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. Immunohist...
| Main Authors: | , , , |
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| Format: | Article |
| Language: | English |
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Georg Thieme Verlag KG
2015-01-01
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| Series: | European Journal of Pediatric Surgery Reports |
| Subjects: | |
| Online Access: | http://www.thieme-connect.de/DOI/DOI?10.1055/s-0034-1395986 |
| _version_ | 1818175456982073344 |
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| author | Oliver Muensterer Rosanna Abellar David Otterburn Rajamma Mathew |
| author_facet | Oliver Muensterer Rosanna Abellar David Otterburn Rajamma Mathew |
| author_sort | Oliver Muensterer |
| collection | DOAJ |
| 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. |
| first_indexed | 2024-12-11T20:00:36Z |
| format | Article |
| id | doaj.art-d023adb9e9c64819a35a60c7cc1cf573 |
| institution | Directory Open Access Journal |
| issn | 2194-7619 2194-7627 |
| language | English |
| last_indexed | 2024-12-11T20:00:36Z |
| publishDate | 2015-01-01 |
| publisher | Georg Thieme Verlag KG |
| record_format | Article |
| series | European Journal of Pediatric Surgery Reports |
| spelling | doaj.art-d023adb9e9c64819a35a60c7cc1cf5732022-12-22T00:52:32ZengGeorg Thieme Verlag KGEuropean Journal of Pediatric Surgery Reports2194-76192194-76272015-01-01030103303910.1055/s-0034-1395986Pulmonary Agenesis and Associated Pulmonary Hypertension: A Case Report and Review on Variability, Therapy, and OutcomeOliver Muensterer0Rosanna Abellar1David Otterburn2Rajamma Mathew3Department of Pediatric Surgery, University Medicine Mainz, Johannes Gutenberg University, Mainz, GermanyDepartment of Pathology, Columbia University, New York, New York, United StatesDepartment of Plastic and Reconstructive Surgery, Weill Cornell Medical College, New York, New York, United StatesDepartment of Pediatric Cardiology, New York Medical College, Valhalla, New York, New York, United StatesAbstract 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.http://www.thieme-connect.de/DOI/DOI?10.1055/s-0034-1395986pulmonary agenesispulmonary hypertensiontissue expanderthoracoscopy |
| spellingShingle | Oliver Muensterer Rosanna Abellar David Otterburn Rajamma Mathew Pulmonary Agenesis and Associated Pulmonary Hypertension: A Case Report and Review on Variability, Therapy, and Outcome European Journal of Pediatric Surgery Reports pulmonary agenesis pulmonary hypertension tissue expander thoracoscopy |
| title | Pulmonary Agenesis and Associated Pulmonary Hypertension: A Case Report and Review on Variability, Therapy, and Outcome |
| title_full | Pulmonary Agenesis and Associated Pulmonary Hypertension: A Case Report and Review on Variability, Therapy, and Outcome |
| title_fullStr | Pulmonary Agenesis and Associated Pulmonary Hypertension: A Case Report and Review on Variability, Therapy, and Outcome |
| title_full_unstemmed | Pulmonary Agenesis and Associated Pulmonary Hypertension: A Case Report and Review on Variability, Therapy, and Outcome |
| title_short | Pulmonary Agenesis and Associated Pulmonary Hypertension: A Case Report and Review on Variability, Therapy, and Outcome |
| title_sort | pulmonary agenesis and associated pulmonary hypertension a case report and review on variability therapy and outcome |
| topic | pulmonary agenesis pulmonary hypertension tissue expander thoracoscopy |
| url | http://www.thieme-connect.de/DOI/DOI?10.1055/s-0034-1395986 |
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