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CME expiration date: June 1, 2021. AMA PRA Category 1 Credits™ may be claimed only once between June 1, 2018, and the expiration date.
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Basic and Clinical Science Course
Louis B. Cantor, MD, Indianapolis, Indiana, Senior Secretary for Clinical Education
Christopher J. Rapuano, MD, Philadelphia, Pennsylvania, Secretary for Lifelong Learning and Assessment
George A. Cio�, MD, New York, New York, BCSC Course Chair
Section 6
Faculty
Robert W. Hered, MD, Chair, Maitland, Florida
Steven M. Archer, MD, Ann Arbor, Michigan
Rebecca Sands Braverman, MD, Aurora, Colorado
Arif O. Khan, MD, Abu Dhabi, United Arab Emirates
Katherine A. Lee, MD, PhD, Boise, Idaho
Gregg T. Lueder, MD, St Louis, Missouri
Mary A. O’Hara, MD, Sacramento, California
Kristina Tarczy-Hornoch, MD, DPhil, Seattle, Washington
The Academy wishes to acknowledge the American Association for Pediatric Ophthalmology and Strabismus (AAPOS) and the American Academy of Pediatrics (AAP), Section on Ophthalmology, for
recommending faculty members to the BCSC Section 6 committee.
The Academy also wishes to acknowledge the following committees for review of this edition:
Committee on Aging: Aaron M. Miller, MD, MBA, Shenandoah, Texas
Vision Rehabilitation Committee: Terry L. Schwartz, MD, Cincinnati, Ohio
Practicing Ophthalmologists Advisory Committee for Education: Alice Bashinsky, MD, Primary Reviewer, Asheville, North Carolina; Edward K. Isbey III, MD, Chair, Asheville, North Carolina; David J. Browning,
MD, PhD, Charlotte, North Carolina; Bradley D. Fouraker, MD, Tampa, Florida; Steven J. Grosser, MD, Golden Valley, Minnesota; Stephen R. Klapper, MD, Carmel, Indiana; James A. Savage, MD,
Memphis, Tennessee; Michelle S. Ying, MD, Ladson, South Carolina
European Board of Ophthalmology: Wagih Aclimandos, MB BCh, DO, FEBO, EBO Chair, London, United Kingdom; Peter J. Ringens, MD, PhD, EBO Liaison, Maastricht, Netherlands; Georges Caputo, MD,
Paris, France; Rosario Gómez de Liaño, MD, Madrid, Spain; Peng T. Khaw, MD, PhD, London, United Kingdom; Birgit Lorenz, MD, PhD, Giessen, Germany; Francis L. Munier, MD, Lausanne, Switzerland;
Seyhan B. Özkan, MD, Aydin, Turkey; Maria Papadopoulos, MBBS, London, United Kingdom; Nicoline E. Schalij-Delfos, MD, PhD, Leiden, Netherlands
The Academy also wishes to acknowledge the following committee for assistance in developing study questions and answers for this BCSC Section:
Self-Assessment Committee: Mitchell B. Strominger, MD, Chair, Boston, Massachusetts; Deborah M. Costakos, MD, Milwaukee, Wisconsin; Theodore Curtis, MD, Mount Kisco, New York; Mark I. Salevitz, MD,
Scottsdale, Arizona
Financial Disclosures
Academy sta� members who contributed to the development of this product state that within the 12 months prior to their contributions to this CME activity and for the duration of development, they have
had no �nancial interest in or other relationship with any entity discussed in this course that produces, markets, resells, or distributes ophthalmic health care goods or services consumed by or used in
patients, or with any competing commercial product or service.
The authors and reviewers state that within the 12 months prior to their contributions to this CME activity and for the duration of development, they have had the following �nancial relationships:*
Dr Sands Braverman: Welch Allyn (C)
Dr Browning: Aerpio Therapeutics (S), Alcon Laboratories (S), Alimera Sciences (C), Genentech (S), Novartis Pharmaceuticals (S), Ohr Pharmaceuticals (S), P�zer (S), Regeneron Pharmaceuticals (S), Zeiss
(O)
Dr Fouraker: Addition Technology (C, L), Alcon Laboratories (C, L), KeraVision (C, L), OASIS Medical (C, L)
Dr Gómez de Liaño: Alcon Laboratories (C)
Dr Grosser: InjectSense (O), Ivantis (O)
Dr Hered: Moria Surgical (P)
Dr Isbey: Alcon Laboratories (S), Allscripts (C), Bausch + Lomb (S), Med�ow (C), Oculos Clinical Research (S)
Dr Khaw: Isarna Therapeutics (C), Novartis (C), Santen (C)
Dr Lorenz: Bausch + Lomb (C), Bayer HealthCare Pharmaceuticals (L), Clarity (L, S), Novartis Pharmaceuticals (L, S), Spark Therapeutics (C)
Dr Miller: Alcon Laboratories (L), Credential Protection (O)
Dr Schalij-Delfos: Novartis (C)
Dr Tarczy-Hornoch: Amgen (O), Mylan (O)
The other authors and reviewers state that within the 12 months prior to their contributions to this CME activity and for the duration of development, they have had no �nancial interest in or other
relationship with any entity discussed in this course that produces, markets, resells, or distributes ophthalmic health care goods or services consumed by or used in patients, or with any competing
commercial product or service.
*C = consultant fees, paid advisory boards, or fees for attending a meeting; L = lecture fees (honoraria), travel fees, or reimbursements when speaking at the invitation of a commercial sponsor; O =
equity ownership/stock options of publicly or privately traded �rms (excluding mutual funds) with manufacturers of commercial ophthalmic products or commercial ophthalmic services; P = patents
and/or royalties that might be viewed as creating a potential con�ict of interest; S = grant support for the past year (all sources) and all sources used for a speci�c talk or manuscript with no time
limitation
Recent Past Faculty
Daniel J. Karr, MD
Sylvia R. Kodsi, MD
Stephen P. Kraft, MD
Kanwal (Ken) Nischal, MD
Evelyn A. Paysse, MD
In addition, the Academy gratefully acknowledges the contributions of numerous past faculty and advisory committee members who have played an important role in the development of previous editions
of the Basic and Clinical Science Course.
American Academy of Ophthalmology Staff
Dale E. Fajardo, EdD, MBA
Vice President, Education
Beth Wilson Daniel Mummert
Director, Cont inuing Professional Development Director, Online Edu cation
Ann McGuire Jasmine Chen
Acquisitions an d Development Manager Manager, E-Lear ning
Stephanie Tanaka Eric Gerdes
Publications Manage r Interactive De signer
D. Jean Ray Donna Scism
Production M anager E-Editor/Proof reader
Beth Collins Naomi Ruiz
Medical Edito r Publications S pecialist
Susan Malloy
Acquisitions Ed itor and Program Manager
American Academy of Ophthalmology
655 Beach Street
Box 7424
San Francisco, CA 94120-7424
Contents
Title Page
Copyright Page
Faculty and Disclosures
General Introduction
Visual Acuity Chart
Objectives
1 The Pediatric Eye Examination
Examination of Children in the Outpatient Setting
History and Examination: General Considerations and Strategies
Examination: Specific Elements
Visual Acuity Assessment
Red Reflex Examination and the Brückner Test
Dynamic Retinoscopy
Visual Field Testing
Pupil Testing
Anterior Segment Examination
Intraocular Pressure Measurement
Cycloplegic Refraction
Fundus Examination
PART I Strabismus
2 Strabismus Terminology
Definitions
Prefixes and Suffixes
Strabismus Classification Terms
Abbreviations for Types of Strabismus
3 Anatomy of the Extraocular Muscles
Origin, Course, Insertion, and Innervation of the Extraocular Muscles
Horizontal Rectus Muscles
Vertical Rectus Muscles
Oblique Muscles
Levator Palpebrae Superioris Muscle
Relationship of the Rectus Muscle Insertions
Blood Supply of the Extraocular Muscles
Arterial System
Venous System
Structure of the Extraocular Muscles
Orbital and Fascial Relationships
Adipose Tissue
Muscle Cone
Muscle Capsule
The Tenon Capsule
The Pulley System
Anatomical Considerations for Ophthalmic Procedures
4 Motor Physiology
Basic Principles and Terms
Ocular Rotations
Positions of Gaze
Extraocular Muscle Action
Eye Movements
Motor Units
Monocular Eye Movements
Binocular Eye Movements
Supranuclear Control Systems for Eye Movement
5 Sensory Physiology and Pathology
The Physiology of Normal Binocular Vision
Retinal Correspondence
Fusion
Selected Aspects of the Neurophysiology of Vision
Visual Development
Effects of Abnormal Visual Experience on the Retinogeniculocortical Pathway
Abnormalities of Binocular Vision
Visual Confusion
Diplopia
Sensory Adaptations in Strabismus
Suppression
Anomalous Retinal Correspondence
Monofixation Syndrome
6 Amblyopia
Epidemiology
Pathophysiology
Classification
Strabismic Amblyopia
Refractive Amblyopia
Visual Deprivation Amblyopia
Detection and Screening
Evaluation
Treatment
Cataract Removal
Refractive Correction
Occlusion Therapy
Pharmacologic or Optical Treatment
Binocular Treatment
Complications and Challenges of Therapy
7 Diagnostic Evaluation of Strabismus and Torticollis
Obtaining a History in Cases of Strabismus or Torticollis
Assessment of Ocular Alignment
Diagnostic Positions of Gaze
Cover Tests
Corneal Light Reflex and Red Reflex Tests
Subjective Tests of Ocular Alignment
Assessment of Eye Movements
Ocular Rotations
Convergence
Fusional Vergence
Forced Duction, Active Force Generation, and Saccadic Velocity
The 3-Step Test
Tests of Sensory Adaptation and Binocular Function
The Red-Glass (Diplopia) Test
Bagolini Lenses
The 4Δ Base-Out Prism Test
The Afterimage Test
Amblyoscope Testing
The Worth 4-Dot Test
Stereoacuity Tests
Assessment of the Field of Single Binocular Vision
The Prism Adaptation Test
Torticollis: Differential Diagnosis and Evaluation
Ocular Torticollis
8 Esodeviations
Epidemiology
Pseudoesotropia
Infantile (Congenital) Esotropia
Pathogenesis
Clinical Features and Evaluation
Management
Accommodative Esotropia
Pathogenesis and Types of Accommodative Esotropia
Evaluation
Management
Acquired Nonaccommodative Esotropia
Basic Acquired Nonaccommodative Esotropia
Cyclic Esotropia
Sensory Esotropia
Divergence Insufficiency
Spasm of the Near Reflex
Consecutive Esotropia
Nystagmus and Esotropia
Incomitant Esotropia
Sixth Nerve Palsy
Other Forms of Incomitant Esotropia
9 Exodeviations
Pseudoexotropia
Exophoria
Intermittent Exotropia
Clinical Characteristics
Evaluation
Treatment
Convergence Insufficiency
Constant Exotropia
Infantile Exotropia
Sensory Exotropia
Consecutive Exotropia
Other Forms of Exotropia
Exotropic Duane Retraction Syndrome
Neuromuscular Abnormalities
Dissociated Horizontal Deviation
Convergence Paralysis
10 Pattern Strabismus
Etiology
Clinical Features and Identification of Pattern Strabismus
V Pattern
A Pattern
Y Pattern
X Pattern
λ Pattern
Management
Surgical Correction of Pattern Deviations: General Principles
Surgical Treatment of Specific Patterns
11 Vertical Deviations
A Clinical Approach to Vertical Deviations
Vertical Deviations With Marked Horizontal Incomitance
Overelevation and Overdepression in Adduction
Superior Oblique Muscle Palsy
Inferior Oblique Muscle Palsy
Skew Deviation
Other Conditions With Incomitant Vertical Deviations
Vertical Deviations With Horizontal Comitance
Monocular Elevation Deficiency
Orbital Floor Fractures
Other Conditions With Comitant Vertical Deviations
Dissociated Vertical Deviation
Clinical Features
Management
12 Special Motility Disorders
Congenital Cranial Dysinnervation Disorders
Duane Retraction Syndrome
Congenital Fibrosis of the Extraocular Muscles
Möbius Syndrome
Miscellaneous Special Forms of Strabismus
Brown Syndrome
Third Nerve Palsy
Fourth Nerve Palsy
Sixth Nerve Palsy
Thyroid Eye Disease
Chronic Progressive External Ophthalmoplegia
Myasthenia Gravis
Esotropia and Hypotropia Associated With High Myopia
Internuclear Ophthalmoplegia
Ocular Motor Apraxia
Superior Oblique Myokymia
Strabismus Associated With Other Ocular Surgery
13 Childhood Nystagmus
General Features
Nomenclature
Types of Childhood Nystagmus
Infantile Nystagmus Syndrome (Congenital Nystagmus)
Central Vestibular Instability Nystagmus (Periodic Alternating Nystagmus)
Fusion Maldevelopment Nystagmus Syndrome (Latent Nystagmus)
Acquired Nystagmus
Nystagmus-Like Disorders
Induced Convergence-Retraction (Convergence-Retraction Nystagmus)
Opsoclonus
Evaluation
History
Ocular Examination
Treatment
Prisms
Surgery
14 Surgery of the Extraocular Muscles
Evaluation
Indications for Surgery
Planning Considerations
Vision
General Considerations
Incomitance
Cyclovertical Strabismus
Prior Surgery
Surgical Techniques for the Extraocular Muscles and Tendons
Approaches to the Extraocular Muscles
Rectus Muscle Weakening Procedures
Rectus Muscle Tightening Procedures
Rectus Muscle Surgery for Hypotropia and Hypertropia
Adjustable Sutures
Oblique Muscle Weakening Procedures
Oblique Muscle Tightening Procedures
Stay Sutures
Transposition Procedures
Posterior Fixation
Complications of Strabismus Surgery
Diplopia
Unsatisfactory Alignment
Iatrogenic Brown Syndrome
Anti-Elevation Syndrome
Lost and Slipped Muscles
Pulled-in-Two Syndrome
Perforation of the Sclera
Postoperative Infections
Pyogenic Granuloma and Foreign-Body Granuloma
Epithelial Cyst
Conjunctival Scarring
Adherence Syndrome
Delle
Anterior Segment Ischemia
Change in Eyelid Position
Refractive Changes
Anesthesia for Extraocular Muscle Surgery
Methods
Postoperative Nausea and Vomiting
Oculocardiac Reflex
Malignant Hyperthermia
Chemodenervation Using Botulinum Toxin
Pharmacology and Mechanism of Action
Indications, Techniques, and Results
Complications
PART II Pediatric Ophthalmology
15 Growth and Development of the Eye
Normal Growth and Development
Dimensions of the Eye
Refractive State
Orbit and Ocular Adnexa
Cornea, Iris, Pupil, and Anterior Chamber
Intraocular Pressure
Extraocular Muscles
Retina
Visual Acuity and Stereoacuity
Abnormal Growth and Development
16 Decreased Vision in Infants and Pediatric Vision Rehabilitation
Visual Development in Young Infants
Classification of Visual Impairment in Infants and Children
Pregeniculate Visual Impairment
Cerebral Visual Impairment
Delayed Visual Maturation
Evaluation of the Infant With Decreased Vision
Pediatric Low Vision Rehabilitation
17 Eyelid Disorders
Congenital Eyelid Disorders
Dystopia Canthorum
Cryptophthalmos
Ablepharon
Congenital Coloboma of the Eyelid
Ankyloblepharon
Congenital Ectropion
Congenital Entropion
Epiblepharon
Congenital Tarsal Kink
Distichiasis
Euryblepharon
Epicanthus
Palpebral Fissure Slants
Congenital Ptosis
Other Causes of Ptosis in Children
Infectious and Inflammatory Eyelid Disorders
Vascular Eyelid Disorders
Port-Wine Stain
Eyelid Hemangioma
Neoplasms and Other Noninflammatory Eyelid Lesions
Pilomatricoma
Epithelial Lesions
Eyelid Nevi
Other Eyelid Conditions
Trichotillomania
Excessive Blinking
18 Orbital Disorders
Abnormal Interocular Distance: Terminology and Associations
Congenital and Developmental Disorders: Craniofacial Malformations
Craniosynostosis
Nonsynostotic Craniofacial Conditions
Infectious and Inflammatory Conditions
Preseptal Cellulitis
Orbital Cellulitis
Childhood Orbital Inflammation
Neoplasms
Differential Diagnosis
Primary Malignant Neoplasms
Metastatic Tumors
Hematopoietic, Lymphoproliferative, and Histiocytic Neoplasms
Benign Tumors
Ectopic Tissue Masses
Cystic Lesions
Teratoma
Ectopic Lacrimal Gland
19 Lacrimal Drainage System Abnormalities
Congenital and Developmental Anomalies
Atresia of the Lacrimal Puncta or Canaliculi
Congenital Lacrimal Fistula
Dacryocystocele
Congenital Nasolacrimal Duct Obstruction
Clinical Features and Examination
Nonsurgical Management
Surgical Management
20 External Diseases of the Eye
Infectious Conjunctivitis
Ophthalmia Neonatorum
Bacterial Conjunctivitis in Children and Adolescents
Viral Conjunctivitis in Infants and Children
Inflammatory Disease
Blepharitis
Ocular Allergy
Ligneous Conjunctivitis
Other Conjunctival and Subconjunctival Disorders
Papillomas
Conjunctival Epithelial Inclusion Cysts
Conjunctival Nevi
Ocular Melanocytosis
Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis
21 Disorders of the Anterior Segment
Abnormalities of Corneal Size or Shape
Megalocornea
Microcornea
Keratoglobus
Keratoconus
Abnormalities of Peripheral Corneal Transparency
Posterior Embryotoxon
Cornea Plana
Epibulbar Dermoid
Dermolipoma
Abnormalities of Central and Diffuse Corneal Transparency
Primary Causes
Secondary Causes
Treatment of Corneal Opacities
Congenital and Developmental Anomalies of the Globe
Microphthalmia, Anophthalmia, and Coloboma
Nanophthalmos
Congenital and Developmental Anomalies of the Iris or Pupil
Abnormalities of the Iris
Abnormalities in the Size, Shape, or Location of the Pupil
Acquired Corneal Conditions
Keratitis
Systemic Diseases Affecting the Cornea or Iris
Metabolic Disorders Affecting the Cornea or Iris
Other Systemic Diseases Affecting the Cornea or Iris
Tumors of the Anterior Segment
Cornea
Iris
Ciliary Body
Miscellaneous Clinical Signs
Pediatric Iris Heterochromia
Anisocoria
22 Pediatric Glaucomas
Classification
Genetics
Primary Childhood Glaucoma
Primary Congenital Glaucoma
Juvenile Open-Angle Glaucoma
Secondary Childhood Glaucoma
Secondary Glaucoma Associated With an Acquired Condition
Glaucoma Following Cataract Surgery
Treatment
Surgical Therapy
Medical Therapy
Prognosis and Follow-Up
23 Childhood Cataracts and Other Pediatric Lens Disorders
Pediatric Cataracts
General Features
Morphology
Evaluation of Pediatric Cataracts
Examination
Cataract Surgery in Pediatric Patients
Timing of the Procedure
Intraocular Lens Use in Children
Management of the Anterior Capsule
Lensectomy Without Intraocular Lens Implantation
Lensectomy With Intraocular Lens Implantation
Postoperative Care
Complications Following Pediatric Cataract Surgery
Visual Outcome After Cataract Extraction
Structural or Positional Lens Abnormalities
Congenital Aphakia
Spherophakia
Coloboma
Dislocated Lenses in Children
Isolated Ectopia Lentis
Ectopia Lentis et Pupillae
Marfan Syndrome
Homocystinuria
Weill-Marchesani Syndrome
Sulfite Oxidase Deficiency
Treatment
24 Uveitis in the Pediatric Age Group
Epidemiology
Classification
Anterior Uveitis
Juvenile Idiopathic Arthritis
Juvenile Spondyloarthropathies
Tubulointerstitial Nephritis and Uveitis Syndrome
Kawasaki Disease
Other Causes of Anterior Uveitis
Intermediate Uveitis
Posterior Uveitis
Toxoplasmosis
Toxocariasis
Other Causes of Posterior Uveitis
Panuveitis
Sarcoidosis
Familial Juvenile Systemic Granulomatosis
Vogt-Koyanagi-Harada Syndrome
Other Causes of Panuveitis
Masquerade Syndromes
Evaluation of Pediatric Uveitis
Treatment of Pediatric Uveitis
Management of Inflammation
Surgical Treatment of Complications of Uveitis
25 Disorders of the Retina and Vitreous
Congenital and Developmental Abnormalities
Persistent Fetal Vasculature
Retinopathy of Prematurity
Hereditary Retinal Disease
Hereditary Macular Dystrophies
Hereditary Vitreoretinopathies
Infectious Diseases
Herpes Simplex Virus and Cytomegalovirus
Human Immunodeficiency Virus
Tumors
Choroidal and Retinal Pigment Epithelial Lesions
Retinoblastoma
Acquired Disorders
Coats Disease
26 Optic Disc Abnormalities
Developmental Anomalies
Optic Nerve Hypoplasia
Morning Glory Disc Anomaly
Optic Nerve Coloboma
Optic Nerve Pit
Myelinated Retinal Nerve Fiber Layer
Tilted Disc
Bergmeister Papilla
Megalopapilla
Peripapillary Staphyloma
Optic Nerve Aplasia
Melanocytoma of the Optic Disc
Optic Atrophy
Dominant Optic Atrophy, Kjer Type
Recessive Optic Atrophy
Leber Hereditary Optic Neuropathy
Optic Neuritis
Papilledema
Idiopathic Intracranial Hypertension
Pseudopapilledema
Drusen
27 Ocular Trauma in Childhood
Special Considerations in the Management of Pediatric Ocular Trauma
Accidental Trauma
Corneal Abrasion
Thermal Injury
Chemical Injury
Corneal Foreign Body
Penetrating or Perforating Injury
Blunt Injury
Orbital Fractures
Traumatic Optic Neuropathy
Nonaccidental Trauma
Abusive Head Trauma
Ocular Injury Secondary to Nonaccidental Trauma
28 Ocular Manifestations of Systemic Disease
Diseases due to Chromosomal Abnormalities
Inborn Errors of Metabolism
Familial Oculorenal Syndromes
Lowe Syndrome
Alport Syndrome
Ciliopathies
Neuro-Oculocutaneous Syndromes
Neurofibromatosis Type 1
Neurofibromatosis Type 2
Tuberous Sclerosis
Von Hippel–Lindau Disease
Sturge-Weber Syndrome
Ataxia-Telangiectasia
Incontinentia Pigmenti
Wyburn-Mason Syndrome
Klippel-Trénaunay Syndrome
Albinism
Diagnosis
Treatment
Diabetes Mellitus
Intrauterine or Perinatal Infection
Toxoplasmosis
Rubella
Cytomegalovirus
Herpes Simplex Virus
Syphilis
Lymphocytic Choriomeningitis
Zika Virus
Malignant Disease
Leukemia
Neuroblastoma
Basic Texts
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