Best's disease

Vitelliform stage.

Pseudohypopyon stage.
The macula contains well circumscribed, rounded macular lesion measuring about 1 to 2 disc diameter. The lesion 
contains yellowish substance show at the inferior half of the lesion (pseudohypopyon). The condition is bilateral 
(although the macula may show different stages of Best's disease).

Other examination:

  • look outside the macula for smaller lesion
Note: Best's disease evolves through five stages. Most patients are over the age of 20 have vitelliform eruptive or 
end stages. The end stage may be indistinguishable from age-related macular degeneration. In young patient with 
macular atrophy or fibrosis always consider Best disease in your differential diagnosis.


1. How is Best's disease inherited?

2. What are the various stages of Best's macular dystrophy?

3. List some of the common hereditary macular disorders.

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