Stargardt's Disease (Fundus Flavimaculatus)

Arjeta Grezda, Ermal Simaku, Besim Boçi

Abstract


Background: Stargardt’s Disease is included in the group of degenerative macular diseases, which consists of the progressive loss of cones in fovea of both eyes, leading to variable levels of central vision loss. It is symptomatically similar to age-related macular degeneration and it affects approximately one in 10,000 children, in ages between 7 and 12 years old. Case Report: We report a case of a 13 years old boy who presented with severely reduced bilateral visual acuity. He was admitted to handicapped children school since he was suspected of malingering. He had no family history or previous ocular symptoms. His visual acuity was 20/200 s.c à 20/50 in mydriasis. Fluorescein Angiography, with the characteristic aspect of "bull's eye" due to a "window effect" at the level of the peri foveolar depigmentation limit, was very helpful to decide the right diagnosis.Conclusion: Currently, there is no effective treatment for Stargardt's disease, but having the right diagnosis may assist the patient, family members and the society to adapt helpful behaviors. Individuals benefit from the use of low vision aids and orientation and mobility training.

Keywords: Stargardt's disease; bilateral visual loss; progressive; children.


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DOI: http://dx.doi.org/10.0001/(aj).v4i10.1181

DOI (PDF): http://dx.doi.org/10.0001/(aj).v4i10.1181.g1572

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