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 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.

THE BILATERAL ULNAR ARTERY INVOLVEMENT IN TYPE IV EHLERS-DANLOS SYNDROME — A CASE REPORT

Hand Surgery ◽  
2012 ◽  
Vol 17 (02) ◽  
pp. 213-216 ◽  
Author(s):  
Kazuo Ikeda ◽  
Naoki Osamura ◽  
Satomi Kasashima
Keyword(s):  
Case Report ◽  
Ulnar Artery ◽  
Ehlers Danlos Syndrome ◽  
Type Iv ◽  
First Case ◽  
Vascular Type ◽  
The World ◽  
Forearm Compartment Syndrome ◽  
Danlos Syndrome ◽  
Hereditary Condition

Ehlers-Danlos syndrome Type IV, the vascular type, is a rare hereditary condition. Major arteries are often affected in this type. In this paper, we present the first case report in the world in which forearm compartment syndrome caused by the bilateral ulnar artery rupture in this type. Successful treatment such as incision of the forearm fascia and ligation of the ulnar artery was performed.

Early and Late Outcomes of Cardiovascular Surgery in Patients With Ehlers-Danlos Syndrome

2021 ◽  
Vol 12 (6) ◽  
pp. 773-777
Author(s):  
Mohamed F. Elsisy ◽  
Alberto Pochettino ◽  
Joseph A. Dearani ◽  
Thomas C. Bower ◽  
Robert D. McBane ◽  
...  
Keyword(s):  
Surgical Outcomes ◽  
Time Frame ◽  
Ehlers Danlos Syndrome ◽  
Vital Status ◽  
Type Iv ◽  
Vascular Type ◽  
Baseline Characteristics ◽  
Danlos Syndrome ◽  
Adult Cardiac Surgery

Background Cardiovascular surgical outcomes reports are few for vascular type IV of Ehlers- Danlos Syndrome (vEDS) compared to non-vascular types I-III (nEDS). Methods To define cardiovascular surgical outcomes among adult patients (≥18 years) with EDS types, a review of our institution's in-house STS Adult Cardiac Surgery Database-compliant software and electronic medical records from Mayo Clinic (1993–2019) was performed. Outcomes were compared for vEDS patients and nEDS patients. Demographics, baseline characteristics, operative, in-hospital complications and follow-up vital status were analyzed. Results Over the study time frame, 48 EDS patients underwent surgery (mean age 52.6 ± 14.6 years; 48% females). Of these, 17 patients had vEDS and 31 patients had nEDS. Six patients (12.5%) underwent prior sternotomy. Urgent or emergent surgery was performed in 10 patients (20.8%). Aortic (vEDS 76.5% vs. nEDS 16.1%) and mitral procedures (vEDS 11.8% vs. nEDS 48.4%) were the two most common cardiovascular surgeries performed (p < .01 and p = .007, respectively). Cardiopulmonary bypass time (CPB) (165 ± 18 vs. 90 ± 13 min; p = .015) and aortic cross clamp times (140 ± 14 vs. 62 ± 10 min; p < .001) were longer for vEDS patients. There was 1 (2.1%) early and 7 (14.6%) late deaths; 6 among vEDS and 2 among nEDS patients. Survival at 5 (80% vs. 93%), 10 (45% vs. 84%) and 15 years (45% vs. 84%) was lower in patients with vEDS (p = .015 for each comparison). Conclusion Cardiovascular surgeries are significantly more complex with longer bypass and cross clamp times for type IV vEDS compared to nEDS patients. Reduced overall survival underscores the complexity and fragility of vEDS patients.

scholarly journals Vascular-type Ehlers-Danlos syndrome caused by a hitherto unknown genetic mutation: a case report

2013 ◽  
Vol 7 (1) ◽  
Author(s):  
Fumihiro Kashizaki ◽  
Atsushi Hatamochi ◽  
Kazunori Kamiya ◽  
Akira Yoshizu ◽  
Hiroaki Okamoto
Keyword(s):  
Case Report ◽  
Genetic Mutation ◽  
Ehlers Danlos Syndrome ◽  
Vascular Type ◽  
Danlos Syndrome

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 Carotid cavernous fistula in a patient with Ehlers-Danlos syndrome type IV: a case report

2013 ◽  
Vol 7 (2) ◽  
pp. 94-100
Author(s):  
Katsunori ASAI ◽  
Shingo TOYOTA ◽  
Kouichi HAYAKAWA ◽  
Yasunori FUJIMOTO ◽  
Fuminori IWAMOTO ◽  
...  
Keyword(s):  
Case Report ◽  
Syndrome Type ◽  
Carotid Cavernous Fistula ◽  
Ehlers Danlos Syndrome ◽  
Cavernous Fistula ◽  
Type Iv ◽  
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
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