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Rhinology and Allergy: Clinical Reference Guide [1st ed.]
 9781597569651

Table of contents :
Contents......Page 6
Introduction......Page 10
Foreword......Page 12
About the Authors......Page 14
Acknowledgments......Page 16
Contributors......Page 18
Section I. History of Rhinology......Page 30
Chapter 1. History of Rhinology......Page 32
Section II. Embryology, Anatomy, and Physiology......Page 38
Chapter 2. Sinonasal Development and Anatomy......Page 40
Chapter 3. Sinonasal Physiology......Page 62
Chapter 4. Olfaction and Taste......Page 66
Section III. Evaluation and Diagnosis......Page 80
Chapter 5. Rhinologic History and Physical Examination......Page 82
Chapter 6. History Taking: Evaluation of Allergic Disorders......Page 90
Chapter 7. Laboratory Tests for the Diagnosis of Allergy......Page 98
Chapter 8. Objective Measures of Nasal Function......Page 108
Chapter 9. Radiologic Imaging of the Paranasal Sinuses and Skull Base......Page 118
Chapter 10. Diagnosis and Classification of Rhinosinusitis......Page 134
Section IV. Sinonasal Diseases......Page 146
Chapter 11. Epistaxis and Its Management......Page 148
Chapter 12. Allergic and Nonallergic Rhinitis......Page 162
Chapter 13. Diseases of the Nasal Cavity......Page 182
Chapter 14. Pathophysiology of Chronic Rhinosinusitis......Page 208
Chapter 15. Acute Rhinosinusitis......Page 220
Chapter 16. Management of Chronic Rhinosinusitis......Page 232
Chapter 17. Fungal Sinusitis......Page 248
Chapter 18. Pediatric Rhinosinusitis......Page 264
Chapter 19. Complications of Rhinosinusitis......Page 272
Chapter 20. Sinonasal Manifestations of Systemic Diseases......Page 282
Section V. Surgical Management of Sinonasal Disease......Page 300
Chapter 21. In-Office Rhinology Techniques......Page 302
Chapter 22. Surgery of the Septum and Turbinate......Page 308
Chapter 23. Endoscopic Sinus Surgery: Concepts, Surgical Indications, and Techniques......Page 314
Chapter 24. Complications of Endoscopic Sinus Surgery......Page 328
Chapter 25. Benign Sinonasal Tumors......Page 348
Chapter 26. Malignant Sinonasal Tumors......Page 366
Chapter 27. External Approach to the Paranasal Sinuses......Page 374
Chapter 28. Dacrycystorhinostomy......Page 394
Section VI. Allergy......Page 402
Chapter 29. Basics of Allergy......Page 404
Chapter 30. Immunology of Allergic Disease......Page 418
Chapter 31. The United Airway......Page 424
Chapter 32. Principles of Allergy Skin Testing......Page 430
Chapter 33. Allergen Immunotherapy: Subcutaneous and Sublingual......Page 438
Chapter 34. Urticaria and Angioedema......Page 446
Section VII. Surgical Management of Skull Base Disorders......Page 460
Chapter 35. The Nasopharynx......Page 462
Chapter 36. Endoscopic Anatomy of the Skull Base and Parasellar Region......Page 474
Chapter 37. Cerebrospinal Fluid Leaks and Encephaloceles......Page 484
Chapter 38. Pathology and Endoscopic Approaches to the Anterior Cranial Fossa......Page 492
Chapter 39. Pathology and Endoscopic Endonasal Approaches to the Sella and Suprasellar Regions......Page 500
Chapter 40. Pathology and Endoscopic Approaches to the Clivus and Posterior Fossa......Page 512
Chapter 41. Endoscopic Skull Base Reconstruction......Page 534
Chapter 42. Complications of Endoscopic Skull Base Surgery......Page 552
Index......Page 562

Citation preview

Rhinology and Allergy Clinical Reference Guide

Rhinology and Allergy Clinical Reference Guide

Brent A. Senior, MD, FACS, FARS Yvonne Chan, MD, FRCSC, MSc, HBSc

5521 Ruffin Road San Diego, CA 92123 e-mail: [email protected] website: http://www.pluralpublishing.com Copyright 2018 © by Plural Publishing, Inc. Typeset in 9/11 Minion Pro by Flanagan’s Publishing Services, Inc. Printed in the United States of America by McNaughton & Gunn, Inc. All rights, including that of translation, reserved. No part of this publication may be reproduced, stored in a retrieval system, or transmitted in any form or by any means, electronic, mechanical, recording, or otherwise, including photocopying, recording, taping, Web distribution, or information storage and retrieval systems without the prior written consent of the publisher. For permission to use material from this text, contact us by Telephone:  (866) 758-7251 Fax:  (888) 758-7255 e-mail: [email protected] Every attempt has been made to contact the copyright holders for material originally printed in another source. If any have been inadvertently overlooked, the publishers will gladly make the necessary arrangements at the first opportunity. NOTICE TO THE READER Care has been taken to confirm the accuracy of the indications, procedures, drug dosages, and diagnosis and remediation protocols presented in this book and to ensure that they conform to the practices of the general medical and health services communities. However, the authors, editors, and publisher are not responsible for errors or omissions or for any consequences from application of the information in this book and make no warranty, expressed or implied, with respect to the currency, completeness, or accuracy of the contents of the publication. The diagnostic and remediation protocols and the medications described do not necessarily have specific approval by the Food and Drug administration for use in the disorders and/or diseases and dosages for which they are recommended. Application of this information in a particular situation remains the professional responsibility of the practitioner. Because standards of practice and usage change, it is the responsibility of the practitioner to keep abreast of revised recommendations, dosages, and procedures. Library of Congress Cataloging-in-Publication Data Names: Senior, Brent A., editor. | Chan, Yvonne, 1973- editor. Title: Rhinology and allergy : clinical reference guide / [edited by] Brent A. Senior, Yvonne Chan. Description: San Diego, CA : LOGO Plural Publishing, [2018] | Includes bibliographical references and index. Identifiers: LCCN 2017058135| ISBN 9781597569651 (alk. paper) | ISBN 1597569658 (alk. paper) Subjects: | MESH: Nose Diseases | Hypersensitivity | Otorhinolaryngologic Surgical Procedures Classification: LCC RF341 | NLM WV 300 | DDC 617.5/23—dc23 LC record available at https://lccn.loc.gov/2017058135

Contents Introduction ix Foreword by David W. Kennedy, MD, FACS, FRCSI xi About the Authors xiii Acknowledgments xv Contributors xvii Section I

History of Rhinology

1

Chapter 1

History of Rhinology Gordon F.Z. Tsang and Yvonne Chan

3

Section II

Embryology, Anatomy, and Physiology

9

Chapter 2

Sinonasal Development and Anatomy Joseph S. Schwartz and Nithin D. Adappa

11

Chapter 3

Sinonasal Physiology Kristian I. Macdonald

33

Chapter 4

Olfaction and Taste Jasper Shen, Kevin Hur, and Bozena B. Wrobel

37

Section III Evaluation and Diagnosis

51

Chapter 5 Rhinologic History and Physical Examination Maheep Sohal and Seth M. Brown

53

Chapter 6

History Taking:  Evaluation of Allergic Disorders Aimee A. Kennedy and Fuad M. Baroody

61

Chapter 7

Laboratory Tests for the Diagnosis of Allergy Elizabeth Bradford Bell and Sarah K. Wise

69

Chapter 8

Objective Measures of Nasal Function Kevin P. Casey and David M. Poetker

79

Chapter 9

Radiologic Imaging of the Paranasal Sinuses and Skull Base Richard Douglas and Tary Yin

89

Chapter 10 Diagnosis and Classification of Rhinosinusitis Abtin Tabaee and Edward D. McCoul v

105

vi   Rhinology and Allergy:  Clinical Reference Guide

Section IV Sinonasal Diseases

117

Chapter 11 Epistaxis and Its Management Garret W. Choby and Zara M. Patel

119

Chapter 12 Allergic and Nonallergic Rhinitis Ashleigh A. Halderman

133

Chapter 13 Diseases of the Nasal Cavity Bobby A. Tajudeen and Pete S. Batra

153

Chapter 14 Pathophysiology of Chronic Rhinosinusitis Patricia A. Loftus, Andrew N. Goldberg, and Steven D. Pletcher

179

Chapter 15 Acute Rhinosinusitis Wirach Chitsuthipakorn and Kornkiat Snidvongs

191

Chapter 16 Management of Chronic Rhinosinusitis Kristine A. Smith and Luke Rudmik

203

Chapter 17 Fungal Sinusitis Patrick Colley and Charles S. Ebert, Jr.

219

Chapter 18 Pediatric Rhinosinusitis Julian A. Vellucci and Sanjay R. Parikh

235

Chapter 19 Complications of Rhinosinusitis Edward C. Kuan and Jivianne T. Lee

243

Chapter 20 Sinonasal Manifestations of Systemic Diseases Jonathan Yip and Eric Monteiro

253

Section V

271

Surgical Management of Sinonasal Disease

Chapter 21 In-Office Rhinology Techniques Amber U. Luong and Martin J. Citardi

273

Chapter 22 Surgery of the Septum and Turbinate Craig Miller and Greg E. Davis

279

Chapter 23 Endoscopic Sinus Surgery:  Concepts, Surgical Indications, and Techniques Javier Ospina and Arif Janjua

285

Chapter 24 Complications of Endoscopic Sinus Surgery Saba Ghorab and Devyani Lal

299

Chapter 25 Benign Sinonasal Tumors Zhong Zheng, Jay Agarwal, and Anthony G. Del Signore

319

CONTENTS

vii

Chapter 26 Malignant Sinonasal Tumors Luis Macias-Valle and Alkis James Psaltis

337

Chapter 27 External Approach to the Paranasal Sinuses Abdullah AlBader and Roy R. Casiano

345

Chapter 28 Dacrycystorhinostomy Adam P. Campbell and Benjamin S. Bleier

365

Section VI Allergy

373

Chapter 29 Basics of Allergy Esther Kim and Thomas G. Townes

375

Chapter 30 Immunology of Allergic Disease A. Simon Carney and Peter K. Smith

389

Chapter 31 The United Airway Taha A. Mur and Elina Toskala

395

Chapter 32 Principles of Allergy Skin Testing Christopher R. Roxbury, Nyall R. London, Jr., and Sandra Y. Lin

401

Chapter 33 Allergen Immunotherapy: Subcutaneous and Sublingual Andrea M. Hebert and Stella E. Lee

409

Chapter 34 Urticaria and Angioedema Nsangou Ghogomu, Robert Kern, and Anju T. Peters

417

Section VII Surgical Management of Skull Base Disorders

431

Chapter 35 The Nasopharynx Peter F. Svider, Sean Mutchnick, and Adam J. Folbe

433

Chapter 36 Endoscopic Anatomy of the Skull Base and Parasellar Region Wayne D. Hsueh, Michael Pfisterer, Suat Kilic, James K. Liu, and Jean Anderson Eloy

445

Chapter 37 Cerebrospinal Fluid Leaks and Encephaloceles Tze Choong Charn and Marc A. Tewfik

455

Chapter 38 Pathology and Endoscopic Approaches to the Anterior Cranial Fossa Christina H. Fang and Waleed M. Abuzeid

463

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Chapter 39 Pathology and Endoscopic Endonasal Approaches 471 to the Sella and Suprasellar Regions Sarah E. Hodge, Satyan Belur Sreenath, Charles S. Ebert, Jr., Adam M. Zanation, and Brian D. Thorp Chapter 40 Pathology and Endoscopic Approaches to the Clivus and Posterior Fossa Jaron Densky, Ricardo L. Carrau, Daniel M. Prevedello, and Alexander Farag

483

Chapter 41 Endoscopic Skull Base Reconstruction Elisabeth H. Ference and Jeffrey D. Suh

505

Chapter 42 Complications of Endoscopic Skull Base Surgery Nsangou Ghogomu and Kevin Welch

523

Index 533

Introduction “Surgery will not only develop new and previously impossible procedures, but all uncertain operations which depended on luck and approximation will become safe under the influence of direct vision, since the surgeon’s hand will now be guided by his eyes.” —Philipp Bozzini, 1806 In 1806, Philipp Bozzini, a physician living in Germany developed what most historians acknowledge to be the first usable endoscope. Termed the “Lichtleiter,” the device included a rudimentary self-contained lightsource allowing the user to peer into previously poorly seen orifices including the urethra, cervix, anus, mouth, and nose. With his quote above, now over two centuries later, Bozzini would undoubtedly be pleased to see how his invention has transformed the way we understand the nose, sinuses, and skull base, and how we perform surgery these structures to improve their function, eradicate tumors, and repair their defects. This book is an attempt to distill the explosion of information about rhinology, allergy and skull base disorders that has occurred over the last 30 years since the modern endoscope was introduced to the rhinology community by the likes of Walter Messerklinger, Heinz Stammberger, and David Kennedy. As a review book, it is ideal for residents in training, and those preparing for board and in-service training examinations as well as medical students looking for a “deeper dive” into the world of rhinology. However, it is also excellent for the practitioner who wants to get a succinct update on the latest, cutting-edge knowledge in rhinology, allergy, endoscopic skull base surgery. The information is authored by a collection of the “who’s who” of modern rhinology, outstanding contributions that are dense and presented in bullet form and best digested slowly lest critical information be missed. In its 42 chapters, you will find all aspects of rhinology reviewed, including medical and surgical management of inflammatory and allergic disease of the nose, sinuses, and skull base, as well as a review of neoplasms, both benign and malignant. We hope that you enjoy this book and we hope Dr. Bozzini would be proud!

ix

Foreword The field of knowledge within rhinology has expanded exponentially in recent years and the expectations for trainees within the field has ballooned accordingly. Rhinology and Allergy: Clinical Reference Guide is a succinct bullet pointed text edited by two internationally renowned rhinologists, with chapters authored by both rhinologists and allergists, each of whom are leaders in the field. The goal of the text is to provide a broad but brief reference text covering these subspecialties. The book is aimed at residents and fellows in training, especially those preparing for the boards, and the chapters provide all of the necessary information in a crisp format. It will also be of benefit to practitioners looking for a brief text to update their reference knowledge with comprehensive factual information within the field. The text is divided into sections such as evaluation and diagnosis, sinonasal diseases, surgical management, allergy, and skull base surgery, with multiple brief chapters in each section. The incorporation of junior co-authors helps to ensure that the text includes the material most relevant for board preparation. In addition to providing comprehensive factual information, a number of the chapters are beautifully illustrated, providing excellent visual clarity to the bullet pointed notes. Overall, this text provides an excellent review of the most salient current knowledge within the fields of rhinology, allergy and skull base surgery. As noted above, it lays out the important facts within each of the areas, providing the information necessary for a resident or fellow preparing for the Boards, or a practitioner needing to update his or her background knowledge. It is often not easy to keep the text in a book like this succinct and to the point, but the editors have done an excellent job in this book. —David W. Kennedy, M.D., FACS, FRCSI Rhinology Professor Perelman School of Medicine University of Pennsylvania

xi

About the Authors

Brent A. Senior, MD, FACS, FARS, is Nat and Sheila Harris Professor of Otolaryngology and Neurosurgery at the University of North Carolina at Chapel Hill, where he also serves as vice chair of Clinical Affairs and chief of Rhinology, Allergy, and Endoscopic Skull Base Surgery. Dr. Senior has lectured and instructed in nearly 120 national and international rhinology courses and authored 120 articles and chapters in the field. In 2005, he coedited the very successful text The Frontal Sinus, now in its second edition. Dr. Senior’s passion for education has been honored with his receipt of the “Cottle Award” from the American Rhinologic Society, the highest award for education from that society. His humanitarian efforts garnered him a “Humanitarian of the Year” Award from the American Academy of Otolaryngology in 2005. He served as president of the American Rhinologic Society in 2010. He currently serves as associate editor of the International Forum of Allergy and Rhinology, president of the Christian Society of Otolaryngology, and president-elect of the International Rhinologic Society, and he sits on the Board of Directors of the American Academy of Otolaryngology/ Head and Neck Surgery and the International Rhinologic Society.

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xiv   Rhinology and Allergy:  Clinical Reference Guide

Yvonne Chan, MD, FRCSC, MSc, HBSc, is an Assistant Professor and the Continuing Professional Development Director in the Department of Otolaryngology-Head & Neck Surgery at the University of Toronto. She is the division head of Otolaryngology at Trillium Health Partners. She is the vice chair of the Canadian Rhinology Working Group. At the Mississauga Academy of Medicine, she is the undergraduate medical lead for Otolaryngology. She finished her otolaryngology residency training at the University of Toronto and subsequently completed a rhinology fellowship at the Georgia Nasal and Sinus Institute. Dr. Chan obtained her medical degree and an MS degree in molecular biology also from the University of Toronto. Since 2015, she has been the coeditor of the world-renowned otolaryngology textbook, K. J. Lee’s Essential Otolaryngology (11th edition). Other texts that she has coedited include Health Care Reform Through Practical Clinical Guidelines and Essential Paths to Life After Residency.

Acknowledgments I would like to thank the many authors who participated in this project and contributed so admirably. I would also like to thank my mentors Drs. David Kennedy and Fred Kuhn without whose guidance and inspiration, rhinology may not have been my chosen field of study. And finally, I would like to thank my long-suffering wife, Dana, and my wonderful children for their interminable support despite long workfilled nights. You are amazing and you are loved! Sole Deo Gloria. —Brent A. Senior I echo Dr. Senior’s gratitude to all the authors who volunteered their time and expertise to help make this book possible. I would like to thank Dr. Senior for his guidance and tremendous input in shaping the contents of this monograph and his eagle eye in the editing process. I would like to thank my mentor Dr. Fred Kuhn for teaching me to “never stop looking and to never stop thinking about what you are looking at.” I would also like to thank my mentor Dr. K. J. Lee for his support and belief in me. Last but not least, I would like to thank my family, especially my parents without their love and support, I would not be who I am today. —Yvonne Chan

xv

Contributors Waleed M. Abuzeid, MD Assistant Professor Director, Rhinology and Skull Base Surgery Department of Otorhinolaryngology Montefiore Medical Center Albert Einstein College of Medicine New York, New York Chapter 38 Nithin D. Adappa, MD Assistant Professor Division of Rhinology and Skull Base Surgery Department of Otorhinolaryngology–Head and Neck Surgery University of Pennsylvania Philadelphia, Pennsylvania Chapter 2 Jay Agarwal, MD Department of Otolaryngology– Head and Neck Surgery New York Eye and Ear Infirmary of Mount Sinai New York, New York Chapter 25 Abdullah AlBader, MD, FRCSC Rhinology–Endoscopic Skull Base Surgery Fellow Department of Otolaryngology– Head and Neck Surgery University of Miami Miller School of Medicine Miami, Florida Chapter 27

Fuad M. Baroody, MD, FACS Professor of Surgery (Otolaryngology–Head and Neck Surgery) and Pediatrics Director of Pediatric Otolaryngology Residency Program Director The University of Chicago Medicine The Comer Children’s Hospital Chicago, Illinois Chapter 6 Pete S. Batra, MD, FACS Stanton A. Friedberg, MD Chair in Otolaryngology Professor and Chairman Co-Director, Rush Center for Skull Base and Pituitary Surgery Co-Director, Rush Sinus Program Chicago, Illinois Chapter 13 Elizabeth Bradford Bell, MD Resident Otolaryngology–Head and Neck Surgery Emory University Atlanta, Georgia Chapter 7 Jason I. Blaichman, MD, CM, FRCPC Clinical Instructor Department of Radiology University of British Columbia Vancouver, Canada Chapter 2 Illustrator xvii

xviii   Rhinology and Allergy:  Clinical Reference Guide

Benjamin S. Bleier, MD Assistant Professor Department of Otolaryngology Massachusetts Eye and Ear Infirmary Harvard Medical School Boston, Massachusetts Chapter 28 Seth M. Brown, MD, MBA, FACS Clinical Assistant Professor of Surgery Division of Otolaryngology University of Connecticut School of Medicine Farmington, Connecticut Chapter 5 Adam P. Campbell, MD Clinical Fellow in Rhinology Department of Otolaryngology Massachusetts Eye and Ear Infirmary Harvard Medical School Boston, Massachusetts Chapter 28 A. Simon Carney, FRACS Professor of Otolaryngology– Head and Neck Surgery Flinders University Southern ENT & Adelaide Sinus Centre Flinders Private Hospital South Australia, Australia Chapter 30 Ricardo L. Carrau, MD, FACS Professor and Lynne Shepard Jones Chair in Head and Neck Oncology Department of Otolaryngology– Head and Neck Surgery Director, Comprehensive Skull Base Surgery Program

The Ohio State University Wexner Medical Center Co-Director, Anatomy Laboratory Toward Visuospatial Surgical Innovations in Otolaryngology and Neurosurgery (ALT-VISION) Columbus, Ohio Chapter 40 Kevin P. Casey, MD Department of Otolaryngology Medical College of Wisconsin Milwaukee, Wisconsin Chapter 8 Roy R. Casiano, MD, FACS Professor and Vice Chairman Director, Rhinology and Endoscopic Skull Base Program Department of Otolaryngology University of Miami Miller School of Medicine Miami, Florida Chapter 27 Yvonne Chan, MD, FRCSC, MSc, HBSc Division Head, Otolaryngology– Head and Neck Surgery, Trillium Health Partners Continuing Education and Professional Development Director, Department of Otolaryngology–Head and Neck Surgery Assistant Professor, University of Toronto Toronto, Canada Chapter 1 and Editor Tze Choong Charn, MBBS, MRCS, MMed, DOHNS FEBEORL Associate Consultant Department of Otolaryngology

CONTRIBUTORS

Sengkang Health and Singapore General Hospital Bukit Merah, Singapore Chapter 37 Wirach Chitsuthipakorn, MD Consultant, Rhinologist Department of Otolaryngology Sawan Pracharak Hospital Nakhon Sawan, Thailand Chapter 15 Garret W. Choby, MD Assistant Professor Rhinology and Endoscopic Skull Base Surgery Department of Otorhinolaryngology–Head and Neck Surgery Mayo Clinic School of Medicine Rochester, Minnesota Chapter 11 Martin J. Citardi, MD Professor and Chair Department of Otorhinolaryngology–Head and Neck Surgery McGovern Medical School University of Texas Health Science Center at Houston Houston, Texas Chapter 21 Patrick Colley, MD Assistant Professor Rhinology and Skull Base Surgery Department of Otolaryngology– Head and Neck Surgery Icahn School of Medicine at Mount Sinai New York, New York Chapter 17 Greg E. Davis, MD, MPH Director of Rhinology and Endoscopic Skull Base

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Associate Professor Department of Otolaryngology– Head and Neck Surgery University of Washington Seattle, Washington Chapter 22 Anthony G. Del Signore, MD, PharmD Assistant Professor Director of Rhinology and Endoscopic Skull Base Surgery Mount Sinai Beth Israel Department of Otolaryngology– Head and Neck Surgery New York, New York Chapter 25 Jaron Densky, MD Department of Otolaryngology– Head and Neck Surgery Ohio State University Columbus, Ohio Chapter 40 Richard Douglas, MD, FRACS, FRACP Associate Professor Department of Surgery The University of Auckland Auckland, New Zealand Chapter 9 Charles S. Ebert, Jr., MD, MPH, FACS, FAAOA Associate Professor University of North Carolina Department of Otolaryngology– Head and Neck Surgery Division of Rhinology, Allergy, and Endoscopic Skull Base Surgery Chapel Hill, North Carolina Chapters 17 and 39

xx   Rhinology and Allergy:  Clinical Reference Guide

Jean Anderson Eloy, MD, FACS Professor and Vice Chair Director, Rhinology and Sinus Surgery Director, Otolaryngology Research Co-Director, Endoscopic Skull Base Surgery Program Director of Rhinology, Sinus, and Endoscopic Skull Base Surgery Fellowship Program Department of Otolaryngology– Head and Neck Surgery Professor of Neurological Surgery Professor of Ophthalmology and Visual Science Neurological Institute of New Jersey Rutgers New Jersey Medical School Newark, New Jersey Chapter 36 Christina H. Fang, MD Resident Department of Otorhinolaryngology–Head and Neck Surgery Albert Einstein College of Medicine Bronx, New York Chapter 38 Alexander Farag, MD Assistant Professor–Clinical Department of Otolaryngology– Head and Neck Surgery The Ohio State University Wexner Medical Center Columbus, Ohio Chapter 40 Elisabeth H. Ference, MD, MPH Clinical Assistant Professor

USC Rick and Tina Caruso Department of Otolaryngology–Head and Neck Surgery Keck School of Medicine University of Southern California Los Angeles, California Chapter 41 Adam J. Folbe, MD, MS, FARS, FACS Academic Vice-Chair Division of Otolaryngology– Head and Neck Surgery Rhinology and Endoscopic Skull Base Surgery William Beaumont Hospital Royal Oak, Michigan Chapter 35 Nsangou Ghogomu, MD, MA Fellow Northwestern University Feinberg School of Medicine Chicago, Illinois Chapters 34 and 42 Saba Ghorab, MD Resident Department of Otolaryngology– Head and Neck Surgery Mayo Clinic Phoenix, Arizona Chapter 24 Andrew N. Goldberg, MD, MSCE, FACS Boles Professor and Vice Chair Director, Division of Rhinology and Sinus Surgery Department of Otolaryngology– Head and Neck Surgery and Neurological Surgery University of California, San Francisco San Francisco, California Chapter 14

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CONTRIBUTORS

Ashleigh A. Halderman, MD Assistant Professor Rhinology and Skull Base Surgery Department of Otolaryngology– Head and Neck Surgery UT Southwestern Dallas, Texas Chapter 12 Andrea M. Hebert, MD, MPH Assistant Professor Department of Otorhinolaryngology–Head and Neck Surgery University of Maryland School of Medicine Baltimore, Maryland Chapter 33 Sarah E. Hodge, MD Department of Otolaryngology– Head and Neck Surgery University of North Carolina at Chapel Hill Chapel Hill, North Carolina Chapter 39 Wayne D. Hsueh, MD Resident Physician Department of Otorhinolaryngology–Head and Neck Surgery Montefiore Medical Center Albert Einstein College of Medicine Bronx, New York Chapter 36 Kevin Hur, MD Caruso Department of Otolaryngology–Head and Neck Surgery Keck School of Medicine University of Southern California

Los Angeles, California Chapter 4 Arif Janjua, MD, FRCSC Clinical Assistant Professor Rhinology, Endoscopic Sinus and Skull Base Surgery Division of Otolaryngology– Head and Neck Surgery Vancouver General Hospital and St. Paul’s Hospital University of British Columbia Vancouver, Canada Chapter 23 Aimee A. Kennedy, MD Section of Otolaryngology–Head and Neck Surgery University of Chicago Medicine and Biological Sciences Chicago, Illinois Chapter 6 Robert Kern, MD Professor of Otolaryngology Northwestern University Feinberg School of Medicine Chicago, Illinois Chapter 34 Suat Kilic, BA Rutgers New Jersey Medical School Department of Otolaryngology– Head and Neck Surgery Newark, New Jersey Chapter 36 Esther Kim, MD Assistant Professor Department of Otolaryngology– Head and Neck Surgery Walter Reed National Military Medical Center Bethesda, Maryland Chapter 29

xxii   Rhinology and Allergy:  Clinical Reference Guide

Edward C. Kuan, MD, MBA Resident Physician University of California, Los Angeles (UCLA) Medical Center Los Angeles, California Chapter 19 Devyani Lal, MD Assistant Professor Mayo Clinic College of Medicine Consultant, Department of Otolaryngology–Head and Neck Surgery Mayo Clinic Phoenix, Arizona Chapter 24 Jivianne T. Lee, MD Department of Otolaryngology– Head and Neck Surgery David Geffen School of Medicine University of California, Los Angeles Los Angeles, California Chapter 19 Stella E. Lee, MD Director of Sinonasal Disorders and Allergy University of Pittsburgh Medical Center Pittsburgh, Pennsylvania Chapter 33 Sandra Y. Lin, MD Professor and Clinical Vice Director Department of Otolaryngology– Head and Neck Surgery Division of Rhinology Johns Hopkins University Baltimore, Maryland Chapter 32

James K. Liu, MD, FACS Associate Professor of Neurological Surgery Associate Professor of Otolaryngology–Head and Neck Surgery Director, Center for Skull Base and Pituitary Surgery Department of Neurological Surgery Neurological Institute of New Jersey Rutgers New Jersey Medical School Newark, New Jersey Chapter 36 Patricia A. Loftus, MD Assistant Professor Division of Rhinology and Skull Base Surgery Department of Otolaryngology– Head and Neck Surgery University of California, San Francisco San Francisco, California Chapter 14 Nyall R. London, Jr., MD, PhD Resident Department of Otolaryngology– Head and Neck Surgery Johns Hopkins University Baltimore, Maryland Chapter 32 Amber U. Luong, MD, PhD Associate Professor and Director of Research Department of Otorhinolaryngology McGovern Medical School University of Texas Health Science Center

CONTRIBUTORS

Houston, Texas Chapter 21 Kristian I. Macdonald, MD, MSc, FRCSC Assistant Professor Department of Otolaryngology– Head and Neck Surgery University of Ottawa Ottawa, Canada Chapter 3 Luis Macias-Valle, MD Rhinology and Endoscopic Skull Base Surgery Fellow Department of Otolaryngology– Head and Neck Surgery The Queen Elizabeth Hospital The University of Adelaide South Australia, Australia Chapter 26 Edward D. McCoul, MD, MPH Associate Professor Department of Otorhinolaryngology Ochsner Clinic Foundation New Orleans, Louisiana Chapter 10 Craig Miller, MD Resident Physician Department of Otolaryngology– Head and Neck Surgery University of Washington Seattle, Washington Chapter 22 Eric Monteiro, MD, MSc, FRCSC Assistant Professor Sinai Health System University of Toronto Toronto, Canada Chapter 20

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Taha A. Mur, MD Resident Otolaryngology–Head and Neck Surgery Boston Medical Center Boston, Massachusetts Chapter 31 Sean Mutchnick, MD Resident Physician Department of Otolaryngology– Head and Neck Surgery Wayne State University School of Medicine Detroit, Michigan Chapter 35 Javier Ospina, MD Fellowship in Rhinology and Skull Base Surgery University of British Columbia Otolaryngologist Javeriana University Vancouver, Canada Chapter 23 Sanjay R. Parikh, MD, FACS Professor of Otolaryngology– Head and Neck Surgery University of Washington Medical Director, Bellevue Clinic and Surgery Center Seattle Children’s Hospital Seattle, Washington Chapter 18 Zara M. Patel, MD Assistant Professor Rhinology–Endoscopic Skull Base Surgery Department of Otolaryngology– Head and Neck Surgery Stanford University School of Medicine Stanford, California Chapter 11

xxiv   Rhinology and Allergy:  Clinical Reference Guide

Anju T. Peters, MD Director of Clinical Research Division of Allergy-Immunology Professor of Medicine Feinberg School of Medicine Northwestern University Chicago, Illinois Chapter 34 Michael Pfisterer, MD Rhinology, Sinus and Skull Base Fellow Department of Otolaryngology– Head and Neck Surgery Rutgers New Jersey Medical School Newark, New Jersey Chapter 36 Steven D. Pletcher, MD Associate Professor Department of Otolaryngology– Head and Neck Surgery University of California, San Francisco San Francisco, California Chapter 14 David M. Poetker, MD, MA Associate Professor Zablocki VAMC, Division of Surgery Department of Otolaryngology Medical College of Wisconsin Milwaukee, Wisconsin Chapter 8 Daniel M. Prevedello, MD, FACS Professor Department of Neurological Surgery ENT–Head and Neck Surgery Department The Ohio State University Columbus, Ohio Chapter 40

Alkis James Psaltis, MBBS(hons), PhD, FRACS Associate Professor Department of Surgery University of Adelaide Head of Department, Otolaryngology–Head and Neck Surgery Queen Elizabeth Hospital South Australia, Australia Chapter 26 Christopher R. Roxbury, MD Resident Department of Otolaryngology– Head and Neck Surgery Johns Hopkins University Baltimore, Maryland Chapter 32 Luke Rudmik, MD, MSC, FRCSC Department of Surgery Division of Otolaryngology University of Calgary Calgary, Canada Chapter 16 Joseph S. Schwartz, MD, FRCSC Assistant Professor Rhinology and Skull Base Surgery Department of Otolaryngology– Head and Neck Surgery Jewish General Hospital McGill University Health Center Montreal, Canada Chapter 2 Brent A. Senior, MD, FACS, FARS Professor of Otolaryngology/ Neurosurgery Vice Chairman for Clinical Affairs

CONTRIBUTORS

Chief, Division of Rhinology, Allergy, and Endoscopic Skull Base Surgery Department of Otolaryngology– Head & Neck Surgery University of North Carolina School of Medicine Chapel Hill, North Carolina Editor Jasper Shen, MD Academic Rhinology Fellow Department of Otolaryngology– Head and Neck Surgery Northwestern Medicine Chicago, Illinois Chapter 4 Kristine A. Smith, MD, FRCSC Department of Surgery Division of Otolaryngology University of Calgary Calgary, Canada Chapter 16 Peter K. Smith, MBBS, BMedSci, FRACP, PhD Professor Clinical Medicine Griffith University Queensland, Australia Chapter 30 Kornkiat Snidvongs, MD, PhD Faculty of Medicine Department of Otolaryngology Chulalongkorn University Bangkok, Thailand Chapter 15 Maheep Sohal, MD Resident Division of Otolaryngology University of Connecticut Storrs, Connecticut Chapter 5

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Satyan Belur Sreenath, MD Department of Otolaryngology– Head and Neck Surgery University of North Carolina at Chapel Hill Chapel Hill, North Carolina Chapter 39 Jeffrey D. Suh, MD Associate Professor Department of Head and Neck Surgery University of California, Los Angeles School of Medicine Los Angeles, California Chapter 41 Peter F. Svider, MD Resident Physician Department of Otolaryngology– Head and Neck Surgery Wayne State University School of Medicine Detroit, Michigan Chapter 35 Abtin Tabaee, MD Associate Professor Department of Otolaryngology Weill Cornell Medicine New York Presbyterian Hospital New York, New York Chapter 10 Bobby A. Tajudeen, MD Assistant Professor Co-Director, Rush Sinus Program Director, Otorhinolaryngology Research Department of Otorhinolaryngology–Head and Neck Surgery Rush University Medical Center Chicago, Illinois Chapter 13

xxvi   Rhinology and Allergy:  Clinical Reference Guide

Marc A. Tewfik, MDCM, MSc, FRCSC Assistant Professor of Otolaryngology–Head and Neck Surgery McGill University Health Centre Sir Mortimer B. Davis–Jewish General Hospital Montreal, Canada Chapter 37 Brian D. Thorp, MD Assistant Professor Department of Otolaryngology– Head and Neck Surgery University of North Carolina– Chapel Hill Chapel Hill, North Carolina Chapter 39 Elina Toskala, MD, PhD, MBA Professor Chief of Otolaryngology–Head and Neck Surgery Director of Allergy Temple Head and Neck Institute Temple University Philadelphia, Pennsylvania Chapter 31 Thomas G. Townes, MD Resident Physician Otolaryngology–Head and Neck Surgery Walter Reed National Military Medical Center Rockville, Maryland Chapter 29 Gordon F.Z. Tsang, MD, FRCSC Otology/Neurotology Fellow University Health Network Department of Otolaryngology– Head and Neck Surgery University of Toronto

Toronto, Canada Chapter 1 Julian A. Vellucci, MD Department of Surgery, Head and Neck Surgery Madigan Army Medical Center Tacoma, Washington Chapter 18 Kevin Welch, MD Associate Professor of Otolaryngology Northwestern University Feinberg School of Medicine Chicago, Illinois Chapter 42 Sarah K. Wise, MD, MSCR Associate Professor Director of Otolaryngic Allergy Department of Otolaryngology– Head and Neck Surgery Emory University Atlanta, Georgia Chapter 7 Bozena B. Wrobel, MD Director, USC Rhinology and Endoscopic Skull Base Surgery USC Caruso Department of Otolaryngology–Head and Neck Surgery University of Southern California Los Angeles, California Chapter 4 Tary Yin, MBChB, BMedSc(Hons) University of Auckland Auckland, New Zealand Chapter 9 Jonathan Yip, MD Resident Department of Otolaryngology– Head and Neck Surgery

CONTRIBUTORS

University of Toronto Toronto, Canada Chapter 20 Adam M. Zanation, MD, FACS Harold C. Pillsbury Distinguished Professor Vice Chairman for Academic Affairs Director of Practice Development Associate Professor of Otolaryngology and Neurosurgery

xxvii

University of North Carolina School of Medicine Chapel Hill, North Carolina Chapter 39 Zhong Zheng, MD Department of Otolaryngology– Head and Neck Surgery New York Eye and Ear Infirmary of Mount Sinai New York, New York Chapter 25

Section

I History of Rhinology

Chapter

1 History of Rhinology Gordon F. Z. Tsang and Yvonne Chan

Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 4 History of Reconstructive Septorhinoplasty . . . . . . . . 4 Septoplasty . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 4 Reconstructive Rhinoplasty . . . . . . . . . . . . . . . . . . . . . . . . . 4 History of the Paranasal Sinuses . . . . . . . . . . . . . . . . . . . . 5 Understanding Sinonasal Anatomy through the Ages . . . 5 Surgical Access and Progress in Anatomic . . . . . . . . . . . . 5 Understanding of the Maxillary Sinus Development of Endoscopic Access to the . . . . . . . . . . . . . 6 Paranasal Sinuses The Concept of Functional Endoscopic Sinus Surgery . . . 6 Evolution of Endoscopic Techniques to Access the . . . . . 7 Anterior Cranial Base

Conclusions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 8

3

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Introduction • Historically, interest in the paranasal sinuses was fairly limited. Although there are some mention of paranasal sinus anatomy and attempts to describe sinonasal pathology, knowledge was limited by a relative inability to assess and diagnose. • The development of endoscopy in rhinology was instrumental in understanding key aspects of anatomy and pathophysiology of the sinuses as well as offering a novel approach towards the anterior skull base, cervical spine, and other areas of the maxillofacial skeleton. • Expertise in reconstructive surgery of external nose and nasal septum was developed separately throughout the 20th century by plastic surgeons and otolaryngologists.

History of Reconstructive Septorhinoplasty Septoplasty • Evolution of reconstructive rhinoplasty and septoplasty evolved separately in the early 20th century. • Foundational work in septoplasty began with work of Freer and Killian in 1902, who popularized the submucosal reduction technique of septoplasty (extensive removal of bone and cartilage to treat a deviated septum). • Killian introduced the idea of the septum as an important support structure for the external nose and nasal tip. He proposed the need to maintain 1cm strut of cartilage to prevent saddle deformities. • In the 1940s, Cottle, Goldman, and Smith popularized a more conservative approach that focused on repositioning the septum rather than removal of bone and cartilage.

Reconstructive Rhinoplasty • Throughout history, the external nose has been seen to be an important aspect of the human face, and interest in rhinoplasty began with a need for reconstruction of defects mostly caused by traumas. • Increasing involvement of OTOHNS in rhinoplasty—Formation of the American Academy of Facial Plastic Surgery in 1964, and the European Academy of Facial Surgery (Joseph Society) in 1977.

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History of Rhinology   5

History of the Paranasal Sinuses Understanding Sinonasal Anatomy through the Ages • The existence of sinus cavities in the skull was first identified in ancient Egyptian medical writings. • Hippocrates may have been the first to describe sinusitis in the 5th century bc, though for other intellectuals and anatomists of the time (ie, Galen and Celsus), there was little interest in sinonasal pathology. • Through the Renaissance, there was still little interest and no precise anatomic or functional description of the paranasal sinuses; for example, Leonardo da Vinci in his work “Homo Universalis” suggested that the maxillary sinus contained a humor to nourish the teeth. • Nathaniel Highmore, a 17th century British anatomist, was the first to describe and illustrate the maxillary sinus in his book Corporis Humani Disquisitio Anatomica in 1651, hence the historical term “Highmore’s antrum.” • Notable 18th and 19th century efforts in accurate anatomic and physiologic descriptions were led by Grunwald and Zuckerkandl, as well as Fallopius who described the absence of sinuses in children. • Notably, Harris Peyton Mosher, from Harvard University was integral in disseminating his knowledge of the sinuses (particularly the ethmoids) gained from countless dissections and from having been the first person to run a sinonasal anatomy course in North America.

Surgical Access and Progress in Anatomic Understanding of the Maxillary Sinus • In 1770, Gooch first described the endonasal antrostomy procedure; however, the procedure only gained popularity after it was written about again by Lichtwitz, Krause, and Mickulicz (1890), who used needle, trocar, and stylet to puncture the inferior meatal wall, and utilized a canalization method invented by Lichtwitz. • Around this time, Caldwell (1893), Luc (1897), and Spicer (1894) independently described more radical approaches to the maxillary sinus for the treatment of sinusitis via a trans-gingivobuccal incision to open the anterior portion of the maxillary sinus.

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• Between the endonasal and transoral or Caldwell-Luc approach, the latter gained more popularity and became the preferred approach to the maxillary sinus due to better lighting and visualization. • In the beginning of the 20th century (c 1900), Adolf Onodi and Hanau Loeb published “The nose: Paranasal sinuses, nasolacrimal passageways and olfactory organ in man,” an important work describing relational anatomy of the middle turbinate. • Further discoveries in this era included that of Anderson Hilding and Malcolm King, who observed ciliary beating and its contribution to mucociliary transport in canine models.

Development of Endoscopic Access to the Paranasal Sinuses • Philipp Bozzini (1773–1809), a German physician, developed the first endoscope for inspection of bladder, rectum, and pharynx using a device called a Light Conductor which consisted of an eyepiece and candle contained within a fully enclosed box. • This was further modified by Nitze in 1879 into a small cystoscope for urologic examinations, which was subsequently adapted for use by Hirschman in 1901 to visualize the maxillary sinus via an oroantral fistula. • A breakthrough was made in 1960 when Harold Hopkins of Imperial College, London, developed a fiber optic rod endoscope (Hopkins rod) for sinuscopy—the advantages of this design were largely increased lighting, visualization, and ease of use. • Storz (Germany) developed this technology further and introduced angled scopes from 0° to 120° in the same design as a Hopkins rod. • Hopkins rod endoscopes were then used to further understanding of the anatomy and physiology of the paranasal sinuses. This renewed scientific interest in the field of rhinology and transition surgical treatment of the sinuses away from radical open and frequently ablative surgery to more directed treatment aimed at opening ostia to enhance drainage and restore function via an endonasal approach.

The Concept of Functional Endoscopic Sinus Surgery • An Austrian otolaryngologist, by the name of Walter Messerklinger, used Storz endoscopes and published his diagnostic examination techniques in “Endoscopy of the nose” (1978).

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History of Rhinology   7

• Professor Messerklinger subsequently described an approach to treating sinonasal disease that targeted obstruction in the paranasal sinuses, thus introducing the foundational principles of endoscopic sinus surgery or the “Messerklinger Technique.” • His work was further expanded upon by his student, Heinz Stammberger, and they wrote extensively about the anatomy and physiology of the paranasal sinuses, including drainage and aeration, anatomy of lateral wall of nose, and mucociliary clearance. • Wolfgang Draf, a German otolaryngologist, published his surgical techniques for using microscopes and endoscopes in treating sinonasal disease in parallel; most notably, he described and created a classification system for surgery of the frontal sinuses. • Otolaryngologists from North America, notably David Kennedy (who coined the term “functional endoscopic sinus surgery”) and Michael Hawke, learned endoscopic sinus surgery techniques from Messerklinger and began to popularize this surgical method throughout North America beginning around 1985. • Concurrently, understanding of the pathophysiologic process of chronic rhinosinusitis and nasal polyposis was improving. • Pathophysiologic connections were made between the upper and lower airways in the unified airway concept, the immune system and its interaction with bacteria in the sinuses, as well as the importance of maintaining a functional sinus cavity to optimize quality of life.

Evolution of Endoscopic Techniques to Access the Anterior Cranial Base • The first neurosurgeon to use an endoscope in transsphenoidal approach was Guiot (Presse Med. 1963;72:1225–1231); however, this was in combination with the operating microscope. • In the 1970s, neurosurgeons Apuzzo and the team of Bush and Halves further reported the use of an endoscope to assist in microscopic resection of pituitary lesions with extrasellar extension. • This began an era of collaboration between otolaryngologists and neurosurgeons in developing a purely endoscopic approach towards accessing pituitary lesions. • In 1992, Jankowski and colleagues from the Central Hospital, University of Nancy, were the first to report an entirely endoscopic transsphenoidal approach to pituitary pathology. • In 1996, Jho (neurosurgeon) and Carrau (otolaryngologist), at the University of Pittsburgh, published a case series of transnasal and transsphenoidal access to the pituitary gland, and can be credited as

8   Rhinology and Allergy:  Clinical Reference Guide

the pioneers of a solely endoscopic approach for treating pituitary adenomas. • Cappabianca and de Divitiis, in Naples, also reported on their experience with the endoscopic approach, termed “functional endoscopic pituitary surgery.” • Kassam, Carrau, and Snyderman, at the University of Pittsburgh Medical Centre, established the first multidisciplinary skull base center in North America and developed techniques and endoscopic approaches to other areas of the anterior skull base.

Conclusions • The field of rhinology was tremendously advanced by the development and modified use of endoscopes to diagnose and treat sinonasal pathology. • Advancements in paranasal sinus physiology and anatomy, as well as better understanding of mucosal and airway immunology, have caused treatment paradigms to evolve from radical surgery to more conservative approaches that preserve function and improve quality of life and symptom outcomes. • Further advancements in surgical techniques have allowed for collaboration with neurosurgery to begin treating anterior skull base pathologies with endoscopic access through the sinonasal cavity, less comorbidity, and better visualization than traditional open approaches.

References • Chandra RK, Conley DB, Kern RC. Evolution of the endoscope and endoscopic sinus surgery. Otolaryngol Clin North Am. 2009;42(5):​ 747–752. • https://www.american-rhinologic.org/history • Lund V. Laryngoscope. 2002;112:415–419. • Weir N. Otorhinolaryngology. Postgrad Med J. 2000;76:65–69.

Section

II Embryology, Anatomy, and Physiology

Chapter

2 Sinonasal Development and Anatomy Joseph S. Schwartz and Nithin D. Adappa

Nasal Cavity . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 12 Nasal Embryology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 12 Nasal Anatomy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 12 Paranasal Sinuses . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 21 Paranasal Sinus Embryology . . . . . . . . . . . . . . . . . . . . . . . . 21 Paranasal Sinus Anatomy . . . . . . . . . . . . . . . . . . . . . . . . . . 21 Abbreviations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 26

11

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Nasal Cavity Nasal Embryology Nasal Turbinates • Arise in eighth week of gestation as ridges along lateral nasal wall • Maxilloturbinal:  arises inferiorly originating from maxillary process; gives rise to inferior turbinate • Ethmoturbinals:  arise superiorly from ethmoid bone; five arise with only four persisting throughout development 1. First ethmoturbinal:  ascending portion gives rise to agger nasi; descending portion gives rise to uncinate process; regresses during development 2. Second ethmoturbinal:  gives rise to middle turbinate 3. Third ethmoturbinal:  gives rise to superior turbinate 4. Fourth and fifth ethmoturbinals:  gives rise to supreme turbinate (when present) • Primary furrows:  form the recesses separating the ethmoturbinals; gives rise to meati 1. First primary furrow:  separates first and second ethmoturbinals; gives rise to middle meatus 2. Second primary furrow:  gives rise to superior meatus 3. Third primary furrow:  gives rise to supreme meatus

Nasal Anatomy External Nasal Anatomy Nasal Surface Landmarks • Nasion:  corresponds to nasofrontal suture • Radix:  nasal root; centered at nasion; extends inferiorly to the level of the lateral canthus and superiorly by equivalent distance • Rhinion:  corresponds to bony-cartilaginous junction along nasal dorsum • Supratip break:  a break in the nasal profile separating the nasal dorsum and lobule located immediately superior to the tip defining point • Supratip lobule:  portion of lobule located superior to tip-defining point • Tip-defining point:  two points located at the highest, medial and cephalic portion of the lateral crus; corresponds to light reflex externally

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Sinonasal Development and Anatomy   13

• Infratip lobule:  portion of lobule located inferior to tip-defining point and superior to infratip break • Infratip break:  lobule-columella junction • Nasal sill:  nostril rim located between columella and alar facial attachment

Nasal Musculature • Elevators:  function to shorten nose and dilate nostrils 1. Procerus 2. Levator labii superioris alaeque nasi 3. Anomalous nasi • Depressors:  function to lengthen nose and dilate nostrils 1. Depressor septi 2. Alar nasalis • Compressors:  function to lengthen nose and constrict nostrils 1. Compressor narium minor 2. Transverse nasalis

Subcutaneous Layers of the Nose • Best remembered using the phrase “Subcutaneous Fat DeeP” 1. S = Superficial fatty layer (connected to dermis) 2. F = Fibromuscular layer (nasal SMAS) 3. D = Deep fatty layers (contains neurovascular system) 4. P = Periosteum/Perichondrium – Optimal plane of dissection is located between D and P as it is avascular and heals with minimal fibrosis.

Nasal Tip Support • Major tip supports 1. Medial and lateral crura 2. Attachment of medial crura to caudal edge of quadrangular cartilage 3. Attachment of upper lateral cartilage to lower lateral cartilage (“scroll area”) • Minor tip supports 1. Skin-soft tissue envelope (attachment of lower lateral cartilage to overlying skin and musculature) 2. Sesamoid complex (located between lateral crura and pyriform aperture) 3. Interdomal ligament (located between lower lateral cartilages) 4. Anterior nasal spine 5. Cartilaginous septal dorsum 6. Membranous nasal septum

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Nasal Bony Anatomy • Comprised of two nasal bones fused in the midline to form a pyramidal shape • Thicker superiorly than inferiorly • Attachments of nasal bones: 1. Superiorly:  nasal process of frontal bone 2. Laterally:  frontal process of maxilla • Pyriform aperture = Bony opening into the nasal cavity bounded as described below: 1. Superiorly:  caudal margin of nasal bones 2. Inferiorly:  alveolar process of maxilla 3. Laterally:  frontal process of maxilla 4. Medially:  nasal septum

Nasal Cartilages • Upper lateral cartilage (ULC):  fuses superiorly with the nasal bones; articulates inferiorly with the cephalic margin of the LLC, most often forming an interlocking scroll; thickens medially where it becomes continuous with the quadrangular cartilage of the septum, forming the cartilaginous portion of the nasal dorsum • Lower lateral cartilage (LLC):  provides the shape of the nasal tip; composed of medial and lateral crura • Sesamoid cartilages:  small cartilages located lateral to the lateral crus

Nasal Septum • Comprised of both bony and cartilaginous components; lined by either a mucoperiosteal or mucoperichondrial layer. 1. Cartilage components = Quadrangular cartilage 2. Bony components = Perpendicular plate of ethmoid, vomer, crest of the maxillary bone, crest of the palatine bone; minor contributions from crest of sphenoid bone and nasal spine of frontal bone • Membranous (mobile) septum:  cartilage deficient membrane adjoining the columella to the caudal septum; site of hemitransfixion/transfixion incision • Keystone area:  corresponds to convergence of caudal margin of nasal bone, perpendicular plate of ethmoid, and cephalic margin of ULC and cartilaginous septum; failure to preserve this region can result in nasal collapse • Vomeronasal organ (VNO or Jacobsen’s organ):  auxiliary olfactory organ involved in the perception of pheromones in mammals; in humans, function is controversial as it largely regresses in utero; identified as a groove in the anterior-inferior nasal septum

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Sinonasal Development and Anatomy   15

Nasal Cavity Nasal Vestibule • Serves as the entrance to the nasal cavity • Lined by hair bearing skin, sebaceous and sweat glands • Boundaries include nasal septum medially, LLC superiorly and laterally, and alveolar process of maxilla inferiorly • Posteriorly bounded by the limen nasi (limen vestibule), formed by the caudal margin of the ULC; this coincides with the transition from the skin of the nasal vestibule to the mucosal surface (pseudostratified ciliated columnar epithelium) of the nasal cavity

Nasal Valves • Important contributors to nasal airway resistance • Regions at greatest potential for collapse resulting in nasal airway obstruction 1. Internal nasal valve:  bounded by nasal septum, caudal edge of ULC, anterior face of inferior turbinate; normally forms 10°–15° nasal valve angle; narrowest cross-sectional area of the nasal cavity and site of greatest nasal airflow resistance; normally does not undergo any change in dimension during inspiration 2. External nasal valve:  bounded by nasal ala laterally, nasal septum and columella medially; situated caudal to internal nasal valve; normally dilates during inspiration

Lateral Nasal Wall • Bony contributions to the lateral nasal wall from anterior to posterior include: 1. Frontal process of maxilla 2. Lacrimal bone 3. Medial maxillary wall inferiorly, lamina papyracea (LP) superiorly 4. Perpendicular plate of the palatine bone 5. Medial pterygoid plate of the sphenoid bone – Latter two structures make up the sphenopalatine foramen. • Lamella of the lateral nasal wall:  five lamella from anterior to posterior 1. First lamella = uncinate process 2. Second lamella = ethmoid bulla (EB) 3. Third lamella = basal lamella of middle turbinate 4. Fourth lamella = basal lamella of superior turbinate 5. Fifth lamella = basal lamella of supreme turbinate (when present)

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• Turbinates:  shelves of bone encased in erectile mucosa capable of altering nasal airway cross-sectional area; inferior, middle, superior, and occasionally supreme; all originate from ethmoid bone except for inferior turbinate • Middle turbinate (MT):  anterior end attaches to frontal process of maxilla forming the axilla of the MT; has the three sites of attachment oriented in three planes 1. Anterior portion of MT:  oriented in sagittal plane; attached to junction of cribriform plate and lateral lamella; delimits fovea ethmoidalis (roof of ethmoid sinus) from cribriform plate 2. Middle portion of MT:  oriented in coronal plane (vertical); also known as the basal (ground) lamella of MT; attached laterally to LP; delimits anterior from posterior ethmoid sinus 3. Posterior portion of MT:  oriented in axial plane (horizontal); attached to the crista ethmoidalis of the perpendicular process of palatine bone just anterior to sphenopalatine foramen (potential site of postoperative epistaxis when MT is resected); preservation of this attachment point reduces risk of postoperative MT lateralization • Possible variations of the MT include: 1. Concha bullosa:  pneumatized MT; present in one-third of the population 2. Paradoxical MT:  the lateral surface of the MT is convex rather than concave • Meatus:  air space located beneath and lateral to the turbinate of the same name 1. Inferior meatus:  contains slit-like opening (known as Hasner’s valve) corresponding to site of drainage of the nasolacrimal duct, typically located ~1 cm posterior to anterior face of inferior turbinate 2. Middle meatus:  site of drainage of frontal, anterior ethmoid, and maxillary sinuses 3. Superior meatus:  site of drainage of posterior ethmoid and sphenoid sinuses; sphenoethmoidal recess located within posterior aspect of superior meatus • Lacrimal (maxillary) line:  endoscopic landmark corresponding to suture line along the medial orbital wall at the junction of the frontal process of the maxilla and lacrimal bone • Site of attachment of the UP to the lateral nasal wall • Uncinate process (UP):  crescent-shaped, mucosal-lined bone of ethmoid origin which shields natural ostium of the maxillary sinus; may be pneumatized, thereby narrowing ethmoid infundibulum; contains a vertical portion, attached anteriorly to the lacrimal

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Sinonasal Development and Anatomy   17

line, and a horizontal portion, attached inferiorly to the inferior turbinate; site of superior attachment of vertical portion determines frontal sinus drainage pathway 1. Attachment to LP:  frontal sinus drainage occurs medial to the UP, directly into the middle meatus; results in blind pouch of the superior portion of the ethmoid infundibulum (termed recessus terminalis); most common configuration (80%) 2. Attachment to fovea ethmoidalis or MT:  frontal sinus drainage occurs lateral to the UP and directly into the ethmoid infundibulum; less common configuration (20%) • Ethmoid infundibulum:  three-dimensional trough bounded by the UP medially, the LP laterally and the EB posterosuperiorly; opens inferiorly at maxillary sinus ostium • Hiatus semilunaris:  two-dimensional, crescenteric window into the ethmoid infundibulum:  bounded by UP anteriorly and EB posteriorly • Nasal fontanelles:  areas of bony dehiscence of the lateral nasal wall typically covered by mucosa of the middle meatus, connective tissue, and mucosa of the maxillary sinus; site of accessory ostia when mucosa absent; most commonly located in posterior fontanelle (up to 30% of people); posterior fontanelle located posterior to horizontal process of UP; anterior fontanelle located anterior to UP articulation with the inferior turbinate; posterior fontanelle located posterior to natural maxillary os and oriented in sagittal plane (compared to oblique orientation of natural maxillary os) • Osteomeatal complex (OMC):  refers to the region within the middle meatus bounded by the basal lamella of the MT posteriorly, MT medially, LP laterally, and fovea ethmoidalis superiorly; bears particular significance in the pathophysiology of acute bacterial rhinosinusitis as it represents a regional confluence of drainage pathways of the maxillary, anterior ethmoid, and frontal sinuses • Nasolacrimal system:  intimately related with the lateral nasal wall; tear drainage pathway as follows: 1. Upper and lower lacrimal punctum → Upper and lower canaliculus → Common canaliculus (present in 90%) → Valve of Rosenmüller (one-way valve preventing reflux into canaliculi) → Lacrimal sac → Nasolacrimal ductàValve of Hasner → Inferior meatus • Lacrimal fossa:  bony, oval-shaped recess containing the lacrimal sac; made up of two parts, an anterior lacrimal crest (formed by frontal process of maxilla) and posterior lacrimal crest (formed by lacrimal bone); the suture line between these two

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bones corresponds to the lacrimal (maxillary) line, an important endoscopic landmark (see above) • Lacrimal sac:  a significant portion of the sac is located above the axilla of the MT (~0.9 cm) and above the common canaliculus (~0.5 cm); relevant during dacryocystorhinostomy to ensure adequate lacrimal sac exposure

Vascular Anatomy External Nose Vascular Supply • Facial artery branches (external carotid artery) 1. Angular artery → lateral nasal artery; supplies lateral surface of caudal nose 2. Superior labial artery → columellar artery; supplies columella and nasal tip • Ophthalmic artery branches (internal carotid artery) 1. Dorsal nasal artery; supplies nasal dorsum 2. External nasal branch of anterior ethmoid artery; supplies nasal dorsum and tip

Nasal Cavity Vascular Supply • External carotid artery branches 1. Internal maxillary artery (IMA) branches – Descending palatine artery → greater palatine artery; courses through the greater palatine canal located along the posterior aspect of the perpendicular plate of the palatine bone; potential for injury with dissection of the posterior medial maxillary wall at its junction with the posterior maxillary wall; supplies anteroinferior nasal cavity, including nasal septum, via incisive foramen – Pharyngeal branch of IMA:  supplies posterosuperior nasal cavity and nasopharynx – Sphenopalatine artery (SPA):  enters nasal cavity via sphenopalatine foramen (SPF); multiple SPA branches and/ or accessory foramina may exist; SPF located within superior meatus, between basal lamella of MT and superior turbinate a. Posterior septal branch:  courses along sphenoid rostrum inferior to natural sphenoid os; risk of injury when extending sphenoidotomy inferiorly; supplies nasal septum (vascular supply of pedicled nasoseptal flap) b. Posterior lateral nasal branch:  runs along the middle and inferior turbinates, providing their vascular supply

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Sinonasal Development and Anatomy   19

2. Facial artery branches – Superior labial artery → alar branch; supplies nasal vestibule and anterior nasal cavity • Internal carotid artery branches (both branches of ophthalmic artery) 1. Anterior ethmoid artery (AEA):  larger than PEA; exits orbit between superior oblique and medial rectus muscle via anterior ethmoid foramen (~24 mm posterior to anterior rim of lacrimal fossa) within the frontoethmoid suture; courses anteromedially along the skull base within a bony canal (dehiscent in 40%) or below skull base within a mesentery; delineates posterior limit of frontal recess; enters anterior cranial fossa medially by piercing the lateral lamella, rendering it the weakest portion of the skull base (one-tenth as strong as the fovea ethmoidalis); supplies anterior one-third of the nasal cavity 2. Posterior ethmoid artery (PEA):  exits orbit via posterior ethmoid foramen (~12 mm posterior to anterior ethmoid foramen, 6 mm anterior to optic ring); supplies posterior nasal cavity • Nasal cavity vascular plexuses 1. Woodruff ’s plexus:  aggregation of vessels (posterior lateral nasal + pharyngeal branch of IMA) within the posterior inferior meatus; previously thought to be arterial, now shown to be venous in nature 2. Kiesselbach’s plexus (Little’s area):  Located in the anterior nasal septum, most common site of anterior epistaxis; formed by posterior septal branch of SPA, AEA, greater palatine artery, and alar branch of superior labial artery • Venous drainage of nasal cavity 1. Anterior and posterior ethmoid veins → superior ophthalmic vein → cavernous sinus 2. Sphenopalatine vein → pterygoid plexus – As this is a valveless venous system, there is a potential for retrograde spread of infection intracranially (particularly the cavernous sinus). 3. Diploic veins of Breschet:  venous drainage of the frontal sinus occurs via diploic veins, which pass through multiple anterior and posterior table foramina (Breschet’s canals); these develop into the frontal diploic veins of Breschet, which communicate with the dural sinuses (cavernous sinus and superior sagittal sinus); contribute to intracranial spread of infection

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Sensory Innervation External Nose • Branches of ophthalmic division of cranial nerve V (V1) 1. Infratrochlear nerve:  supplies lateral nose above medial canthus 2. External anterior ethmoidal nerve:  supplies nasal tip, dorsum, and ala • Branches of maxillary division of cranial nerve V (V2) 1. Infraorbital nerve:  supplies lateral nose and ala 2. Anterior superior alveolar nerve (branch of infraorbital nerve): minor contribution to nasal tip and columella

Nasal Cavity • Branches of ophthalmic division of cranial nerve V (V1) 1. Internal nasal branch of anterior ethmoidal nerve:  supplies anterior and superior regions of the nasal cavity 2. Posterior ethmoidal nerve:  supplies posterior nasal cavity • Branches of maxillary division of cranial nerve V (V2) 3. Sphenopalatine (nasopalatine) nerve (anastomoses with greater palatine nerve):  supplies posterior nasal cavity 4. Anterior superior alveolar nerve:  supplies anterior nasal cavity and nasal floor

Autonomic Innervation Sympathetic Innervation • Function: vasoconstriction • Preganglionic:  sympathetic chain • Ganglion:  superior cervical ganglion • Postganglionic:  deep petrosal nerve joining with greater superficial petrosal nerve (GSPN) → vidian nerve (passes through sphenopalatine ganglion without synapsing) → distributed to nasal mucosa with sensory nerves

Parasympathetic Innervation • Function:  vasodilation and mediation of nasal secretions • Preganglionic:  superior salivatory nucleus (CN VII) → nervus intermedius (leaves CN VII at geniculate ganglion with GSPN to form vidian nerve) • Ganglion:  sphenopalatine ganglion • Postganglioinic:  distributed to nasal mucosa with deep branches of V2

Sinonasal Development and Anatomy   21

CHAPTER 2

Paranasal Sinuses Paranasal Sinus Embryology Paranasal Sinuses (in order of completed development) • Ethmoid sinus:  first to develop in utero; most developed at birth (completely developed in number of cells but not in size); anterior ethmoid originates from middle meatus, posterior ethmoid from superior meatus; other structures originating from ethmoid bone include middle, superior and supreme turbinate, cribriform plate, and perpendicular plate of ethmoid • Maxillary sinus:  In early childhood, floor of sinus is situated above nasal floor due to presence of unerupted dentition; roof of sinus is sloped downward from medial to lateral due to disproportionately large orbit relative to midface. • Sphenoid sinus:  Pneumatization begins postnatally (~1 year of age); pneumatization preceded by conversion of red bone marrow to fatty marrow; pneumatization proceeds following marrow involution with subsequent epithelialization of the sinus mucosal lining. Failure of this conversion process results in arrested pneumatization (see sphenoid sinus section). Pneumatization progresses in an inferior posterolateral direction; adult size reached by age 12, beyond which size remains constant, although shape may change. • Frontal sinus:  last sinus to begin and complete development; majority of development occurs postnatally, with pneumatization continuing into early adulthood 1. Only ethmoid and maxillary sinuses are present at birth.

Order of Paranasal Sinuses According to Size (from largest to smallest) • Maxillary sinus • Frontal sinus • Sphenoid sinus • Ethmoid sinus

Paranasal Sinus Anatomy Maxillary Sinus Boundaries of Maxillary Sinus • Superiorly:  orbital floor

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• Inferiorly:  alveolar and palatine process of maxilla • Laterally: zygoma • Medially:  lateral nasal wall • Posteriorly:  pterygopalatine (PPF) and infratemporal fossa (ITF) • Anteriorly:  facial surface of maxilla

Foramen of Maxillary Bone • Infraorbital foramen:  infraorbital nerve, artery and vein; run along roof of maxillary sinus within infraorbital canal with potential for injury during surgical instrumentation if canal is dehiscent (14% of people) • Incisive foramen:  greater palatine artery and nerve • Maxillary os:  located within posterior one-third of ethmoid infundibulum • Superior alveolar foramen:  posterior, middle and anterior superior nerve, artery and vein

Endoscopic Maxillary Sinus Landmarks • Roof of maxillary sinus:  corresponds to floor of sphenoid sinus • Posterior wall of maxillary sinus:  corresponds to anterior face of sphenoid sinus • Medial maxillary wall of maxillary sinus:  with normal pneumatization, located in line with a vertical line drawn tangential to LP; can be lateral to LP if hypoplastic or atelectatic maxillary sinus

Ethmoid Sinus Anterior Ethmoid Sinus (anterior to basal lamella of MT) • Anterior ethmoid cells are generally more numerous but smaller. 1. Agger nasi:  most anterior ethmoidal cell, projects anterior to the axilla of the MT creating a bulge in the lateral nasal wall; posterior limit forms the anterior border of the frontal recess; anteroposterior distance of the frontal recess largely determined by degree of pneumatization of the agger nasi cell; a large, well pneumatized agger nasi cell confers a small frontal beak, which gives rise to a large anteroposterior distance 2. Ethmoid bulla:  largest anterior ethmoidal cell; attached laterally to LP; most constant and identifiable ethmoidal cell 3. Sinus lateralis:  comprised of the suprabullar and retrobullar recesses; suprabullar recess located above EB in the absence of a suprabullar cell; retrobullar recess located posterior to EB and anterior to basal lamella of MT; boundaries include EB

CHAPTER 2

Sinonasal Development and Anatomy   23

anteriorly, basal lamella of MT posteriorly, MT medially, LP laterally, and fovea ethmoidalis superiorly 4. Suprabullar cell:  ethmoidal cell located above the EB which does not pneumatize into the frontal sinus; roof of this cell formed by fovea ethmoidalis (ethmoid roof/skull base) 5. Frontal bulla cell:  suprabullar cell which pneumatizes into frontal sinus along posterior wall of frontal sinus 6. Supraorbital ethmoid cell:  ethmoid cell located posterolateral to the frontal sinus ostium with pneumatization lateral to the LP and superolateral to the orbital roof (orbital plate of the frontal bone); anterior ethmoid artery typically located within the posterior wall of the cell along or immediately beneath the skull base 7. Haller cell (infraorbital ethmoid cell):  most common anatomic variation within the maxillary sinus; ethmoid cell, most commonly originating from the anterior ethmoid sinus, which pneumatizes along the inferomedial orbit, thereby contributing to obstruction of the natural drainage pathway of the maxillary sinus

Posterior Ethmoid Sinus (posterior to basal lamella of MT) • Posterior ethmoid cells are generally fewer but larger. 1. Onodi cell (sphenoethmoidal cell):  posterior ethmoid cell which pneumatizes posteriorly over the superolateral aspect of the sphenoid sinus; when present, the optic nerve and occasionally the internal carotid artery can project along the superolateral wall of the Onodi cell rather than the sphenoid sinus, placing them at increased risk of injury intraoperatively; reported incidence of ~30%; identified on coronal CT scan as a horizontal septation within the sphenoid sinus, posterior to the bony choanal arch (Onodi cell located superolaterally, sphenoid sinus located inferomedially)

Keros Classification • Classification system for assessing the depth of the olfactory fossa (corresponds to the length of lateral lamella); lateral lamella of cribriform plate is the thinnest bone in the skull base; increased risk of cerebrospinal fluid (CSF) leak during endoscopic sinus surgery (ESS) with increasing Keros type due to low cribriform fossa and increased length of thin lateral lamella (more subject to fracture) 1. Type 1:  1–3 mm (second most common configuration) 2. Type 2:  4–7 mm (majority of cases) 3. Type 3:  8–16 mm (rare)

24   Rhinology and Allergy:  Clinical Reference Guide

Fovea Ethmoidalis and Skull Base • Fovea ethmoidalis (ethmoid roof):  formed by orbital plate of frontal bone; slopes downward (~15°) from anterior to posteriorly and from lateral to medial; posteromedial region of fovea ethmoidalis theoretically at greater risk of injury during ESS given lower height; attaches to lateral lamella medially • Lateral lamella:  formed by ethmoid bone; forms lateral surface of cribriform fossa; thinnest and weakest bone in the skull base • Cribriform plate:  forms floor of cribriform fossa; perforated by multiple olfactory nerve fibers; also slopes downward as it passes posteriorly

Sphenoid Sinus Walls and Recesses of the Sphenoid Sinus • Planum sphenoidale:  forms sphenoid roof; contiguous anteriorly with fovea ethmoidalis • Sphenoid rostrum:  forms sphenoid face and anterior floor; articulates anteriorly with the vomer and perpendicular plate of ethmoid forming a keel • Sella turcica:  Latin for turkish saddle; rounded projection along posterosuperior wall in a well pneumatized sphenoid sinus; forms floor of hypophyseal fossa (containing pituitary gland, middle cranial fossa); may be attenuated or anteriorly displaced in the presence of a pituitary macroadenoma; bounded anterosuperiorly by tuberculum sella and posteriorly by dorsum sella • Lateral pterygoid recess (lateral recess):  inferolateral pneumatization of the sphenoid sinus; common location for spontaneous CSF leak giving rise to encephalocele formation • Lateral wall of sphenoid:  forms medial wall of cavernous sinus; when well pneumatized, bony impressions of the internal carotid artery (partially dehiscent in 25%) and optic nerve (dehiscent in 6%) can be visualized • Opticocarotid recess:  bony depression between the optic nerve superiorly and the cavernous internal carotid artery segment inferiorly; located posterosuperiorly along the lateral wall of a well pneumatized sphenoid sinus • Sphenoid intersinus septum:  often asymmetrically divides the sphenoid sinus; must be removed with caution in transnasal endoscopic approaches to the skull base when inserts onto or in the vicinity of the carotid and/or optic canal • Clival recess:  forms posteroinferior wall of the sphenoid sinus if well pneumatized; separates sphenoid sinus from posterior cranial fossa

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Sinonasal Development and Anatomy   25

• Choanal arch:  forms the floor of the sphenoid; corresponds to roof of nasopharynx; bordered laterally by the medial pterygoid process

Classification of Sphenoid Pneumatization • Conchal:  absent pneumatization, sinus is filled by cancellous bone; rare • Presellar:  pneumatization anterior to a vertical line drawn through the tuberculum sella; second most common pattern • Sellar:  pneumatization extends beyond a vertical line drawn through the turberculum sella; majority of cases • Postsellar:  pneumatization extends beyond the dorsum sella 1. Arrested pneumatization (arrested development):  developmental variant resulting in persistence of atypical fatty marrow within a non-pneumatized region of the sphenoid sinus; incidental finding upon imaging as patients are typically asymptomatic; distinct from sphenoid hypoplasia wherein a normal bony trabecular pattern is present within the sphenoid sinus; may be mistaken for skull base lesion; most commonly occurs in sphenoid sinus

Landmarks for Sphenoid Ostium • 1.5 cm superior to the bony choanal arch • One-third up from choana to skull base • 7 cm at a 30-degree angle from the anterior nasal spine • Between the nasal septum and posterior insertion of the superior turbinate (most reliable landmark)

Frontal Sinus • Made up of two frontal sinuses, frequently asymmetric, separated by an intersinus septum located approximately in the midline • Thick anterior wall, thin posterior wall (anterior wall of anterior cranial fossa) • Frontal beak:  thick bone of the frontal process of maxilla, anterior to the agger nasi, that projects posteriorly into the frontal recess, thereby limiting its anteroposterior distance

Frontal Recess • Refers to an hourglass space with the narrowest portion corresponding to the frontal os; communicates with the frontal sinus superiorly and anterior ethmoid region inferiorly; bordered anteriorly by the frontal beak/agger nasi, medially by the lateral lamella, laterally by the LP, posteriorly by the EB/suprabullar

26   Rhinology and Allergy:  Clinical Reference Guide

recess, and posterosuperiorly by the fovea ethmoidalis; significant variation accounts for the complexity of frontal sinus dissection

Frontal Sinus Cells • Frontal ethmoidal cells:  anterior ethmoidal cells in contact with the anterior wall of the frontal recess (frontal process of maxilla) Two classification schemes exist: 1. Kuhn Classification: – Type 1:  single cell above agger nasi cell – Type 2:  tier of cells above agger nasi cell – Type 3:  single cell pneumatizing into the frontal sinus – Type 4:  isolated cell within the frontal sinus 2. Modified Kuhn Classification (Wormald) – Type 1 and 2:  no change from previous classification – Type 3:  cell pneumatizing into the frontal sinus but less than 50% of the vertical height of the sinus – Type 4:  cell pneumatizing into the frontal sinus greater than 50% of the vertical height of the sinus Change in classification was prompted by rarity of initial definition of Type 4 cell. • Intersinus septal cell:  pneumatization of the interfrontal sinus septum; originates medially, thereby displacing frontal sinus drainage pathway laterally • Other relevant frontal recess cells include agger nasi cell, suprabullar cells, frontal bulla cells, and supraorbital ethmoid cells (see description above).

Abbreviations ULC = upper lateral cartilage LLC = lower lateral cartilage MT = middle turbinate UP = uncinate process EB = ethmoid bulla LP = lamina papyracea (medial orbital wall) GSPN = greater superficial petrosal nerve ESS = endoscopic sinus surgery IMA = internal maxillary artery SPA = sphenopalatine artery SPF = sphenopalatine foramen AEA = anterior ethmoid artery

Figure 2–1.  Coronal Sinus 1. Inferior turbinate 2. Middle turbinate 3. Uncinate process 4. Semilunar hiatus 5. Ethmoid infundibulum 6. Maxillary os

7. Frontal sinus 8. Maxillary sinus 9. Olfactory cleft 10. Nasal septum 11. Lamina papyracea

27

Figure 2–2.  Lacrimal Bone 1. Frontal process of maxilla 2. Lacrimal bone 3. Nasal bone 4. Anterior lacrimal crest

5. Posterior lacrimal crest 6. Lacrimal fossa 7. Lacrimal-maxillary suture (lacrimal line)

28

Figure 2–3.  Nasolacrimal System 1. Upper lacrimal punctum 2. Upper canaliculus 3. Lower canaliculus 4. Lower lacrimal punctum 5. Common canaliculus 6. Valve of Rosenmüller

7. Lacrimal sac 8. Nasolacrimal duct 9. Valve of Hasner 10. Inferior meatus 11. Inferior turbinate 12. Middle turbinate

29

30

Figure 2–4.  Lateral Nasal Wall Bone 1. Frontal process of maxilla 14. Ethmoid bulla 15. Semilunar hiatus 2. Lacrimal bone 16. Ethmoid infundibulum 3. Lacrimal line 17. Incisive canal 4. Medial maxillary wall 18. Tuberculum sella 5. Lamina papyracea 19. Dorsum sella 6. Perpendicular plate of palatine bone 20. Sella turcica 7. Medial pterygoid plate of 21. Planum sphenoidale sphenoid bone 22. Sphenoid sinus 8. Lateral pterygoid plate 23. Clivus 9. Sphenopalatine foramen 24. Frontal sinus 10. Inferior turbinate 25. Anterior nasal spine 11. Middle turbinate (cut) 26. Palatine process of maxilla bone 12. Valve of Hasner 27. Palatine bone 13. Uncinate process

31

Figure 2–5.  Lateral Nasal Wall Mucosa 1. Inferior turbinate 11. Sella turcica 2. Middle turbinate 12. Planum sphenoidale (cut) 13. Sphenoid sinus 3. Valve of Hasner 14. Clivus 4. Uncinate process 15. Frontal sinus 5. Ethmoid bulla 16. Anterior nasal spine 6. Semilunar hiatus 17. Palatine process of 7. Ethmoid maxilla bone infundibulum 18. Palatine bone 8. Incisive canal 19. Soft palate 9. Tuberculum sella 20. Torus tubaris 10. Dorsum sella

32

Figure 2–6. Sphenoid 1. Planum sphenoidale 2. Optic nerve 3. Internal carotid artery 4. Optico-carotid recess (OCR) 5. Sella 6. Clival recess 7. Tuberculum recess

Chapter

3 Sinonasal Physiology Kristian I. Macdonald

Physiology of the Nasal Airway . . . . . . . . . . . . . . . . . . . . 34 Nasal Cycle and Respiratory Airflow . . . . . . . . . . . . . . . . . 34

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34   Rhinology and Allergy:  Clinical Reference Guide

Physiology of the Nasal Airway Nasal Cycle and Respiratory Airflow Function of Paranasal Sinuses • Suggested roles include: 1. Resonance for phonation 2. Humidification 3. Immunologic benefit 4. Filtration 5. Olfaction 6. Mechanical trauma protection 7. Reduced skull weight • Large surface area helps to regulate humidity and temperature.

Mucosal Properties • Pseudostratified, ciliated, columnar epithelium with goblet cells • Secretory blanket is produced by goblet cells (mucous secretions) and submucosal seromucous glands (watery secretions). – Contains glycoproteins, albumin, IgG, IgM, IgA, complement factors, lactoferrin, lysozyme • Cilia transport mucous at 3 to 25 mm/min, propel towards natural sinus ostia • Sinuses are normally sterile. • Nitric oxide is present in large quantities in maxillary sinuses. – Bacterostatic and ciliary stimulating properties • Normal mucous production (~1L/day) drains into pharynx where it is swallowed. • Middle meatus drains frontal, maxillary and anterior ethmoid sinuses. • Superior meatus drains the posterior ethmoid sinuses. • Sphenoethmoid recess drains sphenoid.

Nasal Cycle • Alternating nasal swelling occurs every 1–6 hours. – Results in subjective alternating nasal obstruction • Results from autonomic regulation of venous sinusoids • Diurnal nasal cycle is a normal phenomenon; patients may complain of nighttime nasal obstruction.

CHAPTER 3

Sinonasal Physiology   35

Sinonasal Pathology • Infection/environmental toxins/inflammation/ciliary disorders (cystic fibrosis, Kartagener’s syndrome, primary ciliary dyskinesia) can impair ciliary function. • Immunoglobulin or Ig deficiencies lead to recurrent infections. • Bitter taste receptors (T2Rs), identified in cilia, may mediate ciliary function in response to bacteria. • Chronic exposure to insults can lead to chronic inflammatory disease. • Inflamed/damaged mucosa has regenerative capacity after disease is treated.

Chapter

4 Olfaction and Taste Jasper Shen, Kevin Hur, and Bozena B. Wrobel

Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 38 Olfactory Anatomy and Physiology . . . . . . . . . . . . . . . . 38 Olfactory Neuroepithelium . . . . . . . . . . . . . . . . . . . . . . . . . 38 Central Olfactory Pathway . . . . . . . . . . . . . . . . . . . . . . . . . 39 Olfactory Physiology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 40 Clinical Assessment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 41 History and Physical . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 41 Imaging and Laboratory Tests . . . . . . . . . . . . . . . . . . . . . . 42 Chemosensory Testing . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 42 Psychophysical Tests . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 42 Olfactory Disorders . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 44 Classification of Dysfunction . . . . . . . . . . . . . . . . . . . . . . . 44 Conductive Disorders . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 44 Sensorineural Disorders . . . . . . . . . . . . . . . . . . . . . . . . . . . 45 Aging and Neurodegenerative Processes . . . . . . . . . . . . . 46 Treatment Paradigm . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 47 Taste . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 48 Gustatory Anatomy and Physiology . . . . . . . . . . . . . . . . . 48 Evaluation of Taste . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 49 Disorders of Taste . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 49 Treatment Paradigm . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 50

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38   Rhinology and Allergy:  Clinical Reference Guide

Introduction • Approximately 10% and 5% of American adults reported smell and taste disturbances respectively within the past year. • Olfaction contributes significantly to an individual’s enjoyment and quality of life (QOL): 1. Determining flavor and palatability of food and beverages and affecting appetite 2. Warning system against spoiled foods and environmental hazards, including poisons, toxins, pollution, smoke/fire, or leakage of natural gas 3. Occupational tool (chefs, firefighters, perfumists, wine merchants, plumbers, etc) • Over 80% of taste dysfunction is secondary to a primary olfactory disorder. • Prevalence of olfactory loss is significantly correlated to age:  1% in those younger than 65 years, 50% in those 65–80 years of age, and over 60% in those >80 years old.

Olfactory Anatomy and Physiology Olfactory Neuroepithelium • Olfactory Neuroepithelium (ON) is composed of pseudostratified columnar epithelium, which lines the olfactory cleft in each nasal cavity. • ON is thicker and paler than surrounding respiratory epithelium and can be visualized endoscopically at the roof of the nasal cavity:  bordered by cribriform plate superiorly, nasal septum medially, superior and middle turbinates laterally. • The mucous layer over the ON traps and solubilizes the odorant molecules, which initiates the chemosensory signal to the central pathway and olfactory bulb (OB). • Layers and cell types of the olfactory neuroepithelium (inferior to superior) 1. Olfactory mucosa:  olfactory receptor neurons, sustentacular cells, microvillar cells, basal cells 2. Basal lamella 3. Lamina propria:  Bowman’s glands 4. Cribriform plate (bony) separates ON and OB

Olfaction and Taste   39

CHAPTER 4

Olfactory Receptor Neuron (ORN) • 6 to 10 million ORNs in each nasal cavity; life span of 3 to 7 weeks • Bipolar neurons with dendrites extending inferiorly to the epithelial surface, and axons extending intracranially to synapse at the OB • An ORN dendrite has nonmotile cilia at its thickened ending/ knob, increasing its overall functional surface area to 22 cm2 for odorant binding. • Olfactory receptors are expressed on the nonmotile cilia of the ORN dendrites; there are approximately 1000 different human genes encoding different receptor types. • Unmyelinated axons of ORN join to form myelinated fascicles (fila olfactoria) which travel in bundles of 50 via the foramina of the cribriform plate to synapse in the OB and constitute the olfactory nerve (CN1).

Sustentacular Cell (supporting cells) • Insulates and protects ORN • Has high concentration of cytochrome p450 • Maintains cellular homeostasis and regulates mucus composition • Deactivates odorants

Microvillar Cell (unknown function) Basal Cells (horizontal and globose) • Regenerate all cell types in ON into adulthood, including the ORN. • Regeneration decreases with increasing age of patient or severity of injury. • Viable neural progenitor cells capable of generating ORN can be cultured from middle turbinate biopsies.

Bowman’s Gland • Located within lamina propria • Secretes mucous layer made of IgA/IgM, lactoferrin, lysozyme, and odorant binding proteins • Assists odorant binding and clearing, and prevents pathogen entry

Central Olfactory Pathway • Odorant molecule trapped by the mucous layer over the ON and transported by the odorant binding proteins → odorant receptors

40   Rhinology and Allergy:  Clinical Reference Guide

on ORNs within the olfactory cleft → axons bundle join in myelinated fascicles to become CN1 → passes through cribriform plate → synapse in glomeruli of olfactory bulb → second order neurons (mitral cells) → olfactory tract → olfactory cortex

Olfactory Bulb • The complex processing center is located at the ventral base of the frontal lobe, on top of the cribriform plate • Mitral cells within the OB receive axons of the ORN. • Olfactory nerve bundles synapse with the second order neurons within thousands of glomeruli within the bulb. • Axons from mitral cells leave the bulb as lateral olfactory tract, which extends to olfactory cortex. • Anterior commissure is a small commissure that connects the two halves of the olfactory system.

Olfactory Cortex • Portions of cerebral cortex receiving direct input from the olfactory bulb via mitral cell axons • Located on the base of the frontal lobe and medial aspect of the temporal lobe • Temporal lobe of the olfactory cortex covers a portion of the parahippocampal gyrus known as the uncinate gyrus. • Seizures often arise from the uncinate gyrus and are characterized by hallucinations of smell and taste. • Olfactory information is relayed via the mediodorsal nucleus of the thalamus to the insular cortex (olfactory and gustatory sensory integration), the orbitofrontal cortex (conscious smell perception), and the amygdala (limbic system, memory).

Olfactory Physiology • The Nobel Prize in Physiology or Medicine in 2004 was awarded jointly to Richard Axel and Linda B. Buck for their discovery of olfactory receptors and elucidation of the molecular basis of olfactory system. • The human genome contain 1,000 olfactory receptors genes, of which 350 functionally code for unique receptors. • Each olfactory receptor cell expresses only one type of binding protein. • This receptor is a transmembrane G-protein-coupled receptor.

Olfaction and Taste   41

CHAPTER 4

• Receptor binding by odorant activates an effector enzyme (either adenylate cyclase or phospholipase C, depending on the nature of the odorant). • The secondary messenger (cAMP or IP3), generated by the effector enzyme, opens a Ca2+ channel, followed by a Ca2+ gated Cl- channel. • Cl- leaves the cell and the membrane is depolarized. • Sufficient depolarization causes an action potential along the ORN axon. • Most odorants can stimulate multiple receptors and vice versa, allowing for the permutation of thousands of detectable odors.

Clinical Assessment History and Physical History • Quality: 1. Elucidate primary taste vs smell dysfunction 2. Single sided (more likely obstructive etiology) vs bilateral smell loss 3. Classification of dysfunction (see page 44) 4. Perceived degree of smell loss (complete vs partial) 5. Olfactory status prior to loss • Timing of Onset and Duration: 1. Never had olfaction (ie, congenital) 2. Sudden (ie, trauma, URI) vs gradual (ie, sinonasal disease, tumor, presbyosmia) 3. Persistent vs intermittent (ie, CRS, polyposis) • Associated Factors: 1. Changes to diet 2. Associated symptoms of URI, sinusitis, allergy, epistaxis, seizure, and psychiatric complaints 3. Mental changes or peripheral nerve difficulties (ie, neurodegenerative disorders) 4. Head trauma (ie, shearing injury of ON) • Social History 1. Occupational and environmental exposure to toxins 2. Substance abuse (ie, smoking, cocaine, inhalant)

Physical Exam • Complete ear, nose, throat exam with a focus on the following

42   Rhinology and Allergy:  Clinical Reference Guide

• Nasal endoscopy:  careful evaluation of olfactory cleft and the middle meatus to rule out obstructive etiologies; anterior rhinoscopy significantly less sensitive than flexible endoscopy • Otoscopy:  rule out obvious middle ear pathology affecting the chorda tympani nerve • Neurological exam:  cranial nerve evaluation, optic disc exam • Mini-mental state exam:  perform if dementia or a neurodegenerative disorder are suspected

Imaging and Laboratory Tests • Choice of imaging and laboratory tests is largely dependent on history and physical exam (ie, obvious history of URI-related smell loss in an otherwise healthy individual warrants no further imaging). • CT of paranasal sinuses:  indicated if obstructive etiology or sinus disease suspected • MRI:  often first line for idiopathic smell loss, also indicated for intranasal mass to assess intracranial extension, confirmation of agenesis of olfactory bulbs in Kallman syndrome (see page 46), or history of head trauma or neurologic dysfunction • Idiopathic olfactory loss is often unrevealing on imaging; with only 0.8% of patients found to have a contributing radiographic finding on MRI in retrospective study • Laboratory studies/tests:  targets include CBC, sedimentation rate, plasma creatinine, liver function, antinuclear antibodies, thyroid function, vitamin B12, and other mineral/vitamin deficiencies

Chemosensory Testing • Olfactory testing may establish degree of smell loss; monitor progression/recovery over time, and identify malingering for medicolegal purposes. • Psychophysical testing includes odor identification, detection, or discrimination. • Free nerve endings of the trigeminal nerve (CN5) respond to irritating or pungent odors in the nasal cavity (ie, ammonia).

Psychophysical Tests University of Pennsylvania Smell Identification Test (UPSIT) • Self-administered scratch and sniff identification test

CHAPTER 4

Olfaction and Taste   43

• 40 item (scored 0–40) with four responses for each microcapsulated odor • >34 normosmia, 19–34 microsmia, 6–18 anosmia, though this will vary with age and gender • 60 minutes • Pros:  useful screening technique; materials are readily available and inexpensive; non-invasive, easy to perform • Cons:  non-specific; affected by taste threshold of patient (can be combined with colored dye to improve detection); results can be affected by multiple factors • If positive for prolonged clearance, referral for further testing for primary ciliary dyskinesia, cystic fibrosis, or consideration of secondary ciliary dysfunction necessary

Radioscintigraphy • Technetium-based particles (ie, albumin, resin) placed at inferior turbinate similar to saccharin test and percent of remaining radiation measured after 30 minutes • Sensitive test for measuring objective changes within setting of paranasal surgery follow-up as well as following efficacy of nasal medications • Pros:  not influenced by sniffing • Cons:  limited by price, availability of materials; lack of human study confirming reproducibility; radiation exposure (although very low); false positives

Nasal Nitric Oxide (nNO) Testing • Patients with primary ciliary dyskinesia tend to have 10× less nasal nitric oxide than control subjects. • Screening test for PCD • Pros:  non-invasive and inexpensive

82   Rhinology and Allergy:  Clinical Reference Guide

• Cons:  requires patient participation for breath-holding, making it difficult to perform in patients less than 5 years old; false positives; can be affected by extrinsic factors (ie, obstruction, URI)

Nasal Airflow • Subjective nasal patency has traditionally been considered a factor of nasal resistance. • Although resistance and subjective nasal patency are interrelated, data have also shown increased sensation of nasal patency with mucosal cooling without reduction in nasal resistance. • The nasal valve (within the first 2 to 4 cm of the nasal cavity) is the area of greatest resistance within the nasal cavity and is commonly thought to be the most clinically relevant anatomic unit in regard to nasal airflow. • Even though the most important metrics for assessment of nasal airflow are patient symptoms, objective assessments are useful and attempt to identify objective targets for therapeutic and/or surgical intervention. • Nasal exam +/− endoscopy is essential for clinical correlation. • Objective tests typically correlate better with patient symptoms (a) when a patient is symptomatic (ie, suffers from nasal airway obstruction) and (b) the larger the measured differences measured between a patient’s two nasal cavities (ie, correlation with subjective scores improves when one nasal cavity is severely obstructed and the other is widely patent versus two similarly obstructed nasal cavities). 1. Correlations have been observed between nasal resistance and patient symptoms before and after surgical intervention; however, this does not necessarily imply causation.

Peak Nasal Airflow • Inspiratory or expiratory flow 1. Inspiratory better correlates with nasal resistance. • Pros:  fast, simple, minimal equipment, readily available/portable; of all techniques, most reproducible results during repeated measurements of a single patient (ie, following patient response to medication) • Cons:  relies on patient cooperation and effort; conflicting evidence regarding sensitivity compared to rhinomanometry for detecting changes in nasal patency following pharmaceutical (ie, histamine) challenge

CHAPTER 8

Objective Measures of Nasal Function   83

Rhinomanometry • Measures transnasal pressure and airflow simultaneously • Anterior (most common), posterior, and postnasal techniques described • Resistance recorded at maximum flow and pressure during normal respiration was found to correlate best with patient symptoms. • Measures “physiologic” minimum cross-sectional area • Pros:  anterior rhinomanometry easy to perform; determines nasal patency in terms more representative of how difficult it is for patient to breathe • Cons:  anterior technique confounded by septal perforations; posterior technique poorly tolerated by nearly one-sixth of patients; conflicting evidence regarding correlation with patient symptom scales

Acoustic Rhinometry • Uses acoustic pulses or “clicks” to map nasal cavity and measure cross-sectional area rather than airflow or resistance 1. Measures “anatomic” minimum cross-sectional area 2. Also reports distance to the narrowest area • Gives profile of cross-sectional area through the nose and volume of nasal cavity • Can be done before and after decongestion to compare mucosal and structural components of obstruction • Pros:  favorable test to analyze rapidly changing mucovascular conditions and changes in nasal volume; normal values available for comparison • Cons:  must be done in quiet room with controlled temperature and humidity; can be distorted by very small anterior constriction, probe positioning, movement, seal quality

Computational Fluid Dynamics • Recently developed technology using CT scans to build 3D models of the nasal airway and utilizing software to simulate nasal airflow • Measures objective variables such as wall sheer stress, heat flux, resistance, airflow velocity, airflow partitioning, laminar vs turbulent flow • Good evidence to show that certain objective variables such as heat flux, unilateral resistance of the obstructed nasal cavity, and middle regional airflow (airflow above inferior turbinate and below superior turbinate) reliably correlate with Nasal Obstruction

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Symptom Evaluation (NOSE) and Visual Analog Scale (VAS) scores both pre- and postoperatively • Pros:  potential application for individualized surgery by virtual surgery planning/simulation; ability to identify clinically-relevant anatomical obstruction; correlation with subjective nasal patency scores • Cons:  cross-sectional study; cannot take into effect nasal cycling; radiation exposure; unable to perform multiple tests without subjecting to additional radiation

Nasal Challenge/Provocation Test • Objective evaluation of nasal mucosal change in response to allergen exposure • Rhinomanometry and acoustic rhinometry most commonly used for assessment of nasal patency before and after exposure • Objective measurements can provide quantitative values to allergen response as opposed to reliance on subjective symptoms alone. • Acoustic rhinometry is more sensitive than rhinomanometry to mucosal changes that are not accompanied by increased nasal resistance, provides more consistent results in severely congested patients, and can be performed more quickly and easily than rhinomanometry.

Disease-Specific Health-Related Quality of Life (QOL) Instruments • Despite being quality-of -life diagnoses, both nasal airway obstruction and chronic rhinosinusitis are associated with significant economic and symptomatic burden. • Currently, objective tests fail to adequately and consistently correlate with patient-reported symptoms. • A number of tools have been developed to measure the “health burden” of each of these conditions. • The ideal QOL instrument should have the following characteristics: 1. Reflect important aspects of the disease process 2. Be responsive to the effects of treatment 3. Accurately reflect the impact of disease on patient’s healthrelated QOL • Generic vs specific QOL metrics

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1. Generic instruments measure burden of illness across different disease processes. – Example:  Short Form-36 (SF-36) 2. Specific instruments measure impairments most important to patients, focusing on specific problems and, therefore, are more sensitive to clinically important changes.

Nasal Airway Obstruction (NAO) • No current gold standard objective test for NAO exists. • As it is a quality-of-life condition, patient symptom experience is highly relevant and important to quantify both pre- and post-intervention. • Nasal Obstruction Symptom Evaluation (NOSE) 1. Validated for septoplasty and functional septorhinoplasty 2. Asks patients five questions on a 0 to 4 scale to report symptom severity over the last 30 days; score is then multiplied by 5 for a maximum possible score of 100, with 100 being the most severely symptomatic 3. Meta-analysis derived normative mean scores (standard deviation): – Healthy:  15 (17) – Symptomatic NAO patients:  65 (22) – General population:  42 (27) • Visual Analog Scale (VAS) 1. Validated scale for NAO 2. Simple numerical scale providing real-time, immediate subjective nasal obstruction symptom reporting (crosssectional report) 3. Multiple scales available, however inconsistent among studies – Traditionally asks for patient to report nasal obstruction by placing mark on 10 cm line, with 0 indicating lack of nasal obstruction, and 10 indicating severe obstruction; the score is the numerical value of the centimeters from zero at which the mark is made – Inconsistencies in scales utilized may cause confusion in either or both patient reporting and/or score analysis 4. Meta-analysis derived normative mean scores (standard deviation): – Healthy:  2.1 (1.6) – Symptomatic NAO patients:  6.7 (2.3) – General population:  4.6 (2.6)

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• Both the NOSE and VAS scores can be used to follow pre- and postoperative patient symptoms and to compare with clinical examination and objective testing.

Chronic Rhinosinusitis (CRS) • Chronic Sinusitis Survey (CSS) 1. Duration-based monitor, generating overall CRS symptom score and two subscores for symptoms and medication use 2. High reliability, validity, responsiveness and ease of use 3. Six questions addressing pain/pressure, nasal congestion, rhinorrhea, or post-nasal drip over the last 8 weeks 4. Scores range from 0 to 100, with lower scores indicating more severe disease 5. Advantages include capturing data on recent drug therapy and quick completion • Rhinosinusitis Outcome Measure (RSOM 31) 1. Thirty-one questions divided into seven categories 2. Good validity and responsiveness; however, it was difficult to complete and score and was subsequently modified into the sinonasal outcome scores • Sino-Nasal Outcome Test (SNOT-20 and SNOT-22) 1. Series of 20 and 22 questions, respectively, developed to assess rhinosinusitis health burden in context of physical, emotional, and functional consequences 2. Scale of 0 to 5, with higher scores indicating greater burden 3. Intended to measure effectiveness of treatment of rhinosinusitis 4. SNOT-22 is modification of SNOT-20, including items related to nasal blockage and smell/taste 5. Strong test-retest reliability; high internal consistency • Rhinosinusitis Disability Index (RSDI) 1. Thirty question items related to rhinologic health, divided into three categories:  physical, functional, and emotional 2. Scores range from 0 (lowest disease burden) to 120 (highest disease burden) 3. Excellent test-retest reliability; good internal consistency • Rhinosinusitis Symptom Inventory (RSI) 1. Used in both sinus surgery and nasal airway surgery 2. Patients rate 12 symptoms on a 6-point Likert scale relating to the last 12 weeks 3. Additional 11 questions regarding medication use, missed work, doctor visits, and impact on functioning 4. Good internal consistency and reliability

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• Rhinosinusitis Quality of Life Survey (RhinoQoL) 1. Seventeen questions divided into three subgroups assessing frequency and severity of sinusitis symptoms 2. Scored from 0 to 100 with higher scores indicative of better health 3. High test-retest scores

Olfactory Testing • Chapter 4 addresses “Olfaction and Taste”

References • Corey J, Pallanch J. Evaluation of nasal breathing function with objective airway testing. In: Richardson MA, Flint PW, Haughey BH, et al. (eds.) Cummings otolaryngology: head and neck surgery. 5th edition. 2010;Chapter 42:640–656. • Deborah S, Prathibha KM. Measurement of nasal mucociliary clearance. Clin Res Pulmonol. 2011;2(2):1019. • Keeler J, Most SP. Measuring nasal obstruction. Facial Plast Surg Clin North Am. 2016;24(3):315–322. • Ottaviano G, Fokkens WJ. Measurements of nasal airflow and patency: A critical review with emphasis on the use of peak nasal inspiratory flow in daily practice. Allergy. 2016;71(2):162–174. • Pallanch J, Jorissen M. Objective assessment of nasal function. In: Flint PW, Haughey BH, Lund VJ, et al. Cummings otolaryngology: head and neck surgery. 6th edition. 2014;Chapter 40:644–657.e4.

Chapter

9 Radiologic Imaging of the Paranasal Sinuses and Skull Base Richard Douglas and Tary Yin

Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Acute Sinusitis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Chronic Sinusitis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Inflammatory Conditions . . . . . . . . . . . . . . . . . . . . . . . .

90 90 91 94 Fungal Sinusitis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 94 Cystic Fibrosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 94 Granulomatosis with Polyangiitis (GPA) . . . . . . . . . . . . . 95 “Wegener’s Granulomatosis” Antrochoanal Polyps . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 96 Mucoceles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 96

Neoplastic Lesions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 96 Benign . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 97 Malignant . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 98 Cerebrospinal Fluid Leak . . . . . . . . . . . . . . . . . . . . . . . . . 100 Image-Guided Surgery . . . . . . . . . . . . . . . . . . . . . . . . . . . 102

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Introduction • Developments in CT and MR imaging techniques have left plain sinus films virtually without clinical value in modern practice. • CT has become the most important imaging modality for most sinonasal cases; the majority of radiological evaluations for paranasal sinuses and sinusitis are based on thin slice bony window scans (1 to 1.5 mm thick sections); these allow a detailed evaluation of the bony sinus anatomy and can also demonstrate sinus opacification secondary to inflammation, secretions, or neoplasms. • MRI excels at characterizing soft tissue changes; this modality determines the nature of lesions (fluid or solid, contrast enhancing or not) as well as the pattern of spread (benign pushing margins or malignant destructive infiltration, including perineural spread); sometimes the histopathological diagnosis can be suggested by the MR appearance but of course not confirmed. For these reasons, MRI is particularly useful in assessing suspected neoplastic disease; its diagnostic performance is further improved with gadolinium contrast enhancement. • Treatment of neoplastic disease may cause changes in anatomy that impede the radiological detection of recurrence; to overcome these problems, PET-CT and advanced forms of MRI (such as dynamic contrast-enhanced MRI and diffusion-weighted MRI studies) may be useful. • CT and/or MR image-guided surgery, which allows real-time intraoperative anatomical localization of surgical instruments based on preoperative imaging, is now in common use for endonasal surgery.

Acute Sinusitis • Acute sinusitis is much more common than chronic sinusitis; radiologic imaging is usually only required to assess for complications, which can be divided anatomically into orbital and intracranial complications. CT allows accurate diagnosis and surgical planning in most cases; however, MRI has better sensitivity for the diagnosis of intracranial and vascular complications. • Preseptal cellulitis involves the eyelid and periorbital soft tissues; CT findings include a thickened orbital septum and increased soft tissue radiodensity. • Postseptal complications, such as periorbital abscess, involve the orbit and are usually caused by acute ethmoidal sinusitis penetrating the lamina papyracea; CT findings include increased

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orbital fat density and fluid collections on the medial or superior orbital wall. Subperiosteal abscesses are rim-enhancing collections between the periorbita and orbital wall; these lesions are well demonstrated by contrast CT or MRI. • Intracranial complications include epidural, subdural, and intracerebral abscesses; these are usually secondary to frontal sinusitis, and most commonly occur in children. MRI is more accurate in assessing dural reaction and cavernous sinus thrombosis; CT findings of subdural and epidural abscess include a convex rim-enhancing collection; however, the appearance of intracranial abscesses varies depending on stage; surrounding cerebral edema can be impressive on both imaging modalities. • Vascular complications, such as thrombophlebitis and thrombosis of the sagittal and cavernous sinuses, can be associated with intracranial abscesses and are also better detected on MRI; flow sensitive imaging techniques such as contrast-enhanced venography can be utilized.

Chronic Sinusitis • Patients with acute sinusitis resolve completely, so their CT scans return to normal; in patients with chronic sinusitis, there are always inflammatory changes on the CT scan even if the patient has few symptoms at the time of scanning; within each affected sinus there may be a combination of mucosal inflammation, polyps, and secretions. • The aim of radiologic imaging in CRS is to secure the diagnosis, assess the extent of mucosal edema, and determine the sinus anatomy (the latter two are in preparation for surgical intervention). • CT is the preferred imaging modality due to its superior bony detail; on CT scans, mucosal inflammation is demonstrated by soft tissue thickening, polyps are lobulated masses with soft tissue attenuation, and secretions may have different radiodensities, depending on their protein composition (Figure 9–1). • Affected areas may include ethmoid infundibulum obstruction causing maxillary sinus disease; ostiomeatal complex obstruction causing maxillary, frontal, and ethmoid sinus disease; and sphenoethmoid recess obstruction causing sphenoid sinus disease. In severe cases, all of the sinuses are involved (pansinusitis) (Figure 9–2). • Anatomical risks factors include infraorbital cells, uncinate process variants, maxillary sinus hypoplasia, nasal septal deviation, concha bullosa, and previous trauma (Figure 9–3). Most of these predispose to CRS by causing narrowing of the sinus drainage pathways.

Figure 9–1.  Bone window CT scans reveal the highly variable pattern of sinus pneumatization. Identification of the frontal sinus drainage pathway requires reconstruction of the pathway in three dimensions, and so studying the axial and sagittal scans is very important. In this case the frontal sinus drainage pathway, which is obstructed by mucosal thickening, can be identified posterior to the agger nasi cell and anterior to the front face of the bulla ethmoidalis.

Figure 9–2.  CT scans reveal the extent of mucosal involvement in cases of CRS, which facilitates surgical planning. In this case of pansinusitis, incidental note of a small osteoma arising from the bulla ethmoidalis is made (arrow). 92

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Figure 9–3.  Careful preoperative reading of the CT scan allows for identification of potential intraoperative hazards. In this case there is an old burst fracture of the right medial orbital wall, which was endoscopically obscured by mucosal thickening of the ethmoids. Failure to recognize this abnormality preoperatively would increase the risk of breaching the displaced lamina intraoperatively. The proximity of the superior oblique and medial rectus muscles to the lamina, and the ease of their injury once a limit is breached, is demonstrated by the arrows.

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• Nasal polyposis mainly affects the nasal cavity, middle meatus, and ethmoid; thinning and displacement of thin sinonasal bones may be evident due to mechanical pressure, inflammatory mediators, and/ or bacterial invasion. • Inflammatory mediators (particularly after sinus surgery) can cause neo-osteogenesis leading to sclerosis of the bony walls and septations.

Inflammatory Conditions Fungal Sinusitis • Fungal sinusitis is a relatively common cause of CRS. • Most cases are non-invasive, in which fungal colonization of the sinus cavity incites an immune response in an immunocompetent patient. • Invasive fungal sinusitis is a life-threatening condition that is fortunately a rare complication of immunocompromising conditions. • Non-invasive fungal sinusitis:  includes two subtypes—allergic fungal sinusitis and fungus ball. In allergic fungal sinusitis, CT scans show involvement of multiple sinuses with opacification, expansion, and relatively radiodense allergic mucin (the difference in radiodensity between the mucin within the sinus and the edematous mucosal lining creates the double density sign, Figure 9–4). Untreated disease results in bony erosion with orbital and intracranial extension by pressure expansion; fungal balls usually involve a single sinus (typically the maxillary or sphenoid). CT findings include sinus expansion, calcification (from the fungal hyphae), and bony erosion (although sometimes hyperostosis can be seen). • Invasive fungal sinusitis:  this condition usually occurs as an acute, devastating consequence of severe immunocompromising conditions; however, in certain parts of the world chronic forms of invasive fungal sinusitis are seen. CT findings of acute invasive fungal sinusitis include bony destruction and soft tissue extension outside of the sinuses; vascular invasion leads to necrotic mucosa that does not enhance with gadolinium on MRI; MRI is the best modality to detect cavernous sinus involvement and orbital, dural, and intracerebral extension.

Cystic Fibrosis • In this condition, chloride transport defects cause high mucus viscosity and impaired ciliary motility; this causes sinus ostial

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Figure 9–4.  A bone window axial CT of a case of fungal sinusitis in which the difference in radiodensity between the fungal debris within the sinus and the polypoid mucosal lining is highlighted by the arrows.

obstruction and results in chronic sinusitis, often with associated sinonasal polyposis. • CT scanning generally shows pansinusitis; the most severely affected sinuses include the maxillary (with bulging medial maxillary walls) and anterior ethmoid sinuses; hypoplasia of the frontal and sphenoid sinuses may also be present, particularly in patients with the delta-F508 cystic fibrosis mutation.

Granulomatosis with Polyangiitis (GPA) “Wegener’s Granulomatosis” • This is a granulomatous necrotizing small vessel vasculitis that causes chronic inflammatory changes on sinus imaging; late stage findings include mucosal granuloma formation that result in mucosal hypointensity on MRI and midline bony necrosis involving nasal septum; granulomas may spread to the skull base and involve cranial nerves. MRI findings suggestive of nerve involvement include nerve thickening and surrounding bony destruction; advanced cocaine abuse leads to a midline pattern of bony destruction similar to GPA but may also involve the adjacent turbinate and palate.

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Antrochoanal Polyps • Antrochoanal polyps originate from the maxillary sinus and grow into middle meatus and choana, usually emerging through accessory ostia in the fontanelle of the medial maxillary wall.

Mucoceles • Mucocoeles result from obstruction of the sinus outflow tract; these lesions are expansile, and can cause pressure dissolution of the surrounding bone (Figure 9–5).

Neoplastic Lesions • Radiologic imaging is crucial in the workup of suspected neoplastic lesions—a combination of CT and MRI is required in most cases. CT scanning defines the extent of the lesion and is sensitive for

Figure 9–5.  A case of bilateral frontal sinus mucocele. The pressure of the expanding mucocele causes dissolution of the surrounding bone.

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bony erosion; MRI provides better soft tissue detail and superior evaluation of orbital, intracranial, and perineural spread; CT, MRI, and CT PET may all be helpful in determining the presence of regional or distant metastatic spread

Benign • Fibro-osseous lesions such as osteoma, fibrous dysplasia, and ossifying fibroma are bony masses best assessed by CT, although they may vary in mineralization and thus radiodensity. Osteoma is the most common benign sinonasal tumor; the majority are small and have limited growth potential, although a minority of cases can grow quite rapidly and achieve a large size. In larger lesions it is possible to identify the hard bone of the cortex and the softer vascular medulla on CT scanning. • Inverted papilloma is a benign epithelial tumor that is most common in middle-aged men; on CT scans they are usually seen as a unilateral polypoid soft tissue mass with non-homogeneous contrast enhancement; bony wall hyperostosis indicates site of attachment and vascular supply (Figure 9–6). MRI scans show a cerebriform pattern of the mass due to the presence of multiple epithelial and stromal layers.

Figure 9–6.  A unilateral soft tissue mass in a middle-aged man. This proved to be an inverting papilloma. Identification of a focal area of bony sclerosis often reveals the point of attachment and vascular supply of the tumor.

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• Juvenile nasopharyngeal angiofibroma are rare fibro-vascular tumors that arise in adolescent males; these tumors originate from the region of the sphenopalatine foramen and can grow in a locally aggressive manner. Contrast CT and MR imaging may show enhancing finger-like projections into the nasal cavity, nasopharynx, maxillary sinus, vidian canal, pterygomaxillary fissure, and orbit and middle cranial fossa. Preoperative arteriography and embolization are very helpful maneuvers to facilitate their safe and complete resection as these tumors are very vascular (Figure 9–7); early follow up imaging with MRI is required due to a high rate of incomplete resection and recurrence. • There are a number of less common benign lesions (eg, hemangioma, leiomyoma, schwannoma); radiologic imaging can be used to assess their composition, vascularity, and extension but is generally unable to provide a specific diagnosis.

Malignant • Most sinonasal malignancies arise from the maxillary and ethmoid regions; there is an impressively wide variety of histopathologies including squamous cell carcinoma, adenocarcinoma, olfactory neuroblastoma, lymphoma, and malignant melanoma. Radiologic imaging is required to assess for invasion and local spread (particularly perineural); periosteum provides a more effective barrier against malignant spread than bone itself; these above features are best demonstrated on MRI, and thus MRI is the imaging modality of choice in the assessment of spread of sinonasal malignancies (Figure 9–8). • Maxillary malignancy:  imaging must assess for invasion of the posterior maxillary sinus wall, infratemporal and pterygopalatine fossa, hard palate, and orbital floor; spread into the pterygopalatine fossa is suspected when soft tissue signal replaces the fat of the fossa. • Ethmoid malignancy:  images must be assessed for invasion of the orbit, anterior cranial fossa, and sphenoid sinus. MRI is more accurate at detecting orbital and periobital invasion; involvement of these structures is very important to determine accurately preoperatively as orbital involvement may necessitate orbital exenteration. MRI is usually superior to CT in assessing for skull base invasion; the detail of soft tissue involvement enables categorization of the invasion into extracranial, intracranialextradural (bone-periosteum penetration), intracranial-intradural invasion (thickened and enhanced dura), and brain invasion (edema).

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Figure 9–7.  A case of a JNA, which demonstrates involvement of the pterygopalatine and infratemporal fossae. The vascularity of the tumor is indicated by the small flow voids on this T2-weighted MR image, and confirmed by the arteriogram. The mucocele in the left sphenoid has a higher radiodensity because of its greater water content (arrow).

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Figure 9–8.  A sinonasal undifferentiated carcinoma, a highly aggressive tumor. The MR scan shows dural enhancement and involvement of the right orbit. The benign polyp within the right maxillary sinus does not enhance.

• Follow-up imaging post surgical and radiotherapy treatment may be complicated by altered anatomy that may have similar features to recurrent disease; CT PET, which detects the increased metabolism of neoplastic tissues, may be very helpful in this circumstance.

Cerebrospinal Fluid Leak • Cerebrospinal fluid leaks may be traumatic or spontaneous; diagnosis involves detection of β2-transferrin in the rhinorrhea sample; the aim of radiologic imaging is to detect any underlying defects or lesions and facilitate surgical planning. These goals are usually achieved by thin slice bony window CT; however, MRI is indicated if a meningocele or encephalocele is suspected; both these modalities may pick up the relatively subtle signs of raised intracranial pressure; this condition underlies most patients with spontaneous CSF leaks.

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• CT and MR cisternography are additional modalities that can be used to detect active CSF leaks; these require administration of lumbar intrathecal contrast and imaging of the patient in Trendelenburg position. They may be considered for complex or small active leaks where CT and MRI are negative, however, are contraindicated in meningitis and raised intracranial pressure; usually if a combination of CT and MRI does not identify the source of the leak, intrathecal contrast does not either. • CT findings include bony defects, air-fluid levels, and adjacent sinus or mastoid/middle ear opacification (temporal bone fractures can cause CSF leak that presents as rhinorrhea due to passage of fluid through the Eustachian tube). • MRI findings for meningocele and encephalocele include extracranial subarachnoid CSF extension, extracranial meningeal extension, and/or brain parenchyma herniation; gadolinium improves discrimination of meningocele from secretion and detection of dural enhancement. • Traumatic:  includes fractures and surgical complication; skull base traumatic defects can be located in the anterior cranial fossa (cribriform plate, ethmoid, and frontal sinus), central skull base (sphenoid sinus), and lateral skull base (tegmen tympani). • Spontaneous:  this includes congenital (meningocele, encephalocele, arachnoid granulation, and inner ear anomalies) and neoplastic causes (via bony invasion or secondary to raised intracranial pressure from hydrocephalus) (Figure 9–9). CSF

Figure 9–9.  This patient presented with persistent right-sided rhinorrhea that was β2-transferrin positive. The bony defect, caused by a small meningocele, was found to be in the lateral wall of the sphenoid immediately superior to the foramen rotundum.

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leak may also be secondary to decrease in tumor size from chemotherapy or radiotherapy, revealing base of skull defects; chronically raised intracranial pressure leads to lateral ventricular dilatation anteriorly and CSF leakage usually through the cribriform plate—and sometimes through lateral recesses in the sphenoid sinus.

Image-Guided Surgery • Endoscopic sinus surgery anatomy is hazardous due to the proximity of the orbit and medial skull base; potential complications include orbital hematoma, CSF leak, carotid injury, and intracranial injury; although complication rates are low, morbidity is severe, and this has led to the adoption of imageguidance in sinus and skull base surgery. • Image-guided surgery utilizes preoperative CT or MRI with multiplanar reconstructions and associated image-guidance tracking software; this allows real-time intraoperative anatomical localization of tracking surgical instruments. Additional components of image-guided surgery include a computer workstation, display monitor, and tracking system (utilizing either an electromagnetic field or infrared light camera for detection of the probe or instrument in space) (Figure 9–10). • To link the preoperative imaging with the live patient, registration is required; this involves matching a patient’s anatomical markers to radiologic markers. If the registration accuracy is less than ideal, the patient should be re-registered; patient and headset movement during surgery can also affect accuracy.

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Figure 9–10.  A screenshot of a CT navigation device. The position of the olivetipped suction, touching the defect in the fovea ethmoidalis being repaired with a fat plug, is revealed in three planes.

Chapter

10 Diagnosis and Classification of Rhinosinusitis Abtin Tabaee and Edward D. McCoul

Diagnosis of Rhinosinusitis . . . . . . . . . . . . . . . . . . . . . . 106 Classification According to Time Course . . . . . . . . . . . . 106 Diagnosis of Acute Rhinosinusitis . . . . . . . . . . . . . . . . . . 106 Diagnosis of Chronic Rhinosinusitis . . . . . . . . . . . . . . . . 107 Classification of Rhinosinusitis . . . . . . . . . . . . . . . . . . . . 110 Clinical Approach to Diagnosis . . . . . . . . . . . . . . . . . . . . 112

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Diagnosis of Rhinosinusitis Classification According to Time Course Acute Rhinosinusitis (ARS) • Sinusitis symptoms and objective evidence of inflammation fewer than 4 weeks

Subacute Rhinosinusitis • Sinusitis symptoms and objective evidence of inflammation between 4 and 12 weeks

Chronic Rhinosinusitis (CRS) • Sinusitis symptoms and objective evidence of inflammation 12 weeks or longer

Recurrent Acute Rhinosinusitis • Four or more distinct ARS events in a 12-month period with complete clinical resolution between acute episodes

Chronic Rhinosinusitis with Acute Rhinosinusitis Exacerbation • Acute worsening of sinonasal symptoms in the setting of baseline CRS

Diagnosis of Acute Rhinosinusitis Task Force on Rhinosinusitis of AAO-HNS (1997) • Symptoms for 4 weeks or less • Four major criteria: 1. Nasal obstruction/congestion 2. Rhinorrhea or post-nasal discharge 3. Reduced sense of smell 4. Facial pain/pressure/fullness (secondary criterion) 5. Fever (secondary criterion) • Six minor criteria: 1. Headache 2. Halitosis

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3. Fatigue 4. Dental pain 5. Cough 6. Ear pain/pressure/fullness • Diagnosis requires two major symptoms, or one major and two minor symptoms; secondary criteria only “count” if accompanied by another major criterion. • Purulent nasal secretions on exam confirms the diagnosis.

Clinical Practice Guidelines on Adult Rhinosinusitis (2015) • Revised to simplify diagnosis • Symptoms for 4 weeks or less • Purulent nasal drainage (anterior, posterior, or both) accompanied by nasal obstruction, facial pain-pressure-fullness, or both • Viral rhinosinusitis:  symptoms/signs of ARS fewer than 10 days; symptoms not worsening • Acute bacterial rhinosinusitis (ABRS):  symptom/signs of ARS for 10 days or more, or double worsening (worsen within 10 days after initial improvement) • Purulent nasal secretions on exam • Transillumination not useful • Diagnosis is clinical • Imaging not indicated unless complication suspected

Canadian Rhinosinusitis Guidelines (2011) • Table 10–1 contains the guidelines.

Diagnosis of Chronic Rhinosinusitis Task Force on Rhinosinusitis of AAO-HNS (1997) • Symptoms for 3 months or longer • Two of the following major criteria are required: 1. Nasal obstruction/congestion 2. Thick rhinorrhea or post-nasal purulent discharge 3. Facial pain/pressure 4. Decreased sense of smell (hyposmia) • Seven minor criteria: 1. Headache 2. Halitosis 3. Fever

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Hyposmia/anosmia (Smell)

S

Mild Occasional limited episode

Moderate Steady symptoms but easily tolerated

Severe Hard to tolerate and may interfere with activity or sleep

*For diagnosis of ABRS, patient must have: (1) nasal obstruction OR nasal purulence/discolored postnasal discharge, AND (2) at least one other PODS symptom.

Consider ABRS under any one of the following conditions: 1. Worsening after 5 to 7 days (biphasic illness) with similar symptoms. 2. Symptoms persist more than 7 days without improvement. 3. Presence of purulence for 3 to 4 days with high fever.

Nasal purulence/discolored post-nasal Discharge

Nasal Obstruction

O

D

Facial Pain/pressure/fullness

P

Major ABRS Symptoms

None

Table 10–1.  ABRS Diagnosis Requires the Presence of At Least Two Major Symptoms*

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4. Fatigue 5. Dental pain 6. Cough 7. Ear pain/pressure/fullness • Diagnosis requires two major symptoms, or one major and two minor symptoms • Also requires one finding that confirms inflammation:  mucosal thickening on CT scan; nasal polyps on rhinoscopy; or polyps, purulence, or edema on nasal endoscopy

Clinical Practice Guidelines on Adult Rhinosinusitis (2015) • Revised to simplify diagnosis • Twelve weeks or longer of two or more signs/symptoms: 1. Mucopurulent drainage (anterior, posterior, or both) 2. Nasal obstruction/congestion 3. Facial pain-pressure-fullness 4. Decreased sense of smell • AND inflammation is documented by one or more findings: 1. Purulent (not clear) mucus or edema in middle meatus or anterior ethmoid region 2. Polyps in nasal cavity or middle meatus 3. Radiographic imaging showing sinus inflammation • Need to confirm presence or absence of polyps

European Position Paper on Rhinosinusitis and Nasal Polyps (EPOS 2012) • Defined as inflammation in the nose and paranasal sinuses

Definition in Adults • Two or more symptoms, one being either nasal blockage/congestion or nasal discharge (anterior/posterior), +/− facial pain or pressure, +/− reduction or loss of smell • And either: 1. Endoscopic signs of nasal polyps, mucopurulent discharge primarily from middle meatus, or edema/mucosal obstruction primarily in middle meatus 2. CT showing mucosal changes within the OMC and/or sinuses • Subdivided into CRS with polyps (CRSwNP) and without polyps (CRSsNP)

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• CRSwNP:  bilateral endoscopically visualized polyps in middle meatus • CRSsNP:  no visible polyps in middle meatus (if necessary following decongestant)

Definition in Children • Substitutes +/− cough instead of +/− reduction or loss of smell • Otherwise criteria are the same

Canadian Rhinosinusitis Guidelines (2011) • Table 10–2 shows the guidelines.

Classification of Rhinosinusitis Chronic Rhinosinusitis • Multiple proposals

Phenotype Based on Asthma and Atopy Status

(Han JK, Laryngoscope. 2013;Mar;123 Suppl2:S15–27) • Non-asthmatic CRS w/o allergy:  purulence, low CT score, no polyps, Th1 profile, PMNs, IL-6, IL-8 • Non-asthmatic CRS with allergy:  small polyps, less purulence, moderate CT score, eosinophils/mast cells, interferon-gamma • Asthmatic CRS with allergy:  large polyps, no purulence, high CT score, severe atopy, Th2 profile, IL-5, eos; “unified airway disease” • Asthmatic CRS w/o allergy:  large polyps, no purulence, high CT score, adult-onset asthma, minimal atopy, Th2 profile, eos; possible AERD precursor • Aspirin-exacerbated respiratory disease (AERD): abundant polyps, no purulence, high CT score, adult-onset asthma, NSAIDinduced bronchospasm, IL-4, eos, urine LTE4 • Allergic fungal sinusitis (AFS):  may be unilateral, large polyps, some purulence, allergic mucin, high CT score, IL-5, IgE

Emerging Classification Schemes • Phenotyping by other clinical data:  age, lost productivity, patientreported symptom scores • Endotyping according to cellular and molecular biomarkers • Microbiome composition, diversity, growth patterns • Genotyping of receptors, factors of innate immunity

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Purulent anterior/posterior nasal Drainage

Hyposmia/anosmia (Smell)

D

S

Mild Occasional limited episode

Moderate Steady symptoms but easily tolerated

Severe Hard to tolerate and may interfere with activity or sleep

*A diagnosis requires at least two CPODS, present for 8 to 12 weeks, plus documented inflammation of the paranasal sinuses or nasal mucosa.

CRS is diagnosed on clinical grounds but must be confirmed with at least one objective finding on endoscopy or CT scan.

Nasal Obstruction/blockage

Facial Pain/pressure/fullness

P

O

Facial Congestion/fullness

C

Major Symptoms

None

Table 10–2.  CRS Diagnosis Requires the Presence of At Least Two Major Symptoms*

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Fungal Rhinosinusitis • Classified according to pathology findings including tissue invasiveness, microbiology, radiographic findings, and host immunologic response; diagnosis may require clinical evaluation, imaging (CT scan), culture, and pathology

Allergic Fungal Sinusitis • Non-invasive organism with host hypersensitivity response • Bent and Kuhn criteria: 1. Type I hypersensitivity to molds/fungus 2. Nasal polyposis 3. Characteristic CT findings (typical pattern of opacification with areas of hyperdensity) 4. Eosinophilic mucus 5. Positive fungal smear without tissue invasion

Mycetoma (Fungus Ball) • Non-invasive organism without host hypersensitivity response • May have non-atopic local inflammation

Chronic Invasive Fungal Sinusitis • Invasive organism with immunocompetent host • Indolent

Acute Invasive Fungal Sinusitis • Invasive organism with immune system impairment • Life-threatening

Clinical Approach to Diagnosis Patient History General History • Past medical/surgical history, medications, allergies to medications, family history, social habits including tobacco, alcohol, illicit drug use, occupation • Detailed history for possible sinusitis cofactors including allergic rhinitis, asthma, immune deficiencies • Family history of sinusitis/nasal polyps • History of nasal trauma, sinonasal or rhinoplasty procedures • Review of prior diagnostic tests including radiographic studies (both images and reports), allergy test results, pulmonary testing

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• Obtain operative reports if available, especially for history of sinus surgery

Symptom Characterization • Duration, severity, fluctuation, location, quality, change in time, response to treatment, modifying factors, prior similar symptoms in the past

Sinonasal Symptoms • Nasal congestion/obstruction, facial pressure/pain, rhinorrhea, post-nasal drip, hyposmia, epistaxis • Review of prior medications used and treatment response

Non-Sinonasal Symptoms • Ear pain/pressure/popping/fullness, hearing loss, cough, shortness of breath, wheeze, throat pain, voice changes, fever (uncommon)

Red Flag Symptoms • Alerts the clinician to atypical process • Unilateral symptoms, facial swelling, facial numbness, ocular symptoms (vision loss/ diplopia), older age at first presentation, significant epistaxis

Physical Examination General Otolaryngology Exam • Thorough head and neck exam with a focus on the nasal cavity • Correlate ear, throat, chest, and voice symptoms with respective exam findings

Nasal Cavity Examination • Use headlight and nasal speculum prior to topical decongestant/ anesthesia spray • Assess position of anterior nasal septum, health of the mucosal tissue, hypertrophy of the inferior turbinates, and presence of nasal secretions • Re-examination following topical spray allows for assessment of soft tissue inferior turbinate hypertrophy

Nasal Endoscopy Procedure • Flexible, straight rigid, angled rigid provide complimentary perspectives • Rigid scopes allow for culture, debridement, biopsy, office procedures

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• Topical decongestant/anesthesia allows for better patient tolerance • Recording systems allow for exam archiving for comparison of endoscopies at different times and allow for educational review of endoscopy video/images with the patient • Sites of examination: 1. Nasal septum 2. Nasal cavity 3. Inferior turbinate 4. Middle turbinate and meatus 5. Sphenoethmoid recess 6. Nasopharynx • In postoperative patients, patent paranasal cavities may be directly visualized. • Nasal endoscopy findings:  mucosal changes (edema, erythema, polypoid changes), secretions (clear, mucopurulent, inspissated, crusting), lesions/masses, sinonasal structural findings (nasal septum position, surgical outflow tract patency)

Endoscopic Guided Culture • Similar yield as traditional maxillary antral tap • Performed with endoscopic visualization and placement of culture swab in an area of mucopurulence within a paranasal sinus or outflow tract (middle meatus/sphenoethmoid recess) • Nasal cavity swabs not reliable for sinusitis

Nasal Endoscopy with Biopsy • Office-based procedure to provide pathologic evaluation of a suspicious soft tissue lesion • Should be performed after imaging • Contraindicated if lesion appears vascular, of intracranial origin, or intimately related to critical neurovascular structures • Potential for false negative exists and additional biopsy, including in the operating room, should be considered for suspicious lesions

Future Diagnostic Tools • No clinically available test currently exists for biofilm, microbiome, genetic predisposition, and other pathophysiologic factors.

Imaging Primary Indications • Characterization of the pattern and severity of CRS in a patient who has failed medical therapy

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• Provides anatomic information • Used for surgical planning • Excludes other possible issues

Secondary Indications • Paranasal sinus disease in a patient with a non-specific symptom (eg, headache) • Possible complication from sinusitis (eg, orbital/intracranial infection) • Atypical sinonasal symptoms and/or exam findings (eg, neoplasm) • Bony lesions (eg, fibrous dysplasia, osteoma) • Role in recurrent acute rhinosinusitis unclear; provides evaluation of anatomy; can be used for surgery planning, rules out CRS if performed between ARS events; characterizes pattern of inflammation if performed during ARS event

Computed Tomography (CT) • Non-contrast study, triplanar views, bone window most useful for CRS • Soft tissue window can sometimes characterize areas of opacification • Contrast study selectively used to assess vascularity in atypical events (orbital/ intracranial spread of infection, neoplasm) • Computed tomography findings:  mucosal disease (mucosal swelling, opacification, polypoid changes), secretions (air-fluid levels, bubbly secretions), soft tissue lesions, bony lesions, normal and aberrant sinonasal anatomy including outflow tract patency

Magnetic Resonance Imaging (MRI) • Selectively used for evaluation of atypical process including soft tissue neoplasm, intraorbital and intracranial involvement of paranasal sinus disease

Paranasal Sinus Plain Films • Used historically • Lack specificity, sensitivity, anatomic detail; largely replaced by CT • Waters (occipitomental) view:  maxillary sinus and limited frontal and ethmoid sinuses • Caldwell (occipitofrontal) view:  frontal sinus and inferior aspect of maxillary sinus • Lateral view:  sphenoid and frontal sinuses • Submental vertex view:  sphenoid sinus

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Other Assessments Histopathology • Typically obtained in the setting of surgery • May be used to characterize CRS including polypoid versus nonpolypoid, the pattern of inflammatory cells (eosinophil versus neutrophil predominant) • Important in characterization of fungal sinusitis variants including presence of fungus, tissue invasion, eosinophil break down products (Charcot-Leyden crystals) in AFS, inflammatory changes • Diagnose neoplasm, inflammatory disorders (eg, granulomatosis with polyangiitis)

Adjunctive Testing • Indicated for evaluation of possible co-factors, alternative disorders, underlying primary systemic etiologies, complications of sinusitis:  allergy testing, immunology evaluation, rheumatology evaluation, pulmonary function testing, ophthalmology evaluation (eg, orbital complications of sinusitis), neurology evaluation (eg, primary headache symptoms), neurosurgery evaluation (eg, intracranial spread of sinus infection)

References • Desrosiers M, Evans GA, Keith PK, et al. Canadian clinical practice guidelines for acute and chronic rhinosinusitis. J Otolaryngol Head Neck Surg. 2011 May; 40 Suppl 2:S99–193 • Fokkens WJ, Lund VJ, Mullol J, et al. European position paper on rhinosinusitis and nasal polyps 2012. Rhinol Suppl. 2012 Mar;23(3), 1–298. • Rosenfeld RM, Piccirillo JF, Chandrasekhar SS, et al. Clinical practice guidline (update): adult sinusitis. Otolaryngol Head Neck Surg. 2015 Apr;152(2 Suppl):S1–S39.

Section

IV Sinonasal Diseases

Chapter

11 Epistaxis and Its Management Garret W. Choby and Zara M. Patel

Background . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 120 General . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 120 Anatomy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 121 Work-Up . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 123 Classification . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 123 Systemic Considerations . . . . . . . . . . . . . . . . . . . . . . . . . . 124 Management . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 124 Initial Management . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 124 Intervention . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 126

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Background General Frequency • Epistaxis is one of the most commonly encountered conditions among otolaryngologists. 1. The true incidence of epistaxis is unknown as most patients with self-limited bleeds do not present to a physician for care. • The majority of cases are non-life threatening and self-limiting. • Epistaxis occurs more commonly in a biphasic age distribution. 1. Children – Likely due to digital trauma 2. Elderly – Likely due to nasal mucosal dryness and use of anticoagulants

Etiology • A variety of factors are believed to contribute to epistaxis. 1. Nasal trauma – May range from minor digital trauma in children to traumatic facial injuries with arterial rupture 2. Intranasal drug use – Improper use of medicated nasal sprays – Intranasal drugs of abuse (ie, cocaine, etc) 3. Dry climates and cold weather 4. Anatomic variants – Septal deviation:  sharp spurs may lead to abnormal airflow, which may dry mucosa – Septal perforations:  change in airflow and loss of humidifying mucosal surface leads to dryness and crusting, which secondarily bleeds 5. Systemic – Anticoagulation (aspirin, warfarin, etc) – Liver disease or other disease processes leading to dysfunction of clotting factors – Thrombocytopenia or other disease processes leading to dysfunction of platelets – Hypertension 6. Tumors – Sinonasal tumors, either benign or malignant, may have epistaxis as a presenting symptom

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– Juvenile nasal angiofibromas (JNA) classically present with heavy epistaxis in a teenage male 7. Vascular anomalies – Hereditary hemorrhagic telangiectasia (HHT) causes recurrent epistaxis from vascular anomalies in the sinonasal mucosa. These telangiectasias lack elastic or smooth muscle and therefore are prone to bleeding and resistant to topical decongestant medications; HHT may also affect the gastrointestinal and pulmonary tracts. – Hemangiomas or arteriovenous (AV) malformations can present in the nasal cavity. – Carotid artery aneurysms and pseudoaneurysms dehiscent into the sphenoid sinus can present with a brief, significant but self-limited, episode of epistaxis as a sentinel bleed.

Anatomy Vascular Contributions • The nasal mucosa is rich in vascularity. • External carotid artery (ECA) contributions 1. Internal maxillary artery – Enters the pterygopalatine fossa (PPF) and branches into the following arteries: a. Descending palatine ■ Branches into the greater and lesser palatine arteries ■ Helps to supply lateral nasal wall and anterior septum b. Infraorbital c. Posterior superior alveolar d. Pterygoid canal e. Pharyngeal f. Sphenopalatine artery (SPA) ■ Most common source of arterial epistaxis ■ Supplies the turbinates, lateral nasal wall and surrounding sinuses, and the posterior septum 2. Facial artery – Terminal branch at the superior labial artery a. Supplies anterior septum and anterior floor of nose • Internal carotid artery (ICA) contributions 3. Ophthalmic artery – Enters into the orbit through the superior orbital fissure and branches:

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a. Anterior ethmoid artery (AEA):  enters from orbit into the nose through the anterior ethmoid foramen (located ~24 mm posterior to the anterior lacrimal crest) ■ Traverses the roof of the ethmoids in a posterolateral to anteromedial direction ■ May be contained within the bony skull base or in a mesentery hanging from the skull base (more prone to injury causing iatrogenic epistaxis and possible orbital hematoma during sinus surgery) ■ AEA can typically be identified on coronal CT scan, one slice posterior to the globe with a bony triangular evagination of the medial orbital wall (Figure 11–1). b. Posterior ethmoid artery (PEA):  enters from the orbit into the nose through the posterior ethmoid foramen (located ~36 mm posterior to lacrimal crest and ~6 mm anterior to the optic canal) – These two arteries cannot usually be embolized due to risk of blindness and stroke so should be managed with surgical ligation if they are the source of epistaxis. • Kisselbach’s plexus 1. Located within the septal mucosa covering the anterior cartilaginous nasal septum

Figure 11–1.  Coronal CT scan demonstrating location of AEA inferior to the skull base (arrows indicate bilateral AEA).

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2. Responsible for the majority of epistaxis (>90%) 3. Anastomotic area of vessels with contributions from both the ICA and ECA • Woodruff ’s area 1. Venous plexus located in the posterior nasal cavity along the lateral nasal wall near the root of the inferior turbinate

Work-Up Classification Anatomic Classification • The most important step in management of epistaxis is identifying the exact site of bleeding. • Careful examination with an endoscope will allow for accurate characterization of bleeding in most cases. 1. Suctioning old clots and debris is necessary for proper visualization. 2. This step in work-up will allow for targeted therapy at the site of bleeding, as opposed to an ineffective and complication producing “shotgun” technique of applying cautery or packing to the entire nose. • Anterior epistaxis 1. Thought to be the source in ~95% of epistaxis cases, with the majority occurring near Kisselbach’s plexus 2. Tend to present as smaller or slower bleeding episodes that respond to anterior pressure unless comorbidities are present • Posterior epistaxis 1. Although less frequent, posterior bleeds tend to be arterial in nature and result in brisk bleeding. 2. The majority of posterior bleeds are from branches of the SPA. 3. A heavy posterior arterial bleed can quickly lead to airway compromise and significant blood loss.

Classification of Bleeding Type • Just as important as defining an anatomic location is determining an arterial versus venous bleed. • Contrary to popular belief, anterior epistaxis may still be arterial, arising from the AEA or greater palatine artery via the incisive foramen.

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• Similarly, although posterior epistaxis is typically thought to be arterial, it can also be venous in nature, with bleeding occurring at Woodruff ’s plexus or another venous contribution. • Distinguishing arterial bleeding from venous bleeding may significantly alter treatment plans.

Systemic Considerations Laboratory Tests • In minor bleeds, no laboratory tests are necessary. • In heavy or recurrent episodes, a complete blood count and bleeding profile may be warranted. • In patients on anticoagulation, it is helpful to know their PT/PTT/INR levels, and with newer anti-platelet therapies now on the market, whether their anticoagulant is a reversible or irreversible agent. • Patients with hematologic malignancies or those undergoing chemotherapy may have thrombocytopenia, thus predisposing them to epistaxis. • In patients with family history of epistaxis, laboratory tests to evaluate for more rare familial disorders (hemophilia, etc) may be warranted.

Hypertension • The relationship between hypertension and epistaxis is poorly understood. • In certain situations, extreme hypertension may manifest itself as epistaxis; more often, however, a patient’s blood pressure tends to become elevated when epistaxis occurs, causing an increase in blood loss. • If the blood pressure is significantly elevated, treatment is warranted.

Management Initial Management Initial Considerations • The majority of epistaxis episodes are mild and self-limited.

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• When heavy uncontrolled bleeding occurs, prompt treatment is vital. 1. Airway protection is paramount. 2. If an arterial nosebleed causes airway compromise, strong consideration should be given to securing the airway prophylactically. 3. Early vascular access and intravenous fluid administration is important. 4. A type and screen should be obtained. • The patient should be seated with a forward lean to prevent blood from entering the airway and also to prevent the patient from swallowing a large amount of blood, which will lead to eventual emesis. 1. A Yankauer suction should be provided to evacuate clots from the oropharynx. 2. Topical decongestant medication should be applied and hard pressure held over the cartilaginous portion of the nose for at least 5 to 10 minutes. • Universal protective clothing should always be worn, including a facemask; the risk of bloody contamination from patient coughing or suctioning is high.

Evaluation • Examination should be carried out with both a speculum and headlight, as well as endoscope. 1. Simple anterior sites of bleeding may be visualized and treated with a speculum. 2. Endoscopes are useful for more posterior bleeds and to ensure that other etiologies or sources are not missed. – Endoscopic exam is crucial in young males with epistaxis to rule out the presence of a JNA. 3. Old clots should be carefully evacuated with a nasal suction to allow adequate visualization. – Identifying the specific site of bleeding is the most important step in treating epistaxis. 4. If bleeding is too brisk to identify the exact site accurately, the suspected area may be temporarily packed with a cottonoid, soaked in decongestant, to slow the bleeding; it can then be removed and the area re-examined.

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Intervention Nasal Cautery • In simple anterior bleeds, silver nitrate cautery may be carried out under headlight or endoscopic guidance. • Cautery should be focal. 1. Cauterizing multiple opposing sites in one nasal cavity may lead to synechiae formation. • In general, bilateral cautery on opposing sides of the nasal septum is avoided due to risk of perforation from lack of vascularity to the cartilaginous septum. • Avoidance of accidental cautery of skin of the ala or nasal sill should be avoided by visualizing the silver nitrate stick entering the nose with the endoscope. • If available, electrocauterization may also be considered but is generally less tolerable in the awake patient.

Nasal Packing • If bleeding is uncontrolled or too brisk for cautery, nasal packing is an option. • If possible, packing should also be directed at the specific site of bleeding. • Absorbable packing 1. A number of commercially available products are available for use. 2. Many products are designed to stimulate the coagulation cascade to control bleeding as opposed to traditional packing techniques largely designed simply to apply direct pressure. – Available products include microfibrillar collagen, oxidized cellulose, and human-derived thrombin solutions. • Non-absorbable packing 1. Non-absorbable packing is useful when cautery and/or absorbable packing has failed. 2. Again, the packing should be directed at the site of bleeding whenever possible. 3. The traditional material of choice was layered ribbon petroleum-impregnated gauze; this has largely fallen out of favor, however, due to its time-intensive nature and patient discomfort.

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4. Merocel® sponges (Medtronic Corporation, Jacksonville, FL) are a good option due to their mechanical tamponade of bleeding areas as well as the ability to absorb and wick topical products, such as decongestants, to the site of bleeding. 5. A variety of balloon-type packing is also available; these products tend to be easily inserted but cause significant patient discomfort when expanded; designs are available for anterior or anterior/posterior balloon packs. – Another option for posterior bleeding is a Foley catheter balloon. It is passed through the nasal passage and inflated in the nasopharynx and seated forward towards the choanae; it is then secured anteriorly. If necessary, an additional pack can then be placed anterior to it. – It is imperative to protect the nasal ala from pressure necrosis while the Foley is in place and secured with an anterior clip. – A posterior balloon pack carries the risk of stimulating the nasopulmonary reflex causing hypercarbia and hypoxia; therefore, hospital admission, with continuous pulse oximetry and telemetry, is recommended. 6. Although controversial as to the necessity of this measure, most physicians prescribe prophylactic antibiotics while packing is in place to help prevent secondary sinusitis and theoretically reduce the risk of toxic shock syndrome.

Medical Interventions • Control blood pressure and control pain that could cause elevation in blood pressure. • If patient is on a reversible anticoagulant and is experiencing life threatening bleeding, a reversing agent should be given. • If significant blood loss has been sustained, whole blood and platelet or clotting factor transfusion should be given based on respective laboratory values. • If patient is undergoing treatment such as chemotherapy that is causing recurrent life threatening epistaxis, modulation of therapeutic course should be considered.

Surgical Management • Endoscopic SPA ligation (ESPAL) 1. This technique has been shown to be very effective in managing posterior arterial epistaxis.

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– Endoscopically, an incision is made in the lateral nasal wall just posterior to the fontanelle of the maxillary sinus (near the lateral attachment of the middle turbinate). – A mucosal flap is elevated back to the crista ethmoidalis. – The SPA is identified and ligated with a clip or electrocautery. a. It is crucial to ensure that all branches are addressed (Figure 11–2). b. Some authors recommend entering the medial PPF to ensure the artery has not branched before entering the SPA foramen. – The use of ESPAL as first-line treatment for posterior arterial epistaxis has been shown to increase patient comfort and reduce cost compared to posterior packing with hospital admission. • AEA and PEA ligation 1. If the site of bleeding is suspected to arise from the AEA or PEA, endoscopic or open approaches can be utilized. – Lynch incision

Figure 11–2.  Endoscopic view of two branches of SPA exiting the sphenopalatine foramen.

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a. An incision is created between the medial brow and medial canthus along the curve of the browline, and dissection is carried down to a subperiosteal plane and then carried posteriorly. ■ Maintaining this plane is important to prevent prolapse of orbital fat into the operative field. b. The AEA is classically found ~24 mm behind the lacrimal crest. c. The PEA is classically found ~36 mm behind the lacrimal crest. d. The artery can be ligated with a surgical clip or with bipolar cautery. – Endoscopic approach a. These arteries can also be accessed endoscopically as they cross the ethmoid roof. b. The arteries may be encased in the bony skull base or may hang from a mesentery. c. The AEA may be found posterior to the frontal outflow tract, crossing the skull base from posterolateral to anteromedial, typically in the posterior skull base attachment of the cell just posterior to the frontal sinus (sometimes a supraorbital ethmoid cell, sometimes simply a superior ethmoid cell attaching to skull base). d. The PEA is typically located near the junction of the planum sphenoidale and the cribriform region. e. Again, surgical clips or bipolar cautery may be used. • If bleeding etiology is a sinonasal tumor appropriate for surgical resection, complete resection with ligation of feeding vessels is the best treatment strategy for definitively managing epistaxis. 1. If tumor is highly vascular (eg, JNA), preoperative embolization may be warranted.

Endovascular Embolization • Arterial bleeding from the ECA system may be managed with embolization. • In general, embolization is used as a second line treatment for those patients who are poor surgical candidates or in those for whom surgery has failed. 1. Embolization has been demonstrated to be more expensive than ESPAL and carries a remote risk of stroke. • Pre-procedure angiography may be used to help identify the site of bleeding; in some instances, the ipsilateral internal maxillary

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artery and facial artery are embolized (facial soft tissue necrosis is a known risk of facial artery embolization). • As previously noted, this technique cannot usually be used for the AEA or PEA due to risk of blindness and/or stroke.

Interventions for HHT • HHT is characterized by small clusters of arteriovenous malformations that lack intervening capillaries and contractile elements. • Simple endoscopic cautery in the operating room may be effective for some patients. • Bevacizumab is an antibody to VEGF which helps to inhibit angiogenesis. 1. Current studies are underway evaluating the efficacy of topical and injected bevacizumab for these patients. • Laser photocoagulation 1. KTP is felt to be the most appropriate laser choice. 2. Its 532 nm wavelength (green electromagnetic spectrum) is effective for hemostasis. • Septodermoplasty 1. This is reserved for recalcitrant cases. 2. The anterior nasal septal mucosa is removed to the level of the perichondrium and replaced with a split thickness skin graft (STSG). – Mucosa may be removed with a microdebrider or with traditional elevation. – STSG is secured with sutures. 3. Tends to have good initial success, but bleeding recurs as new lesions form. • Young’s procedure 1. Radical procedure of last resort to close the nose. 2. Flaps are raised in the nasal vestibule and sutured closed. 3. In some cases, the outcome is poorly tolerated due to inability to breathe through the nose, but this is largely successful in preventing further epistaxis from HHT.

References • Dedhia RC, Desai SS, Smith KJ, et al. Cost-effectiveness of endoscopic sphenopalatine artery ligation versus nasal packing as first-line treatment for posterior epistaxis. Int Forum Allergy Rhinol. 2013;3(7):563–566. doi:10.1002/alr.21137.

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• Rudmik L, Leung R. Cost-effectiveness analysis of endoscopic sphenopalatine artery ligation vs arterial embolization for intractable epistaxis. JAMA Otolaryngol-Head Neck Surg. 2014;140(9):802–808. doi:10.1001/jamaoto.2014.1450.

Chapter

12 Allergic and Nonallergic Rhinitis Ashleigh A. Halderman

Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 134 Allergic Rhinitis (AR) . . . . . . . . . . . . . . . . . . . . . . . . . . . . 134 Clinical Presentation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 135 Physical Exam . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 135 Work-Up . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 138 Medical Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 139 Surgical Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 143 Non-Allergic Rhinitis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 143 Clinical Presentation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 144 Review of Medications . . . . . . . . . . . . . . . . . . . . . . . . . . . . 145 Physical Exam . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 146 Work-Up . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 147 Medical Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 147 Surgical Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 148 NAR Subtypes . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 149 Conclusion . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 151

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Introduction • Rhinitis (allergic or non-allergic) is one of the most frequently encountered diseases evaluated and treated by Otolaryngologists. There is tremendous overlap in symptoms between the various forms of rhinitis, and reaching an accurate diagnosis can be challenging. Therefore, practitioners must be well versed in how the various forms of rhinitis differ in presentation, pathophysiology, and treatment to achieve the best results for their patients.

Allergic Rhinitis (AR) • One of most common conditions affecting an estimated 500 million people worldwide. • Reported prevalence of between 10% and 30% of the adult population and up to 40% in children. • It is defined as an IgE mediated type I hypersensitivity response within the nasal mucosa. • The reason some patients become sensitized to allergens and thus go on to develop allergies is not completely understood and is likely multifactorial. • Upon initial exposure, an antigen is taken up and processed by antigen-presenting cells (APC) in the nasal mucosa; these processed antigens are then presented to and recognized by major histocompatibility complex class II molecules. APCs also attract CD4+ T cells and, under the influence of interleukin (IL)-4, these naïve T cells differentiate into Th2 cells. Activation of Th2 cells results in the production of IL-4, IL-5, IL-10, and IL-13, which orchestrate the development of IgE, producing B cells and the recruitment of eosinophils and mast cells. • Memory B cells continue to make IgE, which binds to receptors on mast cells and basophils. • Upon re-exposure to an antigen, the antigen cross-links IgE bound to mast cells and basophils, resulting in degranulation and the release of preformed mediators including histamine, proteases, kinins, prostaglandin 2, and cysteinyl leukotriene. • These substances act locally to produce increased vascular permeability, peripheral vasodilation, and to stimulate glandular secretion leading to nasal congestion, rhinorrhea, pruritus, and sneezing. • The allergic reaction can be divided into early and late phases. • The early phase occurs within minutes of the exposure; the pathophysiology of this phase is detailed above.

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• The late phase occurs 4 to 12 hours later, typically featuring more prominent nasal congestion. The pathophysiology of this phase is largely dominated by eosinophils and basophils recruited by IL-4, IL-5, and IL-13 produced in the early phase. • Table 12–1 summarizes the relevant AR guideline action statements.

Clinical Presentation • A detailed history and physical is the first step in the work-up for AR and includes determining temporal features of symptoms (ie, seasonal, perennial, persistent, intermittent, exposure associated); identification of specific triggers is also helpful; obtaining a family history is important and will frequently be positive for atopic dermatitis, asthma, or AR; finally, determining the severity of symptoms and careful attention to any episodes of anaphylaxis are necessary. Common symptoms of AR are listed below.

Symptoms • Nasal, ocular, palatal, or throat itching • Clear anterior rhinorrhea • Post-nasal drip • Sneezing • Nasal obstruction/congestion • Decreased sense of smell • Throat clearing • Cough, malaise, or fatigue (sometimes the only complaints in children with AR)

Physical Exam • There are several physical exam findings present in AR that are absent in other diseases with similar symptoms. • Although the definitive diagnosis is made with confirmation of an Ig-E mediated response to allergens on serum or skin testing, often history and physical exam are enough to make an initial diagnosis of AR and initiate empiric treatment. Further testing is indicated whenever the diagnosis is in question, patients have failed appropriate empiric treatment, or when the causative agent needs to be identified to guide treatment. • The exam includes the following.

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Action

Clinician should make the clinical diagnosis of AR when parents present with a history and physical examination consistent with an allergic cause and one or more of the following symptoms: nasal congestion, runny nose, itchy nose, or sneezing. Findings of AR consistent with an allergic cause include, but are not limited to, clear rhinorrhea, nasal congestion, pale discoloration of the nasal mucosa, and red and watery eyes.

Clinician should perform and interpret, or refer to a clinician who can perform and interpret, specific IgE (skin or blood) allergy testing for patients with a clinical diagnosis of AR who do not respond to empiric treatment, or when the diagnosis is uncertain, or when knowledge of the specific causative allergen is needed to target therapy.

Clinician should not routinely perform sinonasal imaging in patients presenting with symptoms consistent with diagnosis of AR.

Clinician may advise avoidance of known allergens or may advise environmental controls in AR patients who have identified allergens that correlate with clinical symptoms.

Clinician should assess patients with a clinical diagnosis of AR for, and document in the medical record, the presence of associated conditions such as asthma, atopic dermatitis, sleep-disordered breathing, conjunctivitis, rhinosinusitis, and otitis media.

Clinician should recommend intranasal steroids for patients with a clinical diagnosis of AR whose symptoms affect their quality of life.

Clinician should recommend oral second-generation/less sedating antihistamines for patients with AR and primary complaints of sneezing and itching.

Statement

1. Patient history and physical examination

2.  Allergy testing

3. Imaging

4.  Environmental factors

5. Chronic conditions and comorbidities

6.  Topical steroids

7.  Oral antihistamines

Table 12–1.  Summary of AAO-HNS Allergic Rhinitis (AR) Guideline Action Statements

Strong recommendation

Strong recommendation

Recommendation

Option

Recommendation (against)

Recommendation

Recommendation

Strength of Recommendation

137

Clinician may offer intranasal antihistamines for patients with seasonal, perennial, or episodic AR.

Clinician should not offer oral LTRAs as primary therapy for patients with AR.

Clinician may offer combination pharmacologic therapy in patients with AR who have inadequate response to pharmacologic monotherapy.

Clinician should offer, or refer to a clinician who can offer, immunotherapy (sublingual or subcutaneous) for patients with AR who have inadequate response to symptoms with pharmacologic therapy, with or without environmental controls.

Clinician may offer, or refer to a surgeon who offers, inferior turbinate reduction in patients with AR with nasal airway obstruction and enlarged IT who have failed medical management.

Clinician may offer, or refer to a clinician who can offer, acupuncture for patients with AR who are interested in nonpharmacologic therapy.

No recommendation regarding the use of herbal therapy for patients with AR.

8. Intranasal antihistamines

9. Oral leukotriene receptor antagonists (LTRAs)

10.  Combination therapy

11. Immunotherapy

12. Inferior turbinate reduction

13. Acupuncture

14.  Herbal therapy

No recommendation

Option

Option

Recommendation

Option

Recommendation (against)

Option

Strength of Recommendation

Source:  Seidman MD, Gurgel RK, Lin SY, et al. Clinical practice guideline: Allergic rhinitis. Otolaryngol Head Neck Surg. 2015;152(1 Suppl):S1–43. .

Action

Statement

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Head and Neck Examination • Eyes 1. Red and watery 2. Conjunctival swelling 3. Allergic shiners—darkening and puffiness under the eyes secondary to venous pooling • External nasal exam 1. Clear rhinorrhea 2. Allergic/transverse crease—a line across the nasal bridge

Anterior Rhinoscopy • Septum (+/− deviation) • Inferior turbinates (+/− hypertrophy and edema)

Nasal Endoscopy • Appearance of the mucosa (pale pink or bluish and “boggy”) • Presence and quality of any mucus (clear and watery vs thicker) • If polyps or mucopurulence is noted, consider sinusitis

Work-Up Allergy Testing • Skin testing 1. Antigen presented to skin cross-links IgE antibodies on surface of mast cells, resulting in degranulation and formation of a wheal and flare reaction within 15 to 20 minutes. 2. Different forms include skin prick testing and intradermal testing. 3. Sensitivity ~80% 4. Specificity ~80% 5. Performed on volar surfaces of forearms or back 6. Generally safe but does carry a risk of anaphylaxis 7. Relative contraindications: – Eczema or skin disease at testing site – Dermatographia 8. Contraindications: – Pregnancy – Poorly controlled asthma – Unstable/severe cardiovascular disease – Beta-blocker use

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9. Certain medications can alter results including the following: – Oral antihistamines – Tricyclic antidepressants – Antipsychotics – Benzodiazepines – Omalizumab – Topical skin corticosteroids • Serum/specific IgE testing 1. Allergens are bound to a substrate to which serum is added; specific IgE in the serum binds allergens on the substrate; nonhuman anti-IgE antibodies tagged with a marker are added, and a reaction (radioactive, chemiluminescent, colorimetric, fluorimetric) occurs. The intensity of this reaction is proportional to the amount of specific IgE in the serum. – Sensitivity 67% to 96% – Specificity 80% to 100% – No risk of anaphylaxis – Not contraindicated in any medical condition – Not altered by medication use • Positive test indicates AR. • Negative test supports non-allergic rhinitis.

Nasal Provocation Testing • Perform when allergy testing is negative, but concern for IgE mediated process remains high. • Positive test indicates localized AR. 1. There is on-going debate as to whether localized AR truly exists.

Radiographic Imaging • Not indicated when history and exam are consistent with AR; use should be limited to atypical presentations or when there is concern for co-occurring chronic rhinosinusitis (CRS).

Medical Treatment • The treatment of AR consists of a stepwise process; failure to control symptoms at one step necessitates advancing to the next therapeutic option.

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Avoidance and Environmental Controls • The first step in the treatment of AR • Can efficiently and effectively reduce allergen exposure and possibly reduce symptoms • The following environmental controls have been shown to reduce allergen levels 1. Removal of pets 2. Washing pets twice weekly 3. Acaricides (insecticides that kill dust mites) 4. Impermeable covers for bedding 5. Use of high-efficacy particulate air (HEPA) filters 6. Mechanical laundering in hot water 7. Any or all of these controls can be used in combination

Intranasal Steroid Sprays (INS) • First-line therapy for AR • Directly modulates pathophysiology of AR 1. Reduce inflammatory mediator and cytokine release 2. Inhibit recruitment of eosinophils, basophils, mononuclear cells, and neutrophils 3. Reduce nasal symptoms of congestion, itching, rhinorrhea and sneezing 4. Reduce eye symptoms of itching, swelling, redness and tearing • INS shown to be more superior to oral antihistamines (OA) in controlling nasal symptoms • Once or twice daily dosing • Can be combined with intranasal antihistamine

Oral Antihistamines (OA) • Second generation preferred over first generation due to sedating effects of the latter • Block the action of histamine on the H1 receptor • Reduce nasal symptoms less effectively than INS • Reduce eye symptoms as effectively as INS • Rapid onset of action (advantage over INS) • Once daily dosing • Maximum benefit seen with continuous use, however, can be effective on an as-needed basis

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Intranasal Antihistamine Sprays (INA) • Action similar to OA • Second-line therapy after INS and OA • Has been shown to reduce rhinorrhea, post-nasal drip, congestion, and sneezing • More effective at treating nasal congestion than OA • More rapid onset of action than OA • Twice daily dosing • Can be combined with INS

Anti-Leukotrienes (AL) • Leukotrienes (LTs) are produced from arachidonic acid via the 5-lipoxygenase pathway. • Cysteinyl leukotrienes are released during both the early and late phase of allergic reactions. • LTs are pro-inflammatory mediators that facilitate bronchoconstriction, edema, mucus secretion, and vascular permeability. • ALs are not recommended as a primary therapy in patients with AR. • Patients with concurrent asthma may benefit from ALs as a firstline therapy.

Combination Therapy • Appropriate when a patient has failed monotherapy • Recommended combinations with evidence of efficacy include: 1. INS + INA 2. OA + oral decongestants

Additional Therapeutics • Oral or topical decongestants • Guaifenesin (can thin and help clear thick mucus) • Ipratropium (for persistent watery rhinorrhea) • Cromolyn (mast cell stabilizer—can be used as a preventative measure prior to exposure) • Nasal saline

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Immunotherapy (IT) • Recommended for patients with AR who experience inadequate or incomplete response to appropriate pharmacologic therapy and environmental controls • The only treatment capable of changing the natural course of AR • Allergy testing (skin or serum) must be done prior to initiating IT • Immunologic changes from IT: 1. Promotes a shift from a Th2 to Th1 predominance 2. Suppression of allergen-specific T cell response 3. Immunoglobulin changes – Initial increase in IgE, followed by a gradual decrease – Decrease in serum IgE, IgE-dependent dendritic cell, and T-cell activation – B cell class switching:  initially to IgG1, then to IgG4 and IgA2 a. Results in high levels of IgG4 – Allergen-specific IgG4 (“blocking”) antibodies suppress IgE mediated degranulation, memory B cells, and antigen presentation. • Two forms available:  subcutaneous immunotherapy (SCIT) and sublingual immunotherapy (SLIT) • SCIT 1. Starting dose determined by specific IgE testing (skin or serum) 2. Escalation phase – Dose is gradually increased on a regular schedule until a therapeutic dose (controls symptoms) is achieved 3. Maintenance phase – Continued for 3 to 5 years 4. Administered in the office on a regular schedule (most commonly on a weekly basis) – Patient observed for 20 to 30 minutes after injection, as this is typically when adverse events occur 5. Safety – Local reactions most common—estimated rate of 3% to 10% – Systemic reactions—estimated rate of 0.6% a. Anaphylaxis ■ Occurs most frequently in the escalation phase b. Death ■ Estimated risk:  1 in 2.0 million to 2.5 million injections • SLIT 1. Aqueous and tablet forms

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2. Antigen is delivered to oral mucosa under the tongue and held for several minutes. 3. Likely, local dendritic cells uptake antigen, which is then presented to T cells in local lymphoid tissue. 4. Migration of T cells systemically produces systemic allergen tolerance. 5. Over time, SLIT induces immunologic changes similar to SCIT. 6. Administration can be done at home on a daily basis. 7. Same phases of escalation and maintenance – Tablet forms use either quick-escalation dosing or start at maintenance dose 8. Duration of treatment:  3 to 5 years 9. Safety – Local reactions are the most common—estimated rate of 0.056% a. Mucosal irritation, itching, swelling, ulceration – Anaphylaxis is very rare (case reports) – No deaths identified in large series or meta-analysis • Contraindications to immunotherapy 1. Pregnancy – Except SLIT tablets (pregnancy category B) 2. Poorly controlled asthma 3. Unstable/significant cardiovascular disease

Surgical Treatment • There are no surgical interventions currently recognized as a therapeutic option for AR. Nasal obstruction secondary to an anatomic defect (nasal valve collapse, septal deviation, inferior turbinate hypertrophy) that does not improve with appropriate medical treatment can be addressed surgically. In these cases, surgical intervention could help with a specific symptom; however, the pathophysiology and disease process of AR would, of course, remain unchanged.

Non-Allergic Rhinitis • Non-allergic rhinitis (NAR) is less prevalent than AR, but still impacts tens of millions of people. • Typically presents in adults (after the age of 20) • Shows a slight female predominance • Significant overlap of symptoms between allergic and non-allergic forms of rhinitis

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• The pathophysiology and triggers behind each form of rhinitis are what separate them into different disease processes. • As discussed previously, AR occurs when a patient becomes sensitized to an antigen resulting in a specific inflammatory process modulated by IgE. • Allergy testing, either in the form of skin or blood testing, ultimately differentiates between AR and NAR. • Compared to AR, the pathophysiology underlying NAR is less well understood. • There is evidence supporting both inflammatory and neurogenic mechanisms. • Some patients with NAR show no inflammatory infiltrate in nasal tissue, meanwhile others show increased numbers of neutrophils, eosinophils, or mast cells on histology when compared to controls. • Suggested neurogenic mechanisms revolve around the parasympathetic and sympathetic input balance to the nasal mucosa, with an imbalance between the two resulting in glandular hypersecretion (increased mucus production) and congestion. • Different categories of NAR exist and will be covered later in this chapter. • When evaluating a patient presenting with rhinitis symptoms, it is important to rule out both AR and chronic rhinosinusitis, as treatment of these disease processes differ slightly from that of NAR. • This differentiation begins with the history and physical followed by testing—all of which are highlighted below.

Clinical Presentation • The most crucial component to working up a patient with rhinitis is a detailed history and physical followed by additional testing to differentiate from other disease processes. A detailed history includes determining conditions around the time of onset of symptoms (ie, recent illness, starting a new job, new pregnancy), duration of symptoms, and associated symptoms. Common symptoms and triggers of NAR are listed below.

Symptoms • Nasal obstruction/congestion • Post-nasal drip and/or anterior rhinorrhea • Sneezing • Decreased sense of smell

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• Eustachian tube dysfunction • Throat clearing • Cough • Notably, nasal pruritus and associated ocular symptoms are largely absent. ** Often it is helpful to classify a patient into one of three categories: rhinorrhea dominate type, congestion dominate type, or mixed type NAR.

Triggers • Environmental irritants 1. Tobacco smoke 2. Dust 3. Pollution • Strong odors (eg, perfume or chemicals) • Ingestion of foods (gustatory rhinitis) • Ingestion of alcohol • Cold air • Changes in temperature • Changes in humidity • Changes in barometric pressure • Hormonal changes

Temporal Nature • Seasonal vs perennial • Persistent vs intermittent

Review of Medications • A comprehensive review of current medications is important as some medications can contribute to rhinitis symptoms. Medications known to contribute to or trigger NAR include the following: 1. Alpha-blockers 2. Alpha-agonist (ie, oxymetazoline abuse) 3. ACEI 4. Antidepressants, psychotropics 5. Antiepileptics 6. ASA/NSAIDs 7. Beta-blockers 8. Erectile dysfunction drugs 9. Oral contraceptives (OCPs)

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• For any oral or topical therapies the patient has previously tried for the treatment of rhinitis symptoms, the following questions should be asked: 1. How did you take the medication? (Dose/number of sprays, frequency) 2. How long did you take the medication? (Days, weeks, months) 3. Which of your symptoms did the medication help, and how much better did it make that symptom? • Of note, some medications may have helped with one symptom such as rhinorrhea or post-nasal drip, but not other symptoms such as congestion; therefore, it is important to have the patient consider the benefit for each medication tried for each of his or her predominant symptoms.

Physical Exam • No pathognomonic physical exam findings exist for NAR; notably, exam findings highly suggestive of AR (ie, allergic shiners, transverse nasal crease, ocular conjunctivitis, and tearing) should be absent. If nasal obstruction is a major complaint, then examination for nasal valve collapse, septal deviation, or inferior turbinate hypertrophy should be included. The exam includes:

Head and Neck Examination • Internal and external Cottle maneuvers

Anterior Rhinoscopy • Septum (+/− deviation) • Inferior turbinates (+/− hypertrophy)

Nasal Endoscopy • Appearance of the mucosa (can be normal, edematous, erythematous, or atrophic) • Presence and quality of any mucus (clear and watery versus thicker) • If polyps or mucopurulence noted, consider sinusitis

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Work-Up Allergy Testing • Perform in patients in whom diagnosis is in question or who have failed appropriate medical therapy. • Positive test indicates AR. • Negative test supports NAR.

CT Sinuses • Perform when presentation is atypical or there is concern for CRS. • Presence of paranasal sinus mucosal disease indicates CRS. • Absence of paranasal sinus mucosal disease supports NAR.

Medical Treatment • The treatment of NAR and AR includes a number of the same medications; the predominating symptom or symptoms should drive the initial choice of medical therapy with some options being more efficacious for one symptom over another. If the patient fails an adequate treatment regimen (typically considered 4 weeks), then a second agent can be added to the first; if this again fails to provide significant benefit, then a third medication can be added. There are several treatment modalities for NAR.

Intranasal Steroid Sprays (INS) • First-line therapy for NAR • Shown to significantly reduce rhinorrhea, post-nasal drip, and congestion • Once daily dosing • Requires 3 to 4 weeks of regular daily use before maximal symptomatic benefit will be achieved • Can be combined with INA

Intranasal Antihistamine Sprays (INA) • First-line/second-line therapy for NAR • Shown to reduce rhinorrhea, post-nasal drip, congestion, and sneezing • Twice daily dosing • Can be combined with INS ** Of note, in some studies, combination therapy of INS and INA has shown greater benefit than either as a monotherapy.

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Ipratropium Bromide Nasal Spray • First-line therapy for watery rhinorrhea dominated NAR such as gustatory, senile, or exercise-induced NAR. • Only demonstrated to improve rhinorrhea with no demonstrated benefit for congestion • Requires up to three times daily dosing, or use as needed prior to triggers/exposures that elicit rhinorrhea If a patient’s symptoms persist despite combined INS and INA, then several adjunct therapies can be considered.

If Rhinorrhea Persists, Consider Adding Treatments • Ipratropium bromide nasal spray • Nasal saline irrigations • First-generation oral antihistamines 1. Use with caution as sedation is a side effect 2. Second-generation oral antihistamines are not as drying as first generation • Capsaicin nasal spray 1. Shown to improve total nasal symptom scores 2. Use can be limited by mucosal irritation and burning

If Congestion Persists, Consider Adding Treatments • Nasal saline irrigations • Oral decongestants 1. Use with caution in the elderly and those with hypertension • Capsaicin nasal spray

Surgical Treatment • If a patient experiences debilitating symptoms or has failed maximal medical therapy, defined as combined INS+INA and appropriate adjunct therapies, then surgical intervention can be considered. Any anatomic cause for nasal obstruction or congestion (ie, nasal valve collapse, septal deviation, inferior turbinate hypertrophy) can be addressed surgically. Other surgical procedures geared toward alleviating symptoms of NAR include endoscopic vidian neurectomy and endoscopic posterior nasal neurectomy.

Endoscopic Vidian Neurectomy (EVN) • The vidian nerve is dissected out of the pteryogopalatine fossa and is divided as it exits the vidian canal (VC).

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• In small retrospective and prospective studies, it has been shown to improve nasal obstruction, rhinorrhea, and quality of life in some patients with NAR (vasomotor rhinitis) for a time period of several years. • Complications from EVN include transient dry eye, numbness in the maxillary nerve distribution, and nasal crusting/dryness.

Endoscopic Posterior Nasal Neurectomy (EPNN) • The posterior nasal nerve is identified as it courses from the sphenopalatine fossa towards the inferior turbinate and is divided. • In small prospective and retrospective studies, it has been shown to decrease nasal obstruction, rhinorrhea, and sneezing in most patients with AR. • Not studied as well in NAR cases • Complications from EPNN include transient numbness of the teeth and soft palate, dry eye, and postoperative bleeding.

NAR Subtypes • There are several subtypes of NAR into which patients can be further classified depending on key features, specific triggers, or co-occurring conditions that contribute to the development of NAR. Subtypes of NAR are listed below.

Non-Allergic Rhinitis with Eosinophilia • Also known as NARES • Defined as allergic symptoms with eosinophils on nasal smear but with negative allergy testing 1. May represent local AR—there is on-going debate as to whether this truly exists. • Patients may have more sneezing and nasal pruritus than other NAR subtypes. • Second-generation oral antihistamines may be more helpful for these patients than for those with other subtypes of NAR.

Drug-Induced Rhinitis (Rhinitis Medicamentosa) • Most commonly is secondary to oxymetazoline abuse 1. Risk of rebound nasal congestion and tolerance increases with use greater than 5 days. • Exam can reveal impressively edematous nasal mucosa and clear rhinorrhea.

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• Typically it takes at least a week after stopping the medication for rebound congestion to begin decreasing. • Consider treating the patient with oral steroids during the withdrawal period. • Patients should be reevaluated 3 to 4 weeks after stopping the medication for repeat exam to determine if there are anatomic reasons for the nasal obstruction that led them to abuse oxymetazoline or if there is evidence for AR/NAR.

Hormone-Induced Rhinitis • Causes:  pregnancy (most common), OCPs, hypothyroidism • Rhinitis of pregnancy typically appears during the first trimester and resolves after delivery but can present at any time during the pregnancy. 1. Management is conservative with saline rinses and nasal strips at nighttime; consult with OBGYN prior to recommending treatment with nasal steroids or oral decongestants. • If rhinitis symptoms are determined to be secondary to OCPs, treat as NAR and encourage patient to discuss changing to another form of contraception or a different OCP. • If rhinitis symptoms are determined to be secondary to hypothyroidism, treat as NAR and treat appropriately with thyroid hormone replacement; with correction of hypothyroidism, symptoms will likely improve.

Gustatory Rhinitis • Rhinitis symptoms (typically watery rhinorrhea) brought on by food intake • Can be very disruptive and embarrassing for patients • Treat as NAR with INS and/or INA with early addition of Ipratropium bromide to be used just prior to meal times

Senile Rhinitis • Occurs in the elderly—typically watery rhinorrhea as the predominate symptom • Can have a specific trigger (eg, cold air, eating) but can also have multiple triggers • Etiology is unknown but may be secondary to changes in parasympathetic/sympathetic tone • Treat as NAR with INS and/or INA with early addition of Ipratropium bromide

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Atrophic Rhinitis • Also known as “empty nose syndrome,” but other forms can occur; some may have a genetic component, others are due to chronic infection, nutritional deficiencies, or irritant exposure • Thought to be secondary to squamous metaplasia of the mucosa and degeneration of mucosal glands and sensory nerve fibers • The sensation of nasal obstruction is paradoxical as often, on exam, the nasal cavities are widely patent. • Endoscopy may reveal extensive surgery with absence of the inferior turbinates or it may reveal mucosal atrophy and crusting. • Treatment revolves around moisturization, therefore INS and INA should be avoided and regimens of nasal saline irrigation, saline based gels, and mupirocin should be recommended.

Conclusion • AR and NAR share a number of overlapping features, but the underlying pathophysiology is quite different and therefore treatment approaches differ. Ultimately, obtaining a detailed history and physical exam to arrive at the correct diagnosis is of utmost importance.

References • Brozek JL, Bousquet J, Agache I, et al. Allergic rhinitis and its impact on asthma (ARIA) guidelines—2016 revision. J Allergy Clin Immunol. 2017 Oct;140(4):950–958. • Krouse HJ. Environmental controls and avoidance measures. Int Forum Allergy Rhinol. 2014;4(Suppl 2):S32–S34. • Roche AM, Wise SK. Subcutaneous immunotherapy. Int Forum Allergy Rhinol. 2014;4(Suppl 2):S51–S54. • Seidman MD, Gurgel RK, Lin SY, et al. Clinical practice guideline: Allergic rhinitis. Otolaryngol Head Neck Surg. 2015;152(Suppl 1): S1–S43. • Toskala E. Immunology. Int Forum Allergy Rhinol. 2014;4(Suppl 2):​ S21–S27.

Chapter

13 Diseases of the Nasal Cavity Bobby A. Tajudeen and Pete S. Batra

Congenital Disease of the Nasal Cavity . . . . . . . . . . . 154 Choanal Atresia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 154 Congenital Nasal and Nasopharyngeal Masses . . . . . . . 155 Infectious and Inflammatory Disease of the . . . . . . 160 Nasal Cavity Allergic Rhinitis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 160 Non-Allergic Rhinitis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 160 Viral Rhinitis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 160 Bacterial Rhinitis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 161 Septal Abscess . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 161 Rhinoscleroma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 162 Rhinosporidiosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 162 Actinomycosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 163 Histoplasmosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 163 Blastomycosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 164 Infections of the External Nose . . . . . . . . . . . . . . . . . . . . 164 Systemic Disease Affecting the Nasal Cavity . . . . . . . . . 166 Neoplastic Disease of the Nasal Cavity . . . . . . . . . . . . 166 Benign Neoplasms . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 166 Malignant Neoplasms . . . . . . . . . . . . . . . . . . . . . . . . . . . . 171

153

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Congenital Disease of the Nasal Cavity Choanal Atresia • Pathophysiology:  The condition results from persistence of the buccopharyngeal membrane, failure of the bucconasal membrane to rupture, or medial outgrowth of the vertical and horizontal processes of the palatine bone. • Incidence:  1 in 7000 to 8000 live births • Female preponderance 2:1 • Two-thirds bilateral, one-third unilateral (right-sided predominance) • Pure bony stenosis, 29%; mixed bony-membraneous, 71% • SSx:  bilateral symptomatic at birth due to obligate nasal respiration in neonates; presentation ranges from mild respiratory distress with feeding difficulty to severe airway obstruction; cyclical cyanosis relieved by crying; unilateral atresia presents later in life with unilateral discharge, congestion, and anosmia • Dx:  inability to pass 6-Fr catheter through the nasal cavity 3 to 3.5 cm from the alar rim; nasal endoscopy; high-resolution CT study of choice; CT findings:  widened vomer width, absent choanal airspace with bony anomalies, decreased airspace with membranous anomalies • Associated Syndromes:  CHARGE syndrome (most common concurrent syndrome in bilateral atresia, 50%), Apert, Treacher Collins, Crouzon, trisomy 21, 22q11 deletion • Initial management of bilateral atresia requires prompt recognition and airway stabilization by finger inserted into oral cavity, oropharyngeal airway, McGovern nipple, or intubation; orogastric feedings may be required.

Surgical Management • Transnasal puncture:  oldest technique, puncture of the atretic plate, most commonly with Fearon dilator; often requires revisions and risk for skull base injury • Transpalatal repair:  involves resection of palatine bone, portion of vomer and atretric plates; mucosa over plate used to reline bony surfaces of neochoana; 90% success rate; complications: palatal flap breakdown, palatal fistula; major drawback is adverse effects on long-term palatal growth with risk of crossbite and hard palate deformity

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• Endoscopic repair:  transnasal endoscopic approach; mucosal flaps are elevated posteriorly off the atretic plate and septum; the posterior vomer and atretic plate are drilled; mucosal flaps are used to line the neochoana; avoids midpalatal suture and palatal deformities; no need for stenting; technically challenging in small neonatal nasal cavity

Congenital Nasal and Nasopharyngeal Masses Neurogenic Tumors • Embryologically lesions occur due to faulty closure of anterior neuropore. • Between 50 and 60 days gestation, cartilaginous nasal capsule is formed and membranous bones of fetal skeleton develop, leaving fonticulus nasofrontalis, an opening between developing nasal and frontal bones. • Prenasal space forms between frontal and nasal bones anteriorly, and the cartilaginous nasal capsule posteriorly. • Diverticulum of dura passes through foramen cecum, an opening between the skull base and frontal bone, into the prenasal space; diverticulum can adhere to nasal skin and span entire prenasal space. • Diverticulum regresses in normal development and skull base and frontal bone fuse; failure of regression results in neuroglial or ectodermal elements to remain in prenasal space with potential intracranial connection. • Incidence 1 per 20 000 to 40 000 live births • Three most common lesions:  glioma, dermoid, encephalocele • Biopsy or needle aspiration of midline nasal masses in children contraindicated, given potential for intracranial connection.

Nasal Dermoid • Incidence:  1% to 3% of overall dermoid cysts; 3.7% to 12.6% of head and neck dermoid cysts • Most common midline nasal mass • Contains ectodermal and mesodermal elements; cysts lined by squamous epithelium, filled with keratin debris, surrounded by connective tissue containing hair follicles, sweat glands, sebaceous glands • Pathophysiology:  incomplete regression of dural tissue in prenasal space or fonticulus frontalis

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• Location:  most commonly on nasal dorsum but may occur anywhere from glabella to nasal tip; may present as intranasal, intracranial, or extranasal masses • SSx:  nasal pit present in 45% to 84%, may contain tuft of hair; masses are firm, nontender, noncompressible, nonpulsatile, and do not transilluminate • Dx:  CT and/or MRI to evaluate for intrancranial extension, negative Furstenburg test • Complications:  repeat infection with cellulitis and abscess formation, nasal skin necrosis, cartilaginous and bony destruction, recurrent meningitis, CSF leak • Rx:  early complete excision; requires removal of entire cyst and tract, along with involved bone, cartilage, and septum; open rhinoplasty approach advocated; allows for wide exposure of the entire nasal dorsum, controlled external osteotomies, and improved cosmesis

Nasal Glioma • Present shortly after birth but may present later in life • Composed of ectopic glial tissue • Intracranial connection for 20% of gliomas retained through fibrous stalk • Histology:  demonstrates mass covered by normal nasal mucosa or skin and contains astrocytic neuroglial cells with fibrous and vascular connective tissue • Pathophysiology:  sequestered glial tissue, results in unencapsulated collection of heterotopic glial cells • Location:  60% external, 30% internal, 10% combined, not typically midline • SSx:  firm, nontender, noncompressible, does not transilluminate; external gliomas are red and may have overlying telangiectasias; intranasal masses glistening gray or purple arising from lateral aspect of the nose • Dx:  CT and/or MRI, negative Furstenberg test • Complications:  cosmetic deformity, nasal obstruction, meningitis • Rx:  complete surgical excision; external nasal approach or lateral rhinotomy for extracranial gliomas; gliomas with significant intracranial extension may require craniotomy with exploration of the anterior cranial fossa

Encephalocele • Congenital herniation of CNS tissue through a skull base defect • Incidence:  1 per 4000 live births

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• Types by contents 1. Meningocele:  meninges only 2. Meningoencephalocele:  meninges and brain 3. Meningoencephalocystocele:  meninges, brain, and part of the ventricular system • Types by Location 1. Posterior/occipital:  most common, 75% to 80% of encephaloceles 2. Anterior:  subdivided into sincipital and basal types – Sincipital (frontoethmoidal):  defect between frontal and ethmoid bones at foramen cecum; three types a. Nasofrontal:  glabellar lesion b. Nasoethmoid:  lateral nose lesion c. Nasoorbital:  medial orbital wall lesion – Basal:  Four types a. Transethmoidal b. Sphenoethmoidal c. Transsphenoidal d. Sphenomaxillary • Histologically consist of meningeal sac containing glial tissue • Pathophysiology:  failed closure of neuropore or failed migration of neural crest cells results in ependymal-lined meninges herniating through the base of the skull; communicates with the subarachnoid space (CSF filled) • SSx:  external encephaloceles present at or near the glabella as soft compressible masses; intranasal lesions bluish, pedunculated masses medially and high in the nasal cavity; masses compressible and pulsatile, may enlarge with crying or internal jugular vein compression (positive Furstenberg sign) • Dx:  CT and/or MRI reveals bony defect, positive Furstenberg sign • Complications:  meningitis, nasal obstruction, cosmetic deformity, hydrocephalus • Rx:  surgical resection and repair of skull base defect to prevent CSF rhinorrhea or further herniation 1. Endoscopic repair:  transnasal approach, encephalocele bipolared to skull base defect; multi-layer closure performed with many options; classically, fat underlay, fascia lata underlay, +/− bone graft, with free mucosal graft or pedicled nasoseptal flap overlay 2. Intrathecal fluorescein aids in identification of encephalocele or CSF leak, 0.1 ml of 10% fluorescein in 10 ml of patient’s CSF infused at 1 cc/min

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Teratoma • Primary congenital tumors that arise from all three embryonic germ layers • Incidence:  1 in 4000 live births • Most common locations:  sacrococcygeal, retroperitoneal, mediastinal, and head and neck • Head and neck teratomas comprise 2% to 9% of all teratomas; cervical and nasopharyngeal most common • Occur in children 50%) contributing to locoregional recurrence and distant metastasis • Rx:  surgery and adjuvant radiation therapy for positive margins or advanced disease • Survival at 5 and 10 years 86% and 53%, respectively • Recurrence rate at 5 and 10 years 42% and 73%, respectively • Positive margins and tumors of sphenoid/ethmoid origin:  worse prognosis • Maxillary and nasal cavity origin:  best prognosis

Chordoma • Rare neoplasm arising from vestigial or ectopic notochord • Incidence 0.08 patients per 100,000, presenting in fifth to sixth decades • Lesions arise in axial skeleton in sacrum, spine, and skull base; cranial region most common site (42%); skull base lesions occur in the clivus (spheno-occipital bones) • Histopathology:  key pathologic finding is physaliferous cells

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• SSx:  Slow-growing, destructive malignancy, rarely metastasizes; often present with visual loss, diplopia, cranial nerve deficits (third and sixth most common), hydrocephalus, sensorimotor deficits • Imaging:  CT imaging demonstrates soft tissue mass at central skull base with clival destruction and may have calcifications; MRI delineates soft tissue extent of tumor and relationship to critical structures (nasopharynx, brainstem, internal carotid artery, optic chiasm), and intense enhancement with gadolinium in honeycomb appearance. • Chondroid chordomas in 5% to 15%; present more lateral in position, improved survival rates • Rx:  surgical excision for local control followed by radiation therapy • Expanded-endoscopic endonasal approach most commonly used for midlines lesions; lateral approaches should be considered for lesions with significant lateral extension • 5-year survival 73% to 80.7%; increased age (>50) associated with higher mortality

Sinonasal Lymphoma • Rare in western populations, 1.5% of all non-Hodgkin’s lymphomas; 6% to 8% of all H&N extranodal lymphomas; higher incidence in Asian populations, accounting for 2.6% to 7% of all lymphomas • In Western populations, B cell lymphomas account for 55% to 85% of sinonasal lymphomas (non-Hodgkin’s lymphoma most common), T cell lymphomas constitute 90% of tumors in Asian populations; NK/T cell lymphomas have high incidence of EBV infection • Most frequent sites in Western countries:  maxillary sinus, nasal cavity, and ethmoid; nasal cavity most common site in Asian countries • Radiographic studies can demonstrate sinonasal mass or mucosal thickening without sinus opacification. • B cell lesions present as a submucosal gray, white, or tan mass; NK/T lesions present with extensive ulceration and necrosis due to vascular invasion. • Dx:  biopsy submitted for fresh, unfixed specimen for flow cytometry • Rx:  chemotherapy +/− radiation; surgery only for diagnostic purposes • Five-year survival 50%; improved survival in younger patients, early stage, lack of B symptoms (fever, nightsweats, weight loss); tumor immunophenotype (B or T/NK) outcome varies but not considered a prognostic factor for survival

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Surgical Management of Malignant Sinonasal Tumors • See Chapter 26

Chapter

14 Pathophysiology of Chronic Rhinosinusitis Patricia A. Loftus, Andrew N. Goldberg, and Steven D. Pletcher

Background . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 180 Pathogenesis of CRS Theories . . . . . . . . . . . . . . . . . . . . 180 Infectious/Inflammatory . . . . . . . . . . . . . . . . . . . . . . . . . . 180 Allergy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 182 Immunology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 182 Host Genetics . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 183 Anatomy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 184 Environment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 184 Fungus . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 185 Osteitis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 185 Innate Immunity . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 186 Mucosal Barrier . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 186 Superantigens . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 187 Dysbiosis of the Sinus Microbiome . . . . . . . . . . . . . . . . . 187 Biofilms . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 188 Conclusion . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 189

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Background • Rhinosinusitis is defined as “a group of disorders characterized by inflammation of the mucosa of the nose and paranasal sinuses.” • Unlike the well-established role of bacterial infection in acute rhinosinusitis (ARS), the pathogenesis of chronic rhinosinusitis (CRS) is unclear. 1. Likely the result of a variety of contributing factors rather than the result of a single etiology (Figure 14­–1). • Inflammation is a universal finding in rhinosinusitis. 1. Objective evidence of mucosal inflammation on nasal endoscopy and/or computerized tomography (CT) scan is required for the diagnosis of CRS. • Inflammation may occur from a variety of sources including bacterial, viral, and fungal infection and/or mucosal colonization. • Disorders of the innate or adaptive immune system may also result in chronic inflammation. • CRS is classified as CRS with nasal polyposis (CRSwNP) and CRS without nasal polyposis (CRSsNP). 1. CRSwNP has a characteristic Th2 eosinophilic inflammatory profile with local production of IgE, IL-4, IL-5, and IL-13. 2. CRSsNP has a characteristic Th1 inflammatory profile consisting of neutrophils, lymphocytes, plasma cells, and inflammatory cytokines including Interferon-g and TGF-b. • Other subtypes of CRS include allergic fungal rhinosinusitis (AFRS) and aspirin-exacerbated respiratory disease (AERD). • These contributing factors and the theories behind them are further discussed below.

Pathogenesis of CRS Theories Infectious/Inflammatory • Bacteria can both infect and colonize the nasal and paranasal mucosa, and lead to mucosal inflammation. • The pathogens found in CRS differ from ARS, and include S. aureus, Pseudomonas aeruginosa, and anaerobic pathogens as well as the more common acute pathogens such as Streptococcus spp. and Haemophilus influenza. • Antibiotic therapy may affect the bacteria and temporarily improve the symptoms of CRS, but the efficacy of antibiotics as a treatment for CRS is limited.

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Figure 14–1.  Multiple factors contributing to the pathogenesis of CRS.

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• It is unclear whether bacteria cause CRS due to a classic infectious process versus an inflammatory process. 1. Anti-inflammatory activities of the antimicrobial class of macrolides are currently receiving a lot of attention. • Bacteria can also release exotoxins that result in sinonasal inflammation.

Allergy • Theorized that allergies result in inflammation that can eventually progress to CRS • Higher incidence exists of developing both acute and chronic rhinosinusitis in patients with a diagnosis of allergic rhinitis although direct causal link is unclear. • Incidence of allergies in patients with CRSwNP approximates that of the general population. • Allergies can exacerbate sinonasal symptoms in patients with CRS.

Immunology • Increased risk of infection in patients with compromised immune system • 8% to 63% prevalence of immunodeficiency in patients with refractory sinusitis • Immune function evaluation should be considered in patients with recurrent and severe sinus infections who have failed aggressive medical management, especially if they have a history of pulmonary infections and/or a family history of primary immunodeficiencies. • Primary immunodeficiencies (PIDs) 1. Intrinsic defect in the host immune system that increases host susceptibility to bacterial infection (B cell deficiency), intracellular pathogens/opportunistic infections/fungi (T cell dysfunction), sinopulmonary infections and autoimmune disorders (complement deficiencies), and abscess formation (phagocytic disorders) – Disorders of the adaptive immune system include antibody deficiencies (congenital agammaglobulinemia, selective IgA deficiency, IgG subclass deficiencies), T cell disorders (severe combined immunodeficiency), and combined B and T cell disorders (common variable immunodeficiency) – Disorders of the innate immune system include complement deficiencies and phagocytic disorders

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• Secondary immunodeficiencies 1. Infections – Human Immunodeficiency Virus 2. Malignancy – Lymphoproliferative disorders, chronic lymphocytic leukemia, multiple myeloma 3. Medications – Immunosuppressives, chemotherapeutic drugs, corticosteroids 4. Systemic disease – Malnutrition, diabetes, chronic renal and liver disease

Host Genetics • Evidence of familial patterns and cases of concordant disease in monozygotic twins suggest a genetic basis of CRS. • Family history of CRS more common in patients with CRSwNP. 1. Correlation between a positive family history of nasal polyps and the severity of sinonasal disease in the affected patient • Association with asthma and allergic rhinitis infer genetic predisposition. • Multiple single nuclear polymorphisms associated with inflammatory mediators that increase the risk of CRS have been found in CRS patients. • Genetic alterations that result in loss of the T2R38 bitter taste receptor function are associated with increased rates of gramnegative related CRSsNP. 1. Bitter taste receptors identify quorum-sensing molecules secreted by gram-negative bacteria and contribute to innate immune function. • CRS is associated with genetic syndromes. 1. Kartagener syndrome/primary ciliary dyskinesia (PCD) – Impaired ciliary motility results in decreased mucus clearance and recurrent upper and lower airway infections. – Multiple genes for dynein arms and microtubule binding have been identified in PCD; mutations in these genes are likely responsible for the almost 100% rate of CRS in patients with PCD. 2. Cystic fibrosis (CF) – Thickened secretions result in decreased mucociliary clearance, infection, and inflammation. – Viscous mucus may also cause a local hypoxia that could be associated with biofilm formation.

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– Higher prevalence of CRS is found in CF mutation gene carriers than those who are not carriers of the gene. 3. Churg-Strauss syndrome (CSS)/eosinophilic granulomatosis with polyangiitis (EGPA) – CRS a common finding in patients with EGPA. 4. Ataxia telangiectasia 5. Young’s syndrome • DNA microarray studies have identified potentially causative genes in CRS that are actively being studied. 1. Certain genes have been linked to the development of nasal polyps in patients with CRSwNP, specifically inflammatory mediators such as the toll-like receptor (TLR)-mediated pathway. – CRSsNP expresses different inflammatory mediators as well as a differential expression of TLR pathway genes. 2. AFS patients show variation in MHC class II phenotypes and differential expression of multiple genes on DNA microarray studies. 3. Mutations in genes involved in leukotriene synthesis have been found to be associated with AERD.

Anatomy • Certain anatomical abnormalities can result in ostial obstruction, mucus stasis/decreased mucociliary clearance, infection, and finally inflammation of the sinonasal cavity. 1. Concha bullosa 2. Paradoxical middle turbinate 3. Septal spur • The majority of patients with these anatomic variations do not develop chronic sinus inflammation. • The incidence of anatomic variations is similar in patients with and without chronic sinusitis.

Environment • Certain environmental particulates and pathogens can irritate the sinonasal mucosa and alter the normal sinonasal flora, resulting in a chronic inflammatory reaction that leads to mucus stasis/decreased mucociliary clearance, infection, and symptoms of CRS. 1. Tobacco smoke (primary or secondary) 2. Industrial pollution 3. Noxious chemicals

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4. Medications 5. Foreign bodies:  nasal packing, nasogastric tube

Fungus • AFRS is caused by a predisposed person’s Type I, IgE-mediated allergic response to inhaled mold spores that are ubiquitous in the environment. • Sinonasal eosinophilia and inflammation perpetuates a cycle of chronic edema, mucociliary dysfunction, stasis of secretions, and super-infection. 1. This is secondary to improper functioning of the sinonasal mucosa from the inflammatory response to fungus • Fungus has been found to be an ubiquitous colonizer of the nasal cavity and does not generally result in an inflammatory response. 1. The subset of patients with an inflammatory response to fungus develop an eosinophilic inflammatory response. 2. Excessive host response to the common airborne fungus Alternaria has been postulated to trigger all forms of CRS, but antifungal treatment for CRS has been found to be ineffective. – Overall, the role for fungi in CRS remains uncertain.

Osteitis • CRS was historically thought to be a mucosal disease, but recently subepithelial disease has been studied as a cause of recalcitrant CRS. • Osteomyelitis is a bony infection in the marrow space. 1. Acute bacterial infection in bone → inflammatory response → lowering of pH and oxygen tension → microthrombi → bony necrosis • Osteitic bone may serve as a reservoir for chronic infection and inflammation. 1. No correlation, however, between intraosseous bacteria and osteitic bone in CRS • Bone osteitis thought to be due to tissue remodeling from cytokines that are released by the inflamed sinonasal mucosal overlying the bone • Osteitis found in 36% of CRS patients radiographically • Osteitis associated with greater disease severity and worse postoperative outcomes • Aggressive antibiotic treatment, a stalwart for treatment of osteomyelitis, has proved ineffective in CRS.

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Innate Immunity • Innate immunity does not require prior exposure to a pathogen, and is the first line of defense against airborne organisms that encounter the sinonasal cavity. 1. Functions mostly via increased mucociliary flow/clearance 2. Secretes endogenous antimicrobials that inhibit microbial growth and remove pathogens and particulates 3. Airway mucus contains antimicrobial proteins such as lysozyme that immobilize or kill microorganisms. 4. Pattern recognition receptors (PRRs) on sinonasal epithelial cells become activated when in contact with a pathogen and induce inflammatory cytokine signaling, and mucosal injury repair mechanisms. 5. Sinonasal epithelial cells themselves have been found to express immune genes like complement components and immune mediators. • Disruption of innate immunity (whether overactive or underactive) may disturb normal mucosal homeostasis, permit airborne microorganisms to stimulate an immune response when they otherwise would not, predispose the patient to microbial colonization, and result in a chronic inflammatory state that is the hallmark of CRS. 1. Inflammatory state continues even following removal of the inflammatory trigger, such as an infectious agent. • CRSwNP is believed to be associated with decreased epithelial cell innate immune function, particularly the levels of TLRs in recalcitrant CRS cases. • Eosinophilic polypoid CRS may be associated with decreased antimicrobial innate immunity.

Mucosal Barrier • The sinonasal epithelium provides both a mechanical and innate immune barrier to pathogens and particulates. 1. Also plays a role in recruiting, activating, and regulating the acquired immune response – This occurs via cell surface molecules that activate T cells, cytokines that activate B cells, T cells, and dendritic cells, and chemokines that attract the cells of the adaptive system 2. Antiproteases protect epithelial surface receptors from excessive stimulation by exogenous proteases, which could increase cytokine production and disturb the acquired immune response.

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• Defects in this barrier can result in chronic mucosal inflammation. 1. The breakdown of the mechanical component of epithelial barrier function permits foreign proteins to stimulate an immune response and subsequent inflammatory response. 2. Diminished tight junction proteins, increased ion permeability, and a decrease in proteins involved in maintaining the epithelial barrier function have been demonstrated in CRS patients as compared to normal controls.

Superantigens • Proteins released from bacteria that can produce an aggressive inflammatory reaction via nonspecific T cell activation that results in massive cytokine release 1. Bind directly to T cell receptors at an unconventional binding site, bypassing the usual antigen presenting cell and resulting in constitutive T cell activation 2. May also act as conventional antigens and stimulate IgE and polyclonal B cell activation • Superantigen from S. aureus is an enterotoxin that results in the release of IL-5 from Th2. 1. This activates eosinophils, produces IgE, and promotes the characteristic tissue response seen in polyps in patients with CRSwNP. – ~50% of patients with CRSwNP demonstrate inflammatory responses consistent with superantigen exposure. – Superantigens may also contribute to glucocorticoid insensitivity in CRSwNP. – A higher proportion of S. aureus enterotoxin exists in tissue of CRSwNP patients as compared to healthy controls. 2. No role for superantigens in CRSsNP 3. S. aureus biofilms may act as a reservoir for release of superantigens into the sinuses. 4. Likely a disease modifier rather than an etiologic agent, as there is widespread nasal colonization of S. aureus even in patients without CRS

Dysbiosis of the Sinus Microbiome • The sinus microbiome contributes to the strength of the mucosal barrier and mucus blanket, nutrient metabolism, and the development of innate and adaptive immune properties such as immunoglobulin production.

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• Disruption of the healthy microbial community of the sinonasal cavity can lead to inflammation. • Patients with CRS have been shown to have reduced number of bacterial species, an uneven distribution of bacteria, and poor diversity of the sinus microbiome. • The dominant species of the microbial community of the sinus in a patient with CRS has been shown to be associated with disease severity and recurrence. • There is disagreement as to whether the dysbiotic microbial community in CRS is a result of, or the cause of, inflammation in the sinuses.

Biofilms • Biofilms occur when organized communities of bacteria embed in a matrix of extracellular polymeric substances. 1. Express virulence factors and are more resistant to host defense and antibiotics 2. Low metabolic rate reduces antibiotic susceptibility and makes culture via traditional techniques difficult 3. Associated with epithelial destruction and loss of cilia, which impairs mucociliary clearance and promotes further bacterial growth 4. Bacterial shedding may contribute to intermittent acute exacerbation. • Biofilms have been demonstrated in 25% to 100% of CRS patients. • May play a role in recalcitrant CRS • Most common biofilm pathogens include S. aureus, Pseudomonas aeruginosa, and coagulase-negative Staphylococcus. 1. Fungal biofilms have also been found in up to 64% of CRS patients. • Allergic sensitization to microbes of biofilms can result in inflammatory infiltration. • CRS patients with S. aureus biofilms have been shown to have worse preoperative symptoms and worse outcomes after sinus surgery. • Biofilms are likely only a contributing factor to CRS, as they are also found in healthy subjects; that is, it is likely the composition and effect of the biofilm, rather than the biofilm itself, are important in CRS.

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Conclusion • The pathophysiology behind CRS is characterized by inflammation and likely represents a multifactorial complex interplay between host, pathogens, environmental, and immune factors rather than a single unified etiology. • Research is ongoing in all fields of CRS pathophysiology. • As the pathophysiology behind CRS is better delineated, novel treatment options tailored to these mechanisms may improve outcomes in CRS patients.

Chapter

15 Acute Rhinosinusitis Wirach Chitsuthipakorn and Kornkiat Snidvongs

Definitions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 192 Pathophysiology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 192 Diagnosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 193 Acute Rhinosinusitis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 193 Acute Bacterial Rhinosinusitis . . . . . . . . . . . . . . . . . . . . . 195 Treatments . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 195 Intranasal Corticosteroid (INCS) . . . . . . . . . . . . . . . . . . . 196 Oral Corticosteroid . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 196 Antibiotics . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 196 Antihistamines . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 197 Others . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 197 Surgery . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 198 Conclusions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 198

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Definitions • Acute rhinosinusitis (ARS) is an inflammatory condition in nose and sinuses. • The “rhinosinusitis” term was often used interchangeably with “sinusitis,” emphasizing the inclusion of rhinitis, which mainly occurs in the nose and usually coexists; this is why we now adopt the term “rhinosinusitis” and use it commonly to describe this particular condition involving both sites. • Uncomplicated rhinosinusitis is an inflammatory condition of nose and sinuses without evidence of any orbital or intracranial complications. • Acute bacterial rhinosinusitis (ABRS) is an inflammation of nose and sinuses caused by bacteria, whereas viral rhinosinusitis (VRS) is caused by virus.

Pathophysiology • Normal sinuses have patent sinus ostia, normal mucociliary function, and both normal quality and quantity of secretion; disturbance of any of these factors can lead to rhinosinusitis. • The inflammation is usually induced by infection involving virus or bacteria in the upper airways; this process causes mucosal swelling, obstructing ostia, and blockage of the drainage pathway; fluid collection may occur, providing an environment suitable for growth of pathogens; infection then causes mucosal lining inflammation and disruption of normal mucociliary function. • Normal paranasal sinuses are not sterile but are colonized with many bacteria. Additionally, bacteria from the nasopharynx and nasal cavities can enter the paranasal sinuses by sneezing; bacterial growth may be promoted by preceding viral inflammation (common cold or acute viral rhinitis), becoming ABRS in about 0.5% to 2% of cases. • Anatomic variations—such as septal deviation, infraorbital air cells, accessory ostia, concha bullosae, and frontal air cells— may lead to obstruction leading to rhinosinusitis, especially in recurrent cases. • Other predisposing factors include rhinitis, dental infection, trauma, immune compromised status, genetic diseases, and are discussed further in Chapter 14.

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Diagnosis Acute Rhinosinusitis • According to the American Academy of Otolaryngology’s “Clinical Practice Guideline (Update): Adult Sinusitis,” diagnosis of acute rhinosinusitis can be made clinically by observing presence of purulent nasal discharge with nasal congestion or facial pain, or both, for up to 4 weeks. • If symptoms do not worsen, and they are within a period of 10 days since the onset, this is called viral rhinosinusitis. • Nasal endoscopy is the preferred modality for clinical diagnosis, however, it is not routinely recommended; purulent discharge from middle meatus and/or sphenoethmoidal recess, as shown in Figure 15–1, are the key findings of the disease. Bacterial culture is best obtained from the middle meatus; it correlates with the pathogen in the maxillary sinus or anterior drainage group sinuses, as shown in Figure 15–2.

Figure 15–1.  Purulent discharge from middle meatus indicates acute rhino­ sinusitis.

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Figure 15–2.  A swab is introduced into the middle meatus to obtain purulent discharge for bacterial culture.

• Specimens from the nasal floor and nasopharynx are prone to contamination with poor correlation with the etiological pathogen. • Plain films are of little diagnostic value due to low sensitivity and specificity; this is especially true in children whose paranasal sinuses are not well pneumatized. • CT, MRI, and ultrasound are not routinely used or recommended except in the presence of complications, immune compromise, and very severe disease. CT is useful for providing a road map for endoscopic sinus surgery in indicated cases; anatomic abnormalities such as infraorbital air cell, concha bullosa, and septal deviation that potentially predispose to rhinosinusitis should be evaluated. MRI is the best imaging modality when intracranial and orbital complications are suspected. • Maxillary sinus puncture and aspiration is the gold standard for definite diagnosis and for obtaining specimen for bacterial culture, but this procedure is invasive and requires a skilled specialist to perform safely and reliably.

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Acute Bacterial Rhinosinusitis • Key symptoms of bacterial rhinosinusitis are purulent nasal discharge and local facial pain or dental pain. Important signs of bacterial rhinosinusitis are fever, after typical duration of viral illness, and purulent discharge observed at middle meatus or posterior nasal drip. • According to guidelines from the American Academy of Otolaryngology, certain symptoms of acute rhinosinusitis suffice to diagnose ABRS: those that either persist at least 10 days or clinically worsen (so-called “double worsening”) after a typical duration of viral illness within 10 days from the onset. • According to the 2012 European position paper, on rhinosinusitis and nasal polyps (“EPOS”), the diagnosis of ABRS requires worsening symptoms after 5 days or persistent symptoms after 10 days with at least an additional three of the five following criteria: fever ≥38°C, discolored nasal discharge (with unilateral predominance), local facial pain (with unilateral predominance), elevated ESR or CRP, double worsening. • According to the 2012 guidelines from the Infectious Diseases Society of America (IDSA) on clinical practice for acute bacterial rhinosinusitis in children and adults, the diagnosis of ABRS requires at least one of three of the following criteria: persistent symptoms for more than 10 days, onset of severe symptoms present at the beginning of illness (fever ≥39°C and purulent nasal discharge or facial pain), double worsening. • Definitive diagnosis is the presence of 100,000 colony-forming units of pathogen per milliliter from antral puncture culture; however, the culture yield is largely dependent on sterile techniques, as contamination easily occurs. • Streptococcus pneumoniae, Haemophilus influenzae, and Moraxella catarrhalis are the three most common causative agents. S. pneumoniae has decreased from the most frequent pathogen (38%) to approximately now being the same as that of H. influenzae (36%) possibly due to the increasing use of pneumococcal vaccine; additionally, Staphylococcus aureus is also found in about 10% of the cultures.

Treatments • The goal of treatment is to stop the inflammatory process and to relieve symptoms. The majority of cases are caused by a virus

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and the disease is self-limiting; patients need only symptomatic medications without antibiotics, except when superimposed bacterial infection occurs.

Intranasal Corticosteroid (INCS) • The aim of INCS is to reduce inflammation of the nasal mucosa and the sinus ostium. This helps facilitate sinus clearance, normal ciliary function, and ventilation. • It is recommended as monotherapy for VRS, especially in nonsevere cases; as an adjunct therapy in ABRS, combining with antibiotics is beneficial to facilitate faster symptom reduction. • A 2005 study from Meltzer, Bachert, and Staudinger concluded that double-dosing (twice daily) INCS is more effective than regular (once daily) dose.

Oral Corticosteroid • Oral steroid is not recommended as monotherapy in acute uncomplicated rhinosinusitis—a placebo-controlled study in 2012 did not show any difference from placebo; however, combining oral steroid with antibiotics might reduce facial pain and nasal congestion in ABRS patients.

Antibiotics • Antibiotics are the mainstay treatment in ABRS; Ahovuo-Saloranta and colleagues stated in 2014 that efficacy among different antibiotics are not significantly different. • Some ABRS patients recover without antibiotics; with good ability to follow up, “watchful waiting” may be offered to uncomplicated ABRS cases regardless of severity. According to the American Academy of Otolaryngology guidelines, antibiotics are considered optional. If symptoms are not improved within 7 days or patient’s conditions worsen within 48 to 72 hours, then antibiotics should be started. • The choice of antibiotics is based on suspected pathogens, individual and community risk of resistant strains, immune status, antibiotic properties, tolerability, and cost. • The first-line therapy should be narrow-spectrum antibiotics with high effectiveness, low adverse events, and low cost. Amoxicillin with or without clavulanate is one suitable antibiotic

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• The addition of clavulanate depends on likelihood of resistance from ß-lactamase-producing bacteria, severity, and individual comorbidity. The IDSA guideline recommends using amoxicillinclavulanate in both adults and children as the first line with regard to evidence of increasing resistant strains of H. influenzae. • High-dose amoxicillin formulation (90 mg/kg/day) should be considered in suspected cases of drug-resistant S. pneumoniae due to its penicillin-binding protein-3 mutation. The prevalence of strains is geographically different; however, risks must be considered under certain conditions: 65 years of age, systemic toxicity (fever ≥39°C, impending suppurative complication), attendance at daycare, prior use of antibiotics, recent hospitalization, or poor immune status. The downsides are the additional cost and potentially more adverse side effects. • The second-line antibiotics are doxycycline and levofloxacin/ moxifloxacin. • In patients with penicillin allergy, alternatives can be prescribed: doxycycline, levofloxacin/moxifloxacin, and clindamycin plus third-generation cephalosporin (in cases of non-type I hypersensitivity to penicillin). • The FDA has issued a black box warning for quinolone antibiotics with potentially “disabling side effects involving tendons, muscles, joints, nerves, and the central nervous system,” recommending they be reserved for treatment of rhinosinusitis “when there are no other options available.” • Antibiotics are given for 5 to 7 days in adults and 10 to 14 days in children. • Evidence shows no difference between short (5 days) versus long (10 days) courses of antibiotics; however, long courses are associated with more side effects, higher cost, and poorer compliance.

Antihistamines • Oral antihistamines are only recommended for symptom relief for those who have concomitant allergic rhinitis.

Others • Evidence for antileukotrienes, oral decongestants, topical decongestants, and mucolytic drugs in ARS is still lacking. • Saline irrigation helps to relieve symptoms, facilitates pus removal, and promotes ciliary function.

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• Herbal regimens (eg, Sinupret, Esberitox, Bromelain, Myrtol, Pelargonium sidoides, Cineole, Sinfrontal) are found to have some benefits for symptom reduction in ARS; however, in ABRS these are considered as adjuncts and should be combined with antibiotics. • There is no supportive evidence for steam inhalation, probiotics, or vitamin C in acute rhinosinusitis.

Surgery • Generally, acute rhinosinusitis does not require surgical intervention; however, in a complicated case such as with orbital and intracranial complications, endoscopic sinus surgery (ESS) is considered definitive treatment. • Criteria for orbital complication requiring ESS are subperiosteal abscess with either no response to appropriate intravenous antibiotics for at least 24 to 72 hours or deterioration of visual acuity. In cavernous sinus thrombosis, ESS contributes to treatment by facilitating the removal of the source of infection. • Antral puncture may be indicated for two reasons:  one is to obtain bacterial culture for either definitive diagnosis in clinical research or for antibiotic selection; the other is to relieve the pain of maxillary sinusitis when followed by antral irrigation. • Adenoidectomy is not warranted for a single episode of ARS. Evidence shows benefits in recurrent acute sinusitis cases, especially if accompanied by obstructive symptoms like adenoid hypertrophy with otitis media or obstructive sleep apnea. • Balloon sinuplasty has no role in ARS; however, small case series have shown good outcomes when combined with aggressive antibiotics in critically ill and immunocompromised patients. • Procedures such as nasoantral windows, inferior antrostomies, and Caldwell-Luc operations do not have proven benefit in treating the disease.

Conclusions • ARS is an inflammatory condition, mainly resulting from a virus, and is essentially self-limited. • Antral puncture with aspiration is the gold standard for collecting reliable bacterial cultures. • Intranasal corticosteroid is a standard treatment for uncomplicated ARS and should be combined with antibiotics for ABRS. • Antibiotics are effective for ABRS; however, “watchful waiting” is acceptable and should be considered in uncomplicated cases.

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• Surgical intervention like endoscopic sinus surgery is reserved for complicated cases. • Figure 15–3 presents a brief algorithm of diagnosis and treatments for ARS.

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Figure 15–3.  Algorithm for diagnosis and treatment of acute rhinosinusitis. Abbreviations: URI, upper respiratory tract infection; VRS, viral rhinosinusitis; ABRS, acute bacterial rhinosinusitis; INCS, intranasal corticosteroid; IV, intravenous.

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References • Ahovuo-Saloranta A, Rautakorpi UM, Borisenko OV, Liira H, Williams JW Jr, Makela M. Antibiotics for acute maxillary sinusitis in adults. Cochrane Database Syst Rev. 2014;CD000243. • Chow, AW, Benninger MS, Brook I, et al. IDSA clinical practice guideline for acute bacterial rhinosinusitis in children and adults. Clin Infect Dis. 2012;54:e72–e112. • Fokkens WJ, Lund VJ, Mullol J, et al. Position paper on rhinosinusitis and nasal polyps. Rhinol. 2012;50(1):1–298. • Meltzer EO, Bachert C, Staudinger H. Treating acute rhinosinusitis: comparing efficacy and safety of mometasone furoate nasal spray, amoxicillin, and placebo. J Allergy Clin Immunol. 2005;116:​ 1289–1295. • Payne SC, Benninger MS. Staphylococcus aureus Is a Major Pathogen in Acute Bacterial Rhinosinusitis: A Meta-Analysis Clinical Infectious Diseases. 2007;45(10) e121–127. • Rosenfeld RM, Piccirillo JF, Chandrasekhar SS, et al. Clinical practice guideline (update): adult sinusitis. Otolaryngol Head Neck Surg. 2015;152:S1–S39. • Venekamp RP, Boten MJ, Rovers MM, et al. Systemic corticosteroid monotherapy for clinically diagnosed acute rhinosinusitis: a randomized controlled trial. CMAJ. 2012;184(14): e751–7

Chapter

16 Management of Chronic Rhinosinusitis Kristine A. Smith and Luke Rudmik

Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 204 Medical Management of Chronic Rhinosinusitis . . . 204 Topical Intranasal Therapies . . . . . . . . . . . . . . . . . . . . . . . 204 Systemic Therapies . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 207 Allergy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 211 Biologics . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 212 Surgical Management of Chronic Rhinosinusitis . . 214 Appropriate Medical Therapy . . . . . . . . . . . . . . . . . . . . . . 214 Refractory Chronic Rhinosinusitis . . . . . . . . . . . . . . . . . 215 Recalcitrant Chronic Rhinosinusitis . . . . . . . . . . . . . . . . . 215 Indications for Endoscopic Sinus Surgery . . . . . . . . . . . 215 Preoperative Care . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 216 Intraoperative Care . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 216 Postoperative Care . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 217 Conclusions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 217

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Introduction • Chronic rhinosinusitis (CRS) is a common inflammatory disease of the sinonasal cavity. • The etiology of the inflammation is highly varied. 1. CRS without nasal polyposis (CRSsNP) is characterized by Th-1 mediated inflammation. • Long-term medical therapy is essential to control inflammation and optimize patient outcomes. • Given the differences in risks and benefits from various treatment options, it is important to incorporate patient preferences into treatment decisions. • Endoscopic sinus surgery (ESS) is an important component in the management of CRS when medical therapy does not control disease symptoms. • The objective of this chapter is to discuss the maintenance and perioperative medical management of CRS.

Medical Management of Chronic Rhinosinusitis • There is an abundance of medical strategies for the management of CRS. • This section will review the available medical options, their modalities, risks and benefits, and current recommendations. • Appropriate medical therapy will be reviewed.

Topical Intranasal Therapies Saline Irrigations • Sinonasal saline irrigations are a common treatment modality for CRS. • Sinonasal irrigations serve multiple functions including: 1. Removing pathologic mucous 2. Removing environmental triggers 3. Aiding in mucociliary clearance • High-volume saline irrigations can also be used to deliver medications to the sinonasal cavity (ie, budesonide irrigation). • Saline irrigation has been shown to: 1. Improve symptom scores 2. Be more effective when combined with topical intranasal corticosteroids

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• The risks of use include nasal burning/irritation, ear plugging, and nausea. • Isotonic and hypertonic solutions are equally effective. 1. Hypertonic solutions may provide a decongestant effect but have a higher risk of nasal burning. 2. Hypotonic solutions are not recommended. • Higher volume irrigations (>100 ml) are more effective than low volume options • A frequency of 1 to 3 times a day is recommended • Their use is recommended in CRSwNP and CRSsNP.

Topical Intranasal Corticosteroids • Topical intranasal steroids (INS) play a role in managing CRS by: 1. Reducing sinonasal inflammation 2. Decreasing vascular permeability 3. Reducing glycoprotein release from submucosal glands • INSs have the highest grade of evidence to support their use in CRS (Grade A). • INSs are associated with: 1. Improvement in overall symptom score 2. Improvements in quality of life (QoL) 3. Reduction in polyp size (CRSwNP) • The primary risks include local irritation, epistaxis, headaches, and systemic absorption. 1. The risk of systemic absorption with exogenous hypothalamic pituitary adrenal axis suppression is low. • Multiple formulations are available—no single one has been shown to be superior to the others. • Their use is strongly recommended in the management of CRSwNP and CRSsNP.

Topical Intranasal Antibiotics • The use of topical antibiotics is thought to provide higher concentrations of antibiotics to the sinonasal cavity without subjecting patients to the systemic side effects. • The mechanism of action of topical antibiotics is linked to the type of antibiotic as well as the offending bacteria. • There is little evidence supporting use for routine cases of CRS. 1. Mupirocin irrigations (high volume) may be helpful in CRSsNP patients with positive S. aureus culture. 2. Recalcitrant cases of CRS, including cystic fibrosis and primary immunodeficiency, may benefit from topical antibiotic therapy.

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• Currently, the evidence recommends against the routine use of topical antibiotics in the management of CRS; however, topical antibiotics may be effective in select cases of recalcitrant CRS.

Topical Intranasal Antifungals • Fungal colonization is present in >95% of the population. • The role that colonized sinonasal fungus plays in the pathophysiology of CRS is unknown. • Topical antifungals, namely amphotericin B, have been trialed to attempt eradication of fungal colonization and reduction of the resulting inflammation. 1. Trials have failed to demonstrate a clinical benefit when utilizing topical amphotericin B. • Currently, this evidence recommends against the routine use of topical antifungals in the management of CRS

Alternative Topical Therapies • Many alternative topical therapies have been evaluated such an Manuka honey, surfactants, and xylitol. • Given the paucity of research on the alternative treatments, it is challenging for guidelines to provide recommendations. • Alternative topical therapies are often used in patients with recalcitrant CRS.

Surfactants • Surfactants are amphipathic molecules that act as mucolytics to decrease mucous viscosity; they may have antibacterial or antibiofilm properties. • There is a high rate of local complications including local irritation, ciliary dysfunction, and olfactory dysfunction. • Baby shampoo drops placed into isotonic saline irrigation have been studied as a topical surfactant. • There may be minor improvements in post-nasal drip when using baby shampoo irrigations for CRS. • The use of topical sinonasal surfactants carries a risk of olfactory injury.

Manuka Honey • Manuka honey is a naturally occurring honey with natural antibacterial properties against staphylococcus and pseudomonas biofilms.

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• This property is thought to be secondary to the high levels of methylglyoxal. • In vitro findings (antibiofilm properties) have not been reproduced in clinical trials, however, and data supporting a clinical benefit are limited.

Xylitol • Xylitol is a naturally occurring sugar alcohol. • It is often use in patients with xerostomia. • In addition, it has antimicrobial properties secondary to altering salt properties in sinonasal secretions. • In vivo, it is associated with decreased bacterial loads, specifically pseudomonas. • A study examining the efficacy of xylitol irrigations did show improvements in the disease specific QoL in CRS patients. • Although further study is required, xylitol may be of benefit in selected patients.

Delivery Methods • A variety of delivery methods for topical medications exist: 1. High volume (>100 ml) irrigations 2. Meter-dose low-volume sprays 3. Atomizer 4. Nebulized solutions 5. Nasal drops • Evidence suggests that high-volume irrigations (ie, budesonide irrigations) provide the best distribution and penetration of the paranasal sinuses. • Head position during the topical delivery significantly impacts the distribution to the sinonasal cavity. 1. For high-volume irrigations, the head down forward position provides the best sinonasal penetration and delivery. 2. For low-volume solutions, such as nasal drops, the lying head low and lying head back positions have the best penetration to the olfactory cleft and the nasal cavity.

Systemic Therapies Systemic Corticosteroids • Systemic corticosteroids are commonly used to manage some conditions: 1. CRS with nasal polyposis

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2. Acute exacerbation of CRS (AECRS) • The mechanism of action is similar to topical intranasal steroids in decreasing sinonasal inflammation through a variety of pathways including: 1. Reducing circulating leukocytes (neutrophils, eosinophils, and basophils) 2. Decreasing vascular permeability 3. Reducing glycoprotein release from submucosal glands 4. Inhibiting histamine and leukotriene release (from basophils) 5. Inhibiting arachidonic acid metabolites to decrease inflammation 6. Inhibiting neuropeptide induced inflammation but increasing enzymatic degradation • The use of a short course of systemic corticosteroids have been shown: 1. Reduce polyp size 2. Improve QoL 3. Improve sinonasal symptoms • The specific dosing for short courses of systemic corticosteroid is variable but often includes prednisone (30 mg to 60 mg ,once per day for 10 to 21 days; the taper depends on dose and duration). • Of note, the improvements from a short course of systemic corticosteroids are not sustained past three months in the absence of continued topical intranasal steroid therapy. • There are significant potential adverse effects associated with the use of systemic corticosteroids: 1. Skin thinning 2. Purpura 3. Weight gain 4. Cataracts 5. Glaucoma 6. Cardiovascular disease 7. Pancreatitis 8. Hypertension 9. Osteoporosis 10. Acute psychosis 11. Hypothalamic pituitary adrenal axis suppression 12. Adrenal crisis (when steroids stopped) 13. Hyperglycemia 14. Immune system suppression/infection prone • Use of systemic corticosteroids should be assessed on a caseby-case basis. Clinicians should involve patients in a shared decision-making process in order to balance the benefits with the associated risks.

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Systemic Antibiotics • As our understanding of the underlying pathophysiology of CRS has increased, the reliance on systemic antibiotics to manage CRS has decreased. • Systemic antibiotics may be divided into two therapeutic options: 1. Short-term antibiotics (typically 12 weeks) of anti-inflammatory antibiotics have been used in the management of CRSsNP. • Anti-inflammatory antibiotics work to reduce inflammation in a number of ways: 1. Inhibit the NF-kB pathway to reduce production of IL-2, IL-6, IL-8, TNF-alpha, GM-CSF (inhibit fibroblast and neutrophil activation) 2. Block TGF-beta function 3. Reduce MMP production 4. Scavenge reactive oxygen molecules 5. Improve cilia function and clearance • Anti-inflammatory antibiotics include: 1. Macrolides 2. Trimethoprim-sulfamethoxazole 3. Tetracyclines • Common long-term anti-inflammatory macrolide antibiotics include: 1. Clarithromycin:  250 to 500 mg QD for 12 weeks

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2. Azithromycin:  500 mg QD for 3 days then once per week for 12 weeks 3. Roxithromycin:  150 mg QD for 12 weeks • Their use is associated with improved clinical outcomes: 1. Improved symptoms 2. Improved QoL • These improvements may be sustained up to 12 weeks after completing therapy • Long-term macrolide therapy includes the following risks: 1. Myopathy 2. QT prolongation 3. Rhabdomyolysis 4. Multiple drug interactions • There is evidence to support the use of long-term antiinflammatory antibiotic therapy (>12 weeks) in patients with CRSsNP. • Doxycycline can be considered in patients with CRSwNP.

Leukotriene Pathway Antagonists • Leukotrienes are inflammatory mediators that play a role in the pathophysiology of CRS through a variety of mechanisms including the following: 1. Increased vasodilation and decreased vascular permeability 2. Worsening mucosal edema 3. Increasing mucous secretions 4. Increasing eosinophilic infiltration and inflammation • Leukotriene pathway antagonists (LTA) are a class of medication that block leukotrienes D4, C4, and E4 from binding to the cysteinyl leukotriene receptors. • This blocks the production of leukotrienes from arachidonic acid by inhibiting 5-lipooxygenase, thus decreasing the inflammatory effects of leukotrienes. • Montelukast and Zilueton are oral LTAs used in patients with severe asthma and may improve symptoms in CRSwNP (compared to placebo). • Potential adverse effects of LTAs include the following: 1. Headaches 2. GI Upset 3. Rash 4. Neuropsychiatric episodes (Montelukast) 5. Elevated liver enzymes (Zileuton) • LTA therapy can be considered for patients with CRSwNP.

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Allergy • Approximately 20% to 60% of CRS patients suffer from concurrent allergic rhinitis. • Whether allergy plays a causal role in the pathogenesis of CRS remains unclear. • CRS patients with a significant allergic component receive some improvement in their disease-specific QoL with treatment of the allergic component. • As such, a thorough allergic work-up and treatment of comorbid allergy are recommended in all CRS patients with concurrent allergic symptoms.

Antihistamines • Antihistamines (topical and oral) reduce inflammation by reducing histamine release which: 1. Decreases vascular permeability 2. Decreases vasodilation 3. Decreases mucous production, reducing nasal secretions • Antihistamines are beneficial in allergic rhinitis, and their use is recommended in CRS patients with concurrent allergic symptoms such as: 1. Itchy, watery eyes 2. Itchy nose 3. Sneezing 4. Watery rhinorrhea • The risks of topical and oral antihistamines include: 1. Sedation 2. Xerostomia 3. Xerophthalmia 4. Anticholinergic CNS effects (confusion) • Antihistamines are recommended to treat comorbid allergic rhinitis. • The clinical benefit of antihistamines on symptoms of CRS is unclear.

Allergy Immunotherapy • The usefulness of immunotherapy in allergic disease has been well established. • Allergy immunotherapy is indicated for: 1. Moderate to severe allergic rhinitis in patients with persistent symptoms who, despite allergy avoidance and topical or

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oral antihistamines, have demonstrable evidence of specific Immunoglobulin-E (IgE) antibodies to clinically relevant allergens. • Subcutaneous immunotherapy (SCIT) and sublingual immunotherapy (SLIT) involve exposing the patient to progressively larger doses of their respective allergen(s) in order to down-regulate antigenspecific IgE and eventually decrease the immune reaction to the allergy. • The risks of immunotherapy include a risk of anaphylaxis and involve long (multiple years) duration of treatment. • Immunotherapy may be useful in CRS patients with severe refractory allergic disease.

Aspirin Desensitization • A subset of patients with CRS suffer from aspirin (ASA) exacerbated respiratory disease (AERD). • Desensitizing these patients to ASA may improve their pulmonary and sinonasal disease. • ASA desensitization has a risk of significant adverse events including anaphylaxis and gastric bleeding; therefore it should be performed in an appropriately equipped setting. • ASA desensitization may be considered in patients with AERD who are refractory to both medical and surgical therapies.

Biologics • As the pathophysiology of CRS has been further defined, there is an expanding role for therapies that target specific disease processes. • Due to limited evidence, most of these novel biologic therapies are utilized in the setting of a clinical trial as opposed to routine clinical practice.

Anti-Immunoglobulin-E Therapy • Immunoglobulin-E (IgE) is an inflammatory mediator responsible for mast cell activation. • In CRS, many CRSwNP patients with concurrent asthma and allergy have been found to have elevated serum IgE levels. • Monoclonal anti-IgE antibodies have been developed; they function by binding free serum IgE and decreasing the availability of IgE receptors on 1. Mast cells

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2. Basophils 3. Dendritic cells • Omalizumab is an anti-IgE monoclonal antibody approved for use in patients >6 years of age with moderate to severe uncontrolled allergic asthma. • Potential adverse effects of omalizumab include: 1. Headaches 2. Injection site reactions 3. Pulmonary embolism/deep vein thrombosis 4. Cardiovascular (myocardial infarction, angina) • The use of omalizumab has been evaluated in CRSwNP patients with elevated IgE levels and is associated with mild symptomatic improvements.

Anti-Interleukin-5 Therapy • Over 80% of CRSwNP is associated with eosinophilia, especially for patients with a history of AERD and asthma. • IL-5 may play a role in the pathophysiology of CRSwNP; elevated serum and tissue levels have been documented in CRSwNP. • Interleukin-5 (IL-5) mediates eosinophilic inflammation by increasing growth, recruitment, and activation. • Anti-IL-5 therapy involves humanized IgG monoclonal antibodies that bind free IL-5 and impair eosinophil-mediated inflammation. • Mepolizumab and reslizumab are commercially available anti-IL-5 therapies. 1. Mepolizumab is currently approved for use in severe asthma. • Anti-IL-5 monoclonal antibodies have been shown to induce eosinophil apoptosis and reduce tissue eosinophilia. • In CRSwNP, anti-IL-5 therapy may decrease polyp size and serum eosinophil levels. • Potential adverse effects of anti-IL-5 therapy includes: 1. Headache 2. Opportunistic infection (urinary tract, respiratory tract, herpes zoster) 3. Injection site reactions

Anti-Interleukin-4 Therapy • Interleukin-4 (IL-4) is a mediator of type 2 helper T cell mediated inflammation. • IL-4 has also been shown to potentiate the immune response of fibroblasts in CRS.

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• CRSwNP is characterized by this type of inflammation. • Dupilumab is a monoclonal anti-IL-4 that targets the α subunit of the IL-4 receptor (Rα) and inhibits the subsequent signal pathways. • Dupilumab has been shown to improve other type 2 helper T cell mediated diseases such as asthma and atopic dermatitis. • Potential adverse effects of dupilumab include: 1. Headache 2. Upper respiratory tract infection 3. Injection site reactions 4. Rash • In CRSwNP, a single randomized control trial showed significant improvement in symptom scores and QoL in patients receiving dupilumab (compared to placebo).

Surgical Management of Chronic Rhinosinusitis Appropriate Medical Therapy • Appropriate medical therapy refers to the minimum medications that should be used before diagnosing a patient with refractory CRS. • Refractory CRS refers to persistent symptoms and reduced QoL despite appropriate medical therapy. • The appropriate medical therapy for CRSsNP includes: 1. Topical sinonasal irrigations 2. Topical intranasal steroids for a minimum of 8 weeks 3. Plus either – A short course of broad spectrum/culture-directed systemic antibiotics (2 to 3 weeks) or – A prolonged course of systemic low-dose anti-inflammatory antibiotic (ie, macrolide therapy) for 12 weeks • The appropriate initial medical therapy for CRSwNP includes: 1. Topical sinonasal irrigations 2. Topical intranasal steroids for a minimum of 8 weeks 3. A short course of systemic corticosteroids (1 to 3 weeks) • The use of systemic corticosteroids or antibiotics may be avoided in patients with contraindications such as: 1. Diabetes mellitus 2. History of psychological illness 3. Massive nasal polyposis where topical intranasal steroids cannot be used after a short course of systemic corticosteroids 4. Presence of other contraindications to systemic corticosteroids

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Refractory Chronic Rhinosinusitis • Refractory CRS refers to patients who have persistent symptoms and reduced QoL following appropriate medical therapy (described in Appropriate Medical Therapy). • Ineffective delivery techniques (such as aiming towards the nasal septum with topical intranasal sprays) may contribute to medication failure. • Poor compliance may put patients at higher risk for postoperative complications and disease recurrence. • Educating patients about the chronicity of the disease and the need for daily medical treatment may optimize technique and compliance.

Recalcitrant Chronic Rhinosinusitis • Recalcitrant CRS generally refers to patients with persistent symptoms and reduced QoL despite receiving ESS with continued postoperative medical therapy. • These patients may have severe sinonasal inflammation and may require revision surgery for postoperative synechia, scarring, or recurrent polypoid disease. • These patients may have underlying disease processes such as granulomatous disease, immune deficiencies, or ciliary abnormalities. • A multidisciplinary approach to these patients to optimize contributing factors may be beneficial (ie, rheumatology, immunology).

Indications for Endoscopic Sinus Surgery • Patients who fail appropriate medical therapy and continue to suffer from the impact of CRS on their daily functioning are the most appropriate candidates for ESS. • Current Appropriateness Criteria for ESS in patients with uncomplicated CRS include the following: 1. Received appropriate medical therapy (see Appropriate Medical Therapy section) 2. Persistent reductions in QoL beyond population norms (SNOT-22 score >20) 3. Radiologic confirmation of CRS (Lund-MacKay CT score >1) • These patients may benefit from ongoing medical therapy, but those with significant symptoms may receive greater benefit from surgical intervention.

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• Complicated cases of CRS often have other factors that should be considered when considering ESS and include, but are not limited to: 1. Cystic fibrosis 2. Organ transplant with immunosuppression 3. Chemotherapy with immunosuppression 4. Autoimmune disease affecting the paranasal sinuses 5. Systemic vasculitis 6. Systemic granulomatosis 7. Ciliary dyskinesia 8. Mucocele formation 9. Invasive fungal rhinosinusitis 10. Fungal balls 11. Structural, non-inflammatory CRS (mucous recirculation) 12. Intracranial or orbital complications (impending or current) • Patients with these conditions may be candidates for ESS despite the degree of prior medical therapy and severity of symptoms. • Careful consideration of patient factors—and the risks and benefits of ESS—is essential in order to optimize patient selection and outcomes.

Preoperative Care • If a patient is deemed to be an appropriate operative candidate for ESS, preoperative optimization is essential to facilitate successful surgery. • Preoperative medication may include: 1. Topical sinonasal irrigations 2. Topical intranasal corticosteroids 3. A course of systemic corticosteroids (CRSwNP only) • Perioperative corticosteroids are used to decrease sinonasal inflammation in the perioperative period and are associated with improved endoscopic grades postoperatively in CRSwNP. • Many patients are put on 10 to 14 day courses, with half the course preoperatively and half the course postoperatively. • Caution should be used in prescribing these systemic medications to patients with medical comorbidities like diabetes. • Preoperative consultations with internal medicine and anesthesia may be beneficial in medically complex patients to ensure the safety of the preoperative medications.

Intraoperative Care • Intraoperative management is largely decided by the anesthesia team.

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• Relative hypotension may help to reduce blood loss, however, and improve surgical visualization. • Transexamic acid has been suggested as a possible way to decrease intraoperative blood loss, although there is currently no evidence to support or refute this. 1. Transexamic acid has been associated with an increased risk of deep vein thrombosis, particularly in patients with reduced mobility postoperatively (eg, hip fractures). • Middle meatal packing may be used and may be impregnated with corticosteroids or antibiotics.

Postoperative Care • Following ESS, patients should resume their usual medical regime as soon as possible to treat the ongoing and underlying sinonasal inflammation. • High-volume sinonasal irrigations are important to aid in debridement of sinonasal debris and blood, and aid in restoring mucociliary clearance; irrigations should be started 24 to 48 hours postoperatively. • A short course of systemic antibiotics may also be useful in preventing postoperative sinusitis, improving short-term symptoms, and reducing postoperative crusting. • Topical intranasal steroids may be resumed immediately; however, the significant crusting in the postoperative period likely obstructs the delivery of medication. • Topical intranasal steroids are commonly restarted 1 to 2 weeks postoperatively. • In addition to ongoing medical therapy, an important component of operative care includes postoperative debridements. • Patients are often seen at 1 and 3 weeks postoperatively for debridement of retained products and the removal of fibrinous debris, which may promote inflammation in the postoperative period and increase the likelihood of postoperative stenosis and scarring. • Continued medical therapy is essential for successful longterm outcomes and is usually comprised of high-volume saline irrigations and topical intranasal corticosteroids.

Conclusions • Medical management is the cornerstone for the management of CRS.

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• Long-term therapy is required to control the underlying sinonasal inflammation, even following ESS. • High-volume saline irrigations and topical intranasal steroids are recommended as a part of the initial and ongoing management of both CRSwNP and CRSsNP. • Systemic corticosteroids are useful in CRSwNP, and may be beneficial in selected CRSsNP patients. • Short courses of systemic antibiotics are most useful in addressing the acute exacerbation associated with CRS; culture-directed antibiotics are recommended where possible. • A trial of prolonged macrolide therapy may be useful in CRSsNP. • Topical antifungals and antibiotics are not recommended for routine use. • The treatment of concurrent allergic disease with appropriate therapy (antihistamines, avoidance, and immunotherapy) is recommended. • Many new biologic therapies are being developed. Currently none are recommended for routine use, although further study may reveal a role for these therapies (anti-IgE, anti-IL-5, anti-IL-4).

Chapter

17 Fungal Sinusitis Patrick Colley and Charles S. Ebert, Jr.

Fungal Rhinosinusitis . . . . . . . . . . . . . . . . . . . . . . . . . . . . 220 Invasive Fungal Rhinosinusitis . . . . . . . . . . . . . . . . . . . . . 220 Non-Invasive Fungal Sinusitis . . . . . . . . . . . . . . . . . . . . . 227 Acronyms . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 233

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Fungal Rhinosinusitis • Fungal rhinosinusitis is often divided into two major categories: 1. Invasive fungal rhinosinusitis 2. Non-invasive fungal rhinosinusitis • Amongst the invasive category, there are three further subdivisions: 1. Acute invasive fungal sinusitis 2. Chronic invasive fungal sinusitis 3. Granulomatous fungal sinusitis • The non-invasive fungal sinusitis category is composed of two subdivisions: 1. Allergic fungal sinusitis 2. Fungal balls

Invasive Fungal Rhinosinusitis Acute Invasive Fungal Sinusitis Epidemiology • Acute invasive fungal sinusitis (AIFS) is a fungal infection of the sinonasal mucosa characterized by invasive fungal elements with infarction and necrosis. • The infection typically progresses rapidly and requires emergent attention. • Invasive fungal infections are possible in other areas of the body such as the pulmonary and GI tract as well as the skin. • The species most commonly associated with AIFS are saprophytic fungi of the order Mucorales as well as Aspergillus.

Risk Factors • The majority of patients with AIFS have some underlying disease that predisposes them to this type of infection; the most common underlying diseases are: 1. Diabetes mellitus, particularly with ketoacidosis 2. Hematologic malignancies 3. Hematopoietic stem cell transplantation 4. Solid organ transplantation 5. Glucocorticoid use 6. Treatment with deferoxamine 7. Iron overload 8. AIDS 9. Injection drug use 10. Trauma/burns 11. Malnutrition

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Diabetes • Hyperglycemia, typically with an associated metabolic ketoacidosis, has historically been the most common underlying condition in patients diagnosed with AIFS. 1. Recent studies have shown that hematologic malignancy is now more commonly the predisposing condition in AIFS patients. • An enzyme found in Rhizopus organisms, ketone reductase, allows them to grow rapidly in high glucose, acidic conditions.

Malignancy • Hematologic malignancies are more frequently associated with AIFS than solid tumors. • AIFS occurs in less than 2% of patients with hematologic malignancies. 1. The highest incidence is in patients with graft versus host disease. • One identified independent risk factor for AIFS among patients with hematologic malignancies is voriconazole prophylaxis. 1. Voriconazole use is thought to select for mucormycosis by decreasing aspergillosis and other competitive fungi in the microbiome.

Solid Organ Transplantation • The cumulative rate of invasive fungal infections in solid organ transplant patients is less than 1%. • Risk factors for AIFS among solid organ transplant recipients include renal transplantation, renal failure, diabetes, and prior voriconazole or caspofungin use.

Iron Overload and Defuroxamine • Iron overload and subsequent treatment with deferoxamine increases the risk of mucormycosis by enhancing its growth and pathogenicity. 1. Deferoxamine acts as a siderophore for Rhizopus species and increases iron uptake that stimulates fungal growth.

Outcomes • AIFS carries a poor prognosis with a mortality rate as high as 50% despite aggressive intervention. • Important prognostic factors in patients with AIFS are absolute neutrophil count, bilateral involvement, and intracranial involvement. 1. Patient age, gender, cause of immunodeficiency, and fungal agent have not been associated with outcomes.

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Diagnosis Clinical • AIFS usually presents with symptoms similar to acute sinusitis such as fever, nasal congestion, purulent nasal discharge, headache and facial pain • A high degree of suspicion must be maintained in the immunocompromised patient with sinus complaints, particularly facial pain or headache. • Nasal endoscopy and a detailed head and neck examination are crucial for early detection of this condition. • The fungi in AIFS are angioinvasive; infarction of infected tissues is a hallmark of invasive disease. • Pale or necrotic areas with a black eschar due to vascular invasion can appear on the nares, septum, turbinates, oral mucosa, or any other affected area. • Sensation is frequently lost surrounding the involved areas. • A biopsy of suspicious tissue should be obtained and brought to pathology for prompt frozen specimen examination. 1. Multiple biopsies of insensate or necrotic areas should be obtained to increase the sensitivity of this examination. • The most predictive features for AIFS are an absolute neutrophil count below 500/uL and mucosal abnormalities of the septum or turbinates. • Orbital involvement manifests with periorbital edema, proptosis, and changes in vision. • The infraorbital nerve is the most frequently involved cranial nerve and presents with numbness over the malar eminence. • Involvement of the ethmoid or sphenoid sinuses should prompt concern for intracranial spread. 1. MRI demonstrates involvement of the cavernous sinus or intracerebral contents and should be obtained but should not delay excision of the involved tissue. • Hematogenous spread of AIFS is rare but should be considered if new lesions appear elsewhere during the treatment course.

Radiology • CT scan of the sinuses should be obtained with contrast in patients suspected of having AIFS; contrast will not be taken up by devascularized or necrotic tissue. • The areas that should be evaluated on CT scans include: 1. Periantral fat pad thickening 2. Bone dehiscence or thickening 3. Orbital contents

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4. 5. 6. 7.

Nasal septum Pterygopalatine fossa Nasolacrimal duct Lacrimal sac – An abnormality associated with one of these variables has a sensitivity of 95%, meanwhile involvement of two variables has a sensitivity and specificity of 100%. • An MRI with contrast should be obtained if there is concern for intracranial involvement based on the CT findings or clinical examination.

Histopathology • Diagnosis of AIFS relies primarily upon identification of fungi invasion into tissue by histopathology with culture confirmation. • Staining for fungus requires the use of calcofluor white and methenamine silver stains. • Histopathology reveals hyphal invasion of the mucosa, submucosa, and blood vessels with thrombosis and tissue infarction. • The hyphae of Mucorales are different from those of other fungi and often allow for identification. 1. Mucorales hyphae are broad (5 to 15 microns in diameter) with irregular, 90-degree branching and lack of septations. 2. Ascomycetous molds such as Aspergillus are narrower (2 to 5 microns in diameter) and have regular branching with many septations. • Serum tests such as the (1->3)-beta-D-glucan assay and the Aspergillus galactomannan assay can detect fungal wall components and can be used to aid in the diagnosis of AIFS. 1. Neither test is positive with mucormycosis as Mucorales does not share these wall components. • The presence of hyphae invading into tissue provides a presumptive diagnosis that should lead to further clinical evaluation and treatment. 1. The absence of hyphae does not rule out AIFS when the clinical picture is highly suggestive. – Further evaluation with imaging and possibly surgical exploration may be indicated. • Cultures of the biopsy specimen are important to distinguish Mucormorales from Aspergillus or other molds as the antifungal therapies may differ. 2. Treatment with liposomal amphotericin should be continued until the cultures are complete. – Mucormorales is less sensitive to triazoles than other fungi, and should be the presumed infectious organism until culture shows otherwise.

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Management • Prompt intervention plays an important role in the care of patients with AIFS, and has been shown to lead to improved outcomes. • Treatment of AIFS involves a combination of surgical debridement of involved tissues and antifungal therapy.

Antifungal Therapy • Immediately after the diagnosis of AIFS is made, antifungal therapy should be started. • Liposomal amphotericin B is the appropriate first-line agent due to its broad antifungal coverage. 1. Use of the liposomal formation is particularly important for patients with renal insufficiency. • If tissue culture reveals an organism other than Mucorales, antifungal therapy can be changed to an alternative medication. • Infections due to Aspergillus are treated with voriconazole with or without an echinocandin (caspofungin, micafungin, or anidulafungin). • Oral itraconazole, posaconazole, or isavuconazole can all be used for stepdown therapy after the acute stage of AIFS from any organism has passed. 1. This can take up to 3 or 4 weeks or until the patient shows signs of clinical improvement. • Posaconazole or isavuconazole can also be used as salvage therapy for the patient who does not respond to, or is unable to tolerate, amphotericin B.

Other Medical Management • Correction of the predisposing cause of the infection such as hyperglycemia, metabolic acidosis, iron overload, neutropenia, or immunosuppressive drugs is a critical part of treatment of AIFS. • Patients who are neutropenic secondary to a hematologic malignancy or its treatment are also frequently pancytopenic. 1. Transfusions of appropriate blood products may be required prior to surgical debridement. • Hyperbaric oxygen therapy has been used in the treatment of AIFS, but the benefit of this therapy has not been established.

Surgery • After the diagnosis of AIFS is made, the patient should be scheduled for prompt surgical debridement of the involved tissue. • Imaging studies should be formatted appropriately to allow them to be used for image guidance during the debridement. 1. Normal anatomic landmarks are often altered by the infection.

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• Surgical debridement of all necrotic tissue will often be disfiguring, potentially requiring removal of the palate, nasal cartilages, or the orbit. 1. Surgical goals and options should be discussed with the patient. 2. Discussion of possible orbital exenteration if there is orbital involvement should be discussed prior to the procedure. • In general, debriding or violating the skull base should be avoided as this is difficult to repair in the setting of AIFS. • Orbital exenteration is typically avoided unless there is obvious ocular dysfunction or frank orbital involvement, and the patient or his or her caregiver has consented to this procedure. • Serial debridements may be required during the acute phase of the infection. • Surgery is not curative, but it is believed to serve as a means of decreasing the fungal load to allow for improved response to antifungal therapy.

Chronic Invasive Fungal Sinusitis Epidemiology • Chronic invasive fungal sinusitis (CIFS) presents in a similar manner to chronic rhinosinusitis (CRS) with few, if any, systemic complaints. • Patients with this condition are often older and only mildly immunocompromised. 1. Diabetes mellitus and glucocorticoid use are risk factors for CIFS. 2. This condition has also been reported in intranasal cocaine users. • Aspergillus and brown molds are the fungi responsible for CIFS. 1. Mucorales is not seen in this condition.

Diagnosis • Patients with CIFS have typical symptoms of CRS for months before the development of a complication that signifies the severity of this condition. 1. The most common complication is orbital involvement that manifests as visual changes. – Orbital apex syndrome can be seen with CIFS resulting in proptosis, decreased visual acuity, ophthalmoplegia, and decreased sensation over the forehead area. 2. The brain or cranial nerves can also be involved, causing neurologic sequelae.

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• CT findings are similar to those of AIFS: 1. Soft tissue mass frequently involving one paranasal sinus 2. Bony erosion • Serum tests such as the (1->3)-beta-D-glucan assay and the Aspergillus galactomannan assay can be used to aid in the diagnosis of CIFS. • Histopathology also is similar to AIFS, and demonstrates necrosis of the mucosa and blood vessels with low-grade mucosal inflammation.

Management • Surgical debridement of the involved tissue is the initial treatment of choice for CIFS. 1. Similar to AIFS, a thorough discussion should take place before the surgery regarding the extent of debridement and the potential removal of surrounding structures. • Antifungal therapy should be initiated immediately after diagnosis. 1. Mucorales is not seen in CIFS, so itraconazole or other triazoles can be alternative antifungual medications used instead of amphotericin B in the acute treatment of CIFS. • Antifungal therapy should be continued for an extended period of time until the infection has completely resolved. • The underlying immunocompromising factor should be treated promptly after diagnosis. 1. Patients with diabetes require strict glucose control. 2. Glucocorticoids should be stopped until resolution of the infection.

Granulomatous Fungal Sinusitis Epidemiology • Granulomatous fungal sinusitis (GFS) is a rare condition that has been reported almost exclusively in immunocompetent individuals from North Africa. • It is an invasive fungal sinusitis that is typically associated with Aspergillus flavus.

Diagnosis • Patients with GFS present with symptoms and a history consistent with CRS. • This condition often involves the orbit, resulting in proptosis or other compressive symptoms.

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• CT scan shows one or two opacified sinuses with a lesion isodense or hyperdense to muscle tissue with localized bony erosion rather than sinus expansion. • MR shows a mass that is isointense on T1 and hypointense on T2. • Definitive diagnosis requires the histopathologic findings of granulomas and multinucleated giant cells with pressure necrosis and erosion of the sinonasal mucosa.

Management • Treatment of this condition is similar to that of chronic invasive fungal sinusitis. • The primary treatment is surgical debridement of the involved tissue. 1. Surgery is required for diagnosis of GFS. 2. Large openings allow for endoscopic surveillance of this condition in the postoperative setting. 3. Excision of the involved tissue often results in improvement of the proptosis or other ocular findings. • After surgical removal of the involved tissue, systemic antifungal medications are given until the condition completely resolves. • Recurrence of GFS is rare.

Non-Invasive Fungal Sinusitis Allergic Fungal Sinusitis Epidemiology • Allergic fungal rhinosinusitis (AFS) is a distinct type of CRS that accounts for between 5% and 10% of all CRS cases. • It is one pathologic subtype of eosinophilic CRS. 1. Other subtypes include aspirin sensitive asthma with nasal polyps, allergic fungal sinusitis without fungus, S. aureusinduced superantigen rhinosinusitis, chronic gram-negative rhinosinusitis with nasal polyps, and eosinophilic chronic rhinosinusitis of unknown etiology. • AFS accounts for 7% to 12% of patients with chronic rhinosinusits who undergo sinus surgery in the United States. • AFS is most common among adolescents and young adults; the mean age at diagnosis is 21.9 years. • Patients with AFS are typically immunocompetent and often have asthma, eosinophilia, and elevated total fungus-specific IgE concentrations.

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• This condition is thought to result from chronic allergic inflammation directed against colonizing fungi. • The most commonly cultured fungi are Curvularia lunata, Aspergillus fumigatus, and Bipolaris and Drechslera species. • AFS patients differ from CRS patients in many socioeconomic factors. 1. AFS is found in a higher prevalence in younger patients, African Americans, uninsured patients, and patient from areas with a higher poverty rate. • The geographic incidence of AFS is mostly confined to the Mississippi River Basin and in the southern United States. 1. There are few reports of AFS outside of the United States. • The geographic distribution is thought to be secondary to the ambient mold spore concentration in these areas, although there are no studies to support this belief.

Pathogenesis • It is hypothesized that some external trigger, likely colonizing fungi, activates and upregulates the TH-2 and IL-5 pathways in the setting of a genetic predisposition toward the characteristic eosinophilic response. • The host becomes sensitized to fungal antigens, and the germinating fungal hyphae serve as antigens for a large allergic response. • The eosinophilic response causes massive degranulation and release of inflammatory mediators, cytokines, and growth factors that amplify the inflammatory response. 1. This response leads to nasal polyp formation, airway remodeling, and possibly bony demineralization. • Degranulating eosinophils cause thick, inspissated mucus that fills the sinuses and further damages the sinonasal mucosa. • Bacterial invasion of the damaged sinonasal mucosa leads to biofilm formation and worsening of the inflammatory process.

Diagnosis • Patients typically present with symptoms similar to those of CRS with the four cardinal symptoms of (1) rhinorrhea, (2) nasal obstruction, (3) facial pain or pressure, and (4) hyposmia. • The diagnosis of AFS involves major and minor criteria, as described in Table 17–1. 1. Bent and Kuhn Criteria • Definitive diagnosis of AFS typically requires surgical intervention as multiple major diagnostic criteria rely on pathology findings.

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Table 17–1.  Bent-Kuhn Criteria for Diagnosis of Allergic Fungal Sinusitis Major

Minor

Type I hypersensitivity to fungus

Asthma

Nasal polyposis

Bone erosion

Characteristic CT findings Eosinophilic mucin without invasion Positive fungal stain

Unilateral disease Fungal cultures Charcot-Leyden crystals Serum eosinophilia

• Type I hypersensitivity to fungus should be demonstrated by skin testing or in vitro immunoassay.

Radiology • As with all CRS, a sinus CT is typically the most informative type of radiology and serves as major diagnostic criteria. • CT reveals nasal polyposis with opacification of one or more sinuses. • The majority of cases involve multiple sinuses. 1. Up to one-half of cases present with unilateral disease. 2. In children, asymmetric disease predominates, with 70% of pediatric patients presenting with unilateral disease, as compared to only 37% of adults. • A characteristic CT finding in AFS is hyperattenuated areas within an opacified sinus. 1. Eosinophilic mucin that fills these opacified sinuses contains large amounts of protein and small amounts of water causing this characteristic hyperdense appearance. 2. This finding is often considered pathognomonic for AFS but actually only signifies the presence of allergic mucin, which can be found in other conditions. • MRI in AFS reveals decreased signal intensity on T1 weighted imaging and very decreased signal intensity on T2 weighted MRIs. 1. This was previously thought to be due to calcium, iron, and manganese within the mucin. – It is now believed, however, that the presence of inspissated mucus within the sinus cavity or along the polyps is what results in the increased hypointense T2 weighted signal. • Slow increase in the polyp burden and amount of allergic mucin result in local pressure on the bony walls of the sinus cavity.

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• This slow increase in pressure and the intense inflammatory reaction seen in AFS cause expansion of the sinus cavity and bony demineralization over time. 2. Actual bone erosion only occurs in 20% of cases. 3. More than 50% of AFS cases present with radiographic evidence of skull base or orbital extension of disease. – This is only noted in 5% of CRS patients. 4. The nasal cavity can also become expanded, widening the piriform aperture and causing hypertelorism.

Histopathology • Eosinophilic mucin is thick material ranging from light tan or brown to dark green. • Sheets of degranulating eosinophils and Charcot-Leyden crystals are seen within the mucin. 1. Charcot-Leyden crystals are spear-like structures formed from the degranulation of eosinophils. • Eosinophilic mucin is characteristic of AFS but can be seen in other subtypes of CRS. 1. In AFS the eosinophilic mucin contains fungal hyphae, identified by staining or culture. • Identifying fungal hyphae in mucus requires specific staining, such as Gomori methenamine silver, which stains fungal hyphae black or dark brown.

Management • As with all forms of CRS surgical and medical management both play important roles in the management of AFS.

Surgery • Endoscopic sinus surgery (ESS) serves three roles in the treatment of AFS: 1. Surgical intervention is typically required for diagnosis 2. ESS removes polyps, fungal debris, and allergic mucin – Widely opening the sinuses allows for complete removal of mucin and fungal debris. a. Residual fungus or mucin results in disease recurrence. – ESS allows for postoperative debridement and delivery of topical corticosteroids. 3. Removal of the polyps and mucin allows for reversal of the bony expansion. – Surgical and medical management has been shown to reverse proptosis and other orbital findings in AFS.

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• Allergic mucin can be challenging to remove from the sinuses due to its thick consistency. 1. Irrigation can be effective in the operating room at removing difficult to reach collections of mucin. • As with all sinus surgery, mucosa preservation to maintain mucociliary clearance is important. • Preoperative planning and intraoperative care must be taken in these patients as osseous expansion and erosion often distort the normal sinonasal anatomy and obliterate bony barriers, placing adjacent structures at an increased risk of iatrogenic injury. • Image guidance is particularly helpful for orientation and anatomic confirmation. • Aggressive saline irrigations are recommended in the immediate postoperative period.

Medical Therapy • Preoperative oral glucocorticoids have been shown to decrease bleeding and improve visualization in patients with polyps. • The steroids are typically continued after the surgery for an extended period of time, based on the appearance of the surgical cavity and mucosa. • Postoperative oral steroids are the mainstay of therapy in AFS, and can be used in conjunction with nasal saline irrigations and topical corticosteroids to control mucosal inflammation and recurrence. • Topical corticosteroids can be placed in the patient’s saline irrigations after surgery. 1. Budesonide or mometasone are easily available and can be added to the irrigations. • Serial examinations guide the need for continued systemic or topical therapy. • Fungal immunotherapy, based on the results of skin or in vitro testing, can be used as an adjunct to surgical or medical therapy in AFS. 1. Immunotherapy is safe and has been suggested to reduce the recurrence rate in AFS. • Systemic or topical antifungal medications have not shown a clear benefit in the treatment of AFS.

Future Therapies • Monoclonal antibodies to IgE, such as omalizumab, have a potential role in the treatment of AFS, given the large allergic response seen in this condition. 1. More studies are needed; however, early investigators showed a benefit based on a retrospective chart review.

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• Other monoclonal antibodies to inflammatory mediators such as IL 3, IL 4, and IL 5 may also have potential use in AFS.

Fungus Ball Epidemiology • Fungus balls present as a mass within the lumen of a single paranasal sinus. • The maxillary sinus is the most commonly involved sinus. 1. The sphenoid sinus is also frequently affected. • This condition is seen in immunocompetent individuals who are not sensitive to fungal antigens. • Fungal hyphae gather in dense collections and form fungus balls if there is obstruction of the paranasal sinuses. • Patients are often asymptomatic and the fungus ball is frequently discovered incidentally. • A. fumigatus and dematiaceous fungi are most commonly seen in sinus fungal balls.

Diagnosis • Clinically, patients with sinus fungal balls often have very few symptoms. 1. The inflammatory response and slow growth of the fungal ball, however, can cause compression of structures surrounding the involved sinus. • Endoscopic examination is often normal but can reveal edema or local polyposis surrounding the os of the involved sinus. • The fungal ball consists of thick white- or tan-colored debris that is easily suctioned and does not involve the surrounding mucosa. • No allergic mucin is present.

Radiology • Imaging of the head or paranasal sinuses is often how sinus fungus balls are discovered. • Upon CT imaging, metallic dense spots are seen within the fungus ball. 1. Fungal balls are hyperattenuated due to dense and matted fungal hyphae. – The inflamed mucosa lining the paranasal sinus is hypoattenuated on CT. 2. Punctate calcifications may also be seen in the lesion. • The bony walls of the involved sinus can become sclerotic and thickened, or expanded with areas of thinning and erosion.

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• MRI of fungal balls shows a hypointense mass on T1 and T2 weighted imaging. 1. These findings are due to the absence of free water and the presence of paramagnetic metals such as iron, magnesium, and manganese. • Inspissated secretion is another condition that can have increased attenuation on CT and very low signal intensity on T1 and T2 imaging. 1. This condition should be included in the differential diagnosis, particularly in hospitalized patients and when the sphenoid sinus is involved.

Management • ESS is the treatment of choice to extract the fungal ball and relieve the obstruction of the sinus. • Recurrence is uncommon. • Antifungal agents are not necessary, as the fungi do not invade the sinonasal mucosa.

Acronyms • AIFS:  acute invasive fungal sinusitis • AFS:  allergic fungal rhinosinusitis • CRS:  chronic rhinosinusitis • ESS:  endoscopic sinus surgery • GFS:  granulomatous fungal sinusitis • CIFS:  chronic invasive fungal sinusitis

Chapter

18 Pediatric Rhinosinusitis Julian A. Vellucci and Sanjay R. Parikh

European Position Paper on Rhinosinusitis . . . . . . 236 and Nasal Polyps (EPOS-2012) Definitions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 236 Acute Rhinosinusitis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 236 Background and Pathogenesis . . . . . . . . . . . . . . . . . . . . . 236 Imaging . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 237 Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 237 Chronic Rhinosinusitis . . . . . . . . . . . . . . . . . . . . . . . . . . . 237 Background and Pathogenesis . . . . . . . . . . . . . . . . . . . . . 237 Imaging . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 238 Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 238 American Academy of Otolaryngology-Head . . . . 238 and Neck Surgery (AAO-HNS):  2014 Clinical Consensus Statement: Pediatric Chronic Sinusitis Summary of Essential Statements Reaching . . . . . . . . . . 238 Consensus Management . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 239

235

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European Position Paper on Rhinosinusitis and Nasal Polyps (EPOS-2012) Definitions Acute Rhinosinusitis (ARS) • Inflammation of the mucosa of the nose and paranasal sinuses • Two or more symptoms of 1. Nasal blockage, obstruction, congestion, discolored nasal discharge, cough • Less than 12 weeks

Recurrent Acute Rhinosinusitis • Several episodes per year with complete resolution between infections

Chronic Rhinosinusitis (CRS) • Inflammation of the mucosa of the nose and paranasal sinuses with • Above inflammation plus two or more of the following: 1. Nasal blockage, obstruction, congestion, nasal discharge – With or without cough – With or without facial pain or pressure 2. Also requires objective signs of infection/inflammation on nasal endoscopy and/or CT scan • Duration longer than 12 weeks • Further subclassifications: 1. CRS with nasal polyposis (CRSwNP) 2. CRS without nasal polyposis (CRSsNP)

Acute Rhinosinusitis Background and Pathogenesis • Viral ARS 1. Less than 10 days 2. Adenovirus, rhinovirus, Influenza A and B, Parainfluenza, coronavirus, RSV • Bacterial ARS 1. Double sickening (worsening) after 5 days or lingering symptoms after 10 days

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2. Other symptoms – Purulent rhinorrhea – Fever – Localized pain – Elevated ESR/CRP

Imaging • Usually not indicated unless presenting complicating symptoms: 1. Periorbital swelling, neurologic issues (severe headaches, seizures, etc), visual deficits, decreased ocular movements 2. CT with contrast is imaging modality of choice; MRI may also be helpful

Treatment • Supportive for viral ARS • Antibiotics for bacterial ARS 1. Amoxicillin/clavulanate as first line 2. Consider:  cephalosporins, fluoroquinilones (if penicillin allergic) • Surgery reserved for ARS with complications: 1. Subperiosteal and orbital abscess 2. Intracranial involvement (ie, meningitis, brain abscess, encephalitis, superior sagittal sinus, cavernous sinus thrombosis) 3. Pott’s puffy tumor

Chronic Rhinosinusitis Background and Pathogenesis • Multiple theories exist: 1. Anatomic 2. Bacteriology 3. Biofilms 4. Adenoid hypertrophy 5. Nasal polyposis 6. Comorbid disease – Allergic rhinitis – Asthma – GERD – Immunodeficiency, cystic fibrosis, primary ciliary dyskinesia

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Imaging • Waters’ view plain films:  not recommended due to low sensitivity and specificity • CT without contrast 1. To evaluate structure, development, and extent of disease 2. To assist with surgical planning

Treatment • Medical 1. Nasal steroid spray 2. Topical nasal saline irrigation 3. Antibiotics – No clear efficacy, antibiotic choice, or duration – 20-day course preferable to 10-day course – Culture-directed for those who have not responded to empiric therapy • Surgical 4. Adenoidectomy – First-line surgery in children sphenoid > maxillary. • Orbital involvement is due to direct extension through congenital bony dehiscences, natural suture lines/foramina, or erosion through the lamina papyracea (which may be dehiscent congenitally or secondary to trauma). • Retrograde thrombophlebitis may also occur through valveless orbital veins. • Temporary or permanent loss of vision may occur due to ischemic, compressive, or inflammatory optic neuropathy. • Orbital complications are associated with a 10% incidence of permanent visual loss. • Common pathogens:  Streptococcus pneumoniae, Haemophilus influenza, Moraxella catarrhalis, Streptococcus pyogenes

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Clinical Presentation • Signs of orbital involvement: 1. Proptosis (protrusion and swelling of the orbit and overlying soft tissues) 2. Chemosis (edema of the conjunctiva), restriction of extraocular movements 3. “Tense globe” increased intraocular pressure (>22 mm Hg, papilledema seen on slit lamp exam) 4. Afferent pupillary defect (compression or irritation of CN II) 5. Vision changes

Chandler Classification of Orbital Complications • Describes natural progression of orbital complications of sinusitis but does not necessarily advance in this order • Class I (Periorbital/Preseptal Cellulitis or Inflammatory Edema):  infection limited to soft tissue anterior to orbital septum (protective barrier); presents with unilateral upper and/or lower eyelid edema, tenderness, erythema, mild proptosis but no signs of orbital involvement (no impairment of extraocular movement or visual acuity); imaging shows fat stranding in preorbital soft tissues without orbital findings; Rx: parenteral antibiotics (consider oral antibiotics with close follow up in mild or early symptoms), generally surgery not indicated • Class II (Orbital/Postseptal Cellulitis):  infection has breached the orbital septum and is within the orbit; may present with signs of orbital involvement with diffuse edema, proptosis, orbital pain; imaging shows soft tissue stranding within the orbital space without fluid collections/abscess formation; Rx: parenteral antibiotics, vision checks, consider surgery for refractory cases • Class III (Subperiosteal Abscess):  abscess formation between orbital bony wall and orbital periosteum (usually between lamina papyracea and medial periorbita); signs of orbital involvement with reduced ocular mobility and visual acuity with chemosis as fluid collection enlarges; Rx: for medial wall and/or abscesses 1 cm) 3. Vision changes (acuity 24 hours 6. No improvement or resolution after 48 to 72 hours of medical therapy 7. Involvement of opposite eye (may suggest worsening of sinonasal infection or cavernous sinus involvement) 8. Superior orbital fissure or orbital apex syndrome requires urgent surgical decompression • Surgical intervention entails draining the subperiosteal/orbital abscess, addressing the involved sinuses, and obtaining intraoperative cultures; approaches can be endoscopic, open, or combined, depending on the location and extent of the fluid collection.

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• Orbital or optic nerve decompression is generally not indicated unless vision changes are present.

Intracranial Complications Epidemiology • Intracranial complications are second to orbital involvement in terms of frequency. • Acute rhinosinusitis is the underlying cause of 10% of intracranial suppuration. • Most commonly occurs in adolescent males • Peak incidence between 15 and 20 years of age • Meningitis is the most common intracranial complication of sinusitis in both adults and children.

Pathophysiology • Most commonly involves frontal sinuses, followed by ethmoids > sphenoid > maxillary • Intracranial involvement is due to direct extension of infection to adjacent bone (osteomyelitis) and erosion into the epidural space; may also travel through olfactory pathway or foramina of Breschet. • Retrograde thrombophlebitis may occur through diploic veins; patients with history of craniotomy or head trauma are at risk if they have anomalous connections between the intracranial and nasal cavities. • Common pathogens:  Staphylococcus aureus, Streptococcus, Haemophilus influenza, Bacteroides, Escherichia coli, Pseudomonas, Proteus, anaerobes (abscesses are often polymicrobial)

Clinical Presentation • All intracranial complications have the potential to cause increased intracranial pressure (ICP) which may present with fever, headache, lethargy, malaise, nausea and vomiting, seizures, cranial nerve palsies, paralysis, hypertension, bradycardia, and mental status changes. • Intradural complications have more serious sequelae than extradural complications. • Meningitis:  most common intracranial complication from rhinosinusitis; presents with headache, fever, irritability, altered

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mental status, photophobia, nuchal rigidity, Kernig sign (pain with leg extension while hip in flexion), Brudzinski sign (neck flexion causes a reflexive and painful leg flexion); contrast CT, MRI to rule out other intracranial pathology, imaging may reveal dural enhancement, lumbar puncture is diagnostic • Epidural Abscess:  extradural abscess formation between skull base and dura mater; symptoms often mild with no neurologic deficits initially, present with headache and low-grade fever with possible forehead swelling, increased ICP develops with progressive enlargement; imaging shows biconvex disk between bone and brain matter that does not cross midline • Subdural Empyema:  intradural abscess formation between dura and arachnoid membrane; presents with symptoms of increased ICP, meningitis (high fever, headache, nuchal rigidity), personality changes or focal neurologic deficits if frontal lobe involved, seizures, can progress rapidly to depressed consciousness, bradycardia, cranial nerve palsies, septic shock; imaging shows crescent-shaped enhancement that may cross midline • Cerebral/Brain Abscess:  abscess formation within brain parenchyma, typically at the junction of gray and white matter; stages include (1) encephalitis (fevers, headache, nuchal rigidity) → (2) latency (gradual abscess formation, usually asymptomatic over course of weeks, lack of focal neurologic signs, lethargy, headache) → (3) abscess expansion (seizures, severe neurologic deficits, increased ICP) → (4) abscess rupture (often fatal) • Cavernous Sinus Thrombosis (see above) and superior sagittal sinus thrombosis

Diagnostic Evaluation • Neurosurgical consultation indicated for serial neurologic exams and monitoring of intracranial pressure • Lumbar puncture indicated for suspected meningitis (shows pleocytosis, elevated protein, and decreased glucose); must be cognizant of risk of brain herniation from increased ICP or mass effect in the context of other intracranial complications • CT of paranasal sinuses/cranial with contrast for rapid diagnosis and assessment of bony abnormalities • MRI brain with contrast to evaluate meningeal inflammation, parenchymal involvement, subdural empyema (hypointense T1, hyperintense T2), and brain abscess (hyperintense rim T1) • MR venogram to assess cavernous sinus thrombosis:  will see thrombosed or dilated superior ophthalmic veins, extraocular muscles enlarged from venous engorgement

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Management • Often requires ICU admission • Aggressive, early broad-spectrum IV antibiotics that cross the blood-brain barrier (ie, third-generation cephalosporin) recommended; may adjust antibiotics after obtaining CSF and/or endoscopy-directed sinus cultures • Antiseizure medication may be started depending on neurosurgery/ neurology recommendations • Use of corticosteroids is controversial; may be useful in the context of cerebral edema. • Traditionally, timely surgical intervention is recommended to address both the intracranial complication and underlying sinus infection; however, no current consensus exists regarding appropriate timing of surgery; some advocate simultaneous neurosurgical and sinus drainage procedures, while others recommend addressing the intracranial complication initially with a neurosurgical procedure, then considering subsequent sinus surgery for source control once patient is medically stable. • In some cases, intracranial abscess collection may be accessed via preexisting breach in skull base. • Very high ICP may require craniectomy to prevent herniation. • Meningitis:  parenteral antibiotics (ie, ceftriaxone and flagyl); if no improvement with medical therapy after 24 to 48 hours, surgical intervention to address underlying sinus disease may be required once patient is stable • Epidural abscess:  parenteral antibiotics, often requires craniotomy for drainage of abscess • Subdural empyema:  parenteral antibiotics, often requires craniotomy for drainage of abscess and simultaneous surgery to address underlying sinus infection • Brain abscess:  parenteral antibiotics, possible concurrent craniotomy and sinus surgery for large, well encapsulated abscess • Cavernous sinus thrombosis:  parenteral antibiotics, anticoagulation controversial, sinus surgery when patient stable

Bone Complications • Chronic inflammation/infection causes bony remodeling over time, leading to osteitis, osteomyelitis, and osteoneogenesis. • Best prevented by restoring normal sinus drainage pathways, mucociliary physiology, mucosal sparing techniques, and removal of all diseased bone

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• Diagnostic evaluation:  endoscopy may show exposed nonhealing bone, CT shows bone remodeling and thickening, technetium bone scan lights up over areas of osteoblastic activity, gallium scan to assess initial inflammation and subsequent monitoring • Management:  surgery for osteitis and osteoneogenesis is frequently challenging and may require the use of image guidance and powered instrumentation (drills, ultrasonic aspirator) to identify and restore normal anatomy; larger sinusotomies (Draf III for frontal sinus, “sphenoid drillout” for sphenoid) may be necessary to remove osteitic bone and optimize long-term surgical outcomes

Frontal Sinus Osteomyelitis with Subperiosteal Abscess (Pott’s Puffy Tumor) • Secondary to osteomyelitis of the anterior table or retrograde thrombophlebitis • Clinical presentation:  headache, “doughy” forehead with edema of overlying skin and pus under periosteum; prolonged infection may lead to sinocutaneous fistula • Management:  long-term culture-directed parenteral antibiotics for 6 to 8 weeks, infectious disease consultation, sinus surgery including a modified endoscopic Lothrop for wide marsupialization, refractory cases may require bone removal via an open approach (bicoronal, osteoplastic flap), should involve aggressive debridement of necrotic bone until bleeding viable bone is reached

Mucoceles • Can occur with prolonged bony and mucosal inflammation or obstruction of particular sinus • Presents with expansile fluid-filled mass that erodes but does not invade bone • More common in patients with ciliary dysfunction (cystic fibrosis, primary ciliary dyskinesia) • Treatment:  endoscopic drainage of affected sinus with marsupialization of mucocele, may require open approach in select cases

Chapter

20 Sinonasal Manifestations of Systemic Diseases Jonathan Yip and Eric Monteiro

Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 254 Granulomatous Diseases . . . . . . . . . . . . . . . . . . . . . . . . . 254 Granulomatosis with Polyangiitis . . . . . . . . . . . . . . . . . . 254 Sarcoidosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 255 Churg-Strauss Syndrome . . . . . . . . . . . . . . . . . . . . . . . . . . 256 Autoimmune/Inflammatory Diseases . . . . . . . . . . . . 257 Sjögren’s Syndrome . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 257 Relapsing Polychondritis . . . . . . . . . . . . . . . . . . . . . . . . . . 258 Neoplastic . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 258 Nasal T Cell Lymphoma . . . . . . . . . . . . . . . . . . . . . . . . . . 258 Infectious Diseases . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 259 Rhinoscleroma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 259 Tuberculosis (TB) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 260 Syphilis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 261 Leprosy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 262 Immunodeficiency Diseases . . . . . . . . . . . . . . . . . . . . . . 262 Acquired Immunodeficiency Syndrome (AIDS) . . . . . . 262 Mucociliary Diseases . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 264 Primary Ciliary Dyskinesia . . . . . . . . . . . . . . . . . . . . . . . . 264 Cystic Fibrosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 264 Vascular Disorders . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 265 Hereditary Hemorrhagic Telangiectasia (HHT) . . . . . . 265 Others . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 266 253

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Idiopathic Disorders . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 267 Langerhans Cell Histiocytosis . . . . . . . . . . . . . . . . . . . . . 267 Rosai-Dorfman Disease . . . . . . . . . . . . . . . . . . . . . . . . . . 268

Introduction • Sinonasal manifestations often present with symptoms of chronic rhinosinusitis that fail to respond to standard treatment modalities. • Generally, systemic diseases produce pathological changes in several ways: 1. Pathophysiology of disease specifically affects tissues in the nose 2. Histological properties of nasal mucosa make it more sensitive to changes induced by pathologic process, leading to a more severe presentation 3. Affects nasal tissues as part of symptom complex (eg, granulomatosis with polyangiitis)

Granulomatous Diseases Granulomatosis with Polyangiitis (previously known as Wegener’s Granulomatosis) • Pathophysiology:  uncertain etiology resulting in idiopathic, possible autoimmune, systemic small-medium vessel vasculitis characterized by necrotizing granulomas with glomerulonephritis 1. Accompanied by development of proteinase 3-specific antineutrophil cytoplasmic autoantibodies (PR3-ANCA) resulting in self-perpetuating inflammation • Types: 1. Type 1:  limited disease with upper airway symptoms 2. Type 2:  upper and lower airway involvement 3. Type 3:  widely disseminated form involving progressive renal involvement and cutaneous lesions • Epidemiology:  Age of diagnosis is typically 20 to 50 years; more common among northern Europeans • Sinonasal Sx:  recurrent and chronic rhinosinusitis (ie, nasal congestion, rhinorrhea, anosmia) unresponsive to treatment, which can progress to nasal pain, tenderness, and serosanginous discharge • Sinonasal Findings:  mucosal cobblestoning, edema, extensive crusting, ulceration

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• Other H&N Findings:  laryngeal ulcerations, subglottic stenosis, CHL from serous otitis media, SNHL, lacrimal obstruction, uveitis, keratitis, gingivitis, gingival hyperplasia • Systemic Findings:  pulmonary (cough, hemoptysis, pulmonary infiltrates), cardiac (endocarditis, myocarditis, myocardial infarction), neurological (mononeuritis multiplex, cranial nerve palsy), focal glomerulonephritis, constitutional symptoms, cutaneous eruptions (palpable purpura, ulcers) • Diagnosis:  serologic findings in conjunction with tissue biopsy from site of active disease provides definitive diagnosis 1. C-ANCA:  directed against PR3 and is most specific (87%) 2. Biopsy:  lung (highest yield), nasal mucosa or renal 3. Other Tests:  CBC, ESR, SPEP, BUN, creatinine, UA, autoimmune panel, smooth muscle and neutrophil cytoplasm antibodies; chest and sinus imaging • Histology:  vasculitis of small and medium vessels with intramural, eccentric, necrotizing granulomas (multinucleated giant cells); coalescence of microabscesses • Treatment: 1. Medical:  induction therapy with systemic corticosteroids, followed by methotrexate (mild disease), cyclophosphamide (severe disease), or rituximab; saline irrigation and lubricants for nasal crusting 2. Surgical:  ESS not recommended in active disease unless to treat complications of rhinosinusitis; reconstructive surgery for nasal deformities

Sarcoidosis • Pathophysiology:  multisystem, immune-mediated disease of unknown etiology that manifests as noncaseating granulomas with accumulation of T cells and IL-2 in involved organs, especially lymphatic system, lungs, liver, spleen and bones; may lead to fibrosis of tissue • Epidemiology:  incidence peaks in individuals aged 20 to 40 years, more common among African American women and Scandinavian individuals • Sinonasal Sx:  nasal congestion, nasal obstruction, rhinorrhea, epistaxis, facial/nasal pain, anosmia • Sinonasal Findings:  infrequent (occur in 1% to 5% of cases, but is a poor prognostic indicator); purple, friable nasal mucosa (on septum and inferior turbinate), mucosal hypertrophy, nasal polyps, yellow submucosal nodules (intramucosal granulomas), septal perforation, oronasal fistulae

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• Other H&N Findings:  cervical lymphadenopathy (25% to 50%, most common H&N presentation), uveoparotid fever (Heerfordt’s disease—uveitis, fever and parotid enlargement), supraglottic/ epiglottic submucosal mass, orbital mass, bilateral granulomatous uveitis • Systemic Findings:  incidental hilar lymphadenopathy, cutaneous lesions (erythema nodosum, lupus pernio, rashes) • Diagnosis:  biopsy often required but can be supported by elevated ACE and serum/urinary calcium levels, CXR findings, and negative stains for fungus and acid-fast bacilli • Histopathology:  no specific features; multiple noncaseating granulomas consisting of tightly packed epitheloid cells surrounded by lymphocytes, fibroblasts, and multinucleated giant cells • Treatment: 1. Medical:  most patients experience spontaneous remission within 2 years without treatment; asymptomatic patients do not require treatment; treat mild symptomatic disease with NSAIDs, severe disease with systemic corticosteroids and immunomodulators (ie, methotrexate, antimalarials, TNFalpha inhibitors) 2. Surgical:  ESS can improve quality of life if granulomatous or polypoid lesions present

Churg-Strauss Syndrome (Eosinophilic Granulomatosis with Polyangiitis) • Pathophysiology:  allergic, granulomatous, small-vessel vasculitis of unknown etiology; characterized by triad of bronchial asthma, eosinophilia, and systemic vasculitis • Stages 1. Prodromal/Allergic Stage:  allergic rhinitis, asthma, nasal polyposis, rhinosinusitis 2. Eosinophilic Infiltrative Stage:  eosinophilic pneumonia or gastroenteritis 3. Vasculitic/Systemic Stage:  systemic vasculitis with granulomatous inflammation • Epidemiology:  mean age of diagnosis is 50 years • Risk Factors:  genetic (HLA-DRB4 positivity) • Sinonasal Sx:  allergic rhinitis (48%), progressive worsening of nasal obstruction and rhinorrhea • Sinonasal Findings:  nasal polyposis, crusting • Systemic Findings:  lung lesions, myocardial infarction, constitutional symptoms

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• Diagnosis:  at least four of six criteria; asthma, eosinophilia >10%, neuropathy, nonfixed pulmonary infiltrates, paranasal sinus abnormalities, extravascular eosinophils on biopsy • Histopathology:  necrotizing vasculitis with extravascular necrotizing granulomas; intense eosinophilia of vessels and perivascular tissue • Treatment: 1. Medical:  corticosteroids, cytotoxic agents (cyclophosphamide) for life-threatening cases; medical therapy for sinonasal inflammation 2. Surgical:  sinus surgery as needed

Autoimmune/ Inflammatory Diseases Sjögren’s Syndrome • Pathophysiology:  systemic autoimmune lymphocytic infiltration of exocrine glands causing hypofunction and destruction; postulated to involve initial, inciting event (ie, viral infection) resulting in aberrant immune response in genetically susceptible individuals • Epidemiology:  more common in middle-aged women (fourth to fifth decade) • Sinonasal Sx:  nasal obstruction and dryness (most common), epistaxis, hyposmia, and recurrent rhinosinusitis • Sinonasal Findings:  dry mucosa, crusting, septal ulceration • Other H&N Findings:  keratoconjunctiva sicca (filamentary keratitis), xerostomia (dental caries, dry mucosa), intermittent bilateral parotid swelling, intraoral fungal overgrowth • Systemic Findings:  renal tubular acidosis, Raynaud’s phenomenon, polyneuropathy • Diagnosis:  at least two of the three objective features 1. Positive serum anti-SSA/Ro and/or anti-SSB/La or (positive rheumatoid factor and antinuclear antibody titer ≥1:320) 2. Minor salivary gland biopsy exhibiting focal lymphocytic sialadenitis with a focus score ≥1/4 mm2 3. Keratoconjunctivitis sicca with ocular staining score ≥3 (assuming no treatment for glaucoma and history of eye surgery in past 5 years) • Treatment:  symptom-driven; artificial lubricants and moisturizers, pilocarpine; medical therapy for sinonasal inflammation; corticosteroids and cyclophosphamide for major organ involvement • Prognosis:  associated with non-Hodgkin’s lymphoma

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Relapsing Polychondritis • Pathophysiology:  autoimmune disease of unknown etiology; characterized by recurrent inflammation of cartilage (ie, hyaline) and tissues containing glycosaminoglycans, with resultant replacement by granulation and fibrosis • Epidemiology:  often presents in fourth to fifth decade • Sinonasal Sx:  rhinorrhea, epistaxis, rhinosinusitis • Sinonasal Findings:  crusting, nasal chondritis, cartilaginous destruction (septal perforation, saddle nose deformity) • Other H&N Findings:  auricular chondritis (lobule-sparing), ocular inflammation, collapse of laryngotracheal cartilage framework, and airway compromise • Systemic Findings:  aortic/mitral valve disease, nonerosive polychondritis, skin lesions (aphthous ulcers, purpura, nodules) • Diagnosis:  at least three of McAdam’s criteria with histological evidence of chondritis in two or more locations 1. Bilateral auricular chondritis 2. Nonerosive seronegative inflammatory polyarthritis 3. Nasal chondritis 4. Ocular inflammation 5. Respiratory tract chondritis 6. Audiovestibular damage • Histopathology:  cartilage loses its basophilia, loss of chondrocytes, mixed inflammatory infiltrate in perichondrum; cartilage eventually replaced by fibrous connective tissue • Treatment:  treat acute phase and flare-ups with systemic corticosteroids; immunosuppressives (ie, cyclophosphamide, azathioprine, methotrexate) can control symptoms and possibly delay disease progression

Neoplastic Nasal T Cell Lymphoma (Angiocentric T Cell Lymphoma, Polymorphic Reticulosis, Lymphomatoid Granulomatosis, Lethal Midline Granuloma) • Pathophysiology:  form of extranodal non-Hodgkin’s lymphoma; originates from natural killer or T cells; almost always associated with EBV (>95%) • Epidemiology:  rare; more common among Asians, commonly presents in sixth decade

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• Risk Factors:  EBV infection, autoimmune disease, immunodeficiency, chemical or drug exposure (ex: phenytoin), prior radiation or chemotherapy • Sinonasal Sx:  nasal obstruction (most common initial symptom) followed by purulent rhinorrhea, epistaxis, nasal swelling • Sinonasal Findings:  pale and friable mucosa, crusting, unilateral mucosal ulceration with extension to the palate, maxillary sinus, and/or upper lip, oronasal fistulae, septal perforation, friable necrotic mass • Other H&N Findings:  orbital involvement • Systemic Findings:  constitutional symptoms (fever, weight loss, malaise), cutaneous lesions (maculopapular rash, ulcerative cutaneous lesions); may also involve pulmonary, renal, GI or CNS regions • Diagnosis:  CT, MRI, biopsy • Histopathology:  atypical cells with irregular nuclei, granular chromatin, small nucleoli, and pale-to-clear cytoplasm; inflammatory cell infiltrate may be present • Treatment:  radiation for early-staged (Stage I/II) disease is an option but may result in high rates of recurrence; chemoradiation yields 5-year survival of 20% to 80%

Infectious Diseases Rhinoscleroma • Pathophysiology:  due to inhalation of pathogen (Klebsiella rhinoscleromatis) leading to chronic granulomas and impaired cellular immunity; believed to initially affect mucosal junctions (transition zones) • Stages: 1. Catarrhal:  atrophic rhinitis, honey-colored nasal discharge, nasal crusting 2. Granulomatosis:  painless granulomatous masses in nose and upper respiratory tract (including glottis and subglottis) 3. Fibrotic:  cicatrization of the lesions leading to extensive scarring and dense fibrotic narrowing of nasal passage • Epidemiology:  endemic to Africa, Central America, Southeast Asia, Central and Eastern Europe • Sinonasal Sx:  progressive nasal obstruction (most common), rhinorrhea, anosmia, epistaxis • Sinonasal Findings:  nasal deformity, honey-colored nasal discharge, nasal and nasopharyngeal granulomas, bony remodeling of turbinates and medial maxillary wall

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• Other H&N Findings:  facial deformity, granulomas of larynx • Diagnosis:  culture (positive in 50% to 60% of cases; K. rhinoscleromatis cultured in MacConkey agar is diagnostic), biopsy, CT (showing homogenous, non-enhancing nasal and upper airway lesions) • Histopathology:  Mikulicz cell (foamy histiocytes with intracellular bacilli that they are unable to digest), Russell bodies (bloated plasma cells with inclusions) • Treatment: 1. Medical:  long-term antibiotics (tetracycline, ciprofloxacin) 2. Surgical:  debridement; laser excision or cryotherapy; reconstructive surgery to address airway stenosis and scarring; tracheostomy

Tuberculosis (TB) • Pathophysiology:  inhalation of acid-fast bacilli (Mycobacterium tuberculosis) • Risk Factors:  immunocompromised (historically 50% of HIV population), health care workers, immigrants, elderly, poor • Sinonasal Sx:  nasal obstruction, epistaxis, purulent nasal discharge, nasal discomfort, epiphora • Sinonasal Findings:  nasal TB is rare but cartilaginous septum is the most common site involved; friable nasal mass, nodular thickening of nasal mucosa with or without ulceration, septal perforation, nasal polyps, crusting, nasal fissure, lupus vulgaris (painful nodular tuberculoid lesion), distortion of nasal tip and vestibule in severe cases • Other H&N Findings:  cervical lymphadenopathy (firm, matted, bilateral, non-tender; commonly in posterior triangle and supraclavicular area), ulcerative oral lesions, diffuse salivary gland enlargement, painless otorrhea with multiple TM perforations, ossicular chain erosion, ulceration and granulation of posterior glottis, conjunctivitis, uveitis • Systemic Findings:  Ghon’s complex (calcified lung lesion associated with draining lymph node), pulmonary findings (cavity formation, calcified nodules, miliary TB) • Diagnosis: 1. Screening:  Mantoux tuberculin skin test with purified protein derivative (PPD); positive if induration ≥5 mm in HIV or significant immunosuppression, recent contact with active TB; ≥10 mm in IV drug users, prisoners, homeless; ≥15 mm in low-risk individuals; CXR; interferon-gamma release assay 2. Diagnostic Tests:  sputum culture (Ziehl-Neelsen acid-fast bacilli stain), chest radiography (CXR, CT), biopsy, blood cultures, nucleic acid amplification tests

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3. Other Tests:  HIV, drug susceptibility testing • Treatment: 1. Conservative:  respiratory isolation until three consecutive negative sputum cultures; contact public health 2. Medical:  initial treatment consists of four-drug regimen (isoniazid, rifampin, pyrazinamide, and either ethambutol or streptomycin), followed by repeat smear and cultures to determine remainder of treatment

Syphilis • Pathophysiology:  caused by spirochete Treponema pallidum; sexually transmitted or from maternal transmission • Stages: 1. Primary:  painless chancre at site of inoculation at 3 weeks after exposure 2. Secondary:  contagious stage (approximately 4 to 10 weeks after primary lesion); characterized by fever, hepatosplenomegaly, genital condyloma lata, maculopapular rash 3. Latent:  asymptomatic phase 4. Tertiary:  rare, may occur many years after initial infection; slowly progressive, characterized by gummas (inflammatory granulomas with a center of necrotic tissue); affects CNS (neurosyphilis) and cardiovascular system • Congenital Syphilis:  often fatal; nasal findings are diffuse mucosal inflammation and saddle-nose deformity; Hutchinson’s Triad (abnormal central incisors, interstitial keratitis, deafness) • Sinonasal Sx:  rhinitis, rhinosinusitis • Sinonasal Findings:  diffuse mucosal inflammation, saddle-nose deformity, solitary gumma, septal perforation, chancre on septum (primary syphilis), vestibular fissures (secondary syphilis) • Other H&N Findings:  generalized cervical lymphadenopathy, laryngitis, vocal fold immobility, oral chancres, granulomatous inflammation of tongue/palate, abrupt profound SNHL, interstitial keratitis, Argyll-Robertson pupil, TM perforation, facial bone and soft tissue necrosis • Diagnosis: 1. Screening:  VDRL (Venereal Disease Research Laboratory), rapid plasma reagin (RPR), ICE Syphilis recombinant antigen test 2. Diagnostic Test:  specific fluorescent treponemal antibodyabsorption (FTA-ABS), darkfield microscopy, lumbar puncture (if CNS involvement suspected, CSF VDRL and FTA-ABS)

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• Histopathology:  dense chronic inflammation with many plasma cells, gummas • Treatment: 1. Report to public health agency 2. Medical:  antibiotics (IM Benzathine penicillin G, doxycycline) 3. Surgery:  treat complications of tertiary syphilis

Leprosy (Hansen’s Disease) • Pathogen:  Mycobacterium leprae • Subtypes: 1. Tuberculoid 2. Lepromatous 3. Borderline Tuberculoid 4. Midborderline 5. Borderline Lepromatous • Sinonasal Sx:  nasal congestion, epistaxis, hyposmia • Sinonasal Findings:  mucosal nodular thickening, ulcers, crusting, cartilaginous destruction (septal perforation, saddle nose deformity), oronasal fistulae, facies leprosa (atrophy of anterior nasal spine and premaxillary alveolar process), atrophic rhinitis • Other H&N Findings:  laryngeal infiltration leading to airway obstruction, orbital findings (glaucoma, blindness) • Systemic Findings:  CNS paralysis, peripheral paresthesias, hypopigmented skin lesions • Diagnosis:  skin biopsy and/or nasal smears to assess for acid-fast bacilli using Fite’s stain; biopsy full dermal thickness from edge of active lesion • Histopathology:  numerous acid-fast bacilli with fat-laden macrophages (lepromatous); few acid-fast bacilli with granulomatous changes (tuberculoid) • Treatment:  multidrug antibiotic therapy (dapsone, rifampicin, clofazimine), saline irrigations and lubricants for sinonasal disease

Immunodeficiency Diseases Acquired Immunodeficiency Syndrome (AIDS) • Pathogen:  Human Immunodeficiency Virus (HIV) • Pathophysiology:  lentivirus (subtype of retrovirus) attaches to CD4+ marker of T-Helper cells and other immune cells; reverse transcriptase is used to create viral DNA which integrates into the host’s DNA

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1. Increased risk of rhinosinusitis secondary to immunodeficiency, altered mucociliary clearance, and atopy • Stages:  WHO clinical stages of HIV 1. 1:  asymptomatic (CD4+ >500) 2. 2:  mild (CD4+ 350–499) 3. 3:  advanced (CD4+ 200–349) 4. 4:  severe (AIDS) (CD4+ 60 mEq/L) or genetic testing with one of the following: 1. Typical chronic obstructive pulmonary disease 2. Documented exocrine pancreatic insufficiency 3. Positive family history • Treatment: 1. Conservative:  nasal saline irrigation, mucolytics, allergy avoidance 2. Medical:  intranasal corticosteroids, antibiotics (culture guided, high risk of resistance); can consider topical tobramycin and mupirocin for pseudomonas and MRSA, respectively 3. Surgical:  FESS (failure of medical management, pulmonary exacerbations that correlate with sinonasal exacerbations, declining lung function); literature does not show consistent results in terms of the effect of FESS on pulmonary function – Concerns with surgery—vitamin K deficiency (risk of bleeding), severe lung disease (risk of anesthesia), hypoplastic sinuses

Vascular Disorders Hereditary Hemorrhagic Telangiectasia (HHT) (Osler-Weber-Rendu disease) • Pathophysiology:  autosomal dominant genetic disorder characterized by altered angiogenesis, abnormal endothelial-mural architecture, and weakened vessels, which result in telangiectasias, arteriovenous malformations, increase tendency for bleeding

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• Subtypes:  80% of cases are HHT1 and HHT2 1. HHT1:  ENG gene (encoding endoglin protein) 2. HHT2:  ACVRL1 gene (ALK1) 3. HHT3:  Unknown gene 4. HHT4:  Unknown gene 5. JPHT:  MADH4 (SMAD4) • Epidemiology:  incidence rate of 1:5000 to 1:10 000 but highest rates in Dutch Antilles among Afro-Caribbean population (1:200) • Sinonasal Sx:  recurrent, severe epistaxis (90%) • Sinonasal Findings:  friable mucosa, mucosal telangiectasias in sinonasal mucosa, rarely septal perforations • Other H&N Findings:  circumoral/lip telangiectasias, cutaneous telangiectasias, upper digestive tract telangiectasias • Systemic Findings:  anemia, arteriovenous malformations (lung, liver, brain, spine) • Diagnosis:  genetic testing; can also be confirmed/suspected based on following clinical features • Curacao criteria (3/4 definite HHT, 2/4 possible HHT) 1. Spontaneous recurrent epistaxis 2. Multiple telangiectasias in typical locations 3. Visceral AVMs 4. First-degree relative with HHT • Treatment: 1. Conservative:  nasal ointment, nasal saline irrigation, humidification, avoidance of nasal trauma, nasal packing (preferably absorbable nasal packing) 2. Medical:  estrogen containing creams, tranexamic acid, intravenous bevacizumab may reduce severity of epistaxis 3. Surgical:  cautery, laser ablation (ie, YAG), embolization, septodermoplasty (Saunders’ procedure), Young’s procedure

Others • Can manifest with severe, recurrent epistaxis and/or upper airway bleeding • Acquired coagulopathies:  secondary to disseminated intravascular coagulopathies, liver disease, vitamin K deficiency • Von Willebrand disease (vWD):  autosomal dominant disorder; most common hereditary bleeding disorder • Hemophilia A and B:  X-linked factor VIII and IX deficiencies, respectively

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Idiopathic Disorders Langerhans Cell Histiocytosis • Pathophysiology:  group of idiopathic diseases (thought to either be a reactive or neoplastic process) defined by pathologic behavior of cells involved in phagocytosis and antigen presentation • Subtypes: 1. Eosinophilic granuloma:  localized form; presents in children and young adults; excellent prognosis 2. Hand-Schüller-Christian disease:  chronic multifocal form with peak onset ages between 2 and 10 years; 30% mortality 3. Letterer-Siwe disease:  acute disseminated form; predominantly in children under the age of 2 years; uniformly fatal • Sinonasal Sx:  symptoms related to local effects of mass (ie, nasal obstruction, pain, pistaxis) • Sinonasal Findings:  solitary sinonasal or skull base osseous lytic lesions, septal perforation • Other Findings: 1. Eosinophilic granuloma – Lytic bony lesions in the bony calvaria (can cause otitis media, mastoiditis, middle ear granulation tissue), sphenoid, vertebrae, rib, mandible (loose teeth), femur, iliac crest, scapula 2. Hand-Schüller-Christian disease – Triad of exophthalmos, lytic bone lesion (often in the skull) and diabetes insipidus (pituitary stalk involvement)—seen in 25% – Lesions can involve the liver, spleen, lymph nodes, and pulmonary system – Mucocutaneous ulcerative plaques (oral, genitalia, etc) 3. Letterer-Siwe disease – Presents with signs of systemic infection or malignancy – Proptosis, generalized skin eruption, anemia, hepatosplenomegaly – Bony involvement less common except for mastoiditis • Diagnosis:  biopsy of affected tissue; further workup with bloodwork, imaging as necessary • Histopathology:  identification of pathologic Langerhans cells (nondendritic); Birbeck granules (racquet-appearing intracytoplasmic membranous body)

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• Treatment: 1. Conservative – Expectant management may be appropriate in eosinophilic granulomas (typically a benign course with occasional regression of lesions) – Spontaneous remission may also occur in Hand-SchüllerChristian disease 2. Medical – Local radiation – Low dose chemotherapy to control symptoms in HandSchüller-Christian disease – Additional options in Letterer-Siwe include combinations of steroids and cytotoxic medications, bone marrow or stem cell transplantation 3. Surgical:  curettage of bony lesions in eosinophilic granuloma

Rosai-Dorfman Disease (Sinus Histiocytosis with Massive Lymphadenopathy) • Pathophysiology:  unknown etiology caused by accumulation of histiocytes in nodal and extra nodal tissues (most common sites include the sinuses, skin, and upper respiratory tract) 1. Thought to represent an abnormal histiocyte response to a precipitating cause such as viral or bacterial infection, genetics or environmental triggers • Epidemiology:  most commonly presents in first to second decades • Sinonasal Sx:  rhinosinusitis (including nasal obstruction), rhinitis, epistaxis • Sinonasal Findings:  sinonasal involvement in 16%—sinonasal mass, coarse, reddish yellow-brown polyps, nasal deformity (saddle nose) • Other H&N Findings:  non-tender cervical adenopathy (87%) • Systemic Findings:  leukocytosis, neutrophilia, raised ESR, anemia, low-grade fever; can involve skin (16%), eyes (11%), bones (11%), CNS (7%), salivary glands (7%), kidney (3%), respiratory tract (3%), liver (1%), other (1%) • Diagnosis:  biopsy of affected tissue; further workup required once diagnosis is made with CT/MRI, bone scan, bloodwork, and urine analysis • Histopathology:  dilated sinuses, increased plasma cells, and significant proliferation of histiocytes; cells stain positive for s100 and CD68

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• Treatment: 1. Conservative:  expectant management—70% to 80% of cases may show spontaneous improvement without treatment; disease is usually self-limited with a good outcome 2. Medical:  steroids and, rarely, chemotherapy or radiotherapy 3. Surgical:  indicated to manage symptoms (sinonasal obstruction, rhinosinusitis, etc)

Section

V Surgical Management of Sinonasal Disease

Chapter

21 In-Office Rhinology Techniques Amber U. Luong and Martin J. Citardi

Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 274 Pre-Procedure Considerations . . . . . . . . . . . . . . . . . . . 274 Patient Selection . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 274 Physician-Specific Considerations . . . . . . . . . . . . . . . . . . 274 Procedure Day Considerations . . . . . . . . . . . . . . . . . . . 275 Minimizing Patient Anxiety . . . . . . . . . . . . . . . . . . . . . . . 275 Good Local Anesthesia Important for Pain Control . . . 275 Be Prepared to Manage Bleeding . . . . . . . . . . . . . . . . . . . 276 Common In-Office Procedures . . . . . . . . . . . . . . . . . . . 276 Nasal Endoscopy with Debridement . . . . . . . . . . . . . . . . 276 Balloon Sinus Dilatation . . . . . . . . . . . . . . . . . . . . . . . . . . 276 Endoscopic Polypectomy . . . . . . . . . . . . . . . . . . . . . . . . . 277 Conventional Endoscopic Sinus Surgery . . . . . . . . . . . . 277 Septoplasty . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 277 Inferior Turbinate Procedures . . . . . . . . . . . . . . . . . . . . . 278 Surgical Navigation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 278

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Introduction • In-office rhinology cases have significantly increased over the last several years, initially driven by interest in balloon sinus dilatation and made possible by technological improvements in localizing the balloons from fluoroscopy to transillumination and navigation. • Economic healthcare pressures and patient preference have further supported the expansion of in-office procedures. • Several rhinologic technologies have evolved and other novel innovations have emerged specifically for in-office utilization.

Pre-Procedure Considerations Patient Selection • Set expectations:  patient needs to understand the goals of the procedure and accept any surgical limitations of in-office procedures. • Prepare patients for possible need for additional procedures in the operating room should there be an unexpected pathology and inability to complete in-office procedure. • Patient should have calm temperament with minimal history of anxiety to tolerate potentially long instrumentation within nasal cavity. • Patient should otherwise be an appropriate candidate for any sinonasal procedure.

Physician-Specific Considerations • Designation of procedure room versus performing procedures in the same exam room helps differentiate in-office procedures from typical exam. • Financial and regulatory considerations may preclude the designation of an office-based procedure room as a facility. • Consider utilizing a workflow similar to that used to prepare patients for the operating room (eg, obtain written consent, obtain any necessary insurance preauthorizations, set a procedure date). • Appropriate instrumentation and procedure room setup that is similar to that of the operating room will help physician comfort with in-office procedures. • Consider ergonomics of the patient as well as the physician when planning the setup of the procedure room. • In-office procedures may require large initial investment for instruments, monitors, and chairs.

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Procedure Day Considerations Minimizing Patient Anxiety • Premedicating patient with a short acting benzodiazepine 1 hour prior to procedure may be helpful. • Consider suggesting or offering relaxation techniques prior to the procedure such as meditation or NuCalm (www.nucalm.com). • Offering headphones for music and/or eye covers may help patients minimize stress associated with the procedure. • Consider narrating the procedure in real time to help patient anticipate the next physician action.

Good Local Anesthesia Important for Pain Control • Local anesthesia can be applied via atomizer, packing, and injection. • Aminoamides and aminoesters are the two classes of clinical local anesthetics. • Lidocaine is the common amide and tetracaine is the common ester used in the ENT clinic. • Allergic reactions to local anesthetics are very rare, but they are more common with ester-type anesthetics (like tetracaine). • Table 21–1 compares the pharmacology of lidocaine and tetracaine. • Burning or stinging may occur at the administration site of local anesthetics. • Central nervous system toxicity from local anesthetics includes an initial CNS stimulatory phase (seizures), followed by CNS depression (respiratory arrest); some patients may not exhibit signs of the CNS stimulatory phase; signs of the CNS stimulatory phase may be incorrectly ascribed to the impact of epinephrine or patient anxiety; clinicians must be wary to this fact. Table 21–1.  Pharmacology Comparing Lidocaine and Tetracaine Drug

Onset

Maximum Dose with epi

Max Dose (70 kg)

Duration

with epi

Lidocaine

Rapid

4.5 mg/kg

7 mg/kg

315 mg 490 mg

Tetracaine

Slow

1.5 mg/kg

2.5 mg/kg

105 mg 175 mg

with epi 120 min

240 min

3 10 hours hours

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• Cardiovascular toxicity from local anesthetics includes cardiac depression (bradycardia, arrhythmia, hypotension, etc; concomitant epinephrine may produce hypertension, tachycardia, and angina). • Inadvertent application of topical anesthetics to the pharynx may suppress the gag reflex. • It is important to apply local anesthesia not only to structures that will be directly instrumented but also structures that may be touched or bumped during the procedure, such as the middle turbinate. • A stepwise approach can be highly effective—first with atomizer followed by topical application with pledget or cotton ball, and then infiltration of mucosa as needed. • An example of this stepwise approach could be to atomize 4% lidocaine and oxymetazoline into the nasal cavity, followed by placement of pledgets treated with 2% tetracaine; leave the pledget in placed for at least 15 minutes; then inject certain sites as needed with 1% lidocaine with 1:100 000 epinephrine.

Be Prepared to Manage Bleeding • Consider having bipolar or monopolar cautery available. • Prepare a kit with various packing options available to manage bleeding. • Most common bleeding associated with in-office procedures can be manage with topical application of oxymetazoline on nasal packing.

Common In-Office Procedures Nasal Endoscopy with Debridement • One of the more common in office procedures performed in the ENT office • Typically performed after sinus surgery with the goal of maximizing effectiveness of surgery via clearing retained secretions and crust, removing residual packing material, and managing adhesions • Mucocilliary clearance is not re-established for 3 to 6 weeks after surgery • Performed during normal post-op clinic visits

Balloon Sinus Dilatation • The technological changes for localizing the balloon has allowed balloon sinus dilatation to expand from the operating room into the office.

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• The low-disease burden for patients typically amenable to balloon sinus dilatation makes balloon sinus dilatation a natural fit as an in-office procedure. • Three available technology platforms for sinus balloons exist: wire-based (flexible), malleable/rail-based, and rigid. 1. Wire-based platform:  Wire is guided through the ostium of the targeted sinus and then the balloon is passed over the wire; considered an atraumatic means of following the natural pathway; localization of the wire is typically determined by transillumination (at the frontal and maxillary sinuses); microsensors for electromagnetic-based navigation may be used to track the path of the wire to the target sinus. 2. Malleable/rail-based platform:  Malleable probe is directed to the targeted sinus and the balloon is passed over the probe; positioning is confirmed through transillumination (at the frontal and maxillary sinuses) or microsensor-based navigation 3. Rigid probe-based platform:  Probe is based directly to the targeted sinus, and positioning may be confirmed through surgical navigation.

Endoscopic Polypectomy • Typically performed with microdebriders • Powered microdebriders can be easily used for in-office procedures • Powered microdebriders have a noticeable footprint for in-office procedures with a handpiece, power generator, and foot pedal. • Pre-made angled blades are available. • Debrider can have option of navigation. • Newer microdebriders aim to reduce noise and to make the handpiece wireless.

Conventional Endoscopic Sinus Surgery • Endoscopic maxillary antrostomy, endoscopic frontal sinusotomy, endoscopic ethmoidectomy, and endoscopic sphenoidotomy may also be performed in the office setting. • Techniques and instrumentation are analogous to procedures performed in the standard operating room. • Both primary and revision sinus surgery may be performed in the office setting.

Septoplasty • Septoplasty may also be performed in the office setting. • Techniques are analogous to those employed in the operating room.

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Inferior Turbinate Procedures • Soft-tissue of inferior turbinate may be ablated via energy devices (coblation, bipolar cautery, monopolar cautery, and various lasers). • Soft-tissue and bone of the inferior turbinate may be removed with conventional microdebrider technology.

Surgical Navigation • EM-based navigation systems are increasingly popular for in-office use. • May be incorporated into balloon sinus dilatation procedures and other techniques. • Systems designed for the OR tend to be too bulky for in-office use; newer system have a smaller footprint optimized for office-based use.

Chapter

22 Surgery of the Septum and Turbinate Craig Miller and Greg E. Davis

Septoplasty . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 280 Procedure . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 280 Preoperative Evaluation . . . . . . . . . . . . . . . . . . . . . . . . . . . 280 Indications . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 280 Techniques . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 281 Challenges . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 282 Complications . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 282 Postoperative Care . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 283 Turbinate Reduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 283 Procedure . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 283 Preoperative Evaluation (same as septoplasty) . . . . . . . . 283 Indications . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 283 Techniques . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 284 Challenges . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 284 Complications . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 284 Postoperative Care . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 284

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Septoplasty Procedure • Correction of deformity by repositioning or resecting twisted cartilage and bone 1. Typically performed under general anesthesia; occasionally local anesthesia with IV sedation 2. Outpatient procedure in otherwise healthy patient

Preoperative Evaluation History • Trauma, previous nasal surgery, airway complaints, allergies • Cyclical obstruction = physiology • Persistent, unilateral obstruction = pathologic • Patient may underappreciate his or her obstruction if it has been lifelong, as they would have no basis for comparison. • Trial of nasal steroids/antihistamines, ±saline irrigation; important to document for some insurance coverage for surgery

Physical • Anterior rhinoscopy to evaluate caudal and anterior deflection, IT hypertrophy, synechiae, perforation, spurs, and mucosal contact points; also to evaluate for allergic or inflammatory signs; repeat before and after vasoconstriction • Nasal endoscopy to evaluate the posterior nasal cavity and nasopharynx for obstructing lesion • Observation during quiet and deep inspiration indicates fixed obstruction; obstruction only during deep inspiration may indicate dynamic collapse of nasal valve → evaluate with Cottle’s maneuver (spread nostrils/external nasal valve with cotton-tipped applicator; improvement suggests valve dysfunction and possible need for valvuloplasty or rhinoplasty). • Imaging optional; consider if concern for nonseptal cause of congestion; may underestimate degree of deviation

Indications • Congestion/obstruction/snoring due to septal deviation or septal spur • Perforation repair • Access to sinuses for endoscopic sinus or skull base surgery • Epistaxis due to septal spur; typically occurs on obstructed side due excessive air dryness

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• Mucosal contact; relief of pain or pressure with application of topical anesthetic and decongestant helpful in predicting successful result

Techniques • Typically performed via an endonasal approach • Incisions 1. Killian:  unilateral L-shaped incision made just posterior to mucocutaneous junction 2. Hemitransfixion:  unilateral incision made at the mucocutaneous junction on the caudal border of the septal cartilage; allows for better access to the caudal septum 3. Full transfixion:  bilateral incision started at the mucocutaneous junction on one side and extended through to the contralateral mucocutaneous junction; allows for access to the caudal septum, columella, and medial crura; can theoretically lead to loss of tip support through disruption of septocolumellar ligament 4. External rhinoplasty/degloving:  used for advanced maneuvers necessary to address nasal dorsum and/or caudal septum • Endoscopic-assisted septoplasty 1. Advantages:  magnification of field, improved access and visualization of posterior nasal cavity, ability for limited dissection in cases of isolated spur 2. Limitations:  Unable to address severe deviations of anterior and caudal septum • Surgical steps 1. Decongest, then inject local anesthesia into subperichondrial plane bilaterally 2. Make incisions down to cartilage, identify subperichondrial, avascular plane 3. Elevate mucoperichondrial flaps using broad sweeping movements 4. Disarticulate septal cartilage from bony septum at the bonycartilaginous junction 5. Make a dorsal cut through the cartilage, parallel to the nasal dorsum, leaving at least 1 cm for structural support 6. Resect bony and cartilaginous deviation as necessary 7. Gently pulverize cartilage segment for reimplantation 8. Replace excised cartilage into mucoperichondrial pocket, taking care not to cause further obstruction 9. Repair rents primarily using absorbable suture 10. Close approach mucosal incision first (typically with 4-0 plain gut suture) to prevent shortening of the mucoperichondrial flap

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11. Close septal layers by performing a quilting stitch with absorbable suture (such as 4-0 caprosyn) 12. Can also close septal layers with septal stapler • Splints 1. Splints placed to aid in maintaining septal position during healing and prevent fluid/hematoma accumulation; options include Doyle splints or other nonabsorbable packing; antibiotic prophylaxis while splints in place; typically removed after 3 to 7 days, although longer if septum particularly unstable or concern for septal perforation

Challenges • Caudal septal deformity:  consider open external approach (septorhinoplasty) • Pre-existing perforation:  local flaps, various autologous and biocompatible grafts • Revision septoplasty:  review of previous operative record, may or not have cartilage present, higher risk for perforation, scar tissue may require 15 blade to incise through as natural tissue planes may no longer be present • Widened maxillary crest:  may require osteotomies to remove obstructive bone • Actively smoking patients:  risk of poor wound healing and persistent symptoms with no increase in postoperative complication rate was reported by Yazici et al.

Complications • Persistent congestion • Persistent deformity • Saddle nose:  10 mm “l strut” (dorsal and caudal) to remain after septal resection to maintain stability • Perforation:  unilateral or non-apposing perforations likely inconsequential; if apposing rents occur, persistent perforations may be prevented by placement of cartilage graft or primary closure • Tip ptosis:  may occur with caudal septal dissection through disruption of septocolumellar ligament • Skull base injury:  CSF leak, most commonly through rocking of the ethmoid plate with aggressive dissection leading to microfractures of the cribiform plate • Olfactory loss:  scarring or direct damage to olfactory process • Hemorrhage:  uncommon in septoplasty alone; use of bioabsorbable hemostatic agents if a concern

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Postoperative Care • Splints remain for 3 to 7 days or longer if concern for particularly unstable septum; antibiotic prophylaxis while splints in place (ie, gram-positive coverage to help prevent toxic shock syndrome) • Irrigation/nasal sprays to maintain splint patency • Ointment to incision • Follow up typically in 3 to 7 days for splint removal to ensure appropriate healing and to lyse any early synechiae that may have formed

Turbinate Reduction Procedure • Surgical technique to reduce size of inferior turbinates in order to improve air passage/decrease resistance through the nasal cavity 1. IT occupy a large portion of the nasal airway and can account for up to two-thirds of total airway resistance. 2. Technique usually done under general or local anesthesia either awake or with IV sedation. 3. Pathology may be unilateral (ie, compensatory hypertrophy with septal deviation) or bilateral. 4. Turbinate reduction may be performed with septoplasty.

Preoperative Evaluation (same as septoplasty) • Consider oxymetazoline challenge (evaluation of degree of turbinate obstruction and subjective improvement before and after decongestant administration)

Indications • Congestion/nasal obstruction/snoring • Mucosal contact • Access for ESS

Techniques • Out-fracturing:  involves lateralizing the inferior turbinate using a blunt instrument (Boies elevator or knife handle) • Transmucosal:  coblation/cautery—uses coagulation current to induce necrosis and subsequent tissue fibrosis/reduction; current may be applied to surface or beneath surface (submucous

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diathermy) to minimize mucosal damage; laser cautery—creates tissue injury and subsequent fibrosis; can use either Nd:YAG or CO2 laser; cryotherapy—induces necrosis by freezing and thawing of cells); radiofrequency ablation—uses radiofrequency heating delivered by means of a probe that is inserted directly into the inferior turbinate • Submucosal removal of soft tissue:  incision made typically over head of inferior turbinate, dissection carried down to turbinate bone; options for removal of submucosa with microdebrider or using cautery; may also fracture turbinate bone and remove en bloc taking care to preserve mucosal lining (rents may lead to synechiae) • Partial turbinectomy:  selective trimming of the IT either with sharp instrumentation or microdebrider, studies show improvement or resolution of nasal obstruction • Total turbinectomy:  effective in decreasing nasal resistance and improving nasal obstruction; higher rate of crusting and dryness postoperatively

Challenges • Visualization:  may have improved view with loupes or endoscope

Complications • Synechiae • Hemorrhage:  important for vigilant evaluation of hemostasis at conclusion of case • Dry, crusty nasal passages (“atrophic rhinitis”) • Empty Nose Syndrome • Soft tissue regrowth/persistence of symptoms

Postoperative Care • Saline spray and/or irrigation to prevent early crust formation and help prevent synechiae

Reference • Yazici ZM, Sayin I, Erdim I, Gunes S, Kayhan FT. The effect of tobacco smoking on septoplasty outcomes: a prospective controlled study. Hippokratia. 2015;​19(3):219–224. .

Chapter

23 Endoscopic Sinus Surgery: Concepts, Surgical Indications, and Techniques Javier Ospina and Arif Janjua

General Concepts . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 286 Endoscopic Sinus Surgery . . . . . . . . . . . . . . . . . . . . . . . . . 286 Patient Selection and Preoperative Assessment . . . . . . . 287 Postsurgical Care and Treatment . . . . . . . . . . . . . . . . . . . 288 Surgical Indications . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 288 For Inflammatory Conditions . . . . . . . . . . . . . . . . . . . . . 288 For Sinonasal Neoplasm . . . . . . . . . . . . . . . . . . . . . . . . . . . 289 Other . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 289 Surgical Techniques . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 289 General Concepts . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 289 Originally Described Surgical Techniques . . . . . . . 292 Messerklinger . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 292 Wigand . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 292 Combination . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 292 Surgical Steps . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 293 Uncinectomy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 293 Maxillary Antrostomy . . . . . . . . . . . . . . . . . . . . . . . . . . . . 293 Ethmoidectomy (Anterior and Posterior) . . . . . . . . . . . . 294 Sphenoidotomy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 296 Frontal Sinusotomy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 297

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General Concepts Endoscopic Sinus Surgery • The primary objective of endoscopic sinus surgery (ESS) is to restore healthy sinus function by reestablishing ventilation, promoting sinus drainage, and optimizing mucosal health. The term functional endoscopic sinus surgery (FESS) refers to preservation of the sinonasal mucosal lining during surgery, with the hopes of maintaining/restoring normal nasal functions including mucociliary clearance. • Mucosal preservation is one of the key concepts in modern FESS; this does not imply that all mucosa should be left intact within the nose; as required, removal of internal ethmoid septations along with their mucosa, to create a common ethmoid complex into which the remainder of the sinuses drain into, is appropriate when performing an ethmoidectomy. Any bony walls left intact at the conclusion of the operation should have their mucosa preserved. Preservation of this mucosa limits nasal dysfunction and improves the healing process after surgery. This mucosal sparing prevents osteitis of the exposed bone, crust formation, ostia stenosis, pain, and undesirable scarring. • Although it has been proven that ESS provides significant improvement in overall and disease-specific quality of life (QoL), in many inflammatory conditions, such as chronic rhinosinusitis (CRS), ESS must be undertaken in conjunction with adjunctive medical treatment. Surgery eliminates any obstructions to sinus ventilation and drainage, and allows better postoperative delivery of topical treatments into sinuses to control the ongoing inflammatory condition of the nasal lining. • Surgically created sinus openings should always be connected with the natural ostia. The normal mucociliary clearance pattern in each sinus moves mucus from within the sinus towards the natural ostium; surgeons must aim to allow/restore normal mucus outflow/ drainage and eliminate recirculation. • Visualization in ESS is paramount; surgeons must employ tools/techniques to maintain a well visualized operative field. Furthermore, the use of angle endoscopy (mostly via the use of 30°, 45°, 70° scopes) and an increasing armamentarium of angled instrumentation has allowed improved visualization and manipulation of difficult to reach areas (eg, the frontal recess or laterally within the maxillary sinuses).

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Patient Selection and Preoperative Assessment • Careful selection of patients suitable for ESS is key to achieve desired outcomes. • Surgeons must understand that most patients have a global nasal mucosal inflammatory condition that surgery alone is not going to improve. A significant number of patients need a combination of surgery and ongoing medical treatment. There are, however, a subgroup of patients with focal sinus problem that may be managed with surgery alone (eg, fungal balls, silent sinus syndrome); surgeons should differentiate between these two scenarios. • Accurate additional diagnoses are essential; surgeons must identify patients with immunodeficiencies, cystic fibrosis, other ciliary dysmotility, and/or systemic conditions (such as vasculitis), as they need substantial ancillary treatments for disease control. • Evaluate the patient’s response to previous treatments, particularly response to steroids and previous surgical procedures. Patients with poor response to steroids are likely going to be more challenging to achieve long term symptom improvement than other patients. In the same way, care must be taken in patients who have had previous surgeries that did not improve any of the patient’s symptoms; surgeons contemplating revision surgery must identify the cause of previous surgical failure and identify whether another surgery will solve their patient’s problem. • Nasal obstruction and facial pain/pressure are symptoms that seem most likely to improve with surgery, especially when associated with objective findings of nasal obstruction and/or poor sinus aeration, respectively. • Be cautious to recommend surgery in a patient whose primary complaint is postnasal drainage or an abnormal sense of smell, particularly if other sinonasal symptoms are not present. • Appropriate maximal medical treatment (aka, adequate medical treatment or AMT), is desirable for each patient before considering a surgical intervention. Treatments should be tailored according to individual patient and disease features. • Consider adding septal surgery whenever the surgical access to the sinuses is impaired by a deviated nasal septum. In this setting, septal surgery is not only done to address obstructed nasal breathing; the surgeon must optimize access to the sinus drainage pathways to perform adequate surgical aeration, postoperative surveillance, and debridement. With these additional needs in mind, surgical correction of the nasal septum may be required more often than originally presumed.

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• Preoperative imaging must be carefully evaluated in order to identify possible dangerous areas that may lead to adverse outcomes. It is highly recommended to evaluate the patient’s individual anatomy in coronal, axial, and sagittal image sets. • Patients with unrealistic surgical expectations and symptoms that do not correlate well with endoscopic and CT scan findings may not be good candidates for desired symptom improvement via ESS.

Postsurgical Care and Treatment • Comprehensive postsurgical care—with directed debridement, sinonasal irrigations, and appropriate postoperative medical therapy—is critical to achieve the desired results. • Surgeons should attempt to maintain wide patency of the middle meatal area, avoiding lateralization of the middle turbinate in order to achieve better delivery of topical treatments as well as improved surveillance and postoperative debridements. • The postoperative period is almost as critical to the success of surgery as the surgery itself; optimize the postoperative period by delivering topical treatments and enough debridements to allow postoperative patency of the surgically created sinusotomies. • In the vast majority of patients with CRS, surgery alone is unlikely to completely resolve their symptoms. Patients need to be aware of the need for continuing medical (topical) treatment in order to achieve the best outcomes possible.

Surgical Indications For Inflammatory Conditions • Chronic Rhinosinusitis (with or without nasal polyps), recalcitrant to medical treatment • Fungal Rhinosinusitis (invasive and noninvasive forms):  see Chapter 17 • Recurrent acute rhinosinusitis • Complications of rhinosinusitis (mucoceles, orbital/subperiostial infections, intracranial complications):  see Chapter 19 • Antrochoanal polyp

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For Sinonasal Neoplasm (with or without skull base compromise) • Benign tumors:  see Chapter 25 • Malignant tumors:  see Chapter 26

Other • Silent sinus syndrome:  used to describe an acquired maxillary atelectasis with complete or partial opacification of the sinus; could be associated with enophthalmos and infrequently, diplopia; surgical intervention to restore sinus ventilation, with or without orbital floor reconstruction, is the mainstay treatment for this condition • CSF leak and meningoencephalocele:  see Chapter 37 • Choanal atresia repair • Recurrent epistaxis:  see Chapter 11 • Sinus foreign body removal

Surgical Techniques General Concepts Mucosa Preservation • Regardless of the technique used, the single most important concept that needs to be kept in the surgeon’s mind is mucosal preservation. • Removal of mucosa from any bony surface that is going to remain in place at the conclusion of surgery (eg, lamina papyracea, skull base) should be avoided at every step of the sinus dissection; denuded bone leads to crusting formation, scarring, hyperostosis, pain, and, ultimately, undesirable outcomes in ESS. • Preservation of this lining aids with rapid healing (limits osteitis and crust formation), limits synechiae formation, and minimizes ostial stenosis; furthermore, this native lining is essential to maintaining mucociliary clearance.

Adequate Superficial Exposure to Safely Dissect Deeper Structures • It is highly recommended to avoid working in narrow spaces whenever possible.

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• It is easy to create a “funnel” shape when progressing posteriorly in the ethmoid cavity, increasing the risk of disorientation and complications; surgeons must be aware of the importance of creating enough space superficially within the ethmoids before getting into the most posterior/deep areas. • Often, widening the exposure superficially is avoided to prevent against injury to the orbit and/or skull base; a better strategy for safe dissection that avoids restricted visualization deeper within the ethmoids is early identification of the lamina papyracea +/− skull base.

Sinusotomies Should Include the Natural Ostia • The natural ostium of each sinus should always be included in all sinusotomies since the mucociliary clearance patterns propel mucus toward the natural ostia. • Failure to include natural ostium may lead to recirculation and potentially recalcitrant CRS.

Prevention of Middle Turbinate Lateralization • Surgeons should be particularly interested in minimizing postoperative scarring in all areas that will lead to obstruction of the surgically created sinus drainage pathways and subsequent suboptimal postsurgical results. • Patency of the middle meatus/ostiomeatal complex is key to maintaining adequate long-term ventilation and drainage of the maxillary, ethmoid, and frontal sinuses. • A variety of techniques have been described to prevent middle turbinate lateralization; these include suture techniques, Bolgerization, partial resection of the middle turbinate, and various forms of stents.

Intraoperative Use of Intraoperative Navigation Systems (Image-Guided ESS) • These systems are designed for real-time localization of surgical instruments within the surgical field, based on preoperative thincut CT scans. • They provide assistance in identifying critical (eg, orbit, skull base) structures surrounding the paranasal sinuses, and potentially aid in minimizing complications. • Navigation systems are not a substitute for a thorough understanding of the patient’s anatomy; these systems should not be

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used to undertake a case that the surgeon would not otherwise be comfortable completing. • The American Academy of Otolaryngology–Head and Neck Surgery (AAOHNS) endorses use of these systems to assist the surgeon, in selected cases, to clarify complex anatomy during sinus and skull base surgery. • Indications in which use of computer-aided surgery may be deemed appropriate according to AAOHNS include (1) revision sinus surgery; (2) distorted sinus anatomy of developmental, postoperative, or traumatic origin; (3) extensive sinonasal polyposis; (4) pathology that involves the frontal, posterior ethmoid, and sphenoid sinuses; (5) disease that abuts the skull base, orbit, optic nerve, or ICA; (6) CSF rhinorrhea or conditions in which there is a skull base defect; and (7) benign and malignant sinonasal neoplasms.

Powered Instrumentation • Powered microdebriders are very useful to debulk hypertrophic sinonasal mucosal lining, sinonasal polyps, or tumors. They should be used in such a way to cut the desired mucosa sharply and minimize mucosal stripping. • Microdebrider blades angulated at 15°, 40°, 60°, and 90° are available to address different areas in the nasal cavity/sinuses. • A suction trap attached to the microdebrider may be used to collect tissue for histopathology. • Drills (diamond and cutting burrs) with different angulations have been crucial for bone removal in complex ESS cases and in patients with sinonasal and skull base tumors.

Balloon Sinusotomy • Balloons may be used with the goal of atraumatically dilating the sinus ostia (frontal, maxillary, or sphenoid) to widen the sinus ventilation passageway and restore normal drainage/function. • These balloons can be used alone or in conjunction with other instruments and techniques. • Mild to moderate inflammatory disease is likely most amenable to solo treatment by balloon sinusotomy. • Patients with more severe inflammatory conditions usually require more hypertrophic tissue removal and larger antrostomies performed with conventional ESS techniques. The aim of this is not only to enhance the sinus drainage and relieve sinus obstruction but

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also to allow for saline irrigations, better surveillance/debridements, and the topical delivery of medications—all of which are required to resolve more significant inflammatory sinus disease.

Originally Described Surgical Techniques Messerklinger • Anterior-to-posterior dissection with the following steps 1. Removal of the uncinate process to expose the ethmoidal infundibulum; once the uncinate is removed the anterior face of the ethmoidal bulla is visualized 2. Removal of the ethmoidal bulla, exposure of the frontal sinus outflow tract, and identification of the roof of the ethmoid 3. Dissection of the remaining anterior ethmoid cells (anterior ethmoidectomy) and aeration of the posterior ethmoid cells (posterior ethmoidectomy) by traversing the basal lamella of the middle turbinate 4. Identification and enlargement of the sphenoid ostium (sphenoidotomy) 5. Identification and enlargement (maxillary antrostomy) of the ostium of the maxillary sinus with use of a 30-degree endoscope

Wigand • Posterior-to-anterior approach with the following steps 1. Partial dissection of the low posterior ethmoid cells and removal of the anterior wall of the sphenoid sinus 2. Identification of the skull base at the face of the sphenoid sinus; dissection continued anteriorly through the posterior and anterior ethmoids

Combination • Many surgeons use these techniques in combination when performing ethmoidectomy. Many emphasize early identification of skull base and lamina papyracea in order to avoid complications. An example of a typical combination approach is described below under Surgical Steps.

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Surgical Steps Uncinectomy • The first and one of the most important steps in ESS is identification and complete removal of the uncinate process. • Care must be taken to avoid violation of the lamina papyracea; it may be in close approximation to the superior portion of the uncinated. • The uncinate may be removed in several different ways: front-toback, back-to-front or combination. All forms of uncinectomy tend to follow the subsequent general steps: 1. First step is always visualization of the posterior (free) edge of the uncinate process. 2. A double-ball probe or a backbiter (usually pediatric) can be place behind the posterior free margin of the uncinate and used to probe the maxillary infundibulum (hiatus semilunaris inferioris) and reflect the uncinate process medially. 3. The backbiter is placed between the inferior one-third (horizontal) portion and superior two-thirds (vertical) portions of the uncinate; a backbiter in this position can be used to cut anteriorly, dividing the uncinate into superior and inferior segments. This cut should be continued anteriorly to the maxillary line (the junction of the anterior border of the uncinate and the posterior border of the medial vertical buttress of the maxilla, which houses the nasolacrimal duct). 4. A variety of instruments might be used to remove the vertical/ superior portion of the uncinate at this stage; surgeons may use a Freer elevator, a sickle knife, a microdebrider, angled graspers, or angled true-cutting instruments to complete this step. 5. Sidebiting antral punches may be used to remove the remaining horizontal portion of the uncinate. This may be best accomplished with the use of an angled endoscope (30°, 45°, or 70°), such that the natural maxillary drainage pathway can be simultaneous visualized (see Maxillary Antrostomy below).

Maxillary Antrostomy • Angled visualization is very useful for providing better visualization of the maxillary infundibulum and into the maxillary sinus (30°, 45°, or 70° endoscope). • Early identification of the natural maxillary ostium should always be attempted. The surgeon must avoid blindly penetrating the

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soft medial wall of the maxillary sinus (maxillary fontanelle area) without identification of the natural ostium. • A double-ball probe is commonly used to identify/probe the natural ostium; the probe should be directed laterally and inferiorly in order to follow the orientation of the natural ostium since the ostium opens at a 45° angle in the floor of the infundibulum. Directing the double-ball probe directly laterally, when searching for the natural maxillary drainage pathway, may result in orbital injury. • The existence of an accessory ostium must not be confused with the natural ostium. The surgeon must remember that mucociliary clearance patterns are directed toward the natural maxillary ostium; therefore, natural and accessory ostia should be connected to prevent recirculation. • Depending on the position and size of the natural ostium, it may require expansion in one or several directions:  Through-cutting instruments may be used to expand the antrostomy posteriorly; side-biting antrum punches are useful to expand the antrostomy inferiorly and/or superiorly; backbiters are used for anterior expansion. • Powered instrumentation (microdebrider), potentially with a rotatable angulated blade, may also be useful to enlarge the antrostomy in all directions. • There is no firm evidence about what size a surgical maxillary sinusotomy should be. Most surgeons agree that maxillary antrostomy needs to be tailored according to the patient’s disease.

Ethmoidectomy (Anterior and Posterior) • Dissection of the ethmoid cavity should follow a progression of identifying several landmarks from front-to-back: uncinate process, ethmoid bulla, lamina papyracea, basal lamella of the middle turbinate, superior turbinate and its basal lamella, anterior face of the sphenoid sinus. A complete or “full-house” ethmoidectomy entails visualization within all ethmoid air cells and removal of all internal ethmoid septations. 1. The first step is bulla ethmoidalis resection; when present, a surgeon may identify the retrobullar space by passing a straight curette medial to the bulla and removing the bulla from posterior to anterior, using a straight curette. When there is no retrobullar space, the bulla may be entered inferiorly (and/ or medially), and the bony walls removed with grasper and microdebrider or through-cut of the redundant mucosa.

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2. Once the bulla is completely removed, the basal lamella of the middle turbinate should be identifiable immediately posterior to the bulla. Also, the lamina papyracea, which provides the lateral limit of the ethmoid dissection, should be identified at this stage and used as a landmark for the rest of the ethmoidectomy. 3. Of note, aeration of the agger nasi cell and removal of any suprabullar septations from the skull base is required to perform a complete anterior ethmoidectomy. Surgeons often elect to use angled visualization and instrumentation to complete these steps after the skull base has been identified in the posterior ethmoids. Following posterior ethmoidectomy the skull base can be skeletonized, if desired, from posterior to anterior with an angled endoscope and instruments, often setting the surgeon up for a frontal recess dissection. 4. The next step is identification of the horizontal and oblique (vertical) segments of the basal lamella of the middle turbinate. 5. Penetration into the posterior ethmoids through the vertical segment of the middle turbinate basal lamella can be accomplished with a straight curette; the entrance point should be keep inferior and medial, but not inferior enough to destabilize the remaining horizontal basal lamella of the middle turbinate; straight suction curette or sickle knife could also be utilized. 6. Following safe entry into the posterior ethmoids through the basal lamella of the middle turbinate, appropriate dissection of posterior ethmoid cells can be completed via the middle meatus. A complete ethmoidectomy requires removal of all internal posterior ethmoid septations, such that entire anterior face of the sphenoid sinus has been visualized. The limits of a complete posterior ethmoid dissection are the superior turbinate medially, the basal lamella of superior turbinate inferiorly, the skull base superiorly, the lamina papyracea laterally, and the face of the sphenoid sinus posteriorly. These structures also make up the borders of “Bolger’s box” or “parallelogram” (see Sphenoidotomy below.) 7. The surgeon must be aware of anatomical variants like the sphenoethmoidal cells (aka Onodi cells), in which the optic nerve may be dehiscent on the lateral wall of the ethmoid dissection; and the presence of posterior ethmoid cells pneumatizing infero-medial to the orbit (aka retromaxillary cells), which should be also identified and aerated in a complete ethmoidectomy.

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Sphenoidotomy There are three basic surgical approaches for the sphenoid sinus in ESS: transseptal, transsphenoethmoidal recess, and transethmoidal. • Transseptal approach:  commonly used for pituitary or skull base tumor approaches. Submucosal dissection beneath the mucosa of the septum is followed by identification of the sphenoid rostrum (medially) and both natural sphenoid ostia (superior-lateral to the rostrum); each sphenoid ostium may then be enlarged; additionally, the rostrum may be completely removed to create more medial enlargement of both sphenoid ostia, and/or create a common sphenoidotomy (with concurrent removal of the sphenoid intersinus septum behind the rostrum). • Transsphenoethmoidal approach:  A Freer elevator may be used to gently medialize the middle turbinate and identify the anterior end of the superior turbinate. The sphenoethmoid recess is found medial to the superior turbinate; the natural sphenoid ostium is located medial to the superior turbinate attachment into the anterior face of the sphenoid; additional landmarks may be used to identify the natural sphenoid ostium. Once the superior lip of the choana is seen, a straight suction may be used to walk up the anterior face of the sphenoid; the natural ostium of the sphenoid sinus is found medial to the superior turbinate and approximately 1.0 to 1.5 cm above the choana. When the natural sphenoid sinus ostium is identified, it may be enlarged with through-cutting instruments. A sphenoid mushroom punch or Kerrison Rongeur may be utilized; however, a microdebrider should not be used to enlarge the sphenoid in a lateral direction, in order to avoid potential injury to the critical anatomy on the lateral wall of the sphenoid sinus (eg, carotid artery, optic nerve, cranial nerves, cavernous sinus). Care must be taken if one chooses to enlarge the sphenoidotomy inferiorly, as this commonly results in injury to the septal branch of the sphenopalatine artery and significant bleeding. • Transethmoidal approach:  Sphenoidotomy may be performed after the most posterior ethmoid cell has been opened; identification of the boundaries of Bolger’s parallelogram (Bolger’s box) may be used for safe entrance into the sphenoid sinus when a transethmoidal approach is utilized. The walls of Bolger’s parallelogram are the superior turbinate medially, the basal lamella of superior turbinate inferiorly, the skull base superiorly, and the lamina papyracea laterally. The sphenoid sinus should be entered through the medial and inferior corner of Bolger’s parallelogram to avoid injury to the skull base or orbit.

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• The inferior one-third to one-half of the superior turbinate may be removed in conjunction with a sphenoidotomy. The advantages of partial resection of the superior turbinate include easier visualization of the natural sphenoid ostium within sphenoethmoidal recess and the ability to expand the sphenoidotomy laterally for increased size. This partial resection also provides communication between the surgical sphenoidotomy and the middle meatus for improved postoperative visualization and debridement, with the aim of better postoperative patency. Resection of the inferior half of the superior turbinate does not seem to influence the olfactory function.

Frontal Sinusotomy • The frontal sinus is perhaps the most challenging sinus to address due to a wide range of anatomic variability of the frontal sinus drainage pathway and the dangerous structures surrounding the frontal recess (ie, the skull base and orbit). • Careful preoperative imaging review can decipher difficult versus more simple frontal dissections. Prior to surgery, a surgeon should examine the imaging to (1) identify the anterior-posterior diameter of the frontal recess (contributed by the slope of the bony skull base and the size/shape of the frontal “beak”), (2) identify the number and relationship of the cells within the frontal recess (anterior and posterior to the frontal sinus outflow tract), and (3) identify the location and status of the anterior ethmoidal artery. • The anterior ethmoid artery lies at the skull base at the posterior limit of the frontal recess. The artery may be encased in the bony skull base or may be hanging within a mesentery within the ethmoid cavity, placing it at risk of injury during dissection. • A myriad of techniques have been described to ventilate the frontal sinus surgically; some surgeons advocate manipulation of the anterior buttress of the middle turbinate to allow for 0° endoscopic dissection of the frontal recess others have recommended the use of angled endoscopy and angled instrumentation to allow a better understanding of the in situ frontal recess anatomy and minimize surgical trauma during dissection of the frontal sinus outflow tract. • Of note, a frontal sinusotomy may be performed before ethmoid bulla resection (pre-bulla technique). This has been described to have the advantage of minimizing injury to the anterior ethmoidal artery and/or posterior table of the frontal sinus/ skull base; however, many surgeons find this approach restricts instrumentation and is therefore more cumbersome; those surgeons would rather complete the frontal sinusotomy after complete

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ethmoidectomy, including further identification of the skull base posteriorly. • Several descriptions of the extent of frontal recess/sinus dissection exist; the most commonly referenced description was initially described by Dr. Wolfgang Draf: 1. Draf type 1:  complete ethmoidectomy including removal of all septations within the frontal recess; the inferior part of the frontal infundibulum and its mucosa not touched 2. Draf type 2a:  resection of the floor of the frontal sinus between the lamina papyracea laterally and the middle turbinate medially 3. Draf type 2b:  resection of the floor of the frontal sinus between the lamina papyracea laterally and the nasal septum medially, anterior to the ventral margin of the olfactory fossa 4. Draf type 3 or “modified Lothrop” procedure:  type 2b is extended bilaterally by resecting a portion of the superior nasal septum, followed by resection of the frontal sinus septum, resulting in resection of the floor of the frontal sinus from lamina papyracea to lamina papyracea • In all types of frontal sinusotomy, complete clearance of the internal ethmoid septations from the frontal recess results in the improved ability to instrument the frontal sinus outflow tract and potentially yields improved postoperative surveillance, debridements, and long-term patency. When the entirety of the ethmoid septations is removed from the frontal recess, the frontal recess dissection is funnel shaped and will have the following boundaries: anteriorly— anterior wall of agger nasi cell and frontal beak; laterally—the orbit; posteriorly—skull base/posterior table of the frontal sinus; anterior ethmoid artery located within, or hanging from, this wall; medially—variable, depending on extent of frontal sinus floor removal. Draf 2a = medial aspect of middle turbinate; Draf 2b = nasal septum; Draf 3 = connection to contralateral frontal sinus airspace. • Frontal sinus outflow tract stenting is desirable when frontal sinusotomy is extremely narrow and/or significant mucosal damage/removal occurs during the dissection. • Alternatives to complete endoscopic frontal sinusotomy may be utilized in situations of extremely abnormal frontal recess anatomy or the necessity to access/instrument very laterally within the frontal sinus. These alternatives include frontal sinus trephination via an eyebrow or direct incision, and/or radical external surgical approaches including frontal osteoplastic flap procedures, with or without obliteration.

Chapter

24 Complications of Endoscopic Sinus Surgery Saba Ghorab and Devyani Lal Overview . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 300 Epidemiology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 300 Risk Factors . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 301 Prevention of Complications . . . . . . . . . . . . . . . . . . . . . 302 Preoperative . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 302 Intraoperative . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 302 Postoperative . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 303 Orbital Complications of Endoscopic . . . . . . . . . . . . . 303 Sinus Surgery Anisocoria . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 303 Lamina Papyracea Injury . . . . . . . . . . . . . . . . . . . . . . . . . . 304 Retrobulbar (Postseptal) Hematoma . . . . . . . . . . . . . . . . 305 Optic Nerve Injury . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 307 Extraocular Muscle Injury . . . . . . . . . . . . . . . . . . . . . . . . . 308 Orbital Emphysema . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 309 Lacrimal Duct Injury . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 309 Neurological Complications of Endoscopic . . . . . . 310 Sinus Surgery Cerebrospinal Fluid (CSF) Leak . . . . . . . . . . . . . . . . . . . . 310 Paresthesia/Hypoesthesia . . . . . . . . . . . . . . . . . . . . . . . . . 312 Olfactory Loss . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 313 Other Complications of Endoscopic . . . . . . . . . . . . . 314 Sinus Surgery Vascular Injury . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 314 Synechiae . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 315 Infection . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 316 299

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Overview Epidemiology Trends • Since its advent in the 1980s, significant improvements have been made in the field of endoscopic sinus surgery (ESS). • An increasing number of endoscopic procedures are being performed annually. • When compared to prior published reports, there is a decrease in the incidence of complications associated with ESS. • Reduction in complication rates attributed to improved: 1. Understanding of endoscopic anatomy 2. Surgical experience with refinement of surgical techniques 3. Intraoperative prevention of complications 4. Perioperative imaging studies:  superior quality and increased accessibility (including use of image guidance) 5. Technological advances:  better design and availability of operative instruments, endoscopes, and hemostatic agents

Incidence • Overall ESS complication rate is low. • The major complication rate is 1% and most commonly caused by: 1. Orbital injury:  0.07% 2. Cerebrospinal fluid (CSF) leak:  0.17% 3. Hemorrhage requiring blood transfusion:  0.76%

Pediatric Population • Use of ESS in the pediatric population is conservative and often reserved for extensive disease. • ESS is usually limited to maxillary and anterior ethmoid sinuses. • Sphenoid and frontal sinus surgery is less commonly performed due to incomplete development of these structures. • The complication rate is comparable to adult population although some differences exist. 1. CSF leak is less common due to infrequent sphenoid, posterior ethmoid, and frontal sinus surgery. 2. Orbital injury is more common due to incomplete maxillary and anterior ethmoid sinus pneumatization.

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Risk Factors Patient Factors • Extensive sinus disease caused by: 1. Pansinusitis 2. Obstructive nasal polyposis 3. Sinus mucocele 4. Fungal rhinosinusitis 5. Tumor • Revision ESS • High-risk anatomic variances/abnormalities (listed in Table 24–1) Table 24–1.  Anatomical Variations Associated with Higher Risk of Complications from ESS Type of Injury

High-Risk Anatomic Features

Skull Base

Low-lying skull base with long lateral lamella of the cribriform plate (Keros type 3) Asymmetric skull base Skull base dehiscence Meningocele/encephalocele, often mistaken for polyps Low posterior ethmoid sinus height (posterior ethmoid to maxillary sinus height ratio is ≤1:2)

Orbit

Optic canal dehiscence Sphenoid sinus hyperpneumatization may cause more prominent and exposed optic nerve Sphenoethmoidal (Onodi) cell and its relation to the optic nerve Infraorbital ethmoid (Haller) cell and its relation to infraorbital nerve Lamina papyracea dehiscence Orbital displacement into sinonasal cavity secondary to hypoplastic ethmoid sinus, hypoplastic maxillary sinus, silent sinus syndrome, prior ESS, or prior facial trauma

Vascular

Low-hanging pedicled AEA or PEA located within bony mesentery in ethmoid sinus Carotid canal dehiscence Sphenoethmoidal (Onodi) cell and its relation to ICA Sphenoid sinus hyperpneumatization may cause a more prominent and exposed ICA

Note.  AEA: anterior ethmoid artery; ESS: endoscopic sinus surgery; ICA: internal carotid artery; PEA: posterior ethmoid artery

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Iatrogenic Factors • Absence of computed tomography (CT) scan in operating room at time of surgery; considered grounds for malpractice • Calibration errors with image guidance • Loss of visualization and poor surgical field • Failure to identify complications in a timely manner

Prevention of Complications Preoperative History and Physical Examination • Appropriate patient selection for surgery after optimal medical management has failed • Evaluation for extent of disease, prior craniofacial trauma, surgical history including previous ESS, ocular history, and bleeding risk

Imaging Studies • Review triplanar (axial, coronal, sagittal) CT images for degree and distribution of disease, sinus development, key surgical landmarks, and high-risk anatomical variations

Counseling • Provide informed consent to all patients undergoing ESS; discuss complication rates of orbital, neurological, and vascular injuries. • Review potential complications of ancillary procedures, which may be performed to gain better endoscopic access to diseased sinonasal structures. 1. Septoplasty:  septal perforation, septal abscess, septal deformities 2. Inferior turbinate resection:  crusting, bleeding, empty nose syndrome

Intraoperative Image Guidance • Indications for image-guided ESS 1. Revision sinus surgery 2. Distorted anatomy 3. Frontal, posterior ethmoid, or sphenoid sinus disease

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4. Disease involving skull base, orbit, optic nerve, or carotid artery 5. Presence of CSF rhinorrhea, encephalocele, or other skull base defects 6. Benign or malignant tumors • Confirm accurate image guidance calibration using known bony anatomic landmarks.

Surgical Technique • Optimal visualization with adequate exposure • Meticulous dissection and tissue handling • Lamina papyracea and skull base are key anatomic landmarks to prevent orbital and neurological injuries, respectively. • Careful use of powered instruments, such as the tissue shaver, with full visualization of the cutting edge away from key skull base and orbital structures • Minimal routine use of nasal packing

Postoperative Observation • If there is concern about orbital or skull base injury, monitor patient in hospital with serial ocular and neurological examinations. • Educate patient, family, and nursing staff about clinical presentation of ESS-related complications.

Postoperative Care • Nasal hygiene (nasal saline spray and irrigation) and in-office endoscopic sinonasal debridement to prevent obstruction from adhesions and to decrease clot/crust burden • Long-term medical treatment of chronic sinusitis +/− nasal polyposis to prevent disease recurrence after ESS

Orbital Complications of Endoscopic Sinus Surgery Anisocoria Etiology • Topical or injectable local anesthesia causes nasociliary ganglion block

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Presentation • Loss of corneal reflex with unequal pupillary sizes • Loss of accommodation • Loss of sensation over tip of nose • No associated conjunctival chemosis, proptosis, or globe firmness

Management • Self-limited; resolves in several hours • Monitor with serial ocular examinations until resolution of symptoms

Lamina Papyracea Injury Etiology • May occur during maxillary antrostomy, ethmoidectomy, and/or frontal sinus surgery

Presentation • Ranges from no symptoms to mild periorbital ecchymosis, orbital emphysema, or retrobulbar hematoma

Prevention • Preoperative review of CT scan for: 1. Dehiscent lamina papyracea from previous surgery, polypoid disease, fungal rhinosinusitis, or sinus mucocele 2. Hypoplastic maxillary sinus or silent sinus syndrome where the uncinate process is lateralized and tightly adherent to the orbit with a thin/absent lamina papyracea • Intermittent intraoperative evaluation for lamina papyracea injury 1. Bulb press test:  simultaneous external eye palpation and endoscopic visualization of lamina papyracea to assess for periorbita and/or orbital fat exposure 2. “Fat float” test:  historically described; if positive, be aware of risk of extraocular muscle injury and intraorbital bleeding as well

Management • Avoid further manipulation of exposed tissues if lamina papyracea is penetrated. • Do not remove any exposed periorbita and/or orbital fat.

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• No aggressive nasal packing 1. Nasal packing may push through a dehiscent lamina papyracea into the orbit and cause increased intraocular pressure. 2. If bleeding occurs, nasal packing prevents drainage of blood with subsequent intraorbital accumulation. • Perform serial ocular examinations postoperatively to monitor for evolution of lid edema, ecchymosis, chemosis, visual changes, pupillary changes, and/or proptosis. • Prevent sudden increase in intraocular pressure (IOP), which occurs with straining, vomiting, coughing, and nose blowing.

Retrobulbar (Postseptal) Hematoma Etiology • Venous (slow) hematoma:  entrance through the lamina papyracea +/− periorbita/orbital fat with injury to orbital veins supplying these structures • Arterial (fast) hematoma:  injury to anterior ethmoid artery adjacent to the lamina papyracea with subsequent vessel retraction into orbit; very rarely occurs with posterior ethmoid artery injury • Blood accumulation into retrobulbar space causes increased IOP in range of 30 to 40 mmHg. • Increased IOP compromises optic nerve perfusion within minutes. • Must differentiate from a periorbital (preseptal) hematoma. 1. Often due to injury of angular vessels from needle injection 2. Presents with more diffuse eyelid edema, darker ecchymosis, and more significant orbital emphysema 3. No conjunctival chemosis, proptosis, pupillary changes, and visual changes 4. Will resolve spontaneously; monitor with serial ocular examinations

Presentation • Eyelid ecchymosis and edema • Conjunctival chemosis, proptosis, globe firmness, pupillary changes including mydriasis and non-reactive pupil with afferent pupillary defect (APD) • Blindness that can occur within:. 1. 60 to 90 minutes after venous injury 2. 15 to 30 minutes after arterial injury • Venous retrobulbar hematoma may not develop for 24 to 48 hours after surgery.

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Intraoperative Monitoring • Drape eyes into operative field and cover with transparent tape to allow for regular inspection and palpation throughout surgery. • Perform intermittent bulb press test (see previous). • Proceed with immediate management if there is sudden proptosis, globe firmness, and/or pupillary changes.

Management Conservative Management • Elevate head of bed • Remove nasal packing • Initiate gentle orbital massage to redistribute intraocular blood and allow for hematoma evacuation through the nose • Measure IOP using a tonometer 1. Normal:  10 to 20 mm Hg 2. Elevated:  30 to 40 mmHg 3. Have a low threshold for investigating borderline reading since general anesthesia decreases IOP, thus masking presence of elevated pressure • If a tonometer is not immediately available in operating room, obtain urgent intraoperative ophthalmology evaluation while proceeding with further management. • Do not delay further management while awaiting IOP measurements and/or ophthalmology consultation; these interventions are not necessary when clinical examination is concerning for an expanding retrobulbar hematoma • If there is persistent proptosis, globe firmness, APD, and/or IOP is greater than 40 mmHg despite conservative measures, proceed with immediate surgical intervention and ancillary medical management

Surgical Management • Perform a lateral canthotomy-cantholysis 1. Lateral canthotomy:  make a 1 to 1.5 cm horizontal incision through the lateral canthus followed by dissection through the canthal soft tissue down to the lateral orbital rim 2. Cantholysis:  make vertical cuts along inner aspect of the lateral orbital rim to transect the superior and inferior limbs of the lateral canthal ligament • If a lateral canthotomy-cantholysis procedure is not sufficient in reducing IOP, proceed with medial orbital decompression via endoscopic and/or external Lynch approach. 1. Following sphenoethmoidectomy, remove lamina papyracea

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CHAPTER 24

2. Incise periorbita in a posterior to anterior direction, parallel to medial rectus muscle. 3. Entrance through the periorbita causes extraconal orbital fat to prolapse into nasal cavity; this is further augmented with a gentle external orbital massage. • Once elevated IOP is reversed, identify and stop bleeding vessel(s) using bipolar cautery, surgical clips, and/or hemostatic agents (unless vessel has retracted into globe, in which case avoid further exploration/manipulation).

Medical Management • Administer intravenous mannitol, corticosteroids, and topical IOPlowering agents (eg, timolol) • Medical management serves as adjunctive treatment and does not replace surgical management. • Medical management alone has not been shown to decrease orbital pressure acutely to a safe level; it is of questionable value in an emergent situation, although often used by clinicians.

Postoperative Management • Monitor with serial ocular examinations. • Prevent sudden increase in IOP, which occurs with straining, vomiting, coughing, and blowing nose.

Optic Nerve Injury Etiology • Unrecognized or poorly managed orbital hematoma and/or orbital emphysema increases IOP, which may lead to vascular compromise and optic nerve ischemia. • Direct injury of optic nerve from surgical instrumentation; higher risk of injury exists with presence of optic canal dehiscence, sphenoethmoidal (Onodi) cell, or sphenoid sinus hyperpneumatization. • Unintentional injection of agents into orbit or its vessels causes vasoconstriction or embolization. 1. Intraoral greater palatine foramen lidocaine/epinephrine injection of internal maxillary artery branches can cause orbital arterial spasms via the pterygopalatine fossa, which freely communicates with the inferior orbital fissure 2. Steroid injection of intranasal polyps may reach orbital vasculature and cause spasms or obstruction if the steroid particle size is large.

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• Cortical blindness may occur secondary to ischemia from major vascular injury with massive hemorrhage or cerebellar herniation from pneumocephalus.

Presentation • Partial visual loss, complete blindness, loss of pupillary reflex

Management • If increased IOP is suspected, proceed with decreasing orbital pressure (see previous.) • There is no treatment for blindness after direct nerve injury. • Blindness from inadvertent injection may be treated with high dose steroids and orbital vasodilators.

Extraocular Muscle Injury Etiology • Direct injury of extraocular muscles and/or their neurovascular structures from lamina papyracea penetration, usually with a powered instrument such as a tissue shaver • Unintentional injection of local anesthesia into orbit with transient extraocular muscle paralysis • Heat conduction from cautery use through a dehiscent/ compromised lamina papyracea • Medial rectus muscle:  most commonly injured, particularly during posterior ethmoid dissection where periorbital fat is minimal and provides little buffer from direct injury • Superior oblique muscle:  injury may occur with high frontal recess dissection

Presentation • Diplopia (double vision) from impaired extraocular muscle movement

Management • Postoperative CT scan and MRI to determine cause and extent of injury to extraocular muscle • Early referral to ophthalmology and oculoplastic surgery for consideration of repair, including release of entrapped muscle and reattachment/transposition of transected muscle

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Orbital Emphysema Etiology • Fracture or perforation of lamina papyracea can lead to trapping of outside air within the orbit. • Further intraorbital air accumulation occurs with bag ventilation, emergence from anesthesia, straining, vomiting, coughing, and blowing nose.

Presentation • Mild ecchymosis, eyelid edema, and crepitus with palpation • Extraorbital emphysema involving the head, neck, and chest possible; does not warrant further management not warranted if no airway compromise

Management • Self-limited with resolution of emphysema within 7 to 10 days • Observation and reassurance • Monitor for development of orbital hematoma if concern for possible vascular injury • Prevent sudden increase in IOP, which occurs with straining, vomiting, coughing, and nose blowing.

Lacrimal Duct Injury Etiology • Nasolacrimal duct injury can occur during uncinectomy and/or maxillary antrostomy if dissection is carried anterior to the anterior maxillary line

Presentation • A common injury, it often heals spontaneously without becoming symptomatic. • If symptomatic, epiphora (excessive tearing) often develops within first two weeks postoperatively.

Prevention • Review pertinent anatomy of lacrimal apparatus and its relationship to sinonasal structures.

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1. Lacrimal sac is medially contiguous with ethmoid air cells in majority (90%) of patients. 2. Nasolacrimal duct drains into inferior meatus within 1 cm of the anterior aspect of middle turbinate. 3. True maxillary ostium is less than 5 mm posterior to nasolacrimal duct. • When performing an uncinectomy and maxillary antrostomy, do not extend anterior to the anterior maxillary line or anterior aspect of the middle turbinate.

Management • Initial observation, since most cases resolve spontaneously • If symptoms persist, consider dacryocystorhinostomy (DCR) +/− tube placement for adequate drainage of lacrimal apparatus.

Neurological Complications of Endoscopic Sinus Surgery Cerebrospinal Fluid (CSF) Leak Etiology • Caused most commonly by penetration of the lateral lamella of cribriform plate during endoscopic ethmoidectomy. • Occurs less commonly with Draf IIb or Draf III frontal sinus surgery, sphenoid sinusotomy, and middle turbinate resection.

Risk Factors (see Table 24–1) • Pre-existing skull base dehiscence • Low-hanging skull base as categorized by the Keros classification system 1. Depth of the olfactory fossa is determined by height of the lateral lamella of the cribriform plate, and classified into Keros type 1 (≤3 mm), type 2 (4 to 7 mm), and type 3 (8 to16 mm). 2. Keros type 3 associated with highest risk of injury to skull base. 3. CT scan with coronal cuts at level of anterior ethmoid air cells is best for evaluation of olfactory fossa depth. • Low posterior ethmoid sinus height; CT scan demonstrates posterior ethmoid sinus height to maxillary sinus height ratio of ≤1:2 .

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• Aggressive middle turbinate resection at its vertical attachment to skull base (located at junction of cribriform plate and lateral lamella) • Blind instrumentation of an unknown sinonasal structure

Acute Intraoperative CSF Leak Presentation • Endoscopic evaluation of skull base shows: 1. “Wash-out” sign with a flush of clear fluid 2. Sudden/increased bleeding where bleeding was previously minimal

Immediate Repair • Surgical repair depends on size of the defect and CSF flow rate. • Small defect and/or low-flow leak 1. Overlay mucosal graft is generally sufficient 2. Mucosal graft options include septal mucosa, nasal floor mucosa, temporalis fascia, or fascia lata • Large defect and/or high-flow leak 1. Multi-layered closure with underlay and overlay grafts may be necessary 2. Underlay grafts:  fascia lata, fat, or xenograft 3. Overlay grafts:  mucosal graft or nasoseptal flap 4. Conchal bone and septal cartilage grafts have previously been described but supplanted by nasoseptal flaps 5. Lumbar drain for CSF diversion may be considered • Repair is held in place using a combination of biological glue, absorbable gelatin sponge, and/or absorbable/non-absorbable packing.

Postoperative Management • hospital admission for observation and management • Obtain neurosurgery consultation if lumbar drain is present. • Prevent increased intracranial pressure by: 1. Strict bed rest for 1 to 2 days 2. No nose blowing, straining, or bending over at waist 3. Do not use incentive spirometer, drinking straw, or nasal cannula 4. If supplemental oxygen is needed, use humidified oxygen via face mask/tent 5. Scheduled stool softener and antiemetic medications

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• Antibiotic therapy 1. Perioperative antibiotic therapy is generally recommended. 2. Prophylactic antibiotics are indicated for prevention of toxic shock syndrome while nasal packing is in place. 3. Prophylactic antibiotics for reducing meningitis risk are controversial. • Obtain CT scan for acute mental status changes and/or severe headache to rule out pneumocephalus and intracranial hemorrhage.

Delayed Postoperative CSF Fistula Presentation • Usually manifests within 24 to 48 hours after surgery. • Unilateral clear rhinorrhea +/− positive “halo” sign • Severe headache; usually caused by pneumocephalus from a CSF leak • Mental status and/or visual changes • Nausea/vomiting not related to anesthesia or narcotics

Diagnosis • Nasal discharge positive for beta-2 transferrin test; often this assay is not performed in the hospital and needs to be sent to an outside facility, with results not available for several days. • CT cisternogram with intrathecal contrast is now being replaced with high resolution CT scan. • Intrathecal fluorescein dye injection used for identification and localization of occult fistula. • Surgical re-exploration indicated if clinical suspicion is high.

Management • Urgent surgical exploration and repair performed similarly to an acute intraoperative CSF leak (see previous) with comparable results • Work-up for meningitis with lumbar puncture as needed • Postoperative care same as for acute intraoperative CSF leak (see previous)

Paresthesia/Hypoesthesia Etiology • Infraorbital nerve injury occurs most commonly with endoscopic resection of an infraorbital (Haller) cell or polyps involving the maxillary roof.

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• Frontal trephination performed in conjunction with ESS may injure the supraorbital or supratrochlear neurovascular bundles.

Presentation • Tingling, diminished sensation, or complete numbness in distribution of the affected nerve

Management • Observation; spontaneous resolution in most patients in 3 to 6 months after surgery • If nerve is fully transected, no treatment is available, and full recovery will not occur.

Olfactory Loss Etiology • May be present preoperatively secondary to extensive sinonasal disease or postoperatively from disease recurrence; thus, it is not a complication of ESS but rather a sequela of patient’s pre-existing condition. • Iatrogenic causes include: 1. Direct injury of olfactory fibers through the cribiform plate 2. Loss of olfactory epithelium with large septectomy and middle/ superior turbinate resection 3. Synechiae resulting in middle turbinate medialization

Presentation • Partial (hyposmia) or complete (anosmia) loss of smell • Change in perception of smell (dysosmia)

Management • Observation; olfactory loss may be temporary with improvement over time • Revision ESS for extensive synechiae or recurrent disease

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Other Complications of Endoscopic Sinus Surgery Vascular Injury Etiology • Most common intraoperative and postoperative complication of ESS • Most bleeding from venules and arterioles supplying the nose and sinuses • Brisk bleeding likely from sphenopalatine artery or anterior ethmoid artery • Internal carotid artery bleeding is extremely rare; it usually occurs during sphenoid sinus surgery with injury to a dehiscent carotid canal (present in 20% of patients) or resection of sphenoethmoidal (Onodi) cell. • Meningeal injury may cause a subarachnoid hemorrhage.

Presentation • Ranges from minor bleeding from small vessel injury to massive bleeding from internal carotid artery injury • Subarachnoid hemorrhage may not be evident until general anesthesia is reversed and patient is fully awakened. • Hemorrhage may occur 1. Day of surgery:  20% 2. Within one month:  60% 3. Within two months:  20%

Prevention Preoperative • Cessation of medications that increase risk of bleeding at least 10 days before surgery including anticoagulants, anti-platelet agents, NSAID products and supplements such as gingko biloba, ginseng, garlic, and vitamin E • Hematological work-up for history suggestive of coagulopathy • Preoperative prednisone (7- to 10-day course) to decrease inflammation associated with severe chronic sinusitis +/− polyposis

Intraoperative • Total intravenous anesthesia optimal if available • Blood pressure control with systolic pressure below 100 mmHg

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• Head of bed elevation (15 degrees) • Vasoconstriction through topical/injectable agents • Warm saline irrigation to maintain platelet function • Prophylactic cautery of the horizontal basal lamella of the middle turbinate and posterior septal branch of the sphenopalatine artery, which are the most common sites of postoperative hemorrhage

Management • Abort surgery if bleeding is significant • Cautery:  monopolar and/or bipolar • Nasal packing with absorbent dressing, petroleum jelly impregnated gauze, or Foley catheter • Sphenopalatine artery ligation • If bleeding persists despite above measures, clinician can proceed with angiography and embolization by interventional radiology. • Fluid resuscitation and blood transfusion for massive blood loss • Iron replacement therapy for anemia secondary to blood loss

Synechiae Etiology • Most common postoperative complication/sequela of ESS • Middle turbinate lateralization is the most common cause of synechiae formation and can lead to iatrogenic obstruction of the ethmoid and frontal sinuses. • Risk of middle turbinate lateralization increases with partial resection of diseased/degenerated turbinate, reduction of concha bullosa or polyposis, excessive manipulation with outfracturing, and/or apposing mucosal abrasions.

Presentation • Sinonasal obstruction • Recurrent rhinosinusitis warranting further medical treatment and/ or revision surgery

Prevention • Meticulous tissue handling • Prevention of middle turbinate lateralization through the following techniques. 1. Septoplasty if septal deviation is pushing turbinate laterally

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2. Suture medialization of middle turbinate to septum 3. Placement of middle meatal spacers using packing/stent • Ensure sinus ostia patency with routine in-office endoscopic sinonasal debridement beginning two weeks after surgery • Diligent nasal hygiene using nasal saline spray and irrigation

Management • If symptomatic, clinician may perform in-office lysis of adhesions for minor synechiae or revision ESS for extensive synechiae.

Infection Etiology • Recurrent/persistent rhinosinusitis:  inadequate or improper resection of diseased sinonasal tissue 1. Example:  incomplete uncinate process removal and/or missed true maxillary sinus ostium leads to formation of a surgical ostium that causes mucus recirculation • Meningitis and/or brain abscess:  intracranial penetration and seeding of sinonasal flora • Toxic shock syndrome:  systemic Staphylococcal infection caused by nasal packing or retained clot/crust up to one month postoperatively • Myospherulosis:  foreign body reaction to ointment on nasal packing

Management • Antibiotic therapy for an acute infection • Medical management of chronic rhinosinusitis including nasal saline irrigation, topical nasal antibiotics/steroids, and oral antibiotics/steroids as needed • Revision ESS if initial surgery does not accurately and completely address all diseased sinuses and patient is unresponsive to maximal medical management

References • Goyal P, Lal D, Hwang PH. Postoperative care in skull base surgery. In: Stamm AS, ed. Transnasal Endoscopic Skull Base and Brain Surgery. New York, NY: Thieme; 2011:75–79.

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• Palmer JN, Kennedy DW. Concepts of endoscopic sinus surgery: causes of failures. In: Flint PE et al, eds. Cummings Otolaryngology– Head and Neck Surgery. 5th ed. Philadelphia, PA: Mosby Elsevier; 2011:759–774. • Patel AB, Hoxworth JM, Lal D. Orbital complications associated with the treatment of chronic rhinosinusitis. Otolaryngol Clin N Am. 2015;48:749–768. • Ramakrishnan VR, Kingdom TT, Nayak JV, Hwang PH, Orlandi RR. Nationwide incidence of major complications in endoscopic sinus surgery. Int Forum Allergy Rhinol. 2012;2:34–39 • Stankiewicz JA, Lal D, Connor M, Welch K. Complications of endoscopic sinus surgery for chronic rhinosinusitis: a 25-year experience. Laryngoscope. 2011;121:2684–2701. • Stankiewicz JA, Welch K. Complications of sinus surgery. In: Johnson JT, Rosen CA, eds. Bailey’s Head and Neck Surgery: Otolaryngology (5th ed.). Baltimore, MD: Lippincott Williams & Wilkins; 2014:638–660.

Chapter

25 Benign Sinonasal Tumors Zhong Zheng, Jay Agarwal, and Anthony G. Del Signore

Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 320 Sinonasal Papilloma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 320 Exophytic Papilloma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 321 Oncocytic Papilloma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 322 Inverted Papilloma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 323 Juvenile Nasopharyngeal Angiofibroma (JNA) . . . . 327 Benign Fibro-Osseous Lesions (BFOL) . . . . . . . . . . . 329 Osteomas . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 329 Fibrous Dysplasia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 330 Ossifying Fibroma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 330 Respiratory Epithelial Adenomatoid . . . . . . . . . . . . . . 330 Hamartoma (REAH) Glomangiopericytoma . . . . . . . . . . . . . . . . . . . . . . . . . . . 331 Hemangioma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 332 Neuroendoctrine Tumors (Schwannoma) . . . . . . . . . 333

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Introduction • Sinonasal cavity is lined with a ciliated pseudostratified columnar epithelium, goblet, and mucus producing cells; special sensory olfactory epithelium consists of a pseudostratified columnar neuroepithelial layer with olfactory receptor neurons, located in the olfactory cleft between middle/superior turbinates and the nasal septum. • Benign sinonasal tumor may be of epithelial (hamartoma, inverted papilloma), mesenchymal, neuroectodermal, vascular (ie, angiofibroma, glomangiopericytoma), or osseous origin (ie, osteoma, fibro-osseus lesions). • Left untreated: may lead to mass effect on important adjacent neurovascular structures due to close proximity; structures include carotid artery, optic nerve, orbit, cavernous sinus, and/or skull base • Sinonasal tumors: rare, fewer than 10% of all head and neck malignancies, with 25% of these benign • Initial presenting symptoms: nasal obstruction, headache, hyposmia/anosmia, epistaxis, and symptoms of sinusitis; the large volume of paranasal sinuses may allow tumor progression and prevent early detection • Anterior rhinoscopy and more commonly nasal endoscopy are crucial in office evaluation. Definitive diagnoses often require histologic analysis with immunochemistry; however, biopsy should be avoided in the office setting if vascular tumors are suspected. • CT scans are important in evaluating bony anatomy and surgical planning. MRI is superior in delineating soft tissue borders (eg, orbital or intracranial extensions) and identifying inspissated secretions. MR angiography can be helpful in evaluation of vascular tumors. • For benign sinonasal lesions, the benefits of surgical resection of mass must be carefully balanced with morbidities of surgical resection; recent advancement in endoscopic technique has decreased associated morbidities • Indications for surgical resection include symptoms of sinus outflow tract obstruction, intracranial/intraorbital extension, impending complications for mass, or suspicion and potential of malignancy.

Sinonasal Papilloma (Table 25–1) • Accounts for 0.5% to 4% of all nasal tumors • Benign epithelial neoplasms arising from Schneiderian epithelium • Subtypes include exophytic, oncocytic, and inverted papilloma.

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Table 25–1.  Sinonasal Papilloma Exophytic Papilloma

Oncocytic Papilloma

Inverting Papilloma

Subtypes

• Fungiform • Septal • Squamous

• Cylindrical • Columnar

• Inverting

Attachment Location

• Nasal cavity • Nasal septum

• Lateral nasal wall • Ethmoid sinus • Maxillary sinus

• Lateral nasal wall • Ethmoid sinus • Maxillary sinus

SSx

• Unilateral nasal congestion • Epistaxis • Sinusitis • Nasal mass • Headache • Anosmia

• Unilateral nasal congestion • Epistaxis • Sinusitis • Nasal mass • Headache • Anosmia

• Unilateral nasal congestion • Epistaxis • Sinusitis • Nasal mass • Headache • Anosmia

Treatment

• Complete surgical resection with negative margins • Radiation reserved for malignancy

• Complete surgical resection with negative margins • Radiation reserved for malignancy • May require drilling bone to remove microfocal disease

• Complete surgical resection with negative margins • Radiation reserved for malignancy • May require drilling bone to remove microfocal disease

Exophytic Papilloma • Pathophysiology: 1. More common in males (8:1), most common in fifth decade 2. Most arise from nasal septum; rarely involves vestibule, turbinates, or any of the meatus 3. Associated with HPV 6 and 11 4. Malignant transformation is rare. • SSx: 1. Unilateral nasal congestion and epistaxis are the most common presenting symptoms followed by nasal drainage, nasal mass, sinusitis, headache, anosmia, proptosis, diplopia, and epiphora.

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• Dx: 1. Usually exophytic, grey-tan, small and unilateral with a narrow stalk on nasal endoscopy 2. Diagnosis is made based on tissue biopsy. 3. Diagnostic approach is the same as for inverted papilloma. • Histopathology: 1. Branching, exophytic proliferation with fibrovascular cores with well-differentiated squamous epithelium lining the core 2. Koilocytosis can be seen in superficial squamous cells. 3. Mitotic figures can be seen in some basal cells. 4. There may be microcysts with mucus seen in the intraepithelial regions 5. Subtypes include fungiform, septal, and squamous. • Management: 1. Treatment is similar to treatment for inverted papilloma; complete resection with negative margins is necessary for long term control. 2. Site of attachment is important to address surgically to prevent recurrence. 3. Removal of microscopic foci is not usually needed for exophytic papillomas. 4. Radiation therapy is not needed unless associated with malignancy.

Oncocytic Papilloma • Rarest of sinonasal papillomas • Equally common in males and females • Malignant transformation possible • Not usually associated with HPV • Pathophysiology: 1. Most commonly involves lateral nasal wall; some involve maxillary or ethmoid sinuses; very rarely affect the nasal septum • SSx: 1. Unilateral nasal congestion and epistaxis are the most common presenting symptoms followed by nasal drainage, nasal mass, sinusitis, headache, anosmia, proptosis, diplopia, and epiphora. • Dx: 1. Soft, pink, papillary tissue is seen on nasal endoscopy. 2. Diagnosis is made based on tissue biopsy. 3. Diagnostic approach is the same as for inverted papilloma. • Histopathology: 1. Multilayered columnar or oncocytic epithelium lining thin fibrovascular cores

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2. Endophytic segments with columnar or oncocytic epithelium extending into seromucinous glands 3. Cells have oncocytic cytoplasm with round nuclei 4. Intraepithelial mucous cysts can be seen 5. Needs to be distinguished from adenocarcinoma by presence of stratified epithelium, intact basement membrane, and absence of bony destruction and mitotic activity 6. Subtypes include cylindrical and columnar • Management: 1. Treatment is similar to treatment for inverted papilloma; complete resection with negative margins is necessary for longterm control. 2. Site of attachment is important to address surgically to prevent recurrence. 3. Removal of microscopic foci may be necessary for complete resection. 4. Radiation therapy is not needed unless associated with malignancy.

Inverted Papilloma • Also known as epithelial papilloma, papillary sinusitis, Schneiderian papilloma, inverted Schneiderian papilloma, polyp with inverting metaplasia, transitional cell papilloma • Pathophysiology: 1. HPV (serotypes 6, 11, 16, 18 most common) thought to be important co-factor – Serotype 16 and 18 most commonly associated with malignancy 2. Associated with mutations in tumor suppressor gene p53 and chronic inflammation 3. Possible correlation with exposure to diethylnitrosamine, smoke, dust, aerosols, and industrial occupations 4. Associated with malignant degeneration to squamous cell carcinoma in 5% to 10% of cases 5. Maxillary sinus is the most commonly involved paranasal sinus, followed by the ethmoid sinus. 6. IP usually arise from the lateral nasal wall, most commonly the osteomeatal complex, middle turbinate, and inferior turbinate. • Most common epithelial tumor of sinonasal tract • Second most common benign tumor of the sinonasal tract after osteomas • More common in males, occurring most frequently in the 5th to 8th decade of life

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• SSx: 1. Unilateral nasal congestion and epistaxis are the most common presenting symptoms followed by nasal drainage, nasal mass, sinusitis, headache, anosmia, proptosis, diplopia and epiphora. 2. Exophytic and polypoid appearance on nasal endoscopy 3. Attachment site is typically pedicled to a discrete site, meanwhile adjacent tissue/mucosa remains normal without invasion. • Dx: 1. Definitive diagnosis is based on pathology and tissue biopsy. 2. Preoperative imaging help to evaluate extent of disease (involvement of orbit, skull base, internal carotid artery, etc). – CT paranasal sinus with thin slices (0.625 mm): assess bony involvement (bowing/defects/remodeling of local bony structures); focal hyperostosis and osteitic changes suggest tumor attachment site (Figure 25–1) – MRI: best for evaluating soft tissue anatomy and distinguishing IP from inspissated mucus; hypodense to isodense on T1 weighted images, isodense to hyperdense on T2 weighted images (Figure 25–2) • Histopathology: 1. Digitiform proliferation of squamous or ciliated columnar epithelium with mucocytes 2. Inverts into underlying connective tissue 3. Lacks mucus secreting cells and eosinophils 4. Basement membrane usually remains intact • Krause Classification: 1. Stage 1:  confined to nasal cavity 2. Stage 2:  involving ethmoid sinus, medial, and superior maxillary sinus 3. Stage 3:  involving all paranasal sinuses but confined to nose and paranasal sinuses 4. Stage 4:  not confined to nose or paranasal sinuses or having malignancy • Complications:  epistaxis, epiphora, temporary infraorbital hypoesthesia, periorbital ecchymosis, CSF leak (45 years) • Risk factors:  smoking, industrial fumes, hardwoods, nickel, leather tanning agents

Evaluation Clinical Presentation • Asymptomatic in 10% • Often insidious and usually painless; advanced stage at presentation • Epistaxis, nasal obstruction, sinusitis, proptosis, diplopia, CN neuropathies, facial pain/paresthesia • Red flags:  extremes of age, unilateral symptoms, unexplained epistaxis, neurological or visual symptoms

Physical Examination • General:  facial asymmetry • Nasal:  anterior rhinoscopy and endoscopy—discharge, friable mass • Ophthalmological:  proptosis, chemosis, restricted extra-ocular movements • Neurological:  V2, V3 hypothesia • Oral cavity:  palatal involvement, loose dentition • Metastasis:  cervical ≈5% (1% to 6%); distant, rare

Radiologic Evaluation • Used to evaluate stage, extent of spread, endoscopic resectability 1. Plain films:  may be normal or show bony destruction 2. CT:  bony boundaries (lamina papyracea, skull base, palate, nasolacrimal region) 3. MRI with contrast:  soft tissue (skin, orbital fat/muscles), neurovascular (carotid artery and cranial nerves), dura, or cranial involvement 4. PET scan:  locoregional and distant metastases, staging, and surveillance

Malignant Sinonasal Tumors   339

CHAPTER 26

Biopsy • Endoscopic biopsies prior to definitive surgical procedure and radiological evaluation

Classification • WHO classification for malignant sinonasal tumours 1. Malignant epithelial:  (a) SCC, (b) adenoid cystic carcinoma, (c) adenocarcinoma 2. Neuroendocrine:  (a) carcinoid 3. Malignant soft tissue:  (a) sarcomas 4. Borderline and low malignant potential tumors of soft tissue:  (a) hemangiopericytoma 5. Malignant tumors of bone and cartilage:  (a) chondrosarcoma 6. Hematolymphoid:  (a) lymphoma 7. Neuroectodermal:  (a) olfactory neuroblastoma, (b) mucosal melanoma 8. Germ cell 9. Secondary:  (a) renal, (b) lung, (c) breast

Epithelial • Squamous cell carcinoma:  >80%; most common in maxillary sinus (70%) >nasal cavity (20%) >ethmoid (10%); lymph node metastases rare • Adenoid cystic:  10%—tubular, cribriform, and solid; solid = worse prognosis; perineural involvement 40% to 60%; cervical disease rare; high local recurrence rates • Adenocarcinoma:  5%; mainly ethmoid and nasal cavity; risk factors inclue hardwood dust, lacquers, organic compound; local invasion common; cervical and distant metastases uncommon • Esthesioneuroblastoma:  3%—olfactory epithelium origin near cribriform plate, advanced presentation common (Kadish C in >60%); high rate of intracranial extension; Kadish Grading System (Table 26–1) Table 26–1.  Kadish Staging System for Olfactory Neuroblastoma Stage A

Tumor confined to the nasal cavity

Stage B

Tumor in nasal cavity extending to paranasal sinuses

Stage C

Tumor extending to orbit, base of skull, cranial cavity, or with cervical/distant metastasis

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• Sinonasal undifferentiated carcinoma (SNUC):  advanced disease at presentation, highly aggressive lesions, extensive tissue destruction; overall poor prognosis

Non-Epithelial • Hemangiopericytoma:  vascular lesion, origin from pericytes of Zimmerman, normally arising from superior septum/ethmoid • Rhabdomyosarcoma:  embryological, alveolar, and pleomorphic subclasses

TNM Classification See Table 26–2. Table 26–2.  TNM Classification of Carcinomas of the Sinuses T—Primary Tumor TX

Primary tumor cannot be assessed

T0

No evidence of primary tumor

Tis

Carcinoma in situ

Maxillary Sinus T1

Tumor limited to the antral mucosa with no erosion or destruction of bone

T2

Tumor causing bone erosion or destruction, including extension into hard palate and/or middle nasal meatus, except extension to posterior antral wall of maxillary sinus and pterygoid plates

T3

Tumor invades any of the following: bone of posterior wall of maxillary sinus, subcutaneous tissues, floor or medial wall of orbit, pterygoid fossa, ethmoid sinuses

T4a

Tumor invades any of the following: anterior orbital contents, skin of cheek, pterygoid plates, infratemporal fossa, cribriform plate, sphenoid or frontal sinuses

T4b

Tumor invades any of the following: orbital apex, dura, brain, middle cranial fossa, cranial nerves other than maxillary division of trigeminal nerve V2, nasopharynx, clivus

Nasal Cavity and Ethmoid Sinus T1

Tumor restricted to one subsite of nasal cavity or ethmoid sinus, with or without bony invasion

T2

Tumor involves two subsites in a single site or extends to involve an adjacent site within the nasoethmoidal complex, with or without bony invasion

Table 26–2.  continued T3

Tumor extends to invade the medial wall or floor of the orbit, maxillary sinus, palate, or cribriform plate

T4a

Tumor invades any of the following: anterior orbital contents, skin of nose or cheek, minimal extension to anterior cranial fossa, pterygoid plates, sphenoid or frontal sinuses

T4b

Tumor invades any of the following: orbital apex, dura, brain, middle cranial fossa, cranial nerves other than V2, nasopharynx, clivus

N—Regional Lymph Nodes NX

Regional lymph nodes cannot be assessed

N0

No regional lymph node metastasis

N1

Metastasis in a single ipsilateral lymph node, 3 cm or less in greatest dimension

N2

Metastasis as specified in N2a, 2b, 2c below

N2a

Metastasis in a single ipsilateral lymph node, more than 3 cm but not more than 6 cm in greatest dimension

N2b

Metastasis in multiple ipsilateral lymph nodes, none more than 6 cm in greatest dimension

N2c

Metastasis in bilateral or contralateral lymph nodes, none more than 6 cm in greatest dimension

N3

Metastasis in a lymph node more than 6 cm in greatest dimension

Note:  Midline nodes are considered ipsilateral nodes. M—Distant Metastasis MX

Distant metastasis cannot be assessed

M0

No distant metastasis

M1

Distant metastasis

Stage Grouping Stage 0

Tis

N0

M0

Stage I

T1

N0

M0

Stage II

T2

N0

M0

Stage III

T1, T2

N1

M0

T3

N0, N1

M0

T1, T2, T3

N2

M0

T4a

N0, N1, N2

M0

T4b

Any N

M0

Any T

N3

M0

Any T

Any N

M1

Stage IVA Stage IVB Stage IVC

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Management • Individualized according to patient’s extent of disease and co-morbidities. • Open or endoscopic surgical approaches are used for curative resection, or in the case of disseminated disease, local palliation. • Radiotherapy postoperative or sometimes neoadjuvant used for locoregional control:  advanced disease (T3, T4), positive surgical margins, margins close to vital structures (orbit, carotid, CN) recurrent disease, cervical metastases • Radiotherapy alone:  palliation in unresectable cases, poor surgical candidates, or lymphoreticular tumors • Chemotherapy:  adjunctive to radiotherapy/surgical resection or palliation; used neoadjuvantly in some centers of chemosensitive tumors (eg, esthesioneuroblastoma)

Surgical Approaches Endoscopic Versus Open Approaches (Table 26–3) Controversial • Patient selection is crucial, as is surgeon’s experience. • Endoscopic approaches:  reduced morbidity, improved vascular control and better/equivalent quality of life outcomes; contraindicated with involvement of skin, lacrimal system, palate, periorbital fat, carotid artery

Follow-Up Post Treatment • Every 3 months for first 3 years, then 6-monthly for next 3 years, then annually • CT scan, MRI and/or PET scans at frequent intervals; annual CXR to exclude pulmonary metastases

Prognosis • Generally poor • Locoregional control is main determinant. • Cervical and distant metastases can occur late with ONB, ACC, and mucosal melanoma.

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Table 26–3.  Endoscopic vs Open Approaches Endoscopic

Area Accessed

Medial maxillectomy

Pterygopalatine and infratemporal fossa

Transcribiform, Transplanum

Dura, olfactory bulbs, and lamina papyracea

Transpterygoid

Petrous temporal bone, Meckel’s cave, and middle cranial fossa

Transclival

Nasopharynx, clivus, and the odontoid process

Open

Area Accessed

Lateral rhinotomy

Good visualization of maxillary wall, ethmoid sinuses, sphenoid, and medial orbital wall

Midfacial degloving

Limited to lesions of inferior and medial maxillary walls

Transpalatal approach

Involvement of floor of the nose or inferior portion of maxilla

Weber-Ferguson

Additional exposure of maxilla for total maxillectomy

• Overall survival trends at 5 years: 1. SCC, adenocarcinoma, 50% to 60% 2. Adenoid cystic, 40% to 45% 3. Esthesioneuroblastoma, 80% 4. Melanoma, 20%

Chapter

27 External Approach to the Paranasal Sinuses Abdullah AlBader and Roy R. Casiano

Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 346 The Frontal Sinus . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 346 Surgical Procedures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 347 The Maxillary Sinus . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 354 Surgical Procedures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 354 Complications of the External Approach to the . . . . . . 358 Maxillary Sinus

The Ethmoid Sinus . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 359 Surgical Landmarks . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 359 Lynch Incision . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 359 Complications of the External Approach to the . . . . . . 361 Ethmoid Sinus Transantral Approach . . . . . . . . . . . . . . . . . . . . . . . . . . . . 361

The Sphenoid Sinus . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 362 Surgical Landmarks . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 362 Transseptal Approach . . . . . . . . . . . . . . . . . . . . . . . . . . . . 362 Complications of External Approach to . . . . . . . . . . . . . 364 Sphenoid Sinus

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Introduction • External approaches to the paranasal sinuses were first described in early 19th century. • In the endoscopic era, external approaches have limited indications due to poor cosmesis and higher postoperative morbidity as opposed to the minimally invasive endoscopic approach. • General indications for external approaches: 1. Neoplasm with challenging location 2. Complicated infectious and inflammatory disease (intracranial extension of infections, fistula formation, orbital extension, venous thrombosis) 3. Drainage of mucocele and mucopyocele 4. Unsuccessful endoscopic management 5. Facial and paranasal sinus trauma • Every approach is done under general anesthesia, with proper nasal decongestion and local infiltration, with 1% xylocaine and 1:100000 epinephrine solution topically at the operative site, with or without tarsorrhaphy.

The Frontal Sinus • Riedel first described external approach to the frontal sinus in 1898. • Several historical approaches to the frontal sinus have been reported, some of which are the basis for today’s frontal sinus surgeries. • External approaches to the frontal sinus are largely replaced by endoscopic procedures due to a better understanding of the frontal sinus anatomy and physiology and advancement in endoscopic instrumentation. • Indications for external approaches to the frontal sinus include: 1. Unfavorable anatomy that might complicate an endoscopic approach – Multiple intrasinus septations – Scarring – Neo-osteogenesis 2. Laterally and/or superiorly based frontal sinus pathology – Lateral mucocele – Neoplasms – Osteomas 3. Complicated frontal sinusitis with intracranial extension, frontal osteomyelitis (Pott’s puffy tumor/pericranial abscess)

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4. Chronic frontal sinusitis 5. Frontal sinus obliteration – Comminuted and/or displaced posterior table fractures – Cerebrospinal fluid leak – Severe frontal outflow tract obstruction 6. Reduction of frontal sinus fractures 7. Type 4 frontal cells 8. Neoplasm extending to anterior skull base or ethmoid sinus 9. CSF leak repair • Contraindication to open approach to the frontal sinus: 1. Pathology amenable to endoscopic approach 2. Metastatic malignant disease 3. Massive brain involvement 4. Cavernous sinus involvement

Surgical Procedures Surgical Landmarks to the Frontal Sinus • Supraorbital foramen • Superomedial rim of the orbit • Anterior lacrimal crest

Mapping the Frontal Sinus • Intraoperatively, mapping of the frontal sinus can assist in identifying the boundaries of the sinus for safe entry into the sinus cavity. • Mapping of the frontal sinus can be achieved with several methods: 1. Image-guided navigation systems:  used intraoperatively to identify entry points to the frontal sinus 2. Transnasal illumination with endonasal endoscopes 3. Frontal sinus mini-trephination and drilling a pilot hole in the anterior table; endoscopes are introduced through the minitrephination for transillumination and identification of the frontal sinus cavity 4. Removal of anterior frontal sinus table and exposure of the sinus cavity 5. Conventional 6-foot Caldwell standard radiograph templates: – Templates of the frontal sinus are formed and used – Intraoperatively to demarcate the margins the frontal sinus – Anterior table (Figure 27–1)

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Figure 27–1.  Six-foot Caldwell radiograph of paranasal sinuses.

Bicoronal Flap Incision • Done externally behind the hairline • Incision connects the scalp anterosuperior to the auricles bilaterally • Dissection of the flap is carried along the incision caudally to the level of supraorbital rim while avoiding injury to the supraorbital neurovascular bundle • Laterally, the flap is elevated above the temporalis fascia to avoid injury to the frontal branch of the facial nerve (Figure 27–2) • Dissection plane depends on the surgical objective: 1. If a pericranial flap is planned, dissection is carried in the supraperiosteal plane along the areolar tissue layer of the scalp. 2. Pericranium is incised separately to the level of the bone, then elevated caudally to the supraorbital ridges; the supraorbital neurovascular pedicle is dissected and elevated off the supraorbital notch (Figure 27–3). 3. If a pericranial flap is not required, the initial bicoronal incision is deepened to the level of the bone and dissection is carried in a subperiosteal plane.

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Figure 27–2.  External approaches to the frontal sinus.

• Mapping of the frontal sinus cavity is carried out prior to entry to the cavity using the methods mentioned above. • Entry into the frontal sinus cavity is achieved by drilling several openings in the anterior table that are then connected using an oscillating saw; the bone is cut circumferentially around the anterior table in order to completely separate it for access. • If an osteoplastic flap is planned, the anterior table is cut at the superior and lateral margins; an osteotome is then used to divide the intersinus septum, which is then removed using a rongeur and the anterior table is down-fractured at the supraorbital rim, being attached by the pericranium inferiorly. • The frontal sinus pathology is then addressed. • Advantages:  wide exposure of the frontal bone and the superior orbital rim, preservation of supraorbital neurovascular pedicle • Disadvantages:  scar in patients with male pattern baldness, extensive dissection

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Figure 27–3.  Pericranial flap elevation.

Gull-Wing Incision (Full Brow Incision) • Bilateral supraorbital incisions connected medially at the glabella (see Figure 27–2) • Similar dissection planes and entry to the frontal sinus cavity as the bicoronal approach; however, the superior branches of the supraorbital pedicle are sacrificed, resulting in forehead anesthesia above the incision. • Advantages:  limited dissection compared to bicoronal incision • Disadvantages:  forehead anesthesia/paresthesia, poor cosmesis

Forehead Crease Incision • Indicated mainly for patients with male pattern baldness to avoid scalp scars. • Incision is typically placed along a deep mid forehead rhytid (see Figure 27–2). • Dissection and entry to the frontal sinus is similar to bicoronal incision.

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• If a pericranial flap is planned, then the pericranium can be preserved at the incision site and the forehead skin dissected superiorly to the level of the hairline, where the pericranium is incised and reflected as an inferiorly based flap. • This approach can be combined with endoscopy, either endonasally or through the anterior table of the frontal sinus. • Advantages:  avoids scalp scar, preserves supraorbital neurovascular bundle • Disadvantages:  forehead scar, limited exposure

Frontal Sinus Trephination • The simplest open approach to the frontal sinus • Indications:  biopsy of frontal sinus lesions, acute frontal sinusitis with impending complications, adjunct to endoscopic approach • Procedure: 1. Make a stab incision at inferomedial brow around 1 to 1.5 cm from the midline (Figure 27–4) 2. Soft tissue and periosteum dissection is carried to the level of the bone, sparing the supratrochlear neurovascular bundle. 3. A 4-mm burr is used to enter the inferomedial aspect of the sinus. 4. The frontal sinus opening can be enlarged using a bone rongeur

Figure 27–4.  Frontal sinus trephination.

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5. Instrumentation with endoscopes and irrigation using a cannula or biopsy can be taken through the trephine (Figure 27–5). 6. Reconstruction is achieved with approximation of periosteum and soft tissue. • Advantages:  minimally invasive, better cosmesis • Disadvantages:  limited exposure for any useful frontal sinus surgery as a stand-alone procedure, scar formation

Complications of External Approaches to Frontal Sinus • Frontal sinusitis • Frontal osteomyelitis • Wound dehiscence • Frontal sinus mucocele/mucopyocele • CSF leak • Orbital injury • Intracranial hemorrhage/injury • Forehead anesthesia/paresthesia • Scarring • Frontal recess stenosis • Frontalis nerve injury

Frontal Sinus Reconstruction Post External Approach • Primary closure:  mainly in frontal sinus trephination and after Lynch procedure where anterior table defect is minimal and non-deforming • Bone graft:  for bicoronal and forehead approach; anterior table of the frontal sinus is used as a bone graft; position is secured using miniplates or fixation wires • Local flaps:  can be utilized in osteoplastic flaps; the anterior table along with the overlying periosteum is hinged back to the original position; fixation can be achieved with or without fixation wires and plates • Free flaps: microvascular osteocutaneous free flaps indicated for large skull base and frontal bone defects, if previously operated, or if history of irradiation • Soft tissue:  sutured primarily • Nasofrontal duct:  can be stented using silastic/silicone sheets or polyvinyl chloride endotracheal tubes • Medial canthal ligament:  internasal wiring to contralateral medial canthus or frontal plates are placed to stabilize the medial canthal ligament

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Figure 27–5.  Frontal sinus trephination combined with transnasal endoscopy.

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The Maxillary Sinus • The majority of maxillary sinus surgeries are achieved endoscopically. • Indications for external approach to the maxillary sinus: 1. Disease involving the anterior, inferior, or lateral walls of the maxillary sinus 2. Anterolateral extension of disease into the premaxillary soft tissue 3. Access to pterygopalatine and infratemporal fossae 4. Transantral approach to ethmoid and/or sphenoid sinuses 5. Dental foreign body 6. Compartmentalization of the maxillary sinus 7. Previous open procedure with neo-osteogenesis 8. Orbital floor fractures 9. Decompression of the lateral aspect of the inferior orbital wall (lateral to the infraorbital nerve canal) 10. Chronic maxillary sinusitis 11. Excision of antrochoanal polyp unsuccessfully removed endoscopically 12. Closure of oroantral fistula

Surgical Procedures Surgical Landmarks • Gingivo-mucosal junction • Infraorbital foramen • Pyriform aperture

Sublabial Approach • Anterior antrostomy (Caldwell-Luc procedure): 1. Historical procedure with limited indications nowadays 2. Procedure: – Incision is placed in anterior gingivo-labial sulcus from the frenulum to the canine fossa, through the mucosa and periosteum leaving sufficient gingival mucoperichondrium along the gingival side of the incision to facilitate later suturing (Figure 27–6). – The periosteum is elevated off the anterior maxillary wall superiorly to the infraorbital foramen, with preservation of the infraorbital nerve.

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Figure 27–6.  Anterior antrostomy (Caldwell-Luc procedure).

– Anterior maxillary antrostomy is performed using an osteotome or high-speed drill, enlarging with a Kerrison bone rongeur, and then incising the maxillary sinus mucosa. – After addressing the pathology, mucosa can be closed primarily with absorbable sutures:  no bone reconstruction required. 3. Advantages:  avoids scarring, minimally invasive, preservation of facial contour, can be combined with endoscopic approach 4. Disadvantages:  risk of oroantral fistula, limited exposure, premaxillary soft tissue edema • Midface degloving approach 1. First described in 1974 2. Main indication is sinonasal neoplasms 3. Procedure: – Sublabial incision transecting the frenulum and extending between the first molar tooth bilaterally – Bilateral circumferential nasal vestibular incisions in continuity with a complete transfixion incision through the columella – The sublabial and nasal vestibular incisions are connected and the upper lip, the premaxillary soft tissue, and skin-soft tissue envelope of the nose reflected superiorly (Figure 27–7).

Figure 27–7.  Midface degloving.

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– Dissection is carried in a subperiosteal plane along the anterior wall of the maxillary sinus and nasal dorsum. – Anterior antrostomy is performed similar to Caldwell-Luc procedure. – Medial maxillectomy can be added for further exposure. – Absorbable suture closure of nasal and sublabial incisions with nasolacrimal duct marsupialization 4. Advantages:  wide exposure, good cosmesis 5. Disadvantages:  risk of oroantral fistula and nasal vestibular stenosis

Transfacial Approach • Utilizes natural skin crease to place the incision • Lateral rhinotomy incision: 1. Incision is made at the lateral border of the nose, 1 cm from the midline of the nasal dorsum and along the nasal-facial junction; the incision extends from the medial canthus superiorly to the nasal-alar groove inferiorly (Figure 27–8).

Figure 27–8.  Lateral rhinotomy incision.

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2. Soft tissue is incised to the periosteum, and the flap is elevated on either side of the incision in a subperiosteal plane. 3. Osteotomy is done along the nasomaxillary suture line vertically and above the dental roots horizontally; circumferential osteotomies can be performed around the anterior wall of the maxillary sinus. 4. Osteotomy can also be enlarged using a Kerrison bone rongeur; the maxillary sinus cavity is entered and the pathology is addressed. 5. After conclusion of the procedure, bone is placed into position and fixed with miniplates; soft tissue are closed primarily in layers. 6. Advantages:  direct access to the maxillary sinus and nasal cavity, wide exposure 7. Disadvantages:  external scar, risk of premaxillary edema and anesthesia/paresthesia • Weber-Ferguson incision: 1. Allows en bloc resection of the maxilla 2. Indicated for neoplasms involving superior, lateral, or inferior walls of the maxillary sinus and palate 3. Procedure: – Lateral rhinotomy incision is extended inferiorly towards the philtrum and the midline of the lip (Figure 27–9). – Incision extends in the gingivobuccal sulcus laterally to the retromolar area. – Skin flaps are elevated medially and laterally along the incision. – The maxillary sinus cavity is entered using an osteotome or a drill and the opening enlarged with a Kerrison rongeur. – Reconstruction options: a. Primary closure of the defect b. Palatal obturators for palate defects c. Bone grafts fixation with miniplates or fixation wires d. Free microvascular osseous flaps for major reconstruction e. Soft tissue approximated primarily

Complications of the External Approach to the Maxillary Sinus • Hemorrhage (from sphenopalatine artery branches) • Oroantral fistula • Infraorbital paresthesia or anesthesia • Premaxillary edema • Epiphora and orbital injury • External scar

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Figure 27–9.  Weber-Ferguson incision.

The Ethmoid Sinus Surgical Landmarks • Horizontal line through the medial canthus marking the fovea ethmoidalis • Vertical line through the medial canthus marking the lamina papyracea

Lynch Incision • The external frontoethmoidectomy was first performed in 1897 by Jenson in Germany. • Lynch and Howarth described removal of the floor of the frontal sinus and opening the nasofrontal drainage, combined with an ethmoidectomy and middle turbinectomy, in 1920. • The main objective of this approach was to reestablish the frontal sinus drainage into the middle meatus.

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• Indications:  frontoethmoidal mucocele and mucopyocele, orbital complications of sinusitis, biopsy of anterior skull base neoplasms, excision of orbital tumors, and ligation of anterior and posterior ethmoid arteries • Procedure: 1. The medial canthal curvilinear incision is placed half way between the medial canthus and the nasal dorsum along the lateral nasal wall, extended superiorly to the inferior border of medial end of the brow; the tip of a thumb can be use to outline the incision (Figure 27–10). 2. The incision is carried deep to the periosteum, which is elevated on both sides of the incision (Figure 27–11). 3. The lacrimal sac is elevated laterally from the lacrimal fossa. 4. The medial canthal tendon is retracted laterally by elevation of the periosteum. 5. The anterior ethmoid artery is then identified at 24 mm posterior to the anterior lacrimal crest and ligated; the posterior ethmoid artery is located 12 mm posterior to the anterior ethmoid artery, and the optic nerve at 6 mm posterior to the posterior ethmoid artery: the 24-12-6 rule (rule of halves). 6. The anterior lacrimal crest can be fractured medially into the nasal cavity with a Cottle or Freer elevator, and the anterior ethmoid air cells are entered; the anterior ethmoid cells, anterior middle turbinate, and anterior portion of lamina papyracea are then excised.

Figure 27–10.  Lynch incision.

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Figure 27–11.  Lynch approach.

7. The frontal sinus floor is then excised using a Kerrison bone rongeur. 8. Silastic/silicone sheets or polyvinyl chloride endotracheal tubes can then be used for stenting the frontal sinus. • Advantages:  minimal scarring and direct access to the frontal sinus floor, anterior ethmoid sinus, and the orbit • Disadvantages:  limited exposure, nasofrontal stenosis, orbital complications (telecanthus, epiphora, diplopia, or blindness)

Complications of the External Approach to the Ethmoid Sinus • Hemorrhage (anterior and posterior ethmoid artery) • Orbital injury (diplopia, blindness, hematoma) • CSF leak • Intracranial injury • Frontal stenosis

Transantral Approach • The ethmoid sinus is entered through an external maxillary sinus antrostomy.

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• After the anterior antrostomy is performed, the uncinate process and medial maxillary wall are excised with powered instrumentation or through-cut forceps, and the ethmoid sinus is exposed and entered 1. Sublabial incision techniques – Caldwell-Luc (see page 355) – Midface degloving (see page 355) 1. Transfacial approach: – Lateral rhinotomy (see page 357) – Weber-Ferguson incision (see page 358)

The Sphenoid Sinus • Very limited indications for the external approach exist in today’s practice. • The sphenoid sinus can be approached externally for the following conditions: 1. Extensive lateral pneumatization extending to the infratemporal fossa and pterygoid plates 2. Lateral sphenoid sinus disease 3. Large sphenoid sinus neoplasms 4. Cadaveric teaching purposes

Surgical Landmarks • Nasal septum • Fovea ethmoidalis • Superior turbinate • Lamina papyracea • Superior border of the choana • Base of the pterygoid bone and medial/lateral pterygoid plates

Transseptal Approach • Accomplished through a transfacial or sublabial approach • Transfacial approach: 1. Lateral rhinotomy incision 2. Open rhinoplasty incision: – Make an irregularized external columellar incision (gull-wing, inverted V, or stair step) at the level of foot plate of the medial crura of the lower lateral cartilage (LLC) (Figure 27–12) – Bilateral marginal incisions at the caudal edge of the LLC are connected to the columellar incision (see Figure 27–12).

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Figure 27–12.  Open rhinoplasty incision.

– The soft tissue skin envelope is then dissected above the lower lateral cartilage in an avascular plane, everted, and the nasal septum exposed. • Sublabial approach: • Using either approach, the septum is then exposed and a left hemitransfixion incision is performed. • The mucoperichondrial flap with superior and inferior tunnels is then raised and connected to a tunnel at the floor of the nose. • The septal bony cartilaginous junction is divided and the flap is raised on the right of the bony septum. • The cartilaginous septum is then disarticulated off the maxillary crest; the mucosa is elevated off the floor of the right nasal cavity. • Nasal mucosal incisions are then connected to the initial facial/ sublabial incisions.

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• The bony septum is separated from the skull base with throughcutting forceps and excised with Blakesley forceps. • Anterior wall of the sphenoid sinus is identified and the sphenoid ostium is enlarged with a Kerrison bone rongeur. • Procedure is completed using a self-retaining nasal speculum and a microscope to address the pathology. • Facial and nasal mucosal incisions are sutured primarily at the end of the procedure. • Advantages:  wide exposure, easy access to midline sphenoid and skull base pathology in the sagittal plane • Disadvantages:  prolonged operative time, single surgeon at a time (cannot perform a four-handed technique), possible facial scar, inferior microscopic visualization to endoscopic approach with angled scopes for lateral visualization

Complications of External Approach to Sphenoid Sinus • Injury to internal carotid artery and optic nerve • CSF leak • Intracranial injury • Scar formation • Nasal deformity

References • Fliss DM, Ziv G. Atlas of surgical approaches to paranasal sinuses and the skull base. Berlin, Germany: Springer; 2016. • Kimmelman CP, Weisman RA, Osguthorpe JD, Kay SL. Laryngoscope. 1988;98(11):1178–1182. • Konstantinidis I, Constantinidis J. Indications for open procedures in the endoscopic era. Curr Opin Otolaryngol Head Neck Surg. 2016;24(1):50–56. • Kountakis SE, Senior BA, Draf W. The frontal sinus. Berlin, Germany: Springer; 2005. • Myers EN, Carrau RL. Operative otolaryngology. Philadelphia, PA: Saunders/Elsevier; 2008. • Schneider JS, Day A, Clavenna M, Russell 3rd PT, Duncavage J. Early practice: External sinus surgery and procedures and complications. Otolaryngol Clin North Am. 2015;48(5):839–850.

Chapter

28 Dacryocystorhinostomy Adam P. Campbell and Benjamin S. Bleier

Lacrimal System Anatomy and Physiology . . . . . . . . 366 Anatomy of the Lacrimal System . . . . . . . . . . . . . . . . . . . 366 Differential Diagnosis of Epiphora . . . . . . . . . . . . . . . . . 366 Diagnostic Evaluation of Epiphora . . . . . . . . . . . . . . . . . 367 Surgical Management . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 369 Controversies . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 371

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Lacrimal System Anatomy and Physiology Anatomy of the Lacrimal System • Upper and lower puncta (0.3 mm diameter) lie 5 mm and 6 mm respectively from medial canthal angle, and run vertically for 2 mm. • Superior/inferior canaliculi run medially (8 mm) before forming common canaliculus. • Canaliculus enters the lacrimal sac at the valve of Rosenmüller. • The lacrimal sac lies in the bony fossa of the orbital maxillary and lacrimal bones. • The bony lacrimal canal runs vertically anterior to the middle turbinate insertion. • The canal empties into the inferior meatus at Hassner’s valve (15 mm posterior to the anterior head of the inferior turbinate).

Function • Mediates secretion, transcorneal flow, and drainage of tears • Normal function depends upon: 1. Tear production 2. Tear chemical composition 3. Eyelid position 4. Patent drainage system

Differential Diagnosis of Epiphora Congenital • Congenital nasolacrimal duct membranous obstruction • Congential nasolacrimal duct stenosis • Congenital lacrimal sac mucocele/dacryocystocele

Inflammatory/Infectious • Blepharitis • Conjunctivitis • Iritis • Keratitis • Rhinosinusitis • Dacrocystitis

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• Idiopathic (80%) • Trauma/midface fractures • Iatrogenic 1. Irradiation 2. Radioactive iodine treatment (RAI) 3. Extensive maxillary antrostomy 4. Nasal osteotomy in rhinoplasty

Neoplasm • Inverting papilloma • Lacrimal sac tumor

Systemic • Granulomatosis with polyangiitis (GPA) • Sarcoidosis • Sicca/Sjögren’s syndromes

Anatomic • Eyelid laxity/malposition • Facial palsy/paralysis • Scarring/mass obstruction • Entropion • Ectropion

Diagnostic Evaluation of Epiphora Ophthalmologic Testing • Fluorescein dye test 1. Fluorescein solution of 2% instilled in conjunctival cul-de-sac 2. Persistence/asymmetric clearance at 5 minutes indicates decreased outflow • Jones Test 1. Jones 1:  performed following fluorescein dye test – Dye placed in inferior conjunctival cul-de-sac – Duration of 5 to 10 minutes:  attempt to recover from inferior meatus 2. Jones 2:  performed following Jones 1 testing – Saline irrigation through lacrimal system

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– Irrigant in nose indicates low grade obstruction – Dye in irrigant indicates low sac or duct obstruction and patent canalicular system 3. Lacrimal Probing – Performed after negative Jones 2 test – Measure distance at which obstruction encountered

Imaging • Computed Tomography (CT) 1. Allows identification of associated sinus disease, septal deviation, anatomic abnormality, or tumor 2. Good for evaluation of bony anatomy 3. Useful for intraoperative image guidance • Magnetic Resonance Imaging (MRI) 1. Rarely utilized 2. Good for soft tissue anatomy • Ultrasound 1. Simple, noninvasive evaluation of nasolacrimal system 2. Cannot evaluate canalicular or physiologic obstruction 3. Able to identify dilated sac, neoplasm, or dacryolith • Dacryoscintigraphy (DS) 1. Radionuclide solution instilled in eye and physiologic flow assessed while images obtained 2. Poor resolution limits usefulness • Dacryocystography (DCG) 1. Gold standard of nasolacrimal system imaging 2. Radiopaque contrast solution instilled into canaliculus 3. DCG-CT allows for greater bony detail 4. DCG-MRI allows for fine soft tissue detail and can differentiate mucous, blood, and neoplasm 5. Percutaneous-contrasted DCG allows bypass of common canalicular obstruction and evaluation of lacrimal sac

Rhinologic Evaluation • Nasal Endoscopy 1. Assess for obstruction or purulence at inferior meatus 2. Allows assessment of endoscopic surgical access 3. Evaluate middle turbinate and septal deviation 4. Identify uncinate process and maxillary line 5. Identify sinonasal pathology

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Surgical Management Indications for Surgery • Epiphora • Nasolacrimal duct obstruction • Recurrent acute or chronic dacryocystorhinitis • Evaluate for neoplasm of the lacrimal system

Surgical Technique • Technique is often performed as collaborative approach with oculoplastics team. • Identify the maxillary line, uncinate process (UP), and superior attachment of middle turbinate (MT). • Perform septoplasty if necessary for access. • The flap-preserving technique utilizes a posterior-based flap above and anterior-based flap below the middle turbinate insertion. • Remove the lacrimal bone sharply and drill away the frontal process of maxilla with high-speed diamond drill. • Expose the entire medial wall of the lacrimal sac. • Dilate puncta and pass canalicular probes; tent the medial lacrimal sac. • Vertically incise the sac with sickle knife and create posterior-based flap for interposition with mucosal flaps. • Remove residual medial sac wall with forceps and send for pathology. • Pass lacrimal stents and secure intranasally.

Open Versus Endoscopic Techniques • Endoscopic Endonasal Dacryocystorhinostomy 1. No cutaneous scar 2. Preservation of the lacrimal pump system 3. Excellent, direct visualization of lacrimal sac/pathology 4. No neurovascular disruption and lower risk of CSF leak 5. Earlier postoperative recovery times 6. Lower rates of lacrimal air regurgitation with nose blowing 7. Ability to assess/treat associated intranasal pathology 8. Can convert to open approach if necessary (eg, lacrimal sac tumor) • Open External Dacryocystorhinostomy 1. Cutaneous scar

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2. Potential injury to medial canthus or cerebrospinal fluid (CSF) leak 3. Possible disruption of the physiologic lacrimal pump 4. No assessment or treatment of intranasal pathology

Surgical Outcomes • Endoscopic DCR 1. Anatomic patency >90% success 2. Symptom improvement >80% success • External DCR 1. Anatomic patency >90% success 2. Symptom improvement >80% success

Complications • Bleeding (1%) • Lacrimal stent displacement • Punctal erosion • Orbital injury 1. Subcutaneous emphysema 2. Retrobulbar hemorrhage 3. Medial rectus injury/paresis 4. Postoperative diplopia 5. Orbital fat prolapse/lamina papyracea injury • Cerebrospinal fluid leak • Canalicular obstruction

Failure Rates • Long term patency failures (~7%) • Long term failure in symptomatic improvement (~18%) • Causes of failure 1. Intranasal synechiae 2. Granuloma 3. Membranous obstruction 4. Canalicular stenosis

Revision DCR • Endoscopic DCR appropriate for both failed external and endoscopic DCR

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• Cicatrical closure of neo-ostium is the number one etiology • Long-term revision success rates similar to primary procedures

Postoperative Nasal Symptomatology • Pre- and Postoperative Sino-Nasal Outcomes Test (SNOT) 22 were compared. • An increase in scores was noted at 0 to 30 days. • Total and nasal obstruction scores returned to baseline within 30 to 90 days. • No long-term change in sinonasal symptoms following endoscopic DCR.

Controversies Mitomycin C • Anti-neoplastic agent utilized topically or intramucosally to reduce scar formation via fibroblast inhibition • Conflicting efficacy results in multiple studies • Possibly more useful in revision cases

Stenting • Used to prevent obliteration of intranasal lacrimal sac neo-ostium • Concern that may cause granulation, crusting, infection, adhesions, and canalicular lacerations leading to scarring and ostium closure • Controversy over length of time (6 weeks vs 12 weeks vs 6 months) • One RCT evidenced improved outcomes (94.7 vs 87.8) with use of stents.

Mucosal Flaps • The endonasal mucosa is preserved and connected to the lacrimal sac mucosal to reduce scarring, crusting, infection, and ostial closure. • Some studies have shown improved outcomes to nonsparing techniques with less granulation of tissue and required debridement. • This technique may be more difficult/time consuming for some clinicians.

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References • Ali MJ, Psaltis AJ, Wormald PJ. Long-term outcomes in revision powered endoscopic dacryocystorhinostomy. Int Forum Allergy Rhinol. 2014;4:1016–1019. • Fayers T, Dolman PJ. Bicanalicular silicon stents in endonasal dacrocystorhinostomy: Results of a randomized clinical trial. Ophthalmology. 2016;123(10):2255–2259. • Miyake MM, Gregorio LL, Freitag SK, et al. Impact of endoscopic dacrycystorhinostomy on sinonasal quality of life. Am J Rhinol Allergy. 2016;30(5):189–191.

Section

VI Allergy

Chapter

29 Basics of Allergy Esther Kim and Thomas G. ­Townes

Allergy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 376 Innate and Adaptive Immunity . . . . . . . . . . . . . . . . . . . 376 Epidemiology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 376 Atopy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 378 Hygiene Hypothesis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 378 Genetics . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 378 Evaluation of the Allergic Patient . . . . . . . . . . . . . . . . . 379 History and Physical Exam . . . . . . . . . . . . . . . . . . . . . . . . 379 Diseases of Allergy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 379 Allergic Rhinitis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 379 Asthma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 380 Atopic Eczema . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 380 Food Allergy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 380 Other Diseases . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 381 Allergens . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 381 Indoor Allergens . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 382 Outdoor Allergens . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 382 Allergy Testing . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 382 In Vitro Testing . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 383 In Vivo:  Skin Testing . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 383 Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 385 Avoidance . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 385 Pharmacotherapy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 386 Immunotherapy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 388

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Allergy • Exaggerated response of the immune system to an antigen, resulting in harmful effect • Basis of several disease processes, of particular importance to those concerned with rhinology and sinonasal disease • This chapter addresses the basics of allergy and allergic disease, with attention to pathophysiology, evaluation, and management.

Innate and Adaptive Immunity • The immune system has two components:  innate and adaptive. • Innate system:  variety of elements including barriers, molecules, proteins, and certain cell types that act in predetermined ways to protect the host (eg, skin, inflammatory factors, phagocytes) • Adaptive system:  elements with the ability to recognize a specific molecular pattern or antigen as foreign or host, and then mount a reaction accordingly (eg, T cells, B cells, immunoglobulins) • Importantly, the adaptive system also has memory and can mount a rapid response to an antigen confronted previously. • Hypersensitivity:  exaggerated harmful immune response to foreign antigen; four types of responses (Table 29–1 shows the Gell and Coombs classification) • Allergen:  antigen that induces a type I hypersensitive immune response, causes manifestations of allergic disease • Allergic (type I) response mediated by mast cells, basophils, IgE • Histamine release:  induces inflammatory response with two phases 1. Early phase reaction:  sneezing, itching, rhinorrhea, congestion 2. Late phase reaction:  eosinophil recruitment, cell adhesion, leukotriene secretion • Immunology of allergy discussed further in Chapter 30

Epidemiology • Allergic disease is widely prevalent and represents a significant financial burden, especially when the variety of associated diseases are considered (eg, allergic rhinitis, drug allergy, food allergy, skin allergy, asthma). • Allergic rhinitis (AR) is found in 10% to 30% of the population, with increasing worldwide prevalence, and is the most prevalent chronic disease in children.

377

Mediators

IgE mediated

Antibody mediated (IgG, IgM)

Immune complex mediated

Cell mediated

Type

Type I

Type II

Type III

Type IV

Delayed, usually over days

Usually over hours

Variable

Immediate, within minutes

Onset

Th1 cells, sensitized to foreign antigen, cause an exaggerated response, damaging host tissues in the process.

Antibodies bound to antigen form complexes that are deposited in tissues and activate immune response, damaging the normal tissues where complexes reside.

Antibodies (IgG or IgM) are sensitized to host antigen, activating immune response (esp complement system) directed at host cells.

IgE binds to antigen, crosslinks with other bound IgE, and activates mast cells, releasing histamine and other inflammatory mediators with chain reaction.

Pathologic Mechanism

Table 29–1.  Gell and Coombs Classifications of Hypersensitivity Reactions

Contact dermatitis, chronic transplant rejection

Serum sickness, rheumatoid arthritis, systemic lupus erythematous

Vasculitis, autoimmune hemolytic anemia

Allergy, anaphylaxis

Examples

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• The age group most affected by AR is late teens to early twenties. • Risk factors:  cigarette exposure, family history of atopy, higher socioeconomic status, first born or only child, elevated total IgE level before age of 6

Atopy • Atopy:  predisposition to produce IgE laden response to environmental antigen • Manifestations include eczema, food allergy, rhinitis, asthma • Atopic march:  tendency for topic patients to present with eczema and food allergy in infancy, with food allergy decreasing in incidence sharply and eczema incidence on a slower downtrend; asthma follows, with peak incidence between ages of 5 and 7, then incidence decreasing into adolescence; allergic rhinitis hits peak in early teens and remains the most prevalent manifestation of atopy throughout life

Hygiene Hypothesis • Incidence of allergic and autoimmune disease is noted to be increasing over the last 50 years, whereas the incidence of infectious disease has been falling over the same time period, largely believed due to hygiene improvements. • Allergic disease is more prevalent in higher socioeconomic groups, firstborn and only children. Each of higher socioeconomic groups, firstborn and only children are protective factors for infection. • The theory is that early exposure to antigen associated with infection (such as endotoxin), and consequent mounting of regular healthy immune responses, may be protective against development of the dysregulated immunity seen in allergy.

Genetics • allergic disease has genetic predisposition, 26% of children with one parent affected by asthma develop the disease. • Heredity is complex and multifactorial; genes and environmental influence play a role. • Ongoing studies commonly find certain genes with positive association with atopy (eg, IL 4, IL 13, TNF, high-affinity IgE receptor).

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Evaluation of the Allergic Patient History and Physical Exam History:  Establish Primary Complaints: Itching, Sneezing, Runny Nose, Obstruction • Duration:  since childhood or recent onset? • Frequency:  intermittent or persistent? • Alleviating and exacerbating factors • Associated symptoms • History of other atopic associated complaints:  eczema, asthma, food allergy • Family history • Medication use • Impact on quality of life • Social history:  smoke, smoke exposure, job or environmental change • Include questions about triggers:  pets, gardens, certain buildings, chemicals

Physical Exam • Eyes:  injection, chemosis, allergic shiners (edema or appearance of bruising under lower lid), Dennie-Morgan lines (skin folds on lower lids) • Nose:  large turbinates, boggy and/or bluish mucosa, rhinorrhea, transverse nasal crease (allergic salute:  wiping away rhinorrhea, bending the tip of the nose upward) • Ears:  TM retraction, middle ear effusion • Skin:  stigmata of pruritis (signs of scratching), dry, scaly skin (characteristic of eczema), angioedema

Diseases of Allergy Allergic Rhinitis • Hyperreactivity of the nasal respiratory mucosa to allergen • Mediated by IgE which recognizes allergen and binds to eosinophils, basophils, and mast cells, causing inflammatory factor and histamine release, resulting in manifestations of allergic disease

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• Early allergic response:  tingling, pruritus, sneezing, rhinorrhea, nasal congestion • Edema, inflammation, and rhinorrhea of AR are significant factors in the development and severity of other allergy related diseases, such as nasal congestion, sleep disordered breathing, eustachian tube dysfunction (ETD), and chronic rhinosinusitis. • Medical treatment focused on AR should be considered in all cases of these diseases; often adequate treatment of AR will resolve or greatly improve symptoms. • AR is a risk factor for developing asthma. • See Chapter 12.

Asthma • Hyperreactivity of the lower airways; inflammatory mediators promote smooth muscle contraction, vascular permeability, mucus secretion, and airway remodeling • IgE mediated sensitivity (atopy) to airborne allergens is currently the strongest identifiable risk factor for the development of asthma. • The unified airway:  paradigm gaining acceptance; states that, given the similar pathophysiologic mechanisms of AR and asthma, they should be considered different manifestations on the continuum of the same disease process (see Chapter 31) • Any patient undergoing evaluation for asthma should also be evaluated for AR and vice versa; treating one disease often improves symptoms of the other.

Atopic Eczema • Often the first disease to manifest in an atopic patient, causing dysfunction of epidermal barrier of skin and possibly predisposing to sensitization to allergens • Theorized that adequate treatment of eczema may prevent the rest of the atopic march • Up to 20% of children affected, and 2% to 10% of adults • Characterized by dry, flaky skin and pruritis, with polymorphic features including vesicles, papules, and plaques • Often resolves during childhood or early adolescence

Food Allergy • Should be differentiated from food aversion and food intolerance • Food intolerance is nonimmune mediated, and may be metabolic, pharmacologic, or toxic (lactose intolerance is the most common).

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• Food allergy arises from specific immune response and is reproducible on exposure to the food, and may be IgE mediated, non-IgE mediated, or cell mediated. • Oral tolerance:  normal ability of the immune system to suppress response to food antigen processed through digestive system; process fails in food allergy

IgE Mediated Food Allergy • Manifestations:  urticaria, anaphylaxis, oral allergy syndrome • Reproducible upon exposure • Small quantities of food cause severe reactions • Oral allergy syndrome:  one manifestation of food allergy, fast onset reaction limited to oral cavity and oropharynx, tingling, pruritis, angioedema

Non-IgE Mediated/Cell Mediated • Most commonly, celiac disease, contact dermatitis

Other Diseases • Allergic conjunctivitis, drug allergy, insect allergy, urticaria, and angioedema

Allergens • An allergen is an antigen that, upon exposure can induce a type I hypersensitive immune response in atopic individuals. • Epitope:  site of an antigen actually recognized by antibody; each antigen may have multiple epitopes • Antigens may share epitopes, leading to cross-reactivity, where sensitization to one antigen predisposes an individual to hypersensitive reaction to another similar antigen. • Most antigens are proteins with 10 to 40 kDa molecular weight. • Size effects:  larger airborne allergens typically filtered by the nose, with greatest impact on nasal mucosa; smaller allergens may reach and impact smaller airways in lungs • The terminology traditionally referred to as “seasonal” and “perennial” AR is based on sensitivity to outdoor versus indoor allergens; this paradigm is shifting to “intermittent” and “persistent” disease.

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Indoor Allergens • Dust mite:  common sensitization in patients with persistent allergic rhinitis; most common allergen in asthmatic children; exists on small particles capable of reaching lower airways • Mites found in beds, pillows, drapes; survive on shed human skin • Two species:  D. pteronyssinus (allergens Der p1, Der p2), D. farinae (allergens Der f1, Der f2) • Allergens are mite feces and shed mite skin, averaging 10 microns in size, with moderate cross-reactivity between species. • Cockroach:  allergen associated with asthma and persistent allergic rhinitis, common allergen in urban settings; 10 to 200 microns; significant cross-reactivity between species • Pet Dander:  Fel d1 is a major cat allergen, produced in the salivary and sebaceous glands, that flakes off with old skin; Can f1 is a major dog allergen. Both show minor cross-reactivity between species. • Mold:  many varied allergens with regionally based species • Common molds:  alternaria, aspergillus, cladosporium, helminthosporium, penicillium

Outdoor Allergens Pollen • Male germinal cell of plants; major source of outdoor antigenicity • Common clinically significant allergens seen in North America by season are listed in Table 29–2. It is important to note that, in addition to those listed, there are many allergens that are regionally significant; one should be aware of regionally important allergens when performing testing. • Evaluation and testing for allergen sensitivity should be tailored to high-yield allergens on a regional basis.

Allergy Testing • History remains the most important means of diagnosing allergy, but laboratory and skin testing can be useful adjuncts to confirm diagnosis, identify clinically significant allergens, and quantitate the degree of sensitivity. Positive testing results should always be correlated to clinical symptoms when considering treatment. • The basic dichotomy of testing for allergy is in vivo skin testing versus in vitro serological testing.

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Table 29–2.  Common Allergens by Season Season

Prominent Allergens

Clinically Significant Families

Spring

Tree pollens

Cypress Oak Cedar Juniper Birch Maple/box elder Olive/ash

Summer

Grass pollens

Timothy Bermuda Bahia Johnson

Fall

Weed pollens

Ragweed Sage Amaranth Russian thistle

In Vitro Testing • In vitro assays, while varied, all share the same basic conceptual structure (see Figure 29–1 for an example; lab tests for allergy are further discussed in Chapter 7). • In vitro testing has no risk of anaphylactic reaction, usually requires a single blood sample, and is unaffected by skin conditions and antihistamine use; however, skin testing has higher sensitivity and remains the workhorse for allergy diagnosis.

In Vivo:  Skin Testing • Skin testing involves controlled exposure of patient to antigen, resulting in a measurable response. • Sensitized mast cells in subepithelial skin layer release inflammatory mediators when exposed to allergen, resulting in skin wheal. • The technique requires control pricks along with allergen to ensure normal skin response to histamine and no wheal response to nonantigenic stimuli.

Figure 29–1.  1. Patient’s serum is exposed to isolated antigen of interest, allowing allergen-specific IgE in serum to bind antigen; sample is then washed to discard unbound IgE. 2. IgG, tagged with measurable label targeted to IgE antibodies (ie, anti-IgE IgG Ab), is introduced, and sample is washed again to discard unbound IgG. 3. Anti-IgE IgG is measured according to assay-specific label; different methods of labeling anti-IgE IgG account for variance in different allergy tests (eg, radiation, fluorescence, enzyme-linked assays).

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• Antihistamines and tricyclic antidepressants inhibit formation of wheal, meanwhile glycerine sensitivity and certain skin conditions may cause exaggeration of wheal response. • Negative control:  typically glycerine, the diluent for allergen in most skin tests • Positive control:  histamine, the normal mediator of the wheal response • Skin tests are designed to test multiple allergens and multiple concentrations of patient-sensitized allergens to determine the level of sensitivity, and producing both qualitative and quantitative results. • Concentrations required to induce wheal response are used to estimate the starting doses of immunotherapy. • Skin testing carries the risk of anaphylaxis and should always be performed with a physician immediately available and injectable epinephrine nearby.

Treatment • Three strategies for treatment of allergy:  avoidance of allergens, pharmacotherapy to treat symptoms, and immunotherapy to desensitize the immune system to a certain allergen

Avoidance • Outdoor allergens like pollens may be avoided by staying indoors during certain seasons, using high efficiency air filtration, and sealing doors and windows. • Patients with sensitivity to outdoor allergens often are sensitive to multiple allergens, making avoidance strategies difficult. • Methods for indoor allergen reduction are mite impermeable mattress covers, frequent high-heat washing (or freezing) of linens, air filtration, carpet removal. • Some practices have proven to reduce measured levels of allergen in home, however, few have shown reduction in clinical symptoms of allergy. • To be effective, these measures require rigorous dedication and can be costly; in practice they are difficult for patients to maintain long term. • Avoidance can be an effective strategy in certain disease processes (food allergy) or with regard to certain antigens/irritants (pet dander, tobacco smoke); however, avoiding many allergens, especially in a patient with multiple sensitivities, is often impractical.

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Pharmacotherapy Topical Corticosteroids • Examples:  fluticasone propionate, budesonide, flunisolide, mometasone furoate, fluticasone furoate • Mechanism:  steroids suppress transcription of cytokine and chemokine genes by binding intracellular receptors • Decreased amount and effect of local eosinophils, basophils, mast cells, and other inflammatory factors • Topical steroids achieve anti-inflammatory effects with less exposure to systemic risks. • Adverse effects:  epistaxis, dryness, septal perforation (rarely) • Intranasal corticosteroids are the most effective first-line treatment for allergic rhinitis, and more effective if used continuously rather than in flares. • Intranasal fluticasone furoate has also shown efficacy in treating allergic conjunctivitis.

Topical Antihistamines • Examples:  azelastine, olopatadine • Mechanism:  antagonize H1 receptors, preventing histamine binding and histamine-induced inflammatory response • Better efficacy for congestion symptoms than oral antihistamines; synergistic effects shown when used in conjunction with intranasal corticosteroid

Topical Decongestants • Examples:  oxymetazoline, phenylephrine • Mechanism:  stimulate alpha 1 and alpha 2 receptors, causing vasoconstriction leading to shrinking of nasal tissues and decreased mucus production • Adverse effects: tachyphylaxis, rhinitis medicamentosa (congestion may worsen with prolonged use of topical decongestant due to rebound effect)

Oral Antihistamines • First-generation examples:  chlorpheniramine, diphenhydramine, hydroxyzine • Second-generation examples:  cetirizine, loratadine, fexofenadine

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• Adverse effects:  sedation, decreased coordination and cognitive performance secondary to antagonism of H1 receptors located in central nervous system • Second-generation antihistamines do not cross the blood–brain barrier, and thus avoid the centrally acting effects of first generation drugs.

Oral Decongestants • Examples:  pseudoephedrine, phenylephrine • Mechanism:  stimulate alpha 1 and beta receptors, causing vasoconstriction • Adverse effects:  hypertension, glaucoma, urinary retention, irritability, insomnia

Mucolytics • Examples: guaifenesin • Mechanism:  vagal stimulation, increases parasympathetic tone, decreasing viscosity of mucus • Lacks significant supporting evidence for use in AR

Anticholinergics • Example:  ipratropium bromide • Mechanism:  decreases parasympathetic tone; used to decrease rhinorrhea in common cold, seasonal AR • Does not affect congestion, irritation, itching, sneezing

Leukotriene Receptor Antagonists • Examples:  montelukast, zafirlukast • Mechanism:  block effects of leukotrienes (potent inflammatory mediators)

Corticosteroids • Example: prednisone • Systemic steroids typically used in short courses for severe symptoms, especially with nasal polyposis • Adverse effects:  hyperglycemia, osteoporosis, glaucoma, hypertension, psychosis

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Immunotherapy • Only treatment strategy that provides potential for a “cure” to allergic disease • Repeated intentional exposure to specific antigen, with regular dosing and progressive escalation, ideally inducing immunotolerance to antigen • Usually performed subcutaneously or sublingually; these routes bypass areas with high mast cell density, ideally preventing symptoms of reaction while accessing systemic immune system • Successful treatment increases levels of antigen-specific Th1 cells and IgG, while decreasing Th2 cells, which preferentially increase IgE and activate eosinophils and mast cells. • Prerequisites for treatment 1. IgE mediated hypersensitivity:  allergic rhinitis/conjunctivitis, allergic asthma, insect sting reactions 2. Clinical symptoms and objectively measured sensitivities should be plausibly related. 3. Symptoms should be severe, with pharmacotherapy and environmental avoidance insufficient to control. • Immunotherapy has proven to be the most successful and life-saving intervention for insect sting/venom allergy. • It is important to remember that allergic rhinitis is not life threatening, but immunotherapy is potentially life threatening via an anaphylactic response; immunotherapy should not be undertaken lightly. • For safe immunotherapy practice take these precautions:  Asthma should be stably controlled in asthmatic patients, patients should not be on beta-blockers (higher risk for serious anaphylaxis), patients should remain in office for 30 minutes after injections, epinephrine to treat reactions should be available. Some recommend epinephrine auto-injectors be prescribed to patients undergoing immunotherapy. • Immunotherapy treatment regimens typically take place over 3 to 5 years. • Starting dose, escalation of therapy, maintenance dosing, and discontinuation of therapy are all clinical decisions that require careful consideration and a case-by-case approach. • Due to a better safety profile and more convenient treatment schedule in comparison to subcutaneous treatment, sublingual immunotherapy (SLIT) is poised to become the immunotherapy method of choice for treating allergic disease. • SLIT allows patients to self-escalate their therapy by following a prescribed regimen, using aqueous Ag or tablets (currently only tablets for ragweed and grass pollen are FDA approved).

Chapter

30 Immunology of Allergic Disease A. Simon Carney and Peter K. Smith

Definitions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 390 Hypersensitivity . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 390 Allergy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 390 Atopy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 390 Entopy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 390 Coombs and Gell Classification of . . . . . . . . . . . . . . . 390 Hypersensitivity Type I:  Immediate (Anaphylactic) Hypersensitivity . . 390 Type II:  Antibody-Dependent Cytotoxic . . . . . . . . . . . 390 Hypersensitivity Type III:  Immune Complex Mediated . . . . . . . . . . . . . . 390 Hypersensitivity Type IV:  Cell Mediated (Delayed Type) . . . . . . . . . . . . 391 Hypersensitivity

Mechanism of Nasal Allergy . . . . . . . . . . . . . . . . . . . . . . 391 Antigen Presentation and Sensitization . . . . . . . . . . . . . 391 The Allergic Response . . . . . . . . . . . . . . . . . . . . . . . . . . . . 392 Common Allergens . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 393 Local Allergic Rhinitis (Entopy) . . . . . . . . . . . . . . . . . . 394

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Definitions Hypersensitivity • An increased state of reactivity involving a detrimental immune and/or neural response

Allergy • An inappropriate immune protective response to a protein to which most people do not react, which involves the production of immunoglobulin E

Atopy • The genetic predisposition to allergy

Entopy • Evidence of a localized IgE mediated response in the absence of evidence of systemic IgE (RAST or skin prick tests)

Coombs and Gell Classification of Hypersensitivity Type I:  Immediate (Anaphylactic) Hypersensitivity • Ig-E mediated (eg, allergy and entopy)

Type II:  Antibody-Dependent Cytotoxic Hypersensitivity • IgM and IgG mediated (eg, blood transfusion reaction)

Type III:  Immune Complex Mediated Hypersensitivity • IgM and IgG mediated (eg, Farmers lung, SLE)

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Type IV:  Cell Mediated (Delayed Type) Hypersensitivity • T helper 1 mediated (eg, allergic “late-phase” response)

Mechanism of Nasal Allergy Antigen Presentation and Sensitization • The ciliated epithelium is covered by nasal mucous, which traps airborne molecules. • Common aero-allergens facilitate antigen presentation by their protease activities, which cleave the tight junctions between nasal epithelial cells. • The antigen within the mucous gets between the epithelial cells and is processed in the nasal mucosa by immature dendritic cells and macrophages. • Antigen processing also occurs from the mucosal barrier via microfold “m” cells, which then secrete processed antigen to the basal surface and dendritic cells. • The antigen-presenting cells migrate to the lymph nodes where they present processed allergen to naïve T cells via major histocompatibility class II molecules. • Epithelial inflammatory “danger” signals, such as IL33, TSLP, and IL25, are secreted and influence the immune response towards allergy. • The naïve T cells are skewed towards Th2 differentiation. • Memory allergen-specific Th2 cells produce cytokines such as IL-4, IL-5, IL-9, and IL-13. • IL-4 maintains Th2 lineage and recruits T-helper cells. • IL-13 promotes heavy-chain class switching in B lymphocytes in favor of IgE production. • IL-4, 5, and 13 also promote differentiation of B cells into plasma cells. • Plasma cells produce specific IgE to the antigen and excrete these into the serum. • IgE binds to the high-affinity IgE receptor (FcεRI) on the surface of mast cells, basophils, eosinophils, and antigen-presenting cells, sensitizing these cells to the allergen.

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The Allergic Response Early-Phase Allergic Response • Upon subsequent exposure to the allergen, cross-linking of the antigen occurs with IgE on sensitized cells; this results in a fasttracked, directed, and amplified defensive immune response to a predetermined threat. • IgE-FcεRI interaction with antigen initiates mast cells calcium influx into the mast cell. • Prestored granules move via microtubules to the membrane, and endosomes fuse with the cell surface to release granules into the environment. • Mast cell granules contain histamine; tryptase; leukotrienes C4, D4, and E4; prostaglandins D2; and other mediators including vascular endothelial growth factor. • These mast cell products produce the clinical type I hypersensitivity reaction (immediate—symptoms can be identified in less than a minute). • Clinical effects:  watery rhinorrhea, itching, sneezing and nasal congestion; these are protective responses that clear the antigen, and make the sufferer aware of the threat to which they are sensitized; enzymes such as lysozyme and proteases destroy the antigen, and nasal congestion helps to restrict entry into the airways; by increasing the surface area of the turbinates, there is potential greater surface area to restrict antigen entry to the airways • Histamine causes itching via H1-receptors on sensory nerve endings. • Leukotrienes, PGD2, and VEGF cause capillary leakage, tissue edema, venous congestion, and an increase in glandular secretions (rhinorrhea, nasal congestion).

Late Phase-Allergic Response • Depending on patient factors and allergen dose, more mast cells, eosinophils, and T cells are recruited into the nasal mucosa. • Histamine, PDG2, and leukotrienes are the stimuli for inflammatory cell migration. • The influx of these inflammatory cells is facilitated by the up regulation of adhesion molecules, E-selectin, and IL-5. • IL-5 is important in eosinophil recruitment, adhesion, and activation. • Eosinophils release major basic protein, eosinophilic cationic protein, and eosinophilic peroxidase, which produce and prolong

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the type IV (late-phase) symptoms of chronic nasal congestion 24 to 48 hours after allergen exposure. • Some watery discharge is also produced by the late-phase allergic response.

Priming Effect • The allergen dose needed to elicit a response decreases with multiple exposures to that allergen. • This means patients will often start to experience symptoms very early in the pollen season when levels are still low. • Over the season more inflammatory cells migrate to the submucosa. • Sensory nerves are stimulated by inflammatory cell products to lower their threshold for activation. • Eosinophil-derived nerve growth factor increases sensory nerve expression of transient receptor potential vanilloid 1 receptor; this is the receptor through which histamine largely works through to induce neural symptoms. • Towards the end of the pollen season, a lower level of allergen will cause more symptoms in the presence of activated sensory nerves and more submucosal inflammatory cells. • Patients will also still experience significant symptoms towards the end of the season when pollen levels are dropping.

Common Allergens • Allergens are mainly proteins and glycoproteins found in airborne particles. • House dust mite:  Dermatophagoides pteronyssinus and D. farinae are the major indoor allergens; they do not drink and rely on ambient humidity; they are vulnerable to dessication and die when humidity 3 mm larger than the negative control

Intradermal Testing • Alternative to skin prick testing • More sensitive than skin prick testing • More likely to cause systemic reaction

Single-Dilution Intradermal Testing • Generally performed after a negative skin prick test • Mildly diluted allergen is injected into the dermis to form a small 4 to 5 mm intradermal wheal • Considered positive when the wheal is >5 mm or >2 mm larger than the negative control

Multiple-Dilution Intradermal Testing • First start with extremely dilute allergen (dilution historically used by otolaryngologists is 1:5; dilution #3 is 1:100 w/v, dilution #2 is 1:500 w/v, etc). • Negative test is followed by stronger concentration.

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• Two consecutive wheals enlarging by >2 mm suggest allergic sensitization. 1. The first enlarged wheal is known as the “endpoint.” – It can be used to determine the dose for starting immunotherapy 2. The second enlarged wheal is known as the “confirming” wheal. 3. A series of negative responses followed by a very large positive wheal is known as a flash response. – Stop and retest at a later date. 4. A positive wheal followed by another wheal of similar size is known as a plateau response; the endpoint is the last positive wheal just before the endpoint wheal.

Using Epicutaneous and Intradermal Testing in Combination Modified Quantitative Testing • Cost-effective means of obtaining both qualitative and quantitative data • Epicutaneous prick testing performed first to estimate presence and level of sensitivity • Single dilution intradermal test performed to estimate endpoint • Assumes positive response to prick test suggests level of reactivity to #3–4 dilution in intradermal testing • May be used to identify false negatives on prick testing 1. Can identify individuals who would react to prick testing at a stronger #2–3 dilution

Managing Emergencies Related to Skin Testing Diagnosis of Anaphylaxis • Severe, systemic immediate hypersensitivity reaction leading to the following conditions. 1. Early signs – Tachycardia – Skin flushing

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– Generalized pruritis – Nausea and vomiting – Abdominal cramps – Urticaria 2. Late signs – Dyspnea – Wheezing – Angioedema – Hypotension – Airway obstruction – Cardiovascular collapse • Possibly biphasic 1. Due to late-phase allergic reaction 2. Symptoms may recur up to 28 hours after initial reaction.

Prevention of Anaphylaxis • Understand common risk factors for anaphylaxis 1. Buildup immunotherapy 2. Active asthma 3. First injection of new vial 4. Prior episode of anaphylaxis • Observe patients for 30 minutes after skin testing or immunotherapy. • Have predetermined anaphylaxis treatment algorithm and train clinic staff appropriately. • Have the following medications available for treatment of anaphylaxis: 1. Epinephrine 2. Oxygen 3. Albuterol 4. Diphenhydramine 5. Corticosteroids 6. Glucagon

Management of Anaphylaxis • Immediately contact emergency services and ambulance for transport to nearest hospital. • Provide basic and advanced cardiac life support. 1. Airway and breathing assessment, administration of supplemental oxygen

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Principles of Allergy Skin Testing   407

2. Circulatory support – Place in Trendelenburg position – Pulse and blood pressure monitoring – Place tourniquet proximal to site of immunotherapy/allergen injection, release every 5 minutes – Management of hypotension with fluid resuscitation 3. Early administration of epinephrine; can administer repeat dose in 5 minutes if not adequate response – Adults:  0.3 to 0.5mL of 1:1000 epinephrine – Children:  0.1 to 0.3mL of 1:1000 epinephrine 4. Management of bronchospasm – Albuterol – Ipratropium bromide, particularly if patient on beta blocker 5. Administration of adjunctive medications – Diphenhydramine – Corticosteroids, particularly to avoid biphasic anaphylaxis – Glucagon to help avoid unopposed α adrenergic stimulation if patient on beta blocker

Chapter

33 Allergen Immunotherapy: Subcutaneous and Sublingual Andrea M. Hebert and Stella E. Lee

Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 410 Mechanism of Action . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 410 Goal . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 410 Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 411 Efficacy of Immunotherapy . . . . . . . . . . . . . . . . . . . . . . . 411 Indications for Use of Immunotherapy . . . . . . . . . . . . . . 412 (SCIT or SLIT) SCIT Versus SLIT:  Administration . . . . . . . . . . . . . . . . . 413 Side Effects . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 415

409

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Introduction • Although pharmacotherapy, in conjunction with environmental controls, may be effective in controlling allergic rhinitis symptoms, immunotherapy is the only disease-modifying treatment that results in prolonged benefit; immunotherapy options include subcutaneous immunotherapy (SCIT) and sublingual (SLIT).

Mechanism of Action Goal • To induce immune tolerance to specific antigens by modifying the Th2 skewed immune response

T Cell Response

Shifts towards a regulatory T cell (Treg) response • Mediated by IL-10, TGF-b, and other suppressive factors • IL-10 and Treg cells then shift immune balance by 1. Suppressing IgE and increasing IgG4 production 2. Decreasing mast cell and basophil activation 3. Decreasing IgE mediated histamine release 4. Downregulating eosinophil function and activity 5. Suppressing IL-5 production by T cells • IL-12 is increased and IL-4 and IL-5 are decreased.

B Cell Response

Induction of IgG4, IgA production, downregulation of IgE production • Changes in immunoglobulins:  allergen-specific IgE and IgG4 responses 1. Serum-specific IgE levels transiently increase after immunotherapy and decrease over months to years of treatment. 2. Specific IgG4 levels increase during immunotherapy, suggesting that IgG4 inhibits the following: – Allergen-induced and IgE mediated release of inflammatory mediators from basophils and mast cells – IgE facilitated allergen presentation to T cells – Allergen-induced boost of memory IgE production during allergen exposure

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SLIT:  Specific Mechanism of Action

Induction of tolerance via similar mechanisms as SCIT but via uptake of antigen by dendritic cells in the sublingual region of the oral cavity • Sublingual allergen extracts are primarily taken up by dendritic cells in the mucosa and presented to T cells in the corresponding lymph nodes. 1. Likely mechanisms of action in immune tolerance is similar to what is described above. 2. Within the oral and sublingual mucosa, effector cells, such as mast cells, are less numerous—a possible factor in the lower rates of adverse systemic allergic reactions seen with SLIT.

Treatment Efficacy of Immunotherapy Allergic Rhinoconjunctivitis and Allergic Asthma

Level I evidence that both SCIT and SLIT are effective at decreasing symptoms, prevent new sensitizations, prevent asthma, and provide long-term symptom control • Duration of efficacy:  effects can last for several years after treatment (7 to 12 years) • Multiple studies have demonstrated improvement in symptom score, medication score, favorable changes in immunologic markers, and improvements in quality of life. • Both SLIT and SCIT reduce topical corticosteroid use and improve allergic disease severity.

Prevention • SCIT and SLIT appear to prevent progression of allergic rhinitis to asthma and the development of new allergen sensitivities.

Cost Comparison • Studies comparing cost effectiveness have indicated that immunotherapy compared to pharmacotherapy might be associated with cost savings as high as 80% 3 years after completion of treatment.

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Efficacy of SCIT Versus SLIT

Currently no direct head-to-head studies have been completed but both methods of immunotherapy appear to convey benefit. • Meta-analyses comparing the two treatment options for grass pollen found greater overall benefit with SCIT; however, the data was indirect and limited by a very high degree of heterogeneity. • A small number of trials have directly compared SLIT and SCIT, finding SCIT to be at least somewhat more effective than SLIT. • SLIT appears to be safer with less risk of anaphylaxis.

Indications for Use of Immunotherapy (SCIT or SLIT) Immunotherapy may be used for: • Allergic rhinitis (seasonal or perennial), with or without allergic conjunctivitis • Allergic asthma (seasonal or perennial) • Stinging insect hypersensitivity • Atopic dermatitis:  may respond if the patient is sensitized to inhalant allergen (aeroallergen)

Must Have Clinically Relevant Allergic Component to Their Disease Established By • History of symptoms with exposure to the allergen. This is the most important consideration when deciding whether to test and treat a patient with immunotherapy since a high percentage of the US population is atopic basic on allergy testing, but not all patients with a positive test have clinically relevant symptoms. • Presence of specific IgE to that allergen, demonstrated through allergen skin testing or serum tests (positive allergen test that is clinically significant).

Trial of Control Measures

A trial of pharmacotherapy and environmental control measures is reasonable prior to initiation of immunotherapy; the data regarding environmental control measures and efficacy is weak. • Patients should be considered for immunotherapy for the following: 1. Side effects of medication use 2. Noncompliance with medication regimen or suboptimal use of medication devices

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3. Persistent symptoms with inadequate or partial response to pharmacotherapy/environmental controls 4. Patient preference 5. Cost burden associated with chronic medication use

Patient Selection • Special consideration should be made when deciding to initiate immunotherapy especially in the following patient populations: 1. Severe or unstable asthma:  The World Health Organization (WHO) task force on immunotherapy recommends that SCIT not be administered to patients with asthma who show a forced expiratory volume in one second (FEV1) 65 years of age • Onset 1. Variable timing:  although most cases present shortly after initiation of medication, some do not present until years later; some will occur even after discontinuation of the medication 2. Variable location:  but oral cavity and throat most often affected

Idiopathic Cases (normal C4) • These patients lack family history, have normal C4, and have no clear triggers for their angioedema. • Clinician needs to rule out FXII-HAE with low penetrance.

Work-Up for Angioedema Without Urticaria History and Physical Exam Is Critical • Is there a family history? • Are there any triggers? • Was the patient on ACE-I? • Were urticaria present?

Lab Testing (Figure 34–4) • Screening test 1. C4 is the first test for suspected C1-INH deficiency:  low at all times, even between attacks of angioedema. • If C4 is low, clinician follow-up with tests of C1q and C1-INH levels and function 1. Low C1q:  acquired angioedema – Look for lymphoproliferative or immunomodulatory diseases. – Test for auto-antibodies against C1-INH. 1. Normal C1q:  genetic angioedema – Positive FMHx:  suspect HAE (type 1 vs type 2) – Negative FMHx:  suspect de novo SERPING1 mutation • If C4 is normal (and no ACE-I) 2. Check for FXII mutation and FMHx 3. Positive FMHx and mutation present:  FXII-HAE

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Figure 34–4.  Lab work-up for angioedema without wheals.

4. Negative FMHx and mutation present:  de novo FXII-HAE 5. Negative FMHx, no mutation present:  idiopathic-AAE

Treatment for Angioedema Without Urticaria HAE • C1 esterase inhibitor concentrate (Cinryze, Berinert, or Ruconest) • Bradykinin B2-receptor antagonist (icatibant) • Kallikrein inhibitor (ecallantide) • Fresh frozen plasma (FFP) • Attenuated androgens (danazol, stanozolol)

ACE-I AAE • Stop ACE-I if present (this is important even for patients with known HAE-C1-INH) • Icatibant may be considered.

Idiopathic • Start with monotherapy with normal doses of second-generation antihistamines (eg, cetirizine). • Failure to respond should be followed by off-label, higher doses of second-generation antihistamines.

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• Failure to respond to this can be followed with a short course of systemic steroids with a taper.

FXII-HAE • Consider stopping oral contraceptives or hormone replacement in patients with FXII- HAE.

AAE • Address lymphoproliferative or autoimmune disease.

Angioedema With Urticaria Introduction Overview • Angioedema is seen in 40% of patients with urticarial. • When urticaria is present, with or without edema, the differential diagnosis changes significantly. • The presence of urticaria suggests a histamine-mediated allergic disease.

Classification • By duration 1. Even though each urticarial lesion lasts for less than 24 hours, the development of new rashes can last for weeks, months, or years. 2. Acute urticarial:  defined as duration of urticarial disease 6 weeks • By trigger 1. Spontaneous:  no known triggers 2. Physical:  well-defined physical triggers

Differential Diagnosis • The “diagnosis” of urticaria suggests presence of a very specific lesion, whether a trigger is present or absent; however, there are some rashes that mimic urticarial rash.

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• These other conditions should be suspected in patients who either lack “classic” findings or who fail to respond to appropriate treatment. • Urticarial vasculitis 1. Lesion may last >24 hours 2. Rather than pruritis, pain and burning are significant symptoms. 3. Following resolution, the skin is often hyperpigmented. 4. Key finding on biopsy is leukocytoclastic vasculitis. • Autoimmune diseases 1. The urticarial rashes in these patients are not typical, but more importantly, they are associated with fever, joint pain, and malaise. 2. Examples include: – Hereditary periodic fevers:  genetic testing for NLRP3 gene – Schnitzler syndrome:  look for monoclonal gammopathy

Acute Spontaneous Urticaria Epidemiology • Lifetime risk is about 20%. • Less than 50% are associated with angioedema.

Etiology • Infections:  especially viral infections in children • Allergies:  food, medications (eg, NSAIDS, antibiotics), insect stings

Differential • Anaphylaxis:  This should be suspected if changes in organ systems, aside from skin, are noted (eg, wheezing, vomiting, altered mental status, changes in heart rate).

Work-Up • Your history and physical exam is the most important part of the evaluation in order to determine what triggers were present. • Since most cases will resolve spontaneously, no routine lab evaluation is indicated.

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Urticaria and Angioedema   427

• Skin biopsy is only indicated for cases with atypical presentation in which disease such as urticarial vasculitis is suspected. • Allergy testing may be indicated to confirm suspected triggers.

Treatment • Mild cases:  no treatment required, just withdrawal of offending agents • More severe cases 1. Second-generation antihistamines will be all you need in the majority of cases—cetirizine and levocetirizine may be the most effective. 2. Higher doses of the antihistamines are indicated if initial response is poor. 3. Oral steroids are considered if angioedema is present or if refractory to antihistamines. 4. Epinephrine autoinjector if history suggests anaphylaxis.

Chronic Spontaneous Urticaria Epidemiology • Incidence:  1% per year • Prevalence:  estimated to be between 0.5% and 5% • Associated with angioedema:  >50%

Etiology • Autoimmune 1. Association has been found between chronic spontaneous urticaria and autoimmune thyroid disease, Sjögren’s, SLE, rheumatoid arthritis (RA), and type 1 diabetes (DM1). 2. 50% of these patients have IgG autoantibodies to the IgE receptor (50%) or IgE itself (10%). • Idiopathic 1. Many diseases have been associated with chronic urticaria, but associations remain unproven (eg, hepatitis, EBV, HSV, H. pylori, parasitic infections). 2. The majority of cases of chronic urticaria are of unknown etiology.

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Natural History • Tends to be self-limited with a duration of 2 to 5 years • Continuing symptoms for about 20% of patients after 5 years

Work-Up • No routine lab work-up is indicated, given the lack of knowledge about its origin. • The ASST (autologous serum skin test) can be used to detect IgG autoantibodies to high-affinity IgE receptor or to IgE antibody itself; however, the specificity of the test is very poor, limiting its clinical applications.

Treatment • Avoidance of vasodilatory triggers—alcohol, heat, spicy foods, stress, NSAIDs—is encouraged. • Similar treatment as acute spontaneous urticaria is provided, but additional immunomodulatory agents are used in refractory cases. • Omalizumab (anti-IgE monoclonal antibody) is safe and effective for the treatment of refractory CU. • Cyclosporine is also effective for refractory cases, but risks of hypertension, nephrotoxicity, neurotoxicity, and drug interactions (due to metabolism by the P-450 system) limit its use.

Physical Urticaria (chronic by definition) Introduction (Table 34–3) • Accounts for 20% of cases of chronic urticarial; can co-exist with chronic spontaneous urticaria • The physical urticarias simply represent cases of urticaria with known physical triggers. • Some cases are familial; these tend to be the longest lasting of the chronic urticaria, often for years.

Management • Avoidance • Similar medications as for chronic spontaneous urticaria

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Table 34–3.  Types of Physical Urticaria Type

Description

Pressure, dermatographism

Urticaria shortly after pressure application

Delayed pressure (DPUA)

Delayed urticarial lesions 3–12 hours after pressure application

Vibratory

Seen in people using pneumatic hammers, biking on cobblestoned streets, or using massage devices

Contact

Contact with allergen, eg latex

Cold

Urticaria and angioedema occur following exposure to cold

Cholinergic urticaria

Characterized by tiny urticarial lesions and angioedema when the body temperature rises

Exercise-induced anaphylaxis/urticaria (EIA)

Urticaria or anaphylaxis occurs with exercise

Aquagenic

Contact with water Not associated with angioedema

Solar

Sunlight induced Not associated with angioedema

References • Asero R, Tedeschi A, and Cugno M. Treatment of chronic urticaria. Immunol Allergy Clin North Am. 2014;34(1):105–116. • Bernstein JA, Lang DM, Khan DA et al. The diagnosis and management of acute and chronic urticaria: 2014 update. J Allergy Clin Immunol. 2014;133(5):1270–1277. • Bork K. Angioedema. Immunol Allergy Clin North Am. 2014;34(1):​ 23–31. • Farkas H, Veszeli N, Kajdácsi E, Cervenak L, Varga L. “Nuts and bolts” of laboratory evaluation of angioedema. Clin Rev Allergy Immunol. 2016;51(2):140–151. • Maurer, M et al. Practical algorithm for diagnosing patients with recurrent wheals or angioedema. Allergy. 2013;68(6):816–819. • Wu MA, Perego F, Zanichelli A, Cicardi M. Angioedema phenotypes: Disease expression and classification. Clin Rev Allergy Immunol. 2016;51(2):162–169.

Section

VII Surgical Management of Skull Base Disorders

Chapter

35 The Nasopharynx Peter F. Svider, Sean Mutchnick, and Adam J. Folbe

Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 434 Anatomy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 434 Layers . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 434 Borders . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 434 Anatomic Subsites . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 435 Imaging . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 435 CT . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 435 MRI . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 435 Angiography . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 435 PET-CT . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 435 Presenting Signs and Symptoms of . . . . . . . . . . . . . . . . 435 Nasopharyngeal Lesions Evaluation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 436 Benign Lesions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 438 Malignant Lesions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 439 Nasopharyngeal Carcinoma . . . . . . . . . . . . . . . . . . . . . . . 440

433

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Introduction • Nasopharyngeal lesions encompass a wide range of etiologies, ranging from asymptomatic entities to destructive malignancies. The existence of the nasopharynx as an anatomic “intersection” among oral/oropharyngeal, skull base, and sinonasal structures presents unique diagnostic challenges. The close proximity of critical structures yields a potential for a variety of presenting signs and symptoms. Furthermore, the relatively secluded location of the nasopharynx compared to several other head and neck subsites allows many lesions to grow significantly prior to detection; this relative inaccessibility can make surgical management clinically challenging.

Anatomy (Figure 35–1) Layers • Mucosa • Pharyngobasilar fascia 1. Inserts at posterior aspect of medial pterygoid plate

Borders • Anterior: choanae • Anterolateral:  medial pterygoid plate 1. Sinus of Morgagni is posterior to this

Figure 35–1.  Anatomy of nasopharynx (sagittal).

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• Inferior:  soft palate (free edge) • Superior/posterior:  skull base, clivus, C1

Anatomic Subsites • Roof • Lateral walls • Posterior pharyngeal wall

Imaging CT • Details bony/skull base involvement, destruction, erosion

MRI • Better for evaluation of soft tissue, nerves/perineural involvement

Angiography • Helpful for evaluation of vascular lesions (see Juvenile Nasopharyngeal Angiofibroma below)

PET-CT (Figure 35–2) • As in other head and neck sites, may be useful for differentiating between benign and malignant processes • Can be particularly helpful in follow-up/post-treatment surveillance for NPC • Evaluation of distant metastases/staging in malignant disease • May be useful for differentiating between NPC recurrence versus post-radiation changes

Presenting Signs and Symptoms of Nasopharyngeal Lesions • Nasal 1. Epistaxis (vascular lesions, malignancies) 2. Nasal obstruction • Neurologic 1. Cranial neuropathies

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Figure 35–2.  MR (left panels) of a right-sided nasopharyngeal adenocarcinoma, with corresponding PET-CT imaging (right panels) demonstrating FDG avidity of lesion.

• Otologic 1. Hearing loss (mass effect on Eustachian tube leading to middle ear effusion) 2. Otalgia • Neck mass (NPC)

Evaluation • In addition to nasopharyngoscopy (Figure 35–3), make sure to do a full head and neck exam including cranial nerve exam, neck exam, otologic exam 1. Consider FNA of enlarged cervical lymph nodes • Imaging (see above)

437

Figure 35–3.  Endoscopic anatomy of nasopharynx.

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• Biopsy (imaging prior to biopsy to see if vascular lesion, intracranial involvement, encephalocele, etc) • Dental evaluation (if radiation planned) • Audiometry

Benign Lesions • Tornwaldt Cyst 1. Notochord remnant 2. Midline nasopharyngeal cyst 3. Submucosal 4. If symptomatic can consider removal/marsupialization 5. If asymptomatic can observe – Often discovered incidentally on imaging – Benign-appearing on imaging a. Well demarcated • Mucous retention cyst • Papilloma • Vascular lesions 1. Hemangiopericytoma (considered low-grade/malignant) 2. Juvenile nasopharyngeal angiofibroma – Nasopharyngeal origin – Signs/symptoms:  intermittent epistaxis, nasopharyngeal mass, nasal obstruction – Males, adolescents – Traditionally one-third recur; no evidence that this is related to choice of approach – Locally destructive, can extend as noted below: a. Posteriorly/superiorly:  sphenoid sinus, clivus, skull base, intracranial b. Anteriorly:  nasal cavity, ethmoid sinuses c. Laterally:  pterygopalatine fossa (expansion on imaging; can bow posterior wall of maxillary sinus—HolmanMiller sign on CT) – Management a. Preoperative embolization (onyx) has largely become standard of care ■ Can be transarterial versus direct puncture ■ Decreased intraoperative bleeding ■ Decreased operative times b. Open approaches (transfacial, midface degloving) ■ Advanced lesions

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The Nasopharynx   439

■ In centers where there is not expertise with minimally invasive approaches c. Endoscopic Resection ■ Has become standard of care at most tertiary centers when feasible • Rathke’s Cyst 1. Midline 2. Nasopharyngeal epithelium invagination developing into adenohypophysis (anterior pituitary gland) 3. Failure of this pouch to obliterate results in cyst. 4. Cyst can enlarge, cause endocrine dysfunction, involve optic chiasm. 5. High recurrence rate (12.5%) 6. Less endocrine dysfunction with endoscopic (10%) versus microscopic (25%) resection • Craniopharyngioma 1. Tumor from Rathke’s pouch 2. Can present along course of migration of adenohypopohysis – From third ventricle to nasopharynx • Clival lesions 1. Discussed in Chapter 40 2. These can include hematologic malignancies, including plasmacytomas. 3. Malignant lesions (chordoma, chondrosarcoma) are more common than benign ones. 4. The clivus separates the nasopharynx/sphenoid sinus from the retroclival structures (posterior fossa, basilar artery, brain stem). • Minor salivary gland tumors 1. There are minor salivary glands in the nasopharynx. – Most commonly pleomorphic adenoma; nasopharynx: uncommon location • Teratoma • Congenital Masses 1. Masses more frequently originate in sinonasal locations, but these can include dermoid cysts, gliomas, encephaloceles.

Malignant Lesions • Nasopharyngeal carcinoma (see section below) • Adenocarcinoma • Salivary gland malignancies

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1. Adenoid cystic carcinoma (most common) – Perineural spread a. MRI more sensitive for detection b. Survival rates not stabilized after 5 years ■ Lung metastases many years after initial presentation/ management 2. Mucoepidermoid carcinoma • Clival lesions 1. Chordoma – Most common clival lesion – Histologically derived from notochord remnants – Chordoma can present with CN VI palsy as it is next to Dorello’s canal. – Five-year survival ~65% – Metastasis very uncommon 2. Chondrosarcoma (second most common) – Second most common clival lesion – Derived from endochondral cartilage – Possible association with Paget’s disease – Five-year survival rate ~90% 3. Metastasis – On T2 MRI usually has lower signal compared to chordoma/ chondrosarcoma 4. Endoscopic endonasal approaches have evolved considerably, and are now utilized at leading skull base centers. – Vidian nerve is an important structure to identify, facilitates staying below the ICA intraoperatively. • Lymphoma 1. Presenting S/Sx – Neck mass (42%) – Nasal obstruction (37%) – Hearing loss (32%) 2. Non-Hodgkin’s more common 3. Burkitt’s lymphoma associated with EBV – Association between NPC and EBV (see below)

Nasopharyngeal Carcinoma • Usually originates from fossa of Rosenmüller • Can spread laterally by eustachian tube, involving parapharyngeal space, skull base (foramen ovale) 1. Trotter syndrome – Unilateral soft palate akinesia – Unilateral trigeminal neuralgia

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The Nasopharynx   441

– Unilateral hearing loss • S/Sx 1. Neck Mass (41%) – Level V in more than half of patients – Level II (49% of patients with neck mass) 2. Hearing loss/drainage (27%) 3. Nasal complaints (21%) 4. Cranial neuropathies (8%) – CN VI most commonly • Staging (Table 35–1) 1. Multiple staging systems in use – AJCC a. Relative 5-year survival rates with treatment (www.cancer.org) Table 35–1.  Staging and Classification of Nasopharyngeal Carcinoma WHO Classification Type I

Keratinizing Squamous Cell Carcinoma – Poorest prognosis – Stronger association with alcohol, smoking – More common in United States

Type II

Non-Keratinizing Differentiated Carcinoma

Type III

Non-Keratinizing Undifferentiated Carcinoma – Better prognosis – Stronger link with EBV – More common in south China

AJCC—Primary Site Staging T1

Can involve oropharynx/nasal cavity

T2

Parapharyngeal space involvement

T3

Skull base/bony/sinus involvement

T4

Dural/intracranial/cranial nerve/infratemporal fossa/orbit/ hypopharyngeal involvement

Nodal Staging N1

Foramen of Monro > third ventricle > Aqueduct of Sylvius > fourth ventricle > Foramen of Magendie + Foramina of Luschka > cisterna magna + subarachnoid space • Absorbed at arachnoid villi in subarachnoid space • Production and absorption cycles results:  3 to 5× total volume of CSF turnover per day • Normal ICP:  5 to 15 cm H20 supine • Increased ICP:  sustained pressures of 20 to 30 cm H20 supine • The functions of CSF are to cushion the brain and spinal cord tissues and to metabolize and remove waste products.

CSF Leak • Implies an abnormal communication (= fistula) between the arachnoid space and the sinonasal cavity • Differentials for CSF rhinorrhea 1. Trauma (closed head injuries or skull fractures) 2. Iatrogenic 3. Congenital 4. Arachnoid granulations 5. Increased ICP:  tumors, hydrocephalus, post-trauma, post infectious, benign raised ICP 6. From middle ear roof (ie, CSF otorrhea via the eustachian tube) • Neurosurgery consulted for spontaneous CSF leaks to rule out raised ICP • Most common:  non-iatrogenic traumatic CSF leaks • Iatrogenic CSF leaks are uncommon, and occur in less than 1% of all endoscopic sinus surgeries. • Schlosser and Bolger classified CSF leaks into five categories:  accidental trauma, surgical trauma, spontaneous, congenital, and neoplastic leaks; Lopatin et al. classified them into

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Cerebrospinal Fluid Leaks and Encephaloceles   457

primary (spontaneous) and secondary; Gendeh et al. classified them into congenital, acquired, and spontaneous.

Spontaneous Leaks • The prevalence in various series varies from 3% to 36%. • 75% of these leaks are associated with raised ICP • 50% to 100% of these leaks have associated encephaloceles; likely due to the long-standing nature of these leaks • The leaks are notoriously difficult to seal, and are associated with 25% to 87% recurrence rate.

Encephaloceles • Herniation of intracranial contents through bony defects • Meningocele involves herniation of dura only. • Encephalocele (more accurately termed meningoencephalocele) involves herniation of dura and brain. • Encephalocystocele involves herniation of dura, brain, and part of the ventricular system. • A relatively larger skull base defect can cause CSF leaks with herniation of the above. • These herniated materials are considered non-functioning, and are to be surgically removed prior to the repair of the skull base defect. • Congenital encephaloceles:  three subtypes 1. Occipital:  most common; ~75% of cases 2. Sincipital (frontoethmoidal):  ~15% of cases; nasofrontal (most common subtype), nasoethmoidal, nasoorbital 3. Basal:  ~10% of cases; transethmoidal (most common subtype), sphenoethmoidal, transsphenoidal, sphenoorbital

Diagnosis Signs and Symptoms • Patient often have few symptoms until a significant leak occurs. • They may complain of clear, runny nasal discharge, which is often unilateral, commonly associated with headaches. • This is increased when the patient bends over (reservoir sign) or increases his or her intracranial pressure with Valsalva-like maneuvers. • The expansion of a nasal encephalocele with compression of the internal jugular vein or with crying /straining is known as

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Furstenberg’s sign; this is not present in gliomas or dermoids as there is no associated patent CSF tract. • A central nervous system complication—for example, meningitis or intracranial abscess—may be the first presentation for some patients.

Diagnostic Tests • Clinical:  halo sign; only in traumatic injuries with mixed CSF and bloody rhinorrhea; a drop of the rhinorrhea will separate into two haloes (central ring of blood and an outer ring of CSF) on a filter paper • Clinical:  diagnostic endoscopy with maneuvers like bending forward and Valsalva • Biochemical:  presence of glucose (≥5 mg/dL); absent in nasal mucus • Biochemical:  β2 transferrin (sensitivity 99% and specificity 97%), a protein found in CSF, aqueous humour, and perilymphatic fluid, but not in blood or nasal secretions; the presence of cirrhosis or congenital protein anomalies increases false positive • A newer assay, β trace protein appears very promising (in reports, sensitivity and specificity reaches 100%) . • High-resolution axial CT with coronal and sagittal reconstructions • High-resolution MRI of skull base:  to evaluate any herniation of cerebral tissues and dural through the defect, or any associated complications like meningitis and abscesses • Intrathecal myelograms and cisternograms:  traditional methods, but invasive and with the inherent risk of meningitis • CT cisternogram:  needs intrathecal contrast as well; invasive risk • MRI cisternography:  non-invasive, with T2 and specific sequences • Radionucleotide scanning or scintigraphy:  high false positive rate; often only identifying the side of leak • Intraoperative intrathecal fluorescein 1. 0.1 cc of 10% fluorescein in 10 cc of CSF is infused over a period of 10 minutes (no faster than 1 cc per minute) 2. Intrathecal fluorescein is an off-label use; risks include generalized seizures, opisthotonus, lower limb weakness/ paresthesias, headache, cranial nerve deficits; these are uncommon to rare and are associated with higher doses or faster rates of infusions. 3. Intrathecal fluorescein will stain the CSF leak yellowish green; this is very useful in small or intermittent leaks; Trendelenburg positioning, Valsalva to the ventilation circuit, and a blue light filter are methods to increase identification.

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Treatment and Management Medical Management • Complete bed rest with head elevated at 30 degrees • Advise for avoidance of straining/ bending/ lifting • Stool softeners • +/− Lumbar drains • Acetazolamide:  a diuretic; decreases CSF production by up to 48% • Use of antibiotics is controversial.

Surgical Management • Indication:  failed medical management (eg, traumatic leak persists >10 days) • Approaches for CSF leak repair 1. Intracranial 2. Extracranial external 3. Endoscopic • Choice depends on 1. Site and size of defect 2. Any raised ICP 3. Etiology • Endoscopic closure 1. Many published series show success rates of > 90% (traditional craniotomy success rates are between 70% and 80%). 2. Generally, this is the treatment of choice as there is low morbidity, no external scars, fast recovery, and excellent success rates. 3. Various materials have been used (free mucosal grafts, pedicled grafts, fat, temporalis fascia, muscle and synthetic materials like tutoplast); no one material appears definitely superior in various series. • Principles of endoscopic closure 1. Careful identification of leak site(s) 2. Cauterization and excision of encephaloceles 3. Denuding of all mucosa around defect 4. Meticulous application of graft/ flaps 5. Multilayer closure for large defects (generally >10 to 15 mm) 6. Generally postoperative antibiotics (for about a week)

Closure Ladder • Onlay

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1. With meticulous preparation, the simplest closure is with the application of a graft, commonly a free mucosa graft over the defect. • Bath plug 1. The advantages of the bath plug are it conforms well to the shape of the defect, is resistant to infection, and is very adherent to bare bone. 2. After identifying the defect, mucosa and dural are removed until a definite bony rim is exposed. 3. There should be at least a 5 mm bony rim denuded of mucosa; this allows the free mucosa graft to adhere to the closure site. 4. Fat is harvested from the abdomen, ear lobule, or lateral thigh. 5. The fat plug needs to be about 3 times the width of the bony defect. 6. A Vicryl 4/0 is anchored to 1 end of the fat lump, then lanced through the lump to the other end, thus forming a bath plug with a plumb line. 7. This fat lump is then introduced and pushed into the defect with a ball probe. 8. The plumb line is then gently pulled to ensure that the fat plug is sitting well and forms a snug seal. 9. Reinforce the reconstruction with a free overlay mucosa graft tracked up along the suture. 10. The mucosa side must face the nasal cavity. 11. Fibrin glue is applied, then the Vicryl suture is cut. • Multilayer closure • Composite of underlay and onlay grafts (eg, bone or cartilage underlay; fascia lata or perichondrium onlay) 1. The bony or cartilaginous underlay provides a rigid structural support for the onlay graft to take. 2. Secondly, some believe a rigid graft provides a baffle/resistance against sudden rises in ICP (eg, with straining). • Nasoseptal flap 1. Described by Hadad and Bassagasteguy in 2006 2. Vascularized pedicled flap based on the posterior septal artery 3. This is a supple flap with both mucosa and periochondrium/ periosteum harvested from 1 side of the septum. 4. The anterior limit is the septal mucocutaneous junction. 5. The superior limit is 1 cm below the superior septal edge to spare the olfactory neuroepithelium; the inferiorolateral limit can extend to a variable width of the nasal cavity. 6. This is then raised posterior-laterally to where the posterior septal artery is, whereby the overlying mucosa is raised

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Cerebrospinal Fluid Leaks and Encephaloceles   461

between the inferior end of the sphenoid ostium to superior end of the bony choana. • Regional flaps 1. Example:  nasolabial flap raised and swung inwards • Free flaps 1. Example:  from distant donor site such as the free radial forearm flap • Allogenic versus autogenic grafts 1. No definite superiority in outcomes in various case series 2. The recommended graft is the one with which the surgeon is most familiar and confident.

Postoperative Care • 24 hours of complete bed rest • Bed elevated 30 degrees • Lumbar drain • No nose blowing, no straining • Stool softeners

References • Gendeh BS, Mazita A, Selladurai BM, Jegan T, Jeevanan J, Misiran K, et al. Endonasal endoscopic repair of anterior skull-base fistulas: the Kuala Lumpur experience. J. Laryngol & Oto. 2005;119: 866–74. • Lopatin AS, Kapitanov DN, Potapov AA. Endonasal endoscopic repair of spontaneous cerebrospinal fluid leaks. Arch Otolaryngol Head Neck Surg. 2003;129(8):859–863. • Schlosser RJ, Bolger WE. Nasal cerebrospinal fluid leaks. The Journal of Otolaryngology. 2002;S28–37.

Chapter

38 Pathology and Endoscopic Approaches to the Anterior Cranial Fossa Christina H. Fang and Waleed M. Abuzeid

Pathology of the Anterior Cranial Fossa . . . . . . . . . . 464 Clinical Features . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 464 Squamous Cell Carcinoma . . . . . . . . . . . . . . . . . . . . . . . . 464 Adenocarcinoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 464 Esthesioneuroblastoma . . . . . . . . . . . . . . . . . . . . . . . . . . . 465 Meningioma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 465 Sinonasal Undifferentiated Carcinoma . . . . . . . . . . . . . . 466 Sinonasal Neuroendocrine Carcinoma . . . . . . . . . . . . . . 467 Adenoid Cystic Carcinoma . . . . . . . . . . . . . . . . . . . . . . . . 467 Melanoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 467 Sarcoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 467 Other . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 468 Management of Anterior Cranial . . . . . . . . . . . . . . . . 468 Fossa Pathology Preoperative Management . . . . . . . . . . . . . . . . . . . . . . . . 468 Purely Endonasal Endoscopic Surgical Approach . . . . . 468

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Pathology of the Anterior Cranial Fossa Clinical Features • Prevalence:  3% to 5% of head and neck neoplasms • Sx:  nasal obstruction, epistaxis, headache, facial pain, sinusitis, hyposmia or anosmia, or asymptomatic; orbital invasion results in diplopia, vision loss, proptosis and/or epiphora • Non-specific symptoms usually lead to delay in diagnosis. • No universal staging system but most commonly use TNM staging • General prognostic factors:  tumor stage; tumor grade; intracranial, orbital, carotid involvement

Squamous Cell Carcinoma • Most common sinonasal malignancy (50% to 60%) • Arise most commonly from the maxillary antrum, followed by nasal cavity. • Can arise in association with inverted papillomas. • Male predominance; patients usually >50 years of age • Neck metastasis in ~10% to 20% of patients; distant metastasis in 10% • Histopathology:  large nests of tumor cells with pushing borders with little stromal response and central comedo necrosis; cells ovoid to spindle-shaped with indistinct cell borders and hyperchromatic nuclei that lack prominent nucleoli • Tx:  radical surgery followed by adjuvant radiotherapy to primary site; for locally advanced lesions (T3 to T4), radiotherapy to neck as well

Adenocarcinoma • Second most common sinonasal malignancy (~10% to 20%) • Arises most commonly from ethmoid sinuses (~85%). • Higher incidence in wood and leather workers; male predominance • It can be multifocal, therefore bilateral ethmoidectomies are recommended. • Histopathology:  mostly glandular or papillary patterns; compact acini, back-to-back glands and cystic dilatation; cells with uniform morphology with abundant cytoplasm, mild to moderate nuclear atypia; rare mitotic figures

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• Tx:  surgery adequate for early-stage low-grade lesions (T1 to T2); postoperative radiotherapy recommended for high-grade and advanced-stage (T3 to T4) lesions; elective neck dissection not routine because low risk of regional metastasis (F (5:3), affects all age ranges but peaks in 50s to 60s

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• Natural course:  slow growing, locally osseodestructive midline lesion; potential to metastasize via hematogeneous or lymphatic routes (10% to 20% of tumors) • Differential diagnosis: 1. Chondrosarcoma, 2. Ecchordosis physaliphora 3. Chordoid meningioma, 4. Fibrous dysplasia 5. Metastases (especially mucinous adenocarcinoma) • Symptomatology:  most commonly, headache and cranial nerve deficits (abducens nerve palsy most common), depending on structures involved; symptom range from endocrine dysfunction, stroke, epistaxis, ataxia, ptosis, hearing loss, vertigo, etc • Imaging:  high-resolution CT, MRI, CT angiogram 1. CT shows lytic well-circumscribed soft tissue lesion, hyperintense relative to brain, that moderately enhances after postcontrast. 2. MRI shows hyperintense lesion on T2 with heterogeneous enhancement postcontrast in a lobulated honeycomb pattern. 3. CT angiogram is best to delineate the relationship of major vessels within the tumor and surgical corridor. • Classification: 1. Type I:  limited to 1 compartment of the skull base (rare) 2. Type II:  involving 2+ contiguous compartments but requiring a single surgery (most common) 3. Type III:  involving multiple compartments and requiring 2+ surgeries • Patterns of involvement: 1. Upper clival (basisphenoidal) lesions often involve pituitary gland, hypothalamus, or CN III, V, or VI. 2. Lower clival (basioccipital) lesions often involve CN IX–XII • Treatment:  ideally includes an expanded endonasal approach (EEA) with gross total resection (GTR) and adjuvant proton-beam or hadron-beam radiotherapy 1. Contraindications to GTR include tumor involving brainstemperforating arteries, or lateral cavernous sinus. 2. EEA may need to be staged or combined with the open approach if the tumor extends laterally to the ICA, vertebral arteries, or optic nerves; inferiorly to the dens; or into the lateral condyle, mastoid bone, or lateral jugular tubercle. 3. These naturally radioresistant tumors require high doses of radiotherapy (standard regimen is 75 Gy in 1.8 Gy per day fractions).

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Pathology and Endoscopic Approaches to the Clivus . . .   489

• Histology: 1. Classic subtype (80% to 85%):  physaliphorous cells with lobular pattern of islands of eosinophilic vacuolated cells in basophilic mucoid stroma 2. Chondroid (5% to 15%):  low-grade tumor, mostly composed of chondrocytes and hyaline cartilage 3. Dedifferentiated (1% to 8%):  mesenchymal component with sarcomatoid appearance • Immunohistochemistry:  stains + for S-100, vimentin, cytokeratin, epithelial membrane antigen, CEA, MUC1, brachyury 1. Duplications in brachyury (transcription factor T gene) are unique to chordomas; when immunohistochemistry for brachyury is combined with cytokeratin, the sensitivity and specificity of diagnosis is 98% and 100%, respectively. 2. Absent or reduced expression of fragile histidine triad (FHIT) is found in two-thirds of chordomas. • Outcomes:  high local recurrence rate, overall median survival of 6.29 years; 5-year overall survival of 60% to 80%; 10-year overall survival of 32% to 50% 1. Chondroid subtype:  best prognosis with median survival of 15.8 years 2. Classic subtype:  median survival of 4.1 years

Chondrosarcoma • Etiology:  from degenerated chondroid cells in the petroclival synchondrosis • Epidemiology:  rare tumor accounting for 0.15% of intracranial tumors and 6% of skull base tumors; mean age of presentation, 40 years • Natural course:  slow-growing, locally invasive tumor that invades adjacent regions (cavernous sinus, jugular tubercle, cerebellopontine angle, jugular foramen, middle cranial fossa, hypoglossal canal, brainstem, cerebellum) but rarely transgresses dura • Symptomatology:  headache, cranial nerve deficits (VI and IX–XII) • Imaging:  high-resolution CT, MRI, CT angiogram 1. MRI shows low-to-intermediate signal on T1, and high signal on T2 and FLAIR. 2. CT shows stippled pattern of calcification; 50% of tumors show bone destruction and calcification. 3. CT angiogram is best to delineate the relationship of major vessels within the tumor and surgical corridor.

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• Treatment:  surgical resection (EEA is preferred but may require a combined approach) + adjuvant radiation for Grades 2 to 3 • Subtypes: 1. Conventional (most common) 2. Mesenchymal 3. Clear cell 4. Dedifferentiated • Myxoid chondrosarcoma may appear similar to chordomas on light microscopy. • Immunohistochemistry:  stains + for S-100 and vimentin but negative for cytokeratin, epithelial membrane antigen, CEA, MUC1, and brachyury • Outcomes:  better prognosis than for chordoma; 5-year overall survival of 82%, 10-year overall survival of 50%, survival higher with surgery + radiation than with surgery alone 1. Mesenchymal subtype associated with poorer prognosis

Plasmacytoma • Monoclonal plasma cell proliferation resulting in local destruction of bone • May be solitary plasmacytoma, extramedullary plasmacytoma, or associated with multiple myeloma • Imaging:  may suggest the diagnosis, although not specific 1. CT shows lytic lesion with narrow zone of transition and invasive outline. 2. MRI shows isointense lesion on T1 and hyperintense lesion on T2 with homogeneous contrast enhancement. • Treatment:  radiation for solitary lesions; consider endoscopic resection if gross total resection feasible

Lesions Invading Clivus • Metastases: 1. Malignancies that commonly spread to bone including lung, breast, prostate, renal, thyroid, and lymphoma 2. Most commonly present with abducens nerve palsy and headache • Local invasion:  pituitary macroadenoma, meningioma (clival, petroclival, chordoid), jugular foramen tumors, cerebellopontine angle tumors, craniopharyngioma, nasopharyngeal carcinoma, nasopharyngeal rhabdomyosarcoma, trigeminal schwannomas, cholesteatoma

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Pathology and Endoscopic Approaches to the Clivus . . .   491

• Infectious:  skull base osteomyelitis, malignant otitis externa, sphenoid sinusitis, allergic fungal rhinosinusitis

Congenital, Inflammatory, and Traumatic Disease Ecchordosis Physaliphora • Etiology:  ectopic notochord remnant located in the midline of the craniospinal axis occurring anywhere from the dorsum sellae to the coccyx • Epidemiology:  found in 2% of autopsies • Symptomatology:  often asymptomatic but may become symptomatic secondary to brainstem or cranial nerve compression • Classic appearance:  midline cystic lesion close to dorsum sella and protruding intradurally into prepontine cistern with a stalk-like median bony protuberance connecting to clivus • Imaging:  may suggest the diagnosis but is not specific 1. CT may show lytic lesion undistinguishable from chordoma. 2. MRI shows hyperintense T2 lesion without postcontrast enhancement (chordomas enhance with contrast). • Diagnosis usually requires histological analysis. • Can predispose to intracranial hemorrhage or CSF leak.

Fibrous Dysplasia • Etiology:  developmental anomaly characterized by replacement of mature bone by weak immature woven bone and fibrous tissue • Presentation during first 3 decades of life; symptoms dependent on site involved; 3% of all bone tumors • Monostotic disease:  more common (70%) but rarely involves clivus • Polyostotic disease:  possible involvement of clivus and other associations: 1. Café au lait macules 2. Endocrine disorders 3. McCune-Albright syndrome 4. Mazabraud syndrome • Symptomatology:  clival lesions present incidentally or with headache +/− cranial nerve deficits • Imaging:  usually sufficient to establish the diagnosis and stage the disease 1. MRI shows hypointense lesion on T1 and variably intense lesion on T2, depending on fibrous (hypointense) or cystic

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(hyperintense) nature; MRI findings may suggest an invasive tumor. 2. CT shows lesion with ground glass appearance and thinning of clival cortical bone; less common appearances are homogeneously dense and cystic lesions. • Histology:  shows three patterns—“Chinese writing,” pagetoid, or hypercellular • Treatment: 1. Most often includes observation 2. Occasionally may require a biopsy to establish diagnosis or rule out a sarcoma 3. Surgical resection customized, depending on symptoms – Partial removal to correct a cosmetic deformity or mass effect – Decompression to relief a neuropathy (eg, optic nerve or V2) – Possibly warranted complete resection for other fibroosseous lesions, such as ossifying fibroma

Neurenteric Cysts • Etiology:  benign endodermal lesion resulting from persistence of anterior portion of the transient neurenteric canal (connects endodermal foregut and ectodermal notochord during third week of development) • Spine 3× more common than intracranially; intracranial lesions most common in posterior fossa • Often associated with split notochord syndrome (vertebral anomalies) • Symptomatology: 1. Inconsistent and dependent upon location 2. Common symptoms include headache, cranial nerve deficits, seizure, meningitis, or behavioral changes • Differential diagnosis includes sphenoid sinus mucocele, dermoid cyst, epidermoid cyst, colloid cyst, Rathke’s cleft cyst, arachnoid cyst, choroidal epithelial cyst, and ependymal cyst. • Imaging:  suggests diagnosis in most cases 1. MRI is variable but usually shows hyperintense lesion on T1 and FLAIR, with variable intensity on T2 (depending on cyst contents). 2. CT shows osteolytic cystic lesion with intact clival cortical bone. • Histology:  unilocular smooth thin-walled cyst containing material ranging from clear serous fluid to colloid gelatinous material with cuboidal to pseudostratified columnar epithelium

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Pathology and Endoscopic Approaches to the Clivus . . .   493

Sphenoid Sinus Mucocele • Etiology:  benign, expansile, locally destructive cystic lesion of the sphenoid sinus due to accumulation of mucus because of ostium obstruction 1. May result from congenital structural abnormalities, trauma, sinusitis, nasal polyposis, or surgery. 2. Expansion may occur into nasal cavity, nasopharynx, pituitary fossa, suprasellar region, clivus, orbits, or ethmoid sinuses. • Epidemiology:  most common in third and fourth decades of life; accounts for 2 to 3 mm above Chamberlain’s line.

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Pathology and Endoscopic Approaches to the Clivus . . .   495

• Treatment: 1. Conservative management with physical therapy +/− cervical collar if no compressive symptoms 2. Surgical decompression if refractory to medical management or with neurological deficit

Surgical Approaches to the Clivus and Posterior Fossa Endoscopic Endonasal Approaches • Endoscopic endonasal approaches (EEA) were developed to reduce morbidity associated with pharyngeal incisions (prolonged intubation, tracheotomy, gastrostomy, velopharyngeal insufficiency, retropharyngeal infections). • Advantages: 1. No cerebral retraction 2. Does not cross cranial nerve planes 3. Allows early tumor debulking 4. Better lighting and visualization 5. Allows access to contralateral side 6. Decreased morbidity compared to open surgical approaches – Preserved palatal function – Decreased postoperative cranial nerve deficits (inside-out approach) – Less local disease recurrence (operator dependent) • Disadvantages: 1. Limited access to lesions with lateral and inferior extension 2. Higher CSF leak rates (before development of nasoseptal flap) 3. Contamination from paranasal sinuses 4. Steep learning curve • Contraindications: 1. Active sinonasal infection 2. Lesions lateral to lower cranial nerves • Preoperative care:  nasal swab for MRSA culture followed by nasal application of povidone-iodine; if negative for MRSA, cefepime; if positive for MRSA, cefepime + vancomycin • Midline clival lesions without lateral or posterior extension are resected via a transsphenoidal approach. • Clival lesions with posterosuperior extension are resected by a pituitary transposition/transdorsal posterior clinoid approach. • Clival lesions with lateral extension are resected through a transsphenoidal + transpterygoidal approach +/− endoscopic

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medial maxillectomy +/− modified endoscopic Denker’s (extension of medial maxillectomy anteriorly to remove portion of pyriform aperture and ascending process of maxilla). • Clival lesions with inferior extension are resected via several approaches: transnasal retropharyngeal approach, transnasaltranspalatal approach, transnasal-transoral approach. • In general, the principle of intracapsular debulking followed by extracapsular dissection should be followed during tumor removal. • If dura is not violated, a vascularized pedicled flap is not necessary for reconstruction but may be prudent if the ICAs have been exposed. • Reconstruction: 1. Nasoseptal flap +/− abdominal fat graft + absorbable hemostatic agents + Doyle splints 2. Tunneled temporoparietal fascia is an alternative if unable to harvest a nasoseptal flap • Complications: 1. CSF leak (most common) 2. Vascular injury (compress w/muscle graft from thigh, then take for angiography) 3. Cranial nerve injury (abducens nerve most common) 4. Meningitis • Postoperative care:  antibiotics until nasal packing removed (usually on postoperative day 5), non-contrast head CT immediately after surgery, MRI within first 24 hours, sinus precautions, stool softeners

Transnasal Transsphenoidal Approaches • Initial approach is similar for all approaches and creates wide bilateral exposure of sellar protuberance, clival recess, ICA, optic canal, and opticocarotid recess. • Procedure Details 1. Outfracture inferior turbinates 2. Partially or fully remove middle turbinate on ipsilateral side relative to lesion 3. Harvest contralateral nasoseptal flap based on posterior nasal artery 4. Perforate posterior nasal septum near sphenoid sinus rostrum then resect posterior 2 cm down to hard palate 5. Perform ipsilateral or bilateral posterior ethmoidectomies 6. Create wide anterior sphenoidotomy and reflect mucosa off of clivus 7. Drill skull base to remove involved bone and expose/remove lesion

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Pathology and Endoscopic Approaches to the Clivus . . .   497

• Skull base approaches are classified into approaches to the superior, middle, and inferior clivus, but these approaches are often combined during a single surgery.

Endonasal Transdorsal Posterior Clinoid Approach (aka Pituitary Transposition Approach) • Provides access to lesions involving the superior third of the clivus and interpeduncular cistern • Procedure Details 1. Remove bone covering sella to expose superior (SIS) and inferior (IIS) intercavernous sinuses 2. Ligate SIS then open sellar diaphgram to expose the pituitary stalk 3. Cut ligaments connecting pituitary capsule to medial cavernous sinus 4. Mobilize pituitary gland superiorly, then open posterior sellar diaphragm and transect posterior intercavernous sinus to expose dorsum sella and posterior clinoid processes 5. Drill dorsum sellae and posterior clinoid processes until eggshell thickness then carefully remove 6. Identify basilar plexus then proceed through it while controlling bleeding with warm water irrigation and hemostatic agents 7. Identify margins of lesion and remove • At-risk structures:  paraclinoidal and intracavernous ICA, superior hypophyseal artery, cavernous sinus, oculomotor nerve, basilar artery, superior cerebellar artery, posterior cerebral artery • Considerations:  suprasellar tumors may be firmly attached to neurovascular structures or extend into cavernous sinus or floor of third ventricle, lesions lateral to oculomotor nerve should be addressed through traditional open approach or staged/combined endoscopic + open approach

Transclival Recess Approach • Provides access to lesions involving the middle third of the clivus and prepontine cistern • Usually performed in combination with approach to lower third of clivus or a panclivectomy • Procedure Details 1. Drill clival recess bone to expose dura and basilar plexus 2. Identify vidian nerve and trace posteriorly to anterior genu of ICA at foramen lacerum

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3. Drill paraclival ICA canals if necessary 4. Open dura in midline at level of basion and identify abducens nerve 5. Open dura superiorly to expose prepontine cistern 6. Identify margins of lesion and remove • At-risk structures:  basilar artery, paraclival and lacerum ICA, anterior inferior cerebellar artery (AICA), abducens nerve, ventral pons • Considerations:  lateral extension of a lesion requires paramedian EEA techniques (see below)

Transnasal Retropharyngeal Approach • Provides access to lesions involving the inferior third of clivus, C1, or upper C2 • Limits of dissection include sphenoid sinus superiorly, soft palate inferiorly and eustachian tubes laterally • Procedure Details 1. Incise nasopharyngeal mucosa and superior constrictor muscle in the midline then retract laterally to expose basopharyngeal fascia 2. Strip basopharyngeal fascia to expose sphenoid floor and ventral clivus 3. Drill sphenoid sinus floor until flush with clivus to create wide communication between sphenoid sinuses and nasopharynx 4. Detach longus capitis (lower clivus), longus colli (upper cervical spine), and anterior atlanto-occipital membranes in midline and lateralize to provide access to lower clivus, anterior C1 vertebra, and odontoid process 5. Drill clivus posteriorly, between lacerum segments of ICA superiorly and occipital condyles inferiorly, toward foramen magnum as indicated to expose clival dura 6. If needed, coagulate dura in midline and control basilar plexus bleeding with gentle pressure and hemostatic agents 7. Identify vertebral arteries then remove lesion as necessary 8. If lesion extends lateral to occipital condyle, remove the rectus capitus anterior and the atlanto-occipital joint capsule to expose the atlanto-occipital joint 9. Remove anteromedial portion of jugular condyle as necessary (up to 75%) while leaving the alar ligament intact • At-risk structures:  vertebral artery, hypoglossal nerve, vidian nerve and artery, parapharyngeal ICA

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Pathology and Endoscopic Approaches to the Clivus . . .   499

• Considerations:  lateral extension of a lesion may require paramedian EEA techniques (see below), nasoseptal flap (if required) must be tucked into maxillary sinus until needed for reconstruction, occipitocervical fusion may be necessary if cervical instability, can be combined with transoral or transpalatal routes if inferior extension (see below)

Expanded Endoscopic Endonasal Approach • An expanded endoscopic endonasal approach (EEEA) may be performed to gain access to a midline clival lesion with inferior extension in the sagittal plane or with lateral extension in the coronal plane. • Access to the complete upper cervical spine can be obtained by combining the transnasal approach with a transoral or transpalatal approach. • The coronal or paramedian plane is the region lateral to the ICA along its course across the ventral skull base.

Expanded Endoscopic Endonasal Approaches to the Craniovertebral Junction • EEA to anterior CVJ can be utilized for resection of neoplasms, rheumatoid arthritis pannus, and decompression of the upper cervical spine. • Caudal limit of CVJ exposure by EEA is identified by extrapolating the nasopalatine line (NPL), which is from the inferior nasal bones anteriorly to the hard palate posteriorly. • The odontoid process is always within the surgical exposure provided by EEA. • Exposure to below the level of the oropharynx (C2 to C3 vertebrae) can be attained by a combined endonasal + transoral (endoscopic, microscopic, or robotic) approach. • Advantages:  avoid splitting of soft palate musculature, earlier extubation and postoperative enteral intake, provides access in cases with limited jaw mobility, reduced risk of velopharyngeal insufficiency (VPI), decreased need for tracheotomy • Disadvantages:  inability to primarily close the posterior pharyngeal wall • Transoral robotic surgery (TORS) may be used but is limited by the lack of drilling equipment, bulky instrumentation, and inability to extend below the level of the carotid bifurcation.

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Endoscopic Odontoidectomy • Procedure Details 1. See steps 1 through 4 in “Transnasal Retropharyngeal Approach” above for initial approach 2. After exposing inferior clivus, anterior arch of C1 and odontoid process, drill anterior arch of C1 vertebra to gain access to odontoid process 3. Core the odontoid process to remove the inner portions while maintaining the outer cortex 4. Thin the cap of the odontoid process, then remove to expose the premedullary dura • Considerations:  posterior occipitocervical fusion often necessary, VPI may develop if nasopharyngeal incision is made below the level of the soft palate

Paramedian Expanded Endoscopic Endonasal Approach • Paramedian EEEA allows access to clival lesions with extension into the middle and posterior coronal planes. • Following a systematic modular approach, after performing an initial transsphenoidal approach, provides access to seven different zones within the clivus and posterior fossa. • Creating a transpterygoid corridor is the initial step in gaining endonasal access to zones 2 through 7. • Harvest a nasoseptal flap on the side contralateral to the approach.

Transnasal Transpterygoid Approach • Procedure Details 1. Perform uncinectomy, anterior ethmoidectomy, and wide maxillary antrostomy 2. Identify sphenopalatine and posterior nasal arteries at sphenopalatine foramen, then coagulate them. 3. Visualize infraorbital canal, then remove the posterior wall of the maxillary sinus to expose the pterygopalatine fossa. 4. Sacrifice the pharyngeal artery (branch of internal maxillary artery) and nerve running through the palatovaginal canal. 5. Retract the soft tissues of the pterygopalatine fossa laterally to identify the vidian nerve. 6. Trace the vidian nerve posteriorly to identify the ICA genu, vidian foramen, and foramen rotundum in the sphenoid bone.

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Pathology and Endoscopic Approaches to the Clivus . . .   501

7. Transpose (preferred) or transect the contents of the vidian canal. 8. Drill the base of the pterygoid plate inferomedially to the vidian canal to open the lateral sphenoid recess and approach anterior genu of the ICA. 9. Extend approach as necessary to reach lesions extending posterior to ICA. • At-risk structures:  infraorbital nerve, orbit, internal maxillary artery (IMA), pterygopalatine ganglion, petrous and lacerum segments of ICA, vidian nerve and artery • Considerations:  drilling limited superiorly by horizontal petrous ICA, inferiorly by eustachian tube, laterally by petrous pyramid, and medially by sphenoid floor; bleeding from pterygopalatine venous plexus can be controlled with bipolar electrocautery

Medial Petrous Apex Approach (Zone 1) • Provides access the anterior petrous apex posterior to the paraclival ICA • Common pathologies include cholesterol granuloma and chondrosarcoma. • Procedure Details 1. Perform standard transsphenoidal approach (see above). 2. Remove basopharyngeal fascia from undersurface of sphenoid sinus floor. 3. Drill sphenoid sinus floor posteriorly until flush with the clival recess to identify area of foramen lacerum (by following vidian nerve posteriorly). 4. Remove bone medial and lateral to lacerum segment of ICA to enhance mobilization of ICA. 5. Enter medial petrous apex and remove lesion as indicated. • At-risk structures:  vidian nerve and artery, lacerum segment of ICA, abducens nerve • Considerations:  this approach only exposes medial petrous apex

Petroclival Approach (Zone 2) • Provides access to petrous body inferior to petrous ICA • Common pathologies include chordoma and chondrosarcoma. • Procedure Details 1. Perform zone 1 approach. 2. Follow vidian nerve and artery posteriorly to the lacerum segment of ICA. 3. Completely expose lacerum segment of ICA.

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4. Disconnect eustachian tube cartilage from foramen lacerum using horizontal cuts. 5. Retract eustachian tube inferiorly to visualize petrous apex. 6. If necessary, completely remove eustachian tube to fully expose petroclival synchondrosis. • At-risk structures:  vidian nerve and artery, lacerum segment of ICA, abducens nerve, eustachian tube

Suprapetrous Approach (Zone 3) • Provides access to petrous body superior to petrous ICA and lateral to paraclival ICA • Indicated to remove lesions in Meckel’s cave • Common pathologies include meningioma, schwannoma, pituitary adenoma, and adenoid cystic carcinoma. • Procedure Details 1. Perform transsphenoidal and transpterygoid approaches. 2. Follow vidian nerve and artery to vidian canal. 3. Drill bone separating maxillary branch of trigeminal nerve (V2) and vidian canal to expose the petrous, lacerum, and paraclival segments of the ICA. 4. Drill bone of middle fossa floor to expose the periosteal dura mater. 5. Open dura in medial to lateral fashion from anterior genu of ICA toward V2 and enter the quadrangular space. • At-risk structures:  vidian nerve and artery, ICA, cavernous sinus, Gasserion ganglion, abducens nerve, orbital apex, optic canal, oculomotor nerve • Considerations:  when opening dura, stay below abducens nerve and lateral to ICA to avoid damage to superior cavernous sinus • Quadrangular space boundaries:  V2 laterally, intracavernous abducens nerve superiorly, horizontal petrous ICA inferiorly, and paraclival ICA medially

Lateral Cavernous Sinus Approach (Zone 4) • Provides access to superior cavernous sinus and above Meckel cave • Only indicated in patients with pre-existing cranial nerve deficits • Procedure Details 1. Perform zone 3 approach. 2. Extend dural opening superior to quadrangular space to enter lateral cavernous sinus. 3. Incise periosteum on the superior orbital fissure and cavernous ICA at the level of the siphon.

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4. Dissect lateral to the ICA as necessary. • At-risk structures:  same as zone 3, trochlear nerve, first division of trigeminal nerve • Considerations:  if tumor or cavernous sinus thrombosis present, minimal bleeding will be encountered until lesion is removed, then copious bleeding occurs

Middle Fossa/Infratemporal Fossa Approach (Zone 5) • Provides access lateral to petrous ICA and anterior to parapharyngeal ICA with ability to directly access the infratemporal fossa and middle cranial fossa • Common pathologies include invasive sinonasal malignancies, nasopharyngeal carcinoma, CSF leaks, encephaloceles, schwannomas, and meningiomas. • Procedure Details 1. Perform transsphenoidal and transpterygoid approach; soft tissues of pterygopalatine fossa may be retracted medially or laterally depending on desired exposure. 2. If full exposure of infratemporal fossa is necessary, control IMA and its branches to facilitate dissection. 3. Drill pterygoid process as necessary. 4. To reach infratemporal fossa, dissect lateral to lateral pterygoid plate and identify mandibular branch of trigeminal nerve (V3) immediately posterior to the superior aspect of the lateral pterygoid plate. 5. To reach middle cranial fossa, drill pterygoid base superiorly while preserving vidian nerve and V2, then drill lateral wall of lateral sphenoid recess and incise periosteal dura mater in between V1 and V2 to enter anteromedial triangle of middle cranial fossa. • At-risk structures:  paraclival and lacerum segments of ICA, vidian nerve and artery, branches of trigeminal nerve, IMA

Occipital Transcondylar Approach (Zone 6) • Provides access posterior to eustachian tube and medial to parapharyngeal ICA with the ability to access the hypoglossal canal • Common pathologies include chordoma, paraganglioma, schwannoma, nasopharyngeal carcinoma, and meningioma. • Procedure Details 1. Perform transsphenoidal and transpterygoid approach. 2. Dissect superiorly to petroclival synchondrosis and laterally to occipital condyle to identify cartilaginous and bony portions

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of eustachian tube and parapharyngeal and carotid canal segments of the ICA. 3. If necessary, drill supracondylar area to access jugular tubercle. • At-risk structures:  parapharyngeal and lacerum segments of ICA, vidian nerve and artery, abducens nerve, eustachian tube, hypoglossal nerve • Considerations:  hypoglossal nerve exits hypoglossal canal inferolaterally from the occipital condyle, jugular tubercle is immediately medial to inferior petrosal sinus and jugular foramen

Jugular Foramen Approach (Zone 7) • Provides access posterior and lateral to parapharyngeal ICA • Common pathologies include paraganglioma, invasive carinoma, schwannoma, and meningioma. • Procedure Details 1. Perform occipital transcondylar approach. 2. Remove eustachian tube to provide access to parapharyngeal ICA and petroclival synchondrosis. 3. Dissect through soft tissues lateral to eustachian tube to fully expose ICA. 4. Identify jugular foramen lateral and posterior to parapharyngeal ICA. • At-risk structures:  same as zone 6, internal jugular vein, CN IX, X, XI • Considerations:  Doppler acoustic US may be helpful during dissection to avoid inadvertent ICA injury; endoscopic Denker approach may be necessary

Chapter

41 Endoscopic Skull Base Reconstruction Elisabeth H. Ference and Jeffrey D. Suh

Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 506 Goals of Surgery . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 506 Key Factors to Consider when Reconstructing . . . . . . . 506 A Multilayer Closure Allows for Re-Establishment of Tissue Barriers . . . . . . . . . . . . . 507 Predictors of Successful Reconstruction . . . . . . . . . . . . . 507

Preoperative Evaluation . . . . . . . . . . . . . . . . . . . . . . . . . . 507 Obtain a Detailed History from the Patient . . . . . . . . . . 507 Perform a Detailed Endoscopic Exam . . . . . . . . . . . . . . . 508 Radiographic Considerations . . . . . . . . . . . . . . . . . . . . . . 508 Pearls and Potential Pitfalls . . . . . . . . . . . . . . . . . . . . . . 508 Free Grafts . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 509 Autologous Grafts . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 509 Heterologous Grafts . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 511 Local Pedicled Flaps . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 512 Nasoseptal Flap (aka Hadad-Bassagasteguy Flap) . . . . . 512 Inferior Turbinate Flap . . . . . . . . . . . . . . . . . . . . . . . . . . . 516 Middle Turbinate Flap . . . . . . . . . . . . . . . . . . . . . . . . . . . . 517 Anteriorly Based Pedicled Flaps . . . . . . . . . . . . . . . . . . 518 Extranasal Pedicled Flaps . . . . . . . . . . . . . . . . . . . . . . . . 518 Temporoparietal Fascial Flap . . . . . . . . . . . . . . . . . . . . . . 519 Pedicled Palatal Mucosal Flap . . . . . . . . . . . . . . . . . . . . . . 519 Occipital Galeopericranial Flap . . . . . . . . . . . . . . . . . . . . 520 505

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Intraoperative Care . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 520 Flap Handling . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 520 Bolstering . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 520 Lumbar Drains . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 521 Instructions and Debridements . . . . . . . . . . . . . . . . . . 521

Introduction • Reconstruction of the skull base after transnasal endoscopic surgery remains a major challenge, particularly when dural defects are large or in patients who have had prior surgery or radiation. • Intradural tumor surgery can be divided into two main groups: 1. Intradural but extra arachnoidal, ie, pituitary surgery when the diaphragm is not violated 2. Intra-arachnoidal where, by definition, an intraoperative CSF leak is present 100% of the time – Intra-arachnoidal surgery can be further divided into highflow and low-flow leaks, depending on whether a cistern was directly opened into the sinonasal defect.

Goals of Surgery • Defect coverage to facilitate healing • Watertight and airtight separation for avoidance of postoperative leak and pneumocephalus • Prevention of intracranial infection by separation of the cranial cavity from the sinonasal tract • Elimination of dead space • Preservation of neurovascular and ocular function

Key Factors to Consider when Reconstructing • Availability of tissue/materials for reconstruction • Location of the defect • Presence and flow rate of CSF leak • Prior or future radiation therapy • Other important factors include obesity and states of poor healing (eg, Cushing’s disease or diabetes)

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A Multilayer Closure Allows for Re-Establishment of Tissue Barriers • Underlay materials are subdural to form a watertight layer; a mucosal flap is an onlay flap to provide vascularization. • Multilayer closure allows for excellent long-term prevention of intracranial complications, with perioperative complication rates