Strabismus

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For the protein Strabismus, see Strabismus (protein).
Strabismus
Strabismus.jpg
Strabismus prevents the eyes from aiming at the same point in space.
Shown here is a case of "wall-eye", or exotropic, strabismus.
Classification and external resources
Pronunciation /strəˈbɪzməs/
Specialty Ophthalmology
ICD-10 H49 – H50
ICD-9-CM 378
OMIM 185100
DiseasesDB 29577
MedlinePlus 001004
Patient UK Strabismus
MeSH D013285
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Strabismus is a condition that interferes with binocular vision because it prevents a person from directing both eyes simultaneously towards the same fixation point; the eyes do not properly align with each other. Heterotropia is a medical synonym for the condition. Colloquial terms for strabismus include "cross-eye", "wall-eye", and a "cast of the eye".[1]

Strabismus typically involves a lack of coordination between the extraocular muscles, which prevents directing the gaze of both eyes at once to the same point in space; it thus hampers proper binocular vision, and may affect depth perception adversely. Strabismus is primarily managed by ophthalmologists, optometrists, and orthoptists. Strabismus is present in about 4% of children. Treatment should be started as early as possible to ensure the development of the best possible visual acuity[2][3] and stereopsis. It is from the Greek strabismós.[4]

Signs and symptoms

Aligned vergence; how one ideally views objects
Aligned vergence; how one ideally views objects
Esotropia
Esotropia
Exotropia
Exotropia
Arrow/dotted line indicates fixation distance: All three patients are fixating with their right eye (assuming an overhead view).

When observing a patient 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.

Strabismus can be seen in Down syndrome, Loeys-Dietz syndrome, cerebral palsy, and Edwards syndrome.

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.

Psychosocial effects

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See also: Prevalence and impact of reduced stereopsis in humans

People of all ages who have noticeable strabismus may experience psychosocial difficulties.[5][6][7] Attention has also been drawn to potential socioeconomic impact resulting from cases of detectable strabismus. A socioeconomic consideration exists as well in the context of decisions regarding strabismus treatment[5][6][7] including efforts to re-establish binocular vision and the possibility of stereopsis recovery.[8]

One study has shown that strabismic children commonly exhibit behaviors marked by higher degrees of inhibition, anxiety, and emotional distress, often leading to outright emotional disorders. These disorders are often related to a negative perception of the child by peers. This is not only due to an altered aesthetic appearance, but also because of the inherent symbolic nature of the eye and gaze, and the vitally important role they play in an individual's life as social components. For some, these issues improved dramatically following strabismus surgery.[9] Notably, strabismus interferes with normal eye contact, often causing embarrassment, anger, and feelings of awkwardness, thereby affecting social communication in a fundamental way, with a possible negative effect on self-esteem.[10]

There are indications that children with strabismus, particularly those with exotropia (an outward turn), are more likely to develop a mental health disorder than normal-sighted children. Researchers have theorized that esotropia (an inward turn) was not found to be linked to a higher propensity for mental illness due to the age range of the participants, as well as the shorter follow-up time period; esotropic children were monitored to a mean age of 15.8 years, compared with 20.3 years for the exotropic group.[11][12] [13] A subsequent study with participants from the same area monitored congenital esotropia patients for a longer time period; results indicated that esotropic patients were also more likely to develop mental illness of some sort upon reaching early adulthood, similar to those with constant exotropia, intermittent exotropia, or convergence insufficiency. The likelihood was 2.6 times that of controls. No apparent association with premature birth was observed, and no evidence was found linking later onset of mental illness to psychosocial stressors frequently encountered by those with strabismus.

Investigations have highlighted the impact that strabismus may typically have on quality of life.[14] Studies centered around images of strabismic and non-strabismic persons showed a strong negative bias towards those visibly displaying the condition, clearly demonstrating the potential for future socioeconomic implications with regard to employability, as well as other psychosocial effects related to an individual's overall happiness.[15][16]

Adult and child observers perceived a right heterotropia as more disturbing than a left heterotropia, and child observers perceived an esotropia as "worse" than an exotropia.[17] Successful surgical correction of strabismus -- for adult patients as well as children -- has been shown to have a significantly positive effect on psychological well-being.[18][19]

Very little research exists regarding coping strategies employed by adult strabismics. One study categorized coping methods into three sub-categories: avoidance (refraining from participation an activity), distraction (deflecting attention from the condition), and adjustment (approaching an activity differently). The authors of the study suggested that individuals with strabismus may benefit from psychosocial support such as interpersonal skills training.[20]

Pathophysiology

The extraocular muscles control the position of the eyes. Thus, a problem with the muscles or the nerves controlling them can cause paralytic strabismus. The extraocular muscles are controlled by cranial nerves III, IV, and VI. An impairment of cranial nerve III causes the associated eye to deviate down and out and may or may not affect the size of the pupil. Impairment of cranial nerve IV, which can be congenital, causes the eye to drift up and perhaps slightly inward. Sixth nerve palsy causes the eyes to deviate inward and has many causes due to the relatively long path of the nerve. Increased cranial pressure can compress the nerve as it runs between the clivus and brain stem.[2] Also, if the doctor is not careful, twisting of the baby's neck during forceps delivery can damage cranial nerve VI.[citation needed]

Evidence indicates a cause for strabismus may lie with the input provided to the visual cortex.[21] This allows for strabismus to occur without the direct impairment of any cranial nerves or extraocular muscles.

Strabismus may cause amblyopia due to the brain ignoring one eye. Amblyopia is the failure of one or both eyes to achieve normal visual acuity despite normal structural health. During the first seven to eight years of life, the brain learns how to interpret the signals that come from an eye through a process called visual development. Development may be interrupted by strabismus if the child always fixates with one eye and rarely or never fixates with the other. To avoid double vision, the signal from the deviated eye is suppressed, and the constant suppression of one eye causes a failure of the visual development in that eye.

Also, amblyopia may cause strabismus. If a great difference in clarity occurs between the images from the right and left eyes, input may be insufficient to correctly reposition the eyes. Other causes of a visual difference between right and left eyes, such as asymmetrical cataracts, refractive error, or other eye disease, can also cause or worsen strabismus.[2]

Accommodative esotropia is a form of strabismus caused by refractive error in one or both eyes. Due to the near triad, when a patient engages accommodation to focus on a near object, an increase in the signal sent by cranial nerve III to the medial rectus muscles results, drawing the eyes inward; this is called the accommodation reflex. If the accommodation needed is more than the usual amount, such as with people with significant hyperopia, the extra convergence can cause the eyes to cross.

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See also: Progressive external ophthalmoplegia, Kearns–Sayre syndrome, Brown's syndrome, Duane syndrome, and monofixation syndrome

Diagnosis

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 effect on vision. Retinal birefringence scanning can be used for screening of young children for eye misaligments.

Several classifications are made when diagnosing strabismus.

Latency

Strabismus can be manifest (-tropia) or latent (-phoria). A manifest deviation, or heterotropia (which may be eso-, exo-, hyper-, hypo-, cyclotropia or a combination of these), is present while the patient views a target binocularly, with no occlusion of either eye. The patient is unable to align the gaze of each eye to achieve fusion. A latent deviation, or heterophoria (eso-, exo-, hyper-, hypo-, cyclophoria or a combination of these), is only present after binocular vision has been interrupted, typically by covering one eye. This type of patient can typically maintain fusion despite the misalignment that occurs when the positioning system is relaxed. Intermittent strabismus is a combination of both of these types, where the patient can achieve fusion, but occasionally or frequently falters to the point of a manifest deviation.

Onset

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.[22] 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,[23] 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.[24][25][26] In the extreme, complete blindness in one eye generally leads to the blind eye reverting to an anatomical position of rest.[27]

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.[28] (See also Infantile esotropia.)

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%.[29]

Laterality

Strabismus may be classified as unilateral if the one eye consistently deviates, or alternating if either of the eyes can be seen to deviate. Alternation of the strabismus may occur spontaneously, with or without subjective awareness of the alternation. Alternation may also be triggered by various tests during an eye exam.[3] Unilateral strabismus has been observed to result from a severe and/or traumatic injury to the affected eye.

Direction

Horizontal deviations are classified into two varieties. Eso describes inward or convergent deviations towards the midline. Exo describes outward or divergent misalignment. Vertical deviations are also classified into two varieties. Hyper is the term for an eye whose gaze is directed higher than the fellow eye while hypo refers to an eye whose gaze is directed lower. Cyclo refers to torsional strabismus, which occurs when the eyes rotate around the anterior-posterior axis to become misaligned and is quite rare.

Naming

The directional prefixes are combined with -tropia and -phoria to describe various types of strabismus. For example, a constant left hypertropia exists when a patient's left eye is always aimed higher than the right. A patient with an intermittent right esotropia has a right eye that occasionally drifts toward the patient's nose, but at other times is able to align with the gaze of the left eye. A patient with a mild exophoria can maintain fusion during normal circumstances, but when the system is disrupted, the relaxed posture of the eyes is slightly divergent.

Other considerations

Strabismus can be further classified as follows:

  • Paretic strabismus is due to paralysis of one or several extraocular muscles.
  • Nonparetic strabismus is not due to paralysis of extraocular muscles.
  • Comitant (or concomitant) strabismus is a deviation that is the same magnitude regardless of gaze position.
  • Noncomitant (or incomitant) strabismus has a magnitude that varies as the patient shifts his or her gaze up, down, or to the sides.

Nonparetic strabismus is generally concomitant.[30] Most types of infant and childhood strabismus are comitant.[31] Paretic strabismus can be either comitant or noncomitant. Incomitant strabismus is almost always caused by a limitation of ocular rotations that is due to a restriction of extraocular eye movement (ocular restriction) or due to extraocular muscle paresis.[31] Incomitant strabismus cannot be fully corrected by prism glasses, because the eyes would require different degrees of prismatic correction dependent on the direction of the gaze.[32] Incomitant strabismus of the eso- or exo-type are classified as "alphabet patterns": they are denoted as A- or V- or more rarely λ-, Y- or X-pattern depending on the extent of convergence or divergence when the gaze moves upward or downward. These letters of the alphabet denote ocular motility pattern that have a similarity to the respective letter: in the A-pattern there is (relatively speaking) more convergence when the gaze is directed upwards and more divergence when it is directed downwards, in the V-pattern it is the contrary, in the λ-, Y- and X-patterns there is little or no strabismus in the middle position but relatively more divergence in one or both of the upward and downward positions, depending on the "shape" of the letter.[33]

Types of incomitant strabismus include: Duane syndrome, horizontal gaze palsy, and congenital fibrosis of the extraocular muscles.[34]

When the misalignment of the eyes is large and obvious, the strabismus is called large-angle, referring to the angle of deviation between the lines of sight of the eyes. Less severe eye turns are called small-angle strabismus. The degree of strabismus can vary based on whether the patient is viewing a distant or near target.

Strabismus that sets in after eye alignment had been surgically corrected is called consecutive strabismus.

Pseudostrabismus is the false appearance of strabismus. It generally occurs in infants and toddlers whose bridge of the nose is wide and flat, causing the appearance of esotropia due to less sclera being visible nasally. With age, the bridge of the child's nose narrows and the folds in the corner of the eyes become less prominent.

Management

Surgery to correct strabismus on an eight-month-old infant

As with other binocular vision disorders, the primary therapeutic goal for those with strabismus is comfortable, single, clear, normal binocular vision at all distances and directions of gaze.[35]

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. Strabismus is usually treated with a combination of eyeglasses, vision therapy, and surgery, depending on the underlying reason for the misalignment. For parents it is important to know that strabismus surgery does not remove the need for a child to wear glasses.

In cases of accommodative esotropia, the eyes turn inward due to the effort of focussing far-sighted eyes, and the treatment of this type of strabismus necessarily involves refractive correction, which is usually done via corrective glasses or contact lenses, and in these cases surgical alignment is considered only if such correction does not resolve the eye turn.

In case of strong anisometropia, contact lenses may be preferable to spectacles because they avoid the problem of visual disparities due to size differences (aniseikonia) which is otherwise caused by spectacles in which the refractive power is very different for the two eyes. In a few cases of strabismic children with anisometropic amblyopia, a balancing of the refractive error eyes via refractive surgery has been performed before strabismus surgery was undertaken.[36]

Strabismus surgery attempts to align the eyes by shortening, lengthening, or changing the position of one or more of the extraocular eye muscles. The procedure can typically be performed in about an hour, and requires about one or two weeks for recovery. Adjustable sutures may be used to permit refinement of the eye alignment in the early postoperative period.[37] (For details on the surgical intervention, see: Strabismus surgery.)

Double vision can rarely result, especially immediately after the surgery,[citation needed] and vision loss is very rare. Glasses affect the position by changing the person's reaction to focusing. Prisms change the way light, and therefore images, strike the eye, simulating a change in the eye position.[38]

Early treatment of strabismus in infancy may reduce the chance of developing amblyopia and depth perception problems. Most children eventually recover from amblyopia if they have had the benefit of patches and corrective glasses. Amblyopia has long been considered to remain permanent if not treated within a critical period, namely before the age of about seven years;[22] however, recent discoveries give reason to challenge this view and to adapt the earlier notion of a critical period to account for stereopsis recovery in adults. A meta-analysis found that, while not statistically significant, corrective glasses may result in a reduction in the risk of developing strabismus after 3 years of age, compared with no correction.[39]

Eyes that remain misaligned can still develop visual problems. Although not a cure for strabismus, prism lenses can also be used to provide some temporary comfort for sufferers and to prevent double vision from occurring.

Pharmacologic treatment is used for strabismus in certain circumstances. In 1989, the US FDA approved Botulinum toxin therapy for strabismus in patients over 12 years old.[40][41] Most commonly used in adults, the technique is also used for treating children, in particular children affected by infantile esotropia.[42][43][44] The toxin is injected in the stronger muscle, causing temporary and partial paralysis. The treatment may need to be repeated three to four months later once the paralysis wears off. Common side effects are double vision, droopy eyelid, overcorrection, and no effect. The side effects typically resolve also within three to four months.

Prognosis

When strabismus is congenital or develops in infancy, it can cause amblyopia, in which the brain ignores input from the deviated eye. Even with therapy for amblyopia, stereoblindness may occur. The appearance of strabismus may also be a cosmetic problem. One study reported 85% of adult strabismus patients "reported that they had problems with work, school, and sports because of their strabismus". The same study also reported 70% said strabismus "had a negative effect on their self-image".[45] After surgery, the squint can return, so a second operation is sometimes required to straighten the eyes.[2]

See also

References

  1. ↑ Lua error in package.lua at line 80: module 'strict' not found.
  2. ↑ 2.0 2.1 2.2 2.3 Lua error in package.lua at line 80: module 'strict' not found.
  3. ↑ 3.0 3.1 Lua error in package.lua at line 80: module 'strict' not found.
  4. ↑ Online Etymology Dictionary
  5. ↑ 5.0 5.1 Denise Satterfield, John L. Keltner, Thomas L. Morrison: Psychosocial Aspects of Strabismus Study, JAMA Ophthalmology, August 1993, Vol 111, No. 8, doi:10.1001/archopht.1993.01090080096024
  6. ↑ 6.0 6.1 Lua error in package.lua at line 80: module 'strict' not found.
  7. ↑ 7.0 7.1 Onder Uretmen, Sait Egrilmez, Süheyla Kose, Kemal Pamukçu, Cezmi Akkin, Melis Palamar, Negative social bias against children with strabismus, Acta Ophthalmologica Scandinavica, Volume 81, Issue 2, pages 138–142, April 2003 doi:10.1034/j.1600-0420.2003.00024.x
  8. ↑ See peer discussion in: Lua error in package.lua at line 80: module 'strict' not found.
  9. ↑ Lua error in package.lua at line 80: module 'strict' not found.
  10. ↑ Strabismus, by All About Vision, Access Media Group LLC
  11. ↑ Bruce J. Tonge, George L. Lipton, Gwen Crawford, Psychological and Educational Correlates of Strabismus in School Children, Australian and New Zealand Journal of Psychiatry, 1984, Vol. 18, No. 1 , Pages 71-77
  12. ↑ Brian G. Mohney, Jeff A. McKenzie, Jason A. Capo, Kevin J. Nusz, David Mrazek, Nancy N. Diehl, Mental Illness in Young Adults Who Had Strabismus as Children. Pediatrics, volume 122, issue 5, November 1, 2008, pp. 1033-1038. doi:10.1542/peds.2007-3484
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  14. ↑ Lua error in package.lua at line 80: module 'strict' not found.
  15. ↑ Lua error in package.lua at line 80: module 'strict' not found.
  16. ↑ Lua error in package.lua at line 80: module 'strict' not found.
  17. ↑ Lua error in package.lua at line 80: module 'strict' not found.
  18. ↑ Lua error in package.lua at line 80: module 'strict' not found.
  19. ↑ Lua error in package.lua at line 80: module 'strict' not found.
  20. ↑ Sue Jackson, Kate Gleeson: Living and coping with strabismus as an adult, EMJ Ophth. 2013;1:15-22.
  21. ↑ Lua error in package.lua at line 80: module 'strict' not found.
  22. ↑ 22.0 22.1 Lua error in package.lua at line 80: module 'strict' not found.
  23. ↑ Strabismus, MedLine Plus, U.S. National Library of Medicine, National Institutes of Health
  24. ↑ Lua error in package.lua at line 80: module 'strict' not found.
  25. ↑ Lua error in package.lua at line 80: module 'strict' not found.
  26. ↑ Lua error in package.lua at line 80: module 'strict' not found.
  27. ↑ Strabismus (squint), Encyclopædia Britannica
  28. ↑ Crossed Eyes (Strabismus). Did you really understand what your eye doctor told you? Excerpt from: Taking Care of Your Eyes, 2003, Triad Communications
  29. ↑ Jennifer M. Martinez-Thompson, Nancy N. Diehl, Jonathan M. Holmes, Brian G. Mohney, Incidence, Types, and Lifetime Risk of Adult-Onset Strabismus, Ophthalmology, Volume 121, Issue 4, April 2014, pages 877–882 doi:10.1016/j.ophtha.2013.10.030
  30. ↑ Definition by TheFreeDictionary
  31. ↑ 31.0 31.1 Lua error in package.lua at line 80: module 'strict' not found.
  32. ↑ Adult Strabismus Surgery – 2013, ONE Network, American Association of Ophthalmology, April 2013 (downloaded 6 September 2014)
  33. ↑ James L. Plotnik et al: A-Pattern Esotropia and Exotropia, Medscape (downloaded 8 September 2014)
  34. ↑ Lua error in package.lua at line 80: module 'strict' not found.
  35. ↑ Lua error in package.lua at line 80: module 'strict' not found.
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  38. ↑ Lua error in package.lua at line 80: module 'strict' not found.
  39. ↑ Lua error in package.lua at line 80: module 'strict' not found.
  40. ↑ Lua error in package.lua at line 80: module 'strict' not found.
  41. ↑ Kowal, L., Wong, E., & Yahalom, C. (2007). Botulinum toxin in the treatment of strabismus. A review of its use and effects. Disability & Rehabilitation, 29(23), 1823-1831.
  42. ↑ Lua error in package.lua at line 80: module 'strict' not found.
  43. ↑ Lua error in package.lua at line 80: module 'strict' not found.
  44. ↑ Lua error in package.lua at line 80: module 'strict' not found.
  45. ↑ Scribe/Alum Notes Winter 2001 – Template

Further reading

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External links

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