Pulmonary agenesis is a rare congenital disorder with large variability in presentation and prognosis. We describe a full-term infant born with right-sided pulmonary agenesis who underwent thoracoscopic placement of a tissue expander. He ultimately died of pulmonary hypertension. Immunohistology showed intimal hyperplasia without the loss of endothelial caveolin-1 expression. A literature review revealed that while some of these patients have favorable outcome, many succumb despite therapy.
CITATION STYLE
Muensterer, O., Abellar, R., Otterburn, D., & Mathew, R. (2015). Pulmonary Agenesis and Associated Pulmonary Hypertension: A Case Report and Review on Variability, Therapy, and Outcome. European Journal of Pediatric Surgery Reports, 03(01), 033–039. https://doi.org/10.1055/s-0034-1395986
Mendeley helps you to discover research relevant for your work.