Chapter 13
Concomitant Exodeviations
PAUL R. MITCHELL and MARSHALL M. PARKS
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ETIOLOGY
CLINICAL CHARACTERISTICS EXOPHORIA
OBJECTIVE SIGNS
MANAGEMENT
REFERENCES

Visual axes that form a divergent angle as they proceed outward constitute an exodeviation. Diverged visual axes kept latent by single binocular vision constitute exophoria; when manifest, the misaligned visual axes constitute exotropia. Many patients with divergent deviations vacillate between phoria and tropia; this fluctuating condition is termed intermittent exotropia.
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ETIOLOGY
A disturbance in the tonic horizontal vergence is usually considered the cause of most primary divergent deviations. However, given that so little is known about the total spectrum of vergence innervations that disjugately drive the eyes, other than accommodative convergence and fusional vergence, such speculation is rather meaningless. There appears to be some disruption in the usual balance of tonic vergence innervations; this results in either a deficiency of convergence innervation or an excess of divergence innervation. Whatever the cause of such innervational disruption, it seems to be genetically influenced; hereditary aspects of the primary divergent deviation are inescapably obvious to the ophthalmologist.

Secondary anatomic factors probably occur within and surrounding the extraocular muscles; they account for a gradual increase in the divergent angle in both intermittently and constantly exotropic patients. Excess innervation to the lateral rectus muscles possibly results in their hypertrophy. In addition, in long-standing constant exotropia, the elastic fibers surrounding the fascial and conjunctival tissues may add their force to the divergent pull of the visual axes, further increasing the exotropic angle. Therefore, it may be difficult to determine which part of the cause is primarily innervational and which is secondarily anatomic. The interplay of innervational and anatomic forces is displayed in a patient who sustains a traumatic unilateral cataract. Although the eyes were orthophoric before injury, within a few weeks following disruption of binocular vision by the acquired lens opacity, the injured eye usually begins to diverge in adults and in children older than 5 years of age. In younger children, the injured eye usually converges. Over the years, the divergent injured eye becomes increasingly exotropic, and eventually the traction test reveals resistance to passive adduction and shortening of the temporal conjunctiva and Tenon's capsule extending between the temporal limbus and the outer canthus. Apparently, when ungoverned by binocular vision, tonic divergence prevails over tonic convergence, and over time the original innervational alteration is secondarily reinforced by anatomic changes in the extraocular muscles and surrounding tissues.

Anatomic causes of exotropia may be the primary factor in, rather than the consequence of, prior innervational imbalance to the extraocular muscle. Surgical overcorrection of esotropia,1 trauma to the orbit causing either a palsy of the medial rectus or an adhesive cicatricial pull on the temporal aspect of the globe,2 and osteologic disorders of the face and cranium (e.g., the shallow diverged orbits in Crouzon's disease) are examples of anatomic abnormalities that produce exotropia.

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CLINICAL CHARACTERISTICS EXOPHORIA
Small amounts of exophoria in adults and even relatively large amounts in youngsters are usually asymptomatic. Discomfort or visual disturbances are the usual symptoms. The discomfort is manifest by vague asthenopia, which gradually increases during prolonged detailed visual tasks and dissipates with rest. The visual symptoms are momentary diplopia, vacillating with momentary blurred vision. Symptoms occur when the fatigue threshold of fusional convergence is exceeded. As the patient finds it more difficult to maintain fusional convergence, accommodative convergence may be substituted, which is necessarily associated with accommodation. As the accommodation relaxes, allowing the vision to clear, the accommodative convergence, which was substituted for the exhausted fusional convergence, also decreases. The latent divergent deviation becomes manifest, and while it persists, heteronymous diplopia is present. The response to the diplopia stimulus is again to apply the synkinetic near reflex, namely, overaccommodation and convergence, thus blurring the vision and eliminating the diplopia. Repeating this cycle creates the symptoms of momentary blurred vision vacillating with momentary diplopia.

The symptoms of exophoria occur for distant visual tasks if the exodeviation angle is significant for distance viewing and insignificant for near, and the reverse is true for the opposite distance-near exodeviation measurements. Convergence insufficiency, manifest by a greater near than distance exodeviation, is more prevalent and accounts for the bulk of symptomatic exophoria. Students in the upper grades of school and adults involved in pursuing prolonged near visual tasks can be troubled by the recurrence of these symptoms. Many exophoric patients can anticipate the onset of their symptoms after having read either for a certain number of minutes or a certain number of pages of text. They usually describe the symptoms of “burning and watering eyes,” followed soon thereafter with the “doubling and blurring” of the print if they attempt to persevere with reading. Most exophoric patients learn to solve the latter symptoms, if they continue reading, by closing or covering one eye. This is followed by a soporific state that they describe as “sleepiness,” and the reading they attempted is interrupted either by sleep or the distraction of daydreaming. Poor health and chronic fatigue hasten the onset of the symptoms after the visual tasks are started. These exophoric symptoms probably play a greater role in subtly determining the patient's vo-ca-tion than the patient is aware; they impose a significant hardship on the patient's genuine intent and desire to complete a competitive academic program.

INTERMITTENT EXOTROPIA

Intermittent exotropia is a divergent deviation, the onset of which is usually during infancy or early childhood. It may be noted as early as the first few months of life. The parents are often convinced of the repeated outward turning of one or both of the infant's eyes by the sixth month. In a study of 1219 infants in a normal newborn nursery, Nixon and coworkers3 found that 22% (268) had intermittent exodeviations, 11% (130) had constant exodeviations, 1.4% (17) had intermittent esodeviations, 2% (23) had variable deviations, 49% (593) had orthophoria, and 15% (188) were not able to be classified. In a study of 2271 neonates from birth to 10 months of age, Sondhi and associates4 found constant deviations in 54%, intermittent deviations in 16%, and orthophoria in 30%. The most common was exodeviation in 67%, and the least common was esodeviation at 1%. By 6 months of age, 97% of the infants were orthophoric; and after 6 months of age, exotropia was rare. In a prospective study,5 the same authors performed 6228 examinations in 4211 infants, finding a predominance of exodeviation early in infancy, with a gradual decrease in prevalence and in the amount of the exodeviation. Congenital esotropia developed in three infants who were exotropic or orthophoric at birth, and large-angle exotropia developed in two infants, both of whom were exotropic at birth, as are many visually normal neonates. The characteristic large deviation developed between 2 and 4 months of life, when most normal infants are developing binocular vision. The relatively low frequency of infantile exotropia was reported in a retrospective study of five pediatric ophthalmic practices.6 Only 13 patients were identified with large-angle, constant exotropia before 1 year of age, without other neurologic or pathologic findings. All required surgical correction, with recession of the lateral rectus of each eye. Two reoperations were required. In some children, however, it may never be noticed until they attain a much later age, perhaps the seventh or eighth year of life. All but a small percentage of the total number of cases are manifest during the early years of childhood. After the intermittent exotropia is manifest, it either remains unchanged or gradually deteriorates to a constant exotropia; only rarely does it undergo partial disappearance. Hiles and colleagues reported on long-term observations, up to 22 years, in a small group of 48 patients with intermittent exotropia.7 Deterioration does not occur in time with all intermittent exotropias; some patients may improve both in the quantitative and qualitative aspects of their exodeviation. In some patients with small degrees of intermittent exotropia, surgery can be deferred unless deterioration occurs.

Some cases have been documented of patients who manifested intermittent exotropia during early childhood and then, without receiving treatment, converted to exophoria in late childhood; they seemed to continue to be asymptomatic during their early adulthood. Possibly, these patients are those who supposedly have the onset of intermittent exotropia in adulthood. Because patients' memories fade as they grow older and because they have extremely varied attitudes toward the visual and cosmetic aspects associated with this disorder, the clinical course of intermittent exotropia is difficult to document in those few patients who apparently improve spontaneously with growth. If one disregarded the patient's statements and ignored the matter of relative frequency of exophoria and exotropia, but plotted the alternate cover measurements of the exodeviation over the patient's lifetime, probably only rarely does the patient show a significant permanent reduction in the exoangle with increasing age. Essentially, the exoangle is primarily a motor innervational disorder established during infancy and kept latent in most afflicted people most of the time by the sensorimotor innervation of binocular vision. The interplay of the motor etiology and the sensorimotor compensation allows for a spectrum of possible clinical manifestations of intermittent exotropia. After the tropic aspect of this divergent deviation occurs, it usually recurs according to a pattern unique to each patient throughout the remainder of life. The pattern of recurrence of the exotropia that varies widely is the principal feature distinguishing one case from another.

The tropic phase of intermittent exotropia is aggravated by many factors. Because binocular vision is the essential compensatory sensorimotor factor, the patient must be visually alert to the environment and willing to make the effort to maintain binocular vision. Daydreaming, visual distraction induced by thinking and speaking, fatigue, illness, and drowsiness on awakening are the usual factors that cause exotropia to replace exophoria. Distance viewing rather than near viewing is frequently more productive of exotropia. In some patients, exposure to bright illumination, such as sunlight, is a striking aggravating factor; when exotropia occurs, this exposure causes sufficient annoyance to precipitate the immediate reflex closure of one eye. The unilateral contraction of the orbicularis may be so obvious that the parent presents the child to the ophthalmologist for this reason and is unaware of a divergent deviation.

Campos and Cipolli8 described a weakening of binocular sensory status and deterioration of fusional amplitude during light exposure in patients with intermittent exotropia. There was no eye closure in their control patients, suggesting that the intermittent eye closure was specific to intermittent exotropia. However, von Noorden9 described monocular eye closure also in patients with constant exotropia, esotropia, and in patients without strabismus.

The tropic phase tends to increase in many affected patients, possibly even in most. The increase is by way of changes in frequency and duration. Instead of occurring only once or twice daily as it originally did, the tropia tends to recur many times per day with increasing age. It also tends to remain for many minutes, instead of the original momentary duration. In some patients, increased recurrence and duration of tropia eventually seem almost to merge into a continuous exotropia. In patients whose sensorimotor compensation for the divergent deviation has deteriorated to this extent, the last stage of their intermittent exotropia is usually characterized by retention of the exophoric phase only for near seeing; they are constantly exotropic for distance viewing. Other patients tend to retain the same frequency and duration of intermittent exotropia for years.

Stathacopoulos and coworkers10 studied stereoacuity in normals and in patients with intermittent exotropia. Satisfactory near stereoacuity was found in normals and in patients with intermittent exotropia, but significantly worse distance stereoacuity was found in intermittent exotropia as compared with that in normal patients. This reduction in distance stereoacuity is valuable as an objective measure of poor control of intermittent exotropia and may assist in determining the timing for surgery. After surgery for intermittent exotropia, O'Neal and colleagues11 reported improved distance stereoacuity in 75% with contour circles, and 45% with the random dot E test, using the Mentor B-VAT II Video acuity tester (Mentor O & O, Inc, Norwell, MA) preoperatively and postoperatively. They suggested distance stereoacuity could be restored with surgical realignment of intermittent exotropia, and that distance stereoacuity provided valuable information in patient evaluation. Yildirim and coworkers12 evaluated the near and distance stereoacuity and central and peripheral fusion in patients with intermittent exotropia, before and after surgery, in a prospective study. Successful outcome was achieved in 69%, with exotropia of 10 prism diopters (PD) or less at distance, at least 1 year after surgery. Peripheral fusion was demonstrated in all patients with intermittent exotropia, but 35% demonstrated central suppression both preoperatively and postoperatively. The preoperative distance stereoacuity significantly diminished when compared that in normal controls but improved 58% postoperatively. Improvement in distance stereoacuity postoperatively was more likely to occur in those patients who achieved successful surgical alignment than in those who failed to achieve successful alignment. They concluded that successful surgery may improve distance stereoacuity, and that those with better preoperative distance stereoacuity and central fusion were likely to achieve higher rates of surgical success. Ball and associates13 found excellent stereoacuity of 40 to 60 seconds of arc, in a series of patients with long-standing horizontal strabismus. They suggested that good vision in both eyes and excellent postoperative alignment can produce high grade stereoacuity in some cases, even though the preoperative measurements would suggest otherwise.

Occasionally, they have exacerbations and remissions of the tropic phase related to their general health, state of rest, and various disturbances that may occur in the daily routine. Finally, a few patients may manifest the tropic phase intermittently for several months to years when they are young and then improve spontaneously for several months to years, becoming constantly exophoric and never exotropic. Some of these patients suffer recurrences of the exotropia when they are older, but probably others do not. Most of these latter patients eventually become lost to follow-up, and their exophoria-exotropia status in later life remains unknown.

EXOTROPIA

The clinical characteristics of constant exotropia are determined by its cause, which may include deteriorated intermittent exotropia or a poor-seeing or blind eye. In congenital constant exotropia, binocular vision has never had an opportunity to develop, and the misaligned eyes constantly manifest abnormal motor innervation to the extraocular muscle. This is a rare disorder compared with congenital esotropia, but we in a study conducted with additional colleagues have documented its existence in more than 25 infants younger than 6 months of age.6,14–16

Congenital esotropia may indirectly become the cause of constant exotropia, inasmuch as the absence of the ability to develop binocular vision is characteristic of congenital esotropes, unless the eyes were straightened by surgery while the patient was still young. The congenital esotrope soon becomes too old to initiate the cortical neurophysiologic pathways required by binocular vision, even though surgery has straightened the eyes. Although the eyes may appear well-aligned for a few years after surgery, increasing exotropia may gradually evolve in those without binocular vision, despite normal visual acuity in each eye. In a series of 88 patients with congenital esotropia, Stager and coworkers17 described delayed consecutive exotropia in 27% (24 of 88) after a 7-mm recession of the medial rectus muscle each eye. In infants who had surgery before they were 7 months of age, the rate was 38% (8 of 21), in infants with surgery at 7 to 12 months, 20% (10 of 49), and at 13 months or older, 33% (6 of 18). Because of the delayed appearance of consecutive exotropia, averaging 26.8 months postoperatively, the authors suggested a prospective study to compare 7-mm recession with three and four muscle surgery, with long-term follow-up observation.

In addition, some congenitally esotropic patients gradually improve or change to exotropia without surgery, a common trend among children with cerebral palsy.18–21

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OBJECTIVE SIGNS
The accommodative convergence to accommodation (AC/A) ratio determines the difference between the exodeviation measured at distance and that at near fixation. Patients with exodeviations that measure similarly at 6 m and 0.33 m while the examiner controls the patient's accommodation have a normal AC/A ratio. Greater deviations at distance or at near are the product of an abnormal AC/A ratio; a high AC/A ratio causes greater exodeviation at distance, and a low AC/A ratio causes greater exodeviation at near. According to Duane's classification, exodeviation greater at distance is divergence excess; exodeviation greater at near is convergence insufficiency.22 Common usage of Duane's classification since 1896 has served the clinician well by classifying exodeviation into three categories. The third category is called basic deviation, an exodeviation with neither divergence excess nor convergence insufficiency, in that the exodeviation is the same for distance and near. Variations in these three categories of deviation are found in all types of exodeviations, regardless of whether they represent constant exophoria, intermittent exotropia, or constant exotropia.

EXOPHORIA

Exophoria is a latent deviation with good fusional vergence amplitude and no amblyopia. Bifixation is maintained despite the exophoria, and normal stereoacuity demonstrates its existence.

INTERMITTENT EXOTROPIA

Intermittent exotropia is also associated with normal fusional vergence amplitudes and no amblyopia. In exophoric patients, the fusional vergence amplitudes are used to maintain bifixation; this is sufficient to prevent amblyopia. The stereoacuity is almost always normal when the patient is exophoric, but stereopsis is not present when the patient is exotropic.

Young patients quickly adapt sensorially to the exotropia phase by developing suppression and anomalous retinal correspondence (ARC), thus freeing themselves of diplopia and visual confusion. The suppression scotoma projecting into space soon enlarges, and the involved temporal retina extends from the hemiretinal line to beyond the locus at which the object of regard is projected as an image. Profound as the sensorial adaptations become, the moment the eyes converge to straight, normal retinal correspondence occurs, with bifixation functioning at a peak level. To gain the advantage of stereopsis, visual alertness and willingness to make the effort to attain this visual reward are required. With inattention or reduction in the desire for maintaining stereoscopic viewing, the effort to keep the eyes straight is terminated, and the moment exotropia ensues, suppression and ARC viewing replace the stereoscopic view. Children 10 years of age and older who have not developed the sensorial adaptations when the eyes diverge are beyond the age at which they can develop them. Hence, they experience persistent heteronymous diplopia while the exotropia prevails. Although the diplopia is annoying, perhaps it is more acceptable than the asthenopia produced by struggling to keep the eyes straight when fatigued. However, these patients experience vexing symptoms while they are exotropic during certain visual circumstances; for example, while the patient is driving, the road seems to split into two roads, and oncoming automobile headlights become diplopic. The intermittent exotropic patient who has instantaneous suppression and ARC whereas a tropic patient is much less annoyed visually than her or his counterpart who lacks these sensorial adaptations.

Measurements of the exodeviation while the distance and near accommodation are controlled are best obtained by the cover test, using prisms to quantitate the exoangle. A muscle light should not be used as a fixating target for either distance or near accommodation because the exotropic patient frequently overaccommodates subconsciously, using the extra accommodative convergence to reduce the exoangle. The blurred image of the muscle light offers no deterrence to this method of compensating for the divergent deviation; however, the images of small symbols that are fixated demand an accurate accommodative response to be identified, allowing the examiner to obtain valid measurements of the total exoangle. The exodeviation may also vary in upgaze and downgaze (A or V pattern) and in right and left gaze from the primary position just as the distance and near measurements may vary. These various gaze positions are recorded for distance viewing, as well as for the straight-ahead near position. An increase in the angle of intermittent exotropia at distance may occur after fixating on an outdoor distant target or after 1 hour of monocular occlusion. Kushner23 evaluated the hypothesis that surgery should be performed when a larger deviation is discovered by one of these methods. His prospective study revealed a satisfactory outcome in 86% (43 of 50) of patients who underwent surgery for the largest angle, compared with only 62.5% (25 of 40) of patients in the control group, who had had surgery for the initial measurement of the exotropia at 6 meters. A satisfactory outcome was declared if the examination occurred at least 1 year after surgery (range, 12 to 15 months), and if the final measurement was between 10 PD of exophoria and 5 PD of esophoria. Kushner concluded that surgery should be performed on the largest exotropia angle measured, both while fixating on a distant outdoor target and after 1 hour of monocular occlusion. Prism adaptation was not investigated in this study.

Versions often reveal inferior or superior oblique muscle dysfunction, and this should always be recorded. Long-standing exodeviations have a relatively high percentage of associated oblique muscle dysfunctions, and because A and V patterns are frequently associated with the oblique muscle dysfunctions, long-standing intermittent exotropic patients have an increased incidence of A and V patterns. Wilson and Parks24 studied retrospectively 456 patients with strabismus. Of 148 patients with intermittent exotropia, 32% had primary overaction of the inferior oblique muscles, when observed for 5 or more years, with an average age at onset of 5.2 years. Dissociated vertical deviation was found in only 3% of the patients with intermittent exotropia. Incidence of inferior oblique overaction was not related to the age at onset of the strabismus, onset to time of surgery, age at surgery, or ocular alignment decompensation.

A high AC/A ratio is more frequently encountered in intermittent exotropia than a low AC/A ratio is, and younger children with intermittent exotropia tend to have a significantly higher AC/A ratio than older children. Exodeviations measuring 20PD to 35PD at distance and 5PD to 10PD at near are common in young children, but the near measurement tends to increase with age, causing the distance and near deviation to approximate each other. Viewing the near accommodative target through + 3.00PD lenses increases the near exodeviation measurement in most patients, regardless of the nature of the AC/A ratio; those with a high AC/A ratio experience the greatest effect. However, the near prism and alternate cover measurements obtained through + 3.00 diopter (D) lenses have not provided more helpful information for management of exodeviation than simply the distance and near accommodation-controlled prism and alternate cover measurements.

Burian and Spivey have a different opinion and use the + 3.00-D lens near prism and alternate cover measurement to determine the muscle for surgical correction of the exodeviation.25 Their reasoning is based on the dual premise that a central nervous system center controls divergence and that divergence innervation flows from this center when looking at distance and ceases when fixating at near. Therefore, excessive divergence innervation causes greater exodeviation at distance than at near. They also assume that the lateral rectus muscles diverge and the medial muscles converge. From this conclusion, they deduce that the diverging muscle should be weakened in patients with an excess of divergence. From these premises they reason that patients with similar distance and near exodeviation measurements possess some unknown factor affecting the extraocular muscles and surrounding fascia, causing the divergence of the visual axes; whatever this factor, the exodeviation does not result from excessive innervational flow from the divergence center to the lateral rectus muscles. They call this basic exodeviation, given that excessive contraction of the lateral rectus muscles is not considered to be the primary cause of basic exodeviation. They advise recession of the lateral rectus muscle and resection of the medial rectus muscle of the same eye, presuming that this surgery produces a similar effect on straightening the diverged visual axes for both distance and near. They also reason that the innervational dispatch of the convergence center to the medial rectus muscles is deficient in patients with convergence insufficiency; they advise resection of the medial rectus muscles for this disorder.

If one subscribes to this line of reasoning, prism and alternate cover near measurements through + 3.00-D lenses can provide some changes that make a particular case difficult to classify. For example, a patient's divergence excess measurements may be converted with + 3.00-D lenses to basic exodeviation measurements; consequently, this patient is reclassified as having pseudodivergence excess. Other patients initially classified as having basic exodeviation are changed by + 3.00-D lenses are reclassified as having convergence insufficiency measurements, and presumably they are also reclassified as having pseudobasic exodeviation. Patients with convergence insufficiency cannot have their initial diagnosis changed by + 3.00-D lenses; the only possible change is an increased exodeviation measurement at near.

Two weaknesses in the reasoning given here create unnecessary confusion. The first is the assumption that a patient with divergence excess, a greater exodeviation at distance than at near, is etiologically unrelated to another patient with basic exodeviation, a similar distance and near exodeviation. It is relatively more appealing to theorize that the distance exodeviation in all patients results solely from disturbance in the balance of opposing tonic horizontal vergence innervations, regardless of whether the near exodeviation is identical to, greater than, or less than the distance exodeviation. Congenital esotropia is theorized to result from the opposite disturbance in tonic horizontal vergences, and no consideration is given to whether the near esotropia is identical or dissimilar to the distance esotropia. The second weakness in this reasoning is the reluctance to use the synkinetic near reflex to explain the differences in the distance-near exodeviation measurements. The AC/A ratio is used to explain differences in distance-near esodeviation measurements and is a well-understood and accepted method used in the diagnosis and treatment of esodeviations. Therefore, we believe it should also be used to explain the differences in distance-near exodeviation measurements: a normal AC/A ratio causes similar distance and near measurements, a high AC/A ratio causes greater distance exodeviation, and a low AC/A ratio causes greater near exodeviation. These two weaknesses are overcome by assuming that the interplay between the tonic vergence disturbance and the AC/A ratio explains all possible distance-near measurements encountered in the exodeviations. Therefore, no case need be classified as “pseudo” because of a near measurement obtained with + 3.00-D spheres.

Children with divergent deviations tend to reveal less exodeviation at near than at distance by the prism and alternate cover test. This suggests that their AC/A ratio is pliable to the extent that it can be adjusted to assist offsetting the exodeviation for the benefit of maintaining single binocular vision. Because infants and young children seem to have a more pliable overall sensorimotor innervating system than older children and adults, this could account for the fact that the high AC/A ratio occurs more frequently among exophoric and intermittently exotropic children. By this reasoning, the divergence excess exodeviation measurement results from an attempt to compensate for excessive divergence innervation rather than from excessive divergence innervation as suggested in the preceding paragraphs.

EXOTROPIA

Exotropia is a constant divergent deviation that is usually associated with either a normal or low AC/A ratio. There is seldom a high AC/A ratio except in the surgically overcorrected esotrope who had a high AC/A ratio before surgery, because no sensory reward exists for the constantly tropic patient for evolving a high AC/A. Unless the fixation continues to be alternated between the two eyes, the patient who becomes constantly exotropic while younger than 10 years of age usually has amblyopia. In constant exotropia, suppression and ARC tend to be present unless there is no binocular vision, as encountered in either the congenital exotrope, some congenital esotropes who become exotropic, or the patient who unfortunately has a unilateral abnormal eye.

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MANAGEMENT
The divergent deviations are treated by several methods: compensating for the misalignment with prisms, urging the patient to compensate for the misalignment with fusional convergence, subtly stimulating the patient to compensate for the misalignment with accommodative convergence, or reducing the misalignment with surgery. The ophthalmologist's selection of treatment depends on the nature of the exodeviation and experience with various treatment modalities.

EXOPHORIA

If exophoria is asymptomatic, it requires no treatment. Symptoms are rare in young children and tend to occur with increasing frequency in patients older than 10 years of age.

Fusional convergence training by orthoptics is successful in most affected patients and should be the first method used to alleviate the patient's recurrent symptoms.26 The objective is to enhance the fusional convergence amplitude by various techniques. Treatment is emphasized for distance if the exodeviation is greater at distance than at near; near exercise is emphasized if the exodeviation is greater at near than at distance. While the patient does the exercise, the ophthalmologist should control the accommodation so that the fusional convergence is stimulated rather than the accommodative convergence. Small, detailed, distance targets are fixated for the distance exercises, and small print is read for the near exercise. Various combinations of loose 2PD, 3PD, 10PD, and 20PD prisms can be arranged horizontally to offer the following stepwise increments in stimulus to fusional convergence: 2PD, 3PD, 5PD, 7PD, 8PD, 10PD, 12PD, 13PD, 15PD, 17PD, 18PD, 20PD, 22PD, 23PD, 25PD, 27PD, 28PD, 30PD, 32PD, 33PD, and 35PD. The loose prism fusional convergence technique used in combination with a Polaroid screen fitted over the television while the patient wears Polaroid spectacle analyzers ensures that the patient is fusing at distance rather than suppressing as the base-out prism increments are made. Bar-reading is an excellent method of ensuring against suppression at near as the increments of base-out prisms are made. Bar-reading is accomplished by holding a narrow septum vertically midway between the eyes and the page; this obscures a different portion of the page for the right and left eye, but the entire page is seen undisturbed as long as the patient fuses.

Various stereoscopic techniques are also available for stimulating fusional convergence. One technique involves using a series of stereocards in a Wheatstone stereoscope; increasing convergence is demanded as the patient progresses through the series. Another method is built around the Polaroid vectographic technique: a series of Polaroid vectographic plates are viewed through Polaroid analyzers. As the patient proceeds through the series of plates, progressively more convergence is required to obtain the perception of stereopsis.

Whenever possible, the ophthalmologist should use accommodative convergence to compensate for exophoria in the subpresbyopic patient by undercorrecting hypermetropia and overcorrecting myopia. Forcing 2- to 3-D accommodation by appropriate spectacle power is usually well tolerated in young patients, but in older patients this therapy may provoke symptoms of asthenopia similar to those it seeks to alleviate, and the benefit is thus nil. In a series of 35 children with intermittent exotropia who wore overcorrecting minus lenses, Caltrider and Jampolsky27 reported a qualitative change, with poorly controlled intermittent exotropia improving to well-controlled exophoria in 46%. A quantitative decrease of at least 15 D and latency of the exodeviation while wearing overcorrecting minus lenses was found in 26%. There were no significant qualitative or quantitative changes in 28%. Minus lens therapy has also been advocated for control of postoperative exodeviations.28 In a series of 37 patients, preoperative diagnoses included 10 patients with esotropia and 27 with exotropia. Before treatment with minus lenses, over 75% had poor eye alignment, with manifest exotropia of 10 D or more, exophoria, or intermittent exotropia of 15 D or more. The over-minus therapy improved results to excellent in more than 75%, with intermittent exotropia of less than 10 D, or a exophoria of 7 to 14 D, classified as satisfactory. After a 2-year follow-up without therapy, 50% had excellent to satisfactory alignment and 33% had no to 6 D of intermittent exotropia. In addition, medical therapy is available to stimulate accommodative convergence. By instilling 2% homatropine eye drops, the AC/A ratio is increased, which provides more convergence to offset the exophoria. However, the symptoms of asthenopia and blurred vision that accompany this therapy regardless of the patient's age make it an impractical method for managing exophoria.

Base-in prism spectacles to compensate for the exophoria continue to have a place in the overall therapeutic regimen. Such spectacles are particularly useful in both presbyopic patients and subpresbyopic patients, regardless of age, who are unable to increase their fusional convergence amplitude with orthoptic training. In this connection, there is an established clinical entity that is ideally treated by base-in prism spectacles. It occurs in young people, is life-long, and causes symptoms during near vision manifested by blurred print and diplopia. Orthophoria or minimal exophoria is measured at distance, but a 12PD to 18PD exotropia is measured at near. Most significantly, the amplitude, or range, of accommodation is conspicuously less than the norm for the chronologic age. In addition, all fusional vergence amplitudes are obviously less than normal or almost nil, and, no matter how seriously the patient tries, these amplitudes cannot be expanded. This nearvision disorder presumably results from some deficiency in the optomotor reflexes. Accommodation, accommodative convergence, and fusional vergences are not there, and there is no way to improve them. Such patients should not receive prolonged orthoptic training. Presbyopic therapy with plus lens power is required for reading, along with base-in prisms to compensate for the near exotropia. The optic combination of these two therapies can be provided in reading spectacles; half-eye spectacles probably offer the best solution.

Surgery is seldom the answer for either distance or near exophoria, and it should be deferred until other methods of treatment have failed, unless the prism and alternate cover distance and near measurements are significant. Patients with either distance or near measurements that approach orthophoria are poor surgical candidates; an adult with approximately distant orthophoria and a symptomatic large angle of near exophoria is a particularly poor surgical risk. For such a patient with a low AC/A ratio, some ophthalmologists claim that resections of the medial rectus muscles improve the near exophoria without altering the preoperative distance orthophoria, but few have documented this claim of permanent improvement in near exophoria without a concomitant disturbing postoperative distance esotropia.

A study by von Noorden recommends resection of the medial rectus in persistent symptomatic convergence insufficiency but cautions that temporary consecutive esotropia occurs, requiring temporary prism control for some time after surgery.29

The exophoric patient with a large distance deviation and approximately near orthophoria, who never slips into intermittent exotropia while viewing at distance, is also almost never troubled with symptoms and rarely requires treatment of any kind, including surgery. The patient with a large angle of exophoria at both distance and at near (i.e., in excess of 12PD) and frequent symptoms does well with surgery, and it is justifiable to offer this treatment.

INTERMITTENT EXOTROPIA

Surgery is justified for intermittent exotropia if anisometropia or myopia has been adequately corrected with spectacles and if an eye continues to turn out intermittently. The patient has the best opportunity for a complete cure with elimination of the exoangle prior to the development of suppression and ARC. If the patient is 10 years of age or older and suppression and ARC are not present while the patient is exotropic, these sensorial adaptations to binocular vision will never develop, and the good prognosis for cure will continue unchanged. Until the patient reaches approximately 10 years of age, there is always the risk that suppression and ARC will be learned while exotropic; after these are learned, they can always be retained. These adaptations tend to be used and reused for any residual exodeviation, no matter how small, after surgery. Eventually, the small exodeviation tends to build back toward the preoperative levels. To prevent this discouraging result, the small residual postoperative exodeviation must be kept latent, as it is in patients without suppression and ARC. Attempts to prevent the suppression and ARC from ruining the immediate postoperative result by employing preoperative and postoperative orthoptics have usually failed.

Cooper and Leyman compared the results of surgical and orthoptic treatment of intermittent exotropia.30 Their 673 patients were divided into four categories of treatment: 1) occlusion only, 2) operation only, 3) orthoptic training and operation, and 4) orthoptic training only. Orthoptic exercises were of two types: antisuppression techniques and convergence training. Most of the group with only orthoptic training had exotropia less than 24PD, whereas most patients in the two surgical groups measured 25PD or more.

The highest percentage of good results, 59%, was in group 4, but this group also had smaller degrees of exotropia. Good results were obtained in 42% of group 2 and 52% of group 3. Cooper and Leyman encourage orthoptic training, when available, for small-angle exotropias and also to enhance the surgical results of exotropias above 25PD.

An opposing view was presented by Moore, who reviewed the orthoptic treatment of intermittent exotropia in 180 patients ranging in age from 3 to 18 years.31 None of these patients had good fusion on initial examination, 48 had fair fusion, and 132 had poor fusion. Treatment results were carefully recorded, and of the 57 patients in the surgery group, 33% were cured and 51% improved with a remaining intermittent deviation, for a total of 84% improved. Of the 106 patients receiving surgery plus orthoptic training, 30% were cured and 42% were improved with a remaining intermittent deviation, for a total improvement of 73%. Moore concluded that orthoptic training did not appear to increase the likelihood of a cure in intermittent exotropia.

Therefore, the most logical therapy for intermittent exotropia is surgical straightening of the eyes as soon as possible after a successful evaluation of the alignment is made and daily recurrence of the exotropia is documented.

In a study of lateral rectus recession, both eyes, for exotropia, Stoller and coworkers32 performed survival analysis, with survival time as the time from surgery to the time of failure after surgery. The estimated mean time to failure was 68 months. Surgical success was reported in 58% of cases, but outcome failures occurred in 42%, if 1) there was a distance exodeviation of more than 10PD at any time after surgery, 2) a distance esodeviation of more than 10PD more than 6 months postoperatively, or 3) further surgery was required for esotropia or exotropia. Successful postoperative alignment was more likely to occur with intermittent exotropia than with constant exotropia. Nonpredictors of success included: age at onset of exotropia, age at time of surgery, angle of deviation, early postoperative alignment, and the presence of amblyopia, anisometropia, incomitance, or other symptoms resulting from the exotropia.

Response to the prism adaptation test in 94 consecutive patients with intermittent exotropia has been reported by Ohtsuki and coworkers.33 Nonresponders included 43 patients whose adaptation to Fresnel prisms applied to both eyeglass lenses was 4PD or less, and the responders comprised 51 patients, whose deviation increased by 5 or more prism diopters. Pertinent findings included an increase in deviation with prism correction in patients whose initial deviation was less than 30PD and in patients younger than 7 years of age. Surgical results did not differ between the two groups at 1 and at 3 years. At 3 years postoperatively, the motor alignment, within 8PD of orthophoria, at 52%, and single binocular vision, at 83%, was higher in the prism-adapted group compared with that in nonresponders at 26% and 57%, respectively.

Pratt-Johnson and colleagues reported on the results of surgery on 100 patients with intermittent divergence-excess exotropia.34 Immediate postoperative results, measured on the first or second postoperative day, were compared with measurements a minimum of 1 year after surgery. Their strict criteria for cure included no tropia at any distance; near stereopsis of 40 seconds of arc; divergence amplitude of at least 5PD on the troposcope, with the immediate recognition of diplopia on exceeding the divergence amplitude; and good convergence amplitude, with total convergence and divergence amplitude exceeding 20PD.

Based on these criteria, 41 patients were cured. Of 59 patients who were not cured, 40 were within 10PD of orthophoria, producing a total of 81% of results that were cosmetically satisfactory.

The relationship between age at the time of surgery and final results was carefully studied. There were 39 patients younger than 4 years of age, with a median age of 2½ years, compared with 69 patients 4 years of age or older, with a median age of 5½ years. Of the patients younger than 4 years of age, 61% were cured, compared with 28% of the 4 years and older group, a statistically significant (p < .001) difference. Although the presence of a small vertical strabismus decreased the chances of obtaining a cure, the most significant factor in obtaining a cure was surgery before 4 years of age.

In a retrospective study of 111 consecutive patients with intermittent exotropia, Richard and Parks35 analyzed three groups, according to age at initial surgery. Group I comprised 41 patients younger than 3 years of age; group II, 42 patients aged 3 to younger than 6 years of age; and group III, 28 patients 6 years to younger than 17 years of age. Satisfactory results were defined as 10 D or less intermittent exotropia at distance, using prism and alternate cover testing. Using this definition, after initial surgery of recession of the lateral rectus of each eye, satisfactory results occurred in 61% of group I, 45% of group II, and 64% of group III. Unsatisfactory results were defined as undercorrection, a residual intermittent exotropia of more than 10 D, or overcorrection, consecutive esotropia. Results in group I were unsatisfactory in 39%; in group II, 55%; and in group III, 36%. Additional surgery was recommended for many patients with unsatisfactory initial surgical results. For those patients with at least a 2-year follow-up from the secondary surgery, a satisfactory result was achieved in 89% of group I, 86% of group II, and 100% of group III.

When combining all three groups, an initial satisfactory result was obtained in 56% of the patients after the initial recession of the lateral rectus of each eye. There were 38% undercorrections and 6% overcorrections. In the undercorrected group, additional surgery was necessary, producing 82% satisfactory results. In the overcorrected group, aggressive medical management, and subsequent recession of the medial rectus of each eye in almost all patients of this group, led to satisfactory results in 87%. Statistical analysis of the data failed to assign any significance to the age of the patient or the time intermittent exotropia was first noted and the time of the initial surgery, or of secondary surgery, and obtaining a satisfactory result. There also was no statistically significant difference demonstrated in obtaining a satisfactory surgical result if age groupings for delay in performing surgery were compared. Postponing the time of surgery did not improve the chance for obtaining a satisfactory result.

Intermittent exotropia causes visual symptoms in patients of all ages, unless suppression and ARC have developed. Elimination of the exoangle by surgery is justified in the absence of suppression and ARC simply on the basis of removing the recurrent symptoms and disregarding the merit of preventing the onset of suppression and ARC. The divergent eye is cosmetically disfiguring and always a source of embarrassment to the patient. Regardless of the patient's age and state of sensorial adaptations to the intermittent exotropia, surgery is justified to eliminate the visual symptoms and unusual appearance with which these patients present. In a series of 44 adults with intermittent exotropia ranging in age from 15 to 70, preoperative symptoms included diplopia, headaches, reading difficulty, ocular fatigue, and pain.36 Because of persistent diplopia with overcorrections, the authors recommended deliberate, slight undercorrections in adults, stating that a small, residual exotropia was more desirable than postoperative esotropia and diplopia. It is difficult to justify allowing these patients to remain untreated and to possibly deteriorate gradually from intermittent exotropia to constant exotropia, although not all ophthalmologists agree with this philosophy. Some claim that inasmuch as not all patients with intermittent exotropia manifest their exoangle as frequently when they are older as when younger, performing surgery on all young intermittent exotropes causes some patients to endure unnecessary surgery. This difference of opinion becomes an impossible argument because within the lifetime of one surgeon, all the facts necessary to support the surgeon's position cannot be accumulated. For example, even if the child improves to the extent that the intermittent exotropia occurs less than daily, it may reappear 20 years later with more frequency. This could be the source of the disorder in the rare patient with late onset who appears to develop intermittent exotropia in adulthood first. However, there is some merit in the argument for not operating on every child with intermittent exotropia, because some do spontaneously improve.7 Those with smaller exoangles are more inclined to improve spontaneously than those with larger angles. For this reason, surgery should be deferred in the intermittent exotropic child whose maximal prism and alternate cover distance measurement is less than 15PD, unless the exoangle increases or the intermittent exotropia increases in frequency and duration.

Part-time occlusion with early-onset unilateral intermittent or constant exotropia was reported in a series of 11 patients.37 The visual acuity was equal, and the nondeviating eye was occluded from 4 to 6 hours daily. All patients converted to heterophoria or orthophoria at least a portion of the time, and three became orthophoric without further patching. Three children eventually required surgery, on the average at 2½ years after onset of occlusion therapy. The technique of patching either postponed surgical intervention or converted exotropia to a long-term exophoria or orthophoria.

However, for all patients, regardless of age, whose distance exoangle can be reliably measured as 15PD or more, whose tropia recurs daily, and whose significant refractive error is compensated by spectacles, the ophthalmologist should give serious consideration to proceeding with surgery. Such surgery usually involves recession of the lateral rectus or recession of the lateral rectus muscle along with resection of the medial rectus muscle of the same eye. As an initial procedure, the improvement provided by resecting the medial rectus is usually not durable because the postoperative alignment gradually reverts to the preoperative status. Recession or resection of a single horizontal rectus muscle is not recommended because it produces asymmetry of lateral gaze and little or no change in the primary position alignment.

Nevertheless, several reports have recommended single lateral rectus muscle recession for small angle intermittent exotropia. Feretis and associates38 described minimal abduction deficiency for unilateral recession up to 12 mm when the intermittent exotropia ranged from 14 to 16 D. Deutsch and coworkers39 treated 30 patients with unilateral lateral rectus muscle recessions of 7.0 or 7.5 mm for exotropia of 15 to 20PD and found a mean effect of 17.6PD, stable to almost 24 months' follow-up. In a subsequent paper, Nelson and associates40 reported on 55 patients, with 15 to 20PD of exotropia, and unilateral lateral rectus recessions of 7.0, 7.5, or 8.0 mm. At their latest follow-up examination, 55 patients (51%) were orthophoric, but 24 patients (44%) were undercorrected, with 22 having residual exodeviations less than 8PD, and 3 patients (5%) were overcorrected. Weakley and Stager41 reported a success rate of 73% after unilateral lateral rectus muscle recessions of 6 to 10 mm with small- to moderate-angle intermittent or constant exotropia. Their success increased to 89% in patients under 4 years of age. One advantage reported was elimination of overcorrections, which can occasionally produce amblyopia or reduction of stereopsis in younger patients. Olitsky42 prospectively compared early (1-week) and late (6-month) postoperative alignment after unilateral lateral rectus recession for small to moderate intermittent exotropia. A satisfactory result, within 8PD, was achieved in 77% of patients at the 6-month examination. Olitsky concluded that there was not a significant difference in early versus late alignment with unilateral lateral lateral rectus surgery, that early overcorrection is not desirable, and that acceptable alignment in the first week provides the best prognosis for stability of postoperative alignment.

Unilateral surgery for exotropia with convergence weakness was studied prospectively by Kraft and coworkers.43 Unilateral recession of a lateral rectus and a resection or advancement of a medial rectus was performed, with the medial rectus strengthened more than the lateral rectus was recessed. The mean exotropia at distance was 18.3PD (range 8 to 35PD), and near exotropia was 30.1PD (range 16 to 50PD). The mean follow up was 7.9 months (range 6 to 26 months), with a mean distance deviation of 0.1PD (range 8PD for esotropia to 10PD for exotropia) and mean near deviation of 1.8PD (range 2PD for esotropia to 8PD for exotropia). The difference of near to distance deviation was reduced from a mean preoperative 11.8PD (range 8 to 21PD), to a mean postoperative 1.7PD (range 0 to 6PD). Their approach to surgery, basing the quantity of surgery on the near deviation, and strengthening the medial more than weakening the lateral rectus muscle, reduced the tendency to create large or long-term postoperative esodeviations. They stressed that this type of surgery should be restricted to patients who could not be controlled by nonsurgical therapy or those with large or poorly controlled exodeviations.

Choice of the initial surgical procedure used routinely by surgeons treating intermittent exotropia is rather evenly divided between recession of the lateral rectus and unilateral recession-resection of the horizontal rectus, with equally good results.44,45 It probably is not important, therefore, to argue the merits of either approach, because the overriding factor that dictates the surgeon's choice is confidence in the procedure. Recession of the lateral rectus is advocated by some surgeons because it is neat and symmetric. Some surgeons believe that recession-resection produces a more permanent result than recession alone because the resection of the medial rectus muscle “locks” the eye in better alignment, preserving the benefit of the recessed lateral rectus muscle. They also claim that the asymmetric horizontal measurements produced by the recession-resection are minor and that they gradually improve.

Botulinum treatment for strabismus was first reported in 1981 by Scott.46 A later paper by Scott and colleagues47 reported the results of Botulinum treatment of childhood strabismus. The frequency of correction to 10PD or less was only 45% in exotropia, with a follow-up period averaging 26 months after the last injection, with an average of 1.7 injections per patient. The results in all cases revealed that smaller deviations (10–20PD) were more frequently corrected, 73%, than larger deviations were (20–110PD), 54%. In a more recent paper, Spencer and associates48 reported on a nonrandomized, case-controlled study of patients with intermittent exotropia. Each patient received simultaneous bilateral lateral rectus injections of 2.5 units botulinum toxin type A, under anesthesia, and the mean preinjection deviation was reduced from 29 to 6PD. A satisfactory outcome was stable binocular alignment of orthophoria within 10PD, which was achieved in 69% (22 of 32). Only one set of bilateral injections was required in all patients between 24 and 56 months of age, irrespective of the initial exotropia angle, and was not associated with any secondary abnormalities.

Several factors determine the quantity of surgery used to correct the intermittent exotropia; however, there is still a baseline level of surgical intervention that must be performed according to the distance prism and alternate cover measurements. The baseline quantities are presented in Table 1. The table can be read in either of two ways. First, reading from top to bottom determines the quantity of surgery performed for exoangles ranging between 15PD and 30PD, and the millimeters of recession performed per prism diopter of exoangle as the initial procedure. It also shows the quantity of resections of the medial rectus muscles that are performed per prism diopter of exoangle as the secondary procedure. Second, by reading the table horizontally rather than vertically and from bottom to top rather than from top to bottom determines the millimeters of recession-resection of the horizontal rectus of one eye per prism diopter of exoangle. For 40PD, 50PD, and 60PD of exoangle, the surgeon selects an initial bilateral recession-resection procedure and performs the same quantity of surgery listed for 20PD, 25PD, and 30PD, respectively, on each eye.

 

TABLE 1. Quantity of Surgery Suggested According to Exoangle


XT AngleRecess LROUResect MROU
154 mm3 mm
205 mm4 mm
256 mm5 mm
307 mm6 mm
XT AngleRecess LRResect MR

LROU, lateral rectus muscle both eyes; MROU, medial rectus muscle both eyes; LR, lateral rectus; MR, medial rectus.

 

Berland and coworkers49 described 8 to 9 mm lateral rectus muscle surgery for deviations ranging from 35 to 65PD and reported surgical results, incidence of abduction deficits, and correlation with overcorrections. Only one operation was required in 80% of patients, but 20% required a second operation, two of three for overcorrection and one of three for undercorrection. Mild but persistent abduction deficits were found postoperatively in 30% but were not predictive of poor outcome. Concurrent oblique muscle surgery was associated with a higher risk of a poor result, with inferior oblique weakening associated with horizontal alignment undercorrection in every case reported. Superior oblique weakening was associated with one overcorrection and one undercorrection of the horizontal deviations.

Adjustments in the quantity of surgery performed per prism diopter of exoangle, as listed in Table 1, are required in patients with a low AC/A ratio and in those patients with lateral incomitance. The patient with a low AC/A ratio who manifests a near exoangle that exceeds the distance exoangle by 10PD or more requires 1 mm more surgery per muscle than the Table 1 indicates. The patient having 10PD or more reduction of exoangle on lateral gaze compared with the primary position requires 1 mm less surgery per muscle than Table 1 indicates.

The importance of lateral incomitance was first reported by Parks, who recommended decreasing the amount of recession ordinarily done for primary position exotropia when the measurements in right and left gaze are less than in the primary position.50 A small residual exophoria in the primary position and straight eyes in side gaze are preferable after surgery to orthophoria in the primary position and esotropia on side gaze. Moore subsequently suggested a disorder of divergence or convergence tonus as the mechanism of decreased exodeviation on lateral gaze.51 Concerning intermittent exotropia diminished on side gaze, she reported that 32% of patients experienced overcorrection, with esotropia increased on side gaze. In cases of intermittent exotropia without lateral incomitance, only 4% had overcorrection. These results occurred regardless of the type of intermittent exotropia or of the type of surgery performed. These results suggest that excessive surgery was performed in those patients with lateral incomitance.

Moore51 also reported an incidence of 22% of lateral incomitance in nonparetic exotropia. Repka and Arnoldi52 found lateral incomitance of more than 5 D at a much lower incidence, 9%. Thompson and Guyton53,54 have stressed holding the prism correctly for accuracy in measuring eye deviations. Repka and Arnoldi52 noted that holding the prisms incorrectly when measuring lateral gaze rotations causes the prism to have a different effective power. Further, they found that measurements of lateral gaze deviations produced smaller measurements when the prism was placed over the abducting eye in lateral incomitance, rather than over the adducting eye. Therefore, if the lateral gaze measurements are taken with a maximum 30 degree head turn, with the prism placed over the adducting eye, artifactitious incomitance produced by placing the prism over the abducting eye is minimized.55

Carlson and Jampolsky56 performed bilateral medial rectus and lateral rectus recessions on adjustable sutures in three patients, based on the hypothesis of tight medial rectus muscles in association with tight lateral rectus muscles as a cause of lateral incomitancy in intermittent exotropias. To compensate for the medial rectus recessions, the lateral rectus recessions exceeded the normal amount, and the primary position alignment with alleviation of lateral incomitancy was achieved. For marked lateral gaze incomitance in intermittent exotropia, medial rectus resections, with or without posterior fixation sutures, have been recommended.57 Those who received resections alone had small-angle esotropia persisting in lateral gaze, but those patients who also had posterior fixation sutures had straight eyes in all positions of gaze. There was no primary position near misalignment reported.

The surgeon who recesses the lateral rectus muscles should anticipate ± 20PD esotropia in the primary position on the second or third postoperative day.58,59 This is significantly reduced by the tenth postoperative day, and the immediate overcorrection usually disappears by the third postoperative week. The final result can be appraised by the sixth postoperative week.

Scott and coworkers60 agree that the best alignment 1 to 2 years after surgery occurs in the presence of a moderate overcorrection at the initial postoperative examination, regardless of the presence of amblyopia or the intermittency of the exodeviation.

Kushner and coworkers61 analyzed factors influencing response to strabismus surgery in the degrees of change of alignment per millimeter of surgery and found a positive correlation to the preoperative deviation in patients with exotropia and esotropia. The response was not significantly improved by including age, axial length, or preoperative refractive error. They stressed that although the response to strabismus surgery correlates significantly and inversely with axial length, that the preoperative deviation is a much stronger influence.

The surgeon who performs the recession-resection procedure must not anticipate more than a few prism diopters of immediate postoperative overcorrection. However, the surgeon who strives for “straight eyes” immediately after recession of the lateral rectus muscles consistently produces an undercorrection that requires secondary surgery for the residual exoangle. The surgeon who performs recession of the lateral rectus muscles must be prepared to accept transient postoperative esotropia for the first 10 days to 3 weeks if doing an adequate quantity of surgery. By contrast, Pratt-Johnson34 and associates statistically analyzed their data and found no significant difference in the immediate postoperative period, whether the eyes were esotropic, exotropic, or orthophoric. The possibility of persistent overcorrection with production of the monofixation syndrome, with or without amblyopia, or of an esotropia without fusion, must be considered.

In a series of 69 patients who were initially overcorrected, intentionally, after surgery for intermittent exotropia, Keech and Stewart62 found only eight patients (11.6%) with persistent overcorrection of 3PD or more, and only three (4.3%) with persistent diplopia. Smoot and colleagues63 reported restoration of stereopsis in eight of nine patients who required medial rectus recessions for constant secondary esotropia after bilateral lateral rectus muscle recessions for intermittent exotropia. Stereopsis measured 50 seconds or better on six patients, and 200 and 400 seconds in the other two patients who regained stereopsis. Unilateral or bilateral advancement of the medial rectus muscle to the original muscle insertion64 improved adduction deficiency in 71%, improved convergence insufficiency in 45%, and produced binocular vision in 50% when tested with Bagolini glasses. Secondary exotropia with limitation of adduction, either early, several weeks after surgery, or late, several months after surgery, require careful observation. Further surgery is more likely, as compared with the situation in patients without limitation of adduction.65

The presence of the monofixation syndrome after the surgical correction of intermittent exotropia may be explained by the presence of a preoperative monofixation syndrome.66 The intermittent nature of the exotropia produces the monofixation syndrome less frequently than does esotropia, in which the duration is more constant.67,68

In their outcome study of lateral rectus recession for intermittent exotropia in children, Ing and colleagues69 considered successful alignment to be an absence of any postoperative intermittent or constant tropia in any position of gaze. Their success rate was 62% (32 of 52) for the initial procedure, with a mean preoperative distance deviation of 25PD, a mean of 4 years and 8 months, and a mean follow-up of 4 years and 4 months. Age at initial surgery, initial deviation, initial refraction, and alignment 1 week postoperatively were not predictive of success. However, alignment at 6 months was highly correlated with successful alignment. Secondary surgery was required in 21% (11 of 52), for residual deviation, and an incidence of overcorrection of 17% (9 of 52) occurred, of which 8% (4 of 52) developed the monofixation syndrome. In the discussion of the Ing group's paper, Parks70 included those four patients with monofixation syndrome with excellent alignment as successful, with no deviation to 2PD of esodeviation at distance and 2 to 6 PD esodeviation at near. This increased the success rate to 69% (36 of 52) and reduced the overcorrections to 10% (5 of 52).

Undercorrections are more numerous than overcorrections,35 according to the quantity of surgery advised per prism diopter of deviation (see Table 1). According to Table 1, in approximately 5% of patients with recessions of the lateral rectus muscles as the initial surgery permanent overcorrection remains, compared with approximately 27% of patients who have undercorrection and who require secondary surgery. Patients with undercorrections of 15PD or more undergo reoperation with resections of the medial rectus muscles any time it becomes apparent that secondary surgery is needed, 6 weeks or later after initial surgery. If the initial surgery is a recession-resection procedure, then the secondary surgery is a recession-resection procedure on the opposite eye.

Associated motility problems are treated surgically at the time of exosurgery, except for overacting superior oblique muscles. A and V patterns are corrected by vertically offsetting the horizontal rectus muscles insertions, and overacting inferior oblique muscles are recessed. Overacting superior obliques are largely ignored in patients with intermittent exotropia because of previous bad experience in producing a postoperatively vertical deviation in primary position; this deviation causes conspicuous torticollis in some patients.

Sustained overcorrection of the exoangle deserves prolonged observation, antiaccommodation therapy, compensation of the esoangle with prism spectacles, and occlusion therapy for amblyopia.35,36,59,71–73 This therapy is initiated by the sixth postoperative week, unless it is apparent that straight eyes occur when raising or lowering the chin because of an associated A or V pattern. Miotic therapy rather than spectacles is usually used for this disorder because within a few days it is apparent whether or not antiaccommodation treatment improves the esoangle. If the miotic does not control the esotropia, prism spectacles are prescribed that also fully correct the hypermetropia, incorporating base-out prism power equal to that of the esoangle. If a concomitant high AC/A ratio is present, a miotic is used in conjunction with spectacles fitted with prisms. Prism power is reduced as the esoangle reduces; the Fresnel prism is ideal for this because of the ease in making the change. The patient is reexamined at monthly intervals, and if no improvement occurs within 6 months, surgical intervention is recommended. The surgeon is advised to follow the dictum of Cooper, approaching the case as though it were an unoperated esotropia, unless a duction restriction is apparent in the field of action of the previously operated muscle, requiring investigation and repair.74

EXOTROPIA

Management of exotropia includes treatment of amblyopia and surgery. Patients younger than 10 years of age may have their amblyopia improved before surgery, but most constantly exotropic patients have either alternate fixation or associated pathologic changes that explain the poor sight; therefore, only a minority require therapy for amblyopia.

Patients with congenital constant exotropia should receive surgery as early as 6 months of age, and sufficient surgery should be performed to straighten the eyes, which usually are widely divergent. These patients are managed in a manner similar to those with congenital esotropia (Fig. 1). Infantile exotropia is rare,75 but high levels of binocular function can develop postoperatively in some patients. Oblique muscle overaction and dissociated vertical deviations are common, but nystagmus is rare, compared with congenital esotropia. Hunter and Ellis76 evaluated the prevalence of systemic and ocular disease associated with infantile exotropia. Records of 70 patients diagnosed with exotropia and 136 with esotropia before 1 year of age were evaluated. Some 67% with exotropia and 49% with esotropia had coexisting ocular or systemic abnormalities. Those with constant infantile exotropia were more likely to present with coexisting ocular and systemic disease than those with intermittent exotropia. Ocular diseases found in association with infantile exotropia included congenital cataracts, congenital nystagmus, Duane syndrome, types I and II, iridolenticular abnormalities, ocular motor apraxia, oculocutaneous albinism, optic atrophy, optic nerve hypoplasia, ptosis, and retinoschisis.

Fig. 1. A. A 3-month-old child with congenital exotropia. left exotropia = 55 degrees. B. The same child at 13 months of age has straight eyes 7 months after recession of the lateral rectus muscles, both eyes.

Children with an exotropic poor-seeing or blind eye that is cosmetically disfiguring should receive surgery by 4 years of age to prevent development of psychological problems resulting from the child's unusual appearance and the comments peers may make about the eyes.

Older patients with good vision in each eye receive surgery according to the same criteria discussed for patients with intermittent exotropia. Those with a poor-seeing or blind eye invariably receive recession-resection on that eye. If the exoangle of the poor eye exceeds 30PD, a surgeon is unable to limit the quantity of surgery per muscle to the quantity that does not interfere postoperatively with the normal horizontal ductions of the eye.

The surgeon must do excessive recession-resection to straighten the large unilateral exotropia, thus sacrificing normal postoperative abduction, or must be allowed by the patient to distribute the surgery between the two eyes; this means also operating on the better-seeing eye.77 The latter procedure is the best for motility status, but it may be disturbing to the patient. If the surgeon must confine the surgery to the poor-seeing eye, then the quantities listed in Table 2 are recommended.

 

TABLE 2. Quantity of Surgery Advised According to Exoangle in Patient with Profound Amblyopia


XT AngleRecess LRResect MR
408 mm6 mm
509 mm7 mm
6010 mm8 mm
7010 mm9 mm
8010 mm10 mm

LR, Lateral rectus; MR, medial rectus.

 

In addition to the muscle surgery required to correct the long-standing large angle of monocular exotropia, the tightly contracted lateral conjunctiva and Tenon's capsule must be recessed; otherwise an undercorrection may occur.

Raab described four-muscle surgery for large angle exotropia; this included recessing the lateral rectus 7 to 8 mm, resecting the medial rectus 6 to 7 mm, recessing the inferior oblique, and performing a tenectomy or tenotomy of the superior oblique.78 Additional temporal conjunctival recession was performed. Postoperative hypertropia may be a disadvantage and is avoided by infraplacement of both horizontal recti, ignoring an A or V pattern.

An important factor that alters the usual quantity of surgery per prism diopter of exoangle is the size of the eyeball, with microphthalmia requiring relatively less surgery.

In adults with long-standing horizontal strabismus and absence of fusion, a prospective, randomized clinical trial was reported by Carruthers and coworkers.79 Botulinum treatment was received by 17 and surgery with adjustable sutures by 13 patients. The net change in alignment was 50.3% with Botulinum, but 96% with surgery. A final deviation of 10PD or less at least 6 months after treatment was considered a successful outcome. Although the series was small, the success rate for Botulinum was only 29.4% compared with 76.9% with adjustable suture surgery.

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REFERENCES

1. Raab EL: Consecutive accommodative esotropia. J Pediatr Ophthalmol Strabismus 22:58, 1985

2. Parks MM: Causes of the adherence syndrome. In Symposium on Strabismus, Transactions of the New Orleans Academy of Ophthalmology, p 269. St Louis: CV Mosby, 1978

3. Nixon RB, Helveston EM, Miller K et al: Incidence of strabismus in neonates. Am J Ophthalmol 100:798, 1985

4. Sondhi N, Archer SM, Helveston EM: Development of normal ocular alignment. J Pediatr Ophthalmol Strabismus 25:210, 1988

5. Archer SM, Sondhi N, Helveston EM: Strabismus in infancy. Ophthalmology 96:133, 1989

6. Rubin SE, Nelson LB, Wagner RS et al: Infantile exotropia in healthy children. Ophthalmic Surg 19:792, 1988

7. Hiles DA, Davies GT, Costenbader FD: Long term observations on unoperated intermittent exotropia. Arch Ophthalmol 80:436, 1968

8. Campos EC, Cipolli C: Binocularity and photophobia in intermittent exotropia. Percept Mot Skills 74:1168, 1992

9. von Noorden GK: Binocular Vision and Ocular Motility: Theory and management of strabismus. 4th ed. St Louis: CV Mosby, 1990

10. Stathacopoulos RA, Rosenbaum AL, Zanoni D et al: Distance stereoacuity: Assessing control in intermittent exotropia. Ophthalmology 100:495, 1993

11. O'Neal TD, Rosenbaum AL, Stathacopoulos RA: Distance stereo acuity improvement in intermittent exotropic patients following strabismus surgery. J Pediatr Ophthalmol Strabismus 32:353, 1995

12. Yildririm C, Mutlu FM, Chen Y et al: Assessment of central peripheral fusion and near and distance stereoacuity in intermittent exotropic patients before and after strabismus surgery. Am J Ophthalmol 128:222, 1999

13. Ball A, Drummond GT, Pearce WG: Unexpected stereoacuity following surgical correction of long-standing horizontal strabismus. Can J Ophthalmol 28:217, 1993

14. Baker JD: Personal communication, 1974

15. Maddox SF: Personal communication, 1974

16. Strome RR: Personal communication, 1974

17. Stager DR, Weakley DR, Everett M et al: Delayed consecutive exotropia following 7-millimeter bilateral medial rectus recession for congenital esotropia. J Pediatr Ophthalmol Strabismus 31:147, 1994

18. Seaber JH, Chandler AC: A five-year study of patients with cerebral palsy and strabismus. In Moore S, Mein J, Stockbridge L (eds): Orthoptics: Past, Present and Future. New York: Grune & Stratton, 1976

19. Guibor GP: Some eye defects seen in cerebral palsy, with some statistics. Am J Phys Med 32:342, 1953

20. Hiles DA, Wallar PH, McFarland F: Current concepts in the management of strabismus in children with cerebral palsy. Ann Ophthalmol 7:789, 1975

21. Mitchell PR: Ophthalmologic problems. In Thompson GH, Rubin IL, Bilenker RM (eds): Comprehensive Management of Cerebral Palsy, p 139. New York, Grune & Stratton, 1983

22. Duane A: A new classification of the motor anomalies of the eyes based upon physiologic principles, together with their symptoms, diagnosis and treatment. Ann Ophthalmol Otolaryngol 5:969, 1896; 6:84, 1897

23. Kushner BJ: The distance angle to target in surgery for intermittent exotropia. Arch Ophthalmol 116:189, 1998

24. Wilson ME, Parks MM: Primary inferior oblique overaction in congenital esotropia, accommodative esotropia, and intermittent exotropia. Ophthalmology 96:950, 1989

25. Burian HN, Spivey BE: The surgical management of exodeviations. Am J Ophthalmol 59:603, 1965

26. Hunt J, Rasicovici A, Windsor CE: Comprehensive Review of Orthoptics and Ocular Motility: Theory, Therapy and Surgery, pp 164–165. St Louis: CV Mosby, 1975

27. Caltrider N, Jampolsky A: Overcorrecting minus lens therapy for treatment of intermittent exotropia. Ophthalmology 90:1160, 1983

28. Iacobucci IL, Martonyi EJ, Giles CL: Results of over-minus lens therapy on postoperative exodeviations. J Pediatr Ophthalmol Strabismus 23:287, 1986

29. von Noorden GK: Resection of both medial rectus muscles in organic convergence insufficiency. Am J Ophthalmol 81:223, 1976

30. Cooper EL, Leyman IA: The management of intermittent exotropia: A comparison of the results of surgical and non-surgical treatment. American Orthoptic J 27:61, 1977

31. Moore S: Orthoptic treatment for intermittent exotropia. American Orthoptic J 13:14, 1963

32. Stoller SH, Simon JW, Lininger LL: Bilateral lateral rectus recession for exotropia: a survival analysis. J Pediatr Ophthalmol Strabismus 31:89–92, 1994

33. Ohtsuki H, Hasebe S, Okano M et al: Comparison of surgical results of responders and non-responders to the prism adaptation test in intermittent exotropia. Acta Ophthalmol Scand 75:528, 1997

34. Pratt-Johnson JA, Barlow JM, Tillson G: Early surgery in intermittent exotropia. Am J Ophthalmol 84:689, 1977

35. Richard JM, Parks MM: Intermittent exotropia: Surgical results in different age groups. Ophthalmology 90:1172, 1983

36. Schlossman A, Muchnick RS, Stern KS: The surgical management of intermittent exotropia in adults. Ophthalmology 90:1166, 1983

37. Freeman RS, Isenberg SJ: The use of part-time occlusion for early onset unilateral exotropia. J Pediatr Ophthalmol Strabismus 26:94, 1989

38. Feretis D, Mela E, Vasilopoulos: Excessive single lateral rectus muscle recession in the treatment of intermittent exotropia. J Pediatr Ophthalmol Strabismus 27:315, 1990

39. Deutsch JA, Nelson LB, Sheppard RW et al: Unilateral lateral rectus recession for the treatment of exotropia. Ann Ophthalmol 24:111, 1992

40. Nelson LB, Bacal DA, Burke MJ: An alternative approach to the surgical management of exotropia—the unilateral lateral rectus recession. J Pediatr Ophthalmol 29:357, 1992

41. Weakley DR, Stager DR: Unilateral lateral rectus recessions in exotropia. Ophthalmic Surg 24:458, 1993

42. Olitsky SE: Early and late postoperative alignment following unilateral lateral rectus recession for intermittent exotropia. J Pediatr Ophthalmol Strabismus 35:146, 1998

43. Kraft SP, Levin AV, Enzenauer RW: Unilateral surgery for exotropia with convergence weakness. J Pediatr Ophthalmol Strabismus 32:183, 1995

44. Scott AB, Mash AJ, Jampolsky A: Quantitative guidelines for exotropia surgery. Invest Ophthalmol 14:428, 1975

45. Moore S, Stockbridge L, Knapp P: A panoramic view of exotropias. American Orthoptic J 27:70, 1977

46. Scott AB: Botulinum toxin injection of eye muscles to correct strabismus. Trans Am Ophthalmol Soc 79:734, 1981

47. Scott AB, Magoon EH, McNeer KW et al: Botulinum treatment of childhood strabismus. Ophthalmology 97:1434, 1990

48. Spencer RF, Tucker MG, Choi RY et al: Botulinum toxin management of childhood intermittent exotropia. Ophthalmology 104:1762, 1997

49. Berland JE, Wilson ME, Saunders RA: Results of large (8–9 mm) bilateral lateral rectus muscle recessions for exotropia. Binocular Vision Strabismus Q 13:97, 1998

50. Parks MM: Comitant exodeviations in children. In Strabismus Symposium of the New Orleans Academy of Ophthalmology, p 49. St Louis: CV Mosby, 1962

51. Moore S: The prognostic value of lateral gaze measurements in intermittent exotropia. American Orthoptic J 19:69, 1969

52. Repka MX, Arnoldi KA: Lateral incomitance in exotropia: Fact or artifact? J Pediatr Ophthalmol Strabismus 28:125, 1991

53. Thompson JT, Guyton DL: Ophthalmic prisms: Measurement errors and how to minimize them. Ophthalmology 90:204, 1983

54. Thompson JT, Guyton DL: Ophthalmic prisms: Deviant behavior at near. Ophthalmology 92:684, 1985

55. Wilson ME: Lateral incomitance in exotropia: Fact or artifact? discussion. J Pediatr Ophthalmol Strabismus 28:129, 1991

56. Carlson MR, Jampolsky A: Lateral incomitancy in intermittent exotropia: Cause and surgical therapy. Arch Ophthalmol 97:1922, 1979

57. Diamond GR: Medial rectus resection strategies for laterally incomitant intermittent exotropia. J Pediatr Ophthalmol Strabismus 24:242, 1987

58. Raab EL, Parks MM: Recession of the lateral recti: Early and late postoperative alignments. Arch Ophthalmol 82:203, 1969

59. Hardesty HH, Boynton JR, Keenan JP: Treatment of intermittent exotropia. Arch Ophthalmol 96:268, 1978

60. Scott WE, Keech R, Mash AJ: The postoperative results and stability of exodeviations. Arch Ophthalmol 99:1814, 1981

61. Kushner BJ, Fisher MR, Lucchese NJ et al: Factors influencing response to strabismus surgery. Arch Ophthalmol 111:75, 1993

62. Keech RV, Stewart SA: The surgical overcorrection of intermittent exotropia. J Pediatr Ophthalmol Strabismus 27:218, 1990

63. Smoot CN, Simon JW, Nelson LB: Binocularity following surgery for secondary esotropia in childhood. Br J Ophthalmol 74:155, 1990

64. Ohtsuki H, Hasebe S, Tadokoro Y et al: Advancement of medial rectus muscle to the original insertion for consecutive exotropia. J Pediatr Ophthalmol Strabismus 30:301, 1993

65. Bradbury JA, Doran RML: Secondary exotropia: A retrospective analysis of matched cases. J Pediatr Ophthalmol Strabismus 30:163–166, 1993

66. Baker JD, Davies GT: Monofixational intermittent exotropia. Arch Ophthalmol 97:93, 1979

67. Parks MM: The monofixation syndrome. Trans Am Ophthalmol Soc 67:609, 1969

68. Parks MM: Monofixation syndrome: A frequent end stage of strabismus surgery. Trans Pa Acad Ophthalmol Otolaryngol 79:733, 1975

69. Ing MR, Nishimura J, Okino L: Outcome study of bilateral lateral rectus recession for intermittent exotropia in children. Trans Am Ophthalmol Soc 95:433, 1997

70. Parks MM: Discussion of Ing MR, Nishimura J, Okino L: Outcome study of bilateral lateral rectus recession for intermittent exotropia in children. Trans Am Ophthalmol Soc 95: 443, 1997

71. Hardesty H: Treatment of overcorrected intermittent exotropia. Am J Ophthalmol 66:80, 1968

72. Hardesty H: Treatment of under- and overcorrected intermittent exotropia with prism glasses. Am Orthoptic J 17:110, 1969

73. Knapp P: Use of membrane prisms. Trans Am Acad Ophthalmol Otolaryngol 79:718, 1975

74. Cooper EL: The surgical management of secondary exotropia. Trans Am Acad Ophthalmol Otolaryngol 65:595, 1961

75. Biglan AW, Davis JS, Cheng KP et al: Infantile exotropia. J Pediatr Ophthalmol Strabismus 33:79, 1996

76. Hunter DG, Ellis FJ: Prevalence of systemic and ocular disease in infantile exotropia. Ophthalmology 106:1951, 1999

77. Rayner JW, Jampolsky A: Management of adult patients with large angle amblyopic exotropia. Ann Ophthalmol 5:95, 1973

78. Raab EL: Unilateral four muscle surgery for large angle exotropia. Ophthalmology 86:1141, 1979

79. Carruthers JD, Kennedy RA, Bagaric D: Botulinum vs adjustable suture surgery in the treatment of horizontal misalignment in adult patients lacking fusion. Arch Ophthalmol 108:1432, 1990

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