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 its 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 can even 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 20-74. Diabetic retinopathy will occur in 90% of persons with type 1 diabetes (insulin-dependent, or insulin requiring) and 65% of persons with type 2 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 are most often caused by diabetic retinopathy. Among these new cases of blindness, 12% are people between the ages of 20-44 years, and 19% are people between the ages of 45-64 years.
Causes & 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 on in the disease--symptoms are more systemic. 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 is generally 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 does 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 advise 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 need to 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 may then 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.
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 is generally 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 upon the extent of the retinopathy, the cause, and promptness of treatment.
Prevention
Complete eye examinations done regularly can help to 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. Persons with diabetes must take extra care to be sure to have thorough, periodic eye exams, especially if early signs of visual impairment are noticed. 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 will usually occur in diabetics, given enough time. A proper diet, particularly for those persons with diabetes, and stopping smoking will also help delay retinopathy.
Frequent, thorough eye exams and control of systemic disorders are the best prevention.
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).
Further Reading
For Your Information
Books
- Foster, Daniel, W. "Diabetes Mellitus." In Harrison's Principles of Internal Medicine. Edited by Anthony S. Fauci, et al. New York: McGraw-Hill, 1998.
- Horton, Jonathan, C. "Disorders of the Eye." In Harrison's Principles of Internal Medicine. Edited by Anthony S. Fauci, et al. New York: McGraw-Hill, 1998.
Periodicals
- Zoorob, Roger J., and Michael D. Hagen. "Guidelines on the Care of Diabetic Neuropathy, Retinopathy, and Foot Disease." American Family Physician 56 (November 15, 1997): 202.
Organizations
- American Academy of Ophthalmology. P.O. Box 7424, San Francisco CA. 94120-7424. (415) 561-8500. http://www.eyenet.org.
- The American Diabetes Association. National Center. 1660 Duke Street Alexandria, VA 22314. FAX (703) 549-6294. http://www.diabetes.org.
- American Optometric Association. 243 North Lindbergh Blvd., St. Louis, MO 63141. (314) 991-4100. http://www.aoanet.org.
- The 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.prevent-blindness.org.
Gale Encyclopedia of Medicine. Gale Research, 1999.