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 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 Successful coil embolization for rupture of the subclavian artery associated with Ehlers-Danlos syndrome type IV

2009 ◽  
Vol 50 (5) ◽  
pp. 1191-1195 ◽  
Author(s):  
Yasunori Iida ◽  
Yukio Obitsu ◽  
Hiroyoshi Komai ◽  
Hiroshi Shigematsu
Keyword(s):  
Subclavian Artery ◽  
Coil Embolization ◽  
Syndrome Type ◽  
Ehlers Danlos Syndrome ◽  
Type Iv ◽  
Danlos Syndrome

Endovascular repair of a ruptured subclavian artery aneurysm in a patient with Ehlers–Danlos syndrome using a sandwich technique

Vascular ◽  
2014 ◽  
Vol 22 (5) ◽  
pp. 371-374 ◽  
Author(s):  
Sapan S Desai ◽  
Maria Codreanu ◽  
Kristofer M Charlton-Ouw ◽  
Hazim Safi ◽  
Ali Azizzadeh
Keyword(s):  
Stent Graft ◽  
Subclavian Artery ◽  
Artery Aneurysm ◽  
Innominate Artery ◽  
Ehlers Danlos Syndrome ◽  
Sandwich Technique ◽  
Type Iv ◽  
Subclavian Artery Aneurysm ◽  
The Right ◽  
Danlos Syndrome

We present the case of a type IV Ehlers–Danlos syndrome patient with a ruptured right subclavian artery aneurysm and associated arteriovenous fistula who underwent successful endovascular repair requiring simultaneous stent graft repair of the innominate artery using a sandwich technique. A 17-year-old man with known type IV Ehlers–Danlos syndrome developed right neck and shoulder swelling. CTA study demonstrated a 17 × 13-cm ruptured subclavian artery aneurysm with an associated internal jugular vein arteriovenous fistula. In the hybrid suite, a 7 mm × 15-cm stent graft (Viabahn, WL Gore & Associates, Flagstaff, AZ) was advanced from the right brachial approach into the innominate artery. A separate wire was placed into the right carotid artery via the right femoral approach (7 Fr), and a 7 mm × 10-cm stent graft (Viabahn) was advanced into the innominate artery. An additional 8 mm × 10-cm stent graft (Viabahn) was placed from the right brachial approach to obtain a distal-landing zone in the axillary artery. Complex vascular anatomy, in which graft seal creation may be challenging, does not exclude endovascular approaches as the sandwich technique can be utilized as a suitable alternative to open repair.

scholarly journals Multiexon deletion in the procollagen III gene is associated with mild Ehlers-Danlos syndrome type IV.

1991 ◽  
Vol 266 (8) ◽  
pp. 5244-5248
Author(s):  
H Vissing ◽  
M D'Alessio ◽  
B Lee ◽  
F Ramirez ◽  
P H Byers ◽  
...  
Keyword(s):  
Syndrome Type ◽  
Ehlers Danlos Syndrome ◽  
Type Iv ◽  
Danlos Syndrome ◽  
Procollagen Iii

scholarly journals Hereditary Connective Tissue Diseases in Young Adult Stroke: A Comprehensive Synthesis

2011 ◽  
Vol 2011 ◽  
pp. 1-18 ◽  
Author(s):  
Olivier M. Vanakker ◽  
Dimitri Hemelsoet ◽  
Anne De Paepe
Keyword(s):  
Connective Tissue ◽  
Metabolic Disorders ◽  
Type Iv Collagen ◽  
Connective Tissue Diseases ◽  
Haemorrhagic Stroke ◽  
Monogenic Disease ◽  
Connective Tissue Disorders ◽  
Ehlers Danlos Syndrome ◽  
Type Iv ◽  
Danlos Syndrome

Though the genetic background of ischaemic and haemorrhagic stroke is often polygenetic or multifactorial, it can in some cases result from a monogenic disease, particularly in young adults. Besides arteriopathies and metabolic disorders, several connective tissue diseases can present with stroke. While some of these diseases have been recognized for decades as causes of stroke, such as the vascular Ehlers-Danlos syndrome, others only recently came to attention as being involved in stroke pathogenesis, such as those related to Type IV collagen. This paper discusses each of these connective tissue disorders and their relation with stroke briefly, emphasizing the main clinical features which can lead to their diagnosis.

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