Successful endovascular treatment of delayed arterial rupture from celiac artery dissection in a patient with type IV Ehlers-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.

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Bilateral Vertebral Artery Dissection and Unilateral Carotid Artery Dissection in Case of Ehlers-Danlos Syndrome Type IV

World Neurosurgery ◽

10.1016/j.wneu.2018.09.236 ◽

2019 ◽

Vol 121 ◽

pp. 83-87 ◽

Cited By ~ 2

Author(s):

Tomotaka Ohshima ◽

Shigeru Miyachi ◽

Taiki Isaji ◽

Naoki Matsuo ◽

Reo Kawaguchi ◽

...

Keyword(s):

Carotid Artery ◽

Vertebral Artery ◽

Vertebral Artery Dissection ◽

Carotid Artery Dissection ◽

Artery Dissection ◽

Syndrome Type ◽

Ehlers Danlos Syndrome ◽

Type Iv ◽

Danlos Syndrome

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

Open Access Macedonian Journal of Medical Sciences ◽

10.3889/oamjms.2020.5348 ◽

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.

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Successful stent placement for cervical artery dissection associated with the Ehlers—Danlos syndrome

Journal of Neurosurgery ◽

10.3171/jns.2003.99.6.1077 ◽

2003 ◽

Vol 99 (6) ◽

pp. 1077-1081 ◽

Cited By ~ 18

Author(s):

Akira Kurata ◽

Hidehiro Oka ◽

Taketomo Ohmomo ◽

Hitoshi Ozawa ◽

Sachio Suzuki ◽

...

Keyword(s):

Clinical Course ◽

Dissecting Aneurysm ◽

Artery Dissection ◽

Cervical Artery Dissection ◽

Severe Stenosis ◽

Ehlers Danlos Syndrome ◽

Content Type ◽

Type Iv ◽

The Common ◽

Danlos Syndrome

✓ This 44-year-old man with Ehlers—Danlos syndrome (EDS) Type IV presented with hemiparesis and the Gerstmann syndrome. Left carotid artery (CA) angiography revealed a dissecting aneurysm with severe stenosis located in the common CA; the lesion was successfully treated with a stent graft. The patient's clinical course after endovascular surgery was uneventful, without occurrence of megacolon. The literature for spontaneous CA dissection in EDS Type IV cases is reviewed and points for investigation and treatment are discussed.

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Spontaneous carotid-cavernous fistulae in Ehlers-Danlos syndrome Type IV

Journal of Neurosurgery ◽

10.3171/jns.1991.74.6.0991 ◽

1991 ◽

Vol 74 (6) ◽

pp. 991-998 ◽

Cited By ~ 60

Author(s):

Wouter I. Schievink ◽

David G. Piepgras ◽

Franklin Earnest ◽

Hymie Gordon

Keyword(s):

Early Recognition ◽

Artery Dissection ◽

Cervical Artery Dissection ◽

Syndrome Type ◽

Ehlers Danlos Syndrome ◽

Content Type ◽

Type Iv ◽

Bilateral Carotid ◽

The Mean ◽

Danlos Syndrome

✓ Spontaneous bilateral carotid-cavernous fistulae and cervical artery dissection is reported in a 20-year-old woman with Ehlers-Danlos syndrome Type IV. The clinical features of 16 previously published cases of spontaneous carotid-cavernous fistulae associated with Ehlers-Danlos syndrome Type IV are reviewed, for a total of 17 cases. The mean age of the 14 women and three men was 31.6 years. Only direct fistulae were encountered. Diagnostic neuroangiography carried morbidity and mortality rates of 36% and 12%, respectively; neuroradiological treatment resulted in death in one of six patients. The possible value of desmopressin in the management of these patients is discussed. In view of the risks of arterial puncture and surgery, the authors emphasize the importance of early recognition of Ehlers-Danlos syndrome.

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Endovascular treatment for a spontaneous rupture of the posterior tibial artery in a patient with Ehlers-Danlos syndrome Type IV: Report of a case

Surgery Today ◽

10.1007/s00595-008-3881-9 ◽

2009 ◽

Vol 39 (6) ◽

pp. 523-526 ◽

Cited By ~ 22

Author(s):

Kazuhide Matsushima ◽

Hiroaki Takara

Keyword(s):

Endovascular Treatment ◽

Spontaneous Rupture ◽

Posterior Tibial Artery ◽

Syndrome Type ◽

Ehlers Danlos Syndrome ◽

Posterior Tibial ◽

Tibial Artery ◽

Type Iv ◽

Danlos Syndrome

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

Vascular ◽

10.1177/1708538113505519 ◽

2013 ◽

Vol 22 (5) ◽

pp. 341-345 ◽

Cited By ~ 1

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.

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