Table Of ContentThe Sclera
c.
Stephen Foster
Maite Sainz de la Maza
The Sclera
Foreword by Frederick A. lakobiec
With 134 Illustrations and 33 Color Plates
Springer Science+Business Media, LLC
C. Stephen Foster, MD
Associate Professor of Ophthalmology
Harvard Medical School
Director, Immunology and Uveitis Service
Massachusetts Eye and Ear Infirmary
Boston, MA 02114
USA
Maite Sainz de la Maza, MD, PhD
Assistant Professor of Ophthalmology
Central University of Barcelona
08036 Barcelona
Spain
Cover illustration: The eye of a patient with rheumatoid arthritis who has developed pro
gressively destructive necrotizing scleritis.
Library of Congress Cataloging-in-Publication Data
Foster, C. Stephen (Charles Stephen), 1942-
The sclera/C. Stephen Foster and Maite Sainz de la Maza.
p. cm.
Includes bibliographical references and index.
ISBN 978-1-4757-2345-8 ISBN 978-1-4757-2343-4 (eBook)
DOI 10.1007/978-1-4757-2343-4
1. Sclera-Diseases. I. Maza, Maite Sainz de lao II. Title.
[DNLM: 1. Scleritis. 2. Sclera. WW 230 F754s 1993]
RE328.F67 1993
617.7' 19-dc20
DNLMIDLC
for Library of Congress 93-10235
Printed on acid-free paper.
© 1994 Springer Science+ Business Media New York
Originally published by Springer-Verlag New York, Inc. in 1994
Softcover reprint of the hardcover 1st edition 1994
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987654321
ISBN 978-1-4757-2345-8
To our parents:
Carson and Martha Foster
Julio and Teresa Sainz de la Maza
Foreword
Over the past five years, in sharing patients with him, following his
research, and benefitting from his teaching, I have come to marvel at
Dr. Stephen Foster's mind, dedication, and productivity. No one has a
richer or more challenging clinical practice, has approached his clinical
care with more critical questioning, or has produced as much useful
clinical and basic research in his field.
Steve has kept meticulous clinical records with elegant photographic
documentation, which serve as the basis for the creation of this treatise.
He has been fortunate in his co-author, Dr. Sainz de la Maza, who
initially inveigled Steve to participate in this project and then set herself
the enormous task of repairing the lacuna occasioned by the nonavail
ability of the classic text by Watson and Hazeiman, The Sclera and
Systemic Disorders. Steve has taken great pride in the trainees who have
passed through his fellowship program, and has methodically tried to
select them from around the world in order to extend the influence of his
clinical and research traditions. Dr. Sainz de la Maza, who practices
academic ophthalmology in Barcelona, Spain, is a superb exemplar of the
fruits of this strategy; the ophthalmic communities, both American and
international, are in their debt for producing this textbook.
I have read many of the chapters in this textbook, and they augment
one's impressions of Steve's high standards of scholarship and originality.
Steve has also generously noted that the Massachusetts Eye and Ear
Infirmary's unique resources are very much embedded in the content of
this book. I have had several in-depth discussions with Steve and Dr.
Sainz de la Maza regarding pathogenetic concepts of scleritis, auto
immune diseases, and vasculitis, and have personally profited from those
dialogues. Now trainees, general ophthalmologists, specialists, and parti
cularly patients far and wide will benefit from the dissemination of this
unique database and codification of the principles of clinical management.
My admiration for Steve's work, which was great from a distance when I
was in New York, has simply mushroomed in proximity to him in Boston.
vii
viii Foreword
I am pleased to contemplate that this textbook will bring him and his
accomplishments closer to the entire ophthalmic community.
Frederick A. Jakobiec, M.D.
Henry Willard Williams Professor of Ophthalmology
Professor of Pathology, and
Chairman of Ophthalmology
Harvard Medical School
Chief of Ophthalmology
Massachusetts Eye and Ear Infirmary
Preface
The sclera composes 80% of the geographic extent of the exterior
confines or wall of the eyeball, yet it receives relatively little attention in
the ophthalmic literature. This is understandable, given the fact that
disorders of the sclera are not common and the fact that, when relatively
minor problems of the sclera do develop, healing without consequence is
the usual outcome. After all, a scar in the sclera is of little importance,
because the sclera is an opaque structure. Such a scar in the cornea, or an
opacity in the lens or vitreous, or a scar in the macula, of course, carries
infinitely more visual significance. But it is exactly this rarity of significant
scleral problems, coupled with the profound systemic implications that
some inflammatory disorders of the sclera carry, that makes studies of the
sclera and its disorders important. Indeed, a substantial proportion of
individuals who develop serious scleral inflammation are discovered to
have an occult systemic disease; in The Sclera and Systemic Disorders
Watson and Hazleman* emphasized that 27% of patients who develop
necrotizing scleritis are dead within 5 yr from a systemic, vasculitic lesion.
Watson and Hazleman also emphasized that because of the comparative
rarity of scleral disease, the diagnosis is often missed, and 40% of eyes
reported in one series of enucleated eyes had had a primary diagnosis of
scleritis.
We have written this book because the finest book ever written on this
subject, The Sclera and Systemic Disorders, by Watson and Hazleman
(published in 1976 by W.B. Saunders, Philadelphia, as Volume 2 in their
series, Major Problems in Ophthalmology), has been out of print, un
available through any source whatsoever, since 1985. Dr. Sainz de la
Maza was frustrated by this; she could find no copies of this magnificent
book in the medical library in Barcelona. Watson gave me his last copy of
the book, which he obtained from the attic of his home, and inscribed to
me "with all best wishes." It is this rare treasure that our efforts try to
emulate. The two books are different in style, organization, patient
populations that form the basis of the material, and, to a small degree,
point of view. But in many respects the books are quite similar: our
* Watson PG, Hazleman BL: The Sclera and Systemic Disorders, WB Saunders,
Philadelphia, 1976.
ix
x Preface
experiences corroborate theirs, and the philosophies born of those
separate experiences are identical.
We began with all that we had learned from Watson and Hazleman
and built on that excellent foundation. The basis of our experience
springs from the Immunology Service at the Massachusetts Eye and Ear
Infirmary, which was begun in 1977, and which has been devoted to the
study and care 'of patients with any inflammatory problem related to the
eye, from the lids to the optic nerve. The first Research Fellow joined
the service in 1980, and the first Clinical Fellow arrived in 1984. Between
1977 and 1992 approximately 45,000 patient visits have occurred, ap
proximately 6000 new patients have been evaluated, and 40 Ocular
Immunology Fellows have been trained in the Service. Dr. Sainz de
la Maza was one of those Fellows, and in the course of training she
developed a special interest in and affinity for patients with scleritis. It
was her initiative that was at the heart of the genesis of this project, and
it is entirely through her efforts that this project has been successfully
completed.
Our hope is that this book will serve as a resource for residents in
ophthalmology, for cornea and immunology fellows in training, and for
those ophthalmologists in practice and on faculties who have an interest
in patients with diseases of the sclera. The majority of the book is
devoted to scleral inflammation, because scleritis represents, by far, the
most common scleral disorder encountered in ophthalmic practice, and
because of the profound systemic implications of scleritis. The references
at the end of each chapter, although not exhaustive, are generous in
number and should provide the reader with more than enough original
source material for further reading. Finally, for those who have access to
a copy of the book by Watson and Hazleman, we would enthusiastically
encourage you to read their book as well as this one.
Acknowledgments
We acknowledge the help, patience, and understanding provided by our
patients in the course of treatment. This book, which is based on their
personal experiences, would not exist without them. So, too, we grate
fully acknowledge the generosity of referring physicians throughout New
England: not only have they referred patients, with all their varieties
of ocular inflammatory disorders, to the Immunology Service, but they
have allowed us to care for them longitudinally as well.
The physicians who have chosen to spend additional years training in
ocular immunology deserve special thanks. In the course of their training,
they assumed increasing amounts of responsibility in the care of our
patients, and without their help much of the work that has been done
would not have been finished. These individuals are listed separately on
the following page. The research associates and laboratory technicians
of the Hilles Immunology Laboratory and of the Rhoads Molecular
Immunology Laboratory are also gratefully acknowledged. These in
dividuals include Drs. Robin Campbell, Peter Wells, and Soon Jin Lee,
and Carolyn DiSiena, Lou Ann Caron, Beverly Rice, Tom Ihley, James
Dutt, Tong Zhen Zhao, Victor Correa, and Jane Lui.
Anterior segment and posterior segment photographers Kit Johnson
and Phil Ruderman, and retinal photographers Jeff Napoli, Martha
Cunningham, Ann Elias-Dreiker (CRA), and Alice George are respon
sible for most of the clinical illustrations that are included in this book,
and we are grateful to them for the fine quality of their work. Similarly,
we acknowledge Lauri Cook for her superb medical illustrations and
Richard Fleischer for his help in making publication prints. The per
sonnel of the Immunology Service, technicians, nurses, and secretaries,
are also acknowledged for their help in running an efficient operation,
which allows us to accomplish the day's work.
xi
Fellows of the Ocular
Immunology Service,
Massachusetts Eye and
Ear Infirmary
Former Fellows
LESLIE FUJIKAWA, M.D., Associate, University of the Pacific
RICHARD WETZIG, M.D., Private Practice, Colorado Springs, CO
JAMES KALPAXIS, M.D., Private Practice, San Antonio, TX
ROBIN CAMPBELL, Ph.D., Industry (Burroughs-Wellcome)
INGER SANDSTROM, M.D., Assoc. Professor, Karolinska Institute, Sweden
PAUL THOMPSON, M.D., Assoc. Professor, University of Montreal, Canada
RICHARD BAZIN, M.D., Assoc. Professor, Lasoli University, Quebec, Canada
PETER WELLS, Ph.D., Industry (Upjohn Pharmaceuticals)
LYE PHENG FONG, M.D., Assoc. Professor, University of Melbourne, Australia
MICHAEL RAIZMAN, M.D., Assoc. Professor, Tufts University, Boston, MA
E. MITCHEL OPREMCAK, M.D., Assoc. Professor, Ohio State University
GURINDER SINGH, M.D., Private Practice, Anaheim, CA
BARRY GOLUB, M.D., Assoc. Professor, SUNY Stonybrook
JOSEPH TAUBER, M.D., Assist. Professor, University of Missouri, Kansas City
HUM CHUNG, M.D., Assoc. Professor, Seoul University, Seoul, Korea
MAITE SAINZ DE LA MAZA, M.D., Assist. Professor, University of Barcelona,
Spain
THANH HOANG-XUAN, M.D., Assoc. Professor, University of Paris, France
MANDl ZALTAS, M.D., Fellow in Glaucoma, Boston, MA
NEIL TOLCHIN, M.D., Resident in Ophthalmology
EUGENE Lru, M.D., Resident in Ophthalmology
MARGARITA CALONGE, M.D., Assist. Professor, University of Valladolid, Spain
JOHN BAER, M.D., Assoc. Professor, University of Maryland
ALEJANDRO GARCIA RODRIGUEZ, M.D., University of Monterey, Mexico
TUYET MAl PHAN, M.D., Private Practice, Los Angeles, CA
RAMZI HEMADY, M.D., Assist. Professor, University of Maryland
RICHARD TAMESIS, M.D., University of Nebraska
SARKIS SOUKASIAN, M.D., Private Practice, Lahey Clinic, Burlington, MA
MANFRED ZIERHUT, M.D., Assist. Professor, Tubingen University, Germany
NADA JABBUR, M.D., Resident in Ophthalmology, George Washington University
XIN XIN CAl, M.D., Ph.D. Program, Tufts University, Boston, MA
MARTIN FILIPEC, M.D., Assoc. Professor, University of Prague, Czechoslovakia
SOON JIN LEE, Ph.D., Assist. Professor, University of Missouri
RON NEUMANN, M.D., Director of Immunology Research, Pharmos Corp.,
Israel
NEAL BARNEY, M.D., Assist. Professor, University of Wisconsin
xiii
