2021–2022 BCSC Basic and Clinical Science Course™,Section 4: Ophthalmic Pathology and Intraocular Tumors.

449 Pages·2022·40.7 MB·English

by  coll

4
Ophthalmic Pathology and
Intraocular Tumors
Last major revision 2020–2021
2021–2022
BCSC
Basic and Clinical
Science Course™
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
10 AMA PRA Category 1 Credits™. Physicians should claim only the credit commensurate with
the extent of their participation in the activity.
CME expiration date: June 1, 2023. AMA PRA Category 1 Credits™ may be claimed only once between
June 1, 2020, 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 4
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 information and
recommendations should be verified, prior to use, with current information included in the manufac-
turers’ 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 reflect
indications not included in approved FDA labeling, or that are approved for use only in restricted
research settings. The FDA has stated that it is the responsibility of the physician to determine the
FDA status of each drug or device he or she wishes to use, and to use them with appropriate, in-
formed patient consent in compliance with applicable law. The Academy specifically disclaims any
and all liability for injury or other damages of any kind, from negligence or otherwise, for any and all
claims that may arise from the use of any recommendations or other information contained herein.
All trademarks, trade names, log os, brand names, and ser vice marks of the American Acade my of Oph-
thalmology (AAO), whether registered or unregistered, are the property of AAO and are protected by
US and international trademark laws. T hese trademarks include, but are not l imited to, AAO; AAOE;
AMERICAN ACAD EMY OF OPHTHALMOLOGY; BASIC AND CLINICAL S CIENCE COURSE;
BCSC; EYENET; EYEWIKI; FOCAL POINTS; FOCUS DESIGN (logo on cover); IRIS; IRIS REGISTRY;
ISRS; OKAP; ONE NETWORK; OPHTHALMOLOGY; OPHTHALMOLOGY G LAUCOMA;
OPHTHALMOLOGY RETINA; OPHTHALMOLOGY SCIENCE; OPHTHALMOLOGY WORLD
NEWS; PREFERRED PRACTICE PATTERN; PROTECTING SIGHT. EMPOWERING LIVES.; THE
OPHTHALMIC NEWS AND EDUCATION NETWORK.
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 4
Faculty for the Major Revision
Nasreen A. Syed, MD
Vivian Lee, MD
Chair
Philadelphia, Pennsylvania
Iowa City, Iowa
Jesse L. Berry, MD Kirtee Raparia, MBBS
Los Angeles, California Santa Clara, California
Steffen Heegaard, MD Alison H. Skalet, MD, PhD
Copenhagen, Denmark Portland, Oregon
Theresa Retue Kramer, MD
Baltimore, Maryland
The Acade my acknowledges the American Association of Ophthalmic Oncologists and Pa-
thologists for recommending faculty members to the BCSC Section 4 committee.
The Acad emy also acknowledges the following committees for review of this edition:
Committee on Aging: Patricia Chévez- Barrios, MD, Houston, Texas
Vision Rehabilitation Committee: William M. McLaughlin Jr, DO, Scranton, Pennsylvania
BCSC Resident/Fellow Reviewers: Sharon L. Jick, MD, Chair, St Louis, Missouri; Gordon
S. Crabtree, MD; Lindsay De Andrade, MD; Lilangi S. Ediriwickrema, MD; Brittni A.
Scruggs, MD, PhD; Duo (David) Xu, MD
Practicing Ophthalmologists Advisory Committee for Education: Stephen R. Klapper, MD,
Primary Reviewer, Carmel, Indiana; Bradley D. Fouraker, MD, Chair, Tampa, Florida; Cyn-
thia S. Chiu, MD, Oakland, California; George S. Ellis Jr, MD, New Orleans, Louisiana;
Gaurav K. Shah, MD, Town and Country, Missouri; Rosa A. Tang, MD, MPH, MBA, Houston,
Texas; Troy M. Tanji, MD, Waipahu, Hawaii; Michelle S. Ying, MD, Ladson, South Carolina
In addition, the Acade my acknowledges the following committee for assistance in devel-
oping Study Questions and Answers for this BCSC Section:
Resident Self- Assessment Committee: Robert A. Beaulieu, MD, Royal Oak, Michigan; Ben-
jamin W. Botsford, MD, Pittsburgh, Pennsylvania; Olga M. Ceron, MD, Worcester, Mas-
sa chu setts; Ian P. Conner, MD, PhD, Pittsburgh, Pennsylvania; Kimberly A. Crowder, MD,
Jackson, Missouri; James Andrew David, MD, New Orleans, Louisiana; Claire E. Fraser,
MD, PhD, Lexington, Kentucky; Kevin Halenda, MD, Augusta, Georgia; Rola N. Hamam,
MD, Beirut, Lebanon; Amanda D. Henderson, MD, Baltimore, Maryl and; Joshua Hen-
drix, MD, Dalton, Georgia; Matthew B. Kaufman, MD, Portland, Oregon; Sangeeta
Khanna, MD, St Louis, Missouri; Chandrasekharan Krishnan, MD, Boston, Mas sa chu-
setts; Ajay E. Kuriyan, MD, Pittsford, New York; Kevin E. Lai, MD, Indianapolis, Indiana;
Kenneth C. Lao, MD, Temple, Texas; Ken Y. Lin, MD, Irvine, California; Kelly T. Mitchell,
MD, Lubbock, Texas; Yasha S. Modi, MD, New York, New York; Matthew S. Pihlblad, MD,
McDonald, Pennsylvania; Lorraine A. Provencher, MD, Cincinnati, Ohio; Jamie B. Rosen-
berg, MD, New York, New York; Syed Mahmood Shah, MD, Pittsburgh, Pennsylvania;
Ann Shue, MD, Palo Alto, California; Misha F. Syed, MD, Galveston, Texas; Parisa Tara-
vati, MD, Seattle, Washington; Sarah Van Tassel, MD, New York, New York; Evan L. Wax-
man, MD, PhD, Pittsburgh, Pennsylvania; Jules A. Winokur, MD, G reat Neck, New York
Eu ro pean Board of Ophthalmology: Tero T. Kivelä, MD, Chair, Helsinki, Finland; Edoardo
Midena, MD, PhD, Liaison, Padua, Italy; Nikolaos E. Bechrakis, MD, FEBO, Innsbruck,
Austria; Sarah E. Coupland, MBBS, PhD, FRCPath, Liverpool, United Kingdom; Laurence
Desjardins, MD, Paris, France; Steffen Heegaard, MD, DMSc, Copenhagen, Denmark;
Elisabeth M. Messmer, MD, PhD, FEBO, Munich, Germany; Fiona Roberts, MBChB, MD,
FRCPath, Glasgow, United Kingdom
Financial Disclosures
Acad emy 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 ser vices consumed by or used in patients, or with any competing
commercial product or ser vice.
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 Bechrakis: Alcon Laboratories (C, S), Bayer (C, S), HOYA (S), Novartis (C)
Dr Conner: Ocugenix (C, O, P)
Dr Crowder: Gore (C), Medtronic (C), Phillips (C)
Dr Fouraker: Addition Technology (C, L), Alcon Laboratories (C, L), OASIS Medical (C, L)
Dr Hamam: AbbVie (C, L, S)
Dr Heegaard: MC2 (C), Novartis (L), Sanofi (L), Santen (L), Shire (L), Théa (L)
Dr Klapper: AdOM Advanced Optical Technologies (O)
Dr Kramer: Medical Costing LLC (O), OcuFLOW (O), Share the Cells (O)
Dr Kuriyan: Alimera Sciences (C), Allergan (C), Regeneron (C), Second Sight (S), Valeant (C)
Dr Lin: Johnson & Johnson (C)
Dr Messmer: Alcon Laboratories (C, L), Allergan (C, L), CROMA- PHARMA (C, L),
Dompé (C), Théa (C, L), URSAPHARM (L)
Dr Modi: Alimera (C), Allergan (C), Genentech (C), Novartis (C)
Dr Gaurav Shah: Allergan (C, S), Bausch + Lomb (L), DORC International, B.V./Dutch Oph-
thalmic, USA (S), Johnson & Johnson (L), QLT Phototherapeutics (C, L), Regeneron (C, L)
Dr Syed Shah: Quantum Analytics (O), Quantum Ophthalmic (O), Quantum Vision (O)
Dr Skalet: Castle Biosciences (C), Immunocore (C)
Dr Tang: EMD Serono (L), Horizon (C, S), Immunovant (S), Quark (C, S), Regenera (S),
Sanofi (L), Zeiss (L)
Dr Van Tassel: New World Medical (L)
*C = con sul tant fee, paid advisory boards, or fees for attending a meeting; E = employed by or received
a W2 from a commercial com pany; L = lecture fees or honoraria, travel fees or reimbursements when
speaking at the invitation of a commercial com pany; O = equity own ership/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 sup-
port from government agencies, foundations, device manufacturers, and/or phar ma ceu ti cal companies
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 ser vices consumed by or
used in patients, or with any competing commercial product or serv ice.
Recent Past Faculty
Michele M. Bloomer, MD
Dan S. Gombos, MD
Tero T. Kivelä, MD
Tatyana Milman, MD
Heather A. D. Potter, MD
Robert H. Rosa Jr, MD
In addition, the Acad emy gratefully acknowledges the contributions of numerous past
faculty and advisory committee members who have played an import ant role in the devel-
opment of previous editions of the Basic and Clinical Science Course.
American Acad emy 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 Man ag er
Stephanie Tanaka, Publications Man ag er
Susan Malloy, Acquisitions Editor and Program Man ag er
Jasmine Chen, Man ag er, E- Learning
Lana Ip, Senior Designer
Beth Collins, Medical Editor
Eric Gerdes, Interactive Designer
Lynda Hanwella, Publications Specialist
Debra Marchi, Permissions Assistant
American Acad emy of Ophthalmology
655 Beach Street
Box 7424
San Francisco, CA 94120-7424
Contents
Introduction to the BCSC . . . . . . . . . . . . . . . . . . . . . . xv
Objectives . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1
PART I Ophthalmic Pathology 3
. . . . . . . . . . . . . . . . .
1 Introduction to Part I . . . . . . . . . . . . . . . . . . . . . 5
Highlights . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 5
Overview . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 5
Orga nizational Framework and Basic Pathologic Concepts . . . . . . . . . 6
Topography . . . . . . . . . . . . . . . . . . . . . . . . . . . 7
Disease Pro cesses . . . . . . . . . . . . . . . . . . . . . . . . . 7
Differential Diagnosis . . . . . . . . . . . . . . . . . . . . . . 16
2 Specimen Handling . . . . . . . . . . . . . . . . . . . . . . 19
Highlights . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 19
Overview . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 19
Communication . . . . . . . . . . . . . . . . . . . . . . . . . . 20
Fixatives for Tissue Preservation . . . . . . . . . . . . . . . . . . . 21
Specimen Orientation . . . . . . . . . . . . . . . . . . . . . . . . 21
Gross Examination and Dissection . . . . . . . . . . . . . . . . . . 24
Pro cessing and Staining . . . . . . . . . . . . . . . . . . . . . . . 24
Tissue Pro cessing . . . . . . . . . . . . . . . . . . . . . . . . 24
Slide Preparation and Tissue Staining . . . . . . . . . . . . . . . 26
3 Special Testing and Procedures in Pathology . . . . . . . 29
Highlights . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 29
Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . 29
Immunohistochemistry . . . . . . . . . . . . . . . . . . . . . . . 29
Flow Cytometry, Molecular Pathology, and
Diagnostic Electron Microscopy . . . . . . . . . . . . . . . . . . 33
Flow Cytometry. . . . . . . . . . . . . . . . . . . . . . . . . 33
Molecular Pathology. . . . . . . . . . . . . . . . . . . . . . . 33
Diagnostic Electron Microscopy. . . . . . . . . . . . . . . . . . 39
Special Procedures . . . . . . . . . . . . . . . . . . . . . . . . . 39
Fine- Needle Aspiration Biopsy . . . . . . . . . . . . . . . . . . 39
Frozen Section . . . . . . . . . . . . . . . . . . . . . . . . . 40
4 Wound Repair . . . . . . . . . . . . . . . . . . . . . . . . . 43
Highlights . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 43
General Aspects of Wound Repair . . . . . . . . . . . . . . . . . . 43
vii
viii ● Contents
Wound Repair in Specific Ocular Tissues . . . . . . . . . . . . . . . 44
Cornea . . . . . . . . . . . . . . . . . . . . . . . . . . . . 45
Conjunctiva . . . . . . . . . . . . . . . . . . . . . . . . . . 45
Sclera . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 47
Uveal Tract. . . . . . . . . . . . . . . . . . . . . . . . . . . 47
Lens. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 47
Ret ina . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 48
Vitreous . . . . . . . . . . . . . . . . . . . . . . . . . . . . 48
Orbit and Ocular Adnexa . . . . . . . . . . . . . . . . . . . . 48
Optic Nerve . . . . . . . . . . . . . . . . . . . . . . . . . . 49
Pathologically Apparent Sequelae of Ocular Trauma. . . . . . . . . . . 49
5 Conjunctiva . . . . . . . . . . . . . . . . . . . . . . . . . . 55
Highlights . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 55
Topography . . . . . . . . . . . . . . . . . . . . . . . . . . . . 55
Developmental Anomalies . . . . . . . . . . . . . . . . . . . . . . 57
Choristomas . . . . . . . . . . . . . . . . . . . . . . . . . . 57
Hamartomas . . . . . . . . . . . . . . . . . . . . . . . . . . 57
Inflammation . . . . . . . . . . . . . . . . . . . . . . . . . . . 57
Acute or Chronic Conjunctivitis . . . . . . . . . . . . . . . . . 59
Infectious Conjunctivitis . . . . . . . . . . . . . . . . . . . . . 59
Noninfectious Conjunctivitis . . . . . . . . . . . . . . . . . . . 60
Papillary Versus Follicular Conjunctivitis . . . . . . . . . . . . . . 61
Granulomatous Conjunctivitis . . . . . . . . . . . . . . . . . . 61
Pyogenic Granuloma . . . . . . . . . . . . . . . . . . . . . . 63
Degenerations . . . . . . . . . . . . . . . . . . . . . . . . . . . 64
Pinguecula and Pterygium . . . . . . . . . . . . . . . . . . . . 64
Amyloid Deposits . . . . . . . . . . . . . . . . . . . . . . . . 67
Epithelial Inclusion Cyst . . . . . . . . . . . . . . . . . . . . . 68
Conjunctivochalasis . . . . . . . . . . . . . . . . . . . . . . . 68
Neoplasia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 69
Squamous Epithelial Lesions . . . . . . . . . . . . . . . . . . . 69
Melanocytic Lesions. . . . . . . . . . . . . . . . . . . . . . . 74
Lymphoid Lesions . . . . . . . . . . . . . . . . . . . . . . . 85
Glandular Neoplasms . . . . . . . . . . . . . . . . . . . . . . 88
Other Neoplasms . . . . . . . . . . . . . . . . . . . . . . . . 88
6 Cornea . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 89
Highlights . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 89
Topography . . . . . . . . . . . . . . . . . . . . . . . . . . . . 89
Developmental Anomalies . . . . . . . . . . . . . . . . . . . . . . 91
Dermoid. . . . . . . . . . . . . . . . . . . . . . . . . . . . 91
Peters Anomaly. . . . . . . . . . . . . . . . . . . . . . . . . 91
Inflammation . . . . . . . . . . . . . . . . . . . . . . . . . . . 91
Infectious Keratitis . . . . . . . . . . . . . . . . . . . . . . . 91
Noninfectious Keratitis . . . . . . . . . . . . . . . . . . . . . 98
Degenerations, Depositions, and Ectasias . . . . . . . . . . . . . . . 98
Contents ● ix
Salzmann Nodular Degeneration . . . . . . . . . . . . . . . . . 98
Calcific Band Keratopathy . . . . . . . . . . . . . . . . . . . . 99
Actinic Keratopathy . . . . . . . . . . . . . . . . . . . . . . . 100
Pinguecula and Pterygium . . . . . . . . . . . . . . . . . . . . 101
Pannus . . . . . . . . . . . . . . . . . . . . . . . . . . . . 101
Bullous Keratopathy. . . . . . . . . . . . . . . . . . . . . . . 101
Corneal Graft Failure . . . . . . . . . . . . . . . . . . . . . . 102
Corneal Pigment Deposits . . . . . . . . . . . . . . . . . . . . 104
Ectatic Disorders . . . . . . . . . . . . . . . . . . . . . . . . 104
Dystrophies . . . . . . . . . . . . . . . . . . . . . . . . . . . . 106
Epithelial and Subepithelial Dystrophies . . . . . . . . . . . . . . 107
Epithelial– Stromal TGFBI Dystrophies . . . . . . . . . . . . . . 107
Stromal Dystrophies. . . . . . . . . . . . . . . . . . . . . . . 110
Descemet Membrane and Endothelial Dystrophies . . . . . . . . . 111
Neoplasia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 116
7 Anterior Chamber and Trabecular Meshwork . . . . . . 117
Highlights . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 117
Topography . . . . . . . . . . . . . . . . . . . . . . . . . . . . 117
Developmental Anomalies . . . . . . . . . . . . . . . . . . . . . . 118
Primary Congenital Glaucoma . . . . . . . . . . . . . . . . . . 119
Anterior Segment Dysgenesis. . . . . . . . . . . . . . . . . . . 119
Inflammation . . . . . . . . . . . . . . . . . . . . . . . . . . . 120
Degenerations . . . . . . . . . . . . . . . . . . . . . . . . . . . 120
Iridocorneal Endothelial Syndrome . . . . . . . . . . . . . . . . 120
Secondary Glaucoma . . . . . . . . . . . . . . . . . . . . . . 122
Neoplasia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 128
8 Sclera . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 129
Highlights . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 129
Topography . . . . . . . . . . . . . . . . . . . . . . . . . . . . 129
Developmental Anomalies . . . . . . . . . . . . . . . . . . . . . . 130
Choristoma . . . . . . . . . . . . . . . . . . . . . . . . . . 130
Nanophthalmos . . . . . . . . . . . . . . . . . . . . . . . . 130
Microphthalmia . . . . . . . . . . . . . . . . . . . . . . . . 131
Inflammation . . . . . . . . . . . . . . . . . . . . . . . . . . . 131
Episcleritis . . . . . . . . . . . . . . . . . . . . . . . . . . . 131
Scleritis . . . . . . . . . . . . . . . . . . . . . . . . . . . . 131
Degenerations . . . . . . . . . . . . . . . . . . . . . . . . . . . 134
Senile Calcific Plaque . . . . . . . . . . . . . . . . . . . . . . 134
Scleral Staphyloma . . . . . . . . . . . . . . . . . . . . . . . 134
Melanoma- Associated Spongiform Scleropathy . . . . . . . . . . . 134
Neoplasia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 135
9 Lens. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 137
Highlights . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 137
Topography . . . . . . . . . . . . . . . . . . . . . . . . . . . . 137