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8 Cards in this Set
- Front
- Back
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- Both share the same chromosomal locus
- Both caused by abnormal accumulation of lipofuscin in RPE - Characteristic yellowish-white pisciform flecks What's the Dx? |
Stargardt's/Fundus Flavimaculatus
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- mostly in the periphery
- NO acuity loss unless macula involved - Characteristic yellowish-white pisciform flecks - caused by abnormal accumulation of lipofuscin in RPE - shares similar characteristics as Stargardt's Disease |
Fundus Flavimaculatus
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- Mostly MACULAR
- Characteristic yellowish-white pisciform flecks - caused by abnormal accumulation of lipofuscin in RPE - Presents at age 8-14 years - Slow, symmetrical progressive vision loss to 20/200 by age 30 - Early in disease fundus may appear normal - Later loss of FR, then round, grayish or pigmented macular atrophy (beaten-metal appearance) What's the Dx? |
Stargardt's Disease
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- Sharply defined, yellowish discoid lesion in macular area
- autosomal dominant - diagnosed between 3 and 15 years of age - Vision usually normal to mildly reduced when diagnosed - Very slowly progressive - Later, lesion may break-up & result in atrophic macular changes resulting in 20/200 vision What's the Dx? |
Best's Vitelliform Degeneration
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Heterogeneous group of disorders:
- Most present in 1st to 2nd decades - Abnormal cone ERGs - Bull's Eye Maculopathy - Photophobia - Reduced central vision - Abnormal color vision - Temporal pallor of ONH - May have later evidence of rod dysfunction What's the Dx? |
Cone Dystrophy
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Retinal Signs:
- Marked RPE changes, usually present in 1st decade - "Salt & Pepper" RPE changes in early stage, start in equatorial region - Bone spicules - Optic atrophy - Vessel attenuation Clinical symptoms usually present by age of 30 What's the Dx? |
Retinitis Pigmentosa
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What retinal diseases present in childhood?
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1. Stargardt's Disease
2. Fundus Flavimaculatus 3. Best's Vitelliform Degeneration 4. Cone Dystrophy 5. Retinitis Pigmentosa |
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Which retinal condition is a Bull's Eye Maculopathy seen?
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Cone Dystrophy
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