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James R. Boynton, MD; Thomas R. Pheasant, MD; Bruce L. Johnson, MD; Daniel B. Levin, MD; Barbara W. Streeten, MD

Arch Ophthalmol. 1979;97(5):896-900.

Abstract
• In 1966, Kenny described two patients with an unusual congenital syndrome including dwarfism, thickened long bone cortex, transient hypocalcemia, and normal intelligence. These and other patients previously were incorrectly described as "myopic." Ocular findings in four subjects ranged from uncomplicated nanophthalmos with hyperopia to extreme pseudopapilledema, vascular tortuosity, and macular crowding. Postmortem findings from one patient showed calcium deposits demonstrable only by special histochemical stains that were distributed uniquely in the cornea. This distribution differed greatly from the pattern seen in band keratopathy. Retinal calcification was also an unusual feature. Because one patient exhibited a pseudodoubling of the optic papilla, the literature was reviewed. We conclude that no convincing case of true doubling of the optic nerve has been described. Ophthalmologists should be alert for undiagnosed electrolyte abnormalities, especially hypocalcemia, in patients with Kenny's syndrome.

Author Affiliations
From the Department of Ophthalmology, Warsaw Medical Group, PC, Warsaw, NY (Drs Boynton and Levin); Department of Ophthalmology, University of Rochester, Rochester, NY (Dr Boynton); Division of Ophthalmology, Retina Service, Milton S. Hershey Medical Center, Hershey, Pa (Dr Pheasant); Department of Pathology, University of Pittsburgh School of Medicine (Dr Johnson); and State University of New York, Upstate Medical Center, Syracuse (Dr Streeten).

Footnotes
Accepted for publication Nov 13, 1978.

Reprint requests to Warsaw Medical Group, PC, Warsaw, NY 14569 (Dr Boynton).

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