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Pulmonary interstitial glycogenosis in the setting of lung growth abnormality: radiographic and pathologic

Monette Castillo1, Aruna Vade, Jennifer Eden Lim-Dunham

  • 1Department of Radiology, Loyola University Health System, Maywood, IL 60153, USA. mcastillo@lumc.edu

Pediatric Radiology
|May 5, 2010
PubMed

Insights

Pulmonary interstitial glycogenosis (PIG), a rare pediatric lung disease, can occur with lung growth abnormalities. Diagnosis requires histopathology due to nonspecific imaging findings in infants.

Area of Science:

  • Pediatric Pulmonology
  • Neonatology
  • Pathology

Background:

  • Pulmonary interstitial glycogenosis (PIG) is an uncommon cause of respiratory distress in neonates.
  • Interstitial lung diseases in children often present with nonspecific radiographic findings.

Observation:

  • A term infant boy exhibited tachypnea and required oxygen support shortly after birth.
  • Imaging revealed hyperinflation, diffuse interstitial markings, and cystic changes on chest X-ray and HRCT.
  • Open lung biopsy showed PIG with underlying poor alveolarization.

Findings:

  • This case suggests PIG may be more prevalent in conjunction with lung growth abnormalities than previously recognized.
  • The histopathological findings were crucial for differentiating PIG from other pediatric interstitial lung diseases.

Implications:

  • Early recognition and accurate diagnosis of PIG are vital for appropriate management of affected infants.
  • Further research into the association between PIG and lung development is warranted.

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