Pathology of the hereditary macular dystrophies

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Abstract
THE HEREDITARY macular dystrophies are a small group of disorders, most of which are rarely encountered, that share certain features. The most common presentation is a history of slowly progressive visual loss that occurs early in life and is associated with a bilateral, often symmetric maculopathy. The ophthalmoscopic abnormalities are confined to the macula or posterior pole and visual function outside the central retina is normal. A positive family history should be sought by careful questioning and examination of appropriate family members.

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