Spontaneous Carotid-Cavernous Fistula in the Type IV Ehlers-Danlos Syndrome.
10.3340/jkns.2014.55.2.92
- Author:
Jeong Gyun KIM
1
;
Won Sang CHO
;
Hyun Seung KANG
;
Jeong Eun KIM
Author Information
1. Department of Neurosurgery, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, Korea. hsk4428@yahoo.com
- Publication Type:Case Report
- Keywords:
Carotid-cavernous fistula;
Ehler-Danlos syndrome;
Transvenous embolization
- MeSH:
Cerebral Infarction;
Ehlers-Danlos Syndrome*;
Embolization, Therapeutic;
Female;
Fistula*;
Hemorrhage;
Humans;
Middle Aged;
Ophthalmoplegia;
Seizures
- From:Journal of Korean Neurosurgical Society
2014;55(2):92-95
- CountryRepublic of Korea
- Language:English
-
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.