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KMID : 1001920140550020092
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Journal of Korean Neurosurgical Society
2014 Volume.55 No. 2 p.92 ~ p.95
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Spontaneous Carotid-Cavernous Fistula in the Type IV Ehlers-Danlos Syndrome
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Kim Jeong-Gyun
Cho Won-Sang
Kang Hyun-Seung
Kim Jeong-Eun
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Abstract
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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.
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KEYWORD
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Carotid-cavernous fistula, Ehler-Danlos syndrome, Transvenous embolization
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