Congenital surfactant protein B deficiency - emphasis on imaging

Abstract

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. Three newborn infants with severe respiratory distress exhibited prolonged diffuse pulmonary opacification radiographically. 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. Thin-section chest CT imaging contributes important information when this diagnosis is considered.