Nadya Ben Fadel, Elham Almoli, Joseph de Nanassy, Sally Mashally
Children’s Hospital of Eastern Ontario and University of Ottawa.
Canada
Case Reports in Pediatrics
Case Rep Pediatr 2025;
DOI: 10.1155/crpe/6268296
Abstract
We present a case of a premature infant who had a persistent patent ductus arteriosus (PDA) and subsequently developed severe pulmonary hypertension (PHT) and respiratory failure. A lung biopsy was performed during PDA ligation, revealing a consistent thickening of the interstitial tissue. The biopsy also showed the presence of immature interstitial cells containing high amounts of cytoplasmic glycogen, indicative of pulmonary interstitial glycogenosis (P.I.G). There was no conclusive evidence of maturational arrest, infection, alveolar proteinosis, or alveolar capillary dysplasia. While the association between P.I.G and PHT has been documented in case reports involving children with congenital heart disease (CHD), the majority of these cases involved full-term infants and older children. Notably, there have been no reports on the diagnosis of P.I.G in infants with isolated PDA, without any other congenital heart conditions. This expands the existing knowledge of P.I.G, highlighting the diagnostic value of lung biopsy in premature infants with long-standing PDA and severe PHT exhibiting similar characteristics.
Category
Class I. Pulmonary Hypertension Associated with Congenital Cardiovascular Disease
Class III. Pulmonary Hypertension Associated with Lung Disease
Class III. Pulmonary Hypertension Associated with Developmental Diseases of the Lung
Pulmonary Vascular Pathology
Age Focus: Pediatric Pulmonary Vascular Disease
Fresh or Filed Publication: Fresh (PHresh). Less than 1-2 years since publication
Article Access
Free PDF File or Full Text Article Available Through PubMed or DOI: Yes