6533b7d5fe1ef96bd1263f29

RESEARCH PRODUCT

Pulmonary Agenesis and Associated Pulmonary Hypertension: A Case Report and Review on Variability, Therapy, and Outcome

Rajamma MathewRosanna G. AbellarDavid M. OtterburnOliver J. Muensterer

subject

medicine.medical_specialtyIntimal hyperplasialcsh:SurgerythoracoscopyArticletissue expanderInternal medicinepulmonary hypertensionmedicineThoracoscopyFavorable outcomepulmonary agenesisTissue expandermedicine.diagnostic_testbusiness.industryPulmonary Agenesislcsh:RJ1-570lcsh:Pediatricslcsh:RD1-811medicine.diseasePulmonary hypertensionSurgeryCardiologyPresentation (obstetrics)businessCongenital disorder

description

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.

yearjournalcountryeditionlanguage
2015-01-08European Journal of Pediatric Surgery Reports
https://doi.org/10.1055/s-0034-1395986