Stardgart’s disease is the most common form of juvenile macular dystrophy. It is an autosomal recessively inherited hereditary eye disease.
It usually presents during the first or second decade of life and causes a slowly progressive loss of central vision in both eyes.
The fundus shows a bronze-bronze image of the macula struck with yellowish-white fish-shaped deposits. In late stages, geographic atrophy of the macula appears as a bull’s eye.
How is Stardgart’s disease diagnosed?
Ophthalmology specialists can determine the state of the macula by means of these diagnostic imaging tests:
- Autofluorescence: dark areas corresponding to severe atrophy of the pigment epithelium associated with hyperreflective spots corresponding to deposits are seen.
- Angiographic image of “dark choroid”: The visualization of large retinal vessels over a hidden choroidal vascularization, associated with macular hyperfluorescence in bull’s eye due to its atrophy, is very characteristic.
- Macular OCT: atrophy of the photoreceptor layer and pigment epithelium in the macular area.
Is there any treatment for Stardgart’s disease?
To date, there is no treatment for this disease, although there are currently several avenues of research.
What is the prognosis of this type of macular dystrophy?
The prognosis in general is not good, with a progressive loss of central vision that usually stabilizes in youth, although in any case peripheral vision will always be preserved.
Any advice for patients suffering from Stardgart’s disease?
As general recommendations the patient should:
- Avoid vitamin A intake.
- Ask for a joint assessment by an ophthalmologist and low vision specialists to adapt sunscreens and optical aids.
- Seek guidance from centers such as ONCE.