OBSOLETE: Keratoconus

Keratoconus is a progressive eye disorder in which the normally round cornea (the clear, dome-shaped front surface of the eye) thins and begins to bulge outward into a cone shape. This abnormal curvature changes the way light enters the eye, causing distorted and blurred vision that cannot be fully corrected with standard eyeglasses. Keratoconus typically begins during puberty or the late teenage years and may progress through the twenties and thirties before stabilizing. It usually affects both eyes, though often asymmetrically. Key symptoms include progressive nearsightedness (myopia), irregular astigmatism, increased light sensitivity (photophobia), glare, and frequent changes in eyeglass prescriptions. In advanced cases, corneal scarring may develop, further impairing vision. Some patients experience episodes of acute corneal hydrops, where fluid suddenly enters the cornea causing pain and a sudden decrease in vision. Note: This Orphanet entry (ORPHA:156071) is classified as OBSOLETE, meaning it may have been reclassified, merged, or reorganized within the Orphanet nomenclature. Keratoconus itself remains a well-recognized clinical entity. Treatment options range from rigid gas-permeable contact lenses and specialty scleral lenses in mild-to-moderate cases, to corneal collagen cross-linking (CXL) to slow or halt disease progression, and corneal transplantation (penetrating or deep anterior lamellar keratoplasty) in advanced cases. Intracorneal ring segments (such as Intacs) may also be used to improve corneal shape. The etiology is considered multifactorial, involving both genetic susceptibility and environmental factors such as chronic eye rubbing and atopic disease.

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