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Strabismus

From Wikipedia, the free encyclopedia


For the protein Strabismus, see Strabismus (protein).
Strabismus
Synonyms Heterotropia, crossed eyes, squint[1]
Strabismus.jpg
Strabismus results in the eyes not aiming at the same point in space. Shown here is
a case of the exotropic type
Pronunciation
/str?'b?zm?s/[1]
Specialty Ophthalmology
Symptoms Nonaligned eyes[2]
Complications Amblyopia, double vision[3]
Types Esotropia (eyes crossed); exotropia (eyes diverge); hypertropia (eyes
vertically misaligned)[3]
Causes Muscle dysfunction, farsightedness, problems in the brain, trauma,
infections[3]
Risk factors Premature birth, cerebral palsy, family history[3]
Diagnostic method Observing light reflected from the pupil[3]
Similar conditions Cranial nerve disease[3]
Treatment Glasses, surgery[3]
Frequency ~2% (children)[3]
[edit on Wikidata]
Strabismus, also called crossed eyes among other terms, is a condition in which the
eyes do not properly align with each other when looking at an object.[2] The eye
which is focused on an object can alternate.[3] The condition may be present
occasionally or constantly.[3] If present during a large part of childhood, it may
result in amblyopia or loss of depth perception.[3] If onset is during adulthood,
it is more likely to result in double vision.[3]

Strabismus can occur due to muscle dysfunction, farsightedness, problems in the


brain, trauma, or infections.[3] Risk factors include premature birth, cerebral
palsy, and a family history of the condition.[3] Types include esotropia where the
eyes are crossed; exotropia where the eyes diverge; and hypertropia where they are
vertically misaligned.[3] They can also be classified by whether the problem is
present in all directions a person looks (comitant) or varies by direction
(incomitant).[3] Diagnosis may be made by observing the light reflecting from the
person's eyes and finding that it is not centered on the pupil.[3] Another
condition that produces similar symptoms is a cranial nerve disease.[3]

Treatment depends on the type of strabismus and the underlying cause.[3] This may
include the use of glasses and possibly surgery.[3] Some types benefit from early
surgery.[3] Strabismus occurs in about 2% of children.[3] The term is from the
Greek strabisms meaning "to squint".[4] Other terms for the condition include
"squint" and "cast of the eye".[5][6][7] "Wall-eye" has been used when the eyes
turn away from each other.[8]

Contents [hide]
1 Signs and symptoms
1.1 Psychosocial effects
2 Cause
3 Pathophysiology
4 Diagnosis
4.1 Latency
4.2 Onset
4.3 Laterality
4.4 Direction
4.5 Naming
4.6 Other considerations
4.7 Differential diagnosis
5 Management
5.1 Glasses
5.2 Surgery
5.3 Medication
6 Prognosis
7 References
8 Further reading
9 External links
Signs and symptoms[edit]
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 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[citation
needed]. 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[edit]
See also: Prevalence and impact of reduced stereopsis in humans

Actor Ryan Gosling's strabismus gives him a distinctive look.

Marty Feldman had a long career in comedy.


People of all ages who have noticeable strabismus may experience psychosocial
difficulties.[9][10][11] 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,[9][10]
[11] including efforts to re-establish binocular vision and the possibility of
stereopsis recovery.[12]

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 due not only 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.[13] 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.
[14][unreliable medical source?]

Children with strabismus, particularly those with exotropia (an outward turn), may
be 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.[15][16] 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.[17] Studies in which subjects were shown 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.[18][19]

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.[20] Successful surgical correction of strabismusfor adult patients as
well as childrenhas been shown to have a significantly positive effect on
psychological well-being.[21][22]

Very little research exists regarding coping strategies employed by adult


strabismics. One study categorized coping methods into three subcategories:
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.[23]

No studies have evaluated whether psychosocial interventions have had any benefits
on individuals undergoing strabismus surgery.[24]

Cause[edit]

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Strabismus can be seen in Down syndrome, Loeys-Dietz syndrome, cerebral palsy, and
Edwards syndrome. The risk is increased among those with a family history of the
condition.

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
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.[25][page needed] 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.[26][unreliable medical source] 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.
[citation needed]

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.[25][page needed]

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.[citation needed]

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

Several classifications are made when diagnosing strabismus.

Latency[edit]

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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.[27] 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,[28] 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.[29][30][31] In the extreme, complete blindness in one eye generally
leads to the blind eye reverting to an anatomical position of rest.[32]

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.[33]

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%.[34]

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.
[35][page needed] Unilateral strabismus has been observed to result from a severe
or traumatic injury to the affected eye.[29]

Direction[edit]

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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.[clarification needed]

Naming[edit]

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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.[36] Most types of infant and
childhood strabismus are comitant.[37] 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.[37] 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.[38]
Incomitant strabismus of the eso- or exo-type are classified as "alphabet
patterns": they are denoted as A- or V- or more rarely ?-, 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 ?-, 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.[39]

Types of incomitant strabismus include: Duane syndrome, horizontal gaze palsy, and
congenital fibrosis of the extraocular muscles.[40]

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.

Differential diagnosis[edit]

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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.

Retinoblastoma may also result in abnormal light reflection from the eye.

Management[edit]
Main article: Management of strabismus

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


As with other binocular vision disorders, the primary goal is comfortable, single,
clear, normal binocular vision at all distances and directions of gaze.[41]

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.

Glasses[edit]
In cases of accommodative esotropia, the eyes turn inward due to the effort of
focusing 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.[42]

Early treatment of strabismus when the person is a baby may reduce the chance of
developing amblyopia and depth perception problems. However, a review of randomized
controlled trials concluded that the use of corrective glasses to prevent
strabismus is not supported by existing research.[43] Most children eventually
recover from amblyopia if they have had the benefit of patches and corrective
glasses.[citation needed] Amblyopia has long been considered to remain permanent if
not treated within a critical period, namely before the age of about seven years;
[27] 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 and
to prevent double vision from occurring.

Surgery[edit]
Strabismus surgery does not remove the need for a child to wear glasses. Currently
it is unknown whether there are any differences for completing strabismus surgery
before or after amblyopia therapy in children.[44]

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 six to eight weeks
for recovery. Adjustable sutures may be used to permit refinement of the eye
alignment in the early postoperative period.[45]

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.[28]

Medication[edit]
Medication is used for strabismus in certain circumstances. In 1989, the US FDA
approved Botulinum toxin therapy for strabismus in patients over 12 years old.[46]
[47] Most commonly used in adults, the technique is also used for treating
children, in particular children affected by infantile esotropia.[48][49][50] 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.
Botulinum toxin therapy has been reported to be similarly successful as strabismus
surgery for people with binocular vision and less successful than surgery for those
who have no binocular vision.[51]

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."[52][unreliable medical source] A second operation is sometimes
required to straighten the eyes.[25][page needed]

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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]
Classification V T D
ICD-10: H49 H50 ICD-9-CM: 378 OMIM: 185100 MeSH: D013285 DiseasesDB: 29577
External resources
MedlinePlus: 001004 Patient UK: Strabismus

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Diseases of the human eye (H00H59 360379)
Authority control
NDL: 00571965
Categories: Disorders of ocular muscles, binocular movement, accommodation and
refraction
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