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106 Theory and Practice of Squint and Orthoptics
7. Lancaster WB. "Terminology," with extended comments on the position of rest and fixation. In Allen, JH, editor: Strabismus ophthalmic symposium I, St. Louis, 1950, Mosby -Year Book, Inc.
8. Lyle TK, Wybar, KC. Lyle and Jackson's practical orthoptics in the treatment of squint (and other anomalies of binocular vision), ed. 5, Springfield, III, 1967, Charles C Thomas, Publisher.
9. Paul TO, Hardage LK: The heritability of strabismus, Ophthalmic Genet. 15:1, 1994.
10. Scobee RG. Anatomic factors in the etiology of heterotropia, Am. J. Ophthalmol. 31:781, 1948.
11. Worth C. Squint, its causes, pathology and treatment, ed. 6, London, 1929, Bailliere, Tindall and Cox.
6

Evaluation of a Case of Strabismus

and Orthoptic Instruments including
Computer-based Orthoptic Programs
EVALUATION OF A CASE OF STRABISMUS History, vision evaluation and preliminary examination
History Vision evaluation Preliminary examination
Inspection
• Pupillary reactions
• Media and fundus examination
• Refraction
Motor evaluation
Cover tests
Direct cover test
• Cover uncover test
• Alternate cover test
Quantitative measurement of angle of deviation
• Bruckner pupillary red reflex test Hirschberg corneal reflex test
• Prism and alternate cover test
• Prism reflex test (Krimsky's test)
• Perimeter method
• Maddox rod test
• Synoptophore method
• Double prism test
• Haploscopic test
Assessment of ocular movements
Ductions
• Versions
• Vergences
Assessment of accommodation and AC/A ratio
Heterophoria method
Gradient method
• Fixation disparity method
• Haploscopic methods
Assessment for extraocular muscle paresis
Diplopia charting
• Quantitative measurement of action of extraocu-
• lar muscles
– Lancaster red-green test – Hess screen test – Lees screen test
Field of binocular fixation
• Bielschowsky phenomenon test
• Bielschowsky head tilt test
Sensory evaluation: Assessment for binocular cooperation and sensory anomalies
Tests for fixation behaviour
• Tests for the state of retinal correspondence
• Tests for binocular vision
• Tests for suppression and amblyopia
ORTHOPTIC INSTRUMENTS Conventional Orthoptic Instruments
Synoptophore
• Livingston binocular gauge
• Visuscope
• Euthyscope
Computer Based Orthoptic Programs
• Computerised orthoptic diagnostic programs
• Computer based vision therapy and neurovision therapy programs
• Computer based combined diagnostic and therapeutic programs
EVALUATION OF A CASE OF STRABISMUS
To avoid repetition, the subject of evaluation is discussed even before the clinical description of different varieties of the squint. Though the list of tests is exhaustive, all of these may not be required in every case. An experienced strabismologist chooses a set
of tests to be performed in a particular patient.
Main components of evaluation of a case of strabismus are: I. History, vision evaluation and preliminary
examination
History
Vision evaluation
Preliminary examination
108 Theory and Practice of Squint and Orthoptics
II. Motor evaluation
Head posture evaluation
Evaluation for ocular deviation
– Cover tests – Corneal reflex tests – Subjective tests for measurement of deviation – Measurement for cyclodeviation
Assessment for ocular movements
– Evaluation of ductions – Evaluation of versions – Evaluation of vergence
Assessment of accommodation, and AC/A
ratio
Assessment for extraocular muscle paresis
III. Sensory evaluation
Tests for fusion,
Tests for binocularity and diplopia
Tests for fixation
Tests for status of retinal correspondance
Tests for suppression and amblyopia
Tests for stereopsis.
In general, for a systematic evaluation,
following approach may be adopted.
HISTORY, VISION EVALUATION AND PRELIMINARY EXAMINATION
HISTORY
A detailed and meticulous history taking before beginning the examination is very important in the management of a case of squint. A complete history taken should contain the following information.
1. Age of the patient
2. History of present illness. Information about
deviation of eye should include:
Time when first noticed. It is desirable to
document the age of onset of deviation or symptoms. All available old photographs of the child are invaluable for this purpose.
Onset, sudden or gradual.
Constant or intermittent deviation.
Unilateral or alternating deviation.
If alternating, which eye more frequently
fixates (dominant eye).
Alleged cause, if any, such as trauma, illness,
psychologic disturbance, change in occupation, increase in close work, etc.
Abnormal head posture (ask to demonstrate).
Associated symptoms such as diplopia,
blurred vision, headache, asthenopia.
Any history of closing or covering one eye
in bright light indicates intermittent squint.
3. Birth history. It is especially important in cases of childhood onset deviations. Enquiry should be made regarding:
Problems during pregnancy and
Problems during delivery
4. Family history. History regarding any of the following disorders in grandparents, parents, siblings or even in uncles, aunts and cousins is important;
Strabismus
Refractive errors
Lazy eye
Other ocular defects
5. Past history
General ill health
Any systemic disease
Any ocular defect
6. History of previous treatment
a. Optical treatment
When and how long?
Were glasses worn constantly?
Effect of glasses on the deviation.
Date of last refraction.
b. Orthoptic treatment.
Occlusion. Type, when, how long, effect
on vision.
Exercises. When, type, how long, effect
on deviation.
c. Surgical treatment
When performed?
Eye operated: Right, left or both.
Muscle touched. Medial, lateral or both
(if known).
Patient's/parents' opinion about the result
of surgery.
VISION EVALUATION
Testing of visual acuity of each eye separately is critical in evaluation of any patient with strabismus. Visual acuity should be tested without glasses and with glasses (if worn); and for near and distance vision.
109Evaluation of a Case of Strabismus and Orthoptic Instruments
Methods of testing visual acuity in infants, preschool age children and in school-age children as well as adults have been described in Chapter 3 (see pages 38–54). However, for a ready reference, these are enumerated below.
Methods of estimating visual acuity in infants
1. Fixation behaviour test
2. Preferential looking test
3. Optokinetic nystagmus
4. Visually evoked response
5. CSM method
Methods of estimating visual acuity in preschool-age children
1. Marble game test
2. Hand chart
3. Illiterate E-game test
4. Allen's preschool vision test
5. Sheridan-Gardiner test
Methods of estimating visual acuity in school­age children and adults
1. Snellen's test types
2. E-chart
3. Landolt's broken-C chart
Visual acuity in patients with nystagmus. In patients with nystagmus, visual acuity may be better with both eyes open than with one eye occluded. In order to assess this situation, it may be helpful to place a +5.00D sphere lens in front of the eye not being tested, and then reverse this in order to test the other eye.
PRELIMINARY EXAMINATION
Inspection
Large degree squint (convergent or divergent) is obvious on inspection.
Epicanthus, when observed in infants, may be the cause of pseudoesotropia.
Facial asymmetries may also create the impression of pseudostrabismus especially hypertropia.
Abnormal head posture (AHP) when present, becomes obvious during initial inspection of the patient. Infact observation for AHP should always be made without any instructions to the patient, otherwise a lot of information may be lost after the patient becomes concious of being examined. As described in detail on page 302,
observation should be made for all the three components of head posture, i.e.
Chin elevation or depression (vertical
component),
Face turn to right or left (horizontal
component), and
Head tilt to right or left shoulder (torsional
component)
Interpupillary distance (IPD) should also be inspected and measured. Unusually, narrow IPD may be the cause of pseudoesotropia and an exceptionally wide IPD may be a cause of pseudoexotropia.
Inspection during pen light examination may sometimes erroneously reveal strabismus owing to presence of a large angle kappa. A large positive angle kappa may be a cause of pseudoexotropia and a large negative angle kappa may be a cause of pseudoesotropia. For details of angle kappa and its measurement, see page 200.
Pupillary reactions
Light reflexes may be abnormal in patients with sensory deviations due to diseases of retina and optic nerve and in squint due to pupil involving third nerva paralysis.
Media and fundus examination
It may reveal associated diseases of the ocular media, retina or optic nerve. Fundus should also be evaluated for presence of torsion (extorsion or intorsion).
Refraction
It is most important, because a refractive error may be responsible for the symptoms of the patient or for the deviation itself. Refraction is performed before complete squint check-up. Preferably, refraction should be performed under full cycloplegia especially in children. The commonly used cycloplegics are as follows:
1. Atropine is indicated in children below the age of 5 years. It is used as 1% ointment thrice daily for 3 consecutive days before performing retinoscopy. Its effect lasts for 10–20 days.
2. Homatropine is used as 2% drops. One drop is often instilled every 10 minutes for 6 times and the retinoscopy is performed after 1 to 2 hours. Its effect lasts for 24–28 hours. It is used
110 Theory and Practice of Squint and Orthoptics
for most of the hypermetropic individuals between 7 and 35 years of age.
3. Cyclopentolate is a short-acting cycloplegic. Its effects last for 6 to 18 hours. It is used as 1% eyedrops in patients between 7 and 35 years. One drop of cyclopentolate is instilled every 10 minutes for 3 times (Havener's recommended dose) and the retinoscopy is performed 1 to 1½ hours later after estimating the residual accommodation, which should not exceed one dioptre.
MOTOR EVALUATION
EVALUATION FOR OCULAR DEVIATION
COVER TESTS
Prerequisites for cover tests
Patient should be co-operative enough to
fixate a target.
Should have sufficient vision to see the target.
Should have central fixation in both eyes.
Latent nystagmus should not be present.
Methods of cover tests
The cover tests should be performed with and without glasses at distance (6 m) and at near (33 cm). The patient is asked to fixate a 6/12 visual acuity symbol or any object that keeps patient's attention, such as a small picture or a toy (especially in small children), with one eye. This is to prevent the use of accommodation, when torch light is used (which is a common practice). The other eye is then covered with the help of an occluder or palm of the hand. The interpretations are made as described below.
Fig. 6.1 Direct cover test depicting left exotropia.
No movement of the uncovered eye on covering the seemingly fixating eye indicates any of the following:
No squint (pseudostrabismus)
Gross eccentric fixation (there may be very
small or no movement of redress in the uncovered deviated eye)
No vision in the deviated uncovered eye.
Cover-uncover test
Aim. To establish the presence and type of heterophoria (latent deviation).
Procedure (Fig. 6.2). It is performed, when direct cover test has established that no manifest deviation is present. To perform it, one eye is covered with an occluder and the other is made
Direct cover test
Aim. To confirm the presence of a manifest squint. Procedure. To perform this test, patient is first
asked to fixate (Fig. 6.1) a point with both eyes open. The normal looking eye is covered while observing the movement of the uncovered eye. In the presence of manifest squint, the uncovered eye will move in the opposite direction to take fixation. For example, when an exotropia is present, the eye taking up fixation will move towards nose and in the presence of esotropia, it will move towards the temple.
Fig. 6.2 Cover-uncover test depicting exophoria. Note
orthophoria in primary position (A) and immediate inward movement of right eye on removal of the cover (B and C).
111Evaluation of a Case of Strabismus and Orthoptic Instruments
to fixate an object. In the presence of hetero­phoria, the eye under cover will deviate (since covering one eye of a patient with normal binocular vision interrupts fusion). After a few seconds, the cover is quickly removed and the movement of the eye (which was under cover) is observed. Direction of the movement of the eyeball tells the type of heterophoria (e.g. the eyeball will move towards the nose in the presence of exophoria and towards the temple in the presence of esophoria) and the speed of movement tells whether recovery is slow or rapid.
Alternate cover test
Aim. To establish whether the squint is unilateral or alternate and also to differentiate concomitant squint from paralytic squint (where secondary
deviation is greater than primary deviation). Procedure (Fig. 6.3). To perform this test, patient is asked to fixate an object alternately with each eye. It is important to place occluder alternately in front of each eye several times to dissociate the eyes and to maximize the deviation. Further, the occluder should be quickly transferred from one eye to other to prevent fusion from occurring. Observations made are as follows:
In the presence of an alternate squint, either eye fixates and the opposite eye under cover deviates and maintains the position of deviation on removing the cover; but in the presence of a unilateral squint, after removal of the cover, always the normal looking eye takes up fixation and the squinting eye deviates.
To differentiate concomitant squint from paralytic squint, observation about the degree of deviation in the eye under cover is made while performing the alternate cover test. In concomitant squint, primary deviation is equal to secondary deviation (deviation of the normal eye under cover); while in paralytic squint, secondary deviation is much more than the primary deviation.
Limitations of cover tests
Following deviations may be either overlooked or cannot possibly be diagnosed with cover tests:
A small heterophoria
A small angle esotropia (of less than 5)
A microtropia
A monofixation syndrome
A cyclodeviation
QUANTITATIVE MEASUREMENT OF ANGLE OF DEVIATION
Various methods for quantitative measurement of angle of deviation can be grouped as under:
Objective tests for heterotropia
Hirschberg corneal reflex test
Prism and cover test (prism bar cover test)
Prism reflex test (Krimsky's corneal reflex test)
Synoptophore test
Perimeter method
Subjective tests for heterotropia
Maddox rod test
Synoptophore test
Diplopia test
Hess/Lees screen test
Objective tests for heterophoria
Prism and cover test
Subjective tests for heterophoria
Maddox rod test
Maddox wing test
Double prism testFig. 6.3 Alternate cover test depicting alternate exotropia.
112 Theory and Practice of Squint and Orthoptics
Tests based on corneal reflex
Hirschberg corneal reflex test
Prism and cover test (prism bar cover test)
Prism reflex test (Krimsky's corneal reflex test)
Perimeter method
Synoptophore method
Bruckner test
Dissimilar image tests
Maddox rod test for heterophoria or hetero-
tropia
Double Maddox rod test for cyclodeviations
Red glass test
Dissimilar target tests
Major amblyoscope test
Lancaster red-green projection test
Hess screen test
Bruckner pupillary red reflex test
This is a screening test and does not measure the size of deviation. It is performed with the help of a direct ophthalmoscope. Patient is made to look into the light and examiner compares the brightness of pupillary red reflex of both eyes obtained simultaneously,
In orthophoria, the Bruckner red reflex is
symmetric in the two eyes.
In strabismus, the reflex is brighter in the
deviated eye.
In ocular pathologic conditions such as large
anisometropia, gross retinal pathology, large retinal detachment, media opacities (corneal, lenticular or vitreal), the red reflex is altered in the affected eye.
Hirschberg corneal reflex test
It is a rough but handy method to estimate the angle of manifest squint. In this test, patient is asked to fixate at a point light held at a distance of 33 cm and the deviation of corneal reflex from the centre of pupil is noted in the squinting eye. Hirschberg reported that each 1 mm decent­ration of corneal reflex corresponds to 7° (14) of deviation of the visual axis. Thus, roughly the angle of squint is 15 degree (about 30 prism dioptres) and 45 degree (about 90 prism dioptres) when the corneal light reflex falls on the border of pupil and limbus, respectively (Fig. 6.4).
Fig. 6.4 Hirschberg corneal reflex test depicting ortho-
position (A); right exotropia (B) 15°; (C) 30°; (D) 45° and right esotropia (E:15°, F:30°, G:45°).
Prism and alternate cover test
(prism bar cover test, i.e. PBCT)
In practice, this test is most popular and a simple method of measuring the angle of deviation objectively in various diagnostic positions of gaze.
Prisms of increasing strength with apex towards the deviation are placed in front of one eye and
the patient is asked to fixate a target with the other eye. For large deviations, a loose prism of 30 or 45 prism dioptre is placed in front of one
113Evaluation of a Case of Strabismus and Orthoptic Instruments
Fig. 6.5 Prism and cover test with its optical principle. (A) right esotropia; (B) image of the object fixated by left eye is
projected on major half of retina of the right eye; (C and D) when left eye is covered, right eye moves outwards to take over fixation and under the cover left eye performs an inward movement of equal amplitude following Hering's law of equal innervation; (E and F) when cover is transferred to right eye, the left eye moves outwards to takeover fixation and under the cover right eye performs an inward movement; (G and H) a prism base-out is held before the right eye and cover is transferred to the left eye. There is still outward movement of the right eye when taking over fixation; (I and J) cover is again transferred and a prism of greater power is held before the right eye; (K and L) transfer of cover to the left eye does not show any outward movement of the right eye indicating that it is the end point of the prism and cover test. At this juncture, prism of sufficient power offsets the nasal displacement and the right eye will no longer change its position when left eye is covered. The power of this prism equals the deviation.
eye and the prism bar is used in front of the other eye.
When horizontal and vertical deviations co-exist,
the prisms are placed horizontally in front of one eye and vertically in front of the other eye.
Alternate cover test is then performed till there
is no recovery movement of the eye under cover (Fig. 6.5). This will tell the amount of deviation in prism diopters.
Both heterophoria as well as heterotropia can be measured objectively by this method.
114 Theory and Practice of Squint and Orthoptics
Test should be performed for distance and near fixation, using an accommodative target.
Test should be performed, preferably in all the nine diagnostic positions of gaze. In Boyce-
Smith deviometer (Fig. 6.6), nine prefixed retroilluminated slides (accommodative targets) positioned 35° from the primary position are used to perform cover and prism test in diagnostic positions of gaze.
One great advantage of using a deviometer is that the prism and cover test can be performed in diagnostic positions under exactly the same conditions on different occasions and thus permit meaningful comparison of test results (e.g. preoperative and postoperative).
Comparison of prism and cover test performed in primary position, straight up position and straight down position is very useful in detecting the 'A', 'V' and 'X' pattern heterotropias.
Test should be repeated by holding the prism and occluder before the other eye to detect any
difference between right and left fixation, which would indicate incomitance.
Uses of prism and cover test
A carefully performed PBCT can provide following useful information:
1. Nature of deviation of esotropia (basic or convergence excess or divergence insufficiency type) and exotropia (basic or convergence insufficiency or divergence excess type) can be known from the results of near and distance measurements.
2. Accommodative element of deviation can be known from measuring deviation for far and near with and without glasses (including bifocals, if any).
3. Incomitance, if any, can be detected by performing the test in 9 cardinal positions of gaze.
4. A-V patterns can be detected by measuring the deviation in upgaze of 25° and downgaze of 35°.
5. Primary versus secondary deviation can be detected by measuring the deviation with right and left eye fixating alternately.
6. Divergence excess versus simulated divergence excess exotropia can be differentiated by measuring the deviation before and after prolonged occlusion.
Fig. 6.6 The Boyce-Smith deviometer.
Limitations of prism and cover test
Since, this test requires an accurate fixation,
so it cannot be performed, if the deviating eye is blind or has gross eccentric fixation.
The optical qualities of the prisms also limit
the test accuracy. The stronger the prism, the greater the error.
The spectacle lenses with power more than
±5D introduce a significant artifact in measurement of deviation. Plus lenses decrease and minus lenses increase the measured deviation.
The presence of manifest as well as latent
nystagmus hinders the accurate measure­ments by prism and cover test.
Test accuracy is also limited by the minimum
movement of redress that the examiner can detect with naked eye.
Prism reflex test (Krimsky's corneal reflex test)
Prism reflex test first described by Krimsky has become universally accepted as a practical method of estimating the size of the angle of
115Evaluation of a Case of Strabismus and Orthoptic Instruments
squint in patients with a blind or deeply amblyopic eye with or without eccentric fixation.
To perform the test, patient is asked to fixate on a point light and prisms of increasing strength (with apex towards the direction of manifest squint) are placed in front of the normal fixating eye till the corneal reflex is centered in the squinting eye (Fig. 6.7). The power of prism required to centre the light reflex in the squinting eye equals the amount of squint in prism dioptres. To avoid errors from parallax, the examiner must observe the corneal reflex with one eye by sitting directly in front of the deviating eye while keeping his other eye closed.
In an alternative method, prism of increasing power can be placed in front of the deviating eye until the corneal reflexion is centred.
Fig. 6.7 The Krimsky's corneal reflex test. (A) right exotro-
pia; (B and C) prism base-in of increasing powers are placed in front of the fixing left eye till the corneal reflex centres in the right eye (C); (D) optical principle of the prism reflex test.
However, since the observation of the corneal reflexion through prisms is difficult, therefore, the method described above is preferred.
Advantages of Krimsky's test
Since the test requires only that the patient
fixate the light, being entirely objective otherwise, it is useful in testing small children.
It is quicker to perform than the prism cover test.
It can be used in patients in whom the
deviating eye has a low visual acuity or has lost central fixation.
Limitations of the Krimsky's test
Since the angle kappa is included in the
measurement, the test is inaccurate.
It is impossible to perform the test for distance
fixation, since the position of the examiner's head required to obtain an accurate obser­vation prevents the patient from seeing the fixation light.
Simultaneous prism cover test
Aim. The aim of this test is to measure only the tropia without dissociating the phoria.
This special cover test is used to measure the tropia component of patient with small tropia <10 PD and a larger phoria (i.e. monofixation syndrome). It is not useful for larger tropia (>10 PD), which does not permit fusion and, therefore, does not have an associated phoria.
Procedure. This test is performed as below:
Hirschberg corneal reflex test is performed to
estimate the approximate size of tropia (Fig 6.8A).
Simultaneous prism cover test is then performed
by placing the prism of the size of tropia in front of deviating eye and an occluder in front of the fixating eye (Fig. 6.8B). The power of prism with which the deviated eye does not show refixation shift equals the tropia.
Note. For patients with monofixation syndrome, the amount of tropia is measured with simul­taneous prism cover test and the total angle (tropia plus phoria) is measured with the alternate prism cover test.
Perimeter method
Patient is asked to fixate at O-mark on the arc perimeter with normal eye and a flash light is