Pediatric Ophthalmology, Neuro-Ophthalmology, Genetics (eBook)

Foreword 6
Preface 7
Contents 8
Contributors 14
Chapter 1 16
Epidemiology of Pediatric Strabismus 16
1.1 Introduction 16
1.2 Forms of Pediatric Strabismus 16
1.2.1 Esodeviations 16
1.2.1.1 Congenital Esotropia 17
1.2.1.2 Accommodative Esotropia 17
1.2.1.3 Acquired Nonaccommodative Esotropia 17
1.2.1.4 Abnormal Central Nervous System Esotropia 17
1.2.1.5 Sensory Esotropia 17
1.2.2 Exodeviations 18
1.2.2.1 Intermittent Exotropia 18
1.2.2.2 Congenital Exotropia 18
1.2.2.3 Convergence Insufficiency 18
1.2.2.4 Abnormal Central Nervous System Exotropia 18
1.2.2.5 Sensory Exotropia 18
1.2.3 Hyperdeviations 18
1.3 Strabismus and Associated Conditions 19
1.4 Changing Trends in StrabismusEpidemiology 19
1.4.1 Changes in Strabismus Prevalence 19
1.4.2 Changes in Strabismus Surgery Rates 19
1.5 Worldwide Incidence and Prevalence of Childhood Strabismus 19
1.6 Incidence of Adult Strabismus 22
References 22
Chapter 2 25
Changes in Strabismus Over Time: The Roles of Vergence Tonus and Muscle Length Adaptation1 25
2.1 Binocular Alignment System 25
2.1.1 Long-Term Maintenance of Binocular Alignment 25
2.1.2 Vergence Adaptation 26
2.1.3 Muscle Length Adaptation 26
2.2 Modeling the Binocular Alignment Control System 27
2.2.1 Breakdown of the Binocular Alignment Control System 28
2.2.2 Clarifi cation of Unanswered Questions Regarding the Long-Term Binocular Alignment Control System 28
2.2.3 Changes in Strabismus as a Bilateral Phenomenon 28
2.2.4 Changes in Basic Muscle Length 29
2.2.5 Version Stimulation and Vergence Stimulation 30
2.2.6 Evidence Against the “Final Common Pathway” 31
2.3 Changes in Strabismus 32
2.3.1 Diagnostic Occlusion: And the Hazard of Prolonged Occlusion 33
2.3.2.1 Supporting Evidence for Bilateral Feedback Control of Muscle Lengths 33
2.4 Applications of Bilateral Feedback Control to Clinical Practice and to Future Research 35
References 36
Chapter 3 39
A Dissociated Pathogenesis for Infantile Esotropia 39
3.1 Dissociated Eye Movements 39
3.2 Tonus and its relationship to infantile esotropia 39
3.3 Eaotropia and Exotropia as a Continuum 40
3.4 Distiguishing Esotonus from Convergence 42
3.5 Pathogenetic Role of Dissociated Eye Movements in Infantile Esotropia 43
References 44
Chapter 4 46
The Monofixation Syndrome: New Considerations on Pathophysiology 46
4.1 Introduction 46
4.2 Nirmal and Anomalous Binocular Vision 46
4.2.1 Binocular Correspondence: Anomalous, Normal, or Both? 47
4.3 MFS with Manifest Strabismus 48
4.3.1 Esotropia is the Most Common Form of MFS 48
4.3.2 Esotropia Allows for Better Binocular Vision 48
4.3.3 Esotropia is the Most Stable Form 49
4.4 Repairing and Producing MFS 49
4.4.1 Animal Models for the Study of MFS 50
4.5 Primary MFS (Sensory Signs of Infantile-Onset Image Decorrelation) 51
4.5.1 Motor Signs of Infantile-Onset Image Decorrelation 51
References 52
Chapter 5 54
Visual Cortex Mechanisms of Strabismus: Development and Maldevelopment 54
5.1 Esotropia as the Major Type of Developmental Strabismus 54
5.1.1 Early-Onset (Infantile) Esotropia 54
5.1.2 Early Cerebral Damage as the Major Risk Factor 54
5.1.3 Cytotoxic Insults to Cerebral Fibers 55
5.1.4 Genetic Infl uences on Formation of Cerebral Connections 55
5.1.5 Development of Binocular Visuomotor Behavior in Normal Infants 55
5.1.6 Development of Sensorial Fusion and Stereopsis 56
5.1.7 Development of Fusional Vergence and an Innate Convergence Bias 57
5.1.8 Development of Motion Sensitivity and Conjugate Eye Tracking (Pursuit/OKN) 57
5.1.9 Development and Maldevelopment of Cortical Binocular Connections 57
5.1.10 Binocular Connections Join Monocular Compartments Within Area V1 (Striate Cortex) 57
5.1.11 Too Few Cortical Binocular Connections in Strabismic Primate 59
5.1.12 Projections from Striate Cortex (Area V1) to Extrastriate Cortex (Areas MT/MST) 59
5.1.13 Inter-Ocular Suppression Rather than Cooperation in Strabismic Cortex 59
5.1.14 Naso-Temporal Inequalities of Cortical Suppression 60
5.1.15 Persistent Nasalward Visuomotor Biases in Strabismic Primate 60
5.1.16 Repair of Strabismic Human Infants: The Historical Controversy 63
5.1.17 Repair of High-grade Fusion is Possible 63
5.1.18 Timely Restoraion of Correlated Binocular Input: The Key to Repair 63
5.2 Visual Cortex Mechanisms in Micro-Esotropia (Monofi xation Syndrome) 64
5.2.1 Neuroanatomic Findings in Area V1 of Micro-Esotropic Primates 65
5.2.2 Extrastriate Cortex in Micro-Esotropa 65
References 67
Chapter 6 71
Neuroanatomical Strabismus 71
6.1 General Etiologies of Strabismus 71
6.2 Extraocular Myopathy 71
6.2.1 Primary EOM Myopathy 71
6.2.2 Immune Myopathy 72
6.2.3 Infl ammatory Myositis 73
6.2.4 Neoplastic Myositis 73
6.2.5 Traumatic Myopathy 73
6.3 Congenital Pulley Heterotopy 74
6.4 Acquired Pulley Heterotopy 75
6.5 “Divergence Paralysis” Esotropia 76
6.5.1 Vertical Strabismus Due to Sagging Eye Syndrome 77
6.5.2 Postsurgical and Traumatic Pulley Heterotopy 77
6.5.3 Axial High Myopia 77
6.6 Congenital Peripheral Neuropathy: The Congenital Cranial Dysinnervation Disorders (CCDDs) 78
6.6.1 Congenital Oculomotor (CN3) Palsy 79
6.6.2 Congenital Fibrosis of the Extraocular Muscles (CFEOM) 79
6.6.3 Congenital Trochlear (CN4) Palsy 81
6.6.4 Duane’s Retraction Syndrome (DRS) 81
6.6.5 Moebius Syndrome 82
6.7 Acquired Motor Neuropathy 83
6.7.1 Oculomotor Palsy 83
6.7.2 Trochlear Palsy 83
6.7.3 Abducens Palsy 83
6.7.4 Inferior Oblique (IO) Palsy 83
6.8 Central Abnormalities of Vergence and Gaze 84
6.8.1 Developmental Esotropia and Exotropia 84
6.8.2 Cerebellar Disease 84
6.8.3 Horizontal Gaze Palsy and Progressive Scoliosis 84
References 84
Chapter 7 88
Congenital Cranial Dysinnervation Disorders: Facts and Perspectives to Understand Ocular Motility Disorders 88
7.1 Congenital Cranial Dysinnervation Disorders: Facts About Ocular Motility Disorders 88
7.1.1 The Concept of CCDDs: Ocular Motility Disorders as Neurodevelopmental Defects 88
7.1.1.1 Brainstem and Cranial Nerve Development 89
7.1.1.2 Single Disorders Representing CCDDs 89
7.1.1.3 Disorders Understood as CCDDs 92
7.2 Congenital Cranial Dysinnervation Disorders: Perspectives to Understand Ocular Motility Disorders 94
7.2.1 Congenital Ocular Elevation Defi ciencies: A Neurodevelopmental View 94
7.2.1.1 Brown Syndrome 94
Motility Findings 94
Saccadic Eye Movements 96
Comorbidity 96
Epidemiologic Features 96
Laterality 96
Sex Distribution 97
Incidence 97
Heredity 97
Potential Induction of the Syndrome 97
Radiologic Findings 98
Natural Course in Brown Syndrome 98
Intra-and Postoperative Findings 98
7.2.1.2 Congenital Monocular Elevation Deficiency and Vertical Retraction Syndrome 98
7.2.2 A Model of some Congenital Elevation Deficiencies as Neurodevelopmental Diseases 100
References 102
Chapter 8 106
The Value of Screening for Amblyopia Revisited 106
8.1 Amblyopia 106
8.2 What Is Screening? 107
8.2.1 Screening for Amblyopia, Strabismus, and/or Refractive Errors 107
8.2.1.1 Screening for Amblyopia 108
8.2.1.2 Screening for Strabismus 108
8.2.1.3 Screening for Refractive Error 108
8.2.1.4 Screening for Other Ocular Conditions 108
8.2.2 Difference Between a Screening and Diagnostic Test 108
8.2.3 Justification for Screening for Amblyopia and/or Strabismus 109
8.2.4 Recent Reports Examining Pre-School Vision Screening 109
8.3 Screening Tests for Amblyopia, Strabismus, and/or Refractive Error 111
8.3.1 Vision Tests 111
8.3.2 Cover-Uncover Test 111
8.3.3 Stereoacuity 112
8.3.4 Photoscreening and/or Autorefraction 112
8.3.5 What to Do with Those Who Are Unable to Perform Screening Tests? 113
8.3.6 Who Should Administer the Screening Program? 113
8.4 Treatment of Amblyopia 114
8.4.1 Type of Treatment 114
8.4.2 Refractive Adaptation 114
8.4.3 Conventional Occlusion 115
8.4.4 Pharmacological Occlusion 115
8.4.5 Optical Penalization 115
8.4.6 Effective Treatment of Amblyopia in Older Children (Over the Age of 7 Years) 115
8.4.7 Treatment Compliance 116
8.4.8 Other Treatment Options for Amblyopia 116
8.4.9 Recurrence of Amblyopia Following Therapy 116
8.5 Quality of Life 117
8.5.1 The Impact of Amblyopia Upon HRQoL 117
8.5.2 Stereoacuity and Motor Skills in Children with Amblyopia 117
8.5.3 Reading Speed and Reading Ability in Children with Amblyopia 117
8.5.4 Impact of Amblyopia Upon Education 117
8.5.5 Emotional Well-Being and Amblyopia 118
8.5.6 The Impact of Strabismus Upon HRQoL 118
8.5.7 Critique of HRQoL Issues in Amblyopia 119
8.5.8 The Impact of the Condition or the Impact of Treatment? 119
References 120
Chapter 9 123
The Brückner Test Revisited 123
9.1 Amblyopia and Amblyogenic Disorders 123
9.1.1 Early Detection of Amblyopia 123
9.1.2 Brückner’s Original Description 124
9.2 Corneal Light Reflexes (First Purkinje Images) 124
9.2.1 Physiology 124
9.2.2 Performance 125
9.2.3 Shortcomings and Pitfalls 125
9.3 Fundus Red Reflex (Brückner Reflex) 125
9.3.1 Physiology 126
9.3.2 Performance 129
9.3.3 Possibilities and Limitations 130
9.4 Pupillary Light Reflexes 130
9.4.1 Physiology 131
9.4.2 Performance 131
9.4.3 Possibilities and Limitations 131
9.5 Eye Movements with Alternating Illumination of the Pupils 132
References 132
Chapter 10 135
Amblyopia Treatment 2009 135
10.1 Amblyopia Treatment 2009 135
10.1.1 Introduction 135
10.1.2 Epidemiology 135
10.1.3 Clinical Features of Amblyopia 136
10.1.4 Diagnosis of Amblyopia 136
10.1.5 Natural History 137
10.2 Amblyopia Management 137
10.2.1 Refractive Correction 137
10.2.2 Occlusion by Patching 138
10.2.3 Pharmacological Treatment with Atropine 139
10.2.4 Pharmacological Therapy Combined with a Plano Lens 140
10.3 Other Treatment Issues 141
10.3.1 Bilateral Refractive Amblyopia 141
10.3.2 Age Effect 141
10.3.3 Maintenance Therapy 141
10.3.4 Long-Term Persistence of anAmblyopia Treatment Benefi t 142
10.4 Other Treatments 142
10.4.1 Filters 142
10.4.2 Levodopa/Carbidopa Adjunctive Therapy 143
10.5 Controversy 143
10.5.1 Optic Neuropathy Rather than Amblyopia 143
References 144
Chapter 11 147
Best Age for Surgery for Infantile Esotropia: Lessons from the Early vs. Late Infantile Strabismus Surgery Study 147
11.1 Introduction 147
11.1.1 Definition and Prevalence 147
11.1.2 Sensory or Motor Etiology 147
11.1.3 Pathogenesis: Lack of Binocular Horizontal Connections in the Visual Cortex 148
11.1.4 History 148
11.1.5 Outcome Parameters 148
11.2 Outcome of Surgery in the ELISSS 149
11.2.1 Reasons for the ELISSS 149
11.2.2 Summarized Methods of the ELISSS 149
11.2.3 Summarized Results of the ELISSS 150
11.2.4 Binocular Vision at Age Six 150
11.2.5 Horizontal Angle of Strabismus at Age Six 150
11.2.6 Alignment is Associated with Binocular Vision 151
11.3 Number of Operations and Spontaneous Reduction into Microstrabismus Without Surgery 152
11.3.1 The Number of Operations Per Child and the Reoperation Rate in the ELISSS 152
11.3.2 Reported Reoperation Rates 152
11.3.3 Test-Retest Reliability Studies 154
11.3.4 Relation Between the Postoperative Angle of Strabismus and the Reoperation Rate 155
11.3.5 Scheduled for Surgery, but no Surgery Done at the End of the Study at the Age of Six Years 155
11.3.6 Spontaneous Reduction of the Angle 156
11.3.7 Predictors of Spontaneous Reduction into Microstrabismus 156
11.3.8 Random-Effects Model Predicting the Angle and its Variation 156
Appendix 159
References 159
Chapter 12 162
Management of Congenital Nystagmus with and without Strabismus 162
12.1 Overview 163
12.1.1 Congenital Nystagmus with and Without Sensory Deficits 163
12.1.1.1 The Clinical Characteristics of Congenital Nystagmus 165
12.1.2 Manifest Latent Nystagmus (MLN) 166
12.1.2.1 Clinical Characteristics of Manifest Latent Nystagmus (MLN) 166
12.1.3 Congenital Periodic Alternating Nystagmus (PAN) 167
12.1.3.1 Clinical characteristics of congenital periodic alternating nystagmus 168
12.2 Compensatory Mechanisms 169
12.2.1 Dampening by Versions 169
12.2.2 Dampening by Vergence 169
12.2.3 Anomalous Head Posture (AHP) 169
12.2.3.4 Measurement of AHP 169
12.2.3.5 Effect of Monocular and Binocular Visual Acuity Testing on AHP 170
12.2.3.6 Testing AHP at Near 171
12.2.3.7 The Effect of Straightening the Head in Patients with AHP 171
12.3 Treatment 171
12.3.1 Optical Treatment 171
12.3.1.1 Refractive Correction 171
12.3.1.2 Spectacles and Contact Lenses (CL) 171
12.3.1.3 Prisms 172
12.3.1.4 Low Visual Aids 172
12.3.2 Medication 172
12.3.3 Acupuncture 173
12.3.4 Biofeedback 173
12.3.5 Botulinum Toxin-A (Botox) 173
12.3.6 Surgical Treatment of Congenital Nystagmus 173
12.3.6.1 Management of Horizontal AHP 174
12.3.6.2 Management of Vertical AHP 175
12.3.6.3 Management of Head Tilt 176
12.3.6.4 Artificial Divergence Surgery 176
12.3.6.5 Surgery to Decreasethe Intensity of Nystagmus 177
Retro-Equatorial Recession of Horizontal Rectus Muscles 177
The Tenotomy Procedure 178
References 178
Chapter 13 181
Surgical Management of Dissociated Deviations 181
13.1 Dissociated Deviations 182
13.2 Surgical Alternatives to Treat Patients with DVD 183
13.2.1 Symmetric DVD with Good Bilateral Visual Acuity, with No Oblique Muscles Dysfunction 183
13.2.2 Bilateral DVD with Deep Unilateral Amblyopia 183
13.2.3 DVD with Inferior Oblique Overaction (IOOA) and V Pattern 184
13.2.4 DVD with Superior Oblique Overaction (SOOA) and A Pattern 185
13.2.5 Symmetric vs. Asymmetric Surgeries for DVD 186
13.2.6 DVD with Hypotropia of the Nonfixating Eye 186
13.3 Dissociated Horizontal Deviation 187
13.4 Dissociated Torsional Deviation. Head tilts in patients with Dissociated Strabismus 188
13.5 Conclusions 190
References 190
Chapter 14 193
Surgical Implications of the Superior Oblique Frenulum 193
14.1 Introduction 193
14.2 Clinical and Theoretical Investigations 194
14.2.1 The Effect of Superior Rectus Muscle Recession on the Location of the Superior Oblique Tendon Before and After Cutting the Frenulum 194
14.2.2 The Effect of the Frenulum on Superior Oblique Recession Using a Suspension Technique 196
14.2.3 The Theoretical Effect of the Superior Oblique Frenulum on the Posterior Partial Tenectomy of the Superior Oblique 197
References 200
Chapter 15 202
Pearls and Pitfalls in Surgical Management of Paralytic Strabismus 202
15.1 General Principles of Surgical Treatment in Paralytic Strabismus 202
15.1.1 Aims of Treatment 202
15.1.2 Timing of Surgery 202
15.1.3 Preoperative Assessment 203
15.1.4 Methods of Surgical Treatment 204
15.2 Third Nerve Palsy 205
15.2.1 Complete Third Nerve Palsy 205
15.2.2 Incomplete Third Nerve Palsy 206
15.3 Fourth Nerve Palsy 207
15.4 Sixth Nerve Palsy 211
References 212
Chapter 16 214
Modern Treatment Concepts in Graves Disease 214
16.1 Graves Orbitopathy (GO): Pathogenesis and Clinical Signs 214
16.1.1 Graves Orbitopathy is Part of a Systemic Disease: Graves Disease (GD) 214
16.1.2 Graves Orbitopathy Clinical Signs 215
16.1.2.1 Clinical Changes Result in Typical Symptoms 215
16.1.3 Clinical Examination of GO 215
16.1.3.1 Signs of Activity 215
16.1.3.2 Assessing Severity of GO 216
16.1.3.3 Imaging 218
16.1.4 Classification of GO 218
16.2 Natural History 219
16.3 Treatment of GO 220
16.3.1 Active Inflammatory Phase 220
16.3.1.1 Glucocorticoid Treatment 220
16.3.1.2 Orbital Radiotherapy 220
16.3.1.3 Combined Therapy: Glucocorticoids and Orbital Radiotherapy 220
16.3.1.4 Other Immunosuppressive Treatments and New Developments 220
16.3.1.5 Therapy of Dysthyroid Optic Neuropathy (DON) and Sight-Threatening Corneal Breakdown 221
16.3.1.6 Other Simple Measures that may Alleviate Symptoms 221
16.3.2 Inactive Disease Stages 222
16.3.2.1 Orbital Decompression 222
16.3.2.2 Extraocular Muscle Surgery 223
16.3.2.3 Lid Surgery 224
16.4 Thyroid Dysfunction and GO 227
16.4.1 Association Between Treatment of Hyperthyroidism and Course of GO 227
16.4.2 Relationship Between TSH-Receptor-Antibody (TRAb) Levels and Orbitopathy 227
16.5 Environmental and Genetic Influence on the Course of GO 228
16.5.1 Relationship Between Cigarette Smoking and Graves Orbitopathy 228
16.5.2 Genetic Susceptibility 228
16.6 Special Situations 229
16.6.1 Euthyroid GO 229
16.6.2 Childhood GO 229
16.6.3 GO and Diabetes 229
References 230
Index 233