Journal of Korean Neurosurgical Society

The Korean Neurosurgical Society (대한신경외과학회)
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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)
  • Received : 2013.01.15
  • Accepted : 2014.01.10
  • Published : 2014.02.28
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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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References

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