septal thickening were present on a later CT. Surfactant protein B deficiency was confirmed by alveolar lavage and peripheral blood DNA analysis.
CONCLUSION: Thin-section chest CT imaging contributes important information when this diagnosis is considered.