Keratoconus With Corneal Hydrops

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This 20-year-old woman with Down syndrome has bilateral keratoconus, a common, noninflammatory, paracentral corneal ectasia that is occasionally hereditary. Symptoms vary from none to severely blurred vision. Opacity of this patient's right cornea developed after her eye had teared excessively for 1 day, during which time she continually rubbed it.

This 20-year-old woman with Down syndrome has bilateral keratoconus (A), a common, noninflammatory, paracentral corneal ectasia that is occasionally hereditary. Symptoms vary from none to severely blurred vision. Opacity of this patient's right cornea (B) developed after her eye had teared excessively for 1 day, during which time she continually rubbed it.

Keratoconus can be associated with Down syndrome, atopic dermatitis, or vernal catarrh, all of which are frequently accompanied by eye rubbing. This condition is also associated with retinitis pigmentosa, certain types of retinal degeneration, aniridia, and Marfan syndrome. Any resulting visual defect usually can be corrected with glasses or soft contact lenses; keratoplasty is a measure of last resort.

Corneal hydrops occurs when Descemet's membrane stretches and ruptures, allowing aqueous humor to flow into the stroma. Drs Robert P. Blereau, MD and F. H. Metz, Jr, of Morgan City, La, prescribed a cortisone ophthalmic ointment and referred the patient to an ophthalmologist for further treatment.