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Peter G. Swann BSc (Hons), MAppSc, FCOptom, FAAO

Some aspects of paralytic strabismus


A paralytic, or incomitant strabismus is said to exist when the angle of the deviation varies with the direction of gaze. When the normal eye is fixing, the degree of strabismus shown by the other eye is known as the primary deviation. When the abnormal eye is fixing, the angle of squint is greater, and this is called the secondary deviation. The terms paralysis, paresis and palsy are often interchanged. Strictly, the word paralysis applies when the problem is complete or total, whereas paresis and palsy apply to an incomplete or partial paralysis.
Often, there is not a great deal the optometrist can do to treat these difficulties. In this authors opinion, our primary responsibility is to recognise cases of recent onset that are urgent and require expedient referral, as they may have a highly significant and potentially life-threatening neurological aetiology. include trauma, vascular disease such as diabetes or hypertension, tumours, raised intracranial pressure and infections. Many though can be termed idiopathic, where a cause is never determined. Probably 20-30% of cases fall into this category2. One condition that must always be kept in mind is myasthenia gravis, particularly where a ptosis is present and the signs and symptoms become worse as the day proceeds2. Eye movements may also be mechanically restricted or limited due to muscle fibrosis or entrapment3. Some examples include Browns syndrome, blowout fractures of the orbit and thyroid disease. superior rectus, the inferior rectus, the inferior oblique (isolated pareses of these muscles therefore being very rare), the levator palpebrae, the ciliary muscle and the iris sphincter muscle. If there is a vertical diplopia worse on looking to the right, then the muscle/s involved could be the right superior rectus, right inferior rectus, left superior oblique or left inferior oblique. Again, the likely problem is with the superior oblique as the fourth cranial nerve only innervates that muscle. 4. Is the diplopia worse at distance or near? If there is a horizontal diplopia worse on looking to the right and straight ahead, then the right lateral rectus is probably implicated. If a vertical diplopia is worse on looking to the right and worse at near, then the left superior oblique is the likely culprit.

Muscle actions
The actions of the horizontal rectus muscles are easy to understand, whereas the actions of the vertical rectus and oblique muscles can be more difficult. Our understanding can be helped by remembering one simple anatomical fact the line of pull of the vertically acting muscles lies medial to the eyes centre of rotation1. We must consider the field of action of the muscle. This can be defined as the direction in which the muscles line of pull has the greatest mechanical advantage. Clearly, the lateral rectus field of action will be abduction and that of the medial rectus, adduction. When considering the vertically acting muscles, the superior obliques greatest mechanical advantage, for example, will be seen as the eye is adducted towards the nose, when the muscles role as a depressor is increasingly felt. Thus the field of action of the superior oblique will be down and in. Similarly, with the superior rectus, its greatest mechanical advantage as an elevator will be when the eye is abducted. Therefore its field of action is up and out. From the point of view of clinical diagnosis, we can regard the inferior oblique and inferior rectus as lying in the same vertical plane as the superior oblique and superior rectus1. If, therefore, we wish to look down and to the left, the right eye will be in the field of action of the right superior oblique and the left eye in the field of action of the left inferior rectus. Hence, these are known as yoke muscles. Putting it another way, one is the contralateral synergist of the other. There are yoke muscles for every direction of gaze.

Investigation
Most cases of paralytic strabismus can be satisfactorily diagnosed by the optometrist simply conducting a comprehensive, primary care eye examination. There should be a careful case history followed by a full assessment of ocular health, together with binocular vision tests such as cover test and ocular motility. As recent cases usually have an abrupt onset with the symptom of diplopia that is often distressing, it is worth considering this symptom in greater detail.

Abnormal head posture


Patients assume abnormal head postures to aid in the elimination of diplopia. They consist of turns, tilts, elevations or depressions of the chin or combinations of all three. However, it must always be remembered that other conditions or situations can cause abnormal head postures. Examples include anomalies of neck muscles, (torticollis), nystagmus, visual field restrictions, unilateral deafness, shyness or they may be simply habitual. Old photographs can be very useful here.

Diplopia
When a patient complains of diplopia, some questions should be asked as the answers can give an excellent guide to the correct diagnosis. 1. Is the diplopia monocular or binocular? Cover one eye then the other to find out. 2. Is the diplopia horizontal or vertical, or if it is a combination of the two, which is the greater element? If the diplopia is horizontal, then the difficulty is usually with the lateral or medial recti. Similarly, if it is vertical, then the vertically acting muscle/s will be involved. 3. Is the diplopia worse in certain directions of gaze? For example, if there is a horizontal diplopia that is worse on looking to the right, then the right lateral rectus or left medial rectus is probably at fault. Considering isolated muscle pareses, the problem is most likely to be with the lateral rectus, as the abducens nerve only innervates that muscle. The medial rectus is innervated by the third cranial nerve, which also travels to other structures such as the

Conditions
Clearly, there are many conditions that can lead to incomitant deviations of the eyes. Some that the optometrist may see from time to time include paresis of the third, fourth and sixth cranial nerves, Duanes syndrome and Browns syndrome.

Third nerve paresis


This condition is often caused by vascular disease, neoplasia (including aneurysm), trauma and ophthalmoplegic migraine. The eye is diverged because of the unopposed lateral rectus and to a lesser extent, the superior oblique. The pupil is often dilated and fixed and there may be a profound ptosis. Should the patient present with a suddenly occurring, painful, third nerve paresis, consider an aneurysm, usually of the

Aetiology
Paralytic strabismus may be congenital, where there is a problem with the development of the oculomotor system, or acquired, where injury or disease compromises that system. These problems

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move from the side of the patient, that is in profile, to avoid this mistake. A six-year-old boy was referred by his optometrist because of a right sixth nerve paresis (Figure 2). A brain stem lesion was discovered, but unfortunately the patient was lost to follow up.

Fourth nerve paresis


Figure 1 A right third nerve paresis. The right eye is diverged with a dilated fixed pupil and ptosis. Figure 2 A right sixth nerve paresis. The right eye does not abduct appropriately on right gaze.

Figure 3 A right fourth nerve paresis following severe head trauma. The right eye cannot move satisfactorily into the field of action of the right superior oblique, that is, down and in.

Figure 4 On left gaze, there is an overaction of the ipsilateral antagonist, the right inferior oblique.

This condition is frequently found on routine eye examination. The fourth nerve is interesting for several reasons. It is the only one to emerge from the dorsal aspect of the brain stem, it is the only cranial nerve to completely decussate and it is the longest and most slender of the cranial nerves5. Trauma is a common cause of an acquired fourth nerve paresis, especially if it is bilateral. The diplopia is usually vertical or oblique, and worse at near with activities such as reading, eating and going up and down stairs. An overaction of the ipsilateral antagonist, the inferior oblique, is usually evident. The patient often tilts their head away from the affected side, and any hypertropia may be made worse by tilting the head towards the affected side (the head tilt test). A 20-year-old white female was knocked out when she fell from her horse. On regaining consciousness she reported a vertical diplopia on left gaze and when reading. It was minimised by tilting her head to her left shoulder. She had a right fourth nerve paresis (Figures 3 and 4) and was referred for surgical management which was successful.

Duanes syndrome
There are several types of Duanes syndrome, the most common one showing a marked limitation of abduction (mimicking a sixth nerve paresis), some limitation of adduction, and a globe retraction and palpebral fissure narrowing on adduction. There may be a head turn to the affected side. It is more often seen in the left eye and in females. Again, movement of the eyes should be watched in profile, where retraction of the globe and narrowing of the palpebral fissure may be more easily appreciated. This helps to avoid confusion with a sixth nerve weakness. A young adult white female had always had a face turn to her left and an inability to abduct her left eye (Figure 5). There was also a limitation of adduction together with retraction of the left eye and narrowing of the palpebral fissure on adduction (Figure 6). Her Duanes syndrome was not causing any difficulties and she was advised to report for annual review.

Figure 5 Duanes syndrome in the left eye with a marked loss of abduction.

Figure 6 On right gaze there is some limitation of adduction together with a narrowing of the palpebral aperture and globe retraction.

posterior communicating artery of the Circle of Willis. Classically, if the pupil is dilated, then it is likely to be an aneurysm, (the so called surgical third), and if the pupil is spared, then the cause is probably ischaemia as in diabetes and hypertension, (the medical third). However, the optometrist should treat them all in the same way, whether the pupil is dilated or not, and refer the patient as an emergency to their own doctor or to a hospital. A 65-year-old white male had a right third nerve palsy (Figure 1) following neurosurgery for an aneurysm many years ago. He also had considerable weakness down the left side of his body. As the third nerve palsy was so longstanding and was not causing him any problems, he was not referred.

Sixth nerve paresis


The sixth nerve is particularly vulnerable to assault on its way to the lateral rectus by such problems as vascular disease, tumours, trauma and raised intracranial pressure. An instrument delivery is a potential cause in congenital cases. In acoustic neuroma, the first symptom is hearing loss and the first sign is a depressed corneal reflex4. The sixth nerve may also be involved, and therefore it is essential for all patients with a sixth nerve paresis to have hearing and corneal sensation tested4. Typically, there is a horizontal diplopia, worse on looking into the field of action of the lateral rectus, and a head turn to the affected side. It may be confused with Duanes syndrome, and the optometrist should always watch the eyes

Browns syndrome
Browns syndrome is usually congenital but can be acquired and represents an anomaly

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Figure 7 Abnormal head posture in Browns syndrome. The chin is elevated and the head tilted and turned to the left shoulder.

Figure 8 With the head in the normal position, there is a right hypotropia.

of the superior oblique tendon/trochlear complex6. There is an absence of elevation in adduction of the affected eye, which usually progressively lessens with abduction. It simulates a paresis of the inferior oblique. Other potential features include a downshoot of the affected eye in adduction, hypotropia of the affected eye in the primary position, a V exotropia pattern in upgaze, positive forced duction test and an abnormal head position. The latter includes a head turn to move the affected eye into abduction combined with elevation of the chin for binocularity. The condition can spontaneously improve as the child gets older, but severe cases with markedly abnormal head postures require surgery. A 10-year-old white female was referred to our clinic by an optometrist for evaluation of an anomalous head posture (Figure 7) and unusual eye movements. With her head in the normal position, she showed a right hypotropia (Figure 8) and she could not elevate her right eye in adduction (Figure 9). She was referred to an ophthalmologist for consideration for surgical management.

gaze, it is best to select with the patient an activity where the diplopia is causing considerable difficulties, such as reading, and try to prescribe prisms for that particular task.

References
1. Pickwell, L.D. (1981) Incomitant deviations. Parts 1-5. Optician, March to July. 2. Larkin, G., Elston, J., Bain, P.G. (1995) Disorders of ocular motility. Br. J. Optom. Disp. 3: 5-11. 3. Hosking, S. (1998) Binocular vision. Incomitant strabismus back to basics. Optician 216 (5672): 16-22. 4. Kanski, J.J. (1994) Clinical Ophthalmology. 3rd ed. Butterworth Heinemann, Oxford. 5. Wilson-Pauwels, L., Akesson, E.J. and Stewart, P.A. (1988) Cranial Nerves. B.C. Decker, Toronto. 6. Helveston, E.M. (1993) Browns syndrome: anatomic considerations and pathophysiology. Am. Orthoptic J. 43: 31-35. 7. Taylor, D. (1990) Pediatric Ophthalmology. Blackwells, London.

Treatment
Optometric management of incomitant deviations is difficult and often unrewarding. The patient should wear their best prescription although this is unlikely to alter the parameters of the strabismus1. Temporary patching may be entertained when the diplopia is distressing. In symptomatic cases, surgery may be the best hope of correction. Prisms can be tried, and are usually easier to use in cases involving the horizontal rectus muscles. As the deviation, and therefore the degree of diplopia, varies in different directions of

Acknowledgement
Figure 1 is reprinted with the permission of Clinical and Experimental Optometry 1999; 82: 43-46.

About the author


Peter Swann is Associate Professor in the School of Optometry, Queensland University of Technology, Brisbane, Australia.

Figure 9 There is a complete loss of ability to elevate the right eye in adduction.

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