title: Spontaneous Carotid-Cavernous Fistula in the Type IV Ehlers-Danlos Syndrome.
abstract: Ehlers-Danlos syndrome (EDS) is a rare inherited connective disease. Among several subgroups, type IV EDS is frequently associated with spontaneous catastrophic bleeding from a vascular fragility. We report on a case of carotid-cavernous fistula (CCF) in a patient with type IV EDS. A 46-year-old female presented with an ophthalmoplegia and chemosis in the right eye. Subsequently, seizure and cerebral infarction with micro-bleeds occurred. CCF was completely occluded with transvenous coil embolization without complications. Thereafter, the patient was completely recovered. Transvenous coil embolization can be a good treatment of choice for spontaneous CCF with type IV EDS. However, every caution should be kept during invasive procedure.
authors.affiliation: Department of Neurosurgery, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, Korea.
authors.forename: Jeong Gyun
authors.initials: JG
authors.lastname: Kim
authors.affiliation: Department of Neurosurgery, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, Korea.
authors.forename: Won-Sang
authors.initials: WS
authors.lastname: Cho
authors.affiliation: Department of Neurosurgery, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, Korea.
authors.forename: Hyun-Seung
authors.initials: HS
authors.lastname: Kang
authors.affiliation: Department of Neurosurgery, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, Korea.
authors.forename: Jeong Eun
authors.initials: JE
authors.lastname: Kim
keywords: Carotid-cavernous fistula; Ehler-Danlos syndrome; Transvenous embolization