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Updated: Aug 5, 2026
Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Cystic lung lesions secondary to persistent pulmonary interstitial emphysema in preterm infants: imaging features and
Diana Veiga-Canuto1,2, Jeffrey Traubici3, David Manson3
1Department of Medical Imaging, Hospital Universitari i Politècnic La Fe, Avinguda Fernando Abril Martorell 106, Valencia, 46026, Spain. dianaveigac@gmail.com.
Background:
Persistent pulmonary interstitial emphysema (PIE) with cystic lung formation is a rare but potentially serious complication in preterm infants with respiratory distress syndrome (RDS).
Objective:
To characterize the imaging features and natural history of cystic lung lesions arising from persistent pulmonary interstitial emphysema in preterm infants with respiratory distress syndrome, and to correlate these findings with clinical management.
Materials And Methods:
We retrospectively reviewed preterm infants (≤37 weeks gestational age (GA)) with RDS complicated by persistent PIE and subsequent cystic lung lesions at a dedicated neonatal intensive care unit between 2004 and 2024. Imaging findings, clinical courses, management strategies, and outcomes were reviewed.
Results:
Thirty-one preterm infants (median GA 28 weeks) were included. Persistent PIE was defined as radiographic evidence of pulmonary interstitial emphysema persisting for more than 72 h. Lesions developed at a median of 14 days of life were predominantly unilateral (96.8%), often multiple (74.2%), and mainly in the lower lobes (45.2%). Complications included mediastinal shift in 45.2% and pneumothorax in 29.0%. Conservative management including reduced ventilatory pressures and high-frequency oscillatory ventilation was the most common strategy. PIE resolved in 66.7% of infants with available follow-up data (20/30). Overall mortality was 32.3%. No statistically significant associations were found between short-term mortality and GA, pneumothorax, cyst type, or mediastinal shift; however, given the small sample size, these analyses are exploratory.
Conclusion:
Persistent PIE presenting as cystic lung lesions is associated with significant short-term morbidity, including pneumothorax and mediastinal shift, but short-term mortality appears lower than that reported in historical series, though direct comparisons are limited by differences in population and management. Integrating clinical history and temporal radiographic context is essential for accurate diagnosis and appropriate management.
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