A novel approach to the treatment of a direct carotid-cavernous fistula in a patient with Ehlers-Danlos syndrome type IV

We report a case of a direct carotid–cavernous fistula (CCF) in a patient with Ehlers–Danlos syndrome type IV who presented with progressive chemosis and diplopia. To prevent potential lethal arterial wall injury due to the fragility of the arterial vessel wall, the ipsilateral carotid artery and internal jugular vein were surgically exposed for direct insertion of endovascular sheaths, and transvenous embolization was performed using triple microcatheters with detachable coils. The clinical course was uneventful, and chemosis and diplopia subsequently resolved. By the 6 month follow-up, MRI revealed no recurrence of the CCF. These techniques offer a unique access alternative for endovascular treatment, thereby reducing the risks associated with arterial dissection that often accompanies transfemoral access in this particular condition.

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Treatment of a direct carotid-cavernous fistula in a patient with type IV Ehlers-Danlos syndrome: a novel approach

Neuroradiology ◽

10.1007/s00234-006-0084-1 ◽

2006 ◽

Vol 48 (7) ◽

pp. 491-494 ◽

Cited By ~ 17

Author(s):

J. K. Hollands ◽

T. Santarius ◽

P. J. Kirkpatrick ◽

J. N. Higgins

Keyword(s):

Carotid Cavernous Fistula ◽

Ehlers Danlos Syndrome ◽

Cavernous Fistula ◽

Type Iv ◽

Novel Approach ◽

Danlos Syndrome ◽

Direct Carotid Cavernous Fistula

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Carotid-Cavernous Fistula Associated with Ehlers-Danlos Syndrome Type IV

Interventional Neuroradiology ◽

10.1177/159101999900500408 ◽

1999 ◽

Vol 5 (4) ◽

pp. 313-320 ◽

Cited By ~ 9

Author(s):

Q. Bashir ◽

J. Thornton ◽

S. Alp ◽

G.M. Debrun ◽

V.A. Aletich ◽

...

Keyword(s):

Underlying Disease ◽

Syndrome Type ◽

Review Of The Literature ◽

Carotid Cavernous Fistula ◽

Ehlers Danlos Syndrome ◽

Cavernous Fistula ◽

Type Iv ◽

Danlos Syndrome ◽

Direct Carotid Cavernous Fistula

A case of traumatic, direct, carotid cavernous fistula (CCF) associated with Ehlers — Danlos syndrome (EDS) Type IV is reported along with a review of the literature. Excluding the present case, three similar cases associated with EDS-TypeIV have already been reported by Gerard M. Debrun et Al1. Despite the risks associated with endovascular manipulation, the fistula was successfully closed by intravascular embolisation but the patient expired a few days later because of underlying disease-associated vascular and visceral complications.

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Republished: Successful treatment of direct carotid–cavernous fistula in a patient with Ehlers–Danlos syndrome type IV without arterial puncture: the transvenous triple-overlay embolization (TAILOREd) technique

Journal of NeuroInterventional Surgery ◽

10.1136/neurintsurg-2017-013052.rep ◽

2020 ◽

Vol 12 (11) ◽

pp. e8-e8

Author(s):

Thien J Huynh ◽

Ryan P Morton ◽

Michael R. Levitt ◽

Basavaraj V. Ghodke ◽

Onno Wink ◽

...

Keyword(s):

Successful Treatment ◽

Three Dimensional ◽

Arterial Puncture ◽

Syndrome Type ◽

Carotid Cavernous Fistula ◽

Ehlers Danlos Syndrome ◽

Cavernous Fistula ◽

Type Iv ◽

Danlos Syndrome ◽

Direct Carotid Cavernous Fistula

We report successful transvenous treatment of direct carotid–cavernous fistula in a patient with Ehlers–Danlos syndrome type IV using a novel triple-overlay embolization (TAILOREd) technique without the need for arterial puncture, which is known to be highly risky in this patient group. The TAILOREd technique allowed for successful treatment using preoperative MR angiography as a three-dimensional overlay roadmap combined with cone beam CT and live fluoroscopy, precluding the need for an arterial puncture.

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