Abstract
Background: The purpose of this case report is to illustrate the clinical course of an extremely rare case of Congenital Pulmonary Lymphangiectasia (CPL) presenting as an early pulmonary interstitial emphysema (PIE) complicating respiratory distress syndrome, in an extremely low gestational age neonate (ELGAN) along with a short review of the literature. Case presentation: This 24 weeks ELGAN male with radiologic changes consistent with pulmonary interstitial emphysema (PIE) complicating respiratory distress syndrome which later progressed to bilateral cystic lung changes, expired at 23 days of age. Maternal history was complicated by E. coli urinary tract infection and Group-B Strep chorioamnionitis. The infant remained intubated throughout the hospital course and received antibiotics initially and terminally. His tracheal aspirate cultures also grew Mycoplasma hominis, and Ureaplasma urealyticum, treated with azithromycin. An autopsy revealed diffuse bilateral congenital lymphangiectasia. Postmortem blood and lung tissue cultures were positive for methicillin-resistant Staphylococcus aureus (MRSA). Conclusion: Despite its extreme rarity, the authors recommend considering congenital pulmonary lymphangiectasia in the differential diagnosis, in an extremely preterm neonate, presenting with early pulmonary interstitial emphysema (PIE), and respiratory failure refractory to surfactant, antibiotic therapy, and complex ventilator management.
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