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

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17 PICKWELL’S BINOCULAR VISION ANOMALIES

4th nerve

Optic tract

3rd nerve

Posterior cerebral artery

6th nerve

Superior orbital tissue

1st division of V

Inferior corp.

 

 

2nd division of V

quod.

Superior

 

 

(turned forwards)

 

 

 

cerebellar

Olive

Inferior

 

artery

Apex of

carotid

 

 

petrous

artery

Anterior inferior

temporal

cerebellar artery

Vertebral

 

 

artery

Figure 17.5 Diagram illustrating part of the course of the nerves innervating the

extraocular muscles. The long pathway of the fourth and sixth nerves makes them prone to

damage. The fourth nerve is particularly slender. Where the sixth nerve bends at the petrous

temporal bone it is particularly prone to damage from compressive lesions from above

following raised intracranial pressure or from below, e.g. following otitis media infection.

(Modified after Lindsay 1941.)

 

(e.g. congenital Brown’s syndrome) or acquired (e.g. blow-out fracture).

Depending on the cause, acquired incomitancies may undergo sponta-

neous partial or complete recovery. So surgical intervention is often post-

poned until consecutive Hess plots have been stable for at least 6 months.

Investigation

 

 

 

Most of the rest of this chapter is concerned with the detection of incomitant

 

 

 

deviations and the interpretation of their significance. Most patients in need

 

 

 

of urgent medical attention have symptoms that lead them to consult med-

 

 

 

ical practitioners in the first instance. It is, however, important to be able

 

 

 

to detect incomitancy, as it does not usually respond well to eye exercises,

 

280

 

and occasionally cases of active pathology present themselves and it is essen-

 

 

tial to be able to recognize them.

INCOMITANT DEVIATIONS

17

Normally, the first indication of incomitancy will emerge during a routine eye examination, and this may lead us to carry out additional tests to confirm the diagnosis. The sections of the routine and appropriate additional tests are reviewed below.

History and symptoms

Incomitant deviations due to recent injury or to active pathology nearly always have a sudden and dramatic onset of symptoms, sudden diplopia being the most usual. In long-standing deviations, the symptoms are seldom so disturbing to the patient and of course they are usually reported as having been present for as long as the deviation. The following symptoms may be present:

(1)Diplopia is often present in incomitancy but may not be present in heterophoric incomitancy or in long-standing strabismic incomitancy. The patient may be able to recognize the variation in the degree of doubling in different directions of gaze. There is usually a vertical element in the diplopia. In long-standing cases, it may be intermittent because sensory adaptations have intervened. Two-thirds of patients who acquire strabismus following brain damage (usually stroke or trauma) do not experience diplopia (Fowler et al 1996).

(2)Asthenopia may be present, presumably if the patient has a heterophoric incomitancy, and is not attributable to the emotional state of the patient (Smith 1979).

(3)Blurred vision may be present if the condition involves the third cranial nerve, which also serves the ciliary muscle. For the same reason, the pupil reflexes may be abnormal. Some patients interpret small degrees of diplopia as blur.

(4)Dizziness or vertigo may accompany incomitant heterophoria (Rabbetts 2000, p 179). Normally, a change in the pattern of innervation to the extraocular muscles is associated with a particular movement of the retinal image. Incomitancy results in an imbalance between innervation and retinal image movement and this can make the patient’s surroundings appear to move. If the paresis is mild there may be an incomitant heterophoria rather than strabismus with diplopia. Hence, the symptoms of vertigo and dizziness may be reported.

(5)Other symptoms due to the disease causing the incomitant deviation may be present, e.g. headache in intracranial conditions, neoplasms, vascular disturbances, etc. The diseases most likely to be associated with incomitant deviations are dealt with in a later section of this chapter, where their symptoms are also summarized.

(6)General health deterioration may also occur in accompanying metabolic disorders: loss of weight, changed appetite, general fatigue, loss of muscular ability, muscular tremor, breathlessness, etc.

(7)Injury to the head or orbital regions may be reported and this could cause damage to the muscular apparatus, intracranial bruising, and damage

or pressure from haemorrhage. This can be recent or be the explanation

281

17 PICKWELL’S BINOCULAR VISION ANOMALIES

of a long-standing incomitant deviation. In some cases, the patient may not have thought the injury serious enough to seek medical advice at the time. Injury during the birth delivery sometimes causes lateral rectus palsy. An operation for a previous strabismus can sometimes cause a degree of incomitancy.

(8) Previous comitant strabismus can result in an acquired incomitancy. Months or years of a marked comitant deviation can result in mechanical changes to the extraocular muscles and this can result in an acquired incomitancy (see below).

Anomalous head postures and facial asymmetry

Acquired ocular torticollis is a type of anomalous head posture (AHP) that occurs in some patients with incomitant deviations, most commonly superior oblique paresis (Nucci et al 2005). In nearly every case, the purpose of the AHP is to reduce the effect of the incomitancy, so the AHP turns the head towards the field of action of the affected muscle. Generally speaking, if the underacting muscle is horizontally acting there will be a head turn, if it is a torsional muscle (obliques) there will be a head tilt and if it is a vertically acting muscle there will be an elevation or depression of the chin. The most commonly encountered AHPs are for lateral rectus palsies and superior oblique pareses (right superior oblique paresis: top of head tilted to patient’s left; right lateral rectus palsy: head turn to right), Duane’s syndrome (head turn), pattern deviations (elevation or depression), and Brown’s syndrome (may be turn, elevation and tilt). Sometimes, a head tilt is adopted in a vertical incomitancy to level the diplopic images and so aid fusion. Common AHPs are detailed in Appendix 8.

It should be noted that typical AHPs outlined above apply to the usual situation, when the purpose of the AHP is to reduce the effect of the incomitancy. Very rarely, the AHP may be adopted for the opposite reason: to exaggerate the effect of an incomitancy (von Noorden 1996, p 412). For these rare cases, this can have two advantages: it can cause the deviation to break down and hence eliminate a symptomatic heterophoria, or it can cause diplopic images to move further apart, making them easier to ignore. These cases are easy to detect because patients will be strabismic when they view a straight ahead object using their normal AHP. Another complication is that a compensatory head posture may be provoked not so much by the primary paralysis as by the modifications of other muscles induced by the paralysis (secondary sequelae).

An AHP that has been present for many years is frequently associated with facial asymmetry and this is found in more than 75% of patients with congenital palsy, typically from a congenital superior oblique palsy. The more shallow side of the face is always on the side of the head tilt (Plager 1999). The presence of a facial asymmetry is such a strong sign of an early

onset that it may preclude the need for a neurological investigation (Plager 282 1999).

INCOMITANT DEVIATIONS

17

Since the usual purpose of an AHP is to preserve or enhance some binocularity, the presence of an AHP suggests that the patient has had binocularity at least at some time in the past, and this improves the prognosis for treating sensory factors.

Other visual causes of AHPs are a visual field loss and to move the visual axes into the null zone in congenital nystagmus (Ch. 18). However, most (60%) cases of torticollis in children are non-visual, mainly orthopaedic (Nucci et al 2005) but also sometimes from unilateral deafness, shyness or just habit.

External examination of the eyes

General inspection may show an obvious strabismus. Scars or asymmetry of the orbital region may indicate previous injury. Some eye signs of systemic disease may be seen in conditions that are sometimes accompanied by strabismus: exophthalmos, ciliary hyperaemia, ptosis, etc.

Eyelid signs

Abnormalities of the lids may sometimes be useful in indicating the presence of an incomitant deviation. The width of the palpebral fissure should be noted:

(1)In the primary position when the right and left lid openings are compared. The width may be judged by the amount of the limbus visible through the lid openings. An abnormally wide fissure (Dalrymple’s sign of thyrotoxicosis) may be accompanied by hypophoria or hypotropia that increases on elevation of the eyes. Ptosis and diplopia that are both worse at the end of the day can be an early sign of myasthenia gravis, a rare muscle disease. Ptosis can also be a sign of third nerve palsy. A hypotropic position of one eye may show a ‘pseudoptosis’; the lid is slightly lower as the eye is turned down.

(2)During the motility test, a lag of the lids on downward gaze (von Graefe’s sign) may be present in thyrotoxicosis. A change in lid fissure when looking left or right occurs in Duane’s retraction syndrome (p 310).

Ophthalmoscopy and fundus photography

The internal examination of the eyes may also provide further evidence

 

of pathology such as those present in vascular conditions or metabolic

 

disease. Indirect ophthalmoscopy and fundus photography can be used

 

to provide an objective measure of ocular torsion in which the relative

 

positions of the fovea and optic disc are noted. Normally, the fovea is 0.3

 

disc diameters below a horizontal line extending through the geometric

 

centre of the optic disc. A variation of more than 0.25 disc diameters

 

between the two eyes indicates cyclodeviation (von Noorden 1996). Visual

283

field analysis can also be used in a similar way. A problem with these

 

17

 

PICKWELL’S BINOCULAR VISION ANOMALIES

 

 

 

 

approaches is their sensitivity to improper head position (Phillips &

 

 

 

 

Hunter 1999).

 

 

 

 

Ocular motility test

 

 

 

 

The examination of ocular motility is an essential part of the detection of

 

 

 

 

 

 

 

 

incomitancy. The ocular motility test allows a subjective, and an objective,

 

 

 

 

check that:

 

 

 

 

(1) both eyes move smoothly and follow the target

 

 

 

 

(2) there is a corresponding lid movement accompanying the vertical eye

 

 

 

 

movements

 

 

 

 

(3) there is no underaction or overaction of the movement of one eye in

 

 

 

 

any direction of gaze.

 

 

 

 

The details of procedure for investigating the motility in routine exami-

 

 

 

 

nation are given in Chapter 2. This chapter is mainly concerned with

 

 

 

 

determining the significance of any anomaly and with any additional tests

 

 

 

 

that may give further information. The site of a muscle palsy can be deter-

 

 

 

 

mined from an understanding of the actions of the extraocular muscles, as

 

 

 

 

described at the beginning of this chapter.

 

 

 

 

In the motility test, the patient is asked to keep the head still and to fol-

 

 

 

 

low a pen torch with the eyes as it is moved into the different parts of the

 

 

 

 

visual motor field. The patient is asked to report any diplopia, although

 

 

 

 

patients with an incomitancy may not report any diplopia, because of sens-

 

 

 

 

ory adaptations. Often, the most useful information that the motility test

 

 

 

 

gives relates to the practitioner’s observation of the eye movements.

 

 

 

 

The fixation light is first moved up and down in the median plane, so

 

 

 

 

that lid movements and vertical eye movements (e.g. detecting gaze palsies)

 

 

 

 

can be observed. In the method recommended by Boylan & Clement (1987),

 

 

 

 

the light is then moved across the field at three levels: at the top, at eye

 

 

 

 

level and in the lower part of the motor field. This is done with the patient

 

 

 

 

following the light with both eyes, so that one eye’s position can be judged

 

 

 

 

relative to the other. A failure of one eye to follow the light in the top of the

 

 

 

 

field indicates an anomaly of one of the elevators. To the patient’s right

 

 

 

 

and top, the affected muscle is likely to be either the right superior rectus

 

 

 

 

or the left inferior oblique. Failure of one eye to turn to the right or to the

 

 

 

 

left at eye level is likely to show an anomaly of either medial recti muscles

 

 

 

 

or either lateral recti, and failure in the lower field shows a problem with one

 

 

 

 

of the depressor muscles (Fig. 17.6). In these directions of gaze, each muscle

 

 

 

 

has little or no secondary actions. Alternatively, some authors recommend

 

 

 

 

that a ‘star’ technique is used where the pen torch is moved in the vertical,

 

 

 

 

horizontal (at eye level) and four oblique positions (Mallett 1988a).

 

 

 

 

It should be noted that Figure 17.6 does not show muscle actions but the

 

 

 

 

approximate directions in which the muscles have their greatest ability to

 

 

 

 

move the eyes, excluding torsional movements. These diagnostic positions

 

 

284

 

of gaze are very different from the actions of the extraocular muscles in the

 

 

 

primary position (Table 17.1). This is because the actions of each muscle

 

 

 

INCOMITANT DEVIATIONS

17

 

RSR

 

RIO

 

 

 

LIO

 

LSR

 

 

 

 

R

L

 

 

RLR

 

 

 

 

RMR

 

 

 

 

LMR

 

 

 

 

LLR

 

 

 

RIR

 

RSO

 

 

 

LSO

 

LIR

 

 

Figure 17.6 The six cardinal diagnostic positions of gaze, indicating the muscles that

 

 

should have maximum power to maintain the eyes in these directions. The paired synergists

 

 

(muscles, one from each eye, that act together) are shown.

 

 

will change as the eye moves. The cardinal diagnostic positions of gaze

 

 

are important in interpretation of ocular motility results but knowledge

 

 

of the actions of the muscles in the primary position is required to interpret

 

 

the results of the cover test carried out in the primary position.

 

 

The motility test is the only objective method available for standard

 

 

clinical investigation of muscle paresis. Small deviations of one eye when

 

 

it is in a tertiary position are not easy to detect. Observation of the corneal

 

 

reflection of the fixation light will help, as will the symmetry of the lid

 

 

and eye positions comparing dextroversion with laevoversion. Fortunately,

 

 

from the detection point of view, underaction of a muscle is usually accom-

 

 

panied by an overaction in the paired synergic muscle, which exaggerates

 

 

the deviation.

 

 

 

 

 

Patients with active pathology usually have diplopia and this helps the

 

 

detection. Very small degrees of diplopia may be detected subjectively, which

 

 

makes diagnosis more certain. During the motility test, therefore, the patient

 

 

must be asked to report any doubling and how this varies in different parts

 

 

of the field. A diplopic image due to a paretic muscle is displaced in the same

 

 

direction as the rotation that contraction of that muscle normally produces.

 

 

The eye that sees the outermost diplopic image when the eyes look in the

 

 

direction of maximum separation of the images is therefore the eye with

 

 

the paretic muscle, and this eye can be identified by covering one eye.

 

 

Subjective analysis of ocular motility can be assisted by the use of red–green

 

 

diplopia goggles. A red goggle is worn before the right eye and a green one

 

 

before the left. However, goggles prevent the eyes from being observed.

 

 

It is useful to be able to record the degree to which a deviation is incomi-

 

 

tant, so that any change can be monitored to assess if the condition is getting

 

 

better or worse. This can be done by several methods and Appendix 8 is a

 

 

worksheet for recording these results. This includes three variations of the

 

 

motility test, including cover testing in peripheral gaze as described below.

 

 

Incomitant deviations can be difficult to diagnose and these three versions of

285

 

the motility test are often easiest to interpret if they are carried out separately.

 

 

17

 

PICKWELL’S BINOCULAR VISION ANOMALIES

 

 

 

 

The cover test

 

 

 

 

In the primary position, the cover test can be used to compare the size of

 

 

 

 

the deviation when the patient is fixating with either eye. The deviation is

 

 

 

 

larger (secondary deviation) when the patient is fixating with the paretic

 

 

 

 

eye than when they are fixating with the non-paretic eye (p 278).

 

 

 

 

 

 

 

 

The cover test also can be used to measure the deviation in different pos-

 

 

 

 

itions of gaze during the motility test. The cover/uncover test, alternating

 

 

 

 

cover test or prism cover test can be used in peripheral gaze to provide fur-

 

 

 

 

ther objective information on the motility test results. The alternating

 

 

 

 

cover test is often easiest to interpret, but with just two or three alternate

 

 

 

 

covers in each position of gaze to avoid causing more dissociation than is

 

 

 

 

necessary.

 

 

 

 

The Maddox rod or hand frame

 

 

 

 

Primary and secondary deviations

 

 

 

 

The difference between primary and secondary deviations was explained earl-

 

 

 

 

ier in this chapter. This phenomenon can be investigated with a Maddox

 

 

 

 

rod test in the primary position with the rod first in front of the strabismic

 

 

 

 

eye (measuring the primary deviation) and then in front of the non-

 

 

 

 

strabismic eye (measuring the secondary deviation). If the two readings

 

 

 

 

are different this suggests an incomitancy (Borish 1975, pp 1262–1264).

 

 

 

 

Unfortunately, I have been unable to find any norms for determining what

 

 

 

 

represents a significant difference between the two eyes with this test, but

 

 

 

 

it is included in Appendix 8.

 

 

 

 

Deviation in different positions of gaze

 

 

 

 

The Maddox rod can be used to measure the horizontal and vertical devi-

 

 

 

 

ations in different directions of gaze (Appendix 8). Using a pen torch for

 

 

 

 

fixation, the patient’s head is kept still while measurements are taken in dif-

 

 

 

 

ferent parts of the field. It is important that the light is at a fixed distance

 

 

 

 

and is moved to definite peripheral positions, so that the test is repeatable.

 

 

 

 

It is suggested that it is held at 50 cm from the eyes, and at the corners of a

 

 

 

 

square formation in front of the patient and of 50 cm dimensions.

 

 

 

 

Double Maddox rod test and similar approaches

 

 

 

 

In the double Maddox rod test, two Maddox rod lenses are placed one in

 

 

 

 

front of each eye to measure any cyclodeviation (Phillips & Hunter 1999).

 

 

 

 

The rods are placed exactly vertical in a trial frame and the test should be

 

 

 

 

carried out in complete darkness (Simons et al 1994). If there is no vertical

 

 

 

 

deviation then a vertical prism is introduced to separate the horizontal lines

 

 

 

 

seen by each eye. The orientation of the Maddox rod in the trial frame can

 

 

 

 

be adjusted until the two lines are parallel. This gives a measure of the

 

 

 

 

cyclodeviation but does not differentiate between a cyclophoria and a

 

 

286

 

cyclotropia. A significant cyclodeviation suggests the involvement of an

 

 

 

oblique muscle.

INCOMITANT DEVIATIONS

17

The tilt of the retinal image is opposite to the tilt of the line as seen by the

 

 

observer. So, if patients report that the line seen by the right eye is tilted

 

 

towards the nose then they have right excyclodeviation, suggesting that the

 

 

right superior oblique may be underacting. In summary, the line is perceived

 

 

to be tilted in the direction in which the underacting muscle would rotate the eye.

 

 

Paresis of the superior oblique muscle can be very difficult to detect on

 

 

motility testing (Brazis 1993) and Simons et al (1994) stated that the dou-

 

 

ble Maddox rod test is the standard test for investigating a superior oblique

 

 

paresis. Theoretically, the test can demonstrate which eye(s) manifests the

 

 

paresis and the degree of excyclotorsion (p 306).

 

 

Von Noorden (1996, p 411) cautioned that an excyclodeviation may occur

 

 

in the non-paretic eye in patients who habitually fixate with the paretic eye

 

 

because of a monocular sensorial adaptation to the cyclodeviation. Sensory

 

 

adaptations (harmonious anomalous retinal correspondence (HARC) or

 

 

sensory cyclofusion) and motor cyclofusion (Phillips & Hunter 1999) may

 

 

explain why some patients with congenital superior oblique palsies have

 

 

minimal subjective torsion with the double Maddox rod test (Plager 1999).

 

 

Originally, it was recommended that a red Maddox rod should be placed

 

 

in front of the right eye and a white one in front of the left eye (von Noorden

 

 

1996, p 190). Theoretically, the eye with the underaction would be the one

 

 

whose image was cyclorotated, although von Noorden (1996, p 190) noted

 

 

that exceptions to this rule were common. Simons et al (1994) explained

 

 

these exceptions with an experiment demonstrating that a white rod was

 

 

less disruptive to vision than a red rod. They recommended that two red

 

 

rods be used, in which case the paretic eye is correctly diagnosed in 94% of

 

 

cases. A comparison of five methods of measuring ocular torsional move-

 

 

ments found that the double Maddox rod was reliable when used in the

 

 

primary position (Capdepon et al 1994), although Kraft et al (1993) found

 

 

that the test was best at discriminating superior oblique palsies (normal/

 

 

single/double pareses) in down-gaze.

 

 

A pair of Bagolini lenses can be used, with axes parallel, in an analogous

 

 

way to the double Maddox rod test (von Noorden 1996). When Bagolini

 

 

lenses are used the eyes are not dissociated, so the test is unlikely to work

 

 

in strabismic cases where there is suppression and will be confounded in

 

 

strabismic cases where there is HARC. The result with this test may be simi-

 

 

lar to an assessment of the cyclodeviation with the Mallett fixation disparity

 

 

test, which might be expected to have the same shortcomings with strabis-

 

 

mic patients.

 

 

The Maddox wing test can be used to measure cyclodeviations, but only

 

 

a fairly limited range of cyclodeviations can be measured. It is important

 

 

to keep the patient’s head and the instrument level.

 

 

Rabbetts (1972) described a prototype instrument, the cyclophorometer,

 

 

that used polarized filters to dissociate the eyes. A similar principle is used

 

 

in the commercially available torsionometer, in which the patient views a red

 

 

and a green line on card through red/green goggles (Georgievski & Kowal

 

 

1996). One of the lines is rotated until they are parallel, and the degree of

287

 

torsion is recorded from the required rotation of the line. The test is less

 

 

17

 

PICKWELL’S BINOCULAR VISION ANOMALIES

 

 

 

 

dissociating than the double Maddox rod test but probably more dissociat-

 

 

 

 

ing than the Bagolini lens test. Even for patients with diplopia, where the

 

 

 

 

eyes are effectively already dissociated, there is some variation (more than 5°

 

 

 

 

in 10% of patients) between different methods of measuring cyclodeviations

 

 

 

 

(double Maddox rod, Maddox wing, synoptophore, torsionometer), although

 

 

 

 

no particular test is responsible for the variation (Georgievski & Kowal

 

 

 

 

1996).

 

 

 

 

Screen tests

 

 

 

 

Alternatives to the Hess screen, the Foster and Lancaster screens, employ

 

 

 

 

similar principles to those described for the Hess screen below. These meth-

 

 

 

 

ods provide the most thorough way of recording the degree of incomitancy

 

 

 

 

and other information that will help the assessment of the progress of the

 

 

 

 

condition. During all of these tests it is essential that the patient’s head

 

 

 

 

does not move.

 

 

 

 

Screen tests are essentially dissociation tests that are carried out in dif-

 

 

 

 

ferent positions of gaze. The deviation is plotted in space, and chart plots

 

 

 

 

can be used to give a precise measure of the deviation in different pos-

 

 

 

 

itions of gaze. Another important feature of the test is that it is carried out

 

 

 

 

first with one eye fixating and then the plot is repeated with the other eye

 

 

 

 

fixating. This is to differentiate between the primary and secondary devi-

 

 

 

 

ation (p 278).

 

 

 

 

Hess screen

 

 

 

 

Modern Hess screens are grey in colour, so that the light from two projector

 

 

 

 

torches can be seen on the screen. The patient sits at a distance of 50 cm.

 

 

 

 

The screen is divided into ‘squares’ representing 5° rotations of the eyes. As

 

 

 

 

the screen is flat, tangential to the line of sight, the squares are distorted

 

 

 

 

into a pin-cushion pattern. The practitioner holds the red torch and the

 

 

 

 

patient wears red–green diplopia goggles. On the screen, a red bar image

 

 

 

 

from this torch can be seen only by the eye with the red goggle before it.

 

 

 

 

Thus, when the patient is asked to look at the red bar image on the screen,

 

 

 

 

the eye will be positioned so that the red image falls on the fovea of that eye.

 

 

 

 

The patient holds the green torch and is asked to shine its bar image on the

 

 

 

 

screen so that it appears to cross the red bar. This subjective cross will be

 

 

 

 

formed when the green bar of light is in such a position that its image falls

 

 

 

 

on the fovea, as the foveae in each eye are corresponding points and have

 

 

 

 

the same visual direction. Therefore, the positions of the bar images on the

 

 

 

 

screen mark the points of intersection of the visual axes with the screen.

 

 

 

 

The degree of any deviation can be estimated from the 5° marking. The gog-

 

 

 

 

gles are changed round so that the red goggle is in front of the other eye,

 

 

 

 

so that the plot is repeated with this eye fixating and the first one deviated.

 

 

 

 

A copy of the plot is made on a paper chart with each eye fixating in turn.

 

 

288

 

Some versions are internally illuminated, so that an LED is illuminated

 

 

 

in the appropriate position (Fig. 17.7).

INCOMITANT DEVIATIONS

17

 

 

 

 

 

A

B

Figure 17.7 The Hess screen test. (A) The conventional test. (B) The computerized City University Hess screen test.

289