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Recommendations for anesthesia and perioperative management in patients with Ehlers-Danlos syndrome(s)
Ehlers-Danlos syndrome (EDS, ORPHA98249) comprises a group of clinically and genetically heterogeneous heritable connective tissue disorders, chiefly characterized by joint hypermobility and instability, skin texture anomalies, and vascular and soft tissue fragility. As many tissues can be involved,...
Autores principales: | , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
BioMed Central
2014
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4223622/ https://www.ncbi.nlm.nih.gov/pubmed/25053156 http://dx.doi.org/10.1186/s13023-014-0109-5 |
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author | Wiesmann, Thomas Castori, Marco Malfait, Fransiska Wulf, Hinnerk |
author_facet | Wiesmann, Thomas Castori, Marco Malfait, Fransiska Wulf, Hinnerk |
author_sort | Wiesmann, Thomas |
collection | PubMed |
description | Ehlers-Danlos syndrome (EDS, ORPHA98249) comprises a group of clinically and genetically heterogeneous heritable connective tissue disorders, chiefly characterized by joint hypermobility and instability, skin texture anomalies, and vascular and soft tissue fragility. As many tissues can be involved, the underlying molecular defect can manifest itself in many organs and with varying degrees of severity, with widespread implications for anesthesia and perioperative management. This review focuses on issues relevant for anesthesia for elective and emergency surgery in EDS. We searched the literature for papers related to all EDS variants; at the moment most of the published data deals with the vascular subtype and, to a lesser extent, classic and hypermobility EDS. Knowledge is fragmented and consists mostly of case reports, small case series and expert opinion. Because EDS patients commonly require surgery, we have summarized some recommendations for general, obstetrical and regional anesthesia, as well as for hemostatic therapy. |
format | Online Article Text |
id | pubmed-4223622 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2014 |
publisher | BioMed Central |
record_format | MEDLINE/PubMed |
spelling | pubmed-42236222014-11-08 Recommendations for anesthesia and perioperative management in patients with Ehlers-Danlos syndrome(s) Wiesmann, Thomas Castori, Marco Malfait, Fransiska Wulf, Hinnerk Orphanet J Rare Dis Review Ehlers-Danlos syndrome (EDS, ORPHA98249) comprises a group of clinically and genetically heterogeneous heritable connective tissue disorders, chiefly characterized by joint hypermobility and instability, skin texture anomalies, and vascular and soft tissue fragility. As many tissues can be involved, the underlying molecular defect can manifest itself in many organs and with varying degrees of severity, with widespread implications for anesthesia and perioperative management. This review focuses on issues relevant for anesthesia for elective and emergency surgery in EDS. We searched the literature for papers related to all EDS variants; at the moment most of the published data deals with the vascular subtype and, to a lesser extent, classic and hypermobility EDS. Knowledge is fragmented and consists mostly of case reports, small case series and expert opinion. Because EDS patients commonly require surgery, we have summarized some recommendations for general, obstetrical and regional anesthesia, as well as for hemostatic therapy. BioMed Central 2014-07-23 /pmc/articles/PMC4223622/ /pubmed/25053156 http://dx.doi.org/10.1186/s13023-014-0109-5 Text en Copyright © 2014 Wiesmann et al. ; licensee Biomedcentral Ltd http://creativecommons.org/licenses/by/4.0 This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly credited. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated. |
spellingShingle | Review Wiesmann, Thomas Castori, Marco Malfait, Fransiska Wulf, Hinnerk Recommendations for anesthesia and perioperative management in patients with Ehlers-Danlos syndrome(s) |
title | Recommendations for anesthesia and perioperative management in patients with Ehlers-Danlos syndrome(s) |
title_full | Recommendations for anesthesia and perioperative management in patients with Ehlers-Danlos syndrome(s) |
title_fullStr | Recommendations for anesthesia and perioperative management in patients with Ehlers-Danlos syndrome(s) |
title_full_unstemmed | Recommendations for anesthesia and perioperative management in patients with Ehlers-Danlos syndrome(s) |
title_short | Recommendations for anesthesia and perioperative management in patients with Ehlers-Danlos syndrome(s) |
title_sort | recommendations for anesthesia and perioperative management in patients with ehlers-danlos syndrome(s) |
topic | Review |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4223622/ https://www.ncbi.nlm.nih.gov/pubmed/25053156 http://dx.doi.org/10.1186/s13023-014-0109-5 |
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