2021–2022 BCSC Basic and Clinical Science Course™,Section 12: Retina and Vitreous

489 Pages·2022·41.737 MB·English

by  coll

12
Retina and Vitreous
Last major revision 2018–2019
2021–2022
BCSC
Basic and Clinical
Science Course™
Funded in part by the Educational Trust
Fund/Retina Research Foundation
Published after collaborative
review with the European Board
of Ophthalmology subcommittee
The American Academy of Ophthalmology is accredited by the Accreditation Council for Con-
tinuing Medical Education (ACCME) to provide continuing medical education for physicians.
The American Academy of Ophthalmology designates this enduring material for a maximum of
15 AMA PRA Category 1 Credits™. Physicians should claim only the credit commensurate with
the extent of their participation in the activity.
Originally released June 2018; reviewed for currency August 2020; CME expiration date: June 1, 2022.
AMA PRA Category 1 Credits™ may be claimed only once between June 1, 2018, and the expiration date.
BCSC® volumes are designed to increase the physician’s ophthalmic knowledge through study and
review. Users of this activity are encouraged to read the text and then answer the study questions
provided at the back of the book.
To claim AMA PRA Category 1 Credits™ upon completion of this activity, learners must demon-
strate appropriate knowledge and participation in the activity by taking the posttest for Section 12
and achieving a score of 80% or higher. For further details, please see the instructions for requesting
CME credit at the back of the book.
The Academy provides this material for educational purposes only. It is not intended to represent
the only or best method or procedure in every case, nor to replace a physician’s own judgment or
give specific advice for case management. Including all indications, contraindications, side effects,
and alternative agents for each drug or treatment is beyond the scope of this material. All informa-
tion and recommendations should be verified, prior to use, with current information included in the
manufacturers’ package inserts or other independent sources, and considered in light of the patient’s
condition and history. Reference to certain drugs, instruments, and other products in this course is
made for illustrative purposes only and is not intended to constitute an endorsement of such. Some
material may include information on applications that are not considered community standard, that re-
flect indications not included in approved FDA labeling, or that are approved for use only in restricted
research settings. The FDA has stated that it is the responsibility of the physician to determine
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Cover image: From BCSC Section 8, External Disease and Cornea. Fluorescein brightly stains the base
of the herpes simplex virus epithelial dendritic lesions in a cornea after LASIK. (Courtesy of Arie L.
Marcovich, MD, PhD.)
Copyright © 2021 American Academy of Ophthalmology. All rights reserved. No part of
this publication may be reproduced without written permission.
Printed in China.
Basic and Clinical Science Course
Christopher J. Rapuano, MD, Philadelphia, Pennsylvania
Se nior Secretary for Clinical Education
J. Timothy Stout, MD, PhD, MBA, Houston, Texas
Secretary for Lifelong Learning and Assessment
Colin A. McCannel, MD, Los Angeles, California
BCSC Course Chair
Section 12
Faculty for the Major Revision
Colin A. McCannel, MD
Brian C. Leonard, MD
Chair
Ottawa, Canada
Los Angeles, California
Audina M. Berrocal, MD Richard B. Rosen, MD
Miami, Florida New York, New York
Graham E. Holder, PhD Richard F. Spaide, MD
London, United Kingdom New York, New York
Stephen J. Kim, MD Jennifer K. Sun, MD, MPH
Nashville, Tennessee Boston, Massachusetts
The Academy wishes to acknowledge the American Society of Retina Specialists (ASRS),
the Macula Society, and the Retina Society for recommending faculty members to the
BCSC Section 12 committee.
The Academy also wishes to acknowledge the following committees for review of this edition:
Committee on Aging: Amy C. Shefler, MD, Houston, Texas
Vision Rehabilitation Committee: William M. McLaughlin Jr, DO, Allentown, Pennsylvania
Practicing Ophthalmologists Advisory Committee for Education: David J. Browning, MD,
PhD, Primary Reviewer, Charlotte, North Carolina; Edward K. Isbey III, MD, Chair,
Asheville, North Carolina; Alice Bashinsky, MD, Asheville, North Carolina; Bradley
D. Fouraker, MD, Tampa, Florida; Steven J. Grosser, MD, Golden Valley, Minnesota;
Stephen R. Klapper, MD, Carmel, Indiana; James A. Savage, MD, Memphis, Tennessee;
Michelle S. Ying, MD, Ladson, South Carolina
The Acad emy also wishes to acknowledge the following committee for assistance in devel-
oping study questions and answers for this BCSC Section:
Self- Assessment Committee: Stephen R. Russell, MD, Iowa City, Iowa; Paul B. Griggs, MD,
Seattle, Washington; Rachel M. Huckfeldt, MD, Boston, Mas sa chu setts; Ravi S. J. Singh, MD,
Shawnee Mission, Kansas
In addition, the Acade my wishes to recognize the impor tant contributions of Michael B.
Gorin, MD, PhD, in the development of Chapter 13.
European Board of Ophthalmology: Catherine Creuzot-Garcher, MD, PhD, EBO Chair,
Dijon, France; Peter J. Ringens, MD, PhD, EBO Liaison, Maastricht, Netherlands; Anat
Loewenstein, MD, Tel Aviv-Yafo, Israel; Pascale G. Massin, MD, Paris, France; Edoardo
Midena, MD, Padua, Italy; Ramin Tadayoni, MD, PhD, Paris, France; Sebastian Wolf,
MD, PhD, Bern, Switzerland
Financial Disclosures
Academy staff members who contributed to the development of this product state that
within the 12 months prior to their contributions to this CME activity and for the dura-
tion of development, they have had no financial interest in or other relationship with any
entity discussed in this course that produces, markets, resells, or distributes ophthalmic
health care goods or services consumed by or used in patients, or with any competing
commercial product or service.
The authors and reviewers state that within the 12 months prior to their contributions
to this CME activity and for the duration of development, they have had the following
financial relationships:*
Dr Berrocal: Alcon (C), Allergan (C), Bausch + Lomb (C), Bayer HealthCare Phar ma ceu-
tic als (L), DORC Dutch Ophthalmic Research Center (International)/Dutch Ophthalmic
USA (L), Visunex Medical Systems (C)
Dr Browning: Aerpio Therapeutics (S), Alcon (S), Alimera Sciences (C), Genentech (S),
Novartis Phar ma ceu ti cals (S), Ohr Phar ma ceu ti cal (S), Pfizer (S), Regeneron Phar ma ceu-
tic als (S), ZEISS (O)
Dr Creuzot- Garcher: Allergan (C, L), Bausch + Lomb (C, L), Bayer (C, L), Novartis
Phar ma ceu ti cals (C, L, S), Roche (C), Théa (C, S)
Dr Fouraker: Addition Technology (C, L), Alcon (C, L), KeraVision (C, L), OASIS Medi-
cal (C, L)
Dr Gorin: University of Pittsburgh (P); University of California, Los Angeles (P)
Dr Grosser: Injectsense (O), Ivantis (O)
Dr Holder: Isarna Therapeutics (C), Roche (C), Servier Laboratories (C)
Dr Huckfeldt: Applied Ge ne tic Technologies Corporation (S), Spark Therapeutics (S)
Dr Isbey: Alcon (S), Allscripts (C), Bausch + Lomb (S), Medflow (C), Oculos Clinical
Research (S)
Dr Klapper: AdOM Advanced Optical Technologies (O)
Dr Leonard: Abbott Medical Optics (S), Alcon (S), Allergan (S), Annidis (O), Bausch + Lomb
(S), Bayer HealthCare Phar ma ceu tic als (S), Johnson & Johnson Vision (S), Novartis Phar-
ma ceu tic als (S), Ophthalmic Direct (S), Shire (S)
Dr Loewenstein: Alcon (C), Allergan (C, L), Bayer (C, L), ForSight Labs (C), Notal Vision (C),
Novartis Phar ma ceu ti cals (C, L)
Dr Massin: Allergan (C, L), Bayer (C), Novartis Pharm ac eut ic als (C, L)
Dr McCannel: DORC Dutch Ophthalmic Research Center (International)/Dutch Oph-
thalmic USA (C, L), Genentech (S), Insight Instruments (C), Santen Pharm ac eut ic al (C)
Dr Rosen: Allergan (S), Boehringer Ingelheim (C), cellVIEW (C), Clarity Medical Sys-
tems (C), Genentech (S), Glauco- Health (C), Guardion Health Sciences (C), Nano Ret ina
(C), Ocata Therapeutics (C), OD-O S (C), Opticology (O), Optovue (C, P), Regeneron
Phar ma ceu ti cals (C)
Dr Russell: Acucela (S), IDx (O), Spark Therapeutics (C, S), University of Iowa (P)
Dr Shefler: Allergan (C), Aura Biosciences (S), Genentech (S), Regeneron Phar ma ceu ti-
cals (S)
Dr Spaide: Bausch + Lomb (C), Genentech (C), Topcon Medical Systems (C, P)
Dr Sun: Adaptive Sensory Technology (S), Allergan (C), Bayer HealthCare Phar ma ceu ti-
cals (L), Boston Micromachines Corporation (S), Eleven Biotherapeutics (C), Genentech
(S), KalVista Phar mac eu ti cals (S), Merck & Co. (C), Novartis Pharm ac eut ic als (C), Op-
tovue (S), Regeneron Phar ma ceu ti cals (C)
Dr Tadayoni: Alcon (C, L, S), Alimera Sciences (C, L), Allergan (C, L, S), Bausch + Lomb
(C, L), Bayer HealthCare Phar ma ceu tic als (C, L), Genentech (C), Novartis Phar ma ceu ti-
cals (C, L, S), ThromboGenics (C), ZEISS (C, L)
Dr Wolf: Alcon (C), Allergan (C), Bayer HealthCare Phar ma ceu ti cals (C, L, S), Heidelberg
Engineering (C, S), Novartis Phar ma ceu tic als (C, S), Optos (S), Roche (C)
The other authors and reviewers state that within the 12 months prior to their contribu-
tions to this CME activity and for the duration of development, they have had no financial
interest in or other relationship with any entity discussed in this course that produces,
markets, resells, or distributes ophthalmic health care goods or services consumed by or
used in patients, or with any competing commercial product or service.
* C = consultant fee, paid advisory boards, or fees for attending a meeting; E = employed by or received
a W2 from a commercial company; L = lecture fees or honoraria, travel fees or reimbursements when
speaking at the invitation of a commercial company; O = equity ownership/stock options in publicly
or privately traded firms, excluding mutual funds; P = patents and/or royalties for intellectual property;
S = grant support or other financial support to the investigator from all sources, including research support
from government agencies, foundations, device manufacturers, and/or pharmaceutical companies
Recent Past Faculty
Neal H. Atebara, MD
Emmett Cunningham Jr, MD, PhD, MPH
David Sarraf, MD
In addition, the Academy gratefully acknowledges the contributions of numerous past
faculty and advisory committee members who have played an important role in the devel-
opment of previous editions of the Basic and Clinical Science Course.
American Academy of Ophthalmology Staff
Dale E. Fajardo, EdD, MBA, Vice President, Education
Beth Wilson, Director, Continuing Professional Development
Denise Evenson, Director, Brand & Creative
Ann McGuire, Acquisitions and Development Manager
Stephanie Tanaka, Publications Manager
Susan Malloy, Acquisitions Editor and Program Manager
Jasmine Chen, Manager of E-Learning
Lana Ip, Senior Designer
Beth Collins, Medical Editor
Eric Gerdes, Interactive Designer
Lynda Hanwella, Publications Specialist
American Academy of Ophthalmology
655 Beach Street
Box 7424
San Francisco, CA 94120-7424
Contents
Introduction to the BCSC . . . . . . . . . . . . . . . . . . . . . . xvii
Objectives . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1
Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . 3
PART I Fundamentals and Diagnostic
Approaches 5
. . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
1 Basic Anatomy. . . . . . . . . . . . . . . . . . . . . . . . . . 7
The Vitreous . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 7
Neurosensory Ret ina . . . . . . . . . . . . . . . . . . . . . . . . . 9
Ret i nal Topography . . . . . . . . . . . . . . . . . . . . . . . . 9
Ret i nal Layers and Neurosensory Elem ents . . . . . . . . . . . . . 10
Ret i nal Vasculature and Oxygen Supply . . . . . . . . . . . . . . 16
Ret i nal Pigment Epithelium . . . . . . . . . . . . . . . . . . . . . 17
Bruch Membrane. . . . . . . . . . . . . . . . . . . . . . . . . . 19
Choroid . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 19
Sclera . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 20
2 Diagnostic Approach to Ret i nal Disease . . . . . . . . . . 21
Ophthalmoscopy . . . . . . . . . . . . . . . . . . . . . . . . . . 21
Imaging Technologies . . . . . . . . . . . . . . . . . . . . . . . . 22
Fundus Camera Imaging . . . . . . . . . . . . . . . . . . . . . 22
Scanning Laser Ophthalmoscopy . . . . . . . . . . . . . . . . . 24
Optical Coherence Tomography. . . . . . . . . . . . . . . . . . 25
Optical Coherence Tomography Angiography. . . . . . . . . . . . 28
Fundus Autofluorescence. . . . . . . . . . . . . . . . . . . . . 29
Adaptive Optics Imaging. . . . . . . . . . . . . . . . . . . . . 33
Ret i nal Angiographic Techniques . . . . . . . . . . . . . . . . . 33
Ultrasonography . . . . . . . . . . . . . . . . . . . . . . . . 39
3 Ret i nal Physiology and Psychophysics . . . . . . . . . . . 41
Electrophysiologic Testing . . . . . . . . . . . . . . . . . . . . . . 41
Electroretinography. . . . . . . . . . . . . . . . . . . . . . . . . 42
Full- Field (Ganzfeld) ERG . . . . . . . . . . . . . . . . . . . . 42
Multifocal ERG . . . . . . . . . . . . . . . . . . . . . . . . . 45
Pattern ERG . . . . . . . . . . . . . . . . . . . . . . . . . . 45
Clinical Considerations . . . . . . . . . . . . . . . . . . . . . 46
Electro- oculography . . . . . . . . . . . . . . . . . . . . . . . . 50
Visual Evoked Cortical Potentials . . . . . . . . . . . . . . . . . . . 52
Psychophysical Testing . . . . . . . . . . . . . . . . . . . . . . . 53
vii
viii  Contents
Color Vision . . . . . . . . . . . . . . . . . . . . . . . . . . 54
Contrast Sensitivity . . . . . . . . . . . . . . . . . . . . . . . 56
Dark Adaptometry . . . . . . . . . . . . . . . . . . . . . . . 57
PART II Disorders of the Ret ina and Vitreous 59
. . . . . .
4 Age- Related Macular Degeneration and Other
Causes of Choroidal Neovascularization . . . . . . . . . . 61
Age- Related Macular Degeneration . . . . . . . . . . . . . . . . . . 61
Ge ne tics and AMD . . . . . . . . . . . . . . . . . . . . . . . 62
Nonneovascular AMD. . . . . . . . . . . . . . . . . . . . . . 63
Neovascular AMD . . . . . . . . . . . . . . . . . . . . . . . 71
Other Causes of Choroidal Neovascularization . . . . . . . . . . . . . 86
Ocular Histoplasmosis Syndrome . . . . . . . . . . . . . . . . . 86
Angioid Streaks. . . . . . . . . . . . . . . . . . . . . . . . . 87
Pathologic Myopia . . . . . . . . . . . . . . . . . . . . . . . 89
Idiopathic CNV and Miscellaneous C auses of CNV . . . . . . . . . 89
5 Ret i nal Vascular Disease: Diabetic Retinopathy. . . . . . 91
Terminology and Classification. . . . . . . . . . . . . . . . . . . . 91
Diabetes Terminology . . . . . . . . . . . . . . . . . . . . . . 91
Diabetic Retinopathy Terminology . . . . . . . . . . . . . . . . 91
Epidemiology of Diabetic Retinopathy. . . . . . . . . . . . . . . . . 92
Pathogenesis of Diabetic Retinopathy . . . . . . . . . . . . . . . . . 93
Recommended Diabetes Mellitus– Related Ophthalmic
Examinations . . . . . . . . . . . . . . . . . . . . . . . . . . 93
Systemic Medical Management of Diabetic Retinopathy . . . . . . . . . 96
Abnormalities Associated With Vision Loss From Diabetic
Retinopathy . . . . . . . . . . . . . . . . . . . . . . . . . . . 98
Nonproliferative Diabetic Retinopathy. . . . . . . . . . . . . . . . . 99
Treatment of Nonproliferative Diabetic Retinopathy . . . . . . . . . 101
Proliferative Diabetic Retinopathy. . . . . . . . . . . . . . . . . . . 102
Management of Proliferative Diabetic Retinopathy
and Its Complications . . . . . . . . . . . . . . . . . . . . . 103
Diabetic Macular Edema. . . . . . . . . . . . . . . . . . . . . . . 108
Classification of Diabetic Macular Edema . . . . . . . . . . . . . 109
Treatment of Diabetic Macular Edema . . . . . . . . . . . . . . . 110
Cataract Surgery in Patients With Diabetes Mellitus . . . . . . . . . . . 116
6 Ret i nal Vascular Diseases Associated
With Cardiovascular Disease . . . . . . . . . . . . . . . 121
Systemic Arterial Hypertension. . . . . . . . . . . . . . . . . . . . 121
Hypertensive Retinopathy . . . . . . . . . . . . . . . . . . . . 121
Hypertensive Choroidopathy . . . . . . . . . . . . . . . . . . . 123
Hypertensive Optic Neuropathy. . . . . . . . . . . . . . . . . . 123
Contents  ix
Ret i nal Vein Occlusion . . . . . . . . . . . . . . . . . . . . . . . 125
Branch Ret i nal Vein Occlusion . . . . . . . . . . . . . . . . . . 126
Central Ret i nal Vein Occlusion . . . . . . . . . . . . . . . . . . 130
Pharmacologic Management of Reti n al Vein Occlusion. . . . . . . . 135
Ocular Ischemic Syndrome and Retinopathy of Carotid
Occlusive Disease . . . . . . . . . . . . . . . . . . . . . . . . 138
Symptoms and Signs of Ocular Ischemic Syndrome . . . . . . . . . 138
Etiology and Course of Ocular Ischemic Syndrome . . . . . . . . . 139
Treatment of Ocular Ischemic Syndrome . . . . . . . . . . . . . . 140
Arterial Occlusive Disease . . . . . . . . . . . . . . . . . . . . . . 140
Capillary Ret i nal Arteriole Obstruction (Cotton-W ool Spots) . . . . . 140
Branch Ret i nal Artery Occlusion . . . . . . . . . . . . . . . . . 141
Cilioret i nal Artery Occlusion . . . . . . . . . . . . . . . . . . . 143
Paracentral Acute Middle Maculopathy . . . . . . . . . . . . . . 143
Central Ret i nal Artery Occlusion . . . . . . . . . . . . . . . . . 143
Ophthalmic Artery Occlusion. . . . . . . . . . . . . . . . . . . 146
Arterial Macroaneurysms . . . . . . . . . . . . . . . . . . . . . . 147
7 Other Ret i nal Vascular Diseases. . . . . . . . . . . . . . 149
Sickle Cell Retinopathy . . . . . . . . . . . . . . . . . . . . . . . 149
Nonproliferative Sickle Cell Retinopathy . . . . . . . . . . . . . . 149
Proliferative Sickle Cell Retinopathy . . . . . . . . . . . . . . . . 150
Other Ocular Abnormalities in Sickle Cell Hemoglobinopathies . . . . 154
Management of Sickle Cell Retinopathy . . . . . . . . . . . . . . 154
Vasculitis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 155
Cystoid Macular Edema . . . . . . . . . . . . . . . . . . . . . . . 156
Etiologies of CME. . . . . . . . . . . . . . . . . . . . . . . . 157
Incidence of CME. . . . . . . . . . . . . . . . . . . . . . . . 157
Treatment of CME . . . . . . . . . . . . . . . . . . . . . . . 159
Coats Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . 159
Macular Telangiectasia . . . . . . . . . . . . . . . . . . . . . . . 161
Macular Telangiectasia Type 1 . . . . . . . . . . . . . . . . . . 161
Macular Telangiectasia Type 2 . . . . . . . . . . . . . . . . . . 161
Macular Telangiectasia Type 3 . . . . . . . . . . . . . . . . . . 164
Phakomatoses . . . . . . . . . . . . . . . . . . . . . . . . . . . 165
Von Hippel– Lindau Disease . . . . . . . . . . . . . . . . . . . 165
Wyburn- Mason Syndrome . . . . . . . . . . . . . . . . . . . . 168
Ret i nal Cavernous Hemangioma . . . . . . . . . . . . . . . . . 168
Radiation Retinopathy. . . . . . . . . . . . . . . . . . . . . . . . 170
Valsalva Retinopathy . . . . . . . . . . . . . . . . . . . . . . . . 171
Purtscher Retinopathy and Purtscherlike Retinopathy . . . . . . . . . . 171
Terson Syndrome. . . . . . . . . . . . . . . . . . . . . . . . . . 173