The 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 All rights reserved. This work may not be translated or copied in whole or in part without the written permission of the publisher, Springer Science+Business Media, LLC. except for brief excerpts in connection with reviews or, scholarly analysis. Use in connection with any form of information storage and retrieval, electronic adaptation, computer software, or by similar or dissimilar methodology now known or hereafter developed is forbidden. 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Color separations by Best-set Typesetter Ltd., and color printing by New England Book Components. 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.