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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).
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.
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.
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%.
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.
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".
"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.
The eye drifts upward spontaneously or after being covered. The condition usually affects both eyes, but can occur unilaterally or asymmetrically. It is often associated with latent or manifest-latent nystagmus and, as well as occurring with infantile esotropia, can also be found associated with exotropias and vertical deviations.
DVDs are usually controlled from occurring with both eyes open, but may become manifest with inattention. Usually some level of dissociative occlusion is required - to trigger the brain to suppress vision in that eye and then not control a DVD from occurring. The level of dissociative occlusion required may involve using a red filter, a darker filter or complete occlusion (e.g. with a hand).
Clinically Infantile esotropia must be distinguished from:
1. VIth Cranial nerve or abducens palsy
2. Nystagmus Blockage Syndrome
3. Esotropia arising secondary to central nervous system abnormalities (in cerebral palsy for example)
4. Primary Constant esotropia
5. Duane's Syndrome
Heterophoria is an eye condition in which the directions that the eyes are pointing at rest position, when "not" performing binocular fusion, are not the same as each other, or, "not straight". There can be esophoria, where the eyes tend to cross inward in the absence of fusion; exophoria, in which they diverge; or hyperphoria, in which one eye points up or down relative to the other. Phorias are known as 'latent squint' because the tendency of the eyes to deviate is kept latent by fusion. A person with two normal eyes has single vision (usually) because of the combined use of the sensory and motor systems. The motor system acts to point both eyes at the target of interest; any offset is detected visually (and the motor system corrects it). Heterophoria only occurs during dissociation of the left eye and right eye, when fusion of the eyes is absent. If you cover one eye (e.g. with your hand) you remove the sensory information about the eye's position in the orbit. Without this, there is no stimulus to binocular fusion, and the eye will move to a position of "rest". The difference between this position, and where it would be were the eye uncovered, is the heterophoria. The opposite of heterophoria, where the eyes are straight when relaxed and not fusing, is called orthophoria.
In contrast, fixation disparity is a very small deviation of the pointing directions of the eyes that is present while performing binocular fusion.
Heterophoria is usually asymptomatic. This is when it is said to be "compensated". When fusional reserve is used to compensate for heterophoria, it is known as compensating vergence. In severe cases, when the heterophoria is not overcome by fusional vergence, sign and symptoms appear. This is called decompensated heterophoria.
Heterophoria may lead to squint or also known as strabismus.
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.
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.
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.
When the fusional vergence system can no longer hold back heterophoria, the phoria manifests. In this condition, the eyes deviate from the fixating position.
DVD typically becomes apparent between 18 months and three years of age, however, the difficulties of achieving the prolonged occlusion required for accurate detection in the very young, make it possible that onset is generally earlier than these figures suggest.
A way to demonstrate aniseikonia is to hold a near target (ex. pen or finger) approximately 6 inches directly in front of one eye. The person then closes one eye, and then the other. The person should notice that the target appears larger to the eye that it is directly in front of. When this object is viewed with both eyes, it is seen with a small amount of aniseikonia. The principles behind this demonstration are relative distance magnification (closer objects appear larger) and asymmetrical convergence (the target is not an equal distance from each eye).
Retinal image size is determined by many factors. The size and position of the object being viewed affects the characteristics of the light entering the system. Corrective lenses affect these characteristics and are used commonly to correct refractive error. The optics of the eye including its refractive power and axial length also play a major role in retinal image size.
Aniseikonia can occur naturally or be induced by the correction of a refractive error, usually anisometropia (having significantly different refractive errors between each eye) or antimetropia (being myopic (nearsighted) in one eye and hyperopic (farsighted) in the other.) Meridional aniseikonia occurs when these refractive differences only occur in one meridian (see astigmatism). Refractive surgery can cause aniseikonia in much the same way that it is caused by glasses and contacts.
One cause of significant anisometropia and subsequent aniseikonia has been aphakia. Aphakic patients do not have a crystalline lens. The crystalline lens is often removed because of opacities called cataracts. The absence of this lens left the patient highly hyperopic (farsighted) in that eye. For some patients the removal was only performed on one eye, resulting in the anisometropia / aniseikonia. Today, this is rarely a problem because when the lens is removed in cataract surgery, an intraocular lens, or IOL is left in its place.
The symptoms and signs associated with convergence insufficiency are related to prolonged, visually demanding, near-centered tasks. They may include, but are not limited to, diplopia (double vision), asthenopia (eye strain), transient blurred vision, difficulty sustaining near-visual function, abnormal fatigue,
headache, and abnormal postural adaptation, among others. In some cases, difficulty with making eye contact have been noted as a complaint amongst sufferers.
Note that some Internet resources confuse convergence and divergence dysfunction, reversing them.
Convergence insufficiency or convergence disorder is a sensory and neuromuscular anomaly of the binocular vision system, characterized by a reduced ability of the eyes to turn towards each other, or sustain convergence.
Exophoria can be caused by several factors, which include:
- Refractive errors - distance and near deviation approximately equal.
- Divergence excess - exodeviation is more than 15 dioptres greater for distance than near deviation.
- Convergence insufficiency - near exodeviation greater than distance deviation.
These can be due to nerve, muscle, or congenital problems, or due to mechanical anomalies. Unlike exotropia, fusion is possible in this condition, causing diplopia to be uncommon.
Esophoria is an eye condition involving inward deviation of the eye, usually due to extra-ocular muscle imbalance. It is a type of heterophoria.
Causes include:
- Refractive errors
- Divergence insufficiency
- Convergence excess; this can be due to nerve, muscle, congenital or mechanical anomalies.
Unlike esotropia, fusion is possible and therefore diplopia is uncommon.
Exophoria is particularly common in infancy and childhood, and increases with age.
A rostral lesion within the midbrain may affect the convergence center thus causing bilateral divergence of the eyes which is known as the WEBINO syndrome (Wall Eyed Bilateral INO) as each eye looks at the opposite "wall".
If the lesion affects the PPRF (or the abducens nucleus) and the MLF on the same side (the MLF having crossed from the opposite side), then the "one and a half syndrome" occurs which, simply put, involves paralysis of all conjugate horizontal eye movements other than abduction of the eye on the opposite side to the lesion.
The disorder is caused by injury or dysfunction in the medial longitudinal fasciculus (MLF), a heavily myelinated tract that allows conjugate eye movement by connecting the paramedian pontine reticular formation (PPRF)-abducens nucleus complex of the contralateral side to the oculomotor nucleus of the ipsilateral side.
In young patients with bilateral INO, multiple sclerosis is often the cause. In older patients with one-sided lesions a stroke is a distinct possibility. Other causes are possible.
A determination of the prevalence of anisometropia has several difficulties. First of all, the measurement of refractive error may vary from one measurement to the next. Secondly, different criteria have been employed to define anisometropia, and the boundary between anisometropia and isometropia depend on their definition.
Several studies have found that anisometropia occurs more frequently and tends to be more severe for persons with high ametropia, and that this is particularly true for myopes. Anisometropia follows a U-shape distribution according to age: it is frequent in infants aged only a few weeks, is more rare in young children, comparatively more frequent in teenagers and young adults, and more prevalent after presbyopia sets in, progressively increasing into old age.
One study estimated that 6% of those between the ages of 6 and 18 have anisometropia.
Notwithstanding research performed on the biomechanical, structural and optical characteristics of anisometropic eyes, the underlying reasons for anisometropia are still poorly understood.
Anisometropic persons who have strabismus are mostly far-sighted, and almost all of these have (or have had) esotropia. However, there are indications that anisometropia influences the long-term outcome of a surgical correction of an inward squint, and vice versa. More specifically, for patients with esotropia who undergo strabismus surgery, anisometropia may be one of the risk factors for developing consecutive exotropia and poor binocular function may be a risk factor for anisometropia to develop or increase.