What is the diagnosis and treatment for Neonatal Pulmonary Interstitial Emphysema (PIE)?

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Last updated: March 26, 2025View editorial policy

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From the Guidelines

Pulmonary interstitial emphysema (PIE) in neonates should be managed with gentle ventilation strategies, prioritizing reducing barotrauma and minimizing positive end-expiratory pressure (PEEP) to 4-5 cmH2O, as supported by recent guidelines 1. The primary approach involves decreasing peak inspiratory pressures, using high-frequency ventilation, and conventional ventilation with rates of 60-100 breaths per minute and low tidal volumes (3-4 mL/kg). High-frequency oscillatory ventilation may be beneficial with settings of 10-15 Hz frequency, mean airway pressure of 8-12 cmH2O, and amplitude adjusted to achieve visible chest wall vibration. Some key considerations in the management of PIE include:

  • Reducing barotrauma through gentle ventilation strategies
  • Minimizing PEEP to 4-5 cmH2O
  • Using high-frequency ventilation and conventional ventilation with low tidal volumes
  • Considering selective bronchial intubation of the unaffected lung for unilateral PIE
  • Providing supportive care, including maintaining adequate oxygenation (target SpO2 90-95%), careful fluid management, and positioning the patient with the affected side down when PIE is localized. It is essential to recognize that PIE can progress to pneumothorax and pneumomediastinum if left untreated, highlighting the need for early recognition and intervention 1. In severe cases unresponsive to ventilation strategies, surgical interventions like lobectomy might be necessary, though this is rare. The use of exogenous surfactant may improve lung expansion and reverse PIE, particularly in infants with meconium aspiration syndrome or other significant parenchymal disease, as noted in guidelines for pediatric pulmonary hypertension 1. However, the decision to use surfactant should be made cautiously, considering the potential risks and benefits, and the most recent evidence should guide this decision 1.

From the Research

Diagnosis of Neonatal Pulmonary Interstitial Emphysema (PIE)

  • PIE is a pathological state when air escapes from ruptured alveoli and is trapped along the sheaths surrounding the bronchovascular bundle 2
  • It is a frequent complication in preterm-ventilated infants, who have an increased risk of mortality and bronchopulmonary dysplasia 3
  • Diagnosis can be confirmed by chest radiograph and chest computerized tomography 2

Treatment of Neonatal Pulmonary Interstitial Emphysema (PIE)

  • Various treatments for PIE have been proposed, including:
    • High-frequency ventilation 3
    • Postural therapy 3, 2, 4
    • Selective bronchial intubation 3, 5
    • Surgery 3
    • Therapeutic lung puncture 6
  • Conservative approach should remain the first choice treatment of unilateral PIE in order to limit surgical procedures 3
  • Positional therapy alone can be an effective treatment for unilateral PIE, with significant clinical and radiographic improvement noticed within a few days 2, 4
  • Selective lung ventilation can be a therapeutic option for refractory PIE 5
  • Therapeutic lung puncture with consecutive tension release of the overinflated lung by drainage is a therapeutic option for premature infants suffering from diffuse PIE in whom other conservative measures fail 6

References

Guideline

Guideline Directed Topic Overview

Dr.Oracle Medical Advisory Board & Editors, 2025

Research

Pulmonary interstitial emphysema after resolution of relapsing pneumothorax and discontinuation of mechanical ventilation. An atypical case in a preterm infant.

The journal of maternal-fetal & neonatal medicine : the official journal of the European Association of Perinatal Medicine, the Federation of Asia and Oceania Perinatal Societies, the International Society of Perinatal Obstetricians, 2014

Research

Therapeutic lung puncture for diffuse unilateral pulmonary interstitial emphysema in preterm infants.

European journal of pediatric surgery : official journal of Austrian Association of Pediatric Surgery ... [et al] = Zeitschrift fur Kinderchirurgie, 2008

Professional Medical Disclaimer

This information is intended for healthcare professionals. Any medical decision-making should rely on clinical judgment and independently verified information. The content provided herein does not replace professional discretion and should be considered supplementary to established clinical guidelines. Healthcare providers should verify all information against primary literature and current practice standards before application in patient care. Dr.Oracle assumes no liability for clinical decisions based on this content.

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