Pediatric Ophthalmology, Neuro-Ophthalmology, Genetics: Strabismus - New Concepts in Pathophysiology, Diagnosis, and Treatment

237 Pages·2010·14.777 MB·English

by  Amy E. Green-Simms, Brian G. Mohney (auth.), Birgit Lorenz, Michael C. Brodsky (eds.)

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Essentials in Ophthalmology Pediatric Ophthalmology,
Neuro-Ophthalmology, Genetics

B. Lorenz M. C. Brodsky
Editors
Essentials in Ophthalmology Glaucoma
G. K. Krieglstein R. N. Weinreb Cataract and Refractive Surgery
Series Editors
Uveitis and Immunological Disorders
Vitreo-retinal Surgery
Medical Retina
Oculoplastics and Orbit
Pediatric Ophthalmology,
Neuro-Ophthalmology, Genetics
Cornea and External Eye Disease
Editors Birgit Lorenz
Michael C. Brodsky
Pediatric
Ophthalmology,
Neuro-
Ophthalmology,
Genetics
Strabismus - New Concepts in
Pathophysiology, Diagnosis,
and Treatment
Series Editors Volume Editors
Günter K. Krieglstein, MD Birgit Lorenz, MD
Professor and Chairman Professor of Ophthalmology
Department of Ophthalmology Klinik und Poliklinik für
University of Cologne Augenheilkunde
Joseph-Stelzmann-Straße 9 Justus-Liebig-University
50931 Köln UKGM GmbH Giessen Campus
Germany Friedrichstraβe 18
35392 Giessen
Robert N. Weinreb, MD Germany
Professor and Director
Hamilton Glaucoma Center Michael C. Brodsky, MD
Department of Ophthalmology Professor of Ophthalmology and Neurology
University of California at San Diego Mayo Clinic
9500 Gilman Drive Department of Ophthalmology
La Jolla, CA 92093-0946 200 First Street SW
USA Rochester, MN 55905
USA
ISBN: 978-3-540-85850-8 e-ISBN: 978-3-540-85851-5
DOI: 10.1007/978-3-540-85851-5
Library of Congress Control Number: 2009938957
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Foreword
Th e Essentials in Ophthalmology series represents an ership acceptance of the fi rst two series, each of eight
unique updating publication on the progress in all sub- volumes. Th is is a success that was made possible pre-
specialties of ophthalmology. dominantly by the numerous opinion-leading authors
In a quarterly rhythm, eight issues are published cov- and the outstanding section editors, as well as with the
ering clinically relevant achievements in the whole fi eld constructive support of the publisher. Th ere are many
of ophthalmology. Th is timely transfer of advancements good reasons to continue and still improve the dissemina-
for the best possible care of our eye patients has proven to tion of this didactic and clinically relevant information.
be eff ective. Th e initial working hypothesis of providing
new knowledge immediately following publication in the
peer-reviewed journal and not waiting for the textbook
appears to be highly workable. G.K. Krieglstein
We are now in the third cycle of the Essentials in R.N. Weinreb
Ophthalmology series, having been encouraged by read- Series Editors
Preface
Th e fi eld of strabismology has long suff ered from a dis- philosophy of strabismus that integrates new concepts of
crepancy between its levels of sophistication in practice pathogenesis into the clinic.
and theory. Although its diagnostic and therapeutic arma- Th is book provides a compendium of chapters that
mentarium has become quite advanced, the scientifi c highlight new ideas in the fi eld of strabismus. We have
understanding of disease pathogenesis has remained rudi- assembled an international panel of contributors who
mentary. Consequently, educational training in strabismus have advanced our understanding of strabismus patho-
diagnosis and treatment has become a didactic exercise in genesis. Some chapters are new while others are derived
“learning the rules.” from recent seminal articles that have challenged our
Recent advances in epidemiology, neuroimaging, understanding of strabismus diagnosis and treatment.
genetics, and neurobiology have revolutionized our Original sources for these chapters are appropriately
understanding of strabismus. Conceptualizing strabis- acknowledged. We thank our innovative authors for their
mus within an evolutionary framework has advanced our important contributions, and hope that the reader fi nds
understanding of why it arises and provided new clues to this edition both stimulating and enlightening.
its neurological underpinnings. As new information is
Birgit Lorenz
consolidated, we are beginning to formulate a unifi ed Michael C. Brodsky
Contents
Chapter 1 2.1.3 Muscle Length Adaptation . . . . . . . . . . . . . 12
Epidemiology of Pediatric Strabismus 2.2 Modeling the Binocular
Amy E. Green-Simms and Brian G. Mohney Alignment Control System. . . . . . . . . . . . . . 13
2.2.1 Breakdown of the Binocular
1.1 Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . 1 Alignment Control System. . . . . . . . . . . . . . 14
1.2 Forms of Pediatric Strabismus . . . . . . . . . . 1 2.2.2 Clarifi cation of Unanswered
1.2.1 Esodeviations. . . . . . . . . . . . . . . . . . . . . . . . . . 1 Questions Regarding the
1.2.1.1 Congenital Esotropia. . . . . . . . . . . . . . . . . . . 2 Long-Term Binocular Alignment
1.2.1.2 Accommodative Esotropia. . . . . . . . . . . . . . 2 Control System. . . . . . . . . . . . . . . . . . . . . . . . . 14
1.2.1.3 Acquired Nonaccommodative
2.2.3 Changes in Strabismus as
Esotropia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2 Bilateral Phenomena . . . . . . . . . . . . . . . . . . . 14
1.2.1.4 Abnormal Central Nervous System
2.2.4 Changes in Basic Muscle Length . . . . . . . . 15
Esotropia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2 2.2.5 Version Stimulation and
1.2.1.5 Sensory Esotropia. . . . . . . . . . . . . . . . . . . . . . 2 Vergence Stimulation . . . . . . . . . . . . . . . . . . 16
1.2.2 Exodeviations. . . . . . . . . . . . . . . . . . . . . . . . . . 3 2.2.6 Evidence Against the “Final
1.2.2.1 Intermittent Exotropia. . . . . . . . . . . . . . . . . . 3 Common Pathway”. . . . . . . . . . . . . . . . . . . . . 17
1.2.2.2 Congenital Exotropia. . . . . . . . . . . . . . . . . . . 3 2.3 Changes in Strabismus . . . . . . . . . . . . . . . . . 18
1.2.2.3 Convergence Insuffi ciency. . . . . . . . . . . . . . 3 2.3.1 Diagnostic Occlusion: And the
1.2.2.4 Abnormal Central Nervous System
Hazard of Prolonged Occlusion . . . . . . . . . 19
Exotropia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3 2.3.2 Unilateral Changes in Strabismus . . . . . . . 19
1.2.2.5 Sensory Exotropia. . . . . . . . . . . . . . . . . . . . . . 3 2.3.2.1 Supporting Evidence for Bilateral
1.2.3 Hyperdeviations . . . . . . . . . . . . . . . . . . . . . . . 3 Feedback Control of Muscle Lengths. . . . 19
1.3 Strabismus and Associated
2.4 Applications of Bilateral Feedback
Conditions. . . . . . . . . . . . . . . . . . . . . . . . . . . . . 4 Control to Clinical Practice and
1.4 Changing Trends in Strabismus
to Future Research . . . . . . . . . . . . . . . . . . . . . 21
Epidemiology. . . . . . . . . . . . . . . . . . . . . . . . . . 4 References. . . . . . . . . . . . . . . . . . . . . . . . . . . . . 22
1.4.1 Changes in Strabismus Prevalence. . . . . . 4
1.4.2 Changes in Strabismus Surgery Rates . . . 4 Chapter 3
1.5 Worldwide Incidence and Prevalence A Dissociated Pathogenesis
of Childhood Strabismus . . . . . . . . . . . . . . . 4 for Infantile Esotropia
1.6 Incidence of Adult Strabismus . . . . . . . . . . 7 Michael C. Brodsky
References. . . . . . . . . . . . . . . . . . . . . . . . . . . . . 7
3.1 Dissociated Eye Movements . . . . . . . . . . . . 25
3.2 Tonus and its relationship
Chapter 2
to infantile esotropia . . . . . . . . . . . . . . . . . . . 25
Changes in Strabismus Over Time: The Roles of
Vergence Tonus and Muscle Length Adaptation 3.3 Esotropia and Exotropia as
a Continuum. . . . . . . . . . . . . . . . . . . . . . . . . . . 26
David L. Guyton
3.4 Distinguishing Esotonus
2.1 Binocular Alignment System. . . . . . . . . . . . 11 from Convergence . . . . . . . . . . . . . . . . . . . . . 28
2.1.1 Long-Term Maintenance 3.5 Pathogenetic Role of Dissociated
of Binocular Alignment. . . . . . . . . . . . . . . . . 11 Eye Movements in Infantile Esotropia . . . 29
2.1.2 Vergence Adaptation. . . . . . . . . . . . . . . . . . . 12 References. . . . . . . . . . . . . . . . . . . . . . . . . . . . . 30
x Contents
Chapter 4 5.1.10 Binocular Connections Join
The Monofi xation Syndrome: New Monocular Compartments Within
Considerations on Pathophysiology Area V1 (Striate Cortex). . . . . . . . . . . . . . . . . 44
Kyle Arnoldi 5.1.11 Too Few Cortical Binocular
Connections in Strabismic Primate. . . . . . 46
4.1 Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . 33
5.1.12 Projections from Striate Cortex
4.2 Normal and Anomalous
(Area V1) to Extrastriate Cortex
Binocular Vision. . . . . . . . . . . . . . . . . . . . . . . . 33
(Areas MT/MST) . . . . . . . . . . . . . . . . . . . . . . . . 46
4.2.1 Binocular Correspondence:
5.1.13 Inter-Ocular Suppression
Anomalous, Normal, or Both?. . . . . . . . . . . 34
Rather than Cooperation
4.3 MFS with Manifest Strabismus. . . . . . . . . . 35
in Strabismic Cortex. . . . . . . . . . . . . . . . . . . . 46
4.3.1 Esotropia is the Most Common
5.1.14 Naso-Temporal Inequalities
form of MFS. . . . . . . . . . . . . . . . . . . . . . . . . . . . 35
of Cortical Suppression. . . . . . . . . . . . . . . . . 47
4.3.2 Esotropia Allows for Better
5.1.15 Persistent Nasalward
Binocular Vision. . . . . . . . . . . . . . . . . . . . . . . . 35
Visuomotor Biases in Strabismic
4.3.3 Esotropia is the Most Stable Form. . . . . . . 36
Primate . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 47
4.4 Repairing and Producing MFS . . . . . . . . . . 36
5.1.16 Repair of Strabismic Human
4.4.1 Animal Models for the Study
Infants: The Historical Controversy . . . . . . 50
of MFS. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 37
5.1.17 Repair of High-grade Fusion
4.5 Primary MFS (Sensory Signs of
is Possible. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 50
Infantile-Onset Image Decorrelation) . . . 38
5.1.18 Timely Restoraion of Correclated
4.5.1 Motor Signs of Infantile-Onset
Binocular Input: The Key to Repair . . . . . . 50
Image Decorrelation . . . . . . . . . . . . . . . . . . . 38
5.2 Visual Cortex Mechanisms
References. . . . . . . . . . . . . . . . . . . . . . . . . . . . . 39
in Micro-Esotropia (Monofi xation
Syndrome). . . . . . . . . . . . . . . . . . . . . . . . . . . . . 51
5.2.1 Neuroanatomic Findings in
Chapter 5
Area V1 of Micro-Esotropic Primates . . . . 52
Visual Cortex Mechanisms of Strabismus:
5.2.2 Extrastriate Cortex in
Development and Maldevelopment
Micro-Esotropa. . . . . . . . . . . . . . . . . . . . . . . . . 52
Lawrence Tychsen
References. . . . . . . . . . . . . . . . . . . . . . . . . . . . . 54
5.1 Esotropia as the Major Type
Chapter 6
of Developmental Strabismus . . . . . . . . . . 41
Neuroanatomical Strabismus
5.1.1 Early-Onset (Infantile) Esotropia . . . . . . . . 41
Joseph L. Demer
5.1.2 Early Cerebral Damage as
the Major Risk Factor . . . . . . . . . . . . . . . . . . . 41 6.1 General Etiologies of Strabismus. . . . . . . . 59
5.1.3 Cytotoxic Insults to Cerebral Fibers. . . . . . 42 6.2 Extraocular Myopathy. . . . . . . . . . . . . . . . . . 59
5.1.4 Genetic Infl uences on 6.2.1 Primary EOM Myopathy . . . . . . . . . . . . . . . . 59
Formation of Cerebral Connections . . . . . 42 6.2.2 Immune Myopathy. . . . . . . . . . . . . . . . . . . . . 60
5.1.5 Development of Binocular 6.2.3 Infl ammatory Myositis. . . . . . . . . . . . . . . . . . 61
Visuomotor Behavior 6.2.4 Neoplastic Myositis. . . . . . . . . . . . . . . . . . . . . 61
in Normal Infants. . . . . . . . . . . . . . . . . . . . . . . 42 6.2.5 Traumatic Myopathy . . . . . . . . . . . . . . . . . . . 61
5.1.6 Development of Sensorial 6.3 Congenital Pulley Heterotopy . . . . . . . . . . 62
Fusion and Stereopsis . . . . . . . . . . . . . . . . . . 43 6.4 Acquired Pulley Heterotopy . . . . . . . . . . . . 63
5.1.7 Development of Fusional 6.5 “Divergence Paralysis” Esotropia . . . . . . . . 64
Vergence and an Innate 6.5.1 Vertical Strabismus Due to
Convergence Bias . . . . . . . . . . . . . . . . . . . . . . 44 Sagging Eye Syndrome. . . . . . . . . . . . . . . . . 65
5.1.8 Development of Motion 6.5.2 Postsurgical and Traumatic Pulley
Sensitivity and Conjugate Heterotopy . . . . . . . . . . . . . . . . . . . . . . . . . . . . 65
Eye Tracking (Pursuit/OKN) . . . . . . . . . . . . . 44 6.5.3 Axial High Myopia. . . . . . . . . . . . . . . . . . . . . . 65
5.1.9 Development and 6.6 Congenital Peripheral Neuropathy:
Maldevelopment of Cortical The Congenital Cranial
Binocular Connections . . . . . . . . . . . . . . . . . 44 Dysinnervation Disorders (CCDDs). . . . . . 66
Contents xi
6.6.1 Congenital Oculomotor (CN3) Palsy. . . . . 67 Chapter 8
6.6.2 Congenital Fibrosis of the The Value of Screening for Amblyopia Revisited
Extraocular Muscles (CFEOM). . . . . . . . . . . 67 Jill Carlton and Carolyn Czoski-Murray
6.6.3 Congenital Trochlear (CN4) Palsy. . . . . . . . 69
6.6.4 Duane’s Retraction 8.1 Amblyopia. . . . . . . . . . . . . . . . . . . . . . . . . . . . . 95
Syndrome (DRS). . . . . . . . . . . . . . . . . . . . . . . . 69 8.2 What Is Screening? . . . . . . . . . . . . . . . . . . . . . 96
8.2.1 Screening for Amblyopia,
6.6.5 Moebius Syndrome . . . . . . . . . . . . . . . . . . . . 70
Strabismus, and/or
6.7 Acquired Motor Neuropathy. . . . . . . . . . . . 71
6.7.1 Oculomotor Palsy . . . . . . . . . . . . . . . . . . . . . . 71 Refractive Errors. . . . . . . . . . . . . . . . . . . . . . . . 96
6.7.2 Trochlear Palsy . . . . . . . . . . . . . . . . . . . . . . . . . 71 8.2.1.1 Screening for Amblyopia . . . . . . . . . . . . . . . 97
6.7.3 Abducens Palsy . . . . . . . . . . . . . . . . . . . . . . . . 71 8.2.1.2 Screening for Strabismus . . . . . . . . . . . . . . . 97
6.7.4 Inferior Oblique (IO) Palsy . . . . . . . . . . . . . . 71 8.2.1.3 Screening for Refractive Error. . . . . . . . . . . 97
6.8 Central Abnormalities 8.2.1.4 Screening for Other Ocular Conditions. . 97
8.2.2 Diff erence Between a Screening
of Vergence and Gaze . . . . . . . . . . . . . . . . . . 72
6.8.1 Developmental Esotropia and Diagnostic Test . . . . . . . . . . . . . . . . . . . . 97
8.2.3 Justifi cation for Screening for
and Exotropia. . . . . . . . . . . . . . . . . . . . . . . . . . 72
6.8.2 Cerebellar Disease. . . . . . . . . . . . . . . . . . . . . . 72 Amblyopia and/or Strabismus . . . . . . . . . . 98
8.2.4 Recent Reports Examining
6.8.3 Horizontal Gaze Palsy and
Progressive Scoliosis . . . . . . . . . . . . . . . . . . . 72 Pre-School Vision Screening . . . . . . . . . . . . 98
8.3 Screening Tests for Amblyopia,
References. . . . . . . . . . . . . . . . . . . . . . . . . . . . . 72
Strabismus, and/or
Refractive Error. . . . . . . . . . . . . . . . . . . . . . . . . 100
8.3.1 Vision Tests . . . . . . . . . . . . . . . . . . . . . . . . . . . . 100
Chapter 7
Congenital Cranial Dysinnervation Disorders: 8.3.2 Cover-Uncover Test. . . . . . . . . . . . . . . . . . . . . 100
Facts and Perspectives to Understand Ocular 8.3.3 Stereoacuity . . . . . . . . . . . . . . . . . . . . . . . . . . . 101
Motility Disorders 8.3.4 Photoscreening and/or
Antje Neugebauer and Julia Fricke Autorefraction . . . . . . . . . . . . . . . . . . . . . . . . . 101
8.3.5 What to Do with Those Who
7.1 Congenital Cranial Dysinnervation Are Unable to Perform
Disorders: Facts About Ocular Screening Tests?. . . . . . . . . . . . . . . . . . . . . . . . 102
Motility Disorders . . . . . . . . . . . . . . . . . . . . . . 77 8.3.6 Who Should Administer
7.1.1 The Concept of CCDDs: the Screening Program?. . . . . . . . . . . . . . . . 102
Ocular Motility Disorders as 8.4 Treatment of Amblyopia. . . . . . . . . . . . . . . . 103
Neurodevelopmental Defects . . . . . . . . . . 77 8.4.1 Type of Treatment. . . . . . . . . . . . . . . . . . . . . . 103
7.1.1.1 Brainstem and Cranial 8.4.2 Refractive Adaptation . . . . . . . . . . . . . . . . . . 103
Nerve Development. . . . . . . . . . . . . . . . . . . . 78 8.4.3 Conventional Occlusion . . . . . . . . . . . . . . . . 104
7.1.1.2 Single Disorders 8.4.4 Pharmacological Occlusion. . . . . . . . . . . . . 104
Representing CCDDs . . . . . . . . . . . . . . . . . . . 78 8.4.5 Optical Penalization. . . . . . . . . . . . . . . . . . . . 104
7.1.1.3 Disorders Understood as CCDDs. . . . . . . . 81 8.4.6 Eff ective Treatment of
7.2 Congenital Cranial Dysinnervation Amblyopia in Older Children
Disorders: Perspectives to Understand (Over the Age of 7 Years). . . . . . . . . . . . . . . . 104
Ocular Motility Disorders . . . . . . . . . . . . . . . 83 8.4.7 Treatment Compliance . . . . . . . . . . . . . . . . . 105
7.2.1 Congenital Ocular 8.4.8 Other Treatment Options
Elevation Defi ciencies: A for Amblyopia. . . . . . . . . . . . . . . . . . . . . . . . . . 105
Neurodevelopmental View . . . . . . . . . . . . . 83 8.4.9 Recurrence of Amblyopia
7.2.1.1 Brown Syndrome. . . . . . . . . . . . . . . . . . . . . . . 83 Following Therapy . . . . . . . . . . . . . . . . . . . . . 105
7.2.1.2 Congenital Monocular 8.5 Quality of Life. . . . . . . . . . . . . . . . . . . . . . . . . . 106
Elevation Defi ciency and 8.5.1 The Impact of Amblyopia
Vertical Retraction Syndrome. . . . . . . . . . . 87 Upon HRQoL. . . . . . . . . . . . . . . . . . . . . . . . . . . 106
7.2.2 A Model of some Congenital 8.5.2 Stereoacuity and Motor Skills
Elevation Defi ciencies as in Children with Amblyopia. . . . . . . . . . . . . 106
Neurodevelopmental Diseases . . . . . . . . . 89 8.5.3 Reading Speed and Reading
References. . . . . . . . . . . . . . . . . . . . . . . . . . . . . 91 Ability in Children with Amblyopia. . . . . . 106