Central Carolina Surgical Eye Associates, P.A.


Case Nine: Bull's Eye Maculopathy/Cone-Rod Dystrophy

Color Photo of right eye. Note the beaten-bronze appearance to the central (macular) region. No deep yellow lesions are seen.  Color photo of left eye.  Note  the beaten-bronze appearance of the central region. Irregular coloration and pigmentation is seen in this area.
Red Free photo of right eye.  Note de-pigmentation and the generally oval shape of the disturbed area

Red Free photo of left eye. Note irregular pigmentation

Early phase FA right eye.  Note central blocking by intra-retinal pigment surrounded by transmission defects. Mid Phase FA right eye.  Note focal area of macular disturbance. No 'silent choroid' is seen.
Late phase FA right eye.  Note fading of hyperfluorescence. No leakage of dye or staining is seen. Late phase FA left eye. Note the absence of leakage of dye. No choroidal lesions are seen

Comment

This 21 year old Caucasian female presented because of blurred vision which caused her to fail her drivers license exam. Her vision with glasses was 20/30 right eye and 20/40 left eye. She missed half of the color vision plates in each eye. There was no pupillary abnormality. Her visual fields were normal. She had a bulls-eye and beaten-bronze appearance to each macula. She refused electrophysiologic testing and therefore we were not able to pinpoint her diagnosis. Since she took no systemic medications, a toxic maculopathy is not likely. Since there is no 'silent choroid' and there are no yellow lesions, the diagnosis of Stargardt's disease is unlikely. These bulls-eye maculae most likely represent a cone dystrophy, either the rod-cone variant or the cone-rod variant.

 


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