[Skip to Navigation]
February 1972

Pseudoxanthoma Elasticum: Association With Bilateral Carotid Rete Mirabile and Unilateral Carotid-Cavernous Sinus Fistula

Author Affiliations

San Francisco
From the Neuro-ophthalmology Unit, departments of ophthalmology, neurology, and neurological surgery, University of California, San Francisco. Dr. Rios-Montenegro is now with the Department of Ophthalmology, Universidad Nacional Mayor de San Marcos, Lima, Peru. Dr. Behrens is now with the Neuro-ophthalmology Unit, Department of Ophthalmology, Columbia Presbyterian Medical Center, New York. Dr. Rios-Montenegro is a fellow in neuro-ophthalmology, and Dr. Behrens is a Heed fellow in neuro-ophthalmology.

Arch Neurol. 1972;26(2):151-155. doi:10.1001/archneur.1972.00490080069007

Pseudoxanthoma elasticum in a 20-year-old man was complicated by right-sided proptosis, conjunctival vascular dilation, and subretinal bleeding from preexisting angioid streaks. Angiography showed multiple anomalies of major systemic arteries, including bilateral carotid rete mirabile and right carotid-cavernous sinus fistula. Electrothrombosis of the right cavernous sinus obliterated the fistula, restored cerebral perfusion by the right carotid system, and promoted prompt resolution of the presenting signs.

Add or change institution