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European J Pediatr Surg Rep. 2015; 03(01): 033-039
DOI: 10.1055/s-0034-1395986
Case Report
Georg Thieme Verlag KG Stuttgart · New York

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

Oliver Muensterer
1   Department of Pediatric Surgery, University Medicine Mainz, Johannes Gutenberg University, Mainz, Germany
,
Rosanna Abellar
2   Department of Pathology, Columbia University, New York, New York, United States
,
David Otterburn
3   Department of Plastic and Reconstructive Surgery, Weill Cornell Medical College, New York, New York, United States
,
Rajamma Mathew
4   Department of Pediatric Cardiology, New York Medical College, Valhalla, New York, New York, United States
› Author Affiliations
Further Information

Publication History

06 August 2014

02 October 2014

Publication Date:
08 January 2015 (online)

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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.

Keywords

pulmonary agenesis - pulmonary hypertension - tissue expander - thoracoscopy
 

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