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Jud, P; Gary, T; Hafner, F; Tiesenhausen, K; Ott, T; Oswald, WK; Brodmann, M.
Multiple arterial thromboses due to cystic medial degeneration Erdheim-Gsell: A case report.
Medicine (Baltimore). 2017; 96(47):e8782-e8782
Doi: 10.1097/MD.0000000000008782
(- Case Report)
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- Leading authors Med Uni Graz
- Jud Philipp
- Co-authors Med Uni Graz
- Brodmann Marianne
- Gary Thomas
- Hafner Franz
- Oswald Wolfgang Kurt
- Ott Thomas
- Tiesenhausen Kurt
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- Abstract:
- Cystic medial degeneration Erdheim-Gsell is a vascular pathology mainly of the large vessels, which is mostly associated with Marfan syndrome or Ehlers-Danlos syndrome. The clinical findings of this entity are aneurysms of the aorta or large peripheral arteries which usually present in an acute setting due to rupture of an aneurysm. We present a case of a 43-year-old Caucasian male with histologically proven cystic medial degeneration of the lower limb vessels mimicking peripheral artery occlusive disease. Despite antiplatelet and anticoagulant treatment, the patient suffered multiple vascular stenosis and occlusions. Multiple arterial stenoses and thromboses leading to peripheral artery occlusive disease caused by cystic medial degeneration Erdheim-Gsell. Multiple surgical and endovascular interventions including bypass graft and intra-arterial thrombolysis as well as oral antiplatelet and anticoagulant therapy. Despite dual antiplatelet therapy, anticoagulant therapy with rivaroxaban and multiple surgical and endovascular interventions, the patient developed recurrent arterial thromboses. The patient did not suffer further thrombotic events since clopidogrel and phenprocoumon were administered. Clinical presentation of cystic medial degeneration Erdheim-Gsell mimicking peripheral artery occlusive disease is very unusual. Due to the fragile vessel wall, patients with cystic medial degeneration might have a higher risk to develop arterial thromboses, even under antiplatelet therapy or anticoagulant treatment. Copyright © 2017 The Authors. Published by Wolters Kluwer Health, Inc. All rights reserved.
- Find related publications in this database (using NLM MeSH Indexing)
- Adult -
- Aortic Aneurysm, Thoracic - complications
- Arterial Occlusive Diseases - etiology
- Cysts - complications
- Humans -
- Male -
- Thrombosis - etiology
- Find related publications in this database (Keywords)
- arterial thrombosis
- cystic medial degeneration Erdheim-Gsell
- intra-arterial thrombolysis
- peripheral artery occlusive disease
- procoagulant state