Comprehensive Overview of Visual Impairments

Definition


The terms partially sighted, low vision, legally blind, and totally blind are used in the educational context to describe students with visual impairments. They are defined as follows:

"Partially sighted" indicates some type of visual problem has resulted in a need for special education;

"Low vision" generally refers to a severe visual impairment, not necessarily limited to distance vision. Low vision applies to all individuals with sight who are unable to read the newspaper at a normal viewing distance, even with the aid of eyeglasses or contact lenses. They use a combination of vision and other senses to learn, although they may require adaptations in lighting or the size of print, and, sometimes, braille;

"Legally blind" indicates that a person has less than 20/200 vision in the better eye or a very limited field of vision (20 degrees at its widest point); and

Totally blind students learn via braille or other non-visual media

Visual impairment is the consequence of a functional loss of vision, rather than the eye disorder itself. Eye disorders which can lead to visual impairments can include retinal degeneration, albinism, cataracts, glaucoma, muscular problems that result in visual disturbances, corneal disorders, diabetic retinopathy, congenital disorders, and infection.

Incidence


The rate at which visual impairments occur in individuals under the age of 18 is 12.2 per 1,000. Severe visual impairments (legally or totally blind) occur at a rate of .06 per 1,000.


Characteristics


The effect of visual problems on a child's development depends on the severity, type of loss, age at which the condition appears, and overall functioning level of the child. Many children who have multiple disabilities may also have visual impairments resulting in motor, cognitive, and/or social developmental delays.

A young child with visual impairments has little reason to explore interesting objects in the environment and, thus, may miss opportunities to have experiences and to learn. This lack of exploration may continue until learning becomes motivating or until intervention begins.

Because the child cannot see parents or peers, he or she may be unable to imitate social behavior or understand nonverbal cues. Visual handicaps can create obstacles to a growing child's independence.


Educational Implications

Children with visual impairments should be assessed early to benefit from early intervention programs, when applicable. Technology in the form of computers and low-vision optical and video aids enable many partially sighted, low vision and blind children to participate in regular class activities. Large print materials, books on tape, and braille books are available.

Students with visual impairments may need additional help with special equipment and modifications in the regular curriculum to emphasize listening skills, communication, orientation and mobility, vocation/career options, and daily living skills. Students with low vision or those who are legally blind may need help in using their residual vision more efficiently and in working with special aids and materials. Students who have visual impairments combined with other types of disabilities have a greater need for an interdisciplinary approach and may require greater emphasis on self care and daily living skills.

Specific Visual Disorders

1-Amblyopia

What is amblyopia?

The brain and the eye work together to produce vision. Light enters the eye and is changed into nerve signals that travel along the optic nerve to the brain. Amblyopia is the medical term used when the vision in one of the eyes is reduced because the eye and the brain are not working together properly. The eye itself looks normal, but it is not being used normally because the brain is favoring the other eye. This condition is also sometimes called lazy eye.

How common is amblyopia?

Amblyopia is the most common cause of visual impairment in childhood. The condition affects approximately 2 to 3 out of every 100 children. Unless it is successfully treated in early childhood, amblyopia usually persists into adulthood, and is the most common cause of monocular (one eye) visual impairment among children and young and middle-aged adults.

What causes amblyopia?

Amblyopia may be caused by any condition that affects normal visual development or use of the eyes. Amblyopia can be caused by strabismus, an imbalance in the positioning of the two eyes. Strabismus can cause the eyes to cross in (esotropia) or turn out (exotropia). Sometimes amblyopia is caused when one eye is more nearsighted, farsighted, or astigmatic than the other eye. Occasionally, amblyopia is caused by other eye conditions such as cataract.

How is amblyopia treated in children?

Treating amblyopia involves making the child use the eye with the reduced vision (weaker eye). Currently, there are two ways used to do this:

Atropine
A drop of a drug called atropine is placed in the stronger eye once a day to temporarily blur the vision so that the child will prefer to use the eye with amblyopia. Treatment with atropine also stimulates vision in the weaker eye and helps the part of the brain that manages vision develop more completely.

Patching
An opaque, adhesive patch is worn over the stronger eye for weeks to months. This therapy forces the child to use the eye with amblyopia. Patching stimulates vision in the weaker eye and helps the part of the brain that manages vision develop more completely.

Previously, eye care professionals often thought that treating amblyopia in older children would be of little benefit. However, surprising results from a nationwide clinical trial show that many children age seven through 17 with amblyopia may benefit from treatments that are more commonly used on younger children. This study shows that age alone should not be used as a factor to decide whether or not to treat a child for amblyopia.

2-Anophthalmia and Microphthalmia

What are anophthalmia and microphthalmia?

Anophthalmia and microphthalmia are often used interchangeably. Microphthalmia is a disorder in which one or both eyes are abnormally small, while anophthalmia is the absence of one or both eyes. These rare disorders develop during pregnancy and can be associated with other birth defects.

What causes anophthalmia and microphthalmia?

Causes of these conditions may include genetic mutations and abnormal chromosomes. Researchers also believe that environmental factors, such as exposure to X-rays, chemicals, drugs, pesticides, toxins, radiation, or viruses, increase the risk of anophthalmia and microphthalmia, but research is not conclusive. Sometimes the cause in an individual patient cannot be determined.

Can anophthalmia and microphthalmia be treated?

There is no treatment for severe anophthalmia or microphthalmia that will create a new eye or restore vision. However, some less severe forms of microphthalmia may benefit from medical or surgical treatments. In almost all cases improvements to a child's appearance are possible. Children can be fitted for a prosthetic (artificial) eye for cosmetic purposes and to promote socket growth. A newborn with anophthalmia or microphthalmia will need to visit several eye care professionals, including those who specialize in pediatrics, vitreoretinal disease, orbital and oculoplastic surgery, ophthalmic genetics, and prosthetic devices for the eye. Each specialist can provide information and possible treatments resulting in the best care for the child and family. The specialist in prosthetic diseases for the eye will make conformers, plastic structures that help support the face and encourage the eye socket to grow. As the face develops, new conformers will need to be made. A child with anophthalmia may also need to use expanders in addition to conformers to further enlarge the eye socket. Once the face is fully developed, prosthetic eyes can be made and placed. Prosthetic eyes will not restore vision.

3-Behçet's Disease of the Eye


What is Behçet's disease?

Behçet's disease is an autoimmune disease that results from damage to blood vessels throughout the body, particularly veins. In an autoimmune disease, the immune system attacks and harms the body's own tissues.


What causes Behçet's disease?

The exact cause is unknown. It is believed that an autoimmune reaction may cause blood vessels to become inflamed, but it is not clear what triggers this reaction.

What are the symptoms of Behçet's disease?

Behçet's disease affects each person differently. The four most common symptoms are mouth sores, genital sores, inflammation inside of the eye, and skin problems. Inflammation inside of the eye (uveitis, retinitis, and iritis) occurs in more that half of those with Behçet's disease and can cause blurred vision, pain, and redness.

Other symptoms may include arthritis, blood clots, and inflammation in the central nervous system and digestive organs.

How is Behçet's disease treated?

There is no cure for Behçet's disease. Treatment typically focuses on reducing discomfort and preventing serious complications. Corticosteroids and other medications that suppress the immune system may be prescribed to treat inflammation.

4-What is Blepharitis?


What is Blepharitis?

Blepharitis is a common condition that causes inflammation of the eyelids. The condition can be difficult to manage because it tends to recur.


What causes blepharitis?

Blepharitis occurs in two forms:

Anterior blepharitis affects the outside front of the eyelid, where the eyelashes are attached. The two most common causes of anterior blepharitis are bacteria (Staphylococcus) and scalp dandruff.

Posterior blepharitis affects the inner eyelid (the moist part that makes contact with the eye) and is caused by problems with the oil (meibomian) glands in this part of the eyelid. Two skin disorders can cause this form of blepharitis: acne rosacea, which leads to red and inflamed skin, and scalp dandruff (seborrheic dermatitis).

What are the symptoms of blepharitis?

Symptoms of either form of blepharitis include a foreign body or burning sensation, excessive tearing, itching, sensitivity to light (photophobia), red and swollen eyelids, redness of the eye, blurred vision, frothy tears, dry eye, or crusting of the eyelashes on awakening.

5-Blepharospasm

What is Blepharospasm?

Blepharospasm is an abnormal, involuntary blinking or spasm of the eyelids.

What causes Blepharospasm?

Blepharospasm is associated with an abnormal function of the basal ganglion from an unknown cause. The basal ganglion is the part of the brain responsible for controlling the muscles. In rare cases, heredity may play a role in the development of blepharospasm.

What are the symptoms of Blepharospasm?

Most people develop blepharospasm without any warning symptoms. It may begin with a gradual increase in blinking or eye irritation. Some people may also experience fatigue, emotional tension, or sensitivity to bright light. As the condition progresses, the symptoms become more frequent, and facial spasms may develop. Blepharospasm may decrease or cease while a person is sleeping or concentrating on a specific task.

How is Blepharospasm treated?

To date, there is no successful cure for blepharospasm, although several treatment options can reduce its severity.

In the United States and Canada, the injection of Oculinum (botulinum toxin, or Botox) into the muscles of the eyelids is an approved treatment for blepharospasm. Botulinum toxin, produced by the bacterium Clostridium botulinum, paralyzes the muscles of the eyelids.

Medications taken by mouth for blepharospasm are available but usually produce unpredictable results. Any symptom relief is usually short term and tends to be helpful in only 15 percent of the cases.

Myectomy, a surgical procedure to remove some of the muscles and nerves of the eyelids, is also a possible treatment option. This surgery has improved symptoms in 75 to 85 percent of people with blepharospasm.

Alternative treatments may include biofeedback, acupuncture, hypnosis, chiropractic, and nutritional therapy. The benefits of these alternative therapies have not been proven.

6-Corneal Diseases

Some diseases and disorders of the cornea are:

Allergies: Allergies affecting the eye are fairly common. The most common allergies are those related to pollen, particularly when the weather is warm and dry. Symptoms can include redness, itching, tearing, burning, stinging, and watery discharge, although they are not usually severe enough to require medical attention. Antihistamine decongestant eyedrops can effectively reduce these symptoms, as does rain and cooler weather, which decreases the amount of pollen in the air.

An increasing number of eye allergy cases are related to medications and contact lens wear. Also, animal hair and certain cosmetics, such as mascara, face creams, and eyebrow pencil, can cause allergies that affect the eye. Touching or rubbing eyes after handling nail polish, soaps, or chemicals may cause an allergic reaction. Some people have sensitivity to lip gloss and eye makeup. Allergy symptoms are temporary and can eliminated by not having contact with the offending cosmetic or detergent.


Conjunctivitis (Pink Eye): This term describes a group of diseases that cause swelling, itching, burning, and redness of the conjunctiva, the protective membrane that lines the eyelids and covers exposed areas of the sclera, or white of the eye. Conjunctivitis can spread from one person to another and affects millions of Americans at any given time. Conjunctivitis can be caused by a bacterial or viral infection, allergy, environmental irritants, a contact lens product, eyedrops, or eye ointments.

At its onset, conjunctivitis is usually painless and does not adversely affect vision. The infection will clear in most cases without requiring medical care. But for some forms of conjunctivitis, treatment will be needed. If treatment is delayed, the infection may worsen and cause corneal inflammation and a loss of vision.


Corneal Infections: Sometimes the cornea is damaged after a foreign object has penetrated the tissue, such as from a poke in the eye. At other times, bacteria or fungi from a contaminated contact lens can pass into the cornea. Situations like these can cause painful inflammation and corneal infections called keratitis. These infections can reduce visual clarity, produce corneal discharges, and perhaps erode the cornea. Corneal infections can also lead to corneal scarring, which can impair vision and may require a corneal transplant.

As a general rule, the deeper the corneal infection, the more severe the symptoms and complications. It should be noted that corneal infections, although relatively infrequent, are the most serious complication of contact lens wear.

Minor corneal infections are commonly treated with anti-bacterial eye drops. If the problem is severe, it may require more intensive antibiotic or anti-fungal treatment to eliminate the infection, as well as steroid eye drops to reduce inflammation. Frequent visits to an eye care professional may be necessary for several months to eliminate the problem.


Dry Eye: The continuous production and drainage of tears is important to the eye's health. Tears keep the eye moist, help wounds heal, and protect against eye infection. In people with dry eye, the eye produces fewer or less quality tears and is unable to keep its surface lubricated and comfortable.

The tear film consists of three layers--an outer, oily (lipid) layer that keeps tears from evaporating too quickly and helps tears remain on the eye; a middle (aqueous) layer that nourishes the cornea and conjunctiva; and a bottom (mucin) layer that helps to spread the aqueous layer across the eye to ensure that the eye remains wet. As we age, the eyes usually produce fewer tears. Also, in some cases, the lipid and mucin layers produced by the eye are of such poor quality that tears cannot remain in the eye long enough to keep the eye sufficiently lubricated.

The main symptom of dry eye is usually a scratchy or sandy feeling as if something is in the eye. Other symptoms may include stinging or burning of the eye; episodes of excess tearing that follow periods of very dry sensation; a stringy discharge from the eye; and pain and redness of the eye. Sometimes people with dry eye experience heaviness of the eyelids or blurred, changing, or decreased vision, although loss of vision is uncommon.

Dry eye is more common in women, especially after menopause. Surprisingly, some people with dry eye may have tears that run down their cheeks. This is because the eye may be producing less of the lipid and mucin layers of the tear film, which help keep tears in the eye. When this happens, tears do not stay in the eye long enough to thoroughly moisten it.

Dry eye can occur in climates with dry air, as well as with the use of some drugs, including antihistamines, nasal decongestants, tranquilizers, and anti-depressant drugs. People with dry eye should let their health care providers know all the medications they are taking, since some of them may intensify dry eye symptoms.

People with connective tissue diseases, such as rheumatoid arthritis, can also develop dry eye. It is important to note that dry eye is sometimes a symptom of Sjögren's syndrome, a disease that attacks the body's lubricating glands, such as the tear and salivary glands. A complete physical examination may diagnose any underlying diseases.

Artificial tears, which lubricate the eye, are the principal treatment for dry eye. They are available over-the-counter as eye drops. Sterile ointments are sometimes used at night to help prevent the eye from drying. Using humidifiers, wearing wrap-around glasses when outside, and avoiding outside windy and dry conditions may bring relief. For people with severe cases of dry eye, temporary or permanent closure of the tear drain (small openings at the inner corner of the eyelids where tears drain from the eye) may be helpful.


Fuchs' Dystrophy: Fuchs' dystrophy is a slowly progressing disease that usually affects both eyes and is slightly more common in women than in men. Although doctors can often see early signs of Fuchs' dystrophy in people in their 30s and 40s, the disease rarely affects vision until people reach their 50s and 60s.

Fuchs' dystrophy occurs when endothelial cells gradually deteriorate without any apparent reason. As more endothelial cells are lost over the years, the endothelium becomes less efficient at pumping water out of the stroma. This causes the cornea to swell and distort vision. Eventually, the epithelium also takes on water, resulting in pain and severe visual impairment.

Epithelial swelling damages vision by changing the cornea's normal curvature, and causing a sight-impairing haze to appear in the tissue. Epithelial swelling will also produce tiny blisters on the corneal surface. When these blisters burst, they are extremely painful.

At first, a person with Fuchs' dystrophy will awaken with blurred vision that will gradually clear during the day. This occurs because the cornea is normally thicker in the morning; it retains fluids during sleep that evaporate in the tear film while we are awake. As the disease worsens, this swelling will remain constant and reduce vision throughout the day.

When treating the disease, doctors will try first to reduce the swelling with drops, ointments, or soft contact lenses. They also may instruct a person to use a hair dryer, held at arm's length or directed across the face, to dry out the epithelial blisters. This can be done two or three times a day.

When the disease interferes with daily activities, a person may need to consider having a corneal transplant to restore sight. The short-term success rate of corneal transplantation is quite good for people with Fuchs' dystrophy. However, some studies suggest that the long-term survival of the new cornea can be a problem.

7-Corneal Dystrophies

A corneal dystrophy is a condition in which one or more parts of the cornea lose their normal clarity due to a buildup of cloudy material. There are over 20 corneal dystrophies that affect all parts of the cornea. These diseases share many traits:

  • They are usually inherited.
  • They affect the right and left eyes equally.
  • They are not caused by outside factors, such as injury or diet.
  • Most progress gradually.
  • Most usually begin in one of the five corneal layers and may later spread to nearby layers.
  • Most do not affect other parts of the body, nor are they related to diseases affecting other parts of the eye or body.
  • Most can occur in otherwise totally healthy people, male or female.

Corneal dystrophies affect vision in widely differing ways. Some cause severe visual impairment, while a few cause no vision problems and are discovered during a routine eye examination. Other dystrophies may cause repeated episodes of pain without leading to permanent loss of vision.

Some of the most common corneal dystrophies include Fuchs' dystrophy, keratoconus, lattice dystrophy, and map-dot-fingerprint dystrophy.
 

Herpes Zoster (Shingles): This infection is produced by the varicella-zoster virus, the same virus that causes chickenpox. After an initial outbreak of chickenpox (often during childhood), the virus remains inactive within the nerve cells of the central nervous system. But in some people, the varicella-zoster virus will reactivate at another time in their lives. When this occurs, the virus travels down long nerve fibers and infects some part of the body, producing a blistering rash (shingles), fever, painful inflammations of the affected nerve fibers, and a general feeling of sluggishness.

Varicella-zoster virus may travel to the head and neck, perhaps involving an eye, part of the nose, cheek, and forehead. In about 40 percent of those with shingles in these areas, the virus infects the cornea. Doctors will often prescribe oral anti-viral treatment to reduce the risk of the virus infecting cells deep within the tissue, which could inflame and scar the cornea. The disease may also cause decreased corneal sensitivity, meaning that foreign matter, such as eyelashes, in the eye are not felt as keenly. For many, this decreased sensitivity will be permanent.

Although shingles can occur in anyone exposed to the varicella-zoster virus, research has established two general risk factors for the disease: (1) Advanced age; and (2) A weakened immune system. Studies show that people over age 80 have a five times greater chance of having shingles than adults between the ages of 20 and 40. Unlike herpes simplex I, the varicella-zoster virus does not usually flare up more than once in adults with normally functioning immune systems.

Be aware that corneal problems may arise months after the shingles are gone. For this reason, it is important that people who have had facial shingles schedule follow-up eye examinations.

Iridocorneal Endothelial Syndrome: More common in women and usually diagnosed between ages 30-50, iridocorneal endothelial (ICE) syndrome has three main features: (1) Visible changes in the iris, the colored part of the eye that regulates the amount of light entering the eye; (2) Swelling of the cornea; and (3) The development of glaucoma, a disease that can cause severe vision loss when normal fluid inside the eye cannot drain properly. ICE is usually present in only one eye.

ICE syndrome: This is actually a grouping of three closely linked conditions: iris nevus (or Cogan-Reese) syndrome; Chandler's syndrome; and essential (progressive) iris atrophy (hence the acronym ICE). The most common feature of this group of diseases is the movement of endothelial cells off the cornea onto the iris. This loss of cells from the cornea often leads to corneal swelling, distortion of the iris, and variable degrees of distortion of the pupil, the adjustable opening at the center of the iris that allows varying amounts of light to enter the eye. This cell movement also plugs the fluid outflow channels of the eye, causing glaucoma.

The cause of this disease is unknown. While we do not yet know how to keep ICE syndrome from progressing, the glaucoma associated with the disease can be treated with medication, and a corneal transplant can treat the corneal swelling.


Keratoconus: This disorder--a progressive thinning of the cornea--is the most common corneal dystrophy in the U.S., affecting one in every 2000 Americans. It is more prevalent in teenagers and adults in their 20s. Keratoconus arises when the middle of the cornea thins and gradually bulges outward, forming a rounded cone shape. This abnormal curvature changes the cornea's refractive power, producing moderate to severe distortion (astigmatism) and blurriness (nearsightedness) of vision. Keratoconus may also cause swelling and a sight-impairing scarring of the tissue.

Studies indicate that keratoconus stems from one of several possible causes:

An inherited corneal abnormality. About seven percent of those with the condition have a family history of keratoconus.

An eye injury, i.e., excessive eye rubbing or wearing hard contact lenses for many years.

Certain eye diseases, such as retinitis pigmentosa, retinopathy of prematurity, and vernal keratoconjunctivitis.

Systemic diseases, such as Leber's congenital amaurosis, Ehlers-Danlos syndrome, Down syndrome, and osteogenesis imperfecta.

Keratoconus usually affects both eyes. At first, people can correct their vision with eyeglasses. But as the astigmatism worsens, they must rely on specially fitted contact lenses to reduce the distortion and provide better vision. Although finding a comfortable contact lens can be an extremely frustrating and difficult process, it is crucial because a poorly fitting lens could further damage the cornea and make wearing a contact lens intolerable.

In most cases, the cornea will stabilize after a few years without ever causing severe vision problems. But in about 10 to 20 percent of people with keratoconus, the cornea will eventually become too scarred or will not tolerate a contact lens. If either of these problems occur, a corneal transplant may be needed. This operation is successful in more than 90 percent of those with advanced keratoconus. Several studies have also reported that 80 percent or more of these patients have 20/40 vision or better after the operation.

Lattice Dystrophy: Lattice dystrophy gets its name from an accumulation of amyloid deposits, or abnormal protein fibers, throughout the middle and anterior stroma. During an eye examination, the doctor sees these deposits in the stroma as clear, comma-shaped overlapping dots and branching filaments, creating a lattice effect. Over time, the lattice lines will grow opaque and involve more of the stroma. They will also gradually converge, giving the cornea a cloudiness that may also reduce vision.

In some people, these abnormal protein fibers can accumulate under the cornea's outer layer--the epithelium. This can cause erosion of the epithelium. This condition is known as recurrent epithelial erosion. These erosions: (1) Alter the cornea's normal curvature, resulting in temporary vision problems; and (2) Expose the nerves that line the cornea, causing severe pain. Even the involuntary act of blinking can be painful.

To ease this pain, a doctor may prescribe eye drops and ointments to reduce the friction on the eroded cornea. In some cases, an eye patch may be used to immobilize the eyelids. With effective care, these erosions usually heal within three days, although occasional sensations of pain may occur for the next six-to-eight weeks.

By about age 40, some people with lattice dystrophy will have scarring under the epithelium, resulting in a haze on the cornea that can greatly obscure vision. In this case, a corneal transplant may be needed. Although people with lattice dystrophy have an excellent chance for a successful transplant, the disease may also arise in the donor cornea in as little as three years. In one study, about half of the transplant patients with lattice dystrophy had a recurrence of the disease from between two to 26 years after the operation. Of these, 15 percent required a second corneal transplant. Early lattice and recurrent lattice arising in the donor cornea responds well to treatment with the excimer laser.

Although lattice dystrophy can occur at any time in life, the condition usually arises in children between the ages of two and seven.

Map-Dot-Fingerprint Dystrophy: This dystrophy occurs when the epithelium's basement membrane develops abnormally (the basement membrane serves as the foundation on which the epithelial cells, which absorb nutrients from tears, anchor and organize themselves). When the basement membrane develops abnormally, the epithelial cells cannot properly adhere to it. This, in turn, causes recurrent epithelial erosions, in which the epithelium's outermost layer rises slightly, exposing a small gap between the outermost layer and the rest of the cornea.

Epithelial erosions can be a chronic problem. They may alter the cornea's normal curvature, causing periodic blurred vision. They may also expose the nerve endings that line the tissue, resulting in moderate to severe pain lasting as long as several days. Generally, the pain will be worse on awakening in the morning. Other symptoms include sensitivity to light, excessive tearing, and foreign body sensation in the eye.

Map-dot-fingerprint dystrophy, which tends to occur in both eyes, usually affects adults between the ages of 40 and 70, although it can develop earlier in life. Also known as epithelial basement membrane dystrophy, map-dot-fingerprint dystrophy gets its name from the unusual appearance of the cornea during an eye examination. Most often, the affected epithelium will have a map-like appearance, i.e., large, slightly gray outlines that look like a continent on a map. There may also be clusters of opaque dots underneath or close to the map-like patches. Less frequently, the irregular basement membrane will form concentric lines in the central cornea that resemble small fingerprints.

Typically, map-dot-fingerprint dystrophy will flare up occasionally for a few years and then go away on its own, with no lasting loss of vision. Most people never know that they have map-dot-fingerprint dystrophy, since they do not have any pain or vision loss. However, if treatment is needed, doctors will try to control the pain associated with the epithelial erosions. They may patch the eye to immobilize it, or prescribe lubricating eye drops and ointments. With treatment, these erosions usually heal within three days, although periodic flashes of pain may occur for several weeks thereafter. Other treatments include anterior corneal punctures to allow better adherence of cells; corneal scraping to remove eroded areas of the cornea and allow regeneration of healthy epithelial tissue; and use of the excimer laser to remove surface irregularities.

Ocular Herpes: Herpes of the eye, or ocular herpes, is a recurrent viral infection that is caused by the herpes simplex virus and is the most common infectious cause of corneal blindness in the U.S. Previous studies show that once people develop ocular herpes, they have up to a 50 percent chance of having a recurrence. This second flare-up could come weeks or even years after the initial occurrence.

Ocular herpes can produce a painful sore on the eyelid or surface of the eye and cause inflammation of the cornea. Prompt treatment with anti-viral drugs helps to stop the herpes virus from multiplying and destroying epithelial cells. However, the infection may spread deeper into the cornea and develop into a more severe infection called stromal keratitis, which causes the body's immune system to attack and destroy stromal cells. Stromal keratitis is more difficult to treat than less severe ocular herpes infections. Recurrent episodes of stromal keratitis can cause scarring of the cornea, which can lead to loss of vision and possibly blindness.

Like other herpetic infections, herpes of the eye can be controlled. An estimated 400,000 Americans have had some form of ocular herpes. Each year, nearly 50,000 new and recurring cases are diagnosed in the United States, with the more serious stromal keratitis accounting for about 25 percent. In one large study, researchers found that recurrence rate of ocular herpes was 10 percent within one year, 23 percent within two years, and 63 percent within 20 years. Some factors believed to be associated with recurrence include fever, stress, sunlight, and eye injury.

Pterygium: A pterygium is a pinkish, triangular-shaped tissue growth on the cornea. Some pterygia grow slowly throughout a person's life, while others stop growing after a certain point. A pterygium rarely grows so large that it begins to cover the pupil of the eye.

Pterygia are more common in sunny climates and in the 20-40 age group. Scientists do not know what causes pterygia to develop. However, since people who have pterygia usually have spent a significant time outdoors, many doctors believe ultraviolet (UV) light from the sun may be a factor. In areas where sunlight is strong, wearing protective eyeglasses, sunglasses, and/or hats with brims are suggested. While some studies report a higher prevalence of pterygia in men than in women, this may reflect different rates of exposure to UV light.

Because a pterygium is visible, many people want to have it removed for cosmetic reasons. It is usually not too noticeable unless it becomes red and swollen from dust or air pollutants. Surgery to remove a pterygium is not recommended unless it affects vision. If a pterygium is surgically removed, it may grow back, particularly if the patient is less than 40 years of age. Lubricants can reduce the redness and provide relief from the chronic irritation.

Stevens-Johnson Syndrome: Stevens-Johnson Syndrome (SJS), also called erythema multiforme major, is a disorder of the skin that can also affect the eyes. SJS is characterized by painful, blistery lesions on the skin and the mucous membranes (the thin, moist tissues that line body cavities) of the mouth, throat, genital region, and eyelids. SJS can cause serious eye problems, such as severe conjunctivitis; iritis, an inflammation inside the eye; corneal blisters and erosions; and corneal holes. In some cases, the ocular complications from SJS can be disabling and lead to severe vision loss.

Scientists are not certain why SJS develops. The most commonly cited cause of SJS is an adverse allergic drug reaction. Almost any drug--but most particularly sulfa drugs--can cause SJS. The allergic reaction to the drug may not occur until 7-14 days after first using it. SJS can also be preceded by a viral infection, such as herpes or the mumps, and its accompanying fever, sore throat, and sluggishness. Treatment for the eye may include artificial tears, antibiotics, or corticosteroids. About one-third of all patients diagnosed with SJS have recurrences of the disease.

SJS occurs twice as often in men as women, and most cases appear in children and young adults under 30, although it can develop in people at any age.

8-Retinopathy of Prematurity  
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What is Retinopathy of Prematurity (ROP)?

Retinopathy of prematurity (ROP) is a potentially blinding eye disorder that primarily affects premature infants weighing about 2¾ pounds (1250 grams) or less that are born before 31 weeks of gestation (A full-term pregnancy has a gestation of 38–42 weeks). The smaller a baby is at birth, the more likely that baby is to develop ROP. This disorder—which usually develops in both eyes—is one of the most common causes of visual loss in childhood and can lead to lifelong vision impairment and blindness. ROP was first diagnosed in 1942.

How many infants have ROP?

Today, with advances in neonatal care, smaller and more premature infants are being saved. These infants are at a much higher risk for ROP. Not all babies who are premature develop ROP. There are approximately 3.9 million infants born in the U.S. each year; of those, about 28,000 weigh 2¾ pounds or less. About 14,000–16,000 of these infants are affected by some degree of ROP. The disease improves and leaves no permanent damage in milder cases of ROP. About 90 percent of all infants with ROP are in the milder category and do not need treatment. However, infants with more severe disease can develop impaired vision or even blindness. About 1,100–1,500 infants annually develop ROP that is severe enough to require medical treatment. About 400–600 infants each year in the US become legally blind from ROP.

What causes ROP?

ROP occurs when abnormal blood vessels grow and spread throughout the retina, the tissue that lines the back of the eye. These abnormal blood vessels are fragile and can leak, scarring the retina and pulling it out of position. This causes a retinal detachment. Retinal detachment is the main cause of visual impairment and blindness in ROP.

Several complex factors may be responsible for the development of ROP. The eye starts to develop at about 16 weeks of pregnancy, when the blood vessels of the retina begin to form at the optic nerve in the back of the eye. The blood vessels grow gradually toward the edges of the developing retina, supplying oxygen and nutrients. During the last 12 weeks of a pregnancy, the eye develops rapidly. When a baby is born full-term, the retinal blood vessel growth is mostly complete (The retina usually finishes growing a few weeks to a month after birth). But if a baby is born prematurely, before these blood vessels have reached the edges of the retina, normal vessel growth may stop. The edges of the retina—the periphery—may not get enough oxygen and nutrients.

Scientists believe that the periphery of the retina then sends out signals to other areas of the retina for nourishment. As a result, new abnormal vessels begin to grow. These new blood vessels are fragile and weak and can bleed, leading to retinal scarring. When these scars shrink, they pull on the retina, causing it to detach from the back of the eye.

Are there different stages of ROP?

Yes. ROP is classified in five stages, ranging from mild (stage I) to severe (stage V):

Stage I — Mildly abnormal blood vessel growth. Many children who develop stage I improve with no treatment and eventually develop normal vision. The disease resolves on its own without further progression.

Stage II — Moderately abnormal blood vessel growth. Many children who develop stage II improve with no treatment and eventually develop normal vision. The disease resolves on its own without further progression.

Stage III — Severely abnormal blood vessel growth. The abnormal blood vessels grow toward the center of the eye instead of following their normal growth pattern along the surface of the retina. Some infants who develop stage III improve with no treatment and eventually develop normal vision. However, when infants have a certain degree of Stage III and "plus disease" develops, treatment is considered. "Plus disease" means that the blood vessels of the retina have become enlarged and twisted, indicating a worsening of the disease. Treatment at this point has a good chance of preventing retinal detachment.

Stage IV — Partially detached retina. Traction from the scar produced by bleeding, abnormal vessels pulls the retina away from the wall of the eye.

Stage V — Completely detached retina and the end stage of the disease. If the eye is left alone at this stage, the baby can have severe visual impairment and even blindness.

Most babies who develop ROP have stages I or II. However, in a small number of babies, ROP worsens, sometimes very rapidly. Untreated ROP threatens to destroy vision.

How is ROP treated?

The most effective proven treatments for ROP are laser therapy or cryotherapy. Laser therapy "burns away" the periphery of the retina, which has no normal blood vessels. With cryotherapy, physicians use an instrument that generates freezing temperatures to briefly touch spots on the surface of the eye that overlie the periphery of the retina. Both laser treatment and cryotherapy destroy the peripheral areas of the retina, slowing or reversing the abnormal growth of blood vessels. Unfortunately, the treatments also destroy some side vision. This is done to save the most important part of our sight—the sharp, central vision we need for "straight ahead" activities such as reading, sewing, and driving.

Both laser treatments and cryotherapy are performed only on infants with advanced ROP, particularly stage III with "plus disease." Both treatments are considered invasive surgeries on the eye, and doctors don't know the long-term side effects of each.

In the later stages of ROP, other treatment options include:

Scleral buckle. This involves placing a silicone band around the eye and tightening it. This keeps the vitreous gel from pulling on the scar tissue and allows the retina to flatten back down onto the wall of the eye. Infants who have had a sclera buckle need to have the band removed months or years later, since the eye continues to grow; otherwise they will become nearsighted. Sclera buckles are usually performed on infants with stage IV or V.

Vitrectomy. Vitrectomy involves removing the vitreous and replacing it with a saline solution. After the vitreous has been removed, the scar tissue on the retina can be peeled back or cut away, allowing the retina to relax and lay back down against the eye wall. Vitrectomy is performed only at stage V.



Resources


American Foundation for the Blind. Search AFB's Service Center on the Web to identify services for blind and visually impaired persons in the United States and Canada. Available: www.afb.org/services.asp

Holbrook, M.C. (Ed.). (1996). Children with visual impairments: A parents' guide. Bethesda, MD: Woodbine. (Telephone: 800.843.7323; 301.897.3570. Web: www.woodbinehouse.com)

Lewis, S., & Allman, C.B. (2000). Seeing eye to eye: An administrator's guide to students with low vision. New York: American Foundation for the Blind. (Telephone: 800.232.3044. Web: www.afb.org)

National Eye Institute. (2003, December). Eye health organizations list. (Available online at: http://www.nei.nih.gov/health/resourceAlpha.asp)

 
Organizations


American Council of the Blind
1155 15th St. N.W., Suite 1004
Washington, D.C. 20005
202.467.5081; 800.424.8666
Email: info@acb.org
Web: www.acb.org

American Foundation for the Blind
11 Penn Plaza, Suite 300
New York, NY 10001
800.232.5463 (Hotline)
For publications, call: 800.232.3044
Email: afbinfo@afb.net
Web: www.afb.org

Blind Children’s Center
4120 Marathon Street
Los Angeles, CA 90029-0159
323.664.2153; 800.222.3566
Email: info@blindchildrenscenter.org
Web: www.blindchildrenscenter.org

National Association for Parents of the Visually Impaired, Inc.
P.O. Box 317
Watertown, MA 02472-0317
617.972.7441; 800.562.6265
Email: napvi@perkins.org
Web: www.napvi.org

National Association for Visually Handicapped
22 West 21st Street, 6th Floor
New York, NY 10010
212.889.3141
Email: staff@navh.org
Web: www.navh.org

National Braille Association, Inc. (NBA)
3 Townline Circle
Rochester, NY 14623-2513
585.427.8260
Email: nbaoffice@nationalbraille.org
Web: www.nationalbraille.org

National Braille Press
88 St. Stephen Street
Boston, MA 02115
617.266.6160; 800.548.7323
Email: orders@nbp.org
Web: www.nbp.org

National Eye Institute
31 Center Drive, MSC 2510
Bethesda, MD 20892-2510
301.496.5248
Email: 2020@nei.nih.gov
Web: www.nei.nih.gov

National Federation of the Blind, Parents Division
1800 Johnson Street
Baltimore, MD 21230
410.659.9314, ext. 360
Email: nfb@nfb.org
Web: www.nfb.org/nopbc.htm

National Library Service for the Blind and Physically Handicapped
Library of Congress
1291 Taylor Street, N.W.
Washington, D.C. 20011
202.707.5100; 202.707.0744 (TTY); 800.424.8567 (Toll Free)
Email: nls@loc.gov
Web: www.loc.gov/nls

Prevent Blindness America
500 E. Remington Road
Schaumburg, IL 60173
847.843.2020; 800.221.3004 (Toll Free)
Email: info@preventblindness.org
Web: www.preventblindness.org

The Foundation Fighting Blindness (formerly the National
Retinitis Pigmentosa Foundation)

11435 Cronhill Drive
Owings Mills, MD 21117-2220
888.394.3937; 800.683.5551 (TTY)
410.568.0150; 410.363.7139 (TTY)
Email: info@blindness.org
Web: www.blindness.org

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