Retinopathies


Retinopathies

 

Definition

Retinopathy is a noninflammatory disease of the retina. There are many causes and types of retinopathy.

Description

The retina is the thin membrane that lines the back of the eye and contains light-sensitive cells (photoreceptors). Light enters the eye and is focused onto the retina. The photoreceptors send a message to the brain via the optic nerve. The brain then "interprets" the electrical message sent to it, resulting in vision. The macula is a specific area of the retina responsible for central vision. The fovea is about 1.5 mm in size and located in the macula. The fovea is responsible for sharp vision. When looking at something, the fovea should be directed at the object.Retinopathy, or damage to the retina, has various causes. A hardening or thickening of the retinal arteries is called arteriosclerotic retinopathy. High blood pressure in the arteries of the body can damage the retinal arteries and is called hypertensive retinopathy. The spreading of a syphilis infection to the retinal blood vessels cases syphilitic retinopathy, and diabetes damages the retinal vessels resulting in a condition called diabetic retinopathy. Sickle cell anemia also affects the blood vessels in the eyes. Exposure to the sun (or looking at the sun during an eclipse) can cause damage (solar retinopathy), as well as certain drugs (for example, chloroquine, thioridazine, and large doses of tamoxifen). The arteries and veins can become blocked, thus resulting in a retinal artery or vein occlusion. These are just some of the causes of the various retinopathies.Retinopathies are divided into two broad categories, simple or nonproliferative retinopathies and proliferative retinopathies. The simple retinopathies include the defects identified by bulging of the vessel walls, by bleeding into the eye, by small clumps of dead retinal cells called cotton wool exudates, and by closed vessels. This form of retinopathy is considered mild. The proliferative, or severe, forms of retinopathies include the defects identified by newly grown blood vessels, by scar tissue formed within the eye, by closed-off blood vessels that are badly damaged, and by the retina breaking away from the mesh of blood vessels that nourish it (retinal detachment).While each disease has its own specific effect on the retina, a general scenario for many of the retinopathies is as follows (note: not all retinopathies necessarily affect the blood vessels). Blood flow to the retina is disrupted, either by blockage or breakdown of the various vessels. This can lead to bleeding (hemorrhage) and fluids, cells, and proteins leaking into the area (exudates). There can be a lack of oxygen to surrounding tissues (hypoxia) or decreased blood flow (ischemia). Chemicals produced by the body then can cause new blood vessels to grow (neovascularization), however, these new vessels generally leak and cause more problems. Neovascularization even can grow on the colored part of the eye (iris). The retina can swell and vision will be affected.Diabetic retinopathy is the leading cause of blindness in people ages 20 to 74. Diabetic retinopathy will occur in 90% of persons with type 1 diabetes (insulin-dependent, or insulin requiring) and 65% of persons with type II diabetes (non-insulin-dependent, or not requiring insulin) by about 10 years after the beginning of diabetes. In the United States, new cases of blindness most often are caused by diabetic retinopathy. Among these new cases of blindness, 12% are people between the ages of 20 to 44 years, and 19% are people between the ages of 45 to 64 years.

Causes and symptoms

There are many causes of retinopathy. Some of the more common ones are listed below.

Diabetic retinopathy

Diabetes is a complex disorder characterized by an inability of the body to properly regulate the levels of sugar and insulin (a hormone made by the pancreas) in the blood. As diabetes progresses, the blood vessels that feed the retina become damaged in different ways. The damaged vessels can have bulges in their walls (aneurysms); they can leak blood into the surrounding jelly-like material (vitreous) that fills the inside of the eyeball; they can become completely closed; or new vessels can begin to grow where there would not normally be blood vessels. However, although these new blood vessels are growing in the eye, they cannot nourish the retina and they bleed easily, releasing blood into the inner region of the eyeball, which can cause dark spots and cloudy vision.Diabetic retinopathy begins prior to any outward signs of disease being noticed. Once symptoms are noticed, they include poorer than normal vision, fluctuating or distorted vision, cloudy vision, dark spots, episodes of temporary blindness, or permanent blindness.

Hypertensive retinopathy

High blood pressure can affect the vessels in the eyes. Some blood vessels can narrow. The blood vessels can thicken and harden (arteriosclerosis). There will be flame-shaped hemorrhages and macular swelling (edema). This edema may cause distorted or decreased vision.

Sickle cell retinopathy

Sickle cell anemia occurs mostly in blacks and is a hereditary disease that affects the red blood cells. The sickle-shaped blood cell reduces blood flow. People will not have visual symptoms early in the disease. However, patients need to be followed closely in case neovascularization occurs.

Retinal vein and artery occlusion

Retinal vein occlusion generally occurs in the elderly. There is usually a history of other systemic disease, such as diabetes or high blood pressure. The central retinal vein (CRV), or the retinal veins branching off of the CRV, can become compressed, thus stopping the drainage of blood from the retina. This may occur if the central retinal artery hardens.Symptoms of retinal vein occlusion include a sudden, painless loss of vision or field of vision in one eye. There may be a sudden onset of floating spots (floaters) or flashing lights. Vision may be unchanged or decrease dramatically.Retinal artery occlusion generally is the result of an embolism that dislodges from somewhere else in the body and travels to the eye. Transient loss of vision may precede an occlusion. Symptoms of a central retinal artery or branch occlusion include a sudden, painless loss of vision or decrease in visual field. Ten percent of the cases of a retinal artery occlusion occur because of giant cell arteritis (a chronic vascular disease).

Solar retinopathy

Looking directly at the sun or watching an eclipse can cause damage. There may be a loss of the central visual field or decreased vision. The symptoms can occur hours to days after the incident.

Drug-related retinopathies

Certain medications can affect different areas of the retina. Doses of 20-40 mg a day of tamoxifen usually do not cause a problem, but much higher doses may cause irreversible damage.Patients taking chloroquine for lupus, rheumatoid arthritis, or other disorders may notice a decrease in vision. If so, discontinuing medication will stop, but not reverse, any damage. However, patients should never discontinue medication without the advice of their physician.Patients taking thioridazine may notice a decrease in vision or color vision.These drug-related retinopathies generally only affect patients taking large doses. However, patients need to be aware if any medication they are taking will affect the eyes. Patients should inform their doctors of any visual effects.

Diagnosis

The damaged retinal blood vessels and other retinal changes are visible to an eye doctor when an examination of the retina (fundus exam) is done. This can be done using a hand-held instrument called an ophthalmoscope or another instrument called a binocular indirect ophthalmoscope. This allows the doctor to see the back of the eye. Certain retinopathies have classic signs (for example, vascular "sea fans" in sickle cell, dot and blot hemorrhages in diabetes, flame-shaped hemorrhages in high blood pressure). Patients then may be referred for other tests to confirm the underlying cause of the retinopathy. These tests include blood tests and measurement of blood pressure.Fluorescein angiography, where a dye is injected into the patient and the back of the eyes are viewed and photographed, helps to locate leaky vessels. Sometimes patients may become nauseated from the dye.A newer diagnostic method called digital retinal photography can be used to screen those at high risk for retinopathies, in particular, diabetics. Some researchers say the technique could lead to more cost-effective screening for people with diabetic retinopathy.

Treatment

Retinal specialists are ophthalmologists who specialize in retinal disorders. Retinopathy is a disorder of the retina that can result from different underlying systemic causes, so general physicians should be consulted as well. For drug-related retinopathies, the treatment generally is discontinuation of the drug (only under the care of a physician).Surgery with lasers can help to prevent blindness or lessen any losses in vision. The high-energy light from a laser is aimed at the weakened blood vessels in the eye, destroying them. Scars will remain where the laser treatment was performed. For that reason, laser treatment cannot be performed everywhere. For example, laser photocoagulation at the fovea would destroy the area for sharp vision. Panretinal photocoagulation may be performed. This is a larger area of treatment in the periphery of the retina; hopefully it will decrease neovascularization. Prompt treatment of proliferative retinopathy may reduce the risk of severe vision loss by 50%.Patients with retinal artery occlusion should be referred to a cardiologist. Patients with retinal vein occlusion need to be referred to a physician, as they may have an underlying systemic disorder, such as high blood pressure.

Prognosis

Nonproliferative retinopathy has a better prognosis than proliferative retinopathy. Prognosis depends on the extent of the retinopathy, the cause, and promptness of treatment.

Prevention

Complete eye examinations done regularly can help detect early signs of retinopathy. Patients on certain medications should have more frequent eye exams. They also should have a baseline eye exam when starting the drug. People with diabetes must take extra care to have thorough, periodic eye exams, especially if early signs of visual impairment are noticed. A 2003 report recommended re-screening eye exams every two years for diabetics whose blood sugar had remained in control, and more frequent exams if visual symptoms appear. Anyone experiencing a sudden loss of vision, decrease in vision or visual field, flashes of light, or floating spots should contact their eye doctor right away.Proper medical treatment for any of the systemic diseases known to cause retinal damage will help prevent retinopathy. For diabetics, maintaining proper blood sugar and blood pressure levels is important as well; however, some form of retinopathy usually will occur in diabetics, given enough time. A proper diet, particularly for those persons with diabetes, and stopping smoking also will help delay retinopathy.Frequent, thorough eye exams and control of systemic disorders are the best prevention.

Resources

Periodicals

Selby, Joe, Lynn Ackerson, and Talmadge Cooper. "Three-year Incidence of Treatable Diabetic Eye Disease After Negative Funduscopic Examination." Diabetes June 2003: A60.Usher, David, et al. "Automated Detection of Diabetic Retinopathy in Digital Retinal Images." Diabetes June 2003: A204.

Organizations

American Academy of Ophthalmology. 655 Beach Street, P.O. Box 7424, San Francisco, CA 94120-7424. http://www.eyenet.org.American Diabetes Association. 1701 North Beauregard Street, Alexandria, VA 22311. (800) 342-2383. http://www.diabetes.org.American Optometric Association. 243 North Lindbergh Blvd., St. Louis, MO 63141. (314) 991-4100. http://www.aoanet.org.Foundation Fighting Blindness. Executive Plaza I, Suite 800, 11350 McCormick Road, Hunt Valley, MD 21031-1014. (888) 394-3937. http://www.blindness.org.Prevent Blindness America. 500 East Remington Road, Schaumburg, IL 60173. (800) 331-2020. http://www.preventblindness.org.

Key terms

Exudate — Cells, protein, fluid, or other material that passes through blood vessel walls to accumulate in the surrounding tissue.Neovascularization — New blood vessel formation-usually leaky vessels.Nonproliferative retinopathy — Retinopathy without the growth of new blood vessels.Proliferative retinopathy — Retinopathy with the growth of new blood vessels (neovascularization).