Ocular Pathology - Doody · 2010-09-02 · Ocular Pathology. is most highly recommended for all...

Ocular Pathology

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Commissioning Editor: Russell GabbedyDevelopment Editor: Sharon NashProject Manager: Bryan PotterDesign: Charles GrayMarketing Manager(s) (UK/USA): John Canelon/William Veltre


MYRON YANOFF, MD

Professor and ChairDepartment of OpthalmologyDrexel University College of MedicinePhiladelphia PA

JOSEPH W. SASSANI, MD MHA

Professor of Ophthalmology and PathologyPennsylvania State UniversityHershey Medical CenterHershey, PA

Ocular Pathology

SIXTH EDITION


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Foreword . . . . . . . . . . . . . . . . . vii

Forewords to the First Edition . . . . ix

Preface. . . . . . . . . . . . . . . . . . . xi

Dedication . . . . . . . . . . . . . . . . xiii

Chapter 1 Basic Principles of Pathology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1

Chapter 2 Congenital Anomalies. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 29

Chapter 3 Nongranulomatous Infl ammation: Uveitis, Endophthalmitis, Panophthalmitis, and Sequelae . . . . 57

Chapter 4 Granulomatous Infl ammation. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 73

Chapter 5 Surgical and Nonsurgical Trauma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 105

Chapter 6 Skin and Lacrimal Drainage System . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 165

Chapter 7 Conjunctiva . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 223

Chapter 8 Cornea and Sclera . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 255

Chapter 9 Uvea . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 333

Chapter 10 Lens . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 361

Chapter 11 Neural (Sensory) Retina. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 393

Chapter 12 Vitreous . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 481

Chapter 13 Optic Nerve . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 497

Chapter 14 Orbit. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 529

Chapter 15 Diabetes Mellitus . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 595

Chapter 16 Glaucoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 625

Chapter 17 Ocular Melanocytic Tumors. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 667

Chapter 18 Retinoblastoma and Pseudoglioma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 733

Index . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 765

Contents


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When I was invited to write the Foreword for this sixth edition of Yanoff and Fine’s Ocular Pathology, by Myron Yanoff and Joseph W. Sassani, I felt honored considering that those who have authored the Foreword for the prior editions were amongst the “masters” of academic ophthalmology and ophthalmic pathol-ogy, namely Drs. Morton E. Smith, J. Donald M. Gass, Frederick A. Jakobiec, Paul Henkind, Harold G. Scheie, and Lorenz E. Zimmerman.

Th is textbook is one of the few in my 35 years of practice of ophthalmology and ophthalmic pathology that I have obtained from the immediate availability of each edition, since it fi rst was published in 1975, both for my clinical offi ce and for my oph-thalmic pathology research laboratory that is inhabited by my fellows, residents, and medical students. Furthermore, the authors, originally Myron Yanoff and Ben Fine, and now including Joe Sassani, have been both career-long colleagues and very close friends of mine, mainly as a result of the frequent crossing of our paths at multiple annual academic meetings; these events almost always include our spouses, giving us a feeling of family with the elite in our fi eld.

As expressed by the writers of the Foreword of the previous editions, Ocular Pathology is without a doubt the best current ophthalmic pathology textbook, being a combination of a well organized review text presented in point-like fashion and an atlas with an extensive number of color photographs of most of the described ophthalmic conditions. Furthermore, it is one of the few textbooks on this important subject that is consistently be-ing updated with the latest clinical and pathologic information including more recent “avant-garde” disciplines associated with ophthalmic pathology such as the corresponding advances in molecular genetics.

Th e names, “Yanoff ” and “Fine” as co-authors of major text-books in academic ophthalmology are indicative of a fruitful “marriage” of two of the most outstanding contributors to our knowledge of ophthalmic pathology during the past four decades. Th ese two individuals have shared their extensive experience involving two diff erent approaches to this discipline, namely histopathology and electron microscopy respectively, in a con-tinuous, composite fashion almost unmatched in academic ophthalmology.

Dedicating this sixth edition of Ocular Pathology as a tribute to the late Ben S. Fine is most appropriate. Ben, a fellow Canadian, was a pioneer of the electron microscopic examination of ocular tissues. It was my good fortune that Ben’s academic offi ce was directly across from my cubicle during my fellowship at the Armed Forces Institute of Pathology (AFIP) during the early 1970’s. From Ben I learned not only the basics of electron microscopy, which I still practice today, but also his approach to ocular diseases utilizing uniquely clear thought processes. Ben was always available to share his expertise and gave the premier course on the

Foreword

ultrastructure of normal ocular tissues for several decades in addition to his publications on the electron microscopic fi ndings of numerous pathologic ocular disease processes.

Myron Yanoff has demonstrated amazing energy, dedication and profi ciency in the development of this text while serving both as director of ophthalmic pathology and as a most success-ful chair of the Department of Ophthalmology, currently at Drexel University in Philadelphia. During the past 4 decades, Myron has been a full professor of both ophthalmology and pathology; his distinguished career was culminated by his being the recipient of the American Academy of Ophthalmology’s Zimmerman Gold Medal just prior to the onset of the 21st century. I take my hat off to his continuous productivity and to his seeing to it that this “jewel” in academic ophthalmology is being perpetuated.

Th e addition of Dr. Joe Sassani as a co-author is a masterful step forward to ensure the continuity of this most important textbook in ophthalmic pathology. Joe is a disciple of Myron Yanoff who has evolved to be one of the leaders in the fi eld of ophthalmic pathology including having served as president of the American Association of Ophthalmic Pathologists. Joe is direc-tor of ophthalmic pathology and professor of ophthalmology and pathology at the Milton Hershey Medical Center in Hershey Pennsylvania. He also provides additional perspective in his fi elds of clinical expertise including glaucoma. Having this relatively youthful star participate in the authorship should ensure the continuation of this invaluable resource especially since Joe has broad academic shoulders with a strong reputation of responsibil-ity and commitment.

Ocular Pathology is most highly recommended for all residents in ophthalmology and fellows in ophthalmic pathology and is an excellent resource for medical students, general pathologists who review ocular specimens, and ophthalmologists who desire to fulfi ll their academic curiosity. It provides very succinctly the basics of ophthalmic pathology and includes the key information on almost every described ophthalmological disease process with an extensive reference list to enable more elaborate further studies. I would like to express “Bravo” to the authors for provid-ing this update of their masterpiece from which so many will benefi t.

Seymour Brownstein, MD FRCSC Les Amis Chair in Vision Science Professor, Departments of Ophthalmology

and Laboratory Medicine (Pathology) Director, Ophthalmic Pathology Laboratory University of Ottawa Eye Institute Th e Ottawa Hospital / University of Ottawa Ottawa Health Research Institute Ottawa, Ontario, Canada


Forewords to the First Edition

During the year of the observance of the 100th anniversary (1874–1974) of the University of Pennsylvania’s Department of Ophthalmology, it is exciting to have the publication of a volume whose coauthors have contributed signifi cantly to the strides in ocular pathology taken by the Department in the past several years.

Myron Yanoff , a highly regarded member of our staff , began a residency in ophthalmology in 1962, upon graduating from the University’s School of Medicine. Th e residency continued for the next fi ve years, during the fi rst two of which he also held a resi-dency in the Department of Pathology. His keen interest and ability in ocular pathology were readily apparent, and I encour-aged him to apply for a fellowship at the Armed Forces Institute of Pathology (AFIP), Washington, DC. From July, 1964, through June, 1965, he carried out exceptional research at the AFIP in both ophthalmology and pathology. He returned to our Depart-ment in July, 1965, where the caliber both of his clinical and research work was of the highest. When he completed his resi-dency in June, 1967, I invited him to join the staff , and he has recently attained the rank of full professor. During the ensuing years he has contributed substantially to the literature, particu-larly in the fi elds of ophthalmic and experimental pathology. He is Board certifi ed in ophthalmology and in pathology.

Ben Fine, noted for his work in electron microscopy at AFIP and at George Washington University, has shared his expertise in the fi eld through lectures presented as part of the curriculum of the annual 16-week Basic Science Course in the Department’s graduate program.

It can be said that 100 years ago ophthalmology was a spe-cialty that had been gradually evolving during the preceding 100 years, dating from the time of the invention of bifocals by Benjamin Franklin in 1785. Few American physicians of that era, however, knew how to treat diseases of the eye, but as medical education became more specialized it was inevitable that ophthalmology would also become a specialty.

With the invention of the ophthalmoscope in 1851, great advances were made in the reaching and practice of ophthalmol-ogy. Th is contributed greatly, of course, to setting the scene for the establishment of the University’s Department of Ophthal-mology. It was on February 3, 1874, that Dr. William F. Norris was elected First Clinical Professor of Diseases of the Eye. Similar chairs had been established earlier in only three other institutions. Th e chair at the University of Pennsylvania later became known as the William F. Norris and George E. de Schweinitz Chair of Ophthalmology.

Both Dr. Norris and Dr. de Schweinitz actively engaged in the study of ocular pathology. Dr. Norris stressed the importance

of the examination of the eye by microscopy and of the correla-tion of fi ndings from pathology specimens with the clinical signs. Dr. de Schweinitz was instrumental in having a member of his staff accepted as ophthalmic pathologist with the Department of Pathology.

In the years that followed under succeeding chairmen of the Department, other aspects of ophthalmology were stressed. Th en, in 1947, during the chairmanship of Dr. Francis Heed Adler, Dr. Larry L. Calkins was appointed to a residency. Dr. Calkins, like Dr. Yanoff , displayed a keen interest in ocular pathology. Accord-ingly, he was instrumental in its study being revitalized during the three years of his residency. Another resident, Dr. William C. Frayer, who came to the Department in 1949, joined Dr. Calkins in his interest in ocular pathology. Dr. Frayer received additional training in the Department of Pathology and then became the ophthalmic pathologist of the department.

Th e importance of ocular pathology was increasingly evident, but facilities for carrying out the work in the Department of Ophthalmology were unfortunately limited. Until 1964, the pathology laboratory had been confi ned to a small room in the outpatient area of the Department. Th en we were able to acquire larger quarters in the Pathology Building of the Philadelphia General Hospital located next door to the Hospital of the Uni-versity of Pennsylvania. Although the building was earmarked for eventual demolition, the space was fairly adequate for research and also for conducting weekly ophthalmic pathology teaching conferences. Despite the physical aspects, we saw to it that Dr. Yanoff and his team of workers had a well equipped laboratory.

During the next several years as I saw that my dream for an eye institute with facilities for patient care, reaching and research under one roof was to become a reality, I was delighted to be able to include prime space on the research fl oor for the ever enlarging scope of ocular pathology. In addition to all that Dr. Yanoff has had to build upon from the past tradition of our Department of Ophthalmology, I would like to think that the new facilities at the Institute have in some measure contributed to the contents of this excellent volume. With grateful appreciation, therefore, I look upon this book as the authors’ birthday present to the Department. From these same facilities, as Dr. Yanoff and Dr. Fine continue to collaborate, I can hope will come insights and answers for which all of us are ever searching in the battle against eye disease.

Harold G. Scheie, MD Chairman, Department of Ophthalmology University of Pennsylvania Director, Scheie Eye Institute


x

From their earliest days in ophthalmology Myron Yanoff and Ben Fine impressed me as exceptional students. As they have matured and progressed up the academic ladder, they have become equally dedicated and eff ective teachers. Th eir anatomical studies of normal and diseased tissues have always been oriented toward providing meaningful answers to practical as well as eso-teric clinical questions. Th eir ability to draw upon their large personal experience in clinical ophthalmology, ocular pathology, and laboratory investigation for their lectures at the Armed Forces Institute of Pathology and at the University of Pennsyl-vania have contributed immeasurably to the success of those courses. Now they have used the same time-tested approach in assembling their material for this book. Beginning with their basic lecture outlines, then expanding these with just enough text to substitute for what would have been said verbally in lecture, adding a remarkable amount of illustrative material for the amount of space consumed, and then providing pertinent refer-ences to get the more ambitious student started in the pursuit of a subject, Drs. Yanoff and Fine have provided us with a sorely needed teaching aid for both the student and the teacher of ocular pathology. It should prove to be especially popular among

medical students and residents in both ophthalmology and ocular pathology. With it one gets good orientation from the well-conceived outlines and fi ne clinicopathologic correlations from the selection of appropriate illustrations.

It is with considerable pride and admiration that I’ve watched the evolution of the authors’ work and its fruition in the form of this latest book. I am proud that both authors launched their respective careers with periods of intensive study at the Armed Forces Institute of Pathology and that ever since, they have remained loyal, dedicated, and highly ethical colleagues. I admire their youthful energy, their patient, careful attitude, their friendly cooperative nature, and their ability to get important things accomplished. I’m appreciative of this opportunity to express my gratitude for the work they have been doing. If it is true that “by his pupils, a teacher will be judged,” I could only wish to have had several dozen more like Drs. Yanoff and Fine.

Lorenz E. Zimmerman, MD Chief, Ophthalmic Pathology Division Armed Forces Institute of Pathology Washington, DC

Forewords to the First Edition


Preface

It has been 33 years since the fi rst edition of OCULAR PATHOLOGY was published in 1975. At that time the book contained the basics of eye pathology, which still are very much current. New entities have appeared and have been incorporated into subsequent editions, as well as into this edition. Th e enor-mous recent explosion of information that has expanded our knowledge and understanding of ocular pathology has occurred mainly in the fi elds of genetics, immunohistochemistry, and molecular biology. Newer imaging techniques such ultrasound biomicroscopy and optical coherence tomography are bringing resolution approaching histopathologic techniques to the clinical setting. We have integrated the pertinent information from these fi elds into this sixth edition.

Among the numerous entities introduced into this sixth edi-tion are De Barsy Syndrome; autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy, endothelial dystrophy, iris hypoplasia, congenital cataract, and stromal thinning (EDICT) syndrome; dentatorubropallidoluysian atrophy; microdot stro-mal degeneration; corneal fi brosis syndrome; toxic anterior segment syndrome; laryngo-onycho-cutaneous (LOC or Shabbir) syndrome; conjunctivochalasis; nevus lipomatosus (pedunculated nevus); Laugier-Hunziker syndrome; melanoma-associated spongiform scleropathy; Knobloch Syndrome; Maff uci’s syn-drome, malignant mesenchymoma; mantle cell lymphoma; con-genital simple hamartoma of the retinal pigment epithelium; TNM (tumor, node, metastasis) classifi cation; posterior microph-thalmos; lymphedema–distichiasis syndrome; solitary spindle-cell xanthogranuloma; arteriovenous malformation of the iris; retinal angiomatous proliferation; complications of LASIK surgery; fi brous hamartoma of infancy, and others.

Additionally, numerous existing topics have been updated. Th ey include the classifi cation system for retinoblastoma, genetic features of persistent hyperplastic primary vitreous, pathobiology of Norrie’s disease, multiple new insights into the pathology of incontinentia pigmenti, anatomic and pathologic correlates in the cornea, immunopathology of Herpes keratitis, updated genetics of corneal dystrophies, new features of Schnyder’s corneal crystal-line dystrophy (central stromal crystalline corneal dystrophy) and keratoconus, complications of retinal reattachment and glaucoma surgery, pathobiology of corneal abrasion; extensive revision of such topics as the anatomy of the conjunctiva, the pathobiology of vernal keratoconjunctivitis, and of graft-versus-host disease.

Other updated topics include the pathobiology of conjunctival and orbital lymphoma, squamous cell carcinoma of the conjunc-tiva, the genetics of pseudoexfoliation and uveal melanoma, and the inclusion of specifi c entities such as Fabry disease.

Particular attention has been directed at the pathobiology of diabetes mellitus. New and modifi ed diabetes-related topics include the international clinical classifi cation of diabetic reti-nopathy, diabetic, diabetic macular edema severity scales, and the role of VEGF and other factors in the pathobiology of diabetic complications.

Multiple changes and updates in Glaucoma include; syn-dromes associated with congenital glaucoma such as Hennekam syndrome, nail-patella syndrome, and familial amyloidotic poly-neuropathy type I (Met 30). Th e pathobiology of myocilin/TIGR gene in the development of glaucoma has been revised and new clinical syndromes associated with angle-closure glaucoma have been added. Th e pathobiology of corticosteroid-induced glau-coma, and of glaucoma-associated damage to ocular tissues have been expanded. Latest features on the pathobiology of central corneal thickness as it relates to the development and diagnosis of glaucoma are discussed.

A unique feature, introduced in this 6th edition of OCULAR PATHOLOGY, is a DVD. It contains the contents of seven histopathology glass slides and the ImageScopeTM software required to view them. Th e software permits the observation of these virtual microscope slides on the computer video monitor by varying magnifi cation, fi eld of observation, etc. just as could be done with an optical microscope. Also included on the DVD, are all of the images from the book, which are search-able and able to be exported to HTML/PowerPoint slide shows.

Finally, all illustrations have been digitally enhanced and color corrected. New tables and numerous updated references have been added.

Th is book could not have been completed without the under-standing and patience of our wives Karin L. Yanoff , Ph.D. and Gloria Sassani, M.A. We also wish to acknowledge the help of our assistants, William F. Devers and Sharon Dunkle. Finally, the members of the Elsevier production and editorial team including Russell Gabbedy and Sharon Nash, and also Bryan Potter, Charles Gray and William Veltre have provided invaluable help and guidance in the production of this 6th edition of OCULAR PATHOLOGY.


Dedication

Th e fi fth edition of this book was dedicated to the memory of Fruma I. Fine, the wife of Ben S. Fine, the long-time coauthor of this book. Now Ben is no longer with us but his memory, so vivid, lives on as an inspiration to the multitude of people trained by him, and also to those who have been educated by him through his seminal writings. Ben was one of the earliest pioneers in the fi eld of ocular electron microscopy, fi rst clarifying in exquisite

detail the normal anatomy of the eye, then elucidating the mys-teries of numerous ocular pathological entities. His ground-breaking discoveries remain unchallenged as some of the fi nest work done in the fi eld, and we miss him always. We dedi-cate this sixth edition to his memory, for his spirit lives on in the pages of this book and in the minds and souls of his peers and students.


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