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Congenital surfactant protein B deficiency--emphasis on imaging
B Newman1, J P Kuhn, S S Kramer
1Department of Radiology, Children's Hospital of Pittsburgh, 3705 Fifth Avenue, Pittsburgh, PA 15213, USA. Newmanb@chplink.chp.edu
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.