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

• DOI: 10.3340/jkns.2014.55.2.92
• Publication Date: 2014-03-14T04:23:07Z
• Authors: Jeong Gyun Kim; Won-Sang Cho; Hyun-Seung Kang; Jeong Eun Kim
• Keywords: 03 medical and health sciences; 0302 clinical medicine; Case Report; 3. Good health

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.