Color Atlas of Ophthalmology PDF - PDFCOFFEE.COM (2024)

Color Atlas of Ophthalmology The Quick-Reference Manual for Diagnosis and Treatm ent Second Edit ion

Color Atlas of Ophthalmology The Quick-Reference Manual for Diagnosis and Treatm ent Second Edit ion Amar Agarw al, MS, FRCS, FRCOphth Professor of Ophthalmology Ram achandra Medical College Private Practice Chennai, India

Soosan Jacob, MS, FRCS, DNB, MNAMS Private Practice Chennai, India

Th iem e New York • St u t tgar t

Thiem e Medical Publishers, Inc. 333 Seventh Ave. New York, NY 10001 Editorial Director: Michael Wachinger Managing Editor: J. Owen Zurhellen IV Editorial Assistant: Dom inik Pucek Vice President, Production and Electronic Publishing: Anne T. Vinnicombe Production Editor: Kenneth L. Chumbley, Publication Services Vice President, International Marketing and Sales: Cornelia Schulze Chief Financial Officer: Peter van Woerden President: Brian D. Scanlan Cover illustration drawn by Karl Wesker Compositor: MPS Content Services Printer: Gopsons Papers Ltd. Library of Congress Cataloging -in-Publication Data Color atlas of ophthalm ology : the quick-reference m anual for diagnosis and treatm ent / [edited by] Am ar Agarwal, Soosan Jacob.—2nd ed. p. ; cm. Includes index. Prev. ed. has subtitle: Manhat tan Eye, Ear & Throat Hospital pocket guide. ISBN 978-1-60406-211-3 (alk. paper) 1. Eye—Diseases—Atlases. 2. Eye—Diseases—Handbooks, manuals, etc. 3. Ophthalm ology—Atlases. 4. Ophthalm ology—Handbooks, m anuals, etc. I. Agarwal, Am ar. II. Jacob, Soosan. [DNLM: 1. Eye Diseases—diagnosis—Atlases. 2. Eye Diseases—diagnosis—Handbooks. 3. Eye Diseases—pathology—Atlases. 4. Eye Diseases—pathology—Handbooks. 5. Eye— pathology—Atlases. 6. Eye—pathology—Handbooks. 7. Opthalmologic Surgical Procedures— Atlases. 8. Ophthalmologic Surgical Procedures—Handbooks. WW 17 C7195 2009] RE71.C65 2009 617.70022'3—dc22 2009020093 Copyright © 2010 by Thiem e Medical Publishers, Inc. This book, including all part s thereof, is legally protected by copyright. Any use, exploitation, or com m ercialization out side the narrow lim its set by copyright legislation without the publisher’s consent is illegal and liable to prosecution. This applies in particular to photostat reproduction, copying, m im eographing or duplication of any kind, translating, preparation of m icrofilm s, and electronic data processing and storage. Important note : Medical knowledge is ever-changing. As new research and clinical experience broaden our knowledge, changes in treatm ent and drug therapy m ay be required. The authors and editors of the m aterial herein have consulted sources believed to be reliable in their effort s to provide information that is complete and in accord with the standards accepted at the time of publication. However, in view of the possibilit y of hum an error by the authors, editors, or publisher of the work herein or changes in m edical knowledge, neither the authors, editors, nor publisher, nor any other part y who has been involved in the preparation of this work, warrants that the inform ation contained herein is in every respect accurate or complete, and they are not responsible for any errors or omissions or for the results obtained from use of such inform ation. Readers are encouraged to confirm the information contained herein with other sources. For example, readers are advised to check the product inform ation sheet included in the package of each drug they plan to adm inister to be certain that the inform ation contained in this publication is accurate and that changes have not been m ade in the recom m ended dose or in the contraindications for adm inistration. This recom m endation is of particular importance in connection with new or infrequently used drugs. Som e of the product names, patents, and registered designs referred to in this book are in fact registered tradem arks or proprietary nam es even though specific reference to this fact is not always m ade in the text. Therefore, the appearance of a nam e without designation as proprietary is not to be construed as a representation by the publisher that it is in the public domain. Printed in India 54321 ISBN 978-1-60406-211-3

Noth ing in th is w orld m oves w ith out Him , an d so also th is book w as on ly w rit ten by Him .

Th is book is dedicated to a great frien d, Brian S. Boxer Wach ler, MD

Contents Fo rew o rd . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .ix Preface . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .xi Abo ut the Edito rs. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . xiii Co ntributo rs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . xv Chapter 1

Ocu lar Traum a . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1 Daniele Veritt i, Carlo Sborgia, Francesco Boscia, Giuseppe Sm aldone, and Paolo Lanzetta

Chapter 2

Eyelids an d Lacrim al System . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 33 Christopher I. Zoum alan, Andrea Olm os, and Kim berly P. Cockerham

Chapter 3

Orbit al In fect ion s, In am m at ion , an d Neoplasm s . . . . . . . . . . . . . . . . 69 Praveen Saluja, Sw at i Ravani, Soosan Jacob, and Am ar Agarw al

Chapter 4

Extern al Diseases . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 100 Guillerm o Sim ón-Castellví, Pablo Gili-Manzanaro, Sarabel Sim ón-Castellví, José María Sim ón-Castellví, Crist ina Sim ón-Castellví, and José María Sim ón-Tor

Chapter 5

Corn ea . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 141 W . Barry Lee and Ivan R. Schw ab

Chapter 6

In t raocu lar In am m at ion . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 194 Soosan Jacob, Dhivya Ashok Kum ar, Athiya Agarw al, and Am ar Agarw al

Chapter 7

Len s an d Cataract . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 218 Athiya Agarw al, Soosan Jacob, and Am ar Agarw al

Chapter 8

Glaucom a . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 244 Soosan Jacob and Am ar Agarw al

Chapter 9

Medical Ret in a . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 260 Mandeep Lam ba, Soosan Jacob, and Am ar Agarw al

Chapter 10 Su rgical Ret in a . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 288 Clem ent K. Chan and Dariusz G. Tarasew icz Chapter 11 Ocular Neoplasm s. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 322 Soosan Jacob, Santosh Hanovar, and Am ar Agarw al Chapter 12 St rabism u s . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 339 Federico G. Velez, Noa Ela-Dalm an, and Arthur L. Rosenbaum Chapter 13 Neuro- Op h th alm ology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 359 Soosan Jacob and Am ar Agarw al

vii

viii

Content s

Chapter 14 Oph th alm ic Ph arm acology. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 405 Jam es M. Hill, Lori Vidal Denham , Blake A. Booth, Duncan A. Friedm an, Je ery A. Hobden, Andrea T. Murina, Marie D. Acierno, Jean T. Jacob, Herbert E. Kaufm an, and Donald R. Bergsm a Chapter 15 Ocu lar Man ifest at ion s of System ic Disease . . . . . . . . . . . . . . . . . . . . . 430 Soosan Jacob and Am ar Agarw al Chapter 16 Con tact Len ses . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 447 Kenneth M. Daniels Chapter 17 Oph th alm ic In st r um en t s an d Diagn ost ic Tests . . . . . . . . . . . . . . . . . . 486 Sam uel Boyd and Am ar Agarw al Chapter 18 Oph th alm ic O ce Procedures. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 507 Sam uel Boyd Chapter 19 Refract ive Su rger y . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 518 David R. Hardten, Natalia Kram arevsk y, Louis E. Probst, and Richard L. Lindst rom Index . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 539

Forew ord For som e au th ors of oph th alm ology, w rit ing or edit ing 50 book ch apters is a sizeable accom plish m en t over a lifet im e, let alon e a decade. Th is is n ot t ru e for Professor Agar w al. Sin ce 1998, Am ar Agar w al h as edited on average m ore th an fou r books a year, w h ich is an u npreceden ted an d virt u ally u n im agin able yearly n u m ber for any on e person . His books sp an topics from diagn ost ics an d im aging (su ch as aberrom et r y, angiography, topography, dr y eyes, and neuro-ophthalm ology) to therapeutics and surger y (including contact len ses, lasers, LASIK, strabism us, oculoplastics, cataracts, phako, an d m ore), an d m any h ave been bestsellers. He h as au th ored several com prehensive handbooks and textbooks of oph th alm ology, an d Color Atlas of Ophthalm ology stan ds out as on e of h is best . In it s 19 su bspecialt y ch apters, th is secon d edit ion illust rates over 500 diagn ost ics an d th erapeut ic con dit ion s. Th e com preh en sive an d system at ic form at h elp s th e reader fin d an d view th e facts abou t any con dit ion in oph th alm ology in st an tly, m aking it an essen t ial resou rce for both th e st u den t an d th e pract it ion er in th e field. Anyon e w h o kn ow s Professor Agar w al can tell you th at h e is a con sum m ate in n ovator an d teach er. As a professor of oph th alm ology an d in tern at ion ally ren ow n ed lect urer, h is exp erien ce an d dedicat ion to teach ing speak for th em selves. Over th e years, I h ave p ar t icip ated in several Am erican Academ y of Oph th alm ology an d Am erican Societ y of Cataract an d Refract ive Su rger y cou rses w ith Professor Agar w al an d h ave learn ed a great deal about m icroin cision cataract su rger y an d th e m an agem en t of ch allenging LASIK cases from th em . Person ally, I look for w ard to review ing an d referring to th e con ten t of th is book on a regu lar basis. I recom m en d th at you do so as w ell! Ronald R. Krueger, MD, MSE Medical Director, Depar t m en t of Refract ive Su rger y Cole Eye In st it ute Clevelan d Clin ic Clevelan d, Oh io

ix

Preface Every day you m ay m ake progress. Every step m ay be fruitful. Yet there w ill stretch out before you an ever-lengthening, ever-ascending, ever-im proving path. You k now you w ill never get to the end of the journey. But this, so far from discouraging, only adds to the joy and glory of the clim b. Win ston Ch u rch ill (1874–1965) W h at is an oph th alm ologist? On e w h o h as gradu ated from m edical sch ool an d is in terested in th e eye. On e w h o h as com pleted th ree years of sp ecialized oph th alm ic t rain ing. On e w h o h as sp en t a fur th er t w o years in sub-specializat ion . On e w h o h as m en tored th e n ext gen erat ion of oph th alm ologists. On e w h o h as in n ovated an d ch anged preferred pract ice p at tern s in oph th alm ology. All th ese classes of oph th alm ologists n eed solid kn ow ledge com bin ed w ith pat ien t em pathy to best ser ve p at ien t s. Th e purpose of th is fu lly revised secon d edit ion of th e su ccessful Color Atlas of Ophthalm ology: The Quick-Reference Manual for Diagnosis and Treatm ent is to give you th e kn ow ledge n eeded to face w ith con fiden ce th e fu ll range of pat ien ts’ op h th alm ic disorders. In ou r field, w h ere w e n ecessarily learn , diagn ose, an d teach visu ally, top -fligh t clin ical p h otograph s, like th e h u n dreds in th is book (all n ew to th is edit ion ), are essen t ial. Th e n in eteen ch apters, w rit ten by expert con t ribu tors from North Am erica, Eu rope, an d In dia, are w ell-organ ized an d con cise, an d th e form at of “Presen tat ion -Differen t ial Diagn osis-Man agem en t” is design ed for easy an d speedy referen ce, so you don’t h ave to w ade th rough ext ran eou s m aterial to fin d w h at you n eed. Th is pocket atlas is in ten ded for th e residen t on rou n ds or st udying for an exam , th e pract icing oph th alm ologist w h o n eeds con firm at ion of an un usual fin ding, or th e prim ar y physician w h o requ ires a reliable guide to eye disorders. We are gratefu l to th e people at Th iem e Pu blish ers, especially to J. Ow en Zurh ellen an d Dom in ik Pu cek. We an d Th iem e h ope an d expect th at ou r pocket atlas aids you in p roviding pat ien t s w ith th e best care possible. Am ar Agarw al, MS, FRCS, FRCOphth Soosan Jacob, MS, FRCS, DNB, MNAMS Ch en n ai, In dia

xi

About the Editors Amar Agarw al, MS, FRCS, FRCOphth Dr. Agar w al’s Grou p of Eye Hospitals an d Eye Research Cen t re Ch en n ai, In dia Prof. Am ar Agar w al is th e p ion eer of ph akon it , w h ich is ph ako w ith n eedle in cision tech n ology. Th is tech n ique becam e popularized as bim an u al ph aco, m icroin cision cataract surger y (MICS), or m icroph aco. He is th e first to rem ove cat aracts th rough a 0.7 m m t ip w ith th e tech n ique called m icroph akon it . He is also th e first to h ave discovered an d developed cataract su rger y w ith out an esth esia. An oth er of h is in n ovat ion s is FAVIT (fallen vit reous), a n ew tech n ique for rem oving dropped n uclei. Th e air pu m p, based on th e sim ple idea of using an aqu ariu m pum p to in crease th e flu id in fusion in to th e eye in bim an u al ph aco an d coaxial ph aco, h as h elped preven t u n st able an terior ch am ber an d su rge du ring cataract su rger y. Th is provided th e basis for variou s tech n iqu es of forced in fu sion for sm all in cision cat aract surger y. Dr. Agar w al w as also th e first to use t r ypan blu e for stain ing ep iret in al m em bran es an d p ublish ed th e det ails in h is four-volu m e textbook of op h th alm ology. He h as also discovered a n ew refract ive error called aberropia. He h as been th e first to do a com bin ed su rger y of m icroph akon it (700 µm cataract surger y) w ith a 25-gauge vit rectom y in th e sam e pat ien t , th u s u sing th e sm allest in cision s possible for cat aract an d vit rectom y. An d h e is th e first surgeon to im plan t a n ew m irror telescopic in t raocu lar len s (IOL) Lipsh it z Macu lar Im plan t (LMI) for pat ien ts suffering from age-related m acular degen erat ion . Th e Malyugin ring for sm all pu pil cat aract surger y w as also m odified by h im an d is kn ow n as th e Agar w al m odificat ion of th e Malyugin ring for m iot ic pu pil cat aract surgeries w ith posterior capsular defect s. He h as been th e w orld’s first to im plan t a glu ed IOL. In th is procedure a posterior ch am ber (PC) IOL is fixed in th e eye w ith ou t any capsules, using fibrin glu e. An terior segm en t opt ical coh eren ce tom ography w as u sed for th e first t im e in Dr. Agar w al’s eye h ospital to qu an t ify ch anges in an terior ch am ber (AC) m orp h ology in in flam m at ion , assessing all th e param eters. Th e hyperreflect ive spots detected in th e AC w ere cou n ted m an u ally an d w ith an au tom ated com pu ter algorith m . Also for th e first t im e, pseudoph akic bu llou s keratopathy w as m an aged by a com bin at ion of In t raLase (Abbot t Medical Opt ics, San t a An a, CA) keratoplast y, explan t at ion of th e AC IOL, vit rectom y, an d im plan t at ion of a glu ed PC IOL. A n ew su rgical tech n ique, Jacob-Agar w al sling surger y, w as devised for acquired an d con gen it al ptosis w ith poor levator fu n ct ion in w h ich th e Seiff silicon e su spen sion set is u sed in a fron t alis sling procedu re. Th e en t ire length of th e silicon e set is gu ided in th e m uscle plan e th rough a single st ab in cision .

xiii

xiv

About the Editors

Prof. Agar w al h as received m any aw ards for h is w ork in oph th alm ology, th e m ost sign ifican t being th e Barraquer Aw ard an d th e Kelm an Aw ard. His videos h ave w on m any aw ards at film fest ivals of th e Am erican Societ y of Cat aract an d Refract ive Surger y, Am erican Academ y of Oph th alm ology, an d Eu ropean Societ y of Cataract an d Refract ive Su rger y. He h as also w rit ten m ore th an 40 books, w h ich h ave been p ublish ed in variou s langu ages, in cluding English , Span ish , an d Polish . He t rain s doctors from all over th e w orld in h is cen ter on ph aco, bim an ual ph aco, LASIK, an d vitreo-ret in al su rger y. He is a professor of oph th alm ology at Ram a ch an dra Medical College in Ch en n ai, In dia, an d can be con t acted th rough th e h ospital’s Web site at h t tp://w w w.dragar w al.com .

Soosan Jacob, MS, FRCS, DNB, MNAMS Dr. Soosan Jacob is a sen ior con su lt an t oph th alm ologist in Dr. Agar w al’s Group of Eye Hospit als an d Eye Research Cen t re, Ch en n ai, In dia. Sh e is a gold m edalist in oph th alm ology. Sh e h as w on m any in tern at ion al aw ards at p rest igiou s in tern at ion al con feren ces in th e Un ited St ates. Sh e is a n oted speaker an d h as con ducted cou rses an d delivered lect ures in n u m erous n at ion al an d in tern at ion al con feren ces. Sh e h as a sp ecial in terest in cu t t ing-edge su rgical tech n iqu es for cataract , corn ea, glaucom a, an d refract ive su rger y, in cluding deep lam ellar en doth elial keratoplast y, ocu lar su rface recon st ruct ion , n ew refract ive solut ion s, an d so on . Dr. Jacob w as th e first to bring out th e con cept of an terior segm en t t ran splan t at ion . In th is, th e corn ea, sclera, an d an art ificial iris, pup il, an d IOL are t ran splan ted en bloc in pat ien t s w ith an terior st aphylom a. Sh e also devised a n ew tech n iqu e for ptosis, w h erein th e sling su rger y can be don e prim arily w ith a on e st ab in cision rath er th an th ree, th us im proving th e postoperat ive cosm et ic appearan ce of th e pat ien t , w h ile st ill ret ain ing excellen t fun ct ion al outcom e. Sh e h as auth ored m any ar t icles an d n um erou s ch apters in 27 textbooks by in tern at ion al an d n at ion al pu blish ers an d is also th e editor for th ree textbooks in oph th alm ology. Sh e is am ong th e sen ior facu lt y for th e Diplom ate of Nat ion al Board (DNB) postgradu ate t rain ing p rogram an d ph acoem ulsificat ion an d th e LASIK t rain ing p rogram for overseas doctors.

Contributors Marie D. Acierno , MD Associate Professor Depart m en t of Oph th alm ology Director, Op h th alm ology Residen cy Program Ch ief of Oph th alm ology Earl K. Long Medical Cen ter LSU Health Scien ces Cen ter New Orlean s, LA Am ar Agarw al, MS, FRCS, FRCOphth Professor of Oph th alm ology Ram ach an dra Medical College Dr. Agar w al’s Grou p of Eye Hospitals & Eye Research Cen t re Ch en n ai, In dia Athiya Agarw al, MD, FRSH, DO Director Dr. Agar w al’s Grou p of Eye Hospitals & Eye Research Cen t re Ch en n ai, In dia Do nald R. Bergsm a, MD Herbert E. Kau fm an Professor an d Ch airm an Depart m en t of Oph th alm ology LSU Health Scien ces Cen ter Director LSU Eye Cen ter of Excellen ce New Orlean s, LA Blake A. Bo oth, MD Residen t Depart m en t of Oph th alm ology Un iversit y of Alabam a at Birm ingh am Birm ingh am , AL Francesco Bo scia, MD Dirigen te Medico I Livello Depart m en t of Oph th alm ology an d Otolar yngology Un iversit y Bari Bari, It aly Sam uel Boyd, MD Ch ief Vit reoret in al Depart m en t Boyd Eye Cen ter Pan am a Cit y, Republic of Pan am a

xv

xvi

Contributors

Clem ent K. Chan, MD Presiden t an d Medical Director South ern Californ ia Desert Ret in a Con su ltan t s Associate Clin ical Professor Depart m en t of Oph th alm ology Lom a Lin da Un iversit y Lom a Lin da, CA Kim berly P. Co ckerham , MD, FACS Adju n ct Clin ical Professor Depart m en t of Oph th alm ology St an ford Un iversit y Sch ool of Medicin e Private Pract ice Cockerh am Eye Con su lt an ts Los Altos, CA Kenneth M. Daniels, OD, FAAO Adju n ct Assist an t Clin ical Professor Cen ter for In tern at ion al St u dies Pen n sylvan ia College of Optom et r y at Salus Un iversit y Ph iladelph ia, PA Private Pract ice Hopew ell-Lam bert ville Eye Associates Hopew ell, NJ Lo ri Vidal Denham , MS Research Assistan t Depart m en t of Bioch em ical Engin eering Tu lan e Un iversit y New Orlean s, LA No a Ela-Dalm an, MD Clin ical In st r uctor Depart m en t of Oph th alm ology Jules Stein Eye In st it u te UCLA Sch ool of Medicin e Los Angeles, CA Duncan A. Friedm an, MD, MPH Residen t Physician Depart m en t of Oph th alm ology Un iversit y of Alabam a at Birm ingh am Birm ingh am , AL Pablo Gili-Manzanaro Depart m en t of Oph th alm ology Fu n dación Hospital de Alcorcón Barcelon a, Spain Santo sh Hanovar, MD LV Prasad Eye In st it u te, Hyderabad, In dia

Contributors

David R. Hardte n, MD, FACS Adju n ct Associate Professor Depart m en t of Oph th alm ology Un iversit y of Min n esot a Min n esot a Eye Con sult an t s Min n eap olis, MN Jam es M. Hill, PhD Assistan t Professor Depart m en t of Microbiology, Im m u n ology, an d Parasitology LSU Health Scien ces Cen ter New Orlean s, LA Jeffery A. Ho bde n, PhD Assistan t Professor Depart m en t of Microbiology, Im m u n ology, an d Parasitology LSU Health Scien ces Cen ter New Orlean s, LA Jean T. Jaco b, PhD Professor of Oph th alm ology an d Neu roscien ce Depart m en t of Oph th alm ology LSH Health Scien ces Cen ter Director of Research LSU Eye Cen ter New Orlean s, LA So o san Jaco b, MS, FRCS, DNB, MNAMS Dr. Agar w al’s Grou p of Eye Hospitals & Eye Research Cen t re Ch en n ai, In dia Herbert E. Kaufm an, MD Em erit us Boyd Professor of Oph th alm ology, Ph arm acology, an d Microbiology LSU Health Scien ces Cen ter New Orlean s, LA Natalia Kram arevsk y, MD Virgin ia Beach Eye Cen ter, P.C. Sen tara Virgin ia Beach Gen eral Hosp ital Virgin ia Beach , VA Dhivya Asho k Kum ar, MD Con su ltan t Depart m en t of Oph th alm ology Dr. Agar w al’s Grou p of Eye Hospitals & Eye Research Cen t re Ch en n ai, In dia Man de ep Lam ba, MBBS, DNB, FERC Con su ltan t , Ret in a Fou n dat ion Dr. Agar w al’s Grou p of Eye Hospitals & Eye Research Cen t re Ch en n ai, In dia

xvii

xviii

Contributors

Pao lo Lan zetta, MD Associate Professor Depart m en t of Oph th alm ology Un iversit y of Udin e Udin e, Italy W. Barry Lee, MD Private Pract ice Corn ea, Extern al Disease, an d Refract ive Surger y Eye Con sult an ts of Atlan t a, Piedm on t Hospit al Atlan ta, GA Richard L. Lindstro m , MD Adju n ct Professor Em erit u s Depart m en t of Oph th alm ology Un iversit y of Min n esot a Fou n der an d At ten ding Surgeon Min n esot a Eye Con sult an t s Min n eapolis, MN Andrea T. Murina, MD Residen t Depart m en t of In tern al Medicin e LSU Health Scien ces Cen ter New Orlean s, LA Andrea Olm o s, BS Depart m en t of Oph th alm ology Un iversit y of Californ ia, San Fran cisco San Fran cisco, CA Lo uis E. Pro bst, MD Nat ion al Medical Director TLC Th e Laser Eye Cen ters Ch icago, IL Sw ati Ravani, MS Associate Professor BMJ Region al In st it ute of Oph th alm ology Ah m edabad, In dia Arthur L. Ro se nbaum , MD Brin dell an d Milton Got tlieb Professor of Oph th alm ology Ch ief, Division of Pediat ric Oph th alm ology an d St rabism us Vice Ch airm an Depart m en t of Oph th alm ology Jules Stein Eye In st it u te UCLA Sch ool of Medicin e Los Angeles, CA Pravee n Saluja, MS Dr. Agar w al’s Grou p of Eye Hospitals & Eye Research Cen t re Ch en n ai, In dia

Contributors

Carlo Sbo rgia, MD Ch air, In st it u te of Oph th alm ology Depart m en t of Oph th alm ology an d Otolar yngology Un iversit y of Bari Bari, It aly Ivan R. Schw ab, MD, FACS Professor Depart m en t of Oph th alm ology Un iversit y of Californ ia, Davis Health System Eye Cen ter Sacram en to, CA Cristina Sim ó n-Castellví Sim on Eye Clin ic Barcelon a, Spain Guillerm o Sim ó n-Castellví Ch ief, An terior Segm en t Su rgeon Sim on Eye Clin ic Depart m en t of Oph th alm ology Un iversit y of Barcelon a Barcelon a, Spain Jo sé María Sim ó n-Castellví An terior Segm en t Con su lt an t Em ergen cy Room Sim on Eye Clin ic Presiden t World Federat ion of Cath olic Medical Associat ion s Barcelon a, Spain Sarabel Sim ó n-Castellví Ch ief, Posterior Segm en t Su rgeon Sim on Eye Clin ic Barcelon a, Spain Jo sé María Sim ó n-To r Ch airm an Glaucom a Sen ior Con su ltan t Sim on Eye Clin ic Barcelon a, Spain Giuseppe Sm aldo ne, MD Residen t Depart m en t of Oph th alm ology an d Otolar yngology Un iversit y of Bari Bari, It aly Dariusz G. Tarasew icz, MD, PhD Ret in a Sect ion Ch ief Depart m en t of Oph th alm ology Kaiser Perm an en te Sacram en to, CA

xix

xx

Contributors

Fede rico G. Velez, MD Assistan t Clin ical Professor Depart m en t of Oph th alm ology Jules Stein Eye In st it u te UCLA Sch ool of Medicin e Los Angeles, CA Director Division of Pediat ric Op h th alm ology an d St rabism u s Olive View -UCLA Medical Cen ter Sylm ar, CA Daniele Ve ritti, MD Regist rar Depart m en t of Oph th alm ology Un iversit y of Udin e Udin e, Italy Christo pher I. Zo um alan, MD Clin ical In st r uctor Depart m en t of Oph th alm ology Division of Oph th alm ic Plast ic an d Recon st ruct ive Su rger y New York Un iversit y Langon e Medical Cen ter Man h at tan Eye, Ear, an d Th roat Hospital New York, NY

1 Ocular Trauma Daniele Verit t i, Carlo Sborgia, Francesco Boscia, Giuseppe Sm aldone, and Paolo Lanzet ta

Anterior Segment Trauma Eyelid Laceration Blun t an d pen et rat ing facial t raum a m ay result in eyelid lacerat ion . Th e lacerat ion m ay be ext ram argin al, m ay involve th e eyelid m argin , or m ay cau se t issue loss. Eyelid t rau m a is often associated w ith veh icle acciden ts, falls, sport-related t raum as, an d assau lts. Eyelid lacerat ion is m ore com m on in young m ales du e to occup at ion al an d recreat ion al preferen ces. Proper m an agem en t is n ecessar y to preser ve correct lid dyn am ics an d cosm et ic appearan ce.

Presentation Pat ien ts u sually com plain of m ild pain an d epiph ora. Displacem en t or abn orm alit ies of th e can th al angles m ay in dicate can th al ligam en t injur y. Lacerat ion s of th e deep h ead of th e m edial can th al ligam en t m ay cau se telecan th u s. Hyph em a, oth er ocu lar adn exa t raum as, an d orbit al fract ures m ay be presen t (Fig. 1.1 ).

Management Th e m ech an ism of inju r y sh ould be invest igated first , follow ed by a com plete ocu lar exam in at ion to ru le out injuries to th e globe. If n o globe rupt u re is presen t , lids sh ou ld be everted, palpated, an d exam in ed for foreign bodies. Th e lacerat ion sh ould be carefu lly exam in ed to determ in e depth , exten sion , an d m argin involvem en t . Ph otography of th e lesion s is recom m en ded. Can alicular involvem en t an d inju r y to th e levator an d th e supraorbit al n er ve sh ould be exclu ded. A com pu ted tom ograph ic scan sh ou ld be obt ain ed w h en globe rupt ure an d foreign bodies are

Fig. 1.1 Eyelid laceration involving the eyelid margin with loss of tissue. 1

2

Color Atlas of Ophthalm ology

su spected. Tetan u s prophylaxis an d baselin e serology for h u m an im m un odeficien cy viru s (HIV) an d h epatot rop ic viru ses sh ou ld be con sidered. Surgical repair sh ould be perform ed un der local an esth esia, w ith good ligh t ing an d m agn ificat ion . After adequate an esth esia, w ou n d clean ing, an d decon t am in at ion , th e lacerat ion sh ould be repaired using Vicr yl (Eth icon , In c., Som er ville, NJ) or silk 6–0 su t u re. Posterior ten don repair an d can alicu lar repair sh ould precede lid su t uring. Eyelid m argin lacerat ion sh ould be su t ured w ith a ver t ical m at t ress tech n ique. Fin ally, an t ibiot ic oin t m en t sh ou ld be applied to th e w ou n d, an d system ic an t ibiot ic th erapy sh ou ld be con sidered if con t am in at ion is su spected. Possible com plicat ion s in clu de post t raum at ic u pper lid ptosis an d corn eal u lcerat ion due to corn eal exposu re or an exposed sut u re.

Lacrimal System Trauma Th e lacrim al drain age apparat u s con sist s of th e lacrim al pu n cta on th e upp er lid an d th e low er lid, th e can aliculi, th e com m on can aliculu s, th e lacrim al sac, an d th e n asolacrim al du ct . From th eir origin at th e pun ct a, th e can alicu li run m edially tow ard th e in tern al angulu s of th e eye, w h ere th ey join to form th e com m on lacrim al can alicu lus th at open s in th e lacrim al sac. Can alicu lar lacerat ion s are th e m ost frequen t cau se of injur y to th e lacrim al system an d occu r in up to 16% of all eyelid injuries. Com m on cau ses of can alicu lar lacerat ion in clu de veh icle acciden t s, falls, assau lts, sh arp t rau m a, an d an im al bites. Su ccessfu l m an agem en t of th ese inju ries depen ds on prom pt in ter ven t ion an d good surgical tech n ique to m in im ize th e in ciden ce of post t rau m at ic epiph ora due to scarring an d sten osis in any t ract of th e lacrim al drain age system .

Presentation Pat ien ts u su ally presen t w ith a h istor y of t raum a an d m ild pain . Th e lacrim al drain age system lesion m ay be obvious or occult . Th e u se of m ethylen e blu e or fluorescein -t inged w ater irrigat ion th rough th e pu n cta an d subsequ en t visu alizat ion of th e dye in th e w oun d m ay be h elpful in iden t ifying th e cut en d (Fig. 1.2A,B).

Differential Diagnosis Lid lacerat ion n ot involving th e lacrim al drain age system , p reexist ing epiph ora

Management Th e m ech an ism of inju r y sh ou ld be invest igated, an d a com plete oph th alm ic exam in at ion sh ould be perform ed. Th e injur y to th e lacrim al drain age system can be proven w ith Bow m an probe in sert ion in th e pun ct a or by irrigat ion w ith fluorescein -stain ed salin e solu t ion . Tet an us p rophylaxis sh ou ld be con sidered. Surgical repair sh ould p rovide accu rate app roxim at ion of th e severed en ds to prom ote m u cosal h ealing. Most su rgeon s use silicon e in t u bat ion s of th e system , follow ed by apposit ion of th e perican alicu lar t issues w ith m icroscopically assisted 7–0 su t u re. Th e m edial can th al ligam en t is often injured from th e t raum a an d m u st be repaired to restore lid fun ct ion an d an atom y. Th e success rate w ith silicon e in t ubat ion an d m icroscopic rean astom osis ranges from 86 to 95%.

1 Ocular Traum a

3

Fig. 1.2 (A) Lacrimal system trauma with laceration of the inferior canaliculus. (B) Canalicular injury with eyelid laceration.

A

B

Subconjuctival Hemorrhage Su bconju n ct ival h em or rh age follow s t h e bleed in g of conju n ct ival an d ep iscleral blood vessels in to t h e su bconju n ct ival sp ace. It is u su ally associated w it h m in or t rau m a or ar ises sp on t an eou sly w it h in creased ven ou s p ressu re d u e to violen t Valsalva m an eu vers. Less frequ en t ly su bconju n ct ival h em or rh age can be associated w it h severe hyp er ten sion an d coagu lop at h ies. Var iou s d r ugs, su ch as w ar far in , n on steroidal an t iin flam m ator y d r ugs (NSAIDs), an d steroid s can m ake con ju n ct ival vessels m ore su scept ible. It is also a n or m al sequ ela of ocu lar su rger y.

Presentation A brigh t red an d flat collection of blood is seen underneath the conjunctiva; it is usually sharply dem arcated at the lim bus and surrounded by n orm al conjunctiva. This condition is usually asym ptom atic. If pain, ph otophobia, or dim inished visual acuit y occurs, a m ore serious pathological condition should be considered (Fig. 1.3 ).

Differential Diagnosis Th e differen t ial diagn osis of subconju n ct ival h em orrh age in clu des oth er cau ses of red eye, such as conju n ct ivit is, episclerit is, irit is, acu te glaucom a, an d den drit ic ulcer. Kaposi sarcom a, or oth er conju n ct ival n eoplasm s w ith secon dar y h em orrh age sh ould be t aken in to con siderat ion .

4

Color Atlas of Ophthalm ology

Fig. 1.3 Subconjunctival hemorrhage. A bright red and flat collection of blood is seen underneath the conjunctiva; it is sharply dem arcated and surrounded by normal conjunctiva.

Management Blood pressure sh ould be ch ecked in all pat ien t s, an d if th ere is a h istor y of recurren t , u nprovoked subconju n ct ival h em orrh ages, a bleeding diath esis sh ou ld be invest igated. Th e u n com plicated h em orrh age, n ot associated w ith any sign ifican t t raum a or bleeding diath esis, is t ypically a self-lim it ing con dit ion th at requ ires on ly reassu ran ce. Cold com presses for 24 h ou rs an d ar t ificial tears can be used for m ild irrit at ion . Hem orrh age clears spon tan eously in 1 to 2 w eeks. Elect ive use of NSAIDs is t ypically discou raged.

Conjunctival Laceration Th e conju n ct iva is a st rong an d resilien t t issue, but it m ay be lacerated in cases of ocu lar t raum a w ith poin ted an d sh arp objects, such as broken glass. It m ay be isolated or par t of m ore severe in t raocular injuries.

Presentation Pat ien ts u su ally presen t w ith a h istor y of ocu lar t rau m a an d com plain of red eye, m ild pain , an d foreign body sen sat ion . Slit-lam p exam in at ion reveals a conju n ct ival surface defect . Th e edges are u su ally ret racted an d rolled up, disclosing th e un derlying w h ite sclera. Subconjun ct ival h em orrh ages an d ch em osis are often presen t . Flu orescein stain ing u n der th e cobalt filter w ill en h an ce th e visualizat ion of th e defect ( Fig. 1.4 ).

Management An accu rate h istor y of ocular t raum a an d a com plete oph th alm ic exam in at ion are n ecessar y: topical an esth esia m ay be used to accurately invest igate th e u n derlying sclera in search of inju ries an d su bconjun ct ival foreign bodies. How ever, pat ien t s un der topical an esth esia m ay lose sym ptom s associated w ith th e presen ce of a foreign body. A Seidel test sh ould be perform ed to ru le ou t a ru pt ured globe. B-scan ult rason ography an d a com puted tom ograp h ic scan of th e orbit m ay be usefu l to exclu de in t raocular or in t raorbit al foreign bodies.

1 Ocular Traum a

5

A

B Fig. 1.4

Conjunctival laceration and foreign body. (Courtesy Pablo Gili M.D.)

In th e absen ce of a rupt ured globe or perforat ing inju ries, sm all conjun ct ival lacerat ion s h eal w ith out su rgical repair. Large lacerat ion s (e.g., greater th an 1.0 to 1.5 cm ) m ay be su t ured (e.g., Vicr yl 8–0). Pressu re patch ing for 24 h ou rs an d p rophylact ic an t ibiot ic oin t m en t (e.g., gen tam icin ) th ree t im es a day for 4 to 7 days sh ould su ffice.

Chemical Exposure Chem ical burns constit ute a true ocular em ergency and should be treated prom ptly. Ch em ical burn s m ay be caused by eith er acidic or alkalin e agen ts. Acid bu rn s cau se coagulat ive n ecrosis of th e corn eal epith elium . Th e form at ion of a coagulu m lim its pen et rat ion an d corn eal dam age. Hydroflu oric acid is an except ion becau se it cau ses liquefact ive n ecrosis. Com m on acids causing ocu lar burn s in clude su lfu rous acid (presen t in som e bleach es), sulfuric acid (presen t in car bat teries), hydroch loric acid (u sed in sw im m ing pools), n it ric acid, ch rom ic acid, an d acet ic acid. Alkali burn s are t ypically m ore severe becau se alkalin e agen t s are lipoph ilic an d pen et rate m ore rapidly th an acids. Th ey com bin e w ith cell m em bran e lipids an d cau se sapon ificat ion of cell m em bran es, cell death , an d disr upt ion of th e ext racellular m at rix. Th e release of collagen ases an d p roteases after th e inju r y leads to corn eoscleral m elt ing. Alkali su bstan ces th at com m on ly cause ocular burn s con tain sodiu m hydroxide (caust ic soda), am m on ium hydroxide (fert ilizer produ ct ion ), potassium hydroxide, an d calciu m hydroxide. Ch em ical burn s are often bilateral an d are frequ en tly du e to in du st rial an d occupat ion al exposu res.

Presentation Th e diagn osis of ocular ch em ical burn is t ypically based on h istor y of con t act w ith alkalin e or acid agen t s. Th e sym ptom s u su ally in clude pain , ph otoph obia, bleph arospasm , reduced vision , an d excessive tearing. If th e bu rn is m ild or m oderate, th e conju n ct iva is hyperem ic. Focal conju n ct ival ch em osis, hyperem ia, or h em orrh ages can be presen t . Eyelid edem a an d first- to secon d-degree periocular skin burn s can be seen . Corn eal fin dings m ay range from superficial pu n ctate kerat it is (SPK) to focal epith elial defect s. In severe con dit ion s w h ite areas of conju n ct ival an d lim bal isch em ia can be seen . Corn eal fin dings u sually con sist of tot al epith elial loss, st rom al h azing, an d, in sam e cases, com plete opacificat ion . Oth er sign s in clu de an terior ch am ber react ion an d secon d- or th ird-degree periocu lar bu rn s. ( Fig. 1.5A,B).

6

Color Atlas of Ophthalm ology

Fig. 1.5 (A) A moderate chem ical injury with 6 hours of limbal blanching, a large epithelial defect, and strom al haze. (B) The sequelae of a severe chemical injury demonstrating a scarred and vascularized cornea. This eye underwent a perm anent keratoprosthesis. (Courtesy of Christopher Rapuano)

A

B

Differential Diagnosis Th erm al bu rn s, u lt raviolet (UV) kerat it is, ulcerat ive kerat it is

Management Ch em ical bu rn s are con sidered a t ru e oph th alm ologic em ergen cy an d requ ire im m ediate care. Th e first p riorit y is im m ediate an d copious irrigat ion w ith sterile irrigat ing solut ion or salin e solu t ion . If th ese solu t ion s are n ot available, t ap w ater can be used. Irrigat ion sh ould be con t in u ed u n t il n eut ral pH is reach ed. In sert ion of a lid specu lum an d topical an esth et ic p rior to irrigat ion facilit ates th e procedu re. After irrigat ion a good h istor y w ith an exact iden t ificat ion of th e ch em ical agen t sh ou ld be obtain ed. Slit-lam p exam in at ion w ith flu orescein stain ing sh ould be perform ed. Eyelids sh ould be everted to search for residual ch em icals an d foreign bodies. Th e goal of th erapy is to reduce pain , in flam m at ion , an d risk of in fect ion . Th u s cyclop legic agen ts (avoid ph enyleph rin e becau se it is a vasocon st rictor), oral an algesics (avoid repeated ap plicat ion s of topical an esth et ics becau se th ey can delay epith elial h ealing), an d oph th alm ic an t ibiot ics (avoid am in oglycoside an t ibiot ics because th ey im pair ep ith elial h ealing) sh ould be adm in istered. Th e u se of topical steroids rem ain s con t roversial. Th ey can lim it in flam m at ion -m ediated ocu lar dam age, bu t th ey ret ard w ou n d h ealing an d predispose to in fect ion . Severe burn s can be m an aged w ith adju n ct ive th erapy: ocular hypoten sive m edicat ion s if th e in t raocu lar pressure is elevated, collagen ase in h ibitors if any m elt ing of th e

1 Ocular Traum a

7

corn ea occu rs, lysis of conju n ct ival adh esion s if presen t , an d act ive surgical rem oval of n ecrot ic t issu e. Long-term com plicat ion s of ch em ical burn s in clu de perforat ion , scarring, corn eal n eovascularizat ion , sym bleph aron , glaucom a, cat aract s, an d ret in al dam age. Ult im ate progn osis is related to th e degree of lim bus isch em ia, th e depth of th e corn eal inju r y, an d th e presen ce of sym blep h aron .

Corneal Abrasion Corneal abrasions represent one of the m ost com m on ophthalm ic problem s seen in em ergency departm ents. A corneal abrasion is the disruption of the protective epithelium covering the cornea; it m ay be caused by direct or tangential im pact. Com m on causes are scratches from fingernails, anim al paw s, tree branches, or a paper cut . An oth er com m on cause is con t act len s over w ear. A large n u m ber of corn eal abrasion s are preven table. High -risk w orkers (e.g., w oodw orkers, m etal w orkers) an d players of cert ain spor ts (e.g., h ockey, racquetball, cross-coun t r y skiing, m oun tain biking) sh ould w ear ap propriate eye protect ion .

Presentation Th e pat ien t’s h istor y t ypically in clu des eye t raum a an d subsequ en t acute pain . Presen t ing sym ptom s u sually in clu de severe pain , excessive tearing, ph otoph obia, foreign body sen sat ion , bleph arospasm , an d blu rred vision . At slit-lam p exam in at ion diffuse corn eal edem a, ep ith elial disru pt ion , an d circu m corn eal inject ion can be seen ( Fig. 1.6 ).

Differential Diagnosis Acute angle glau com a, h erpes ulcers an d oth er corn eal u lcers, corn eal foreign body, an d corn eal perforat ion

Management Visual acuit y should be assessed because it m ay be significantly reduced if th e abrasion is on the optic axis. Upper and low er tarsal conjunctiva should be inspected carefully for foreign bodies. If exam ination is lim ited by excessive pain, one drop of topical anesthetic could be adm inistered for diagnostic purposes. At slit-lam p ex-

Fig. 1.6 Corneal defect stained with fluorescein. (Courtesy of Nibaran Gangopadhyay)

8

Color Atlas of Ophthalm ology

am ination the visualization of the corneal abrasion can be im proved using fluorescein staining under blue-cobalt filtered light. The abrasion should be docum ented in size, shape, depth, and localization. A Seidel test should be perform ed to rule out possible full-thickness injury. Intraocular pressure should be m easured in both eyes, and the anterior cham ber should be carefully investigated for evidence of iritis. Preven tion of infection is a key point in corneal abrasion treatm ent. An antibiotic ointm ent should be used; consider an tipseudom onas coverage for abrasions due to con tact lens overw ear. Patients w ith con tact len s–associated corn eal abrasion or a w ound th at is caused by vegetable m at ter should have antipseudom onas coverage (e.g., tobram ycin, ciprofloxacin, gentam icin, ofloxacin). Oral analgesics are often necessary ow ing to the severit y of pain. Topical NSAIDs (e.g., diclofen ac, ketorolac) m ay be useful in reducing pain. Patients using topical NSAIDs m ay take few er oral analgesics. Never provide topical an esthetics to take hom e because they can delay w ound healing. One drop of topical cycloplegic can be used if the patien t is really photophobic. This relieves ciliar y spasm , reduces pain, and im proves com fort. Pressure patching is no longer recom m ended. It sh ould be used for 6 hours only if pain is severe. Given the risk of infection, do n ot patch if the lesion is caused by vegetable m at ter or contact lenses. Healing of sm all abrasions is expected w ithin 24 to 48 hours. Deep and large abrasions m ay require 5 to 7 days to heal. Most corneal abrasions (sm all and peripheral) do not need any follow -up. How ever, contact lens w earers or patients w ith a central or large abrasion should be reevaluated in 24 hours and ever y 2 to 3 days until abrasion clears. Patients should return sooner if sym ptom s w orsen.

Corneal Foreign Body A corn eal foreign body is a com m on cau se of visits for oph th alm ic em ergen cies. It frequ en tly occurs w h en on e is grin ding an d drilling steel w ith ou t w earing p rotect ive goggles.

Presentation Th e pat ien t’s h istor y u sually in clu des an ocu lar t rau m a. Th e m ore frequen t sym ptom s are m ild or m oderate pain , foreign body sen sat ion , excessive tearing, ph otoph obia, an d blu rred vision . At slit-lam p exam in at ion on e or m ore object s can be seen lodged superficially or em bedded w ith in th e corn ea. Metallic foreign bodies m ay leave rust rings in th e su rrou n ding corn ea. Oth er sign s in clu de a circu m lim bal conju n ct ival inject ion , eyelid edem a, an d a sterile in filt rate su rroun ding th e foreign body ( Fig. 1.7 ).

Fig. 1.7

Corneal foreign body.

1 Ocular Traum a

9

Differential Diagnosis Corn eal abrasion , in t raocu lar foreign body, bacterial or fungal kerat it is

Management After h aving assessed visual acuit y, it is im por tan t to ru le ou t a p ossible perforat ing inju r y. Th is can be don e using a Seidel test (in st ill flu orescein to in spect for aqu eous leakage), m easu ring in t raocu lar pressu re, an d paying at ten t ion to an terior ch am ber react ion . Con sider a b-scan ult rasou n d an d an orbit al com pu ted tom ograph ic scan to exclu de in t raocular an d in t raorbital foreign bodies. If th ere is n o perforat ion , th e object can be rem oved u n der topical an esth esia (e.g., proparacain e 0.5%) u sing a foreign body spud or a 25-gauge n eedle. Th is operat ion can be facilit ated by sterile irrigat ion . Th e rust ring can be rem oved u sing an oph th alm ic drill. Th ese procedures sh ou ld be perform ed at slit lam p by w ell-t rain ed an d experien ced physician s. Before an d after th e rem oval, an t ibiot ic drops sh ou ld be applied u n t il h ealing. A top ical cycloplegic can be used to reduce ph otoph obia an d pain . Pat ien t s sh ou ld be reevalu ated ever y 2 to 3 days u n t il th e w ou n d is h ealed an d th e in filt rate resolved.

Corneal Laceration Th e lacerat ion can be par t ial th ickn ess or fu ll th ickn ess.

Presentation In part ial-th ickn ess lacerat ion , th e an terior ch am ber is n ot en tered, an d, th erefore, th e corn ea is n ot perforated. If th e Seidel test is posit ive, a full-th ickn ess lacerat ion is p resen t . In full-th ickn ess lacerat ion th e pat ien t presen ts w ith tearing, pain , an d loss of vision . Associated fin dings in clude: sh allow an terior ch am ber, an terior syn ech iae, corn eal opacit y w ith en doth elial dysfun ct ion , or cataract . In t raocular pressure m ay be ver y low ( Fig. 1.8 ).

Management A h istor y an d com plete oph th alm ic exam in at ion are required to ascer tain th e diagn osis. W h ile m an aging a part ial-th ickn ess lacerat ion , a cycloplegic (e.g., scopol-

Fig. 1.8

Penetrating corneal laceration with iris prolapse.

(Courtesy Pablo Gili M.D.)

10

Color Atlas of Ophthalm ology

am in e 0.25%) an d an an t ibiot ic (e.g., frequ en t polym yxin B/bacit racin oin t m en t su ch as polysporin ) or flu oroquin olon e drops, depen ding on th e n at u re of th e w ou n d, are st arted im m ediately. W h en a m oderate to deep corn eal lacerat ion is accom pan ied by w oun d gape, it is often best to su t u re th e w ou n d closed in th e operat ing room to avoid excessive scarring an d corn eal irregu larit y, especially in th e visu al axis. Tet an us toxoid for dir t y w ou n ds is a m ust . Note th at sm all, self-sealing, or slow -leaking lacerat ion s m ay be t reated w ith aqu eou s suppressan ts, ban dage soft con tact len ses, flu oroquin olon e drop s four t im es a day. Altern at ively, a pressu re patch an d t w ice-daily an t ibiot ics m ay be used. Avoid top ical steroids.

Traumatic Iritis A blun t t raum a to th e eye can cau se t rau m at ic in flam m at ion of th e iris or, m ore accu rately, of th e an terior uveal t ract . Th is leads to th e presen ce of in flam m ator y cells in th e an terior ch am ber of th e eye. Traum at ic irit is gen erally develops qu ickly after th e t rau m a an d u su ally affect s on ly th e inju red eye.

Presentation Pat ien ts usu ally presen t w ith a h istor y of ocu lar t rau m a. Sym ptom s in clude pain , ph otoph obia, an d possibly h eadach e. Pain t ypically w orsen s w h en eith er th e inju red eye or th e u n involved eye is exposed to brigh t ligh t (du e to con sen su al pupillar y con st rict ion ). Sign s in clude cells an d flare in th e an terior ch am ber an d perilim bar inject ion ( Fig. 1.9 ). Th e iris pu pillar y m argin of th e involved eye m ay be differen t in sh ape com pared w ith th e con t ralateral.

Differential Diagnosis Trau m at ic corn eal abrasion , t raum at ic m icrohyph em a, oth er causes of an terior uveit is

Management Post t raum at ic pain w ith out corn eal abrasion or ulcer sh ould suggest th e diagn osis of t raum at ic irit is. Th is diagn osis can be con firm ed by th e presen ce of cells an d

Fig. 1.9 Post traumatic hyphema and iritis. (Courtesy of Amar Agarwal, Dr. Agarwal’s Eye Hospital, Chennai, India)

1 Ocular Traum a

11

flare in th e an terior ch am ber at slit-lam p exam in at ion . A com plete oph th alm ic evalu at ion sh ou ld be perform ed, in cluding ton om et r y an d fu n dus exam in at ion . Treat m en t t ypically con sists of cycloplegic agen t s. In refractor y cases an d if n o corn eal epith elial defect is detected, a steroid drop could be given . Pat ien ts sh ou ld be re-evalu ated w ith in a w eek; if irit is is resolved, m edicat ion can be discon t in u ed.

Iris Sphincter Tear Blun t inju r y often causes tears in th e sph in cter p upillae of th e iris.

Presentation Th e pat ien t m ay be asym ptom at ic or m ay h ave glare an d ph otoph obia. Th e tears in th e pupillar y m argin can be visu alized on slit lam p exam in at ion (Fig. 1.10 )

Differential Diagnosis Oth er causes of a dilated p upil (e.g., ph arm acological m ydriasis)

Management A th orough ocu lar exam in at ion is don e to ru le ou t any oth er coexist ing dam age. It m ay be left alon e un t reated. If cau sing sym ptom s, an d if cat aract ext ract ion is also being plan n ed, on e m ay perform a pupilloplast y or use an iridia segm en t s.

Traumatic Cataract Trau m at ic cataract can develop after various t ypes of in sult: blun t or perforat ing t raum a, elect ric sh ock, in frared, UV, an d ion izing radiat ion . Blun t t rau m a is th e m ost com m on cause, an d cou p an d con t recoup inju ries, along w ith equ atorial expan sion , are th e path ophysiological m ech an ism respon sible for ocu lar dam age. As regards len s inju r y, “coup” is th e cau se for Vossius ring (iris pigm en t rem ain s im prin ted on th e an terior capsule), an d “con t recou p” is respon sible for th e sh ock w aves th at m ay lead to an terior or posterior capsular ru pt u re an d su bsequ en t len s opacificat ion . Th e equatorial st retch ing can disr upt th e zon ules an d capsu le. Th e

Fig. 1.10

Sphincter tear. (Courtesy of Amar Agarwal, Dr.

Agarwal’s Eye Hospital, Chennai, India)

12

Color Atlas of Ophthalm ology

release of len s protein s du e to capsu le rupt u re can lead to ph acoan aphylact ic uveit is, ch aracterized by th e presen ce of polym orph on uclear leukocyte (eosin oph ils) an d gian t cell in filt rat ion su rroun ding len s m aterials. Th e occlusion of th e t rabecular m esh w ork du e to len s protein s an d m acroph ages can lead to an acu te rise in in t raocular p ressure. Glaucom a can also be secon dar y to relat ive pup illar y block du e to posterior syn ech iae or len s sw elling (ph acom orph ic glaucom a).

Presentation If no perforating traum a or traum a-related sym ptom atic iritis occurs, the patient could w ait for days, weeks, or m onths before searching for m edical care. The patient usually presents w ith a history of traum a and m ay com plain of decreased vision and m onocular diplopia. At slit lam p exam ination, cataract associated w ith blunt traum a usually appears as stellate or roset te-shaped “visual axis” opacification located inaxis and involving the posterior capsule. Perforating traum a leads to cortical opacification at the site of injur y. This opacification usually rem ains localized, but if the capsular tear is large enough, the entire lens m ay opacify. Hyphem a, signs of iritis, and lens dislocation or sublu xation m ay be present (Fig. 1.11A,B).

A

B Fig. 1.11 (A) Cataract and iridodialysis secondary to severe penetrating trauma. (B) Post traum atic endophthalmitis.

1 Ocular Traum a

13

Differential Diagnosis Sen ile cat aract , ectopia len t is, angle recession glaucom a, an d hyph em a

Management Mech an ism of injur y an d past ocular h istor y sh ould be invest igated first . Th en a rupt ured globe an d in t raocular foreign body sh ou ld be ru led ou t an d a com plete oph th alm ic exam in at ion sh ou ld be perform ed. Type an d exten t of len s opacificat ion an d th e presen ce of ocu lar in flam m at ion , hyph em a, ph acodon esis, iridodon esis, angle recession , len s sw elling, len s dislocat ion , or sublu xat ion sh ould be docum en ted. Zon u lar disru pt ion m ay be detected gon ioscopically th rough a dilated pu pil. Posterior segm en t t rau m a-related p ath ology sh ould be invest igated by fu n du scopy. If opacificat ion obst ruct s th e view of th e posterior segm en t , u lt rason ography m ay be h elpfu l. Medical t reat m en t sh ou ld be directed to focal an d off-a xis opacit ies, in flam m at ion , an d in t raocular pressu re rise. Miot ics can h elp to obt ain a clear visual axis, in flam m at ion can be con t rolled w ith cor t icosteroids, in creased in t raocu lar pressu re can be t reated w ith st an dard ocu lar hypoten sive m edicat ion s. How ever, su rgical rem oval of th e len s u sually resolves th ese com plicat ion s. Decreased visual acu it y, len s-in duced in flam m at ion or glau com a, capsular rupt ure w ith len s sw elling, an d poor visualizat ion of posterior segm en t path ology are in dicat ion s for su rger y. St an dard ph acoem u lsificat ion is preferred if th e len s capsule is in tact an d th ere is su fficien t zon u lar su ppor t; in t racapsu lar ext ract ion is in dicated for zon ular in st abilit y or an terior dislocat ion . In cases of posterior dislocat ion or posterior capsular rupt u re, pars plan a len sectom y an d vit rectom y m ay be preferred. As regards len s im plan tat ion , capsu lar fixat ion is in dicated if zon u lar su ppor t an d th e len s capsu le are in tact; capsu lar ten sion rings m ay h elp in cases of lim ited zon ular dialysis. If th e posterior capsule is com prom ised but sufficien t zon ular su ppor t rem ain s, su lcus fixat ion sh ould be ch osen . A sut u re fixat ion approach w ou ld be th e best if both zon u lar an d capsu lar su ppor t are in adequ ate. If n o posterior supp or t is m ain t ain ed, an terior ch am ber posit ion ing sh ou ld be con sidered. Com plicat ion s associated w ith t raum at ic cat aract in clude glau com a (pup illar y block glau com a, p h acolyt ic glaucom a, ph acom orph ic glau com a, angle recession glaucom a), ph acoan aphylact ic uveit is, hyph em a, ret in al det ach m en t , ch oroidal ru pt u re, t raum at ic opt ic n eu ropathy, an d globe rupt u re.

Lens Dislocation/Subluxation Su blu xat ion is par t ial disrupt ion of th e zon u lar fibers; th e len s is decen tered bu t rem ain s par t ially in th e pu pillar y apert u re. Dislocat ion is com plete disru pt ion of th e zon ular fibers; th e len s is displaced ou t of th e pu pillar y apert u re. Trau m a (m ost com m on cau se), Marfan syn drom e, h om ocyst in u ria, Weill–March esan i syn drom e, acqu ired syph ilis, congen it al ectop ia len t is, an iridia, Eh lers–Dan los syn drom e, Crou zon disease, hyperlysin em ia, sulfite oxidase deficien cy, h igh m yopia, ch ron ic in flam m at ion s, an d hyperm at ure cat aract are som e of th e cau ses of len s su blu xat ion .

Presentation Decreased vision an d dou ble vision th at persist w h en covering on e eye (m on ocu lar diplopia) are th e m ain sym ptom s. Crit ical sign s are decen tered or displaced len s, iridodon esis (qu ivering of th e iris), an d p h acodon esis (qu ivering of th e len s). Oth er sign s in clu de m arked ast igm at ism , cataract , angle closure glaucom a as a resu lt of pupillar y block, acqu ired h igh m yopia, vit reou s in th e an terior ch am ber, an d asym m et r y of th e an terior ch am ber depth ( Fig. 1.12 )

14

Color Atlas of Ophthalm ology

Fig. 1.12

Lens subluxation and zonular disruption.

Management Fam ily, person al, m edical, an d t rau m a h istor y is ver y im port an t . System ic exam in at ion sh ould evalu ate st at u re, ext rem it ies, h an ds, an d fingers as n ecessar y. Rapid plasm a reagin test an d fluorescen t t rep on em al an t ibody absorpt ion test , sodiu m n it ropru sside test , ech ocardiography, an d urin e ch rom atography to rule out h om ocyst in uria are n eeded. Lens dislocated into the vit reous: Surgically rem ove th e len s. Lens capsule intact, pat ient asym ptom at ic no signs of inflam m at ion : Obser ve. Lens capsule broken eye inflam ed : Len sectom y is don e eith er th rough th e pars plan a or by using a lim bal approach Sublu xat ion Asym ptom at ic: Obser ve. High uncorrectable ast igm at ism or m onocular diplopia : Su rgical rem oval of th e len s . Sym ptom at ic cataract : Opt ion s in clu de surgical rem oval of th e len s. Pupillary block : Treat m en t is iden t ical to th at for aph akic pup illar y block . Marfan syndrom e is present : Refer th e pat ien t to a cardiologist for an an n u al ech ocardiogram an d m an agem en t of any cardiac-related abn orm alit ies. Prophylact ic system ic an t ibiot ics are required if th e p at ien t un dergoes su rger y (or a den tal procedu re) to preven t en docardit is. Hom ocyst inuria is present : Adm in ister pyridoxin e, 50 to 1000 m g by m outh fou r t im es a day. Redu ce diet ar y m eth ion in e. Avoid su rger y if possible becau se of th e risk of th rom boem bolic com plicat ion s. If surgical in ter ven t ion is n ecessar y, an t icoagu lan t th erapy is in dicated.

Microhyphema/ Hyphema Trau m at ic hyph em a is defin ed by post inju r y accu m ulat ion of blood w ith in th e an terior ch am ber. Microhyph em a con sist s of su spen ded er yth rocytes in th e an terior ch am ber, gen erally visible at slit lam p. Equatorial expan sion after blu n t t raum a in duces st ress to angle st ru ct u res, w h ich can lead to ru pt u re of iris an d ciliar y body

1 Ocular Traum a

15

vessels w ith su bsequen t h em orrh age. Lacerat ing injur y can be associated w ith direct dam age of blood vessels an d hypotony. Som e con dit ion s such as ru beosis iridis, juven ile xan th ogran u lom a, h em oph ilia, leu kem ia, an d th e use of dr ugs th at alter platelet or th rom bin fu n ct ion m ay facilit ate th e on set of hyph em a. A sign ifican t n um ber of sigh t-th reaten ing com plicat ion s m ay develop, w h ich requires careful follow -u p for hyph em a pat ien ts.

Presentation Pat ien ts usu ally presen t w ith a h istor y of blun t t rau m a. Pain an d blu rred vision are com m on sym ptom s. Hyph em as are graded on th e am ou n t of blood w ith in th e an terior ch am ber: grade I is less th an on e th ird filling of th e an terior ch am ber, grade II hyph em as h ave m ore th an on e th ird but less th an on e h alf of th e an terior ch am ber filled w ith blood, grade III is m ore th an on e h alf bu t less th an total filling, grade IV is a total hyph em a, also kn ow n as eigh t-ball hyph em a ( Fig. 1.13 ).

Differential Diagnosis Uveit ic glau com a an d cau ses of spon tan eou s hyph em a such as juven ile xan th ogran ulom a, iris cavern ous h em angiom a, hyper ten sion , an d bleeding disorders

Management Mech an ism an d t im e of inju r y sh ou ld be invest igated carefully. A h istor y of sickle cell t rait or disease sh ou ld be sough t ou t . In spect ion for gross ocu lar injur y an d evaluat ion of th e adn exa sh ou ld be perform ed. A ru pt u red globe sh ould be ruled ou t . A com plete ocu lar exam in at ion is im perat ive an d m ust in clu de in t raocu lar pressure m easu rem en t an d dilated fun du scopic evalu at ion . Gon ioscopy sh ould be deferred un t il hyph em a resolves to detect poten t ial rebleeding sites an d angle recession . A draw ing of th e hyph em a docum en t ing sh ape an d size sh ou ld be recorded at ever y oph th alm ic evalu at ion . Depen ding on th e pat ien t’s h istor y, h em oglobin opath ies an d bleeding disorders sh ou ld be invest igated. B-scan u lt rason ography m ay be u sefu l in pat ien t s w ith large hyph em as, w h en oph th alm oscopy is n ot feasible. Non com plian t pat ien t s or th ose w ith in creased risk of rebleeding, u n con t rolled

Fig. 1.13 Post traum atic hyphema. Grade II hyphemas have more than one third but less than one half of the anterior chamber filled with blood.

16

Color Atlas of Ophthalm ology

glaucom a, posit ive sickle cell t rait , or an em ia sh ou ld be con sidered for inpat ien t h ospitalizat ion . Th e elevat ion of th e pat ien t’s h ead to 30 to 45 degrees w h ile lying su pin e m ay facilit ate th e set tling an d layering of th e hyp h em a in th e in ferior an terior ch am ber, allow ing an easier classificat ion of th e hyph em a, an earlier evalu at ion of th e posterior pole, an d a m ore rap id im provem en t in visual acu it y. A t ran sparen t plast ic sh ield sh ould be u sed to protect th e involved eye from fur th er inju r y. It s t ran sparen cy allow s recogn izing rebleeding or su dden visu al loss. Medical t reatm ent in cludes topical cycloplegics (1 drop of 1% at ropin e three tim es a day for up to 5 days) to in crease the pat ient’s com fort (con sider th e risk of precipitating acute glaucom a in pat ien ts w ith a n arrow ch am ber angle) and topical steroids (0.1%dexam eth ason e) to decrease inflam m at ion, reduce anterior ch am ber reaction , an d prevent the incidence of secon dar y hem orrh age (caution sh ould be exerted if steroids are used for a prolonged period because th ey can in crease the risk of cataract an d glaucom a). Topical and system ic an tifibrin olytics, such as am inocaproic acid, could be used to prevent rebleeding an d retarding clot lysis. Th e m ore com m on side effects of am in ocaproic acid in clude vom iting, diarrhea, and post ural hypoten sion . Its system ic use sh ould be avoided in patien ts w ith hepatic or ren al disease. Persistent in creased in traocular pressure should be treated in itially w ith topical -blockers. If th is treatm en t is unsuccessful, topical -agon ist or carbonic an hydrase in hibitor m ay be added in patients w ith out sickle cell t rait or disease. Aspirin an d other NSAIDS should be discont in ued. Un con trolled elevated int raocular pressure (at least 45 m m Hg for 5 days) could be surgically t reated w ith paracentesis and an terior ch am ber w ashout . Other indicat ions to surger y are early corneal staining or rebleeding hyph em as. Sm aller hyphem as are usually self-lim iting an d clear w ith in 5 days. Large hyphem as are associated w ith com plication s an d th e w orst progn osis. Such com plicat ions are secon dar y h em orrhage, corneal blood stain ing, glaucom a, an terior an d posterior syn ech iae, cataract, an d opt ic atrophy.

Ruptured Globe A rupt ured globe is a devastating injur y w ith significant long-term con sequen ces for the pat ien t. It represen ts a discont in uit y of the eye’s outer m em branes caused by blunt or pen et rating t raum a. Rupt ures resulting from blunt traum a usually occur at th e sites w here the sclera is w eakest, such as at th e in sertion of th e extraocular m uscles, around the opt ic ner ve, an d at the lim bus. Sh arp objects w ith sufficient m om en t um m ay directly perforate th e globe. Globe rupt ure is m ore com m on in young m ales ow ing to their occupation al and recreation al preferen ces. High m yopia and previous eye surger y can m ake t issues m ore vulnerable to rupture. A rupt ured globe is an ophth alm ic em ergen cy and requires surgical repair as soon as possible. Th e visual outcom e depends largely on early recogn ition and prom pt in ter vent ion.

Presentation Th e pat ien t usu ally p resen t s w ith a h istor y of ocu lar t rau m a. Sym ptom s in clude pain , w h ich can be n ot ext rem ely severe in th e case of sh arp injur y, an d decreased vision . Diplopia m ay be presen t du e to ext raocu lar m u scle en t rapm en t or dysfun ct ion an d t raum a-associated cran ial n er ve palsy. At physical exam in at ion th e globe rupt ure m ay be obviou s or occu lt . A fu ll-th ickn ess corn eal or scleral lacerat ion is a sign of globe perforat ion . Prolapse of th e iris or ext rusion of ocular con ten t s m ay be presen t . Severe conju n ct ival h em orrh age, usually involving 360 degrees of bu lbar conju n ct iva, t ypically in dicates globe ru pt ure. Oth er accom panying sign s in clu de irregu lar pupil, hyph em a, len s injur y, com m ot io ret in ae, vit reou s h em orrh age, ch oroidal rupt ure, ret in al tears an d detach m en ts, an d t raum at ic opt ic n eu ropathy. A ru pt u red globe m ay presen t w ith both en oph th alm os an d exoph th alm os, depen ding on th e presen ce of an associated ret robu lbar h em orrh age ( Fig. 1.14 ).

1 Ocular Traum a

Fig. 1.14

17

Perforating ocular trauma.

Management Th e m ech an ism an d th e circu m st an ces of injur y an d th e n at ure of th e t raum at izing object sh ou ld be invest igated. Visu al acuit y sh ould be docu m en ted an d ext raocu lar m u scle fu n ct ion sh ou ld be evaluated. Pup ils sh ould be exam in ed for size, sh ape, an d ligh t reflex. Th e diagn osis of a rupt u red globe sh ould be m ade by slit lam p or pen ligh t . Th e orbit an d adn exa sh ou ld be exam in ed for inju ries, foreign bodies, bon e deform it y, an d eyeball displacem en t . In t raocu lar pressu re m easu rem en t is con t rain dicated to avoid pressure to th e globe. Th e eye sh ou ld be p rotected w ith a sh ield. System ic prophylact ic an t ibiot ics an d an algesics, if advisable, sh ou ld be adm in istered. Th e pat ien t sh ou ld receive tet an u s im m un izat ion if in dicated an d be kept n oth ing per os. Th e im aging st u dy of ch oice is com pu ted tom ography; if it is n ot available a p lain x-ray film sh ou ld be obt ain ed. Magn et ic reson an ce im aging m ay be usefu l to iden t ify soft t issue an d globe inju ries, bu t it is con t rain dicated if a m et allic foreign body is suspected. Careful B-scan ult rason ography m ay be u sefu l to iden t ify th e site of ru pt u re an d in t raocular foreign bodies. Surgical repair sh ou ld be prom pt . If th ere is n o expect at ion to restore vision , en ucleat ion sh ou ld be con sidered. En dop h th alm it is an d sym path et ic oph th alm ia are possible sigh t-t reat ing com plicat ion s th at sh ould be born e in m in d.

Posterior Segment Trauma Posttraumatic Vitreous Hemorrhage Vit reous h em orrh age resu lt s from bleeding in to on e of th e several poten t ial spaces form ed aroun d an d w ith in th e vit reou s body. Th is con dit ion can follow injuries to th e ret in a an d uveal t ract an d th eir associated vascu lar st ru ct u res. Neovascu larizat ion occu rring in diseases like proliferat ive diabet ic ret in opathy m ay predispose to bleeding, even if th e t raum a is m ild. Oth er disorders th at p rom ote th e release of angiogen ic vasoact ive factors an d subsequ en t form at ion of n eovascu lar an d fragile vessels th at can easily bleed are isch em ic ret in op athy secon dar y to ret in al vein oc-

18

Color Atlas of Ophthalm ology

clusion , ret in opathy of prem at u rit y, an d proliferat ive sickle cell ret in opathy. Trau m at ic vit reou s h em orrh age in ch ildren m ay be a sign of ch ild abu se (sh aken baby syn drom e).

Presentation Pat ien ts w ith t raum at ic vit reou s h em orrh age usually presen t w ith a com plain t of decreased visu al acu it y, floaters, cloudy vision , percept ion of sh adow s, visual h aze, an d ph otoph obia. Pat ien ts m ay n ot rem em ber th e t raum at ic in su lt . Direct oph th alm oscopy reveals a dim in ish ed red reflex th at can be black in severe cases. In direct oph th alm oscopic exam in at ion discloses th e presen ce of blood in th e an terohyaloid or ret rohyaloid spaces or w ith in th e vit reou s gel. Usu ally a subhyaloid h em orrh age suggest s a source of bleeding an terior to th e ret in a, w h ereas a h em orrh age posterior to th e in tern al lim it ing m em bran e im plies a source of bleeding w ith in th e ret in a. Long-stan ding h em orrh ages can evolve in w h ite m asses ( Fig. 1.15 ).

Differential Diagnosis Differen t ial diagn osis of t raum at ic vit reous h em orrh age in clu des oth er vit reous h em orrh ages n ot related to t rau m a. Spon t an eou s vit reou s h em orrh age m ay occu r in con dit ion s like proliferat ive ret in opath ies, ch oroidal or ciliar y body m elan om a, ret in oblastom a, uveit is, sarcoidosis, ocu lar m an ifestat ion of syph ilis, or h istoplasm osis.

Management A detailed h istor y is ver y im port an t . Un derlying path ologies an d m ech an ism of t raum a sh ou ld be docu m en ted. A com plete eye exam in at ion sh ould be perform ed, in clu ding slit lam p exam in at ion , in t raocu lar p ressure m easu rem en t , an d dilated fun du s evalu at ion . Globe perforat ion an d in t raocu lar foreign body sh ou ld be ruled out . B-scan ult rason ography can be u sed w h en th e fu n dus is difficu lt to visu alize, disclosing th e presen ce of ret in al det ach m en t , ret in al tears, in t raocu lar foreign body, or in t raocular t um or. In it ial th erapy con sist s of bed rest w ith 30- to 45-degree h ead elevat ion (allow s th e blood to set tle in feriorly) an d avoidan ce of an t icoagu lat ive drugs an d in ten se Valsalva m an euvers. Con clu sive th erapy is fired at th e u n derlying cau se: ret in al breaks can be closed w ith laser ph otocoagulat ion , an d surger y can resolve ret in al det ach m en t s. Vit rectom y is also in dicated in longstan ding vit reou s h em orrh age ( 2 to 3 m on th s) an d w h en vit reous h em orrh age is associated w ith ru beosis an d gh ost-cell glaucom a. Com plicat ion s of vit reou s h em -

Fig. 1.15 Post traum atic vitreous hemorrhage.

1 Ocular Traum a

19

orrh age usu ally develop w h en large am oun ts of blood rem ain for long periods in th e vit reou s cavit y an d in clude en h an ced proliferat ive ret in opathy, h em osiderosis bulbi an d con sequen t iron toxicit y, gh ost-cell glaucom a, am blyop ia (resu lt ing from visu al deprivat ion ), an d m yopic sh ift in in fan t s.

Commotio Retinae Com m ot io ret in ae is a clin ical en t it y first described in 1873 by Berlin an d is ch aracterized by a t ran sien t w h iten ing at th e deep sen sor y ret in a. Th is con dit ion is com m on ; it h as been sh ow n to be resp on sible for 9.4%of all p ost t raum at ic fu n dus ch anges. Th e m ech an ism of inju r y is th e con t recoup force follow ing blun t ocu lar t raum a th at causes degen erat ion of th e ph otoreceptors’ ou ter segm en t s an d subsequ en t ph agocytosis by ret in al p igm en t epith eliu m cells. Th e presen ce of edem a in th e ou ter plexiform layers, n uclear layers, an d su bret in al space h as been dem on st rated. Angiograph ic eviden ce h as sup por ted th e belief th at ret in al an d ch oroidal vessels do n ot play a sign ifican t role in th e path ogen esis of th is con dit ion .

Presentation Pat ien ts m ay be asym ptom at ic if com m ot io ret in ae is lim ited to th e periph eral ret in a, or th ey m ay com plain of decreased vision if th e w h iten ing occurs in th e foveal region . Visu al acuit y m ay be variably affected an d does n ot alw ays relate to th e degree of opacificat ion . Oph th alm oscopic exam in at ion reveals a cloudy opacificat ion of th e ret in a, u su ally w ith poorly defin ed m argin s. It can be located anyw h ere w ith in th e posterior segm en t . In som e cases th e en t ire posterior pole can be involved, an d it m ay appear as a pseudoch err y red spot . Ret in al vessels are clearly visible an d ap pear un dist u rbed. Oth er associated t raum at ic path ology m ay be presen t , such as su bret in al, in t raret in al, an d preret in al h em orrh ages; m acu lar h oles; m acu lar detach m en ts, an d ch oroidal ru pt ures ( Fig. 1.16 ).

Differential Diagnosis Differen tial diagnosis of com m otio ret in ae in cludes retinal detach m ent , cen tral arter y occlusion, bran ch ret in al arter y occlusion , and ret in al w h ite w ith out pressure.

Fig. 1.16 Peripheral comm otio retinae with undefined posterior borders.

20

Color Atlas of Ophthalm ology

Management Th e m ech an ism of t raum a sh ou ld be docu m en ted. A com plete op h th alm ic exam in at ion sh ould be perform ed, in clu ding dilated fu n dus evaluat ion an d scleral depression if th ere is n o eviden ce of hyph em a, m icrohyph em a, or irit is. Th e ret in al w h iten ing u su ally fades w ith in som e w eeks, an d n o t reat m en t is available, on ly obser vat ion . Abou t 60% of pat ien ts fu lly recover vision , an d 40% su stain perm an en t visu al loss. Com plicat ion s of com m ot io ret in ae in clu de cystoid areas th at m ay degen erate in to m acular h oles, ph otoreceptor loss, ret in al p igm en t epith elium (RPE) m igrat ion , degen erat ion , at rop hy, or hyperplasia.

Choroidal Rupture Traum atic choroidal rupt ure is a com m on occurrence after a blunt ocular traum a (5 to 10%). It is a defect in th e Bru ch m em bran e, th e ch oroid, an d th e ret in al pigm en t epith eliu m . W h en su dden an teroposterior com pression an d equ atorial expan sion subsequ en t to ocu lar blu n t t raum a t ake p lace, th e sclera h as en ough ten sile st rength an d th e ret in a h as en ough elast icit y to be relat ively protected. Becau se th e Bru ch m em bran e does n ot h ave th ese proper t ies, it is pron e to break . Th e dam age at th e ch oriocapillaris vessels m ay lead to su bret in al, sub-ret in al p igm en t epith eliu m , or in t rach oroidal h em orrh age. In th e acute ph ase th e overlying h em orrh age an d th e ret in al edem a m ay obscure th e ch oroidal ru pt ure itself. Typically, during th e h ealing p h ase, ch oroidal n eovascu larizat ion occu rs an d in m ost cases resolves spon t an eou sly. Con dit ion s associated w ith an in creased fragilit y of th e Bru ch m em bran e, su ch as angioid st reaks, are risk factors for t rau m at ic ch oroidal ru pt u re.

Presentation Pat ien ts u su ally p resen t w ith a h istor y of ocu lar blu n t t raum a, decreased vision , an d a variet y of visu al field defects (paracen t ral, cen t ral, sector scotom as). At oph th alm oscopic exam in at ion th e ch oroidal lesion appears as a yellow -w h ite, crescen t-sh aped, su bret in al st reak, con cen t ric to th e opt ic disc. Th e border of th e rupt u re m ay be hyperpigm en ted or hypopigm en ted. Often th e overlying h em orrh age m ay obscu re th e ch oroidal ru pt u re ( Fig. 1.17A,B).

A

B Fig. 1.17

(A) Traumatic choroidal rupture. (B) Traumatic choroidal rupture.

1 Ocular Traum a

21

Differential Diagnosis Angioid st reaks, h igh m yopia, subret in al n eovascu lar m em bran es, ocular h istoplasm osis syn drom e, ch oroidal n eovascularizat ion , pseudoxan th om a elast icum

Management A com p lete ocular exam in at ion is m an dator y. Fluorescein angiography m ay be con sidered to con firm th e presen ce of ch oroidal ru pt ure an d to detect ch oroidal n eovascularizat ion . In docyan in e green angiography m ay be u sefu l w h en su bret in al h em orrh age obscures ch oroidal n eovascu larizat ion recogn it ion . Con ser vat ive t reat m en t is advised for m ost t raum at ic ch oroidal rupt u res. Ext rafoveal ch oroidal n eovascularizat ion m ay be t reated w ith laser ph otocoagu lat ion . Pars plan a vit rectom y an d m em bran e ext ract ion m ay be con sidered for subfoveal an d ju xtafoveal ch oroidal n eovascu larizat ion . Good visu al ou tcom es are expected if th e rupt u re does n ot involve th e fovea. Possible com p licat ion s are h em orrh agic or serou s m acular detach m en t .

Posttraumatic Retinal Tears and Detachment Ocu lar t rau m a is respon sible for 10% of ret in al det ach m en t s. Usu ally th e t rau m at ic inju r y causes an an terior-posterior com pression of th e globe an d a lateral expan sion of th e equator. Th is resu lts in a t ract ion al force on th e vit reou s base, w h ere th e vit reous body is physiologically adh eren t to th e p eriph eral ret in a. Ret in al breaks are th e resu lt of vit reou s t ract ion at th e ora serrat a or in sites of focal vit reoret in al adh esion (such as corioret in al scars an d lat t ice degen erat ion ). In th e presen ce of vit reou s syn eresis, fluid dissect s th e ret in a, giving rise to ret in al detach m en t . Com m on abn orm alit ies cau sing post t raum at ic ret in al det ach m en t s are ret in al dialysis an d gian t ret in al tears. An oth er m ech an ism of inju r y is ret in al n ecrosis as a result of direct t raum a to th e sclera. It is often associated w ith ret in al h em orrh ages an d edem a an d leads to large an d irregularly sh aped ret in al tears. High m yopia an d sites of focal vit reoret in al adh eren ce are risk factors for t raum at ic ret in al detach m en t .

Presentation Ret in al detach m en ts an d ret in al tears can be diagn osed m on th s or years after th e traum a, so th e causal n exu s is n ot alw ays easy to iden t ify. Pat ien t s can presen t com plain ing of m ild blu rring of vision , floaters, ph otopsia, an d visual-field defects. Oph th alm oscopic fin dings th at suggest a vitreoret in al in terface involvem en t after a t raum a in clude vit reous base avulsion , retin al dialysis, ret in al tears of various sh apes an d dim en sion s (gian t, roun d, h orsesh oe), an d ret in al detach m en t . On ce th e ret in a becom es detach ed, it appears as an elevated, sligh tly opaqu e, corrugated surface th at un du lates freely w ith eye m ovem en ts. In th e cases of retin al detach m en t in t raocular pressu re is u sually low er th an th at of th e fellow eye (Fig. 1.18 ).

Differential Diagnosis Pen et rat ing t raum a, ret in al det ach m en t s cau sed by oth er con dit ion s (proliferat ive, t ract ion al, postoperat ive, exu dat ive), acute ret in al n ecrosis, sen ile ret in osch isis

Management A com plete oph th alm ic evaluation should be perform ed, in cluding int raocular pressure m easurem en t an d accurate retinal exam inat ion. Retinal abnorm alities, vitreo-

22

Color Atlas of Ophthalm ology

Fig. 1.18 Retinal detachment secondary to a retinal dialysis.

retin al tractions, tears, an d detachm en ts m ust be recorded. B-scan ultrason ography and opt ical coh eren ce tom ography are useful im aging st udies w hen m edia opacities im pair a com plete ophthalm oscopic retinal exam in ation. Retinal tears m ay be treated successfully by laser photocoagulation an d cr yopexy. How ever, som e gian t retinal tears m ay progress to retin al detachm en t regardless of therapy. For th is reason a prophylactic scleral buckle m ay be con sidered in th e cases of an elevated tear flap or focal vit reoretinal t raction . Retinal detach m en ts are essent ially m anaged w ith surger y. Com m on procedures are vitrectom y, pneum atic ret in opexy, and scleral buckling to support th e dialysis. Perfluorocarbonate liquids or gas bubbles can be used in traocularly to facilitate th e retin a’s adh eren ce. The final postsurger y visual acuit y depen ds prim arily on w h eth er the m acula w as involved in th e retinal detach m en t: once the m acula is detached, ph otoreceptors start to degen erate, im pairing visual recover y. Other concurren t dam ages to th e m acula, such as m acular holes, com m otio ret in ae, or ch oroidal rupt ure, m ay lim it final visual acuit y.

Traumatic Macular Hole A m acular h ole is a full-th ickn ess defect of th e ret in a involving th e foveal region . Trau m at ic m acu lar h ole w as first described in 1869 by Kn ap p. Sin ce th en a large n um ber of cases h ave been reported an d, despite several publicat ion s, th e exact m ech an ism of t rau m at ic m acular h ole form at ion rem ain s con t roversial. Som e th eories h ave been proposed to explain developm en t of t rau m at ic m acu lar h oles: h istorical hypoth eses claim ed t raum at ic, cyst ic degen erat ion , an d vit reous an d vascu lar et iologies. In m ore recen t t im es, Joh n son et al advan ced th at equ atorial expan sion cau ses ret in al flat ten ing an d tangen t ial t ract ion . Yam ada et al obser ved th at vit reous t ract ion m ay play a role in th e form at ion of som e t rau m at ic m acular h oles. Torn am be proposed th e experim en tal hydrat ion th eor y, st at ing th at th e altered h om eostasis du e to a break in th e in tern al ret in al layer leads to in t raret in al sw elling an d h ole form at ion . Th e in ciden ce of t rau m at ic m acular h oles varies from 1 to 9%. Pat ien ts are u su ally young an d m ale. Most t rau m at ic m acu lar h oles derive from closed-globe con t u sion inju ries from variou s in sult s, th e m ost com m on being blun t ocular t rau m a caused by a variet y of t ypes of balls . Trau m at ic m acu lar h oles can also be cau sed by acciden t al yt t rium -alu m in u m -garn et (i.e., YAG) laser burn s.

Presentation Pat ien ts u sually presen t w ith a h istor y of ocu lar t rau m a an d subsequ en t reduct ion of cen t ral visual acuit y, w h ich is u su ally 20/80 to 20/400. Oph th alm oscopic

1 Ocular Traum a

23

exam in at ion n orm ally discloses a fu ll-th ickn ess an d w ell-defin ed h ole in th e cen ter of th e m acu la. It is usually roun d or ellipt ical an d m easures 300 to 500 m . Oth er com m on fin dings are th e presen ce of sm all yellow deposits at th e level of th e ret in al pigm en t epith eliu m (RPE) an d a ring of subret in al flu id su rroun ding th e h ole. Associated epiret in al m em bran e an d operculu m are t ypically m issing. Er yth rocytes an d in flam m ator y cells m ay be presen t in th e vit reous, an d associated ocu lar inju ries are com m on ( Fig. 1.19 ).

Differential Diagnosis Idiopath ic m acu lar h ole, epiret in al m em bran e

Management A com plete oph th alm ic exam in at ion sh ou ld be perform ed, in cluding in t raocu lar pressure m easurem en t an d carefu l posterior segm en t evalu at ion . Useful im aging st u dies in clu de flu orescein angiography, opt ical coh eren ce tom ography, an d B-scan u lt rason ography. Microperim et r y m ay docum en t th e pat tern of visual acu it y loss. Vit rectom y h as been sh ow n to close t raum at ic m acular h oles effect ively an d im p rove vision . Curren t tech n ique in cludes rem oval of th e posterior hyaloid an d all epiret in al m em bran es from th e m acu lar area an d prolonged postoperat ive m acular gas tam pon ade. Spon tan eous closure of t rau m at ic m acular h oles is relat ively frequen t . Th erefore, a period of obser vat ion before deciding on su rgical in ter ven t ion is recom m en ded. Associated m acular RPE at rophy an d ch oroidal in jur y m ay lim it visual outcom es.

Intraocular Foreign Body Th e oph th alm ic pathologies caused by an in traocular foreign body arise from t w o m ech anism s: th e direct dam age caused by the penet rating injur y an d its associated com plicat ion s, depen ding on th e size, sh ap e, an d m om en t um of th e object; an d th e dam age caused by th e existen ce of an in t raocular foreign body, su ch as m etal toxicit y an d m icrobial en doph th alm it is. Met allosis bu lbi is an exten sive ocular dam age cau sed by th e ch ron ic presen ce of a react ive m et allic foreign body, m ost com m on ly m ade of iron or copper. Siderosis is ch aracterized by a ru st y brow n deposit an d discolorat ion involving th e len s an d th e iris, an d ret in al degen erat ive

Fig. 1.19

Traumatic macular hole.

24

Color Atlas of Ophthalm ology

pigm en t ar y ch anges. Ch alcosis is m ade dist in ct ive by th e presen ce of a green ish blue ring in th e periph eral corn ea (Kayser-Fleisch er ring), green ish colorat ion of th e iris, an terior subcapsular cat aract , an d refract ive deposit s on th e surface of th e ret in a. Com m on ly, in t raocu lar foreign bodies arise from h am m ering an d u sing pow er tools. Protect ive eyew ear can preven t m ost inju ries.

Presentation Pat ien ts usu ally presen t w ith a suggest ive h istor y, but oph th alm ologist s sh ould take in to accou n t th at a pat ien t m ay be u n aw are of any object pen et rat ing th e eye. Pat ien ts m ay be asym ptom at ic or com plain of decreased vision an d eye p ain . Th e foreign body m ay be visible at slit-lam p exam in at ion of th e an terior segm en t; oth er sign s in clude corn eal en t r y w ou n d, iris t ran sillu m in at ion defect , irregular pupil, len s dam age, an d an terior ch am ber react ion . Dilated in direct oph th alm oscopy m ay reveal a posterior segm en t foreign body an d associated inju ries, su ch as vit reous h em orrh age, ret in al tears, an d detach m en t ( Fig. 1.20 ).

Differential Diagnosis Oth er causes of sudden visu al loss

Management Histor y sh ou ld be carefu lly invest igated, in clu ding m ech an ism of inju r y an d foreign body com p osit ion . Ocular exam in at ion sh ould be perform ed, w ith at ten t ion to possible sites of ocu lar perforat ion . Th e an terior ch am ber an d posterior segm en t sh ou ld be evalu ated carefu lly. Th e direct visualizat ion of th e foreign body is u su ally ver y in form at ive for th e surgeon . Com pu ted tom ography is th e im aging st udy of ch oice; if it is u n available a plain x-ray m ay be con sidered in th e case of a m etallic foreign body. A carefu l u se of B-scan ult rason ography m ay be conven ien t to localize th e foreign body even if th e globe is open . If a ch ron ic in t raocu lar foreign body is fou n d, elect roret in ography is a u seful for evaluat ing ret in al fu n ct ion in th e m et allosis bu lbi. Top ical an d system ic an t ibiot ic th erapy, topical steroids, an d tet an us prophylaxis (if n eeded) are requ ired p rior to th e surgical in ter ven t ion . Th e t im ing of su rger y depen ds on th e n at u re an d locat ion of th e foreign body

Fig. 1.20

Intraocular foreign body.

1 Ocular Traum a

25

an d on th e risk of en doph th alm it is. Foreign bodies in th e an terior ch am ber sh ou ld be ext racted th rough a paracen tesis an d w ith th e au xiliar y u se of viscoelast ics to reduce possible dam age to th e len s an d th e corn eal en doth elium . Foreign bodies em bedded in th e len s do n ot autom at ically result in cat aract . If n o opacificat ion is eviden t an d th ere is n o risk of siderosis, th en th ey can be left in sit u . Vit rectom y is th e surgical procedu re of ch oice for posterior segm en t foreign bodies. In th e case of m agn et ic foreign bodies, th ey can be rem oved w ith th e u se of a st rong in t raocu lar m agn et . Proper forceps sh ou ld be u sed for n on m agn et ic foreign bodies. Associated inju ries sh ould be t reated accordingly. If possible a cu lt u re of th e foreign body or of a sam ple of vit reous m ay be usefu l if an in fect ion is su spected. Possible com plicat ion s of in t raocular foreign bodies in clude en doph th alm it is, m etallosis, corn eal scarring, cat aract , ret in al det ach m en t , an d elevated in t raocu lar pressu re.

Traumatic Optic Neuropathy Trau m a-associated lesion of th e opt ic n er ve can occur anyw h ere in th e course of th e n er ve. Th e injur y can be du e to lacerat ion of th e n er ve by a foreign body or a bon e fragm en t , com pression of th e n er ve, an d h em orrh age or perin eural edem a. It is u sually associated w ith h ead t rau m a or m idfacial fract ure. Opt ic n er ve t raum a is often du e to veh icle acciden ts, falls, recreat ion al spor ts, assau lt s, or pen et rat ing orbit al t rau m a. Th e frequ en cy of opt ic n er ve injur y in th e Un ited States occu rring in closed h ead t raum a varies from 0.5 to 5.0% ( Fig. 1.21 )

Presentation Typically, pat ien ts presen t w ith a h istor y of h ead injur y an d repor t a classic sequ en ce of even ts: th e pat ien t recovers con sciou sn ess after h ead inju r y an d experien ces a post t rau m at ic loss of visu al fun ct ion in on e eye. Visual acu it y an d color vision m ay be altered, an d visual field defects m ay be p resen t . Th e crit ical sign is a n ew ipsilateral afferen t pupillar y defect . Opt ic at rophy u su ally occu rs w eeks after ret robu lbar t rau m a. Inju ries to th e opt ic n er ve m ay be eith er direct or in direct . Direct inju ries in clude th e follow ing:

Fig. 1.21

Traumatic optic neuropathy. (Courtesy of Athi-

ya Agarwal, Dr. Agarwal’s Eye Hospital, Chennai, India)

26

Color Atlas of Ophthalm ology

Opt ic nerve avulsion : It u sually follow s severe orbit al t rau m a w ith an acute an d seriou s visu al loss. Oph th alm oscopy sh ow s th e absen ce of th e opt ic disc an d peripapillar y h em orrh age. Opt ic nerve t ransect ion : Th e vision loss is im m ediate an d com plete, an d com puted tom ograph ic scan n ing reveals th e bon e fragm en t or th e foreign body t ran sect ing th e opt ic n er ve. Opt ic nerve sheath hem orrhage : Visu al fu n ct ion abn orm alit ies m ay var y an d proptosis m ay n ot be presen t . Magn et ic reson an ce im aging m ay be h elpful in con firm ing th e diagn osis. Th e visu al loss associated w ith th is con dit ion m ay be reversible via sh eath fen est rat ion . Orbital hem orrhage : It is associated w ith proptosis an d oph th alm oplegia. Raised in t raocular pressu re m ay be in it ially con t rolled w ith topical ocular hypoten sive agen ts. If con ser vat ive m easu res fail, lateral can th otom y an d h em orrh age drain age sh ou ld be con sidered. Orbital em physem a : Injuries to th e th in bon es lim it ing th e paran asal sin us m ay produ ce a on e-w ay valve th at results in an air accum ulat ion in th e orbit w ith subsequent com pression of th e opt ic n er ve, proptosis, and elevat ion in th e in t raocular pressure. Drainage of th e in traorbital air usually resolves th is con dition . In direct opt ic n er ve inju r y usually resu lts from a blu n t t raum a to th e superior orbit al rim or th e fron t al area. Th e com pression forces are th en t ran sm it ted via orbit al bon es to th e orbital ap ex an d opt ic can al. Com pression an d con t u sion of th e n er ve produce a com par t m en t syn drom e th at result s in localized opt ic n er ve isch em ia an d edem a.

Differential Diagnosis Post t raum at ic in t raocu lar lesion s, preexist ing n europath ies, fact iou s am blyopia

Management Th e m an agem ent of in direct optic n er ve injur y sh ould in clude com plete ocular exam in ation , color vision testing, visual field testing, com puted tom ographic scan ning of the head an d orbit , an d B-scan ultrasonography. Oth er tests that m ay be useful are visual evoked potent ial an d electroretin ography. Th e t reatm en t of optic ner ve in direct injur y is som ew h at con troversial. Ver y h igh-dose cor ticosteroids have been proposed to lim it free-radical am plificat ion of th e injur y respon se. Surger y m ay be reser ved, w h en in dicated, for the cases of direct injur y or to decom press th e opt ic can al in indirect injuries. Nevertheless, th e serious com plicat ion s of surger y, such as iatrogenic dam age of th e optic n er ve or of th e adjacen t struct ures, should be carefully considered.

Orbital Trauma Orbital Fractures Blow -out fract u re of th e in ferior w all of th e orbit is th e m ost com m on of th e orbit al fract u res. Th e m edial w all of th e orbit is th e th in n est of all an d is com m on ly associated w ith m u lt iple w all fract u res of th e orbit .

1 Ocular Traum a

27

Presentation Pat ien ts presen t w ith pain (especially on at tem pted ver t ical eye m ovem en t), local ten dern ess, bin ocular double vision , eyelid sw elling an d crepit u s after n ose blow ing, an d recen t h istor y of t rau m a. Exam in at ion reveals rest ricted eye m ovem en t (especially in upw ard or lateral gaze), subcut an eou s or conjun ct ival em physem a, hypoesth esia in th e dist ribut ion of th e in fraorbital n er ve (ipsilateral ch eek an d upper lip), an d en oph th alm os (m ay in it ially be m asked by orbital edem a). Associated sign s in clu de n osebleed, eyelid edem a, an d ecchym osis. Superior rim an d orbital roof fract ures m ay sh ow hypoesth esia in th e dist ribut ion of th e sup rat roch lear or su praorbital n er ve (ipsilateral foreh ead) an d ptosis. Trism u s, m alar flat ten ing, an d a p alpable step-off deform it y of th e in ferior orbital rim are ch aracterist ic of t ripod fract u res ( Fig. 1.22A,B).

Differential Diagnosis Orbit al edem a an d h em orrh age w ith ou t a blow -ou t , cran ial n er ve palsy

Fig. 1.22 (A) Blow-out fracture inferior wall. (B) Orbital blowout fracture of the lateral wall. (Image (A) Courtesy of Soosan Jacob, Dr. Agarwal’s Eye Hospital, Chennai, India)

28

Color Atlas of Ophthalm ology

Management Com plete oph th alm ologic exam in at ion , in cluding m easurem en t of ext raocu lar m ovem en t s an d globe displacem en t . Ch eck p upils an d color vision carefu lly to rule out a t raum at ic opt ic n eu ropathy. Forced-duct ion test ing is perform ed. Com puted tom ograp h ic scan of th e orbit s is obt ain ed in all cases of su spected orbit al fract u res. Treat m en t in cludes n asal decongest an ts (e.g., pseudoeph edrin e n asal spray, t w ice a day); broad-spect ru m oral an t ibiot ics (e.g., ceph alexin 250 to 500 m g by m outh four t im es a day, or er yth rom ycin 250 to 500 m g by m ou th fou r t im es a day) for 7 days m ay be used bu t are n ot m an dator y. Apply ice packs to th e orbit for th e first 24 to 48 h ours. Su rgical repair sh ould be con sidered based on th e follow ing criteria. Im m ediate repair (u su ally w ith in 24 h ou rs) is requ ired if th ere is eviden ce by com pu ted tom ograph ic scan of en t rapped m u scle or periorbit al t issu e in com bin at ion w ith diplopia an d n on resolving bradycardia, h ear t block, n ausea, vom it ing, or syn cope. Rep air in 1 to 2 w eeks is don e if th ere is eviden ce of p ersisten t , sym ptom at ic diplopia in prim ar y or dow ngaze th at h as im proved at 1 w eek, w ith posit ive forced du ct ion s an d eviden ce of en t rapm en t on com puted tom ography or large floor fract u res (m ore th an on e h alf of th e orbital floor) th at h ave caused or are likely to cau se cosm et ically un acceptable en oph th alm os.

Intraorbital Foreign Body In t raorbital foreign bodies can occu r eith er from h igh -velocit y injuries or from relat ively m in or t raum as. Th e n at u re of th e object is fun dam en tal in determ in ing th e severit y of ocu lar an d orbit al com plicat ion s. Organ ic foreign bodies are p oorly tolerated an d often lead to in flam m at ion . Most m et als, ston e, glass, an d plast ic are u su ally in ert an d w ell tolerated. Th u s in organ ic foreign bodies t ypically cause decreased vision or orbital com plicat ion s due to direct t rau m a, w h ereas organ ic foreign bodies can easily develop orbit al in fect ion s.

Presentation Pat ien ts m ay presen t w ith a recen t h istor y of t rau m a an d severe pain . How ever, th ey can also be asym ptom at ic an d do n ot recall th e t rau m a at all. Pain , decreased vision , an d dip lopia are com m on presen t ing sym ptom s. In t raorbit al foreign bodies can be su btle an d n ot easily iden t ifiable on exam in at ion . Clin ical sign s in clude palpable ocu lar m ass, proptosis, afferen t pu pillar y defect , edem a an d ecchym osis of th e eyelids, lacerat ion of th e conju n ct iva or th e periocu lar t issu es, an d lim itat ion of th e ext raocu lar m ovem en t s. Organ ic foreign bodies m ay in duce a m arked in flam m ator y respon se w ith elevat ion of th e serum w h ite cell cou n t ( Fig. 1.23 ).

Management A detailed h istor y is n ecessar y to determ in e th e m ech an ism of injur y an d th e n at u re of th e foreign body. A com plete op h th alm ologic exam in at ion sh ould be perform ed, w ith par t icular at ten t ion to fu n duscopic exam in at ion , in t raocu lar pressu re, an d pu pillar y react ion . Ocu lar an d periocular in spect ion sh ould be addressed to discover an en t r y w ou n d. Neu rological test ing an d at ten t ion to th e pat ien t’s m en tal stat us are requ ired to evalu ate a possible n eurological inju r y. Th e im aging st u dy of ch oice is com pu ted tom ograph ic scan . It can reveal m ost foreign bodies, an d it is safe in case of m et allic foreign bodies. How ever, w ooden or plast ic foreign bodies can be m issed on com puted tom ograp h ic scan or can be m isiden t ified as in t raorbital air. On ce a m et allic foreign body h as been exclu ded, m agn et ic reso-

1 Ocular Traum a

29

A

B

C

D Fig. 1.23 Orbital foreign body removal. (A) Computed tomographic scan showing radiopaque foreign body within the orbit. (B) Foreign body approached through wound of entry. (C) Foreign body located and removed. (D) Final appearance after rem oval of both foreign bodies and closure of wound. (All images courtesy of Soosan Jacob, Dr. Agarwal’s Eye Hospital, Chennai, India; courtesy, Pablo Gili)

n an ce im aging can be u sefu l in diagn osing w ooden an d plast ic foreign bodies. Ult rason ography represen t s a com plem en t ar y test . Th e m edical t reat m en t con sists of tetan u s prophylaxis an d broad-spect rum system ic an t ibiot ic th erapy. Surgical rem oval of th e foreign body dep en ds on th e n at u re an d th e locat ion of th e object . Surgical in ter ven t ion is in dicated if sign s of in fect ion or opt ic n er ve com pression are eviden t . Moreover, all organ ic an d poorly tolerated foreign bodies sh ou ld be su rgically rem oved. Asym ptom at ic pat ien ts w ith sm all, n on organ ic in t raorbital foreign bodies do n ot requ ire any su rgical in ter ven t ion .

Retrobulbar Hemorrhage Orbit al h em orrh age in th e poten t ial sp ace su rrou n ding th e globe m ay occur after blun t t rau m a an d subsequen t inju r y to th e orbit al vessels. Th e orbit is an en closed space w ith lim ited capacit y for expan sion . Th e globe an d sept u m can be disp laced an teriorly to som e exten t , giving rise to p roptosis. How ever, th is for w ard m ovem en t is lim ited, an d th e in creased volum e resu lts in in creased in t raorbital pres-

30

Color Atlas of Ophthalm ology

su re an d com pression of th e st ru ct u res con t ain ed in th e orbit . Trau m at ic h em orrh age in th e ret robu lbar space m ay lead to acute loss of vision du e to cen t ral ret in al ar ter y occlusion , direct opt ic n er ve com pression , or com pression of th e opt ic n er ve vascu lat u re. Acu te ret robulbar h em orrh age is a rare an d sigh t-th reaten ing com plicat ion of blu n t eye t raum a, bu t it can be reversible w h en diagn osed an d t reated prom ptly.

Presentation Pat ien ts u su ally presen t w ith a recen t h istor y of t rau m a or orbital surger y, pain , an d decreased vision . Acute ret robulbar h em orrh age gives rise to m arked clin ical sign s: pain fu l exoph th alm os or proptosis w ith resistan ce to ret ropu lsion , rest rict ion of ext raocular m ovem en ts, diffu se subconjun ct ival h em orrh age, periorbit al edem a, an d ecchym osis. In t raocular pressu re is t ypically raised. Congested con jun ct ival vessels, par t ial or com p lete oph th alm oplegia, afferen t p upillar y defect , an d color vision dist urban ces m ay also be presen t . An orbit al com pu ted tom ograp h ic scan dem on st rates a ret robu lbar h em atom a ( Fig. 1.24 ).

Differential Diagnosis Orbit al cellu lit is, isolated orbit al fract ure, globe ru pt u re, carot id cavern ous fist ula, an d varix

Management Com puted tom ography is th e im aging st u dy of ch oice to determ in e ret robulbar h em orrh age an d associated orbit al inju ries. How ever, it sh ou ld be delayed in sigh tth reaten ing cases. Medical th erapy con sist s of ocu lar hypoten sive m edicat ion s, but it is con sidered an an cillar y procedu re for pat ien t s presen t ing w ith in creased orbit al pressu re an d decreased vision . Th ese pat ien ts sh ou ld u n dergo em ergen t decom pression of th e orbit al sp ace via su rgical drain age. Su rgical procedu re con sists of lateral can th otom y an d can th olysis. Early recogn it ion an d prom pt surgical in ter ven t ion preser ve an d restore vision in m ost cases.

Fig. 1.24

Retrobulbar hemorrhage.

1 Ocular Traum a

31

Posttraumatic Pulsating Exophthalmos Th e classic clin ical pict ure of p ulsat ing exoph th alm os, w h ich is a rare con dit ion , can be produ ced by post t raum at ic carot id-cavern ou s fist u las. Cerebral t raum as accou n t for 75% of carot id-cavern ous fist ulas, w h ich are in it iated by tears in th e w alls of th e in t racavern ou s in tern al carot id arter y or it s bran ch es. Th us ar terial blood m ay sh ort-circuit in th e ven ou s com plex of th e cavern ous sin uses. Oth er cau ses of pu lsat ing exoph th alm os are congen ital arterioven ous m alform at ion s, arteriosclerosis-related ret robulbar an eu r ysm s, an d n eu rofibrom atosis.

Presentation Pat ien ts t yp ically com plain , days or w eeks after t raum a, of a severe an d su dden ceph alic an d orbital pain , a roaring sou n d in th e h ead syn ch ron ous w ith th e pulse, decreased vision , diplopia, an d op h th alm oplegia. Th e pulsat ing exoph th alm os is usually reducible. In sp ect ion reveals engorged an d ch em ot ic conju n ct iva. Palpat ion of th e eye discloses a th rill, an d auscu ltat ion reveals an ocu lar or ceph alic bruit syn ch ron ou s w ith th e pulse. Oth er ocular sign s in clu de dilated ret in al vein s, disk edem a, ret in al vein occlusion s, ven ou s st asis ret in opathy, an d in creased in t raocu lar pressure due to altered outflow in th e vor tex vein s ( Fig. 1.25A,B).

A

B

Fig. 1.25 (A) Post traumatic carotid cavernous fistula. (B) Engorged vessels and chem otic conjunctiva in a patient with a traum atic carotid cavernous fistula.

32

Color Atlas of Ophthalm ology

Differential Diagnosis Pu lsat ing exop h th alm os th at is n ot t raum a related

Management A com plete oph th alm ological exam in at ion sh ou ld be perform ed, in cluding dilated fun du scopic exam in at ion an d in t raocular pressu re m easu rem en t . Th e fun ct ion of cran ial n er ves III, IV, an d VI sh ou ld be tested. Th e diagn osis can be con firm ed by ech ography, digit al angiography, an d com pu ted tom ography. Th erapy is directed to th rom bosis of th e fist u la an d n orm alizat ion of orbital h em odyn am ics via t ran sorbit al or t ran sven ou s em bolizat ion . Th e in crease in in t raocular pressu re can be in it ially t reated m edically.

2 Eyelids and Lacrimal System Christopher I. Zoum alan , Andrea Olm os, and Kim berly P. Cockerham

Ectropion Ect rop ion is a frequ en tly seen con dit ion in elderly people; it is an eyelid m alposit ion in w h ich th e eyelid (usu ally low er) is ever ted aw ay from th e globe.

Presentation Many sym ptom s are a resu lt of ch ron ic irritat ion an d exposu re to th e eye an d eyelids. Pat ien ts can com plain of excessive tearing, conju n ct ivit is, corn eal epith eliopathy, an d kerat it is, all of w h ich can resu lt in decreased vision . Th e tarsal con jun ct iva can be ch ron ically in flam ed, w ith secon dar y ch anges in cluding th icken ing an d kerat in izat ion . Th ere are five gen eral classificat ion s of ect ropion : involut ion al, cicat ricial, paralyt ic, congen ital, an d m ech an ical: Involut ional: At t ributed to age-related ch anges th at affect th e low er lids. Th ere is loss of elast icit y of th e lid com par t m en t s, an d often th ere is m edial an d lateral can th al ten don laxit y. Th e dist ract ion test an d th e sn ap-back test can determ in e an abn orm alit y in h orizon tal lid laxit y. An terior lid dist ract ion greater th an 6 to 8 m m (w h ere th e cen t ral par t of th e eyelid can be pu lled aw ay from th e globe) suggest s a posit ive lid dist ract ion test . If th e low er lid is pu lled in feriorly, th e lid sh ould qu ickly ret u rn to it s previou s posit ion . If n ot , th is m ay be in terpreted as an abn orm al sn ap-back test result ( Fig. 2.1A). Paralyt ic: Because of ipsilateral facial n er ve palsy, often associated w ith lid ret ract ion an d subsequ en t lagoph th alm os. Exposu re keratopathy an d epiph ora are com m on com p licat ion s ( Fig. 2.1B). Cicat ricial: Often , scarring from ch ron ic in flam m at ion such as t rich iasis or sun exposure can lead to a con t ract u re of th e anterior lam ellae (skin an d orbicularis m uscle). Th ere is a sh or tage of an terior lam ellae skin such th at th e low er lid can not be superiorly lifted past th e in ferior lim bus in excess of 2 m m ( Fig. 2.1C). Congenital ect ropion : May be seen in th e low er lid an d is gen erally seen in associat ion w ith con dit ion s such as bleph aroph im osis syn drom e an d ich thyosis. Mechanical: Discrete eversion of th e eyelid du e to a lid lesion .

Differential Diagnosis Make sure to rule out oth er cau ses of ect ropion such as floppy eyelid syn drom e described earlier.

Management Depen ds on exten t of ect ropion an d sym ptom s. Con ser vat ive m easures in clu de aggressive lu bricat ion of th e ocu lar surface. Surger y is often n eeded for a defin ite solut ion . Involut ion ect ropion : A variet y of su rgical opt ion s are available depen ding on th e exten t of ect ropion , h orizon tal lid laxit y, an d degree of can th al ten don laxit y. Gen eralized ect ropion can be corrected by h orizon t al lid sh or ten ing via fu llth ickn ess excision or reat tach ing it to th e lateral can th u s th rough a lateral t arsal st rip su spen sion tech n ique. 33

34

Color Atlas of Ophthalm ology

A

B

C Fig. 2.1 (A) Involutional ectropion (with punctal ectropion and stenosis). (B) Paralytic ectropion secondary to seventh nerve palsy (brow ptosis and lagophthalmos are also present). (C) Cicatricial ectropion caused by a lower eyelid scar. (Courtesy of Dr. Soosan Jacob, Dr. Agarwal’s Eye Hospital, Chennai, India)

2 Eyelids and Lacrim al System

35

Paralyt ic ect ropion : Tem porar y t reat m en t w ith a t arsorrh aphy can h elp redu ce th e exten t of exposu re keratopathy. Perm an en t cases m ay require th e use of m edial can th oplast y, m edial w edge resect ion , an d/or lateral can th al su spen sion to redu ce th e h orizon t al an d ver t ical dim en sion s of th e palpebral aper t ure. Cicat ricial ect ropion : Use of skin grafts or t ran sposit ion al flaps to restore th e n orm al an terior lam ellae Congenital ect ropion : Recon st ru ct ing th e an terior lam ellae w ith th e u se of skin graft s or t ran sposit ion al flaps

Entropion Inw ard rot at ion of th e eyelid

Presentation Th e m argin of th e eyelid an d lash es m akes con tact w ith th e globe an d, in cert ain cases, can lead to corn eal epith eliopathy an d subsequ en t u lcerat ion or pan n u s form at ion . Irrit at ion an d epiph ora are com m on presen t ing sign s. Th ey can be involu t ion al, congen it al, an d cicat ricial: Involut ional: Age-related ch anges involving degen erat ion of th e elast ic an d fibrous t issu es, u sually affect ing th e low er lid. Th e lateral an d/or m edial h orizon tal laxit y is associated w ith in creased orbicu laris ton e causing inw ard rot at ion of th e eyelid ( Fig. 2.2A). Cicat ricial: Scarring from t raum a, Steven s-Joh n son syn drom e an d oth er cicat rizing con dit ion s, ch em ical bu rn s, an d t rach om a can lead to sh or ten ing of th e posterior lam ellae ( Fig. 2.2B). Congenital: Often seen in low er lids, u sually related to th e lack of n orm al developm en t of th e ret ractor apon eu rosis.

Differential Diagnosis Epibleph aron , dist ich iasis, t rich iasis, bleph arospasm , an d r uling ou t oth er cau ses of en t ropion already m en t ion ed

Management Tem porar y t reat m en t can be ach ieved by ocular lu bricat ion an d lid taping. Bot u lin um toxin h as been used w ith su ccess in involu t ion al or congen it al cases. Surgical correct ion is often used in severe cases. Involut ional: If th ere is lit tle h orizon tal lid laxit y, t ran sverse evert ing su t ures can provide a tem porar y solu t ion . Horizon t al lid split t ing w ith in ser t ion of ever t ing sut ures provides a last ing correct ion . In cases w ith associated h orizon tal lid laxit y, h orizon tal lid sh orten ing can provide ben efit in addit ion to th e aforem en t ion ed procedures. Cicat ricial: Mild cases can be corrected w ith a t ran sverse t arsotom y (tarsal fract u re) w ith an terior rotat ion of th e lid m argin . More exten sive cases w ill often em ploy th e use of com posite grafts (m ucou s m em bran e or palate) to recon st ru ct th e dam aged posterior lam ellae. Congenital: Can be corrected w ith excision of a st rip of skin an d u n derlying orbicularis m uscle an d w ith possible fixat ion of th e skin crease to th e tarsal plate.

36

Color Atlas of Ophthalm ology

Fig. 2.2 (A) Involutional entropion with trichiasis. (B) Cicatricial entropion with associated trichiasis.

A

B

Ptosis An abn orm ally low posit ion of th e upper eyelid, w h ich m ay be congen it al or acquired

Presentation Pat ients com plain of a tired appearan ce and deficits in their superior visual field. To overcom e this, patien ts m ay elevate th eir ch in posit ion or subsequently contract their fron talis m uscle to raise th eir brow s. Certain m easurem en ts are key in the evaluation. Margin to lid reflex (MRD) is the distance from the m argin of th e upper lid to the central corn eal reflex (norm al is 4.0 to 4.5 m m ). Levator function m easures the distan ce of excursion of th e upper eyelid m argin from far dow ngaze to upgaze w h ile the fron talis m uscle is held still w ith th e exam in er’s th um b (n orm al is 14 m m or m ore). Th e palpebral fissure is th e distance from th e upper to th e low er eyelid m argin w h en the patient is in prim ar y gaze (norm al range can var y from 7 to 12 m m and is greater in w om en th an in m en ). Superior lid crease is th e vertical distan ce of th e superior lid m argin from th e lid crease in dow ngaze (norm al 8 to 10 m m ). Th ere are variou s cau ses of ptosis, in clu ding th e follow ing: Congenital: Failu re of n eu ron al m igrat ion w ith in th e levator com plex. Can be un ilateral or bilateral w ith variable severit y. Poor levator fu n ct ion an d absen t lid crease. Ptosis im proves on dow ngaze. Need to be evalu ated for am blyopia ( Fig. 2.3A).

2 Eyelids and Lacrim al System

37

A

B

C

D Fig. 2.3 (A) Congenital ptosis, right eye. (B) Bilateral ptosis, compensating with frontalis overaction. (C) Suprabrow and lid scars following conventional sling surgery (D) Presurgical and postsurgical pictures following Jacob Agarwal guided sling surgery. Note only a single scar over the forehead. ([A] Courtesy of Deborah Alcorn, MD; [C–D] Courtesy of Soosan Jacob, Dr. Agarwal’s Eye Hospital, Chennai, India)

38

Color Atlas of Ophthalm ology

Aponeurot ic: Most com m on t ype of ptosis, u su ally seen in elderly pat ien ts. Can be bilateral or u n ilateral. Du e to a deh iscen ce or disin ser t ion of th e levator apon eu rosis, u su ally th e result of involu t ion al ch anges. Norm al levator fun ct ion bu t h igh superior lid crease (> 12 m m ) ( Fig. 2.3B). Neurogenic: In n er vat ion al defect du e to an ocu lom otor n er ve palsy Myogenic: Seen in defects in th e n eurom u scular jun ct ion it self or w ith in th e levator com plex; can be du e to m yasth en ia gravis or m uscu lar dyst rophy Mechanical: Secon dar y to a gravit y m ass effect or con t ract ion from a scar

Differential Diagnosis It is im port an t to differen t iate t ru e ptosis from pseudoptosis, w h ich can be caused by con t ralateral lid ret ract ion , ipsilateral hypot ropia, brow ptosis, an d derm atoch alasis. Oth er differen t ial diagn oses in clu de Marcu s-Gun n jaw w in king syn drom e, aberran t th ird or seven th n er ve regen erat ion , an d bleph arop h im osis syn drom e .

Management Depen ds on th e severit y of th e ptosis an d its et iology. Usu ally severe ptosis w ith poor levator fu n ct ion w ill n eed to be addressed by a fron t alis-sling procedure. Levator resect ion is in dicated in cases w ith fair to good levator fun ct ion (at least 5 m m ). Cases w ith reason ably good or excellen t levator fu n ct ion can be addressed by eith er a posterior approach or an an terior apon eu rosis repair. Congenital ptosis: Usu ally n eeds to be addressed early if am blyopia is a con cern , especially in u n ilateral cases. Depen ding on th e levator fu n ct ion , differen t procedu res can effect ively correct th e ptosis. Poor levator fun ct ion (< 4 m m ) w ill requ ire a fron talis-sling procedu re, w h ereas fair levator fu n ct ion (> 4 m m ) m ay be corrected w ith a levator resect ion . Aponeurot ic ptosis: Several opt ion s are available, depen ding on th e severit y of th e ptosis. For in stan ce, a posterior approach (e.g., Fasan ella-Ser vat procedu re or Mü ller-conjun ct ival resect ion ) can correct m ild cases. Altern at ively, an an terior app roach w ith rein ser t ion or advan cem en t of th e apon eurosis can correct cases w ith excellen t levator fu n ct ion . Ja cob-Aga rwa l Technique of Guided Sling Surgery with Single Sta b Incision Fron t alis m u scle su spen sion procedu re is th e gold st an dard for th e t reat m en t of congen ital ptosis w ith poor levator fun ct ion . It creates a lin k bet w een th e fron t alis m u scle an d th e tarsus of th e up per eyelid, w h ich allow s for a bet ter eyelid posit ion in prim ar y gaze. Th e Jacob-Agar w al tech n iqu e differs from th e conven t ion al p rocedu res by th e use of a single-stab in cision in m aking th e pen t agon an d guiding th e silicon e sling in th e su rgical plan e w ith on e extern al in cision w h ile suspen ding th e fron talis m uscle ( Fig. 2.3C,D). Surgica l Technique A pen t agon sh ape is m arked over th e skin w ith a m arker. A single su praeyebrow st ab in cision of ~2.5 to 3.0 m m is pu t on th e su perior m ark of th e pen t agon ~5 m m from th e eyebrow, an d a subp eriosteal pocket is dissected upw ard ( Fig. 2.4 ). A sterile fron t alis su spen sion set (Seiff) dipped in an t ibiot ic is used as th e sling m aterial. Th is h as a long, solid silicon e rod/t u be w ith a length of 40cm (15¾ in ) an d a diam eter if .80m m (.032 in ), w ith a stain less steel n eedle on eith er en d (20G × 2½ in ). Th e silicon e t ube is provided w ith a silicon e sleeve, w h ich is rem oved from th e t ube before su rger y begin s ( Fig. 2.5 ). Th e n eedle is first passed th rough th e ep i-t arsal t issu e bet w een th e m arks m ade on th e u p per eyelid. Th e lid is ever ted an d ch ecked to ascer t ain th at it h as n ot gon e th rough th e fu ll th ickn ess of th e t arsu s. W ith a lid gu ard beh in d th e lid, th e m edial en d of th e n eedle is th en in ser ted th rough th e m edial n eedle exit p oin t on

2 Eyelids and Lacrim al System

39

A

B

C

D Fig. 2.4 (A) Upper eyelid elevation and contour checked preoperatively. (B) Pentagonal shape as in conventional sling surgery is m arked over the skin. (C) Single supraeyebrow stab incision of ~ 2.5 to 3.0 mm is put on the superior mark of the pentagon ~ 5 mm from the eyebrow. (D) Subperiosteal pocket dissected upward. (All images courtesy of Soosan Jacob, Dr. Agarwal’s Eye Hospital, Chennai, India)

Fig. 2.5 Seiff Silicone frontalis suspension set (BD Ophthalmic Systems, Bidford on Avon, UK). (Courtesy of Soosan Jacob, Dr. Agarwal’s Eye Hospital, Chennai, India)

40

Color Atlas of Ophthalm ology

th e eyelid an d advan ced u pw ard, dipp ing beh in d th e sept u m , to th e m ark m ade on th e m edial eyebrow. W h en th e u p per m edial corn er of th e p en t agon is reach ed, th e n eedle is th en t u rn ed an d gu ided in th e sam e su rgical plan e (w ith ou t exteriorizing), u sing a com bin at ion of visu alizat ion an d p alpat ion an d is brough t ou t th rough th e cen t ral su p rabrow in cision ( Fig. 2.6 ). Th e sam e p rocedu re is repeated w ith th e n eedle on th e lateral side so th at it t races th e path of th e lateral lim b of th e pen t agon , dip s beh in d th e orbit al sept u m , t u rn s in direct ion above th e su praorbit al rim , an d th en exter iorizes th rough th e cen t ral sup rabrow in cision . Th e su rgeon’s n on dom in an t in dex finger can be u sed to p alp ate th e n eedle as it is being advan ced. Th e t w o en ds of th e silicon e rod are th readed th rough th e silicon e sleeve, an d th e lid m argin an d con tou r are adju sted according to th e am ou n t of correct ion requ ired an d for m axim al cosm esis. Th e t w o en ds of th e silicon e rod s are also kn ot ted togeth er, an d a 6-0 silk su t u re is t ied bet w een th e kn ot s to p reven t late slip page. Th e sleeve w ith th e kn ot s is th en bu ried in to th e su bp eriosteal pocket . If n eed ed, on e m ay also h itch th e silicon e t u be kn ot to th e u n derlying periosteu m . Th e single su prabrow st ab in cision is closed w ith silk su t u re or fibrin

A

B

C

D Fig. 2.6 (A) The needle is passed through the superficial tarsal tissue bet ween the marks made on the upper eyelid. (B) The medial end of the tube is advanced through the medial needle exit point on the eyelid and advanced upward, dipping behind the septum, to the mark m ade on the m edial eyebrow. (C) When the upper medial corner of the pentagon is reached, the needle is then turned and guided in the same surgical plane (without exteriorizing) using a combination of visualization and palpation. (D) The needle is brought out through the central suprabrow incision. The same is repeated on the other side. (All images courtesy of Soosan Jacob, Dr. Agarwal’s Eye Hospital, Chennai, India)

2 Eyelids and Lacrim al System

41

glu e to m in im ize scar form at ion . Th e Oth er n eed le pu n ct u re sites n eed n ot be su t u red ( Fig. 2.7 ). Th e advan tage of th e tech n ique is th at w ith m in im al skin in cision s an d less su rgical t im e, th e clin ical ou tcom e of a conven t ion al fron talis sling procedu re is obt ain ed. Postoperat ive lid an d brow edem a, ecchym osis, pain , su t u re-related com plicat ion s, an d scarring du e to m u lt iple in cision s an d su t u res can be avoided. Th e tech n iqu e can be perform ed in all eyes w ith ptosis an d poor levator fu n ct ion th at n ecessit ate a fron talis sling. Th e stab in cision used is on ly ~2.5 to 3.0 m m . It is th us advan tageou s over th e conven t ion al procedure th at involves five stab in cision s, w h ich creates m ore bleeding an d edem a in th e im m ediate postoperat ive period an d m ore scarring in th e late postoperat ive period. Mild im m ediate postoperat ive edem a m ay occu r, but it gen erally resolves spon tan eously w ith in 24 h ou rs. Th ough silicon e m aterial for fron t alis sling su spen sion h as been t ried successfully, th e gu ided sling procedure w ith a silicon e sling h as n ot been rep or ted. Th ere h ave been report s in th e past of sling procedures u sing a m in im u m or n o in cision . Th e Jacob-Agar w al tech n iqu e differs from th ese procedures in th at th e en ds of th e sling m aterial are un ited in th e cen t ral foreh ead in cision (rath er th an in th e eyelid in cision as previou sly described by oth er au th ors), w h ich provides an up w ard direct ion of t ract ion for bet ter lid h eigh t an d con tour. Also, th e m aterial used for th e sling is th e com m on ly available Seiff silicon e su spen sion set , w h ich ptosis su rgeon s are m ore fam iliar an d com for t able w ith th an n on absorbable sut u re. Lid closure is also bet ter w ith silicon e sling m aterial rath er th an w ith n on absorbable slings. Th is tech n iqu e th erefore provides bet ter aesth et ic an d fun ct ion al resu lts in pat ien t s w ith poor levator fu n ct ion an d congen ital ptosis. It is also opt im al for pat ien ts w ith m yopath ies, m yasth en ia, th ird n er ve palsy, an d sim ilar con dit ion s w h ere silicon e suspen sion is conven t ion ally preferred over oth er sling m aterials becau se of its in h eren t elast icit y. Th is tech n ique is un ique in its sim ple learn ing cur ve, good cosm esis, sm aller n um ber of su t u res w ith bet ter fun ct ion al result s w h ile ret ain ing th e u sual advan tages of stan dard sling procedu res.

A

B Fig. 2.7 (A) The t wo ends of the rod are passed through the silicone sleeve, and the lid margin and contour are adjusted according to the amount of correction required and for maximal cosm esis. (B) Measurement of the incision site is shown. (Both images courtesy of Soosan Jacob, Dr. Agarwal’s Eye Hospital, Chennai, India)

42

Color Atlas of Ophthalm ology

Dermatochalasis Th is is a ver y com m on con dit ion seen in elderly pat ien ts, u su ally p resen t s bilaterally. It m ay be asym ptom at ic.

Presentation Pat ien ts presen t w ith redu n dan t upper lid skin th at can be associated w ith fat h ern iat ion th rough a w eak sept um . In m oderate to severe cases, th e excess lid can obst ruct th e sup erior visu al field ( Fig. 2.8A,B,C).

Differential Diagnosis Acqu ired or congen ital ptosis, eyebrow ptosis, floppy eyelid syn drom e, prolapsed lacrim al glan d an d eyelid edem a.

A

B

C

Fig. 2.8 (A) Bilateral dermatochalasis. (B) Dermatochalasis and brow ptosis. (C) Dermatochalasis with lash ptosis.

2 Eyelids and Lacrim al System

43

Management In cludes bleph aroplast y to rem ove excess skin . Pat ien ts often h ave a com bin ed ptosis an d n eed to be evaluated for th is com pon en t . If so, th ey m ay ben efit from ptosis su rger y as w ell.

Eyelid Retraction Eyelid ret ract ion occurs w h en th e upper lids rest above or at th e level of th e superior lim bus.

Presentation Pat ien t s often h ave oth er sign s, in clu ding proptosis, in ferior scleral sh ow, lagoph th alm os w ith resu lt ing exp osure keratopathy, an d oth er sign s of acu te in flam m at ion (conju n ct ivit is, ch em osis). Th is is m ost com m on ly seen in thyroid eye disease ( Fig. 2.9 ).

Differential Diagnosis Thyroid eye disease, as already m en t ion ed. Oth er causes in clude con t ralateral ptosis w ith ipsilateral lid ret ract ion secon dar y to Herring’s law, aberran t th ird n er ve regen erat ion , cicat ricial ch anges or scarring of th e upper lid, sym path etom im et ic oph th alm ic drops, overcorrect ion of ptosis su rger y, ch ron ic con tact len s u se, Parin au d syn drom e, an d Miller Fish er varian t of Guillain -Barré syn drom e.

Management W h en sym ptom s are m ild, art ificial lubricat ion of th e ocular surface can often am eliorate th e sym ptom s. Severe cases w ith lit tle p roptosis m ay requ ire eyelid su rger y. Th ere h ave been a variet y of tech n iqu es described to su rgically correct eyelid ret ract ion . Both posterior (t ran sconju n ct ival) an d an terior (cut an eou s) approach es h ave been described. Both approach es involve a variable degree of levator m u scle com plex recession an d/or m u llerectom y to correct th e ret ract ion .

Fig. 2.9 disease.

Eyelid retraction and proptosis from thyroid eye

44

Color Atlas of Ophthalm ology

Floppy Eyelid Syndrome Th is syn drom e is a frequ en tly m isdiagn osed con dit ion th at can presen t un ilaterally or bilaterally. It t ypically affect s obese m en w h o m ay also su ffer from obst ru ct ive sleep apn ea an d is also associated w ith tobacco sm oke.

Presentation Th e upper eyelids are loose an d rubber y an d ten d to evert during sleep. Th e lax upper eyelid is ever ted easily w h en pulled superiorly tow ard th e eyebrow du ring physical exam in at ion . Th e soft an d ru bber y tarsal plate can also be folded up on itself. Th is resu lts in t raum a to th e exposed tarsal conju n ct iva w ith su bsequen t ch ron ic papillar y conjun ct ivit is ( Fig. 2.10A,B,C).

A Fig. 2.10 (A) Floppy eyelid syndrome. (B) Floppy eyelid syndrome with papillary tarsal conjunctival changes. (C) Floppy eyelid syndrome. (Image [A] courtesy of Soosan Jacob, Dr. Agarwal’s Eye Hospital, Chennai, India; Image [C] courtesy of Pablo Gili)

B

C

2 Eyelids and Lacrim al System

45

Differential Diagnosis Allergic conjun ct ivit is, gian t p apillar y conjun ct ivit is, atop ic keratoconju n ct ivit is, su perior lim bic kerat it is, ect ropion , derm atoch alasis

Management In m ild cases, taping of th e eyelids or n oct u rn al eye sh ields can am eliorate sym ptom s. Severe cases m ay requ ire h orizon t al lid sh orten ing using fu ll-th ickn ess pen tagon al w edge resect ion in th e u pper an d low er eyelids.

Chalazion Ch ron ic, lipogran u lom atous in flam m ator y lesion located w ith in th e tarsus. Th ese occur frequ en tly an d are cau sed by a bu ildup or blockage of secret ion from m eibom ian glan d orifices.

Presentation Can be seen in all ages, and presents w ith a gradually enlarging painless nodule. If large enough, upper eyelid lesions can induce astigm atism . The lesions can be m ultiple and bilateral. Make sure to evaluate the patient for blepharitis, ocular rosacea, and seborrheic derm atitis, w hich can predispose patients to chalazia (Fig. 2.11A,B).

Fig. 2.11 (A) Upper eyelid chalazion. (B) Pyogenic granulom a. (Courtesy of Soosan JaA

B

cob, Dr. Agarwal’s Eye Hospital, Chennai, India)

46

Color Atlas of Ophthalm ology

Differential Diagnosis Hordeolu m (ten der to palpat ion ) an d sebaceou s cell carcin om a

Management Lesion s th at are n ew or sm all m ay resolve spon t an eou sly. Warm com presses of 30 m in utes, fou r t im es per day, m ay h elp relieve or reduce th e in flam m at ion . Topical an t ibiot ics can also be u sed. Persisten t lesion s m ay be t reated eith er by excision (posterior approach w ith th e u se of a ch alazia clam p) or th rough in t ralesion al steroid inject ion (0.5 to 2.0 m L t riam cin olon e aceton ide, 5 m g/m L). Biopsy of recu rren t lesion s sh ou ld be don e to ru le out a sebaceou s cell carcin om a .

Hordeolum Painful nodules as a result of an acute bacterial infection, m ost com m only from Staphylococcus aureus. In contrast, chalazia are chronic lesions and t ypically not painful.

Presentation Th ese can eith er presen t as an extern al h ordeolum (m argin of eyelid) or in tern al h ordeolum (seen by ever t ing eyelid). In tern al h ordeolu m s can cause severe con jun ct ivit is or ch em osis. Usu ally associated w ith m eibom ian glan d dysfun ct ion an d bleph arit is ( Fig. 2.12 ).

Differential Diagnosis Ch alazion (n ot ten der to palpat ion ), blep h arit is

Management Most cases are self-lim ited an d w ill resolve w ith in 5 to 7 days. Lid hygien e an d w arm com p resses can h elp in th eir resolut ion . Som e m ay persist an d becom e cystlike, w h ich m ay requ ire su rgical in cision an d cu ret t age (see above t reat m en t for

Fig. 2.12

External hordeolum.

2 Eyelids and Lacrim al System

47

ch alazion ). Treat m en t of accom panying bleph arit is an d m eibom ian glan d dysfun ct ion w ill h elp to p reven t recu rren ces.

Eyelid Edema Sw elling of eyelids due to flu id collect ion w ith in th e su bcu tan eous t issu es

Presentation The thin skin and loose subcutaneous tissue are susceptible to water accum ulation and edem a, especially in the upper lids. Sym ptom s can be either inflam m atory or noninflam m atory. Inflam m atory signs can include redness, sensation of heat and pain, and m arked unilaterality. This can be secondary to infections such as cellulitis (preseptal or orbital), eczem a, and abscess. In contrast, noninflam m atory signs include pale skin color, cool skin, absence of pain, and bilaterality. This can be seen m ore com m only w ith system ic disorders (heart, kidneys, thyroid eye disease) and secondary to an allergic response. Recurrent episodes of persistent eyelid edem a can result in stretching of the overlying thin skin and m ay result in blepharochalasis (Fig. 2.13A,B).

Differential Diagnosis Allergic respon se, h ordeolu m , abscess, eczem a, dacr yocyst it is, cellu lit is (preseptal or orbit al), thyroid eye disease, derm atoch alasis

Management Treat th e u n derlying system ic or in flam m ator y disorder.

A

B Fig. 2.13 (A) Left eyelid edema in a female with thyroid eye disease. (B) Left eyelid edema in a male with acute thyroid eye disease.

48

Color Atlas of Ophthalm ology

Blepharoptosis and Blepharophimosis Syndrome A rare, autosom al dom in an t , congen ital syn drom e

Presentation Moderate to severe ptosis (sym m et rical) w ith poor levator fu n ct ion , sh or ten ed h orizon t al palpebral fissures, lateral ect ropion of th e low er lids, telecan th us (abn orm ally in creased dist an ce bet w een th e m edial can th i of both eyelids), an d epican th al inversus (see th e sect ion on Epican th us) ( Fig. 2.14 ).

Fig. 2.14

Blepharophimosis syndrome. (Courtesy of Soosan Ja-

cob, Dr. Agarwal’s Eye Hospital, Chennai, India)

Differential Diagnosis Congen it al or acquired ptosis, congen it al fibrosis syn drom e

Management In it ially involves t reat m en t of epican th al folds an d telecan th u s follow ed later (u sually a few m on th s) by bilateral fron t alis su spen sion . Th e p at ien ts n eed to be screen ed an d follow ed up for am blyopia because it can develop in u p to 50% of cases.

Epiblepharon An ext ra h orizon t al fold of skin exten ding across th e an terior lid m argin resu lt ing in ver t ically orien ted lash es

Presentation A com m on fin ding am ong eastern Asian s, th is sh ou ld n ot be m ist aken for congen ital or acqu ired en t ropion . Un like en t ropion , th e lash es do n ot m ake con t act w ith th e corn ea in prim ar y gaze, bu t th ey often can in dow ngaze. Th e n orm al locat ion of th e lid becom es apparen t w h en th e fold of skin is pulled dow n (Fig. 2.15 ).

2 Eyelids and Lacrim al System

Fig. 2.15

49

Epiblepharon. (Courtesy of

Soosan Jacob, Dr. Agarwal’s Eye Hospital, Chennai, India)

Differential Diagnosis Congen it al or acqu ired en t ropion

Management Man agem en t is usually n ot requ ired because m ost cases spon tan eously resolve w ith age. How ever, persisten t cases or on es th at cau se sym ptom s (corn eal epith eliopathy) are su rgically m an aged by excising a st rip of skin an d orbicularis m u scle (an terior lam ellar resect ion ) w ith or w ith out fixat ion of th e skin to th e tarsus.

Epicanthus Th ese are bilateral w ebs of skin th at exten d from th e up per to low er eyelids tow ard th e m edial can th u s. If large en ough , th ey cau se pseudoesot ropia. Th ey can be seen in syn drom es su ch as bleph aroph im osis syn drom e an d t risom y 21.

Presentation Epican th al folds can presen t in fou r variou s su bt ypes: Epicanthus tarsalis: Th e folds origin ate in th e upp er m edial eyelid an d exten d in to th e m edial can th us. Th is is th e m ost com m on t ype seen in eastern Asian s ( Fig. 2.16 ). Epicanthus inversus: Th is t ype origin ates in th e low er eyelid an d exten ds in to th e m edial can th u s. Th is is associated w ith blep h aroph im osis syn drom e. Epicanthus palpebralis: Th e folds exten d from th e u pper to low er eyelids in a sym m et ric dist ribu t ion . Epicanthus superciliaris: Th ese are broad folds th at exten d from th e eyebrow dow n to th e low er orbital rim .

Differential Diagnosis Epibleph aron . Rule ou t syn drom es associated w ith epican th al folds su ch as bleph aroph im osis syn drom e an d t risom y 21.

50

Color Atlas of Ophthalm ology

Fig. 2.16

Epicanthus tarsalis.

Management Epican th u s u su ally resolves by th e age of 4. Several su rgical procedu res such as Z- or Y-V-plast ies can be em ployed to repair persisten t cases.

Eyelid Coloboma Th is is a par t ial- or fu ll-th ickn ess eyelid defect due to an em br yon ic cleft , u sually t riangular in sh ape, w ith it s base at th e m argin .

Presentation Th ese are rare defect s, u su ally congen ital an d result ing from a lack of closu re of th e opt ic cu p. Th ey are m ost com m on ly seen in th e u pper lids (at th e ju n ct ion of th e m iddle an d outer th irds), eith er in isolat ion or w ith oth er syn drom es. Kerat in izat ion or exposure keratopathy can occu r depen ding on th e severit y of th e colobom a. Colobom as can be accom pan ied by addit ion al deform it ies such as m icroph th alm os or derm oid cysts ( Fig. 2.17 ).

Fig. 2.17

Upper eyelid coloboma. (Courtesy of Soosan Jacob, Dr.

Agarwal’s Eye Hospital, Chennai, India)

2 Eyelids and Lacrim al System

51

Differential Diagnosis Ableph aron (congen it al absen ce of eyelid), t rau m a. Ru le out oth er syn drom es associated w ith colobom as such as Golden h ar syn drom e (ocu loau riculover tebral dysplasia) an d Fran cesch et t i syn drom e (m an dibulofacial dystosis).

Management Con ser vat ive t reat m en t w ith th e u se of lubricat ing drops an d oin t m en ts if sm all an d th ere is n o risk of exposure keratopathy. How ever, defects u sually n eed to be closed by prim ar y, direct closure or, if large en ough , w ill requ ire th e use of skin graft s or rotat ion flaps or both .

Eyelid Tumors Papilloma Th e m ost com m on ben ign t u m or of th e eyelids.

Presentation Th ese presen t as a pedu n culated or sessile (broad-based) lesion . Th ey are a ben ign t u m or of epith elial origin (Fig. 2.18A,B).

Differential Diagnosis Molluscu m con tagiosum , ch alazion , squ am ous cell carcin om a, basal cell carcin om a

Management Man agem en t can con sist of obser vat ion or elect ive rem oval by excision . Sh ave biopsies can be perform ed if th e diagn osis is u n cer tain .

A

B Fig. 2.18

(A) Papilloma. (B) Upper eyelid margin papilloma.

52

Color Atlas of Ophthalm ology

Seborrheic Keratosis Slow -grow ing, discrete, greasy lesion w ith a friable surface

Presentation Th ese often ap pear to be “st u ck on ” th e skin . Th e lesion is usually brow n an d flat but can often be pedu n cu lated ( Fig. 2.19A,B).

Differential Diagnosis Nevus, m elanom a, squam ous cell carcinom a, acrochordon (skin tag), actinic keratosis

Management Cu ret t age an d excision are cu rat ive.

Actinic Keratosis Also term ed solar keratosis, th is is a “prem align an t” lesion seen in fair-skin n ed in dividuals w h o h ave been exposed to excessive su n ligh t .

Presentation Lesion s can be scaly, flat , w ith hyperkeratot ic feat u res. Th ey often begin as sm all, rough m acu les or papules ( Fig. 2.20 ).

Fig. 2.19 (A) Seborrheic keratosis. (B) Seborrheic keratosis, lower eyelid.

A

B

2 Eyelids and Lacrim al System

53

Fig. 2.20 Actinic keratosis (arrow s depict lesions).

Differential Diagnosis Basal cell carcinom a, cutaneous horn, squam ous cell carcinom a, seborrheic keratosis

Management Th ese lesion s sh ou ld be biopsied for diagn osis an d t reated w ith com plete excision or cr yoth erapy.

Keratoacanthoma Th is is an un com m on but rapidly grow ing lesion w ith a cen t ral kerat in m ass.

Presentation Th ey are often seen in fair-skin n ed in dividuals w ith excessive su n ligh t exp osure. Th ey can often regress spon tan eously, leaving a cen t ral, su n ken scar. Th ey are h istopath ologically in clu ded in th e spect rum of squ am ou s cell carcin om as an d clin ically appear sim ilar to squ am ous cell carcin om as ( Fig. 2.21 ).

Fig. 2.21

Keratoacanthoma.

54

Color Atlas of Ophthalm ology

Differential Diagnosis Basal cell carcin om a, cu tan eous h orn , act in ic keratosis, squ am ous cell carcin om a, an d seborrh eic keratosis

Management Biopsy su spicious lesion s. Com plete su rgical excision w ith free m argin s is recom m en ded. Laser or cr yoth erapy can be applied to sm all lesion s.

Molluscum Contagiosum Th ese are virally t ran sm it ted lesion s u sually seen in you nger pat ien t s (teen agers an d ch ildren ) or in im m u n ocom prom ised pat ien ts.

Presentation Th ey are n on in flam m ator y, sm ooth , p early, dom e-sh ap ed papules w ith cen t ral depression s often fou n d n ear th e upper an d low er eyelids. Th ey are t ran sm it ted by direct con t act an d are u su ally asym ptom at ic but can be associated w ith ch ron ic conju n ct ivit is (Fig. 2.22 ).

Fig. 2.22

Molluscum contagiosum.

Differential Diagnosis Basal cell carcin om a, squam ou s cell carcin om a, papillom a

Management Lesion s can be excised by a curet or cr yoth erapy.

Nevi Nevi are ben ign lesion s th at occu r w ith in th e epith elium an d derm is.

Presentation Th e lesion s are derived from m elan ocyt ic cells an d can be eith er pigm en ted or n onpigm en ted. Nevi can be h istologically classified as ju n ct ion al, com pou n d, an d

2 Eyelids and Lacrim al System

55

in t raderm al. In t raderm al n evi are con fin ed w ith in th e derm al layer. Th ey are usually n onpigm en ted an d elevated. Th ey h ave n o m align an t poten t ial (Fig. 2.23A). Jun ct ion al n evi are w ell circum scribed, flat , an d u n iform ly brow n . Th ey are located at th e ju n ct ion of th e epiderm is an d derm al layers an d h ave a low poten t ial for m align an cy ( Fig. 2.23B). Com poun d n evi h ave both ju n ct ion al an d in t raderm al involvem en t ( Fig. 2.23C).

A

B

C Fig. 2.23 (A) Intraderm al nevi. (B) Junctional nevus. (C) Caruncular compound nevus.

56

Color Atlas of Ophthalm ology

Differential Diagnosis Malign an t m elan om a, basal cell carcin om a, ben ign lesion s

Management Carefu lly docu m en t th e size of th e lesion w ith ph otograph s. Su rgically rem ove lesion s th at in crease in size.

Nevus of Ota Congen it al oculoderm al m elan ocytosis th at involves both skin (derm is) an d eye (ep isclera, sclera, an d uveal t issu es)

Presentation Pat ien ts presen t w ith deep, u n ilateral hyp erpigm en tat ion of th e eyelid skin an d ocu lar st ru ct u res. Th ese n evi are associated w ith iris hyperch rom ia an d fu n du s hyperpigm en t at ion . Pat ien t s are at an in creased risk of glaucom a an d, th ough rare, m elan om a ( Fig. 2.24 ).

Differential Diagnosis Malign an t m elan om a

Management Follow regularly for m align an t ch ange an d glaucom a screen ing.

Fig. 2.24 Nevus of Ota. Note the relative sparing of dermal involve ment.

2 Eyelids and Lacrim al System

57

Xanthelasma Xan th elasm a is a com m on ly seen con dit ion th at is frequen tly bilateral an d often seen in elderly pat ien ts or th ose w ith hyperlip idem ia. How ever, m ost pat ien t s w ith xan th elasm a are n orm olipoprotein em ic. Th ese can rarely be th e presen t ing sign of xan th ogran ulom atou s disease.

Presentation Yellow ish su bcu t an eou s plaques are often foun d aroun d th e eyelids, especially arou n d th e m edial can th al areas.

Differential Diagnosis Am yloidosis, eccrin e hydrocystom a, at ypical lym ph oid in filt rate sarcoid

Management Th e lesion s can be su rgically rem oved elect ively; h ow ever, recurren ces are com m on . Altern at ively, excision or dest r uct ion by carbon dioxide, argon laser, cr yoth erapy, an d ch em ical cau terizat ion (ch lorin ated acet ic acids) can be perform ed, th ough scarring an d hyperpigm en tat ion can occu r.

Cysts—Moll/Zeis/Sebaceous Cysts of th e glan ds can resu lt in roun d, clear, an d t ran sillum in at ing lesion s .

Presentation Th ere are various t yp es of du ctal cysts. Cysts of Moll (ap ocrin e sw eat glan d hydrocystom a) are usually foun d on th e an terior lid m argin an d t ran sillu m in ate w ell. Th ey can be foun d in th e m edial can th al angle, an d gravit y can often result in ect ropion . Eccrin e sw eat glan ds, th ough n ot con fin ed to th e lid m argin , appear like apocrin e cyst s. Cysts of Zeis an d sebaceous cyst s con tain oily secret ion s an d th erefore do n ot t ran sillum in ate ( Fig. 2.25A,B,C).

Differential Diagnosis Ben ign an d m align an t lesion s, ch alazion , extern al h ordeolum

Management Warm com presses an d topical an t ibiot ics are h elpfu l. Marsupializat ion of th e cyst s is usu ally curat ive. Suspiciou s lesion s sh ou ld be sen t for biopsy.

Syringoma Syringom as are ben ign skin t u m ors of eccrin e differen t iat ion , m ore often fou n d in w om en .

Presentation Skin -color papu les are u su ally located on th e eyelids an d can in crease in size an d qu an t it y.

58

Color Atlas of Ophthalm ology

Fig. 2.25 (A) Apocrine hydrocystoma. (B) Eccrine hydrocystoma. (C) Sebaceous hydrocystoma.

A

B

C

Differential Diagnosis Verr uca, xan th elasm a, cylin drom a

Management Pap ules can be elect ively rem oved by su rgical excision or elect rodissect ion an d curet tage. Th e lesion s can recur.

Neurofibroma Neu rofibrom as are in filt rat ive n er ve cell t u m ors th at are largely com p osed of Sch w an n cells.

2 Eyelids and Lacrim al System

59

Fig. 2.26

Infiltrative neurofibroma of left orbit. (Courtesy of Deborah Alcorn, MD).

Presentation Th e t um ors u sually occur early in life an d can be eith er n odu lar or plexiform . Th ey can involve th e upper lid (classic-sh aped appearan ce) an d frequen tly cau se a m ech an ical ptosis ( Fig. 2.26 ).

Differential Diagnosis Cap illar y h em angiom a, lym ph om a, rh abdom yosarcom a

Management Su rgical excision can be at tem pted, bu t th ese lesion s are ver y difficu lt to rem ove su ccessfully.

Tumors Basal Cell Carcinoma Th is is th e m ost com m on eyelid m align an cy. Th ere is an in creased risk in people w ith fair skin an d in in dividuals w ith in creased exposure to ult raviolet radiat ion (ch ron ic skin dam age).

Presentation Th ese t ypically presen t as a firm lesion w ith raised m argin s. A cen t ral crater w ith su perficial vascularizat ion or u lcerat ion can often be seen . Loss of eyelash es (m adarosis) alm ost alw ays suggest s m align an cy. Most com m on ly seen (in decreasing order of relat ive frequ en cy) in th e low er eyelids, m edial can th u s, up per eyelid, an d lateral can th us ( Fig. 2.27A,B,C,D).

Differential Diagnosis Nevu s, papillom a, keratoacon th om a, m align an t m elan om a, squ am ous cell carcin om a

60

Color Atlas of Ophthalm ology

Fig. 2.27 (A) Basal cell carcinom a. (B) Ulcerating basal cell carcinoma. (C) Ulcerating basal cell carcinoma with necrosis of surrounding skin. (D) Ulcerating basal cell carcinoma with umbilicated, central crater.

A

B

C

D

2 Eyelids and Lacrim al System

61

Management Biopsy su spicious lesion s. Su rgical excision w ith free m argin s (recom m en ded 3 to 5 m m ) of h ealthy t issu e sh ould be p erform ed. Altern at ive t reat m en t m odalit ies in clu de cr yoth erapy, elect rodissect ion , cu ret t age, Moh s m icrograph ic surger y, an d radioth erapy. Th ese lesion s h ave rare m etast at ic poten t ial.

Squamous Cell Carcinoma Squ am ous cell carcin om as com prise less th an 5%of eyelid m align an cies. Sim ilar to basal cell carcin om a, th e prim ar y cau se of m ost squam ou s cell carcin om a is cu m ulat ive lifet im e su n exposu re, especially in fair-skin n ed in dividu als.

Presentation Lesion s can presen t as clin ically sim ilar to basal cell carcin om as, bu t th ey com m on ly grow rapidly w ith spread to region al lym ph n odes. Th ey can also exten d in to th e in t racran ial cavit y via perin eural spread ( Fig. 2.28A,B,C). Clin ically th ese can presen t as th ree su bt ypes:

A

B

Fig. 2.28 (A) Squamous cell carcinoma. (B) Squamous cell carcinom a of upper eyelid. (C) Cystic squam ous cell carcinoma.

C

62

Color Atlas of Ophthalm ology

Plaquelike : Scaly, hyperkeratot ic lesion s at site of preexist ing act in ic keratosis Nodular: Hyperkeratot ic n odu le w ith cru st ing fissu res Ulcerat ing: Well-defin ed evert ing borders w ith an er yth em atous an d ulcerated base

Differential Diagnosis Basal cell carcin om a, keratoacan th om a, act in ic keratosis

Management Su rgical excision w ith free m argin s (recom m en ded 3 to 5 m m ) of h ealthy t issu e or Moh s m icrograph ic surger y sh ou ld be p erform ed. Lesion s n ot com pletely resectable can be t reated w ith adjun ct ive radiat ion or cr yoth erapy or both .

Sebaceous Cell Carcinoma Th ese are older-grow ing lesion s frequ en tly arising from th e m eibom ian glan ds an d usually seen in th e upper eyelids. Th ey are m ore com m on ly seen in older w h ite w om en . Th ey com prise approxim ately 5% of eyelid m align an cies. Th ere is often a delay in diagn osis given its in sidiou s clin ical appearan ce.

Presentation Can presen t eith er as a n odu lar or a pagetoid spreading m eibom ian glan d carcin om a. Th e n odu lar t ype presen ts as a discrete n odu le th at often is m istaken for a ch alazion . Th e pagetoid sp reading su bt ype spreads in to th e derm is an d epith elium in a diffu se pat tern , often m im icking ch ron ic conju n ct ivit is ( Fig. 2.29A,B).

Fig. 2.29 (A) Sebaceous cell carcinoma of eyelid margin. (B) Sebaceous cell carcinoma involving m ost of the upper tarsal conjunctiva.

A

B

2 Eyelids and Lacrim al System

63

Differential Diagnosis Bleph arit is, ch alazion , superior lim bic keratoconju n ct ivit is, ch ron ic conju n ct ivit is, cicat ricial pem ph igoid

Management Su rgical excision w ith w ide su rgical m argin s w ith frozen -sect ion con t rols is often n ecessar y. Conju n ct ival m apping h elps evaluate pagetoid spreading. Evalu ate local lym ph n odes (preauricular an d cer vical), an d perform a system ic evaluat ion for m etast at ic spread.

Cutaneous Malignant Melanoma Th is is rarely seen on th e eyelids but can m anifest as potent ially lethal skin lesion s.

Presentation Th ese often present as a slow ly grow ing pigm en ted lesion, but alm ost h alf of lesions can be n onpigm ented ( Fig. 2.30A,B,C). Th ey are clinically seen in three t ypes:

A

B

Fig. 2.30 (A) Lentigo maligna. (B) Melanoma of medial canthus. (C) Melanoma of upper eyelid.

C

64

Color Atlas of Ophthalm ology

Lent igo m aligna (pre-m elanom a lesion) : A slow -grow ing pigm en ted lesion , often affect ing elderly pat ien t s, w h ich can develop in to a m elan om a Superficial spreading: Su perficially spreading lesion w ith an irregu lar ou tlin e, variable pigm en tat ion , an d delay in pen et rat ion in to deeper layers Nodular: A m ore aggressive lesion th at h as a ten den cy to invade deeper layers early in it s grow th

Differential Diagnosis Nevus, basal cell carcin om a, keratoacon th om a, seborrh eic keratosis, squ am ous cell carcin om a

Management Excision al biopsy w ith 3- to 5-m m free m argin s is recom m en ded for th in cut an eou s periocular m elan om as. Melan om as th icker (u sually greater th an 2m m in th ickn ess) an d located elsew h ere m ay require 1 to 3 cm free m argin s depen ding on th eir th ickn ess. Th e exten t of surgical an d adju n ct ive th erapy is determ in ed by t u m or t ype, level, an d clin ical stage.

Distichiasis and Trichiasis Dist ich iasis can be eith er a congen it al or an acquired con dit ion of th e eyelids an d involves th e abn orm al grow th of lash es from th e orifices of th e m eibom ian glan ds. Trich iasis is an acqu ired con dit ion of eyelash es th at are m isdirected tow ard th e globe.

Presentation Dist ich iasis can presen t in a variet y of w ays. Th e dist ich iat ic lash es can be th in or of n orm al th ickn ess, pigm en ted or n onpigm en ted, h ave n orm al orien tat ion , or m ay be m isdirected. Acqu ired cases of dist ich iasis can be seen in longst an ding cicat rizat ion associated w ith t rach om a, ch em ical inju r y, Steven s-Joh n son syn drom e, an d ocular pem p h igoid. Trich iasis can be th e result of scarring of th e lid m argin secon dar y to ch ron ic t rach om a, bleph arit is, Steven s-Joh n son syn drom e, an d h erpes zoster oph th alm icu s. In all cases, th e lash es ru b again st th e eye an d can cau se irrit at ion , tearing, an d corn eal epith eliopathy. Longst an ding cases can resu lt in corn eal u lcerat ion an d pan n us ( Fig. 2.31A,B).

Differential Diagnosis En t ropion , ep ibleph aron , bleph arit is, an d topical prostaglan din an alogue m edicat ion s for glaucom a

Management Num erou s approach es h ave been reported for t reat m en t . Ep ilat ion is accom plish ed by lash rem oval w ith forceps (n ot a p erm an en t solut ion ) or m ore effect ively w ith elect rocau ter y, cr yoth erapy, or argon laser to in dividu al lash es. Altern at ively, th ey can be surgically approach ed in a variet y of w ays, in cluding a com bin at ion of lam ellar eyelid division w ith cr yoth erapy to th e aberran t lash es or direct surgical excision by w edge resect ion .

2 Eyelids and Lacrim al System

65

Fig. 2.31 (A) Distichiasis. (B) Trichiasis (of upper lid). Note the cicatricial changes of both upper and lower lids.

A

B

Lacrimal System Disorders Canaliculitis Can alicu lit is con sist s of eith er or both in flam m at ion an d in fect ion of th e upp er or low er can alicu lus.

Presentation Th e can alicu lar region is er yth em atou s, in du rated, an d ten der to p alpat ion . Pat ien t s com plain of ep iph ora w ith ch ron ic m u copuru len t disch arge. Th e m ost com m on bacterial path ogen is Act inom yces, w h ich produces gran ular-like con cret ion s th at are difficu lt to express from th e pun ct i ( Fig. 2.32A, B).

Differential Diagnosis Ch ron ic dacr yocyst it is, eth m oidal m u cocele

Management Local an t ibiot ics sh ould be u sed according to th e path ogen s iden t ified on cult u res an d sen sit ivit y (gen erally su scept ible to pen icillin s an d ceph alosporin s). Surgical in cision (can alicu lotom y) w ith drain age an d curet tage of th e con cret ion s is often used for su ccessful t reat m en t .

66

Color Atlas of Ophthalm ology

Fig. 2.32 (A) Chronic canaliculitis. (B) Eikenella canaliculitis.

A

B

Dacryocystitis Th is is an in fect ion of th e n asolacrim al sac; it is often u n ilateral an d secon dar y to an acqu ired obst ru ct ion of th e n asolacrim al du ct .

Presentation Presen tat ion can be acute or ch ron ic. A sten osis or obst ruct ion w ith in th e n asolacrim al du ct can lead to reten t ion of tear flu id w ith su bsequen t superin fect ion . An acute cou rse presen t s w ith a pain ful, localized er yth em a an d in flam m at ion arou n d th e lacrim al sac. An abscess can often develop w ith even a spon tan eous rupt ure of th e an terior skin leading to a drain ing fist ula. Neon ates can also presen t w ith dacr yocyst it is secon dar y to n asolacrim al du ct obst ru ct ion . Ch ron ic in fect ion produ ces epiph ora w ith associated conju n ct ivit is an d m in im al ten dern ess. Lacrim al sac m assage produ ces reflu x of m ucopu rulen t m aterial from th e pu n ct i ( Fig. 2.33 ).

Differential Diagnosis Nasolacrim al du ct obst ruct ion , orbital cellulit is, conju n ct ivit is, h ordeolu m

2 Eyelids and Lacrim al System

67

Fig. 2.33 Dacryocystitis, left lacrim al sac with associated preseptal cellulitis.

Management If m ild, acu te an d ch ron ic presen t at ion s can be in it ially t reated w ith local an d oral an t ibiot ics, an d on ce th e acu te sym ptom s h ave resolved, a dacr yocystorh in ostom y is often requ ired. Severe dacr yocyst it is w ith secon dar y orbital cellulit is or abscess form at ion m ay require in t raven ous an t ibiot ics. Abscess form at ion s n eed to be t reated w ith in cision an d drain age.

Nasolacrimal Duct Obstruction Th is can be congen it al or acquired. In congen it al cases, th ere is a delay in th e can alicu lizat ion of th e low er port ion of th e n asolacrim al du ct , w h ich is seen in up to 20% of in fan t s du ring th e first year of life bu t is sym ptom at ic in less th an 4% of th ese ch ildren . Acquired cases can be secon dar y to t rau m a or in fect ion .

Presentation Con st an t ep iph ora an d w et t ing of th e eyelash es. Mu copuru len t m aterial is often expressed from th e low er pu n ct i after lacrim al m assage. Sym ptom s can w orsen du ring an u pper resp irator y in fect ion ( Fig. 2.34 ).

Fig. 2.34

Left nasolacrimal duct obstruction.

68

Color Atlas of Ophthalm ology

Differential Diagnosis Congen it al glau com a in in fan t s, eth m oidal m u coceles, lacrim al sac t u m ors (ben ign or m align an t), dacr yocyst it is, can alicu lit is

Management Treat m en t sh ou ld be delayed in in fan t s un t il about 1 year of age becau se m ost (up to 95%) of cases self-resolve. Nasolacrim al du ct probing an d irrigat ion are usually curat ive in over 90% of in fan t s. Recu rren t failures often im ply an an atom ical problem an d m ay require silicon e in t ubat ion or balloon dilat ion . Persisten t failu res m ay n eed a dacr yocystorh in ostom y.

3 Orbital Infections, Inflammation, and Neoplasms Praveen Saluja, Sw at i Ravani, Soosan Jacob, and Am ar Agarw al

Preseptal Cellulitis Preseptal cellu lit is is defin ed as a soft t issue in fect ion an terior to th e orbit al sep t u m . In fect ion p osterior to th is sept um , anyw h ere in th e orbit , is orbit al cellulit is. Orbit al cellu lit is is a dangerous con dit ion ow ing to th e close proxim it y to th e orbital apex, cavern ous sin us, m en inges, an d brain . Bacterial in fect ion of th e eyelid an terior to th e orbital sept um t ypically affects ch ildren , usu ally secon dar y to lid in fect ion su ch as severe acu te h ordeolu m , skin lacerat ion , an in sect bite, or th e spread of in fect ion from th e su rroun ding st ruct ures (paran asal sin uses, lacrim al sac, u pper respirator y t ract , in cluding th e m iddle ear). Th e in fect ion does n ot pen et rate th e orbital sept um , w h ich separates th e an terior st ru ct u res from th e orbit .

Presentation Sym ptom s in clu de eyelid edem a (w h ich m ay lead to in abilit y to op en th e eye), periorbit al sw elling, rubor, color, ten dern ess, w ith out proptosis. Un like orbit al cellulit is, th ere is n o pain w ith eye m ovem en t s. Ocu lar m ot ilit y, visu al acuit y, an d pupillar y react ion s are all n orm al (Fig. 3.1A,B,C).

Differential Diagnosis Orbital cellulit is: Decreased visu al acu it y, decreased sen sat ion along th e first division of th e t rigem in al n er ve, eyelid edem a, proptosis, ch em osed conjun ct iva, pain w ith eye m ovem en t s, rest ricted eye m ovem en ts, sign s of ocu lar m ot ilit y disorders Cavernous sinus throm bosis: Bilateral, decreased visual acu it y, decreased sen sat ion along th e first an d secon d division of th e t rigem in al n er ve, p roptosis an d paresis of cran ial n er ves III, IV, an d VI, ch em osed conju n ct iva Chalazion: Focal, usu ally w ith ou t ten dern ess, gradu ally progressive ch ron ic in flam m at ion of th e m eibom ian glan d Allergic edem a of the eyelid Contact derm at it is Viral conjunct ivit is associated w ith lid edem a: Watering, itch ing, st ickin ess of th e eyelash es, conjun ct ival follicu lar react ion , w ith or w ith ou t disch arge an d palp able preauricular lym ph n ode Erysipelas: Acu te st reptococcal cellu lit is (m ostly h as a clear-cut dem arcat ion lin e) w ith sign s of toxem ia, in clu ding h igh -grade fever an d ch ills Others: In sect bite, angioedem a, t raum a, osteom yelit is of paran asal sin u ses, especially m axillar y sin us

Management Ch eck for a h istor y of t rau m a, rapidit y of on set , pain , fever, ch ills, can cer, diabetes, pulm on ar y diseases, an d ren al diseases. Ch ar t th e vit als (pu lse, respirat ion , tem perat u re, blood pressure). Exam in e for exoph th alm om et r y, globe displacem en t , an d resistan ce to ret ropulsion an d exam in e th e orbital rim . Record ocular m ovem en t an d m easure deviat ion w ith a prism bar. Pup ils m u st be evalu ated for ligh t reflexes, in cluding relat ive afferen t pupillar y defect (RAPD). Color vision , in 69

70

Color Atlas of Ophthalm ology

A

B

C Fig. 3.1 (A) Preseptal cellulitis, allergic reaction. (B) Preseptal cellulitis, bacterial infection final. (C) Preseptal cellulitis, fungal infection.

t raocu lar pressu re (in clu ding th e pressu re in variou s gazes), an d ret in al evalu at ion sh ould be recorded. Evaluate th e cran ial n er ves (especially III, IV, V1 , V2 , VI,). Exam in e th e h ead an d n eck for lym p h aden it is. Gram stain ing an d cu lt u re of any open w ou n d an d disch arge sh ould be perform ed at th e earliest opport u n it y. A com plete an d differen t ial blood cou n t is perform ed if sign s of toxem ia exist .

3 Orbital Infections, In am m ation, and Neoplasm s

71

Oral an t ibiot ics are in dicated in cases of m ild in flam m at ion , afebrile pat ien t , age m ore th an 5 years, good pat ien t com p lian ce. Drugs of ch oice in clu de am oxicillin clavu lan ate, or cefaclor, or cot rim oxazole, or er yth rom ycin , clin dam ycin , am oxicillin -cloxacillin for a durat ion of 10 days. In t raven ou s an t ibiot ics are in dicated in m oderate to severe in flam m at ion s. In cases of pat ien t age less th an 5 years, p oor pat ien t com plian ce, im m un ocom prom ised pat ien ts, n o im provem en t w ith or w orsen ing w ith oral an t ibiot ics, dr ugs of ch oice in clu de ceft riaxon e w ith van com ycin . Supp or t ive t reat m en t in clu des h ot fom en t at ion s, local an t ibiot ics (p olym yxin w ith bacit racin oin t m en t), an d n on steroidal an t i-in flam m ator y drugs (NSAIDs). Explorat ion of th e w oun d is perform ed if n eeded.

Orbital Inflammation Orbital Cellulitis/Subperiosteal Abscess/Cavernous Sinus Syndrome In orbit al cellu lites, in fect ion occu rs posterior to th e orbit al sept um , usually secon dar y to th e sp read of in fect ion from th e su rroun ding st ruct ures (paran asal sin u ses, lacrim al sac, upper respirator y t ract in clu ding th e m iddle ear) or lid in fect ion , su ch as severe acu te h ordeolu m , skin lacerat ion , or an in sect bite (Fig. 3.2A,B,C).

Presentation Orbital cellulit is: Eyelid edem a (usually leading to in abilit y to open th e eye), periorbit al sw elling, rubor, ten dern ess, proptosis, pain w ith eye m ovem en t s, rest ricted ocular m ot ilit y, decreased vision , ret in al ven ou s congest ion , opt ic disk edem a, pu rulen t disch arge, an d decreased periorbital sen sat ion . Th e follow ing are m ain t ypes: Sin u s-related is th e m ost com m on an d is secon dar y to eth m oidal sin u sit is; it affect s ch ildren an d young adu lts. Caused by adjacen t st ru ct u res like dacr yocyst it is, m idfacial in fect ion , or den tal in fect ion . Post t raum at ic m ost com m on ly develops w ith in 48 to 72 h ou rs of an inju r y th at pen et rates th e orbit al sept u m . Subperiosteal abscess: Most frequ en tly located along th e m edial w all of th e orbit . Orbital abscess is relat ively less com m on w ith sin usit is but is m ore com m on in post t rau m at ic or postoperat ive cases. Usually presen t s w ith m edial m ass, n on axial proptosis, local ten dern ess, in creased in t raocu lar pressure, abscess (in t racon al/ext racon al). Cavernous sinus throm bosis: Bilateral, decreased visu al acuit y; decreased sen sat ion along th e first an d secon d division of th e t rigem in al n er ve; rapidly progressive proptosis; paresis of cran ial n er ves III, IV, an d VI; congest ion of th e conju n ct ival vein s; ch em osed conjun ct iva; dilated an d sluggish pu pil; sign s of toxem ia in clu ding h igh -grade fever, decreased level of con sciou sn ess, n au sea, an d vom it ing.

72

Color Atlas of Ophthalm ology

A

B

C Fig. 3.2 (A) Ten-year-old boy with orbital abscess. (B) Magnetic resonance imaging (MRI) of an orbital abscess. (C) MRI, coronal section.

Differential Diagnosis (Table 3.1) Carot id cavernous fist ula: Spon tan eous or post t rau m at ic, bru it on au scult at ion of globe; ar terialized conju n ct ival vessels an d conjun ct ival ch em osis are n ot un com m on on com puted tom ograph ic scan . En larged su perior oph th alm ic vein (SOV), en larged ext raocu lar m u scles, orbit al color Doppler u lt rasou n d sh ow s reversed ar terialized blood in SOV. Erysipelas: Acute st reptococcal cellulit is. Mostly h as a clear-cu t dem arcat ion lin e w ith sign s of toxem ia in clu ding h igh -grade fever, ch ills. Others: In sect bite, angioedem a, t raum a, osteom yelit is of paran asal sin uses (especially m axillar y sin us), ch alazion , allergic edem a of th e eyelid, con t act derm at it is, viral conju n ct ivit is associated w ith lid edem a.

3 Orbital Infections, In am m ation, and Neoplasm s

Table 3.1

73

Di erential Diagnosis of Orbital In ammatory Conditions

Feature

Pseudotumor

1. Lateralit y 2. Age

Unilateral 20 to 50 years

3. Onset

Acute, subacute, chronic Proptosis, ptosis, chemosis with pain

4. Clinical presentation 5. Laboratory ndings

Increased ESR

6. Systemic symptoms 7. Response to Steroids

Malaise Small doses

Thyroid Exophthalmos

Orbital Cellulitis

Bilateral Fourth to fth decade Chronic

Unilateral Children and young adults Acute

Proptosis with lid signs

Periorbital swelling and tenderness Increased WBC

Abnormal thyroid function tests Thyroid symptom s Higher doses

Fever Responds to antibiotics

ESR, erythrocyte sedim entation rate; WBC, white blood cell count

Management Th e pat ien t m u st be adm it ted to th e h ospit al. Gram st ain ing an d cult u re of any open w ou n d an d disch arge sh ou ld be don e at th e earliest oppor t u n it y along w ith com plete an d differen t ial blood cou n t an d blood cu lt ures. Mu corm ycosis m u st be kept in m in d, especially in diabet ic an d im m u n osu ppressed p at ien ts. Lum bar pun ct u re for suspected m en ingit is is perform ed un der a physician’s su per vision . Neu rologic opin ion sh ould be un der t aken if th e gen eral con dit ion of th e pat ien t dict ates th e sam e. In t raven ous an t ibiot ics su ch as ceft riaxon e plu s van com ycin , or van com ycin plus gen tam icin , or van com ycin plu s clin dam ycin w ith or w ith ou t m et ron idazole are given in it ially follow ed by oral an t ibodies for 7 to 14 days. Hot fom en t at ion is applied fou r to five t im es a day. Local an t ibiot ics in clude polym yxin w ith bacit racin oin t m en t . For corn eal exposu re, NSAIDs h elp com bat pain an d in flam m at ion . Mon itor for th e follow ing w arn ing sign s: Dilated pu pils Marked oph th alm oplegia Loss of vision Relat ive afferen t p upillar y defect Papilledem a Perivasculit is Violaceou s lids Explorat ion of th e w ou n d is in dicated if th e pat ien t is un respon sive to an t ibiot ics, vision is decreasing, orbital abscess is presen t , an d a diagn ost ic biopsy is n eeded. It is im port an t to drain th e orbit al abscess as w ell as th e in fected sin u ses. Th e follow ing oral an t ibiot ics are given on ly after th e con dit ion im proves sign ifican tly: am oxicillin -clavulan ic acid, or cefaclor, or cot rim oxazole, or er yth rom ycin , clin dam ycin , am oxicillin -cloxacillin w ith or w ith out m et ron idazole.

74

Color Atlas of Ophthalm ology

Thyroid-Related Ophthalmopathy Graves’ disease or diffuse toxic goiter, is an au toim m u n e process th at in clu des on e or m ore of th e follow ing: hyper thyroidism , oph th alm opathy, an d in filt rat ive derm opathy.

Presentation Graves’ disease u sually occu rs w ith hyper thyroidism , but n orm al thyroid fu n ct ion can also be n ot iced. It is five t im es m ore com m on in fem ales (Fig. 3.3A,B). Th ere are t w o t ypes according to level of severit y: 1. Noninfilt rat ive (m ild): Min im al in flam m ator y react ion leading to m ild sym ptom s an d sign s. 2. Infilt rat ive (severe): Th is t ype h as a m ore fu lm in an t course w ith in flam m at ion , in filt rat ion , an d scarring. Th ese pat ien t s h ave ch em osis, proptosis, corn eal exposu re, m yosit is, an d en largem en t of m uscles. It ult im ately leads to corn eal exposu re, rest ricted m ovem en t s, an d diplopia. Werner classification reflects the severit y of the ophthalm opathy and is well know n by the acronym of NO SPECS (as described below ). Each grade is further subdivided as 0 to 4 and a to c: Grade 0: Grade 1: Grade 2: Grade 3: Grade 4: Grade 5: Grade 6:

No sign s or sym ptom s On ly sign s (lid ret ract ion ) Soft t issu e involvem en t (e.g., ch em osis) Proptosis (m in im um ) Ext raocu lar m u scle involvem en t Corn eal involvem en t Sigh t loss

Th ere are variou s sign s in thyroid eye disease, w h ich go by th e discoverers’ n am es (Fig. 3.3C,D,E) (Table 3.2).

A

B Fig. 3.3 (A) Thirt y-five-year-old woman with dysthyroid orbitopathy with lid retraction. (B) Lid lags behind when patient looks down.

3 Orbital Infections, In am m ation, and Neoplasm s

75

C

D

E Fig. 3.3 (Continued) (C) Dysthyroid orbitopathy. (D) Com puted tomographic (CT) scan, extraocular muscle enlarge ment sparing muscle tendons. (E) Coronal section CT scan.

76

Color Atlas of Ophthalm ology

Table 3.2

Ophthalmic Manifestations of Graves Disease

Most important signs Von Graefe: Upper lid lag on downgaze Dalrymple: Upper eye lid retraction Upper eyelid signs Von Graefe: Upper lid lag on downgaze Dalrymple: Upper eyelid retraction Boston: Uneven jerky movement of upper lid on inferior movement Jellinek: Abnormal pigmentation of upper lid Kocher: Retraction of upper lid during xation Gi ord: Di cult eversion of upper eyelid Pupillary signs Cowen: Extensive hippos of consensual papillary light re ex Lowey: Dilatation of pupil with 1:1000 epinephrine Knies’ sign: Uneven di cult dilatation in dim light Bruit signs Riesman: Bruit over eyelid Snellen sign: Bruit over the eye Eye movement signs Ballet: Paralysis of one or more extraocular muscles (EOM) Möbius: De cient convergence Suker: Inabilit y to maintain xation at extrem e lateral gaze Wilder: Jerking of eyes on movement from abduction to adduction Blinking signs Pochin: Reduced amplitude of blinking Stellwag: Incomplete or infrequent blinking Lag signs Von Graefe: Upper eyelids lag on downgaze Gri th sign: Lower eyelid lag on upgaze Conjunctival signs Goldzieher: Deep injection of temporal conjunctival vessels

Differential Diagnosis Orbital pseudotum or, cavernous sinus throm bosis, orbital cellulitis (see Table 3.1)

Management Man agem ent opt ion s for thyroid eye disease in clude obser vat ion , con ser vat ive in ter ven tion s, oral cort icosteroids, injected cor ticosteroids, extern al-beam radioth erapy, an d surger y. Obser vat ion an d con ser vative m easures are appropriate for m ild conjun ctival inject ion an d ch em osis w ith n orm al corn ea and opt ic n er ve fu nction . Advice to th e pat ien t in cludes sleeping w ith th e h ead of the bed elevated an d avoiding salt or m on osodium glutam ate to m in im ize fluid reten tion . Sunglasses are recom m en ded to m in im ize ph otoph obia. Nonpreser ved art ificial tears h elp decrease th e ocular irritat ion . Glaucom a m edicat ion s sh ould be used on ly in patien ts w ith ver y h igh in t raocular pressures and a fam ily h istor y of glaucom a. Surgical in terven t ion sh ould be perform ed in a stepped fash ion : decom pression first, strabism us surger y second, an d eyelid surger y last . Pat ien ts should stop sm oking an d avoid secon dh an d sm oke to lim it th e autoim m un e exacerbat ion of thyroid eye disease. Man agem en t of t hyroid orbitop at hy m u st t ake in to accou n t w h eth er th e disease is act ive or ch ron ic an d t h e d egree to w h ich th e m an ifest at ion s im p act t h e

3 Orbital Infections, In am m ation, and Neoplasm s

77

p at ien t ’s daily life or t h reaten sigh t . For exam p le, m ild eyelid ret ract ion w ith m in im al or n o d r y eye sym ptom s m igh t be m an aged w it h con ser vat ive lu bricat ion p r ior to elect ive eyelid su rger y on ce th e p at ien t h as st abilized . At t h e op p osite en d of t h e sp ect r u m is t h e p at ien t p resen t ing w it h acu te opt ic n eu rop athy resu lt in g from ap ical crow d in g th at requ ires u rgen t m ed ical an d /or su rgical m an agem en t . Pat ien ts sh ould be m an aged in close correspon den ce w ith an en docrin ologist , w h o m ay elect to t reat system ic hyp erthyroidism via ph arm acological supp ression , surgical resect ion , or radioact ive iodin e. Recen t st udies suggest a ben efit to steroid th erapy in th e peri-in ter ven t ion al period in m in im izing th e progression of orbitopathy. Gen eral t reat m en t opt ion s for th e m an agem en t of sequ elae of thyroid orbitopathy in clude ph arm acological th erapy, radiat ion th erapy, an d surgical in ter ven t ion . Mild cases w ith m in im al ocular irritat ion or sym ptom at ic diplopia m ay in it ially be m an aged con ser vat ively w ith lu bricat ing eyedrops an d oin t m en t , n oct u rn al taping, an d Fresn el prism s. Radioth erapy, gen erally 20 Gy delivered in 10 fract ion s over 2 w eeks, h as long been used as t reat m en t for th e orbit al m an ifestat ion s of thyroid disease as h as been sh ow n to be of ben efit in im provem en t of m ot ilit y. How ever, a recen t prospect ive, ran dom ized st u dy by Gorm an et al dem on st rated n o ben eficial th erapeut ic effect of radioth erapy in m oderate, sym ptom at ic thyroid orbitopathy. Surgical m an agem en t can be divided in to elect ive an d u rgen t in ter ven t ion s. Elect ive su rger y sh ould proceed in th e order of orbit al decom pression , st rabism us su rger y, an d fin ally lid surger y, becau se each in ter ven t ion as listed can in fluen ce th e ou tcom e of th e su bsequen t in ter ven t ion s. Th ere are a m u lt it u de of ap proach es to orbit al decom pression en com passing on e to all w alls, w ith or w ith ou t en doscopic an d t ran sn asal exposure an d w ith or w ith ou t orbit al fat decom pression . St rabism u s su rger y sh ould be u t ilized to m axim ize th e field of bin ocu lar vision an d gen erally in cludes recession of m uscles on adjust able sut u res. Eyelid procedu res in clu de bleph arop last y w ith or w ith ou t rem oval of orbital fat , release of u pper lid ret ract ion via levator an d or Mu ller m u scle recession , an d repair of low er lid ret ract ion w ith spacer grafts. In dicat ion s for u rgen t su rgical in ter ven t ion in clude opt ic n europathy from apical crow ding an d corn eal u lcerat ion secon dar y to exposu re. Th e m an agem en t of th ese con dit ion s can in clude m any of th e procedures outlin ed earlier in addit ion to urgen t m edical m an agem en t w ith pu lse cor t icosteroids, ph arm acoth erapy, an d orbit al irradiat ion . Cor t icosteroids can provide sh ort-term relief for sym ptom s an d sign s of thyroid eye disease, but hyper thyroid pat ien t s can suffer sign ifican t m ood sw ings. In addit ion , tapering th e steroids often result s in rebou n d in flam m at ion at least as severe as th e origin al presen tat ion . Oth er im m u n osu ppressive agen ts h ave been used in th e t reat m en t of thyroid orbitopathy as steroid sparing agen ts, in cluding cyclosporin e, cytoxan , m eth ot rexate, an d azath ioprin e. Som e favor com bin at ion th erapy w ith cyclosporin e an d cor t icosteroids. Oth er t reat m en t m odalit ies in clude som atost at in an alogu es (oct reot ide), plasm aph eresis, an d in t raven ou s im m u n oglobulin th erapy.

Orbital Inflammatory Pseudotumor Orbit al in flam m ator y p seu d ot u m or (OIP) con sist s of a sp ect r u m of n ongran u lom atou s in flam m ator y con d it ion s of th e orbit , w ith n o kn ow n et iology or system ic associat ion s, th at p rodu ce p roptosis du e to a n on n eop last ic in flam m ator y m ass in th e orbit . (Note: Som e au th ors classify Tolosa-Hu n t syn drom e as a su bt yp e of OIP.)

78

Color Atlas of Ophthalm ology

Fig. 3.4 Middle-aged woman with left eye pseudotum or.

Presentation OIP presents w ith abrupt onset of pain, proptosis (unilateral), conjunctival chem osis, epibulbar injection, visual loss, diplopia, and restricted ocular m ovem ents (Fig. 3.4). According to the different tissues involved, it is classified as one of the follow ing: Myosit is Dacr yoaden it is Periopt ic n eurit is Posterior sclerit is or ten on it is It s clin ical cou rse is variable, an d it m ay be regressed spon tan eously w ith out any t reat m en t , or it m ay h ave prolonged in flam m at ion or in term it ten t act ivit y. A prolonged course m ay result in a frozen orbit secon dar y to fibrosis. Bilateral involvem en t is rare.

Differential Diagnosis Graves oph th alm opathy, orbital cellulit is, leu kem ia, cavern ou s sin us th rom bosis, rh abdom yosarcom a (see Table 3.1)

Management Com puted tom ograph ic (CT) scan s an d m agn et ic reson an ce im aging (MRI) are essen t ial in th e w orkup of susp ected OIP. In flam m ator y sign s predom in ate an d m ay in clu de involvem en t of th e ext raocu lar m u scles (EOMs), orbit al fat , lacrim al glan d, ch oroid, an d sclera. Orbit al ult rasoun d m ay be of use in dem on st rat ing th icken ing of th e posterior Ten on capsu le an d in dist ingu ish ing th e m yosit is of OIP from EOM involvem en t in thyroid orbitopathy becau se th e m uscu lar ten don s w ill classically be involved in OIP an d spared in thyroid disease. Th e CT orbit sh ow s ext raocu lar m uscle th icken ing involving ten din ous in sert ion . In flam m at ion of th e ret robu lbar fat pad an d con t rast en h an cem en t of th e sclera du e to ten don it is m ay produ ce a T sign or ring sign . Orbit al ult rasou n d sh ow s th icken ing of th e posterior Ten on capsule along w ith m uscle belly th icken ing (un like thyroid related orbitop athy, w h ich t ypically spares th e ten don s). System ic steroids (60 to 80 m g/day) are given . Rapid respon se is p ath ogn om on ic. Taper slow ly over m on th s to avoid recu rren ce. Pu lsed in t raven ou s steroids are given in severe vision -th reaten ing cases. Radioth erapy is recom m en ded in steroidresist an t cases. Ch em oth erapeut ic agen ts su ch as cycloph osph am ide, m eth ot rexate, an d cyclosporin e are used for cases resist an t to steroids an d radioth erapy an d in pat ien t s in toleran t to steroids.

3 Orbital Infections, In am m ation, and Neoplasm s

79

Orbital Lymphoma Orbit al lym ph om a is a low -grade m align an cy ch aracterized by proliferat ion of m on oclon al B cells (n on -Hodgkin disease), w h ich arises in lym ph n odes or in ext ran odal sites su ch as th e orbit .

Presentation Th e disease presen t s bet w een th e ages of 50 an d 80 years w ith involvem en t of any par t of th e orbit . Bilateral involvem en t is rare. It occu rs rarely in ch ildren . Most orbit al lym ph om as are low grade. Malign an t lym ph om as can produ ce a palpable m ass th at m ay be presen t in th e an terior orbit . On e can h ave pain less progressive proptosis, accom pan ied by vision loss, occasion al diplopia, lid edem a, ptosis, an d lacrim al glan d involvem en t . A salm on -colored conju n ct ival t um or is ch aracterist ic (Fig. 3.5A,B,C,D).

A

C

B Fig. 3.5 (A) Fift y-five -year-old m an with lymphoma. (B) Patient with lymphoma. (C) Salmon patch in conjunctiva.

80

Color Atlas of Ophthalm ology

Differential Diagnosis Metastasis, react ive lym ph oid hyperplasia, pseudot um or, sarcoidosis

Management Com pu ted tom ograph ic scan sh ow s a w ell-defin ed m ass, located m ostly in th e an terior-su perior lateral orbit , w h ich m olds to en com pass adjacen t st ru ct u res. Th e lacrim al glan d is frequen tly involved. Ult rason ography sh ow s variable sh ape an d borders of th e lesion , w h ich h as low to m edium in tern al reflect ivit y. Radioth erapy (2500 to 3000 cGy) is th e t reat m en t of ch oice for less w elldifferen t iated lesion s. Ch em oth erapy can also be t ried. A w ell-differen t iated lesion w ith ou t system ic involvem en t can be obser ved .Visu al progn osis is excellen t if th e disease is con fin ed to th e orbit .

Other Orbital Neoplasms Dermoid Cyst Derm oid cyst is a developm en t al, slow -grow ing ch oristom a (t u m ors w ith h istologically n orm al cells in an abn orm al locat ion ), lin ed w ith st rat ified squam ou s epith eliu m an d filled w ith kerat in ized m aterial an d/or lipid. Most of th ese cyst s are located in th e eyelid an d orbit , represen t ing th e single m ost com m on cause of periorbital n eoplasm in ch ildren . Th ey develop because of sequ est rat ion of th e su rface ectoderm pin ch ed off at th e bon e su t u re lin es or along th e lin es of em br yon ic closure. Th e cyst s are lin ed w ith epiderm is w ith derm al appen dages su ch as h air follicles an d sebaceous glan ds in th e w all.

Presentation Derm oids are classified according to th e an atom ical site of presen tat ion : Superficial derm oids (Fig. 3.6A) In fron t of th e orbital sept um an d su perotem poral or su peron asal quadran ts Presen t at ion in in fan cy an d ch ildh ood Palpable, firm , un ilateral, localized m ass, u sually asym ptom at ic, m ay be m obile or fixed to th e un derlying st ruct ures an d free from th e overlying skin Deep derm oids (Fig. 3.6B) Posterior to th e orbital sept um , associated w ith bony sut u res in th e orbit but m ay exten d across th e bon es in th e fron t al sin us, tem poral fossa, or cran iu m Presen t in adolescen ce, m ay be seen in ch ildren an d adu lt s Proptosis, ocu lar displacem en t an d bony defect , m ot ilit y rest rict ion , decreased vision . Spon t an eou s rupt u re produces severe orbit al in flam m at ion .

3 Orbital Infections, In am m ation, and Neoplasm s

81

Differential Diagnosis Cavern ou s h em angiom a, m u cocele, opt ic n er ve gliom a, m en ingiom a, n eurilem m om a

Management Superficial derm oids CT scan of the orbit : Rou n d, w ell-defin ed lesion s w ith en h an cing rim an d a lucen t a cen ter, w h ich m ay con t ain calcium . Th ere m ay be a w ell-cort icated bon e defect . Echography : A w ell-defin ed lesion w ith m edium to h igh in tern al reflect ivit y, w ith an irregular acoust ic st r uct ure; usu ally sh ow s som e com pressibilit y Com plete surgical excision : In on e piece Incom plete excision or capsular rupt ure : May lead to recurren ce w ith in filt rat ion Deep derm oids CT scan of the orbit : Well-defin ed lesion s w ith an en h an cing rim . Th ey m ay con tain areas of calcificat ion . Th e cen t ral lu m en is n on en h an cing, of variable den sit y, an d m ay sh ow a fluid–fat in terface. Th ere m ay be a bon e defect . Echography : A cyst ic m ass w ith low to m edium in tern al reflect ivit y is seen . High er ech oes occu r on ly w h en th e cyst is filled w ith kerat in debris an d fat . Com plete surgical excision : In on e piece, w ith out ru pt ure of cap su le ( Fig. 3.6C– F).

Capillary Hemangioma Capillar y h em angiom a is a p rim ar y, u n ilateral, ben ign h am ar tom a of t igh tly packed cap illaries, ap paren t at birth or w ith in th e first 8 w eeks of life, st raw berr y red to pu rp le. Most regress com pletely w ith in 7 years of age. Th ey are visible on th e surface bu t m ay lie deep in th e orbit . It is m ore com m on ly seen in th e superon asal qu adran t of th e u pper eyelid.

Presentation More com m on in girls. Involvem en t of sup erficial st ru ct u res (derm is) results in a st raw berr y m ark (st raw berr y n evus), single or m u lt iple, u sually elevated. Su ch pat ien t s m ay presen t w ith ptosis, som et im es associated w ith ast igm at ism an d am blyopia. Involvem en t of th e deep par ts (an d an terior orbit) appears as a blu ish m ass w ith a spongy text u re. W h en th e ch ild cries or st rain s, th e m ass becom es m ore p rom in en t an d deep en s in color. On exam in at ion , it is a circu m scribed, soft red m ass w ith a m u lt in odular surface. Large feeding vessels are seen an d can be th e source of bleeding ( Fig. 3.7 ).

82

Color Atlas of Ophthalm ology

A

B

C Fig. 3.6 (A) Young girl with a superficial derm oid. (B) A 4-year-old child with a dermoid involving the lateral canthal area. (C) A 9-year-old girl with a deep dermoid in the lateral orbit pushing the left eyeball medially.

3 Orbital Infections, In am m ation, and Neoplasm s

83

D

E

F

Fig. 3.6 (Continued) (D) Sagit tal plane computed tomographic (CT) scan of a dermoid. (E) Coronal section CT scan. (F) Specimen of dermoid cyst excised, same patient specimen.

84

Color Atlas of Ophthalm ology

Fig. 3.7

Eight-month-old boy with a capillary hemangioma.

Differential Diagnosis Nevus flam m eus (darker, does n ot blan ch w ith pressu re), derm oid cyst , en cep h alocele, lym ph angiom a, in fect ion , n euroblastom a ( Table 3.3 )

Management Pat ien t m u st be referred to a pediat rician for w orkup of system ic associat ion (s) like h igh -out put cardiac failu re, Kasabach -Merrit t syn drom e (an em ia, th rom bocytopen ia, low levels of coagulat ing factors due to th eir sequ est rat ion in th e lesion ), or Maffucci syn drom e (en doch ordom atas an d skin h em angiom as). Com plete oph th alm ologic exam in at ion m u st be don e to rule ou t poten t ial secon dar y even t s, n am ely, am blyopia, com pressive opt ic n eu ropathy, an d corn eal exposu re. Orbit al ult rasoun d suggest s a poorly ou tlin ed lesion , irregu lar in sh ape, h igh in tern al reflect ivit y, an d an irregu lar acoust ic st ruct ure w ith variable sou n d at ten uat ion . CT scan sh ow s con t rast en h an cem en t an d defin es th e exten t of th e lesion . Deeper lesion s are w ell defin ed w ith m oderate to in ten se en h an cem en t . On MRI, th e lesion sh ow s h om ogen eous an d h eterogen eou s sign als, being hypoin ten se on T1 an d hyperin ten se on T2 w edging. Flow voids appear as hyperin ten se region s. Gadolin iu m en h an ces th e lesion m oderately. Obser vat ion is pract iced in m ost of th e cases becau se involu t ion usu ally occu rs in th e follow ing con dit ion s: Superficial condit ions: Severe cosm et ic deform it y an d deprivat ion am blyopia are th e m ajor in dicat ion s of in ter ven t ion . Th e t reat m en t opt ion s in clu de in t ralesion al (40 m g/m L t riam cin olon e plu s 6 m g/m L betam eth ason e) or system ic (predn isolon e 1 to 2 m g/kg/day) steroid. Oth er opt ion s in clude radioth erapy, yellow -dye laser, an d topical cort icosteroids. Su rger y sh ou ld be reser ved for sm all, circum scribed lesion s. Deep condit ions: Large lesion s or am blyopia u sually w arran t t reat m en t w ith local radioth erapy (500 cGy) or system ic or local cor t icosteroids. Surger y m ay be at tem pted in sm all, circu m scribed lesion s. Progn osis is good for vision an d for life.

3 Orbital Infections, In am m ation, and Neoplasm s

Table 3.3

85

Vascular Lesions

Features Disease Onset Clinical features

Capillary Hemangioma Benign lid and orbital hamartoma Soon after birth

Strawberry nevus and thrombocytopenia (KasabachMerrit t syndrome) Ultrasonography High internal x-ray re ectivit y

Computed tomographic scan Treatment

Lymphangioma

Cavernous Hemangioma

Orbital Varices

Benign lid and orbital tum or

Benign orbital tumor

Children

Adults

Ectatic vascular channels Young adults

Chocolate cysts

Axial proptosis

Exophthalm os or enophthalmos

Cystic pat tern

Well-de ned Calci caround tion tumor with high internal echoes In ltrative, Late enhance- Enlarged multilobulated ment with vessels lesions contrast

Irregular, poorly circum scribed mass Observation, Observation, intralesional steroids, steroids, surgery system ic steroids, laser, radiation

Observation, steroids

Conservative

Lymphangioma Lym ph angiom a is a rare vascu lar h am ar tom a of lym ph at ic ch an n els th at is h em odyn am ically isolated from th e vascular system . Occu rring predom in an tly in ch ildren an d teen agers (m ost frequ en tly in th e first decade of life), th e size of th e lesion fluct uates w ith post ure an d Valsalva m an euver an d w ith u pper respirator y t ract in fect ion s.

Presentation Su perficial lesion s occu r in th e conjun ct iva or lid an d are visible as cyst ic spaces w ith clear flu id par t ially filled w ith blood. Deep lym ph angiom atous lesion s classically presen t w ith acute ou t set of pain ful proptosis result ing from spon t an eou s h em orrh age w ith in th e orbit . Su ch lesion s are called ch ocolate cysts. Th e t u m or m ass m ay com press th e globe or opt ic n er ve, causing visu al loss, refract ive errors, secon dar y glau com a, congest ion of th e opt ic n er ve, an d visu al field defects ( Fig. 3.8A,B).

86

Color Atlas of Ophthalm ology

A

B Fig. 3.8 (A) Ten-year-old boy with lymphangioma involving the lower nasal orbit. (B) Coronal section, computed tom ographic scan.

Differential Diagnosis Opt ic n er ve gliom a, plexiform n eurofibrom a, capillar y h em angiom a, pseudot u m or

Management Th e orbital lesion is seen as low -den sit y cyst ic, in t racon al an d ext racon al m asses, w ith variable en h an cem en t on m agn et ic reson an ce im aging. With T1 w edging

3 Orbital Infections, In am m ation, and Neoplasm s

87

th ey are hypoin ten se, w h ereas T2 w edging respon se is variable, dep en ding on th e state of h em oglobin degen erat ion . Angiography sh ow s n o vascular com pon en t . Man agem en t of lym ph angiom as is ch allenging. Radiat ion an d system ic steroids sh ow lim ited sen sit ivit y. Com p lete su rgical excision is ver y difficu lt because of th e in filt rat ive n at u re of th e t u m or. If acu te h em orrh age cau ses sym ptom s, CO2 laser or con tact n eodym ium :yt t rium -alu m in um -garn et can be t ried for h om eostasis an d obliterat ion of t um or as an altern at ive to evacu at ion , p ar t ial resect ion , or ligat ion . Am blyopia is com m on an d is m ostly from recu rren t h em orrh age or globe com pression .

Rhabdomyosarcoma Rh abdom yosarcom a is th e m ost com m on prim ar y m align an t orbit al t um or in ch ildren (70%arise w ith in th e first decade of life). Th is soft t issue m esen chym al t um or accou n ts for u p to 4% of all ch ildh ood m align an cies. It arises from pleuripoten t m esen chym al precu rsors th at n orm ally differen t iate in to st riated m uscle cells.

Presentation Presen tat ion is u sually in th e first decade of life w ith a rapidly progressive proptosis, m ore com m on ly in boys. It frequ en tly sh ow s a m ass in th e u pper par t of th e orbit , ptosis, an d eyelid edem a. Th e diagn osis is con firm ed by biopsy. It can be grouped in to four categories: em br yon al, alveolar, pleom orph ic, an d bot yroid in th e orbit . Th e m ost com m on h istological varian t is em br yon al follow ed by th e alveolar t ype ( Fig. 3.9A,B,C,D).

Differential Diagnosis Orbit al cellu lit is, pseu dot u m or, lym ph angiom a, m etastat ic n eu roblastom a, ru pt u red derm oid cyst

Management In th e past , p at ien ts w ith orbit al rh abdom yosarcom as u n der w en t orbital exen terat ion . Because of th e m align an t n at ure of th e t um or, it w as th ough t th at radical resect ion p rovided th e best ch an ce for su r vival. Despite th ese m easures, m ort alit y rem ain ed as h igh as 70%. Over th e past 30 years, w ith a com bin at ion of surger y, radiat ion , an d ch em oth erapy, sur vival h as approach ed 90%. A staging classificat ion w as proposed by th e In tergroup Rh abdom yosarcom a St u dy grou p in 1972. Com plete resect ion of localized disease is categorized as group I. In gen eral, m icroscopic residual disease or lym ph n ode involvem en t is categorized as grou p II. Grou p III in clu des gross residu al disease or in com plete resect ion . Group IV disease in clu des cases w ith m et astasis at presen t at ion . Th e st age of disease is depen den t n ot on ly on th e exten t of th e t u m or but largely on th e exten t of resect ion . Th e sam e t u m or can be a group I or II versu s a grou p III depen ding on w h eth er th e su rgeon perform ed an excision or in cision al biopsy, respect ively. Th e recom m en ded regim en of radiat ion an d ch em oth erapy is based on th e stage of disease an d is sum m arized follow ing h ere. Becau se of rh abdom yosarcom as’ sen sit ivit y to ch em oth erapy an d radiat ion , an in cision al biopsy follow ed by eith er ch em oth erapy, radiat ion , or both is p referred by m ost oph th alm ologist s. Th is approach is especially pruden t w ith large t um ors or t um ors in w h ich an excision al biopsy w ould likely h arm th e opt ic n er ve, ext raocu lar m uscles, or oth er im port an t orbital st ruct ures. Th e decision is m ore difficu lt for th ose sm aller, m ore an terior t um ors w h ere com plete excision w ith ou t en dangering oth er vit al orbit al st ru ct u res is feasible. Most orbit al rh abdom yosar-

88

Color Atlas of Ophthalm ology

Fig. 3.9 (A) Three-year-old boy with rhabdomyosarcoma. (B) Computed tomographic (CT) scan, sagit tal section.

com as are located su peron asally an d in th e ext racon al space w h ere excision w ould n ot violate th e opt ic n er ve or ext raocular m u scles. Su ch an app roach m ay perm it low er doses of radiat ion . Judiciou s review of th e CT an d MRI scan s is crit ical for surgical plan n ing. In cision s directly overlying th e t um or are th e preferred approach to biopsy. For exam ple, m ore p osterior t um ors are best ap proach ed via a cu tan eou s in cision th rough th e lid, w h ereas m ore an terior t u m ors th at are visible in th e conju n ct ival forn ices m ay be ap proach ed via a t ran sforn iceal approach . For excision al biopsies, care sh ould be taken to con t ain th e t u m or in it s pseudocapsu le an d n ot to violate th e periosteu m to preser ve th e n at u ral barrier to spread ou tside th e orbit . Irradiat ion for orbit al rh abdom yosarcom as plays a secon dar y role to ch em oth erapy in m an agem en t . Conven t ion al fract ion ated doses totaling 4000 to 5000 cGy are u su ally su fficien t to con t rol t um or recurren ce. How ever, at th ese doses, ocular com plicat ion s of orbit al irradiat ion , in clu ding radiat ion ret in op athy, cat aract , dr y eyes, an d radiat ion -associated keratopathy, are relat ively com m on .

3 Orbital Infections, In am m ation, and Neoplasm s

89

Fig. 3.9 (Continued) ( C) CT scan, coronal section. (D) CT scan showing involvement of the eyeball.

With a com bin at ion of su rger y, ch em oth erapy, an d radiat ion , it is p ossible to con t rol th e t u m or an d salvage th e eye in ~90% of cases of orbit al rh abdom yosarcom a. Supplem en t al ch em oth erapy h as su bstan t ially im proved su r vival rates of pat ien t s w ith orbit al rh abdom yosarcom as. Pat ien ts w h o u n dergo surger y, radiat ion , an d ch em oth erapy for rh abdom yosarcom a in cluding but n ot lim ited to th e orbit h ave been sh ow n to h ave a 2-year disease-free su r vival rate of 82%, com pared w ith 53% in th ose w h o un dergo su rger y an d radiat ion alon e. Vin crist in e an d act in om ycin D h ave been th e m ain stays of ch em oth erap eut ic agen ts em ployed in cases of orbit al rh abdom yosarcom a. New er agen ts such as ifosfam ide an d etoposide h ave been sh ow n to produce a favorable respon se.

90

Color Atlas of Ophthalm ology

Cavernous Hemangioma Cavern ou s h em angiom a is usually seen in m iddle-aged w om en an d is th e m ost com m on ben ign in t raorbit al t u m or in adult s.

Presentation Th e t u m or is u n ilateral, solit ar y, an d t ypically located in th e in t racon al area. Proptosis is of th e axial t ype. Th ere is a predilect ion for m iddle-aged w om en , an d th e t u m or m ay grow faster during pregn an cy. Th e t u m or m ay be associated w ith opt ic disk edem a an d ret in al folds (st riae). Th e vision m ay decrease by on e or t w o lin es. Th ere m ay be rest ricted m ovem en t s in ext rem e fields of gaze (Fig. 3.10A– C).

Differential Diagnosis Oth er in t raorbit al m ass lesion s su ch as derm oid cyst , lym ph om a, sch w an n om a

Management Com puted tom ograph ic scan con firm s th e diagn osis. Most cavern ou s h em angiom as can be obser ved. If su rger y is don e, th e t um or is seen to be a w ell-circum scribed, pu rp le, en capsu lated lesion w ith dist in ct vessels on it s surface.

Carotid Cavernous Fistula Orbit al vascular abn orm alit ies are a grou p of orbit al disorders, congen it al or acqu ired, arising from a variet y of un derlying con dit ion s. Arterioven ous m alform at ion s, h aving feeder vessels from both in tern al an d extern al carot id circulat ion s, m ostly occur after t rau m a (m ales, 15- to 30-year age range) bu t m ay also arise spon tan eously (fem ales, 30- to 60-year age range). Carot id cavern ou s fist u las (CCFs) occur m ostly after basal skull fract ures or pen et rat ing orbit al t rau m a an d can occu r spon tan eously in person s w ith system ic hyperten sion . Th e h igh -flow CCFs arise w h en th e in tern al carot id ar ter y develops a defect w ith in th e cavern ous sin us, w h ereas low -flow CCFs develop from th e com m u n icat ion bet w een th e m en ingeal bran ch es of th e in tern al carot id ar ter y an d th e cavern ou s sin u s .

Presentation Th e pat ien t n ot ices a sw ish ing n oise in th e h ead th at is syn ch ron ou s w ith th e pulse. Becau se of th e proxim it y of th e ocu lar m otor n er ves to th e cavern ous sin u s, im paired ocular m ot ilit y an d diplopia are early fin dings. Proptosis, lid an d orbital edem a, an d dilated an d tor t u ou s conjun ct ival an d episcleral vein s develop later. Elevated episcleral ven ou s pressu re leads to ocular hyper ten sion . High-flow CCF: Ch em osis, corkscrew dilatat ion of th e epibulbar vessels, orbital edem a, proptosis, pu lsat ile exoph th alm os, audible bru it , secon dar y glau com a, ret in al vascu lar dilatat ion , papilledem a, rapid afferen t papillar y defect , decreased vision , occasion al n er ve palsies (III–VI are m ost com m on ) Low -flow CCF: Ch em osis, in creased episcleral ven ous pressure, ven ous dilatat ion ( Fig. 3.11A,B,C).

3 Orbital Infections, In am m ation, and Neoplasm s

91

A

B

C Fig. 3.10 (A) Young adult presenting with proptosis, a case of cavernous hemangioma. (B) Coronal section computed tomographic (CT) scan, cavernous hemangioma. ( C) CT scan, cavernous hemangioma.

92

Color Atlas of Ophthalm ology

A

B

C Fig. 3.11 (A) Carotid cavernous fistula with left eye proptosis conjunctival congestion. (B) Restricted movements in the left eye. (C) Computed tomographic scan showing cavernous sinus involvement. Arrow marks cavernous sinus and also note prominent superior ophthalmic vein.

3 Orbital Infections, In am m ation, and Neoplasm s

93

Differential Diagnosis Cavern ou s sin u s th rom bosis, pseudot um or, thyroid orbitopathy

Management Com puted tom ograph ic scan w ith con t rast rem ain s th e in it ial procedu re of ch oice. It sh ow s dilated su perior oph th alm ic vein w ith en largem en t of th e superior orbital fissure an d erosion of th e an terior clin oid process. Ult rason ography of th e orbit sh ow s a dilated superior oph th alm ic vein , m ild th icken ing of th e EOMs, an d m ediu m to h igh in tern al reflect ivit y from edem a. MRI w ith gadolin iu m or m agn et ic reson an ce angiography is a u sefu l tool to invest igate fu rth er, su pplem en t ing th e CT scan . Sm all, spon t an eou s, low -flow fist ulas resolve frequen tly (u p to 40%) from th rom bosis. Em bolizat ion is in dicated on ly w h en vision loss, glaucom a, or severe pain is presen t . Traum at ic h igh -flow fist ulas rarely resolve on th eir ow n . With a h igh rate of visual loss in th ese pat ien ts in ter ven t ion becom es th e ru le. Th e cu rren t t ren d involves in ter ven t ion al radiology w ith in t ravascular balloon s or oth er em bolizat ion via cath eter in th e in tern al carot id arter y.

Orbital Varices Orbit al varices m ay represen t congen ial foci of an abn orm al vessel (th e m ost com m on site is th e upp er n asal qu adran t , an d it is usu ally un ilateral) or m ay be th e late stage of oth er vascu lar abn orm alit ies.

Presentation In term it ten t proptosis, w h ich is n onpu lsat ile an d n ot associated w ith bru it . It can affect pat ien t s from early ch ildh ood to late m iddle age.

Differential Diagnosis Derm oid cyst , lym ph angiom a

Management Su rger y is difficu lt because th e lesion s are friable an d bleed easily, an d in m ost cases, excision is incom plete. Indications for surgical intervention include re peated episodes of pain , th rom bosis, severe proptosis, an d opt ic n er ve com pression . Con ser vat ive t reat m en t by CO2 laser, yt t riu m -alum in u m -garn et laser, or cau ter y is recom m en ded. Em bolizat ion is possible if feeder vessels can be iden t ified.

Mucocele Mu coceles are cyst ic lesion s origin at ing from prim ar y obst ru ct ion of a paran asal sin us (m ost com m on ly fron t al or eth m oid sin uses) follow ing t rau m a, sin usit is, or, rarely, a t um or, th at slow ly en large causing bon e deform it y w ith erosion s of th e orbit . Con sist ing of a cyst ic m ass filled w ith m ucu s, m ucoceles m ay be bou n d by an eggsh ell layer of bon e an d w h en in fected are referred to as pyoceles.

94

Color Atlas of Ophthalm ology

Presentation Mu coceles m ost com m on ly arise from th e fron t al an d eth m oidal sin us. Pat ien ts presen t w ith a com bin at ion of proptosis, a palpable flu ct u an t m ass, h eadach e, diplopia, ptosis, an d epiph ora or globe displacem en t . Sw elling of th e u pper eyelid m edially is com m on . Pain is n ot com m on in th e absen ce of in fect ion (Fig. 3.12A– D).

Differential Diagnosis Derm oid cyst , osteom a, pseudot um or

Management Com pu ted tom ograph ic scan sh ow s an op acified fron t al or eth m oidal sin u s, loss of eth m oid septae, an d a bony deh iscen ce. Th e cyst ic con ten t sh ow s variable den sit y an d is n on en h an cing. Ult rason ography reveals a ver y w ell-defin ed m ass w ith sh arp su rface spikes an d low in tern al reflect ivit y. Mucocele is associated w ith a ver y large bony defect adjacen t to a paran asal sin u s. Treat m en t is su rgical rem oval of th e cyst lin ing an d reestablish m en t of n orm al drain age. Obliterat ion of th e sin u s w ith fat or m u scle m ay be n ecessar y to t reat recu rren ces.

Metastatic Orbital Lesions Th ese lesion s represen t 2 to 10% of all orbital t um ors. In ch ildren , n euroblastom a, Ew ing sarcom a, an d acute m yeloid leu kem ia are com m on . Th e m ost com m on prim ar y sites in adu lt s are th e breast , bron ch u s, p rostate, skin m elan om a, gast roin test in al t ract , an d kidn ey.

Presentation A m ass in th e an terior orbit cau sing axial or n on axial displacem en t of th e globe is m ost com m on . In filt rat ion of orbit al t issue ch aracterized by ptosis, diplopia, an d in durated skin su rroun ding th e orbit is com m on . In flam m ator y react ion is seen . It m ay be seen presen t ing eith er as proptosis w ith decreased visual acuit y, diplopia, pain , paresth esia, in creased in t raocular pressure, an d exp osure keratopathy, or in cases of cicat rizing carcin om as su ch as cer t ain secon daries from th e breast , as en oph th alm os ( Fig. 3.13A– D).

Differential Diagnosis Orbit al pseudot um or, rh abdom yosarcom a, leukem ias

Management Treat m en t is aim ed at preser ving vision an d relieving p ain . Th e m ain opt ion s are radioth erapy an d h orm on al th erapy (th e lat ter in cases of breast an d prost at ic m et ast asis). Ch em oth erapy is often useful in con t rolling th e system ic disease. A biopsy m ay som et im es be requ ired to est ablish th e n at u re of th e prim ar y. Gen erally, on ly palliat ive th erapy can be offered.

3 Orbital Infections, In am m ation, and Neoplasm s

95

Fig. 3.12 (A) Orbital abscess from frontal sinus with pansinusitis. (B) CT scan axial section showing the orbital abscess. ( C) CT scan sagit tal section showing spread of infection behind the orbital septum . (D) The patient also has ethmoidal and maxillary sinusitis.

A

B

C

D

96

Color Atlas of Ophthalm ology

A

B

C

D Fig. 3.13 (A) Eight-year-old girl suffering from leukemia. (B) Computed tomographic scan, leukemia involving the orbit. (C) Neuroblastoma. (D) Secondaries in orbit.

3 Orbital Infections, In am m ation, and Neoplasm s

97

Lacrimal Gland Enlargement Lacrimal Gland Inflammation In flam m ator y cau ses, w h ich are n ot u n com m on , in clude dacr yoaden it is, sarcoidosis, an d orbital in flam m ator y pseudot u m or. A decreased Sch irm er test suggest s an in flam m ator y lesion .

Lacrimal Gland Tumors Benign Mixed Tumor (Pleomorphic Adenoma) Th ese are slow ly grow ing lesion s u su ally seen in th e fou r th to fifth decades of life.

Presentation A long h istor y of m ore th an 1 to 2 years is gen erally obtain ed, an d it u su ally presen ts as a n on in filt rat ing lesion in th e lacrim al glan d area w ith fulln ess of th e su perotem poral lid an d orbit an d pain less in feron asal proptosis. Th e upper lid con tour m ay t ake an ̴ sh ape ( Fig. 3.14 ).

Differential Diagnosis In flam m ator y lesion s, t u m ors of th e lacrim al glan d, derm oids

Management Com pu ted tom ograp h ic scan sh ow s a w ell-circum scribed, pseu doen capsu lated lesion in th e lacrim al fossa. Pat ien ts w ith a long-st an ding, pain less, slow ly grow ing m ass w ith a w ellcircum scribed appearan ce on im aging st u dies are presu m ed to h ave a pleom orph ic aden om a. Treat m en t is ext irpat ion , con sist ing of a lateral orbitotom y w ith in t racapsular rem oval of all lesion al t issue w ith carefu l at ten t ion to preven t violat ion of th e pseu docapsu le.

Fig. 3.14

Mass in upper orbital region in young girl.

98

Color Atlas of Ophthalm ology

In cision al biopsy of th ese lesion s is con t rain dicated becau se, alth ough h istologically ben ign , in com plete excision often leads to repeated recu rren ces (as h igh as 30% in som e st udies) an d m align an t t ran sform at ion . Sm all, fingerlike prot u beran ces ou tside th e m ain t u m or bu lk w ith su bsequen t seeding of th e residu al t u m or are believed to be respon sible for th is ph en om en on . For pleom orph ic aden om as, long-term st u dies reveal an in creased in ciden ce of m align an t t ran sform at ion (10% at 20 years an d 20% at 30 years) associated w ith m ult ip le recu rren ces for lesion s th at h ad frequen t in cision al biopsies an d in com p lete rem oval of th e prim ar y t u m or.

Adenoid Cystic Carcinoma Aden oid cyst ic carcin om a is th e m ost com m on m align an t lacrim al glan d t u m or, represen t ing 50%of m align an t t um ors of th e lacrim al glan d an d 25%of all lacrim al glan d t u m ors.

Presentation Most cases are seen in th e th ird decade of life w ith a secon d bim odal peak in th e teen age years. Aden oid cyst ic carcin om as an d oth er m align an cies can also presen t w ith pain secon dar y to perin eu ral or bony involvem en t . Dip lopia an d dim in ish ed visu al acuit y can be seen w ith rapidly progressive lesion s. Aden oid cyst ic carcin om a u su ally p resen t s as an irregular m ass, produ cing bony erosion (70%) an d occasion al calcificat ion (20%).

Differential Diagnosis In flam m ator y lesion s, t u m ors of th e lacrim al glan d, derm oids

Management Aden oid cyst ic carcin om as carr y a poorer progn osis becau se of bony exten sion an d perin eu ral in filt rat ion . Th ese pat ien t s h ave a 50%at 5-year an d 75%at 15-year m ort alit y rate. Death is com m on ly due to in t racran ial spread an d pu lm on ar y m etast asis. Histological pat tern is also of progn ost ic sign ifican ce, w ith a cribriform pat tern h aving a 70% at 5-year sur vival com pared w ith a 20% at 5-year su r vival w ith a basaloid pat tern . CT scan , along w ith clin ical appearan ce, h elps in preoperat ive diagn osis. Treat m en t con sist s of en bloc com plete su rgical excision of th e orbit an d its con ten t s ( Fig. 3.15A– D).

3 Orbital Infections, In am m ation, and Neoplasm s

99

A

B

C

D Fig. 3.15 (A) Exenteration done in a 35-year-old woman with adenoid cystic carcinoma. (B) Spectacle prosthesis. (C) Exenteration done in a 35-year-old wom an with adenoid cystic carcinoma. (D) Same patient, spectacle prosthesis.

4 External Diseases Guillerm o Sim ón-Castellví, Pablo Gili-Man zanaro, Sarabel Sim ón-Castellví, José María Sim ón-Castellví, Crist ina Sim ón-Castellví, and José María Sim ón-Tor

Blepharitis and Ocular Rosacea Anterior blepharitis is a bilateral chronic inflam m atory process of the eyelids, w hich m ay secondarily result in corneal and conjunctival changes, w ith severe dry eye. It m ay result in corneal and conjunctival irritation due to the secretion into the eye of inflam m ator y substances and alteration of the oily layer of the tear film . St aphylococcal bleph arit is, seborrh eic bleph arit is, an d acn e rosacea w ith lid an d ocu lar involvem en t are com m on ly foun d in pat ien ts w ith bleph arit is. Hordeolum an d ch alazion form at ion is also com m on ly seen .

Presentation Presen tat ion in clu des itch ing, irritat ion , tearing, foreign body sen sat ion , crust ing on th e lid m argin s, lash loss (m adarosis) or lash m isdirect ion (t rich iasis), u lcerat ion of th e lid m argin (t ilosis), red an d th icken ed eyelids, an d ch ron ic conju n ct ivit is. Meibom it is (sebaceou s glan d dysfu n ct ion ) m ay also be presen t (Fig. 4.1A–D). Infect ious: Fibrin collaret tes on th e lash es Seborrheic: Seborrh eic derm at it is, tear film in st abilit y Ocular rosacea : Greasy skin ; facial telangiect asia; er yth em a of th e ch eeks, foreh ead; an d n ose; rh in ophym a. Com m on ly, p eriph eral corn eal im m un e in filt rates (asept ic, du e to staphylococcal t ype IV hypersen sit ivit y)

A

B

C

D Fig. 4.1 (A) Fibrin collaret tes; (B,C) lid margin telangiectasia; (D) erythem a of the cheeks in a patient with ocular rosacea. 100

4 External Diseases

101

Differential Diagnosis Oth er kin ds of conju n ct ivit is (in fect ious, allergic, or toxic), an d dr y eye. Ru le ou t sebaceou s glan d carcin om a in u n ilateral cases.

Management Lid hygiene : Com m ercially available ready-for-use lid scru bs or w arm -w ater soaks w ith dilu ted n eu t ral baby sh am poo in th e m orn ing an d at n igh t , tear su pplem en t s, an t ibiot ic gels (e.g., fu sidic acid) or oin t m en t (e.g., er yth rom ycin or bacit racin ) Diet : Vitam in an d om ega-3 su pplem en tat ion , salm on in t ake, olive oil in t ake Flax oil: Th e best source is grou n d flax seeds so ligin an d fiber are in clu ded Tapered topical steroids: For severe in flam m at ion or corn eal in filt rates or ph lycten u les. Dual-act ion topical an t iallergic m edicat ion provides relief (e.g., ketot ifen fu m arate 0.025%, azelast in e hydroch loride 0.05%, olopatadin e hydroch loride 0.1%.) Oral system ic tet racyclines: For acn e rosacea in adu lts (oral doxycyclin e, 500 m g 1 g/ever y 6 h ours for 1 m on th of t reat m en t) Abundant art ificial tears and topical lubricants: For dr y-eye sym ptom s (e.g., topical sodium hyalu ron ate 0.18%) Treat m en t is t apered according to sym ptom s

Conjunctivitis Any in flam m at ion of th e conju n ct iva is referred to as conjun ct ivit is.

Adenoviral Conjunctivitis (Epidemic Keratoconjunctivitis, Pharyngoconjunctival Fever) Adenoviral infections predom inate in sum m er m onths. Most textbooks refer to viral conjunctivitis as infections produced by adenoviruses (epidem ic conjunctivitis, adenoviral conjunctivitis). But different viruses are responsible for different t ypes of conjunctivitis. Picornavirus (m ainly enterovirus 70 and Coxsackievirus A24) are responsible for acute hem orrhagic conjunctivitis, w hich is clinically sim ilar to adenoviral conjunctivitis but m ore severe. It is highly contagious and occurs in epidem ics. Epidem ic keratoconjunct ivit is: Cau sed by aden oviru ses 8 an d 19 w ith ch aracterist ic preauricu lar lym ph n ode, p h ar yngit is, an d subepith elial in filt rates 5 to 10 days after th e in it ial sym ptom s Pharyngoconjunct ival fever: Cau sed by aden oviru ses 3 an d 7, w ith fever an d ph ar yngit is; associated w ith pu blic sw im m ing pools in sum m er

Presentation Classic sign s in clu de red eye (u n ilateral or bilateral), ciliar y inject ion (m ild irit is), an d epip h ora. In cases of aden oviral conjun ct ivit is, th e p at ien t refers to recen t exposure to an in dividual w ith red eyes at h om e, sch ool, or w ork or h as a h istor y of recen t sym ptom s of an upper resp irator y t ract in fect ion . Th e in cu bat ion period is 5 to 12 days. Acute follicu lar conju n ct ival react ion m ain ly in th e in ferior tarsal conju n ct iva, ch em osis, preauricu lar aden opathy, su bconjun ct ival petech iae (ver y sm all h em orrh ages), an d som et im es early, n on specific m ild pu n ctate keratopathy

102

Color Atlas of Ophthalm ology

m ay be seen . Su bepith elial in filt rates develop 5 to 12 days after th e in it ial sym ptom s (suggest aden oviru s serot ypes 8 an d 19). Corn eal opacit ies can persist for a few w eeks to m on th s (w e h ave seen u p to 2 years). Th ey can decrease visu al acuit y an d cau se glare sym ptom s. Epith elial ulcerat ion (par t ial or total) m ay occu r. Eyelid edem a an d su b-conju n ct ival h em orrh age suggest s acute h em orrh agic conju n ct ivit is. In severe cases, m em bran es an d pseu dom em bran es can lead to conju n ct ival scarring an d sym bleph aron . Sym ptom s in clude ph otoph obia an d eye pain if th ere is corn eal involvem en t (aden oviru s), in ten se w ater y disch arge (serou s), an d itchy eye(s). It is usu ally ben ign an d self-lim ited an d gen erally h as a longer cou rse th an acute bacterial con jun ct ivit is, last ing for ~2 to 4 w eeks ( Fig. 4.2A,B).

Differential Diagnosis Oth er epidem ic keratoconjun ct ivit is, h erpes sim plex in fect ion , h erpes zoster in fect ion , in fect iou s m on on u cleosis (w ith eye involvem en t), Epstein -Barr virus in fect ion , Dim m er kerat it is, bru cellosis

Management Most cases are self-lim ited, w ith n o m orbidit y, an d requ ire n o specific t reat m en t . High ly con tagious cases requ ire st rict hygien e m easu res. In st ru ct you r pat ien t th at

A

B Fig. 4.2

(A, B) Adenoviral conjunctivitis.

4 External Diseases

103

despite sym ptom at ic t reat m en t th e con dit ion m ay w orsen . Cool com presses an d ar t ificial refresh ed tears provide relief (fou r to eigh t t im es daily for 2 to 4 w eeks). Dark glasses can h elp. Provide relief for flu like sym ptom s w ith oral an t ih istam in es an d decongestan t s. Low -dose topical steroids (e.g., fluorom eth olon e) com bin ed w ith vasocon st rictors an d dual-act ion topical an t iallergic m edicat ion provide com for t (e.g., ketot ifen fu m arate 0.025%, azelast in e hydroch loride 0.05%, olopat adin e hydroch loride 0.1%.). Topical gel of gan ciclovir h as clearly proven effect ive in sh or ten ing disease course. Topical broad-spect rum an t ibiot ics m ay h elp to preven t secon dar y bacterial in fect ion . Topical steroids are u sed for pseudom em bran es or w h en subepith elial in filt rates im pair vision . Th ey dram at ically suppress conjun ct ival in flam m ator y sign s, relieve sym ptom s, an d are associated w ith resolut ion of th e corn eal su bepith elial in filt rates w h en presen t . We alw ays prescribe topical steroids after ru ling ou t h erpes sim plex in fect ion . We h ave n ever seen recurren ce of su bepith elial in filt rates after gradu ally t apering steroids. Be carefu l: topical steroids m ay w orsen an un derlying h erpes sim plex virus in fect ion !

Acute Hemorrhagic Conjunctivitis (Epidemic Hemorrhagic Keratoconjunctivitis) Th is t ype of conjun ct ivit is is also kn ow n as Apollo 11 conju n ct ivit is an d is due to picorn aviru s (coxsackieviru s A24 or en teroviru s group 70). Acute h em orrh agic conju n ct ivit is affect s m ostly ch ildren an d young adult s in th e low er socioecon om ic classes.

Presentation It begin s w ith an in it ial period of catarrh al in flam m at ion , follow ed, in a day or t w o, by th e appearan ce of conju n ct ival petech iae th at coalesce to form subconju n ct ival h em orrh ages. Th e explosive on set is a pain ful, rapidly progressive follicu lar con jun ct ivit is. It is self-lim ited in a couple of w eeks an d star ts w ith su bconjun ct ival diffuse h em orrh age, m ore frequ en t in th e upper bulbar, an d sym ptom s of viral conju n ct ivit is su ch as p reau ricu lar lym ph at ic n ode an d an terior segm en t in flam m at ion togeth er w ith flu like sym ptom s. Th ere is periorbital pain . Th e lids often becom e sw ollen an d in durate (lid edem a). Ch em osis, serom u cou s disch arge, ph otoph obia, an d tearing are also seen ( Fig. 4.3 ).

Differential Diagnosis Aden oviral or bacterial in fect ion , subconju n ct ival h em orrh age, kerat it is

Fig. 4.3

Acute hemorrhagic conjunctivitis.

104

Color Atlas of Ophthalm ology

Management No t reat m en t is usu ally n ecessar y. Bed rest , dark glasses, cold com presses, an d an algesics (e.g., paracetam ol 500 m g ever y 8 h ou rs) are h elpfu l. Given th at th is is h igh ly con t agious, st rict hygien e m easures sh ould be obser ved. Provide relief of flu like sym ptom s w ith oral an t ih ist am in es an d decongest an ts. Low -dose top ical steroids com bin ed w ith vasocon st rictors an d du al-act ion topical an t iallergic m edicat ion provide com for t (e.g., ketot ifen fum arate 0.025%, azelast in e hydroch loride 0.05%, olopat adin e hydroch loride 0.1%.). Topical gel of gan ciclovir h as proven efficacy in sh or ten ing disease course. Topical broad-spect ru m an t ibiot ics m ay h elp to preven t secon dar y bacterial in fect ion .

Herpes Simplex Keratoconjunctivitis Most textbooks refer to viral conjun ct ivit is as th at produced by aden oviruses (epidem ic conjun ct ivit is, aden oviral conjun ct ivit is), bu t differen t viruses are respon sible for differen t t ypes of conju n ct ivit is or keratoconju n ct ivit is. Herpes sim plex virus (HSV) is th e m ost dangerous cause, especially HSV t ype 1 (m ou th , gen it al). HSV t ype 2 m ay also be a cau se, esp ecially in ch ildren an d n eon ates. HSV affects a variet y of ocular t issu es an d m ay cau se ocular derm at it is, epith elial an d st rom al kerat it is, an d even iridocyclit is ( Fig. 4.4A,B).

Presentation Red pain fu l eye (un ilateral or bilateral, usually u n ilateral), ph otoph obia-epiph ora, acute follicular conjun ct ival react ion m ain ly in th e in ferior tarsal conju n ct iva,

A

B

Fig. 4.4 (A) Herpetic dendritic corneal ulcer. (B) Associated skin lesions.

4 External Diseases

105

ch em osis, som et im es early n on specific m ild pu n ct ate keratopathy, classic den drite-like corn eal u lcerat ion w ith term in al bulbs. Flu orescein stain s th e u lcer base, w h ereas rose bengal st ain s th e u lcer edges. Usually occu rs in sexu ally act ive adu lt s. In cases left u n t reated or in late diagn osis or in m u n ocom prom ised pat ien t s it can lead to disciform kerat it is, in terst it ial kerat it is, an d iridocyclit is.

Differential Diagnosis Epidem ic keratoconjunctivitis, varicella-zoster herpetic infection, corneal abrasion

Management Ophthalm ic ganciclovir gel (preferred) or acyclovir ointm ent (five tim es daily, 7 to 10 days is usually enough). Ganciclovir gel is m uch less toxic for the corneal epithelium than acyclovir. Use in children m ay not be approved for herpes sim plex in som e countries. Alternatives include 3%vidarabine ointm ent (five tim es daily, until reepithelialization or 7 days), 1% trifluridine drops (1 drop every 2 hours, m axim um 9 drops), or 0.5%idoxuridine drops (one drop, five tim es per day, 7 days). Epithelial debridem ent and patching: Use gan ciclovir an d an t ibiot ic oin t m en t . Add topical antibiotic drops, gel, or ointm ent to avoid superinfection : Dark glasses, antihistam ines (e.g., levocabastine every 8 to 12 hours) and vasoconstricting drops provide relief. Oral fam ciclovir reduces duration and the risk of recurrence. Concom itant use of topical ganciclovir, steroids, and oral fam ciclovir: Required for disciform kerat it is, in terst it ial kerat it is, an d iridocyclit is. In th e case of iridocyclit is, prescribe cycloplegia.

Acute Bacterial Conjunctivitis Becau se of th e excellen t defen se system s of th e eye, acute bacterial conju n ct ivit is is un com m on , bu t it is st ill th e m ost com m on eye disease. In m ost cases self-lim ited an d ben ign , it is ch aracterized by th e presen ce of abun dan t m ucopu rulen t disch arge in a pat ien t w ith red eye. Th ere is con siderable overlap in th e presen t ing fin dings from differen t bacteria. On ly exper t clin ician s m ay be able to recogn ize th e probable in fect ive agen t at a clin ical level. Epidem iology /t ransm ission : In fect ious for th e first 48 h ours of t reat m en t

Presentation Signs: Redn ess w ith variable conju n ct ival inject ion (hyperem ia), palpebral conju n ct iva being m ore affected th an bu lbar, lid sw elling (m arked lid edem a ch aracterist ic of Haem ophilus influenzae an d Corynebacterium diphtheriae ), m em bran es th at are com m on ly seen w ith St reptococcus pyogenes an d Corynebacterium diphtheriae , an d ch em osis (bulbar conju n ct iva an d forn iceal). Th ere is u su ally n o preauricu lar aden opathy or skin involvem en t , th e corn ea is usu ally clear (possible corn eal ulcerat ion if un t reated), an d th e pupil reacts n orm ally. Sym ptom s: Unilateral, sudden onset of red eye w ith significant irritation and foreign body or grit t y sensation (no outstanding pain), w hich progresses to the other eye in 2 to 5 days. It is usual for sym ptom s to be present for several days or w eeks at the tim e of presentation. An uncom m only long duration or frequent recurrences suggest that other factors or conditions m ay be present (e.g., chronic dacryocystitis, urethritis). The patient has sticky lids or m at ting of eyelashes, especially in the m orning, w ith serom ucoid (at the beginning) or m ucopurulent copious grayish, yellow, or green discharge (later). W hite discharge is due to abundant m ucus and suggests allergic reaction. Visual acuit y is preser ved except for the expected m ild blur due to the discharge and debris in the tear film . Consider gonococcal conjunctivitis if there is excessive purulent discharge.

106

Color Atlas of Ophthalm ology

B

A

Fig. 4.5 (A) Conjunctival congestion. (B) Mucopurulent discharge. (C) Conjunctival congestion and lid edem a.

C

Com plicat ions: Staphylococcus aureus blep h arit is is seen in ch ron ic bacterial conju n ct ivit is an d extern al h ordeolu m (st ye). Gon ococcu s can pen et rate th e in t act corn eal epith eliu m leading to perforat ion ( Fig. 4.5A– C).

Differential Diagnosis Any cause of red eye (e.g., viral conju n ct ivit is, allergic conju n ct ivit is, any kerat it is, uveit is, acu te angle closure glau com a)

Management Lid cleansing: It is im por tan t to keep th e lids clean an d rem ove all disch arge. Broad-spect rum topical ant ibiot ic: Most cases of bacterial conju n ct ivit is clear in 48 to 72 h ou rs w ith a low dose of any broad-spect rum an t ibiot ic applied topically. Con t in ue t reat m en t for at least 7 days. High ly respon sive to em pirical t reat m en t (e.g., tobram ycin , n orfloxacin , levofloxacin ). Tw o drops in affected eyes, ever y 3 to 4 h ours, for 1 w eek. Oint m ents and gels: Ver y usefu l w ith ch ildren (day an d n igh t) or for overn igh t use in adult s Cult ures: Con sider for cases refractor y to topical t reat m en t

Chlamydia Trachoma Ch lam ydial organ ism s are respon sible for n eon at al conju n ct ivit is, adu lt in clu sion conju n ct ivit is, t rach om a, an d lym ph ogran ulom a ven ereu m (Nicolas-Favre disease, in t ropical region s, rarely affects th e eye). Ch lam ydial (in clusion ) conju n ct ivit is t ypically affects sexu ally act ive teen s an d you ng adult s, an d Chlam ydia is th e m ost frequ en t in fect iou s cause of n eon atal conju n ct ivit is in th e Un ited States. Adult in clusion conju n ct ivit is, also called parat rach om a, is du e to Chlam ydia t rachom at is serot ypes D to K, w h ereas t ru e t rach om a is du e to Chlam ydia t rachom at is serot ypes A to C ( Fig. 4.6A,B).

Presentation ( Table 4.1 )

4 External Diseases

107

B

A Fig. 4.6 (A) Trachom a, cicatricial ptosis, lid everted following trachoma. (B) Trachom a corneal ulceration.

Table 4.1

Presentatio n o f Chlam ydia Tracho m a Adult Inclusio n Co njunctivitis (Paratracho m a o r Oculo genital Syndro m e)

Transmission Epidemiology

Tracho m a

Oculogenital (venereal) Sporadic

Ocular Endem ic Flies and other fomites ease spreading Endem ic region Developed countries Underdeveloped countries (Africa, (Europe, United States) Asia, Middle East); hot, dry climates Reservoir Eye and nose Eye and nose Genital tract in males and females Age of infection Young adults (men and Children (100% are infected in women, 15–30 years) endemic areas before age 2) Sexually active Incubation 8–10 days 5–8 days period 6 days in children Presentation Acute or chronic Acute or chronic Lateralit y Uni-/bilateral (may be Almost always bilateral asymmetric) First signs Follicular conjunctivitis Red eye and mucopurulent discharge Mucopurulent discharge (acute or chronic) Follicular conjunctivitis (superior tarsal follicles and papillae) Very slow evolution (in years) No membranes Conjunctival injection, punctate keratitis, superior corneal pannus, follicles (most dense in the inferior cul-de -sac) may be present A palpable preauricular node is almost always present (prominent lymphoid reaction) (Continued on page 108)

108

Color Atlas of Ophthalm ology

Table 4.1

(Con t in u ed) Presen tatio n o f Chlam ydia Tracho m a Adult Inclusio n Co njunctivitis (Paratracho m a o r Oculo genital Syndro m e)

Tracho m a Ocular signs and symptoms include the chief complaint that an eye infection has persisted longer than 3 weeks despite treatm ent with topical antibiotics Conjunctival scarring (limbal Herbert pits, von Arlt lines in superior and sometim es inferior tarsal conjunctiva) Epithelial super cial keratopathy, pannus: super cial corneal vascularization Dry eye Bacterial superinfection Blindness Chronic (years) May have acute start

Complications

No scarring of the conjunctiva

Course

Weeks (may have acute start) Rule out other venereal infections (gonorrhea and syphilis) Good Poor in chronic untreated cases or reinfection (common) Reinfection with other pathogens is frequent

Prognosis

Management Salin e rin sing clears th e m u copur ulen t debris from th e lids an d conjun ct iva. Azithromycin (Zithrom ax) 1 g by m outh is the drug of choice. One dose has been docum ented as being as effective as doxycycline for the treatm ent of genital chlamydial infection. For greater safety we prescribe 500 m g, once every day for 3 days. Altern at ives: Oral am oxicillin or er yth rom ycin 250 to 500 m g, orally, fou r t im es daily, for 3 or 4 w eeks Oral doxycyclin e 100 m g, orally, t w ice daily, for 1 w eek (be aler t for p regn an cy or lactat ion ) Oral tet racyclin e 250 to 500 m g, orally, fou r t im es daily, for 1 w eek (con t rain dicated in pregn an t or lact at ing w om en an d ch ildren u n der age 8) Topical antibiotics are relatively ineffective (but useful). Topical therapy is adjunctive but not essential: rifam picin (drops or ointm ent), or erythromycin ointm ent (drug of choice for pregnant wom en), or tetracycline drops or ointm ent, three tim es daily, for 7 days. We always prescribe adjunctive topical therapy. In ch ildren u se oral er yth rom ycin su spen sion , 40 m g/kg/day, in fou r divided doses for 2 w eeks.

4 External Diseases

109

In pregn an t w om en use er yth rom ycin 250 to 500 m g, orally, fou r t im es daily, for 3 w eeks. Topical flu oroquin olon e (ofloxacin or ciprofloxacin ) if th ere is a corn eal in fect ion Topical steroids can give tem porar y relief (st art a few days after topical an t ibiot ics). Treat sexual partners. A condom does not protect against chlamydial conjunctivitis.

Chlamydia/Adult Inclusion Conjunctivitis Th is con dit ion is sexually t ran sm it ted an d t ypically foun d in young adu lts. It presen ts as ch ron ic follicular conju n ct ivit is cau sed by Chlam ydia t rachom at is serot ypes D to K. A h istor y of vagin it is, cer vicit is, or u reth rit is m ay or m ay n ot be presen t .

Presentation Presen tat ion in clu des ch ron ic, bilateral, red, an d irrit ated eye. In ferior tarsal or bulbar conjun ct ival follicles, su perior corn eal pan n us, palpable p reau ricu lar n ode, or t iny, gray-w h ite periph eral subepith elial in filt rates m ay be presen t . A st ringlike, m u cous disch arge is t ypical.

Differential Diagnosis All oth er form s of ch ron ic conjun ct ivit is

Management Diagn ost ic test s in clu de slit-lam p exam in at ion , direct ch lam ydial im m u n oflourescen ce test , or Chlam ydia cu lt ure of th e conju n ct iva. Conju n ct ival scraping w ith Giem sa st ain sh ow s basoph ilic in t racytop lasm ic in clusion bodies in epith elial cells, polym orph on u clear leukocytes, an d lym ph ocytes in n ew born s. Azith rom ycin 1 g by m ou th single dose, doxycyclin e 100 m g by m outh t w ice a day, or er yth rom ycin 500 m g by m outh four t im es a day for 7 days is given to th e pat ien t an d sexual part n ers. Topical er yth rom ycin an d tet racyclin e t w o to th ree t im es a day for 2 to 3 w eeks are oth er t reat m en t opt ion s.

Chronic Conjunctivitis and Recurrent Corneal Abrasions Ch ron ic conju n ct ivit is an d recurren t corn eal abrasion s m ay h ave th eir origin s in a lid disease (e.g., bleph arit is), a lid m alp osit ion (e.g., en t ropion ), or a lacrim al w ay dysfu n ct ion (e.g., dacr yocyst it is). Left un t reated, th ese con dit ion s can resu lt in severe dam age to th e ocu lar su rface as a resu lt of differen t corn eal an d con jun ct ival com plicat ion s. Determ in ing th e cause is essen t ial to in dicate adequ ate t reat m en t .

Presentation Sym ptom s in clu de recu rren t or ch ron ic conjun ct ivit is (red eye, disch arge, etc.), spasm odic inversion of th e eyelid m argin (spasm odic en t ropion ), foreign body sen sat ion , ocu lar su rface irrit at ion , corn eal abrasion s, an d scars. In advan ced cases th ere can be vision problem s (ast igm at ism , leu kom as in th e visu al axis), rest ric-

110

Color Atlas of Ophthalm ology

Fig. 4.7 Spasmodic entropion with corneal ulceration sequelae.

t ion of ocu lar m ot ilit y (pseudopter ygiu m , sym bleph aron ), an d blin dn ess (in un derdeveloped coun t ries w h ere su rger y is un available) ( Fig. 4.7 ).

Differential Diagnosis Trich iasis, dist ich iasis, dacr yocyst it is, t rach om a, ch em ical bu rn s, au toim m u n e disorders (e.g., pem ph igoid)

Management Treat m en t varies w ith th e cause of recu rren t conju n ct ivit is. Un t il su rger y can be perform ed, bot u lin u m toxin inject ion s provide im m ediate tem porar y relief of spast ic en t ropion by w eaken ing th e pretarsal orbicularis oculi m u scle. Dacr yocystorrh in ostom y is perform ed in com plete obst ru ct ion of lacrim al p ath w ays. Treat com plicat ion s w h en n ecessar y (e.g., topical an t ibiot ic drops an d oin t m en t s for in fect ious conjun ct ivit is). Provide ext raocular lubricat ion w ith gels an d oin t m en t s to reduce th e risk of abrasion s.

Giant Papillary Conjunctivitis Papillae m ay be presen t in an oth er w ise h ealthy person . Papillae are gen erally presen t in th e sup erior lid t arsal conju n ct iva, m ost often papillae are due to an in ten se allergic respon se, eith er con t act (con t act len s u sers, as part of a Gell- Coom bs t ype 1 im m u n oglobulin E–m ediated hypersen sit ivit y react ion ) or spring allergy (vern al keratoconju n ct ivit is). Gian t pap illar y conju n ct ivit is is a ch ron ic reversible in flam m ator y con dit ion . In th e presen ce of gian t papillae con sider th e follow ing: Vern al conjun ct ivit is Atopic keratoconju n ct ivit is Gian t papillar y conjun ct ivit is of con tact len ses Gian t papillae of prosth eses, corn eal scleral sh ields En ds of nylon sut ures, cyan oacr ylate glue, ext ruded scleral buckles

Presentation Pat ien ts presen t w ith itch ing, red eyes, foreign body sen sat ion , m ucou s disch arge, an d th e presen ce of gian t papillae at slit-lam p exam in at ion (cobbleston e aspect), usually in prosth eses or con tact len ses w earers (associated w ith u se of all t ypes

4 External Diseases

Fig. 4.8

111

Giant papillae. Vernal conjunctivitis.

of con tact len ses—h ard, hydrogel, scleral, prosth et ic, etc.). Th e con dit ion can be m ore aggressive in ch ildren , w ith lid sw elling an d pseudoptosis of th e su perior lid ( Fig. 4.8 ).

Differential Diagnosis Atopic conju n ct ivit is, vern al conju n ct ivit is

Management Discon t in ue con t act len s or prosth esis w ear for at least 3 to 4 w eeks. Most pat ien ts do n ot require m ore aggressive t reat m en t . Topical m ast cell–st abilizing solu t ion s, an t ih istam in es, an d top ical steroids (drops an d oin t m en t s) can be used. Except ion ally, gian t papillae m ay requ ire steroid depot inject ion at th e t arsu s. Silver n it rate or radiosu rger y su rgical curet tage of large/gian t papillae m ay be useful in som e cases (e.g., corn eal ulcerat ion ). Plasm aph eresis h as been su ccessfully u sed as adjun ct th erapy for pat ien ts w ith h igh im m u n oglobu lin E levels (e.g., vern al conju n ct ivit is). En cou raging th e pat ien t to replace con tact len ses frequen tly, u se preser vat ive-free len s solut ion , an d in crease len s hygien e all are good preven t ive m easu res.

Cicatrizing Disorders Ocular Cicatricial Pemphigoid Cicat ricial pem ph igoid is an autoim m u n e disorder of un kn ow n et iology, w h ere circulat ing an t ibodies an d com plem en t bin d to th e basem en t m em bran e of m ucosal t issues. It is a ch ron ic, bilateral, papillar y cicat rizing conjun ct ivit is ( Fig. 4.9 ).

112

Color Atlas of Ophthalm ology

Fig. 4.9

Benign pemphigoid.

Presentation Age of presentat ion : Aged adu lts (rarely seen in ch ildren ) Sex : Wom en are affected t w ice as frequ en tly as m en . Main early feat ure : Ch ron ic bilateral papillar y cicat rizing conju n ct ivit is Ocular findings: Conju n ct ival hyp erem ia w ith dr y eye sym ptom s, tearing, itch ing, ph otoph obia, foreign body sen sat ion s Disch arge (i.e., catarrh al, m ucou s, m em bran ou s) Eyelids m an ifest t rich iasis, dist ich iasis, m eibom ian glan d dysfun ct ion , bleph arit is Conju n ct iva m an ifest s pap illae, follicles, kerat in izat ion , su bepith elial fibrosis, conju n ct ival kerat in izat ion w ith foresh orten ing of th e forn ices an d sym bleph aron an d/or an kylobleph aron (en d stage, im m obilizes th e eye) Corn ea m an ifests su perficial pu n ctate kerat it is, epith elial defect , u lcerat ion s, n eovascu larizat ion , kerat in izat ion , st rom al opacit ies, perforat ion (pseu dot rach om a)

Differential Diagnosis Chlamydial infections (trachom a, inclusion conjunctivitis), atopic keratoconjunctivitis, adenoviral conjunctivitis, long-term ocular m edication (old antiglaucom a m edications, e.g., epinephrine), chem ical (alkali) or therm al burns, radiation exposure (therapeutic or not), Sjögren syndrom e, sarcoidosis, derm atobullous disorders (toxic epiderm al necrolysis, congenital ichthyosiform erythroderm a, epiderm olysis bullosa), erythem a m ultiform e, Corynebacterium diphtheriae conjunctivitis, epitheliom a

Management No topical agen t is really effect ive in stop ping act ivit y (dexam eth ason e oin tm en t m ay h elp). Best con t rolled w ith system ic th erapy. To slow disease p rogression t r y th e follow ing: Subconjunctival steroid injections such as triam cinolone (Trigon, Bristol-Myers Squibb, New York) or betam ethasone (Celestone, Schering Corp., Kenilw orth, NJ). We use prolonged-release betam ethasone, injecting as m uch as possible, subconjunctivally (Celestone-Cronodose, Schering Corp., Kenilw orth, NJ the strongest “depot” steroid).

4 External Diseases

113

Abu n dan t ar t ificial tears an d topical lu brican ts in pat ien t s w ith dr y eye sym ptom s Steroid oin t m en t s (e.g., dexam eth ason e oin t m en t , t w o to four t im es daily) System ic im m u n osu ppressive th erapy, u su ally con t in ued for 9 to 12 m on th s. Mu st be con t rolled by a specialist in im m u n osup pressive th erapy (e.g., diam in odiph enylsu lfon e or cycloph osph am ide + system ic predn ison e) System ic cor t icosteroids for severely in flam ed eyes th at do n ot respon d to im m u n osu ppression alon e Treat com plicat ion s su ch as epilat ion of an aberran t lash (m ech an ical, laser, cr yodest ru ct ion ), pu n ctal occlu sion for dr y eye, or lid su rger y for en t ropion .

Stevens-Johnson Syndrome Stevens-Johnson syndrom e is an acute hypersensitivity reaction consisting of an inflam m atory vesiculobullous reaction of the skin and m ucous m em brane. Im m une complex deposition is incited by m edications, including sulfonam ides, anticonvulsants, aspirin, penicillin, isoniazid, diam ox (acetazolam ide), and m any m ore, and som etim es infectious organism s (herpes simplex virus, streptococci, adenovirus, mycoplasm a).

Presentation Th ere is an acu te on set of fever, rash , red eyes, m alaise, ar th ralgias, an d respirator y t ract sym ptom s. Classic “t arget” skin lesion s (m aculop apu les w ith a red cen ter an d a w h ite surroun d on an er yth em atous base) are con cen t rated on th e h an ds an d feet . Oth er sym ptom s in clu de u lcerat ive stom at it is an d h em orrh agic lip cru st ing. Th e m or talit y rate is 10 to 33%. Ocu lar sign s in clu de m ucopu ru len t or pseu dom em bran ou s conjun ct ivit is, episclerit is, an d irit is in th e acu te ph ase. Late com plicat ion s in clu de conju n ct ival scarring or sym bleph aron , t rich iasis, eyelid deform it ies, tear deficien cy, corn eal n eovascularizat ion , u lcer, perforat ion , or scarring. Erythem a m ult iform e m ajor (Stevens-Johnson syndrom e) : Im m u n e com plex deposit ion in th e derm is w ith su bepith elial vesiculobu llou s react ion of th e skin an d m u cou s m em bran es Erythem a m ult iform e m inor: On ly skin involvem en t Toxic epiderm al necrolysis: Th e m ost severe form , causing exten sive in t raepith elial vesicu lobu llou s erupt ion s an d epiderm al n ecrosis; m ore com m on in ch ildren an d im m un osu ppressed pat ien ts

Differential Diagnosis Ocular cicatricial pem phigoid, chem ical burns, radiation, squam ous cell carcinom a

Management Taking a h istor y to r ule out a precip itat ing factor is im por tan t . Slit-lam p exam in at ion , in clu ding eyelid eversion w ith exam in at ion of th e forn ices, conjun ct ival or corn eal cu lt ures, an d con su ltat ion w ith in tern al m edicin e, is a m u st . Hospit alize th e pat ien t , rem ove th e in it iat ing factor, an d provide sup por t ive care as th e m ain stays of t reat m en t . Ocu lar m an agem en t in cludes m an agem en t of associated dr y eye, irit is, topical steroids for ocular surface in flam m at ion , daily pseu dom em bran e peel, an d sym bleph aron lysis w ith a glass rod or m oisten ed cot ton sw ab, system ic or topical vitam in A, an d in t raven ous im m u n oglobulin . To m an age th e late com plicat ion s pen et rat ing keratop last y w ith stem cell t ran splan t , an d am n iot ic m em bran e t ran splan t , or perm an en t keratoprosth esis m ay be requ ired.

114

Color Atlas of Ophthalm ology

Dry-Eye Syndrome Most dr y eyes are m u lt ifactorial. Th e eye can be dr y as a result of defect ive product ion of tears (e.g., age-related dr y eye in m en opau sal w om en ) or excessive evaporat ion (e.g., exoph th alm os).

Presentation Sym ptom s: Foreign body sen sat ion , sen sat ion of ocu lar dr yn ess an d grit t in ess (in it ially at th e en d of th e day an d later th rough out th e w h ole day), hyperem ia an d ocu lar irrit at ion (exacerbated by sm oky or dr y environ m en t s, in door h eating system s, prolonged reading, or u se of com puters), m u cous disch arge, excessive tearing (secon dar y to reflex secret ion ) Signs: Red eyes, conju n ct ival hyperem ia, decreased tear m en iscus, in creased debris in th e tear film , su perficial pu n ctate keratopathy (w ith flu orescein , lissam in e green , an d/or rose bengal posit ive st ain ing), m u cous plaques an d disch arge, corn eal filam en t s (severe cases), corn eal epith elial defect s or ulcers (in m ore severe cases) ( Fig. 4.10 ).

Differential Diagnosis Any conjun ct ivit is (especially toxic form s, w h ich are m ore difficult to differen t iate). Careful h istor y an d w orku p sh ou ld h elp to establish th e origin of th e dr y eye (e.g., air con dit ion ing, Sjögren syn drom e)

Management Tru e dr y eye can n ot be cu red, bu t eye sen sit ivit y can be lessen ed an d m easu res taken so th e eyes rem ain h ealthy by m ean s of art ificial tears or tear su bst it u tes. No con t act len s pat ien t or glau com a t rabeculectom ized pat ien t sh ould be out of ar t ificial tear lu bricat ion or tear subst it utes! First step : Sup plem en tal lubricat ion (m ild an d m oderate kerat it is sicca) Art ificial tears: Preferably preser vat ive-free ar t ificial tears (drops 4 to 14 t im es a day, depen ding on th e severit y of th e case). Our preferred ocu lar lu brican t is sodiu m hyaluron ate (0.18 to 0.4%).

Fig. 4.10 Dry eye, lagophthalm os, corneal and conjunctival rose bengal staining.

4 External Diseases

115

Viscous artificial tear drops or gels: How ever, they tem porarily blur the vision. Lubricat ing oint m ents: For m ore severe cases (gen erally reser ved to bedt im e, becau se vision blur lasts m in utes or h ours). Not to be used w ith con t act len ses. In severe cases: Use a patch w ith lubricat ing oin t m en t at n igh t . In case of abundant m ucus: Rem ove th e m ucou s an d add 10% N-acet ylcystein e, th ree to fou r t im es daily. Art ificial tear insert (e.g., Lacrisert, Aton Pharm a, Inc., Law renceville, NJ) : Place in to th e in ferior cu l-de-sac ever y m orn ing. Special goggles, m oist ure cham ber glasses: To redu ce evap orat ion an d ret ain h um idit y aroun d th e globe. In case of suspected inflam m at ion or in case of unsat isfactory results : Tr y topical steroids (before cyclosporin e). Treat any associated abnorm alities: (e.g., in blepharitis, suppress inflam m ation w ith topical steroids and local antibiotics, and/or system ic tetracyclines) Interm ediate step : Tem porary punctal occlusion : With collagen (dissolvable) or silicon e (perm an en t) plugs in case of severe aqueou s tear deficien cy (to preser ve en dogen ou s w ater) In-office cauterizat ion of the inferior lacrim al punct i: If th e pat ien t is sat isfied w ith tem poral occlusion result s Minim ize exposure: Consider tarsorrhaphy or botulinum -toxin-induced ptosis. Last step : Su rgical t reat m en t (on ly for ver y severe cases, w ith u lcerat ion or corn eal perforat ion ) Closure of perforat ion or descem etocele : Cyan oacr ylate t issu e adh esive Corneal or corneoscleral patch : For an im pen ding or fran k perforat ion (am n iot ic m em bran e, fascia lat a) Lateral tem porary tarsorrhaphy : For exam ple, after facial n er ve paralysis, after t rigem in al n er ve lesion s, or for severe exoph th alm os secon dar y to thyroid disease Conjunct ival flap: Aids in preven t ing corn eal m elt an d perforat ion by covering th e corn ea w ith conjun ct iva Several variet ies of con tact len ses can aid in th e t reat m en t of dr y eye. Hard con tacts m ay st im u late reflex tearing an d th u s in crease th e volu m e of tears. Som e h ard scleral con t act len ses m ay be ben eficial by preven t ing evap orat ion from a large por t ion of th e ocular su rface. Th e U.S. Food an d Drug Adm in ist rat ion h as approved on e t ype of con tact len s (Proclear Toric XR len s, CooperVision , Fairpor t , NY) w ith an in dicat ion for dr y eyes. Th is is a soft len s th at h as som e u n iqu e proper t ies. It has a h igh w ater con ten t like oth er soft len ses, bu t it also h as a com pon en t th at retain s w ater bet ter th an m ost oth er soft len ses. Th is reduces th e dehydrat ion th at occu rs w ith m ost soft con tact len ses. Clin ical st u dies h ave dem on st rated im provem en t in com fort an d sign s of dr yn ess on th e surface of th e eye w ith th is con t act lens w h en com pared w ith a grou p of oth er len ses w ith w h ich it w as tested. Neverth eless, con tact len ses alon e h ave n o place in th e t reat m en t of dr y eyes; con com itant u se of art ificial teardrops (an d periodic ch ecku ps) is essen t ial. In som e cases, sm all pu n ctal plugs m ay be in ser ted in th e in ferior lacrim al pun ct u m to slow drain age an d loss of tears. Cyclosporine 0.05% ophthalm ic em ulsion (Restasis, Allergan, Inc., Irvine, CA) : Can be u sed to relieve dr y eyes caused by su ppressed tear produ ct ion secon dar y to ocu lar in flam m at ion . Safet y for u se in ch ildren an d du ring p regn an cy h as n ot been establish ed. Autologous serum : It h as a ben eficial effect on corn eal epith elium (20% au tologou s serum dilu ted in n orm al salin e).

116

Color Atlas of Ophthalm ology

Tet racyclines: Tradit ion ally u sed as an t ibiot ics, th ey also h ave im port an t an t iin flam m ator y proper t ies. Ver y u sefu l (doxycyclin e) in t reat ing acn e rosacea dr y eye. Tet racyclin es are also effect ive again st recu rren t corn eal erosion s an d ph lycten u lar keratoconju n ct ivit is. Oral pilocarpine (Salagen , Novart is Pharm aceut icals UK Ltd., Surrey, UK) : In it ially u sed in xerostom ia (salivar y glan d dysfu n ct ion , com m on after irradiat ion of th e n eck in on cology), it h as been fou n d u sefu l in severe xeroph th alm ia by st im ulat ing tear secret ion (5 to 10 m g/8 h ). Good result s are seen in Sjögren syn drom e after a few w eeks of t reat m en t . Salivary gland t ransplantat ion into the inferior tarsal conjunct iva : Has been repor ted to be usefu l in dr y-eye con dit ion s w ith severe perm an en t lacrim al glan d dysfu n ct ion s (n ot u sefu l in Sjögren syn drom e). Tech n ically com p lex.

Symblepharon Sym bleph aron is an adh esion bet w een th e palpebral conjun ct iva an d th e bulbar conju n ct iva or corn ea. Sym bleph aron is usually th e result of a t raum a (surger y, ch em ical or radiat ion bu rn s) or sup erficial eye in flam m at ion (er yth em a m u lt iform e, t rach om a, burn s, pem ph igoid, Steven s-Joh n son syn drom e).

Presentation Sym ptom s in clu de adh esion bet w een th e palpebral conju n ct iva an d th e bulbar conju n ct iva or corn ea, lid deform it ies, fu n ct ion al lacrim al occlu sion an d tearing, en t ropion , an d dist ich iasis ( Fig. 4.11 ).

Differential Diagnosis Diagnosis is apparently clinical and is seldom confused w ith other disease entities .

Management Treatm ent is often frustrating, and progressive scarring cannot be com pletely controlled or reversed. Grow th prevention includes breaking early adhesions w ith a rectal therm om eter t w ice a day and steroid ointm ents. Conform ers can be tried. Surgical Z-plast y w ith am niotic m em brane or m ucosal graft can be tried. The use of

A

B Fig. 4.11 Symblepharon: (A) bet ween bulbar and palpebral conjunctiva; (B) at the lateral canthal angle.

4 External Diseases

117

am niotic m em brane can be beneficial in that it facilitates epithelialization, m aintains norm al epithelial phenot ype (w ith goblet cells w hen perform ed on the conjunctiva), and effectively helps in reducing inflam m ation, vascularization, and scarring. Im m unosuppressant therapies (m ethotrexate, cyclophospham ide, cyclosporine, azathioprine, etc.) can help in the acute episodes of the illness. In late cicatricial stages, treat collateral effects such as dr y eye syndrom e, and punctal occlusion.

Pinguecula, Pterygium, and Lipoid Degeneration Pinguecula Pinguecu lae are th e m ost com m on ben ign conjun ct ival t u m ors. Th ey u su ally affect m iddle-aged in dividuals bu t can also be foun d in ch ildren an d young peop le, especially th ose w ith dyslipidem ia. Pingu ecu lae h ave n o sex or racial predilect ion . Th ey seem related to ch ron ic exposure to th e su n .

Presentation Pingueculae are usually asym ptom at ic, yellow ish /w h it ish , sligh tly raised, in terpalpebral lipid-like dep osits in th e n asal (m ore frequen t) an d tem poral lim bal con jun ct iva. Un i- or bilateral (usually bilateral an d asym m et rical), th ey m ay becom e vascu larized an d in flam ed (pingu eculit is). In a case of in flam m at ion , pat ien t s experien ce foreign body sen sat ion an d conju n ct ival redn ess arou n d th e pingu ecu la, corn eal p un ct ate epith eliopathy, an d corn eal th in n ing secon dar y to dr yn ess (dellen u lcerat ion ). Th ese pat ien ts m ay com plain of dr y-eye sym ptom s an d foreign body sen sat ion ( Fig. 4.12A,B).

Fig. 4.12 (A) Pingueculae. (Continued on page 118)

A

118

Color Atlas of Ophthalm ology

B Fig. 4.12

(Continued) (B) Pinguecula and melanoma.

Differential Diagnosis Pter ygiu m , conju n ct ival derm oid, conju n ct ival n eoplasia (a u n ilateral, w h ite, vascularized m ass), p h lycten ule, pan n u s, conju n ct ival reten t ion cyst (a clear, fluidfilled sac), lim bal follicles

Management No t reat m en t is n ecessar y in cases w ith good lubricat ion resu lts, w h ich h ave a low er risk of pingu eculae form at ion (preser vat ive-free tear subst it u tes are preferable). Con sider surgical part ial or tot al resect ion u n der local an esth esia in th e follow ing: Severe cases (gian t pingueculae) w h ere pter ygiu m is presen t an d in terfering w ith vision Ch ron ically in flam ed severe cases Un com for table con t act len s w ear Bad corn eal lu bricat ion w ith dellen form at ion Cosm et ic problem s In th e case of in flam m at ion , u se ocu lar lu bricat ion an d m ild topical steroids (e.g., flu orom eth olon e), w ith decongest an ts (e.g., n aph azolin e). Preven t ion is possible for peop le w ith occupat ion s or h obbies th at in crease th e risk of pinguecu la (sailing, fish ing, skiing, garden ing, outdoor con st ru ct ion w ork). Sun goggles, u lt raviolet-blocking coat ings, or goggles th at lim it dust exposure are h elpful.

Pterygia Like pinguecu lae, pter ygia h ave been related to exposu re to u lt raviolet ligh t (both ult raviolet A [UV-A] an d UV-B). Risk factors in clu de living in su bt ropical an d t ropical clim ates, ou tdoor act ivit ies (e.g., golf, sailing, fish ing), dr y w in dy clim ates, dust , an d fu m es. A gen et ic predisposit ion h as been described. Th ere are t w o groups of pter ygium pat ien t s: pter ygia w ith m in im al proliferat ion an d at roph ic appearan ce

4 External Diseases

119

A

B

Fig. 4.13 (A) Pterygium. (B) Pte rygium postsurgical papilloma. (C) Terrien’s marginal degeneration. C

(slow -grow ing pter ygia, w ith low in ciden ce of recu rren ce after excision ) an d elevated fibrovascu lar pter ygia (rapid grow th , aggressive clin ical cou rse, an d h igh rate of recu rren ce after excision ) ( Fig. 4.13A,B,C).. Th ere is a predilect ion for m ales, an d occu rren ce is m ore frequ en t in th e n asal conju n ct iva.

120

Color Atlas of Ophthalm ology

Presentation A t riangular, fleshy, elevated m ass of bu lbar conjun ct iva grow s over th e corn ea w ith in th e in terpalpebral fissure. Sm all pter ygia are asym ptom at ic. Th ey can becom e in flam ed, w ith redn ess, foreign body sen sat ion , an d ocular irrit at ion (dr yeye sym ptom s). In advan ced cases, pat ien t s experien ce vision problem s (ast igm at ism , progression over th e visu al axis), rest rict ion of ocular m ot ilit y, an d blin dn ess (in u n derdeveloped cou n t ries w h ere surger y is u n available).

Differential Diagnosis Pinguecu lae, am yloidal degen erat ion of th e conju n ct iva, pseu dopter ygium , n eoplasia (e.g., carcin om a in sit u , squ am ous cell carcin om a)

Management Good in ten sive lubricat ion is essen t ial. Topical steroids su ch as predn isolon e acetate (Pred For te, Allergan , In c., Ir vin e, CA) 1%, th ree to fou r t im es daily an d an t ih ist am in es provide relief of in flam m at ion . In dicat ion s for surger y in clude th e follow ing: Cosm et ic reason s Discom for t due to recu rren t in flam m at ion Before it en croach es on th e pu pillar y area Mult ip le differen t surgical procedu res w ork in th e first grou p of pter ygiu m pat ien t s (at roph ic, slow grow ing), bu t n on e can be said to w ork sat isfactorily in th e secon d group of pat ien ts (fast grow ing, aggressive clin ical course): Sim p le excision Sim p le excision an d repair w ith conju n ct iva autop last y Sim p le excision an d sliding flaps of conjun ct iva With an d w ith ou t adjun ct ive extern al β radiat ion th erapy (1000 to 2000 rep s at lim bu s or th iotepa 1:2000 solu t ion ) an d/or in t raoperat ive topical m itom ycin - C or postoperat ive 5-flu orou racil Prim ar y excision plus free graft s of conjun ct iva an d lim bal t issu e (lim bal au tograft) or am n iot ic m em bran e: for aggressive or recu rren t pter ygia

Lipoid/Lipid Degeneration of the Cornea and Conjunctiva Lipid degen erat ion of th e corn ea an d conju n ct iva m ay occur in prim ar y or secon dar y form . Th e prim ar y form is usu ally bilateral an d m ore diffu se an d is cau sed by lipid seru m dyscrasias su ch as Tangier fam ilial h igh -den sit y lipoprotein deficien cy or lecith in ch olesterol acylt ran sferase deficien cy. Th e secon dar y form is by far th e m ost com m on form of th is rare disease, is m ore localized, an d is due to th e presen ce of corn eal blood vessels after ocu lar t rau m a or in terst it ial or h erpet ic kerat it is.

Presentation W h ite, yellow, or cream -colored den se opacificat ion of th e corn eal st rom a is seen in a diffu se or localized m an n er. Ch olesterol cr yst als occu r in th e corn eal or con jun ct ival st rom a su rrou n ding aberran t blood vessels. Th e conju n ct ival feeder vessels are dilated, an d th ere are sym ptom s of dr y eye ( Fig. 4.14 ).

Differential Diagnosis Aspect at slit lam p is diagn ost ic. Ru le out degen erated epith eliom a.

4 External Diseases

121

Fig. 4.14 Cornea conjunctival lipoid degeneration.

Management Treat th e u n derlying con dit ion to redu ce th e risk of progression (reduce fat serum levels). At tem pt closing th e feeder vessels w ith argon laser ph otocoagu lat ion . Severe cases w ith affected pu pillar y area m ay requ ire pen et rat ing keratoplast y, alth ough th is degen erat ion m ay recur in th e graft .

Conjunctival Retention Cysts Conjun ct ival lym ph at ic cysts are soft , t ran slu cen t , an d m obile. Large cyst s m ay give dr y eye or foreign body sen sat ion (eye discom for t or tearing). A conju n ct ival cyst m ay reveal differen t possible origin s an d can be due to parasit ic in festat ion (e.g., cist icercosis), w h ich h as to be ruled ou t w h en th e p at ien t h as t raveled to en dem ic region s, or it m ay be an accessor y lacrim al glan d, a post t raum at ic in clusion cyst (of conjun ct ival epith eliu m ), or sim ply a lym ph at ic cyst (th e m ost com m on ).

Presentation Soft , t ran slucen t , an d m obile flu id-filled m ass w ith in th e conjun ct iva, alon e or in groups, fou n d at slit-lam p exam in at ion . More frequ en t in th e bu lbar conju n ct iva an d in th e in ferior tarsu s or in ferior cul-de-sac ( Fig. 4.15 ).

Fig. 4.15

Conjunctival lymphatic retention cyst.

122

Color Atlas of Ophthalm ology

Differential Diagnosis Cyst of m align an t origin (ru le out parasit ic in festat ion an d h em at ic dyscrasia)

Management Th ese cysts n eed n o t reat m en t u n less th ey cau se persisten t dr y eye or foreign body sen sat ion . Topical lubrican t s provide relief. Th eir w all can be easily broken in feriorly w ith a few sh ot s of n eodym ium :yt t rium -alu m in um -garn et laser app lied in feriorly on th e cyst w alls, w ith care to avoid conju n ct ival vessels. Postsu rgical cyst can be excised.

Superior Limbic Keratoconjunctivitis Th is con dit ion is of un kn ow n et iology an d associated w ith thyroid disease. In su perior lim bic keratoconjun ct ivit is, conju n ct ival laxit y m igh t in du ce in flam m ator y ch anges from m ech an ical t raum a of hyper t roph ic pap illae of th e u pper t arsal con jun ct iva. It affects m ain ly adu lt w om en . A sim ilar con dit ion is foun d in soft con tact len s w earers using th im erosal-preser ved solut ion s.

Presentation Sym ptom s in clu de tearing, bu rn ing, superior hyp erem ia, an d foreign body sen sat ion . Pat ien ts w ith filam en ts are ext rem ely sym ptom at ic. In flam m at ion of th e su perior bu lbar conjun ct iva result s in edem a w ith redun dan t conjun ct iva. Th ere is predom in an t involvem en t of th e superior lim bu s, adjacen t epith elial kerat it is, an d papillar y hyper t rophy of th e u pper t arsal conjun ct iva. Th e in ferior conjun ct iva an d corn ea appear n orm al. Th e age range is 20 to 70 years. Occu rren ce is predom in an tly in fem ales, is usu ally bilateral, an d m ay be asym m et rical, w ith rem ission s an d exacerbat ion s. Dr y eye is often presen t ( Fig. 4.16 ).

Fig. 4.16

Superior limbal keratoconjunctivitis.

4 External Diseases

123

Differential Diagnosis Clin ical fin dings are diagn ost ic (in flam m at ion of th e su perior bu lbar conju n ct iva w ith absolutely n orm al appearan ce of th e in ferior conjun ct iva an d corn ea).

Management Con ser vat ive t reat m en t offers on ly tem porar y relief of sym ptom s (pressu re patch ing, Plan o T ban dage con tact len s, Baush & Lom b In c., St . Lou is, MO). Silver n it rate (0.5 to 1.0% solut ion ) is applied w ith a sat urated cot ton sw ab. It u su ally relieves sym ptom s for 1 m on th an d can be repeated safely. Topical an t ih istam in es an d m ast cell st abilizers (e.g., olopat adin e hydroch loride 0.1%, ketot ifen fu m arate 0.025%, azelast in e hydroch loride 0.05%) are h elpfu l. Moist urizing drop s an d oin tm en ts provide on ly m in im al relief. Cr yoth erapy an d a su rgical approach can be taken in recalcit ran t cases involving resect ion or recession of th e su perior bu lbar conju n ct iva or in -office th erm ocau terizat ion of th e su perior bulbar conju n ct iva w ith a disposable oph th alm ic cauter y u n der topical an esth esia. Th e u se of topical steroids is usually in effect ive.

Episcleritis Episclerit is is a t ran sien t , self-lim ited in flam m ator y process of th e episclera. Most episclerit is is idiopath ic, but it can also be associated w ith system ic disorders su ch as rh eu m atoid ar th rit is, acn e rosacea, an d atopy.

Presentation Th ere is an acute on set an d diffuse or n odu lar ocu lar redn ess w ith out irrit at ion or pain . In flam ed ep iscleral vessels radiate posteriorly from th e lim bus (Fig. 4.17A,B).

Fig. 4.17 (A) Episcleritis. (Continued on page 124)

124

Color Atlas of Ophthalm ology

Fig. 4.17

(Continued) (B) Nodular episcleritis.

Differential Diagnosis Sclerit is, uveit is, conjun ct ivit is

Management Th e con dit ion is self-lim ited; n o t reat m en t is n eeded. Topical steroids or n on steroidal an t iin flam m ator y drugs (NSAIDs) or a com bin at ion of th ese can accelerate recover y. Vasocon st rictors an d refrigerated art ificial tears p rovide relief.

Scleritis Sclerit is is a gran u lom atous in flam m at ion of th e sclera th at m ay presen t in associat ion w ith system ic diseases such as rh eu m atoid ar th rit is, system ic lu pu s er yth em atosu s, polyarterit is n odosa, or Wegen er gran ulom atosis. Sclerit is can be self-lim it ing or can progress to a p oten t ially blin ding n ecrot izing process. Possible com plicat ion s in clude scleral th in n ing (especially in recurren t sclerit is), sclerom alacia perforan s, sclerosing kerat it is, perip h eral corn eal m elt ing, uveit is, cat aract , m acu la edem a, ch oroidal gran ulom as, an d ret in al det ach m en t . Posterior sclerit is occurs m u ch less frequen tly th an an terior sclerit is an d m ay exten d in to th e an terior segm en t of th e eye. In cases of n ecrot izing sclerit is, in fect ion h as to be ruled out .

4 External Diseases

Fig. 4.18

125

Scleritis table.

Presentation Th ere is severe ocu lar pain w ith or w ith ou t decreased vision (suspect posterior involvem en t if vision is com prom ised). On set of sclerit is is m ore gradu al th an is seen in episclerit is an d is accom pan ied by u n i- or bilateral red eye, lacrim at ion , an d ph otoph obia ( Fig. 4.18 ).

Differential Diagnosis Conju n ct ivit is, uveit is, episclerit is

Management Man agem en t in cludes system ic NSAIDs (e.g., in dom eth acin , ibuprofen , n aproxen ), topical steroids (e.g., bet am eth ason e, dexam eth ason e) an d system ic oral steroids (oral steroids preferred over topical), an d im m u n osup pressan t th erapies (e.g., m eth ot rexate, cycloph osph am ide, cyclosporin e, azath ioprin e). System ic t reat m en t w orks bet ter th an topical, w h ich h as to be con sidered accessor y to a system ic an t iin flam m ator y regim en .

Dilated Vessels Dilated Vessels and Ocular Vein Varicosities Dilated vessels are com m on ly seen in h ealthy pat ien t s, especially w om en w ith ven ou s in su fficien cy. Th ey m ay be a sign of ven ous congest ion or raised episcleral pressure, eith er due to carot id cavern ous fist u la or to any orbit expan sive process or in flam m ator y disease of th e eye. Dilated episcleral sen t in el vessels can be presen t in cases of m align an t m elan om a of th e ch oroid or ciliar y body.

126

Color Atlas of Ophthalm ology

Fig. 4.19

Dilated vessels.

Presentation Presen t at ion is asym ptom at ic. Foreign body sen sat ion occurs in th e case of varicosit ies. Oth er sign s an d sym ptom s of red eye or orbit expan sive process m ay also be seen . Thyroid disease presen t s w ith dilated vessels, m ain ly over th e extern al rect us m u scles ( Fig. 4.19 ).

Differential Diagnosis Any cau se of red eye (conjun ct ivit is, idiopath ic raised ep iscleral pressure, ven ous in sufficien cy, carot id cavern ou s sin us fist u la, episclerit is, sclerit is, ch ron ic irritat ion , glau com a su rger y)

Management No t reat m en t is n eeded if th e con dit ion is idiopath ic. Varicosit ies can be successfully redu ced using argon laser ph otocoagulat ion . Th e occasion al u se of vasocon st rictors im proves cosm et ics but th ere is n o defin it ive t reat m en t . Treat th e con com it an t illn ess.

Dilated Episcleral Vessels in Sturge -Weber Syndrome St u rge-Weber syn drom e is a rare n eu rological disorder presen t at birth , ch aracterized by a birth m ark (u sually on th e face) kn ow n as a port-w in e st ain cau sed by an overabun dan ce of capillaries arou n d th e t rigem in al n er ve ben eath th e surface of th e face. Neu rological problem s arise du e to a loss of n er ve cells an d calcificat ion of t issu e in th e cerebral cortex of th e brain on th e sam e side of th e body as th e bir th m ark (angiom atosis of th e cen t ral n er vous system ). Neu rological sym ptom s in clu de seizu res th at begin in in fan cy an d m ay w orsen w ith age. Convulsion s usu ally h appen on th e side of th e body opposite th e birth m ark an d var y in severit y. Th ere m ay be m u scle w eakn ess on th e sam e side. Som e ch ildren w ill h ave developm en t al delays an d m en tal retardat ion . St u rge-Weber syn drom e rarely affects oth er body organ s.

4 External Diseases

Fig. 4.20

127

Sturge-Weber syndrom e table.

Presentation Ch aracterist ic facial bir th m ark (can var y in color from ligh t pin k to deep purple). Most pat ien t s w ith angiom a th at affect s th e su perior lid h ave glaucom a at bir th or w ill develop it later. Dilated episcleral vessels are seen , along w ith bup h th alm os, seizu res, convulsion s, m uscle w eakn ess, m en tal ret ardat ion or learn ing disabilit ies, an d possible developm en t al delay (Fig. 4.20 ).

Differential Diagnosis Facial angiom a w ith ou t n eu rological m an ifestat ion s, oth er cau ses of elevated episcleral pressu re (e.g., thyroid oph th alm opathy, th rom bosis of th e cavern ous sin u s, carot id-cavern ou s fist ula)

Management Treat th e sym ptom s. Treat in creased in t raocu lar pressu re w ith eyedrops (avoid m iot ics). Su rger y can be perform ed on serious cases of glaucom a. Laser t reat m en t can ligh ten or rem ove th e facial bir th m ark. An t iconvu lsan t m edicin es are u sed to con t rol seizu res. Physical th erapy is h elpfu l in ch ildren w ith m u scle w eakn ess.

Carotid Cavernous Sinus Fistula Carot id cavern ous fist u las are un i- or bilateral abn orm al com m u n icat ion s (sh u n t s) of th e carot id ar teries directly or in directly in to th e vein s of th e cavern ous sin u s. Th ey are frequ en tly cau sed by t raum a, alth ough th ey can be spon t an eou s in m en opau sal w om en . Depen ding on th e am ou n t of blood injected in to th e sin u ses, th ey can produce differen t am ou n t s of n eu ro-oph th alm ological m an ifest at ion s.

Presentation Patients present w ith arterialized dilated vessels of the conjunctiva and orbit, unilateral or bilateral, proptosis, lid edem a, papilledem a, retinal edem a w ith hem orrhages, uveitis, secondary glaucom a associated w ith elevated venous pressure, anterior segm ent ischem ia, and iris atrophy. There can also be visual loss and dysfunction of the extraocular m uscles. Pulsating exophthalm ia occurs in advanced (i.e., high-flow )

128

Color Atlas of Ophthalm ology

cases. Patient refers to an audible sound that follow s the heart rate. Cavernous sinus syndrom e (cranial ner ve II, IV, and VI palsy) and Tolosa-Hunt syndrom e m ay also present (Fig. 4.21A,B).

Differential Diagnosis Cavern ous sin u s th rom bosis, du ral sin u s ar terioven ou s fist u la, cavern ou s sin us ar terioven ou s m alform at ion s, orbital pseudot um or, thyroid orbitopathy, orbital am yloidosis, orbital t u m or, orbit al cellu lit is, m ucorm ycosis

A

B Fig. 4.21 (A) Carotid cavernous fistula. (B) Carotid cavernous fistula.

4 External Diseases

129

Management Neu rosu rger y by a n eu roradiologist (det ach able balloon or m etallic coil em bolizat ion ) accom plish es reversal of ocu lar m an ifest at ion s in p at ien ts w ith vision at risk or pat ien ts w ith in tolerable sym ptom s (h igh -flow sh un ts). Low -flow sh u n t s (m ain ly t raum at ic) spon t an eou sly occlude w ith t im e. Th e gam m a kn ife can be used w h ere available.

Pigmented Conjunctival Lesions Ocular or Oculodermal Melanocytosis (Congenital Melanosis Oculi) Ocular Melanocytosis Th is n on h eritable congen it al hyperpigm en tat ion of th e eye h as in creased frequ en cy in w h ites. Path ology sh ow s an in creased n um ber of m elan ocytes in th e affected t issu es. Th e con dit ion can progress w ith th e u se of topical prostaglan din an alogues (e.g., latan oprost) u sed to t reat glau com a.

Presentation Presen tat ion in clu des in creased gray or bluish pigm en tat ion of th e globe (sclera an d episclera) th at is u su ally un ilateral ( Fig. 4.22 ).

Differential Diagnosis Prim ar y acqu ired m elan osis (affect s th e conju n ct iva on ly an d oth er m u coses), ocu loderm al m elan ocytosis (n evus of Ot a), n evu s, m elan om as, pigm en ted dep osits, foreign body iron deposit s

Fig. 4.22

Ocular melanocytosis.

130

Color Atlas of Ophthalm ology

Management No t reat m en t is n eeded. Carefu l periodic ocular exam in at ion s are required becau se of th e in creased risk of uveal m elan om as in affected in dividuals.

Oculodermal Melanocytosis Th is con dit ion is caused by diffuse dist ribut ion of proliferated m elan ocytes an d is m ore com m on in Asian s an d blacks. It is associated w ith ip silateral glau com a.

Presentation Asym ptom at ic. Un ilateral facial an d/or blue-gray pigm en t at ion of th e globe, usually follow ing th e ipsilateral dist ribu t ion of th e t rigem in al n er ve (V1 an d V2) (Fig. 4.23 ).

Differential Diagnosis Prim ar y acqu ired m elan osis, n evu s, m elan om as, pigm en ted dep osits, foreign body iron dep osits, facial angiom a

Management No t reat m en t is n eeded. Ru le out glaucom a, ret in it is pigm en tosa, an d congen it al cataract . Ocu loderm al m elan ocytosis rarely u n dergoes m align an t t ran sform at ion .

Fig. 4.23

Oculoderm al melanocytosis. Heterochromia.

4 External Diseases

131

Conjunctival Pigmentations 1 Melan ocyt ic ben ign conjun ct ival lesion s are ver y com m on , especially in darkerskin n ed in dividu als, in clu ding blacks or African Am erican s, Hispan ics, an d Git an os, in th e lim bal area or caru n cle. Presen t at ion greatly differs from on e pat ien t to an oth er. Th e lesion s h ave n o m align an t poten t ial, alth ough n evi ver y rarely give rise to m align an cy (jun ct ion al an d com pou n d n evi). Ch ron ic u se of topical epin eph rin e (as a cosm et ic), silver-con tain ing com pou n ds (argyrosis), ret ain ed iron foreign body, atabrin e, tet racyclin es, clofam icin e, im bibed m ascara gran u les, radiat ion , h orm on es (pregn an cy, Addison disease), ch em icals (arsen ic, th orazin e), an d som e ch ron ic disorders (like t rach om a or xeroderm a pigm en tosu m ) are poten t ial cau ses of pigm en t at ion in crease (darken ing of th e lesion s). Never th eless, th e m ost com m on cau se of pigm en t at ion in crease is th e topical use of prostaglan din an alogues to low er in t raocular pressure. Conjun ct ival pigm en t at ion is n o sign of m align an cy.

Presentation Conju n ct ival ben ign epith elial m elan osis presen t s as a flat brow n ish pigm en t at ion seen especially in blacks or African Am erican s, in th e lim bal area or caru n cle. Con jun ct ival p igm en ted lesion s close to th e lacrim al glan d can be ben ign , bu t alw ays con sider m align an cy because m align an cy m ay be m ore exten sive th an is clin ically apparen t . Su spect m align an cy if pigm en t at ion in creases in parallel to su rface grow th ( Fig. 4.24A– D).

Differential Diagnosis Conju n ct ival m elan om a

A

B

C

D Fig. 4.24

(A–D) Conjunctival pigm entations.

Nom en clat u re m ay be con fu sin g, an d classificat ion is often con t roversial: w e h ave u sed th e m ost w id ely accepted n am es. 1

132

Color Atlas of Ophthalm ology

Management Use close obser vat ion an d follow -u p w ith ph otograph ic docu m en tat ion . Fluorescein angiography m ay h elp to visualize feeder vessels in m elan om as. In case of dou bt , excision al biopsy sh ou ld be p erform ed.

Malignant Melanoma of the Conjunctiva Malign an t m elan om a of th e conjun ct iva is rare, but it is th e m ost com m on pigm en ted m align an cy of th e conjun ct iva an d accou n t s for 2% of all ocu lar m align an cies. It is far less com m on th an in t raocular ch oroidal m elan om a. It can appear in a previou sly h ealthy par t of th e conju n ct iva (de n ovo, from m elan ocyt ic cells of th e basal layer of conju n ct iva, in ~20 to 25% of cases), from a preexist ing conju n ct ival jun ct ion al or com p oun d n evu s (~20%of cases), from a prim ar y acquired conju n ct ival m elan osis (~60% of cases). Th e in ciden ce of conjun ct ival m elan om a is in creasing am ong w h ite m en in a t ren d sim ilar to th at of skin m elan om a. Conju n ct ival m elan om a is probably related to su n exposu re ( Fig. 4.25 ).

Presentation Clin ical presen tat ion is variable, w ith a raised, pigm en ted or n onpigm en ted lesion (som e h ave lit tle or n o pigm en t , w h ich is rare) th at grow s an d/or bleeds an d/or fixates to th e un derlying t issu es. Periph eral corn eal in filt rat ion is possible. Som e grow aroun d th e lim bu s. Som et im es th ere is pigm en t at ion of th e lid m argin s or lid skin (rare, poor progn osis). Region al lym ph n odes (parot id, p reau ricu lar, subm an dibu lar, an d cer vical) m ay be affected. Distan t m et ast ases are possible, w ith spread by th e lym ph at ic vessels an d bloodst ream . Direct exten sion to th e eyeball an d orbit is possible if th ere is late diagn osis. Susp ect m elan om a if a preexist ing n evu s grow s or h as in creased vascularit y a preexist ing conjun ct ival n evus at th e lim bu s h as rap id ver t ical grow th a p reexist ing n evu s grow s or h as in creasing n odu larit y or ch anges in pigm en t at ion or bleeds, or develops in flam m at ion a previou sly flat area of pigm en tat ion develop s n odu lar th icken ing th ere is local conjun ct ival in creased vascu larit y (w ith or w ith out a pigm en ted lesion )

Fig. 4.25

Semilunar fold melanoma.

4 External Diseases

133

th e conju n ct iva fixates to th e sclera you obser ve h em orrh age in a pigm en ted lesion you obser ve an u lcerat ive area in th e conjun ct iva th at does n ot h eal w ith topical m edicat ion

Differential Diagnosis Ben ign pigm en ted lesion s [n evu s, m elan osis, foreign body, pigm en ted dep osits (ch em icals, m ascara gran u les)]. Suspect m align an t m elan om a in th e case of a grow ing pigm en ted lesion . Not all conju n ct ival m elan om as are pigm en ted; th ey can look like a squam ou s or sebaceous glan d carcin om a, a papillom a, lym ph oid hyperplasia, an d even a pter ygiu m . Metastat ic t u m or to th e conju n ct iva (ext rem ely rare, e.g., secon dar y m elan om a from cut an eou s t um or).

Management Man agem en t depen ds on path ological staging [varies in differen t cou n t ries: clin ical t um or, n ode, m et ast ases (TNM) classificat ion , path ological classificat ion , an d h istopath ological t ype an d grade]. Can be t reated con ser vat ively w ith th e proton accelerator. Com plete resect ion of t u m or (w ith care to avoid t issue dissem in at ion ). Path ological exam in at ion is requ ired at th e t im e of excision . Conju n ct ival m elan om as at th e lim bu s: Absolu te alcoh ol epith eliectom y + w ide local resect ion (par t ial lam ellar scleroconju n ct ivoten on ectom y w ith a 2–3 m m clear zon e) + cr yoth erapy of th e bed an d borders of excision Palpebral conju n ct ival m elan om as (or forn ical): Surgical resect ion w ith absolute alcoh ol t reat m en t to th e scleral base an d cr yoth erapy to th e surrou n ding conju n ct iva Large invasive m elan om as: Orbit al exen terat ion ? (alm ost alw ays m etast ases h ave already occurred at th e t im e of t reat m en t). We prefer th e proton accelerator. A con ser vat ive approach w ith topical m itom ycin - C is un der research . Adju n ct ive radioth erapy an d/or ch em oth erapy m ay be n eeded. Life-long close obser vat ion follow -up w ith palpat ion of lym ph n odes (th ree to fou r t im es yearly)

Blue Sclera and Scleral Staphyloma Blue Sclera in Osteogenesis Imperfecta Blu e sclerae in in fan cy an d ch ildh ood reflect eith er an abn orm al th in n ess of th e sclera or an in creased scleral t ran sparen cy. Th e u n derlying uveal pigm en t produ ces th e blu e-gray appearan ce. Sligh t blue sclerae are com m on in n eon ates, part icu larly if th ey are prem at u re. It can be con sidered a n orm al varian t in th e first several m on th s of life. In case of persist ing pron oun ced blu en ess, op h th alm ological evaluat ion is n eeded to ru le ou t th e presen ce of elevated in t raocular pressu re (IOP). Pediat ric an d or th opedic evalu at ion is n eeded to rule ou t in h erited diseases resp on sible for th e blue-t in ted sclerae, such as osteogen esis im perfect a. Osteogen esis im p erfect a is a rare in h erited congen it al disorder w ith ext rem e bon e fragilit y, caused by m ut at ion s in th e gen es th at codify for t yp e I procollagen ( COL1A1 an d COL1A2 ). At least four t ypes of osteogen esis im perfect a h ave been

134

Color Atlas of Ophthalm ology

Fig. 4.26

Osteogenesis imperfecta. Blue sclera.

described. Th e age w h en fract ures begin varies w idely. Pat ien t s w ith m ild form s m ay presen t w ith fract ures in in fan cy or m ay n ot h ave fract ures u n t il adu lth ood. Th e m ore severe cases m ay h ave fract u res in u tero.

Presentation Presen t at ion is asym ptom at ic; th ere are n o ocu lar sym ptom s. In pat ien ts w ith osteogen esis im perfect a, th e sclera can be blue or w h ite ( Fig. 4.26 ).

Differential Diagnosis Blue sclera also m ay occu r in oth er disorders, su ch asalkapton uria, progeria, cleidocran ial dysplasia, cu t is laxa (pseu doxan th om a elast icum ), Eh lers-Dan los syn drom e, Marfan syn drom e, Ch en ey syn drom e, Men kes syn drom e, an d pykn odysostosis. Som e h igh or ext rem e m yopes m ay presen t w ith “blu e-sclera” due to scleral th in n ing.

Management No ocu lar t reat m en t is n eeded. Oth er system ic invest igat ion s an d t reat m en t are to be don e. Gen et ic coun seling is h elpfu l.

Blue Sclera and Scleral Staphylomas Congen it al glaucom a in an in fan t or a you ng ch ild is ch aracterized by th ree m ain sym ptom s: excessive tearing (epiph ora), sen sit ivit y to ligh t (ph otoph obia), an d spasm s or squ eezing of th e eyelids (bleph arospasm ). Th ere are both prim ar y (m aldevelopm en t of an terior ch am ber angle) an d secon dar y form s w ith diverse cau ses. Th e con dit ion is m ore frequen tly bilateral. Its severit y is variable (Fig. 4.27 ). W h eth er en largem en t of th e eye occurs or n ot depen ds on th e age of on set of th e glau com a. Th e eye being disten sible in in fan cy, elevated in t raocu lar pressure m ay result in eye en largem en t (buph th alm os). Corn eal en largem en t can also occur in th e early first years, an d th e sclera can expan d during th e first decade. Scleral th in n ing (ect asia) an d bu lging blu ish areas of th in n ed sclera lin ed by uveal t ract (staphylom a) can also be seen in advan ced cases. Th e corn eal h orizon tal diam eter also in creases. Corn eal en largem en t resu lts in ru pt u res in th e Descem et m em bran e (Haab st riae).

4 External Diseases

135

Fig. 4.27 Staphylomas in congenital glaucoma buphthalmos.

Presentation Epiph ora, p h otoph obia, an d blep h arospasm are seen in a n ew born or you ng in fan t . Bu ph th alm os, ectasia, st aphylom as, an d in creased corn eal diam eter m ay also be presen t . Corn eal edem a or Haab st riae m ay be seen at slit-lam p exam in at ion , (w h ich m ay n ot alw ays easy to perform in babies).

Differential Diagnosis With causes of blu e sclera (e.g., osteogen esis im perfecta), n eoplasias, an d causes of congen ital glaucom a: Dysgen esis of th e iris, angle, an d periph eral corn ea w ith or w ith out system ic abn orm alit ies (e.g., Rieger an om aly syn drom e, Axen feld an om aly syn drom e, Peter an om aly, an iridia, Marfan syn drom e, Weill-March esan i syn drom e) Ph akom atoses (e.g., n eu rofibrom atosis, St urge-Weber syn drom e) Metabolic disease (e.g., ocu locerebroren al syn drom e or Low e disease, h om ocyst in u ria) In flam m ator y (e.g., r ubella, juven ile xan th ogran ulom a) Ch rom osom al delet ion /du plicat ion (e.g., Tu rn er syn drom e, t risom y 13–15)

Management Topical m edical t reat m en t is used to redu ce IOP un t il surger y can be safely p erform ed. Beta-blockers an d m iot ics rarely w ork (th ough th ey m ay h elp). Carbon ic an hydrase in h ibitors can be u sed to h elp redu ce corn eal edem a an d allow a bet ter in spect ion of an terior ch am ber st ru ct u res. Modern drugs (e.g., prostaglan din an alogues, brim on idin e tar t rate) can be t ried, alth ough th eir u se in in fan t ile glaucom a is n ot curren tly accepted. Surger y aim s at redu ct ion of com p licat ion s of IOP in crease (e.g., am blyopia, staphylom as, opt ic n er ve at rophy). We use ou r ow n su rgical tech n ique, called Sim on’s pect in otom y (surgical ablat ion of t rabecu lodysgen et ic t issue–iridopect in eal t issue by m ean s of an in st ru m en t of our ow n design ), com bin ed, or n ot , w ith a gon iotom y, t rabecu lotom y, or t rabeculectom y. Glau com atous cu pping in in fan ts h as proven to be reversible w h en su ccessful su rger y is perform ed early.

136

Color Atlas of Ophthalm ology

Conjunctivalization of the Cornea Den se leu kom as, severe scarring, an d conjun ct ivalizat ion of th e corn ea can be fou n d after bon e m arrow t ran splan t at ion , severe corn eal or in t raocu lar in fect ion s, or ocular radiat ion th erapy. A sim ilar con dit ion can be th e result of severe ch em ical burn s or au toim m un e disorders.

Presentation Severe scarring, leukom a, an d conjun ct ivalizat ion of th e globe are seen (Fig. 4.28 ).

Differential Diagnosis Ph th isis bu lbi, at rofia bu lbi, en doph th alm it is sequ elae, Steven s-Joh n son syn drom e, er yth em a m ult iform e, ch em ical bu rn s

Management Palliat ive t reat m en t to alleviate discom for t con sists of surface lubricat ion w ith n onp reser vat ive art ificial tears or oin t m en t s, pun ct al occlu sion , an d h u m idifiers or m oist u re ch am bers to decrease tear film evaporat ion . Surgical tarsorrh ap hy is used for in t ractable dr y eye. Mydriat ics an d covering th e globe w ith am n iot ic m em bran e can provide relief. Cosm et ic prosth esis after en ucleat ion is p erform ed if n eeded.

Fig. 4.28 Massive corneal neovascularization and opacification following radiotherapy.

4 External Diseases

137

Conjunctival Metaplasia in Ectropion Metaplasia of th e conju n ct iva is an un com m on disease w h ere th e conju n ct ival m u cosa ch anges in to a skin like surface due to ch ron ic exposu re of th e conju n ct iva to air an d ligh t . We also describe kerat in izat ion of th e conju n ct iva.

Presentation Conjun ct ival hyp erem ia an d lacrim at ion are seen in a pat ien t su ffering from in ten se ch ron ic lid eversion (ect rop ion ). Th ere is also foreign body sen sat ion (Fig. 4.29 ).

Differential Diagnosis Squ am ous cell carcin om a of th e conju n ct iva

Management In ten se lu bricat ion w ith eye drops, gels, an d oin t m en ts u n t il su rger y of ect ropion is perform ed.

Fig. 4.29

Conjunctival m etaplasia in ectropion.

138

Color Atlas of Ophthalm ology

Chemosis Ch em osis con sists of conjun ct ival an d subconju n ct ival edem a. In gen eral, ch em osis is a n on specific sign of eye irrit at ion .

Presentation Liquid collect ion is seen u n der th e conjun ct iva. Som et im es th e conjun ct iva becom es so sw ollen th at th e eyes can n ot close properly. Oth er sign s an d sym ptom s depen d on th e cause of ch em osis ( Table 4.2 ) ( Fig. 4.30A– C).

Differential Diagnosis Any cau se of ch em osis ( Table 4.2 )

Table 4.2

Co m m o n Causes o f Che m o sis

Severe local in am mation: Gonococcus, viral or chlamydial conjunctivitis Allergic or hypersensitivit y reaction Endophthalm itis Panophthalmia Local irritation Trauma: Ruptured globe Post trabeculectomy Postsurgical after scleral or vitreoretinal surgery Generalized edema: Nephropathy (of any origin) Heart disease (congestive) Quincke edema Urticaria Myxedema Venous congestion Cavernous sinus Orbital tumor Orbital cysts Orbital in am mation: Orbital cellulitis Endocrine thyroid disease (thyrotoxicosis) Drugs: Atropine Epinephrine Glaucoma medications Tri uridine Parasites: Trichinella spiralis dissemination

4 External Diseases

139

A

B

C Fig. 4.30 (A) Mild chem osis. (B) Massive chem osis. (C) Massive chem osis.

140

Color Atlas of Ophthalm ology

Management Treat th e un derlying con dit ion (e.g., t reat allergic conju n ct ivit is if ch em osis is due to an allergic react ion ). Ch em osis after t rabecu lectom y is h igh ly desirable an d does n ot n eed to be t reated: th e n ovice m ay be tem pted to perform early surgical revision of th e filtering bleb, w h ich is on ly n ecessar y if atalam ia w ith corn eal edem a is presen t .

Hyaline Pillat Scleral Plaque Pillat scleral plaqu e is an involut ive th in n ing of th e sclera, w ith hyalin e degen erat ion , resu lt ing from t ract ion forces of ext raocu lar m uscles, especially th e lateral rect us.

Presentation A grayish pun ch ed-ou t area is seen an terior to th e in ser t ion of th e lateral rect us m u scles. Slit-lam p exam in at ion is diagn ost ic. Th ere is n o clin ical m an ifestat ion , but th e p laqu es are visible in older pat ien ts ( Fig. 4.31 ).

Differential Diagnosis Sclerom alacia, m elan osis bulbi, scleral ect asia, staphylom as

Management No t reat m en t is n eeded.

Fig. 4.31

Hyaline scleral Pillat’s plaque.

5 Cornea W. Barry Lee and Ivan R. Schw ab

Cornea Infections Herpes Simplex Virus Herpes sim plex viru s (HSV) is a DNA viru s th at involves various stages of in fect ion , in clu ding a prim ar y system ic in fect ion , an in act ive laten t stage, an d a recu rren t in fect iou s st age follow ing react ivat ion of th e laten t viral st ate. Un ilateral in fect ion is m ost com m on , bu t bilateral in fect ion can occu r in up to 10%of cases (m ore likely in atopic in dividu als) ( Fig. 5.1A,B).

Presentation Prim ary infect ion stage : Upp er respirator y in fect ion sym ptom s w ith or w ith ou t prodrom al sign s of fever, m alaise, an d fat igu e. Associated preauricular lym ph aden opathy is n ot un com m on . Ocular involvem en t in th e prim ar y stage m ost com m on ly involves vesicles on th e periorbital skin w ith or w ith ou t a follicular bleph aroconjun ct ivit is. In fect ion m ay rarely presen t in th e conju n ct iva or corn ea w ith a follicular conjun ct ivit is an d pu n ctate kerat it is or den drit ic kerat it is. In th e laten t stage, th e virus rem ain s dorm an t in th e sen sor y n er ve ganglia.

A

Fig. 5.1 (A) Corneal dendrite highlighted by rose bengal from herpes simplex virus (HSV) intraepithelial keratitis. (B) Corneal scar and iris atrophy from HSV stromal keratitis.

B 141

142

Color Atlas of Ophthalm ology

Recurrent infect ion stage : Th e virus t ravels dow n n er ve a xon s to sen sor y n er ve en dings to in fect th e ocular su rface. Corn eal fin dings in clu de th e follow ing: Pu n ctate epith elial keratopathy Dendrit ic int raepithelial kerat it is : An u lcer of th e epith eliu m w ith th in , bran ch ing figures an d term in al bu lbs at th e en d of each bran ch w ith sw ollen borders Im m une strom al kerat it is : Grou n d-glass–like corn eal h aze, scarring, an d poten t ial th in n ing w ith late n eovascularizat ion an d lipid deposit ion . Th e epith elium is often in t act but m ay break dow n in severe cases w ith p rogression to n ecrot izing kerat it is. Necrot izing st rom al kerat it is: A corn eal ulcer w ith an epith elial defect , st rom al in filt rat ion , th in n ing, an d n ecrosis. Th ere is a h igh risk for perforat ion . Marginal ulcer: A perilim bal epith elial lesion w ith st rom al in filt rat ion , pan n us, an d th in n ing Neurot rophic ulcer: A corn eal ulcer result ing from corn eal an esth esia from viral dam age to sen sor y corn eal n er ves. Im p aired corn eal in n er vat ion leads to an u lcer w ith an epith elial defect con tain ing sm ooth , rolled borders. Endothelit is: Focal or disciform corn eal st rom al edem a w ith en doth elial kerat ic precipitate an d an terior ch am ber cellular react ion

Differential Diagnosis Differen t ial diagn osis of den drit ic epith elial kerat it is: Oth er viruses [h erpes zoster vir us, Epstein -Barr viru s, epidem ic keratocon jun ct ivit is (EKC)] Healing epith elial defect s Soft con tact len s w ear Tyrosin em ia t ype II Acantham oeba Rosacea Superficial hyper t roph ic den driform epith eliopathy (SHDE) Differen t ial diagn osis of st rom al kerat it is Herpes zoster Bacterial kerat it is Acantham oeba St aphylococcal m argin al kerat it is (m argin al u lcer cases) Rosacea Collagen vascular disease Mooren ulcer (m argin al ulcer cases)

Management Diagn osis is u sually clin ical, bu t scrapings can be perform ed w ith Giem sa stain or Papan icolaou sm ear. Polym erase ch ain react ion or an t igen detect ion can be used as a diagn ost ic tool to detect viral par t icles from scrapings or cult u re. Dendrit ic int raepithelial kerat it is : Debridem en t of den drite m ay or m ay n ot be perform ed. Begin t riflu ridin e 1%, on e drop ever y 2 h ou rs (n in e t im es daily) or vidarabin e oin t m en t five t im es daily for 10 to 14 days. Th e Herpet ic Eye Disease St udies (HEDS) foun d oral an t ivirals do n ot preven t th e subsequ en t risk of st rom al kerat it is. Con sider cyclop legic agen ts an d avoid cort icosteroids. Im m une st rom al k erat it is : An t ivirals w it h eit h er oral agen t s (acyclovir, valacyclovir, or fam ciclovir) or top ical t r iflu r id in e can be u sed in conju n ct ion w it h top ical cor t icosteroid s in cases w it h severe st rom al scar r ing an d d ecreased visu al acu it y; h ow ever, top ical an t ivirals d o n ot p en et rate in t act ep it h eliu m

5 Cornea

143

w ell; t h u s for d eep st rom al kerat it is cases, oral agen t s m ay w ork best . Th e HEDS t r ial sh ow ed t h at top ical cor t icosteroid toget h er w it h an t ivirals red u ced p ersisten ce an d p rogression of st rom al in flam m at ion an d sh or ten ed t h e d u rat ion of st rom al kerat it is. Oral an t ivirals (valacyclovir 500 m g on ce daily or acyclovir 400 m g t w ice a day) can be u sed for long-ter m su p p ression an d avoid cor n eal toxicit y. Long-ter m su p p ression is esp ecially h elp fu l for p at ien t s w it h m u lt ip le recu r ren ces of st rom al kerat it is. High er d oses sh ou ld be u sed for act ive d isease. Cor t icosteroid s sh ou ld be t ap ered to t h e low est d ose t h at con t rols in flam m at ion . Neurot rophic kerat it is: Preser vat ive-free lu brican t drop s an d oin t m en ts can be ben eficial w ith or w ith ou t blan d an t ibiot ic oin t m en t use to preven t secon dar y bacterial in fect ion (i.e., er yth rom ycin oin t m en t). Persisten t epith elial defects can be t reated w ith t arsorrh aphy or au tologous seru m drops. Th erapeut ic ban dage len s w ear can be used tem porarily w ith close obser vat ion an d con siderat ion of prophylact ic an t ibiot ic t reat m en t . Em ergen t surgical t reat m en t m ay in clude lam ellar or pen et rat ing keratop last y, cyan oacr ylate glu e patch ing, am n iot ic m em bran e graft ing, or conjun ct ival flaps in sit uat ion s w h ere visual reh abilit at ion is poor.

Herpes Zoster Virus Herpes zoster viru s (HZV) is a DNA virus also kn ow n as varicella virus. It h as a prim ar y in fect ion m an ifested as a self-lim ited in fect ion of ch ildh ood (ch icken pox) follow ed by a p eriod of laten cy in w h ich th e viru s is dorm an t in th e n eural ganglia. React ivat ion cau ses h erpes zoster (sh ingles) in ~20% of in dividuals. Herp es zoster cases in clu de 15% th at affect th e oph th alm ic n er ve dist ribut ion of th e t rigem in al n er ve (cran ial n er ve V1 division ). In fect ion alw ays involves a single derm atom e, m aking it un ilateral.

Presentation Prim ary infect ion stage : Ch icken pox. Occurs as a self-lim ited in fect ion in ch ildren . Sym ptom s in clude a m aculopapu lar rash , along w ith p rodrom al sym ptom s of fever, m alaise, an d upp er respirator y in fect ion sym ptom s. A vaccin e is n ow available for ch ildren t ypically at 12 m on th s of age to p reven t ch icken pox. A vaccin e is also available for im m un ocom p eten t in dividuals over th e age of 65. Can be life th reaten ing if a prim ar y in fect ion develops in adu lth ood or in im m u n osu ppressed pat ien t s. Latent stage : Viru s is dorm an t in th e n eural ganglia. Recurrent or react ivat ion stage : Viru s t ravels dow n a single derm atom e w ith pain , paresth esias, an d dysesth esia, follow ed by a u n ilateral m aculopapular rash along th e involved derm atom e. Ocular involvem en t occu rs in m ore th an 70% of pat ien ts w ith cran ial n er ve V1 involvem en t . Corn eal fin dings in clu de a pu n ctate or den drit ic epith elial kerat it is, n u m m u lar su bepith elial in filt rates, st rom al kerat it is, disciform kerat it is, an d gran u lom atou s kerat ic precipit ate from uveit is. Neu rot roph ic keratopathy, corn eal scarring, corn eal n eovascu larizat ion , in terst it ial kerat it is, lipid keratopathy, an d keratolysis can also occu r ( Fig. 5.2A,B,C).

Differential Diagnosis HSV, bacterial, or fungal in fect ion ; collagen vascular disease or im m u n e-related corn eal u lcers; exposure-related corn eal ulcers

144

Color Atlas of Ophthalm ology

A

B

Fig. 5.2 (A) Dendrite from primary herpes zoster virus (HZV) infection. (B) Neurotrophic ulcer following herpes zoster ophthalm icus. (C) Corneal melt after HZV necrotizing stromal keratitis.

C

Management Oral an t ivirals (acyclovir, valacyclovir, or fam ciclovir) reduce viral sh edding from lesion s an d decrease th e in ciden ce an d severit y of th e m ost com m on ocular com plicat ion s for h erpes zoster oph th alm icu s. Oral th erapy, if begu n w ith in 72 h ou rs of sym ptom on set , m ay redu ce th e in ciden ce an d durat ion of posth erpet ic n eu ralgia. Treat m en t is t ypically used for 7 to 10 days (valacyclovir, 1 g th ree t im es a day; acyclovir, 800 m g five t im es daily; or fam ciclovir, 500 m g th ree t im es a day. Topical an t ivirals are n ot effect ive in h erpes zoster. In t raven ous an t ivirals are in dicated for p at ien ts at risk for system ic dissem in at ion due to severe im m u n osu ppression .

5 Cornea

145

Topical cycloplegia an d topical cor t icosteroids can be ben eficial for severe st rom al scarring an d uveit is.

Epstein-Barr Virus Epstein -Barr viru s (EBV) is a DNA virus in th e h erpesviru s fam ily. Typically t ran sm it ted from saliva w ith a su bclin ical in fect ion m ost com m on ly occu rring in th e first decade of life. In th e laten t period th e viru s is dorm an t in B lym ph ocytes an d m u cosal epith elial cells of th e ph ar yn x. Ocular disease is un com m on bu t can occu r. In fect ion later in life causes m on on ucleosis.

Presentation Pain , redn ess, an d decreased vision along w ith an acu te follicular conju n ct ivit is can occu r w ith en largem en t of th e lacrim al glan ds an d lym p h aden opathy. Corn eal fin dings in clude epith elial den drites or pu n ctate kerat it is, corn eal n eovascularizat ion , an d in terst it ial an d/or st rom al kerat it is. A n um m ular kerat it is as seen w ith HZV or HSV can occur ( Fig. 5.3 ).

Differential Diagnosis See th e differen t ial for den drit ic kerat it is an d st rom al kerat it is listed earlier.

Management Diagn osis is depen den t on th e detect ion of EBV an t ibodies to variou s viral com pon en ts. Viral capsid an t igen s app ear in acu te in fect ion w ith im m u n oglobulin M (IgM) an t ibodies appearing first follow ed by IgG an t ibodies. An t ibodies to early an t igen s also occur in acute in fect ious st ages an d decrease to low or u n detect able after in it ial in fect ion . An t ibodies to EBV n u clear an t igen s ap pear w eeks to m on th s after in fect ion an d can be used as a m arker of previou s EBV in fect ion . Support ive t reat m en t w ith topical lu brican t drop s, gels, or oin t m en t s can im prove epith elial kerat it is. Oral an t ivirals rem ain un st udied w ith EBV st rom al kerat it is. Topical cort icosteroids m ay redu ce su bepith elial in filt rates, n u m m u lar kerat it is, an d corn eal n eovascularizat ion .

Fig. 5.3 Multiple cornea stromal opacities from Epstein-Barr virus stromal keratitis.

146

Color Atlas of Ophthalm ology

Bacterial Corneal Ulcer An in fect iou s in filt rate of th e corn ea, bacterial corn eal ulcer is also kn ow n as a bacterial corn eal u lcer. Risk factors in clude con tact len s w ear (m ost com m on cause), corn eal t rau m a, an d an altered corn eal surface such as a corn eal abrasion or pun ct ate keratopathy. Any disorder com prom ising th e corn eal epith elial in tegrit y can lead to a bacterial in fect ion . Com m on gram -posit ive bacterial cau ses in clude Staphylococcus species, St reptococcus species, an d Bacillus species (com m on after t rau m a). More com m on gram -n egat ive bacterial causes in clude Pseudom onas aeruginosa , Proteus, Enterobacter, an d Serrat ia .

Presentation Often acu te pain , redn ess, ph otoph obia, tearing, an d disch arge. A w h ite spot m ay be visible to th e pat ien t th at m ay represen t eith er th e in filt rate w ith in th e corn ea or a layered hypopyon if in th e low er port ion of th e corn ea. Bacterial kerat it is m ay presen t w ith con curren t corn eal edem a, corn eal st rom al th in n ing, n ecrosis, pu rulen t debris w ith in th e u lcer, an d w h ite blood cell in filt rat ion w ith in th e corn ea or th e an terior ch am ber ( Fig. 5.4 ).

Differential Diagnosis Viral cau se (part icu larly HSV or HZV), fu ngal cause, Acantham oeba , sh ield ulcer from vern al keratoconjun ct ivit is, im m un e-m ediated corn eal u lcer, sterile m argin al ulcer, staphylococcal m argin al kerat it is

Management Obtain a h istor y of con t act len s w ear, t raum a, or foreign body. Obtain an ocular history of system ic disease or previous eye conditions that m ay predispose to breakdow n or instability of the corneal epithelium (exposure keratopathy, recurrent erosion, dry-eye disease, corneal abrasions, eyelid abnorm alities). Obt ain a corn eal scraping for various cu lt ure m edia an d a Gram st ain or pot assium hydroxide prep on glass slides. Con sider cu lt u ring con t act len ses, cases, an d solut ion w h en available.

Fig. 5.4 Central bacterial corneal ulcer, epithelial/mucous debris, and hypopyon from Pseudom onas aeruginosa .

5 Cornea

147

Large cen t ral u lcers sh ould be placed on broad-spect rum for t ified topical an t ibiot ics u sing eith er cefazolin (50 m g/m L) or van com ycin (25 or 50 m g/m L), on e drop ever y 30 m in u tes for gram -p osit ive coverage along w ith a topical am in oglycoside for gram -n egat ive coverage. A com m on agen t is tobram ycin (14 m g/m L), on e drop ever y 30 m in utes. Sm all periph eral u lcers can be t reated w ith m on oth erapy w ith a fluoroquin olon e u sed on e drop ever y 30 m in utes. Treat m en t can be adju sted w ith close follow -up exam in at ion s an d by ut ilizat ion of bacterial cu lt u re resu lt s, in cluding iden t ificat ion an d m edicat ion sen sit ivit ies/su scept ibilit ies. Som e physician s m ay also u se a loading dose of an t ibiot ics ever y 15 m in u tes for th e first 2 h ou rs. Cycloplegia (h om at ropin e or scopolam in e, on e drop t w ice a day) is h elpfu l for pain relief from ciliar y spasm an d preven t ion of posterior syn ech iae. Con tact len s w ear sh ould be discon t in u ed w h en app licable. Hospit alizat ion sh ould be con sidered if com p lian ce is an issue w ith m edicat ion use.

Aca ntha moeba Acan th am oeba kerat it is is a corn eal in fect ion or u lcerat ion caused by a u biqu itou s protozoan foun d in fresh w ater an d soil. It exist s as a dorm an t cyst or an act ive m obile form kn ow n as a t roph ozoite. Most cases occu r in associat ion w ith p oor con tact len s hygien e, con t act len s w ear in fresh w ater sources, or clean ing con tact len ses in fresh w ater, w ell w ater, or h om em ade salin e solut ion s.

Presentation Pat ien ts experien ce acu te, severe pain , often ou t of proport ion to corn eal fin dings. Associated redn ess, ph otoph obia, tearing, an d blurred vision p rogress over th e cou rse of several w eeks. Seek a con t act len s h istor y or fresh w ater exp osure in associat ion w ith con t act len s w ear or clean ing. Fin dings begin w ith a pu n ctate epith elial kerat it is follow ed by su bepith elial h aze an d in filt rat ion . A radial perin eurit is m ay be seen along w ith pseudoden drit ic kerat it is. A late an d om in ous fin ding is st rom al ulcerat ion , n ecrosis, an d su bsequen t ring u lcerat ion w ith keratolysis ( Fig. 5.5A,B,C).

Differential Diagnosis HSV, HZA, bacterial kerat it is, fu ngal kerat it is, im m un e-related u lcer

Management Obtain a corn eal scraping or corn eal biopsy. Ut ilize calcofluor w h ite, Giem sa, H&E, or Gram st ain to iden t ify cyst s of t roph ozoites. Corn eal cult u re on n on n ut rien t agar w ith Escherichia coli overlay can detect in fect ion . Con sider cult u ring th e con t act len s or case. Con focal m icroscopy w h en available can be ben eficial for iden t ificat ion of th ese st ru ct u res in th e absen ce of severe st rom al u lcerat ion . Discon t in ue con t act len s w ear. Com bin at ion th erapy is m ost effect ive, bu t early diagn osis is th e m ost crit ical factor for su ccessful t reat m en t: Diam idin es (propam idin e, h exam idin e) Biguan ides (ch lorh exidin e diglu con ate, polyh exam ethylen e bigu an ide, alexidin e)

148

Color Atlas of Ophthalm ology

A

B

C Fig. 5.5 (A) Acantham oeba keratitis with severe punctate keratopathy and early central corneal haze. (B) Central corneal ulcer and radial perineuritis from Acantham oeba . (C) Ring ulcer and stromal necrosis from late Acantham oeba keratitis.

Am in oglycosides (parom om ycin , n eom ycin ) Im idazoles/t riazoles (clot rim azole, it racon azole, ketocon azole, m icon azole, m et ron idazole)

5 Cornea

149

Biguan ides h ave becom e first-lin e th erapy becau se of cyst icidal act ivit y, an d th ey can be u sed alon e or in com bin at ion plus a diam idin e an d/or a topical or oral am in oglycoside agen t . Treat m en t is often n eeded for m any m on th s. Cor t icosteroids sh ould be avoided if possible un less severe in flam m at ion an d pain do n ot im prove. Careful obser vat ion m ust occu r if cor t icosteroids are used becau se of poten t ial react ivat ion of t roph ozoites from dorm an t cyst s. Keratoplast y tech n iques m ay be n eeded, bu t outcom es im prove if surger y can be delayed after several m on th s of an t iam oeba t reat m en t .

Fungal Keratitis Fu n gal in fe ct ion involvin g t h e cor n ea. Risk factors m ost com m on ly in clu d e t rau m a involvin g breakd ow n of t h e cor n eal ep it h eliu m from p lan t or veget able m at te r. Trau m a from con t act le n s w ear is an ot h er r isk factor, alt h ough a relat ively n ew r isk factor from con t act le n s w ear in clu d es clean in g solu t ion s, as se en w it h t h e n ow d iscon t in u e d ReNu w it h Moist u re Loc solu t ion (for m erly m an u fact u re d by Bau sch & Lom b, Roch ester, NY), an d t h e d evelop m e n t of a Fu sariu m kerat it is ou t break w orldw id e. System ic an d top ical cor t icosteroid s are ad d it ion al r isk factors to fu n gal in fe ct ion an d can in cite d ram at ic p rogression of a fu n gal kerat it is or u lce rat ion . Com m on fu n gal agen t s m ay in clu d e Candida , Fusarium , Paecilom yces , an d Aspergillus sp e cies am on g ot h ers.

Presentation Fungal kerat it is in fect ion s are m ore in dolen t th an bacterial or viral corn eal in fect ion s un less topical cor t icosteroids h ave been u sed. Sym ptom s in clu de eye pain , redn ess, tearing, ph otoph obia, an d decreased vision . Th e h istor y can be an im p ortan t factor in assessing th e risk of fungal in fect ion s. A w h ite spot over th e corn ea is com m on ly seen from eith er th e st rom al ulcer or hypopyon . An terior uveit is, corn eal edem a, posterior corn eal plaques, an d a layered hypopyon are com m on ( Fig. 5.6 ).

Differential Diagnosis Bacterial, viral, or Acantham oeba kerat it is

Fig. 5.6 Fungal corneal ulcer and hypopyon from Candida albicans in im munosuppressed patient.

150

Color Atlas of Ophthalm ology

Management All corn eal ulcers sh ou ld be t reated as w ith bacterial kerat it is in it ially an d star ted on broad-sp ect ru m topical an t ibacterial m edicat ion s un t il cu lt ure-proven fu ngal in fect ion is iden t ified from corn eal scrapings or cu lt ure m edia. Scrapings w ith Giem sa, periodic acid–Sch iff, or Grocot t- Gom ori m eth en am in e-silver n it rate st ain s can iden t ify fu ngal elem en ts. Cult ure m edia sh ou ld in clu de eith er Sabou raud agar or brain –h ear t in fu sion broth . Nat am ycin 5%is th e t reat m en t of ch oice for filam en tous Fusarium in fect ion s, w h ereas topical m icon azole 1%is th e t reat m en t of ch oice for Paecilom yces. Am ph otericin B 0.15% or topical voricon azole is th e t reat m en t of ch oice for yeast kerat it is an d Aspergillus in fect ion . Drops are t ypically star ted ever y 1 to 2 h ou rs an d often supp lem en ted w ith oral an t ifu ngal im idazoles or voricon azole. Corn eal debridem en t m ay be n eeded in su bsequ en t visit s to im prove topical m edicat ion pen et rat ion . Surgical keratoplast y tech n iqu es m ay be n eeded in severe cases w ith keratolysis or sign ifican t n on respon sive posterior corn eal plaqu es. Treat m en t is often n eeded for several m on th s, an d cor t icosteroids sh ould be avoided du ring th is t im e. Cycloplegia m ay be ben eficial in cases w ith sign ifican t uveit is or hypopyon to preven t p osterior syn ech iae form at ion .

Corneal Inflammation and Surface Disorders Interstitial Keratitis Th is is a n on suppu rat ive in flam m at ion of th e corn eal st rom a th at t ypically in volves corn eal n eovascularizat ion an d even t ual lipid deposit ion an d scarring. Most cases resu lt from a hypersen sit ivit y react ion to in fect ious m icrobes or associated an t igen w ith in th e corn ea. Several viral, bacterial, an d h elm in th in fect ion s are associated w ith th is con dit ion .

Presentation In it ial sym ptom s in clu de pain , tearing, p h otoph obia, an d perilim bal inject ion . Kerat ic precipit ate an d an terior uveit is m ay develop. With progression , deep st rom al n eovascularizat ion develops w ith cen t ral spread. Corn eal scarring an d edem a m ay en sue. A salm on -pin k patch m ay appear w ith in th e corn ea from severe st rom al n eovascularizat ion cases. Gh ost vessels even t u ally develop w ith lipid an d scar form at ion . St rom al scarring an d opacificat ion can becom e severe. ( Fig. 5.7A,B).

Differential Diagnosis Congenital syphilis (usually bilateral and develops late in the first decade of life) Acqu ired syph ilis (rare an d usu ally u n ilateral w ith occu rren ce in adu lth ood) Viral corn eal in fect ion s (HSV, HZV, EBV, m u m ps, rubeola) Tu bercu losis (con firm w ith Tb skin test) Leprosy Chlam ydia t rachom at is (h istor y of sexu al con t act) Helm in th in fect ion s [Onchocerca volvulus (on ch ocerciasis) from foreign t ravel in en dem ic region ] Lym e disease (h istor y of t ick exposu re; ch eck Lym e t iters) Sarcoidosis (ch est x-ray, ser um calcium , lysosym e, an d angioten sin -conver t ing en zym e level) Cogan syn drom e (autoim m u n e disorder w ith in terst it ial kerat it is, ver t igo, an d h earing loss)

5 Cornea

151

A

B Fig. 5.7 (A) Interstitial keratitis from congenital syphilis with ghost vessels on retroillum ination. (B) Herpes simplex virus interstitial keratitis and corneal scarring.

Management Treat m en t begin s w ith iden t ificat ion of th e cau se. Serological test ing is ver y im por tan t in m aking a diagn osis. A screen ing test such as a Rapid Plasm a Reagin (RPR) or a t repon em e-specific test su ch as Flu orescen t Tropon em al An t ibody (FTA-ABS) or m icroh em agglut in at ion assay (MHA-TP) can detect syph ilis. System ic t reat m en t is in dicated depen ding on th e u n derlying cau se (e.g., system ic an t ibiot ics w ith syph ilis, t u bercu losis, Lym e disease). In flam m at ion in in terst it ial kerat it is cases is con t rolled w ith topical cor t icosteroids an d cycloplegic agen t s on ce an in fect ious cau se is elicited. See th e sect ion on HSV an d HZV for t reat m en t .

152

Color Atlas of Ophthalm ology

Fig. 5.8 Punctate keratitis and multiple corneal opacities t ypical of Thygeson superficial punctate keratitis.

Thygeson Superficial Punctate Keratitis Th is n on specific su perficial pun ct ate kerat it is t ypically occurs in you ng ch ildren to older adu lts. Th e cau se is u n kn ow n , alth ough specu lat ion exists as to w h eth er a virus is th e u n derlying agen t . Mult iple corn eal lesion s develop bu t t ypically respon d rapidly to cor t icosteroids, suggest ing an im m un ological basis for kerat it is.

Presentation Sym ptom s in clude recu rren t episodes of tearing, ph otoph obia, foreign body sen sat ion , an d m ild blurred vision . Conju n ct ival inject ion is usually m in im al or absen t . Corn eal fin dings in clu de m u lt iple raised epith elial lesion s th at are gray to w h ite gran ular opacit ies th at w a x an d w an e in locat ion an d n u m ber an d are u sually bilateral w ith poten t ial asym m et r y ( Fig. 5.8 ).

Differential Diagnosis Subepith elial in filt rates w ith epidem ic keratoconju n ct ivit is (EKC), EBV, staphylococcal hypersen sit ivit y, con tact len s over w ear kerat it is

Management Su ppor t ive t reat m en t w ith topical lubrican t tears or gels w ith or w ith out ban dage con tact len s w ear can be u sefu l in m ilder cases. Low -dose topical cort icosteroids can prom ote rapid resolut ion of corn eal lesion s; h ow ever, lesion s t ypically recur w ith discon t in uat ion of drops. Long-term cort icosteroid t reat m en t w ith flu orom eth olon e 0.1% or lotepredn ol 0.5% can w ork w ell at var ying dose regim en s w ith slow ly tapering doses from an in it ial dosing of fou r t im es a day. Topical cyclosp orin e an d top ical t rifluridin e h ave also been u sed w ith an ecdot al success. Th e lesion s even t u ally resolve, bu t th is m ay take m on th s to years for perm an en t resolu t ion of recu rren t episodes.

Shield Ulcer Sh ield ulcer is a n on in fect ious corn eal u lcer in th e set t ing of vern al keratoconju n ct ivit is th at is often bilateral an d season al (m ore com m on in spring). A h istor y of atopy is com m on . Th e con dit ion m ore often presen ts in m ales.

5 Cornea

153

Fig. 5.9 Peripheral vernal shield ulcers seen in vernal keratoconjunctivitis.

Presentation Presen tat ion in cludes itch ing, ph otoph obia, foreign body sen sat ion , blu rred vision , redn ess, an d m ucou s disch arge. Sh ield ulcer can be associated w ith gian t papillar y conju n ct ivit is an d lim bal follicles kn ow n as Horn er-Tran t as dots (collect ion s of degen erated eosin op h ils an d epith elial cells). A su perior pu n ctate keratopathy t yp ically develops follow ed by a breakdow n of epith elium an d even t ual oval-sh aped ulcers (sh ield ulcers) w ith u n derlying st rom al opacificat ion . Associat ion can occur w ith keratocon u s an d floppy eyelid syn drom e ( Fig. 5.9 ).

Differential Diagnosis Atopic keratoconju n ct ivit is, in fect ious u lcer, im m u n e-m ediated u lcer

Management Pat ien t s sh ou ld avoid p oten t ial allergen s an d u se cool com p resses, p reser vat ive-free lu br ican t s, top ical an d /or system ic an t ih ist am in e or m ast cell st abilizers, an d top ical or system ic cor t icosteroid s. Alter n at ive top ical or system ic im m u n osu p p ressan t agen t s su ch as cyclosp or in e A m ay p rovid e ben efit w it h close follow -u p .

Exposure Keratopathy Exposu re keratopathy is also referred to as n europaralyt ic keratopathy. Causes in clude facial n er ve palsy, severe proptosis, an d scarring of th e lids. Th e con dit ion m ay be associated w ith w eak Bell p h en om en on . It is th e resu lt of im proper w et t ing of th e ocu lar su rface by th e tear film .

154

Color Atlas of Ophthalm ology

Presentation Sym ptom s range from m ild in ferior pu n ctate epith elial ch anges to severe corn eal m elt ing w ith corn eal perforat ion .

Differential Diagnosis Neu rot roph ic keratopathy, recu rren t erosion s, in fect iou s keratopathy, dr y eye, au toim m un e diseases

Management Frequen t in st illat ion s of lubrican t s an d t aping th e lids form th e m ain stay of th e t reat m en t . Lid surgeries to correct th e lid abn orm alit ies m ay be required.

Filamentary Keratitis An ocu lar surface in flam m ator y con dit ion ch aracterized by m ucou s plaques an d st ran ds of th e corn eal surface. Filam en ts represen t st ran ds of epith elial cells attach ed to th e corn eal su rface over a core of m u cus w ith firm at t ach m en t to th e adjacen t corn eal n er ves. Com m on con dit ion s associated w ith th is fin ding in clude eyelid m alposit ion su ch as lagop h th alm ia an d poor eyelid blin k, con tact len s over w ear kerat it is, atop ic con dit ion s, an d ch ron ic ocu lar surface in flam m ator y con dit ion s such as su perior lim bic keratoconju n ct ivit is, aqueou s tear deficien cy, an d dr y-eye syn drom e.

Presentation Pat ien ts experien ce pain , foreign body sen sat ion , redn ess, dr yn ess, an d ph otop h obia. Mu cous plaqu es in st ran ds or globules are visu alized en t rapped w ith in th e corn eal epith elium . Th e filam en t s st ain w ith fluorescein dye. Conju n ct ivoch alasis, pun ct ate corn eal st ain ing bet w een filam en ts, an d a low tear m en iscus are com m on associated fin dings ( Fig. 5.10 ).

Fig. 5.10

Filamentary keratitis.

5 Cornea

155

Differential Diagnosis Recurren t erosion s, epith elial defect s

Management Debridem en t of th e filam en t s can be perform ed w ith a cot ton -t ipped applicator follow ing a topical an esth et ic. Lu brican t drops, gels, or oin t m en ts can be ben eficial from four t im es a day to h ou rly dosing. Sodium ch loride drops or acet ylcystein e drops can also be used four t im es a day. Addit ion al t reat m en t s m ay in clu de a th erapeu t ic ban dage len s an d autologou s ser um drops. Pun ct al plugging sh ou ld be avoided u n t il filam en t recu rren ce is preven ted.

Neurotrophic Keratopathy Neu rot roph ic keratopathy is a ch ron ic surface disorder resu lt ing from hypoesth esia or an esth esia of th e t rigem in al n er ve. A variet y of factors, in cluding HSV, HZV, radiat ion th erapy, previou s brain t um or resect ion , or t rau m a to th e t rigem in al n er ve, as w ell as diabetes, topical an esth et ic or preser vat ive abu se, ch em ical burn s, an d leprosy are associated w ith th is con dit ion .

Presentation Blurred vision , redn ess, an d ch ron ic epith elial defects or ulcerat ion are seen at presen tat ion , along w ith pu n ctate corn eal stain ing in th e cen t ral or in ferior corn ea w ith or w ith out associated epith elial defects. Corn eal th in n ing, filam en t ar y kerat it is, scarring, corn eal surface asperit y, an d an elevated hyperplast ic epith eliopathy can be associated fin dings ( Fig. 5.11 ).

Fig. 5.11

Neurotrophic keratopathy.

156

Color Atlas of Ophthalm ology

Differential Diagnosis Corn eal abrasion , recu rren t erosion , in fect ious u lcer, dr y-eye syn drom e, an d autoim m un e disease

Management Identify the underlying cause. Lubricant drops, gels, and ointm ents can protect the corneal surface from further breakdow n. Temporary or perm anent punctal closure can also benefit. A tarsorrhaphy is often needed to decrease the am ount of exposed surface area to the cornea. Therapeutic bandage lens wear should be used cautiously w ith close follow-up. A conjunctival or am niotic m em brane graft can be used in severe situations, w ith lam ellar or penetrating keratoplasty used in cases of severe corneal m elting or perforation. Autologous serum drops can also be a useful adjunct to treatm ent.

Recurrent Erosion Recurren t corn eal erosion (RCE) syn drom e is ch aracterized by a dist urban ce at th e level of th e corn eal epith elial basem en t m em bran e, resu lt ing in defect ive adh esion s an d recu rren t breakdow n s of th e epith elium . Com m on cau ses of recu rren t erosion s in clude epith elial basem en t m em bran e dyst rophy, corn eal inju ries, alkali burn s, foreign bodies, post in fect iou s ulcers from h erp es sim plex, Cockayn e syn drom e, Reis-Bü cklers dyst rophy, p ost vit rectom y, ph otocoagu lat ion , an d con tact len ses, am ong oth ers.

Presentation Many pat ien t s (80 to 90%) are asym ptom at ic. Som e can presen t w ith pain , blurred vision , ast igm at ism , epith elial blebs, an d foreign body sen sat ion w ith recu rren t erosion . Pat ien t s can presen t w ith a variet y of sign s such as epith elial loss, epith elial m icrocyst s, bu llae, lack of adh eren ce of sh eet s of epith elium , epith elial filam en t form at ion , corn eal abrasion , brow n ish gran u lar edem a (braw ny edem a), an d areas of h ealed epith eliu m , w h ich m ay even resem ble a den drit ic figu re, a pseu doden drite.

Differential Diagnosis Corn eal abrasion , corn eal dyst rophy, corn eal foreign bodies, dr y eye, an d in fect ive kerat it is

Management Man agem en t of RCE syn drom e is u su ally aim ed at regen erat ing or repairing th e epith elial basem en t m em bran e to restore th e adh esion bet w een th e epith elium an d th e an terior st rom a. Recurren t corn eal erosion s respon d to top ical lu bricat ion th erapy, ban dage soft con tact len ses, debridem en t , an t ibiot ic oin t m en t , an d patch ing. Su rgical opt ion s in clu de an terior st rom al pu n ct ure, excim er laser diam on d burr keratectom y, n eodym iu m :yt t riu m -alu m in u m -garn et (Nd:yAG) laser t reatm en t , an d su perficial ph ototh erapeut ic keratectom y.

5 Cornea

157

Congenital Anomalies Microcornea Th e n orm al corn eal diam eter is n orm ally 9.5–10m m at bir th an d 10–12.5m m at adulth ood.

Presentation Th e term m icrocorn ea is given to an adu lt corn ea w ith less th an 10m m diam eter. Most cases occur sporadically. Microcorn ea m ay occu r in isolat ion or par t of a gen erally sm all eye (m icroph th alm os). If it occurs as an isolated en t it y, th e n orm al len s size cau ses a disparit y w ith th e sm all corn ea resu lt ing in angle-closu re glau com a.

Differential Diagnosis Nan oph th alm os, m icrop h th alm os, sclerocorn ea

Management Treat m en t is directed at any associated ocular abn orm alit ies

Megalocornea Presentation Megalocornea is the term given to the condition in w hich the corneal diam eter exceeds 11m m at birth, or 12m m after 2 years of age. It has been associated w ith ectopia lentis, iris transillum ination, pigm ent dispersion, arcus and m ental retardation.

Differential Diagnosis Buph th alm os, an terior ch am ber dysgen esis syn drom es. As com pared to buph th alm os, th e size of th e globe an d th e in t raocular pressu re are n orm al.

Management Treat m en t is directed at any associated ocular abn orm alit ies

Anterior Embryotoxon It is a congen ital an om aly of th e corn ea w h ich is n ot visually sign ifican t .

Presentation An terior em br yotoxon refers to a congen it al broad superior lim bu s w ith an oth erw ise n orm al an terior ch am ber. It is also u sed to describe a congen it al arcu s, arcu s juven ilis, sim ilar to th e arcus sen ilis th at occu rs in old in dividu als.

Differential Diagnosis Posterior em br yotoxon , Arcus sen ilis, Axen feld Reiger syn drom e.

Management Obser vat ion an d follow -u p.

158

Color Atlas of Ophthalm ology

Posterior Embryotoxon It represents a thickened and anteriorly displaced Schwalbe’s line that is easily visible.

Presentation Posterior em br yotoxon is th e m ost com m on an om aly of th e corn ea. It m ay be seen as a w h ite ring n ear th e lim bus on slit-lam p exam in at ion an d as a prom in en t , an teriorly displaced Sch w albe’s lin e on gon ioscopy.

Differential Diagnosis An terior em br yotoxon , Arcus sen ilis, Axen feld Reiger syn drom e.

Management Obser vat ion an d follow -u p.

Sclerocornea It is a congen ital an om aly of th e corn ea resu lt ing in decreased vision .

Presentation Sclerocorn ea refers to a sclera-like appearan ce of th e corn ea, w h ich m ay be p eriph eral or involve th e w h ole corn ea. It is associated w ith corn eal flat ten ing an d oth er an om alies of an terior ch am ber developm en t .

Differential Diagnosis Microph th alm os, bu ph th alm os, an d oth er cau ses of opaque corn ea.

Management Visual progn osis in severe cases is ver y p oor.

Dystrophies Anterior Corneal Dystrophies Epithelial Basement Membrane Dystrophy (EBMD) Also kn ow n as Cogan m icrocyst ic edem a or m ap-dot-fingerprin t corn eal dyst rophy, th is is perh ap s th e m ost com m on of all corn eal dyst roph ies. Bilateral (bu t m ay be asym m et rical) w ith autosom al dom in an t in h erit an ce, bu t m ost cases are sporadic. Th e con dit ion resu lts from abn orm al epith elial t urn over an d redun dan t basem en t m em bran e.

Presentation Pat ien ts m ay rem ain asym ptom at ic or m ay develop decreased vision , foreign body sen sat ion , ph otop h obia, tearing, an d poten t ially pain fu l corn eal erosion s. Slit-lam p exam in at ion of EBMD sh ow s gray-w h ite su bepith elial patch es (m aps), gray-w h ite

5 Cornea

159

A

B Fig. 5.12 (A) Fingerprint pat tern seen with epithelial basement m em brane dystrophy (EBMD). (B) Map pat tern seen with EBMD.

parallel lin es (fingerprin ts), or su bepith elial m icrocysts (dots). Th e fin dings are best seen on re t roillu m in at ion ( Fig. 5.12A,B).

Differential Diagnosis Trau m at ic corn eal erosion , Meesm an n dyst rophy, early Reis-Bü cklers dyst rophy, corn eal in t raepith elial n eop lasia (CIN)

Management No t reat m en t is required for asym ptom at ic cases. For sym ptom at ic cases, sodiu m ch loride drops or oin t m en t can be ben eficial. Pat ien ts w ith con curren t dr y-eye disease sh ould use addit ion al preser vat ive-free ar t ificial tears or gels. Th erapeu t ic ban dage len ses can provide tem porar y relief. Surgical opt ion s in clu de an terior st rom al pun ct u re, Na:Yag st rom al pu n ct u re, epith elial debridem en t , ph ototh erapeut ic keratectom y, or diam on d bu rr keratectom y.

160

Color Atlas of Ophthalm ology

Fig. 5.13

Meesmann dystrophy.

Meesmann Dystrophy Meesm an n dyst rophy is a rare bilateral au tosom al dom in an t con dit ion occu rring early in life th at resu lts from a th icken ed basem en t m em bran e w ith a fibrogran u lar “pecu liar su bstan ce” (possibly hyalin e) w ith in th e ep ith elial cells.

Presentation Pat ien ts m ay presen t w ith pain , blu rred vision , ph otoph obia, tearing, an d redn ess from recu rren t erosion s. Tiny epith elial vesicles are seen from lim bus to lim bu s, m ost clearly on ret roillu m in at ion ( Fig. 5.13 ).

Differential Diagnosis Cyst in osis, corn eal gu t tae, epith elial basem en t m em bran e dyst rop hy (EBMD), t raum at ic recu rren t erosion

Management No t reat m en t is requ ired u n less recu rren t erosion s develop. Sim ilar t reat m en ts m ay be perform ed as w ith EBMD, alth ough a deep an terior lam ellar keratoplast y can also be useful in cases recalcit ran t to recurren t erosion t reat m en t opt ion s.

Reis-Bücklers Dystrophy Reis-Bü cklers dyst rophy is a progressive bilateral autosom al dom in an t dyst rophy th at develops early in life. Gen et ic lin kage occurs from a m u tat ion of th e BIGH3 gen e on ch rom osom e 5q31.

Presentation Pat ien ts presen t w ith recurren t erosion sym ptom s of blurred vision , pain , ph otoph obia, tearing, an d redn ess. Exam in at ion reveals a superficial gray-w h ite ret icular h aze m ore con cen t rated in th e cen t ral corn ea. Sign ifican t scarring can lead to Salzm an n n odules an d an irregu lar corn eal su rface ( Fig. 5.14 ).

5 Cornea

Fig. 5.14

161

Reis-Bücklers dystrophy.

Differential Diagnosis Meesm an n dyst rophy, EBMD, gran u lar dyst rophy

Management In it ial t reat m en t is directed at recu rren t corn eal erosion t reat m en t as w ith EBMD. Surgical p rocedu res m ay in clude lam ellar keratoplast y, pen et rat ing keratoplast y, or ph ototh erapeut ic keratectom y. Recu rren ce is com m on w ith p en et rat ing keratoplast y.

Stromal Corneal Dystrophies Granular Dystrophy Gran u lar dyst rophy is a com m on bilateral au tosom al dom in an t st rom al dyst rophy lin ked to a m ut at ion in th e BIGH3 gen e on ch rom osom e 5q31. St rom al deposits con sist of hyalin e, w h ich can be h igh ligh ted w ith Masson t rich rom e stain on corn eal h istopath ology. Several form s of th is dyst rophy exist .

Presentation Sym ptom s t ypically occur as a result of recurren t erosion s an d m ay in clude blu rred vision , pain , foreign body sen sat ion , ph otoph obia, an d tearing. Exam in at ion sh ow s bread crum b –like op acit ies, t yp ically in th e cen t ral por t ion of th e an terior st rom a, w ith sparing of th e lim bu s ( Fig. 5.15 ).

Differential Diagnosis Avellin o dyst rophy, m acular dyst rophy

Management Treat m en t is directed at recurren t erosion s. Ph ototh erapeu t ic keratectom y, lam ellar keratoplast y, an d pen et rat ing keratoplast y can be u sed for t reat m en t . Keratoplast y h as a good progn osis in cases w ith poor vision , bu t recu rren ce is possible.

162

Color Atlas of Ophthalm ology

Fig. 5.15

Granular corneal dystrophy.

Lattice Dystrophy Th is is a bilateral autosom al dom in an t st rom al dyst rophy lin ked to a m u t at ion in th e BIGH3 gen e on ch rom osom e 5q31. St rom al deposits con sist of am yloid, w h ich can be h igh ligh ted on corn eal h istopath ology w ith Congo-red stain . Several t ypes of th is dyst rophy exist , in clu ding an au tosom al recessive form .

Presentation Pat ien ts are asym ptom at ic un less recurren t erosion s develop . Decreased vision can resu lt from deposit s. Exam in at ion reveals cen t ral refract ile bran ch ing lin es w ith in th e an terior corn eal st rom a th at are best seen on ret roillu m in at ion . Th e lim bus is spared ( Fig. 5.16A,B).

B

A Fig. 5.16 (A) Lat tice corneal dystrophy. (B) Lat tice corneal dystrophy on retroillumination. (Both im ages courtesy of Mark J. Mannis, MD)

5 Cornea

163

Differential Diagnosis En larged corn eal n er ves, gh ost vessels of in terst it ial kerat it is

Management No m an agem en t is required if th e p at ien t is asym ptom at ic. Treat recurren t erosion s as described in sect ion on Recu rren t Erosion s (p . 156). Recurren ce is com m on despite corn eal t ran sp lan tat ion , bu t th e procedure can be ben eficial in som e pat ien t s w ith a good long-term progn osis.

Macular Dystrophy Macu lar dyst rophy is a rare bilateral au tosom al recessive corn eal dyst rophy w ith m u copolysacch aride deposit ion in any or all p or t ion s of th e st rom a. Alcian blue stain can detect th e dep osits on corn eal h istopath ology.

Presentation Th ere m ay be recu rren t erosion sym ptom s. Decreased vision can occu r early in th e cou rse. Exam in at ion sh ow s gray-w h ite opacit ies w ith p oor m argin s, separated by in ter ven ing h aze or clou din ess w ith in th e st rom a. Lesion s often coalesce w ith t im e an d often involve th e en t ire corn ea, lim bu s to lim bu s (Fig. 5.17 ).

Differential Diagnosis At ypical lat t ice or gran u lar dyst rophy

Management Recurren t erosion sym ptom s can be t reated. Pen et rat ing keratoplast y or deep lam ellar keratoplast y is often th e on ly surgical opt ion because of th e depth of st rom al opacit ies. Larger graft sizes m ay lim it recu rren ces.

Fig. 5.17

Early macular corneal dystrophy.

164

Color Atlas of Ophthalm ology

Fig. 5.18 Central crystalline dystrophy of Schnyder. (Courtesy of Mark J. Mannis, MD)

Schnyder Corneal Dystrophy Th is con dit ion is a bilateral, slow ly progressive autosom al dom in an t st rom al dyst rop hy, w h ich is often referred to as Sch nyder cr ystallin e corn eal dyst rophy. It can be detected as early as 1 year of age w ith accum ulat ion of ch olesterol an d ph osph olipids w ith in th e st rom a secon dar y to abn orm al corn eal lipid m et abolism .

Presentation Decreased vision is a presen t ing sym ptom . Exam in at ion sh ow s cen t ral corn eal opacificat ion w ith sparing of th e lim bu s. Th e epith elium rem ain s in tact w ith associated corn eal arcus developm en t in th e secon d or th ird decade of life. Su bepith elial cr ystals m ay be presen t along w ith m idperiph eral corn eal opacificat ion in som e cases ( Fig. 5.18 ).

Differential Diagnosis Oth er st rom al dyst roph ies, cen t ral corn eal scars, hyp erlip oprotein em ia

Management Ch eck th e pat ien t’s fast ing lip id p rofile because 50% h ave elevated ch olesterol. If vision declin e progresses, pen et rat ing keratoplast y is w arran ted, w ith a p oten t ial for recurren ce.

Fleck Dystrophy Fleck dyst rophy is an un com m on n onprogressive au tosom al dom in an t con dit ion th at begin s early in life. Affected keratocytes con tain t w o abn orm al subst an ces: glycosam in oglycan an d lipids.

5 Cornea

165

Presentation Discrete, flat , gray-w h ite, dan dru ff-like opacit ies ap pear th rough out th e st rom a. Sym ptom s are m in im al, an d vision is n ot affected. Th e con dit ion m ay be associated w ith decreased corn eal sen sat ion , lim bal derm oid, keratocon u s, atopy, or pseudoxan th om a elast icum .

Differential Diagnosis Posterior polym orph ou s dyst rophy, pre–Descem et dyst rophy, ich thyosis

Management No t reat m en t is requ ired.

Central Cloudy Dystrophy of François Th is is a bilateral sym m et rical st rom al dyst rophy th at is slow ly progressive an d au tosom al dom in an t . It can be associated w ith m egalocorn ea.

Presentation Patients are asym ptom atic. Exam ination shows central corneal opacities w ith polygonal gray areas separated by intervening clear zones resem bling cracks. Haze extends into the anterior corneal strom a unlike w ith posterior crocodile shagreen (Fig. 5.19 ).

Differential Diagnosis Posterior crocodile sh agreen , oth er st rom al dyst roph ies

Management No t reat m en t is n ecessar y because vision is usu ally n ot redu ced.

Pre–Descemet Dystrophy Th is is an acqu ired con dit ion .

Fig. 5.19

Central cloudy dystrophy of François.

166

Color Atlas of Ophthalm ology

Presentation Pat ien ts are gen erally asym ptom at ic bu t visu al acu it y can be affected. Sm all lin ear or pu n ctate gray-w h ite flecks are appreciated in th e deep st rom a. Th e con dit ion can be associated w ith keratocon us, posterior polym orph ous dyst rophy, an d epith elial basem en t m em bran e dyst rophy.

Differential Diagnosis Fleck dyst rophy, posterior polym orph ou s dyst rophy, ich thyosis

Management No t reat m en t is requ ired .

Posterior Amorphous Stromal Dystrophy Th is is a rare, bilateral, slow ly progressive, au tosom al dom in an t con dit ion presen ting in ch ildh ood.

Presentation Pat ien ts p resen t w ith diffu se, gray-w h ite lesion s in th e posterior st rom a, usu ally involving th e cen t ral area, bu t m ay involve up to th e lim bu s. Corn eal th in n ing w ith flat topography an d result ing hyperopia, cen t ral corn eal th in n ing, an d periph eral iris processes m ay be seen .

Management Rigid gas perm eable con t act len ses are u sed to correct th e ast igm at ism . Visu al acuit y is rarely affected.

Congenital Hereditary Stromal Dystrophy Th is is an au tosom al dom in an t , bilateral, sym m et rical, n onprogressive st rom al dyst rop hy seen in th e n ew born .

Presentation Cen t ral corn eal clou ding is seen in th e n ew born w ith sparing of th e periph eral st rom a. In fan t s develop am blyopia w ith nystagm u s an d squin t .

Differential Diagnosis Congen it al h ereditar y en doth elial dyst rophy, congen it al glau com a, m ucopolysacch aridosis, bir th t rau m a, posterior polym orph ous corn eal dyst rophy

Management Pen et rat ing keratoplast y is th e t reat m en t of ch oice.

5 Cornea

167

Posterior Corneal Dystrophies Corneal Guttae Th is is a focal accum u lat ion of collagen on th e posterior surface of th e Descem et m em bran e associated w ith en doth elial dysfun ct ion , corn eal clou ding, an d poten t ial visu al loss.

Presentation Th e lesion appears as a w art or excrescen ces in relat ion w ith th e en doth elial cells. Th e lesion s, called Hassall–Hen le bodies, ap pear at th e periph er y of th e corn ea an d are associated w ith aging. A “beaten m et al” appearan ce, visible during th e specu lar reflect ion , is associated w ith m elan in deposits.

Differential Diagnosis Fu ch s en doth elial dyst rophy, Hassall-Hen le bodies

Management Con sider specu lar m icroscopy. Han dling of th e corn ea m u st be gen tle during in t raocu lar surgeries becau se th is m ay h asten en doth elial com prom ise.

Fuchs Endothelial Dystrophy Th is is a bilateral en doth elial dyst rophy result ing in progressive dam age to th e en doth elium . It is rarely sym ptom at ic before 50 years of age. Reduct ion in th e n u m ber an d fun ct ion of sodium an d pot assiu m aden osin e t riph osph at ase pum ps in th e en doth eliu m occu r, creat ing progressive corn eal edem a an d gut tae. Th e con dit ion is autosom al dom in an t or sporadic.

Presentation Sym ptom s in clude decreased vision (w orse in th e m orn ing), foreign body sen sat ion , ph otoph obia, tearing, an d pain . Exam in at ion resu lts can var y from m ild guttae to severe m icrocyst ic an d st rom al edem a w ith bu llae form at ion . Th e Descem et m em bran e becom es th icken ed w ith an in crease in corn eal th ickn ess as fluid reten t ion in creases w ith in th e corn eal st rom a. Su bepith elial fibrosis an d scarring m ay occur in later st ages ( Fig. 5.20A,B,C).

Differential Diagnosis Hassall-Hen le bodies, pseu doph akic or aph akic bullous keratopathy, Ch an dler syn drom e, h erpes sim plex kerat it is

Management Begin w ith su ppor t ive th erapy, in cluding art ificial tears an d in part icu lar sodium ch loride drops or oin t m en t . Hair dr yers can in crease fluid evaporat ion from th e corn ea if used carefu lly. Defin it ive surgical t reat m en t in clu des eith er a pen et rating keratoplast y or en doth elial keratoplast y (becom ing a preferred tech n iqu e w ith Fu ch s dyst rophy). Graft su r vival progn osis is good.

168

Color Atlas of Ophthalm ology

A

B

C Fig. 5.20 (A) Gut tae seen on retroillumination in Fuchs endothelial dystrophy. (B) Microcystic and strom al edema seen in Fuchs endothelial dystrophy. (C) Fuchs endothelial dystrophy with subepithelial bullae.

5 Cornea

169

Posterior Polymorphous Dystrophy Th is t ype of dyst rophy is a bilateral en doth elial dyst rophy w ith gradu al progression . It m ay be au tosom al dom in an t or recessive an d h as been m apped to ch rom osom e 20q11.

Presentation Decreased vision an d pain m ay develop, bu t pat ien t s are often asym ptom at ic. Exam in at ion of th e posterior corn ea reveals grou ped vesicles, geograph ic gray lesion s, an d/or broad ban ds w ith scallop ed edges. Associated fin dings can in clu de corn eal edem a, h aze, corectopia, an d iridocorn eal adh esion s as w ell as glau com a. In fan t s m ay p resen t w ith cloudy corn eas ( Fig. 5.21A,B).

Differential Diagnosis Iridio-corneal endothelial syndrom e, congenital hereditary endothelial dystrophy, aphakic or pseudophakic bullous keratopathy, Fuchs dystrophy

Management Pen et rat ing keratop last y is required for cases w ith sym ptom at ic decreased vision . Obser ve carefu lly for con curren t open -angle glau com a.

A

Fig. 5.21 (A) Edema and haze seen in posterior polymorphous dystrophy. (B) Broad bands seen on slit illumination in posterior polymorphous dystrophy.

B

170

Color Atlas of Ophthalm ology

Fig. 5.22 Diffuse epithelial and stromal edem a in child with congenital hereditary endothelial dystrophy.

Congenital Hereditary Endothelial Dystrophy Th is is a bilateral corn eal dyst rophy w ith au tosom al dom in an t an d recessive form s. It cau ses en doth elial dysfu n ct ion .

Presentation Sym ptom s in clu de bilateral clou dy corn eas an d diffuse epith elial an d st rom al edem a w ith a n orm al in t raocular p ressure an d th icken ed Descem et m em bran e. Th e recessive form p resen t s w ith bilateral corn eal edem a at birth w ith nystagm u s. Th is con dit ion appears w ith out ph otoph obia an d tearing, an d th ere is a lack of dyst rophy progression . Th e dom in an t form is eviden t by age 2 w ith gradual progression of th e dyst rophy. Pain an d tearing are presen t in th e absen ce of ph otoph obia ( Fig. 5.22 ).

Differential Diagnosis Congen ital glau com a, m u copolysacch aridosis, congen it al h eredit ar y st rom al dyst rop hy, posterior polym orph ou s corn eal dyst rophy, birth t raum a

Management Topical sodium ch loride drops or oin t m en t s are h elpfu l. Pen et rat ing keratoplast y is used in cases w ith corn eal decom pen sat ion . Th ere is a con cern for am blyopia in in fan t s.

Ectatic Disorders Keratoconus Keratocon u s is a com m on bilateral, but often asym m et rical, disorder of corn eal th in n ing in w h ich th e cen t ral or in ferior paracen t ral corn ea un dergoes progressive th in n ing to t ake on th e sh ape of a con e. It begin s in adolescen ce an d progresses slow ly w ith st abilit y in late adu lth ood. A gen et ic predisp osit ion is suspected w ith gen e lin kages in cer t ain fam ilies, bu t eye ru bbing an d eye t rau m a rem ain a sig-

5 Cornea

171

Fig. 5.23 Central corneal thinning and protrusion with keratoconus.

n ifican t factors. Th ere is a h igh associat ion w ith con dit ion s su ch as atopy, Dow n syn drom e, Marfan syn drom e, floppy eyelid syn drom e, an d Leber congen it al h ereditar y opt ic n eu ropathy. Acu te hydrops can occur.

Presentation Vision is decreased as a result of progressive m yopic ast igm at ism from alterat ion of th e sh ape of th e corn ea. Keratocon us t ypically develops in th e oblique axis an d is associated w ith irregular ast igm at ism . Exam in at ion sh ow s scissoring of th e red reflex an d apical corn eal th in n ing. Associated fin dings m ay in clude breaks in th e Descem et m em bran e, apical corn eal scarring, st ress lin es in th e st rom a (Vogt st riae), an d iron lin e form at ion (Fleisch er ring) (Fig. 5.23 ).

Differential Diagnosis Con tact len s scarring, pellu cid, keratoglobus

Management Corn eal topography, keratom et r y, or ph otokeratoscopy can im prove accu racy of diagn osis by dem on st rat ing in ferior corn eal steepen ing. Scissoring reflex on ret in oscopy is path ogn om on ic. Treat w ith spectacle correct ion of m yopic ast igm at ism or rigid gas perm eable or hybrid con tact len ses w h en spectacle correct ion is poor ow ing to irregu lar ast igm at ism . In tacs corn eal im plan t s (Addit ion Tech n ology, In c., Sun nyvale, CA) can h elp in som e cases of con tact len s in toleran ce an d poten t ially provide in creased corn eal stabilit y w ith or w ith ou t corn eal collagen cross-lin king. Pen et rat ing or deep an terior lam ellar keratoplast y provides th e u lt im ate cure.

Keratoglobus Keratoglobus is a rare n on in flam m ator y con dit ion th at presen t s at bir th as opposed to oth er corn eal ect at ic disorders w ith m idperiph eral corn eal th in n ing. Acute hydrops can occu r.

172

Color Atlas of Ophthalm ology

Fig. 5.24

Corneal hydrops in keratoglobus.

Presentation Exam in at ion sh ow s a globular sh ape of both corn eas w ith large corn eal diam eters. Associated gen eralized th in n ing occu rs, m ore con cen t rated in th e m idperiph er y. Th e an terior ch am ber is ver y deep ( Fig. 5.24 ).

Differential Diagnosis Keratocon u s

Management A lam ellar or pen et rat ing keratoplast y is u su ally required. Progn osis is gu arded becau se of th e n ecessar y large graft diam eter.

Pellucid Marginal Degeneration Pellu cid m argin al degen erat ion is a n on h eredit ar y, bilateral, periph eral corn eal th in n ing disorder th at m ost often involves th e in ferior corn eal periph er y but m ay occur in th e superior periph eral corn ea. It presen t s in early adulth ood, t ypically bet w een 20 an d 40 years of age.

Presentation Pat ien ts experien ce blurred vision as a resu lt of progressive, again st th e rule ast igm at ism or oblique ast igm at ism . Exam in at ion dem on st rates corn eal prot rusion above th e area of th in n ing. St rom al scarring can occur w ith in th e th in n ed areas, an d rarely p erforat ion m ay develop ( Fig. 5.25 ).

Differential Diagnosis Keratocon u s, Terrien m argin al degen erat ion , collagen vascular disease

5 Cornea

173

Fig. 5.25 Inferior corneal thinning with pellucid marginal degeneration.

Management Rigid gas perm eable len ses are recom m en ded. Spect acle correct ion is often n ot h elpfu l. Crescen t ic lam ellar or pen et rat ing keratoplast y can occur in severe cases of vision loss or corn eal ectasia.

Corneal Degenerations and Deposits Arcus Senilis Arcu s sen ilis is an accu m u lat ion of ext racellular lipid in th e periph eral corn eal st rom a. It can be associated w ith hyerlipidem ic states.

Presentation Pat ien t s are asym ptom at ic, bu t th e con dit ion can be visible cosm et ically as a ring of periph eral opacificat ion th at begin s in th e su perior an d in ferior periph eral corn eal st rom a w ith a th in , clear zon e bet w een th e lim bu s. It t ypically presen ts in a 360-degree ring an d can be associated w ith correspon ding carot id ar ter y disease ( Fig. 5.26 ).

Differential Diagnosis Hyp erlipoprotein em ia, pseu dogeron toxin , arcu s juven ilis

Management No ocu lar t reat m en t is n ecessar y. Con sider obt ain ing a lipid profile in you ng adu lt s.

174

Color Atlas of Ophthalm ology

Fig. 5.26

Peripheral lipid deposition with arcus senilis.

Limbal Girdle of Vogt An elastot ic degen erat ion of collagen develops in th e periph eral corn ea an d m ay con tain par t icles of calciu m .

Presentation An asym ptom at ic, periph eral corn eal opacit y t yp ically begin s at 3 an d 9 o’clock along th e lim bu s. Th ere m ay be a clear lucid in ter val bet w een th e opacit y an d lim bus, depen ding on w h ich t ype of lim bal girdle develops. Ch alklike opacificat ion is com m on ( Fig. 5.27 ).

Management No t reat m en t is n eeded becau se visual fun ct ion is n ot affected.

Fig. 5.27

Vogt limbal girdle.

5 Cornea

Fig. 5.28

175

Dense primary lipid keratopathy.

Primary Lipid Keratopathy A yellow - or cream -colored lipid deposit ion com posed of ch olesterol develops in th e su perficial or deep corn eal st rom a. Un like secon dar y lipid keratopathy w h ere an an teceden t corn eal in flam m ator y con dit ion such as h erpes sim plex, h erpes zoster, or t rach om a is presen t , prim ar y cases lack corn eal n eovascu larizat ion an d previou s in fect ion or in flam m at ion .

Presentation Pat ien ts are asym ptom at ic un less decreased vision develops from obscu rat ion of th e visual axis. Cream or yellow deposits develop in th e paracen t ral or cen t ral corn ea ( Fig. 5.28 ).

Differential Diagnosis Crocodile sh agreen , cen t ral clou dy dyst rophy of Fran çois, Sch nyder corn eal dyst rophy, hyperlipoprotein em ia

Management No t reat m en t is n ecessar y un less th e visual a xis causes decreased vision . A deep an terior lam ellar keratoplast y or pen et rat ing keratoplast y m ay be n eeded depen ding on th e depth of lipid deposit ion in th e corn eal st rom a.

Corneal Keloid Corn eal opacit ies are com posed of irregu lar pat tern s of collagen ase bu n dles. Th ey can be associated w ith ocu locerebroren al (Low e) syn drom e, an autosom al dom in an t con dit ion in ch ildren , in w h ich th ey are bilateral. Adu lt cases t ypically occu r after previou s corn eal perforat ion or corn eal t rau m a.

176

Color Atlas of Ophthalm ology

Fig. 5.29

Corneal keloid.

Presentation Pat ien ts presen t w ith decreased visual acu it y an d foreign body sen sat ion . A w h ite, elevated, corn eal lesion can progressively en large an d cover th e visual axis of th e corn ea ( Fig. 5.29 ).

Differential Diagnosis Scar from in fect ious corn eal ulcer; derm oid

Management If visu al acu it y is com prom ised, a lam ellar keratectom y or pen et rat ing keratop last y m ay be n eeded for vision reh abilit at ion . Bilateral cases in ch ildren sh ou ld receive a system ic evaluat ion for Low e syn drom e w ith referral to a pediat rician .

Calcific Band Keratopathy Th is con dit ion con sist s of calcific degen erat ion of th e su perficial corn ea involving th e Bow m an layer. System ic cau ses m ay in clu de ren al disease, hypercalcem ic states, gou t , sarcoidosis, an d elevated p h osph oru s levels. Con dit ion s of ch ron ic ocu lar disease such as glau com a, kerat it is, uveit is, an d th e p resen ce of in t raocular silicon e oil m ay also cau se th is con dit ion . A h eredit ar y form is presen t . Ch ron ic m ercur y exposure m ay be an oth er cause.

Presentation Fin e gray-w h ite du stlike opacit ies develop in th e Bow m an layer in th e periph eral corn ea, t ypically at 3 an d 9 o’clock. A lu cid in ter val separates th e opacit ies from th e adjacen t lim bu s. Th e dep osits t ypically coalesce to form a h orizon tal ban d of corn eal op acificat ion in th e in terpalp ebral zon e of th e corn ea ( Fig. 5.30 ).

Management Determ in e th e un derlying cause. Any un derlying elect rolyte or ren al disease m u st be corrected. If pain or foreign body sen sat ion is sign ifican t , a lam ellar keratectom y w ith adju n ct disodium ethylen ediam in etet raacet ic acid (EDTA) (1, 1.5, or 2%) can be u sed to rem ove calcific deposit s. If un derlying con dit ion s are n ot corrected,

5 Cornea

Fig. 5.30

177

Band keratopathy.

th e calcific deposits w ill recur. Ph ototh erapeu t ic keratectom y m ay also be u sed to rem ove th e dep osits.

Salzmann Nodular Degeneration A n on in flam m ator y corn eal degen erat ion develops eith er from idiopath ic cau ses or as a sequela of prior ch ron ic kerat it is. Som e causes m ay in clu de ph lycten ulosis, bleph arit is, t rach om a, con tact len s kerat it is, or in terst it ial kerat it is. It m ay also be associated w ith recurren t corn eal erosion s an d ep ith elial basem en t m em bran e dyst rop hy.

Presentation Pat ien ts eith er m ay be asym ptom at ic or m ay presen t w ith foreign body sen sat ion , tearing, an d decreased vision . Elevated gray-w h ite or blu ish n odules develop rep resen t ing fibrillar m aterial th at h as replaced th e Bow m an layer. Th e n odules often develop in a circu lar fash ion in th e cen t ral or paracen t ral corn ea, alth ough th ey m ay also be presen t ing adjacen t to th e lim bus ( Fig. 5.31 ).

Differential Diagnosis Ph lycten u losis, keloid, staphylococcal m argin al u lcer, corn eal scar from in fect ious kerat it is

Fig. 5.31 Salzmann’s nodular degeneration.

178

Color Atlas of Ophthalm ology

Management Support ive care w ith art ificial tears, gels, or oin t m en t can h elp. If recurren t erosion s are presen t , a tem porar y ban dage len s or topical osm ot ic agen t su ch as sodiu m ch loride drops m ay im prove th e sym ptom s. If visual acu it y is decreased or sign ifican t discom fort develops, a su perficial keratectom y can be perform ed to rem ove th e n odules. Recurren ce m ay develop despite debridem en t . Pen et rat ing procedu res are n ot com m on ly n eeded for th is con dit ion .

Spheroidal Degeneration Sph eroidal degen erat ion is also referred to as corn eal elastosis, Labrador keratopathy, clim at ic droplet keratopathy, an d Biet t i n odu lar dyst rop hy. It is a bilateral con dit ion seen m ain ly in m en , kn ow n to h ave an associat ion w ith ult raviolet exposure.

Presentation Th e pat ien t presen ts w ith irrit at ion an d foreign body sen sat ion . In ext rem e cases visu al im pairm en t m ay occur. Clin ical exam in at ion reveals sm all am ber-colored gran ules in th e su perficial st rom a of th e periph eral in terpalpebral corn ea. In creased opacificat ion , coalescen ce, an d cen t ral spread occur in th e late presen t at ion s.

Management Lam ellar keratoplast y an d pen et rat ing keratoplast y are opt ion s w h en th ere is visu al im pairm en t .

Polymorphic Amyloid Degeneration Bilateral corn eal opacit ies con tain ing am yloid develop in late adu lth ood w ith in th e corn eal st rom a.

Presentation Pat ien ts are often asym ptom at ic w ith n o vision reduct ion . Gray to w h ite polym orph ic an d/or filam en tous flecks appear in th e m id- to deep st rom a w ith in th e cen t ral or paracen t ral corn ea. Th e dep osits appear refract ile bu t are t ran slu cen t on ret roillu m in at ion . Th e in ter ven ing st rom a is clear ( Fig. 5.32 ).

Differential Diagnosis Corn eal gu t t ae, corn eal farin at a, lat t ice dyst rophy

Management Visual acu it y is t ypically n ot involved so n o t reat m en t is n ecessar y.

5 Cornea

179

Fig. 5.32 Polym orphic amyloid deposits seen on retroillumination.

Iron Lines Iron deposit ion occu rs in th e epith eliu m as a result of tear pooling abn orm alit ies from asperit y of th e corn eal su rface ( Fig. 5.33 ).

Presentation Hudson-Stähli line : A h orizon t al lin e at th e ju n ct ion of th e low er th ird an d upper t w o th irds of th e corn ea Ferry line : A lin e an terior to th e edge of th e conju n ct iva from a filtering bleb Stocker line : A lin e an terior to th e h ead of a pter ygiu m Fleischer ring: A con t in uous circular or ellipt ical pat tern surroun ding th e area of corn eal steepen ing in keratocon us Mannis line : A con t in uous 360-degree ring ju st an terior to th e sut u res of a corn eal graft

Fig. 5.33 Fleischer ring from keratoconus outlined with cobalt blue light filter.

180

Color Atlas of Ophthalm ology

Management No m an agem en t is n ecessar y.

Kayser-Fleischer Ring Th is is a rare au tosom al recessive disorder in w h ich copp er deposit ion occu rs th rough out th e body, in clu ding th e Descem et m em bran e. It is also kn ow n as h epatolen t icular degen erat ion .

Presentation Corn eal fin dings are asym ptom at ic, but exam in at ion by gon ioscopy or slit lam p reveals a Kayser-Fleisch er ring (a golden brow n to green 360-degree ring pat tern at th e lim bu s w ith in th e Descem et m em bran e).

Management System ic pen icillam in e causes th e ring to disappear gradu ally. Liver t ran splan tat ion is often requ ired.

Corneal Verticillata Lysosom al or lipid deposits occur in th e basal epith elial layer of th e corn ea in associat ion w ith several system ic m edicat ion s. Th is can also occu r in Fabr y disease.

Presentation Pat ien ts are u sually asym ptom at ic but m ay experien ce blurred vision .

Differential Diagnosis Fabr y disease ( Fig. 5.34A), am iodaron e ( Fig. 5.34B), ch loroquin e, hydroxych loroqu in e, ph en oth iazin es, in dom eth acin , n aproxen , st riate m elan okeratosis ( Fig. 5.34C)

Management Obser ve or perform epith elial debridem en t if vision is decreased. If th e con dit ion is severe, con sider discon t in u at ion of system ic m edicat ion un less it is essen t ial for t reat m en t of system ic disease.

Peripheral Thinning Mooren Ulcer Th is is an aggressive p eriph eral u lcerat ive kerat it is w ith a h igh risk of corn eal m elt ing an d perforat ion n ot associated w ith system ic collagen vascular disease. An au toim m un e role is th ough t to play a role in th e developm en t of u lcerat ion an d st rom al m elt ing based on kn ow n su ppressor T-cell deficien cy, in creased im m u n oglobu lin A an t ibody levels, an d in creased levels of plasm a cells, lym ph ocytes, im m u n oglobu lin s, an d com p lem en t factor in th e periph eral corn ea an d adjacen t

5 Cornea

181

A

Fig. 5.34 (A) Corneal verticillata seen with Fabry disease. (B) Corneal verticillata from amiodarone. (C) Striate melanokeratosis with melanin deposition in the corneal epithelium.

B

C

conju n ct iva. Risk factors in clu de prior ocular t rau m a or ocular surger y as w ell as a su bset w ith prior parasit ic in fect ion w ith h elm in th s.

Presentation Sym ptom s in clude in ten se pain , redn ess, tearing, an d ph otop h obia. A ch ron ic an d progressive corn eal ulcerat ion begin s in th e periph eral corn ea w ith circu m feren t ial spread follow ed by cen t ripet al spread. A leading edge of deepith elialized t issu e is presen t w ith keratolysis. Sign ifican t corn eal n eovascu larizat ion an d fibrosis can develop. Pat ien ts are often in late adu lth ood w ith u n ilateral presen tat ion . A secon d form of u lcerat ion occurs in p at ien ts w ith preceding parasit ic h elm in th

182

Color Atlas of Ophthalm ology

Fig. 5.35

Mooren ulcer.

in fect ion ; th is form is m ore com m on in en dem ic areas of Africa w ith h igh populat ion s of parasitem ia. Th is form is often bilateral an d h igh ly associated w ith corn eal perforat ion . Mooren u lcer can be associated w ith con curren t h epat it is C in fect ion ( Fig. 5.35 ).

Differential Diagnosis Perip h eral u lcerat ive kerat it is, in fect iou s kerat it is, rosacea, st aphylococcal m argin al ulcerat ion

Management A conjun ct ival recession can be ut ilized for in it ial t reat m en t of ulcerat ion , perh aps by severing con n ect ion of th e lim bal vasculat ure an d associated in flam m ator y cells from th e region of u lcerat ion . Lam ellar keratoplast y is often n eeded in cases w ith im p en ding or fran k perforat ion . System ic im m un osuppressive agen t s such as oral cort icosteroids, m eth ot rexate, cyclosporin e, an d cycloph osph am ide h ave sh ow n prom ise in t reat m en t . Hep at it is C–associated cases h ave sh ow n im provem en t w ith in terferon th erapy. Despite t reat m en t opt ion s, th is form of ulcerat ion often h as a poor progn osis w ith a h igh corn eal perforat ion rate.

Peripheral Ulcerative Keratitis A periph eral corn eal u lcerat ion is associated w ith epith elial breakdow n an d keratolysis. Th e con dit ion can be associated w ith any con n ect ive t issue disorder (collagen vascu lar disease) bu t is m ost com m on ly seen w ith rh eu m atoid ar th rit is.

Presentation Presen tat ion in cludes pain , redn ess, an d decreased vision in th e set t ing of a con n ect ive t issu e disorder. Periph eral corn eal in filt rat ion is presen t w ith an associated epith elial defect except in th e early st ages. St rom al m elt ing m ay be th e first sign of system ic disease an d is correlated w ith exacerbat ion s of system ic disease act ivit y. Sym ptom s are u sually u n ilateral but m ay be bilateral in presen t at ion . Associated lim bal vaso-occlu sion can be seen w ith th e adjacen t lim bal vessels ( Fig. 5.36A,B).

5 Cornea

183

A

B Fig. 5.36 (A) Peripheral ulcerative keratitis from rheumatoid arthritis. (B) Peripheral corneal ulcer associated with system ic lupus erythem atosis.

Differential Diagnosis In fect ious kerat it is, Mooren ulcer, Terrien m argin al degen erat ion , furrow degen erat ion , rosacea, exposu re kerat it is

Management Th e goal of th erapy is to preven t corn eal m elt ing an d p rom ote reepith elializat ion . Surface lu brican t s su ch as art ificial tears, gels, or oin t m en t s sh ould be u sed ever y 1 to 2 h ours w ith or w ith ou t a blan d oph th alm ic an t im icrobial an t ibiot ic such as er yth rom ycin to t reat th e im m un e-m ediated dr y-eye disease. Tem porar y or perm an en t pu n ctal cau ter y can also in crease th e su rface m oist u re. System ic collagen ase in h ibitors (m acrolides) m ay be u seful. Con t rol of system ic in flam m at ion is essen t ial w ith im m u n osu ppression m edicat ion s such as predn ison e, cyclosp orin e, azath iop rin e, cylcoph osph am ide, or m eth ot rexate; th is can be adm in istered in con cer t w ith a rh eum atologist depen ding on th e oph th alm ologist’s com fort w ith system ic th erapy. Cyan oacr ylate glu e or th erapeu t ic ban dage len ses m ay be

184

Color Atlas of Ophthalm ology

n eeded in cases of severe st rom al th in n ing. A conju n ct ival recession of adjacen t lim bal conju n ct iva can prom ote h ealing of th e adjacen t periph eral st rom al m elt ing an d ulcerat ion , perh ap s becau se of elim in at ion of a sou rce of in flam m ator y cells an d collagen olyt ic en zym es from severing th e con n ect ion to th e lim bal vessels. Lam ellar an d pen et rat ing keratoplast y m ay be n eeded in cases of im pen ding or fran k perforat ion .

Terrien Marginal Degeneration Th is is an idiopath ic periph eral corn eal th in n ing disorder th at can be localized or diffuse. Th e ocu lar surface t ypically sh ow s on ly m ild in flam m at ion in associat ion w ith th e periph eral corn eal th in n ing. Th e con dit ion is u sually bilateral but m ay be un ilateral or asym m et rical in presen tat ion . Elect ron m icroscopy reveals th e p resen ce of h ist iocytes in th e corn eal lam ellae, in dicat ing a possible im m u n e-m ediated role.

Presentation Sym ptom s in clude foreign body sen sat ion , blurred vision , an d on ly m in im al sign s of redn ess or conju n ct ival inject ion . Periph eral corn eal th in n ing occu rs m ost often su periorly an d p rogresses in an an n ular pat tern w ith an overlying in t act epith elium . Th e th in n ing is accom pan ied by an an terior lipid border an d bridging vessels exten ding tow ard th e lipid base. Th e an terior lipid border is often steep, w ith slop ing of th e lim bal border. Again st-th e ru le-ast igm at ism often develops as th e th in n ing progresses. Spon tan eous perforat ion is rare bu t m ay occu r, especially w ith ocu lar t rau m a ( Fig. 5.37 ).

Differential Diagnosis Perip h eral ulcerat ive kerat it is, fu rrow degen erat ion , at ypical pellucid m argin al degen erat ion , Fu ch s su perficial m argin al kerat it is

Fig. 5.37

Terrien marginal degeneration.

5 Cornea

185

Management Man agem en t often con sists of suppor t ive care w ith lu brican t tears, gels, or oin tm en ts. Topical cyclosporin e m ay add ben efit in som e cases. Lam ellar corn eal patch graft s can be useful in cases of im pen ding perforat ion . An n u lar lam ellar graft s h ave also been used in severe cases w ith 360 degrees of progressive p eriph eral th in n ing.

Furrow Degeneration Th is m ay be an opt ical illu sion , th ough som et im es t ru e th in n ing does occur.

Presentation Th e pat ien t is u su ally asym ptom at ic. Th is m ay occu r as an idiopath ic con dit ion in th e elderly as a lu cid area separat ing th e corn eal arcu s from th e lim bu s. Corn eal th in n ing is eviden t . Th e ep ith eliu m is in tact w ith n o vascularizat ion .

Differential Diagnosis Terrien m argin al degen erat ion

Management No t reat m en t is requ ired.

Aphakic and Pseudophakic Bullous Keratopathy Refer to th e ch apter 7 sect ion on corn eal edem a as a com plicat ion of cataract su rger y.

Corneal Surgery Penetrating Keratoplasty Pen et rat ing keratoplast y con sists of a fu ll-th ickn ess replacem en t of diseased corn ea using a don or corn ea h ar vested from a h ealthy corn eoscleral don or rim . In dicat ion s for surger y in clu de corn eal edem a, corn eal scarring, corn eal dyst roph ies, keratocon u s, corn eal u lcerat ion or perforat ion , in fect ion , an d failed corn eal t ran splan ts, am ong oth ers. Th e don or t issue is secu red to th e periph eral h ost corn eal rim w ith a variet y of su t ure p lacem en t tech n iques, in cluding in terrupted su t ures, a con t in uous r un n ing sut ure, or com bin ed tech n iqu es. Risks of su rger y in clu de but are n ot lim ited to sut u re in fect ion s an d graft reject ion . Graft reject ion m ay be epith elial, subepith elial, or en doth elial in n at ure (Fig. 5.38A,B).

186

Color Atlas of Ophthalm ology

A

Fig. 5.38 (A) Penetrating keratoplast y with com bined interrupted and running suture technique. (B) Castroviejo square graft.

B

Fig. 5.39

Lam ellar keratoplast y for recurrent pterygium.

5 Cornea

187

Lamellar Keratoplasty Th is is a part ial replacem en t of th e corn ea w ith don or corn eal t issue. Th e procedu re can be u sed in keratocon us, an terior corn eal dyst roph ies, an terior corn eal scars, recurren t pter ygia, an d corn eal m elt s. Lam ellar grafts can be perform ed using an ar t ificial an terior ch am ber an d m icrokeratom e or using a w h ole globe w ith h an dh eld p ar t ial-th ickn ess don or t issu e dissect ion ( Fig. 5.39 ).

Endothelial Keratoplasty Th is corn eal t ran splan t tech n iqu e replaces th e diseased en doth eliu m w ith a posterior don or corn eal but ton con sist ing of en doth elium , Descem et m em bran e, an d a th in layer of posterior corn eal st rom a. It is used for diseases of th e en doth eliu m w h en th e epith eliu m an d st rom a are essen t ially n orm al, su ch as Fuch s dyst rophy, bullous keratopathy, en doth elial graft failu re, an d iridocorn eal en doth elial syn drom e. Tissue can be prepared u sing an ar t ificial an terior ch am ber an d m icrokeratom e or by h an dh eld dissect ion . Descem et st ripp ing en doth elial keratoplast y (DSEK) h as becom e a popu lar m eth od of perform ing th is tech n iqu e w ith st ripping of th e Descem et m em bran e an d en doth elium , follow ed by replacem en t w ith a th in don or posterior corn ea. Th e part ial-th ickn ess don or corn ea is h eld in place w ith air t am pon ade ( Fig. 5.40A,B).

A

B Fig. 5.40 (A) Endothelial keratoplast y. Preoperative corneal edema from Fuchs dystrophy. (B) Endothelial keratoplast y. Postoperative Descemet stripping autom ated endothelial keratoplast y.

188

Color Atlas of Ophthalm ology

Fig. 5.41 Khodadoust line with endothelial rejection of penetrating keratoplast y.

Graft Rejection Th e 5-year failu re rate for corn eal graft s is ~35% across th e Un ited States. Corn eal graft reject ion is th e m ost com m on cau se of graft failure in th e late postoperat ive period. Diagn osis of corn eal graft reject ion sh ould be m ade on ly in graft s th at h ave rem ain ed clear for at least 2 w eeks follow ing keratoplast y.

Presentation Pat ien ts m ay com p lain of a decrease in visu al acuit y, redn ess, ph otoph obia, p ain , an d irrit at ion . Epith elial reject ion is m arked by an elevated epith elial reject ion lin e th at stain s w ith flu orescein or rose bengal or by th e presen ce of subepith elial in filt rates. St rom al reject ion is ch aracterized by periph eral full-th ickn ess h aze w ith lim bal inject ion in a previou sly clear graft . An arc-sh aped in filt rate m ay be n oted periph erally at th e graft–h ost ju n ct ion th at progresses cen t rally. Classic en doth elial reject ion presen ts w ith an en doth elial reject ion lin e (Kh odadoust lin e) th at usually begin s at a vascularized por t ion of th e perip h eral graft–h ost ju n ct ion an d progresses ( Fig. 5.41 ). Th e com bin at ion of kerat ic precipit ates, an an terior ch am ber react ion , circu m corn eal inject ion , an d region s of corn eal edem a sh ould be diagn osed as corn eal graft reject ion .

Management Reject ion m ay be preven ted w ith steroids, cyclosporin e A, an d oth er im m u n om odu lators.

Keratoprosthesis An ar t ificial device is u sed to restore vision in con dit ion s of corn eal blin dn ess. Com m on ly u sed devices in clu de th e Boston Doh lm an Keratoprosth esis an d Alph aCor (Addit ion Tech n ology, In c., Des Plain es, IL; th e Type I device is m an u fact u red an d curren tly available th rough th e Massach u set t s Eye an d Ear In firm ar y at cost: h t t p://w w w.m asstech por tal.org/IP1416.aspx). In dicat ion s in clu de m u lt iple failed cadaveric allograft su rgeries w ith lit tle h ope for su ccess u sing fut u re cadaveric t issu e. Th is m eth od avoids allograft reject ion , bu t corn eal m elt ing, in fect ion s, an d glaucom a rem ain a con cern w ith th is device. It can also be u sed for stem cell disease in pat ien t s w h o are poor can didates for im m un osupp ression ( Fig. 5.42A,B).

5 Cornea

189

A

B Fig. 5.42 (A) Dense corneal opacification prior to Dohlman keratoprosthesis placement. (B) Postoperative Dohlman keratoprosthesis with 20/40 uncorrected Snellen visual acuit y.

Anterior Staphyloma Managed by Anterior Segment Transplantation An an terior staphylom a is a ch allenging en t it y. Mult ip le causat ive factors h ave been n oted in th e literat u re, th e com m on on es being kerat it is an d congen ital m alform at ion s, w ith sp oradic report s of disorders su ch as n eu rofibrom atosis an d sarcoidosis .Th e surgical opt ion s for an terior st aphylom a an d sim ilar diffu se corn eal lesion s in clu de a conven t ion al kerat roplast y, overlay graft s, an d par t ial- or full-th ickn ess sclerokeratoplast y. How ever, an terior st aphylom a is associated w ith addit ion al p roblem s oth er th an corn eal ectasia. Th e len s is often cataractous w ith com prom ised zon ules, w h ich h as to be t aken care of at th e t im e of th e surger y. In addit ion , staphylectom y leads invariably to loss of iris t issue, creat ing an iat rogen ic an iridia. Th erefore, an ideal t ran splan t for an an terior staphylom a or a sim ilar diffuse an terior path ology sh ould address th ese issues. Soosan Jacob h as develop ed a n ew m eth od of an terior segm en t t ran splan t at ion th at can be u sed to t reat a m alform ed an terior segm en t an d t ran splan t a n ew, bioprosth et ic graft sim u lat ing th e an terior segm en t ( Figs. 5.43A,B). Th is tech n iqu e is an exten sion of th e glu ed in t raocu lar len s (IOL) tech n iqu e inven ted by Am ar Agar w al.

190

Color Atlas of Ophthalm ology

A Fig. 5.43 (A) Anterior segm ent transplantation done on a 4-m onth-old child with anterior staphylom a. (Top) Preoperative appearance. (Bottom ) Postoperative day 1 appearance. (B) Biosynthetic graft being prepared for transplantation. One haptic of the aniridia IOL being externalized (top). Biosynthetic graft seen after both haptics have been externalized (center). The bioprosthetic graft prepared for anterior segment transplantation. Note the aniridia intraocular lens haptics externalized at the scleral level below scleral flaps (bot tom). The graft has biological components: the cornea and sclera, and synthetic components: the IOL optic, the artificial iris, and the edge of the artificial iris form ing the pupil.

Presentation Diffuse corn eal involvem en t an d cicat rizat ion of th e uveal t issu e along w ith h igh in t raocular pressu re resu lts in a large ect at ic area of th e corn ea an d lim bus.

Differential Diagnosis Peters an om aly, diffuse corn eoscleral kerat it is

B

5 Cornea

191

Management Th e bioprosth et ic graft con sists of a biological an d a prosth et ic p ar t . Th e biological par t is fash ion ed from a cadaveric w h ole globe (corn ea an d sclera), an d th e prosth et ic par t con sists of an an iridia IOL (iris, pu pil, an d IOL). Tw o par t ial-th ickn ess, lim bal-based scleral flaps of 3-m m size are created 180 degrees apart on th e don or sclera. Tw o st raigh t sclerotom ies are th en m ade w ith an 18-gauge n eedle 1.5 m m from th e lim bu s un der th e exist ing scleral flaps. A corn eo-scleral rim is th en cu t th e en t ire length of th e sclera, in clu ding in th e scleral flaps. A cyclodialysis is th en in duced to separate th e uveal t issue from th e dissected corn eoscleral but ton . Th e corn eoscleral graft is placed con cave (i.e., en doth elial side up ), an d th e an iridia IOL h apt ics are extern alized th rough th e sclerotom y un der th e scleral flaps u sing a 23-gauge forceps. Th e biosyn th et ic assem bly th u s con sist s of a don or corn ea an d sclera an d an ar t ificial iris an d len s (an iridia IOL). In th e recipien t eye, th e staphylom atous corn ea is excised. Un derlying cat aract is m an aged w ith len sectom y an d vit rectom y. Th e biosyn th et ic graft is th en placed on th e h ost an d sut u red. Th e IOL h apt ics are th en t ucked in to a scleral p ocket created at th e edge of th e scleral flaps, an d th e flap is th en glu ed dow n w ith t issu e glu e (Tisseel, Baxter, Deerfield, IL). Th e conju n ct iva is th en closed by gluing.

Enlarged Corneal Nerves Presentation Prom in en t corn eal n er ves m ay be seen radiat ing cen t rally from th e corn eal periph er y.

Differential Diagnosis Mu lt ip le en docrin e n eop lasia t ype IIb, icthyosis, Refsu m disease, leprosy, keratocon us, Fuch s en doth elial dyst rophy, osteogen esis im p erfect a, ocu lar pem ph igu s, n eurofibrom atosis, ph th isis bu lbi, posterior polym orp h ou s dyst rophy, h erpes sim plex, h erp es zoster, p rim ar y am yloidosis

Management Man agem en t is aim ed at th e un derlying con dit ion .

Corneal Neovascularization Th e corn ea is t yp ically devoid of blood vessels; h ow ever, in con dit ion s of in fect ion , in flam m at ion , or ocu lar surface in sult , blood vessels can abn orm ally exten d in to th e corn ea. Th is is often referred to as corn eal n eovascularizat ion or pan n us. It is m ain ly seen in in flam m ator y corn eal diseases such as t rach om a.

Presentation Pan n u s presen t s as a vascu lar ingrow th in to th e corn ea from th e lim bal vascu lat u re. It can be superficial or deep ( Fig. 5.44 ).

192

Color Atlas of Ophthalm ology

Fig. 5.44 A slit-lamp photograph demonstrating severe corneal neovascularization from diffuse stem cell deficiency.

Differential Diagnosis Trach om a, leprosy, h erpes sim plex or h erpes zoster kerat it is, ph lycten u lar keratoconju n ct ivit is, toxic conjun ct ivit is, acn e rosacea, bu llou s keratopathy, m ollu scum con tagiosum , vern al conjun ct ivit is, keratoconju n ct ivit is sicca, con tact len s u se, in clusion conju n ct ivit is (m icrop an n u s), su perior lim bic keratoconjun ct ivit is, con tact len s kerat it is, Fu ch s m argin al kerat it is, hypoparathyroidism , vitam in B deficien cy, an d pellagra .

Management Man agem en t is aim ed at th e un derlying con dit ion .

Leukocornea Leu kocorn ea m ay arise due to a w ide variet y of con dit ion s in clu ding in fect ion , in flam m at ion , an om alies, h eredit ar y con dit ion s, an d t rau m a.

Presentation Opacificat ion of th e corn ea is eviden t , even w ith out th e slit-lam p exam in at ion .

Differential Diagnosis Infect ions: Bacterial, fu ngal, h erpet ic u lcers, st rom al scarring, t rach om a, Acantham oeba Inflam m atory : aph akic an d pseudoph akic bullous keratopathy, Steven Joh n son s syn drom e, graft reject ion , ocular cicat ricial pem ph igoid Congenital: An terior ch am ber cleavage syn drom es, sclerocorn ea, congen it al glaucom a, derm oid, am yloidosis Hereditary : Congen it al h eredit ar y corn eal dyst rophy, Dow n syn drom e, Patau syn drom e, in born errors of m etabolism (m u copolysacch aridosis, Low e syn drom e, m ucolipoidosis) Traum a : Descem et tear (bir th t rau m a), th erm al inju r y, ch em ical burn s

5 Cornea

193

Management Man agem en t con sist s m ain ly of doing a corn eal t ran sp lan tat ion in su itable cases. Any un derlying cause sh ould also be addressed.

Chemical Burn See th e ch apter 1 sect ion on ch em ical exposu re bu rn s.

6 Intraocular Inflammation Soosan Jacob , Dhivya Ashok Kum ar, Athiya Agarw al, and Am ar Agarw al

Acute Anterior Nongranulomatous Uveitis HLA-B27-Associated Uveitis Ankylosing Spondylitis An kylosing spon dylit is (AS) is an ar th ropathy ch aracterized by back pain an d st iffn ess after in act ivit y. HLA-B27 is fou n d in u p to 90%of pat ien ts w ith AS. Th e ch an ce th at an HLA-B27–posit ive pat ien t w ill develop spon dyloar th rit is or eye disease is 1 in 4. Not all HLA-B27–posit ive pat ien ts develop disease. Sacroiliac x-ray film s sh ould be obt ain ed w h en in dicated by a suggest ive h istor y in a p at ien t w ith ocular disease con sisten t w ith HLA-B27 syn drom e. Sacroiliac xray film s sh ow sclerosis an d even t ual n arrow ing of th e join t space, ligam en tou s ossificat ion .

Complications Bony deform it y, pu lm on ar y apical fibrosis, aort it is, aor t ic valvular in su fficien cy

Uveitis Management Topical steroids an d cycloplegics. Sacroiliac join t radiograph , HLA-B27 screen ing rh eu m atology con su ltat ion

Reiter Syndrome Reiter syn drom e is ch aracterized by th e follow ing: Non specific u reth rit is Polyar th rit is Conju n ct ival in flam m at ion , often accom pan ied by irit is

Presentation Th is is com m on ly seen in young adult m ales (90%), w h ereas fem ales con st it u te on ly 10%. Arth rit is is t ypically asym m et ric an d in oligoart icular dist ribu t ion , in volving kn ees, an kles, feet , an d w rist s. Sacroiliit is is presen t in as m any as 70% of pat ien t s. Keratoderm a blen n orrh agicum (a scaly, er yth em atous disorder of th e palm s an d soles) an d circin ate balan it is (a persisten t , scaly, er yth em atous, circu m feren t ial rash of th e dist al pen is) m ay be foun d. Less com m on fin dings are plan t ar fasciit is, Ach illes ten din it is, sacroiliit is, n ailbed pit t ing, p alate u lcers, an d tongu e ulcers. Ocu lar involvem en t ranges from conju n ct ivit is (m u copur ulen t an d papillar y) to kerat it is (pu n ct ate an d subep ith elial) to an terior, n ongran ulom atou s in flam m at ion . 194

6 Intraocular In am m ation

195

Uveitis Management Topical steroids an d cycloplegics HLA-B27 screen ing becau se it is presen t in 85 to 95% of pat ien t s Rh eu m atology con sult at ion

Inflammatory Bowel Disease (IBD) Ulcerat ive colit is (diffu se in flam m at ion of th e colon ic m u cosa) an d Croh n disease (gran ulom atous iliocolit is) are both associated w ith acute irit is.

Presentation Bet w een 5 an d 12% of pat ien ts w ith u lcerat ive colit is, an d 2.4% of pat ien ts w ith Croh n disease develop acu te an terior uveit is (AAU). Of pat ien t s w ith in flam m ator y bow el disease, 20% m ay h ave sacroiliit is, an d of th ese pat ien t s, 60% are HLAB27 posit ive. Ocular involvem en t m ay occu r first an d m ay in clu de an terior uveit is, conju n ct ivit is, kerat it is, ep isclerit is, sclerit is, ext raocu lar m u scle palsies, opt ic n europathy, ret in al vascu lit is, n eu roret in it is, an d orbital in flam m at ion . System ic sym ptom s in clu de bloody diarrh ea, cram py abdom in al pain , skin rash , ar th ralgia, er yth em a n odosu m , pyoderm a gangren osum , sacroiliit is, ren al ston es, an d h epatobiliar y abn orm alit ies.

Uveitis Management Topical steroids an d cycloplegics. Treat in conjun ct ion w ith an in tern ist .

Psoriatic Arthritis Ocular involvem ent : Nongran u lom atou s, an terior in flam m at ion , n odular episclerit is, kerat it is, keratoconjun ct ivit is sicca Uveit is is n ot associated w ith psoriasis w ith ou t arth rit is. System ic: Er yth em atous, hyperkeratot ic rash , n ail pit t ing, an d distal in terp h alangeal join t ar th rit is

Uveitis Management Topical steroids, cycloplegics, im m un osup pressives. Treat in conju n ct ion w ith an in tern ist .

196

Color Atlas of Ophthalm ology

Glaucoma-Related Uveitis Posner-Schlossman Syndrome Presentation Man ifests as u n ilateral m ild acu te irit is sym ptom s th at in clu de discom for t , blurred vision , or h aloes. Sign s in clu de m arkedly elevated in t raocular pressure, corn eal edem a, fin e keriat ic p recipit ates, low -grade, an d sligh tly dilated pu pil. Posn er-Sch lossm an syn drom e m ay be associated w ith HLA-B54 gen e locus.

Management Topical steroids an d in t raocular p ressure–low ering agen ts

Uveitis-Glaucoma-Hyphema Syndrome Irritat ion of th e iris root by th e w arped foot plates of poorly m ade, rigid, an terior ch am ber in t raocular len s im plan t s cau ses th e uveit is-glaucom a-hyp h em a t riad.

Presentation In creased in t raocular pressu re, an terior ch am ber in flam m at ion , an d hyph em a form at ion in th e presen ce of an in t raocular len s

Management Topical steroids, cycloplegics, an d in t raocu lar pressure–low ering agen t s

Phacolytic Uveitis/Glaucoma Th is involves an acu te in crease in in t raocu lar pressure cau sed by blockage of th e t rabecu lar m esh w ork by len s protein an d engorged m acroph ages.

Presentation Low -grade anterior cham ber inflam m ation, increased intraocular pressure, lack of keratic precipitates, and synechiae. Aqueous tap m ay reveal swollen m acrophages.

Management In t raocu lar pressu re redu ct ion w ith osm ot ic agen t s as w ell as topical m edicat ion s. Th e cat aract n eeds to be rem oved.

Chronic Nongranulomatous Uveitis Juvenile Rheumatoid Arthritis–Associated Uveitis Juven ile rh eum atoid ar th rit is (JRA) is a grou p of diseases w ith on set before 16 years of age.

6 Intraocular In am m ation

197

Presentation System ic onset : Usu ally seen in ch ildren u n der th e age of 5 years, of w h ich < 6% presen t w ith uveit is; rash , fever, lym ph aden opathy, h epatosplen om egaly, pericardit is, an em ia, psoriasis; pat ien t s presen t ing w ith system ic on set accou n t for ~20% of all cases of JRA. Polyart icular onset : Sh ow s involvem en t of five or m ore join ts in th e first 6 w eeks of th e disease. It con st it utes 40%of JRA cases overall bu t on ly 7 to 14%of cases of JRA-associated iridocyclit is. Panciart icular onset : Th is in cludes th e vast m ajorit y (80 to 90%) of pat ien ts w ith JRA w h o h ave uveit is. Type 1 : Girls un der age 5, posit ive for an t in u clear an t ibody (ANA). Ch ron ic iridocyclit is occurs in up to 25% of th ese pat ien t s. Type 2 : Older boys, seronegative spondyloarthropathy (75%are HLA-B27 positive). Uveitis tends to be acute and recurrent rather than chronic as in t ype 1. Ocular involvem ent : It usu ally occu rs w ith in 5 to 7 years of on set of arth rit is, but th e risk rem ain s in to adulth ood. Ocular in flam m at ion occu rs in 2 to 12% of all cases an d is u sually bilateral. Un ilateral cases often progress to bilateral w ith in 12 m on th s. Th e ocu lar an d join t disease act ivit y are n ot associated. It m ay be an t in u clear an t ibody test posit ive, rh eum atoid factor n egat ive. Ch ildren are often asym ptom at ic w ith in sidious disease on set . Th ere m ay be m ild ocular pain , h eadach e, ph otoph obia, an d decreased vision . A w h ite eye w ith act ive an terior ch am ber in flam m at ion , kerat ic precipit ates, posterior syn ech ia, cataract form at ion , glau com a, an d ban d sh aped keratopathy m ay even be fou n d on th e first exam in at ion .

Management Uveitis is treated w ith topical steroids and cycloplegics. System ic or periocular steroids are som etim es needed. Ethylenediam inetetraacetic acid (EDTA) chelation is done for band-shaped keratopathy. Rheum atology consultation is often necessar y.

Fuchs Heterochromic Iridocyclitis Presentation Presen t at ion is u sually u n ilateral; sym ptom s var y from n on e to m ild blu rring an d discom fort .

Signs Diffuse iris st rom al at rophy w ith variable pigm en t epith elial layer at rophy Sm all w h ite stellate kerat ic precip itates scat tered diffu sely over th e en t ire en doth elium Cells presen t in th e an terior ch am ber as w ell as th e an terior vit reou s. Syn ech iae alm ost n ever form . Glaucom a an d cat aract s occu r. Abn orm al vessels m ay bridge th e angle on gon ioscopy. Fu n dus lesion s are absen t; som et im es toxoplasm a scars h ave been reported.

Management Cat aract su rger y an d in t raocular len s im plan t can be don e successfu lly. Glaucom a is difficult to con t rol an d m ay n eed surger y.

198

Color Atlas of Ophthalm ology

Granulomatous Uveitis Syphilis Presentation Syph ilit ic involvem en t of th e uveal t ract can presen t in t w o w ays: 1. Congenital syphilit ic uveit is: Here th e involvem en t is bilateral, th e periph er y of th e ret in a is involved, an d occasion ally on e or t w o qu adran ts of th e posterior pole can be involved rarely. Th us th e vision is n ot affected. Th e t yp ical appearan ce is th at of a salt an d pepper fun du s du e to areas of p igm en t accum ulat ion in terspread w ith areas of pigm en t loss. An associated ocu lar feat ure is th e presen ce of bilateral in terst it ial kerat it is. Secondary ret inal pigm ent degenerat ion m ay be seen , w h ich is a con dit ion th at is progressive an d is associated w ith con st rict ion of th e blood vessels of th e ret in a an d th e ch oroids in th e form of sclerosis. Th e opt ic disk is pale w ith sh arp ly defin ed borders. Pigm en t s dispersed are also sh arply dem arcated w ith a st ar sh ape or bony corpu scle form at ion . Th e posterior pole or th e periph er y can be affected, an d th e con dit ion is bilateral. 2. Acquired syphilis: Th is h as th ree com pon en t s: irit is, ch orioret in it is, an d n eu roret in it is. Th e irit is is ch aracterized by th ree form s: irit is papulosa, irit is n odosa, an d irit is roseat a. Ch orioret in it is is ch aracterized by vit reou s h aze, fin e pun ct ate gray to yellow exu dat ion areas, pigm en t accum ulat ion along th e opt ic n er ve an d blood vessels, an d flam e-sh aped h em orrh ages w ith ch orioret in al edem a. Neu roret in it is con sists of opt ic n er ve h ead involvem en t w ith vascular involvem en t of th e su rrou n ding ret in al vessels.

Management Blood tests in th e form of Ven ereal Disease Research Laborator y test (VDRL) an d th e fluorescen t t repon em al an t ibody–absorpt ion test (FTA-ABS) are don e. Uveit is is t reated w ith topical steroids an d cycloplegics. Th e system ic disease sh ou ld be t reated in conju n ct ion w ith an in tern ist .

Sarcoidosis Sarcoidosis is a ch ron ic gran u lom atous uveit is of u n kn ow n et iology. It m ay also affect th e lu ngs, eyes, an d skin .

Presentation Ocular: Sym ptom s of uveal involvem en t are variable an d frequ en tly in clude m ild to m oderate blurring of vision . It m ay involve all st ru ct ures of th e eye. A sizable propor t ion of pat ien t s develop ch ron ic gran ulom atou s iridocyclit is. Typical fin dings are m u t ton fat kerat ic precipitates, Koeppe an d Bu sacca iris n odules, an d sn ow balls in th e in fect iou s an terior vit reous. Nu m m u lar corn eal in filt rates, en doth elial op acificat ion , an d large iris gran ulom as also occu r. Posterior syn ech iae can be exten sive an d m ay lead to iris bom bé an d angle closure glaucom a. Periph eral an terior syn ech ia m ay be exten sive, involving 360 degrees in advan ced cases. Secon dar y glaucom a can be severe.

6 Intraocular In am m ation

199

Posterior segm ent involvem ent is characterized by nodular granulom as m easuring ¼ to 1 disc diam eter that occur in both the retina and the choroid. Irregular nodular granulom as along venules have been term ed candlew ax drippings or taches de bougie. Linear or patchy retinal periphlebitis presents as sheathing. Cystoid m acular edem a is com m on; retinal neovascularization, disk edem a, and optic nerve granulom as also occur. Palpebral and bulbar conjunctival nodules also occur. Lungs: Hilar aden opathy. Central nervous system (CNS): Cran ial an d periph eral n europathy, asept ic m en ingit is. Cardiovascular system : Cardiac arrhyth m ias, pericardit is, ar th rit is, m yosit is, er yth em a n odosa, ren al involvem en t h epatosplen om egaly an d bon e m arrow in filt rat ion .

Management Serum angioten sin -conver t ing en zym e an d lysozym e levels are in creased. Ch est x-ray sh ow s h ilar aden opathy.

Uveitis Topical, periocu lar, an d system ic steroids, topical cycloplegics. Treat in conju n ct ion w ith an in tern ist .

Tuberculosis Presentation Tu bercu lou s uveit is can presen t in clu de th e follow ing w ays: Acute n ongran ulom atous in fect ion —im m u n ological or allergic in n at ure Nodu lar gran u lom atous in filt rates th at are fulm in at ing an d caseat ing. Tw o t ypes of n odu les are presen t as sh ow n in Table 6.1 . Relapsing an d recurren t irit is Low -grade ch ron ic in flam m at ion th at presen ts w ith cataract , glaucom a, or pth isis bu lbi Ch oroidal lesion s appear as raised m ult iple yellow n odules w ith blurred m argin s. Ret in al periph lebit is an d su bret in al vascu larizat ion s are seen . Oth er ocu lar feat ures of t uberculosis in clu de ph lycten u losis, episclerit is, n odu lar sclerit is, an d opt ic n eu rit is.

Management An t it uberculous t reat m en t is started in conjun ct ion w ith an in tern ist . Steroids sh ould be started on ly after star t ing an t it u bercu lou s t reat m en t . Table 6.1

Tw o Types o f No dular Granulo m ato us In ltrates

So litary o r co nglo m erate no dule

Miliary no dules

Otherwise healthy immune responsive patients Single large tum orlike nodule yellow to white

Im munocompromised patients, severely debilitated patients Multiple nodules over the iris at the papillary margin and ciliary body with dissemination of bacilli

200

Color Atlas of Ophthalm ology

Intermediate Uveitis/Pars Planitis Presentation The disease is t ypically seen in children. It is also know n as chronic cyclitis. Patients have visual sym ptom s like blurred, distorted vision secondar y to m acular edem a. They also com plain of floaters. The active vitreous inflam m ation show s cells that are sm all, round, nonpigm ented, and num erous. The old vitritis has cells that are irregular, pigm ented, and rem ain in the form ed vitreous. At the edges of the ora serrata there is snow banking that is characteristic of deposition of inflam m atory cells at this region. Anterior segm ent inflam m ation signs such as congestion and tenderness are absent. The sym ptom s of pain or photophobia are also absent or m inim al. Th ere are several key differen t iat ing feat u res: Periph eral ret in it is Perivasculit is Vit rit is Sn ow ban king at th e in ferior periph eral ret in a

Differential Diagnosis Mu lt iple sclerosis, sarcoidosis, t u bercu losis, toxocariasis, syph ilis, Lym e disease

Management Steroids are given via topical, su bten on s, or oral route. System ic im m u n osup pressan t s an d vit rectom y m ay also be n ecessar y in som e cases.

Posterior Uveitis Toxoplasmosis Toxoplasm osis is th e m ost com m on cau se of ch orioret in it is, congen it al in origin . W h en th e m oth er is affected in th e first t rim ester, th ere is a 40% risk of t ran sm ission to th e fet u s.

Presentation Tw o form s of toxoplasm osis exist: congen ital an d adult . Both form s are ch aracterized by acu te focal ch orioret in it is ( Fig. 6.1 ). Congenital toxoplasm osis: It is bilateral in 85% of ch ildren an d 80% h ave ch orioret in it is. W h en CNS sym ptom s su ch as convulsion s, hydrocep h alus, m en tal retardat ion , an d in t racran ial calcificat ion s are absen t , congen ital lesion s are diagn osed w h en th e ch ild p resen t s w ith esot ropia or exot ropia or decreased vision . Th e clin ical ch aracterist ics of th e congen it al form are as follow s: Bilateral Mu lt ip le ch orioret in al lesion s, especially in th e m acular area Pu n ch ed-ou t lesion du e to fu ll-th ickn ess n ecrosis Heavily p igm en ted scar, m ist aken to be congen ital colobom as

6 Intraocular In am m ation

201

A

B

C

D Fig. 6.1 (A) Arterial phase fluorescein angiogram (FA) demonstrating active toxoplasma chorioretinitis adjacent to a classic chorioretinal scar. (B) Arteriovenous phase FA demonstrating the classic old pigmented lesion with hypofluorescence at the center and hyperfluorescence at the margins of the lesion. (C) Late -phase FA showing juxtapapillary multiple hyperfluorescent areas corresponding to new, active lesions. (D) Active lesions, areas of hyperfluorescence, are usually seen in the vicinit y of old scars, as evidenced here. (Courtesy of J. Fernando Arevalo, Venezuela)

Adult toxoplasm osis: Th ere is react ivat ion of in flam m at ion at th e edges of a preexist ing scar; th is is called a satellite lesion . Vit rit is is severe an d th e m argin s of th e lesion are yellow an d blurred. Th ere is a hypersen sit ivit y react ion to th e t roph ozoites th at are released from th e cysts. Th e follow ing term s h ave been used to describe th e lesion : Searchlight in fog: Yellow lesion s seen th rough th e vit reous h aze Grapevines: Of th e m em bran es an d cells of vit rit is W et snow : St icky vit reou s exudates in vit rit is

Differential Diagnosis In ch ildren , differen t ials in clude congen it al colobom as, cytom egaloviru s in clusion ch orioret in it is, h erp es sim plex ch orioret in it is, toxocariasis, ret in oblastom a, an d cerebral t rau m a. In adu lt s, differen t ials in clu de t u berculous ch orioret in it is, can didiasis, an d h ist ioplasm osis.

202

Color Atlas of Ophthalm ology

Management An t ibiot ics in th e form of Bact rim DS (dou ble st rength ), clin dam ycin , or pyrim eth am in e w ith folin ic acid are given for 6 w eeks in conjun ct ion w ith an in tern ist . Oral steroids m ay be in dicated w h en th e disease spreads close to th e opt ic n er ve or m acu la.

Presumed Ocular Histoplasmosis Histoplasm a capsulat um causes th e ch aracterist ic presu m ed ocu lar h istop lasm osis syn drom e (POHS). Im m un ocom prom ised pat ien t s are affected by th e presen ce of an isolated gran u lom a or en doph th alm it is. POHS t ypically affects you ng people an d is respon sible for bilateral visu al loss. Th e ch aracterist ic feat u res of th e syn drom e are th e appearan ce of isolated disciform m acular lesion s an d scars th at are w ell circu m scribed. Th ese are quiet lesion s w ith ou t th e presen ce of act ive in flam m at ion an d th e presen ce of peripapillar y pigm en t at rophy ( Fig. 6.2 ).

A

B

C

D Fig. 6.2 (A) Disseminated choroiditis (histo spots), maculopathy, and peripapillary chorioretinal degenerative changes in a patient with the presumed ocular histoplasmosis syndrome. (B) Disseminated choroiditis (histo spots), maculopathy, and peripapillary chorioretinal degenerative changes in another patient with the presumed ocular histoplasmosis syndrome. (C) Fluorescein angiography of the patient in Fig. 6.2B, revealed serosanguinous retinal detachment with faint pigment halo in right macula and choroidal neovascularization. (D) Fluorescein angiography of patient in Fig. 6.2B, revealed hypofluorescence after photodynamic therapy with verteporfin. (Courtesy of Steve Bloom, MD).

6 Intraocular In am m ation

203

Management Follow up for developm en t of ch oroidal n eovascu lar m em bran e an d t reat accordingly.

White Dot Syndrome Multifocal Choroiditis Mu lt ifocal ch oroidit is is a rare disorder involving idiopath ic in flam m at ion of th e ch oroid an d ret in al pigm en t epith eliu m (RPE).

Presentation Presen t at ion is u sually bilateral, w ith sym ptom s such as ph otoph obia, blu rred vision , eye pain , an d decreased visual acu it y. Th e pat ien t m ay also h ave m etam orph opsia, floaters, scotom as, an d ph otopsia. Th ere m ay be sign s an d sym ptom s of an terior uveit is. Mu lt iple, sm all, yellow /gray-w h ite spot s m ay be seen along w ith cystoid m acu lar edem a, ch oroidal n eovascular m em bran e, an d subret in al fibrosis.

Management Cor t icosteroids are h elpfu l in resolving th e lesion s.

Multiple Evanescent White Dot Syndrome Mu lt iple evan escen t w h ite dot syn drom e (MEW DS) is an acute bu t ben ign , rare, un ilateral disease of u n kn ow n et iology involving th e RPE an d ch oroidal capillaries. It u su ally affect s young fem ales. It can rarely be bilateral ( Fig. 6.3 ).

B

A Fig. 6.3 Multiple evanescent white dot syndromes (MEWDS). (A) Fundus examination revealed sm all and large spots scat ted through the fundus in the left eye. (B) Fluorescein angiography showed punctate hyperfluorescence. (Courtesy of Antonio Ciardella, MD). (Continued on page 204)

204

Color Atlas of Ophthalm ology

C

D Fig. 6.3 (Continued) Multiple evanescent white dot syndromes (MEWDS). (C) Indocyanine green videoangiography demonstrated small and large hypofluorescent spots. A diagnosis of MEWDS was made. (D) One month later the spots in the fundus had disappeared, and visual acuit y recovered. (Courtesy of Antonio Ciardella, MD).

Presentation Mu lt iple w h ite dot s at th e level of th e RPE an d opt ic n er ve h ead sw elling m ay be seen . Th e m acula often h as a gran u lar appearan ce. Visual-field defects in th e form of an en larged blin d spot an d paracen t ral an d cen t ral scotom as m ay also be seen . Visual loss, even if sign ifican t in it ially, alm ost alw ays ret urn s to n orm al later. Th e pat ien t m ay also h ave ph otopsia.

Management Fu n du s fluorescein angiography sh ow s focal areas of early pu n ct ate hyperfluorescen ce th at corresp on d to th e w h ite dots. Th e ch anges on in do-cyan in e green angiography m ain tain longer th an fu n dus ph oto an d FFA. Th e progn osis for visu al acuit y in MEW DS is ver y good, an d it resolves on its ow n .

Acute Multifocal Posterior Placoid Pigment Epitheliopathy Th is con dit ion closely resem bles MEW DS. It m ay be associated w ith HLA-B7.

Presentation Sim ilar to MEW DS, it can also presen t w ith a viral predrom e follow ed by t ran sien t visu al loss in young to m iddle-aged adu lt s. It is u su ally bilateral. Ret in al lesion s con sist of m u lt iple yellow -w h ite placoid lesion s, w h ich are larger th an in MEW DS, involving th e RPE ( Fig. 6.4 ).

6 Intraocular In am m ation

205

A

B

C

D Fig. 6.4 (A) Early phase fluorescein angiography in acute posterior multifocal placoid pigment epitheliopathy (APMPPE) shows irregular areas of blocked fluorescence characteristic of acute lesions. (B) At the arteriovenous phase, acute lesions still block fluorescence and are well demarcated. (C) Mid- and (D) late -phase angiograms dem onstrate progressive, diffuse, even staining of the acute lesions. (Courtesy of J. Fernando Arevalo, Venezuela)

Management FFA sh ow s early blocked fluorescen ce w ith late st ain ing. Th e lesion s slow ly h eal w ith scarring an d leave beh in d exten sive RPE defects th at persist .

Serpiginous Choroiditis Th is is a recurren t in flam m ator y disease of th e RPE an d ch oroid th at is gen erally bilateral.

206

Color Atlas of Ophthalm ology

A

B Fig. 6.5 (A) Fundus photograph and (B) hyperfluorescence on fluorescein angiography of a patient with serpiginous choroiditis demonstrating choroidal neovascularization. (Courtesy of J. Fernando Arevalo, Venezuela)

Presentation Serpigin ou s ch oroidit is is seen m ost com m on ly in adu lts in th e fou rth to sixth decade of life. Blu rred vision is th e first sym ptom . Vit reou s varies from clear to m ildly cellular. A serpigin ou s or geograph ic (m aplike) pat tern of scars m ay presen t in th e posterior fun du s. Edges of th ese lesion s m ay be act ive, w ith a yellow -gray an d edem atou s appearan ce. As act ive lesion s becom e at ioph ied over w eeks to m on th s, n ew lesion s can occur elsew h ere or con t iguously in a sn ail-like pat tern . Scotom as m ay be seen . FFA sh ow s early hypoflu orescen ce w ith late hyp erflu orescen ce of lesion s during act ive disease ( Figs. 6.5 an d 6.6 ).

Management Oral steroids along w ith im m u n osu ppressan t s

Birdshot Retinochoroidopathy (Vitiliginous Chorioretinitis) Th is is a cau se of ch ron ic p osterior uveit is, w ith a fem ale predilect ion . It is also associated w ith HLA-A29.

Presentation Mu lt iple sm all w h ite/yellow spot s are seen scat tered about th e posterior pole in th e deep ret in a an d ch oroid. Vit rit is an d m acular edem a w ith or w ith ou t epiret in al m em bran e form at ion m ay be seen . Disk edem a an d opt ic n er ve in flam m at ion w ith peripapillar y at rophy m ay be seen ( Fig. 6.7 ).

Management Th ough it gen erally ru n s a ben ign course, it is poten t ially blin ding secon dar y to m acu lar in flam m at ion an d perm an en t dam age. Hen ce, in cases w ith sign ifican t in flam m at ion or vision -affect ing m acular edem a, aggressive t reat m en t sh ou ld be given .

6 Intraocular In am m ation

A

207

B

C

D

Fig. 6.6 Serpiginous choroiditis. (A) Color fundus photo. (B) Fluorescein angiography shows early blockage. (C) As the angiogram proceeds, the active margins progressively becom e hyperfluorescent (D) and spread toward the center of the lesion as it absorbs dye from the choriocapillaris. (Courtesy of J. Fernando Arevalo, Venezuela)

A

B

C

D

Fig. 6.7 Birdshot retinochoroidopathy. (A) Color fundus photo of right and (B) left eye. (C) Early fluorescein angiography with choroidal infiltration and m inim al retinal pigm ent epithelium atrophy. The spots are hypofluorescent. (D) The lesions become m ildly hyperfluorescent in the late phases of the study as dye from the choriocapillaris stains the extrachoroidal vascular space. (Courtesy of J. Fernando Arevalo, Venezuela)

208

Color Atlas of Ophthalm ology

Cytomegalovirus (CMV) Retinitis Presentation Congenital CMV: Most com m on ly seen at th e posterior pole w ith overlying vit reous h aze an d pigm en t clum ps. Th ere can be m ild ch orioret in it is or a severe n ecrot izing ch orioret in it is. Th e lesion s are m u lt iple, an d hyperplast ic m acular scarring can be seen . Th e vision loss can be secon dar y to opt ic n er ve abn orm alit y. Im m unosuppressed CMV ret init is: Ret in al in farcts m an ifest ing as cot ton w ool spot s, ret in al h em orrh ages, ret in al n ecrosis, vit reou s h aze, an d ret in al vasculit is are seen ( Figs. 6.8 an d 6.9 ).

Management CMV ret in it is is t reated w ith in t raven ou s gan ciclovir (5 m g/kg ever y 12 h ou rs) or in t raven ous foscarn et (60 m g/kg ever y 8 h ours) or cidofovir. In t ravit real inject ion s m ay also be given .

Fig. 6.8

Cytomegalovirus retinitis.

6 Intraocular In am m ation

209

A

B Fig. 6.9 Cytomegalovirus (CMV) retinitis in an AIDS patient. (A) Early indocyanine green angiography (ICG-V) frame shows the onset of fluorescence. Engorged leaking choroidal vessels (white arrows) indicating inflammatory choroidal vasculopathy. (B) Maximum fluorescence on ICG-V of leaking choroidal vessels (white arrow). (Courtesy of J. Fernando Arevalo, Venezuela)

Acute Retinal Necrosis Syndrome Presentation Th is syn drom e is a form of severe posterior uveit is th at is cau sed by varicella zoster virus an d h erp es sim plex viru s t ypes 1 an d 2. It is defin ed by th e follow ing ver y defin ite clin ical ch aracterist ics: presen ce of focal, defin ed areas of ret in al n ecrosis in th e perip h eral ret in a out side th e vascular arcades, rapid p rogression to con fluen t circu m feren t ial n ecrosis, occlusive vasculopathy, m arked vit rit is, an d irit is. Oth er feat ures are opt ic at rophy, sclerit is, an d p ain .

Management System ic an t ivirals su ch as acyclovir an d valacyclovir are given in it ially follow ed by oral steroids. An t icoagu lan t th erapy m ay be requ ired. Prophylact ic laser ph otocoagulat ion or pars plan a vit rectom y m ay be required.

210

Color Atlas of Ophthalm ology

Vogt-Koyanagi-Harada Syndrome Presentation Headach e, n au sea Bilateral vit rit is, ch oroidit is, an d m ult iple oval det ach m en t s of th e ret in a th at are exu dat ive Ret in al an d peripapillar y n eovascu larizat ion s Mu t ton fat kerat ic precipit ates Papillar y n odules an d sh allow an terior ch am bers Differen t ial diagn osis: sym path et ic oph th alm ia, sarcoidosis, acu te posterior m u lt ifocal placoid pigm en t epith eliopathy Associated fin dings in clude alopecia, vit iligo, poliosis, an d h earing loss ( Figs. 6.10 an d 6.11 ).

Management Treat aggressively w ith cycloplegics; top ical, periocular, an d system ic steroids; an d/or im m u n osu ppressive agen t s.

Fig. 6.10 Occlusion (arrows) of choroidal vessels in a patient with Vogt-Koyanagi-Harada syndrome. (Courtesy of J. Fernando Arevalo, Venezuela)

6 Intraocular In am m ation

211

A

B

C

D Fig. 6.11 Vogt-Koyanagi-Harada syndrome. (A) Individual leaking choroidal vessels (black arrow s) indicating inflamm atory choroidal vasculopathy and patchy hypofluorescent areas (w hite arrow s) were visible in the early-phase indocyanine green videoangiography (ICG-V) in the acute disease. (B) Note m arked decrease in the number of large choroidal vessels (black arrow s) in the early phase ICG-V in the acute disease. (C) Evenly sized hypofluorescent spots are observed in the intermediate phase ICG-V at the acute phase of the disease. (D) Some of the spots are persisting into the late phase. (Courtesy of Leyla Atmaca, MD)

212

Color Atlas of Ophthalm ology

Behçet Syndrome (Oculo-Oro-Genital Syndrome) It w as first described by Beh çet as a syn drom e ch aracterized by recurren t oral aph th ous u lcers, gen ital u lcers, an d uveit is ( Fig. 6.12 ).

Presentation Major feat u res Recurren t aph th ou s ulcerat ion of th e oral m ucou s m em bran e Skin lesion s (er yth em a n odosum –like lesion s), su bcu tan eous th rom boph lebit is, folliculit is (acn elike lesion s), cu t an eou s hyp ersen sit ivit y Eye lesion s (iridocyclit is, ch orioret in it is, ret in ouveit is) an d a defin ite h istor y of ch orioret in it is or ret in ouveit is Gen ital u lcers Mino r features Ar th rit is w ith out deform it y an d an kylosis Gast roin test in al lesion s ch aracterized by ileocecal u lcers Epididym it is Vascular lesion s CNS sym ptom s

A

B

C

D Fig. 6.12 Behçet syndrome. (A) Hypofluorescent spots in the late phase of indocyanine green videoangiography (ICG-V). (B) These spots cannot be seen on fluorescein angiography. Hyperfluorescence due to pigment epithelial changes can be observed. (C) Hyperfluorescent spots in the late phase of ICG-V. (D) These spots cannot be seen on fluorescein angiography. (Courtesy of Leyla Atmaca, MD)

6 Intraocular In am m ation

213

Differential Diagnosis Com plete : Fou r m ajor feat u res Incom plete : (1) Th ree m ajor feat ures, (2) t w o m ajor an d t w o m in or feat u res, or (3) t ypical ocu lar sym ptom an d on e m ajor or t w o m in or feat ures Possible : (1) Tw o m ajor feat ures or (2) on e m ajor an d t w o m in or feat u res

Management Cor t icosteroids an d oth er im m u n osup pressives h ave been t ried. Em pirical t reatm en t is given .

Sympathetic Ophthalmia Presentation Characterist ic sym ptom s: Pain , ph otoph obia, an d decreased accom m odat ion Characterist ic signs: In flam ed an d th icken ed uveal t issu e w ith edem atous iris, disk sw elling, n odu les, papillit is, an d yellow n odu les called Dalen -Fuch s n odules Characterist ic associat ions: vit iligo, poliosis, alopecia Different ial diagnosis: Vogt Koyan agi Harada, len s-in duced uveit is

Treatment Steroids via all routes an d im m un osu ppressan ts are in dicated.

Endophthalmitis Traumatic Endophthalmitis Bacteria or fungi are in t rodu ced at th e t im e of inju r y in t rau m at ic en doph th alm it is. It can occur in u p to 13%of cases of pen et rat ing globe t rau m a. Th e cau sat ive organ ism differs from oth er en doph th alm it is w ith gram -p osit ive bacteria accou n t ing for 61.0% cases, gram -n egat ive bacteria for 10.2% of cases, fungi in 8.3% cases, an d polym icrobial in fect ion s in 15.6% cases. Th e m ost com m on gram -posit ive organ ism s w ere coagu lase-n egat ive Staphylococcus (21.5%) an d Bacillus species (18.5%), follow ed by St reptococcus species (14.8%) an d Staphylococcus aureus (8%).

Presentation Becau se ocular t raum a gen erally occu rs in a n on sterile environ m en t , m ost inju ring object s are con tam in ated w ith m ult iple in fect ious agen ts. Pat ien t s w ith a larger area of lacerat ion , delay in su rger y, rupt u red len s capsu le, retain ed in t raocular foreign body, n on m et allic foreign body, an d dir t y w ou n d are m ore com m on ly associated w ith post t rau m at ic en doph th alm it is. Th e pat ien t presen t s w ith pain , ph otop h obia, an d decreased vision occurring a variable period, even years, after pen et rat ing ocu lar t raum a. Sign s of in t raocu lar in fect ion are seen .

214

Color Atlas of Ophthalm ology

Management Early repair of th e injur y is n ecessar y. A retain ed in t raocu lar foreign body m ay requ ire a vit rectom y. If th e risk of en doph th alm it is is greater, con sider t aking an aqu eou s an d vit reous t ap for cu lt ure an d sen sit ivit y. In t ravit real an t ibiot ics such as van com ycin hydroch loride (1 m g in 0.1 m L) an d am ikacin (0.4 m g in 0.1 m L) or ceftazidim e (2.25 m g in 0.1 m L) m ay be given at th e t im e of su rger y along w ith in t racam eral an d in ten sive postop erat ive an t ibiot ics, w h ich are ch anged according to cult u re an d sen sit ivit y report s. On ce th e in fect ion is con t rolled, steroids m ay be added to decrease th e in flam m at ion .

Postoperative Endophthalmitis Any su rgical procedure on th e eye th at disru pts th e in tegrit y of th e globe, h ow ever m in or th e breach m ay be, can lead to postop erat ive en dop h th alm it is, su ch as cat aract , glau com a, vit rectom y, an d radial keratotom y. Postoperat ive en doph th alm it is represen ts 70% of in fect ive en doph th alm it is. Th e large m ajorit y of cases follow cataract su rger y, w ith an approxim ate prevalen ce of 0.082 to 0.1%. It can occur secon dar y to periocu lar flora gain ing access in to th e eye du ring su rger y, organ ism s being carried in to th e eye as su rface flu id reflu xes th rough th e w oun d du ring surger y, secon dar y to in t raocu lar len s con t am in at ion if it tou ch es th e ocular surface, or w ith th e use of con tam in ated irrigat ion solut ion s (Fig. 6.13 ).

Presentation Th e pat ien t gen erally presen t s w ith pain , redn ess, decreased vision , lid edem a, h azy corn ea, an d hypopyon . Th ree form s of presen tat ion m ay be seen : Acute form : Usu ally fulm in an t , occurs 2 to 4 days postop, m ost com m on ly du e to S. aureus or st reptococci Delayed form : Moderately severe, occurs 5 to 7 days postop, due to Staphylococcus epiderm idis, coagu lase-n egat ive cocci, rarely fungi Chronic form : Occu rs as early as 1 m on th postop, due to Propionibacterium acnes, Staphylococcus epiderm idis, or fungi

Fig. 6.13

Endophthalmitis—conjunctivitis.

6 Intraocular In am m ation

215

Management Cer tain m easures, such as 3 days of p reoperat ive prophylact ic an t ibiot ics an d preoperat ive povidon e-iodin e (5%) scr ub, can h elp reduce th e risk of postoperat ive en doph th alm it is (POE). Prim ar y use of preoperat ive topical fou rth -gen erat ion flu oroqu in olon es su ch as m oxifloxacin an d gat ifloxacin is ben eficial an d is bet ter for preven t ing resistan ce. Th e En dop h th alm it is Vit rectom y St udy w as a m u lt icen ter ran dom ized t rial perform ed at 24 cen ters in th e Un ited St ates (1990 to 1994) to determ in e th e role of in t raven ou s an t ibiot ics in th e m an agem en t of POE an d th e role of in it ial vit rectom y in m an agem en t . Th e st udy con clu ded th at system ic an t ibiot ics w ere of n o ben efit an d th at in it ial vit rectom y w as on ly ben eficial for pat ien t s presen t ing w ith a ver y poor visu al acu it y. Cu lt u res sh ou ld be t ake n from t h e aqu eou s an d vit re ou s. Th e p ossibilit y of isolat in g an organ ism from t h e vit reou s is 56 to 70%, w h ereas from t h e aqu eou s it is 36 to 40%. In est ablish ed e n d op h t h alm it is, oral or in t rave n ou s an t ibiot ics h ave p oor p e n et rat ion in to t h e vit re ou s cavit y. Hen ce in t ravit real inject ion s are t h e t reat m en t of ch oice. For gram -p osit ive organ ism s, a sin gle d ose of in t ravitreal van com ycin 1 m g in (0.1 m L) h as ad e qu ate an t ibiot ic con cen t rat ion s for over 1 w eek. Am ikacin (0.4 m g in 0.1 m L) an d ceft azid im e (2.25 m g/0.1 m L) are effe ct ive again st gram -n egat ive organ ism s. Th u s van com ycin com bin e d w it h am ikacin or ceft azid im e ap p ears to be t h e best com bin at ion for t h e t reat m e n t of POE. Dose of subconjunct ival ant ibiot ics: Van com ycin (25 m g in 0.5 m L) an d ceftazidim e (100 m g in 0.5 m L) or am ikacin (25 m g in 0.5 m L) Dose of topical fort ified ant ibiot ics: Van com ycin (50 m g/m L) an d am ikacin (20 m g/m L), altern at ing ever y 1 to 4 h ours Dose of cort icosteroids : Topical, sub-conju n ct ival inject ion (dexam eth ason e, 6 m g in 0.2 m L), oral (p redn isolon e 30 m g by m ou th t w ice a day for 5 to 10 days), or in t ravit real

Endogenous Endophthalmitis En dogen ou s en doph th alm it is is gen erally of h em atogen ou s origin an d u su ally affect s adult s w ith predisposing con dit ion s such as diabetes, u rogen it al an d gast roin test in al t ract disorders, en docardit is, an d pat ien ts on im m u n osu ppressives or h aving u n dergon e invasive procedures. Th e et iological organ ism m ay be gram posit ive or n egat ive bacteria or fungi. In th e p ediat ric age grou p, n eon atal in fect ion h as been seen w ith grou p B st reptococcal or Candida albicans.

Presentation It m ay presen t acutely or as a slow ly progressive con dit ion . Th e p at ien t m ay presen t w ith pain an d decreased visual acu it y. Exam in at ion m ay sh ow a spect rum of clin ical sign s ranging from m in im um sign s of in flam m at ion , hypopyon , vit rit is, Roth spot s, ret in al periph lebit is, to pan oph th alm it is in severe cases. W h ite ch orioret in al in filt rates w ith fluffy w h ite vit reou s opacit ies (“st ring of pearls” appearan ce) m ay be seen in Candida endophthalm it is.

Management Blood cu lt u res, in t raocu lar cu lt u res obtain ed from both ch am bers, an d cu lt ures from oth er sites su ch as an in dw elling cath eter are taken . Early in t raven ou s an t ibiot ic th erapy is crucial. Th e pat ien t sh ou ld be w orked up system ically to de-

216

Color Atlas of Ophthalm ology

term in e th e et iology, source, an d cau se for th e en dogen ou s en dop h th alm it is. Th e role of in t ravit real an t ibiot ics an d vit rectom y is con t roversial u n like in exogen ous en doph th alm it is. Top ical an d periocu lar an t ibiot ic/an t ifungal agen t s along w ith cycloplegics m ay be used.

Masquerade Syndromes Masquerade syn drom es are disorders th at occu r w ith in t raocu lar in flam m at ion an d are often m isdiagn osed as a ch ron ic idiopath ic uveit is. Th ese are m align an cies th at presen t w ith react ion s in th e an terior an d posterior segm en t of in flam m at ion th u s m asqu erading as cases of uveit is ( Table 6.2 ).

Intraocular Lymphoma Most pat ien t s w ith in t raocu lar lym ph om a h ave im m un osuppression . Th ey gen erally p resen t w ith blurr y vision an d floaters, w ith slit lam p exam in at ion often sh ow ing m ild cells an d flare an d kerat ic precipitates. Vit rit is m ay also be seen w ith su bret in al yellow in filt rates an d som et im es h em orrh agic ret in al vascu lit is.

Intraocular Leukemia In t raocu lar leu kem ia m ay presen t w ith hypopyon , vit reous an d opt ic n er ve in filt rat ion , an d vascu lit is.

Retinoblastoma Ret in oblastom a sh ou ld be ruled ou t in ch ildren less th an 3 years of age presen t ing w ith uveit is. Pseudohypopyon m ay be seen along w ith vit reous seedlings.

Choroidal Melanoma Approxim ately 5% of uveal m elan om as p resen t w ith ocu lar in flam m ator y react ion s. A black hypopyon m ay be seen (see ch apter 11, sect ion on Ch oroidal Melan om a).

Table 6.2

Masquerading Cases o f Uveitis

Retino blasto m a

Juvenile xantho granulo m as

Pseudohypopyon Recurrent hyphema nodules on iris yellow, poorly de ned Retinal lesions tumors. X-ray shows calci cation

Malign an t leukem ias and lym pho m as Anterior cham ber Aspirate Iris tissue biopsy Central nervous system lymphoma presents with vitritis and choroidal nodules

6 Intraocular In am m ation

217

Intraocular Foreign Body A retain ed in t raocu lar foreign body can cau se siderosis, m an ifest ing as a focal low grade in flam m at ion , cat aract form at ion , or ch ron ic in flam m at ion .

Schw artz-Jampel Syndrome Rh egm atogen ous ret in al detach m en t m ay be associated w ith an terior ch am ber react ion an d glau com a.

Juvenile Xanthogranuloma Juven ile xan th ogran ulom a is a rare pediat ric disorder affect ing th e h ist iocytes of th e skin . It m ay som et im es affect th e eye an d can presen t w ith m any differing ocu lar m an ifest at ion s su ch as m asquerade uveit is, h eteroch rom ia, hyph em a, or glaucom a.

Paraneoplastic Syndrome Tu m or-associated ret in op athy secon dar y to cu tan eous m elan om a or bron ch ial carcin om a m ay som et im es occu r. Su ch pat ien t s m ay h ave bilateral ret in opathy an d loss of vision .

7 Lens and Cataract Athiya Agarw al, Soosan Jacob , and Am ar Agarw al

Posterior Polar Cataract Th e posterior polar cat aract h as a un ique circular w h orl-like app earan ce located in th e cen t ral axis n ear th e n odal poin t of th e eye w ith th e rest of th e len s rem ain ing clear. It is frequ en tly associated w ith a w eaken ed or deficien t posterior capsule. Missing th e diagn osis in a posterior polar cataract can be cat ast roph ic an d a n igh tm are.

Presentation Th e bu ll’s-eye appearan ce is path ogn om on ic of posterior polar cataracts. How ever, th is en t it y could be cam ouflaged u n der a den se n uclear sclerosis or a tot al w h ite cataract ( Fig. 7.1A).

Differential Diagnosis In terdigit at ion w ith th e posterior cap su le is ch aracterist ic as opposed to a posterior su bcapsular cat aract .

Management A sm all, con t in u ous cur vilin ear cap su lorh exis is aim ed for in th e even t u alit y of th e in t raocu lar len s h aving to be placed in th e su lcu s. Hydrodissect ion m ay cau se hydraulic perforat ion at th e w eaken ed area of th e capsu le; h en ce on ly a careful, con t rolled hydrodelin eat ion is p referred. Th is epin uclear sh ell provides addit ion al protect ion by tam pon ading any vit reous or capsular breach du ring ph acoem ulsi-

A

B Fig. 7.1 (A) Posterior polar cataract. Note hydrodelineation only done. No hydrodissection has been done. (B) Microphakonit is started. Note the 0.7-mm irrigating chopper and 0.7-m m phako tip without the sleeve inside the eye. All instruments are made by MicroSurgical Technology, Redmond, WA. The assistant continuously irrigates the phaco probe area from outside to prevent corneal burns. 218

7 Lens and Cataract

219

ficat ion . A sm all am oun t of viscoelast ic can be injected ju st u n der th e rim of th e rh exis all arou n d to form a m ech an ical barrier for flu id from acciden tally en tering th e subcapsular plan e w h ile perform ing hydrodelin eat ion . Because th e n u cleu s after hydrodelin eat ion is ver y sm all, it can be rem oved easily eith er by carou selling (con st an tly rot at ing th e n ucleus to prolong occlusion an d allow m ore effect ive breakdow n of th e cataract) it ou t w ith th e ph aco t ip or by u sing a ch op m an euver. Ph aco ch op is esp ecially h elpfu l in case of associated n uclear sclerosis. If th e cen t ral plaque w as n ot rem oved at th e t im e of su rger y, it can be tackled by a yit t riu m alu m in u m garn et capsu lotom y postoperat ively. Sub 1 mm 700-Micrometer Ca ta ra ct Surgery—Micropha konit Microph akon it or bim an ual ph acoem u lsificat ion th rough t w o 0.7-m m in st rum en ts (an irrigat ing ch opper an d a ph aco t ip) can be u sed effect ively to t ackle a posterior polar cat aract . Hydrodelin eat ion can be don e th rough both por ts h ere. An oth er advan t age of th is tech n ique is th at on e can easily rever t to bim an ual vitrectom y in case of vit reou s loss. Th e advan tage of m icroph akon it over ph aco is th at on e h as a closed ch am ber th rough ou t su rger y becau se both th e in cision s are so sm all ( Fig. 7.1B).

Subluxated Cataract Sublu xated cataracts pose a risk of n u cleu s drop du ring cataract su rger y, an d h en ce requ ire sp ecial precau t ion s.

Presentation Th ere can be a zon u lar deh iscen ce or w eakn ess presen t .

Differential Diagnosis Colobom a of th e len s. Th ere can be colobom a w ith a su blu xat ion ( Fig. 7.2A).

A

B Fig. 7.2

(A) Subluxated colobomatous lens. (B) Aniridia rings being implanted. ([A] Courtesy of Lincoln L. Freitas)

220

Color Atlas of Ophthalm ology

Management Cat aract su rger y in th e presen ce of zon ular w eakn ess or a su blu xated len s is a great ch allenge. In th e past , su rgical in ter ven t ion in th ese cases w as difficu lt , leading to com plicat ion s. Th e u se of an en docapsular flexible polym ethyl m eth acr ylate (PMMA) ring h as ch anged th e su rgical approach to su blu xated cataracts. Im plan tat ion of a capsular ten sion ring (CTR) st abilizes a loose len s an d allow s th e surgeon to place th e in t raocu lar len s in th e m ost desirable p lace—th e capsular bag. Th ere are n u m erous oth er advan tages: vit reous h ern iat ion to th e an terior ch am ber is redu ced, a t aut capsu le gives coun tert ract ion to all t ract ion m an euvers m aking th em easier to perform , capsular su ppor t for an “in th e bag” im plan t is obtain ed, an d m ost im por tan tly, th e capsu lar bag m ain t ain s its sh ape, avoiding capsu lar fibrosis syn drom e an d in t raocu lar len s decen t rat ion . Do n ot use t r ypan blue in sublu xated cataracts because th e tr ypan blue w ill go into th e vit reous cavit y through th e zonular dehiscen ce an d m ake th e w hole vitreous cavit y blue. W h en zonular deh iscence is large in extent or progressive in n at ure, capsular bag shrin kage resulting in in traocular len s decen tration and pseudoph akodonesis m ay occur even after a successful surger y w ith a capsular ring. Com plete lu xation of the bag an d its con ten ts has also been reported. For such cases, Cionn i’s m odified design w ith a fixat ion h ook is a good solution . The h ook is kept in th e area of dialysis an d is pulled periph erally using a t ran sscleral fixation sut ure to counteract capsular bag decen trat ion an d tilt . In severe cases, t w o such rings or th e t w ohooked m odel can be used. An altern ative in cases of severe decentration is to m ake a sm all equatorial capsulorrhexis through w h ich a standard capsular tension ring can be in serted. A scleral sut ure can th en be passed around th e exposed capsular ten sion ring, w h ich is th en used to cen ter th e lens before capsulorrh exis. Peribulbar anesth esia is suitable for creat ion of scleral w indow s an d transscleral sut uring of th e capsular ring or of th e in traocular lens if n ecessar y. CTRs can also be placed to cover sector iris defects or colobom a. Th ese colobom a sh ields h ave an in tegrated 60- to 90-degree sector sh ield to protect again st glare an d m on ocu lar diplopia. More th an on e capsu lar ten sion ring can be used if m ore th an 90 degrees of defect is presen t . Mult isegm en ted colobom a rings are available for an iridia as w ell as for cases w ith large perm an en tly dilated pu pils secon dar y to any cause. In sert ion of t w o of th ese rings so th at th e in terspaces of th e first ring are covered by th e sector sh ields of th e secon d m akes a con t iguous ar t ificial iris possible ( Fig. 7.2B).

Miotic Pupil Cataract A w ell-dilated pu pil is th e gatew ay to a sm ooth , easy, an d rew arding cataract su rger y. Bu t th e su rgeon m ay n ot be lucky en ough to sail sm ooth ly th rough th e w ell-ch arted path of a dilated pu pil each t im e. Som et im es th e door looks n arrow an d un invit ing even for th e best . A m iot ic pu pil is a com m on bugbear th at ever y su rgeon faces at som e t im e.

Presentation A sm all pu pil affects all steps of ph acoem ulsificat ion , righ t from capsu lorrh exis to in t raocu lar len s in ser t ion . Difficult m an euvering cau ses iris dam age, sph in cter tears, zon u lar dialysis, bleeding, an d so on . Poor exposure th rough a sm all pu pil forces th e surgeon to m ake a sm aller rh exis, adding to th e difficu lt y an d frequen tly

7 Lens and Cataract

221

leading to capsu lar deh iscen ce an d n ucleus drop—th e w orst n igh t m are. Th e prolonged surgical t im e takes it s toll th ereafter. Corn eal edem a, uveit is, secon dar y glaucom a, cystoid m acu lar edem a, distor ted pu pil—th e list is en dless.

Differential Diagnosis Causes of sm all pup il

Management Sphincter-Spa ring Techniques Ph arm acological m ydriasis alon e m ay n ot be effect ive in cases w ith posterior syn ech iae, pu pillar y m em bran e, or scarred pupils. Su ch pu pils n eed in t raoperat ive procedu res su ch as h igh -m olecu lar-w eigh t coh esive viscoelast ics, syn ech iolysis, viscom ydriasis, an d/or pupillar y m em bran e st ripping. Sphincter-Involving Techniques Min isp h in cterotom ies (less th an 1 m m ) lim ited to th e sph in cter t issu e can be m ade w ith eith er Van ass scissors or th e vit reoret in al scissors. Dilat at ion can also be ach ieved by pu pillar y st retch ing u sing push -pull in st ru m en t s. Bip ronged, t ripronged, an d quadripronged pu pil st retch ers are also ver y effect ive. Oth er altern at ives are com m ercially available iris h ooks, pupil ring expan ders, an d th e Malyugin ring ( Fig. 7.3A,B).

A

Fig. 7.3 (A) Tri-pronged pupil stretchers. (B) Iris hooks inserted to enlarge the pupil.

B

222

Color Atlas of Ophthalm ology

Mature Cataract One of the biggest bugbears for a phaco surgeon is to perform a rhexis in a m ature cataract. Once one perform s rhexis in m ature and hyperm ature cataracts, then phaco can be done in these cases and a foldable intraocular lens can be im planted.

Presentation Th e solu t ion to m at u re cat aract s is to h ave a dye th at st ain s th e an terior capsu le. Th is dye is t r ypan blu e (Fig. 7.4A,B). On e can also use in docyan in e green .

Differential Diagnosis Ch eck for variou s causes of m at u re cataracts.

Management Th e problem w h en on e operates a m at ure cataract is th e creat ion of th e rh exis, w h ich can be solved u sing a dye. Th e oth er problem is surge. On e sh ou ld un derstan d th e w orking of a ph aco m ach in e for un derstan ding surge. W h en an occlu ded fragm en t is h eld by h igh vacu um an d th en abruptly aspirated, fluid ru sh es in to th e

A

B

Fig. 7.4 (A) Rhexis being done in a mature cataract with cystotome. (B) Rhexis forceps (MicroSurgical Technology, Redmond, WA) used to perform the rhexis in a m ature cataract. Note the trypan blue (Blurhex, Dr. Agarwal Pharm a Ltd., Chennai, India) staining the anterior capsule.

7 Lens and Cataract

223

ph aco t ip to equ ilibrate th e bu ilt-up vacu um in th e asp irat ion lin e, causing su rge. Th is leads to sh allow ing or collapse of th e an terior ch am ber. Differen t m ach in es em ploy a variet y of m eth ods to com bat surge. An oth er m eth od is th e air pu m p. An au tom ated air pu m p is used to pu sh air in to th e in fu sion bot tle, th us in creasing th e pressure w ith w h ich th e fluid flow s in to th e eye. Th is in creases th e steady-state pressure of th e eye, m aking th e an terior ch am ber deep an d w ell m ain tain ed during th e en t ire procedure. It m akes ph akon it an d ph acoem ulsificat ion a relat ively safe p rocedu re by reducing su rge even at h igh vacu um levels ( Figs. 7.5 , 7.6 , 7.7 , an d 7.8 ).

Fig. 7.5 The principles of how the phaco m achine works. This conceptual view shows the three main elements of m ost phaco systems. (1) The irrigation (red): Intraocular pressure is maintained and irrigation is provided by the bot tle of balanced salt solution (B) connected via tubing to the phaco handpiece (F). It is controlled by the surgeon. Irrigation enters the eye via an infusion port (H) located on the outer sleeve of the bi-tube phaco probe. Height of the bot tle above the eye is used to control the inflow pressure. (2) Aspiration (blue): (I) enters through the tip of the phaco probe, passes within the inner tube of the probe, travels through the aspiration tubing, and is controlled by the surgeon by way of a variable-speed pump (J). The peristaltic t ype pump is basically a motorized wheel exerting rotating external pressure on a portion of the flexible aspiration line, which physically forces fluid through the tubing. Varying the speed of the rotating pump controls the rate of aspiration. Aspirated fluid passes to a drain (l). (3) Ultrasonic energy (green) is provided to the probe tip via a connection (M) to the unit. All three of these m ain phaco functions are under control of the surgeon by way of a multicontrol foot pedal (N). (Courtesy of Benjamin F. Boyd, MD, FACS, Editor-in-Chief, “The Art and the Science of Cataract Surgery.” Highlights of Ophthalmology, English Edition, 2001.)

224

Color Atlas of Ophthalm ology

Fig. 7.6 Mechanism of the undesirable surge phenomenon. One problem area of the closed phaco system occurs during abrupt dislodging of an occluding piece of lens material so that it no longer occludes the aspiration port of the phaco tip. A sudden drop in intraocular pressure occurs as the fluid rate into the eye fails to imm ediately match the sudden fluid rate out of the eye. This is known as the surge phenomenon. (A) A piece of lens material occluding the aspiration port of the phaco tip is held in place by vacuum pressure created by the operating pump (D). (Note there is no drainage (E) from the blocked system.) Infusion from the irrigating bot tle (C) has ceased, but it is still providing controlled intraocular pressure due to its elevated position above the eye. With sufficient vacuum pressure from the pump and/or emulsification from the ultrasonic energy, the nuclear piece will abruptly enter the aspiration port and the fluid system will once again open (B). Because the plastic infusion/aspiration lines and the eye walls are flexible in absorbing the sudden inflow–outflow pressure differential, there occurs a moment when the infusion fluid (G, small arrow) does not effectively enter the eye fast enough to replace the fluid suddenly moving out of the unblocked system (F, large arrow). Outflow rate from the force of the pump is momentarily greater than the replacing infusion rate. This out-of-balance system (out of balance in not providing constant intraocular pressure) in which the eye m omentarily absorbs the inflow–outflow rate differential may traumatically collapse the eye for a short period. (Courtesy of Benjamin F. Boyd, MD, FACS, Editor-in-Chief, “The Art and the Science of Cataract Surgery.” Highlights of Ophthalmology, English Edition, 2001.)

7 Lens and Cataract

225

Fig. 7.7 Technical solution to prevent the undesirable surge phenomenon. One technical solution for elim inating the surge phenomenon involves the use of a high-tech microprocessor. When a nuclear piece (F) occludes the aspiration port and then suddenly (B) is aspirated (F, arrow) by the vacuum pressure of the pump (P), a sensor (E) located on the aspiration line signals a microprocessor (G) in the unit that an abrupt surge in aspiration flow has begun to take place. Within milliseconds, the microprocessor directs the motor of the pump (P) to slow down. The reduction in aspiration rate resulting from the slowed pump occurs before the eye can collapse from any volum e differential encountered bet ween sudden inflow and outflow rates. The potentially dangerous surge phenomenon is avoided. This elimination of the surge phenomenon allows the surgeon to safely use higher vacuum rates (necessary in some situations) with a reduction in the need to use potentially damaging high ultrasonic power set tings. Surgery becomes safer and faster. (Courtesy of Benjamin F. Boyd, MD, FACS, Editor-in-Chief, “The Art and the Science of Cataract Surgery.” Highlights of Ophthalmology, English Edition, 2001.)

226

Color Atlas of Ophthalm ology

Fig. 7.8 Diagrammatic representation of the connection of the air pump to the infusion bot tle.

Senile Cataract On e sh ould perform cat aract su rger y w ell in sen ile cataracts (Fig. 7.9 ).

Presentation Th e fu n dam en tal goal of Ph aco is to rem ove th e cat aract w ith m in im al dist urban ce to th e eye u sing th e least n um ber of su rgical m an ipulat ion s. Each m an euver sh ou ld be perform ed w ith m in im al force, an d m a xim al efficien cy sh ould be obtain ed.

Fig. 7.9 Rhexis being done in a case of senile cataract.

7 Lens and Cataract

227

Differential Diagnosis Oth er system ic causes of cataract

Management After viscoelast ic is injected in to th e eye th rough a 26-gauge n eedle, a globe stabilizat ion rod is in ser ted an d a clear corn eal in cision is m ade. On e can th en rem ove th e cataracts u sing th e divide an d con quer tech n iqu es. We prefer th e ch opping tech n ique ( Figs. 7.10 an d 7.11 ). Th e Ph aco probe is in ser ted th rough th e in cision sligh tly sup erior to th e cen ter of th e n u cleu s, an d th e ph aco t ip is em bedded in th e n u cleu s w ith th e t ip directed obliqu ely dow nw ard tow ard th e vit reou s an d n ot h orizon t ally tow ard th e iris. On ce th e t ip is em bedded, w h ile in foot posit ion 2, th e n u cleus is ch opped w ith a st raigh t dow nw ard m ot ion at th e en d of w h ich th e ch op per m oves to th e left on reach ing th e cen ter of th e n ucleus (i.e., like a laterally reversed L) ( Fig. 7.12 ). Th e n u cleu s is th en rot ated 180 degrees an d cracked again to get t w o h alves of th e n u cleu s. Th is is th en repeated to fu r th er ch op th e cataract in to sm aller pieces, w h ich are th en brough t in to th e an terior ch am ber on e by on e an d em ulsified. On ce th e n u cleus an d cor tex are rem oved th e foldable in t raocular len s is im plan ted ( Fig. 7.13 ).

Fig. 7.10 A diam ond knife blade (D) enters the first incision (1), the second tunnel incision (2), and is then directed slightly oblique to the iris plane and advanced (arrow) into the anterior chamber. This forms the internal aspect of the incision into the cham ber (A). This is the third step (3) in the three -step self-sealing incision. (Courtesy of Benjamin F. Boyd, MD, FACS, Editor-in-Chief, “The Art and the Science of Cataract Surgery.” Highlights of Ophthalmology, English Edition, 2001.)

228

Color Atlas of Ophthalm ology

Fig. 7.11 Emulsification of lens fragments: This surgeon’s view shows the manage ment of the lens quadrants. The apex of each of the four loose quadrants is lifted with the second instrument (S), the ultrasound phaco tip (P) is em bedded into the posterior edge of each, and by m eans of aspiration the surgeon centralizes each quadrant for emulsification. U.S., ultrasound; Asp., aspiration flow rate; Vac., vacuum; C, capsule; F, fragment. (Courtesy of Benjamin F. Boyd, MD, FACS, Editor-in-Chief, “The Art and the Science of Cataract Surgery.” Highlights of Ophthalmology, English Edition, 2001.)

Fig. 7.12 This cross-section view shows the phacoemulsification probe removing the nucleus fragm ents within the capsular bag. Note the apex of one of the fragments created in the nucleus being lifted with the second instrument (arrow) and the ultrasound tip embedded into the posterior edge of each segm ent ready for emulsification. The epinucleus and cortex will then be removed during the phaco process. If we operate on a softer cataract, the freed fractured pieces are emulsified immediately. (Courtesy of Benjamin F. Boyd, MD, FACS, Editor-in-Chief, “The Art and the Science of Cataract Surgery.” Highlights of Ophthalmology, English Edition, 2001.)

7 Lens and Cataract

229

Fig. 7.13 This cross-section view shows the movement of the foldable intraocular lens during insertion. Folding forceps removed for clarit y. (1) Folded lens outside the eye. (2) Folded lens passing through small incision. (3) Folded lens placed posteriorly into the capsular bag through anterior capsule opening and then rotated 90 degrees. (4) Lens slowly unfolded in the bag. (5) Final unfolded position of lens within the capsular bag. (Courtesy of Benjamin F. Boyd, MD, FACS, Editor-in-Chief, “The Art and the Science of Cataract Surgery.” Highlights of Ophthalmology, English Edition, 2001.)

Glued Intraocular Lens In t raocu lar len s (IOL) im plan t at ion in eyes th at lack posterior capsular su ppor t h as been accom plish ed in th e past by m ean s of an iris-fixated IOL, an terior ch am ber IOL, an d t ran sscleral IOL fixat ion th rough th e ciliar y su lcus or pars plan a. Su rgical exper t ise, prolonged su rgical t im e, su t u re-in du ced in flam m at ion , su t u re degradat ion , an d delayed IOL sublu xat ion or dislocat ion due to broken su t u re are som e of th e lim itat ion s in sut u red scleral fixated IOLs. It is possible to p lace a posterior ch am ber IOL in eyes w ith a deficien t posterior capsule using a glu ed IOL tech n iqu e.

Technique After clam ping th e su perior rect us, an in fu sion can n u la or an terior ch am ber m ain tain er is in serted, preferably in th e in feron asal quadran t to preven t in terferen ce in creat ing th e scleral flap s, w h ich is th e n ext step in th e surger y. Tw o part ial-th ickn ess lim bal-based scleral flaps ~2.5 × 3.0 m m are th en created exactly 180 degrees

230

Color Atlas of Ophthalm ology

B

A Fig. 7.14 (A) Scleral flaps (sf) of 2.5 x 3.0 mm made ~ 1.5 m m from the limbus. (B) Two flaps are made exactly 180 degrees diagonally apart.

diagon ally apar t ( Fig. 7.14 ) up to th e lim bus. Th is is follow ed by vit rectom y via a pars plan a or an terior route to rem ove all vit reous t ract ion . Tw o st raigh t sclerotom ies w ith a 22-gauge n eedle are m ade ~1.5 m m from th e lim bu s un der th e exist ing scleral flaps. A corn eo-scleral or scleral t u n n el in cision is th en prepared for in t rodu cing th e IOL in secon dar y IOL im plan tat ion . W h ile th e IOL is being in t rodu ced w ith on e h an d u sing a McPh erson forceps, an en d-gripping 23-gauge MST m icro rh exis forceps (MicroSurgical Tech n ology, Redm on d, WA) is passed th rough th e in ferior sclerotom y w ith th e oth er h an d. Th e t ip of th e leading h apt ic is th en grasped w ith th e m icro rh exis forceps, pulled th rough th e in ferior sclerotom y follow ing th e cu r ve of th e h apt ic (Figs. 7.15 an d 7.16 ), an d extern alized un der th e in ferior scleral flap. Sim ilarly, th e t railing h apt ic is also extern alized th rough th e su perior sclerotom y u n der th e scleral flap. Scleral t u n n els are m ade w ith a 26-gauge n eedle at th e edge of th e scleral flap an d th e h apt ics are th en t u cked in to th ese scleral t u n n els for addit ion al stabilit y of th e IOL (Fig. 7.17). Recon st it u ted fibrin glu e is th en injected th rough th e can n ula of th e dou ble-syringe deliver y system un der th e su perior ( Fig. 7.18 ) an d in ferior scleral flap s. Local pressure is given over th e flap s for ~10 to 20 secon ds for th e form at ion of fibrin polypept ides. In pat ien t s w h o h ave a lu xated IOL, sim ilar lam ellar scleral flaps as

A

B Fig. 7.15 (A) The IOL is inserted with a McPherson forceps, and the leading haptic is grasped by an end-gripping 23-gauge micro rhexis forceps (f) passed through a scle rotomy wound. The haptic is then externalised under the scleral flap (sf). (B) The trailing haptic is also externalized in a similar manner.

7 Lens and Cataract

231

A

B Fig. 7.16 (A) One haptic of the IOL is grasped by an end-gripping 23-gauge micro rhexis forceps (f) passed through sclerotomy wound. The haptic (h) is then externalised under the scleral flap (sf). (B) The second haptic is also externalized in a similar manner.

Fig. 7.17 (A) The haptics are externalized and a 26-gauge needle is taken to create a scleral tunnel. (B) 26-gauge needle is seen making a scleral tunnel near the edge of the flap, parallel to the limbus. (C) The haptic is tucked into the tunnel made with the 26gauge needle. This provides it additional stabilit y.

A

B Fig. 7.18 (A) Reconstituted fibrinogen and thrombin preparation of fibrin glue (FG) injected beneath the scleral flaps. (B) Scleral flaps (arrow) sealed well with the scleral bed.

232

Color Atlas of Ophthalm ology

Fig. 7.19 Conjunctiva closed with fibrin glue.

described earlier are m ade an d th e lu xated IOL h apt ic is th en grasped w ith th e 23gauge m icro rh exis forceps an d extern alized, t ucked in to th e scleral t u n n el m ade at th e edge of th e flaps, an d th en glu ed un der th e scleral flaps. Th e in fu sion can n ula is th en rem oved. Conjun ct iva is also closed w ith th e sam e fibrin glu e ( Fig. 7.19 ). Th is tech n ique is u seful in a m yriad of clin ical sit uat ion s w h ere scleral-fixated IOLs are in dicated, such as a lu xated IOL, dislocated IOL, zon ulop athy, or secon dar y IOL im plan t at ion . In dislocated posterior ch am ber polym ethyl m eth acr ylate (PMMA) IOL, th e sam e IOL can be reposit ion ed, th ereby redu cing th e n eed for furth er m an ipu lat ion . It can be perform ed w ell w ith a rigid PMMA IOL, IOLs w ith m odified PMMA h apt ics, or m u lt ifocal glued IOLs. On e th erefore does n ot n eed to h ave special scleral fixated IOLs w ith eyelet s or n ew er h apt ic design s. Becau se

Fig. 7.20 Im age of the scleral flap as seen by anterior segment optical coherence tomography on day 1 (above) and well sealed scleral flaps at 6 weeks (below).

7 Lens and Cataract

233

Fig. 7.21 Postoperative anterior segm ent optical coherence tomography showing 360 degrees well-centered intraocular lens at 6 weeks.

th e h apt ic is being placed in its n orm al cur ved con figu rat ion w ith ou t any t ract ion , th ere is n o distort ion or ch ange in sh ape of th e IOL opt ic. Extern alizat ion of th e greater par t of th e h apt ics along it s cu r vat u re stabilizes th e a xial posit ion ing of th e IOL an d th ereby preven ts any IOL t ilt ( Figs. 7.20 an d 7.21 ).

Temporary Haptic Externalization On e of th e m ost difficult steps of reposit ion ing a dislocated posterior ch am ber IOL is secu ring a sut u re on th e h apt ics. In 1992, Clem en t Ch an first in t roduced th e con cept of tem porar y h apt ic extern alizat ion to en h an ce th e ease of su t ure placem en t an d ch anged IOL reposit ion ing from an un con t rolled to a h igh ly con t rolled set t ing th at allow s fixat ion of th e dislocated IOL in th e ciliar y su lcu s on a con sisten t basis ( Fig. 7.22 ). A th ree-port pars p lan a vit rectom y is perform ed for th e rem oval of th e an terior an d cen t ral vit reou s adjacen t to th e dislocated IOL to p reven t any vit reoret in al t ract ion during th e process of m an ipu lat ing th e IOL. Tw o diam et rically opposed lim bal-based part ial-th ickn ess t riangu lar scleral flaps are prepared along th e h orizon tal m eridian s at 3 an d 9 o’clock. Cau terizat ion is don e to preven t any bleeding An terior sclerotom ies w ith in th e beds un der th e scleral flaps are m ade at 1 to 1.5 m m from th e lim bus. As an altern at ive to th e scleral flaps, th e an terior sclerotom ies m ay be m ade w ith in th e scleral grooves at 1.0 to 1.5 m m from th e h orizon tal lim bu s. A fiberopt ic ligh t pipe is in serted th rough on e of th e posterior sclerotom ies, w h ile a pair of fin e, n on angled posit ive-act ion forceps (e.g., Griesh aber 612.8, Alcon Griesh aber, Ltd., Sch affh au sen , SZ) is in ser ted to h old th e IOL an d bring it an teriorly. A forceps is th en passed th rough th e an terior sclerotom y of th e oppos-

234

Color Atlas of Ophthalm ology

B

A

C Fig. 7.22 (A) A forceps is passed through a sclerotomy made under a scleral flap in the opposite quadrant and one haptic of the dislocated IOL is caught for temporary externalization. (B) A double -armed 9–0 or 10–0 polypropylene suture is tied around the externalized haptic to make a secured knot and it is re -introduced into the vitreous cavit y. The same process is then repeated for the other haptic. (C) The internalized haptics are anchored securely in the ciliary sulcus by taking scleral bites with the external suture needles.

ing quadran t to engage on e h apt ic of th e dislocated IOL for th e tem porar y extern alizat ion . A dou ble-arm ed 9–0 (Eth icon TG 160–8 plus, Som er ville, NJ) or 10–0 polypropylen e sut u re (Eth icon CS 160–6, Som er ville, NJ) is t ied arou n d th e extern alized h apt ic to m ake a secured kn ot . Th e sam e process is repeated for th e oth er h apt ic after th e su rgeon sw itch es th e in st ru m en ts to th e opposite h an ds. Th e extern alized h apt ics w ith th e t ied sut u res are rein tern alized th rough th e corresp on ding an terior sclerotom ies w ith th e sam e forceps. Th e in tern alized h apt ics are an ch ored securely in th e ciliar y sulcus by taking scleral bites w ith th e extern al su t u re n eedles on th e lips of th e an terior sclerotom ies. By adjust ing th e ten sion of th e op posing sut u res w h ile t ying th e polypropylen e sut u re kn ot s by th e an terior sclerotom ies, th e opt ic is cen tered beh in d th e p upil, an d th e h apt ics are an ch ored in th e ciliar y sulcus.

Intraoperative Complications Posterior Capsular Rupture If a capsular tear does occu r, several steps can h elp m in im ize vit reou s loss. A closed system sh ould be m ain t ain ed by inject ing viscoelast ic before w ith draw ing th e ph aco t ip. Th is h elps to tam pon ade th e vit reou s backw ard w h ere a capsu lar deh iscen ce is presen t . Cor t ical rem oval sh ould th en proceed w ith eith er low in fusion or by a dr y tech n ique filling th e ch am ber w ith viscoelast ic m aterial an d us-

7 Lens and Cataract

235

A

B Fig. 7.23 (A) Bimanual vitrectomy being done in a case after posterior capsular rupture and vitreous loss. One should never do a coaxial vitrectomy. (B) Case of a dropped intraocular lens and nucleus (see the white reflex) after a posterior capsular rupture.

ing a Sim coe aspirator. If th e hyaloid face is broken an d vit reou s presen ts th rough th e ru pt u re, a rout in e t w o-por t m ech an ized vit rectom y using an Accur us Vit rector (Accuru s 800 CS, Alcon Laboratories, For t Worth , TX) using low -flow an d low vacu um param eters is perform ed. Th e tear can th en be converted in to a roun d an d stable open ing. Th e IOL can th en be safely placed in to th e capsu lar bag. An u n con t rolled tear n ecessitates im plan t at ion in to th e ciliar y su lcu s. A 6-m m opt ic acr ylic len s is preferred w h en im plan t ing in th e bag as it un folds slow ly, th us causing less st ress on th e posterior capsu le. Th e leading edge of th e len s sh ould be directed aw ay from th e area of a w eak capsu le. On e can develop a dropped IOL an d n ucleu s also ( Fig. 7.23A,B).

Iridodialysis On e can develop an iridodialysis, w h ich w ill requ ire su t u ring (Fig. 7.24 ).

Fig. 7.24

Iridodialysis.

236

Color Atlas of Ophthalm ology

Fig. 7.25

Torn intraocular lens.

Torn Intraocular Lens An IOL can get torn during im plan t at ion . In such a case it sh ould be explan ted an d replaced ( Fig. 7.25 ).

Postoperative Complications Corneal Edema after Cataract Extraction Corn eal edem a after a cataract surger y can be iden t ified im m ediately after th e cataract su rger y. It is best appreciated a few h ou rs after th e cataract surger y. Most com m on causes th at lead to epith elial or st rom al edem a after th e cataract surger y are m ech an ical t rau m a, prolonged in t raocular irrigat ion , in flam m at ion , in creased in t raocular pressu re (IOP), Descem et m em bran e det ach m en t , in t raocu lar toxin s, an d a com p licated cataract su rger y.

Presentation An associated en doth elial abn orm alit y or decom p en sat ion h as to be ruled out preoperat ively. Th ere is an associated in crease in th e corn eal th ickn ess. An in creased epith elial th ickn ess w ith associated h azy corn ea, irregu lar surface, an d epith elial defect form th e h allm arks of epith elial edem a. Th e th ickn ess ret urn s to n orm al after a few h ou rs of su rger y. St rom al edem a is associated w ith a m arked in crease in th e corn eal th ickn ess, corn eal h aze, an d en doth elial folds involving m ain ly th e cen ter of th e corn ea ( Fig . 7.26 ). Brow n -McLean syn drom e, a clin ical con dit ion arising after cat aract surger y, is ch aracterized by periph eral corn eal edem a w ith a clear cen ter.

7 Lens and Cataract

237

A

Fig. 7.26 Postoperative striate striate keratitis.

Management As a r ule, if th e corn eal p eriph er y is clear, th e corn eal edem a reduces in a few h ou rs. Cases w ith epith elial edem a requ ire a m edical reduct ion of IOP if th e edem a does n ot resolve in a few h ou rs. A st rom al corn eal edem a p ersist ing for a few days is requ ired to be m an aged w ith hyper ton ic eyedrops an d steroids. A vit rectom y is in dicated in pat ien t s w ith exist ing vit reocorn eal adh eren ce w ith associated corn eal edem a. A Descem et m em bran e detach m en t can be h an dled by inject ing air or gas (C3 F8 or SF6 ). Edem a exist ing for m ore th an 3 m on th s u sually requ ires a p en et rat ing keratoplast y.

Hypotony and Wound Leak after Cataract Extraction Hyp otony due to w ou n d leak after cataract su rger y is a com m on com plicat ion of th e cat aract surger y. Th e leakage can occur from th e in cision site, th e side por t , or th e bleb in cases w ith th e com bin ed t rabecu lectom y su rger y.

Presentation Wou n d leakage can be picked u p on slit lam p an d by em ploying a Siedel test . Mild to m oderate leaks presen t w ith a sh allow an terior ch am ber, leaking w oun d, irrigat ion , tearing, con t act len s in toleran ce, in fect ion , an d sign ifican t hypotony. Severe leaks m ay be associated w ith iridocorn eal touch w ith severe corn eal edem a.

B

238

Color Atlas of Ophthalm ology

Management Most of th e leaks are self-lim it ing an d requ ire obser vat ion w ith patch ing an d use of topical an d system ic aqu eou s suppressan ts. Tem porar y redu ct ion of th e topical steroids h elps in prom pter h ealing by in creasing in flam m at ion . In ch ron ic cases use of cyan oacr ylate glu e, sim ple w oun d sut uring, an d an terior ch am ber reform at ion w ith salin e or air follow ed by w ou n d sut u ring h elps.

Elevated IOP after Cataract Extraction An IOP fluct uat ion im m ediately after cataract surger y is a com m on bu t self-lim it ing p roblem in m ost of th e cases. Reten t ion of viscoelast ic m aterial in th e eye du ring th e su rger y is on e of th e m ost com m on cau ses of p ostop erat ive in flam m at ion an d IOP rise. Oth er cau ses of in creased IOP after cat aract su rger y in clude pupillar y block, hyph em a, ciliar y block, en doph th alm it is, retain ed len s m aterial, iris pigm en t release, preexist ing glaucom a, use of steroids, an d periph eral an terior syn ech iae.

Presentation Th e pat ien t u sually com plain s of h azy vision an d pain . Hazy corn eas du e to epith elial or st rom al edem a an d associated an terior ch am ber in flam m at ion are com m on .

Management Most of th e cases are m ild an d self-lim it ing an d requ ire on ly m on itoring. A rise in IOP n oted in t raoperat ively can be m an aged by depressing th e low er lip of th e paracen tesis site to evacuate som e of th e fluid out of th e eye. How ever, a sign ifican t an d su stain ed rise in IOP m ay n ecessitate t im ely an d specific m an agem en t of several circu m stan ces. Secon dar y glau com as are h an dled by t reat ing th e u n derlying cau se.

Cystoid Macular Edema after Cataract Extraction Cystoid m acu lar edem a (CME) is a pain less con dit ion in w h ich cyst ic sw elling or th icken ing occurs of th e cen t ral ret in a (m acu la) an d is u su ally associated w ith blurred or distor ted vision . W h en CME develops follow ing cat aract su rger y an d its cau se is th ough t to be directly related to th e surger y, it is referred to as Ir vin e- Gass syn drom e. Th e Mü ller cells in th e ret in a becom e over w h elm ed w ith fluid, leading to th eir lysis. Th is result s in an accu m ulat ion of fluid in th e ou ter plexiform an d in n er n u clear layers of th e ret in a. Usual cau ses of CME are vascu lar in st abilit y, in t raocular in flam m at ion , an d m ech an ical forces (e.g., epiret in al m em bran e, vitreom acu lar t ract ion ). Th e in ciden ce of CME is 1% after ph acoem u lsificat ion an d 20% after ext ra-capsular cat aract ext ract ion (ECCE).

Presentation Pat ien ts w ith CME u sually presen t w ith decreased or blurr y vision . Slit-lam p biom icroscopy reveals blun ted or irregu lar foveal ligh t reflex, ret in al th icken ing, an d/ or in t raret in al cyst s in th e foveal region . Opt ic disk edem a, epiret in al m em bran e/ m acu lar pu cker, diabet ic ret in opathy, an d uveit is m u st be looked for to rule out oth er causes. On fu n dus fluorescein angiograp hy a pet aloid pat tern of leakage in th e m acu la occurs. Opt ical coh eren ce tom ography (OCT) is h elpful in establish ing a diagn osis an d in m easu ring th e th erapeut ic respon se (Fig. 7.27 ).

7 Lens and Cataract

239

A

B

C

D Fig. 7.27 (A) Color photograph of the fundus shows an absent foveal reflex with cystoid edema. (B) Fundus fluorescein angiography shows a characteristic patelloid pat tern of hyperfluorescence in the early venous phase, which increases in intensit y in the later phases (C,D) of the angiogram.

Differential Diagnosis Diabet ic m acu lar edem a, ret in it is pigm en tosa, juven ile ret in osch isis, vit reom acular t ract ion syn drom e, epiret in al m em bran e, Goldm an n -Favre syn drom e, m acu lar cyst , an d foveal sch isis in h igh m yop es

Management Treat m en t is aim ed at th e u n derlying et iology; h ow ever, several of th e com m on t reat m en ts m ay h elp differen t cau ses of CME. Medical t reat m en t m odalit ies in clude cor t icosteroids (topical, oral, in t ravit real, or in th e su b-Ten on sp ace), n on steroidal an t iin flam m ator y drugs, carbon ic an hydrase in h ibitors, an d YAG laser lysis of th e vit reou s st ran ds. Surgical th erapy in clu des pars p lan a vit rectom y to rem ove vit reou s st ran ds t racking to th e surgical w ou n d or pup il st at u s after com p licated ocular su rger y, peeling of th e p osterior hyaloid face from th e su rface of th e m acula in vit reom acular t ract ion syn drom e, peeling of th e ep iret in al m em bran es, rem oval of in flam m ator y m ediators from th e vit reou s cavit y, rem oval of retain ed n u clear len s fragm en ts, an d reposit ion ing of a dislocated or sublu xed IOL.

Retinal Detachment, Suprachoroidal Hemorrhage/ Effusion after Cataract Extraction In t raocu lar su rger y is a m ajor risk factor in th e developm en t of rh egm atogen ou s ret in al det ach m en t (RRD). Becau se cataract surger y is th e m ost com m on in t raocu lar procedu re, it is also th e m ost com m on risk factor for RRD. It h as been est im ated

240

Color Atlas of Ophthalm ology

Fig. 7.28 ment.

Aphakic retinal detach-

th at 20 to 40% of RRDs occur in eyes th at h ave un dergon e cataract ext ract ion . Aph akia an d p seu doph akia, especially after YAG capsulotom y, predispose to p osterior vit reou s det ach m en t (PVD). Previou s st u dies h ave sh ow n th at th e in ciden ce of PVD in creased w ith age an d w ith durat ion of th e aph akia. In pseudoph akic pat ien t s, perip h eral capsu lar op acificat ion , len t icular rem n an t s, an d opt ical effects in duced by th e rim of th e IOL m ay im pair visu alizat ion of th e sm all periph eral ret in al breaks by in direct oph th alm oscopy, leading to m issed breaks during su rgical repair.

Presentation Because m ost postoperat ive ret in al detach m en ts are rh egm atogen ous in n at u re, sim ilar sym ptom s, such as ph otopsias, floaters, visual-field defects, an d cen t ral visu al loss are experien ced by p at ien t s. Th e ret in al breaks are often sm all, difficu lt to visu alize, an d located along th e posterior border of th e vit reous base. RRD is often exten sive an d com m on ly involves th e m acu la. Th e m ost im p or tan t p ostop erat ive factor is YAG capsu lotom y. An terior ch am ber IOL an d iris clip len ses in duce m ore in flam m at ion , resu lt ing in a h igh er in ciden ce of p roliferat ive vit reoret in opathy ( Fig. 7.28 ).

Differential Diagnosis IOL dislocat ion , lat t ice degen erat ion , an d oth er t ypes of ret in al detach m en t

Management B-scan u lt rasou n d is th e m ain invest igat ion . As w ith all RRDs, th e goal is to iden t ify an d close all th e ret in al breaks. Vit rectom y w ith fluid-air exch ange, vit rectom y an d scleral bu ckling, an d pn eum at ic ret in opexy are som e of th e t reat m en t m odalit ies.

Endophthalmitis after Cataract Extraction Postoperat ive en doph th alm it is is defin ed as severe in flam m at ion involving both th e an terior an d p osterior segm en t s of th e eye after in t raocu lar surger y. Typically, postoperat ive en doph th alm it is is cau sed by th e perioperat ive in t rodu ct ion of m i-

7 Lens and Cataract

241

crobial organ ism s in to th e eye eith er from th e pat ien t’s n orm al conjun ct ival an d skin flora or from con tam in ated in st ru m en t s. Alth ough m ost cases of postoperat ive en doph th alm it is occur w ith in 6 w eeks of su rger y, in fect ion s seen in h igh -risk pat ien ts or in fect ion s caused by slow -grow ing organ ism s m ay occur m on th s or years after th e procedu re.

Presentation Pat ien ts w ith acute postoperat ive en doph th alm it is t ypically p resen t w ith in 6 w eeks of in t raocu lar su rger y w ith m oderate to severe eye pain an d decreased vision . Th e h allm ark fin dings on oph th alm ic exam in at ion are posterior an d an terior ch am ber in flam m at ion , hypopyon , conju n ct ival hyperem ia an d ch em osis, corn eal edem a, w oun d abn orm alit ies, an d associated eyelid or orbit al in flam m at ion . In rare circu m st an ces, pat ien ts m ay develop ch ron ic, in fect ious en doph th alm it is m on th s to years after in t raocu lar surger y ( Fig. 7.29 ). Risk factors for developm en t of postoperat ive en doph th alm it is m ay in clu de in creased operat ive t im e, posterior capsu le ru pt u re/vit reous loss, ret ain ed len s fragm en t s, in adequate sterilizat ion of th e operat ive field, an d con t am in at ion of su rgical in st ru m en ts. In th e en doph th alm it is vit rectom y st u dy, th e m ost com m on organ ism s isolated w ere coagu lase-n egat ive staphylococci (70%), Staphylococcus aureus (9.9%), an d st reptococci species (9.0%). In fect ion s cau sed by gram -n egat ive organ ism s w ere seen in 6% of cases. In ch ron ic postop erat ive en doph th alm it is, an im port an t causat ive organ ism is Propionibacterium acnes, a slow -grow ing, gram posit ive bacillu s th at is associated w ith a ch aracterist ic w h ite, in t racapsu lar plaqu e th at develops w eeks to m on th s after cataract su rger y.

Fig. 7.29 Postoperative endophthalmitis with corneal melt and intraocular lens extrusion.

242

Color Atlas of Ophthalm ology

Differential Diagnosis En dogen ou s en doph th alm it is, in t raocu lar foreign body, vit reou s h em orrh age, glaucom a (len s p ar t icle, ph acolyt ic, ph acom orph ic, an d uveit is), an d vit reou s w ick syn drom e

Management Obt ain vit reou s, aqu eou s sam p les an d conju n ct ival cu lt u res for m icrobiological id en t ificat ion of t h e offen d ing organ ism . B-scan is d on e to con fir m t h e d iagn osis. Th e resu lt s of an en d op h t h alm it is vit rectom y st u dy d em on st rated n o d ifferen ce in fin al visu al ou tcom es in p at ien t s w h o u n d er w en t in it ial in t raocu lar an t ibiot ic inject ion (vit reou s t ap ) or im m ed iate p ars p lan a vit rectom y (vit rectom y) if p resen t ing visu al acu it y w as bet ter t h an ligh t p ercept ion . How ever, in p at ien t s p resen t ing w it h ligh t p ercept ion vision , t h ose w h o u n d er w en t in it ial VIT w ere t h ree t im es m ore likely to ach ieve 20/40 vision , t w ice as likely to m ain t ain 20/100 vision , an d h ad a n early 50% red u ct ion in t h e r isk of severe visu al loss ( 20/40), but m etam orph opsia an d scotom a m ay rem ain .

Differential Diagnosis Blun t ocu lar t rau m a, RP

Management Th ere is n o t reat m en t . Th e p at ien t is follow ed u p.

10 Surgical Retina Clem ent K. Chan and Dariusz G. Tarasew icz

Developmental Anomalies and Degenerations Cystoid Degeneration Reticular Cystoid Degeneration Ret icular cystoid degen erat ion is a t ype of periph eral ret in al cystoid degen erat ion th at h igh ly correlates w ith age. It occurs in approxim ately 18% of adult pat ien t s an d can be bilateral m ore th an 40% of th e t im e. It is n ot as com m on as t ypical cystoid degen erat ion . It can be radially orien ted an d often follow s th e cou rse of ret in al vessels. Frequen tly, it is fou n d posterior to t ypical cystoid degen erat ion .

Presentation It is asym ptom at ic. A st ip p led ap p earan ce to t h e in n er ret in al su r face is com m on . Ret icu lar cystoid d egen erat ion is t yp ically fou n d in t h e in fer ior tem p oral qu ad ran t .

Differential Diagnosis Oth er periph eral ret in al degen erat ion s

Management Th orough oph th alm oscopic exam in at ion is requ ired. Man agem en t con sist s of obser vat ion on ly.

Typical Cystoid Degeneration Typical cystoid degen erat ion is th e m ost com m on periph eral ret in al degen erat ion . Spaces form in th e outer plexiform an d in n er n uclear layer an d coalesce to form t u n n els. W h ere t u n n els h ave n ot form ed, ret in al “pillars” rem ain . Th ese fin dings occur as early as age 1. It is fou n d in alm ost all eyes exam in ed. Typical cystoid degen erat ion is m ore com m on in th e su perior an d tem poral qu adran ts.

Presentation No sym ptom s are n ot able. Th e in n er ret in al su r face acqu ires a st ip p led ap p earan ce. Pillars cau se d ep ression s on t h e su r face, w h ereas d om elike areas rep resen t cystoid sp aces. Th e d egen erat ion alw ays begin s at t h e ora ser rat a an d sp read s p oster iorly.

Differential Diagnosis Oth er periph eral ret in al degen erat ion s

288

10 Surgical Retina

289

Management Th orough oph th alm oscopic exam in at ion is requ ired. Man agem en t con sist s of obser vat ion on ly.

Meridional Folds, Complexes, and Other Peripheral Lesions A m eridion al fold is a redu n dan cy of th e periph eral ret in a at th e border of th e ora serrata. It is m ost com m on ly foun d in th e supran asal qu adran t an d is bilateral for 55% of th e cases. Its length can be variable. It h as been est im ated th at 26% of th e popu lat ion h as m eridion al folds. W h en a m eridion al fold is con t igu ou s w ith a den t ate process, it is called a m eridion al com p lex. A m eridion al com plex can exten d to th e pars plan a ( Fig. 10.1A). An en closed oral bay (EOB) is a sm all islan d of n onpigm en ted ciliar y body ep ith elium th at h as becom e isolated an d surroun ded by n eurosen sor y ret in a. Clin ically, it m ay resem ble a ret in al break ( Fig. 10.1B).

Presentation Both th e m eridion al folds an d th e EOB are in ciden tal fin dings in n orm al eyes an d are u su ally n ot associated w ith any sym ptom s. Th e locat ion of m eridion al folds m ay aid th eir diagn osis. Th ey are alw ays close to th e ora serrata. Th ey are alm ost n ever associated w ith ret in al breaks.

Differential Diagnosis A ret in al break is a differen t ial diagn osis for EOB. Scleral in den tat ion sh ow s gradually slop ing edges un like th e sh arp an d abru pt ch ange in ret in al breaks. Also th e color in th e oral bay correspon ds to th e adjacen t pars plan a.

Management Careful oph th alm oscopy w ill differen t iate EOB from t ru e ret in al breaks. Th e color w ith in th e EOB is th e sam e as th e pars plan a. A m eridion al fold is frequ en tly located in th e sam e m eridian as an EOB. Un like ret in al breaks, th ese lesion s do n ot progress rapidly over t im e. Th ere is n o role for prophylact ic t reat m en t .

A

B Fig. 10.1

(A) Meridional fold complex. ( B) Enclosed oral bay.

290

Color Atlas of Ophthalm ology

Pars Plana Cyst Pars p lan a cyst s are convex, cystoid lesion s fou n d an terior to th e ora serrat a. Th ey are often adjacen t to oral bays an d can be m ult iple. Th e exact et iology is u n kn ow n , but th ey are probably th e result of a degen erat ive p rocess. Microscopically th ey represen t a separat ion of th e n onpigm en ted from th e pigm en ted epith elial cell layers of th e pars plan a.

Presentation Pars plan a cysts are asym ptom at ic. An au topsy series fou n d th eir presen ce in 16 to 18% of cases. Th ey are m ore frequen tly fou n d on th e tem poral side. Th ere is n o associat ion w ith oth er eye diseases.

Differential diagnosis Non e

Management Th orough op h t h alm oscop ic exam in at ion reveals t h e cyst . No t reat m en t is in d icated .

Pars Plana Pearls Pars plan a pearls are st riking, glisten ing w h ite lesion s fou n d in th e far an terior ret in al periph er y. Th ey are alw ays ben eath a den tate process an d represen t dr usen -like deposit s on th e in n er surface of th e Bru ch m em bran e.

Presentation Pars plan a pearls ser ve as good lan dm arks in th e far periph eral ret in a. Th ey are alw ays located ben eath a den tate process. Th ey are drusen -like st ru ct u res on th e Bruch m em bran e.

Differential Diagnosis Oth er periph eral ret in al degen erat ion s

Management Pars plan a pearls h ave been fou n d in 20% of au topsy subject s an d appear in all age groups. Th ere are n o kn ow n associat ion s w ith oth er eye path ologies. Th orough oph th alm oscopic exam in at ion is requ ired. No t reat m en t is in dicated.

Pavingstone Degeneration Also com m on ly term ed cobblestone degenerat ion, th ese are discrete foci of ou ter ret in al at rophy th at st art at th e ora an d progress posteriorly. It h as been est im ated th at th ese lesion s occur as early as age 8. Histologically th ey are ch aracterized by loss of rods, ret in al pigm en t epith elium (RPE), an d th e un derlying ch oriocapillaris ( Fig. 10.2 ).

10 Surgical Retina

Fig. 10.2 tion.

291

Pavingstone degenera-

Presentation In addit ion to th e outer ret in al at rophy, th ese lesion s sh ow prom in en t ch oroidal vessels an d are easy to see on in direct oph th alm oscopy. Th ey can h ave a yellow ish h ue, an d pigm en ted borders are com m on . Th ese lesion s ten d to clu ster on th e in ferior ret in a.

Differential Diagnosis Oth er periph eral ret in al degen erat ion s

Management Th orough op h th alm oscopic exam in at ion is required. Th ese lesion s are n ot associated w ith any sym ptom s or risk for ret in al det ach m en t (RD). Man agem en t con sist s of obser vat ion on ly; n o t reat m en t is in dicated.

Senile Tapetochoroidal Degeneration Tapetoch oroidal degen erat ion represen t s a pron oun ced degree of depigm en t at ion in th e periph eral ret in a. It is cau sed by a loss of pigm en t gran ules in th e RPE, usu ally m ore pron ou n ced w ith advan cing age.

Presentation Exam in at ion reveals a circum feren t ial ban d run n ing from th e ora serrata to th e equ ator.

Differential Diagnosis Oth er periph eral ret in al degen erat ion s

Management Tapetoch oroidal degen erat ion is foun d in 20%of pat ien t s older th an 40 an d is bilateral in all cases. Th orough oph th alm oscopic exam in at ion is requ ired.

292

Color Atlas of Ophthalm ology

Lattice Degeneration, Retinal Tufts, and Retinoschisis Lattice Degeneration Lat t ice degeneration is a periph eral retin al pathology that carries a m oderate risk for a retinal detach m en t. It h as been estim ated th at lat tice degen eration is foun d in only 6 to 8% of the gen eral population . It is bilateral in up to 48% of n on selected patients. Mult iple st udies h ave show n, h ow ever, that 20 to 30% of patients w ith a retin al detachm ent h ave lat tice degen eration. Lat t ice degeneration h as been associated w ith m ultiple nam es, including sn ail-track degen erat ion an d retinal and vitreous base excavation s, am ong others. Th ere is a h igh correlation bet w een lat tice degen eration an d m yopia. No defin itive h ereditar y pat tern has been establish ed.

Presentation A h allm ark feat u re of lat t ice is its variabilit y in presen t at ion . Classically it is described as a patch of ret in al th in n ing w ith abn orm al overlying vit reou s. Vit reous liquefact ion over a lat t ice lesion is com m on , an d vit reoret in al adh esion on it s borders is t aut an d st rong. W h ite lin es frequ en tly crisscross th e ret in al su rface of latt ice degen erat ion . Perip h eral pigm en t ar y ch anges an d w h ite flecks are often fou n d on th e posterior surface of th e lesion . Th e w h ite lin es corresp on d to sclerot ic vessels, an d th e flecks are con sisten t w ith glial cells. Progressive th in n ing of th e ret in al surface of lat t ice degen erat ion leads to form at ion of at roph ic h oles. Th ese h oles are n ot associated w ith vit reous t ract ion . Alth ough m ild su bret in al fluid m ay accu m u late, asym ptom at ic at roph ic ret in al h oles associated w ith lat t ice degen erat ion rarely lead to RD. Lesion s of lat t ice degen erat ion are u su ally foun d bet w een th e 11 an d 1 o’clock an d 5 an d 7 o’clock m eridian s for un kn ow n reason s. Th e dim en sion s of lat t ice patch es are h igh ly variable, even w ith in th e sam e eye. Lat t ice lesion s are t ypically asym ptom at ic.

Differential Diagnosis Oth er periph eral ret in al degen erat ion s

Management Myopia is a w ell establish ed risk factor for lat t ice degen erat ion . Lat t ice degen erat ion h as been lin ked to pigm en t dispersion syn drom e, alth ough th e reliabilit y of th e associated st u dies h as been qu est ion ed becau se of th eir sm all sam ple sizes. Myopia is sh ared by both con dit ion s, an d com m on et iological factors m ay con t ribute to th e developm en t of both con dit ion s. Lat t ice degen erat ion is best revealed th rough careful in direct oph th alm oscopy or periph eral ret in al biom icroscopy w ith scleral depression . Sign ifican t debate exist s on th e t reat m en t of lat t ice degen erat ion . Th e presen ce of lat t ice degen erat ion in creases th e risk of ret in al detach m en t from 0.01 to 0.5%. Mu lt iple au th ors h ave poin ted to th e fut ilit y of prophylact ic t reat m en t of asym ptom at ic lat t ice degen erat ion . Even if at roph ic h oles are foun d in a patch of lat t ice, th ey rarely result in RD. A ret rospect ive st u dy sh ow ed th at for eyes w ith RD, prophylact ic t reat m en t of lat t ice degen erat ion in th e fellow eyes w as associated w ith a risk reduct ion of RD from 5.9 to 1.8%. Previou s st u dies sh ow ed n o ben efit w ith prophylact ic t reat m en t for eyes w ith greater th an –6 diopters of m yopia an d m yopic eyes w ith m ore th an 6 clock h ours of lat t ice degen erat ion .

10 Surgical Retina

293

Close m on itoring of asym ptom at ic lat t ice lesion s is th e rout in e. Prophylact ic t reat m en t is reser ved for th e follow ing sit uat ion s: sym ptom at ic lat t ice lesion s, sign s of prom in en t vit reoret in al t ract ion , person al or fam ily h istor y of RD.

Retinal Tufts Cystic Retinal Tufts Th ese are discrete n odules of glial proliferat ion th at en t rap overlying vit reou s. Th us th e bon d bet w een t uft an d vit reous is par t icu larly st rong. It is con sidered a congen it al varian t . Approxim ately 5% of th e populat ion h as t u fts. Tufts h ave been fou n d as early as in fan cy an d do n ot sh ow a par t icu lar age predilect ion .

Presentation Cyst ic t u ft s can be located in all four qu adran ts. Th e average diam eter is from 0.1 to 1.0 m m . On clin ical exam th ey are u sually w h ite an d oval in appearan ce. Th ey are elevated on th e ret in al su rface but n ot t ran slu cen t like a ret in al break. Becau se of th e t igh t adh esion bet w een a ret in al t uft an d th e overlying vit reous, a ret in al flap tear or an opercu lated break m ay develop at th e site of th e t uft during a posterior vit reous detach m en t (PVD). Associated sym ptom s in clu de floaters, flash ing ligh ts, “cu r tain effect s,” an d loss of vision .

Differential Diagnosis Oth er periph eral ret in al degen erat ion s

Management Careful in direct oph th alm oscopy or p eriph eral ret in al biom icroscopy w ith scleral depression . Approxim ately 10% of RDs are origin ated from cyst ic ret in al t ufts. How ever, because cyst ic ret in al t uft s are foun d in on ly 5% of th e gen eral popu lat ion , th e overall risk of a ret in al det ach m en t from a t u ft is calcu lated to be on ly 0.28%. Ow ing to th is low in ciden ce, rou t in e prophylact ic t reat m en t of asym ptom at ic t u ft s is n ot recom m en ded. If a ret in al tear is associated w ith a t u ft , laser or cr yoth erapy is in dicated.

Noncystic Retinal Tufts Th ese t u ft s are ver y th in aggregates of ret in al t issu e fou n d in clusters at th e vit reous base. Th ey are less th an 0.1 m m in diam eter an d con sist of processes of Mü ller cells.

Presentation Non cyst ic t u ft s are fou n d as early as th e first decade of life. Th ey are foun d in 33% of adu lts an d usually on th e n asal p eriph eral ret in a. Th ere are n o sym ptom s.

Differential Diagnosis Oth er periph eral ret in al degen erat ion s

294

Color Atlas of Ophthalm ology

Management Man agem en t con sist s of obser vat ion on ly, w ith carefu l in direct oph th alm oscopy or p eriph eral ret in al biom icroscopy w ith scleral depression .

Zonular Traction Retinal Tuft Zon ular t ract ion t u fts (ZTTs) are dist ingu ish able from cyst ic an d n on cyst ic t u ft s by th eir greater size, closer locat ion to th e ora serrata, an d predilect ion for cau sing sm all, full-th ickn ess ret in al h oles in th e far ret in al periph er y. ZTTs are cau sed by abn orm al zon u lar con n ect ion s to th e periph eral ret in a. Th e result is a t aut t u ft of t issue pulled an teriorly tow ard th e ciliar y body.

Presentation ZTTs can be fu r th er subdivided in to ju xtabasal or in t rabasal t ufts. On ly in t rabasal ZTTs h ave clin ical sign ifican ce. Ret in al tears occur in 4% of all ZTTs. Th ey are th e cau se for 6% of ret in al tears in au topsy eyes. Associated tears are alm ost alw ays in t rabasal (w ith in th e vit reou s base) lesion s. Even w h en RD occurs, it is localized an d n onprogressive. Sym ptom s occu r on ly if com plicated by ret in al tears or RD.

Differential Diagnosis Oth er periph eral ret in al degen erat ion s

Management ZTTs occur th ree t im es m ore com m on ly in m en an d h ave a prevalen ce of 15% in au topsy cases. Th ey are also bilateral in 15% of cases an d m ore com m on in th e n asal ret in a. Th ey are best revealed th rough careful in direct oph th alm oscopy or periph eral ret in al biom icroscopy w ith scleral depression . Laser or cr yoth erapy is in dicated if th ere is eviden ce of a fu ll-th ickn ess ret in al break. Prophylact ic t reatm en t of ZTTs is n ot recom m en ded because associated ret in al breaks an d det ach m en ts are u su ally n onp rogressive.

Senile Retinoschisis Sen ile ret in osch isis is a degen erat ive process in w h ich th e ret in a split s in to t w o dist in ct layers at th e level of th e outer plexiform layer, in con t rast to juven ile ret in osch isis, in w h ich th e ret in a split s at th e n er ve fiber layer. Dysfu n ct ion of Mü ller cells likely con t ributes to th e form at ion of sen ile ret in osch isis. Th e ret in al sch isis cavit y th at form s over t im e m ay resem ble a ret in al det ach m en t . Tw o form s of sch isis h ave been described: t ypical degen erat ive an d ret icu lar degen erat ive.

Presentation In t ypical degen erat ive ret in osch isis th ere are oval areas of ret in al sp lit t ing an d fusiform elevat ion of th e in n er ret in al layer. Th e in n er layer con sist s of in tern al lim it ing m em bran e, in n er p lexiform layer, an d ret in al vessels. It can h ave a text u red appearan ce, an d w h ite dot s can be seen on it s surface. Th e outer layer con sists of p or t ion s of ou ter plexiform , outer n u clear, an d ph otoreceptor com pon en t s. It appears em pt y an d can h ave a beaten -m etal appearan ce. Ret in al breaks are n ot ch aracterist ic for t ypical degen erat ive ret in osch isis. Posterior exten sion tow ard th e m acula is un com m on .

10 Surgical Retina

295

Ret icular degen erat ive ret in osch isis h as m ore exten sive ret in al involvem en t . The in n er ret in a can becom e ext rem ely th in , bu llou s, an d dom e-sh aped. It con sists of on ly in tern al lim it ing m em bran e, rem n an t s of n er ve fiber layer, an d residu al vessels th at appear susp en ded in m id-air du e to th e th in ret in al appearan ce. Th ere is com plete loss of radial pillars. Typical cystoid degen erat ion is com m on ly seen an terior to areas of ret icu lar degen erat ive ret in osch isis. Ret in osch isis can be exacerbated by a posterior vit reous detach m en t . Th e sch isis cavit y m ay en large an d som et im es progress tow ard th e m acu la. How ever, th e m acula is frequen tly spared, an d th e rate of progression is u sually ver y slow. Outer ret in al breaks are m ore com m on th an in n er ret in al breaks in ret icu lar degen erat ive ret in osch isis. Th e appearan ce of dem arcat ion lin es in dicates th e det ach m en t of th e ou ter ret in a of a ret in osch isis an d its even t ual conversion to a full-th ickn ess RD. Th e rate of such a conversion is ver y slow. Th e n onprogressive t ype of det ach m en t occurs m uch m ore frequ en tly th an th e progressive t ype. How ever, th e form at ion of in n er ret in al h oles along w ith th e preexist ing ou ter ret in al h oles m ay lead to a rapid progression of a fu ll-th ickn ess RD th at requires prom pt t reat m en t . Sym ptom s occu r in case of ret in al breaks, ret in al detach m en t , or posterior extension tow ard th e m acu la.

Differential Diagnosis Oth er periph eral ret in al degen erat ion s

Management For au topsy eyes, t ypical degen erat ive ret in osch isis is seen in 1%of th e adult popu lat ion an d is bilateral in 33%of cases. Ret icu lar degen erat ive ret in osch isis is seen in 1.6% of adu lt s, an d bilateral on ly 16% of th e t im e. Both lesion s are com m on ly seen in th e in ferotem poral qu adran t . Man agem en t con sists of careful in direct oph th alm oscopy or periph eral ret in al biom icroscopy w ith scleral depression . Rout in e prophylact ic t reat m en t of m ost ret in osch isis is n ot n ecessar y ow ing to th eir usual lack of progression . Th eir in discrim in ate t reat m en t m ay cause n ew ret in al breaks, ret in al detach m en t , an d even p roliferat ive vit reoret in opathy. W ith sign s of progression , t reat m en t m ay in clude laser, cr yoth erapy, scleral buckling, an d vit rectom y, depen ding on th e locat ion an d exten t of th e path ology.

Posterior Vitreous Detachment Presentation Floaters (“cobw ebs,” “bugs,” “t adpoles,” or com m a-sh ap ed object s th at ch ange posit ion w ith eye m ovem en t), blurred vision , flash es of ligh t , w h ich are m ore com m on in dim illu m in at ion an d are tem porally located, are th e m ain com plain t s of th e pat ien t . On e or m ore discrete ligh t gray to black vit reou s op acit ies, often in th e sh ape of a ring (Weiss ring) are su spen ded over th e opt ic disk. Th e opacit ies float w ith in th e vit reous as th e eye m oves from side to side.

Differential Diagnosis Vit rit is, m igrain e, vit reous h em orrh age, RD

296

Color Atlas of Ophthalm ology

Management Com plete ocu lar exam in at ion , par t icularly a dilated ret in al exam in at ion , by using in direct oph th alm oscopy an d scleral depression to r ule ou t a ret in al break an d detach m en t is m an dator y. Th ere is n o sim ple t reat m en t for vit reous floaters. Using th e Neo-dym ium -YAG laser to break up vit reou s floaters is a con t roversial pract ice an d m ay be associated w ith th e risk of creat ing ret in al breaks an d RD. A vit rectom y is reser ved on ly for ver y den se vit reous floaters.

Retinal Tears and Retinal Detachment Th e posterior fun du s st r uct ures involved w ith th e developm en t an d m an agem en t of a ret in al detach m en t in clu de th e n eu rosen sor y ret in a, ch oroid, RPE, pars plan a, an d sclera. A rh egm atogen ou s RD is th e m ost com m on t ype of RD. It is cau sed by on e or m ore ret in al breaks. Most ret in al breaks are located bet w een th e equator an d th e ora serrata (28%) or n ear th e equator of th e globe (45%). Ch oroidal vortex vein s are th e m ost visible lan dm arks adjacen t to th e equ ator. Periph eral ret in al lesion s (see Ch apter 9), in cluding 12% of sm all ret in al breaks, are frequ en tly located close to th e ora serrat a, th e scalloped ju n ct ion bet w een th e n eu rosen sor y ret in a an d th e pars plan a at th e vit reous base. Th e distan ce bet w een th e equator an d th e ora m easures bet w een 5 an d 6 m m , an d it is sligh tly greater in th e tem poral th an in th e n asal ret in a. Th e pars plan a con sists of an in n er n onpigm en ted an d an ou ter pigm en ted layer th at span s ~6 m m from th e posterior lim bus to th e ora serrata radially at th e an terior fu n dus. It represen ts a su rgical “safe” zon e, th rough w h ich n eedles an d surgical in st ru m en ts m ay be passed w ith out cau sing ocu lar dam ages. Rare pars plan a breaks in clude focal tears an d dialyses associated w ith cert ain con dit ion s (e.g., congen it al develop m en t , t raum a, aph akia, an d atopic derm at it is). On ly 15% of ret in al breaks develop posterior to th e equator, in clu ding m acu lar h oles (1%), w h ich m ay in du ce a ret in al detach m en t in h igh ly m yopic eyes. In teract ion s bet w een cen t rifugal an d cen t rip et al forces across th e n eu rosen sor y ret in a determ in e th e st at u s of ret in al at t ach m en t versu s detach m en t . Cen t rifugal forces th at in du ce ret in al detach m en t in clude (1) vit reou s flu id en t r y in to th e subret in al space th rough ret in al breaks an d (2) vit reoret in al t ract ion al forces. Cen t ripet al forces th at prom ote ret in al at t ach m en t in clude (1) out w ard hydrostat ic vit reous flu id pressu re on th e ret in al su rface, (2) m ucopolysacch aride adh esives an d in terdigitat ing cellular processes bet w een th e ret in al ph otoreceptors an d th e RPE, (3) ch oroidal on cot ic pressu re, an d (4) m etabolic pum p of th e RPE. An im balan ce in favor of cen t rifugal forces th at leads to a ret in al detach m en t is m ost frequ en tly cau sed by th e on set of ret in al break(s).

Presentation Th e an n ual in ciden ce of rh egm atogen ous RD is close to 1 per 15,000 p eople, an d th e prevalen ce is 0.3% of th e gen eral populat ion . Th e prevalen ce is in creased for “h igh -risk” groups: 2% for aph akic pat ien ts, 2% for pat ien t s w ith asym ptom at ic lat t ice degen erat ion , 5% for h igh ly m yopic pat ien t s, an d 10% for pat ien ts w ith vitreou s loss du ring cat aract ext ract ion . Most ret in al breaks an d rh egm atogen ou s RDs develop spon t an eou sly. Th e aging p rocess gen erates vit reous syn eresis. Th e en suing fibrillar degen erat ion of th e vit reous predisposes an acu te PVD an d associated ret in al breaks/RD. Th erefore, th e m ost com m on age grou p for RD is bet w een 40 an d 70 years. Approxim ately 40

10 Surgical Retina

297

Fig. 10.3 Types of retinal break. (A) flap tear; (B) atrophic retinal hole; (C) operculated break; (D) retinal dialysis.

to 50% of RDs are associated w ith m yopia, 30 to 40% are associated w ith aph akia or p seu doph akia, an d 10 to 20% are associated w ith direct ocular t raum a. Close to 60% of RDs occur in m ales, possibly because th ere are m ore m ale th an fem ale m yopic p at ien t s. Abou t 15% of RDs even t ually develop in th e fellow eyes as w ell. Bilateral RDs are m ore com m on in aph akic eyes (25 to 30%). Gen et ic predisposit ion to RD in clu des Jew ish race an d fam ilial ten den cy (i.e., Jen sen disease). Th e black populat ion h as a low in ciden ce of RD. Visual-field loss an d scotom as are com m on feat u res for all categories of ret in al det ach m en t . Macu lar involvem en t leads to cen t ral vision loss. Th e locat ion of th e ret in al detach m en t is in th e opposing quadran t(s) to th e visu al-field defect ow ing to th e gen erally con t ralateral topograph ical correspon den ce bet w een th e ret in a an d th e visu al field. An terior vit reou s pigm en t gran ules detected on slitlam p biom icroscopy con st it u te a reliable h allm ark sign (posit ive Sh afer sign ) for th e presen ce of ret in al break(s). Vit reou s h em orrh age is frequen tly but n ot alw ays associated w ith ret in al breaks/RD. Th ere are several t ypes of ret in al breaks ( Fig. 10.3 ): Flap tears: Th ese are also kn ow n as h orsesh oe tears du e to th eir U-sh aped m orph ology. An terior vit reoret in al t ract ion on th e ret in al flaps con n ected to th e an terior m argin s of th e tears m akes th e flaps poin t an teriorly tow ard th e vit reou s base. Sym ptom at ic flap tears are h igh risk. Su bret in al fluid ten ds to accum ulate arou n d th e breaks, an d h en ce, breaks sh ou ld be t reated prom ptly. A gian t tear span s 3 or m ore clock h ours an d u su ally h as a scrolled flap th at m ay roll over on itself. It requ ires a vit rectom y as m an agem en t . At rophic ret inal holes: Th ese lesion s are due to progressive ret in al th in n ing w ith ou t associated vit reoret in al t ract ion . Th ey are often w ith in patch es of latt ice degen erat ion . Most at roph ic h oles h ave a low risk an d do n ot requ ire prophylact ic t reat m en t , par t icu larly w h en located in feriorly.

298

Color Atlas of Ophthalm ology

Operculated break s: Persisten t vit reoret in al t ract ion m ay lead to a com plete separat ion of a free-float ing vit reou s opercu lu m from th e u n derlying break. It ten ds to locate m ore posterior th an flap tears. It is in term ediate in risk (bet w een flap tears an d at roph ic h oles) for an RD. Su perior tem poral opercu lated ret in al tears are m ore vuln erable th an in ferior n asal opercu lated tears for vision loss. Treat m en t or obser vat ion is on a case-by-case basis. Dialysis: Th is is a circum feren t ial ret in al tear by th e ora serrata. Un like oth er breaks, vit reoret in al t ract ion is on th e posterior m argin of th e dialysis. It occurs m ore com m on ly in th e you ng. Ret in al dialysis m ay be congen it al (m ostly in th e in ferior tem poral qu adran t) or t raum at ic (usually in th e superior n asal qu adran t). Ret in al dialysis carries m oderate to h igh risk for RD an d m ay requ ire t reat m en t , in clu ding laser, cr yoth erapy, or scleral bu ckling/vit rectom y. Acute : For acute ret in al breaks leading to rh egm atogen ou s RD in duced by PVD, cardin al sym ptom s in clu de flash ing ligh t s, floaters, cobw ebs, an d a cur t ain effect . Th e m ech an ical dist urban ce of th e ret in a by vit reoret in al t ract ion is respon sible for th ese ph otopic ph en om en a.

Differential Diagnosis Ret in al det ach m en t s ( Fig. 10.4 ) are divided in to th ree t ypes: rh egm atogen ou s, exu dat ive, an d t ract ion al ( Table 10.1 ). Rhegm atogenous RD: Th ese are th e m ost com m on . Th ey involve ret in al breaks. Exudat ive RD: Th ese are caused by excessive accum ulat ion of subret in al serous flu id th at m ay sh ift w ith ch anges in body posit ion . Exam p les of con dit ion s associated w ith an exu dat ive RD in clude cen t ral serou s ch orioret in opathy, various ch oroidal t um ors, an d diverse posterior in flam m ator y diseases. Tract ional RD: Th ese are caused by vit reoret in al t ract ion (e.g., proliferat ive diabet ic ret in opathy). W h ereas a su rgical in ter ven t ion is u su ally in dicated for a rh egm atogen ous RD, it is often n ot requ ired for an exudat ive RD. A t ract ion al RD involving th e m acu la requ ires su rger y. A ret in osch isis m ay m im ic a ret in al detach m en t . Dist in ct ive feat ures of ret in osch isis in clude a th in an d dom e-sh ap ed elevated in n er layer, absen ce of dem arca-

Fig. 10.4 View of a tum or associated with a retinal detachm ent. The detached retina is denoted with blue arrow.

10 Surgical Retina

Table 10.1

299

Di e rences betw een Types o f Retinal Detachm ent

Feature

Rhegm ato geno us

Exudative

Tractio nal

1. Retinal break

Yes

No

2. Shifting uid 3. Choroidal mass

No No

4. Intraocular pressure 5. Transillumination

Low

Yes May be present Varies

No primary break; secondary break can occur No No

Normal

6. Retinal mobilit y

Undulating folds

7. Extent of RD

Extends to ora quickly Mostly present

8. Pigm ent in vitreous 9. Subretinal uid

Clear

Normal

Blocked transNorm al illumination If pigmented choroidal lesion is present Smoothly elevated, Taut retina no folds Gravit y dependent Does not extend to ora No Present in trauma cases Turbid Clear

RD, ret in al det ach m en t .

t ion lin es, an d clu sters of ou ter oval ret in al h oles ( Fig. 10.5 ). It is often bilaterally sym m et rical an d frequen tly localizes to th e in ferotem poral quadran t . If diagn osis is u n cer tain , a laser spot test can be perform ed. A m ild ret in al laser u pt ake on th e ou ter ret in al layer con firm s th e iden t it y of ret in osch isis. Most ret in osch isis lesion s do n ot progress an d requ ire n o th erapy. Developm en t of both in n er- an d outerlayer h oles m ay conver t a ret in osch isis in to a rh egm atogen ous RD, w h ich requires prom pt t reat m en t . A rare progression of a ret in osch isis close to th e m acula also requ ires barrier laser th erapy, scleral bu ckling, or vit rectom y.

Fig. 10.5

Retinoschisis.

300

Color Atlas of Ophthalm ology

Management Th e effect ive t reat m en t of a rh egm atogen ous RD requ ires th e iden t ificat ion an d localizat ion of all ret in al breaks ( Fig. 10.6 ). In direct oph th alm oscopy w ith scleral in den t at ion is an in dispen sable tool for accom p lish ing th is goal. An cillar y tech n iqu es for revealing ret in al breaks in clude biom icroscopy an d ult rason ography. Historical details of th e RD m ay provide clues of th e respon sible breaks. For in stan ce, an in ferior visual-field defect poin t s to a su perior tear an d RD. Rapid progression of th e

Fig. 10.6 Algorithm for localizing retinal break(s) associated with a retinal detachment. RD, retinal detachm ent; SRF, subretinal fluid; VF, visual field.

10 Surgical Retina

301

Fig. 10.7 Final view of buckle with proper retinal indentation and positioning of the tear. This internal/external conceptual illustration shows a cross section and the corresponding surgeon’s view of the final configuration of a circumferentially placed sponge exoplant (E). The cross section shows a portion of the globe without sclera (S) to enhance clarit y for the reader. The surgeon’s view is through the indirect ophthalmoscope (O). Note the indented configuration in both views. The retina is reat tached and the tear (T) is flat. Also note that the tear is properly positioned on the anterior slope of the buckle, as indicated by the cot ton-tipped applicator (A). (Courtesy of Highlights of Ophthalmology, “Retinal and Vitreoretinal Surgery: Mastering the Latest Techniques” English Edition, 2002. Editorin-Chief: Benjamin F. Boyd, MD., FACS; Co-Editor: Samuel Boyd, MD.)

RD suggests a large break, a p osterior break, or a su perior break. Previou s report s also poin t out th e im port an ce of th e pat tern of su bret in al fluid dist ribu t ion in relat ion to th e locat ion s of th e prim ar y ret in al breaks. Th e closure of all ret in al breaks by in duct ion of firm ch orioret in al adh esion s bet w een th e breaks an d un derlying RPE is th e key to successfu l m an agem en t of a rh egm atogen ou s RD. Adh esive m odalit ies in clu de diath erm y, cr yoth erapy, an d ph otocoagu lat ion ( Fig. 10.7 ). Diathermy Electrical current generates heat at the tip of the diatherm y probe, used for localizing retinal breaks. Diatherm y lesions on partial-thickness sclera induce strong chorioretinal adhesions as w ell. Full-thickness treatm ent is avoided due to scleral necrosis.

302

Color Atlas of Ophthalm ology

Cryothera py Low -tem perat ure freezing at th e t ip of th e cr yoprobe allow s ret in al t reat m en t th rough full-th ickn ess sclera w ith ou t scleral dam age. Direct m on itoring w ith in direct oph th alm oscopy for a w h it ish ice-ball su rroun ding th e ret in al break is th e best m eth od. Photocoagula tion Precise ch orioret in al upt akes w ith laser ph otocoagulat ion can be applied on ly on at t ach ed ret in a or areas of m in im al su bret in al fluid, lim it ing it s u t ilit y in RD repair. Tran sscleral applicat ion of a diode laser sh ow s prom ising resu lts. Supplem en tal ph otocoagu lat ion on th e buckle en h an ces resorpt ion of residual su bret in al flu id adjacen t to ret in al breaks du ring or after scleral bu ckling. In t raocu lar inject ion of gas before or after supplem en tal laser for t am pon ade of th e su perior ret in al breaks on th e bu ckle m ay fur th er facilitate th e resolut ion of th e residu al subret in al fluid. Sclera l Buckling Th e th ree cardin al step s for successfu l scleral buckling in clu de (1) accu rate localizat ion of all ret in al breaks, (2) in duct ion of ch orioret in al adh esion s, an d (3) suppor t of ret in al breaks on th e buckle. Localizat ion of breaks is at tain ed w ith extern al scleral m arks by diath erm y or sim ilar devices u n der gu idan ce of in direct oph th alm oscopy. Ch orioret in al adh esion s are ach ieved w ith cr yoth erapy, diath erm y, or ph otocoagu lat ion . Su ppor t of ret in al breaks is accom plish ed w ith extern al scleral in den t at ion w ith don or t issues or syn th et ic m aterials. Solid silicon e ru bber or sponges are th e m ost com m on ly em ployed bu ckling elem en ts. Th ey are an ch ored in place w ith scleral su t ures or scleral belt loops, placing th e breaks on th e an terior crest of th e buckle. Scleral buckling tech n iqu es are broadly divided in to episcleral versus in t rascleral, en circling versu s segm en t al, an d drain age versus n on drain age. Ep iscleral tech n iques h ave largely supp lan ted in t rascleral m eth ods in m odern scleral bu ckling. Table 10.2 outlin es th e algorith m s for ch oosing en circling versu s segm en t al buckling procedu res. Su bret in al flu id drain age is perform ed th rough a ch oroidotom y u n der a radial scleral cut-dow n w ith a tapered su t ure n eedle, diath erm y n eedle, 27- or 30-gauge n eedle, or en dolaser probe. In dicat ion s for drain age in clu de (1) bu llou s RD w ith h igh ly elevated tears, (2) p seu doph akic or h igh ly m yopic eyes w ith m ult iple periph eral breaks, (3) ch ron ic RD w ith viscou s subret in al fluid an d cellu lar elem en t s, (4) eyes w ith poor ch oroidal or RPE fu n ct ion , an d (5) severe glau com a or fresh cat aract w oun d n ot toleran t of ocu lar hyper ten sion . Recurren t RD du e to drain age com plicat ion s occurs in 12% of cases. Long-term successes of drain age an d n on drain age bu ckling procedu res are equ ivalen t .

Table 10.2

Cho ice o f Buckling Pro ce dures

Segm ental Few peripheral breaks Single break Mobile retina Clear vitreous Healthy sclera Good visualization

versus Encircling Many peripheral breaks Extensive breaks Rigid retina with folds Cloudy vitreous, vitreous strands and m em branes Thin and weak sclera Suboptimal visualization

10 Surgical Retina

303

Alterna tive Techniques Altern at ive tech n iqu es in clu de pn eu m at ic ret in opexy, tem porar y orbit al balloon , an d prim ar y vit rectom y. Pn eum at ic ret in opexy involves cr yoth erapy or laser to in duce ch orioret in al adh esion s an d inject ion of a sm all gas bubble to t reat lim ited su perior ret in al breaks (w ith in 1 to 2 clock h ours ap ar t) of a sim ple RD. A tem porar y orbital balloon allow s t reat m en t of sim ple RDs w ith lim ited su perior or in ferior breaks w ith ou t n eed of perm an en t buckling. Vit rectom y ach ieves direct in tern al flat ten ing of com plex breaks an d RDs w h ile avoiding extern al bu ckling elem en t s. In t raoperat ive an d postoperat ive factors con t ribute to su rgical failure. In t raoperat ive causes in clu de (1) in adequate localizat ion , ch orioret in al adh esion , or su ppor t of breaks; (2) m arked su bret in al or ch oroidal h em orrh age; (3) in adequate relief of vit reoret in al/ch orioret in al t ract ion ; (4) com plicat ion s of subret in al flu id drain age (in carcerat ion , perforat ion , h em orrh age); (5) in adverten t ret in al, ch oroidal, scleral perforat ion s; an d (6) severe ch oroidal detach m en t . Postoperat ive causes in clude (1) m arked exudat ive ret in al an d ch oroidal det ach m en t , (2) an terior segm en t isch em ia, (3) proliferat ive vit reoret in opathy, (4) ext r usion or in t rusion of bu ckling elem en ts, an d (5) in fect ion s (episcleral, en doph th alm it is). Num erou s advan ces in vit reoret in al su rgical tech n iqu es p rom ise m ore precise an d qu icker surgical t reat m en ts for RD on an ou t pat ien t basis (Figs. 10.8 , 10.9 ,

Fig. 10.8 Retinal reat tachment with perfluorocarbon liquid in case of giant tear. In the case of retinal detachment with a giant retinal tear, perfluorocarbon liquid (P) is injected into the vitreous cavit y via a Chang cannula (N). Because the liquid has a specific gravit y greater than water, it gravitates (blue arrow) to the posterior pole. This forces the subretinal fluid (S, red arrows) out through the giant retinal tear and out of the eye via the Chang cannula. The retina (R) is being forced to reat tach (green arrow) in this manner. E, endoillum inator; I, infusion cannula. (Courtesy of Highlights of Ophthalmology, “Retinal and Vitreoretinal Surgery: Mastering the Latest Techniques” English Edition, 2002. Editor-in-Chief: Benjamin F. Boyd, MD, FACS; Co-Editor: Samuel Boyd, MD.)

304

Color Atlas of Ophthalm ology

Fig. 10.9 For surgical treatment of proliferative vitreo-retinopathy, perfluorocarbon liquid (L) m ay be injected. This will reveal any persistent retinal traction related to epiretinal membranes (P) that must be rem oved. A vitreoretinal pic or Grieshaber mini-diamond forceps (F) is used to remove such a m em brane (P). Note the subretinal mem brane (S), endoilluminator (E), and infusion terminal (I). (Courtesy of Highlights of Ophthalmology, “Retinal and Vitreoretinal Surgery: Mastering the Latest Techniques” English Edition, 2002. Editor-in-Chief: Benjam in F. Boyd, MD, FACS; Co-Editor: Sam uel Boyd, MD.)

Fig. 10.10 Air-fluid exchange and internal drainage of subretinal fluid (white arrow) with an extrusion needle (A) is performed to flat ten the retina. E, endoilluminator. (Courtesy of Highlights of Ophthalmology, “Retinal and Vitreoretinal Surgery: Mastering the Latest Techniques” English Edition, 2002. Editor-in-Chief: Benjamin F. Boyd, MD, FACS; Co-Editor: Samuel Boyd, MD.)

10 Surgical Retina

305

Fig. 10.11 Combined m icrophakonit (700 µm cataract surgery) and 25-gauge transconjunctival sutureless vitrectomy.

10.10 ). Pn eum at ic ret in opexy an d vit rectom y h ave becom e in creasingly p opu lar tech n iques for repairing RD. Recen t advan ces in 25-gauge vit rectom y tech n ology m ay allow it s applicat ion on cer tain sim ple RDs ( Fig. 10.11 ).

Scleral Buckle Procedure Scleral buckling procedure is a t im e-h on ored surgical tech n ique first popu larized by Cu stodis for repairing an RD. Su ccessful scleral bu ckling requires th e fu lfillm en t of several im port an t criteria, in clu ding (1) th e accu rate assessm en t an d localizat ion of all ret in al breaks an d vit reoret in al t ract ion , (2) appropriate applicat ion of ret in opexy for all associated ret in al breaks, (3) precise p lacem en t of scleral bu ckling elem en t s to sup por t th e u n derlying ret in al breaks an d relief of vit reoret in al t ract ion , an d (4) proper drain age of subret in al flu id. Drain age of su bret in al flu id is n ot alw ays n ecessar y, an d som e su rgeon s prefer th e n on drain age scleral bu ckle procedu re.

Accurate Localization of Retinal Breaks Th e con tour of an RD is frequ en tly determ in ed by th e locat ion s of its associated ret in al breaks. Figure 10.12 depicts th e rules for predict ing th e locat ion (s) of th e ret in al break(s) resp on sible for th e RD based on it s con tou r. Lin coff h as poin ted ou t th at for RDs w ith an asym m et rical con tou r n ot crossing th e 12 o’clock m eridian , th e resp on sible break is fou n d w ith in 1.5 h ours from its h igh est level. In case th e RD crosses th e 12:00 m eridian , th e prim ar y break(s) can be detected w ith in 1½ h ou rs from 12:00. Sch epen s an d Hilton h ave also in dicated a series of rules regarding fin ding th e respon sible ret in al breaks: (1) th e prim ar y break is close to th e u pper m argin of th e RD for a su perior quadran t ic detach m en t , (2) th e prim ar y break is close to 12:00 for a superior sym m et rical h em isph eric RD, (3) th e prim ar y break

306

Color Atlas of Ophthalm ology

Fig. 10.12 The distribution of the subretinal fluid associated with a retinal detachment (RD) points to the likely location of the retinal break(s): (a) superior RD: break is near the superior edge; (b) superior symmetrical hemispheric RD: break is close to 12:00; (c) inferior quadrantic RD: break is near superior edge; (d) inferior hemispheric detachment: break is by 6:00; (e) asym metrical inferior RD: break is close to side with higher level; (f) superior RD crossing 12:00: break is within l½ clock hours from 12:00; (g) RD with a demarcation line: break is along meridian bisecting the demarcation. (Adapted from Agarwal et al. Textbook of Ophthalmology (4 Vols.). New Delhi, India: Jaypee Brothers.)

is close to th e upper m argin or along th e m eridian bisect ing th e region of th e RD for an in ferior quadran t ic RD, (4) th e prim ar y break is close to 6 o’clock for a sym m et rical in ferior h em isph eric RD, (5) th e break is close to th e u pper m argin for an asym m et rical in ferior h em isph eric RD, (6) th e respon sible break is u su ally foun d w ith in 12:00 to 2:00 m eridian s in cases of a com plete RD, (7) th e ret in al break is along th e m eridian bisect ing th e dem arcat ing zon e for an RD w ith a dem arcat ion lin e. Deviat ion s from th ese ru les can be expected in case of ch orioret in al scarring an d t ract ion or reoperat ion s.

Color-Coded Diagram A color-coded ch ar t w ith specific sym bols represen t ing variou s ret in al lesion s an d t reat m en t m odalit ies allow s detailed docu m en tat ion of th e path ology of th e RD an d it s m an agem en t in an organ ized m an n er. Figure 10.13 dem on st rates su ch a ch ar t frequ en tly used along w ith scleral bu ckling tech n iqu es.

10 Surgical Retina

307

Fig. 10.13 A color-coded fundus chart with specific symbols allows the docum entation of the relevant retinal lesions associated with a retinal detachm ent, and the detailed steps of the associated scleral buckling procedure. (Adapted from Agarwal et al. Textbook of Ophthalmology (4 Vols.). New Delhi, India: Jaypee Brothers.)

308

Color Atlas of Ophthalm ology

Fig. 10.14 The parallax associated with a bullous retinal detachment tends to cause the m istaken localization of the retinal break to be m ore posterior than it should be. A compensatory anterior adjustment during the localization is necessary.

Parallax Associated w ith View ing Paralla x is a frequen tly en coun tered ph en om en on w h en view ing an RD w ith bullous subret in al flu id, w h ereby th e associated ret in al break appears m ore posterior th an it s act u al locat ion . Th u s a com pen sator y an terior sh ift ing is n eeded to accu rately localize th e ret in al break ( Fig. 10.14 ).

Shafting during Localization Figure 10.15 sh ow s th e ph en om en on of sh aft ing th at m istakes th e locat ion of th e com pressing diath erm y sh aft on th e globe for th e t ip of th e probe. To avoid th is error, th e t ip of th e diath erm y probe is placed on th e extern al locat ion of th e ret in al break first an d th en arch ed for w ard to p reven t exten sive con tact of th e sh aft of th e probe w ith th e globe du ring localizat ion of th e ret in al break.

Exoplant Material Soft an d h ard silicon e exoplan ts h ave w ith stood th e test of t im e to be du rable an d safe bu ckling m aterial for su ppor t ing ret in al breaks in th e repair of an RD. Figure 10.16 sh ow ed details of soft silicon e sp onges an d h ard silicon e r ubber. Com m on ly used silicon e sponges in clu de 3, 5, an d 7.5 m m diam eter sponges. Sponges m ay also be t rim m ed to a precise dim en sion to cu stom ize th em for specific cases. Th ere are n um erou s st yles of h ard silicon e ru bber w ith variable diam eters. More com m on st yles of solid silicon e im plan ts in clu de 20, 40, 41, 42, 220, 240, 276, 277, an d 287 ban ds or t ires. A silicon e t ire h as a groove along it s long axis to accom m odate a silicon e ban d. Th e groove m ay be cen t rally located as in 20, 210, an d 277 t ires. An off-cen tered groove (e.g., 276 t ire), allow s a m ore posterior bu ckling effect . Th e m ajorit y of silicon e ru bbers are design ed for circu m feren t ial placem en t , w h ereas m ost silicon e sponges are design ed for radial placem en t . How ever, circu m feren t ial

10 Surgical Retina

309

Fig. 10.15 (A) Mistaking the shaft for the tip of the diathermy probe may result in too posterior a placem ent of the localizing diathermy mark. (B) This error may be avoided with an anterior arching of the distal end of the probe, and indenting with only the tip of the probe.

Fig. 10.16 Solid silicone is well tolerated by the eye and is the most commonly used material for an implant or explant during scleral buckling in the modem era. (A) It is available in (A) the form of soft sponges, or (B) solid rubber. A rubber silicone tire has a groove along its long axis to accommodate an encircling band. (C) The groove may be centrally located as in a 277 tire, or (D) off-centered as in a 276 tire associated with a m ore posterior buckling effect. (E) A meridional silicone wedge allows the radial support of a retinal break.

310

Color Atlas of Ophthalm ology

sponges an d radial silicon e ru bbers (e.g., silicon e w edges) are available but u sed less frequ en tly. A silicon e sponge w ith a h ollow in terior to accom m odate in sert ion of a circum feren t ial ban d allow s a h igh er bu ckling effect correspon ding to th e locat ion of it s placem en t (e.g., 507T).

Proper Suturing Technique Spat u lated n eedles are u sed for an ch oring su t ures on th e sclera. To avoid in adverten t scleral perforat ion , th e angle of th e n eedle m ust be relat ively parallel to th e plan e of th e sclera ( Fig. 10.17 ).

Fig. 10.17 The angle of the spatulated needle m ust be parallel to the scleral surface to avoid scleral perforation.

Fig. 10.18 A distance of 6 mm is required bet ween the t wo circumferentially placed sutures to anchor a solid silicone rubber implant of 4 mm width and 1 mm thickness (1 mm + 4 mm + 1 mm = 6 mm).

10 Surgical Retina

311

Calculating the Distance of Suture Placement for Silicone Exoplant In gen eral, th e cross-sect ion al circu m feren t ial dim en sion of th e silicon e exoplan t is calcu lated to est im ate th e distan ce of su t ure placem en t , as sh ow n by th e follow ing exam ples: Circum feren t ial im plan tat ion of solid silicon e ru bber w ith a 4-m m w idth an d 1 m m th ickn ess requ ires a sut u re distan ce of 6 m m (1 m m + 4 m m + 1 m m = 6 m m ) ( Fig. 10.18 ). Th e size of a tear n eeds to be w ith in 4 m m to fit on th e an terior slope of a circum feren t ial bu ckle created by a 5-m m sponge (π x D/4 = 3.1416 × 5/4 = 3.927) ( Fig. 10.19 ). Th e an ch oring of th e m at t ress su t u re corresp on ding to th e m eridian (s) of th e ret in al break(s) m axim izes th e h eigh t of th e buckle for proper su pport of th e break(s). Th e sut u re n eedle is placed at 1 to 2 m m from th e m argin s of th e silicon e ru bber ( Fig. 10.20 ).

Fig. 10.19 To fit on the anterior slope of the buckle created by a circumferentially placed 5-mm sponge, the size of the tear needs to be within 4 mm (π × D/4 = 3.1416 × 5/4 = 3.927).

Fig. 10.20 The anchoring suture is placed at 1 to 2 mm from the margins of the circumferential solid silicone implant corresponding to the m eridian of the retinal break to maximize the support of the break.

312

Color Atlas of Ophthalm ology

Fig. 10.21 When placing a radial sponge for a large tear, the size of the sponge should be 1 to 2 mm wider than the tear (e.g., 5-mm sponge for a 3-mm tear). The horizontal distance bet ween the t wo radial bites of the double -armed suture should be 2 to 3 mm beyond the width of the sponge (e.g., 7 to 8 mm for a 5-mm sponge). The lat ter distance equals the half circumference of the sponge and can be calculated with the formula: Distance = ½ circumference = πD/2 = (3.416)(5)/2 = 7.85 mm. (Courtesy of Retina Consultants Ltd., St. Louis, MO.)

Radial placem en t of a 5-m m sponge requires a su t ure distan ce of 7.85 or 8.0 m m . In gen eral, th e h orizon tal distan ce bet w een th e radial bites of th e doublearm ed su t u re sh ould be 2 to 3 m m beyon d th e w idth of th e sponge (e.g., 7 to 8 m m for a 5-m m sponge). Th e precise calcu lat ion is h alf circu m feren ce of th e radial sponge [½ (π diam eter) or 3.1416 × 5/2 = 7.85 or 8.0 m m ] ( Fig. 10.21 ). For proper sup por t of a ret in al tear w ith a radial sponge, th e m at t ress sut ures n eed to be placed from 1 to 2 m m an terior to th e an terior h orn s of th e tear an d 3 to 4 m m posterior to th e apex of th e tear. To ach ieve p roper su t u re ten sion , th e assist an t h olds th e su t ure kn ot after th e surgeon m akes th e first double th row of th e sut ure kn ot ( Fig. 10.22 ). For a large ret in al tear, a 12-m m sponge or t w o 5-m m sponges are placed side by side. Th e proper h orizon tal dist an ce bet w een th e t w o radial su t ure bites in th e lat ter sit u at ion is calcu lated w ith th e follow ing form ula: 7.85 m m + 5 m m – com pression factor = 11.5 m m ( Fig. 10.23 ).

10 Surgical Retina

313

Fig. 10.22 For a radial sponge to support a large tear, the anterior mat tress suture should start at 2 mm anterior to the anterior horn of the tear, whereas the posterior suture should extend 3 to 4 mm posterior to the apex of the tear. Achieving the proper tension on the suture may also require the assistant to hold the suture knot after the surgeon makes the first double throw of the suture knot.

Fig. 10.23 For a very large tear, a 12-mm sponge or t wo 5-m m sponges may be placed side-by-side. The distance bet ween the t wo radial bites of the mat tress suture for t wo 5mm sponges placed side-by-side can be calculated with the following formulas: distance across 2 sponges = 7.85 m m + 5 mm = 12.85 mm; 12.85 mm – compression factor = 11.5 m m. (Courtesy of Retina Consultants Ltd., St. Louis, MO)

314

Color Atlas of Ophthalm ology

Fig. 10.24 If a large staphyloma is present along the course of an encircling band, it can be covered by a wider silicone groove-piece or tire under the band, which is then sutured on the thicker sclera outside of the staphyloma. (Courtesy of Retina Consultants Ltd., St. Louis, MO.)

Anterior Shifting of Suture Tying for a Posterior Buckle To ease t ying of th e sut u re kn ot for a radial bu ckle, th e su t u re kn ot can be sh ifted from th e p osterior to th e an terior por t ion of th e m at t ress su t u re for t ying (Fig. 10.24 ).

Drainage of Subretinal Fluid Drain age of su bret in al flu id can be accom plish ed (1) via a radial scleral in cision or (2) un der a p ar t ial-th ickn ess t riangular-sh aped scleral flap . Th e scleral cut-dow n is m ade by a n o. 64 or 69 Beaver blade. Diath erm y m ay be used to ach ieve h em o-

Fig. 10.25 A radial scleral drainage site is made with a 3- to 5-mm incision with a no. 64 or 69 Beaver blade. A 5–0 suture may be preplaced on the scleral edges and the edges may be diathermized. A tapered suture needle, a sharp diathermy electrode, a 27- or 30gauge needle may be used for the transchoroidal penetration during drainage.

10 Surgical Retina

315

Fig. 10.26 A triangular scleral flap may also be made for a drainage site. A 5–0 nonabsorbable suture is preplaced on the apex of the scleral flap and the corresponding corner of the scleral bed. A minimal radial cut-down through the rem aining thin scleral fibers of the drainage bed allows a quick exposure of the choroid for drainage.

stasis. Th e ch oroid is p en et rated w ith a tapered n eedle (e.g., 6–0 silk C-1 n eedle), sh arp diath erm y t ip, 27- or 30-gauge n eedle, or sh arp kn ife. Th e altern at ive is th e use of laser delivered by an en dolaser probe or a laser in direct oph th alm oscope ( Figs. 10.25 an d 10.26 ). Cot ton applicators are used to gen tly com press th e sclera adjacen t to th e drain age site (especially an terior to it) to en h an ce drain age of su bret in al flu id. Th e appearan ce of sm all pigm en t clum ps in th e exit ing flu id in dicates th e elim in at ion of m ost of th e su bret in al fluid. In direct oph th alm oscopy is perform ed to in spect th e com pleten ess of th e fluid drain age an d detect any com plicat ion s associated w ith th e drain age site ( Fig. 10.27 ).

Fig. 10.27 Gentle traction of the edges of the sclerotomy and mild indentation of the sclera with a cotton-tipped applicator allow further drainage of the residual subretinal fluid.

316

Color Atlas of Ophthalm ology

Conclusion Adh eren ce to th e foregoing proper tech n iqu es for scleral buckling en h an ces th e ch an ce of su ccessful repair of a ret in al det ach m en t . Despite th e in creasing popu larit y of altern at ive su rgical procedu res, scleral buckling rem ain s a reliable su rgical tech n ique for repairing m ost prim ar y an d cert ain recurren t ret in al detach m en ts. It s vit al role in ach ieving favorable an atom ical an d visu al outcom e for rep airing ret in al detach m en t persist s in th e m odern era of vit reoret in al su rger y.

Choroidal Detachment Serous Choroidal Detachment Int raoperat ive or postoperat ive : Woun d leak, perforat ion of th e sclera from a superior rect us, bridle su t u re, irit is, cyclodialysis cleft , leakage or excess filt rat ion from a filtering bleb, or after laser ph otocoagu lat ion or cr yoth erapy Traum at ic: Often associated w ith a rupt ured globe, rh egm atogen ous ret in al detach m en t , or after scleral bu ckling repair or a detach m en t

Presentation Pat ien ts presen t w ith decreased vision or are asym ptom at ic. Th ere m ay be m oderate to severe pain . Decreased vision m ay occur if th e ch oroidal det ach m en t s (CDs) are tou ch ing (“kissing ch oroidals”) or h em orrh agic. Sm ooth , bu llou s, orangebrow n elevat ion of th e ret in a an d ch oroid usually exten ds 360 degrees aroun d th e periph er y in a lobu lar con figu rat ion . Low in t raocular pressu re (IOP) (often less th an 6 m m Hg), sh allow an terior ch am ber w ith m ild cell an d flare, an d posit ive t ran sillu m in at ion s are associated fin dings.

Differential Diagnosis Melan om a of th e ciliar y body an d RRD

Management Check history : Gon ioscopy, fu n du s exam in at ion of both eyes, B-scan , an d absen ce of t ran sillu m in at ion con firm th e diagn osis. Cycloplegic, topical steroid, an d surgical drain age of th e su prach oroidal flu id are th e t reat m en t opt ion s. Repair th e un derlying problem . W ound leak or leak y filtering bleb : Patch for 24 h ou rs, su t u re th e site, u se cyan oacr ylate glue, p lace a ban dage con tact len s on th e eye, or a com bin at ion of th ese. Cyclodialysis cleft : Laser th erapy, diath erm y, cr yoth erapy, or su t ure th e cleft to close it . Uveit is: Topical cycloplegic an d steroid as discu ssed previou sly Inflam m atory disease : See th e specific en t it y. Ret inal detachm ent : Surgical repair. Proliferat ive vit reoret in opathy after repair is com m on .

10 Surgical Retina

317

Hemorrhagic or Expulsive Choroidal Detachment In t raoperat ive or postoperat ive (from an terior disp lacem en t of th e ocular con ten ts an d rupt u re of th e sh or t posterior ciliar y ar teries) is th e m ost com m on cau se.

Presentation Sym ptom s are th e sam e as for serous ch oroidal detach m en t . Pain an d red eye m ay be p resen t m ore often . High IOP (if detach m en t is large), sh allow an terior ch am ber w ith m ild cell an d flare, an d absen ce of t ran sillum in at ion are feat u res in w h ich h em orrh agic CD differs from serous CD.

Differential Diagnosis Melan om a of th e ciliar y body an d RRD

Management Ch eck the histor y. Confirm ation of th e diagnosis depen ds on h istor y, gon ioscopy, fundus exam in ation , B-scan , and absen ce of transillum in ation . An an terior vit rectom y an d drain age of th e ch oroidal det ach m en t is perform ed for severe cases w ith ret in a or vit reous to th e w ou n d. Oth er w ise u se gen eral t reat m en t .

Macular Hole A m acular h ole is a roun d break involving th e layers of th e ret in a, eith er in a part ial- or fu ll-th ickn ess m an n er, in th e m acu lar region . Th e lesion is fou n d m ore com m on ly in w om en an d is predom in an t in th e fifth to seven th decades (Fig. 10.28 an d 10.29 ).

A

B Fig. 10.28 (A) Color fundus photograph showing a well-circumscribed full-thickness macular hole with minimal surrounding fluid cuff. (B) Fundus fluorescein angiography of the sam e patient showing a fading fluorescence in the late phase indicating the window defect, the most com mon FFA finding in macular holes.

318

Color Atlas of Ophthalm ology

A

B Fig. 10.29 Optical coherence tomography showing (A) a full-thickness and (B) lamellar macular hole.

Presentation Slit-lam p biom icroscopy of a full-th ickn ess m acu lar h ole sh ow s a w ell-circum scribed pu n ch ed-ou t lesion th at predom in an tly involves th e fovea. A par t ial-th ickn ess m acu lar h ole can be an ou ter lam ellar h ole or an in n er lam ellar h ole, depen ding on th e et iology beh in d it . It can be classified as follow s: Fu ll-th ickn ess m acular h ole Outer lam ellar h ole In n er lam ellar h ole Gass Classifica tion for Stages of Idiopa thic Macula r Hole Stage 1A: Im pen ding h ole, foveal det ach m en t w ith yellow spot Stage 1B: Im pen ding h ole, foveal det ach m en t w ith yellow h alo Stage 2 : Full-th ickn ess h ole (cen t ral or eccen t ric) form at ion w ith ou t vit reofoveal detach m en t or a PVD Stage 3 : Full-thickness hole w ith focal vitreofoveal detachm ent but absence of a PVD Stage 4 : Fu ll-th ickn ess h ole w ith a PVD

Differential Diagnosis Various t ypes of m acu lar h oles su ch as lam ellar m acular h ole, pseudoh ole, m yopic m acu lar h ole, m icroh ole an d m acular h ole associated w ith epiret in al m em bran e an d ret in al det ach m en t s, an d pseu dom acular h ole

Management Observation Th ere are few st udies describing stage 0 m acular hole, w h ich is a n orm al an d h ealthy retinal m orphology w ith altered vitreoretinal in terface. Stage 0 m acular h ole is a clinically silen t finding detected on optical coheren ce tom ography w here a parafoveal posterior hyaloid separat ion is presen t an d a m in im ally reflective preretin al band is obliquely inserted at one end of th e fovea. A sim ilar fin ding w h en foun d in both th e eyes is associated w ith a sixfold rise in th e incidence of m acular h ole. Th us such cases m ust be follow ed closely an d such patients m ust be coun seled. Surgica l Management Vit rectom y is th e m ain stay in th e m an agem en t of m acular h oles. Surger y for th e t reat m en t of m acu lar h ole revolves aroun d t w o prin ciples: (1) to relieve all vit reoret in al t ract ion by m et iculous rem oval of p osterior cor t ical vit reou s an d (2) to use a tam pon ade to close th e h ole ( Fig. 10.30 ).

10 Surgical Retina

319

Fig. 10.30 “Apple peeling” technique for removal of the internal limiting mem brane (ILM). (1A) The ILM is stained with indocyanine green (ICG) stain. The ILM is grasped with the ILM forceps 500–700 µm above or below the fovea, and a thin strip is peeled radially (arrow) almost to the fovea and released. (1B) This shows the extent of the initial peeled flap of ILM. (1C) The exposed edge is then grasped at its midpoint and a parafoveal strip of ILM is started with a circumferential movement (arrow) around the fovea. (2A) This parafoveal circumferential rhexis is continued (arrow), releasing and regrasping as necessary. (2B) The rhexis halfway around the fovea. (3A) The rhexis approaching a full circle around the fovea as an out ward force vector (arrow) is then intentionally applied so that the ILM strip expands out wardly in a continuous fashion. (3B) Regrasping as necessary, this m aneuver is continued (arrow) until the macular ILM has been removed in a single strip. (3C) The single -piece ILM strip ready for rem oval from the eye, avoiding the need for multiple forceps removals and reinsertions. (4) A conceptual view of the microforceps holding the single-piece removed ILM strip as removed from the retina. Note the unstained area of the retina from which this ILM strip was removed. Light is provided by an endofiberoptic and infusion via a separate infusion port. (Courtesy of Highlights of Ophthalmology, “Retinal and Vitreoretinal Surgery: Mastering the Latest Techniques” English Edition, 2002. Editor-in-Chief: Benjam in F. Boyd, MD, FACS; Co-Editor: Sam uel Boyd, MD.)

320

Color Atlas of Ophthalm ology

Idiopathic Epiretinal Membrane Iw an off w as th e first to describe idiopath ic epiret in in 1865. It is com m on ly fou n d in older age an d h as been called by variou s n am es: Macu lar p ucker Preret in al m acu lar fibrosis Epiret in al fibrosis Ep iret in al gliosis Celloph an e m acu lopathy Surface w rin kling ret in opathy Most of th ese epiret in al m em bran es (ERMs) are idiopath ic an d are called prim ar y epiret in al m em bran es. W h en associated w ith som e oth er ocu lar disorders, th ey are called secon dar y ERM ( Fig. 10.31 ).

Presentation Pat ien ts com plain of slow progressive loss of vision , m ild to severe m et am orph opsia, an d m on ocu lar diplopia. Com plain ts u su ally progress over years. Th ey m ay give som e associated past or t reat m en t h istor y revealing th e cause for th e ERM. Clin ical ch aracterist ics var y w ith th e grade of th e ERM. An early ERM presen ts as an altered n orm al m acular text u re w ith a glistering sh in e on th e surface. It is difficult to detect on oph th alm oscopic exam in at ion . A lit tle m ore con t ract ile m em bran e presen ts as fin e ret in al st riae radiat ing from th e cen ter of th e ERM.

A

B

Fig. 10.31 (A) Fundus picture of patient showing gray-white epim acular membrane. Picture shows distortion of macular retinal vessels. (B) fundus fluorescein angiography (FFA) of the same patient.

10 Surgical Retina

321

Th e t ypical appearan ce of th e blood vessels h elps in th e diagn osis. Dilated ret in al vein s an d vascular tor t uosit y w ith teth ering an d st raigh ten ing of th e vessels are seen directed to th e cen ter of th e con t ract ing m em bran e. Foveal ectopia is a com m on fin ding. A w ide range of oth er associated fin dings are n oted, such as pun ct ate h em orrh ages, m icroan eu r ysm s, profoun d vascu lar leakage an d ret in al edem a, soft exu dates, an d ret in al tear. Macu lar pucker u sually develops after ret in al det ach m en ts. Th ey are opaqu e th ick m em bran es w ith associated fu ll-th ickn ess ret in al folds.

Differential Diagnosis Prim ar y cau ses of ERM: idiopath ic. Secon dar y cau ses of ERM: postocular in flam m at ion , uveit is, ret in al det ach m en t , ret in al vascu lar occlu sion s, m acu lar h oles, post in t raocular su rgeries, post ret in al laser, cr yopexy.

Management Vit rectom y is th e on ly t reat m en t for epiret in al m em bran es. After com plet ing th e vit rectom y, posterior hyaloid th at is adh eren t to th e ret in a is rem oved. In t ravit real t riam cin olon e can be u sed to facilit ate th is step. Th en th e m em bran e is peeled off th e su rface of th e ret in a, p referably from th e cen ter to th e p eriph er y after fin ding an edge of th e m em bran e. Recen tly in t ravit real stain s h ave been described. Tr ypan blue an d in docyan in e green are used to stain th e epiret in al m em bran e an d in tern al lim it ing m em bran e, resp ect ively.

11 Ocular Neoplasms Soosan Jacob , Santosh Hanovar, and Am ar Agarw al

Iris Tumors and Nodules Iris Nevus Iris n evu s presen t s as a sm all (3 × 0.5m m ), w ell-defin ed spot on th e iris. It is com m on ly located in th e st rom a an d presen ts eith er as a t ypical lesion (i.e., w ell-defin ed) or as a diffu se lesion . Th e lesion h as to be differen t iated from oth er pigm en ted lesion s. Cogan -Reese syn drom e is th e com m on associat ion .

Presentation Susp icion for m align an t ch ange is h igh w h en th e n evu s in creases in size an d ch anges color, in creases in vascularit y, rise in in t raocu lar pressu re (IOP), an d th ere is pu pil peaking, ect ropion uveae, an d iris splin t ing. Histopath ological exam in at ion of th e n evu s sh ow s proliferat ion of m elan ocytes, predom in an tly of spin dle cells, th ough occasion ally den drit ic an d balloon cells m ay occu r.

Differential Diagnosis Iris freckle, iris m elan om a, Lisch n odu les, ocular m elan ocytosis

Management Man agem en t con sist s of reassu ran ce of th e pat ien t regarding th e ben ign n at u re of th e lesion an d sim ple obser vat ion for m align an t ch ange after carefu l ph otography an d clin ical docu m en tat ion .

Iris Pigment Epithelial Cyst Iris pigm en t epith elial cyst is rare an d arises from th e epith elium bet w een layers of pigm en t epith elium . It presen t s differen tly depen ding on w h eth er it is located in th e epith eliu m or in th e st rom a.

Presentation W h en it is epith elial in locat ion it can presen t as u n ilateral or bilateral as a solit ar y or m u lt iple lesion s. It is usually asym ptom at ic an d obscures vision on ly w h en large. It is a globu lar lesion located at th e papillar y border or th e m idzon e or th e iris root . It is brow n w h en iris epith elium is involved or t ran sparen t w h en th e iridociliar y epith eliu m is involved. It can get dislodged an d float in th e an terior ch am ber. Lesion s located in th e st rom a are usu ally sm ooth , u n ilateral, solit ar y t ran slucen t lesion s presen t ing in th e first year of life. W h en th ey in crease in size th ey lead to secon dar y glaucom a, w h ich in t urn leads to corn eal decom pen sat ion an d pseudohypopyon .

Differential Diagnosis Iris an d ciliar y body m elan om a

322

11 Ocular Neoplasm s

323

Management Th e n at ure of th e lesion can be ascert ain ed by eith er fin e-n eedle aspirat ion or excision biopsy. If th e t u m or is large it can be t reated w ith argon laser ph otocoagu lat ion or by inject ing eth an ol in to th e lesion w h en it is recalcit ran t .

Iris Melanoma Iris t u m ors con st it u te 8% of uveal t u m ors presen t ing in th e younger age grou p (40 to 50 years) w ith a sligh t fem ale predilect ion . Th ey u su ally h ave a bet ter progn osis th an oth er uveal m elan om as.

Presentation Th e lesion can presen t as t ypical (spot), diffu sely grow ing (diffuse color ch ange), or t apioca m elan om a (m ult iple su rface n odu les). Lesion s are m ostly asym ptom at ic, involving th e in ferior iris m ore com m on ly, bu t can presen t as a rise in IOP, hyph em a, or cat aract . Risk factor for m align an t ch ange is th e sam e as th at for iris n evus.

Differential Diagnosis Iridocorn eal en doth elial (ICE) syn drom e, n evus, aden om a an d ciliar y body t u m or, iris cyst , in t raocular foreign body (IOFB), juven ile xan th ogran u lom a, an d leiom yom a

Management B-scan an d biopsy h elp to con firm th e diagn osis. Biopsy can be fin e n eedle asp irat ion cytology or in cision al biopsy, eith er th rough a corn eal or a lim bal approach . Th e lesion can be obser ved u n t il sym ptom at ic or over t m align an t ch anges occu r for w h ich excision (iridectom y or iridocyclectom y), proton -beam radioth erapy, or rarely en ucleat ion h ave to be u n der taken . Th e progn osis is usu ally good w h en th e lesion is sm all w ith out ciliar y body or ext rascleral exten sion .

Juvenile Xanthogranuloma Juven ile xan th ogran u lom a is a rare idiopath ic gran ulom atou s disease of ch ildh ood du e to proliferat ion of n on -Langerh an s h ist iocytes. It can presen t eith er as cu t an eous lesion s or iris lesion s. Iris in filt rat ion is th e m ost com m on ocu lar m an ifestat ion . It m ay also rarely involve th e orbit , eyelids, corn ea, episclera, ciliar y body, ch oroid, an d opt ic disk.

Presentation Cu tan eous lesion s presen t as yellow ish papu les w ith spon t an eou s regression . Iris lesion s are eith er localized or diffu se. Th e pat ien t m ay presen t as h eteroch rom ia of th e iris. Th e lesion s are usu ally associated w ith spon t an eou s hyph em a. An terior uveit is an d glau com a are oth er less com m on presen t at ion s.

Differential Diagnosis Ret in oblastom a, sarcoid, leukem ia, foreign body, m eduloepith eliom a

324

Color Atlas of Ophthalm ology

Management Treat m en t opt ion s in clude local cor t icosteroids, irradiat ion , an d a com bin at ion of both an d excision .

Leiomyoma (Uveal) Leiom yom a is an ext rem ely rare ben ign lesion arising from th e sm ooth m uscle. Th is is ver y sim ilar to am elan ot ic m elan om a, bu t gen erally it is n ot con fin ed to th e in ferior iris on ly.

Presentation Pat ien ts t ypically presen t w ith a localized, flat to m ildly elevated, ligh tly pigm en ted or n onpigm en ted, vascular lesion in th e area of th e iris sph in cter. Th e lesion m ay also occu r periph erally or in th e an terior ch am ber angle.

Differential Diagnosis Am elan ot ic iris m elan om a, m et astat ic iris lesion s

Management Fin e-n eedle aspirat ion cytology (FNAC) w ith elect ron m icroscopy an d im m un oh istoch em ist r y can diagn ose an d differen t iate th e con dit ion from oth ers. Th ere is n o kn ow n m et astat ic p oten t ial.

Leukemic Iris Nodules Leu kem ic iris n odules are m ore com m on in ch ron ic leu kem ias rath er th an in acu te leukem ia. Iris in filt rat ion can presen t as iris n odules.

Presentation Iris involvem en t m ay be u n ilateral or bilateral. It is a vascularized lesion . It m ay be associated w ith an terior uveit is w ith or w ith out hypopyon . In filt rat ion can also occur in to th e conju n ct iva, orbit , an d opt ic n er ve. Glau com a m ay be p resen t due to blockage in th e filt rat ion from th e t rabecu lar m esh w ork. Leu kem ic ret in opathy du e to eith er direct in filt rat ion or secon dar y to an em ia h as to be looked for.

Differential Diagnosis Ret in oblastom a, uveit is, t rau m a

Management Workup involving an on cologist is p referred. Treat m en t con sists of system ic ch em oth erapy w ith localized radioth erapy. Topical steroids cau se quick bu t tem p orar y resolu t ion . Cen t ral n er vou s system (CNS) evaluat ion is m ust .

Melanocytosis Ocu lar m elan ocytosis is an un com m on , congen ital, prem align an t con dit ion . Nevu s of Ot a is th e com m on varian t follow ed by th e lim ited derm al an d ocu lar form s. Th is is m ore com m on in fem ales an d Asian s.

11 Ocular Neoplasm s

325

Presentation Melan ocytosis presen ts as un ilateral hyperpigm en tat ion of th e face w ith ipsilateral iris hyperch rom ia, iris m elan ocytosis, glau com a (10%), an d lid pigm en t at ion . Th e con dit ion m ay be associated w ith pigm en tat ion of th e ext raocular skin w ith ipsilateral uveal t ract an d m en ingeal involvem en t .

Differential Diagnosis St u rge-Weber syn drom e, racial p igm en tat ion

Management Becau se th e hyperpigm en ted area h as a predisposit ion to develop m align an t m elan om a, regular close follow -u p ever y 6 to 12 m on th s is requ ired. Any suspiciou s lesion sh ould be w orked up accordingly for m align an t m elan om a.

Brushfield Spots (Dow n Syndrome) Br ush field spot s are seen in Dow n syn drom e. Th ey involve th e periph eral st rom a, can be m u lt iple, an d th ey occu r as m u lt iple, pale, ben ign lesion s involving th e periph eral iris st rom a. Th e lesion s appear as pale lesion s. Th ese can also be seen in n orm al in dividu als. Sarcoid n odu les, iris n evu s, or freckles m ust be ru led out . No t reat m en t is required.

Lisch Nodules (Neurofibromatosis) Lisch n odules are u su ally associated w ith n eurofibrom atosis. Th ey are sm all an d m u lt iple. Th ey ap pear sim ilar to iris n evi, w h ich are ben ign an d bilateral. Th ey are presen t in n eurofibrom atosis 1 in all pat ien t s after age 16 years. Th ey con sist of a collect ion of glial cells an d m elan ocytes. Th ey n eed to be differen t iated from iris n evus. Clin ical docum en t at ion an d follow -u p are required.

Ciliary Body Tumors Ciliary Body Melanoma Ciliar y body m elan om as con st it u te ~12% of all m align an cies an d u sually appear bet w een th e ages of 50 an d 60 years.

Presentation Clin ically th ey are asym ptom at ic, alth ough th ey can produce visual sym ptom s occasion ally. Th ey are seen as ciliar y body m ass w ith sen t in el vessels overlying. Th ey can exten d on to th e iris or globe. Len s sublu xat ion , angle-closu re glau com a, spon tan eous hyph em a, secon dar y cat aract , an d an terior uveit is are com m on associat ion s. Ciliary body m elanom as are well detected by ultrasound and biopsy using FNAC.

Differential Diagnosis Ciliar y body aden om a, ciliar y body cyst , uveal effusion syn drom e, m eduloepith eliom a, leiom yom a, an d m et astat ic lesion s

326

Color Atlas of Ophthalm ology

Management Th e t u m ors are am en able to excision w h en sm all. Radioth erapy, eith er as brachyth erapy or p roton beam th erapy, is an oth er opt ion . En ucleat ion is don e if oth er m odalit ies are n ot possible. Progn osis is u su ally w orse.

Medulloepithelioma (of the Ciliary Epithelium) Medulloepith eliom a arises from im m at u re epith elial cells of th e em br yon ic opt ic cu p an d are rare, slow -grow ing t u m ors th at presen t in ch ildren you nger th an 10 years of age, w ith n o sexual predilect ion . On e th ird are ben ign an d t w o th irds are m align an t . Th ey can be eith er teratoid or n on teratoid. Th ey origin ate from th e n onpigm en ted ciliar y epith eliu m predom in an tly involving th e iris an d ret in a, th ough oth ers can also be involved. Metast asis is rare.

Presentation Clin ically pat ien t s can presen t w ith red eye an d decreased vision or w ith an iris m ass lesion w ith a ch ange of color. Tu m or m ay also be seen at th e opt ic disk. Neovascu lar glaucom a, len s colobom a, or sublu xat ion an d cataract are th e com m on com plicat ion s.

Differential Diagnosis Persisten t hyperplast ic prim ar y vit reou s (PHPV), pars plan it is, en doph th alm it is, congen it al glaucom a, ret in oblastom a, m align an t glau com a

Management Ult rasoun d is useful in th e diagn osis. Sm all, w ell-defin ed lesion s of a ben ign n at u re are t reated by iridocyclectom y, an d en ucleat ion is reser ved for t u m ors n ot am en able to oth er t reat m en t m odalit ies.

Choroidal Tumors Choroidal Nevus Ch oroidal n evus is a ben ign m elan ocyt ic t u m or of th e ch oroid an d is fou n d in 5 to 30% of w h ite person s. Th ese are usually foun d in ciden t ally because th ey are asym ptom at ic an d are th e com m on est of all in t raocu lar m align an cies.

Presentation Clin ically th ey are asym ptom at ic but can p resen t w ith a decrease in vision . Lesion s close to th e fovea can cau se vision loss. Th ey are sm all, gray-brow n , h om ogen eou s lesion s. Th e lesion m ay be associated w ith lip ofu scin deposit s an d dru sen , or w ith su bret in al flu id (SRF) is absen t . Th ese lesion s gain im port an ce in th e fact th at th ey h ave to be differen t iated from m align an t m elan om a. Malign an t t ran sit ion is h er-

11 Ocular Neoplasm s

327

Fig. 11.1 Choroidal nevus, fundus photograph shows a pigmented, placoid, well-demarcated choroidal mass with overlying drusen located temporal to the fovea.

alded by th e presen ce of sym ptom at ic lesion s, w ith in creasing th ickn ess, lesion s w ith lipofu scin pigm en t an d SRF, an d an in crease in IOP ( Fig. 11.1 ).

Differential Diagnosis Ch oroidal m elan om a, congen it al hyper t rophy of ret in al pigm en t epith eliu m (RPE), com bin ed h am ar tom a, hyperplasia of RPE, subret in al h em orrh age, ch oroidal h em angiom as, ch oroidal osteom a, an d m etast at ic t um or.

Management Lesion m u st be invest igated w ith fu n dus fluorescein angiograp hy (FFA) an d B-scan to ru le ou t oth er lesion s, especially m align an t m elan om a. Lesion s sh ould be closely m on itored. Any ch anges in th e m orph ology of th e lesion s sh ou ld be alarm ing.

Choroidal Melanoma Ch oroidal m elan om as con st it u te ~80% of uveal m elan om as presen t ing aroun d 50 to 60 years of age. Th ey are classified based on th e size of th e t um or as sm all (15).

Presentation Th ey can presen t as a decrease in vision or field loss, or flash es of ligh t m ay be th e presen t ing feat ures, bu t th ey are oth er w ise asym ptom at ic. Th ey can presen t as an elevated su b-RPE m ass an d can be am elan ot ic. Lipofu scin deposit s, exu dat ive ret in al det ach m en t , an d Bruch m em bran e ru pt ure w ith vit reous h em orrh age are t ypical feat u res of th e lesion . Cert ain t u m ors are diffu se rath er th an being n odular. Pat ien ts at t im es m ay com plain of blurr y vision , visual-field loss, floaters, ph otopsia, an d pain . Oth er associated fin dings can be ret in al detach m en t , angle-closu re

328

Color Atlas of Ophthalm ology

glaucom a, rubeosis iridis, vit reous an d subret in al h em orrh ages, an d len s su blu xat ion s ( Fig. 11.2 ).

Differential Diagnosis Ch oroidal n evus, congen ital hyper t rophy of RPE, com bin ed h am ar tom a, hyperplasia of RPE, subret in al h em orrh age, ch oroidal h em angiom as, ch oroidal osteom a, an d m et astat ic t u m or

A

B

C

Fig. 11.2 (A) Choroidal m elanoma, dome -shaped choroidal mass with moderate intrinsic pigmentation and subretinal fluid (SRF). (B) Choroidal melanoma, dome-shaped choroidal m ass with mild intrinsic pigmentation. (C) Choroidal m elanoma: wide -angle colored fundus photograph shows a dark-pigmented, dome -shaped choroidal mass with a smaller elevated nodule on its surface.

11 Ocular Neoplasm s

329

Management Ch oroidal m elan om as can usu ally be p icked u p by u lt rasoun d as acoust ically h ollow, low in tern al reflect ivit y solid t u m ors associated w ith ch oroidal excavat ion . Ext raglobular exten sion is to be ru led ou t by com pu ted tom ography/m agn et ic reson an ce im aging (CT/MRI) along w ith a system ic w orkup for m et astasis. FNAC is con t rain dicated. Treat m en t guidelin es are given by ocu lar m elan om a st u dy. Tran s-pu pillar y th erm oth erapy can be t ried w h en th e lesion is aw ay from th e disk an d th e m acu la an d w h en th e lesion is pigm en ted. Radioth erapy eith er as plaqu e or as proton beam irradiat ion . Local resect ion can be used w h en th e t u m or is sm all an d th ere is n o m etast asis. In advan ced cases orbital exen terat ion is th e opt ion .

Choroidal Metastasis Ch oroidal m etast asis is th e m ost com m on form of in t raocu lar m align an cy in adu lts, w ith m ore th an 85% of m etast at ic uveal n eoplasm s being ch oroidal. It usually m etast asizes from breast can cer in w om en an d lung can cer in m en . Less com m on sites of origin in clude th e prost ate, th e kidn ey, th e thyroid, an d th e gast roin test in al t ract . Lym ph om as an d leukem ias m ay also m etast asize to th e eye an d th e orbit . Metastases are usu ally bilateral, m u lt iple lesion s.

Presentation Most pat ien t s are asym ptom at ic or m ay com plain of flash es, floaters, or m et am orph opsia. Ch oroidal m etastases are u sually seen as solid, flat , plaqu elike, m ot tled, yellow -brow n lesion s w ith or w ith ou t associated serou s ret in al det ach m en t (Fig. 11.3 ).

Differential Diagnosis Ch oroidal h em angiom a, ch oroidal osteom a, exophyt ic ret in al cap illar y h em an giom a, oth er m etast at ic carcin om as, posterior sclerit is, h em orrh agic RPE det ach m en t , an d am elan ot ic ch oroidal m elan om a

A

B Fig. 11.3 (A) Choroidal metastasis, tallow pink smooth, dome -shaped juxtapapillary choroidal mass. (B) Choroidal m etastasis. Fundus fluorescein angiography (FFA) shows hypofluorescence in the arterial and early venous phases.

330

Color Atlas of Ophthalm ology

Management A com bin ed approach in con su ltat ion w ith th e m edical on cologist an d radiat ion th erapist is requ ired. Th ough ch em oth erapy m ay be in dicated in m any cases, radiat ion th erapy in th e form of extern al beam radioth erapy is usually th e m ore defin it ive t reat m en t . Early t reat m en t offers th e best h ope for preser ving vision . Surger y is rarely required. If th e prim ar y is n ot readily iden t ifiable, a biopsy m ay be required prior to t reat m en t .

Cavernous Hemangioma of the Choroid Cavern ou s h em angiom a is a ben ign vascular h am artom a. Lesion s can be localized or diffuse.

Presentation Cavern ou s h em angiom a is congen it al an d asym ptom at ic u n t il adulth ood, at w h ich t im e it can presen t w ith loss of vision . It can presen t eith er as an isolated, circum scribed lesion w ith ou t system ic associat ion or as a diffu se form along w ith oth er system or ocu lar abn orm alit ies (St urge-Weber syn drom e). Circum scribed lesion s are seen as an elevated, orange-red, ch oroidal m ass. Th e diffuse form is seen as a deep red ch oroid, especially in th e posterior pole com pared w ith n orm al. Oth er feat ures in clu de fibrous ch ange of RPE, tor t u ou s ret in al vessels, cyst ic ch ange, or serou s det ach m en t of ret in a ( Fig. 11.4 ).

Differential Diagnosis Ch oroidal n evus, congen ital hyper t rophy of RPE, com bin ed h am artom a, hyperplasia of RPE, su bret in al h em orrh age, ch oroidal m elan om a, ch oroidal osteom a, an d m etast at ic t u m or

Management Ult rason ography is th e com m on invest igat ion an d sh ow s h igh in tern al reflect ivit y. Fu n dus flu orescein angiography (FFA) sh ow s early hyperflu orescen ce due to in t ra lesion al vessels an d later hyperfluorescen ce of th e w h ole lesion . In docyan in e green (ICG) h elps in delin eat ing th e lesion bet ter th an FFA. MRI h elps in diffuse form . Brain an d system ic w orkup to r ule ou t h em angiom a in oth er sites is essen t ial. Histopath ology reveals cavern ou s vascular ch an n els (n orm al en doth elial cells an d su ppor t ing sept a) an d capillaries like vessels in th e diffuse form .

Fig. 11.4 Choroidal hemangiom a, dome -shaped, juxtapapillary, reddish-orange choroidal mass.

11 Ocular Neoplasm s

331

Treat m en t opt ion s range from ph otodyn am ic th erapy (PDT), TTT, an d irradiat ion . A n eurologist is to be con sulted w h en th ere is CNS involvem en t .

Choroidal Osteoma Ch oroidal osteom a is foun d m ore com m on ly in w om en in th e secon d or th ird decade of life. It m ay be bilateral in 25% of cases.

Presentation Ch oroidal osteom a is seen as a w ell-dem arcated, relat ively flat (less th an 2 m m th ick), yellow -w h ite lesion w ith clu m ps of black or brow n pigm en t on th e su rface, gen erally n ear th e opt ic n er ve h ead. Th e ch oroidal osteom a im pedes th e circu lat ion to th e overlying ret in a an d m ay be associated w ith adjacen t RPE at rophy, su bret in al n eovascularizat ion , su bret in al fluid, an d su bret in al h em orrh age. For t y percen t of cases en large on long-term follow -u p. FFA sh ow s early, patchy hyperflu orescen ce w ith in ten se late stain ing. Ult rasou n d sh ow s in ten se reflect ivit y from th e t um or w ith acou st ic sh adow ing of th e posterior orbit al con ten t s. CT scan sh ow s a bon elike sign al ( Fig. 11.5 ).

Differential Diagnosis Ch oroidal n evus, congen ital hyper t rophy of RPE, com bin ed h am artom a, hyperplasia of RPE, su bret in al h em orrh age, ch oroidal m elan om a, ch oroidal osteom a, an d m etast at ic t u m or

A

B

Fig. 11.5 (A) Choroidal osteoma: a well-defined, yellow-orange, placoid juxtapapillary choroidal mass with scalloped margins. (B) Choroidal osteoma: fundus fluorescein angiography (FFA) shows diffuse hypofluorescence in the arterial phase. (C) Choroidal osteoma: fluorescence intensifies diffusely in the late phase.

C

332

Color Atlas of Ophthalm ology

Management Man agem en t con sists of periodic obser vat ion for grow th an d su bret in al n eovascu larizat ion . Su bret in al n eovascularizat ion m ay be t reated w ith laser.

Retinal Pigment Epithelium Tumors Congenital Hypertrophy of the Retinal Pigment Epithelium Congen it al hyper t rophy of th e RPE is gen erally discovered during rout in e eye screen ing an d h as an associat ion w ith fam ilial aden om atou s polyposis an d Gardn er syn drom e (in test in al polyposis, h am ar tom a of th e skeleton , an d m u lt iple soft t issue t u m ors).

Presentation Lesion s are gen erally seen in th e perip h er y bu t m ay occasion ally be seen n ear th e disk. Th ey occu r as rou n d, w ell-circu m scribed, pigm en ted lesion s, eith er solit ar y or m ult iple w ith flat or scalloped m argin s. Th ey h ave a m ore dist in ct border an d are darker th an th e ch oroidal n evu s. Th ere m ay be depigm en ted h alos w ith in th e lesion . Rarely, th ere are m u lt ip le pigm en ted lesion s in th e sam e area (bear-t rack lesion s) ( Fig. 11.6 ).

Differential Diagnosis Ch orioret in al scarring, ch oroidal m elan om a, ch oroidal n evu s, m elan ocytom as of th e ch oroids, hyp erplasia of th e RPE, posth em orrh age h em osiderin deposits

Management Screen ing is perform ed by a gast roen terologist an d is follow ed u p by periodic ocu lar screen ing.

Fig. 11.6 Congenital hypertrophy of the retinal pigm ent epithelium, fundus photograph of the deeply pigmented, flat, well-circum scribed retinal lesion with characteristic halo and lacunae of depigmentation.

11 Ocular Neoplasm s

333

Fig. 11.7 Combined hamartoma of the retinal pigm ent epithelium and sensory retina, grayish juxtapapillary placoid mass with variable pigmentation and intrinsic vascularit y. Note the altered configuration and dragging of retinal vessels with partial obscuration by fibroglial tissue.

Combined Hamartoma of the Retinal Pigment Epithelium and the Retina Th is rare, ben ign t u m or is form ed by a h am ar tom atou s overgrow th of several con st it uen ts su ch as th e RPE, vascu lar elem en t s, an d glial t issu e of th e ret in a to var ying degrees.

Presentation Pain less visu al loss is th e m ost com m on sym ptom . Ret in al vascu lar tor t uosit y, hyperpigm en t at ion , elevat ion of th e lesion , an d an epiret in al m em bran e m ay be fou n d. Macu lar fu n ct ion m ay be affected secon dar y to t ract ion al distort ion of th e ret in a. Com bin ed h am ar tom as are m ost com m on ly located close to th e opt ic disk follow ed by th e m acu la an d occu r least com m on ly in th e periph eral ret in a. It h as been described to occur in n eu rofibrom atosis 1 ( Fig. 11.7 ).

Differential Diagnosis Pigm en ted fu n dus t um ors, ret in oblastom a, ch oroidal m elan om a

Management Angiography m ay sh ow vascu lar ch anges periph eral to th e lesion referred to as “p seu doavascularit y.” Vit rectom y m ay be at tem pted in selected cases.

Retinal Tumors Retinoblastoma Ret in oblastom a is th e m ost com m on prim ar y ocular m align an cy of ch ildh ood, w ith an in ciden ce of 1 in 15,000 live bir th s. It is cau sed by m u tat ion in th e long arm of ch rom osom e 13q14, w h ich codes for th e RB1 gen e, a t u m or su ppressor gen e. It affects th e p recu rsor cells th at form th e in n er an d outer ret in al cells, leading to th eir m align an t t ran sform at ion .

334

Color Atlas of Ophthalm ology

Th e m ode of in h eritan ce m ay be h eritable (germ -lin e m ut at ion ) or n on h erit able (som at ic m ut at ion ). In h eritable m u tat ion on e allele of th e RB1 gen e is m u tated in all cells of th e body. A “secon d h it” affect s th e secon d allele, leading to m align an t t ran sform at ion . Th ese pat ien t s are predisposed to n on ocu lar t u m ors such as pin ealom a (t rilateral ret in oblastom a), osteosarcom a, an d m align an cies of th e brain an d lung.

Presentation In bilateral cases presen t at ion is usu ally arou n d age 1 year an d in un ilateral cases 2 years. Leu kocoria (w h ite pupillar y reflex, cat’s eye reflex) is th e com m on est sign . St rabism u s, secon dar y glaucom a, proptosis, an d in flam m at ion secon dar y to t u m or n ecrosis are oth er sign s th at can be seen . It can som et im es m im ic uveit is in ch ildren , presen t ing as a m asquerade syn drom e. Ocu lar exam in at ion by scleral in den t at ion sh ow s a w h ite m ass w ith calcificat ion . It m ay be in t raret in al or an en dophyt ic t um or project ing an d seeding in to th e vit reous or m ay be exophyt ic w ith a subret in al m ass an d overlying ret in al det ach m en t . Histopath ology sh ow s th e ch aracterist ic Flexn er-Win terstein er roset tes, Hom er Wrigh t roset tes, an d fleu ret tes.

Management A th orough system ic an d gen et ic evalu at ion is required. Ult rason ography an d CT assess t um or size an d calcificat ion . MRI detect s opt ic n er ve, ext raocu lar exten sion , an d pin ealoblastom a. Prim ar y en ucleat ion is advised for un ilateral cases w ith advan ced disease an d large t um ors, longstan ding ret in al detach m en ts, n eovascu lar glau com a, or suspicion of opt ic n er ve invasion /ext rascleral exten sion . It is also u sed in bilateral cases for th e m ore advan ced eye. Treat m en t m odalit ies available in clu de ph otocoagu lat ion , ch em oth erapy, t ran sp upillar y th erm oth erapy, cr yoth erapy for sm all t um ors an d extern al-beam radioth erapy (EBRT), an d p laqu e brachyth erapy for m edium -sized t u m ors. EBRT m ay be associated w ith developm en t of n on ocular t u m ors in th e irradiated field. Ch em oth erapy m ay be u sed prior to oth er t reat m en t m odalit ies to decrease th e size of th e t um or or in pat ien ts w ith system ic m etast asis.

Capillary Hemangioma Th is is a ben ign h am ar tom a of th e ret in al (or opt ic disk) vasculat ure, con sist ing of capillar y-like vessels. Th ough m ost com m on ly diagn osed in you ng adult s, it m ay p resen t in any age. Isolated lesion s are n ot associated w ith any system ic in volvem en t , w h ereas von Hippel-Lin dau disease presen ts w ith bilateral/m u lt iple t u m ors.

Presentation Pat ien ts can be asym ptom at ic (diagn osed on fam ily screen ing) or m ay p resen t w ith a decrease in visual acu it y. On exam in at ion it is seen as a red n odular lesion w ith tort uosit y an d dilatat ion of th e feeding ar ter y an d drain ing vein w ith or w ith ou t exudat ion , exudat ive ret in al detach m en t , rubeosis/n eovascular glaucom a, epiret in al m em bran es, t ract ion al ret in al detach m en t , an d vit reou s h em orrh age ( Fig. 11.8 ).

11 Ocular Neoplasm s

335

Fig. 11.8 (A) Retinal capillary hemangioma: montage of fundus photographs shows a well-defined, intense, orange-red retinal lesion with prom inent feeder and drainage vessels: (B) Retinal capillary hemangioma, higher magnification demonstrates intense vascularit y of the lesion. (C) Optic disc capillary hemangiom a: fiery red vascular mass located over the optic nerve head.

A

C

B

Differential Diagnosis Coat s disease, racem ose h em angiom a, cavern ous h em angiom a, en doph th alm it is, ret in oblastom a, ast rocytom as, fam ilial exudat ive vit reoret in opathy (FEVR), sickle cell ret in opathy, p apilledem a, an d papillit is

Management FFA reveals rapid sequen t ial filling of th e feeder arter y, h em angiom a, an d vein w ith exten sive late leakage. Leakage in to th e vit reou s m ay m ake late im ages h azy. System ic disease n eeds m u lt idiscip lin ar y care. Ocu lar diseases can be m an aged w ith ph otocoagu lat ion (0.8 m m an isocoria sign ifican t). Noradren alin reu ptake is blocked by cocain e cau sing dilatat ion . In Horn er syn drom e n o n oradren alin is p resen t at th e n er ve en dings. Hydroxyam phetam ine test : Hydroxyam ph etam in e (1%) is in st illed in both eyes. In preganglion ic lesion s both pu pils w ill dilate an d in postganglion ic lesion s th e Horn er pu pil w ill n ot dilate. Noradren alin e release is poten t iated by Paredrin e (hydroxyam ph et am in e) causing dilat at ion . In postganglion ic lesion s n eu ron s are dest royed so n o dilatat ion is seen .

13 Neuro-Ophtham ology 377

A

Fig. 13.8 (A) Horner syndrom e. (B) Pharm acological tests to localize Horner syndrom e.

B

378

Color Atlas of Ophthalm ology

Adrenaline test : Adren alin e 1:1000 is in st illed in both eyes. No dilatat ion is seen in preganglion ic lesion s, bu t dilat at ion of a Horn er pu pil is presen t in postgan glion ic lesion s because adren alin e is n ot broken dow n becau se of th e absen ce of m on oam in e oxidase (MAO) in h ibitor in dest royed n er ve en dings. Th is is du e to “den er vat ion hypersen sit ivit y” to adren ergic n eu rot ran sm it ter. Other investigations: Magnetic resonance im aging (MRI) of the brain/spinal cord/ orbit; com puted tom ography (CT) of the thorax; Doppler of the carotid; ear, nose, and throat (ENT) assessm ent; chest x-ray. Treatm ent depends on the exact cause and site of the lesion. The patient is referred to a suitable specialist.

Argyll-Robertson Pupil Th is con dit ion is u su ally seen in pat ien ts suffering from syph ilis. It sh ow s a bilateral an d asym m et rical involvem en t . Lesion s u sually affect th e n eu ron s of th e pretectal area.

Presentation Sign s in clu de bilateral, m iot ic, irregular pup il w ith asym m et rical respon se to ligh t an d n ear st im ulu s for accom m odat ion . Ligh t reflex is absen t an d accom m odat ion is ret ain ed. Th e m ain associated causes are tert iar y syph ilis, diabetes m ellit u s, Wern icke en ceph alopathy, en ceph alit is, h eredit ar y n eu ropath ies, m idbrain t um ors, h erpes zoster oph th alm icu s, an d ch ron ic alcoh olism .

Management Man agem en t addresses th e u n derlying cau se.

Adie Pupil/Tonic Pupil Adie ton ic pu pil result s from dam age to th e postganglion ic parasym path et ic n er ve su pply to th e sp h in cter pu pillae. Th is is seen in young adult s an d is un ilateral in 80%.

Presentation Sign s in clude a large, regu lar pu pil w ith poor ligh t react ion an d exaggerated, slow, su st ain ed n ear respon se (ton ic) w ith ch aracterist ic verm iform m ovem en t s of th e pupillar y border on slit lam p. Ligh t-n ear dissociat ion is p resen t . In ch ron ic cases, th e pup il becom es poor an d poorly dilat ing. Th is con dit ion is associated w ith dim in ish ed deep ten don reflexes (Adie syn drom e) an d patchy hypoh idrosis (Ross syn drom e).

Management Invest igat ion s in clu de ph arm acological test s (e.g., in st illat ion of 0.125% pilocarpin e in both eyes). An Adie pu pil con st ricts, w h ereas th e n orm al pu pil does n ot becau se of parasym path et ic den er vat ion supersen sit ivit y. Reassu re th e pat ien t about any obvious an iscoria an d accom m odat ive dysfu n ct ion . Pilocarp in e (0.1%) h elps in redu cing th e m ydriat ic blu rring. Glasses also h elp w ith accom m odat ive dysfu n ct ion .

13 Neuro-Ophtham ology 379

Fig. 13.9 Arterial loop. Color photograph shows a tortuous vessel above the optic disk. (Courtesy of Robin D. Hamilton, A.M. Hamilton)

Developmental Optic Nerve Anomalies Prepapillary Vascular Loop These blood vessels project from the optic disk into the vitreous and return to the optic disk to continue their usual course; 80 to 90%are arterial in origin (Fig. 13.9 ).

Presentation Prepapillar y vascu lar loop is an in ciden t al fin ding.

Optic Nerve Hypoplasia Th is con dit ion is ch aracterized by a decreased n u m ber of opt ic n er ve axon s an d n orm al m esoderm al an d glial supp or t ing t issue.

Presentation Visual acu it y m ay range from 20/20 to percept ion of ligh t . Op h th alm oscopically th e disk appears sm all an d pale. A peripapillar y h alo surroun ded by a pigm en t ring (dou ble-ring sign ) is alw ays presen t . De Morsier syn drom e is a com bin at ion of opt ic n er ve hypoplasia, absen ce of sept um pellu cidu m , an d agen esis or par t ial developm en t of th e corpus callosum . Coexisten t h em isph eric abn orm alit ies an d absen ce of pit uit ar y in fun dibulu m w ith or w ith ou t post pit u it ar y ectopia m ay be presen t . Pit uitar y h orm on e deficien cy m ay be p resen t .

Management Man agem en t con sist s of n eu rological an d en docrin ological evalu at ion s w ith su pplem en tat ion of requ ired h orm on es.

380

Color Atlas of Ophthalm ology

Optic Nerve Pit Opt ic n er ve pit is h ern iat ion of rudim en t ar y n eu roectoderm al t issu e in to a pocketlike depression w ith in th e n er ve substan ce w ith u n kn ow n p ath ogen esis.

Presentation A roun d or oval gray-w h ite or yellow ish depression is seen in th e opt ic disk, preferably at th e tem poral m argin . Associated visu al field defects m ay be presen t , m ost com m on ly paracen t ral arcuate scotom a. Th ere is a 45% risk of serous ret in al detach m en t .

Differential Diagnosis Acquired depression in th e opt ic disc in n orm al ten sion glau com a pat ien t s, opt ic n er ve colobom a, cen t ral serou s ret in opathy (CSR), age-related m acular degen erat ion , pigm en t ep ith eliu m det ach m en t

Management Pat ien ts sh ou ld be given regular follow -u p. Laser p h otocoagu lat ion is p erform ed at th e peripapillar y ret in a adjacen t to th e pit if serou s ret in al det ach m en t is presen t . In tern al gas t am pon ade is recom m en ded for ret in al detach m en t .

Optic Disk Coloboma Opt ic disk colobom a is a sp oradic or au tosom al dom in an t con dit ion arising from fau lt y closure of th e em br yon ic opt ic cup.

Presentation Visual acuit y is variably affected. Th ere is visu al-field defect depen ding on th e n er ve fiber involvem en t . An en larged, w h ite, glisten ing, bow l-sh aped disk w ith cen t ral excavat ion , w h ich m ay exten d to th e ret in a an d ch oroid. Microoph th alm ia m ay presen t . Associat ion s in clu de CHARGE syn drom e ( colobom a of iris or ret in och oroid, h eart an om aly, ch oan al at resia, ret ardat ion , gen ital an om alies, e ar an om alies), Golt z focal derm al hypoplasia, Walker-Warburg syn drom e, lin ear n evus sebaceous syn drom e, an d Golden h ar syn drom e. Opt ic disk colobom a is rarely associated w ith t ran ssph en oidal en ceph alocele ( Fig. 13.10A).

Management Visual-field test ing m ust be don e. Obser vat ion is n eeded.

Morning Glory Syndrome Morn ing glor y syn drom e is due to a fun n el-sh aped expan sion of th e distal por t ion of th e opt ic stalk, w h ich is due to n orm al closure bu t abn orm al progression of closu re of th e em br yon ic fissure in th e distal por t ion of th e opt ic st alk. It is sporadic in occurren ce ( Fig. 13.10B).

13 Neuro-Ophtham ology 381

A

B Fig. 13.10

(A) Coloboma of the optic disk. (B) Morning glory syndrome. (Courtesy of

Robin D. Hamilton, A.M. Hamilton)

Presentation Visual acu it y m ay be variably affected. Oph th alm oscopy reveals an en larged, p in k disk sit u ated in a fun n el-sh aped excavat ion w ith overlying glial t issu e. Ret in al blood vessels are in creased in n u m ber, arise from th e disk periph er y, an d ru n an abn orm ally st raigh t cou rse w ith acu te bran ch ing. Th e m acu la m ay be in corporated in th e postglial excavat ion (m acular capt u re). Th ere is an in creased risk of ret in al detach m en t . Associat ion s in clu de t ran ssph en oidal en ceph alocele, ipsilateral in t racran ial vascu lar hyp oplasia, hypopit u it arism , an d facial abn orm alit ies-hypertelorism , depressed n asal bridge, an d m idlin e u pper lip n otch cleft palate.

Management Man agem en t con sists of regular follow -up an d pat ien t assu ran ce.

Tilted Disk Tilted disk is bilateral, n on h ereditar y, presum ably du e to ect asia of th e in feron asal fun du s.

Presentation Pat ien ts p resen t w ith an obliqu ely placed disk w ith a posteriorly displaced in feron asal port ion an d sit us inversu s of th e vessels. Bilateral h em ian opia th at does n ot resp ect th e ver t ical m eridian is seen . Myopic ast igm at ism is p resen t . Th e con dit ion is associated w ith visu al-field defects. It m ay be associated w ith congen it al su prasellar t u m or.

Management Man agem en t con sists of correct ion of th e refract ive error an d pat ien t assu ran ce.

382

Color Atlas of Ophthalm ology

Optic Disk Pathology Optic Neuritis In flam m at ion of th e opt ic n er ve m ay be a ret robu lbar n eu rit is w ith n orm al opt ic disk (m ost frequen tly m ult iple sclerosis, young adult s), papillit is w h ere th e disk is sw ollen (m ost com m on ly in ch ildren ), or n eu roret in it is-associated in flam m at ion of ret in al n er ve fibers w ith m acu lar st ar form at ion . Dem yelin at ing opt ic n eu rit is is due to ph agocytosis of th e m yelin n er ve sh eath s an d su bsequ en t lay-dow n of fibrou s plaqu es. It affect s th e w h ite m at ter t racts, brain stem , an d spin al cord, bu t th e periph eral n er ves are spared.

Presentation Presen tat ion is u su ally at age 20 to 30 years an d is subacute an d u n ilateral. Th ere is decreased vision , pain exacerbated by eye m ovem en ts, an d t iny flash es of ligh t (ph osph en es). Visu al acuit y usually varies bet w een 20/60 (6/18) to 20/200 (6/60) in ret robulbar n eurit is an d papillit is. Afferen t pu pillar y defect , dysch rom atopsia (n ot proport ion al to visu al loss), dim in ish ed ligh t an d brigh t n ess appreciat ion , an d decreased con t rast sen sit ivit y are presen t . Th ere is diffuse depression of th e cen t ral 30 degrees of th e visu al field. Dem yelin at ing diseases causing ocular involvem en t in clude th e follow ing: Mult iple sclerosis: Most com m on . MRI sh ow s p eriven t ricu lar plaques. Devic disease (neurom yelit ica opt ica) : Bilateral opt ic n eurit is w ith t ran sverse m yelit is. Severe bilateral dim in ish ed visual acuit y w ith paraplegia. Schilder disease : On set before 10 years an d death w ith in 1 to 2 years. Bilateral, progressive opt ic n eurit is.

Differential Diagnosis Com pressive opt ic lesion s, sarcoidosis, vascu lit is (e.g., system ic lu pus er yth em atosu s), syph ilis, an terior isch em ic opt ic n europathy (AION), Leber’s h ereditar y opt ic n europathy (LHON), toxic or n ut rit ion al n eu ropath ies, post viral dem yelin at ion

Management Presentation Presen tat ion is u sually at age 20 to 30 years an d is su bacu te an d u n ilateral. Diagn osis is usu ally clin ical. Th ere is decreased vision , p ain exacerbated by eye m ovem en ts, an d t iny flash es. In th e case of an at ypical presen t at ion , progressive opt ic n europ athy n eeds to be ru led ou t . Invest igat ion s requ ired in clude MRI, ch est x-ray, serological tests an t in u clear an t ibody (ANA), er yth rocyte sedim en t at ion rate, syph ilis, an t in eut roph il cytoplasm ic an t ibodies (ANCA), an d cerebrospin al fluid (CSF) an alysis. Typ ical w h ite m atter plaques in MRI w ith oligoclon al ban ds in CSF suggest a dem yelin at ing cau se. Treat m en t m odalit ies in clude in t raven ou s m ethylpredn isolon e 1 g daily for 3 days follow ed by oral predn isolon e 1 m g/kg/day for 11 days, th en t apered over 3 days. Th e resu lts of th e ONTT (opt ic n eu rit is t reat m en t t rial) sh ow th at th e above regim en h asten s visual recover y, but does n ot affect long term visu al outcom es. In t ram u scular in terferon β-1a at th e first episode delays th e developm en t of m u lt iple sclerosis.

13 Neuro-Ophtham ology 383

Band Optic Atrophy Also called bow t ie at rophy, th is is a kin d of prim ar y opt ic at rophy seen w ith ch iasm al syn drom e w ith bitem poral h em ian opic field defects in th e ipsilateral eye, an d opt ic t ract lesion s in th e con t ralateral eye.

Presentation Opt ic disk pallor is seen prim arily at th e n asal an d tem poral p or t ion s of th e opt ic disk. Sup erior an d in ferior port ion s are n ot involved because of sparing of th e su perior an d in ferior bu n dles of arcuate fibers.

Differential Diagnosis Oth er causes of opt ic at rophy

Management MRI an d n eu rological evaluat ion s are a m u st . Treat m en t is in collaborat ion w ith a n eurologist an d n eu rosurgeon .

Papilledema Papilledem a is bilateral disk sw elling due to raised in t racran ial ten sion (ICT), w h ich is t ran sm it ted along th e subarach n oid space via n er ve sh eath s to block th e axoplasm ic flow. Causes in clude in t racran ial t um ors, pseu dot u m or cerebri, subdu ral an d epidural h em atom as, subarach n oid h em orrh age, brain abscess, sagit tal sin us th rom bosis, an d posterior fossa t u m ors (in ch ildren ).

Presentation Pat ien ts presen t w ith t ran sien t visual obscu rat ion s of a few secon ds, w h ich m ay be accen t u ated w ith post u re or st rain ing, h eadach e, n au sea, vom it ing, dou ble vision , an d decreased vision . Bilateral asym m et ric disk sw elling, disk hyperem ia w ith dilated capillaries, dilated vein s, loss of spon t an eou s ven ous pu lsat ion s, splin ter h em orrh ages, cot ton -w ool spots, obscurat ion of th e blood vessels en tering an d leaving th e disk, an d con cen t ric ret in al folds (Paton lin e) form th e h allm arks of th e acute papilledem a. Elevat ion of th e disk w ith out h em orrh ages (ch am pagn e cork disks), decreased ven ous dilat at ion , an d even t u al at rophy of th e disk w ith a decrease in th e disk elevat ion over a period of 6 w eeks is ch aracterist ic of ch ron ic papilledem a. En largem en t of a blin d spot is seen in field exam in at ion ( Fig. 13.11 ).

Fig. 13.11

Papilledema.

384

Color Atlas of Ophthalm ology

Differential Diagnosis In t racran ial space-occu pying lesion s (t um ors, h em orrh age, abscess, etc.), in filt rat ive (leukem ia, lym ph om a), in flam m ator y (opt ic n eurit is, sclerit is, etc.), gran ulom atou s (sarcoidosis, t u bercu losis, etc.), an d vascu lar causes (AION, diabet ic papillopathy, etc.) of disk sw elling an d pseudopapilledem a

Management Fundus fluorescein angiography (FFA) show s leakage from dilated disk capillaries, and late pooling of the dye is evident. Magnetic resonance venography is done to check the cerebral sinuses and lum bar puncture for CSF analysis. Treatm ent is directed tow ard the underlying cause; neurosurger y m ay be required. Regular ophthalm ic follow -up w ith disk status, vision, color vision, and field changes is required.

Pseudopapilledema An om alies resem ble papilledem a but are n ot du e to raised ICT. Th ey can in stead be du e to disk drusen , t ilted disks, hyperopic disks, m yopic disks, an d m yelin ated peripapillar y n er ve fibers. Disk drusen is autosom al dom in an t , bilateral lum py disk w ith absen t cu p an d cen t rally em erging vessels w ith an abn orm al bran ch ing pat tern . Visu al acuit y is u su ally n orm al bu t associated field defects are usually presen t . Diagn osis is by autoflu orescen ce, B-scan , ult rason ography, an d CT scan . Sim ilarly m yopic disks m ay be elevated n asally an d m ay leak on FFA. Hyperopic disk is crow ded an d elevated an d th us can lead to con fu sion . Tilted disks are elevated su perotem porally ( Fig. 13.12 ).

Pseudotumor Cerebri Pseu dot u m or cerebri is usually a ben ign , self-lim it ing disease of un kn ow n et iology ch aracterized by sign s an d sym ptom s of raised in t racran ial pressu re w ith n orm al CSF, pap illedem a, an d n orm al n er vous system im aging. It can be idiopath ic an d is kn ow n to be associated w ith vit am in A in toxicat ion , tet racyclin e th erapy, steroid w ith draw al, n alidixic acid, im pairm en t of cen t ral ven ous drain age, an d system ic lupu s er yth em atosu s.

Fig. 13.12 Optic nerve head drusen. (Courtesy of Robin D. Hamilton, AM Hamilton)

13 Neuro-Ophtham ology 385

Presentation Presen t ing an d diagn ost ic feat ures of pseu do t u m or cerebri are h olocran ial h eadach e, m on ocu lar or bin ocu lar blackout s of vision (du e to m icrocirculat ion s dist u rban ce at th e tem poral lobe), sixth -n er ve palsy w ith diplopia, t in n it u s, papilledem a, arcu ate scotom as in th e n asal region , an d relat ive afferen t pu pillar y defect (RAPD).

Differential Diagnosis Brain t um ors (m ost com m on ly gliom a), ar terioven ous m alform at ion s, in fect iou s diseases su ch as viral en ceph alit is, an d an om alous opt ic n er ve w ith h eadach e

Management Asym ptom at ic p at ien ts sh ould be follow ed up regu larly. Carbon ic an hydrase in h ibitors (in h ibit th e CSF produ ct ion ), cor t icosteroids, oral hypoglycem ic agen ts, lum boperiton eal sh u n t , an d opt ic n er ve sh eath decom pression (ONSD) are oth er t reat m en t m odalit ies.

Arteritic Ischemic Optic Neuropathy (Giant Cell Arteritis) Th is in farct ion of th e opt ic disk is associated w ith gian t cell arterit is (GCA). Tw en t y percen t of cases do n ot h ave any system ic sign s of GCA at th e t im e of presen t at ion (occult GCA).

Presentation Th e pat ien t presen ts w ith sudden , profou n d, u n ilateral visual loss w ith periocu lar pain (preceded by t ran sien t visu al loss an d flash es of ligh t u n like n on arterit ic isch em ic opt ic n europathy. Alt it u din al field defect is com m on in th ese pat ien ts. Pat ien ts m ay experien ce associated sym ptom s su ch as h eadach e, n eck pain , jaw pain w ith claudicat ion , scalp ten dern ess, fevers, w eigh t loss, an d m yalgias. Th e age of th e pat ien t is m ore th an 55 years. Palp able an d ten der tem poral ar ter y, elevated er yth rocyte sedim en t at ion rate, an d coexist ing an em ia are also presen t .

Differential Diagnosis Non ar terit ic isch em ic opt ic n eu ropathy, opt ic n eu rit is, opt ic n er ve com pression , opt ic n europathy, cen t ral ret in al vein occlu sion (CRVO).

Management ESR, C-react ive protein (CRP), an d tot al blood cou n t-raised platelet s are th e blood invest igat ion s requ ired to diagn ose an d follow up w ith th e pat ien t . Tem p oral arter y biopsy sh ow s gran ulom atou s in flam m at ion w ith a grossly n arrow lu m en . FFA sh ow s severe hypoperfu sion of ch oroid. Pat ien ts are t reated w ith in t raven ous m ethylpredn isolon e 1 g/day w ith 80 m g oral predn isolon e for 3 days. After 3 days, 60-m g an d 50-m g oral dose each for 1 w eek follow ed by gradu al redu ct ion of 5 m g each w eek t ill 10 m g/day, w h ich is th e m ain ten an ce dose. Azath ioprin e h as been t ried in pat ien ts in toleran t of steroids. Progn osis is poor in spite of early st art of steroids, an d it w orsen s w ith t im e an d su bsequen t episodes.

386

Color Atlas of Ophthalm ology

A

B Fig. 13.13 (A) Nonarteritic ischemic optic neuropathy (diabetic), color picture. (B) Nonarteritic ischemic optic neuropathy (diabetic), fundus fluorescein angiography.

Nonarteritic Ischemic Optic Neuropathy Th is n europathy occurs becau se of occlusion of th e sh or t posterior ciliar y ar teries. It is m ost com m on in elderly pat ien ts, w ith st ruct ural crow ding of th e opt ic n er ve h ead du e to a sm all or absen t physiological cu p. System ic hyper ten sion , hyperch olesterolem ia, diabetes m ellit u s, hypercoagulat ion st ates, an d silden afil in take are som e of th e predisposit ion s for th e con dit ion .

Presentation Pat ien ts h ave a sudden un ilateral pain less visu al loss, par t icularly after aw aken ing, associated w ith color vision abn orm alit ies (propor t ion al to visu al loss) an d in ferior alt it u din al field defects. Late stages p resen t w ith opt ic at rophy follow ing ch ron ic vision loss. Disk pallor w ith disk edem a (diffu se or sectoral) w ith peripapillar y splin ter h em orrh ages is th e t ypical presen t at ion ( Fig. 13.13 ).

Differential Diagnosis AION, opt ic n eu rit is, opt ic n er ve com pression , opt ic n europathy, an d CRVO

Management Lipid profile, blood glu cose, com plete blood cou n t , an t in uclear an t ibody, flu orescein t repon em a an t ibody (FTA)/ven ereal disease research laborator y test , ESR, an d C-react ive protein (to rule ou t GCA) m ust be don e for all pat ien t s. FFA sh ow s early disk hyperflu orescen ce, w h ich in creases in in ten sit y in th e late st ages. In term itten t blocked fluorescen ce is seen ow ing to splin ter h em orrh ages. Treat m en t con sists of m an aging th e u n derlying m edical con dit ion . Progn osis is favorable w ith m ain ten an ce of good visu al acu it y in m ost pat ien ts.

Toxic or Nutritional Optic Neuropathy Th is con dit ion is also referred to as tobacco-alcoh ol am blyopia because it is prevalen t in h eavy drin kers an d ch ron ic pip e or cigar sm okers w h o n eglect th eir diet . Oth er et iologies in clude th iam in e an d vit am in B12 deficien cy, m eth an ol, eth am butol, ch loram ph en icol, ison iazide, rifam picin , lead, an d digit alis.

13 Neuro-Ophtham ology 387

Presentation Presen t at ion in cludes in sidious, progressive, bilateral visual im pairm en t w ith dysch rom atopsia. Th e opt ic disk m ay be n orm al or tem poral pallor w ith splin ter h em orrh ages arou n d th e disk. Cen t rocecal scotom a (bet ter appreciated by red target in stead of w h ite) is presen t .

Differential Diagnosis Bilateral AION, hypoten sive sh ock, radiat ion inju r y, in filt rat ive opt ic n europathy, Leber opt ic n europathy, an d bilateral com pressive opt ic n europathy

Management Blood invest igat ion s m igh t reveal an associated pern icious an em ia an d vit am in B12 deficien cy. Weekly inject ion s of 1000 un its of hydroxycobalam in for 10 w eeks along w ith m ult ivitam in s are th e m ain stay of th e t reat m en t . Abstain from drin king an d sm oking. Progn osis is good w ith t reat m en t in th e early st ages.

Leber Optic Neuropathy Leber opt ic n eu ropathy is a m atern ally t ran sm it ted t rait arising from p oin t m ut at ion s (m ost com m on ly 11778 m ut at ion ) in m itoch on drial DNA. It t ypically affect s m en (3:1) in th eir t w en t ies or th irt ies. It is u n ilateral in it ially w ith subsequ en t involvem en t of th e oth er eye.

Presentation Pat ien ts presen t w ith pain less un ilateral gradual loss of vision . Th e oth er eye is involved su bsequen tly. Dysch rom atopsia is presen t . Th ere is a m ildly edem atou s, hyperem ic disk w ith telangiect at ic vessels in th e peripapillar y ret in a. Th e late stage presen t s w ith opt ic at rophy. Den se cen t ral or paracen t ral scotom a is seen . Th e con dit ion m ay be associated w ith sp ast icit y an d gait dist urban ces ow ing to th e in abilit y to detoxify cyan ide.

Differential Diagnosis Bilateral AION, hypoten sive sh ock, radiat ion inju r y, in filt rat ive opt ic n europathy, n ut rit ion al opt ic n europ athy, an d bilateral com pressive opt ic n europathy

Management A detailed h istor y h elps in ru ling ou t oth er causes of opt ic n europ athy. CT scan an d MRI scan h elp to rule ou t a com pressive lesion . Con sider a blood test to detect m itoch on drial ch rom osom al m u t at ion s. Gen et ic coun seling h elps.

Compressive Optic Neuropathy Com pressive opt ic n eu ropathy result s from com pression of th e pregen iculate port ion of th e opt ic n er ve. Com m on causes in clude Graves thyroid oph th alm opathy, m en ingiom as, orbit al space-occu pying lesion , pseu dot u m or, cran ioph ar yngiom a, an d pit uitar y t u m ors.

388

Color Atlas of Ophthalm ology

Presentation Visual acuit y, color vision , an d con t rast sen sit ivit y are u sually affected. In it ially disk sw elling is presen t , follow ed by at rophy in th e late st ages. Afferen t pu pillar y defect an d cen t ral an d arcu ate scotom as are com m on fin dings. Proptosis m ay or m ay n ot be associated.

Differential Diagnosis Bilateral AION, hypoten sive sh ock, radiat ion inju r y, in filt rat ive opt ic n europathy, n ut rit ion al opt ic n europ athy, an d Leber opt ic n eu ropathy

Management CT scan an d MRI of th e orbit an d brain are perform ed to n ote th e com pressive lesion an d it s exten t . Treat m en t is directed tow ard th e u n derlying cau se of com pression .

Infiltrative Optic Neuropathy In filt rat ion of th e opt ic n er ve can be by n eoplast ic or in flam m ator y cells from lym p h om as, leu kem ias, sarcoidosis, syph ilis, t u berculosis (TB), fu ngal in fect ion , or m etast asis from lu ng or breast carcin om a.

Presentation Pat ien ts presen t w ith progressive, bilateral, severe vision loss. In it ially th e disk is n orm al oph th alm oscopically but later m ay becom e sw ollen due to in filt rat ion at th e opt ic n er ve h ead.

Differential Diagnosis Bilateral AION, hypoten sive sh ock, radiat ion injur y, com pressive opt ic n europathy, n ut rit ion al opt ic n europ athy, an d Leber opt ic n eu ropathy

Management MRI of th e orbit an d brain , CSF an alysis, an d screen ing test s for gran u lom atou s disorders an d blood-related disorders are required to con firm th e cau se. Palliat ive radioth erapy for in filt rat ive n eoplasm s an d cor t icosteroids or an t im icrobials for in filt rat ive or in fect iou s disorders are advocated form s of t reat m en t .

Radiation Optic Neuropathy Th e n eu ropathy is usu ally delayed by 2 years after st an dard doses of radiat ion but m ay be seen m any years after th e t reat m en t . Th e opt ic n er ve can be affected by radiat ion to th e eye, orbit , sin us, n asoph ar yn x, or brain .

Presentation Bilateral decreased visu al acu it y is th e presen t ing sym ptom .

Differential Diagnosis Bilateral AION, hypoten sive sh ock, in filt rat ive opt ic n europathy, com pressive opt ic n europ athy, n ut rit ion al opt ic n europathy, an d Leber opt ic n europ athy

13 Neuro-Ophtham ology 389

Management Th e h istor y is sign ifican t . CT an d MRI are don e to evalu ate th e opt ic n er ve. No t reat m en t is available.

Tumors of Neural Origin Optic Nerve Glioma Low -grade, pilocyt ic ast rocytom a can involve opt ic n er ve an d ch iasm ; 30% are associated w ith n eurofibrom a t yp e 1 (NF-1) an d h ave a bet ter progn osis.

Presentation Opt ic n er ve gliom a u su ally presen ts in th e first decade w ith gradu al visu al loss associated w ith proptosis ( exten ded [3–7 days] > flexible [1–3 days] > daily w ear [1 day] > single use or daily disposable len ses.) It is described

16 Contact Lenses

451

as a cut an eou s basoph ilic hypersen sit ivit y to surface protein s. It is con sidered th at th e com bin at ion of th e irritat ive m ech an ics in du ced by surface deposit s as w ell as th e an t igen ic respon se of th e den at u red surface protein s w ith in th e dep osits lead to th e in flam m ator y resp on se an d th e developm en t of th e papillae. Mech an ical irrit at ion of th e conju n ct iva n ot on ly occu rs w ith con t act len ses bu t can also occur in isolated areas associated w ith su t u res. Also, th e in t roduct ion of silicon e hydrogel con t act len s m aterials, an d th e related con t in u ou s w ear, h as given a rebirth to an in creasing in ciden ce of CLPC. Th is is related to a h igh er m odulus of elast icit y (1.1 to 1.2 m egapascals) m aking th e len s st iffer th an hydrogel len ses. Th e rigidit y of th e m aterial en courages m ech an ical irrit at ion by ru bbing again st th e su perior palp ebral conju n ct iva, producing a local respon se. Papillae are m orph ologically differen t from follicles an d less severe com pared w ith an acu te, hypersen sit ive follicu lar or cobbleston e ap pearan ce of a vern al con jun ct ivit is. GPC or CLPC is best obser ved using a w h ite, diffuse ligh t on low m agn ificat ion . Papillae, w h ich are space-occu pying elevat ion s, w ill ten d to grab th e len s u pon th e blin k an d h old it in a sligh tly su perior, decen tered posit ion w h ile im peding th e dow nw ard t ran slat ion of th e len s. Because of th e con stan t irrit at ion of th e palpebral conju n ct iva, a react ive m u cous disch arge w ill in crease, leading to addit ion al len s su rface deposit ing. Th e palpebral conju n ct iva can be described as sm ooth or sat in , un iform or n on un iform . Un der low m agn ificat ion , w ith or w ith ou t NaFl stain ing, a variable level of hyp erem ia w ill appear w ith an in creasing degree of edem a. Th e papillae w ill be of var ying sizes, from 0.5 m m an d greater. With th e in st illat ion of NaFl, dist in ct crevices can be visu alized bet w een each papillae, w h ich w ill assist in th e delin eat ion of th e severit y of th e pap illar y react ion . In th e earliest stages th e pat ien t is gen erally asym ptom at ic yet com plain s of frequ en t deposit ing of len ses an d variable vision . As th e con dit ion progresses, th e pat ien t st ar t s to fin d th at clean ing th e len ses becom es som ew h at fu t ile, an d th ere is a sligh t to m ore puru len t m ucou s disch arge. Th is m ay or m ay n ot be sym m et ric to both eyes an d is often bilateral, asym m et ric in presen tat ion . As th e disch arge an d th e vision depreciate, so does th e pat ien t’s len s-w earing t im e. Th e pat ien t w ill also fin d th at th e len s ten ds to decen ter sign ifican tly an d m ay even com plain of a greater len s aw aren ess. Most often th e pat ien t w ill presen t w ith a self-diagn osis of a “com m on conjun ct ivit is or red eye” an d m ay h ave self-t reated w ith over-th ecou n ter vasocon st rict ive agen ts or been t reated by th e prim ar y care physician for a “garden variet y” bacterial conjun ct ivit is w ith out respon se to an t ibiot ics. Th ere m ay be som e ten dern ess to t act ile m an ipulat ion of th e lid bu t n o sign ifican t pain , n or do system ic sym ptom s suggest bacterial or viral conju n ct ivit is. CLPC can be st aged in to four levels of severit y. Stage 1 dem on st rates n o an atom ical sign s, an d on ly m in or sym ptom s of m u cous disch arge an d itch ing. Stage 2 exh ibit s p apillar y en largem en t to 0.5 m m but less th an 1 m m , m ucou s st rain s, hyperem ia, an d an in crease in len s deposits. Th e pat ien t w ill describe itch in ess, disch arge, len s aw aren ess, an d blu rred acu it y. Stage 3 sh ow s papillae greater th an 1 m m , in creased m u cu s, len s aw aren ess, hyperem ia, edem a, an d len s decen t rat ion su periorly. Th e pat ien t w ill describe m oderate to severe sym ptom s w ith decreased w ear t im e, frequ en t len s deposit ing, an d in creased len s m ovem en t an d blu r. Stage 4 dem on st rates pap illae larger th an 1 m m , w h ich h ave a m u sh room sh ape accom pan ied by severe sym ptom s an d sign s ( Fig. 16.2 ).

452

Color Atlas of Ophthalm ology

A

B

C

D

E Fig. 16.2 (A) Papillary formation right eye (ocular dextrose, OD). (B) Nonpapillary satin left eye (ocular sinistras, OS). (C) Grade 1 contact lens–induced papillary conjunctivitis (CLPC) marginal papillary formation with hyperemia Pre -Tx OD. (D) Grade 3 CLPC with isolated giant papillary formation with hyperemia Pre-Tx OS. (E) Grade 1 CLPC with hyperem ia post-Tx OD 2 weeks: significant decrease in hyperemia and early papillary formation.

16 Contact Lenses

453

F

G Fig. 16.2 (Continued) (F) Grade 3 CLPC with hyperemia post-Tx OS 2 weeks: significant decrease in hyperem ia and early papillary form ation. (G) Zonal diagram of the superior everted lid.

Differential Diagnosis Involves iden t ifying th e un derlying cu lprit of m ech an ics, protein den at urat ion , an t igen ic-related respon se, or environ m en t al in flu en ces giving rise to sim ilar fin dings an d sym ptom s exacerbated by th e use of con t act len ses, yet th ey are n ot th e cau se. By h istor y alon e, th e et iology an d differen t ial can be m ade. If th ere is hydrogel con tact len s w ear, exten ded m ore th an daily; if th ere is a h istor y of cat aract or corn eal procedures w ith su t u res; or if th e pat ien t h as a sign ifican t h istor y of season al or vern al allergy, th e h istor y is th e t ru e stor y. Treat m en t w ill th us follow th e scen ario of th e h istor y. CLPC m ay also presen t w ith sim ilar sym ptom s t ypical of a variet y of conjun ct ivit is, in cluding bacterial, viral, vern al, atopic, or m ech an ically in du ced by su t u res postoperat ively. Th e h allm ark differen t ial of CLPC is a m ore rapid on set w ith in creased m u cous disch arge an d th e in abilit y to properly m ain tain th e con t act len s on th e eye, u su ally exh ibited by excessive len s m ovem en t du e to th e m ech an ical in fluen ce of th e en larged pappilae. Follicles of variable size are seen in a hyperem ic conjun ct iva, in ferior m ore so th an sup erior (un like CLPC seen in th e su perior palp ebral conju n ct iva) w ith t ran slu cen t , avascular fluid–lym p h oid accu m ulat ion an d are accom pan ied by system ic fin dings as in ph ar yngoconjun ct ival fever (PCF) or epidem ic keratoconjun ct ivit is (EKC), as w ell as ru ling out ch lam ydial disease. Th e Academ y of Op h th alm ology h as presen ted an excellen t differen t ial form at for conju n ct ivit is as seen in Table 16.3 .

454

Color Atlas of Ophthalm ology

Table 16.3

Typical Clinical Signs o f Co njunctivitis Typical Clinical Signs o f Co njun ctivitis

Type o f Co njunctivitis Clinical Signs Allergic/immunologic Seasonal allergic

Bilateral; conjunctival injection, chem osis, watery discharge, mild m ucous discharge

Vernal

Bilateral; giant papillary hypertrophy of superior tarsal conjunctiva, bulbar conjunctival injection, conjunctival scarring, watery and mucoid discharge, limbal Trantas dots, limbal “papillae,” corneal epithelial erosions, corneal neovascularization and scarring, corneal vernal plaque/shield ulcer

Atopic

Bilateral; eczematoid blepharitis; eyelid thickening, scarring; lash loss; papillary hypertrophy of superior and inferior tarsal conjunctiva; conjunctival scarring; watery or mucoid discharge; boggy edem a; corneal neovascularization, ulcers, and scarring; punctate epithelial keratitis; keratoconus; subcapsular cataract

Giant papillary

Lateralit y associated with contact lens wear pat tern; papillary hypertrophy of superior tarsal conjunctiva, mucoid discharge; in severe cases: lid swelling, ptosis

Mechanical/irritative Superior lim bic keratoconjunctivitis (SLK)

Bilateral superior bulbar injection, laxit y, edem a, and keratinization; superior corneal punctate epitheliopathy and laments

Contact lens-related SLK Injection of superior bulbar conjunctiva, epithelial thickening of limbus with neovascularization and/or extension of conjunctival epithelium onto superior cornea; papillary hypertrophy of tarsal conjunctivitis is variable Floppy eyelid syndrome

Upper eyelid edema; upper eyelid easily everted, som etimes by simple elevation or lifting of lid; di use papillary reaction of superior tarsal conjunctiva; punctate epithelial keratopathy; pannus; bilateral often asym metric

Pediculosis palpebrarum Unilateral or bilateral follicular conjunctivitis; adult lice (Pthirus pubis) at the base of the eyelashes, nits (eggs) adherent to the eyelash shafts, blood-tinged debris on the eyelashes and eyelids Medication-induced keratoconjunctivitis

Lateralit y based on drug use; conjunctival injection, inferior fornix conjunctival follicles; distinctive signs: contact dermatitis of eyelids with erythema, scaling in some cases

16 Contact Lenses

Table 16.3

455

(Continued) Typical Clinical Signs o f Co njunctivitis Typical Clinical Signs o f Co njun ctivitis

Type o f Co njunctivitis Clinical Signs Viral Adenoviral

Herpes simplex virus

Abrupt onset; unilateral or bilateral; varies in severit y; bulbar conjunctival injection, watery discharge, follicular reaction of inferior tarsal conjunctiva, chemosis Distinctive signs: preauricular lymphadenopathy, petechial and subconjunctival hemorrhage, corneal epithelial defect, multifocal epithelial punctate keratitis evolving to anterior stromal keratitis, mem brane/pseudom em brane formation, eyelid ecchymosis Unilateral: bulbar conjunctival injection, watery discharge, mild follicular reaction of conjunctiva; may have palpable preauricular node Distinctive signs: vesicular rash or ulceration of eyelids, pleom orphic or dendritic epithelial keratitis of cornea or conjunctiva

Molluscum contagiosum Typically unilateral but can be bilateral: m ild to severe follicular reaction, punctate epithelial keratitis; may have corneal pannus, especially if longstanding Distinctive signs: single or multiple shiny, dom e-shaped umbilicated lesion(s) of the eyelid skin or margin Bacterial Nongonococcal

Unilateral: bulbar conjunctival injection, purulent or m ucopurulent discharge

Gonococcal

Unilateral or bilateral: marked eyelid edem a, marked bulbar conjunctival injection, marked purulent discharge, preauricular lymphadenopathy Important sign to detect: corneal in ltrate

Chlamydial Neonate/infant

Unilateral or bilateral Eyelid edema, bulbar conjunctival injection, discharge may be purulent or mucopurulent, no follicles

Adult

Bulbar conjunctival injection, follicular reaction of tarsal conjunctiva, mucoid discharge, corneal pannus, punctate epithelial keratitis, preauricular lymphadenopathy

Immune -mediated Ocular cicatricial pemphigoid

Distinctive sign: bulbar conjunctival follicles Bilateral: bulbar conjunctival injection, papillary conjunctivitis, conjunctival subepithelial brosis and keratinization, conjunctival scarring beginning in the fornices, punctal stenosis and keratinization, progressive conjunctival shrinkage, symblepharon, entropion, trichiasis, corneal ulcers, neovascularization, and scarring (continued on page 456)

456

Color Atlas of Ophthalm ology

Table 16.3

(Continued)

Typical Clinical Signs o f Co njunctivitis

Typical Clinical Signs o f Co njun ctivitis Type o f Co njunctivitis Clinical Signs graft-versus-host disease

Neoplastic Sebaceous gland carcinoma

Bilateral; conjunctival injection, chemosis, pseudomembranous conjunctivitis, keratoconjunctivitis sicca, superior lim bic keratoconjunctivitis, cicatricial eyelid disease, episcleritis, corneal epithelial sloughing, limbal stem cell failure, calcareous corneal degeneration; rare intraocular involvem ent Unilateral: intense bulbar conjunctival injection, conjunctival scarring; corneal epithelial invasion may occur Eyelids may exhibit a hard nodular, nonm obile mass of the tarsal plate with yellowish discoloration; may appear as a subconjunctival, m ultilobulated yellow mass, may resem ble a chalazion

Note: Typical clin ical sign s m ay n ot be p resen t in all cases. Dist in ct ive signs are m ost u sefu l in m aking a clinical diagn osis, bu t m ay occu r u n com m on ly. In all en t it ies, lateralit y m ay var y and m ay be asym m et rical. Source : Matoba AY. Preferred Pract ice Pat tern s, Conjun ct ivit is. San Fran cisco: Am erican Acad em y of Op h th alm ology; 2003. Available at: h t t p ://w w w.aao.org/aao/edu cat ion /librar y/p pp /u p load/ Conjun ct ivit is_.p df. (Accessed 12–08–2008). Reprinted w ith p erm ission .

Management As w ith any poten t ial an t igen ic–allergic respon se, on e m u st first rem ove th e in it iat ing st im u lus. Th e sim p le rem oval an d discon t in u an ce of th e len s are th e easiest t reat m en t , bu t for som e pat ien ts th e m ost t rau m at ic. Th erefore a com bin at ion th erapy is suggested based on th e level of disease. It is im port an t to n ote th at th e use of vasocon st rict ive agen ts, an t ibiot ics, an d/or an t ivirals th at h ave been star ted prior to presen tat ion can be safely discon t in u ed. How ever, if th ere is con cern abou t bacterial coin fect ion , m ain t ain th e appropriate level of an t ibiot ics such as four th gen erat ion fluoroquin olon e w h en involving con tact len s. The m ost expedient treatm ent is the sim ple discontinuation of w earing contact lenses and converting to eyeglass w ear until the condition im proves. How ever, in m any cases, the patient m ay not have eyeglasses or m ay not be tolerant of the alternative. Therefore, the use of soft daily disposable lenses w ith a high m oisture content in conjunction w ith a steroidal antiinflam m atory serves as the best overall therapy. Stage 1 CLPC requires m inim al intervention, such as refitting the patient w ith a frequent replacem ent or disposable lens. In this situation, the simple conversion to a “daily disposable–single use lens” is the m ost appropriate. Another option would be to continue conventional, disposable, or frequent replacem ent lenses but change the care product to a peroxide-based system and possibly add an enzym atic cleaning solution. In an unpu blish ed st u dy by K. Dan iels, daily disposable len ses dem on st rated a m ore rapid resolu t ion of p at ien t sym ptom s w ith grade 2 to 4 CLPC w ith ou t th e u se of m edicin als follow ed by 1-w eek an d 2-w eek disposable len ses, respect ively. Th is suggest s th at th e sim ple u se of daily disposable–single use len ses m igh t be th e m ost appropriate single or adjuvan t t reat m en t for CLPC. St age 2 CLPC requ ires len s replacem en t , frequen t irrigat ion w ith lu bricat ing drops to rid m ucus, lid hygien e to avoid lid w ipers epith eliopathy, an d possibly

16 Contact Lenses

457

a prescript ion for a m ast cell st abilizer such as such as lodoxam ide (Alom ide, Allergan , Ir vin e, CA), crom olyn (Crolom , Bausch & Lom b, Roch ester, NY) (Opt icrom , Allergan , Ir vin e, CA), n edocrom il (Alocril, Allergan ), pem irolast (Alam ast , Vist akon Ph arm aceu t icals, Jacksonville, FL), or olopatadin e (Pataday or Patan ol, Alcon Laboratories, For t Wor th , TX) for sh ort-term to ch ron ic th erapy. Stage 3 CLPC requires a discontinuation of lenses for a short tim e w hile prescribing a m ast cell stabilizer or low -concentration steroid such as prednisolone 1%. Lenses can be refit to daily disposable or short-term frequent replacem ent lenses w ith peroxide care products until resolution of the papillae to a w hitened cap called hypertrophy. St age 4 CLPC requires com plete discon t in u at ion of len ses an d m ore aggressive steroid in ter ven t ion u n t il resolu t ion . Up on resolut ion , frequ en t replacem en t or disposable len ses sh ould be fit ted u sing a peroxide-based product system . In gen eral, w h en u t ilizing steroids for t reat m en t , it is h igh ly suggested to adju vantly treat the patient w ith single-use daily disposable lenses, w hich w ill satisfy the patient’s needs w hile allow ing for a bandage lens–drug delivery efficiency. The steroid (prednisolone 1%) should be aggressively dosed for the first 1 to 2 w eeks at four tim es a day and then taper slow ly over the next 2 to 3 w eeks. As one tapers the prednisolone dow n to t w ice a day, start the addition of a soft steroid such as loteprednol either 1%or 0.2%for 1 to 2 weeks or until resolution of clinical findings. Long-term m aintenance is m ost appropriate w ith shorter-term , frequently replaced hydrogel lenses of 2 to 4 w eeks of nonionic, high-w ater-content m aterials and peroxide cleaning or a conversion to gas perm eable high Dk–plasm a-treated designs. Also con sider th e long-term u se of an t ih ist am in e–m ast cell st abilizer for longterm m ain ten an ce. Addit ion ally, if on e w as to be con ser vat ive w ith long-term m edicin als, con sider t w ice-a-day to th ree-t im es-a-day u se of physiologically based w et t ing drops such as vit am in A drops (ViVa, Corn eal Scien ces, Gaith ersbu rg, MD) or elect rolyte-balan ced form ulas such as Th era–Tears (Advan ced Vision Research , Boston , MA) or Sooth e (Bau sch & Lom b, Roch ester, NY; Alm ira Scien ces, Atlan t a, GA). Th ere is also suggest ion th at cyclosporin e drops (Rest asis, Allergan , Ir vin e, CA) m ay also be h elpful in a t w ice-a-day dosage for long-term con t rol of ocular in flam m ator y respon se as w ell as being a veh icle carrier h elpfu l in m ain tain ing a h ealthy ocu lar m ucin surface to avoid m ech an ical irritat ion from th e len s m aterial.

Vascularization Vascularizat ion is con sidered to be th e gen eral form at ion an d exten sion of capillaries th at h ad n ot previously existed w ith in th e avascu lar corn ea. Neovascularizat ion is th e form at ion of n ew vessels as an exten sion or sh u n ts to preexist ing vascularized areas of th e avascular corn ea. To differen t iate fur th er is to classify form s of redn ess an d vascu larizat ion by locat ion . Lim bal engorgem en t or hyperem ia is th e disten sion of lim bal blood vessels in th e absen ce of n ew vessel grow th . Vessel ingrow th or pen et rat ion is n ot n eovascularizat ion , bu t sim ply an exten sion of a vessel inw ard tow ard th e cen t ral corn ea. Pan n u s, w h ich is h igh ly vascularized, is exten sion of conju n ct ival t issue overlapping th e clear avascular corn ea seen as an an atom ical varian ce or in duced by t rau m a to th e eye. Ch ron ic hypoxia is th e u n derlying con dit ion th at in it iates th e vascu larizat ion respon se. Hypoxia cau ses lactate acidosis, w h ich decreases th e in tegrit y of th e epith elium an d st rom al soften ing. Th is yields an oppor t un it y for vessel ingrow th . Du e to hyp oxia, an early ph ase of vascu larizat ion occu rs in ducing a release of in flam m ator y m ediators. Th is st im ulates addit ion al vessel grow th called an angiogenic response. Tigh t len ses, lim bal com pression , an d/or t rau m a m ay also st im ulate a

458

Color Atlas of Ophthalm ology

vascu lar respon se, w h ich w ill in crease th e release of in flam m ator y an d vasost im u lator y m ediators. Th ere are several p ossibilit ies th at en cou rage vascu larizat ion un der hypoxic con dit ion s: (1) vasost im u lat ion an d in flam m at ion , (2) t igh t len s syn drom e, (3) lim bal-plexal com pression , (4) solut ion sen sit ivit y, an d (5) vasogen ic resp on se to t raum a ( Fig. 16.3 ). Th e m ain cau ses of vascularizat ion associated w ith con tact len s w ear are t igh t len s (edge su ct ion ) cen t ral corn eal edem a (hyp oxia), solut ion toxicit y, m ech an ical abrasion , len s dam age an d m ech an ical st im u lu s associat ion w ith irrit at ion , surface dep osit ion , an d poorly fit t ing len ses.

A

B

C

D

E

F

G

H

I

Fig. 16.3 (A) Limbal congestion–hyperemia. (B) Com bination of vasodilatation (corkscrew vessels)–vasoproliferation–vasolimbal congestion. (C) Severe lim bal vascularization congestion with early neovascularization. (D) Vessel penetration with early pannus. (E) 4+ superior lim bal neovascularization leading toward the central cornea and papillary zone encroachment. (F) Intracorneal hemorrhage from neovascularization post-LASIK on an extended-wear contact lens wearer. (G) Sectoral pannus associated with overwear. (H) Sectoral pannus with corneal decompensation. (I) Sectoral pannus with corneal decompensation–placido topographic image.

16 Contact Lenses

459

Presentation Vascular respon ses to ch ron ic hyp oxia can var y from m in or to severe based on th e associat ion w ith possible m icrobial in filt rat ion . Som e form of corn eal vascularizat ion occurs in ~34% of cases associated w ith hydrogel len s u se versu s 2% w ith n on len s w earers, w ith 98% of th e vascularizat ion occu rring w ith in th e superficial st rom a. Th e pat ien t is oth er w ise asym ptom at ic oth er th an n ot ing an apparen t hyperem ia or lim bal engorgem en t , w h ich app ears to th e pat ien t as a “ch ron ic red eye” w h en w earing con tact len ses. Th is is sim ply an engorgem en t of th e m argin al arcade capillaries. Th ese vessels h ave a st raigh t prot uberan ce w ith a defin ed loop at th e en d. Hyperem ic episcleral lim bal vessels are differen t iated from n eovessels th at exten d for w ard w ith leaflike fron ds th at in terdigitate. Vascu larizat ion appears sim ilar to a m esh like plexal grow th in th e m idepith elium project ing tow ard th e corn ea like sm all, lin ear spikes an d bran ch es called fronds (sim ilar to th e vein s w ith in a leaf). Th ere is gen erally n o sym ptom atology associated w ith th e fin dings. Th ese are differen t iated from n orm al lim bal vessels th at “loop” back tow ard th e lim bu s. Low -grade (grade 1) vessels w ill ten d to m igrate inw ard to approxim ately 0.4 to 0.6 m m (daily w ear) to 1.4 m m (exten ded w ear). Grade 2 w ill be grade 1 vessels th at w ill ten d to m igrate tow ard th e pupil w ith ou t p assing in to th e pupillar y zon e. Th e m ost severe, grade 3, w ill pen et rate th e pu pillar y region . It is im por tan t to ph otodocum en t th e vessels an d determ in e th e locat ion on th e lim bus, depth (su perficial or deep), degree of p en et rat ion , an d severit y defin ed as th e depth of pen et rat ion (an d th e advan cem en t of grow th tow ard th e papillar y region ).

Differential Diagnosis Vascularizat ion an d n eovascu larizat ion are differen t iated from lim bal vessels th at m ay be dilatated by t rau m a, in fect ion , in flam m at ion , t um or, conjun ct ival in grow th or pter ygiu m , or postoperat ive com plicat ion . Differen t iat ion m ust also be m ade from vascu larized pan n us, w h ich is th e ingrow th of vessels an d con n ect ive w ith in th e epith eliu m . Th e differen t iat ion of vessel depth is im port an t . Su perficial vessels or vascularizat ion in it iates from th e lim bal capillar y arcade, an d th e vessels are m ore tor t u ou s an d of sm aller caliber th an deep st rom al vessels, w h ich em erge from w ith in th e lim bal m idst rom al region an d are larger in caliber, h ave abr upt en d bulbs, an d m ay disru pt th e regu larit y of th e lim bal corn ea. Oth er factors th at m ay be associated w ith con t act len s–related vascularizat ion are dr y eyes (keratoconjun ct ivit is sicca) or ocular su rface disease an d oth er diseases, su ch as bleph arit is, acn e rosacea, Sjögren syn drom e, an d im m u n e dysfun ct ion , as w ell as in terst it ial kerat it is, h erpes kerat it is, t u bercu losis, m easles, syph ilis, an d th e possibilit y of am in o acid deficien cies. Because of th e p oten t ial of n on –con t act len s–related con cern s associated w ith corn eal vascularizat ion , biom icroscop ic exam sh ould in clude direct illu m in at ion an d ret roillu m in at ion , part icu larly of th e lim bal periph eral vessel arcades. In gen eral, superficial vessels w ill em erge in to th e an terior st rom a an d appear as single or m ult ip le (pan n u s) tor t uous vessels un der low m agn ificat ion , yet deeper st rom al vessels course th rough th e corn ea as m ore lin ear vessels th at arborize. Lip id deposit ion appears as yellow -w h ite opacit ies at th e leading edge or surrou n ding th e st rom al vessels. Obser vat ion of lipid exu dates su rrou n ding an act ively engorged vessel(s) sh ould raise th e con cern of a possible iris-angle carcin om a requiring diligen t gon ioscopic exam in at ion . If th ere is a conju n ct ival grow th closely ju xt aposed to th e lim bu s adjacen t to th e corn eal vascularizat ion , conju n ct ival carcin om a m ay be suspected.

460

Color Atlas of Ophthalm ology

Management Th e pat ien t w ith lim bal hyp erem ia or vessel engorgem en t ten ds to self-t reat w ith over-th e-cou n ter vasocon st rict ive agen t s. Th ese pat ien ts w ill fin ally presen t st ating th at even w ith th ese agen t s th eir len ses feel dr y an d th eir eyes are red. In th is sit u at ion , sim ply an d forcefu lly tell th e pat ien t to discon t in u e drops th at “get th e red ou t .” Th ese drop s ten d to yield reboun d congest ion as w ell as a m ild m ydriasis an d slow ing of accom m odat ion du e to th e sym path om im et ic effects. In th is sit uat ion , it is best to discon t in u e th e len ses for a sh or t period—2 to 3 days—an d prescribe a soft steroid to rid any low -level in flam m ator y com pon en t s. In conju n ct ion , im plem en t physiologically based w et t ing drops su ch as vit am in A (ViVA, Corn eal Scien ces, Gaith ersbu rg, MD), Th era-Tears (Advan ced Vision Research , Boston , MA), Bion Tears (Alcon Laboratories, For t Wor th , TX), or Sooth e (Bau sch & Lom b, Roch ester, NY, Alm ira Scien ces, Atlan ta, GA). In addit ion to topical su pplem en t at ion , con sider th e u se of om ega 3-6 com bin at ion s for th eir in h eren t an t iin flam m ator y an d m u cin com plem en tar y abilit ies. Also, on e sh ou ld con sider agen ts su ch as Syst an e (Alcon Laboratories, Fort Wor th , TX) or En dura (Allergan , Ir vin e, TX) for topical su rface t reat m en t . After th e discon t in uan ce of th e con tact len s u se an d after vessel regression , th ere w ill be gh ost vessels or ch an n els th at develop. Th ese m u st be w ell docu m en ted to differen t iate th en from gh ost vessels th at m ay be associated w ith an in terst it ial kerat it is. On ce th e lim bal hyperem ia is ten ded to, a refit to con tact len ses sh ou ld be com pleted w ith th e ph ilosophy of h igh w etabilit y, m oderate m odu lu s, an d h igh oxygen perm eabilit y. Th e pat ien t can be refit to n on ion ic, h igh -w ater-con ten t hydrogel, silicon e hydrogel of a low er m odu lu s. If th e m odu lus is h igh , th ere ten ds to be st iffn ess to th e len s th at can in du ce conju n ct ival irritat ion an d repeat of conju n ct ival hyperem ia. In addit ion , on e sh ou ld con sider a h igh diffu sion coefficien t of a rigid gas p erm eable len s w ith su fficien t axial edge clearan ce to en h an ce th e flu id–tear ch an n el an d avoid corn eal an d lim bal edge in flu en ces. Th ese len ses sh ould also in corporate an app ropriate plasm a t reat m en t for w et abilit y. Care produ cts an d len s sch edu les sh ould be revisited, an d pat ien t educat ion n eeds to be com preh en sive. In th e even t of m ore pron ou n ced an d progressive vascu larizat ion , such as n eovascu larizat ion , on e m u st be con cern ed abou t th e fragilit y of th ese vessels. It is n ot un com m on to obser ve an in t racorn eal h em orrh age as a direct sequ ela of n eovascu larizat ion . If a h em orrh age occu rs, it w ill app ear as a “red spot on th e corn ea” sim ilar to a “petech ial conjun ct ival h em orrh age” located at th e proxim al en d of a vascular fron d. Th ese h em orrh ages sh ould be ph otograph ed an d m on itored for spread. Th e pat ien t sh ou ld refrain from any agen t th at h as an an t icoagulan t effect su ch as aspirin , Plavix, w arfarin , as w ell as n eut raceu t icals th at h ave an an t ith rom bot ic ch aracter. Addit ion ally, th ese n eed to be w ell docum en ted as w ell as th e n eovascu larizat ion in th e even t th e pat ien t decides to pursu e in t racorn eal refract ive surgical procedu res in w h ich in t raoperat ive corn eal h em orrh age could be sign ifican t . Essen t ially, th e t reat m en t for any vascular respon se w ith in th e corn ea from con tact len ses is th e sam e, an d th e varian ce is th e un derlying cau se an d in ter ven t ion . On ce th e pat ien t h as been properly refit , edu cat ion an d m on itoring are th e keys to avoidan ce of recu rren ce. Addit ion ally, as part of th e t reat m en t gu idelin e, on e m ust clin ically m on itor for issu es th at m ay in duce ocu lar irrit at ion an d vascu larizat ion oth er th an con t act len ses, such as acn e rosacea, system ic m edicat ion s, an d con dit ion s th at in clu de con n ect ive t issu e disorders, autoim m u n e an d in flam m ator y disease, im m u n ocom prom ised disorders, vascular st im u lator y disease su ch as kidn ey problem s an d diabetes, keratoconjun ct ivit is sicca, an d associated h orm on ally related disorders of th e eye, as w ell as environ m en tal irritan t s, use of diu ret ics (m edicin al or as beverage–alcoh ol an d caffein e), an d sm oking.

16 Contact Lenses

461

Subepithelial Infiltrates Su bepith elial in filt rates (SEIs) are an in flam m ator y react ion secon dar y to ch ron ic hypoxia or an acu te react ion th at th reaten s th e avascular corn ea an d an terior segm en t . Th is acute react ion in duces an in flam m ator y react ion th at en courages an aggregat ion or accu m u lat ion of cellu lar com pon en ts, su ch as p olym orph on u clear cells, to m igrate th rough th e avascular corn eato an d set tle w ith in th e subepith eliu m adjacen t to Bow m an’s layer or basem en t m em bran e. Th is occurs in ~2% of len s w earers regardless of w ear an d replacem en t sch edule. Th e et iology of SEI, w h en associated w ith con t act len ses, is ch ron ic hypoxia, prolonged edem a, an im m u n e resp on se, solut ion toxem ia, m ech an ical irrit at ion –foreign debris, or a local in fect ion or th e exotoxin s from bacteriu m residen t in th e ocular flora. Th ey are th ough t to represen t a delayed hypersen sit ivit y im m u n e respon se to viral an t igen s in th e corn eal st rom a. Corn eal in filt rates are aggregates of gray or w h ite m igrat ing in flam m ator y cells arising from n orm ally t ran sparen t corn eal t issue. Th e in flam m ator y respon se st im ulates th e m igrat ion from th e lim bal vascu lat u re or from th e tears as a respon se to t issue dam age or a secon dar y ch em ot act ic react ion associated w ith an environ m en t al an t igen ic act ivit y or toxin s, con t act len s solut ion s, or from m icrobial organ ism s th em selves. In filt rates are defin ed as polym orph on uclear leu kocytes (n eu t roph ils) but m ay also con t ain lym ph ocytes an d m acroph ages. In con tact len s w ear, in filt rates are m ost often sterile (n on in fect ious) but can also be in fect iou s ( Fig. 16.4 ).

A

B

C

D

E

F

Fig. 16.4 (A) Subepithelial infiltrate with limbitis secondary to contact lens wear. (B) Subepithelial infiltrate (SEI) secondary to extended-wear contact lenses pretreatment. (C) Epithelial compromise with migration of SEI forward through epithelium. (D) Subepithelial Infiltrate secondary to extended-wear contact lenses post treatment with steroid. (E) Adenovirus with infiltrates. (F) Viral Infiltrates with secondary scarring. (Continued on page 462)

462

Color Atlas of Ophthalm ology

Fig. 16.4 (Continued) (G) Viral infiltrates with secondary scarring topography.

G

Presentation Th ere is an in it ial an d p ron ou n ced lim bal–vascu lar resp on se in th e area of th e ocu lar in su lt . Su bsequen tly th ere is a release of m ediators from th e lim bal p lexu s. Th e cellular or h u m oral com pon en ts w ill m igrate in to an d th rough th e corn eal t issu e leading to th e accu m ulat ion of cells th at w ill appear as discrete w h ite-gray subepith elial p ockets or opacit ies. SEIs are seen as h azy gray, circum scribed in filt rates, at an in t ra- or su bepith eliu m (at th e surface of Bow m an’s layer w ith ou t in filt rat ion in to th e st rom a; th erefore, n o scarring) or w ith an terior st rom al level w ith in filt rat ion , th u s w ith a poten t ial for scarring. Th ey w ill be predom in an tly u n ilateral an d con cen t rated focally or diffuse w ith a preferen ce to th e lim bal an d paracen t ral areas of th e corn ea. Adjacen t to th e SEI m ay be an area of localized conjun ct ival inject ion at th e lim bal ju n ct ure of m ild to m oderate severit y. If th ere is sign ifican t corn eal vascu larizat ion of any form , th ere w ill be th e poten t ial for a h igh er in ciden ce of subep ith elial in filt rates. As SEI m igrates for w ard, th ere m ay be a subtle epith elial com prom ise or break th at w ill st ain . Th e p at ien t m ay h ave subtle sym ptom s ranging from m ild to m oderate. With in flam m at ion of any form th ere w ill be an associated hyperem ia localized to th e area of occu rren ce, localized edem a, an d a variable degree of discom fort . Th e pat ien t m ay also exp erien ce a m ild to m oderate level of lacrim at ion an d ph otoph obia an d a decrease in visu al acuit y based on th e locat ion of th e in filt rates, irrit at ion , an d/or foreign body sen sat ion .

Differential Diagnosis If th ere is an oth er an terior ch am ber react ion , on e m u st differen t iate bet w een an in filt rat ive kerat it is associated w ith several oth er an terior segm en t path ologies. Th ese m ay also h ave a system ic relat ion sh ip th at n eeds to be looked for. Th ese in clu de episclerit is, m argin al ulcer, irit is, aden ovirus or EKC, or keratoconjun ct ivit is. Addit ion ally, quiet n on in flam m ator y opacit ies m ay be in act u alit y a su btle asym ptom at ic in filt rate or a sim ple scar. Histor y in th is case w ill assist in th e differen t ial. If th ere is sign ifican t corn eal vascu larizat ion of any form , th ere w ill be th e poten t ial for a h igh er in ciden ce of subep ith elial in filt rates. NaFl w ill be an im port an t differen t ial in dist inguish ing bet w een a scar, u lcer, or SEI. Scars an d SEI w ill n ot stain , bu t u lcerat ion w ith an epith elial defect w ill. Th is w ill allow a differen t ial bet w een SEI an d m icrobial epith elial defects. Upon t reatm en t , th e in filt rates m ay m igrate for w ard an d disru pt th e epith elial surface, cau sing a top ograph ic irregu larit y an d p ossibly a n egat ive stain ing su perficial pun ct ate keratopathy. To en su re n o epith elial com prom ise an d to ru le ou t an early stage of

16 Contact Lenses

463

m asquerader such as a h erpet ic lesion (den drit ic) Pseudom onas, Acantham oeba , or Fusarium , th e u se of rose bengal or lissam in e green w ill st ain early bu lbs of a den drit ic lesion an d detail devitalizat ion of t issue m u ch m ore readily th an sodium flu orescein . If t h e SEI is associated w it h an ad en ovir u s, t h ere w ill be system ic an d con st it u t ion al fin d in gs su ch as feve r, m alaise, let h argy, m yop at hy (m u scle w eakn ess), p er iau r icu lar lym p h ad en op at hy, an d /or t h e p rese n ce of a follicu lar conju n ct ivit is. If t h e SEI is associated w it h a ke ratoconju n ct ivit is, t h e clin ician sh ou ld con sid er t h e p ossibilit y of a t ran sm it t able d isease. An exam p le w ou ld be a ch lam yd ial in fect ion if t h e re is a seve re follicu lar conju n ct ivit is an d h istor y of u rogen it al in fect ion . Th e p at ie n t sh ou ld be refer red to an in te r n ist , p ar t icu larly in p ed iat r ic cases. An terior segm en t fin dings associated w ith SEI m ay also be fou n d w ith an episclerit is, w h ich m ay h ave a relat ion sh ip to a con n ect ive or collagen t issue disorder (rh eu m atoid). In flam m ator y con dit ion s, su ch as a rh eu m atologic disorder, in flam m ator y bow el disease, or sacroidosis m ay h ave an associated irit is, w h ich presen t s w ith an acute red eye, discom for t or pain , m iosis, decrease in IOP, an d a decrease in acuit y. Even th ough corn eal su bepith elial in filt rates are con sidered a rep resen t at ion of a low -grade im m u n e respon se to bacterial exotoxin s, su bepith elial in filt rates can com plem en t oth er vasost im ulator y resp on ses as seen w ith corn eal vascularizat ion , atopic or viral disease, as w ell as postsurgical causes such as p ostLASIK. Scars an d u lcers can easily m asqu erade as an in filt rate becau se of th eir sim ilar appearan ce of h azy, gray opaqueing, n on t ran slucen t corn ea, an d locat ion at th e su bepith elial an terior st rom al level. Pat ien t s w ith corn eal scars w ill h ave a posit ive h istor y an d w ill n ot respon d to any th erapy. Also, m argin al u lcers can be m istaken for in filt rates barring th e h istor y. Ulcers w ill h ave a m ore rapid on set an d n ot iceable inject ion an d decreased com fort . Ulcers w ill ten d to be located cen t rally. In filt rates m ay be diffuse an d cen t ral; h ow ever, m ore t ypically th ey are lim bal. SEIs w ill appear less den se th an m argin al u lcerat ion s an d dem on st rate a lesser an terior ch am ber an d conjun ct ival react ion . For a differen t ial of u lcers versu s in filt rate see Table 16.4 .

Management In th e m ost basic t reat m en t form at it w ou ld be appropriate to discon t in ue con tact len s u se u n t il resolu t ion . Len s w ear sh ould n ot be resu m ed u n t il all sign s an d sym ptom s are com pletely resolved. Medicat ion is usu ally un n ecessar y in m ost cases of in filt rat ive kerat it is (IK), w ith palliat ive use of preser vat ive-free ocu lar lubrican ts. Th e use of a hyperosm ot ic agen t is prescribed, su ch as NaCl 5% prescribed fou r t im es a day is m ore th an su fficien t if vision is n ot affected an d th ere is a lim ited vascular resp on se. If th ere is a greater vascu lar respon se an d vision is decreased a m ore aggressive approach w ith a steroid su ch as predn isolon e 1%four t im es a day for 1 w eek w ith a slow taper to a soft steroid (lotepredn ol 1%) is h igh ly recom m en ded. Prophylact ic use of an t ibiot ics to preven t secon dar y in fect ion or an t ibiot ic/steroid com bin at ion drops to m it igate th e in flam m ator y respon se is som et im es ben eficial. Such topicals w ould in clu de tobram ycin w ith dexam eth ason e or lotepredn ol (Tobradex, Alcon Laboratories) or Zylet (Bau sch & Lom b) fou r t im es a day for 5 to 7 days an d slow t ap er. Cau t ion on ce again for steroid respon se an d a corn eal toxic keratop athy to tobram ycin . Because m any cases of recu rren t IK are secon dar y to exotoxin s released by lid m argin bacteria ( Staphylococcus an d St reptococcus), it is w ise to recom m en d lid hygien e in th ese cases an d to lim it len s w ear to daily w ear com plem en ted by a

464

Color Atlas of Ophthalm ology

Table 16.4

Di e rential o f Ulcers versus In ltrate

Ulcer

Infiltrate

Epidemiology: relatively rare

Epidemiology: relatively com mon; usually the result of hypoxia Represents m igration of in ammatory white blood cells from the lim bal vasculature and precorneal tear lm Pain is mild to moderate; rarely marked Tends to be peripheral because of proximit y to the cellular in ammatory mechanisms released from the limbal blood vessels

Represents active bacterial infection Generally causes signi cant pain Tends to be central rather than peripheral (Staphylococcus, exotoxin “peripheral ulcers” are toxic/in am matory epithelial defects) Usually a solitary lesion Size of the uorescein epithelial staining defect closely mirrors the underlying stromal lesion

There is alm ost invariably a cellular in am matory response in the anterior chamber Pat tern of bulbar conjunctival injection is usually generalized rather than sectoral

Possible tear lake debris Treatment options: Aggressive use of a topical uoroquinolone with uoroquinolone or polysporin ointment at bedtime and daily followup until good control is achieved Forti ed tobramycin or gentamicin (for gramnegative) and forti ed cephazolin or bacitracin (for gram-positive); therapeutic cycloplegia with 5% hom atropine or 0.25% scopolamine is usually wise

Can be multiple lesions Size of the uorescein epithelial staining defect is usually much sm aller than the underlying stromal lesion; in any situation where there is a strom al in ammation, it is a real challenge for the overlying epithelial cells to remain physiologically intact, which explains why there can be some uorescein staining even in these strom al in am matory responses Secondary anterior chamber reaction is rarely elicited The pat tern of bulbar conjunctival injection is usually sectored and proximally associated with the in ltrate; even if there is 360degree injection, the vascular injection pat tern is skewed toward the sector nearer the in ltrate, particularly if it is peripherally located Tear lake is clear There are t wo therapeutic approaches: If diagnosis is clear: Treat with antibiotic/ steroid combination such as tobramycin with dexam ethasone, or tobramycin with loteprednol, one drop every 2 h for 2 days, and then modify and taper according to circumstances If diagnosis is unclear: Treat with a uoroquinolone every 1 to 2 h and follow up in 24 hours; if it is an ulcer, there may be no or minimal improvement in 24 h; if the defect is an in ltrate, it will be the same or worse the following day; at day 1 followup, the conservative antibiotic therapy can be continued for another day, or if your diagnostic decision is now in ltrate, then add loteprednol four tim es a day while continuing the antibiotic

16 Contact Lenses

465

peroxide care system . Lid t reat m en t w ould in clu de stan dard lid scru bs w ith n on irrit at ive agen ts, doxycyclin e 20 m g, 50 m g by m outh , or up to a 100 m g for bleph arit is or m in ocyclin e w ith lid clean sing (Cleeravu e–M, Ston ebridge Ph arm a, Du luth , GA) an d/or possibly cyclosporin e A drops—Restasis t w ice a day if th ere is a h istor y of ch ron ic rosacea. Th e m ajor con cern is w h eth er th e in filt rate is act ually a n on in fect iou s sterile ulcer or con tact len s–related periph eral u lcer (CLPU). If su spicious of u lcers, p rescribe an t ibiot ics on ly. Refrain from steroid u se, cu lt ure w h en possible, an d t reat w ith flu oroqu in olon e an t ibiot ics. In th is case, th e in it ial u se of a steroid w ou ld be con t rain dicated un t il after a sh or t course of a poten t an t ibiot ic su ch as m oxifloxacin (Vigam ox, Alcon Laboratories) or gat ifloxacin (Zym ar, Allergan ). In th e case of in filt rates, th ere w ill be n o respon se to an t ibiot ics. If th ere is an u lcer, th ere w ill be a favorable respon se to an t ibiot ics, w h ich can be follow ed by th e in t roduct ion of steroids after th e loading dose h as redu ced th e bacterial burden . As an added com m en t for pain con t rol w ith corn eal u lcerat ion , am ple cycloplegia u sing h om at ropin e 2 to 5%or a m ore frequ en t dose of cyclopen tolate 1%w ill in m any cases suffice w ith ou t th e n eed for steroid ut ilizat ion . Oral an algesia for pain can be in t rodu ced using basic acet am in oph en or ibuprofen or both as n eeded. Th e progn osis of t reat ing in filt rates is h igh ly favorable w ith sym ptom s an d fin dings dissipat ing in a sh ort cou rse of a few days. In filt rates th at are den ser an d m ore cen t ralized, su ch as w ith an aden ovirus, w ill t ake longer to resolve an d m ay h ave a profoun d effect on vision requ iring longer-term care an d slow t apering of m edicat ion s, part icu larly w h en u sing steroidal th erapy from h ard to soft steroid topicals.

Contact Lens-Related Acute Red Eye Con tact len s-related red eye (CLARE) is an acu te, n on specific, n on ulcerat ive sterile keratoconju n ct ivit is h as in flam m ator y associat ion w ith th e adh eren ce of debris from exogen ou s m at ter, m et abolic by-p rodu cts, or vest iges of bacterial debris an d exotoxin s th at in duce th e recruit m en t of in flam m ator y cells. Th e exotoxin s are from th e breakdow n of t rapped debris or devitalized bacteria w ith in th e closed eye environ m en t . Presu m pt ively, th e greater risk is bacterial in filt rat ion an d colon izat ion by Staphylococcus an d Pseudom onas th at m ay lead to CLPU; th ere is suggest ion th at som e pat ien ts h ave h igh er levels of gram -n egat ive con tam in at ion . Con tact len s acute red eyes (CLAREs) h ave a variet y of cau ses. CLARE cou ld be con sidered an in flam m ator y con dit ion associated w ith hypoxia, toxic effect s from post-len s tear debris, m ech an ical irrit at ion from a poorly fit t ing len s, dehydrat ion of th e len s du ring sleep, solut ion hypersen sit ivit y or toxicit y, or a react ion to bacterial toxin s. Du e to poten t ial hypoxic con dit ion s associated w ith len s u se, cellular glu cose convert s to lact ate. In addit ion , lactate diffu sing in to th e st rom a in creases th e osm olarit y, leading to m et abolic acidosis w ith resu ltan t corn eal edem a. A decrease in n orm al corn eal m etabolism com p rom ises corn eal t issue leading to CLARE. W h ite blood cells m igrate from th e lim bal vasculat ure an d form in filt rates in th e periph eral corn ea.

466

Color Atlas of Ophthalm ology

Causes of Acute Red Eye Th e con t act len s relat ion sh ip is via len s-in duced m ech an ical factors or from len s deposit s th at lead to inju r y or m icrot rau m a to th e corn ea. Microt rau m a en cou rages th e m igrat ion an d in filt rat ion of in flam m ator y cellu lar con st it u en t s. In th e case of len s deposits, th ese ser ve as an an t igen et ic sou rce th at t riggers an im m u n e respon se leading to in filt rates. Th e casu al relat ion sh ips are eith er (1) t igh t len s syn drom e, (2) tear-film deficien cy/dr y eye [i.e., con t act len s–in du ced dr y-eye (CLIDE)], (3) bacterial conju n ct ivit is, (4) in flam m ator y react ion to debris on th e back su rface debris (m et abolic an d/or exogen ous debris stagn an t bet w een th e len s an d corn eal su rface), (5) m ech an ical irrit at ion /abrasion , (6) solu t ion toxem ia/ hypersen sit ivit y, or (7) irrit at ion to len s deposits. Th e in ciden ce of corn eal fin dings w ith 30-day con t in uou s-w ear silicon e hydrogels h as been foun d at an occurren ce rate of 10% for CLPU an d 29% for CLARE ( Fig. 16.5 ).

A B

C

D

Fig. 16.5 (A) Contact lens acute red eye (CLARE) secondary to a small foreign body. (B) CLARE secondary to a small foreign body. (C) Sectoral CLARE—tight lens syndrome—differential diagnosis episcleritis. (D) CLARE secondary to solution toxemia.

16 Contact Lenses

467

E

F Fig. 16.5 (Continued) (E) CLARE—tight lens syndrome. (F) CLARE—tight lens syndrom e—CLIDE.

Presentation CLARE h as a gen eric appearan ce of a n on descript “red eye” directly associated w ith th e u se of con t act len s exten ded w ear m ore often th an w ith daily w ear. Th e acu te react ion could be obser ved as a n on specific red eye w ith lim bal hyperem ia, con jun ct ival inject ion , corn eal in filt rates, an d a possible corn eal edem a th at is lim bal m ore so th an cen t ral. Upon len s rem oval, th e pat ien t m ay experien ce a greater level of ocular discom for t an d m ay exh ibit pu n ctate keratopathy eviden ce w ith posit ive fluorescein st ain ing associated m ostly w ith t rapped debris, a prim ar y cau se for th e in duced in flam m ator y con dit ion . Th e pat ien t w ill describe a “garden variet y” red eye w ith un ilateral, som etim es bilateral, variable levels of discom fort or pain , redn ess, epiph oria, ph otoph obia, and disch arge described as w ater y to m ucopurulen t. The am ount of vision reduction , pain , and discharge w ill assist in th e different ial diagn osis an d un derlying et iology. If th e con dit ion is contact lens related, a histor y of exten ded or con tin uous len s use w ill have a h igher in cidence th an daily w ear reusable m ore so th an single-use len ses. If a contact len s patient presen ts w ith an ARE (acute red eye), it is im portan t in the h istor y to determ in e th e w ear m odalit y of th e len s. Con tinuous an d exten ded w ear schedules w ill dem on strate a h igher incidence of CLARE th an daily w ear or single use lens w ear sch edules. It should be assum ed th at all con tact len s w earers m ay h ave th e presen ce of m icrobial kerat itis an d ulcer, unt il proven oth er w ise. Th is is im portan t in clin ical m an agem en t, for m any patients ten d to self-treat or h ave been treated in appropriately for a “garden variet y conjunct ivitis” by a prim ar y care physician (PCP). Because of th e potent ial of a potent ially devastating ulcer, such as Acantham oeba , Pseudom onas, or Fusarium , it is im portant to stress to PCPs th at if a “red eye–contact lens” patient presents to th eir office, they should defer treatm en t and seek a con sult w ith an ophth alm ologist or optom etric physician.

Differential Diagnosis As st ated, an acu te red eye presen t s as a garden variet y of red eye th at h as a dist in ct ch aracterist ic of rapid on set w h en related to con t act len s, w ith th e h igh poten t ial of being ulcerat ive, bu t can also m im ic or be directly related to m any oth er form s of ocu lar disorders su ch as bacterial, viral, allergic, or ch lam ydial in fect ion s. If th e pat ien t is n ot a con tact len s w earer, th is is n ot CLARE bu t is m ore probably a bacterial conju n ct ivit is. Th e differen t ial of th e CLARE pat ien t , due to th e con t act len s

468

Color Atlas of Ophthalm ology

associat ion , is alw ays u lcer first un t il proven oth er w ise. On ce proven oth er w ise, by cult u re or by an t ibiot ic t reat m en t , CLARE w ill rem ain as a red eye given the inflam m ator y nature. The inflam m at ion cou ld also be an u n derlying irit is in absen ce of an an terior ch am ber react ion an d n orm al pu pils. If th ere is a sectoral com pon en t to th e CLARE, th en con sider a con tact len s periph eral ulcer (CLPU), episclerit is, su perior lim bic kerat it is (ru le out thyroid disease), vascu larized lim bic kerat it is, or ocu lar su rface in flam m at ion associated w ith a pingueculae or pter ygiu m . Th ese are an atom ically obviou s.

Management In m any cases, th e pat ien t self-t reat s w ith over-th e-coun ter vasocon st rict ive lubrican t drop s w ith ou t relief. In m any oth er cases, th e pat ien t w ill presen t to a PCP for a garden variet y conju n ct ivit is th at is first t reated w ith an t ibiot ics. Precau t ion ar y care is requ ired. In m any in stan ces, a n on oph th alm ic–n on optom et ric provider m ay h ave star ted t reat m en t , th ereby disgu ising a possible et iology of CLARE. As such , th e pat ien t m ay h ave already been t reated w ith a sulfacet am ide 10% op h th alm ic preparat ion th at does n ot allow th e con dit ion to resolve an d in fact w orsen s th e con dit ion if th e pat ien t h as su lfa drug sen sit ivit y. Or in oth er cases, th e pat ien t m ay h ave been given a variet y of eith er am in oglycosides, flu oroqu in olon es, m acrolide, or an t iallergy m edicat ion s, som e h aving an effect or n o effect at all. In m any cases th e sim ple discon t in uan ce of th e con t act len s an d use of glasses for a few days is sat isfactor y. If th e con dit ion resolves w ith th is m ode of t reat m en t , it suggests a sim ple m aterial an d w ear con dit ion issue th at n eeds to be addressed. If th e pat ien t rein t roduces, or rech allenges th e u se of th e sam e m aterial an d w ear sch edu le (i.e., exten ded-w ear or con t in u ou s-w ear m odalit y), an d th e con dit ion rem an ifest s, th e rech allenge defin es th e n eed to readdress len s u se by refit t ing th e pat ien t w ith a n ew m aterial, w ear sch edu le, an d care p rodu ct . Treat m en t som et im es determ in es th e differen t ial diagn osis in th e absen ce of corn eal fin dings. As th e caveat w ou ld suggest , “do n o h arm ,” th erefore it is best to t reat th e eye w ith an t ibiot ics, an d if n eeded for cycloplegia, for a m in im um of 24 to 48 h ou rs prior to th e in t rodu ct ion of a steroid to avoid a possible exacerbat ion of an u n derlying ulcerat ive or h erpet ic: viral, fungal, or protozoan en t it y. Aggressive an t ibiot ic th erapy sh ou ld be th e first course of th erapy w h en m aking th e assum pt ion of ulcer, an d a flu oroqu in olon e sh ould be in t roduced. Moxifloxacin (Vigam ox, Alcon Laboratories) or gat ifloxacin (Zym ar, Allergan ) sh ould be th e first ch oice; h ow ever, th ird-gen erat ion flu oroqu in olon es w ill su ffice. If th ere is som e resolu t ion w ith th e an t ibiot ic, th en th e CLARE w as n ot in flam m ator y but in fect iou s. If th ere is m in im al to n o respon se to an t ibiot ics, th en a steroid, such as predn isolon e 1% fou r t im es a day, to rid th e in flam m ator y com pon en t of CLARE can be in t rodu ced safely, after th e loading dose of an t ibiot ic redu ces th e bacterial load. After th e successfu l resolu t ion of CLARE, th e pat ien t sh ould be refit w ith a n on ion ic, h igh -w ater-con ten t , deposit-resistan ce len s or a n on ion ic silicon e hydrogel len s m aterial w ith th e rest rict ion to daily w ear u se an d n o exten ded or con t in u ous w ear. Peroxide-based care produ ct s are recom m en ded w ith vigorous rubbing to clean se debris an d con t am in an ts. Also con sider gas-perm eable len ses th at w ill allow for n ot on ly an appropriate h igh oxygen perm eabilit y bu t also a flat ter or hyperbolic periph eral cu r ve an d edge design th at facilit ates su fficien t tear pu m p an d exch ange.

16 Contact Lenses

469

Contact Lens-Induced Dry Eye Som et im es described as th e m in im al sicca syn drom e, con tact len s–in duced dr y eye forces a borderlin e keratoconju n ct ivit is sicca pat ien t in to a full m an ifest at ion of sym ptom s an d fin dings associated directly w ith a fu lly m an ifested dr y eye w ith th e in t rodu ct ion of a con tact len s on to th e ocu lar su rface. Th e len s act s as an obst acle an d com pet itor w ith th e n at ural tear film , leading to in sult th at w ill ju st ifiably cau se a react ion by th e eye leading to th e ch ange in its n at u ral tear film physiology an d m etabolism . Th is w ill lead to in toleran ce, in flam m at ion , an d a com prom ise of th e ocu lar su rface.

Causes of Contact Lens-Induced Dry Eye Th e n orm al tear-film environ m en t is at t acked by th e in t roduct ion of a con t act len s. In it ially, th ere is a reflexive in crease in tear product ion . How ever, over t im e tear produ ct ion w ill “fat igu e” th e system , decreasing th e effor t s of th e lacrim al system an d in creasing th e poten t ial for con t act len s deposits, m icrobial in fect ion , an d corn eal in filt rat ion , corn eal edem a, an d u lt im ately p at ien t dissat isfact ion an d in toleran ce to con tact len ses. Th e in t roduct ion of th e con t act len s to th e ocular surface w ill disru pt th e h om eostat ic balan ce of th e tear film , requiring a n ew balan ce to be establish ed bet w een th e pre-len s ocular tear film an d th e post-len s ocu lar tear film –precorn eal tear film . As deposit s or su rface film accum u lates, blin king com presses th e tear film an d rem oves th e lipid-con t am in ated, hydroph obic m u cu s an d debris from th e len s–tear su rface. Th e in tegrit y of th e precorn eal an d len s tear film is directly propor t ion al to th e abilit y to m ain t ain proper con tact len s w et tabilit y an d len s su rface hydrat ion . If th e lipid layer is poor, th e evaporat ive process in creases, leading to a greater loss of aqueous an d th e in duct ion of a for w ard osm ot ic draw across th e con t act len s surface leading to len s dehydrat ion an d corn eal desiccat ion . W ith len s dehydrat ion , th e hydroph ilic len s w ill steepen , m ech an ically pu lling on a w eaken ed ep ith elial su rface, allow ing for corn eal com prom ise visualized as cen t ral corn eal epith elial desiccat ion an d/or cell jun ct u re split t ing or separat ion . Also, w h en th e ocular su rface becom es “u nprotected,“ th ere is th e developm en t of n eu ron al hyposen sit ivit y associated w ith hypoxia an d th e barrier effect created by th e con t act len s in terface. As th e con tact len s develops a su bst an t ial dehydrat ion it w ill ten d to vau lt aw ay from th e ocu lar su rface, leaving an exposed gap bet w een th e post-len s surface an d th e corn eal su rface. Th e gap h ow ever is n ot flu id filled an d leaves th e ocu lar surface u nprotected, leading to com prom ise an d dessicat ion of th e epith eliu m an d aberrat ion to n eural regulat ion an d biofeedback to th e lid st ru ct ure ( Fig. 16.6 ).

470

Color Atlas of Ophthalm ology

A

B

C

D Fig. 16.6 (A) Classic appearance of contact lens acute red eye (CLIDE) in patient exhibiting circumlimbal injection, marginal erythema, conjunctival injection, and immobile lenses. (B) Minim al lacrimal lake as demonstrated by lissam ine green. (C) Disrupted tear film spread with subsequent paracentral punctate keratopathy associated with CLIDE. (D) Lissamine green staining of the conjunctiva in a CLIDE patient.

Presentation Th e pat ien t w ill presen t w ith a CLARE-t ype ap pearan ce th at h ad been som ew h at ch ron ic. Th e eyes w ill be described as feeling t ired, dr y, an d irritated an d alw ays red, par t icularly later in th e day. It w ou ld also be n oted th at pat ien t s h ave difficu lt len s rem oval an d describe a feeling of relief u pon len s rem oval. Often th e pat ien t w ill proceed w ith vigorou s eye rubbing after len s rem oval. In som e cases of difficu lt len s rem oval, th e eye feels overly sen sit ive an d presen t s w ith an in crease in inject ion du e to th e in adver ten t rem oval of su perficial epith elial t issu e an d n eu ron al exp osure due to epith elial com prom ise during len s w ear associated w ith len s dr yn ess an d bin ding. Su pplem en tat ion w ith topical drops su ch as lubrican t s m ay

16 Contact Lenses

471

or m ay n ot ben efit th e pat ien t , leading to self-lim itat ion of len s w ear or even discon t in u an ce. Th e clin ician w ill obser ve a CLARE-t ype red eye w ith ou t in fect ion th at appears to be ch ron ic. Th ere m aybe a m ild to m oderate corn eal stain ing presen t due to len s vau lt . Th e len s m ay appear som ew h at im m obile, suggest ing an in duced t igh t len s syn drom e or sm all petech ial h em orrh ages on th e conju n ct iva ju xtaposed to th e len s edge or on th e paralim bal conjun ct iva. Th ere is n eith er apparen t disch arge n or follicu lar n or papillar y react ion . How ever, in th e long-term sufficien t debris an d len s su rface dr yn ess can in duce a CLPC react ion . Tear film spreading abn orm alit ies w ill be seen w ith th e u se of various diagn ost ic test ing su ch as Sch irm er st rips, lissam in e green , assessm en t of th e tear break-u p t im e, sodiu m flu orescein stain ing of th e corn ea an d conjun ct iva, assessm en t of th e lacrim al lake–m argin al tear volum e as a variet y of test .

Differential Diagnosis Con t act len s–in duced edem a, w arpage, over w ear, oth er causes of redn ess an d discom for t associated w ith con tact len s u se. CLIDE is directly related to oth er ocu lar con dit ion s affect ing th e lacrim al–ocular su rface balan ce in cluding eyelid an d glan du lar dysfu n ct ion or disease, poor blin king m ech an ism su ch as lagoph th alm os, floppy lid, an d/or dysfun ct ion of th e lacrim al an d m eibom ian glan ds. Oth er associat ion s to CLIDE an d dr y eye in clude Sjögren syn drom e, au toim m un e disease, rh eu m atoid disorders, an d m edicat ion s, especially an t ih ist am in es, an t idepressan t s, an d oral con t racept ives.

Management Th e t reat m en t for dr y eye an d CLIDE is to relieve th e un derlying problem by first iden t ifying th e por t ion of th e tear film th at is dysfun ct ion al. On ce iden t ified, th e t reat m en t sh ou ld be biased to com plem en t th e len s w ith m in im al com plexit y to th e pat ien t . W ith th e u se of ocu lar lubrican ts as a su pplem en t or st im u lan t , th e eye becom es “subject ively com for table,” bu t lit tle is kn ow n in regard to th eir longterm effect on th e various tear film st ruct ures an d corn eal physiology. W ith respect to su pplem en t in teract ion w ith th e m aterial an d th e m aterial’s in teract ion w ith th e eye, th e con t act len s design an d m aterial are th e key long-term com for t an d physiological balan ce in th e poten t ial su ccess of th e con t act len s pat ien t . To ach ieve th e proper tear-film balan ce, th e CLIDE p at ien t m u st be t reated as a n orm al dr y-eye pat ien t . Follow ing a flow ch ar t of t reat m en t such as proper tear an d n ut rit ion al supplem en tat ion is th e first step. Th e select ion of supplem en tat ion an d/or m edicin al t reat m en t is crit ical. I prefer to t r y to defin e m edicin al care by th e determ in at ion of dr y eye as a “w h ite” or “red” dr y eye. If th e pat ien t presen ts sym ptom at ically an d object ively as a dr y eye yet h as a w h ite, n on in flam ed conju n ct iva, th e use of goblet cell–m ucin en h an cers in conju n ct ion w ith lacrim al glan d st im u lus (e.g., cyclosporin e) w ould be con sidered appropriate. If th e pat ien t appears w ith a red, in flam ed dr y eye, th en th e in ter ven t ion w ith steroids w ould be deem ed m ore ap propriate. Th is m ay also be com plem en ted w ith th e use of n u t rit ion al su pplem en t at ion of om ega 3 an d 6 essen t ial oils (fish an d seed oil sou rces), w h ich h ave a n at ural n on steroidal an t iin flam m ator y effect . In addit ion , tear an d len s rehydrat ion is w ell accom plish ed by using an t ioxidan t or elect rolyte-balan ced tear su pplem en t s. At th e sam e t im e, a clin ical decision m ust be m ade to refrain from con tact len s u se or lim it it during th e in it ial stages of th erapy. Pun ctal occlusion sh ould be reser ved for long-term len s com for t m ain ten an ce un t il posit ive result s are est ablish ed w ith topical an d oral th erapies.

472

Color Atlas of Ophthalm ology

Material select ion is crit ical in th e t reat m en t of th e CLIDE pat ien t . Alw ays con sider RGP len ses first for borderlin e dr y eye pat ien ts. A deficien t or u n st able tear film requires h igh oxygen perm eabilit y an d a len s w ith low surface react ivit y th at m oves adequ ately to m in im ize th e risk of com plicat ion s. Also con sider th e rech allenge of hydrogel-based grou p 2 m aterials of h igh -w ater, n on ion ic ch aracter such as h ioxifilcon an d ph osph at ylch olin e. As n oted, silicon e hydrogel m aterials m ay be appropriate for oxygen en h an cem en t bu t are n ot prom ising w h en t reat ing a defin ed CLIDE pat ien t . Silicon e hydrogels w ou ld be h igh ly desirable on ce th e CLIDE p at ien t h as been t reated an d th e eye h as resu m ed a feasible level of com for t an d p roper tear film balan ce.

Superior Epithelial Arcuate Lesions Th e separat ion of th e corn eal epith elium , occurring m ore so at th e superior lim bal m argin , is know n as superior epith elial accurate lim bal split (SEALS), or a sim ilar form of epith elial split ting th at follow s th e corn eal-lim bal border aroun d the cornea is know n as int ra-epithelial split ting (ILES) . It is ch aracterized by an obser vat ion of an arcuate staining ju xtaposed to the lim bus. Th e pat tern of th e full-th ickness corneal epithelial separation usually occurs in th e lim bal corn ea covered by th e upper eyelid, w ithin 2 to 3 m m of th e superior lim bus in th e 10- and 2-o’clock region. Th e m ain cause for th is en t it y is con sidered to in clude m ech an ical irrit at ion an d dehydrat ion of th e len s su rface. Th e et iology of SEALS an d/or ILES is con sidered to be eith er a m ech an ical pull or st ress on th e epith elial ju n ct ures du e to a t igh t len s or len s dehydrat ion or a m ech an ical ch afing w ith in th e paralim bal corn ea. As for SEALS, sup erior m ech an ical ch afing m ay be th e resu lt of inw ard pressure of th e upper lid at th e su perior in t ralim bus associated w ith len s design , rigidit y, an d surface ch aracterist ics. In com bin at ion th e excessive “frict ion al” p ressure an d abrasive sh ear force on th e epith elial surface in du ce a separat ion of th e epith elial cell jun ct u res. New m aterials, first-gen erat ion silicon e hydrogels, w h ich possess a st iffer elast ic m odu lu s, m ay cau se an in crease in th e sh earing force n ot on ly on th e corn ea, but also m ay in duce a sim ilar force on th e conju n ct iva leading to a pseu doim pression ring called a conju n ct ival flap. Th e con cern s w ith SEALS or ILES are th e provocat ion of an in flam m ator y an d/ or bacterial in filt rat ive respon se. Addit ion ally, excessive sh earing forces m ay lead to a degradat ion of lim bal stem cells require for corn eal repair m ech an ism s ( Fig. 16.7 ).

Presentation Presen tat ion is gen erally asym ptom at ic oth er th an m ild, obser vable inject ion to th e eye, par t icu larly upon len s rem oval. Th e pat ien t m ay also com plain of a m ild to m oderate len s aw aren ess, difficu lt len s rem oval, burn ing, or itch ing sen sat ion . Th e pat ien t m ay describe a sligh tly greater len s in toleran ce to stan dard low m odu lu s 2-hydroxyethyl m eth acr ylate-based len ses as com pared w ith h igh er m odu lus silicon e–hydrogel com bin ed polym ers. Upon biom icroscopic exam , th e gen erally asym ptom at ic p at ien t m ay presen t w ith conjun ct ival inject ion ju xt aposed to th e affected area. In th e case of SEALS, th e conju n ct ival inject ion w ou ld appear m ore st rictly bet w een 10 o’clock an d 2 o’clock versu s a 360-degree-t ype epith elial split t ing, w h ich w ou ld app ear m ore as th e CLARE-t ype eye associated w ith a t igh t len s syn drom e. Su bsequen tly, sign ifican t paralim bal stain ing appears in an arc abou t th e in t ralim bal corn ea.

16 Contact Lenses

473

Fig. 16.7 (A) Classic circumlimbal epithelial split ting with tear-film irregularit y. (B) Classic circumlimbal superior epithelial arcuate limbal split ting (SEALS) due to a tight lens syndrome with tear-film disruption and conjunctival congestion [contact lens acute red eye (CLARE)]. (C) Classic SEALS due to a tight lens syndrome with tear film disruption and conjunctival congestion (CLARE). A

B

C

Differential Diagnosis Th is is sim ply related to th e h istor y of len s use or th e lack th ereof. If th ere is n o h istor y of len s u se, th en on e n eeds to evalu ate th e localized conjun ct ival respon se to determ in e th e level of inject ion . A su perior lim bic kerat it is (SLK) m ay be an in dicator of su bsequ en t thyroid disease. If th e conju n ct ival respon se is tem poral or n asal, th en determ in e in flam m ator y con cern s of vascu larized lim bic kerat it is, episcleri-

474

Color Atlas of Ophthalm ology

t is, phylcten ulit is, pin qu ecu lit is, pter ygium , an d/or early m argin al degen erat ion s. For t un ately, SEALS or ILES, are m ore con sisten t w ith con t act len s use, exten ded w ear m ore so th an daily w ear. If su perior lim bal inject ion occu rs, part icu larly bilateral, con sider oth er issues su ch as thyroid disorder or corn eal disorder.

Management Sim p le len s discon t in u an ce is th e m ost appropriate, allow ing th e lesion to recover w ith in a few days to several w eeks. If th e SEALS or split is m in or, th e sim ple discon t in u an ce of th e len s is prop er based on th e pat ien t’s abilit y to fun ct ion w ith spectacles. As a precau t ion again st bacterial in filt rat ion , a sh or t course of topical an t ibiot ics m ay be ben eficial. In th is scen ario, a sim ple refit to a daily disposable len s w ith adequ ate an d frequ en t rehydrat ion is deem ed appropriate. Th e refit of len s sh ould n ot on ly reevaluate th e hydrat ion abilit y of th e len s but th e len s’ relat ion to th e corn eal topography an d asp h ericit y or corn eal con tou r. Th erefore, th e len s design sh ou ld com plem en t th e an atom y suggest ing a flat ter base cur ve or h igh er eccen t ricit y value. In conjun ct ion , sh or t-term pu n ctal p lugs can be con sidered an am ple adjuvan t th erapy to en h an ce epith elial h ealing an d m ain t ain len s an d corn eal hydrat ion . If th ere is a low -grade conjun ct ival respon se, a break w ith in th e epith elium cou ld be th e p oten t ial opp or t u n it y for bacterial con t am in at ion an d/or in flam m ator y respon ses. As such , th e level of th e con dit ion w ill w arran t th e level of th erapy. Again , discon t in u an ce is ap propriate, yet th e addit ion of a soft steroid w ith m ild an t ibiot ics su ch as tobram ycin /lotepredn ol (Zylet , Bausch & Lom b) fou r t im es a day for 5 to 7 days w ith th e addit ion of a bacit racin oin t m en t during sleeping h ou rs is con sidered prophylact ically safe. If th ere is a greater (m ild–m oderate–severe) ocu lar inject ion an d sign ifican t split t ing, com plete discon t in u an ce of th e len s is required w ith a m ore aggressive th erapy ut ilizing a com bin at ion steroid-an t ibiot ic drop an d oin t m en t or a separate flu oroqu in olon e drop an d oin t m en t w ith a secon dar y steroid su ch as p redn isolon e acet ate if th e con dit ion h as associated in filt rates or m ay lead to an u lcerat ive keratopathy. On ce th e con dit ion is resolved, th e pat ien t can be refit to a low er elast ic m odulu s len s, avoiding exten ded or con t in u ou s w ear or be refit to a h igh Dk gas perm eable len s.

Dellen-Epithelial Hyperplasia Dellen are focal areas of corn eal th in n ing t ypically located, usu ally at th e 3- to 9-o’clock lim bu s du e to dehydrat ion of th e region . Th ese form at ion s are th e act ive state of m ech an ical irritat ion of a sector or por t ion of th e corn ea ju xtaposed to th e edge of th e gas perm eable con tact len s. Th ey are con sidered a t ran sien t form of corn eal degen erat ion th at resu lts from st rom al dehydrat ion secon dar y to poor w et t ing by th e tear film seen n ot on ly w ith con t act len s w ear but also p ostsu rgically (e.g., cataract , LASIK, st rabism u s). It represen t s a localized th in n ing of th e corn eal an d scleral t issu e. Epith elial hyperplasia is m ore related to th e ch ron ic dehydrat ion an d m ech an ical ch afing of th e gas perm eable len s in a specific ju xt aposed area of th e corn ea. Th e et iology of th e dellen is local dehydrat ion . It is th ough t th at breaks in th e oil layer of th e tear film preven t th e proper w et t ing of th e t issue, leading to devitalizing dehydrat ion of th e epith elial cells. Du e to th e lack of proper oil an d aqueou s

16 Contact Lenses

475

A

B Fig. 16.8 (A) Corneal dellen secondary to gas permeable lens wear. (B) Epithelial hyperplasia secondary to gas permeable lens wear.

flu ids in th e locat ion , th ere is a greater ten den cy for len s bin ding an d m ech an ical ch afing. Mech an ical in flu en ces are in du ced by th e eyelids, w h ich are u n able to follow th e ch anging con tou r of th e len s edge forcing it to lift or bore in to th e epith eliu m . If th e len s edge creates a gap, th en th ere is a su bsequen t lack of w ett ing an d hydrat ion of th e corn eal t issu e in w h ich m u cin s are n ot spread over th e epith eliu m , resu lt ing in an area of n onw et t ing resu lts. Fur th er dehydrat ion eith er even t u ally causes corn eoscleral th in n ing to occur, w ith th e form at ion of a dellen or long-term local epith elial hyperplasia ( Fig. 16.8 ).

Presentation Pat ien ts w ith corn eal dellen m ay be asym ptom at ic but often report m ild ocu lar discom fort or a foreign body sen sat ion . Th e pat ien t m ay describe a sectoral redn ess to th e eye w ith len s aw aren ess over t im e. Th ere m ay be in creased epiph oria as w ell as n ot iceable len s decen t rat ion . Su bsequen tly, th e pat ien t m ay describe dr y eye–t ype sym ptom s an d in creasing len s in toleran ce. Addit ion ally, th e pat ien t m ay exh ibit an in com plete or errat ic blin k reflex w h en ut ilizing len ses. Dellen appear as pale–kerat in ized w h ite “saucerlike” depression s w ith in , usually th e 3- to 9-o’clock area in t ralim bal ju xt aposed to th e edge of th e len ses. Rarely are th ese lesion s larger th an 2 m m . If th e overlying epith elium is in t ake, th ey w ill n ot st ain . Th ey m ay h ave a sligh tly vascularized su rface w ith adjacen t sectoral con jun ct ival inject ion . Flu orescein m ay appear to “gu t ter” an d surrou n d th e base of th e dellen . Th e st ain ing, at or n ear th e 3- an d 9-o’clock posit ion s, appears on or adjacen t to th e lim bu s of rigid len s w earers. Breaks in th e epith elium m ay allow for periph eral bacterial con t am in at ion leading to a kerat it is or u lcerat ion or in flam -

476

Color Atlas of Ophthalm ology

m ator y in filt rat ion . Becau se of th e localized dehydrat ion , subsequ en t cell desiccat ion occu rs an d n ecrosis of th e epith elial cells. Th is leads to epith elial hyperplasia sim ilar to corn eal scarring. Epith elial hyp erplasia is obser ved as a m ou n d of t issue th at becom es eroded an d irritated by th e len s edge due to ch ron ic dehydrat ion of t issue exposu re.

Differential Diagnosis Several p oten t ial differen t ials n eed to be con sidered w h en obser ving a dellen or epith elial hyperplasia. If th ere is sign ifican t sectoral conjun ct ival inject ion , th en on e sh ou ld also con sider vascularized lim bic kerat it is, episclerit is, ph lycten u lit is, pin qu ecu lit is, pter ygiu m , or early m argin al degen erat ion s su ch as Terrien’s. Th e m ost im por tan t differen t ial w ould be m argin al ulcerat ion , part icu larly if epith elium com prom ise is obser ved. If th e adjacen t conju n ct iva is quiet , th en on e m ay con sider ru ling ou t a preexist ing scar or postoperat ive scar from cataract or ext raocu lar m uscle surger y, filtering blebs, an d sclerit is, obviously differen t iated by h istor y.

Management Th erapy focuses on th e proper reepith elializat ion an d rehydrat ing th e corn ea. Copious lubricat ion ever y 1 or 2 h ours an d blan d oin t m en t at n igh t are recom m en ded. In par t icu lar, n u t rien t-based th erapy is m ost appropriate ut ilizing an elect rolyte-balan ced or an t ioxidan t-based drop. In addit ion , a dem ulcen t su ch as Syst an e (Alcon Laboratories) or En du ra (Allergan ), w h ich form s a n et w ork gel-like con sisten cy on th e ocu lar su rface an d acts as a tem porar y ban dage en h an cing epith elial h ealing an d revitalizing th e m icrovilli an d surface glycocalyx. In it ial t reatm en t w ill dem on st rate efficacy in 48 to 72 h ours, bu t rein sult occurs easily. Topical cyclosporin e can also be con sidered in sh ort- or long-term care. Topical an t ibiot ics are u n n ecessar y, except in ext rem e cases involving sign ifican t epith elial com prom ise to preven t in fect ion an d ulcerat ion . In th ese cases, th e in flam m ator y com pon en t is raised, an d th erefore a com bin at ion tobram ycin w ith dexam eth ason e or lotepredn ol m ay be appropriate in a sh or t cou rse of 7 days at fou r t im es a day. On ce th ere is proper resolu t ion , th e gas perm eable len ses can be refit w ith a diligen t reevaluat ion of th e p eriph eral corn eal an atom y versu s th e periph eral cu r ve an d edge profile of th e len s. Addit ion ally, blin king exercises en couraging th e pat ien t to force a com plete lid closu re, on ce ever y 2 or 3 secon ds, are essen t ial. Also, con t in u e proper lubricat ion w ith rew et t ing drops com plem en t ar y to th e m aterial of ch oice. Con sider h igh er dK m aterials w ith plasm a t reat m en t for en h an ced w ettabilit y. Ep ith elial hyp erplasia m ay resolve follow ing discon t in u at ion of len s w ear. W h en poor resolut ion occurs an d is an obst ru ct ion to len s refit t ing an d PTK excim er laser procedu re m ay be con sidered to flat ten th e area an d elevate th e devitalized cells.

16 Contact Lenses

477

Contact Lens-Induced Corneal Warpage Syndrome Th e syn drom e of “corn eal w arpage” im plies th at th e con t act len s h as iat rogen ically in duced an atom ical ch anges to th e corn eal surface. Th e w arpage is visu alized as an irregular ret in oscopic reflex, distort ion to topography, an d keratom et ric m ires, in duced irregu lar corn eal ast igm at ism , an d obser vable distor t ion s to th e corn eal su rface u pon len s rem oval. Con t act len s–in du ced corn eal w ar p age is m ore frequ en tly associated w ith polym ethyl m eth acr ylate (PMMA), m ore so t h an gas-p er m eable len ses; h ow ever, ~27% of rep or ted cases of cor n eal w ar p age h ave been at t r ibu ted to hyd rogel len s w ear. Corn eal con tou r–in du ced irregu lar it ies are con sid ered th e resu lt of p robable m ech an ical deform at ion , ch ron ic m et abolic in su lt , or a com bin at ion of both . Ad dit ion ally, w ar page is seen as secon dar y to len s bin ding or st agn at ion du e to len s d ehydrat ion or a CLIDE syn d rom e. Also, som e solu t ion in teract ion s m ay ten d to in du ce len s im m obilit y du e to th e level of solu t ion viscosit y leading to len s bin d ing. More com m on ly, h ard PMMA or rigid gas-perm eable len ses are recogn ized for th e in duct ion of corn eal w arpage or iat rogen ically in duced resh aping of th e corn ea w h en im properly fit or align ed to th e corn eal su rface. Th e corn eal epith eliu m , h aving a ver y “p last ic” or “m oldable” ch aracter, is easily resh aped by th e h igh m odu lus or st iffn ess of th e len s, w h ich m olds th e corn ea to th e sh ape of th e len s cur vat ure(s) un in ten t ion ally. Th is can be in ten t ion ally accom plish ed in a process called orthokeratology. Rigid con tact len s–in du ced corn eal w arpage is easily docum en ted an d m on itored via obser vat ion of topograph ic abn orm alit ies. Th ere w ill be in du ced cen t ral irregu lar ast igm at ism , in ferior steepen ing or sm ile-like pat tern (pseu dokeratocon us-like im ages), su perior flat ten ing, loss of radial sym m et r y, h igh ly irregular pattern s w ith loss of surface sym m et r y, or an im pression of a dem arcat ion con tou r lin e con sisten t w ith th e len s edge. In du ced w arpage w ith hydrogels are con sidered m ore t ran sien t; h ow ever, gas perm eable m ay be t ran sien t to p erm an en t . Hydrogel-in duced deform at ion s are related to a w eaken ed epith elium du e to lesser th an opt im al oxygen t ran sm issibilit y th rough various hydrogel len s m aterials. Subsequ en tly, a m isalign ed, t igh t , u lt rath in len s or ext rem e varian ts in elast ic m odu lus of len ses can in duce hydroph ilic corn eal w arp age syn drom e. In a posit ive effect , a h igh elast ic m odu lu s len s w orn in reverse can act u ally in du ce a subtle flatten ing–or th okeratological effect to th e corn ea of 0.50 to 1 diopter ( Fig. 16.9 ).

478

Color Atlas of Ophthalm ology

A

B

C

D Fig. 16.9 (A) Corneal wrinkling (convolutions) induced by ultrathin hydrogel lenses. (B) Hydrogel corneal warpage as seen with sodium fluroscein stainage. (C) Corneal im pression ring induced by orthokeratologically fit lens. (D) Irregular keratometric mires by induced corneal warpage from a rigid gas permeable lens.

Presentation Pat ien ts w ill com plain of poor or distor ted vision w ith len ses w ith su bsequen t reduct ion in visual acu it y. Th ey w ill ten d to u t ilize m ore rew et t ing drops th at ten d to assist in clearing th e vision m om en t arily, yet do n ot resolve th e visu al decrem en t . Th e u n in ten t ion al m olding or w arpage of th e corn ea leads to subject ive visu al distort ion an d com plain t s of postlen s rem oval spectacle blu r or in abilit y to w ear glasses an d object ive m easu res of regression of pow er if th e len s is fit flat an d reduct ion of ast igm at ism can be m ade. In m any cases, par t icularly w ith hydrogel len ses, su btle distor t ion s can n ot be obser ved by biom icroscopy bu t m ay be m ore obviou s on topography an d aberrom et r y. In th ese cases, h istor y of p oor vision or n on opt im al vision is th e on ly descriptor by th e pat ien t . Th is w ill often occur w h en on e is refit t ing from older conven t ion al design s to frequ en t-replacem en t , th in n er len s design s th at m ay lack th e proper align m en t to th e corn eal topography.

16 Contact Lenses

479

E Fig. 16.9 (Continued) (E) Topography of warpage—RGP-induced corneal warpage at present and at 1 week lens discontinuance.

Clin ical sign s of corn eal w arpage are first docum en ted by redu ced, su bject ive an d object ive, visu al acu it y w ith con t act len ses an d subsequ en tly persisten t acu it y reduct ion w ith spectacles. Th e visual redu ct ion m ay be su btle (20/20 m in us) or pron ou n ced. On ce th e len ses are rem oved for an adequate period, th e p at ien t’s m an ifest refract ion ret u rn s to n orm al w ith con sisten t im provem en t in acuit y m easures. During th e exam in at ion , ret in oscopy reflexes w ill appear “w arped” or yield a “scissor” reflex often seen in keratocon u s. Keratom et r y an d topograph ic m easures dem on st rate varian t levels of irregu larit ies. Par t icu larly, descript ive st at ist ics w ith topography w ill be h igh ly variable as com pared w ith prefit m easures, som et im es m im icking a disease st ate.

480

Color Atlas of Ophthalm ology

Differential Diagnosis Histor y of len s use an d subject ive com plain t s w ill defin e th e con cern . How ever, a spect acle blur from len s-in du ced w arp age occurs eith er due to th e m ech an ical distor t ion or from corn eal edem a w ith corn eal h aze. If th ere is a w eaken ed en doth eliu m , part icularly w ith th e exten ded-w ear pat ien t or an older in dividu al, corn eal edem a m ay occur secon dar y to im bibem en t of flu id th rough en doth elial cell jun ct ion s an d m ay lead to associated cen t ral corn eal st riae, folds, or clouding. Th erefore, en doth elial polym egath ism , polym orph ism , an d reduced en doth elial cell cou n t m ay be th e u n derlying cau se. Advan ced or u n con t rolled glaucom a w ith sign ifican t corn eal edem a can also cau se corn eal w arpage or distor t ion s, part icu larly w ith a low er th an n orm al (540 µm ) cen t ral corn eal th ickn ess. On ce th e IOP is con t rolled, th e distort ion of th e corn ea dissipates an d refract ive recover y occu rs rapidly. High ly ast igm at ic, n on diseased, corn eal an atom y m ay also dem on st rate topograph ic irregularit ies. Th e irregu lar corn eal ast igm at ism m ay h ave a decen tered corn eal cap or a h igh asym m et r y of sup erior versus in ferior, tem poral versus n asal, sect ion s of th e corn ea. In th ese cases, a con tact len s is ver y difficult to cen ter on th e u n u su al lan dscape of th e corn eal topography an d w h en don e so, it w ill h ave a h igh er propen sit y to decen ter tow ards th e steeper m eridian an d bin d, cau sing in duced m olding to th e corn ea. Redu ced vision in th ese pat ien ts is n ot alw ays in du ced by th e con t act len s, bu t w ill h ave an en h an ced ap preciat ion for visu al aberrat ion s an d m ay also h ave a m eridion al am blyopia. Scarring or oth er path ologies th at in duce m ech an ical distor t ion s to th e corn ea can also m im ic corn eal w arpage. In part icular pter ygium , pulling across th e corn eal su rface, h as a m ore global th an localized effect , an d pan n u s or aberran t con jun ct ival t issue m ay in du ce sim ilar m ech an ical distor t ion s. Ect act ic corn eal degen erat ion s such as p ellu cid or keratocon u s m ay n ot h ave been determ in ed at th e in it ial len s fit t ing of th e pat ien t m any years prior. Form e fruste keratocon us (absen ce of classic an atom ical fin dings) is often m issed in th e in it ial fit if topography an d aberrom et r y are n ot properly ut ilized. Du e to th e in creased in terest in refract ive surgical procedures, m any of th ese pat ien ts are being discovered in preoperat ive care an d ju stly den ied th e procedure. Literat u re suggest s th at PMMA an d gas-perm eable len ses at on e t im e in duced keratocon us. In fact , th e len ses did n ot in du ce th e disorder, bu t in stead cau sed a “pseudokeratocon ic” distort ion of th e corn ea, w h ich , in som e cases, is perm an en t . Pseu dokeratocon u s in du ced by con t act len ses w arping of th e corn ea produ ces a pat tern th at m im ics keratocon us eviden ced in a localized area of in ferior corn eal steepen ing. Upon len s discon t in uan ce th e in du ced steepen ing w ill regress to a n orm al “bow t ie” or “h ourglass-sh aped” topography w ith sym m et r y, m u ch un like a t ru e keratocon ic eye.

Management Diagn osis by h istor y, refract ion , ret in oscopy, an d corn eal im aging is crit ical. Serial m easu res after len s discon t in u an ce w ill determ in e th e severit y, t ran sien ce, or p erm an en cy an d th e fin al en d poin t prior to refit or su rgical procedu re. If con cern s of corn eal edem a arise, serial pachym et r y is a requ ired m easu re. If th ere is sign ifican t corn eal edem a, len s discon t in u an ce is recom m en ded w ith th e in corporat ion of hyperosm ot ic agen t s w ith repeat pachym et r y t ill th e pat ien t dem on st rates refract ive recover y an d stable cen t ral corn eal th ickn ess (CCT) m easu res. If th e corn eal distortion s are in duced by hydrogel soft len ses, th e sim ple discontin uance for a few days and refit to a slightly thicker or interm ediate elast ic m odulus, high-w ater, high -oxygen perm eabilit y, an d param eter-stable lens is appropri-

16 Contact Lenses

481

ate. Th ese w ould include a group 2 len s such as Proclear (CooperVision, Fairport, NY), Extrem e Water (Hydrogel Vision Corp., Sarasota, FL) or a low er elastic m odulus silicon e hydrogel. In these cases, the in duced distortion is tran sisent an d resolves in a few h ours. Mon itor w ith topography an d aberrom et r y un til stabilit y of refract ion and vision occurs prior to com pleting a refit ting of the contact lenses. In t reat ing corn eal deform it y or w arpage in duced by gas-perm eable len ses, on e cou ld take several approach es. Sim ply discon t in ue con t act len ses an d rever t to eyeglass w ear for several days to w eeks u n t il th e stabilit y of th e corn ea can be determ in ed. In th e m ajorit y of cases, th is is n ot an at t ract ive opt ion for th e pat ien t becau se vision w ill dyn am ically ch ange an d th e pat ien t likely does n ot h ave a pair of glasses to w ear du ring th is period. Or first obser ve th e len s on eye, th en rem ove an d perform a dr y refract ion , p achym et r y, an d topograph ic or aberrom et r y m easu res. Th ese m easu res w ill be repeated several t im es in th e “reh abilit at ion process” u n t il st abilit y of m easu res occu rs. Defer cycloplegia u n t il resolu t ion after several serial m easu res. Du ring th e “recover y” or “corn eal reh abilitat ion ” period, refit th e pat ien t w ith hydrogel soft len ses to th eir best ach ievable acu it y. On aftercare, repeat diagn ost ic m easu res an d refit w ith soft len ses according to th e resu lt s. Con t in u e th is process u n t il corn eal an d refract ive stabilit y is ach ieved. A crit ical poin t in pat ien t care is to w arn th e pat ien t of th e dyn am ic ch ange to vision an d th e frust rat ion th at w ill probably pursu e. Pat ien t reassuran ce is vital, en couraging th em to im m ediately ret u rn for con t in ued care if th e vision becom es u n acceptable. St abilizat ion of th e corn ea is defin ed as th e poin t th at th e m an ifest refract ion is con sisten t t w o to th ree t im es w ith in a –0.50 diopters sph ere an d cylin der, w ith axis w ith in 10 to 15 degrees of origin al m an ifest , proper sym m et r y to th e topography an d p achym et r y w ith in n orm al ranges of prefit est im ates or m easu res. Not ing th at , based on th e exten t an d severit y of corn eal w arpage, 20/20 (6/6) m ay n o longer be ach ievable by th e pat ien t . On ce st able, th e p at ien t can be refit to any len s th at is deem ed app ropriate, be it soft hydrogel or gas perm eable or orth okeratology or a refract ive surgical procedure. If con sidering or th okeratology or refract ive su rger y, even cataract procedu res on pat ien ts w h o h ave w orn gas-perm eable len ses for any exten t of t im e, a “corn eal reh abilit at ion ” sh ou ld be in corp orated in to th e prefit or preoperat ive care. Th ere is n o single r ule for p reoperat ive care. Som e m igh t suggest th at corn eal stabilit y is ach ieved bet w een 8 an d 20 w eeks, or even u p to 6 m on th s. In m y experien ce it is suggested th at approxim ately 1 to 2 w eeks’ discon t in u an ce of gas perm eable len ses for ever y year of w ear be obser ved prior to a refract ive su rgical procedure, m ore t im e if th e pat ien t h ad w orn PMMA len ses on ly.

Contact Lens Deposits Deposit s are th e accu m ulat ion of debris on th e surface. Th e variou s t ypes of deposit accu m u late based on th e m aterial ch em ist r y ch aracterist ics su ch as w ater con ten t , ion ic versu s n on ion ic surface t reat m en ts, or physiological in teract ion s of th e con st it u en t s of th e tear film or th e abilit y of th e lids to spread th e tear film an d clean se th e con t act len s surface. Deposit s are m u lt ifactorial w ith a variet y of t ypes ( Fig. 16.10 ).

482

A

Color Atlas of Ophthalm ology

B

C

D

E

F

G Fig. 16.10 (A) Mucin balls and surface film silicone hydrogel lenses. (B) Mucin balls and surface film silicone hydrogel lenses. (C) Severe lipid–protein –m ucous surface film on soft hydrogel lenses. (D) Severe lipid protein calcium deposits—lens calculi. (E) Severe protein film rigid gas permeable (RGP). (F) Severe nonwet ting secondary to surface film RGP. (G) Filming of a prosthetic eye.

16 Contact Lenses

483

Presentation Th e un derlying problem is th e im proper clearan ce of m et abolic an d/or exogen ou s debris th at accum u lates on or pen et rates w ith in th e len s m aterial. Th is accum ulat ion of debris an d w aste is due to poor tear ch em ist r y, in appropriate or in com p lete clean ing by th e len s care product , or a poor lid in teract ion to spread th e tear film an d clean se th e len s su rface. Th is leads to deleteriou s in teract ion s w ith th e palpebral conjun ct iva of th e su perior eyelid th at leads to a m ech an ical an d an t igen ic resp on se causing CLPC. Protein s in th e tear film are com p osed of organ ic am in o acids of variou s elect rolytes. Oxidat ion by h eat ing or ult raviolet exposu re en courages th e den at uring (ch em ical t ran sform at ion ) or alterat ion of th e protein s, th e m ost predom in an t being lysozym e, w h ich h as a st rong an t im icrobial ch aracterist ic. W h en th e protein s ch ange th eir ch aracter, th ey n ow becom e foreign to th e system , in it iat ing an autoim m u n e hypersen sit ivit y respon se. An t ibodies are st im u lated leading to su bsequen t in flam m at ion , inject ion or er yth em a, an d pruisit is. As th e deposit s accum u late, th ey becom e “space occupying” n odu les on th e back surface th at lead to localized im pression s on th e corn eal epith elial su rface, seen as discrete pu n ct ate stain ing. Also, th e den at ured protein s can lead to a cytotoxic respon se an d a gen eralized su perficial pu n ctate kerat it is. Th e su rface protein s can also act as n u t rit ion to n orm al flora, w h ereas m ucu s can en capsulate an d n ur t ure th e colony. As such , th e flora rep licate in to a larger colony, w h ich is beyon d th e n orm al balan ce an d defen ses of th e ocu lar surface. If th ere is a break in th e ep ith eliu m , a sequela of in filt rat ion w ith resu lt an t m icrobial kerat it is or ulcerat ion m ay occu r. Th e pat ien t , in m any cases, is asym ptom at ic oth er th an describing variable dr yn ess, itch , redn ess, an d visual con cern s w ith th e len ses. In th e m ajorit y of cases, w h en frequ en t replacem en t len s m odalit ies are properly u sed, th e deposits do n ot becom e sign ifican t en ough to cause a n ot iceable p roblem . Rarely, in few pat ien ts, len s deposits form qu ickly du e to th eir poor tear ch em ist r y. In frequ en t-replacem en t len ses, len ses used less th an 30 days, deposit s ten d to create a “film ” th at clean s off easily w ith th e prop er care product , yet reaccum u lates m ore rapidly each day of len s w ear u n t il it is discarded. In pat ien ts w h ere deposits becom e excessive, th e len s w ill in teract aggressively again st th e lid, leading to in flam m at ion such as CLPC. Th e pat ien t w ill on ce again be som ew h at asym ptom at ic oth er th an describing a len s th at frequ en tly decen ters or even dislodges. Th ere w ill also be a “conju n ct ivit is t ype” disch arge an d redn ess w ith ou t discom for t . In th ese cases, ver y often , th e PCP is con sulted an d w ill prescribe an an t ibiot ic in appropriately (see CLPC an d ARE). In oth er cases, th e p at ien t w ill presen t w ith an acute red eye, w ith discom fort to pain , copiou s w ater y or pur ulen t disch arge, w ith possible effects on visu al abilit y. Th is w ou ld im ply a secon dar y bacterial con tam in at ion du e to a con t act len s–associated n ut rien t- an d p rotein -rich biofilm . Th e biofilm allow s bacteria, even n orm al flora, to “feast” an d in crease colon izat ion beyon d levels in w h ich th e eye can defen d it self. Also, w ith th e lack of tear lysozym e, th e bacterium h as a great abilit y to popu late an d invade. In th ese cases of u su al abuse to th e con tact len s an d lack of proper care, th e corn ea is also com prom ised, allow ing for a vast oppor t un it y for bacterial in filt rat ion an d su bsequen t u lcerat ion of th e corn ea. Th e m ost com m on are Staphylococcus epiderm idis an d aureus, follow ed by Pseudom onas aeruginosa . As m icroorgan ism s spread, th ey w ill eith er im m ediately at tack th e corn ea or serpigin ou sly spread over th e len s su rface. Th ese are gray, black, brow n , or w h ite grow th s in th e len s m at rix. Fu ngi or yeast w ill appear in a filam en t ar y pat tern , obser ved as h aving a cen t ral den sit y an d t ran slucen t fron ds prot r uding from th e core. Most com m on ly, surface m icroorgan ism s are associated w ith con tam in at ion

484

Color Atlas of Ophthalm ology

from variou s n on sterile w ater sources, su ch as pools, recreat ion al w aters (pon ds, rivers, lakes), con tam in ated tap w ater, n onp reser ved salin e, or, as m ore recen tly seen , in effect ive con t act len s care produ cts. Cat ast roph ic in fect ion s related to th e acan th am eoba (a protozoa) an d fusarium (a fu ngal in fect ion ) h ave been cited in an outbreak of cases w orldw ide in 2005 th rough 2006.

Differential Diagnosis Protein Deposits Th e m ost com m on t ype of deposit is protein based origin at ing from tear com pon en ts of album in , globu lin , an d lysozym e. Th e deposit is ch aracterized by it s opaque, th ick, den se, w h ite film w ith st riat ion s, fou n d on hydrogel len ses, p redom in an tly on th e an terior su rface versus gas perm eables th at accum ulate th ese deposit s on both surface an d par t icu larly in th e perip h eral cur ves. Protein u ptake occurs w ith in m om en t s upon len s in ser t ion an d varies to th e t im e of m at rix sat urat ion . Sat u rat ion of th e m at rix is h igh ly depen den t on th e polym er ch aracterist ic. Ion ic len ses w ill ten d to absorb an d bin d a great am ou n t of protein , n on ion ic as w ell as plasm a or surface-t reated len ses ten d to retard th e absorpt ion . Lipid Deposits Lipids are a com pon en t of th e tear film arising from th e m eibom ian glan ds to preven t tear evaporat ion . W h en lipids accum u late on th e len s surface th ey h ave a sm eared, greasy, w h it ish appearan ce. In dividu als w ith dietar y or m etabolic deficien cy in potassiu m , h ave dr y eye, use diu ret ics or alcoh ol, h ave poor tear osm olarit y, or h ave h igh er-fat diet ten d to be m ore pron e to th ese form s of deposits. Lens Ca lculi or Jelly Bumps Com bin at ion deposits of lipid an d protein s accu m ulate as m ore discrete, rou n d bum ps an d localized “droplets” th at bore in to th e len s m at rix. As a com bin ed deposit , variou s m in erals such as calcium from th e tear film m ay also be par t of th e com posit ion . Th ese t ypes of deposits are m ost com m on in ion ic, h igh -w ater-con ten t len ses u sed in a con t in uous or exten ded-w ear m odalit y. Mucin Ba lls Most syn onym ou s w ith silicon e hydrogel len ses, th ese are recogn ized as sm all, roun d, discrete par t icles or plugs seen bet w een th e con tact len s an d corn eal su rface. Th ey are com posed of a com bin at ion of m u cin , tear p rotein s, an d lipids. Th e appearan ce w ill var y in size from 10 to 20 µm in diam eter an d is t ypically t ran sparen t , sim ilar to th e appearan ce of m icrocyst s. Th ey are differen t iated from m icrocysts by th eir im pregn at ion of th e len s surface, versu s m icrocyst s, w h ich are in t raepith elial. Mu cin balls m ay accu m ulate an d clu m p ; th u s th ey m ay n ot m ove as th e len s m oves, appearing t rapp ed again st th e corn eal su rface. Upon len s rem oval or su bsequ en t blin king, th ey w ill dislodge, leaving an in den tat ion in th e corn eal su rface th at also appears sim ilar to discrete pu n ctate st ain ing associated w ith back surface debris. Exogenous Deposits Th ese t ypes of deposits can be from foreign bodies su ch as ru st , cosm et ics, pain t , lot ion s an d cream s, an d oth er form s of airborn e debris. Th ese deposit s w ill be discrete an d obvious upon obser vat ion . Rust or m et allic foreign bodies appear rou n d, sm all, an d brow n -red th at h ave becom e em bedded in th e len s su rface. Cosm et ics ten d to spread across th e len s surface in a vor tex m an n er, w h ereas pain t (an d som e oil-based cosm et ics) ten d to variably “spot” th e surface. In gen eral, exogen ou s debris is u su ally airborn e or physically im paled on to th e len s surface an d w ill quickly bin d w ith ou t pat ien t sym ptom s.

16 Contact Lenses

485

Medica tions Medicat ion s can also create deposits or len s discolorat ion . Th is is due to th e w ater solubilit y of hydroph ilic soft len ses th at allow for th e ease of absorpt ion by th e byprodu cts th at circu late in to th e tear film . An t ibiot ics su ch as ph en azopyridin e or n it rofu ran toin (u rin ar y in fect ion ) w ill discolor len ses to a yellow or pin k. Ph enyleph rin e, propin e (prodrug), an d ep in eph rin e w ill appear as brow n -black deposit s on th e len s su rface (aden och rom e st ain ing), w h ich can also be foun d on th e con jun ct iva an d lid m argin . Tet racyclin e, used for derm atological con cern s or upp er resp irator y in fect ion , m ay be obser ved as grayish brow n dots. Rifam p in u sed in th e t reat m en t of t ubercu losis–m en ingococcal in fect ion s cau ses orange-pin k tear excret ion th at absorbs in to th e len s. Iodin e an d sulfa-based drugs such as sulfasalazin e (t reat m en t for u lcerat ive colit is an d Croh n disease) as w ell as stool soften ers su ch as ph en olp h th alein w ill yield a yellow discolorat ion of th e len s. Th ese are easily defin ed by m edical h istor y. Any supp ression of th e lacrim al or exocrin e system w ill allow for in creased len s deposit ing.

Management Th e t reat m en t is sim ple bu t th e h ardest th ing for m any pat ien ts to com plete. It is called proper len s care an d hygien e. Th e sim plificat ion of care system s is a dou bleedged sw ord. On th e posit ive side, it is easier, less cum bersom e, an d m ore user frien dly w ith good effect iven ess in m icrobial ch allenge m ult i-item test ing. On th e n egat ive, sim p licit y is follow ed by com placen cy an d w orsen ed by th e com m odit izat ion of con t acts th rough th ird-par t y ven dors. Th e gen eral pu blic does n ot look at con tacts as a m edical device th at requires proper care un t il som eth ing goes w rong. Th e best t reat m en t for deposits is good educat ion an d in st ru ct ion s on th e proper use of th e recom m en ded an d p rescribed len s care product . In recen t st u dies, peroxide, act ing as a solven t clean ser an d h igh efficaciou s an t im icrobial solut ion , h as been sh ow n to be th e m ost efficien t for clean sing an d m icrobial reduct ion . Mult ipurpose solu t ion s, w h ich at on e t im e recom m en ded n o rub, h ave sin ce gon e back to “ru b.” Ru bbing can redu ce th e bacterial load by 90% an d pu rges th e len s su rface of th e protein –lipid accum ulat ion .

17 Ophthalmic Instruments and Diagnostic Tests Sam uel Boyd and Am ar Agarw al

Cover Testing Cover/ Uncover Tests Cover Test Th e cover test is don e to con firm th e presen ce of a m an ifest squ in t . Th e pat ien t is asked to look at th e fixat ion target (a flash ligh t sh ou ld n ever be u sed as a fixat ion target because it fails to con t rol accom m odat ion —an accom m odat ive fixat ion t arget h eld at 33 cm is used for n ear an d th e Sn ellen 6/9 visu al acu it y sym bol is u sed for dist an ce fixat ion ). Th e apparen tly fixat ing eye is th en covered an d th e beh avior of th e un covered eye is n oted. Each eye is tested in t u rn for n ear (33 cm ) an d distan ce (6 m ). It is perform ed in all n in e posit ion s of gaze. Head post u re sh ould be st raigh t . Th e test is perform ed w ith an d w ith out eyeglasses. In th e presen ce of squ in t , th e un covered eye m oves to take up fixat ion . If th ere is n o m ovem en t of th e un covered eye, th at eye is th en covered an d th e oth er eye obser ved. No m ovem en t of eith er eye is seen in p seu dosqu in t .

Uncover Test Cover th e apparen tly fixing eye an d obser ve th e oth er eye (apparen tly deviat ing eye). If m ovem en t is seen , th en th ere is h eterotopia.

Cover/ Uncover Test Th is test tells abou t th e presen ce an d t ype of h eteroph oria. Th e pat ien t looks at th e fixat ion target an d on e eye is covered w ith an occlu der. Th e occluder is th en rem oved, an d th e eye u n der cover is obser ved. Th e fin dings var y depen ding on th e diagn osis: In a person w ith n orm al vision , covering eith er eye w ill n ot produ ce any m ovem en t of th e oth er eye. On rem oving th e occluder, th ere is n o m ovem en t of th e un covered eye, w h ich con t in u es to look st raigh t ah ead. In h eteroph oria, th e eye u n der cover w ill deviate in th e direct ion of th e h eteroph oric posit ion . On u n covering, it w ill m ove in th e opposite direct ion to reestablish bin ocular fixat ion . Th e opposite eye con t in u es to m ain tain fixat ion an d m akes n o m ovem en t . Th us on ly on e eye m oves in case of a h eteroph oria. In h eterot rop ia, on covering th e fixat ing eye, th e op posite eye, p rovid ed it is able to do so, w ill m ake a m ovem en t from th e h eterot rop ic p osit ion to t ake u p fixat ion , an d th e covered eye w ill m ake a corresp on ding m ovem en t in accordan ce w ith t h e Hering law. On u n cover ing th e for m erly fixat ing eye, it w ill eith er m ove again to t ake u p fixat ion or m ay con t in u e to rem ain d eviated de-

486

17 Ophthalm ic Instrum ents and Diagnostic Test s

487

pen d ing on w h et h er it is a u n ilateral or an altern ate h eterot rop ia. On e can also m ake ou t th e fixat ion p at tern , th at is, w h eth er th ere is st rong fixat ion p referen ce for on e eye, free alter n at ion (form erly d eviated eye con t in u es to m ain t ain fixat ion in defin itely), w eak altern at ion (form erly deviated eye m ain t ain s fixat ion for som e t im e, su ch as u n t il a blin k), or eccen t r ic fixat ion (on covering th e fixat ing eye, th e deviated eye m akes n o m ovem en t or an in com plete m ovem en t) is p resen t .

Prism Bar Cover Test Th is is an object ive m eth od of m easu ring th e deviat ion s. Apply th e follow ing rule: th e apex of th e prism sh ou ld poin t tow ard th e deviat ion : Esodeviat ions: Place th e p rism base out . Exodeviat ions: Place th e prism base in . Right hypert ropia : Place th e prism base dow n in fron t of th e righ t eye. Right hypot ropia : Place th e prism base up in fron t of th e righ t eye. Com binat ion of vert ical and horizontal deviat ion : Place h orizon tal prism s in fron t of on e eye an d ver t ical prism s in fron t of th e oth er eye.

Alternate Cover Test In th is test , th e pat ien t looks at th e fixat ion t arget w ith both eyes open , an d th e occluder is altern ately m oved bet w een th e t w o eyes to produ ce m axim al dissociat ion of th e t w o eyes. Th is preven t s fu sion bet w een th e t w o eyes an d decom pen sates any laten t squin t . Th e pat ien t sh ould n ot be allow ed to regain fu sion w h ile th e cover is being t ran sferred. It can be u sed to diagn ose a laten t squ in t of even 2 degrees an d sm all degrees of h eterot ropia. It also differen t iates con com it an t squ in t from paralyt ic squ in t .

Rod Tests Maddox Rod Test Th e pat ien t is asked to fix on a poin t ligh t in th e cen ter of th e Maddox t angen t scale at a dist an ce of 6 m . A red Maddox rod (w h ich con sists of m any glass rods of red color set togeth er in a m et allic disk) is placed in fron t of on e eye w ith th e axis of th e rod at a righ t angle to th e axis of deviat ion . Th e Maddox rod convert s th e poin t ligh t im age in to a lin e. Th us th e pat ien t w ill see a poin t ligh t w ith on e eye an d a red lin e w ith th e oth er. Due to dissim ilar im ages of th e t w o eyes, fusion is broken an d h eteroph oria becom es m an ifest . Th e n um ber on th e Maddox t angen t scale w h ere th e red lin e falls w ill be th e am oun t of h eteroph oria in degrees (Fig. 17.1 ).

488

Color Atlas of Ophthalm ology

Fig. 17.1

Maddox rod test.

Double Maddox Rod Test Th is test h elps in detect ing an d m easu ring cyclodeviat ion s. Place a red Maddox rod ver t ically in fron t of th e pat ien t’s righ t eye an d a w h ite Maddox rod also ver t ically in fron t of th e oth er eye in a t rial fram e. Th e axes of th e Maddox rod(s) are rotated un t il th e t w o lin es seen by th e pat ien t are parallel. Th e degrees of cyclodeviat ion an d direct ion are m easu red from th e t rial fram e w ith excyclodeviat ion h aving outw ard rot at ion an d in cyclodeviat ion s h aving inw ard rot at ion s.

Maddox Wing Test Th e Maddox w ing is an in st rum en t by w h ich th e am oun t of h eteroph oria for n ear (at a dist an ce of 33 cm ) can be m easu red. It is based on th e p rin ciple of dissociat ion of fusion by dissim ilar objects. It h as t w o slit h oles in th e eyepiece. Th e fields th at are exposed to each eye are separated by a diaph ragm in su ch a w ay th at th ey glide tangen t ially in to each oth er. Th e righ t eye sees a w h ite arrow poin t ing ver t ically upw ard an d a red arrow poin t ing h orizon t ally to th e left . Th e left eye sees a h orizon tal row of figures in w h ite an d a vert ical row in red. Th ese are calibrated in diopters of deviat ion . Th e arrow p oin t ing to th e h orizon tal row of figures an d th e arrow poin t ing to th e vert ical row are both at zero in th e absen ce of a squin t or in th e presen ce of squin t w ith a h arm on iou s abn orm al ret in al correspon den ce. Clin ically im por t an t poin ts are as follow s: Th e Maddox w ing sh ould be h eld poin t ing 15 degrees in feriorly, as for reading. It is im p or tan t to do th e test w ith an d w ith out correct ion for refract ive errors. Th e abilit y of th e pat ien t to give an an sw er on th e Maddox w ing does n ot m ean th e pat ien t h as n orm al bin ocu lar vision becau se th e pat ien t can h ave abn orm al ret in al correspon den ce (ARC) or rapid alterat ion .

17 Ophthalm ic Instrum ents and Diagnostic Test s

489

Stereopsis and Fusional Testing Stereopsis Stereopsis is th e visual appreciat ion of th ree dim en sion s during bin ocular vision . It is a fu n ct ion of spat ial disparit y an d arises w h en h orizon tally disparate ret in al elem en t s are st im u lated sim ultan eou sly. Th e fu sion of th ese disparate ret in al im ages w ill result in a single visu al im pression perceived in depth , provided th e fu sed im age lies w ith in th e Pan u m area of bin ocular single vision .

Tests for Stereopsis Titmus Test Th e t it m us test con sists of a th ree-dim en sion al Polaroid vectograph —t w o plates in th e form of a booklet , w h ich h as to be view ed th rough Polaroid spect acles. On th e righ t th ere is a large fly an d on th e left a series of circles an d an im als. Th e w orking distan ce is 16 in ch es (40 cm ). Fly Test Th e fly test is for gross stereop sis (degree of disparit y is 3000 secon ds of arc). Th e fly sh ou ld appear solid an d th e su bject sh ould be able to p ick up on e of th e w ings of th e fly. On invert ing th e book, th e t argets w ill appear to recede. If th e fly appears as a flat ph otograph , th e subject is n ot appreciat ing stereoscopic vision . Circles Test Th e circles test m easu res fin e stereopsis (degree of disparit y is 800 to 40 secon ds of arc). Th ere are n in e squ ares, each of w h ich con t ain s four circles. On e of th e circles in each squ are w ill appear for w ard of th e plan e of referen ce in th e presen ce of n orm al stereopsis. Th e subject th at perceives th e circle to be sh ifted off to th e side is n ot appreciat ing stereoscopic vision but is using m on ocu lar clues in stead. TNO Test (degree of dispa rit y is 480 to 15 seconds of a rc) Th e TNO test con sist s of seven plates to be view ed w ith red-green spectacles. Each plate h as variou s sh apes created by ran dom dot s in com plem en t ar y colors. Som e sh apes are visible w ith out glasses, w h ereas oth ers can be appreciated in th e presen ce of stereopsis on ly. It h as n o m on ocular clu es. La ng Test (degree of dispa rit y is 1200 to 600 seconds of a rc) Th e t arget s are seen altern ately by each eye th rough th e built-in cylin drical len s system ; h en ce th ere is n o n eed for special spect acles. Frisby Test (degree of disparit y is 600 to 15 seconds of a rc) Th ere are th ree t ran sparen t p lates of var ying th ickn ess. On th e su rface of each plate th ere are prin ted four squ ares of sm all ran dom sh apes. On e of th e squ ares con tain s a h idden circle in w h ich th e ran dom sh apes are prin ted on th e reverse of th e plate. Th e su bject m ust iden t ify th is h idden circle.

Base -Out Prism Th is is a quick an d sim ple m eth od using a 20 diopter base-ou t prism to detect bin ocular single vision (BSV).

490

Color Atlas of Ophthalm ology

Synoptophore All t ypes of h eteroph orias an d h eterot rop ias can be m easured accu rately w ith a syn optop h ore (both object ive an d subject ive angle of squ in t). In th e syn optoph ore th e rays of ligh t from th e target h it a m irror an d th en p ass th rough a convex len s of + 6.5 diopters to reach th e eye. Th us th e im age is seen beh in d th e m irror, for exam ple, at a dist an ce of 6 m , w h ich w ill be equal to th e focal length of th e len s. Th u s th e syn optoph ore im ages are seen at a dist an ce an d n ot n ear. Th is is becau se w e do n ot w an t th e pat ien t to use h is or h er accom m odat ion . Th e tech n ique for u sing th e syn optop h ore is as follow s ( Fig. 17.2 ): In terpupillar y distan ce (IPD) is ch ecked an d adjusted. Angle kappa is m easu red. Sim ult an eou s m acular percept ion is tested for. We u se th e sim u ltan eou s param acular percept ion slides. Both th e object ive an d su bject ive angles of squin t are ch ecked in all n in e cardin al p osit ion s of gaze (on e is th e prim ar y posit ion an d th e oth er eigh t are 15 degrees from th e prim ar y posit ion ). To test th e object ive angle, on e arm of th e syn optoph ore is fixed at zero degrees. Th e oth er arm is m oved un t il th ere is n o m ovem en t of th e eyes w h en th e tester altern ately sw itch es on an d off th e ligh t s of th e t w o arm s. Th e poin t w h ere th e eyes do n ot m ove is th e object ive angle. To test th e su bject ive angle, on e arm of th e syn optoph ore is fixed at zero degrees. Th e pat ien t is sh ow n slides of a lion an d it s cage. Th e slide of th e cage is kept in th e arm th at is fixed. Th e p at ien t is asked to m ove th e oth er arm (con t ain ing th e slide of th e lion ) so as to put th e lion in th e cage. Th e angle at w h ich th is is don e is th e su bject ive angle of squin t .

Fig. 17.2

Synaptophore

17 Ophthalm ic Instrum ents and Diagnostic Test s

491

To detect abn orm al ret in al correspon den ce (ARC) w ith th e syn optop h ore, th e object ive angle (OA) an d th e su bject ive angle (SA) of squ in t are first determ in ed, w hich gives th e angle of an om aly (AOA). AOA = OA – SA In th e case of n orm al ret in al correspon den ce (NRC), th e SA is equ al to th e OA, an d th e AOA w ill be zero. In un h arm on iou s ARC, th e su bject ive angle w ill be less th an th e object ive angle, an d th e differen ce bet w een th e t w o sh ou ld be at least 5 degrees or m ore. In th e case of h arm on ious ARC, th e su bject ive angle w ill be zero. Th us th e angle of an om aly in a h arm on iou s ARC w ill be equ al to th e object ive angle. Fusion : For test ing fu sion , t w o slides are used: on e is th at of a rabbit w ith out a tail an d th e oth er is th at of a rabbit w ith ou t ears. Th e t w o slides are kept in each arm of th e syn optoph ore an d th e arm s are fixed at th e angle of squ in t . If th e pat ien t sees both th e ears an d th e tail, th en fu sion is presen t . If th e pat ien t sees eith er th e tail or th e ears, fusion is absen t . Stereopsis: Stereop sis can be tested w ith slides con tain ing parat roopers w ith a plan e in th e backgrou n d. Th e pat ien t sh ou ld be able to tell w h eth er th e parat roopers are in fron t of th e plan e or n ot , w h ich in dicates good stereopsis. After im ages: After im ages can also be don e.

Visual-Field Testing Th e visu al field is th e por t ion of sp ace th at is visible to th e fixat ion eye. Visual-field exam in at ion is th e exam in at ion of th e fun ct ion of th e visu al system in th e field an d n ot on ly th e determ in at ion of th e lim its of th e field. Th e differen ce th resh old is th e sm allest m easu rable differen ce in lum in an ce bet w een a st im ulu s an d th e backgroun d ( Fig. 17.3 ).

Automated Static Perimetry Th e differen t test s in autoperim et r y are as follow s: Suprathreshold test : Th is test is u sed as a screen ing device for severe or m oderate defect s. Threshold related st rategy : Th e act ual th resh old is determ in ed at a sm all n u m ber of p oin t s an d th ey are u sed to ext rapolate th e h ill of vision . Threshold test ing: Th resh old test ing is th e stan dard of care for glau com a m an agem en t . Many p oin t s are tested an d th ere are differen t st rategies used to accurately defin e th e visu al field. Th ese tests can be long an d th e pat ien t s can becom e fat igu ed. SITA: Becau se fu ll th resh old can be t im e con su m ing, sh or ter algorith m s h ave been developed. Th e Sw edish In teract ive Th resh olding Algorith m (SITA) uses m ath em at ical m odeling an d presen t un derstan ding of th e visu al field to in crease accu racy w h ile speeding up th e test . Short-w avelength autom ated perim etry (SW AP) : Th is select ively tests th e sh or tw avelength path w ay by presen t ing a blue t arget on a yellow backgroun d. SWAP m ay be able to sh ow defect s 1 to 3 years before st an dard tech n iques.

492

Color Atlas of Ophthalm ology

Fig. 17.3

Humphrey autoperimetric 30–2 test.

Th e various field defects seen in glaucom a are gen eralized depression , baring of th e blin d spot , isolated p aracen t ral scotom a, Seidel scotom a, Bjerru m scotom a or arcuate scotom a, dou ble arcuate scotom a, Ron n e n asal step (w h ich respects th e h orizon tal m idlin e), tem poral w edge defect , p eriph eral breakth rough , alt it udin al defect , cen t ral an d tem poral islan ds, an d split fixat ion .

17 Ophthalm ic Instrum ents and Diagnostic Test s

493

Automated Corneal Topography Im aging tech n iqu es of th e corn ea are developing rapidly, m ain ly because of con t in uou s advan ces in refract ive an d cat aract su rger y. It is crucial to u n derst an d th e sign ifican ce of n ew im aging tech n iques an d th e relevan t prin ciples of corn eal opt ics. Th e discu ssion of th e m ost com m on clin ical m eth od of Placido-based corn eal topography em ph asizes im por tan t con cept s of it s clin ical in terpret at ion (Fig. 17.4 ).

A

B Fig. 17.4 (A) Astigmatic corneal topography. (B) Orbscan topography of an eye with keratoconus.

494

Color Atlas of Ophthalm ology

Optical Properties of the Cornea Several con cepts are u sed to ch aracterize opt ical proper t ies of th e corn ea, such as cur vat ure, sh ape, local su rface, p ow er, expressed as refract ion in diopters, th ickn ess, an d th ree-dim en sion al st ru ct u re. Th e keratom et ric value is a con cept in h erited from keratom et r y an d is calculated sim ply from radii of cu r vat u re as follow s: K = refract ive in dex of 337.5/radiu s of cur vat u re Th e in tact cen t ral corn eal th ickn ess of ~560 µm is con sidered en ough to en su re long-term m ech an ical stabilit y of th e corn ea. Th e periph eral th ickn ess ( ~600 µm ) is cer t ain ly clin ically im por tan t in som e refract ive procedures su ch as in t racorn eal rings, ast igm at ic keratotom y, an d cat aract surger y. W ith th e advan ces in corn eal im aging an d w idesp read refract ive su rgeries, corn eal beh avior w ill likely be bet ter un derstood. Corn eal topography in st ru m en ts u sed in clin ical pract ice m ost often are based on Placido reflect ive im age an alysis. Th is m eth od of im aging of th e an terior corn eal surface uses th e an alysis of reflected im ages of m u lt iple con cen t ric rings projected on th e corn ea.

Interpretation of Topographic Maps Ever y m ap h as a color scale th at assign s par t icu lar color to a cer tain keratom et ric diopt ric range. Never base an in terpret at ion on color alon e. Th e value in keratom et ric diopter is crucial in th e clin ical in terpret at ion of th e m ap an d h as to be looked at w ith th e in terpret at ion of ever y m ap. Absolute m aps h ave a preset color scale w ith th e sam e diopt ric step s an d diopt ric m in im um an d m axim u m assign ed to th e sam e colors for par t icu lar in st ru m en t . Norm alized m aps h ave differen t color scales assign ed to each m ap based on in st ru m en t soft w are th at iden t ifies th e act u al m in im al an d m axim al keratom et ric diopt ric valu e of a par t icu lar corn ea. Th e diopt ric range assign ed to each color is gen erally sm aller com pared w ith th e absolu te m ap, an d, con sequ en tly, m aps sh ow m ore det ailed descript ion of th e su rface. Th e disadvan tage is th at th e colors of t w o differen t m aps can n ot be com pared directly an d h ave to be in terpreted based on th e keratom et ric valu es of th eir differen t color scales.

Specular Microscopy Th is is an im port an t par t of th e preoperat ive evaluat ion , especially if corn eal guttata or oth er sign s of a low en doth elial cell coun t are fou n d. It is clin ically an d m edical-legally im por t an t to docu m en t a low en doth elial cell coun t before LASIK, rath er th an w orr y later w h eth er LASIK caused it . It m u st be don e before any exam in at ion th at m igh t rough en or dr y th e corn eal su rface. Alth ough en doth elial cell ch anges follow ing lam ellar surger y (in cluding LASIK) h ave n ot been reported clin ically, th ey h ave been seen experim en t ally w h en excim er ablat ion of greater th an 90% depth w as ach ieved. Th is depth is of cou rse en t irely con t rain dicated in pat ien t s, bu t t w o cases of corn eal perforat ion du ring LASIK h ave recen tly been reported, an d on e w ou ld assu m e th at oth er cases w ith deep ablat ion m ust also exist ( Fig. 17.5 ).

17 Ophthalm ic Instrum ents and Diagnostic Test s

Fig. 17.5

495

A normal specular microscopy.

Corneal Pachymetry St u dy of th e cen t ral corn ea w ith ult rasoun d pachym et r y is fu n dam en tal. Th e surface of th e u lt rason ic pachym eter p robe is w iped w ith alcoh ol an d does n ot n eed to be sterilized. Th is in st ru m en t is used to take a ver y carefu l an d accurate reading of th e th ickn ess of th e cen t ral corn ea. Th e pachym eter probe is placed on th e cen ter of th e corn ea, perpen dicu larly, to determ in e corn eal th ickn ess. Th e pachym eter h as a con sole displaying th e corn eal th ickn ess reading ( Fig. 17.6 ).

Clinical Importance Ideally, w e m ust preser ve a m in im um of 250 µm in th e posterior st rom al bed. Oth er invest igators con sider th at preser vat ion of a m in im u m of 50% of th e preoperat ive cen t ral pachym et r y is essen t ial. Th is is referred to as th e Barraquer law of corneal thickness. Th is is par t icu larly im por t an t in th e t reat m en t of h igh refract ive errors w ith th e excim er laser an d in “en h an cem en t” procedures. Un less sufficien t corn eal st rom a rem ain s, as determ in ed by Barraqu er’s basic prin ciple, th ere is an in creased risk of developing corn eal ect asia. It is gen erally est im ated th at 10 µm of ablat ion corrects on e diopter of m yopia.

496

Color Atlas of Ophthalm ology

Fig. 17.6 Illustration of a corneal pachymetry in a patient undergoing LASIK. P, ultrasound pachymeter; A, flap depth in case of LASIK; B, amount of strom a ablated for correction of refractive error; C, residual bed thickness. (From Boyd, BF. Pre operative evaluation and considerations. In: Atlas of Refractive Surgery. Highlights of Ophthalm ology, 2000, 45. Courtesy Jaypee Highlights Medical Publishers Inc., Panama.)

A-Scan Ultrasonography Ocu lar biom et r y m u st be perform ed prior to cat aract su rger y. Biom et r y is th e discip lin e in ch arge of th e physical param eters of th e eyeball an d in clu des t w o fu n dam en tal explorat ion s, keratom et r y an d th e a xial length m easurem en t of th e eye. Usu ally, in clin ical pract ice, th e term biom et ry refers to th e lat ter an d con siders keratom et r y to be a separate procedure. Th ere is n o qu est ion th at w h en w ell selected an d properly don e th e m odern m eth ods of part ial coh eren ce in terferom et r y (opt ical coh eren ce biom et r y), th e pen t acam (Ocu lu s, In c., Lyn nw ood, WA) an d th e advan ce con t act an d im m ersion ult rason ography afford u s th e best w ay of ach ieving th e desired postoperat ive refract ion . Determ in at ion of in t raocular len s pow er th rough m ean ingful keratom etric readings an d a xial length m easu rem en ts h as becom e th e stan dard of care. Th is is a ch allenging tech n iqu e to obt ain good visual resu lts an d pat ien t sat isfact ion ( Fig. 17.7 ).

17 Ophthalm ic Instrum ents and Diagnostic Test s

497

Fig. 17.7 Determination of intraocular lens power in patients with normal axial length (normal eyes)—mechanism of how ultrasonography measures distances and determines axial length. The use of ultrasonography to calculate the intraocular lens power takes into account the variants that may occur in the axial diameter of the eye and the curvature of the cornea. The ultrasound probe (P) has a piezoelectric crystal that electrically emits and receives high-frequency sound waves. The sound waves travel through the eye until they are reflected back by any structure that stands perpendicularly in their way (represented by arrows). These arrows show how the sound waves travel through the ocular globe and return to contact the probe tip. Knowing the speed of the sound waves and based on the time it takes for the sound waves to travel back to the probe (arrows), the distance can be calculated. The speed of the ultrasound waves (arrows) is higher through a dense lens (C) than through a clear one. Soft-tipped transductors (P) are recomm ended to avoid errors when touching the corneal surface (S). The ultrasonography equipment computer can automatically multiply the time by the velocit y of sound to obtain the axial length. Calculations of intraocular lens power are based on program s such as SRK-II, SRK-T, Holladay, or Binkhorst, am ong others, installed in the computer. (From Boyd, BF. IOLpower calculation in normal cases. In: The art and science of cataract surgery. Highlights of Ophthalmology, 2001, 41. Courtesy Jaypee Highlights Medical Publishers Inc., Panam a.)

498

Color Atlas of Ophthalm ology

Fig. 17.8 Mode B-scan showing retinochoroidal colobom a.

B-Scan Ultrasound Lin ear A scan s are sum m ated in th e B-scan . Th is gives a t w o-dim en sion al crosssect ion al im age of th e eye an d orbit . It is of great valu e in evaluat ing th e posterior segm en t in eyes w ith opaque m edia (Fig. 17.8 ).

Potential Acuity Meter Testing Th e Poten t ial Acu it y Meter (PAM; Marco Oph th alm ic In c., Jacksonville, FL) is an in st r um en t th at at tach es to a slit lam p. It ser ves as a vir t ual pin h ole by project ing a regu lar Sn ellen visual acuit y ch ar t th rough a ver y t iny aerial p in h ole aper t u re ~0.1 m m in diam eter. Th e ligh t carr ying th e im age of th e visu al acu it y ch art n arrow s to a fin e 0.1-m m beam an d is directed th rough clearer areas in cataracts (or corn eal disease), allow ing th e pat ien t to read th e visual acu it y ch ar t as if th e cat aract or corn eal disease w ere n ot th ere. Th e PAM is taken from it s st an d an d placed directly on to th e slit lam p in th e sam e m an n er as th e detach able t ype of Goldm an n ton om eter. Th e exam in at ion takes from 2 to 5 m in utes per eye, depen ding on th e den sit y of th e cat aract ( Fig. 17.9 ). As poin ted ou t by Guyton , for th e PAM to w ork adequ ately, th ere m ust be som e sm all h ole in th e cat aract for th e ligh t beam to pass th rough . You m ay fin d su ch a h ole even in cat aract s th at h ave m edia clou ding of up to 20/200 an d bet ter. W h en you fin d it , th en you can avoid th e ligh t scat tering produ ced by th e op acit ies. It is th is ligh t scat tering th at w ash es ou t th e ret in al im age an d decreases vision beh in d cataracts. By project ing th e im age of th e visual acuit y ch art th rough on e t iny area, w e avoid th e scat tering effect , an d th e pat ien t can see th e ch art .

17 Ophthalm ic Instrum ents and Diagnostic Test s

499

Fig. 17.9 Potential Acuit y Meter Guyton Test. C, cornea; R, retina. (From Boyd, BF. Preoperative evaluations. In: The art and science of cataract surgery. Highlights of Ophthalmology, 2001, 16. Courtesy Jaypee Highlights Medical Publishers Inc., Panama.)

Color Vision Tests (Ishihara Plates, Depth Perception Tests) People w ith n orm al con es an d ligh t sen sit ive pigm en t (t rich rom asy) are able to see all th e differen t colors an d su btle m ixt ures of th em by u sing con es sen sit ive to on e of th ree w avelength s of ligh t—red, green , an d blu e. A m ild color deficien cy is presen t w h en on e or m ore of th e th ree con es’ ligh t sen sit ive pigm en t s are n ot qu ite righ t an d th eir peak sen sit ivit y is sh ifted (an om alou s t rich rom asy—in clu des protan om aly an d deu teran om aly). A m ore severe color deficien cy is presen t w h en on e or m ore of th e con es’ ligh t sen sit ive p igm en ts is really w rong (dich rom asy—in cludes prot an opia an d deuteran opia). Th e Ish ih ara test , for exam ple, is com posed of a series of colored cards on w h ich n um bers or lin es of equal sh ade can be read by a person w ith n orm al color vision but n ot by som eon e w ith defect ive color vision . It m akes use of th e peculiarit y th at in red-green blin dn ess, blu e an d yellow appear rem arkably brigh t com pared w ith red an d green . Th e color p lates are en cased in a specially design ed album -t ype book for ease of h an dling. Th e set in clu des fou r special plates for test s to determ in e th e kin d an d degree of defect in color blin dn ess. Th is color blin dn ess test is accepted by leading au th orit ies ( Fig. 17.10 ).

Fig. 17.10

Ishihara plate.

500

Color Atlas of Ophthalm ology

Fluorescein Angiography Flu orescein angiography can be a ver y u sefu l procedure for assessing ret in al disease by delin eat ing areas of involvem en t , gu iding t reat m en t , an d form u lat ing a progn osis for ch anges in th e pat ien t’s vision . To in terpret fluorescein angiograph ic im ages, kn ow ledge of ret in al/ch oroidal an atom y an d circulat ion is essen t ial. Arterial an d ven ou s circu lat ion differen ces, as w ell as th e ret in a’s barriers again st th e passage of sodium fluorescein (NaFl) dye, in cluding th e ret in al pigm en t epith eliu m (RPE) (ou ter blood–ret in al barrier) an d th e ret in al vascu lar en doth elium (in n er blood–ret in al barrier), m ust be un derstood. Kn ow ledge of fu n dus path ophysiology an d an atom y h as been greatly en h an ced by research using flu orescein angiograp hy ( Fig. 17.11 ). For purposes of angiogram in terpretat ion , th e sen sor y ret in a can be divided in to vascu lar an d avascu lar por t ion s. Th e vascu lar port ion is com posed of th e in tern al lim it ing m em bran e (ILM), n er ve fiber layer (NFL), ganglion cell layer (GC), in n er plexiform layer (IPL), an d in n er n uclear layer (INL). Th ese por t ion s of th e ret in a receive direct m etabolic support from ret in al blood vessels.

A

B Fig. 17.11 (A) Fundus photo of neovascularization. (B) Fluorescein angiography of neovascularization.

17 Ophthalm ic Instrum ents and Diagnostic Test s

501

The sensory retina’s avascular portion consists of the outer plexiform layer (OPL), the outer nuclear layer (ONL), and rod and cone photoreceptor cells. These structures receive m etabolic support from the choroidal vessels via the pigm ent epithelial cells.

Optical Coherence Tomography Optical coherence tom ography (OCT) is an im aging m odalit y that perform s high-resolution, m icrom eter-scale cross sections of the eye and other biological structures. At th is t im e th e tech n ology is an alogou s to B-scan ult rasoun d. Its ut ilit y to th e clin ician lies in th e abilit y to accurately m ake n on invasive an atom ical m easurem en ts in vivo ( Fig. 17.12 ). Prior to th e developm en t of OCT, th e m ain stays of clin ical exam in at ion for m any ret in al diseases w ere slit-lam p biom icroscopy, fun du s ph otography, fluorescein angiography, an d in docyan in e green angiography. Alth ough th ese diagn ost ic tech n iques rem ain essen t ial to diagn osis an d m an agem en t , OCT h as em erged as an invalu able diagn ost ic tool as w ell, providing quan t itat ive an d qualit at ive in form at ion th at w as previou sly un at t ain able. In addit ion to presen t ing a clearer pict ure of th e path ophysiology of disease, OCT h as been ext rem ely u seful in determ in ing respon se to th erapy. Mult ip le st u dies, for exam ple, h ave u sed OCT to exam in e ocu lar respon se follow ing m acu lar t reat m en ts determ in ing th at th ere is an in it ial in crease in su bret in al an d in t raret in al flu id, follow ed by a gradual declin e over th e n ext days.

Lasers In Ophthalmology Laser Peripheral Iridectomy Becau se of th e coagu lat ing effect of argon laser ligh t , iridectom y perform ed by argon laser offers advan t ages over in cision al iridectom y or n eodym ium :yt t rium alu m in u m -garn et (Nd:YAG) laser iridectom y in pat ien t s predisposed to bleeding con dit ion s, such as th ose taking an t icoagulan t s or w ith kn ow n blood-clot t ing disorders. Th e laser iridectom y is perform ed as an office p rocedu re in a closed eye—a con siderable advan t age over surgical iridectom y (Fig. 17.13 ).

Fig. 17.12

Optical coherence tomography of a macular hole.

502

Color Atlas of Ophthalm ology

Fig. 17.13 Iridectomy with argon laser—opening a narrow angle in chronic, narrow angle glaucoma. A laser iridectomy is the procedure of choice for narrow angle glaucoma except in cases such as (A) where the pe ripheral iris lies too close to the cornea for treatment. Laser applications (D) are placed in the midstroma area of the iris to open the angle. These nonperforative laser applications cause heat, which in turn causes shrinkage of the iris collagen fibres in the direction of the arrow. The iris sphincter muscle (S) and the laser beam (L) are shown in (B), shrinkage from laser applications (D) has opened the angle to an acceptable position (C). A peripheral laser iridectomy is then executed. The normal iris location is shown on dot ted line (N). The angle is now sufficiently open for laser trabeculoplast y if indicated. Laser beam (L) is shown producing burns (E) in the now visible trabeculum. (From Boyd, BF, Lunt z, M. Acute and chronic angle closure. In: Innovations in the glaucomas. Highlights of Ophthalmology, 2002, 277. Courtesy Jaypee Highlights Medical Publishers Inc., Panama.)

It is an effective w ay of producing an open ing in th e iris but should n ot be used w hile the eye is congested or in flam ed. Clear m edia are essent ial. Th e eye is prepared w ith topical an esth esia. The surgeon sh ould have com fortable arm supports. Th e Abrah am tech n ique is h igh ly u seful an d effect ive. Th ese bu rn s im m ediately cause iris con t ract ion an d put th e iris cr ypt on st retch . Oth er surgeon s fin d th at th e st retch burn is gen erally u n n ecessar y if th e Abrah am con tact len s is used. In th e m ajorit y of cases, an iridectom y is ach ieved at th e first session . As p en et rat ion of th e iris st rom a reach es th e pigm en ted epith elium of th e iris, bu rst s of p igm en t appear in th e an terior ch am ber (“sm oke sign als”). Pow er is th en reduced, an d fu r th er burn s are applied u n t il a m ush room clou d of aqueous an d pigm en t balloon s th rough th e iridectom y, in dicat ing pen et rat ion of th e iris.

Argon Laser Trabeculoplasty Th e Glaucom a Laser Trial, a m ajor prospect ive, ran dom ized st udy, con cluded th at laser t rabeculoplast y as an in it ial t reat m en t for op en -angle glaucom a is as safe an d as effect ive as m edical t reat m en t . In som e cases, it m ay be m ore appropriate as in it ial th erapy. Th ese cases in clude (1) pat ien ts w h o can n ot or w ill n ot com p ly

17 Ophthalm ic Instrum ents and Diagnostic Test s

503

w ith prescribed m edical th erapy, (2) areas of th e w orld w h ere adequ ate m edical t reat m en t is un feasible because of povert y. In all cases, to be su ccessful, th e angle does h ave to be open , th e m edia m u st be clear, an d on e m u st h ave access to th e t rabecular m esh w ork. Jam es B. Wise, M.D., w h o developed argon laser t rabecu loplast y (argon laser t rabecu loplast y), h as obser ved th at populat ion group s of ph akic pat ien ts do bet ter th an aph akic. It ap pears th at aph akia does in terfere w ith respon se to th e laser, probably by th e in fluen ce of vit reous in th e an terior ch am ber an d th e t rabecu lar m esh w ork. In terest ingly en ough , p seu doph akic pat ien t s resp on d to th e laser ver y sim ilarly th an ph akic pat ien t s. Th at is, th e presen ce of th e posterior ch am ber len s im plan t keeping th e vitreou s out of th e an terior ch am ber greatly im p roves th e respon se to th e laser. Eyes w ith an terior ch am ber len ses an d glaucom a u sually sh ow a p oor laser respon se du e to uveit is an d t rabecu lar dam age from th e len s ( Fig. 17.14 ).

Fig. 17.14 Applying laser burns correctly in argon laser trabeculoplast y. Cross section to the left; cornea (E), Schlemm canal (C), scleral spur (S), Schwalbe line (G), anterior corneoscleral meshwork (A), pigmented band (P), and uveal meshwork (U). Proper placement of the 50 µm laser burn (L) is shown at the posterior margin of the pigmented band (P). To the right is a gonioscopic view with iris (I) below. Properly placed 50 µm laser burn at the posterior pigment band (P) shown at (1). Another burn is shown at (2) along the posterior margin of the pigment band (P). An oversized burn is shown at (3), spanning the entire pigment band. A slightly misplaced burn is shown at (4) in the middle of the pigment band. A seriously misplaced burn into the uveal meshwork (5). (From Boyd, BF, Luntz, M. Argon laser trabeculoplast y. In: Innovations in the glaucomas. Highlights of Ophthalmology, 2002, 149. Courtesy Jaypee Highlights Medical Publishers Inc., Panama.)

504

Color Atlas of Ophthalm ology

Retinal Laser Photocoagulation Many sign ifican t applicat ion s h ave been discovered for clin ical use of th e laser in oph th alm ology. Th e un ique feat u res of th e eye allow ing in t raocular t ran sm ission an d select ive absorpt ion of ligh t en ergy accoun t for a w ide range of laser t reatm en ts. Essen t ially, t reat m en t m ay be categorized in to five gen eral effect s: (1) in du ct ion of ch orioret in al bu rn s or ret in al scar th at lead to (possible ph arm acological) n eut ralizat ion of isch em ia-in duced ret in al n eovascu larizat ion ; (2) redu ct ion in ret in al vascu lar perm eabilit y via direct vascular closu re or un kn ow n m ech an ism s; (3) ablat ion of un desired t issue su ch as ch oroidal n eovascu larizat ion , t u m ors, abn orm al n at ive vessels, or aqu eou s-produ cing t issu e; (4) in du ct ion of a ch orioret in al scar th at m ay ser ve as a barrier to exten sion of subret in al fluid; an d (5) lysis of t ract ion -in ducing or m edia-opacifying t issu es (Fig. 17.15 ). Th e tech n iques of laser ph otocoagu lat ion in clu de th e follow ing broad categories: scat ter t reat m en t , focal t reat m en t , ablat ive t reat m en t , dem arcat ing t reatm en t , an d cut t ing t reat m en t .

Focal Macular Treatment Macu lar edem a occurs from a variet y of disease m ech an ism s, bu t th e feat u re com m on to each is in creased ret in al vascular perm eabilit y. Con t ribut ing factors in clude ret in al isch em ia, in flam m at ion , an d t ract ion . Som e en t it ies in th e first of

Fig. 17.15 Argon laser treatm ent of a retinal tear (T). (From Cortez, R. Managem ent of retinal detachment. In: Retinal and vitreoretinal surgery. Highlights of Ophthalmology, 2002, 402. Courtesy Jaypee Highlights Medical Publishers Inc., Panama.)

17 Ophthalm ic Instrum ents and Diagnostic Test s

505

th ese categories are resp on sive to focal laser t reat m en t: clin ically sign ifican t diabet ic m acular edem a an d bran ch vein occlu sion -associated m acu lar edem a. Macu lar edem a du e to cen t ral vein occlusion m ay resolve after t reat m en t , but th is is n ot usually accom pan ied by a visu al ben efit .

Nd:YAG Laser Posterior Capsulotomy Posterior capsu le opacificat ion (PCO) is curren tly th e m ost frequ en t postoperat ive com plicat ion in cat aract su rger y. Th e u se of hypoton ic solu t ion an d preser vat ive free lidocain e are being invest igated in th e preven t ion of PCO. St udies w ith in vivo sp ecular m icroscopy suggest th at a hydrop h ilic acr ylic in t raocu lar len s w ou ld h ave h igh er biocom pat ibilit y th an th e hydroph obic acr ylic len s, con t rar y to som e p reviou s st udies, w h ich affirm ed th at hydroph obic acr ylic len s h ad h igh er biocom pat ibilit y. Opacificat ion of the posterior capsule is an in adequ ate term becau se it is n ot th e capsule th at opacifies bu t an opaque m em bran e th at grow s, origin at ing from th e epith elial cells th at w ere ret ain ed, w h ich proliferate an d m igrate on th e posterior capsule ( Fig. 17.16 ). Th e pearls of Elsch n ig origin ate from th e superim p osed epith elial cells of th e flexible w ing of th e an terior capsu le an d m igrate to th e posterior capsu le. Opacificat ion of th e posterior capsu le is t reated w ith Nd:YAG laser, w h ich is a ph otodisru ptor laser. In oth er w ords, it u ses a h igh p ulse of ion izing elect rom agn et ic en ergy to break th e t issue. To ach ieve th e open ing of th e capsu le, begin from th e periph er y at 12 o’clock an d go to 6 o’clock w ith a size larger th an th e pupil (un dilated). Make th e in cision going from 3 o’clock to 9 o’clock u sing th e Abrah am len s. Fin ally clean th e residues to avoid leaving float ing fragm en t s.

Fig. 17.16

Nd:YAG laser posterior capsulotomy.

506

Color Atlas of Ophthalm ology

Cryoprobes Cr yoprobes are used for perip h eral ret in opexy in pat ien ts w ith periph erally located ret in al breaks an d tears, in th e t reat m en t of ret in al t u m ors an d ret in al vascular m alform at ion s. Th ey h ave also been u sed for perform ing cyclocr yoth erapy in en d-st age glaucom as. Th e t ip of th e probe is placed over th e globe at th e edge of th e break w h ile visu alizing w ith th e in direct oph th alm oscope. Th e freezing is th en applied un t il th ere is w h iten ing of th e ret in a (freezing of th e vit reous sh ou ld be avoided). On ce th e t ip h as th aw ed, it is rem oved an d th e n ext ap plicat ion is m ade ( Fig. 17.17 ).

Fig. 17.17

Cryoprobe.

Index Note: Page n u m bers follow ed by f an d t in dicate figu res an d t ables, respect ively. A Ablepharon, differen tial diagn osis, 51 Abscess differen t ial d iagn osis, 47 orbital, 72f, 95f su bp eriosteal, 71–73 presen tat ion , 71 Acantham oeba kerat itis, 147–149, 148f differen t ial d iagn osis, 146, 149, 192 Accom m odat ive esot rop ia, 341–342, 341f h igh , 341f, 342 part ially, 342 Acetam ide, for cen t ral ret in al arter y occlu sion , 268 Acet ic acid bu rn , 5–7 Acid bu rn s, 5–7 Acn e rosacea, 100–101 differen t ial d iagn osis, 192 ep isclerit is in , 123 Acroch ord on , 52 Act in ic keratosis, 52–53, 53f differen t ial d iagn osis, 52, 54, 62 Act inom yces can alicu lit is, 65 Acu te m u lt ifocal p osterior p lacoid p igm en t ep ith eliop athy, 204–205, 205f Acu te m yeloid leu kem ia, orbit al involvem en t in , 94 Acu te ret in al n ecrosis syn drom e, 209 Aden oid cyst ic carcin om a, lacrim al glan d, 98, 99f Aden om a ciliar y body, 325 pleom orp h ic, lacrim al glan d, 97–98, 97f Aden oviral conju n ct ivit is, 101–103, 102f Age-related m acular degen eration , 276–278, 277f differen t ial d iagn osis, 269, 272, 274, 276, 279 exu dat ive, 276, 277f an d vit reou s h em orrh age, 270 m an agem en t , 276–278 n on exudat ive, 276, 277f presen t at ion , 276 risk factors for, 276 Age-related m acu lop athy, 276 Albinism , 285 Alkali burn s, 5–7 differen t ial d iagn osis, 112 an d recu rren t corn eal erosion , 156 Alkapton uria, blue sclera in , 134 Allergic conjun ct ivit is, 45 Allergy, 47 an d eyelid ed em a, 47, 69, 72 Alph a- Cor, 188 Am aurosis fugax, 267, 268 Am blyopia, vit reou s h em orrhage an d, 19 Am iodaron e, corn eal vert icillata cau sed by, 180, 181f Am m on ium hydroxide burn s, 5–7 AMPPPE. See Acute m ult ifocal posterior placoid p igm en t ep ith eliopathy Am yloid degen erat ion conju n ct ival, 120 corn eal, p olym orph ic, 178, 179f Am yloidosis differen t ial d iagn osis, 57, 128, 191, 192 orbital, 128

prim ar y, 191 An em ia, 261. See also Sickle cell anem ia An esth et ics, topical, an d n eurot ropic keratopathy, 155 Angioedem a, 69, 72 Angioid st reaks, 270–271, 271f causes, 270 differen t ial d iagn osis, 21, 271, 272, 276, 281, 282 m an agem en t , 271 presen t at ion , 270–271 Angiom a, facial, 130 Angle kappa, 339, 339f, 340 An iridia, 135 an d len s su blu xat ion , 13 An kylosing spondylit is, ocular involvem en t in , 194 An terior basem en t m em bran e dyst rop hy, an d recu rren t corn eal erosion , 156 An terior ch am ber cleavage syndrom e, 192, 258 An terior ch am ber dysgen esis syn drom es, 157 An terior corneal dyst rophy(ies), 158–161 An terior em br yotoxon, 157, 158 An terior sclerit is, 124 An terior segm en t t ransplan tat ion , for anterior st aphylom a, 189–191, 190f An terior uveit is, 10 Aph akia, and ret in al detach m en t , 296–297 Apocrine hydrocystom a, 57, 58f Apon eurosis repair, for ptosis, 38 “Apple peeling” techn iqu e, for rem oval of in tern al lim it ing m em bran e, 318, 319f Arcu s juven ilis, 173 Arcu s senilis, 173, 174f differen t ial d iagn osis, 157, 158 ARM. See Age-related m aculopathy ARMD. See Age-related m acular degen erat ion Ar teriosclerosis, an d m acroan eur ysm , 269 Ar terioven ou s m alform at ion , 90 cavern ous sin us, 128 ret in al, 337, 337f Ar terioven ou s n icking, 260 Ar tificial tears, 114–115 Aspergillus kerat it is, 149, 150 Ast rocytom a, retin al, 335, 336, 337 Ath erosclerosis, an d ocu lar isch em ic syn drom es, 268–269 Atop ic keratoconju n ct ivit is, 45, 110, 112, 153 Atopy ep isclerit is in , 123 an d filam en tar y kerat it is, 154 an d keratocon u s, 171 At rial sept al d efect , 353 At rop h ic bu lbi, 136 At rop h ic retin al h oles, 297, 297f Autoim m u ne disorders, 110. See also speci c disorder Avellin o dyst rop hy, 161 AVM. See Ar terioven ous m alform at ion Axen feld an om aly syndrom e, 135 Axen feld Reiger syn drom e, 157, 158 B Bacillus corn eal u lcer cau sed by, 146 trau m at ic en doph thalm it is caused by, 213

539

540

Index

Bacterial corn eal ulcer, 146–147, 146f Bacterial in fect ion differen t ial d iagn osis, 192 endogenous en dophth alm itis caused by, 215 traum at ic en doph th alm it is caused by, 213 Bacterial keratit is, 9, 143, 147, 149 Ballet sign , 76t Ban d keratop athy, 176–177, 177f Bardet-Biedl syn drom e, an d retin it is pigm en tosa, 285 Basal cell carcin om a, 59–61, 60f differen t ial d iagn osis, 51, 53, 54, 56, 62, 64 Bassen -Korn zw eig synd rom e, an d ret in it is pigm en tosa, 285 Bear-t rack lesion s, 332 Behçet syn drom e, ocu lar involvem en t in , 212–213, 212f Berlin edem a, 275 Best disease, 280–281 Biet t i’s cr yst alline retin op athy, 280 Birdsh ot ch orioret in it is, 280 Birdsh ot ret in och oroidop athy, 206–207.207f Birth t raum a, corn eal involvem en t in , 166, 170, 192 Bleeding disorders, an d hyp h em a, 15 Bleph aritis, 100–101 differen t ial d iagn osis, 46, 63, 64 infect iou s, 100, 100f pem p h igoid an d, 112 seborrh eic, 100 st aphylococcal, 100, 106 an d trich iasis, 64 Bleph arop him osis syn d rom e, 33, 38, 48, 48f, 49 Bleph aroptosis, 48 Bleph arospasm in congen ital glaucom a, 134–135 differen t ial d iagn osis, 35 Blood vessels, dilated, 125–126, 126f in St u rge-Weber syn drom e, 126–127, 127f Blow -ou t fract u res, orbit al, 26–28, 27f Blue sclera causes, 134 in osteogen esis im p erfect a, 133–134, 134f Bon e m arrow t ran sp lan tat ion , an d conju n ct ivalization of corn ea, 136 Boston sign , 76t Brain t um or su rger y, an d n eu rotrop ic keratopathy, 155 Bran ch ret in al arter y occlu sion , 265–266 differen t ial d iagn osis, 19, 266 Bran ch ret in al vein occlu sion , 265f differen t ial d iagn osis, 261, 263, 269 BRAO. See Bran ch ret inal ar ter y occlusion Breast can cer, orbit al m et astases, 94 Bron ch ial carcin om a orbital m etast ases, 94 ret in op athy secon dar y to, 217 Brow n -McLean syn d rom e, 236 Brow n syn drom e, 353–354, 353f Brucellosis, 101 Bruch m em bran e, defect s, 20 Brush field spot s, 325 BRVO. See Bran ch ret in al vein occlu sion Bu llae, subepith elial, Fuch s end oth elial dyst rophy an d, 168f Bu llous keratop athy ap h akic, 167, 169, 192 differen t ial d iagn osis, 167, 169, 192 pseu dop h akic, 167, 169, 192 Bu ph th alm os in congen ital glau com a, 134–135, 135f differen t ial d iagn osis, 157, 158 Bu rn(s) acid, 5–7 alkali, 5–7, 112, 156

ch em ical, 5–7, 6f, 64, 110, 112, 113, 136, 155, 192 an d sym bleph aron , 116 th erm al, 6, 112, 192 C Calcific ban d keratop athy, 176–177, 177f Calcium hydroxide bu rn s, 5–7 Can alicular lacerat ions, 2, 3f Can aliculit is, 65, 66f, 68 Candida albicans, n eon at al en dogen ous en doph th alm it is cau sed by, 215 Candida kerat it is, 149, 149f Can did iasis, ocu lar involvem en t in , 201 Can d lew ax d rip p ings, 199 Cap illar y h em angiom a, 81–84, 84f, 85t d ifferen tial d iagn osis, 59, 86 opt ic disk, 334, 335f ret in al, 334–335, 335f d ifferen tial d iagn osis, 329, 335, 336, 337 exop hyt ic, 329 p resen tat ion , 334 Capsular ten sion ring, 220 Carbogen th erapy, for cen t ral ret in al arter y occlu sion , 268 Cardiac failure, h igh -out put , 84 Carot id cavern ou s fist ula, 90–93, 92f, 127–129, 128f differen t ial d iagn osis, 30, 72 h igh -flow, 90, 93 low -flow, 90, 92f, 93 post t rau m at ic, 31–32, 31f, 90, 93 traum at ic, 31–32, 31f, 90, 93 Cast roviejo square graft , 186f Cat aract an d d egen erat ive m yop ia, 272 hyperm at ure, an d len s sublu xat ion , 13 m at u re, 222–223, 223f–226f m iot ic pu p il, 220–221, 221f posterior polar, 218–219, 218f posterior su bcap su lar, an d ret in it is pigm en tosa, 285 sen ile, 226–227, 226f–229f differen t ial d iagn osis, 13 su blu xated, 219–220, 219f traum at ic, 11–13, 12f Cataract su rger y. See also In t raocular len s corn eal edem a after, 236–237 an d cystoid m acu lar edem a, 238–239, 239f, 274, 275f elevated in t raocu lar p ressu re after, 238 en doph th alm it is after, 240–242, 241f hypotony after, 237–238 in t raop erat ive com p licat ion s, 234–236 iridodialysis in , 235, 235f posterior cap su lar ru pt u re in , 234–235, 235f postop erat ive com p licat ion s, 236–243 ret in al d etach m en t after, 239–240, 240f sph in cter-involving tech n ique, 221, 221f sph in cter-sparing tech n ique, 221 su b 1 m m 700-m icrom eter (Microp h akon it), 218f, 219 w oun d leak after, 237–238 Cavern ous h em angiom a, 85t, 90, 91f differen t ial d iagn osis, 81, 335, 336, 337 iris, an d hyp h em a, 15 ret in al, 335, 336, 336f, 337 Cavern ous sin us arterioven ou s m alform at ion , 128 th rom bosis, 71–73 differen t ial d iagn osis, 69, 76, 78, 93, 128 presen t at ion , 71 Cavern ous sin us syndrom e, 128 CCF. See Carot id cavern ou s fist u la Cellu lit is

Index 541 acu te st reptococcal, 69 orbital, 69, 71–73, 73t adjacen t in fect ion an d , 71 differen t ial d iagn osis, 30, 47, 66, 69, 73t, 76, 78, 87, 128 m an agem en t, 73 post t rau m at ic, 71 presen tat ion , 71 sin u s-related, 71 w arning signs w ith , 73 presept al, 69–71 w ith allergic react ion , 70f w ith bacterial infect ion, 70f w ith dacr yocyst it is, 67f differen t ial d iagn osis, 47 w ith fungal infect ion, 70f Cen t ral clou dy dyst rophy of Fran çois (corn eal), 165, 165f, 175 Cen t ral cr ystallin e dyst rop hy of Sch nyder, 164, 164f, 175 Cen t ral ret inal ar ter y occlu sion , 266–268, 267f differen t ial d iagn osis, 19, 266 w ith du rat ion of on set less th an 24 h ours, 268 m an agem en t, 268 Cen t ral ret inal vein occlu sion , 264, 265f differen t ial d iagn osis, 261, 263, 264, 269 m an agem en t, 264 presen tat ion , 264, 265f Cen t ral serou s ch orioret in opathy, 273–274, 273f differen t ial d iagn osis, 274 ep idem iology, 273 m an agem en t, 274 precip itat ing factors, 273 presen tat ion , 273 Cen t ral serou s ret in op athy, 263 CFEOM. See Ext raocular m uscle(s), congenit al fibrosis Chalazion , 45–46, 45f bleph arit is an d, 100 differen t ial d iagn osis, 46, 51, 62, 63, 69, 72 Chalcosis, 24 Chan dler syn drom e, 167, 258 Chem ical burn s, 5–7, 6f an d conju n ct ivalizat ion of corn ea, 136 differen t ial d iagn osis, 110, 112, 113, 136, 192 an d dist ich iasis, 64 an d n eurot ropic keratop athy, 155 Chem osis, 138–140 in acu te bacterial conjun ct ivit is, 105 causes, 138t m assive, 139f m ild, 139f Chen ey syndrom e, blue sclera in , 134 Cherr y-red spot , 267 Chickenpox, 143 Child abuse, t raum at ic vit reou s h em orrhage in , 18 Chlam ydia t rach om a, 106–109 Chlam ydia t rachom at is, 106, 150 Ch loroqu in e, corneal vert icillata cau sed by, 180 Chorioret inal scarring, 332 Chorioret init is syp h ilit ic, 198 Toxoplasm a, 200–202, 201f t uberculous, 201 vit iligin ou s, 206–207.207f Ch orioret inop athy, cen t ral serou s, 273–274, 273f differen t ial d iagn osis, 274 ep idem iology, 273 m an agem en t, 274

precip itat ing factors, 273 presen tat ion , 273 Ch oroid at rophy, age-related, 272 cavern ous h em angiom a, 330–331, 330f dyst roph ies, 285 h em angiom a, 327, 328, 329 m elan ocytom a, 332 m elan om a, 132, 327–329, 328f am elan ot ic, differen t ial diagn osis, 329 differen t ial d iagn osis, 216, 327, 328, 330–333, 338 m an agem en t, 329 presen tat ion , 327–329, 328f vit reou s h em orrh age in , 18 m et astat ic t u m or, 329–330, 329f differen t ial d iagn osis, 327–331 n eovascularizat ion an d cystoid m acu lar edem a, 274 differen t ial d iagn osis, 21, 275 n evu s, 326–327, 327f differen t ial d iagn osis, 328, 330, 331, 332 osteom a, 327, 331–332, 331f differen t ial d iagn osis, 327–331 ru pt u re differen t ial d iagn osis, 276 traum at ic, 20–21, 20f t um ors, 326–332 Ch oroidal det ach m en t , 316–317 expulsive, 317 h em orrh agic, 317 serou s, 316 in t raop erat ive, 316 postop erat ive, 316 trau m at ic, 316 Ch oroiderem ia, 285 differen t ial d iagn osis, 286 Ch oroidit is m u lt ifocal, 203 serp igin ou s, 205–206, 206f Ch rom ic acid bu rn , 5–7 Ch rom osom al deletion /du plication , 135 Cicatrizing disorders, 111–116 Ciliar y body aden om a, 325 cyst , 325 m elan om a, 325–326 differen t ial d iagn osis, 322 vit reou s h em orrh age in , 18 m et astat ic lesion s, 325 t um or, 323, 325–326 CIN. See Corn eal in t raepith elial n eoplasia Cleft p alate, 353 Cleidocran ial dysp lasia, blu e sclera in , 134 CME. See Cystoid m acular edem a CNV, 283 CNV m em bran es, 276–278 Coat s disease, 286, 335, 336, 337 Cobbleston e degen erat ion , 290–291, 291f Cockayn e syn d rom e, an d recu rren t corn eal erosion , 156 Cogan m icrocyst ic edem a, 158–159, 159f Cogan -Reese syn d rom e, 258, 322 Cogan syn drom e, 150 Collagen vascu lar disease differen t ial d iagn osis, 143, 172, 261 perip h eral u lcerat ive keratit is in , 182 Colobom a(s), 50–51, 50f, 219, 219f congen it al, 201 syn drom es associated w ith , 51 Com m ot io ret in ae, 19–20, 19f Con e dyst rop h ies, 281–282 differen t ial d iagn osis, 282 gen et ics, 281 m an agem en t, 282 presen tat ion , 282

542

Index

Congen ital an om aly(ies), corn eal, 157–158 Congen ital h ereditar y corneal dyst rop hy, 192 Conju n ct iva am yloid degen erat ion , 120 edem a, 138. See also Ch em osis foreign body, 5f lacerat ion , 4–5, 5f lip oid/lip id d egen erat ion , 120–121, 121f m elan om a, 132–133, 132f m et ap lasia, in ectrop ion , 137, 137f m et astases to, 133 n eop lasm s differen t ial d iagn osis, 118, 120 w ith secon dar y h em orrh age, 3 pigm en ted lesion s, 129–133 reten t ion cyst , 121–122, 121f differen t ial d iagn osis, 118 salm on p atch in , 79, 79f Conju n ct ivit is, 101–111 acu te bacterial, 105–106, 106f acu te h em orrh agic, 101, 103–104, 103f aden oviral, 101–103, 102f differen t ial d iagn osis, 112 adu lt in clu sion , 106, 107t–108t, 109 differen t ial d iagn osis, 112 allergic, d ifferen t ial diagn osis, 45 atop ic, differen t ial diagn osis, 111 bacterial, associated w ith eyelid edem a, 105, 106f ch lam ydial (in clu sion ), 106, 107t–108t, 109 differen t ial d iagn osis, 112 ch ron ic, 109–110 w ith bleph arit is, 100 differen t ial d iagn osis, 62, 63 differen t ial d iagn osis, 3, 66, 101, 114, 124, 125 gian t papillar y, 110–111, 111f differen t ial d iagn osis, 45 gon ococcal, 105–106 m u cop u ru len t , in Steven s-Joh n son syn drom e, 113 n eon atal, ch lam ydial, 106 pseu dom em bran ou s, in Steven s-Joh n son syn drom e, 113 recu rren t , 109–110 toxic, differen tial diagn osis, 192 vern al, 110–111, 111f differen t ial d iagn osis, 111, 192 viral, 101, 104 associated w ith eyelid edem a, 69, 72 Con n ect ive t issu e disease (CTD), perip h eral u lcerat ive kerat it is in , 182 Con stan t exot rop ia, 344, 344f Con t act derm at it is, d ifferen tial d iagn osis, 69, 72 Con t act len ses clean ing solu tion s, an d kerat it is, 147, 149 corn eal scarring cau sed by, differen t ial diagn osis, 171 for dr y eye, 115 gian t p ap illar y conju n ct ivit is of, 110–111 over w ear, keratit is cau sed by, 154 differen t ial d iagn osis, 152 an d recu rren t corn eal erosion , 156 u se, differen t ial diagn osis, 192 Con t recou p inju r y(ies), 11, 19 Convergen ce in su fficien cy, 343 Corn ea abrasion s, 7–8, 7f differen t ial d iagn osis, 9, 10, 105 recu rren t , 109–110 cen t ral clou dy dystrop hy of Fran çois, 165, 165f differen t ial d iagn osis, 175 cen t ral cr ystallin e dyst rop hy of Sch nyder, 164, 164f

differen t ial d iagn osis, 175 ch em ical bu rn . See Ch em ical bu rn s congen it al an om alies, 157–158 conju n ct ivalization , 136, 136f degen erat ion , 173–180 polym orp h ic am yloid , 178, 179f Salzm an n n od u lar, 177–178, 177f sph eroidal, 178 den drite in EBV in fect ion , 145 in HSV kerat it is, 141f in HZV in fect ion , 144f dep osits in , 173–180 dyst rophy. See Corn eal dyst rop hy(ies) ectat ic disorders, 170–173 edem a after cataract ext ract ion , 236–237 in congen ital h ered itar y en doth elial dyst rop hy, 170, 170f Fuchs en dothelial dyst rophy an d, 167, 168f in p osterior p olym orph ous dyst rophy, 169, 169f en larged n er ves in , 191 differen t ial d iagn osis, 163 en largem en t , in congen ital glau com a, 134–135 erosion s, trau m at ic, differen tial d iagn osis, 159, 160 farin ata, d ifferen t ial d iagn osis, 178 Fleck dyst rophy, 164–165 differen t ial d iagn osis, 166 foreign body, 8–9, 8f differen t ial d iagn osis, 7 Fu ch s en dothelial dyst rop hy, 167, 168f differen t ial d iagn osis, 167, 169, 191, 192 graft reject ion , 188, 188f gu t tae, 167, 168f differen t ial d iagn osis, 160, 178 hydrops in keratocon u s, 171 in keratoglobu s, 171, 172f in fect ion s, 141–150 in flam m at ion , 150–153 differen t ial d iagn osis, 192 inju r y, an d recu rren t corneal erosion , 156 keloid, 175–176, 176f lacerat ion , 9–10, 9f lip oid/lip id d egen erat ion , 120–121, 121f n eovascu larizat ion , in Steven s-Joh n son syn drom e, 113 perforat ion differen t ial d iagn osis, 7 in Steven s-Joh n son syn drom e, 113 recu rren t erosion , 156 scarring cen t ral, 164 in HSV kerat it is, 141f in terst it ial kerat it is an d , 150, 151f in Steven s-Joh n son syn drom e, 113 su rface d isord ers, 150–153 th in n ing. See also Keratocon u s; Pellu cid m argin al d egen erat ion (corn ea) perip h eral, 180–185 u lcer, 7 from Acantham oeba, 147, 148f bacterial, 146–147, 146f differen t ial d iagn osis, 7, 143 fu ngal, 149, 149f h erpet ic, d ifferen t ial diagn osis, 192 h erpet ic den d rit ic, 104f im m u n e-m ediated , 146, 147, 153 in fect iou s, differen t ial diagn osis, 153 m argin al in HSV in fect ion , 142 st aphylococcal, 177, 182

Index 543 sterile, 146 n eurot ropic in HSV in fect ion , 142, 143 in HZV in fect ion , 144f in Steven s-Joh nson syn d rom e, 113 t rach om a, 107f viral, differen t ial diagn osis, 146 Corn ea gu t tata, 167 Corn eal dyst rophy(ies), 158–170 an terior, 158–161 cen t ral clou dy dystrop hy of Fran çois, 165, 165f d ifferen t ial d iagn osis, 175 cen t ral cr ystallin e dyst rop hy of Sch nyder, 164, 164f d ifferen t ial d iagn osis, 175 congen it al h eredit ar y, differen t ial diagn osis, 192 congen it al h eredit ar y en d oth elial, 170, 170f d ifferen t ial d iagn osis, 166, 169 Fleck, 164–165 d ifferen t ial d iagn osis, 166 Fu ch s en doth elial, 167, 168f d ifferen t ial d iagn osis, 167, 169, 191, 192 granular, 161, 162f d ifferen t ial d iagn osis, 161, 163 lat t ice, 162–163, 162f d ifferen t ial d iagn osis, 178 p osterior, 167–170 p osterior polym orph ou s, 169, 169f d ifferen t ial d iagn osis, 166, 170, 191, 258 p re-Descem et , 165–166 st rom al, 161–166 congen it al h eredit ar y, 166 differen t ial d iagn osis, 170 d ifferen t ial d iagn osis, 164 p osterior am orp h ou s, 166 Corn eal elastosis, 178 Corn eal in t raep ith elial n eop lasia, differen t ial diagn osis, 159 Corn eal su rger y, 185–191 Corn eal vert icillata, 180, 181f Corynebacterium diphtheriae, conjun ct ivit is cau sed by, 105 d ifferen t ial d iagn osis, 112 Cou p injur y(ies), 11 Cow en sign, 76t Coxsackievirus A24, conju n ct ivit is cau sed by, 102, 103 Cranial n er ve(s) III. See Third-n er ve palsy IV. See Fou rth -n er ve p alsy VI. See Sixth -n er ve p alsy CRAO. See Cen t ral ret in al arter y occlu sion Crocodile sh agreen , differen t ial diagn osis, 175 Crocodile tears, 353 Croh n’s disease (CD), ocu lar involvem en t in, 195 Crouzon disease, an d lens sublu xat ion , 13 CRVO. See Cen t ral ret in al vein occlu sion Cr yoth erapy, for ret in al det ach m en t, 302 Cr ystallin e retin opathy, d ifferen tial d iagn osis, 280 CSCR. See Cen t ral serou s ch orioret in op athy CSR. See Cen t ral serous ret in opathy CTR. See Capsular ten sion ring Cushing syn drom e, an d cen t ral serous ch orioret in opathy, 273 Cutan eous h orn , differen t ial diagn osis, 53, 54 Cu tis laxa, blue sclera in, 134 Cyclit is, ch ron ic, 200 Cylin drom a, differen t ial diagn osis, 58 Cyst(s) ciliary body, 325 conjun ct ival lym ph at ic, 121, 121f

in parasit ic in festat ion , 121, 122 p ost t rau m atic in clu sion , 121 d erm oid. See Derm oid cyst(s) d uctal, 57–59 foveal, 275 of iris, 323 of Moll, 57, 58f p ars p lan a, 290 sebaceou s, 57, 58f of Zeis, 57–59 Cyst in osis, differen t ial diagn osis, 160 Cystoid m acular edem a, 274–276, 275f after cataract su rger y, 238–239, 239f, 274, 275f causes, 274 differen t ial d iagn osis, 263, 274, 275 m an agem en t, 275–276 path ogen esis, 274 postcat aract surger y, 238–239, 239f, 274, 275f presen tat ion , 275 Cytom egaloviru s (CMV) congen it al in fect ion , 208 in im m u n osu pp ressed p at ien t , 208, 209f in clu sion ch orioret init is, d ifferen t ial diagn osis, 201 ret in itis caused by, 208, 208f D Dacr yoaden it is, 78, 97 Dacr yocyst itis, 66–67, 67f ch ron ic, differen t ial diagn osis, 65 differen t ial d iagn osis, 47, 68, 110 Dalyr ym p le sign , 76t Degen erat ive m yopia, 272, 272f Derm atobu llou s d isord ers, differen t ial diagn osis, 112 Derm atoch alasis, 42–43, 42f differen t ial d iagn osis, 45, 47 Derm oid cyst(s), 50, 80–81, 82f–83f deep, 80–81, 82f–83f differen t ial d iagn osis, 84, 90, 93, 94, 118, 192 ru pt u red , differen t ial d iagn osis, 87 su perficial, 80–81, 82f Descem et m em bran e breaks, keratoconu s an d, 171 ru pt u re, 134 tears, differen t ial diagnosis, 192 Descem et st ripp ing en doth elial keratoplast y, 187, 187f Diabetes m ellit u s. See also Maculopathy, diabet ic an d n eu rot ropic keratopathy, 155 Diabet ic ret in opathy, 261–264 differen t ial d iagn osis, 261, 263, 264, 269, 286 m an agem en t, 263–264, 263f n onp roliferat ive, 261, 262f differen t ial d iagn osis, 263 presen tat ion , 261 proliferat ive, 261, 262f differen t ial d iagn osis, 263 w ith preret in al h em orrh age, 262f–263f an d vit reou s h em orrh age, 270 Diath erm y, for ret in al det ach m en t , 301 Diffu se corn eoscleral kerat it is, 190 Dilated vessels, 125–126, 126f in St u rge-Weber syn drom e, 126–127, 127f Dim m er kerat it is, 101 Dissociated st rabism u s com p lex, 346–347, 347f Distich iasis, 64, 65f differen t ial d iagn osis, 35, 110 pem p h igoid an d, 112 Distract ion test , 33

544

Index

Divergen ce excess, 343 Doh lm an keratoprosth esis, 188, 189f Dow n syn drom e Brush field sp ot s in , 325 corn eal involvem en t in , d ifferen tial diagn osis, 192 an d keratocon u s, 171 Drug(s), an d hyp h em a, 15 Drug abu se, differen t ial d iagn osis, 263 Drug-in d u ced degen erat ion s, differen t ial diagn osis, 281 Drug-ind uced ret in op athy, differen t ial diagn osis, 282 Dru sen fam ilial, differen t ial diagn osis, 279, 280 opt ic n er ve h ead , an d ret in itis p igm en tosa, 285 Dr y eye causes, 114 differen t ial d iagn osis, 101 Dr y-eye syn d rom e, 114–116, 114f an d filam en tar y kerat it is, 154 DSEK. See Descem et st ripping en dothelial keratoplast y Du an e syn drom e, 351–353, 352f bilateral, 352, 352f system ic association s, 353 t ypes, 352f, 353 Du ral sin u s arterioven ou s fist u la, differen t ial diagn osis, 128 Dysthyroid orbitopathy, 74–77, 74f–75f differen tial d iagn osis, 93, 128 m an agem ent , 76–77 E Eales disease differen tial d iagn osis, 286 an d vit reou s h em orrh age, 270 EBMD. See Epith elial basem en t m em bran e dyst rophy Eccrin e hydrocystom a, 57, 58f differen tial d iagn osis, 57 Eclip se ret in op athy, 287 Ectop ia len t is, 251 congen it al, an d len s su blu xat ion , 13 differen tial d iagn osis, 13 Ect rop ion , 33–35, 34f in bleph aroph im osis syn drom e, 48, 48f cicat ricial, 33–35, 34f congen ital, 33, 35 conju n ct ival m etaplasia in , 137, 137f differen tial d iagn osis, 45 involut ion al, 33, 34f m ech anical, 33 paralyt ic, 33–35, 34f Eczem a, differen t ial d iagn osis, 47 Edem a. See also Angioedem a; Cystoid m acu lar edem a Cogan m icrocyst ic, 158–159, 159f conju n ct ival, 138. See also Ch em osis corn eal after cataract ext ract ion , 236–237 in congenit al heredit ar y en doth elial dyst rophy, 170, 170f Fu ch s en doth elial dyst rophy an d, 167, 168f in posterior polym orph ou s dyst rop hy, 169, 169f eyelid, 47, 47f in acu te bacterial conju n ct ivit is, 105, 106f allergy an d, 47, 69, 72 in thyroid eye disease, 47, 47f in viral conju n ctivit is, 69, 72 m acular clin ically sign ifican t , 261, 262f diabet ic, 283

d ifferen tial d iagn osis, 283 orbit al, 27 ret in al, post t rau m at ic, 21–22 su bconju n ct ival. See Ch em osis Eh lers-Dan los syn d rom e an d angioid st reaks, 270 blue sclera in , 134 an d d egen erat ive m yop ia, 272 an d len s su blu xat ion , 13 Eikenella can aliculit is, 66f Elsch n ig spot s, 260–261 Em boli, in bran ch ret in al arter y occlu sion , 265–266 En ceph alocele, 84 En closed oral bay, 289, 289f En dop h th alm it is, 213–216 after cataract surger y, 240–242, 241f Candida, 215 differen t ial d iagn osis, 326, 335, 336, 337 en dogen ou s, 215–216 postop erat ive, 214–215, 214f, 240–242, 241f post t rau m at ic, 12f sequ elae, differen t ial diagn osis, 136 traum at ic, 213–214 En doth elial keratop last y, 187, 187f En doth eliitis, in HSV in fect ion , 142 En op h th alm os, ru pt u red globe an d, 16 Enterobacter, corn eal u lcer caused by, 146 En teroviru s grou p 70, conju n ct ivit is cau sed by, 102, 103 En t rop ion , 35, 36f cicatricial, 35, 36f congen it al, 35 differen t ial d iagn osis, 48, 49, 64 involu t ion al, 35, 36f spasm odic, 109, 110f EOB. See En closed oral bay Epiblep h aron , 48–49, 49f d ifferen tial d iagn osis, 35, 49, 64 Epican th al fold s, syn drom es associated w ith , 49 Epican th u s, 49–50, 50f Epican th u s inversu s, 49 in blep h aroph im osis syn drom e, 48, 48f Epican th u s p alp ebralis, 49 Epican th u s su p erciliaris, 49 Epican th u s tarsalis, 49, 50f Epid erm olysis bu llosa, 112 Epin eph rin e, top ical, an d cystoid m acu lar edem a, 274 Epip h ora, 2 in congen ital glau com a, 134–135 Epiret in al m em bran e d ifferen tial d iagn osis, 23, 321 id iopath ic, 320–321, 320f p rim ar y, 320 secon dar y, 320 Episcleral ven ou s pressu re, elevated, 252–253 Episclerit is, 123–124, 123f–124f differen t ial d iagn osis, 3, 125 n odular, 123, 124f in Steven s-Joh n son syn drom e, 113 Ep ith elial basem en t m em bran e dyst rop hy, 158–159, 159f differen t ial d iagn osis, 160, 161 Ep ith eliom a, 112 Ep stein -Barr viru s (EBV), in fect ion corn eal, 145, 145f, 150, 152 differen t ial d iagn osis, 101, 152 ERM. See Ep iretin al m em bran e Er ysip elas, 69, 72 Er yth em a m u lt iform e differen t ial d iagn osis, 112, 136 an d sym bleph aron , 116 Er yth em a m u lt iform e m ajor, 113

Index 545 Er yth em a m u lt iform e m in or, 113 Er yth roderm a, congen ital ich thyosiform , 112 Esot rop ia accom m odat ive, 341–342, 341f refract ive, 341f, 342 in fan t ile, 340–341, 340f Ew ing sarcom a, m etast atic, 94 Exoph th alm os post t rau m at ic, 31–32, 31f pu lsat ing, 127–128 causes, 31 post t rau m at ic, 31–32, 31f ru pt u red globe an d , 16 in thyroid eye d isease, 73t Exot rop ia con secu t ive, 344 con st an t , 344, 344f in fan t ile, 344 in term it ten t , 342–343, 343f secon dar y, 344 sen sor y, 344 Exp osu re kerat itis, 183 Exposu re keratopathy, 153–154 Ext raocu lar m u scle(s). See also speci c m uscle congen it al fibrosis, 357–358, 357f Eyelid (s) bacterial in fect ion , 69 colobom a, 50–51, 50f deform it ies, in Steven s-Joh n son syn drom e, 113 dist ract ion test , 33 edem a, 47, 47f in acu te bacterial conjun ct ivit is, 105, 106f allergic, 47, 69, 72 in thyroid eye d isease, 47, 47f in viral conjun ct ivit is, 69, 72 inw ard rotat ion . See En t ropion lacerat ion , 1–2, 1f, 3f ou t w ard rotat ion (eversion ). See Ectrop ion papillom a, 51, 51f ret raction , 43, 43f t um ors, 51–57, 59–64 u pp er abn orm ally low p osit ion . See Ptosis cicat ricial ch anges, 43 scarring, 43 F Fabr y disease, corn eal vert icillata in , 180, 181f Fam ilial aden om atou s p olyp osis, 332 Fam ilial exu dat ive vit reoret in op athy, 335, 336, 337 Ferr y lin e, 179 FF. See Fun dus flavim aculat us Fibrin collaret tes, 100, 100f Filam en tar y keratit is, 154–155, 154f Fleck dyst rop hy (corn eal), 164–165 differen t ial d iagn osis, 166 Fleisch er ring, 179, 179f keratocon u s an d , 171 Fleu ret tes, 334 Flexn er-Win terstein er roset tes, 334 Flop py eyelid syn drom e, 44–45, 44f an d keratocon u s, 171 Foreign body conju n ct ival, 5f, 129, 130 corn eal, 7, 8–9, 8f differen t ial d iagn osis, 7, 9, 129, 130, 133, 323 in t raocu lar, 9, 23–25, 24f, 217, 323 int raorbit al, 28–29, 29f an d recu rren t corn eal erosion , 156 Fourth -n er ve palsy, 349–350, 349f–350f Foveal cysts, 275 Foveom acu lar ret in it is, 287 Fract ure(s), orbit al, 26–28

isolated, 30 Francesch et t i syn drom e, 51 Front alis-sling procedure, for ptosis, 38 Fu ch s en dothelial dyst rop hy, 167, 168f differen t ial d iagn osis, 167, 169, 191, 192 Fuchs h eteroch rom at ic iridocyclit is, 197 Fuchs h eteroch rom ic iridocyclit is, 197 Fuchs spot , 272 Fuchs superficial m argin al kerat it is, 184 Fun dus albipun ctat us, 280, 280f differen t ial d iagn osis, 279 Fun dus flavim aculat us, 278–279, 279f differen t ial d iagn osis, 279 m an agem en t, 279 presen tat ion , 278, 279f Fungal in fect ion differen t ial d iagn osis, 192 en dogen ou s en dophth alm it is caused by, 215 postop erat ive en doph th alm it is caused by, 214 trau m at ic en doph thalm it is caused by, 213 Fungal kerat it is, 149–150, 149f differen t ial d iagn osis, 9, 143, 146, 147 Furrow degen erat ion , 185 differen t ial d iagn osis, 183, 184 Fusarium keratit is, 149, 150 G Gardn er syn drom e, 332 Gast roesoph ageal reflu x disease, and cen t ral serou s chorioret in opathy, 273 Gastroin test in al m align an cy, orbit al m et astases, 94 Gen t am icin , in t raocu lar inject ion , 267 Gh ost vessels, 150, 151f differen t ial d iagn osis, 163 Gian t cell arterit is, 268–269 Gifford sign , 76t Glau com a acu te, 3 acu te angle, 7 angle-closu re, 254–257 acu te, 254–255, 254f, 255f ch ron ic, 256, 256f w ith pupillar y block, 254 w ithout pupillar y block, 254 angle recession , 13 congen it al, 127, 134 differen t ial d iagn osis, 68, 166, 170, 192, 326 gh ost-cell, vit reou s h em orrh age an d , 18–19 len s-in du ced, 250–251 len s p art icle, 250 m align an t , 258–259 differen t ial d iagn osis, 326 n eovascular, 256–257 n orm al-ten sion (low -ten sion ), 246–248, 246f–247f op en -angle an d d egen erat ive m yop ia, 272 prim ar y, 244, 245f an d ret in it is pigm en tosa, 285 secon dar y, 248, 249f ph acoan ap hylact ic, 251 ph acolyt ic, 251 ph acom orp h ic, 12, 251 pigm en tar y, an d degen erat ive m yopia, 272 in p osterior p olym orph ou s dyst rophy, 169 post t rau m at ic, 12 in sarcoidosis, 198 uveit ic, 251–252, 252f, 253f differen t ial d iagn osis, 15 in uveit is-glau com a-hyp h em a syn drom e, 196 uveit is related to, 196

546

Index

Globe, rupt u red, 16–17, 17f differen t ial d iagn osis, 30 Golden h ar syn d rom e, 51, 353 Goldzieh er sign , 76t Grapevin es, in adu lt toxop lasm osis, 201 Graves’ disease, 74–77 in filt rat ive (severe), 74 n on in filt rat ive (m ild), 74 op h th alm ic m an ifest at ion s, 76t Graves oph th alm op athy, 355–356, 355f. See also Thyroid eye disease; Thyroidrelated op h th alm op athy Grid laser, for diabet ic ret in op athy, 263, 263f Griffith sign , 76t Grön blad-St ran dberg synd rom e, an d angioid st reaks, 270 Guillain -Barré syn drom e, Miller-Fish er varian t , eyelid ret ract ion in , 43 Gyrate at rop hy differen t ial d iagn osis, 272, 285, 286 gen et ics, 286 m an agem en t, 286 presen tat ion , 286 H Haab st riae, 134–135 Haem ophilus in uen zae conju n ct ivit is, 105 Ham artom a, com bin ed, of ret in al p igm en t ep ith eliu m an d ret in a, 333, 333f differen t ial d iagn osis, 327, 328, 330, 331, 333 Han d an om alies, 353 Hassall-Hen le bodies, 167 Hearing loss, 353 Helm in th in fect ion , 150 Hem angiom a. See also Cap illar y h em angiom a; Cavern ous h em angiom a ch oroidal, 327, 328, 329 exophyt ic ret in al, 329 racem ose, 335, 336, 337 Hem op h ilia, an d hyp h em a, 15 Hem orrh age opt ic n er ve sh eath , 26 orbital, 26 ret robu lbar, 29–30, 30f ru pt u red globe an d , 16 su bconju n ct ival, 3–4, 4f su bret in al, 327, 328, 330, 331 vit reou s. See Vitreou s h em orrh age Hem osiderosis bu lbi, vit reou s h em orrh age an d, 19 Hep atolen t icu lar degen erat ion , 180 Herp es sim p lex ch orioret in it is, 201 keratit is, 150, 151f, 167, 192 keratoconju n ctivit is, 104–105, 104f Herp es sim p lex viru s acu te ret in al n ecrosis syn drom e cau sed by, 209 infect ion corn eal, 141–143, 141f differen t ial d iagn osis, 142, 147 in it ial, 141 recu rren ce, 142 differen t ial d iagn osis, 101, 143, 191 an d in terstit ial kerat it is, 150, 151f an d recu rren t corn eal erosion , 156 skin lesion s in , 104f an d n eurot ropic keratop athy, 155 Herp es zoster. See also Varicella-zoster viru s infect ion , 101, 191 corn eal, 143–145, 144f, 150 differen t ial d iagn osis, 147 kerat it is, 192 an d n eurot ropic keratop athy, 143, 144f, 155 Herp es zoster op h th alm icu s, 143–145, 144f

an d trich iasis, 64 Herring’s law, 43 High accom m odat ive esot ropia, 341f, 342 Histoplasm osis ocu lar differen t ial d iagn osis, 21, 201, 272 presu m ed, 202–203, 202f vit reou s h em orrh age in , 18 Histospot s, 202f differen t ial d iagn osis, 279, 280 Hollen h orst p laqu es, 265 Holt-Oram syn d rom e, 353 Hom er Wrigh t roset tes, 334 Hom ocyst in u ria, 135 an d len s su blu xat ion , 13, 14 Hord eolu m , 46–47, 46f bleph arit is an d, 100 differen t ial d iagn osis, 46, 47, 66 extern al, 46–47, 46f in tern al, 46–47 Hu dson -Stäh li lin e, 179 Hu rler syn drom e, 267 Hyalin e Pillat scleral plaque, 140, 140f Hydroch loric acid bu rn , 5–7 Hydroxych loroquin e, corn eal ver ticillat a cau sed by, 180 Hyperbaric oxygen , for cent ral ret inal ar ter y occlu sion , 268 Hyperlipoprotein em ia, 173, 175 Hyperlysinem ia, and len s sublu xat ion , 13 Hyperten sion, 267 an d angioid st reaks, 270 an d cen t ral serou s ch orioret in opathy, 273 an d hyp h em a, 15 an d m acroan eu r ysm , 269 Hyp erten sive ret in opathy, 260–261, 260f differen t ial d iagn osis, 263, 264, 286 Hyperthyroidism , 355–356. See also Graves’ disease; Graves oph th alm opathy Hyphem a differen t ial d iagn osis, 13 grad ing system , 15, 15f post t rau m at ic, 10f sp on tan eou s, cau ses, 15 traum at ic, 14–16, 15f in uveit is-glau com a-hyp h em a syn drom e, 196 Hypoparathyroidism , 192 Hypopyon, in fu ngal in fect ion , 149, 149f Hypotony, after cataract su rger y, 237–238 I Ich thyosis, 33 differen t ial d iagn osis, 166, 191 In born errors of m etabolism , corn eal involvem en t in , 192 In clusion conjun ct ivitis, 192 In dom eth acin , corneal vert icillata caused by, 180 In fan t ile esot ropia, 340–341, 340f In fan t ile exot ropia, 344 In fect ion(s) bacterial, eyelid, 69 an d conju n ct ivalizat ion of corn ea, 136 corn eal, 141–150 differen t ial d iagn osis, 84, 192 orbital, 69–99 In fect ious keratit is, 182, 183 In fect ious m onon ucleosis, 145 w ith eye involvem en t , differen t ial diagn osis, 101 In ferior obliqu e m u scle, palsy, isolated, 348 In ferior oblique overact ion , 345–346, 346f In flam m at ion ch ron ic, an d len s su blu xat ion , 13 corn eal, 150–153

Index 547 differen t ial d iagn osis, 192 d ifferen t ial d iagn osis, 135 in traocu lar, 194–217 lacrim al glan d, 97 orbital, 71–80 differen t ial d iagn osis, 73t an d sym bleph aron , 116 In flam m ator y bow el d isease (IBD), ocu lar involvem en t in , 195 In sect bite, differen t ial diagn osis, 69, 72 In term it ten t exot ropia, 342–343, 343f basic t ype, 343 convergen ce insu fficien cy, 343 d ivergen ce excess, 343 In tern al lim it ing m em bran e, rem oval, “ap ple peeling” tech n iqu e, 318, 319f In terst it ial kerat it is, 150–151, 151f In t raarterial fibrin olysis, for cen t ral ret in al arter y occlu sion , 268 In traocu lar len s d islocated, tem p orar y h apt ic extern alizat ion , 233–234, 234f foldable, im p lan t at ion , 227, 229f glued , 229–233, 230f–233f opacificat ion , 242–243, 242f torn , 236, 236f In traocu lar p ressu re, elevated , after cataract su rger y, 238 IOL. See In t raocu lar len s Irid ocorn eal en doth elial syn drom e, 258 d ifferen t ial d iagn osis, 169, 323 Irid ocyclit is Fuch s h eteroch rom ic, 197 h erpet ic, 104–105 Irid od ialysis, p ost t rau m at ic, 12f Irid od on esis, 13 Iris at rop hy in HSV kerat it is, 141f progressive, 258 cavern ou s h em angiom a, an d hyp h em a, 15 cyst , 323 freckle, d ifferen t ial diagn osis, 322 juvenile xan thogran ulom a an d, 323–324 leukem ic n odu les, 324 m elanom a, 323 differen t ial d iagn osis, 322 m et astat ic lesion s, differen t ial diagnosis, 324 n evus, 322 p igm en t epith elial cyst , 322–323 t u m ors an d n odu les, 322–325 Iris bom bé, in sarcoidosis, 198 Iris sph in cter tear, 11, 11f Irit is d ifferen t ial d iagn osis, 3 in Steven s-Joh n son syn drom e, 113 syphilit ic, 198 t raum at ic, 10–11, 10f Irit is n odosa, in syph ilis, 198 Irit is papu losa, in syph ilis, 198 Irit is roseat a, in syp h ilis, 198 Iron dep osit s, 179 conju n ct ival, differen t ial diagn osis, 129, 130 Iron lin es, 179–180, 179f Ir vin e-Gass syn drom e, 274, 275 Isch em ic opt ic n eu ropathy, differen t ial d iagn osis, 261, 267 J Jacob-Agar w al sling surger y, 37f, 38–41, 39f–41f Jellin ek sign , 76t Juven ile ret in osch isis an d cystoid m acu lar edem a, 274 d ifferen t ial d iagn osis, 275

Juven ile rh eum atoid arthrit is ocu lar involvem en t in , 196–197 pau ciar t icu lar on set , 197 polyar t icu lar on set , 197 system ic on set , 197 t ype 1, 197 t ype 2, 197 uveit is in , 196–197 Juven ile xan th ogran ulom a, 135, 216t, 217 differen t ial d iagn osis, 323 an d hyp h em a, 15 iris lesion s, 323–324 K Kap osi sarcom a, w ith secon dar y h em orrh age, 3 Kap p a angle, 339, 339f, 340 Kasabach -Merrit t syndrom e, 84 Kayser-Fleischer ring, 24, 180 Kearn s-Sayre syn drom e, an d ret in it is p igm en tosa, 285 Keloid, corn eal, 175–176, 176f d ifferen tial d iagn osis, 177 Kerat it is Acantham oeba, 147–149, 148f d ifferen tial d iagn osis, 146, 149, 192 Aspergillus, 149, 150 bacterial, 9, 143, 147, 149 Candida, 149, 149f con t act len s clean ing solu t ion s an d , 147, 149 con t act len s over w ear, 152, 154 d en drit ic in t raepithelial d ifferen t ial d iagn osis, 142 in HSV in fect ion , 141, 141f, 142 d iffu se corn eoscleral, 190 d im m er, 101 d isciform , h erp et ic, 104–105 exp osu re, 183 filam en tar y, 154–155, 154f Fu ch s su p erficial m argin al, 184 fu ngal, 149–150, 149f d ifferen t ial d iagn osis, 9, 143, 146, 147 Fusarium , 149, 150 h erpes sim plex, 150, 151f, 167, 192 h erpes zoster, 192 h erpet ic d en d rit ic, 141, 141f, 142 in t raep ith elial, 141f, 142 st rom al, 141f, 142 im m u n e st rom al, in HSV in fect ion , 141, 141f, 142–143 in fect iou s, 182, 183 in terst it ial, 150–151, 151f h erpet ic, 104–105 n ecrotizing st rom al in HSV in fect ion , 142 in HZV in fect ion , 144f n eurot ropic, in HSV in fect ion , 142, 143 n um m ular, 143, 145 Paecilom yces, 149, 150 p un ctate, in viral in fect ion , 141–142, 143, 145 st ap hylococcal m argin al, 146 st riate, after cat aract ext ract ion , 237f st rom al d ifferen tial d iagn osis, 142, 145 in EBV in fect ion , 145, 145f su p erficial pu n ct ate, w ith ch em ical bu rn , 5 su perior lim bic, 45 Thygeson superficial pun ctate, 152, 152f u lcerat ive d ifferen tial d iagn osis, 6, 182, 184 p erip h eral, 182–184, 183f, 184 u lt raviolet (UV), 6 viral, 149, 150

548

Index

Keratoacan th om a, 53–54, 53f differen t ial d iagn osis, 59, 62, 64 Keratoconju n ct ivit is atop ic, 110 differen t ial d iagn osis, 45, 112, 153 ep idem ic, 101–103 differen t ial d iagn osis, 105, 152 ep idem ic h em orrh agic, 103–104, 103f h erp es sim p lex, 104–105, 104f ph lycten u lar, 192 su perior lim bic, 122–123, 122f differen t ial d iagn osis, 63, 192 an d filam en tar y kerat it is, 154 vern al, 110, 146 sh ield u lcer in , 152–153, 153f Keratoconju n ct ivit is sicca, 192 Keratocon u s, 170–171, 171f differen t ial d iagn osis, 172, 191 an d ret in it is pigm en tosa, 285 Keratoglobu s, 171–172, 172f Keratopathy ban d, 176–177, 177f bullous ap h akic, 167, 169, 192 differen t ial d iagn osis, 167, 169, 192 pseu dop h akic, 167, 169, 192 calcific ban d, 176–177, 177f exposure, 153–154 n europaralyt ic, 153–154 n eurot ropic, 143, 144f, 155–156, 155f in HZV in fect ion , 143, 144f, 155 prim ar y lipid , 175, 175f Keratoplast y Descem et st rip p ing en d oth elial, 187, 187f graft reject ion in , 188, 188f, 192 lam ellar, 186f, 187 pen et rat ing, 185, 186f graft reject ion in , 188, 188f Keratop rosth esis, 188, 189f Kh odadou st lin e, 188, 188f Kidn ey, m align an cy of, orbital m etast ases, 94 Klipp el-Feil syn drom e, 353 Kn ies’ sign , 76t Koch er sign , 76t L Lacerat ion (s) can alicu lar, 2, 3f conju n ct ival, 4–5, 5f corn eal, 9–10, 9f eyelid, 1–2, 1f, 3f Lacrim al glan d(s) accessor y, 121 aden oid cyst ic carcin om a, 98, 99f en largem en t , 97–99 in flam m at ion , 97 t um ors, 97–98 ben ign m ixed, 97–98, 97f Lacrim al sac t u m or, 68 Lacrim al system disord ers, 65–68 traum a, 2, 3f Lagop h th alm ia, 154 Lam ellar keratop last y, 186f, 187 Lateral rect us m u scle palsy, 350–351, 351f Lat tice corn eal dyst rop hy, 162–163, 162f differen t ial d iagn osis, 178 Lat tice degen erat ion , 292–293 Leber congen ital hereditar y opt ic n eu rop athy, an d keratocon u s, 171 Leiom yom a differen t ial d iagn osis, 323, 325 uveal, 324 Len s dislocat ion , 13–14 asym ptom at ic, 14

w ith broken capsule an d in flam m at ion , 14 in to vit reou s, 14 su blu xat ion , 13–14, 14f asym ptom at ic, 14 w ith cataract , 14 w ith h igh un correct able ast igm at ism , 14 w ith m on ocular diplopia, 14 Len t igo m align a, 63f, 64 Lep rosy differen t ial d iagn osis, 150, 191, 192 an d n eu rot ropic keratop athy, 155 Leu kem ia differen t ial d iagn osis, 78, 94, 323 an d hyp h em a, 15 in t raocu lar, 216, 216t iris n odu les in , 324 orbital involvem en t in , 96f Leu kocorn ea, 192–193 Levator m u scle fu n ct ion , m easu rem en t , 36 palsy, isolated, 348 Lim bal follicles, 118 Lim bal girdle of Vogt , 174, 174f Lipid degen erat ion of corn ea an d conjun ctiva, 120–121, 121f Lipid s, seru m , abn orm alit ies. See also Hyperlipoprotein em ia an d m acroan eu r ysm , 269 Lipoprotein em ia, 164 Lisch n odules differen t ial d iagn osis, 322 in n eu rofibrom atosis, 325 Low e syn d rom e, 135, 175 corn eal involvem en t in , 192 Low ey sign , 76t Lym e d isease, an d in terst it ial kerat it is, 150, 151 Lym ph angiom a, 85–87, 85t, 86f differen t ial d iagn osis, 84, 93 Lym ph ogran u lom a ven ereu m (LGV), 106 Lym ph oid hyp erp lasia, 133 Lym ph om a(s) differen t ial d iagn osis, 59, 90 in t raocu lar, 216, 216t orbital, 79–80, 79f M Macroaneur ysm , 269–270 differen t ial d iagn osis, 263 Macular degen erat ion age-related. See Age-related m acular degen erat ion differen t ial d iagn osis, 280 juven ile, 278 Macu lar dyst rop hy, 163, 163f differen t ial d iagn osis, 161 vitelliform , 280–281 Macular edem a clin ically sign ifican t , 261, 262f diabet ic, 283 differen t ial d iagn osis, 283 Macular h em orrhage, 267 Macular h ole, 267, 296, 317–318, 317f, 318f differen t ial d iagn osis, 274, 318 idiopath ic, 23 m an agem en t by obser vat ion , 318 su rgical, 318, 319f st ages, Gass classificat ion, 318 traum at ic, 22–23, 23f Macular ret in al vein occlusion, 265f Maculopathy age-related. See Age-related m acu lopathy diabet ic, 261, 262f an d cystoid m acu lar edem a, 274

Index 549 differen t ial d iagn osis, 263, 269 Madarosis w ith bleph arit is, 100 w ith m align an cy, 59 Maffu cci syn drom e, 84 Malign an t m elan om a. See Melan om a Man dibu lofacial dysostosis. See Fran cesch et t i syn d rom e Man n is lin e, 179 Map-d ot-fingerprin t corn eal dyst rop hy, 158–159, 159f Marcu s- Gu n n jaw -w in king syn drom e, 38 Marfan syn d rom e, 135 blue sclera in , 134 and d egen erat ive m yop ia, 272 an d keratocon u s, 171 and len s su blu xat ion , 13, 14 Margin to lid reflex, 36 Masqu erade syn d rom es, 216–217, 216t Medial rect us m uscle palsy, isolated , 348, 348f Medu lloep ith eliom a of ciliar y ep ith eliu m , 326 d ifferen tial d iagn osis, 323, 325, 326 Meesm an n dyst rop hy, 160, 160f d ifferen tial d iagn osis, 159, 161 Megalocorn ea, 157 Meibom ian glan d dysfu n ct ion , p em p h igoid an d , 112 Meibom it is, w ith blep h arit is, 100 Melan ocytom a(s), 338, 338f choroidal, 332 Melan ocytosis ocu lar, 129–130, 129f, 324–325 differen tial d iagn osis, 322 ocu lod erm al, 130, 130f differen tial d iagn osis, 129 Melan om a am elan otic ch oroidal, 329 differen tial d iagn osis, 324, 329 choroidal, 132, 327–329, 328f am elan ot ic, 329 differen tial d iagn osis, 216, 327–333, 338 m an agem en t , 329 presen tat ion , 327–329, 328f vit reou s h em orrh age in , 18 ciliar y body, 325–326 differen tial d iagn osis, 322 vit reou s h em orrh age in , 18 conju n ct ival, 132–133, 132f differen tial d iagn osis, 131 cutan eou s, 63–64, 63f ret in op athy secon dar y to, 217 d ifferen tial d iagn osis, 52, 56, 59, 129, 130 d ilated episcleral sen t in el vessels in , 125 of iris, 323 n odular, 64 orbital m etast ases, 94 p ingu ecu la an d , 118f sup erficial spreading, 64 su r veillan ce, w ith m elan ocytosis, 325 Melan osis conju n ct ival ben ign ep ith elial, 131–132, 131f d ifferen tial d iagn osis, 133 p rim ar y acqu ired differen tial d iagn osis, 129, 130 m align an t poten t ial, 132 Melan osis bu lbi, 140 Men ingiom a, 81 Men kes syn drom e, blu e sclera in , 134 Meridion al com p lex, 289 Meridion al folds, 289, 289f Metabolic d isease, 135 Metallosis bu lbi, 23 Metastat ic d isease

ch oroidal, 329–330, 329f d ifferen tial d iagn osis, 327–331 ciliar y body, 325 conju n ct ival, 133 d ifferen tial d iagn osis, 80, 324 of iris, 324 orbital, 94, 96f MEW DS. See Mult iple evan escen t w h ite dot syn d rom e Microan eu r ysm s, 261 Microcorn ea, 157 Microhyp h em a, 14–16 t raum at ic, 10 Microp an n u s, 192 Microp h akon it, 218f, 219 Microp h th alm os, 50 d ifferen tial d iagn osis, 157, 158 Migrain e, 295 Möbiu s sign , 76t Mollu scu m con tagiosu m , 54, 54f d ifferen tial d iagn osis, 51, 192 Mon ocu lar elevat ion deficien cy, 354–355, 354f Mooren u lcer, 180–182, 182f d ifferen tial d iagn osis, 183 MRD. See Margin to lid reflex Mu cocele, 93–94, 95f d ifferen tial d iagn osis, 65, 68, 81 eth m oidal, 65, 68 Mu colip idosis, corn eal involvem en t in , 192 Mu cop olysacch arid osis, corn eal involvem en t in , 166, 170, 192 Mu corm ycosis, 128 Mu lt iple en d ocrin e n eoplasia, t yp e IIb, 191 Mu lt ip le evan escen t w h ite d ot syn drom e, 203–204, 203f–204f Mum ps, corn eal involvem en t in, 150 Myasth en ia gravis, 356–357, 356f Myopia degen erat ive, 272, 272f ocu lar associat ion s, 272 system ic associat ion s, 272 h igh , 272, 272f “blu e sclera” in , 134 differen t ial d iagn osis, 21, 286 an d len s su blu xat ion , 13 an d t rau m at ic ret in al detach m en t , 21 an d ret in al d etach m en t , 296 an d ret in it is pigm en tosa, 285 Myopic sh ift , in in fan ts, vit reou s h em orrh age an d, 19 Myosit is, 78 Myoton ic dyst rop hy, 281, 285 Myoton ic ret inop athy, 282 N Nanoph th alm os, 157 Naproxen , corn eal vert icillata caused by, 180 Nasolacrim al duct obst ruct ion, 67–68, 67f Neoplasm (s), 322–338. See also Tum or(s) conju n ct ival, w ith secon dar y h em orrh age, 3 Neovascu larizat ion ch oroidal an d cystoid m acu lar edem a, 274 differen t ial d iagn osis, 21, 275 corn eal, in Steven s-Joh n son syn d rom e, 113 in diabet ic ret in op athy, 261 differen t ial d iagn osis, 263 in ocu lar isch em ic syn drom e, 268, 269f Neovascu lar m em bran es, su bret in al, 21 Ner ve fiber layer, m yelin ated, 266 Neu rilem m om a, 81 Neu roblastom a differen t ial d iagn osis, 84 m et astat ic, 87, 94

550

Index

Neu rofibrom a, 58–59, 59f plexiform , 86 Neu rofibrom atosis, 135 differen t ial d iagn osis, 191 Lisch n odules in , 325 Neu roparalyt ic keratop athy, 153–154 Neu roret in it is differen t ial d iagn osis, 263 syp h ilit ic, 198 Neu rot ropic keratopathy, 143, 144f, 155–156, 155f Nevu s (pl., n evi), 54–56, 55f ch oroidal, 326–327, 327f differen t ial d iagn osis, 328, 330, 331, 332 com p ou n d, 54–56, 55f, 131, 132 differen t ial d iagn osis, 52, 59, 64, 129, 130, 133, 323 int raderm al, 54–56, 55f of iris, 322 jun ct ional, 54–56, 55f, 131, 132 m align an t , sign s, 132, 133 m align an t poten t ial, 131, 132 st raw berr y, 81 Nevu s flam m eu s, 84 Nevu s of Ot a, 56, 56f, 324–325 differen t ial d iagn osis, 129 Nicolas-Favre disease, 106 Nicot inic acid, an d cystoid m acular edem a, 274 Nigh t blin dn ess, congen it al st at ion ar y, 280 differen t ial d iagn osis, 285 Nit ric acid burn, 5–7 Nod u le(s), of iris, 322–325 Nonproliferat ive diabet ic ret in op athy. See Diabet ic ret inopathy, n onproliferat ive Non steroidal an t i-in flam m ator y drugs (NSAIDs), an d su bconju n ct ival h em orrh age, 3–4 NPDR. See Diabet ic ret in op athy, n onp roliferat ive O OAG. See Glau com a, open -angle Ocu lar isch em ic syn drom e, 268–269, 269f differen t ial d iagn osis, 263, 264, 269 Ocu lar m assage, 268 Ocu lar vein varicosit ies, 125–126 Ocu loau ricu lovertebral dysp lasia. See Golden h ar syndrom e Oculocerebroren al syndrom e, 135, 175 Oculogen it al syndrom e, 107t–108t OIP. See Orbital in flam m ator y pseudot u m or On chocerciasis, 150 Open-angle glaucom a. See Glaucom a, open angle Oph th alm ic arter y occlusion , 266–268 Opt ic disk capillar y h em angiom a, 334, 335f m elanocytom a, 338, 338f Opt ic n er ve avu lsion , 26 gliom a, 81, 86 t ran sect ion , 26 Opt ic n er ve sheath, h em orrh age, 26 Opt ic n europathy, trau m at ic, 25–26, 25f Ora serrata, 296 Orbit foreign body in , 28–29, 29f fract u res, 26–28 in fect ion s, 69–71 in flam m at ion , 71–78 differen tial d iagn osis, 73t n eoplasm s, 79–94 t raum a, 26–32 Orbital balloon , tem p orar y, 303 Orbital em physem a, 26

Orbital h em orrh age, 26 Orbital in flam m ator y pseu dot u m or, 77–78, 78f, 97 Orbital varices, 85t, 93 Osteitis deform an s, an d angioid st reaks, 270 Osteogen esis im p erfect a blue sclera in , 133–134, 134f d ifferen tial d iagn osis, 191 Osteom a ch oroidal, 327–332, 331f d ifferen tial d iagn osis, 94, 327–331 Osteom yelit is, of p aran asal sin u ses, 69, 72 P Paecilom yces kerat it is, 149, 150 Paget disease, an d angioid st reaks, 270, 271 Palpebral fissure, 36 in blep h aroph im osis syn drom e, 48, 48f Pan n us, 191–192, 192f d ifferen tial d iagn osis, 118 Pan uveitis, 269 Papillae, 110 Papilledem a, 264, 335, 336, 337 Papillit is, 264, 335, 336, 337 Papillom a d ifferen tial d iagn osis, 54, 133 eyelid, 51, 51f p ostsurgical, 119f Paracentesis, an terior ch am ber, 268 Parafoveal telangiectasia d ifferen tial d iagn osis, 263, 269, 283 id iopath ic, 282–283, 283f m an agem ent , 283 p resen tat ion, 282 Paran eoplast ic syn drom e, 217 Parasit ic in fest at ion , conjun ct ival cysts in , 121, 122 Parat rachom a, 106, 107t Parin aud syn drom e, eyelid ret ract ion in, 43 Pars plan a, 296 breaks, 296 Pars plan a cyst , 290 Pars plan a pearls, 290 Pars plan it is, 200 d ifferen tial d iagn osis, 326 Par t ially accom m odat ive esot ropia, 342 Patau syn drom e, corn eal involvem en t in , 192 Pat tern dyst rophy d ifferen tial d iagn osis, 279, 280, 281 gen et ics, 281 m an agem ent , 281 p resen tat ion , 281 Pat tern st rabism us, 344–345, 345f A pat tern , 344–345, 345f V p at tern , 344–345, 345f Pavingstone degen erat ion , 290–291, 291f d ifferen tial d iagn osis, 286 PDR. See Diabet ic ret in opathy, proliferative Pellagra, 192 Pellu cid m argin al degenerat ion (cornea), 172–173, 173f at yp ical, 184 d ifferen tial d iagn osis, 171, 184 Pem ph igoid cicat ricial, 63, 192 d ifferen tial d iagn osis, 63, 110, 113, 192 ocu lar, an d d ist ich iasis, 64 ocu lar cicat ricial, 111–113, 112f an d sym bleph aron , 116 Pem ph igus, ocular, 191 Penet rat ing keratoplast y, 185, 186f graft reject ion in, 188, 188f Perioptic n eurit is, 78 Persisten t hyperplastic prim ar y vit reous, 326 Peters an om aly, 135, 190 PEX. See Pseudoexfoliat ion syn drom e

Index 551 Ph acoan aphylact ic uveit is, 12 Ph acod on esis, 13 Ph aco m ach in e an d con n ection of air p u m p to in fu sion bot tle, 226f prin ciples, 223, 223f su rge p h en om en on m ech an ism of, 224f preven tion , 225f Ph acom atoses, 135 Ph ar yngoconju n ct ival fever, 101–103 Ph en oth iazin es, corn eal vert icillata cau sed by, 180 Ph en oth iazin e toxicit y, 285 Ph lycten u lar keratoconju n ct ivitis, 192 Ph lycten u le, 118 Ph lycten u losis, 177 Ph otocoagu lat ion an d recu rren t corn eal erosion , 156 for ret in al detach m en t , 302 Ph otoph obia, in congen ital glau com a, 134–135 Ph ototoxic inju r y, 275 PHPV. See Persisten t hyp erplast ic p rim ar y vit reou s Ph th isis bu lbi, 136, 191 Picorn aviru s conju n ct ivit is, 101, 103–104, 103f Pigm en t at ion s, conju n ct ival, 131–132, 131f causes, 131 Pigm en ted dep osits, 129, 130, 133 Pillat scleral p laqu e, 140, 140f Pingu ecu la, 117–118, 117f, 118f differen t ial d iagn osis, 120 Plasm in ogen act ivator in h ibitor 1, an d cen t ral serous ch orioret in op athy, 274 Pleom orp h ic aden om a, lacrim al glan d , 97–98, 97f Pn eum at ic ret in opexy, 303 Poch in sign , 76t POHS. See Presum ed ocular h istoplasm osis syn drom e Polyarterit is nodosa, sclerit is in , 124 Polym orph ic am yloid degen erat ion , 178, 179f Por t-w in e stain, 126, 127f Posn er-Sch lossm an syn drom e, 196 Posterior am orp h ou s st rom al dyst rop hy, 166 Posterior em br yotoxon , 157, 158 Posterior sclerit is, 78, 124, 125 differen t ial d iagn osis, 329 Posterior vit reous detach m ent , and vit reous h em orrh age, 270 Pot assium hydroxide burn s, 5–7 Pre-Descem et dystrophy, 165–166 Pregn an cy, an d cen t ral serou s ch orioretin op athy, 273 Preser vatives, top ical, an d n eu rot ropic keratopathy, 155 Presu m ed ocu lar h istop lasm osis syn drom e, 202–203, 202f Prim ar y lip id keratopathy, 175, 175f Progeria, blu e sclera in , 134 Proliferat ive d iabet ic ret in op athy. See Diabet ic ret in op athy, p roliferat ive Proliferat ive ret in op athy, vit reou s h em orrh age an d, 18, 19 Propionibacterium acnes, p ostop erat ive en doph th alm it is cau sed by, 214, 241 Proptosis in Graves oph th alm opathy, 355, 355f in thyroid eye d isease, 43, 43f, 355, 355f Prost aglan din (s), top ical, 64 Prost ate can cer, orbital m etastases, 94 Proteus, corn eal u lcer caused by, 146 Pseu d oesot ropia, 49, 339f Pseu d oexfoliat ion syn drom e, 249–250, 250f

differen t ial d iagn osis, 280 Pseu d ogeron toxin , 173 Pseudom onas aeruginosa, corn eal u lcer cau sed by, 146 Pseu d oph akia, an d retin al detach m en t , 297 Pseu d opter ygiu m , 120 Pseu doptosis, 38 w ith m on ocu lar elevat ion deficien cy, 354–355, 354f Pseu dostrabism u s, 339–340, 339f Pseu d ot rach om a, 112 Pseu d ot u m or, orbital, 73t differen t ial d iagn osis, 76, 80, 86, 87, 93, 94, 128 in flam m ator y, 77–78, 78f, 97 Pseu d ovascu larit y, 333 Pseu doxan th om a elast icu m an d angioid st reaks, 270, 271 d ifferen t ial d iagn osis, 21 Psoriatic arth rit is, ocu lar involvem en t in , 195 Psych op h arm acological m ed ication s, an d cen t ral serous ch orioret in opathy, 273 Pter ygium (pl., pter ygia), 118–120, 119f d ifferen t ial d iagn osis, 118, 133 Ptosis, 36–41 ap on eu rot ic, 37f, 38 m an agem en t, 38 bilateral, 37f in bleph arop h im osis syn drom e, 48, 48f congen it al, 36, 37f m an agem en t, 38 con t ralateral, w ith ip silateral lid ret ract ion , 43 d erm atoch alasis an d, 42–43, 42f m echanical, 38, 59, 59f w ith m on ocu lar elevat ion deficien cy, 354–355, 354f m yogen ic, 38 n eurogenic, 38 overcorrect ion , 43 p resen tat ion , 36–38 Pu p illar y block, w ith len s su blu xat ion , 14 Pup illar y sp h in cter, p alsy, isolated, 348 PVD. See Posterior vit reous det ach m en t Pykn odysostosis, blu e sclera in , 134 Pyocele, 93 Pyogen ic gran u lom a, 45f Q Qu in in e toxicit y, 267 R Racial pigm en tat ion, 325 Radiat ion exposure, 112, 113 Radiat ion ret in opathy, 286–287 d ifferen t ial d iagn osis, 261, 263, 264, 286 p resen tat ion , 286 Radiat ion th erapy an d conju n ct ivalizat ion of corn ea, 136, 136f an d n eu rot rop ic keratopathy, 155 RCE. See Recurren t corn eal erosion React ive lym ph oid hyp erplasia, 80 Recu rren t corn eal erosion , 156 Red eye causes, 3, 126 d ifferen t ial d iagn osis, 126 Refractive accom m odat ive esot ropia, 341f, 342 Refsu m disease d ifferen t ial d iagn osis, 191 an d ret in it is pigm en tosa, 285 Reis-Bü cklers dyst rop hy, 160–161, 161f d ifferen t ial d iagn osis, 159 an d recu rren t corn eal erosion , 156

552

Index

Reiter syn d rom e, ocu lar involvem en t in , 194–195 ReNu w ith Moist u reLoc, 149 Ret icu lar d egen erat ive ret in osch isis, 294–295 Ret in a con t u sion , 266 cystoid degen erat ion , 288–289 ret icu lar, 288 t yp ical, 288–289 degen erat ion s, 288–291 develop m en t al an om alies, 288–291 edem a, p ost t rau m atic, 21–22 en closed oral bay, 289, 289f equ ator, 296 exophyt ic capillar y h em angiom a, differen t ial d iagn osis, 329 flecked, 280, 280f. See also Fu n du s flavim aculat us; Stargardt disease h em orrh ages, post t rau m at ic, 21–22 m ed ical, 260–287 m eridion al com p lex, 289 m eridion al fold s, 289, 289f n ecrosis acu te, d ifferen t ial diagn osis, 21 post t rau m at ic, 21–22 su rgical, 288–321 tears, post t raum at ic, 21–22 t um ors, 333–337 Ret in al breaks acu te p resen tat ion , 298 id en t ificat ion , 300–301, 300f localizat ion , 300–301, 300f, 305–306, 306f op ercu lated , 297f, 298 t ypes, 297–298, 297f Ret in al cap illar y h em angiom a, diabet ic, 269 Ret in al det ach m en t , 296–305 acu te p resen tat ion , 298 after cataract su rger y, 239–240, 240f ap h akic, 239–240, 240f causes, 21 cen t rifugal forces an d, 296 differen t ial d iagn osis, 19, 274, 295, 298–299 exu dat ive ch aracterist ics, 299t differen t ial d iagn osis, 285, 298, 299t localized serou s. See Cen t ral serou s ch orioret in op athy m an agem en t, 300–305 ou t p at ien t tech n iqu es, 303–305, 303f, 304f, 305f su rgical failu re, 303 path ology, color-coded diagram , 306, 307f post trau m at ic, 21–22, 22f rh egm atogen ou s, 239–240, 296 ch aracterist ics, 299t differen tial d iagn osis, 298, 299t epidem iology, 296 m an agem ent , 300–301, 301f presen tat ion , 296–298 risk factors for, 296–297 t ract ion al ch aracterist ics, 299t differen tial d iagn osis, 298, 299t in proliferat ive ret in opathy, 261, 263f Ret in al dialysis, 297f, 298 an d ret in al detach m en t , 21, 22f Ret in al pigm en t degen erat ion , secon dar y, in syph ilis, 198 Ret in al pigm en t ep ith eliu m congen it al hypert rop hy, 332, 332f differen tial d iagn osis, 327, 328, 330, 331, 332 h em orrh agic det ach m en t , differen t ial diagn osis, 329

hyperplasia, differen tial diagn osis, 327, 328, 330, 331, 332 in farct ion s, 260–261 p osth em orrh age h em osid erin dep osits, d ifferen tial d iagn osis, 332 t u m ors, 332–333 Ret in al tears, 296–305 flap , 297, 297f h orsesh oe, 297, 297f Ret in al telangiectasis an d cystoid m acu lar edem a, 274 differen t ial d iagn osis, 275 Ret in al t u fts, 293–294 cyst ic, 293 n on cyst ic, 293–294 zon ular t raction , 294 Ret in al vein occlu sion , an d cystoid m acu lar edem a, 274 Ret in it is acu te, d ifferen t ial diagn osis, 264 cytom egaloviru s, 208, 208f differen t ial d iagn osis, 266 Ret in it is p igm en tosa, 284–285, 284f an d cystoid m acu lar edem a, 274 differen t ial d iagn osis, 285 m an agem en t, 285 ocu lar associat ion s, 285 presen tat ion , 284–285 system ic associat ion s, 285 Ret in it is pu n ct a albescen s, 279, 280 Ret in oblastom a, 216t, 333–334 associated m align an cies, 334 differen t ial d iagn osis, 201, 216, 323, 324, 326, 333, 335, 336, 337 ep idem iology, 333 gen et ics, 333–334 m an agem en t , 334 presen t at ion , 334 vit reou s h em orrh age w ith , 18 Retin op athy cen t ral serou s, 263 t um or-associated, 217 Retin op athy of p rem at u rit y, an d degen erat ive m yop ia, 272 Retin op exy, pn eu m at ic, 305 Retin osch isis differen t ial d iagn osis, 298–299, 299f ret icu lar degen erat ive, 294–295 sen ile, 294–295 Rh abd om yosarcom a, 87–89, 88f–89f differen t ial d iagn osis, 59, 78, 94 Rh egm atogen ou s ret in al det ach m en t , 239–240, 296 ch aracterist ics, 299t differen t ial d iagn osis, 298, 299t ep idem iology, 296 m an agem en t , 300–301, 301f presen t at ion , 296–298 risk factors for, 296–297 Rh eu m atoid arth rit is ep iscleritis in , 123 perip h eral u lcerat ive kerat it is in , 182–184, 183f sclerit is in , 124 Rh exis in m at u re cat aract , 222–223, 223f–226f in sen ile cataract , 226–227, 226f–229f Rieger an om aly syn drom e, 135 Riesm an sign , 76t Rosacea differen t ial d iagn osis, 182, 183 ocu lar, 100–101, 100f RPE. See Ret in al p igm en t ep ith eliu m RP inversu s, differen t ial diagn osis, 279, 280 RRD. See Rh egm atogen ou s ret in al det ach m en t

Index 553 Rubella, 135 congen it al, 285 Rubella ret in opathy, 281, 282 Rubeola, corn eal involvem en t in , 150 Rubeosis iridis an d hyp h em a, 15 vit reou s h em orrh age an d, 18 S Salzm an n n od u lar degen erat ion , 177–178, 177f Sarcoid at ypical lym p h oid in filt rate, 57 differen t ial d iagn osis, 57, 323 Sarcoidosis, 97 cardiovascular involvem en t in , 199 differen t ial d iagn osis, 80, 112, 150, 263 ocu lar involvem en t in , 198–199 pu lm on ar y involvem en t in , 199 vit reou s h em orrh age w ith , 18 Satellite lesion , in adu lt toxop lasm osis, 201 Scarring ch orioret in al, 332 corn eal cen t ral, 164 in HSV kerat it is, 141f from infect ion, 177 in terst it ial kerat it is an d , 150, 151f in Steven s-Joh n son syn d rom e, 113 st rom al, 192 of lid m argin an d dist ich iasis, 64 an d trich iasis, 64 u pp er eyelid, 43 Sch nyder corn eal dyst rophy. See Cen t ral cr ystallin e dystrophy of Sch nyder Sch w an n om a, 90 Sch w ar t z-Jam p el syn drom e, 217 Scleral bu ckling en circling, 302, 302t proced u re, 305–316 color-coded d iagram , 306, 307f drain age of su bret in al flu id, 314–315, 314f, 315f exoplan t m aterial, 308–310, 309f localizat ion of ret in al breaks, 305–306, 306f parallax associated w ith view ing, 308, 308f sh aft ing du ring localizat ion , 308, 309f su t u re p lacem en t for silicon e exp lan t , calculat ing distance of, 310f, 311–312, 311f–313f su t u re t ying, an terior sh ift ing, 314, 314f su t u ring tech n iqu e, 310, 310f for ret inal detachm en t , 302 segm en t al, 302, 302t Scleral ectasia, 140 Sclerit is, 124–125, 125f an terior, 124 differen t ial d iagn osis, 124, 329 n ecrot izing, 124 posterior, 78, 124, 125, 329 Sclerocorn ea, 158 differen t ial d iagn osis, 157, 192 Sclerom alacia, 140 Search ligh t in fog, in adu lt toxop lasm osis, 201 Sebaceou s cell carcin om a, 62–63, 62f differen t ial d iagn osis, 46, 101, 133 Sebaceou s hydrocystom a, 57, 58f Seborrh eic blep h arit is, 100 Seborrh eic keratosis, 52, 52f differen t ial d iagn osis, 53, 54, 64 Sen ile ret in osch isis, 294–295 differen t ial d iagn osis, 21 Sen ile tapetoch oroidal degen erat ion , 291

Sen sor y exot rop ia, 344 Serp igin ou s ch oroidit is, 205–206, 206f Serrat ia corn eal u lcer, 146 Seton im plan t at ion , 252, 253f Sh afer sign , 297 Sh aken baby syn drom e, 18 Sh ield ulcer, 146, 152–153, 153f Sh ingles, 143 Sickle cell an em ia, an d angioid st reaks, 270, 271 Sickle cell ret in op athy, 283–284 differen t ial d iagn osis, 263, 286, 335, 336, 337 m an agem en t , 284 presen t at ion , 283 proliferat ive, an d vit reou s h em orrh age, 270 Siderosis, 23–24 Siegrist st reaks, 261 Silden afil cit rate, an d cen t ral serou s ch orioret in opathy, 273 Sin u sit is, an d m u cocele, 93–94, 95f Sixth-n er ve palsy, 350–351, 351f Sjögren syn drom e, differen t ial diagn osis, 112 Skin t ag. See Acroch ordon Sling surger y conven t ion al, 37f Jacob-Agar w al, 37f, 38–41, 39f–41f Sn ap -back test , 33 Sn ellen sign , 76t Sodiu m hydroxid e bu rn s, 5–7 Solar ret in it is, 287 Solar ret in opathy, 287 Sp h eroidal corn eal d egen erat ion , 178 Sp h ingolip idoses, 267 SPK. See Superficial pun ct ate kerat it is Squ am ou s cell carcin om a, 61–62, 61f conju n ct ival, 137 cyst ic, 61f differen t ial d iagn osis, 51–54, 59, 64, 113, 133, 137 n odu lar, 62 plaqu elike, 62 u lcerat ing, 62 St ap hylococcal hyp ersen sit ivit y, 152 St ap hylococcal m argin al u lcer, 177, 182 Staphylococcus coagu lase-n egat ive, p ostoperat ive en doph th alm it is cau sed by, 241 corn eal u lcer cau sed by, 146, 177, 182 trau m at ic en doph thalm it is caused by, 213 Staphylococcus aureus blep h arit is cau sed by, 106 h ordeolu m cau sed by, 46–47, 46f postop erat ive en doph th alm it is caused by, 214, 241 trau m at ic en doph thalm it is caused by, 213 Staphylococcus epiderm idis, postoperat ive en doph th alm it is cau sed by, 214 St ap hylom a(s) an terior, an terior segm en t t ran sp lan tat ion for, 189–191, 190f congen it al, 272 posterior, w ith degen erat ive m yop ia, 272 scleral, 134–135, 135f differen t ial d iagn osis, 140 St argardt disease, 278–279, 279f differen t ial d iagn osis, 279, 280 m an agem en t, 279 presen tat ion , 278, 279f Stellw ag sign , 76t Steroid s exogen ou s, an d cen tral serous ch orioretin opathy, 273, 274 injection s, 267 an d su bconju n ct ival h em orrh age, 3

554

Index

Steven s-Joh n son syn drom e, 113 differen t ial d iagn osis, 136, 192 an d dist ich iasis, 64 an d sym bleph aron , 116 an d trich iasis, 64 St ickler syn d rom e, an d degen erat ive m yop ia, 272 Stocker lin e, 179 St rabism u s, 339–358 pat tern , 344–345, 345f St raw berr y n evu s, 81 St reptococcus corn eal ulcer caused by, 146 grou p B, n eon at al en dogen ou s en doph th alm it is cau sed by, 215 postoperat ive end oph th alm it is cau sed by, 214, 241 traum at ic en doph th alm it is caused by, 213 St reptococcus pyogenes conju n ct ivit is, 105 St riate kerat itis, after cataract extract ion , 237f St riate m elan okeratosis, corn eal vert icillata in , 180, 181f St ring of p earls app earan ce, 215 St u rge-Weber syn d rom e, 135, 330 differen t ial d iagn osis, 325 dilated episcleral vessels in , 126–127, 127f Su bconju n ct ival h em orrh age, 3–4, 4f Su bretin al h em orrh age, 327, 328, 330, 331 Su ker sign , 76t Su lfite oxidase deficien cy, an d len s su blu xat ion , 13 Su lfu ric acid bu rn , 5–7 Su lfu rou s acid bu rn , 5–7 Su p erficial p u n ctate keratitis, w ith ch em ical burn , 5 Su p erior lid crease, 36 Su p erior lim bic keratoconju n ctivit is, 122–123, 122f differen t ial d iagn osis, 63, 192 an d filam en tar y kerat it is, 154 Su p erior obliqu e p alsy acqu ired, 349 bilateral, 349–350, 350f congen it al, 349 u nilateral, 349f, 350 Su perior rect u s m uscle palsy, isolated, 348 Su pranu clear p alsy, 354f Sym bleph aron , 116–117, 116f in Steven s-Joh n son syn d rom e, 113 Sym pathet ic oph th alm ia, 213 Syph ilis acqu ired an d in terstit ial kerat it is, 150, 151 an d len s su blu xat ion , 13 ocu lar involvem en t in , 198 congen it al an d in terstit ial kerat it is, 150, 151, 151f ocu lar involvem en t in , 198 differen t ial d iagn osis, 285 vit reou s h em orrh age in , 18 Syringom a, 57–58 System ic lupus er yth em atosus an d cen t ral serous ch orioret in op athy, 273 perip h eral u lcerat ive keratit is in , 183f sclerit is in , 124 T Taches de bougie, 199 Tapetoch oroidal degen erat ion , sen ile, 291 Tay-Sach s disease, 267 Tear deficien cy an d filam en tar y kerat it is, 154 in Steven s-Joh n son syn d rom e, 113 Tears, art ificial, 114–115 Telecan thus, in bleph aroph im osis syndrom e, 48, 48f

Tem porar y orbital balloon, 303 Tenon it is, 78 Terrien m arginal degen erat ion , 119f, 184–185, 184f differen t ial d iagn osis, 172, 183, 185 Th erm al bu rn (s), 6, 112, 192 Th ird-n er ve p alsy, 347–349 com p lete, 348, 348f congen it al, 349 differen t ial d iagn osis, 349 in ferior, 348, 348f m an agem en t, 349 pu p illar y involvem en t, 348, 349 secon dar y, 349 su perior bran ch , 348, 348f Th rom bosis, cavern ou s sin u s, 71–73 differen t ial d iagn osis, 69, 76, 78, 93, 128 presen tat ion , 71 Thygeson su perficial pu n ct ate kerat it is, 152, 152f Thyroid eye disease, 74–77, 74f–75f differen t ial d iagn osis, 78, 93, 128 dilated vessels in , 126 exoph th alm os in , 73t eyelid edem a in , 47, 47f eyelid ret ract ion in, 43, 43f m an agem en t, 76–77 proptosis in , 43, 43f Thyroid-related op h th alm opathy, 74–77, 74f– 75f. See also Graves oph th alm opathy; Thyroid eye disease differen t ial d iagn osis, 78, 93 in filt rat ive (severe), 74 m an agem en t , 76–77 n on in filt rat ive (m ild), 74 Wern er classificat ion , 74 Tilosis, w ith blep h arit is, 100 Tolosa-Hun t syn drom e, 77, 128 Toxic epiderm al n ecrolysis, 112, 113 Toxocariasis, 201 Toxoplasm osis adu lt , 201 congen it al, 200 ocu lar involvem en t in , 200–202 Trach om a Chlam ydia, 106–109, 107f, 107t–108t differen t ial d iagn osis, 110, 112, 192 an d sym bleph aron , 116 an d dist ich iasis, 64 an d trich iasis, 64 Trau m a, 1–32 an terior segm en t , 1–17 an d bacterial corn eal u lcer, 146 carot id cavern ou s fist u la caused by, 31–32, 31f, 90, 93 cat aract cau sed by, 11–13, 12f cerebral, in p ed iatric pat ien t , 201 corn eal involvem en t in , 192 differen t ial d iagn osis, 69, 72, 324 en doph th alm it is cau sed by, 213–214 an d fu ngal kerat itis, 149 lacrim al system , 2, 3f an d len s su blu xat ion , 13 opt ic n er ve, 25–26, 25f orbital, 26–32 pen et rat ing, 21 perforat ing, 16–17, 17f posterior segm en t , 17–26 an d ret in al d etach m en t , 297 an d sym bleph aron , 116 to t rigem in al ner ve, and n eurot ropic keratopathy, 155 an d vit reou s h em orrh age, 270 Trich iasis, 64, 65f w ith bleph arit is, 100 differen t ial d iagn osis, 35, 110

Index 555 pem p h igoid an d, 112 in Steven s-Joh n son syn d rom e, 113 Trisom y 13, 135 Trisom y 15, 135 Trisom y 21, 49. See also Dow n syn drom e Tuberculosis ch orioretin it is in , 201 an d in terstit ial kerat it is, 150, 151 ocu lar involvem en t in , 199, 199t Tum or(s) an d bilateral superior obliqu e palsy, 349 ch oroidal, 326–332 differen t ial d iagn osis, 274 ciliar y body, 323, 325–326 eyelid, 51–57, 59–64 fu n dal, p igm en ted , 333 in t raocu lar an d cystoid m acu lar edem a, 274 an d vit reou s h em orrh age, 270 of iris, 322–325 lacrim al glan d, 97–98 orbital, 69–99 differen t ial d iagn osis, 128 ret in al, 333–337 an d ret in al d etach m en t , 298f ret in al p igm en t epith eliu m , 332–333 Turn er syndrom e, 135 Type A p erson alit y, and cen t ral serou s ch orioretin op athy, 273 U Ulcer corn eal from Acantham oeba, 147, 148f bacterial, 146–147, 146f differen t ial d iagn osis, 7, 143, 146, 153, 192 fu ngal, 149, 149f h erp et ic, 192 den d rit ic, 104f im m u n e-m ediated , 146, 147, 153 in fect iou s, 153 m argin al in HSV in fect ion , 142 st aphylococcal, 177, 182 sterile, 146 n eu rot ropic in HSV in fect ion , 142, 143 in HZV in fect ion , 144f in Steven s-Joh n son syn d rom e, 113 trach om a, 107f viral, 146 den drit ic differen t ial d iagn osis, 3 h erp et ic, 104f h erp et ic den d rit ic, 104f differen t ial d iagn osis, 7 Ulcerat ive colit is, ocu lar involvem en t in , 195 Ulcerat ive kerat it is differen t ial d iagn osis, 6, 182, 184 perip h eral, 182–184, 183f Ultraviolet (UV) kerat it is, 6 Ush er syn drom e, an d ret in it is p igm en tosa, 285 Uveal effu sion syn drom e, 325 Uveit is acu te an terior n ongran u lom atou s, 194–197 in an kylosing sp ondylit is, 194 an terior, 10 in Beh çet syn drom e, 212–213, 212f ch ron ic n ongran u lom atou s, 196–197 congen it al syp h ilit ic, 198 an d cystoid m acu lar edem a, 274, 276 differen t ial d iagn osis, 124, 125, 324 disord ers m asqu erading as, 216–217, 216t

glau com a-related, 196 gran ulom atous, 198–199 HLA-B27-associated , 194–195 in in flam m ator y bow el disease, 195 in term ed iate, 200 in juven ile rh eu m atoid ar th rit is, 196–197 p h acoan ap hylact ic, 12 p h acolyt ic, 196 p osterior, 200–209 in psoriat ic ar th rit is, 195 in Reiter syn drom e, 194–195 in sarcoidosis, 198–199 t u bercu lou s, 199, 199t in uveit is-glau com a-hyp h em a synd rom e, 196 vit reous h em orrh age w ith, 18 Uveit is-glau com a-hyp h em a syn d rom e, 196 V Valsalva ret in opathy, 263 Varicella-zoster virus. See also Herpes zoster acu te ret in al n ecrosis syn d rom e cau sed by, 209 in fect ion , 105 Vascular lesion s, orbit al, 85t, 90. See also Cap illar y h em angiom a; Cavern ou s h em angiom a; Lym ph angiom a; Orbital varices Vasculit is d ifferen tial d iagn osis, 269 ret in al, an d cystoid m acu lar edem a, 274 Verruca, 58 VH. See Vit reous h em orrhage Viral in fect ion , corn eal, 146 Viral kerat it is, 149, 150 Vision loss, w ith ret in al breaks/ret in al d etach m en t , 297 Vitam in A d eficien cy, 285 Vitam in B deficien cy, 192 Vitelliform m acu lar dyst rop hy, 280–281 Vit iligin ou s ch orioret in it is, 206–207.207f Vit rectom y for ep iret in al m em bran e, 321 25-gauge t ransconjun ct ival, 305, 305f p n eu m at ic, 305 p rim ar y, 303 an d recu rren t corn eal erosion , 156 Vit reom acu lar t ract ion syn drom e, an d cystoid m acular edem a, 274 Vit reoret in al adh eren ce, focal, an d t rau m at ic ret in al detachm en t , 21 Vit reou s d etach m en t , p osterior, 295–296 d ifferen tial d iagn osis, 295 an d retin it is pigm en tosa, 285 Vit reou s h em orrh age, 270 am blyop ia an d, 19 cau ses, 270 diabet ic ret in op athy an d, 270 differen t ial d iagn osis, 18, 270, 295 Eales disease an d , 270 an d exu dat ive age-related m acu lar degen erat ion , 270 an d gh ost-cell glau com a, 18–19 an d h em osiderosis bu lbi, 19 in h istoplasm osis, 18 m an agem en t, 270 in m elan om a, 18 posterior vit reou s detach m en t an d , 270 post t rau m at ic, 17–19, 18f, 270 presen tat ion , 270 an d p roliferat ive ret in opathy, 18, 19 proliferat ive sickle cell ret in op athy an d, 270 w ith ret in al breaks/ret in al detach m ent , 297 w ith ret in oblastom a, 18 sp on tan eou s, 18 in syp h ilis, 18

556

Index

Vit reou s h em orrh age (cont inued) traum at ic, 17–19, 18f, 270 in ch ild abu se, 18 Vit rit is in ad ult toxoplasm osis, 201 differen t ial d iagn osis, 295 Vogt-Koyan agi-Harada syn drom e, 210, 210f, 211f Vogt lim bal gird le, 174, 174f Vogt st riae, keratocon u s an d, 171 Von Grafe sign , 76t von Hipp el-Lin dau disease, 334 Vossius ring, 11

Wegen er gran u lom atosis, sclerit is in , 124 Weill-March esan i syn drom e, 135 an d len s su blu xat ion , 13 Weiss ring, 295 Wet sn ow, in adu lt toxop lasm osis, 201 W h ite dot syn drom e, 203–204 Wilder sign , 76t Wou nd leak, after cat aract su rger y, 237– 238

W Warfarin , an d su bconjunct ival hem orrh age, 3

Z ZTTs. See Ret in al t uft s, zon ular t raction

X Xan th elasm a, 57, 58

Color Atlas of Ophthalmology PDF - PDFCOFFEE.COM (2024)
Top Articles
Latest Posts
Recommended Articles
Article information

Author: Moshe Kshlerin

Last Updated:

Views: 6415

Rating: 4.7 / 5 (57 voted)

Reviews: 80% of readers found this page helpful

Author information

Name: Moshe Kshlerin

Birthday: 1994-01-25

Address: Suite 609 315 Lupita Unions, Ronnieburgh, MI 62697

Phone: +2424755286529

Job: District Education Designer

Hobby: Yoga, Gunsmithing, Singing, 3D printing, Nordic skating, Soapmaking, Juggling

Introduction: My name is Moshe Kshlerin, I am a gleaming, attractive, outstanding, pleasant, delightful, outstanding, famous person who loves writing and wants to share my knowledge and understanding with you.