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Retinopathy is any damage to the
retina The retina (from la, rete "net") is the innermost, light-sensitive layer of tissue of the eye of most vertebrates and some molluscs. The optics of the eye create a focused two-dimensional image of the visual world on the retina, which then ...
of the eyes, which may cause vision impairment. Retinopathy often refers to retinal vascular disease, or damage to the retina caused by abnormal blood flow.
Age-related macular degeneration Macular degeneration, also known as age-related macular degeneration (AMD or ARMD), is a medical condition which may result in blurred or no vision in the center of the visual field. Early on there are often no symptoms. Over time, however, som ...
is technically included under the umbrella term retinopathy but is often discussed as a separate entity. Retinopathy, or retinal vascular disease, can be broadly categorized into proliferative and non-proliferative types. Frequently, retinopathy is an ocular manifestation of systemic disease as seen in
diabetes Diabetes, also known as diabetes mellitus, is a group of metabolic disorders characterized by a high blood sugar level ( hyperglycemia) over a prolonged period of time. Symptoms often include frequent urination, increased thirst and increased ap ...
or
hypertension Hypertension (HTN or HT), also known as high blood pressure (HBP), is a long-term medical condition in which the blood pressure in the arteries is persistently elevated. High blood pressure usually does not cause symptoms. Long-term high bl ...
. Diabetes is the most common cause of retinopathy in the U.S. as of 2008.
Diabetic retinopathy Diabetic retinopathy (also known as diabetic eye disease), is a medical condition in which damage occurs to the retina due to diabetes mellitus. It is a leading cause of blindness in developed countries. Diabetic retinopathy affects up to 80 perc ...
is the leading cause of blindness in working-aged people. It accounts for about 5% of blindness worldwide and is designated a priority eye disease by the World Health Organization.


Signs and symptoms

Many people often do not have symptoms until very late in their disease course. Patients often become symptomatic when there is irreversible damage. Symptoms are usually not painful and can include: *
Vitreous hemorrhage Vitreous hemorrhage is the extravasation, or leakage, of blood into the areas in and around the vitreous humor of the eye. The vitreous humor is the clear gel that fills the space between the lens and the retina of the eye. A variety of conditio ...
*
Floaters Floaters or eye floaters are sometimes visible deposits (e.g., the shadows of tiny structures of protein or other cell debris projected onto the retina) within the eye's vitreous humour ("the vitreous"), which is normally transparent, or between ...
, or small objects that drift through the field of vision * Decreased
visual acuity Visual acuity (VA) commonly refers to the clarity of vision, but technically rates an examinee's ability to recognize small details with precision. Visual acuity is dependent on optical and neural factors, i.e. (1) the sharpness of the retinal ...
* "Curtain falling" over eyes


Pathophysiology

The development of retinopathy can be broken down into proliferative and non-proliferative types. Both types cause disease by altering the normal blood flow to the retina through different mechanisms. The retina is supplied by small vessel branches from the
central retinal artery The central retinal artery (retinal artery) branches off the ophthalmic artery, running inferior to the optic nerve within its dural sheath to the eyeball. Structure The central retinal artery pierces the eyeball close to the optic nerve, sendin ...
. Proliferative retinopathy refers to damage caused by abnormal blood vessel growth. Normally, angiogenesis is a natural part of tissue growth and formation. When there is an unusually high or fast rate of angiogenesis, there is an overgrowth of blood vessels called
neovascularization Neovascularization is the natural formation of new blood vessels ('' neo-'' + '' vascular'' + '' -ization''), usually in the form of functional microvascular networks, capable of perfusion by red blood cells, that form to serve as collateral circu ...
. In the non-proliferative type, abnormal blood flow to the retina occurs due to direct damage or compromise of the blood vessels themselves. Many causes of retinopathy may cause both proliferative and non-proliferative types, though some causes are more associated one type.


Non-proliferative retinopathy

Non-proliferative retinopathy is often caused by direct damage or remodeling of the small blood vessels supplying the retina. Many common causes of non-proliferative damage include
hypertensive retinopathy Hypertensive retinopathy is damage to the retina and retinal circulation due to high blood pressure (i.e. hypertension). Signs and symptoms Most patients with hypertensive retinopathy have no symptoms. However, some may report decreased or blurr ...
,
retinopathy of prematurity Retinopathy of prematurity (ROP), also called retrolental fibroplasia (RLF) and Terry syndrome, is a disease of the eye affecting prematurely born babies generally having received neonatal intensive care, in which oxygen therapy is used due to ...
,
radiation retinopathy Radiation retinopathy is damage to retina due to exposure to ionizing radiation. Radiation retinopathy has a delayed onset, typically after months or years of radiation, and is slowly progressive. In general, radiation retinopathy is seen around 18 ...
,
solar retinopathy Solar may refer to: Astronomy * Of or relating to the Sun ** Solar telescope, a special purpose telescope used to observe the Sun ** A device that utilizes solar energy (e.g. "solar panels") ** Solar calendar, a calendar whose dates indicate t ...
,
sickle cell retinopathy A sickle, bagging hook, reaping-hook or grasshook is a single-handed agricultural tool designed with variously curved blades and typically used for harvesting, or reaping, grain crops or cutting succulent forage chiefly for feeding livestock ...
, and anemic retinopathy (including secondary to
vitamin B12 deficiency Vitamin B12 deficiency, also known as cobalamin deficiency, is the medical condition in which the blood and tissue have a lower than normal level of vitamin B12. Symptoms can vary from none to severe. Mild deficiency may have few or absent symp ...
). There are three main mechanisms of damage in non-proliferative retinopathy: blood vessel damage or remodeling, direct retinal damage, or occlusion of the blood vessels. The first mechanism is indirect damage by altering the blood vessels that supply the retina. In the case of
hypertension Hypertension (HTN or HT), also known as high blood pressure (HBP), is a long-term medical condition in which the blood pressure in the arteries is persistently elevated. High blood pressure usually does not cause symptoms. Long-term high bl ...
, high pressures in the system cause the walls of the artery to thicken, which effectively reduces the amount of blood flow to the retina. This reduction in flow causes tissue ischemia leading to damage.
Atherosclerosis Atherosclerosis is a pattern of the disease arteriosclerosis in which the wall of the artery develops abnormalities, called lesions. These lesions may lead to narrowing due to the buildup of atheroma, atheromatous plaque. At onset there are usu ...
, or hardening and narrowing of blood vessels, also reduces flow to the retina. The second mechanism is direct damage to the retina usually caused by free radicals that causes
oxidative damage Oxidative stress reflects an imbalance between the systemic manifestation of reactive oxygen species and a biological system's ability to readily detoxify the reactive intermediates or to repair the resulting damage. Disturbances in the normal r ...
to the retina itself. Radiation, solar retinopathy, and retinopathy of prematurity fall under this category. The third common mechanism is occlusion of blood flow. This can be caused by either physically blocking the vessels of the retinal artery branches or causing the arteries to narrow. Again, the result is reduced blood flow to the retina causing tissue damage. Sickle cell disease compromises blood flow by causing blood to sludge, or thicken and flow slowly, through the retinal arteries. Other disorders that cause
hyperviscosity syndrome Hyperviscosity syndrome is a group of symptoms triggered by an increase in the viscosity of the blood. Symptoms of high blood viscosity include spontaneous bleeding from mucous membranes, visual disturbances due to retinopathy, and neurologic sympt ...
may also cause blood sludging. Lastly, clots or central artery thrombosis directly blocks flow to the retina causing the cells to die.


Proliferative retinopathy

Proliferative retinopathy is the result of aberrant blood flow to the retina due to blood vessel overgrowth, or neovascularization. These pathologically overgrown blood vessels are often fragile, weak, and ineffective at perfusing the retinal tissues. These weak, fragile vessels are also often leaky, allowing fluids, protein, and other debris to leach out into the retina. They are also prone to
hemorrhage Bleeding, hemorrhage, haemorrhage or blood loss, is blood escaping from the circulatory system from damaged blood vessels. Bleeding can occur internally, or externally either through a natural opening such as the mouth, nose, ear, urethra, v ...
due to their poor strength. This makes proliferative types of retinopathy more risky since vessel hemorrhaging often leads to vision loss and blindness. Many of the causes mentioned in non-proliferative retinopathy may also cause proliferative retinopathy at later stages. Angiogenesis and neovascularization tend to be a later manifestation of non-proliferative retinopathy. Many types of non-proliferative retinopathies result in tissue ischemia or direct retinal damage. The body responds by trying to increase blood flow to damaged retinal tissues.
Diabetes mellitus Diabetes, also known as diabetes mellitus, is a group of metabolic disorders characterized by a high blood sugar level ( hyperglycemia) over a prolonged period of time. Symptoms often include frequent urination, increased thirst and increased ap ...
, which causes
diabetic retinopathy Diabetic retinopathy (also known as diabetic eye disease), is a medical condition in which damage occurs to the retina due to diabetes mellitus. It is a leading cause of blindness in developed countries. Diabetic retinopathy affects up to 80 perc ...
, is the most common cause of proliferative retinopathy in the world.


Other causes

Genetic mutations are rare causes of certain retinopathies and are usually
X-linked Sex linked describes the sex-specific patterns of inheritance and presentation when a gene mutation (allele) is present on a sex chromosome (allosome) rather than a non-sex chromosome (autosome). In humans, these are termed X-linked recessive, ...
including ''NDP'' family of genes causing
Norrie disease Norrie disease is a rare disease and genetic disorder that primarily affects the eyes and almost always leads to blindness. It is caused by mutations in the ''Norrin cystine knot growth factor (NDP)'' gene, which is located on the X chromosome. In ...
, FEVR, and Coats disease among others. There is emerging evidence that there may be a genetic predisposition in patients who develop retinopathy of prematurity and diabetic retinopathy. Trauma, especially to the head, and several diseases may cause Purtscher's retinopathy. Physical exertion like weight lifting and aerobic exercise, coughing, sneezing, straining at stool, vomiting, sexual intercourse, blowing up balloons, blowing musical instruments, cardiopulmonary resuscitation or compression injuries may cause
valsalva retinopathy Valsalva retinopathy is a form of retinopathy due to retinal bleeding secondary to rupture of retinal vessels caused by intrathoracic or intra-abdominal pressure due to physical activities. It can occur in any person irrespective of age, gender, r ...
.


Diagnosis

Retinopathy is diagnosed by an
ophthalmologist Ophthalmology ( ) is a surgery, surgical subspecialty within medicine that deals with the diagnosis and treatment of eye disorders. An ophthalmologist is a physician who undergoes subspecialty training in medical and surgical eye care. Followin ...
or an optometrist during eye examination. The clinician will need to examine the retina, at the back of the eye, to make this diagnosis. There are several ways to examine the retina. The clinician can directly view the retina by looking through the pupil with a light. In most cases, the clinician will dilate the pupil to make for better visualization. Stereoscopic fundus photography is the gold standard for the diagnosis of retinopathy.


Access to care

Telemedicine programs are available that allow primary care clinics to take images using specially designed retinal imaging equipment which can then be shared electronically with specialists at other locations for review. In 2009, Community Health Center, Inc. implemented a telemedicine retinal screening program for low-income patients with diabetes as part of those patients' annual visits at the
Federally Qualified Health Center A Federally Qualified Health Center (FQHC) is a reimbursement designation from the Bureau of Primary Health Care and the Centers for Medicare and Medicaid Services of the United States Department of Health and Human Services. This designation is si ...
.


Prevention

Retinopathy is often secondary to diseases such as diabetes or hypertension. Controlling blood sugar levels and blood pressure have been shown to help decrease incidence of retinopathy. Blood sugar control: If someone has diabetes, or is at high risk for diabetes, it is important for them to have their blood sugar levels checked. The gold standard blood sugar test is the A1C test. Many studies have suggested that lowering A1C levels in someone with elevated A1C levels can lower the incidence and progression of retinopathy. Fortunately, blood sugar control can have benefits beyond just the eye. A primary care physician can help with blood sugar control strategies. Blood pressure control: Controlling blood pressure can also lower the incidence and progression of retinopathy. A primary care physician can help with blood pressure control strategies. Other: Besides blood sugar and blood pressure control, there are other modifications that can help. Regular exercise may help lower the incidence and progression of retinopathy. If someone has sleep apnea, treatment of sleep apnea may help as well.


Treatment

Treatment is based on the cause of the retinopathy and may include laser therapy to the retina. Laser photocoagulation therapy has been the standard treatment for many types of retinopathy. Evidence shows that laser therapy is generally safe and improves visual symptoms in sickle cell and diabetic retinopathy. In recent years targeting the pathway controlling vessel growth or angiogenesis has been promising.
Vascular endothelial growth factor Vascular endothelial growth factor (VEGF, ), originally known as vascular permeability factor (VPF), is a signal protein produced by many cells that stimulates the formation of blood vessels. To be specific, VEGF is a sub-family of growth factors, ...
(VEGF) seems to play a vital role in promoting neovascularization. Using anti-VEGF drugs (antibodies to sequester the growth factor), research have shown significant reduction in the extent of vessel outgrowth. Low quality evidence supports the use of anti-VEGF antibodies, such as bevacizumab or
pegaptanib Pegaptanib sodium injection (brand name Macugen) is an anti-angiogenic medicine for the treatment of neovascular (wet) age-related macular degeneration (AMD). It was discovered by NeXstar Pharmaceuticals (which merged with Gilead Sciences in 1999 ...
which seems to improve outcomes when used in conjunction with laser therapy to treat
retinopathy of prematurity Retinopathy of prematurity (ROP), also called retrolental fibroplasia (RLF) and Terry syndrome, is a disease of the eye affecting prematurely born babies generally having received neonatal intensive care, in which oxygen therapy is used due to ...
, longer term systemic effects are not known however. The evidence is poorer for treatment of diabetic retinopathy. Use of anti-VEGF drugs did not appear to improve outcomes when compared to standard laser therapy for diabetic retinopathy.


Epidemiology

The two most common causes of retinopathy include diabetic retinopathy and retinopathy of prematurity. Diabetic retinopathy affects about 5 million people and retinopathy of prematurity affect about 50,000 premature infants each year worldwide.
Hypertensive retinopathy Hypertensive retinopathy is damage to the retina and retinal circulation due to high blood pressure (i.e. hypertension). Signs and symptoms Most patients with hypertensive retinopathy have no symptoms. However, some may report decreased or blurr ...
is the next most common cause affecting anywhere from 3 to 14% of all non-diabetic adults.


See also

* List of eye diseases and disorders *
List of systemic diseases with ocular manifestations An ocular manifestation of a systemic disease is an eye condition that directly or indirectly results from a disease process in another part of the body. There are many diseases known to cause ocular or visual changes. Diabetes, for example, is th ...


References

{{Medical resources , ICD10 = {{ICD10, H, 35, , h, 30-{{ICD10, H, 35, 2, h, 30 , ICD9 = {{ICD9, 362.0-{{ICD9, 362.2 , SNOMED CT = 35426003 Disorders of choroid and retina