Abstract
BackgroundPulmonary interstitial glycogenosis (PIG) arises from a developmental disorder of the pulmonary mesenchyme and presents clinically with reversible neonatal respiratory distress and/or persistent pulmonary hypertension of the newborn (PPHN).ObjectiveWe report two cases of PIG in patients with congenital heart disease (CHD) and evidence of PPHN.ResultsBoth cases demonstrated the hallmark PIG histologic finding of diffuse, uniform interstitial thickening due to the presence of immature interstitial cells containing abundant cytoplasmic glycogen.ConclusionsWe report the second and third patients with PIG associated with CHD. Because histologic examination is required to establish the diagnosis, we speculate that PIG, although rare, may be underrecognized in neonates presenting with PPHN in the setting of CHD.
Highlights
Pediatric interstitial lung disease has an estimated prevalence of 1.3 to 3.6/1,000,000 [5, 6]
We report two cases of Pulmonary interstitial glycogenosis (PIG) in patients with congenital heart disease (CHD) and evidence of pulmonary hypertension of the newborn (PPHN)
We report the second and third patients with PIG associated with CHD
Summary
Pediatric interstitial lung disease has an estimated prevalence of 1.3 to 3.6/1,000,000 [5, 6]. Objective We report two cases of PIG in patients with congenital heart disease (CHD) and evidence of PPHN. Results Both cases demonstrated the hallmark PIG histologic finding of diffuse, uniform interstitial thickening due to the presence of immature interstitial cells containing abundant cytoplasmic glycogen. Keywords Congenital heart defects Á Glycogen storage diseases Á Interstitial lung diseases Á Neonatal respiratory distress syndrome Á Persistent pulmonary hypertension of the newborn
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