What is the prognosis of pulmonary interstitial emphysema (PIE)?

Updated: Jun 25, 2019
  • Author: Abhay J Bhatt, MD, MBBS; Chief Editor: Muhammad Aslam, MD  more...
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Pulmonary interstitial emphysema (PIE) can predispose an infant to other air leaks. In a study by Greenough et al, 31 of 41 infants with PIE developed pneumothorax, compared to 41 of 169 infants without PIE. [8] In addition, 21 of 41 babies with PIE developed intraventricular hemorrhage (IVH), compared to 39 of 169 among infants without PIE.

PIE may not resolve for 2-3 weeks; therefore, it can increase the duration of mechanical ventilation support as well as the incidence of bronchopulmonary dysplasia (BPD). Some infants may develop chronic lobar emphysema, which may require surgical lobectomies. [19]

In a study in the postsurfactant era, 4 of 11 infants with PIE developed severe IVH (grade 2 or higher) compared to 4 of 34 infants without PIE. Additionally, PIE remained significantly associated with death (odds ratio, 14.4; 95% confidence interval, 1-208; P = 0.05). [18]

Long-term follow-up data are scarce. Gaylord et al demonstrated a high (54%) incidence of chronic lung disease in survivors of PIE compared to their nursery's overall incidence of 32%. [20]  In addition, 19% of the infants with PIE developed chronic lobar emphysema; of these babies, 50% received surgical lobectomies.

The mortality rate associated with PIE is reported to be as high as 53-67%. [20, 25] Lower mortality rates of 24% and 38% reported in other studies could result from differences in population selection. [8, 11] Morisot et al reported an 80% mortality rate with PIE in infants with a birth weight of fewer than 1600 g and severe respiratory distress syndrome. [28]

The early appearance of PIE (< 48 h after birth) is associated with increased mortality. However, this may reflect the severity of the underlying parenchymal disease. [11, 28]

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