title: Transvenous embolization for carotid-cavernous fistula in a patient with vascular type of Ehlers-Danlos syndrome--direct superior ophthalmic vein approach: case report.
abstract: The vascular type of Ehlers-Danlos syndrome (vEDS) is an autosomal dominant hereditary disease characterized by connective tissue fragility throughout the body, including the arteries, viscera, and gastrointestinal tract. We report a case in which we performed transvenous embolization (TVE) via direct superior ophthalmic vein (SOV) approach to treat a direct carotid-cavernous fistula (CCF) in a patient with Ehlers-Danlos syndrome (EDS). The patient was a 37-year-old woman who developed tinnitus in her left ear and a headache during examination in the outpatient clinic of another hospital in order to make a definitive diagnosis of vEDS, and she was referred to our hospital and examined. Based on the results of all of the studies she was diagnosed with a CCF. Conservative treatment was attempted, but was not very effective. Because of progressing aphasia, TVE was performed via the SOV direct cut. There were no intraoperative or postoperative complications. It has been reported that cerebral angiography is generally contraindicated in vEDS and that the morbimortality associated with endovascular treatment is very high. When performing treatment it is necessary to be sufficiently aware of the risks it entails.
authors.affiliation: Department of Neurosurgery, Fujita-Health University School of Medicine.
authors.forename: Teppei
authors.initials: T
authors.lastname: Tanaka
authors.forename: Motoharu
authors.initials: M
authors.lastname: Hayakawa
authors.forename: Akiyo
authors.initials: A
authors.lastname: Sadato
authors.forename: Kazuhide
authors.initials: K
authors.lastname: Adachi
authors.forename: Takeya
authors.initials: T
authors.lastname: Watabe
authors.forename: Shingo
authors.initials: S
authors.lastname: Maeda
authors.forename: Masahiro
authors.initials: M
authors.lastname: Ohmura
authors.forename: Yuichi
authors.initials: Y
authors.lastname: Hirose
mesh_terms: D000328:Adult; D015901:Angiography, Digital Subtraction; D001037:Aphasia / Q000209:etiology; D001164:Arteriovenous Fistula / Q000209:etiology / Q000628:therapy*; D002343:Carotid Artery, Internal / Q000473:pathology*; D002426:Cavernous Sinus / Q000473:pathology*; D002533:Cerebral Angiography; D002550:Cerebral Veins; D004535:Ehlers-Danlos Syndrome / Q000150:complications*; D004621:Embolization, Therapeutic / Q000379:methods*; D057510:Endovascular Procedures / Q000379:methods*; D005260:Female; D006801:Humans; D002538:Intracranial Arteriovenous Malformations / Q000209:etiology / Q000628:therapy*; D014012:Tinnitus / Q000209:etiology