The condition in which the patient sees not much and the practitioner nothing at all… ! It is always (or almost always) associated with strab, aniso or form deprivation in early life. Diagnosed when there reduced letter acuity, absence of pathology and when any ametropia is corrected. The results of pyschophysical studies & electrophysiological investigations suggest little primary retinal or LGN abnormality. Instead, the most profound effects of the amblyopic process are found in the striate cortex.
NATURE AND MANAGEMENT OF COMITANT CONVERGENT STRABISMUS
Before dealing with specific types of strabismus, there are general factors that must be considered when assessing the suitability for treatment, which apply to all cases regardless of the condition.
Evaluation
Age
Age at onset
Type of strabismus
Angle of strabismus
Depth of sensory adaptations
Cooperation of patient and parent
Age
under 4 years – no co-operation for exercises: Rx and occlusion possible only
Over 8-9 years – not possible to restore BSV: cosmetic treatment only
Most adults have come to terms with the strabismus and require refraction only. Some may want cosmetic surgery.
Age at Onset
this is critical (may have to refer to old photos)
especially important to establish if the onset was within the first year (Px is more likely to have eccentric fixation, DVD, latent nystagmus)
Next establish if onset is within the first 3 years
Significance? : the younger the Px is at onset and the longer time to presentation, the worse the prognosis.
NB May never have developed binocularly driven cells and therefore cannot expect to obtain BV
If very recent onset the Px is more likely to have distressing symptoms and may require immediate referral (esp. if incomitant)
Type of Strabismus :
some Pxs respond better to refractive/orthoptic treatment than others
different methods of treatment are more appropriate for different types.
Angle of Strabismus
the greater the angle the worse the prognosis
>20∆ - surgery indicated
15-20∆ - other factors must be favourable for orthoptics
<15∆ - good for orthoptics
<6∆ - microtropia, no orthoptics necessary
Depth of Sensory Adaptations
the deeper, the worse the prognosis
Cooperation of Patient and Parent
must have high levels of interest and perseverance and reasonable intelligence.
Parents must give time for supervision of exercises
Refractive Esotropias
Fully accommodative esotropia
Partially accommodative esotropia
Convergence excess
Fully Accommodative Esotropia
Deviation is secondary to the presence of hyperopia
Excessive accommodation for distance and near stimulates excessive convergence sufficient to cause a strabismus
Onset usually 2-5 years; coincident with the increased use of accommodative effort
AC/A ratio is usually normal
BSV present in nearly all cases; may have microtropia if anisohyperopic
Usually no or only slight amblyopia, unless strabismus is present for a long time
Management
Correction of refractive error
full cycloplegic Rx for constant wear
Review 3-4 weeks later
Check that no more latent hyperopia has become manifest and alter Rx is big difference
Check state of BV and microtropia if aniso.
Treat any suppression still present (simultaneous vision cards on the Holmes stereoscope; bar reading; appreciation of physiological diplopia)
Management
If amblyopia worse than 6/12, may require period of direct part-time occlusion if under 7 years. NB if microtropia present, VA will never be equal
NB Often a refractive error is all that is necessary
Carefully selected Pxs may be able to remove Rx for sports or social events etc and can be helped to gain BSV without Rx by orthoptics
Criteria:
hyperopia < 4.00DS
Astigmatism < 1.00DC
Cooperation – Px should be of reading age (7 ish)
Motivation – older children are best as they are more self-conscious regarding specs
Parent’s cooperation is also essential. Warn that the Px will still need Rx at least for closework
Method
(i) teach Px recognition of pathological diplopia (eg coloured filters & spotlight, with or without vertical prism or CT to reveal other image) or train to stop accommodating (make the image go blurred) For many control of esotropia will occur spontaneously after (i).
Method
If not: (ii) Obtain BSV at intersection of visual axis and extend this area. Exercise fusional ranges (esp negative). Practice at home.
(iii) Improvement of binocular VA without Rx by increasing negative relative convergence (difficult) eg bar reading whilst gradually reducing the Rx and print size; -ve lenses whist watching TV (take care to maintain BSV and not suppression); stereoscope; miotics for 3-4 weeks (HES; dual action)
NB Most hospital departments want to see all fully accommodative strabismics. Any treatment should be taken cautiously and preferably in liaison with the HES.
Partially Accommodative Esotropia
Constant deviation but increases with accommodative effort
Associated with hyperopia but residual angle still present with when corrected
Usually amblyopia
Onset 1-3 years; insidious
BSV depends on the age of onset; usually not present
Associated vertical deviation common (IO overaction of one or both eyes)
Management
depends on the size of the residual angle.
Referral is likely in most cases
Full cycloplegic result given
Treat any amblyopia by occlusion
Re-inforcement and extension of BV – if it can be found to exist where the visual axes cross
Introduce objects closer than and further than object of fixation to try to get Px to appreciate physiological diplopia – gradually move object in towards fixation object to overcome suppression, maintaining physiological diplopia
Management
bar reading at fixation distance where axes cross
if above obtained easily , try to move point of fixation to reduce angle of deviation
Refer for surgeons opinion:- for functional result if BSV demonstrated; otherwise cosmetic
Convergence Excess
High AC/A ratio (>6:1)
Strabismus at near only OR angle much greater at near
Strabismus may only be present when looking at fine detail
Onset 2-5 years, occasionally earlier
Most have BSV, some microtropia
Amblyopia rare (except in anisometropia)
Most hyperopic but some are emmetropic or even myopic.
It is important to differentiate convergence excess from non-accommodative near SOT (has normal AC/A ratio) and undercorrected hyperopia ( eg do cycloplegic).
Management
Depends on the AC/A ratio
If >8-10∆/D, unlikely to respond conservatively
Also if near deviation is >25 - 30∆, refer for surgery
Management
Otherwise:
correction of refractive error – full cycloplegic Rx, unless Px is myopic then give a slight undercorrection
amblyopia treatment (if necessary) by PT occlusion
Bifocal spectacles – find an add that eliminates near deviation (by CT) enabling PX to maintain comfortable BSV with adequate binocular VA for all near activities
Start with +1.00 Add and increase in 0.50 steps
Try Fresnels on one month trial
Give large flat top seg set high bisecting pupil
Carefully fit and give full instructions
Gradually reduce add in time and discard if possible
Use a bar reader to establish BSV
Problem: dependency on add by young adults, poor fitting and compliance by children and wear and tear
Management
Contact Lenses – Calcutt (1984) reported that CLs (sometimes with extra +1.00D) reduced the angle by up to 15∆, often producing a latent deviation and useful BSV. Needs further trials
Miotic drugs – combined with orthoptics (particularly good for 2-5 year olds whose cooperation is poor for other methods. Use over a period of weeks)
Orthoptics for use without the Rx – minority of Pxs > 6 years; good cooperation and small deviations
Antisuppression treatment
Management
Control of deviation by extending area of BSV – move target closer
Increase binocular VA – increase –ve fusional reserves (usually needs some optical correction to achieve)
Of minimal value on its own but can be useful in conjunction with other treatment or surgery
Surgery – especially where the prognosis is poor for orthoptics or other methods have failed
Non-refractive Esotropias
Constant:
Onset 1-2 years
Strabismic amblyopia common
Often have an associated vertical deviation
Intermittent:
Near esotropia
Ortho or small SOP on distance fixation
Moderate/large SOT for near
No amblyopia
Often no significant refractive error
Normal or low AC/A ratio
Normal near point of accommodation
No reduction in angle with plus lenses
Normal sensory and motor fusion
Thought to be due to high proximal convergence or high tonic convergence
Distance Esotropia
Rare
SOT for distance, SOP for near
No significant refractive error
VA normal and equal
Full ocular movements (differentiates 6 th nerve palsy or dysthyroid eye disease)
Cyclic Esotropia
Esotropia occurring at regular intervals of time, BSV at others
Gradually becomes constant - then surgery can be considered
Often associated with a psychogenic disturbance
Consecutive Esotropia
Spontaneous – following XOT (rare – occurs with DVD)
Post-operative – following overcorrection of an XOT
Symptomatic (secondary) esotropia
Following severe visual loss in childhood, due to muscle tonus
The management of non-refractive SOT is almost always surgical.
Can try prism for distance SOT where angle < 10 ∆ (Use Fresnel lens initially – adaptation)
Correct the refractive error to remove any astenopia (independent of the deviation)
Any amblyopia must be treated.
Essential Infantile Esotropia
Occurs before 6 months (usually 3-6 months)
Usually large angle (>40 ∆)
Same angle distance and near
Crossed alternating fixation
Less than half have ambyopia with EF (Dale, 1982)
May have latent nystagmus
Sometimes have DVD
Looks like a bilateral LR palsy (distinguish by “dolls head” movement)
Nystagmus Blocking (or compensation) Syndrome
Convergent strabismus is adopted to lessen the nystagmoid movements which are reduced on convergence of the eyes
Pxs head usually turned away from side of fixing eye – produces greater convergence of this eye.
Accommodative Infantile Esotropia
Rare
Most are hyperopic
Half have a high AC/A ratio
BV weak and unstable compared with later onset accommodative strabismus
Strabismic amblyopia
Sixth Nerve Palsy
Rare in isolation, but can occur with neurological disorders eg hydrocephalus
Can be caused by trauma in forceps delivery NONE OF THESE CONDITIONS CAN BE TREATED BY REFRACTIVE OR ORTHOPTIC TREATMENT ALONE : SURGICAL TREATMENT SHOULD BE SOUGHT AS SOON AS POSSIBLE
Nature and Management of Comitant Divergent Strabismus
Divergence Excess
Manifest for distance fixation only, usually intermittently but may be constant
Most apparent during inattention, ill health and fatigue, after alcohol and in bright light
Mostly females
Little refractive error
VA usually good and equal
Usually no symptoms as the sensory adaptations are good
Px may not have known about strabismus until told by others
AC/A is normal in true, but high in pseudo-divergence excess
Management
Correction of myopia or anisometropia
Low degrees of hyperopia best left uncorrected
Most require referral for surgery
Where angle is <15∆ (rare – usually much larger) and BSV maintained most of the time, optical &/or orthoptic treatment may be of benefit – but usually only in the short term to delay surgery
Management
Orthoptics : anti-suppression exercises (only if NRC) Exercise base out prism vergences Teach physiological diplopia (so Px knows when strabismic)
Optical:negative lenses can be successful in the short term, where accommodation is good
Prisms (full base in – then gradually reduce) – short term
Tinted spectacles – useful in countries with high light intensity – again only short-term- high illumination has a dissociating effect in exodeviations – can cause suppression or closing of one eye PROBABLY BEST TO REFER FOR SURGERY FROM OUTSET
Near Exophoria (Convergence Insufficiency)
Most commonly occurs in the mid-teens when reduced convergence &/or increased myopia break down the BV, can be in adults
Rarely occurs at around 5-7 years old with an accommodative element – usually low AC/A
Typically XOP at distance XOT at near
Pxs present with symptoms (diplopia, astenopia)
Usually equal VAs, poor or no convergence, NRC and normal sensory fusion with poor positive fusional amplitude
Often myopic
Management
If strabismus is constantly manifest for near and angle >25∆ - refer
For smaller angles and only occasionally manifest:
Correct any myopia (this may be enough to make deviation latent)
Orthoptics – exercise base out prism vergences (often successful)
Prisms – base In just sufficient to enable BSV for near (Usually tolerated for distance)
gradually reduce the strength of the prism and combine with orthoptics
Occasionally try negative adds
If no improvement – refer for surgery.
Constant (Basic) Exotropia
Constant divergent strabismus, equal angles distance and near
Onset in early childhood: no symptoms, no sensory fusion
Closure of one eye in bright light
Often alternating, with equal VAs; homonymous fixation
Management
Surgical correction for cosmetic (or occasionally functional) result
Occasionally in children <7 years old – try –ve additions to eliminate strabismus on the CT in conjunction with exercises to establish BSV.
Gradually try to phase out the –ve add over several years.
If a divergent strabismus has a vertical component – orthoptics not successful
Consectutive Exotropia
Spontaneous – usually occurs following early onset partially accommodative esotropia with a high degree of hyperopia.
Develops as the amplitude of accommodation decreases, or precipitated by the late correction of hyperopia
Post-operatively – usually several years after surgical correction of accommodative SOT – especially if hyperopic correction is now prescribed
Management
Partial correction of hyperopia + Base out prism
vergence exercises sometimes helps
Remedial surgery
Symptomatic (Secondary) Exotropia
Due to severe loss of vision in one eye in adult life
Management = Cosmetic surgery
Onset of Exotropia before 1 year old
Usually symptomatic (or secondary) due to visual loss from birth
Rarely congenital exotropia, often with nystagmus and DVD
Management
No optometric treatment - refer
Vertical Heterophoria Hyperphoria
a potential deviation of one eye upwards which becomes an actual deviation when the two eyes are dissociated and recovers when the dissociating factors are removed
15-30% patients have a measureable hyperphoria (not necessarily decompensated).
Two types
Secondary
Primary
Secondary hyperphoria
Horizontal heterophoria
Incomitant deviations
Tilted spectacles
Primary/True hyperphoria –
seldom >3
is considered largely due to slight anatomical misalignment of the eyes or orbits or muscle insertions for which there is physiological compensation
Investigation
Symptoms
Marked with low degrees
Frontal HA’s; pain, soreness of lid margins
Head tilt alleviates symptoms
Prefers monocular vision
Motility - Evaluate carefully to ensure there is no paresis
Refraction – very important to assess binocular balancing, and check if both images are aligned (Mallett units are useful for this or infinity balance techniques).
Management
Remove cause of decompensation ie pay attention to working conditions, any stress or ill health of the patient
RE: Rx may alleviate symptoms anisometropia – take care to prevent vertical problems.
Orthoptics: if associated with horizontal phoria, development of horizontal fusional reserves may result in the vertical phoria becoming compensated.
Prisms: perhaps best method of treatment especially for primary vertical hyperphoria. The minimum amount of prism should be prescribed and the prism should be divided between the two eyes or the maximum prism is placed before the non-dominant eye.
Surgery: May be necessary in high hyperphoria or Incomitancy
These deviations show variations in horizontal magnitude as the fixation point moves up and down.
may be associated with convergence or divergence and occur in both heterotropia and heterophoria.
Convergence
A pattern Increased convergence on elevation Decreased convergence on depression
V pattern increased convergence on depression decreased convergence on elevation
Divergence
A pattern increased divergence on depression decreased divergence on elevation
V Pattern -increased divergence on elevation decreased divergence on depression
The aetiology of A and V patterns
is not agreed and may vary between individuals although over- and under-action of the oblique muscles appears to be the most likely cause.
It is thought that bilateral inferior oblique overaction produces a V pattern in both eso and exo deviations; and bilateral superior oblique overactions produces the A pattern.
Cyclodeviations
Incidence not known
Usually caused by an imbalance between the muscle pair affecting intorsion (SO and SR) and the muscle pair inducing extorsion (IO and IR ).
Doubtful if cyclophoria exists as a primary cyclophoria although authorities give methods for its detection.
Pseudostrabismus
Epicanthus in infants can be more or less pronounced.
Epicanthus can obscure the inner canthus giving rise to the appearance of esotropia when none is present.
In time epicanthal fold usually disappears with the development of the bridge of the nose
Other examples of pseudostrabismus include
facial asymmetry
failure of the optical (centres of the cornea and lens) and visual axis (fovea to fixation point) to coincide
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