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Ruptured abdominal aortic aneurysm repair in pediatric Marfan syndrome patient
Marfan syndrome is a well-described autosomal dominant connective tissue disorder with a constellation of anatomic characteristics including aortic degeneration as a result of the spontaneous mutation of the fibrillin gene, FBN1. Whereas life-threatening dissection and ascending aneurysmal rupture h...
Autores principales: | , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Elsevier
2018
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5849780/ https://www.ncbi.nlm.nih.gov/pubmed/29541693 http://dx.doi.org/10.1016/j.jvscit.2017.11.008 |
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author | Lu, Joyce J. Slaikeu, Jason D. Wong, Peter Y. |
author_facet | Lu, Joyce J. Slaikeu, Jason D. Wong, Peter Y. |
author_sort | Lu, Joyce J. |
collection | PubMed |
description | Marfan syndrome is a well-described autosomal dominant connective tissue disorder with a constellation of anatomic characteristics including aortic degeneration as a result of the spontaneous mutation of the fibrillin gene, FBN1. Whereas life-threatening dissection and ascending aneurysmal rupture have been thoroughly documented in the literature, aneurysms of the abdominal aorta and those present in the pediatric population have only rarely been reported. In this case report, we describe presentation, successful open surgical repair, and recovery of a pediatric Marfan syndrome patient with a ruptured abdominal aortic aneurysm. |
format | Online Article Text |
id | pubmed-5849780 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2018 |
publisher | Elsevier |
record_format | MEDLINE/PubMed |
spelling | pubmed-58497802018-03-14 Ruptured abdominal aortic aneurysm repair in pediatric Marfan syndrome patient Lu, Joyce J. Slaikeu, Jason D. Wong, Peter Y. J Vasc Surg Cases Innov Tech Aortic aneurysm and dissection Marfan syndrome is a well-described autosomal dominant connective tissue disorder with a constellation of anatomic characteristics including aortic degeneration as a result of the spontaneous mutation of the fibrillin gene, FBN1. Whereas life-threatening dissection and ascending aneurysmal rupture have been thoroughly documented in the literature, aneurysms of the abdominal aorta and those present in the pediatric population have only rarely been reported. In this case report, we describe presentation, successful open surgical repair, and recovery of a pediatric Marfan syndrome patient with a ruptured abdominal aortic aneurysm. Elsevier 2018-02-14 /pmc/articles/PMC5849780/ /pubmed/29541693 http://dx.doi.org/10.1016/j.jvscit.2017.11.008 Text en © 2018 The Authors http://creativecommons.org/licenses/by-nc-nd/4.0/ This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/). |
spellingShingle | Aortic aneurysm and dissection Lu, Joyce J. Slaikeu, Jason D. Wong, Peter Y. Ruptured abdominal aortic aneurysm repair in pediatric Marfan syndrome patient |
title | Ruptured abdominal aortic aneurysm repair in pediatric Marfan syndrome patient |
title_full | Ruptured abdominal aortic aneurysm repair in pediatric Marfan syndrome patient |
title_fullStr | Ruptured abdominal aortic aneurysm repair in pediatric Marfan syndrome patient |
title_full_unstemmed | Ruptured abdominal aortic aneurysm repair in pediatric Marfan syndrome patient |
title_short | Ruptured abdominal aortic aneurysm repair in pediatric Marfan syndrome patient |
title_sort | ruptured abdominal aortic aneurysm repair in pediatric marfan syndrome patient |
topic | Aortic aneurysm and dissection |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5849780/ https://www.ncbi.nlm.nih.gov/pubmed/29541693 http://dx.doi.org/10.1016/j.jvscit.2017.11.008 |
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