Features

Gyrate atrophy

Disease
Gyrate atrophy of the choroid and retina is a rare metabolic chorioretinal dystrophy that is inherited as an autosomal recessive trait. It is bilateral and slowly progressive, commencing as a mid-peripheral atrophy of choroidal vessels, the RPE and photoreceptors.

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Symptoms

Typically, patients with gyrate atrophy develop night vision difficulties in their teens and 20s, and sometimes in the first decade.

In the early stages, central vision is good. As the condition advances, central vision deteriorates either from involvement of the maculae in the disease process or from potential complicating factors such as cystoid macular oedema or posterior subcapsular cataract.

Signs

The classical signs are mid-peripheral areas of chorioretinal atrophy that have scalloped edges and are separated from each other by thin bands of pigmentation.

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