PIE vs. Cystic BPD

[abeluchin]

How do you differentiate PIE vs. early cystic BPD?

A former 25week preemie developed cystic lung lesions as evidence of chest x-rays at 2weeks of age. The infant was born through maternal chorio, no NAS given, developed RDS, required two doses of survanta and antibiotics started for suspected sepsis, completed 7 days. Initial Hct was 19, infant received multiple PRBS transfusions, but developed pressor-resistant hypotension requiring Dopa/Dobutamine/Epinephrine/Hydrocortisone. A left grade 4 IVH and right grade IVH was noted on DOL2, stable on follow ups. Pt remained on CMV, moderate settings until DOL7 that was switched to HFOC due to requiring significant peak pressures on CMV and higher FiO2 requirments. A BCx was repeated(grew staph epi) and broad-spectrum antibiotics started (vancomycin and gentamicin). A repeat ECHO showed a large PDA with left-right shunt and left atrial enlargement. Pt was treated with Indomethacine with resolution of the PDA. DOL7-10, Pt developed hypotension requiring dopamine and hydrocortisone, and worsening lung compliance requiring higher means on HFOV to maintain lung inflation. FiO2 requirement was 80 to 100% throughout. Amphotericin B was added in the mist of the set-back to due concerns with fungal infection. On DOL12 lungs were noted hyperexpanded, unable to wean mean airway pressure due to profound desaturations wih every attempt. Pt on heavy sedation and paralysis at this point. On DOL13 bubbly lucencies were noted on x-ray. No pneumothorax seen.

1. My best guess is that this is stage 3 of the old BPD, given late presentation of the bubbly lusencies, non-association with pneumothorax or other air leak problems and very strong predisposition to severe BPD, such as ELGA, maternal chorio, no ANS, PDA, sepsis and mechanical ventilation.

2. PIE is a probably diagnosis, but I've seen PIE presenting earlier (particularly in the first 1-2 days of life), almost always associated with pneumothorax, in babies with hypoplastic lungs, mec aspiration or uneven surfactant distribution and severe RDS.

I do not think we can differentiate radiologically PIE from early cystic BPD, so that we cannot make a radiologic distinction between SIP and early NEC with perforation.

I would like to hear your opinions on how you differentiate these two entities in clinical practice? Do you usually make a distinction between the two? or you think they are a continuous of the same disease process?

Thanks in advance for you inputs

Edited March 15, 2012 by abeluchin