- •Foreword
- •Preface
- •Contributors
- •Contents
- •1. Epidemiology of Pediatric Strabismus
- •1.1 Introduction
- •1.2 Forms of Pediatric Strabismus
- •1.2.1 Esodeviations
- •1.2.1.1 Congenital Esotropia
- •1.2.1.2 Accommodative Esotropia
- •1.2.1.3 Acquired Nonaccommodative Esotropia
- •1.2.1.4 Abnormal Central Nervous System Esotropia
- •1.2.1.5 Sensory Esotropia
- •1.2.2 Exodeviations
- •1.2.2.1 Intermittent Exotropia
- •1.2.2.2 Congenital Exotropia
- •1.2.2.4 Abnormal Central Nervous System Exotropia
- •1.2.2.5 Sensory Exotropia
- •1.2.3 Hyperdeviations
- •1.3 Strabismus and Associated Conditions
- •1.4.1 Changes in Strabismus Prevalence
- •1.4.2 Changes in Strabismus Surgery Rates
- •1.5 Worldwide Incidence and Prevalence of Childhood Strabismus
- •1.6 Incidence of Adult Strabismus
- •References
- •2.1 Binocular Alignment System
- •2.1.2 Vergence Adaptation
- •2.1.3 Muscle Length Adaptation
- •2.2 Modeling the Binocular Alignment Control System
- •2.2.1 Breakdown of the Binocular Alignment Control System
- •2.2.4 Changes in Basic Muscle Length
- •2.2.6 Evidence Against the “Final Common Pathway”
- •2.3 Changes in Strabismus
- •2.3.1 Diagnostic Occlusion: And the Hazard of Prolonged Occlusion
- •2.3.2.1 Supporting Evidence for Bilateral Feedback Control of Muscle Lengths
- •2.4 Applications of Bilateral Feedback Control to Clinical Practice and to Future Research
- •References
- •3.1 Dissociated Eye Movements
- •3.2 Tonus and its relationship to infantile esotropia
- •3.5 Pathogenetic Role of Dissociated Eye Movements in Infantile Esotropia
- •References
- •4.1 Introduction
- •4.2.1 Binocular Correspondence: Anomalous, Normal, or Both?
- •4.3 MFS with Manifest Strabismus
- •4.3.1 Esotropia is the Most Common Form of MFS
- •4.3.2 Esotropia Allows for Better Binocular Vision
- •4.3.3 Esotropia is the Most Stable Form
- •4.4 Repairing and Producing MFS
- •4.4.1 Animal Models for the Study of MFS
- •References
- •5.1 Esotropia as the Major Type of Developmental Strabismus
- •5.1.2 Early Cerebral Damage as the Major Risk Factor
- •5.1.3 Cytotoxic Insults to Cerebral Fibers
- •5.1.5 Development of Binocular Visuomotor Behavior in Normal Infants
- •5.1.6 Development of Sensorial Fusion and Stereopsis
- •5.1.7 Development of Fusional Vergence and an Innate Convergence Bias
- •5.1.8 Development of Motion Sensitivity and Conjugate Eye Tracking (Pursuit/OKN)
- •5.1.9 Development and Maldevelopment of Cortical Binocular Connections
- •5.1.10 Binocular Connections Join Monocular Compartments Within Area V1 (Striate Cortex)
- •5.1.11 Too Few Cortical Binocular Connections in Strabismic Primate
- •5.1.12 Projections from Striate Cortex (Area V1) to Extrastriate Cortex (Areas MT/MST)
- •5.1.15 Persistent Nasalward Visuomotor Biases in Strabismic Primate
- •5.1.16 Repair of Strabismic Human Infants: The Historical Controversy
- •5.1.18 Timely Restoraion of Correlated Binocular Input: The Key to Repair
- •References
- •6. Neuroanatomical Strabismus
- •6.1 General Etiologies of Strabismus
- •6.2 Extraocular Myopathy
- •6.2.1 Primary EOM Myopathy
- •6.2.2 Immune Myopathy
- •6.2.4 Neoplastic Myositis
- •6.2.5 Traumatic Myopathy
- •6.3 Congenital Pulley Heterotopy
- •6.4 Acquired Pulley Heterotopy
- •6.5 “Divergence Paralysis” Esotropia
- •6.5.1 Vertical Strabismus Due to Sagging Eye Syndrome
- •6.5.2 Postsurgical and Traumatic Pulley Heterotopy
- •6.5.3 Axial High Myopia
- •6.6 Congenital Peripheral Neuropathy: The Congenital Cranial Dysinnervation Disorders (CCDDs)
- •6.6.1 Congenital Oculomotor (CN3) Palsy
- •6.6.3 Congenital Trochlear (CN4) Palsy
- •6.6.4 Duane’s Retraction Syndrome (DRS)
- •6.6.5 Moebius Syndrome
- •6.7 Acquired Motor Neuropathy
- •6.7.1 Oculomotor Palsy
- •6.7.2 Trochlear Palsy
- •6.7.3 Abducens Palsy
- •6.7.4 Inferior Oblique (IO) Palsy
- •6.8 Central Abnormalities of Vergence and Gaze
- •6.8.1 Developmental Esotropia and Exotropia
- •6.8.2 Cerebellar Disease
- •6.8.3 Horizontal Gaze Palsy and Progressive Scoliosis
- •References
- •7.1 Congenital Cranial Dysinnervation Disorders: Facts About Ocular Motility Disorders
- •7.1.1 The Concept of CCDDs: Ocular Motility Disorders as Neurodevelopmental Defects
- •7.1.1.1 Brainstem and Cranial Nerve Development
- •7.1.1.2 Single Disorders Representing CCDDs
- •7.1.1.3 Disorders Understood as CCDDs
- •7.2 Congenital Cranial Dysinnervation Disorders: Perspectives to Understand Ocular Motility Disorders
- •7.2.1.1 Brown Syndrome
- •Motility Findings
- •Saccadic Eye Movements
- •Comorbidity
- •Epidemiologic Features
- •Laterality
- •Sex Distribution
- •Incidence
- •Heredity
- •Potential Induction of the Syndrome
- •Radiologic Findings
- •Natural Course in Brown Syndrome
- •Intra-and Postoperative Findings
- •References
- •8.1 Amblyopia
- •8.2 What Is Screening?
- •8.2.1 Screening for Amblyopia, Strabismus, and/or Refractive Errors
- •8.2.1.1 Screening for Amblyopia
- •8.2.1.2 Screening for Strabismus
- •8.2.1.3 Screening for Refractive Error
- •8.2.1.4 Screening for Other Ocular Conditions
- •8.3 Screening Tests for Amblyopia, Strabismus, and/or Refractive Error
- •8.3.1 Vision Tests
- •8.3.3 Stereoacuity
- •8.3.4 Photoscreening and/or Autorefraction
- •8.3.6 Who Should Administer the Screening Program?
- •8.4 Treatment of Amblyopia
- •8.4.1 Type of Treatment
- •8.4.2 Refractive Adaptation
- •8.4.3 Conventional Occlusion
- •8.4.4 Pharmacological Occlusion
- •8.4.5 Optical Penalization
- •8.4.7 Treatment Compliance
- •8.4.8 Other Treatment Options for Amblyopia
- •8.4.9 Recurrence of Amblyopia Following Therapy
- •8.5 Quality of Life
- •8.5.1 The Impact of Amblyopia Upon HRQoL
- •8.5.3 Reading Speed and Reading Ability in Children with Amblyopia
- •8.5.4 Impact of Amblyopia Upon Education
- •8.5.6 The Impact of Strabismus Upon HRQoL
- •8.5.7 Critique of HRQoL Issues in Amblyopia
- •8.5.8 The Impact of the Condition or the Impact of Treatment?
- •References
- •9. The Brückner Test Revisited
- •9.1 Amblyopia and Amblyogenic Disorders
- •9.1.1 Early Detection of Amblyopia
- •9.1.2 Brückner’s Original Description
- •9.2.1 Physiology
- •9.2.2 Performance
- •9.2.3 Shortcomings and Pitfalls
- •9.3.1 Physiology
- •9.3.2 Performance
- •9.3.3 Possibilities and Limitations
- •9.4.1 Physiology
- •9.4.2 Performance
- •9.4.3 Possibilities and Limitations
- •9.5 Eye Movements with Alternating Illumination of the Pupils
- •References
- •10. Amblyopia Treatment 2009
- •10.1 Amblyopia Treatment 2009
- •10.1.1 Introduction
- •10.1.2 Epidemiology
- •10.1.3 Clinical Features of Amblyopia
- •10.1.4 Diagnosis of Amblyopia
- •10.1.5 Natural History
- •10.2 Amblyopia Management
- •10.2.1 Refractive Correction
- •10.2.2 Occlusion by Patching
- •10.2.3 Pharmacological Treatment with Atropine
- •10.2.4 Pharmacological Therapy Combined with a Plano Lens
- •10.3 Other Treatment Issues
- •10.3.1 Bilateral Refractive Amblyopia
- •10.3.3 Maintenance Therapy
- •10.4 Other Treatments
- •10.4.1 Filters
- •10.4.2 Levodopa/Carbidopa Adjunctive Therapy
- •10.5 Controversy
- •10.5.1 Optic Neuropathy Rather than Amblyopia
- •References
- •11.1 Introduction
- •11.1.2 Sensory or Motor Etiology
- •11.1.4 History
- •11.1.5 Outcome Parameters
- •11.2 Outcome of Surgery in the ELISSS
- •11.2.1 Reasons for the ELISSS
- •11.2.2 Summarized Methods of the ELISSS
- •11.2.3 Summarized Results of the ELISSS
- •11.2.4 Binocular Vision at Age Six
- •11.2.5 Horizontal Angle of Strabismus at Age Six
- •11.2.6 Alignment is Associated with Binocular Vision
- •11.3 Number of Operations and Spontaneous Reduction into Microstrabismus Without Surgery
- •11.3.1 The Number of Operations Per Child and the Reoperation Rate in the ELISSS
- •11.3.2 Reported Reoperation Rates
- •11.3.3 Test-Retest Reliability Studies
- •11.3.6 Spontaneous Reduction of the Angle
- •11.3.7 Predictors of Spontaneous Reduction into Microstrabismus
- •Appendix
- •References
- •12.1 Overview
- •12.1.2 Manifest Latent Nystagmus (MLN)
- •12.1.2.1 Clinical Characteristics of Manifest Latent Nystagmus (MLN)
- •12.1.3 Congenital Periodic Alternating Nystagmus (PAN)
- •12.1.3.1 Clinical characteristics of congenital periodic alternating nystagmus
- •12.2 Compensatory Mechanisms
- •12.2.1 Dampening by Versions
- •12.2.2 Dampening by Vergence
- •12.2.3 Anomalous Head Posture (AHP)
- •12.2.3.4 Measurement of AHP
- •12.2.3.6 Testing AHP at Near
- •12.3 Treatment
- •12.3.1 Optical Treatment
- •12.3.1.1 Refractive Correction
- •12.3.1.2 Spectacles and Contact Lenses (CL)
- •12.3.1.3 Prisms
- •12.3.1.4 Low Visual Aids
- •12.3.2 Medication
- •12.3.3 Acupuncture
- •12.3.4 Biofeedback
- •12.3.6 Surgical Treatment of Congenital Nystagmus
- •12.3.6.1 Management of Horizontal AHP
- •12.3.6.2 Management of Vertical AHP
- •12.3.6.3 Management of Head Tilt
- •Retro-Equatorial Recession of Horizontal Rectus Muscles
- •The Tenotomy Procedure
- •References
- •13.1 Dissociated Deviations
- •13.2 Surgical Alternatives to Treat Patients with DVD
- •13.2.1 Symmetric DVD with Good Bilateral Visual Acuity, with No Oblique Muscles Dysfunction
- •13.2.2 Bilateral DVD with Deep Unilateral Amblyopia
- •13.2.3 DVD with Inferior Oblique Overaction (IOOA) and V Pattern
- •13.2.4 DVD with Superior Oblique Overaction (SOOA) and A Pattern
- •13.2.5 Symmetric vs. Asymmetric Surgeries for DVD
- •13.3 Dissociated Horizontal Deviation
- •13.4 Dissociated Torsional Deviation. Head tilts in patients with Dissociated Strabismus
- •13.5 Conclusions
- •References
- •14.1 Introduction
- •14.2 Clinical and Theoretical Investigations
- •References
- •15.1 General Principles of Surgical Treatment in Paralytic Strabismus
- •15.1.1 Aims of Treatment
- •15.1.2 Timing of Surgery
- •15.1.3 Preoperative Assessment
- •15.1.4 Methods of Surgical Treatment
- •15.2 Third Nerve Palsy
- •15.2.1 Complete Third Nerve Palsy
- •15.2.2 Incomplete Third Nerve Palsy
- •15.3 Fourth Nerve Palsy
- •15.4 Sixth Nerve Palsy
- •References
- •16.1 Graves Orbitopathy (GO): Pathogenesis and Clinical Signs
- •16.1.1 Graves Orbitopathy is Part of a Systemic Disease: Graves Disease (GD)
- •16.1.2 Graves Orbitopathy−Clinical Signs
- •16.1.2.1 Clinical Changes Result in Typical Symptoms
- •16.1.3 Clinical Examination of GO
- •16.1.3.1 Signs of Activity
- •16.1.3.2 Assessing Severity of GO
- •16.1.3.3 Imaging
- •16.2 Natural History
- •16.3 Treatment of GO
- •16.3.1.1 Glucocorticoid Treatment
- •16.3.1.2 Orbital Radiotherapy
- •16.3.1.3 Combined Therapy: Glucocorticoids and Orbital Radiotherapy
- •16.3.1.4 Other Immunosuppressive Treatments and New Developments
- •16.3.2 Inactive Disease Stages
- •16.3.2.1 Orbital Decompression
- •16.3.2.2 Extraocular Muscle Surgery
- •16.3.2.3 Lid Surgery
- •16.4 Thyroid Dysfunction and GO
- •16.5.1 Relationship Between Cigarette Smoking and Graves Orbitopathy
- •16.5.2 Genetic Susceptibility
- •16.6 Special Situations
- •16.6.1 Euthyroid GO
- •16.6.2 Childhood GO
- •16.6.3 GO and Diabetes
- •References
Chapter 10
Amblyopia Treatment 2009 |
10 |
Michael X. Repka |
|
Core Messages
■Wearing optimum refractive correction before initiation of patching or other amblyopia therapy is associated with improvement in amblyopia in about three quarters of children and a cure in about one fourth. This improvement may facilitate subsequent treatment.
■For initial therapy of moderate anisometropic
and strabismic amblyopia among children 3–7 years of age, patching and atropine are equivalent. Atropine is slightly more acceptable than patching on the basis of parental questioning.
■For initial therapy of moderate amblyopia, 2 h of daily patching or twice weekly topical atropine
administered to the sound eye are equally e ective.
■For initial therapy of severe amblyopia for children 3 to less than 7 years of age, 6 h of daily patching and full-time patching appear to be equally e ective.
■Amblyopia therapy can be beneficial for older children up to 17 years of age, especially if they have not been previously treated.
■There have not been any studies to date which demonstrate the best therapy for patients with residual amblyopia following initial therapy. There are also no studies that have identified the best treatments for deprivation amblyopia.
10.1Amblyopia Treatment 2009
10.1.1Introduction
Amblyopia management, long based on consensus or clinical wisdom [1, 2], has been developing an evidencebased foundation over the last decade. We have seen the completion of a series of randomized treatment trials and prospective observational studies over the last 10 years. These studies have dealt solely with the most common forms of amblyopia, those due to anisometropia, strabismus or a combination. Spectacle correction is the base on which all treatment for amblyopia must be built. Both patching and atropine penalization are e ective as initial management of moderate amblyopia. Initial dosages of 2 h daily of patching or twice weekly atropine have been shown to be e ective and can be considered suitable for initial therapy. Severe amblyopia may be initially managed with 6 h of patching. Intensified treatment for patients who are incompletely treated is logical to prescribe, yet not proven in clinical trials.
The strict age cut-o of 7 or 8 years for therapy has been shown to be incorrect. Children through at least 13 years of age should be considered suitable for a trial of amblyopia therapy, as a large proportion will experience improvement [3]. Management of deprivation amblyopia, such as seen with unilateral aphakia or trauma, remains di cult, frustrating to the families, and often unsuccessful. There is little new information on management of these patients.
10.1.2Epidemiology
Amblyopia is considered the most common cause of monocular visual impairment in both children and young and middle-aged adults, in up to 4% of individuals [4].Simons, 1996 #181; [5]. It has been suggested that the prevalence is higher in underserved communities [6]. A study conducted by the National Eye Institute found amblyopia to be the leading cause of monocular vision loss in the 20–70-year-old age group [4]. These
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10 Amblyopia Treatment 2009 |
estimates have been based on schoolor clinic-based studies.
Two very recent population-based studies from the United States have reported prevalence estimates for ambly- 10 opia among preschool-aged children in urban areas. One study from Baltimore, Maryland, found the prevalence of amblyopia to be 1.8% in Whites and 0.8% in AfricanAmericans [7]. The authors extrapolated their finding to suggest that there are approximately 271,000 cases of amblyopia among children 30–71 months of age in the United States. The second study, completed in Los Angeles, California, detected amblyopia in 2.6% of Hispanic/Latino children and 1.5% of African-American children, with 78%
of cases of amblyopia attributable to refractive error [8].
A study of a birth cohort at age 7 years in the United Kingdom found 3.6% of children to have amblyopia [9]. There was a suggestion in this latter study that amblyopia prevalence correlated mildly with lower socioeconomic status.
Whatever the actual percentage of amblyopia in a population, this disease remains a common ocular problem among children. The causes of amblyopia depend on the population studied. In one treatment trial, amblyopia was associated with strabismus (37%), Anisometropia (38%) or both combined (24%) [10]. In another retrospective series, amblyopia was associated with strabismus (57%), anisometropia (17%) or both (27%) [11].
10.1.3Clinical Features of Amblyopia
Visual loss in amblyopia as measured with high-contrast opotoypes varies from mild to severe. The literature suggests that about 25% of cases have visual acuity in the amblyopic eye worse than 20/100 and about 75%, 20/100 or better [12, 13]. The more common causes of amblyopia are strabismus and moderate anisometropia, each accounting for about 35%, with 25% having both anisometropia and strabismus [10, 11]. Much less common is amblyopia related to high anisomyopia, bilateral high ametropia and disease of the anterior visual pathways (e.g., optic nerve hypoplasia). Although good results have been occasionally reported with conventional treatment, these cases are typically more di cult to treat successfully.
Other features of amblyopia include a reduction in contrast sensitivity and possibly reading ability. Most studies have found a reduction in contrast sensitivity in eyes with amblyopia using sinusoidal gratings [14–16], whereas minimal loss has been reported with Pelli-Robson charts, which test intermediate spatial frequencies [16, 17]. Detection of a deficit of contrast sensitivity after treatment
of strabismic and anisometropic amblyopia is slight in the intermediate spatial frequencies tested with the low-con- trast letters of the Pelli-Robson charts [16, 17]. We have recently confirmed this finding of only a minimal deficit with Pelli-Robson charts 3–7 years after enrollment in an amblyopia treatment trial [18].
Most studies of reading ability of amblyopic patients have tested the subjects binocularly, rather than monocularly, generally over a wide range of ages. Some of these studies have indicated that binocular reading ability in children with amblyopia is impaired [19, 20], whereas others have reported that reading ability is not a ected [21]. PEDIG recently reported the monocular oral reading speed, accuracy, fluency and comprehension of 79 children with previously treated amblyopia at a mean age of 10.3 years [22]. We found the amblyopic eyes to be slightly slower and less accurate compared with fellow eyes, while comprehension was similar. Because of our study design we could not compare these children to a non-amblyopic population, so the impact of the monocular loss of vision on the patient’s binocular reading ability remains to be thoroughly explored.
10.1.4Diagnosis of Amblyopia
The diagnosis of amblyopia requires detection of a di erence in visual acuity between the two eyes while wearing a necessary spectacle correction. For children who can have optotype acuity accurately measured, this remains the method of choice, in fact arguably, the only method. The test should employ either crowded or line optotypes. The clinician should exercise caution when interpreting the results of optotype testing. The variability of the instrument needs to be considered. Specifically, what is the expected variability of a second measurement when there has been no actual change in the visual acuity? For the Amblyopia Treatment Study Visual acuity testing protocol of single surrounded HOTV, we found high testability after age 3 years, with 93% of retests within 0.1 logMAR. More importantly, the visual acuity needs to di er by more than 0.18 logMAR for the di erence to likely be true [23]. In my experience a one-line change from a prior visit nearly always led to a change in therapy prescribed, usually an escalation. In children the test– retest variability is very high. For children 7–<13 years, a change in visual acuity must be at least 0.2 logMAR (ten letters) from a previous acuity measure to be unlikely resulting from measurement variability [24]. These two studies of rigorously administered visual acuity testing protocols remind clinicians that substantial variability of visual acuity results is present in children and careful
