Best disease (also known as vitelliform macular dystrophy) is an inherited form of macular degeneration characterised by a reduction in central vision. The disease begins in childhood and affects the central part of the retina (known as the macula) which is responsible for fine visual detail and colour perception. The progression of visual loss varies between those affected by Best disease but side or peripheral vision generally remains unaffected.

Early signs of Best Disease usually develop between the ages of three and 15. During the early stages, the disease doesn’t always have much effect on vision so the child may not notice any issues with their sight. It is often picked up at a routine eye examination before it has a chance to affect the vision.

The disease is increasingly being picked up through screening programmes. This allows family members of a patient who has Best disease to have a genetic test to identify if they may develop the condition.

Sometimes a child may notice a change in their vision with the resulting eye test confirming they have retinal changes which could indicate Best disease. Although a child may notice early vision problems they may not develop vision problems until later on in life – generally over the age of 40 or 50.