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Posterior Keratoconus Associated With Systemic Abnormalities
Barbara W. Streeten, MD;
Alice G. Karpik, MD;
Kenneth H. Spitzer, MD
Arch Ophthalmol. 1983;101(4):616-622.
Abstract
• A 16-year-old girl with bilateral congenital posterior keratoconus of the localized type had corneal transplantation performed on one eye. Around the scarred central corneal cone there was a ring of Descemet's membrane with large knoblike excrescences. A small anterior subcapsular cataract was also present in this eye. The pattern of corneal scarring, as seen by electron microscopy, suggested an iridocorneal or iridocorneolenticular adhesion present at the 8- to 20-mm embryonic stage. Associated systemic defects, which could be dated to the same period, were median facial clefting and severe genitourinary abnormalities. Several external features, such as short stature, abnormal gait, broad nose, and upward displacement of the lateral canthi, were similar to those in the Haney-Falls syndrome of familial posterior keratoconus. No appreciable chromosomal abnormalities were found.
Author Affiliations
From the Departments of Ophthalmology (Drs Streeten and Spitzer) and Pathology (Drs Streeten and Karpik), State University Hospital, Upstate Medical Center, Syracuse, NY. Dr Karpik is now with Rush-Presbyterian-St Luke's Medical Center, Chicago.
Footnotes
Accepted for publication May 25, 1982.
Read before the Verhoeff Society, Washington, DC, April 18, 1977.
Reprint requests to Department of Pathology, Upstate Medical Center, Syracuse, NY 13210 (Dr Streeten).
This study was supported in part by Public Health Service grant EY01602 from the National Eye Institute.
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