Question
This is a fundus of a 15-year-old boy with a visual acuity of 6/9 in both eyes and a severely reduced electro-oculogram. The fellow eye has a similar appearance. What is the most likely diagnosis
| A. |
Stargardt disease
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| B. |
Best disease
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| C. |
Fundus flavimaculatus
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| D. |
Sticklers Syndrome
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Show Answer
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Correct Answer � B
Explanation
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Answer B) Best disease
· The picture shows Best disease with classical egg yolk appearance at macula.
· Inheritance is autosomal dominant with variable penetrance and expressivity.
The five stages of Best’s disease are:
• Stage 1—pre vitelliform (normal fundus but abnormal EOG)
• Stage 2—vitelliform (egg-yolk stage) as shown in this case
• Stage 3—pseudohypopyon (part of lesion becomes absorbed)
• Stage 4—vitelliruptive (scrambled-egg)
• Stage 5—end stage is characterized by one of the following: Hypertrophic macular scar, Atrophic macular scar, Vascularized fibrous macular scar.
· Visual acuity is usually no worse than 6/60.
· EOG is severely subnormal during all stages and also abnormal in carriers with normal fundi
