Aims and objectives
We describe our experience in endovascular percutaneous treatment in patients with Ehlers-Danlos syndrome (EDS) type IV,
reporting the outcomes and procedure-related complications,
focusing on the management of the vascular access site by means of closure device use. EDS type IV (also known as vascular type or vEDS) is an autosomal dominant inherited condition due to the mutation in the COL3A1 gene,
which encodes for type III collagen chains.
Considering all of the different forms of EDS...
Methods and materials
This is a retrospective monocentric study.
From January 2009 to December 2017,
8 consecutive patients (4 male,
and 4 female; mean age ± SD 42 ± 19 years,
range 23-53 years) with molecular diagnosis of EDS type IV,
who underwent endovascular procedures performed by the interventional radiology team,
were identified by consulting the angiographic suites’ procedure file database and digital archive. Patients were referred to us from the Vascular Surgery Department and the Centre for Inherited...
Eight patients found positive for COL3A1 gene mutation underwent a total of 12 procedures (2 4Fr diagnostic artertiographys and 6 6Fr endovascular treatments) which consisted of: • 3 embolizations (1 symptomatic carotid cavernous fistula,
1 hepatic and 1 splenic aneurysm) • 6 stent graft placements (2 superior mesenteric,
1 internal carotid,
1 renal and 1 symptomatic vertebral aneurysm) • 1 renal stent grafting with embolization. Endovascular treatment was successful in 100% (12)...
Elective endovascular procedures can safely be performed in order to prevent frequent catastrophic vascular events in EDS type IV patients,
with potentially both high clinical and technical success.
Vascular access can be performed percutaneously.
Safe hemostasis without complications can be managed with closure devices.
MD University of Pavia,
Fondazione IRCCS Policlinico San Matteo,
Department of Diagnostic and Interventional Radiology and Neuroradiology,
Unit of Interventional Radiology - Pavia,
Italy. via Chiozzo 11,
27100 Pavia (PV),
Italy Phone: + 393483073031 e-mail: firstname.lastname@example.org
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