Although bronchopulmonary dysplasia (BPD) is a common cause of interstitial lung disease in chronically intubated premature neonates, other interstitial lung disease in nonintubated infants is rare. We present a case of pulmonary interstitial glycogenosis that developed in a nonintubated, 31-week gestation infant in whom infectious etiologies had been excluded. The infant was well initially and then developed respiratory distress at 18 days of life. Radiographs at first day of life were normal, but CT and radiographic findings at 18 days of life showed severe interstitial lung disease, mimicking BPD. Lung biopsy showed pulmonary interstitial glycogenosis. This entity is not well described in the pediatric radiology literature and is important to consider, as the condition is responsive to a course of corticosteroids.

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