Coexistence Of Congenital Lobar Emphysema And Pulmonary Interstitial Glycogenosis In A Neonate

Hilvers, Pamela; Polak, Mark J.; Bacaj, Patrick; Gustafson, Robert A.

doi:10.1164/ajrccm-conference.2010.181.1_meetingabstracts.a6737

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2022

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“…Lobectomy of the left upper lobe and pathology revealed extended alveoli and alveolar ducts consistent with CLE. In addition, there was septal widening with glycogen laden cells, supporting PIG 14…”

Section: Discussionmentioning

confidence: 56%

Rare interstitial lung disease in a preterm neonate with congenital lobar emphysema

et al. 2022

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Congenital lobar emphysema (CLE)/congenital alveolar overdistension/congenital lobar overinflation or infantile lobar emphysema is a rare developmental anomaly of the lower respiratory tract which is characterised by hyperinflation of one or more of the pulmonary lobes. Histopathology may be variable, which may show abnormality in the cartilage, granulation tissue, mucosal folds, etc. We report a rare underlying histopathology in a preterm neonate with CLE. This entity referred to as pulmonary interstitial glycogenosis (PIG) is a group of heterogeneous lung disease affecting the lung parenchyma and is characterised by the presence of glycogen laden cells in the lung interstitium. This impairs the gas exchange and typically manifest as tachypnoea, retractions, hypoxia and increased respiratory support. Our case report highlights the association of CLE and PIG and a review of literature. One should always have a detailed histopathology in children presenting with CLE to rule out PIG.

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Section: Discussionmentioning

confidence: 56%