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April 1988

Variable Expressivity of Autosomal Dominant Microcornea With Cataract

Author Affiliations

From the Departments of Ophthalmology (Drs Salmon and Murray) and Human Genetics (Dr Wallis), University of Cape Town and Groote Schuur Hospital, Cape Town, South Africa.

Arch Ophthalmol. 1988;106(4):505-510. doi:10.1001/archopht.1988.01060130551034

• Autosomal dominant microcornea with a cataract, previously described in four families, was documented in a seven-generation family. Eighteen family members had microcornea and a cataract, and an additional six had sclerocornea or Peters' anomaly. Most individuals with microcornea had a corneal diameter of less than 11 mm in both meridians, with moderately steep corneal curvatures. The inherited cataract progressed to form a total cataract after visual maturity had been achieved. In the four affected children who had not undergone cataract extraction, the common abnormality was a posterior polar lens opacity. The variability of expressivity of the dominant gene would suggest that the embryological origins of microcornea and sclerocornea are similar.

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