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Fuch's dystrophy is a dominantly inherited condition affecting the cornea. Statistically, half of the members of a family may be affected ;
however , the expression of severity is variable and visual acuity may or may not be disturbed. It is a major cause for corneal transplantation.
Women are more commonly affected then men , and Fuch's dystrophy becomes apparent only after the fifth decade of life , gradually getting
worse with age
The cornea is the ' clear window ' in the front of the eye that brings light rays into the eye. The cornea is composed of three layers -the
outer epithelium , a tough middle layer called the stroma , and a delicate single cellular , inner lining called the corneal endothelium. The corneal endothelium functions as a barrier to prevent water inside the
eyeball from moving into and hydrating the cornea. If the corneal endothelium does not function normally , then water will migrate into the cornea , and corneal clouding will develop - thus blurring vision. The more
corneal swelling or edema , the more severe the vision is blurred. Corneal edema may develop to the point were the surface epithelium forms blisters that intermittently rupture , causing sharp pain - a condition
termed ' bullous keratopathy '.
Corneal endothelial cells do not divide or replace themselves. As we all get older , we gradually lose corneal endothelial cells through
individual cell attrition. Fortunately , most people have enough reserve endothelial cells throughout life to prevent corneal edema. For unknown reasons , patients with Fuch's dystrophy have fewer corneal
endothelial cells , which die off faster , eventually falling below a critical cell density needed to prevent corneal edema.
Anything that damages corneal endothelium - including inflammation inside the eye ( uveitis ) , extremely high-pressure inside the eye (
acute glaucoma ) , or trauma from intraocular surgery can hasten further loss of corneal endothelial cells. During cataract removal surgery , small bits of cataract fragments may hit the endothelium , which along
with irritating fluid circulating inside the eye can damage the corneal endothelium further. For patients with Fuch's dystrophy , combining cataract surgery with corneal transplantation may be necessary.
Treatment of Fuch's dystrophy becomes necessary only when the vision becomes blurred to the extent that the patient is having trouble
seeing. Concentrated saltwater eye drops can dehydrate the cornea and improve vision in the early stages. Also , blowing dry de-humidified air across the cornea with a hair dryer can sometimes help. If corneal
blisters develop , soft bandage contact lenses can provide some pain relief. Eventually , corneal transplantation surgery may be required to restore vision in both patients with severe Fuch's dystrophy. Fuch's
dystrophy is a leading cause of corneal transplantation in the United States.
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