Pulmonary Interstitial Emphysema

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Pulmonary interstitial emphysema (PIE) is a rare, abnormal pathology that occurs more commonly in neonates but can be seen in adults as well. The increased air pressure within the alveoli and alveolar airspaces can disrupt the adjacent lung interstitial tissue, damaging the lung structure and causing linear and cystic spaces that can be complicated with air leaks. The leaked air is collected outside normal air passages and inside the interstitium or bronchovascular complexes. Premature infants with pulmonary interstitial emphysema can develop respiratory distress syndrome. The critical goal is to be able to maintain sufficient gas exchange. Without adequate gas exchange, lungs will be damaged, resulting in prolonged hypoxia, respiratory acidosis, and pulmonary hypoperfusion. PIE is a diagnosis based on imaging and histopathology. Administration of surfactant and high-frequency ventilation has been shown to decrease the incidence of PIE in premature infants. The latest management of infants with respiratory distress syndrome is prophylaxis with synthetic surfactant and continuous positive airway pressure, possibly without mechanical ventilation.

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