Persistent pulmonary interstitial emphysema

Case contributed by Dr Jeremy Jones

Presentation

Born at 32/40. Required 2 days of CPAP. Never ventillated. Discharged on day 3. Readmitted with progressive increase in work of breathing.

Patient Data

Age: 4 weeks
Gender: Male
X-ray

Day 0

see below

X-ray

Day 30

The left lung is hyperexpanded with multiple cyst-like lucencies throughout the hemithorax. The diaphragm is clearly visualised.

The CT confirms the expansion of the left lung by cyst-like lucencies, but the bronchovascular bundle traverses the cysts rather than being between them. Features here are of gas within the bronchial interstitium, PIE.

Case Discussion

Features here are most in keeping with pulmonary interstitial emphysema. Other differentials include:

The lobe was removed and the pathologist confirmed persistent pulmonary interstitial emphysema.

PIE in the neonatal setting is usually seen in premature infants who have been ventilated. Their stiff lungs (in the setting of RDS) predispose them to alveolar or interstital rupture leading to dissection of gas along the bronchial interstitium.

This case highlights that PIE can occur unilaterally and asymmetrically, and does not only occur in a preterm ventillated neonate.

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Case information

rID: 27281
Case created: 26th Jan 2014
Last edited: 12th Nov 2017
Systems: Chest, Paediatrics
Inclusion in quiz mode: Included

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