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Deep Learning Technology: Sebastian Arnold, Betty van Aken, Paul Grundmann, Felix A. Gers and Alexander Löser. Learning Contextualized Document Representations for Healthcare Answer Retrieval. The Web Conference 2020 (WWW'20)
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Childhood blindness is an important cause contributing to the burden of blindness. Blindness in children can be defined as a visual acuity of <3/60 in the eye with better vision of a child under 16 years of age. This generally means that the child cannot see something three feet (about one meter) away, that another child could see if it was 60 feet (about 20 meters) away.
There are many causes of blindness in children. Blindness may be due to genetic mutations, birth defects, premature birth, nutritional deficiencies, infections, injuries, and other causes. Severe retinopathy of prematurity (ROP), cataracts and refractive error are also causes.
The most frequently affected parts of the eyes are:
- Whole globe (36%)
- Cornea (36%)
- Lens (11%)
- Retina (6%)
- Optic nerve (5%)
- Uvea (2%)
The definition of visual impairment is reduced vision not corrected by glasses or contact lenses. The World Health Organization uses the following classifications of visual impairment. When the vision in the better eye with best possible glasses correction is:
- 20/30 to 20/60 : is considered mild vision loss, or near-normal vision
- 20/70 to 20/160 : is considered moderate visual impairment, or moderate low vision
- 20/200 to 20/400 : is considered severe visual impairment, or severe low vision
- 20/500 to 20/1,000 : is considered profound visual impairment, or profound low vision
- More than 20/1,000 : is considered near-total visual impairment, or near total blindness
- No light perception : is considered total visual impairment, or total blindness
Blindness is defined by the World Health Organization as vision in a person's best eye with best correction of less than 20/500 or a visual field of less than 10 degrees. This definition was set in 1972, and there is ongoing discussion as to whether it should be altered to officially include uncorrected refractive errors.
Visual impairment, also known as vision impairment or vision loss, is a decreased ability to see to a degree that causes problems not fixable by usual means, such as glasses. Some also include those who have a decreased ability to see because they do not have access to glasses or contact lenses. Visual impairment is often defined as a best corrected visual acuity of worse than either 20/40 or 20/60. The term blindness is used for complete or nearly complete vision loss. Visual impairment may cause people difficulties with normal daily activities such as driving, reading, socializing, and walking.
The most common causes of visual impairment globally are uncorrected refractive errors (43%), cataracts (33%), and glaucoma (2%). Refractive errors include near sighted, far sighted, presbyopia, and astigmatism. Cataracts are the most common cause of blindness. Other disorders that may cause visual problems include age related macular degeneration, diabetic retinopathy, corneal clouding, childhood blindness, and a number of infections. Visual impairment can also be caused by problems in the brain due to stroke, premature birth, or trauma among others. These cases are known as cortical visual impairment. Screening for vision problems in children may improve future vision and educational achievement. Screening adults without symptoms is of uncertain benefit. Diagnosis is by an eye exam.
The World Health Organization (WHO) estimates that 80% of visual impairment is either preventable or curable with treatment. This includes cataracts, the infections river blindness and trachoma, glaucoma, diabetic retinopathy, uncorrected refractive errors, and some cases of childhood blindness. Many people with significant visual impairment benefit from vision rehabilitation, changes in their environment, and assistive devices.
As of 2015 there were 940 million people with some degree of vision loss. 246 million had low vision and 39 million were blind. The majority of people with poor vision are in the developing world and are over the age of 50 years. Rates of visual impairment have decreased since the 1990s. Visual impairments have considerable economic costs both directly due to the cost of treatment and indirectly due to decreased ability to work.
Signs and symptoms vary depending on the type of cataract, though considerable overlap occurs. People with nuclear sclerotic or brunescent cataracts often notice a reduction of vision. Those with posterior subcapsular cataracts usually complain of glare as their major symptom.
The severity of cataract formation, assuming no other eye disease is present, is judged primarily by a visual acuity test. The appropriateness of surgery depends on a patient's particular functional and visual needs and other risk factors, all of which may vary widely.
Aside from a complete inability to see color, individuals with complete achromatopsia have a number of other ophthalmologic aberrations. Included among these aberrations are greatly decreased visual acuity (<0.1 or 20/200) in daylight, Hemeralopia, nystagmus, and severe photophobia. The fundus of the eye appears completely normal. Also see Pingelap.
The first symptom of this disease is usually a slow loss of vision. Early signs of Retinitis include loss of night vision; making it harder to drive at night. Later signs of retinitis include loss of peripheral vision, leading to tunnel vision. In some cases, symptoms are experienced in only one of the eyes. Experiencing the vision of floaters, flashes, blurred vision and loss of side vision in just one of the eyes is an early indication of the onset of Retinitis.
Total color blindness can be classified as:
- Acquired achromatopsia (Cerebral achromatopsia)
- Congenital/inherited achromatopsia
- Complete typical achromatopsia
- Incomplete atypical achromatopsia or incomplete atypical dyschromatopsia
Related terms:
- Achromatopsia–The complete lack of the perception of color in a subject, seeing only in black, white, and shades of grey.
- Amblyopia–Defined conceptually by Duke-Elder (1973) as a monocular acuity deficit which is not due to refractive error or any organic abnormality. A neural condition. Poor spatial performance of the precision optical servomechanism of the eyes at nominal illumination levels without any morphological cause. One form of lazy eye.
- Hemeralopia–Reduced visual capacity in bright light. Colloquially, day-blindness.
- Nystagmus–This term is used variously to describe both normal and pathological conditions related to the oculomotor system. In the current context, it is a pathological condition involving an uncontrolled oscillatory movement of the eyes during which the amplitude of oscillation is quite noticeable and the frequency of the oscillation tends to be quite low.
- Photophobia–The avoidance of bright light by those suffering from hemeralopia.
Based on clinical appearance, color blindness may be described as total or partial. Total color blindness is much less common than partial color blindness. There are two major types of color blindness: those who have difficulty distinguishing between red and green, and who have difficulty distinguishing between blue and yellow.
Immunofluorescent imaging is a way to determine red–green color coding. Conventional color coding is difficult for individuals with red–green color blindness (protanopia or deuteranopia) to discriminate. Replacing red with magenta or green with turquoise improves visibility for such individuals.
The different kinds of inherited color blindness result from partial or complete loss of function of one or more of the different cone systems. When one cone system is compromised, dichromacy results. The most frequent forms of human color blindness result from problems with either the middle or long wavelength sensitive cone systems, and involve difficulties in discriminating reds, yellows, and greens from one another. They are collectively referred to as "red–green color blindness", though the term is an over-simplification and is somewhat misleading. Other forms of color blindness are much more rare. They include problems in discriminating blues from greens and yellows from reds/pinks, and the rarest forms of all, complete color blindness or "monochromacy", where one cannot distinguish any color from grey, as in a black-and-white movie or photograph.
Protanopes, deuteranopes, and tritanopes are dichromats; that is, they can match any color they see with some mixture of just two primary colors (whereas normally humans are trichromats and require three primary colors). These individuals normally know they have a color vision problem and it can affect their lives on a daily basis. Two percent of the male population exhibit severe difficulties distinguishing between red, orange, yellow, and green. A certain pair of colors, that seem very different to a normal viewer, appear to be the same color (or different shades of same color) for such a dichromat. The terms protanopia, deuteranopia, and tritanopia come from Greek and literally mean "inability to see ("anopia") with the first ("prot-"), second ("deuter-"), or third ("trit-") [cone]", respectively.
Anomalous trichromacy is the least serious type of color deficiency. People with protanomaly, deuteranomaly, or tritanomaly are trichromats, but the color matches they make differ from the normal. They are called anomalous trichromats. In order to match a given spectral yellow light, protanomalous observers need more red light in a red/green mixture than a normal observer, and deuteranomalous observers need more green. From a practical standpoint though, many protanomalous and deuteranomalous people have very little difficulty carrying out tasks that require normal color vision. Some may not even be aware that their color perception is in any way different from normal.
Protanomaly and deuteranomaly can be diagnosed using an instrument called an anomaloscope, which mixes spectral red and green lights in variable proportions, for comparison with a fixed spectral yellow. If this is done in front of a large audience of males, as the proportion of red is increased from a low value, first a small proportion of the audience will declare a match, while most will see the mixed light as greenish; these are the deuteranomalous observers. Next, as more red is added the majority will say that a match has been achieved. Finally, as yet more red is added, the remaining, protanomalous, observers will declare a match at a point where normal observers will see the mixed light as definitely reddish.
A cataract is a clouding of the lens in the eye which leads to a decrease in vision. Cataracts often develop slowly and can affect one or both eyes. Symptoms may include faded colors, blurry vision, halos around light, trouble with bright lights, and trouble seeing at night. This may result in trouble driving, reading, or recognizing faces. Poor vision caused by cataracts may also result in an increased risk of falling and depression. Cataracts are the cause of half of blindness and 33% of visual impairment worldwide.
Cataracts are most commonly due to aging but may also occur due to trauma or radiation exposure, be present from birth, or occur following eye surgery for other problems. Risk factors include diabetes, smoking tobacco, prolonged exposure to sunlight, and alcohol. Either clumps of protein or yellow-brown pigment may be deposited in the lens reducing the transmission of light to the retina at the back of the eye. Diagnosis is by an eye examination.
Prevention includes wearing sunglasses and not smoking. Early on the symptoms may be improved with glasses. If this does not help, surgery to remove the cloudy lens and replace it with an artificial lens is the only effective treatment. Surgery is needed only if the cataracts are causing problems and generally results in an improved quality of life. Cataract surgery is not readily available in many countries, which is especially true for women, those living in rural areas, and those who do not know how to read.
About 20 million people are blind due to cataracts. It is the cause of approximately 5% of blindness in the United States and nearly 60% of blindness in parts of Africa and South America. Blindness from cataracts occurs in about 10 to 40 per 100,000 children in the developing world, and 1 to 4 per 100,000 children in the developed world. Cataracts become more common with age. More than half the people in the United States had cataracts by the age of 80.
Hemeralopia (from Greek "ημέρα", hemera "day"; and "αλαός", alaos "blindness") is the inability to see clearly in bright light and is the exact opposite of nyctalopia (night blindness). Hemera was the Greek goddess of day and Nyx was the goddess of night. However, it has been used in an opposite sense by many non-English-speaking doctors. It can be described as insufficient adaptation to bright light. It is also called heliophobia and day blindness.
In hemeralopia, daytime vision gets worse, characterised by photoaversion (dislike/avoidance of light) rather than photophobia (eye discomfort/pain in light) which is typical of inflammations of eye. Nighttime vision largely remains unchanged due to the use of rods as opposed to cones (during the day), which are affected by hemeralopia and in turn degrade the daytime optical response. Hence many patients feel they see better at dusk than in daytime.
There is another retinal disease in Briards known as hereditary retinal dysplasia. These dogs are night blind from birth, and day vision varies. Puppies affected often have nystagmus. It is also known as lipid retinopathy.
The initial retinal degenerative symptoms of retinitis pigmentosa are characterized by decreased night vision (nyctalopia) and the loss of the mid-peripheral visual field. The rod photoreceptor cells, which are responsible for low-light vision and are orientated in the retinal periphery, are the retinal processes affected first during non-syndromic forms of this disease. Visual decline progresses relatively quickly to the far peripheral field, eventually extending into the central visual field as tunnel vision increases. Visual acuity and color vision can become compromised due to accompanying abnormalities in the cone photoreceptor cells, which are responsible for color vision, visual acuity, and sight in the central visual field. The progression of disease symptoms occurs in a symmetrical manner, with both the left and right eyes experiencing symptoms at a similar rate.
A variety of indirect symptoms characterize retinitis pigmentosa along with the direct effects of the initial rod photoreceptor degeneration and later cone photoreceptor decline. Phenomena such as photophobia, which describes the event in which light is perceived as an intense glare, and photopsia, the presence of blinking or shimmering lights within the visual field, often manifest during the later stages of RP. Findings related to RP have often been characterized in the fundus of the eye as the "ophthalamic triad". This includes the development of (1) a mottled appearance of the retinal pigment epithelium (RPE) caused by bone spicule formation, (2) a waxy appearance of the optic nerve, and (3) the attentuation of blood vessels in the retina.
Non-syndromic RP usually presents a variety of the following symptoms:
- Night blindness
- Tunnel vision (due to loss of peripheral vision)
- Latticework vision
- Photopsia (blinking/shimmering lights)
- Photophobia (aversion to glare)
- Development of bone spicules in the fundus
- Slow adjustment from dark to light environments and vice versa
- Blurring of vision
- Poor color separation
- Loss of central vision
- Eventual blindness
Nyctalopia (from Greek νύκτ-, "nykt-" "night"; ἀλαός, "alaos" "blind, not seeing", and ὄψ, "ops" "eye"), also called night-blindness, is a condition making it difficult or impossible to see in relatively low light. It is a symptom of several eye diseases. Night blindness may exist from birth, or be caused by injury or malnutrition (for example, vitamin A deficiency). It can be described as insufficient adaptation to darkness.
The most common cause of nyctalopia is retinitis pigmentosa, a disorder in which the rod cells in the retina gradually lose their ability to respond to the light. Patients suffering from this genetic condition have progressive nyctalopia and eventually their daytime vision may also be affected. In X-linked congenital stationary night blindness, from birth the rods either do not work at all, or work very little, but the condition doesn't get worse.
Another cause of night blindness is a deficiency of retinol, or vitamin A, found in fish oils, liver and dairy products.
The opposite problem, the inability to see in bright light, is known as "hemeralopia" and is much rarer.
Since the outer area of the retina is made up of more rods than cones, loss of peripheral vision often results in night blindness. Individuals suffering from night blindness not only see poorly at night, but also require extra time for their eyes to adjust from brightly lit areas to dim ones. Contrast vision may also be greatly reduced.
Rods contain a receptor-protein called rhodopsin. When light falls on rhodopsin, it undergoes a series of conformational changes ultimately generating electrical signals which are carried to the brain via the optic nerve. In the absence of light, rhodopsin is regenerated. The body synthesizes rhodopsin from vitamin A, which is why a deficiency in vitamin A causes poor night vision.
Refractive "vision correction" surgery (especially PRK with the complication of "haze") may rarely cause a reduction in best night-time acuity due to the impairment of contrast sensitivity function (CSF) which is induced by intraocular light-scatter resulting from surgical intervention in the natural structural integrity of the cornea.
In almost all cases, color blind people retain blue–yellow discrimination, and most color-blind individuals are anomalous trichromats rather than complete dichromats. In practice, this means that they often retain a limited discrimination along the red–green axis of color space, although their ability to separate colors in this dimension is reduced. Color blindness very rarely refers to complete monochromatism.
Dichromats often confuse red and green items. For example, they may find it difficult to distinguish a Braeburn apple from a Granny Smith or red from green of traffic lights without other clues—for example, shape or position. Dichromats tend to learn to use texture and shape clues and so may be able to penetrate camouflage that has been designed to deceive individuals with normal color vision.
Colors of traffic lights are confusing to some dichromats as there is insufficient apparent difference between the red/amber traffic lights and sodium street lamps; also, the green can be confused with a grubby white lamp. This is a risk on high-speed undulating roads where angular cues cannot be used. British Rail color lamp signals use more easily identifiable colors: The red is blood red, the amber is yellow and the green is a bluish color. Most British road traffic lights are mounted vertically on a black rectangle with a white border (forming a "sighting board") and so dichromats can more easily look for the position of the light within the rectangle—top, middle or bottom. In the eastern provinces of Canada horizontally mounted traffic lights are generally differentiated by shape to facilitate identification for those with color blindness. In the United States, this is not done by shape but by position, as the red light is always on the left if the light is horizontal, or on top if the light is vertical. However, a single flashing light (red indicating cars must stop, yellow for caution/yield) is indistinguishable, but these are rare.
Retinitis is inflammation of the retina in the eye, which can permanently damage the retina and lead to blindness. The retina is the part of your eye that is also known as the "sensing tissue." Retinitis may be caused by a number of different infectious agents. Retinitis, also called Retinitis pigmentosa, has a prevalence of one in every 2,500-7,00 people. This condition is one of the leading causes that leads to blindness in patients in the age range of 20-60 years old.
Retinitis may be caused by several infectious agents, including toxoplasmosis, cytomegalovirus and candida. Cytomegalovirus retinitis is an important cause of blindness in AIDS patients, and is the most common cause of vision loss in AIDS patients. Candida may spread to the retina from the bloodstream, which usually leads to the production of several abscesses in the retina.
Signs and symptoms of macular degeneration include:
- Visual symptoms
- Distorted vision in the form of metamorphopsia, in which a grid of straight lines appears wavy and parts of the grid may appear blank: Patients often first notice this when looking at things like miniblinds in their home or telephone poles while driving. There may also be central scotomas, shadows or missing areas of vision
- Slow recovery of visual function after exposure to bright light (photostress test)
- Visual acuity drastically decreasing (two levels or more), e.g.: 20/20 to 20/80
- Blurred vision: Those with nonexudative macular degeneration may be asymptomatic or notice a gradual loss of central vision, whereas those with exudative macular degeneration often notice a rapid onset of vision loss (often caused by leakage and bleeding of abnormal blood vessels).
- Trouble discerning colors, specifically dark ones from dark ones and light ones from light ones
- A loss in contrast sensitivity
Macular degeneration by itself will not lead to total blindness. For that matter, only a very small number of people with visual impairment are totally blind. In almost all cases, some vision remains, mainly peripheral. Other complicating conditions may possibly lead to such an acute condition (severe stroke or trauma, untreated glaucoma, etc.), but few macular degeneration patients experience total visual loss.
The area of the macula comprises only about 2.1% of the retina, and the remaining 97.9% (the peripheral field) remains unaffected by the disease. Even though the macula provides such a small fraction of the visual field, almost half of the visual cortex is devoted to processing macular information.
The loss of central vision profoundly affects visual functioning. It is quite difficult, for example, to read without central vision. Pictures that attempt to depict the central visual loss of macular degeneration with a black spot do not really do justice to the devastating nature of the visual loss. This can be demonstrated by printing letters six inches high on a piece of paper and attempting to identify them while looking straight ahead and holding the paper slightly to the side. Most people find this difficult to do.
Oguchi disease present with nonprogressive night blindness since young childhood or birth with normal day vision, but they frequently claim improvement of light sensitivities when they remain for some time in a darkened environment.
On examination patients have normal visual fields but the fundos have a diffuse or patchy, silver-gray or golden-yellow metallic sheen and the retinal vessels stand out in relief against the background.
A prolonged dark adaptation of three hours or more, leads to disappearance of this unusual discoloration and the appearance of a normal reddish appearance. This is known as the Mizuo-Nakamura phenomena and is thought to be caused by the overstimulation of rod cells.
Amaurotic nystagmus is defined as the nystagmus associated with blindness or the central vision defects. It is characterized by the pendular or jerky movements of the eyes in the patients who have visual impairement for a long period of time.
There are various kinds of color blindness:
- Protanopia is a severe form of red-green color blindness, in which there is impairment in perception of very long wavelengths, such as reds. To these individuals, reds are perceived as beige or grey and greens tend to look beige or grey like reds. It is also the most common type of dichromacy today. This problem occurs because patients do not have the red cone cells in the retina. Protanomaly is a less severe version.
- Deuteranopia consists of an impairment in perceiving medium wavelengths, such as greens. Deuteranomaly is a less severe form of deuteranopia. Those with deuteranomaly cannot see reds and greens like those without this condition; however, they can still distinguish them in most cases. It is very similar to protanopia. In this form, patients do not have green cone cells in the retina, which makes it hard to see the green color.
- A rarer form of color blindness is tritanopia, where there exists an inability to perceive short wavelengths, such as blues. Sufferers have trouble distinguishing between yellow and blue. They tend to confuse greens and blues, and yellow can appear pink. This is the rarest of all dichromacy, and occurs in around 1 in 100,000 people. Patients do not have the blue cone cells in the retina.
Hemeralopia is known to occur in several ocular conditions. Cone dystrophy and achromatopsia, affecting the cones in the retina, and the anti-epileptic drug Trimethadione are typical causes. Adie's pupil which fails to constrict in response to light; Aniridia, which is absence of the iris; Albinism where the iris is defectively pigmented may also cause this. Central Cataracts, due to the lens clouding, disperses the light before it can reach the retina, is a common cause of hemeralopia and photoaversion in elderly. C.A.R (Cancer Associated Retinopathy) seen when certain cancers incite the production of deleterious antibodies against retinal components, may cause hemeralopia.
Another known cause is a rare genetic condition called Cohen Syndrome (aka Pepper Syndrome). Cohen syndrome is mostly characterized by obesity, mental retardation, and craniofacial dysmorphism due to genetic mutation at locus 8q22-23. Rarely it may have ocular complications such as hemeralopia, pigmentary chorioretinitis, optic atrophy or retinal/iris coloboma, having a serious effect on the person's vision.
Yet another cause of hemeralopia is uni- or bilateral postchiasmatic brain injury. This may also cause concomitant night blindness.
In general, PRAs are characterised by initial loss of rod photoreceptor cell function followed by that of the cones and for this reason night blindness is the first significant clinical sign for most dogs affected with PRA. As other retinal disorders, PRA can be divided into either dysplastic disease, where the cells develop abnormally, and degenerative, where the cells develop normally but then degenerate during the dog's lifetime.
Generalized PRA is the most common type and causes atrophy of all the neural retinal structures. Central progressive retinal atrophy (CPRA) is a different disease from PRA involving the retinal pigment epithelium (RPE), and is also known as retinal pigment epithelial dystrophy (RPED).
There are many causes of blurred vision:
- Use of atropine or other anticholinergics
- Presbyopia—Difficulty focusing on objects that are close. Common in the elderly. (Accommodation tends to decrease with age.)
- Cataracts—Cloudiness over the eye's lens, causing poor night-time vision, halos around lights, and sensitivity to glare. Daytime vision is eventually affected. Common in the elderly.
- Glaucoma—Increased pressure in the eye, causing poor night vision, blind spots, and loss of vision to either side. A major cause of blindness. Glaucoma can happen gradually or suddenly—if sudden, it is a medical emergency.
- Diabetes—Poorly controlled blood sugar can lead to temporary swelling of the lens of the eye, resulting in blurred vision. While it resolves if blood sugar control is reestablished, it is believed repeated occurrences promote the formation of cataracts (which are not temporary).
- Diabetic retinopathy—This complication of diabetes can lead to bleeding into the retina. Another common cause of blindness.
- Hypervitaminosis A—Excess consumption of vitamin A can cause blurred vision.
- Macular degeneration—Loss of central vision, blurred vision (especially while reading), distorted vision (like seeing wavy lines), and colors appearing faded. The most common cause of blindness in people over age 60.
- Eye infection, inflammation, or injury.
- Sjögren's syndrome, a chronic autoimmune inflammatory disease that destroys moisture producing glands, including lacrimal (tear)
- Floaters—Tiny particles drifting across the eye. Although often brief and harmless, they may be a sign of retinal detachment.
- Retinal detachment—Symptoms include floaters, flashes of light across your visual field, or a sensation of a shade or curtain hanging on one side of your visual field.
- Optic neuritis—Inflammation of the optic nerve from infection or multiple sclerosis. You may have pain when you move your eye or touch it through the eyelid.
- Stroke or transient ischemic attack
- Brain tumor
- Toxocara—A parasitic roundworm that can cause blurred vision
- Bleeding into the eye
- Temporal arteritis—Inflammation of an artery in the brain that supplies blood to the optic nerve.
- Migraine headaches—Spots of light, halos, or zigzag patterns are common symptoms prior to the start of the headache. A retinal migraine is when you have only visual symptoms without a headache.
- Myopia—Blurred vision may be a systemic sign of local anaesthetic toxicity
- Reduced blinking—Lid closure that occurs too infrequently often leads to irregularities of the tear film due to prolonged evaporation, thus resulting in disruptions in visual perception.
- Carbon monoxide poisoning—Reduced oxygen delivery can effect many areas of the body including vision. Other symptoms caused by CO include vertigo, hallucination and sensitivity to light.
Other conditions with similar appearing fundi include
- Cone dystrophy
- X-linked retinitis pigmentosa
- Juvenile macular dystrophy
These conditions do not show the Mizuo-Nakamura phenomenon.
The X-linked varieties of congenital stationary night blindness (CSNB) can be differentiated from the autosomal forms by the presence of myopia, which is typically absent in the autosomal forms. Patients with CSNB often have impaired night vision, myopia, reduced visual acuity, strabismus, and nystagmus. Individuals with the complete form of CSNB (CSNB1) have highly impaired rod sensitivity (reduced ~300x) as well as cone dysfunction. Patients with the incomplete form can present with either myopia or hyperopia.