Strabismus

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For the protein Strabismus, see Strabismus (protein).
Strabismus
Classification and external resources
Strabismus.jpg
Strabismus prevents the eyes from aiming at the same point in space
ICD-10 H49H50
ICD-9 378
OMIM 185100
DiseasesDB 29577
MedlinePlus 001004
Patient UK Strabismus
MeSH D013285

Strabismus (/strəˈbɪzməs/, from Greek strabismós[1]), also known as heterotropia or colloquially as crossed eyes, is a condition in which the eyes are not properly aligned with each other. It typically involves a lack of coordination between the extraocular muscles, which prevents bringing the gaze of each eye to the same point in space, thus hampers proper binocular vision, and which may adversely affect depth perception. Strabismus is primarily managed by ophthalmologists, optometrists, and orthoptists. Strabismus is present in about 4% of children. Treatment should be started as soon as possible to ensure the development of the best possible visual acuity[2][3] and stereopsis.

Diagnosis and classification[edit]

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. Several classifications are made when diagnosing strabismus.

Latency[edit]

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[edit]

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.[4] Acquired and secondary strabismus are developed 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.[5] 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.[6][7][8] In the extreme, complete blindness in one eye generally leads to the blind eye reverting to an anatomical position of rest.[9]

Although many possible causes of strabismus are known, in many cases no specific cause can be identified. This is typically the case when strabismus is present since early childhood[10] (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%.[11]

Laterality[edit]

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]

Direction[edit]

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[edit]

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[edit]

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.[12] Most types of infant and childhood strabismus are comitant.[13] 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.[13] 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.[14] 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.[15]

Types of incomitant strabismus include: Duane syndrome, horizontal gaze palsy, and congenital fibrosis of the extraocular muscles.[16]

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.

Signs and symptoms[edit]

Aligned vergence; how one ideally views objects
Aligned vergence; how one ideally views objects
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.

This symptom can be seen in Down syndrome, cerebral palsy, and Edwards syndrome.

Symptoms of strabismus include diplopia 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.

Pathophysiology[edit]

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 muscles are controlled by cranial nerves III, IV, or 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.

Evidence indicates a cause for strabismus may lie with the input provided to the visual cortex.[17] 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.

Psychosocial effects[edit]

People of all ages may experience psychosocial difficulties if they have noticeable strabismus.[18][19][20]

Attention has also been drawn to the potential socioeconomic impact of strabismus. This is also a socioeconomic consideration in the context of decisions on strabismus treatment[18][19][20] including efforts to re-establish binocular vision, possibly with stereopsis recovery.[21]

One study showed that behaviour of strabismic children was marked by inhibition, anxiety, and emotional disorders. These disorders, considered to be due to the manner in which others look at the child in view of their altered esthetic appearance and the symbolic nature of the eye and gaze, improved after strabismus surgery.[22] 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 affect on self-esteem.[23] There are indications that children with strabismus, in particular those with exotropia, are more likely to develop a mental health disorder than normal-sighted children.[24][25] In one study, mothers of children with strabismus were shown to have higher depression scores, lower tendency to constitute a supportive relation with their children, and lower satisfaction with maternal role, in comparison with the control group; the mothers also had poor role functioning in the family in relation to food, clothing and support needs and poor affective responsiveness.[26]

Investigations have highlighted the impact that strabismus may typically have on the quality of life.[27] Studies on the basis of surveys using pictures of strabismic and non-strabismic persons demonstrated psychosocial effects and socioeconomic implications with regard to employability.[28][29]

Adult and children observers perceive a right heterotropia as more disturbing than a left heterotropia, and children observers perceive an esotropia as worse than an exotropia.[30] Successful surgical correction of strabismus is known to have positive effects on psychological well-being, even when implemented with adult patients.[31][32]

A study performed on persons with strabismus found that the psychological effects of strabismus did not depend on the patient’s angle of deviation, age, sex, presence of diplopia, visual acuity or direction of deviation.[33] A post-operative study found that strabismus surgery performed on adults had the effect, among other results, of reducing subjects' social anxiety levels.[34] A further post-operative study found that the quality of life of the subjects was the higher, the smaller the postoperative angle was.[35]

Another study showed that certain psychosocial aspects undergo changes for many months after strabismus surgery.[36]

There is very little research into coping strategies employed by adults with strabismus. One study categorized coping strategies into three categories: avoidance (not doing an activity), distraction (deflecting attention away from strabismus) and adjustment (doing the activity in a different way). The authors of the study suggested that individuals with strabismus may benefit from psychosocial support such as interpersonal skills training.[37]

Management[edit]

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.[38]

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 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.[39]

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.[40] (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.[41]

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;[4] 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.

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.

Botulinum toxin therapy is used for treating strabismus in certain circumstances. In 1989, the US FDA approved botulinum toxin type A (BT-A) as a treatment for strabismus in patients over 12 years old.[42][43] Most commonly used in adults, the technique is also used for treating children, in particular children affected by infantile esotropia.[44][45][46] 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[edit]

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".[47] After surgery, the squint can return, so a second operation is sometimes required to straighten the eyes.[2]

See also[edit]

References[edit]

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  47. ^ Scribe/Alum Notes Winter 2001 – Template

Further reading[edit]

  • Donahue, Sean P.; Buckley, Edward G.; Christiansen, Stephen P.; Cruz, Oscar A.; Dagi, Linda R. (August 2014). "Difficult problems: strabismus". Journal of American Association for Pediatric Ophthalmology and Strabismus (JAAPOS) 18 (4): e41. doi:10.1016/j.jaapos.2014.07.132. 

External links[edit]