CHAPTER 10 Introduction to Strabismus and the Ocular-Motor Examination

EVALUATION OF THE STRABISMIC PATIENT The goals of the strabismus examination are to: (1) establish a cause for the strabismus, (e.g., pseudo-esotropia, congenital esotropia, cranial nerve palsy, or restrictive strabismus), (2) assess the binocular sensory status, (3) measure the deviation, and (4) diagnose amblyopia. A well-focused goal-oriented evaluation prevents laborious exams that result in patient fatigue and the collection of spurious data. Even after a full evaluation, a patients strabismus may not be clearly fall into a specific catogory, and the diagnosis may be nebolous. In these cases, the patient can still be appropriately manage if evaluated for amblyopia, sensory status, size of the deviation, and the possibility of an underlying neurological problem or systemic disease.

HISTORY As in all aspects of medicine, the patients history provides the foundation for making correct diagnosis. The onset and character of the strabismus are important. Is the strabismus constant or intermittent, and has there been an associated face turn or head tilt? A history of an intermittent strabismus or compensatory head posturing speaks for the prescence of binocular fusion, whereas constant strabismus indicates a lack of fusion. The onset of the strabismus can provide information about the patients fusion potential. Patients with straight eye during the first several months of life will have developed at least some binocular vision and are likely to have fusion potential. In contrast, children with incorrected congenital strabismus who have never experienced fusion have a poor fusion prognosis if corrected after 2 years of age. The history of acquired strabismus may also be an indication of acquired neurologic or systemic disease, especially when the strabismus is acquired, incomitant, and associated with limited ductions. An unexplained acquired incomitant strabismus should be a red flag to the clinician and requires neurologic evaluation. Documenting the onset and type of strabismus can be facilitated by examining baby photographs, the family album, or a patients drivers license under magnification. Additionaly, patients should be questioned about the presence of diplopia, as diplopia usually indicates acquired strabismus with onset after 5 to 7 years of age.

History regarding birth weight, complications of birth, the health of the child, and the developmental milestones are also an integral part of complete evaluation. Finally the family history is important because even though the exact hereditary pattern of strabismus is unclear, many types of strabismus are familial.

PHYSICAL EXAMINATION Try to obtain as much information as possible by inspection, without touching the child. Use toys to play with the child in order to observe the eye alignment and the eye movement. Save the more intrusive parts of the examination for last. The steps for the strabismic examination are listed in order in the box on page 140. Traditionaly, binocular sensory testing is performed before tests that require accluding one eye, such as visual aculty testing and the cover-uncover tests.covering one eye dissociates the eye, and may disrupt fusion in a patients with latent strabismus (large phoria or intermittent tropia). This theoretical consideration may not be of practical concern, since Biedner et al2 found no significant difference in stereopsis tested at the beginningversus the end of the examination. In the children able to cooperate with optotype acuity testing, the arthor prefers to test vision early, before sensory testing, because knowledge of the visual acuity sets the tone and expectations for the rest of the examination.

INSPECTION The physical examination actually starts as the patient enters the room. While talking the history, it is important to observe the patients visual behaviour, eye movements, fixation, alignment, and head posturing. By the time the history is done, a good observer will have established a presumptive diagnosis. An initial differential diagnosis helps guide the direction of the physical examination, minimizing extraneous tests.

VISUAL ACUITY Techniques for measuring visual acuity are covered in Chapter 3: however, points that directly relate to strabismus are covered here. Patients with strabismus often have latent nystagmus. Covering one eye often increase nystagmus and diminishes visual acuity. In patients with latent nystagmus, it is preferable to blur one eye with a plus lens rather than occlude one eye to achieve monocular visual acuity, because blurring of the one eye causes less nystagmus than occlusion. The plus lens should be the minimum amount necessary to force fixation to the fellow

eye. Test which eye is fixing by placing a vertical prism over one eye (i.e., vertical prism test). Usually a +5.00 lens is sufficient to blurr one eye enough to force fixationto the fellow eye. Linear presentation of optotypes tend to run together, so try a single optotype presentation. Also take a binocular visual acuity measurement in addition to a monocular acuity in patients with nystagmus because binocular vision is usually better than monocular vision. To assess the best functional visual acuity potential in a patient with nystagmus, test binocular vision while allowing the patient to adept thier preffered face turn or head tilt. When evaluating amblyopia, linear acuity is desirable because single optotype presentation underestimates the degree of amblyopia. There are many ways to test visual acuity in preschool children including, Allen picture cards, HOTV, and illiterate E game. In young children, linear Allen figures are usefull assessing linear visual acuity and provide a way to compare the visual function of the two eyes. Fixation Testing for Amblyopia Preverbal children can be tested for amblyopia by examinning the quality of monocular fixation of binocular fixation preference. MONOCULAR FIXATION TESTING Normal children over 2 months of age should show central fixation with accurate smooth pursuit and saccadic refixiation eye movements. Test for central fixation by covering one of the patients eyes, then move a small toy (1 to 2 cm) slowly back and forth in front of the child to observe the accuracy of fixation. A child with central fixation looks directly at the target, visually locks on the target, and accuretly follows the moving target. Infatns often find the human face a much more compelling target than toys so the examiner often finds the infant fixed on the examiner rather than the intended fixation target.in this case move your head side-to-side to evaluate the quitly of fixation and following. Central fixation indicates foveal vision usually in the range of 20/80 or better. Eccemtric fixation, on the other hand, means the fovea is not fixating and the patient is viewing from an extrafoveal part of the retina. Eccentric fixation is a sign of poor vision and dense amblyopia, usually 20/200 or worse. Patients with eccentric fixation do not have steady fixation and they appear to be not working directly at the fixation target.

VISUSCOPE One way to identify the eccentric fixation point in older cooperative children is to use visuscope, which is a type of direct ophtamolmoscope that projects a focused image onto the retina so the examiner can see the image on the retina. First the image is projected onto parafovual retina then the patient is asked to look at the image. If the patient has central fixation the patient refixates to the place the image precisely on the fuvea. However, if the patient has eccentric fixation he will view with parafoveal retina and show wandering steady fixation. The more peripheral the eccentric fixation, the denser the amblyopia. Patients with eccentric fixation do not have specific point og fixation but view from a relatively large area of parafuveal retina. BINOCULAR FIXATION TESTING Standard Fixation Preference Testing. Standard fixation preference testing is useful in preverbal strabismic children for indentifiying amblyopia that might be missed by monocular fixation testing. This test is based on the premise that strong fixation preference indicated amblyopia. If a patient with strabismus spontaneously alternates fixation, using one eye then the other, this indicates equal fixation preference and no amblyopia (figure. 10-1). In contrast, strong fixation preference for one eye indicates amblyopia. The stronger the fixation preference, the worse the amblyopia. In patients with a fixation preference, the degree of fixation preference can be estimated by briefly covering the preferred eye to force fixation to the nonpreferred eye. Remove the cover from the preferred eye then observe how well and how long the patient maintains fixation with the nonpreferred eye before refixating back to the preferred eye. If fixation immediately goes back to the preferred eye after the cover is removed, this indicates strong fixation prefence for the preferred eye and amblyopiaof the deviated eye (fig. 10-2 A). If the patient maintains fixation with the non preferred eye through smooth pursuit , through a blink or for atleast 5 seconds, there is mild fixation preference and no significant amblyopia (vision within two Snelles lines difference) (Fig.10-2 B). The ability to maintain fixation with the nonpreferred eye while following a moving target is a very reliable indicator for equal vision and no significant amblyopia. The reliability of fixation preference testing has been shown to be quite good in patients with large angle stabismus over 10 to 15 PD, accurately diagnosing amblyopia of two Snelles lines or more in voer 90% of cases.12 Patients with small angle strabismus, however, show strong fixation preference in 50% to 70% of cases, even if the vision is equal to within two Snellen lines difference. This high overdiagnosis rate in children with small angle strabismus is because they have the monofixation syndrome. These patients have peripheral

fusion and they fixate with one eye. The overdiagnosis of amblyopia in patients with small angle strabismus can be rectified by using the vertical prism test, which dissociates peripheral fusion and temporarily breaks down the monofixation syndrom (see Vertical Prism Test below).

Vertical Prism Test. Standard fixation preference testing as described in the previous section is very reliable dor diagnosing amblyopia in the patients with large angle strabismus, but overdiagnoses amblyopia in children with small angle strabismus (<10 PD), and cannot be used in patients with straight eyes. The vertical prism test, or 10 diopter orism test, allows fixation preference testing to accurately diagnose amblyopia in patients with small angle strabismus or straight eyes.10,11 The test is performed by placing a 10 to 15 PD prism base-up or base-down in front of one eye, thereby inducing a vertical strabismus. A 15 prism diopter (PD) base-down prism placed of the fixing eye causes both eyes to elevate. Thus if the base-down prism is placed over the right eye and there is a fixation movement upward, then the right eyei is fixing. If there is no movement of either eye when the prism is placed over the right eye, then the left eye is fixing (the eye without the prism). With the induced vertical strabismus, fixation preference can be determined (Fig. 10-3). Additionally, the vertical prism dissociates the eyes, which temporarily breaks peripheral fusion in patients with monofixation syndrome. This eliminates the over-diagnosis of amblyopia associated with standard fixation testing in patients with small angle strabismus, and accuracy with this procedure becomes approximately 90%.10,11 Crossfixation. Crossfixation is when there is a large esotropia and the patient fixes with the adducted eye with a face turn. The right adducted eyes fixes on objects in the left gaze, and the left adducted eye fixates on the objects in the right gaze (Fig. 10-4). Crossfixation in patients with large angle esotropia has been said to be a sign of euqal vision.A study by Dickey4 showed that some patients with crossfixation will have mild amblyopia and that crossfixation does not guarantee equal vision. The ability to hold fixation past midline or hold fixation through smooth pursuit with either eye is a better criterion for equal vision.

DUCTIONS AND VERSIONS Ductions test single eye movements and are examined with one eye occluded, while versions test binocular eye movements and show how well the eyes move together in synchrony. Figure 10-5 shows normal and limited abduction.

Both horizontal and vertical ductions are quantitated with a graded 0 to minus 4 scale, with minus one limitation meaning slight limitation and minus four indicationgsevere limitation with the inability of the eye to move past midline. Evaluating of versions should include eye movements through all 9 cardinal position of gaze. Abnormal versions can be noted on a scale of +4 through 0 to -4 with 0 indicating normal and +4 indicating maximum overaction (Fig. 10-6), while -4 indicates severe underaction (Fig. 10-7). It is important to remember that, when observing for oblique dysfunction, one makes sure that the abducting eye is fixing so the adducting eye is free tomanifest the oblique dysfunction. This can be accomplished by partially occluding the adducting eye (with your thumb or occluder) and looking around the occluder to see if the eye manifests the oblique dysfunction (Fig. 10-8). MEASURING OCULAR DEVIATION The mothods for measuring the angle of strabismus have been devided into the following categories: light reflex tests and cover tests. Light reflex tests are the easiest to perform on young children and infants. These tests, however, are not as precise as the other tests, such as the cover test. The Lancaster red-green test is useful in adult patients with diplopia and incomitant deviations. The amblyoscope and Lancaster red-green test are described in chapter 11. Most methods for measuring ocular deviations involve prisms, and there is a basic discussion on the use of prisms in strabismus in chapter 8. For prism neutralization of a deviation, remember to orient the prism so the apex points in the same direction of the deviated eye. Esotropia is corredted with a base-out prism, exotropia with a base-in prism, and hypertropia is by a base-down prism. When measuring any deviation, it is critical to keep the patients attention and be sure the patient is appropriately accommodating on an accommodative fixation target. Accurate measurements cannot be obtained if the patient is gazing around the room or day dreaming and not accommodating on the fixation target. An accommocative target is one that has fine detail, which requires accurate accommodation in order to be seen. A pen light, for example, is the antithesis of an accommodative target as there is no fine detail, and accommodation is not requiredto see the light. One of the best accommodative target for adults, in the distance or near, is Snellen letters at a size close to visual threshold. By having the patient read the letters, the examiner knows that the patient is accommodating on the fixation target. For young children small detailed toys or small pictures with fine detial can be used at near and a cartoon movie or animated toys in the distance.