The American Academy of Ophthalmology is accredited by the Accreditation Council for Continuing Medical Education 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.

Originally released June 2011; reviewed for currency September 2, 2014. CME expiration date: June 1, 2016. AMA PRA Category 1 Credits™ may be claimed only once during this period.

BCSC® volumes are designed to increase the physician’s ophthalmic knowledge through study and review. Users of this activity are encouraged to read the text and then answer the study questions provided at the back of the book.

To claim AMA PRA Category 1 Credits™ upon completion of this activity, learners must demonstrate appropriate knowledge and participation in the activity by taking the posttest for Section 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 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 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, informed 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.

AAO, AAOE, American Academy of Ophthalmology, Basic and Clinical Science Course, BCSC, EyeCare America, EyeNet, EyeSmart, EyeWiki, Focal Points, IRIS, ISRS, OKAP, ONE, Ophthalmic Technology Assessments, Ophthalmology, Preferred Practice Pattern, ProVision, SmartSight, The Ophthalmic News & Education Network, and The Eye M.D. Association are, among other marks, the registered trademarks and trademarks of the American Academy of Ophthalmology.

Cover image: From BCSC Section 12, Retina and Vitreous. Ultra-wide-field fundus photograph from a patient with von Hippel–Lindau disease. Courtesy of Colin A. McCannel, MD.

Copyright © 2015

American Academy of Ophthalmology

All rights reserved

Basic and Clinical Science Course

Louis B. Cantor, MD, Indianapolis, Indiana, Senior Secretary for Clinical Education Christopher J. Rapuano, MD, Philadelphia, Pennsylvania, Secretary for Ophthalmic

Knowledge

George A. Cioffi, MD, New York, New York, BCSC Course Chair

Section 4

Faculty

Robert H. Rosa, Jr, MD, Chair, Temple, Texas

Ronald Buggage, MD, New York, New York

George J. Harocopos, MD, St Louis, Missouri

Theresa Retue Kramer, MD, Tucson, Arizona

Tatyana Milman, MD, New York, New York

Nasreen Syed, MD, Iowa City, Iowa

Matthew W. Wilson, MD, Memphis, Tennessee

Jacob Pe’er, MD, Consultant, Jerusalem, Israel

Robert G. Fante, MD, Denver, Colorado

Practicing Ophthalmologists Advisory Committee for Education

Ron W. Pelton, MD, PhD, Colorado Springs, Colorado

Practicing Ophthalmologists Advisory Committee for Education

The Academy wishes to acknowledge the American Association of Ophthalmic Pathology for recommending faculty members to the BCSC Section 4 committee.

Financial Disclosures

Academy staff members who contributed to the development of this product state that within the past 12 months, 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 state the following financial relationships:

Dr Buggage: Novartis Pharmaceuticals, employee, equity ownership/stock options

Dr Rosa: Genentech, grant recipient; National Eye Institute, grant recipient

The other authors state that they have no significant financial interest or other relationship with the manufacturer of any commercial product discussed in the chapters that they contributed to this course or with the manufacturer of any competing commercial product.

Contents

Title Page

Copyright Page

Faculty and Disclosures

General Introduction

Visual Acuity Chart

Objectives

PART I Ophthalmic Pathology

1Introduction to Part I

Organization

Topography

Disease Process

General Diagnosis

Differential Diagnosis

2Wound Repair

General Aspects of Wound Repair

Healing in Specific Ocular Tissues

Cornea

Sclera

Limbus

Uvea

Lens

Retina

Vitreous

Eyelid, Orbit, and Lacrimal Tissues

Histologic Sequelae of Ocular Trauma

3Specimen Handling

Communication

Orientation

Transillumination

Gross Dissection

Processing and Staining

Fixatives

Tissue Processing

Tissue Staining

4Special Procedures

Immunohistochemistry

Flow Cytometry, Molecular Pathology, and Diagnostic Electron Microscopy

Flow Cytometry

Molecular Pathology

Diagnostic Electron Microscopy

Special Techniques

Fine-Needle Aspiration Biopsy

Frozen Section

5Conjunctiva

Topography

Congenital Anomalies

Choristomas

Hamartomas

Inflammations

Papillary Versus Follicular Conjunctivitis

Granulomatous Conjunctivitis

Infectious Conjunctivitis

Noninfectious Conjunctivitis

Pyogenic Granuloma

Degenerations

Pinguecula and Pterygium

Amyloid Deposits

Epithelial Inclusion Cyst

Neoplasia

Squamous Lesions

Melanocytic Lesions

Lymphocytic Lesions

Glandular Lesions

Other Neoplasms

6Cornea

Topography

Introduction to Corneal Pathology

Congenital Anomalies

Congenital Hereditary Endothelial Dystrophy

Posterior Polymorphous Dystrophy

Dermoid

Peters Anomaly

Inflammations

Infectious Keratitis

Noninfectious Keratitis

Degenerations and Dystrophies

Degenerations

Dystrophies

Neoplasia

7Anterior Chamber and Trabecular Meshwork

Topography

Congenital Anomalies

Primary Congenital Glaucoma

Anterior Segment Dysgenesis

Degenerations

Iridocorneal Endothelial Syndrome

Secondary Glaucoma With Material in the Trabecular Meshwork

8Sclera

Topography

Episclera

Stroma

Lamina Fusca

Congenital Anomalies

Choristoma

Nanophthalmos

Inflammations

Episcleritis

Scleritis

Degenerations

Senile Calcific Plaque

Scleral Staphyloma

Neoplasia

Fibrous Histiocytoma

Nodular Fasciitis

9Lens

Topography

Capsule

Epithelium

Cortex and Nucleus

Zonular Fibers

Congenital Anomalies

Congenital Aphakia

Lens Coloboma

Anterior Lenticonus (Lentiglobus)

Posterior Lenticonus (Lentiglobus)

Inflammations

Phacoantigenic Uveitis

Phacolytic Glaucoma

Propionibacterium acnes Endophthalmitis

Degenerations

Cataract and Other Abnormalities

Neoplasia and Associations With Systemic Disorders

Pathology of Intraocular Lenses

10 Vitreous

Topography

Congenital Anomalies

Persistent Fetal Vasculature

Bergmeister Papilla

Mittendorf Dot

Prepapillary Vascular Loops

Vitreous Cysts

Inflammations

Degenerations

Syneresis and Aging

Posterior Vitreous Detachment

Rhegmatogenous Retinal Detachment and Proliferative Vitreoretinopathy

Macular Holes

Hemorrhage

Asteroid Hyalosis

Vitreous Amyloidosis

Neoplasia

Intraocular Lymphoma

11 Retina and Retinal Pigment Epithelium

Topography

Neurosensory Retina

Retinal Pigment Epithelium

Congenital Anomalies

Albinism

Myelinated Nerve Fibers

Vascular Anomalies

Congenital Hypertrophy of the RPE

Inflammations

Infectious

Noninfectious

Degenerations

Typical and Reticular Peripheral Cystoid Degeneration and Retinoschisis

Lattice Degeneration

Paving-Stone Degeneration

Ischemia

Specific Ischemic Retinal Disorders

Diabetic Retinopathy

Retinopathy of Prematurity

Age-Related Macular Degeneration

Polypoidal Choroidal Vasculopathy

Macular Dystrophies

Diffuse Photoreceptor Dystrophies

Neoplasia

Retinoblastoma

Retinocytoma

Medulloepithelioma

Fuchs Adenoma

Combined Hamartoma of the Retina and RPE

Adenomas and Adenocarcinomas of the RPE

12 Uveal Tract

Topography

Iris

Ciliary Body

Choroid

Congenital Anomalies

Aniridia

Coloboma

Inflammations

Infectious

Noninfectious

Degenerations

Rubeosis Iridis

Hyalinization of the Ciliary Body

Choroidal Neovascularization

Neoplasia

Iris

Choroid and Ciliary Body

Metastatic Tumors

Other Uveal Tumors

Trauma

13 Eyelids

Topography

Congenital Anomalies

Distichiasis

Phakomatous Choristoma

Dermoid Cyst

Inflammations

Infectious

Noninfectious

Degenerations

Xanthelasma

Amyloid

Cysts

Epidermoid and Dermoid Cysts

Ductal Cysts

Neoplasia

Epidermal Neoplasms

Dermal Neoplasms

Appendage Neoplasms

Melanocytic Neoplasms

14 Orbit

Topography

Bony Orbit and Soft Tissues

Congenital Anomalies

Dermoid and Other Epithelial Cysts

Inflammations

Noninfectious

Infectious

Degenerations

Amyloid

Neoplasia

Lacrimal Sac Neoplasia

Lacrimal Gland Neoplasia

Lymphoproliferative Lesions

Soft-Tissue Tumors

Vascular Tumors

Tumors With Fibrous Differentiation

Tumors With Muscle Differentiation

Nerve Sheath Tumors

Adipose Tumors

Bony Lesions of the Orbit

Metastatic Tumors

Retinal Angiomas

Arteriovenous Malformation

19 Retinoblastoma

Genetic Counseling

Diagnostic Evaluation

Clinical Examination

Differential Diagnosis

Classification

Associated Conditions

Retinocytoma

Trilateral Retinoblastoma

Treatment

Enucleation

Chemotherapy

Photocoagulation and Hyperthermia

Cryotherapy

External-Beam Radiation Therapy

Plaque Radiotherapy (Brachytherapy)

Targeted Therapy

Spontaneous Regression

Prognosis

20 Ocular Involvement in Systemic Malignancies

Secondary Tumors of the Eye

Metastatic Carcinoma

Direct Intraocular Extension

Lymphomatous Tumors

Primary Intraocular Lymphoma

Uveal Lymphoid Infiltration

Ocular Manifestations of Leukemia

Appendix: American Joint Committee on Cancer (AJCC) Staging Forms, 2010 Basic Texts

Related Academy Materials

Requesting Continuing Medical Education Credit Create a Self Test