title: A novel approach to the treatment of a direct carotid-cavernous fistula in a patient with Ehlers-Danlos syndrome type IV.
abstract: 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.
authors.affiliation: Department of Neurosurgery, Nara Medical University, Kashihara, Nara, Japan.
authors.forename: Ichiro
authors.initials: I
authors.lastname: Nakagawa
authors.affiliation: Department of Neurosurgery, Nara Medical University, Kashihara, Nara, Japan.
authors.forename: Hun Soo
authors.initials: HS
authors.lastname: Park
authors.affiliation: Department of Radiology, Nara Medical University, Kashihara, Japan.
authors.forename: Takeshi
authors.initials: T
authors.lastname: Wada
authors.affiliation: Department of Radiology, Nara Medical University, Kashihara, Japan.
authors.forename: Katsutoshi
authors.initials: K
authors.lastname: Takayama
authors.affiliation: Department of Radiology, Nara Medical University, Kashihara, Japan.
authors.forename: Hiroyuki
authors.initials: H
authors.lastname: Nakagawa
authors.affiliation: Department of Radiology, Nara Medical University, Kashihara, Japan.
authors.forename: Kimihiko
authors.initials: K
authors.lastname: Kichikawa
authors.affiliation: Department of Neurosurgery, Nara Medical University, Kashihara, Nara, Japan.
authors.forename: Hiroyuki
authors.initials: H
authors.lastname: Nakase
mesh_terms: D000328:Adult; D020216:Carotid-Cavernous Sinus Fistula / Q000000981:diagnostic imaging / Q000209:etiology / Q000628:therapy*; D002404:Catheterization / Q000379:methods; D004535:Ehlers-Danlos Syndrome / Q000150:complications*; D004621:Embolization, Therapeutic / Q000379:methods*; D006801:Humans; D018810:Magnetic Resonance Angiography; D008297:Male; D011859:Radiography; D055815:Young Adult
keywords: Fistula; Intervention; Vessel Wall