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Ординатура / Офтальмология / Английские материалы / Oxford American Handbook of Ophthalmology_Tsai, Denniston, Murray_2011

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576 CHAPTER 17 Strabismus

Amblyopia

Amblyopia is a developmental defect of central visual processing leading to reduced visual form sense. Effectively, this means that during the first 8 to 10 years of life, our capacity for high-level vision is vulnerable. Anything less than perfect, balanced foveal images from both eyes can lead to loss of vision in one or both eyes.

With increasing age, this is harder to reverse and by about 10–12 years of age is usually permanent. However, some recent studies have shown success of amblyopia treatment even in older children.

Causes of amblyopia

No or reduced image

Deprivation amblyopia

Constant monocular occlusion for >1 week/year of life is very likely to lead to amblyopia in those <6 years. Significant unilateral congenital cataracts require urgent removal with optical correction in the first few weeks of life; significant bilateral congenital cataracts should be removed in the first 6–8 weeks of life.

Image blurring from refractive error

Anisometropic amblyopia: there is significant risk with difference of refraction of >2.5D, and increased risk if present >2 years. This is a highly amblyogenic stimulus.

Ametropic amblyopia: significant risk if refractive error is greater than +5.00D or –10.00D; bilateral amblyopia may occur if uncorrected.

Astigmatic/meridional amblyopia: significant risk if >0.75D cylinder; risk is increased if there is a different axis and/or magnitude between the two eyes.

Abnormal binocular interaction

Strabismic amblyopia: significant risk if one eye is preferred for fixation; if it is freely alternating, then there is low risk. This is more common in esotropia than in exotropia.

Clinical features

Reduced visual acuity in the absence of an organic cause and despite correction of refraction.

Exaggeration of the crowding phenomenon (better visual acuity with single optotypes).

Tolerance of a neutral density filter (for a specific filter, VA is reduced significantly less in amblyopia than in organic lesions).

Treatment

The critical period during which visual development may be influenced is up to 10 years. Newer research shows promise at up to 12 or more years of age, but with less effect.

At younger ages, there is more rapid reversal of amblyopia but increased risk of inducing occlusion amblyopia in the covered eye.

AMBLYOPIA 577

Occlusion

Adjust for age, acuity, and social factors. Practice is very variable, but longer episodes (time per day) and longer treatment (weeks of patching) are required for older patients and those with worse VA. This may range from 10 min/day in a 6-month-old to full-time in a 6-year-old. Most often, 1 to hours of patching per day is prescribed.

Penalization

Atropinization may reduce the VA in the better eye to around 20/80. This is only effective if the amblyopic eye has VA >20/80.

578 CHAPTER 17 Strabismus

Binocular single vision

Binocular single vision (BSV) is the ability to view the world with two eyes, form two separate images (one for each eye), and yet fuse these centrally to create a single perception.

The development of BSV depends on correct alignment and similar image clarity of both eyes from the neonatal period. This permits normal retinal correspondence in which an image will stimulate anatomically corresponding points of each retina and subsequent stimulation of functionally corresponding points in the occipital cortex to produce a single perception.

The points in space that project onto these corresponding retinal points lie on an imaginary plane. The horopter. Panum’s fusional area is the narrow plane in front and behind the horopter in which, despite disparity, points will be seen as single.

Levels of binocular single vision

Binocular vision may be graded as follows:

1.Simultaneous perception: simultaneously perceives an image on each retina;

2.Fusion: stimulation of corresponding points allows central fusion of image;

3.Stereopsis: images are fused but slight horizontal disparity gives a perception of depth.

Fusion has sensory and motor components. Whereas sensory fusion generates a single image from corresponding points, motor fusion adjusts eye position to maintain sensory fusion. Fusional reserves (also called fusional amplitudes) indicate the level at which these mechanisms break down (usually seen as diplopia) (Table 17.3).

Abnormalities of BSV

Confusion and diplopia

These are abnormalities of simultaneous perception.

Confusion is the stimulation of corresponding points by dissimilar images (i.e., two images appear superimposed in the same location).

Diplopia is the stimulation of noncorresponding points by the same image (i.e., double vision).

Adaptive mechanisms

Adaptive mechanisms include suppression, abnormal retinal correspondence, and abnormal head posture.

Suppression is a cortical mechanism to ignore one of the images causing confusion (central suppression at the fovea) or diplopia (peripheral suppression). Monocular suppression leads to amblyopia if not treated; alternating suppression (between the two eyes) does not, but depth perception and stereopsis will be decreased. The size and density of the suppression scotoma is also variable.

BINOCULAR SINGLE VISION 579

Table 17.3 Fusional reserves (approximate values)

Horizontal Near

Convergent

32

BO

 

Divergent

16

BI

Distance

Convergent

16

BO

 

Divergent

8

BI

Vertical

 

2–3

 

 

 

 

Abnormal retinal correspondence (ARC) is a cortical mechanism to allow anatomically noncorresponding points of each retina to stimulate functionally corresponding points in the occipital cortex to produce

a single perception. This allow a degree of BSV despite a manifest deviation.

Abnormal head posture is a behavioral mechanism that usually brings the object into the field of single vision.

Microtropia

The advantages of the above adaptive mechanisms are seen in a microtropia. This is a small manifest deviation with a degree of BSV permitted by variable combinations of ARC, eccentric fixation, and central suppression scotoma.

There is usually no movement on cover test (microtropia with identity), unless the eccentric fixation is not absolute (microtropia without identity).

580 CHAPTER 17 Strabismus

Strabismus: assessment

Although the patient’s (or parents’) primary concern is likely to be the ocular misalignment (strabismus), it is imperative to step back and consider the whole child, their visual development, and their ophthalmic status. Proper assessment requires taking a history (visual, birth, developmental; see Table 17.4), appropriate measurement of vision, refraction and ophthalmic examination (Table 17.5), and consideration of any amblyopic risk.

Strabismus may be the first presentation of a serious ocular pathology (e.g., retinoblastoma, cataract), thus careful ophthalmic examination (including dilated fundoscopy) is essential.

The general ophthalmic approach to examining the child (p. 606) must be adapted to include orthoptic examination and refraction. Turn the examination into a game whenever possible. Efficient examination helps reduce patient (and examiner) fatigue. When there is concern over possible systemic abnormalities, refer the child to a pediatrician.

The individual tests are discussed as part of clinical methods (pp. 29–32).

History

Table 17.4 An approach to assessing strabismus— history

Visual symptoms

Duration, variability, and direction of strabismus;

 

precipitants, fatigability, associations (visual acuity

 

and development, diplopia, abnormal head position)

POH

Previous or current eye disease; refractive error; any

 

previous surgery, especially on extraocular muscles

PMH

Obstetric or perinatal history; developmental history

Review of systems

Any other systemic (especially CNS) abnormalities

SH

Family support (for children)

FH

Family history of strabismus or other visual problems

Drug history

Drugs

Allergy history

Allergies

 

 

 

 

STRABISMUS: ASSESSMENT

581

Examination

 

 

 

 

 

 

 

 

Table 17.5 An approach to assessing strabismus—examination

 

 

 

Observation

Whole patient (e.g., dysmorphic features, use

 

 

 

 

of limbs, gait), face (e.g., asymmetry), abnormal

 

 

 

 

head posture, globes (e.g., proptosis), lids (e.g.,

 

 

 

 

ptosis), alignment of the eyes

 

 

 

Visual acuity

Use age-appropriate test (p. 9) when quantitative

 

 

 

 

not possible, qualitatively grade ability to fix and

 

 

 

 

follow (i.e., is it central, steady, and maintained?)

 

 

 

Visual function

Check for RAPD

 

 

 

Cover test

Near, distance, far distance

 

 

 

Deviation

Measure with prism cover test or estimate with

 

 

 

 

Krimsky or Hirschberg test; may be measured

 

 

 

 

with synoptophore

 

 

 

Fusional reserves

Measure prism (horizontal and vertical) tolerated

 

 

 

 

before diplopia or blurring

 

 

 

Motility

Ductions and versions (9 positions of gaze)

 

 

 

 

Convergence

 

 

 

 

Saccades

 

 

 

 

Doll’s eye movements

 

 

 

Accommodation

AC/A ratio, deviation with correction of

 

 

 

 

refractive error

 

 

 

Fixation

Fixation behavior, normal vs. eccentric, visuscope

 

 

 

Binocularity

Check for simultaneous perception with Worth

 

 

 

 

4-dot test or Bagolini glasses

 

 

 

Suppression

Detect with Worth 4-dot test, 4 base-out

 

 

 

 

prism test, or Bagolini glasses

 

 

 

Correspondence

Detect anomalous retinal correspondence with

 

 

 

 

Worth 4-dot, Bagolini glasses, or after-image test

 

 

 

Stereopsis

Measure level with Titmus, TNO, Lang, or Frisby

 

 

 

 

tests, or with synoptophore

 

 

 

Refraction

Cycloplegic refraction (for children)

 

 

 

 

 

Ophthalmic

This should include dilated funduscopy. Identify

 

 

 

 

 

 

any cause of d VA or associated abnormalities

 

 

 

 

(p. 611)

 

 

 

Systemic review

Notably cranial nerves; sensory, motor,

 

 

 

 

cerebellar function; speech; mental state

 

 

 

 

 

 

 

582 CHAPTER 17 Strabismus

Strabismus: outline

Esodeviations: the eye that turns in

Is there a deviation?

Abnormalities of the face, globe, or retina may simulate an esodeviation.

Table 17.6 Causes of pseudo-esotropia

Specific

Epicanthic folds

 

Narrow interpupillary distance

 

Negative angle kappa

General

Face—asymmetry

 

Globe—proptosis, enophthalmos

 

Pupils—miosis, mydriasis, heterochromia

 

 

Esophoria vs. esotropia

Phorias are latent deviations that are controlled by fusion. In certain circumstances (specific visual tasks, fatigue, illness, etc.), fusion can no longer be maintained and the eyes deviate.

Tropias are manifest deviations (Table 17.7). Some individuals may be phoric in one situation (e.g., for distance) and tropic in another (e.g., for near).

Table 17.7 Esotropia

Primary

 

 

 

Accommodative

Varies with

Normal AC:A ratio

Fully accommodative

 

accommodation

Resolves with

esotropia

 

 

hypermetropic

 

 

 

correction

 

 

 

Normal AC:A ratio

Partially

 

 

Improves with

accommodative

 

 

hypermetropic

esotropia

 

 

correction

 

 

 

High AC:A ratio

Convergence excess

Nonaccommo-

Constant

Starting <6 months

Infantile esotropia

dative

 

Starting >6 months

Basic esotropia

 

 

 

Varies with

Near fixation only

Near esotropia

 

fixation distance

 

(nonaccommodative

 

despite relief of

 

convergence excess)

 

accommodation

Distance fixation

Distance esotropia

 

 

 

 

only

(divergence

 

 

 

insufficiency)

 

Varies with time

Cyclic

Cyclic esotropia

Secondary

 

Organic dVA (e.g.,

Secondary

 

 

media opacities)

esotropia(sensory)

Post-exotropia

 

Previous surgery

Consecutive

 

 

for exotropia

esotropia

STRABISMUS: OUTLINE 583

Exodeviations: the eye that turns out

Is there a deviation?

As with esodeviations, structural abnormalities may simulate an exodeviation. Angle kappa (the difference between the pupillary axis and the optical axis) is usually slightly positive. An abnormally large positive angle kappa simulates an exodeviation.

A negative angle occurs from abnormal nasal positioning of the fovea (high myopia, traction, etc.). This simulates esodeviation.

Table 17.8 Causes of pseudo-exotropia

Specific

Wide interpupillary distance

 

Postive angle kappa

General

Face—asymmetry

 

Globe—proptosis/enophthalmos

 

Pupils—miosis/mydriasis/heterochromia

 

 

Exophoria vs. exotropia

Exophorias are latent deviations that are generally asymptomatic. However, when fusion can no longer be maintained, they decompensate with symptoms of asthenopia (eye strain), blurred vision, or diplopia. Exotropias are manifest deviations that may be variable or constant (Table 17.9).

Table 17.9 Exotropia

Primary

Constant

Starting <6 months

Infantile exotropia

 

 

 

Starting >6 months

Basic exotropia

 

 

Variable

Worse for near

Convergence weakness

 

 

 

Worse for distance

Simulated divergence excess

 

 

 

High AC:A ratio

 

 

 

 

 

Worse for distance

True divergence excess

 

 

 

Normal AC:A ratio

 

 

 

Secondary

 

Organic dVA (e.g.,

Secondary exotropia

 

 

 

media opacities)

 

 

 

 

 

 

 

 

Post-esotropia

 

Develops with time

Consecutive exotropia

 

 

 

 

 

in absence of fusion

 

 

 

 

 

 

 

 

 

584 CHAPTER 17 Strabismus

Comitant strabismus: esotropia

Esotropia is a manifest inward deviation of the visual axes relative to each other. It is the most common form of strabismus. The condition may be primary, secondary (most commonly due to poor vision), or consecutive (after surgery for an exodeviation). Primary esotropias are classified as accommodative or nonaccommodative.

As with all strabismus, the assessment should include refraction, full ophthalmic examination, and addressing of amblyopic risk. It is essential to detect or rule out underlying pathology (e.g., intraocular tumor, cataract) at the outset.

Accommodative esotropia

Accommodative esotropia usually presents between 1 and 5 years of age. It may be refractive or nonrefractive. In the refractive group, increased accommodation tries to compensate for uncorrected hypermetropia and is accompanied by excessive convergence.

In the nonrefractive group, there is an abnormal accommodative con- vergence–accommodation (AC:A) ratio. There may be overlap between these groups.

Refractive: fully accommodative esotropia

Esotropia fully corrected for distance and near by hypermetropic (usually +2 to +7D) correction; normal AC:A ratio; normal BSV if corrected; often intermittent initially (e.g., with fatigue, illness).

Treatment

Full hypermetropic correction is needed; treat any associated amblyopia. Orthoptic exercises may overcome suppression or improve fusion range.

Refractive: partially accommodative esotropia

Esotropia only partially corrected by hypermetropic correction; BSV absent, or limited with ARC; ± bilateral IO overaction.

Treatment

Full hypermetropic correction is needed; treat amblyopia. Consider surgery if there is potential for BSV (aim to convert to a fully accommodative esotropia) or cosmesis (if cosmetically unacceptable despite glasses).

Nonrefractive: convergence excess esotropia

Esotropia for near due to high AC:A ratio; orthoor esophoric for distance; dBSV for near, normal BSV for distance; usually hypermetropic.

Treatment

Treat any associated hypermetropia or amblyopia. Consider surgery (bilateral MR recession ± posterior fixation sutures), orthoptic exercises, executive bifocal glasses, or miotics.

COMITANT STRABISMUS: ESOTROPIA 585

Nonaccommodative

The most common esotropia is the nonaccommodative infantile esotropia (also called congenital esotropia). This is a large-angle esotropia presenting before 6 months, with poor BSV potential and near-normal refraction. Fixation often alternates between the eyes.

Other nonaccommodative esotropias usually present later (i.e., after 6 months of age).

Infantile esotropia

Esotropia presenting before 6 months, large angle (>30 ), crossfixation is common (if present, low risk of amblyopia), poor BSV potential; often emmetropia/mild hypermetropia; ± dissociated vertical deviation (DVD: upward deviation on occlusion with recovery on removal of cover and no movement of other eye); ± manifest latent nystagmus (p. 557).

Treatment

Treat any associated amblyopia (e.g., occlusion of better eye if not freely alternating); correct hypermetropia if >2D. The aim of surgery ocular alignment by 12 months (with better potential BSV) and usually comprises symmetrical MR recessions (± LR resection).

Additional IO-weakening procedures should be used with caution. Botulinum toxin may be used as an alternative to surgery.

Other nonaccommodative esotropias

Basic esotropia: constant esotropia for near and distance; treat surgically.

Near esotropia (nonaccommodative convergence excess): esotropia for near, orthoor esophoria for distance but with normal AC:A ratio. Treatment, if any, is surgical (medial recti > lateral recti).

Distance esotropia (divergence insufficiency): esophoria (or small esotropia) for near, larger esotropia for distance; associated with poor fusional divergence. Rule out bilateral CN VI palsies.

Cyclic esotropia: rare, periodic (e.g., alternate days), may proceed to constant esotropia.

Secondary esotropias

Esotropia may arise secondary to dVA, thus full ocular examination is vital in all cases with esotropia. Some esotropic syndromes may arise secondary to intracranial pathology.

Sensory deprivation: secondary to unilateral/bilateral dVA.

Divergence paralysis: secondary to tumor, trauma, or stroke. Unlike a bilateral CN VI palsy, the esodeviation remains constant or even decreases on lateral gaze.

Convergence spasm: usually functional. The esotropia is intermittent and is associated with miosis and accommodative spasm resulting in pseudomyopia. Ductions are normal. Treat with cycloplegia and full hypermetropic correction.

Pseudoesotropia

Various conditions may mimic an esotropia (see Table 17.6).