Pulmonary interstitial glycogenosis in the setting of lung growth abnormality: radiographic and pathologic correlation.
Pulmonary interstitial glycogenosis (PIG) is a rare pediatric interstitial lung disease. We report a case of a term boy presenting with tachypnea at birth requiring supplemental oxygen. Chest radiographs followed by high-resolution CT (HRCT) demonstrated hyperinflation and diffuse interstitial markings interspersed with multiple cystic spaces. An open lung biopsy demonstrated a minor component of PIG superimposed upon poor alveolarization. PIG in the setting of lung growth abnormality might be more common than previously described. Additionally, radiographic findings associated with most pediatric interstitial lung diseases are nonspecific, and histopathologic correlation is essential for diagnosis.
Castillo
Monette
M
Department of Radiology, Loyola University Health System, Maywood, IL 60153, USA. mcastillo@lumc.edu
Vade
Aruna
A
Lim-Dunham
Jennifer Eden
JE
Masuda
Emi
E
Massarani-Wafai
Rasan
R
D001706:Biopsy; D006008:Glycogen Storage Disease / Q000000981:diagnostic imaging* / Q000473:pathology; D006801:Humans; D007231:Infant, Newborn; D008168:Lung / Q000002:abnormalities* / Q000000981:diagnostic imaging; D017563:Lung Diseases, Interstitial / Q000000981:diagnostic imaging* / Q000473:pathology; D008297:Male; D013902:Radiography, Thoracic; D014057:Tomography, X-Ray Computed / Q000379:methods*