Re: Carotid Cavernous Fistula

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SPONTANEOUS CAROTID-CAVERNOUS SINUS FISTULA CAUSED BY CONNECTIVE TISSUE

DISEASE

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