Fig. 1. Colored fundus photo. White ischemic posterior pole with a «cherry-red spot» aspect at the macula in an area supplied by a cilioretinal artery
Fig. 2.In harmony fluorescein angiogram
Young patients with cilioretinal artery occlusion; without classical vascular risk factors and identifiable sources of emboli or inflammatory diseases may have Flammer Syndrome.
An occlusion of a cilioretinal artery (CLRAO) which may be associated with a significant loss of central vision, indicates 5% of all retinal arterial occlusions.
CLRAOs in patients younger then 20 years of age are rare and have been described in conjunction with general conditions including; elevated intracranial pressure, hypercoagulable states and cardiac diseases.Flammer syndrome has been reported to predispose to central retinal venous occlusion (CRVO) and CLRAO in young patients.
An 18-year-old female applied to our clinic with history of a central scotoma that lasts for 12 hours which has appeared suddenly and remained unchanged afterwards in her right eye.
Visual acuity was 1.0 sc in the lefteye and finger count at 10 cm sc in the right. Ocular fundus in the right eye showed sharply demarcated, whitishgreyish fading at the posterior pole, reaching from close to the optic disc margin to temporally of the fovea.
The fovea had a “cherry-red-spot” appearance. Standard therapy was performed to patient. Hematologic, biochemical and rheumatologic workup was negative. Although there were no classical vascular risk factors or identifiable sources of emboli, she had signs of Flammer Syndrome.
Conclusion
We described a case with Flammer syndrome, with the absence of classical risk factors for arterial occlusions and of identifiable sources of emboli; disturbed regulation of retinal vessels led us to the supposition that Flammer syndrome has predisposed this young patient to develop CLRAO.
