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MAJOR REVIEW - Keratoconus

297 MAJOR REVIEW SURVEY OF OPHTHALMOLOGY VOLUME 42 NUMBER 4 JANUARY FEBRUARY 1998 1998 by Elsevier Science $ rights S0039-6257(97)00119-7 Keratoconus YARON S. RABINOWITZ, MD Cornea-Genetic Eye Medical Clinic, Burns and Allen Research Institute, Cedars-Sinai Medical Center and the Department of Ophthalmology, UCLA School of Medicine, Los Angeles, California, USA Abstract. Keratoconus is a bilateral noninflammatory corneal ectasia with an incidence of approxi-mately 1 per 2,000 in the general population. It has well-described clinical signs, but early forms of thedisease may go undetected unless the anterior corneal topography is studied. Early disease is now bestdetected with videokeratography. Classic histopathologic features include stromal thinning, iron depo-sition in the epithelial basement membrane, and breaks in Bowman s layer. Keratoconus is most com-monly an isolated disorder, although several reports describe an association with Down syndrome,Leber s congenital amaurosis, and mitral valve prolapse.

KERATOCONUS 299 ultimately affecting both eyes, although only one eye may be affected initially. 71,105 Symptoms are highly variable and, in part, depend

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