2018-2019 BCSC (Basic and Clinical Science Course), Section 06: Pediatric Ophthalmology and Strabismus PDF
Preview 2018-2019 BCSC (Basic and Clinical Science Course), Section 06: Pediatric Ophthalmology and Strabismus
The American Academy of Ophthalmology is accredited by the Accreditation Council for Continuing Medical Education (ACCME) to provide continuing medical education for physicians. The American Academy of Ophthalmology designates this enduring material for a maximum of 15 AMA PRA Category 1 Credits™. Physicians should claim only the credit commensurate with the extent of their participation in the activity. CME expiration date: June 1, 2021. AMA PRA Category 1 Credits™ may be claimed only once between June 1, 2018, and the expiration date. BCSC® volumes are designed to increase the physician’s ophthalmic knowledge through study and review. Users of this activity are encouraged to read the text and then answer the study questions provided at the back of the book. To claim AMA PRA Category 1 Credits™ upon completion of this activity, learners must demonstrate appropriate knowledge and participation in the activity by taking the posttest for Section 6 and achieving a score of 80% or higher. For further details, please see the instructions for requesting CME credit at the back of the book. The Academy provides this material for educational purposes only. It is not intended to represent the only or best method or procedure in every case, nor to replace a physician’s own judgment or give speci�c advice for case management. Including all indications, contraindications, side e�ects, and alternative agents for each drug or treatment is beyond the scope of this material. All information and recommendations should be veri�ed, prior to use, with current information included in the manufacturers’ package inserts or other independent sources, and considered in light of the patient’s condition and history. Reference to certain drugs, instruments, and other products in this course is made for illustrative purposes only and is not intended to constitute an endorsement of such. Some material may include information on applications that are not considered community standard, that re�ect indications not included in approved FDA labeling, or that are approved for use only in restricted research settings. The FDA has stated that it is the responsibility of the physician to determine the FDA status of each drug or device he or she wishes to use, and to use them with appropriate, informed patient consent in compliance with applicable law. The Academy speci�cally disclaims any and all liability for injury or other damages of any kind, from negligence or otherwise, for any and all claims that may arise from the use of any recommendations or other information contained herein. AAO, AAOE, American Academy of Ophthalmology, Basic and Clinical Science Course, BCSC, EyeCare America, EyeNet, EyeSmart, EyeWiki, Femtocenter, Focal Points, IRIS, ISRS, OKAP, ONE, Ophthalmic Technology Assessments, Ophthalmology, Ophthalmology Retina, Preferred Practice Pattern, ProVision, The Ophthalmic News & Education Network, and the AAO logo (shown on cover) and tagline (Protecting Sight. Empowering Lives.) are, among other marks, the registered trademarks and trademarks of the American Academy of Ophthalmology. Cover image: From BCSC Section 12, Retina and Vitreous. End-stage chorioretinal atrophy in pathologic myopia. (Courtesy of Richard F. Spaide, MD.) Copyright © 2018 American Academy of Ophthalmology. All rights reserved. No part of this publication may be reproduced without written permission. 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 Related Academy Materials Requesting Continuing Medical Education Credit Create a Self Test
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