Corneal Degeneration
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There are a group of hereditary or congenital degenerations affecting the cornea. A few of them manifest themselves at birth, but mainly they occur in childhood and adolescence. Their onset is slow, and they are sometimes difficult to diagnose without special instruments. Ophthalmologists consider them to be metabolic in origin, most probably the result of an abnormality in one of the enzymes servicing the cornea. Most of them are treatable by corneal grafting (keratoplasty), at least in the short term. But in many cases where a corneal graft has been made, the new cornea begins to exhibit the same symptoms as the original cornea within a few years of the operation, albeit at a slower rate of progression. However, the operation does in most cases ensure at least a few years’ good sight.
The chief symptom of corneal degeneration is corneal opacity. This results not only in the gradual loss of sight, but occasionally in pain as well, and fear of bright light. The commonest degeneration is a condition known as keratoconus, or conical cornea. This usually first presents itself in the patient’s teens, and almost always affects one eye to begin with, though almost certainly the other eye will eventually suffer as well. Here the central area of the cornea, instead of being spherical, gradually assumes a thin conical shape. The concomitant visual distortion cannot be corrected by spectacles, although contact lenses, because they almost completely negate the power of the cornea, can give such eyes good vision, at least for a while. In some cases contact lenses do seem able virtually to halt the degeneration, or at least slow it down, but at least 10 per cent of patients will require a corneal graft within a ten-year period of the onset of the disease, whether or not contact lenses are worn.
Graft operations for keratoconus are successful in almost 90 per cent of cases. The grafted eye will, with the aid either of spectacles or contact lenses, see almost normally in many cases, although it is important to note that the new cornea may not be ready to function for at least a year after surgery. The graft itself is removed from the donor almost immediately after death, and the donor material is transplanted as quickly as possible. It should also be noted that the reasons for corneal grafting vary somewhat from country to country. Thus in Britain keratoplasty is generally performed for keratoconus or for the ravaging complications of viral infections of the cornea, while in the United States it is most commonly used following cataract surgery.
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