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During an eye examination, a test such as cover testing or the Hirschberg test is used in the diagnosis and measurement of strabismus and its impact on vision. Retinal birefringence scanning can be used for screening of young children for eye misaligments.
Several classifications are made when diagnosing strabismus.
Amblyopia is diagnosed by identifying low visual acuity in one or both eyes, out of proportion to the structural abnormality of the eye and excluding other visual disorders as causes for the lowered visual acuity. It can be defined as an interocular difference of two lines or more in acuity (e.g. on Snellen chart) when the eye optics is maximally corrected. In young children, visual acuity is difficult to measure and can be estimated by observing the reactions of the patient reacts when one eye is covered, including observing the patient's ability to follow objects with one eye.
Stereotests like the Lang stereotest are not reliable exclusion tests for amblyopia. A person who passes the Lang stereotest test is unlikely to have strabismic amblyopia, but could nonetheless have refractive or deprivational amblyopia. It has been suggested that binocular retinal birefringence scanning may be able to identify, already in very young children, amblyopia that is associated with strabismus, microstrabismus, or reduced fixation accuracy. Diagnosis and treatment of amblyopia as early as possible is necessary to keep the vision loss to a minimum.
Screening for amblyopia is recommended in all people between three and five years of age.
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.
Cyclotropia can be detected using subjective tests such as the Maddox rod test, the Bagolini striated lens test, the phase difference haploscope of Aulhorn, or the Lancaster red-green test (LRGT). Among these, the LRGT is the most complete. Cyclotropia can also be diagnosed using a combination of subjective and objective tests. Before surgery, both subjective and objective torsion should be assessed.
Experiments have also been made on whether cyclic deviations can be assessed by purely photographic means.
The cross-cover test, or alternating cover test is usually employed to detect heterophoria. One eye is covered, and then the cover is moved immediately over to the other eye. With heterophoria, when the cover is moved to the other eye, the eye that has just been uncovered can be seen to move from a deviated point. The difference between heterotropia and heterophoria can be easily understood as follows. With heterotropia, a correcting movement of the eye can be detected already by the simple cover test; with heterophoria, such correcting movement only takes place in the cross-cover test. People with heterophoria are able to create and maintain binocular fusion through vergence, and the cross-cover test purposely breaks this fusion, making the latent misaligment visible.
Whereas the cross-cover test allows a qualitative assessment to be done, a quantitative assessment of latent eye position disorders can be done using the Lancaster red-green test.
As with other binocular vision disorders, the primary goal is comfortable, single, clear, normal binocular vision at all distances and directions of gaze.
Strabismus is usually treated with a combination of eyeglasses, vision therapy, and surgery, depending on the underlying reason for the misalignment.
Whereas amblyopia (lazy eye), if minor and detected early, can often be corrected with use of an eye patch on the dominant eye and/or vision therapy, the use of eye patches is unlikely to change the angle of strabismus.
Between 2 and 5% of the population in western countries have amblyopia. In the U.K., 90% of visual health appointments in the child are concerning amblyopia.
Depending on the chosen criterion for diagnosis, between 1 and 4% of the children have amblyopia.
During an eye examination, the presence of suppression and the size and location of the suppression scotoma may be the Worth 4 dot test (a subjective test that is considered to be the most precise suppression test), or with other subjective tests such as the Bagolini striated lens test, or with objective tests such as the 4 prism base out test.
In order to understand how heterophoria occurs, we must understand of how the eye can maintain proper fixation with non aligned visual axis. Heterophoria is actually the misalignment of the visual axis of both eyes. In other words, one or both eyes are not properly fixated to an object of interest. However, we must know that the eyes have a fusional vergence system which corrects this misalignment.
Hyperopia is typically classified according to clinical appearance, its severity, or how it relates to the eye's accommodative status.
There are three clinical categories of hyperopia.
- Simple hyperopia
- Pathological hyperopia
- Functional hyperopia
There are also three categories severity:
- Low
- Moderate
- High
Other common types of refractive errors are near-sightedness, astigmatism, and presbyopia.
The prognosis for each patient with esotropia will depend upon the origin and classification of their condition. However, in general, management will take the following course:
1. Identify and treat any underlying systemic condition.
2. Prescribe any glasses required and allow the patient time to 'settle into' them.
3. Use occlusion to treat any amblyopia present and encourage alternation.
4. Where appropriate, orthoptic exercises can be used to attempt to restore binocularity.
5. Where appropriate, prismatic correction can be used, either temporarily or permanently, to relieve symptoms of double vision.
6. In specific cases, and primarily in adult patients, botulinum toxin can be used either as a permanent therapeutic approach, or as a temporary measure to prevent contracture of muscles prior to surgery
7. Where necessary, extra-ocular muscle surgery can be undertaken to improve cosmesis and, on occasion, restore binocularity.
Controversy has arisen regarding the selection and planning of surgical procedures, the timing of surgery and about what constitutes a favourable outcome.
1. Selection and planning
Some ophthalmologists, notably Ing and Helveston, favour a prescribed approach often involving multiple surgical episodes whereas others prefer to aim for full alignment of the eyes in one procedure and let the number of muscles operated upon during this procedure be determined by the size of the squint.
2. Timing and outcome
This debate relates to the technical anatomical difficulties of operating on the very young versus the possibility of an increased potential for binocularity associated with early surgery. Infants are often operated upon at the age of six to nine months of age and in some cases even earlier at three or four months of age. Some emphasize the importance of intervening early such as to keep the duration of the patient's abnormal visual experience to a minimum. Advocates of early surgery believe that those who have their surgery before the age of one are more likely to be able to use both eyes together post-operatively.
A Dutch study (ELISSS) compared early with late surgery in a prospective, controlled, non-randomized, multicenter trial and reported that:
"Children operated early had better gross stereopsis at age six as compared to children operated late. They had been operated more frequently, however, and a substantial number of children in both [originally-recruited] groups had not been operated at all."
Other studies also report better results with early surgery, notably Birch and Stager and Murray et al. but do not comment on the number of operations undertaken. A recent study on 38 children concluded that surgery for infantile esotropia is most likely to result in measureable stereopsis if patient age at alignment is not more than 16 months.
Another study found that for children with infantile esotropia early surgery decreases the risk of dissociated vertical deviation developing after surgery.
Aside the strabismus itself, there are other aspects or conditions that appear to improve after surgery or botulinum toxin eye alignment. Study outcomes have indicated that after surgery the child catches up in development of fine-motor skills (such as grasping a toy and handling a bottle) and of large-muscle skills (such as sitting, standing, and walking) in case a developmental delay was present before. Evidence also indicates that as of the age of six, strabismic children become less accepted by their peers, leaving them potentially exposed to social exclusion starting at this age unless their eye positioning is corrected by this time ("see also:" Psychosocial effects of strabismus).
According to a Cochrane review of 2012, controversies remain regarding type of surgery, non-surgical intervention and age of intervention.
The aims of treatment are as follows:
The elimination of any amblyopia
A cosmetically acceptable ocular alignment
long term stability of eye position
binocular cooperation.
If only small amounts of torsion are present, cyclotropia may be without symptoms entirely and may not need correction, as the visual system can compensate small degrees of torsion and still achieve binocular vision ("see also:" cyclodisparity, cyclovergence). The compensation can be a motor response (visually evoked cyclovergence) or can take place during signal processing in the brain. In patients with cyclotropia of vascular origin, the condition often improves spontaneously.
Cyclotropia cannot be corrected with prism spectacles in the way other eye position disorders are corrected. (Nonetheless two Dove prisms can be employed to rotate the visual field in experimental settings.)
For cyclodeviations above 5 degrees, surgery has normally been recommended. Depending on the symptoms, the surgical correction of cyclotropia may involve a correction of an associated vertical deviation (hyper- or hypotropia), or a Harada–Ito procedure or another procedure to rotate the eye inwards, or yet another procedure to rotate it outwards. A cyclodeviation may thus be corrected at the same time with a correction of a vertical deviation (hyper- or hypotropia); cyclodeviations without any vertical deviation can be difficult to manage surgically, as the correction of the cyclodeviation may introduce a vertical deviation.
Refractive surgery causes only minimal size differences, similar to contact lenses. In a study performed on 53 children who had amblyopia due to anisometropia, surgical correction of the anisometropia followed by strabismus surgery if required led to improved visual acuity and even to stereopsis in many of the children ("see:" Refractive surgery#Children).
There are several methods to quantify fixation disparity. The Mallett card, the Bernell lantern slide, the Wesson Card and the Disparometer may be used. A patient's associated phoria is the amount of prism needed to reduce their fixation disparity to zero minutes of arc.
The Mallett Fixation Disparity Unit
Instrument used to measure the associated heterophoria (or compensating prism). It consists of a small central fixation letter X surrounded by two letters O, one on each side of X, the three letters being seen binocularly, and two coloured polarized vertical bars in line with the centre of the X which are seen by each eye separately. The instrument can be swung through 90° to measure any vertical fixation disparity. The associated phoria is indicated by the misalignment of the two polarized bars when the subject fixates the X through cross-polarized filters in front of the eyes. The amount of associated phoria is given by the value of the base-in or base-out prism power necessary to produce alignment and the eye. The unit can also be used to detect suppression. See Disparometer; associated heterophoria; uncompensated heterophoria.
A comprehensive eye examination including an ocular motility (i.e., eye movement) evaluation and an evaluation of the internal ocular structures will allow an eye doctor to accurately diagnose the exotropia. Although glasses and/or patching therapy, exercises, or prisms may reduce or help control the outward-turning eye in some children, surgery is often required.
There is a common form of exotropia known as "convergence insufficiency" that responds well to orthoptic vision therapy including exercises. This disorder is characterized by an inability of the eyes to work together when used for near viewing, such as reading. Instead of the eyes focusing together on the near object, one deviates outward.
"Consecutive exotropia" is an exotropia that arises after an initial esotropia. Most often it results from surgical overcorrection of the initial esotropia. It can be addressed with further surgery or with vision therapy; vision therapy has shown promising results if the consecutive exotropia is intermittent, alternating and of small magnitude. (Consecutive exotropia may however also spontaneously develop from esotropia, without surgery or botulinum toxin treatment.)
Because of the risks of surgery, and because about 35% of people require at least one more surgery, many people try vision therapy first. This consists of visual exercises. Although vision therapy is generally not covered by American health insurance companies, many large insurers such as Aetna have recently begun offering full or partial coverage in response to recent studies.
Strabismus surgery is sometimes recommended if the exotropia is present for more than half of each day or if the frequency is increasing over time. It is also indicated if a child has significant exotropia when reading or viewing near objects or if there is evidence that the eyes are losing their ability to work as a single unit (binocular vision). If none of these criteria are met, surgery may be postponed pending simple observation with or without some form of eyeglass and/or patching therapy. In very mild cases, there is a chance that the exotropia will diminish with time. The long-term success of surgical treatment for conditions such as intermittent exotropia is not well proven, and surgery can often result in a worsening of symptoms due to overcorrection. Evidence from the available literature suggests that unilateral surgery was more effective than bilateral surgery for individuals affected with intermittent exotropia.
The surgical procedure for the correction of exotropia involves making a small incision in the tissue covering the eye in order to reach the eye muscles. The appropriate muscles are then repositioned in order to allow the eye to move properly. The procedure is usually done under general anesthesia. Recovery time is rapid, and most people are able to resume normal activities within a few days. Following surgery, corrective eyeglasses may be needed and, in many cases, further surgery is required later to keep the eyes straight.
When a child requires surgery, the procedure is usually performed before the child attains school age. This is easier for the child and gives the eyes a better chance to work together. As with all surgery, there are some risks. However, strabismus surgery is usually a safe and effective treatment.
In general, strabismus can be approached and treated with a variety of procedures. Depending on the individual case, treatment options include:
- Correction of refractive errors by glasses
- Prism therapy (if tolerated, to manage diplopia)
- Patching (mainly to manage amblyopia in children and diplopia in adults)
- Botulinum toxin injection
- Surgical correction
Surgical correction of the hypertropia is desired to achieve binocularity, manage diplopia and/or correct the cosmetic defect. Steps to achieve the same depend on mechanism of the hypertropia and identification of the offending muscles causing the misalignment. Various surgical procedures have been described and should be offered after careful examination of eyes, including a detailed orthoptic examination focussing on the disturbances in ocular motility and visual status. Specialty fellowship trained pediatric ophthalmologists and strabismus surgeons are best equipped to deal with these complex procedures.
"Congenital esotropia," or "infantile esotropia," is a specific sub-type of primary concomitant esotropia. It is a constant esotropia of large and consistent size with onset between birth and six months of age. It is not associated with hyperopia, so the exertion of accommodative effort will not significantly affect the angle of deviation. It is, however, associated with other ocular dysfunctions including oblique muscle over-actions, Dissociated Vertical Deviation (DVD,) Manifest Latent Nystagmus, and defective abduction, which develops as a consequence of the tendency of those with infantile esotropia to 'cross fixate.' Cross fixation involves the use of the right eye to look to the left and the left eye to look to the right; a visual pattern that will be 'natural' for the person with the large angle esotropia whose eye is already deviated towards the opposing side.
The origin of the condition is unknown, and its early onset means that the affected individual's potential for developing binocular vision is limited. The appropriate treatment approach remains a matter of some debate. Some ophthalmologists favour an early surgical approach as offering the best prospect of binocularity whilst others remain unconvinced that the prospects of achieving this result are good enough to justify the increased complexity and risk associated with operating on those under the age of one year.
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.
Suppression may treated with vision therapy, though there is a wide range of opinions on long-term effectiveness between eye care professionals, with little scientific evidence of long-term improvement of suppression, if the underlying cause is not addressed (strabismus, amblyopia, etc.).
A diagnosis of far-sightedness can be made via a slit lamp test which examines the cornea, conjunctiva, and iris.
In severe cases of hyperopia from birth, the brain has difficulty in merging the images that each individual eye sees. This is because the images the brain receives from each eye are always blurred. A child with severe hyperopia can never see objects in detail. If the brain never learns to see objects in detail, then there is a high chance of one eye becoming dominant. The result is that the brain will block the impulses of the non-dominant eye. In contrast, the child with myopia can see objects close to the eye in detail and does learn at an early age to see detail in objects.
The appropriate treatment for binocular diplopia will depend upon the cause of the condition producing the symptoms. Efforts must first be made to identify and treat the underlying cause of the problem. Treatment options include eye exercises, wearing an eye patch on alternative eyes, prism correction, and in more extreme situations, surgery or botulinum toxin.
If diplopia turns out to be intractable, it can be managed as last resort by obscuring part of the patient's field of view. This approach is outlined in the article on diplopia occurring in association with a condition called "horror fusionis".
Diplopia has a diverse range of ophthalmologic, infectious, autoimmune, neurological, and neoplastic causes.
Typically a coloboma appears oval or comet shaped with round end towards the centre. There may be a few vessels (retinal or choroidal) at the edges. The surface may have irregular depression.