title: Spontaneous carotid-cavernous fistula in a patient with Ehlers-Danlos syndrome type IV--case report.
abstract: A 30-year-old female complained of sudden onset of severe proptosis, chemosis, diplopia, and bruit. Right carotid angiography showed a high-flow direct carotid-cavernous fistula (CCF) draining into the engorged superior ophthalmic vein, inferior petrosal sinus, and pterygoid plexus. The patient experienced retroperitoneal bleeding from a ruptured right renal artery after undergoing cerebral angiography. We suspected Ehlers-Danlos syndrome (EDS) type IV, which was confirmed by showing cultured fibroblasts failed to secrete procollagen type III. Endovascular surgery cannot be considered the treatment method of choice in view of the fragility of the arteries and veins in patients with EDS type IV. We treated our patient with extracranial internal carotid artery ligation. Currently, there is no ideal treatment for CCF in patients with EDS type IV. Since CCF is rarely life-threatening, the investigative approach and course of treatment must consider the associated vascular fragility.
authors.affiliation: Department of Neurosurgery, Juntendo University School of Medicine, Tokyo, Japan.
authors.forename: Takashi
authors.initials: T
authors.lastname: Mitsuhashi
authors.forename: Masakazu
authors.initials: M
authors.lastname: Miyajima
authors.forename: Rikizou
authors.initials: R
authors.lastname: Saitoh
authors.forename: Yasuaki
authors.initials: Y
authors.lastname: Nakao
authors.forename: Makoto
authors.initials: M
authors.lastname: Hishii
authors.forename: Hajime
authors.initials: H
authors.lastname: Arai
mesh_terms: D000328:Adult; D020216:Carotid-Cavernous Sinus Fistula / Q000209:etiology*; D004535:Ehlers-Danlos Syndrome / Q000150:complications*; D005260:Female; D006801:Humans