Pediatric Radiology

, Volume 48, Issue 8, pp 1066–1072 | Cite as

High-resolution CT findings of pulmonary interstitial glycogenosis

  • Jason P. WeinmanEmail author
  • Christina J. White
  • Deborah R. Liptzin
  • Robin R. Deterding
  • Csaba Galambos
  • Lorna P. Browne
Original Article



Pulmonary interstitial glycogenosis is a form of childhood interstitial lung disease characterized by the histological finding of abundant glycogen-laden mesenchymal cells within the pulmonary interstitium. Patients present in the neonatal period with disproportionate respiratory distress. Often, pulmonary interstitial glycogenosis is accompanied by alveolar simplification complicating recognition and diagnosis. Despite the recognition of pulmonary interstitial glycogenosis as a distinct entity, only a few case reports describing imaging findings are found in the literature, with no published systematic review available.


The purpose of this review is to provide a review of CT findings of pulmonary interstitial glycogenosis with histological correlation to aid in early diagnosis and management.

Materials and methods

A 10-year retrospective review was performed to identify pediatric patients <18 years who underwent biopsy and CT within the last 10 years at our institution. The inclusion criteria include patients who had a CT within 3 months of biopsy and pathology-proven pulmonary interstitial glycogenosis CTs that were evaluated by three radiologists using a standardized scoring system.


Fifteen patients met inclusion criteria (9 male, 6 female). At the time of initial pre-biopsy CT, ages ranged from 2 weeks to 5 months. Pulmonary symptoms presented at birth in the majority of patients (n=13). Two patients presented in early infancy at 3 months (n=1) and 5 months (n=1). Ground glass opacities were the most common CT finding (n=14), which varied from diffuse to scattered. Cystic lucencies (n=11) were noted in the majority of patients as well. Interlobular septal thickening (n=10) and architectural distortion (n=8) were less common findings.


The most common CT findings of pulmonary interstitial glycogenosis are ground glass opacities with cystic lucencies. While the imaging findings are distinct from the typical presentation of neuroendocrine hyperplasia of infancy, there is significant overlap of these findings with surfactant dysfunction mutations, entities that also present with respiratory distress in the neonatal period. Therefore, imaging findings in pulmonary interstitial glycogenosis are helpful in guiding the need for genetic testing and/or biopsy.


Chest Children Computed tomography Interstitial lung disease Lungs Pulmonary interstitial glycogenosis 


Compliance with ethical standards

Conflicts of interest

Dr. R. R. Deterding discloses the following: cofounder and board member of Triple Endoscopy Inc., patent on novel endoscopy scope, and advisory board of Pediatric Interstitial Lung Disease for Boehringer Ingelheim.


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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2018

Authors and Affiliations

  • Jason P. Weinman
    • 1
    Email author
  • Christina J. White
    • 2
  • Deborah R. Liptzin
    • 3
  • Robin R. Deterding
    • 3
  • Csaba Galambos
    • 4
  • Lorna P. Browne
    • 1
  1. 1.Department of RadiologyChildren’s Hospital ColoradoAuroraUSA
  2. 2.Diversified RadiologyLakewoodUSA
  3. 3.Department of Pediatrics and the Division of Pediatric Pulmonary MedicineChildren’s Hospital ColoradoAuroraUSA
  4. 4.Department of Pathology and Laboratory MedicineChildren’s Hospital ColoradoAuroraUSA

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