J Korean Neurol Assoc.  1997 Oct;15(5):1148-1154.

Bilateral spontaneous carotid-cavernous fistula(CCF) presenting as painful ophthalmoplegia: Two cases

• Jun SM
• Park KW
• Gha JK
• Kim SH
• Kim JW
• Choi SS

Abstract

Carotidcavernous fistula(CCF) is a syndrome in which arteriovenous sinus shunts exist between the carotid artery and the cavernous sinus. These shunts have very wide spectrums in pathogenesis, angiografic findings, hemodynamics and teatment. Spontaneous dural CCF is dural vascular malformations that usually runs a benign course with variable clinical findings. We experienced two case of bilateral spontaneous CCF presenting as painful ophthalmoplegia. In the first case, a 53-years old hypertensive, diabetic woman had previous history of unilateral painful ophthalmoplegia eight months before admission and as partially recovered. On examination, she had slowly progressive bilateral chemosis, exophthalmos, and sixth nerve palsy. MRI showed abnormal vessels in the cavernous sinus from prominent superior ophthalmic vein. Angiographic findings showed fistulas of both external carotid and left internal carotid arteries draining into the cavernous sinus. We had multiple trials of transarterial and transvenous embolization with gelfom material or platinum coils with partial improvement of chemosis and exophthalmos. In the second case, a 45-years old woman noted slowly progressive headache, ptosis, dilatation of pupil in the left side, and diplopia suggesting paralysis of the left inferior rectus muscle. MRI showed signal voids in the cavernous sinus. Angiographic findings showed fistulars of the right and left internal carotid arteries draining into the cavernous sinus. CCF should be consider as a underlying cause in patient presenting as painful ophthalmoplegia. We report two cases of bilateral spontaneous CCF presenting as painful ophthalmoplegia.

MeSH Terms

Abducens Nerve Diseases
Carotid Arteries
Carotid Artery, Internal
Cavernous Sinus
Dilatation
Diplopia
Exophthalmos
Female
Fistula
Headache
Hemodynamics
Humans
Magnetic Resonance Imaging
Middle Aged
Ophthalmoplegia*
Paralysis
Platinum
Pupil
Vascular Malformations
Veins
Platinum