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Macular Degeneration

DESCRIPTION: Hereditary and untreatable group of diseases which affect the macular area of the retina. When the disease affects the choriocappilaris, as in central areolar choroidal sclerosis, there is gradual loss of central vision in middle life. Genetically, this type of macular degeneration is autosomal dominant or recessive. Other types of macular degeneration affect the pigment epithelium. Stargardt-Behr disease is transmitted genetically as an autosomal recessive factor; it results in gradual deterioration of the pigment epithelium, which begins at different ages and progresses at different rates; rate and degree of visual loss varies from family to family. In Best's vitelliform macular degeneration (an autosomal dominant genetic pattern), changes are confined to the macula. Initially, it resembles a poached egg "sunny side up;" later, the "yolk" appears scrambled, and central vision is seriously decreased. (see also Scotomas)

TREATMENT: Untreatable.

IMPLICATIONS: Identification of the type of deterioration may be helpful in educational planning, however, the wide range of resulting visual loss and rate of degeneration makes prediction of final visual status impossible. Regular fundus examination may indicate changes which will have implications for educational and/or rehabilitative planning.

Genetic counseling is indicated.

Eccentric viewing may be observed in an attempt to utilize peripheral input.

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