97th DOG Annual Meeting 1999



T. Hansen1, M. Warmuth-Metz2

Direct high-flow carotid-cavernous sinus fistulas (CCSF´s) are usually caused by trauma. However, they may also arise spontaneously after a rupture of an intracavernous carotid aneurysm or due to a collagen vascular disease like Ehlers-Danlos syndrome.

Case report: A 24-year-old woman complained of an acute developing proptosis and reduced visual acuity in the left eye. She had no history of trauma, but her mother was said to have lost vision because of an connective tissue disorder.
On admission, the patient exhibited characteristic symptoms of a carotid-cavernous sinus fistula such as arterialization of conjunctival blood vessels, increased left intraocular eye pressure and a bruit over the left orbit. In the left eye corrected visual acuity was 0.6 and a complete third and sixth nerve palsy was present. Inspection of the body showed a superficial varicosis on both legs and several bruises on the limbs. Therefore the working diagnosis was a spontaneous CCSF associated with a hereditary connective tissue disease.
Although the histology was compatible with Ehlers-Danlos syndrome, none of the kown mutations could be found in dermal fibroblasts. Cerebral arteriography showed a direct high-flow CCSF on the left side with retrograde filling of the superior ophthalmic vein and luminal border irregularities of the right internal carotid artery. Two interventions of transarterial balloon obliteration were needed to occlude the fistula, because the first placed balloon dislocated causing a worsening of the symptoms. During the following months vision recovered with a slight remaining third and sixth nerve paresis.

Conclusions: Spontaneous direct carotid-cavernous sinus fistulas require intensive investigations for associated disorders. In case of proven or suspected connective tissue disease interventional therapies must be indicated and performed with great caution.

Departments of Ophthalmology1 and Neuroradiology2, Julius-Maximilians-University, Würzburg