title: Aggressive change of a carotid-cavernous fistula in a patient with Ehlers-Danlos syndrome type IV.
abstract: The authors report a rare case of a carotid-cavernous fistula (CCF) secondary to Ehlers-Danlos syndrome (EDS) type IV which showed an aggressive angiographical change.A 59-year-old woman presented with headache, right pulsatile tinnitus, and diplopia on the right side. The diagnostic angiography demonstrated a right CCF. Accordingly transarterial embolization of the fistula was attempted 5 days later. The initial right internal carotid angiography showed an aneurysm on the petrous portion of the internal carotid artery (ICA) which was not recognized in the diagnostic angiography. Spontaneous reduction of the shunt flow and long dissection of the ICA were also revealed. The aneurysm was successfully occluded with coils, and only minor shunt flow was shown on the final angiogram. EDS type IV was diagnosed with a skin biopsy for a collagen abnormality. After the operation, the stenosis of the right ICA gradually progressed, although there was no recurrence of the CCF.Interventional treatment for patients with EDS can cause devastating vascular complication. We should be aware of the possibility of EDS type IV when a spontaneous CCF shows unusual angiographical change because early diagnosis of EDS type IV is crucial for determination of the optimum treatment option.
authors.affiliation: Department of Neurosurgery, Saitama City Hospital, Saitama, Japan akojima-nsu@umin.ac.jp.
authors.forename: Atsuhiro
authors.initials: A
authors.lastname: Kojima
authors.affiliation: Department of Neurosurgery, Saitama City Hospital, Saitama, Japan.
authors.forename: Isako
authors.initials: I
authors.lastname: Saga
authors.affiliation: Department of Neurosurgery, School of Medicine, Keio University, Tokyo, Japan.
authors.forename: Ryosuke
authors.initials: R
authors.lastname: Tomio
authors.affiliation: Department of Medical Genetics, Shinshu University School of Medicine, Nagano, Japan.
authors.forename: Tomoki
authors.initials: T
authors.lastname: Kosho
authors.affiliation: Department of Dermatology, School of Medicine, Dokkyo Medical University, Tochigi, Japan.
authors.forename: Atsushi
authors.initials: A
authors.lastname: Hatamochi
mesh_terms: D015901:Angiography, Digital Subtraction; D001706:Biopsy; D020216:Carotid-Cavernous Sinus Fistula / Q000000981:diagnostic imaging* / Q000628:therapy*; D002533:Cerebral Angiography; D003937:Diagnosis, Differential; D018450:Disease Progression; D004535:Ehlers-Danlos Syndrome / Q000000981:diagnostic imaging* / Q000628:therapy*; D004621:Embolization, Therapeutic / Q000379:methods*; D057510:Endovascular Procedures / Q000379:methods*; D005260:Female; D006801:Humans; D018810:Magnetic Resonance Angiography; D008875:Middle Aged
keywords: Aneurysm; Ehlers–Danlos syndrome type IV; carotid-cavernous fistula; transarterial embolization