Pediatric Innominate Artery Pseudoaneurysm Rupture in Vascular Ehlers-Danlos Syndrome: A Case Report
Introduction: Ehlers-Danlos syndrome is a well classified connective tissue disorder recognized by its features of hyperextensibility of joints and hyperelasticity of the skin. However, the rare vascular type (Ehlers-Danlos type IV) is more difficult to identify in the absence, rarity, or subtlety o...
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2021-05-01
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doaj-6acd7e432cd94e1e8c2aeee79589ea882021-05-24T21:03:03ZengeScholarship Publishing, University of CaliforniaClinical Practice and Cases in Emergency Medicine2474-252X2021-05-015210.5811/cpcem.2021.3.51787cpcem-05-226Pediatric Innominate Artery Pseudoaneurysm Rupture in Vascular Ehlers-Danlos Syndrome: A Case ReportAimee VosKatharine M. BurnsIntroduction: Ehlers-Danlos syndrome is a well classified connective tissue disorder recognized by its features of hyperextensibility of joints and hyperelasticity of the skin. However, the rare vascular type (Ehlers-Danlos type IV) is more difficult to identify in the absence, rarity, or subtlety of the classical physical features. Patients presenting to the emergency department (ED) with acute complications of vascular Ehlers-Danlos syndrome may be critically ill, requiring accurate diagnosis and tailored management. Case Report: This report details a case of spontaneous innominate artery pseudoaneurysm rupture in a pediatric patient with previously undiagnosed Ehlers-Danlos syndrome. Initial ED evaluation was followed by urgent operative intervention and subsequent genetic testing to confirm final diagnosis. Conclusion: Due to its high morbidity and mortality, vascular type Ehlers-Danlos syndrome should be considered in the differential for otherwise unexplained spontaneous vascular injury.https://escholarship.org/uc/item/2q32s86v |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Aimee Vos Katharine M. Burns |
spellingShingle |
Aimee Vos Katharine M. Burns Pediatric Innominate Artery Pseudoaneurysm Rupture in Vascular Ehlers-Danlos Syndrome: A Case Report Clinical Practice and Cases in Emergency Medicine |
author_facet |
Aimee Vos Katharine M. Burns |
author_sort |
Aimee Vos |
title |
Pediatric Innominate Artery Pseudoaneurysm Rupture in Vascular Ehlers-Danlos Syndrome: A Case Report |
title_short |
Pediatric Innominate Artery Pseudoaneurysm Rupture in Vascular Ehlers-Danlos Syndrome: A Case Report |
title_full |
Pediatric Innominate Artery Pseudoaneurysm Rupture in Vascular Ehlers-Danlos Syndrome: A Case Report |
title_fullStr |
Pediatric Innominate Artery Pseudoaneurysm Rupture in Vascular Ehlers-Danlos Syndrome: A Case Report |
title_full_unstemmed |
Pediatric Innominate Artery Pseudoaneurysm Rupture in Vascular Ehlers-Danlos Syndrome: A Case Report |
title_sort |
pediatric innominate artery pseudoaneurysm rupture in vascular ehlers-danlos syndrome: a case report |
publisher |
eScholarship Publishing, University of California |
series |
Clinical Practice and Cases in Emergency Medicine |
issn |
2474-252X |
publishDate |
2021-05-01 |
description |
Introduction: Ehlers-Danlos syndrome is a well classified connective tissue disorder recognized by its features of hyperextensibility of joints and hyperelasticity of the skin. However, the rare vascular type (Ehlers-Danlos type IV) is more difficult to identify in the absence, rarity, or subtlety of the classical physical features. Patients presenting to the emergency department (ED) with acute complications of vascular Ehlers-Danlos syndrome may be critically ill, requiring accurate diagnosis and tailored management. Case Report: This report details a case of spontaneous innominate artery pseudoaneurysm rupture in a pediatric patient with previously undiagnosed Ehlers-Danlos syndrome. Initial ED evaluation was followed by urgent operative intervention and subsequent genetic testing to confirm final diagnosis. Conclusion: Due to its high morbidity and mortality, vascular type Ehlers-Danlos syndrome should be considered in the differential for otherwise unexplained spontaneous vascular injury. |
url |
https://escholarship.org/uc/item/2q32s86v |
work_keys_str_mv |
AT aimeevos pediatricinnominatearterypseudoaneurysmruptureinvascularehlersdanlossyndromeacasereport AT katharinemburns pediatricinnominatearterypseudoaneurysmruptureinvascularehlersdanlossyndromeacasereport |
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1721428221269901312 |