open access

Vol 21, No 2 (2015)
Case reports
Published online: 2015-10-21
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Vascular complications in Ehlers-Danlos syndrome type IV — case report

Katarzyna Grygiel, Maciej Jedrasik, Hamid Feiz Allah Poor, Slawomir Nazarewski, Jacek Szmidt
DOI: 10.5603/AA.2015.0012
·
Acta Angiologica 2015;21(2):61-65.

open access

Vol 21, No 2 (2015)
Case reports
Published online: 2015-10-21

Abstract

Ehlers-Danlos syndrome is the most common inherited connective tissue disease and is a consequence of abnormal collagen synthesis. Type IV of this syndrome, called vascular, is considered to be the most severe. Patients with vascular type are prone to large arteries rupture and have higher likelihood of aneurysms development. We present a case of a patient with type IV Ehlers-Danlos syndrome, who suffered aortic rupture after surgical treatment of coeliac trunk compression syndrome. During the operation, the suturing the prosthesis to aorta was impossible due to the fragility of the aorta wall. Instead, a portion of a Dacron graft was used to envelop the aorta at the level of the rupture. Postoperative period was uneventful. The patient required delayed stent placement in common iliac artery due to its dissection. After 8 years of follow up patient remains in good condition.

Abstract

Ehlers-Danlos syndrome is the most common inherited connective tissue disease and is a consequence of abnormal collagen synthesis. Type IV of this syndrome, called vascular, is considered to be the most severe. Patients with vascular type are prone to large arteries rupture and have higher likelihood of aneurysms development. We present a case of a patient with type IV Ehlers-Danlos syndrome, who suffered aortic rupture after surgical treatment of coeliac trunk compression syndrome. During the operation, the suturing the prosthesis to aorta was impossible due to the fragility of the aorta wall. Instead, a portion of a Dacron graft was used to envelop the aorta at the level of the rupture. Postoperative period was uneventful. The patient required delayed stent placement in common iliac artery due to its dissection. After 8 years of follow up patient remains in good condition.

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Keywords

Ehlers-Danlos syndrome, coeliac trunk compression syndrome abdominal aorta, aortic rupture, dissection

About this article
Title

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

Journal

Acta Angiologica

Issue

Vol 21, No 2 (2015)

Pages

61-65

Published online

2015-10-21

DOI

10.5603/AA.2015.0012

Bibliographic record

Acta Angiologica 2015;21(2):61-65.

Keywords

Ehlers-Danlos syndrome
coeliac trunk compression syndrome abdominal aorta
aortic rupture
dissection

Authors

Katarzyna Grygiel
Maciej Jedrasik
Hamid Feiz Allah Poor
Slawomir Nazarewski
Jacek Szmidt

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