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http://dx.doi.org/10.3340/jkns.2014.55.2.92

Spontaneous Carotid-Cavernous Fistula in the Type IV Ehlers-Danlos Syndrome  

Kim, Jeong Gyun (Department of Neurosurgery, Seoul National University Hospital, Seoul National University College of Medicine)
Cho, Won-Sang (Department of Neurosurgery, Seoul National University Hospital, Seoul National University College of Medicine)
Kang, Hyun-Seung (Department of Neurosurgery, Seoul National University Hospital, Seoul National University College of Medicine)
Kim, Jeong Eun (Department of Neurosurgery, Seoul National University Hospital, Seoul National University College of Medicine)
Publication Information
Journal of Korean Neurosurgical Society / v.55, no.2, 2014 , pp. 92-95 More about this Journal
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.
Keywords
Carotid-cavernous fistula; Ehler-Danlos syndrome; Transvenous embolization;
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