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September 2008

Chorioretinal Degeneration With Atypical Fundus Autofluorescence
Written by John P. Berdahl, MD, Jeanne Lee Queen, CPT, and Srilaxmi
Bearelly, MD, Duke University Eye Center, Durham, N.C.

A60-year-old Caucasian woman presented for a second opinion regarding a prior diagnosis of retinitis pigmentosa. She reported poor night vision for the past 25 years with it worsening over the last five. BCVA was 20/200 in the right eye and 20/30 in the left. Color vision was 0/15 in the right eye and 4/15 in the left. The retinal vasculature was attenuated and optic nerves demonstrated pallor. There were a few pigment clumps in the posterior pole of both eyes, but no peripheral bone spicules. OCT showed a diffuse retinal atrophy. Goldmann visual field revealed central scotomas in both eyes with peripheral constriction. Autofluorescence imaging demonstrated areas of missing autofluorescence corresponding with retinal pigment epithelium loss. A differential diagnosis included choroideremia, generalized choroidal dystrophy, atypical retinitis pigmentosa and gyrate atrophy.

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