Established choroideremia

End-stage choroideremia
This is an X-linked inherited condition (rarely other inheritance patterns) affecting both eyes. The majority of the patients 
seen will be male.Most cases seen in the examination are either established or end-stage choroideremia.

In established cases, there is atrophy of the retinal pigment epithelium and choroid with exposure of the sclera and 
large choroidal blood vessels. The condition usually starts in the equator and spread centrally and peripherally. There 
are usually pigmentary changes on the fundus giving it a salt and pepper appearance.

In advanced cases, the fundus appears 'white-out' with total choroidal atrophy and exposure of the sclera. 


1. Which conditions apart from choroideremia are characterized by choroidal atrophy?

2. What is the visual prognosis of choroideremia?

3. What fundal changes would you expect  in carrier of choroideremia?


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