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

Journal of Korean Neurosurgical Society 2014³â 55±Ç 2È£ p.92 ~ p.95

±èÁ¤±Õ(Kim Jeong-Gyun) - Seoul National University College of Medicine Seoul National University Hospital Department of Neurosurgery
Á¶¿ø»ó(Cho Won-Sang) - Seoul National University College of Medicine Seoul National University Hospital Department of Neurosurgery
°­Çö½Â(Kang Hyun-Seung) - Seoul National University College of Medicine Seoul National University Hospital Department of Neurosurgery
±èÁ¤Àº(Kim Jeong-Eun) - Seoul National University College of Medicine Seoul National University Hospital Department of Neurosurgery

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.

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Carotid-cavernous fistula, Ehler-Danlos syndrome, Transvenous embolization
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