scholarly journals Urgent Endovascular Treatment of Iliac Artery Pseudoaneurysm in Patient with Ehlers-Danlos Syndrome: A Case Report

2020 ◽  
Vol 8 (C) ◽  
pp. 156-160
Author(s):  
Aleksandar Gjoreski ◽  
Ivona Jovanoska ◽  
Gjorgi Dungevski ◽  
Nikola Lazovski ◽  
Menka Lazareska
Keyword(s):  
Endovascular Treatment ◽  
Iliac Artery ◽  
Significant Risk ◽  
Vascular Complications ◽  
High Morbidity ◽  
Ehlers Danlos Syndrome ◽  
Artery Pseudoaneurysm ◽  
Type Iv ◽  
The Right ◽  
Danlos Syndrome

BACKGROUND: Ehlers-Danlos syndrome (EDS) type IV is a heritable disorder of connective tissue that is mainly associated with vascular maladies such as aneurysms, pseudoaneurysms, and dissections with or without spontaneous rupture. Historically, vascular complications in EDS IV have been treated conservatively whenever possible, due to the high morbidity and mortality after vascular interventions, whether open or endovascular. We present a case of a ruptured pseudoaneurysm of the right common iliac artery in a 18-year-old male, who was successfully treated by endovascular approach and later diagnosed with EDS type IV. CASE PRESENTATION: A 18-year-old male patient was admitted in ER with sharp pain in the right hypogastrium, hypotensive and with reduced blood parameters. Multiphasic modern computed tomography (MDCT) scan of abdomen and pelvis revealed massive ride sided pelvic and retroperitoneal hematoma. The presence of pseudoaneurysms on both common iliac arteries (CIA) was detected, with small ulcer on the right side and a focal dissection on the left side. An urgent endovascular repair of the ruptured pseudoaneurysm on the right CIA with covered stent was performed. Patient’s laboratory parameters and clinical status improved significantly within the next few days. CONCLUSIONS: Vascular repair in EDS-IV patients carries significant risk and should be indicated very carefully. Endovascular treatment for these patients is feasible and should be considered as an alternative to open surgery in some challenging cases as this one.

scholarly journals Pediatric Innominate Artery Pseudoaneurysm Rupture in Vascular Ehlers-Danlos Syndrome: A Case Report

2021 ◽  
Vol 2 (5) ◽  
pp. 226-229
Author(s):  
Aimee Vos ◽  
Katharine Burns
Keyword(s):  
Case Report ◽  
Innominate Artery ◽  
Final Diagnosis ◽  
Connective Tissue Disorder ◽  
High Morbidity ◽  
Ehlers Danlos Syndrome ◽  
Artery Pseudoaneurysm ◽  
Type Iv ◽  
Vascular Type ◽  
Danlos Syndrome

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.

scholarly journals Internal carotid artery dissection in a patient with Ehlers-Danlos syndrome type IV: diagnosis and management

2013 ◽  
Vol 12 (2) ◽  
pp. 174-179 ◽  
Author(s):  
Michel Nasser ◽  
Murilo Bucci Vega ◽  
Luca Giovani Antonio Pivetta ◽  
Ana Izabel Nasser ◽  
Debora Gusmao Melo
Keyword(s):  
Carotid Artery ◽  
Internal Carotid Artery ◽  
Internal Carotid ◽  
Vascular Complications ◽  
Carotid Artery Dissection ◽  
Artery Dissection ◽  
Internal Carotid Artery Dissection ◽  
Ehlers Danlos Syndrome ◽  
Type Iv ◽  
Danlos Syndrome

Ehlers-Danlos syndrome (EDS) type IV, also known as vascular EDS, is an inherited connective tissue disorder with an estimated prevalence of 1/100,000 to 1/250,000. In EDS type IV, vascular complications may affect all anatomical areas, with a preference for large- and medium-sized arteries. Dissections of the vertebral and carotid arteries in their extra- and intra-cranial segments are typical. The authors report the case of a patient with EDS type IV for whom the diagnosis was established based on clinical signs and who developed internal carotid artery dissection at the age of 44 years. In the absence of a specific treatment for EDS type IV, medical interventions should focus on symptomatic relief, prophylactic measures, and genetic counseling. Invasive imaging techniques are contraindicated, and a conservative approach to vascular complications is usually recommended.

Spontaneous Ruptured Dissection of the Right Common Iliac Artery in a Patient with Classic Ehlers–Danlos Syndrome Phenotype

2015 ◽  
Vol 29 (3) ◽  
pp. 595.e11-595.e14 ◽  
Author(s):  
Rick Gaines ◽  
Brad T. Tinkle ◽  
Pegge M. Halandras ◽  
Omar Al-Nouri ◽  
Paul Crisostomo ◽  
...  
Keyword(s):  
Iliac Artery ◽  
Common Iliac Artery ◽  
Ehlers Danlos Syndrome ◽  
The Right ◽  
Danlos Syndrome

scholarly journals Vascular complications in Ehlers-Danlos syndrome type IV — case report

2015 ◽  
Vol 21 (2) ◽  
pp. 61-65
Author(s):  
Katarzyna Grygiel ◽  
Maciej Jedrasik ◽  
Hamid Feiz Allah Poor ◽  
Slawomir Nazarewski ◽  
Jacek Szmidt
Keyword(s):  
Case Report ◽  
Vascular Complications ◽  
Syndrome Type ◽  
Ehlers Danlos Syndrome ◽  
Type Iv ◽  
Danlos Syndrome

Acute brain ischemia as a complication of the Ehlers–Danlos syndrome, the case series

Vascular ◽  
2013 ◽  
Vol 22 (5) ◽  
pp. 341-345 ◽  
Author(s):  
Michal Pajak ◽  
Marcin A Majos ◽  
Wojciech Szubert ◽  
Ludomir Stefanczyk ◽  
Agata Majos
Keyword(s):  
Brain Ischemia ◽  
Case Series ◽  
Vascular Complications ◽  
Limb Ischemia ◽  
Radiology Department ◽  
Artery Dissection ◽  
Ehlers Danlos Syndrome ◽  
Type Iv ◽  
Vascular Type ◽  
Danlos Syndrome

Vascular type of Ehlers–Danlos syndrome involves many severe complications leading not only to organ-specific symptoms but often ends in a sudden death. The aim of this paper was to present a diagnostic possibilities and its efficiency rate in patients with vascular complications of Ehlers–Danlos syndrome who suffered from artery dissection resulting in acute brain or limb ischemia. We analysed three patients with diagnosed Ehlers–Danlos syndrome who were referred to radiology department for diagnostic imaging of affected vascular beds, each experienced brain ischemia. The paper also aims at offering some general recommendations for patients suffering from possible complications of type IV Ehlers–Danlos syndrome basing on our own experience and available literature data.

scholarly journals Subclavian artery pseudoaneurysm in type IV Ehlers-Danlos Syndrome

1998 ◽  
Vol 27 (3) ◽  
pp. 549-551 ◽  
Author(s):  
Peter I. Rossi ◽  
Larry A. Scher ◽  
Steven G. Friedman ◽  
Michae H. Hall ◽  
Robert A. Boxer ◽  
...  
Keyword(s):  
Subclavian Artery ◽  
Ehlers Danlos Syndrome ◽  
Artery Pseudoaneurysm ◽  
Type Iv ◽  
Danlos Syndrome

scholarly journals Asymptomatic bilateral common iliac artery dissections in previously undiagnosed vascular Ehlers-Danlos syndrome

2019 ◽  
Vol 12 (11) ◽  
pp. e231537
Author(s):  
Matthew Lavoie ◽  
Jason Reese
Keyword(s):  
Iliac Artery ◽  
Common Iliac Artery ◽  
Clinical History ◽  
Artery Dissection ◽  
Intravenous Contrast ◽  
Ehlers Danlos Syndrome ◽  
Left Common Iliac Artery ◽  
Ct Angiogram ◽  
The Right ◽  
Danlos Syndrome

We report a case of a 35-year-old woman found to have vascular Ehlers-Danlos syndrome (vEDS) after family history of sudden death due to aortic dissection in her otherwise healthy brother prompted further imaging workup and consideration of an underlying heritable genetic condition. CT angiogram of the aorta with intravenous contrast revealed an abdominal aortic artery dissection below the level of the renal arteries extending from the bifurcation into the left common iliac artery with an additional focal dissection of the right common iliac artery. To the author’s knowledge, this is the first report of asymptomatic bilateral common iliac artery dissections as a part of the initial presentation of a patient with underlying vEDS. Additionally, this case highlights the importance of familial diagnostic screening in inherited vasculopathies. Clinical history, genetic testing and management are discussed.

Sign in / Sign up

Export Citation Format

Share Document