Ophthalmic Knowledge Assessment Program

OKAP®

Examination

CONTENT OUTLINE

American Academy of Ophthalmology P.O. Box 7424 San Francisco, CA 94120-7424

© 2017 American Academy of Ophthalmology All rights reserved TABLE OF CONTENTS

OKAP Content Outline

Section Page

1. GENERAL MEDICINE 1

2. FUNDAMENTALS AND PRINCIPLES OF OPHTHALMOLOGY 4

3. CLINICAL OPTICS 7

4. OPHTHALMIC PATHOLOGY AND INTRAOCULAR TUMORS 13

5. NEURO-OPHTHALMOLOGY 19

6. PEDIATRIC OPHTHALMOLOGY AND STRABISMUS 24

7. ORBIT, EYELIDS, AND LACRIMAL SYSTEM 34

8. EXTERNAL DISEASE AND CORNEA 40

9. INTRAOCULAR INFLAMMATION AND UVEITIS 47

10. GLAUCOMA 51

11. LENS AND CATARACT 53

12. RETINA AND VITREOUS 58

13. REFRACTIVE SURGERY 64

CONTENT OUTLINE

Content Outline Number

SECTION 1: GENERAL MEDICINE ...... 1.0

Scored as part of Subtest 1: General Medicine

Infectious Disease ...... 1.1

Staphylococcus ...... 1.1.1 Streptococcus ...... 1.1.2 Neisseria...... 1.1.3 Pseudomonas aeruginosa ...... 1.1.4 Treponema pallidum ...... 1.1.5 Borrelia burgdorferi ...... 1.1.6 Chlamydia trachomatis ...... 1.1.7 Mycoplasma pneumonia ...... 1.1.8 Mycobacteria ...... 1.1.9 Tuberculosis ...... 1.1.9.1 Fungal Infections...... 1.1.10 Toxoplasma ...... 1.1.11 Herpesvirus...... 1.1.12 Herpes Simplex ...... 1.1.12.1 Varicella-Zoster ...... 1.1.12.2 Cytomegalovirus ...... 1.1.12.3 Epstein-Barr Virus ...... 1.1.12.4 Influenza ...... 1.1.13 Hepatitis...... 1.1.14 Hepatitis A ...... 1.1.14.1 Hepatitis B ...... 1.1.14.2 Hepatitis C and Other Forms of Hepatitis ...... 1.1.14.3 Human Papillomavirus ...... 1.1.15 Acquired Immunodeficiency Syndrome ...... 1.1.16 Etiology and Pathogenesis ...... 1.1.16.1 Clinical Syndromes ...... 1.1.16.2 Seroepidemiology ...... 1.1.16.3 Modes of Transmission ...... 1.1.16.4 Prognosis and Treatment...... 1.1.16.5 Opportunistic Infections ...... 1.1.16.6 Ophthalmic considerations ...... 1.1.16.7 Update on Antibiotics ...... 1.1.17 Antibacterial Agents ...... 1.1.17.1 New Antibiotic Classes ...... 1.1.17.2 Antifungal Agents ...... 1.1.17.3 Antiviral Agents ...... 1.1.17.4

Hypertension ...... 1.2

Classification of Blood Pressure and Diagnosis of Hypertension ...... 1.2.1 Etiology and Pathogenesis of Hypertension ...... 1.2.2 Evaluation of Hypertension...... 1.2.3 Treatment of Hypertension ...... 1.2.4 Lifestyle Modifications...... 1.2.4.1

1 Pharmacologic Treatment ...... 1.2.4.2 Antihypertensive Drugs ...... 1.2.5 Special Considerations...... 1.2.6

Cerebrovascular Disease ...... 1.3

Cerebral Ischemia ...... 1.3.1 Carotid Occlusive Disease ...... 1.3.2 Intracranial Hemorrhage ...... 1.3.3

Acquired Heart Disease...... 1.4

Ischemic Heart Disease ...... 1.4.1 Congestive Heart Failure ...... 1.4.2 Disorders of Cardiac Rhythm ...... 1.4.3

Hypercholesterolemia ...... 1.5

Risk Assessment ...... 1.5.1 Management ...... 1.5.2

Pulmonary Diseases...... 1.6

Hematologic Disorders...... 1.7

Anemia ...... 1.7.1 Disorders of Hemostasis ...... 1.7.2

Rheumatic Disorders ...... 1.8

Rheumatoid Arthritis...... 1.8.1 Spondyloarthropathies ...... 1.8.2 Systemic Lupus Erythematosus ...... 1.8.3 Antiphospholipid Antibody Syndrome ...... 1.8.4 Scleroderma ...... 1.8.5 Sjogren Syndrome ...... 1.8.6 Polymyositis and Dermatomyositis ...... 1.8.7 Relapsing Polychondritis ...... 1.8.8 Vasculitis ...... 1.8.9 Behcet Syndrome ...... 1.8.10 Medical Therapy for Rheumatic Disorders ...... 1.8.11

Endocrine Disorders ...... 1.9

Diabetes Mellitus ...... 1.9.1 Classification ...... 1.9.1.1 Clinical Presentation of Diabetes ...... 1.9.1.2 Diagnosis and Screening ...... 1.9.1.3 Prevention of Diabetes ...... 1.9.1.4 Management ...... 1.9.1.5 Glucose Surveillance ...... 1.9.1.6 Acute Complications of Diabetes ...... 1.9.1.7 Long-term Complications of Diabetes ...... 1.9.1.8 Ophthalmic considerations ...... 1.9.1.9 Thyroid Disease ...... 1.9.2 Hyperthyroidism ...... 1.9.2.1

2 The Hypothalamic-Pituitary Axis ...... 1.9.3 Multiple Endocrine Neoplasia Syndromes...... 1.9.4

Geriatrics ...... 1.10

Surgical Considerations ...... 1.10.1 Osteoporosis...... 1.10.2 Systemic Diseases ...... 1.10.3

Cancer ...... 1.11

Etiology ...... 1.11.1 Therapy ...... 1.11.2 Ophthalmic considerations ...... 1.11.3

Behavioral and Neurologic Disorders ...... 1.12

Behavioral Disorders ...... 1.12.1 Pharmacologic Treatment of Psychiatric Disorders ...... 1.12.2 Neurologic Disorders ...... 1.12.3 Parkinson Disease...... 1.12.3.1 Epilepsy ...... 1.12.3.2 Alzheimer Disease and Dementia ...... 1.12.3.3

Preventive Medicine ...... 1.13

Screening Procedures ...... 1.13.1 Immunization ...... 1.13.2

Medical Emergencies ...... 1.14

Cardiopulmonary Arrest ...... 1.14.1 Shock ...... 1.14.2 Seizures and Status Epilepticus ...... 1.14.3 Toxic Reactions to Local Anesthetics and Other Agents ...... 1.14.4 Ocular Side Effects of Systemic Medications ...... 1.14.5

Perioperative Management in Ocular Surgery ...... 1.15

Malignant Hyperthermia ...... 1.15.1

Using Statistics in Practice and Work ...... 1.16

Obtaining Useful Information From Published Studies ...... 1.16.1 Clinical Study Designs...... 1.16.2 Discussing Benefits, Risks, Probabilities, and Expected Outcomes With Patients ...... 1.16.3 Applying Statistics to Measure and Improve Clinical Practice ...... 1.16.4

3 CONTENT OUTLINE Content Outline Number

SECTION 2: FUNDAMENTALS AND PRINCIPLES OF OPHTHALMOLOGY ...... 2.0

Scored as part of Subtest 2: Fundamentals and Principles of Ophthalmology

Orbit and Ocular Adnexa ...... 2.1

Orbital Anatomy ...... 2.1.1 Cranial Nerves ...... 2.1.2 Ciliary Ganglion...... 2.1.3 Extraocular Muscles ...... 2.1.4 Eyelids ...... 2.1.5 Lacrimal Gland and Excretory System ...... 2.1.6 Conjunctiva ...... 2.1.7 Tenon Capsule...... 2.1.8 Vascular Supply and Drainage of the Orbit ...... 2.1.9

The Eye ...... 2.2

Cornea ...... 2.2.1 Sclera ...... 2.2.2 Limbus ...... 2.2.3 Anterior Chamber ...... 2.2.4 Trabecular Meshwork ...... 2.2.5 Uveal Tract ...... 2.2.6 Iris ...... 2.2.7 Ciliary Body...... 2.2.8 Choroid ...... 2.2.9 Lens...... 2.2.10 Retina ...... 2.2.11 Retinal Pigment Epithelium ...... 2.2.11.1 Neurosensory Retina ...... 2.2.11.2 Macula ...... 2.2.12 Ora Serrata ...... 2.2.13 Vitreous ...... 2.2.14

Cranial Nerves: Central and Peripheral Connections ...... 2.3

Cranial Nerve I (Olfactory Nerve)...... 2.3.1 Cranial Nerve II (Optic Nerve) ...... 2.3.2 Cranial Nerve III (Oculomotor Nerve)...... 2.3.3 Cranial Nerve IV (Trochlear Nerve) ...... 2.3.4 Cranial Nerve V (Trigeminal Nerve) ...... 2.3.5 Cranial Nerve VI (Abducens Nerve) ...... 2.3.6 Cranial Nerve VII (Facial Nerve) ...... 2.3.7 Cavernous Sinus...... 2.3.8

Ocular Development ...... 2.4

Eye Development ...... 2.4.1 Genetic Cascades and Morphogenic Gradients ...... 2.4.2 Homeobox Gene Program ...... 2.4.2.1

4 Molecular Genetics ...... 2.5

Gene Transcription and Translation: The Central Dogma of Genetics ...... 2.5.1 DNA Damage and Repair ...... 2.5.2 Mutations and Disease ...... 2.5.3 Mitochondrial Disease ...... 2.5.4 Gene Therapy ...... 2.5.5

Clinical Genetics...... 2.6

Patterns of Inheritance ...... 2.6.1 Autosomal Recessive Inheritance...... 2.6.2 Autosomal Dominant Inheritance ...... 2.6.3 X-Linked Inheritance ...... 2.6.4 Maternal Inheritance ...... 2.6.5 Genes and Chromosomes...... 2.6.6 Chromosomal Analysis...... 2.6.7 Mutations ...... 2.6.8 Genetic Counseling ...... 2.6.9

Tear Film ...... 2.7

Cornea...... 2.8

Epithelium ...... 2.8.1 Bowman Layer ...... 2.8.2 Stroma ...... 2.8.3 Descemet Membrane and Endothelium ...... 2.8.4

Aqueous Humor, Iris, and Ciliary Body ...... 2.9

Dynamics of the Aqueous Humor ...... 2.9.1 Composition of the Aqueous Humor ...... 2.9.2 Clinical Implications of Breakdown of the Blood–Aqueous Barrier ...... 2.9.3 Introduction to the Iris and Ciliary Body ...... 2.9.4 Eicosanoids ...... 2.9.5

Lens ...... 2.10

Structure of the Lens ...... 2.10.1 Chemical Composition of the Lens ...... 2.10.2 Physiologic Aspects of the Lens ...... 2.10.3 Lens Metabolism and Formation of Sugar Cataracts...... 2.10.4

Vitreous ...... 2.11

Retina ...... 2.12

Neural Retina—The Photoreceptors ...... 2.12.1 Inner Nuclear Layer...... 2.12.2

Retinal Pigment Epithelium...... 2.13

Anatomical Description...... 2.13.1 Major Physiologic Roles of the RPE ...... 2.13.2 The RPE in Disease ...... 2.13.3

5 Free Radicals and Antioxidants ...... 2.14

Oxidative Damage to the Lens ...... 2.14.1 Vulnerability of the Retina to Free Radicals ...... 2.14.2 Antioxidants in the Retina and RPE ...... 2.14.3

Pharmacologic Principles ...... 2.15

Ocular Pharmacotherapeutics ...... 2.16

Legal Aspects of Medical Therapy ...... 2.16.1 Cholinergic Drugs ...... 2.16.2 Adrenergic Drugs ...... 2.16.3 Carbonic Anhydrase Inhibitors...... 2.16.4 Prostaglandin Analogues ...... 2.16.5 Combined Medications ...... 2.16.6 Osmotic Drugs ...... 2.16.7 Anti-inflammatory Drugs ...... 2.16.8 Glucocorticoids ...... 2.16.8.1 Nonsteroidal Anti-inflammatory Drugs ...... 2.16.8.2 Antiallergic Drugs: Mast-Cell Stabilizers and Antihistamines ...... 2.16.8.3 Antifibrotic Drugs ...... 2.16.8.4 Medications for Dry Eye ...... 2.16.9 Ocular Decongestants ...... 2.16.10 Antimicrobial Drugs ...... 2.16.11 Penicillins and Cephalosporins ...... 2.16.11.1 Other Antibacterial Drugs...... 2.16.11.2 Antifungal Drugs ...... 2.16.11.3 Antiviral Drugs ...... 2.16.11.4 Medications for Acanthamoeba Infections ...... 2.16.11.5 Local Anesthetics ...... 2.16.12 Purified Neurotoxin Complex ...... 2.16.13 Hyperosmolar Drugs ...... 2.16.14 Irrigating Solutions ...... 2.16.15 Diagnostic Agents ...... 2.16.16 Viscoelastic Agents ...... 2.16.17 Fibrinolytic Agents ...... 2.16.18 Thrombin ...... 2.16.19 Antifibrinolytic Agents ...... 2.16.20 Vitamin Supplements and Antioxidants...... 2.16.21 Interferon ...... 2.16.22 Growth Factors ...... 2.16.23

6 CONTENT OUTLINE

Content Outline Number

SECTION 3: CLINICAL OPTICS ...... 3.0

Scored as part of Subtest 3: Clinical Optics

Geometric Optics ...... 3.1

Rays, Refraction, and Reflection ...... 3.1.1 Point Sources, Pencils, and Beams of Light ...... 3.1.1.1 Object Characteristics ...... 3.1.2 Image Characteristics ...... 3.1.3 Magnification ...... 3.1.3.1 Image Location ...... 3.1.3.2 Depth of Focus ...... 3.1.3.3 Image Quality ...... 3.1.3.4 Light Propagation ...... 3.1.4 Optical Media and Refractive Index ...... 3.1.4.1 Law of Rectilinear Propagation ...... 3.1.4.2 Optical Interfaces ...... 3.1.4.3 Law of Reflection (Specular Reflection) ...... 3.1.4.4 Law of Refraction (Specular Transmission) ...... 3.1.4.5 Normal Incidence ...... 3.1.4.6 Total Internal Reflection ...... 3.1.4.7 Dispersion ...... 3.1.4.8 Reflection and Refraction at Curved Surfaces ...... 3.1.4.9 The Fermat Principle ...... 3.1.4.10 Pinhole Imaging ...... 3.1.4.11 Locating the Image: The Lensmaker’s Equation ...... 3.1.4.12 Ophthalmic Lenses ...... 3.1.5 Vergence ...... 3.1.5.1 Reduced Vergence ...... 3.1.5.2 Thin-Lens Approximation ...... 3.1.5.3 Lens Combinations ...... 3.1.5.4 Virtual Images and Objects ...... 3.1.5.5 Focal Points and Planes ...... 3.1.5.6 Paraxial Ray Tracing Through Convex Spherical Lenses ...... 3.1.5.7 Paraxial Ray Tracing Through Concave Spherical Lenses ...... 3.1.5.8 Objects and Images at Infinity ...... 3.1.5.9 Principal Planes and Points ...... 3.1.5.10 Focal Lengths ...... 3.1.6 Gaussian Reduction ...... 3.1.7 Knapp’s Law, the Badal Principle, and the Lensmeter ...... 3.1.7.1 Afocal Systems ...... 3.1.8 Power of a Lens in a Medium ...... 3.1.9 Spherical Interface and Thick Lenses ...... 3.1.10 Thick Lens ...... 3.1.10.1 Back Vertex Power Is Not True Power ...... 3.1.10.2 Aberrations of Ophthalmic Lenses ...... 3.1.11 Third-Order Seidel Aberrations ...... 3.1.11.1 Chromatic Aberrations ...... 3.1.11.2 Avoiding Aberrations ...... 3.1.11.3 Mirrors ...... 3.1.12

7 Reflection From a Plane Mirror ...... 3.1.12.1 Spherically Curved Mirrors ...... 3.1.12.2 Reversal of the Image Space ...... 3.1.12.3 The Central Ray for Mirrors ...... 3.1.12.4 Vergence Calculations for Mirrors ...... 3.1.12.5 Spherocylindrical Lenses ...... 3.1.13 Combination of Spherocylindrical Lenses ...... 3.1.13.1 The Conoid of Sturm ...... 3.1.13.2 The Jackson Cross Cylinder ...... 3.1.13.3 Prisms ...... 3.1.14 Prism Diopter ...... 3.1.14.1 Prismatic Effect of Lenses and the Prentice Rule ...... 3.1.14.2 Prism Aberrations ...... 3.1.14.3 Fresnel Prisms ...... 3.1.14.4

Optics of the Human Eye ...... 3.2

The Human Eye as an Optical System ...... 3.2.1 Schematic Eyes ...... 3.2.2 Important Axes of the Eye ...... 3.2.3 Pupil Size and Its Effect on Visual Resolution ...... 3.2.4 Visual Acuity ...... 3.2.5 Contrast Sensitivity and the Contrast Sensitivity Function ...... 3.2.6 Refractive States of the Eyes ...... 3.2.7 Binocular States of the Eyes ...... 3.2.8 Accommodation and Presbyopia ...... 3.2.9 Epidemiology of Refractive Errors ...... 3.2.10 Developmental Myopia...... 3.2.11 Developmental Hyperopia ...... 3.2.12 Prevention of Refractive Errors ...... 3.2.13

Clinical Refraction ...... 3.3

Objective Refraction Technique: Retinoscopy ...... 3.3.1 Positioning and Alignment ...... 3.3.1.1 Fixation and Fogging...... 3.3.1.2 The Retinal Reflex...... 3.3.1.3 The Correcting Lens...... 3.3.1.4 Finding Neutrality ...... 3.3.1.5 Retinoscopy of Regular Astigmatism ...... 3.3.1.6 Aberrations of the Retinoscopic Reflex ...... 3.3.1.7 Subjective Refraction Techniques ...... 3.3.2 Astigmatic Dial Technique ...... 3.3.2.1 Stenopeic Slit Technique ...... 3.3.2.2 Cross-Cylinder Technique ...... 3.3.2.3 Refining the Sphere ...... 3.3.2.4 Binocular Balance ...... 3.3.2.5 Cycloplegic and Noncycloplegic Refraction ...... 3.3.3 Overrefraction ...... 3.3.4 Spectacle Correction of Ametropias ...... 3.3.5 Spherical Correcting Lenses and the Far Point Concept...... 3.3.5.1 The Importance of Vertex Distance ...... 3.3.5.2 Cylindrical Correcting Lenses and the Far Point Concept ...... 3.3.5.3 Prescribing for Children...... 3.3.6 Myopia ...... 3.3.6.1 Hyperopia ...... 3.3.6.2

8 Anisometropia ...... 3.3.6.3 Clinical Accommodative Problems ...... 3.3.7 Presbyopia ...... 3.3.7.1 Accommodative Insufficiency ...... 3.3.7.2 Accommodative Excess ...... 3.3.7.3 Accommodative Convergence/Accommodation Ratio ...... 3.3.7.4 Effect of Spectacle and Contact Lens Correction on Accommodation and Convergencev 3.3.7.5 Prescribing Multifocal Lenses ...... 3.3.8 Determining the Add Power of a Bifocal Lens ...... 3.3.8.1 Types of Bifocal Lenses ...... 3.3.8.2 Trifocal Lenses ...... 3.3.8.3 Progressive Addition Lenses ...... 3.3.8.4 The Prentice Rule and Bifocal Lens Design ...... 3.3.8.5 Occupation and Bifocal Segment ...... 3.3.8.6 Prescribing Special Lenses ...... 3.3.9 Aphakic Lenses ...... 3.3.9.1 Absorptive Lenses...... 3.3.9.2 Special Lens Materials ...... 3.3.9.3 Therapeutic Use of Prisms ...... 3.3.9.4

Contact Lenses ...... 3.4

Contact Lens Glossary ...... 3.4.1 Clinically Important Features of Contact Lens Optics ...... 3.4.2 Field of Vision ...... 3.4.2.1 Image Size ...... 3.4.2.2 Accommodation ...... 3.4.2.3 Convergence Demands ...... 3.4.2.4 Tear Lens ...... 3.4.2.5 Correcting Astigmatism ...... 3.4.2.6 Correcting Presbyopia ...... 3.4.2.7 Contact Lens Materials and Manufacturing ...... 3.4.3 Materials ...... 3.4.3.1 Manufacturing ...... 3.4.3.2 Patient Examination and Contact Lens Selection ...... 3.4.4 Patient Examination ...... 3.4.4.1 Contact Lens Selection ...... 3.4.4.2 Contact Lens Fitting ...... 3.4.5 Soft Contact Lenses ...... 3.4.5.1 Rigid Gas-Permeable Contact Lenses...... 3.4.5.2 Toric Soft Contact Lenses ...... 3.4.5.3 Contact Lenses for Presbyopia ...... 3.4.5.4 Keratoconus and the Abnormal Cornea ...... 3.4.5.5 Contact Lens Overrefraction ...... 3.4.5.6 Gas-Permeable Scleral Contact Lenses ...... 3.4.5.7 Therapeutic Lens Usage ...... 3.4.6 Orthokeratology and Corneal Reshaping ...... 3.4.7 Custom Contact Lenses and Wavefront Technology ...... 3.4.8 Contact Lens Care and Solutions ...... 3.4.9 Contact Lens–Related Problems and Complications ...... 3.4.10 Infections ...... 3.4.10.1 Hypoxic/Metabolic Problems ...... 3.4.10.2 Toxicity ...... 3.4.10.3 Mechanical Problems ...... 3.4.10.4 Inflammation ...... 3.4.10.5 Dry Eye ...... 3.4.10.6

9 Intraocular Lenses ...... 3.5

Intraocular Lens Designs ...... 3.5.1 Classification ...... 3.5.1.1 Background ...... 3.5.1.2 Optical Considerations for Intraocular Lenses ...... 3.5.2 Intraocular Lens Power Calculation ...... 3.5.2.1 Piggyback and Supplemental Intraocular Lenses ...... 3.5.2.2 Intraocular Lens Power Calculation After Corneal Refractive Surgery ...... 3.5.3 Instrument Error ...... 3.5.3.1 Index of Refraction Error ...... 3.5.3.2 Formula Error ...... 3.5.3.3 Power Calculation Methods for the Post–Keratorefractive Procedure Eye ...... 3.5.3.4 Intraocular Lens Power in Corneal Transplant Eyes ...... 3.5.4 Silicone Oil Eyes ...... 3.5.5 Pediatric Eyes ...... 3.5.6 Image Magnification ...... 3.5.7 Lens-Related Vision Disturbances ...... 3.5.8 Nonspherical Optics ...... 3.5.9 Multifocal Intraocular Lenses ...... 3.5.10 Types of Multifocal Intraocular Lenses ...... 3.5.10.1 Clinical Results of Multifocal Intraocular Lenses ...... 3.5.10.2 Accommodating Intraocular Lenses ...... 3.5.10.3 Intraocular Lens Standards ...... 3.5.11

Optical Considerations in Keratorefractive Surgery ...... 3.6

Corneal Shape ...... 3.6.1 Angle Kappa ...... 3.6.2 Pupil Size ...... 3.6.3 Irregular Astigmatism ...... 3.6.4 Application of Wavefront Analysis in Irregular Astigmatism...... 3.6.4.1 Causes of Irregular Astigmatism ...... 3.6.4.2

Optical Instruments and Low Vision Aids ...... 3.7

Magnification ...... 3.7.1 Telescopes ...... 3.7.2 Galilean Telescope ...... 3.7.2.1 Astronomical Telescope ...... 3.7.2.2 Accommodation Through a Telescope ...... 3.7.2.3 Surgical Loupe ...... 3.7.2.4 General Principles of Optical Engineering ...... 3.7.3 Terminology ...... 3.7.3.1 Measurements of Performance of Optical Systems ...... 3.7.3.2 Optical Instruments and Techniques Used in Ophthalmic Practice ...... 3.7.4 Direct Ophthalmoscope ...... 3.7.4.1 Indirect Ophthalmoscope ...... 3.7.4.2 Fundus Camera ...... 3.7.4.3 Slit-Lamp Biomicroscope ...... 3.7.4.4 Gonioscopy ...... 3.7.4.5 Surgical Microscope ...... 3.7.4.6 Geneva Lens Clock ...... 3.7.4.7 Lensmeter ...... 3.7.4.8

10 Knapp’s Rule ...... 3.7.4.9 Optical Pachymeter ...... 3.7.4.10 Applanation Tonometry ...... 3.7.4.11 Specular Microscopy ...... 3.7.4.12 Keratometer ...... 3.7.4.13 Topography ...... 3.7.4.14 Ultrasonography of the Eye and Orbit...... 3.7.4.15 Macular Function Tests ...... 3.7.4.16 Scanning Laser Ophthalmoscopes ...... 3.7.4.17 Scheimpflug Camera...... 3.7.4.18 Autorefractors ...... 3.7.4.19 Optical Coherence Tomography ...... 3.7.4.20 Optical Aids ...... 3.7.5 Magnifiers ...... 3.7.5.1 Telescopes ...... 3.7.5.2 Prisms ...... 3.7.5.3 High-Add Spectacles...... 3.7.5.4 Nonoptical Aids ...... 3.7.6 Electronic Devices ...... 3.7.6.1 Lighting, Glare Control, and Contrast Enhancement ...... 3.7.6.2 Nonvisual Assistance ...... 3.7.6.3 Eccentric Viewing or Fixation Training ...... 3.7.6.4 Instruction and Training...... 3.7.7 Approach to the Patient With Low Vision ...... 3.7.8

Physical Optics ...... 3.8

The Corpuscular Theory of Light ...... 3.8.1 Diffraction ...... 3.8.2 The Speed of Light ...... 3.8.3 The Superposition of Waves ...... 3.8.4 Coherence ...... 3.8.5 Electromagnetic Waves ...... 3.8.6 Polarization ...... 3.8.6.1 Refractive Index and Dispersion ...... 3.8.6.2 Reflection, Transmission, and Absorption ...... 3.8.6.3 The Electromagnetic Spectrum ...... 3.8.6.4 Frequency and Color...... 3.8.6.5 Energy in an Electromagnetic Wave ...... 3.8.6.6 Quantum Theory ...... 3.8.7 Light Sources ...... 3.8.8 Thermal Sources ...... 3.8.8.1 Luminescent Sources...... 3.8.8.2 Fluorescence ...... 3.8.8.3 Phosphorescence ...... 3.8.8.4 Lasers ...... 3.8.8.5 Light–Tissue Interactions ...... 3.8.9 Photocoagulation ...... 3.8.9.1 Photoablation ...... 3.8.9.2 Photodisruption ...... 3.8.9.3 Photoactivation ...... 3.8.9.4 Light Scattering ...... 3.8.10 Rayleigh Scattering ...... 3.8.10.1 Mie Scattering ...... 3.8.10.2 The Tyndall Effect ...... 3.8.10.3 Radiometry and Photometry ...... 3.8.11

11 Light Hazards ...... 3.8.12 Clinical Applications ...... 3.8.13 Polarization ...... 3.8.13.1 Interference ...... 3.8.13.2 Diffraction ...... 3.8.13.3 Imaging and the Point Spread Function ...... 3.8.14 Image Quality—Modulation Transfer Function ...... 3.8.15

12 CONTENT OUTLINE

Content Outline Number

SECTION 4: OPHTHALMIC PATHOLOGY AND INTRAOCULAR TUMORS ...... 4.0

Scored as part of Subtest 4: Ophthalmic Pathology and Intraocular Tumors

Wound Repair ...... 4.1

General Aspects of Wound Repair ...... 4.1.1 Healing in Specific Ocular Tissues ...... 4.1.2 Cornea ...... 4.1.2.1 Sclera ...... 4.1.2.2 Uvea ...... 4.1.2.3 Lens ...... 4.1.2.4 Retina ...... 4.1.2.5 Vitreous ...... 4.1.2.6 Eyelid, Orbit, and Lacrimal Tissues ...... 4.1.2.7 Histologic Sequelae of Ocular Trauma ...... 4.1.3

Specimen Handling ...... 4.2

Communication ...... 4.2.1 Fixatives for Tissue Preservation ...... 4.2.2 Orientation ...... 4.2.3 Gross Dissection ...... 4.2.4 Processing and Staining ...... 4.2.5 Tissue Processing ...... 4.2.5.1 Tissue Staining and Slide Preparation ...... 4.2.5.2

Special Procedures ...... 4.3

Immunohistochemistry ...... 4.3.1 Flow Cytometry, Molecular Pathology, and Diagnostic Electron Microscopy ...... 4.3.2 Flow Cytometry ...... 4.3.2.1 Molecular Pathology...... 4.3.2.2 Diagnostic Electron Microscopy ...... 4.3.2.3 Special Techniques ...... 4.3.3 Fine-Needle Aspiration Biopsy ...... 4.3.3.1 Frozen Section ...... 4.3.3.2

Conjunctiva ...... 4.4

Topography ...... 4.4.1 Congenital Anomalies ...... 4.4.2 Choristomas ...... 4.4.2.1 Hamartomas ...... 4.4.2.2 Inflammations ...... 4.4.3 Papillary Versus Follicular Conjunctivitis ...... 4.4.3.1 Granulomatous Conjunctivitis ...... 4.4.3.2 Infectious Conjunctivitis ...... 4.4.3.3 Noninfectious Conjunctivitis ...... 4.4.3.4 Pyogenic Granuloma (Exuberant Granulation Tissue) ...... 4.4.3.5 Degenerations ...... 4.4.4

13 Pinguecula and Pterygium ...... 4.4.4.1 Amyloid Deposits ...... 4.4.4.2 Epithelial Inclusion Cyst ...... 4.4.4.3 Neoplasia ...... 4.4.5 Squamous Lesions...... 4.4.5.1 Melanocytic Lesions ...... 4.4.5.2 Lymphoid Lesions ...... 4.4.5.3 Glandular Lesions ...... 4.4.5.4 Other Neoplasms ...... 4.4.5.5

Cornea ...... 4.5

Topography ...... 4.5.1 Congenital Anomalies ...... 4.5.2 Dermoid ...... 4.5.2.1 Peters Anomaly ...... 4.5.2.2 Inflammations ...... 4.5.3 Infectious Keratitis ...... 4.5.3.1 Noninfectious Keratitis ...... 4.5.3.2 Degenerations and Dystrophies ...... 4.5.4 Degenerations ...... 4.5.4.1 Dystrophies ...... 4.5.4.2 Ectatic Disorders ...... 4.5.5 Keratoconus ...... 4.5.5.1 Neoplasia ...... 4.5.6

Anterior Chamber and Trabecular Meshwork ...... 4.6

Topography ...... 4.6.1 Congenital Anomalies ...... 4.6.2 Primary Congenital Glaucoma ...... 4.6.2.1 Anterior Segment Dysgenesis ...... 4.6.2.2 Degenerations ...... 4.6.3 Iridocorneal Endothelial Syndrome ...... 4.6.3.1 Secondary Glaucoma...... 4.6.3.2 Neoplasia ...... 4.6.4

Sclera ...... 4.7

Topography ...... 4.7.1 Congenital Anomalies ...... 4.7.2 Microphthalmos ...... 4.7.3 Inflammations ...... 4.7.4 Episcleritis ...... 4.7.4.1 Scleritis ...... 4.7.4.2 Degenerations ...... 4.7.5 Senile Calcific Plaque ...... 4.7.5.1 Scleral Staphyloma ...... 4.7.5.2 Neoplasia ...... 4.7.6

Lens ...... 4.8

Topography ...... 4.8.1 Congenital Anomalies ...... 4.8.2 Congenital Aphakia ...... 4.8.2.1 Anterior Lenticonus and Lentiglobus...... 4.8.2.2

14 Posterior Lenticonus (Lentiglobus) ...... 4.8.2.3 Inflammations ...... 4.8.3 Phacoantigenic Uveitis ...... 4.8.3.1 Propionibacterium acnes Endophthalmitis ...... 4.8.3.2 Degenerations ...... 4.8.4 Cataract and Other Abnormalities ...... 4.8.4.1 Pathology of Intraocular Lenses ...... 4.8.5

Vitreous ...... 4.9

Topography ...... 4.9.1 Congenital Anomalies ...... 4.9.2 Persistent Fetal Vasculature ...... 4.9.2.1 Other ...... 4.9.2.2 Degenerations ...... 4.9.3 Syneresis and Aging ...... 4.9.3.1 Posterior Vitreous Detachment ...... 4.9.3.2 Rhegmatogenous Retinal Detachment and Proliferative Vitreoretinopathy ...... 4.9.3.3 Macular Holes ...... 4.9.3.4 Hemorrhage ...... 4.9.3.5 Asteroid Hyalosis ...... 4.9.3.6 Vitreous Amyloidosis ...... 4.9.3.7 Neoplasia ...... 4.9.4 Intraocular Lymphoma ...... 4.9.4.1

Retina and Retinal Pigment Epithelium ...... 4.10

Topography ...... 4.10.1 Neurosensory Retina ...... 4.10.1.1 Retinal Pigment Epithelium ...... 4.10.1.2 Congenital Anomalies ...... 4.10.2 Albinism ...... 4.10.2.1 Myelinated Nerve Fibers ...... 4.10.2.2 Vascular Anomalies ...... 4.10.2.3 Congenital Hypertrophy of the RPE ...... 4.10.2.4 Inflammations ...... 4.10.3 Infectious ...... 4.10.3.1 Noninfectious ...... 4.10.3.2 Degenerations ...... 4.10.4 Typical and Reticular Peripheral Cystoid Degeneration and Retinoschisis ...... 4.10.4.1 Lattice Degeneration ...... 4.10.4.2 Paving-Stone Degeneration ...... 4.10.4.3 Ischemia ...... 4.10.4.4 Specific Ischemic Retinal Disorders ...... 4.10.4.5 Diabetic Retinopathy ...... 4.10.4.6 Retinopathy of Prematurity ...... 4.10.4.7 Age-Related Macular Degeneration ...... 4.10.4.8 Polypoidal Choroidal Vasculopathy ...... 4.10.4.9 Macular Dystrophies ...... 4.10.4.10 Diffuse Photoreceptor Dystrophies ...... 4.10.4.11 Neoplasia ...... 4.10.5 Retinoblastoma ...... 4.10.5.1 Retinocytoma ...... 4.10.5.2 Medulloepithelioma ...... 4.10.5.3 Fuchs Adenoma ...... 4.10.5.4 Combined Hamartoma of the Retina and RPE ...... 4.10.5.5

15 Adenomas and Adenocarcinomas of the RPE ...... 4.10.5.6

Uveal Tract ...... 4.11

Topography ...... 4.11.1 Iris...... 4.11.1.1 Ciliary Body ...... 4.11.1.2 Choroid ...... 4.11.1.3 Congenital Anomalies ...... 4.11.2 Aniridia ...... 4.11.2.1 Coloboma ...... 4.11.2.2 Inflammations ...... 4.11.3 Infectious ...... 4.11.3.1 Degenerations ...... 4.11.4 Sympathetic ophthalmica ...... 4.11.5 Vogt-Koyanagi-Harada syndrome ...... 4.11.6 Sarcoidosis ...... 4.11.7 Neoplasia ...... 4.11.8 Iris...... 4.11.8.1 Choroid and Ciliary Body ...... 4.11.8.2 Metastatic Tumors ...... 4.11.8.3 Other Uveal Tumors ...... 4.11.8.4

Eyelids ...... 4.12

Topography ...... 4.12.1 Congenital Anomalies ...... 4.12.2 Inflammations ...... 4.12.3 Infectious ...... 4.12.3.1 Noninfectious ...... 4.12.3.2 Degenerations ...... 4.12.4 Xanthelasma ...... 4.12.4.1 Amyloidosis ...... 4.12.4.2 Cysts ...... 4.12.5 Epidermoid Cysts ...... 4.12.5.1 Ductal Cysts ...... 4.12.5.2 Neoplasia ...... 4.12.6 Epidermal Neoplasms ...... 4.12.6.1 Dermal Neoplasms...... 4.12.6.2 Neoplasms and Proliferations of the Dermal Appendages ...... 4.12.6.3 Melanocytic Neoplasms ...... 4.12.6.4

Orbit ...... 4.13

Topography ...... 4.13.1 Congenital Anomalies ...... 4.13.2 Inflammations ...... 4.13.3 Noninfectious ...... 4.13.3.1 Infectious ...... 4.13.3.2 Degenerations ...... 4.13.4 Neoplasia ...... 4.13.5 Lacrimal Gland Neoplasia ...... 4.13.5.1 Lymphoproliferative Lesions ...... 4.13.5.2 Soft-Tissue Tumors ...... 4.13.5.3 Vascular Tumors ...... 4.13.5.4 Nerve Sheath Tumors ...... 4.13.5.5

16 Bony Lesions of the Orbit ...... 4.13.5.6 Secondary Tumors ...... 4.13.5.7

Optic Nerve ...... 4.14

Topography ...... 4.14.1 Congenital Anomalies ...... 4.14.2 Inflammations ...... 4.14.3 Infectious ...... 4.14.3.1 Noninfectious ...... 4.14.3.2 Degenerations ...... 4.14.4 Optic Atrophy ...... 4.14.4.1 Drusen ...... 4.14.4.2 Neoplasia ...... 4.14.5 Melanocytoma ...... 4.14.5.1 Glioma ...... 4.14.5.2 Meningioma ...... 4.14.5.3

Melanocytic Tumors ...... 4.15

Iris Nevus ...... 4.15.1 Nevus of the Ciliary Body or Choroid ...... 4.15.2 Melanocytoma of the Iris, Ciliary Body, or Choroid ...... 4.15.3 Iris Melanoma ...... 4.15.4 Melanoma of the Ciliary Body or Choroid ...... 4.15.5 Clinical Characteristics ...... 4.15.5.1 Diagnostic Evaluation...... 4.15.5.2 Differential Diagnosis ...... 4.15.5.3 Classification ...... 4.15.5.4 Metastatic Evaluation ...... 4.15.5.5 Treatment ...... 4.15.5.6 Prognosis and Prognostic Factors ...... 4.15.5.7 Pigmented Epithelial Tumors of the Uvea and Retina ...... 4.15.6

Angiomatous Tumors ...... 4.16

Hemangiomas ...... 4.16.1 Choroidal Hemangiomas ...... 4.16.1.1 Retinal Angiomas ...... 4.16.1.2 Arteriovenous Malformation ...... 4.16.2

Retinoblastoma ...... 4.17

Genetic Counseling ...... 4.17.1 Diagnostic Evaluation ...... 4.17.2 Clinical Examination ...... 4.17.2.1 Differential Diagnosis ...... 4.17.2.2 Classification ...... 4.17.3 Associated Conditions...... 4.17.4 Retinocytoma ...... 4.17.4.1 Primitive Neuroectodermal Tumor ...... 4.17.4.2 Treatment ...... 4.17.5 Enucleation ...... 4.17.5.1 Chemotherapy ...... 4.17.5.2 Laser Therapy Including Transpupillary Thermotherapy ...... 4.17.5.3 Cryotherapy ...... 4.17.5.4

17 External-Beam Radiation Therapy ...... 4.17.5.5 Plaque Radiotherapy (Brachytherapy) ...... 4.17.5.6 Prospective Trials ...... 4.17.6 Spontaneous Regression ...... 4.17.7 Prognosis ...... 4.17.8

Ocular Involvement in Systemic Malignancies ...... 4.18

Secondary Tumors of the Eye ...... 4.18.1 Metastatic Carcinoma ...... 4.18.1.1 Direct Intraocular Extension ...... 4.18.1.2 Lymphomatous Tumors ...... 4.18.2 Primary Intraocular Lymphoma ...... 4.18.2.1 Uveal Lymphoproliferative Lesions ...... 4.18.2.2 Secondary Involvement of Systemic Lymphoma ...... 4.18.2.3 Ocular Manifestations of Leukemia ...... 4.18.3

18 CONTENT OUTLINE

Content Outline Number

SECTION 5: NEURO-OPHTHALMOLOGY ...... 5.0

Scored as part of Subtest 5: Neuro-Ophthalmology

Neuro-Ophthalmic Anatomy ...... 5.1

Bony Anatomy ...... 5.1.1 Skull Base ...... 5.1.1.1 The Orbit ...... 5.1.1.2 Vascular Anatomy ...... 5.1.2 Arterial System ...... 5.1.2.1 Venous System ...... 5.1.2.2 Afferent Visual Pathways ...... 5.1.3 Retina...... 5.1.3.1 Optic Nerve ...... 5.1.3.2 Optic Chiasm ...... 5.1.3.3 Optic Tract...... 5.1.3.4 Cortex ...... 5.1.3.5 Efferent Visual System (Ocular Motor Pathways)...... 5.1.4 Cortical Input ...... 5.1.4.1 Brainstem ...... 5.1.4.2 Ocular Motor Cranial Nerves...... 5.1.4.3 Extraocular Muscles ...... 5.1.4.4 Sensory and Facial Motor Anatomy ...... 5.1.5 Trigeminal Nerve (CN V)...... 5.1.5.1 Facial Nerve (CN VII)...... 5.1.5.2 Eyelids ...... 5.1.5.3 Ocular Autonomic Pathways ...... 5.1.6 Sympathetic Pathways ...... 5.1.6.1 Parasympathetic Pathways ...... 5.1.6.2

Neuroimaging in Neuro-Ophthalmology...... 5.2

Computed Tomography...... 5.2.1 Magnetic Resonance Imaging ...... 5.2.2 Vascular Imaging ...... 5.2.3 Catheter or Contrast Angiography ...... 5.2.3.1 Magnetic Resonance Angiography and Magnetic Resonance Venography ...... 5.2.3.2 Computed Tomography Angiography and Computed Tomography Venography ....5.2.3.3 Metabolic and Functional Imaging Modalities ...... 5.2.3.4 Sonography ...... 5.2.3.5 Retinal and Nerve Fiber Layer Imaging ...... 5.2.3.6 Fundamental Concepts in Localization ...... 5.2.4

The Patient With Decreased Vision: Evaluation ...... 5.3

History ...... 5.3.1 Unilateral Versus Bilateral Involvement ...... 5.3.1.1 Time Course of Vision Loss ...... 5.3.1.2 Associated Symptoms ...... 5.3.1.3 Examination ...... 5.3.2

19 Best-Corrected Visual Acuity ...... 5.3.2.1 Color Vision Testing ...... 5.3.2.2 Pupillary Testing ...... 5.3.2.3 Fundus Examination ...... 5.3.2.4 Visual Field Evaluation ...... 5.3.2.5 Adjunctive Testing ...... 5.3.2.6

The Patient With Decreased Vision: Classification and Management ...... 5.4

Ocular Media Abnormality ...... 5.4.1 Retinopathy...... 5.4.2 Acute Idiopathic Blind-Spot Enlargement and Multiple Evanescent White Dot Syndrome ...... 5.4.2.1 Vitamin A Deficiency ...... 5.4.2.2 Hydroxychloroquine and Chloroquine Retinopathy ...... 5.4.2.3 Cone Dystrophy ...... 5.4.2.4 Paraneoplastic Syndromes ...... 5.4.2.5 Optic Neuropathy ...... 5.4.3 Visual Field Patterns in Optic Neuropathy ...... 5.4.3.1 Anterior Optic Neuropathies With Optic Disc Edema ...... 5.4.3.2 Anterior Optic Neuropathies Without Optic Disc Edema ...... 5.4.3.3 Posterior Optic Neuropathies ...... 5.4.3.4 Optic Atrophy...... 5.4.3.5 Chiasmal Lesions ...... 5.4.4 Visual Field Loss Patterns ...... 5.4.4.1 Etiology of Chiasmal Disorders ...... 5.4.4.2 Retrochiasmal Lesions ...... 5.4.5 Optic Tract...... 5.4.5.1 Lateral Geniculate Body...... 5.4.5.2 Temporal Lobe ...... 5.4.5.3 Parietal Lobe ...... 5.4.5.4 Occipital Lobe...... 5.4.5.5 Visual Rehabilitation...... 5.4.6

The Patient With Transient Visual Loss...... 5.5

Transient Monocular Visual Loss ...... 5.5.1 Ocular Causes...... 5.5.1.1 Orbital Causes...... 5.5.1.2 Systemic Causes ...... 5.5.1.3 Vasospasm, Hyperviscosity, and Hypercoagulability ...... 5.5.1.4 Transient Binocular Visual Loss ...... 5.5.2 Migraine ...... 5.5.2.1 Occipital Mass Lesions ...... 5.5.2.2 Occipital Ischemia ...... 5.5.2.3 Occipital Seizures ...... 5.5.2.4

The Patient With Illusions, Hallucinations, and Disorders of Higher Cortical Function ...... 5.6

The Patient With Visual Illusions and Distortions ...... 5.6.1 Ocular Origin ...... 5.6.1.1 Optic Nerve Origin ...... 5.6.1.2 Cortical Origin...... 5.6.1.3 The Patient With Hallucinations ...... 5.6.2 Ocular Origin ...... 5.6.2.1 Optic Nerve Origin ...... 5.6.2.2

20 Cortical Origin...... 5.6.2.3 The Patient With Disorders of Higher Cortical Function...... 5.6.3 Disorders of Recognition...... 5.6.3.1 Disorders of Visual–Spatial Relationships ...... 5.6.3.2 Disorders of Awareness of Vision or Visual Deficit ...... 5.6.3.3

The Patient With Supranuclear Disorders of Ocular Motility ...... 5.7

Anatomy and Clinical Testing of the Functional Classes of Eye Movements ...... 5.7.1 Ocular Stability ...... 5.7.1.1 Vestibular Ocular Reflex ...... 5.7.1.2 Optokinetic Nystagmus ...... 5.7.1.3 Saccadic System ...... 5.7.1.4 Pursuit System ...... 5.7.1.5 Vergence ...... 5.7.1.6 Clinical Disorders of the Ocular Motor Systems ...... 5.7.2 Ocular Stability Dysfunction ...... 5.7.2.1 Vestibular Ocular Dysfunction ...... 5.7.2.2 Optokinetic Nystagmus Dysfunction ...... 5.7.2.3 Saccadic Dysfunction ...... 5.7.2.4 Pursuit Dysfunction ...... 5.7.2.5 Vergence Disorders ...... 5.7.2.6

The Patient With Diplopia ...... 5.8

Monocular Diplopia ...... 5.8.1 Differentiating Paretic From Restrictive Etiologies of Diplopia ...... 5.8.2 Comitant and Incomitant Deviations ...... 5.8.3 Localization ...... 5.8.4 Supranuclear Causes of Diplopia ...... 5.8.5 Skew Deviation ...... 5.8.5.1 Thalamic Esodeviation ...... 5.8.5.2 Vergence Dysfunction...... 5.8.5.3 Nuclear Causes of Diplopia ...... 5.8.6 Internuclear Causes of Diplopia ...... 5.8.7 One-and-a-Half Syndrome ...... 5.8.7.1 Infranuclear Causes of Diplopia ...... 5.8.8 Third Nerve Palsy ...... 5.8.8.1 Fourth Nerve Palsy ...... 5.8.8.2 Sixth Nerve Palsy ...... 5.8.8.3 Neuromyotonia ...... 5.8.8.4 Paresis of More Than One Cranial Nerve ...... 5.8.8.5 Cavernous Sinus and Superior Orbital Fissure Involvement ...... 5.8.8.6 Neuromuscular Junction Causes of Diplopia ...... 5.8.8.7 Myopathic, Restrictive, and Orbital Causes of Diplopia ...... 5.8.9 Thyroid Eye Disease...... 5.8.9.1 Posttraumatic Restriction ...... 5.8.9.2 Post–Cataract Extraction Restriction ...... 5.8.9.3 Orbital Myositis ...... 5.8.9.4 Neoplastic Involvement ...... 5.8.9.5 Brown Syndrome ...... 5.8.9.6

The Patient With Nystagmus or Spontaneous Eye Movement Disorders ...... 5.9

Early-Onset (Childhood) Nystagmus ...... 5.9.1 Infantile Nystagmus Syndrome (Congenital Nystagmus) ...... 5.9.1.1

21 Fusional Maldevelopment Nystagmus Syndrome (Latent Nystagmus) ...... 5.9.1.2 Monocular Nystagmus of Childhood...... 5.9.1.3 Spasmus Nutans ...... 5.9.1.4 Gaze-Evoked Nystagmus ...... 5.9.2 Rebound Nystagmus ...... 5.9.2.1 Vestibular Nystagmus ...... 5.9.3 Peripheral Vestibular Nystagmus ...... 5.9.3.1 Central Forms of Vestibular Nystagmus ...... 5.9.3.2 Acquired Pendular Nystagmus ...... 5.9.4 Oculopalatal Myoclonus or Tremor...... 5.9.4.1 See-Saw Nystagmus ...... 5.9.5 Dissociated Nystagmus ...... 5.9.6 Saccadic Intrusions ...... 5.9.7 Saccadic Intrusions With Normal Intersaccadic Intervals ...... 5.9.7.1 Saccadic Intrusions Without Normal Intersaccadic Intervals ...... 5.9.7.2 Voluntary Flutter (“Nystagmus”) ...... 5.9.7.3 Additional Eye Movement Disorders ...... 5.9.8 Convergence-Retraction Nystagmus...... 5.9.8.1 Superior Oblique Myokymia ...... 5.9.8.2 Oculomasticatory Myorhythmia ...... 5.9.8.3 Treatment of Nystagmus and Other Eye Movement Disorders ...... 5.9.9 Eye Movements in Comatose Patients ...... 5.9.10 Ocular Bobbing ...... 5.9.11

The Patient With Pupillary Abnormalities ...... 5.10

Pupil Irregularity ...... 5.10.1 Anisocoria ...... 5.10.2 Anisocoria Equal in Dim and Bright Light ...... 5.10.2.1 Anisocoria Greater in Dim Light ...... 5.10.2.2 Anisocoria Greater in Bright Light ...... 5.10.2.3 Disorders of Pupillary Reactivity: Light–Near Dissociation ...... 5.10.3 Afferent Visual Pathway ...... 5.10.3.1 Midbrain ...... 5.10.3.2 Aberrant Regeneration ...... 5.10.3.3

The Patient With Eyelid or Facial Abnormalities ...... 5.11

Examination Techniques ...... 5.11.1 Ptosis...... 5.11.2 Congenital Ptosis...... 5.11.2.1 Acquired Ptosis ...... 5.11.2.2 Pseudoptosis ...... 5.11.2.3 Apraxia of Eyelid Opening ...... 5.11.2.4 Eyelid Retraction ...... 5.11.3 Abnormalities of Facial Movement ...... 5.11.4 Seventh Nerve Disorders ...... 5.11.5 Disorders of Underactivity of the Seventh Nerve ...... 5.11.5.1 Disorders of Overactivity of the Seventh Nerve ...... 5.11.5.2

The Patient With Head, Ocular, or Facial Pain ...... 5.12

Evaluation of Headache ...... 5.12.1 Migraine and Tension-type Headache ...... 5.12.1.1 Trigeminal Autonomic Cephalgias and Hemicrania Continua...... 5.12.1.2 Idiopathic Stabbing Headache ...... 5.12.1.3

22 Inherited Encephalopathies Resembling Migraine ...... 5.12.1.4 Ocular and Orbital Causes of Pain ...... 5.12.2 Trochlear Headache and Trochleitis...... 5.12.2.1 Photophobia ...... 5.12.2.2 Facial Pain ...... 5.12.3 Trigeminal Neuralgia...... 5.12.3.1 Glossopharyngeal Neuralgia ...... 5.12.3.2 Occipital Neuralgia ...... 5.12.3.3 Temporomandibular Disease ...... 5.12.3.4 Carotid Dissection ...... 5.12.3.5 Herpes Zoster Ophthalmicus ...... 5.12.3.6 Neoplastic Processes ...... 5.12.3.7 Mental Nerve Neuropathy ...... 5.12.3.8

The Patient With Nonorganic Ophthalmic Disorders ...... 5.13

Examination Techniques ...... 5.13.1 Afferent Visual Pathway ...... 5.13.1.1 Efferent Visual Pathway ...... 5.13.1.2 Management of the Patient With Nonorganic Complaints ...... 5.13.2

Selected Systemic Conditions With Neuro-Ophthalmic Signs ...... 5.14

Immunologic Disorders...... 5.14.1 Giant Cell Arteritis...... 5.14.1.1 Multiple Sclerosis...... 5.14.1.2 Myasthenia Gravis ...... 5.14.1.3 Thyroid Eye Disease...... 5.14.1.4 Sarcoidosis...... 5.14.1.5 Inherited Disorders With Neuro-Ophthalmic Signs ...... 5.14.2 Myopathies ...... 5.14.2.1 Neurocutaneous Syndromes...... 5.14.2.2 Selected Neuro-Ophthalmic Disorders Associated With Pregnancy ...... 5.14.3 Posterior Reversible Encephalopathy Syndrome ...... 5.14.3.1 Lymphocytic Hypophysitis...... 5.14.3.2 Cerebrovascular Disorders ...... 5.14.4 Transient Visual Loss ...... 5.14.4.1 Vertebrobasilar System Disease ...... 5.14.4.2 Cerebral Aneurysms ...... 5.14.4.3 Arterial Dissection...... 5.14.4.4 Arteriovenous Malformations ...... 5.14.4.5 Cerebral Venous Thrombosis ...... 5.14.4.6 Neuro-Ophthalmic Manifestations of Infectious Diseases...... 5.14.5 Human Immunodeficiency Virus Infection ...... 5.14.5.1 Herpesvirus ...... 5.14.5.2 Mycobacterium ...... 5.14.5.3 Syphilis...... 5.14.5.4 Progressive Multifocal Leukoencephalopathy ...... 5.14.5.5 Toxoplasmosis ...... 5.14.5.6 Lyme Disease...... 5.14.5.7 Fungal Infections ...... 5.14.5.8 Prion Diseases ...... 5.14.5.9 Radiation Therapy ...... 5.14.6

23 CONTENT OUTLINE

Content Outline Number

SECTION 6: PEDIATRIC OPHTHALMOLOGY AND STRABISMUS ...... 6.0

Scored as part of Subtest 6: Pediatric Ophthalmology and Strabismus

The Pediatric Eye Examination ...... 6.1

Preparation ...... 6.1.1 Examination: General Considerations and Strategies ...... 6.1.2 Examination: Specific Elements ...... 6.1.3 Visual Acuity Assessment ...... 6.1.3.1 Alternative Methods of Visual Acuity Assessment in Preverbal Children ...... 6.1.3.2 Red Reflex Examination (Brückner Test) ...... 6.1.3.3 Dynamic Retinoscopy ...... 6.1.3.4 Visual Field Testing ...... 6.1.3.5 Pupil Testing ...... 6.1.3.6 Anterior Segment Examination ...... 6.1.3.7 Intraocular Pressure Measurement ...... 6.1.3.8 Cycloplegic Refraction ...... 6.1.3.9 Fundus Examination ...... 6.1.3.10 Examination of the Uncooperative Child ...... 6.1.4

Strabismus Terminology ...... 6.2

Strabismus Classification Terms ...... 6.2.1 Age of Onset ...... 6.2.1.1 Fixation ...... 6.2.1.2 Variation of the Deviation Size With Gaze Position or Fixating Eye ...... 6.2.1.3 Miscellaneous Terms ...... 6.2.1.4

Anatomy of the Extraocular Muscles...... 6.3

Origin, Course, Insertion, Innervation, and Action of the Extraocular Muscles ...... 6.3.1 Horizontal Rectus Muscles ...... 6.3.1.1 Vertical Rectus Muscles...... 6.3.1.2 Oblique Muscles ...... 6.3.1.3 Levator Palpebrae Superioris Muscle ...... 6.3.1.4 Relationship of the Rectus Muscle Insertions ...... 6.3.1.5 Blood Supply of the Extraocular Muscles ...... 6.3.2 Structure of the Extraocular Muscles ...... 6.3.3 Orbital and Fascial Relationships ...... 6.3.4 Adipose Tissue ...... 6.3.4.1 Muscle Cone ...... 6.3.4.2 Muscle Capsule ...... 6.3.4.3 The Tenon Capsule ...... 6.3.4.4 Pulley System...... 6.3.4.5 Lockwood’s Ligament ...... 6.3.4.6 Anatomical Considerations During Surgery ...... 6.3.5

Amblyopia ...... 6.4

Epidemiology ...... 6.4.1

24 Detection and Screening ...... 6.4.2 Pathophysiology...... 6.4.3 Classification...... 6.4.4 Strabismic Amblyopia ...... 6.4.4.1 Refractive Amblyopia ...... 6.4.4.2 Visual Deprivation Amblyopia ...... 6.4.4.3 Evaluation ...... 6.4.5 Treatment ...... 6.4.6 Address issue causing form vision deprivation (cataract, ptosis, corneal opacities) 6.4.6.1 Refractive Correction ...... 6.4.6.2 Occlusion and Penalization ...... 6.4.6.3 Complications of Therapy ...... 6.4.6.4

Motor Physiology ...... 6.5

Axes of Fick and Ocular Rotations...... 6.5.1 Positions of Gaze ...... 6.5.2 Extraocular Muscle Action ...... 6.5.3 Eye Movements ...... 6.5.4 Monocular Eye Movements ...... 6.5.4.1 Binocular Eye Movements ...... 6.5.4.2 Supranuclear Control Systems for Eye Movement ...... 6.5.4.3

Sensory Physiology and Pathology ...... 6.6

Physiology of Normal Binocular Vision ...... 6.6.1 Retinal Correspondence ...... 6.6.1.1 Fusion ...... 6.6.1.2 Selected Aspects of the Neurophysiology of Vision ...... 6.6.2 Visual Development ...... 6.6.2.1 Effects of Abnormal Visual Experience on the Retinogeniculocortical Pathway ...... 6.6.2.2 Abnormalities of Binocular Vision ...... 6.6.3 Visual Confusion...... 6.6.3.1 Diplopia ...... 6.6.3.2 Sensory Adaptations in Strabismus ...... 6.6.4 Suppression ...... 6.6.4.1 Anomalous Retinal Correspondence ...... 6.6.4.2 Monofixation Syndrome ...... 6.6.4.3

Diagnostic Evaluation of Strabismus and Torticollis ...... 6.7

History and Presenting Features of Strabismus ...... 6.7.1 Assessment of Ocular Alignment ...... 6.7.2 Positions of Gaze ...... 6.7.2.1 Cover Tests ...... 6.7.2.2 Corneal Light Reflex Tests ...... 6.7.2.3 Assessment of Eye Movements ...... 6.7.3 Ductions and Versions ...... 6.7.3.1 Convergence/Divergence ...... 6.7.3.2 Fusional Vergence...... 6.7.3.3 Special Tests ...... 6.7.4 Motor Tests ...... 6.7.4.1 Assessment of the Field of Single Binocular Vision ...... 6.7.4.2 3-Step Test...... 6.7.4.3 Prism Adaptation Test ...... 6.7.4.4 Forced Ductions ...... 6.7.4.5

25 Active Force Generation ...... 6.7.4.6 Saccadic Velocity ...... 6.7.4.7 Torticollis: Differential Diagnosis and Evaluation ...... 6.7.5 Ocular Torticollis ...... 6.7.5.1 Tests of Sensory Adaptation and Binocular Cooperation ...... 6.7.6 Red-Glass Test ...... 6.7.6.1 Bagolini Lenses ...... 6.7.6.2 4D Base-Out Prism Test ...... 6.7.6.3 Afterimage Test ...... 6.7.6.4 Amblyoscope Testing ...... 6.7.6.5 Worth 4-Dot Test ...... 6.7.6.6 Stereoacuity Testing ...... 6.7.6.7

Esodeviations ...... 6.8

Epidemiology ...... 6.8.1 Pseudoesotropia ...... 6.8.2 Infantile (Congenital) Esotropia ...... 6.8.3 Pathogenesis...... 6.8.3.1 Evaluation ...... 6.8.3.2 Management ...... 6.8.3.3 Accommodative Esotropia ...... 6.8.4 Pathogenesis and Types of Accommodative Esotropia ...... 6.8.4.1 Evaluation ...... 6.8.4.2 Management ...... 6.8.4.3 Acquired Nonaccommodative Esotropias ...... 6.8.5 Basic Acquired Nonaccommodative Esotropia ...... 6.8.5.1 Cyclic Esotropia ...... 6.8.5.2 Sensory Esotropia ...... 6.8.5.3 Divergence Insufficiency ...... 6.8.5.4 Spasm of the Near Reflex ...... 6.8.5.5 Consecutive Esotropia ...... 6.8.5.6 Nystagmus and Esotropia ...... 6.8.6 Incomitant Esotropia ...... 6.8.7 Sixth Nerve Palsy ...... 6.8.7.1 Other Forms of Incomitant Esotropia...... 6.8.7.2

Exodeviations ...... 6.9

Pseudoexotropia ...... 6.9.1 Exophoria ...... 6.9.2 Intermittent Exotropia ...... 6.9.3 Clinical Characteristics ...... 6.9.3.1 Evaluation ...... 6.9.3.2 Classification ...... 6.9.3.3 Treatment ...... 6.9.3.4 Convergence Insufficiency Exotropia ...... 6.9.4 Constant Exotropia...... 6.9.5 Infantile Exotropia ...... 6.9.5.1 Sensory Exotropia ...... 6.9.5.2 Consecutive Exotropia ...... 6.9.5.3 Other Forms of Exotropia ...... 6.9.6 Exotropic Duane Retraction Syndrome ...... 6.9.6.1 Neuromuscular Abnormalities ...... 6.9.6.2 Dissociated Horizontal ...... 6.9.6.3 Convergence Paralysis ...... 6.9.6.4

26 Pattern Strabismus ...... 6.10

Etiology ...... 6.10.1 Clinical Features and Identification ...... 6.10.2 V Pattern ...... 6.10.2.1 A Pattern ...... 6.10.2.2 Management of Specific Patterns ...... 6.10.3

Vertical Deviations ...... 6.11

Incomitant Vertical Tropias ...... 6.11.1 Overelevation and Overdepression in Adduction ...... 6.11.1.1 Superior Oblique Muscle Palsy ...... 6.11.1.2 Inferior Oblique Muscle Palsy ...... 6.11.1.3 Other Incomitant Vertical Tropias ...... 6.11.1.4 Comitant Vertical Tropias ...... 6.11.2 Monocular Elevation Deficiency ...... 6.11.2.1 Orbital Floor Fractures ...... 6.11.2.2 Other Comitant Vertical Tropias ...... 6.11.2.3 Dissociated Vertical Deviation ...... 6.11.3 Clinical Features ...... 6.11.3.1 Management ...... 6.11.3.2

Special Forms of Strabismus ...... 6.12

Congenital Cranial Dysinnervation Disorders ...... 6.12.1 Duane Retraction Syndrome ...... 6.12.1.1 Congenital Fibrosis of the Extraocular Muscles ...... 6.12.1.2 Möbius Syndrome...... 6.12.1.3 Miscellaneous Special Forms of Strabismus ...... 6.12.2 Brown Syndrome ...... 6.12.2.1 Third Nerve Palsy ...... 6.12.2.2 Sixth Nerve Palsy ...... 6.12.2.3 Thyroid Eye Disease...... 6.12.2.4 Chronic Progressive External Ophthalmoplegia ...... 6.12.2.5 Myasthenia Gravis ...... 6.12.2.6 Esotropia and Hypotropia Associated With High Myopia ...... 6.12.2.7 Internuclear Ophthalmoplegia ...... 6.12.2.8 Ocular Motor Apraxia ...... 6.12.2.9 Superior Oblique Myokymia ...... 6.12.2.10 Strabismus Associated With Other Ocular Surgery ...... 6.12.2.11

Childhood Nystagmus ...... 6.13

Evaluation ...... 6.13.1 History ...... 6.13.1.1 Ocular Examination ...... 6.13.1.2 Types of Childhood Nystagmus ...... 6.13.2 Congenital Nystagmus ...... 6.13.2.1 Acquired Nystagmus...... 6.13.2.2 Nystagmus-Like Disorders ...... 6.13.3 Convergence-Retraction Nystagmus...... 6.13.3.1 Opsoclonus ...... 6.13.3.2 Treatment ...... 6.13.4 Prisms ...... 6.13.4.1

27 Surgery for Nystagmus ...... 6.13.4.2

Surgery of the Extraocular Muscles ...... 6.14

Indications for Surgery ...... 6.14.1 Planning Considerations ...... 6.14.2 Visual Acuity ...... 6.14.2.1 Incomitance ...... 6.14.2.2 Cyclovertical Strabismus...... 6.14.2.3 Prior Surgery ...... 6.14.2.4 Surgical Techniques for the Extraocular Muscles and Tendons ...... 6.14.3 Approaches to the Extraocular Muscles ...... 6.14.3.1 Rectus Muscle Weakening Procedures ...... 6.14.3.2 Rectus Muscle Strengthening Procedures ...... 6.14.3.3 Rectus Muscle Surgery for Hypotropia and Hypertropia ...... 6.14.3.4 Adjustable Sutures ...... 6.14.3.5 Oblique Muscle Weakening Procedures...... 6.14.3.6 Oblique Muscle Tightening (Strengthening) Procedures ...... 6.14.3.7 Stay Sutures ...... 6.14.3.8 Transposition Procedures ...... 6.14.3.9 Posterior Fixation...... 6.14.3.10 Complications of Strabismus Surgery ...... 6.14.4 Diplopia ...... 6.14.4.1 Unsatisfactory Alignment ...... 6.14.4.2 Iatrogenic Brown Syndrome ...... 6.14.4.3 Anti-Elevation Syndrome ...... 6.14.4.4 Lost and Slipped Muscles ...... 6.14.4.5 Pulled-in-Two Syndrome...... 6.14.4.6 Perforation of the Sclera ...... 6.14.4.7 Postoperative Infections...... 6.14.4.8 Foreign-Body Granuloma and Allergic Reaction ...... 6.14.4.9 Epithelial Cyst...... 6.14.4.10 Conjunctival Scarring ...... 6.14.4.11 Adherence Syndrome ...... 6.14.4.12 Dellen ...... 6.14.4.13 Anterior Segment Ischemia ...... 6.14.4.14 Change in Eyelid Position ...... 6.14.4.15 Refractive Changes ...... 6.14.4.16 Anesthesia for Extraocular Muscle Surgery ...... 6.14.5 Methods ...... 6.14.5.1 Postoperative Nausea and Vomiting ...... 6.14.5.2 Oculocardiac Reflex ...... 6.14.5.3 Malignant Hyperthermia ...... 6.14.5.4 Chemodenervation Using Botulinum Toxin ...... 6.14.6 Pharmacology and Mechanism of Action ...... 6.14.6.1 Indications, Techniques, and Results ...... 6.14.6.2 Complications ...... 6.14.6.3

Growth and Development of the Eye ...... 6.15

Normal Growth and Development...... 6.15.1 Dimensions of the Eye ...... 6.15.1.1 Refractive State ...... 6.15.1.2 Orbit and Ocular Adnexa ...... 6.15.1.3 Cornea, Iris, Pupil, and Anterior Chamber ...... 6.15.1.4 Intraocular Pressure ...... 6.15.1.5

28 Extraocular Muscles ...... 6.15.1.6 Retina...... 6.15.1.7 Visual Acuity and Stereoacuity ...... 6.15.1.8 Abnormal Growth and Development ...... 6.15.2

Decreased Vision in Infants and Children ...... 6.16

Normal Visual Development ...... 6.16.1 Classification of Visual Impairment in Infants and Children ...... 6.16.2 Delayed Visual Maturation ...... 6.16.2.1 Pregeniculate Visual Impairment...... 6.16.2.2 Retrogeniculate Visual Impairment, or Cerebral Visual Impairment ...... 6.16.2.3 Pediatric Low Vision Rehabilitation ...... 6.16.3

Eyelid Disorders ...... 6.17

Congenital Eyelid Disorders ...... 6.17.1 Telecanthus ...... 6.17.1.1 Cryptophthalmos ...... 6.17.1.2 Congenital Coloboma of the Eyelid ...... 6.17.1.3 Ankyloblepharon ...... 6.17.1.4 Congenital Ectropion ...... 6.17.1.5 Congenital Entropion ...... 6.17.1.6 Epiblepharon ...... 6.17.1.7 Congenital Tarsal Kink ...... 6.17.1.8 Distichiasis ...... 6.17.1.9 Euryblepharon ...... 6.17.1.10 Epicanthus ...... 6.17.1.11 Palpebral Fissure Slants ...... 6.17.1.12 Blepharophimosis–Ptosis–Epicanthus Inversus Syndrome...... 6.17.1.13 Congenital Ptosis...... 6.17.1.14 Marcus Gunn Jaw-Winking Syndrome ...... 6.17.1.15 Neoplasms and Other Noninfectious Eyelid Lesions ...... 6.17.2 Capillary Malformations ...... 6.17.2.1 Congenital Nevocellular Nevi of the Skin...... 6.17.2.2 Other Acquired Eyelid Conditions ...... 6.17.3 Excessive Blinking ...... 6.17.4

Orbital Disorders ...... 6.18

Abnormal Interocular Distance: Terminology and Associations ...... 6.18.1 Congenital and Developmental Disorders: Craniofacial Malformations ...... 6.18.2 Craniosynostosis ...... 6.18.2.1 Nonsynostotic Craniofacial Conditions ...... 6.18.2.2 Infectious and Inflammatory Conditions ...... 6.18.3 Preseptal Cellulitis ...... 6.18.3.1 Orbital Cellulitis ...... 6.18.3.2 Childhood Orbital Inflammation ...... 6.18.3.3 Neoplasms ...... 6.18.4 Differential Diagnosis ...... 6.18.4.1 Primary Malignant Neoplasms ...... 6.18.4.2 Metastatic Tumors ...... 6.18.4.3 Hematopoietic, Lymphoproliferative, and Histiocytic Neoplasms...... 6.18.4.4 Benign Tumors ...... 6.18.4.5 Ectopic Tissue Masses...... 6.18.5 Cystic Lesions ...... 6.18.5.1

29 Teratoma ...... 6.18.5.2 Ectopic Lacrimal Gland ...... 6.18.5.3

Lacrimal Drainage System Abnormalities ...... 6.19

Congenital and Developmental Anomalies ...... 6.19.1 Atresia of the Lacrimal Puncta or Canaliculi ...... 6.19.1.1 Congenital Lacrimal Fistula ...... 6.19.1.2 Dacryocystocele ...... 6.19.1.3 Nasolacrimal Duct Obstruction ...... 6.19.2 Clinical Features ...... 6.19.2.1 Nonsurgical Management ...... 6.19.2.2 Surgical Management ...... 6.19.2.3

Diseases of the Cornea, Anterior Segment, and Iris ...... 6.20

Congenital and Developmental Anomalies of the Cornea ...... 6.20.1 Abnormalities of Corneal Size and Shape ...... 6.20.1.1 Abnormalities of Peripheral Corneal Transparency...... 6.20.1.2 Abnormalities of Central and Diffuse Corneal Transparency ...... 6.20.1.3 Treatment of Corneal Opacities ...... 6.20.1.4 Congenital and Developmental Anomalies of the Globe...... 6.20.2 Microphthalmos ...... 6.20.2.1 Anophthalmos ...... 6.20.2.2 Nanophthalmos ...... 6.20.2.3 Congenital and Developmental Anomalies of the Iris and Pupil ...... 6.20.3 Abnormalities of the Iris ...... 6.20.3.1 Abnormalities in the Size, Shape, or Location of the Pupil ...... 6.20.3.2 Systemic Diseases Affecting the Cornea or Iris ...... 6.20.4 Metabolic Disorders Affecting the Cornea or Iris ...... 6.20.4.1 Other Systemic Diseases Affecting the Cornea or Iris ...... 6.20.4.2 Tumors of the Cornea, Iris, and Anterior Segment ...... 6.20.5 Cornea ...... 6.20.5.1 Iris ...... 6.20.5.2 Ciliary Body ...... 6.20.5.3 Miscellaneous Clinical Signs ...... 6.20.6 Pediatric Iris Heterochromia ...... 6.20.6.1 Anisocoria ...... 6.20.6.2

External Diseases of the Eye ...... 6.21

Infectious Conjunctivitis ...... 6.21.1 Ophthalmia Neonatorum ...... 6.21.1.1 Bacterial Conjunctivitis ...... 6.21.1.2 Viral Conjunctivitis ...... 6.21.1.3 Inflammatory Disease ...... 6.21.2 Blepharitis ...... 6.21.2.1 Ocular Allergy ...... 6.21.2.2 Ligneous Conjunctivitis ...... 6.21.2.3 Miscellaneous Conjunctival Disorders ...... 6.21.3 Papillomas...... 6.21.3.1 Conjunctival Epithelial Inclusion Cysts ...... 6.21.3.2 Conjunctival Nevi ...... 6.21.3.3 Ocular Melanocytosis ...... 6.21.3.4 Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis ...... 6.21.3.5

30 Pediatric Glaucomas ...... 6.22

Genetics ...... 6.22.1 Classification...... 6.22.2 Primary Childhood Glaucoma ...... 6.22.3 Primary Congenital Glaucoma ...... 6.22.3.1 Juvenile Open-Angle Glaucoma...... 6.22.3.2 Secondary Childhood Glaucoma ...... 6.22.4 Glaucoma Associated With Nonacquired Ocular Anomalies ...... 6.22.4.1 Glaucoma Associated With Nonacquired Systemic Disease or Syndrome ...... 6.22.4.2 Secondary Glaucoma Associated With an Acquired Condition ...... 6.22.4.3 Glaucoma Following Cataract Surgery ...... 6.22.4.4 Treatment ...... 6.22.5 Surgical Therapy ...... 6.22.5.1 Medical Therapy ...... 6.22.5.2 Prognosis and Follow-Up ...... 6.22.6

Childhood Cataracts and Other Pediatric Lens Disorders ...... 6.23

Pediatric Cataracts ...... 6.23.1 Etiology ...... 6.23.1.1 Morphology ...... 6.23.1.2 Examination ...... 6.23.1.3 Cataract Surgery in Pediatric Patients ...... 6.23.2 Timing of the Procedure...... 6.23.2.1 Intraocular Lens Use in Children ...... 6.23.2.2 Management of the Anterior Capsule ...... 6.23.2.3 Lensectomy Without Intraocular Lens Implantation ...... 6.23.2.4 Lensectomy With Intraocular Lens Implantation ...... 6.23.2.5 Postoperative Care ...... 6.23.2.6 Complications ...... 6.23.2.7 Visual Outcome After Cataract Extraction ...... 6.23.2.8 Structural or Positional Lens Abnormalities ...... 6.23.3 Congenital Aphakia ...... 6.23.3.1 Spherophakia ...... 6.23.3.2 Coloboma ...... 6.23.3.3 Dislocated Lenses in Children ...... 6.23.4 Isolated Ectopia Lentis ...... 6.23.4.1 Ectopia Lentis et Pupillae...... 6.23.4.2 Marfan Syndrome ...... 6.23.4.3 Homocystinuria ...... 6.23.4.4 Weill-Marchesani Syndrome ...... 6.23.4.5 Sulfite Oxidase Deficiency ...... 6.23.4.6 Treatment ...... 6.23.4.7

Uveitis in the Pediatric Age Group ...... 6.24

Epidemiology and Genetics ...... 6.24.1 Classification...... 6.24.2 Anterior Uveitis...... 6.24.3 Juvenile Idiopathic Arthritis ...... 6.24.3.1 Tubulointerstitial Nephritis and Uveitis Syndrome ...... 6.24.3.2 Kawasaki Disease ...... 6.24.3.3 Intermediate Uveitis ...... 6.24.4 Posterior Uveitis ...... 6.24.5 Toxoplasmosis ...... 6.24.5.1

31 Toxocariasis ...... 6.24.5.2 Panuveitis ...... 6.24.6 Sarcoidosis...... 6.24.6.1 Familial Juvenile Systemic Granulomatosis...... 6.24.6.2 Masquerade Syndromes ...... 6.24.7 Treatment of Pediatric Uveitis...... 6.24.8 Management of Inflammation...... 6.24.8.1 Surgical Treatment of Uveitis Complications ...... 6.24.8.2

Disorders of the Retina and Vitreous ...... 6.25

Congenital and Developmental Abnormalities...... 6.25.1 Persistent Fetal Vasculature ...... 6.25.1.1 Retinopathy of Prematurity ...... 6.25.1.2 Hereditary Retinal Disease ...... 6.25.1.3 Hereditary Macular Dystrophies ...... 6.25.1.4 Hereditary Vitreoretinopathies ...... 6.25.1.5 Infections ...... 6.25.2 Herpes Simplex Virus and Cytomegalovirus...... 6.25.2.1 Human Immunodeficiency Virus...... 6.25.2.2 Tumors ...... 6.25.3 Choroidal and Retinal Pigment Epithelial Lesions ...... 6.25.3.1 Retinoblastoma ...... 6.25.3.2 Acquired Disorders...... 6.25.4 Coats Disease ...... 6.25.4.1 Systemic Diseases and Disorders With Retinal Manifestations ...... 6.25.5 Diabetes Mellitus ...... 6.25.5.1 Albinism ...... 6.25.5.2 Sickle Cell ...... 6.25.5.3

Optic Disc Abnormalities ...... 6.26

Developmental Anomalies ...... 6.26.1 Optic Nerve Hypoplasia ...... 6.26.1.1 Morning Glory Disc Anomaly...... 6.26.1.2 Coloboma of the Optic Nerve ...... 6.26.1.3 Myelinated Retinal Nerve Fibers ...... 6.26.1.4 Tilted Disc Syndrome...... 6.26.1.5 Bergmeister Papilla ...... 6.26.1.6 Megalopapilla ...... 6.26.1.7 Peripapillary Staphyloma ...... 6.26.1.8 Melanocytoma ...... 6.26.1.9 Optic Atrophy ...... 6.26.2 Optic Neuritis ...... 6.26.3 Papilledema ...... 6.26.4 Idiopathic Intracranial Hypertension ...... 6.26.5 Pseudopapilledema...... 6.26.6 Drusen ...... 6.26.6.1

Ocular Trauma in Childhood ...... 6.27

Orbital Fractures ...... 6.27.1 Nonaccidental Trauma ...... 6.27.2

Ocular Manifestations of Systemic Disease ...... 6.28

32 Diseases due to Chromosomal Abnormalities...... 6.28.1 Neurofibromatosis ...... 6.28.1.1 Tuberous Sclerosis ...... 6.28.1.2 Von Hippel–Lindau Disease ...... 6.28.1.3 Sturge-Weber Syndrome ...... 6.28.1.4 Ataxia-Telangiectasia ...... 6.28.1.5 Incontinentia Pigmenti ...... 6.28.1.6 Wyburn-Mason Syndrome ...... 6.28.1.7 Klippel-Trénaunay-Weber Syndrome ...... 6.28.1.8 Intrauterine or Perinatal Infection ...... 6.28.2 Toxoplasmosis ...... 6.28.2.1 Rubella ...... 6.28.2.2 Cytomegalovirus ...... 6.28.2.3 Herpes Simplex Virus ...... 6.28.2.4 Syphilis...... 6.28.2.5 Malignant Disease...... 6.28.3 Leukemia...... 6.28.3.1 Neuroblastoma ...... 6.28.3.2

33 CONTENT OUTLINE

Content Outline Number

SECTION 7: ORBIT, EYELIDS, AND LACRIMAL SYSTEM ...... 7.0

Scored as part of Subtest 7: Orbit, Eyelids, and Lacrimal System

Orbital Anatomy...... 7.1

Dimensions ...... 7.1.1 Topographic Relationships ...... 7.1.2 Roof of the Orbit ...... 7.1.2.1 Lateral Wall of the Orbit ...... 7.1.2.2 Medial Wall of the Orbit ...... 7.1.2.3 Floor of the Orbit...... 7.1.2.4 Apertures ...... 7.1.3 Ethmoidal Foramina ...... 7.1.3.1 Superior Orbital Fissure ...... 7.1.3.2 Inferior Orbital Fissure ...... 7.1.3.3 Zygomaticofacial and Zygomaticotemporal Canals...... 7.1.3.4 Nasolacrimal Canal ...... 7.1.3.5 Optic Canal ...... 7.1.3.6 Soft Tissues ...... 7.1.4 Periorbita ...... 7.1.4.1 Intraorbital Optic Nerve ...... 7.1.4.2 Extraocular Muscles and Orbital Fat ...... 7.1.4.3 Annulus of Zinn ...... 7.1.4.4 Vasculature of the Orbit ...... 7.1.4.5 Nerves...... 7.1.4.6 Lacrimal Gland ...... 7.1.4.7 Periorbital Structures ...... 7.1.5 Nose and Paranasal Sinuses ...... 7.1.5.1

Evaluation of Orbital Disorders ...... 7.2

Physical Examination ...... 7.2.1 Primary Studies...... 7.2.2 Computed Tomography ...... 7.2.2.1 Magnetic Resonance Imaging ...... 7.2.2.2 Comparison of CT and MRI ...... 7.2.2.3 Ultrasonography ...... 7.2.2.4 Secondary Studies ...... 7.2.3 Venography ...... 7.2.3.1 Arteriography ...... 7.2.3.2 CT and MR Angiography ...... 7.2.3.3 Pathology...... 7.2.4 Laboratory Studies ...... 7.2.5

Congenital Orbital Anomalies ...... 7.3

Anophthalmia ...... 7.3.1 Microphthalmia...... 7.3.2 Craniofacial Clefting ...... 7.3.3 Congenital Orbital Tumors...... 7.3.4

34 Hamartomas and Choristomas ...... 7.3.4.1 Teratoma ...... 7.3.4.2

Orbital Inflammatory and Infectious Disorders ...... 7.4

Infectious Inflammation ...... 7.4.1 Cellulitis ...... 7.4.1.1 Necrotizing Fasciitis ...... 7.4.1.2 Orbital Tuberculosis ...... 7.4.1.3 Zygomycosis ...... 7.4.1.4 Aspergillosis ...... 7.4.1.5 Parasitic Diseases ...... 7.4.1.6 Noninfectious Inflammation ...... 7.4.2 Thyroid Eye Disease...... 7.4.2.1 Vasculitis ...... 7.4.2.2 Sarcoidosis...... 7.4.2.3 Nonspecific Orbital Inflammation...... 7.4.2.4

Orbital Neoplasms and Malformations ...... 7.5

Vascular Tumors, Malformations, and Fistulas ...... 7.5.1 Capillary Hemangiomas ...... 7.5.1.1 Cavernous Hemangioma ...... 7.5.1.2 Hemangiopericytoma ...... 7.5.1.3 Lymphatic Malformation ...... 7.5.1.4 Venous Malformation ...... 7.5.1.5 Arteriovenous Malformations ...... 7.5.1.6 Arteriovenous Fistula ...... 7.5.1.7 Orbital Hemorrhage ...... 7.5.1.8 Neural Tumors ...... 7.5.2 Optic Nerve Glioma ...... 7.5.2.1 Neurofibroma...... 7.5.2.2 Neurofibromatosis 1 ...... 7.5.2.3 Meningioma ...... 7.5.2.4 Schwannoma...... 7.5.2.5 Mesenchymal Tumors ...... 7.5.3 Rhabdomyosarcoma...... 7.5.3.1 Miscellaneous Mesenchymal Tumors ...... 7.5.3.2 Lymphoproliferative Disorders ...... 7.5.4 Lymphoid Hyperplasia and Lymphoma ...... 7.5.4.1 Plasma Cell Tumors ...... 7.5.4.2 Histiocytic Disorders ...... 7.5.4.3 Xanthogranuloma ...... 7.5.4.4 Lacrimal Gland Tumors ...... 7.5.5 Epithelial Tumors of the Lacrimal Gland ...... 7.5.5.1 Nonepithelial Tumors of the Lacrimal Gland ...... 7.5.5.2 Secondary Orbital Tumors...... 7.5.6 Globe and Eyelid Origin ...... 7.5.6.1 Sinus Origin ...... 7.5.6.2 Metastatic Tumors...... 7.5.7 Metastatic Tumors in Children ...... 7.5.7.1 Metastatic Tumors in Adults ...... 7.5.7.2 Management of Orbital Metastases...... 7.5.7.3

Orbital Trauma ...... 7.6

35 Midfacial (Le Fort) Fractures ...... 7.6.1 Orbital Fractures ...... 7.6.2 Zygomatic Fractures ...... 7.6.2.1 Orbital Apex Fractures ...... 7.6.2.2 Orbital Roof Fractures ...... 7.6.2.3 Medial Orbital Fractures...... 7.6.2.4 Orbital Floor Fractures ...... 7.6.2.5 Intraorbital Foreign Bodies ...... 7.6.3 Orbital Hemorrhage...... 7.6.4 Traumatic Visual Loss With Clear Media ...... 7.6.5 Management ...... 7.6.5.1

Orbital Surgery ...... 7.7

Surgical Spaces ...... 7.7.1 Orbitotomy ...... 7.7.2 Superior Approach...... 7.7.2.1 Inferior Approach ...... 7.7.2.2 Medial Approach...... 7.7.2.3 Lateral Approach ...... 7.7.2.4 Orbital Decompression ...... 7.7.3 Postoperative Care for Orbital Surgery ...... 7.7.4 Special Surgical Techniques in the Orbit ...... 7.7.5 Complications of Orbital Surgery ...... 7.7.6

The Anophthalmic Socket ...... 7.8

Enucleation ...... 7.8.1 Guidelines for Enucleation ...... 7.8.1.1 Enucleation in Childhood ...... 7.8.1.2 Intraoperative Complications of Enucleation...... 7.8.1.3 Evisceration ...... 7.8.2 Advantages of Evisceration ...... 7.8.2.1 Disadvantages of Evisceration ...... 7.8.2.2 Techniques of Evisceration ...... 7.8.2.3 Orbital Implants ...... 7.8.3 Prostheses ...... 7.8.3.1 Anophthalmic Socket Complications and Treatment ...... 7.8.4 Deep Superior Sulcus ...... 7.8.4.1 Contracture of Fornices ...... 7.8.4.2 Exposure and Extrusion of Implant ...... 7.8.4.3 Contracted Sockets ...... 7.8.4.4 Anophthalmic Ectropion ...... 7.8.4.5 Anophthalmic Ptosis ...... 7.8.4.6 Lash Margin Entropion ...... 7.8.4.7 Cosmetic Optics ...... 7.8.4.8 Exenteration...... 7.8.5 Considerations for Exenteration ...... 7.8.5.1 Types of Exenteration ...... 7.8.5.2

Facial and Eyelid Anatomy...... 7.9

Face ...... 7.9.1 Eyelids ...... 7.9.2 Skin and Subcutaneous Tissue ...... 7.9.2.1 Protractors...... 7.9.2.2

36 Orbital Septum ...... 7.9.2.3 Orbital Fat ...... 7.9.2.4 Retractors...... 7.9.2.5 Tarsus ...... 7.9.2.6 Conjunctiva ...... 7.9.2.7 Additional Anatomical Considerations ...... 7.9.2.8

Classification and Management of Eyelid Disorders ...... 7.10

Congenital Anomalies ...... 7.10.1 Blepharophimosis Syndrome ...... 7.10.1.1 Congenital Ptosis of the Upper Eyelid ...... 7.10.1.2 Congenital Ectropion ...... 7.10.1.3 Euryblepharon ...... 7.10.1.4 Ankyloblepharon ...... 7.10.1.5 Epicanthus ...... 7.10.1.6 Epiblepharon ...... 7.10.1.7 Congenital Entropion ...... 7.10.1.8 Congenital Distichiasis ...... 7.10.1.9 Congenital Coloboma ...... 7.10.1.10 Congenital Eyelid Lesions...... 7.10.1.11 Acquired Eyelid Disorders ...... 7.10.2 Chalazion ...... 7.10.2.1 Hordeolum...... 7.10.2.2 Eyelid Edema ...... 7.10.2.3 Floppy Eyelid Syndrome ...... 7.10.2.4 Trichotillomania ...... 7.10.2.5 Eyelid Neoplasms ...... 7.10.3 Clinical Evaluation of Eyelid Tumors ...... 7.10.3.1 Benign Eyelid Lesions ...... 7.10.3.2 Benign Adnexal Lesions ...... 7.10.3.3 Benign Melanocytic Lesions...... 7.10.3.4 Premalignant Epidermal Lesions...... 7.10.3.5 In Situ Epithelial Malignancies ...... 7.10.3.6 Premalignant Melanocytic Lesions ...... 7.10.3.7 Malignant Eyelid Tumors ...... 7.10.3.8 Eyelid Trauma ...... 7.10.4 Blunt Trauma ...... 7.10.4.1 Penetrating Trauma ...... 7.10.4.2 Secondary Repair ...... 7.10.4.3 Dog and Human Bites...... 7.10.4.4 Burns ...... 7.10.4.5 Eyelid and Canthal Reconstruction ...... 7.10.5 Eyelid Defects Not Involving the Eyelid Margin...... 7.10.5.1 Eyelid Defects Involving the Eyelid Margin ...... 7.10.5.2 Lateral Canthal Defects ...... 7.10.5.3 Medial Canthal Defects ...... 7.10.5.4

Periocular Malpositions and Involutional Changes ...... 7.11

History and Examination ...... 7.11.1 Preoperative Considerations ...... 7.11.1.1 Ectropion ...... 7.11.2 Involutional Ectropion ...... 7.11.2.1 Paralytic Ectropion...... 7.11.2.2 Cicatricial Ectropion ...... 7.11.2.3

37 Mechanical Ectropion ...... 7.11.2.4 Entropion ...... 7.11.3 Congenital Entropion ...... 7.11.3.1 Acute Spastic Entropion...... 7.11.3.2 Involutional Entropion ...... 7.11.3.3 Cicatricial Entropion ...... 7.11.3.4 Symblepharon ...... 7.11.4 Trichiasis ...... 7.11.5 Management ...... 7.11.5.1 Blepharoptosis ...... 7.11.6 Evaluation ...... 7.11.6.1 Physical Examination...... 7.11.6.2 Ancillary Tests ...... 7.11.6.3 Classification ...... 7.11.6.4 Treatment of Ptosis ...... 7.11.6.5 Eyelid Retraction ...... 7.11.7 Treatment of Eyelid Retraction ...... 7.11.7.1 Facial Paralysis ...... 7.11.8 Paralytic Ectropion...... 7.11.8.1 Paralytic Lagophthalmos...... 7.11.8.2 Facial Dystonia ...... 7.11.9 Benign Essential Blepharospasm...... 7.11.9.1 Hemifacial Spasm...... 7.11.9.2 Involutional Periorbital Changes ...... 7.11.10 Dermatochalasis ...... 7.11.10.1 Blepharochalasis ...... 7.11.10.2 Blepharoplasty ...... 7.11.11 Upper Eyelid ...... 7.11.11.1 Lower Eyelid ...... 7.11.11.2 Preoperative Evaluation for Blepharoplasty ...... 7.11.11.3 Technique ...... 7.11.11.4 Complications ...... 7.11.11.5 Brow Ptosis ...... 7.11.12 Treatment of Functional Brow Ptosis...... 7.11.12.1 Cosmetic Facial Surgery ...... 7.11.13 Pathogenesis of the Aging Face ...... 7.11.13.1 Physical Examination of the Aging Face ...... 7.11.13.2 Facial Rejuvenation Surgery ...... 7.11.14 Laser Skin Resurfacing ...... 7.11.14.1 Cosmetic Uses of Botulinum Toxin...... 7.11.14.2 Forehead Rejuvenation ...... 7.11.14.3 Midface Rejuvenation ...... 7.11.14.4 Lower Face and Neck Rejuvenation...... 7.11.14.5

Development, Anatomy, and Physiology of the Lacrimal Secretory and Drainage Systems...7.12

Development...... 7.12.1 Secretory Apparatus ...... 7.12.1.1 Excretory Apparatus ...... 7.12.1.2 Normal Anatomy ...... 7.12.2 Secretory Apparatus ...... 7.12.2.1 Excretory Apparatus ...... 7.12.2.2 Physiology ...... 7.12.3

Abnormalities of the Lacrimal Secretory and Drainage Systems...... 7.13

38 Congenital Lacrimal Drainage Obstruction...... 7.13.1 Evaluation ...... 7.13.1.1 Punctal and Canalicular Agenesis and Dysgenesis ...... 7.13.1.2 Congenital Nasolacrimal Duct Obstruction ...... 7.13.1.3 Dacryocystocele ...... 7.13.1.4 Acquired Lacrimal Drainage Obstruction...... 7.13.2 Evaluation ...... 7.13.2.1 Punctal Disorders ...... 7.13.2.2 Canalicular Obstruction ...... 7.13.2.3 Acquired Nasolacrimal Duct Obstruction ...... 7.13.2.4 Therapeutic Closure of the Lacrimal Drainage System ...... 7.13.3 Trauma ...... 7.13.4 Canaliculus...... 7.13.4.1 Lacrimal Sac and Nasolacrimal Duct...... 7.13.4.2 Infection ...... 7.13.5 Lacrimal Gland (Dacryoadenitis) ...... 7.13.5.1 Canaliculus (Canaliculitis) ...... 7.13.5.2 Lacrimal Sac (Dacryocystitis) ...... 7.13.5.3 Neoplasm ...... 7.13.6 Lacrimal Gland ...... 7.13.6.1 Lacrimal Drainage System ...... 7.13.6.2 Developmental Abnormalities ...... 7.13.7 Lacrimal Secretory System ...... 7.13.7.1 Lacrimal Drainage System ...... 7.13.7.2

39 CONTENT OUTLINE Content Outline Number

SECTION 8: EXTERNAL DISEASE AND CORNEA ...... 8.0

Scored as part of Subtest 8: External Disease and Cornea

Structure and Function of the External Eye and Cornea ...... 8.1

Anatomy ...... 8.1.1 Eyelids ...... 8.1.1.1 Conjunctiva ...... 8.1.1.2 Lacrimal Functional Unit ...... 8.1.1.3 The Tear Film ...... 8.1.1.4 Cornea ...... 8.1.1.5 Sclera ...... 8.1.1.6

Examination Techniques for the External Eye and Cornea ...... 8.2

Evaluation of Vision in the Patient With an Abnormal Cornea ...... 8.2.1 External Examination ...... 8.2.2 Slit-Lamp Biomicroscopy...... 8.2.3 Direct Illumination Methods ...... 8.2.3.1 Indirect Illumination Methods ...... 8.2.3.2 Clinical Use ...... 8.2.3.3 Stains ...... 8.2.4 Fluorescein ...... 8.2.4.1 Rose Bengal and Lissamine Green ...... 8.2.4.2 Evaluation of the Tear Film and Tests of Tear Production ...... 8.2.5 Tear Composition Assays ...... 8.2.5.1 Imaging Technologies ...... 8.2.5.2 Impression Cytology...... 8.2.5.3 Corneal Pachymetry ...... 8.2.6 Measurement of Corneal Biomechanics ...... 8.2.7 Measurement of Corneal Curvature ...... 8.2.8 Zones of the Cornea ...... 8.2.8.1 Shape, Curvature, and Power ...... 8.2.8.2 Keratometry ...... 8.2.8.3 Computerized Corneal Topography ...... 8.2.8.4 Corneal Tomography ...... 8.2.8.5 Indications ...... 8.2.8.6 Additional Imaging Techniques of the Cornea and Anterior Segment ...... 8.2.9 Ultrasound Biomicroscopy ...... 8.2.9.1 Anterior Segment Optical Coherence Tomography ...... 8.2.9.2 Confocal Microscopy ...... 8.2.9.3 External and Slit-Lamp Photography ...... 8.2.9.4 Specular Microscopy ...... 8.2.9.5 Anterior Segment Fluorescein Angiography ...... 8.2.9.6 Esthesiometry ...... 8.2.10 Retinoscopy ...... 8.2.11

Clinical Approach to Ocular Surface Disorders ...... 8.3

40 Common Clinical Findings ...... 8.3.1 Conjunctival Signs...... 8.3.1.1 Corneal Signs ...... 8.3.1.2 Clinical Approach to Dry Eye ...... 8.3.2 Mechanisms of Dry Eye ...... 8.3.2.1 Aqueous Tear Deficiency ...... 8.3.2.2 Evaporative Dry Eye ...... 8.3.2.3 Treatment of Dry Eye ...... 8.3.2.4 Other Eyelid Diseases Associated With Ocular Surface Disorders ...... 8.3.3 Rosacea ...... 8.3.3.1 Seborrheic Blepharitis ...... 8.3.3.2 Staphylococcal Blepharitis ...... 8.3.3.3 Hordeola and Chalazia ...... 8.3.3.4 Structural and Exogenous Disorders Associated With Ocular Surface Disorders...... 8.3.4 Exposure Keratopathy ...... 8.3.4.1 Floppy Eyelid Syndrome ...... 8.3.4.2 Superior Limbic Keratoconjunctivitis ...... 8.3.4.3 Recurrent Corneal Erosion ...... 8.3.4.4 Neurotrophic Keratopathy and Persistent Corneal Epithelial Defects ...... 8.3.4.5 Trichiasis and Distichiasis ...... 8.3.4.6 Factitious Ocular Surface Disorders ...... 8.3.4.7 Dellen ...... 8.3.4.8 Limbal Stem Cell Deficiency ...... 8.3.5 Systemic Conditions Associated With Ocular Surface Disorders ...... 8.3.6 Sjögren Syndrome ...... 8.3.6.1 Ichthyosis ...... 8.3.6.2 Ectodermal Dysplasia ...... 8.3.6.3 Xeroderma Pigmentosum ...... 8.3.6.4 Vitamin A Deficiency ...... 8.3.6.5

Infectious Diseases of the External Eye: Basic Concepts and Viral Infections ...... 8.4

Defense Mechanisms of the External Eye ...... 8.4.1 Normal Ocular Flora ...... 8.4.2 Pathogenesis of Ocular Infections ...... 8.4.3 Virulence ...... 8.4.3.1 Inoculum ...... 8.4.3.2 Host Defense ...... 8.4.3.3 Ocular Microbiology ...... 8.4.4 Diagnostic Laboratory Techniques ...... 8.4.5 Specimen Collection ...... 8.4.5.1 Staining Methods ...... 8.4.5.2 Virology and Viral Infections ...... 8.4.6 DNA Viruses: Herpesviruses ...... 8.4.7 Herpes Simplex Eye Diseases ...... 8.4.7.1 Varicella-Zoster Virus Dermatoblepharitis, Conjunctivitis, and Keratitis ...... 8.4.7.2 Epstein-Barr Virus Dacryoadenitis, Conjunctivitis, and Keratitis ...... 8.4.7.3 Cytomegalovirus Keratitis and Anterior Uveitis ...... 8.4.7.4 DNA Viruses: Adenoviruses ...... 8.4.8 DNA Viruses: Poxviruses ...... 8.4.9 Molluscum Contagiosum ...... 8.4.9.1 Vaccinia ...... 8.4.9.2 DNA Viruses: Papovaviruses ...... 8.4.10 RNA Viruses ...... 8.4.11

Infectious Diseases of the External Eye: Microbial and Parasitic Infections ...... 8.5

41 Bacteriology ...... 8.5.1 Gram-positive Cocci ...... 8.5.1.1 Gram-negative Cocci ...... 8.5.1.2 Gram-positive Rods ...... 8.5.1.3 Gram-negative Rods ...... 8.5.1.4 Gram-positive Filaments ...... 8.5.1.5 Chlamydia Species ...... 8.5.1.6 Spirochetes ...... 8.5.1.7 Mycology ...... 8.5.2 Yeasts ...... 8.5.2.1 Septate Filamentous Fungi ...... 8.5.2.2 Nonseptate Filamentous Fungi ...... 8.5.2.3 Parasitology ...... 8.5.3 Protozoa ...... 8.5.3.1 Helminths ...... 8.5.3.2 Arthropods ...... 8.5.3.3 Prions ...... 8.5.4 Microbial and Parasitic Infections of the Eyelid Margin and Conjunctiva ...... 8.5.5 Staphylococcal Blepharitis ...... 8.5.5.1 Fungal and Parasitic Infections of the Eyelid Margin ...... 8.5.5.2 Bacterial Conjunctivitis in Children and Adults ...... 8.5.5.3 Parinaud Oculoglandular Syndrome ...... 8.5.5.4 Microbial and Parasitic Infections of the Cornea and Sclera ...... 8.5.6 Contact Lens–Related Infectious Keratitis ...... 8.5.6.1 Bacterial Keratitis ...... 8.5.6.2 Atypical Mycobacteria ...... 8.5.6.3 Fungal Keratitis ...... 8.5.6.4 Acanthamoeba Keratitis ...... 8.5.6.5 Corneal Stromal Inflammation Associated With Systemic Infections ...... 8.5.6.6 Microsporidiosis ...... 8.5.6.7 Loiasis ...... 8.5.6.8 Microbial Scleritis ...... 8.5.6.9

Ocular Immunology ...... 8.6

Overview of the Ocular Surface Immune Response ...... 8.6.1 Tear Film ...... 8.6.1.1 Immunoregulation of the Ocular Surface ...... 8.6.1.2 Angiogenesis and Lymphangiogenesis in the Cornea ...... 8.6.1.3 Tissue-Specific Patterns of Immune-Mediated Ocular Disease ...... 8.6.2 Conjunctiva ...... 8.6.2.1 Cornea ...... 8.6.2.2 Sclera ...... 8.6.2.3 Diagnostic Approach to Immune-Mediated Ocular Disorders ...... 8.6.3

Diagnosis and Management of Immune-Related Disorders of the External Eye ...... 8.7

Immune-Mediated Diseases of the Eyelid ...... 8.7.1 Contact Dermatoblepharitis ...... 8.7.1.1 Atopic Dermatitis ...... 8.7.1.2 Immune-Mediated Disorders of the Conjunctiva ...... 8.7.2 Hay Fever Conjunctivitis and Perennial Allergic Conjunctivitis ...... 8.7.2.1 Vernal Keratoconjunctivitis ...... 8.7.2.2 Atopic Keratoconjunctivitis ...... 8.7.2.3 Ligneous Conjunctivitis ...... 8.7.2.4

42 Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis ...... 8.7.2.5 Mucous Membrane Pemphigoid ...... 8.7.2.6 Ocular Graft-vs-Host Disease ...... 8.7.2.7 Other Immune-Mediated Diseases of the Skin and Mucous Membranes ...... 8.7.2.8 Immune-Mediated Diseases of the Cornea ...... 8.7.3 Thygeson Superficial Punctate Keratitis ...... 8.7.3.1 Interstitial Keratitis Associated With Infectious Diseases ...... 8.7.3.2 Reactive Arthritis ...... 8.7.3.3 Cogan Syndrome ...... 8.7.3.4 Marginal Corneal Infiltrates Associated With Blepharoconjunctivitis ...... 8.7.3.5 Peripheral Ulcerative Keratitis Associated With Systemic Immune-Mediated Diseases 8.7.3.6 Mooren Ulcer ...... 8.7.3.7 Corneal Transplant Rejection ...... 8.7.4 Immune-Mediated Diseases of the Episclera and Sclera ...... 8.7.5 Episcleritis ...... 8.7.5.1 Scleritis ...... 8.7.5.2

Clinical Approach to Neoplastic Disorders of the Conjunctiva and Cornea ...... 8.8

Approach to the Patient With a Neoplastic Conjunctival Lesion ...... 8.8.1 Management of Patients With Conjunctival Tumors ...... 8.8.2 Surgical Treatment ...... 8.8.2.1 Topical Chemotherapy ...... 8.8.2.2 Tumors of Epithelial Origin ...... 8.8.3 Benign Epithelial Tumors ...... 8.8.3.1 Preinvasive Epithelial Lesions ...... 8.8.3.2 Malignant Epithelial Lesions ...... 8.8.3.3 Management of Atypical Epithelial Tumors ...... 8.8.3.4 Other Malignant Epithelial Lesions ...... 8.8.3.5 Glandular Tumors of the Conjunctiva ...... 8.8.4 Oncocytoma ...... 8.8.4.1 Sebaceous Gland Carcinoma ...... 8.8.4.2 Tumors of Neuroectodermal Origin ...... 8.8.5 Benign Pigmented Lesions ...... 8.8.5.1 Preinvasive Pigmented Lesions ...... 8.8.5.2 Malignant Pigmented Lesions ...... 8.8.5.3 Neurogenic and Smooth-Muscle Tumors ...... 8.8.5.4 Vascular and Mesenchymal Tumors ...... 8.8.6 Benign Tumors ...... 8.8.6.1 Malignant Tumors ...... 8.8.6.2 Lymphatic and Lymphocytic Tumors ...... 8.8.7 Lymphangiectasia and Lymphangioma ...... 8.8.7.1 Lymphoid Hyperplasia ...... 8.8.7.2 Lymphoma ...... 8.8.7.3 Metastatic Tumors ...... 8.8.8

Basic and Clinical Concepts of Congenital Anomalies of the Cornea, Sclera, and Globe ...... 8.9

Developmental Anomalies of the Globe and Sclera ...... 8.9.1 Cryptophthalmos ...... 8.9.1.1 Microphthalmos ...... 8.9.1.2 Nanophthalmos ...... 8.9.1.3 Blue Sclera ...... 8.9.1.4 Developmental Anomalies of the Anterior Segment ...... 8.9.2 Anomalies of Size and Shape of the Cornea ...... 8.9.2.1 Abnormalities of Corneal Structure and/or Clarity ...... 8.9.2.2

43 Congenital Corneal Opacities in Hereditary Syndromes and Chromosomal Aberrations 8.9.2.3 Secondary Abnormalities Affecting the Fetal Cornea ...... 8.9.3 Intrauterine Keratitis: Bacterial and Syphilitic ...... 8.9.3.1 Congenital Corneal Keloid ...... 8.9.3.2 Congenital Corneal Anesthesia ...... 8.9.3.3 Congenital Glaucoma...... 8.9.3.4 Birth Trauma ...... 8.9.3.5 Arcus Juvenilis ...... 8.9.3.6

Corneal Dystrophies and Ectasias ...... 8.10

Corneal Dystrophies ...... 8.10.1 Epithelial and Subepithelial Dystrophies ...... 8.10.1.1 Bowman Layer Corneal Dystrophies ...... 8.10.1.2 Stromal Corneal Dystrophies: TGFBI Dystrophies ...... 8.10.1.3 Stromal Dystrophies: Non-TGFBI Dystrophies ...... 8.10.1.4 Endothelial Dystrophies ...... 8.10.1.5 Ectatic Disorders ...... 8.10.2 Keratoconus ...... 8.10.2.1 Pellucid Marginal Degeneration ...... 8.10.2.2 Keratoglobus ...... 8.10.2.3

Systemic Disorders With Corneal Change ...... 8.11

Disorders of Carbohydrate Metabolism ...... 8.11.1 Mucopolysaccharidoses ...... 8.11.1.1 Diabetes Mellitus ...... 8.11.1.2 Disorders of Lipid Metabolism and Storage ...... 8.11.2 Disorders of Amino Acid Metabolism ...... 8.11.3 Cystinosis ...... 8.11.3.1 Tyrosinemia ...... 8.11.3.2 Alkaptonuria ...... 8.11.3.3 Disorders of Protein Metabolism ...... 8.11.4 Amyloidosis ...... 8.11.4.1 Disorders of Immunoglobulin Synthesis ...... 8.11.5 Noninflammatory Disorders of Connective Tissue ...... 8.11.6 Ehlers-Danlos Syndrome ...... 8.11.6.1 Marfan Syndrome ...... 8.11.6.2 Disorders of Nucleotide Metabolism ...... 8.11.7 Gout ...... 8.11.7.1 Disorders of Mineral Metabolism ...... 8.11.8 Wilson Disease ...... 8.11.8.1 Hypercalcemia ...... 8.11.8.2 Corneal and External Disease Signs of Systemic Neoplasia ...... 8.11.9 Enlarged Corneal Nerves ...... 8.11.9.1

Clinical Approach to Depositions and Degenerations of the Conjunctiva, Cornea, and Sclera 8.12

Degenerative Changes of the Conjunctiva ...... 8.12.1 Age-Related (Involutional) Changes ...... 8.12.1.1 Pinguecula ...... 8.12.1.2 Pterygium ...... 8.12.1.3 Conjunctival Concretions ...... 8.12.1.4 Conjunctival Inclusion Cysts ...... 8.12.1.5 Conjunctivochalasis ...... 8.12.1.6 Conjunctival Vascular Tortuosity and Hyperemia ...... 8.12.1.7

44 Degenerative Changes in the Cornea ...... 8.12.2 Age-Related (Involutional) Changes ...... 8.12.2.1 Epithelial and Subepithelial Degenerations ...... 8.12.2.2 Stromal Degenerations ...... 8.12.2.3 Endothelial Degenerations ...... 8.12.2.4 Scleral Degenerations ...... 8.12.3 Drug-Induced Deposition and Pigmentation ...... 8.12.4 Corneal Epithelial Deposits ...... 8.12.4.1 Stromal and Descemet Membrane Pigmentation ...... 8.12.4.2 Endothelial Manifestations ...... 8.12.4.3

Clinical Aspects of Toxic and Traumatic Injuries of the Anterior Segment ...... 8.13

Injuries Caused by Temperature and Radiation ...... 8.13.1 Thermal Burns ...... 8.13.1.1 Ultraviolet Radiation ...... 8.13.1.2 Ionizing Radiation...... 8.13.1.3 Chemical Injuries ...... 8.13.2 Alkali Burns ...... 8.13.2.1 Acid Burns ...... 8.13.2.2 Management of Chemical Injuries ...... 8.13.2.3 Toxic Keratoconjunctivitis From Medications ...... 8.13.3 Animal and Plant Substances ...... 8.13.4 Insect Injuries ...... 8.13.4.1 Vegetation Injuries ...... 8.13.4.2 Concussive Trauma ...... 8.13.5 Subconjunctival Hemorrhage ...... 8.13.5.1 Corneal Changes ...... 8.13.5.2 Traumatic Mydriasis and Miosis ...... 8.13.5.3 Traumatic Iritis ...... 8.13.5.4 Iridodialysis and Cyclodialysis ...... 8.13.5.5 Traumatic Hyphema ...... 8.13.5.6 Nonperforating Mechanical Trauma ...... 8.13.6 Conjunctival Laceration ...... 8.13.6.1 Conjunctival Foreign Body ...... 8.13.6.2 Corneal Foreign Body ...... 8.13.6.3 Corneal Abrasion ...... 8.13.6.4 Perforating Trauma ...... 8.13.7 Evaluation ...... 8.13.7.1 Management ...... 8.13.7.2 Surgical Trauma ...... 8.13.8 Corneal Epithelial Changes From Intraocular Surgery ...... 8.13.8.1 Descemet Membrane Changes During Intraocular Surgery ...... 8.13.8.2 Corneal Endothelial Changes From Intraocular Surgery ...... 8.13.8.3 Conjunctival and Corneal Changes From Extraocular Surgery ...... 8.13.8.4

Treatment of Ocular Surface Disorders ...... 8.14

Surgical Procedures of the Ocular Surface...... 8.14.1 Limbal Transplantation ...... 8.14.1.1 Autologous Conjunctival Transplantation ...... 8.14.1.2 Pterygium Excision...... 8.14.1.3 Mucous Membrane Grafting ...... 8.14.1.4 Conjunctival Flap ...... 8.14.1.5 Conjunctival Biopsy ...... 8.14.1.6 Conjunctivochalasis Excision ...... 8.14.1.7

45 Therapeutic Interventions for Corneal Disease ...... 8.14.2 Superficial Keratectomy and Corneal Biopsy ...... 8.14.2.1 Management of Descemetocele, Corneal Perforation, and Corneal Edema...... 8.14.2.2 Corneal Tattoo ...... 8.14.2.3 Tarsorrhaphy ...... 8.14.2.4

Clinical Approach to Corneal Transplantation...... 8.15

Corneal Transplantation ...... 8.15.1 Eye Banking and Donor Selection ...... 8.15.2 Criteria Contraindicating Donor Cornea Use ...... 8.15.2.1 Surgical Approach to Corneal Disease ...... 8.15.3 Preoperative Evaluation and Preparation ...... 8.15.3.1 Penetrating Keratoplasty ...... 8.15.4 Surgical Technique for Penetrating Keratoplasty ...... 8.15.4.1 Combined Procedures ...... 8.15.4.2 Intraoperative Complications ...... 8.15.4.3 Postoperative Care and Complications...... 8.15.4.4 Control of Postoperative Corneal Astigmatism and Refractive Error ...... 8.15.4.5 Diagnosis and Management of Graft Rejection ...... 8.15.4.6 Pediatric Corneal Transplantation ...... 8.15.5 Corneal Autograft Procedures ...... 8.15.6 Keratoprosthesis ...... 8.15.7 Lamellar Keratoplasty ...... 8.15.8 Anterior Lamellar Transplantation ...... 8.15.8.1 Surgical Technique ...... 8.15.8.2 Postoperative Care and Complications...... 8.15.8.3 Endothelial Keratoplasty ...... 8.15.9 DSEK Surgical Technique and Complications ...... 8.15.9.1 Descemet Membrane Endothelial Keratoplasty ...... 8.15.9.2

46 CONTENT OUTLINE Content Outline Number

SECTION 9: INTRAOCULAR INFLAMMATION AND UVEITIS ...... 9.0

Scored as part of Subtest 9: Intraocular Inflammation and Uveitis

Basic Concepts in Immunology: Effector Cells and the Innate Immune Response ...... 9.1

Components of the Immune System ...... 9.1.1 Leukocytes ...... 9.1.1.1 Overview of the Innate Immune System ...... 9.1.2 Immunity Versus Inflammation ...... 9.1.2.1 Triggers of Innate Immunity ...... 9.1.3 Bacteria-Derived Molecules That Trigger Innate Immunity...... 9.1.3.1 Other Triggers or Modulators of Innate Immunity ...... 9.1.3.2 Innate Mechanisms for the Recruitment and Activation of Neutrophils ...... 9.1.3.3 Innate Mechanisms for the Recruitment and Activation of Macrophages ...... 9.1.3.4 Mediator Systems That Amplify Immune Responses ...... 9.1.4 Plasma-Derived Enzyme Systems ...... 9.1.4.1 Lipid Mediators ...... 9.1.4.2 Cytokines ...... 9.1.4.3 Reactive Oxygen Intermediates ...... 9.1.4.4 Reactive Nitrogen Products ...... 9.1.4.5 Neutrophil-Derived Granule Products ...... 9.1.4.6

Immunization and Adaptive Immunity: The Immune Response Arc and Immune Effectors ....9.2

Phases of the Immune Response Arc...... 9.2.1 Afferent Phase...... 9.2.1.1 Processing Phase...... 9.2.1.2 Effector Phase ...... 9.2.1.3 Concept of Immunologic Memory ...... 9.2.2 Antibody-Mediated Immune Effector Responses ...... 9.2.3 Lymphocyte-Mediated Effector Responses ...... 9.2.4 Combined Antibody and Cellular Effector Mechanisms ...... 9.2.5

Ocular Immune Responses ...... 9.3

Immune Responses of the Conjunctiva ...... 9.3.1 Features of the Immunologic Microenvironment ...... 9.3.1.1 Immunoregulatory Systems ...... 9.3.1.2 Immune Responses of the Anterior Chamber, Anterior Uvea, and Vitreous...... 9.3.2 Features of the Immunologic Microenvironment ...... 9.3.2.1 Immunoregulatory Systems ...... 9.3.2.2 Immune Responses of the Cornea ...... 9.3.3 Features of the Immunologic Microenvironment ...... 9.3.3.1 Immunoregulatory Systems ...... 9.3.3.2 Immune Responses of the Retina, RPE, Choriocapillaris, and Choroid ...... 9.3.4 Features of the Immunologic Microenvironment ...... 9.3.4.1 Immunoregulatory Systems ...... 9.3.4.2

Special Topics in Ocular Immunology ...... 9.4

47 HLA Associations and Disease...... 9.4.1 Normal Function of HLA Molecules ...... 9.4.1.1 Allelic Variation ...... 9.4.1.2 Disease Associations...... 9.4.1.3

Clinical Approach to Uveitis ...... 9.5

Classification of Uveitis ...... 9.5.1 Anterior Uveitis ...... 9.5.1.1 Intermediate Uveitis ...... 9.5.1.2 Posterior Uveitis ...... 9.5.1.3 Panuveitis...... 9.5.1.4 Symptoms of Uveitis ...... 9.5.2 Signs of Uveitis ...... 9.5.3 Anterior Segment...... 9.5.3.1 Intermediate Segment ...... 9.5.3.2 Posterior Segment ...... 9.5.3.3 Review of the Patient’s Health and Other Associated Factors ...... 9.5.4 Differential Diagnosis of Uveitic Entities ...... 9.5.5 Laboratory and Medical Evaluation...... 9.5.6 Therapy ...... 9.5.7 Medical Management of Uveitis ...... 9.5.8 Mydriatic and Cycloplegic Agents ...... 9.5.8.1 Nonsteroidal Anti-Inflammatory Drugs ...... 9.5.8.2 Corticosteroids ...... 9.5.8.3 Immunomodulatory Medications ...... 9.5.8.4 Surgical Management of Uveitis ...... 9.5.9

Noninfectious (Autoimmune) Ocular Inflammatory Disease ...... 9.6

Noninfectious Scleritis ...... 9.6.1 Treatment of Noninfectious Scleritis ...... 9.6.1.1 Anterior Uveitis...... 9.6.2 Acute Nongranulomatous Iritis and Iridocyclitis ...... 9.6.2.1 Chronic Anterior Uveitis (Iridocyclitis) ...... 9.6.2.2 Intermediate Uveitis ...... 9.6.3 Pars Planitis ...... 9.6.3.1 Multiple Sclerosis...... 9.6.3.2 Posterior Uveitis ...... 9.6.4 Collagen Vascular Diseases ...... 9.6.4.1 Inflammatory Chorioretinopathies of Unknown Etiology ...... 9.6.4.2 Panuveitis ...... 9.6.5 Sarcoidosis...... 9.6.5.1 Sympathetic Ophthalmia ...... 9.6.5.2 Vogt-Koyanagi-Harada Syndrome ...... 9.6.5.3 Behçet Disease ...... 9.6.5.4

Infectious Ocular Inflammatory Disease ...... 9.7

Viral Uveitis ...... 9.7.1 Herpesviridae Family ...... 9.7.1.1 Rubella ...... 9.7.1.2 Measles (Rubeola) ...... 9.7.1.3 West Nile Virus ...... 9.7.1.4 Rift Valley Fever ...... 9.7.1.5 Fungal Uveitis ...... 9.7.2

48 Ocular Histoplasmosis Syndrome ...... 9.7.2.1 Protozoal Uveitis ...... 9.7.3 Toxoplasmosis ...... 9.7.3.1 Helminthic Uveitis ...... 9.7.4 Toxocariasis ...... 9.7.4.1 Cysticercosis ...... 9.7.4.2 Diffuse Unilateral Subacute Neuroretinitis ...... 9.7.4.3 Onchocerciasis ...... 9.7.4.4 Bacterial Uveitis ...... 9.7.5 Syphilis...... 9.7.5.1 Lyme Disease...... 9.7.5.2 Leptospirosis ...... 9.7.5.3 Ocular Nocardiosis ...... 9.7.5.4 Tuberculosis ...... 9.7.5.5 Ocular Bartonellosis ...... 9.7.5.6 Infectious Scleritis ...... 9.7.6 Etiology ...... 9.7.6.1 Clinical Features ...... 9.7.6.2 Diagnostic Workup ...... 9.7.6.3 Treatment ...... 9.7.6.4 Prognosis ...... 9.7.6.5

Endophthalmitis ...... 9.8

Chronic Postoperative Endophthalmitis ...... 9.8.1 Endogenous Endophthalmitis ...... 9.8.2 Endogenous Bacterial Endophthalmitis ...... 9.8.2.1 Endogenous Fungal Endophthalmitis ...... 9.8.2.2

Masquerade Syndromes ...... 9.9

Neoplastic Masquerade Syndromes ...... 9.9.1 Primary Central Nervous System Lymphoma ...... 9.9.1.1 Neoplastic Masquerade Syndromes Secondary to Systemic Lymphoma ...... 9.9.1.2 Neoplastic Masquerade Syndromes Secondary to Leukemia ...... 9.9.1.3 Neoplastic Masquerade Syndromes Secondary to Uveal Lymphoid Proliferations .9.9.1.4 Nonlymphoid Malignancies ...... 9.9.1.5 Metastatic Tumors ...... 9.9.1.6 Bilateral Diffuse Uveal Melanocytic Proliferation ...... 9.9.1.7 Nonneoplastic Masquerade Syndromes ...... 9.9.2 Retinitis Pigmentosa ...... 9.9.2.1 Ocular Ischemic Syndrome ...... 9.9.2.2 Chronic Peripheral Rhegmatogenous Retinal Detachment ...... 9.9.2.3 Intraocular Foreign Bodies ...... 9.9.2.4 Pigment Dispersion Syndrome ...... 9.9.2.5

Complications of Uveitis ...... 9.10

Calcific Band-Shaped Keratopathy ...... 9.10.1 Cataracts ...... 9.10.2 Management ...... 9.10.2.1 Complications ...... 9.10.2.2 Glaucoma ...... 9.10.3 Uveitic Ocular Hypertension...... 9.10.3.1 Uveitic Glaucoma ...... 9.10.3.2 Corticosteroid-Induced Ocular Hypertension and Glaucoma ...... 9.10.3.3

49 Management ...... 9.10.3.4 Hypotony ...... 9.10.4 Cystoid Macular Edema ...... 9.10.5 Vitreous Opacification and Vitritis ...... 9.10.6 Rhegmatogenous Retinal Detachment ...... 9.10.7 Retinal and Choroidal Neovascularization ...... 9.10.8

Ocular Involvement in AIDS ...... 9.11

Ophthalmic Manifestations ...... 9.11.1 Cytomegalovirus Retinitis ...... 9.11.1.1 Necrotizing Herpetic Retinitis ...... 9.11.1.2 Toxoplasma Retinochoroiditis ...... 9.11.1.3 Syphilitic Chorioretinitis ...... 9.11.1.4 External Eye Manifestations ...... 9.11.2 Herpes Zoster...... 9.11.2.1

50 CONTENT OUTLINE Content Outline Number

SECTION 10: GLAUCOMA ...... 10.0

Scored as part of Subtest 10: Glaucoma

Introduction to Glaucoma: Terminology, Epidemiology, and Heredity ...... 10.1

Definitions ...... 10.1.1 Open-Angle, Angle-Closure, Primary, and Secondary Glaucomas ...... 10.1.1.1 Epidemiologic Aspects of Glaucoma ...... 10.1.2 Primary Open-Angle Glaucoma ...... 10.1.2.1 Primary Angle-Closure Glaucoma ...... 10.1.2.2 Genetics, Environmental Factors, and Glaucoma ...... 10.1.3

Intraocular Pressure and Aqueous Humor Dynamics ...... 10.2

Aqueous Humor Production and Composition ...... 10.2.1 Aqueous Humor Outflow ...... 10.2.2 Trabecular Outflow ...... 10.2.2.1 Uveoscleral Outflow ...... 10.2.2.2 Episcleral Venous Pressure ...... 10.2.3 Intraocular Pressure ...... 10.2.4 Distribution in the Population and Relation to Glaucoma ...... 10.2.4.1 Factors Influencing Intraocular Pressure ...... 10.2.4.2 Clinical Measurement of Intraocular Pressure ...... 10.2.4.3

Clinical Evaluation ...... 10.3

History and General Examination ...... 10.3.1 Gonioscopy ...... 10.3.2 Direct and Indirect Gonioscopy ...... 10.3.2.1 The Optic Nerve ...... 10.3.3 Anatomy and Pathology ...... 10.3.3.1 Glaucomatous Optic Neuropathy ...... 10.3.4 Examination of the Optic Nerve Head...... 10.3.4.1 The Visual Field ...... 10.3.5 Factors Affecting Perimetry Results ...... 10.3.5.1

Open-Angle Glaucoma ...... 10.4

Primary Open-Angle Glaucoma ...... 10.4.1 Risk Factors ...... 10.4.1.1 Open-Angle Glaucoma Without Elevated IOP (Normal-Tension Glaucoma, Low-Tension Glaucoma) ...... 10.4.2 Risk Factors and Clinical Features ...... 10.4.2.1 Differential Diagnosis ...... 10.4.2.2 Ocular Hypertension ...... 10.4.3 Secondary Open-Angle Glaucoma ...... 10.4.4 Pseudoexfoliation Syndrome ...... 10.4.4.1 Pigment Dispersion Syndrome ...... 10.4.4.2 Lens-Induced Glaucoma ...... 10.4.4.3 Intraocular Tumors ...... 10.4.4.4 Ocular Inflammation and Secondary Glaucoma ...... 10.4.4.5 Elevated Episcleral Venous Pressure ...... 10.4.4.6

51 Trauma and Surgery ...... 10.4.4.7 Drugs and Glaucoma ...... 10.4.4.8

Angle-Closure Glaucoma ...... 10.5

Pathogenesis and Pathophysiology of Angle Closure ...... 10.5.1 Pupillary Block ...... 10.5.1.1 Angle Closure Without Pupillary Block...... 10.5.1.2 Primary Angle Closure ...... 10.5.2 Acute Primary Angle Closure ...... 10.5.2.1 Subacute or Intermittent Angle Closure ...... 10.5.2.2 Chronic Angle Closure ...... 10.5.2.3 Plateau Iris Syndrome ...... 10.5.3 Secondary Angle Closure Without Pupillary Block ...... 10.5.4 Neovascular Glaucoma ...... 10.5.4.1 Iridocorneal Endothelial Syndrome ...... 10.5.4.2 Tumors ...... 10.5.4.3 Inflammation ...... 10.5.4.4 Epithelial and Fibrous Ingrowth ...... 10.5.4.5 Trauma ...... 10.5.4.6 Retinal Surgery and Retinal Vascular Disease ...... 10.5.4.7 Nanophthalmos ...... 10.5.4.8 Drug-Induced Secondary Angle-Closure Glaucoma ...... 10.5.4.9

Glaucoma in Children and Adolescents ...... 10.6

Genetics ...... 10.6.1 Primary Congenital Glaucoma ...... 10.6.2 Juvenile Open-Angle Glaucoma ...... 10.6.3 Developmental Glaucomas With Associated Ocular or Systemic Anomalies ...... 10.6.4 Axenfeld-Rieger Syndrome ...... 10.6.4.1 Peters Anomaly ...... 10.6.4.2 Aniridia ...... 10.6.4.3 Sturge-Weber Syndrome ...... 10.6.4.4 Evaluating the Pediatric Glaucoma Patient...... 10.6.5 Treatment Overview ...... 10.6.6 Surgical Management ...... 10.6.6.1

Medical Management of Glaucoma ...... 10.7

Medical Agents ...... 10.7.1 General Approach to Medical Treatment ...... 10.7.2

Surgical Therapy for Glaucoma ...... 10.8 Laser Surgery ...... 10.8.1 Laser Trabeculoplasty ...... 10.8.1.1 Laser Iridotomy ...... 10.8.1.2 Laser Gonioplasty, or Peripheral Iridoplasty ...... 10.8.1.3 Cyclodestruction ...... 10.8.1.4 Incisional Surgery ...... 10.8.2 Trabeculectomy ...... 10.8.2.1 Combined Cataract and Trabeculectomy ...... 10.8.2.2 Cataract Extraction...... 10.8.2.3 Tube Shunt Implantation ...... 10.8.2.4 Nonpenetrating Glaucoma Surgery ...... 10.8.2.5 Other Glaucoma Surgeries ...... 10.8.2.6 Incisional Surgeries Specific to Angle-Closure Glaucoma ...... 10.8.2.7

52 CONTENT OUTLINE Content Outline Number

SECTION 11: LENS AND CATARACT ...... 11.0

Scored as part of Subtest 11: Lens and Cataract

Epidemiology of Cataracts ...... 11.1

Risk Factors for the Development of Cataract ...... 11.1.1

Anatomy ...... 11.2

Normal Crystalline Lens ...... 11.2.1 Capsule ...... 11.2.1.1 Zonular Fibers ...... 11.2.1.2 Lens Epithelium ...... 11.2.1.3 Nucleus and Cortex...... 11.2.1.4

Biochemistry and Physiology ...... 11.3

Crystallin Proteins ...... 11.3.1 Carbohydrate Metabolism ...... 11.3.2 Oxidative Damage and Protective Mechanisms ...... 11.3.3 Lens Physiology ...... 11.3.4 Maintenance of Lens Water and Cation Balance ...... 11.3.4.1 Accommodation and Presbyopia ...... 11.3.5

Embryology and Developmental Defects ...... 11.4

Normal Development of the Lens ...... 11.4.1 Primary Lens Fibers and the Embryonic Nucleus ...... 11.4.1.1 Secondary Lens Fibers ...... 11.4.1.2 Lens Sutures and the Fetal Nucleus ...... 11.4.1.3 Tunica Vasculosa Lentis ...... 11.4.1.4 Congenital Anomalies and Abnormalities ...... 11.4.2 Congenital Aphakia ...... 11.4.2.1 Lenticonus and Lentiglobus ...... 11.4.2.2 Lens Coloboma ...... 11.4.2.3 Mittendorf Dot ...... 11.4.2.4 Epicapsular Star ...... 11.4.2.5 Peters Anomaly ...... 11.4.2.6 Microspherophakia ...... 11.4.2.7 Aniridia ...... 11.4.2.8 Congenital Cataract ...... 11.4.2.9 Developmental Defects ...... 11.4.3 Ectopia Lentis ...... 11.4.3.1 Ectopia Lentis et Pupillae ...... 11.4.3.2 Persistent Fetal Vasculature ...... 11.4.3.3

Pathology ...... 11.5

Age-Related Lens Changes ...... 11.5.1 Nuclear Cataracts ...... 11.5.1.1

53 Cortical Cataracts ...... 11.5.1.2 Posterior Subcapsular Cataracts ...... 11.5.1.3 Drug-Induced Lens Changes ...... 11.5.2 Corticosteroids ...... 11.5.2.1 Phenothiazines ...... 11.5.2.2 Miotics ...... 11.5.2.3 Amiodarone ...... 11.5.2.4 Statins ...... 11.5.2.5 Trauma ...... 11.5.3 Contusion ...... 11.5.3.1 Perforating and Penetrating Injury ...... 11.5.3.2 Intraocular Procedures ...... 11.5.3.3 Intralenticular Foreign Bodies ...... 11.5.3.4 Radiation ...... 11.5.3.5 Metallosis ...... 11.5.3.6 Electrical Injury ...... 11.5.3.7 Chemical Injuries ...... 11.5.3.8 Metabolic Cataract ...... 11.5.4 Diabetes Mellitus ...... 11.5.4.1 Galactosemia ...... 11.5.4.2 Hypocalcemia ...... 11.5.4.3 Wilson Disease ...... 11.5.4.4 Myotonic Dystrophy...... 11.5.4.5 Effects of Nutrition, Alcohol, and Smoking ...... 11.5.5 Cataract Associated With Uveitis ...... 11.5.6 Pseudoexfoliation Syndrome ...... 11.5.7 Cataract and Atopic Dermatitis ...... 11.5.8 Phacoantigenic Uveitis ...... 11.5.9 Lens-Induced Glaucoma ...... 11.5.10 Phacolytic Glaucoma ...... 11.5.10.1 Lens Particle Glaucoma ...... 11.5.10.2 Phacomorphic Glaucoma ...... 11.5.10.3 Glaukomflecken ...... 11.5.10.4 Ischemia ...... 11.5.11 Cataracts Associated With Degenerative Ocular Disorders ...... 11.5.12

Evaluation and Management of Cataracts in Adults ...... 11.6

Clinical History: Signs and Symptoms ...... 11.6.1 Decreased Visual Acuity ...... 11.6.1.1 Glare and Altered Contrast Sensitivity ...... 11.6.1.2 Myopic Shift ...... 11.6.1.3 Monocular Diplopia or Polyopia ...... 11.6.1.4 Indications for Surgery ...... 11.6.2 Preoperative Evaluation ...... 11.6.3 General Health of the Patient ...... 11.6.3.1 Pertinent Ocular History ...... 11.6.3.2 Social History ...... 11.6.3.3 Measurements of Visual Function ...... 11.6.4 Visual Acuity Testing ...... 11.6.4.1 Refraction ...... 11.6.4.2 Glare Testing ...... 11.6.4.3 Contrast Sensitivity Testing ...... 11.6.4.4 External Examination ...... 11.6.5 Motility ...... 11.6.5.1 Pupils ...... 11.6.5.2

54 Slit-Lamp Examination ...... 11.6.6 Conjunctiva ...... 11.6.6.1 Cornea ...... 11.6.6.2 Anterior Chamber and Iris ...... 11.6.6.3 Crystalline Lens ...... 11.6.6.4 Limitations of Slit-Lamp Examination ...... 11.6.6.5 Fundus Evaluation ...... 11.6.7 Ophthalmoscopy ...... 11.6.7.1 Optic Nerve ...... 11.6.7.2 Fundus Evaluation With Opaque Media ...... 11.6.7.3 Special Tests ...... 11.6.8 Potential Acuity Estimation ...... 11.6.8.1 Visual Field Testing ...... 11.6.8.2 Objective Tests of Macular Function...... 11.6.8.3 Preoperative Measurements ...... 11.6.9 Biometry ...... 11.6.9.1 Corneal Topography ...... 11.6.9.2 Additional Evaluation of the Cornea ...... 11.6.9.3 IOL Power Determination ...... 11.6.10 IOL Calculation ...... 11.6.10.1 Preventing Errors in IOL Calculation, Selection, and Insertion ...... 11.6.10.2 Improving Outcomes ...... 11.6.10.3 Patient Preparation and Informed Consent...... 11.6.11

Surgery for Cataract ...... 11.7

Anesthesia for Cataract Surgery ...... 11.7.1 Antimicrobial Prophylaxis ...... 11.7.2 Before Surgery ...... 11.7.2.1 In Surgery ...... 11.7.2.2 Ophthalmic Viscosurgical Devices ...... 11.7.3 Physical Properties ...... 11.7.3.1 Uses of Ophthalmic Viscosurgical Devices ...... 11.7.3.2 Phacoemulsification: Instrumentation, Terminology, and Key Concepts ...... 11.7.4 Instrumentation ...... 11.7.4.1 Ultrasonic Technology Terminology...... 11.7.4.2 Vacuum Terminology ...... 11.7.4.3 Key Concepts and Advances in Phaco Power Delivery ...... 11.7.4.4 Irrigation ...... 11.7.4.5 Fluidics and Phacodynamics Terminology...... 11.7.4.6 Aspiration Pumps ...... 11.7.4.7 Outline of the Phacoemulsification Procedure ...... 11.7.5 Eye Marking and Time-Out ...... 11.7.5.1 Paracentesis ...... 11.7.5.2 Clear Corneal Incision ...... 11.7.5.3 Scleral Tunnel Incisions ...... 11.7.5.4 Continuous Curvilinear Capsulorrhexis...... 11.7.5.5 Hydrodissection ...... 11.7.5.6 Hydrodelineation ...... 11.7.5.7 Nuclear Rotation ...... 11.7.5.8 Nucleus Disassembly and Removal ...... 11.7.5.9 Techniques of Nucleus Disassembly ...... 11.7.5.10 Irrigation and Aspiration ...... 11.7.5.11 Insertion of the IOL...... 11.7.5.12 After IOL Insertion ...... 11.7.5.13 IOLs: Historical Perspectives and Lens Modifications ...... 11.7.6

55 Posterior Chamber IOLs and Other Lens Modifications ...... 11.7.6.1 Multifocal Lenses ...... 11.7.6.2 Modification of Preexisting Astigmatism ...... 11.7.7 Incision Size and Location ...... 11.7.7.1 Astigmatic Keratotomy ...... 11.7.7.2 Limbal Relaxing Incisions ...... 11.7.7.3 Toric IOLs ...... 11.7.7.4 Outcomes of Cataract Surgery ...... 11.7.8

Complications of Cataract Surgery ...... 11.8

Corneal Complications ...... 11.8.1 Corneal Edema ...... 11.8.1.1 Incision and Wound Complications ...... 11.8.1.2 Descemet Membrane Detachment ...... 11.8.1.3 Induced Astigmatism ...... 11.8.1.4 Other Anterior Segment Complications ...... 11.8.2 Epithelial or Fibrous Ingrowth ...... 11.8.2.1 Epithelial Downgrowth ...... 11.8.2.2 Toxic Anterior Segment Syndrome ...... 11.8.2.3 Shallow or Flat Anterior Chamber ...... 11.8.2.4 Elevated Intraocular Pressure ...... 11.8.2.5 Intraoperative Floppy Iris Syndrome ...... 11.8.2.6 Lens-Iris Diaphragm Retropulsion Syndrome ...... 11.8.2.7 Iridodialysis and Iris Trauma ...... 11.8.2.8 Ciliary Block Glaucoma ...... 11.8.2.9 Postoperative Uveitis ...... 11.8.2.10 Retained Lens Material ...... 11.8.2.11 Capsular Rupture ...... 11.8.2.12 Vitreous Prolapse in the Anterior Chamber ...... 11.8.2.13 Complications of IOL Implantation ...... 11.8.3 Decentration and Dislocation ...... 11.8.3.1 Pupillary Capture ...... 11.8.3.2 Capsular Block Syndrome ...... 11.8.3.3 Uveitis-Glaucoma-Hyphema Syndrome...... 11.8.3.4 Pseudophakic Bullous Keratopathy ...... 11.8.3.5 Unexpected Refractive Results ...... 11.8.3.6 IOL Glare, Dysphotopsia, and Opacification ...... 11.8.3.7 Capsular Opacification and Contraction ...... 11.8.4 Posterior Capsule Opacification ...... 11.8.4.1 Anterior Capsule Fibrosis and Phimosis ...... 11.8.4.2 Nd:YAG Laser Capsulotomy ...... 11.8.4.3 Hemorrhage ...... 11.8.5 Systemic Anticoagulation ...... 11.8.5.1 Retrobulbar Hemorrhage ...... 11.8.5.2 Hyphema ...... 11.8.5.3 Suprachoroidal Effusion or Hemorrhage...... 11.8.5.4 Expulsive Suprachoroidal Hemorrhage ...... 11.8.5.5 Delayed Suprachoroidal Hemorrhage...... 11.8.5.6 Endophthalmitis ...... 11.8.6 Diagnosis ...... 11.8.6.1 Treatment ...... 11.8.6.2 Retinal Complications ...... 11.8.7 Cystoid Macular Edema ...... 11.8.7.1 Retinal Light Toxicity ...... 11.8.7.2 Retinal Detachment...... 11.8.7.3

56 Needle Penetration of the Globe ...... 11.8.8

Preparing for Cataract Surgery in Special Situations ...... 11.9

Anticoagulation Therapy or Bleeding Disorders ...... 11.9.1 External Ocular Abnormalities ...... 11.9.2 Blepharitis and Acne Rosacea ...... 11.9.2.1 Keratoconjunctivitis Sicca ...... 11.9.2.2 Corneal Conditions ...... 11.9.3 Corneal Disease ...... 11.9.3.1 Cataract and Keratoplasty ...... 11.9.3.2 Cataract Following Keratoplasty ...... 11.9.3.3 Cataract Following Refractive Surgery ...... 11.9.3.4 Compromised Visualization of the Lens...... 11.9.4 Small Pupil ...... 11.9.4.1 Poor Red Reflex ...... 11.9.4.2 Altered Lens and Zonular Anatomy ...... 11.9.5 Intumescent Cataract ...... 11.9.5.1 Advanced Cataract...... 11.9.5.2 Posterior Polar Cataract ...... 11.9.5.3 Zonular Dehiscence With Lens Subluxation or Dislocation ...... 11.9.5.4 Pseudoexfoliation Syndrome ...... 11.9.5.5 Conditions Associated With Extremes in Axial Length ...... 11.9.6 High Myopia ...... 11.9.6.1 High Hyperopia and Nanophthalmos ...... 11.9.6.2 Glaucoma and Cataract ...... 11.9.7 Assessment ...... 11.9.7.1 Cataract Surgery in the Glaucoma Patient ...... 11.9.7.2 Cataract Surgery in an Eye With a Functioning Filter ...... 11.9.7.3 Uveitis...... 11.9.8 Retinal Conditions ...... 11.9.9 Retinal Disease ...... 11.9.9.1 Cataract Following Pars Plana Vitrectomy ...... 11.9.9.2 Cataract With Intraocular Silicone Oil ...... 11.9.9.3 Ocular Trauma ...... 11.9.10 Ocular Assessment ...... 11.9.10.1 Visualization During Surgery ...... 11.9.10.2 Retained Foreign Matter ...... 11.9.10.3 Cataract in an Eye With Damage to Other Ocular Tissues ...... 11.9.10.4 Removal of Traumatic Cataract ...... 11.9.10.5

57 CONTENT OUTLINE Content Outline Number

SECTION 12: RETINA AND VITREOUS ...... 12.0

Scored as part of Subtest 12: Retina and Vitreous

Basic Anatomy ...... 12.1

The Vitreous ...... 12.1.1 Neurosensory Retina ...... 12.1.2 Retinal Pigment Epithelium ...... 12.1.3 Bruch Membrane ...... 12.1.4 Choroid ...... 12.1.5 Sclera ...... 12.1.6

Diagnostic Approach to Retinal Disease ...... 12.2

Techniques of Examination ...... 12.2.1 Retinal Angiographic Techniques ...... 12.2.2 Fluorescein Angiography ...... 12.2.2.1 Indocyanine Green Angiography ...... 12.2.2.2 Optical Coherence Tomography ...... 12.2.3 Scanning Laser Ophthalmoscopy ...... 12.2.4 Fundus Autofluorescence ...... 12.2.5 Infrared Reflectance Imaging ...... 12.2.6 Fundus Near-Infrared Autofluorescence ...... 12.2.7

Retinal Physiology and Psychophysics ...... 12.3

Electroretinogram ...... 12.3.1 Electro-oculogram and RPE Responses ...... 12.3.2 Cortical Evoked Potentials ...... 12.3.3 Psychophysical Testing...... 12.3.4 Dark Adaptation ...... 12.3.4.1 Color Vision ...... 12.3.4.2 Contrast Sensitivity ...... 12.3.4.3 Systemic Drug Retinal Toxicity ...... 12.3.5

Age-Related Macular Degeneration ...... 12.4

Age-Related Macular Degeneration ...... 12.4.1 Genetics and AMD ...... 12.4.1.1 Nonneovascular Abnormalities in AMD ...... 12.4.1.2 Neovascular AMD ...... 12.4.1.3 Therapy for AMD ...... 12.4.2

Other Causes of Choroidal Neovascularization...... 12.5

Ocular Histoplasmosis Syndrome ...... 12.5.1 Idiopathic CNV ...... 12.5.2 Angioid Streaks ...... 12.5.3 Pathologic Myopia ...... 12.5.4 Miscellaneous Causes of CNV ...... 12.5.5

58 Therapy for Secondary Causes of CNV ...... 12.5.6

Diabetic Retinopathy ...... 12.6

Terminology and Classification ...... 12.6.1 Epidemiology of Diabetic Retinopathy ...... 12.6.2 Pathogenesis of Diabetic Retinopathy ...... 12.6.3 Systemic Medical Management of Diabetic Retinopathy ...... 12.6.4 Conditions Associated With Vision Loss From Diabetic Retinopathy ...... 12.6.5 Nonproliferative Diabetic Retinopathy ...... 12.6.6 Diabetic Macular Edema ...... 12.6.6.1 Diabetic Macular Ischemia ...... 12.6.6.2 Severe Nonproliferative Diabetic Retinopathy ...... 12.6.6.3 Proliferative Diabetic Retinopathy ...... 12.6.7 Nonsurgical Management of Proliferative Diabetic Retinopathy ...... 12.6.7.1 Surgical Management of Proliferative Diabetic Retinopathy ...... 12.6.7.2 Vitrectomy Surgery for Complications of Diabetic Retinopathy ...... 12.6.7.3 Cataract Surgery in Patients With Diabetes Mellitus ...... 12.6.8 Recommended Diabetes-Related Ophthalmic Examinations ...... 12.6.9

Other Retinal Vascular Diseases ...... 12.7

Systemic Arterial Hypertension ...... 12.7.1 Hypertensive Retinopathy ...... 12.7.1.1 Hypertensive Choroidopathy ...... 12.7.1.2 Hypertensive Optic Neuropathy ...... 12.7.1.3 Sickle Cell Retinopathy ...... 12.7.2 Nonproliferative Sickle Cell Retinopathy ...... 12.7.2.1 Proliferative Sickle Cell Retinopathy ...... 12.7.2.2 Other Ocular Abnormalities in Sickle Cell Hemoglobinopathies ...... 12.7.2.3 Management of Sickle Cell Retinopathy ...... 12.7.2.4 Peripheral Retinal Neovascularization ...... 12.7.3 Venous Occlusive Disease ...... 12.7.4 Branch Retinal Vein Occlusion ...... 12.7.4.1 Central Retinal Vein Occlusion ...... 12.7.4.2 Ocular Ischemic Syndrome and Retinopathy of Carotid Occlusive Disease ...... 12.7.5 Symptoms and Signs ...... 12.7.5.1 Etiology and Course ...... 12.7.5.2 Treatment of Ocular Ischemic Syndrome ...... 12.7.5.3 Arterial Occlusive Disease ...... 12.7.6 Capillary Retinal Arteriole Obstruction (Cotton-Wool Spots) ...... 12.7.6.1 Branch Retinal Artery Occlusion ...... 12.7.6.2 Cilioretinal Artery Occlusion ...... 12.7.6.3 Central Retinal Artery Occlusion ...... 12.7.6.4 Ophthalmic Artery Occlusion ...... 12.7.6.5 Vasculitis ...... 12.7.7 Cystoid Macular Edema ...... 12.7.8 Coats Disease ...... 12.7.9 Parafoveal (Juxtafoveal) Retinal Telangiectasia ...... 12.7.10 Arterial Macroaneurysms ...... 12.7.11 Phakomatoses ...... 12.7.12 Retinal Angiomatosis ...... 12.7.12.1 Congenital Retinal Arteriovenous Malformations ...... 12.7.12.2 Retinal Cavernous Hemangioma ...... 12.7.12.3 Radiation Retinopathy ...... 12.7.13 Valsalva Retinopathy ...... 12.7.14

59 Purtscher Retinopathy and Purtscherlike Retinopathy ...... 12.7.15 Terson Syndrome ...... 12.7.16

Retinopathy of Prematurity ...... 12.8

Epidemiology ...... 12.8.1 Classification and Terminology ...... 12.8.2 Pathogenesis ...... 12.8.3 Screening Recommendations ...... 12.8.4 Prevention and Risk Factors ...... 12.8.5 Treatment ...... 12.8.6 Laser and Cryoablation Surgery ...... 12.8.6.1 Anti-VEGF Drugs ...... 12.8.6.2 Vitrectomy and Scleral Buckling Surgery ...... 12.8.6.3

Choroidal Disease ...... 12.9

Central Serous Chorioretinopathy ...... 12.9.1 Choroidal Perfusion Abnormalities ...... 12.9.2 Increased Venous Pressure ...... 12.9.2.1 Hypertension ...... 12.9.2.2 Inflammatory Conditions ...... 12.9.2.3 Thromboembolic Disease ...... 12.9.2.4 Iatrogenic Abnormalities ...... 12.9.2.5 Choroidal Hemangioma ...... 12.9.3 Uveal Effusion Syndrome...... 12.9.4 Bilateral Diffuse Uveal Melanocytic Proliferation ...... 12.9.5

Focal and Diffuse Choroidal and Retinal Inflammation ...... 12.10

Noninfectious Retinal and Choroidal Inflammation ...... 12.10.1 White Dot Syndromes ...... 12.10.1.1 Acute Macular Neuroretinopathy ...... 12.10.1.2 Acute Idiopathic Maculopathy ...... 12.10.1.3 Acute Retinal Pigment Epitheliitis ...... 12.10.1.4 Solitary Idiopathic Choroiditis ...... 12.10.1.5 Inflammatory Vasculitis ...... 12.10.1.6 Intermediate Uveitis ...... 12.10.1.7 Panuveitis ...... 12.10.1.8 Uveitis Masquerade ...... 12.10.1.9 Infectious Retinal and Choroidal Inflammation ...... 12.10.2 Cytomegalovirus Infection ...... 12.10.2.1 Necrotizing Herpetic Retinitis ...... 12.10.2.2 Endogenous Bacterial Endophthalmitis ...... 12.10.2.3 Fungal Endophthalmitis ...... 12.10.2.4 Tuberculosis ...... 12.10.2.5 Syphilitic Chorioretinitis ...... 12.10.2.6 Cat-Scratch Disease ...... 12.10.2.7 Toxoplasmic Chorioretinitis ...... 12.10.2.8 Toxocariasis ...... 12.10.2.9 Lyme Disease ...... 12.10.2.10 Diffuse Unilateral Subacute Neuroretinitis ...... 12.10.2.11 West Nile Virus Chorioretinitis ...... 12.10.2.12

Congenital and Stationary Retinal Disease ...... 12.11

60 Color Vision (Cone System) Abnormalities ...... 12.11.1 Congenital Color Deficiency ...... 12.11.1.1 Achromatopsia ...... 12.11.1.2 Night Vision (Rod System) Abnormalities ...... 12.11.2 Congenital Night-Blinding Disorders With Normal Fundi ...... 12.11.2.1 Congenital Night-Blinding Disorders With Prominent Fundus Abnormality ...... 12.11.2.2

Hereditary Retinal and Choroidal Dystrophies ...... 12.12

Diagnostic and Prognostic Testing ...... 12.12.1 Diffuse Photoreceptor Dystrophies ...... 12.12.2 Retinitis Pigmentosa ...... 12.12.2.1 Cone Dystrophies ...... 12.12.2.2 Cone–Rod Dystrophies ...... 12.12.2.3 Macular Dystrophies ...... 12.12.3 Stargardt Disease ...... 12.12.3.1 Vitelliform Degenerations ...... 12.12.3.2 Familial (Dominant) Drusen ...... 12.12.3.3 Pattern Dystrophies...... 12.12.3.4 Sorsby Macular Dystrophy ...... 12.12.3.5 Choroidal Dystrophies ...... 12.12.4 Diffuse Degenerations ...... 12.12.4.1 Regional and Central Choroidal Dystrophies ...... 12.12.4.2 Inner Retinal and Vitreoretinal Dystrophies ...... 12.12.5 X-Linked Retinoschisis ...... 12.12.5.1 Goldmann-Favre Syndrome ...... 12.12.5.2

Retinal Degenerations Associated With Systemic Disease ...... 12.13

Disorders Involving Other Organ Systems ...... 12.13.1 Infantile-Onset to Early Childhood–Onset Syndromes ...... 12.13.1.1 Bardet-Biedl Complex of Diseases ...... 12.13.1.2 Hearing Loss and Pigmentary Retinopathy ...... 12.13.1.3 Neuromuscular Disorders ...... 12.13.1.4 Other Organ System Disorders ...... 12.13.1.5 Paraneoplastic Retinopathy ...... 12.13.1.6 Metabolic Diseases ...... 12.13.2 Albinism ...... 12.13.2.1 Central Nervous System Metabolic Abnormalities ...... 12.13.2.2 Amino Acid Disorders...... 12.13.2.3 Mitochondrial Disorders ...... 12.13.2.4 Systemic Drug Toxicity ...... 12.13.3 Chloroquine Derivatives ...... 12.13.3.1 Phenothiazines ...... 12.13.3.2 Other Drugs ...... 12.13.3.3

Retinal Detachment ...... 12.14

Retinal Breaks ...... 12.14.1 Traumatic Breaks ...... 12.14.1.1 Posterior Vitreous Detachment ...... 12.14.2 Examination and Management of PVD ...... 12.14.2.1 Lesions Predisposing Eyes to Retinal Detachment ...... 12.14.3 Lattice Degeneration ...... 12.14.3.1 Vitreoretinal Tufts ...... 12.14.3.2 Meridional Folds, Enclosed Ora Bays, and Peripheral Retinal Excavations ...... 12.14.3.3

61 Lesions Not Predisposing Eyes to Retinal Detachment ...... 12.14.4 Paving-Stone, or Cobblestone, Degeneration ...... 12.14.4.1 Retinal Pigment Epithelial Hyperplasia ...... 12.14.4.2 Retinal Pigment Epithelial Hypertrophy ...... 12.14.4.3 Peripheral Cystoid Degeneration ...... 12.14.4.4 Prophylactic Treatment of Retinal Breaks...... 12.14.5 Symptomatic Retinal Breaks ...... 12.14.5.1 Asymptomatic Retinal Breaks ...... 12.14.5.2 Prophylactic Treatment of Lattice Degeneration ...... 12.14.5.3 Aphakia and Pseudophakia ...... 12.14.5.4 Fellow Eye in Patients With Retinal Detachment ...... 12.14.5.5 Subclinical Retinal Detachment ...... 12.14.5.6 Retinal Detachment ...... 12.14.6 Rhegmatogenous Retinal Detachment ...... 12.14.6.1 Tractional Retinal Detachment ...... 12.14.6.2 Exudative Retinal Detachment ...... 12.14.6.3 Differential Diagnosis of Retinal Detachment ...... 12.14.7 Retinoschisis ...... 12.14.7.1 Optic Pit Maculopathy ...... 12.14.8

Diseases of the Vitreous and Vitreoretinal Interface ...... 12.15

Posterior Vitreous Detachment ...... 12.15.1 Epiretinal Membranes ...... 12.15.1.1 Vitreomacular Traction Syndrome ...... 12.15.1.2 Idiopathic Macular Holes ...... 12.15.1.3 Developmental Abnormalities ...... 12.15.2 Tunica Vasculosa Lentis ...... 12.15.2.1 Prepapillary Vascular Loops ...... 12.15.2.2 Persistent Fetal Vasculature ...... 12.15.2.3 Hereditary Hyaloideoretinopathies With Optically Empty Vitreous: Wagner and Stickler Syndromes ...... 12.15.3 Familial Exudative Vitreoretinopathy ...... 12.15.4 Vitreous Opacities ...... 12.15.5 Asteroid Hyalosis ...... 12.15.5.1 Cholesterolosis ...... 12.15.5.2 Amyloidosis ...... 12.15.5.3 Spontaneous Vitreous Hemorrhage ...... 12.15.5.4 Pigment Granules ...... 12.15.5.5 Vitreous Abnormalities Secondary to Surgery ...... 12.15.6

Posterior Segment Manifestations of Trauma ...... 12.16

Evaluation of the Patient After Ocular Trauma ...... 12.16.1 Blunt Trauma ...... 12.16.2 Vitreous Hemorrhage ...... 12.16.2.1 Commotio Retinae ...... 12.16.2.2 Choroidal Rupture ...... 12.16.2.3 Posttraumatic Macular Hole ...... 12.16.2.4 Traumatic Chorioretinal Disruption (Retinal Sclopetaria)...... 12.16.2.5 Scleral Rupture ...... 12.16.2.6 Lacerating and Penetrating Injuries ...... 12.16.3 Perforating Injuries ...... 12.16.4 Intraocular Foreign Bodies ...... 12.16.5 Surgical Techniques for Removal of Intraocular Foreign Bodies ...... 12.16.5.1 Retained Intraocular Foreign Bodies ...... 12.16.5.2

62 Posttraumatic Endophthalmitis ...... 12.16.6 Sympathetic Ophthalmia ...... 12.16.7 Shaken Baby Syndrome/Nonaccidental Trauma ...... 12.16.8 Avulsion of the Optic Disc ...... 12.16.9 Photic Damage ...... 12.16.10

Laser Therapy for Posterior Segment Diseases ...... 12.17

Basic Principles of Photocoagulation ...... 12.17.1 Choice of Laser Wavelength ...... 12.17.1.1 Indications ...... 12.17.1.2 Complications of Photocoagulation ...... 12.17.1.3 Transpupillary Thermotherapy ...... 12.17.2 Photodynamic Therapy ...... 12.17.3 Complications of Photodynamic Therapy ...... 12.17.3.1

Vitreoretinal Surgery ...... 12.18

Vitrectomy for Selected Macular Diseases ...... 12.18.1 Macular Epiretinal Membranes ...... 12.18.1.1 Vitreomacular Traction Syndrome ...... 12.18.1.2 Idiopathic Macular Holes ...... 12.18.1.3 Submacular Hemorrhage ...... 12.18.1.4 Subfoveal Choroidal Neovascularization ...... 12.18.1.5 Vitrectomy for Complications of Diabetic Retinopathy ...... 12.18.2 Vitreous Hemorrhage ...... 12.18.2.1 Diabetic Tractional Retinal Detachment...... 12.18.2.2 Diabetic Macular Edema ...... 12.18.2.3 Vitrectomy for Posterior Segment Complications of Anterior Segment Surgery ...... 12.18.3 Postoperative Endophthalmitis ...... 12.18.3.1 Retained Lens Fragments After Phacoemulsification ...... 12.18.3.2 Posteriorly Dislocated Intraocular Lenses ...... 12.18.3.3 Cystoid Macular Edema ...... 12.18.3.4 Suprachoroidal Hemorrhage ...... 12.18.3.5 Retinal Detachment Surgery ...... 12.18.4 Techniques for Surgical Repair of Retinal Detachments ...... 12.18.4.1 Complications of Pars Plana Vitrectomy ...... 12.18.5

63 CONTENT OUTLINE Content Outline Number

SECTION 13: REFRACTIVE SURGERY ...... 13.0

Scored as part of Subtest 13: Refractive Surgery

The Science of Refractive Surgery ...... 13.1

Corneal Optics ...... 13.1.1 Refractive Error: Optical Principles and Wavefront Analysis ...... 13.1.2 Measurement of Wavefront Aberrations and Graphical Representations ...... 13.1.2.1 Lower-Order Aberrations ...... 13.1.2.2 Higher-Order Aberrations ...... 13.1.2.3 Corneal Biomechanics ...... 13.1.3 Corneal Imaging for Keratorefractive Surgery ...... 13.1.4 Corneal Topography ...... 13.1.4.1 Corneal Tomography ...... 13.1.4.2 Indications for Corneal Imaging in Refractive Surgery ...... 13.1.4.3 The Role of Corneal Topography in Refractive Surgery ...... 13.1.4.4 Corneal Effects of Keratorefractive Surgery ...... 13.1.5 Incisional Techniques...... 13.1.5.1 Tissue Addition or Subtraction Techniques ...... 13.1.5.2 Alloplastic Material Addition Techniques ...... 13.1.5.3 Collagen Shrinkage Techniques ...... 13.1.5.4 Laser Biophysics ...... 13.1.6 Laser–Tissue Interactions ...... 13.1.6.1 Fundamentals of Excimer Laser Photoablation ...... 13.1.6.2 Types of Photoablating Lasers ...... 13.1.6.3 Corneal Wound Healing ...... 13.1.7

Patient Evaluation ...... 13.2

Patient History ...... 13.2.1 Patient Expectations ...... 13.2.1.1 Social History ...... 13.2.1.2 Medical History ...... 13.2.1.3 Pertinent Ocular History ...... 13.2.1.4 Patient Age, Presbyopia, and Monovision ...... 13.2.1.5 Examination ...... 13.2.2 Uncorrected Visual Acuity and Manifest and Cycloplegic Refraction ...... 13.2.2.1 Pupillary Examination...... 13.2.2.2 Ocular Motility, Confrontation Fields, and Ocular Anatomy ...... 13.2.2.3 Intraocular Pressure ...... 13.2.2.4 Slit-Lamp Examination ...... 13.2.2.5 Dilated Fundus Examination ...... 13.2.2.6 Ancillary Tests ...... 13.2.3 Corneal Topography ...... 13.2.3.1 Pachymetry ...... 13.2.3.2 Wavefront Analysis ...... 13.2.3.3 Calculation of Residual Stromal Bed Thickness After LASIK ...... 13.2.3.4 Discussion of Findings and Informed Consent ...... 13.2.4

Incisional Corneal Surgery...... 13.3

64 Incisional Correction of Myopia ...... 13.3.1 Radial Keratotomy in the United States ...... 13.3.1.1 Incisional Correction of Astigmatism ...... 13.3.2 Coupling ...... 13.3.2.1 Arcuate Keratotomy and Limbal Relaxing Incisions ...... 13.3.2.2 Instrumentation ...... 13.3.2.3 Surgical Techniques...... 13.3.2.4 Outcomes ...... 13.3.2.5 Complications ...... 13.3.2.6 Ocular Surgery After Arcuate Keratotomy and Limbal Relaxing Incisions ...... 13.3.2.7

Onlays and Inlays ...... 13.4

Keratophakia ...... 13.4.1 Homoplastic Corneal Inlays ...... 13.4.1.1 Alloplastic Corneal Inlays ...... 13.4.1.2 Epikeratoplasty ...... 13.4.2 Intrastromal Corneal Ring Segments ...... 13.4.3 Background ...... 13.4.3.1 Instrumentation ...... 13.4.3.2 Technique ...... 13.4.3.3 Outcomes ...... 13.4.3.4 Intacs and Keratoconus ...... 13.4.3.5 One or Two Intacs Segments? ...... 13.4.3.6 Complications ...... 13.4.3.7 Ectasia After LASIK...... 13.4.3.8 Uses for Intrastromal Corneal Ring Segments After LASIK ...... 13.4.3.9 Orthokeratology ...... 13.4.4

Photoablation: Techniques and Outcomes ...... 13.5

Excimer Laser ...... 13.5.1 Background ...... 13.5.1.1 Surface Ablation ...... 13.5.1.2 LASIK ...... 13.5.1.3 Wavefront-Optimized and Wavefront-Guided Ablations ...... 13.5.1.4 Patient Selection for Photoablation ...... 13.5.2 Special Considerations for Surface Ablation ...... 13.5.2.1 Special Considerations for LASIK ...... 13.5.2.2 Surgical Technique for Photoablation ...... 13.5.3 Calibration of the Excimer Laser ...... 13.5.3.1 Preoperative Planning and Laser Programming ...... 13.5.3.2 Preoperative Preparation of the Patient ...... 13.5.3.3 Preparation of the Bowman Layer or Stromal Bed for Excimer Ablation ...... 13.5.3.4 Application of Laser Treatment ...... 13.5.3.5 Immediate Postablation Measures ...... 13.5.3.6 Postoperative Care ...... 13.5.3.7 Refractive Outcomes ...... 13.5.4 Outcomes for Myopia ...... 13.5.4.1 Outcomes for Hyperopia ...... 13.5.4.2 Wavefront-Guided and Wavefront-Optimized Treatment Outcomes for Myopia and Hyperopia ...... 13.5.4.3 Re-treatment (Enhancements) ...... 13.5.5

Photoablation: Complications and Adverse Effects ...... 13.6

65 General Complications Related to Laser Ablation ...... 13.6.1 Overcorrection ...... 13.6.1.1 Undercorrection ...... 13.6.1.2 Optical Aberrations...... 13.6.1.3 Central Islands ...... 13.6.1.4 Decentered Ablations ...... 13.6.1.5 Corticosteroid-Induced Complications ...... 13.6.1.6 Central Toxic Keratopathy ...... 13.6.1.7 Infectious Keratitis ...... 13.6.1.8 Complications Unique to Surface Ablation...... 13.6.2 Persistent Epithelial Defects ...... 13.6.2.1 Sterile Infiltrates ...... 13.6.2.2 Corneal Haze ...... 13.6.2.3 Complications Unique to LASIK ...... 13.6.3 Microkeratome Complications ...... 13.6.3.1 Epithelial Sloughing or Defects ...... 13.6.3.2 Flap Striae ...... 13.6.3.3 Traumatic Flap Dislocation ...... 13.6.3.4 LASIK-Interface Complications ...... 13.6.3.5 Visual Disturbances Related to Femtosecond Laser LASIK Flaps ...... 13.6.3.6 Ectasia...... 13.6.4 Rare Complications ...... 13.6.5

Collagen Shrinkage and Crosslinking Procedures ...... 13.7

Collagen Shrinkage ...... 13.7.1 Laser Thermokeratoplasty ...... 13.7.1.1 Conductive Keratoplasty ...... 13.7.1.2 Collagen Crosslinking ...... 13.7.2

Intraocular Refractive Surgery ...... 13.8

Phakic Intraocular Lenses ...... 13.8.1 Advantages ...... 13.8.1.1 Disadvantages ...... 13.8.1.2 Patient Selection ...... 13.8.1.3 Surgical Technique ...... 13.8.1.4 Outcomes ...... 13.8.1.5 Complications ...... 13.8.1.6 Refractive Lens Exchange ...... 13.8.2 Patient Selection ...... 13.8.2.1 Surgical Planning and Technique ...... 13.8.2.2 IOL Power Calculations in Refractive Lens Exchange ...... 13.8.2.3 Complications ...... 13.8.2.4 Advantages ...... 13.8.2.5 Disadvantages ...... 13.8.2.6 Monofocal Intraocular Lenses ...... 13.8.3 Toric Intraocular Lenses...... 13.8.4 Patient Selection ...... 13.8.4.1 Planning and Surgical Technique ...... 13.8.4.2 Outcomes ...... 13.8.4.3 Complications Specific to Toric IOLs ...... 13.8.4.4 Light-Adjustable Intraocular Lenses ...... 13.8.5 Accommodating Intraocular Lenses ...... 13.8.6 Multifocal Intraocular Lenses ...... 13.8.7 Patient Selection ...... 13.8.7.1

66 Surgical Technique ...... 13.8.7.2 Outcomes ...... 13.8.7.3 Adverse Effects, Complications, and Patient Dissatisfaction with Multifocal IOLs .. 13.8.7.4 Bioptics ...... 13.8.8

Accommodative and Nonaccommodative Treatment of Presbyopia ...... 13.9

Theories of Accommodation ...... 13.9.1 Nonaccommodative Treatment of Presbyopia ...... 13.9.2 Monovision ...... 13.9.2.1 Conductive Keratoplasty ...... 13.9.2.2 Multifocal IOL Implants ...... 13.9.2.3 Custom or Multifocal Ablations ...... 13.9.2.4 Corneal Intrastromal Femtosecond Laser Treatment ...... 13.9.2.5 Corneal Inlays ...... 13.9.2.6 Accommodative Treatment of Presbyopia ...... 13.9.3 Scleral Surgery ...... 13.9.3.1 Femtosecond Lens Relaxation ...... 13.9.3.2 Accommodating IOLs ...... 13.9.3.3

Refractive Surgery in Ocular and Systemic Disease ...... 13.10

Ocular Conditions ...... 13.10.1 Ocular Surface Disease ...... 13.10.1.1 Herpesvirus Infection ...... 13.10.1.2 Keratoconus ...... 13.10.1.3 Post–Penetrating Keratoplasty ...... 13.10.1.4 Ocular Hypertension and Glaucoma ...... 13.10.1.5 Retinal Disease ...... 13.10.1.6 Amblyopia and Strabismus in Adults and Children ...... 13.10.1.7 Systemic Conditions ...... 13.10.2 Human Immunodeficiency Virus Infection ...... 13.10.2.1 Diabetes Mellitus ...... 13.10.2.2 Connective Tissue and Autoimmune Diseases ...... 13.10.2.3

Considerations After Refractive Surgery ...... 13.11

IOL Calculations After Refractive Surgery ...... 13.11.1 Eyes With Known Pre– and Post–Refractive Surgery Data ...... 13.11.1.1 Eyes With No Preoperative Information ...... 13.11.1.2 The ASCRS Online Post-Refractive IOL Power Calculator ...... 13.11.1.3 Retinal Detachment Repair After LASIK ...... 13.11.2 Corneal Transplantation After Refractive Surgery ...... 13.11.3 Contact Lens Use After Refractive Surgery ...... 13.11.4 Indications ...... 13.11.4.1 General Principles ...... 13.11.4.2 Contact Lenses After Radial Keratotomy ...... 13.11.4.3 Contact Lenses After Surface Ablation ...... 13.11.4.4 Contact Lenses After LASIK ...... 13.11.4.5 Glaucoma After Refractive Surgery ...... 13.11.5

67