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Gerhard W. Cibis, MD; Martha Morey, MS; David J. Harris, MD

Arch Ophthalmol. 1980;98(10):1785-1789.

Abstract
• A family had dominantly inherited macular dystrophy with flecks and without evidence of major cone dysfunction. This family shows that the clinical picture of Stargardt's disease can result from at least two different genes: one with dominant and another with recessive heredity. Some patients had fundus flavimaculatus flecks of Stargardt's disease; others did not. In our opinion progressive atrophic macular (foveal) dystrophy as Stargardt's disease without flecks is not a separate clinical entity but part of the same genetic defect. Furthermore, a dominant hereditary pattern in patients without flecks or major cone dysfunction is insufficient evidence for classification of such patients as having cone dystrophy.

Author Affiliations
From the Children's Mercy Hospital Departments of Ophthalmology (Dr Cibis) and Genetics (Ms Morey and Dr Harris), University of Missouri School of Medicine, Kansas City.

Footnotes
Accepted for publication Dec 4, 1979.

Reprint requests to 4620 J. C. Nichols Pkwy, No. 421, Kansas City, MO 64112 (Dr Cibis).

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