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Orbital

Disease

Orbital

Disease

Present Status and

Future Challenges

edited by

Jack Rootman

Boca Raton London New York Singapore

Published in 2005 by Taylor & Francis Group

6000 Broken Sound Parkway NW, Suite 300 Boca Raton, FL 33487-2742

© 2005 by Taylor & Francis Group, LLC

No claim to original U.S. Government works

Printed in the United States of America on acid-free paper 10 9 8 7 6 5 4 3 2 1

International Standard Book Number-10: 0-8247-4089-0 (Hardcover)

International Standard Book Number-13: 978-0-8247-4089-4 (Hardcover)

This book contains information obtained from authentic and highly regarded sources. Reprinted material is quoted with permission, and sources are indicated. A wide variety of references are listed. Reasonable efforts have been made to publish reliable data and information, but the author and the publisher cannot assume responsibility for the validity of all materials or for the consequences of their use.

No part of this book may be reprinted, reproduced, transmitted, or utilized in any form by any electronic, mechanical, or other means, now known or hereafter invented, including photocopying, microfilming, and recording, or in any information storage or retrieval system, without written permission from the publishers.

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Catalog record is available from the Library of Congress

Visit the Taylor & Francis Web site at

http://www.taylorandfrancis.com

Taylor & Francis Group

is the Academic Division of T&F Informa plc.

Contents

Introduction . . . . xi

Contributors . . . . xiii

ORBITAL INFLAMMATORY DISEASE

1.Orbital Inflammatory Disease: Classification and New Insights . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1

Jack Rootman

Definition, Classification, and Changing Paradigm in Orbital Inflammatory Disease . . . . 2

Role of Biopsy . . . . 2

The Role of Immune Mediators in Specific Inflammations . . . . 8

Changing Paradigms in the Treatment of Inflammation: The Rheumatologic Approach . . . . 9

Directed Treatments of Distinct Orbital Inflammations . . . . 9

2.Changing Paradigms in the Treatment

of Inflammatory Disorders . . . . . . . . . . . . . . . . . 15

Kam Shojania

Abstract . . . . 15

iii

iv

Contents

3.Bacterial Infections of the Orbit: Current Status and Future Challenges . . . . . . . . . . . . . . . . . . . . 25

Gerald J. Harris

4.Inflammation: Definitions, Regulatory Mechanisms,

and Contributions to Disease . . . . . . . . . . . . . .

33

Bruce M. McManus and Jonathan C. Choy

 

5.Future and Emerging Treatments for Microbial Infections . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 45

Anthony W. Chow

Challenges in the Treatment of Infections . . . . 45

Microbiologic Diagnosis . . . . 46

New Antimicrobials and Pharmacologic Principles of

Antimicrobial Therapy . . . . 51

Emerging Antibiotic Resistance . . . . 55

ONCOLOGY

6.The Science and Value of Lymphoma Classification . . . . . . . . . . . . . . . . . . . . . . . . . . . . 65

Randy D. Gascoyne

Historical Perspective . . . . 65

Current Approach . . . .

71

Lymphomas of the Ocular Adnexa and Eye . . . . 72

Future Directions . . . .

74

7.Non-Hodgkin’s Lymphoma . . . . . . . . . . . . . . . . . 77

Joseph M. Connors

Incidence and Epidemiology

. . . . 77

Etiology and Risk Factors

. . . . 78

Diagnosis, Classification, and Staging . . . . 80

Treatment . . . . 80

Special Comments Lymphoma Associated

with the Eye . . . . 82

8. Epithelial Lacrimal Gland Tumors: Pathologic Classification and Current Understanding . . . 87

Thomas J. Joly, Jack Rootman, Kenneth W. Berean, Valerie A. White, Hind M. Al-Katan, Sylvia Pasternak, Guilio Bonavolonta, Diego Strianese, Peerooz Saeed, Kenneth A. Feldman, Sumalee Vangveeravong, and Jocelyne S. Lapointe

Contents

v

9.Chemotherapy of Epithelial Lesions: New Approaches and Directions . . . . . . . . . . . . . . . . 95

James H. Goldie

Abstract . . . . 95

 

 

 

Introduction . . . . 96

 

 

 

Steps Toward Tumor Specific Therapy . . . .

96

 

Cancer Specific Molecular Targets

. . . . 97

 

 

DNA Array Analysis . . . . 100

 

 

 

Which Molecular Targets? . . . .

101

 

 

What is the Fundamental ‘‘Cause’’ of Cancer?

. . . .

102

Bureaucratic Obstacles in Drug Development

. . . .

103

Conclusions . . . . 104

 

 

 

10.Malignant Lacrimal Gland Tumors . . . . . . . . . 107

George B. Bartley

11.The Management of Optic Nerve Sheath

and Sphenoid Wing Meningiomas . . . . . . . . . . 117

John S. Kennerdell, Kimberly P. Cockerham, Roger E. Turbin, and Joseph C. Maroon

Therapeutic Recommendations . . . . 120 Sphenoid Wing Meningiomas . . . . 123

12. Gliomas of the Anterior Visual Pathway . . . . 129

Jonathan J. Dutton

 

Clinical Characteristics . . . .

130

Presenting Signs and Symptoms

. . . . 131

Radiographic Imaging . . . . 131

Ultrasound . . . . 133

 

Histopathology . . . . 135

 

Treatment and Prognosis . . . .

136

13.Stereotactic Radiotherapy for Optic Nerve

and Meningeal Lesions . . . . . . . . . . . . . . . . . . . 139

Roy Ma

Background . . . . 139

Definitions . . . . 140

Methods of Stereotactic Radiation Therapy . . . . 140

Future Developments . . . . 146

Summary . . . . 146

vi

Contents

THYROID ORBITOPATHY

14.Pathogenesis of Graves’ Ophthalmopathy . . . 147

Colum A. Gorman

Abstract . . . . 147 Introduction . . . . 148

How are the Clinical Features of Graves’

Ophthalmopathy Evoked?

. . . .

148

Consequences of Swelling

 

 

in the Retrobulbar Space

. . . .

149

Consequences of Restricted Extraocular Muscle Motion . . . . 149

What Measurements Reflect the Underlying Pathology in Graves’ Ophthalmopathy? . . . . 150

Why do Gags Accumulate in the Orbit in Graves’ Disease? . . . . 150

Is Graves’ Ophthalmopathy Definitely an Autoimmune Disease? . . . . 151

Could Graves’ Orbitopathy Be an Infectious Disease? . . . . 152

15.Thyroid-Associated Ophthalmopathy: Principal Antigens and Significance of Corresponding Antibodies . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 157

Ian Epstein, Donald Smallman, Stephen Baker, Kate Lazier, Jack R. Wall, and Mario Salvi

Abstract . . . . 157

Introduction . . . . 159

Clinical Subjects and Methods . . . . 160

Results . . . . 162

Discussion . . . . 165

16.Phenotypic Attributes of Orbital Fibroblasts Underlie Their Susceptibility in Graves’

Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 169

Terry J. Smith

Complex Activities of Human Fibroblasts are Generally Underappreciated . . . . 170

Orbital Fibroblasts are Comprised of Discrete Subpopulations of Cells . . . . 171

Contents vii

Orbital Fibroblasts Synthesize High Levels of PGE2 When Treated with Proinflammatory

Cytokines . . . . 172

Proinflammatory Cytokines Enhance Hyaluronan Synthesis in Orbital Fibroblasts . . . . 173

Orbital Fibroblasts Display Cell-Surface CD40 and Respond to CD154 . . . . 175

Fibroblasts from Several Anatomic Regions Can Express

the Thyrotropin Receptor

. . . . 176

 

Conclusions . . . . 177

 

 

17. Somatostatin Analogs in Ophthalmic and Orbital

Disease: A Rationale for Their Use . . . . . . . . .

183

Steven E. Katz, Marko I. Klisovic, Martin Lubow, and

Dino D. Klisovic

 

 

Introduction . . . . 183

 

 

Intraocular Tissues . . . .

186

 

Extraocular Muscles and Orbital Fibrocytes . . . .

192

Choroid Plexus and Arachnoid Granulations . . . .

193

18.Predictors of Disease Severity in Thyroid-Related Orbitopathy . . . . . . . . . . . . . . . . . . . . . . . . . . . . 203

Peter J. Dolman, Jack Rootman, and Jugpal Arneja

Introduction . . . . 203 Methods . . . . 204 Results . . . . 205 Discussion . . . . 209

19. Prognostic Factors . . . . . . . . . . . . . . . . . . . . . .

213

Claudio Marcocci, Michele Marino`, Roberto Rocchi,

 

Barbara Mazzi, Francesca Menconi, Eugenia Morabito,

Aldo Pinchera, and Luigi Bartalena

 

20.Relationship Between Thyroid Disorders and Orbitopathy, Including Management

Factors . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 223

Wilmar M. Wiersinga

Graves’ Hyperthyroidism and Graves’ Ophthalmopathy: One or Two Disease Entities? . . . . 224

viii Contents

Genetic and Environmental Factors Involved in Phenotypic Appearance of Graves’ Disease . . . . 226

Influence of Thyroid Function on Graves’ Ophthalmopathy . . . . 227

Effect of Antithyroid Drugs, Thyroidectomy, and Radioactive Iodine on Graves’ Ophthalmopathy . . . . 228

Prevention of Graves’ Ophthalmopathy by Earlier Diagnosis and Treatment of Graves’ Hyperthyroidism? . . . . 231

21.Monitoring Activity of Graves’

Ophthalmopathy . . . . . . . . . . . . . . . . . . . . . . . . 237

Wilmar M. Wiersinga

Clinical Utility of Measuring Disease Activity in Graves’ Ophthalmopathy . . . . 237

Methods to Assess Disease Activity in Graves’

Ophthalmopathy . . . . 240

 

Clinical Activity Score

. . . . 240

 

Orbital Echography . . . .

241

 

Orbital Octreoscan

. . . .

242

 

Orbital Magnetic Resonance Imaging

. . . . 243

Urinary Glycosaminoglycans . . . .

244

Serum Cytokines

. . . .

245

 

Serum Thyroid Stimulating

 

 

Immunoglobulins . . . . 246

 

Conclusion . . . .

246

 

 

 

22.VISA Classification for Thyroid-Related Orbitopathy . . . . . . . . . . . . . . . . . . . . . . . . . . . . 253

Peter J. Dolman and Jack Rootman

Background . . . . 253

VISA Classification . . . . 254 Individual Section

Measurements . . . . 254 Discussion . . . . 258

Contents

ix

23. Current Medical Management

 

of Thyroid Orbitopathy . . . . . . . . . . . . . . . . . .

261

Claudio Marcocci, Michele Marino`, Roberto Rocchi,

 

Barbara Mazzi, Francesca Menconi, Eugenia Morabito,

Aldo Pinchera, and Luigi Bartalena

Introduction . . . . 261

Nonsevere Graves’ Ophthalmopathy . . . . 262

Severe Graves’ Ophthalmopathy . . . . 262

24.Surgical Paradigms in the Management of Thyroid Orbitopathy . . . . . . . . . . . . . . . . . . . . . . . . . . . . 273

Jack Rootman

Introduction . . . . 273

Surgical Intervention During Active Orbitopathy . . . . 275

Stable Orbitopathy . . . .

276

Eye Muscle Surgery . . . .

280

Lid Procedures . . . . 281

 

25. Graves’ Orbitopathy: New and Future

Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 285

Michael Kazim

Pathophysiology of the Disease . . . . 286

Medical Therapy . . . . 289

Improvements in Orbital Decompression

. . . . 293

Improvements in Eyelid Surgery . . . .

294

Strabismus Surgery . . . . 294

 

26.Management Controversies and Preferences in Thyroid Orbitopathy: Panel Discussion

and Question Period

. . . . . . . . . . . . . . . . . . . . 301

Jack Rootman and Frank Buffam

Panelists’ Presentations

. . . . 302

Question and Answer Session . . . . 310

VASCULAR INTERVENTION

27. A New Technique in Managing Distensible Venous

Vascular Malformations of the Orbit

. . . . . . . 321

Thomas R. Marotta, Douglas A. Graeb, and Jack Rootman

Introduction . . . . 321

 

Classification of Orbital Vascular Lesions

. . . . 322

x

 

Contents

Anatomy of Orbital Region Venous Structures

and Relationship with DVVMs . . . .

322

Clinical Manifestations of DVVMs . . . .

324

Investigation of DVVMs . . . .

325

 

Options for Treatment with Emphasis on New

Technique . . . . 330

 

 

Results and Complications of Treatment

. . . . 332

Future Considerations . . . .

332

 

Conclusion . . . . 333

 

 

28.Vascular Intervention: Current and Future Opportunities . . . . . . . . . . . . . . . . . . . . . . . . . . . 335

Alan A. McNab

 

 

 

 

Introduction . . . . 335

 

 

 

Carotid-Cavernous Sinus Fistulas . . . . 336

 

Arteriovenous Malformations . . . .

345

 

Distensible Venous Anomalies . . . .

345

 

Preoperative Embolization of Tumors

. . . . 347

Aneurysms . . . . 349

 

 

 

Future Directions

. . . .

349

 

 

THE FUTURE

 

 

 

 

29. The Future of Imaging in Orbital Disease

. . . 353

Wieslaw L. Nowinski

 

 

 

Abstract . . . . 353

 

 

 

Introduction . . . . 354

 

 

 

Technological Advancements . . . .

354

 

Human Body Models . . . . 361

 

 

Present Computer-Aided Surgery

 

 

and Simulation

. . . .

362

 

 

Future Computer-Aided Orbital Surgery . . . .

364

Summary . . . .

366

 

 

 

30.The Future of Orbital Surgery . . . . . . . . . . . . . 371

Robert Alan Goldberg

Index . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 381