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 Cardiovascular Disease (CICD) in San Matteo University Hospital IRCCS,
a tertiary referral center for rare cardiovascular conditions.
The operative strategy for each patient was planned after a multidisciplinary evaluation.
All procedures were carried out electively.
Five patients (5/8,
62.5%) had previously undergone vascular surgery for lesions in other vessels.
The underlying genetic disease was known in 6/8 (75%) patients at the time of first intervention.
All subjects gave their written informed consent.
The study was approved by the institutional review board.
Twelve procedures (of which 2 arteriographies for pre-operative planning) were performed in 8 patients with the following clinical indications (arterial aneurysms,
pseudo-aneurysms,
carotid-cavernous fistula) in different anatomical sites.
One patient underwent 3 procedures, two patients 2 procedures,
and the other five 1 procedure.
Eleven of twelve cases (91.6%) had arterial vessels as targets,
whereas 1/12 targeted a carotid-cavernous fistula (CCF).
The procedures consisted in stentgraft deployment (6/8,
75%),
coil embolization (2/8,
25%),
stentgraft plus coil embolization (1/8,
12.5%) and non-adhesive glue injection (1/8,
12.5%).
(Tables 1,
2)
Vascular access was achieved through puncture of the common femoral artery (11/12,
91,6% right common femoral artery; 1/12 8,4% left common femoral artery) in all patients.
In the treatment of the CCF,
an additional femoral vein access for reaching the right cavernous vein was employed (Table 3).
Complications were classified according to the Clinical Practice Guidelines of the Society of Interventional Radiology [8].
All data values are shown as means ± SD (Standard Deviation).
Statistical analysis was performed using Fisher exact test for independence of qualitative variables and using the Student ttest for unpaired samples,
where appropriate,
for comparisons between the two groups.
Differences with p values less than .05 were considered significant.
Software STATA Version 13 (Stata Corporation,
College Station,
2013,
Texas,
USA) was used for all statistical analysis.