title: Pulmonary Agenesis and Associated Pulmonary Hypertension: A Case Report and Review on Variability, Therapy, and Outcome.
abstract: 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.
authors.affiliation: Department of Pediatric Surgery, University Medicine Mainz, Johannes Gutenberg University, Mainz, Germany.
authors.forename: Oliver
authors.initials: O
authors.lastname: Muensterer
authors.affiliation: Department of Pathology, Columbia University, New York, New York, United States.
authors.forename: Rosanna
authors.initials: R
authors.lastname: Abellar
authors.affiliation: Department of Plastic and Reconstructive Surgery, Weill Cornell Medical College, New York, New York, United States.
authors.forename: David
authors.initials: D
authors.lastname: Otterburn
authors.affiliation: Department of Pediatric Cardiology, New York Medical College, Valhalla, New York, New York, United States.
authors.forename: Rajamma
authors.initials: R
authors.lastname: Mathew
keywords: pulmonary agenesis; pulmonary hypertension; thoracoscopy; tissue expander