What is best disease?

Best disease (or best vitelliform macular dystrophy) is a genetic condition you are born with, although it doesn't usually start to affect your vision until later in life. Best disease affects the macula, the part of your retina at the back of your eye. There is no current treatment for best disease at present, although research into gene therapy is on-going, which may lead to a treatment in the future.

There are five stages of best disease

  • Stage 1 - The macula looks healthy and no change can be seen.
  • Stage 2 - Vitelliform Sage - At this stage there is a blister on the macula area which looks similar to an egg yolk.
  • Stage 3 - Pseudohypopynon stage - some of the yellow matter which causes the egg yolk-like blister can breakthrough a layer under the retina. This leads to a cyst forming under the retina.
  • Stage 4 - Vitelliruptive stage - The lesion begins to break up and can cause damage to some of the cells in the layers of the retina. At this point you may start to experience changes in vision.
  • Stage 5 - Atrophic Stage - The yellow material which caused the lesions begins to withdraw and disappear. However, it leaves behind scarring and damaged cells on the retina. At this stage your sight is more seriously affected.

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