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Many people with amblyopia, especially those who only have a mild form, are not aware they have the condition until tested at older ages, since the vision in their stronger eye is normal. People typically have poor stereo vision, however, since it requires both eyes. Those with amblyopia further may have, on the affected eye, poor pattern recognition, poor visual acuity, and low sensitivity to contrast and motion.
Amblyopia is characterized by several functional abnormalities in spatial vision, including reductions in visual acuity, contrast sensitivity function, and vernier acuity, as well as spatial distortion, abnormal spatial interactions, and impaired contour detection. In addition, individuals with amblyopia suffer from binocular abnormalities such as impaired stereoacuity (stereoscopic acuity) and abnormal binocular summation. Also, a crowding phenomenon is present.
These deficits are usually specific to the amblyopic eye. However, subclinical deficits of the "better" eye have also been demonstrated.
People with amblyopia also have problems of binocular vision such as limited stereoscopic depth perception and usually have difficulty seeing the three-dimensional images in hidden stereoscopic displays such as autostereograms. Perception of depth, however, from monocular cues such as size, perspective, and motion parallax remains normal.
Refractive amblyopia may result from anisometropia (unequal refractive error between the two eyes). Anisometropia exists when there is a difference in the power between the two eyes. The eye which provides the brain with a clearer image typically becomes the dominant eye. The image in the other eye is blurred, which results in abnormal development of one half of the visual system. Refractive amblyopia is usually less severe than strabismic amblyopia and is commonly missed by primary care physicians because of its less dramatic appearance and lack of obvious physical manifestation, such as with strabismus. Given that the refractive correction of anisometropia by means of spectacles typically leads to different image magnification for the two eyes, which may in turn prevent binocular vision, a refractive correction using contact lenses is to be considered. Also pediatric refractive surgery is a treatment option, in particular if conventional approaches have failed due to aniseikonia or lack of compliance or both.
Frequently, amblyopia is associated with a combination of anisometropia and strabismus. In some cases, the vision between the eyes can differ to the point where one eye has twice average vision while the other eye is completely blind.
The signs and symptoms of far-sightedness are blurry vision, headaches, and eye strain. The common symptom is eye strain. Difficulty seeing with both eyes (binocular vision) may occur, as well as difficulty with depth perception.
Far-sightedness can have rare complications such as strabismus and amblyopia. At a young age, severe far-sightedness can cause the child to have double vision as a result of "over-focusing".
An eye that has no refractive error when viewing distant objects is said to have "emmetropia" or be "emmetropic" meaning the eye is in a state in which it can focus parallel rays of light (light from distant objects) on the retina, without using any accommodation. A distant object in this case is defined as an object located beyond 6 meters, or 20 feet, from the eye, since the light from those objects arrives as essentially parallel rays when considering the limitations of human perception.
An eye that has refractive error when viewing distant objects is said to have "ametropia" or be "ametropic". This eye cannot focus parallel rays of light (light from distant objects) on the retina, or needs accommodation to do so.
The word "ametropia" can be used interchangeably with "refractive error". Types of ametropia include myopia, hyperopia and astigmatism. They are frequently categorized as spherical errors and cylindrical errors:
- Spherical errors occur when the optical power of the eye is either too large or too small to focus light on the retina. People with refractive error frequently have blurry vision.
- Nearsightedness: When the optics are too powerful for the length of the eyeball one has myopia or nearsightedness. This can arise from a cornea or crystalline lens with too much curvature (refractive myopia) or an eyeball that is too long (axial myopia). Myopia can be corrected with a concave lens which causes the divergence of light rays before they reach the cornea.
- Farsightedness: When the optics are too weak for the length of the eyeball, one has hyperopia or farsightedness. This can arise from a cornea or crystalline lens with not enough curvature (refractive hyperopia) or an eyeball that is too short (axial hyperopia). This can be corrected with convex lenses which cause light rays to converge prior to hitting the cornea.
- Presbyopia: When the flexibility of the lens declines, typically due to age. The individual would experience difficulty in near vision, often relieved by reading glasses, bifocal, or progressive lenses.
- Cylindrical errors cause astigmatism, when the optical power of the eye is too powerful or too weak across one meridian, such as if the corneal curvature tends towards a cylindrical shape. The angle between that meridian and the horizontal is known as the axis of the cylinder.
- Astigmatism: A person with astigmatic refractive error sees lines of a particular orientation less clearly than lines at right angles to them. This defect can be corrected by refracting light more in one meridian than the other. Cylindrical lenses serve this purpose.
Concomitant esotropia – that is, an inward squint that does not vary with the direction of gaze – mostly sets in before 12 months of age (this constitutes 40% of all strabismus cases) or at the age of three or four. Most patients with "early-onset" concomitant esotropia are emmetropic, whereas most of the "later-onset" patients are hyperopic. It is the most frequent type of natural strabismus not only in humans, but also in monkeys.
Concomitant esotropia can itself be subdivided into esotropias that are ether "constant," or "intermittent."
- Constant esotropia
- Intermittent esotropia
A patient can have a constant esotropia for reading, but an intermittent esotropia for distance (but rarely vice versa).
The earliest sign of exotropia is usually a noticeable outward deviation of the eye. This sign may at first be intermittent, occurring when a child is daydreaming, not feeling well, or tired. It may also be more noticeable when the child looks at something in the distance. Squinting or frequent rubbing of the eyes is also common with exotropia. The child probably will not mention seeing double, i.e., double vision. However, he or she may close one eye to compensate for the problem.
Generally, exotropia progresses in frequency and duration. As the disorder progresses, the eyes will start to turn out when looking at close objects as well as those in the distance. If left untreated, the eye may turn out continually, causing a loss of binocular vision.
In young children with any form of strabismus, the brain may learn to ignore the misaligned eye's image and see only the image from the best-seeing eye. This is called amblyopia, or lazy eye, and results in a loss of binocular vision, impairing depth perception. In adults who develop strabismus, double vision sometimes occurs because the brain has already been trained to receive images from both eyes and cannot ignore the image from the turned eye.
Additionally in adults who have had exotropia since childhood, the brain may adapt to using a "blind-spot" whereby it receives images from both eyes, but no full image from the deviating eye, thus avoiding double vision and in fact increasing peripheral vision on the side of the deviating eye.
Concomitant esotropias can arise as an initial problem, in which case they are designated as 'Primary,' as a consequence of loss or impairment of vision, in which case they are designated as 'Secondary,' or following overcorrection of an initial Exotropia in which case they are described as being 'Consecutive'. The vast majority of esotropias are primary.
Although astigmatism may be asymptomatic, higher degrees of astigmatism may cause symptoms such as blurry vision, squinting, eye strain, fatigue, or headaches. Some research has pointed to the link between astigmatism and higher prevalence of migraine headaches.
When observing a person with strabismus, the misalignment of the eyes may be quite apparent. A patient with a constant eye turn of significant magnitude is very easy to notice. However, a small magnitude or intermittent strabismus can easily be missed upon casual observation. In any case, an eye care professional can conduct various tests, such as cover testing, to determine the full extent of the strabismus.
Symptoms of strabismus include double vision and/or eye strain. To avoid double vision, the brain may adapt by ignoring one eye. In this case, often no noticeable symptoms are seen other than a minor loss of depth perception. This deficit may not be noticeable in someone who has had strabismus since birth or early childhood, as they have likely learned to judge depth and distances using monocular cues. However, a constant unilateral strabismus causing constant suppression is a risk for amblyopia in children. Small-angle and intermittent strabismus are more likely to cause disruptive visual symptoms. In addition to headaches and eye strain, symptoms may include an inability to read comfortably, fatigue when reading, and unstable or "jittery" vision.
Refractive error, also known as refraction error, is a problem with focusing light accurately onto the retina due to the shape of the eye. The most common types of refractive error are near-sightedness, far-sightedness, astigmatism, and presbyopia. Near-sightedness results in far away objects being blurry, far-sightedness and presbyopia result in close objects being blurry, astigmatism causes objects to appear stretched out or blurry. Other symptoms may include double vision, headaches, and eye strain.
Near-sightedness is due to the length of the eyeball being too long, far-sightedness the eyeball too short, astigmatism the cornea being the wrong shape, and presbyopia aging of the lens of the eye such that it cannot change shape sufficiently. Some refractive errors occur more often among those whose parents are affected. Diagnosis is by eye examination.
Refractive errors are corrected with eyeglasses, contact lenses, or surgery. Eyeglasses are the easiest and safest method of correction. Contact lenses can provide a wider field of vision; however they are associated with a risk of infection. Refractive surgery permanently changes the shape of the cornea.
The number of people globally with refractive errors has been estimated at one to two billion. Rates vary between regions of the world with about 25% of Europeans and 80% of Asians affected. Near-sightedness is the most common disorder. Rates among adults are between 15-49% while rates among children are between 1.2-42%. Far-sightedness more commonly affects young children and the elderly. Presbyopia affects most people over the age of 35. The number of people with refractive errors that have not been corrected was estimated at 660 million (10 per 100 people) in 2013. Of these 9.5 million were blind due to the refractive error. It is one of the most common causes of vision loss along with cataracts, macular degeneration, and vitamin A deficiency.
Strabismus may also be classified based on time of onset, either congenital, acquired, or secondary to another pathological process. Many infants are born with their eyes slightly misaligned, and this is typically outgrown by six to 12 months of age. Acquired and secondary strabismus develop later. The onset of accommodative esotropia, an overconvergence of the eyes due to the effort of accommodation, is mostly in early childhood. Acquired non-accommodative strabismus and secondary strabismus are developed after normal binocular vision has developed. In adults with previously normal alignment, the onset of strabismus usually results in double vision.
Any disease that causes vision loss may also cause strabismus, but it can also result from any severe and/or traumatic injury to the affected eye. Sensory strabismus is strabismus due to vision loss or impairment, leading to horizontal, vertical or torsional misalignment or to a combination thereof, with the eye with poorer vision drifting slightly over time. Most often, the outcome is horizontal misalignment. Its direction depends on the patient age at which the damage occurs: patients whose vision is lost or impaired at birth are more likely to develop esotropia, whereas patients with acquired vision loss or impairment mostly develop exotropia. In the extreme, complete blindness in one eye generally leads to the blind eye reverting to an anatomical position of rest.
Although many possible causes of strabismus are known, among them severe and/or traumatic injuries to the afflicted eye, in many cases no specific cause can be identified. This last is typically the case when strabismus is present since early childhood.
Results of a U.S. cohort study indicate that the incidence of adult-onset strabismus increases with age, especially after the sixth decade of life, and peaks in the eighth decade of life, and that the lifetime risk of being diagnosed with adult-onset strabismus is approximately 4%.
Nobel-prize winner David H. Hubel described suppression in simple terms as follows:
Suppression is frequent in children with anisometropia or strabismus or both. For instance, children with infantile esotropia may alternate with which eye they look, each time suppressing vision in the other eye.
In with-the-rule astigmatism, the eye has too much "plus" cylinder in the horizontal axis relative to the vertical axis (i.e., the eye is too "steep" along the vertical meridian relative to the horizontal meridian). This causes vertical beams of light to focus anterior to (in front of) horizontal beams of light, within the eye. This problem may be corrected using spectacles which have a "minus" cylinder placed on this horizontal axis. The effect of this will be that when a vertical beam of light in the distance travels towards the eye, the "minus" cylinder (which is placed with its axis lying horizontally – in line with the patient's excessively steep horizonal axis/vertical meridian) will cause this vertical beam of light to slightly "diverge", or "spread out vertically", before it reaches the eye. This compensates for the fact that the patient's eye converges light more powerfully in the vertical meridian than the horizontal meridian. Hopefully, after this, the eye will focus all light on the same location at the retina, and the patient's vision will be less blurred.
In against-the-rule astigmatism, a plus cylinder is added in the horizontal axis (or a minus cylinder in the vertical axis).
Axis is always recorded as an angle in degrees, between 0 and 180 degrees in a counter-clockwise direction. Both 0 and 180 degrees lie on a horizontal line at the level of the center of the pupil, and as seen by an observer, 0 lies on the right of both the eyes.
Irregular astigmatism, which is often associated with prior ocular surgery or trauma, is also a common naturally occurring condition. The two steep hemimeridians of the cornea, 180° apart in regular astigmatism, may be separated by less than 180° in irregular astigmatism (called "nonorthogonal" irregular astigmatism); and/or the two steep hemimeridians may be asymmetrically steep—that is, one may be significantly steeper than the other (called "asymmetric" irregular astigmatism). Irregular astigmatism is quantified by a vector calculation called topographic disparity.
Suppression of an eye is a subconscious adaptation by a person's brain to eliminate the symptoms of disorders of binocular vision such as strabismus, convergence insufficiency and aniseikonia. The brain can eliminate double vision by ignoring all or part of the image of one of the eyes. The area of a person's visual field that is suppressed is called the suppression scotoma (with a scotoma meaning, more generally, an area of partial alteration in the visual field). Suppression can lead to amblyopia.
Diplopia can also occur when viewing with only one eye; this is called monocular diplopia, or, where the patient perceives more than two images, monocular polyopia. While there rarely may be serious causes behind monocular diplopia symptoms, this is much less often the case than with binocular diplopia. The differential diagnosis of multiple image perception includes the consideration of such conditions as corneal surface keratoconus, subluxation of the lens, a structural defect within the eye, a lesion in the anterior visual cortex or non-organic conditions, however diffraction-based (rather than geometrical) optical models have shown that common optical conditions, especially astigmatism, can also produce this symptom.
Exotropia is a form of strabismus where the eyes are deviated outward. It is the opposite of esotropia and usually involves more severe axis deviation than exophoria. People with exotropia often experience crossed diplopia. Intermittent exotropia is a fairly common condition. "Sensory exotropia" occurs in the presence of poor vision. Infantile exotropia (sometimes called "congenital exotropia") is seen during the first year of life, and is less common than "essential exotropia" which usually becomes apparent several years later.
The brain's ability to see three-dimensional objects depends on proper alignment of the eyes. When both eyes are properly aligned and aimed at the same target, the visual portion of the brain fuses the forms into a single image. When one eye turns inward, outward, upward, or downward, two different pictures are sent to the brain. This causes loss of depth perception and binocular vision. There have also been some reports of people that can "control" their afflicted eye. The term is from Greek "exo" meaning "outward" and "trope" meaning "a turning".
Anisometropia is the condition in which the two eyes have unequal refractive power. Each eye can be nearsighted (myopia), farsighted (hyperopia) or a combination of both, which is called antimetropia. Generally a difference in power of two diopters or more is the accepted threshold to label the condition anisometropia.
In certain types of anisometropia, the visual cortex of the brain will not use both eyes together (binocular vision), and will instead suppress the central vision of one of the eyes. If this occurs often enough during the first 10 years of life while the visual cortex is developing, it can result in amblyopia, a condition where even when correcting the refractive error properly, the person's vision in the affected eye is still not correctable to 20/20.
The name is from four Greek components: "an-" "not," "iso-" "same," "metr-" "measure," "ops" "eye."
An estimated 6% of subjects aged 6 to 18 have anisometropia.
Temporary binocular diplopia can be caused by alcohol intoxication or head injuries, such as concussion (if temporary double vision does not resolve quickly, one should see an optometrist or ophthalmologist immediately). It can also be a side effect of benzodiazepines or opioids, particularly if used in larger doses for recreation, the anti-epileptic drugs Phenytoin and Zonisamide, and the anti-convulsant drug Lamotrigine, as well as the hypnotic drug Zolpidem and the dissociative drugs Ketamine and Dextromethorphan. Temporary diplopia can also be caused by tired and/or strained eye muscles or voluntarily. If diplopia appears with other symptoms such as fatigue and acute or chronic pain, the patient should see an ophthalmologist immediately.
Refractive errors such as hyperopia and Anisometropia may be associated abnormalities found in patients with vertical strabismus.
The vertical miscoordination between the two eyes may lead to
- Strabismic amblyopia, (due to deprivation / suppression of the deviating eye)
- cosmetic defect (most noticed by parents of a young child and in photographs)
- Face turn, depending on presence of binocular vision in a particular gaze
- diplopia or double vision - more seen in adults (maturity / plasticity of neural pathways) and suppression mechanisms of the brain in sorting out the images from the two eyes.
- cyclotropia, a cyclotorsional deviation of the eyes (rotation around the visual axis), particularly when the root cause is an oblique muscle paresis causing the hypertropia.
Hypertropia is a condition of misalignment of the eyes (strabismus), whereby the visual axis of one eye is higher than the fellow fixating eye.
Hypotropia is the similar condition, focus being on the eye with the visual axis lower than the fellow fixating eye.
Dissociated Vertical Deviation is a special type of hypertropia leading to slow upward drift of one or rarely both eyes, usually when the patient is inattentive.
Cyclotropia is a form of strabismus in which, compared to the correct positioning of the eyes, there is a of one eye (or both) about the eye's visual axis. Consequently, the visual fields of the two eyes appear tilted relative to each other. The corresponding "latent" condition – a condition in which torsion occurs only in the absence of appropriate visual stimuli – is called cyclophoria.
Cyclotropia is often associated with other disorders of strabism, can result in double vision, and can cause other symptoms, in particular head tilt.
In some cases, subjective and objective cyclodeviation may result from surgery for oblique muscle disorders; if the visual system cannot compensate for it, cyclotropia and rotational double vision (cyclodiplopia) may result. The role of cyclotropia in vision disorders is not always correctly identified. In several cases of double vision, once the underlying cyclotropia was identified, the condition was solved by surgical cyclotropia correction.
Conversely, artificially causing cyclotropia in cats leads to reduced vision acuity, resulting in a defect similar to strabismic amblyopia.
"Cross-fixation congenital esotropia", also called "Cianci's syndrome" is a particular type of large-angle infantile esotropia associated with tight medius rectus muscles. With the tight muscles, which hinder adduction, there is a constant inward eye turn. The patient cross-fixates, that is, to fixate objects on the left, the patient looks across the nose with the right eye, and vice versa. The patient tends to adopt a head turn, turning the head to the right to better see objects in the left visual field and turning the head to the left to see those in the right visual field. Binasal occlusion can be used to discourage cross-fixation. However, the management of cross-fixation congenital esotropia usually involves surgery.
Historically the term 'congenital strabismus' was used to describe constant esotropias with onset between birth and six months of age. However, this term was felt to be an inadequate classification as it covered a variety of esotropias with different causes, features and prognoses.
In 1988, American ophthalmologist Gunter K. Von Noorden discussed what he described as 'Essential Infantile Esotropia'. He described the condition as:
"early acquired, not... congenital ..., although congenital factors may favor its development between the ages of 3 and 6 months"
1. Onset between birth and six months of age.
2. Large size (greater than 30 dioptres)
3. Stable size
4. Not associated with abnormalities of the central nervous system.
5. Often associated with defective abduction (outward movement) and excessive adduction (inward movement) of the eyes.
6. Also associated with oblique muscle dysfunction and Dissociated Vertical Deviation.
7. Initial alternation of the squint present with crossed fixation, i.e. the affected individual uses the left eye to look right and the right eye to look left.
8. Limited potential for binocular vision.
The same condition had also previously been described by other ophthalmologists, notably Cianca (1962) who named it Cianca's Syndrome and noted the presence of manifest latent nystagmus, and Lang (1968) who called it Congenital Esotropia Syndrome and noted the presence of abnormal head postures. In both cases, however, the essential characteristics were the same, but with emphasis placed on different elements of the condition.
Helveston (1993) further clarified and expanded upon von Noorden's work, and incorporated the work of both Lang and Cianca into his summary of the characteristics of the condition:
1. Esotropia between 10 and 90 dioptres in size
2. Either alternation or fixation preference may be present (if the latter then amblyopia may result).
3. Neurologically normal.
4. Hyperopic correction does not eliminate or significantly reduce the squint size.
5. Frequent nystagmus (latent or manifest latent).
6. The patient may or may not have any or all of the following associated conditions: Oblique muscle dysfunction, vertical incomitance, dissociated vertical deviation, asymmetric optokinetic nystagmus, torticollis.
7. Presence will be 'confirmed' by six months.
8. Best treatment results in subnormal binocular vision.
The expressions "congenital esotropia", "infantile esoptropia", "idiopathic infantile esotropia" and "essential infantile esotropia" are often used interchangeably.
The effects a coloboma has on the vision can be mild or more severe depending on the size and location of the gap. If, for example, only a small part of the iris is missing, vision may be normal, whereas if a large part of the retina or optic nerve is missing, vision may be poor and a large part of the visual field may be missing. This is more likely to cause problems with mobility if the lower visual field is absent. Other conditions can be associated with a coloboma. Sometimes, the eye may be reduced in size, a condition called microphthalmia. Glaucoma, nystagmus, scotoma, or strabismus may also occur.