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Updated: Aug 18, 2026

Protocol and Guidelines for Point-of-Care Lung Ultrasound in Diagnosing Neonatal Pulmonary Diseases Based on International Expert Consensus
Published on: March 6, 2019
Persistent interstitial pulmonary emphysema: another complication of the respiratory distress syndrome
Insights
Persistent interstitial pulmonary emphysema (PIPE) in infants can be localized or diffuse. Localized PIPE showed better outcomes with surgical resection, while diffuse PIPE led to high mortality, often with bronchopulmonary dysplasia.
Area of Science:
- Neonatal Medicine
- Pediatric Pulmonology
- Pathology
Background:
- Persistent interstitial pulmonary emphysema (PIPE) is a rare neonatal respiratory condition.
- Neonatal respiratory distress syndrome (RDS) is a common precursor, often treated with mechanical ventilation and oxygen therapy.
Purpose of the Study:
- To describe the clinical presentation, management, and outcomes of persistent interstitial pulmonary emphysema (PIPE) in neonates.
- To differentiate between localized and diffuse forms of PIPE and their associated prognoses.
Main Methods:
- Retrospective review of 22 infants diagnosed with PIPE during the neonatal period.
- Analysis of treatment strategies including artificial ventilation, oxygen therapy, and surgical resection.
- Histological examination of lung tissue.
Main Results:
- 21 of 22 infants received treatment for respiratory distress syndrome.
- Ten infants had localized PIPE; 7 underwent successful lung resection, and 3 died.
- Twelve infants had diffuse PIPE, with 11 developing bronchopulmonary dysplasia due to prolonged oxygen use; all 12 died.
- Histology revealed air-filled interstitial cysts, sometimes lined by giant cells.
Conclusions:
- Localized PIPE may be effectively treated with surgical resection, offering a survival advantage.
- Diffuse PIPE carries a poor prognosis, frequently complicated by bronchopulmonary dysplasia and leading to mortality.
- Histological features of interstitial cysts are characteristic of PIPE.
Abstract:
Persistent interstitial pulmonary emphysema (PIPE) was observed in 22 infants during the neonatal period. All of the infants experienced respiratory distress during the first few days of life, and 21 of them were treated for the respiratory distress syndrome with artificial ventilation or oxygen or both. Ten infants developed a localized form of PIPE, with air-filled interstitial cysts measuring up to 3.0 cm in diameter confined to one or more lobes of lung. The involved segment of lung was resected in seven of these infants, all of whom survived. The remaining three died and autopsies were performed. A diffuse form of PIPE was observed in the other 12 infants. Numerous cysts that were predominantly small (less than 0.3 cm) were seen in all lobes of both lungs. These infants received high concentrations of oxygen for prolonged periods, resulting in bronchopulmonary dysplasia in 11 of the infants. All 12 infants died. PIPE is characterized histologically by air-filled interstitial cysts partially lined by multi-nucleated giant cells.
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