Binocular Vision - Evans
Binocular Vision - Evans
Binocular Vision - Evans
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Preface
vii
Preface
viii
Further reading
Evans, B.J.W. and Doshi, S. (2001). Binocular Vision and
Orthoptics. Butterworth-Heinemann: Oxford.
Evans, B.J.W. (2002). Pickwells Binocular Vision Anomalies.
Butterworth-Heinemann: Oxford.
Evans, B. (2004).The Diploma in Orthoptics. Part 1: A how to
guide. Optician 226, 2627.
Noorden, G.K.V. and Campos, E. (2002). Binocular Vision and
Ocular Motility:Theory and Management of Strabismus. Mosby: St
Louis.
Rabbetts, R. B. (2000). Bennett & Rabbetts Clinical Visual Optics.
Butterworth: Oxford.
Rosenbaum, A.L. and Santiago, A. P. (1999). Clinical Strabismus
Management.W.B. Saunders & Company: Philadelphia.
Foreword by series
editors
Acknowledgment
xi
Overview of
binocular vision
anomalies
10
Comitant/incomitant
Deviation
Heterophoria
Strabismus
Compensated/
decompensated
Constancy
constant/intermittent
Testing distance
far/inter/near
Test distance
far/inter/near
Direction of deviation
exo/eso/hyper/cyclo
Sensory adaptation
HARC/suppression
Laterality
unilateral/alternating
Direction
exo/eso/hyper/hypo/cyclo
Figure 1.1
How do I investigate?
deviations may be strabismic or heterophoric and strabismic
conditions may be comitant or incomitant.
Incomitancy (Chapter 6) is defined as a deviation that varies in
different positions of gaze and that varies depending on which eye
is fixing. Incomitant deviations affect about 0.5% of the population.
A strabismus (Chapter 3), also known as a heterotropia or
squint, occurs when the visual axes are deviated: where the line
of sight of one of the eyes does not fall on the object of regard.
Strabismus affects around 2.54% of the population. A strabismus
can be constant or intermittent, and can be unilateral or
alternating.Young patients develop sensory adaptations to a
strabismus, typically harmonious anomalous retinal
correspondence (HARC) or suppression (see p. 42).
Most people do not have a strabismus and the eyes are kept in
perfect, or very close to perfect, alignment. But when one eye is
covered, or the two eyes are dissociated (prevented from viewing
the same scene), most people develop a latent deviation
(heterophoria). A heterophoria (Chapter 2) is a normal finding: it
is only apparent when the eyes are dissociated and is not present
under normal viewing conditions. Occasionally, a heterophoria
may decompensate, when it can cause symptoms and in some
cases might break down into a strabismus. As well as being
classified as compensated or decompensated, a heterophoria can
also be classified according to the testing distance.
Both strabismus and heterophoria can be classified according
to the direction of the deviation: eso for when the visual axes
turn inwards, exo for outwards, hyper for upwards, hypo for
downwards, cyclo for cyclorotation. Heterophoria is sometimes
described as a latent strabismus, but this term can be confusing
since a heterophoria is a normal finding which is usually not a
cause for concern.
How do I investigate?
Symptoms and history are crucial to the investigation of binocular
vision anomalies, and the symptoms of decompensated
heterophoria are listed in Table 2.1. Most children with strabismus
How do I investigate?
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How do I investigate?
watched as the cover approaches since if a dissociated vertical
deviation (p. 56) is present then a movement of an eye is often
seen before the cover actually reaches the eye.
The use of the cover/uncover test to detect strabismus is
described in Table 1.1, and its use to detect heterophoria is
described in Table 1.2. It is useful to estimate the angle of any eye
movements, and a method for this is described in Table 1.3. In
Table 1.1
1. As the cover moves over one eye then the practitioner should
watch the uncovered eye. It is the behavior of the uncovered eye
that reveals whether the patient has a strabismus
2. For example, as the left eye is covered the practitioner should
watch the right eye. If the right eye moves then this suggests that
there is a strabismus in this eye (Figure 1.2, the behavior of the
right eye from (a) to (b))
3. The direction and amplitude of the movement should be estimated
(see Table 1.3)
4. The cover is then slowly removed from the eye that has been
covered and this eye is observed to see if a movement occurs,
signifying heterophoria (see Table 1.2)
Table 1.2
1. As the cover is slowly removed from the eye that has been
covered then this eye is observed to see if a movement occurs. If a
movement occurs as an eye regains fixation after being covered
then this indicates a heterophoria (e.g., in Figure 1.2 the right eye
moves out as the cover is removed from (b) to (c))
2. The direction and amplitude (Table 1.3) of the movement should
be estimated
3. The quality of this recovery movement should also be recorded.
This gives an objective indication of how well the patient is able to
compensate for the heterophoria (Table 1.4). A higher grading in
Table 1.4 is more likely to be associated with decompensation and
thus more likely to require treatment
Table 1.3
testing
How do I investigate?
Table 1.4 A grading system that can be used to gauge cover test
recovery in heterophoria
Grade
Description
Slightly slow/jerky
Table 1.5
10
With the alternating cover test, as the angle builds towards the
total angle, it becomes easier to detect vertical deviations which
are typically smaller than horizontal deviations
Fusional reserves
Motor
Sensory
fusion
fusion
Fusion lock
Compensated heterophoria
or
Decompensated heterophoria
or
Strabismus
Figure 1.4 A simple model of binocular vision (reproduced with
permission from Evans, B.J.W. (2002) Pickwells Binocular Vision Anomalies,
4th edition, Butterworth-Heinemann)
11
12
What do I do?
When binocular vision anomalies require an intervention then
there are several possible options:
1. treat with eye exercises
2. treat with refractive modification
What do I do?
3.
4.
5.
6.
7.
13
Heterophoria
What is heterophoria?
16
How do I investigate?
16
Symptoms
16
Cover test recovery
17
Mallett fixation disparity test (aligning prism)
17
Fusional reserves
19
Foveal suppression test
20
Other tests relevant to the assessment of
heterophoria
22
Summary of the diagnosis of decompensated
heterophoria
23
When do I need to do something?
26
What do I do?
27
Remove the cause of decompensation
27
Eye exercises
28
Refractive modification
32
Prismatic correction
33
Surgery
33
Specific types of heterophoria
35
Mixed esophoria (basic esophoria)
35
Divergence weakness (distance esophoria)
36
Convergence excess (near esophoria)
36
Mixed exophoria (basic exophoria)
36
Divergence excess (distance exophoria)
36
Convergence weakness (near exophoria)
37
Convergence insufficiency
37
Binocular instability
39
Heterophoria
16
What is heterophoria?
Heterophoria is a tendency for the eyes to move out of
alignment when one is covered or when they view dissimilar
objects.Types of heterophoria are exophoria, esophoria,
hyperphoria, and cyclophoria (see pp. 23). Some authors
used to call a heterophoria a latent strabismus or latent squint.
One way of thinking about heterophoria is to consider the
resting position of the vergence system. In total darkness the eyes
adopt a resting position (tonic vergence) where, for most people,
they align on an imaginary object about 12 m away. Distance
vision can be thought of as requiring divergence away from this
resting position and similarly near vision requires convergence
away from this resting position.When one eye is covered, that
eye is dissociated and tends to revert towards the resting
position.That reversion is the heterophoria.Typically, the
heterophoria is visualized during the cover test when the cover is
removed and the eye re-aligns to take up fixation.This way of
thinking about heterophoria in terms of the typical resting
position explains why most people have a small esophoria for
distance fixation and a slightly larger exophoria at near.
How do I investigate?
Symptoms
Symptoms are important in determining whether a heterophoria
is decompensating.Typical symptoms that may be present in
decompensated heterophoria are listed in Table 2.1. It was
noted on p. 10 that some patients do not appreciate that they
have symptoms until these have been corrected. Occasionally,
patients may not have symptoms from a decompensating
heterophoria because they develop a sensory adaptation
(e.g., foveal suppression; see below). Additionally, many of the
symptoms in Table 2.1 are non-specific: they could result from
other, non-orthoptic, causes. So clinical tests are necessary to
How do I investigate?
determine whether any symptoms are likely to result from an
orthoptic problem and therefore to be treatable by orthoptic
means.These tests are described below.
Table 2.1
Number
Type of symptom
Symptom
1
2
3
Visual
Blurred vision
Double vision
Distorted vision
4
5
6
Binocular
7
8
9
Asthenopic
Headache
Aching eyes
Sore eyes
10
Referred
General irritation
17
Heterophoria
18
Figure 2.1
How do I investigate?
fusion lock. It is the presence of such a good central and
peripheral fusion lock on the Mallett unit that makes this test so
valuable. Since the test very closely resembles normal viewing
conditions, if a fixation disparity is detected during this test then
it is also very likely to be present during everyday life.
Although the Mallett unit test detects fixation disparity, the
degree of fixation disparity is not measured with the test. Instead,
the test measures the aligning prism (associated heterophoria) or
aligning sphere.This is the prism or sphere that is required to
align the strips.This is clinically relevant for two reasons:
the size of the aligning prism (with a genuine Mallett unit) has
been correlated with the likelihood of the patient having
symptoms
the aligning prism or sphere is usually equal to the prism or
sphere that is required to render the heterophoria
compensated.
The exact method and questions that are used with the Mallett
unit fixation disparity test have been shown to be important.
These are summarized in Table 2.2. All research on this test has
used genuine Mallett units.There are some copies which often
have differences in design and it is unclear whether these will give
the same results.
Using the norms in Table 2.2, the near Mallett unit fixation
disparity test has a sensitivity and specificity for detecting
symptomatic heterophoria of about 7580%. For a clinical test,
these results are impressive, but are of course not 100%.
Therefore other clinical tests are sometimes useful (see Table 2.5)
and these will now be described.
Fusional reserves
Figure 1.4 shows that a key factor determining a persons ability to
compensate for a heterophoria is the fusional reserve that they use
to overcome the heterophoria.This is called the opposing
fusional reserve and, for example, is the convergent fusional
reserve in exophoria.The convergent fusional reserve can be
measured by introducing base out prisms.This can be a source of
19
Heterophoria
20
Table 2.2 Test method and norms for the near Mallett unit
fixation disparity test
1. Without the polarized visors, show the patient the horizontal test
(green strips vertically placed above and below the O X O) and
check that the patient perceives both green strips in perfect
alignment. Increase ambient illumination
2. Introduce the polarized visor and ask the patient if the two green
strips are still exactly lined up, whilst they keep their head still
3. If the patient says that they are exactly lined up, then ask if one or
both of the strips ever moves a little
4. If (2) or (3) reveals a misalignment, then introduce prisms in front
of the relevant eye(s) or spheres in front of both eyes until the
strips are aligned and stay aligned. For an exo-slip (right eyes strip
moving to the patients left) add base in prism or minus spheres
5. After changing the prism or spheres, have the patient read a line of
text to stabilize their binocular vision before re-testing for fixation
disparity
6. Make a note of the results in (4), remove the aligning prism or
sphere, and then repeat for the vertical test
7. An aligning prism of 1 or more in pre-presbyopes or 2 or more
in presbyopes is very likely to be associated with symptoms
8. With pre-presbyopes, it is therefore useful to initially introduce
prisms in 1/2 steps
How do I investigate?
Table 2.3
1.
2.
3.
4.
5.
6.
First, the patient should report when the target goes blurred,
which is recorded as the blur point. Sometimes, there is no blur
point
7.
8.
The prism is then reduced until single vision and ocular alignment
is restored
9.
10. Results can be compared with norms, but the precise norms
depend on the test conditions. It is more meaningful to relate the
results to the heterophoria
11. Sheards criterion says that the fusional reserve (to blur or if no
blur to break) that opposes the heterophoria should be at least
twice the heterophoria. For example, if a patient has 8
exophoria at near, then their convergent fusional reserve should
be at least 16.This criterion works well for exophoria
12. Percivals criterion says, in essence, that the fusional reserves should
be balanced so that the smaller fusional reserve is more than half
the larger one. For example, if the convergent fusional reserve to
break point is 20, then the divergent fusional reserve should be at
least 10. Percivals criterion is useful for esophoric patients
21
Heterophoria
22
How do I investigate?
20
T H O
20
T H O
20
T H O
15
L O A
15
L O A
15
L O A
10
A H T
10
A H T
10
A H T
H A O E T L
H A O E T L
H A O E T L
O D E C A H
O D E C A H
O D E C A H
(a)
(c)
(b)
20
T H O
20
T H O
15
L O A
15
L O A
10
A H T
10
A H T
H A O E T L
H A O E T L
O D E C A H
O D E C A H
(d)
(e)
23
Heterophoria
24
Table 2.4
test
How do I investigate?
Table 2.5 Scoring system for diagnosing decompensated
heterophoria and binocular instability. This scoring algorithm is
most appropriate for horizontal heterophoria: for vertical
heterophoria, if aligning prism of 0.5 or more is detected then,
after checking trial frame alignment, measure the vertical dissociated phoria. If this is more than the aligning prism and there are
symptoms then diagnose decompensated heterophoria; but still
complete the worksheet for any horizontal phoria that may be
present
DISTANCE/NEAR
(delete)
score
If no, score +1
>5 treat,
(continued)
25
Heterophoria
Table 2.5
26
Continued
DISTANCE/NEAR
(delete)
score
7. Sheards criterion:
(a) measure the dissociated phoria (e.g., Maddox wing, prism
cover test); record size & stability
(b) measure the fusional reserve opposing the heterophoria
(i.e., convergent, or base out, in exophoria). Record as
blur/break/recovery in .
Is the blur point, or if no blur point the break point, [in (b)] at
least twice the phoria [in (a)]?
Yes or No
If no, score +2
8. Percivals criterion: measure the other fusional reserve and
compare the two break points.
Is the smaller break point more than half the larger break point?
Yes or No
If no, score +1
9.When you measured the dissociated heterophoria, was the
result stable, or unstable (varying over a range of 2 or more)?
(e.g., during Maddox wing test, if the Hz phoria was 4 XOP and
the arrow was moving from 2 to 6, then result unstable)
Stable or Unstable
If unstable, score +1
10. Using the fusional reserve measurements, add the divergent
break point to the convergent break point. Is the total
(=fusional amplitude) at least 20?
Yes or No
If no, score +1
Add up total score (from both sections of table) and enter in right hand
column. If total score: <6 then diagnose compensated heterophoria, if >5
diagnose decompensated heterophoria or binocular instability (see pp.
3740).
Reproduced with permission from Evans, B.J.W. (2002) Pickwells Binocular Vision
Anomalies, 4th edition, Butterworth-Heinemann.
What do I do?
The general advice on pp. 1011 about managing binocular
vision anomalies apply: action is only required if the binocular
vision anomalies are causing problems, are likely to deteriorate if
left untreated, or if they are a sign of pathology. It is very rare for
decompensated heterophoria to be a sign of ocular pathology, but
a marked change in the magnitude of a heterophoria should
arouse suspicion.This is one reason why it is useful to always
record an estimate of the size of the heterophoria, for example
based on cover testing (see Table 1.3).
The investigation of heterophoria is mostly about determining
whether it is compensated or not.This was discussed in the
previous section.
What do I do?
Decompensated heterophoria is fairly commonly encountered in
primary care optometric practice and nearly all cases can be
managed in the primary care setting.The main treatment options
are listed below:
1.
2.
3.
4.
5.
27
Heterophoria
28
2. Eye exercises
Eye exercises are most likely to be effective in small or moderate
exophoria. It is harder to achieve success with exercises for
esophoria and much harder (arguably impossible) for
hyperphoria. Exercises can work in patients of any age, but the
patient needs to be old enough to understand what is required
and in older people the exercises tend to take longer to work.
Without doubt, the most important factor is patient motivation: if
the patient lacks motivation and enthusiasm then exercises are
very unlikely to be effective.
The purpose of eye exercises for decompensated heterophoria
is to increase the fusional reserve that opposes the heterophoria;
for example, to increase the convergent fusional reserves in
exophoria. Exophoric conditions are treated most often in this
way and will be described here. Similar principles apply to the
training of divergent fusional reserves in esophoria.
Controlled trials show that convergent fusional reserves can
be trained with exercises, and a key component is probably the
mental effort to keep the eyes over-converged during the
exercises. Exercises may use a haploscopic device (e.g., a form of
stereoscope) or free-space techniques. Free-space techniques are
probably the most common form of fusional reserve exercises
these days, and can be further sub-divided into two types:
What do I do?
29
Heterophoria
Table 2.6
30
Table 2.7
Goal
Design feature
Affordable
Easy to understand
Comprehensive instructions
Fun to do
Motivating
Checks on progress
10 self-test questions
Variety of stimuli
Control/treat suppression
What do I do?
31
Heterophoria
32
child identifies the letter correctly then the parent knows that
they are doing the exercises properly.These exercises have proved
popular with both optometrists and orthoptists.
It should be stressed that there are literally dozens of different
types of fusional reserve exercises and only a brief introduction
has been given here.The IFS exercises are suitable for use by the
patient at home.This is a popular approach with many
practitioners, although some practitioners prefer treatment in the
practice.The more complex cases may require a battery of
exercises rather than just one type. A key factor is the ability of
the patient and (if a child) their parent to understand the
instructions and the practitioner must check that these are fully
understood before the patient leaves the practice. It is generally
advisable to give written instructions, like those that are included
with the IFS exercises.
Most practitioners would agree that it is much better to have
a brief period of aggressive exercises (e.g., 23 times a day for
1015 minutes a time for 3 weeks) rather than a half-hearted
attempt to do the exercises over several months. Ideally a
follow-up appointment should be booked about 3 weeks after the
exercises are issued. Another advantage of this approach is that, if
the exercises are not working, this fact is identified rapidly so that
another management option (or referral) can be pursued.
3. Refractive modification
In some cases an uncorrected refractive error is the cause for
the decompensated heterophoria. For example, there may be
uncorrected hypermetropia in esophoria, uncorrected myopia in
exophoria, or astigmatism impairing sensory fusion. In these
cases, the refractive correction is the only proper treatment,
since this will remove the cause of decompensation.
In other cases there may be no significant refractive error, or a
refractive error that is already being adequately corrected, and
yet a refractive modification might be an appropriate method
of correcting the decompensated heterophoria. A common use of
this approach is in patients with convergence excess esophoria:
an esophoria that is worse at near than at distance. Bifocal or
What do I do?
varifocal spectacles would be likely to render the heterophoria
compensated.
Many decompensated exophorias are also very amenable to
treatment in this way. A negative add can be used (e.g., giving
minus lenses to an emmetrope) to induce accommodative
convergence which helps the patient to overcome an exophoria.
It is important to explain to the patient and the parent that these
are exercise glasses and the goal is to reduce the add over
time as the patient gradually becomes able, by increasing degrees,
to compensate for the deviation.This very simple method of
treatment is often highly effective and is sadly under-used.The
method is summarized in Table 2.8.
The patients who are most amenable to treatment by
refractive modification are those with a high AC/A ratio.The
AC/A ratio defines how much the vergence changes per diopter
of accommodation. For example, during a Maddox Wing test a
patient might have an esophoria of 8. If +2.00DS lenses are
added, the esophoria reduces to 2.The vergence has changed by
6 (82) for 2D of relaxation in accommodation, so the AC/A
ratio is 3/D (6/2). A value of 34/D is normal.
4. Prismatic correction
Prismatic correction is quite commonly used to correct a
decompensated heterophoria in older patients, typically as base in
prism in reading glasses. Small vertical heterophoria, although
rare, also responds well to prisms. In other cases prisms may be
used as a temporary correction, for example in a child who is
taking examinations and who will return for exercises in the
school holidays.The method is summarized in Table 2.9.
5. Surgery
Surgery is only very rarely required for decompensated
heterophoria.Typically, these are the cases where the
heterophoria is very large and is causing considerable symptoms,
yet is too large to treat with the other methods described above.
These cases will require referral to a surgeon.
33
Heterophoria
34
35
Heterophoria
36
Convergence insufficiency
Convergence insufficiency is characterized by a remote near
point of convergence: usually it is more remote than 810 cm.
This condition is often (but not always) associated with
convergence weakness exophoria. Convergence insufficiency is
very amenable to treatment with eye exercises, and examples, in
increasing order of complexity, are listed in Table 2.10.Table 2.10
stresses the need to keep the object single, but usually the
patient is asked to also keep the object clear.This is especially
important where the patient has a combined convergence and
accommodative insufficiency (see Chapter 8). In older patients,
where the near point of accommodation may naturally be further
out than the near point of convergence, then the instructions
should stress single vision (as in Table 2.10), not clear vision, and
non-accommodative targets will be more appropriate.
37
Heterophoria
38
Details of exercise
Simple push-up
Push-up with
physiological
diplopia
Distance to near
1. The patient views a detailed near target and
(jump convergence)
brings it towards their nose, trying to keep it
single for as long as possible.The distance
when doubling occurs is the near point of
convergence
2. This near target is then held just beyond
this point
3. The patient then looks at a distant target and
makes this single
4. Then the patient looks back at the near
target, making this single
5. Steps 3 and 4 are repeated, every now and
then checking that the near target is close to
the near point of convergence
Distance to near
1. As above, but the patient holds an additional
(jump convergence)
target a few centimeters beyond the near
with physiological
target
diplopia
2. This target is seen in physiological diplopia
and the patient is made aware of this
Binocular instability
The eyeballs are visco-elastic spheres surrounded by relatively
soft orbital fat and connective tissue. Considering this, and the
fact that the eyes move at incredibly high speeds, it is amazing
that they usually keep as well co-ordinated as they do. It is not
surprising that small vergence errors occur, and it was noted
above that Panums fusional areas allow some free play in the
sensory visual system. Similarly, the fusional reserves allow some
tolerance in the motor system.This is why orthophoric patients
still need to possess fusional reserves. A corollary of this is that
39
Heterophoria
40
Strabismus
What is strabismus?
42
Sensory adaptations to strabismus
42
How do I investigate?
44
Motor
44
Sensory
44
When do I need to do something?
47
If the strabismus is causing symptoms or decreased
visual function
48
If the situation is likely to worsen if left
untreated
50
If the strabismus is likely to be a sign of ophthalmic or
systemic pathology
50
What do I do?
53
New or changed strabismus
53
Long-standing strabismus
55
Strabismus
42
What is strabismus?
Strabismus occurs when the visual axes are misaligned: the two
eyes do not point directly at the object of regard. Panums fusional
area allows some free play in the visual system: the visual axes
can be slightly misaligned (by a few minutes of arc; fixation disparity)
without the patient losing normal binocular vision. Strabismus
usually signifies a deviation that is an order of magnitude greater
than this: typically, the visual axes are misaligned by several
degrees, rather than minutes of arc. An exception to this is
microtropia, when the visual axes are misaligned by less than 10;
this is discussed in Chapter 4.When the visual axes are
misaligned by an amount that is too great to allow sensory fusion
within Panums fusional areas then a strabismus is present. Small
angle strabismus may not be recognizable by looking at the
patient and it is these cases that are particularly challenging for
the eyecare professional to determine.
What is strabismus?
different since the whole binocular field of the strabismic eye will
need to be suppressed. For this reason, the suppression in
strabismus has been called global suppression.
Rather confusingly, patients who, instead of global suppression
have HARC, often have two small suppression areas in their
strabismic eyes visual field: one at the fovea of their strabismic eye
and one at the zero point.The zero point is the part of the
strabismic eyes visual field that neo-corresponds with the fovea of
the non-strabismic eye.The reason that these two small
suppression areas are necessary is because it is at the two foveae
that Panums fusional areas are very small, and this makes
anomalous correspondence at these two positions very difficult. It
should be stressed that these two small suppression areas occur in
the presence of HARC, and are quite different to the global
suppression which occurs as the alternative diplopia avoidance
mechanism to HARC. For this reason, these small suppression
areas have been called local suppression areas.These local
suppression areas in HARC have been said to subtend 12,
although it is sometimes argued that they are in some cases larger
and can coalesce into one suppression area, with HARC existing in
the periphery.The debate over this is probably fuelled by the fact
that the dimensions of these areas may not be fixed, but may vary
with different test conditions.
The factors that predispose a patient to develop either HARC
or global suppression are summarized in Table 3.1. It should be
Table 3.1 Factors which influence the likelihood of a child with
strabismus developing either HARC or global suppression. Note:
exceptions to these tendencies occur in some cases
Factor
More likely to
develop HARC
Size of deviation
Small
Large
Stability of deviation
Constant
Variable
Type of deviation
Esotropia
Exotropia
Age of onset
Young child
Older child
43
Strabismus
44
noted that the table only represents tendencies: there are many
exceptions who have not followed the predispositions in Table 3.1.
A useful mnemonic is that the factors favoring HARC can be
remembered as the five Ss: small, stable, eso, started soon.
How do I investigate?
As in any binocular vision anomalies, it is important to be sure of
the nature of the condition. For example, regular checks on
ocular health and comitancy should take place. Indeed, all the
factors in Table 3.3 need to be regularly reviewed.
Motor
It is important to know the size of the deviation, since any
change in the angle must be detectable. But it must be borne
in mind that this is likely to increase with increasing degrees
of dissociation.The standard method is the cover test (see
Figure 1.2 and Table 1.1), recording the size of deviation
(Table 1.3) on the initial covering and then the degree to which
this builds with repeat covering (Table 1.5).
Other dissociation tests can also be used to obtain an
estimate of the degree of deviation, such as a Maddox wing or
Maddox rod. If deep sensory adaptations are present then these
can interfere with the measurement, and some alternating
covering may be necessary to break down the adaptation, but this
will be likely to increase the angle.
Sensory
HARC is an acquired adaptation and it must be remembered that
normal retinal correspondence is still the innate correspondence
which will be present underneath the adaptation. If the patient
is presented with artificial test situations then the adaptation can
be broken down and the patient may revert to normal retinal
correspondence. Of course, they will still have the strabismus so
if they revert to normal retinal correspondence then they will
How do I investigate?
have diplopia at this point in the test.The goal of clinical tests is
to determine the sensory adaptations that operate during the
patients normal everyday visual activities.To do this, the clinical
tests need to replicate these everyday conditions as accurately as
possible: they should be naturalistic.This is why artificial,
dissociating, instruments such as a synoptophore are
inappropriate for determining sensory status.The two best tests
are the Bagolini Lens Test and the Mallett Modified OXO Test
(Figure 3.1). Nearly all primary eyecare practices have a Mallett
unit, so this technique will be described here.
The small OXO tests, which are designed to detect fixation
disparity in the presence of heterophoria, should not be used to
test for HARC or global suppression.This is because of the small
local suppression areas that can occur in HARC (see p. 43) and
which can cause the Nonius strip with the small OXO tests to
disappear which could cause HARC to be mis-diagnosed as global
Figure 3.1 The Mallett Near Vision Unit.The Modified OXO Test for
testing for HARC or global suppression is the top left large OXO test
(courtesy of i.O.O. Sales Ltd.)
45
Strabismus
46
HARC:
SUPPR:
OXO
OXO
OXO
NRC:
OXO
OXO
UARC:
OXO
OXO
Figure 3.2 Use of the Mallett Modified OXO Test to detect HARC or
global suppression in strabismus (reproduced with permission from
Optician, 11 June, 1999, No. 5705,Vol. 217)
47
Strabismus
48
CLINICAL FINDINGS:
ocular motility
Refractive error:
R=L=+2.50DS
Cycloplegic:
R=L=+3.25DS
Cover test:
D=N=12 LSOT
HARC
Stereopsis (Randot):
49
Strabismus
50
Rationale
What to do
Detect
incomitancy
Any new or
changing
incomitancy
requires prompt
referral to a
hospital
ophthalmological
unit
Orbital pathology
can cause
strabismus, although
this is rare
Pathology that
destroys or
diminishes the
vision in a
significant part of
the visual field of
one eye can
dissociate the eyes
and cause
strabismus
Look for
orbital
pathology
Detect any
ocular
pathology
Look for
neurological
problems
Pathology in the
brain can cause
comitant as well as
incomitant
deviations
51
Strabismus
Table 3.3
52
Continued
Step
Rationale
What to do
Look for
obvious
causes of the
strabismus
There will be a
reason why a
patient develops a
strabismus. If you
find a nonpathological reason,
then the likelihood
of there being a
pathological reason
is greatly reduced
Monitor the
size of the
deviation
If the deviation is
increasing then
there must be a
reason
Is the
strabismus
responding
to
treatment?
What do I do?
binocular status will identify the cause. Less commonly ocular
pathology will be present and might be detected with the
ophthalmoscope. If an obvious cause of the strabismus is found
(e.g., hypermetropia in accommodative esotropia), then this is
treated. In these cases, the patient only requires further
investigation if the findings are suspicious, or if the situation
worsens or does not respond to treatment (see Table 3.3). It is
important to monitor the general health of the patient. If this is
poor or there are other signs that are suggestive of neurological
problems (e.g., headaches, tremor, unusual gait) then this raises
the index of suspicion and referral is indicated.
What do I do?
Intermittent strabismus can be thought of as one step worse than
decompensated heterophoria, and the same considerations apply as
for treating decompensated heterophoria (pp. 2735). Since the
strabismus is intermittent, the patient is likely to have normal retinal
correspondence and binocular single vision when the strabismus is
not present. So, if the deviation is treated by any of the methods on
pp. 2735 then sensory factors are unlikely to cause problems.
If children with strabismus are seen within the sensitive period
(until the age of 712 years) then treatment of any amblyopia is a
priority.This is discussed in Chapter 5.
53
Strabismus
54
What do I do?
Long-standing strabismus
Approximately 24% of the population have a strabismus, so
primary eyecare practitioners will often encounter patients
attending for routine eye examinations who have a long-standing
strabismus.Typically these cases will be adults: if previously
untreated strabismic amblyopia is detected in children who are at
the upper age range for patching (712 years) then urgent
treatment is required by a practitioner who is able to monitor
treatment carefully to ensure that any binocular sensory
adaptation is not adversely affected by patching (Chapter 5).
In most cases of adults with long-standing strabismus, the
patient will report a history of a long-standing deviation that is
not changing and they will have no symptoms relating to their
strabismus.The usual advice for such cases is that if the
strabismus is not causing any problems then it does not require
any treatment. Indeed, if there is any pre-existing prismatic
correction, spectacle lens decentration, or partial correction of
anisometropia then it is best to mimic this in any new refractive
correction.
Comitant (or indeed incomitant) strabismus can decompensate
and worsen at any time in life.This is one reason why it is useful
to measure the angle in these cases so that any change can be
detected in the future, although this is usually obvious from the
patients symptoms. A strabismus that is changing will require
referral to find the reason why it is changing (see Table 3.3).
This may be idiopathic, but should still be investigated and may
require surgery. One possible reason why a long-standing
unilateral strabismus with sensory adaptation might break down
is if the patients sensory status is changed.This might occur, for
example, if the patient is prescribed monovision contact lenses or
monovision refractive surgery. Monovision is therefore
contraindicated in these cases.
Occasionally, patients will be encountered who have a stable
long-standing strabismus that is cosmetically noticeable where the
patient is now concerned by the cosmesis, which they previously
found tolerable. If the deviation is large enough to be cosmetically
distressing then few of these cases will be likely to be treatable in
55
Strabismus
56
Microtropia
What is microtropia?
58
How do I investigate?
59
When do I need to do something?
What do I do?
61
61
Microtropia
58
What is microtropia?
Microtropia is defined in different ways by various authors.To
many, microtropia simply means a small angle strabismus: often
defined as less than 10 or sometimes as less than 6. Many
different factors are typically associated with microtropia, and
these are listed in Table 4.1. Some authors define microtropia
according to some of these associated factors.An example of this
is microtropia with identity.This is a microtropia when the angle
of eccentric fixation is the same as the angle of the strabismus.
This means that no strabismic movement (see Table 1.1) is seen
on cover testing: indeed, a movement may be seen which looks
Table 4.1 Algorithm for the diagnosis of microtropia. Reproduced
with permission from Evans, 2002, Pickwells Binocular Vision
Anomalies, 4th edition, Butterworth-Heinemann
All the following characteristics must be present for a diagnosis of
microtropia
Angle less than 10
Amblyopic eye with morphoscopic acuity at least one line worse
than non-amblyoptic eye, unless alternating microtropia (rare)
Eccentric fixation, unless alternating microtropia (rare)
HARC detected by Bagolini striated lens test, or by Modified
Mallett OXO Test
And at least three of the following characteristics should also be present
How do I investigate?
like heterophoria. Some authors only use the term microtropia to
describe these cases of microtropia with identity. Other authors
say that microtropia just means a small angle strabismus, and to
these authors microtropia with identity is just one particular type
(subdivision) of microtropia.
An attempt has been made to bring these different viewpoints
together in Table 4.1.The features that are almost universally
accepted as present in every case of microtropia are listed in the
first part of the table.The second part of the table lists features
which are commonly present in microtropia. In Table 4.1 these
factors are given a hierarchy to create a diagnostic algorithm for
microtropia.
How do I investigate?
Table 4.1 summarizes the investigation of microtropia. Most cases
of microtropia will have amblyopia and this should be investigated
as detailed in Chapter 5.The detection of HARC is summarized
in Chapter 3.
The cases that are hardest to investigate are microtropia with
identity, when no sign of the strabismus will be seen on cover
testing.These cases may present as unexplained poor acuity in
one eye.There may be anisometropia, in which case the differential
diagnosis is between pure anisometropic amblyopia and, if a
microtropia is present, mixed strabismic and anisometropic
amblyopia. It can be important to make this differential diagnosis in
patients who are keen to pursue treatment of their amblyopia.This
is because patching can be tried in adults with pure anisometropic
amblyopia but should not be attempted in adults with strabismic
amblyopia (see Chapter 5). In these cases, many of the tests in
Table 4.1 will help with the differential diagnosis, but the most
useful tests are to look for eccentric fixation with the
ophthalmoscope (see Table 5.4) and to test for central suppression.
Unfortunately, the standard test for suppression in microtropia, the
4 base out test, does not always give conclusive results.This is
probably because too little attention is paid to the fixation target.
The assumption behind the test is that a 4 base out prism in front
59
Microtropia
60
What do I do?
Results with the 4 base out test can be inconclusive, and it is
best not to base the diagnosis of microtropia on this test alone.
The test is meant to detect central suppression and the foveal
suppression test described in Table 2.4 may be a better test. In
any event, the best method of diagnosing a complex condition like
microtropia is to combine the results of several tests, as
suggested in Table 4.1.
What do I do?
As mentioned above, most cases of microtropia do not require
treatment. Unless the patient has symptoms, it is best to do as
little as possible to interfere with the patients current status. If
there is a prism or decentration in the glasses, then it is best to
replicate this in any new glasses. If any anisometropia is only
partly corrected, then do not make large changes to the degree
to which this is corrected.
If the patient is desperate to improve their binocular status,
then a few practitioners with particular experience in this field
might consider treatment (see pp. 5556).Treatment of these
cases is not without risk (principally, of diplopia) and should only
be undertaken by experienced practitioners who can monitor the
patient appropriately.
61
Microtropia
62
Amblyopia
What is amblyopia?
64
How do I investigate?
65
Visual acuity assessment in pre-school children
65
Differentially diagnosing amblyopia and reduced vision
from pathology
68
Differentially diagnosing strabismic and anisometropic
amblyopia
68
When do I need to do something?
68
Strabismic amblyopia
71
Anisometropic amblyopia
73
What do I do?
73
Strabismic amblyopia
74
Anisometropic amblyopia
76
Amblyopia
64
What is amblyopia?
Amblyopia has been defined as a visual loss resulting from an
impediment or disturbance to the normal development of vision.
It usually exists when an early interruption to the development of
vision causes a visual deficit which, in later life, cannot be
corrected refractively.
There is a sensitive period during which the visual system is
still capable of developing amblyopia. For humans, this is said to
extend until the age of about 78 years. During this period, any
interruption to binocularity or to a clear image in one or both
eyes is likely to cause amblyopia.There is no abrupt ending to the
sensitive period: in reality, disruption to the visual system
becomes progressively less likely to cause amblyopia as the child
ages and the likelihood of this happening is close to zero by
about the age of 8 years.
Amblyopia can be broadly classified into organic and functional.
Organic amblyopia results from some pathological abnormality,
such as retinal eye disease or toxic factors. Organic amblyopia is
rare and practitioners are much more likely to encounter
functional amblyopia.The classification of functional amblyopia is
summarized in Figure 5.1. Stimulus deprivation amblyopia (e.g.,
from congenital cataract) and strabismic amblyopia used to
collectively be called amblyopia ex anopsia, but this term is
seldom used nowadays.The two most common forms of
Functional
amblyopia
Stimulus
deprivation
Strabismic
Anisometropic
Isometropic
(refractive)
e.g.
Meridional amblyopia
Figure 5.1
Psychogenic
(hysterical)
How do I investigate?
amblyopia are strabismic and anisometropic, and these two types
have rather different characteristics. Strabismic and anisometropic
amblyopia often co-exist, when the characteristics of the
amblyopia are best considered as strabismic amblyopia.
Isometropic amblyopia occurs when both eyes have an
amblyogenic refractive error (e.g., high astigmatism or high
hypermetropia). Hysterical amblyopia is a form of visual
conversion reaction and is discussed below.
Two quantitative approaches are commonly used to diagnose
amblyopia: a difference between the best corrected acuity of the
two eyes of two lines or more and/or acuity in the amblyopic eye
of less than 6/9 (assuming that the child is old enough for the
visual acuity norms to be 6/6). Additionally, the person should
show no sign of another condition (e.g., pathology) that might
account for the reduced vision.
The prevalence of amblyopia is about 3%.The vast majority of
cases of amblyopia are either strabismic or anisometropic or
both.There is some debate over whether strabismic or
anisometropic amblyopia is more common, with some studies
suggesting that they are equally prevalent.
How do I investigate?
Visual acuity assessment in pre-school children
Amblyopia is initially detected as reduced visual acuity and, for
strabismic amblyopia, the visual acuity is much worse for crowded
than for isolated targets. Since one of the main goals of measuring
visual acuity in young children is to detect strabismic amblyopia, it
follows that crowded targets should wherever possible be used
when measuring the vision of young children. By the age of about
45 years nearly all children can match letters on a Snellen chart
with similar letters on a matching card and this type of acuity test
is the goal. As soon as letters can be recognized then a proper
Snellen test can be conducted.
For children aged 24 years, various crowded acuity charts
are available, which usually come with matching cards. A range of
65
Amblyopia
66
6/19
0.50
Figure 5.2 Lea Symbols Test, as presented in crowded format with the
computerized Test Chart 2000 (reproduced with permission from
Thomson Software Solutions)
such tests are available with the computerized Test Chart 2000
(Figure 5.2), which has the advantage of allowing optotypes to be
randomized.This is especially important for monitoring the
effects of amblyopia treatment, since it prevents children from
memorizing the test charts. One of the limitations of
conventional Snellen charts is a very limited number of optotypes
for lower acuities (e.g., only two optotypes on most 6/24 lines),
and this greatly increases the risk of children memorizing a
certain line. It may be necessary to periodically check the vision
in a different consulting room to guard against this.
The Lea Symbols Test (Figure 5.2) can be made into a matching
or naming game and can be used to obtain binocular acuities
with most children from about the age of 2 years. Of course,
monocular testing is important for detecting amblyopia, but this
can be problematic for some children around the age of 23 years
who object to occlusion. In some cases, occlusion for visual acuity
testing is just not possible and the best that can be done is to see
if the child is equally unhappy to have either eye occluded, which
suggests approximately equal acuity.The child should be tested
frequently (e.g., every 34 months) until monocular visual acuities
can be recorded, which is nearly always possible in the third year of
life. If a child refuses to have one eye covered, but quite happily
tolerates occlusion of the other eye, then this is a highly suspicious
sign and might suggest poor vision in the eye that is uncovered
when they object most. If good results are not obtained with such
a child, then a cycloplegic or very early retest is indicated. If there
is any doubt about such cases then they should be referred.
Below the age of about 2 years, preferential looking tests may
be required, such as the Cardiff acuity test.This works well for
How do I investigate?
children aged 12 years, but is not good at detecting strabismic
amblyopia.This is why crowded tests with pictures or letters
should be used as soon as possible (e.g., Figure 5.2).
Typical tests for different ages with typical results are given in
Table 5.1. As one might expect, different tests produce results
that are not perfectly equivalent, so the figures in Table 5.1 are an
approximation.To give maximum clinical validity, it is important to
test patients with standard letter charts as soon as they can cope
with these. In vision screening programs it is important to have a
strict criterion and every child who achieves below this level
would be referred. In a primary care eye examination the
situation is rather different and so the values in Table 5.1 are only
for guidance, since other factors will need to be taken into
consideration. For example, a significant refractive error,
symptoms or reduced vision in solely one eye will raise the index
of suspicion.
Test
Approximate norms
01 year
13 years
35 years
Over 5 years
3 months: 6/90
6 months: 6/30
12 months: 6/24
12 months: 6/38
24 months: 6/15
36 months: 6/12
36 months: 6/12
48 months: 6/9
60 months: 6/7.5
Snellen
6/7.5
67
Amblyopia
68
What to do
Is the amblyopia
responding to
treatment?
69
Amblyopia
70
Rationale
What to do
Detect
strabismus
Isometropic
amblyopia is rarer, but
can occur in high
bilateral uncorrected
refractive errors
Look for
other
refractive
errors
Foveal reflex
Fixation star
1.5 dd
Fixation slightly unstable compared with dominant eye
Figure 5.3 Suggested method of recording eccentric fixation. It is
important to label the foveal reflex and the fixation target.The degree of
eccentric fixation can be recorded, using a disc diameter (dd) or drawing
the ophthalmoscope graticule (if present) as the unit of measurement. If
the ophthalmoscope graticule is used then record the make and model
of ophthalmoscope.There may be a slight vertical element to the
eccentric fixation
Strabismic amblyopia
The sensitive period for the development of amblyopia was
discussed above. For strabismic amblyopia, there is also a sensitive
period for the treatment of amblyopia. If strabismic amblyopia is
treated before the age of about 712 years then treatment is
much more likely to be effective than after this age. Over the age
of 712 years, there is another reason why treatment is contraindicated in addition to the decreased likelihood of success.This
is because of the binocular sensory adaptations to strabismus
(HARC or global suppression; Chapter 3), which are very likely
to be present in cases of strabismus which develop before
712 years of age. Although these binocular sensory adaptations
are distinctly different from amblyopia, the binocular sensory
adaptations might nonetheless be broken down by prolonged
periods of treatment.This could cause diplopia. For these two
reasons (low likelihood of success and risk of diplopia), it is
usually recommended that strabismic amblyopia should not be
treated over the age of 712 years, and should only be treated in
this age range with caution (p. 77).Therefore, the period up to
71
Amblyopia
72
Anisometropic amblyopia
The treatment of anisometropic amblyopia is an interesting
example of the situation where the scientific literature tells a
rather different story to the clinical received wisdom. It is often
stated, sometimes even in textbooks, that anisometropic
amblyopia must, like strabismic amblyopia, be treated before age
712 years. However, the scientific evidence suggests that there is
no such sensitive period for anisometropic amblyopia. For cases
of pure anisometropic amblyopia (i.e., when there is no strabismus)
treatment can be attempted at any age. Most studies have failed
to find a significant effect of age on treatment: treatment is almost
as effective in adults as it is in younger children. It seems implausible
that there is no effect of age at all, but the effect is certainly weak.
What do I do?
As noted above, strabismic amblyopia and anisometropic amblyopia
need to be considered differently. If a patient has anisometropia
and strabismus (common in microtropia) then the patient should
be treated as a strabismic amblyope.
The treatment of amblyopia, particularly patching, is not without
disadvantages.Whilst the patch is worn the child will be forced to
live with reduced vision, and the psycho-social consequences of
patching (e.g., disruption to the childs quality of life) are a cause
for concern. As always, the decision to treat should only be made
after due discussion with the parent and (if old enough) the child.
In a given case, the disadvantages of patching need to be weighed
against the chances of success, which will depend on several factors
(e.g., age, degree of any anisometropia, etc.). But although patching
is never popular, the risk of the person losing sight in their good
eye in later life (e.g., from injury or AMD) cannot be ignored and
is a powerful reason to treat.
73
Amblyopia
74
Strabismic amblyopia
In strabismic amblyopia the strabismus may need treatment, as
outlined in Chapter 3.The present section just deals with
amblyopia, and the management of this will differ for different age
groups, as detailed below.The most important advice when seeing
a patient with strabismic amblyopia who is within the sensitive
period is to do something and to do this decisively. Either the
patient should be treated, or referred to someone else to treat. If
they are treated, the parents/child should be given clear
instructions and monitored frequently.
What do I do?
Table 5.5 Method for treating strabismic amblyopia in children
under the age of 3 years
1. For these young children there is a significant risk of causing
occlusion amblyopia: amblyopia of the occluded eye.To prevent
this, the usual advice is to occlude the good eye for one day per
year of age, then patch the other eye for one day; and then repeat
the cycle. For example, in a 2-year-old the good eye would be
patched for 2 days, then the amblyopic eye for one day, and so on
2. Amblyopia treatment can only be fully effective if there is a clear
image. Appropriate refractive correction for the amblyopic eye is
essential
3. Total occlusion is generally recommended, usually with an
adhesive patch
4. Full time occlusion is also usually recommended, but with the
patch removed for any tasks when normal vision is needed for
safety (e.g., riding a bike in older children)
5. Explain to the parent how the child might cheat (e.g., lifting
edge of patch and turning head). Children cannot be blamed for
trying to use their better eye, but patching will only work if the
parent detects and stops any cheating
6. The patient should be encouraged to do tasks that involve
detailed vision. For example, playing with small toys or watching a
favorite television program or video.To begin with, encourage the
parents to give the child a treat when the patch is put on
7. Careful monitoring is required, typically 3 weeks after treatment
starts and regularly thereafter. In addition to monitoring acuities
in the amblyopic and non-strabismic eye, the parents
observations should also be recorded and cover test, motility and
periodically ophthalmoscopy and retinoscopy repeated
8. If the acuity does not improve significantly (e.g., by a line on
the LogMAR Crowded Test) then re-evaluate the diagnosis (see
Table 5.2)
9. Occlusion is continued until there has been no further
improvement over 56 weeks
10. The patient is then monitored every few months to ensure
that the acuity in the strabismic eye does not drop back. If so,
re-instigate treatment
75
Amblyopia
76
Anisometropic amblyopia
A typical approach to treating anisometropic amblyopia is
outlined in Table 5.6. Anisometropic amblyopia is a refractive
problem, so accurate refractive correction is at the core of the
What do I do?
Table 5.6 Method for treating anisometropic (non-strabismic)
amblyopia
1. Prescribe refractive correction, fully correcting the degree of
anisometropia (usually revealed by cycloplegic refraction)
2. Check the patient after about 46 weeks. If there has been a
significant (typically, one line or more) improvement, then
continue with refractive correction alone until the amblyopic eye
either (a) stops improving or (b) the acuity of the amblyopic eye
equals that of the better eye
3.
4.
77
Amblyopia
78
10. Patients should be warned to look out for diplopia and parents
to look out for a turning eye when they remove the patch. If this
occurs, then they should stop patching and return for their
binocularity to be checked to ensure that any heterophoria is not
decompensating (see Chapter 2)
11. Careful monitoring is required, typically 46 weeks after
treatment starts and regularly thereafter. In addition to
monitoring acuities in both eyes, the parents observations should
also be recorded and cover test, motility, stereopsis, and
periodically ophthalmoscopy and retinoscopy repeated
12. If the acuity does not improve significantly (e.g., by a line on
the LogMAR Crowded Test) then re-evaluate the diagnosis
(see Table 5.2)
13. Occlusion is continued until there has been no further
improvement over 56 weeks
14. The patient is then monitored every few months to ensure that
the acuity in the strabismic eye does not drop back. In
anisometropic amblyopia this is unlikely
What do I do?
been partly corrected. If these patients are asymptomatic and
happy to carry on as they are then this may be the best course of
action. But it is possible that any amblyopia might reduce with
appropriate refractive correction, possibly with additional
patching. If the anisometropia is high (over 34D), then
appropriate refractive correction is likely to mean contact lenses
or refractive surgery. Indeed, through reducing aniseikonia,
contact lenses or refractive surgery are likely to be the best
modes of correction for all cases of anisometropia, and therefore
will increase the chances of the vision improving without the
patient requiring occlusion. In pure (non-strabismic) cases of
anisometropic amblyopia, occlusion can be attempted in adults,
but this is usually only carried out for brief periods (e.g., 30
minutes) of detailed use of the amblyopic eye. Patients should be
monitored closely and warned as noted in point 10 of Table 5.6.
79
Incomitant
deviations
82
92
Incomitant deviations
82
Vascular
Neurological
Other
Diabetes
Tumor
Trauma
Vascular hypertension
Multiple sclerosis
Stroke
Migraine
Toxic
Aneurysm
Myasthenia gravis
Iatrogenic
Idiopathic
R
C
L
R
M
Abduction Adduction
Abduction Adduction
(a)
(b)
Figure 6.1 Plan view of right orbit. (a) The plane and direction of pull of
the superior and inferior recti muscles, RA, which passes medial (M) to
the plane of the center of rotation of the eye (C). (b) The plane
containing the superior and inferior oblique muscles; their direction of
pull is almost the same. It passes behind and medial to the center of
rotation (reproduced with permission from Evans, B.J.W. (2002) Pickwells
Binocular Vision Anomalies, 4th edition, Butterworth-Heinemann)
83
Incomitant deviations
84
Table 6.2
position
Muscle
Primary
action
Secondary
action
Tertiary
action
Medial rectus
Adduction
None
None
Lateral rectus
Abduction
None
None
Superior rectus
Elevation
Intorsion
Adduction
Inferior rectus
Depression
Extorsion
Adduction
Superior oblique
Intorsion
Depression
Abduction
Inferior oblique
Extorsion
Elevation
Abduction
How do I investigate?
The ocular motility test is the most important part of the eye
examination for detecting incomitant deviations. It is essential to
How do I investigate?
RSR
LIO
RIO
LSR
85
RLR
LMR
RMR
LLR
RIR
LSO
RSO
LIR
Figure 6.2 The six cardinal diagnostic positions of gaze, indicating the
muscles which should have maximum power to maintain the eyes in these
directions.The paired synergists (muscles, one from each eye, which act
together) are shown (reproduced with permission from Evans, B.J.W. (2002)
Pickwells Binocular Vision Anomalies, 4th edition, Butterworth-Heinemann)
carry out this test on every new patient, every time children are
seen, if adults present with new symptoms, and periodically over
the years as adults are monitored.There are really three different
tests that can be carried out during the ocular motility test, and
with incomitant patients the situation can be made simpler by
performing the test three times to gather these three sets of
information.The first of these three tests, the basic objective
ocular motility test, is described in Table 6.3.
Table 6.3 Procedure for carrying out the basic objective ocular
motility test
1. The best target is a point light source, which should be bright
enough to allow the corneal reflexes to be clearly seen, but not
so bright as to cause blepharospasm. For infants, any target that
will catch their attention is recommended
2. The light should be held at about 50 cm from the patient. It should
be moved quite slowly, so that it takes about 5 seconds to move
from extreme gaze on one side to extreme gaze on the other
(continued)
Incomitant deviations
Table 6.3
86
Continued
How do I investigate?
p. 10, this is an inaccurate method of assessing ocular alignment.
The cover test is more accurate and can be carried out during
ocular motility testing, as outlined in Table 6.4. Cover testing in
peripheral gaze is an extremely useful test and, like the basic ocular
motility test, provides objective data. It requires practice, but is well
worth the time that it takes to become skilled at this test.
Table 6.4 Procedure for cover testing in peripheral gaze during
the ocular motility test
1. A point light source is usually used, although in deviations with a
significant accommodative element an accommodative target is
better
2. An advantage of using a light is that binocular fixation can be
monitored (see point 10 in Table 6.3). It is pointless to carry out a
cover test if one eyes view of the target is already obscured by
the nose
3. A quick cover/uncover and alternating cover test (see Tables 1.1
and 1.2) is carried out in each cardinal position of gaze. Usually,
about 46 covers are required. Either the size of deviation can be
estimated (see Table 1.3) or measured with prisms
4. Care must be taken to ensure that the occluder does fully
occlude: it will need to be angled as the target is moved into
peripheral gaze
5. The result is recorded as illustrated in Figure 6.3
87
Incomitant deviations
88
How do I investigate?
Left
gaze
"4
DOFAHJ
"4
DOFAHJ
"4
DOFAHJ
$4
DOFAHJ
"4
DOFAHJ
"4
DOFAHJ
4
DOFAHJ
&4
DOFAHJ
"4
DOFAHJ
89
Right
gaze
Incomitant deviations
90
How do I investigate?
Table 6.6 Procedure for Lindbloms method of differentially
diagnosing cyclo-vertical incomitancies.The test instructions are
given on the left and the paretic muscles indicated by a given
answer are on the right
If the patient has vertical diplopia then they can view, from a
distance of 1 m, a 70 cm horizontal wooden rod (if a wooden rod
is not available, then a 50 cm or 1 m ruler will suffice)
If the patient does not have vertical diplopia, then 2 Maddox rods
can be used, placed in a trial frame with axes at 90, so that when
the patient views a spotlight at a distance of 13 m they see two
horizontal red lines
91
Incomitant deviations
92
What do I do?
The main role of the primary eyecare practitioner is to detect
new or changing cases and to refer these.The urgency of referral
What do I do?
Table 6.7 Differential diagnosis of long-standing and recentonset
incomitant deviations
Factor
Congenital or
long-standing
Diplopia
Unusual
Usually present in at
least one direction of gaze
Onset
Ambylopia
Often present
Comitance
Typically markedly
incomitant
Secondary
sequelae
Usually present
Fusion range
May be large in
vertical incomitancies
Usually normal
Abnormal
head posture
(if present)
Facial
asymmetry
May be present
Old
photographs
Normal
Other
symptoms
Unlikely
Recent onset
depends on the diagnosis, so this is discussed below. Some longstanding cases have directions of gaze in which they are
comfortably binocular, other positions of gaze in which they have
a decompensating heterophoria, and other positions in which
they are diplopic. It is sometimes possible to help these cases if
there is a deviation in the primary position, since correcting this
93
Incomitant deviations
94
Feature
Detail
Etiology
Symptoms
(if recent
onset)
Clinical
signs
Management
Additional
comments
Can be congenital
Trauma (quite commonly road traffic accident)
Other etiologies in Table 6.1
Diplopia that is predominantly vertical and which is:
possibly described as one image tipped
more marked at near (downgaze) than distance
more marked when looking down and inward
(for the affected eye)
Also may be symptoms related to the etiology of
the palsy
Head tilt and/or chin tucked down
Hyper-deviation in the affected eye which is larger:
at near than at distance
when looking down and inward (for the affected
eye)
Underaction of superior oblique on motility testing.
In long-standing cases, there can be a variety of
secondary sequelae
If recent onset: urgent referral
If long-standing: varifocals or bifocals are
contra-indicated
A superior oblique palsy can be difficult to detect
on motility because:
the main action of the muscle is torsional, which
is not assessed on motility testing
What do I do?
Table 6.9
Feature
Detail
Etiology
Symptoms
(if recent
onset)
Clinical signs
Management
Additional
comments
Can be congenital
Trauma
Vascular (e.g., high blood pressure)
Other etiologies in Table 6.1
Head turn
Diplopia that is predominantly horizontal and
which is:
more marked at distance than near
more marked when looking to the side of the
affected muscle
Also may be symptoms related to the etiology of
the palsy
Eso-deviation which is larger:
at distance than near
when looking to the side of the affected muscle
Underaction of lateral rectus on motility testing,
with overaction of contralateral synergist (medial
rectus in the other eye)
If recent onset: urgent referral
If long-standing: base out prism in distance glasses
may help
It can be difficult to differentially diagnose lateral
rectus palsy from one type of Duanes syndrome
(see Table 6.11)
95
Incomitant deviations
Table 6.10
96
Feature
Detail
Etiology
Symptoms (if
recent onset)
Clinical signs
Management
Additional
comments
What do I do?
Table 6.11
Feature
Detail
Etiology
Symptoms
Clinical signs
Head turn
On motility testing, it is as if one eye is tethered:
the eye does not move (or barely moves) beyond
the horizontal in abduction and/or adduction
there may be up-shoots: an eye aberrantly moves
upwards when trying to look to one side
usually, globe retraction is present causing the
palpebral aperture to narrow on adduction
There are three types depending on whether
abduction, adduction or both are affected.
Confusingly, there are two different classifications so
it is best to describe the features rather than
worrying about the label
Even when the adduction is normal, the near point
of convergence is often remote
Management
Additional
comments
97
Incomitant deviations
Table 6.12
98
Feature
Detail
Etiology
Symptoms
Clinical signs
Management
Nystagmus
What is nystagmus?
100
How do I investigate?
101
When do I need to do something?
What do I do?
104
102
Nystagmus
100
What is nystagmus?
Nystagmus is a regular, repetitive, involuntary movement of the
eyes whose direction, amplitude and frequency is variable. It is
rare and most commonly has an onset in the first six months of
life.The classification of nystagmus is made complicated by
various synonyms for the different types, and these are included
in parentheses in Figure 7.1.
The two most common types of nystagmus are early-onset
nystagmus and latent nystagmus. Early-onset nystagmus occurs
in the first six months of life. It may occur secondary to a
sensory visual defect (e.g., congenital cataracts, albinism), or
Nystagmus
Early onset nystagmus
(congenital nystagmus)
(infantile nystagmus syndrome)
Latent nystagmus
(fusion maldevelopment
nystagmus syndrome)
Acquired
(neurological)
nystagmus
Latent latent
nystagmus
Gaze paretic
(evoked) nystagmus
Manifest latent
nystagmus
Acquired pendular
nystagmus
Acquired jerk
nystagmus
Convergence retraction
nystagmus
Vestibular
nystagmus
See-saw
nystagmus
Figure 7.1
Classification of nystagmus
How do I investigate?
there may be no apparent cause (idiopathic), in which case it is
presumed to result from a defect in the motor control of eye
movements.
Latent nystagmus becomes much more marked when one eye
is covered. In fact, it may not be apparent at all until one eye is
covered (latent latent nystagmus); or in other cases it is always
present but worsens on occlusion (manifest latent nystagmus).
The direction of the nystagmus always reverses when the cover is
moved from one eye to the other. Latent nystagmus typically
follows an early interruption to the development of binocularity
and is most commonly associated with infantile esotropia
syndrome. Latent nystagmus therefore occurs early, usually in the
first year, of life.
Acquired (neurological) nystagmus can occur at any time,
usually after the first few months of life. It is caused by trauma
or a lesion (e.g., in multiple sclerosis) affecting the motor
pathways.
Two other eye movement anomalies will be mentioned.
Ocular flutter is a burst of horizontal saccades which can
occur in healthy infants, or can result from pathology.About 5%
of the population can simulate this effect with voluntary
nystagmus. Spasmus nutans occurs in the first year of life
and is characterized by nystagmus, head nodding and abnormal
head posture. It is generally benign, but can be associated with
pathology.
There are certain problems associated with the evaluation of
nystagmus and these are summarized in Table 7.1.The main role
for the primary eyecare clinician is to detect the condition,
diagnose which of the three main types it is (Figure 7.1), and refer
promptly if it is acquired.
How do I investigate?
There are two main aspects to the investigation of nystagmus.
First, determine the type of nystagmus and second make a note
of the characteristics so that any future change can be detected.
The key features of the main types of nystagmus are summarized
101
Nystagmus
Table 7.1
102
Early-onset
nystagmus
Latent nystagmus
Acquired
nystagmus
Presents in first
6 months of life
Oscillopsia common;
may also have
diplopia
Usually horizontal;
although small
vertical and torsional
movements may be
present. Pure vertical
or torsional
presentations are
rare
Oscillations may be
horizontal, vertical,
or torsional
depending on the
site of the lesion
Oscillations may be
disconjugate and in
different planes
Usually occurs
secondary to infantile
esotropia syndrome;
sometimes in
association with DVD
(p. 56)
Results from
pathological lesion
or trauma affecting
motor areas of brain
or motor pathways
(continued)
103
Nystagmus
Table 7.2
104
Continued
Early-onset
nystagmus
Latent nystagmus
Acquired
nystagmus
There may be a gaze
direction in which
nystagmus is absent,
and a corresponding
head turn
What do I do?
For the new or changing cases described above, the management
is referral.The urgency of the referral depends on a variety of
factors, most significantly the speed of onset. If a patient reports
nystagmus and oscillopsia for the first time today, then very
urgent referral is indicated. If their long-standing nystagmus
appears to be gradually worsening over a period of several
months, then non-urgent referral is indicated. It is also important
to take account of the patients general health. If this is poor, or
there are other neurological signs (e.g., headache, unsteady gait),
then the referral becomes more urgent.
One or two things need to be borne in mind in dealing with
patients with long-standing and stable nystagmus. If they have a
null position, then do not do anything to make it harder for them
to use this zone. For example, if they have a null zone in upgaze
and they use this when reading then bifocal or varifocal
spectacles are contra-indicated. If there is a latent component
(worse with monocular viewing) then monovision may be contraindicated.
Many cases of long-standing nystagmus have quite high
refractive errors and careful correction of these can help the
What do I do?
Table 7.3
Characteristic
Observations
General
observations
Apparent type
of nystagmus
Direction
Amplitude
Frequency
Constancy
Conjugacy
Latent
component
Position of
gaze changes
patient to make the most of the vision that they have. If there is a
latent component to the nystagmus then it is better not to
occlude during the refraction. Instead, the Humphriss Immediate
Contrast method can be used.There is some evidence that
contact lenses, particularly gas-permeable rigid contact lenses,
might help the patient to subconsciously control the nystagmus a
little better.
Every now and then vision therapies or surgical treatments for
nystagmus are advocated. So far, none of these has been
supported by randomized controlled trials.There is a very good
support group for people with nystagmus: the Nystagmus
Network (www.nystagmusnet.org).
105
Accommodative
anomalies
108
111
Accommodative anomalies
108
How do I investigate?
The first suggestion of an accommodative problem usually comes
from the patients symptoms (Table 8.1). Careful refraction is
essential, and it is usually necessary to rule out latent
hypermetropia by cycloplegic refraction. Accommodative
amplitude is easily measured with the push up test, typically using
the RAF rule (see Table 8.2). Amplitude can also be assessed with
negative lenses, although this method gives different results to the
push up test.
Accommodative facility (the rate at which the accommodation
can be changed) is usually measured with flippers (Figure 8.1).
The procedure for this test is described in Table 8.3.The range
of responses at this test in a typical population is very wide
(Table 8.3) and this makes results difficult to interpret.
Undoubtedly, one reason for this is that the test is very
subjective. In view of the variability of the test, it is unwise to
base a treatment on this test result alone, unless the symptoms
are strongly suggestive of accommodative infacility.
It is usual for accommodation to lag a little behind the target,
but only by 0.500.75D so that the target does not appear
significantly blurred. Accommodative lag can be assessed by
dynamic retinoscopy and this is the only routine clinical test that
can be used to assess accommodative function objectively. It is an
extremely useful test, especially with children where a visual
conversion (hysterical) reaction is suspected.There are two main
approaches to measuring the accommodative lag by retinoscopy.
One of these, MEM retinoscopy, is described in Table 8.4.The idea
Near blur
Low
Accommodative
amplitude
(Table 8.2)
Accommodative
facility (Table 8.3)
Accommodative
lag (Table 8.4)
Accommodative
insufficiency
Normal
Poor
Normal
Difficulty changing
focus (e.g., copying
from board)
Accommodative
infacility
Accommodative
fatigue
Normal
Accommodative
spasm (excess)
The four main types of accommodative anomaly and their symptoms and clinical signs
Symptoms
Table 8.1
How do I investigate?
109
Accommodative anomalies
110
Figure 8.1
Minimum (D)
Minimum (cm)
14.00
7.00
13.50
7.50
13.00
7.75
10
12.50
8.00
12
12.00
8.25
14
11.50
8.75
20
10.00
10.00
30
7.50
13.25
40
5.00
20.00
50
2.50
40.00
111
Accommodative anomalies
112
Table 8.3
flippers
What do I do?
Table 8.4 Procedure for measuring accommodative lag with
MEM retinoscopy
1.
2.
The patient wears any refractive correction that they usually use
for reading
3.
4.
5.
6.
7.
8.
9.
What do I do?
A sudden-onset marked decrease in the amplitude of
accommodation can be a sign of accommodative paralysis, which
can result from neurological pathology. Such cases should be
113
Push up exercises
Plus lenses or
multifocals
Refractive
Accommodative
insufficiency
If severe, multifocals
Facility exercises
Accommodative
infacility
Eye exercises
Table 8.5
Plus lenses or
multifocals
Push up or flippers
Accommodative
fatigue
Rarely multifocals
Distance to near or
flipper or push up
Accommodative
spasm (excess)
Accommodative anomalies
114
What do I do?
referred. In other less acute and milder cases of accommodative
anomalies, where there are no suspicious signs (for example,
exclude the signs in Table 3.3), the patient can be treated but
should be carefully monitored to make sure that the condition
does not deteriorate.Treatments are outlined in Table 8.5. A
letter of information to the general practitioner is advisable in
case the patient presents to them with any other symptoms
which, together with the accommodative anomaly, might raise the
index of suspicion of pathology.
115
Index
A
A-syndrome, 84
Accommodative amplitude, 108, 109,
111
Accommodative anomalies, 108115
classification, 109
clinical characteristics, 109
with convergence, 110
intervention reasons, 111112
investigation, 108111
ruling out latent hypermetropia, 108,
112
treatment options, 113115
Accommodative facility, 108, 109, 110
Accommodative fatigue, 109, 114
Accommodative flippers, 108, 109, 110
Accommodative infacility, 109, 114
Accommodative insufficiency, 109, 114
Accommodative lag, 108110, 113
Accommodative paralysis, 113115
Accommodative spasm, 109, 114
Albinism, 100
Alternating cover test, 910
Amblyopia, 6480
anisometropic, 65, 73
bilateral, 70
differential diagnosis, 68
effect of age on treatment, 73
treatment, 59, 7579
classification, 6465
differential diagnosis, 68
amblyogenic factor detection, 68,
70
between strabismic and
anisometropic, 68
exclusion of pathology in, 68, 69
functional, 64
hysterical, 65
inherited, 68
intervention reasons, 6873
investigation, 6568
isometropic, 65, 70
occlusion, 7476
organic, 64
prevalence, 65
sensitive period, 64
stimulus deprivation, 64
strabismic, 6465, 7173
differential diagnosis, 68
sensitive period for treatment,
7173
treatment, 50, 59, 7477
visual acuity in, 65
treatment options, 50, 59, 7379
Aniseikonia, 12, 79
Anisometropia, 12, 28
correction, 12, 28, 79
microtropia with, 59, 61
see also Amblyopia, anisometropic
Autostereograms, 31
117
Index
118
B
Bagolini Lens Test, 45
Basic esophoria, 3435
Basic exophoria, 36
Bifocals, contraindications, 96, 104
Binocular instability, 24, 3940
scoring system for diagnosing,
2526
Binocular vision anomalies, 2
classification, 23
intervention options, 1213
intervention reasons, 1012
investigation, 310
symptoms, 34
Blur point, 21
Break point, 21
Browns syndrome, 98
C
Cardiff acuity test, 6667
Cataract, 28
congenital, 100
Contact lenses
in anisometropic amblyopia
correction, 7879
in nystagmus control, 105
Contoured tests, 22
Convergence excess esophoria, 36
Convergence insuffiency, 37
eye exercises for, 3839
Convergence weakness exophoria,
37
Cover test, 410
alternating, 910
cover/uncover test, 79
heterophoria detection with, 7
movement amplitude estimation, 8
strabismus detection with, 5, 7, 44
in esophoria, 6
heterophoria recovery movement
grading system, 9
in ocular motility testing, 87
recording of results, 9
in strabismus with hypertropia, 5
Cyclophoria, 16
D
Diplopia
in incomitant deviations, 87, 88, 91,
92, 9396
in spasm of the near reflex, 111
in strabismus, 42
Dissociated deviation, 1112
vertical, 56
Dissociation tests, 22
Distance esophoria, 3536
Distance exophoria, 36
Divergence excess exophoria, 3637
Divergence weakness esophoria,
3536
Duanes syndrome, 97
Dyslexia, 40
E
Eccentric fixation, 68
detection using direct
ophthalmoscope, 72
recording method, 71
Esophoria, 16
convergence excess (near), 36
divergence weakness (distance),
3536
eye exercises in, 28
mixed (basic), 3435
Esotropia, hypermetropia with, 4950,
54
Exercise glasses, 33
Exophoria, 16
convergence weakness (near), 37
divergence excess (distance), 3637
eye exercises in, 28
mixed (basic), 36
Exotropia, myopia with, 54
Extra-ocular muscles, actions, 8384
Eye exercises, 2832
for convergence insufficiency,
3839
Institute Free-space Stereogram
(IFS), 2932
in strabismus, 49
three cats, 29, 30
Index
F
Fixation, eccentric see Eccentric
fixation
Fixation disparity, 18, 42
Mallett unit test for, 1719, 20, 40
4-base out test, 5961
Foveal suppression, 22
test for, 22, 23, 24, 61
Fusion lock, 11, 18
Fusional reserves, 11, 12, 1920, 39
convergent, 1920, 28
opposing, 19
in people with dyslexia, 40
test method and norms, 21
G
Global suppression, 43
factors predisposing to, 4344
testing for, 4447
H
Harmonious anomalous retinal
correspondence (HARC), 42, 43
factors predisposing to, 4344
in microtropia, 61
testing for, 4447
Hess plot, 8889, 90
Heterophoria, 3, 1640
break down into strabismus, 54
cover test recovery assessment, 9,
17
detection with cover/uncover test, 7
intervention reasons, 2627
investigation, 1626
scoring system for diagnosing,
2526
symptoms, 1617
treatment options, 2735
eye exercises, 2832
prismatic correction, 33, 35
refractive modification, 3233, 34
removal of cause of
decompensation, 2728
surgery, 33
types, 16, 3540
K
Keeler preferential looking cards, 67,
74
L
Latent nystagmus, 56, 100, 101,
103104
latent, 101
manifest, 101
Lateral rectus palsy, 94, 95
119
Index
120
M
Maddox rod test, 22, 44
double, 92
Maddox wing test, 22, 40, 44
Mallett fixation disparity test, 1719, 40
test method and norms, 20
Mallett Modified OXO Test, 4547
MEM retinoscopy, 108, 113
Microtropia, 5862
with anisometropia, 59, 61
decompensation of
pseudo-binocularity, 62
diagnosis algorithm, 58
4_ base out test, 5961
HARC in, 61
with identity, 5859
intervention reasons, 61
investigation, 5961
treatment options, 6162
Mixed esophoria, 35
Mixed exophoria, 36
Monovision, 28
contraindications, 28, 55, 98, 104
Motor fusion, 11, 12
Myopia, with exotropia, 54
N
Near esophoria, 36
Near exophoria, 37
Negative add, 33, 37
Nonius markers, 18, 40
Null zone, 102, 105
Nystagmus, 100105
acquired (neurological), 101
classification, 100
clinical observations, 105
differential diagnosis, 103104
early-onset, 100, 102, 103104
evaluation problems, 102
intervention reasons, 102
investigation, 101102
Index
Sensory fusion, 11, 12, 18
Sixth nerve palsy, 95
Snellen charts, 6566, 67
Spasm of the near reflex, 110111
Spasmus nutans, 101
Squint see Strabismus
Stereo-acuity tests, 22
Strabismic amplyopia see Amblyopia,
strabismic
Strabismus, 3, 4256
break down of heterophoria into, 54
detection with cover/uncover test, 5,
7, 44
determination if pathology present,
5153
eye exercises in, 49
fully adapted, 61
incidence, 3, 55
infantile esotropia syndrome, 56, 101
intermittent, 50, 53
intervention reasons, 4753
investigation, 4447
latent, 3
refractive treatment, 4950
sensory adaptations to, 4244, 47
symptoms, 34
treatment options, 5356
for cosmetic reasons, 5556
long-standing strabismus, 5556
121