Cilio-optic vein associated with phakomatosis.

This presentation demonstrates the fluorescein angiographic characteristics of the cilio-optic vein. These congenitally enlarged vessels appear at the disc edge and dip into the optic nerve to anastomose with branches of the central retinal vein. Fluorescein angiography shows lamellar filling of the vessels in the early choroidal phase. In one patient, these findings are demonstrated bilaterally with the use of bilateral simultaneous angiography. A second type of communication is presented, a retinociliary vein which drains the retina into the choroidal circulation. In both types presented, a branching hypofluorescent pattern extends from the disc vessel into the choroid. Our patients have evidence of a phakomatosis: neurofibromatosis or Sturge-Weber syndrome. This anomalous disc vessel should not be confused with optociliary shunt, disc neovascularization, cilioretinal artery, or arteriovenous shunt.