Innominate Artery Involvement in Type IV Ehlers-Danlos Syndrome

1991 ◽  
Vol 5 (1) ◽  
pp. 41-45 ◽  
Author(s):  
Alain Valverde ◽  
Jean-François Tricot ◽  
Bruno de Crepy ◽  
Hayssam Bakdach ◽  
Koskrow Djabbari
Keyword(s):  
Innominate Artery ◽  
Ehlers Danlos Syndrome ◽  
Type Iv ◽  
Danlos Syndrome

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.

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

Ehlers-Danlos syndrome type IV: Keloidal plaques of the lower extremities, amniotic band limb deformity, and a new mutation

2007 ◽  
Vol 56 (2) ◽  
pp. S53-S54 ◽  
Author(s):  
Cynthia J. Burk ◽  
Cheryl Aber ◽  
Elizabeth Alvarez Connelly
Keyword(s):  
Lower Extremities ◽  
Syndrome Type ◽  
New Mutation ◽  
Ehlers Danlos Syndrome ◽  
Limb Deformity ◽  
Amniotic Band ◽  
Type Iv ◽  
Danlos Syndrome

scholarly journals Spontaneous rupture of the renal artery in a patient with ehlers-danlos syndrome type IV

1997 ◽  
Vol 13 (5) ◽  
pp. 509-512 ◽  
Author(s):  
O. Øyen ◽  
O.P. Clausen ◽  
I.B. Brekke ◽  
A. Bakka ◽  
F.M. Pope
Keyword(s):  
Renal Artery ◽  
Spontaneous Rupture ◽  
Syndrome Type ◽  
Ehlers Danlos Syndrome ◽  
Type Iv ◽  
Danlos Syndrome

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.

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.

Cerebrovascular complications in Ehlers-Danlos syndrome type IV

1995 ◽  
Vol 38 (6) ◽  
pp. 960-964 ◽  
Author(s):  
Kathryn N. North ◽  
David A. H. Whiteman ◽  
Melanie G. Pepin ◽  
Peter H. Byers
Keyword(s):  
Syndrome Type ◽  
Ehlers Danlos Syndrome ◽  
Cerebrovascular Complications ◽  
Type Iv ◽  
Danlos Syndrome
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