C. Rupture of terminal airways distal to termination of fascial sheath
Discussion
This patient has pulmonary interstitial emphysema (PIE) with chest CT
showing extensive interstitial emphysema, diffuse heterogeneous
opacification, and pneumomediastinum. The other answers choices are not
commonly seen in PIE. Abnormal bronchial dilation caused by infection
and inflammation refers to bronchiectasis, altered embryologic airway
branching creates congenital pulmonary airway malformation, and
pulmonary vascular occlusion due to thrombotic events indicates
pulmonary embolism.
PIE occurs in infants when damage to terminal airways results in dissection of air into the surrounding interstitium.2,3,4 This may occur in a single lobe, in multiple lobes, or bilaterally.1,2
It is commonly associated with barotrauma in preterm neonates, but may
also be secondary to surfactant deficiency or interstitial lung disease.2,4,5
When found in patients with interstitial disease, it portends a poor
prognosis with increased risk of pneumothorax and mediastinal emphysema.5
Chest radiographs in PIE typically show cystic and linear radiolucencies.1,3
CT features include hyperexpanded cystic radiolucencies in a
characteristic line-and-dot pattern due to air in the interstitium
surrounding bronchovascular bundles, which appear as lines or punctate
densities depending on the orientation of the vessels.2
Treatment is mainly supportive and often involves minimizing mean
airway pressure to decrease further air leak, sometimes achieved by high
frequency ventilation in neonates.1,4 In localized disease,
decubitis positioning and selective lung intubation may be utilized, and
lobectomy may be required for patients unresponsive to medical
management.1
This patient received mechanical ventilation, including high
frequency oscillatory ventilation, for a total of 13 days. Extensive
infectious work-up revealed PJP and CMV pneumonia, and treatment with
Bactrim, ganciclovir, and steroids was initiated. The infant made an
excellent recovery and was eventually able to be discharged home without
supplemental oxygen. Primary immunodeficiency is suspected but remains
unidentified.
References
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emphysema in a preterm infant in the absence of mechanical ventilation.
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Donnelly LF, Lucaya J, Ozelame V, Frush DP, Strouse PJ, Sumner TE,
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