- by Dr Alok B S
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- Jul 11 2017
Corneal Dystrophy — An unusual but alarming eye condition
A corneal dystrophy is a scarce genetic eye disorder in which one or more parts of the clear outer layer of the eye (the cornea) no longer have their normal clarity as a result of an accumulation of cloudy substance. The general term corneal dystrophy refers to a group of corneal diseases.
There are many kinds of corneal dystrophies, and they are distinguished by the particular part or parts of the cornea influenced.
The cornea is made up of 5 layers: an outer layer (the epithelium), 4 middle layers (Dua’s layer, Bowman’s layer, the stroma, and Descemet’s membrane), and an inner layer (the endothelium).
These layers shield the eye from infectious or irritating substance, and they work as a refractive medium that flexes incoming light toward the inner lens, where the light is directed to the retina, which transforms it into images to be sent to the brain.
A blurred cornea can hinder with the eye’s capacity to focus incoming light, and thus may impair vision.
Some individuals with corneal dystrophies may have no indications, whereas others may have notably impaired vision. The distinct symptoms can differ, depending on the type and the age at which the disorder develops. However, there are various characteristics that all corneal dystrophies share:
They are generally inherited
They are bilateral -they influence the right and left eyes equally
They are not caused by exterior influences, such as diet or injury
Most advance slowly
Majority do not damage other areas of the body
Apart from that most can occur in healthy individuals, female or male (except for Fuchs corneal dystrophy, which affects women about 3 times as often as men)
Common symptoms of corneal dystrophy
Although a corneal dystrophy may not cause indications at first, there is always the chance that it could in the future. The build-up of scar tissue or foreign substance in one or more of the layers of the cornea, which arises with all genetic disorder of the cornea, may cause it to lose its clarity, possibly causing loss of vision or blurred vision.
Most people with corneal dystrophies have irregular-shaped corneal opacities in their corneas (an opacity is an area that is non transparent. These may be seen by the naked eye, but are more often noticeable only by a specialist during an eye examination.
Many forms of corneal dystrophy are distinguished by repeated corneal abrasion. In this disorder, the epithelium, the outermost layer of the cornea, repeatedly fails to attach to the eye properly.
People with recurrent corneal abrasions may have uneasiness or extreme pain, an unusual sensitivity to light (photophobia), the feeling of a foreign body (such as dirt or an eyelash) in the eye, or muddy vision.
Causes and types of corneal dystrophy
Corneal dystrophy is an inherited genetic disorder. Categorization of corneal dystrophies is based on clinical, genetic and clinical details. Clinically, the corneal inherited disease can be divided into 3 groups, based on the anatomical position of the deformities. These 3 groups are:
Superficial Corneal Dystrophies
Corneal Stromal Dystrophies
Posterior Corneal Dystrophies
Your specialist may find a corneal dystrophy by accident during a routine eye examination. Your diagnosis will depend in part on the age at which your signs developed and the clinical appearance of your cornea when detected under a slit-lamp, a special microscope used to view the eye.
To confirm the diagnosis, your doctor will perform a clinical assessment during which you will be asked to give a detailed medical history — including your family medical history — and undergo a range of tests. One test may involve taking away of a piece of corneal tissue so that it can be examined.
Certain corneal dystrophies can be diagnosed with molecular genetic tests even before signs develop. Genetic guidance can usually be provided with detailed information about your particular corneal dystrophy.
Treating Corneal Dystrophy
How you are treated will depend on what kind of corneal dystrophy you have, how critical your symptoms are, how far your disease has advanced, and your overall health and quality of life.
If you have no signs or only moderate signs, your doctor may decide to postpone treatment and follow up with you regularly to watch the progression of the disease.
Traditional treatments for this disorder may include eye drops and ointments. Repeated corneal abrasions may be treated with moisturizing eye drops, ointments, hypertonic salt drops, antibiotics, or bandage contact lenses.
For repeated corneal erosions that continue despite traditional treatment, your physician may suggest corneal scraping or the use of excimer laser therapy, which can remove deformity from the surface of the cornea. This method is called phototherapeutic keratectomy.
If your symptoms are drastic or advanced, a corneal transplant, also known as a keratoplasty, may be essential. Even though corneal transplants are effective for treating symptoms of corneal dystrophies, there is a possibility that the contributed (graft) cornea may finally be affected by the disease.
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