- •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
146 |
11 Best Age for Surgery for Infantile Esotropia |
of children operated once and 36% of children operated twice. In the studies by Costenbader [1], by Birch (1990) and in the ELISSS [57], children who had been scheduled for surgery but who had not been operated at final assess-
11 ment had better binocular vision than those who had been operated.
Spontaneous resolution of infantile strabismus has first been reported by Clarke & Noel [58]. In a study by the Pediatric Eye Disease Investigator Group [59], among 170 children with IE (age ±3 months at recruitment), of those who had had an angle of strabismus >21.8° during two examinations at least one week apart, 2.4% had an angle <4.6° at ±7 months. Among those children who had had an angle of strabismus >11.3° during two examinations at recruitment, 27% had an angle <4.6° at ±7 months.
Reduction of the angle within 5° frequently results in microstrabismus with peripheral fusion, central suppression, and a favorable appearance. Due to the peripheral fusion, the strabismus remains stable and rarely needs additional surgery, as has been found for small angles postoperatively in the study by Van de Vijver et al. [55].
11.3.6Spontaneous Reduction of the Angle
In the ELISSS, more than half of the children who were scheduled for surgery, but had not been operated at the age of 6 years, had a spontaneous reduction of the strabismus into a microstrabismus (Fig. 11.8).
There are few studies with similar longitudinal measurements of the angle of strabismus in a large group of children. In a recent study by Pediatric Eye Disease Investigator Group [60], the angle of strabismus was measured in 81 children with IE aged 6.0 ± 1.7 months (range 2.4–9.5) at baseline and at 6-week intervals for 18 weeks, using prism and alternate cover test at near (70% of the children) or a modified Krimsky at near (30%). In 20%, all four measurements were within 2.9° or less than one another. In 46%, any two of the four measurements differed by 8.5° or more.
Could we have distinguished the ELISSS children who were scheduled for surgery but, in the end, were never operated, at an early age? In other words, can the reduction of the angle be predicted and, hence, unnecessary operations be avoided in individual cases by waiting? This line of reasoning only pertains to the majority of cases where microstrabismus with peripheral fusion is the best possible result. One cannot exclude the rare possibility that an occasional child, with a pure motor cause of IE, would achieve full binocular vision with 60 arc seconds stereopsis by very early surgery.
11.3.7Predictors of Spontaneous Reduction into Microstrabismus
In the ELISSS, of all parameters assessed in the baseline examination at approximately 11 months, only the angle of strabismus at baseline predicted, to some extent, whether a child had been operated at the age of 6 years or not (Fig. 11.9). Among children with an angle equal or smaller than 13° at baseline at approximately 11 months, 34.9% had not been operated at the age of 6 years. Hypermetropia around spher. + 4 increased the likelihood of regression without surgery, emphasising the need for full refractive correction (there may have been some very early cases of accommodative esotropia). Age at recruitment, age that strabismus reportedly had started and degree of amblyopia at baseline examination seemed not predictive.
11.3.8Random-E ects Model Predicting the Angle and its Variation
In the 532 children of the ELISSS, the angle of strabismus, refraction, and visual acuity was assessed at baseline at approximately 11 months and every 6 months thereafter, until the final evaluation at the age of 6 years. The resulting, slightly more than 6,000, orthoptic exams were used to construct a random-e ects model [61] that forecasts the expected angle and its variation years ahead, on the basis of one or more measurements of the angle and refraction in infancy.
Angles of strabismus measured at di erent ages and the refraction of the patient can be entered in the model. On entering successive measurements of the angle of strabismus, the model adjusts the slope, i.e., yearly increase or decrease of the expected angle, according to the trend. The uncertainty about the slope decreases with additional measurements because the random e ect of the slope of the lines decreases. The uncertainty about the slope is compounded by additional variation of the angle around this slope for an individual child (Fig. 11.10).
In simulations with the random-e ects model, it was found that the chance of a spontaneous reduction of a strabismus into a microstrabismus is considerable when an angle of strabismus 14° or less is found repeatedly at the age of 1 or 2 years. In the ELISSS, esotropia 13° or less at baseline at approximately 11 months of age had not been operated at the age of 6 years in 35% of the cases (Fig. 11.7). If the angle is large on multiple measurements, the chance that the esotropia will decrease into a microstrabismus spontaneously is very small.
11.3 Number of Operations and Spontaneous Reduction into Microstrabismus Without Surgery |
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Fig. 11.8 T he upper panel shows the 6-monthly measurements of the angle of strabismus in those ELISSS children who had been scheduled for early surgery at baseline at approximately 11 months of age, but had not been operated at the age of 6 years (14, 8.2%). The lower panel shows these measurements for the children who had been scheduled for late surgery, but had not been operated at the age of 6 years (47, 20.1%). These children correspond to the white bars in Figs. 11.1, 11.2, and 11.9
148 |
11 Best Age for Surgery for Infantile Esotropia |
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Fig. 11.9 Angle of strabismus at baseline at approximately 11 months for all 414 operated (black) and unoperated (white) patients who underwent the final examination at the age of 6 years (same group as in Figs. 11.1 & 11.2). Children who had not been operated at the age of 6 years (white bars) had had smaller angles at baseline (See Ref. [57])
In the model, refractive error exerted its largest influence, i.e., causing the largest chance of spontaneous reduction into a microstrabismus, at a spher. + 4. Some children in the ELISSS study population may actually have been very early cases of accommodative esotropia. In case of hypermetropia, especially with convergence excess, a large reduction in the angle may occur after fitting full correcting glasses, thereby avoiding surgery.
Summary for the Clinician
■The chance of a spontaneous reduction of the esotropia into microstrabismus is considerable when an angle of strabismus of 13° or less is found repeatedly at the age of 1 year.
■Fit full-correcting glasses in case of hypermetropia accompanying esotropia at an early age because a large reduction of the angle of strabismus can be achieved without surgery and with better binocular vision.
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Fig. 11.10 Random-e ects model predicting the angle and its variation based on one or more measurements of the angle and refraction in infancy. For the construction of this model, the random e ect for a patient was defined as the deviation of the average angle, the fixed e ect.A vector was defined based on age and spherical equivalent of the patient.A covariance matrix of the random- e ects estimations was defined and filled with the values from the approximately 6,000 orthoptic exams in 532 children. The model predicts the average angle in relation to age. A linear relation su ced. The variance around the prediction (curved lines represent one and two standard deviations) consists of uncertainty in the estimations, random e ects and the residuals. Left: an example prediction based on three increasing angles measured at 9, 12 and 15 months. Right: an example prediction where the angle decreases in successive measurements; the chance that spontaneous reduction into a microstrabismus occurs is considerable
