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Ординатура / Офтальмология / Английские материалы / Pickwell's Binocular Vision Anomalies 5th edition_Evans_2007

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15 PICKWELL’S BINOCULAR VISION ANOMALIES

Esotropia

Acquired non-accommodative esotropia is characterized by: sudden onset usually between 6 and 24 months of age, normal binocular vision before onset, constant comitant deviation between 30–70 , normal AC/A ratio, negligible effect of refractive correction on the angle, positive family history and normal neurological health (Frane et al 2000).

Some cases occur following a childhood febrile illnesses. These cases of strabismus can usually be treated by correcting any refractive error and strengthening the fusional reserves by the type of exercise described in Chapter 10. These patients have had binocular vision prior to the illness and if they are seen soon after the recovery in general health the binocular vision can often be restored. However, care must be taken to be sure that the strabismus was not present before the illness, as parents are not always certain about this.

In some cases, the onset of the strabismus may follow emotional trauma. These cases are very much more difficult to treat as the traumatic scars do not have any optometric treatment and recovery from them may be very slow. Caution should be exercised before any treatment is given. A refractive correction and fusional reserve exercises may help, but the treatment can be very protracted. Patients in heroin detoxification become less exo/more eso at distance and this can trigger acute comitant esotropia (Firth et al 2004).

Some esotropia may apparently occur spontaneously in children over the age of 1 year. A careful check for pathology must be carried out. This requires an ophthalmoscopic check for the white patches on the fundus indicative of retinoblastoma. This is rare but serious and, particularly where central areas of the fundus are involved, a strabismus may be the first sign. It usually occurs before the age of 4 years. Other neurological disorders usually produce non-comitant deviations so that a motility test is essential. A sixth nerve palsy is the most likely. This will show as a restriction of abduction, and an increase in the angle of the strabismus in distance vision and as the patient looks towards the affected side (Ch. 17).

Non-accommodative esotropia with a spontaneous onset can occur without such pathology and the aetiology may be an idiopathic increase in tonic convergence (Frane et al 2000). It often occurs intermittently at first. If it can be detected at this stage, developing divergent fusional reserves can help to check it. If there is a significant anisometropia that may lead to amblyopia, this should be corrected. Although the strabismus is non-accommoda- tive, some cases benefit from bifocals, which, even with a normal AC/A ratio, may correct the deviation at near. These are prescribed in the same way as for convergence excess deviations. Clearly, the bifocal will not help distance vision, but normal binocular vision at near is better than no binocular vision at all. This also makes eye exercises easier as the patient can build on the binocular vision at near.

Another type of non-accommodative esotropia that can occur spontaneously is a form of divergence weakness esotropia, typically in an elderly

258patient with a sudden onset of horizontal diplopia during distance vision. This has been termed divergence paralysis, although whether divergence is

OVERVIEW OF THE MANAGEMENT OF STRABISMUS

15

an active process remains controversial (Lim 1999). The condition should

 

 

be differentially diagnosed from a bilateral sixth nerve palsy (where an

 

 

incomitancy will be detected on motility testing) but still requires referral

 

 

to investigate possible neurological causes.

 

 

Von Graefe and later Bielschowsky described a type of acute-onset

 

 

esotropia that is associated with high myopia. It usually occurs in young

 

 

adults at distance and then at near, and is associated with diplopia (von

 

 

Noorden 1996). Usually this is comitant (rarely, there is limited abduc-

 

 

tion), unlike another type of esotropia associated with very high myopia

 

 

( 15 D or more) in which there appears to be a limitation of motility in all

 

 

directions of gaze (von Noorden 1996). Indeed, acute onset esotropia in

 

 

adults was reported to be associated with some degree of myopia in nine out

 

 

of ten cases, but all cases regained stereoacuity after surgery (Spierer 2003).

 

 

When non-accommodative esotropia has become established for several

 

 

years, non-surgical treatment may not be successful. Prism relief may assist

 

 

(Ch. 14). Extraocular muscle surgery may be indicated when 20 or more

 

 

of esotropia remains after full correction of the refractive error or if optical

 

 

correction has no effect on the angle (Frane et al 2000). The chances of

 

 

achieving stereopsis after surgery for esotropia improve if the onset is after

 

 

the second year, but coexisting hypertropia worsens the prognosis (Kora

 

 

et al 1997).

 

 

Exotropia

 

 

Exotropia (constant or intermittent) affects about 1% of children by the age

 

 

of 11 years, with diagnosis most common at the ages of 3 years and 9 years

 

 

(Govindan et al 2005). The most common form of exotropia (Mohney &

 

 

Huffaker 2003) to occur under the age of 19 years is intermittent exotropia

 

 

(50%); other common types are exotropia associated with neurological

 

 

abnormalities (21%; mostly cerebral palsy or developmental delay), con-

 

 

vergence weakness (12%) and sensory exotropia (10%; mostly optic nerve

 

 

hypoplasia or cataract). Intermittent exotropia is more likely to be associ-

 

 

ated with neurological disease (e.g. developmental delay, cerebral palsy,

 

 

attention deficit disorder) if it is of the convergence weakness type rather

 

 

than the other types listed below (Phillips et al 2005).

 

 

The management options for intermittent exotropia were reviewed by

 

 

Coffey et al (1992) and negative lens therapy often achieves long-term suc-

 

 

cess (Caltrider & Jampolsky 1983). For many patients with intermittent

 

 

exotropia, the deviation reduces in angle and/or changes to an exophoria

 

 

over time, and this seems to happen regardless of the management of the

 

 

case (Rutstein & Corliss 2003). In contrast, another study found that more

 

 

than 50% of children with intermittent exotropia have an increase of

 

 

10 or more within 20 years of their diagnosis (Nusz et al 2006).

 

 

Divergent strabismus can be classified under four headings, there being

 

 

a different management and prognosis for each type.

 

 

(1) Divergence excess This presents typically as an intermittent divergent

 

259

 

 

 

strabismus for distance vision only. It becomes most apparent during periods

 

 

 

 

 

15

 

PICKWELL’S BINOCULAR VISION ANOMALIES

 

 

 

of inattention and day-dreaming, and is worse during ill health. Patients

 

 

 

are photophobic and bright light can cause the strabismus to occur (Eustace

 

 

 

et al 1973, Wiggins & von Noorden 1990). Most patients are female and

 

 

 

there is little refractive error. Because of its intermittent nature, the acuities

 

 

 

are generally good and nearly equal. There are usually no symptoms as there

 

 

 

are sensory adaptations when the eye is deviated. Patients usually seek

 

 

 

advice because some relative tells them that one eye sometimes deviates

 

 

 

(see also Ch. 8). Where the V syndrome exists, optometric treatment may

 

 

 

be more difficult.

 

 

 

Divergence excess is further discussed in Chapter 8, where the distinc-

 

 

 

tion is drawn between true and simulated divergence excess. It is also

 

 

 

noted in Chapter 8 that some authors have suggested that occlusion may

 

 

 

be an effective treatment for intermittent exotropia, although there have

 

 

 

been no randomized controlled trials.

 

 

 

The condition is usually improved by eye exercises designed to overcome

 

 

 

sensory adaptations and to build up the convergent fusional reserves. Also

 

 

 

useful are exercises to teach an appreciation of physiological diplopia and

 

 

 

the movement of the diplopic images during changes of fixation from near

 

 

 

to distance and back. If the patient has adequate accommodation and a mod-

 

 

 

erate to high AC/A ratio, it may be possible to help this deviation refractively

 

 

 

by prescribing distance glasses that are overminused with a near addition

 

 

 

(e.g. 2.00 DS at distance with a 2.00 add at near) to prevent the patient

 

 

 

developing an eso-deviation at near (Mallett 1988b).

 

 

 

(2) Near vision exotropia This is also called ‘convergence weakness type’

 

 

 

strabismus. The onset is usually in the mid-teens when binocular vision

 

 

 

breaks down for near fixation. It seems that binocular vision has been

 

 

 

maintained by the high convergence impulses that exist in children, but

 

 

 

in these cases it cannot be sustained for near when these impulses lessen.

 

 

 

Binocular vision may also break down for distance vision but the angle of

 

 

 

the strabismus is greater for near. These patients complain of symptoms

 

 

 

including diplopia. They are usually myopic and have equal acuities.

 

 

 

If the angle is over 20 , it is not possible to restore binocular vision by

 

 

 

optometric methods and the patient should be referred for a surgeon’s opin-

 

 

 

ion. In other cases, treatment may not be lasting. The approach is to correct

 

 

 

any significant refractive error and increase the convergent fusional reserves.

 

 

 

With smaller angles, prism relief is also useful. Some patients with adequate

 

 

 

amplitude of accommodation respond well to the prescribing of reading

 

 

 

glasses that are ‘overminused’. The minimum ‘negative add’ to transform the

 

 

 

near exotropia into a compensated exophoria should be prescribed and

 

 

 

the effect of this on distance ocular motor balance should be investigated.

 

 

 

Care must be taken to ensure that the glasses do not cause an eso-deviation

 

 

 

for distance vision or that they are only worn for reading. An alternative is

 

 

 

to prescribe executive bifocals and glaze them upside down (p 12).

 

260

 

(3) Basic exotropia In these cases, there is a constant divergent strabismus

 

 

of approximately equal angle for distance and near vision. It is often

 

 

 

OVERVIEW OF THE MANAGEMENT OF STRABISMUS

15

alternating with nearly equal acuities. As the onset is likely to be early in

 

 

life, there are no symptoms.

 

 

Any significant refractive error is corrected, following the general guide

 

 

of maximum minus or minimum plus. In children young enough to have

 

 

an adequate amplitude of accommodation, the effect of prescribing ‘nega-

 

 

tive additions’ to the prescription (overminusing or underplussing) can be

 

 

tried, as described in Chapter 14. Exercises for simultaneous binocular

 

 

vision may also be required. Divergent strabismus is sometimes accompan-

 

 

ied by a vertical component, which will make eye exercises more difficult.

 

 

Patients may be better with surgical correction for cosmetic reasons.

 

 

(4) Consecutive exotropia There are a number of circumstances in which a

 

 

convergent strabismus may become divergent. It is sometimes seen in

 

 

patients who had accommodative esotropia on which an operation was per-

 

 

formed in early childhood. No spectacles have been worn but in adolescence

 

 

or adulthood these patients develop symptoms due to the hypermetropia.

 

 

Correction of the refractive error relieves the facultative accommodation

 

 

and a divergent strabismus with diplopia occurs. A partial correction and

 

 

convergent fusional reserve exercises can sometimes help to maintain

 

 

binocular vision. Other cases need further surgery.

 

 

Vertical strabismus

 

 

About 1 in 400 people develop vertical strabismus by the age of 18 years

 

 

(Tollefson et al 2006). When vertical strabismus presents in adulthood it is

 

 

usually the result of an incomitant deviation (Ch. 17), usually fourth nerve

 

 

palsy (Tollefson et al 2006). This is a major reason why nearly 90% of cases

 

 

of vertical strabismus are hypertropia, and hypotropia is very likely to be

 

 

Brown’s syndrome (Tollefson et al 2006) (Ch. 17). Hypertropia can rarely

 

 

be exacerbated by convergence and accommodation (Thomas et al 2005).

 

 

Spasm of the near reflex

 

 

Aetiology

 

 

The term spasm of the near reflex (Rutstein 2000) is more accurate than its

 

 

synonym, convergent spasm (Bishop 2001), since all three components of the

 

 

near triad (convergence, accommodation, miosis) are typically involved

 

 

(Rutstein 2000). It usually has a psychogenic origin but can be organic

 

 

(Bishop 2001), when there may be other clinical findings such as nystag-

 

 

mus or papilloedema (Rutstein 2000).

 

 

Investigation

 

 

Symptoms include headache, visual discomfort, dizziness and print blur-

 

 

ring, doubling, becoming smaller or merging. A cycloplegic refraction is

 

 

indicated to rule out latent hypermetropia (Box 2.1). The condition is

 

 

characterized by intermittent and variable episodes of esotropia, pseudomy-

 

261

 

opia and pupillary miosis. There may also be limited abduction and the

 

 

 

 

 

PICKWELL’S BINOCULAR VISION ANOMALIES

condition has been observed in patients with previously well controlled accommodative esotropia (Rutstein 2000).

Management

The underlying cause should be treated, if it can be identified. The condition does not usually respond to exercises but a near addition may help (Bishop 2001) and cycloplegic agents (e.g. cyclopentolate 1%) are sometimes helpful (Rutstein & Marsh-Tootle 2001).

Clinical Key Points

At every visit look for active pathology: if present refer

Infantile esotropia syndrome does not respond to optometric management. Exclude high hypermetropia and refer

Only treat a motor deviation in strabismus if any sensory adaptation is very superficial or can be eliminated with treatment

Microtropes are very often asymptomatic and best left alone (Ch. 16)

Large (more than about 20 ) deviations are difficult to treat and surgery is often the most appropriate management

If you find an esotropia at near, suspect hypermetropia. If hypermetropia is not readily apparent, do a cycloplegic refraction. If you find significant hypermetropia in esotropia, then prescribe

If hypermetropia is causing accommodative esotropia, the hypermetropia will probably require correction for life

‘Negative adds’ can be an effective treatment for many cases of exotropia

262

MICROTROPIA 16

Microtropia (Lang 1966), or microsquint, may be found as an apparently primary condition or may be present as a residual deviation after the treatment of a larger strabismus. It has been suggested that it has inherited characteristics (Burian & von Noorden 1974). Anisometropia is often a major factor and a foveal scotoma results from the confusion of the blurred image with the sharp one in the other eye. The condition has also been called Parks’ monofixational syndrome (Parks & Eustis 1961, Parks 1969). Typically, microtropia develops before the age of 3 years but it may break down into a larger-angle strabismus and give the impression that a strabismus has come on in later childhood. It is usually an eso-deviation but microhypertropia (Lang 1966) and microexotropia (Stidwill 1998) have also been described.

Classification

Primary microtropia is used to describe microtropia when there is no prior history of a larger deviation and secondary microtropia occurs when a primary comitant larger-angle deviation has been reduced as a result of treatment by optical or surgical methods or by exercises (Houston et al 1998). Another cause of secondary microtropia is a foveal lesion. Secondary microtropia is more common than primary microtropia (Griffin & Grisham 1995).

Clinical characteristics

The terminology surrounding small-angle strabismus has been very con-

 

fused but microtropia is now recognized as having certain characteristics in

263

very many cases. These characteristics are listed below and are incorporated

16 PICKWELL’S BINOCULAR VISION ANOMALIES

into a diagnostic algorithm at the end of the chapter. It is hoped that this will help to standardize the diagnosis of microtropia.

(1) Small angle. The microtropia is less than 6 in angle. Some authors have argued for a less stringent criterion such as less than 10 (Lang; cited by Mallett 1988a); other authorities have advocated a more stringent criterion, such as less than 5 (Caloroso & Rouse 1993, p 26). The deviation may not show on the cover test; not because it is too small but because it is a fully adapted strabismus (see below). Microtropia is usually constant at all positions of gaze and fixation distances.

(2)Anisometropia. There is usually a difference between the refractive errors in the two eyes (Hardman Lea et al 1991) of more than 1.50 D. Occasionally, microtropia will be found in patients with equal refractive errors.

(3)Amblyopia. There is reduced acuity in one eye and, as the deviation may not be apparent on the cover test, the amblyopia may be the first indication of the microtropia. Usually the acuity is only reduced 1–2 lines to 6/9 or 6/12. Very rarely, microtropia can be alternating, when there will be no amblyopia.

(4)Eccentric fixation. Central fixation is lost in microtropia and there is likely to be a suppression scotoma in the foveal area of the amblyopic eye. The angle of the eccentric fixation is usually the same as the angle of the strabismus, and this is the reason why the eye does not move on the cover test: the area of the retina on which the image falls in binocular vision is the same as the eccentrically fixating area (the area used for fixation when the other eye is covered). Occasionally in microtropia, the degree of eccentric fixation is less than the angle of the strabismus and in these cases a very small cover test movement may be seen. Some authors (e.g. Jennings 1985) define microtropia as a strabismus in which no movement is seen on the cover test and hence would not classify this latter type as microtropia.

(5)Anomalous correspondence. Harmonious anomalous retinal correspondence (HARC) is present in microtropia. Therefore, in most cases there will be identity of the retinal area on which the image falls in the patient’s habitual vision with both the area used for fixation and the anomalously corresponding area. This has been referred to as microtropia with identity, but most microtropia is of this type. In these cases, the strabismus is said to be fully adapted.

(6)Peripheral fusion. The eyes in microtropia seem to be held in the nearly straight position of the small angle by the fusional impulses provided by peripheral vision. A form of ‘pseudofusional reserves’ can be measured. During the cover test it is therefore important to position the cover close to the eye in order to ensure complete dissociation; otherwise peripheral fusion may reduce the magnitude of any ocular movement and prevent an accurate diagnosis.

(7)Pseudoheterophoria. In many cases of microtropia, the angle of the deviation may increase on the alternating cover test or even if one eye is

264

covered for a slightly longer time than normal for the cover test. When

MICROTROPIA 16

the cover is removed from both the eyes, the eye that was last covered will be seen to return to the microtropia position. It is as if a heterophoric movement is superimposed on the strabismus. The ‘pseudoheterophoria’ may be larger and more obvious than the microtropia, which may not show at all on the cover test. This cover test recovery movement can be described as an anomalous fusional movement. Mallett (1988a) felt that these cases did not have a strabismus but in fact had a heterophoria with normal retinal correspondence (NRC) and a gross fixation disparity. This fixation disparity is much larger than that normally found in heterophoria but does not cause diplopia because of a large foveal suppression area in the strabismic eye. Pickwell (1981) suggested a sequence of events that linked these features and could explain the development of at least some cases of microtropia. He argued that a decompensating heterophoria leads to an increasing fixation disparity, which in time becomes associated with an enlargement of Panum’s area and an increase in the deviation. This results in a microtropia with identity. It is interesting that Pickwell suggested an enlargement of Panum’s area, as had Goersch (1979), in contrast to the foveal suppression that Mallett proposed and that is detected with the Mallett foveal suppression test (Jennings 1996; see Fig. 4.7).

(8)Stereopsis. A low grade of stereopsis has been reported in microtropia (Okuda et al 1977), although it is not always detected with standard clinical tests (Pickwell & Jenkins 1978). Cooper & Feldman (1978) argued that all cases of strabismus, including microtropia, show subnormal performance at random dot stereopsis tests.

(9)Symptoms. There are usually no symptoms and a good cosmesis.

Investigation and diagnosis

The investigation and diagnosis of microtropia ensue from a full routine eye examination, but the following aspects are particularly useful in the detection of this condition. These are summarized in Box 16.1.

Amblyopia

The presence of amblyopia in one eye is usually the first clue that

 

microtropia may be found. The amblyopic eye usually shows the crowding

 

phenomenon referred to in Chapter 13, i.e. single letter acuity is better than

 

line acuity. The foveal scotoma may also result in patients missing out letters

 

when reading lines of Snellen letters, or they may read the line more easily

 

backwards (see Fig. 13.1).

 

The patient should be tested for eccentric fixation (Ch. 13 and Appendix 6).

 

Eccentric fixation in microtropia is usually parafoveal and slightly nasal and

 

superior to the fovea in microesotropia. The other diagnostic features of stra-

265

bismic amblyopia are summarized in Table 13.1.

16

PICKWELL’S BINOCULAR VISION ANOMALIES

 

Box 16.1 Algorithm to assist in the diagnosis of

 

microtropia

 

All the following characteristics must be present for a diagnosis of microtropia

 

Angle 10

 

Amblyopic eye with morphoscopic acuity at least 1 line worse than dominant

 

eye, unless alternating microtropia (rare)

 

Eccentric fixation (Ch. 13), unless rare alternating microtropia

 

HARC detected by Bagolini striated lens test or by modified Mallett unit (Ch. 14)

 

And at least three of the following characteristics should be present

 

Angle 6

 

Anisometropia 1.50 D

 

Microtropia with identity: angle of anomaly angle of eccentric fixation, so

 

no movement when dominant eye is covered

 

Monofixational syndrome: apparent phoria movement on cover test

 

Motor fusion: ‘pseudofusional reserves’ can be measured

 

Stereopsis of 100 or more on contoured tests such as Titmus circles or

 

Randot contoured circles

 

Four-prism dioptre test shows positive response

 

Lang’s one-sided scotoma demonstrated with Amsler charts

 

Cover test

 

For microtropia without identity, the diagnosis is usually made on the

 

basis of a positive unilateral cover test result of between 1 and 10 . But as

 

explained above, microtropia with identity is unlikely to be detected as a

 

strabismic movement with the cover test. There may be an apparent

 

heterophoria movement when the cover is removed and this could result

 

in the microtropia being missed.

 

Four prism dioptre test

 

In this test (Irvine 1948), a 4 base-out prism is placed before one eye and

 

the eye movements are observed. The typical response in normal eyes

 

(Ciuffreda & Tannen 1995) is a small initial vergence movement (which

 

may not be seen) followed by a conjugate saccade (version movement) and

 

then a symmetric vergence movement. The theory behind the test (Frantz

 

et al 1992) is that, in microtropia, if the prism is placed before the strabismic

266

eye the image will move within the suppression area, and there will be no

movement of either eye. If the prism is placed before the non-microtropic

MICROTROPIA 16

eye, both eyes will make the initial version movement but the microtropic eye will fail to make the subsequent vergence movement. The test should only work if the patient is fixating an angular, isolated target on a large featureless background. If this is not the case (e.g. if the patient fixates a letter chart), other detail in the field of view will also appear to move, not just the fixation target. This artefact probably explains much of the confusion that has arisen from this test.

In some cases where there is amblyopia in one eye and no movement on the cover test it is important to differentiate microtropia from organic amblyopia. It is possible that a central scotoma in organic amblyopia could cause a 4 test result similar to that in microtropia. In these cases, it may be useful to occlude the good eye and repeat the 4 base-out test monocularly. If there is a large pathological scotoma, as in many cases of organic amblyopia, then there will be no monocular response to the prism. Because any monocular suppression area in a microtropic eye is likely to be lighter than the larger suppression area that occurs under binocular viewing, a microtropic eye should make a version movement to a 4 lens that is introduced monocularly.

Although the 4 base-out test has been proposed as a diagnostic test for microtropia, the test often gives atypical responses, particularly in esophoric patients where the 4 base-out may correct the eso-deviation (Romano & von Noorden 1969). Frantz et al (1992) also advised caution in using this test. They found test–retest repeatability to be low and that normal and microtropic children and adults exhibit many atypical responses. I speculate that this may be because too little attention has been paid to the test target that is used.

Harmonious anomalous retinal correspondence

The Bagolini lens test or Mallett modified OXO test will show the response typical of HARC (Ch. 12). The HARC is usually deeply ingrained and will require a neutral density filter value of 1.0 log unit or more to suppress the Bagolini streak.

Amsler charts

The scotoma may show on the Amsler charts or as a disturbance of a page of print (p 195] and Appendix 6).

Stereopsis

This will depend on the type of test used. The TNO test measures random

 

dot stereopsis and the patient is unlikely to do better than 2000 of arc,

 

whereas the result from the Randot or Titmus stereotest circles, which

 

measure contoured stereopsis, may be as high as 100 of arc (Stidwill 1998).

 

Griffin & Grisham (1995) state that in microtropia central stereopsis is

267

absent or greatly reduced, especially with random dot targets.